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Trimethylaminuria


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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3052392/

 

Trimethylaminuria is a disorder in which the volatile, fish-smelling compound, trimethylamine (TMA) accumulates and is excreted in the urine, but is also found in the sweat and breath of these patients. Because many patients have associated body odours or halitosis, trimethylaminuria sufferers can meet serious difficulties in a social context, leading to other problems such as isolation and depression. TMA is formed by bacteria in the mammalian gut from reduction of compounds such as trimethylamine-N-oxide (TMAO) and choline. Primary trimethylaminuria sufferers have an inherited enzyme deficiency where TMA is not efficiently converted to the non-odorous TMAO in the liver. Secondary causes of trimethylaminuria have been described, sometimes accompanied by genetic variations. Diagnosis of trimethylaminuria requires the measurement of TMA and TMAO in urine, which should be collected after a high substrate meal in milder or intermittent cases, most simply, a marine-fish meal. The symptoms of trimethylaminuria can be improved by changes in the diet to avoid precursors, in particular TMAO which is found in high concentrations in marine fish. Treatment with antibiotics to control bacteria in the gut, or activated charcoal to sequester TMA, may also be beneficial.

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https://www.genome.gov/11508983

 

Learning About Trimethylaminuria

There is currently no cure for trimethylaminuria. However, it is possible for people with this condition to live normal, healthy lives. The following are some ways a person with trimethylaminuria can lower symptoms of odor:

  • Avoiding foods containing trimethylamine and its precursors (choline, lecithin and trimethylamine N-oxide).
  • Trimethylamine is present in high levels in milk obtained from wheat-fed cows
  • Choline is present in high amounts in:
    • Eggs
    • Liver
    • Kidney
    • Peas
    • Beans
    • Peanuts
    • Soy products
    • Brassicas (brussel sprouts, broccoli, cabbage, and cauliflower)
    • Lecithin and lecithin-containing fish oil supplements
       
  • Trimethylamine N-oxide is present in seafood (fish, cephalopods, crustaceans). Freshwater fish have lower levels of trimethylamine N-oxide.
  • Taking low doses of antibiotics to reduce the amount of bacteria in the gut. This suppresses the production of trimethylamine.
  • Taking laxatives can decrease intestinal transit time and reduce the amount of trimethylamine produced in the gut.
  • Taking supplements to decrease the concentration of free trimethylamine in the urine.
  • Activated charcoal taken at a dose of 750mg twice daily for ten days. Copper chlorophyllin taken at a dose of 60mg three times a day after meals for three weeks.
  • Using soaps with a moderate pH, between 5.5 and 6.5. Trimethylamine is a strong base (pH 9.8), thus soaps with pH closer to that of normal skin help retain the secreted trimethylamine in a less volatile form that can be removed by washing.
  • Taking riboflavin (vitamin B2) supplements to enhance any residual FMO3 enzyme activity. Recommended intake is 30-40mg taken 3-5 times per day with food.
  • Avoiding factors that promote sweating, such as exercise, stress, and emotional upsets.

It is important that a person who has trimethylamuinuria follow the treatment advice of their health care provider. They should not attempt to self-administer these treatment approaches. Medications and supplements can have unintended interactions, and dietary restrictions can result in nutritional deficits. Choline is essential for nerve and brain development in fetuses and infants, therefore, pregnant and breast-feeding women should consult with their health care provider before restricting their dietary choline.

 

People with trimethylaminuria may also find the following to be helpful:

  • 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 this disorder on to the next generation.

Edited by cerebral ecstasy
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  • 8 years later...

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