Home > 2015 ICD-9-CM Diagnosis Codes > 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-
Specific code 2015 ICD-9-CM Diagnosis Code 271.2
Hereditary fructose intolerance
  • 2015
  • Billable Thru Sept 30/2015
  • Non-Billable On/After Oct 1/2015

  • ICD-9-CM 271.2 is a billable medical code that can be used to indicate a diagnosis on a reimbursement claim, however, 271.2 should only be used for claims with a date of service on or before September 30, 2015. For claims with a date of service on or after October 1, 2015, use an equivalent ICD-10-CM code (or codes).
Convert to ICD-10-CM: 271.2 converts approximately to:
  • 2015/16 ICD-10-CM E74.11 Essential fructosuria
    Or:
  • 2015/16 ICD-10-CM E74.12 Hereditary fructose intolerance
Approximate 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
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
 271.1 ICD9Data.com 271.3 
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 2015 ICD-9-CM Index or use the search engine at the top of this page to lookup any code.
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