Oasthouse urine disease

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oasthouse urine disease

An inherited metabolic defect in the absorption of methionine which is converted by intestinal bacteria to alpha-hydroxybutyric acid; characterised by diarrhoea, tachypnea, and marked urinary excretion of alpha-hydroxybutyric acid (causing an odour like that of an oasthouse).

Origin: oast, kiln for drying hops, malt, or tobacco