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Rare complication of inflammatory bowel disease-like colitis from glycogen storage disease type 1b and its surgical management: A case report
BACKGROUND: Glycogen storage disease (GSD) is an autosomal recessive inborn metabolic disorder. Patients with GSD are prone to hypoglycaemia, hyperlactacidemia and bleeding. GSD type 1b (GSD-1b) patients specifically can develop neutropenia, recurrent bacterial infection and inflammatory bowel disea...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Baishideng Publishing Group Inc
2021
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8180209/ https://www.ncbi.nlm.nih.gov/pubmed/34141769 http://dx.doi.org/10.12998/wjcc.v9.i16.4081 |
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author | Lui, Frederick Chi-Wai Lo, Oswens Siu-Hung |
author_facet | Lui, Frederick Chi-Wai Lo, Oswens Siu-Hung |
author_sort | Lui, Frederick Chi-Wai |
collection | PubMed |
description | BACKGROUND: Glycogen storage disease (GSD) is an autosomal recessive inborn metabolic disorder. Patients with GSD are prone to hypoglycaemia, hyperlactacidemia and bleeding. GSD type 1b (GSD-1b) patients specifically can develop neutropenia, recurrent bacterial infection and inflammatory bowel disease (IBD). Documentation of the long-term outcomes of surgical management of GSD-1b has been scarce, especially for Asian patients. We herein describe a case of GSD-1b complicated by IBD-like colitis and coloduodenal fistula. The patient was managed successfully with surgical intervention. CASE SUMMARY: A 20-year-old Chinese lady confirmed by genetic testing to have GSD-1b was initially managed with uncooked cornstarch and granulocyte-colony stimulating factor. With recurrent abdominal symptoms, her condition was treated as clinical “Crohn’s disease” with mesalazine, prednisolone and azathioprine conservatively. Colonoscopy showed a tight stricture at the hepatic flexure. Subsequent computerized tomographic colonography revealed a phlegmon at the ileocaecal region with a suspected coloduodenal fistula. Eventually an exploratory laparotomy was performed and severe colitis at the ascending colon with coloduodenal fistula was confirmed. Right hemicolectomy with primary anastomosis and repair of the duodenum were performed. Surgical management of complications from GSD-1b associated IBD-like colitis has rarely been described. First-line treatment would usually be conservative. Surgical intervention like hemicolectomy is mainly reserved for refractory cases. CONCLUSION: Surgical management of coloduodenal fistula in GSD-1b patients is a feasible and safe option when failed conservative management. |
format | Online Article Text |
id | pubmed-8180209 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Baishideng Publishing Group Inc |
record_format | MEDLINE/PubMed |
spelling | pubmed-81802092021-06-16 Rare complication of inflammatory bowel disease-like colitis from glycogen storage disease type 1b and its surgical management: A case report Lui, Frederick Chi-Wai Lo, Oswens Siu-Hung World J Clin Cases Case Report BACKGROUND: Glycogen storage disease (GSD) is an autosomal recessive inborn metabolic disorder. Patients with GSD are prone to hypoglycaemia, hyperlactacidemia and bleeding. GSD type 1b (GSD-1b) patients specifically can develop neutropenia, recurrent bacterial infection and inflammatory bowel disease (IBD). Documentation of the long-term outcomes of surgical management of GSD-1b has been scarce, especially for Asian patients. We herein describe a case of GSD-1b complicated by IBD-like colitis and coloduodenal fistula. The patient was managed successfully with surgical intervention. CASE SUMMARY: A 20-year-old Chinese lady confirmed by genetic testing to have GSD-1b was initially managed with uncooked cornstarch and granulocyte-colony stimulating factor. With recurrent abdominal symptoms, her condition was treated as clinical “Crohn’s disease” with mesalazine, prednisolone and azathioprine conservatively. Colonoscopy showed a tight stricture at the hepatic flexure. Subsequent computerized tomographic colonography revealed a phlegmon at the ileocaecal region with a suspected coloduodenal fistula. Eventually an exploratory laparotomy was performed and severe colitis at the ascending colon with coloduodenal fistula was confirmed. Right hemicolectomy with primary anastomosis and repair of the duodenum were performed. Surgical management of complications from GSD-1b associated IBD-like colitis has rarely been described. First-line treatment would usually be conservative. Surgical intervention like hemicolectomy is mainly reserved for refractory cases. CONCLUSION: Surgical management of coloduodenal fistula in GSD-1b patients is a feasible and safe option when failed conservative management. Baishideng Publishing Group Inc 2021-06-06 2021-06-06 /pmc/articles/PMC8180209/ /pubmed/34141769 http://dx.doi.org/10.12998/wjcc.v9.i16.4081 Text en ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved. https://creativecommons.org/licenses/by-nc/4.0/This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. |
spellingShingle | Case Report Lui, Frederick Chi-Wai Lo, Oswens Siu-Hung Rare complication of inflammatory bowel disease-like colitis from glycogen storage disease type 1b and its surgical management: A case report |
title | Rare complication of inflammatory bowel disease-like colitis from glycogen storage disease type 1b and its surgical management: A case report |
title_full | Rare complication of inflammatory bowel disease-like colitis from glycogen storage disease type 1b and its surgical management: A case report |
title_fullStr | Rare complication of inflammatory bowel disease-like colitis from glycogen storage disease type 1b and its surgical management: A case report |
title_full_unstemmed | Rare complication of inflammatory bowel disease-like colitis from glycogen storage disease type 1b and its surgical management: A case report |
title_short | Rare complication of inflammatory bowel disease-like colitis from glycogen storage disease type 1b and its surgical management: A case report |
title_sort | rare complication of inflammatory bowel disease-like colitis from glycogen storage disease type 1b and its surgical management: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8180209/ https://www.ncbi.nlm.nih.gov/pubmed/34141769 http://dx.doi.org/10.12998/wjcc.v9.i16.4081 |
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