Isolated Glomerular Diseases with Recurrent Gross Hematuria Flashcards Preview

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Flashcards in Isolated Glomerular Diseases with Recurrent Gross Hematuria Deck (35):
1

Presentation with gross hematuria is common within ___ days after the onset of an apparent viral upper respiratory tract infection in IgA nephropathy

1-2

2

Gross hematuria in IgA nephropathy typically resolves within

5 days

3

Latent period between onset of streptococcal pharyngitis or impetigous skin infection and development of acute PSGN

7-21 days

4

Gross hematuria in acute PSGN can last as long as

4-6 weeks

5

MC glomerular disease in children

IgA nephropathy

6

T/F IgA nephropathy is characterized by a predominance of IgA within mesangial glomerular deposits in the presence of systemic disease

F, ABSENCE of systemic disease

7

T/F Dx of IgA nephropathy requires renal biopsy

T

8

Renal histology in IgA nephropathy demonstrates mesangial proliferation that may be associated with formation of

Epithelial cell crescent

9

IgA deposits in the mesangium in IgA nephropathy is often accompanied by

C3 complement

10

IgA nephropathy is an immune complex disease initiated by excessive amounts of ___ causing production of IgA and IgG autoantibodies

Poorly galactosylated IgA1 in the serum

11

T/F Familal clustering is seen in cases of IgA nephropathy

T

12

IgA nephropathy is seen more often in what gender

Male

13

T/F The clinical presentation of childhood IgA nephropathy is often benign in comparison to that of adults

T

14

T/F IgA nephropathy is an common cause of end-stage renal failure during childhood

F, UNCOMMON

15

___ help to distinguish IgA nephropathy from post- streptococcal glomerulonephritis

Normal serum levels of C3 in IgA nephropathy

16

T/F Serum IgA levels have diagnostic value in IgA nephropathy

F, they are elevated in only 15% of pediatric patients

17

Poor prognostic indicators at presentation or follow-up in patients with IgA nephropathy include

1) Persistent hypertension 2) Diminished renal function 3) Significant, increasing, or prolonged proteinuria

18

The primary treatment of IgA nephropathy is

1) Appropriate blood pressure control 2) Management of significant proteinuria

19

In patients with IgA nephropathy ___ and ___ are effective in reducing proteinuria and retarding the rate of disease progression when used individually or in combination

Angiotensin-converting enzyme inhibitors and angiotensin II receptor antagonists

20

In patients with IgA nephropathy, if renin-angiotensin blockade proves ineffective and significant proteinuria persists, then addition of ___ is recommended

Immunosuppressive therapy with corticosteroids

21

Corticosteroids reduce proteinuria and improve renal function in those patients with a glomerular filtration rate is

>60 mL/min/m2

22

AKA hereditary nephritis

Alport syndrome

23

Alport syndrome is a genetically heterogeneous disease caused by mutations in the genes coding for ___

Type IV collagen

24

Approximately 85% of Alport Syndrome patients have ___ inheritance

X-linked

25

Lipid-containing tubular or interstitial cells, called foam cells are seen in biopsy specimens of what disease entity

Alport syndrome

26

Progressive proteinuria, often exceeding 1 g/24 hr, is common by the 2nd decade of life and can be severe enough to cause nephrotic syndrome

Alport syndrome

27

Pathognomonic of Alport syndrome

Anterior lenticonus

28

Alport syndrome is highly likely in the patient who has hematuria and at least 2 of the following characteristic clinical features

1) Macular flecks 2) Recurrent corneal erosions 3) GBM thickening and thinning 4) Sensorineural deafness

29

Absence of epidermal basement membrane staining for the α5 chain of type IV collagen in male hemizygotes and discontinuous epidermal basement membrane staining in female heterozygotes on skin biopsy is pathognomonic for ___

X-linked Alport Syndrome

30

Risk factors for progression of Alport Syndrome to ESRD are

1) Gross hematuria during childhood 2) Nephrotic syndrome 3) Prominent GBM thickening

31

___ can slow the rate of progression of Alport Syndrome to ESRD

ACEi and ARBs

32

Pharmacologic treatment of proteinuria in Alport Syndrome is done with the use of

ACEi and ARBs

33

Defined by the presence of persistent microscopic hematuria and isolated thinning of the GBM (and, occasionally, tubular basement membranes) on electron microscopy

Thin basement membrane disease (TBMD)

34

Isolated hematuria in multiple family members without renal dysfunction is referred to as

Benign familial hematuria

35

TBMD may be sporadic or transmitted as a ____ trait

Autosomal dominant