Severe primary trimethylaminuria (TMAU) is a rare metabolic disorder that causes an individual to produce an abnormally strong odor of fish or rotting eggs. It is caused by a genetic mutation that affects the body's ability to break down trimethylamine (TMA), a compound found in certain foods. People with TMAU have an inability to break down TMA, which builds up in their bodies and is released through their sweat, urine, and breath.

The symptoms of severe primary trimethylaminuria (TMAU) include:
* A strong, fish-like body odor that is present from birth or develops during childhood
* Body odor that is worse after eating certain foods, such as eggs, fish, legumes, and some vegetables
* Body odor that is worse during times of stress or when the body is warm
* Body odor that is not improved by frequent bathing or using deodorants
* Low self-esteem and social isolation due to the body odor

The cause of severe primary trimethylaminuria is unknown, but it is believed to be caused by a genetic mutation that affects the body's ability to break down trimethylamine, a compound found in certain foods. This mutation results in an enzyme deficiency that prevents the body from breaking down trimethylamine, causing it to accumulate in the body and be released through sweat, urine, and breath.

1. Dietary modifications: Avoiding foods high in trimethylamine (TMA) such as fish, eggs, legumes, and certain vegetables can help reduce the amount of TMA in the body.

2. Activated charcoal: Taking activated charcoal can help reduce the amount of TMA in the body by binding to it and preventing it from being absorbed.

3. Antibiotics: Antibiotics such as metronidazole and neomycin can help reduce the amount of bacteria in the gut that produce TMA.

4. Choline supplementation: Choline is an essential nutrient that helps the body break down TMA. Supplementing with choline can help reduce the amount of TMA in the body.

5. Probiotics: Probiotics can help restore the balance of bacteria in the gut, which can

1. Genetic mutation: Severe primary trimethylaminuria is caused by a genetic mutation in the FMO3 gene, which is responsible for producing an enzyme that helps break down trimethylamine.

2. Age: Severe primary trimethylaminuria is more common in children and adolescents than adults.

3. Diet: Eating foods high in trimethylamine, such as fish, eggs, and legumes, can increase the risk of developing severe primary trimethylaminuria.

4. Stress: Stress can trigger the release of hormones that can increase the production of trimethylamine in the body.

5. Certain medications: Certain medications, such as antibiotics, can interfere with the body’s ability to break down trimethylamine.

There is no cure for severe primary trimethylaminuria (TMAU). However, there are medications that can help reduce the symptoms of TMAU. These medications include antibiotics, antifungals, and probiotics. Additionally, dietary changes such as avoiding foods high in trimethylamine (TMA) can help reduce the symptoms of TMAU.