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.
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References
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Acknowledgments
We are thankful to Eman Megdad, Dana AlQasaby, and all the physicians for their contribution in the management of these patients.
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Communicated by: Avihu Boneh, MD, PhD, FRACP
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Synopsis
Successful management of pregnancy and delivery in these patients includes multidisciplinary approach with maintenance of adequate caloric and protein intake, hydration, electrolyte balance, and minimizing physiological stress.
Details of the Contributions of Individual Authors
RAS, MN, and ZNH devised the prenatal and perinatal management protocol.
RAS participated in the management of both patients, planned, and wrote the manuscript.
ZNH, MN, RK, BSH, and MA participated in the management of patients and contributed to the manuscript.
Corresponding Author: Raashda A. Sulaiman.
Guarantor: Zuhair N. Al-Hassnan.
RAS, MN, RK, BSH, MA, and ZNH declare that they do not have any competing interest.
No funding was required for this report.
Informed consent has been taken from the patients.
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Sulaiman, R.A., Al-Nemer, M., Khan, R., Almasned, M., Handoum, B.S., Al-Hassnan, Z.N. (2017). Successful Management of Pregnancies in Patients with Inherited Disorders of Ketone Body Metabolism. In: Morava, E., Baumgartner, M., Patterson, M., Rahman, S., Zschocke, J., Peters, V. (eds) JIMD Reports, Volume 38. JIMD Reports, vol 38. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2017_24
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DOI: https://doi.org/10.1007/8904_2017_24
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