Tuesday, April 11, 2006

Not As Sweet As It Sounds

Diseases are not pleasant things, but sometimes you read about one that just ratchets up the grossness factor simply by virtue of its assigned designation. (That means it's name, kids.) A case in point is one that makes the news today: Maple Syrup Urine Disease (MSUD).

It sounds like something that affects one's bathroom output due to consumption of that stuff that a lot of us like to pour on pancakes. Not quite. From the MSUD Family Support Group Page, we learn that:

MSUD derives its name from the sweet, burnt sugar, or maple syrup smell of the urine. The disorder affects the way the body metabolizes (processes) certain components of protein. These components are the three branched-chain amino acids leucine, isoleucine, and valine. These amino acids accumulate in the blood causing a toxic effect that interferes with brain functions.

There's nothing sweet about the effects of the disease. The good news is that MSUD patients are receiving help in the form of liver transplants. Now, they can eat things that a lot of us take for granted, like cheeseburgers. It's a very happy development.

I would not blame these patients one bit if they opted not to chow down on pancakes dripping with Vermont's finest. I'm not to keen on the idea myself after reading about the main symptom.

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