Hereditary fructose intolerance

This code represents a rare, inherited metabolic disorder where the body is unable to properly break down fructose due to a deficiency in the enzyme aldolase B. Ingesting fructose, sucrose, or sorbitol leads to the accumulation of toxic byproducts, primarily fructose-1-phosphate, in the liver, kidneys, and small intestine. This accumulation can cause severe hypoglycemia, liver damage, and kidney dysfunction.

Use this code for patients diagnosed with hereditary fructose intolerance, typically confirmed through genetic testing or enzyme assay. It applies when the patient exhibits symptoms such as vomiting, hypoglycemia, jaundice, hepatomegaly, or renal tubular dysfunction following fructose intake. Documentation should clearly indicate the diagnosis and the patient's reaction to fructose.

• Ensure documentation specifies "hereditary" fructose intolerance to differentiate from other fructose malabsorption issues.
• Consider sequencing this code as a primary diagnosis when it is the reason for the encounter, especially for dietary counseling or management of acute symptoms.
• Do not confuse with general fructose malabsorption (which is not typically coded with a specific ICD-9-CM diagnosis code for the malabsorption itself, but rather symptoms).