Reversible severe glycogenic hepatopathy in type 1 diabetes

CASE PRESENTATION: We report a case of severe glycogenic hepatopathy in a 17-year-old boy with poorly controlled type 1 diabetes. On presentation, major findings included unexplained pronounced hepatomegaly and increased liver enzymes, ferritin, and triglycerides. Histology and electron microscopy e...

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Autores principales: de Laffolie, Jan, Kamrath, Clemens, Burchert, Diana, Böttcher, Claudia, Wudy, Stefan Alexander, Zimmer, Klaus-Peter
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Vienna 2021
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8975768/
https://www.ncbi.nlm.nih.gov/pubmed/33471221
http://dx.doi.org/10.1007/s10354-020-00795-6
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author de Laffolie, Jan
Kamrath, Clemens
Burchert, Diana
Böttcher, Claudia
Wudy, Stefan Alexander
Zimmer, Klaus-Peter
author_facet de Laffolie, Jan
Kamrath, Clemens
Burchert, Diana
Böttcher, Claudia
Wudy, Stefan Alexander
Zimmer, Klaus-Peter
author_sort de Laffolie, Jan
collection PubMed
description CASE PRESENTATION: We report a case of severe glycogenic hepatopathy in a 17-year-old boy with poorly controlled type 1 diabetes. On presentation, major findings included unexplained pronounced hepatomegaly and increased liver enzymes, ferritin, and triglycerides. Histology and electron microscopy evaluation showed severe glycogen storage, steatosis, and signs of fibrosis, resembling the histomorphological findings of Mauriac syndrome. After information about the nature of the disease and intensification of insulin therapy with insulin pump, liver enzymes, ferritin, and triglycerides normalized within 1 month. CONCLUSION: Glycogenic hepatopathy is a rare but important potential complication in poorly controlled juvenile diabetic patients. With improved metabolic control, it is fully reversible.
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spelling pubmed-89757682022-04-07 Reversible severe glycogenic hepatopathy in type 1 diabetes de Laffolie, Jan Kamrath, Clemens Burchert, Diana Böttcher, Claudia Wudy, Stefan Alexander Zimmer, Klaus-Peter Wien Med Wochenschr Case Report CASE PRESENTATION: We report a case of severe glycogenic hepatopathy in a 17-year-old boy with poorly controlled type 1 diabetes. On presentation, major findings included unexplained pronounced hepatomegaly and increased liver enzymes, ferritin, and triglycerides. Histology and electron microscopy evaluation showed severe glycogen storage, steatosis, and signs of fibrosis, resembling the histomorphological findings of Mauriac syndrome. After information about the nature of the disease and intensification of insulin therapy with insulin pump, liver enzymes, ferritin, and triglycerides normalized within 1 month. CONCLUSION: Glycogenic hepatopathy is a rare but important potential complication in poorly controlled juvenile diabetic patients. With improved metabolic control, it is fully reversible. Springer Vienna 2021-01-20 2022 /pmc/articles/PMC8975768/ /pubmed/33471221 http://dx.doi.org/10.1007/s10354-020-00795-6 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Case Report
de Laffolie, Jan
Kamrath, Clemens
Burchert, Diana
Böttcher, Claudia
Wudy, Stefan Alexander
Zimmer, Klaus-Peter
Reversible severe glycogenic hepatopathy in type 1 diabetes
title Reversible severe glycogenic hepatopathy in type 1 diabetes
title_full Reversible severe glycogenic hepatopathy in type 1 diabetes
title_fullStr Reversible severe glycogenic hepatopathy in type 1 diabetes
title_full_unstemmed Reversible severe glycogenic hepatopathy in type 1 diabetes
title_short Reversible severe glycogenic hepatopathy in type 1 diabetes
title_sort reversible severe glycogenic hepatopathy in type 1 diabetes
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8975768/
https://www.ncbi.nlm.nih.gov/pubmed/33471221
http://dx.doi.org/10.1007/s10354-020-00795-6
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