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儿童戈谢病与系膜毛细血管性肾小球肾炎——一种巧合?

Gaucher's disease and mesangiocapillary glomerulonephritis in childhood--a coincidence?

作者信息

Halevi R, Davidovitz M, Mann S, Ben-Bassat M, Stark H, Eisenstein B

机构信息

Department of Pediatrics B, Haemek Hospital, Afula, Israel.

出版信息

Pediatr Nephrol. 1993 Aug;7(4):438-40. doi: 10.1007/BF00857561.

Abstract

A 6-year-old boy, presenting with a nephritic syndrome, was diagnosed as suffering from Gaucher's disease (GD) and mesangiocapillary glomerulonephritis (MCGN). GD was suspected because of aseptic necrosis of the femoral heads on X-ray and later confirmed by bone marrow aspiration and a lack of glucocerebrosidase activity in white blood cells; MCGN was documented on renal biopsy. The child was treated with prednisone, dipyridamole and aspirin, and recovered completely clinically. A second biopsy was not performed. The connection between these two rare diseases, and between nephritis and GD in general, is discussed.

摘要

一名6岁男孩,表现为肾病综合征,被诊断为患有戈谢病(GD)和系膜毛细血管性肾小球肾炎(MCGN)。由于X线显示股骨头无菌性坏死而怀疑患有GD,随后经骨髓穿刺及白细胞中缺乏葡萄糖脑苷脂酶活性得以确诊;肾活检证实患有MCGN。该患儿接受了泼尼松、双嘧达莫和阿司匹林治疗,临床症状完全恢复。未进行第二次活检。本文讨论了这两种罕见疾病之间的关联,以及一般情况下肾炎与GD之间的关联。

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