Other disorders of fructose metabolism
ICD-10 E74.19 is a billable code used to indicate a diagnosis of other disorders of fructose metabolism.
E74.19 encompasses a range of metabolic disorders related to the abnormal metabolism of fructose, a simple sugar found in many foods. These disorders can arise from various enzyme deficiencies, particularly those affecting the conversion of fructose into glucose. The most notable condition associated with this code is hereditary fructose intolerance (HFI), which results from a deficiency of the enzyme aldolase B. Patients with HFI may experience severe hypoglycemia, gastrointestinal distress, and potential liver damage upon ingestion of fructose or sucrose. Other less common disorders may include fructose-1,6-bisphosphatase deficiency and other enzyme deficiencies that disrupt normal fructose metabolism. Diagnosis typically involves clinical evaluation, dietary history, and biochemical testing to identify enzyme deficiencies. Management primarily focuses on dietary restrictions to avoid fructose and sucrose, alongside monitoring for complications. Understanding these metabolic pathways is crucial for accurate diagnosis and treatment, as well as for coding purposes.
Detailed dietary history and symptomatology, including growth and development assessments.
Diagnosis of fructose intolerance in infants presenting with failure to thrive or gastrointestinal symptoms.
Consideration of family history and genetic testing for hereditary conditions.
Comprehensive evaluation of gastrointestinal symptoms and metabolic testing results.
Adult patients presenting with unexplained abdominal pain or malabsorption issues.
Need for collaboration with metabolic specialists for accurate diagnosis.
Used to evaluate fructose metabolism disorders.
Document the indication for the test and results.
Pediatric specialists may require additional documentation regarding growth and dietary history.
Common symptoms include abdominal pain, hypoglycemia, vomiting, and failure to thrive in children. Symptoms can vary based on the specific enzyme deficiency and dietary intake.