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[小儿IgA肾病]

[IgA nephropathy in pediatrics].

作者信息

Marinaki M, Benini D, Fasoli E, Fanos V

机构信息

Ospedale E. Venizelou, Heraklion, Creta.

出版信息

Pediatr Med Chir. 2003 Nov-Dec;25(6):409-14.

Abstract

IgA nephropathy is a primitive cronic idiopatic glomerulonephritis, characterized by diffuse depositis of IgA in the glomeruler mesangium. Familial cases are also descripted. IgA nephropaty is more frequent in males and in white rase. In Italy it's the most frequently recognized glomerulonephritis in renal biopsia (20%), especially in patients with dismorfic micro or macroematuria and nephrotic proteinuria. Clinical presentation is often in association with respiratory tract or gastrointestinal disorders. The most relevant pathogenetic hypothesis suggest an IgA abnormal glycosilation, with mesangial IgA aggregation, increased mesangial reactivity and release of inflammatory mediators and fibrotic agents. Treatment is considered in rapidly progressing forms. At the present, there is no treatment of proven value in all patients, althoug interesting results have been published with prednison, ACE-inhibitors or fish-oil in decresing renal deterioration rate. Natural history varies in different series. Renal survival at 10 years is 85% in Italy, 94% in France, 97% in the USA. Poor prognostic factor are heavy proteinuria and hypertension. However a wide inter-individual variability is observed.

摘要

IgA肾病是一种原发性慢性特发性肾小球肾炎,其特征是IgA在肾小球系膜中弥漫性沉积。也有家族性病例的描述。IgA肾病在男性和白种人中更为常见。在意大利,它是肾活检中最常被识别的肾小球肾炎(20%),尤其是在有畸形镜下或肉眼血尿及肾病性蛋白尿的患者中。临床表现常与呼吸道或胃肠道疾病相关。最相关的发病机制假说认为是IgA糖基化异常,伴有系膜IgA聚集、系膜反应性增加以及炎症介质和纤维化因子的释放。对于快速进展型病例会考虑进行治疗。目前,尚无对所有患者都有确凿疗效的治疗方法,不过已发表有趣的结果表明,泼尼松、血管紧张素转换酶抑制剂或鱼油在降低肾脏恶化率方面有一定作用。不同系列研究中其自然病程有所不同。在意大利,10年肾脏存活率为85%,法国为94%,美国为97%。不良预后因素是大量蛋白尿和高血压。然而,个体间存在很大差异。

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