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埃及原发性肾病综合征活检患儿的病理特征:15年单中心经验

Pathological profile of biopsied Egyptian children with primary nephrotic syndrome: 15-year single center experience.

作者信息

Bakr Ashraf, Eid Riham, Sarhan Amr, Hammad Ayman, El-Refaey Ahmed Mahmoud, El-Mougy Atef, Zedan Mohammed Magdy, Moustafa Fatma, Abdelrahman Ashraf

机构信息

Pediatric Nephrology Unit, Mansoura University Children's Hospital, Mansoura, Egypt.

出版信息

J Nephrol. 2014 Aug;27(4):419-23. doi: 10.1007/s40620-013-0032-1. Epub 2014 Jan 16.

DOI:10.1007/s40620-013-0032-1
PMID:24430764
Abstract

BACKGROUND

Primary nephrotic syndrome (PNS) in children is a common problem worldwide. The pathological pattern of PNS differs between countries. However, data on the pathological pattern of PNS in Egyptian children are scant. This study was conducted to determine indications and results of renal biopsy in Egyptian children with PNS from a single tertiary children's hospital.

METHODS

Medical records of all children with PNS aged 3 months-18 years who underwent renal biopsy from 1998 to 2012 at Mansoura University Children's Hospital were retrospectively reviewed.

RESULTS

A total of 741 patients (441 males, 300 females) underwent 798 biopsies of which only four specimens were insufficient. Mean age at time of biopsy was 7.4 ± 3.6 years. The main indication for biopsy was steroid resistance (n = 354, 44.4%) followed by atypical PNS (n = 234, 29.3%) of which gross hematuria was the most common cause. Minimal change disease (MCD) and its variants were the most frequent pathology (n = 431, 54.3%) irrespective of biopsy indication, and incidence of focal segmental glomerulosclerosis was observed to be increasing over the years.

CONCLUSION

This is the first large study on the pathological pattern of PNS in children from Egypt, and it shows that MCD is the most frequent underlying pathology and steroid resistance is the most frequent indication for biopsy.

摘要

背景

儿童原发性肾病综合征(PNS)是全球范围内的常见问题。PNS的病理模式在不同国家有所不同。然而,关于埃及儿童PNS病理模式的数据却很少。本研究旨在确定来自一家单一的三级儿童医院的埃及PNS患儿肾活检的指征和结果。

方法

回顾性分析了1998年至2012年在曼苏拉大学儿童医院接受肾活检的所有3个月至18岁PNS患儿的病历。

结果

共有741例患者(441例男性,300例女性)接受了798次活检,其中只有4份标本不足。活检时的平均年龄为7.4±3.6岁。活检的主要指征是激素抵抗(n = 354,44.4%),其次是非典型PNS(n = 234,29.3%),其中肉眼血尿是最常见的原因。微小病变肾病(MCD)及其变异型是最常见的病理类型(n = 431,54.3%),与活检指征无关,且局灶节段性肾小球硬化的发病率多年来呈上升趋势。

结论

这是第一项关于埃及儿童PNS病理模式的大型研究,表明MCD是最常见病理类型,激素抵抗是最常见的活检指征。

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本文引用的文献

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The increasing incidence of initial steroid resistance in childhood nephrotic syndrome.儿童肾病综合征初始激素耐药发生率的增加。
Pediatr Nephrol. 2012 Jun;27(6):927-32. doi: 10.1007/s00467-011-2083-7. Epub 2012 Jan 10.
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Epidemiology of primary nephrotic syndrome in Egyptian children.埃及儿童原发性肾病综合征的流行病学。
J Nephrol. 2012 Sep-Oct;25(5):732-7. doi: 10.5301/jn.5000051.
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Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience.埃及儿童原发性局灶节段性肾小球硬化症:10 年单中心经验。
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Therapies for steroid-resistant nephrotic syndrome.类固醇抵抗型肾病综合征的治疗方法。
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Focal segmental glomerulosclerosis--epidemiology aspects in children and adults.局灶节段性肾小球硬化——儿童和成人的流行病学方面
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Experience of renal biopsy in children with nephrotic syndrome.儿童肾病综合征肾活检的经验
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High incidence of initial and late steroid resistance in childhood nephrotic syndrome.儿童肾病综合征初始和晚期激素抵抗的高发生率。
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