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Background: Children affected with sickle cell disease (SCD) are at increased risk for severe morbidity and mortality, especially during the first 3-5 years of life. It is suggested that early treatment can improve the condition. The aim of this pilot study was to estimate the incidence of hemoglobin S (HbS) by umbilical cord blood screening in Khorramshahr and Abadan cities in southwest of Iran. Materials and Methods: Cord blood sample (10 ml) was collected and stored in ethylenediaminetetraacetic acid (EDTA) precoated tubes and transported to the reference laboratory in Ahwaz. Samples were analyzed for presence of HbS by hemoglobin electrophoresis. Positive specimens were confirmed by sickle preparation test. Results: Four patients (1.3%) with sickle cell disease were identified among 308 screened newborns, all with Arabian ethnic origin. Conclusion: We concluded that HbS is a common disease in Khuzestan Province.

Keywords: Sickle cell anemia, Neonatal screening, Iran.

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