Isolated Glomerular Diseases with Recurrent Gross Hematuria

Question 1

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

Answer 1

1-2

Question 2

Gross hematuria in IgA nephropathy typically resolves within

Answer 2

5 days

Question 3

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

Answer 3

7-21 days

Question 4

Gross hematuria in acute PSGN can last as long as

Answer 4

4-6 weeks

Question 5

MC glomerular disease in children

Answer 5

IgA nephropathy

Question 6

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

Answer 6

F, ABSENCE of systemic disease

Question 7

T/F Dx of IgA nephropathy requires renal biopsy

Answer 7

T

Question 8

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

Answer 8

Epithelial cell crescent

Question 9

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

Answer 9

C3 complement

Question 10

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

Answer 10

Poorly galactosylated IgA1 in the serum

Question 11

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

Answer 11

T

Question 12

IgA nephropathy is seen more often in what gender

Answer 12

Male

Question 13

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

Answer 13

T

Question 14

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

Answer 14

F, UNCOMMON

Question 15

___ help to distinguish IgA nephropathy from post- streptococcal glomerulonephritis

Answer 15

Normal serum levels of C3 in IgA nephropathy

Question 16

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

Answer 16

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

Question 17

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

Answer 17

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

Question 18

The primary treatment of IgA nephropathy is

Answer 18

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

Question 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

Answer 19

Angiotensin-converting enzyme inhibitors and angiotensin II receptor antagonists

Question 20

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

Answer 20

Immunosuppressive therapy with corticosteroids

Question 21

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

Answer 21

> 60 mL/min/m2

Question 22

AKA hereditary nephritis

Answer 22

Alport syndrome

Question 23

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

Answer 23

Type IV collagen

Question 24

Approximately 85% of Alport Syndrome patients have ___ inheritance

Answer 24

X-linked

Question 25

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

Answer 25

Alport syndrome

Question 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

Answer 26

Alport syndrome

Question 27

Pathognomonic of Alport syndrome

Answer 27

Anterior lenticonus

Question 28

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

Answer 28

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

Question 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 ___

Answer 29

X-linked Alport Syndrome

Question 30

Risk factors for progression of Alport Syndrome to ESRD are

Answer 30

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

Question 31

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

Answer 31

ACEi and ARBs

Question 32

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

Answer 32

ACEi and ARBs

Question 33

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

Answer 33

Thin basement membrane disease (TBMD)

Question 34

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

Answer 34

Benign familial hematuria

Question 35

TBMD may be sporadic or transmitted as a ____ trait

Answer 35

Autosomal dominant