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# ENJOY STUDY :

## Odds ratio(OR) - Use in case-control study.

Read it as ( case OR control )

## Phases of clinical trial : Safe Work -Better Stay

Phase 1- is it safe?
phase 2 - Does it works?
Phase 3 - Is it as good or better?
Phase 4 - Can it stay?

## Making of 2 x 2 table : DISEASE is always ABove.

So, on the top there will be always disease,
and , a and b ( from a,b,c,d ).
a for all ( disease + & test + ) , d for deny ( disease - and test - 0 )

## seNsitivity = 1 - false Negative rate. ( N is included in both)

sPecificity = 1 - false Positive rate. ( P is included in both )

## Observer expectancy bias = Pygmelion effect ( Observer expecting PIG-me-LION )

Confounding bias = Related to both exposure and outcome, but not on the causal pathway.
Strategy to reduce Confounding bias = MMC is RaRe
( M = Multiple studies , M = matching , C= Crossover studies ,
Ra = RAndomization, Re = REstriction )

## Mean,median, mode : This serial always will be constant.

Positive skew = Mean > Median > Mode ( Direction of arrow is to the direction of the tail of the
positive skew ) - see graph in First aid

Negative skew = Mean < Median < Mode ( Direction of arrow is to the direction of the tail of the
negative skew )

## Increase power and decrease Beta by = E S R

( E = Expected effect size , S = Sample size , R = Reliability, also called precision)

## 1) Administer hepatitis B vaccine if mother is HBsAg negative

2) If mother is HBsAg positive , administer hepatitis IVIG along with the hepatitis B vaccine

## 4) Neonatal screening tests.

- Phenylketonuria
- Galactosemia
- Hypothyroidism

## Sturge - Weber syndrome :

evaluate for glaucoma and give anticonvulsives.

Rx of Hemangioma :
steroids or pulsed laser if large or interferes with organ function.
Preauricular tags / pits : DO the followings-
Hearing test
Ultrasound of kidneys

Aniridia :
Screen for Wilms tumor with abdominal USG every 3 months until age 8

## Rx of Thyroglossal duct cyst:

Surgical removal
Thyroid scans and thyroid function test preoperatively

Rx of Gastroschisis
Surgical correction

Umbilical hernia :
Screen with TSH
May close spontaneously

Undescended testes :
Associated with malignancy if > 1 year of age
No treatment until 1 year of age
(1) Hormone injections ( -hCG or testosterone )
(2) Surgery ( orchiopexy )

Very important concepts of usmle step 1 and step 2 ck ( Neurology ) - source ; FA(step 1)

Effects of strokes

Anterior circulation :

## 1) Middle cerebral artery

- Motor and sensory corticesupper limb and face.
- Aphasia if in dominant (usually left) hemisphere.
- Hemineglect if nondominant (usually right) side.

## 2) Anterior cerebral artery

- Contralateral paralysis and sensory losslower limb.
3) Lenticulo- striate artery
- Contralateral paralysis and/or sensory lossface and body.
- Absence of cortical signs (eg, neglect, aphasia, visual field loss).

Posterior circulation :

## 1) Anterior spinal artery (ASA)

( Medial medullary syndrome )
infarct of paramedian branches of ASA and / or vertebral arteries.
a. Contralateral paralysis upper and lower limbs.
b. Decrease contralateral proprioception.
c. Ipsilateral hypoglossal dysfunction (tongue deviates ipsilaterally).

## 2) Posterior inferior cerebellar artery (PICA) -

Lateral medullary (Wallenberg) syndrome.
Dont pick a (PICA) horse (hoarseness) that cant eat (dysphagia).
Specific C/F
- dysphagia,
- hoarseness,
- decrease gag reflex;
- ipsilateral Horner syndrome;
Nucleus ambiguus effects are specific to PICA lesions.

## 3) Anterior inferior cerebellar artery (AICA):

( Lateral pontine syndrome ).
Facial droop means AICAs pooped.
Facial nucleus effects are specific to AICA lesions.
Specific C/F:
- Paralysis of face,
- decrease lacrimation, salivation,
- decrease taste from anterior 23 of tongue.

## 4) Basilar artery - Locked-in syndrome.

- Preserved consciousness,
- vertical eye movement,
- loss of voluntary facial, mouth, and tongue movements.

## 5) Posterior cerebral artery

- Contralateral hemianopia with macular sparing.
High yield concepts of gynaecology ( for step 2 ck ) , source-MTB 3
( 2 - 3 Q from these concepts will be definitely in the exam )

## Gestational Trophoblastic Disease -

Management
Baseline quantitative -hCG titer
Chest x-ray (rule out lung metastasis)
Suction D&C
effective contraception (oral contraceptive pills)

## Infertility - ( Steps in workup )

1) Semen analysis
. If abnormal , repeat the analysis in 6 - 8 weeks.

## . Abnormal semen analysis : (fertility options)

a) Intrauterine insemination,
b) intracytoplasmic sperm injection and
c) in vitro fertilization (IVF)

. No viable sperm:
Artificial insemination by donor .

2) Anovulation
. Hypothyroidism and hyperprolactinemia are causes of anovulation that can be treated.

. Ovulation induction:
Clomiphene citrate is the agent of choice.
Human menopausal gonadotrophin (hMG) is used if
clomiphene fails

3) Tube Abnormalities:
. Hysterosalpingogram (HSG):
No further testing if the HSG shows normal anatomy.

. Laparoscopy :
If the HSG is abnormal to visualize oviducts and attempt reconstruction (tuboplasty).
If tubal damage is severe, IVF should be planned.

Endometriosis :

Treatment
a First-line therapy:
Continuous oral progesterone or OCP .

b Second-line therapy:
Testosterone derivatives (Danocrine or danazol) or GnRH analogs (Lupron or leuprolide)

Laser vaporization of lesions can improve fertility.

## d TAH and BSO :

for severe symptoms when fertility is not desired.

Tuboovarian Abscess

Treatment :

## 2. If no response within 72 hours or if there is abscess rupture, perform an exploratory laparotomy

TAH and BSO or percutaneous drainage.

Acute Salpingo-oophoritis

Treatment

## Outpatient : One dose of IM ceftriaxone plus doxycycline

Inpatient : IV cefotetan or cefoxitin plus doxycycline

Treatment:

## when any of the following conditions is present:

- Metastasis to lymph nodes
- Tumor size > 4 cm
- Poorly differentiated lesions
- Positive margins
- Local recurrence

## Management of Abnormal Histology During Pregnancy

1) CIN/dysplasia:

## Pap smear and colposcopy every 3 months during pregnancy

- Repeat Pap and colposcopy 6-8 weeks postpartum .
Any persistent lesion are then definitively treated postpartum

## Cone biopsy (to ensure no frank invasion)

Deliver vaginally, reevaluate and treat 2 months postpartum

3) Invasive cancer

## b) Diagnosed after 24 weeks:

Conservative management up to 3233 weeks
Cesarean delivery and begin definite treatment

## Ovarian pathology ( for step 1 ) source - FA

( atleast 1-2 Q will be in exam)

Ovarian cysts:

Follicular cyst -
Associated with hyperestrogenism, endometrial hyperplasia.

Theca-lutein cyst -
Associated with choriocarcinoma and hydatidiform moles.

Ovarian neoplasms

## Risk increase with -

. infertility,
. endometriosis,
. PCOS,
. genetic predisposition (BRCA-1 or BRCA-2 mutation, Lynch syndrome, strong family history).
Risk decreses with -
. previous pregnancy,
. history of breastfeeding,
. OCPs,
. tubal ligation.

## Benign ovarian neoplasms

. Lined with fallopian tubelike epithelium.

. Lined by mucus-secreting epithelium .

3) Endometrioma

## Endometriosis (ectopic endometrial tissue) within ovary with cyst formation.

Chocolate cyst endometrioma filled with dark, reddish-brown blood.

## Germ cell tumor.

A monodermal form with thyroid tissue(struma ovarii) uncommonly presents with hyperthyroidism .

5) Brenner tumor

## Looks like Bladder ( both contains B )

Coffee bean nuclei on H&E stain.

6) Fibromas

## Bundles of spindle-shaped fibroblasts.

Meigs syndrometriad of ovarian fibroma, ascites, hydrothorax.

7) Thecoma

## Like granulosa cell tumors, may produce estrogen.

Usually presents as abnormal uterine bleeding in a postmenopausal woman.

## 1) Granulosa cell tumor :

- Most common malignant stromal tumor.
- Often produces estrogen and/or progesterone and presents with postmenopausal bleeding,
- breast tenderness.

- Histology shows Call-Exner bodies (granulosa cells arranged haphazardly around collections of
eosinophilic fluid, resembling primordial follicles).

Most common malignant ovarian neoplasm,
Psammoma bodies.

Pseudomyxoma peritoneiintraperitoneal accumulation of mucinous material from ovarian or
appendiceal tumor.

4) Immature teratoma
Aggressive, contains fetal tissue, neuroectoderm.

5) Dysgerminoma

## Equivalent to male seminoma .

Sheets of uniform fried egg cells .
hCG, LDH = tumor markers.

## Aggressive, in ovaries or testes (boys) and sacrococcygeal area in young children.

Schiller-Duval bodies (resemble glomeruli) .
AFP = tumor marker.

7) Krukenberg tumor

Source - FA

## (2 - 3 Q. will be from these concepts)

Immunodeficiencies :
B-cell disorders -

## Defect in BTK > no B-cell maturation.

Recurrent bacterial and enteroviral infections after 6 months (decrease maternal IgG).
Absent B cells in peripheral blood, decrease Ig of all classes.
Absent/scanty lymph nodes and tonsils.

## 2) Selective IgA deficiency

Can see Airway and GI infections, Autoimmune disease, Atopy, Anaphylaxis to IgA-containing
products.

## Defect in B-cell differentiation.

Increase risk of autoimmune disease, bronchiectasis, lymphoma, sinopulmonary infections.
Decrease plasma cells,
Decrease immunoglobulins.

T-cell disorders

## 22q11 deletion detected by FISH.

Recurrent viral / fungal infections (T-cell deficiency)
Conotruncal abnormalities (eg, tetralogy of Fallot, truncus arteriosus).
Decrease T cells, PTH, and Ca2+.

## Decrease Th1 response.

Disseminated mycobacterial and fungal infections;
May present after administration of BCG vaccine.
Decrease IFN-.

## 3) Hyper-IgE syndrome (Job syndrome)

Deficiency of Th17 cells due to STAT3 mutation > impaired recruitment of neutrophils to sites of
infection.
Increase IgE, decrease IFN-.
FATED : coarse Facies, cold (noninflamed) staphylococcal Abscesses, retained primary Teeth,
increase IgE, eczema.

## Absent in vitro T-cell proliferation in response to Candida antigens.

Absent cutaneous reaction to Candida antigens.

## 1) Severe combined immunodeficiency

(autosomal recessive).

## T-cell receptor excision circles (TRECs).

Absence of thymic shadow (CXR), germinal centers (lymph node biopsy), and T cells (flow
cytometry).
Treatment: bone marrow transplant (no concern for rejection).

2) Ataxia-telangiectasia

Defects in ATM gene > failure to repair DNA double strand breaks > cell cycle arrest.
Increase AFP.
Decrease IgA, IgG, and IgE.
Lymphopenia, cerebellar atrophy.

3) Hyper-IgM syndrome

## Defective CD40L on Th cells > class switching defect;

Severe pyogenic infections early in life;
opportunistic infection with Pneumocystis, Cryptosporidium, CMV.

4) Wiskott-Aldrich syndrome

## Mutation in WAS gene;

T cells unable to reorganize actin cytoskeleton.

## WATER: Wiskott-Aldrich: Thrombocytopenia, Eczema, Recurrent infections.

Risk of autoimmune disease and malignancy.
Decrease to normal IgG, IgM.
Increase IgE, IgA.
Fewer and smaller platelets.
Phagocyte dysfunction

## Defect in LFA-1 integrin (CD18) protein on phagocytes;

Impaired migration and chemotaxis;
Recurrent bacterial skin and mucosal infections, absent pus formation, impaired wound healing,
delayed separation of umbilical cord (> 30 days).
I
ncrease neutrophils.
Absence of neutrophils at infection sites.

2) Chediak-Higashi syndrome

## Defect in lysosomal trafficking regulator gene (LYST).

Microtubule dysfunction in phagosome-lysosome fusion;

## Recurrent pyogenic infections by staphylococci and streptococci, partial albinism,

peripheral neuropathy,
progressive neurodegeneration,
infiltrative lymphohistiocytosis.
Giant granules in granulocytes and platelets.
Pancytopenia.

## 3) Chronic granulomatous disease

Defect of NADPH oxidase > decrease reactive oxygen species (eg, superoxide) and
decrease respiratory burst in neutrophils;
Increase susceptibility to Catalase organisms .

## Abnormal dihydrorhodamine (flow cytometry) test (decrease green fluorescence).

Nitroblue tetrazolium dye reduction test obsolete.

## Screening Guidelines of Cancer and other diseases( For step 2 ck )

( source - MTB 3 )

( Atleast 2 - 3 Q in exam )

Breast cancer
. Mammography at age 50, then every 12 years, upto age 75.
Cervical cancer

## . Pap smear at age 21, then every 3 years

. stop at age 65 unless no previous screening

Colon cancer

## . Colonoscopy at age 50, then every 10 years

. If a close family member has had the disease
- screening should begin at 40 or 10 years earlier than the family member was diagnosed, whichever
is earlier.

## . Hereditary nonpolyposis colon cancer syndrome (HNPCC), or Lynch syndrome

- colon cancer in 3 family members in 2 generations having the disease, with 1 having it prematurely
(defined as occurring before the age of 50).
- screening should begin at age 25 and be done every 12 years.

Prostate Cancer

. No recommendation to screen patients routinely for prostate cancer with either a PSA or a digital
rectal examination.
( But if the patient want to do the these tests , you have to give )

. Recommend against the PSA for men > 75 ( Even if the patient wants )

Osteoporosis
. All women should be screened with bone densitometry at age 65.

## Abdominal Aortic Aneurysm

. All men about age 65 who were ever smokers should be screened once with an ultrasound.

Diabetes
. Diabetes screening is routine only in those with hypertension

Hypertension

. All patients above age 18 should have their blood pressure checked at every office visit.

Hyperlipidemia
. Men > 35 and women > 45 should be screened for hyperlipidemia.

## High-Yield Concepts of pediatrics ( For step 2 ck ) source - MTB 3

( 1-2 Q will be in the Exam)
Congenital Heart Defects

..................Acyanotic lesions

## 1) Ventricular septal defect

loud pulmonic S2
Surgical repair if - failure to thrive, pulmonary hypertension, or right-to-left shunt > 2:1

## Loud S1, wide fixed splitting of S2,

systolic ejection murmur along left upper sternal border.
Majority are of secundum types, and close by age 4.
Primary and sinus types require surgery.
Most common type: patent foramen ovale
Late complications: Mitral valve prolapse, dysrhythmias, and pulmonary hypertension

3) Atrioventricular canal

## Presentation similar to ventricular septal defect

Perform surgery in infancy before pulmonary hypertension develops.

4) Pulmonary stenosis

## Give prostaglandin E1 infusion at birth.

Attempt balloon valvuloplasty.

## More common in maternal rubella infection , and premature infants

Wide pulse pressure, bounding arterial pulses
Indomethacin-induced closure helpful in premature infants.
Term infants often require surgical closure.

6) Aortic stenosis
Valve replacement and anticoagulation may be required.

## 7) Coarctation of the aorta

Ductus dependant: Give PGE1 infusion to maintain ductus patent (ensures lower extremity blood
flow)
Surgery repair after stabilization
..................Cyanotic lesions

1) Tetralogy of Fallot

## Intermittent hyperpnea, irritability, cyanosis with decreased intensity of murmur

Treatment: Give oxygen, beta blocker, PGE1 infusion for cyanosis present at birth Surgical repair at
412 months

## Most common cyanotic lesion presenting in the immediate newborn period.

More common in infant of diabetic mother.
S2 usually single and loud, murmurs usually absent
Ductus-dependent : Give PGE1 to keep ductus open.
Definitive surgical switch of aorta and pulmonary artery needed as soon as possible.

## Inflammatory bowel diseases ( For step 1 and step 2 ck ) source- FA

----------------------Crohn disease

## . Skip lesions, rectal sparing.

. Transmural inflammation> fistulas.
. Cobblestone mucosa, creeping fat, bowel wall thickening (string sign on barium swallow x-ray ),

## . Linear ulcers, fissures.

. Noncaseating granulomas and lymphoid aggregates.
. Colorectal cancer ( Increase risk with pancolitis).

## . Fistulas (eg, enterovesical fistulae ) , phlegmon/abscess,

. strictures (causing obstruction), perianal disease.
. Diarrhea that may or may not be bloody.
. Kidney stones (usually calcium oxalate), gallstones.

## . Rx - Corticosteroids, azathioprine, antibiotics (eg, ciprofloxacin, metronidazole), infliximab,

---------------------------Ulcerative colitis

## . Always with rectal involvement.

. Mucosal and submucosal inflammation only.
. Friable mucosal pseudopolyps with freely hanging mesentery.
. Loss of haustra > lead pipe appearance on imaging.
. Crypt abscess and ulcers , bleeding , no granulomas.
. Colorectal cancer (increase risk with pancolitis).

## . Fulminant colitis, toxic megacolon, perforation.

. Bloody diarrhea.
. 1 sclerosing cholangitis.
. Associated with p-ANCA.

## . Rx - 5-aminosalicylic preparations (eg, mesalamine), 6-mercaptopurine, infliximab, colectomy.

-----common in both

## . Rash (pyoderma gangrenosum, erythema nodosum),

. Eye inflammation (episcleritis, uveitis), .
. Oral ulcerations (aphthous stomatitis),
. Arthritis (peripheral, spondylitis).

## High yield concepts of hematology ( For step 1 and step 2 ck )

Source- FA
( Atleast 1- 2 Q in exam )
.....................Hodgkin vs nonHodgkin lymphoma
------Hodgkin
. Localized, single group of nodes;
. contiguous spread (stage is strongest predictor of prognosis).
. Reed-Sternberg cells.
. Associated with EBV.
------Non-Hodgkin
. Multiple lymph nodes involved;
. extranodal involvement common;
. Majority involve B cells; a few are of T-cell lineage.
. May be associated with HIV and autoimmune diseases.
++++ ReedSternberg cells
. Giant cell seen in Hodgkin lymphoma;
. binucleate or bilobed with the 2 halves as mirror images (owl eyes ).
. RS cells are CD15+ and CD30+ B-cell origin.
. Necessary but not sufficient for a diagnosis of Hodgkin lymphoma.
>>>>>>>>> NonHodgkin lymphoma <<<<<<<<<
...................Neoplasms of mature B cells
1) Burkitt lymphoma
. t(8;14)translocation of c-myc (8) and heavy-chain Ig (14)
. Starry sky appearance,
. sheets of lymphocytes with interspersed tingible body
macrophages .
. Associated with EBV.
2) Diffuse large B-cell lymphoma
. Most common type of non-Hodgkin lymphoma in adults.
3) Follicular lymphoma
. t(14;18)translocation of heavy-chain Ig (14) and BCL-2 (18)
. Bcl-2 inhibits apoptosis.
. Presents with painless waxing and waning lymphadenopathy.
4) Mantle cell lymphoma
. t(11;14)translocation of cyclin D1 (11) and heavy-chain Ig (14)
5) Primary CNS lymphoma (PCNSL)
. Most commonly associated with HIV/ AIDS
. Considered an AIDS-defining illness.
. Mass lesion(s) on MRI, needs to be distinguished from toxoplasmosis via CSF analysis or other lab
tests.
.......................Neoplasms of mature T cells
. Caused by HTLV (associated with IV drug abuse)
. Adults present with cutaneous lesions;
. Lytic bone lesions, hypercalcemia.
2) Mycosis fungoides/ Sezary syndrome
. skin patches / plaques (cutaneous T-cell lymphoma),
. atypical CD4+ cells with cerebriform nuclei.
. May progress to Sezary syndrome (T-cell leukemia).

## High yield concepts of Dermatology ( For step 1 and step 2 ck ) (

( source - FA )

( 1 - 2 Q in the Exam )

...........Acne

## . Pilosebaceous follicles with increase sebum, keratin,

Propionibacterium acnes .

## . Obstruction (comedones) and inflammation (papules/pustules , nodules, cysts).

. Rx - retinoids, benzoyl peroxide, antibiotics.

## . commonly on skin flexures.

. Increase serum IgE.
. Usually appears on face in infancy and then antecubital fossae .

## ........Allergic contact dermatitis

. Type IV hypersensitivity.
. Lesions occur at site of contact(eg, nickel , poison ivy, neomycin ).

.........Melanocytic nevus

. Common mole.
. melanoma can arise in congenital or atypical moles.
. Intradermal nevi are papular .
. Junctional nevi are flat macules .

.........Psoriasis

## . Papules and plaques with silvery scaling

. Acanthosis with parakeratotic scaling (nuclei still in stratum corneum),
. Munro microabscesses.
. Increase stratum spinosum,

## . Decrease stratum granulosum.

. Auspitz sign pinpoint bleeding spots from exposure of dermal papillae when scales are scraped
off.
. Associated with nail pitting and psoriatic arthritis.

..............Rosacea

## . Erythematous papules and pustules , but no comedones.

. May be associated with facial flushing in response to external stimuli (eg, alcohol, heat).

## . Phymatous rosacea can cause rhinophyma (bulbous deformation of nose).

...........Seborrheic keratosis

. Flat, greasy, pigmented squamous epithelial proliferation with keratin-filled cysts (horn cysts) .
. Looks stuck on.

## . Leser-Trelat sign sudden appearance of multiple seborrheic keratoses, indicating an underlying

malignancy (eg, GI, lymphoid).

.............Verrucae

## . Warts; caused by HPV.

. Tan-colored, cauliflower-like papules.
. Epidermal hyperplasia, hyperkeratosis, koilocytosis.
. Condyloma acuminatum on genitals .

.............Urticaria

## xxxxxxxxxx Vascular tumors of skin xxxxxxxxxx

............Angiosarcoma

## . Elderly, on sun-exposed areas.

. Associated with radiation therapy and chronic postmastectomy lymphedema.

## . Hepatic angiosarcoma associated with vinyl chloride and arsenic exposures.

...........Bacillary angiomatosis

## .Benign capillary skin papules found in AIDS patients.

. Caused by Bartonella henselae infections.
. Frequently mistaken for Kaposi sarcoma, but has neutrophilic infiltrate.

...........Cherry hemangioma

## . Benign capillary hemangioma of the elderly .

. Does not regress.

...........Cystic hygroma

## Cavernous lymphangioma of the neck .

Associated with Turner syndrome.
............Glomus tumor

## . Benign, painful, red-blue tumor, commonly under fingernails.

. Arises from modified smooth muscle cells of the thermoregulatory glomus body.

............Kaposi sarcoma

. Endothelial malignancy most commonly of the skin, but also mouth, GI tract, and respiratory tract.
. Associated with HHV-8 and HIV.

## . Frequently mistaken for bacillary angiomatosis, but has lymphocytic infiltrate.

............Pyogenic granuloma

## . Polypoid lobulated capillary hemangioma that can ulcerate and bleed.

. Associated with trauma and pregnancy.

............Strawberry hemangioma

## . Benign capillary hemangioma of infancy .

. Appears in first few weeks of life ;
. Grows rapidly and regresses spontaneously by 58 years old.

Very high yield topics of Nephrology ( For step 1 and step 2 ck ) Source- FA

## When involves glomeruli > hematuria and RBC casts in urine.

Associated with azotemia, oliguria, hypertension (due to salt retention), proteinuria.

## . LMglomeruli enlarged and hypercellular .

. IF(starry sky) granular appearance (lumpy-bumpy) due to IgG, IgM, and C3 deposition along
GBM and mesangium.

## . EMsubepithelial immune complex humps.

. Occurs 24 weeks after group A streptococcal infection of pharynx or skin.

## . Type III hypersensitivity .

. Positive strep titers/serologies,
. decrease complement levels due to consumption.
...............Rapidly progressive (crescentic) glomerulonephritis

## . LM and IFcrescent moon shape .

. Crescents consist of fibrin and plasma proteins (eg, C3b) with glomerular parietal cells, monocytes,
macrophages.

## . antibodies to GBM and alveolar basement membrane > linear IF

. Hematuria/hemoptysis.
. Treatment: emergent plasmapheresis.

## --- b) Granulomatosis with polyangiitis (Wegener)

. PR3-ANCA/c-ANCA.
. Pauci-immune (no Ig/C3 deposition).

## --- c) Microscopic polyangiitis

. MPO-ANCA/p-ANCA.
. Pauci-immune (no Ig/C3 deposition).

## . Due to SLE or membranoproliferative glomerulonephritis.

. LMwire looping of capillaries.

. IFgranular.

## . A common cause of death in SLE (think wire lupus).

. DPGN and MPGN often present as nephrotic syndrome and nephritic syndrome concurrently.

## ...............IgA nephropathy (Berger disease)

. LMmesangial proliferation.
. EMmesangial IC deposits.
. IFIgA-based IC deposits in mesangium.

## . Renal pathology of Henoch-Schonlein purpura.

. Episodic gross hematuria that occurs concurrently with respiratory or GI tract infections (IgA is
secreted by mucosal linings).
.............Alport syndrome
. Mutation in type IV collagen > thinning and splitting of glomerular basement membrane.
. Eye problems (eg, retinopathy, lens dislocation),
. glomerulonephritis, sensorineural deafness;
. Basket-weave appearance on EM. .

## . Type Isubendothelial immune complex (IC) deposits with granular IF;

. tram-track appearance on PAS stain and H&E stain due to GBM splitting caused by mesangial
ingrowth.

## . Type IIalso called dense deposit disease.

. MPGN is a nephritic syndrome that often copresents with nephrotic syndrome.

## . Type 1 may be 2 to hepatitis B or C infection.

. Type II is associated with C3 nephritic factor (stabilizes C3 convertase > decrease serum C3
levels).

## . massive proteinuria (> 3.5 g/day)

. Frothy urine with fatty casts.
. Due to podocyte damage disrupting glomerular filtration charge barrier.

## . May be 1 (eg, direct sclerosis of podocytes) or

. 2 (systemic process [eg, diabetes] secondarily damages podocytes).

## . hypercoagulable state (eg, thromboembolism) due to -

antithrombin III loss in urine
. increase risk of infection (due to loss of immunoglobulins in urine and soft tissue compromise by
edema).

. Severe nephritic syndrome may present with nephrotic syndrome features (nephritic-nephrotic
syndrome) -- if damage to GBM is severe enough to damage charge barrier.

## ...................Minimal change disease (lipoid nephrosis)

. LMnormal glomeruli (lipid may be seen in PCT cells).
. F .
. EMeffacement of foot processes .

. Often 1 (idiopathic) and may be triggered by recent infection, immunization, immune stimulus.
. may be 2 to lymphoma (eg, cytokine-mediated damage).
. 1 disease has excellent response to corticosteroids.

## . LMsegmental sclerosis and hyalinosis .

. IFoften , but may be for nonspecific focal deposits of IgM, C3, C1..
. EMeffacement of foot process similar to minimal change disease.

. Can be 1 (idiopathic) or 2 to other conditions (eg, HIV infection, sickle cell disease, heroin abuse,
massive obesity, interferon treatment, chronic kidney disease due to congenital malformations).

## . LMdiffuse capillary and GBM thickening .

. IFgranular as a result of immune complex deposition.
. Nephrotic presentation of SLE.

## . EMspike and dome appearance with subepithelial deposits.

. Can be 1 (eg, antibodies to phospholipase A2 receptor) or
2 to drugs (eg, NSAIDs, penicillamine), infections (eg, HBV, HCV), SLE, or solid tumors.

.................Amyloidosis

. LMCongo red stain shows apple-green birefringence under polarized light due to amyloid
deposition in the mesangium.
. Kidney is the most commonly involved organ (systemic amyloidosis).

. Associated with chronic conditions that predispose to amyloid deposition (eg, AL amyloid, AA
amyloid).

## . LMmesangial expansion, GBM thickening,

. eosinophilic nodular glomerulosclerosis (Kimmelstiel-Wilson lesions ).

## . Nonenzymatic glycosylation of GBM > increase permeability, thickening.

. Nonenzymatic glycosylation of efferent arterioles > increase GFR > mesangial expansion.