Management of a Woman With Maple Syrup Urine Disease During Pregnancy, Delivery, and Lactation

被引：13
作者：
Wessel, Ann E. [1 ]
Mogensen, Kris M. [2 ]
Rohr, Frances [1 ]
Erick, Miriam [2 ]
Neilan, Edward G. [3 ,4 ]
Chopra, Sameer [5 ]
Levy, Harvey L. [3 ,4 ]
Gray, Kathryn J. [6 ]
Wilkins-Haug, Louise [6 ,7 ]
Berry, Gerard T. [3 ,4 ]
机构：
[1] Boston Childrens Hosp, Ctr Nutr, Boston, MA 02115 USA
[2] Brigham & Womens Hosp, Dept Nutr, Boston, MA 02115 USA
[3] Boston Childrens Hosp, Div Genet & Genom, Boston, MA USA
[4] Harvard Univ, Sch Med, Dept Pediat, Boston, MA 02115 USA
[5] Brigham & Womens Hosp, Dept Med, Boston, MA 02115 USA
[6] Brigham & Womens Hosp, Div Maternal Fetal Med & Reprod Genet, Boston, MA 02115 USA
[7] Harvard Univ, Sch Med, Dept Obstet Gynecol & Reprod Biol, Boston, MA 02115 USA
来源：
JOURNAL OF PARENTERAL AND ENTERAL NUTRITION | 2015年 / 39卷 / 07期
基金：
美国国家卫生研究院;
关键词：
maple syrup urine disease; MSUD; parenteral nutrition; pregnancy; lactation; BODY-MASS INDEX; CARDIAC SURGICAL-PATIENTS; LENGTH-OF-STAY; RISK-FACTORS; NOSOCOMIAL INFECTIONS; PROLONGED VENTILATION; SURGERY; PREALBUMIN; PREDICTORS; MORTALITY;
D O I：
10.1177/0148607114526451
中图分类号：
R15 [营养卫生、食品卫生]; TS201 [基础科学];
学科分类号：
100403 ;
摘要：
Maple syrup urine disease (MSUD) is an inherited disorder of metabolism of the branched-chain amino acids leucine, isoleucine, and valine. Complications of acute elevation in plasma leucine include ketoacidosis and risk of cerebral edema, which can be fatal. Individuals with MSUD are at risk of metabolic crisis throughout life, especially at times of physiological stress. We present a case of successful management of a woman with MSUD through pregnancy, delivery, postpartum, and lactation, including nutrition therapy using modified parenteral nutrition.
引用
收藏
页码：875 / 879
页数：5
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