Fibrillary glomerulopathy in a 10-year-old female
Fibrillary glomerulopathy in a 10-year-old female
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10岁女性纤维性肾小球病
DOI：
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发表时间：
2001
期刊：
影响因子：
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通讯作者：
R. Baliga
中科院分区：
文献类型：
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作者：
D. Bahrami;J. Henegar;R. Baliga
抽象的。一个10岁的非洲裔美国女性被评估为无症状的蛋白尿。初步调查未发现其肾病的病因。她随后接受了经皮肾活检，这与肾小球疾病一致。这种情况在儿童中很少见，在组织学上通过直径约为18-22 nm的固体、随机排列的淀粉样纤维结构来识别。这是一种排除性诊断，其临床表现可以是多种多样的。儿童通常表现为肾病综合征。目前尚无有效的治疗方法。类固醇、细胞毒性药物和血浆置换治疗在稳定或改善肾功能方面没有任何真实的获益。而血管紧张素转换酶抑制剂可减少蛋白尿。已知50%的患者在诊断后4年内发生终末期肾病。我们的病人在没有任何治疗的情况下，在4年的随访中，肾病范围的蛋白尿显著减少，同时保持正常的肾功能。
Abstract. A 10-year-old African-American female was evaluated for asymptomatic proteinuria. Initial investigation did not reveal the etiology of her renal disease. She subsequently underwent a percutaneous renal biopsy, which was consistent with fibrillary glomerulopathy. This condition is rare in children and is identified histologically by a solid, randomly arranged, amyloid-like fibrillar structure with a diameter of about 18–22 nm. It is a diagnosis of exclusion, the clinical presentation of which can be quite varied. Children usually present with nephrotic syndrome. There is no effective therapy for this condition. Therapy with steroids, cytotoxic agents, and plasmapheresis does not confer any real benefit in stabilizing or improving renal function. However, angiotensin converting enzyme inhibitors can decrease the proteinuria. End-stage renal disease is known to occur in 50% of patients within 4 years of diagnosis. The nephrotic-range proteinuria in our patient significantly decreased during a 4-year follow-up without any therapy, while maintaining normal renal function.