[Glycogenic hepatopathy/hepatic glycogenosis: an underappreciated but benign and reversible diabetic complication].

Almost 100 years ago, the French physician Pierre Mauriac described a syndrome named after him, resulting from poorly controlled type 1 diabetes, with growth retardation, delayed puberty, Cushingoid features and hepatomegaly. With modern diabetes care, this is very rare but does occur; however, despite the condition having important clinical implications and being easily treatable, this diabetes complication remains relatively unknown. We present here an authentic patient case in the form of a young man with glycemically poorly controlled type 1 diabetes who developed hepatomegaly, hyperlactatemia and histopathological changes in the liver consistent with glycogenosis, a state readily reversed by normalization of glycemia.

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Journal
PubMed
Published
2024-11-05
Primary Topic
Glycogen Storage Diseases and Myoclonus
Type
article
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article

[Glycogenic hepatopathy/hepatic glycogenosis: an underappreciated but benign and reversible diabetic complication].

Åke Sjöholm, Havraz Mahma
PubMed
Glycogen Storage Diseases and Myoclonus
article

[Glycogenic hepatopathy/hepatic glycogenosis: an underappreciated but benign and reversible diabetic complication].

Åke Sjöholm, Havraz Mahma
article en

Abstract

Almost 100 years ago, the French physician Pierre Mauriac described a syndrome named after him, resulting from poorly controlled type 1 diabetes, with growth retardation, delayed puberty, Cushingoid features and hepatomegaly. With modern diabetes care, this is very rare but does occur; however, despite the condition having important clinical implications and being easily treatable, this diabetes complication remains relatively unknown. We present here an authentic patient case in the form of a young man with glycemically poorly controlled type 1 diabetes who developed hepatomegaly, hyperlactatemia and histopathological changes in the liver consistent with glycogenosis, a state readily reversed by normalization of glycemia.

PubMedVol. 121
Gävle Hospital (SE), Endokrinologikum (DE)
Openalex Percentile: Top 64%
Glycogen Storage Diseases and Myoclonus
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