- A Case of Maple Syrup Urine Disease Associated with Acrodermatitis Enteropathica-like Syndrome Due to Iisoleucine Deficinecy During Diet Therapy
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Moon KH, Gwon OS, Lee JI, Rho SW, Jeon SS, Seo SS
- KMID: 2335536
- J Korean Pediatr Soc.
- 2001 Apr;44(4):469-474.
Maple syrup urine disease(MSUD) is an autosomal recessive disorder involving the metabolism of the branched-chain amino acids(BCAA) such as leucine, isoleucine and valine. The disorder is due to a defect... -
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