Study of Transcranial Doppler Ultrasound and Endothelin 1 in Children with Sickle Cell Anemia: Single Center Study

Аннотация

Objectives: To assess the impact of our transcranial Doppler ultrasonography (TCD) program on the incidence of first stroke in children of sickle cell anemia .and elevated serum endothelin1 as an inflammatory mediator in these children Background: Stroke is a major complication of sickle cell disease (SCD), even in very young children. About 11% of children with homozygous sickle cell anaemia (SS) develop stroke by the end of the second decade of life. The underlying etiology in most cases is an ischemic stroke caused by large-vessel stenosis or occlusion. Transcranial Doppler (TCD) recommended as a routine screening test to identify children at high risk of developing stroke, measures flow velocities within large intracranial arteries. TCD should be routinely performed in children between 2 and 16 years, age group at the highest risk of sickle cerebral vasculopathy.

Material and method: : This was a prospective case control study included 2 groups: patients group consisted of 30 children with sickle cell anemia and sickle thalassemia compared to 30 healthy children of matched age and sex . Both groups subjected to thorough clinical examination, investigations in form of CBC, Liver and renal function, s. ferritin and endothelin 1. TCD for patients group.

Results: Our study enrolled 30 patients of sickle cell anemia with TCD examinations, 11 were males (36.5%) and 19 females (63.5%). Patients with( TAMMX 170 or less )were 20 (66.7%), 4 patients (13.3%)were  (170-200 cmH2o) and 20 of them (20%) were  (> 200CM H2O) .Endotheline 1 enrolled  the 30 patients of sickle cell anemia and  other 30 apparantly healthy children, endotheline in cases was 57.1±91.3 and in healthy children was 21.9±14.8.

Conclusion :  each group was 11 males (36.5%) and 19 females (63.5%) with age ranging from 2-17ys. Hemoglobin was significantly lower in patients than control but reticlocytes, WBCs, serum ferritin were significantly higher in patients than the control. Regarding TCD of the patients: the highest time average mean maximum velocity was 6 (20%) patients (high risk), in 4(13.3%) patients (conditional) and 20(66.7%) patients (normal). Endothelin 1 was significantly higher (57.1±91.3) in patients than the control (21.9±14.80).