The assessment of pulmonary hypertension in children and adolescents with sickle cell anemia

Purpose: In this study, pulmonary hypertension, which is a serious complication of sickle cell disease that causes high insidence of mortality and morbidity, in early childhood with noninvasive methods was searched in the children and adolescents who were followed up in Çukurova University Medical Faculty Department of Pediatric Haematology with the aim of finding out the predisposing factors and enlightening the treatment protocols. Materials and Methods: We enrolled 29 pediatric and adolescent patients with the age of 10 or more who were followed up in Çukurova University Medical Faculty Department of Pediatric Haematology with sickle cell disease diagnosis and 23 healty children and adolescents with the same age and gender followed in general pediatrics policlinics as control group between February 2015 and October 2015. Blood samples were analysed for complete blood cell counts, hemoglobin electrophoresis, BNP, total serum bilirubin, direct bilirubin, AST, ALT, BUN, Cr, LDH, uric acid, iron, TIBC, ferritin and transferin. Echocardiographies and six minute walk tests were performed by Pediatric Cardiology and respiratory function tests were performed by Pediatric Allergy-İmmunology in the same day. Obtained results were compared statistically. Results: Hemoglobin and hematocrit levels were lower in sickle cell diase group (p=0,0001). The mean HbS value was % 71,9±14,9 in the patient group. Serum BNP level was higher in the patient group (p=0,001). According to respiratory function tests FEV1 (p=0,002), PEF (p=0,010) and MEF 25-75 (p=0,023) measurements were lower in the patient group while FEV1/FVC measurements showed no statistically significant differences (p=0,760). According to echocardiography results, the myocardial performance index of right and left ventricles were higher in patient group (respectively p=0,001, p=0,010). The pulmonary artery systolic pressures calculated with tricuspid regurgitant velocity were higher in patient group than control group (p=0,0001). Mean pulmonary artery pressure that is calculated by using pulmonary regurtitation measurements showed no statistically significant differences between the groups (p=0,573) while mean pulmonary artery pressure that is calculated by using pulmonary artery acceleration time was higher in patient group (p=0,017). With six minutes walk test, lower walking distances were detected in patient group (p=0,0001). Conclusion: In our study, even though any pulmonary hypertansion was not identified, we showed the affected systolic and diastolic functions with increased BNP levels. Because the pulmonary hypertension is an important mortality and morbidity reason in sickle cell anemia, further multicentric studies with long term close follow ups with the aim of detecting the predisposing factors for pulmonary hypertansion and enligthening the treatment protocols are needed. Keywords: child, adolescent, sickle cell anemia, pulmonary hypertension, echocardiography, BNP, respiratory function test