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King syndrome in pregnancy
David E Abel1, Chad A Grotegut
1Department of Obstetrics and Gynecology, Duke University Medical Center, Durham, North Carolina 27710, USA. abel0002@mc.duke.edu
Obstetrics and Gynecology
|May 10, 2003
Summary
King syndrome management in pregnancy requires avoiding malignant hyperthermia triggers and addressing respiratory compromise due to myopathy. A multidisciplinary approach ensures optimal outcomes for mother and infant.
Area of Science:
- Genetics
- Obstetrics
- Anesthesiology
Background:
- King syndrome presents with myopathy, malignant hyperthermia susceptibility, and Noonan-like dysmorphic features.
- Pregnancy in women with King syndrome poses unique challenges due to potential complications.
Observation:
- A primigravida with King syndrome managed through pregnancy and delivery.
- Nocturnal mechanical ventilation via tracheostomy was required, with concerns for increasing respiratory support needs.
Findings:
- The patient underwent a successful forceps-assisted delivery at 35 weeks' gestation.
- Both mother and infant experienced positive outcomes and were discharged on postpartum day 3.
Implications:
- Avoidance of malignant hyperthermia-triggering agents is crucial in pregnant individuals with King syndrome.
- Proactive management of myopathy-related respiratory compromise and a multidisciplinary approach are essential for successful maternal-fetal outcomes.