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1型糖尿病控制不佳患者的局灶性肝糖原贮积症

Focal Hepatic Glycogenosis in a Patient With Uncontrolled Diabetes Mellitus Type 1.

作者信息

Glushko Tetiana, Kushchayev Sergiy V, Trifanov Dmitry, Salei Aliaksei, Morales Diego, Berry Gerard, Mackey Justin, Teytelboym Oleg M

出版信息

J Comput Assist Tomogr. 2018 Mar/Apr;42(2):230-235. doi: 10.1097/RCT.0000000000000673.

DOI:10.1097/RCT.0000000000000673
PMID:28937487
Abstract

Hepatomegaly and elevated liver enzymes in patients with diabetes are commonly associated with fatty liver disease. However, physicians often forget about another intrinsic substance that can cause a similar clinical picture-glycogen. Liver stores approximately one third of the total body glycogen and is responsible for blood glucose homeostasis. Excessive hepatocellular glycogen accumulation occurs not only in congenital glycogen storage diseases, but also in acquired conditions associated with hyperglycemic-hyperinsulinemic states such as uncontrolled diabetes mellitus, high-dose corticosteroid use, and dumping syndrome. All reported cases of acquired abnormal glycogen deposition described a diffuse form of hepatic glycogenosis with the entire liver involved in the accumulating process. To our knowledge, this is the first reported case of abnormal focal glycogen deposition in a patient with diabetes mellitus type 1 with imaging and pathologic correlation. Awareness of the imaging appearance of focal glycogen deposition can help to distinguish it from other pathologic conditions.

摘要

糖尿病患者出现肝肿大和肝酶升高通常与脂肪肝疾病有关。然而,医生常常忽略另一种可导致类似临床表现的内在物质——糖原。肝脏储存了全身约三分之一的糖原,并负责血糖稳态。肝细胞糖原过度积累不仅发生在先天性糖原贮积病中,也发生在与高血糖 - 高胰岛素血症状态相关的后天性疾病中,如未控制的糖尿病、大剂量使用皮质类固醇以及倾倒综合征。所有报道的后天性异常糖原沉积病例均描述为弥漫性肝糖原沉着症,整个肝脏都参与了积累过程。据我们所知,这是首例通过影像学和病理学相关性报道的1型糖尿病患者局灶性异常糖原沉积病例。认识局灶性糖原沉积的影像学表现有助于将其与其他病理状况区分开来。

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Focal Hepatic Glycogenosis in a Patient With Uncontrolled Diabetes Mellitus Type 1.1型糖尿病控制不佳患者的局灶性肝糖原贮积症
J Comput Assist Tomogr. 2018 Mar/Apr;42(2):230-235. doi: 10.1097/RCT.0000000000000673.
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Hepatomegaly and abnormal liver tests due to glycogenosis in adults with diabetes.糖尿病成人患者因糖原贮积症导致肝肿大及肝功能检查异常。
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Hepatic glycogenosis: a rare cause of hepatomegaly in Type 1 diabetes mellitus.肝糖原累积症:1型糖尿病患者肝肿大的罕见病因。
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引用本文的文献

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Type 1 diabetes and combined acute and chronic complications are associated with risk of progression of liver fibrosis: a Mendelian randomization study.1 型糖尿病及其合并的急慢性并发症与肝纤维化进展风险相关:一项孟德尔随机研究。
Front Endocrinol (Lausanne). 2024 Aug 5;15:1302611. doi: 10.3389/fendo.2024.1302611. eCollection 2024.
2
Hepatocellular Ballooning is Due to Highly Pronounced Glycogenosis Potentially Associated with Steatosis and Metabolic Reprogramming.肝细胞气球样变归因于高度明显的糖原贮积症,可能与脂肪变性和代谢重编程有关。
J Clin Transl Hepatol. 2024 Jan 28;12(1):52-61. doi: 10.14218/JCTH.2023.00242. Epub 2023 Oct 19.
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Hepatopathy Associated With Type 1 Diabetes: Distinguishing Non-alcoholic Fatty Liver Disease From Glycogenic Hepatopathy.
1型糖尿病相关肝病:非酒精性脂肪性肝病与糖原性肝病的鉴别
Front Pharmacol. 2021 Oct 25;12:768576. doi: 10.3389/fphar.2021.768576. eCollection 2021.