Anesthesia management in pediatric patient with glucose 6 phospate dehydrogenase deficiency

Year 2017, Volume: 42 Issue: 3, 552 - 555, 30.09.2017

Sedef Gülçin Ural , Meltem Aktay İnan

https://doi.org/10.17826/cutf.323982

Abstract

Glucose 6 phospate dehydrogenase (G6PD) deficiency is the most common enzyme deficiency. It is inherited in an X-linked recessive pattern and cause some disorders like hyperbilirubinemia in newborns, acut and chronic hemolysis. The first and main goal of the anethesia in this patient is to prevent oxidative stress. In this case we aim to share applying general anesthesia safely in patients with G6PD deficiency

Keywords

Glucose 6 phospate dehydrogenase deficiency, anesthesia management, pediatric patient.

Glukoz 6 fosfat dehidrogenaz eksikliği olan pediatrik hastada anestezi yönetimi

Abstract

Glukoz 6 fosfat dehidrogenaz (G6PD) enzim eksikliği en sık görülen enzim eksikliğidir. X e bağlı resesif geçişli olan bu hastalık yenidoğan hiperbilirübinemisi, akut veya kronik hemoliz gibi bir dizi bozukluklara neden olur. Bu hastalar için genel anestezinin ilk ve temel hedefi oksidatif stres gelişimini önlemektir. Bu vakayı G6PD enzim eksikliği bulunan hastalarda genel anestezinin güvenli bir şekilde uygulanabileceğini vurgulamak amacı ile paylaşmayı amaçladık.

Keywords

Glukoz-6-fosfat dehidrogenaz eksikliği, anestezi yönetimi, çocuk hasta

References

• 1. Reclos GJ, Hatzidakis CJ, Kruithof RA. G-6-PD Diagnosis: Modification of the standard method eliminates the need for an additional hemoglobin determination. Pharmakeftiki. 1999;12:25-31.
• 2. Glader BE. Glucose-6-phosphate dehydrogenase deficiency and related disorders of hexose monophosphate shunt and glutathione metabolism. In Wintrobe’s Clinical Hematology 10 th ed. (Eds GR Lee, J Foerster, J Lukens):1176-90. Baltimore, Williams & Wilkins, 2008.
• 3. Cheney CA, Lee A. Sequence of human G-6-PD cloned in plasmids and a yeast artificial chromosome (yac). Genomics. 1991;10:79-86.
• 4. Reclos GJ, Hatzidakis CJ, Schulpis KH. Glucose-6-phosphate dehyrogenase deficiency neonatal screening: preliminary evidence that a high percentage of partially deficient female neonates are missed during routine screening. J Med Screen. 2000;7:46-51.
• 5. Luzzatto L, Poggi V. Glucose 6 phosphate dehydrogenase deficiency and hemolytic anemia. In Nathan and Oski'sHematology of Infancy and Childhood, 7th ed. (Eds DG Nathan, SH Orkin):883-900. Philadelphia, Elsevier, 2009.
• 6. Salvati AM, Maffi D, Caprari P, Pasquino MT. G6PD deficiency and hereditary hemolytic anemia. Ann Ist Super Sanita. 1999;35:193-203.
• 7. Tamer I, Ünal B, Aksoy K. Glukoz-6-fosfat dehidrogenaz enzim eksikliği gözlenen olgularda eritrosit zarı Na+-K+/Mg++ 5’-trifosfataz, eritrosit süperoksit dismutaz ve plazma malondialdehit düzeyleri. Çukurova Üniversitesi Tıp Fakültesi Dergisi. 1998;23:114-8.
• 8. Beutler E. Screening for Glucose-6-Phosphate dehydrogenase deficiency. Israel J Med Sci. 1973;9:1350-2.
• 9. Valiaveedan S, Mahajan C, Rath GP, Bindra A, Manish MK. Anaesthetic management in patients with glucose-6-phosphate dehydrogenase deficiency undergoing neurosurgical procedures. Indian J Anaesth. 2011;55:68-70.
• 10. Elyassi AR, Rowshan HH. Perioperative management of the glucose-6- phosphate dehydrogenase deficient patient: a review of literature. Anesth Prog. 2009;56:86–91. 11. Chowdhry V, Bisoyi S, Mishra B. Perioperative challenges in a patient of severe G6PD deficiency undergoing open heart surgery. Ann Card Anaesth. 2012;15:50-3. 12. Altikat S, Ciftçi M, Büyükokuroğlu ME. In vitro effects of some anesthetic drugs on enzymatic activity of human red blood cell glucose 6-phosphate dehydrogenase. Pol J Pharmacol. 2002;54:67-71. 13. Büyükokuroğlu ME, Süleyman H. Glukoz 6-fosfat dehidrogenaz eksikliği. Turkiye Klinikleri Journal of Medical Sciences. 2001;21:415-9. 14. Younker D, DeVore M, Hartlage P. Malignant hyperthermia and glucose-6-phosphate dehydrogenase deficiency. Anesthesiology. 1984;60:601-3. 15. Smith CL, Snowdon SL. Anaesthesia and glucose-6-phosphate dehydrogenase deficiency: a case report and review of the literature. Anaesthesia. 1987;42:281-8. 16. Güneş Y, Özcengiz D, Özalevli M, Gündüz M, Işık G. Genel anestezi sırasında tanı konulan glukoz - 6 fosfat dehidrogenaz (G-6pd) Enzim Eksikliği. Çukurova Üniversitesi Tıp Fakültesi Dergisi. 2003;28:36-9.