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.
- FSGS LM
- focal sclerosis of glomeruli; focal fibrosis of glomeruli; with HIV nephropathy--tubules dilated and filled with eosinophilic material; interstitium with lymphoplasmacytic infiltrate
- FSGS IF
- 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
- MN- IF
- granular peripheral staining of IgG and C3 along entire GBM; very irregular deposits
- MN- EM
- 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.
- MPGN - LM
- 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.
- MPGN - IF
- 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
- MPGN type II EM
- 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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