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E72.52

Billable

Trimethylaminuria

HCC Category Mapping

V24HCC 23Other Significant Endocrine and Metabolic Disorders
0.230
ESRDHCC 23Other Significant Endocrine and Metabolic Disorders
0.000
RxHCCHCC 43Other Significant Endocrine and Metabolic Disorders
0.000

What This Code Means

A rare metabolic disorder where the body cannot properly process trimethylamine, causing a characteristic fish-like body odor and potential neurological symptoms.

Coding Tips

  • Document the presence or absence of the characteristic odor to help confirm diagnosis
  • Note any associated neurological or psychiatric symptoms that may accompany this condition

Clinical Significance

Trimethylaminuria (fish odor syndrome) is a metabolic disorder where the body cannot convert trimethylamine to its odorless oxide form, resulting in a characteristic strong fishy body odor. While not life-threatening, it causes significant psychosocial burden, depression, and social isolation. Accurate coding supports appropriate risk adjustment for the ongoing management and psychological care these patients need.

Documentation Requirements

  • Provider diagnosis of trimethylaminuria
  • Urine trimethylamine testing showing elevated levels or abnormal trimethylamine-to-trimethylamine N-oxide ratio
  • Genetic testing for FMO3 gene mutations if available
  • Documentation of whether the condition is primary (genetic) or secondary (dietary/gut microbiome related)
  • Current management plan including dietary restrictions and any psychological support

Commonly Confused Codes

Code Hierarchy

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