Specific code 2014 ICD-9-CM Diagnosis Code 271.0
Glycogenosis
  • Billable Code
  • ICD-9-CM 271.0 will be replaced by an equivalent ICD-10-CM code (or codes) when the United States transitions from ICD-9-CM to ICD-10-CM on October 1, 2015.
  • ICD-9-CM 271.0 is a billable medical code that can be used to specify a diagnosis on a reimbursement claim.
Disease Synonyms
  • Deficiency of glucose-6-phosphate dehydrogenase
  • Generalized glycogen storage disease of infants
  • Glucose 6 phosphatase deficiency
  • Glycogen storage disease
  • Glycogen storage disease type III
  • Glycogen storage disease type VIII
  • Glycogen storage disease, type 1
  • Glycogen storage disease, type 2
  • Glycogen storage disease, type 3
  • Glycogen storage disease, type 4
  • Glycogen storage disease, type 5
  • Glycogen storage disease, type 6
  • Glycogen storage disease, type 7
  • Glycogen storage disease, type 8
  • Glycogen storage disease, type 9
  • Glycogen storage disease, type I
  • Glycogen storage disease, type II (disorder)
  • Glycogen storage disease, type III (disorder)
  • Glycogen storage disease, type IV
  • Glycogen storage disease, type IX
  • Glycogen storage disease, type V
  • Glycogen storage disease, type VI
  • Glycogen storage disease, type VII
  • Glycoprotein storage disorder
Clinical Information
  • Any of a group of metabolic disorders characterized by excessive storage of glycogen.
  • 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 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.
  • 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.
  • Common hereditary enzyme deficiency causing varying degrees of hemolytic anemia; can cause favism, some drug induced hemolytic anemias, and chronic nonspherocytic hemolytic anemia.
  • Glycogenosis due to muscle phosphorylase deficiency; characterized by painful cramps following sustained exercise.
Applies To
  • Amylopectinosis
  • Glucose-6-phosphatase deficiency
  • Glycogen storage disease
  • McArdle's disease
  • Pompe's disease
  • von Gierke's disease
Convert to ICD-10-CM: 271.0 converts approximately to:
  • 2014 ICD-10-CM E74.00 Glycogen storage disease, unspecified
    Or:
  • 2014 ICD-10-CM E74.01 von Gierke disease
    Or:
  • 2014 ICD-10-CM E74.04 McArdle disease
    Or:
  • 2014 ICD-10-CM E74.09 Other glycogen storage disease
    Or:
  • 2014 ICD-10-CM E74.4 Disorders of pyruvate metabolism and gluconeogenesis
ICD-9-CM Volume 2 Index entries containing back-references to 271.0:
  • Aglycogenosis 271.0
  • Amylopectinosis (brancher enzyme deficiency) 271.0
  • Andersen's glycogen storage disease 271.0
  • Cardiomegalia glycogenica diffusa 271.0
  • Cardiomegaly (see also Hypertrophy, cardiac) 429.3
    • glycogen 271.0
  • Deficiency, deficient
    • brancher enzyme (amylopectinosis) 271.0
    • debrancher enzyme (limit dextrinosis) 271.0
    • glucose-6-phosphatase 271.0
    • glycogen synthetase 271.0
    • hepatophosphorylase 271.0
    • liver phosphorylase 271.0
    • lysosomal alpha-1, 4 glucosidase 271.0
    • myophosphorylase 271.0
    • phosphoglucomutase 271.0
    • phosphohexosisomerase 271.0
    • phosphorylase kinase, liver 271.0
    • UDPG-glycogen transferase 271.0
  • Dextrinosis, limit (debrancher enzyme deficiency) 271.0
  • Disease, diseased - see also Syndrome
    • Andersen's (glycogenosis IV) 271.0
    • Forbes' (glycogenosis III) 271.0
    • Gierke's (glycogenosis I) 271.0
    • glycogen storage (Andersen's) (Cori types 1-7) (Forbes') (McArdle-Schmid-Pearson) (Pompe's) (types I-VII) 271.0
      • cardiac 271.0 [425.7]
      • generalized 271.0
      • glucose-6-phosphatase deficiency 271.0
      • heart 271.0 [425.7]
      • hepatorenal 271.0
      • liver and kidneys 271.0
      • myocardium 271.0 [425.7]
      • von Gierke's (glycogenosis I) 271.0
    • Hers' (glycogenosis VI) 271.0
    • McArdle (-Schmid-Pearson) (glycogenosis V) 271.0
    • Pompe's (glycogenosis II) 271.0
    • storage
      • glycogen (see also Disease, glycogen storage) 271.0
      • lipid 272.7
      • mucopolysaccharide 277.5
    • van Creveld-von Gierke (glycogenosis I) 271.0
    • von Gierke's (glycogenosis I) 271.0
  • Disorder - see also Disease
  • Forbes' (glycogen storage) disease 271.0
  • Gierke's disease (glycogenosis I) 271.0
  • Glycogen
    • infiltration (see also Disease, glycogen storage) 271.0
    • storage disease (see also Disease, glycogen storage) 271.0
  • Glycogenosis (see also Disease, glycogen storage) 271.0
    • Cori, types I-VII 271.0
    • diffuse (with hepatic cirrhosis) 271.0
    • generalized 271.0
    • glucose-6-phosphatase deficiency 271.0
    • hepatophosphorylase deficiency 271.0
    • hepatorenal 271.0
    • myophosphorylase deficiency 271.0
  • Hepatomegalia glycogenica diffusa 271.0
  • Hers' disease (glycogenosis VI) 271.0
  • Infiltrate, infiltration
    • glycogen, glycogenic (see also Disease, glycogen storage) 271.0
    • liver 573.8
      • fatty - see Fatty, liver
      • glycogen (see also Disease, glycogen storage) 271.0
  • Limit dextrinosis 271.0
  • McArdle (-Schmid-Pearson) disease or syndrome (glycogenosis V) 271.0
  • Pompe's disease (glycogenosis II) 271.0
  • Syndrome - see also Disease
    • McArdle (-Schmid) (-Pearson) (glycogenosis V) 271.0
  • Thesaurismosis
    • glycogen (see also Disease, glycogen storage) 271.0
  • van Creveld-von Gierke disease (glycogenosis I) 271.0
  • von Gierke's disease (glycogenosis I) 271.0
ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions. ICD-9-CM 271.0 is one of thousands of ICD-9-CM codes used in healthcare. Although ICD-9-CM and CPT codes are largely numeric, they differ in that CPT codes describe medical procedures and services. Can't find a code? Start at the root of ICD-9-CM, check the 2014 ICD-9-CM Index or use the search engine at the top of this page to lookup any code.