High childhood serum triglyceride concentrations associate with hepatocellular adenoma development in patients with glycogen storage disease type Ia

Martijn P.D. Haring,Fabian Peeks,Maaike H. Oosterveer,Martijn C.G.J. Brouwers,Carla E.M. Hollak,Mirian C.H. Janssen,Janneke G. Langendonk,Alexander J.M. Rennings,Margreet A.E.M. Wagenmakers,Henkjan J. Verkade,Terry G.J. Derks,Vincent E. de Meijer

JHEP Reports（2022）

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摘要

•Glycogen storage disease Ia (GSDIa) is a metabolic disease caused by mutations in glucose-6-phosphatase catalytic subunit 1 (G6PC1).•Patients with GSDIa often develop hepatocellular adenoma (HCA), with unclear risk factors.•Metabolic control in GSDIa is commonly evaluated through serum triglyceride concentration (TG).•Patients with GSDIa with high childhood TG had increased risk and earlier onset of HCA.•Sex-associated hypertriglyceridaemia and G6PC1 genotype were not associated with HCA.

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关键词

Benign neoplasm,Hepatic adenoma,Glycogen storage disease type Ia,Metabolic disorder,Glucose-6-phosphatase triglycerides

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