Baby with a portal shunt and later trimethylaminuria

http://www.ncbi.nlm.nih.gov/pubmed/9069234

A case of congenital portal-systemic shunting due to an intrahepatic connection diagnosed by ultrasound scanning and color Doppler in an 8-month-old girl is reported. She began to manifest trimethylaminuria 3 years later. At 7 years of age, she is asymptomatic without therapeutic measures except for diet. This is the seventh reported case and the third in a child to our knowledge.

This paper from 1997 is a case study about a baby girl who was diagnosed as having a hepatic portal shunt. This means her absorbed gut fluid (good and bad) is going directly into the systemic blood system unfiltered. Normally the absorbed fluid from the gut goes into the portal vein and then is carried to the liver to be filtered (often by the P450 and FMO3 enzymes etc). In her case there is a connection between the portal vein and the systemic system, so it bypasses the liver. This means her systemic system is subject to 'untreated' gut blood.

They say around age 3 she developed trimethylaminuria but it could be controlled by diet. One point to remember is that probably her liver metabolizing enzymes were normal. However, who knows what the girl would say what she smelt/smells of ? She would be perhaps around 19 now.

Another interesting popint is that medical students will be taught that someone with a portal shunt will be likley be on their death bed, whereas she seems to be fine. They say this is the 7th reported case in pubmed. Again proving the theory that people can have all sorts of gut metabolites in their systemic system and be 'healthy'.

It's very unlikely that most sufferers of metabolic body odor (including fecal body odor) have a portal shunt.