Successful Management of Pregnancies in Patients with Inherited Disorders of Ketone Body Metabolism

  • Raashda Ainuddin Sulaiman
  • Maha Al-Nemer
  • Rubina Khan
  • Munirah Almasned
  • Bedour S. Handoum
  • Zuhair N. Al-Hassnan
Research Report
Part of the JIMD Reports book series (JIMD, volume 38)

Abstract

Patients with succinyl-CoA:3-oxoacid CoA transferase (SCOT) deficiency and 3-hydroxy-3-methylglutaryl (HMG)-CoA lyase deficiency are at increased risk of developing metabolic acidosis and hypoglycemia during pregnancy, delivery, and postpartum period. This can be fatal if not treated appropriately. Pregnancy in such patients should be managed in a specialist center by a multidisciplinary team including metabolic physician, high-risk obstetrician, and metabolic dietician. We report two pregnancies in women with SCOT deficiency and HMG-CoA lyase deficiency, which were successfully managed at this tertiary care center. The patient with SCOT deficiency had recurrent ketoacidosis due to severe nausea and vomiting requiring several hospital admissions during pregnancy, while the patient with HMG-CoA lyase deficiency remained metabolically stable. Both patients, nevertheless, had normal delivery of live-born infants and had uneventful postpartum period.

Keywords

HMG-CoA-lyase deficiency Metabolic acidosis Pregnancy SCOT deficiency 

Notes

Acknowledgments

We are thankful to Eman Megdad, Dana AlQasaby, and all the physicians for their contribution in the management of these patients.

References

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Copyright information

© SSIEM and Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  • Raashda Ainuddin Sulaiman
    • 1
    • 2
  • Maha Al-Nemer
    • 3
  • Rubina Khan
    • 3
  • Munirah Almasned
    • 4
  • Bedour S. Handoum
    • 4
  • Zuhair N. Al-Hassnan
    • 1
    • 2
  1. 1.Department of Medical GeneticsKing Faisal Specialist Hospital and Research CentreRiyadhSaudi Arabia
  2. 2.College of MedicineAlfaisal UniversityRiyadhSaudi Arabia
  3. 3.Department of Obstetrics and GynecologyKing Faisal Specialist Hospital and Research CentreRiyadhSaudi Arabia
  4. 4.Department of Nutrition ServicesKing Faisal Specialist Hospital and Research CentreRiyadhSaudi Arabia

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