2006 ICD-9-CM Volume 1 Diagnosis Codes Home > Endocrine, Nutritional And Metabolic Diseases, And Immunity Disorders 240-279 > Other Metabolic Disorders And Immunity Disorders 270-279 > Disorders Of Carbohydrate Transport And Metabolism 271.* > 2006 ICD-9-CM Diagnosis 271.0 Glycogenosis View the most recent version of ICD-9-CM 271.0 An 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. An 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 (MUSCLE, SKELETAL); HEART; LIVER; SPINAL CORD; and BRAIN. Three forms have been described: infantile, childhood, and adult. The infantile form is fatal in infancy and presents with hypotonia and a hypertrophic cardiomyopathy (CARDIOMYOPATHY, HYPERTROPHIC). 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. Glycogenosis due to muscle phosphorylase deficiency. Characterized by painful cramps following sustained exercise. A group of inherited metabolic disorders involving the enzymes responsible for the synthesis and degradation of glycogen. In some patients, prominent liver involvement is presented. In others, more generalized storage of glycogen occurs, sometimes with prominent cardiac involvement. An 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. 271.0 is a specific code that can be used to specify a diagnosis 271.0 contains 59 index entries View the ICD-9-CM Volume 1 271.* hierarchy Alternate Terminology Amylopectinosis Glucose-6-phosphatase deficiency Glycogen storage disease McArdle's disease Pompe's disease von Gierke's disease  Index entries containing 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 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] 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 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