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II型阿伯内西畸形与肾病综合征的罕见表现:病例报告及文献复习

A rare presentation of type II Abernethy malformation and nephrotic syndrome: Case report and review.

作者信息

Wu Xin, Gu Weizhong, Lin Yongzhi, Ye Lina

机构信息

Department of Pediatrics, Taizhou Central Hospital (Taizhou University Hospital), Taizhou 318001, China.

Department of Pathology, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310005, China.

出版信息

Open Life Sci. 2022 Jul 21;17(1):794-799. doi: 10.1515/biol-2022-0086. eCollection 2022.

Abstract

Type II Abernethy malformation is an extremely reported congenital extrahepatic portosystemic shunt in complication with nephrotic syndrome. We present the case of an 8-year-old boy who presented with symptoms of type II Abernethy malformation and nephrotic syndrome. This diagnosis of this type II Abernethy malformation was based on physical examination, blood tests, urinalysis, nephrotic and hepatic function tests, routine clinical lipid measurements, abdominal ultrasonography, and computed tomographic angiography. A kidney biopsy revealed the pathological features of nephrotic syndrome. This is the second reported patient diagnosed with type II Abernethy malformation and nephrotic syndrome. Captopril treatment was effective in improving the symptoms of this case. A patient with type II Abernethy malformation related to immune complex-mediated glomerular injury was effectively improved with medication. Type II Abernethy malformation is a causative factor of immune complex-mediated glomerular injury in nephrotic syndrome. Captopril treatment significantly improved the symptoms in this case.

摘要

II型阿伯内西畸形是一种极为罕见的先天性肝外门体分流,常并发肾病综合征。我们报告了一例8岁男孩,他表现出II型阿伯内西畸形和肾病综合征的症状。该II型阿伯内西畸形的诊断基于体格检查、血液检查、尿液分析、肾病和肝功能检查、常规临床血脂测量、腹部超声以及计算机断层血管造影。肾活检揭示了肾病综合征的病理特征。这是第二例被诊断为II型阿伯内西畸形和肾病综合征的患者。卡托普利治疗有效改善了该病例的症状。一名与免疫复合物介导的肾小球损伤相关的II型阿伯内西畸形患者通过药物治疗得到有效改善。II型阿伯内西畸形是肾病综合征中免疫复合物介导的肾小球损伤的一个致病因素。卡托普利治疗显著改善了该病例的症状。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/094b/9319661/7bea8c2d32ad/j_biol-2022-0086-fig001.jpg

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