Sanjurjo P, Centeno C, Vallo A, Ojembarrena E, Rodríguez Soriano J
An Esp Pediatr. 1983 Nov;19(5):389-92.
A case of maple syrup urine disease is described presenting during diet therapy a clinical picture with predominant cutaneous symptoms attributed to a dietary deficiency. It is believed that probable cause was the coexistence of low serum levels of isoleucine with still elevated levels of leucine. Given the metabolic interrelationship existing between different branch-chained amino acids, an independent dietary correction of each amino acid is recommended.
本文描述了一例枫糖尿症患者，在饮食治疗期间出现以皮肤症状为主的临床表现，这些症状归因于饮食缺乏。据信，可能的原因是血清异亮氨酸水平低与亮氨酸水平仍升高并存。鉴于不同支链氨基酸之间存在代谢相互关系，建议对每种氨基酸进行独立的饮食调整。
