Objective: To estimate the prevalence of obstructive sleep apnea syndrome (OSAS) in children and adolescents with sickle cell anemia (SCA); to investigate the possible correlation between mean annual hemoglobin level and total sleep time with SpO2 < 90%, as well as between mean annual hemoglobin level and total sleep time with SpO2 < 80%; and to investigate the possible correlation between apnea-hypopnea index (AHI) and painful crisis. Methods: The study involved 85 patients with SCA. The patients completed a questionnaire, were submitted to polysomnography and underwent clinical evaluation (by a pediatrician and an otolaryngologist). An AHI > 1 was considered indicative of a diagnosis of OSAS. Results: The prevalence of OSAS was 10.6%. We found a negative correlation between mean annual hemoglobin level and total sleep time with SpO2 < 90% (r = −0.343; p = 0.002), as well as between mean annual hemoglobin level and total sleep time with SpO2 < 80% (r = −0.270; p = 0.016). There was no association between AHI and painful crisis. Conclusions: The prevalence of OSAS in this population was high (10.6%). Therefore, it is important to identify signs of OSAS as soon as possible and to determine the mean annual hemoglobin level because of the inverse correlation between that level and the total sleep time with SpO2 < 90% or < 80%.

Keywords: Prevalence; Sleep apnea, obstructive; Anemia, sickle cell; Polysomnography; Sleep apnea syndromes.

The physiopathology of obstructive sleep apnea syndrome is multifactorial. Gender and obesity status, as well as genetic, anatomic, and hormonal factors, together with ventilatory drive, interact in a diverse manner in the physiopathology and clinical expression of the disease. Obesity is the main risk factor, since increases in body mass index, visceral fat, and neck circumference are strong predictors of the disease. Progesterone increases the activity of the upper airway dilator muscles and therefore plays a protective role in premenopausal women. This explains the fact that the prevalence of the disease is higher in postmenopausal patients, in patients with polycystic ovary syndrome, as well as in males. Evidence supports the fact that, as individuals grow older, there is a decrease in muscle tonus, with a consequent reduction in the dimensions of the upper airway lumen. Craniofacial anomalies, such as in retrognathia or micrognathia, are accompanied by posterior positioning of the tongue and can result in narrowing of the upper airway lumen. Finally, decreased ventilatory drive has been detected in patients with obstructive sleep apnea syndrome and hypercapnia.

Keywords: Sleep apnea obstructive/physiopathology; Body mass index; Obesity