赞比亚卢萨卡大学教学医院儿科患者原发性肾病综合征的组织病理学亚型

A. Mweemba, Paul Mashanga, Justor Banda
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摘要

背景:在发达国家,微小改变病(MCD)已被证明是儿童肾病综合征(NS)的常见组织学表现。在非洲,关于小儿神经痛的临床和组织学特征的信息很少。本研究评估了NS的特征和组织学亚型。方法:这是一项前瞻性研究,对在赞比亚卢萨卡最大的三级教学医院转诊的2-16岁诊断为NS的儿童进行连续索引。经伦理批准,在超声指导下对13例NS患儿进行活检。该研究的主要结果是描述小儿NS的组织学特征。结果:13名非洲裔儿童被纳入研究,平均发病年龄为9.25岁(2.0-15.0岁)。组织病理病变如下:4例MCD, 4例局灶节段性肾小球硬化(FSGS), 1例免疫复合物介导的肾小球肾炎(ICMN), 4例不确定。13例患者中有8例出现血尿(61.5%),5例出现高血压(38.0%)。三个孩子既没有血尿也没有高血压。13名参与者中有10人有原发性类固醇抵抗。结论:鉴于不典型的表现、多变的组织病理学结果和不可预测的类固醇治疗反应,本研究已经证明有必要对患有NS的儿科患者进行治疗前肾活检。
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Histopathological Subtypes of Primary Nephrotic Syndrome in Paediatric Patients at the University Teaching Hospital in Lusaka, Zambia
Background: Minimal change disease (MCD) has been shown to be a common histological presentation in children presenting with nephrotic syndrome (NS) in developed countries. In Africa, information on clinical and histological characteristics of paediatric NS is scarce. This study assessed the characteristics and histological subtypes of NS. Methods: This was a prospective study that consecutively indexed children aged 2-16 years with the diagnosis of NS who were referred to the largest tertiary teaching hospital, Lusaka, Zambia. Thirteen children presenting with NS were biopsied under ultrasound guidance after ethical approval. The primary outcome of the study was to describe the histological characteristics of paediatric NS. Results: Thirteen children of African descent with a median age of onset of NS of 9.25 years (2.0-15.0) were enrolled in the study. The histopathologic lesions were as follows; four had MCD, four had focal segmental glomerulosclerosis (FSGS), one had immune complex mediated glomerulonephritis (ICMN) and four were inconclusive. Haematuria was present in eight out of 13 patients (61.5%) and hypertension was present in five of 13 patients (38.0%). Three children did not have either haematuria or hypertension. Ten of the 13 participants had primary steroid resistance. Conclusion: This study has demonstrated the need to perform a pre-treatment renal biopsy in paediatric patients presenting with NS in view of the atypical presentation, variable histopathologic findings, and unpredictable response to steroid therapy.
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