ICD-10-CM 2027
ICD-10 code for propionic acidemia
From the official ICD-10-CM alphabetic index entry “Acidemia › propionic”. Page updated September 29, 2026.
About coding propionic acidemia
The ICD-10-CM code for propionic acidemia is E71.121 (Propionic acidemia).
Within E71.12 (disorders of propionate metabolism), E71.121 is specifically for propionic acidemia. Related codes cover Methylmalonic acidemia (E71.120) and other disorders of propionate metabolism (E71.128).
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.
Common questions about propionic acidemia ICD-10 codes
What is the ICD-10 code for propionic acidemia?
- E71.121 — Propionic acidemia.
Is E71.121 billable?
- Yes. E71.121 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E71.121 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E71.121 as a principal diagnosis.
Is E71.121 a CC or MCC?
- E71.121 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.