ICD-10-CM 2027
ICD-10 code for Pipecolic acidemia
Disorders of lysine and hydroxylysine metabolism
From the official ICD-10-CM alphabetic index entry “Acidemia › pipecolic”. Page updated September 29, 2026.
About coding Pipecolic acidemia
The ICD-10-CM code for Pipecolic acidemia is E72.3 (Disorders of lysine and hydroxylysine metabolism).
Within E72 (other disorders of amino-acid metabolism), E72.3 is specifically for disorders of lysine and hydroxylysine metabolism. Related codes cover disorders of amino-acid transport (E72.0), disorders of sulfur-bearing amino-acid metabolism (E72.1), disorders of urea cycle metabolism (E72.2), disorders of ornithine metabolism (E72.4), disorders of glycine metabolism (E72.5), other specified disorders of amino-acid metabolism (E72.8) and disorder of amino-acid metabolism, unspecified (E72.9).
Don't report E72.3 together with the conditions in its Excludes1 note: glutaric aciduria type II (E71.313), Refsum's disease (G60.1) and Zellweger syndrome (E71.510).
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.
Don't confuse with (Excludes1 for E72.3)
Common questions about Pipecolic acidemia ICD-10 codes
What is the ICD-10 code for Pipecolic acidemia?
- E72.3 — Disorders of lysine and hydroxylysine metabolism.
Is E72.3 billable?
- Yes. E72.3 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E72.3 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E72.3 as a principal diagnosis.
Is E72.3 a CC or MCC?
- E72.3 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.