Glomerulonephritides in Kids Flashcards
Edema of nephritic syndrome is typically most prominent on which part of the day?
Early in the morning (esp. on px dependent side)
T/F. Pregnancy is not an exacerbating factor for lupus nephritis
False (an exacerbating factor)
T/F. Acute nephrotic syndrome commonly affects adults
False (young children)
T/F. Typical prodrome of acute nephrotic syndrome in children
Gastroenteritis
Clinical criteria of acute nephritic syndrome
Edema, hypertension, hematuria, oliguria
Differentiate hpn of nephritic and nephrotic syndrome
Nephritic: HPN due to fluid retention VS Nephrotic: hypoalbuminuria → 3rd spacing → IV volume depletion
Most benign criteria in nephritic syndrome and also most common cause of consult
Hematuria
Hereditary nephritis
Alport syndrome
Most common cause of glomerulonephritis 2⁰ to isolated renal disease in our locality
Post infectious/strep GN
Most common cause of glomerulonephritis 2⁰ to isolated renal disease globally
IgA nephropathy
T/F. Membranous nephropathy is typically a disease of children
False (adults)
Commonly misdiagnosed as post strep GN
Membranoproliferative GN
Cause of GN with worst prognosis
Focal segmental glomerulosclerosis (FSGS)
T/F. Sickle cell glomerulopathy is common in Ph
False (seldom seen)
Typical picture of hemolytic uremic syndrome
GI symptoms prodrome then hematuria and abdominal pain
Difference of HUS from SLE
Type of rash and elevated BUN and creatinine
Most common glomerular cause of gross hematuria in children
Acute post streptococcal GN
Organisms associated with APSGN
Group A β-hemolytic streptococci
Immunofluorescent microscopy of APSGN
Lumpy bumpy deposits of Ig and complement in glomerular basement membrane
Reward given by doc to px after 6-12 weeks of management
McDonald’s
T/F. In a suspected APSGN px, confirm ddx if manifestations are triggered after 6-12 weeks of management
False (consider other ddx)
APSGN common in this age group
5-12 y.o.
Acute nephritic syndrome develops when post strep pharyngitis
1-2 weeks
Acute nephritic syndrome develops when after strep pyodermata
3-6 weeks
T/F. Px referred late for APSGN should not be dialyzed
False (undergo dialysis)
Management of rapidly progressing glomerulonephritis
Immediate prednisolone tx and a course peritoneal dialysis
Phases of APSGN
Oliguric, Diuretic, Convalescence
Why is there edema in APSGN?
Salt and water retention
Adequate urine output
≥1 cc/kg/hour
What to monitor in diuretic phase of APSGN
Dehydration and electrolytes (e.g. Na, K)
C3 levels in APSGN
Reduced because they get deposited in the glomerular basement membrane
C3 levels in rapidly progressing glomerulonephritis
Elevated
T/F. ASO titers are always elevated after strep skin infection
False (rarely only compared to throat infection; anti-DNase B level used to document cutaneous strep)
Which is more specific: Streptozyme test or ASO titers?
Streptozyme test but ASO is enough for ddx
C4 levels in APSGN
Often normal or only mildly depressed
Neurologic involvement in APSGN
Posterior leukoencephalopathy in the parieto-occipital areas
Most common complication of APSGN
HPN
Drug of choice for APSGN
Penicillin for 10 days
1st line drugs for HPN
Diuretics
Safest anti-HPN in children
Calcium channel blockers
T/F. ACE inhibitors and ARBs may worsen renal hypoperfusion
True, use w/ caution
T/F. Skin infections are not risk for PSGN
False (risk)
Clinical criteria for acute nephrotic syndrome
Edema, Normal BP, Proteinuria, Hypercholesterolemia
Edema of nephrotic syndrome
Anasarca and 2⁰ to protein loss and drop in oncotic pressure
Proteinuria in pedia
≥40 mg/m2
Most common cause of nephrotic syndrome
Primary (idiopathic) nephrotic syndrome (INS)
Nephrotic common in
Males; 2-6 y.o.
Common cause of INS
Minimal change disease
Pathology of minimal change disease
T cell dysfunction → alteration of cytokines → loss of negative charged glycoprotein in glomerular capillary
Mutated podocyte proteins in FSGS
Podocin and alpha actinin
Mutated podocyte proteins in steroid resistant nephrotic syndrome
NPHS2 (podocin) and WT1 genes
Reason why FSGS still recur in transplanted kidneys
Plasma factor by lymphocutes causing permeability
WT1 gene also expressed in what tumor?
Wilm’s tumor
What to give in px with genital edema since it’s very painful
Albumin
Proteinuria in INS
+3 or +4 proteinuria
Serum creatinine, C3 and C4 in INS
Normal
T/F. Children with uncomplicated nephrotic syndrome, 1-8 y.o. likely to be unresponsive to steroid
False (responsive)
Gold standard drug for steroid resistant nephrotic syndrome
Prednisone, 60 mg/m2 for 6 weeks
Alternative tx aside from steroid
Cyclophosphamide
Steroid sparing agent
Cyclosporine
Adjunct tx to reduce proteinuria
ACE inhibitors and angiotensin II blockers
Most frequent type of infection in nephrotic syndrome
Spontaneous bacterial peritonitis by strep pneumonia
Also known as Berger Nephropathy and may cross between nephritic and nephrotic and the most common chronic glomerular disease worldwide
IgA Nephropathy
Part where IgA is deposited in IgA nephropathy
Mesangium of glomerulus
C3 in IgAN
Normal vs post strep nga reduced
T/F. Ddx of IgAN also does not require renal biopsy
False (requires)
Closely related disease of IgAN
Henoch-Schonlein purpura
What to suspect if px has gross hematuria 2-3 days after URTI
IgA Nephropathy
Mainstay of tx in IgAN
ACEIs or ARBS (primary tx is proper bp control and reduce proteinuria)
PSAGN vs IgAN
PSAGN: hematuria 2-3 weeks after infection
IgAN: within days of iinfection
Form of hereditary nephritis with mutation in the COL4A5 gene encoding for the alpha chain of type IV collagen
Alport syndrome
Extrarenal manifestations of alport syndrome
Hearing deficits and ocular abnormalities
Presence of persistent microscopic hematuria and isolated thinning of the basement membrane on electron microscopy
Thin glomerular basement membrane disease
Most common cause of nephrotic syndrome in adults, but an uncommon cause of hematuria in children
Membranous nephropathy
Most common cause of Membranous nephropathy worldwide
Malaria
Most common small vessel vasculitis in pedia
Henoch schonlein purpura
Features of HSP
Purpuric rash and commonly accompanied by arthritis and abdominal pain
T/F. The glomerular findings can be indistinguishable from those of IgA nephropathy
True
T/F. Mild HSP nephritis requires treatment because it usually does not resolve spontaneously.
False (no tx because it resolves spontaneously)
One of the most common causes of community acquired acute kidney failure in pedia (<1 y.o. with acute gastroenteritis)
Hemolytic uremic syndrome
Triad of HUS
Acute renal failure, anemia, hx of AGE
Typical agents in HUS
E. coli and STX producing shigella dysenteriae; Non STX assoc (atypical)
Pathologic hallmark of HUS
Fibrin-platelet thrombi deposition in affected organs
T/F. Biopsy needed in ddx of HUS
False
Symptoms in HUS appear when post exposure
1-8 days ave 3 days
T/F. The prognosis for HUS not associated with diarrhea is more severe
True
