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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