Sickle cell disease is a genetic, hereditary and chronic disease that affects the health of its carriers and might impair their health-related quality of life. The aim of the current study was to determine the sickle cell trait frequency in Sudanese patient living in Heglig area in Western of Southern Kordofan state from November 2008 to February 2009. An analytical, descriptive and cross-sectional study conducted for one hundred participants who had confirmed patient diagnosed as (Hb S disease) homozygosis patient,2-5 mL of venous blood was collected for the measurements of complete blood count, sickling test and Hb electrophoresis. Demographic data and family history were collected in a pre designed questionnaire with written consent all participants. In this study the frequency of sickle cell trait and sickle cell disease were (52%) and (14%) respectively and 34% were normal. The sickling test showed that 71 % of the study population were negative sickling test, the remaining 29 % were positive. The total erythrocytes was significantly decreased in sickle cell disease (p< 0.000) compared with normal and sickle cell trait, also the hemoglobin concentration and packed cell volume were significantly lower than that of normal individuals and sickle cell trait patients. The frequencies of sickle cell trait was higher among the participants and patients of sickle disease showed lower values of red blood cells parameters , but higher values of white blood cells and platelets compared to haemoglobin phenotype AA control participants.
Desferrioxamine (DFO) is used to treat an excess accumulation of iron in the body and is currently the most commonly used iron chelator for the treatment of 'iron overload' disorder. However, the disadvantages of DFO surround its high toxicity and very short plasma half-life. Here, the detailed in vitro evaluation of a novel class of high molecular weight iron chelators based on DFO and polyethylene glycol methacrylate is reported. Reversible addition fragment chain transfer (RAFT) copolymerization afforded polymer conjugates (P-DFO) with well-controlled molecular weight (27-127 kDa) and substitution of DFO (5-26 units per chain) along the copolymer. Human umbilical vein endothelial cell (HUVEC) based cell viability assays showed that the cytotoxicity of P-DFO decreased more than 100-fold at identical concentrations of DFO. The hemocompatibilities of various P-DFO samples were determined by measuring prothrombin time (PT), activated partial thromboplastin time (APTT), thrombelastograph parameters (TEG), complement activation, platelet activation, and red blood cell aggregation. Furthermore, the iron binding properties and chelating efficiency of P-DFO were compared to DFO by measuring the spectral properties upon binding to iron(III), while the prevention of iron(III) mediated oxidation of hemoglobin was also determined. Degradation of the P-DFO conjugates via cleavable ester linkages between the polymer backbone and the PEG side chains was evaluated using gel permeation chromatography (GPC) and NMR. Since the chelating ability of DFO remains intact after conjugation to the copolymer backbone, these macromolecular, blood compatible and degradable conjugates are promising candidates as long circulating, non-toxic iron chelators.