الفهرس 
Sickle Cell DiseaseOnly 14 pages are availabe for public view
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Abstract
Sickle cell disease is a genetic multisystem disease that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disease have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle or crescent shape. This can cause pain and other serious problems such infection, acute chest syndrome and stroke.
Neopterin is a metabolite of guanosine triphosphate by activated monocytes, macrophages, dendritic cells, and endothelial cells upon stimulation mainly by interferon gamma. Neopterin is a biomarker of cell-mediated immunity. It has been shown that neopterin (NP) levels increase in several inflammatory pathological conditions including viral, bacterial, and parasitic infections. So it was supposed that NP levels may increase in patients with SCD.
This case control study was conducted on 35 children from Hematology unit, Pediatric department, Menoufia University Hospital in the age group of 2-18 years considered as a case group, and 35 apparently healthy children with age and sex matched to the first group as a control group. All patients were diagnosed as sickle cell anemia based on clinical and hematological characteristics. Full history taking and complete clinical examination were done. Complete blood count (CBC), serum ferritin, Hb electrophoresis. Serum neopterin concentrations was detected by commercial enzyme-linked immunosorbent assay (ELISA) kits.
This study is to assess neopterin concentration in patients with SCD.
Summary
95
In our study there was a high statistical significant difference in the consanguinity between patient group and control group while there was no statistically significant difference between both groups regarding age, sex, height and BMI.
Also, our study shows that 42.9 % of patients presented with pallor, 37.1 % presented with jaundice and 57.1 % had splenomegaly.
Majority of patients presented with different types of crisis 71 % had vaso-occlusive crisis, 29 % presented with hemolytic crisis.
Regarding intake of hydroxyurea and iron chelator (deferasirox), 65.7 % of patients received hydroxyurea and 51.4 % of patients received deferasirox.
Patient group was subdivided into sickle cell anemia and sickle thalassemia 80 % and 20 % respectively.
Also, there was a high significant difference in Hb, HCT and MCH between patients and control group.
Also, there was a high significant difference in platelets, HbA1, HbA2, HbF and HbS between patients and control group, with no significant difference in MCV or WBCs between both groups.
Regarding serum ferritin, there was a high significant difference in serum ferritin between patient and control group, serum ferritin was significantly higher in patient group (mean = 933.2) than in control group (mean = 60.42).
There was no significant difference in AST, ALT, serum urea and serum creatinine between patient and control group.
There was also a high significant difference in serum neopterin level between patient and control group.
Summary
96
Serum neopterin was significantly higher in patients (mean = 6.83) than control (mean = 2.90) group.
In this study variable outcome of transcranial Doppler presented in patient group as majority of patients were conditioning (68.8%) , 11.4 % were high risk and 20% of patients were normal while there was normal transcranial Doppler in all controls.
Echocardiography was normal in both patient and control group.
Also, there was a high significant relation between serum neopterin and transcranial Doppler of patient group while there was no significant relation between serum neopterin and demographic and clinical data of patients.
There was a significant positive correlation between serum neopterin regarding MCH, platelet count, reticulocytes, HbF, HbS, HbA2 and AST.
While there was a significant negative correlation between serum neopterin regarding height, Hb, HCT and HbA1.