1. U06-7465 #355924710 Proteinuria

2. 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.

3. 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.

4. 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

5. IgM

6. C3

7. Fibrin

10. Diagnosis: Renal Biopsy: Focal segmental glomerulosclerosis with glomerulomegaly, secondary to cyanotic congenital heart disease.