methionine malabsorption syndrome

methionine malabsorption syndrome
an autosomal recessive disorder of methionine absorption in which the urine has a characteristic odor resembling that of the interior of an oasthouse (room where tobacco, hops, and malt are dried). It is due to alpha-hydroxybutyric acid formed by bacterial action on the unabsorbed methionine. Characteristics include white hair, mental retardation, convulsions, and attacks of hyperpnea. Called also oasthouse urine disease and Smith-Strang disease.

Medical dictionary. 2011.

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