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儿童特发性系膜IgA肾小球肾炎:19例儿科病例描述及文献综述

Idiopathic mesangial IgA-glomerulonephritis in childhood Description of 19 pediatric cases and review of the literature.

作者信息

Michalk D, Waldherr R, Seelig H P, Weber H P, Schärer K

出版信息

Eur J Pediatr. 1980 Jun;134(1):13-22. doi: 10.1007/BF00442397.

Abstract

Nineteen out of 83 children with asymptomatic haematuria were classified as having IgA glomerulonephritis, characterized by diffuse mesangial deposition of IgA in the absence of systematic disease. Different histological patterns were observed: i.e., minor glomerular lesions (5 cases), focal and segmental proliferative glomerulonephritis (8 cases) and diffuse proliferative glomerulonephritis (6 cases). Recurrent gross haematuria is the clinical hallmark of the disease. Proteinuria was present in 14 children and exceeded 1 g/m2/day in 3 patients. Clinico-pathological correlations showed a close relation between the degree of proteinuria and the histological lesions. Serum IgA levels were elevated in 3 children. Glomerular filtration rate remained above 80 ml/min/1.73 m2 in a 1 to 9 year follow-up.

摘要

83例无症状血尿患儿中,19例被诊断为IgA肾病,其特征为在无系统性疾病的情况下,IgA在肾小球系膜弥漫性沉积。观察到不同的组织学类型:即轻度肾小球病变(5例)、局灶节段性增生性肾小球肾炎(8例)和弥漫性增生性肾小球肾炎(6例)。反复肉眼血尿是该疾病的临床特征。14例患儿出现蛋白尿,3例患儿蛋白尿超过1g/m²/天。临床病理相关性分析显示蛋白尿程度与组织学病变密切相关。3例患儿血清IgA水平升高。在1至9年的随访中,肾小球滤过率保持在80ml/min/1.73m²以上。

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