ICD-10 code for Lesch-Nyhan syndrome

The ICD-10-CM code for Lesch-Nyhan syndrome is E79.1 (Lesch-Nyhan syndrome).

Within E79 (disorders of purine and pyrimidine metabolism), E79.1 is specifically for Lesch-Nyhan syndrome. Related codes cover Hyperuricemia without signs of inflammatory arthritis and tophaceous disease (E79.0), Myoadenylate deaminase deficiency (E79.2), other disorders of purine and pyrimidine metabolism (E79.8) and disorder of purine and pyrimidine metabolism, unspecified (E79.9).

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.