Glucose 6 phosphate dehydrogenase deficiency

G6PD deficiency is one of the most widespread genetic disorders in the world, affecting over 400 million people, mainly from the Mediterranean basin. Being an X-linked disease, it mainly affects boys, although girls can also be affected (de novo mutations). The WHO has classified the deficiency into 3 classes. Class 2 and 3 deficiencies are characterized by a risk of acute hemolytic shock due to oxidative stress.Outside the acute episode, management is based on preventive measures, avoiding drugs and foods that may precipitate an intravascular hemolysis crisis.

Autorentext

Senior Lecturer, Department of Pediatrics, CHU Hédi Chaker Sfax, Faculty of Medicine, Sfax TunisiaInter-university diploma in pediatric immuno-hematology, Claude Bernard Lyon Universitycoordinator of the Certificate of Complementary Studies in Pediatric Hematology and Immunology at the Sfax Faculty of Medicine