Periodic limb movements in sleep (PLMS) are associated with sleep fragmentation and sympathetic nervous system activation, which in turn have been linked to cognitive and behavioral deficits in children and cardiovascular morbidity in both adults and children. Emerging evidence indicates that many children with sickle cell disease (SCD) have elevated PLMS. The specific aims of this prospective, repeated-measures, descriptive study were to assess the agreement between PLMS measurement by actigraphy and concurrent polysomnography (PSG), to test the feasibility of measuring PLMS by actigraphy at home, to evaluate PLMS variability over consecutive nights by actigraphy, and to provide preliminary data on objective and subjective correlates of PLMS. Twenty children with SCD and restless legs syndrome (RLS) symptoms or polysomnography-documented PLMS underwent concurrent attended polysomnography and ankle activity monitoring over one to two nights and home activity monitoring for three nights. Serum iron and ferritin were measured pre- and post-polysomnography. The datasets associated with this study include demographic data, SCD subtype, polysomnographic/activity monitor values, medical history, and clinical measures.
This was a retrospective medical record review of 55 consecutive children aged 2-18 years with sickle cell disease (SCD) (hemoglobin [Hb] SS and Hb SC genotypes) undergoing polysomnography for evaluation of sleep disordered breathing. Polysomnography values were compared between SCD genotypes, 4 age groups, and adenotonsillectomy status using descriptive and nonparametric statistics. Medical record data were collected for 12 months pre-polysomnography and 12 months post-polysomnography/adenotonsillectomy. This dataset includes demographic data, SCD type, polysomnographic values, and clinical measures.
Children with sickle cell disease (SCD) experience neurodevelopmental decline over time. They also tend to have short duration, poor quality sleep and elevated fatigue levels. This study measured sleep via actigraphy over one week and cognitive and behavioral measures in 19 children and adolescents with SCD. Aged 7-18 years, the majority of participants were referred for neurodevelopmental testing due to academic or behavioral difficulties. Data was collected from parent report, medical record, and included age, sex, race, SCD genotype, results of neuroimaging studies including brain magnetic resonance imaging (MRI) and transcranial Doppler (TCD) velocities, and current use of hydroxyl urea or chronic blood transfusion. Additionally, parents completed the Behavior Rating Inventory of Executive Function (BRIEF), participants completed the Wide Range Achievement Test (WRAT), and both completed the PedsQL Multidimensional Fatigue Scale.