hepatic glycogenosis


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hepatic glycogenosis

[he′pad·ik ‚glī·kə·jə′nō·səs]
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Glycogenic hepatopathy was first described in 1930 by Mauriac as "hepatic glycogenosis, characterized by hepatic glycogen deposition in patients with poorly controlled type 1 diabetes mellitus" [1].
We diagnosed the patient as Mauriac syndrome with the findings of growth retardation, poorly controlled diabetes, hepatomegaly, and hepatic glycogenosis. Genetic analysis was performed to exclude congenital glycogen storage disease type-1 (GSD-1).
Long-term course of hepatic glycogenosis. A retrospective study of 76 cases.
Although hepatic glycogenosis is common in this population, hepatomegaly or elevated transaminase are more often attributed to steatosis than to glycogen excess, and so as glycogenosis may be considered a rare cause of these findings.