~ ~

,

HIGH YIELD NOTESPATBOILOGY 0

us!tfLE pictures; see h~d()utdescrl~ingb.igh.yieldpietures in ~obhin's Sixth edition ,U. '·-S'··'-··ML·-"-··· 'E-·" ,b· ema'tology'-' .:' p'lctures not 10 R.obblDS,: • ,supraV1"'~:t""_l- sta "~n"" sh -.-".' , " ". -,' ". ., -, -

.. , -' " " _ . :.:Wl '--';( '·,:QMDO' ...... ticu,l,,',-,-,' ''':'''' -, '-,,- "

b h '!"l· tio 1"'" -'" 'P"b · .. - - -,Ct. "u' "" "QC,yte RNA'

'filaments, • coarse ,aSQP: t tc S 'pp tng .In', -'-' porsonmg; '. x"'ray" with d _.,~ty-- " "', ,-, .-- -., -,' ,', : .. ' -

, '.' '.' -" - I. ~' ' " _, . ',' . _ ,_._, " , ~ J) "-' ,,' " "" 1,enSl -,' III eplphyses- Ph

poisoning. • ringed sIdcrob!~st, .~ acute I'ro~ul~yl1C leukemia- lots of Auer rods "i ...•.. '

D"'C h" - 'H'-..J,' kin" 'S' Iymphomaen ,- - I' -', h' and, Hx of

I ..' - '. gro' '- SS 01' non~" vug',' ,_: -,' 'J""'~!II' ," '- ',:, ~ .. a'Ctrc. lng· trachea : an td arch V"", '1'-' '~- .•. 'L -,.... ,-~, '- ' :

-' - ~ , ' , '-' . - '-, -c;q, liWf esse S WI 1.lI\.Jut ' vad'"

them. • micro ,of '8 cell fol.lic:ular lymphoma, • 'multiple, myeloma marrow "~.'L plasma · '1:~ '. ~tng,

- ••.. - . _. • -, .'. _ .' _. .-, .. ..... - i '. " .' , •. .. _ " ., • " . '" i,UI ,- ~...:.;t,(llQ eel~ BJ' 'in

unne, • Reed-Sternberg cell. • applegreen birefringence of amyloid •• back of hand 'r"lld! .. -_-'

. '. 4th-- ·1 - , ",' '- - , ' ,--'" --',' 'f-" "ld -, ]- '"'-" . ,. ' ,~Q I el 'erlv

.. ,~ - - . ,- - - -, ' , ' . • arms 0 e - ' '. -. , --- . ~ , J'.

'pa'tleJl't 'WI,: '.' SCDl·e purpura, ',' .-:. ',' .:'~,r::r. y patient with multiple bruises- probabl" -, ab r'. 'I

midbrain with yellow discoloration 0 f kernicterus •• fetus with hydrops fetalis .... Y a. use, •

US~EcardiovasctllaJ;" pictures not in Robbins: .x.anthe]asma. ". Achilles tendon :xanthomapathognomonic. of !"amHi~~ hYJ.lercbolesterol~a.. turbid supranate (chylomi~) and iIifranate (VLDL}t - stasis dennatltls.of lower leg- SIgn of deep venous thrombosis, - Osler-Webcr""Rendu

di- I..·... fin' d bran -

sease-- telangiectasia 'Oi1,,_,,-,;'gers an'" mu~us memb ... ,. nes, • 'spider telangiectasia, '. lYll,lphedeutd,

t 'l"'ll"i6 st '. t· ..' Ka' po- SIIS sarco" ... Tn,~ • m·, r-Y"'O of . '" " U ] ,: - .. ~ .

pos ":I .. ,~,_e.c' ,omy~, ' .. ' : " ": ,: ,:. '.' " _~~~'" '''~''''';4,' ':, .' ~ - ' vess,e,·. 'V3SC,U1I.tIs. with fibrinoid necrosis, .'

J ' .. :0.' i; bI- . ..» ". ith H 'h" . "

thromboangiitis 0 uiterans • patient VII.' -', ",'~noc':-S:c'h,onle~n, purpura, .' gross: 'of renovascular

hypertension due to' atherosclerosis,. capillary hemangioma onthe face of a newbom,. Stnrge Weber-- po .,: rt wine nevus in trigeminal nerve distribution • gr .. ·05S 'and 'micro of pul irnonarv .. ,:i...,._"",,' .

. ", .. ".-" ,... -" _ . ' , -, , ..•..... ,_ .••..• ',' < '.,' ,~"'- ""~'~,J ~\U.Ld..,I·'

coronary artery« .' identify platelet thrombus, *. fibrofatty plaque, .' dystrophic calcification, • atherosclerosis (empty slits), • section through ascending 'aorta-.., 'what part dilated with postductal

. '. ' ".+ ? ... - - I . . .'. tur -, f' ',' , t' ,-', ,- di 1-' ,., "1'1" \ i --,' -. .

cearctatton rproxrma aorta, " rupnn e oi posterome na papi tary muscle In an ~ .. rupture of

anterior wall of" 'LV' in an ~ • ruptured .interventricular septum in patient with an acute myocardial infarction- rupture occurs in site of coagulation necrosis and leads to right heart fai I ure , • mural thrombus in 'an Nvn, .' fibrinous pericarditis, '. old A1vfi' with scartissue. on anterior walldo not confuse with a pale infarct, • 'valve. 'vege:tations-, - MV" vegetations in rheumatic fever: along the lines of closure of the valve, • infective endocarditis: see' des' tructior of 'valve. .' marantic,

_' f, , ,- -_ ' .' - , " __, ; _ ' -, '.' _ ., ',- , , _ ,_ n, , ,- '11 - - - '"

"""ta·'tJ..'"_· .. MV"··'''''. ,;, ""1'" ;-' annearanceto rhet ticf - .·Hx'·- of colo . ",-, ulcerative

ve,gc·· :',ons on", '. '.' - SlIm ar 10 . ,Py'_:'_M. .. ·· " .. T .. ,cuma'·c ,l,ev er" '" 0 coon, cancer or u,c '. ,"'VC:,

colitis, '. Libman Sacks endocarditis: occur on, MV' in patients' with S"L'E, .. gross of 'mitral valve '~, prolapse, • micro '0'£ myocardium with Iymphocyric infiltrate ....... coxsackie myocarditis

tlS·l\ILE: respiratory pictures, 'not in Robbins: • "x'-Ta.Y with spontaneous pneumothorax (tracheal Shift, to; side of the' pneumothorax) or tension pneumothorax (tracheal shift to co'n~.la,teral side), ,.' ar"'2rn ~~. . f St 't-" " o~ x y- -0 gross -, 0-' f 10- '-b', ar pneumonia • '(:rrnm stain of

~""""~,,, .~.~m, 0, : r~p, ococcus ,pneumQnl:Q;e, '. , .' ·,ra,'.f' :,'.; ,',,: I', ',', ..•. ':, r ',,'- :- .... " , "~-",,, .• , ",

fil __ ento ·'U·S·, uriI'm ',,',' .. iti .. ,,'-, bacter -a- Actinomyces o-r Nocardia. (P" artially acid fast), • micro of

_LdU~l., . t:7"'-f>" poSl,,-,ye _ 3_ n . - , ,- .,' --, - ._ - .

macrophage with Histoplasma yeasts, • gross of cavitary apical lesion ofTB- possible acid-fast

.stain of organisrrts.,. micro of a macrophage. With acid-fast bacteria+ TB. • micro of'broad based bud of Blastomyces, " micro, of wide-angled non-septate hyphae. of Mucor > • gross of lung abscess, =micro of non-cas~tin.ggranuloma in sarcoidosis? .• gross of Ha.mman-rucb honeycomb lung. • )(-

'ray o:f classic' 'COPD, • gross of ma'in-stc,m primary lung'. cancer . ,~

USMl.Egasti'ointestiJ1al picture.snot in Robbins; • pa.timtwiili He~s sirople~ • exudative tonsillitis,.. hairy leukoplakia, ., thrush with or without gram stain of pscudohypbae and yeas~. • gross or two tongues with Icukoplaki.a~one tongue is squamous dysplasia and the other is in~lve

" -, -, ,,', - ("1- "k' &'. _... . ' .. · .. th-· --'",' - ,'.,-, ',- ""',' 'P"'e--u,,~ ... Jeg,hers, ,hp.s .• '

cancer.' 09"" lQr asymmetry o'f to'n,gue.).). • lo'w:er llP VII •. " squamous 'can,cct2/· : .. -~, , '.' "." ~ .. _

,;. - - - -" . 4 ,-, r-j'Ze' - nk' - t. d' ··rt1'· .. ·u)'-Uft1 • ornSS ,of

patient WIth, nuxe.d, tumor ~fp'arotid', • gross or banum snldy O",'_", __ ,ers 'lV~_ .. ~,~ ,~,,!!.,,- O~'- "

esophageal cancer- think squamous if mid~sophagus or adenocarcinoma if dista1 esophagus. • x-.

f ]" + ,,-, " " .... , , ," '-, .,' '- 1'" "d ,- '.' "I d'de' 'r'- e"ffi' ect' • gro .. ,.' S$ of

ray 0' ,e 8SS10Small bowel ObStructlo,n WIth 31r-flut.d, leve',~ an .. , st,ep~' a".-- ,-' '~ - -, -.

i,ntussusceptionin a child.. • micro' of absent gangIloD cells in rectal hi.opsyin Hirschsprung"s, •

'!

'-N'" .- .... TIl' '-Is'" R1I.' -.,·.,iiiiilln· ·', .. '1 il;.,eoPy·rlg'hted. All rigbts neserv~. Ed".rd ·CoI)., M.D .. lOOl.

. 01'" "". ' 1Ij.~ _, '.

. .~.. . ...' '

[

. ,

,.. cum". gr ·os-'s· of c .' "dl tu (y 1- I

micro of"angjodysplasla I:n':~~ .; ."': ~.: ':. ,:'. _:C:~Ir:'CJ1._~. l_·_~r,.··:e. .low co1or) of li-,- of .. 3' . : ... ~. .' , .

... robe sed inten)al hemQtr:hOld- pro~dmg mass •. from _an~ In adult ip P'PCtld.ix.,.,

PU·"'-S·."!.IC". b·,ep.atobilia·ry/pa:Dcreas pictures Dot 1'0 Robbins: .• eyes '\\lith .l~·aund·I"~. -, " . "." ". ". r.'

, .. .I ......... 1:.j - ' - ". . . "'"'-'.J." gross or'rm' -

of amebic Hvcrabsecss or flask rshaped ulcers -in ceCUm- 190k fOr tmphozoites ~h .. ere

'L'A." '.' ce' .'. yt'o·st~s··· .'. gr~s's of liver echinococcosis, .. gr'.' ~O"SS':: or micr "':0", of . o"'b's'tru' .. "C'tl"I''i~ r: : di .:t... RBc'

'pl,usg . ".' ,'. '" " . . .'IV.. -' . . . . , , " '..,',.' ", ...._:' J aun i Ct..... l~'" - ~ . -

g ~. look .... fo r s·-tone· in common 'bile' duct, ... Kayser-Fle:isc.her ring',: in Wl··.l.sn.o"s. d:·'l.S·P~P- . '.' ·lVer 'is '~

·1",";"1, &,., .. l,~ '. " . v. '~' 'reOl' bet ,~

(otal~Pper is low, ceruJ~pl3Sm,in.is l~wf ~d free copper is hi~ ... patient wi1h~~:', ~tnt_, ~.

h ._' .. ' ·1· ,,,"fi"C3' '11'0'" .,' .' ·th·, "." .,' -_. f hr " . puytren,s

contrae~f • X.-I1i~ '8 ow~n~~C~1 .. ns in me pancreas in cnromc pancreatitis" ~ gross ,of r\,',

pancreatic canc~ of head o~f pancreas _ .. ... . .. . .

US}\;U.J: geo.itonrhrary {Jlctures Dot In Robbins: ;C3sts in ~ne- 'hyalme (fuzzy and round borders) vs waxy (sharp borders), RBC cast. renal 'tubular cast, fatty cast with Maltese erosses.» , cells in urine- 'WBCs, RBCs. • crystalsm urine- uric acid, calcium OXalate, C}'Stinecrystals:. \ horseshoe kidney, • gross or x~ra?, of duplicat~ ~eter, • ~<Jss of bilateral polycystic kidney disease in a 'child, .tt. gross of adult polycystl.c. disease with or without brain vi-ith either .a subarachnoid bleed (rup~dbelIY aneurysm} or intracerebral-bleed (hyPertensive bleed), -linear immunofluorescence- tliink Goodpasture's syndrome. • lUUlPY bumpy JF- thiN; type m immunocomplex glomerulonephritis; • EM pictures- fusion of poooeytes in lipoid nephrosis, subepithelial deposits (post-streptococcal, membranous), subendothelial (~ 'IV SLE,

membran.op:ro~liferatiyeJ., • micra of nodular glomerulosclerosis in D'M- look for hyaJ:in~ arteriolosclerosis .. 0'[ afferenvefferent and 'pink balls in rnesangium, "Christmas ball" 'disease •• micro of ischemic ,A·TN_. look for coagulation necrosis of tubular cells." • picture of kidney and show: where Hg-or Pb .damages - 'proximal tubule, • gross of staghorn calculus, • gross or micro or renal infarction- look for pale, sunken areas on the, cortex and coagulation 'necrosis, • gross of 'benign nephrosclerosis-: granular appearing cortical surface, • gross O.r malignant hypertensionlook for 'petechial lesions orr surface of the kidney, •. micro of foreign body giant cell reaction in tubules- Bfprotein 'reaction in multiple.myeloma, • gross ofprostate hyperplasia- bulging.nodular surface with.occlusion ,o'f the, urethra, - patient with Klinefelt<:rs- look for signs of feminization, • patient with testicular feminization= looks like a woman but they will givehistoryof blind vaginal pouch, '. gross- of; renal a'denocan;ulo:ma- yellow mass, with hemorrhage, yellow mass, extendingup the inferior vena cava

U.SML,E 19yif.ecologypictu·r'es' not in, R;Q'b'bins: .' Herpes genitalis with 'or without a, TZanCK prep showing multinucleated squamous cells with intranuclear .inclusions, • condyloma a.cumiDatalooks "like' 'a, :fCID1 Hf'V 6/11, ,e Chlamydia- cell with vacuole 'In .the cytoplasm containing an elementary body. _. cervix; or' penis with thick exudate of GC' with or without gram stain showing gram -negative diplococci in. polys, .·clue cell of Gardnerella vaginalis= squamous ce'll covered by' 'bacteria, • 'primary 'syphilitic chancre on penis 'or labia- iii painless, .• ifpainful look for Rem~philus' d"Cteyi~ ..• picture. of lymphogranuloma venereum- C.. trachomatis subtype, papules 'with: draining sinusfrom nodes; lymphedema •• Candida- cottage cheese discharge. with or without·gram stain of pseudohyphae and yeasts ... squamous .cell carcinoma of vulva- large ulcer on labia, • gross of cervical cancer, • micro 'ofsquamous dysplasia, • woman with hirsutism- .' increased, testostero~e.

• Ai· . • th' .

indicates ovarian origin, • increased DHEA.-s:ulfat.e. indicates adrenal cortex .. origin, • ovary \V1,:,

'blood in. cyst- endometriosis, • gross ofruptured tube (smallembryo) from ectopic ·pregnanc.y'~ • gross of follicular -cyst, •. gross or micro of serous cystadcnocarcinorna of ovary- look for 'psanunoma bodies" • micro of signet ring cells with. mucin, ·l,n ovaries+ Krukenberg tumor from stomach metastasis; • gross of abruptio placenta, • micro of choriocarcinoma- look for tnultinu'cleated cells representing syncytiotrophoblast and clearcelts representing cy,totropbOblast; • gros~ of fibrocystic changc,.patient with Pagers disease of nipple,- patient witb infl8ll1ll1&toJ;y carcinoma ofthe breast 'with peau du orange appearance

Note: 101s material 'is co'pyrigbted. All rigbts raervecL Edward Goljau. M.D~ 2001

USMLE", ,," eDdocriDe, pict'ores, Dot in RObb. l'Gs: • patient ",""th acrom e'~';-. , ' ,;~: ','

.' '. ' ".' ' , , , '. o--'Iy- somclImes ask ' bou

visual field defect, which . is bltem~ral hemianopsia, • patient with midline' c>, ~ I" ' 1

thyroglossal ~uct cyst. ~ think branchial cleft cystiC it i~ in. the anterolateral nec~ I"~CC~-.

. . f tlary thyroid can' .cer-- look for pS·'ft"III'~ma"'" bod· gro andJor

'mlcro 0 papa,. ",' ,,' '".', ,"_'" ,. :-... .. I.A.'A,I, .: ,,', ,1es, • panent with- r~" _" "'" ',,' _ '-

, . ith H"" h" t th . ' ) U&4ve s dlsease and

exophth almos • patient WI' "', 'as' imoto's ,'.yrQi,ditis-, look' fOIT PC' morbital puffine ' .. ,' . ~',

._,. . . . ~ . . ' ' . · ,- "'.' , " .llness .• pabent .. ' h

thumb' adducted into palm.- tetany, • rrucro, of adrenal cortexlmed.ul1a·-," _ .. ",.: gl .. :",'Ntt

.. ' , .. , ids" . ~ - ii, . . ZOna ,omcrulosa

synthesizes 'nuneralocortlcol " .• 'zona tascicutata synth'csizes cortisol • ",' :, ,'. ,_~', ,.' .~: : .

. '.. ' . -" ' ' dren . I ' ., 'd' 1'- ' - ,'~ , ". zona rebculans,

synthesizes sex hormones; .. ~ . . _a me· u la syntheslUS catecholamincs •• patient with C cr , _ ..

syn drome= Iook for purple stna '. brown celoredtumor in. adrenal med u ,11:a", ...... ph .. eocbr .. .ushing

. ~ ~' '.' .' __ ... _ ,. .'. ." ':-- ~olmocytoma ..

patient with buccal 'mucosa hyperp_ -Igmentabon- Addison's wnh increase in ACTH _ • ch ild "~th:''-'

. 11 h dr '1 d fi " cru W1

ambiguous genitalia+ 21 or " . y" oxyiase - .eficiency, • foot 'with dry gaJ1gTcene in a d,'~'_-~:·"-

. ith mi . - ...a.OC I;.lC

ccagulation necro~is. .. re~tna WI. ' .. ~croaneurysms in a diabetic- due. to osmotic damage of

pencytes, • well circumscribed tumor m adrenal cortex ..... could. 'be Cushing's Co mn's syndro .' .

• • '. - , • , t, - t, -.' _... ~ me or

non-functioning adenoma depending on the question . - - ' 1,' - -

VSl\fLE musculos~eletal. ~ictures not in Robbins: .. synovial fluid crystals- • yellow and parallel to slow ray IS MSU m gout. * blue and parallel is. calcium. pyrophosphate in pseudogout;« chunky crystal in calcium pyrophosphate, • patient with osteoarthritis of hands-- Heberden's nodes in DIP joint, • patient. with rheumatoid arthritis in the hands", • patient with inflamed. big' toe- acute gauL • patient with tophus with or without microscopic showing 'multinucleated giant. cells and polarizable material, • patient with ankylosing spondylitis, • patient with. Reiter's syndrome who has conjunctivitis with or without history of Achille's tendon 'periostitis, • gross of osteomyelitis with or without picture of sickle cells- .' Salmonella osteomyelitis, * 'if no Hx 'of sickle cell, Staphylococcus aureus is the answer, • young 'patient 'with inflamed knee with or without pustules on wrists or feet- disseminated gonococcernia, • 'patient with Lyme's disease and rash .of erythema. chronicum rnigrans, .' patient 'with blue sclera, of osteogenesis imperfecta- • defect in synthesis of

~ .

type .I collagen, • blue color due to loss: of' collagen in sclera and visualization of 'underlying

choroidal veins, • gross 'of bone with osteoporosis, • MRI. of the' knee- mow how to identify anterior and posterior cruciate ligaments, • gross of. osteochondroma- cartilage ova-lying 'bone protuberance, e· micro of muscle. atrophy, • patient 'With Duchenne's muscular dystrophy, • patient with, myotonic dystrop'hy- '. look for drooping mouth and frontal baldness, « trinucleotide repeat disorder, • patient with myasthenia gravis-, look for drooping eyelids, •. gross of Iipoma.> LE cellneutrophil with 'phagocytosed red staining altered DN~ •. serum, ANA with rim pattern- SLE

.. ith

'patient with, .anti-dsDNA and renal disease, • patient, with malar rash of S.LE~ • patient wt ... ,

sclerodactyly ofPSS or CRES,T syndrome, - patient with dry tongue ofSjogren's syndrome . USMLE dermatology pictures not in Robbins: .. weeping rash on skin- acute eczema, • c~l.d with rash on cheeks- 'atopic dermati tis , • eczematous rash. on hands Of' fee:[- contact dermantis {type IV hypersensitivity}, • erythematous diaper rash of Candida. • thickened, scaly skinichthyosis vulgaris with increase, in stratum corneum, '. KOH' showing' "spaghetti and' meatballs"Malassezia furfur in tinea versicolor, • bald spot- tinea capitis due to T. tonsurans, • picture of crateriform lesion with central umbilication of molluscum contagiosum, • picture of farmer's ann with large •. crateriform lesion- squamous cell carcinoma, • child with "slapped face" appearance-parvovirus, • picture of pityriasis rosea with herald patch and rash on the trunk that fol~ows ~e lines of Langer •• patient with varicella- vesicles at different stages of development, • panent ~lth shingles. • patient's face with impetigo, • patient with Cl esterase inhibitor deficiency. • patient with sporomchosis, • patient with keratoacanthoma- l-ooks: like a 'basal cell cancer or~ ~uamo_u~ cancer bUI comes up rapidly in 2-3 months,- patient with acanthosis nigricans+ assoc~atton, with stomach cancer, • patient with acral lentiginous malignant melanoma under the,nad- do not confuse with a subungual bematoma •• picture ofa black widow or brown recluse spider

....... 4,-_

• .' .. '.' 'd'-'- '"' .iI IS" ". A.iiI'IIIpyn·'~crht',ed. All rights rtserved_ Edward Go.(J"'a.·~ 'M.D,. '2002'-

Note: TIJ·g· maten.. . LV· 'b· .

USMLE CNS pietur~ lIot ill'cRtur.Obcbolllf;Sa: n·ere~a wi~th .. papil1edebalrna~ • gross orbtain \\lith cn1ar-.A

'" ... -~ 1···· '--, hydroce p. halus. '. pl' .m . _.... .·· .. w' .. ' 'm WI ' .. anencenl '- .. y'" • pictu po., o-':'f' ,.._,.,c..t' ".... ,$~

'vmtnces-· "UlV'"' '. . , -I"". II •. "~I'· I ~C' lai'

in neurofibromatosis, •. brain wi~.~ystiC ca~ty- ~ld atheToscler~tic stroke. tiq~faetiV::ecr~=ts

~ '. ss of a brain abscess. • Purkinje c~" n withan mtracytoplasInlc Negri bod", of .' .. b .. _'i'l . . .' ,.~ ..

gro . . ... . . tho AID' S' b + ith rnu' 'J' Ta tes • cyst of Toxoplasma in the brain in a patient WI' . '.. "' .. '-raln wu . multiple calcifi.ed, MIr:ts;._ . ." .._ .... ":. I

k 1" s: . • th 1 _. .1 Q. cy sticcrcos 1 S

gros s" of ath erosc.1erotlc stro .c- pa .. e. · .. ntarct W1.... . arge cystic sp i. ace .' gr: O'SS" . .f .. : "' .. ' ." ., :,.

• .'. . . '. ". " . '., 0 mtracer··b· "1

herna aroma .. ···· in a h·:~rt·enslve- blood clot in the basal ganglia area .• gr o'S"S'" of a b ~ .~. ' ... e .• ra

I " •. ". • .•. J Y_- " . . .... '. _ _ . . . ~'i '.. '. . ram COV'ered b '

blood' S· ·;!t1obara·: '. chn ioid bleed due to ruptured berry anc:I'ntSTn ..•. gr .. ross '. of b ira un .' WI: ith . d' '. *' y

...- .:u. . . - J~."'" , '.. . . ,···..1· cgCU'et"ab f

I···en.· tJ··c··u. 'I· ar n ulclei- Wilson's disease, • myelin stain of spinal cord with de nIl}' 'ell~na" ··.tt· ".'. . . f' 'don 0 , . ". .' ... , ". ".' on O'orsa1

colunms and lateral eorticospinal .tract- Btz de~eiency. demyelination of posterior colw:nns istabe~

dorsalis, • gross of hem~rrhage into the ~nullary bodies and around the ventricles- Wernicke-

Korsakoff syn ; idrome • nucro ofan acoustic ncW"O'ma-loo.k like zebra stripes. gr. "O'ss· .. o···f.'1 . . .. d'

. . .... ~. .. - ,,1'" . •. ..; .. ' ow gra. :e

aseocvrcma- look for as~ctry ~ the. brain. - retina with cotton wool exudates in a patient~th

AIDS- CMV rerinitis~ • patient with unilateralBell's palsy, =retina with optic atrophy •• inflamed ear drum in otitis media

Terms for lab test results: • .trllt positive (TP)- ~itive test result in a patient with the disease, • false negatfve (FN)- negative (normal) test result 10 a patient with the disease, • true Degative (fN)- negative (normal) ~est result In. a patient with~ut the disease, • false posittve (FP)- positive test result in a patient WIthout the dlseas:, -negative predictive value of a test result (PV)chance that a negative (normal) test result IS a. TN 'rather than a FN~ positive predictive value Q·f a test result (PVj-- chance that a positive test result, is a. TP rather than, a FP

.S·ens:itirtty of.8 'test:'

I~. "positivity io disease ,t_ formula 'for sensitivity: TP l TP + FN

2.. nse of a test. with 100 % seasitlvity- • best used to screen fof' ~is.ease., .' .excludes disease'

when the test result returns 'negative, • includes. people with disease 'when the test result returns positive, .' buzz words: excludes a..ndincludes

negative predictive value (PV-) of a test 'with 100% sellsitivity:-. PV must bc·lOOl% since there are no 'FN test results in a test with 100% sensitivity- this underscores whytests with

l.'OO~1o sensitivity exclude disease 'when the test result returns 'negative, • e.g.; serum antinuclear antibody test (ANA) has .100% sensitivity in patients with S'LE, therefore repeatedly negative serum .!\NAs excludes SLE

SLE N'o'SLE

Positive t.est (T'P) 100 (F'PJ 20

Negative test (FN) 0 (TN) 80

Sensitivity of.ANA:· TP 11P + FN = 100/100 + 0 == 100'o/Q PV-': 1N I TN' ,+ FN = .. 80 180 +·0'= 100%

positlve predictive value (PV) of a. test wtth 10'0%' sensitivity- _. test result is either a'TP or FP. therefore, the PY+ is never 100% owing to the chance that it may be a FP'l' • Reop!e Ylith the disea;se are alwaY§ inc1u,ded • e.g., a PQsitive serum ANA_re~uh include~ ~U peoplt ~th SLE..t however, it does. not confirm .SLE since other diseases also have a cPOS10,ye serum ,ANA (e~g .. , rheumatoid arthritis, progressive systemic sclerosis)

SLE NoSLE

Positive test (T.P) '100 '(FP) 2.0

Nega~ve test (FN) 0 (TN) 8:0

py+'.;::: 'TP ITP + FP: '= 100/100 + 2'0·= 83% :Specificity of a test:

1.. H negativity is healtht1,-· formula for specificity is TN I rn + .FP· .

2. use of a test with 100°.4 specificity- confmns disease since there are no FP test results

3.

4.

..

py+ ia III test with 100% specificity-. pv+ is always 100%. since the FP ..• . ... ,'. . . ..

un· ders sco res whv test: with 100'0/0. spe .' 'cificity:··r are used to e o n'fi'::'~1 d.+.·-' _ rate ts zero- this.

. I:"wo. .J,. .. . .' ~ - ,U .. ,q·,U.l 'Iseasc • e - ..

S.L-E· has lO:O°X, ·.Sp:ccificity (no' FPs). and 30% sensirivirv-, a"··ll''('Ia-,t'I~··.e'n'.t''''·'t;· .. ·g~~ ~~tt .. Sm for ha . ,", S· "" !~'E . OJ ·c' .,::. .wWl!J a PQS1h\,eaatl-'sm ve ~,~,_

PI!. •• ,-.. • •

SLE No,SLE

Posttlve a.nti-S-:n. (TP) 30 (FP) 0

Negarive ~.:D~i~,Sm. (FN) 7.0 (TN) 100

Spec i:-fici ty: 'TN r TN +.: 'FP ==' 10·0 (1 0.0:+ 0 = 1. 000/0 ' .

. Sensitivity·,: TP I 11' ,+ FN ~ .3.0 I 3.0· + 7"O"=; 300/0

p~:- ::::'n t tp + F.P· ~ 3'Q 130'+:0 =·100%.

PV- in a (est with 1.0'00'/0 s.p,e.cific;·j.ty·_ • PV' may be a TN'->" or' FN:_, * it dl~S'. n t ... 1 d

" < < < " • • •.•. , <.', ' '. '~' o· exc: u' e

disease, • e.g.; anti-Sm is. ~egati~e i~ a patient- '" does not e~eludc SlE, • useQther~sts .~~

confirm SL.E if your suspicrons.are hlgh- e.g., anti-d·o·ubJc:strand,ed DNA .

SLE No·~·LE

Positive auti-Sm (TP} 30 (FP) 0

··Negative· auti-Sm .<Fl'f) 70 (TN) 100

.PV=·TN'I TN +'FN = 100'/ roo + 70':·=: 59%

r' Calculate the reference interval of tb.e test when given the mean of the test and 1 SD (s tanda rd. devia tion):

I ~ remember 'to' double the SD- '2 SD· covers 9·5:% of the: normal' population

2~. ·exa.M.pie-. 'mean of the test .:::: 10Q" ·.m.gidL 'and .1 SD·. =:. ':5 mg/dl, (2 SO == 10 .mgldL>., e· reference .interval ='90-110 rng/dl, . .(lOO - 10 =; 9'0' and 100 +. 10 ==:. llO)

3. for each test, 5'0/0 ofnorma! people will have t'est results 'outside the ·[.efetence·wterva.I-. c:ha.t),e·e of a~ FP irtcre'ases wherr·m.ore th,an one test is 'ord·ered o·n a pa.tie·J.lt:, .' example, :2 tests on a patient increases the chance ofa FP test result on one of those tests to ~10%, .1 formula to calculate percent chance ihata 'test result will 'be outside the .nQ.~l·ran,ge-· '$! 1. - 0.9.5"" •. ·e+:g~,. chance for 1 of2 tests beingoutsidereference interval is 1 - 0.'9.52 =: 1 .. 0 .. O~90:: 10·%, ~ chance fat 1 of6 tests being outside reference interval is .1 - '0.:5):5.6 .. = r.o- O,.74.~,= 26%·

,4·. SD is • marker 0',£ the precision (rep .. roducibiliry) -of t.he test- i.t. is not .. a marker of how accurate tho' test result is.

.3:~

Aecnracy; poor P.recisio.[i,·: .g.ood

'~Q- E·ltec·J;:of test sensitivity/specificity ·of .•. testen prevalence: ._ testwith the' big.hest· sensitivity (not specificity) increases' preva lence -of disease (nwn~ber of people in. .'8. population that have disease), • it. picks up' more people with the-disease since 'it .is a good screening test

~ t I . " ,".It, It .

. t7. :Effect of :i,acreasiDg the opperlliD.it of .Dorm·al of a test. refereace inte'nai (~g., r~lag a,

reference Interval of 0-4 IlgIJnL to a-tong/mI.) en sensitivity, spe~i.ficity, rv', add rv: ..

1. increases speciticlty and positive ptedidive :v1IIue- • higher 'values arc mote likely to

represent TPs than FPs~ •. always increases, which ~utomaricaUy increasesPv"

2. decreases seD$itivity anduegative predictive value (pV)- • increasing specifiCi~ 0d··~ a test always decreases its sensitivity and .. PV\ .' .fN rate. ·increases,. since more people _~"'.~" lS~.S·e.· are encountered as the reference interval increases, • a normal test result is 'more .likely to be a

FN rather than a, TN' .

17' Effect ordecreas~g the upper limit of normal of .. test referenc;e l .. tenal :(~g.,loweriag [he

fasting glucose level 'for diaposing diabetes memtus IDM} frOID >140 IJlgldL to >126 PIg/tIL) 08 sCDSltivi:ty,. speclficity, PV+"and PV:

iaeresses $eD~itivity llnduegatlve p1"etflctive v .... ue (PV) - 41 dro,' , •. .... _ '.

~. terval to a 10' "w-er' valu e that - ppmg the ~'I ... f'

the reler~nce.· In ·.~l ~... .•. .. . . . .'. . '-: '-!f;I. -. -' - means ~. '.' t more nro,p~, 1::-· lidth':' - . '.. . ~. ~ i, _ . '-r'~ .11lld:·O'

r- '"" '0"1 ... a ne'gattv - test

likely to ": J!'Is (not have OM) ~ther t~ FNs. • sensitivity and p~ al. I .. ~,Tesult ate

the tipper lirnit.of 3 test reference: Interval IS lowered ways mcr:case when

dec.rea:ses speci'fi.cily_ and po.sitivepredic~.ive valu'e' (Pvj-, .. fewer peo.I, .. ", .',., .-, . _. .

- , - l' 26' - gldL . '1- ·'k I' -.- . . . ~p c, are tllce1y to ha .•. .NIo

OM", a test result .> .' .. ·m_·.· .' ". IS ·more· ·,.1' e .y' to be a FP than a TP . _'. ,_ .. .: ... _ -. ", -. ~,.'!IW

" . _ ~. -- test result- • s.uml'n".ll~

S-C herna tl c: ..." . -,. ....... IIII&I. . .J

Norma]

o ·4 10

Interval t..:.. 4

. .-

Sensitivity 100% (no FNs)

:PV- 100%

Specificity decreases

"p.y+ decreases

Interval Q' - 10

Specificity l~OO% (no FPs) pv. 100%

Sensi tivity decreases ,PV decreases

C7 Prevalence:

1.. .lrevalence (number ·of people 'with . dis ease' in. the p,.opu·latioD studied) =. !ncldeace

-, .'

(number 0.£ new cases over a period of time) 'X .!!ur.:a.tio·q. ofthe diseas·e- • P = I X ,D'1 .i as

duration: (D) decreases, prevalence (P) decreases, .•. as D mcreases; P increases ... • _in~i.dence (nl .. sa .constant in this rc;lationship

.2.. prevalence calclll.atiO·O- TP'T m (all people-with diseasej/ TP +. FN + TN .+ FP (a1l people with .. and Ylithout. disease)

3~ e,u:mple-- if treatment lor' leukemia lengthens the survival period but does not lead' to :11$ cure, prevalence (P) of'leukemia increases OWing' to-the increase in, duration (0)-. no effect O'n incidence (number 'of new C3S'CS. ·of·lcukemia)

.a- Example o·t a ealculatiou fo-r $eDsidvi~,.specifici*y, rv', PV,.·pr,e·va!ence:

Disease 'No: ,Dis'ease

P,ositlve ·tes·t (TPl 60 (FP) 40

Nega.tive !:.est (FNj :Z.O ~,80

Sensitivity ofthetest; 'lP I TP: +, fN = 60 /80.= ?,-SQ/.,

Specificity of the test: TN I'm +. fP ;::: 8·0 lI20 ;:; '66%

PV-': 'TN I'~ ,+ 'FN' '::: 8.0 l 100 ::::.·80% (8.0%. chance itis a. IN' and a .20"~ ehance it is a FN) .PV: lP .I-·TP .+.'pp =·601 too .= 6'0°/6 (60% chance. ,j't is' a U' 'and 4Q01O"'C~CC j:t is • FP) Prevalence:"TP + FN J TP' +. FN' + TN + FP = ,g'O .12·00 =~40'%

cr NorQ1al t:hanges in pregnancy: ..1 ..

1. gruter Increase in plasma volllD1e thaa. RBCma5$- • decreaseS hemoglobin (Hb) and t

hematocril(Hct)- dilutional effect, .. increascs.glomenU3.f filttadon l'8.te(GFJt) and ~rcalin~

clearance (C'Cr;}- due to iaereased plasma volume" • decreases serum BUN/creamtinelunc .t-., ...

aci·d~di.ltlti:onai. effe·ct· + increased renal· clearance

6

iqc:reased alkaline pbos'_'b-atasew- placental_ origin

respirato.ry a.lb.IOSir·S'-c- O·estro .. 8enfb··· . ··progesierone effect on. CNS rC5piratory' f"iJI!HIIII

in··c-reased.'-c,l~c·e o.· -; · .. ·;_~. per breath .. ..... ""-.,' ~nter Ca.~ing

iacr~ased T~.lld cortisQl- • increased synthesis of their binding proteins (tbyr" "'.~. bi globulin, ~d·. imlSCOhrtiD. re~nl~vely) •• free hontK)ne levels are notl1lal .•... n:I:.Itt<ti~gf~·

.hyperthyrot,l~..yper~OrnS(HSm-e.g .•. norm.alserum TSl{ and ACffin' " .wgns 0

Mai~ Iaboratory dirference _u 8:da~.( male and (em.ale::·., lr.o-D.sfudies .... '_. • rlespccU~t;IY

, . - .. . - - .. are al lower In w

·e·_.g~,_ ·s-erum ir~n and ferritin..» lower Bg'b £oD-ce·o·tra«o._A_ ia women' - - -._ - ..... - -. ·:'9~e:D--

C'hildren-':

I. i~l_c'r~as'ed $~eru·~halkalitt_'.ue PI'~'OSd:'Pb'ha~e (~p)-. 3-5 times higher-than adults .. o ... eob li-as. _(g_

release enzyme.w -en, s·-1;Itu ate Y vitamin 0' - ~ ., ~-~ -':1"-.,

2~ increased serum ;pho-s·p.hate- required to drive 'calcium into bone,

3. slight~ecrW'e inll~lUog'obiD eOD~eD.traijon ~hell cl)Ulpand t~ ad,ult levels

Ne-wb·o-rn: high hemo glob' m (Hgb) due to.increase in .H,g;o:F-

1.. _I·eft- shi(ts ~-xygen dissoctadon curve (O·D·q-- causes tissue hypoxia-»

.2. snmulas fer ··~rythn)PQieti_n; (E:PO) 'release ~

3'.. Increases .. RBC produetion '~tl;t,:sllb:sequ,eDf-incr~eiil Hg." COD-cen.tr:ation

'1Ig' . -. 'b' F·

"'~' . > . .•

t, I~ft ·shifts .Qxygen disscciaricncurve (O:Dq.

2·.. protects newborns 'with sickle cell disease- • most of the, RBCs at birth, contain HbFjnhib!tssi~kling. • less HgbS- " concentration not high enough.for sickling •• HgbSJDUst be >60,% .in 'RBe for spontaneous sickling, • dactylitis (bone infarctions' of digits) begins In ~9 mths

3.. BgbF synthests ls increased wi~' hydroxyurea- used t~l reduce sickle cell-crises

Analytes increased with hemolyzed blood sample secondary 'to vealpuncture:

'I.. ·~pH- • LOH', isoenzyme fraction is 'primarily increased and is: ~ eater than LDHz isoenzyme' fraction (LO.H:'J_fLDH2 flip), • false positive diagnosis ofacute myocardial infarction- LD'H·., isoenzyme is '3]5-0- in-cardiac muscle

2.. pctassium=e pseudohyp~rkalemia- false increase •• ·K .... · is the majorintracellular cation, .ECG VliII not sh-ow a peaked T wav-e-

Lipids and fasting';'

1. trigJyce·rid~ (TO) component coming fro-m :cbylomi,cnins fals~ly increases' sentm T'G

leveb-- chylomierons contain diet-derived TG,. hence theparient must. be: fasting.to eliminate'

...

this variable

2~ fastlDg or Iaek of Iasting does not affect .cholesterol (CH) and high-den:sity lipop~tein

(IJl)1.) c(ulceDtr2.tioD- ~ normally, CH is <3% of the chylomicron fraction. • fasting is

unnecessary for an .accurate eE{ or W;)L ._

J. fasting. is necessary for anaccurate calculated. lo",,-d'ensity ijpoproteiD~~r. '!II u>l= CH - HOt - to/Sf' if TG is falsely increased by'Chylomierons from rhe diet, It will falsely

lower the calculated LDL

Drugs enhan.cing thecYt~chrome system in the liver smooth endoplasmic reticuilim (SER):

1. dru,gs- alcohQl. barbiturates, rifampin, phenytoin ... ' . . . -.. .... GGT)'

2. ettec:t Oil SER-e .SER hyperplasia, • increased synthesis of y-~lutam~ltransf~rase (.' :.'-

, . 1'1 '1'-- d" '"5· 1:-1:) d dru '. levels owm g- ~O' [ncreased metabolIsm

enzyme IS normat y . O·C3te·.~:. tn' ~ ~- ecreases .. ' 'g. ,'Wi"'- .. : .. , . '.':- ~-.' '.,."~ -.' .

of the drug _ .... ~ .' _ .. _

3. diuical sc'e,nado- Woman on phenytoin w.ho.is a~ takin~ ~inh cpntro] !,dls gets pregn~u-

phenytotn revved lip the. SER and increased I1ld3bobsm of birth control pili

Drugs 'u.h'ibitilig tyto-cbrome system in. the liver;

1. drug$-' Hi blockers, proton blockers

" ' ..

2.

.3 .•

·4.:

'r;r

Qp-

.,

2:,. daager or driJ,g ,toxl:clty

·SAga_ticaa-ce· of .erythr,cyt'e sed-imeat3_~oD f"ate- .. (ESa) I,D 'old, a~e: pr ~--O' b", . b-,",l" ~ ,', . i ~ ,_. -

. . _, b :,' a:.y l.~d'lc:ates·'·' .'. d"oil _ '. -, _

process .. " al: lsease'

LabOr8to,1y cest. 'a'lter*,tioD:~~ ill .1eo·haH,c's':

1. enhancement. of th~ liver cyjochrome P-4so _ systelD- • increased. ,.-'.c .'

') ltran ferase '(~~T)' '. excellen tte '. , k C". ' synthesIS of '\I'

g'u~y, .. _.' 'Sl:~.," ' .. :,"._ '~:'_.,~':_, '_" ~:~':.. '. _c-..e~e,~ ter lor alcoholi.c liver ill , .... :.;'.',. -.': I-

.I'liMo., ... : 'd 'pr'o·du··l'tti· 00 or NADH OlD Its m~ eta bO"I4! . b k d sease

IDctease .. ~: ',';' .' ~. '.,: ... , .... , ',' , .. ;'.-'. '_ .. ' Icrea,lown Cc.a-uses bioc,hemical·-" -4.1'

'lu¥oivta.g ·NAJ)'B,·to,mo'v~ In Its dire-c'lioD r'estlltJ,b,ti' I'n the -, ·"'o-'Uo .. -Wl,W'·-1 .:····-'.'-t, ",:, _' re,~~.UoDS

. · .6. . .~ . " Dg~ ··lactlc actdo -, - .

pyruvate-e- laetate.: .. fasting hypoglycemia- pyruvate is unavailablefi '1 .' ': . 1;5lS-

h . "' ... ., '1 -.,'.- ide - .. '- "_, '1 3;' b"~':' ohc ,h', -' ,11"" -" ;',.' ~ i.. ... " .. ' '.,' o,r g' u~neog~esis. •

ypertng .~. emra .. ,. nsp .. osp .oglyc;erate-+ dihydroxyacetone, phO$phare.-4 .• ], ..............•. ,_

phosphate-e- TG g ycerol 3

Iaerease in ketoaeld synthesb-acetyl GoA. the end product of-alcohol metabor err ' , .. ' ...•... in the following s:eactio~~'acctyICoA + acetyl eeA -+ acetoacetyl CoA-+ ~t: ~used

acetoacetic acid- m~rease.l11 N:rnH c()nv~·!t into f3 hydtQxybutyric acid <a-OHB)OA4'

increase ill: fatty .CJ~ synthesis- due to the mcrease in .,aCetyl-CoA... the initial substtatc for

the .synthesis of..FAs ,

S. lIyp~rnrice~a~lactic acidlk~to3c~dS'c~mpetcwjth. uric acid for excretion in the kidneys

6;>· increased :a~.lo~ gap-metabolic 'a,cldoS1S~ lactate + r3.~HB

Laboratery test alterations In smokers:

l..,r¢s·piratory· 'acidosiS- .air gets 'in butcannot.get .out, so COl is. 'retained

·2,. IIypf)xemia (low ,PaO;z).

3. Increased carbou monoxide (CO) le.vel.s- CO 'is-present in cigarette smoke

4.~ secondary polycyth~'mia~ 10.\\1 P,~O'2 stimulates.erythropoietin release

5_ absolute' :Delitrop.h~c .leu·kocyto~$i.s- .metabolites in smoke mobilize the neu,trap'hil

mat.ptin,g pool in the circulation by decreasing' leukocyte adhesion to endothelial cells Plasmafseram. tn.rb·jdity:

1.: .. , doe ·to an Increase "in -, triglyce.ride (TG)... turbidity does not occur With an increase: in cholesterol (eH) in. plasma

'TIG Is carried by l.i',po,prOteiO$- •. chylomicrons- 85% •• very low-density lipoprotein

.,

(VLDL)- 5,5·%

TG Is fals:ely' Increased after ,eatin;g- due to, diet-derived chylomicroas

chylomlcrnns form a. supranate in plasma- contain very little protein: less dense than, VLDL

- .... '

\ILD,L forms an Infraaate (no supranarej- contains more protein' than chylomicrons 'and

does not float 'QD' the surface of-plasma .

6., Increased turbidity Interferes with measurement ef'enzymes and serum Na'" - falsely low

enzyme values .. and sodium (pseudohyponatremia)

.Sllpra~te· ,lafraa3.te (VLDL)

·(c:hylo:mic:ro·DS)

....... .... -

2 ..

:3.

....... ~,

- ...

4· ..

5 •.

RelatiOQ of ' serum. ' UtD.,iU·CODccDtrad.OD with ,serum ealciu.1d ~Q,CeDtration: ,

l.albllmiu binds 400/0 of totalealcium iD blood,... • 13% of calcium is bound to other

. . . . .' - - - . -'" boJ~ca1' 1'· " 'b·'V calciUm

,substrates·i::.470/f, calcium is' flee, ionized' calcium-: meta ': ..... I' ::Y ac-:, ,e ._ .... ' .~. ~~.' .. ' " ~

,2. I,ow' se.um album.in decreases ·dI.I.£iu.m baua.d. to ·albu·miu:-·" bypocaj,cm;ua, ., no tetan,x I·S

present, since the ionized levels ate ,normal,'. FI4H, i$':nonnal

,r7

Lyon's hypo'th,es'is':

'I.. O'Be of' t'he 2 X chromosomes "in a, 'fem.l .,", ~ --"- -. __ ,_ ~, ~

, _ ' _ " _,_"',, ~":,', _ ,'_' e .IS randomly .Baetiv,- :-'1 ,__, _', ,

chromosomes arc: maternal, ." -:-50% X chromosomes paternal. _ i ......•.... ated._. -50% X

becomes a Ban' body- projecnon from the nucleus counted in.~cb:ated X_ Chromosome

~ fro- the b ucca I muc-osa . squamous ce'Is' obta-' d

scrap'lngs ,'--m.I-' - '. rc :' _ '-- -' _.;_:':.' . '.' "-.~' . "- -:ltne··, by

2. n.,rmalfemales have 1 Barr bod!: and Dormal males do Dot hav .... B. .. • ..

3. male.with Klinefelter syndrome OOCV.genotype) has 1 Barr ::~, .....• arr bodies

4. fem:ldewitb Turner's syndrome (XO) has no Barr bodies Y

Mol.eculs.r genetics.

1. cystic fibrosis- • deletion of 3 nucleotides coding for phenylalanm '. hr . . trans~embrane regula~or is defective and is degraded before· rea:~~c th~m()~me7 •• CF after it leaves the Golgi apparatus g cell membrane

2. :ray Sac_h~- - 4 base insertion produces a frameshift mutation, • codes ii.. ...

hexosaminidase or defecttve

3~, sickle ceU ,d.is·easeltr·sit- point mutation involving, thymidine, replaced 'by- ad · . ~" '00'. ',' _ -',, __ ,_

· ., ,-, - " f- l' - .. - <II -:- '" 6-" ., ~.- - - ' ' " enme c' es for

valine instead 0" gutann'~ acid 1Il, ',·th position of J):-globin, chain .

4. IJ-tbal~s~mia major~ poin~ mutation produces a stop codoq leading to temtinatiou of DNA transcription of :J3 ... :g10 bin cham

5. trinucleotide repeat disorders- • progressively worse disease occurs in future generations (phenomenon is called anticipation), • constant repetition of 3 nucleotide bases (e.g., CAG •. CAG, CAG et~.), • more trinucleotide repeats are added each generation leading to p~gresslve~y worse disease. and .. earlier manifestation of the disease, • examplesHuntington's disease (AD), fragile ,X syndrome. '(SXR female carriers with trinucleotide repeats eventually become symptomatic), Friedreich's ataxia (AD), myotonic dystrophy (lill)

Nondisjunction: .• cause of the majority 0'[ chromosome 'number disorders (e.g .. ., trisomy 21 ~ Turner's .XO), • due to unequal separation o,f chromos-omes in -tirstph,ase of_mejosis,

Mosaicism: .• nondisjunction ofchromosomes in mitotic division in the early embryonic period, I. two chromosomally' different cell lines are derived from a single fertilized e.gg', '. most cases- involve sex chromosomes- e .. g., gonadal dysgenesis with xorxx, 'xorxy

Translecation; .' one part of' a chromosome is transferred to' a non-homologous or homologous chromosome, .• · called a balanced translocation if the translocated fragment is functional, .. Roberts,oniaD 'translocation In Down syndrome: .' type of balanced translocation with a reciprocal translocation between 2 3ctOcentric chromos6m~s (-L'l £), • usually chromosomes 21 and. 14, • produces 1 long chromosome (14-21): extremely small translocated chromosome usually lost, • mother has the translocation: 45 chromosomes with 1 long #14.21, 1 normal #14, and 1 normal #21, • mother is normal, since both translocated fragments are functional, • Down child receives 1 norma] #21 from uninvolved parent (father) and 1 14-21 and 1 normal #21 from affected mother for a total of 46 chromosomes, but all 3 #21 s are functioning

21
4 14 21 21.
, I I I
14 14 [~

,.... . .,...

,rr

, 11

,·s deleti<m lit ~fmttQ_m..UttiliDi·"llAp~y p~ppY'~ '5~~m~ (ohild alway. ha. .." ... "."" '. '.

c.nnOI t.lk)1 - term apphed t~1 tl~e!le s~dromel III lmo~ lmRrintiOIl PPYl1lUghtnl but

rr.1.·k·- o-"Ir-- recu r"r'l!'ftC'e o·r. A'~"etlc disorders: Mendelian d'<I~II·o~··r·d·· :r:r'-s" L......... th

ru· . • . .,: . . ". :". '. .. . I UlVe t·

~ecu.iTC~\Ce! AR.·(llsC.asca ha.'!e D 25·% recurrence rate .. ' .'. . '.' .. ' .;. ~ ~e~t~'lt ri'lk of

Gen"Uc, disorders-In Arri(.~-AlnerlcAn5: ·5kkl~;cel1 tnlitldiRcase- lr-l0%~r' _. .... . . ," cell ~ne.. ?,../p.~tllal41,S5~ll1lfl. • glucose 6-phospha'e dchYdrogena~~ (o:prialen~e ~{~lekle hereditary persi stenee of HgbF ) deficiency. •

(-;e:··1n: 'e{il"c disorders in A'II,kcnall .Je~s; • Iactor .X.D deficien' 'C,·.y-'· •. ··o':·,::tlu,p.h·· ... ·t- d-,Ij _.

. - . . '.' , .. • . Q r\.oI.' ...,:r· SIS - . , . T'

d.ISCd5C .' .' .' '. '. \ -," ;'. ~Cl.ge~, •. · [j·ay, .. Sachs,

Geuelle d's~rdel' In North.,rn F:uropea.ns: cystic fibrosis- Me gcmtic discas '<ft., " , .", I1llicnfs IbiljlYJ9..oan:.Q.Q_U.~ owing to carly death or problems with fertility _t Intc.,~mJ.&\!\t the

Genetic dlsotders ill Mcdltnrllonesan peeples:- G6PD d~t1ciency, .. sickle .cell ttaitldisea. c .'

tJ;\DI':ls~emiB. . , , .. '. . sc..·.. p.-!o

.Geae·tl,c disord.er :In. Sou:tb·east .A·sia,D5: a-tiut'las-scmia

Me Kenede syndrome .sS()-cha.~cd with ·.adVSliD<:ro materna) age: trisomy 21

1>0",. sylidr-ome pachogelu;!$hi:. trisomy 2.1- ... 9.s.Gi.9 of aU case~. • 47 chromosomes • maternal origin for extra ehromesome, Robertsonian 'transloc.atio·n-· • '/4o/b ... 4~6 chr rom oso rn 'e' s , .. - . h:"ld': .1.

.. .'- .. . , . " . ,.... -' .,.' .. :.' '. '.; .-.', ," l.n ell ...

m~lh~r, w1~ 45 chromosomes- usually- mother, • Me genetic cause of men:talr.etardation-·IQ 25 --5.0· in :80.~ 'J

DoWb'J'cUulcal ,~diDgs~ • .cpicanthal folds with up;'aidslanting. - simian palmarcrease, • poor rdlexes/hypotommty,. cardlOvascular- ·endoc:ardmlcushion d,cfec~ (combined ASOand VSD)

mai2[ g~1am.ming factor 12uurvivaJ ill- early infanc},and childhood. • 01- .. duodenal atresi~ (polyhydramnios, "Vomits bile at birth. double bubble- sign on x-ray) .... l-lirschsprung's disease, ,~ hematologic- increased incidence of leukemia, - cNS- .. Al7hdmer's disease, .. chromosome 21 codes 'for p:-~ylo'ld, proteins, which .. arc converted .into amyloid and are toxic to neurons, • universalby age 35 (any patient with Alzheimer's disease under 40 is a patient with DOwn

syndrome), .. reproductive- • ~·I.l n'n]~'~ 'ate'" ~t~ri_lc" : •.. f¢m~lc.s t13ve. 5.()%1 ch~1)ce, '·0:( having a. c'h"ild roth 12o.\'ffi·'S

Risk 'ror futu r.e childreu ·witla. D~O'Vll'IS': .' t -2?/o overall 'risk, for tri.SQtnY ,21, .• 1 maternal agc--

women '~3~·5. yrs of age, • 5---·t5~~ risk for parent with a balanced translocation, • karyotype: of affected child 'should always be determined to evaluate risk for siblings' ·to, have' affected children, .

Ttisqm.y' 'f8 (Edward's syndrome): • severe mental retardation, .•. "clcnc·hcd han'ds wi,th overlaPDing 2nd ,8l]d~5th fingers.,. • ·roc·kcr~.otto·m fce~

Trisomy 13 (patau's syndrome): • "Cleft lip/palate, • severe mental retardation. • PQIY£tacty~, • cn~~ _kidnc:is,

Men,tle-liaa ,,'isQrdersin' desceading order 'of frequ,c·ocY:.1 'autosomal dominant (AD)., •

autosomal recessive (AA) ... sex-linkedrecessive fS.XR)., 4<sc'x,-linke·d dominant (S·,XD) .

AI) disorders: • only 1 abnormal allele is necessary to express the disease- • "dormnant gen~'\ 'oJ e.g., aa (disease, usually lethal) or An (disease),.:. only one parent naji to t.mvc the. ,ell!: t.Q.Y8SS It on 19 Iheircbildren, • 'one affected parent + normal parent- 50% normal children. 50% a ffec ted ch tldre ,

"_I~:cn . .

'~ disorders: • bothabnormal alleles m~st be'~resent (homozygous)t~expre~sthedisease-·I:~ (drsease), .Aa (heterozygote asymptomaticearner), -- both parents must.have ~eab~0nJ18la~le- ., • 2 asymptomatic carriers (~hildictl- 25% normal AA. 50% Aa, 2$% aa), • panent with the disease

has children with an Asymptomatic carrier (children 50% Aa, $,OO(oaa) ". I.' .. '. '.' .H<'h> sxa disorders: • males with abnormal allele express disease-: males arc ·h~mO~gQU$.,smce t'Je~

only have I X chromosome. • affected males transmit the disease to both daug~t~f$ . but, none 0

th ., . . -. t"_. 1 . carrier transnuts the disease to!

err sons~ daughters are usually asymptomatic earners, • I:cma'e C - I'; .' ':_1' ') ;.:' '- r : --

50010 of the boys and 25% of daughters are asymptomatic carriers, • female earlier + affected maleS()O/o of males are affected, 25%. of females are asymptomatic carriers, 25% of females are s\:mprom3.tic- maternal and paternalX chromosome are abnormal .. '

'C7' ·SXD disorders: males. and heterozygous females both express the' disease

~ AD disorders: .. associated with structural defects in proteins and receptors- • enzyme deficiencies are uncommon, .. AD .. di~otders that .. are . e~e d~ficiencies- acute intermittent porphyria (deficiency of' uroporphyrinogen synthase), .. hereditary angioedema. (Cl esterase inhibitor

deficiency), • late .manifestations of disease.- e.g .. , Huntington's disease with c'ho,reatdenlClltia later in life, • exhibit ISletr.mce- • person has the abnormal gene but never expresses the disease. however, the gene is transmitted to their children. .. some AD disorders have 100% penetrance (e.g., familial polyposis, adult polycystic kidney disease). while others have incomplete penetrance

(e.g., Marfan's), • exhibit ,'ariable expressivity- affected people have different levels of severity of the djscasey ..• mechanisms of AD. disease without a family Hx: ~ Mec 'is mcolxIQ!ete 'pen~trdnce;; • new mutation, • examples of AD, diseases (in order ofdecreasing frequency): '. 'von Willebrand's disease • familial hypercholesterolemia, adult polycystic kidney disease, hypertrophic cardiomyopathy, .. Huntington's disease, .• netrre fibromaros is" • congenital spherocytosis, *" familial polyposis, • acute intermittent' porphyria, • osteogenesis imperfecta, • Marian syndrome

~ Example of a pedigree with complete penetrance:

r7" Example ofa pedigree with Incomplete penetrance:

: .... i. I' . ~. . (. a, I'

.. _ ~ b 1 . ... late manifestat1ODS, • most . DO"

·,AR. diseases: • no evidence of- penetrance, vanae expressivity, ' . "._ , .. ..... ~. , .. ',..: ....

aU) AR diseases are ~ deficiencies- inborn cmn of metabol~ .. acute mtCIuu1lCllt

... ' ··:·h· .... ,:~'" . ,'. " .. d C,-l esterase inhibitor deficiency. are both autosomal dominant, • G6PD and ~hporp.yna an . . ... ... fibr . •

N' ',~ft. ~r.II!I both·· sxs • exanples of those that are notmzyme defiClens::1CS- .. cysnc .: .. ~ ..

"I.~ 811'.... . ~. '_'. r :., "_'.~ - . -_ . . .

. . -' ilson' di- ...

. . .' '_ . ., . , .. , .• ' ' .' sease

sickle cell Iral tid I sea se. • hemochromatosis, _. WI ... ' .'.~'. s " ,

r (

Note: Tbism2CeriaJ is <:opyrlghted. All rights reserved, Ed",ard Golj:l,D, M.Jl .. 2002

1

SXR. _." diS.o~r·d.ers:: examples (in order of decreasing i;._"uen·c-y)· • '~aile' X svndr _ 06- .

, . . . ~.' - ." -' .. ~:~ -.' ..•. ,U4b~'·.,' ·'·J'I.ir.'ome ., -·,PD

.deficiency, ,. Duchenne 's muscular dys trap hy - .• ' hemop : hilia. AlB' .'. - '. .' sev " .. ' .. ' 1-.. . - ibi . ied

• , y '!! . -. - - '. ",' "'" ere com' 1.0 .

i.mm~ade~ciency, '" ·W!skort- .hlrl?~h syndrome. -. testicutar feminizarion, '" coI~t blindne~' .•

chronic granulomatousdisease of childh~ =Bnrton's agammaglobu1inelJ'lia •

~

F·ragile X'syndreme: ... some geneticists say itis a sex-linked dominant disease, owing to the fact

that· female carriers may express' the disease in future generations {con.c·ept of anticipation)". '. second M(;, genetic cause of-mental retardation, ., M,e Mendelian disor.der associated with mental

. . . -. . ' . ." .

. retardation, '. clinical- .: mental retardation, * macroorchidisffi-' at puberty, • ....w~30% of' female

carriers are mentally retarded or have 'impaired Iearning= (jue_ to .. anricipa·tion .and ~additi_QJl 0.(

. . .

. tlin~cJeotide rg:teats y\.vith futme 'generati'9n's'J ~. abnormal fragileX chromosome- DNA: analysis for

'carrier identification (identify C'GO trinucleotide 'repeal is the.best test, to c9~firm)

Lesch Nyhan syndrome: .' :S)(R. disease with ,3 deficiency of 'HGPRT'~ no jnhibiticn of P,RPP in purine metabolism, • clinica.l--, .' .hyperuricemia, .• mental retardation, .... self'murilation

SXD:' disorders: • percentages of children With the: abnormal allele are the same as thosein .. SXR. disorders; '., dominant abnormal allele G3JlSes disease In, both males and females, • af~e,ct(;d woman transmits symptomatic disease ~tb 5,00/0 .of 'her daughters and. 500/0' '0.£ her sons •.• 'affected 'males transmit. symptomatic diseaseto all of their daughters and none of their sons, • examples: .. familial hypophosphaternia (defect in the proximal reabsorption cfphosphate.and conversion o{25(Qfl)D1'

to "1<~25' (OHh..:O))~ ... Alpo-rf.· ' (hereditary .. glomerulonephritis associated with nerve deafness)

M· J '"'I': ial (.p ..... Q .. ·ly .. 2.e· n .. ·.l .. C·).,·.·.· 1.",,0&_ eritanee; .• rnultip Ie small .mutations plusille effect. o .... ~.f.·

, .' _',: ,~I:aq'o;rl _' . __

environment- • should be .suspected when there is an increased prev:a]~cCi of d~sea$Camong

~latives ofaffecred individuals. • parents and .offspring have 50% of the..- genes m common, •

~,

'".-

.,..._ .....

.-.~ -~- --"'I' ....... _ ............... ' ... & ..... ~--"" .. _- ... 1;r,"'&~i(·t:S ~-.riI Ed' ..... . ani' ".' '.

'II- .., g, 11-1" ... ~...." Go." 'M'

- '.. ' " h JaD, .' .D: .. 2002

!fxamp I es: • cle ft 1 ip Of' pa la te, II! Con genital heart d- -.' .... ."_ ..... . . .

II -'. . '. tsease .. ,coronary an' d-'

... DM,' essential hypertension,. open neural defects .' ~ •... ' ~ .JSease gout,. type

'M"", b d: . ') 'D' 'N" d'''' , ". congeru,tal'pylonc St· "._- . ," .

r . rtoct on .. na; , . A disorders. • mtDNA primaril .. c . . ' . enOSlS

OXidati~~hosphorylatio? reactions •• ova conta~ a.!:!:::::eSalnV01Vtd inmitochondria.l

syrrrptomatic abnormal allele to all their children • . . ff~cted. women transmit

fer1iliza[i~n- affe;ted ~les do, not transmit. abn'orm:i::i~l!OS: ~lr rm[och~dri~ durin'g examples ... Leber s heredltary optlc neuropathy .. m y ':. ,'1.' .... ". , .. ':;'1- .. Y?f theIr chIldren, •

'. '. . . .' .' '.' ,..oc ante epi epsy .. I . t' id wi .

Example 10(,:1 mtDNA pedi,gree.: .' '. ..... ".'" ,:,' - ..... ; , ·_aC·.lC act··.', W],th.strok.e

Calculation of the prevalence O~"':r a' 'ge" netic d ·IS· .a ...... se oive'D th"_· .... iii... , _

'. .. . .'. ". ~" I:I~ e carner 'rate' .' f1

(C'F), with a carrier .rate of C. F 'is 1125- • number of c~op·lesat risk is •..•......... ·1 :~·thg·· cYStl.clbros~s

....... ', ,. -". .,., +. . .' . .'- ,. . eq,ua:, to ·"C earner rate. m~

males .x; the carner rate m females, or 1/25 X 1125 =: 11625 couples ... ... ."k. . . . . . .' .... , ..

c~ild with CF (AR disease) is 1/4, hence 11625 x 114 = prevalence Of_~~~~s. • risk of haVing a

S~mplewayo{caJ~ulating the. car~ierrate of a~is~~ given the prevalence or a genetic disease (H~rdy Weinberg equation) .• reflects the dtstnbutton of a mutant gene' in the population

e~le usmg prevalence of C~ of 1/2500- • number of couples at risk == 112500+ 114 = 11625, ;

carrier rate ofCF ~ 1./v625 = 1125 .

DeformatioDS:_ • anatomical defects resulting from mecbanicalfactors (extrinsic forces) that usually. occur. In the _last two tri~e~ters. after organs have developed..» e.g., oligohydramnios

producingfacial and hrnbabnormahnes (called Potter's facies) .

Malformation: • ·disturbance (e.g .• drugs, infection) that. OCcurs in the morphogenesis of an organ/s), • hypospadias faulty closure of urethral folds, • epispadias due to a defect in genital

tubercle, .. club foot, '. ventricular septal defect +

Mechanisms or teratogensc • teratogens are most detrimental during the 'embryonic pcriod.-. first 9 weeks of life (4th-5th week most sensitive fOT teratogens), Oil e.g., open neural defects occurwhen tube normally closes between the 23rd- 28th day, • specificeffects of some teratogens;e some interfere with formation 'of mitotic spindle', '. interfere with production of A'TP, • some interfere with gene production (e.g .• isotretinoin effect on Hex/hedgehog genes, which are. important. in embryonic patteming)

Teratogen cocalne: '. maternal .effects: .' hypertension, * abruptio placenta, • newborn, effects: • hyperactivity, '.' microc·e.p'haly,(Me effect)" • interruption ofblood flow Ieading to infarction (CNS,. bowel, missing, digits)

Maternal diabetes mellltus and the teratogenic effectsin newberns; • increased birthweigh.t (macrosomia, large for gestational age]: •. hyperinsulinism in, the ferns from poor maternal glycemic' control increases muscle mass: (insulin increases 'B:Dl in 0 acid 'uptake in 'muscle), '. hyperinsulinism increases fat deposition (insulin increases deposition ofTG in adipose),. open neural tube defects, • cleft lip/palate", '. respiratory distress syndrome: fetal hyperinsulinism in, response to maternal

hyperglycemia. inhibits fetal surfactant production by type n pneumocytes .

Terategen diethylstilbestrol (DES): • mothers received DES to prevent. threat~edabOltlO~S: • 'DES interferes 'with the development of mullerian structures in female, fetus causing abnormalities

in the tubes, uterus, cervix, upper one-third of vagina, • female siblings: • va.gina) a~enosis is .the ,Me' abnormality 'and is the precursor of clear cell, adenocarcinoma of the, vagina/cervix, • ,ccT"Vlcal

l3

mcompctcncc- increased indd~n,ceQf spontaneous abortions, • uterine a.bner . ,'. . .. '.

with .implantation .• fallopian tube abnormalities:- fenility.PrQb1ems tma Ibes- problems

Feta.. .1c()~Ol .syn4,rome 3n,d. ter.t()ge~ic eiTeet$ ~D newbQfDs: • Me terato 'en ., . _. .. .'

States: 2 .. ~ 1000 live births, • clinical ..... · • mental retarda atirm (·M. ···C .. : .3'bno··'rma· ,tl';'ry"~")' ~ '. ~ .. HI United

. .. . . . . '. ,. . ' '.. '. . . .Ii' . ".' intra .. ' ,0

• • ..' • • 0 • _ '. • ~ •• ..... a.U ten ne gro\Vttt

retardation, Ji ~naxlll,at)f hypoplasia, ... microcephaly, + atrial septal defecl$ (least Commonfi~ , .....

... hypoglycemia al birth .\ .. dmg.),

Te'ratogen heroin: • smal 1. for gestational age, • . irritability.' /h\l1"Vl'T'3" . ··;'t~~"'::ru • high .,' ... ·h-...J .

, J t"'''" ,"",1r,.,I; .. hJ l' [U· . , puc '~ cry' .. ' lh

excessive hun ge r, salivation .. sweating .. tremors, fist.sucking -te.mp, eratur re in 'S' tab 'l&l~ty" ~. ', v wr .'

_. ", .' ,. I, .' '.,.'" .' ..... ,',1' e', seizures' ..

'T" ,.," .... ' d ~ 'if:,..'~ /. .', 'I ' .... -~ •

' .. , e'ratog~D:'lsotrettDOI:D: ' •. use'.· m Q.·eal,~ng· cvsuc acne- •. must order a' p··'r·'gnatl' :.':, , -" be- ~.",

. J .,... . . . . ."!W!.&. ........' .... . cy [est " ore

)• 0 th dru 0 L_ .' _' '.' . . '. . '_ . .' .

p acmg 3 woman on . e. •. ,'g." patient must be on birth control pills while lakin th-d,ru" •

newborn effects (3 C's):'" craniofacial .abaormalities fsmall ears). wicro~athia clgn 1:1 )' .. cardiac defects. CNS malfqonations(hydrocqlnalus. microcephaly) . • c:. pa ate.. ..

Teratogen pheoytoin: • nail hypoplasia, • eNS abnocrnalities, • deft lip/palate lI! con'g" enital h.J!I'Io~"'"

dis '. ,. ,_. '.~"

". l$e3Se. - '.

~ternal,smoking: • vasoconstrictive effects of nicotine ptoduceplaeental ischemia-. endothelial

damage increases the .risk for thrombosis in placental vessels •• low birth weight babies .

M()tberswith SLE; SLE patients who have anti-Ro (anti-SS.,A) 19B antiboAies. in thclrserum may

have-newborns with completeheart 'block : .'

Tetat9gen th;pidomide: • previously used -in the. United States to control nausea associated. With pregnancy- currently Used in Rx of leprosy, • limb abnormalities-, • amelia (absent limbs), •

phocomelia (seal-like limbs) . .

Teratogen valproate: op:n :ne,~ tube defects

Terarogen warfarin: • contraindicated in pregnancy: should use heparin, • CN"S defects •• n3~ hypoplasia

Cong enital Infections: • TO,RCH syndrome: * Toxoplasmosis .. , '. Other ·~.V AID·S·. ~,T""\lO' m'l""'il:C"

, .' • .' • ',.' • • v , '.~ , \I;,~ 't. .. ::J r--'" .. ~ ~,.,

syphilis, etc.], "._ Rubella, '" Cl\{\! (cytomegalovirus),. Herpes, • increase: in IgMm cord

'blood, • vertical transmission (mother to: ·fetus)-·· transplacental Me type; • blood: contamination during deliveryte.g .• HB V); + cervical infection (ll~V 2, Chlamydia), '" breast feeding (HIV. HBV., eM¥): "

- - ...

Congealtal ,01"; '. .M·e. in.-ut·ero .. -'viral in,fectio.n.: majority are asymptomatic, ". primarily transplacental, • clinical+ .•. bila.teral.~e~sorin(:~l. hearing'los~ ~,C c_omplicati~on .... periventricular caicification~ '" hepatosplenomegaly, chorioretinitis (may lead to blindness), • urine culture is gold standard= urine cytology reveals large, basophilic intranuclear inclusions ("'owl eyes") in. renal tubularcells, • Rx- 4< ganciclovir (begin with this). " foscarnet (if the former is not worlc::i.Jig) . Congenital I11b~Ua.: .• primarily ~lacental- highest incidence of-congenital anomalies in first.'8 "'weeks- virus interferes with protein synthesis 3n9 producesa vasculitis, '. clini~l- =sensorineural

deafness Me' complication" ,. catarac·~~ * 'patent ductus arteriosus

€ong.enitaJ toxoplasmosis. • 'primarily transplacental, • ~.ontracte:~, 'by 'W'omen. arter_e_xpo§Urt:, 'to cal litter- , pregnant women should avoid cleaning' cat litter during pregnancy, - contracted also by handling' or eating under-cooked meat products? • greater risk of fetal infection later in pregnancy than earlier in. pregnancy, • clinical- .. 'chorloretinitis Me la£2te .. complicatio~:· (often leads 19 blindness). 4< calcifications in: basal ganglia, -Sabin .Felstmandye test (uses live organisms)- "gold standard' test but.is rarely performed

Coage:ait2l Herpes type 2: • primarily contracted by passing through the binb canal in women actively shedding the VllUS- .' women. actively shedding the HSV -2 virus are delivered. b~ C~ section, .. greater chance of fetal infection 'with, primary rather than, recurrent Herpes, '11 clinicallocal or systemic 'disease (~.p.balitis,: skin infections), .• Rx: acyclovir

..,..._ ...... '

:~

CODgeDita~ sypbiU~:.. pri~ri Iy transp1acenlal- Um:OllUIlOn. infect,ion . dwing tim S lltQnths pf pregnancy anatomical barriers p~cvent access to the fetal circulation. • earl r neona . '.' .....

(firs '1 2 yrs . ) .• hepatomcga;l~i' .Me SIgn '. o'st"oc,hondri'-t:r, . (infl '_, '_, ... "'." . "'. Y tal syphlhs

, ' ". , ','. _' - .'- < , , .tw,_., IS In· arnmanon of bone) .' ',',.,' ... " ..

lesions, " pneumonia alba (lobar pneumonia), persistent rhinitis (snuffles.) • 1 '. '. mUCOCUtane~~s

(>2 yrs};> fro,ntaI bossing is Me silm •• saber shins .... rhagades (' .rio:1 fi~O~talsYPhl~lS Hutchinson's ·tri.ad- teeth, (notched upper central incisors, called H' utc"hinsope. '.th .. -,lned3.r sc~rs)~, •

. .. ' .'. .. I· ".' -Ii ~' . n s teet . an'·· 'ma" tt'", - d:

. . I " . 'II" 'd' ' , 'I k..... ..' .' - 1 - ",' s) " . .. - '- . ~., . . I k ,.; . ,., . , -~ 10 nne

mo, ars ca 'e·· mu.·ucrry mo ars,,' • .mtersnna eranns (b. lindness) • sen's' ori ... ' ,-, hes .... '

. • • . • ' . 1 ' " n,eural . eaTIng:' los'S '.

Rx.,: penicillin ' •

Congenital Varicella-zoster virus. « chorioretinitis-potential for blindness • r b h . '., ...

cortical atrophy in the brain, • vesicular skin lesions ' imn yPOplasla~

~e ~epeDdent djsor~ers (iDevitabl~ witbage); • iocreased body fat: decreases the number of insulin receptors leading to glucose Intolerance, - 'respiramry-, • obstru~tive type of .... ' '. .

pulmonary function tests (so-called "senile emphysema"), .• decreased elasticity (;educe,t;~:~~: expiration), * decreased forced expiratory vol urne 'in 1 second, (F •. "EV , ),. decrea sed forced ,'_ . tal' . "

. ',' ~SCC ll,~ .1.. 1~ VI '

capacity (fVC), • increased total lung capacity (TLC), • increased residual volume (RV) ... low

normal PaOlI • ca,rdiovaS(:ll,iar- • loss of elasticity in aorta, • decreased cardiac output and heart rate in response to .. stress, • at rest, the cardiac output is unchanged, • mDsculoskeletal~ osteoarthritis in weight bearing joints, • CNS- ... c:ercbralatrophY with mild forgetfulness, • impaired sleep patterns (insomnia, early wakening), .. Parkinsonian-like gait. • sensory changes- • cataracts, • arcus senilis (ring of cholesterol around the cornea) ... presbyopia (inability to focus on near objects), .. presbycusis (sensorineural hearing loss particularly at liigltfrequency), •

'1' . ~I:.. .. -- ~ ,'" th . 1 rod '. d

otosc, ~OSlS I.. .. ustcr, or " e ear essie cs p . ucmg con uotive hearing loss) •• immune system- •

decreased T cell function (often anergic to common skin 'antigens, positive PPD non-reactive or absent), .. loss of isohemagglutinins to blood. antigens (may not develop a hemolytic trans~ion reaction, ifbJQod is mismatched). • skin. cbanges- .. loss of skin elasticity (increased cross-bridging of collagen), .. senile purpura over the dorsum of Jhe hands (common USMLE picture) and: lower legs where bumping into objects is most likely to occur,. decreased sweating (eccrine glands fibrosed, danger of heat stroke), • G,[ tract- '. decreased gastric acidity'".· decreased colonic

motility (constipation 'which predisposes to diverticulosis); '. decreased activity of tilt: hepatic cytochrome 'P4S'O system (danger of drug toxicities), • male, reproductive-- .' prostate .. ,hypet,plasia (increased 'urine, residual volume' with subsequent: increase in urinary tract. infections)" .:, prostate c'ancer (only cancer that is age "dependent), • decreased te.stosteron"c,. Iemale reproductive- • 'breast and vulvar' atrophy due to decreased estrogen, .' increased gonadotropins, • endocrine system ...... !D_c;reased glucose intolera'oce (due to increase in body fat and subsequent reduction in insulin receptor synthesis), '. renal ch'ang,es,-, ,d,ecrea.sed,G'FR \vi.th .reduction in the creatininc:' c;learance (risk: of drug toxicity due to slow' clearance: of drugs),

. . -

Age-related changes (increased incidence .in elderly but not inevitable): .• cardiov~cular- •

atherosclerosis with increased incidence ofcoronary artery' disease, peripheral vascular disease, and

strokes, '. temporal arteritis, • aortic stenosis <(){C valvular abnormality in the' elderly)" • systolic hypertension due to loss o fao rtic elasticity, • musculoskeletal system- • osteoporosis (particularly the vertebral column in females and femoral head in males), .. Paget's disease of bone, • respiratory system= pneumonia usually Streptococcus pneumoniae (underscores importance of Pneurnovax vaccination in elderly), • CNS- .. Alzheimer's disease; • Parkinson's disease, .. strokes (!ltherosderotic type, is 'M..C typ<:' of strOke), • subdural hematomas (falls cause tearing of bridging veins in subdural space leading to a venous c10t)l- sensory changes- .• macular degeneration (MCC of blindness in elderlY), • skin disorders-. UVB light-induced cancers,. •

.. · (. . Iar ') ke .' .- .. " , ( .. , .. ' .'. 'US'ML" : E·· .. ,.......... ·· ... curs······ or of s-quam: 0, 'us c· ancer) • endocnne

acnmc '. SQ,' '"' ,. eratosis .. c-onun'on . '. '. ... .. PICl.u.I C, Pl.,,,, . .:: ... -' .. , ..... , - . . - . , - . ..... I ., ,

system- type Ildiabetesmellitus

15

_ . .....

Top 5 causes of dearb in ma.le5 2a4 females regardless orage. ADd sex 111 the U'nitecl.Sta '., .. ',

d d· .. d 'r',j • hea ...... disease • cancer • stroke ehrcnic ob tes ID

.~sceD mgo r e.'.~l-..... ":'" - · .: • . . .' " ," CUI orne Q;st:ructive pulIll9nary disease

'(. 'COPD)' . • motor vehicle accidents-- Me COD In· ·males.·an·d females be"':h'Wleen 1-'3"9 'tl'rI!" '.

. . I ". . ". 1....,.... ~ .. ·..;'or· ag

Top.3 ~2 US~ of death in children ag~d J -44 yrs In desc(ndingorder: - a~cid~t.s_'.1; -5~ '.

M'V tl 'II 25 300/ .&. b . S I 0 I So/ ) -, . , . 1 1 <I . - • Vo

:' M, .: .3 ,: .S',· '_'.: .. zo, '!'·um· .. : -'.' 1'1.)' ' .• canc,c:r~ Ii' congeruta anoma 'ICS

Top,.3 risk. factors .1c~2di~~. to' i D.cre~ec;lmorbidity I~~~n~ ,in tbe U.~ited Stares in 'descend~Dg orderre clgarette~mqking, • dietary fac~ors and acnvuy patterns; + high saturated fat, low fiber

diet, .. _ lack ofexercise, e· alcohol abuse . _

GUDsbot wounds: .. contact wounds- contain sootand.gunpowder inthe wound (called fouling) • intermediate wounds- powder tattooing (stippling of skin around' the: entrance ,site)· • distant

,., . d I ., .

w~lUnds- no pOWdCT tattoomg, • eXI t woun: s are arger 2JJd more ,irreguiar than entrance woUnds

Motor vefiideac:d~e:nts ~As). • MeC ac~i~ent31 death between 1-39 yes of age: commonly alcOh?l~r~Lated. particularly l~ te~ag,CI'S •• 5eatb~lts' and air. bags have ,redu.cejl mo:rbidity~mQt'"Sllity Drowning: • common COD In children from 1-14 yrs -of a.ge, =near drowning; survival fo1.fQwing asphyxia secondary to submersion, • wet ~ownin:g: • 90% of cases, .. initial laryngospasm on <Co 0. tact with water...-+- relaxation/aspiration of water, • fresh/salt water .drowning: whether fresh or salfwater drGwning. suifactant is destroyed in lungs-4 atelectasis with intrapulmonary sh~ti;ng-40 diffuse alveolar damage ana initiates spasm in the bronchioles. -immediate ·COD in drOWning is cardiac urh'Vthrnia.

. . .

Burns: .' first degree.burns: '. Bainful partial thickness burns (e.g. .. .sunburn), •. produce-cell necrosis limited to the epidermis, ,. heal without 'SCaI;" • second degree burns: "'pamful partial thickness bl,1I1lS. • involve entire epider mis.s forTti blisters within epidermis, heal without scarring-unless they are deep, • third degree burns: * .painless 'full thickness burns, .' extensive n ecro sis of epidermis/adnexa, • 'extensive scarring complicated :by. 'keloid formation (propensity f~Yt.sg·uamQ·\1S cell carcinoma), •. healing. comes £ro,m Tesidual~ithelium a_t the 'wargins .of the .bum and, from adnexal structun:;s; ~. COD: infection due [0 most <colI)lIlonly:toP.reudom.onas aeruginosa followed, by Staphylococcusaureus,» house 'tires: '. smoke' inha.Jatiori .. 'Me' CO,D" ':if, 'carbon' mon:oxide {coy and ,evan ide· po·isoning:·commonly·occ'uf (sourceofcyanide 1S polyurethane i.n upholstery]

Heat .inj'u.'ri·es.: •. hyperthermia; core 'body temperature >37·,~'2.oC., • .heat cramPs,:.:* afebrile" ' •. common in untrained athletes/laborers 'who become :v'6I. ume' depleted, ~ lose excess .amounts of "Sa 1 t!wa ter l' .. hc"-t exhausti'on- '. mild el'evarion: in core bod~' ·temp~ture (s39~'C)~: • common in 'athletes training .in hot/humid .environrnent, • develop severe volume depletion, e· eX.a tio·nal type. of 'heat stro,k,t; .. : .• ' core body temperatures, ~ 4,loC<f • people working or running' 'on hot day, -- skin hQ~ld.Iy., .... profound lactic acidosis, .• rhabdomyolysis common, • non'~·,x~rtiona]. type o'{ h~t. 'S,WOKe- ~,·eld·etly/chro.nically ill patients, .. skin, hot/dry wlthout sweatiug (hypohidrosis), .. lactic'

acidosis/rhabdomyolysis uncommon.> trialignant hwerthemya-· AD disease, 4!defect in calcium .release channels. '.~ 'the 'muscle sarcoplasmic reticulum, .,. massive muscle contractions with extremely high-temperatures after induction of anesthesia by halothane and. succinylcboline (mUscle·

-relaxant], iii :Rx' with .~troltne, .• screen family members. with muscle biopsy and caffeirt.dha'lothane contraction tesr OIl muscle, .• body. tempctature and r~dness: of·s.kin. for- a patien t. \\'31klngbriskly on a:honiay- • no increase in rectal temperature, • vasodilatarionof'vesselsin skin producing redness,- choice D In schematic, -marathon rpllDet on a hot day- "increase in rectal temperature (probably 'heat exhaustion), ... vasodilatation of vessels in 'skin producing redness, •.

choice C in schematic B C

R,erta1-T

, '

A

D

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16

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Cold i~Ju.ries: • n:--ostbite: .l-oea~~~d ti_ssueinjtiry s~condary to direct (ice cryst a llitation in: ';" "

and indirect damage (vasodi 1 atatro'nI'th:Fo-rnbosl:s)' 'Ii tiss iue 'P'" ~'';''n' 1 ~ ''': . '. ".:. .~Jls)

, , . '. i, 4 .... .~ g.a - ,leSS·" • general 'Zed

hypothermia: • CQ:rcl' b~dy ~~IJ1Pt:raturl'~ <3-5rIJC. • whQl~ body exposed to- freezing' ,ternpet;:~~s ;01' a

prolonged peried ,0"1,' nrne, .. uncoup .mg. ()' o'xidative ph~lsp':ho,rvlation. '. ven 0, -' -.~' ,', . ". "'lj>,~" .,,~, ... ,,".'"

v • • ".~ J "' .. ..... ,u~, 1100 mg· u~t 'rna' , 'y

progress into .eirculatory failure and death . . ,'. '" '" .' ' " , '. ".'

Electrfca] htju?: -Ohm's uv.:: .. curr~nt (I.. amps) :: voltage:: (E}{n:sistance (I,t.,ohms).,. namps) =. E (volts)!R~o~~, • current ls',mo:t Impo~tfaetb[' in C!le(.:tJCX;:llti,9n~alt.emating,e~enttAC)::.direct current (DC) risk for electrocution, '-resIstance: • dry skinhas the highest tissue resistance to

c urren t., particular 1 y 'the' hands and, feet, '. wet skin lowers the res istanee to' c ",..,....., t, "S' ;...t"".. .. ' 1" ta ' .- ..

, .' . ' ,. '.., . ,'. ,Y.lI',","U~ ',U,I~ va, :ge lS

~: constant, lowering resistance increase~ current. .' cUQltnt:moving from the left, a~ [0 (he right leg is most ,da'ngerous'- .' involves the heart-v ve,tt.tricular,fibriIla''';:o''', .. b;:'I:'ood," ."~'I'~'S"" ·an.:·" _'~c""e' 1"1' ~'t,"c'/ ,. ,,'d'-,"- '.',

, . ,. , - .. ." 'h' "'.' ,,:1., ".: '",,~,. lrw&.I.' ~ ,on' uctor

ofcurrent, • COD: cardierespiratory arrest withventriculat flbrillation'andrespiratotypatalysis' ,

l'O'Dl"nu'g radiation; .. ex'ampl~s- y-.ray.s,'. x-rays, particulate radiation emi rt ·,d·' bv ra .dioa ctiv '

, , , . , . 'lru.., , , J . .' . , , .. c:

substances (alP particles), '. shorter the 'wavelength' the greater penetration- • lo'~

peaetration ,include:', wf3~patticles.;,. ~ high penetration include y-rays, '. tadiatiOl1 inimi":.'tiirect"Qt' indirect .in] ury to DNA, ,. indirect ~m>e produces <h~ydJ:oXylfree 'radicals from, ~ydrolysiS of W";!~' .in. the-tissue, '. ,:DNA.,'is '[IlOSt :stisceptible·'protein~ - tissue susceptibility.to ra~ia'tio:n,,-,:. directly related 'to' degree ofmitotio activity, with the greatest effect-on actively mitosing cells; .. i:n,djr'c='ctly related' t,o degree of tissue .specialization (seebelow), '. cell cycle-- .: peak sensitivity is in G,:! (synthesis of' tubulin formitotic spindle) ,and M phase (assembly and disassembly of mitallc,spindle), • Sphase least sensitive, • OJ phase, intermediate sensitivity; .' tissues With ,:3, high radiosensitiviry- • hematepoietio cells 'are most affected, (e.g., -lymphocytes :> granulocytes > platelets :> mature

RB,C'sJ:~: • germinal. cells in 'the testes, '0 varies, ~ .tis:s;ucs, with lo~' ~dJosensirivitv-, .. brain is most

'resistant lQ, radiation, ' •. ~bo:oe, II mature cartilage, ., muscle, .' total body lrradia.tio,n: * .h;emat'op;Oieric, System fi~, ~ystern ,affeCted (greatest overall effect-s- 1 ymphopenia "(~t cbangej-» thrombocytepenia-» bone marrow' hypoplasia), :~ vessel cbanges= thrombosis (early), fibrosis (tate" ischemia), .* . skin, changes including erythema-s- edema-s- blistering-s chronic radiodermatitis-o , x t, ~" tial fors uamous celt care .. inorna '.' GI tract.with.diarrhea

po ,en. " ,.or- q, _, .,. A'IL~

'UJtr:aviole-t li'gh,t (nou-Ionizing radiation): I.' IN A- Wood's lamp, (black light), • uvs- • sunburn, .• corneal bourns from .skiing, • mutagenic effect On skin (mymi~:in,e., dimers), • 't;v:Cgermicidal, • UVB"'rela,ted, can,ce:rs~, ." basal eell. carcinoma i.s Me .skin cancer, '. squamous carcinoma (precursor is actinic/solar keratosis), •. malignant: melanoma

Laser' radiation: intense 'localized heat that is.equivalent to a, third degree bum

Mlcrowave radlation. « produces, a skin burn, •. adversely ,a ffect pacemaker devices, • inconclusi ve cornplications-: • 'cataracts, •. cancer ••. ',sterility·

Infrared radiatien: • bums, '.' cataracts

High altitude injury; .. ,gen~l- '" O:;t cQnceIltrationis 21% (norrnat} ... decreased barCl.metricr

pre~sure (e.g., only 200 mm Hg on top of ,Mt. Everest),.' hlPCrvmtila~on is:-essentia.l~t ~igb altitude': since it lowers 'alveolar C.02, (respiratory alkalosis) -+ automatically mcreases alveolar

PA02--+ increases arterialP02 •• acute mountain sickness: ~ occurs withinthe first 24-36 hrsof an ascent above 8000-10.000 feet, • headache, lethargy, insomnia, dYspnea~ .. RX with descent, mc're3scffuid intake (increased .insensible water Joss from mucous membrane evaporation). and

oxygen, '" preventien. by ic,c H mati xing before '3S(';,ending and usingaceta2~lamidel.,which is ,a, carbonic anhydrase inhibitor that produces metabolic acidosis-s compcnSanQIl 'for the expected

'respiratory alkalosis, .. high altitude pulmonary edema: non-cardiogenic

Cbanges after- death: rigor mGrtis is due to decrea-se, inATP in muscle, , o.

Sites of al¢Obol(etbyhtlcohol, ethanol) reabsorption; • small ihtestine- 7S%t· stomach- ... 25 ¥q •

• partla,lly metabolized by-alcohol .dehydrogenase.

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Llver ructabo:lis'm of alcohol;

. , alcahoJ.deh)?drogenase, . . alde,hyde .de.~ydrogen~e'· (blocked b· :d~"··, .1#;:---: '.

'. I' . 'h' .. ) III . . tald hv d ' 'N" ':AnH' . .. y. lSU, I:UiUlI)

a~o, . ' .• " '. d3cee y e + ,'ALI" • "ac~ta,te +'NA1)H ..., a,

Pbarma"'ologl~' acnou of -lc.Abo'l~ • CN· S depress .... nt d ~'~' .cety1.,CoA

, . ,:'. """ ',' '\.,h ....... :1:".,: ,..', 4iU;,:v,', ~",' p, ,:,C::~,& ',;In .. :"escenumg·,'··ord. ..... '~~· eer \..........._:, ,,' '., '

_ , " ' ,",,",I., ----,. , .... re.us:;al cortex

limbio' sysrem-e cerebellum-s- lowerbrain stem, • potentiatesinhibitory neurc ,~,~~~:'~ . '.(:','~'~

I . ' ,., ~ ,~O.~ u.&',~"·l;Utters like v

aminobenzoic acid, .'. aldehyde dehydrogenase- deficiency- ~ • affe cts·'_Ano/' . f A .... ,+'· .", "." I""

, :',""',"",' " ... .. ",:" ':,' . " '-". "'.",' ' .. '''' .: ,J:l~ .,' -"\1"$ ·(l Asians • 'b:' . fId:' ,:. ". 'f"

'" ,,', ,'",'"., . , . ,u..&'~1! .Ul -u ... ' .

acetaldehyde .. 'and ,01 upset (similar to action 0.£ disulfiram) ,.' alcohol taxi '~'w·' ·'·s'··O··",,_-po.,'

'. ' ~..' .1I,iI" :1 ... , '!IiiiL4,1;" t, ~ .. """,,, . . 'U' let ~ .' " . . ,·ftJdL' .

" h'··· '. '~',' • 100" --gJS'f~ I' l't ' '", .. ' r>. ", " :, ," ',: m:&,",

eUl?,ona~ sreg<!tl.0 us , ..•. ' ••.•• <m-.-uLe~lydnmkm m05~s~t~ (slurred Speech.'UriCQOtdinatcd)., ~

300-:l50~ rng/ dL stupor or coma . • ,:>500 m~ldL d ea ... :a.:.., .' delirium " ,; tremens ".' '~o' '1'1' . ' .'.

,. ,. .' . iIII''''~ ,. " 51', , . \.1.1.;;, ~. ,',,' ,..",. " ...... ".w:- ., " . I o~'t.l"t''I'''i.;g '~~ ...... 3' 5'

days after complete withdrawal-etremulousness, disorientation, visualhaUucinati ~~J7";' .. -. &x 'With IV di@Z9'am and't}1iaminc . < ..QllS" agnanen, t;

Diseases wher'eal~hol is th~ leildin? ',cause:.'. thiamine deficiency: Wetliicke.;:ssyndl'om~ ,Ko~ofP~~sY~h~S.IS\CQ~s,~lve, .. card~~myopa~Y> •. ~~IoeYtic,anemia:folat~ deficiency, ! acquired sideroblastic ~~a- microcytic ane~ WIth ringed sideroblasts •• ' Mallory Weiss

syncm:m~,: tear .of thedl~l. esophagus/proximal .stomach from retthihgj\. Boerhaave-s syndrOme:: rupture of the. .distal esopha,~uslpioxinla] stomach from f'C,{C,hin·g .. , • cirrhosis ,.,~

.. . tt:. ,. . " ~

esophageal ~c'es~'·e'.llect .. of 'portal vein. 'hypertension due to alcoholic cirrhosis, '. fatrv chang' ,'C in

.. L '1· h ~d · ' ., -J .

ulCLyer ••. cmOS.l.erosls- :akoholincreaseS .~ reabsarptionof iron, • acute and chronic

pancreatitis, .• type ,N 'h:yp'erlipid:emia,~' alcohol 'increases 'synthesis of. VLDL, .' Kle.bsi~-l'la

pneumaniae pneumonia . '

Al,c:oho:1 :is' a cancer rtsk: squamous -, carcinoma (~ergjstic. 'with smoking): oropharynx, mid-

esophagus, larvnx • adenocarcinoma: :p,allcl:e3S. liver '

. ;:.-~., ~ J "_,__"Jto • ' ,. 1 . '.. '. _ ", ''''''.

,A]colJ.:o.l. effeets on, ~SIPNS~. ~,., Wernickers syndrome/Korsakoff's' psychosis). cerebellar

degeneration: Hu and Yo antibodies noted in spinal fluid, .' dementia • DTs .• 1 d:iSta.l·'~:h'· era' " "1'

, .. ' ' , . " .., " ". , '.t"~"".r··'

.' " th " '. ·':1" .'. tine ., 'I'~ .' "1"· ..... ',;;'" d _. ._, eli .'. _ .,. " , . -, idro '.. du ,.. ,',,"'. '. '.'. . ~

neuropa ,YI.> • centrai POlllBC mye mo ¥SlS~, ,: emyennanon syrl, ", ,~mc: ,~U,~: to, rap,~~)IV Rx Qf

h '.- . ~ "tt·· . .. ~,

.. 'UTW"lna'·'- ,~.1:a

- ' .:J F·...., ~_ -' "_ ~ ... --t.-~ ,

S,m:o·kj'Dg~~,epidem.iolo·gr: • 'M,te of premature death 'iI;t theUnited States, • Me" single preventable cause of cancer; .: incidence of smoking is increasing' in "women, and decreasingin men, • nicotine

~'. .._I_ , oj. , __;;;:-A:' 'b" . , '~'1' ,; '1 ' 'fr " '. '~ '.,;, .. ". -;f' .

intake can be momtoren .'''y. measuring p asma or urme: level 0.,' ,cotlni.n:e:,- cetmme is. pnty derived

.' '... fmi '. ,.... ..' ,. . . ~. ~ .

from -the. metabolism n rncotme, '. 'MOA of mcotine: '. absorbed 'rap';ldly into 'tb~, pulmonary

circulation, .•. moves into the brain where .. jit attaches to nicotinic-cholinergic teteptorS' to produce its gratifying ,(;ffectslcomplica;ti9Il -of'srnoking, .hlgbl,yaddtctlVe, agen_!;· inactivation of the p-53

b + • hr 1/~' .;1.. M' p.. , ; def ~ 1,";';_

suppressor .gene y a pomt rnutanon on c . ornosome .. ',~ is' :U:lC' ,'., .. \..... genetic tetect 'm smoking-

;i; d' '

m • uced cancer

Cancers -where smoking is: tiLe leading cause: '. lung, cancer: squamous, small cell, and

, '

ad,mocarcinO:lIl!! 'to a lesser extent, ,. MOe of death .due to cancer inboth men and WQJllCll,. til oral

. ""oi:I"",,," ... ,I' -. ,'- d "1" .' ,', ,,'I." d r tid . :' 'ha" eal . . ,', , "','", ,,' • '.';.... ,'n'·· , ad .' .... rcinoma '. ~p,.IJiGJ:J',,,~g~,. ·an: ,,:aryn,gea. an , [Ill. -e.sop . ,g .', , ,.squamo~ cancer. .... p an,c rea, ',C, a . enocar I:, ,.', .. ~ ,

~~.ti,o,nal cell C3tcm,o,ma, .of bladder" • r~l a~e"QCarcintima~,

Cancers where smoking itasbeen impUca*ed but iSD.Ot the Me,risk fa.ctor: -cervical ~cer'_"

. ," ~". 1...,'"

'M'ua.m' .. , '·o·us·_ ,..~'on"...;..r,·. ·catcinog·:01s.' foUnd in .c'ervicaJ. sccIthons, ... ' stomach aden:ocarcino~ •. u.reast.

~'" '::"'" ~"'~-4't , .... ", .. ',., '",,' "','" ',' .. , . .., ., .. ".,., '

adenocarcinoma-primarily in WQnien who'are'slow~acetylators ofN-acetyl1ransJense 2cnzymts •. •

prostar~ aden()(,'::arcinoma. • cQlon adenocarcinoma, .. leuke~a:- inctQ,S:tdrisk Qf bothlym~hoid and myeloid lCuk.emia5\·~ :.alCohol is a ~ocarctnogen vAthsm9JQngthatt\Jrtha efihartces the nsk of

.oropbazyng~l, esophageal. i.aryrtgeal canCC1"S, • SIllQklng +as~to~ ~xposuil::.~kedl~ en~ces the;inc:idence of primary lung cancer-·no association of ~king With mesothelioma. whether the patient is '3' smoker 'or not.~ IWlg cancer is the most. COmtT1O!1 cancer associated with asbestoS

.' • ~IJ : ~~.

eYfV\C::'Dr' . e" . ,....... ,.. .

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. •..... ~ ... '~

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18

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N"ote: This' 'material is cop,yrigbted,~, All rigbts reserved. ,Ed,w.rd GoIjan, M.D .. 2002

Smoking effects on the ~diovascular/CNS systems: • increases risk for AMt- ine eased " '" ',,' .

recurrent AMI as welt • tncTeases risk' for sudd ", rd ~ d ,," ,0 " • I, , nsk,-for

" - • s , ' , en car lac ·ea,th". Increases risk for " '. he' 1

vascular disease, • increases risk for strokes, • cQ,ntrlb'urln,g facto'rs~ " enhane , 'd: ath ,pen~ra

~ ,. k d" , ," ,,' , cc a ttosclerosl's due

to chemicals m smo e an, mccune effect on blood 'p,ressure and heart rate • "th'" ',", , +' •• ,':'

,I - " ~"'" ' _ ' , ' , '1: a " CTogen I.e IIp! d

profiI~. • tissue hypoxia secon~ry to excess carbon monoxide (CO) .... hypercoagulabili

Smoking effects on the respiratory system~ • COPD- -80% of an cases hr .' tybro'" , :',0

, , . C ;, .' , ,J ~, ,ORIC, nchitis

ernph~sanat,·recurrent mrecnons- pneumonia, URIs, • exacerbates bronchial asthma, • cancer' 1

Smoking' effects '00, the GI system: • GERD;, - delay's the rate of ulcer h,,-""iIlm~'" g " '" d' , " 'k. C

' ,- ,_"', "'""'.!wQ. ': .. mcrea.se 'ns lor

oral, upper and lower GI cancer; .. USMLE scenario-. advice to give a srnoker:~th rust" " ,f ",'" ",0,

I di T....:..... _ . . ,ory 0 rw-nUc,

u cer '; isease: stop smoking r--t'

Effects ~f smoke~~ toba~co (snuff, cbewiug tobacco): ·e nicotine addiction, oral leukoplakialcancer- inside the~lp. under ~e tongue m: cheek. • verruCOUS· squamous cancer, e nasal c.anc'er-' snuff users, .' aggravation of cardiovascular' disease-. nicotine effect

Smoking effects on bone and menopauser s increases the risk for osteoporosis in men and women,.. b~ochemi~1 ~e.action in wo~en-, ... CSfi'adi?l (most potent estrogen) is normally metabolized U1 the liver IOtO estrone, which IS metabolized into methoxyestrone (no hormonal

activity) or estriol (strong estrogen activity)-+ smokers have: greater conversion of estrone into the inactive metabolite leading to low estriol levels-e. low levels of estrogen increase the risk for

osteoporosis and premature ,menopause, '

Passive smoking effect O,D t'hildren: • pathogenesis of passive smoke, effects-, * ...... 75% of total combustion product' in a cigarette 'is exhaled, '. risk of passive smoke. extends. to, children as well as adults, '. increases the 'incidence of SIDS, • 'increases risk for lung cancer- t~2 times increased cancer risk, • exacerbates asthma, • mer-eases' 'risk for otitis media, • increases risk for recurrent upper land lower respiratory infections:

Miscellaneous smoking effects: '. increases. risk for developing proteinuria in diabetes mellitus, directly responsible for ~25% of residential fires, '. 'vitamin C deficiency

Beneficial effects smoking cessation: • longevity- smokers who quit, before 50 yrs of age have half the risk of dying over the next 15 yes than, a, smoker has, • lung cancer- '. in 10 )TS, there is a, ,50% reduction in lung cancer When compared to a smoker, after 15 yrs, there: is only a 160/0 risk, for

lung cancer 'when compared to a smoker, .' ,A?vfi- ANI! risk approaches that 0,[ a nonsmoker after I yr ofabstinence, • 'prcgnancy- pregnant women who stop smoking in 'the first trimester reduce the

'risk of a low birthweight baby' to that of a nonsmoker, • forced expiratory volume in 1 second

(FE V IS(;C)- • it is not improved 'by cessation of smoking, i. rate of decline is similar to that, of a nonsmoker

Drugs and interstitial pulmonary 'fibrosis: • amiodarone, • bleomycin, •. busulfan, .' cyclophosphamide, • nitrofurantoin, • nitrosourea, • ,m,ethysergidt:,- also retroperitoneal fibrosis and Raynaud phenomenon, • methotrexate, • procarbazine

Occupation e~osure relationships: • automobile tnechanic- carbon monoxide, • pestici~e industry- • organophosphates, .' arsenic, • meat packing- polvvinvl chloride with risk of hepatic angiosarcoma, • insulation/demolition/roofing material= • asbestos: lung cancer, mc:s()theli~ma, fibrous pleural plaques (Me overall complication of asbestos), • formaldehyde, .. dry cleaningcarbon tetrachloride with liver necrosis due 'to free radicals, • rubber/chemical industry- • 'benzene: aplastic anemia, leukemia, = amline dyes: bladder cancer, • battery, smelter, plumber/fO\mdry-lead poisoning. painter- ... methylene chloride: converted into carbon monoxide. • solvents. • lead,. petroleum- • benzene .. • polycyclic hydrocarbons: lung cancer, • sewer workcr-' hydrogen sulfide

gas: sulfhemoglobinemia ... . .. .

Isopropyl alcohol (rubbing alcohol) poisoning: • metabolism- .. metaboltc end-2roduct m the

liver j's acetone: ao metabolic acidosis 'unlike other alcohols" '. increases serum osmolal gap:

19

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... . - '---.-., .. '": ~.. .,&,. uv ~

..

t

difference between-calculated 'and measured serum osmolahtv :»'0 • eli ni ··'1 -'.,

" '1r;,J ,1nlca,- d~ . ,

hyporeflexia ' '" ~t-- co~_ "with

"Me(iiyf ~I.(::oho-l ~o9d"s alcobol): :. 'increased anion gap -metabolic a.cid,' •. :" .'. '. . .. " ~,

t" ~ id ,. '. ~ d -" OSlS (converted mt

romuc acid), ,.h optic neurius ,an" potential for blindness, • Rx '. W'l+th"'" ·IV·.' .• :\... .... ~>; •.... '.,.' .:"0

-- , ' t ". . "eul4llo1· than 1 i,

competitive antagonist with methy! alcohol '{or alcohol de'hyd(ogen.ase' '.' '. ',+ e·, 'o.! IS a

Ethylene ~lycOl (a'o.tifr·ee·ze)': .. ~ncreased anio,n"gap.,metabolic acidosis (Converted.'~., ' .. ,,' .. z ,

• renal failure from calcium ox a la te crystals obstructingthe lUIIiens".Rx with IV ~:':~~lC acid), is a competitive antagonist withmethylalcohol fa:r alcohol dehydrog~c:" Q • ethanol

Mer~~ryp<lisoui~g': • ,MOA- " toxic in inorganic (elemental) Iorm: dental amalga,ms. used to be used In hat making Industry (u:mad hatter' disease") *: ,tOXl:"C: in or'~nl·C· C-.' .' fun' , .. , " -- ,

• '" '_. ,': 0 _ ,_ ' t, '''' "eJ~"" 1.0rm.:, ' mgicides,

contaminated fish, .• ' cltnlcal- If' diarrhea, ~ visible. on: x~raYSt ., n.qihr·~t·oxic A TN ~n~' ,1" . ~' .. , .• L"

, 1-' I ' 'braIt· bel '. .' . 1. 0, .V1ng :U1C

proxuna,. tubules, ". ceret '.': ·ce.r~' Ilar neuronloss, •. constricted visual fj·l',.lds'· ";, • D'._, - eli'- , .', 1. "

, . ' , ' -e- , ". " , ••. ~ '. "\I ('-A-.' m ercapr 0'1

Arsenic poisoning: • sources=e pesticides, ... animal dips .... Fowler's solution .~ Riof.·· .hi·::l' ~','. ,

. 'a .. "', .' " '.' ,., , .. , syp .IS" In

.1 930'~. • cllJ,uca~- • garltc. ~or .to breath~ ... ~vere diarrheait'rice waterlfstool·s similar- to Cholera, 40

~~lC mdartoS1S:&f'a:Y skin with dar~. macules, • squamouS cell oarcinoma of skin and tun~. " nalls ba'~~e tr~ns~"ers~ ,_,bands (Mees nails): concentrates in keratinlhair/nail~ .' conV:1l1sionSiconta:

M.e ,qoD,~ .' nephrotoxic acute tubular necrosisinvolving proximal tubules:" .' Jivcr'angio,sarcorna~ .1 Rx~ qimerca.ptcH.

I\1·~,shr.o:oJD poisoning (Amanita): .. MOA7"" 'toX,in inhibits RN'A polymerase, • .abdorninal pain/vomiting, '. bloody diarrhea, =jaundice: 'extensive. fatty change

Petroleum producr (gasoline, kerosene) disorders: ,_ euphoria. (drunk acting) 'when 'inhaled (or ingested], • addicting, .• '(oxic, doses- .•. convulsions, * tinnitus, .. non-cardiogenic pulmonary edema Strychnine pqisoning:, • MOA-, CNS stimulant that blocks postsynaptic. jnbioition'l .' clinical

:(similar to t,et.an,usl- •. tetanic convulsions, • opisthotonus, • risus sardonicus, ;. .death

. ... ;. :

Poisonous snake envenomatioas: :. 'types-- • I!it 'v1,pers,: rattlesnakes (M'e bite), water moccasins,

copperheads, if true cobras: coral snake (neurotoxin, thathlocks· acetylc~oline ~re1.ease,-+· paralysis and-death, similar to botulinum poison), coral snake has following color banding= "red ·an.d yellow kill a fellow", harmless scarlet, lang sna](t;,- "red and. black .friend of 'jack" ,. • 'pit viper envenomations-- ., local swelling/necrosis, •. hematologic problems: DIC, • antivenin isavailable:

danger of serum sickness

CD~o:. COI.iUl.lQn,,·a.c'ute lymphoblastic leukemia antigen _(C·A.(:~)

Kid staring. 'into 'space that has broken bones: absence seizures

Testicles, ;i·D,:,ft·e:rni.a,'sa~:, • testicles are not translucent, _..' -translucency ,in' the ,"scroraI .sac implies '3,

hydroce 1 e .. __ ' , , ' ' Dock worker for 20yrs with bing,. mass: primary lung cancer secondary to asbestos -cxpQs~

from asbestos insulation around 'pipes in ships

Belly full of scars: acute intermittent porphyria nut to deficiency of uroporphyrinogen synthase

Flame hemorrhages in the retina: sign of hypertensive retinopathy. . .' .. ' ..

Visual field defect with ~n.niOphaungic)m3; bitellIpt':u;alhemianop$la-(uDpmges on optic chiasm)

:Sta, .. icks '0:( b'em,o.g·lo,bio W.b.e,o ,It the blood'Is deox.:Ygenated:, H~S .

.. . . ' ." (h f' I '3 •

Patient with Dormal PrJ P'fT, bleeds. after surgery even after ~ec:el\ring · res .' ·.:rozen p 35m .

probable . platelet problem+ e.g .• thrombocytopenia. patient on NSAlDs . ., ,'.. ,.

, cc· _..... IS' (" . 'mary'" .. ).. • histoplasmoSIS •

Cavitary lesions in upper Iobe: • M IS reactrvanooLl > , no~ pn ., ". '. . ' ' ..,

Klebsiella' p,neumoniae, • ;squ:amotlS carcmoma . ' -:. : ... ' k Wdl-differenti:lt~ sqU2.~9US cell carciiu)Ma. or lung or 2Dy other tissue: • keratin IS red, • leo .

~:;i~:;~;;: =~anis syndrome bas diastolic murmur: aonic;t'Cgurgitation due to sttetching~of aortic valve ring ~by the dissection.

.20

N'Ole: ··Tbis n~Jrtfti:d is copyrbJbted.,...All 'rigb,ts~ .rtser"1'ed.. i:" , ·Got'·" - M- . ft. '" ,

,- .• . . .~ u.. .... . Ja.. '''';' .. 2002

,~

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r::I"'"

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X;"I"2\~ fi,D·diD:g·S h1 os·teo.g' enic sarco"1:na: •. n;"!II.,~'" in 13· ph': , ' , "th." ,

.. . ... . ' '. . . .' ... ~ .;:.. -, YSlS wnn '"., . ~,

.. ' -b" - -'1-" rrf ' '.' , '.. :' .Invasion, :mto SUlTnund",··, '

m usc.'·' ,I~. '. u pper' tl 13 0', f' ower ~ #!11m· ur , • x-ra y_ •.. r.;...,..I~_,. ,.: gl' '.'"4,1.' lng ..

. :.te., '. t"' _.... . .. '-'. . 'I ~ . . .. '-'JU.I,1JiiUl··S trian 'e • SUnbUISt'a. " , .",.... ..

made by osteoid. • Rb suppressor gene relationship . ..•. . ... ~ of bone

X-r2Y findi.ngs- inEwing's sa.rc;om.: onion skinning around bone shaft

:YOUD'g 'wo'm:an with eprsodic be,ma.tu·rb 'aite,r u. '."pn..w:" 't', -.tp·I....._.'.Q . .- ry" .;Iiioiio .. ....."..; .. ". _ . .

. .. .............~, .:.. D.ll"Y'L,-,OQ and 'D:"" tiv s·"

tit'er,; • classic h istorv for 19A..: :.' g·lornerulone.p"hri·' .,' n"' . (:Ber'- < .. . ,1' )'" '.. "M' ' ~ .' "'. " ".: ' eg~' rve A.' 0

. '. ."J.,. - " . . .'. '.' ' s. '. ger.s. I • . "c· type of.,n_.10 ........ .,....... .. I('ft'Vllnbri .. , _. "

R ;0, •• • [. d n - ~ . . b". 6..4,~1 \.lJ -- ---r. . tl.s

etmorc .aIel.': n.A lor acute prog'ran:ulocwic .ltuke'mia 1:l\A"'~). _. • .':1:' •. _-" " id , ..,.' '._.

J. - \ 1, .... .&..J .. re:u...L rv! C ac:t UlCTe3 scs

d~fferenna~.o~nfleukemic ed1:, ~ 1; 15:17 transloeanen, • lots-of Aua rod$,. ole alwa ...•..... __ - .

F _.. _. 'ID' worl'~' . p of oi!Ii n ern b I bl' d r·' ys present

tr ~ .. ,3..eP • ...~u ... rre., en.. 00 smear o DliCfOcytic: anemia: ardel'serumfc::rtltin

B·s.rbltD rare IQ,d'DCIOg-, acute po rp.' :; .. h vrrc a:ttack • [~J""U·· '1- 1- n: terrmttem porp '·h: \I'T:1-.~ •.. AD'" . d _'.. . -, "th.,

. - . .. . c;u,;. . - II-. . . ' " ,,"'_ ill rr - . .' J .''''_'' . ..".! cisease "W1: .

absent.uroporphyrinegen synthase, • belly full of, scars' .

!\fo~~r witb ·msculopapub.r rash dUring pregu.aQcyud DeGnate witb saber sh.ins: congenital syphilis

Hemorrhagic Infarct of brain in patient With atri21 fibri1l2tion: embolic infarct

Patient ~ith twitdiin.g of face with tapping. of fadal nerve: • tetany dueto decrease in ionized calcium= threshold potential is lowered so lllUSClcJnc::rve are partially dqmlhiZat. • Cbvosteko-s sign. • TroUSSC3u's sign is when thumb adducts .intopalm·when taking blood pressure

Elderly patient . does n~t develop a .hemolytic transfusion reaction when giVen wrong ABO blood gTon,p: elderlypatients normally I"oseiso'"hemagglutinins with age. sonone maybe present to

1 • .-,:°0 ' " .... d'bl ....... ,......,~

attac·~ ... ~ . .- 31'iD2enS m transrusee .'··'·VuU

~ . .

:Screen for autotmmune disease in· relatives: SCrc:en for HLA antigens unique to the autoimmune disease .orserurn ANA 'depending on the 'Wa,yr the. question Is worded ~

Source of ::.egicr.;;e1hz Infections; • air conditioning cooling towers/condensers. • showers, " \~ge..table ~:~t-=r.s in gracey-v stores~ • 'produces interstitial nephritis with type rV~"renal tubular acidosis: destruction of JG' apparatus with hyporeninemia and hypoald,ost;enJ.n;ism

Patient 'taking penicillin for streptococcal infection develops be,mol)n.c, 2D,emia-: due to .autoimmune hemolytic anemia. (type IT) againstBf'O attached to RB.C' membrane

Epidural hematoma; • fracture of temporeparietal bone andrupture of middle meningeal artery Malab.sotption ~~itb, blunt villi: • celiac disease, .• order anti-gliadin OF endomysial antibodies [J~K disease from brain instrument treated. with formaldehyde: prions are the. infective agent:

"':B· . .' di .. · 'b . . n" h" ..... ' ··h'.. . ' .. ''- ,~.

, rOQ2e,' a. -etes t. -e , ernocnrcrnatosis

40 IT .. al'd ma,D with dementia and 'senile plaques in brain: [)Q\\Tl syndrome patient' with

~ .

Alzheimer's

Easy bruising in, hospitalized patient on autibiotics: vitamin K, deficiency from destruction of colon bacteria b)' the .annbionc

Pancreatic tumor associated with peptic ulcers: ZE syndrome with .secretion of gastrin Mu.ltinucleated giant cell associated withvir'al infection: rneas 1 es- Warthia-Finketdey.giem.cell Mechanism 0'( cerebral edema in Pb poisoning: • increase in &-'amiIloleyulinic acid in brain is roxie and produces demyelination and 'increased vesselpermeability • ca·n be prevented by t3king-

.heme, which inhibits J\LA synthase

Alcoholic with bad breatb and cavitary [uag lesion.: • lung abscess from aspiration of oropharyngeal materia] I .• ' mixed aerobes and anaerobes

M:aiD; s~eror acetamiaepheu toxicity in liver:-. around terminal hepatic venule (ccntrilobular}least amount t';'J f oxygen in this site (zone Ill], -also the site for fany change in alcoholic or .shock Block leIt renal vein: • produces a left-sided varicocele, • left spermatic vein normally empnes into the left renalvein- mechanism for varicocele; • the right.spelDlati~ vem .. e~~cs inw.Ne lujory to head with polyuria: central diabetes insipidus fromtransecnon OJ pituitary stalk S.pine and ""he,Fe does. forelgn body g,Q.:'·superioc' scgma1t,-;o.fRII ..

21

'r;r-

,a-'

a-'

~

'~'

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,~ ,...,-

In a P' at,ient. '\'V''11'O is I,ving' {YO t'he, ':tiQ"bt, side 2 ,[o'rel4!'g, 'n': b··,o: d"Y' er 'as,'p';j"l~ _ .. ..I, _

' - ,_ t; -"., " " .' " '~ra :'~ mat ' .Ii', '

commonly localize to these primary sites'ill the lung: • right middleldbe, ':":'I1¥Duld 1I1O'St posterior; segment ", ' . ':. '.. " .,' '. n:ght.tipper lobe

ID~:a p 3. tlent w'h,(r ls .Ir· . tog,' on t'b,ei:t ba C:,'~'" .asp "1 rat a-I .,.,.'~t~'n, ''':'2.1' w", 0 Y' I' 'd" 'm'" '. '~I.·I:~ I' , .

' ,. '. ~,.Q.J~,,!I;:;.: ,",.' ,'os.; la;.e'-"'-I""

.segment.cftbe lUb.g::.y"right, lower lobe, sUpmor so·gJllent· "", "", ." ""Y 'OO:aJi2e,'to this

L)n..,p,b node: • S'." cells i:O follic~1e·s;, _' T c'~')ls in 'p~f()mCtl1ar area • h' istiocvr ;. ''- ... " _

,. .,' " " ',_ 't',. Y. ~J"~es In,_smUR:S

Patieat \vit~h, I(b. Increase fa amylas,e, and li,p·,asc,,: '., p· .. ati~,t, m 0" st l·'I·l ........ I>Y', an': . '-1"" 'h··.· .,"'l,,~,,' , '" ",

' '"",.... , , .'.' I\.C . . a co O'llC th

'pancreatitis •• ' cannot: be mump,s,becausc'o'flipase' '. " ," . ',.- ',' ,,', WI'" acute

'St .. a ·,gb,Q,hi. c.alcu'lu.s!' • magnesium ammonium, 'ph,o,~'hate • ureaSjIIi ur".--O'·na:th:,.og"en" 't-1:. .. -. D, .. ,

. - , " - ... ~ ~ , , ~ , " .. ,t'" _,' . " I :I·IA:;C· rroteu«

alkaline:' urine.pH '. . '!'." . - . W~''t •

Overdose of barhitu . rates: acute reSpiratory acidosis

EyeelfectS InCushlngs syndrome: ca.mnctsftom increased gl uCQCarricoids

.Flankmass in a ~b,ild: .Wilm's tumor, • associated withaniridia and hetnl'·'hvne [trio h' ~~ ,.L AD

" , -',. , , . . , ,,' '_,' , ,',' . '. .' )' ':i'y'l '~p, :Y 'In 'iwe - .'

'I)'p' ... ofWilms , < , '. " ,: r ' •. ' .

. . . . ~ . '., 1.&.J..j.

I _ .. ~ . , .... .'. ~', -' .

What makes a fabpratory test more specific?: raise the upper limit of normal Which', , .... soecifi ·'c'-ty·~,('l"'ss',·.· FP: .;. 's) an' td nositi '+'v" e'predictive vah . J ' , '," IncreaseS,

,~t""'~" "'1, . ... """". " " ': ". I"Hy 1:. '_ ~ : 'I, ~ f...l'.:' " ,,~e

Fiadipgs at 'autopsy of ~S: • hyalin.cf membranes. with massive .atelectasis,. atelectasis leads to

massive intrapulmonary s·hnntmg. .

Baby b[ee~s after ci~CUIUC~iQn on lOth day in a mother wllao. is, breast: feeding her ba,by: no

vttarnm K, In breast milk '

Abetalipoprnteinemia;-s absence of ape B leads to low CFI levels" • malabsorption. • 19W levels of all lipid fractions, .. blindness".". hemolytic anemia, • treated, with vitamin.E

Sudden unset ',Ieft flank pain, hypotensive, pldsatile mass; ruptured. abdominal aortic aneurysm Chest' P'~ alp •. ' italians, particularty when anxious: • 'mitral. valve prolapse .' mvxo ma •.. aro ]!~.;~

. ' ',. , ", 'J ......... J lI!'!Ii.. ,,", ~

degeneration-due to an, increase in. dermatan sulfate

Click murmur relations In mitralvalve prolapse:- decrease pte load causes clickand murmur to come closer to S 1~ .' anxiety .... standing up, • Valsalva, .. increase preload causes click and murmur to come closer to '52- .. lying down. ... clenching fist (increases sys,temic V3SCU[;;lf

'resistanceenddecreases cardiae output), squatting (increases systemic vascular resistance).

AOrUc stenosis; Me valvular lesion associated with- .. hemolytic. -anemia with.schistocytes, • syncope with exercise, ". angina 'with, exercise:

28-:ye.ar-.old patient has/a f'a.n;dIyhist.ory DC suddencardiac death at a youDgage~bypertr:()phic cardiomyopathy

Chambers orvessels with the highest. S~02 in 1"etralQgy of Fallot: • pulrnonary vein. .·left

'"

atnum

Chambers or vessels has the lowest S.Oz]n. a newborn with a macliiDe.ry murmur (rI>Al:.'

· _. h . of I

right atnum, • ng'·~':t: venmc .e

Shunts or groups. '0:( shunts ~a['di,(j.p·rotective' in Tetralogy :o'f F'aJJot: • patent ductusunexvaenated blood goes to PA for oxygenation in rungs ••.. atrial: septal de:ftct- stepup ef oxygen

... b' _

;. ,..'1. .';n", 'L., . , ,

In 1,I1enl7'.J: heart • . . .

Viral mvocardltls, develop s hyp ·ote:nsio'u" aeck vein distention, '3; drop 'In, bleed pressare Oil,

. •. J. • . .. • B f

iliSpirad~n,ail.dDiU·med heartseunds: pericardia 1 effusion from coxsackie m:yocarditls- .;x"O.

heart would' show' 'a lymphocytic infiltrate with, 'destruction ofmuscle

~i~ step i~ D,lall~.eJIlelit of;.t~C3r"di:d effusron: echocardiogram . ..~ •... ... Newborn babygid,. 'swelling 'Of dorsum of bands/fee'. (J;ymp~:edema), cYSt1c ~2S,S,.~"D~! (~il2ted. ,1'YiD,p:bati:tS that stretch skin and produce webbed neck): • lymphedema in.child WI.

Tumer's syndrome, • XO~ -"defects in lymphatics . f

,", ~,' , .... "';AID',:,S: " ed, ,_._ . ed '1" ~"o'D.s'o· 'n° b'~ar:d-- p_,'I_,telii.: Ka posi'ssarcoma • most eQmmo,n~slte: ,c'()'r

12, y'r' old" ' .', ' C', ,r,' , e5,I·' ... ' "' ... , ·.iIIid .. '. ",'. ,." .. _ ",,&"~,

, .. ", .. ,.~. :, .. ,' .... ,.. ., ". , , "

. " .,. . .'," .,. .. , _.." ,,--.,... . ',-

KSin GI tract, due to fellatio, .• Rx with intralesional o-interferon

I WU!: l!li$ material is copyrighted. All rigbts reserved. Edward Goljua., M.n.l.OOl

.. -

S8 yr old smoker, painless jaundice with Clay ~otored stoo.s (obstructlen to bU fl. .. '. urnbllia i~ stool),p.Ili.,nfut \,2ricOSitie&iDri?~t.lipper ~boulder area, similar lesi:Di:~e;l:t: a few weeks ago; • migratory thromoophlcbltJ.s m a patient with carcinoma of the head .f. ,. ,.&

.' h bsi · fb')-- -fl . 11- d T - ." ", 0 pancre,as

wrtn 0 struction 0, 1 e: ' OW, '. 'ca e rousseauts .Slgn . ,

Patien( with dinrhe~ and episodic flushing of tbe skin (due to suotonin) nodul··, r. . (metastasis from carcinoid tumor of terlDinal ileum), elevated urinary S-bYdr~xyi ,. d .~ ., Iv~r

acid lev,el (metabolite of nro~on~n), thick., . pb,qbe-Uke deposits fou~d 0" the tri:'::C.::~ pulmo.Dl~ valve lell~ets (c:llrcID~ld b~rt dls~e. with tricuspid insufficiency aDd p:lmOJ1k stenesls from ,(jbrot.~c effect of seroronln): 'carclnold, bean disease:

16 yr old died $u.ddenly ~b.ying basketball! • hypertrophic cardiomyopathy, • asymmetric hypertrophy of the interventn~ulat septum with conduction defects •• murmur intensity mcreases (worse) with factors. dccreasm.g prel~d (standing, Valsalva, venodilaror), • murmur intensity decreases (better) mID factors mcreasmg preload (sustained hand grip, squatting, lying down. p~

blockers) ..

Pulmonary capillary wedge pressure readings (normal < 12 mm Hg) expect in hypovolemic shock (volume depletion) versus an acute myocardial infarction involving the left ventride (Increased hydrostatic pressure in, left' heart and pulmo'u2'ry vein):'

Hypovolemic shock Acute myocardial infarction

2 (low) 30 (high)

Hypertensive l-yr old child has multiple nodular masses in the skin; small, basophilic staining cells SIOO antigen positive, primary lesion in: • adrenal medulla- neuroblastoma, • an APUD tumOr

2-yr old girl with necrotic, bloody, grape-like mass protruding frnm the vagina; biopsy reveals malignant cells with cross-strtanons; tumor stains negative for cytokeratin, factor vm related antigen, and carcinoembryonic antigen, and, positive for desmin; cancer is mest Iikely derived (tom: • muscle- embryonal rhabdomyosarcoma., • Me sarcoma in, children

6s...yr old woman with abdominal distention, prim.ary cancer most likely responsible for 'iDdurati,on in pouch of 'Douglas 0:0 rectal examinatieu: ovarian canceT- induration due to seeding by' the cancer

7,S-yr old man with point tenderness Ia the lower vertebral column, elevated serum a:lkaline phosphatase (osteoblastic metastasis), tests 0[" procedures 'would be your first step in the

evaluanon of this patient: • digital rectal exam: think, cheap, .' bone' tenderness implies stage 'IV disease and DRE should be positive

Metastatic adenocarcinoma in left supraclavicular, 'node (called Virchow's node) would most likely have its primary origin in':' stomach adenocarcinoma

'. - . .

.I S-yr old girl, sudden onset of right Inwer abdominal pain, pregnancy' test negative, cysnc

mass w.tb bone and calcifications In the right ovary: cystic teratoma

Pediatric cancers into the order' of decreasing frequency: acute lymphoblastic leukemia-s'primary eNS turnors-e Burkitt's lymphoma-s neuroblastoma

Qt-' Most ,~o:mmOD ionizing' radiation-iuduced 'C3Dc.er': acute leukemia, .

rr An, ulcerated 'lesion that develops' in a keloid secondary to a third degree burn or' an ulcer

_ . "O.Oa- loate4.u.. the orifice of .. chronically draining sinus that does Dot respond to medical

management is most likely due to; development of a squamous cell carcinoma.. ,". . .

Hematuria in a 58-yrold smoker would most Ukely be associated with which of the follOWing

groups of cancers: renal adenocarcinoma/transitional cell carcinoma of the bladder. ,. . .. ..

MO$t responsible for tbe increased' incldence of basal cell carciuoma in the United States: sun

exposure beginning' a'( an early ag,e, ,__, ~

Exudative lung reaction associated w'ith exposure to 'mold,Y bay:' Farmer's lung~ thermophilic

-

actinomycetes

23

" .. "''1'.;'<~ ,g,a 'nta(~enal I,S f;u.p'"yn.'v'U,lCU .. ~ • ~&U ~",I ''C:H;I Y:t,:'U .. L'U W"lIlTU, UilIJ. an 1\:iI'. D,: ~·DO'-' 2-"

. .... .. . "_ II:' . ~/" 1'1:.... . • .£i'. .' .

A 4· ~T' old/ ch'~d, has eaten rat polson and is' b:e,in:o.rr·haglJig~: ,. rat poison is, warfarin, which blocks all the, vitamin ·,K-dependent. factors, :. 'beth, 'PT .and P'I'T are; prolonged, '. Rx with '1M:

:;, '~'K""-'

vitammr

C,hlId·l,iv,in:g~wi·th elderly 'graD.dp,are·,n·~~ develops-a GI,.·'bleed: .child ate' grandparents warfarin

,/\ child has eaten :.-'3W ~h,~mb:urge'FS~'and now has a~.,h.:etn'olyti.c' anemia and renalIailure; • ,HUS: due: to 0,1'57:, 'H'7 serotype '()f~~, coli,» lew platelet-countand prolonged 'bleeding time ~

__ ~~~ AwomaD hasmencrrhagia, e!ilSy ~t11iSing, andepistaxis;» VWD~· prolonged bleeding timeand

-prolonged Pl,.( _. _

-~ f' bl -A....I'· di dh b-' • h b-" . ith 'h'" ,.L. t,- father" •

A man has 3: [a,~U,Y hlstory 0.-, ,$ n '~cu~'g ~_ . isorner 'W,_' ren begaa wit .. ' 15 mowers : ,"- ,.' :o~:

hemophilia. Al • maternal father transmits ~~. disease to all his datighter~s(SXR trait) who are .asymptomatic carriers •• the daughters nansmit the gene to 50% oftheirsons •• · prolonged P"IT

A 49 yr old woman wittifibromyalgia is taking NSAIDS for pain: ,. qualitative platelet defect, ,e

,prolong,ed bleeding time _, _ ... ,." ,,',

-,._.-~,'.. ,-' " h-', • ,- .' ,4 - ,t ': - ,,", .- '.', ,-,,:'t' ~d" . " ;'I!' ~- t d '-0'-' 'RB'Cs"-, '. do es no t': '(I1,'Q~inlcc SW"'Vlwl,of

MalO:£ crossmatc '_~ .' patien serum, J:cac' _e" - ,aga,ms :Q,n,_[ '. _.,._. -1' -e -r c" .' . ,c .b~'7"_ ,_. - _' _,.' ,

the infused donor 'RBCs~ .• ;does, not prevent patient anlib@i~s developing ~t dOD()r RBC

an.tigens; •• detects ,the'presence of patientantibodiesagainsl don~or RB<Cantigens

tr'

' ......... 1...,,- ."

,cr-

-' ' .... -

r;zr:r

cr'

,;:r

~' '.....,.

t _ .

Yo,n,a;g, ,iDaopreseO($ with be:moptysis. and then 'devel,op, 's' a,tu:t:;e': 'g--'l,hDl· ..... ,.,-:1 .. , _ '_. "

. " - - ., ~e. ~OD'epbnti

~pa~tureis syndrome •• ~~i-base~ent.mcmb~ne antibodies;- ~ n h~ensitivity· ; .... s: it

,H;yp· .. · ·;'erse~lisitiVilJ'· pneumeuitisthat .IS ,p~I.U1ariI:y' seen in textile, work ers ,. b~-""" .,." +.-." »s

- - . . - • '. , F • - -. ~ Y:SsmoslS

Hypersensitivity pnculno'nitisfhatr:.oD'unordy occurs i~. (arm_ers" whoe~ter a c.', cd .. .. . .....

rermen:tingcorn: silo filler's disease .... nitrogen dioxide . .. .0$.. rOom 'WIth

Pneumoeonfosis' whose m~~!2~:~dmmolJ lesion isa benign pleural plaque! llsbestosis

A pregnant woman in her jJ' D ,.' week of ,ges(atjOb, has pr:eIDatq,r.e conttactions . Wbic. .' ... ' .. (oUo;wing drug£ should she- be gixen tQ protect her baby from develoPlo·. tia· ., .~,o(the

~·:.;..,tr·· '·6.:'1C' '$ nd or' -,' - -'e-'" glucoeo _. ft'" icoids -, inc - - , " ', .. ,.i.i...-.l:' ,,-, - th' '. ~ .. c· g, - - e, res.p .• ratory

UUi, __ ::~~ .. ~ -t ' rom ... '. ".~~ ," .. U""~. - .... reese sunactant ~.'<'CS'IS, In, the, baby

,A. 3,8 .Yr old woman -wltb chranic .. headaches develops e',pisodicastbmatic 2ttaCks- T- ~'., '... .. _. 'U·k 'I" ,.' <". - - f- . - . 'oidar ar +.. . - -'_' - be, most :'I'e ~ea~se 1~~~~C'sh()n'O .non-sterordat anti-inflammatory agents with release of kukotrien~-

c ,as'~lC triad as'll~~l,@ . ".-.

Farmer and his wife a~e .broughr totbe:ER by their SOD. because they are tOG weak t- -.' ._:1'1:,.-,- , ... ,

d .. d th'" .... ' "" bl··· d .. -- ' 0 W-iU.A. or

.. ,D".e ,ao .....• ear ViSIOD, IS : u.~ry air" exam re"~Is: ptosiS?'fiadal weakness, nODrea~ti"e' dilated.

pupns,~ry muC()~s mem~?es~,2Dd,l1onnai D~-? ~llOsis: ~'C botulinum food poisoning; •. 'I) .... WIth botulism anntoxm danger "of' , - ·t - Ivsi . ~ g~

, • '~ ; ,,-',: 1- '"u:; 1,.,_: -" .. I - ~~~' ~l-·.'· - , respira '-ory' .'paraYStS~ toxin blocks the release of

acetylcholine

:oung woman witb lntermitteDtb~ut$ of, d~~rrbea andcondipationa$s6dated witb cramping raght .and I~ft lower quadrant pain; .' irritable bowel 's,yn,dro,me:_, intrinsic mq,:t~JiiY' defect in

bowel 'fl' . +b-] " ... ~ - ~..' ~ d" -' ., ~i -r:» , • 'f! "0' • v -

. ·,e,~ • '. _ ext. . , _ c: slgm,or "osc·opy, . s u· .ga, _' e

Intraveneus.drug abuser ln, prtson-« ?typ'e ~Qf hepatitis: ··HB'V Countyjail ~w'ith outbreak of hepatttis; ,HA~V mostlikely-

Hepatitis 'assnciared with urticaria, ~ev:er, arthralgias, and 'the nephrotic s;yu<lr,ome,: ._ HE'V·,:." serum sickness 'Ij'P'e of disease= type: :m .imrnunocomplex mechanism, • vasculitis .associated.with pplyarteri tis nodosa

Immmuzatlons 'giv:e<u, at birth to ". a baby whose mether is,'"positive for HBs·p._g,: • Rep 'S, vaccine-active immunization, • HBIG,-, passive, irnmunizati on

.I:D a patient-with ,Bl~·deficiency W.b,9 is ~eJ:n<g 'trf!at~«(wit'h pharmacologic doses of folate, 'which of th,e following will,'; be ~Q .. rrected: • neurologic deficits 'remain~, hence the 'lrnportalu;::e of making. {he correct diagnosis, '. megaloblastic anemia is. corrected

Ia 'a'n alcoholic with a,; macrocytic anemia and b')1lers~·,li.teB.t:ed, neutrophlls and a normal neurologic exam, which of the (p.Ilo\Yi,ng tests 'is most indicated: RBC:~ folate is ·more sensitive than. serum folate

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A pb!eooto mist inadveHe'utly sth:kS binls.elf witll ~ lieN'e- ar~l!r drawing blood f.··· .. . ~ .'r

with AIDS. \Vhicb of~be _followin~_ infections is the phltbot~.mi~~ 2t most risk ro;~:a~:~:~

1. :3'00 chance of becoming HI\' pos!.nve . g

A 'p: 'bleb.o'to.mis,t Inadvertently .sticks hir:gs'elf' w'iJ,h' a beedle" .af'l·er ·d·' raw ··."DO' 'b' 10' o'd'; f' .. , . .,;. 'II!

. " - r; '. . '. .. . 4' .. 'bi ',",' .' ·rom a patleDL

Wbi~h of th~ f~nowi~ginrectio~s is .tbepblebotomist at most rn1c. fOf eOlltnctiog:.ltBv has

the greatest viral load m blood of a11 viruses, ~ y<:>u. cannot get SYPhilis from transfused blood

Most .commoi1antib~d.y .. ~n(:ou~tered mtUaical prattice~ if. is almost impossible to 'fmd blooq

that is negative for .antibodies against 'CM¥: ,. . '., .. '

Bemonbqic skin ilecrosisass~ci_aled witll_ w~rfa.riD thenpy: heterozygote: carriers for protein C become homozygote .wh!!l1 given Wtuf3.rin _~. -.&.8 hrs when the half-1if~ of previQllsly .~

carboxylated protem C dlsappears- now the, panent is hypen::03g}.llaote "t

A~atie~t, n:bo .b:as £h~ODi(' bepa~tis .Ddhasb~n transfused ip: the past most likely has antibodies ,dlr«ted, .l.g31Dst:,_HCV IS the ,MCC o·f.tr3I;lsfUs-ion hepatitis

A 2S yr old woman b·as u.anterior m~a.l mass and .aoa...:teader lympb2d~Dopathy in th~ ·rig.lll ,sopnlpa,i(ular node: nodular scl.e.roSin'g HQ<;lgkin1s ·discitSe

An. afebrile 72. year old ~ m .. n ~th 'non-tf!uder; geB~ Iymphadeaopathy, hepatosplenomegaly, normocytrc anemia, thrombocytopenia. and bypogammaglobulinerma

most Jikel~' h:as: ~ eLL 1S a B tell malignancy, • neoplastic B. cells cannot transforndnto plasma cells

Rationale for b'egill:D"i~g heparin and warfarln a"( the same time: _ the shortest. -are factor 'V"U and protem C4I)d the longest is prothrombin, • previously activated vitamin K....:dependent factors 'have Iong half-lives

A -15 'year Q:1.d WOIQ.an in. a nursing 'home "has non-palpable ecchymoses li~Diit'ed to the back of

her hands, A CBC"is unremarkable, The patient most likely has; senile PUIp1m3 .

Mo,st common type of hereditary thrombosis disorder: factor V Leideri- cannot be, degraded ·b,.y protein 'C and .S

M()st. com mOB Infection 'trnl)s,mi.ft·ed, ~,y a blo-o'dt("ansfu'sioo' is due to: • 'CMV' ~'S present 'in donor lymphocytes; .' radiation of the" 'blood. kills the lymphocyt~" and prevents infection in the recipient, '. bone marrow transplant patients are particularly' prone to CMV infections in me lungs

- ... .

'Wh:ich. of th.e rou·Q~~~~.n·g vitamin toxicities ptedispoSes, 'a. patient .who is: "~'Dg" Wa.rfariillo,:'bl,ee(f

.and have an ll\tR··. outside the normal r,ange: vitamin E, toxicity decreases the. synthesis of vitamin K:.·de:pend.enl factors-in theliver

A, ·,,~fi.gbtlir~e:T develops a sudden onsetof abdominal pain alQ~g with ·~,)llovo.letiJj·c shock, ,A'C s'urgery, his abdominal cavity Isfilled up wi.th,b~.ood. Til·e· ~aDseo(the.iDtrU.b·do,mirial bleed is most Ilkelyassoeiated with: anabolic steroids cause: liver cell adenomas which bavea tendency to bleed

A 30 "ear old man With 3.tQ u:i red immuDpdei'icieoq' syndrome has pittiDg edema, hypertension, protelauria :> 3_5 g!Z4 hrs, 3:D,d fatty CaStS in the uri-ne. A rCllalbiopsy e:a:bibits glomerular disease: focal segmental glomerulosclerosis .. .. A ,2,-9-yea:r-old woman presents with g .. eneralized pitting edema and byperteasi,o.D... A re·aaI biopsy reveals dl"fIllse glomeruh-r disease with Increased thiekn~ or basement membr1la.es and hypercellularity, Silver stains ·r~:"eal "tram. trac.k"- ,spli·ttiDg of"th'e ba'semeD~: membranes.. An electrna micrograph e.#a.ibits "dense deposits" in. the g!fj'm'e'rular basement membranes, The patient has a persistentlyIew serum 0: • rype IT MPGtf or "dense deposit disease", • patients have C3 nephritic factor- autoantibody against C3 convertase in alternative ,s:ysle.m 'that causes it to continually activate··;C.3 causing very low levels

A 2$-year-(Jld maa,who io.itially presented to the bospital with Q~moptys~ bas pn'tgl'essea iato r,t,D.a), fallure, A renal biopsy shows linear ·i;mmuiloRlioresc::e·D.ce..:. Goodpasture's syndromepatient has rapidly progressive crescentic glomerulonephritis

2_5

A 7'4-ye'ar-old man, ·with (:010.0 cancer d.:,evelops generalized anU'ar'CL, 'He has pr.o,r",,';liiIi;c : .',_' ._: .. -,

;0; h I' .. A' I bl einnrra > 3, 5

'g/24 hr and fatty casts 10 ,C-1Sb·· urme, .. ~~naIOpsy shows diffuse glomerula.- disease. Silv;.1'

5[1· ains demonstrate ePd~:~e": m.reamnob· ·':UOSPU'IS-' '·GesN··· ... and the presence ofs'ilbepitbelial deposits OD

electron microscopy: . '1 '; '., ,. : ..• '.. . '. ' '

A 10 YT old boy living fn Salt Lake City, Utah has bilateral sensorlDebl'1l1 hearing I. . 1 .

abnormalities, and glomerulonephritis. Both IF. and EM are negative.. Foam ceUsa';;S~:~~~: visceral epithelial cells: Alpert's syndrome- sex-linked, dominant. disease '

A 65 )'r old D),an with chronic.HC's" hepati~ bas a h~tory o-f cyanosis oIb1s hands, tip of nose, aud ears dun,ng cold weather. These rmdrogs SQb~ldewhen be comes indoors. He now bas palpable purpura in cold-exposed areas: czyoglol;:Jullnemia

A 12 year old bas a. history of cellulit~ -2 we~ks ag,o. H~DOW presents with periorbital edema, hypertension, and smoky-colored atone. Urmalysis shows RBe casts with mild proteinu.ria ~1.5 g12,4 ~)_ ~en~d biopsy reveals a_ diffusei~cre·~se in cellularity along with a neutrophilic Inflltrate. IFSh?w~grall,uI2r.depos~ts, Subepithelial ,deposits are noted on EM: acute post-

sncp~oc_occa1. GN- immunologic reaction secondary to either a group. A stleptococcus pharyngitis.

o'r skin infection ..

A 62:-ye.ar-ol'd woman with a long-history of severe rheumatoid arthritis, and restrictive cardiomyopathy D{)W presents with pitting edema and hypercholesterolemia. Urinalysis shows. oval fat bodies and fatty casts. Protein dipstick is '2'+7 sulfosalicylic acid 2+ .. Renal biopsy shows hyalinization 'of the glomerular mesangium. A special stain is ordered: systemic amyloidosis

.4..8 8·9~ye3.r-old woman with 'a history of chronic 'H~CV hepatitis froma blood 'tranS:fUI'iOD, ,DQW presents with. 'geDe,ralized puffiness and mild hype,rt.e'nsion., Urinalysis show's oval '[at bodies, fatty casts with, Maltese crosses, and 4+ protein (>' 33 g pr,otein124 hr), A, renal biopsyreveals hypercellular glomeruli with an increasein basement membrane thickness 'and tram-track splitting of the basement membranes. 'The IF is granular ·an.d, ,E'M r'eveals subendothelial deposits. The patient 'has, depressed levels, of C3: 'type, I MPGN with, HCV association

,A ,24 'year old, mag with a pri:o,r history of an upper respir-atnry i!_lfectioEl 'is noted to cave, microscopic hematuria and mild proteinuria (1.5 g/24 hrs] durIng 3 routine physical exam, He is, not: hypertensive, A renal biopsy shows ,3, g:raDular IF with predominantly IgA deposition in the mesangium and electron dense depesits in the same area: 19A glomerulonephritis ' A 48-y'e',ar'-old woman has proteinuria (> ,3.5 :gm12,4 hrs), hypertension, an,d fatty: casts ill the urine, ,A renal 'biopsy reveals eosinophilic nodular masses 'in the mesangium of the glomeruli, hyaline arteriolosclerosis of the afferent and efferent arterioles, and Increased thlckness of the basement membranes of the tubules .. , IF Is negative, EM reveals fusion. of the' podocytes and Increased collagen deposition in the. mesangium and basement membranes: nodular glomerulosclerosis, in diabetes mellitus

A '28 yr old man, witb ,3, history of renal disease in his maternal grandfather, has end .... stage renal' disease requiring dialysis .. , Physical exam reveals, papular red Iesieus OD, his skin, peripheral neuropathy, and corneal disease, Previous ,re"oal. biopsies exhlblted vacuolated visceral epithelial cells which correspond, with lamellar bodies OD EM:' Fabry's disease- S,J{R 'disease With deficiency of cc-galactocerebrosidase A and accumulation of ceramide trihexoside

A, nermotenslve 10. yr old boy', witb a previous history o'f an u'pper' res'pi,~,toryinfecti.oD" presents with generalized pitting edema, He has had. problems with allergies siBc~ ear~y childhood. Urioalysis reveals proteinuria (> 3.5g/24 hrs), fatty casts, and oval fat bodies. His

clinical findiags Improve rapidly with high dose corticosreroids: lipoid nephrosis .

A 25 yr old woman presents with hypertension, A urinalysiS reveals mild. protei~uri~ hemarurla, and, RBC casts. A, serum ANA exhibits, a 'rim pattern, A.n anti,-dsDNA nter 15

extremely hlgh: type IV SLE, glomerulonephritis

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A·,:c"bild with Turner's s~'Q~d.r,om·e is noted to h.v~ ~ abD,o.rll1aI ').;.i,.doe'U ou ,renal' ultr " '. , d 'ot

~ . . - '1 fu . .' . 'k -h" - J.. . &so U. b ',. •

horseshoe kidney, • lower po e sion; ~ be :lnd'the in:f¢tior mc:senteri.C .arteri,es,

:Paiieft'. ""'.it,b "'\~e~encr's gra'Duiomatoisis de.\~e·lo'PS" bem.aturi~ and a'a abn'f.u;,ma·' u,riDe 'cyt'ol'" ',,'

. 1 - .. .ogy.

• ~siti\'.ma]ceL carcmorna, • patient is on cyclophosphamide. which produces hcmolTba' c

cvstitrs an d T C'C' !1:

.. - -

. .\n ~~·pti.aD ~allbas mj~rosCopic beDlatu_ri~~nd2.~ abnor,mat,ul'ioe cytology: squamous tell carcmoma: p.a.n~n has Schistosoma .. ~em:'QlobIUm '~n'vOIV1,ng: the: bladder plexus

An unclrcumcised male bas an ulcerative lesion on the u Dd.ersor(a~e '(If the glans .'pellis. and palpable ing.u~n21 adenopathy: • squamous. cell. carcinoma (penis) 1 ~ lack or circumcision, is.

gtt3'tcst risk factor .

~, man, 'W:,lfO:' b'as w '0 r ked' witb :a;·nillne' .~yes. i'n the past, develops mic·r'oscopic, b,em.-aturia and au abnormal u"rine 'c~"t.oJag}·': • transitional ceil.carcinoma. • most .likely it is. bladder, but it could, 'be

_ ...... 1 ~lV1.· is a,~' 'we:)'l', .

I ;~;;;tJlS_l, r'lllr. ,~ "' .. _ J

A 45 )T old, woman ,,<pith, a Ioug-hlstory of headaches develops toicroscop.ie beDUitllria and an abno"rm:al urioe cytolo:rot: • transitional cell carcinoma, :.: analgesic 3Q\lSe' predisposes to Tee . A '72 ~T 'Old man has bilatera,I painless masses in his testicles: • mal.ignant j·ympboma.! metastatic, not primary, .• note the' 'bilaterality

.4. p,~tjellt with acute myelogenous leukemia developed acute rena) failure after being .aggresslvely treated 'With multiple c:hemo,tberapy agents: • uric acid crystals, • example of' tumor lysis syndrome

Crysts (.2SS0 elated wlth ethylene g:fycol paisonlng: calcium oxalate

-lllbo~rD error of metabohsm associated with 'reual stones and .be'ugonal crystals i·u the urme: cySrli1t- cystinuria

Compllc2.,(i~,J1 .assuclated with sickle ,~eII t·r;-ai:t acute pyetenephrms, a.D21gesic' abuse; and, diabetes ,lQ~.t.litu,s: rena] papil lary necrosis

Depression related 'lo' <a \i6ulliQ deflclency: niacin, defi¢'j,en~y- correlation with decreased tryptop han conversion .into -seroton in.

Vitamin difference between. ovolactcvegetarian 2D;d a, pure ~"ega'n: pure' vegan lacks ,Bt~ .• while o volacto .does not

'F armer' rubs Iesi 011 o,(r from the back 0".,( his neck but it gre.w back: .actinic (solar) keratosis Iaborn error Qf"'metabolistn, associated with, pellagra; Hartnup's disease- .. loss of neutral amine' acids in 'the 01 and. G'U' tract, • Joss. of tryptophan leads to pellagra due to decrease .: in, synthesis .of

,. .

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MCC ufIacreased plasma h.OJ;D~,cys.(.eiDe in United 'States: folate deficiency

Patient t.akin'g dapsone, .d,evt.l.o.p.s. c.y,=mosiS' resistaa-(, to' '01 tberapy: patient has" methemeglobinernia ,d:u~ to oxidation of iron to ferric-state

Di(fe.rep(e of' p' •. and J3~ th:'lassemia': fi"" synthesizes some () chains, 'while' po does not 'wthcsizc any i3 chains due [0 a stcp-coden

':Sequence' In bypoxi'c' eel! tUj'Ut)I': '. hypoxia-e. J. oxidative: phosphorylanor; in mitocnondria Ieading to,~· J.. ATP-4·:J. ,ATP Ieads.to: '. t anaerobic glycolysis (J" .. intracellular 'pH' from lactic acid, J. gIycogcn)jf '. dysfunction of Na~IK,· ATP,ase pump {reversible. cellular .,~welling),,_. ribosomes

'detach from ,RER .(~, protein synthesis, fatty change] -+ I. ~:treversibl"e c~!l mctBbrane: in.jwy: '. intracellular release of Iysoscmal enzymes damages membrane. '. endogenous activation of phospholipases (iinfluxofO!:- into cytosol) with release of toxic lipid prodiJ~ts •. ~ cytoskeletal alterations (activationof proteases by ca .... }, 2. irreversibtenucilear changes: • ic.ttvatlOn of n~clear enzymes by' Can, ... nuclearpyknosis and lysis) -t 3~ :Weversib1e ~!1Xhondria~ dys~enon: . • entry of C.a-..l 'into mitochondria with acrivation 'of pho~holi'pases causing, destruction of inner and

outer membrane, '.' Ca - produces large densiaes ~ . i' . _',

SLE g,ltlmerulo'Dep,britis: antibody excess' immun,e,·d'cposits combmed, with 18;9

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,AfriCo-n, American ~oC),k,prim*q':une ,and ,tileD devetoped .an ~Demia with: blt:~~~;~ de ficiency

,P,al·ie'D,t with Intense ~o'cc.ipi,tal headache and blood in ·CSF,: '-P, 'To·ba·bl;.. s:-uL.';';;;",,_,"hno'l"d ble d .', ,

"~ , ' ". "'" .uara"""., '.', .'ee. In '3

pan:ent with.a ruptured congenital berry-aneurysm . '.' ".

Se'ptic. aJthr:iti's'.:io y·oung. ad'u]t:,: Neisseria' gonorrhoeae

.~Cbi.td ,witb. disease cb:2'ra(!leriZe,d 'by 'b,eulr'o'p,biis. t'bal,c.a:,Q,Do't kill b·alCteti~,. co u'J'd ''L_ ,L.,_. -,

.. ' h ' ,~, • • • '. ,. . ": ,'_,,i; '. " .. uc::;. C:I:U on] c

granulomatous disease ofchildhood Of pl~yeloP:tl'o,xidas:e, deficiency , '.

Throm'~otic;: :t~t0m.bI)C~C;:, purp,ura: • '. pl~tc:ltr thrombi (not DIC)~ • CNS' hemorrhage •• bemolync anemia with Sch~st~~yt~,. r~i (~~~ure! • throm~ytopenia ~C(lnsumed in thrombi) 'CSF'a:DalysiS in ADJS pat~eDI with me,?D;gl~~"h~wed an,encapsulated ot:ganism: ~tqcQcc\l$

Calcillate'anion gap: • AG~ serum Na - (serurnCl' + serum IiCOJ') •• increased AG (add-acid) due 'to- lactate. salicylate!. P:~HB. AcAc:~ .oxalate (ethylen~ glycol), formate (methyl alcohol), phosphate/Sulfate (renal failure), .• ' normal AG (lose biearbcnate) due t'b- diarrhea, renal tubular

ld ~"~ximaI' d' distal)' , '.'

a'C),. ',0S1 s U'~'u·...· . '8Jl ' •. '.., , .

Orpnism csnslng ,pye'loliephrim:= .' ,E~ coli, _. ascending.infection d'~e to. yesic'o'Ufeteta},'re,flllx, Febrile- "·,om·an 'with .:O'OD radia,ting: ·flauk. 'pain 0:0. 'ri,g,b,t Side.. Wb:a,l would be' 'pres.eat· j·D. ,Iier' QriDe:?~ \V'B:C casts (pyelonephritis)

. ~

WOPLaD with painless nodule lateral to cricoid.,cartiIage: probable papillary cancer of thyroid

ME~ I,: • pituitary rumor, ' •. parathyroid adenoma, • pancreatic 'tupl9r'(.us~1Iy ,.ZE.), :. peptic ulcer (due to ZE and' hypercalcemia from nypeI-parathyrQidis:To)-

MEN IIa:. • medullarycarcinoma thyrcid, • b~ar;1thymiJ:lism, • pheochromocytoma

:ME'N' Ifb: .'~ medutlary carcinoma, '~., pheochromocytoma, • ~m,ut<?sal neuromas .

'C2I·cltomn is marker 'fo'r' medullary carcinoma 01. thyrQid': calcitenin lowers calcium ··'by :in1libiJing'. os teoclasts

,Hyp.er~~.e:~Dia in, ,a patient-with IVJEN>s·YD:.,dr~,Dl'e: .hyperparath)Toidism with, increased PTE '(:O'D"D'S (prim,a'r}'" aldosteronism) syndrome: • key findings.:-' -.: bypet:lension; .. no pi'tting, edema, •.. b~tre.mi.a. (mild at u;pper Iimit normalj;> hypokalemia, * metabolic. alkalosis Hypoaldo:s(,ero:oi.sm, effects (spironolactone, Ad.dls'~'D's~, destrucrien '0'( J'(; apparatns): .• ' hypovolemia from-salt loss, .·,·h.yper;kal~~t. • normal AG metabolic .acidosis

.Mutation producing neu r oli'bromatosis~ inactivation of'NF suppressor gene:

8.epatic encephalopatby with flapping 'tremors: .. relates to false neurotransmitters and increase in' ammonia. from bacterial degradation of urea in the' mtestine.

Sexually active ~an witb sterilepyaria and 110 nrganisms seen on gr4\m stain:' pro,bably Chlamydia·'lrachafTUll:i.s non-spec'} fie urethritis

Man with pain in posterior aspect 'O( t~(t testicle: • epididymitis, • depending on ag~ or the patient organism could'be NeisserialChlanrytita if"< .. 3S,·or E.,~coli/Rs,e:udomonas.·a;etufil.osa. if'> .35 Y,Q:ung' woman witli '3 .stroke. and irreg_ular left ventricle ftillng,: embolization: from atria]

'myxoma

<T I.adY 'tbat jUst returned frobl A:si2 has severe dyspnea 'withl11 .ad,ay of anival: probable

pulmonary embolus from, sitting too 10'D,$:' .

a-'. Cysticb,ygroma, io., the neck .area Ia a fetus:' • TUrners syndrome; .' I,yl.uphati~: defects ,ar~ common- cystic, hygroma becomes webbed neck, .··lymphedema. of~d~rsurn'~':[hand5 ~d, feet,

'cT' Tumor of 4~' ventricle: '. ependymoma+neoplastic ependymal cells, .~usua.tly}n children

'7" Ascites '" a .. 'pali,e.nl ~with, 'a, D,Or.mai Iiver biop$)~,: 'portal vein thiOmbo'sis, .Ieadin,g to 'portal

hyperre nsi on ""

<r EI,d,e;rj:y wqman, 'wi.tb 2 days,' of eye pain, Hod dilated, vessels OR whit'c of the ,eye ~.hali dO.II'I,

blanch' with vasoceastrtcting dnags':' probable glaucoma

<7; A,(roph,y ar·,tail of ,caudate nucleas: Huntin,gton's disease

(7' Vita'~iQ D~ excess: • 'hypercalcemia, '. 'uriJ!ary' stones

Vitamin C excess: calcium oxalate stones

Paget'~ disease of bone in an eld~rly wom~b: • early p~.of1Y$is (resembles multiple myeloma except H does not have clear margins) and then bone deposItion with soft mosaic bone (Pth I." fractures), • increased 'serum alkaline phosphatase .... .' . a 0 Ogt~

MAD witb se'\·ere~yponatremi.3_ ~nd hi~h urine osmolality: '" inappropriate ADH syndrome from small cell cancer In the. lun~l .. hIgh. unne osmolality is due to increased pcritubular hydrostatic pressure and loss of sodiumin the. nrmeas well as constant concentration of the urine from excess AO'H .

Factitious in$~lin ~jection: .-. ~glycemia, - high serum insulin,. low C-peptide (suppressed

by hypoglycemia), • If Cspeptide 'IS mcreased, 'patient. has insulinoma

Type 1 )'S type n:· type I. has ~nsulitis due to autoimmune destruction .. type IT has amyloid in lsle~. • ~ I has HLA relatlon.shIP: type n has f~iJy history.- type I has antibodies against islets ~d '. ~sul1n, ~ D .~ no antlbod~es, .~ type r IS complete 'insulin deficiency, type n is relative insulin deficiency with decreased insulin receptors (increased plasma insulin) and postreceptor defects

Post-transfuston hepatltls: HCV

Pulmonary fibrosis with pulmon .. ary artery' hypertension: • could be. due 'to drugs (amiodarone, bleomycin .. busulfan, methotrexate), • Harnrnan'-Ricb lung from alveolitis syndromes

AIDS patient with purple lesions on skin: Kaposi's sarcoma due: to Herpesvirus 8

C2Se study of child with medulloblast.oma: malignant cerebellar tumor

.Alzheimer"s disease: • remember Do·WJ1 'syndrome relationship and chromosome 21: 40 yr old with ,t\D is an adult with DOVinIS syndrome, • senile plaques, = loss of 'higher intellectual function

M'a,n with extrapyramidal signs: Parkinson's disease due to loss ofdopamine

Association of polyarterttis nodosa with HBs.Ag

C:3.$e study Of a woman with gonorrhea: probable P'ID shortly after menses Anorexic patient bas more 'risk for osteoporosis due to loss ofestrogen

Left sided heart murmurs and. abnormal heart sounds increase In expiration Right sided heart murmurs and abnormal heart sounds Increase iu i~s.piratioD

Aortic stenosis: • systolic ejection murmur with radiation into carotids (S4 and increases on expiration), '. diminished pulse .• ,MCC is congenital bicuspid aortic valve, • .MCC· of' microangiopathic hemolytic 'anemia with schistocytes, •. Me' valvular lesion associated with syncope and angina

Description of an x-ray of emphysema: • increased A-P diameter, • depressed diaphragms, • vertically oriented heart

Tension pneumothorax, spontaneous pneumothorax, and atelectasis: • spoataneoussubpleural' blebs in tall, slender males is a common cause of spontaneous pneumothorax, pleural 'pressure same as atmospheric pressure, lung collapses, diaphragm rises .. trachea shifts to side of collapse, decreased breath. sounds, tympany to percussion, • tension. pneumothorax- tear in pleura 'acts like check valve) pleural pressure higher than. atmospheric, lung compressed and. not collapsed, 'mediastinal structures shift to opposite side, diaphragms low, decreased breath sounds, t:ympany to percussion, • atelectasis. (collapse 0'[ alveolij- decreased, percussion, high diaphragm, increased. tactile frenums, decreased breath sounds, inspiratory lag, trachea shift to side. of atelectasis Chronic alcohol abuse leads to congestive cardiomyopathy and cardiac failure: could be due to

thiamine deficiency or direct toxic effect on the heart. . . .

Type n ·pneum.oc.)1.es are the repair cells of the lung in pneumonia and ARDS: lamellar bodies

(surfactant) are cytoplasmic markers of the cell

Dr'llg addict wit,h fever' and pansystelic mu.rmur increasing O'D Inspiratien; tricuspid,

regurgitation due to S. aureus infective endocarditis.

Type '2 diabetic: with 0.0 diet central: Hgb Ale is high

29

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Polycystic o\'~r.an ~ynd"Dme:. 17.;ketosteroids (DHEA and andr,Ostenedione)~ testOstetone,' ,d f

estrone ate elevated, ". LH.> FSH . ~·an.,

BeD2'~~e: causes aplastic anemia and acute 'leuKemia r

Testicular torsion: testisraised on affectooside arid toss Qf:cretnasteric reflex I

Clinlcal case-of ulcerative 'colitis in youo,g m.'Q:.bloOOy··diarrhea ..

·C .. -.lin"ical- case of 2.'D .elderty man wi(ti 'pr'osta.;te cane er: • rem em , be 'r- osre obl as' .,..;, 1,'

. ' . - . " " :,' - · . . ,'., ... ,. '.:. " , '-;', ",~ . , .... C: metastasis i:

I~w~ l~~ vertebra, .. If the ~lStOry IS only.one of urinary retention and problems withettin " "n at' night, l.t is prostate hyperplasia and not cancer :$ .. g. up

'21~HvdroiYl:ase ,deficieD.CY~,. salt loser, .," increased 17~KS and decvrcased 17'~h-"ydro:' ""r\1' 'm-"" ids

'" ". . . ",.,' :..', .~,Jco : COl· .•

increased ACfH~.· female pseudoh~pbrQditism . ~" - . .'1'

~ 1- Hydroxylase dencie~cy.:. hypertrnsion-. increased deoxyconicos!erone •• inclttSed 17-KS.

Increased, '17":hy'droxy,-cort1'C:Q~ds, (,ll.;;(},eoxY,;cortt,rol)'., • increased A"··r-rt:J' '. d:'~,ft.-....Jc:-,.."....,_ -, ,',.' 1

. .' , hro ~ . .: . .. , , , ~. J.. ,~ .._, '.' '~'" 1,;,,;CL~ ~'" Ulil C OTt1.S0 "'Ii

.•. female, pseudohermap odmsm

11- Hydroxylase deficiency; • hypertension- incr:easedmih.ex-aloco1"tic:oi~ •• decreased t7~K.s, • decreased 17-hydroxycorticoids •. • increased ACTII, • decreased serum cortisel •• f6t~le hypogonadism, • male pseudohermaphrodite

Cardiac tamponade occurs :3,~ 7' days after an ,AMI.

Pr;egu.ant patient acctdentally swallowed 11J,: baby Will probably develop hypothyroidism Duchenne's muscular dystroph)+:"S~XR disorder With absence "of'dySl:rophin

PGEI synthesized in the placenta keeps the ductas arteriosus -open during pr-egD2nC)'

In a patient with acute nephritis, 'what lab test should be ordered: ASO titels to lVO PO$t. streptococcal glomerulonephritis

Re'D2l Casts and 'their .disorders: ; .• ' RBC, casts...... nephritic syndrome" .i \VEe 'Cast$-: acute pyelonephritis and drug induced tubulointersritial nephritis, • 'renal tubular casts- acute tI,lbular necrosis, •. fa~ casts~, nephrotic syndrome, .'. \V8Xy casts- chronic 'renal .failbte

Teratomas: '. gel in cell origin, • located, .in midlinc-- test~o,vaties~ anterior mediastinum, pineal 'G12ndular metaplasia of distal esophagus: Barrett's esophagus in GEm

,~was'h;iorkor: '. 'has decreased oncoric 'pressure due "tq decreased protein, intake, but ad.equa:te .number of calories, '. defects in, cellular immunity ~ .:.' anemia, _. ascites, • fany liver from decreased . apoprote ins ~

Marasmus: • total calorie deprivation, _. broomstickextremiries

Mycoplasma pneumoniae is the Me organism for aQlllcal pneumoliia i'a, adults .. Neurrophils phagocytose mouosodium urate. crystals '. ·io. g~'Dt

Pancreas biopsy in ,cys'dc ·fi.b'rosis' patient: atrophy is the growth altera .. rion Epidural hematoma: ruptured middle meningeal artery

Rap.i4ly pregressive d,eme,ntia ,w'ith neuroflbrillary ~,~,gles: CreutzfeldtJacob Defecrtvesyathesls oftype I collagen iQ, Ehlers-Danlos .. syndrome

Auer rods: • only' ~en in acute myelogenous leukemia (includes progranulocytie), .: 'not: seen .in

any chronic leukemia (includes CML) 0'1' monocytic leukemia _

Case of extravascular hemolytic anemia, Hypersensldvity 'is, e'quivale,n:t to: ERA is. a ~' II

rea cri on " solook for 'another rype:fi ,reactio,o- e.g ... .Goodpasture's Graves, myasthenia

Primary hyperparathyrcldlsm electrolytes: • hypercalcemia, • hypophosphatemia, '. normal ,AG

metabolic acidosis ..

Cue of hemolytic uremic syndrome: • schistocytes, • D-dimers (fibrin .strands with.links between them), • thrombocytopenia- platelets consumed, • increased bleeding time, .• , normal PT and PTT,-, n,otDIC

Common C;o'lQpliQition of severe hemophilia ,A: hemarthroses. .

;lOia,efelter syndrome: • 47 XXY~ '. low testosterone'. '. high 'FS:H and :LH, - hi:~ estrogen Macrocytic~ anemia ·with neurelogtc sy'mptoms.~ cobalamin deficiency (B'll)

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Mesothelioma: • asbestosexposure (:shit:md• roofer >20~), • no smoking rebttionshi Tem~oul a:rteri~is: ·0. ~ulomatoU$ gient cell·arteritis, - ineteased sed rate, • jaw :am when chewing, • potential blindness

,Ptoximal dtssecttegaortic ,aneu'ry~m: • aortic regurgitation'mwmur, • w1d~;·ng. ,of' Q~:c"'kn'" '0" b' . '.

. .' . I .. 'tw!,......, .', '!f&~'1 U. ' " ,'.' , .1

loss 0 f pulse, • potential for tamponade .. . .':; 'I

RypopbYS:KI(lmy: zona fascicufata under:goes: atrophy' due to loss of',ACTH

Case, oJ emp~yseDla .. ad .pathol(lgy; • .destruction of elastic tissue support in TCSpiratory unit, • centn lobular .Invo.~ves respiratory b~chroles ••. panacinar involves respiratory bronchicle, alveolar duct, and alveoli, • paraseptal involves thcalveoIar duct and alveoli- common cause of pneumothorax

Down syndrome with v·omiting. ·add maternal polybydramQ'ios:. duodenal atresia Down syndrome with (Q.llsd:patio,1] at :birth: Hirschsprung's disease.

lIi.gb risk' 'of colon cancer: muhiplc .. polyps '

Elevated: alpha feteproteia in, preg'DaDt W·O·lilab~: do an amniocentesis to rule QU,t open neural 'tube ·d~{e.ct

Decreased ,alpha feto'pliJ,tein -in .. pr~aat: wom:aD: Down :syndrQme-

CQm.pliUtiODS -of prostate .hyperplasia: • infection, iii urinary retention. -it does no! progress to cancer

mg.h serum TSH: 'primary: (not.secondary) hypOthyroidism

Thyromegaly, normal T$,g, and Til, increased 2Dtimicr:o-somal and th.yF.~g,lob·QJin an,tlbod.ies:

'early Hashimoto's thyroiditis -

Osteogenesis imperfecta: • deficiency of type I collagen, .•. blue' sclera- due to visualization of choroidal veins

MCC·of a fatty Iiver and cirrhosis: alcohol abuse

Case of mass in fallop·iaa tube. in a pati.ent with au Increase in. JJ...hCG: ectopic

Bacterial menlngitis: • increased opening pressure, .• high protein; ~ low glucose ... ' increased n eutrophi ls

BraiD tumor lin the. third. ventricle: could be. colloid cyst or choroid plexus papilloma. Patient deterieration after eNS trauma: herniation

Karug.eaer's'syn·dr.Qme: absenrdyneinarm in cilia

Rinne and We.b.er test. are like·.if you have cerumen in your ear: _. Rinne lateralizes to affected ear and Webers has bone conductionoair conduction, • signsofconductive bearing loss.

lojedi'OD or 1 liter 0·( saUne,. start seeing, diuresis: due to an increase in atrial natriuretic factor After' head rrauma, ·the:D.rine osm'o'lality is, 100' .mOs.DJ/kg: • inappropriate ADH Syndrome,. not diabetes insipidus .(lo'w Urine Osm)

Prrmary bypo·p·arathyrOidis.m,:, primary due' to' previous-surgery or autoimmuae destruction

1~ diby.droxyebol·ecalcife.r·ol:' ~ active Conn of vitamin D,. .• :requires·.Pl.H for i~::~tbeSis-· I a.hydroxylase

G'rav~ .dlsease: -, ·- typeIl hypersensitivity: IgG antibodyagainst TS'lt,recep:1OT ... unique to Graves; exophthalmos, pretibial myxedema, '. TSH low and T4 'hi·~ • Rx with ~Iockcrs to inhibit· adrenergic effects· and propylthiouracil to inhibit enzymcr·.syntb<:$is .

. Dlffere'u,ces betweea prlm·2J1i/s'~D,d.,ry bypoth,yroidiSDi 'ID telad.oa to T.. ,.".d TSR': .• balh

have low T~" •. primary 'has high TSHt • secondary has low. TSH'

'Worn'3n ·,.h(J Wa$ vomiti·~g.: • hypokalemia, • metabolic alkalosis

Di'arthea; • normal·AG metabolic acidosis. '. normal serum scdiumIisotonic loss), • .hypo~lemi·a A palient iD the hospital accidentally gets infused with sanae coataDU_aated ~ith E: coli· ad develops shQ:ck,· ,2: days later there is 'bleediag. from aU puac:tu,re ·S·ltes, wbat WID .~ ~be

findin'gs: • OIC with decreased fibrinogen and platelets, • increased o.dimers·

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A 16 yeai' old football p.layer- suddenly di.es dJlr:lag. a gawe, "bat will b .. c" .' . '.' . . •. ,.... '.

diss . f th '.' d" I fibers ,"' ." " .." '1 '. ~ seeD on, autop·C1./.:

. isarray o .... e ~yocarla ~ .. ' ..• ml?t~~c.uar $eptum..hypertrophic cardiomy~pathy' -J"

A person develops eongesnve cardiac: failure and decreasedGFR., The reaaJ ·tubl . ....1Is .' ...• hydropic cbange. What is the cause of the hydropiccbaRge?: damag~ to the Na_¥:.:ae.c:e. show

Graphsbowing collagen fermation after. aD AMI: graph·that starts and increases att~ 14 da .

AD AID"S patient develops' diu" "r'be"a" WIth .··D a,'cid I'-: t • . , ys

' ••. ',' -e ' .', : .'., ., ..... ;:0:': . ".,: '.'.: .. _., """ .' ... ~dlS (lrgamsID. What is-the m,oS( U'ket,'

cause?'; Cryptosporidium y

A padent: with liver 'cirrhosls and ascites develops SPOIiUDe<lPS bacterial peritQnitis . hat .. the mest likely org,anism?: EO' coil" " n "W, .. IS

A 'p,.tie,ut. is be~lng:: ,treated Cor leukemia with .antl-cau\cer'" d·,· ... ,',u-.:'. u.tL.'ltIJt/ '''':;-'d' o r- ki,·liJd· ··'Da'y ste ,. II! ,

' -, . '. ,,. ''''',f:ljf,"",T .. .,. U ... ,M..lII, '~.. ones l'S

h·e.lik:e'ly to d,evelop:?: uric acid from, urate 'nephropathy' """,' ",',' "

A perso~ bas, II crescen~o-decresc.eDdo n:'urm.ur b:~d in the right second mtercoStaI space

[,b'a,t' radiates to the car-o'ods, 'what IS the defect,?! .aortic stenosis . '

Wbj,ch type 0'(' hepatitis Is DOt affected 'by alcobo,I?: HAV

46 XX female is b()I"D. with male genitalia ud • vagina that ends bs a .,lind pouch" has bypetteDsjOll and hype.-:oatremia 1:- adrenal hypetplasia,. 11 hydroxylasedcficimcy . Ba;by Is b'o:rn ,w'itb p'orry face, s'lnggis'b aDd dimirtished deep tendo'D ren·e~es?,:, cretin

(hypothyroidism) .'

A systolic: murmur is heard io t~e mitral area. It increases in intepsity by 1 grad.e after a

pa u se, What murmur Is it,?: mitral regurgitation .

Girl comes in wjth corneal r-ing_s., hepatic and brain changes. What i"OilieraJ defect?: copper in

Wi-lson's disease ' ,

Patient comes in with Ieukemia, Over the past: year she h·1.S developed • decreased be~to,~t wiih ~.(,rOc}1i~ hypochromlc.anemia: anemia of chronic disease

Pregnaut'wo'man has-polyhydramnies, What does the Cetus h2ve?: anencephaly

Tumor' with loss or ,1;J53, suppressor ,gelle~ Loss of'rtg"_I3.qo:~ at, ,,-that, ,p:o,'mt?,: Gi to ·S' phase

BQ:y with pica for paint liu an,e'ma with basop'lUlic'sti:ppliug" :What will be-the fmdillg in.this

.. ?" d free ..,.. tth.. ' hvrin j Pb ", r

patielit~·:· increase " tree erythrocyte protoporp rynn 1Il.:.' poisoning

,91) yr o'l,d lady suddenly dies, On '3Q't,o'ps,y the 'cerebral, veatricles are enlarged. Wh,a.t i,s rhe' most Ukely cau,s.e~·: cerebralatrophy

Previously h·ea)th·y'1.2· yr old girl develops :ap.lasti.c. ·:~e'm.ia. C,aas,e?:' parvovirus

Difteren(' ,dia gra,·ms. ~r: wound bealiD:g, and must id,eQ.«fy which one is correct, Pick ·the ,O'De with the 'basal Iayer O.r the-epidermis Joining wi.th the Gut, 011 the other side .. ,

P,.,tie.n( 'ft~it:h s,maJl-,medium vesseldisease is HBsA'gpo5,itiv~.DiagQ·as'is,?:: 'polyarteritis nodose Graph with; Increased basophlls, .eutr0J;l'hils· aa,d monecytes, Wh,at 'is:' the .. caase'?: GM-CSF Patleut has a Slab. wound to chest and there is luag coll2ps,e. What is the .p,r~,1ln iDside the.' pleural, ,C2'vity?: same as atmospheric pressure

Scenario o,r a mao 'who, h~as ,h:ad a stroke and died, AUlOpsy sho,ws, .b,n.in with, heDio'rrhate• Wh,at Is th,e cause?,: stroke' involving lenticulostriate-branches ofthe.MCA dueto hypertension lnhe,ritanc:e patterns for geDeti,c, disease: 'see' High Yield pathology

M .... , 'wo.rldag'm·th rocks develops pulDlo,aary' s.ympto·ms~ silicosis

KDo'w' Rh ,and ABO bemol,ytic disease .. of newbern: 'set' blood bank notes

,Bud,d~'hia,ti syndrOme. (he,p.,tic.veiD thrOmbos'is)~. see'hepatobiliary notes . .

Physical diagDosi$of hlDg. coadidoas:- • tactilefremirus- increased in Consolidations. and decreased in pneumothorax, • egophony- e sounds like :1" sign, of consolidation, • percussion+ decreased in-consolidation, pleural c'ffu:siont't}1rtpanitic inpneumothorax

Sear tissue La braiB; calledgliosis and due to astrocyte proliferation . .' · .... k . f Tissue 'lDost affected by decreased oxyge~: renal medulla (parPcularly ,StlaJ.,pat. porncn 0:',,'

proximal tubule) followed by neurons in the brain

B'yp"ert~Dsiv,e b:r.aiD. 'iDj'8ry~ intracerebral hemorrhage

,Patient wit'b acute pye:l.oae.p,britis:,· WB'e casts

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ComplicatioQs of anoreXia ~erv{)s~ (ttIa;tathon nlDIlf!:I'): ~ seeondary amenCJn'hea d,ue" .. to' decreased GnRH\ • osreoporos 1S

.Patic·ot.-with 'bilateral h',ydro.n,e·pb:ro"s.iS:: urethral' ob.struct'Jon

Hormone iacr·tased .fa aseltes: aldosterone due to':' dec" reased ", d';, ... ' .

. ' .. "*""' , .', ' ~ , car ·lac outpu· 't 'd" d ", .

metabolisrrt by Iiver' ". .,',', ·an.., e'CJ;eascc;t:

Orga:D s~~.Q·dzn..·ly affect~ io ci.ribosi',_ Q.{ li·~,e r :· spleen enlarge.d due: to portal h .. ', ~": .. _,

Pa '.' re. tttts: ". ferr d 10' th b: .L.. . .' '. ' PO. ypenerunon

, . '.J;lC," .• ' ,: . 5. paln rei·I, rrc .. "" e ae.n... S.lnce .It IS .. re·ttoperitoneal '

A1Jlbiotic. fl,u'id embol.is.m: causes D.le·

-S~r.u.IXI· h.pto·gJ"obin: decreased in intravascular hemolytic anemia.

Type of aD~m.ia Iachromc lymphocytic leuateJDi.: autOimmune htmQlytic anemia.

Neo·.lIatal resptratcry distress syadrome; no surfactant· ....

F:iDdiog's 30. ~i'D ~e~ acute my:oc.ardla1lnfarqio'n: no changes Phosphnrus iD.~:ru.s·td in chronic renal failure

.M.e TB: site in reactiv.ation; .• upper lobe: 'of lung~ .• high 01· tension

PaQ~~t tumor causes Horner syndrome: d~truction of superior cervical ganglion in pdster:iOt mediastinum

Gp 1.20 .artaeb ment ·of·mV virus to· ~"' moiec·ule Pedigrees: all types .+. mitochondrial DNA

Causes of children getting,·a: ~ise.a.se .. when parents don't ha,vl!- any evideQce orit: incomplete penezrance m. an autosomal dominant disease

Parieut witb. ·3' hlstory of a, boDe marrow' transplam and 'who' is O'D, ·'C·y,cl·o.spo~. has neutropenia a.ud- develops esophagitis: • picture of intranuclear inclusion- if a single inclusion or ~ owleye, pick C;~ .. 'if multinucleated cell with. intranuclear inclusions, pick Herpes

Enzymes decreased .iu lead PQiso,gi~g:; ferrochelataseand ,ALA dehydrase

Patient witha b~t:ory ef hypertension deveIQ.ps.n iDtr.acran,iat: hemor'·rbage •. Wbat p:art .. ofth.e b .... ain is ~:rrecte·d?:· basal ganglia (putamen is the. 'most coramen area)

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U·U'D.'li.ngton'.s disease: • caudate atrophy, • dementia, • odd movements .... autosomal dominant, •

trinucleotide repeat disorder

Fire.fighter· wi.th a· history of dyspnea 6 hou·rsfoU.owi.ng a. fir.e... .Eiistoi·ogic s·ecti.O.Q (Jf ,alveOli.·wi.tb. hvaline membranes: t\RDS

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~ Patient with. septic arthritis·. Wbat Is inost :Jikel.y organis'm:?,: Neisseria

7' .Alcohollc ·wit.1i a .hist,ory' of depression and 'bypog~ycemic: 'episodes. Low bl~ .glu:cose··.d .. D,O~ C-pep,tide': patient bas been. qkin.g insulin

'7 Cause of .acute renal r2ilure. iu a patiear in a ·Ilu.rsi"ng home who bUD·tt tate. or' druJlk 3oythiog (or' a ·w,b.n·e.. P·adent···is: de.b:yd·1"2ted, has a .. hi$:tory of a stroke 6 "months ago, aDd, bas iacreased 'BUN and creatinine ,from p·revious"'va.iu:e:5. He· bas. pyuria 'but no hemaQUi,.L. Be has' DO't urinated Ia .1 i hours, .Aft.e.r· a.th.eterizatio,tL, lOOm') f: of very dark yellow uriDe .is: removed. What is it?: probably prostatic 'hyperplasia: causing urinary retention .. and.' infection

::r Cause-of paeumo .. nj:a, i..o cystic fibrosIs:' Pseudomonas aeruginosa _

::r AD aJ<:o·iaolic has :a. ,K1~bs~a iafectiou i'nvo'l'Vin'g th.e upper lobe of the ,rig:bl.la:nt,.:wbat· kind: of

necrosis Is it: liquefactive necrosis .

7' . G.raauLar' IgG and. C~), deposits are (o~u.,lld ill. the 'i1om.e.ruiar baselile~Dt membraae·: type II

rnembranoproliferative glcmerulonephritis (dense deposit GN)

;r' What· ·.p2.rt,of th~ kidn·ey· is most Involved .in. dhbe.c:es·:.~glomerulus· " .. ', . ~ .,

7 Tall, S'kinay .17 year' Q·ld basketball pl'a.yer 'with 'Joag arms ad legs' and bype·rOe,nb·le JO'IDI$,

W .bat.is the boy likely to" develop: Marfan's Wl.U;l danger or dissecting' aortic aneurysm ," . ,~

7 A &. year old boy ingests rat poisolling., along with supportive therapy, what should be glVt.:

.11'11 vitaminK injecrion . ."~ "

:r ,P£e,D,ataJ. vi.ta:·mjus: • iren: women.only have 400 mg ofironstores and lose 5"00 mg U1 pregnancy,

~ folate: only 3-4:mthsupply in liver;> Bll'iS not necessary unless a woman is a pure ve.gan

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Patient wj,tb 'prosthetic, beart 'valve . bas,' $C,bistocytes iu peri,pheni blood; pro .. sthene, valve< is m8lfim:ctioning;caOsm.:g. hemolytic anemia,

MC'C':of bladder dive. d,tqla: prostatic hyperplasia

Prevent kelo.id ro:rm~.tio~D::, intral,esional. 'injeotion of certicesterolds ,. M~o,tli.e.r and father blind b'Q,f ItO .familyhiStory Q,f' bliDdnqs, ODe ddld ~dy' hlla,d, ch·uce 'o,ther ,c::biJd, will be 'b,'lind,:, • pati.ents. 'probably' have ~ nOrIlO7;Ygous AR" 'disease' and parents Wac

- - ~ ~ -:'1 hi~'l'~ ·1>'1 ha .. 1-.- di ('1 L\rr;IO" )

asvmotomatic earners, '. ~".'C __ "·Uletl wu. navetnecsseasej ~:~,o.,

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Vitamin', d',efidency 'm ,pll~hrD,moqtoma~ vitamin, C-~:eatalyzcs dopamine, ttl ,norepinephrine

'PhaM' of .~,te lDn'amm~1iO,D,;' begins with, ·tnnsient vaseconstricnon 'be.fo,~ v:asodi1atation fi:9m' histaminerelease

\,'ell:o" m~ :ute,D~g, up :iDre:~or 'yea. Q'¥a·: renal .adenocareiaema in~ding: renal vein 'Mth~ extension up X\le,

G'nlD,aiom., ,m Am'S patient: 'would .have macrcphages butthey would no' 'be ,.c,b:vated· to kill

pbagocytosed ol.ganislllS ;smec·vCD. T'helpereells.are ,go;ne, , .

'~-.- '.- ... - · ... (:.'PC··,o,( iD~'f2sed sernm cortisel ,au,d tliyro:J.j:ne ID 'p·r'IPaa:cy:' •. increase in synthesis: of<tbeir·

respective 'birl,~g ~ptoteins by estrogen stimulation-of liver, • TSH' 'and ,ACTII·att' both. normal .• • flee hormone levels-are normal .. ,

Modlerwith polyhydramnios andfems with aDal atresia ~ V A TI:1Lsyndtome- 'VCI'tdnalt1efcclS •.

!~l~~si~ ~ch.phageal flStUla~!Bdial· (absentl'an(i!~abnomwitiC$ -. .

Po. ~ ·'U, , •.. : .t·' - ..... L.' d' .. ~~ -v." e ~D,a-u', '5-' tjL, rn ~"',bo,sis o.ol:y·- mea, 'warf8ria: (',D,ot hep_~;'ariD) experi,mces, fDl1b,R'

a e,n Wl&JI_ up .. _ ... e- <u.u,.~~ - _ - ... r.... . I,.' ~ _ ~.

propaga.UoD of ~eDODS dot: " htterQzygoteprotcin C 4e6ciency~ • short half ~fe of protem c n:nders the- patient hyper¢oa.gulabte in 6-B hrs, since factoxs V and vm· are tnercascd duc to

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Patie'Dt develops oliguria after an .o'traveaous pyei~ftII,~ • develooed nephre '1:'" e ,

' , "-', '. -b·'~· "," ' .. ~. , .. oxrc acute'

tubular necrosis. • dangerous ,to order ~ IVPin tmiltiple myelorna~and diabetes mellitus .. RhellJD;.:foj,d' fa~ro;r: IgM antibody against IgG

Child wbose rat~er bas a history _ or OSt~eD.ie '_"COma <I~velops lUI eye problem.: • child probably has retinoblastema with macnvanon of Rb sUPPressor gene on chromosome 13 •

~steogmic ~oma and breast cancer are ~thc:r. CUlCt.::rS associated with this s~g~~ •• likelythat ~s ~. a:p_ au~o~maI d~~tirthct1tance: pattern, Wane of the Rb suppressor genes \VIS. Already Ulac~vatcd at birth, _. I~f rt~tiV~S on Jnp~ets side are all noemal, ~ the child's eye problem would likely be~spo~dic ma.ctivanoo. (lI1~tlon) of the Rb suppressor gene. where both chromosomes ,have to be inactivated,

Weight lifter developS .DRuibness ud dagliJlg fa. the arm. while sleqd:ug: thoracic outlet syndrome with compression of subclavian artery and brachial plexus by hypel(tJophicd anterior scalenus muscles

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Malt chlld with chrome iAfectlGII5 'With • gTllillllomatons reacdOll".' chronic gnmulomatous disease of childhood, • XR mheritanee, • absent NADH oxidase in neutrophilsand IOOnocy1I:s therefore cannot convertmolecular oxygen into superoxide free radicals (absent 'respiratory' bUm) and cannot kill bacteria, especially Slaphylococcu.s aureus, '. without superoxide, JX%.OxidC cannot 'be produced by superoxide dismutase, • 'Without peroxidase" rnyeloperoxidase cannot. combine peroxide wi lit chloride to produce b leach.

Chromosome 21 codes.for an Alzheimer precursor protein (APP). part of-which is tmyloid-j3 (A-fJ) protem: • toxic to neurons, association with Down syndrome (3 functional chromosome 215) M:;CC q:f Alzheimer's in: patient's under 40,

.Apoli,pop'co'tein. geneE, allele &4, located on chromesome .19'·:· ,. produces a product.that 1n:Cle2'$CS the neurotoxicity pfthe.A-f} protein in Alzheimer's disease, • cause of familia] Iate-onset type .AbDo:nn.ality OD c,hromosome' 14· ill A IZbeimer 's dis""~e: synthesizes a Tau microtubuleassociated protein located -in. neurofibrillary tangles

H'amartoma:' •. overgrowth of tissue. normally- present. in the. organ, '. bronchial harnartoma: increase in cartilage, .• 'Peutz Jeghers polyp, • hyperplastic polyp in the' sigmoid eolon, .•

angiomyolipoma ofkidney: seen in tuberous sclerosis ~

Beteretoptc rest (cb:oristo .. ma): .··no.l.nml, tissue in a place it is, Dot normallypresent, • pancreatic

f ~ ,. . 'M' ·t ...... \II di . til

tissue in wallo . stomacn, • gastric mucosa 10 .8 ,.' . 'ec'~I, s· . ", '.VO: DC' 'h mit

P,a,tient 'w'ith restrictive' lung disease", Rayn,aad.'s .paeuo·me·DOD,. :f,eaal. disease.: • progressive systemic sclerosis (scleroderma), • esopbageal dysmoti.1ity· is another major problem leading 19

replacement of smocth.musele.by collagen. .·dysphagia for solids and liquids .

AA,T deficJeo,cy,: _' ..AR disease, • .AAT is an anti-elastase that d·~oys. elastase (protease) released by neutrephils, • children ·with certain phenotypes develop cirrhosis: PAS + AAi in the hepa~ (defect in secretion of AAT out ofbepatocytes) •• young adults lVi~ CCl1ain~phcno~, have complete deficiency ofAAT and develop panacinar emphysema (cnbIe. resptratory W'lU

destroyed] in lower lobes (absent exl globin peak ena serum protein.electrcpheresis)

FUSt step La management of tenSion pneumothOrH1 • positive pressures-in 'l'leunl cavity push the lungs and mediastinum in oppesite.direction, hence-compromising respiratioaand ~o'US. rerum to the heart, • insert needle into .seeond intercostal space (anterior chest) to release positive pressure

buildup in the pleural cavity . .. ;. .

Gnve5 disease; IgG antibody directed against the TSHreceptor: type n hyperscnslttVlty. reacllOD,

Newbotnwith tetey ·.Dd small eus:. DiGcorge syndrosne, •. 3m and 4th pharyn&ea1·~b~s dQ not. develop (absent" thymus 'and thymic shadow and absent paratbyE:pid$), • expect hypocalcemia

3S

'"

N'o'te':, 'Tbit ma:ttri'.'1 Is coprrig'btee,,,, All rights reserved, Ed,ward ,GoIJUl.; ·M.D .. , 2002

• <

r 1

"_ --

~,

NSAlDs: • prolongs bleeding ~m,e:lnhibiti",g cycloo~ygen.ase4t:creases synthesis :of 1XA .. I

platelets cannot .aa~,O'1ife. • 'normal·p·lalel.et count and p rothrom . bin .!!:I, d p--~r.ka1' ..• ·~~L\_~., _.; 2.' .~d

. et-b" ... ·O·-· '.' , . ,., ,;1; . ~,'U .UUU!lo..w.u}'l4:I:itJ.n .

N~t DlQ'Dtb'ol'd bla,c;:k child, bu.d .• ctylltis~ • sickle.cell crisis. _. ,sic:.k1··~'·e 'c. e'l'l"~i~~,~"---'t~'e,'du' .... , .. to . n~ I'., ',_

. ."" , "', , .~ '~' '4: '3 POUlt

:~:::~·ssense mutation) where valine replaces glutamic acid ill lhc 6th ~tion of me·'~

fJ'ern~cious aD.em~~: • auto. immune dcstrUc_tion of parietal cells, • .absent "intrinsic factor (B . r:

deficiency) and acid production •• achlorhydriacauses mCl"-sein.serum pstrin level$' II l

Be~ophWa~: • S~ ·deficient Vilt coagulant, VIR complexes with platelet factor·-3,.1Xa, and

calcium 1.0 activate factor X .

NSAID$: (e.g~ lD«lO:IQ~~.cia:):·· decrease PGE'l1 which ,Ji<»: lua.I1y maintains the rnueeus barrier in ~

the stomach and, proximal.duodenum, • predisposes to .. pstrie erosions, and peptic ulcers

Wo.mea, .with exeesstve weigbt '10,5$ '(~~I., marat'bo'u rull.Der, aerobics iDstructo.r, &DO rem,

nervesa): decrease secretion o:f~, from ~ypotbal~~US·lcading, to 9,~ in ,gona'dotropms-f!" secoadary amenorrhea-e osteoporosis (lack of estrogen)

WO'manla 4():'·s· with mallgBan,t' Qdtes ad large. ov,arlaa mass:' .. probably' a :mueinous cystadenocarcfnoma: very "large ovarian mmor, often associated, 'with p~ud()I:n)?:olua. peritonei, if it was bilateral, then. a' 'serous cystadenocarcinoma would be-roost li~ly

Posterior acute ,rQyoCAr'dial i,marction, right heart eDlarge$ in, 1 wee'k,. ,pa~oDar, capUlary 'w,ed.g~ pr'essnre -,~.~'d«reased: • probable right, ventricular infarctiOn with pure' right..heart failure" •

potentially could be a pulmoaaryembolus producingright heart strain ' .

¢r,oss. (G)' ~D'd· Mieroscopi·,c (M) changes in -, an acute my'~cardi'a'_ inJarc:tiO"D "(AMI):

.' 0--4 b'r5-' 'no"GlM change

• 4-1:2~ ~'~ .• ~ no 6' change - M shows coagulation necrosis after 6 hrs

• 12-,24 h,rS'-,. G shows early pallor, ··,M shows more advanced co agUb.tio 11, llea~sb

• l-:~ d,- • 6. _ definite 'pallor; ~ M - neutrophilic .. infiltrate comingin from, the ,perimeter of the .. infarct :r advanced coagulation necrosis

• 3-7 d~ • period of.rnaximal softness; .: time for ruptures, .• 'DJ2ttop:h'aI:es 'mcOY:e' . in, frOID .the

perhneter 'to remove dead tissae

.' 7-10 d,-, '. G'is the same as, 3 . ....:7 d, • M shows collagen deposition. .

Thyromeg,aI.y:, normal thyro'sille and 'TS'a ~r~e'Dce;'or antiQ:Ji erose mal ,,:m,d antitll!~lobaliD,

antibodies: early- :sta"ge. of H'ashimotQt,s thyroiditis . . .. \ . ~ .' ,~

'Patieot develops dementia after ·instnlmeu,ta1 mvolvem,e,li:t .in the brain: CJ d.isea~ due to

..

r.:; with syndactyly; defc:ctin apoptosis, which normally would have-caused regression of those

tissues 'between the 6ng~s:. and/or 'toes, " ..... .

'Bipolar patient develops po.yu.m: probably on lithium.' which pt'Oducesnephrogenlc diabetes

insip'l'dus _

HematOpo.iesis in Wt month· or fetus: occurs m bone, .. marrow ."

'B' 'ab' '_If. -'L'~ Wl'·t!t· every &:~ .... ...:II':'-g' and' develops p.lleumonia: • TE fistula, '. blind 'pro,nmal , ..,J ~O~:' .. ,,- '. ,:-.,' ha&~':~ ._- '-_'. "fro' 'm·,:'tra··· ·C·k~ .• · st oma . ·c·h· di:·.··~-..i~~· .' ·tDOther with

esophagus, • distal ,csop .',gus anse·~, '" .,' .. .s • ·11Cd-, "v" ".' , ~u;..u.~ "." .'

'polyhydrann'lios .

DidbylstilhdtJ"ol: intcrfercswitb devtlop~( ofM~lerUm$truc~ ,. . .... ' ..... '. e·tha into the paneal witb a eeDtrai line develops beDllpareslSc. • ~tral lme rs .. inserted 1 _ .. "t

· .. . : :_'.'_ i. ".: their ernal iuzular vein .' clot. develops tn· the centralline and, mibol~$ to ngJ1,

subclaVlBll vern or .. , c mta,UAI JU:5~'" a.a.,.. ., . ._ .

. + d .. ' t: ...... SY' stemic ·circulation·tbrougp ,an' atrial septal defect . .' ._,. "..: D

,atnum ,an . en, .CT'S .. .... '. ' , .'. . .,' . . , , - (' + .....~. t .. _At ''MiH C'\Mdrome), u..

." . coul :'d t........ d" to - ,. A' excess of 'ficc water mappr:opna,c':ruJ -~ ~Jt!.' ',\. . '-.' _.

Bypona~emi2: <, '.... ..:(11;;' ue ...• an. ............., ~ states like riiht heart failure. citrho~s),

reabso rp' .' b: ' on ·of p. TOpa. . ... rtionatel Y I'JlOre water than salt ( . ,... . . .. '.. ... ' . . ~ _. 2.' 1 ~"~Y·Y'-~"'.,

, ':. -, ~. :". . OA.l'lt.nJt."i.-ridc diuretics; . ~'I'"'v.n. ,~

or: C~ 'loss of pr.oportionattly more salt than water .', 'UVJN~· ' .. ' ...", , - .'

'!Ii'

'7"

Note: This natmal is copyrighted. All riEbb: reserved, 1:4" .... GoIJaD. M.D. ZOOt

deficiency. Addison's disem:e with loss of,min~oconicoids. Rx A by :«strictiq :' water .. ' .. ' ' .•...•. . ,reshictino wa'[e'r' and salt Intake and usme dJured~, RI" C,", b' y' oivl .. e; "ISO' "'t"o' .... 1(. rmaf, Rx, B, by

'I!) . 'b ,j ... ~ "',' W"'" .... fii. . DlC DO." "sali'.;',

Se dj' ft' 0· n' '" Y bra . oso 'e de term '1:'''' ' ,. " ' De

. :r 'Ilerea anon; •. C . m . m ....... : •• :".es gClletlc sex: absence of Y cbromoson:e ~Its in

differentiation of germinal tlS5~e m(~ 0van.cs. :""ol~ d~t structures undergo apoptosis. senee of Y chromosome causes germinal (Issue to differentIate into testes: muUerian inhibit_..'.~,

. , ~ f 0'("'·' .. .v, - • th' , --"7 'actor 1$,

synthesized-o apoP(oslSO m , uenan [Issue En .'. e male fetus, • feta] testosterone ~1 ...•.

epididymis. seminal ve~icles, vas"deferens, •. fetal dihy~testosterone (DHl) develops: pro:: (undersco~s,why ~HT is responsible for prostate hyperp,l8SlalC8nceT and not testosterone), c.xtc:maI male genitalia: fus10n, of the labia -+ scrotum., extension of clitoris -+ penis. • S-<l-feductase: converts testosterone mto DHT

T~c ~ermaphtod1ti5m: • patient has both malelfemalegorwls: ovary and. 1CS1i.s. .or ovotestis, • majonty are 46 XX genotype

P5eudohenoaphrodlte: • patient whose phenotype (appearance) is not in agreemear with the genotype (true gonadal sex) ••. male: pseudohermaphrodite: genotypic male (XY with testes), phenorype appears female (female genitalia). e.g., testicular feminizanon, • female pseudohermaphrodite: genotypic female (XX with ovaries). phenotypically resembles a male. e.g.,

virilization in adrenogenital syndrome '

. .

5-«-reducUse deficiency: • male pseudohermaphrodite: not present in females •• absence: of DID

effect on male fetus: testes located ininguinal canals, absence of prostate gland: no DHT effect, absence of all mfi II erian structures: no robes!uteruslccrvixluplJCf one-third of vagina since m\lllc:rian inhibitory factor is present. external genitalia female due to absence: of Dlff effect; blind vaginal

pouch, vagina partly milllerian in origin and partly of urogenital sinus origin. • p:rescnce of testosterone effect including: epididymis" seminal vesicles, vas defer ens

Testicular feminization: • SXR disease: MCC of male pseudohemtapbroditism. • deficiency of androgen receptors: DHT and testosterone are present but cannot function without a receptor, III no mullerian structures: rnullerian inhibitory factor is present, • no male accessory structures: 00

.. testosterone effect, absent: epididymis/seminal vesicles/vas defer ens/prostate gland, • external genitalia remain female: no Dlff' effect" 'vagina ends' as a blind pouch,« testicles located in inguinal canal Or abdominal cavity: surgically removed owing to a risk for seminoma;» estrogen unopposed, since' estrogen receptors' an: normal: secondary female characteristics are well developed. .• patient is reared as a female, e· laboratory findings: normal testosterone/DlfT, increased lH: LH does not ~ respond to the negati ve feedback of testosterone .

Smoker w,ith flank pain and mass, hematuria: • renal adenocarcinoma .• derives fiom, proximal renal tubular epithelium, '. smoking MC'C

Family tree with AD Inheritance pattern showing disease occnrring at aD earlier age ia affected members: • trinucleotide repeat disorder: disease gets worse: in future genentions due 'to addition of trinucleorides (e'.g., CA,G), • e.g., Huntington's disease an AD movolrnt disorder associated withdementia would occur at an earlier age. - e.g., female carriersof fragile X (SXR.

disease) will develop symptoms of mental retardation (makes it look. lila: an SXD disoeder), .

StiUbora of diabetic mother. ia poor glycemi'c cODtrol: likely sec hyperplasialhypcrtrOpby of P-

islet cells in the pancreas as a fetal response to maternal hyperglycemia . '. .

Clrrbosis of' liver: nodules are regenerati ve nodules composed of hepatocytcs WIth no -eord-

sinusoid-cord. pattern ,.. .....

Cirrhosis of Ii'v.·e'r!· • antibody test that would be helpful in determining the. cause would be

antibodies against HCV, • antigen that would be useful is HBsAg . '. .

Newborn has cataracts and vomiting: • probably an inborn error of metabolism (e.g.,

galactosemia or hereditary fructose intolerance) •• order a urine for reducing substances: detects fruct ' , ,'. d gal" ac' tose

, 'osc: an' ., .,'., •. ,'

..

I

,~.

IV' .i~fDSio'D, o't'tbolam'ine (B 1.) 'iD, all a1·c:·oho'lic·~ • thiamin'c is a c':olac,tor Ear' .'-' _: '.,' ._. t, _. -. '

. "k': ,_', -'. I .-.. - d - h _ -dr ,.,'," ,c. ',' d k' ,-., . .'.' .. " " . . .. '_ PYl:Uvate' deh'~-o~~C! ...

. ~~",et~.g u~,te _ se y.ogenas~,. ,an·" ,Ct~-~t·Q.ac'ld dchydrog.enase, •. all these ,'-. '. ~:. 7"""'.c,~.,~"

N.ADH, Wh.1Ch~ In tum, generated 6, ATPs rcactlOD$ produce

- . . .

E·ledrolyte· abn·o'rm·alities in dJab'eti'c kefG·acido·sis:· in~ anior ,'... , " .' _ '. ' .. , '. ,." ~

t: ,. . ",'. A'~' A", d A-O"HB' , a,;.:'.',. ",,'." ' d' ~'- ,., ,.,' . on .gap metabolic, acidosis:

increase ~ " .,C.C ·an·,~ ~ .+,.:, Jlypo~,a1re.~~: . ·ll.uttonal e·ffect·ofwater movrD.·. '.:' :f.'.' .. < ~._:: .:' ',~

by.' ,OsmoSIS', hvoerkalernia: transcellular s h· ift of K' ... · cu.' t'· '0' 'f" . II" ~ ." .' "i • 8 .~ut O. ICF ,~to ECF

. , .. J ~- . ' . " ." -, C.c -as n moves' to the '11 '

~ . ~ d" fi . ~. ,L 'K" d , " d'" .. , ' .' - . ,' •. m .~. ·c:e .' actually··the

~~tten~ lS.,e:lc,l~t 10 '.:+-' ue to osmone uuresis .• IO:sS·(Jf"tX~~.j.vc· .am6tlnt ofNa"': .' '~':"':"':~:'_ ,', ._;

,OSlDOtJC dlUI"e.SI-S' - and K. In urine:

'Child with petecb.ia... th-rombocytopenia." normal" aad PT·r 'D'O 'bist'_'" di

thrombocytopenic purpura, type Ilhypqsensitivity ......,' .. ' sCocytes;: 'idiopathic

VlftiCl'etaria.q w1th microevtle a·Dem .• ;,· :. 'Po robable ~'NWI' defi ciency plaat ;"'00.- ... ,. r. · . d . "

..... 1lI . ,'" ~ "'Ii ." "....,~... ." ',', ''IWoA1 . ..' " .... ' .u.:' .' 'lS· ",Clte an' . ust'be

converted into f~us tobe reabsorbed;» verr s~n risk for iron de fie ieney e- '.' I, m .... '

Womaa with raC:181ras~ ~hralgi.~ ~sitlveVDRL 'fritIa negative FTA-AlJS~ .' .' ... ,..". ..', . ...... ',.~~tOSUS., note the biolozic false: POS1:";~~' SYP' ':hi' ilis sero ,,' ",'10' gy'" due toan ·b .. -cardioli ~~bod' c l~us,

L.-& .1,:.UA.-.I,U,A . ' b·· . . ,", , . -. w· ... """ . . . ." '11.1,,,,'.' ' .' .. ~:.: . lpm 'an'I ,', nes

Raised . rat lesi,oDs· in AIDS pa1ie,B,t: '.' most ·ofte.ri due to Kapo:- sits' sarcoma . '(HS' . sv '~)' '.~.~. '1~ A~I"

• - . .. ,. '. , .', ,'. '.,..._ ,~.~ • . ". ~,' I • coum AlSO

be bacillary angiomatosis due: to Bartonella henselae: silver stains identify orgmi~ "also ~ cause of

cat $cratc.h,disease· .. · "..... - '. ' . ,.

ADi~aJ wUh ,AB alleles . crossed with aDim:iil 'With AC 2Ilele$. What petceDt of,offspriD.g aD.

receive .. .AA organ? 75°iQ " '.

A ;C'

A, AA, ,AC

S' AD

Classic ABG patterns; pH.

(7.-3.5-·7,.45)

'7' '22,''''

.. -

. ,..

7.2'6' 7.33 7~4,2

' ..

7.5'1.

7,~.56

Be

'P·aCOz. 'K.C·O:;"

(33. 45, 81m. ,Kg) '(Z2~2$m.EqIL)

.6,9 l.7·

.26 11

68 34

, .'

22 14

4.8 ·38

,24 2'1.

A. ~B~

. "

C

.

D.~ ,E., 'F

"" ~.

A . Acute uncompensated respiratory acidosis; 'Pmi>itu:ratc::' overdose, CNS, trauma, AlWS, paralyzed diaphragm or other muscles of'respiration

B~. Metabolic acidosis with partial compensation: any cause of increased AG metabolic acidosis (lactate, AcAc, ~':'oHB, formate [In(:thy-:l alcohol poisoning], oxalate [ethylene. 'glycol poisoning], renal fa ilure , salicylate), any cause of normal Au metabolic acidosis. (diarrhea, renal tubular

acidosis)

C... Chronicrespiratory acidosis with. partial compensation: chronic bronchitis, bronchiectasis, lungs in

C~C~6 ,

D. Mixed primary respiratory alkalosis and, prima.ry metabolic acidosis: salicylate intoxication.

endotoxie shock

E,Me.tae6lic alkalosis with partial compensation; vomiting, loop/thiazide diuretic. mmcraiocorticQid

excess (primary aldosteronism) i •

F. Respirateryalkalosis with partial compensation: pulmonary embolus, anxiety. restncnve lung

diseases,

38

cr'

ClaS.s·ic. :e~ectrolyte ,p:.-o,'1lI es;

Seru m.· N .. ·' Serum, K: s

" ·,trQ,m cr Senlqa ,BCo,~'

(135-147 mEq/L) (J·.S~~O mEqlL) (9.S-10S JDEqfL\ 121-211." ,," .. )iI' ......... :.,.n. '.:.')'.".

A. . 118 3..0" Sa' '. I " '.2,",,,~ ~LI

B.. l'U 3.0 80 36

C.130 5.5 ,88 10

D. 128 5.8 114 14

E.140 3:0 U4 14

F. 130 .2.~O 107)6

Pattent A.b,~ inappropriate: AIlS syo.tlrome--nOte Ilu!:·dil~pn:ai e:ffectou all th~ ~yt4. especially the serum, Na

. .... . -

Patiene Bcould hu.metilb(}liC:.a1blo5is.~De to 'Von:dtillgor diuretics (thiazide, '01' loops)

P,g·tie{llt ,C bas increased .au'lo'n g- a'p: (. 3:1 wn,'~";'''' .).. m~" 'e'""#I!,'L.,,,,,,,-, 'L1' c .' "c;lt:,d· .o··~s· .1$ ..... '- ~~',' ii, '~:, d " " "',

,~ " ',. ,," • -.' < • , , ~ ~ . "~,, " "'''l~ ~,'A.A1Qn1a lS ue. to'

transce Ilular ,s':ru.ft.

r~t'ient n b~ Addison's disease-or a type IV.RTA due to IIline,ralO(:Qrticoiddefici_g- note. that:

~sepaotien~ have a nonnal AG metabolic acidosisand hypetka].emia .

PatieQ:t .~ has: a 'nor~ai anion gap meta'OOUc acidosis due- to .diarrhea l)r type J nr type U RTA- note

the hypokalemia '.

Parienr F has mineralocorticoid excess (e.g • ., PrlllJ;;U}' alclosterOiibDl)

<4 ,.. "·th·· h-' __ '1 . . . . "~. 1 l~ ,; ...... ..J' ith 'C'DV' ,

:r::r ,~SlaJI ma,D WI " 'naso,p','a'rv.ng,,~" ~mass,:· :. n:::.'~ c:' " . ":g' .'ea, carcmo,1"n~ '. associated 'WI'· .. ' ~" ... '

. . ~ . . . ....',~.'- -; ' .-Vl', .,. .""' .. ~ " . , '

Myeloperoxidase defieieney; =acqaired or gen~eti.e,. abscnHa:rurophilic ~ules:.jn n~hils in

peripheral blood. .' respiratory burst is nl)rmal- can generate superoxide FRs, .' cannot kill

., , . (

bacteria= • microbicidal defect, •. 1:10 .MPO, ,to produce bleach,

. -. ...... ~

CongeniW adhesion ~olecQJe (13:z- integrins) defect: ~'fa:n1lre ohhe· umbilieaI conUo sepan.~ in newborns, ",'no adhesion ofneutrophils to the cmdotl1eli~ cells, • ne inflammatory cells. irttbe . ·b .'l"j" :+. , 'j ~'l'"""",

um :,.l: ·lea :' ,~.~u.t;u:p ,

Comp.ar~o nofperni¢lous., anemia (FA), othn auseso{ Budefi¢ieDq'od folate, defideacy:

'~ P'erDij:iou~s aaemia :. OO:er ,B'!ldef F9late ~,eolidea.g ,

.Mev Increased Increased : Increased

"

.~ ~;.

.Schilling's test ." Corrected byadd- NQtc~ by · N9t.l~

· '.. , ing:intIiu.sic factor' .int:rinSic factor .

• ..,. 0; ••. , '. , • bo' Id' eel . '. . '. ' .. ',. t· t· . . differeatlAl PO' ,iRa

Differential featur.e$oftbe ~~roqti.c Hew.s: .........•.•..•....•. areasrepl;eseD. .. 'l' " ' .•....... 0 •••••• '

. . . Fe deficiency AqJ e, ~TIW miDC)r Sideroblastic (pb polIO.)

Me" 'V' .': La' ~w Low 'U.Jw .WW'

. '~' .: '~ . '-."J ' 'L' ',' ., 'Nonna.,.'~ '1·' 'Hi.gIl·

Serum iran Low' .. :',()W

TIBC' Higb Low Normal Low

D··' S . ; Lo ~ .. ,.. La NQmtal Ri~

% Saturanon .... WoW H.gb

Serum ferritin Low High NOllua~ N' I

'RDW High Normal Nom~lr.::ua .

RBC count Low Low High , .,' ...

-: ".

N one,:

serum zastrin levels , 'Increased . Normal

, .. " .. " u ", "

Present, . PI CSQ1t

._ "

"., ",' .

' .. R ypersegrnented . .'neutro_p_hils . Present

I Autoantibodies; against ]F' and . Present

parietal cells

A:.:\.._ 0 t Ab, ",' .. ,.Sen,",' ."" '"I

.A.Usen:

I· Achlorhydria Present .;\:bs¢nt .

N' /A:~'1.' ~ :~ ~

, " ,.

I Plasma homocysteine . " .In'c.~ea:sed Increased

. Risk of stomach .adenccarcinorna • 'Increased NOD,e

. None'

, , .

. Urine 'metbylmalonic acid. 'increased, Increased

'N" ormal

.~ " .' . - - "

I : .",: 1.:', ,

--! ". _.' ..

RBC"FS" Hgb electro,

-

,F:e "de"ficieb¢~y

l{'igl;t

. Norma I

A~CD High Normal

~ ~ That ,rg.i·nor Normal

Sidero~blastic: roL, po-' 'ISO"- -. -)

, ... ~ U . ft.

.. . .. '.~.

-H:-gh-I ..

. '1'. (Ph' ,. -

Q.-tl1al; normal Norma. POlSOn)

r3-thal~. ,!Hgp ~'l and F" No~l'

'l:l.gb electre,

gold. 'standard

r" ~

'~~,- I

[I

r

NoC~.~ This material bcopyrlghttd. ~U rtihtsres;erved.,Edward SoIJan.M.D.1OQl

'Marro\Y iron Absent

Miscellaneous 'Ferritin best,

Hi-gh

High

,...,--'"!l.....,._. baso . 'hi' '·1~

'\.;.\J~ ,..>ir;,;- " - p"" . l' ~

, -:,., ' ,- . "',' "'",

'stipp,' lino' rin " .-..:1

~ ,: "cp:,,' g~

siderobl:asts;, " - '

,{

r. ,A~.·,,~Jlp~.r~,D~.p;ri~; a~-te~"po~'" ···~·t~YC;yth~.-- . '~" e~: nu~,',,:~'i2.; a~. -tln~'" ~et~~ 'e~ased;:' ';-',' ,~.' -t'~N~' o;'~~:'laa~···fl ~"---~~~;=~'~~ .. ,~ .. ~:','!,I,~,- .,~.:, :::~- f·:",..,

COPD. c: .oti¢;OID Ittciblsed:

• Ectopic polY~Ylhemla . • Increased ...

! y " '''' ~_ ,,: ":",, ,.. - ~ eereased . Normal

.. volume ,d:- , letion ' :, ,', ..

tl~

Right ventricle

Righ '.,

, ,1. amURl,

75 75 80

.' ,:',' "",,' '- 75, '75 80

Pu Im "0' marv :",,"-.~ [' " ,_: _ ,". ~~ __ J _ ·,ill-l

80'

Sao·:t =: ,oxygen saturation

l~, -~ YSD, B. :; A:S:D:!t 'C '~, P,'DA.,',D ~: Tetralogy; 'E =. transposition

~ .. oSutnmazy ofpulmenary (audioDtests in'Qbsttnctiye 2I1d restri¢ve luugdisdse!

Parameter Obstl urove RestrictiVe .

Toad lung capaciry (TLC)Jncreascd ~cd

Residual volume, ,~V) Increased Decreased

Ti.daI. volume (TV) ~)~

Vital capacity CV'C) Decreased Decreased

FEY'l~_' Decreased Decreased -- .. , ...... ",

.".' ,"_~. . _--,. - ..

FVC f)Ccreased Decreased

FEV j.~C ])ec.Teas~d Normal to inOf~sea

FEV'1K =forced expiratory volume 1 second. FVC ~ iorceclvital.capacity

cr Comnarison of ween dive ColitiS ., .. ,·aad·Crob.n'-s.diselIse'CD ::

.' - _ ... - ." . . -'

.Cbanderistic Ulcerative Colitis Crob.Q'sDikase

, , '

. 'Mucosal, and S,'lb,mocow

... ~ - - - -

.'

[ t~'DSmJJrIl - ~.

- -

c TlrDlinal' Heu'm,i.Dvo,IY~ .,'80"1 •. 10 Illina] , ileum alone (30C'A;). ilturnlcolon (5"'-0).. eolQn ~

alone (2~/c,)?AlIal involvelllCDt' 'So/a- ,I,:;:

.fiS$u.re$~ ·fist:ulast ab~, IIlwlves 'other ..

areas,' of'·GI 'tract,moath :to .. as .

, . -

y. __ OIIiIi '.

Locanon

40"

.

.

._. - -'II"~-l'J'·&d,;I; _L'UU~

......

:Cbaract.erlstic.

Ulcerative Colitis Cl'Obn'sDbeue

p'

e Mi·c.r·osco·pic

~ .

I fe:a;tu r·e.s

Inflammatory pQlyps~ psendopolypsFa.tcrecpsatOl.1J'ld.the serosa. Thick bowel

. rep;esenting areas Q: lnflatn~ ~all and narrow lumen. Skip areas

. residual muc()S~a.F~l.ble,r~ ... . . . . . .(l~~t~eatute). StridDres, fistUlas (very .• Dl·coS~ bleeds easily WbC!D 'o~ch~ - a.mgnostiC) m areas other than an~ Deep I

Ulceration/ hemorrhage. No- slcip hnear. ' I _. ulcers, ~()bblestori.e pa~." aphthoid :1

areas. ulcers (!:\1ll3.11 mucosal ulcers, origin oflinear

- - - I weers, early· $'~gn).~ _

Active disease: mucosal inflamma- · Very diagnosticfearures: lloDcueating

.. non with crypt abscessestneutro- , granuiPIJIAS (600/0), tra,nsmlIral

phils). Dysplasia/caned' may be innaD1ina:tiott,nth subseroSailymphocytic;

· present, Chronic :dis~:-' neutrophils iDflJ-tratioD~ . . !

repl aced 'by' lymphocytes/ plasma ~

- cells, Dys.plasia/ cancer maybe

present, _

me.g.ac.olon:- hypotonic/distended bowe 1., 2 .. sclerosing pericholangitis,

3. HLA 8-2'7 + ankylosin g' spondylitis! uveitis, 4. pyoderma gangrenosum, .,)", adenocarc inoma- .... 10%) greatest risks are pancolitis, tar~,y .onS(:tofU·C,

i : and, d~ti_on 'of,dfsea;se>'lO years

I Mor-e"common thaD :U':'C,:''''1 t.;~ .. I_.·

.' , '.' ,._: _-. • l~·1..U.Li&

formation, '2. obstruction, 3· .. disease in other areas of Gltract, 4:, calcium oxalate renal stones'

Clinical

- j

Left sided abdominal c~famping. (not . Rigbt .lo'w·er quadrant· ~6cky p;,.iD

obstruction), diarrhea withblood/ (obstruction in area ofterminal ileum) With

. mucus, rectal bleeding, tenesmus diarrhea, Bleeding' ifeolon invelvement,

(painful_ stoolin,g).. .

t

.•

:.

, . .-

_" ",

! Radi "L

.: ~~ uograpn

Lead pipe. a.ppearan.ce: in. chronic, disease,

~·S·triDg" sign, in. the ~J ileum from 1 luminal narrowing. 'by 4rllarlbuation. .Fisrulas .... ,:

'lote .' .retati~lD

HB·sA·~: 'HBeA - . A,,:J.ti,..HBc-!'M:

~

ne ranve

·positiv·e

posmve

·nc· . - ti ve'

positive

ne .. tive

-" .

. acute infection or chronic if

. - - -. '.

>6';mths

negative

nt, "atiV'e: ". ne' . "rive nef,. -n've' ne··.:_ tive . .-' - siti,ve U.fhllunized, ~.

1- . "sltiv·e, ne:-' :tive Ol:.--.: rive I ne _ .... bve. .' n,t:" -,·.tive· .. earliest.- :h,ase"of,scute, Hl3~V' .1

'-.7 .. SumHl~ table (or polynria: CDI = central diabe~· .insipidus, NUl =. nephrogenic diabetes.

insipidus

N:'O-"'_'RMJ\L-'- - ... .:»

I . . -. .

"," )".' ,'. .

CDI

. '

I\'DI

P.S'.YCBO;GEl\'l'C

.P'Os·m· post H20

deprivatiou 292

31-.9

3"1.2

2·88

UOsm :post.81·O dep.r:i·vil,tif;l D, 7'S:O

11'(j1

-.. .

98

760

U.Os·m post ADD. (v-aso-

• ). - ·~:Oll pre$SlD. IDJ~~'~-"~

760 S50i 120;

780

4·1

' ....

l

r.:r _ .·Silmm,ary' t2:i),le of tbYTOid dlserders:

, Disorder , Serum T"

~,F'actitlous thyrotoxicosis .

. Increased

Increased .. Sqppressed tilcreased -:

. . . t

TS8· .

1

i. Primary hypothyroidism

Decreased

, < •

RTUff···B: 'R' ....

. ,"'" .'

:. I~I

.. -

!

i

Ii ~reased TB.G· 'Decreased Incre ase . d'· . ':" N' o:rJ"1-.,· .' i!IiI' 1', .

.. 'W ... " ..... ~' Normal

I (increased androgens) ... ..

Increased . Increased

•. Increased

Grave's

,! . Thyroiditis (acute, -s,ubac'ut.e.)· Increased.

Increased

'1 (Hash:irnoto} I Secondary :hYPQlhyroidism,

: Decreased

Increased

De.creased

:]j~ . DeCreased

, -,

! :(h'}rpupi~,tarlsmlh~thal) ; Increased LBG·

Decreased

Decreased

: (e .. g .. ,. mcreased estrogen)

Normal ..

'Norm_al,

. Normal

. . ....

·Il

~

!

Normal

"H.ig,b dose dexamethasone

cr Summary oflaboratory .. studi.e$. io. CIl5:biug_'s SYD'drome:

LtbOnatory!est Pituitary ~J,lShing"s Adrenal'~~biug's

-,S~,rum Cortisol Increased Increased,

Urine for free-corrisol - Increased . Increas ed

Low dosedexamerhasoue Cortisol not Cortisolnot

. suppressed . sup pre s sed

Cortisol suppressed Cortisol 'not

suppressed Decreased

'uto.pie· C·tLI,~a;g',s Increased Increased,

Cortisol not supp •. ·ressc,d

. ~

'Corti~J not suppressed

'.'

"Norm al" to

, . ~ - -

Intrelsed

·cr

Nen-caseatmg 'graD,ulDma::, in an Airic3D.(.ADlerican, with bypertenston- sarcoidosis

'Soft, tender Iump in thejnguinal area o·f' a t'hild.; CIJ1?'torc1;iid, testis, ' .

Kid'De'y of h'yper:tel:l$io:D:n~;hroscl~.sis- due to hyaline arteriolosclerosis

Woman, with ·a,3. 'em mass removed from breast, I 'year later, the lung'. is involved. 'Mec'b'2Jnism ,or',sprea,d?: hematogenous

Increased j,D,trao,ttliar pressure ao.d swe'l1ing~ of ,r'e1lna': glaucoma

Woman wi.t.b left flank pain? microscopic hematuria, CT' shows enlarged bladder 'and 'kl~Dey.

Site o,:{ ob$tnldion?,; bladder-urethra junction ..

. CrateriIorm lesion OD Inner side: 0(' eye, Site,. ,of origin?~. basal cell carcinoma- derivesfrom basal .cell layer

Parotid mass ''Wi~h and without atypiar« with atypia is a mucoepidermoid carcinoma, • without atypia is pleomorphic adenoma

Patient with AIDS'has, '. spsceoccapying Iesloa u.·tlte brain: toxoplasmosis

,C7"

'mGH' YlE~D NOTES ANATOIVIVc

Qr Arteriogram of upper arm with 2. history of au absent ndial pulse: damage to brachial artery-

extension of the axillary artery' and divides into -the radial and ulnar artery tr 8ypoglossainerve ill brain stem: exits in-medulla at preoli~ sulcus .

7 Cerebellum: Purkinje (rabies inclusions), mossy fibers 'in lower cerebellum

~ Spiual spinorhalamic tract and medial lemniscus: both go ~o ~1~1JS '. .' _ .. ,

rT Typesof junctions in lateral region 'of cells: " zonaoccludens (nghtJunlmqns~~. zona.adhereas, •

desmosome, '. gap junction (nexus. passage .of materia.ls, .dye passes from one cell to the next)

~ Leaky JUDCtiOns: presentin proximaltubules ofth_elddneys, .... .. ,.

f7 ,Acd.n:,present in: muscle ,in ips but ~e.nat responsible for motility ,m spe:am

42

~.

:r'

~.

~. ~.

:r

Si,g'ns of rig-:'bt hem is ecti on: • bilateral loss 0.£ pain and te~ . ..,,'t"""" t::',~r,"'!IIh, '':'.L.;~ d

" " ", " " .... t' ...... u.~1.iL4~ ·.n... .. ~uon W1.U.~Ull tT'lua " "f

involved segments (crossed spinothalamic in Vetttilcommissure) •• ipsilateral it.ceidvi .•. ~~ 0.'

affected myotomes (ventral horn), • contralateral 1.0:" f', "'!,, :, .. , " '., :'. ~ .YS1~ In

,.' ' , ss. 9., P' 31n, and t~· . ..,.·'11"'" ~~iP .. n ..,.,

' "] I ,_ .. L. al '. ,. - ... ~~ !lii 'W.I:._. ~"'1,..3G on one'

segm" -' e·nt. 'below lesion (," atera ,sp. l~1.0u·:1a arruc tract) • ip S··l··la-·~,· "'·1' soastie ·n!tT"2·I " ~ . ~th ' , ..

.. , . . ,.'. , , '." " ~I,G, -~u.' . ,r~~ YSIS W1 '. -. ida

signs Y'oss er lateral corticospinal tn.ct),. ipsiJatet:al 'loss of tac~leciiscrintinati~}ll~'

. , ib ·to ...... ;, S C'·~non' from "'!Ii'*"""""'" and leg (I . f" . , posrD.onaJ

S,ens.e/".'ra:·'l~~ .. en~. '" .,: '.~"I~.·' ,,' , . .,~S$ 0 'postenor celurrms) : ,.

Brain stem for h)(::aliz:.Uion of eN VD.: patient history was p;snlysis of orbkulari culi on - n4. .. side and buccina tor and facial muscles .. on right side " .", SOC, ,I on n~,t

BnIi,:n . stem . ~o r loealindon ·of eN VOl: • history of senSorincW'al hearirig loss, •. located. at

cerebellopontine angle . ' . .

Brain stem {or 10("21iz3;tioD of CN VI: history of p(Oblcm. with lateral gaze- loss offunction of

lateral recrus .

Solitary nucleus:. receives gene_ml visc~ afferents fromCN IX .and X and Special visceral

-afferents rfrotn·· ~ Va IX, and X and projects these to the pons 'and, thalamus

0;teurQ~natomy of brain slices for locatioos OrS~uttD.res- iav()lv~ in dlffereat diftses: • caudate nucleus-oabsent in Runtington's chorea, .lenticuJarnucl~- degenerated in patients with \\"i 1'50.0.1,5 disease and 'is.associated, • 'substantia nigra-esee depigmentation in' ,Park;insonl'S"disease Hemiballismus, in' patient 'wit.h Wil$o.n'.s disease: degenera'rJop of the. subthalamic nucleus

Tabes dorsaliscdorsal column syndrome-. loss. of tactile: diseriminanca, loss of jpint and vibratory sensation, paresthesias and' pain (dorsal root .irritarion), astereogacsis, ,hyp()larcflexla ,(dorsal root

.deafferentation), urinary incontinence/constipationlimpotence (dorsal root deafferentation), Romberg's sign (standing patient unsteady with-eyes closed but okay wnen open)

,CT"of abdomen:' kidney is behind the pancreas '

'cr of thQr-ax: identify.structure-at the level of the: sternal angle"

~MDscl'e: th.at attaches at iliac crest; • 'gluteus medius, whtchaboucts and .. rotates the thigh~ .•. -drstal attachment is greater trochanter of femur

Visual field defect with craniepharyugloma or' ':acr:omegaJy: b'itc:mporal hemianopsia- 'imptil'ges

, ,

on' optic chi as m

Outpouching' G'! 'no~,r of ,dieD~f:p'haloD~·cere.brum ,N eurotransmitter- '0 rra.pht. nucleus: serotonin

Lecadon ln a cystourethrogram, of .th,~·. urog,elii,taI ·di2p.hraglii:: •. muscles ~~ deep transverse perinealand sphincter urethra muscle, • 'in male. 'it is located just distal to prostate, • in female .. probable 3'l beginning of urethra 'when it-exits-the 'bladder

Spinal cord site: [or loss of two point discrimi:aatioD in le·ft leg:. '. fasciculus gr:acilis in: dorsal ~olunms,cO'llerstwQ point discrimination for U1~ ipsilateral leg (closest to midline),. fasciculus

cuneatus (lateral to cuneatus) covers two point discrimination for the ipsilateral ann ..' .

Spinal cord si,e for Iess ·,if paiD s~'DSati.ob in left leg; lateral spinothalamic tract 'on right side :·b(' cord (remember the cross-fibers)

Palie:D,t 'With dyscalculia, tm.ger agaosia, leftJrig'bt. tClufu'g'oo:.· • lesion, in 'visual assOciation cortex (angular gyrus) in. left parietal lobe, , . cal led Qerstmannts ,·svn.drome

· . .. 1

PapiUedtma; swelling of the optic nerve- indicates-an increase 1Il mtra.a:arua. ~ .'. .

Omph.aI&t:eJe:. midgut loop fails to return "lo abdo.tninal cavity, • shiny sac VISIble atbase of

umbilicus

X ... rsv IO~{. duplicated ureter; eongeaital ~omaly

HO~eSboe kldney: .·lower poles fuse, • kidney '.~ behind infe;i0r ~teric~ .. " Nerve cut producing sensory loss DD. medial sid~ of I~ when .. takb:i;g sap'beaous velD. for CABG

prncedare: saphenous nerve, which 'is a branch-of femeral.nerve

~' ~.

or r:r

.~'

~, ~'

CT ot abdomea: st.ru,ctD,r~ that splenic vein empti6lato: • en!pties intQ """'_1 v'. I_ .... "

'.' 'p'V" ~ " ", d ' C· . 'I .' ,'" d ,~. ~ .. ~ em ~$ten,or 10'

neck of'pancreas] ••... IS compose otsp erne vern an SupenOf mesenteric: vein (1..

interior 'Mesenteric, vein' ends byjoining the' splenic vein . - , argest vessel),.

maar nerve '{aDeno·Ds,: .• medial epicondyle injuries, • nonna_lly, _ ad..;a~",_,..~ fin get·s du e" to' _', _' " -.

- ~, '. uu~~ -, _- , ... '.. ' mnervatlon

of'palrnsr and dorsal interosseus muscles, .' adducts thumb- addUCtor pollicis muscle: ' '.' .- '

."L·JC'ble'~iDg pains _~beD·collg_b"."g= '~ue 'to her:tia.~, disc conljJtessing. sensory' root

EM of plasma ceO: ~td: antibodies, ~n::uncnt rough Citooplasmie reticulum, eecemnc nucleus with cartwheel a1?pearm~g nuclear chromatin -

CiW"aderisdcs of ~ostg~DgliOllic peripbenaJ Bervoo, ~emraben: '" shQrt. • near end organ

Duodenal ulcer perferation and bleed·~ gastroduodenal artery -

M1lI 9f I_OI~, i'oint in. 2 patieDr witb fa po'si,d,v'e posr~nor a.raw' sign.: identify .posterior cruciase

ligament .

Bico,ru'uate "uterus: failure of fusion of pararnesonephric duel

CN· V' • L,_"";:' gh . -L. • tal fi-

~ --'; gOCSULLull:- _ . supenor ortnta . 'SSt.I:(C

Md&toD.iD·: produced in the, pineal gland

,A\KIo'miD:U iD·~.ioD_.to, avoid iD,terr-uptiJ;a.:g bl,m_$.IlPPly! midline

'Qeft' palate; maxillary 'prominence fails to' fuse.' with medial na'sal prominence He~topo-iesis. at ,28th week: bone ,JnaJ:ro.W main site

Sc'Bwau ceWo.ligodeD~dr'ocyte: • Schwarm cell makes myelin for·'PNS oligodendrocyte, for CNS" '. $c.h~ cells develop from 'neural crest cells

Location of neuro:b.yp,ophysis in, thE:' P.igaitary,:, neurohypophysis is, posterior lobe where: .. WH is stored

Loss 0':( paia sad temperature O_D side of (ace': CN V lesion

-n' '. "'" f d a1 .:.....;..1 ,.i_ _ __ I ,.. (S 100"" _. - 1;..'

·_:envaaoo· 0 a r'eu', meonua; .' .neurai crest ongm ,::,- ,anngen positive) •. '. neuroblasts

develop' into ganglia, - know the' layers of the adrenal glan,d, from outside .in fromthe cortex tQ the .med~1a--, .glomerulcsa (aldosterone), fasciculata (cortisol), reticularis (sex. bormones), medulla (catecholamines )

Tibial <~e'fVe ,tun, coo ,D";'. • plantar flexion of toes" '.' injury causes- .'- Joss of plantar flexion, •. foor dorsiflexed and everted (calcaneovalgus-cavusj, • sensory loss on sole of foot

Co~mpoS~tio,D of aertie valve (also p:olm'oaic valve): • lined by' endothelium .and have' abundant fibroelastic tissue plus a dense cellagenous core, ' •. avascular, • MY' and TV havea loose connective

~~_.,;f f d -- .. lfa L.': h- .' .' :..--. ...... .J. "' ''II· "d" val' la

tissue COil: 'CO,I"~ 'Q,' ermatan sur .. te wrucn IS increased In' mJu.i4.U-.'I;n'C).lW.·,'.·ve. ~~pro _ pse

(myxomatous, de generation)

Break humerus, wris-' ,drop: radial nerve injury

. , " • '" th' I th.. 1! '.,

,P·os.t~ra·dica1 rrU.stedo··mY--'winged, scapula: indicates IllJutr to, e _oog·_ oraCle D,erve;' 'paral,YSI$

of the serratus anteriormuscle .

Pa,beD.t with::a nosebleed :aJI:d rhjn,orrh'u,: fractareof'eribriform plate 'm. ethmoid sinus,

'M'ed121 loilgitlld:inal fasciculas dem,yilinaoo'D ia multiple sclerosis: b,iWCral. mternuclear ophtha:1mop legia

P'arsdtyroid' derivatiob: third-and fourih·pbaIyngeal.poucbes

, .,... fi' 'J f '~llary·· . - " -", - - ,...I ~~~"lIIl ,- rd bva"d 'is"''''''' .'

Ao,rdc' arch de;nvatiVes: • nrst- partot 'rna.Xl .":. artery. ·'·'SCCODu- .~~ :aDi_: -,Tv'l.1.' "'--'/":0'

third- common carotid ~ and proximal portion ~if the lntcma1 carotid .artery. • ·fourth-· aOl1K· ,~11 on the left. and' proximal portion 'of the righE SUbclavian artery' Oil the, ~gbt, • mth- proximal portion ofthe pulmonary' artery 011 the ,left and the, ductus' art~rtosus

:Nerve' tba't;" :ru..D.S alo,ag the 'radial art,try: median nerve . " _,

ArterY affected :ir:a femo"raI beck f~Ctare';at<!dial femoral circumflex artet;y-' ~F' ,leads to

asepne.rrecrcsis-of the; femoral head __

EM ,of egg~ where does sperm, penetrate (Zona pellucida]

44

No,~.t:·: thi$ 'mI.,·r~ri:l1 is: ·c~·pyriC·~,tect -. All· rigb.ts·~.~rved. Eclw.td QoIiaa.:MJ)· .. 2.0'02,

. ~ ~ .

~.

.....-. ....

,c:r

.;;;-

........ ....

'_',

,..., .

.r;r

Types of co:Ua:ge,Ii.: • I- • bone, * tendon .. * skin, •. gt.e.a1e~t tensile strenft'1L~ • In ... _ '* :', _"-.". 1-' '., "

• ~ • •• • • '., -. .'. >C. • -&~"" "., .' mlttt, collagen

of wound repair, . ._ replaced by type 1. 'VIa collag.·.c:.nase wi.th Zn as a' cofacto r .-. IV.' .- ~_.~, -',_,: _

-- . , '. ~.. - . -. u-~rnent

membrane. • x- epiphyseal plate .'. -'" ~ . . '.' ~> ' .

WaUerian degeneration: • Schwarm celts begin to' proliferate and form a tube'thatWiU serve to guide ~0n sprouts in ther~gm~tion p~ocess. ~. ~~eneratioo of the rIervcQCCUCS by &, olltgrowili of multiple axon sprouts f!om the proximal surv1Vmg segment Qf tile axon, • sprouts atedirectc:d distally (growth rate of 1~3 ram/day) dawn the tube established by the proliferating Schwam cells. • sprouts are rernyelinated and reestablish continuity with the motor end plate oftbc muscle . . • Circle of Wi:llis_: name-arteries and: .. knowhow 10· idcn.tifY them on an angi 0 gram

cr Ilver: hepatic. vein drains into the inferior vena cava

Eye closed ~2"DO~ open aDd eyedeYbted downud Ollt: oculomotor nerve palsy; ey~,doWD.Dd i.n: trochlear nerve palsy

Blunt iiijury to th~ back of the throat: potential injury to the injury to the cervical sympathetic ganglion- ptosis and meiosis ofthe 'right eye:

Vertical.diplopia:: cranial nerve IV' palsy

Padent wi~ .b.~~.ldac.&~ and p.h.ysieal'flDdiDgs~ of'mydri~ in the .rtght;··ey.e .kt.·.~t:I,OD. with mUd :lld. "I~g,.: and de:vi~tio·D :or the ey~ d.~W·D. a.ad 'o·o·t·: an aneurysm compressing crania] nerve m-headache is the. giveaway for aneurysm

Patient With. a. r«tlll .hiStory: 0:( baetertal meningitis b .. as h,Clrii~Dial dip,Jo'l'ia in tbe left eye, ~.hi.c.h· is "tors·e· 'OD ~:aze to the I·eft: cranial nerve VI palsy- lateral rectus w~ess from 'VI. nerve palsy

Patient with bllateralIateral rectus m:n.~cl.e weak:Dess:~inCn:ase- in intracranial pressure- .. classic sign, •. papilledema usually 'present

ParaJ:ysis of upward g$ze in . an iDf~t: ., hydrocephalus .secondary to stenosis of the aqueduct of Sylvius, • this' is. called' ·Parina.ud-'s ·~vnd:rome.

t~fultip:l·e oCQtar motor' nerve diserders: diabetes mellitus> C·O~. cranial 'nerve palsies from . osmotic damage to nerves

'Weakn$ of~the quadrfceps muscle and an absent.kneejerk re.fles.: herniated ll·-L.·disk.

'Fall on ouistre..tcb,ed, arm wi~tb 'pain in the middle and lateral pOrtio. of clayid¢ and ~pper' extremity remains in atidDcdo'~ eneas·io·D, and iD'_entaJr'Ot2Q,oD: ,~ nerve injured is C's--4- Erb-Duchenne syndrome, or superior brachial plexus injury due to a, clavicular fracture (most common fractures in newborns), • C:I~1\ are' inferior brachial plexus injures or KIUJ;npkc'·s syndrome

P:ati:e'nt bas' p'antlysis' of "the ::ocaJ9m,9·tor aerve after a 'bead 'iDJary:: uncal 'herniation with

compression 'o~f the 1I1rd nerv.t-, ptosis of eye, mydriasis

Nllmltness t),ftbe. thenar' 2Spec( :of tbl!: ·haJId·:'tned.ian n'eTVC (carpal tunnel) W·rist boae wrttb ·greatest· '~ddeoce o.r .8$cpti.c 'aecrQ$is: scaphoid bone

Fetal clr'calatio.n:' ductus venosusand umbilical vein have the highest" OxygCD~·Contcnt. .

. M'·, I" aJ'" I' '!IIth . hoGlU,' fiitn. II:' ..... 1'!"'ttI't_. (IllDeI'Uatr bocli'es Isurfactaat'I:)"

E.- :c., 0', -: .. ·veo· ,u:s wt.·. :macrop e, :'_3-re.. . ·pll;eQm.o~J.~.~0~~ » - :. ',,:' ~'" : '.. '. .

EM' of small bo'wel: look. for microvilli .on the surface . .

B;sm'IOgi~' Section 01 seminiferous tDbulte: Serteli ~U~~. sex hQnno,nc bindingglc:1bu1m and also' .synthesizes inhibin, which has a negative feedback. WIth ·FSH

.Ban·dS: .i-u s.keletal muscle:' .A ~3J;1d has, myosin ~TP·ase.: contracts

Respiratory brontbirde: • last airway stni.cture with ci1ii:.,.,~ ~ryunit ·whete gas exchanae

OCC·lJlS· is the respiratory bronchiole, 'alveolar duct, and alveoli 4' _ '.. r

Termin21 bronchiolE$: • cilia but, no goblet cells, • site of obsaUcti9D In ~ cystlc ~~1S.

. .' . j, ~.. _. . .- .. _ 11 . _. L~~ l·· ... ~nQ. due f'~ ,-rAl'le" ~"'clri.Dg a·f

and chronic bronchitis, ... SIte wbere turbulent au . 'ow ~11~._.Glu.u ..... ' . ~ ~.-:.,:./. " WiUl. -."" .. , .

the airways ~

r;r:'

- ~

,..-.; ~

, "

:r

, ,

....... "-II'

'(7

No.nuu E,," of ,a cell: I! know ,,~lr thc::' normal organelles in "a cell ,. G" "1" .. ' '". " ."

' , .. ~ ,0 IP. a~tus

~pos~.sI3.tio'nal (t1th:9djrja~On of ~ous ;tompqunds. • tough endoplasmic telicUium- protel~

synthesis • smoo .. ' eneoptasrrnc re(lc'uIum- • site ofcytt>chromc' P4S:0 . ,-'," "'"" " .. , -, steroids,: site ofy-glutamyl ~r~ . ., ..... "' .. system. • synthes.is of

Vo,lce. boa'{Seiless '~st ~by,,~~,i,d s'~,rgery::' injury'tO" laryngeal. nerve

MRI of Qr,bit~ locariorr of the superior oblique muscle

,MRI or abd:om,'a~"splenic artery isabove f,h,e, pancreas

Cbn~t :r.":,ra,Y: fluid in the costophrenic sulcus :'in<CHF

X~ny: 'Willi. ellbargem.e~! or Ule posterior ~urt: .:cnlarged left atrii:.up ina patient with mitral ~tenoslS:." most postenorly:l~catcd chamber in the heart. • sec best with ~sesopha8eal uttt3sound MRI Qf the carotids W'1thQcdqsl!)D,(Jr da~ Ulterior cerebral artery: wquhleffect the

contralateral leg' ' ,

S(:"_w~DDom.a. In jupl'arr~ra1Den! • weakness o>{~atel1osi gag ref)exllatrngca1 ~lysis (X), trapezius/sternocleidcmastoid (XI). • loss taste: sensatien posten or third of tongue (lX)

'Weber sya~ro'Qle; • .lesion of oculornctcr nerve and ,PUN' 'signs, • usually, associated with a

midline, midbrain, lesion '

Bonaer's syudro.D)e: • diagram of vert-ebra and sympathetic trunk, • pick ~or cervical syrnpathetic ganglion

P'Dp,ij ligh'f reflexes wl.tb .eye diagra,ms 0.( pupUs: oneset is an O('ulomQ,to~r nerve lesion 'with, the. light in the pupil in a. down and.out locaticn

Bl-uast-em :a"lIte,rior 'View': 'find. area of oculomotor nerve Fr 9 Dial lobe Iesion: affects personality

Infertor q'u.d'ranta'ilopia:' defect in l;h~ superior fibers in 'the parietallobe

LoSS' of 5ellsa:tton in, tbe b,2D~, history' Qf"burn,s 'withoat 'kD,cfwing it;, syrinLgomyclia in cervical

cord and involvement of crossed spinothalamics "

'C'z,.t.ransectioQ o~ ras'dc:-..I,u$: g'n,dlis,:, effects vibration and fine touch of lower c'xtrenuty only eN' ,m and trM::l'l sigas O'D. op~p().site' side:, midline midbrain lesion

U;,S$ Of'P2iu ,u'd tempe,F*hlr,e, and, ~"signs 0,11 oppO iit'e "s,ide:, mid pons l(sl,ora

,Homer's ,syad,ro'me with,' ,localizsti,O:1I oC'I',esioD' 0. '. di2gfll~~ lateral medullary ~droine with associated cranial nerve 'palsy in the medulla and h~'tf13.lam'us, 'with associated' tctrtperature regulation problems

PatkiD'=OQ~',II~~: 'MYIP drug of abuseassociatioa- derivative of meperidine Cross-seedon of u. elDb,ryo: identifyneural crest tissue

:BI,o,b(f, produ;~o,. p'rior'I,~ blrth,~' yolk-sac-s- Iiver-ebone 'marrow'

Boc'h'dalek he,rnia ill ,pos~te'rolateraJ, part of 4i~,p,h,ragm ,00 Ie-itt • ~t early in life, ,. visceral C,O'DleD ts extend into the chest .cavity causin'g' sever C ,n:sp'iratolj' dis,.I:I'~ at b'irth,. paraslemal

'diaphragmatic hernias extend through 'the fo~ of Morgagni beneath the sternum and do not, usually cicv"clop symptoms until later in life

Art"FY ,.$SOdated with fortgat, rtUdgut; hiDdp:t: • forcgut- celiac, • mjdpt~ superier mesenteric, .' hindgut~ inferior 'mesenteric'

Da.ulage to .eariDg ill • rode. aDd roO bud player. injury to cochlea . . ' .

,Small'vs ,lng't, :bo'wel: plicae semilunans go around, entire circumference.of small b(,wel and, arc'

d ' ~"-...I. 'I' bo 1

interrupted 1ll :,arge ..we . " .' . ,

Am'lI-Jtob~rts(f: .. papil; accommodates when 'patient follows finger mOVinZ' towardS, the nose ,bu,t

does not react to direct light; ntUm$)'phiJis

MRI: of"liver: hepatic veiD, __ -vessel ~'~g into the in(mot vena' cava

Patb.ogeueSU of liytJOspadias: fawry closure of urethral fol~ _ . ".....". .." .

Pa:tb.ogenesis of epispadias; 4efcc.t in genital tu~le, asso(:t~~dW1~~trop~ofbl~ Feces draining from limbilicus in 4 day old: persistent umblh~. (VltcUmc) sinus

U riD.,e dr-aiDing' '(r~'m umbWc:Qs ,i:. 4 ~y 0,11.1:, persistent urachal S,~US,

46

cr femoral neck fnctur .. : -bleeds. una the capsule and may colDproniise medial ff'!U'lQrllcin:umf1ex artery leading' to 'avascular necrosis

:7 EiJJ.bryology of miUeri2p .duet, woUT...a,n duet, urogenital sinu: • strucrures derived 'from me

Mullerian duct indude- • fallopian bJ.bes~· uterus;" cervix, • up~ 113r~ of vagi'1a. • in male "fetuses. milllerian inhibitory factor (MIF)'Quses regression of the mullerian duct hyapoprosis •.• in 'male fetuses, the paired wolffian (mesonephric ducts) fonn the qljdi~ymis:t seminal vesicles .: and vas deferens+ • testosterone. controls this. • dihydrotestostcr.one 'develops the extetoal gentalia of 'the male fetus and .the prostate gland, • in female fetuses, the wolffian duct ·no.rmally'regressesinclusions may ~ist fa form cysts (e.g.;. Gartner duct cyst in the, v~ginaLcervlX)~ • s·trucrure.:$ derived from urogenita! sinus. include ...... It· vestibule. ..... lower W.rds of the vagina

'T' AnatomylhistOIOgy (Jof 'ovary: • lined by coelomic ,epithelium- surface derived ovarian tumors derive from- this (e.g .. j serous cystadenoma), • outer cortex contains the .follicles ~,~ medulla contains mesenchymal tissue and steroid-producing hilar cells=strcma] theeosis of the hi.l3r·c:e:ll~;· leads to hirsutism, _. ovaries 'in a postmeaopausal . women. sho'(.dd:.'n.ot be palpab·le- enlarged. ovary IS-

cancer until p·ro:vCl .. otherwise .

~ Oogonla .at birth: • .arrested in pro'phase I. of meiosis· and are called 'primary oocytes .. ~ arrested celts are diploid (2n). and have :46' chromosomes

,. Primary oocyte: • primaryoocyte completes the firsr meiotic division 'within, the maUU'C folliele shortly before ovulation-s secondary 0QCyt~, • secondary oocyte is. haploid (In) and. contains 23 chromosomes, ~ it enters the-second meiotic diVis.loD but is arrested In metapb'aR JI- at'Qe :tlme of o.vul2..tio:14 .• me' secondary oocyte completes meiosis Il during fertilization

~ Anato·m.ytbistology of cervix: • cervix :~. endocervix (mucous secreting columaar «'llS) + exocervix (squamous cells, begins at cervical a'sr' squamocolumnar junction (5.0) is w~. the two epithelium's meet, ... endocervical epithelium normally migrates down into the exocervix and replaces the normally squamous epitheliam with. JllUCUS secretingcolumnar epithelium- '. coJuiriz,ar cells' exposed 'at the cervical os undergo squamous metaplasia (called the transformation zone) •• ' ,!:h·e 'TZ;' is the area. where squamous 'dysplasia and cancer develop

f7 .B:ibteral Internuclear op'hthalmo'plegia: '. demyelination of medial longitudinal fasciculus •. pathognomonic of multiple sclerosis, .: when th,e:·.pati~lt is asked. 'to .. look right, the 'right eye: moves and has jerk 'nystagmus but theleft eyc~ is. ,stillloO'king straight ahead, .• 'when the' 'patient is: .~ to lOQ~ left, the. left ey·e· moves 'and has jerk: nystagmus but ~·e .rjght ~.yc is' stiH, lookinS' strai~' 'ahead;

7' Rctater cuff tear: • common cause of shoulder pain, e· components of the rotator ;~·inc.lude ,the tendon insertions ()f- .. supraspinatus, .• infraspinatus, .• ~ teres minor, '. subscapularis muscles, !' SIS. of rotator cuff inj"uries- pain/weakness. ·with· active .$hoWder abduction

':7 .Sh.olild;er diSJO~tiOD': ., majority are anteriorly dislocated, •. often inj,me 'tbc:. axillary artery/nerve

ar 'T~a'Bis elbow: .. pam occurs in the-area where. the extensor 'muscle tendcns insert. ncar the la~ epicondyle .. • :commQn' ,lI)'_ .: raq~ sperts, • repetitive usc. ofa ~ or .~drivcr

'7 GQ,.Jfer'·s elbOw: pain is located where the .flexor muscle tendoas insert-near th¢ medial qJlccndylc

~ U1n2r nerve cempression: .. the ulnar nerve may be;' ~~ in the foll.owing· areas- • transverse carpal ligament, • elbow' (~~y. bone ~-).'" tht(e. is pain and. numbb~ of ~ uma,t aspect <-fthe forearm, and ring. and 'little finger plus' weakn~ of the. intrinsic muscles of' the hand

;:,- Retl~ex.loss·'in. C4~5 dl'sc:·"blce;ps reflex.associated 'with musculocutaneous nerve c,re

Qr ·h-.I'I· .J I -, ill! C ._r',~ di . · .. ' .'. . .s-

nI:1IU,.~ OSS. 1D. '. s- ~ ,-. sc. Sllp1Il3.,tQr ..

" ReO;ex 1955 ,iii C'-C7 disc: mceps reflex associated. with, radial. nerve ere .•

. ~ ',DeQilervain"s ·d~: •. chronic ··.Slenes.ing· tenos;yrioVitis cf the. first dorsal cC)rnparlllJent of the

'wrist, • . due to' overuse' of the. hands and wrist, ~ ttte· ~ .dOi~. compartmen.t containstbe abd~or pellicis longus (APL) and the extensor pollicis . brevis. (EPB)- excessive lH~tion ~1l$C$ thickening

47

- . ......

~! the tendo-It sheath and d~;tbtnoS1S of ~~ o.~eot1b-lbto~ ~thel. • painocours onthe'ta(iiaJ aspecr of tne 'M:i,st"~(i is aggravstec . '.y moving "Ute. uitm1" pamm ,,:':e:regja:Q of the. radial stylQid process- '

Gallgl~QIl cyst;- ~ommon ~auscur a~Ur$e o~the dursun:- c;,f fht wrist when the wrist is flexed, • I,f&&n,glio:n." ,(synoVl,al) ~y:~l' IS filled ~Wl·th mucmous material, •. some'tImes called the Bible tumor since' a Bible: is often 'used to smash the cyst ' .

Compartment: syndro roes: • ~ ncteas~ of pressure in.a ,confinedspace-pressurereducesperilJ$ion, whichmay Iead to permanent .tscO.C1l1J'C; contractures of the; muscle (5.) in that 'C9:mpa(in1cn~, ~ SIS, of a compartment syncito,mcr-· .'. p'ain., - paresthesias;> pallor; .' paralysis, • pulselessness, .• ' 'c;au,ses of compartment synd.fQrncs:-". fractures, • .injuries 'to arteries/soft tissue, .' :ptolo,n,gQd limb compression (~~g." tight fittingeast), • 'V<ol~ts iscbemic, oo'n,ttac11n:c.-:-' • it IS a c'ompli.cation ot a, supracondylar fracture of th'e humerus, • tbc::rc. 'is~.inj':ury to 'the brachial artery' and me di: an, n,erv.e~, • brachial artery ischemia leads to increased pressure in the 'closed muscle cornpartments of the forearm with a subsequent decrease in 'VClOUS Bn"d, then arterial perfusion: this 'may head to

permanent ischemic contractures ofthe muscle . .. '.' '. .

~~a.rp·al tunnel synd:r()'~e:' '.' entrapment. syndrome of' the med'ia'Q nerve in ·the transverse carpal ligament of the. wrist, • it mayalso be entrapped 'between the 'belli.es ef.the pronator teres muscle along w.ith the radial artery. '. .C3;use:s~ il., 'R.i.\1pregnan,cy:., " oyc:ruseo;f the hands/wrist, • amyloid:osi.s~·, hypothyroidism, ..• S)S, __ , .• pain, numbness, or paresthesias in, the WlJl1lb'jf, index fttlg'ex~, second (mger, third finger, and the radial side, of the fourth finger, • thenar 'atrophy (".ape""

'han;d appearance), .' pain is 'reproduced 'by tappm.~g, over the median, n,trv-e . .

"Claw hand": .' ulnar nerve palsy, • ulnar 'nerve functions- '. adduction of tbe fingers due to innervation o'f' the palmar and dorsal interosseus muscles, ~ adductioa '0'£ the thupID·(addutk).:r pollicis muscle)

""Wr,ist' drop": • radial nerve palsy, .• ' radial nerve fimctiona- • e:xt,enstif' muscles of the" wrist and ,dt,gi.ts" ' ... wrist drop 'refers to a.hand that is flexed at the wrist-and. cannot be c:x.tended,., injury' may be due, ,t(J- • midshaft fractures of the' humerus, '. draping Ute arm' over a park bench (called

• U Saturday' night palsy")

n'Walt'er':s tip deformity": .: brachial ~plexus. lesion involving C, and '4- upper tnJnk: inj"W)'" •

1'"'· I fin' din: t· Erb-Du h "1 birthiai f tho \..-. hi 1'1 ~iIoL . ..,1 ... __ ,,...

c :lrttca. n ~. ;'~ In, '-:~~.:' ,.:'c·:,.e,nne,s )23.':5):-' ' •. '.' , 'mj'llrY ortne ,V44C:, .a 'p exus Wl.·w U.4I.u,J4.ge- t'Q

~CrC6 nerves ...... , 'up'per 'trunk"jnjDry,,·. loss' of abduction of the' ann from. the shoulder, '. inability to ~xtct'llal~y' 'rotate the arm, .' inability to ,supina,tc .the forearm, • ;ab.scnt: biceps rc'fl~ .: a..sym;memc ,MorC reflex: no movementon. the affected. side

Klu,~p,k.ei,$: paralysls: ,. birth, injury ,of the brachial plexus with inj'~ to, the. ClrC'l: and T),-'lo'\1iCr ._' ...... -1..,.." - ." lysis ofthe 1.. __ .~ • Horner's 'syndrome

':u u.u . .I\. tnJury,~". parao.~·.; 1 ,' .. '" ,Ul . l'W:lJ.u, _.,'.' t : '1;1 .. ' ".'.' .' ,. ' '

).;~n:arY nerve JDj~ (~): • fracture of the ~urgiC31 neck of the humerus, • dislocation of the shoulder j.o.int-, '.' usually anterior dislocation, .' may also injury .the .. :axil'l3l}" artery. • paricnl' eum~tl abduct 'the arm to 'the herizontal position or hold the'. oo'riZori.ml position whena ,doom\Vard, force is

applied' to the ann- paralysis of deltoid muscle, '. w~g' 0'£ lateral [QI'taliou, 0;(" ·the~ 'arrn-. paralysis o~f·:to C$, minor.muscle

Arte'ries,. nerves, teD,d,g'os :cuC, 'witb a.. deep. la,c.er2.D.o'li tlf 'tile radial' sider ,of the' wrist:: • ane!yradial arterY" .: nerve- median nerve: courses 'alon,8: the. radial ,artay'i~. tenoons- 4' palmaris lon,~, . ., flexor carpi radialis

Arteries, nerves, te:D:do:D:S cut wlth a deep lace;ratio'D, 0'( ~lIe 'lI.blar'· ,$,i,d'e: ,of the, w,rist: .anery-'

u 1'-il!li"~ ~ r:rery. ':;. .' n. ierve- lil~' ... :- 'n° ierve; • IcndoD-" flexor cami ulaaris

. .L1,l.al, Q.J...... " . ,",Ii'.' . .u~.141 '." . . ti ... -,r

Cause of ~'SIUB splhthi": repetitive loading of the ~tcriof C(J:mpartmt:n.l muscles o(thc ubi!.- • inflammation occurs.artbe rnuseulotendinous insernons: these"arc often called "$.rress, iractu:r\;S'" MC: si't'e (o,r' a~ CQ'Q1,partal:eat: 'QPoD,droDle la, the leg! anterior COmpallliiCnt of the tibia

-------1

t:7'"

...

r,r.

~M.ec;baDism'5 O()OW back paia: ., M~CC is sp'asnl'ofthe paraspmal muscles • ·lim.ftVint,.s"~' . '.

. ,., I" . '.' t. 0-.1 .......... , ,uoms'- ..

.anterior/posrerior longitudina J1gaments.· n~'e root ini,tation ....... e, g. in ·f ...... ,~ .... ·b .. ra··" I d.' ., . 'd: '", .' " .

. ~ . ,', . ~, ",~,.L y~, ~. " ,,1SC lsease'

with nerve. root eomp iressten, • diseases Involving:' v ebra ',J' ""0- 'j"wnn,,:, '- .'-' '~"'-I!'tas-, . " . ~'~'l' ~ 1

' . "' ·.L"", 'W"", ". , .... ""'~ 'IS .. m'w'h:' , .

myel orna, .•. osteoporosis 'with compression fractures '. t '" 'P', e

M~C' C of 'intervertebral disc disus'e: degeneration '. o 'f" ')fj, br'- :n.j<o."':II......;}'·'agein·u· c Ieus 'pu"l: . th'-

. .. . , '''. ".' w, " ' V"atGI. ,U· . , ' .... ~ , , :POS\lS .. . '.,

~turcd. d~sc rnat~al may herniate posteriorly and compress the nerve'root iuuVor sp~~ cord,.:

P~l1l, (scl~tt~a) radiates 'r:o~ the low bac~ to Ih.~ buttocks. down the leg, below theknee •• srs er

disc h,en11atlOn,,....·· te,g pam ,is aggravated by straight Ieg:"ntising ,

lI~ml:.atjon of~, disc : ., ~~?' 10ss- -Iataal and posterior qI( .,' plantar aspect of the fOOt, • reflex loss:~' Achilles'! r~fl,~~. (tibialnerve L.~~J)' .• motor deficit- ~: loss q.(:pIUttar, flexion .. ~, loss of foot eversion (weakness ofpergneU-§ longusl]:)T.eVi~)

Hernbrion of L.~Ls dlsc: • senscry loss- ., dorsum of the.foot, .. webbed spaeeberween the. great 'toe, .. reflex loss- none, .~ motor deficit- loss pf dorsiflexion of the' big (great) toe 'clue to weakneSs'

-of the.extensor ·haIlu,cj§::.IQ,ngus, . .

e"rni2tioD pf ~]-,Lt disc: it. sensory Ioss - medial leg, to tIi(: malleolus, '~ re-flex' loss- knee.Jerk (femoral 'i1'ervc~ ,L~-,~), • motor d'e:fi,ci(~- *" qua,dticeps~wcakness due to weakness Qf knee extension .. , . .. loss ofdorsiflexion ofthe footdue to weakness ~of the tibialis, anterior

Cau-da equina syndrome: • bowel or bladder dysfunction, '. saddle area anesthesia S·pou.d'Ylo-list·hes.i.s;: • forward subluxation of one vertebral body on another •• ' ~CC' 'is:~spondylocsis;, defect i~ the vertebral lamina (pars interarticularis) with separauon from tb~ main body of the

vertebra .

Superier ghiteal nerve, iq,ju,ry! • causes- '. ,5Ut&Q"Y', • Duchenne's muscular dystrophy" •. waddling ~i.i~ .. the superior gluteal nerve 'supplies the gluteus medius and minimus muscles, • there is a loss' of abduction of tile. thigh and, inability to pull the pelvis down " .• there -, is a positive

Trendelenburg's "sign: • raising of the foot on the injured side causes- '

Inferior gl·u.teal nerve inJu',ry: ~ 'patient 'is unable. 10 ''W31k- .. the. Inferior-gluteal nerve supplies the, gluteus maxirnus muscle, * the panentleans backward when, the heel strikes.the ground

J\{.C .. Site 'o,r ,$ci:a'tic nerve entrapment: sciatic: 'notch mthe buttocks

Peroneal nerve Injury: • ,Me site of entrapment is behind the '"knee- .. common in, people wno' cross their legs 'a 10·t. .,.. the padet:lt has a slaWing· gait, .!It motor deficits- .: loss of foot eversion due to weakening .of . tile, peroneus longus and brevis muscles, ._ loss, of foot dorsiflexion due to weakening. ofthe 'tibialis, anterior muscle • loss of toe extension .due to' weakening·of.the·cxt.ensbr digitcrum longus and hallucisIongus muscles, '. combined effect of all the above ~uees an ~au.in'o,yaru,s deformity (plantar' flexion M.~ foot ,drop, ,3JlQ. inversion of the toot), .' loss of'the ankle

jerk reflex J~ • ,

,M·e sire ro'r lateral femo,raI 'Dene. elitn'p'meD.,t: inguinal .. ligament- • entrapment of the ,I)~ produces rneralgj~ p'aresihetig. '. ~:is a numbness. or. burning. sensation over the latcnU pan .of

the thigh when walking .. or prolonged standing, •. it,iS common in obese ~tie.ntS .

O·btu~r2.tot, De~r. iajury' CLi~~):' • motordeficit- the leg ",swings .eut when wal1cfog -sinee 'the obturator nerve supplies muscles that are involved with hip adduction. .' "sensoty 10ss- medial

, .' . f·th· .L':!I'ii,lt - 1-' " f .,1. kn . 'ft

aspect ~ . e u:1:I6u-t'· OSS, .0 ·u,C _. ,ec re,' ~ex .' " _

Tib.bl nerve iJJ.jury (L., ....... g"J: • motor d,c'fi.citS-, .. loss of plantar flexion of the root due to weakening' cr:the' gastrocnemius, soleus, 'and pl~taris,muscl~ ,. loss O,['f1ex.iCnJ of the'. toes due to weakening of the flexor 'digitorurrt longus and hallucis longus muscles, • foot inVcISion'due to weakening of the tibialis pestericr muscle, • ·the above mO~ deficits combine to~~c,C ,~alcaneovalgocavus: Where there is dorsiflex .. ion and inversion .-of the foo!"1 .··loss of 1he. ankle Jedc.

're·aet

4,9

·~'

;z--.

....... ...- '

~emor~ ~e~e JDti .(LrL.tl: • fe~o~ ncr;: inj~occur due to injuty in the area of the ,e:morat, 'tl: trlanthg em' ~, ~g' 'h Cd-u:St, oawc~na: Ukr:m~' g~1tef" :th°r ~'artal!t~bd·O?I·_Punctures, • motor <leficits- • patient canno'., c:x.." .. e", ~,", "'"", ',,," '~ .... ' '.0, , ,e $", 'onus .an, uiaeus m',usc' scles :.' ", 'h", '" " ,,' '~"

, ", , .. ' , " ' """ ,;i '- pa~enl,cannOt.'extend

thc'leg due to weakeningof the quadriceps muscle, • Joss of the kneejerk ~flc'l' ten II l' . d"'" ,~

,-, di '1 f th' th . , ~ kyphosi '"1-1 ' U app'le to a

forward ,'I,'SP, acement 0' r, e oracle,~, nne, '-~ , " osts fl:-.ltrnn 'bac-"k')--" '. PhI,.'_..1 "!IIi,,,,,,,,,, .. L,_:, , · k'~', ' '

IS an increase in the normal posterior convexity '::r;e thoracic ~"'sp' ~U1 -:UJa:th .. , .wa~. ;yhOS,iS

, .' , '" , ' ·t' ",ere, IS, £'ofW3,rd

disp~acernent .~f ~~ spine: hump back, II if severe, kyphosis may lead to chest restricti~nand respiratory-acidosis

Kyphosis in J.d~tscents:. • forward bending of the ~ine •• MeC is mUScular/postural pTOblem.scom.m0nly ~~ in .~11 adolescents; • Sche~mnann 's. disease- abnormal vertebral end-plates leadin r to disc hem I a tl ons mto the vertebrae Called Schmorl's nodes .g

Scoliosis: • termapplied to latera} curvature of the spine. • idiopathic scoliosis- • usually affects adolescent girl~ between ~O-l.6 yrsof a~ ... Usually a right (boracic curve,!Ii fOlWU"d bending causes a paraspinous prommence on the right from a hump in' the ribs due to a to~tiQnal component of the 'vertebra

Test used to evaluate meniscus lajurfes: McMurray test- • test for the medial meniscus: patient is supine-s- knee is acutely flexed .... foot is grasped and the l'eg is externally rotated-s- knee is

slowly extended while the other hand feels the postercmedial margin of the: knee jOint4 a click along the posteromedial margin indicates a medialmeniscustear: femur passes over the: tear, .. test for a lateral meniscus injury: same procedure as above except the leg is rotated internally and

extendcd-e- a click-is palpated along 'the: posterolateral margin of the JOint .

Location and function ofthe 2Dtetior cruciate lig:ament (ACL); ACL attaches the anterior pan of the tibia to the lateral condyle of the femur'- 'prevents .. anterior movementef'the tibia in rc::lation to the: femur

Location and Iunction .. of the posterior eructate lig2men.t (PCL)~ ,peL extends from' the., posterior part of the, tibia [0 the 'medial condyle of the femnr-: prevents posterior movement ofthe tibia in. relation to the femur

Tests used to evaluate the crnciate Iigaments in the knee: II :ant,eriordraw teS,t to evaluate ,ACt-, patient supine, hip flexed 4.5°, knee flexed ·,,90·o~ examiner 'places hand on the posterior aspect of the tibia-e- anterior farce, is,a,p'plied in-neutral, external, internal direction-s positive anterior draw 'test is 'when there, is anterior displacement ofthe tibia, :. RQstqior, O:raw ,th$t to evaluate PCL· __ patient supine, hip flexed 4.5°, knee flexed ,90~'~ examiner' places hand on the anterior aspect of proximal tibia-+ posterior force: is applied in neutral, external; internal direction-s positive

Posterior draw test is, when there is posterior displacement ofthe tibia .

Functions of the m~edial, cellateral ligameat: supports the-medial $ide of the kncejoint-'., 'attaches the medial epicondyle 0,[, the femur with the shaft of the tibia" • resists valgus and external rotational forces of the proximal tibia in relation to' the distal femur'

Functions of··,:he lateral c'oUatenI 1ig2meDI~ s up ports' 'the lateralsideoffhe kneejoint- =attaches the lateral epicondyle of the femur to the: 'head o,f the fibula, •. resists 'varus forces; and, rotational forces" of the proximal fibula in relation ro the distal femur

SIS 0'( '. mebisc.d,$ Injury: • pain, • knee catcbes, locks" 'or gives way when \II3lking. _ .

. swelling/popping of 'Ute knee ,

Medbl meo:~U5 injury: • mechanism of injury- • Me internal derangement of knee !oinr. • ~'.

, . • ~. '" I:':. ba:l1') "", ~""-.A _;.), • medial

commonly part ofa valgus 'injury (e .. g., clipping m ';00("':'1",. • structures camageo- ,' ... ': ,'.'

meniscus, .··medial collateral Iigament, .. r. ACI~ .' positive McM'un-ay's [est~ click on posteromedIal

margin with the knee flexed, externally rotated, and slowlycx~~ .. . . ' ... '.' .. '. > ... ' Lateral meniscus injUry: • mechanism of injJ,Jzy- • varus Injury, • LDJu:ry to lato:al eollateral

i • 'II' M' 'M f 14k 'rV"'\d' la t' ~,I, ,,.,,.n:ri,n: with me' knee flexed.

llg;lmen:t,. posltlv:e"'c""unays. test-, C:IC·",': 'o'n ·~~·cro: ,~u,' ~"·~O~Ii' .,',,', ".'"

exremallyrotated. and slowly extended

50

Note: This material.Is copyrighted. All ri'ghts resened~ Ed"ard, Goljan" MJ) •. 1,OO2

A.CL injury: • mech~i~m of in~~- • M~ ligament injury,. • torn most commonly in a val .. injury secondary to clipping or skiing, • positrve anterior draw sign gus

peL injury: • mechanism of injury- '" hyperextension of the knee seconda .. • to an ammo, : ...... ,

' .' L , _ d" , . ~y r lorce

pushing the tibia In a posterior nrectton .. • posmve posterior dta.w test

M'edlal coU,ate'ra,lliga'ment inj.UI")': torn with valgus injuries

Lateral collateral ligament injury: tom with varus injuries

MCC of ~D ankle sprain: sprain of lat~ral ~e ligaments from inversion of a plantae flexed footcommon in basketball players, volleyball, and football

Me, l~te~aJ ligament tbat. is ~praiDed: anterior- talofibular ligament- very important ligament in stabilization of plantar flexion m the foot

Positions the bee joint is forced ioto that results iu injury: • valgus position- angulation away from the midline, e.g. clipping injury in football, '. varus' position- angutanon towards the: midline

medially originating force is applied to the knee t

Me' nerve Injured with clavicular' fractures: ulnar nerve

,M~ nerve injured ln :proxjmal humerus fractures: axillary nerve

Me nerve injured ln, mid-shaft/distal third of humerus: radial nerve= • nerve travels in the spiral gr roove • wrist drop

~"~ , ,

M'C'C of pain la 'the elbow and ,iD2bility to supinate the forearm in 1-4:yr old: subluxation of

the head of the radius- usually due to jerking-anne fiaridDyatJ.uXlpatient or abusing parcot- ~--

'Me fracture associated with falling ·O.D the outstretched hand: • Calles fracture of the distal radfus- • Me fracture of the wrist, .' radiologically, it. produces a, "dinner fork" deformity of the. proximal radial fragment (displaced upward and backward), III second most common fracture in'

osteoporosis m women

,r;r M'e carpal bone (('3 ctu re: scap,hoid- • pain. in the anatomical snuffbox located below the radial styloid 'process" .• high incidence of aseptic 'necrosis

r7 Me ,hip dislocations: • posterior- • usually due to a, 'car accident with. the. flexed knee in an abducted' position is forced 'into the dashboard, • .limb is. shortened, flexed, adducted, internally rotated at 'the hip, .' danger of damage to sciatic nerve .. • an teri 0 r-' • limb is shortened, • abducted, .. externally rotated at the hip, .... 'neurovascular compromise of the femoral artery vein" 'nerve, .. 'aseptic necrosis 0 f femoral head

f7 Me femoral fracture: • femoral neck fracture- • ir 'most commonly occurs in the elderly male' patient with osteoporosis, '. groin or knee pain is present, • complications,- aseptic necrosis of femoral head due to. damage to the. medial femoral. circuM'flex artery

ar- MCfool bone fractured. after ,a, fall from a, height: calcaneus

::r Me fracture associated with. ecchymoses' 0'( the mastoid, 'b2.Sil2r ,skuU fracture: • petrous portion of the: temporal bone, • otorrhea (CSF fluid leaking out of the ear) may a1so occur],

:r M'e fracture associated with rbinorrhea: :. orbital fractures, • orbital fracrures also produce

raccoon eyes (periorbital hemorrhage and ophthalmoplegia (eye muscle entrapmentj], .

a-" Cross-sectiou of spinal cord: _. location of' pain in right hip. .' location for sympathenc

preganglionic. fibers , . , " , " .

0" ShiDing light ta left eye causes pupU to eoDStrict in left but Dot ~e rigbt. eye. WheJl. slab .

Ught ill rigbt eye, pupU constricts in tile left bat Dot the right ey~: T"I~ht eNm dysfunction O __ O _

,~" " .. _ ._. __ --- Psdenrwtlll 'sp,astldty-a1lrt' .IGsr.,r .. t2ctd~ dlSCtlttlIutrci,1I i.ad viti rato ry' seasatio,a: subacute

combined degeneration in B 1'1 deficiency

:r ,Foreign body i.a alveolus: phagocytosed by alveolar macrophage

7' Site lor ,beminegiect: right parietal , ' _ " ,',

~ Pa,tieut: sm.eUs rubber, Mass louted, ia left temporal, lobe. Vl$QaJ, field 'defect: nght upper

q uadramanopia

:r Pineal gland: site for melatonin formation

....... ...

..

SI

I

Su!per6daJ eereb ral veins: drain. into' superior 'sagittal sinus

Si.re ,odootobluts develop from: • Qdontoblas~developintQ den';" • ...:.Jon'.·:to'bl .... ,~- ~r' .. " .

~....,. 'VU" ".~ts are on mner

aspect 0 f the developing tooth, • ou ter ,pomon deve laps enamel from 3J'ne1~blasts'" , ~'.. .' .'

HIGH YIELD' NO'TES B'IOCHEMISTRY C

Ryper-lipqproteinernias; • t);"e I and V have chylomicrons associated. WIth them. • type n is' mcreasem LDL. • type m is remnant disease (dysbetalipoprotc~ernia) •• increase in VLDr.. in ~

N, .' d rvoe V

" an '. oI'! l'"''''''!' '

-, FeR mecba~m; uses DNA~lymcrase tobreak.down DNA into fragments

Locations of biochfmiCai precessesm cell: • cytosol- ., .gJ)tt:()lysis.* pentose phosphate shunt. • FA synthesis, * glycogen synthesis; • mitochondrialmatrix.-:- .. J,}-oxidatiOQ ofF.As, .. TeA. cycle;. jnner mitochondrial membr.me- oxidative phosphorylation. • both .cytoSoland. Illttochondria- ,. ,glutorhtqgenesis;, .' ,,·urea cycle, .,., . heme ~thesis

Neurotnulsmi~er from q e5.WlltiaJ amino a,dd: serotonin colllingfrom tryptophan

Brain energy-durmg starvation- - ketone bodies, • usesglucoscduring fed and fasting state GlucoaeogeDic enzymes: • pyruvate carboxylase, • phosphoenolpyruvate carboXykinaSe\ • fructose 1,6...bisphosphatase (rate limiting), • glucose 6-pb:o.spharase (deficient invon' Git;rke's glycogenosis)

ltBC ribosOmes: • tost after RBC .Ieaves bone 'i'naiTQ,w,. persistence in peripheral blood produces, basophilic stippling, • .coarse basophilic .stippling signofPb poisoning (ribonuclease 'denatured by

. " . .

:Pb)

p-Thai2S$emi2 mechanisms: • most often a-splicing defect fur mild forms, • severe f)-thaI isdue .toa .stop . codon preventing f3-chain transcription

Man with 2900 c.aIo"tiediet with 30%' of it represeanag fat, how nj3liY gr2DU is fat: .. fat has-9 ~llg,t .' 2:900 x 0,30 ~ 8iO calories.is fat • ,879 .. +'9 = -:91 grams.

V _ and K.; -glucokiriase- high Km (low affinity for glucose) and high y.·m (only reacts 'With .glucose) ••• hexokinase- low KID (high affinity for glucose, good 'for fasting-state) and lowVm (reacts.with all hexose sugars)

EsseD.tiaI r.tt,."· acids (F As): _. Iin'oleic a'ci,d'- • C 1.8~:2ID6~. .. produces arachidonic 'acid" iii, not eardioprotecuve ... com oil/safflower 9~· a:-1iD9Ieoit-· CI8~3cil3. • caroi~plote<:tiVc ,(lower triglycerides, inhibit platelet aggregation, produce anti-inflammatory prostaglandins, less damage to

myocardial tissue in mfarctions.> found-infish.cils, canola oil (best-oil)

1 cell disease: inability to pbospborylatetbe .mannose residues .of potential lysosoma] ~s .located in Golgi apparatus, .hence they. cannot, be t21o:n lip' by the lysosomes to 'degrade' compla

substrates .

NIl~'of glacoses necessary to.bWld pal'mine add':a 16 carbo. a.mpollDd: ·4 glucoses, e:i.th

glucose.run produces 2 acetyl CoA.the latta coataining 2 ~ ~li.,.. '.' ' .. ' .~

Iiasu.wa I2ck'ill DKA: decreased glycolysis, glycogenesis, fatty acid syn:~ storage of fat In

-. ~

adipose

Comp2liso.~ Chart of .the·WelI Fed State, FLui..v Sta~ and StarVed Sate: .', '.

. . - . '. Well fed state - Fasdag state - "Stal"'ml state

. '

-Increased: ,eat'ly sUpp,ly NOne:, .g)ycogen used up

of' gluc9$e derived from ,

liver not- musCl~

Decreased: none in " lhe-.liva".some in

; muscle.

"5,2

.. .~
"tell fed. state .. F&$tlb!. sta.te
! :Sr'3.rv~.d
< .$ta't,e
..
, GI . Qbetlgenesis None 'In '0' eased' .'
I .. 'cr~ ,' .. ::: pnmary . Decreased, .' ..
. uc ... .. . • • . I J.USt enough to
I . . . .
'_ '. I
source Of gl~co~ after .' . I: -.
supply 'RBCs, .
...... I
. glyco~oJ'ysjs •
.. . ~
Increased " !
TriacyIgJy,c'er'ol -s,y lith esis None ,None ..
. . ... I'
I :a.verJa.dipos'l!
I'Q -
•.. ,!'
! L ip'~.l vsi S~ NOlte' mcreased Ulcreased"
.. ~
'S vn thes i ze " . ;
F·ate of .gl,yce:rol S'~b's1:rlte for ... -
more .Su bstrate for'
... I
.' •
macy 19lyt;e:'rQl '. liver _gi'ucone.o:genes·is •
" In .glucOneo_genesls •
~
.. -
13-o~~tiQ,n o'ffatt\' acids, I None Matke.dly
Increased increased:
": .", ..
. ' .,. , ..
' ... - !
I
,. . primary fuel far muscle
·
; Mo·se.le catabolisnr None: increased Increased; supply:amino Decrea $Cd:, ~,
I consctv.e ,
I
i protein synthesis and 'acids for muscle for impo(tlnt '~y I
1 . uptake yof ~o .acids ,gluconCog'ci1e~is.· ,. functions 1
, SV n t b esis/excret] on Remains constant:
U' deamination Decreased
rea Increased: less: muscle
! , ' .. ' '.' ... ~
. .
... ....: . . '.' . . ' ~ -. '. .
- handles Nrt.~ loa4 ofamino acids . d for breakdown
.' use of protein with
. . , .
from "protein gluconeogenesis less amino -acids to.
degradati on . I J
,gut-'by ~ . . urea .synthesis
- in d' d
I Increases egrade
:
ba:ct~~ ~
~ , i
., Ketone bodv synthesis None, Increased ,Mark¢dly increased: by .. , ~
...
product of acetyl Co,A i
"
!
from mcreased P- I
, , .. _ ,'_ ~ . '_ '- .: " I
I oxidation of fatty acids
, "
I Muscle 'use, of ~J.ucose·for·· Primary fuel Decreased "None: mainly uses fany ·
! . '" __ ' . ' .', " _ -: ~
! (Q.el acids
,
.. ,
.
.,
Muscle efIatty 'acids None ,. fuel I Markedly increased,
use Increased ,", pnmary
I ' '. ~ -, ~ . " ....
!
I fOT fuel ~~ fuel f
I
J
.. r
• . Muscle:' use of 'ketones , None 'Some: alternative fuel None: ·alJQW$ thebrain '[0
l -
. . . - . . "
'" rO'r fuel ketones' for me]
use "
·
_', .. '
for Remains .. constant 'R - .. Decreased' allows ·.RBGs I
.. Braln US'e .of glucose .emains constant . -"." .-' _" -" . ~ I
~, " ., .. _" , .. i
"
I fuel to primarily 'USf;. glucose: ;!
. .
I forfue] I'
I
. '. ..
~o,n~ . fuc'l
! BraiD use-of ketones (O~ ,None. Increased" pnmary .
I .. " "-:"_ "' . - . 1liii, ..
. . •
I :fuel ·
.~ ~~
; ~
.. .. . ,
1 of glucose. for· R " constant Remains: constant Rtmam.s"C'()nstant j
RBC' emams
. , use 1
I . '. - ... '. "' '" _ . - . - ~"." " . _:
i
.fuel·
.'
~ S'3

: 'Type .n· I .•. AD. disorder with. premature

• Familial. hypercholester- . C.A.D~.·' Ach.illes··'lendon xanth-

olemia . omas pathognomonie, .•. Rx:

'PathogeaHis:' 'absent or . "starin" drugs

defective ID.L. receptors ' •. Acquired causes': diabetes

'. . . - ~f

, hypothyroidism, obstructive

jaundice, progerlClon,':' in. birth control ' .• ills.

:'T)~m

I. F3Dli.1iaI dysbetalipoproteinemia "remnan u disease"

. . :'.t'. L •• "

I ." • "'~

. .. . '. .

Pathogeaesb: deficiency of aeo E. Cbvlcmicron

~......- ~

'I an.": d !D'. ·to . -rem! an'" .. ~ . are not,

· ., ,. " .. .1 ._~ ..

metabolized in the liver,

· . ..'

, II' Incr~:ed·C·.Wtis~ • hyperuri. cemia, .• ··o'besity •• diabetes,

T· "-",'"

.,~ .1;.::":'

.,'_r·-

F ............. ·:: f' a]

• armna,

hypertriglyceridemia

. .PathQge'D,esis·: decreased : catabolism .or increased

,

: synthesis of VLDL

""""'d· td

'. '4-".1-i ' nso . ··cr~ • most common

h."t~l:fnn~,teine.m.i~ • T'C;

~ .. r-...!"'8 ·r ..... ~r- .. ~ . . I.. •

~ ¥II ~. in "'-'-"-1 sing 3 ... t pu~ .1.....-..-.;;, ~ '.

I IV\-_~.L.I..~ . ~ ,.. .. ~.........,~; '.' I' • -IJIf;J. "'Y., .

incr eased incidence of CAD' 'and

oencheral vascular disease .• Rx:

.f"'~.~ . 4. . . ., ~ •..•

fibric acid derivatives, reduce

. . . ,. ..:'J

,+ . h ..J:_ d"- I" bol .. ' . k '

, carpo· }~uJ"idle an.· a COw· U1ta;.C.. .

• Acq " uired causes" alcobol '-'(:'T.n

... .. ... .. ' .. . .. ," ,.'. . l~.a:~

di uretics .. 'R -bloc kers, renal

• -LII[~.

.... "}' .

rauure.

; , "

l

• Type IIa: inere..sed.LDL (often >260

, mgldL,~ ~d~ cholesterol, .normal TG. '. ' ; ~ ~.: ~ ·LD.~ c,hole.m:rol

, and··TG,. . ,

.~

I

. ' ... Cholesterol and 'TG eq.llIlly elevated, .~ increase in chylOmicm'n and IDl

. ~

remnants, • ultraccntrifuga·tion follo,wod, 'j . by' e.l:cc~hores1S .identifies remeants, !'

.'

e. Marked increase m. T'G and slight

~ease, in c·bolesterol .... , saoo'lng

chylomicrontesc 41rb'id ~ • :HDL. dCC1~$Cd. (in~·rel.Qll,sbIp

with 'VWL)~ .

'.

I

Type V' • Particularly common. in

Most comroonlya alcoholicsead D',~.· hYf'O' ...

. familial chvlcmicronemia svndrome:

~ ~J.~

,h.,)! .. ~,.··.· .. ·:·.," iglyc .. ·· eridemia with bd _;:, __ 1· ~";iII!"'

· -'1""'''''''''-'. 3. . 01l.~ pam. pan~'u~

exacerbating factors . dyspnea (~ o~ygen

°P,athQg.f:D'esis:· exchange), hepatospienomega.ly

~ combination oftype I·and· (fatty change), papules QQ. skin

.... ... IV ~hanisms:... ." . .

.: -IC)\D' ~ coronary, artery disease. OKA :.lIE diabetit ~doSis. 1DL .. mternx4iarc dcnsltY,·:~ lDL , __ '10·.

demlty 1ipoproa:~, Rx,····tt'CatxXkut.. TO - -, ttMcyi.giyccroL VLDL" veI)'~. dc.i1slty J~ias . '. 7" Rite ,UmldDl ·rescti.O·, •.. :iD.· dJo'lestero:1 syD.theSts: ... JrM:G Co 'reductase:: lnhibited bycho~lcSteIol, ancI

~ d:rup (rompetitive inhibition ~th ~pnic acid). • eazyme coaverts HMG CoA intO

____ ....... 1.--,,;- ~~""'" ~~ ~

:;r a..le lim'id"l readlf;la. iDfan,· add ,5.yD;thesis·: ., 'aceryt CoA carboxyl.ase~. 'crrlJItDe eon"Vens

acetyl CoA 'into"malonyl CoA ,_~, .. h __ • -_.:,._.

(7" RaI4!: limiting reaction ill ~iidatio. ·of fany acids: .. camitinc ~ltrmsferasc l • qtzyme in outer membrane of mitochondria removes' acyl group from fatty acyl CoA and h3D-4ifers· It to

camitine (acylcarnitme), • ~ membrane .. ~ ~YCS aq',l group from. aqlcanUtine aDd

tran;S·ftrs i1 back to ;C,oA tc prodUce fatty 'acyl CO.A .

c:r Rale U'miriDg 'rea,cOQII ~ 'x:tycolysi:s; •. phosphofructokinase· .1, '. 'enZ)1nc. CODVcns '~osc &. phosp'hare into fructose. li6-bisp,hosphatc.·

: - Markedly increased IG with nee.u·"] ': 1D.~ '. st.mding·.chylomIoon test:

:. S'Uplan&te and infranate

,_

,:r

.;r

,7'

";T'

Rate lim'iting' reac:tio'D in u'luco-D,eog,'ene5'ls· • c..... ... c',to",' ", 1 £"L' 00' ""',-_

" ' b, . ,~,U,U, se 'IU'4Ulsp' sphatase • "" "

fructose. l,,&.Qisp,'hos,pMte to fructose &phosphate' 41 " enzyme convens·

Rate ~~~I reaction in glyrogeD SYU,thesis: • glycogen synthase ••. ' •.•........ ' •. for .._ .. gl~:C~SldlC l,inbgc:s ~tween a glucose umt from UDP-glucose and the nO=in8~ oaflA existing glycogen chain " " an

bte 1imi~1 reacdoD in glycog~lloIYsis: • ~iver Phosphorylase. • enzyme cleaves a .. l,41inb ..... (rel~ ~~ucose l-~ho~haJc) bUI stops working four glucose units from the branch point ges

Ra,te liDll~g raCUQDi.n pentese phosphate pathway: • glucose 6;>hosphate dehydrogenase •

G6PD converts glucose 6-ph.osphate to 6-pho,sphogluconate ' , ,

Rate· limiting reactioa in lipolysis: • hormone sensitive lipase, • t"n?Vn>e hvdrol ,,' , '

, I 1 J ~ "fa' ids d 'I I --J .".. . y , ryzes

tn3Cyg,y'<r~' mto r tty aCl ' &n', gtycero '

Rate6mitlng _reactioD i.a are. cycle: • carbamyl phosphate synthase I. • enzyme converts C~ + NIt. ,+ 2 ATP into carbamyl phosphate

R».te limiting re.ctioB in p~~e mt~lis.m: • carbamyl phosphate synthase 1L • enzyme

convertsZ An- + C(h +. glutamine into carbamoyl phosphate .

Rate limiting reaction in purine metabolism: • glutamine-PRPP amino transferase, • enzyme

convet ts PRPP + glutamine' into 51-phosphoriboysylaminc, .

Rate litniting reaction in heme s~"Dthesis: • ALA synthase, • enzyme converts succinyl CoA + glycine mto tS.am:inole'vulinic acid

Rate limiting reacriou in ketone bodysynthesis; • HMG CoA synthase •• converts acetoacetyl Co'A into HM:'G CoA

Uncoapliag agents: •. render' the' inner mitochondrial membrane permeable and carry protons ~ith 'them into the mitochondrial matrix. (destroys the 'proton, gradient): e .. g., diDitropbeDoL peatachlorophenol (used, to treat wood to prevent insect. invasion). tIlenooge'ain (naro:ral Uncoupling agent in brown fat of newborns that helps keep newborns internal temperatures

higher)" :. 'rate of chemical reactions Increases to prodaee more N .. 4DB and NADP,B since it, is, being siphoned off into the mitochondrial mamx without the synthesis of A,TP~ poten,tiaI "or bypertbermi2J .' mitochODdrial'po'iSo,DS (alcohol, sa'liC}utes) also render the inner mitochondrial membrane permeable to protons, but the,Y do not directly take protons with them into the. matrix. (not true uncoupling agents): ,J,. ATP ,~tbesis

'Patien:t witb pheochromocytoma: • decrease 'tyrosine (not an essential Al~) in, thediet, 'since it is, 'the, precursor for the catecholarnines, • also decreased, ascorbic acid which converts dopamine in 00'

norepinephrine ..

Lesca Nyhau: • ,SXR VJi,th absent HGPRT. • self .. mutilation, '. hyperuricemia, .. mental retardation G·luco,LiD,ase and ,hexokiDase: ., glucokinase: only in liver .. high. Vm and 'high, Km, not' inhibited by

glucose' 6-phospha~ • hexokinase: in all tissues, inhibited by glucose ~h,ospha,tet lew Vm and low Km

Bra'Delied chain am'iDo. aci,d$ and map'le syrup IlriDe, disease: • only muscle can metabolize,

branched chain amino acids" • missing debydrcgenase enzyme , Locado'DS of' glDoose 6-p110 sp'hataH' (glacoatOgellic Ilormo'~):: •. liver, • kidney, • intestinal

epithelium (lesser extent than others),. Ibsen. in YOIl Gierkets disease . c.' . ..

S'b:uUles aDd, wbal they carry; • caminne+ even chained fatty' acids, • malate and ·glycerol .J ..

- ~ pMip-ute=,NADH .... ·.. , . . ,',

FunctioDs of choleste;rol: • vitamin 0 synthesis in the skin (7-dchydrocholc:sterol) .. • steroid

synthesis. • cell membranes .. , • synthesis' of bile .salls/acids. . ' ... .' . Acetyl eoA uses: • FA synthesis. • CH synthc:sis, • ketone ~ synthCSls" • syntbesis of curate

along with oxaloacetic acid" '.' 1-\01 a sufJ,Sb!~for .g'lucon~lqs'1S,

5S

I. ~

__ , ....- -

Ketone bodv syotbesb:· I:IMG . eoA synthase is the rate·limiting CilZ)'thc • f:i.1\iiO C A I coaverts HMG 'CoA into acetoacetic aci<L. whilCin chOlesterol synthesis. 'HMG' GOA'~.I.,yase

" , - ,. .. ...... 1 ,., .';1: 23 - 'Tn! ,- .- ,,l~uctase

converts, ~m1'G Co'A mto mevaiome ~Clti, • "'" A .l:t- prod;uc:e:ctfer fuel ' -

-- .w

Enzyme ~edcs: • v~ ~~m~,~c.~imUm velocity of an CIl%yrt1e reatti,on When all e " ..•.. ", • '. ' , Sites are fully sa~ted. • Kill (~l,cha,ehs"Menton c0nstan0 of an epzymc rtpr'cSetlts"lbe =

concentration at which the 'reaction velocity isone-half of Vmax (Vt6:axJ2). -1m deseribesthc enzymes affinity for substrate •• increased ,~ indicates decreased affinity 'of !he enzyme'ror

substrate, '. decreased.Ks, indicates increased a:ffinitY ofthe cnzymc~f()r 'subscr3:h: . - ."

CcmperitiveIahlbltors: • V_ is not changed since the, reaction rate is unchanged whether the competitive inhibitor (e.g., ethylene gIyco]. nu:thyl alcohol.) is binding to the active silt! of the

enzyme (e.g .• akohol,dehydrogenase) ~ralcoho~. '.'~ in,~ (decrease ill the affinity orthe enzyme for alcohol), smce the enzyme, IS also actively bmding'with ethylene glycol/methyl alcohol,

• infusing alcohol decreasesthe metabolism of ethylene glYCOV~yla}conol, hence rite effect'oh e:ompetith-e inhibitor 'is reversed bymc(caSmg substrate, itmc::d1(]trexate is also a coit1petitive

inhibit,or ' -

Reversible noncompetitive ia'hlbictots:, - V ~ is d~hQwever the: K... n;ulains the· saroc: •• reversible noncompetitive 'inhibitors bind reversibly (non-covalent bonds) awa.y from, the; active, binding site of the enzyme and form either lmn:a·ctive enzyrnc~irJhjbitor complexes Or enzyme, .. ,

substrate-inhibiror complexes, • 'affinity of the .. enzyme .fOT substrate is unchanged '~'t since' they a ctive binding site .i:;~ unaltered and normally binds with. substrate, .V mu, is; decreased since the' inhibitor .inacrivates Due' enzyme, whieh' automancally decreases the effective cQn~tration, of

'active enzyme '.' increasing substrate concentration ,doqS' not reverse the effeer of the inhib i tor. since: the. inhibitor is blocking 'en.zrtne activity .away 'from' the active .b.inding site, • examples ,of reversible noncompetitive itiliibito rs ·:m:clude. "angiotens'in mnv,erting:' enzyme in,hlbitors ('~s1rate' is angiotensin 0; physostigmiae, which is a cholinesterase inhibitor (substratc:acetylcho'lme);. and" allopurinol, an xanthine oxidase 'inhibitor (substrate xanthine), • irr,everslnle "iDhibiton

,pemianOlUy inactivate enzymes by fonning strong cov31en~bonds~e;amples inclUde Ie.d (irreversibly inhibits ferroc he I. a rase "and ;&,,'aminolevnlinic' acid ,dehydrasf:}~ ·u.piriD (irreversibly' inhibits 'platelet cyclooxygenase), 'and org·ano:p.bospltates (im:,vasib1:t inhibit cholm~:), enzyme kinetics are the-same as those for reversiblenoacorrtpetitive inhibitors

,A TP"COUUt- USing: palmitic .a,cld as ,aD example: _ '.

{J l divide the number of carbons in ~thc FA, by' ,2, 'to arrive .at the number of acetyl Co.A;s 'produced;:

pal mi PC" acid, '= ·16··~nS' .... :8, 3:'Cety'l CQA " _ e , '_,'.' " _ • _ __ ,

(2) each acetyl Co,A produces t2 ATP' when converted "to C'Ol, and H,o m the' TeA cycle!' S·'·,x 12,

:::: .9,6 ALP _ ,

(3) subtract. 1 fro~ll' the 'number of a~tyl eoA produced to arrive at 'the 'number of NADH'l and,

F·~A. nt;J pr--- odue _ . ...1'., '7 N ADH +, '7:lFAl)H~2-

.~rll. _ - ,r;::u... ~ I" .' I . _' • , - "0- '_.

(4) eaCh: NAD.H produces 3 ,ATP': '1 x, 3 ,== 2'1.

(.5) eac'h.'~Jo\D·H'J ·~'u.ccs·.2 ,AU:"7 x 2 ':: 1,4

( 6) total ATP ~;::z, 1..3 1 , '. .~,

Urea cyde: • method of elittrimumg ammonia, • located in the bcpa~ .chrooj;c livcrdi.sca,v~

low' B'UN~ elevated ammonia , ,_ _ _ . . , ' "

EpiBephdDe given and owy smaO braDdied clIaiIJS ofglycogea[o1llld: <k~hct enzyme

deficiency ' .. ,.. ,.,< _ .. _ _. _' _ 'RMGCoAlyasc

Chcleszerol :sytit"bes~::' firs,t fewsteps ,are, .similar to ketone bOdy ,s:ynmes,lS, except -. - ..

is, used instead of IDdG· C:oA reductase

Apolip,tJ:prot.eiD ,100 (liv,er) .u.d,48 (iDtestiD:eJ

I

!

!

,

56

Note:Tbis nu.t.eri:i.l is copyrigbt~. AU rigbts re$erVed. Edward GoIjiUL, M.D. 2001

"~, .... --

:.:r :7!;

:~

....

.~.

.. ~'

Liver can not use ketones for fuel: liver cannot activate'acetoacetate' " "L-,. !'. . " .... , "'..

' " " '1 C A - C-" A' -. -, < ,m lilt nutochondria 'WhICh

~~~::~:~C~;I :~~~oacetateo,. tr.lnsferase (a thiotmJsfctase~) in'. t()T~onvcrt

Pregnan.t "om2D is. 2. beer drinker-what sllpp'leBleD~ does she need' st ·~l],. : ._ , ';.,,' , __ , '_ ,'._ '

'1 hol i . I" . f t" I " , , " '. -' <0 S I needs folate because

8' CO' 0, increases ioss ot .JO' 'ate, In' urme an' d' steo :! whi ieh o ffse . ts .. 'L ... 'amo- "'''''''·t" f &':,'1 ' , ,-' ,-' -', ,--

- . . "', ' ., , - 1.1; - -U"'_: - ~~ 0 Ii - te

Ib$ulia:, •• J<e~ho~,oncofthe. fc:dsta~ •• a~tivates ~hospbara~ (dephosphoryl:ti:njrcsent in~.

Gluca,go .. u. • k,ey hormone of'the fasting state ... '.·ac- ~va' '-.,p pro tein t...:''';'''':I~J!III' (p- hos h - la" )_

~- , '. , ., '!" ' ,U U!.o~ - ~ - AJJl~.·,: - sp , ory' - b n-

Manllose 6-pbosphate: • involved in ~fcr of dolic::hOJ(lipip) in-the RER in the .' ... '':b'' .. '. f . linked glycosides, • \il·vdlve.d in transfer of IVW\SorrieS··from G.' a i'm, app _ am' 'h",JP'I ... .ft tho,· e I' .~. CSlS 0,0-

. ._. . ,J""'_ .. '" " , .Q'" " '_ ...~ ""' . ..", Iysosomes

Maj~r sou r ce of NADPJ.J: • penrose ph~sphat~ s~unJ, •. malate debydrCfgen:ase (tna1i~enzyme) reaction toa lesser extent, • NADPH supplies reduCUlg equIValents

Mllt.;itiolJ cbanges an aminq add sequence-wb.lch OQ.t would have the greatest effect .. ' ~g~~OD 'in ~ serum pr~teiQ electrophoresis,: • ODI! with: the most ne:~tivc charges (m:= ,ac~dle }-~l~~e.. one that would remain closest to the, anode ( .. pole) is the most basic amino :2:cld,-argutine

l\'Iech~~1D :of ke~~~cidos~ in DI(A,: in<:;~cd Il-oxidation Qf'f;atty acids and production of acetyl Co'A, which' ,IS used by the: IIV~ tQ ,syntbestle kc:to'ne' bodies

Promcter' locauon: upstream Ioeation

'Energy source Cot protein synthesu': G']?'

Isoe:ttzym_,e With, 2 genes, 4 subunits: LDB i~es: 5; isotyp(:s: LLlL. !LilI, LLHH. untH, HlffiH

.". .

S,ecoacJ messengers: atrial natriurene peptide: cGMP" insulin: tyrosine kinase, mcotinic: ion

- ,

channels

Best iDCthoo: Gf detecting relatedness of a neVI' 'bacteria: restriction fragment length polymorphism

BiOchemjs~ of hepatic eacephalopathy; • increase: in aromatic amino acids. phenylalanine, tyrosine, tryptophan (mneIIJOn-ic-' P'Tf) leads to increased syn·th es is, .of false neuro.transllli.tters (GADA,. octopamine) in hepatic encqJbaippathy; • branched chain amino .aeids inhibitsynthcsls of false' neurotransmirters- reason whytheyare given for Rx

:E,.neqor i..a; cardiacmuscle: .I3-Oxiliation, of fatty-acids

Lipid facts:' • functions of ant- • reservoir for .ap(J1ipOptuteiris- in the blood, .. transport of esterified cholesterol t·o, the, liver, ~, transfers cholesterol esters to 'VLDL in exchange for

" .

triglyceride using cholesterol ester' transport protein..« bleeD up by' scavenger'reeeptcrs ia the ]i~~

• in pati,e:bts 'w:itb dh,be.Qc: ke(02cidosU' .you :would ~',~,~" activation qf hormQn,~, sensitive lipase. in the' adipose; conversion of glycerol 3-phosphatc in~o.·,Cijh,ydroxyacet,one phosphare, '.1 mthe fasting State. you would expect~ loss oiinhibitioo ofcamitine acyltransferase by inaIonyl.:coA. • in the fed state, you would ~t- • activation of citrate lyase: in the C)1oSoI. • ina,ctiw.tion of hormonesensitive lipase in adipose, + in;~ production ofpalmitic.aeid in· tbe,~qtOsO) ,Firidings, in, ~:~: ... AS <.!iseaseVJi'th deficientl: ofph,ct1"'hdanine.hydrox}!l!s', .• ncWbom~USt .. ~ CJJ:pQSCd 10 phenylalanine O'HY) in the diet before it ($ increased, • ~usy odor, ·iJfOJ~ttle vomiting simulating congenital pyloric stenosis, '.' tvrosin,e,missillJ. beqce.llmust·be suopl,·t;d·m ~ diet. .. can diagnose ()y amniocentesis and fmding- the abnormal ~e1. elimina~ pbenylallUU~ from ·diet;~·'Nu·trasw'eet is,'lmanate' 'aJl,dj;bglylaIaninc'1 SO cannQtYS' It, '. wO'~' YfI~.PK~, who as pregnant must be on a 'plfY' free: die-t.-, atr~,'ted fetus will dcvcl,op' .pc:rQl8ncnt .CNS damaFlrf ulC(2

C;>w1ng to exposure ~t PRY . .'... . .. .'. _. ". . .

Dlsorders of gaiaClose metabolism: • gaJ.actdSe. dcrivts from laC,rose mc:tabobsm- lactose +

lac (.,n·'("'e:____l;. &1, ·u,'CO':r:IfIIII. + galactose • ~lactose:metaboli.sm ,ia~ce ssas follows- .. galact~.+".

a ..... --, ..' ...... , • &"" ' , . . .. ha • ....;~'l

g41acl(Jkinas~ -+ galactose' l-P04• • galactoSe I-PO .. + GALT (galactose .. l.-phosptc -:-.1 .. . , UDP .ft!iI!!IIJ1'II!I. t' • o'w=ose 1 PO +

rransferase) + UD'P-gluco5e--+ ,.gI UC'ose' 1".;P04 + ",' "~~c ose.!"· 01> -', _', ~ c, "

5'7

.~

...

phosphoglLlco.muUlse -+ s.lucose 6"PO" {6 carbonjntmnediate). glucose 6-1'0 +. '1' .' '. '. . phosphatase (gluconeogenie enzyme)-+ glllco5e. • galactQkiJ)a.K dSfj~iene~- • ben'~ .. ' k u~se, 6- • positive urine ,Clin. itest: CIi-nit~st detects all reducing sugars txcP!'tt sueros '. '~h.' ":b" ,.dl$ease,

'S .'" '1" , ~ , '.' " ","n d ., '. ,. , . " . -r e, .. I.C. , IS not I

reducing sugar Q3ctQ,SemI3- .. -l'"U\., ISCa:seWlth deficiency of GALT • ex' '1 ' _

converted .into .. thegalactitol ~p()-lyQl or alcohol sugar), which, like .-.L.1·,t'o·.11 ! :', cess,_ ~l'~'~~O~' ,IS'

.' . , ," " . '. _ u., .>V.ru:. I.S ,osmotlC«l 'y aCQ.Ye':

d3rna~es lens, nerve tissue, CNS~'. liver, .. .exc~: galactose. t -eo, is toxic: elrrhosis, , mental

retardauon, re.na1daroag_c, ,neonatal hypog~ycerrua. (Jack; of glucOSe 6-PO.. a substrate for. gluconedge:ocs~s), • R3 :IS a,lacto~~ .~9Ietfql". the, first two Y!l, • pregnant women w,tb 8Plactosc;mulca~ ~YDthcSJz:l! laetosc m thcllbreaS;J Wlk Vluhc fo.uo\\'Ulg'rca.etions: tJDP"glut;;ose-+ UDP-hexose eptmerase ..... UDP-galactosc, UDP-galsctQse + lactose synthetase-+ lactose +- (JDP

Disorders in fruetose meuboUsm: • fructose metabolism in, sequence- .. sucrose +- sucrase 4- glucose ... fructose; .' fructose, + fructolcinase-+ fructose I-P04• Of fructose I-PO .. + ti/40Lu6 B ...-+ glyteral~ehydeJ.. pbos,hate +. dibydroxyacrtolle pbespbale' (DHAP~ bG.lh are 'lcarbgn

mft1U1edlates: that a~gluconeogenlc substrat~).. • fructose can besyntbesized from rnannose (and vice versa) and sorbitol". fructoseis.an essential nutrient for $j)et'm.stQted in the seminal vesicles, • essenti.al fn,l.etosuri~- •. AR disease 'with missing ·f.ru~tqkinase1.' • positive urine Clinitest due [0 fructose, • he'redrtar:y fructose intotenmce,-· _6Rdiseag witl.l a deficiency 2'( ald.!2ilSQ B ... accumulation of fructose I-PO~. which is toxic lathe liver (cirrhosis) .• fasting hypoglYCemia due '[-0 a decrease in 3 carbon intermedtates for gluconeogenesis, •. severe hypo.pha·sp:halenua: excess fructose lrap's phosphate in cells, ,dep1e:,tion,'ofATP leads to RB'C hemolysis and rhabdomyolysis, increased adenosine-monopuosphate (AMP). which IS a purine. is converted 'into. uric-acid ieading to gout, ... must ,~Iimina~te: 'ta.hle sugar ,fs'uC1Vscl ·frOrtlJhe. ill':.t

Sorb:itol':. _. osrcerically acti ve sol ute that is synthesized 'in those tissue' containing aldose reductase, 'which include- • lens, -. ova, • semina] vesicles (note in tIl·e. bi.ochemical reaction listed below how glucose js· converted into fructose 'in the-seminal vesicles), • :SC;h~ru:l cells • retina. • kidneys, • aldose .reductase converts glucose into sorbitol. and 'sorbitol dehydrogenase -converts. sorbuol into Jrilc'tbse--

NAP H _ ~ADP· N~ADal .

Glucose ----."""". " . 'Sorbitol .,. - .. It, Fructose

aldosereductase .sorbitol deq,ydrogQtaSe

.. 'in h'fPC!&lyc~mlc s·tate.s, like diabetes 'm,eUitus~ there' is an excess of'sornitcl produced-in

the above tissues leadingto-osmotic damage-- • cataracts ••. peripheral neuropathy-due ... to destruction of Schwarm cells, • microaneurysms in retinal vessels due re .destruetioa of'pericyt.es :Bom~lcy·s'tjJi.u·rta·~ • AR: disease with '3 "deficiert,cy. ::Qf cystath.ionio£. smtba}qll • ·.metabOlism .of homocystiIle in sequence is as follows- •. metblollia.e ,+. ,ATP' ,_'" S...adcnosy1mcthionine· '(SAM) +. methyl acceptors ,+. methylrraltS/erase ,_.. S ... adenosylhornocysteine + methylated'·pro.d.uct;s (donates memyJ groups for .1 carbcn transfers), • S .. denosylhomcx;ysteinc .+ .. H10 _. bO~ocysI~e·.,.+ adenosine, .. homocysteine + ... serine + ·~cystJltlt.i.Q"in:e sytrtAtu'C ,-+ cys1:&thfonine, • 1.0 bcmocystinuria, both. .homocysteine and methio ninc· ire increased in' serum, .' :SlS of homocystinuria that .resernble Marian .syndrQ~. (example of' genetic heteto.gcncitY)-· • disl~ted lens. • ar8'chn.od.acty·ly, .•. -eunuchoid, - distinctive [eaNteS of'bcmocystinuria- • iDc:rease.·in plasma 'homocysteine Ievcls leads 10 vessel dama·Ffthrombosis (strokes, AJdI). . •. men·tal retUd;ation.. •

increased, urine homocysteine and i;n,creasetj'·scrwn/urine.tnethiOninc· . ,

AJc2pto~nur.i2. and 'b'trct:dit2'ry 'lY.r,os.i.DO,sis: • metabolism. of phetlylalanine is: as f~Uows- ,'.: phenylalanine + phenyJaJani~ hydrorylas~ (deficient in PKt1);-+ tyrOsiIte. • ,~iDe , ...... ~nydroxyphenyl pyruvate _., homogentisate, • homogentisate + iI.(J1fIpgentisaie O1;idtis« (deficlCIlt m

alkaptonuria) _. maleylacc.to.acetat~.. rnaleylacetoacetate + fimrtJ,rylaceroacetDl ", It~e (deficient in hereditary tyro.sine-rota) -if. ,furruuy·lacctoaceta~.e, • ·fwna.r:rlacctoacetatc·-+ ,acet08.ceta,te

58

'~

...

+~~e (present "l~ TCAcyde) •• ~lkallt2!'uri.a- ,.oAR ~S'! with an a~CC"ofhomopntiSate oXI~e ... accumulation of black, homogenti.$8.te pigment In join1:Sf~lage leads to degenerative jo int disease '. urine turns black when oxidized U1'Vm ~rnt'\~I"""" ·tln 'I'; "':'h'" '.' ~!!Iru '~~ _' ,_,' "

. _ , 'r¥~;1.' ......... ':"':f,""- ~1IotI61W IIo.V ," ,&1-'''''' ".:i:.t!2:!! =:..z. ~1ItOS'1:S- ".

AR disease With a de~cien~y offim@ylacetoacetate h~~tase) • incr~in stnml.tyrosme':

cabbag:"like odo~. " ~l.rrh?SIS Wlth ~eX[Temelybi~~ _inQldencr,: of hepalOCClIuiar ~moma.. • renal disease (aminoac Iduna l~ • death In 'the firsr ,yr ,of life.

L,ys,oso'lil3l'Slo'.rage,~dis'e:as·~: '. deflnition- .. absen,cc'of,de:grading enzymes in.lysosomes: contain hydrolyti-~' enzymes, _ ~ accumulation ,of :cornph:,~, substrates in ly.so,somt: sp'bing,oiipids" glycosaminoglyeans, glycog~,' (Pornpe's d~s~J.,.'.' most Ire' AR, diseases wj,:th the exception ot:'t'wo' diseases, which are 'SXR·- " Fabry's disease, ,. :Hu:nter·~,s disease, .,- biochemistry of' lysosomes __ :.' lysosomal enzymes arc synthesized in 'the rough -endoplasmie reticulum ...... 'enzymes are transported to the' Golgi ·appar<u:ps4; ~es undergo 'post..:translatio,nal modifica;~,ofl~ enzrmes "are' phospherylated ,at one or more 'n1amlQSyI residues to form :marm9.$E 6=phowb~. wru:ch is attached to the side, chaj·tt$4 mannose ~h,6sp·baJ.C receptors :011 the: inn,a surface o:r,me:

GOlgi apparatus membranes b.i~d, to the mannose. ·&.p'hosphate residues', .on 'the tatg:eted lysosomal C~1t1eS'--+' small transporr vesicles arc piached off the-Golgi membranethat~con,utiD l:hcrec,t:ptor-. 'bound enzymes-a- the vesicles fuse and release-enzymes into lysosomes located in the :cy1O$Ol--+; receptors rerum to-the Golgi apparatus to-repeat the prQ.C,C,SS over again.

Glyc,o.saminogi,yc2:BS· (G'AGs) and their disorders: "., G:A'Gs- complexes of predominantly branched, strongly negatively charged polysaccharide' cha ins, with repeating 1JIlits of' amino, sugars (D~g~u~'o.sa.miIte or D-galac~o,saminel and acid sugars (L:-i.,d.luOJlic acid cr D:..gluc,uronic acid), , .chandra.inn sulfate- '. most abundant GAG, "·l·~rtanl' component in-cartilage, -"hcparan sul_fa~ rrtainly responsible fer the negative charge of 'the glomerular bas'~r membrane, • bc:pa.ri;g~, anticoagulant, • keratan .sulfate, • ,hvaluronic .. a.cid- major eomponent ·of ,synovial fluid .(Jow lubricant), ,.' d,etma_tan_ s1J.lfate:~ .' ground SUbstance 'in heart valves: that is, increased in mitral valve prolapse, • increased. in, pretibial. myxed~,·. ~'HurIer~s, ;di~~· AA disease with, a ~eficien~J~ a-'l-iduton.idas'e'; ;., Iysosomal accumulation of dermatanzheparan sulfate I *; severe rnen:'tli retardation, '. coarse facial fearures, ... corneal clouding, " coronary' artery disease: lipid accumulates ·in coronary vessels, =vacuoles in peripheral blood 'leukocytes, .: ·H:unter;.$ d~~~' SXR.disea-~: with a de.ti,cienc}f of :L-id,tJ:ron,osulfate s\dfatase, ._ ,:YsosomaJ accumulanoa of ao._tuatanJ hepar ·~an

sulfate, • milder diseasethan Hurler's

·Sp.&in;,o'U·p,i:d.s and their· ~,rders:. • sphingolipids includes- • ~~gamyelin~ • cerebrosides, .. gangliosldes,,~. sp,hingomye.1'in-' .• involved in the synthesis of cell membranes 'in, 'nerve tissue, ..

'sp'hingo_sine is: the. backbone of ~hin,gomyclm, • sphingosine is used to prp4uce, ceramides: sphingo'sine +, farty acids ~ c~mi'de-, , • ttralnidc'~' phoSPhorylc'boline~ sp~gomye.lm, '. ceramide +. glucoseor galactose-s .glueo-or .p1.acto.ecrebrosideS •• "caamidc'·+'oligoSI.C:charide:s-+ gangliosides, ',. ·T.av-Sachs ,disease- *: J\R vAth a ,dCfici'ency ,of 1:t'exosam.inida:g (a.,'SUbt_.tnit): a, 4, n~ucleOti·de m.sertion leads '1:0 a ,fi¥m,es·'hift, rnutatiQo and an abnormal hexosamiaidase, :* lysosomal accumulation ·of: GeM! gpngliosiae_,' -., commoain ,Ashkenazi Jews, ... nOI lUU a't· b,ri~, severe mental

retardarion by tl months, • blindness ,with a c,~ red spot in 'the:, macula; ';COIDmQD. pic'~ oa U;,S,Ml~, ,f' no ~h,epat;osp'lenomega'ly;: • electron 'microscOpy exhibits ;OJhorled, c,QI1,figuranons, :in 'I ysosemcs , 'that lodle" exactly the satr.te as lam¢l.lar bodies wi'th surfacJ:a.ntr in type D pneumoc~ •. N'ie,m"aDD-l'ii:lt- ,AR, disease with 'a defi,oiency' of ~hingo~lin:ase" _. lySosomal accumulation. ~of sphi'ggog1vc'lin:;' b,ub:b~y appearance in. ~hagcsl neurons, ~. mental. :rewtlano,I\,. '. hqJ·sto$p:lenomegal.y.:1 II, ,EM. exhibits: .zebr,a'bodies, in ,ly$OSomcs: look like zebra stripes, • ,G~II.cli,er' ~disease-' ., •. ,ARdi~ wi~ deficiency of gIUcocqcbro,sid'ag .• ' .lysoso:tUal 'a,ccumulatJon of

gluc~ere·bt,oside': fibrillary appeamncc' (crumbled up ,n,ewspapcr) in macrop~J:1a:~ ... ,ad~l, 'O,pc associated with massive hepatosplenomegaly and 'an ',increase' in_j~ t'Q'raJ. 'ac:td, PbPmhaq.se

. 'f7'

"5/9

' _

~Qt-e:: l ms materiar rs CUPJII;.U\'CU .. J-UI I"I;UU cqefYaJ.i awwaro· \.rOIJaa, M.D ... 2002

derived frOrtlH maCr~pha$S! • metachromadc leqkodystt'9pb)!- AR diseasc'tlJ ... d fi; '.... m:lsulfataseA and a lysosomal accumulation of sulfatide: results in theWl~ •. e ciency ~f

. · f: COd"~ ,- ',' ,',', .• ~ -., ith " hro ;.~ synthCS1S of abnormal

myelin, • SU1.'3tl"'CS stain poSlnVe.· WI·· metac n :manc stams, •. pe,rip,hera) neuropa' th', ' .•.. ,

- L d edJ b ' ' ,~y, unne

arylsulfatase ac~V1ty <ecreasasen~ • ~~be disease» AR dis.:=ase with a deficiency of

galactQ..sylceraml<iase ~d a lysosO~I accumulation of galacroccrebroslCic: results in the synthesis

of an abno~l. ll1yehn, .. pr~~ess.ve PSYCho~()tor retardation. • multinucleated globoid celis (hisnocytes) rn eNS, • FabrydlS~e- + SXR d.,sease ~th a deficiency of a~galactoc(t'ebrosidasc:

A. and ~ lYSO$omal, accumulation of cetamIde tn hex 0 side, ·angiokeratomas on skin, • hypertension, _. renal failure

Glycogen synthesis (glycogenesis): " glycogen synthesis (glycogenesis. occurs in the fed state) in sequence- • glucose -+- glucokinase _. ·~P04,. ~PO~ + ph'osph'oglucorttutasB -+ G 1 ,.po~ • GI-PO~ + iJDP. glucose pyropbosphorylase-« UDP-glucoSe + UTP + PPi •• UDP~glucose' + glycoge_n .:s)tnt'h~ttni! (insuiin enhanc~ @tc limitinR rnzymcH' glycQ,p: branched, chain polysaccharide'ofD-glucose residues with (;(-1-4 linkages, " glycogen synthetase produces 0:.1.4 linkages between the. glucose residuesby adding li.nb~s' tc an already existing glycogen prim~r,'. glucosyl 4:,6 transferase makes branches by' transferring 5-8 glucosyl residues from the nonreducing end of the linear glycogen chain to another residue on the chain and attaching it to the chain by a a-l,6 linkage- glycogen synthetase men adds glucose residues to the new rica-reducing ends on (he branches and t,o the old non-reducing ends. ,. liver glycogen maintains blood glucose during the fasting state until its s-tores are depleted- gluconeogenesis is. the most. important factor maintaining glucose in the fasting state" ~ 'muscle glycogen is used only by muscle

. .'

Gly'coge·oo.lysis~ • glycogenolysis occurs in the fasting state-·· • glucagon/epinephrine activate

adenylate 'cyclase' ~ increases cyclic AMP (cAJ.AP), • cAMP activates protein kinase ,A:, phosphorylation inactivates glycogen . synthase , • activated protein kinase A activates pho'sphorylase kinase, .. activated phosphorylase kinase 'activates glycogen phosphorylase ~ .• ~ctivated·glvc.ogen .p .. bosphoryJ'ase A (rate Iimiting enzyme, muscle, and liver phosphorylases) cleaves a.1-4 bonds up to 4 glucose' residues of a. branch point, • glucoS)'1 (~,:4)tran,sferase (debrancher enzyme) removes 3 of 'the outer glucose residues 'that. arc left on the. branch and transfers them to 'the non-reducing end of' another chain where glycogen phosphorylase A cleaves

off more glucose l-phosphares, .' amy I. 0- ,a-I,6 glucosidase: (debrancher enzyme) cleaves off the remaining 1 glucose on the chain leaving behind' a frc,e glucose; ratio of glucose l-PO~ to free

glucose is ~lO/li '. glLicose l-phosphate + phosphoglucosmuase-« glucose 6-phosphate~ •. glucose 6-phosPhat,e + glucos« ,6 phosph·at45e (g!'uconeo·genic.:enzyme deficient in, von GiqXetsH glucose, • small amounts of glycogen are degraded in. Iysoso mes by c-I ,,4 glucosidase (~cid.,'(na.lt;!Se, which is' deficient in P'ompe'~s disease)

'Vo,p (;'ie:rke',s .glycogenosis: - .AR disease with a deficiency of ,'gluc()se 6$ 'p'hosp'hataSe'~ ,a,

gluconeogenic enzyme that isprimarily located in the liver and. kidneys, • glucose is M:1eascd in the. fasting state: (fasting, h~cemia) and glucose 6-phosp'hate accumulates leading to an

increased synthesis of 'nopna] glycogen primarily; in the liver and kidneys (bcDatQrellOmemx)'" • glycogen excess in renal tubules interferes with lactic acid and uric acid ~crerio~-+inCle:ascd anion gap metabolic acidosis and increased incidence of gout, • mmuIangn .. [SIS for gluconeogenesis tiS.")!: glucagon... fructose.&!lacto$ecannQtinc~ blood glUC:osc: Q~ to the

"," I' ' " '.', iLn.hosohatasc

mlsslngg \lCOse yy .' _ ' .. .........._ ,,'. '''''~~~

Pompe's glyc:ogeoosis; « AR disease with a deficiency of the lysosomal enzyme(1.1~4 glucos!..._..,.

(acid maltasej- only glycogenesis that is a lysosomal storage disease. • accumulanon ofno~1 glycogen in lysosomes in multiple organs, • restrictive cardiomyopathy from glycogen depostnOO

in the heart is the Me c·on·

I I

f

4

l

1

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L

60

L

..

McAtdles disuse (glycogenosis): • .A..R._disease·Witha defieiency of m~cle RhoSDhotyIU~ •• muscle .gl~cogen cannot be ,degraded Jea~mg tP~educed amoUnts of glucose for'mUScle energy, • early fatigue wlthexerctse_ (no A TP) leading to muscle cramps-s fhabd()m",ol~" '.

f 1 . . d + b'l J' r .... 15 .......

myoglobinuria. • absence 0 a'cnl: act·' In qoodafter eXeTCi~. • normal blood glucose: muscle

d~es ~qt contribute to blood glucose, • enzyme assay of muscle .confirrns dia8l"losis- c;ompatible wrth life

Debrancher andbrancher deficiencies: • debomchglbrancher deficiencicl are all associated with an accumulation of abnormal gJycogen, • glucosyl 4:6 ~sferag (blancher) deficic;ncx- no branches on glycogen. • glucosyl (4:4) transferasedebranch(T deficimcy- • increase in a-limit dextrins (small branched oligosaccharides) and decrease in free glucose, .. epinephrinr:: challenge leads to an increase. in o-Iimit dextrins and a decrease: in free glucose ... arnyl~ 0:-1,6 glucosidase debrancher deficiency- decreased amounts of free glucose, since the remaining glucose: cannot be clea ved ,otT

Glycogeneses with fasting hypoglycemi.:a: • VOrI Gierke's, • deficiency of liver debrancher enzymes, • deficiency' of liver phosphorylase

Sequence of collagen synthesis: • initial synthesis in fibroblasts- .. formation ofpolypeptide pro- 0.1 and pro-<I2 chains, • hydroxylation 0 f proline and lysine by .vitamin: C: site for cross-linking outside the fibroblast. • g!vcosylanon of lysine residues with glutose and galactose. • assemblY of 3 pro-c. chains- inter and in tra chain, disulfide bonds, at. C tel minal 'ex tensions, .' formation o:f trip1s:, ,heli.x_" • procollagen molecules translocated to .Golgi apparatus' fot packaging and secretion •• extracellular synthesis of collagen= • procollagen molecule secreted into extracellular space, • procollagen peptidases cleave N-tcnninal and C-terminal propeptides to release triple helix. •• ' co llagen molecu les form fibri Is, ,~oss-linking ~f co Ilagen fibrils to increase tensile so ength: lys)!J oxidase (copper is a cofactor) is, a cross-linking enzyme

'IV infusion ofthiamine (BI) in an alcQb;olic; • thiamine is a cofactor for pyruvate dehydrogenase, o-ketoglutarate dehydrogenase, and o-ketoacid 'dehydrogenase, • all these reactions produce' 'NADH. which, in turn, generated 6 ,A TP s'

Types ofmutatioDs~. '.. .' . _ .. .

1. point mutation of a sin,gle, nucleoride base wi.thin a prot,em-codiDg g'eoe ~'Y' ,bav'e J.

'potentialo'ulcomes- '". "_. . ",.,

,A .. , silent mntatfen: altered codon specifies the same ammo acid Without alto m,g, the

phenorypic effect- e.g._, UUG (lysine) rl CUG (lysine) _ '. .

missense mutation: .altered codon, specifies a different amino acid leading to variable

phenotypic effects- e.g .• UUG (lysine) ~ UCG (wine) .

'. sickle cell disease/trait:" point ,to,aation, occurs' where:adeD.ine ,replaces thymidine causing 'v'aliD'e, to replace' gluDlUiicacid in the 6t11 ,posilioll of the

p-g;l'obiD, chain Dorm,aJ ~ chain DNA

B.

CTC

In

sic,lde DNA. fH:haiD, CAe

A replaces T'J II

c.

GAG, GUG

Gl V,a!

.. ·U '.

, ;.;....1 cod' · "' Sf"P cod' 'OD (U' "A_ A UAG UGA)· caus.l'ng·

'Do'nsea5e mutation: altered ' .. " 0 a U' a' ,0 _ ,',.. '-, '~,_', ' ,c' ". '.'_"."' , '. ... "

premature te'r'mi,D.atioD, dU'riag 'proteia syutbesis- e.g., UUG (lysine) ...... - UAG (stop,

C •. _, odon~ h al .. ' mj- •• point mutation produces a $tOp codon 1eadinS to

..,--t 2-,asse ,-2 major.. a, r= : . .

termination of ON A transcription of J3-g lob in chain

fr:amtsbii't mutatioa- . 'd . . dlvtsibl' by 3

A. caused by ,iD,S'e'r1iOD or de,letioD of auy Damber of QII,deob .es BOI, -'_, . ' .. _.. e .,

6,1:

!'lot.e; This material is copyrighted. All rigb.U reserved .. Edward Goljaa, M-D .. 2'00.1

shifts, the' r~:adiDg, .fidram,e dori.ag dtranSlatiob of xaRNA leading to a random1y ~ncorrcct,amma acu sequence an prQductlQn of a tt'4ncated proteinduet .. introduction 0 f a premature .stop codon . 0

·mRN.A: 5' Gee AAA _AGU V'AU UUG Gee 3'

Ala Lys Sa Tyr Leu Ala

B~

5t ace AAA GUll AUU UGG 3' framesbift mu.tatio ..

Val Ile Trp

.... ,

·C. Tay Sachs:

( I i 4 base iasertioD produces a fra·meshi.rt mutation (2) codes for defective bexosaminidase

D.. cystic fibrosis:

(1) 3 base deletion with loss of phenylalanine on chromosome 7: thi·.s is. DO.t a fr2mesh.ift. mutatiOn since it is a, .muitip,le· of 3

ne D'e Phe O'Iy Val

normal DNA ATe ATe TTT GGT GIT

, , .

,

CF DN'A ATe AT- -T GGT ·GIT

AU

nc .Dc Gly Val

(2) transcribes, a··defettive CF transmembrane regulator tf;i2t disiD'tegrates before it 'goes to the e.en 'membnme

7' Glycine: • smallest amino acid, inhibitory neurotransmitter in spinal cord (blocked by toxin in

tetanus), synthesis of heme and. collagen and bile salts and acids. .

~ Alanine:. alanine cycle during fasting, major substrate for gluconeogen'esis]. (transaminated, into 'pyruvate)

V'aline: • essential M _. branched-chain amino acid, • not degraded in liver •.• utilized 'by muscle" • increased in maple. syrup urine disease

a"" Leucine» essential ·M • branched-chain amino acid, • not degraded in liver, • ketogenic, • utilized 'by muscle, '. increased in maple syrup urine disease

rz- Isol·euciDe:. essential AA, '. branched-cham ~Q acid" • not degraded in liver, .' utilized by muscle, • increased in maple syrup urine disease

r:r" 'Metllionin~:. essential M • polypeptide chain initiation. • methyl donor (as S~adenosyl

methionine) _. ,

:r' ProliD'e:. helixbre.a:ket1 • only amino acid with side chain attached to o.~am:ino 'group, • hydroxylation in collagen aided by ascorbic acid, .~. binding site for cross-bridges in collagen P'beoylal.aioe:. • essential Mt •. increased in phenylketonuria (pKU), • aromatic side chains:

increased in hepatic. coma ,

'7"' t:ryp,toph.:u: , .• essential AA., • serotonin. niacin, and melatonin precursor, • aromatic side chains: increased in hepatic. coma

7' ~teiDe: • forms disulfide bonds, • component Of glutathione; an important antioxidant in RBCs (deficient in G6PD deficiency)

'7" Se·rine: '. single-carbon donor: converted into glycine 'when carbon .. removed, • phosphorylated 'by kinases

~ lbr..eoD.iae: • essential M • phosphorylated by kinases

(;r Tyrosinei > precursor of t21~bolalDiDes" 'meianj'.a" thyroid' hormoBe$t • phosphorylaled by

_ , , •• . 4 ,'. .. , • • heoatic .ust

kineses: unportantm second messensers,. '. aromatic SIde: chains; increase m. epanc coma,« m ... _:

be supplied in diet in, phenylketonuria. (PKU)

62

L,

Asparagine: • insufficiently synthesized by neoplastic cells, • asparaginase used for·trcument f

leukemIa ' 0,

Ghiltamine:' • most abundant amino acid, • DlAl·or carrier'· o·:r·, .mmonia' _ n;~g'- " d .

. ~. ;. -, '. ' .... , .' :,at .. , 'u en .. 'onor U1

synthesisof purinesand pyrimidines, • Nfl) dctpxifica:tion in brain and liver •• ammo group . . ....

from skeletal .muscle to other tissues in fasting state; • rue,. for kidney. gui, and ctlts in i:: system l'n 'fasting-state

Lysine: • es~ential AA. • basic AA. • posi~vc charge at pH 7, • ketogenic •• abundanr in histones • hy~x}'lanon In collagen ;ude~ by ascortn: ~cid, • bind.ing site for cross·bridges in collagen ~ ArgJ.PID~: - essential AA, • bas~c AA,- posmve charge at pH 1, • essential for growth .in clUl~en:

stimulates growth hormone and insulin, • abundantin histones .

Hisridiac; • essential M. • basic M, • positive charge 3tpH 7, .. effective pbysiologic: b.ffer, • residue in hemoglobin coordinated to heme Fe:1., • aseatial for growth. in chUdreo. • zero cbarge at 'pH 7.40

Aspartate: • acidic AA. - strong negative charge 8t"pH 7: important for binding properties of

albumin, ~ forms oxaloacetate (substrate. for glucon~8cn~is) by transamination, ,

Glu,um,il.te: • acidic AA.,. strong negative charge at pH 7: important for binding properties of albumin, • fonns o-ketoglutarate by transamination

Know h,ow, to' calcalate number of amlnu acids in. 8: gene given the' Dumber' oJ nncleotides .to exens, lD'tron·s, and'S" lJTR

Genetic basis of mild f3-tbal:usemia: primarily a splicing defect. Severe p·thaiassemia is a Stop codon (nonsense mutation)

Effect of decreased 'LDL receptors ·O'D HM:G C:o.A rednetsse; • normally, increased uptake of cholesterol in a cell decreases synthesis of LDt receptors and reduces gene transcription of HMG

CoA reductase resulting in less de' novo cholesterol synthesis in the, cell, • decrease in, LDt receptors decreases the uptake 'of chol estero I in the cell. therefore, less cholesterol causes increased transcription 'of HMG· CoA 'reductase, hence an increase in. cholesterol synthesis in the cell

, .

", " . " " "_'" ". ·'c

RIG,R YIELD. NOTE.S: MICROBiOLOGY"

~ Dipht.heria ,oxin= • diphtheria toxin inhibits protein synthesis ;by ~~p ribosylation of' elongation fa.c·tor ,2; .' antitoxin and: erythromycin are the RJ;:, of choice, .' prevent with diphtheria toxoid immunization

~ ,Ps~ud~monQS aeruginosa to:dn: exotoxin A inhibits protein 'synthesis using. the same 'mechanism as diphtheria toxin (see above)

r;r Neissl!.riil. gonorrhoeae: • gram negative diplococcus, • endotoxin is li,pQOijgosacc'haridc (not Iipopolysaccharide like N meningitidis}, • oxidase 'positive-, contain cytochrome c, • chocolate 'agar'- modified Thayer-Marten ~. • 'pili- • attach to' mucosal surfaces, • resists .phagoC)10SIS, by· neutrophils, .• antigenic change responsible 'for repeated, infections, • IgA protease- hydrolyzes

secretory. :~gA to make it easier to stick to vagina and urethra .. '. capsWe-, protects 8'gainst phagocytosis, • .sugar fermentation- glucose (N.' meningitidis is maltose and glucose), • plasmid mediated penicillinase producing strains, • discharge in first week after sexual' centact, ~, Rx-,

ceftriaxone or spectinornycin i.f allergic to penicillin ,

Francisdla tularensis: ~ gram negati\~ 'rod, • can survive in macrophages for prolonged periods, .' antiphagocytic capsule" • vector- d:cks using a, wild rabbi.~ reservoir, • Me transmission- c,lcaning animal hides (e.g., rabbits) •• requires cysteine fiJ.r groYlth. '. Rx- streptomycin.

cr CampYlDblJCIU jejulfi: • comma/Svshaped gram ncgatiw rods, • ingestion ofeon~ted~ulgy (fowl are reservoir), milk, or 'Water ... decreased gastric' acidity 'increases chance of infecnon, ~. grows at 42°C't • 'M'CC bacterial gastroenteritis in United States, • some strains have enterotoxin

63

i

~

I

I

I

. T

similarto' cholera, • bloody stools resembling ulc:~tive colitis •. ar~~'~a' '''on' , .: ~·th· . Guill .z ",' [:

.' ~~, u. . ' ·WI uw . am Barre

syndrome-~tibOOyag~inst organis~ cross-react with neurons, • Rx: erythromyCin .-....

MCIC of otitis medla/SIOBS.iUs iD cblldrr'D: StrqJlococ~ pneumonia.e

MCCof ~dtis externa: • i'seudo",:onas Q~ginosa- ~ ~th topical polymyxin B + neomycin + r hydrocortisone, .•. also cause of malignant onus eX.temaJ m "da.betics,

H~mophilus influeliZiU: • .grnm negative coccobacillus, • requires factor X (hemin, blood) and'V i

(N ~ D) fOT gro rowth J ru.I!

M:I.~ropbage5;- multilobulated ~ucleus ~d have granules, • previous monocytes, • nUCt'oglial cell macrophage' of CN-S and reservoir armY m CNS

NK cells:- type II hytJCl's~itivi~ rt:a~tions., • graft vs host reaction •• kill tumor cells. called large granular lymphocytes with antigenically $t4mJJated

Clue cell: • Gardnerella vagina/is adherent to squamous cells, • bacterial Vaginosis •• RJ(wjth metronidazole

Sporvtrichosis: •. prick of thorn from rose, • prick: from lobster spine-- packed in· sphagnwn moss

bi h has · •

W Ie .'.'., . ·orgarusm

P.atieat sticks himself with· a knife aJld develops trismus.: tetanus

Rx of '''~unrDC}~tis carinii pDe'amoola ht my: ttimethoprim-su1fametho.xazolc

Picture of buddiog .. yeast 'with. narrow based bad: • Cryptococcus neoformans" • R.x with, amphotericin

Rx of choice for ChltUft)YlU: rrachQmlltis:: doxycycline:. competitively 'block binding qf tRNA. to

the 30S ribosomal subunit. involved' in protein.syn.thesis .

. Chocolate .• ~pr: contains blood-which supports growth of bacteria

Child. with m·eningitis and picture of gram Deg.iiv~ diplococcus: .• Neisseria. meningitidis; .. 'capsule prevents phagocytosis, ..•. endotoxin (lipopol:ysaccbaridc) produces shock" • Ig.~ protease ·degra.des secretory IgA to allow adherence of organismto mucosa in posterior nasopharynx, •

prevention- use vaccine; give rif@mOin, to. close contaCts.·. Rx with penicillin G

Normal miero fiora. of nasopharynx;' • anaerobic bacteria-· .. Peptostreptococcus; •. Fusobacterium, .•. Bacteroides, • viridans .group streptococci •• coagulaseneganve staphylococcus, •. avirulent Hemophilus and Neisseria meningitidis species

Normal microOora. 0'( skin: • .Me is coagulase negative staphylococcus. • less. commoniy-, • StaphylocOCcuS aureus~ • Corynebacteria; _. Propionibacter (impOrtant .in acne), ., 'Clostridium perfringens (20% of healthy people), .•. Candida, • Malassezia

Picture or As~.rgilbts: look for narrowangled septate hyphae: and fruiting. body Coccldloldemyccsls: • think Southwest, •. a:rthrospores in dust, .• spherules with .. endospores in tissue

Recall eat v(Sicle:s around mou·th ad veJTp)ilioD border ofli'ps: • Herpes simp/a·l .. latent in' cranial 'sensory ganglia

EId.erly .,maa '''·irk pai.D.ful vesicles in. aQy dermatome·:·,. Herpes zoster (shingles), e' latent. in . 'sensory .ganglia

~ co,.~pt. of comp·'lementadoa. to defective (mUtaDl) virus; replicatiOri' of another virus 'prov-ides missing' function required by·the1l1utant. virus

l'aJdaJ .d·efell5e apinst SJImonelb i.a blood.: endot,oxins released by battetU, activate the,

alternative complement system. .' .. '."

SeIf-lnducedabortiOb:' cervical swab and pu~. into anaerobicmedium for C/cstriilJum perjri.lIgDU

Kaow' bactertal reproduction types .

Spirochetes in syDoviaJ nald··: Lyme's disease due to Borrelia burgdoif6i ..

[Dftrtile WOatU willa :scarred raIlop.iu tabes:, ChltZl1t)'dia .tnlcllonttzliS MCC ." . . . J·-K.disease from 'braia iDS'IrUmea('beated with .formal·d.ehyde: priaas'is, tra"SDDSSlblc agc:o~

64

CO~~~~: MCC of myocarditis (dilated cardiomyopathy with lymphocytes in biopsy) and pen ... rdms

Wound Infection: gram positive cocci ~ S. aureus

Middle aged woman with meningitis; Streptococcus pnewnoniae- gram positive dit lococc .. Patient with pneumonia has elevated ASO titer: group A streptococcus P us G,ram positive rod resistant to beat: Bacillus anthrax

MOA of StTf!ptDCOCCUS aga/acriat (group B streptococcDs);inhibits phagoC}1osis Cause of aD immunodeficiency with a high IgM: defect in isotype switching

Cyclosporlae uecessary in identical twin· n-a.nspll.Dt-?why: there is still some diffelcoce in fv!HC sites from 'crossover ofchromosomes during 'meiosis

Major drift w~th influenza: maj or c~ges in the reassoi tment of genome pieces indicating a need fOT a new vaccine; only protects against influenza A; egg based vaccine; killed virus vaccine

Receptor for HIV: CD", molecule on helper T cells (also macrophages. dendritic cells) C,D common to 'both B and T cells: CD.s is present in all leukocytes

CDlo: common acute lymphoblastic leukemia antigen, (CALLA)

Complement ilxlltion reactions: hemolysis of test system RBCs is a negative test, while lack of hemolysis is a positive test

Hanta virus: • carried in rodents (deer mice), • ARD,S,.' •. hemorrhage •• renal failure, • viral RNA in lung tissue-- PCR test is best overall test

SlIlpliylocDccus aureus: protein A attaches to Fe receptor of macropbages, hence blocking opsonization of bacteria

Proteus mirabilis: moves 'with flagella; 'urease producer Mycopiasma,pneumorrirl£. requires sterols:

Latex agglutination reactions: antibody to capsular antigens is attached to the beads, 1.,00:00'0$ of cells in lymph nodee B, (follicles), T (paracortex), histiocytes (sinuses) Cells that attack protozeans; CD. T cells

'What gives 'bacteria their shape: peptidoglycan layer in the cell wall

Tumbling motility, gram, positive rods: • Listeria monocytogenes, • invades mononuclear cells, • beta-hemolysis' in blood agar, • transplacental infection in fetus or occurs in renal transplant patients, • mainly contracted from, eating unpasteurized, cheese- goat's milk, cheese

Gray membrane that bleeds when removed: diphtheria,

Dengue: • transmitted by mosquito- • Aedes ae gyp ti, • same mosquito as in, yellow' fever. '.,~ "breakbone fever", '. 'hemorrhagic fever

T ' Weedon associated with premature rupture of the membrane: • group B streptococcus (s. agalactiaey,» C~ test

D..,-1 fuo,moB on B Iymphocytes; activates B cells

Loeaden {or Stllphylococau IUlTeIl.S carriers: anterior narcs

R hi DO viras: • common cold occurs more often in fall and winter .. '. [00 many types to ~c~ an, effective vaccine ... person to person droplet infection and, contamina,tion of hands, • acid-labile--

does not cause gastroenteritis because oftbis _

Virus responsible for ,a coldiD I,priag and summer: adenovirus LactobadllBS in 'vaginA,: responsible for the acid 'pH

In.Oueau vaccine: • killed" • egg-based

Bib vaccine: antibody against capsular polysaccharide ',. . , .'

. hU'dh-- ..... • 'III .. d ftC ..,.L--t ad:di'doaal lmmanm,tio,D5 are,

ID 'addition to the DOfmal 'C"":_:_VVU ImmaDIZ8 .o.._." ""'.. . .. __ .'- ...

. d: ' .... ...1 ,"' .•. kI" . ....If' disease '-'''d ~c fibrosis: Pnecmococcus and lnfiUenz:a--'

recommenc c:u. LD SIC: e Cal ~. -.u. "~i,7"

Pneumovax is given after 2 years of age . .. .. ..' .. . onl bcca .. the Whic'" live vaccine can be given to • p.dut 1ritb AIDS: ~- MMR 15 BlvCrt. ....y. • use ted

_..-.1' :._r. .. ~ s. . 1'··',· .. L __ tb 00- that po. ·---tJa11y could happen \VItb the at1CDUI_

namrai mtecuon ,lor meas es 1$, worse UUlU ... e. ... "" .. ..'. ~ . ~ , .

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...... ~

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.._ ' ...

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LiY~ vaL:dne$:" cannot give to inuntmocompmmised patientS: e.g organ transpI" ..' MMR: only one that you can give to AIDS patient, • ·variten~.Opv, .' BCG :nt.Pl:ben~ • yellow lever t smallpox, ,!I;

'Po'I'Ysac-e:b,arid'e' vaccines: ,. Pneumococcus ... ' Hi.~ men-'" . in 'g' 0(' . '~"""iI'IIiII va-·- c' c in c· I'S' tllft - th-' s., ,. - ,

... .• -.' - ' .. - ~ - .... ,IOcr' :fIiiY'.tnft) -

Killed virus vaccines: • influenza, ~. rabies, -SALK vaccine .... '. . .. • .' -- • .pC

Immllniza~ons that are.eollt~aindJeated l~ p.tteDtswitll, u.pbylactic reactiollS aaiast .... 's:

.l\{M}t.. Influenza".. yellow fever g egg

ImmlluizatioDstbat. ar~coDtraindic~t~ mpadeuts with anaphylacdc .. ,anions agaiDst '~eo'mycin·: • ~, • varicella= neomycin is used .. as~',a ,p'reservan:vc,

V'enlicald,lesioD hi lower extre~ty 1m ap.ti~Dt returning from SOQth America: • South ~can b~~omycosis- yeast with a ships wheel appearance, • North .AmericanbllSt()mycosi~

has wid .. 'L..\iiII;"';;;. __ ~ buds

'_'" ''r. ~ , t ~

'_ - . . . "'_'._ .:. '. ..' :. - - .' .' . ~

NewborDba~'y in mv posi~"~ mothe r ; .newborn ~ anti-gp 120 in the Strum(IgO anil"body) ••

prevent HIV in newborn by gtVlD,g mother' AIr --

Most '('0 mm'o n cause, Q,f' .diaribe:a in chil,dco.': rotavirus

E. coli: attaches to the urogenitalepithelium, hence: its #1 status for urinary tract infections ,8ru'108,"5> ag,a'mDiaglobulllie,~i.;a: .·S~ • defect in pre-B to, B cells- no germinal follicles in nodes or 'plasma, cells, • prone t,o, respiratory infections, • Rx with IV gamma. globulin

scm:· combined B and T cell deficiency. ·,ftrst immunodeficieacy treated with genethcra.pyreplacement of adenosine deaminase, .' D,O germinal follicles or 'plasma cells, .• no, T cells .. 'm' parafollicular area, <accumulation of dATI', which, inhibits ribonucleotide reductase \JII,tb subsequent decrease in deoxynucleoside triphosphate precursors tor DNA, which reduces the formation of '8 and T' cell precursors, • 8,M: transplant helpful

'Wis:'kon Aldrich:" - S){R, .'. 'mad sinopulmonary infections, eczema, thrombocytopenia, • 'BIT cell deficiency- • .l. IgM: poor antigenic response to bacterial polysaccharide .... normallgG •• 't IgA and 19E,t '. defects in, C!dI, develop late, • iriereased incidence of leUkemia! lymphoma, .' Rx- BM' transplant

CamillO"a v~able Immune deficleac)' (CVID-);. 'DO inheritance pauem, • intrinsic defect in B cell maturation into antibody-producing plasmacells, .. presents between 15,...35 yrs of age, -

-.. . :-.:. - - - ...: .

recurrent sinopulrnonary 'infec:tio'ns- decreased. Jg production, • giardiasis, malab'soq;ti'oo' due, 'to

celiac' sprue, '. all Igs decreased, • ~--. rv y~lobulm, "~

Selective .l.gA deflcieacy: .. M:C hereditary, imrmmodeficiency, 4t:" intrinsic defect' in B' cell differentiation inte committed B cells synthesizing 19A, andlor, possible T cell defeat that. preven~ts B cells from synthesizing [~ '. clinical ........ recurrent sinopulmonary infections- lac:k: of secretory l~ • giardiasis, • autoimmune di sease " =allergies, .'d~"tt:1Qp ,m·ti-~ an'tibodies'wirh,~ 'to blood products-sdanger of anaphylactic reaction When exposed to blood products wim ('gA. -serum

and 'secretory ,lgA levels decreased . ,_ ..

Sex ... liitked lywp,hoproUfe,ra.rtve' syadro:me':, • ,Sxa ,. B cell deficienej-- EBV~re'lted dIsease, •

hypogammagl,o·bulincmia.. • malignant: lymphoproli:ferati~" disorders

DiGeorge syu.droble (thymic hypoplasia): • pUre T cell deficiency- no UlhcriW1ce pat.W:nl. • failure of the 3td (inferior parathyrQidsl1:hymus)l4th (superior parathyroids) pliarynpl pouches to

develop.- clinical- • abnormal facies. • hypOparathyroidism: with hypocalccnn.. .and tc~Y', • absent thymic shadow, truncus arteriosus (cY,8Ilotic congenital heart disease). ~.cIuowC ~dtdlaslS,

'-' ~"~".- - ... (pcp.:" .) -, ........a ,A , -.1. ...... .-..- ... -:b." .( ... ~"e1' ":~..:Ilaf!:- 'b-lood to, des.trO~

'. Pneumocystis carmu pneumorua r ,'.' ...• , • ftiil5Llo" VS I,~.t, reac 'o'n .. ll'\II.MI' UTaU_ .. ~"', .. ", ..... _.-.. ~

donor iramunocornpetent lymphocytes •• RX- • thymic gra~ • bone marrow ~lInts .

AtaO.lela.ngiectasia~ • AR ,diSease. ,- Bff inDI1~odcficiency- dcVdops m2-5.)UI' .C)lds.. ~

clinical- • cerebellar ataxia; • I~langicctasias in 'b?l~~~~ta=n:OO

,~bromosQ_mi'Il$,t3',bility svndrq,llW- • increasedSusceptl'-ll~ tor~M,U'mo,',-, '-" .. , .n - '.~':'-

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enzyme repair defects (increased risk for lymphotnas/leukcmias), • tbymichyPOplasia. • low

19A1IgE, • increased serum n-fetoprotein ~

Part of·. vaccluethae is a'adgeu'ic: polysaccharide capsule'

Person workingwitb. aniUlal hide:s.dev~IQ~ lung disease: Bacillus anthrax- Woolsorter"s disea .",

Cryproeeecus: 'evades host. desrrocnon VIa its polysaccharide capsule ,.5e.

Child With aaemia and diarrhea: • bookwonn(NecatDr) produces iron. deficiency, to Rx-

a I ben da'7AJIe .

Lyn:aphoCl1~D.eoUS nod~es:tqa rose' gardeller.·sporotrit:ho~ .' RX- .. itracQnarole, • old R:x was treat with potassium Iodide

Elderly male smoker with _DdD~prodQcdve· COQg~ bacteria fa:iJs to 1t9'" 1fD' onllrwy media (lD~st be sbpplemen.ted WIth 11"00". and~tel~eh D~ Dieterle silver staiD to ideotify: • Legionella, • fluoroquinolones or ~myc'ln + ntampul

Macrophag.e •. ctiva.tio.a: y-inIerfelon secreted from helper T cell

AIDS:. most common acquired ~Qdeficic;ncy

BLA system coded O.D. chromosome 6'

Hyperacute rejeeticu 0('2 tralisp&ut: • ,ABO incompatibility or pa,tient bad .anti~FU.A ancbodles againstan Hl.A antigeninthegraft, •.. type. nbypers(nsitivity reaction.

HLA-A,. B, ·C' code for elassI .. 1ig~115: CD.· cytotoxic T cells recognize the·se:.antigens 'JJI;A-'D loci code for class If aotig·e·ns: • CD. helper' T cells, • ·macrophages.rccog;nize

:G.raft ,\:7$ host r~ctioD: • NK. cell mediated, • ~CQIIm1O'n in bone' marrow and liver transplants" • clinical- .• rash, • jaundice (necrosis o.fbile ducts), .•. diarrhea, .• danger in. T' cell defieiearpatients

Receptor for EBV: • CD11 on B· cells; • polyclonal stimulator causing Increased synthesis 'of ·immun.ogtobulins-· reason' for hypt;rganUn3gIobulincmia in AlD'S'~ • mcreased cell divisions increases risk for t;8: 14 and development of Burkitt's .lymphoma

CD type for hfstiocytes: • CDI'~ • positive in patients. 'With histiocytosis X- •. Hand-SchilllerChristian, • Lctterer-Siwe, • eosinophilic granuloma

Destroy C ·t#fficiJe in bedpan: autoclave

:~. rnberculosts: mycolic acid in cell "Wall is responsible for acid-fastness

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Blastemycosis: :. spores associated with beaver dams and inland water ways, • broad-based-bud, •

verrucoid skin lesion resembles squamous cancer

Impetigo: most commonly caused by group A streptococcus CD):. antigen recognition -. site forT cells

Ab'tig·en that: binds to CD4. OD T reUs iu. "mv': gpl20

~isk:' Cor mv if accidental needle .stic-k, from an ,~V positive .patient.: • l!3JO~ Rx wi~. triple therapy for 6. months and gC.t £1 ISA '1est .. at: repeated intervals

Fever in m.·al.a.ria: coincides :with rupture ofRB.Cs , .

,BeDtatologic: 2bnonlia1ity a.ssoda.~ with Rx ·of malaria: treat with primaquine and develop

acute intravascular hemolysis in G6PD deficiency .

P.adl.og.ea comm·u·ni.ty· acqnired laftctiOD rather tban a 'oo,socomiaJ iDlec.tioa: ·MJ!Cf?pkuma·

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pneumomae _ . .

Single most important Iafeetioas cause of death La 'the wQ'rld: D.JbcrculOStS : .

Astbm.a, massj'V'e h.emoptysh. and erleraal. otitis: Aspergt1Jus' .fum,~gQ1uS: narrow angle, septate~

corona

Ca.vit.ary lesion on 'a chest x-r.ay; :~ primary squamous. carcinoma of the lWi.~ ~ Histoplasmos~ .-

lung abscess, • reactivation. ~ is cavitary . ... • ..." .. ~ . .'. . . '. _.

,Myco6,aamuM: tuberculosis and' sarcoidosis' ·ut .BOTH. ~:ted ~tIa:~, ~ulomatous

heoati - tis the former the. Me infectious cause' and the latter the Me non.-infi._. ecIlOUS ... cause .'

-r- -_. . _ " , . .. ~ 'fib .... MOsr

Gr'eenish ·discolora.tio·:D of the spatum' iD a feb·rile 4 'yeat ·old ,child with cystic .. ' 'fOSis 1$ .. ..;,

LIKELY dlle to: Pseudomonas aeruginosa» .~

Parhogens that-are br.-ached with a standard Gram .st1ia: • Nocardia usteroides-: • paniall" acid-fast. • .smct aerobe, • ACllnomyces- ._ stnct anaerobe, • sulfur oT~nl'ul ... ~ • ri_';:'_1'~'g' si " ... 'I ...

, . . ~~'.~, WG..lIllII. UlUS tracts

in jsw, thorax, or .abdomen ' .... . . .

65 "ear old cave explore,:r for' Indian ,arqfacf5 iD,tb·~ SODoru d ..... rt 'of .Ai. ......:...._ _ . . _, '. ... ..

.. . ~. , l"LI uu presents Wlda

fever, f1u-l~ke symptoms, a.Don-productive cougb and p2iaful red ilodules Oil tbeancerlor

asp~t.of hls lo.werleftleg (eryt~em~D~dosUID). A solitary Cilia Jesioa witII aa egg sbelJ..Uk cavtry is noted I~ tbe up.p~er portion olhis left lower lobe OD a chest x-ny: coccidioidomycosis .Au asympromanc, afebrile 48 y"ear old Black man, wbo b2.S lived, .aD of .bis life ia Ohio is a.~ted. to h~ve multiple calcifications throagbollt both lung Odds and ill the SPI~u: histoplasmosis

Seetochromogen associated witb: painless' c·enical sdene .. pathy 'iD cb1l<!·r.en= Mycobacterium s crofu la ceu m

Most tommoli C2U·S~ of .dissemjnated TB i.a AIDS: Mycobacterium' avium-introcellulare Most common causeof a swimmia.:g 'pool gruu'lom.a:, Mycobacterillm marinum

Most com~on cause of iutestinal taberculosis in. the United States:: Mycobatleri:wn tuberculosis= swallo w infected. sputum. and organisms. taken .. up 'by' rnaerophages in, Peyer's patches Photochromogen that produces pulmonary tnbercalosts: MYca~leriW71. kansasii

Rapidly growing TB associated .with· infection iu imtD,tlDoto.m,prpmised·ho~. ail.d la. prostfl.etic devises: Mvcobacterium [ortuitum

- . . -

Pathogen that commonly produces lung' abscesses, common secondary mvad:er lu the IUDg in

patients "'i.th rubeola or fnftueoz3', produces teasicn pDe'umatoq'"su· iudiDg, to t-eosi:o:a pneumothorax In cystic fibrosis panents: Staphylococcus QWeus

.Pathogen that is 'contracted when the' new-bo.rn passes through the birth 'canal, It. produces a paeumenia characterized au abrupt otJse~ of tachypaea, w.heeziDg, hyperaeradoD,. eosinophilia and a conspicuous lack oi fever .. It is often associated. with a coaj1lD'ctivitis:

Chlamvdia trachomatis

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Systemic p2t:hogeq tb.at l.s often associated ·~ith the presence of iDd.w·elliDg "·eaollSfaneriaJ

.. catheters and immunodeficiency states, 'It produces .. pDeumo,nia c.han.cteriZed by diffuse nodular infiltrates and evidence of vessel invasien: Candida. albicans

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Childhood pathogen tbat ·D12Y produce. ~ PQea.molJ,ia. associated with WanhiD-FiDkeidey

multinucleated gitnt, cells: rubeola

Water Iovtng pathogen that is' most commonly seen in, ~e'D over 40 years old 'P;b.o ~ are smokers, It produces a confluent bro.n<::hop.D·eamonia., with fever, Doa-prodactive ·coagb, hemoptysis and other systemic siglis and symptODlS.. .It ~mmoDly produces hypoaatnmi'a related to development of lnterstitial nephritis leading to . b ypo're aJa, emit hypoaidostero·D_ism.. The pathogen is best visualized ·with direct .itD..tlulno·fiaoresceat:e or a sil.yer .Sf1!.i'n: Legionella pneumophila

Respiratory pathogen ",;th a. significant. moruJi.ty· :iD. those over' S5 years of'age, .. d w.ho uve u'D'derlyiag renal, cardiac or lung. problems. Ifprodeces a. severe, ~:mLbtive. p·D·eamoDia 1Iritb a propellSity for secondary baete.riaI 'in·vuio.n .. There ts aa a:ssoci2tioa with Re:ye's sya_drome .iII ~blldreb that take aspirin: influenza

~espiratory p·l.thogen that is traDsmitt~ wilbollt • vector, a~Dlike other pathogatS in its. family .group .. lt is ·p·ri'Dl2rily·traasmitte.d by inhal.atiOD .. by iDdiridaals "'''0 •• ve,~ -, ~do. with the birthingproc.ess iD sheep,. co·WS· aDd goats or in those who .... 'ork in tile .~"k ·tadasb"y ..

ItIs ·p.rti~Qlarly ~om.rnOD iu 'workers who shovel feces ill sheep:' Coxiella b.um~"' .

R·espirato·ry patbogen that is transmitted: 'by direct b •. ad to oad traDSrer ofillfected IQteria1 and by respiratory drople-t iDfecli·o,D .. Its maiD reserveir is s.chool. chDdreL [)e·ve40pmea, o( a

vaccine is highly lInlik.ely.: rhinovirus .' . " '" .' Respiratory pathog~1i. tr2ll5uiitted bydtoplet infe(tKau. It actoDuts for ._'roXi..,.lety 10% 0{

(OnimUDity acquired atypical pneumonias .. it a sUIaller percea~ of eases of ~~ 'Cold 2.gglu·ti.n.i:ns are Dot ~ted with this pailt·ogea. 'TIl«e may be aD assodatiO. ,.idI

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~h~:~~d;q~~:::~::~ ;;;PGbdS well to dQx:cydine, like· ;othfr QlelDbers of its. family;

S~ct ·aQ.erobe that. C.2D prC)~uce an . ~iI1p'yema that drUBs tbrough a sinus tract out to the ,kin su.r-face.,. Y t~low~ec~ (if mate~:d IDtb~ draiaage flllidsbollid be CrlUll staiDed to demonstrate Its' ch •. racterisnc m.orphoh)gy: ACIl.h·omyct.3 .iSra'elii

Pathoge'l1, 'that is commonly co,Dtr:--c:.ted In :military $tatf'o'll$ ad 'lQ crow.ded .. ,t.,..,~ , ' I:'"

", ~" . .., I . .' - .. ' .' ~ ... au:ollS., t

produces an lil.~erStiti2 pneu,moDl2.a.ad. U often associated with er.y:theQJa mQltiform.e aud

bullous myrin.gltis ... It respouds w,eD, 10 ~·ryth·romy.ciu: Mycoplasma,pn,eumoniae .

Respiratory pa.thogen that is 2IJ. 6a'Dlpl~ of a ZOOD~Sis. It Is .assodat.~ with a:It luterstitlaJ p.u.eo~onia. Its incideace .has declined. by pilttingtetratydiDe ia: animal feed: ChlamyditJ.

psittaci

Pf"edomiaantly a respirat.oty p:l~ogel4 t~is:· strict aerobe is most commoo.y seen h~ patftlHS with, defects in cellular ,1mm,UIli~,. 'partl,cmbrly m the s:ettin,g 0'( bean' traJa.plaatatio~ It produces microab5C,f$Ses in tbe' IllIIg$:, ofte'q with 'graD,alo,1Da (ormation.. A characteristic feature, aSide froID its unusual Cram. 's(aill mo.rpbology, is £hat it is·· partially add fut:

Nocardia asteroides

!\!os.t .CDmmOD C2..U~ .of d.e.ath, .m. p'atieou-1V'itb cysdc. fibrosis·: Pseudomonas aervgtnosa'

Most common-eauseof ptle~'mo'Dia and. bron:cbioliti$, ia i.D.faafS: respiratory syncynal viras !\J.ost common -cause 0·[ croup with . inspIratory· stridor .. A'I·ate·nd neck x-ray reveals'. "steeple 'sign "::;~ parainfluenza, • tracheal obstruction

M'ost· commen cause. 0·.( lnspiratory strfdor ia I., child w'h'o~ la:fe·raJ Deck x-n.·y' reveals; fbe T"th.umbp·riot" S~gD .. Its incidence. has decreased. owin'g to the use or 'a vaccine: Hemophiius influenzae

Most .common ~aqs:e o'r bmachopneumonia ~au·d tobar ,pneum.'o:u.ia. iD the general populati~D:' Ssreptococcus pneumoniae

An executive in an. offlce 'hi ,N,ew York ·C·it)i has a window' air c.ollditiooer ,·hat is.'~ ra,v.orite~ roost '(or pigeo'D..5.,She and her sec~~'~ ·b9.th ·dev.e'lop.lung dise25e:: CryplOCO,CCU$ neoformans

Narrow based bud , ",~

cr' AD. HlV posirive patient with fever, night SlV.·ea·ts" :cougb't d~,p.Dea ·~th e,xertioll,a CD:.. r

helper CODD,t (l·t 3:50' cells .J.1IL,. and C2,vitary api'~ Ittag·· disease: • Mycobacterium tuberculosis, • note that M.A.l an.t.y' comes when the 'helper T cell count is < lOO cellsi~, ~ T ·Most. commou fU',ngai i .. fectiou in'an BIV' p,,.sitive patient: candidiasis

tr Mo,st eemmon C2US, of recarrent pDe·amoni~ in ,2. patieat with AIDS; Streptococcus

.'

pneumonla( .

'T" Inspiratory stridor is' ce~'m'onJy ·,aSSociated, with: • parainfluenza virus infection (croup), .'3tute

epiglottitis . ' . ...

rr Chlamydia ullch'o'matis G,d tbe r~pita.tQry ~yncytia.l 'vira:s are BO.fH. :cQ,mmo,Dly AS$OCD.ted.

wjtb.~ interstitial type of pneumonia . '

''7 Kh:h5id11l plle.:umo.niu.: .• upper lobe cavitation, .• thick mucous and fat gram 'n.e.gaa,ye rods; •

associati on' v~rilh an ale 0 ho lie .. _ . ' .

~ 10 ·a: 30 year old, me w.bo lives InTennessee, ·Y9u.:wo~d· tJ;peC1·. a\caI.cified solitary· CO'ID Its,lOIl

,j'l the lung to represent: an old granuloma .. ·~s'topl~.isl . . " ,.... '-', ' . .,..,.,

(F A bridge· paiuEe,. h1 BrooldYJI, New York de-velops • pulmoaary IDilltrare. Whlc~ of (be.

(o"Uowing pathogens ate: OD .yo,u,r differcatiaJ list: .. Histopkuma capsula ta (sta:rllnss).1 •.

-Cryptococcus n··e:ofbrman.s' (pigeons) ,

,., 26 year eldmale, who presented -ith. sudde ... ~DUbt ~f l~~iF ::Pri:··Cgr:::.rp~~::'"Ac:..u:! productive efrusry-cotored spu.llUll, and pleuritic e 1St P"'II.:· ...•...... ,_ ... ~, -~ .. :, ~

x-ray reveals a right tipper lo~ C"oasondatiolL A gram staiD of SpBtltlD. is pea_,

Streptococcus pneumoniQe:-pneumonia~ • Rx with Penicillin G

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Systemic fungus. witb b r()a~"-basedbuds presents 'With. skin disorder sim.latin,.. . .....

· . . ·'U·' l' dis' se 81' d' .. -d . g, sq uamODS

C3.rcmtlm.a as we as Iuo'g' ea"': ',' ascomyces -, ermatu i is . ' '. ,

System.ic fungustbat mosrslmulates primary and reactivation- TB aad has . east f .

phagocytosed by alveolar macrophages: ,His.toplasma.capsularum .Y. orms

~ulm6.n.ary pDthoge~s tba~ are vessel. invaders a,ud have the capacity to prodaceuimoD .... lnfa~ctiorlS: • Candida a/bream, • Pseudomonas aeruginosa, • Aspergi_llu.s furnigan! ... Mu~ species

Patients. who are 'b~iDg' treated for ~tuberclll:osis 'may develop sideroblastic a,Demia: ,.:' comp~ication o~ isoniazid. - produces p~doxine ~eficiency •• B& is necessary in heme synthesis in the mitochondria o'f.RBCs" ,IIJ' leads to a sideroblasne anemia

SlrqItqCOCCUS pneumoniae: Mec of- • mcningrtism patients > 18 yrs old..-spcntaneQus peritonitis in children with ascites;" · •. otitis media, •. sinusitis

28 year old man with AIDS wh? presents with chroniC, ·recqrre.c, profuse, noabkHJdy. watery dlarraea, An Entero-Test (string test)" reveals oocySf$ ·th.a·t are partfatly add-rut po.sitive:

Cryptosporidium parvum

Afebrtle .2 .. 2 }~ear old man and. several other' members Qif b.Is familY"dev,elo~ severe vomitiDg. without di.arrhe.a ,-1-6 hours 2fter eatiug potato Salad at • p·le-Die. They- aD 'rec::overed uneventfully 12~24 hours Iater: S~ uureus with 'preformed toxin .

23 year old 'man -developed explosive, watery diarrhea 'with blood; le'ukocytes., aad mucus -3 days after earing chicken that 'was im~roperl'y' cooked..,··Co.mma-sb:2p~, orgattisms are Doted iD the few smear ofstool along w.tb. RBe·s and leukocytes; Campylobacter pylori

Febr'ile.Itl year old child . presents ·wit:b severe right lower qu·a·dra.nc. pain that is iDt,erpr.eted by the attending physician as acute appeodicitis.. .. At laparo·tom,y,· the sur-g·e.:»Q nctes that the appendix is, normal, Mesenteric Iymph nodes are. markedly enlarg.ed and ba:v.e .foal areas of

microabscess formation on 'cut section: Yersinla enterocoluica -

Afebrile 28' year old medical .stndent develops 'vomitiDg a:od diarrhea· -4 hours after .e:a.ting rewarmed fried rice. Irorn a Mexian 'restaurant, ·Gram positiv'e rods are present in his stooL Be recovers uneventfully in 12 h.ou~: Bacillus cereus.- preformed toxin

2;5 year old. medical student during ,S'pring break in Tlju.3na) ~e.nco develops (ever" "'''Q,mitiog; 2bdominal cramps, and '~ater:r diarrhea '~14 bours after eatlug·a few-tacos .PQr·~hased from a street vendor. 'He' recovers uneventfully "in 48 boars: enterotoxigenic ·E .. coli- secretory diarrhea Afebrile 30 y,ur' old man develops profuse watery diarrbea" With abdO'ni,jnai, aamps -48 hours after ~:a.d.ng:·a dozen raw oysters 'at a cafe -aJODg the Loaisia'Da coast. :He is 'hypotensive_, volume depleted, andhas a hypokalemlc' D.onna! "aniOD gap meta,boli.c :acidosis:' Vibrt« choleras On ·a trip [0 the Far East, a man develops a Jiigh fever ass0c.i8ted, witb bradycardia,. absohl"te .neutrepenia; 2Dd splenomegaly •. A, blood culture is positi .. e fo:r' • gram a,qative' o:tgaaism.:

Salmonella typh.i

2:8 vr old man presents with di2rrhea witb. mucus and blood A. stool. for r«1l Ieakoeyres reveals blood .and neutrophds, Pst!Q,dome:mbranes are DOted '0.. a co 10 Dq5 co PY:' could be- •

Shigella sonnet, '. Campyiobacter [ejuni. . _ .

Patient with AIDS develops diarrhea 'withst'e2torthea., A biopsy uvea Is' tiUlcrGp,bages W·tth :a.

foamy ~()pl25·m .. : Mycobacterium avium-intracellulare with Whipples-like '~~. '._ , OdYDopbagia in 2 HIV positive 28 'Yea( Qld mg, wi.th wllite :p~que-.like· ~terial Oil bls (oD'gue

·ao.·d buccal mucosa ~at scrapes off 2Dd. 'leaves: a, bloody base: Candid4 a/bt'CQJf.S. .' .

Elevated serum amylase associated with melliDgoellcephalitis: ~~oVU'US infecrion

~=~~testina1 lymphomas arisiDg fraXQ mucosa associated lymphoid tissue'(MAL n u. die stomach: Helicobacter pylori d. :r.fost' common tnvastve eaterecolftts rtb2t 'commo'my slmu.lates ul,e;endve co,lIds D,·'·

psendemembranous colitis: CampylobQaer )+eju'n£

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·1\1.'OSI ~Om~OD ,t'.ause. of diaT~.bea in cbiJd're.n daring:tbe winter' 'mO'Dths ·1bat b,u 'aJJ ,E:U' "·S.·A" t" . ',:

,r ;i.d· i · di '" . ' .. ' est

•. 0.1'" ,0' 'J! I'D "lagno~IS: rotavu'us ' .-

Common QlI, .. se oftiiarrhea in ~S aad Illost tcorumoD caUS4:i oJ bUiary tn.ctdlsease .. d

paacreariris in ,AIDS: cytomegalovirus ,. . - aD,

M'o'st' c;O.mmQD C2U5,t:: of g'astroeDte'r,itis i"D a.d·ults: 'Norwalk virus

Mo,s' common cause 0'( a secretory diarrbea when o.utside lb.e UDitedStates:,Enterotoxigenie E. coli,

Mo"s~. common cause ~,f a ",mi:cro,~giop.thic·· 3D,enlia" thr<Jmbocytop'ebia,. aad renal faDar',!: ill children: Enterohemorrhagic E'. {:011-' serotype Ol5:7~~H'7

Elderly' patient d:e,y,elop'5, d.i~r.rhea a ''Week, ,afte't' 'being treated for p'DeamoD·ia. w:hD,e io. th,e b~$'p"'taL ,A .'fl,exible Sig.~Oldo5COP,~ reveals 11. gray~y:eU~:w exud.at.e in, the rectosigmoi,d,:

Clostridium difficile- Itt with metronidazole

,C'Q,mmOD (',2USes of dy,seD.te'f'}' and ,hemolyti,c a·re.mie, syudrome In chlldren: Shigella sonnei and' Enterohemorrhagic E. coif

Most cammou cause.of Bon-typ.boid enteric-fever- ·aad cause of' o'$C:eG'mye.litis iD padepts wi,~ sickle cell disease: Salmonella paratyphi

Tran s mitt ed by a, human carrier sad produces' a disease associated 'witb Iever, Sf-.PSis, VK5.c:uJ.iti5",djarrb:e1; ,he'pa,[os;plen:(Jm.egaly·, and 'gaUbbdde'F disease, JtIs bot 'tbe most CO:,mmOD, cause of osteomyeflds ,in children with sickle cell disease: Salmon~lla ·typ,hi

'M:C' urganism ,COQ,I'amj'IJ,.atillg. bl-ead trau~{U:S-iODS, and, .IJD:y be auoci·sted With trigge'ria.g :.okj'losio'g spo'ndy,litis,:· re~in.ia enserocoluica

Produces ebstructioa of the: terminal ileum: ". Mycobacterium tuberculosis, .• swallowed

organisms from, a primary' in the lungs .

In v as, sive hel ',m, ". inth ·that ls often. disseminated m" " AID· " . ~.",S'" 'p.a''', ients an·, 'd."

- ~ - , LI a., comlllo'q caQ,se 'of

antoinfecticn and superinfectiou: Strongyioidesstercoralis

Type (s) ofhepatitis with no protective ~ti'bodies: Hepatitis C···and D' Type (s) of .Jle:p2titis ~wlth. ,DO chroaic state: Hepatitis A and E

Mo.st common typ,e o.fhe,p2titis fO:UDd in ,day care centers: Hepatitis:A

M'o, st. CO'mmOD rype (s) (I'f ,r.~,p3titis "leadjng to 1i~:pa;tocdlDllr .eareinoma; Hepatitis B .an~f C ·Mos't.,c'ommo"D hepatitisasseeiated 'V ,i tb, po'ly.a'rte.ritiS· nodosa: HBV

'M'ost common ·'bepatitis.la, trav~le£t·s to places o.Dtside the UDited States: Hepatitis A

M·o·st eommon ,h,e',p,atitis 'ill Jails' add corrective iastitati·O'DS:, Hepatitis A Most eemmon sexually transmitted types of hepa.-titis.:" Hepatitis' A, B, C Mo;st common cause 'of ,pos(tT2.Dsfas.ioD bepatitiS and, chrome hepatitis,: Hepatitis C

M'ost CQDl,mOQ type (s) of hep,atltis prevented by, 'tm'moai7,aiio:n .with hepatitis B vatcl.a:e:, • Hepatitis ,8. ,0,. • also prevents hepatocellular carcinoma from, HBV cirrhosis

Mo'st (:,QIDmO'D, 'rype (s) of h:epadtistransmitted p'are.nteraHy: Hepatitis B~ C, D

Most ccmmon typ'c (5) of bep'adds' wi,tIl preteetive antibodies: Hepatitis A. ·:B. and, E,

Masl' commolJ bepatitis p,ro:dudag: fuJmju'ut hepatitis, in a patieD't 'with ,a :pnHDstiag bepatiD:s: Hepatitis 0: requires- HB sAg. to infect hepatocytes .

M'ost CO'IDBl,OII, type .(~) 0" bepatitis trans mitt ed by··the fecaJ...onl route: Hepatitis -, A and, E

Mos. common 'type of b,ep'atitisin ho:mosexuals,:' Hepatitis ,A,~ unprotected anal intercourse

Most- common type (s) Of 'bepa:ti,tis th.t 'm ... )' lead, 10' c,br'oDic bepa,titis: Hepatitis B •. C •. '0 . .

Most COcJD;mO',D typ',e of b,epa,titis associated with, eryoglobg1ips and membraaop.rofi,ferad\le glo,merulo.D ep,b:'riru:: Hepatitis C.

Mosreommon t,ype ofhepatitis transmitted by accidental, atedle' sti,ck:' H:epaticD,S·8

. ., - .,..... d I -th'... b' ". d' ··t··

Most comman cause of 'S,PO'DtaneoUS bact;e.ri,al pei1tODltiS m ' •. g ts· '.t. ~ err 'D_15 n', asenes .. _

E~ coli,"; '. Streptococcus pneumoniae is Met in ascites associated ,With nephrotic syndrome in cbi I dren

..

71

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,.,....., ' ....

Pathogen associated .witll ibgqiDa! lymph n~des cODtaioiDI gr&auloauttous Dlicroabsc" " .. "

and: localized lymp,bede.ma and rectal strictures as a" "'o'm~lp' ·UoI'-A'..c:O·· 'D-+' L' O'!'J .... L .. ...esl ses.

. . .' .• ., "-. " .• ,....~ .' +. ~ I •• Innoo:r-a· .... u· '.0' .

. m" 'tb I ,r:: o~··: f,,: ,17fQ

ven..ereum·-' only ·S .. -' ,'\.V1.. : out U ·cers .. .' .'

32~·9.I",old ,~'o~.Ju.q c?lDpla~,Ds o~. ".ginal dis(bargerbat flu * ·tlS~Y Oda .. '. It is panieulariJ noticeable durtngcoltus, The dlscbarge has a ()'H of S. EUnsiaauob of tbe diScbllrae Show".

ri db · .. i· b. Hal U G ~ ll. . I' ---6l'o S

bacte . a aU,II;1gto e~t . e ce '5: •.•. artmere a vagllia is- clue cells, • not an Sm .• oniy Rx

thepanenr with metronidazole

35 year old diabt.tic p ... esents wbb • vagiualdrscharge fha. is thick and bas tb:eapp~lIce of

·co.tta.~ge cheese: Can-dida albicans-. RX with fluconazole .

. 3'2 year old femal·~ p~~nts ·wi·t·h. bltease VQ.lv~r p'ruritu$. She .has •. ·parule.at malo.dorOQs frothy veea \'agillaJ. discharge. The _dischargeca.o be Wi~ frOID tbe "aUofthevaginlJ, and leaves patcbyvaginal erythema (strawben:yvagm.). De pH of the discharge is 5.6:

Trichomonas vaginalis+ Rx both partners with rretronidazcle

Most- common eauseof neonatal me~giUs.. Causes t:bodoamoiooltis dae to aD as~efldiag la.fectiOD from the vagina .·an.·d eervix, ~k. ·o·r iDr~q'lI of D.eoo.'·e is· greater' Wh.~D th.ere i5 premature :ru·pture 0:( the membranes prior to deli:ve·ry.'; group B Streptococci (StreptococcuS

.agala·cti"t(1-:e) ,

23 'year old WQ:m:a'D O:Q, the i'bird .d:.ay ·of 'menses preseats with fever, severe low'e'r abdomill,1l paiD and adnesaltenderuess wi.th movement ·0(' tb~. cervix, ~·to.ry reveals se·xu.aJ iD,.ercQ~.rse. ,3-4· day'5 prior to menses: • Neisseria gonorrhea» the . sh 0 rt incubation period is most cpns'istent with OC· rather than. Chlamydia, '. she has P:ID and should be I:rcated with ceftriaxone and doxycycline, the latter to cover possible Ch·[am)·dia trachomatis as a coinfection

. .

~ :yea.r old patient has had a'D rUD (intrautertne devtce) in place .(or· 9 months ·as· ~ m~thod, (J.f

~bir1h centrol and DOW· Co:m~plaiDs ·oC,tagiDal,disc:b·arge.. .At the req:oestorthe :p~tie;R~ tbe;.ILD is removed, Yellow O.ec:ks of material are attacbed. "to· th·e .nJD whick ·O·b Gram 'stai:D reveal a~ filamentous gram positive bacteria: Actino.myceS

46 'y'ea~r old woman presents wi-th d)"Su'ria, increased .. frequency, and '3. DlDrGp:urulent \":a·gill.aJ discharge, \~agiD3J smears 0'( t"he :e:mdat·C'! ud e~amin.d.O:D .of the urinary $ediiDeat· 3," neg'adve for ·0 rgaIiis'ID$' but show iD:auy :Ieukocytes. ·a.~r l.ast. s.e:mai exposure wa.s 10 cby·s ~:gG,:

Chlamydia trachomatis

...

33 year old fenu"ie fro.m Eqaader bas. r·tce·q:dy bHD diaguQsed. widl. c:broDic eBdometritis. A.

microscopic eX'mJu·a:nO,Q of '(be elldo·m~trial. tissue reveals poorly .fonU:ed· granulomas ud &b illflltta.te o.f plas.ma cells: .M~oba·t;l:eri·urn tuberculosis

3·2 ')~ old ·wo·maD. bas a p·ainfill ulcer 011 the left· labia snajora p.d 'ipsilate'ra) 'paiaftd iDpiliaJ JyrD.p·b nodes, A .. gnm stain from. the 'base :of' t·b:e. ulcer ,r~veals grapI. D.egati·"Ve rods. ia ,'a "sth.ool of·fisb I, orieD"t3.tio_.: H~ophilus' duereyi

'25 ')T old 'Woma·o. has ,a p:aiDI:f;$S ulcer ,G.D, tbe left I·abia majora and l:psib..e:rai p .• io·fal iapi~.

'lympb.a.de~Do.p·atb.y:. Treponema pallidum- primary syphilis, .

23 yr old ~om· •. g. develops fever, pAlatal Iymphad,ea·o:p:.athy, ad. 'paiDfal vesicles oa, b·~ exteru.1J gerut.J'. ,(h·.1 Iater ulcerate, A Ta'Dc'k p:te.p' (rolD the. b:a5e of PDIOf tile liken, 'is

positive:, Herpesvirus TYPe .2.-·note how primary infecncn is ~c: .

'PadeDC from. New Guines. presents ·with • paiDfat: Serp~.O·IlS. t)?e. of raised' sore··o. tile la~l* ·m.JorL Thet(: is Dot. I·ymph.d.enopathy. A 'biopsy is take. ·.aD,d .n.vea:ls macro:~~' ~.dI iDtt-acellular orga·oisms: Caiymmalobacl.ui'um granulomatis: the patient .has'·granwoma l.~guinale and m.e· intracellular bodies are called Oono-vnt bodies:

2J yr' old se.xu,aUy active. WOlDaa 'i$ .. ated to b·.vt Cent-like.· 'lesJ"ODS ·~tla·d, tile. lab,~._d perianal area. ·O:er .RPR. is 'negative: human papilloma 'virus~ the patieat has condyloma

acuminata, or venereal warts . ucI 30·yrar-.Old O'tnitbology graduate student presents with altuedlli.~taI status., $t1Ipor~ .' .... '. headache. There is no Duc·hal ·rigi~di·ty" B'~ b·.s: been ·stadyiDg lD.usiJJ birds ·over the 1as1;, lQO.dI

rr"

in a mosquit~infes~ed sw .. mp ,nea in the MidwesL A gr-a:m 5taill aud culture oCtile: CSF is negarive: • patient has.an arbcrvirus type of encephalitis. . - ., .

IJ..year"'4ld ~I.v)' recruitpresents ",ithfever, ~ea:dacla:e, and a pO$.itiv~ Kenlig's lest. He· .. b ... 10th case in the put week Ibat bas occlirred at the base: the patient basmen:ingiti .. ~kt Ie

Neisseria m~"ingirit,lis based on the crowded conditions . . .. . .. '... . 5, lllO~ I te 'Y

32-year~id m2Q renaltraIiSp~aIi~ ,patient Wlro is immIlDo~ppt'essed has II positive ID.dia iak preparano n: cryptococcal menmgrtrs

Bilat~r.i1 ophthaJnri~ neonatorum during rhe ftrst week: .• N. ~cno"lr.:oeae, - transmitted on the

-way through the' cervix . .

Corneal transplant, bntin lnstrument.«oal can transmit the.prions of Crcutzfeldt-la.kob disease Diabetic ketoacidosis: • mucormycosis in frontal lobes, - ketoacidosis accelerates the growth of

the fun ',. O'1.t c::

.' ._- 0-'

MCC' of meDu.·girl$~ after 18 yrs old; ,Srreptof;QCCUS.p"_t!Um:oniae

. .

R·ubeol.·: causecfsubacute sclerosing·panencep'h.alitis-· slow' virus. disease

Papovavirus .in fecti on::' viral 'agent ofprogressive multifOea.J leukodyStrOPhy. a slow virus disease

"Bubbles and boles" spongifor:m clmngeu. 2.l;:IraiD.: • describes C-l disease, .pr:ions .

y~lD.terf*roQ: • 'produced by ~ T 'helper cells and NK. cells, ._ ftinctions-· .•. activates rnacrophages to kill mi.cro·bi.~l. p.a.$o:g~J. ~ ~ti~I activity, .,. induces class I and D antigens, • increases ·,:nuQU.ction ~of'IL·~2 and n-i 2 b.y CD4- r helpercells

Interteukins: • '1L*:1- • produced by macrophages, .. ~ fever of inflammation, .• acute phase. reactant synthesis 01 proteins in liver. • osteoclast activator. • stimulates B cell production and antibody production, • Q.~- +. produced by 'C'D-4 T helper cells, • .• primarily a T' cell growth factar, ._ promotes B. ce.lItNK .cell proliferation, • IL,'!"3·_ .•. produced by 'T cells and thymic· .~ithelia1 ~U.$'I' • stimularea pluripctential stem cell marrow, • 'increases hematopoiesis, • IL4-, '. 'produced by activated T cells, • mainly P[O~o~' growth of B ce I ls, • switch of·-rglVl.'.s)rnthesi.s in.B cells to 19B synthesis in. type I ·hypets·ensitivizy reactions, _. :~-5~ .• pro¢h:1c~ by T cells and, ~ cells, •

promotes growth of eosinophils, •. . promotes 19E synthesis, .• IL-6- • produced :by 'T cells, macrophages, endothel ial cells, fibroblasts, epithelial c.ells;~ .. - primarily stimulates synthesis of acute: phase reactants in. the liver in acute: inflammation, • .It-12- •. produced; by maerephages, ... promotes .growth of C:P. T' cells;> promotes, differentiation 6fCD~. T helper cells mto·THI. and·T,.2 classes ... promotes production of.·y~inteIfel0Ilt ,. enhances NlC· activity ..

G~~Qt;yt~. ~I:ODY .sti.'oJ.a·t:ibg. factor (Q,.,C·SF): ~: produced by fibroblasts, • sPmnJates neutrophil develepment in. thebone.marrow

'Granulocyte/macrophage co:[on:y sti:ma.I.~tlDg (a(:(o.r «(iM~F).: • produced b~ macro'pbages and t cell~ I • stimulates neutrophil and monoCyte development in the' ·OO.n(: marrow

B cells. • 1:0---:2:0% of total Iymphecyte count, • markers'~ •. intracyto·plasmic. J..l heavy cba.i'1l$= pre-B cell, .. surface f.l and 0 heavy chains: maror:e 13 cell and antigen < recognition. site, •. function. anti body synrhesis, • surface ~q,.~rs~· .. IsO Fe receptor, ·mt for EBVt • tesrma- •. B cell. count: flow !C)tOmC:'D)'" .• immunoglobultn concentratioa: order of decreasing 'concentration, 19Ci.

19A...lgM. [gO, and 19E, .. detect 'isohemagglutinin:s;. mitogen-stimulationrpokeweed .

T cells: - 6O-·7()OIo.oftotallymph.oc.yt:e CouO~ ..• 'rilaikers-. monoclonalantibody ~ -. studies, f~ clusterdesignation (CD) typeS,,'. "immature. T· cells bave ·"uclear ~·tertIlina·l·deo·x,yn~JCQtidyl. transferase (id1). on their' surface, .. _ ·functions-' • J:ypC .. 'tV hype:rsc:asidvity~ : •. cy.1oldnes regulate: B

cells, .•. defense against intracellular patbegens (e .. ~ .• rs, protozoa), • ~~~g~ • mitogen· as~~= funcuoningT cells arc specifically activatedby PPyt9hemagglutinin and concanavalin A: • skin tests 1.0 evaluate ecl~i.dar immunity: .~Caru:Iida is. the.main an~gen used, +abscnee of· an. unm),mC. indicates anergy or -a lac:tC 9' f cellular immunity (e:~g.·l AJDS.l

:cr

.- . .....

'-:,r

,.

Note: Tbis ID2t~ri:.d is copyrigbtfll.AIl rigbts reserved. EdwII"'Ii Gorj ..... M.D~ Z002

...._. . ..... -

AIDS:epidemiology: - gn_120~ralenvelope prote~ ~.~Virusattaches to CD. mOlecule ofT hele .• .cells and other, ceUs-· mono~~esJmacrophag~sldendritic censlmiCrO~ial cells- (CNS macr _ ha per " astrocyte~_,- n24 core amtetn- surrounds viral genomic RNA- • onIyjn~ duri op._~~). infection and when,' - 'the p arien, .' t develops .. AlDS., 4)·'2 separate .~~, .•. rT\ 'T' .:. h'e"l' .'. l~l~g m_ "dlna,1

':. - ". - '. - - '~. ~_.-t'" -~,.:v!:!! '- . -~pet c'e.~_ IS use· , b .

the vu'Us;"_ usually direct HIV cytctoxicrty, • reversetranlsaiQtass converts genomic RNA ".~

proviral double strande~ l?~A-- in~egrated into host '~lI's DNA with virally.;ncoded integJ~

enzyme" _. after transcnpnon, HIV ,messenger RN,& IS translaled' ,-' in to' van' r ious oroee .', -'. '. -, _

. " '-. . " , ,..... , .Ul$_.'.' env

enc:odes gp 120f~ 1. • pol t;rl~~des reversetranscriptasclint~graSe. • gag encodes p24 core-anti •. '.

• VIral core CQn51Sts of g~om.lcRNA surrounded .by- an inner mcmbranecDi'I:1ppsed of p24 antig,.....!~::

assembled near ~e host ccll's- ~lasma -mem~el • budding-Qf the 'progeny virion through the host c~l~ membrane IS. where the viralcore acquires the exterrii.len'Ydopc [Q become a mature; HIV vmon

,Mode of tn,nsmission in the United ,:States ill descending order. • receptive anal intercourse bc~ecn men..» V3ginal interc~~ maleto female: ~~ semen' has more surface arm to-infect,

• female ,to malerless surface area in male-urethra 'to infe '

...... , .... - .... ,. ,. '" . ' '- ." ' ... ect

Positive enzyme .immu.noabsorbent assay '(EIA) test rot'mvia a D-e1'VQorD.: • due to

transplacental transmission of the IgG. antibody 'from the' infected mother, .. ' ~t :mv infection In newborn by detection ofmv-RNAby.. PeR (best test) .and p24 antigen capture assay

AIDS testing with e~e iIJ1~uQoabsorbent_asSJIy- (EtA): • initial screeningtest, .. derects annAAl20 antibody- • scnsitivity99.5-99_8%, • poor 'specificity due to low prevalence of.HIV positivity in tb·e 'general 'population

AIDS testing with western blot: .• .cenfirmatory test for indeterminete or positive -ErA,. • positive westan,blot- • PRSCJ;1Ce. 'of p:24 and .gp4'1. antibodies. 'and. either gp.l;2:0 or -, gp·l60 antibodies, •

combined.positive-predictive value of'a positive EWwestern. blot is ·99_5%·, .

. r.UDS tests .f~lr: mo:Dito·ring immnue status: • CD~ T helper cell count, • HIV ~A. by PCR- .. best -overall test to monitOr 'viral burden_

... . .. . . .....

NOQ~AIDS dertD.iDginfecti~D$' • oral thrush, • oral hairy leukoplakia (EBV glossitis) •• shingles (H zoster), .• molluscum contagiosum (poxvirus)

'Diaposis of AIDS:IDV positive pl-U$- • CD~ T helper cell count: <200 cells1~. -specificmalignancies: e .. g., Kaposi's.sarcoma, ' .• specific infections; e'~.g·~, P, carinii-'pneumonia. '(M,e AID~ ~~g~~) ~ AiDS miSceU3DeoQS 'iJlf~ODS,: • bacillary :'angiomatc;;sis- ._. due to Bartonella ,/z'enselae: iden-rj,zy with silver .. stains, .. simulates Kaposj'ssarcoma » recurrent bacterial pn-eUntonia-" Streptococcus pneumoniae, .. iafections encountered .Mth,·cp,·T hC:I~ count .1~2~OO c'clI.slJJ.L, • Me COD, m. AID.S:, '~ infections .eacountered '~.th .CD •. T helper' count <100·: cellsl~- • d"sseminated: MAl.:.

usually <75' . c~llS1~ ~ Candida esophagitis, .~ ,C:MV retinitis/esophagitis, .• Toxoplasma

encephalitis, • Cryptosporidiosis: diarrhea. cryptococcal meningitis .

Testing 0:(' comple'meo( system: • classical pathway= low C4 or-:C2 if' activated, • alternative pathway- low factor B. ifactivated, .' intef;rit}' or both pathways- tQ:VV 0,. if ejther syst~ is activated, ... activation. .iacreases the concentration of Split. fra~t;s-, e.g., C3a, 0', '~C3b; ,.

functional assessment of the complement ~Jem-tot:aI hemolyticcomplement assay (Olso). .

Major- Histocompatibility Complex (MBC): • matUre RBCs lack class I antigens, • individuals inherit I m:..A haplotype fromeachparent in c~~mioantfashion: "both haplotypes are expressed TraospJ,antatiOD. success requirements: • ABO blood group (:om:p_atibi.lity- most. impOrtant test, • absence of preformed ,mo·HLA .eytotoxic .an~h9die$ in the recipient's serum.. •. close n-.tches for HiA-A, B and 0 loci between. recipient and .donor

Lymphocyte crossmatchtscreens for recipient anti-IU.A antibodies against donor lYD1pbocytes

74

,

Note: This mat I rl:a I is copyrighted. AJl rlgbts rese:rvecI~Ec"'.rd GoIJ ..... M.D. 2001

- .....

Lympbocyte microcytotoxiciry t~t:tdentifies HtA-A and B; derived classI anti en ,.' .jil ", ',' recipient and donor lympbocytes usingknown test. sera - ~ pro - .. es 01:1

Mixed .~ymp.b oeyte. reaction: .•. us_ed' for class Il antigen (D-,- .:. loci) ·matcL!:-.: _ _. i;c'_., _~ _._.

'- - fro' . "'.' d" ,L, . !WIg. , ,wlctl·onal

lymphocytes ,',,11\ the recrpient aa 'preVIoUsly rrradi:ated (kiiIed) donor l)rmphnrVt~ " : . ,,','

th th . ,.. d th idi d tho - . '-"" . .I'~ are .ln1Xed

toge er wi '" tntiate yrru' me to ,etect"e degree of compatibilitv between the· D·: . ) .. >;~

~ ed' d· .. . dicates i ·b~l· - - "J. If OCt

I~Creas,' "", ',13, roacavtty In rcates ~ncornpatl. hlly, ., recipient"s Iymphotytes ate imtdi~ (lei.led)

and ~,ctlonlll d~nor lymphocytes arc reacted agarnst the host's HLA-D loci rtr check fOr a '_A versus host reaction (GVHJ ~.'Ul

!ullsplant-doDors: • sibl~gs ,arc best soun:e- ~hance ofasibl~g baYinganothcr sibling with a O. ,l.o~ 2 haplotype, match Is 25%, 500/0jand 250(01 r~tiveiy •• parents an:autontatieally a 1 haplotype. match.

Graft types: .,autpgrafi- •. 'transf~ of>~ssue from self to'~l£, .. best survival •• syngeneic graft (isograft)- graft betWeen, identicai twins, ~ alJograft- graft bcnveentllU'tIated in<;ijviduals. • xenograft-. transplant of tissue from one species to ~othe:rt '" e.g., pig heart b1lnsplant

Cornea! transplants; best. overall allograft survival rate: -

IDA haplotypes and disease: • familial predisposition 10 diSea.se-. weak penetrance •• disea~ is not invariable, • usually requires ~xpb~urc 'to an enviroIl11leIltal factor- (e.g., virus) .. ,. ffi.A .. A3. hemochromatosis. • HLA-B81DR3-, celiac. .disease, • ffiA-B27:.. ankylosing spondylitis..» liLADR2- multiple sclerosis. • HLA-DR3IDR4-- type I ins ulin.-depeu4 entdiabetes mellitus, -HLA.-:-

'DR 4' b . d ;,;. .... L...: ~

.- -. -:: _. r:. ";CUIDalOl -. 4i·UU.l·tJS:·

Examples 'of' type I bypers~nsi"ti:vi.ty 'f,ea'ctiOIl$.: .:. atopy- familial 'pre.diSpoSition, (ml1Itifactori.aJ rriheritance) to .developan allergic reaction, -examples-'.cczcma (face, flexorlexu:nsorsurfaces), .• hives, ~ seasonal conjunctivitis, • seasonal rhinitis, •. asthma, • hypersensitivity to bee/wasp/hornet stings: Rx of anaphylactic reactions-. sc. administration ofaqueous epinephrine 1: lOOO··.·dilution

a- .E:xam·.pl·es of type' II: cytot'oxic hypersensitfvfty ,·;r~ctioas; .• involves 'antibody reactions M.th ~or without complement, • examples- Goodpasture's syndrome: anti-glomerularrpulmonary capillary basement membrane antibodies" •. .warm autoimmWlc hemolytic anemias and. cytopenias, • 'Rl~"ABO hemolytic. disease of newborn, e' c.ellslhelminths· coated by specific IgGlIgE" antibodies, respectively (withour.eomplementjare destroyed by cells (e.g., :NK cells/ecsinophils, respectively) with low affinity 19G1lgE 'Fe receptors, • myasthenia 'gravis; anti-acetylcholinereeepter antibodies," .• Grave's.disease: IgG' thyroid-simulating Ig directed a.gainst.TSH receptor

:7"" u EX3'mpl'es, o~{ type m.i.uImUd·o.comple.x. (lq hyperseas'itivity reactions: • ci¢u1ating .. ICS, (antigen + IgG/lgM)deposir .in target tissue (e.g., glomerulus, small vessel)-+activate co~lctnenl s.ystem~ _c.h~taC·tic agents 'recruit neuttophilslmat;rOp'hq,e$ that .damage the DSSIJ¢. • pathogenesis of localized Ie reactions (Artb\lS reactions)- first antigen exposure results in antibody production-e second exposure to antigen deposited, in tissue leads antip..antibod)t ICs-+

complement system activation-e- ncutropfUVmacro~bage damage of tissue. • ,~l~ .. , ~ 'sickness; Rx. of rattlesnake envenomations 'With use o'f~' horse serumannto~ .• SLE,

glomerulonephritis (ON): anti .. :DNA +. 'DNA. lCs, .. posr-streptococeal :Q·N:': .... lDti~~. antigen. antibodies +bacterial antigen res.. Henocb...schanlc:;ln purpura; anti IgAantibodies agaiD$t [gA.', ~ polyarteritis nodosa: HB$Ag, + anti-fIBs, ,ICs. oirheumatoid arthritis: IgM antibodies a~ ~ (rheumatoid factor), • clinical example of an Arthus n:action-· Farmer's lung;· :an~bgen IS:

thermophilic actinomycetes. ~ " ."

cr- EumpleS O(lyp. rYT eeU-m~ed hyp4:rsellSilh'i~ n.ac:tiollS: • antibQdy-~dent cellular

immune reactions involving OD4 helper T cells (OM ~tionS) and CO. cyt~XlC r cells •• ~ of ORB reactioJiS- .. allergic contact dennaritis:ptiisoD ivy~ ~Ckel, 01, $kia, tCStS: ~Iilin sensirivity, patch test in contact dermatitis, • 'p1~,omast • ·pathogcn~lS· of ·Cyto,toXl.C T cell,

7S

..

te3ctions.- • .cyto[oxic T cell$1'Io~lIy interact ~tb class I antieens on ~uc'ea1Cd celt '. ...•... .... .... . of class. I antigens on targe~ c e llsactivates cytctoxrc. T cells to release, pcrforins tha. de ~ altcrabon

• examples: neoplastic/virally mfected cell, foreign, antigens in'a. transplant eel)' t stroy thcceU,

:Paruito~ogy t'enbS; • definitive host- hos.tthat harbors. the adult, or sexual sta. of the . . ....• '. intermediate host- host that harbors. 'the "larval. Or asexual' s ..... g·,., o 'f' tho e re&~~t. paras.lte, ..

. '. . - . ., "F' '. ". ,~, "" "" f--~,_I e • "protozoa •

amebae .... ciliates', • flagellates, .. ~rQZ9a",.'. 'helminths i-n"c.I'·ud-e-~ '~. neniat'~~F ·ro····· -. d ',' .. - ..

. . !'-",....- '.' .. . ~ '\Dl'WOJj-,s'_

cestodes: :tapeworms, .. trematodes: flukes . . . , .. ", '. : ,,·II.·_

EatAm·eba his(oi)'tlca (amcbJ~isl: • key w'<?rdS- • erythronha~osis - bl:oOOy··:/'···· dysert. 'f", .,." .

, r 0"':"'-',/ ~ .. "" '. ' '. t'TV •

1+.. n.., .. ...:1' ... - t' . . -"'J 'J'

rver abscess, • :('Jt.,- rnetromdazo e '. "

,Naeglen. "'a fowleri ,(p'i;~i:mary' ame:b:ic 'm-e DingQen,ce.p Il,afitisl: • t.-,~ words- .' -sun'~~'-g .', fre h

'. ' . """'1 '" ... , . """ • .u.~ ~... .. m. .. es '

waterlakes, • .meaingoencephalitis, • .. Rx- 3JTtPbot~c"in.B " ,

AcaD~aro.oeb2 (k.,eratitis): • key word,... soft lens wearers that keep them in overnight or do not clean in proper solutions, .•. ,Rx-· ~d.ine -

Cryptosporidium parvum (spotozoall): • key. words- • acid-foist oocysts. • MCpathogen in AJDS diarrhea, • contaminatcsrc:servoirs-leading to wide-spread diarrhea, • positive Enf.ero..Tcst,.-. Rx-, paromomycin

toxopla,sma gOQdii (sporozoan]: • key words- • MCCof space occupying lesion-of brain in AJI)Sj • pregnant.women avoid eats and eating undcrcooked meat, .• '. 'R.x:- pyrimethamine

B.abes,ia specles (sperozoan): '., key words- • carried tnIxodes dammini (same tick. as Bo,"eiiQ: l?urgdorfen) •.. intnaerythIticytit parasite withh:::molytic;an~a.. Rx- clind.amyc_m + quinine Plas.mowa. species (sporozoans): • types- , •. P. vivlU: ,Me, •.. F -. ftilciparum: most .deadly, .. P. malariae, '. P. ovale, '. female Anopheles mosquito is: the vector for 'malaria- .. : sexual. cycle (Schizogony} develops in 'the mosquito, '. asexual .eycle, (sporogony) develops in: humans •• reinfection 0:£ 'hepatocytcs by' merozoites 'as uiP: viv4X"and P. ovale infestations is responsible fQr

relapses, • patients v(ith sickle celltrait/disease, :gIucosc~pbbspbate dehydrogenase deficiency, 0\[

. . .

. .. . P fa , =_ &'_' . •. multi I :Ii l' " '

J¥thal·assCtTUa are resistant 1'0 .. ,_wc'zpantrrJ; mfection: " rnu trpte reasons. 'me t.td<t~:g- '.··shOrt life-span

ofthe 'RB.Cs, + increased fra,giH~' of RBCs'"<.,,: increased leV,els';ofHgF": parasites cannot feed-on this: type. of' Hgb, • most "blac~ art; Duffy blood group (Fr.) antigen negative, w.hi~h renders the(ll resistant-to P. vivax mfections- the organism requires the antigen 8.$ a receptorbefore it parasitizes

. .'

the· RBC,. • pathogenesis of ,anemia in malaria 'relates to .bo~dt intravascular and extravascular

hemolysis .of RB'Cs'·; the, latter by removal of infected cells by splenic '~opb~g,~~ .• ' clinical picture for me "benign" ,Coo tllS .of' malaria includes-. '-: periodic paroxysms. of :shaking chills; correlates with intravascular rupture' of RBe. • high fever with a specific pattern in all type except P. falciparum, • quotidian (P··fti!ciparum)_: dai~y spikes '. (parO~) offever thai persist, fot a. f~: days aadoccasionally (no specific .pattern) break every couple' of days~ ~, tertian (P, Viwtt ,and'

ova/e): paroxysms 0'( fever every 48 hrs (alternate days}. '. quartan (f.~, mi:Jlariae): paroxysros of fever c:vtry 72 hrs .. -splenomegaly .~ a consistent fc:atute it! alIrnaIarias: spl~ may sponr.an_eouslY

rup. ......... • '0 malariae infesta.rions tnay. ' .lead 1.0 membranous glomct ulonephritis, • PifalctRQ!"Unl'

t.U.& "-,, r .. ." . . . ' . ,~ , . . _ . .

malaria- .• multi'-iI)fesration: of RB'C's by 'ring forms is common, • oaly type that 'D:DlY has ring

forms and gametocytes in the Fa: common 1JSML£ picture •. • ma.ssive intravascular bQnalysis results in hemoglobinuria: Itgb turns ~black color in the presence of an acid 1'N. b~ the term 'blackwater: fever, • chlorbgumc- .' ,M·C ,pl'oP.hy·laxi~i 'II ,safe· durin.g, pregnancy, .• ~~lS ~100d schizonts 'and is gametocidal te allrnalaria species e~cq>~' falciparurn, resistant ·Sti~lI1.S. o.r .. ~~, " ..

Jalciparum._l1Se mefloguin~. • tJ:C:3W1tnt- ,. .P. }liwuJovaIe:ChI~a.e-. ~. pn~"qIlUJe

(, 1..":__ " id . I" .. "'lI ..... ~' D"2'1"n .... ' ... ooidal .' to .r· .... lciparurn ,)., p. fi,JIt:tc,.·nnrum'· IV 'q' uinidine gl. uc,onatc· for

SC lll.G.Un DC rein ,·ver w 6.U b~·. _."""" . , '. 1..'. .' " . . ". . .... ~,',~ "r- -'~-,,,. . " . .',

:~le. ~() cannoltakt:drugs:oraIty. or quinine ~&te+ do xyeyc I inc if they WI take ~ orally .

Ba.laDtidill~~61i (sporozoan}; ., keyWOi"ds- .i.x-ge cysts, • colonie ulcers with bloodyd.iarihe'-a. ..

Rx+terracycline

'SOle~' 'This hD'tHial is' :NW'IIl~~ttd· All rlIbts rtsen"ed.., 'Ed~ r~i.. 'u n. """':z'

,.. '. . .. ' ,. ", ' ' '.; ·--:r'.' ........ w .' ..... ",' .' ... - ' ., . '. . "~---. .~.,; a¥" '

-7'

'7'-

'ow·

,~

"~ ......

Gi~ II.Q)blia ((bge",a~r);. ,. key \VQIds- • Me 'protozoal <;aX1s.e'of ebrQmc dian·heil/~absorptlon in United States .•. day em centers, .. mounuinsprings. • positive EnteJt)Test, • Rx-. metronidazole

TnchQmODn"-agina:lis'{f1agella~e):. key wotds- "sm.· Rx botb:partIle:rS... tumblirigmotiIirv

Rx ... '. 1 ' . "'J.

_'. - fTld:n)ruoaz'o ,~ "

H ' 'n. fti-"'II_t' ,,"", • h" "11 t rn; a 'n ;,_ ,l"......-.h ODS'·._,:. • I·isbman'- 1 ~ . th . -:.

, ~OA~.;wa es,~' u..Li~.. u~~'~~, . "C'1:1Tl11< ;OIa.1$ ~ VfI<m Ol8Q.opbages. ._

Jrypanosomes .are. extracellular and circulate In blood, ... lcishnmriD~ Only me ,in1nCelhWrr

.,' ' . ~'---...:..":., '[1' ~"'", '," sresent in bn"n"1-i!1"n,e' '. Afri-·" ' .,.. ,'. .: ,- .: .'

tet:tu.nwuu·a: 1.OtmS,.~ pt;'~'~. ,.' "~~,' LV'can bJRa:Do~.- only the '~lJular'

(h)~OS()~). forms are present in h~~ .. i\maic;an ~osomJiSis?, ,or'ch.'gas~di~se-' l'Cth leishma.nta1.(ca~ tissue don uction) and ~ 'COItus are present in h·umans

AfrieaD tr}7pa:liQsomiasis (slerpiag sickaess): • cauSed by Trypanosoma bruce: gtl1'lJbit!1'1S~ or Trypanosoma brucei rhodesiense, • bi~ 'o,f 'an infected tsetse fly, '. c~irucal- .' mcep.bali~, • increased 19..\,{, somnolence: release of sleep :n;.:djalOrs' by' the organiSirls" • tr}]lallosOmes 'art

capable of ~t:igcn, variation, .. ~,-, ~ for c;arfy '~on ,(non-CNS~ disease) ~ nE'l~:J. later stages (CN",S infection)

Ameri-ea~b')tpaaOSOmi2sis (Chtgas .. disease); • caused by ,1T):par.t.oscima cnai; .• bite 0-( the reduviid bug (Triatoma, or kissmg bug), • cl~.cal-, • majQr' cause of pro~\w-e heart bilurc: leading to death, • acquired achalasia/ Hirsthspn:mg~s disease ... _,·R.x;-, ru·filr.tnnQx

Leishmaniasis.: • \'1$C~, leishma:niasis,~ LeisJuna.nUi tio'f'bwmi cotnnlex '. cutaneous,

. ~. .

le~s.hrn.L~asis,- Leishmania trooica comnlex, ., leishmania' introduced 'by:" the bite oi' an 'mfec~

... ".- _- .. ' ' . .- ~ ... .- ',' ~ -,- .. _ ..... ," -. - .. -'_-'.", " _"_.. "._ .. -' .'

.~"1.dflv (Phl.ebot·orr.u.s), • visceral Ieishmaniasis, 'or bi.a-anr- • massive ~.~,r~leno~'~!'· and

~. ...~~ .I..I#P~.' .

. anemia, ,~, Rx:' antim9'~ny sabogluconate, - .eutaneous leislun.aniasis- .. involves, Skin, alonc;.: WCO:,SI

r . ble .~. th', , "G'" -lt~·· '} ... Rx' ~ antimony stibo ·,o'ln.~<1!1,"""

t,p!p" 'u~tn In, , '.~ ",: u· "'\1.~~" ' '" . ,E ~w.J."'A,I!"4, ~, ....... , . b'"'~~

'N'ema't,ocIes: • 'those: "iib larval phases in, the tun~' leadin,$ to' pn~nitis/b~:moPtysis-, .. Ascari;

.. . • '," .,.' i ('t,......... ,1 ides stercar: .... I . , '. . , ,

lumbricouies, • Necator a.'m~C4!UJ.S,'t; • sarongytosaes stereo, wU, •. Srro'n.gyt.Qides stercoralis, has,

the -only freeliving larval form, that can survive in naDut- . ,

'E' : • • 1._' '" (P'" '. _ .. ...I' )'; l_.. 'II' 1 '. eI~A'; - .

, .'lltero.b·lU5. yeran,(D.a.i.trlS ',,' .ID' .... ,OI"'ln, .u,matyU,e : • 1t4=J.' iilloras;-' • m.a~ .pruri.~ .. ~CltlS;, •

urethritis-in girls, • ne eosinophilia: not iavasive, '*:' ~ ~, .' Rx; albeadazole

'Tri:ch:llm Ule:ruun (w.hj'p\1t~,orm., eematode): • key' words- .:* diarrhea, ., rectal prol",*'~ II

, "hi'l· n.. albenda "'1'

eosmep ' L. sa, • r...;.'"(:-" ~J~7.o~·~

.4scaris Iumbrtecides (nematcde): • keY \1;0~- ~' larval phase in· lungs: cough, pnewl,iODi,~:~' eosiaopbiha,> 'adults cause intcsrirta~ obstruction; no eosinophilia (nOt: in"~\'1:)lI.! ax-<~Ie or -,mebcndawle.

~ ..

~~cvdosto.ma da:odeaale· aad 'N'eator ,ameri,QD:uS (h: .·oopona., g.emat,od,f;s):, - key \VOrds,_, •

~. , . .

larval 'phase in hmgs, '. tnQUm' parts a,q3:c.b to, tips .ofvilli, '., iron deficiency ~ • Jar..~ ~

soil and can l~le.tr&te' feet, -. eosinophilia, .' Rx- ~le.

StrOlI;gyloi(le:5 stereeralis (u,em.a·t6de),: • .key ,words-, ·lan,al phase' i;n lungs, '. rba'bdirifOilD lan-ae.'

, .

, . , . - -, " ;_.&: . fit . ~ 1_- _";..' ; -Ii' . ~;- .. ' . , ;,_,_.r:' ~ . , ..... __ ...

(not eggs) pass 'in stool, • mreenve ruanrorm W.·VIU; m. SC,I:l caapenetrate .l.~· ,aUlOWl.ccnon 4IN,

~~ftreCtion ('esr«iallv in AIDS)"l • eosinop ,'hilia. • PQSitive Entt:fO..,T~ • Rx- iva "lItnCilu ex

~~ -[i.U-A~ " - . _.. ' .. --'r:' . . J" . . . ... . . .. -.. a . _" .... . .

albendazcle

'T,ri.chiDeib $.P.lralis ·f~~bi~ao~. 'Dem,.tode): '. 'key ~ '. con~ in mart' by the eating': of: raw 'or poorly. cooked pOrk' containing the eacysted 'iuvae'of1hc ,~ism (Pjg.jnt~,.I;he(iiate·host), III.

. " . ~),._. '

larvae have ,,3 propens,ity for skeletal muscle: .Q'~ become' CaJcifi~ .' ~ ~Ml~, '.

muscle-pain, • orbi~ edema, ; •. Rx.-, certieosteroids and ~le

'TO·S:CK2ra ta.D'is: or C:a:Q ;(visceral'iarv.a mil,l,aa$, '_): •. ktzy' words,- .. 'TozoO:zi:a calli;s

(dogs) and TOXOCDra: cati (cats] are the. ctiolO:gic ,agenlS in viscc:rallarv. migrans, til. ",,:n, (uPrl1ly

t, _- dJ: ,.' . ~L.__. I L.'_~, --., ....... __ ,!J ,1 __ ";';' .1_....;.:, .. • ,,+' " . di ~. ~.~ .: viSC¢:lal t_ .... ml"""'ft~'

children) lS an, ~O_i,'ii8x n:u;:.t. ailU UiUyuu-y,,. l,IIW"YQopA mlmll<!C8- ~, . '~ . ~ y."O"'~.

hep3to'splcn,o.mepty. p(Ono\)nC~ eo~ipop,hil,ia.. '.' Rx- 'di;etb:ylcarbamazmc:

n

7."'

AII~dt)s(oma spp- \cutaaeous J.a.n, ... DJigrus Otc'rH:pi"C'en.pdoa, -fIIII.lOd.es): • kcyWO«Js.,..

•. ~.. " L __ o~'l-' '.'. '11 -, ~ b L "Id!' .,

• causec .. ~~'y cog .. 'B:Da. CJ,·t nQ'J~",\,\Drms.;,' uSu~". ~y c. on[nlC'u;:u ' .~ em '~.'cn P:~YUlg· on '$IlKiy bcac.hes or

sandv ·~la\"£.rounds- \~,;'he~ do'S'S an,d~' -, or cats urinate, • larvae 'fViIin .. ..._:t'-; ·.L;;,., #~ .~ . ,':" ".,

.. t-- ,~.... ,...~..: '.. .. .' y-..n.,.u· .. ·.u; UJIII; ~, AUU 'p~u~~

serpjgm~~ runnels U"i the sxm winch causes tntense pnmtus andsc:atemng.. Rx- .ethyl thlon& spray andivermecnn

."-aisakis sim.pln (nemarode): • keywords- CQtln.clcd by,ea~r:aw (i$h. ~sucb as sushi.and sashimi, as ","CIl- as pickled herrmg .•. larvae pc:netratl:; gastric and iDtestmIJ mUcosa. andpt'Qduce crarnpmg&bdoQuI131 pain, ep\gastrl~ distress. \'tirriltmg mddiuThea: Wlthi .. 'l a few ~ ittc- bemg ingested, • .R."t- phystcal removal 01 the orgamsms 'by" 01~SCQP1C, f~

"·uda.em :bm'c'r1)r~ B.MJ.·gta mala}i. lrllariasis, b~m.,todes'):: ~' key wo'rtJ.s- • "bncrofilana DJ,i' '~Uchl'T.'.a bancrofti or Brugia .malavi are ~'" 'causatl,\'C agentS." ,. transnnned 'bv .. the: bite of infected mosquitos: .;frrop~dt!:S. _l{eti~. Clll~. --Jnicrofilana characteristically eJtCtil.~·in·tbe·Q~ &1

nilbl 1I.l1d enter into the tymph.atiC$~ ma~ and produce an inrla rnmauxy reactioo. resUlting Iil .l~llJtih£d¢!nI [elepbantiasis) of the legs, .. scrotum, ete., •. ~:d ·m:jcrofihril.. 'in the blood ,a;[. ni·gbt:· sheathed and lsck nuclei .: ttl the. tail, .• ' Rx-- i'~tin

O.D,ch,ote·rc:S. \~ot"'DI'D.s·lri"",~r bUndn~ ne'D1I.tode)..= • key \ltvrds-· • ·t:Elia·Gnlaria of: nn.smittea to man via ~e bite of 3..') infected blackfly (SimUii:tm'.-tm~ rmgrate across the ~ye and produce blindness, • adult worms lodge in the lyrnpharics: Pl·_1~JU.~1 subcutan¢ou.S nodules, • rnicrofibn;,a do ~ot, enter ~ bloodstream: no periodicity ,tnight. ,or da,Y time "rh}~l~ • find rmcroiibL..;a in·s skin biopsy '~..at, are unsbeethed and do not M\1=; nuClei exteoding' to the tait • R..'t- ·l'l;1et,mectm and

-'''' _.. . ...... -

remove' the nodules

Dirofilaria .imDlitU (heart worm-disease, nematode]: • kev words= .: d.og 'heart, 'a:OIUI that useallv

. .... -', ,~. .-

lives in ~ right ventricle of .. the: dog, .~ mierofilari . .a transmitted ':0 OJ3.D. thraU,gil the bite, o,{ 3-

mosquito, •. 1~,":3 enter the :l,ght heart and embolize to: the hm&: s:rn"n ':pul:no~' ii1fuctpresOt,ting. as a .coin lesion. ·.Il't~ no etfecnve drugs

a·y~enolepis 'Ila.n.a and diminuras (cestode): .' key '\Votds- hie· tapeworm infection in United States, • ~~.- praziquantel

D:~'ph~~,UQ"bo:th'rinm btum (f:is:h 'ts,pe.~.o,~ c~odt'): •. key 'YIOrds-, • larvae.in 1m ·trcut.. ., ~!ISt.· ·ofB,I: deficiency, • R'"(- praziquantel

Taenia. sa,ginau (~'f v.pe'ft··o~ cestode): • key words- '. ingest larvae hi uadercooked ~~ '. man is- definitive best, • Rx. _, 'prazi~t;eJ "

Ta~oia soliam ,(pork t:apewo-rm., cestode): ... k,ey '\1;"Ords~ .. can contract by 'e3~g larvae in, undercooked pork (intermediate 'oo5·tl: man d,efinio':\l:' host, .' can contraet b~' eating eggS from ali. infected human (de':finiti\~ host) and only :I2;f""l' develeps in, p.a,ricnt (iI}tou.oo.ialC·;bQst): .• called cvsticereosis, • CNS 'lesions, with seizures .•. R:( ....... praziquante]

'L :&. -- •

EchiuocO(::cu.s granalo'§i$ or mD~qcula.rl.$ (h yd. dd. clisea~ cestode): .! key .word$-. • sbeep

\ViIh Iarvae (intermediate bO.st)~ eaten 'by dog (devel.op. aduJ~:· ~ilo lay ~ dcfini.ti.'\~ haSl)-+. man -ingests the egg (develops larvae which 'pate1:r2b:. the liver,. in,~.te host)" .• :.Mxpb,yla;tJe reaction. if cyst' fluid eaters perit<?~ • ;~ek and' BasqUe: shccphcrd.e.rS. • Rx,_" po:;·u~COtJS draina~, + ·albend·'X91(:

Trematede dlsorders: • 'trematodes. Or flukes. an: ~at ~tcd '\liU .. UlS, \VitD 'a compleX. developmental cycle, • in schist·OSomaJ disease, ·the cyc:)c, is as fotlo .. ~-· ~ (lluma:a) ..... ,;·diatcd. miracidia: larvae -. infect .snails (1 $T inta fpedialC bost) -+ produe¢ fod t3.i rai cacana. larvae ---+ penetrate skm of human ~ produce disease, • in non-,sc:histosamal disease, the cycle IS ~ .. faIlo·,.rs~

egg [human) __. ciliatedrairacidia larvae·-+ infeCt mans (1ST ~~~ ~J ~ ~e ~ tailed .eercaria larvae' -4 iD.fec,( a 2;1d intCtlltedi.~ .host: •. :aquabc: \"'egCtlQ'OJl lF~ClCiJopsa' ,~.

f:as:ciOiJl1:epati.C3). • fish (Clonor'cAis- .s~isj. • ,CJ1lhS (~ Wut~) -- fuUD

78

infectiv,~ metacet_'Caria .: man .. ingests the ~1Id. intemlediatc host ,: diseaset• note thaI maUs arc !lwan lnvolved In the hfe: cycle_ofboth S_chlstosQrnal and non-schJ$losomal disease

Schistosoma mansoni, jap.onicum. and bematobium (schistosomiasis ·or bilhaniasi tre-:na~odes): • i~fecti~n acquired by the pene~tion of the ciliated miracidia larvae: from infec~ snails into the skin which then enter the Iymphatics and distribute into subcutaneous tissue and the

mesenteric veins, • S', ma~on~ favors ~e ~trabepatic portal ~ns- • penetration of the skin by the larva~ p("Od~ces pruritus ( .swmuners Itch It • larvae develop mto adult worms that travel against the circulation and deposit eggs (egg ha~ sharp lateral spine). to which the host develops an

inflammatory response marked by concentric fibrosis ("pim; stern cirrhosis") in the vessel wall, .' S

· th ~ ·

~e,,!atobju~ favors developm~nt in e ~ary venous plexus where the eggs (nipple at end of egg)

incite an mflammatory reaction (hematuria common) and squamous metaplasia of the bladder epithelium, which predisposes to ~quamous carcinoma afthe bladder •• Rx- praziquantcl Clonorchis sinensis (Chinese liver fluke, trematode): • key words- • ingest larvae in fish (usually fish paste). • larvae ascend bile ducts into liver and gallbladder and become adults in the bile ducts. • causes jaundice and cholangiocarcmoQ'Yb • Rx- praziquantel

Scorpions: • key words- • poisonous species capable of causing fatality lives in Southwestern deserts (Centruroides gertschis, • neurotoxin, • bite site shows no initial reaction-s increased sensation-s no sensation in the area of' the bite-+ whole exn emity becomes numb-e- increased blood pressure-e- ascending motor paralysis_" death, • Rx - no specific treatment for the poisonous

..

species

Mites: • key words for chiggers- pruritic dermatitis best treated with topical antipruritic agents (crotamiron and calamine lotion), • human itch mite (Sarcoptes scabies}- • tissue injury by adult females boring into the stratum corneum, • burrows are visible as dark lines between the' fingers; at the wrists, on the 'nipples, or on the scrotum, • females lays eggs at the end of the tunnel: responsible for intensely pruritic lesion, • adults= disease is limited, to the webs between the fingers, intern iginous areas, and spares the soles" palms, face and head, • infants: no bw lOWS, and the palms, soles, face and head are involved, .: Rx: permethrin Clcsm

'Bead, 'body, and the public lice ("crabs"):, • Pediculus humanis capitis is the head louse- .. lays its "nits," or eggs, on hair shafts, • R.x: permethnn (kills newly hatched lice) followed by lindane, (Kwell), if the initial R.x is unsuccessful. • Pediculus humanis corporis is the body louse- • li~ on the surface of the skin and breeds in the clothing, • Rx the clothing, not the' patient: usc malathion powder or D'DT powder, • Phthirus pubis is the pubic louse: (crabs)-o II lives in the pubic hairs:' looks like a crab, • Rx: pennethrin

Flre ant bites: • sharp, painful bite, • wheal/flare reaction: type I hypersensitivity reaction, • reaction followed by vesiculation/skin necrosis" • bites commonly occur in people that crawl under houses

Patient with fever, cough, diarrhea/vomiting, organism requires special medium, for clIlhlre: most likely transmitted 'by aerosolization from, environmental sources

Viroses: all have protein and nucleic acid

PatieD'ts serum is reacted against ,andge,a, .seDSiti%ed RBes, and complement.. RBCs do Dot, hemolyze: • complement fixation test, • lack of hemolysis indicates patient has antibodies against

the antigen, • presence ofbcmolysis indicates no antibodies ~ . . ~

Interpretation of minimal, inhibitory CODceDtradoD~ lowest concentranon that ~ I nhi.blts,

growth, determine minimal bactericidal concentration: must culture the rubes (0 see which one has·

no growth of me organism . ~ . .

Bloody diar'rhea after returning from Mexico .. Neutro,pbil.s iii stool: • amebiasis, • Rx WIth metronidazole

TB reactivates in apex: • highest ~, • 1B is strict aerobe

79

' .... ·v'" ..... ~ ~- !I ... _ .... -- -.~.- - oil... ~

-·'·~o··-- -·'··L.O .. ····G .. ·-~y· .. c

.' '~".'" .• ' .' I

.Mlo··pu·riJ;iQ,I:, inhibits xanthine oxidase

Isonlazid: • causes pyridoxine deficiency h:ading to petiphcnl neuropathy 8Jld sideroblastic anemia, • MOA- 1NH is nicotinic acid derivative that inhibitS Synthesis of myoolic acid in 'mycobacteria cell wall

.Sacciaylcholine: ., depol~zing agent used ,3$ muscle relaxant .during surgery •• binds to' nicotinic receptors in skeletal muscle causing peoistent 4epotarizatiOQ at motor end-plate. '. hydrolyzes plasmacholinesterase, • initial fasciculations followed by .muscle paralysis, - duration of ac~OD only 5-10 min. • effects not reversed by,cboHnestetaSe inhibitOrs-no pbarmacotogie antidote to

overdose. • some patients have atypical cholinesterase and cannot metabolize.the dr:ug ,

Preganglionic Dearotransmitte~ acetylcboliDt; -activates muscarinic iU14 nicotinic receptors •• muscarinic: effects- • pupillary miosis (eontracts iris sphincter. USed after catuact surgery), • accommodation of lens for near vision (contracts ciliary muscles), bronchoconstrictor •• slow hem rate (SA node effect), .. increase PR interval (A V node effect), .. stimulate GI secretions. "

.. " . .

increases 0.1 motility (entericnervous System stimulation). "micturition (stimulate'detrusormusde, which relaxes internal sphincter ofbladder) .

~itri£, o'xide: '. 'potent vasodilator synthesized ill endothelia! cells" .' activates cyclic €i?\'{P~ y.Jruc'h inactivates myosin ''light chain kinase' in smooth muscle: cells leading to vasodilatation, .• Ach can lead to its synthesis when' :inj"e(;:'te~ into vessels

\\'oma:a in tbir·d ':trimester has-premature contractiens: use terbutaline, a selective a2~adt:ener~'c receptor agonist that inhibits uterine contractions

E,ftect.Q,r asl'irin. :OD kidney: .. decrease 'P.G·E2, synthesis "(vasodilator) leading to unopposed At' n effect; .. renal papillary necrosis

~'[.etb:otr,eX2te: .' blocks dihydrofolate reductase-- macrocytic' anemia due to folate deficiency, .' S phase inhibitor, • leuccvonnrescue, .: interstitial fibrosis ... in lungs

Periph-tral neuropathy and cancer dnig: vincristine

Nerv'e-endigg·.loca:ti'o:ns affected by ,bo'tuiin;om poison, reserplae, .go .. an.~dilie M()Aofsllcralfate: • viscous pOl~of sucrose -oetasulfate + aluminum. hydroxide that adheres to ulcer crater, • stimulates ,pa:E1 in' hlUCO,US cells- responsible for mucous barrier

'Efr~ of 'rn.ixiD'g statiD drugs ~. ni'ad~:: rlt,abd.o'mydiysis- each drug by ,itself can prod.uce. raabdcrnyolysis . -,

!\{,OA, 'o'r amphotericfn s,: • binds ergosterol in, fungal cell membrane, which tacreases itS,] ': permeability, • nystatin has samemecbanism

~f.O,A of clo~m,al'.ole: ~ inhibits ergosterol ,syqthcsis:1, .• other azoic .. compounds have' ~similar' acti'Dn

~r;fOA, 0'( f1~cyto:$iue: inhibitsnucleic acid.synthesis .

MOA 'of' griseofulvUa': inhibits microtubule ,function ,and mitosis

KD,Ow the'd("finitiOns for potency mdefficacy 0'( dnig .. / .

PI'O'pyi,thiODQ,cil: • drug ofchoice fO'f ,decreasing.·,synihc.$is. 'ofth)!rQ,id,"bormonc in Graves disease, .. agran,' " ·uI"t'\rv'I'QS)S·::': is '.' onlv drug' . that- can be used in .~!2'~'

_' .. '~J~""'_1I ',I.., .. r"~~~'.J :.",~ ..

Fastest way toinQ"eaSe heartbdt ha $hOck: • order of drugs with deereasing tffect;lv~ 15

isoproterenol, dobutamine, and dopamine,. dopamine in low(ioscs is bese (or let,"':'~UtlOn .. Dap$Olie: • sulfone thatinhibits ~rhcSis of folic acid by M. leprae; • may pn.~c~tau:: hcm61yuc .anemia in ti6PD deficiency, • may produce metbemoglobinemia, '.: nepl::in>bc syndrome ... ' peripheral neuropathy

[. '.'

Pro:lq'ugati,QD, .of Q'RS GD E.CG·: quinidine

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Anurou,-ulSantlantiepilep;tit . d~g with efTea. 08 ~ip~YSeal ptate: phenytoin via its ."_ .un of the cvtochrome system rn me, liver and, causing vitamJn D, dc:ficiency" - inCleased mett ~bo··· 'l:'~: ,- ~f'"

25" h- vdro ~. xyl- at~ vi '<t,~"",:,;_ D . "". . "" .'. ..<. "" '.' rsm 0:

.. . .Y I: ,."Ii •• ~ : . ,c;u. " I ~"""-f,l 'y

..

)fOA of ondansetron: - selective serotonin 5~HT3recq,tQtantagOniSt., • useful in block:mg chemoreceptor rrigger.zo.ne (CTZ) in t11~ area postrema, hence' preventing vommng . ......•

Optoid o"~~rdose: • .~lratory depl essl_on~ tespii3.toty .i~dQ$is. • 'nriOtlc pupils

First ~or21coholic .0 ~R:gIve I:' ~anu~e topt,event acute Wernicke's encephaJopathy Beparia: M.C;:C of thrombocytopenia In. hospltaJ.

A~te Rl: fQr i:ou.~a·~Do,"·erdose w~eb bl~ing il,Ule-thi'earenlag: • fresh frozen plasma best choice, '. use [M>:Vl~~, for less SCI10us" bleeds

NitrofDi1lntofD':.:~ concentrated in urine" ', •. 'urinary nact.antiseptie.

'C\;_ "clopbosp:'h,amide: •. hernorrhazic ~:V<:. ,"; ntis, • tran.sltlan~J. eel] carcin oma, t, •• ' •• ..... :~ (

e'· J' Y " ~ ... _ a,en vatec ,: n'DI

metabohzed) in 'the liver

:OOxo,rubid:a ,an·d p.r¢dlUw.ne::' metabolized in li"1:f

Gout after caRcer'tlirrapy: • prevent by giving allopuriJlol (unthi:neoxidase mhibitor), •. danger QfW'3'~ nephropathy

Give.Q I'og dose eUOI! for ,2 c;I,nJg$ .and M,ust. ~m.pare efficacy and pOteD'C:Y ~lu;scle fasciculations a.fte;~ n,¢urolD,us'tqJar 'b"lock: 'initial effect of su'ccinyJch'oline M,O~, oflevodopa: blocks peripheral dopa decarboxylase

Brob'c'~:i~i .asthma; • albuterol ·~,d~e.r rI)OS,~ ,'o~, used fo:!' mi14 to'. moderate asthma, • coni cost eroid rnedihaler used.for severe asthma

••• .J. ..

RI of'parox:ysmal" sup·raventricuta·r tac,'h,y-c;ac,d.i.a.'~ adenosine; including l,ts: association M.th \\rpl~l svndrome

~. ".'

'~OA 9f (:.I~loidin~: activates CX:2~~,giC and: imidazole recepters. in, $e central nervous system 'which reduces sympathetic outflow-from the 'YaSQrD9t.,?1" center in the medulla

Capr,o.ptit • bradykinin side-effects include cough .and angioedema-, ACE normally degrades bradykinir; • cough not seen. with losartan

·B.3(d["aJa'riD~,: ~ better tolerated in fast acetylators, • ~USt; 'ot.~g-ind~cd siz, • :can be used in hypertension 'in ,pregnancy

P,atient ·with hyperteuslou, h}lJe·-marremi.. and h}-pO,kalemj.~ '. loSattah; an ~"rn' receptor antagonist, is the.best drug, '." cerrectsHfN and clecr;ro~:yte problem~.(bloc,ks, release of aldbstc:rooe,. ~ hence sodium -is'losl( in the urineand potassiurn retained):

~.'Or plllmoaary edema: loop diuretic is very Useful_., decreases preload

~ of Q'pio.i'd overdose: naloxone, which 'is' a-competitive epioid receptor antagonist

, ' ... ' . . . ," . b . ':1 ~ . . l' . ,.r. . b' locl .. ,-- ,_1 .... 'w;, ··til mUsclt to. Urmar)'! retC:'DUOD lD prostate ,yperp :ana·:· - 'U$C, $e. .. ccnvc ,0.1- ': oc~~- I'~,.smoo:. . .... . .... '

bladder.neck and prostate. ~ eX.aInples-, ~ prazosin, '. terazosin . .

'MOA "of ~~" p2tme,~,o.: • anri-aridirogeri ·effec,[~ blecks 'androgen, uptake and, a,~ilabilily ·~dlout

. . .' ". .' f d the ttreibra

altering serum levels, ... causes shrinking of hnsltl,oga.l zone 0, , prostate aroun;.', .... :.- .. _' .

'N atcoleps.j: Rx with· amphetamines , .

Ptematilretbbor and must ·deHver baby: give glucocorticoids'to increase: surfactant synthesiS

Interpret dose/response' canes: md·.helber' "~D·j~ is co~pedtiv~:Il.II~ DOla-co.m.pditiY~

Drug a"sed~ to ellmi'tl2.t·~·,aJdnm in patient With hyperd.lcemj~:, leopdiuretic '.,

Dra:g . .sed to rtm9'V~ calcium from uriae lli .• aJ·lcilllD sto'ile rOnD:~~: b,~bl~~3nde' r2ti,at wi~.,resp.iratD.ryal~o,51s a'. a, thea melabolic' acidosis: sal.1cyl~,te ... mtoxicanon S-p~ase drugused ill, treatmen·i: ot aCut:el:ympboblas'Q,c' Jeake:'mia;, methotrexate

A_lalO'~g of bypo.xaDthi.De req;u~ 'HGPRTase -Co.r bioadiva,tio:EI:' '. 6~l1l(fCapt,~c~ .' 3'll~p'Uri:ricl. could cause toxicity, since .the drug is a, ·pmin,e .

Dnlg' that comperes with, dDW' for tb,ymid:~te ~th.se: 5 .. ,~UOroCma(:ll

D'["~g 'a.sed in Rx, Of'Wilm's 'tumor: actinomycin D (dactinomyem)

,81

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A.ad~e·taboUda"le tbal. t~ge(the.~~itb I~D ' .... 'Ji.bb~tiCis l.Ued Ja Rx of .. cute myelogeaous ltaJc_ia' I cytarabine +.: . unornycm ·_·~qu.aCYC·Jne:anb iotic) - II

Nep hren site r-:$p01isibl~fo~ ACE'iJlbfbitors ~1IsiQC incnase i.$quQl cr.eatiQle: block in AT

D (works as vasoconsmctor of efferent .artetlole in g. Io~·'~us'.;)· by A···C;E· . inhi "'b:- I··tor-' re .: -, ... , -' '. ". -' , . ·I,.I~·W . . ... '. ,..UJQves

important control .fQrmair'ltam.ing tn~alblood flow causingpotcntiaJ for renal failure (pani'cularlywith. bilateral renal artery stenosis)

Nepb,rou site of actio. of thiazides: NaT ler pump in cortical thict ascending limb

Vitatningiven ~ ax Qr~: • pyridoxine (B6)- used up when .USing isoniazid, ' •. risk ofpenpheraI neuropathy and sideroblastic anemia

Drngs that Be generally ufe inpreglluc:y: • sa£e.- ... methyJdopl:; • hydralazine •• low doses of propylthiouracil, • ~ephalospo~. ... peni~illin. • not safe- • methotrexate, • ACE inhibitor. ... retinoic acid, * .sulfur containing drugs

W)ay is gentamicin il1~ffediv·e ill I.egio1tdlii in:f'ecdODS: •• aminoglycOsid~ .affect the: 30S ribosomal subunit. • resistance to any dotes that arc:·pr;oteinsyndtesis inhibitors may be due to- • inactivation q.f the drug by bacterial tm:yltles,. decreased uptake of drugs via porins in bacteria] membranes.· decreased 'binding of drug to 30·S. ribosomal subunit

Classic ECG'cbuges fb·r c.ardlova.sculardrugs: • prolonged QR8::with quinidine. arrtiodaront •• prolonged QT interval With sotalol, amiodarone, • prolonged PR interval with digitalis; _ p.. blockers, ,. calcium channel blockers, • giant U "?I&V~ with quinidme ... ST .dept""eS$ionwitb ~gitalis (hockey stick configuration); quinidine, .. short .QT interval with digitalis, • flat T wave 'with quinidine, .• bradycardiawith quinidine

Kno'w mecbanis'ms for resistance -.9 various classes or ·~ti~~otics: note the ones above for ~30$. protein synthesis mh·ib.ito'rs- • tetracyclines, • amirroglycosides, • ~tinomycin

'Pade'Dt' w-j·th ·Sa.lmonella .2D.d 'G6PD ·defidenc.y.:· avoid. TMPlS·~ (Sulfur drug)

'l\10A o'f·d.isuUiram. reaction '1\-ith m,tronidazol.e::· inhibits. aldeh.yde d~ydrog'enasc

MIQ,A. of Dortt-oDem: inhlbits 'DNA gyrase.

A.·void ~e·01Ic.)'~IiD·e.iD. pr·.:g,nancy

MOA of erythromycin: • inhibits 50S ribosomal subunit in bacterial protein synthesis, • also other macro lides, _. chloramphenicol, • ~.1 indamycin

CbJ~ramphellicol:: aplastic anemia 'in adults-, idiosyncratic, not.dose dependent as it isin newborns

(gray baby-syndrome) ~

Know blockers (or 4.ifTe·r:.~~nt. a~e'tylch'O'lille ·recepto·rs

Child eats. boney. and g.etS ·w.eak: • botulism, • intestinal colonization of organism ~1..th: toxin production- toxin 'blocks' the release' of Acn

A.a,ti,h'j'pettens.ives iD~re·2.$Ing bleed lipi~; • ~-b,lockers, • thiazides MO:A ~o;f·tri·D.1.etlio:prim~· 'blocks ~fqlatc reductase.

M:as~le ·relu:ao.t for electrocenvalstve Itx: .. succinyichotine

Rt lif .ptMUl.te.,. .'CaDce.r: .. Ieuprolide- GhRH' agonist blocks fSa. and tH·. and. lowers 1·~t·O$~c and ,DHT~.'. flutamide- competes with testosterone for androgen receptor, • ~de- • bl~ks 'S(l~taset _. decreases DHT~ .• increases ·te$tOsterone·(gQOd .f()r ~·Ven'in·g ilupottn,cy. andhair

growth), ~ mainly' used in prostate h~laSi~ • ke·tocon:3TD·Ie-, it reduce t~[ostcrone synthesiA ..

more often used in hyperplasia . .

·Y·O·tJ:·llg· 'woman ·with. va$ina)·cyto)o,gy co.~~·i·~ mall,paut. eells: DES· e:xposure:'·Wi.th .. clear eelJ

adenocarcinoma

csrv iQfectioa: ganciclovir 'fi~ then foscarnet if it. does not work .' . _ . ,Most c otnmo D" side-effect of' ·immu.a.o.$II·ppressl::Qi, dmgs: •. infection, .• squamous' cancer ef skin .Me cancer

'ax (J·f absence ,seim res:- ethosuximide

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EEG.in p~~Dl'withab~en<:~seizures:. shows 3/seC~ilcc and wave discharge, .increasedwitb hypervenrilarion

D'rug a·bas~r. with flu-like syndrome, fever, mydriuiis·.: methadone' +. naloxone

f7' _. ~atieD~witbpol~!i~d~o~~d~~~_ talci1'!gj~Jhi\W::;-PJ:od"cC,!; .ncph~ic diabetesuwpidus __ . __

cr' ax·o·r ADBD: methylphenidate .. __ __ . _ _

Rx of ~epr~sio~: tricyclic antidepressants- c10mipnmine blocks neiJronalreuptake of NOR (+2)

and, serotonin (+3) .

Phenytoin i:Ilterll~oD:with theophylline; phenytoin . revs. up -CytQcmomc system and Increases

metabolism of·theophyiline

'Effect 0,( chronic use of a hyplI.otic· drug to induce sleep: p~·~,nolo'gic. dependence

Patient with Wegener's granuloma.tos", b_u bellJ,orrh.gi.c.:cystitis.: cyclophospJwn]de effect

K, is the active' fO'MD, of vitamin ·K -

'Patient with hyperteusinn bas SL~like· ~dro:me: hydralazine Vaseeoastricters thSl,t incr-eases IP3~' drugs activating q.l-adren.ergic receptors

.Know drags that have their effects o:n pacemaker adi(Jq, poteatlals

·KIlow mechanisms .of action at~ecepto~ (adreDergi~ choliner-git; DY)M)th:i.laQlic releasing factors, h.istamiD~ YIP): e .. g:" ,cA:MP'" IP'3Idi3cycJgIye'erol, etc,

Iron. polsening 'in child: biggest concern ishemorrhagic gastritis and liver necrosis

Receptors, meehanistns of trausdectlon, nenro aal tracts" functions: .' a 1 'I • a1~t • ~ 1.~ ~ ',Pi~ '.1 0 types fordopamine.:« GABA, • .histamine, • 5-HT ttJ>es for serOtonin.,," muscarinic •• nicotinic •• NO" • opioid receptors+ 0', x, j.1,

~g -·f"m.ger 'with blue d,isco:I'o.ra·tio,q ·O,D~ undersurface and HI of d~g' .abnse 2.IId frequent s'exual actiyity'~ ,IV heroin, abuser using finger' as site ofinj ection that is .selling sex for drugs

Gold. salts: :. used in 'R%, of rheumatoid arthritis. '.; oral drug called anranofin, • parenteral (ann, called, gold 'sodium thiomalate, • takes '3'--6 rnths before effects are noticed, '.. c'omplications'- .: potentially fatal dermatitis/stomatitis COr.mI.lOIi, .' bone marrow depression, • flushing, '.'

hYPotensio~ • tachycardia, ,. )renal disease

By,droxychloroquiDe: ~., used in Rx of rheumatoid' arthritis, .complicati,Q,ns- ., retinal degeneration, '. del matitis, .• bone marrow depr ession

'. .

'Pe"aidOamibe~ .' . used in Rx of rheumatoid arthritis, '11) cQ'mplitations-' -,aplasti'c . anemia, • renal

damage (membranous ~Qmerolon·ephritis)"

,:Pe:Dicillin' MOA: • .~i'ac'tam antibiotic= .cell wall synthesis inhibitor, • binds to receptors .in

.bacterial cyto plasmic membrane..» inhibits transpeptidase enzymes thar cross .. link peptidoglycan

• I 11,............. . ~ .

chains in bacterial c'ell'waI1,.;.'activatcs.autolytic enzymes: in bacterial cell wall

Chlonmpl;1eaicol M10A·: • mru~its bacterialprotein synthesis, .·'binds~:o SO,S ribosomal subunits, hence' indirectly inhibiting transpeptidation by peptidyltransferase

. ". . . iated with

M~'D'a: • mercaptoethanesulfonate, • reduces incidence 'of hemorrhagic 'CYSb1lS' assocta: __ " '\VI .. ' .

cyclophosphamide by reducing formation 'of acrolein , . . Rib aVi rin- ,MO,A:·: •. inhibits: guanosine triphosphate formation; which prevents capping of Viral

mRNJl,. _ .. blocks 'RNA~epeu,&n~ RNA, pQlYJDCtaSeSl • used -in _ 19c' (?f·RSV· irifections Ondailsetr,o:u M,O:A!' ,5-HT1 'receptor; blocker-i.central antemetic

Know pharmaooki.aeti.es.: .. half-'Iitet • volume of d,istnbutiOn, • maintenance.dose . Aminoglycosides: • affect 30S .ribosomal Subunit in.bacterial protein synthesis. • nepbrQtoxic- and .o;toto.~ic.· .- 'MCC' 0'( drug-induced nephrotoxin acute tubular necrosis; _. ototoxicity related 10

accumulation in labyrinth . and, hair cells, of cochlea, .• vestibular .si,gns/symptoms-:- • nys~~us .. • dlzzin.ess" • vertigo, '. 'NN .• cochlear 'signs!symptoms:- '. tinnitus; .. sensorineural bearing loss

,H~ pylori: _. R.:<~ amoxicillirr '+' clanthremyein'+ proton')jump blocker, • 'pre. v en tie n-' 'proton pump blocker + bismuth

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AttlifQugaJ drug d~trOyillg fungal cell, membranes: ~,pglyencs like amphotericii:tB- -I' ',. ',u, bind to ergosterol rn cell membrane and cause formation of artificial POres in the, ~ yenes

. . h .. d· I' R'T":A "th-' '. "OED1'1:arte1 ,*

toxicitv of amp iotencm: 15,m ... ,.' ,,' .'. W1 " maanesium an :d: po,·fift;:ifIIs··:)··Dm.'- . U:I'I:I~'& 'J' hI'"

.- .~.I ; . " ". _. _. .,.... .' _ -_ :~. ~ .- _ .","~"';':rw:~ Wl_" .arz.o ,CS," ock

~th~IS ~f ergosterol In cell membrane, • flucytostne bloc1c$ nucleic acid syn~i~, • :griseoful vin

disrupts microtubules .

AlkylltiDglgen~ M.0A: ~.al~l~t~ D~A~ RN~ o~CIprQteins, '. leads to breakage of DNA

strands and, cross-linking which inhibits strand repllcatlon ..'

BM:~Co~ reduc~e inhibitors: ,- s~tirt ~gs bl~k CH synthesis •• hepatocytcsCOIlipensatcbup-regulation of LDL receptor synthesis leading to ancreased clearanceof'IDL, 'andIDL rernnan~

d - '.' ed '. fro- '. VLD" -' .. "L'-' , -

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SildeD2.r~:. • drug, :orRxof erectile dYSfuncti~ • inhibits breakdown of c:GMP by type 5 phosphodiesterase- increases levels ofcGl-4P; which causes 'vasodilatation. incorpuscaVe:Jnosum 'and ~is~, ~ sh~uldDotreceive nitrogIY~rin. since it causes release of nitric oxide (potent vasodilator), which elevates levels o'f<cGl\!P

Nitroglycerili' MOA; • release nitric oxide (vasodilator) in endothecal cells,- primarily a venodilator .... causes venous pooling and reduces prelOad,. has-some artrial vasodilatation when

administered IV -vreduces afterload, • uses- • angina, • acute rnyocardial1nfarction

Nitroprusside MOA: ,0 used ,in hypertensive emergencresand in dissecting aortic,c3Dew:ysms,. metabolized to cyanide in RBCs- cyanide converted 'to, thiocvanate .. mtlSt measure' levels of

.... ~J~ ,~ - _ .... ' ,

'thiocyanate during Rx to avoid toxicity

Phen(olamine: • nonselective o-adrencrgic.reccptor ;mtagoni,st~compe,titive receptor aatagonist;« produces vasodilatation- reduces TPa and 'blood pressure, Ii: reverses effects of epinephrine. • clinical USCS,_ '. hypertensive episodes' due' to pheochromocytoma, • reversal of effects of accidental

injection ofepinephrine in subcutaneous tissue .

,N.p:ro::I:e'D: • newer, Iong-acting, NS1\1I) that, b.1ocks,,~,cyc:lO(}:(y~ • very significant petential for renal damage in those-withpreexisting renal.disease

. ~.

Doxorubiein: • antibiotic, '. :M'OA-- • intercalate base 'pairs? • interact 'with topoisomeraseIl, .' generate free. radicals, ,. block synthesis of DNAJRNA and cause :DNA strand. scission, • side ~£rectS- ., dose-dependent congestive=eardiomyopathy via Uee radical dams.g<; • maITOW

suppression, .' dexrazoxane, ,3 :FR scavenger.may proteer against cardiotoxicity ~

MAO ',i:gbibitor used in treating a, ,pa'DeDt With .restiag ·tremor: • selegiline, .' blocks MOA, type, .

B" which normally metabolizes dopamine, so 'brain levels increase, .' 'hepatic metabolism produces

amphetamine ,.

Dantrnlene 'l\IOA": .' blocks calcium release from sarcoplasmic reticl:ll'W?J of skeletal muscle, • OOA in Rx of malignant hyperthermia j 0 used in Rx ofmuscle spasms in cerebral palsy

. . , and2v:'+ 'f'

D,igitalis: blocks N,a"/K+' ,ATPa~ 'p'uup in cardiac muscle- 3,·'N.~ into muscle ," -"' .. :' ~ . o~· 0_

muscle

:i...l' Rh "

A.atib:ypertensi.'ve 'wi~ C~mb5' 'po'sltive ,b,e'lDQlyti,c :aa:emj'~,:' .' mcul,yldopa" .• 'aterS .•.... - . anbF

on SUrface of RB,C'" so' IgG autoantibodies react ,aPiDSt 'i~ '. '~' Il 'hyper$enSiti'vity

, .;;.a.:1W' "'- II": " tiO· - ~ d ti' A* 0/ r"' o .. ·~ .. t·, of' acti'·tAity

,Know UuJ.ere'Dt ,ui5U1..I..II;, p're'para, ..... D$ an"· _' m'~'. _':' .. ~. ..,:- .. - ,,.-'.: . .. . .

Flu'orOqllmoloaeMOA: Ii blocks bacterial DNA synthesis by 'inhibiting toPO~~ ~ ,(I)NA

. , IV" "ON" A - .. ' . ,. - _., .. /: '1:- " " . ~. ". 'f'C1~'!Jcd D·· NA··, Involved

gyrase) and topoisomerase .. , l' • ~ _.'. , .. ,:', gyra.Sl: Impo~~ 11\ rc_axanon 0'·. W!~.~~~_ . . ... - .~ .,'.' "

· '. ~. . d - 1· ~ y - - IV:, .~' - . -. ·1"n"I"W'\~-'t- m " seearanon of

mnonnaJ transcnpnon and r up. icanon, ~ type .r '. "topQlsomerasc:~ 1.,-r:~WII' -' j~,~- •. , .'

o=p'l icated chromosomal DNA during cell division

Benzedlazepines: retrograde amnesia ,_ '.', ~ .. ;. .",'

d l~ e MIT and DIr to

o ~.-.. • • " • ~. '. , _ ... .~' '. " • ~ ".' ,. 0':"" , "', I"' . . .' , ~";I. . .: .,' .

Propylthio'u'r.acil MOA: '. reduces iodination of tyrosme, • rc uccs coup _mg 0 ". ... .. - -. _ •

formT, and r, _. inhibits peripheral conversion of T4, to T, by . inhibiting, the outer nag S _,

deiodinase • side-effects- rash, agran ... nulocytosis

- ., 'I', .. ,. .. - .. - '. ... _. .

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....... ...-

3-'

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D'ni·'g· loweriag' ',H'n.L;, ,.probu,c·ol, • 'may' cause torsa ',..:,I'""", d e 'PO'" "m~' ' •. A.i!'!' .' .~:" of." Q'.' 'RS' , ..... ,'.:'

. . " , . .' - ". . \JC -v-= ','.; .~J-' vananon

configuratron, ., type of po lyrnorp rue ventricular iachyca,rdi~ ., may prolong QT in~

Dmg~ -, atlsing rersade de.pointes: drugs that slow v~tricular repolatization and. I ... · Q'.T-

'", .' - -1 . ' . ,. ~~'-, ' .. ' ta 'I ':1 .• 1.._. thi . " - . .pro oog..

l~t~- •. q~~.h;lJ.u.~.:·· 50.,_. ~.~~.:~.~o,.;~~~~_~~",.~. c~pt]:~. . " '.

MOA .iDh:i.bit.or usociati'nDs: .: _ inhibit MAO .. ~ ,A (Inea.boli:as ,N···.'.O··;p· - seroumji~ftO;'::}-~"-. -·d'~

"":J .,f''W '. - ..'-, . . .. .... J ill: M4.-&.u.a.... an I

B (metabol izes dopamine), '.' hypert .. ·. ensive reactions iftaJO"g foods with tyramint-.b~ .. ·.· .• f:

.• 1.._· ...... ·1 ~ 1- .... _ ... _- ~ &_. , .~ . ,I Y'··':I,·ilJCt. .0 .

tyrosine metabolism, .: 'LJdJUu.la:;, • termented products like cheese beer wine •. ~.th: .... "' ... _".'

, ..' '''11 . '. ..,..... u .. .-.. . '_:J ...... a.yw. .,' OlIUmenc

reaction in that It causes the release 0.( stered catech~Jlamines· leadin,g'-' to, h.~~~o·· _n° ~

..J_;':_",; ~.L fl' ,. (S'S'D"" . .- J ~ ....... ~.... ._.." '

administered with · uoxetme .....• ~, producesserotonm ,syndrome- • hypertheunia.. "myoclonus:

(raj 1 ure, to relax muscle) " '.' ". '. ,

.Stag.e fri.gbtltes("~.Dxi·ety; give propranolol

8:1 aD.tag·o.Dim for ~, .o(bay .. revc·r

,13-2. ago'llists:~: activate adenylate cyclase to increase c,MiP

Bronchial asthma:: .rileuton- inhibits 5-IipoXygoiase,. zafirlukast and monteluk.a.st block Ieukotriene receptors

MOA .of m~th:ldoll~;. Rx of opioid abuse, • saturates CNSopiatt; receptors

Receptor involved ·ill Q.-d"iv~ ,d}~.ldne$-= ~, .

Phencyelldiue toxicity: -angel dust. reacts with opioid-like sigma receptors and subtypes of glutamate receptors. • dissociative anesthesia-. e.g., loss of sensarion un lower limbs, • agitation/violent behavior- cannot bold patient down

Aq,ciovir MOA: • guanosine analog that is activated to form acycloVir 'triphosphate a competitive substrate for D'NA polymerase+ subsequent chain ~p·on- followin:8 incorporation into viral DNA" • clinical uses- varicella/zoster virus, mueocutanecus/ genital herpes-

,Girl o·q rifampio 'becomes pregnant 'while OD ,birth CODtro:~ :pilh.~· rifampin revs up the liver

bra (1~'''''''';' al h' 1 barb ~ 1.,;_") d ....... ' __ ..J +' .......... ,...1 tabol i fb~

cytoc 'me system .'~.l.lU:; aico 0'·. ···~ .: ulturateS... P·UClyt:OUl· an . caused mcreasec metaoonsm O' " irth

control pills

i\ntago:Bist of Ci:l ... blocker: c l-agonist like, pseudoephedrine

Complicanons 0.( thiazid.es::· • hyponatremia, • ·hyp~)~.Ieml~ • metabolic alkalosis, .' hyperuricemia, • hyperglycemia, .!II ~ypcrcalcemla- thiBzid'cs are used in, the Rx of calcium stone formers 'owing' [0 increased reabsorption. ofcalcium out ofthc: urine

ACE inhibitor ~(f~ O'n' p'w'ma renin activity: mCI ¢a:,$CS due. to decrease irr A·T' n and. aldosterone ~ Drugs afTectiDg' rnbulin 111 mito·tic phase: '.·~'vinca, 'alkaloids, • paclitaxel

.J.t_x Q·f beazodlazepam 'overdose'; 'flUnmzenil

Use-of aqaeeuseptaepbrtne.ia $_~ .J : .. J'OQQ dilution', subcutaneously

·D2Jl·uolene.: used in the treatment of malignant hyperthmnia post halothane t;XpOSUrC

T·reatme.at of ac·e:bm1aop·bea overdose: acetyleysteine to replace llsed 'up' G·S·H;.... neutralizes

.a,ce~~ free radicals -fUJII.wed.:in the .liY"Cr·~me systetJ1. .

MO' r I dj'" .;j;. bl 'I-'N'" ·K· 2Cl~ 'f"roan-en.rw1'· w:np" " ·1..,oIIJIIio thi' ick ~~,;;...~ f;;:'_L in· the

'. _:' ·A o,oop' urenc.> ~ .. :oC-~ ..... a' -: .. .... 'CO---.ir~iIl~ p .. '. '. m .:W~ '.. .' -rr:;",~'6 lU.lW .".'

renalmedulla .• also blocks calcium reabsorpticn-- used-in the ax of hyperealcemie CyaDldepo~DiDg tl'e2tmeat:· atnYlnitrite is first administered to produce rtk;-mem6globin" which combines with cyanide to fOlln cyamnethemoglob1n" .. thiosuJra~' is added and competes with methemoglobin. {or cyanide to Conn. thiOCyanate.·. which is excreted

Thr1l'sb: tnay be acomplication ofa corticcsteroid inll.Jer . . .

Isotretieetn: • a.I\1(8.YS order a _ test in .texnaJ·es, • putpanents on BCP ."

MOA.o(propyltbjoonacil: • ·blocks iodinationof-tbe t)TOS1nc:: residues of thyroglobubn, •. also

blocks .: coupling of Dfl' and ~~ :. only q tha·t. can be ll$hl in pregnancy (low doses) but'IDI·Y produce goiter in th~, newborn: and naildefects _

P· 450· . . .;j;. ..... u . 1 __ ~ ~ ....... soluble

' .. ~.", system.LD .. l.Ue ... Y,c'r:: 'ma~ ~w~ wa~' .' '.' ..... ,. .

A.D·gioedeDi2 and reol (ailb.re:·.ACE inhibitor relati·onship·

·8S

......... ~

~, .......

..._ ......

.rr-

_' ...... :.

.t:r

'cr

MOA of retinoie ~c~d: ,~ behaves like a ~er(}idin that it binds .to receptors in the nucleus with ~bseque_nt. transcnpnon ~f genes, • protel~ produced b,y tlUsaction are i~t m growth, dlffermt1abo~. reproduction, an~ .embryoruc developmen~ -also used in treatment qf acute

progranulocytic leukemia, where It mcreases the matllration of the blasts _

AUop.~.OI action.ill .purine $}'1l!h~:,. blocks. xanthine oxidase- schematic of purine metabolism was provided and had to identify the reacnons hypo:u.nthine to xanthine and xanthine

to uric acid, •. mercaptopurine degradation is inhibited as well . ,

Most common IlDtibiotic used to prevent -eodOQl'ditis ,ill patients with valvulardiuase: • amoxicillin is the drug of choice, • all valvular diseases except asymptomatic MVP and aU

congenital hart disease 'except asymptomatic ASD .

YeUow coloration oftbe skin that aD be mistaken ror jauDdlte-? drug: • quinacrine. • chlorpromazine and arsenic produce a blue-gray eolor to the skin

Diffuse erythema followed by separadoD ,of the skill(scald~ skin. syndrome or toxic epidermal Qecrolysis}- ?drng; • barbiturates..« sulfonamides, • phenytoin, .• NSAIDs

Rai,. loss in a WqmaD-? drug::. oral c.~tracepti~- predictable side effect, • estrogen causes hair

to be at Same stage of development, • may also occur after' d~livery '.

Erythemato .. s, hyperpigmented plaque-llke lesion ,that recurs at 'the .same site every time: fixed drug eruption- • phenolphthalein, • NSAIDsy '. tetracycline;,. • Bactrim, • barbiturates Groap of drugs has the highest assodatiOD' w:jtli Drticari.aJ 2nd _ctllopapRlar laioDS: • amoxicillin, • TMP/SMX. • ampicillinlpenicillin. • rashesare the Me adverse reaction to drugs. with maculopapular rashes leading the list, • most .drug reactions involving skin are not type I hypersensitivity histamine-related, however, tbose involving penicillin are usually type I hypersensitivity

'Elderly woman (lD' thiazldesIs most at risk,: for develop'in,g?: gout

Tardive dyS'L.in:eSia., malignant syndtome (S1Veatjl1'~ hyPerpyrexj2~ :,a,uton,omic' i.ust2.bility):,: neuroleptics,

.,4...ntipsych,oti.c drug requiring vis,aal examjnatio'D: tnioridazine- also produces heart conducucn

: defects

Nephrogenic diabetes insl'pidus--? drug: lithium for bipolar disnzrbances

Drug contraindicated with MAO iabibitors; epinephrine , Use of' pheJltolamiDe: non .. selective ,a~Iockc:r that, . lowers blood pressure during surgery fora pheochromocytoma

,MO,A 'of ~AZT: .' inhibits reverse transeriprase, .• ' PfOduCC$. a macrocytic anemia unrelated. to folate/B 12~ • initial drU.g regimen 'used. in Rx of 'HI\l-' 2 nucleoside analogs (e.g., AZT. larttivudine)

+ 1 protease inhibitor (e.g., mdinavir) '. .

Tests, to monitor'Rx of .. HIV: ~ 'HIV RNA by polymerase chain reaction (PCR)- :. momtors Viral

burden during, • best test, • CD. T helpct cOtmt- •. itnmune status, ,. prophylaxis marker

'M,OA of .Ilcleosi,de drugs:' blockreverse transcriptase .

MOA ~f protease inhibitors: s~ my replication by blocking protein processing later in the HlV cycle

MoA' 0(' DOBbadeosid't, reverse ~tase inhibitors: non-competitively inhibit reverse

transcriptase-- c.g .• nevirapine .

Side-e'ffeds o'{ didaaosine: • pancreatitis, .' hepatitis, • peri'phera1 neuropathy .

S'ide-e:ffects of ~miVD,dine (lTe): • rash. • peripheral neuropathy, ,. bone marrow toxicity Side ,effects 'of 'in din, vir. protease' inhibitor associated .with·· renal stones,

Sid~ffect,of uen-nncleoside' ,revers~ o-aascriptase bliibi,to,n:, rash _ _ . ,

CD. belper T cell count for propbylnfs agaiDSt PCP: <2QO ~ellsl,.u..- RX withTMP/SMX, '" CD. belper T ceO COUDt for pl'9~bylaxis agaiaSt 'tomplasmosis: <100 ,ceJlslpL~ Rx wrth

TMP/SMX

,86

CD~ helper T cell count for prophyJ.xi~ •• bast MAl: <50-loo cel1s1J1l.- RX lVitb clarithromycin

.:7 R.s of 'Pb 'poisoning: BAL and EDT A

cr, __ _I~ru". i~,!qJ!~_ ip. folate metaboUsm: • phenYtoin blocks. intestiruiI-CODjugasc.... po!yglvtamate to _ mo~oglutamate. • BCPand a!coht)l block _ uptake of monoglU1amatc in the jejllDUtDt •

'methotrexateffMP-$MX block dihydrofolate reductase' .

Cromolya sodium: stabilizes mast cell Il'lCtIlbranc preventing release of preformed mediators and 'release ofprostaglandins/leukotnenes aftet the release reaction,

Metbotre~te: blocks dihydrofolate reductase and the conversion of dihydrofolate _to tetrahydrofolate

Cyclooxygeaase: • aspirin inhibits irrcvasiblr,.· NSAIDs reversible

TIlrom.boune A1: • synthesized in platelet" • vasoconstrictor .. • increases platelet aggregation. • bronchoconstrictor

MOA of protOIl blockers: • blocks H'"-K+ .. ATPase proton pump in parietal cell- not a receptor mediated event, • useful :in the treatmem o'f,ZE syndrome, GERD,. and ,8.. pylori infections

MOA of Hz blockers: • blocks H~ receptor, which normally activates adenylate cyclase producing

cMiP which.stimulates protein kinase ~

''T' l\IOA of acetylcholine: activates cholinergic receptor causing the release of calcium, which. stimulates protein kinase

·MO.A of'mlsoprostoh.blocks the prostaglandin 'receptor, which normally iaaibirs adenylate cyclase and cAlvfP production

7-'fold me·mbran,e·'spa:nni·og protein-? drug': propranolol, a. non-selective ~bloc'ker Phase .3 .clin·j,caI trials: double blind

or n,do:p'i~,e: • MOA- inhibits AD'P.:induced expression of platelet glycoprotein. receptors, which reduces fibrinogen binding and plateletaggregation, • substinrte for aspirin in preventing strokes, CAD if the-patient is allergic to aspirin, • causes neutropenia, • prolongs the bleeding time

Drug Induced SLE:. procainamide, • hydralazine

Overdose of suecinylchaline: use, acetylcholine 'blockers.

CE r, lbi ~ +, d AUb ....l ~ xrn d Jd' _1...1_,_,,_

(7 ,A ·..abl ·ltO.I"5·:·. mcrease m rerun 'an,' . .L ut a ceerease In .r ,', ; , an ,~, a, .osterone;« aicosterone

eventually increases, hence 'the' addition. of spironolactone to keep aldosterone' suppressed-s

..

increases longevity in CHF

~ .AtseDic poisoning: Rxwithdimercaprol

ChioroqQiDe was, used in the "treatmeat of' malaria aDd ;maIaria. :~rred--wby?: • exoerythrocytic/hepatic stage-: 'C: .• g .. , P. vivaxt Po ovale, • drug kills active disease but does not

eradicate hepatic stage ,

Primaquine.in treatmeat of mglarla:' not. good in th~· active stage. but does kill the hepatic stage 'of

P". 'vivax and ovale

D.aotroleae: • reduces' the release of calcium from the sarcoplasmic reticulum of SkelC121 muscle, •

. . . .

.' also d . ~ mali h "ttb .~

antispasmodic drug, .... '.' . use: ' 1Il treanng r - I.gnant, 'ypcc_OXln3 .

Metb.a-ol: • increased anion gap metabolic acidosis due to conversion of methanol into ~Qmnc acid, .. optic nerve degeneration ~d blmdness, • treat \Vim alcohol infusion to block, metabolism of metbanol by' alcohol dehydrogenase

'Botu:Us;DJ ,toxin:: • blocks the release. of acetylcboline- diagram. may be given of neurotransmitter

synthesis and must locate the block; .·used to 'treat I FS spasm 'in achalasia

7' Ribaviria: used in severe .RSV infections m,clUldren

~. ax, of asthma with aIbuterol: .• ' :~,-sel.~tivc agpnist'-' bronchodilator, • albuterol may' cause.

hypokalemia- drives K· into cells by activating-the ATPase ,pUmp .,..," _ ,

(r' Atetylcholiae:breakdowD: •. occurs in the synapse into ,choline',and acetate by 'acety'lcholxnesteraSC

in the, cleft, • products.' are' recycled and DOt' excreted

~, .....

.-....

·87

-

.._. ' ...

'II''". .: .... ~.:.·i~l'··ts· the metabol ~ f" nnnl:M"h~,ft

.... Ui U' ...... taoo 1~. 0 ~..;~ aatitris:amtnes like Sd~ ieadmg 10

cardiac arrhythrruas

. -

CodtiD~~ ~~_itzed m.tQ morphine In small amounts. o~mg to Slgrrifican: first pzssmea.bohsmof rrorphine In me ·1 1 ver

Delirium .. tremeas: Rx Vrl,th benzodiaz.epmes Lovastatin: mrubus miG C'oA reductaSe' Patent ductus arteriosus: kcep'()peQ,wttb, PGE:z

Cepbalosperins ..;. aminoglyccmdes: S}'Ilergi$tic:effeet of ~g ncph: oroxiclrv

Erythromycin: • interacts .... "Tt:h the 50S tuburut of bact.c:riaJ ribosorees leadmg- to lninbttioo of protein synthesis, • mhibus the' fcrmanon Q·fthe cmaDon complex and interfeles "'"1m tra:lS1tx:atJoc reactions, • fCS15"'..anCC develops :seconda:ry to plbuud-medi.a.ted fo.munoa of euzy::u:s :hal me .... hvlate the receptor th2t t:')~:ycm binds. wTth. • roIUOl1I15 produce 3 r::zusnzj~ble P2snnd

that produces an esterase that .hydroly~ the: laa.one nng.of e:rythr-Qzrr/cin . - .

Chloramphenicol; gray baby' S)nd:rome-: dose, related ap,lastic 3:Oe.t*Ui1,

.~: " inhibits dihydrofolate reductase. • plasmid-mediated rtsiS1arr.e, • TMP .;. SMX has :2 Synergetic effect from !he sequential blockade of folare ~ sitxe SMX :,iocb dihydropterate synthase, which is an enzyme that con:vC""'.s-paraarnnKi;er!zoic 2.cidimo&ihvdrofolic

. ~

acid

~f'KTo,ajda201e: produces 2 disulfiram-like reaction Flnecuazole. rreai;:nalt of esophageal candidiasis -In ~AJI)S

Cbolinomimetic used ill truting open aDgl~ glaucoma; • pilocerpme, 'I! l'-ID:sogi~t'iZle

~ .

Dobutaraine: • inotropic (incr-ease contractility) '-a.sodila~' (decreases afu:rload) that a'C=,l~tes' c:,

and ~1 > t:32 acnvity ~ithoUl T;illf"h chronotropic effect, .. ttsed to the neaww.:ut of shock (cardiogenic) associated with hypoteasion and bypotcr.siOD. associazed ~ith reaal failun or CHF'

Dopamlne; • stimulates cardiac fn receptors, PC::ip?laaJ c-reeepeees, and rlopaittfCtdgIC res.ep+.ors in vessels in the 'renal 2Dd splanchnic' bed, • arlew doses .. h is PI turan1y 2.'\'asodJJator that' 0,-... ezse.s

'"' '" - ... · fl · P gil ~ ~ - ., ~ 'I,; ( , -. d

renal and saiancrrnc ,01'000 1· O~/, • at 01. ' .. ooses, .If' mereases czttl3C' ~L1)f ;'IDDtro;:nCJ 'zn':

+ " , .

cardiac 9U!p1.!r via its activation of cerdiacBl receptors

- ... ,P ~ :.1...-1 !~ __ ~ _..03 ~ _'1

Benzodiazepiaes: ., "~2., its own receptors m Ll~ l.I"2;aItD'lS~ .'t-illmC' ~ ~' a::ie:!l;;!J. ~

which zre pan of !he GA..BA ~-chloride., lon, cba,nei maaolD()lea~lzr CO:lq:>:ic:x.. benzodizzepmes 'f2:Ciiitzte the iahibitcey acaon of Go&AJ3 .. -'\ via :mt.1 E2sed ,conduc~, iIi, !be cilJo:ide

19,!] channels, • tl,w,~1Zzenil blocks this effect 'by. blpc,hng the leceptOr for ixnn--dlazrpiHe.s md is 'tiJC ' ..

treatment of cboicefor berzzooiaZepam ova dose

A.D. elderlv womap OD rhi:aride:sls most 2t risk (or- dtvdopillg?: diahetcs mellinzs:

A 'chUd ~hQ iDgens.30 :adalt up~ ....m most likdylk-velop?: • an mctltued al1)on gap ~

metabolic. acidosis, • children, u;:Uike adults~ do noe etrdttxmly £kvelop a ilJiXt;d·mc=a.bnbc acidosts

Uld respiratory alkalosis, • Rx is to pqiOlm gastric la~ and ader actiQSQd cha.coaI and to

produce an alkaline urine for hx::reased e:tc:n:.tiooof tbe.2Cld. '. _ . . .

Ope:u aagle: gbacoma is best treared 'WillI?:. jiJand tJ2 blocker. • p~)QI 15 & f.avtnd apt.·

':-1~' :-' .' . . ), ."~... '~" ... ' : anM.. gj.1iI~-ama 15' .L._ Me ft.'!Y' of gJaDC(WTJaI" PSodJJCeSI

p1JUC1lpUle :nay 2:; SO. lJ!I: t!~, • opcu '-~-~-""'" .. ' .. " u.-:;: . "'Jr- " '

gradual.loss ofperipberal vision (runnel visioa) aM (ytJric mprphy .

' , .' , ~.'L.:L;...';. i of . ~ ............ r.- • sedlnon.. •

Trazodo:De: • second, generanon ~~Sl;anr, ~ ,uuUVIu,OD 0 SCOtomn .-'uy.~ - " "' .. ",

ma)t cause pnapism

Thicridaziae; retinitis pignltXUOSUDi may be- a complication _ . . ' '.

L_ "' . - bI st OSSl' ... (t!SI:ttJP COt'IIae1£S G,m

Ta.tDO:tifeD: • weak estrogen {nar ~. as '311 a:nt2gotUS% m r:el ~"+N"" . ". <,- " = '.' ." .. , '. .

, . ' '" " - .. " .L... " -...a: ~'"!rl '~onn;tt ill U%Ci'DS (can plcdace; cr-4omeaw

tamoxrfen for tnnding. to 4UI;i ~. -..-..~ -;.--- .. ~. . .' ' .... ~A.

,r . -, __ .... 'L.... I$ed 'm womrlll vmo Mwe ~

h}'PO'P1a.slA). • also' protects aputst ~ ~ can; LjIII; 1:>: ~'." .. " ' .. _.--, itxs

posmve-tumors • can be used in beanngprogcslUHrs'Sf3jd ('QdoaJ¥uia)c •• u. • .cc::~!BaIE';"

.' II , ~,u"" ,~ ". .'. : •. .-- . , ." .w ......... piuzalc.w:u

fiushmg (menopausal ~symptotns)~ 'wgmaJ b~JDg .. , pou:ntW £« ~JD, . U~,< .

'~'

88

Note: This mat~rial is tOI»pigbte<i. AU rigbts rese",td. Edward CoIjlllltM.D. 2"2

'cr-

AcetamiDo~heD:. ~,lgesicand 'antipyretic but not an a.nti~inflammatory agent, • inhib' prostaglan~m synthes~s, In the CNS.-, very weak: cyclooxygenase inhibitor,. Me dru •. cat ... I~ acute fulminant h~atlns. • converted mto fN:e radicals in the liver- • glutathione inac:vateus:g

-,---·,~~ .• -acctylcys'telne trea~t,'replaees-'GSH ._~~_.. _ .. _r •• s. e

". Digitalis tox.i(:ity! treat with digoxin antibodies (F AS fragments)

,ar <=e,phdal()sPOri~$:. flTSRxt genf·.· ~tio~~ dru(. .. !.~f cboice for surgical prophylaxis in many cases, •

sec on, genera.tlon-'." ,.,' 0' smus.·lfis ·CC .. furo ·xime·),. Rx of mixed ana .. aerobic inf .. " .I..::,._ ... ...i,.

. .' _'.' . . ' .. ' ',. .~ '. . ecnon.s, ..• :uwu

generauctl-',· Rx of meningitis, • Rx"ofGC and Lyme's disease: ceftnaxone

Clezaptae: blocks O. (dopamine) and 5~HT 7a receptors morese than ~ receptors

Fever in .• paneaten ~ loop dinretlc: • ~o not use any type ofNSAID (including aspirin) since It blocks , renal synthesis of pro~tagl~din. which vasodiJates the afferent arteriole; • use acetaminophen eo lower revert since it does not interfere with prostaglandin synthesis. loop diuretics lead to volume depletion. hence angiotensin n will be elevated (-vasoconslricts 'efferent arteriolej- loss of prostaglandin effect ptedi,sposcs' the patient to renal failure

r7" Red man syndrome: [\r vancomycin

D.rug for bacterial carrier st2tes(S.4u~eus, !"- menin:giiiiIis, H~ injluentJU:): rifampin

,q,- .Slngle 'do,se drug for GC and Chlamydia: 3Z1throm,ycm

cr· IU of ' malignant hypertension: nitroprusside

Antfhypertensive and antiarrhymic drug that, lowers bl:ood pressure and increases heart rate, calcium channel blocker- e.g., nifedipine

Estrogen: • lipid.,effe.cts.- .. lowers LDlIVLD,~ .' prevents. 'osteQooros-iS'- inhibits osteoclast activating facto!' (IL ... l) secreted by osteoblasrs, • .thr~mbogenic-, • natural estrogens are less thrombogenic than synthetic estrogens" • increase synthesis of coagulation (actors ... decrease ATm, • increases, liver synthesis oftranscortin and, thyroid binding globulin- increases .total cortisol/thyroxine levels without an. increase in, free' hormone, • tn,creases. Jiyc:r synthesis ·Qf §&X; ,hormone (testosterone or ,an,dtogen) 'bin'ding globulin- lowers i"tcc testosterone •• cane'a risk- • endometrial •.• , breast..» intrahepatic cholestasis, .. cholelithiasis

... . .

a- Oralcentraceptives: • pill effa:ts- .•. inhibit Ill' surge, which prevents ovulation, • increase malar

'eminence' pigmentation- "pregnancy mask", •. etbinyJ estradiol (synthetic estrogen) increases liver synthesis of many pro reins, :.. t '9,-nortestosterone (progestational agent) effects-, • 'water ~

retention/weight 'gain, • reduction in estrogen receptor synthesis (atrophy of endom'etrial glands). • increase LDLl .' decrease HDL, • complications- .' thrombogenic. .• stimulate' tryptophan metabolism: lowers serotonin-» deprcssi~.' increases liver.synthesis of'angiotensinogen: 'MCC of hypertension in young women, -. intrahepatic cholestasis, • hepatic. adenoma; tendency to rupnrre, •. increase: gallstone formation, • cancer risks- .' cervical, • breast, controversial, '. bepatocellular earcinoma;« protective/preventive effects of pills- • .. fibrocystic change inthe breast, • eadometrial cancer, .• ovarian: cancer: less ovulation- reduces risk {or cancer •.• ' pelvic'. in1lammalOry disease:

Neisseria gonorrhoeae not 'Chlamydia 'trachomatis, .. uterine-Ieiomyomas, .' endometnosis, • acne. * rheumatoid arthritis '. hirsutism

~ Iron toxicity: • clinical setting-« accidental, overdose o,f' fC1IOUS sulfate. in children, • SIS of iron

toxicity- • bemorrhagic gastritis, • hepatic necrosis with liver failure, '. shocklmetabolic' acidos~s" It x-raysreveal undrgested.radiopaquepills in GI tract, .Rx·_" iron .. bin·din.g agents, t, oral pbos,phare-'

or bicarbonate salts (precipitate unabsorbed iron),,·· parenteral deferoxamine

~ Orgaaophospbate -poi$oaia.g: •• MOA- • irreversible bl9Sk of acr;.tylcho~e e~tgag (noncompetitive inhibitor), • accumulation of acetylc·ltoline. at syDap~myo~e~l junc~on~ • source.-- pesticides, • initial autonomic system o'YCra.ctivity- •. -tXCCS$l Y'C' l'8.cnmabon/sah,vanon,

fecal incontinence; constricted pupils, '. nicotinic, effects Ja~ in toxicity- til muscl~

·CIr

89,

...

weakness/paralysis, '. muscle fasciculations, • low serum and RBe cholinesterase

(pseudocholinesterase), • Rx- • ,atropine ~ o~ c~oicc. • pralidoxime (2-PAM) also may be used ·

c:r- IU of hyperteusioa (BTN): weIght reduction IS the most important factor in lowering BP

;r C,a:rbonic anhydrase illhibito'rs: .. MOA--i" .. blacks the proximal reclamation of bicarbonate, • bicarbonate binds with Na" ions and 'is excreted in the urine (diuretic effect), • prodUCes a normal AG metabolic acidosis from bicarbonate. lost in the urine, • clinical uses- • chronic 1I13l1.a&eg!Cl!t of glaucoma: reduces the rate of aqueous humor formation and reduces intraocular pressure, .. alka,linizes 'the urine: good for Rx of drug' toxicities (e.g., salicylate, 'intoxication), • 'Rx at.acute mountain. sickness: produces metabolic acidosis, which is the compensation for respiratory alkalosis

rr C1in"ical uses of "('hiazides: .: initial drug used in Rx of HIN in elderlypeneets- also reduces dle

incidence of strokes and fatal Al\1.ls in this age group~ '. initial .. drug. 'used in Rx of systolic HTN in the elderly, •. one of the initial drugs used in Rx o.f,HTN in African-Americans, • R.x ofchoice in treatment of nephrogenic diabetes insipidus: ·volume depletion from increased proximal reabsorption of Na" and water reduces degr cc of polyuria, • Rx of hyperealciuria in, calcium stone. formers, • Rx of'proxirnal.RT A- increases reclamation cf bicarbonate

(F CliDJcal uses of Ioop diuretics: e· initial drug' used in Rx .of HTN 'in chronic rena] failure, • Rx of choice for hypercalcemia after a, diuresis is: started 'With isotonic saline" • congestive heart failure with acute pulmonary' edema. .' Rx of halide ,poi sonin gs-, • fluoride. • bromide, • acute renal failure- flush out tubular cells obstructing thelumen and can change an oliguric U) ·3 ,po.Jyuri~ renal failure

'7 MOA of sptronolacrone: • blocks the aldosterone-enhanced Na+/K+ pump in the late distal convoluted tubule and ·collecting duct- •. K'" sparing effect, • danger of hyperkalemia, • blocks the proton/K" ... A.. TP·as~ pump in the .collecting tub ul es-· danger of normal AG 'metabolic acidosis, .' blocks androgen receptors- • Rx of hirsutism, •. produces gynecomastia in men

CT' MOA of triamtereDelamiloride:·. '. they are not aldosterone inhibitors, • they' block, Na" reabsorption and 'the secretion of K"'" in the a1dostcrone-enhantcd Na" fK.* pump- K .... sparing effect

3:"' MO.A 01' nonselecrive I3-blotke,rs: • block J3,-receptors in the. h"eart- • reduce sympathetic stimulation, • reduce blood pressure, • decreased contractility of the. heart, • decrease heart rate, .. reduce secretion Cjf renin. in kidneys, • block i32~recqltors. in the smooth muscle and JjVCl"-' blocks

catecholamine-induced glycogenolysis ,

~ MOA. of selective 13 r-b 10 ckers 1 cardioselective l3-,bloc'ke.t:s- • primarily target" f3',-receptoTS, in heart, • less bronchoconstriction than nonselective blockers

':r' Side-effects of I3-bloc.kers: • conduction disturbances= e.g., AV' block, • block adrenergic signs/symptoms of insulin/oral sulfonylurea-induced hypoglycemia "in diabetes rneIlitus- does not, block sweating, • induce bronchospasm in those with asthma- less bronchoconstriction with selective types. • depression, • tipid. effects (mainly' in non-selective J3-b'lockel"s}- '. ·iltcrease TG. • decrease HDL? • rebound angina, • hypertension if abruptly withdraYl'll-' up-regulation of J3- adrenergic' receptors occurs when they are.blocked

or OiDical uses of p..blo.cke"rs:' • hypertension, : .. angina, • congestive heart failure' and acute myocardial infarction- increases survival, .• cardiac arrhythmias; • essential 1rcI11Or- benign condition. with trembling of hands, • prevention ofmigraine headaches. • Graves' disease- blocks

adrenergic signs and .symptoms, • pheochromccytoma,« hypertrophic subaortic .stenosts +

~ MOA of n-adrenergie bleekers: • in lowering Bf. they block post-synaptic e-adrenergic I receptors- • vasodilate arterioles/venules, • reduc~ vascular 'rcsistance~ • relax. smooth muscle .in

urinary bladder and prostate: useful in Rx ofBPH. '" unlike J3-adrenergic blockers. they lower TO , I'

and CH. and in~se ,HDL, • nonselective types block, c.'J and ol-adrenergic: bloekere- produce. too

much compensatory types of effects (e.g., tachycardia)

90

,.

Side-effects af a-adr.ellerglc: bJocke~: • dizziness due to orthostatic hypotension • . ramsin . '.

'produce' a positi ve· s~m ANA . . , ., p . . may

.,.. <x-Adrenergic blCfckers. pheDo~beaz~miDe ~d. p.eDtolamiDe~ • 'produces a chemical

- -- sympathccto~~_~ orll):'Pe.rtc1slv~ ep~es WlthpbeochrolDOCytoma until-smg;catrcm::wal of

tumor .. ,.· .phentol~ne:-,. ~ o.~h_~slve~i~ due to phcoc . ':during- . .•

.Rxofskinn·ecroslSandJschenua·lnaccl,dentali~lec···b··-·· ··f····:· "" ihrin '". ' .. ' '. ' .. '.SUtg." ery,

.' '.' .' "+.. .. . , r • ~.J' ODS 0 epmep, e or other vasoconstrictors.

MO~ of domdine:· centrally act1nga~e[~c ~gs ..... examples include clOnidine; methyld~

• stimulate al-adrenerglc receptors: and ImI,da'7{)lme reccp" tors . in tho "e CN: S' ~ red ;' '. - &r.. " .

I .,. + +' . . .... • . . ", "'P-. uces Cl.LC1 ent

penpheralsyrnpath.etic()utflo~. • ~educ~on _In blood pressure due to reduction in cardiac oU!put:

decreased heart rate and vasodllatatJon of resisrance vessels, • increase renal blood t1()w •• MOA of methyldopa- • same as for .;.1 onidine, • ,converted by-central nonadIellergic neurons into methylnorepinephrine, its active metabolite. • Side-effects: for methyldopa- .. Coombs' positive

hemolytic anemia, • drug-induceSj.E, • drug~induccd hepatitis: may be; fulminant .

MOA of ~alclDDI chaanel blockers:- examples- .. verapamil, • diltiazem, '" nimodipine, • nicardipine, '" block (-type: .caJciwn channels in smooth muscle and cardiac muscle •• peripheral arteriole vasodilator: no venular dilatation, * decrease cardiac contractility and heart rate: negative inotropic and chronotropic effect. respectively, • coronary artery vasodilatation

MOA of "ydralaziD~minoxidil, nitroprusside: .. arteriolar vasodilator- hydfalazine and rninox idil , .anenolar/venular vasodilator- nitroprusside. ... no effect on reducing, leftvenmcular muscle mass. '. side effects- .' drug-induced SLE with a positivc:SCrum ,ANA: hydralazine. '" rninoxidil: hypertrichosis, • nitroprusside: thiocyanate .toxicity

Initial drugs used in Rx of hypertension (llTN) in AfriClD-American~:. diuretics, '. calcium channel blockers- added. if the desired goal is notestablished;« ACE inhibitors or AT lI-rcceptor blockers

.~. 'iDitW d'rug used' in Rx of HYI'N U1. D'M: ACE inhibitors are theRx o,{ .c,hoicc- lITN control is' the

.single most .important factor . in: preventing a loss of renal function in "DM

Initial· drug used in ax of .8'IN' in CHF~ ACE inhibitors are RA ci choice

a- ~. Drugs used in Rx of H'l~~ In (diastolic BP :> .100· mm Hg] in ,p',regnancy: - methyldopa, .' hydralazine- alternative drug choice

Drug ased ~,Jq of ,HluN in a patient w.itlt '~ri.Dary 'retention from prostate hyperp1asia~' a: ... ' adrenergic 'blocker

Drugs used.In Rx of KIN in asthmatics: calcium channel blockers ' ..

Initial drug used in Rx of Rl'N jn, a patient. with . osteoporosis: thiazides- increased calcium

reabsorption in the kidneys . . .... .

A~ inhibitor: • decreases preload- .inhibit aldosterone, • decreases afterload~ inhibit.A'I' D

UydntJaziAe, minoxidil: decrease afterload alone-- vasodilators ',' .

Nltroprussider« .decreases preload- venodilator, • decreases afterload- vasodl~atOr. • .l_U of choice in treatment 0'£ malignant hypertension and . lowering blood 'pressure in ',2 dissecting aortic- aneurysm

'Thiazides: decreases ·preload.'alone- reduces. volume by losing-salt 'and water

Calcium ehannelblocker; decreases afterload alone-. vasodilator . ....

b I -, ~. i·A' ... nt that,: used in treatin g recurrent xan ·l..elasmas. may ·prod.uce··,t()rsade·

Pro' 'DCO!, e· an ,an.tlOXIY41i· . 15.· " . I . " . ". ' l'_'_I.U.I.~"'" , U,J .. "., ....

de- pointes (POlymorphic ventricular tachycardia~ low~ HDL ,levelS ... ' . .' '" .. " . . . .' . '.'

Nicotinic acid and flbric acid derivattves: a,COWIe 'caplilary lipeprctem lipase, hence mcreasng

the hydrolysis of VLOL in the circulation. • .' . . . '. ... .... r. .'. th .. 'since

IIMG eoA reductaseinhlbiters. and. fiboC' , aCId denvaUV6- . should not be used loge er.' ..

.. .. " ith ' '00' bd '. :1'" . 'd the po" tential for polyunc acute renal failure

they both may be associated WL r .. OmyOyslS an, .' .. '. '. '. . d d ri d . associated with

HMG C,oAreductase illhibit~rs, nicotinic acid, ad fibric at! e . ·va 'Ve!: ..... '. . : .... . ....

drug-induced hepatitis with elevation ofthe trans;amina'5CS,

'a-'

cr'

'cr"

9.1

.. ...

NQI"~e' 'Tb- ,. 1$"- material is co·pyn· . ~:1Jhted".AI1.rlD'hts r .... • ..... - .. 'Ed" .',i.--a,...., ...... :. .

,I .' .' .~. ". ' .. ' ~'" " . b:. . .. ~. Y~.. .W~.u volJ..-, Mn. 200.2

,~. ~.

(I"">.~

r:!Ir'"

ftM,:G eoA l"edu~tase inlribltors and bUe acid ..... ~b·Sojo· upr ... gul····ate··· L,'D'L'" ·,t· .' . lb" .

. l , .. .. ~,~,. . '- . . receo OJ Q"\In" eslS

hepatocytes, hence lowering seru'm CH 'and. LDL' ".. ~',' .... ':' -:r . :~.j4OJ'·· ... " ,,'n

JD:IG Co A r~u~e blhibit~rS aDd aicOtbdc atid: inhibit enzymes in their mechanism l)f

action of Iowering lipids - -- ,_. _. ._ .. , _ .. ~. . .... -

.Fluslling .s$Q(:.iat~ wi~hni~otlnie add: controlled. by PfCtrea1lncntWith aspirin

fib"G+C ~cid de::~.tJ"ts:. ~~ab···~ ovc'd·al1n'·e1fect o('alJ lipid Io,weringage:nts in raising HDL levels ~'. eoA i'roUcta:sf m I tor an ... brte .~iddem.tives: increase the cbance for warfarin

lOX lCl~, ,~

Bile .'cid resins: lower warfarin levels, hence causing'UndQ'Coagulation of'patients on warfarin BUe acid derivatives, cause a malassimilatiOn Qfdru~,that 3.fC 'commonly used in the O:ealmentof heart disease '.,

Nfcotinitatid: drug of.eh~i:ce·fo~ familial com~iried hyptn:hoJestctol~a and in JOV(eQng Lp Cal

HM~ Co.A .. redaetase .'Dhltiitors:: drugs 'of choiee for lowering t,OL ..

Nicotio:ic acid,3D~ 'tibric add 4er:iy2D.V~~ :$Ignlficant effect in lowering triglyceride levels Enro:gen:': ~ lowersLfrl, levels, .• increases bone···denSi"ty·

'Fibric acid (leri,t'litives: associated wi~ :the inamo.Pfi-ate AnH synOrome,

Types of drug's of .abuse"(DOA): .• sedatives"':' ,,·baibiiura~ ~alc~hoi.·. stimulants- cocaine •

. '.. , . • . l!J. . . M.a... . ~I I

'hallUc inogens= lysergic acid. die.thylamide. .. ->.

'Me 'DOAs in .adolesceets: • marijuana, ..• ', alcohol

tNS ert:ec;~s oflong-term drug abuse: damage to neurp~ttcr reeeptorsites

Drug screening: • urincbest screening medium for DOA:, • blocdalso used in dtu:gassays ·SYJllp~tb.QmiIPetic .DOA syJidrome:. epmp1es-" ~betunm,cs. • cocaine, • S/S- • tachycardia/sweating, ,. mydriasis: pup.iUaxydilatati~ • hypertension,. .' h~

,Opb.te.lsedanve DOAs3-:ndtome: • ·~)es- .. heroin, • benzodiazepines ... barbiturates .• S/S-

'.: respiratory d'e'pression.~ respiratory acidosis, • rni...9ric.:pupils·_·Plnpoin.l pupils, • . 'hypotension AnticboUnehrgi:t DOA syndreme: _. -examples._ • antidepressants, ,~. .3.Iilihistaniines, •

.aatiparkiason-type medications, • atropine, • muscle relaxants, _. SIS .... • mydriasis, • fever, •. dry skin

'P'syched·.eii,cJballno,DogeD;i:c n·OA. !!yn.drome:~ examples- .... PCP, ~. LSD·

;\otid.otes. used io an co D,S cious patients·: ~'d'extro~, RIO- possible hypoglycemia. from insulin

. . . . .~

overdose, " naloxone- possible opiate overdese, • intravenous thiamine- glucose may precipitate

Wernicke .. s encephalopathy in alcoholics with thiaminedeficiency

I.n(~vellous, drttg· ·,abu.s~~f (IVD:"Al:. MC· localized infection is skin abscesses due tc Staphylococcus aureus; • IiBV' ,M<:;: systemic. infection, • ~ it .' infective endocarditis- • .Me tricuspid and aortic valve, :.: .S. aureus M€C;.·.··teranus:- complication o(~$k.in, popping!'

Heroin': e· derived from 'PQP;py· plant, • usually ""c~t''' wi.th. SO.Ole. ,agent (e .. g .. " quinine, talc)-: granulomatous reactions occur in·: 'skinl1un,gs ]rorn the cutting agents •.• nQn-catdiOgspic' pulmonary' edcm.a;-- frothing from themouthis COQUIlOl'L, .,focal se&mcm'tal,glcimCruloSlcrosi§- hypertension + nephrotic syndrome, ,.~ Rx- naloxone ... a rrtorphine;,deriva!jvc,'\Vith a higA ~ty for opioid binding

sites of the mu receptor, type. .

Meperid~e~ .. Me DOA m health professip~s,. l-~~lA-phcnXH, 2~ 3 •. 6.te~~~ :(WTP.)- • by-product of ' attempted synthCSls of meperidine, •. produces .·'geY-CrSlbJ.e Patldnso;n' s ....

cytotoxic to neurons: in nigrostriatal dopaminergic pathways. .." . . '

Methadone: " legal synthetic opioid taken o~lly- mairiJy usedto deroxtfy opiate ab~ .lo~g.

acting drug- • sanaates <:;N·S· opiate receptors, • prevents, sudden .euphoric .sction assceiared "WlQl

heroin ... causesphv.1ic:aIdepegdg}c,c::Iro]gAAce: '.. '. ' .' ., .

B~nzodiazepjne toxicity: • MOA-. enhance, the fu;g!1qK:¥ of opening uP. of<iAE!AA~~W-

. . .,. d . O' 'ABA IYd'ft"nn.o· ... "tvn·c ,e·~d) IS an

'chloride ion Ychanne,ls'--+ incressee chloride ion: ton uctance, • ." " . ·=&t~~~'.'·~~\d<: ri

, :..,; ,~ I h' I ~thdra'" .. ) c: ·dtome • RX of

. l. 'b:[ 0.'- .~'~.·:O"~ .. "'·· ',5. n.u_·~.·n.·. cr· .. · • drug~·.'.. of choIce. [or aC9~Q: wun '. wa I !_m~i. '. "

)n.nl:.r orv ~ .... ~. u f;iI"U ~ _

9,2

toxicity- flumazenil .. an antagonist of bcllzodiazepine and does not block barbitur.a:tes or other depressants

7' Barblturare toxi,~i~·:, • MOA- • 'enhance' the' duratiOn of epening nn, of" tbe . G"AB", ,'A,., ., ,'.' , ",

, .'r'-." <"'r . ' "A receptor ..

- . _, cltlOrick:.ion'C~-+h·'b~~~·Ch~bl~de.i,:-conduc.tanee. .. ~ nc~ aGtivitym.m;eularactivating system-e 101 nts t e m ·.l.Uory el~~ts ofOABA!lnd glycine'(an amino.ad inhibitory

neurotransmi ner). •. bullae 9vcr p~~sun: P9lnlS: 'erythema malolo. me. • alkallDb:I~1 tile urine increases its excrenon ,aad lowers blood cOllcelltnl'tioa.s

. , ,

Cocaine: • Me COD from DOA in United States, .. MOA- blocks uptake of neurotransmitters

dopamine!NOR by presynaptic axon, •. prcdi$p05es to-. sudden deata, .. acutcmyocardial infarction (AW),. '. stroke, 4pulmo~ry edema, ., ventricular :arm)1hmlas, • myocarditis, ' .• S/S- • hypertension/sinus tachycardia, 'psy~hQsislseizurc, activity, mydriasi~

AmpbetaQJiDts; • MOA- release catechclemines from presynaptic terminals, • exampla- of amphetamines: • dextroamphetamine: Rx obesity, • methylphenidate: Rx ADHD and narcolepsy •.• methamphetamine: "ice" is: street fonn of the drug, • halluctnations- .drug that most simulates . schizophrenia

~. MarijUllD·a:. M'e illegal OOA tlSe.d in United States" :. MOA~ • contains the psychoactive stimulant /19 -tetrahydrocannabinol rrnC). • TIle binds to receptors in substantia nigra. globus pallidus, 'hippocampus. cerebellum, ... derives from leaves/flowering tops 'of hemp plants (Ctz.lv1abis s.a-civa).. • . has hi sh is extracted resin" of marijuana that has 5 -1.0 times the potency' of' the parent compound, ' •. high.lipid .solubility: me' is' present in urine for more' than a week, ~. clinical ,use;S- •.

cancer: decrease NN in 'cancer patients, •. lower intraocular pressure in glaucoma. • analgesia, • SIS of marijuana use- • reddening of conjunctiva, '. euphoria, ... delayed 'reaction. time: engineer driving a 'train involved in.a crash 'with 3fJ oncoming train was· foundto have IRe metabolites in his urine.

LSD. (lyserp,c acid diethylamide): .. MOA- =ergot alkaloid that binds to D,i"do_pamirie. receJj:tors in. the brain. =alsc blocks 5~RT2, serotonin receptor in peripheral tissue, ·.·,prediSQQ_ses to c·~mo:~o.m:al 'breekage leading _to con.gepital defects, • SIS, of LSD' toxicity- .. hallucinations, - flashbacks

p~p (phencyclidine): •. angel dust, • ,MO,A- reacts' with ,op,tOld-lik.e sigIll! receptors an,d subtypes, of glutamate receptOrs (antagonist), •. initi~l)y introdiLced as'_ a .di~iatiye anesthetic,: separates bodily functions from 'the mind without a. loss of consciousness • SIS of PCP, toxicity- •. :agitao,onlviolent behavior. '. coma with the eyes open, • impervious to pain.

Use, of ~b'locker in ,Rx of ·Qr"ave·s disease: • thyroid hormones normally upregulate synthesis of J3.-receptors. thai interact with catecholamines and produce many of the symptoms of thyrotoxicosis.

'e giving 114lloc;kers, blocks the ,J>-rec ep tors , hence blocking the' adrenergic symproms of

thyrotoxicosis . ~ ~.

Elec~rolyte changes .ihell· giving' insulln; drives glucose into the cell along WIth rpotaSSlUJn and

phosphate, " .. , ' .

Rx oC'cm-ciDoid syndrome: • octreotide (somatostatin analogue], • also used til Rx ofacromegaly

and VIPomas

Patient . webt into COD.cc:stiye bart r.tlnre. What drugeould have potenCialed this?:·

verapamil, • potent negative inotropic effect '. . ., . : . ," .,' '"

Patient with ·asthtD2 :OO .. t respondlng to albnterol; Rx with CQ~costerold medihaler .. " "

Patient witb pneumonia due II,) a g, am pOsitive diplococcns· (Streptococcus p'tltumo1JUle): Rx

'with penici II in G 1 which blocks. C'e:1I wall synthesis

'Yohimbe: vasodilator used to Rx impotence G"iakgo bllcba: improves memory' Gime'Dg: stress-preteen ve

(r' Saw palmetto: Rx prostate hyperplasia

d h 1'· ,.. ,.. lant

Echiaacea: - WO.~ ': ~ 'ea mg. • Imm·unOSt)_mu.,." .

93

NOI~; Tbis maurial is copyrlthtH. All richts reterve(t •. £dwud CoIJ., M.D. 2001

c:r 'Kava: sedative

Melatonin: • sleep, • Rx. of jet-lag St, J,Ob'D Wort: Rx of depression Jojo'ba: cosmetic, 'hair growth Soy: phytoestrogen

C,reea tea: antioxidant, decrease cancer

'V I· I

". * ertan: steep

.. ..,. - .. --_. -!' '.' ... ' ... ,

HIGH YIELD NOTES PHYSIOLOGy"

Pr:onmal.l'eaal tubllle~obctioDs (s~ Dephroa diagram): • primary site for Na+ reabsorption .• pnnury SIte' for reclamation of HC~- loss of reclamation leads to type. 0 pro,xima) renal n.tbular acidOSis. due to loss 0 f "'i~na~. • ~mary nephron site for syntbesis of ammonia via enzymatic co~verston of glutamr- : (non-toxic vehicle for carry NIL'· in t;,19Od) into NIL'· ,+ u~g]U'tanlte't·. primary site fOT reabsorption of glucose (cotransport with Nil). urea, amino acids •. phosphate F1U.cd~~ of thiD ,des~elldiog liDlb: only permeable to water, hence the urine becomes extren)Cly hypertonic .. by the tune It. reaches the loop of Henle

'FUD.cOODS 0'( thio. ascending Ilmb: impermeable to water but permeable 'to .... Na ... and C)"I hence the U'Osm begins to decrease

Functions of ,thick ascending Um,b. (TAL. m~uJlary segment): • generation of free' water via the active Na" -,K· -2 Cl' co-transport 'pwnp, • 'th:i,$Pump 'is the, most sensitive part of the nc:pblvn for damage due t~ tissue hypoxia, .: blocked by loop diuretics" • pump . also' reabsorbs calcium (not ofT! ,H -enhanced)

Fuacrions of'tbe cortical T AL segment: • Na,"/C1~ pump in. early distal tubule- Ns" and Ca ..... (PI,H:~ccd reabsorption) cations'share the same' channel for reabsorption, • blocked by thiazides

'"7 Functious of the~ ma,Cllb densa: • interacts with, the juxtaglomerular (Ie) apparatus on the afferentarteriole; • increased Na" In. the urine .inhibits .renin release, and vice versa

.~ FUDctions of .ald,oste'rone-ea.h.'Dced ATPase Na+~~ acb.nge pamp, iJi diStal CfJUectiag tubule aDd: coUecting dacts: '. Na" is reabsorbed in exchange. for K*', -e.ffect of ina eased distal delivery . 'of Na ... from more proximal actmg diuretics (e.g., loop diuretic or thiazide. diuretic~ .·.there is an augmented Na "/K.' exchange, which may lead .to hypokalemia and 'increased reabsorpnoa of bicarbonate (metabolic :alkaloS'is)

r EuoctionS of the aldcsterone eub.'Dced,:O+1'I\'" A1?ase ,p'u'mp aa;d ~ A1:Pase pallJ'ps:La tlI~ a ... intercalated cells ia tbe coU.ectiDg ducts. (USMLE.): primary sites for tbC,CXQetion of excess H"

ions-cdysfunction ofthe.lrfl(~ A~ pump. is th.e primar.y' cause for type J distal RTA

~ NO'rnJ·'·' dilution of urine: ." 'U.Osm in. the. late distal collecting.··rubuldoollecrin,g duct is' normally -1 SO mOsrnIkg~ primarily contains free water and a .smaller amo1:Mt, of obligated water 'that .must accompany solute, • when POsm. is low.,IillH is inhibited-. absence of ADH causes the. loss, of free water in the urine.> positivefree waterclearance _ .• 0110 .• v, COsrn.. ~bcre.OI:iO'- free' water clearance, V ':: volume of wine in mU~, cOsm m 'obligated lVIttt .... to calculate CO$m:' COsn1 _,

UOsm x VlPOSJI1, .: a 'positive CHlO' indicstcJ.diluriog (free \V4tcr is lost ~ ~ ~). • ~!e~, urine volume 10 rnL, POsm 250'me>sm, u'Osm 150 mOSm: COsm. II;; 150 x l OIlS O' ... 6 ml., CH~O a

1.0 ' ... 6 = +4 ml,

~ Normal co DC entratio B, of uriue: :. increase in 'POsm ·is a stimulus: for ADH release .. • ,ADH ~ders

. - . bl & (N ... t --.t..sorb obhOtiled

the late distal and collecting ducts permeame to ~ water not a , canno IQW:' .'. ··r.·

v.'ater)- urine. is .eoncentrated, • negative CH10 (free, water is reabsorbed back into me. .bl00d)-, e .. g+1

94

~otlf:Tbis material is copyrithttd.AII ricIa~ rtsc:t1al. Ed:wvd Col _. _ r. .., ......

- ~.~.

urmc volume ro, mL POsm 300 mOsmlkg. uOsm 900 tnOsmIq: eOsIn - 900 1 [01300 ' " , CH::O:: 10 - 30·,= -20 rnL. ". ability to coneerurate urine: is ~ fl· ,.~_~,:_.' ,.-.30 mL.

- - - . --' ". . .- . -. '.' ..' _. . - ast GUI..lUlllla.Uty In 10);1) failure

CeatrtI ad :bepbrogelll~:DI: • both have low UOsm and increased. POsm, • -', - I DJ sboovws _

500.-. increase in UOsm w,th administration or VZSOpres.sin. • ~c- OI .,1.~tta~lW; - _ ':

tn llOsm "Aim vasopressin ........ ~ ... ~ . ~ <."J"\,F.I'. IQQ:easc

R·es:pira·tioa chaD'ges with incrnsi.RC .aldbld.... • rcspit"'~J1 ... '-I- .. :r ._-= -:_ '~. _ . _._ _

- .' - . Too, '. a"'-K'! ~U~ .4:Iyv%ODJ.J due to

:;;~ atmospheric pi essure not a dcacase m %oxygm in aJr, • ina else m zj BPe; right shift,

£ffKt o~ 'lP (,,-.soiate$ti.ru.l ~f) ad "UpbaliQ5 08 GI trztt • VIP- relaxes smooch muscle. U14;ICSsa mteSti:nal secretion, _ maeascs_ pat¥;le1_tlc sc:crttion,. mkephaliDs (op1arcs)contract .. SUJOO'th muscle. decreasemresnnal secretIon

KDow PCOt dissocb.-tiOD· ·curv·1!

. - . -

labibilt:· syntbesizedm Sertoh cells in sc::nunikrous tubules, • DC~ feedback wjth FSH. • mcreased if semmiferous tubules are ~ -normal if Lqdig cells are dc.stroyed, slnae

tes -: .sterenebas a. negative feedback. v.ith LH "'. .

C.alca.l.atioDS~ • alveolar ventilatioo-- 'p~Q., ;:;:. PiQ., - P~C~I'D ._ . .:&.._ Pi.~r... ~~.J'1f' oj _ " .

. - -' . .. . -' . ~ - " .~ to· - ..... ,n.., w,~ . '-'2. ~.~ f, oxygen x

:13) anil R is the resprratory quoneat that nOI:Il::ally equals 0.8. subtract Pa~ from PAOz and you

ha .. -et.'le:A,.a gradient. • Fick's equation for -ca.":diac;: output- card:.:ac output ~mm = a:t)gt:n consumntion 'of-- oxygen rn pulmonary vein ... Ox)"gen In pulmonary arta'V

.• - .',,, .~.. i

Hormone iJl,crelsingldecre25iJlggastric SKl"rdoa: • g:aS.t:'ln. • hormones inhibiting acid secreoon- secretin and zastric inaibitorv oeatide

- ~ ~-~

·P'Ia.cenul ·2_'natom·y(-·ph~-siolog)·: •. rratemal' surface. ra'5- slightly bulging areas called ~

which.are CO\~. by 3. layer or decidua basalis .. • fetal surface is entirely C(J\'e: ed by tbe cboriOllic plate- chori·on.ic'vessels conver ge with the umbilical cord, g.ilich. is composed of:2 wabilica1. arteries (venous blood returning from tbe fetal hart) and 1 umbilical vein (carries oxygc:wcd

blood. from' the placenta), • chorionic villus/umbilical . cor c--· ., ·chcn.ooic ,..111i pt·Qj.ect in. the mtervillous space, which contains .,!l13.n:t"T!3j- blood from l't·hieh oxygar is extracted; spiral ar~es;

from the=: Utc'US empry into t.y spacc~ . ., chcriooic \1]1. zre L4'""leQ by trophoblastlc ri$Sl'':: 0UlS~ layer is composed ofsyncynotropboblast: synthesizes hCG ·ar£{ human pll(O-L:ai.lactogcl (gto\\'th

.... .• T'-... • mposedof ;;.. -'L,;_1 1 _11#· - - f

hOI'UlODe of ~.): .and. insice ·~\~.15 cor . '.~' " ... ot C\tocroo··Uut;j;2St: clear ceus, me· mtenor 0;'

.",._.. .. . .(... ~ ..

tile chononic \.111us has ktaJ blood vessels, \\-mc:b. coalesce to form the· :chorion~ v~·S$C1s that·

converge v.,'t.l] the umbilical cord, • the umbiliC3l cord contains 2 umbilical arter.ies (COIl 0 ins deoxygeaated blood exiting the fetal heart 'and returning to .t!:tc placenta) 3lld 1 tJ:lIblical llciJ:a (contams oxvzenated blood)

\. -- .

·£trect· 0'( mcreased .. ·tottl peripheral 'I e$b---u:ace OD \""1SCU.lar mactie., es.rve~ • sa_me "as !be \:¥:DOUS

return curve, which depicts the relationship beotVleca veooes return and right atriaJ. pRSSW.~. • ~g TP.& (vasoccesmction) decreases venous 'rerum to ~ Ca:reasc "p' atrial ~ znd dc:xease cardiac' outDut-, this causes-a coWliU_ .Autio. of tlte ~br .fww;dtMI

L • _ ..

carve, • do:re1sm.g TP'R (vasodilanoe) C3X·JSC'-S··a. clockwise row:Ic:m oftbe curve.- \,.'C!M)U.S !enatt

i:nae.aSC'S ... right atrial pce.s.sun: lnaeasc:s •. and cardW: output increases , _

IICG:- bas lutrinizing bormooe acti'\ity1 .• kcc.ps corpus lmeum of pr¢8PIDCY' sytJEhrsrmlg.

'progesterone Ull.til 8-10 \\T5 and tbcn·.·pIKeDta 'Dkes Q"w"C",thar ~'~~on ~ .'_ .

StimDb,tio'u of hi.stunlne H, reaptors:,increases se:renOQ er acid by ~rietsl.cells _

GI peptide iaj·ect,e(f iDEO (ereb~ hid ·lbt iacreases ~ .f. ~JII:niU::

S01IU~cn-- dns normally IS made in hypothalamus w~ it fimctioas to inhibit growdt homo""". hence ,"~·to· unng G-'H" ·~USeS .b,,~I.-cemia and dx: bodv woeld crave too1C ~ IOiIlUUge

• ,._. ~ -L.ii.I..... ...........~. .. ._" WQ. ~,I',~;etJ,- ....

. .

· 1 - ~

!~~ ':~:etic peptide: mediarrd. by guanylate cyclase, increased if ldtor O&ht &II"1&m IS

1 •. , ....... _.J_.J

vo ~ UIIlt 0\ C. ioacec

9.5

Ceatral diabel:es~asipidD$: U1Jecongva~opressin CRUses an increase in unne~ (POsm).

and. decrease m 'UI1l"1e: volume .

~tio. O~'~'~e:a~~ CO·,,·ertiIl·~·e~~ • 'pu)mcmary czpillari~ • lnClrased. in .SUC01~ •

_n~~~~ pWbtn'OE.. bv ~A,.CE mru~~ ..

VlDmi. Q_ tl'P~ iakicbrrys: • 1.25 {QHh~-·l"__·_'__· -:-ki~~dn:-·.··-~- .. _-=-ba~· -$ ~J-a-.~-., -~-: p-xy-" -las-se-:' -in-' '-p-rox-·-u-n-·.·'l ........ ·. ·tu-,.··'~bu1""""'~IL""_'_1:S· .•

(prE snmulates synthesrs], '. renal disease MCC ~fbypovitJ;millosis '0'

.E.:'ea of'thyroid hormone' excess on booe: osteopotosis- matl5eS bone:" tumoVtt -resulting in loss of boae mass .

Primary sitr for' tempenttllre :regcdatio·.,: • ant:crlor· bypob:laDl11S." • fever is _. to release ot p)~ stimulat:tng· Il, .. l release nom. maaopbages- ·n. ... l inaea.ses synt1v:sis of PGE.,. in' the an'te;ri~··hyf)O~ "PGE: raises 'the ~])otbalam:ic SCl-PO'lnt (normal core'.to)tpetarurc.JL,;~, &5' 100 Iow), ~ce anterior hypo'~t. reactions of beat gcn.erl.ti~ prevail, • bal gcx:ranng mecbanisnlS if core letnpennrre 1S below set-point ~1ude- ·mcreasing ~ releast; of thyroid hot :tones (incre-ases: metabolic rate), if:"SOCXJItS1Ilctian. of' ski:n \"eSSels (sympaIbetic ,stimulati em of c-reeeprors ,in .smooth mascle), *S)nll.-.atbctic stiInulatitm olf ~ In bro'Pltl fat (inCreases metabolic rate and ·heat· ~on), ·sbi~·ing (ll1OS1 efi"ec,tive sy~ center: l~. ~ postC.i~ hypotbalamus, leads to, 3cri~-;ation -pf a ~ r tnOtoneurons ilmcn.~g skeletal tnllSCilei .. heat dissipatmg mecharasms (coordinated in posterior h~1'Othalamus) if care'tem:oetzrurc, is. above setpoint mc,iude-- ~L,g' S!·.,&ilfiAthetic tone (vasodflatarion) of skin vessels l~ding to slmn~g of blood 't!Iro~ venous plexus 'm the: skin, -increased S)inpathetic activity of cholinergic fibers l~-o.~-"\ ;an._~,£ . S'\\"CSt gla."ds lcad-~g IO sweatmg

..... ,_.

Cause of decrease in jUgnlar \te'DOas p'ulse oa. i.Dsp:iratioa.:· .• decrease: in lung compliance" (e.g.,

sarcoidosis), • decreased volume oi ait in the alveoli .leadS to less comnression of vessels m' the

ntcsriti!J tissue, hence :he\~ ::.11.. t!O better and ~. blood ott" easier · . .

. '...

l.roa re2bSDrption:' •. meat has 'heme L~ \\~ch is ft;110US ,and easy to reabsorb in ~ small

mtestine .. (primarily the duodenum): • once absorbed into ·:al~~ hetnc: is 'enzyma:rically degt"aded to release; iron, • ~ of tbe UOIt is diverted to storage as farit!!J in~. enteroeyte (~le.d apofer .. itm), while: 3. sr.naU amount is delivered ttl, pla'sma t.a.~iell !r4.!he' circulating binding, 'protem of iron. • nlants hav ~ 'non -beme iron, .... ruch is m the ferric state: .. non -beme troI1 firs! blnds to

- - ,.. - .. .' .' . - .

mucm in the stomach (renders n soluble), • the. mtIC,m-nou-beme complex then binds to Plotems Oil, ~ ~~ .. ~"CO' ~'";I;··1 surface of .. he A ... ~-~·-urn·· frwr' tr.:rn~ into t..~ cvtosol '\N.l,...~: it is ~nnrat:, .... allv

,j,J,,Ji; .. ii;,,i,~ .~ .... ~""""", .' : ,~- ~~," 'VI --f.""""""""" - , ..... J.'~. :')1;1~ . -J. . ..... II"".A.L.~.

deeraded to release Ir0i4 .' ~ cvtosolic ~teIn called mobilferrin transfers the' mm so the muC~

~ _. ~A'-~

~crntm stores or to transferrin in the p'iSSl.ItA.+ .• 9ihen body ~!e5 of iron are replete, mucosal cells are shed ",",th theirstored mucosal ferritin iri order to ~,~t. iron,' ~\~ioad and, Inl;.~idl'in.s:~thesis

'Uj t..'ie 11\-~' IS. decreased, • l\'hcl body stores are depleted most of tile- rron is directed towards transfen iIl in the. plasma and trBnsf6,i ill synthesis in the liver. is incrtasoi.

Kno,.- ventricular Vltbtmt. CIIl"Yt ad wll.en ,differst'.earl sollllds are .Iocated: e..g409 ~. (closare of ~~.\(+ and P\1

Rea.". re.bso,rpoo'. ante: glucose is most o,ftc:n asked. about

Demo,· hypothalamus... ",~b:at lJormoile mere:a·sa.: prolactin- no dooammc to inlnllit

meet ',~t ~ti.d .m.ssag~: sJ09r-S; heart ~' •

Major site <tf ,,"a.let' rnrb$qrptioD ia the GI. tract: tn. ~g otder._j~junUIT' ... , il~ colon M:ost efft<:tive nephrQD site (~r acid Me edOa.: proXimal tubule: of the bdnC)~'

!' eparon site lor ~.W B effect: collecting r.ub~;1l:c

.Sephron ;site tor 'greates.t t:.eaua.tipD: of (ret '_..ter"/1DoSI sasc:~tiblt to iscbemi,,~ tlock

ascendina limb In the medulla

COllsai:dioD of the IttTe·TH.l a"e:rio~: mer-cases the GFR and. decreases renal plasma. flow .

Flltn.tio·1l fra,ctiC,lD La tb:e gl:omemllS: FF = 'GFRJRPF; constnct aifC'enl.anenol~ no change in. fF

(.!. GFRl; RP'f"l. constrict ,~ffC:Cll arteriole j,nauses FF -ctt GFRI ~ RPF)~ :inCrcase.. plasma protcirt

96

concentration. hen,ce increasing XGc'. ~ the FF (,J. GFR!no change RPF); decrta5ing pl.sma protein concentranon, hence decreasmg ~. mcreases the fF (t GFlUno change RPF):eoma icting

the ureter, decreases PBS, hence d~ _G~ ~d the fF without affecting RPF (~ GFRIno

change RPF) , ._._._ '-_.p - _T, .- _ ... _ ..

~ Starling eqaatioa for GFR mJlmin: GFR "" Ki[cPGc - Pas> ~ 1Gxj. where Kfm filtraliancocfficient In ml!rnin . rnrn Hg, P GC = hydrostatic ~ in the glomerular capillary in mm Hg (pushes flmd out of the capillary; e.g., +45 rom Kg; .11 1$ mCte3scd if the eff6ent arteriole is c::onstticte<l arid

decreased if the afferent arteriole is constricted). Pas - hydrostatic pressure in Bo'\VJna.D·s ~pace ia nml Hg (pushes fluid into the capillary; e.g .• -10 nun Hg), and 1toc 0::: dlConcotic pteSSIlre in the glomeIwar capillary in mm Hg. (brings fluid bad: into the capillary; e.g; -19 ·mm Hg); the net filtration is !he algebraic sum -of the above 3 parametc:rs (in the above e:ump1c. lhe nC! ~ would be -10 + 45 -19 == + 16 nnn Hg}--fluid should move out of-the glomerularcapilbries; question: what Starling force changes to producea net ultrafiltration of zero (answt::r: 1tc;c. which becomes increased [pulls fluid back into the glolDl:(lllar capillary; using the·above exampie- 10 + 45 - ~ = 0 netpressure]; it is the only factor thatdoes not remain COnStanlaJong the lCDgth o{thc capillary owing to differences in proteineonccntration; the high. 1toc at the end of the: glomcruIa:r capillary extends into the peritubular capillaries that surrounds the nephrons and allows for the' reabsorption of solutes).

~ Gra.p.b of pEl, PCO:., bic:atbonate: ~ 40 mm Hg is normal, b.icarbonate.24 mEql1.. js.,.OOllna1

.•

.. . ..

..

., .

...

") .. rnFqJL

_, .. '~~-" ."

Bicarbonate

.,

. '~ F

.. .. ..

" ..

.. iii .. ..

• •

iii,.

..

7 .. 0

7 .. 46

1.8

, Arterial. pH' ,

• panent .A-- ac.ute respiratory acidosis ·~th 'very lime. bicarbonate ~¥,?satiCXl. • patien~·. ~ chronic respiratory .acidosis where there is more compensation by inc;t:aslng bicar~1Ie (~~C alkalosis) and .pH comes closer to normal range ... patient C- metabofic alblOS1s· ~: there 1S compensation by increasing PC0;z (respiratory acidosis) • patient D- ~ actdosis .wbe:e there is compensation by decreasing PCO: (rcspinttoI)' alkalosis) •• patJcl1t E- ~. ~ alkalosis where there is very little drop in bi~tc. for COtn:peILqtion (mecabo·uc .~dosisJ. • patient F- cbroruc respiratory alkalosis where there is good, cornpcllsa·tion by droppiagbica:rbolJate· (metabolic acidosis) and. pH is closer to 'normal

(r • Negative cbarge of GBM: heparan sulfar: . . ... ,. . ... ... ( tIM:

51 hHI1 souod correlates with CwaVt.1D the JllgalarVetlOas pa.1_ses (C waWI$ t.Iosare0.'

tricuspid valve); know the jugular veaous pa1ses: see box in cardioV3SClllar clwpter 00 .. Jlb:ysicaI

diagnosis . ~.. t-_...__,

Ca - id d' hea rate and' . 'm' a'eases ;i----...I.,;:IIII!Ii·toft.~ (~d $IIlUS ..... y.~

.... ron m.ssage: • .;.ec.lrases u~.... . . - ,~ ~~ ...-..........~- .•. · of

. . ed b' ... l...-·IX and X·,th· nerve • I~,lses: generatedin this receptor inhibi.t tQQJC disc:bat&c .

fnncr,l3.[ .' y u~, .' '. ' . >. . , .' , '"'"" .. ..,.'t _ !t&~ "

NOle:' 'This material. is co·p.yrigbled. All richts reserved, Edward GGIJaa...M.D. 200,2,

. ~

...

the vasoconstrictor nerves and excite vagal innervation of the heart producing vasodilatation venodilation, drop in blood pressure and heart rate,' anda decrease in.cardiac output) . ,

3% Hypertonic sallne . and. effect on POsm 2Dd ADD le\lels: -3% hypertonic saline increases POsm.,._. incrcased·POsm. stunulat~_~ rclcase,af ADH(iru:tcascs) •• atrial nattUueti.c .pcpIide is. also-stimulated and n'!"rma1ly does ·.nhlblt.~H.·n:lease., however",.hypertoniciry overrides ANP

~aU~ilt walking briskly OD a hot day: no mcreasc in rectal temperature, vasodilatation of vessels In skin

MarathoD runner ~D !II; bot day: • increase in rectal temperature • ., vasodilatation of vessels in skin.

Vagus nerve fI,lDt;tiO~ in the. stomach: - .i.ncreasesgastric acid secretion and gastric motiliry'vagotomy decrease~ acid ~n'on and .morility, • the vagus nerve.represents the paras.yntpathe·tic innervation of the stomach and duodenum

Sympathetic nervous system in t.e ~omaclaldq~eaIlDl; • inhibits secretion and motility ••

sympathetic fibersare located in the celiac plexus' from T5~T9 .

'M'otilin: 'hormone modulating GI muscle. activity

0'.9'%· .Dom:la:1 sa)-in&! with KQ :is :the crys.tal.l·oid solotioDthat· is most ofiea usedl to replace . gastric loses

Creanaine clearance (CCr).: • creatinine clearance fonnula- • CCr = UOsm x V l PQSJn. where V == volume of a ,~4. hr urine collection in mIJITtin. •. creatinine is not a perfect clearance substance, •

causes of a decreased CC·r-· increasing. age, • inadequate 24 hr. urine collection : decreases urine. volume In the numerator, • renal failure, - causes of an increased cCr--· .• normal pregnancy: increase in plasma volume increases GFR and .CCt, • early diabetic nephropathy:

FEN.a + (fradioD.Q .excrettcn o.r .so<l:i ... m): ~. useful in the 'workup of C?lig~ia- f'ENa~ .~ (UNa· x . per) I (pNa.· x UCr) x 100., ..... values < 1 indicate. intact tubular function, .• J values> l{USWllly:> 2) indicate: tubular dysfunction

My·acardia •. p.hysiology: • .. cardiac hypertrophy increases wall stress, sinus tachycardia reduces filling of the coronary arteries, .• 'increasing heart nile raises myocardial. oxygen consumptioncardiac muscle uses ~xidation of Catty acids for energy, .:. venoconstricuon- incleases preload alone .• • restrict salt and water .intake- decreases, preload alone, .: increase cardiac contractility- n~ change -in preload/afterload

";

Normal g~ physiology at tbe .tisslle level: • ~~ derived from tissue enters the RBe' and

combines with. H20 via carbonic anhydrase to form H2CO,~ • H2COJ dissociates into W and HCO.~~ the .. latter leaving the. .RB·C in exchange for cr anions,·. W combines with oxygenated Hgb (HgbO2), which releases-Oj (Bohr effect), • 02 leaves the RBe. dissolves' in 'plasma and increases

capillary POl

Normal 'gas pbysi.o·Jogy at- the .pD)mODa,ry Ieve]: • Alveolar Oz diffuses into the plasma of the pulmonary capillary owing to a greater partial pressure of alveolar O.2~ ~. O2 enters the RBG and

combines with ferrous ions.on deoxyhemoglobin 'to form QxyHgb and 'W·i. • HCOj"· enters the RB~ from the: plasma and combines with a+ .t.o form .H2CO]'t .• Cl" anions leave: the RBC: to counterbalance the entry of .HeO)" I' .. H2CO, dissociates into Co, and H20;, CO2 leaves' . the RB·C and dissolves in the 'plasma to increasethe PC'Olf which enters tbe alveoli for excretion

Important anatomical considerations; for urine CQDt.rol:. • urogenital diaphragm- • muscles of the urogenital diaphragm are the-deep transverse perineal and sphincter urethra muscle, .. ,: location of the urogenital diaphragmen a cystourethrogram is just distal to the prostate ina male. -'III locatio~ of the. urogenital diaphragm on a cystourethrogram in a female is the beginning of urethra where ·tt

exits the bladder ~ '. functions of' the detrusor muscle- • relaxed: storage of urine ;in the bladder, • contracted: emptying of bladder, • functions of '~c sympathenc in. bladder control-· • relaxes. the detrusor muscle: aids in urine storage in the bladder, • contracts internal sphincter: in-creases urine'

9.8

. -&I "', .'. bt ...... · AD' ....... · Ed

'N"O,~e:. This I1li.len', IS cop:ynz.;u. ",' " '. ~ ",,;,u, reserved.. '," • (want (""...AII,_. '. M.D 1'~'

.~~ .. , .• ,~£ .

cr·

:cF ,CF-

. .

storage" • functions ,of ~,~thetiC system-- • contl~ the~dctrusdr" muscle: empties !he .. bladder, .• relaxes the internal sphincter muscle .~' bl;V-~ft,. :~theti .< ·'httiiti· " a1:',,,,,,:- ,,~;;;_ , "

. ~~ ~ -,,-,..,.' C mm 'oro luw::il

emptying 0 f the bladder . . . . . '.' .. ' . . . .... '. .' .....

Thyroid bOi mODe .- 4ilIuscs 'into ~. • b~ ,to, receptors intbc nucleus! DNA biucr :"

d· .. L-.. - fl'" . ..___ 1.._......._ ,,' ··-tIt ~~I-- bind~' . . , 109 " Oma'U11143' zmo ' m:gers" Ourc:' llUll,Janle! 'Wl' .. :sum.·nU;' '., .. -~tilg: ,stcroid$,.~ aQd",.vitamil1 '0 ;

Ih ;~~' I~' .L

AnPO"tl;'ilSiD·og,e~: • syntnesizeo in :Iver. ~ increased D'ntbesis' flom, esbO,gen.,

.Action PO'" " teDti21 curve of cardiac muscle: know' what part ~A:~, to' .1.._ T':"'~ '" ".. (. ~ ft,L+- .' "

. " ", ' . . .. . ., '. ,~ , , " ~""'~I""&~ .: ". ,U;.~ '. 'wa~' n~ II' the~·

'en,d).'· .

Ve,otricu.lar- .ud ~a,o·rti,c pressure Cine: where isS, ~ .. of'diastole)

S'D.~ce :r'eqairiq, A.TP 'fo'r r.tabso:rptioa Ia the:·sm'.IJ. bowel: •. glucose, • ,ATP ~M~'. far

-'th' N .... ... ''''''"'1w.&.11. """""

cotransport wrtn , ,a,

M.lor vehicle ro·r· cstTyiDg, co, in 'blood: . bicarbonate. (~7O-h).

Vo,I'ames/capacities 'DOt- dl~ 'm"Sitred 'by···· U\iro,"~ • residual ·Yolu"',6 rl:J'V)"', ",- YO'lmlle O"r':'

-, -:'. I' r ' --"- .. ' ~ ... "_ ......... ,~ ..... :J'- -,"._ -;. .. I ..... ~ ... ~""J'. '_ .... '-_".-_ -: .. ,"-.....- .. \~ II! ' .. •. •.. :,"

air left over after maxima] expiration, '. 'total IBag cap'.cIty (TLC):' amount 'of air in a :fWly expanded Iung, measured with a nitrogen 'QT ~li1JlJ1, ,dilutioo '_tbod.. • mac:tIQ'uJ resid.,a! cap.dty (FRC):' total amount of air in the lungs at the end of a aormal expiration, (~d 'of the' tidal

. . .

1 [TV]' ') b' ined b =, rheli diluti 'techni " .~.'~ 1_',,""~1"'Ii"_t

VO' ~e, . '. . '." o~ ,~t;.U . 'Y ,a ~. e Iwn .. nnroutecn ,': que or ~,i."P ,~cU:&j~'N!7L~'ui

Forced "itaJ 'C2p.'city (FVC), forced, ·expi:tatory' V9ID'JDe q" 1 ~8d ·.(FEV t.J .aad FEV"l..JF'lC:: - ',F\fC: is the total amount-of air expelled after a IilaxinlaI insp .':., iration; the :RV' is 'what.

, '

'is left over ar the end of maximal .expirarion: normal FVC. is ~ liters, ' •. forced expilauxy vql\lllJe: 'm

I second (FEY 1*): 'FEV"1scc' is '00,\11 much air a 'person can' expel from, the 'lun,~ in 1 second, after a maximal inspiration, normal FEV'lxc' is 4 liters, '. ~o~ of ,FE,V I.IFVC is noram.Dy ·'O.~80,:, 4 ,1i~S Iiters = ~O .. 8Q; • peak: expiratory flow' meter: outpatient method or evaluating' forced \rltal ,capac1.ty'

(F1lC).~ commonlyused by asthmatics to evaluate their airways ..

Method of measuring RV': • subtract' the expiratory' reserve: volume (ERV) franl the fimctiona:l residual capacity (FRC), '.' ~V" is the amount of- air f9fCibly expelled :at t;be end of a notI.nal

expiration . (' en, d', '0' , f. '. the 1V) .. , • ERe' '=, ER V ,+ RV' therefore'. :RV'·~: FRC' -' 'ER V'

. . ., .... G "","F ,..',1! .. , . .. . I" '.' .,' ' , . ,. .. .

Diffg,si,o:Q' capacity '(DLco): ' •.. D·Lce is the method of :measUrulg.'~ .mlity of a ps to, diffuse.

through the alvcola:r/capillaxy interface: utilizes carbon· monoxide (CO). • DLeo is primarily· dependent on the following ~e~; COreacJling ~the alveoli (decreaStd due to _V/Q mismatches: e.z .. atelectasis; ,COPD)t CO. crossing, the a1v-cOlar/Capillary interface, (decreased due' to

u,~ " . ,,- .. ,~.~. ',' '_' " .,' ... . "."..... -:

'pulmonary fibrosis-or fluids, in the mterface)

...

99

'.-'~ _' ,.- ,._-_' _,- , ,-, , - ,.,---,,~- _'_- -_. -,~~ ., .. --'_' _'-. . --- -'~'~~~'-------""'_~'---------=----,

r------------~~~~~~--~----------------------------

. - .,- .~,

~ Seconds

a 1 ·2, 3 4

I

, , -

TlCVC

J

5 . - EV_c __

t

RV FRC,

il

A

Fl,GURE 11-1. Schematic of: the normal IUDg vol'UIIIeS and capacities and the forced expiratory volume 1 second (fEV'u-J and forced vital capacity (rYe) in a DOJTIlal person (A), a ,pei50D witbr'c sttictive lUJIg disease (8),: and a person with obsCructi,ve lung diwase (Q., ,FRe (functional residual capacity) represents the volume of gas that. remains in the lung at rest at the end of a normal respiration and is the most reproducible part of a, pulmo-

fundi- t . .." it d t · + elf p'

,nary , ." . ,on '. 'es ... SIn,ce, I " oes ,no reqwre patient .', art. . ,erson

A (normal individual) has an FEVlMC of 4 L and an FVC of' 5 ~ with FE\' ~C' ratio = 0.80.. Person B with restrictive Jlmg

disease 1...- - . .. ........ ntarunzed" .....,1...-t:PA f .l N t ... 'L.._. tho e

e' lJ.c1.:) a 1.I..LLLJ..LCU"u.J" ~ .. , ..... I.&.I.,.~ 0, person, ,,,....,. ·,'oe U~ '.'.

FEY tMC and FVC are the same (3 L) owing' to the increase' in eiastidty in the lungs, 'hence FEY ~C ratio = 1 .. 0. Person 'C

... th th I.....-...._,.,. ...' ha + difficultv · II"'~'

W1 •. : t e obstructrve pattern IS •..... vmg ~1~~U&L;t In ext>e.)lug aJr

from the lungs owing to decreased lung elasticity.. FE\' ~ = .1 .L 'FVC' = 3 L. and FEV...,JFVC ratio = 033. Both people B and C, have reduced values for FE.V 1..: and FVC 'when compared wrtb

th I h B • -...:..,. L. ~ ..,..,...,..;......... I' dise norma person; iowever; person ,'·.'W.I,UI, restncnve . lIng as i

ease has values. between those of the normal individual and person C With obstruction, Person B. has decreased compliance, in the: lungs, hence less air enters the lUDgs~ but owing to the, increased eJa~ti~ the air that, isJn the luogs is expelled faster., Person C, has Increased compliance (air easily' enters the I.lmp); however. reduced elasttcity causes the lungs to trap air on expira-lion. hence FEY be and FVC are much lower than in person B and the ffYl..JFVC ratio is, reduced. Tl.C, total, 11101 capacity;, Tv.

tidal 'volume; VC. vital capacity ..

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