Study objectives

Few studies have examined the validity of actigraphy in school-aged children. The objective of this study was to examine the validity of a commonly used actigraph compared to polysomnography (PSG) in a sample of children age 5 to 12 y born prematurely, sleeping in their natural home environment.

Methods

148 children born preterm (85 boys and 63 girls), ages 5-12 y (mean = 9.3 y, standard deviation = 2.0) wore the Philips Respironics Actiwatch-2 for 1 night concurrently with comprehensive, ambulatory PSG in the child's home. Sleep outcome variables were sleep onset latency, total sleep time (TST), and sleep efficiency. Epoch-by-epoch comparisons were used to determine sensitivity, specificity, and accuracy. Secondary analyses examined differences between children with no sleep issues, obstructive sleep apnea syndrome, and periodic limb movements in sleep (PLMS).

Results

Actigraphy significantly underestimated TST (30 min) and sleep efficiency (5%). Actigraphy underestimated or overestimated sleep onset latency by at least 10 min for a third of the children. Sensitivity and accuracy were good at 0.88 and 0.84, respectively, whereas specificity was lower at 0.46. Differences between actigraphy and PSG for TST and sleep efficiency were greatest for children with PLMS.

Conclusions

This study adds to the small existing literature demonstrating the validity of actigraphy in middle childhood. Although actigraphy shows good sensitivity (ability to detect sleep), specificity (ability to detect wake) is poor in this age group. Further, the results highlight the importance of considering whether a child has PLMS when interpreting actigraphic data, as well as the difficulties in accurately capturing sleep onset latency with actigraphy.

Objective

Plexiform neurofibromas (PNs) are complex, benign nerve sheath tumors that occur in approximately 25%-50% of individuals with neurofibromatosis type 1 (NF1). PNs that cause airway compromise or pulmonary dysfunction are uncommon but clinically important. Because improvement in sleep quality or airway function represents direct clinical benefit, measures of sleep and pulmonary function may be more meaningful than tumor size as endpoints in therapeutic clinical trials targeting airway PN.

Methods

The Response Evaluation in Neurofibromatosis and Schwannomatosis functional outcomes group reviewed currently available endpoints for sleep and pulmonary outcomes and developed consensus recommendations for response evaluation in NF clinical trials.

Results

For patients with airway PNs, polysomnography, impulse oscillometry, and spirometry should be performed to identify abnormal function that will be targeted by the agent under clinical investigation. The functional group endorsed the use of the apnea hypopnea index (AHI) as the primary sleep endpoint, and pulmonary resistance at 10 Hz (R10) or forced expiratory volume in 1 or 0.75 seconds (FEV1 or FEV0.75) as primary pulmonary endpoints. The group defined minimum changes in AHI, R10, and FEV1 or FEV0.75 for response criteria. Secondary sleep outcomes include desaturation and hypercapnia during sleep and arousal index. Secondary pulmonary outcomes include pulmonary resistance and reactance measurements at 5, 10, and 20 Hz; forced vital capacity; peak expiratory flow; and forced expiratory flows.

Conclusions

These recommended sleep and pulmonary evaluations are intended to provide researchers with a standardized set of clinically meaningful endpoints for response evaluation in trials of NF1-related airway PNs.