What’s Trimethylaminuria?

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Trimethylaminuria is a genetic condition that causes a fishy body odor due to a lack of the enzyme that breaks down trimethylamine. It can be managed by controlling diet and taking activated charcoal tablets. Psychological distress can be reduced through therapy.

Trimethylaminuria is a rare recessive genetic condition that causes people to have a fishy body odor. This condition is not harmful to physical health, although some people with trimethylaminuria experience emotional distress and social difficulties due to the smell, which can sometimes be quite strong. There is no cure for this condition, but it can be managed, and there are several ways people can reduce fishy odor if it becomes a problem.

To have trimethylaminuria, someone must inherit both copies of the faulty gene. The condition is actually a metabolic disorder, caused by a lack of the enzyme that breaks down trimethylamine. Since the body cannot break it down, trimethylamine is expressed in body fluids such as sweat, urine and mucus, and this compound is responsible for the fishy smell. Some people who carry one copy of the gene may have body odor problems, but usually both copies are needed for symptoms to appear.

This condition appears to be more common in women, although it is not linked to gender. Researchers have theorized that female hormones could make trimethylaminuria worse or more noticeable, and that men who have the condition may not always be aware of it because symptoms are low-level. A doctor can diagnose trimethylaminuria by testing urine for trimethylamine, performing genetic testing to look for the rogue gene responsible, or by giving the patient a challenge test in which a large dosage of trimethylamine is given and followed through the body.

Controlling your diet is the most effective way to reduce fishy odor by reducing foods that contain trimethylamine precursors. Foods rich in sulphur, nitrogen and choline such as eggs, fish and beans should be avoided or eaten in moderation. Some patients also have success with activated charcoal tablets, which appear to reduce odor for many patients. A doctor or nutritionist can help a patient find safe foods to eat by balancing nutritional needs.

People with trimethylaminuria can experience psychological distress due to their body odor, and some doctors recommend getting treatment as well as pursuing measures to control the disorder. Patients may find it helpful to attend therapy so they can talk about their experiences and develop coping techniques for social situations. Especially young children can benefit from the therapy, as their classmates may tease them about their body odor.




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