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amylopectinosis

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amylopectinosis /am·y·lo·pec·ti·no·sis/ (-pek´tĭ-no″sis) glycogen storage disease, type IV.
am·y·lo·pec·ti·no·sis (m-l-pkt-nss)
n.
Glycogenosis due to an enzyme deficiency that causes abnormal glycogen to be stored in the liver, kidney, heart, muscle, and recticuloendothelial system.

amylopectinosis.
amylopectinosis [am″ĭ-lo-pek´tĭ-no´sis]
glycogen storage disease (type IV), a condition in which deficiency of the brancher enzyme amylo-1:4,1:6-transglucoside results in cirrhosis of the liver, hepatosplenomegaly, and progressive hepatic failure and death. Called also Andersen's disease.

amylopectinosis
glycogenosis type IV.


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[FIGURES A-D OMITTED] Type IV glycogen storage disease (type IV GSD), also known as Andersen disease or amylopectinosis, is a rare autosomal-recessive disorder caused by the deficiency of glycogen branching enzyme.
 
 
 
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