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Sanjay Sharma Summary of Difficult Topics
Sanjay Sharma Summary of Difficult Topics
15. (32) alports syndrome: x linked dominant: renal failure in males (females haematuria),
senorineural deafness, ocular abnormailities (myopia, cataract, retinitis pigmentosa see
pic). Post renal transplantation anti-GBM disease because original defect was type 4 collagen
in GBM (goodpasture antigen).
16. (34) complications of nephrotic syndrome: portal vein thrombosis (abnormal lfts)
diagnose by US, venography Rx. Thrombolysis
17. (40, 123) Chronic Active hepatitis risk: old blood transfusion, high risk profession.
Usually asymptomatic, chronic progressive. Causes: Hep B (eAg present) and Hep C (antiHCAb's) also SLE, LKM, methyldopa, isoniazid, antytipsin, Wilsons, UC). AST, BR elevated
>>ALP. Piecemeal necrosis. Rx. Iv IFN, success 30%, often relapse. consider HIV screening.
18. (41) Cardiac catheter values 1. look for unusual pressures and saturations, 2. look for
gradients across pulmonary and aortic valves. mitral valve disease reflected in PCWP.
19. (44) Acute intermittent porphyria aut dom, Chr 11 PBG deaminase deficiency. 5F>M.
Increased dALA and PBG blood and urine--. gastro and neuro sequelae, (skin manifestations
rare cf PCT). Precipitants: drugs (barbiturates, anticonvulsants), alcohol, fasting, sepsis,
OCP. Presents as acute abdominal abdomen (can mimic surgical abdomen but AXR normal).
Neuropathy/ANS excitation/motor paralysis including CN's, epilepsy, hyponatraemia
(SIADH). Rx 1. withdraw precipitant, 2. high carbohydrate diet /iv dextrose (reduces haem
precursors in blood by reducing ALA sythetase activity), 3. haematin iv, 4. opiate analgesia.
Avoid metoclopramide (another precipitant), B blockers for tachy. Epilepsy: calories, fluid
restriction (inc Na), chlormethiazole or diazepam (most other anticonvulsants are
preciptants). Tests: erlichs dye --> red (also jaundice) PLUS chloroform --> purple sedimnt
(porhyria).clear (bilirubin). (Watson Scwarz test). Levels elevated in attack but may be
normal when well. Can assay red cell PBG deaminase (low), ALA dehydrogenase (high).
20. (51) Causes of erythema multiforme. Infection (mycoplasma, HSV, orf, TB), drugs
(penicillin, barbs, sulphonamides), other (conn tiss dx, vasculitis, internal malignancy)
21. (52) Hereditory angioedema (c1 esterase inhibitor deficiency) aut dom. Attacks
preceded by painful macular rash. precipitants: trauma. Laryngeal oedema, abdominal pain.
Normall rx supportive responds in 72 hours. Can give FFP if severe/protracted. Long-term
rx. Danazol (inhibits plasmin) Hydrocortisone/chlorpheniramine less effective cf.
anaphylaxis. Diagnosis C1 esterase inhibitor (low), c1 esterase (high), c2 & c4 both low.
22. (57) Aortic dissection: proximal --> urgent surgery. Seen clearly on 2D echo. Important
to exclude before thrombolysing any MI. assoc. hypertensive blacks, Marfans, Ehrler
Danlos, pregnancy, coarctation.
23. (58) Scurvy: elderly, normocytic anaemia, bruising (haematomas), prolonged bleeding
time --> always think of vitC deficiency. Easy bruising, bleeding gums, periosteal
haemmorhage, muscle haematomas. Classic: perifolicular haemorhage, corkscrew hairs.
APTT/PT normal, BT increased. Dx by plt or leucocyte ascorbate concentration. Rx. Vit C.
24. (71) livedo reticularis: assoc vasculitis and conn tiss disease e.g. PAN
25. (71) polyarteritis nodosa males 60-70 myalgia, night sweats, weight loss, livedo
reticularis, testicular pain, assoc HbsAg. Multisystem (neuro, gi, renal, cvs), aneuysm -->
thrombosis -->organ infarction. dx. ANCA suggestive, organ biopsy , microaneurysms on
angiography. Rx. Steroids.
26. (72) Heinz bodies: low Hb, high retic count -> bleed or haemolysis. Differentiate by
blood film --> Hz bodies (pptd Hb) seen in Hbopathies or metHbaemia (Fe3+) e.g.
Sulphasalasine Rx esp if G6PD deficiency. Cf Howell Jolly bodies, cf. Malaria blood films
(can be confused)
27. (74) Apical lung fubrosis: TB, EAA, ankylosing spondylitis, apergillosis, radiation,
sarcoidosis, histoicytosis X.
28. (76) Familial benign hypocalciuruc hypercalcaemia aut dom. Usually lifelong
asymptomatic hypercalcaemia occasional gall stones/pancreatitis/joint deposition. Difficult
to distinguish between 1ry PTHism so patients often have uneccesary parathyroidectomy
and FH parathyroidectmy. Can distgiuish because urinary calcium is LOW (it is high in all
other causes of hypercalcaemia). Conservative management.
29. (77) Eruptive xanthomas: think hypertriglyceridaemia (associated with pancreaitis).
There is pseudohyponatreamia due to lipids binding Na the free sodium only is normally
measured. Can differentiate by looking at serum osmolality.
30. (84) Wolf Parkinson White syndrome: accessory fast conducting atrioventricular
pathway (Bundle of Kent). Ventricles depolarise prematurely --> short PR, slurred (delta
wave) upstroke of QRS. 2 arrythmias possible: AV re-entrant tachycardia (P burried AFTER
QRS Rx. Vagal stim, adenosine) , AF (delta waves sometimes seen Rx adenosine, vagal stim
usually fails). 2nd line --> cardioversion. Definitive management radiofrequesncy ablation.
Prevention with class I/III
(sotalol, flecainide, amiodarone, disopyramide). Avoid digoxin and verapamil as they increase
accessory pathway conduction predisposing to malignant ventricular arrythmias.
31. (87) Polycythemia rubra vera Hb can be normal if coexisting Fe deficiency anaemia (low
MCV), look at PCV and rbc count to make the diagnosis. All marrow cells increased (wcc, plt)
and splenomegally.
Incidious onset assoc lethargy, depression, vertigo, tinnitus, amaurosis fugax. Plethora,
hypertension, spenomegally. Bledding (usually upper GI) due to throbocythaemia, stroke
(hyperviscocity), gout (increased cell tunover). Can progress to myelofibrosis or AML. Rx.
Venesection (can cause fe def anaemia). Busuphan and hydroxyurea to control
thrombocytosis.
32. (91) Hypokalaemic periodic paralysis aut dom, episodic paralysis classically while
patient asleep or with prolonged rest. Ppt by dextrose and insulin, alcohol, cho, anxiety,
tension. Rx can give K supplements or longeterm K sparing duiretics. Associated with
orientals with thyrotoxicosis. Hypokalaemic arrythmias. Rx of thyrotoxicosis prevents
paralysis too, propranolol also works. Only affects males. Need to exclude GBS or
myasthenia gravis.
33. (94) Schmidtz syndrome (polyendocrine deficiency type II) 10% of addisons pients
have other endocrine abnormailies too - autoimmune hypothyroidism and IDDM. Type I PED
= parathyroid, adrenal, candida, alopecia, pernicous anaemia. Vitiligo and hypogonadism occur
in both. Hypothyroidism can cuase a large heart.
34. (97) Leprospirosis (rat faeces) liver failure common, renal failure can also occur.
Brucellosis (unpasteurised cows milk) liver failure rare, assoc hepatosplenomegally, high
fever and leucopenia.
35. (98) Atrial enlargement on ECG. R atrium = tall P II, V1 >2.5 small squares tall. L atrium=
bifid P in II >3 small squares wide, V1 negative portion p wave >1 small square duration
AND amplitude.
36. (98) Sokolow's criteria for ventricular hypertrophy look at V1 ad V5/6: Right ventricle
>12.5mm. Left ventricle >40mm. LVH shows more toward V6, RVH in V1.
37. (101) HIV infections: oral hairy leucoplakia (H=EBC infection) - white plaques on the
lateral border of tongue Rx. Acyclovir cf candida rx. Nystatin, flucanazole.
Crypotosporidium diarrohea ZN stain of stool --> red cysts. Rx. Symptomtic +
spiramycin/paromomycin.
38. (103) SLE arthropathy, fever, polyserositis. 10F:1M, blacks, usully <40yrs. Multisystem
involvement: renal, resp, CVS, neuro, haematologic, cutaneous. DsDNA positive in 60%, ANA
in 90%. ESR usually high, CRP characteristically normal. C3 depressed. Lupus anticoagulant.
Rx. NSAIDs (joints), chloroquine (skin), corticsteroids, azathioprine, cyclophosphamide.
39. (103) coxsackie virus A/B - epidemic myalgia (Borholm's syndrome) usually
children/young adulys. Sudden abdo/thoracic pain. Myalgia, fever. Polyserositis. Virus in
faeces, retrospective diagnosis by serum antibody x4 rise.
40. (107) Guillain Barre syndrome onset often heralded by SEVERE lumbar or intrascapular
pain. Ans neuropathy e.g. Bladder atony. Csf albuminocytogenic dissociation. Rx. Iv Ig. Poor
prognstic markers: FVC<1litre, paO2 <8, paco2>6, LRTI, polm oedema, accelerated
hypertension, arrythmia, bulbar involvement, campylobacter infection.
41. (110) Homocytinuria cystathionine deficiency, aut recessive, chr 21---> homocysteine
accumulation--> oxidised to homocystine (elevated urine levels). Cystine NOT produced.
Defects in collagen crosslinking. Marfanoid features (tall, arachnodactyly, high arched
palate, lens dislocation), osteoperosis, epilepsy/mental hadicap, venous/arterial thrombosis
(OCP contraindicated), folate deficiency (inc MCV), premature coronary art disease. Rx.
Restrict methionine, add cystine. Give Pyridoxine as soon as diagnosis is made.
42. (112) hypotension, hyperkalaemia, metabolic acidosis --> Addisons, diuretics/salt loosing
nephritis.
43. (114) hypokalaemia --> gi or renal loss. RENAL if 1. HYPERTENSIVE with high renin
(malignant hypertension, RAS, diuretics), with low renin (conn's, cushing's, liddle's=tubular
defect mimics conn's biochemistry) 2. ACIDOTIC think RTA as most other causes give
alkalosis. 3. NORMOTENSIVE diuretics, carbenoxole, liquerish, aminoglycosides,
Barters=child JGA hyperplaia, weakness, nocturia, failure to thrive. liquorish and
carbenoxalone can also cause hypertension. THINK OF GASTRO CAUSES IF K IS AVIDLY
RETAINED FROM KIDNEY ie. <20 MMMOL/L URINARY K.
44. (115) Coeliac disease - whites esp. irish HLA A1,B8,DR7,DQW2, gluten sensitive
enteropathy. 20% do not have diarhhoea. anti gliadin antibodies. children:failure to thrive,
adults:abdo pain, fatgue, wt loss. Ankle oedema (albumin), mouth ulcers (Fe), osteomalacia
(vit D), hypocalcaemia. Anaemia may be Fe>>B12 but both occur. hyposplenism (Howell Jolly
bodies). endoscopic D2 or jejunal bx shows villous atrophy, anti gliadin/anti retiuculin
antibodies seen in most patients. antiendomyesial can be absent but are more specific.
Rx.gluten free diet. assoc with autoimmune thyroid dx, liver dx, fibrosing alveolitis, IDDM.
complications: lymphoma, adenocarcinoma, splenic atrophy.
45. (116) Cerebral manifestations of HIV - toxoplasmosis (IgM ,Sabin Feldman dye test,
CT...Rx pyrimethamine and sulphadiazine), TB meningitis (ZN stain...Rx Ansamycin and
clofazamine), crytococcal meningitis (India ink stain, CT..Rx amphotericin), lymphoma (dx
brain bx or csf cytology...rx radiotherapy). Uncertain dagnosis on CT --> Rx for
toxoplasmosis and repeat CT 2 weeks. biopsy if lesions persist. Other manifesations of HIV:
HIV dementa, PMLE, Herpes encephalitis, periph neuropathy).
46. (118) Charcot Marie Tooth disease - Hereditory Sensory Motor Nueropathy /Peroneal
Muscular Atrophy. HSMN1: familial diffuse demyelinating neuropathy, presents <20yrs.
wasted lower limbs --> inverted champagne bottle legs, foot deformity,scoliosis, sensory
ataxia. dx: FH, nerve conduction, chr 1q. HSMN2 milder. HSMN3 ( Dejerine Sottas dx) aut
rec. mixed neuropathy. presents <10yrs with palpable peripheral nerves (palpble nerves also
seen in acromegally, leprosy, nft, hiv)
47. (120) Methaemoglobinaemia (i.e. Haem Fe2+-->Fe3+) assoc with G6PD (low NADPH so Hb
more readily oxidatised). MetHb high afinity O2--> ox diss curve left shift--> dec tissue
oxygenation. variable degree of cyanosis. metHb level 20% (dyspnoea, headache, angina)
60%+ collapse, sudden death. Blood film intravascular haemolysis, Heinz bodies. Causes:
G6PD, drugs (sulphonamide, primaquin, phenacetin), toxins (ferric cyanide, chlorate, aniline
dyes)
48. (120) left shift of O2 dissociation curve: metHb, sulphHb, COHb, FetalHb, decreased
23BPG, alkalosis, hypocapnia, hypothermia.
49 (121) pain relief and renal failure --> think aute interstitial nephritis most likely.
50. (123) Hepatitis B serology. Onset of infection-->HBcAb IgM, Marker of previous
infection-->HBcAb IgG. HbeAb--> reduced infectivity (cf HBeAg--> increased risk). HBsAb-> immunity, last one to appear. CAH -> usually HBeAg amd HBsAg detected and history is for
several months.
51. (124) Rhematoid arthritis - episcleritis (painless), scleritis (painfull, bilateral, if
recurrent can cause perforation). anaemia, Felty's syndrome, renal, resp. Drugsfor rh pancytopenia (penicillamine or Felty's), nephrotic syndrome (pnicillamine membranous GN,
renal amyliod, NSAIDS/analgesics), anaemia (as above plus NSAIDS, chr dx, folate def,
atoimmune haemolysis, pernicious). eye dx (scleritis, sjorgens, steroid cateracts, chloroquine
retinopathy, eye movement - muscle synovitis, peniciillin inducedmyasthenia, mononeuritis).
Resp problems: effusions, fibrosis, bronchiolitis obliterans, pneumonitis, pulm nodules,
caplans (nodules plus PMD in coal workers, cricoarytinitis)
52. (125) vit D resistant rickets- no po4 resorption, high urinry po4 plus/minus paraplegia
53. (128) Kawasakais dx Hx, O/E, echo Rx. 1. aspirin PO, 2. iv Ig.
54. (129) atypical lymphocytes - HIV seroonversion (2-6weeks after exposure), CMV, EBV,
toxoplasma, secondary syphilis.
55. (129) syphilis serology: VDRL negative--> test FTA for previous infection. HIV IgG
(detctable at 6 weeks), p24 (72 hrs) or PCR for HIV RNA.
56. (130) renal tubular acidosis: serum K USUALLY FOLLOWS serum H but RTA is an
exception. hypokalaemic acidosis with hypocalcaemia and hypercholraemia. manifests
clinically as osteomalacia and muscle weakness. dx: abnormal acid load test, early morning
uirne pH>5.5. distal=RTA 1 (defective H excretion. failure to thrive, renal colic).
proximal=RTA 2 (absent H excretion, NaHCO3 loss. polyuria, polydypsia). Rx. 1. Na
bicarbonate (correct acidosis), 2. Potassium citrate (provides K and prevents Ca excretion).
Can be inherited, drug induced(acetazolaminde, tetracycline, Pb, Argon, vit D), assoc with
PBC, CAH, myeloma, hyperPTH, Fanconi, obstructive uropathy.
57. (131) Pulmonary embolisn - sudden onset, R heart failure, normal CXR, restictive lung
defect, low KCO, low pao2 low paco2. pulm bp.
58. (133) metabolic acidosis and resp alkalosis always think salicylate poisoning! Rx 1. fluids
if <500. 2. alkaine duiresis if <750. 3. haemodialysis if >750.
59. (135) paradoxical emobous with a soft (?stolic) murmur ---> ASD. ECG changes: RBBB,
RVH. LAxD-primum RAxD-secundum. for VSD to be soft takes v large defect.
71. (153) high wcc, myeoblasts, splenomegally --> diff diag CML, myelofibrosis. CML --> low
neutophil alk phosphatase, 90% philidelphia chr positive. blood film shows granulocytes if
varying maturity.
72. (156) Parkinsons plus syndromes: 1. multisystem atrophy=sporadic adult onset
parkinsonism, cerebellar signs, ANS failure. Parkinsone + ANS failure (e.g. impotence, resp
stridor, dysarthria) is called Shy Drager. shortened life expectancy (10 years). postural
hypotension --> compression stockings, fludrocortisone, ephidrine, poor respons to Ldopa. 2.
Steele Richardson syndrome = NFT's, 70's progressive supranuclear palsy (absence of
voluntary downgaze gaze), perseverence in speech(palililia, palilogia), falling backwards,
involuntary eye closure, frontalis hyperactivity (7 year survival). both are completely
different entities to PD.
73. (159) Hypogonadism - some causes: 1. PCOS - oestradiol slightly reduced, FSH low, 2.
coeliac dx - short stature, delayed puberty, 3. testicular feminization syndrme - no
receptors on end organs. phenotypic females, intrabdomial testicles, testosterone levels
very high. LH and FSH also very high (no testosterone Receptors on pituitry) --> high
oestrogens --> breasts. scanty or absent pubic hair. 4. Pituitry Cushings (basophil adenoma).
Usuallly cortisol supressed by high dose dexamethasone otherwise petrosal sinus sampling
for ACTH.
74. (162) Sarcoidosis: multisystem granulomas, f > m. assoc blacks, Lofgrens syndrome
(erythemna nodsum, arthralgia, hilar lymphadenopthy, ant uveitis). lacrymal or parotid gland
enlargement
(Mikulicz syndrome seen in sarcoid, TB, lymphoma/leukaemia. with uveitis, facial nerve palsy
known as Heerfort's syndrome). most patients resolve <2 years, a few chronic lung disease
--> apical fibrosis. full spectrum of eye , skin papules (nose=lupus pernio), arthralgia
xray=luscent phalenges, aseptic meingitis, psychosis, mononeuritismultiplex, hypercalcaemia
(1, 25oh vitD from granlomatous cells).
75. (162) erythema nodosum: common: strep, sarcoid, ocp, UC/Crohns, sulphonamides, rest:
behcets, TB, leprosy, histpolasma, yersinia, neiserria meningitis/gonnorrohea, pasteurella.
76. (162) diabetes insipidus: abnormal water depriviation test ..urine osmolality < 720.
cranial: (respond to desmopressin) familial, aut dom, DIDMOAD, cerebral tumor,
sarcoidosis, TB, hitiocytosis X, trauma, piruitry haemmohrage. nephrogenic (unresponsive to
desmopressin)x linked vasopressin R defect, aut rec aquaporin defect, hypokalaemia,
hypocalcaemia, Lithium, postobstructive uropathy, sicke cell, glibenclamide, aminoglycosides,
amphoteracin.
Pictures to know:
1.Heinz bodies (denatured Hb seen in haemolysis)
2.Livedo reticularis
3.Lymphoerythroblastic bone marrow
4.retinitis pigmentosa
5.eruptive xanthomas
6. dermatitis herpetiformis (rx dapsone --> metHb)
7. atypical lymphocyte
Approach
A&E dept, think overdose
CRF with normal Hb (APKD). due to epo ectopic secretion.
chornic GI and RESP infection in a child --> always think CF
smoker think malignancy
Calcium metabolism
1. raised ALP (bone): 1. function hyperparathyroidism 2. damaged cortex 3. low vit D
2. hypercalcaemia: 1. 1ry/3ry PTH, 2. malignacy, 3. myeloma 4. vit D intox, 5.some sarcoid
3. low calcium: rickets/osteomalacia, hypoparathyroidism, 2ry hyperpth
4. po4 metabolism: po4 follows calcium in vit D pathology, in parathyroid dysfunction it does
the opposite. in other pathologies usually normal.
Disease.....................
Ca.............
PO4..................ALP
1ry/3ry pth................
high...........
low.................. high
malignancy................
high...........
norm/high........
high
high............
norm............
vit D intox................ .
high............
high..............
normal
sarcoid.................... .
high/norm.....
norm.............
norm
osteoperosis..............
norm...........
norm..............
norm
norm...........
norm..............
high
low/norm.....
high..............
high
rickets/osteomalacia...... .
low...........
low...............
high
hypoPTH/psudohypoPTH..
low...........
high..............
norm
myeloma no fracture.