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[委内瑞拉IgA肾病患儿的临床和病理特征]

[Clinical and pathological features in Venezuelan children with IgA nephropathy].

作者信息

Coronel Valerio, Mora-Orta Sioly, Domínguez Luis, Corado José, Zibaoui Patrícia I, Bethencourt Sarah, Weffer María, Pinto Jose A, Lara Elsa, Orta Nelson

机构信息

Servicio de Nefrología Pediátrica, Hospital de Niños y Universidad de Carabobo, Valencia, Venezuela.

出版信息

Invest Clin. 2007 Jun;48(2):167-74.

Abstract

IgA nephropathy (IgAN) is the most common glomerulonephritis in humans worldwide; its prevalence and prognosis vary according with geographical areas. The incidence is higher in adults under 30 years of age and in children, it occurs more frequently in patients between 3 and 10 years. Hematuria is the predominant manifestation at presentation of the disease and 20-40% of the cases progress to terminal chronic renal disease. Renal biopsies were performed in 426 children during the period 1980-2002, of them, 12 cases corresponded to IgAN. The clinico-pathological characteristics and evolution of patients were evaluated during an average of 3.85 years. Mean age of patients was 6.2 years, and it was more frequent in males. Hematuria and proteinuria were found in 100% of cases and proteinuria of nephrotic range in 75%. Hypertriglyceridemia and hypercholesterolemia in 91%, arterial hypertension in 50% and acute renal failure at presentation in 25%. The predominant histopathological patterns (WHO) were II and III, deposits of mesangial IgA, IgG and C3 were observed in all cases and C4 deposits in 25%. 41.7% of cases had complete remission, 41.7% maintained normal renal function with persistent proteinuria and 16% progressed to terminal chronic renal failure. The actuarial survival of patients was 100% at 3 years, 87% at 4 years and 76% at 8 years. Two patients died during the period of study, at 3.5 and 8.5 years. The variability of presentation of IgA nephropathy was confirmed in this study, which could be attributable to geographical differences, racial influences and clinicopathological features related to sanitary conditions. Despite of the frequency of bad prognosis characteristics at presentation of IgAN in our series, the evolution was similar to reports of other groups.

摘要

IgA肾病(IgAN)是全球人类中最常见的肾小球肾炎;其患病率和预后因地理区域而异。30岁以下成年人及儿童的发病率较高,在3至10岁患者中更为常见。血尿是该疾病初发时的主要表现,20% - 40%的病例会进展为终末期慢性肾病。1980年至2002年期间,对426名儿童进行了肾活检,其中12例为IgA肾病。对患者的临床病理特征及病情演变进行了平均3.85年的评估。患者平均年龄为6.2岁,男性更为常见。100%的病例出现血尿和蛋白尿,75%的病例出现肾病范围的蛋白尿。91%的病例出现高甘油三酯血症和高胆固醇血症,50%的病例出现动脉高血压,25%的病例初发时出现急性肾衰竭。主要组织病理学类型(世界卫生组织分类)为II型和III型,所有病例均观察到系膜IgA、IgG和C3沉积,25%的病例有C4沉积。41.7%的病例完全缓解,41.7%的病例肾功能正常但持续存在蛋白尿,16%的病例进展为终末期慢性肾衰竭。患者的精算生存率在3年时为100%,4年时为87%,8年时为76%。在研究期间,两名患者分别在3.5年和8.5年时死亡。本研究证实了IgA肾病临床表现的变异性,这可能归因于地理差异、种族影响以及与卫生条件相关的临床病理特征。尽管在我们的系列研究中IgA肾病初发时不良预后特征较为常见,但其病情演变与其他组的报告相似。

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