E71.313Glutaric aciduria type II

E71.313 is the ICD-10-CM diagnosis code for Glutaric aciduria type II. It belongs to category E71 (disorders of branched-chain amino-acid metabolism and fatty-acid metabolism), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within E71.31 (disorders of fatty-acid oxidation), E71.313 is specifically for Glutaric aciduria type II. Related codes cover long chain/very long chain acyl CoA dehydrogenase deficiency (E71.310), Medium chain acyl CoA dehydrogenase deficiency (E71.311), short chain acyl CoA dehydrogenase deficiency (E71.312), muscle carnitine palmitoyltransferase deficiency (E71.314) and other disorders of fatty-acid oxidation (E71.318).

Don't report E71.313 together with the conditions in its Excludes1 note: glutaric aciduria (type 1) NOS (E72.3).

As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.

In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Before ICD-10, this condition was coded in ICD-9-CM as 277.85 (disorders of fatty acid oxidation).