type 4 glycogenosis


Glycogenosis due to brancher enzyme deficiency, resulting in the accumulation of abnormal glycogen in liver, kidney, muscle, and other tissues and characterized by progressive cirrhosis of the liver, leading to liver failure and death, usually before age 2.Andersen's disease brancher deficiency amylopectinosis

The American Heritage® Stedman's Medical Dictionary Copyright © 2002, 2001, 1995 by Houghton Mifflin Company. Published by Houghton Mifflin Company.