2012 ICD-9-CM Diagnosis Code 271.0
Glycogenosis
- any of a group of metabolic disorders characterized by excessive storage of glycogen.
- common hereditary enzyme deficiency causing varying degrees of hemolytic anemia; can cause favism, some drug induced hemolytic anemias, and chronic nonspherocytic hemolytic anemia.
- autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose production; accumulation of glycogen in liver and kidney leads to organomegaly, particularly massive hepatomegaly; increased concentrations of lactic acid and hyperlipidemia appear in the plasma; clinical gout often appears in early childhood.
- autosomal recessively inherited glycogen storage disease caused by glucan 1,4-alpha-glucosidase deficiency; large amounts of glycogen accumulate in the lysosomes of skeletal muscle, heart, liver, spinal cord, and brain; three forms have been described: the infantile form is fatal in infancy and presents with hypotonia and a hypertrophic cardiomyopathy; the childhood form usually presents in the second year of life with proximal weakness and respiratory symptoms; the adult form consists of a slowly progressive proximal myopathy.
- autosomal recessive metabolic disorder due to a deficiency in expression of branching enzyme (alpha-1,4-glucan-6-alpha-glucosyltransferase), resulting in an accumulation of abnormal glycogen with long outer branches; clinical features are muscle hypotonia and cirrhosis; death from liver disease usually occurs before age 2.
- glycogenosis due to muscle phosphorylase deficiency; characterized by painful cramps following sustained exercise.
- ICD-9-CM 271.0 is a billable medical code that can be used to specify a diagnosis on a reimbursement claim.
- You are viewing the 2012 version of ICD-9-CM 271.0.
- More recent version(s) of ICD-9-CM 271.0: 2013.
Applies To
Convert 271.0 to ICD-10-CM 
ICD-9-CM Volume 2 Index entries containing back-references to 271.0:
glycogen 
brancher enzyme (amylopectinosis) 
