Molecular, Biochemical and Hematological Investigations of b-Thalassemic Children in Gaza Governorate

Background: Thalassemias are hereditary anemias mostly common in the Mediterranean, the equatorial, or near equatorial regions of Africa and Asia. They are classified according to which particular globin chain(s) is/are produced in a reduced amount: a, b, db, d, and gd thalassemias. In the Gaza Strip, more than 300 patients have been diagnosed with β-thalassemia major, they are currently being transfused and managed in local hospitals. Aims: To investigate molecular, biochemical and hematological aspects of β-thalassemic children aged 5-12 years in Gaza City. Methodology: Blood samples were collected from 53 β-thalassemic children who are transfused and managed at the pediatric hospitals at Gaza City. Blood withdrawals were performed just before the scheduled blood transfusion. In addition blood samples were also collected from 53 apparently healthy children. Cases and controls were age and sex matched. Part of data was collected by using close-ended questionnaire. Complete blood count and biochemical tests were performed. Screening for possible mutations in HBB gene was performed at the molecular medicine laboratories of the Bernhard Nocht Institute (BNI), Germany, according to Dynamic Allele-Specific Hybridization (DASH) method. This work was performed according to the cross-sectional descriptive study design. An official approvals letters were obtained from Helsinki committee at the Palestinian ministry of health and from the Palestinian Thalassemia Center who approved performing the study on the thalassemic children. The data were tabulated, encoded and statistically analyzed using the IBM SPSS Statistics (version 17, IBM Corporation, Somers, NY). The Chi square test, the independent-samples t-test, and One-Way analysis of variance (ANOVA) were performed aiming at the description, identification of significant relationship, correlations and differences between the study items, variables and parameters. A p-value < 0.05 was considered statistically significant. Results: A significant difference was reported in the parents' consanguinity of the two groups (P=0.001). About 71% of the β-thalassemia major children parents are 1st degree cousins compared to the control group where the percentage is