Isolated Glomerular Diseases with Recurrent Gross Hematuria Flashcards
Presentation with gross hematuria is common within ___ days after the onset of an apparent viral upper respiratory tract infection in IgA nephropathy
1-2
Gross hematuria in IgA nephropathy typically resolves within
5 days
Latent period between onset of streptococcal pharyngitis or impetigous skin infection and development of acute PSGN
7-21 days
Gross hematuria in acute PSGN can last as long as
4-6 weeks
MC glomerular disease in children
IgA nephropathy
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
T/F Dx of IgA nephropathy requires renal biopsy
T
Renal histology in IgA nephropathy demonstrates mesangial proliferation that may be associated with formation of
Epithelial cell crescent
IgA deposits in the mesangium in IgA nephropathy is often accompanied by
C3 complement
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
T/F Familal clustering is seen in cases of IgA nephropathy
T
IgA nephropathy is seen more often in what gender
Male
T/F The clinical presentation of childhood IgA nephropathy is often benign in comparison to that of adults
T
T/F IgA nephropathy is an common cause of end-stage renal failure during childhood
F, UNCOMMON
___ help to distinguish IgA nephropathy from post- streptococcal glomerulonephritis
Normal serum levels of C3 in IgA nephropathy
T/F Serum IgA levels have diagnostic value in IgA nephropathy
F, they are elevated in only 15% of pediatric patients
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
The primary treatment of IgA nephropathy is
1) Appropriate blood pressure control 2) Management of significant proteinuria
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
In patients with IgA nephropathy, if renin-angiotensin blockade proves ineffective and significant proteinuria persists, then addition of ___ is recommended
Immunosuppressive therapy with corticosteroids
Corticosteroids reduce proteinuria and improve renal function in those patients with a glomerular filtration rate is
> 60 mL/min/m2
AKA hereditary nephritis
Alport syndrome
Alport syndrome is a genetically heterogeneous disease caused by mutations in the genes coding for ___
Type IV collagen
Approximately 85% of Alport Syndrome patients have ___ inheritance
X-linked
Lipid-containing tubular or interstitial cells, called foam cells are seen in biopsy specimens of what disease entity
Alport syndrome
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
Pathognomonic of Alport syndrome
Anterior lenticonus
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
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
Risk factors for progression of Alport Syndrome to ESRD are
1) Gross hematuria during childhood 2) Nephrotic syndrome 3) Prominent GBM thickening
___ can slow the rate of progression of Alport Syndrome to ESRD
ACEi and ARBs
Pharmacologic treatment of proteinuria in Alport Syndrome is done with the use of
ACEi and ARBs
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)
Isolated hematuria in multiple family members without renal dysfunction is referred to as
Benign familial hematuria
TBMD may be sporadic or transmitted as a ____ trait
Autosomal dominant
