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Trimethylaminuria

Trimethylaminuria is a disorder in which the body is unable to break down trimethylamine, a compound derived from the diet that has a strong odor of rotting fish. As trimethylamine builds up in the body, it causes affected people to give off a fish-like odor in their sweat, urine, and breath. The intensity of this odor may vary over time. In many cases, the strong body odor interferes with an affected person's relationships, social life, and career. People with trimethylaminuria may experience depression and social isolation as a result of this condition. more...

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Diagnosis

The only test for trimethylaminuria at this time is a urine test for elevated levels of trimethylamine.

A similar test can be used to identify carriers of this condition - those individuals who carry one copy of a mutated gene but do not have symptoms. In this case, the person would be given a high dose of choline (one of the precursors of trimethylamine) and then have their urine tested for elevated levels of trimethylamine.

Treatment

Although there is no cure for trimethylaminuria, it is possible for people with this condition to live normal, healthy lives without the fear of being shunned because they smell like rotten fish. Ways of reducing the odor include:

  • Avoiding foods containing choline and trimethylamine-oxide, the precursors of trimethylamine.
  • Taking low doses of antibiotics to reduce the amount of bacteria in the gut.
  • Using soaps with a moderate pH, between 5.5 and 6.5.

Also helpful are:

  • Behavioral counseling to help with depression and other psychological symptoms.
  • Genetic counseling to better understand how they developed the condition and to be aware of the risks of passing it on to the next generation.

Genetics

Most cases of trimethylaminuria appear to be inherited in an autosomal recessive pattern, which means two copies of the gene in each cell are altered. Most often, the parents of an individual with an autosomal recessive disorder are carriers of one copy of the altered gene. Carriers may have mild symptoms of trimethylaminuria or experience temporary episodes of fish-like body odor.

Mutations in the FMO3 gene cause trimethylaminuria. The FMO3 gene makes an enzyme that breaks down nitrogen-containing compounds from the diet, including trimethylamine. This compound is produced by bacteria in the intestine as they digest proteins from eggs, meat, soy, and other foods. Normally, the FMO3 enzyme converts fishy-smelling trimethylamine into another molecule that has no odor. If the enzyme is missing or its activity is reduced because of a mutation in the FMO3 gene, trimethylamine is not broken down and instead builds up in the body. As the compound is released in a person's sweat, urine, and breath, it causes the strong odor characteristic of trimethylaminuria. Researchers believe that stress and diet also play a role in triggering symptoms.

Although FMO3 mutations account for most known cases of trimethylaminuria, some cases are caused by other factors. A fish-like body odor could result from an excess of certain proteins in the diet or from an increase in bacteria that normally break down trimethylamine in the digestive system. A few cases of the disorder have been identified in adults with liver damage caused by hepatitis. Temporary symptoms of trimethylaminuria have also been reported in a small number of premature infants and in some healthy women at the start of menstruation.

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The 'fish odor' syndrome - Tips from Other Journals
From American Family Physician, 4/1/94

Persistent body malodor can cause great psychosocial distress to patients. One of the most pungent is the "fish odor" syndrome, or trimethylaminuria. In this condition, metabolism of food constituents such as choline and carnitine to trimethylamine N-oxide is impaired. As a result, the urine, sweat, breath and body secretions contain trimethylamine. This amine smells strongly of rotting fish and can be detected by the human nose at concentrations less than one part per million. Although approximately 1 percent of the population may be affected, little is known about this condition. Ayesh and colleagues used interest generated by a British newspaper article on the syndrome to learn more about the condition, particularly its mode of inheritance.

Following the newspaper article, 187 letters of inquiry were received from people who suspected they or their children had the syndrome. These individuals were asked to provide two urine samples for trimethylamine excretion assessment: the first a 24-hour collection under normal dietary conditions and the second an eight-hour collection following an oral challenge test with 600 mg of trimethylamine.

Eleven cases of trimethylaminuria were clinically and biochemically confirmed. These six females and five males ranged in age from infancy to 70 years and were all in good general health. A variety of adverse psychosocial reactions was reported, including depression, difficulties with relationships and career advancement, substance abuse and paranoia.

The parents of six of the 11 patients originally identified were also tested. All of the parents showed impaired trimethylamine metabolism after oral challenge, suggesting heterozygous characteristics in the carrier.

The authors conclude that the fish odor syndrome can be inherited as an autosomal recessive condition and that the malodor may cause severe distress to patients. To manage the problem, they recommend reducing carnitine and choline in the patient's diet, prescribing neomycin and metronidazole, and informing the patient that certain events might exacerbate the problem, including menstruation, fever or stress. (BMJ, September 11, 1993, vol. 307, p. 655.)

COPYRIGHT 1994 American Academy of Family Physicians
COPYRIGHT 2004 Gale Group

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