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非洲儿童的肾病综合征:“热带肾病综合征”缺乏证据?

Nephrotic syndrome in African children: lack of evidence for 'tropical nephrotic syndrome'?

作者信息

Doe James Yao, Funk Matthias, Mengel Michael, Doehring Ekkehard, Ehrich Jochen H H

机构信息

Battor Hospital, Pediatrics, PO Bx 25, Jirapa, Ghana.

出版信息

Nephrol Dial Transplant. 2006 Mar;21(3):672-6. doi: 10.1093/ndt/gfi297. Epub 2005 Dec 2.

Abstract

BACKGROUND

Infections such as malaria, schistosomiasis, hepatitis B and HIV have been suggested as major causes of the nephrotic syndrome (NS) in African children. We retrospectively analysed the course of the NS in 32 children from Ghana and reviewed the literature on NS from 18 different African countries for the presence of 'the tropical nephrotic syndrome'.

METHODS

Thirty-two children (22 boys, 10 girls, median age 12 years, range 1-18 years) with NS were treated from 2000-2003 at Battor Hospital, Ghana. Thirteen out of 32 children underwent a renal biopsy which was investigated by light, immune and electron microscopy. All 32 patients were initially treated with oral prednisone (PRED) therapy (29 with standard therapy for 8 weeks and three individually tailored), and steroid-resistant children received also intravenous methylprednisolone pulses (three children) or oral cyclophosphamide (two children).

RESULTS

All patients fulfilled the clinical and laboratory criteria of a NS. The initial median serum creatinine was 65 micromol/l (range 44-133 micromol/l). Renal biopsy was performed in 13/32 children and revealed focal and segmental glomerulosclerosis (FSGS) in 10 patients, minimal change disease (MCNs) in two and no conclusive result in one patient. Glomerular immune complex deposition was absent in all biopsies. After treatment with PRED, oedema disappeared in 24/32 patients; however, proteinuria normalized in 16/32 patients only. The NS relapsed in 9/16 steroid-sensitive patients after cessation of PRED therapy, and two children were frequent relapsers. The steroid-resistant NS did not respond to an intensified immunosuppression in 5/16 children receiving methylprednisolone or cyclophosphamide. Five out of 32 children died, all were steroid resistant.

CONCLUSIONS

There was no evidence for a dominating role of steroid-resistant 'tropical glomerulopathies' in children with a NS in Ghana. Similar to South Africa, focal and segmental glomerulosclerosis (FSGS) and minimal change disease were the most frequent findings on histology. Contrary to Nigeria, membrano-proliferative glomerulonephritis was not found in these patients. We conclude from this data and from the literature that the histological pattern of NS may vary between different African countries. Concerning therapy of NS under tropical conditions, we emphasize that despite the limited therapeutic facilities half of these patients may benefit from corticosteroids; however, steroid resistance and FSGS resulted in a high mortality.

摘要

背景

疟疾、血吸虫病、乙型肝炎和艾滋病毒等感染被认为是非洲儿童肾病综合征(NS)的主要病因。我们回顾性分析了32名加纳儿童的肾病综合征病程,并查阅了来自18个不同非洲国家关于肾病综合征的文献,以寻找“热带肾病综合征”的存在情况。

方法

2000年至2003年期间,在加纳的巴托尔医院对32名肾病综合征患儿(22名男孩,10名女孩,中位年龄12岁,年龄范围1至18岁)进行了治疗。32名儿童中有13名接受了肾活检,通过光镜、免疫和电镜进行检查。所有32例患者最初均接受口服泼尼松(PRED)治疗(29例采用标准治疗8周,3例个体化治疗),激素抵抗患儿还接受了静脉注射甲泼尼龙冲击治疗(3例患儿)或口服环磷酰胺(2例患儿)。

结果

所有患者均符合肾病综合征的临床和实验室标准。初始血清肌酐中位数为65微摩尔/升(范围44至133微摩尔/升)。13/32名儿童进行了肾活检,其中10例显示局灶节段性肾小球硬化(FSGS),2例为微小病变病(MCNs),1例未得出明确结果。所有活检均未发现肾小球免疫复合物沉积。经PRED治疗后,24/32例患者水肿消失;然而,仅16/32例患者蛋白尿恢复正常。16例激素敏感患者中,9例在停用PRED治疗后肾病综合征复发,2例患儿为频繁复发者。在接受甲泼尼龙或环磷酰胺治疗的16例儿童中,5例激素抵抗性肾病综合征对强化免疫抑制无反应。32名儿童中有5例死亡,均为激素抵抗者。

结论

在加纳患有肾病综合征的儿童中,没有证据表明激素抵抗性“热带肾小球病”起主导作用。与南非类似,局灶节段性肾小球硬化(FSGS)和微小病变病是组织学上最常见的发现。与尼日利亚不同,这些患者中未发现膜增生性肾小球肾炎。根据这些数据和文献,我们得出结论,肾病综合征的组织学模式在不同非洲国家可能有所不同。关于热带条件下肾病综合征的治疗,我们强调,尽管治疗设施有限,但这些患者中有一半可能从皮质类固醇中获益;然而,激素抵抗和FSGS导致了高死亡率。

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