Adhikari M, Bhimma R, Coovadia H M
Department of Paediatrics and Child Health, Faculty of Medicine, University of Natal, KwaZulu/Natal, South Africa.
Clin Nephrol. 2001 Jan;55(1):16-24.
The aim of this paper was to describe the clinical course, histological grading, response to therapies and complications of nephrotic children with focal segmental glomerulosclerosis (FSGS) in Durban, South Africa.
The demographic data, response to drug therapy, complications, renal function and disease status at the last hospital visit were recorded in children diagnosed as FSGS on histology. Biopsies were graded according to the degree of glomerulosclerosis and interstitial involvement.
Seventy-five children, 32 Indian and 43 Black were studied. FSGS as a proportion of all children seen with nephrotic syndrome, has risen between 1970 to 1995 from 1.8% to 20% in Indian children and 5% to 28% in Black children. Of those children followed for two years or more, the two racial groups were similar with respect to age at presentation, gender, histological grading, hypertension, renal outcome and response to therapies except for cushingoid features (p < 0.7), alopecia (p < 0.003) and respiratory tract infections (p < 0.001) which occurred more frequently in Indian children. Forty-three children were followed for between 2-16 years: 14 (10 Indian and 4 Black (p < 0.5)) (29%) had grade 1 changes, 24(13 Indian, 11 Black (p < 0.98)) (56%) had grade 2 changes, 3 (Indian (p < 0.26)) (7%) had grade 3 changes and 2 (Black (p < 0.17)) grade 4 changes. Over this period 27 (63%) had normal renal function, 6 (14%) died, 4 (9%) developed impaired renal function, 6 (14%) chronic renal insufficiency and 6 (14%) end-stage renal disease. Three of the latter were transplanted. At the last hospital visit, Indian children had remitted more often (p < 0.6) and Black children suffered relapse more frequently (p < 0.03). Two children had spontaneous disease remission, and 5 had sustained remission following a course of prednisolone and/or cyclophosphamide.
This, one of the largest studies of FSGS in children, shows a marked rise in this condition and some differences between Indian and Black children. The outcome in Black children was not as poor as anticipated.
本文旨在描述南非德班局灶节段性肾小球硬化（FSGS）肾病患儿的临床病程、组织学分级、对治疗的反应及并发症。
记录经组织学诊断为FSGS患儿的人口统计学数据、药物治疗反应、并发症、肾功能及上次就诊时的疾病状态。根据肾小球硬化程度和间质受累情况对活检标本进行分级。
共研究了75名患儿，其中32名印度裔和43名黑人。FSGS在肾病综合征患儿中所占比例，在1970年至1995年间，印度裔患儿从1.8%升至20%，黑人患儿从5%升至28%。在随访两年或更长时间的患儿中，除库欣样特征（p<0.7）、脱发（p<0.003）和呼吸道感染（p<0.001）在印度裔患儿中更常见外，两个种族在就诊年龄、性别、组织学分级、高血压、肾脏转归及对治疗的反应方面相似。43名患儿随访了2至16年：14名（10名印度裔和4名黑人（p<0.5））（29%）有1级改变，24名（13名印度裔，11名黑人（p<0.98））（56%）有2级改变，3名（印度裔（p<0.26））（7%）有3级改变，2名（黑人（p<0.17））有4级改变。在此期间，27名（63%）肾功能正常，6名（14%）死亡，4名（9%）出现肾功能损害，6名（14%）出现慢性肾功能不全，6名（14%）进入终末期肾病。其中3名接受了移植。在最后一次就诊时，印度裔患儿缓解更频繁（p<0.6），黑人患儿复发更频繁（p<0.03）。2名患儿疾病自发缓解，5名在接受泼尼松龙和/或环磷酰胺治疗后持续缓解。
这项针对儿童FSGS的最大规模研究之一表明，这种疾病显著增加，且印度裔和黑人患儿之间存在一些差异。黑人患儿的转归并不像预期的那么差。
