| 英文描述 | Hereford-type and Polled Shorthorn calves have been affected by 'maple syrup urine' disease. Inherited as an autosomal recessive, and caused by a deficiency of branched chain keto acid dehydrogenase activity. Calves are reported to be normal at birth but 1-3 days later become dull and recumbent, with opisthotonus, and are dead in less than a week. Urine ketone levels are high, and urine has a distinct aroma of burnt sugar. A yellowish stain can become apparent on the white hairs of the coat. CNS tissue has an elevated ratio of branched to straight chain amino acids. Genotyping can be used for diagnosis. |
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