Cardiac Pathology: Genetic: Glycogen storage disease Type I (Von Gierke's disease)

01 May 2011
Presented by Mary Sheppard


Cardiac manifestations of Von Gierke's disease


A 1940 autopsy specimen from a boy of sixteen, one of three apparently healthy siblings, who collapsed and died whilst on his way home from school. He had not made any complaint and had been well even on the day of his death. There had been no jaundice and P.M. showed the liver unaffected by the disease. The heart weighed 450g ( normal for age 280g ).


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