Purpose of the Review The purpose of this study is to review nificantly improved their outcome and improved metabolic the pathobiology of the hepatic glycogen storage diseases control can prevent many long-term complications. (GSD) and recent developments in their management. Recent Findings Increasingly, noninvasive mutation detec- Keywords Glycogen storage disease Inherited metabolic . . . tion has replaced biological and tissue samples as the first disorder Dietary treatment Metabolic control Hepatic . . line diagnostic method. For type 1 GSD, continuous sub- adenomata Hepatocellular carcinoma Liver transplantation cutaneous monitoring has transformed patient monitoring. There is evidence that improved metabolic control can prevent complications such as adenoma formation and Introduction renal dysfunction. Improved understanding of the patho- physiology of hepatic adenomata may allow rationaliza- Short-term blood glucose control is mediated by the liver via tion of their management. Hepatocellular carcinoma only glucose-6-phosphate (G6P). Postprandially, the liver imports develops in established adenomata, offering an option for glucose to form G6P which is used for glycogen synthesis. prevention. For type 111 GSD, intrinsic liver disease and The process is then reversed resulting in hepatic glucose ex- myopathy become the major clinical concerns after child- port by dephosphorylation of G6P by glucose-6-phosphatase hood.
Current Pathobiology Reports – Springer Journals
Published: Jun 6, 2017
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