Mortality rate with maple syrup urine disease?

Varies: This autosomal recessive genetic disorder of branched chain ketoaciduria requires testing and strict dietary adherence for management. If this is followed, individuals can lead normal lives with excellent health, exempting them from the neurologic complications which are responsible for morbidity and mortality.

Depends: The earlier the disease is detected and the patient is placed on an appropriate diet, the better the long term prognosis is.