الفهرس | Only 14 pages are availabe for public view |
Abstract Sickle cell disease or sickle cell anemia is a life -long blood disorder characterized by red blood cells that assume an abnormal, rigid, sickle shape. Sickling decreases the cells’ flexibility and results in a risk of various complications. The sickling occurs because of a mutation in the hemoglobin gene. Kynurenine is the main metabolite of tryptophan degradation. The rate-limiting step in the kynurenine pathway is catalyzed by indoleamine 2,3- dioxygenases (IDO1 and IDO2) and tryptophan 2,3- dioxygenase (TDO). TDO is known to be induced by Trp and steroids, whereas IDO is induced by proinflammatory stimuli and cytokines like IFN-γ, tumor necrosis factor (TNF)-α, interleukin (IL)-6. Hence, IDO is responsible for the immune mediated Trp degradation. Kynurenine carry out diverse biological functions, including dilating blood vessels during inflammation and regulating the immune response. This case control study was conducted on 40 children from hematology unit, pediatric department, Menoufia University Hospital in the age group of 2-18 years.40 children with sickle cell disease as a patient group and 40 apparently healthy children, age, sex, and socioeconomic standard matched as a control group. Full history taking and complete clinical examination was done. Complete blood count (CBC), serum ferriti, Hb electrophoresis, Serum Kynurenine concentrations will be determined by commercial enzyme-linked immunosorbent assay (ELISA) kits. In the present study Consanguinity was significantly higher in patients group than control but there no significant difference between both groups regarding age, sex, height and BMI. Also in our study Clinical variabilities as pallor, jaundice, splenomegally and criseses presented in patients in different ratios, patients had multiple episodes of pain yearly.patients presented in different ratios regarding intake of hydroxyurea, its dose, iron chelation,its dose and transfusion index per year.patient group was subdivided to sickle cell anemia and sickle thalassemia by about 67,5% and 32, 5%respectively. |