Dumas R, Dumas M L, Baldet P, Bascoul S
Arch Fr Pediatr. 1982 Feb;39(2):75-8.
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.
对两名患有膜性肾小球肾炎的兄弟进行了解剖学和免疫学研究。年龄较大的儿童出现肾衰竭、范科尼综合征，其血浆中存在抗肾小管基底膜（TBM）抗体。肾活检显示为伴有膜性肾小球肾炎的严重肾小管间质性疾病。由于肾功能迅速恶化，该患者开始接受血液透析。进行了尸体肾同种异体移植，但未成功。肾移植活检显示严重的血管排斥病变，原发性疾病未复发。年龄较小的儿童在6个月时因肾病综合征伴轻度肾功能不全接受检查，于9个月时死亡。其血清中未检测到抗TBM抗体。患者的母亲表现为无症状蛋白尿，其血浆中存在抗TBM抗体。在同一家庭中，叔叔在3个月时死于类固醇抵抗性肾病综合征。本文讨论了家族性膜性肾小球肾炎与肾小管间质性疾病之间的关系。
