Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II

Frontiers in Pediatrics

article

Xue He¹  Yueling Zhu²  Haidong Fu¹  Chunyue Feng¹  Zhixia Liu¹  Weizhong Gu³  Yanyan Jin¹  Binbin Yang⁴  Huijun Shen¹ 

[1] Department of Nephrology, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine;Department of Traditional Chinese Medicine, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine;Department of Pathology, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine;Department of General Surgery, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine

关键词: Abernethy malformation;    congenital extrahepatic portosystemic shunt;    membranoproliferative glomerulonephritis;    arachnoid cyst;    focal nodular hyperplasia;   

DOI  :  10.3389/fped.2021.647364

学科分类：社会科学、人文和艺术（综合）

来源: Frontiers

【 摘 要 】

This report describes an 8-year-old male who presented with clinical manifestations including systemic edema, heavy proteinuria, hypoproteinemia, and persistent hypocomplementemia. Arachnoid cysts and focal nodular hyperplasia were also detected. Imaging examination and renal biopsy were performed, and Abernethy malformation type II with immune complex-mediated membranoproliferative glomerulonephritis was considered the diagnosis. Due to the persistence of embryonic vessels, Abernethy malformation is a rare congenital vascular malformation of the splanchnic venous system, which can be classified as type I (end-to-side shunt) and type II (side-to-side shunt). Abernethy malformation with glomerulonephritis remains extremely rare. In the patient described, glomerulonephritis mediated by immune complex with “full-house” positive immunohistochemistry was confirmed on renal biopsy. In addition, he was treated with glucocorticoids and tacrolimus. Whether surgical treatment is necessary should be determined according to the state of the disease in the later stages. The present case reflects the association between the congenital portosystemic shunt and the renal region and, to the authors' knowledge, may be the first report to describe arachnoid cysts as a symptom of Abernethy malformation.

【 授权许可】

CC BY