This Rare Metabolic Condition Causes Your Body To Produce An Odor That Smells Like Rotten Fish

Schooling pelagic fish in clear blue water
Aaron - stock.adobe.com - illustrative purposes only

For some people with a rare metabolic condition called trimethylaminuria (TMAU), or fish odor syndrome, their body produces an odor that smells like rotten fish.

The odor is caused by a chemical compound known as trimethylamine (TMA), which is produced in the gut. The compound is often associated with cardiovascular disease.

Trimethylaminuria is an uncommon genetic disorder, but it occurs more frequently in women than in men. There is evidence that hormones in females, such as progesterone, can make symptoms worse.

The exact number of individuals who have this condition is unclear, ranging from one in a million to one in 200,000 people.

Some clinicians believe that it is underdiagnosed because trimethylaminuria tends to go unrecognized by many healthcare professionals, and people with mild symptoms do not always seek medical help.

Typically, trimethylaminuria is caused by mutations to the FMO3 gene. So, the compound trimethylamine is a byproduct of the digestion of certain foods like eggs, liver, legumes, and seafood, including squid, fish, and crabs.

Normally, an enzyme encoded by the gene FMO3, breaks trimethylamine down into an odorless chemical, which is excreted through urine. But in patients with trimethylaminuria, this enzymatic process does not occur.

As a result, trimethylamine builds up in the body and is released in large amounts through sweat, breath, and urine, giving rise to the stench of rotten fish.

In most cases, mutations to FMO3 are inherited in a recessive pattern, meaning that both parents carry at least one copy of the mutated FMO3 gene for the condition to develop. Carriers may have no signs of trimethylaminuria, mild symptoms, or only temporary episodes.

Schooling pelagic fish in clear blue water
Aaron – stock.adobe.com – illustrative purposes only

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Symptoms of trimethylaminuria may be present from birth or arise near the start of puberty. The fish-like odor may stay at a strong level all the time or vary in consistency. Stress levels, diet, and exercise can worsen symptoms by increasing sweat production.

Trimethylaminuria usually does not have any other symptoms besides a fishy smell, but the condition can have devastating effects on people’s quality of life. It can cause people to socially isolate themselves and lead to depression and anxiety.

Currently, no cure exists for trimethylaminuria. However, healthcare providers recommend that patients avoid foods that contain trimethylamine or substances that can be broken down by the chemical, such as eggs, liver, kidney, seafood, milk from wheat-fed cows, and peas.

Patients may also be advised to use a slightly acidic soap or shampoo, along with antiperspirant, and to avoid strenuous exercise.

In addition, doctors can prescribe low doses of antibiotics to minimize the bacteria in the gut that metabolize trimethylamine.

Certain supplements like activated charcoal can reduce the amount of trimethylamine absorbed from the gut. Activated charcoal should be used with caution, though, because it can interact with other medications.

Emily  Chan is a writer who covers lifestyle and news content. She graduated from Michigan State University with a ... More about Emily Chan

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