Treatment of Glycogen Storage Disease Type IV in Hepatomegaly with Elevated ALT and AST

This protocol covers Glycogen storage disease type IV (GSD IV) presenting with hepatomegaly alongside elevated alanine aminotransferase (ALT) and aspartate aminotransferase (AST) — a constellation indicating symptomatic hepatic involvement that calls for structured clinical assessment and management.

Hepatomegaly combined with elevated ALT and AST in a patient with GSD IV signals active liver involvement. Evaluation of the degree of hepatic disease — including consideration of liver biopsy — is integral to determining the appropriate management pathway.

Care is directed at supportive management of hepatic complications. This encompasses strategies for managing ascites and portal hypertension, nutritional measures to maintain normoglycemia, and supplementation as disease progresses. The complete structured regimen — including the full sequence, all relevant interventions, and specific considerations — is available in the protocol below.