Evaluation of Respiratory Function Test in Children with Sickle Cell Anemia with Acute Chest Syndrome

Sickle cell disease is caused by mutant recessive autosomal in β-globin chain leading to deformation of sickle cells. Hemoglobin S in deoxygenated conditions causes microvascular obstruction and adverse effects on various organs. The manifestation of sickle cell in lungs is as a thromboembolism, acute chest syndrome and abnormal lung activity test. This study aimed to Evaluation of the respiratory function test in children with sickle cell and its relationship with age, sex, allele type, acute chest syndrome and pain crisis, that lung injury of these patients to be diagnosed early. This descriptive-analytical study was done on 6-18 years old children in the Pediatric Hematology Clinic at Bandarabbas Hospital in 2011 to 2012. Following the CBC, Hb electrophoresis, spirometery and records of the patients (acute chest syndrome, pain crisis), theses date were recorded and imported to SPSS20 statistical software (mean ± SD) and analyzed by Chi-squar test. Of 29 patients, 17 were male and 12 were female. Highest genotype was SB 44.8 % (13 cases) and lowest was SA and SB0 6.9 % (2 cases). Results of lung function test were as follows: restrictive pattern (72.4%, 21 cases), normal pattern (20.7%, 6 cases) and obstructive pattern (6.9%, 2 cases). There was no significant difference in pattern of pulmonary function test in patients with and without history of previous acute chest syndrome. There was also no significant correlation between spirometric pattern and age, sex, type allele and pain crisis. Pulmonary function is abnormal in 79.3%