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在印度北部,原发性 IgA 肾病:它是否有所不同?

Primary IgA nephropathy in north India: is it different?

机构信息

Department of Histopathology, Post-graduate Institute of Medical Education & Research, Chandigarh, India.

出版信息

Postgrad Med J. 2012 Jan;88(1035):15-20. doi: 10.1136/postgradmedj-2011-130077. Epub 2011 Nov 3.

DOI:10.1136/postgradmedj-2011-130077
PMID:22052882
Abstract

BACKGROUND

Immunoglobulin A (IgA) nephropathy is the most common glomerulonephritis worldwide, but has a variable geographic distribution. the bulk of the disease burden is borne by asian countries. however, its exact prevalence or clinicopathologic spectrum in india is not well documented.

METHODS

This cross sectional study analysed the renal biopsy findings and clinical features at presentation in 66 patients of primary IgA nephropathy diagnosed over a period of 2 years (2007-2008). The results were compared with studies from other centres in the country and elsewhere.

RESULTS

IgA nephropathy comprised 8.1% of all native kidney biopsies. The mean age of the patients was 29.9 years with a male:female ratio of 4.4:1. Most patients presented with renal failure and a significant percentage (23%) also had nephrotic range proteinuria. Renal biopsies were classified by the Haas classification and were further scored by the MEST scoring system of the Oxford classification. By Haas classification, 41 cases (62%) showed advanced sclerotic lesions of class V. Active crescents (cellular or fibrocellular) were seen in 42% of cases, and 26% of cases showed endocapillary proliferation. Serum creatinine values were highest in the presence of proliferative lesions. MEST scoring of the Oxford classification was not applicable in approximately 18% of cases because of the presence of advanced sclerotic lesions. On immunofluorescence, the majority of the cases showed both mesangial and membranous positivity for IgA antisera. Electron microscopy revealed para-mesangial location of immune complex deposition in the majority of the cases. It also showed glomerular basement membrane abnormalities in two cases.

CONCLUSION

Comparison of clinical and pathological features revealed that this disease presents as an advanced disease in much younger individuals in this study compared to other studies. Elucidation of the underlying factors may have immense therapeutic implications.

摘要

背景

免疫球蛋白 A(IgA)肾病是世界上最常见的肾小球肾炎,但分布具有地域差异。该病的主要负担由亚洲国家承担。然而,其在印度的确切流行率或临床病理谱尚未得到很好的记录。

方法

本横断面研究分析了在 2 年期间(2007-2008 年)诊断的 66 例原发性 IgA 肾病患者的肾活检结果和首发时的临床特征。将结果与来自该国其他中心和其他地区的研究进行比较。

结果

IgA 肾病占所有原发性肾活检的 8.1%。患者的平均年龄为 29.9 岁,男女比例为 4.4:1。大多数患者表现为肾功能衰竭,有相当比例(23%)的患者出现肾病范围蛋白尿。肾活检按 Haas 分类进行分类,并按牛津分类的 MEST 评分系统进一步评分。根据 Haas 分类,41 例(62%)显示 V 级硬化病变。42%的病例存在活跃的新月体(细胞性或纤维细胞性),26%的病例存在毛细血管内增殖。存在增生性病变时血清肌酐值最高。由于存在晚期硬化病变,约 18%的病例无法应用牛津分类的 MEST 评分。在免疫荧光检查中,大多数病例的 IgA 抗血清均显示系膜和膜阳性。电子显微镜检查显示,大多数病例的免疫复合物沉积位于系膜旁。在两个病例中还显示了肾小球基底膜异常。

结论

临床和病理特征的比较表明,与其他研究相比,该疾病在本研究中表现为更年轻患者的晚期疾病。阐明潜在因素可能具有重要的治疗意义。

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