Progressive myopathy in a patient with type IIIa glycogen storage disease. The patient has a debrancher deficiency in both liver and muscle (subtype IIIa).

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Progressive myopathy in a patient with type IIIa glycogen storage disease. The patient has a debrancher deficiency in both liver and muscle (subtype IIIa).
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Progressive myopathy in a patient with type IIIa glycogen storage disease. The patient has a debrancher deficiency in both liver and muscle (subtype IIIa). As a child, he had hepatomegaly, hypoglycemia, and growth retardation. After puberty, he no longer had hepatomegaly, and his final height is normal. Note the muscle wasting in the lower legs and both hands at age 44 years of age (left panel); this condition progressed to pronounced muscle atrophy at age 53 years (two right panels). (Source: CR Scriver et al [eds]: The Metabolic and Molecular Bases of Inherited Disease online, 8th ed. New York, McGraw-Hill, www.ommbid.com.) See Chap. 433e. Source: Atlas of Clinical Manifestations of Metabolic Diseases, Harrison's Principles of Internal Medicine, 19e Citation: Kasper D, Fauci A, Hauser S, Longo D, Jameson J, Loscalzo J. Harrison's Principles of Internal Medicine, 19e; 2015 Available at: http://accessmedicine.mhmedical.com/DownloadImage.aspx?image=/data/books/1130/kas_ch436e_f019.png&sec=98734146&BookID=1130&ChapterSecID=79754603&imagename= Accessed: October 27, 2017 Copyright © 2017 McGraw-Hill Education. All rights reserved

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