Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + At a Glance Download Section PDF Listen ++ An inherited disease caused by malabsorption of methionine and secondary malabsorption of other amino acids. The unabsorbed methionine is converted to α-hydroxybutyric acid by intestinal bacteria. Urine has characteristic smell of dried malt. Clinical characteristics include mental retardation, seizures, white hair, diarrhea, and episodes of generalized edema. + Incidence and Genetic Inheritance Download Section PDF Listen ++ Only a single case described. Autosomal recessive. + Clinical Aspects Download Section PDF Listen ++ Patient had white hair, rapid breathing, convulsions, and mental retardation. Urine had characteristic oasthouse smell. Another patient who may have had this condition had mental retardation, diarrhea, convulsions, and white hair. + Anesthetic Considerations Download Section PDF Listen ++ Anesthetic management in this condition has not been described. No particular problems are anticipated based on the clinical description. Agents that may precipitate seizures, such as enflurane, should be avoided. + References Download Section PDF Listen ++Ulshen M: Malabsorptive disorders, in Behrman RE, Kliegman RM, Arvin AM (eds): Nelson Textbook of Pediatrics. 15th ed. Philadelphia, WB Saunders, 1995.