maple syrup disease

[Berdena K]

I have just started my last rotation in nursing school --OB. We need to select a topic for presentations. In the list of possible topics was "maple syrup disease". Does any one know what this is and if so have you had any clinical experience with it?

[noyesno, MSN, APRN, NP]

I have just started my last rotation in nursing school --OB. We need to select a topic for presentations. In the list of possible topics was "maple syrup disease". Does any one know what this is and if so have you had any clinical experience with it?

It's a disease where you can't break down branched chain amino acids. Your urine smells like maple syrup in result of this. I learned about it in my undergraduate degree as a nutrition major. I remember it being called, "maple syrup urine disease." Try looking it up under this name.

Good luck!

[NRSKarenRN, BSN, RN]

Maple syrup urine disease (MSUD) is an inherited metabolism disorder passed down through families in which the body cannot break down certain parts of proteins. Urine in persons with this condition can smell like maple syrup or burnt sugar.

A lifelong low protein diet is needed to prevent neurological damage. Infants require a special low protein amino acid formula.

The Old Order Mennonite/Amish have highest rate of this genetic condition,...especially in Lancaster County PA ..

Dr D Holmes Morton devoted most of his career to increasing understanding of this disease, especially testing procedures and treating children--- clinic link below.

See:

Maple syrup urine disease - Genetics Home Reference

Welcome to MSUD Family Support Group

The Clinic for Special Children