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IgA肾病合并威尔逊病:一例报告及文献综述

IgA Nephropathy with Wilson's Disease: A Case Report and Literature Review.

作者信息

Bhandari Gaurav, Tiwari Vaibhav, Gupta Anurag, Gupta Pallav, Bhargava Vinant, Malik Manish, Gupta Ashwani, Bhalla Anil K, Rana Devinder S

机构信息

Department of Nephrology, Sir Ganga Ram Hospital, New Delhi, India.

Department of Pathology, Sir Ganga Ram Hospital, New Delhi, India.

出版信息

Indian J Nephrol. 2021 Sep-Oct;31(5):474-477. doi: 10.4103/ijn.IJN_227_20. Epub 2021 Feb 20.

Abstract

The most common glomerulonephritis seen in the world is immunoglobulin A nephropathy (IgAN). It can be primary or secondary associated with various conditions like Chronic Liver disease, Crohn's disease, neoplasms, etc. However, IgAN secondary to Wilson's disease is very rare. A 9 year old boy presented with gross hematuria and proteinuria. He had a history of recurrent jaundice in the past. Ultrasonography (USG) whole abdomen showed altered echotexture of the liver with normal-sized kidneys. An extended workup for liver disease was done, and the diagnosis of Wilson's disease was confirmed with decreased serum ceruloplasmin levels, increased urinary copper, and the Kayser-Fleischer ring. Urine routine microscopy showed numerous red blood cells, few red blood cell casts, and mild proteinuria. Renal biopsy showed IgAN. The patient was started on D-penicillamine. On follow-up at 3 months, he showed complete resolution of proteinuria and hematuria. Thus, we suggest that Wilson's disease should be considered as one of the causes of secondary IgAN in pediatric patients with hematuria, proteinuria with liver dysfunction.

摘要

世界上最常见的肾小球肾炎是免疫球蛋白A肾病(IgAN)。它可以是原发性的,也可以是继发于各种疾病,如慢性肝病、克罗恩病、肿瘤等。然而,继发于威尔逊病的IgAN非常罕见。一名9岁男孩出现肉眼血尿和蛋白尿。他过去有反复黄疸病史。全腹超声检查显示肝脏回声纹理改变,肾脏大小正常。对肝脏疾病进行了全面检查,血清铜蓝蛋白水平降低、尿铜增加以及出现凯-弗环,确诊为威尔逊病。尿常规显微镜检查显示大量红细胞、少量红细胞管型和轻度蛋白尿。肾活检显示为IgAN。患者开始使用青霉胺治疗。在3个月的随访中,他的蛋白尿和血尿完全消失。因此,我们建议,对于有血尿、蛋白尿且肝功能异常的儿科患者,应将威尔逊病视为继发性IgAN的病因之一。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/64ef/8597791/2c7745754713/IJN-31-474-g001.jpg

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