Short answer · Medically reviewed summary · Last updated: 2026-05-08
The condition clinically known as Hereditary Fructose Intolerance (HFI) is assigned the ICD-10 code E74.12, while it was historically classified under the ICD-9 code 271.2. These codes are essential for medical billing and identifying the specific metabolic deficiency of the enzyme aldolase B in patient health records. What is the clinical significance of Hereditary Fructose Intolerance? Hereditary Fructose Intolerance is a rare autosomal recessive metabolic disorder caused by a deficiency of the enzyme aldolase B.
ICD10 code of Fructose Intolerance and ICD9 code
ICD-10 and ICD-9 codes for Fructose Intolerance, with classification details for clinicians, coders and patients.
The condition clinically known as Hereditary Fructose Intolerance (HFI) is assigned the ICD-10 code E74.12, while it was historically classified under the ICD-9 code 271.2. These codes are essential for medical billing and identifying the specific metabolic deficiency of the enzyme aldolase B in patient health records.
What is the clinical significance of Hereditary Fructose Intolerance?
Hereditary Fructose Intolerance is a rare autosomal recessive metabolic disorder caused by a deficiency of the enzyme aldolase B. When individuals with Hereditary Fructose Intolerance ingest fructose, sucrose, or sorbitol, toxic metabolites accumulate in the liver, kidneys, and small intestine. If left unmanaged, the condition can lead to severe hypoglycemia, jaundice, and potential organ damage.
How are the ICD codes used for Hereditary Fructose Intolerance?
The ICD-10 code E74.12 is used globally to standardize the reporting of Hereditary Fructose Intolerance in hospital and clinical settings. Using the correct diagnostic code ensures that patients receive appropriate dietary support and monitoring. Because Hereditary Fructose Intolerance requires lifelong dietary modifications, accurate coding is critical for insurance coverage of specialized nutritional counseling and metabolic monitoring.
What diagnostic markers confirm the condition?
Diagnosis of Hereditary Fructose Intolerance typically involves a combination of clinical assessment and genetic testing. Key diagnostic features include:
- Molecular genetic testing identifying mutations in the ALDOB gene.
- History of symptoms following the introduction of fruit, juice, or formula containing sucrose.
- Elevated liver enzymes and persistent hypoglycemia after fructose ingestion.
- Clinical improvement observed upon the strict elimination of dietary fructose.
How does the community support patients?
At DiseaseMaps.org, 93 people with Hereditary Fructose Intolerance have joined our community to share their experiences and coping strategies. Connecting with others who manage this complex metabolic profile can significantly reduce the isolation often felt when navigating a restricted diet.
Next steps
- Consult a metabolic specialist or a genetic counselor to confirm your diagnosis via ALDOB gene sequencing.
- Work with a registered dietitian who specializes in inborn errors of metabolism to ensure nutritional adequacy.
- Join the DiseaseMaps.org community to connect with other families living with Hereditary Fructose Intolerance.
- Carry an emergency medical alert card specifying your diagnosis and the need to avoid fructose-containing intravenous fluids.
Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment; always seek the guidance of your physician regarding any medical condition.
References
- NIH Genetic and Rare Diseases (GARD) Information Center: Hereditary Fructose Intolerance.
- OMIM (Online Mendelian Inheritance in Man): Aldolase B Deficiency (#229600).
- Orphanet: Hereditary Fructose Intolerance (ORPHA:337).
