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热带非洲的儿童肾病综合征:过去与现在。

Childhood nephrotic syndrome in tropical Africa: then and now.

作者信息

Olowu Wasiu A, Ademola Adebowale, Ajite Adebukola B, Saad Yauba M

机构信息

a Paediatric Nephrology and Hypertension Unit , Obafemi Awolowo University Teaching Hospitals Complex , Ile-Ife , Nigeria.

d Department of Paediatrics , University of Maiduguri/University of Maiduguri Teaching Hospital , Maiduguri , Nigeria.

出版信息

Paediatr Int Child Health. 2017 Nov;37(4):259-268. doi: 10.1080/20469047.2017.1374002. Epub 2017 Sep 26.

DOI:10.1080/20469047.2017.1374002
PMID:28949280
Abstract

This descriptive and comparative review examines the changing epidemiology, treatment, renal and patient outcome of childhood nephrotic syndrome (NS) in tropical Africa (TpAfr). In the 1960s to 1980s, corticosteroid-resistant non-minimal change disease (nMCD) including quartan malaria nephropathy (QMN) was the dominant renal histopathology type. The overall incidence of NS was 0.35-1.34% of hospital admissions. Median age at onset of NS ranged between 4.0 and 12.0 years while the mean (SD) age range was 5.8 (3.8) to 10.3 (4.8) years across studies. The male: female ratio was 1.6:1.0. The overall mean (SD) incidence of idiopathic minimal change disease (MCD) [21.6 (18.6%)] compared with idiopathic nMCD [59.1 (25.7%)] demonstrates significant dominance of the latter (p = 0.0001). Post-1989, the following mean (SD) incidences of histopathological types were: MCD 20.4 (17.7%), focal segmental glomerulosclerosis 39.0 (26.3%), membranoproliferative glomerulonephritis 25.4 (16.8%), proliferative glomerulonephritis 16.7 (27.0%) and membranous nephropathy 7.4 (4.5%). While the mean (SD) proportion of steroid resistance (SR) [73.5 (19.2%)] was significantly greater than the mean complete remission (CR) [26.5 (19.2%)] during 1960-1989 (p=0.005), mean (SD) SR [27.4 (25.3%)] was significantly lower than mean (SD) CR [66.1 (28.0%)] post-1989 (p < 0.001). Unlike QMN, hepatitis B virus, HIV infection, sickle cell disease and systemic lupus erythematosus are now increasingly being associated with NS in TpAfr. Mean (SD) renal survival post-1989 was 58.3 (37.0%) while all-cause mortality was 9.8%. Children with NS now survive better than before, reflecting improved access to healthcare and transition to a clinical pattern favouring idiopathic NS and increased sensitivity to corticosteroids.

摘要

本描述性和比较性综述探讨了热带非洲地区儿童肾病综合征(NS)流行病学、治疗方法、肾脏及患者预后的变化情况。在20世纪60年代至80年代,包括四日疟肾病(QMN)在内的皮质类固醇抵抗性非微小病变疾病(nMCD)是主要的肾脏组织病理学类型。NS的总体发病率占住院人数的0.35 - 1.34%。NS发病的中位年龄在4.0至12.0岁之间,而各研究中平均(标准差)年龄范围为5.8(3.8)至10.3(4.8)岁。男女比例为1.6:1.0。特发性微小病变疾病(MCD)的总体平均(标准差)发病率[21.6(18.6%)]与特发性nMCD[59.1(25.7%)]相比,显示出后者的显著优势(p = 0.0001)。1989年后,各组织病理学类型的平均(标准差)发病率如下:MCD为20.4(17.7%),局灶节段性肾小球硬化为39.0(26.3%),膜增生性肾小球肾炎为25.4(16.8%),增生性肾小球肾炎为16.7(27.0%),膜性肾病为7.4(4.5%)。虽然1960 - 1989年期间类固醇抵抗(SR)的平均(标准差)比例[73.5(19.2%)]显著高于完全缓解(CR)的平均比例[26.5(19.2%)](p = 0.005),但1989年后SR的平均(标准差)比例[27.4(25.3%)]显著低于CR的平均(标准差)比例[66.1(28.0%)](p < 0.001)。与QMN不同,乙型肝炎病毒、HIV感染、镰状细胞病和系统性红斑狼疮现在在热带非洲地区与NS的关联日益增加。1989年后的平均(标准差)肾脏生存率为58.3(37.0%),全因死亡率为9.8%。患有NS的儿童现在比以前存活得更好,这反映出获得医疗保健的机会有所改善,并且临床模式已转变为有利于特发性NS,同时对皮质类固醇的敏感性增加。

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