Glucose-6-phosphate dehydrogenase activity in Jordanian female children with acute hemolytic crises

ABSTRACT

The study objective is to determine the mode of inheritance in Glucose-6-Phosphate Dehydrogenase [G6PD]-deficient female children who have a history of acute hemolytic crises. Also, it aims to study the relationship between the type of inheritance and the severity of hemolysis, and to determine the factors incriminated in provoking hemolytic crises on them. The study included 40 female children with confirmed diagnosis of G6PD deficiency who were admitted to Princess Rahma Teaching Hospital with acute hemolytic crisis between June 2002 and April 2005. G6PD enzyme levels were studied in their mothers and in thirty-seven fathers to determine the mode of inheritance. In addition, 45 unaffected volunteers were evaluated as a control group. The results showed that 65% were heterozygous females and 35% were homozygous for G6PD deficiency. The mean G6PD level for homozygous females was 4.79 +/- 2.67 mU/10[9] RBCs compared to 54.1 +/- 23.2 mU/10[9] RBCs in heterozygous females. In 92.5% of children there was a history of fava beans ingestion before crisis. The mean time for appearance of symptoms after ingestion of fava beans was 10.57 +/- 4.99 hours compared to 23.77 +/- 5.23 hours in heterozygous females, and the duration of hemolytic crises was higher in homozygous females 78 +/- 12.2 hours compared to 53 +/- 10 hours in heterozygous females. Two breast-feed infants gave history of fava beans ingestion by their mothers before crisis. This study demonstrated that females with G6PD activity level of less than 10% are usually homozygous females with rapid onset of symptoms and longer duration of hemolytic crisis following exposure to triggering factors, especially uncooked green beans, whose metabolites may be excreted in breast milk