Burden of Hemoglobinopathies (Thalassemia, Sickle Cell Disorders and G6PD Deficiency) in Iran, 1990- 2010: Findings from the Global Burden of Disease Study 2010

Hemoglobinopathies are known as the most common genetic disorders in Iran. The paper aims to provide global estimates of death and disability adjusted life years (DALYs) due to hemoglobinopathies in Iran by sex and age during 1990 to 2010 and describe the challenges due to limitations of the GBD 2010 study. GBD 2010 estimates of the numbers of deaths and years of life lost (YLLs) due to premature mortality were calculated using the Cause of Death Ensemble model (CODEm). Years of life lost due to disability (YLDs) werecomputed by multiplication of prevalence, the disability weight for occurrence of sequelae, and the duration of symptoms. Prevalence was estimatedthrougha systematic search of published and available unpublished data sources, with a Bayesian meta-regression model developed for GBD 2010. Disability weights wereproducedusing collected data from population-based surveys. Uncertainty from all inputs was incorporatedinto the computations of DALYsusing simulation methods. We aim to prepare and criticizethe results of GBD 2010 and providesome recommendations for reaching better conclusions about the burden of hemoglobinopathies in Iran. Between 1990 and 2010, the overall deaths attributed to hemoglobinopathies decreased from 0.51% to 0.36% of total deaths, with the corresponding burden declining from 1% to 0.82% of total DALYs. There was a reduction in death and DALYs rates for all agesand the rates attributed to all ages followed the same pattern in Iranian men and women. The highest DALYs for hemoglobinopathies, thalassemia, sickle cell disorder, and glucose-6-phosphate dehydrogenase deficiency (G6PD-D) were found in those aged less than 5 years. The collective burden of all of these hemoglobin disorder was lower in 2010 than in 1990. The screening programs in Iran have been very successful in reducing the number of thalassemia patients. In ordertoprovide a better estimation of the burden of hemoglobin disorders, it is necessary to performa national and sub-national study of hemoglobinopathies using multiple national and sub-national surveys.