Predictors of severe hemolysis in patients with glucose-6-phosphate dehydrogenase deficiency following exposure to oxidant stresses

BACKGROUND AND OBJECTIVESGlucose-6-phosphate dehydrogenase (G6PD) deficiency is a genetic enzymatic disorder that affects millions of people worldwide, and is a major health problem in Jordan. We studied factors that may predict severe hemolysis in children with G6PD deficiency.METHODSWe reviewed the records of patients with low G6PD activity admitted to a teaching hospital between 1996 to 2007. We collected demographic data, details of sign and symptoms, history and type of fava bean ingestion, blood and Rh group, history of neonatal jaundice, history and type of drug use, abdominal pair at admission and the results of tests for hemoglobin, white blood cells (WBC), and hepatic function. We classified patients into mild and severe groups based on hemoglobin levels at admission.RESULTSOf 428 children with G6PD deficiency, 79 (18%) were severe cases and 349 (82%) patients with milt disease. There were no statistically significant differences in most factors between the two groups. Factors tha achieved statistical significance for severe hemolysis included younger age (P < .05), male gender (P < .05), highe alkaline phosphatase (ALP) (P < .05), presence of fever at admission (P < .01), presence of vomiting during the attack (P = .006), and a negative family history for G6PD deficiency (P = .005).CONCLUSIONSSevere hemolysis can be predicted during hemolytic episodes in children with low G6PD by young age, male gender, a negative family history of G6PD deficiency, the presence of fever and vomiting and a high ALP.