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Table 1 Overview on all pregnancies in MSUD mothers published in the literature

From: Successful pregnancy in maple syrup urine disease: a case report and review of the literature

  MSUD subtype presentation in metabolic outpatient clinic (week/month of gestation) compliance during pregnancy leucine tolerance before pregnancy (mg/kg/d) natural protein tolerance before pregnancy maximum leucine tolerance during pregnancy (mg/kg/d) maximum protein tolerance during pregnancy mode of delivery peripartal infusion therapy discharge from hospital (day postpartum) offspring breastfeeding additional information Reference
1 classical 2 months good n.a. 0.6-0.8 g/kg/d n.a. 1.5 g/kg/d spontaneous, 40 weeks of gestation yes n.a. healthy no slowed fetal growth in week 37 with concomitant low BCAA levels, 9 days postpartum dizziness and lethargy, leucine level 1015 μmol/l Van Calcar 1992 [5]
2 classical 6th week good n.a., 350-750 n.a. 2100 n.a. spontaneous, 36 weeks of gestation no n.a. healthy yes leucine peak of 1100 μmol/l on day 9 postpartum Grünewald 1998 [8]
3 n.a. 24th week good n.a. n.a. n.a. 1.2 g/kg/d spontaneous, 36 weeks of gestation n.a. day 16 healthy n.a. elevated BCAA levels postpartum, death of the mother on day 51 postpartum, bilateral pulmonary contusions and brain edema Yoshida 2003 [7]
4 classical 10 weeks poor n.a. 15–35 g/d n.a. n.a. spontaneous, 40 weeks of gestation n.a. day 8 healthy n.a. metabolic decompensation with several seizures and unconciusness in week 14 of gestation; mastitis on day 12 post partum, leucine level 549 μmol/l. Tchan 2013 [10]
5 classical 27 weeks poor n.a. 30-50 g/d n.a. n.a. emergency Caesarean section due to rupture of membranes and failure to progress, 41 weeks of gestation n.a. selfdischarged against medical advice on day 12 healthy n.a. Poor compliance with leucine levels typically 700–1300 μmol/l; acute febrile illness and threatened premature labour in week 28 of gestation; mineral, vitamin and protein deficiency diagnosed durig pregnancy Tchan 2013 [10]
6 classical 10 weeks good 600-900 n.a. 2400 n.a. induced labour in week 37, chorioamnionitis and failure to progressafter 48 h, secondary Caesarean section yes day 10 healthy yes mild nausea and vomiting in early pregnancy; mild increase in blood pressure and concerns about preeclampsia in week 36; 4 episodes of elevated leucine levels up to 1082 μmol/L during lactation Wessel 2015 [4]
7 classical 7th week good 300-500 5 g/d 3000 30 g/d spontaneous, 41 weeks of gestation yes day 5 healthy yes no complications Heiber 2015 [9]
8 moderate n.a. n.a. n.a. 30 g/d n.a. n.a. n.a. n.a. n.a. healthy n.a. same mother, first pregnancy of the mother not reported in deteil, but „was managed in a similar way to the second one“; persistent nausea throughout the pregnancy Brown 2017 [6]
9 moderate n.a. good n.a. 30 g/d n.a. 60 g/d spontaneous, 40 weeks of gestation no day 4 healthy no Brown 2017 [6]
10 classical 7th week good 750 5-10 g/d 2400 30 g/d Caesarean section, 38 weeks of gestation yes day 7 healthy yes no complications this case
  1. n.a. not available