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Flashcards in Glomerular Disease Details Deck (65)
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31

Alport Syndrome presentation?

Eye problems (retinopathy, lens dislocation) + glomerulonephritis + sensorineural deafness
+ Family hx

32

Alport Syndrome pathogenesis

Mutation in type IV collagen --> thinning and splitting (lamellated) of glomerular BM

33

Hereditary pattern of Alport Syndrome?

X-linked dominant

34

Alport Syndrome EM?

"Basket weave" appearance

35

Membranoproliferative glomerulonephritis presentation?

Nephritic syndrome that often copresents with nephrotic syndrome

36

Type I MPGN associations?

Hepatitis B or C

37

Type I MPGN Pathology

- SUBENDOTHELIAL IC deposits
- Granular IF
- "tram track" on PAS stain due to GBM splitting caused by mesangial ingrowth

38

Type II MPGN associations?

C3 nephritic factor --> stabilizes C3 convertase --> decreased serum C3 levels

39

What is Type II MPGN also called?

Dense deposit disease

40

Characteristics of nephrotic syndrome?

Massive proteinuria with hypoalbuminemia --> edema, hyperlipidemia (blood becomes thin so liver increases fat)
- Frothy urine with fatty casts

41

What is nephrotic syndrome caused by?

Podocyte damage --> disrupts glomerular filtration charge barrier

42

Why is nephrotic syndrome associated with hypercoagulable state?

Pee out ATIII in urine

43

What is nephrotic syndrome associated with infection?

Pee out Ig in urine

44

Presentation of minimal change disease (liphoid nephrosis)?

- KIDS
- Idiopathic
- Triggered by recent infection, immunization, immune stimulus
- May be secondary to lymphoma (CK-mediated damage)

45

Minimal change disease LM

NORMAL glomeruli

46

Minimal change disease IF

Negative

47

Minimal change disease EM

Effacement of foot processes due to CKs

48

Describe the proteinuria of Minimal change disease?

Selective (loss of Alb but not Ig)

49

Treatment of minimal change disease?

Excellent response to steroids

50

Focal Segmental Glomerulosclerosis presentation?

AA and hispanics
Idiopathic
HIV
Sickle Cell
Heroin abuse
Massive obesity
Interferon treatment
Chronic kidney disease due to congenital malformations

51

Focal Segmental Glomerulosclerosis LM

Segmental sclerosis and hyalinosis

52

Focal Segmental Glomerulosclerosis IF

Often negative
May be positive for nonspecific focal deposits of IgM, C3, C1

53

Focal Segmental Glomerulosclerosis EM

Effacement of foot processes

54

Focal Segmental Glomerulosclerosis treatment?

Inconsistent response to steroids, may progress to chronic renal disease

55

Membranous Nephropathy (Membranous GN) presentation?

Caucasian adults
Primary - Ab to phospholipase A2 R
Secondary - drugs (NSAIDs, penicillamine), infections (HBV, HCV), SLE, or solid tumors

56

Membranous Nephropathy LM

Diffuse capillary and GBM thickening

57

Membranous Nephropathy IF

Granular as a result of IC deposition (nephrotic presentation of SLE)

58

Membranous Nephropathy EM

"Spike and dome" appearance with subepithelial deposits

59

Membranous Nephropathy treatment?

Poor response to steroids, may progress to chronic renal disease

60

Diabetic Glomerulonephropathy presentation?

Diabetes w/ proteinuria