Specific code 2014 ICD-9-CM Diagnosis Code 271.2
Hereditary fructose intolerance
  • Billable Code
  • Short description: Hered fructose intoleran.
  • ICD-9-CM 271.2 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 271.2 is a billable medical code that can be used to specify a diagnosis on a reimbursement claim.
Disease Synonyms
  • Deficiency, fructose 1,6 diphosphatase
  • Essential benign fructosuria
  • Fructokinase deficiency
  • Fructose 1,6 diphosphatase deficiency
  • Fructose metabolism disorder
  • Fructose-1,6-bisphosphate aldolase B deficiency (disorder)
  • Fructose-biphosphatase deficiency
  • Hepatic fructokinase deficiency (disorder)
  • Hereditary fructosuria
Clinical Information
  • A genetic disorder characterized by the absence of the enzyme aldolase-b from the liver. This enzyme is essential for the metabolism of fructose. Signs and symptoms from fructose ingestion are evident in infancy and include vomiting, abdominal pain and hypoglycemia. Long term complications include hepatic and renal failure.
  • 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.
  • 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; 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.
Applies To
  • Essential benign fructosuria
  • Fructosemia
Convert to ICD-10-CM: 271.2 converts approximately to:
  • 2014 ICD-10-CM E74.11 Essential fructosuria
    Or:
  • 2014 ICD-10-CM E74.12 Hereditary fructose intolerance
ICD-9-CM Volume 2 Index entries containing back-references to 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
  • 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
ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions. ICD-9-CM 271.2 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.