271.0 GLYCOGENOSIS


Code Information

Diagnosis Code: 271.0

Short Description: Glycogenosis

Long Description: Glycogenosis

Code Classification:

  • Endocrine, nutritional and metabolic diseases, and immunity disorders (240–279)
    • Other metabolic disorders and immunity disorders (270-279)
      • 271 Disorders of carbohydrate transport and metabolism
        • 271.0 Glycogenosis

Code Version: 2015 ICD-9-CM


References to Index of Diseases and Injuries

The code 271.0 has the following ICD-9-CM references to the Index of Diseases and Injuries
  • 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
  • Cardiomyopathy (congestive) (constrictive) (familial) (infiltrative) (obstructive) (restrictive) (sporadic) 425.4
    • due to
      • cardiac glycogenosis 271.0 [425.7]
    • glycogen storage 271.0 [425.7]
  • 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
    • heart (organic) 429.9
      • due to
        • cardiac glycogenosis 271.0 [425.7]
      • glycogen storage 271.0 [425.7]
    • Hers' (glycogenosis VI) 271.0
    • liver 573.9
      • glycogen storage 271.0
    • McArdle (-Schmid-Pearson) (glycogenosis V) 271.0
    • Pompe's (glycogenosis II) 271.0
    • storage
      • glycogen (see also Disease, glycogen storage) 271.0
    • van Creveld-von Gierke (glycogenosis I) 271.0
    • von Gierke's (glycogenosis I) 271.0
  • Disorder - see also Disease
    • metabolism NEC 277.9
      • glycogen storage NEC 271.0
        • hepatorenal 271.0
  • 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
    • cardiac 271.0 [425.7]
    • Cori, types I-VII 271.0
    • diabetic, secondary 250.8 [259.8]
      • due to secondary diabetes 249.8 [259.8]
    • 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
    • pulmonary interstitial 516.62
  • Hepatomegalia glycogenica diffusa 271.0
  • Hers' disease (glycogenosis VI) 271.0
  • Infiltrate, infiltration
    • glycogen, glycogenic (see also Disease, glycogen storage) 271.0
    • heart, cardiac
      • glycogenic 271.0 [425.7]
    • liver 573.8
      • glycogen (see also Disease, glycogen storage) 271.0
    • myocardium, myocardial
      • glycogenic 271.0 [425.7]
  • Limit dextrinosis 271.0
  • McArdle (-Schmid-Pearson) disease or syndrome (glycogenosis V) 271.0
  • Myocardiopathy (congestive) (constrictive) (familial) (idiopathic) (infiltrative) (obstructive) (primary) (restrictive) (sporadic) 425.4
    • due to
      • cardiac glycogenosis 271.0 [425.7]
    • glycogen storage 271.0 [425.7]
  • 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

Crosswalk Information

The code 271.0 converts into the following ICD-10 code(s):
ICD-9 Code ICD-10 Code ICD-10 Description
271.0 Right Arrow E74.00 Glycogen storage disease, unspecified
E74.01 von Gierke disease
E74.04 McArdle disease
E74.09 Other glycogen storage disease
E74.4 Disorders of pyruvate metabolism and gluconeogenesis
This ICD-9 to ICD-10 data is based on the 2018 General Equivalency Mapping (GEM) files published by the Centers for Medicare & Medicaid Services (CMS) for informational purposes only. The data is not an ICD-10 conversion tool and doesn’t guarantee clinical accuracy.

Similar Codes

ICD-9 Code ICD-9 Description
271.1 Galactosemia
271.2 Hereditary fructose intolerance
271.3 Intestinal disaccharidase deficiencies and disaccharide malabsorption
271.4 Renal glycosuria
271.8 Other specified disorders of carbohydrate transport and metabolism
271.9 Unspecified disorder of carbohydrate transport and metabolism


This page was last updated on: 10/1/2014