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ICD-10-CM Code E88.89
Other specified metabolic disorders

Billable Code
Billable codes are sufficient justification for admission to an acute care hospital when used a principal diagnosis.
| ICD-10 from 2011 - 2016

E88.89 is a billable ICD code used to specify a diagnosis of other specified metabolic disorders. A 'billable code' is detailed enough to be used to specify a medical diagnosis.

The ICD code E888 is used to code Trimethylaminuria

Trimethylaminuria (TMAU), as defined by the KIM consortium of endocrinology disorders as fish odor syndrome or fish malodor syndrome, is a rare metabolic disorder that causes a defect in the normal production of an enzyme named flavin-containing monooxygenase 3 (FMO3). When FMO3 is not working correctly or if not enough enzyme is produced, the body loses the ability to properly convert trimethylamine (TMA) from precursor compounds in food digestion into trimethylamine oxide (TMAO), through a process called N-oxygenation. Trimethylamine then builds up and is released in the person's sweat, urine, and breath, giving off a strong fishy odor or strong body odor. A variant of TMAU (secondary TMAU or TMAU2) exists where there is no genetic cause, yet excessive TMA is secreted, possibly due to intestinal dysbiosis, altered metabolism, or hormonal reasons.

Specialty: Endocrinology
ICD 9 Code: 270.8


Source: Wikipedia

Coding Notes for E88.89 Info for medical coders on how to properly use this ICD-10 code

Inclusion Terms:
Inclusion Terms
Inclusion Terms are a list of concepts for which a specific code is used. The list of Inclusion Terms is useful for determining the correct code in some cases, but the list is not necessarily exhaustive.
Launois-Bensaude adenolipomatosis
Code Type-1 Excludes:
Type-1 Excludes
Type-1 Excludes mean the conditions excluded are mutually exclusive and should never be coded together. Excludes 1 means "do not code here."
Adult pulmonary Langerhans cell histiocytosis - instead, use code J84.82

MS-DRG Mapping

  • DRG Group #642 - Inborn and other disorders of metabolism.

Related Concepts SNOMET-CT

  • Liposynovitis prepatellaris (disorder)
  • Dilated cardiomyopathy secondary to deficiency (disorder)
  • Pancreatic colipase deficiency (disorder)

Coding Advice SNOMET-CT

  • Consider additional code to identify specific condition or disease

ICD-10-CM Alphabetical Index References for 'E88.89 - Other specified metabolic disorders'

The ICD-10-CM Alphabetical Index links the below-listed medical terms to the ICD code E88.89. Click on any term below to browse the alphabetical index.

Steatosis (E88.89)
Launois-Bensaude adenolipomatosis (E88.89)
Liposynovitis prepatellaris (E88.89)
Ketosis NEC (E88.89)
Hoffa-Kastert disease (E88.89)
Hoffa's disease (E88.89)
Dihydropyrimidine dehydrogenase disease(DPD) (E88.89)
DPD(dihydropyrimidine dehydrogenase deficiency) (E88.89)
Adenolipomatosis, Launois-Bensaude (E88.89)


Other disorders of lipoid metabolism (approximate match)
Other specified disorders of metabolism (approximate match)

This is the official approximate match mapping between ICD9 and ICD10, as provided by the General Equivalency mapping crosswalk. This means that while there is no exact mapping between this ICD10 code E88.89 and a single ICD9 code, 277.89 is an approximate match for comparison and conversion purposes.

Parent Code: E88.8 - Other specified metabolic disorders

** This Document Provided By ICD.Codes **
Source: http://icd.codes/icd10cm/E8889