Other specified disorders of carbohydrate metabolism

This code represents various rare or less common inherited or acquired conditions affecting the body's ability to process carbohydrates, excluding those with more specific ICD-10-CM codes. These disorders involve enzymatic defects or transport protein abnormalities that disrupt the normal metabolic pathways of sugars other than glucose, such as fructose or galactose, or more complex carbohydrates.

Assign this code for documented carbohydrate metabolism disorders that do not have a more specific code within the E74 category. Examples include essential fructosuria, hereditary fructose intolerance (when not specified as aldolase B deficiency), or other unclassified disorders of carbohydrate metabolism. Documentation should clearly state the specific carbohydrate metabolism disorder, and that it is not otherwise classified.

• Do not use this code if a more specific E74 subcategory code is available (e.g., E74.0- for glycogen storage disease, E74.2- for disorders of galactose metabolism).
• Ensure the physician's documentation clearly identifies the specific disorder and confirms it is not classifiable elsewhere.
• Consider sequencing this code as a primary diagnosis when it is the reason for the encounter, or as a secondary diagnosis when it is a co-morbidity.