van den Brand M, Elving L D, Drenth J P H, van Krieken J H J M
Departments of Pathology, Radboud University Nijmegen Medical Centre Nijmegen, the Netherlands.
Neth J Med. 2009 Dec;67(11):394-6.
Glycogenic hepatopathy (GH) is a rare cause of serum transaminase elevations in type 1 diabetes mellitus (DM). We describe a 29-year-old woman with a history of poorly controlled type 1 DM who presented with hepatomegaly and severe transaminase flares. Liver histology confirmed GH, with glycogen accumulation due to severe fluctuations in both glucose and insulin. GH can be regarded as an adult variant of Mauriac's syndrome. Despite severe laboratory abnormalities, it does not cause liver cirrhosis. Treatment consists of improving glycaemic control.
糖原性肝病（GH）是1型糖尿病（DM）患者血清转氨酶升高的罕见原因。我们描述了一名29岁的1型糖尿病女性患者，其血糖控制不佳，出现肝肿大和严重的转氨酶激增。肝脏组织学检查确诊为GH，因血糖和胰岛素的剧烈波动导致糖原积累。GH可被视为Mauriac综合征的成人变异型。尽管实验室检查结果严重异常，但它不会导致肝硬化。治疗方法包括改善血糖控制。
