Background: We aimed to determine the incidence of co-inheritance as well as interaction of sickle cell trait (SCT) and athal/bthal mutations in south and south central of Iran.Method: We employed a PCR and restriction fragment length polymorphism techniques to confirm diagnosis of sickle cell trait. All subjects were screened for any a/b –thalassemia mutations using a gap-polymerase chain reaction and amplification refractory mutations system.Results: Our results showed combination of sickle cell trait and b-globin mutation results in a severe clinical course of similar to sickle cell disease, while coinheritance of a-globin gene defects usually modulates the clinical course. A coexistence of sickle cell trait and a-globin gene mutation was the frequent genotype in overall samples (57.5%).Conclusion: Sickle cell trait mainly co-inherits with a-globin gene mutation in the south and south central region of Iran. This combination modulates hematological indices and interferes with the SCT diagnosis.

Background: Studies have demonstrated that sickle cell trait can be found in an asymptomatic healthy carrier with normal complete blood count (CBC) and red blood cell (RBC) indices. According to Iranian Ministry of Health bulletin instructions, prenuptial Thalassemia Screening Program (TSP) primarily depends on RBC indices which are measured through a routine CBC. Only when these levels are below the standard values in both couples, hemoglobin electrophoresis is performed. Because of normal values of RBC indices in the sickle cell trait, prenuptial TSP might miss it.Materials and Methods: To approach and prove this hypothesis, we carried out a prospective randomized pilot study of one year duration on 50 known cases of sickle cell trait (the parents of sickle cell and sickle-Thalassemia referral cases to Ahvaz Sickle Cell Center, affiliated to Ahvaz Jondishapour University of Medical Sciences). An informed written consent was obtained from all patients. Complete blood count was done including a blood film, and CBC was done by Helena Automated Cell Counter. Sickle preparation was done by freshly made sodium bisulfate 2%. It was read after one hour for the first time and was then read after 24 hours by macroscopic and microscopic methods. Hemoglobin electrophoresis was done by Helena apparatus. Serum ferritin was measured by Immunoradiometric Assay (IRMA). Iron deficient cases were treated by oral Iron agents.Results: Data of the first screening line showed MCV>80 and MCH>27 in more than 94% and HbA2 value of less than %3 in %100 of sickle cell trait cases.Conclusion: Sickle cell trait with normal indices might be skipped through pre-marriage TSP, and their carrier status is not determined without performing hemoglobin electrophoresis. We recommend mandatory hemoglobin electrophoresis and sickle preparation to be added to the first line of prenuptial Thalassemia Screening Program in the sickle target zones such as the southern parts of Iran.

Background: Early diagnosis and initiation of prophylactic penicillin and pneumococcal vaccine may reduce the risk of sickle cell complications that is a common hemoglobin disorder in Southwest Iran. This study aimed at determining the incidence of Sickle Cell Disease (SCD) and other Hemoglobinopathies in newborn being at risk based on ethnic origin.Materials and Methods: In this descriptive epidemiologic study, between September 2013 and September 2015, 8363 newborn blood samples were tested in four maternity units from Ahvaz, Khoramshahr, Sosangerd and Dezful. Complete cell count and cellulose acetate electrophoresis at pH 8.4 were performed on each blood sample. Parent's clinical status was also checked for more information. Presence of an abnormal band in the EDTA treated samples were further confirmed by citrate agar gel electrophoresis and automated high performance liquid chromatography (HPLC). Results were analyzed statistically by the One-Way ANOVA analysis.Results: Among 8363 screened samples, 118 (1.41 %) samples were heterozygous for Hb S, and four (0.047%) for Hb C; none of newborns were Hb SS homozygotes. The incidence of silent and alpha thalassemia minor based on RBC indices was nearly 10%.Conclusion: Present findings indicated the high quality and considerable impact of conducted screening program starting in 2007 at significantly decreasing the prevalence of SCD among newborns born between 2013 and 2015. The results also showed that the neonatal screening for SCD was not weighed to add as a new program in national health network.

A 43-year-old functionally monocular African American woman with longstanding type 2 diabetes mellitus presented for care of her better-seeing left eye. Originally suspected of having proliferative diabetic retinopathy (PDR) as the cause of her bilateral visual impairment, fluorescein angiography and optical coherence tomography angiography revealed a marked peripheral nonperfusion which was out of proportion for a typical diabetic retinopathy (Figure 1). A comprehensive uveitic and vasculopathic workup was therefore initiated. The workup was largely negative except for hemoglobin electrophoresis, which was consistent with the sickle cell trait (or hemoglobinopathy) (Table 1)...

Background: The cesarean delivery rate tends to be higher in women with sickle cell anemia (SCA) and sickle cell trait (SCT) for various reasons. However, pelvic contracture, which may lead to cesarean sections, has never before been studied in these groups. Objectives: The aim of this study was to investigate magnetic resonance (MR) pelvimetry findings of women with SCA and SCT. Patients and Methods: We studied 66 women who underwent MR pelvimetry between March and June 2015 at our center. Our prospective study included 21 women with SCA, 20 women with SCT, and 25 control women. Sagittal inlet, sagittal mid-pelvis, transverse inlet, transverse mid-pelvis and transverse outlet diameters were measured on MR images. The mean diameters were compared with each other and with the control group Results: Sagittal inlet and transverse inlet were not significantly different between the groups. Also, mid-pelvic diameters for the sagittal and transvers mid-pelvis were similar between the groups. Even though the transverse outlet seems to be lesser in the SCA group compared with other groups, it was not statistically significant. Conclusion: Our study demonstrated that the pelvic roofs of women with SCA and SCT are indistinguishable from healthy women. Thereby, maternal pelvic structures that impact delivery types may not be significantly changed in SCA and SCT diseases. We believe that when making decisions about modes of delivery, these results should be taken into consideration.