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Table of Contents > Health Dictionary > Oasthouse urine disease

Oasthouse urine disease

An autosomal recessively inherited metabolic defect in the absorption of methionine that is converted by intestinal bacteria to a-hydroxybutyric acid; characterized by diarrhea, tachypnea, and marked urinary excretion of a-hydroxybutyric acid (causing an odor like that of an oasthouse).
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