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This paper describes the development and psychometric properties of a condition-specific quality of life instrument for children with cerebral palsy (CP QOL-Child). A sample of 205 primary caregivers of children with CP aged 4 to 12 years (mean 8y 5mo) and 53 children aged 9 to 12 years completed the CP QOL-Child. The children (112 males, 93 females) were sampled across Gross Motor Function Classification System (GMFCS) levels (Level I=18%, II=28%, III=14%, IV=11%, V=27%). Primary caregivers also completed other measures of child health (Child Health Questionnaire; CHQ), QOL (KIDSCREEN), and functioning (GMFCS). Internal consistency ranged from 0.74 to 0.92 for primary caregivers and from 0.80 to 0.90 for child self-report. For primary caregivers, 2-week test-retest reliability ranged from 0.76 to 0.89. The validity of the CP QOL is supported by the pattern of correlations between CP QOL-Child scales with the CHQ, KIDSCREEN, and GMFCS. Preliminary statistics suggest that the child self-report questionnaire has acceptable psychometric properties. The questionnaire can be freely accessed at http://www.deakin.edu.ac/hmnbs/chase/cerebralpalsy/cp_qol_home.php. [ABSTRACT FROM AUTHOR]

Increasingly long and complex informed consents have yielded studies demonstrating comparatively low participant comprehension and satisfaction with traditional face-to-face approaches. In parallel, interest in electronic consents for clinical and research genomics has steadily increased, yet limited data are available for trio-based genomic discovery studies. We describe the design, development, implementation, and validation of an electronic iConsent application for trio-based genomic research deployed to support genomic studies of cerebral palsy. iConsent development incorporated stakeholder perspectives including researchers, patient advocates, institutional review board members, and genomic data-sharing considerations. The iConsent platform integrated principles derived from prior electronic consenting research and elements of multimedia learning theory. Participant comprehension was assessed in an interactive teachback format. The iConsent application achieved nine of ten proposed desiderata for effective patient-focused electronic consenting for genomic research. Overall, participants demonstrated high comprehension and retention of key human subjects' considerations. Enrollees reported high levels of satisfaction with the iConsent, and we found that participant comprehension , iConsent clarity , privacy protections , and study goal explanations were associated with overall satisfaction . Although opportunities exist to optimize iConsent, we show that such an approach is feasible, can satisfy multiple stakeholder requirements, and can realize high participant satisfaction and comprehension while increasing study reach., Competing Interests: Conflicts of Interest Paul Gross is President and CEO of the CP Research Network, which contributed to the funding of this project. Mr. Gross personally made financial contributions (donations) to CPRN to support this work, but receives no financial compensation related to either. Dr. Noritz has consulted for Abbott Nutrition, unrelated to this project. Dr. Shrader receives research funding from NIH and serves on the National Advisory Board for Medical Rehabilitative Research for NIH/NICHD.

Background: Selective dorsal rhizotomy (SDR) is an established procedure for the treatment of spasticity associated with cerebral palsy (CP). With the indications for the procedure expanding, we aimed to investigate provider perception about SDR candidates against the characteristics of those undergoing SDR in the CP Research Network (CPRN) registry., Methods: This was a mixed methods study. Descriptive data on 2486 individuals in the CPRN registry and results of semistructured interviews with 41 providers about the effect of age, Gross Motor Function Classification System (GMFCS) level, and dystonia on consideration for SDR are reported., Results: SDR was performed in 238 individuals, majority aged eight to 12 years (n = 105), GMFCS level II (n = 46), and white (n = 183). Most neurosurgeons perform a single-level SDR. Providers believe the majority of individuals undergoing SDR are between five and six years and GMFCS level II with variable agreement. There was no significant agreement about the youngest age (P = 0.451) or ideal GMFCS level (P = 0.451) for SDR. Providers had agreement on the oldest age for SDR (P = 0.041), how to screen for dystonia (P < 0.001), and dystonia as a contraindication for SDR (P < 0.0005)., Conclusions: Providers show variation in regard to what they believe the youngest age or ideal GMFCS level is for SDR but agree on performing SDR in older age groups, screening for dystonia with a neurological examination, and being less likely to perform SDR in the presence of dystonia., Competing Interests: Declaration of competing interest None., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Aim: To understand the mobility experiences, supportive mobility device (SMD) use, and desired participation outcomes of individuals with cerebral palsy (CP) across the life span, and describe how perspectives of rehabilitation care and professional resources may influence mobility decision-making processes and outcomes., Method: In the second phase of an overarching study, focus groups were conducted with 164 participants (68 individuals with CP; 32 females, 36 males; mean age 17y 8mo, SD 11y 11mo, range 3-68y), 74 caregivers (50 females, 24 males), and 22 healthcare providers (14 females, eight males) across four US cities. Sessions were audio-recorded, transcribed, and analysed using constant comparison., Results: Six themes emerged. Five presented across all stakeholder groups: (1) the system is broken; (2) equipment is simultaneously liberating and restricting; (3) adaptation across the life span; (4) designed for transport, not for living; and (5) sharing our stories and sharing resources. One theme (theme 6) was specific to healthcare providers: caught in the middle., Interpretation: This qualitative study underscores the simultaneous value and frustration associated with SMDs as described by the community with CP, and recognition among all stakeholders of the need to improve connections and resource networks within the community with CP to improve SMD design and provision processes across device types and across the life span for individuals with CP., What This Paper Adds: Supportive mobility devices (SMDs) were most often equated with freedom, participation, and independence. Frustration with SMDs across the life span persisted with regard to design, function, cost, and maintenance. Stakeholders in the community with cerebral palsy are seeking greater networking and resource sharing to enhance SMD provision processes. Access to appropriate SMDs across the life span and the need for system improvement are critical., (© 2022 Mac Keith Press.)

Background: Pediatric patients with oncologic and hematologic diagnoses who experience newly acquired functional deficits during a hospitalization may benefit from intensive therapies. However, acute medical issues or disease treatment plans may prevent a safe transfer to the inpatient rehabilitation unit. Accordingly, Short-term Pediatric Rehabilitation Intensive Therapy (SPRINT), a 2-week inpatient intensive therapy program, was developed for pediatric patients on an acute care service., Objective: To assess functional outcomes of SPRINT participants, adverse events associated with the program, and measures of patients' fatigue, sadness, nervousness, and pain by parents and patients at the start and end of SPRINT., Design: Retrospective cohort study., Settings: Hematology-Oncology and Bone Marrow Transplant units at regional pediatric tertiary care hospital., Participants: Eighteen pediatric patients (50% female, age 1.9-17.8 years) participated in SPRINT, and 11 parents and 4 children completed questionnaires., Interventions: Not applicable., Main Outcome Measures: Functional outcomes of SPRINT participants measured by Caregiver Assistance section of the Pediatric Evaluation of Disability Inventory (PEDI), adverse events identified on chart review, and inquiry of participants' symptoms before and after SPRINT with a questionnaire., Results: Common diagnoses included leukemia and lymphoma (N = 9, 50%) and central nervous system tumor (N = 6, 33%). Deconditioning (N = 18, 100%) and peripheral neuropathy (N = 8, 44.4%) were common rehabilitation diagnoses. Significant gains were found in tasks in self-care and mobility domains of PEDI (all P < .05), as well as functional expression in social function domain (P = .03). No adverse events related to SPRINT participation were identified. There was no significant difference between pre- and post-SPRINT questionnaire responses., Conclusions: SPRINT is an alternative model for intensive rehabilitation care delivery. Data suggested that SPRINT participation was safe and resulted in significant gains in mobility, self-care, and functional expression for pediatric patients with hematologic and oncologic diagnoses while receiving acute care. No difference was found in questionnaire responses after SPRINT participation., (© 2021 American Academy of Physical Medicine and Rehabilitation.)

Introduction: When studying the effect of ankle-foot orthoses (AFOs) on gait, it is important to know their sagittal plane stiffness. However, there are no established thresholds for stiffness of non-articulated AFOs designed to be rigid. If wanting to implement published algorithms for ankle-foot orthosis-footwear combinations (AFO-FCs), the AFOs must be equally as stiff as those of the developer of the published AFO-FC algorithms. Hence, the aim of this work was to compare the sagittal plane stiffness of AFOs designed to be rigid, made for a clinical trial in the USA, and following algorithms for AFO-FC designs, to those made and used clinically in the UK by the developer of the AFO-FC algorithms., Materials and Methods: Stiffness of 9 pediatric polypropylene AFOs was tested (UK: 6; USA: 3). A computer-controlled motorized device was used in which all AFOs were clamped with the calf shell in a fixed vertical component and the foot section in a rotating plate. Each AFO was tested for 3 trials, loading the foot plate 30 Nm towards dorsiflexion and 20 Nm towards plantarflexion. Torque-angle graphs were plotted and deflection and stiffness compared descriptively across AFOs., Results: Average deflection of AFOs was UK: 3.42±0.83° and USA: 4.81±1.05°. Average stiffness of AFOs was UK: 14.34±3.34 Nm/° and USA: 10.30±1.92 Nm/°., Conclusions: All tested AFOs deflected only a few degrees in either direction (range: 2.59° to 6.02°), providing the first information reported for the stiffness of rigid pediatric non-articulated AFOs. Overall, the UK AFOs were stiffer and deflected less than the USA study AFOs. AFO design features should be carefully considered as they likely influence sagittal plane stiffness and deflection under load., Competing Interests: Conflicts of Interest: The authors declare that they have no other conflicts of interest.

Competing Interests: The authors declare no conflicts of interest.

Competing Interests: The authors declare no conflicts of interest.

Purpose: This exploratory study assessed postural control and muscle activity in children with cerebral palsy while standing barefoot (BF), in prescribed ankle-foot orthoses (AFOs) and in distal control orthoses (DCOs), which stabilized foot-ankle and deliberately aligned the shank., Methods: This within-subject study evaluated 10 participants, Gross Motor Functional Classification System level III, across the 3 ankle-foot conditions in: (1) static standing duration and (2) modified Clinical Test of Sensory Interaction on Balance with electromyography (EMG) on 7 muscles., Results: Participants had significantly decreased center of gravity (COG) velocity sway in DCO versus BF and AFO, decreased loss of balance (LOB), and increased standing for DCO versus BF. DCO had minimal effect on EMG activity., Conclusions: DCO provided significant stabilizing effects on COG sway velocity, standing duration, and LOB. DCO may be effective in balance training. It is unclear whether benefit was derived from stabilization of the ankle joint, the resultant shank alignment, or both., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2021 Academy of Pediatric Physical Therapy of the American Physical Therapy Association.)

Background: Children with hemiparesis are commonly prescribed ankle foot orthoses to help improve gait; however, these orthoses often result in only small and variable changes in gait. Research with adult stroke survivors has suggested that orthoses that extend beyond the ankle using long, passive tendon-like structures (i.e. exotendons) can improve walking., Objectives: The aim of this study was to quantify the impact of an exotendon-based exoskeleton on pediatric gait., Study Design: Repeated-measures study., Methods: Two typically-developing children and two children with hemiparesis completed a gait analysis, walking without and with the exoskeleton. The exotendon was tested at three stiffness levels., Results: All children were able to walk comfortably with the exoskeleton, with minimal changes in step width. Walking speed increased and lower limb joint symmetry improved for the children with hemiparesis with the exoskeleton. Each participant had changes in muscle activity while walking with the exoskeleton, although the impact on specific muscles and response to exotendon stiffness varied., Conclusion: Exotendon-based exoskeletons may provide an alternative solution for optimizing gait in therapy and in the community for children with hemiparesis. Determining the optimal stiffness and configuration for each child is an important area of future research., (Copyright © 2020 International Society for Prosthetics and Orthotics.)

Purpose: The aim of this study was to examine whether the translocator protein 18-kDa (TSPO) PET ligand [ 18 F]FEPPA has the sensitivity for detecting changes in CD68-positive microglial/macrophage activation in hemiparkinsonian rhesus macaques treated with allogeneic grafts of induced pluripotent stem cell-derived midbrain dopaminergic neurons (iPSC-mDA)., Methods: In vivo positron emission tomography (PET) imaging with [ 18 F]FEPPA was used in conjunction with postmortem CD68 immunostaining to evaluate neuroinflammation in the brains of hemiparkinsonian rhesus macaques (n = 6) that received allogeneic iPSC-mDA grafts in the putamen ipsilateral to MPTP administration., Results: Based on assessment of radiotracer uptake and confirmed by visual inspection of the imaging data, nonhuman primates with allogeneic grafts showed increased [ 18 F]FEPPA binding at the graft sites relative to the contralateral putamen. From PET asymmetry analysis of the images, the mean asymmetry index of the monkeys was AI = - 0.085 ± 0.018. Evaluation and scoring of CD68 immunoreactivity by an investigator blind to the treatment identified significantly more neuroinflammation in the grafted areas of the putamen compared to the contralateral putamen (p = 0.0004). [ 18 F]FEPPA PET AI showed a positive correlation with CD68 immunoreactivity AI ratings in the monkeys (Spearman's ρ = 0.94; p = 0.005)., Conclusion: These findings reveal that [ 18 F]FEPPA PET is an effective marker for detecting increased CD68-positive microglial/macrophage activation and demonstrates sufficient sensitivity to detect changes in neuroinflammation in vivo following allogeneic cell engraftment.

Purpose: This study aims to present developmental trajectories for physical activity (PA) and walking performance for children with cerebral palsy (CP). Materials and methods: Seventy-nine children with CP, 39 (49%) female, Gross Motor Functional Classification System levels I-V, and mean age 91.3 months (+/-27.7 SD) participated. Participants in levels I-V wore the Actigraph to capture PA and children in levels I-II also wore a StepWatch (SW) ( n  = 43) to measure walking performance. Trajectories for average PA counts/minute and number of minutes of moderate to vigorous PA were generated for levels I, II, and III/IV/V (aggregate). Single leg strides/day and average strides faster than 30 strides/min trajectories were generated for levels I-II. Results: Participants did not display plateaus in PA or walking performance based on functional level. Children in all levels showed a decrease in amount and intensity of PA from 3.0 to 12 years old, with participants in level I demonstrating the steepest decline. Children in level I decreased slightly, and level II increased slightly in both walking performance measures from 3.0 to 12 years old. Conclusions: Longitudinal curves demonstrate variations in PA and walking performance by functional level and provide prognostic information as to what changes may be anticipated for children with CP.Implications for rehabilitationLongitudinal developmental trajectories for physical activity and walking performance for children with cerebral palsy across functional levels are documented.Trajectories have potential to support collaborative intervention planning between therapists and families relative to physical activity and walking performance.

Objective: To compare the effect of low-intensity (LI) vs high-intensity (HI) treadmill training (TT) on walking attainment and overall walking activity in children with cerebral palsy (CP)., Design: Prospective, multisite, randomized controlled trial., Setting: Homes of the participants., Participants: Children with spastic diplegic CP, Gross Motor Function Classification System Level I and II, ages 14-32 months (N=19; male, n=8)., Interventions: The children were randomized to LI TT (2×/wk for 6wk) (n=10) and HI TT (10×/wk for 6wk) (n=9). The TT was carried out by the families with weekly instruction by the researchers., Main Outcome Measures: Children were assessed at study onset, post intervention, and 1 and 4 months post intervention with the Gross Motor Function Measure Dimension D/E (GMFM D/E), average strides per day and percentage of time spent walking with accelerometers, the Peabody Developmental Motor Scales-2 (PDMS-2), Pediatric Evaluation of Disability Index Mobility Scale, timed 10-m and 1-minute walk test, and Functional Mobility Scale. Blinding was conducted for GMFM D/E and PDMS-2. Linear mixed effects regression models were applied to all outcomes., Results: No significant between-group differences were found in any outcome measure at any of the time points. Children in the HI group did not show significant improvement immediately following the intervention in GMFM E (P=.061), while children in the LI group did (P=.003), but no statistically significant differences were detected over time (P=.71). Children in the HI group showed better walking independence on the Functional Mobility Scale at all postintervention assessments., Conclusions: A twice-weekly dosage was equally effective in improving skills related to walking compared with a 10×/wk program and can be more readily implemented into clinical practice., (Copyright © 2019 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.)

Large-scale population analyses coupled with advances in technology have demonstrated that the human genome is more diverse than originally thought. To date, this diversity has largely been uncovered using short-read whole-genome sequencing. However, these short-read approaches fail to give a complete picture of a genome. They struggle to identify structural events, cannot access repetitive regions, and fail to resolve the human genome into haplotypes. Here, we describe an approach that retains long range information while maintaining the advantages of short reads. Starting from ∼1 ng of high molecular weight DNA, we produce barcoded short-read libraries. Novel informatic approaches allow for the barcoded short reads to be associated with their original long molecules producing a novel data type known as "Linked-Reads". This approach allows for simultaneous detection of small and large variants from a single library. In this manuscript, we show the advantages of Linked-Reads over standard short-read approaches for reference-based analysis. Linked-Reads allow mapping to 38 Mb of sequence not accessible to short reads, adding sequence in 423 difficult-to-sequence genes including disease-relevant genes STRC , SMN1 , and SMN2 Both Linked-Read whole-genome and whole-exome sequencing identify complex structural variations, including balanced events and single exon deletions and duplications. Further, Linked-Reads extend the region of high-confidence calls by 68.9 Mb. The data presented here show that Linked-Reads provide a scalable approach for comprehensive genome analysis that is not possible using short reads alone., (© 2019 Marks et al.; Published by Cold Spring Harbor Laboratory Press.)

Purpose: The purposes of this study were to document longitudinal developmental trajectories in 6-minute walk test (6MWT) distances and to develop age-specific reference percentiles for children across different Gross Motor Function Classification System (GMFCS) levels., Methods: A TOTAL OF: 456 children with cerebral palsy ages 3 to 12 years of, GMFCS levels I to III participated. Children's motor function was classified on the GMFCS, and children completed the 6MWT 2 to 5 times in 2 years., Results: Longitudinal developmental trajectories support that 6MWT distances increase with age followed by a tapering, as children approach their functional limit relative to their GMFCS level. Reference percentile graphs were created to monitor change over time., Conclusions: The 6MWT longitudinal developmental trajectories, reference percentiles, and interpretation of percentile change should assist collaborative and proactive intervention planning relative to functional walking capacity for children with cerebral palsy.

Background: Cerebral palsy (CP) is a chronic condition that affects children and has an impact on social and physical activity, as well as participation in daily life. Participation and quality of life (QOL) are 2 important measures of successful rehabilitation that have not been well studied in children with CP., Objective: To report levels of participation and examine the relationship of participation to QOL in ambulatory children with CP., Design: Secondary analysis, cross-sectional cohort study., Setting: Regional pediatric specialty care center., Participants: A cohort of 128 ambulatory children with CP, Gross Motor Function Classification System levels I-III, age 2-9 years., Interventions: Not applicable., Main Outcome Measures: Pediatric Quality of Life Inventory; Assessment of Life Habits; and Children's Assessment of Participation and Enjoyment and Assessment of Preschool Children's Participation., Results: Participation was negatively associated with increasing Gross Motor Function Classification System but not age. Level of participation was associated with psychosocial QOL (2.97, P = .001) and total QOL (54.70, P = .03) but not physical QOL. There was a positive relationship between physical activity performance (0.63, P = .001), walking performance (0.002, P = .01), and communication level (7.23, P = .05) with physical QOL. Increasing age and decreased frequency of participation were negatively associated with all QOL domains., Conclusions: The results suggest that participation in daily life is negatively influenced by physical impairment and that levels of participation are positively associated with psychosocial and total QOL among ambulatory children with CP. This suggests that participation influences QOL and further studies are needed to determine the aspects of participation directly impacting QOL in ambulatory children with CP., Level of Evidence: III., (Copyright © 2018 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.)

Background: Neonatal nurse practitioner (NNP) procedural competence is essential. Procedural simulation provides opportunity to practice high-risk, low-frequency procedures and helps improve skill retention. A formal procedural simulation experience was created in an attempt to provide sufficient experience for NNPs. While preparing for the simulation experience, the group recognized the lack of validated competency metrics for most neonatal intensive care unit (NICU) procedures., Purpose: The purpose of this study was to create and validate procedural checklists measuring competence of NNPs participating in simulated performance of 9 high-risk NICU procedures., Methods: IRB approval was obtained and NNPs agreed to data collection. A self and peer assessment tool was developed and distributed to NNPs, nurses, and neonatologists to gather a baseline perception of procedural competency. Procedural checklists were then developed to measure/assess individual skills against a standard. Benchmark videos were created to demonstrate the standard approach to procedural performance. Each procedural skill performed by the NNP participant was scored by 3 individual evaluators: once in real time and twice by video recording analysis., Findings/results: A Cronbach α was used to measure the reliability and validity of the procedural checklists. Comparison was made between live grading and video-based grading scores using analysis of variance. Difference between grading modalities was less than 3% and nonsignificant for 8 of 9 procedures. No significant difference was found between individual graders for any of the skills being evaluated and suggested high interrater reliability of the checklists., Implications for Practice: Objective, standardized procedural checklists are valid tools to evaluate NNP procedural skills in simulation. Procedural simulation experiences help strengthen skills, improve performance, and possibly improve patient outcomes.

Purpose: The study purpose was to determine the minimum number of monitoring days necessary to reliably capture walking among individuals with lower limb salvage., Methods: Nineteen participants with lower limb salvage wore an ankle-mounted motion sensor over a 7-day period to obtain step counts. Generalizability theory was used to examine the variance components in step counts (G study) and to determine the appropriate length of activity monitoring using various combinations of days (D study)., Results: Mean step counts were higher on weekends than on weekdays. Fifty percent of the total variance in step counts was accounted for by interindividual variability in walking (D study). Eighty percent was reached individually with 4 weekdays, 2 weekend days, or 3 week days + weekend days., Conclusion: The study provides data for an appropriate monitoring method to track walking outcomes of rehabilitation for individuals with lower limb salvage.