Other biotin-dependent carboxylase deficiency

This code represents a rare inherited metabolic disorder characterized by a deficiency in one or more of the biotin-dependent carboxylase enzymes, excluding holocarboxylase synthetase deficiency (D81.810) and biotinidase deficiency (E78.81). This deficiency impairs the body's ability to metabolize certain amino acids, fatty acids, and glucose, leading to a range of neurological, dermatological, and metabolic symptoms.

Assign this code when documentation specifies a biotin-dependent carboxylase deficiency that is not holocarboxylase synthetase deficiency or biotinidase deficiency. This typically applies to deficiencies of specific carboxylases such as propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, or pyruvate carboxylase, when the specific enzyme deficiency is known but doesn't have a more specific ICD-10-CM code. Supporting documentation includes genetic testing results, enzyme assays, and clinical findings consistent with these specific carboxylase deficiencies.

• Ensure the documentation clearly specifies a biotin-dependent carboxylase deficiency other than holocarboxylase synthetase or biotinidase deficiency.
• Do not use this code if the documentation indicates a deficiency of holocarboxylase synthetase (D81.810) or biotinidase (E78.81).
• Review the medical record for specific enzyme names (e.g., propionyl-CoA carboxylase deficiency) to confirm the appropriate "other" category.