الفهرس 
Aim of the WorkOnly 14 pages are availabe for public view
 |  | from | 98 |  |  |
| | | from | 98 | | |
Abstract
Sickle cell disease is a spectrum of disorders of the red blood cell, sickle cell disease (SCD) is one of the most common hematologic disorders associated with neurological disease in children. Children with SCD have a high risk of developing cerebrovascular complications. Ischemic lesions of the brain that occur without clinical evidence of neurological deficits, termed silent cerebral infarcts, are also very common in children with SCD Vasculopathy involving the large intracranial arteries, including the supraclinoid internal carotid artery (ICA), middle cerebral artery (MCA), and anterior cerebral artery (ACA). Inflammation plays a critical role in the complex pathophysiology of sickle cell disease (SCD) and drives both the acute and chronic processes leading to vascular injury. Endotheline 1 produced as an inflammatory mediator in children with sickle cell anemia. The present study was conducted on 30 patients with documented sickle cell anemia. In addition, 30 apparently healthy age and sex. All pediatrics selected from the hematology pediatric department, Menoufia University and blood outpatient clinic of Al-Helal hospital, Egypt from the period of December 2016 and April 2018. Clinical and laboratory data of the studied groups were tabulated and statistically analyzed. The clinical data also reported that weight,pallor, jaundice, vasoocclusive crises (dactylitis –avascular necrosis – acute chest syndrome) and recurrent headace, (manifestation of hemolytic anemia) were significantly higher in patients than control. Also hemoglobin concentration and platelets were significantly lower in patients than control group while MCV and MCH there were no significance. In our study of transcranial Doppler ultrasound in children with sickle cell anemia, we found that the patients divided according to the velocity of cerebral blood flow into three groups, (high risk-conditional –normal group. Endotheline1 is an inflammatory mediator to the vascular injury in sickle cell anemia; in our study it was significantly higher in patient than control group. In the light of the present study findings, it can be concluded that there is significance between patient and control regarding trascranial Doppler ultrasound and there was significant correlation between TCD and hemoglobin while no correlation between TCD and MCV, MCH, AST, Ferretin in patients with sickle cell anemia. Based on the findings of this study, the following recommendations are proposed. Good follow-up is important for sickle cell patients specially CNS manifestation as these children of high risk to stroke. Transcranial Doppler ultrasound is an important investigation in follow up. Monitoring of liver function, renal function, and serum ferretin were important.
Extent the study to include larger number of patients of different age groups. Transcranial Doppler ultrasound and serum endothelin 1 should be done for all patients.