Função pulmonar em portadores de anemia falciforme

Abstract

Objective: To evaluate the pulmonary function in children and adolescents with sickle cell disease (SCD) and to associate the findings with clinical and hematologic characteristics of the studied population. Methods: Male and female SCD patients with ten or more years old, clinically stable and without acute clinical problems were tested by spirometry. At that time, total pulse oximetry values, hemoglobin and total white blood cell count were verified. Association between altered pulmonary function tests and demographic, clinical and laboratorial characteristics of the patients were assessed. Statistical analysis included chi-square and t-test, being significant p<0.05. Results: Among the 51 studied patients, 40 (78%) had abnormal spirometric results: 20 (50%) had mixed or combined ventilatory disorders, 13 (33%) had classical restrictive profile and seven (18%), obstructive ventilatory disorder. Out of these seven patients, five (71%) had a positive response to bronchodilators. Increased total white blood cells count was associated with abnormal pulmonary function. Patients with previous acute pulmonary hospital admissions had lower forced expiratory volume in one second without bronchodilators, lower forced vital capacity before and after bronchodilators, and lower forced expiratory flow between 25 and 75% of the forced vital capacity after bronchodilators. Conclusions: Most SCD patients presented abnormal pulmonary function, being predominant the mixed or combined type, followed by the classical restrictive pattern. Increased white blood cell count in the blood, without acute clinical events, was associated to abnormal pulmonary function.