Glossary of glomerular diseases 2

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nephrotic syndrome
proteinuria; hypoalbuminemia; generalized edema; hyperlipidemia; hyperlipiduria; oval fat bodies or maltese crosses in urine; vulnerability to infections- esp staph and pneumococci; thrombotic and thromboembolic complications; loss of size and charge barrier of GBM
tx of nephrotic syndrome
ACEI or ARB; decrease proteinuria, salt restriction, and diuretics; low cholesterol diet & statins; prophylactic ASA and anticoagulants
nephritic syndrome
caused by diseases that evoke inflammatory proliferative response within the glomeruli; hematuria; proteinuria; diminished GFR; hypertension
Minimal Change nephropathy
most frequent cause of NS in kids (85-90%); peak age 2-8 yrs; males 2:1; no HTN and renal fxn preserved in most; allergic history in kids; paraneoplastic in adults or NSAID therapy
MC disease LM
normal appearance of glomeruli; cells of PT filled with lipids--foam cells
MC disease IF
no stains
MC disease EM
fusion of foot processes; microvilli formation; directly related to degree of proteinuria
tx of minimal change disease
90-95% show complete remission within 8 wks of corticosteroid therapy; 75% relapse; then might treat with cyclophosphamide or cyclosporine; no tendency to progress to chronic disease
Focal segmental glomerulosclerosis
10-15% of nephrotic syndrome in kids and adults; HTN, renal failure, and/or microscopic hematuria; assoc with HIV, heroin, massive obesity, unilateral renal agenesis, vesicoureteric reflux, and pamidronate; very bad prognosis.
focal sclerosis of glomeruli; focal fibrosis of glomeruli; with HIV nephropathy--tubules dilated and filled with eosinophilic material; interstitium with lymphoplasmacytic infiltrate
IgM and C3 in areas of sclerosis
FSGS EM for HIV nephropathy
tubuloreticular inclusions noted in endothelial cells; fusion of foot processes and wrinkling of basement membrane;
prognosis and tx of FSGS
not much tx; prolonged corticosteroids >6 mo; HAART in AIDS; 50% suffer progressive decline in renal fxn for period up to 10 yrs; 20% progress to ESRD in less than 3 yrs; disease recurs often in transplanted kidneys. recurrence 40-50% in allografts.
membranous nephropathy
most frequent cause of NS in adults (25-30%); idiopathic form in 85%; associations in secondary form- tumors, SLE, drugs, infections; in situ complex formation but in 85% no antibody detected
membranous neph - LM
enlarged glom but normocellular; capillary walls thickened; late: sclerotic and hyalinized; "bowl of cheerios"
MN- jones silver
granular peripheral staining of IgG and C3 along entire GBM; very irregular deposits
sub-epithelial electron dense deposits that sit on top of GBM
prognosis of membranous nephropathy
idiopathic: chronic proteinuria and slow deterioration to renal failure within 5-15 yrs in 33%; spntaneous remission in 33%; proteinuria with stable renal fxn in 33%; young females and kids without renal insufficiency are easier to treat; recovery seen in secondary form once offending antigen eliminated. if male, >50 yrs, severe NS, renal insufficiency--corticosteroids and cytotoxic agents;
membranoproliferative glomerulonephritis (MPGN)
membrane thickening; endothelial and mesangial cell proliferation; ages 5-30; usual presentation is NS in 50%, but also asx proteinuria (30%) and acute nephritis (20%)
types of MPGN
type I: chronic with glomerular deposition of circulating immune complexes, but inciting antigen is unknown; assoc with hep C and B, SLE, mixed cryo
type II MPGN
dense deposit disease; unclear pathogenesis; assoc with partial lipodystrophy. Low serum C3 levels-- in 70% at onset or during dz course; serum C3 nephritic factor = IgG autoantibody that stabilizes C3 convertase and can activate alternate complement pathway. LOW LEVEL OF C3.
essential mixed cryoglobulinemia
mixed cryos with monoclonal IgM RF and polyclonal IgG. middle aged women; palpable purpura, fever, raynaud's, arthralgias. Serum C3 normal, low serum C4, RF +. Hep C in 50%; HCV antibody, HC core antigens, and HC RNA in cryo and renal deposits.
lobular accentuation of glomeruli secondary to mesangial hypercellularity and increased mesangial matrix; thickening of capillary loops.
MPGN - jones silver
splitting/double contour of BM (tram track) due to mesangial interposition.
differentiates two types; type I has granular peripheral deposits of C3, IgG, C1q, C4+; type II has C3+ as mesangial rings. type II only stains C3.
MPGN type I EM
sub endothelial electron dense deposits
electron dense deposits in the region of the lamina densa; so called Dense Deposit Disease.
prognosis of MPGN
poor; most to end stage renal failure; type II prognosis worse than type I. no evidence that steroids or immunosuppressives work; pts with hep C can be treated with alpha interferon and plasmapheresis in cases of severe renal dz; recurrent disease in transplanted kidney is common- esp type II.

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