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KMID : 1189120160130010026
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대한의학유전학회지
2016 Volume.13 No. 1 p.26 ~ p.30
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Effectiveness of premarital screening program for thalassemia and sickle cell disorders in Ras Al Khaimah, United Arab Emirates
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Rasha Aziz Attia Salama
Abeer Kamal Saleh
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Abstract
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Purpose: Genetic disorders can be prevented by basic public health measures and activities that focus primarily on education and approaches in Primary Health Care. Premarital screening is one such approach that can identify asymptomatic carriers of hemoglobinopathies and provide genetic counseling to couples for a healthy reproductive life. This study aimed to estimate the prevalence of beta thalassemia and sickle cell disorders in the adult population screened as a part of the United Arab Emirates Premarital Screening Program and to measure the effectiveness of the program in decreasing high-risk marriages in Ras Al Khaimah (RAK).
Materials and Methods: A retrospective, population-based study was conducted at the RAK Primary Health Care Center, where the National Premarital Screening Program is implemented. The study included data collected from the premarital screening records of all couples who had applied for a marriage license during 2008-2015.
Results: Of the 17,826 individuals screened during the studied period, 4.02% (717) were diagnosed as positive for hemoglobinopathies. The prevalence of beta thalassemia and sickle cell disorders among the total study population was 2.98% and 1.05%, respectively. The hemoglobinopathy trait was more prevalent than the disease. Among these 8,913 couples who were issued certificates for compatibility based on screening tests, 28 (0.31%) couples were declared high-risk (unmatched). Seventy percent of these unmatched couples reported consanguineous marriages.
Conclusion: The program was successful in achieving its objective of identifying high-risk marriages. In spite of the counseling, however, all of the high-risk couples still married each other.
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KEYWORD
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Premarital screening, Beta thalassemia, Sickle cell disorders, United Arab Emirates
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