一名儿童的肾病综合征和原发性肾小管淀粉样变性：病例报告

Nephrotic syndrome and primary tubular amyloidosis in a child: a case report.

作者信息

al Kahtani W, al Edressi H, Wright E A, Abdurrahman M B

机构信息

Department of Paediatrics, College of Medicine, Riyadh, Saudi Arabia.

出版信息

Pediatr Nephrol. 1989 Oct;3(4):454-7. doi: 10.1007/BF00850227.

DOI:10.1007/BF00850227

PMID:2642117

Abstract

Nephrotic syndrome in a 19-month-old child was associated with an unusual form of primary renal amyloidosis. The amyloid deposit was in the tubulo-interstitium and not in the glomeruli. Although the deposit did not stain with Congo red, it had the ultrastructural characteristics of amyloid. The patient had resistant nephrotic syndrome, and he subsequently developed microhaematuria and glucosuria.

摘要

一名19个月大儿童的肾病综合征与一种不寻常的原发性肾淀粉样变性有关。淀粉样沉积物位于肾小管间质，而非肾小球。尽管该沉积物刚果红染色阴性，但具有淀粉样蛋白的超微结构特征。该患者患有难治性肾病综合征，随后出现镜下血尿和糖尿。