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2007 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.* > 2007 ICD-9-CM Diagnosis 271.2 Hereditary fructose intolerance This code may be outdated. View the most recent version of ICD-9-CM 271.2 An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet. 271.2 is a specific code that can be used to specify a diagnosis 271.2 contains 13 index entries View the ICD-9-CM Volume 1 271.* hierarchy 271.2 also known as: Essential benign fructosuria Fructosemia Index entries containing 271.2: Deficiency, deficient aldolase (hereditary) 271.2 fructokinase 271.2 fructose-1, 6-diphosphate 271.2 fructose-1-phosphate aldolase 271.2 phosphofructokinase 271.2 Disorder - see also Disease metabolism NEC 277.9 fructosemia 271.2 fructosuria 271.2 Disturbance - see also Disease fructose metabolism 271.2 Fructosemia 271.2 Fructosuria (benign) (essential) 271.2 Hyperfructosemia 271.2 Intolerance fructose (hereditary) 271.2 Levulosuria 271.2