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

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,...

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Autores principales: He, Xue, Zhu, Yueling, Fu, Haidong, Feng, Chunyue, Liu, Zhixia, Gu, Weizhong, Jin, Yanyan, Yang, Binbin, Shen, Huijun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8010253/
https://www.ncbi.nlm.nih.gov/pubmed/33816407
http://dx.doi.org/10.3389/fped.2021.647364
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author He, Xue
Zhu, Yueling
Fu, Haidong
Feng, Chunyue
Liu, Zhixia
Gu, Weizhong
Jin, Yanyan
Yang, Binbin
Shen, Huijun
author_facet He, Xue
Zhu, Yueling
Fu, Haidong
Feng, Chunyue
Liu, Zhixia
Gu, Weizhong
Jin, Yanyan
Yang, Binbin
Shen, Huijun
author_sort He, Xue
collection PubMed
description 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.
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spelling pubmed-80102532021-04-01 Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II He, Xue Zhu, Yueling Fu, Haidong Feng, Chunyue Liu, Zhixia Gu, Weizhong Jin, Yanyan Yang, Binbin Shen, Huijun Front Pediatr Pediatrics 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. Frontiers Media S.A. 2021-03-17 /pmc/articles/PMC8010253/ /pubmed/33816407 http://dx.doi.org/10.3389/fped.2021.647364 Text en Copyright © 2021 He, Zhu, Fu, Feng, Liu, Gu, Jin, Yang and Shen. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Pediatrics
He, Xue
Zhu, Yueling
Fu, Haidong
Feng, Chunyue
Liu, Zhixia
Gu, Weizhong
Jin, Yanyan
Yang, Binbin
Shen, Huijun
Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II
title Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II
title_full Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II
title_fullStr Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II
title_full_unstemmed Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II
title_short Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II
title_sort case report: membranoproliferative glomerulonephritis, a rare clinical manifestation of abernethy malformation type ii
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8010253/
https://www.ncbi.nlm.nih.gov/pubmed/33816407
http://dx.doi.org/10.3389/fped.2021.647364
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