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

-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%), fragmented sleep (7.5%), apnea and hypopnea (5%), EEG features epileptoid (5%), normal (17.5%). Patients with less severe clinical behavior were those with minor changes in polysomnography.

The study of sleep in patients with sickle cell disease, clinical and laboratory is essential to identify risk factors for situations of hypoxia. SDB is common in patients with DF, and their identification by clinical history and physical examination should be encouraged and be part of their clinical follow-up, indicating the completion of laboratory investigation when it will be necessary.

Identification of SAHOS is rarely addressed in routine pediatric consultations or hematological and should be part of the script of an interview and clinical examination.

The finding of the PSG member movements deserves further study because it has to be superior to those described in the literature.

No relevant conflicts of interest to declare.