ASSOCIATION OF THALASSEMIA MAJOR WITH CONSANGUINEOUS MARRIAGES IN THE PROVINCE OF KHYBER PAKHTUNKHWA- A CASE CONTROL STUDY

BACKGROUND: Beta Thalassemia is the commonest genetic disorder worldwide. It is one of thetypes of hemoglobinopathies which results from either deficient synthesis or complete loss of one of theBeta chains of hemoglobin. Children with Thalassemia major or minor might have defective genes intheir parents. Consanguineous marriages are important risk factors in transmission of thalassemia fromparents to their children. So avoidance of cousin marriages can prevent thalassemia in children.Premarital counseling with couples having thalassemia trait can be an important step in prevention ofthalassemia. Pre-marriage chromosomal studies can be useful in prevention of thalassemia.OBJECTIVE: The main objective of the study is to establish an association of cousin marriages withthalassemia major in the Pathan community of Khyber Pakhtunkhwa.CONCLUSION: Thalassemia is the commonest genetic disorder in the world. It is more prevalent inthose families where cousin marriages are frequent. The same is situation in the province of KPK whereprevalence of cousin marriages is more than 55 %.RESULTS: Total 180 cases and controls were analyzed with 1:1 ratio. Out of the total 109 (60%) weremale children while 71 (40%) were female children. Father literacy rate was 54% and mother literacyrate was 18%. 66% parents of the cases were knowledgeable about the disease. Association of cousinmarriages with thalassemia major was significant with an odds ratio of 2.25 (Cl 95%, P value 0.05).Rest of the variables had no significant association with thalassemia major.Key words: Thalassemia, Consanguineous Marriages