What’s Fishy Smell Syndrome?

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Fishy odor syndrome is a rare metabolic disorder caused by a lack of enzymes to digest trimethylamine, leading to a strong odor in sweat, urine, and other body fluids. It is not curable, but a low-protein diet, activated charcoal, and medication can help manage the odor. The condition is recessive and more noticeable in women, possibly due to estrogen. Psychological distress may occur, and deodorants should be used with caution.

Fishy odor syndrome is an unusual metabolic disorder found in people who lack enzymes to digest trimethylamine, an organic compound that is a byproduct of digestion. This compound accumulates in the body instead of being broken down and is expressed in sweat, urine and other body fluids. The strong odor may be evident, especially when the patient is sweating heavily, and may cause social discomfort. Also, some foods can cause an elevated heart rate and an increase in blood pressure as the body’s metabolism struggles to cope with them.

Known formally are trimethylaminuria, this condition is recessive. People need to inherit two copies of the gene for it to be expressed, and individuals with fishy odor syndrome will pass one copy of the faulty gene to their children. The children will become carriers, unless the other parent also has a copy of the gene to pass on. It’s also quite rare; a very small percentage of the population has this condition.

It is not possible to fix the metabolism to make it possible to digest the trimethylamine and resolve the fishy smell syndrome, but there are ways to keep the strong smell down. Patients may be advised to follow a low-protein diet, avoiding foods with trimethylamine precursors such as carnitine, sulfur, choline, and nitrogen. The strong odor can be exacerbated by bacteria in the gut, as the balance of organisms in the digestive tract can be upset when a patient’s metabolism is malfunctioning. Sometimes taking medications to kill some bacteria can help. Patients also sometimes experience relief from consuming activated charcoal to reduce odor.

Detergents with a high pH may be helpful in removing odor from clothes and keeping odors down when patients with fishy odor syndrome sweat. People may also choose to live in cool climates and limit activities known to induce heavy sweating. Fishy smell syndrome has been associated with psychological distress in some patients and it may be helpful to consult a mental health professional for talk therapy and access to medications to balance brain chemistry and address suicidal thoughts and depression.

This condition tends to be more noticeable in women than in men. Some researchers theorize that this is a result of female hormones such as estrogen. Women may experience cyclical changes in the strength and nature of odor, strongly suggesting it is related to fluctuating hormone levels. Using deodorants to manage odor can be helpful, although patients may want to be careful with scented products, as odor can react with trimethylamine and produce a strong, off-putting odor.




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