ICD-10 code for phenylketonuria

The ICD-10-CM code for phenylketonuria is E70.1 (Other hyperphenylalaninemias). The official index lists 2 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on E70.1.

Within E70, choose E70.1 (Other hyperphenylalaninemias) only when documentation doesn't support a more specific option: classical phenylketonuria (E70.0), disorders of tyrosine metabolism (E70.2), albinism (E70.3), disorders of histidine metabolism (E70.4), disorders of tryptophan metabolism (E70.5), other disorders of aromatic amino-acid metabolism (E70.8) and disorder of aromatic amino-acid metabolism, unspecified (E70.9).

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.