Maple syrup urine disease
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A genetic metabolic disorder of branched-chain amino acids (leucine, isoleucine, valine), a type of ketonuria. The condition begins a few days after birth. These amino acids and the ketone bodies derived from them are excreted in the urine, causing symptoms such as acidosis, convulsions, and coma, leading to death. The disease was named after the characteristic maple syrup-like odor of the urine. Congenital metabolic disorder [Ryōmasa Nakamura] Source: Heibonsha World Encyclopedia, 2nd Edition Information |
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分枝鎖アミノ酸(ロイシン，イソロイシン，バリン)の遺伝性代謝障害で，ケトン尿症の一つ。生後数日に発病する。尿中にこれらのアミノ酸やそれから誘導されるケトン体が排出され，アシドーシス，痙攣（けいれん），昏睡の症状を示し，死に至る。メープルシロップ様の尿臭が特徴で，この病名がつけられた。先天性代謝異常【中村 了正】
出典 株式会社平凡社世界大百科事典 第２版について 情報 |
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