WEIRD SCIENCE

Baby disease that smells like maple syrup

THE peculiarly named maple syrup urine disease is a rare, potentially fatal condition that almost exclusively effects babies and infants.

The inherited disorder causes toxic levels of amino acids to build up in the blood which produce a sweet, maple syruplike odour in the urine and ear wax – hence the odd name.

The disorder affects about one in 185,000 births, and babies with it struggle to feed, are lethargic and, if left untreated, suffer seizures.

It can be managed with a special diet but sufferers tend to stay vulnerable to attacks which require hospitalisation.

The condition occurs more frequently among Mennonites in the US, though, affecting one in 380 births. Mennonites are a Christian denomination and are known to marry one another.