Study of Some Predictors of Severe Hemolysis in Children with Glucose-6-Phosphate Dehydrogenase Deficiency

Abstract
Background: Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is one of the most common inherited disorders of mankind; more than 400 million people are affected worldwide, and it is a major health problem in Egypt.
Aim of Work: To study some predictors of severe hemolysis in children with glucose-6-phosphate dehydrogenase deficiency and the degree of DNA damage in these patients.
Patients and Methods: Seventy-two patients with G6PD deficiency admitted with acute hemolytic attacks to Diarb Negm Central Hospital-Sharkia Governorate, Egypt-over a period of 1 year from 1st May 2014 to 30th April 2015. We collected demographic data, details of signs and symptoms; special attention was given to history of fava bean ingestion, history of drug use or history of recent infection, history of neonatal jaundice, family history of G6PD deficiency disease, abdominal pain, vomiting or fever at admission and the results of tests for hemoglobin, reticulocytic count, hepatic function, G6PD enzyme activity and detection of DNA damage by DNA extraction, purification and agarose gel electrophoresis. We classified patients into mild and severe groups based on hemoglobin levels at admission. The mild cases were consid-ered as the control group.
Results: 72 children with G6PD deficiency admitted with acute hemolytic attacks were included in this study, 52 (72%) patients with severe hemolysis and 20 (28%) patients with mild hemolysis (control group). There were no statistically significant differences in many factors between the two groups, but some factors were detected to have statistical significance for severe hemolysis included younger age (p<.0001), male gender (p<.0001), presence of fever at admission (p<.0001), presence of vomiting (p<.0001) and abdominal pain (p<.0001) during the attack, higher Alkaline Phosphatase (ALP) (p <.0001), Aspartate Aminotransferase (AST) (p<.0001), Lactate Dehydrogenase (LDH) (p<.0001) and Total Serum Bilirubin (TSB) (p<.05). DNA damage was significantly higher in the severe cases compared to the mild cases (p<.0001).
Conclusion: Severe hemolysis can be predicted during hemolytic episodes in children with G6PD deficiency by young age, male gender, the presence of abdominal pain, vomiting and fever, high levels of ALP, AST, LDH enzymes and elevated TSB. This severe hemolysis predisposes patients to higher degree of DNA damage.