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Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome
Hepatic glycogenosis in type 1 diabetes mellitus (DM) can be caused by poor glycemic control due to insulin deficiency, excessive insulin treatment for diabetic ketoacidosis, or excessive glucose administration to control hypoglycemia. Mauriac syndrome, which is characterized by hepatomegaly due to...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Korean Pediatric Society
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4510358/ https://www.ncbi.nlm.nih.gov/pubmed/26213553 http://dx.doi.org/10.3345/kjp.2015.58.6.234 |
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author | Jung, In Ah Cho, Won Kyoung Jeon, Yeon Jin Kim, Shin Hee Cho, Kyoung Soon Park, So Hyun Jung, Min Ho Suh, Byung-Kyu |
author_facet | Jung, In Ah Cho, Won Kyoung Jeon, Yeon Jin Kim, Shin Hee Cho, Kyoung Soon Park, So Hyun Jung, Min Ho Suh, Byung-Kyu |
author_sort | Jung, In Ah |
collection | PubMed |
description | Hepatic glycogenosis in type 1 diabetes mellitus (DM) can be caused by poor glycemic control due to insulin deficiency, excessive insulin treatment for diabetic ketoacidosis, or excessive glucose administration to control hypoglycemia. Mauriac syndrome, which is characterized by hepatomegaly due to hepatic glycogenosis, growth retardation, delayed puberty, and Cushingoid features, is a rare diabetic complication. We report a case of hepatic glycogenosis mimicking Mauriac syndrome. A 14-year-old girl with poorly controlled type 1 DM was admitted to The Catholic University of Korea, Seoul St. Mary's Hospital for abdominal pain and distension. Physical examination revealed hepatomegaly and a Cushingoid face. The growth rate of the patient had decreased, and she had not yet experienced menarche. Laboratory findings revealed elevated liver enzyme levels. A liver biopsy confirmed hepatic glycogenosis. Continuous glucose monitoring showed hyperglycemia after meals and frequent hypoglycemia before meals. To control hyperglycemia, we increased insulin dosage by using an insulin pump. In addition, we prescribed uncooked cornstarch to prevent hypoglycemia. After strict blood glucose control, the patient's liver functions and size normalized. The patient subsequently underwent menarche. Hepatic glycogenosis is a complication of type 1 DM that is reversible with appropriate glycemic control. |
format | Online Article Text |
id | pubmed-4510358 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | The Korean Pediatric Society |
record_format | MEDLINE/PubMed |
spelling | pubmed-45103582015-07-24 Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome Jung, In Ah Cho, Won Kyoung Jeon, Yeon Jin Kim, Shin Hee Cho, Kyoung Soon Park, So Hyun Jung, Min Ho Suh, Byung-Kyu Korean J Pediatr Case Report Hepatic glycogenosis in type 1 diabetes mellitus (DM) can be caused by poor glycemic control due to insulin deficiency, excessive insulin treatment for diabetic ketoacidosis, or excessive glucose administration to control hypoglycemia. Mauriac syndrome, which is characterized by hepatomegaly due to hepatic glycogenosis, growth retardation, delayed puberty, and Cushingoid features, is a rare diabetic complication. We report a case of hepatic glycogenosis mimicking Mauriac syndrome. A 14-year-old girl with poorly controlled type 1 DM was admitted to The Catholic University of Korea, Seoul St. Mary's Hospital for abdominal pain and distension. Physical examination revealed hepatomegaly and a Cushingoid face. The growth rate of the patient had decreased, and she had not yet experienced menarche. Laboratory findings revealed elevated liver enzyme levels. A liver biopsy confirmed hepatic glycogenosis. Continuous glucose monitoring showed hyperglycemia after meals and frequent hypoglycemia before meals. To control hyperglycemia, we increased insulin dosage by using an insulin pump. In addition, we prescribed uncooked cornstarch to prevent hypoglycemia. After strict blood glucose control, the patient's liver functions and size normalized. The patient subsequently underwent menarche. Hepatic glycogenosis is a complication of type 1 DM that is reversible with appropriate glycemic control. The Korean Pediatric Society 2015-06 2015-06-22 /pmc/articles/PMC4510358/ /pubmed/26213553 http://dx.doi.org/10.3345/kjp.2015.58.6.234 Text en Copyright © 2015 by The Korean Pediatric Society http://creativecommons.org/licenses/by-nc/3.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Jung, In Ah Cho, Won Kyoung Jeon, Yeon Jin Kim, Shin Hee Cho, Kyoung Soon Park, So Hyun Jung, Min Ho Suh, Byung-Kyu Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome |
title | Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome |
title_full | Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome |
title_fullStr | Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome |
title_full_unstemmed | Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome |
title_short | Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome |
title_sort | hepatic glycogenosis in type 1 diabetes mellitus mimicking mauriac syndrome |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4510358/ https://www.ncbi.nlm.nih.gov/pubmed/26213553 http://dx.doi.org/10.3345/kjp.2015.58.6.234 |
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