5th February 2024

A 5-month-old male presents with hepatomegaly.

A 5-month-old male presents with hepatomegaly. His mother has noted poor growth, and he has dropped from the 25th centile to less than the fifth centile for height and weight. Blood tests show deranged liver function tests and clotting studies. The viral hepatitis screen was negative. A liver screen shows marked intracytoplasmic glycogen deposits, liver fibrosis, and cirrhosis. 

Which type of glycogen storage disorder is associated with this?