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oasthouse urine disease


Definition: 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).

[oast, kiln for drying hops, malt, or tobacco]

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.