Maple syrup urine disease (MSUD, MIM #248600) is an autosomal recessive metabolic disorder that results in elevation of the branched-chain amino acids (BCAA) leucine, isoleucine, and valine. Elevation of BCAA and certain alpha keto-acids is associated with a catabolic state and may result in neurological and developmental delays, feeding problems, and a urine and cerumen odor of maple syrup. Pregnancy is a period of multiple adaptations necessary to support fetal growth and development. Both the third trimester of pregnancy and the postpartum period present the possibility for catabolic states. We describe our treatment of an adolescent patient with intermittent MSUD and her resulting positive pregnancy outcome.

枫糖浆尿病（MSUD，MIM＃248600）是一种常染色体隐性代谢疾病，可导致支链氨基酸（BCAA）亮氨酸，异亮氨酸和缬氨酸升高。BCAA和某些α-酮酸的升高与分解代谢状态有关，并可能导致神经和发育延迟，进食问题以及枫糖浆的尿液和陶粒气味。怀孕是支持胎儿生长发育所必需的多种适应期。妊娠晚期和产后期都可能出现分解代谢状态。我们描述了我们对一名患有间歇性MSUD的青春期患者的治疗，以及由此产生的积极妊娠结局。