Management and communication problems in a patient with succinyl-CoA transferase deficiency in pregnancy and labour

International Journal of Obstetric Anesthesia

Volumn 18, Issue 3, 2009, Pages 280-283

  Merron, S ^a   Akhtar, R ^a  

^a UNIVERSITY HOSPITAL OF NORTH STAFFORDSHIRE   (United Kingdom)

Author keywords

Epidural analgesia; Pregnancy; Succinyl CoA transferase deficiency

Indexed keywords

3 OXOACID COENZYME A TRANSFERASE; BICARBONATE; BUPIVACAINE; FENTANYL;

3 OXOACID COENZYME A TRANSFERASE DEFICIENCY; ADULT; ARTICLE; CALORIC INTAKE; CASE REPORT; CLINICAL FEATURE; ELECTROLYTE BALANCE; ENZYME DEFICIENCY; EPIDURAL ANESTHESIA; FATTY ACID METABOLISM; FEMALE; FLUID THERAPY; GESTATIONAL AGE; HUMAN; LABOR; LABOR INDUCTION; OBSTETRIC ANESTHESIA; PHOBIA; PREGNANCY; PRENATAL CARE;

ADOLESCENT; ANESTHESIA, EPIDURAL; ANESTHESIA, OBSTETRICAL; ANESTHESIOLOGY; COENZYME A-TRANSFERASES; ENDOCRINOLOGY; FEMALE; HUMANS; INTERDISCIPLINARY COMMUNICATION; LABOR, INDUCED; PREGNANCY; PREGNANCY COMPLICATIONS; TREATMENT OUTCOME;

EID: 67649472452     PISSN: 0959289X     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.ijoa.2009.02.001     Document Type: Article

References (16)

1. Tildon J.T., and Cornblath M. Succinyl-CoA:3-ketoacid CoA-transferase deficiency. A cause for keto-acidosis in infancy. J Clin Invest 51 (1972) 493-498
2. Spence M.W., Murphy M.G., Cook H.W., Ripley B.A., and Embil J.A. Succinyl-CoA:3-ketoacid CoA-transferase deficiency: a "new phenotype". Pediatr Res 7 (1973) 394
3. Saudubray J.M., Specola N., and Middleton B. Hyperketotic states due to inherited defects of ketolysis. Enzyme 38 (1987) 80-90
4. Sakazaki H., Hirayama K., Murakami S., and Yonezawa S. A new Japanese case of succinyl-CoA:3-ketoacid CoA-transferase deficiency. J Inherit Metab Dis 18 (1995) 323-325
5. Pretorius C.J., Loy Son G.G., Bonnici F., and Harley E.H. Two siblings with episodic ketoacidosis and decreased activity of succinyl-CoA:3-ketoacid CoA-transferase in cultured fibroblasts. J Inherit Metab Dis 19 (1996) 296-300
6. Snyderman S.E., Sansaricq C., and Middleton B. Succinyl-CoA: 3-Ketoacid CoA-Transferase Deficiency. Pediatrics 101 (1998) 709-711
7. Baric I., Sarnavka V., Fumic K., et al. A new case of succinyl-CoA:acetoacetate transferase deficiency: favourable course despite very low residual activity. J Inherit Metab Dis 24 (2001) 81-82
8. Berry G.T., Fukao T., Mitchell G.A., et al. Neonatal hypoglycaemia in severe succinyl-CoA:3-oxoacid CoA-transferase deficiency. J Inherit Metab Dis 24 (2001) 587-595
9. Fukao T. Succinyl-CoA:3-ketoacid (SCOT) transferase deficiency. Orpha.net (internet resource) 2004; www.orpha.net/data/patho/GB/uk-scot.pdf.
10. Kubli M., Scrutton M.J., Seed P.T., and O'Sullivan G. An evaluation of isotonic "sport drinks" during labor. Anesth Analg 94 (2001) 404-408
11. Fukao T., Mitchell G.A., Song X.-Q., et al. Succinyl-CoA: 3-ketoacid CoA transferase (SCOT): cloning of the human SCOT gene, tertiary structural modeling of the human SCOT monomer, and characterization of three pathogenic mutations. Genomics 68 (2000) 144-151
12. Fukao T., Song X.-Q., Mitchell G.A., et al. Enzymes of ketone body utilization in human tissues: protein and mRNA levels of succinyl-CoA:3-ketoacid CoA transferase and mitochondrial and cytosolic acetoacetyl-CoA thiolases. Paediatr Res 42 (1997) 498-502
13. Fukao T., Shintaku H., Kusubae R., et al. Patients homozygous for the T435N mutation of succinyl-CoA: 3-ketoacid CoA transferase (SCOT) do not show permanent ketosis. Pediatr Res 56 (2004) 858-863
14. Hardardottir H., Lahiri T., and Egan J.F. Renal tubular acidosis in pregnancy: case report and literature review. J Matern Fetal Med 6 (1997) 16-20
15. Singhi S. Effect of maternal intrapartum glucose therapy on neonatal blood glucose levels and neurobehavioral status of hypoglycemic term newborn infants. J Perinat Med 16 (1988) 217-224
16. Mendiola J., Grylack L.J., and Scanlon J.W. Effects of intrapartum maternal glucose infusion on the normal fetus and newborn. Anesth Analg 61 (1982) 32-35