E74.19

Applicable To

• Fructose-1, 6-diphosphatase deficiency

Approximate Synonyms

• Deficiency, fructose 1,6 diphosphatase

• Fructose-biphosphatase deficiency

Clinical Information

• An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Details

• E74.19 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM E74.19 became effective on October 1, 2022.

• This is the American ICD-10-CM version of E74.19 – other international versions of ICD-10 E74.19 may differ.