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微小病变病及两种系膜增生性肾小球肾炎变体所致儿童肾病综合征肾活检的临床特征及肾小球免疫荧光

Clinical features and glomerular immunofluorescence of renal biopsies from children with nephrotic syndrome due to minimal change disease and two variants of mesangial proliferative glomerulonephritis.

作者信息

Hui A, Poucell S, Thorner P, Rance C P, Baumal R

出版信息

Int J Pediatr Nephrol. 1984 Mar;5(1):5-10.

PMID:6370883
Abstract

Response to initial course of prednisone and clinical outcome were reviewed in 82 children with nephrotic syndrome (NS) due to minimal change disease (MCD) and 2 variants of mesangial proliferative glomerulonephritis (MesPGN). Renal biopsies in Type I MesPGN showed only increased mesangial cells and matrix, whereas those in Type II MesPGN also showed interstitial fibrosis, tubular atrophy, global glomerulosclerosis and hyalinosis. Response to prednisone was complete in most cases (95% in MCD, 82% in Type I and 72% in Type II MesPGN). Remission of NS for more than 1 year while off prednisone occurred in 91% of patients with Type I MesPGN and 49% with MCD, but in only 14% with Type II MesPGN. The influence of immunoglobulins +/- complement in mesangial regions of glomeruli on initial response and outcome was assessed. Response to prednisone and outcome did not differ in IMF-positive and -negative MCD or in Type I and Type II MesPGN.

摘要

对82例因微小病变病(MCD)及2种系膜增生性肾小球肾炎(MesPGN)变体所致肾病综合征(NS)患儿的初始泼尼松治疗反应及临床结局进行了回顾性研究。I型MesPGN的肾活检仅显示系膜细胞和基质增多,而II型MesPGN的肾活检还显示间质纤维化、肾小管萎缩、球性肾小球硬化和透明变性。大多数病例对泼尼松治疗反应完全(MCD为95%,I型为82%,II型MesPGN为72%)。I型MesPGN患者中91%在停用泼尼松后NS缓解超过1年,MCD患者为49%,而II型MesPGN患者仅为14%。评估了肾小球系膜区免疫球蛋白±补体对初始反应和结局的影响。免疫荧光阳性和阴性的MCD或I型和II型MesPGN对泼尼松的反应及结局并无差异。

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