Hyperphenylalaninemia due to dehydratase deficiency

Overview

A rare metabolic disorder where the deficiency of an enzyme (pterin-4-alpha-carbinolamine dehydratase) can caused high blood phenylalanine levels. The condition is generally harmless and the disorder is usually only discovered accidentally in routine blood tests.

Symptoms

- Reduced muscle tone - High blood phenylalanine level - Delayed psychomotor skills - High blood phenylalanine level

Treatment

Limited phenylalanine consumption in diet