This was a cross-sectional, contemporary cohort study with sequential allocation of 85 patients with SCA enrolled in a referral center for hematology and transfusion medicine, between May of 2007 and May of

2008. The following inclusion criteria were used: diagnosis of SCA attained through the quantitative analysis of hemoglobin by hemoglobin electrophoresis or high-performance liquid chromatography (HPLC), performed with Variant II equipment (Bio-Rad, USA); age between 2 and 19 years; clinically stable; completed the questionnaire; allowing pediatric and otorhinolaryngological evaluation; and undergoing nocturnal polysomnography. The following exclusion criteria were used: other genetic syndromes, Panobinostat nmr debilitating diseases, acute hepatitis, previous treatment for OSAS or recent craniofacial trauma; using hypnotic drugs; having been treated with corticosteroids; pregnancy; and presence of infection during the evaluation. The PEPI-Sample program (Sagebush Press,USA) was used to calculate sample size, and the following parameters were used: a confidence level of 95%, and the prevalence of OSAS in children/adolescents of 5% (4.9% as an acceptable prevalence difference). The sample was obtained from a population of PS 341 approximately

1,000 children and adolescents with SCA, registered at a referral center for hematology and transfusion medicine. Therefore, to meet the objectives, the calculated sample size consisted of 71 patients. Considering 10% losses, the total (n) consisted of 78 patients. Age was measured in full years, according to the birth date. Ethnicity was self-reported, according to the official nomenclature of demographic censuses, using skin color as reference (white, mixed-race, or black). Weight was measured using a mechanical

scale (model 131; Filizola – Brazil). Length was measured with a stadiometer. These measurements were compared to the growth charts of the National Center for Health Statistics and Cyclin-dependent kinase 3 converted into Z-scores for body mass index (BMI), weight/age, and height/age based on age and gender, using the Epi-Info software (release 3.4.1;CDC- USA). The oral cavity assessment was performed by a single otolaryngologist in the Frankfurt position, using a standard tool. The measures of the oral cavity, except the overjet (OJ), were made with the tongue in a relaxed position and with a mouth opening angle of 20° to the mandibular condyle. For that purpose, a dry-point, 20° fixed-aperture compass was used, which was placed on the topography of the temporomandibular joint, the tip of its upper leg aligned with the upper central incisors and the lower compass leg aligned with the lower central incisors, to yield the desired mouth opening.