[Membranous glomerulonephritis in two brothers associated in one with tubulo-interstitial disease, Fanconi syndrome and anti-TBM antibodies (author's transl)].
Anatomical and immunological studies were performed in two brothers with membranous glomerulonephritis. The older child presented with renal failure, Fanconi syndrome and anti-TBM antibody in his plasma. Renal biopsy revealed severe tubulo-interstitial disease with membranous glomerulonephritis. Because of rapidly progressive renal insufficiency the patient was started on hemodialysis. A cadaver renal allotransplantation was performed without success. Renal transplant biopsy showed severe lesions of vascular rejection without recurrence of the primary disease. The younger child was examined at six months for a nephrotic syndrome with mild renal insufficiency, and died at nine months. Anti TBM antibodies were not detected in his serum. The patient's mother presented with asymptomatic proteinuria, and anti TBM antibodies in her plasma. In the same family the uncle died at 3 months with a steroid resistant nephrotic syndrome. The relationships between the familial membranous glomerulonephritis and the tubulo interstitial disease are discussed.
D001323:Autoantibodies / Q000032:analysis*; D001485:Basement Membrane / Q000276:immunology; D002648:Child; D005198:Fanconi Syndrome / Q000175:diagnosis*; D005455:Fluorescent Antibody Technique; D005921:Glomerulonephritis / Q000235:genetics* / Q000473:pathology; D006801:Humans; D007223:Infant; D007684:Kidney Tubules / Q000276:immunology; D008297:Male; D009395:Nephritis, Interstitial / Q000235:genetics* / Q000473:pathology
D001323:Autoantibodies
Glomérulonéphrite extra-membraneuse chez deux frères associée dans un cas à une néphropathie tubulo-interstitielle avec syndrome de Fanconi et anticorps antimembrane basale tubulaire.