Definition:
An autosomal recessive metabolic disorder due to a deficiency in expression of 1 (alpha-1,4-glucan-6-alpha-glucosyltransferase), resulting in an accumulation of abnormal with long outer branches. Clinical features are and . Death from liver disease usually occurs before age 2.
Synonym(s):
Amylopectinosis /
Andersen Disease /
Brancher Deficiency /
Glycogenosis 4 /