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南非儿童的肾病综合征:20 年中的观点变化

Nephrotic syndrome in South African children: changing perspectives over 20 years.

作者信息

Bhimma R, Coovadia H M, Adhikari M

机构信息

Department of Paediatrics and Child Health, University of Natal, Faculty of Medicine, Congella, South Africa.

出版信息

Pediatr Nephrol. 1997 Aug;11(4):429-34. doi: 10.1007/s004670050310.

Abstract

We review our 20-year experience of 636 children with nephrotic syndrome (NS) in Durban, South Africa; 306 (48.2%) were blacks, 307 (48.2%) Indians and 23 (3.6%) were a mixed group (coloured); 91 (14.3%) could not be categorised and were excluded from the analysis. In Indian children, 134 of 286 (46.8%) had biopsy-proven minimal change NS (MCNS) and 94.8% of these were steroid sensitive (SS); 60 (21%) had SSNS but without renal biopsy; 59 (20.6%) had focal segmental glomerulosclerosis (FSGS), with only 4.4% of these being SS. In blacks, membranous nephropathy accounted for 40% of cases; 86.2% were associated with hepatitis B virus antigens. Typical SSNS continues to be uncommon among blacks. Only 14.4% had either biopsy-proven SS-MCNS or SSNS; 32 had MCNS lesions on biopsy, but 18 were steroid resistant (SR); 67 of 236 (28.4%) had FSGS, all of whom were SR. Among coloured patients, 5 of 23 (21.7%) had biopsy-proven SS-MCNS and or unbiopsied SSNS; 10 (43.5%) had FSGS and 6 of 23 (26.1%) had membranous nephropathy. Proliferative lesions were present in only 2 of 23 (8.6%) coloured patients and was uncommon in all population groups. Overall mortality was 3.1%. In brief, this is the largest reported series of NS among children in Africa and shows a typical pattern in Indians, an unusual pattern of histological types in blacks and an intermediate picture in coloureds.

摘要

我们回顾了在南非德班对636例肾病综合征(NS)患儿的20年经验;306例(48.2%)为黑人,307例(48.2%)为印度人,23例(3.6%)为混血儿(有色人种);91例(14.3%)无法分类,被排除在分析之外。在印度儿童中,286例中有134例(46.8%)经活检证实为微小病变型肾病综合征(MCNS),其中94.8%对类固醇敏感(SS);60例(21%)为类固醇敏感型肾病综合征(SSNS)但未进行肾活检;59例(20.6%)为局灶节段性肾小球硬化症(FSGS),其中只有4.4%对类固醇敏感。在黑人中,膜性肾病占病例的40%;86.2%与乙型肝炎病毒抗原相关。典型的SSNS在黑人中仍然不常见。只有14.4%经活检证实为类固醇敏感型MCNS或SSNS;32例活检显示有MCNS病变,但18例对类固醇耐药(SR);236例中有67例(28.4%)患有FSGS,均为SR。在有色人种患者中,23例中有5例(21.7%)经活检证实为类固醇敏感型MCNS和/或未经活检的SSNS;10例(43.5%)患有FSGS,23例中有6例(26.1%)患有膜性肾病。增殖性病变仅在23例有色人种患者中的2例(8.6%)中出现,在所有人群中都不常见。总体死亡率为3.1%。简而言之,这是非洲报道的最大系列儿童NS病例,显示出印度人的典型模式、黑人组织学类型的不寻常模式以及有色人种的中间情况。

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