U06-7465#355924710

Proteinuria

• 52 year old female followed for dextrocardia and Tetralogy of Fallot complicated by pulmonary hypertension and right heart failure. A valve repair ? commissurotomy had been done at age 12. Currently under evaluation for heart transplant listing vs repair of stenotic/ regurgitant Ao, mitral and pulmonary valves. Also noted asplenia.

• Referred to evaluate proteinuria. NetCare records 1+ proteinuria intermittently for 3 years, with benign urine sediment and stable creatinine ~ 80 uM. 4 months prior to referral proteinuria quantitated at 400 mg/d.

• On evaluation Scr 79 uM. Urinalysis 1-5 rbc/hpf and P/Cr ~195 mg/mmol. Viral serology negative. SPEP, ANA, and complements normal.

• IgG- negative, moderately high background• IgA- negative• IgM- mild mesangial staining• C3- strong vascular and mild vascular pole• C1q- negative• Kappa- moderate to strong background• Lambda- mild to moderate background• Fibrin- mild non-specific interstitial staining• Albumin- moderate diffuse background with some

glomerular accentuation (similar to the IgG, Kappa and Lambda)

IF

IgM

C3

Fibrin

Diagnosis:Renal Biopsy:

Focal segmental glomerulosclerosis withglomerulomegaly, secondary to cyanoticcongenital heart disease.