2025年1月21日 · The immediate management of acute hypoglycemia involves rapid correction with oral carbohydrates or parenteral glucose. Glucagon is effective only in insulin-mediated …

GSD 0 (240600*) Glycogen synthase. Onset: Variable but often after cessation of nighttime feedings or intercurrent illness. Clinical features: Fasting hypoglycemia and ketosis, …

A glycogen storage disease (GSD, also glycogenosis and dextrinosis) is a metabolic disorder caused by a deficiency of an enzyme or transport protein affecting glycogen synthesis, …

2010年9月1日 · Results: This management guideline specifically addresses evaluation and diagnosis across multiple organ systems (cardiovascular, gastrointestinal/nutrition, hepatic, …

Branching enzyme deficiency (See GSD IV.) Chronic visceral ASMD (Niemann-Pick disease type B) 2 (See ASM Deficiency.) 1. Fructose-1,6-bisphosphatase deficiency is one example of a …

2014年11月6日 · Glycogen storage disease type I (GSD I) is a rare disease of variable clinical severity that primarily affects the liver and kidney.

2025年1月5日 · Glycogen storage disorders (GSDs) are inherited disorders of metabolism resulting from the deficiency of individual enzymes involved in the synthesis, transport, and …

2010年3月9日 · Glycogen storage disease type III (GSD III) is characterized by variable liver, cardiac muscle, and skeletal muscle involvement. GSD IIIa is the most common subtype, …

* There are many different glycogen storage diseases. Each is identified by a Roman numeral. GSD = glycogen storage disease.

Glycogen storage diseases (GSDs) are genetically determined metabolic diseases that cause disorders of glycogen metabolism in the body. Due to the enzymatic defect at some stage of …