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Department od  Gynaecology  and  Obstetrics , University  Hospital  Ostrava Head  od Department: doc. MUDr. Ondřej Šimetka, Ph.D., MBA

Photo by The New York Public Library on Unsplash

Ornithine transcarbamylase deficiency –  life-threatening complications in pregnancy and  postpartum period 

Pavla Miřátská,  Richard Špaček,  Martina Romanová,  Ondřej šimetka. University hospital Ostrava, Czechia 

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  - 27-year-old G2P1 - VACTERL association –  multiple reconstruction surgeries in childhood - caesarean section 2016 - scoliosis – previous neurosugical and  orthopedic surgeries - gestational DM – dietary measures - ornithin transcarbamylasr deficiency –  heterozygote – no dietary restrictions - hypoacusis 

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Photo by Reproductive Health Supplies Coalition on Unsplash

Photo by RhondaK Native Florida Folk Artist on Unsplash

For orthopedic indication scheduled  a C-section after 39th week Two days later – PROM and  onset of uterine contractions  emergency C-section performed , Glucose i.v. in peri- and postoperative period   

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- Glucose i.v. early post partum  Monitoring of ammonium level - Uncomplicated course Newborn – a boy, OTC deficiency confirmed , transfer to specialized metabolic center  

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 26-year-old G1P0, g.w. 35+4 OTC deficiency – no dietary restrictions GDM – diet  preeclampsia Induction of labour at week 37+0,  uncomplicated labour, Glucose i.v. administered, uncomplicated postpartum period  Newborn – a girl, OTC heterozygote   

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 Rare X-linked disease  Pregnancy as a possible trigger  Symptoms mimic postpartum depression  Several case studies resulting in maternal death   

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 Personal and family history  Preventive measures  Cautious monitoring  Do not underestimate!   

Ornithine transcarbamylase deficiency – life-threatening complications in pregnancy and postpartum period

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