Descriptive Study: sleep disorders breathing and Polysomnographic changes in Children and Adolescents with Sickle Cell Disease

• Published in: Blood 2011-11, Vol.118 (21), p.4849-4849
• Main Authors: Campanaro, Celia M., Pereira, Jose Carlos, Pessoa, Jose Hugo Lins, Halimann, Marcia Pradella, Favoreto, Monica
• Format: Article
• Language: English
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.V118.21.4849.4849

Abstract 4849 -Sickle Cell Disease (SCD) is a chronic inflammatory disease and a public health problem in Brazil, due its prevalence and Brazilian people high miscegenation. The hypoxia is one of the trigger for vessel occlusive crises in SCD and the quality of sleep is important to maintain a good tissue oxygenation. The search for sleep-disordered breathing (SDB) must be made based on history, physical examination and polysomnography (PSN). These include the diagnosis of airway obstruction and sleep apnea/hypopnea, obstructive syndrome (SAHOS). The objective confirmation of disordered breathing during sleep is made by polysomnography, which identify and record and hypoxemic respiratory events during sleep. Identify the most common sleep disordered breathing and changes in PSG in patients with sickle cell disease. We evaluated 38 children and adolescents with sickle cell disease (SS, SC, S-Beta), regularly monitored at the outpatient pediatric hematology ICP Bolivar Risso/Grendacc-FMJ, the clinical behavior before (two years) by a clinical protocol, physical examination and polysomnography. This study was approved by the Ethics Committee and signed Informed Consent. Characterization of the group:distribution by sex: 19 boys/19 girls aged 2 to 17 years; genotype: Hb SS 20, 11 HbSC and 7 Sβ thalassemia. Clinical history and examination: It was found snoring present in four or more days a week and audible from other rooms in 14 patients (36%), difficulty breathing during sleep moderately difficult, with occasional sounds of “choking” the very difficult, paradoxical breathing in 6 patients (15%). Apnea witnessed over 2 to 3 times per night, in 4 patients (10%), diaphoresis during sleep over 2 to 3 times per week in 15 patients (39%) hyper-extended position of the neck during sleep or discreetly evident for almost all night in 4 patients (10%); mouth breathing at night several hours at night, almost every day, every night and night, in 20 patients (52%), restless sleep, with stirring frequently messy and covered in 23 patients (60%). On physical examination, he met retrognathia moderate and severe in 4 patients (10%), maxillary atresia moderate to severe in 10 (26%); goodwill previous moderate to severe in 12 patients (31%). The hypertrophy of tonsils (Brodsky) grade III and IV in 12 children (31%), the Mallampati score of 3 and 4 was identified in 7 patients (18). Polysomnography: periodic movements of the lower limbs (47%), from mild to severe snoring (37%),