A G Rajab1, M A Patton, B Modell. 1. Genetic Blood Disorders Unit, DGHA, Ministry of Health, PO Box 880, Muscat 113, Sultanate of Oman. drarajab@omantel.net.om
Abstract
OBJECTIVES: A national register of symptomatic hemoglobinopathies has been developed in Oman to facilitate the development of the National Program for the control of genetic blood disorders. METHODS: The information was initially collected retrospectively through hospital records and was refined prospectively with data collected through a survey of pediatricians. The percentages of heterozygotes in different population groups and geographical locations, birth prevalence, age distribution of cases and factors determining frequencies of Hemoglobinopathies in different regions of the country were studied from the register. RESULTS: The register has identified 1757 cases of homozygous Sickle Cell Anemia and 243 cases of beta-thalassemia major in a population of 1.5 million in 1995. Register based national figures of heterozygote carriers approximate 10% for Sickle Cell Anemia and 4% for beta-thalassemia major. CONCLUSION: Defining regional and tribal variations can assist efficient targeting of health resources. This approach provides a simple model for other countries or regions to follow providing there is a health care system that facilitates registration.
OBJECTIVES: A national register of symptomatic hemoglobinopathies has been developed in Oman to facilitate the development of the National Program for the control of genetic blood disorders. METHODS: The information was initially collected retrospectively through hospital records and was refined prospectively with data collected through a survey of pediatricians. The percentages of heterozygotes in different population groups and geographical locations, birth prevalence, age distribution of cases and factors determining frequencies of Hemoglobinopathies in different regions of the country were studied from the register. RESULTS: The register has identified 1757 cases of homozygous Sickle Cell Anemia and 243 cases of beta-thalassemia major in a population of 1.5 million in 1995. Register based national figures of heterozygote carriers approximate 10% for Sickle Cell Anemia and 4% for beta-thalassemia major. CONCLUSION: Defining regional and tribal variations can assist efficient targeting of health resources. This approach provides a simple model for other countries or regions to follow providing there is a health care system that facilitates registration.
Authors: Mohamad H Qari; Yasser Wali; Muneer H Albagshi; Mohammad Alshahrani; Azzah Alzahrani; Ibrahim A Alhijji; Abdulkareem Almomen; Abdullah Aljefri; Hussain H Al Saeed; Shaker Abdullah; Ahmad Al Rustumani; Khoutir Mahour; Shaker A Mousa Journal: Orphanet J Rare Dis Date: 2013-09-17 Impact factor: 4.123