id author title date pages extension mime words sentences flesch summary cache txt work_zvmri2xdbrcjjfwqemsclx7n6u Sarah Catharina GrĂ¼nert Successful pregnancy in maple syrup urine disease: a case report and review of the literature 2018 7 .pdf application/pdf 5070 1952 85 Successful pregnancy in maple syrup urine disease: a case report and review of the literature Background: Maple syrup urine disease (MSUD) is an autosomal recessive disorder of branched-chain amino acid trimester until delivery, protein and leucine intake increased continuously to about threefold compared to prepregnancy values. To maximize patient safety during delivery and the postpartum period, a detailed plan including Conclusions: Management of pregnancy, delivery, postpartum period and lactation may be challenging in patients Keywords: Maple syrup urine disease, MSUD, Pregnancy, Leucine tolerance, Protein tolerance, Branched-chain amino Only few cases of pregnancies in MSUD patients have Additionally, we provide a review of the literature including all cases of pregnancies in MSUD patients published As the patient lived quite far from the metabolic center delivery was planned as Cesaerean section Only nine cases of pregnancy in classical MSUD patients Grossly elevated leucine levels throughout the whole course of pregnancy have also been reported in 2 cases by Tchan et al. ./cache/work_zvmri2xdbrcjjfwqemsclx7n6u.pdf ./txt/work_zvmri2xdbrcjjfwqemsclx7n6u.txt