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Physical and occupational therapists play important roles in wheelchair prescription for children with disabilities. Matching a child's seating and functional needs with a particular power mobility device can be challenging, especially as more power wheelchair options become available. Information about potential performance in home and school settings cannot be inferred from manufacturer's basic descriptions of wheelchairs. In this article, we describe features of a newly available power wheelchair for children and young adults and also report on the evaluation of this wheelchair using a structured protocol to assess functional capacity. The focus of the study was the wheelchair's performance in commonly accessed environments. To allow evaluation of maximal performance of all features, an able-bodied child was the subject of this study. In this wheelchair, the subject was successful in performing indoor functional activities, maneuvering on smooth level surfaces, traversing rough terrain, and in negotiating curbs. [ABSTRACT FROM AUTHOR]

Objective: While prior studies have found parental socioeconomic status (SES) affects the outcomes of pediatric traumatic brain injury (TBI), the longitudinal trajectory of this effect is not well understood., Methods: This prospective cohort study included children 8-18 years of age admitted to six sites with a complicated mild (n = 123) or moderate-severe TBI (n = 47). We used caregiver education and household poverty level as predictors, and multiple quality of life and health behavior domains as outcomes. Differences at 6, 12, and 24 months from baseline ratings of pre-injury functioning were compared by SES. We examined the association between measures of SES and domains of functioning over the 24 months post-injury in children with a complicated mild or moderate- severe TBI, and determined how this association varied over time., Results: Parental education was associated with recovery among children with complicated mild TBI; outcomes at 6, 12, and 24 months were substantially poorer than at baseline for children with the least educated parents. After moderate-severe TBI, children in households with lower incomes had poorer outcomes compared to baseline across time., Implications: Parental education and household income were associated with recovery trajectories for children with TBI of varying severity.

This study created a framework incorporating provider perspectives of best practices for early psychosocial intervention to improve caregiver experiences and outcomes after severe pediatric traumatic brain injury (TBI). A purposive sample of 23 healthcare providers from the emergency, intensive care, and acute care departments, was selected based on known clinical care of children with severe TBI at a level 1 trauma center and affiliated children's hospital. Semistructured interviews and directed content analysis were used to assess team and caregiver communication processes and topics, prognostication, and recommended interventions. Providers recommended a dual approach of institutional and individual factors contributing to an effective framework for addressing psychosocial needs. Healthcare providers recommended interventions in three domains: (1) presenting coordinated, clear messages to caregivers, (2) reducing logistical and emotional burden of care transitions, and (3) assessing and addressing caregiver needs and concerns. Specific family-centered and trauma-informed interventions included: (1) creating and sharing interdisciplinary plans with caregivers, (2) coordinating prognostication meetings and communications, (3) tracking family education, (4) improving institutional coordination and workflow, (5) training caregivers to support family involvement, (6) performing biopsychosocial assessment, and (7) using systematic prompts for difficult conversations and to address family needs at regular intervals. Healthcare workers from a variety of disciplines want to incorporate certain trauma-informed and family-centered practices at each stage of treatment to improve experiences for caregivers and outcomes for pediatric patients with severe TBI. Future research should test the feasibility and effectiveness of incorporating routine psychosocial interventions for these patients.

Purpose The purpose of this study was to evaluate the internal consistency and construct validity of the Traumatic Brain Injury Quality of Life Communication Item Bank (TBI-QOL COM) short form as a parent-proxy report measure. The TBI-QOL COM is a patient-reported outcome measure of functional communication originally developed as a self-report measure for adults with traumatic brain injury (TBI), but it may also be valid as a parent-proxy report measure for children who have sustained TBI. Method One hundred twenty-nine parent-proxy raters completed the TBI-QOL COM short form 6 months postinjury as a secondary aim of a multisite study of pediatric TBI outcomes. The respondents' children with TBI were between 8 and 18 years old ( M age = 13.2 years old) at the time of injury, and the proportion of TBI severity mirrored national trends (73% complicated-mild; 27% moderate or severe). Results The parent-proxy report version of the TBI-QOL COM displayed strong internal consistency (ordinal α = .93). It also displayed evidence of known-groups validity by virtue of more severe injuries associated with more abnormal scores. The instrument also showed evidence of convergent and discriminant validity by displaying a pattern of correlations with other constructs according to their conceptual relatedness to functional communication. Conclusions This preliminary psychometric investigation of the TBI-QOL COM supports the further development of a parent report version of the instrument. Future development of the TBI-QOL COM with this population may include expanding the content of the item bank and developing calibrations specifically for parent-proxy raters. Supplemental Material https://doi.org/10.23641/asha.7616534.

Background: As far as we know, there are no tested in-hospital care programmes for paediatric traumatic brain injury. We aimed to assess implementation and effectiveness of the Pediatric Guideline Adherence and Outcomes (PEGASUS) programme in children with severe traumatic brain injury., Methods: We did a prospective hybrid implementation and effectiveness study at the Harborview Medical Center (Seattle, WA, USA). We included children (aged <18 years) with traumatic brain injury (trauma mechanism and image findings). We assessed service provision, adherence to three key performance indicators, and discharge outcomes associated with the PEGASUS programme. The three key performance indicators were early initiation of enteral (oral or tube feeds) or parenteral nutrition; avoidance of any unwanted hypocarbia (PaCO 2 <30 mm Hg) without brain herniation; and maintenance of cerebral perfusion pressure (>40 mm Hg) for 72 h after the diagnosis of severe traumatic brain injury. Poisson regression with robust standard errors was used to estimate the association between adhering to key performance indicators and discharge outcomes., Findings: Between May 1, 2011, and July 1, 2017, 199 children (median age 11·9 years [IQR 3·4-16·1]) participated in the PEGASUS programme, of whom 193 (97%) had severe traumatic brain injury and six (3%) had moderate traumatic brain injury. 105 patients contributed data for all three key performance indicators. Adherence to at least one key performance indicator was achieved by 101 (96%) of 105 participants, and 44 (42%) achieved adherence to all three key performance indicators. Programme participants achieved adherence to the key performance indicators of hypocarbia (76 of 105 [72%]), nutrition (162 of 199 [81%]), and cerebral perfusion pressure (128 of 199 [64%]). Adherence to the nutrition key performance indicator was associated with higher discharge survival (relative risk [RR] 2·70, 95% CI 1·54-4·73) and a more favourable discharge disposition (3·05, 1·52-6·11). Adherence to the cerebral perfusion pressure key performance indicator was also associated with higher discharge survival (RR 1·33, 95% CI 1·12-1·59) and favourable disposition (1·53, 1·19-1·96). Adherence to each additional key performance indicator was associated with higher survival (RR 1·27, 1·12-1·44) and a more favourable discharge disposition (1·46, 1·23-1·72), in a dose-response manner., Interpretation: The multilevel, hospital-wide, high-fidelity PEGASUS programme might benefit children and adolescents admitted to the emergency department with severe traumatic brain injury. Cerebral perfusion pressure, nutrition, and hypocarbia targets are essential components of the PEGASUS programme and are associated with favourable discharge outcomes., Funding: National Institutes of Health., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Objectives: In this study, we describe unmet service needs of children hospitalized for traumatic brain injury (TBI) during the first 2 years after injury and examine associations between child, family, and injury-related characteristics and unmet needs in 6 domains (physical therapy, occupational therapy, speech therapy, mental health services, educational services, and physiatry)., Methods: Prospective cohort study of children age 8 to 18 years old admitted to 6 hospitals with complicated mild or moderate to severe TBI. Service need was based on dysfunction identified via parent-report compared with retrospective baseline at 6, 12, and 24 months. Needs were considered unmet if the child had no therapy services in the previous 4 weeks, no physiatry services since the previous assessment, or no educational services since injury. Analyses were used to compare met and unmet needs for each domain and time point. Generalized multinomial logit models with robust SEs were used to assess factors associated with change in need from pre-injury baseline to each study time point., Results: Unmet need varied by injury severity, time since injury, and service domain. Unmet need was highest for physiatry, educational services, and speech therapy. Among children with service needs, increased time after TBI and complicated mild TBI were associated with a higher likelihood of unmet rather than met service needs., Conclusions: Children hospitalized for TBI have persistent dysfunction with unmet needs across multiple domains. After initial hospitalization, children with TBI should be monitored for functional impairments to improve identification and fulfillment of service needs., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2018 by the American Academy of Pediatrics.)

Objective: The aim of this study was to develop and evaluate the content validity of quality criteria for providing patient- and family-centered injury care., Background: Quality criteria have been developed for clinical injury care, but not patient- and family-centered injury care., Methods: Using a modified Research AND Development Corporation (RAND)/University of California, Los Angeles (UCLA) Appropriateness Methodology, a panel of 16 patients, family members, injury and quality of care experts serially rated and revised criteria for patient- and family-centered injury care identified from patient and family focus groups. The criteria were then sent to 384 verified trauma centers in the United States, Canada, Australia, and New Zealand for evaluation., Results: A total of 46 criteria were rated and revised by the panel over 4 rounds of review producing 14 criteria related to clinical care (n = 4; transitions of care, pain management, patient safety, provider competence), communication (n = 3; information for patients/families; communication of discharge plans to patients/families, communication between hospital and community providers), holistic care (n = 4; patient hygiene, kindness and respect, family access to patient, social and spiritual support) and end-of-life care (n = 3; decision making, end-of-life care, family follow-up). Medical directors, managers, or coordinators representing 254 trauma centers (66% response rate) rated 12 criteria to be important (95% of responses) for patient- and family-centered injury care. Fewer centers rated family access to the patient (80%) and family follow-up after patient death (65%) to be important criteria., Conclusions: Fourteen-candidate quality criteria for patient- and family-centered injury care were developed and shown to have content validity. These may be used to guide quality improvement practices.

Background: Mild traumatic brain injury injuries (mTBIs), including concussions, represent >2 million US pediatric emergency department visits annually. Post-mTBI mental health symptoms are prominent and often attributed to the mTBI. This study examined whether individuals seeking post-mTBI mental health care had previous mental health diagnoses or a new onset of such disorders, and determined if mental health care utilization differed by race/ethnicity., Methods: Retrospective cohort study, using the Medicaid Marketscan claims national dataset (2007-2012). Utilization of mental health services 1 year before and 1 year after mTBI was compared between children with and without mental health diagnoses before injury. Primary outcome was receipt of post-mTBI outpatient mental health care., Results: A total of 31 272 children 20 years or younger were included, 8577 (27%) with mental health diagnoses before their mTBI and 22 695 without one. After injury, children without previous mental health disorders increased mental health services utilization; however, most (86%) postinjury mental health care was received by children with previous mental health disorders. Having a mental health diagnosis pre-mTBI was the most important risk factor for receiving post-mTBI mental health care (odds ratio 7.93, 95% confidence interval 7.40-8.50). Hispanic children were less likely to receive post-mTBI mental health care., Conclusions: mTBI was associated with increased utilization of mental health services but most of these services were received by children with previous mental health disorders. Our documentation of racial/ethnic disparities in mental health care utilization reemphasize the importance of providing individualized, culturally, and linguistically competent care to improve outcomes after mTBI for all children., (Copyright © 2017 by the American Academy of Pediatrics.)

Objectives: To describe the prevalence of postdischarge outpatient rehabilitation among Medicaid-insured children hospitalized with a traumatic brain injury (TBI) and to identify factors associated with receipt of services., Methods: Retrospective cohort of children <21 years, hospitalized for a TBI between 2007 and 2012, from a national Medicaid claims database. Outcome measures were receipt of outpatient rehabilitation (physical, occupational, or speech therapies or physician visits to a rehabilitation provider) 1 and 3 years after discharge. Multivariable regression analyses determined the association of demographic variables, injury severity, and receipt of inpatient services with receipt of outpatient rehabilitation at 1 and 3 years. The mean number of services was compared between racial/ethnic groups., Results: Among 9361 children, only 29% received any type of outpatient rehabilitation therapy during the first year after injury, although 62% sustained a moderate to severe TBI. The proportion of children receiving outpatient therapies declined to 12% in the second and third years. The most important predictor of receipt of outpatient rehabilitation was receipt of inpatient therapies or consultation with a rehabilitation physician during acute care. Compared with children of other racial/ethnic groups, Hispanic children had lower rates of receipt of outpatient speech therapy., Conclusions: Hospitalized children who received inpatient assessment of rehabilitation needs were more likely to continue outpatient rehabilitation care. Hispanic children with TBI were less likely than non-Hispanics to receive speech therapy. Interventions to increase inpatient rehabilitation during acute care might increase outpatient rehabilitation and improve outcomes for all children., (Copyright © 2016 by the American Academy of Pediatrics.)

Objective: The objective of this study was to explore associations between English proficiency, insurance status, outpatient rehabilitation service availability, and travel time for children with traumatic brain injury., Design: The authors used an ecologic cross-sectional design. Data were analyzed from a cohort of 82 children with moderate to severe traumatic brain injury and rehabilitation providers in Washington State. Main measures included availability and travel time to services., Results: Less than 20% of providers accepted children with Medicaid and provided language interpretation. Mental health services were most limited. Adjusted for median household income, multilingual service availability was lowest in counties with greater language diversity; for every 10% increase in persons older than 5 yrs speaking a language other than English at home, there was a 34% decrease in availability of multilingual services (prevalence ratio, 0.66; 95% confidence interval, 0.48-0.90). Adjusted for education and Medicaid status, children from Spanish-speaking families had significantly longer travel times to services (mean, 16 additional minutes to mental health; 9 to other therapies)., Conclusions: Children in households with limited English proficiency and Medicaid faced significant barriers in availability and proximity of outpatient rehabilitation services. Innovative service strategies are needed to equitably improve availability of rehabilitation for children with traumatic brain injury. Similar studies in other regions will inform one's understanding of the scope of these disparities.

Cilia are microtubule-based projections that function in the movement of extracellular fluid. This requires cilia to be: (1) motile and driven by dynein complexes and (2) correctly polarized on the surface of cells, which requires planar cell polarity (PCP). Few factors that regulate both processes have been discovered. We reveal that C21orf59/Kurly (Kur), a cytoplasmic protein with some enrichment at the base of cilia, is needed for motility; zebrafish mutants exhibit characteristic developmental abnormalities and dynein arm defects. kur was also required for proper cilia polarization in the zebrafish kidney and the larval skin of Xenopus laevis. CRISPR/Cas9 coupled with homologous recombination to disrupt the endogenous kur locus in Xenopus resulted in the asymmetric localization of the PCP protein Prickle2 being lost in mutant multiciliated cells. Kur also makes interactions with other PCP components, including Disheveled. This supports a model wherein Kur plays a dual role in cilia motility and polarization., (Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.)

Objective: To examine variations in processes of pediatric inpatient rehabilitation care related to family-centered care, management of neurobehavioral and psychosocial needs, and community reintegration after traumatic brain injury., Setting: Nine acute rehabilitation facilities from geographically diverse areas of the United States., Participants: A total of 174 children with traumatic brain injury., Design: Retrospective chart review., Main Measures: Adherence to care indicators (the number of times recommended care was delivered or attempted divided by the number of times care was indicated)., Results: Across facilities, adherence rates (adjusted for difficulty of delivery) ranged from 33.6% to 73.1% (95% confidence interval, 13.4-53.9, 58.7-87.4) for family-centered processes, 21.3% to 82.5% (95% confidence interval, 6.6-36.1, 67.6-97.4) for neurobehavioral and psychosocial processes, and 22.7% to 80.3% (95% confidence interval, 5.3-40.1, 68.1-92.5) for community integration processes. Within facilities, standard deviations for adherence rates were large (24.3-34.9, family-centered domain; 22.6-34.2, neurobehavioral and psychosocial domain; and 21.6-40.5, community reintegration domain)., Conclusion: The current state of acute rehabilitation care for children with traumatic brain injury is variable across different quality-of-care indicators addressing neurobehavioral and psychosocial needs and facilitating community reintegration of the patient and the family. Individual rehabilitation facilities demonstrate inconsistent adherence to different indicators and inconsistent performance across different care domains.

This study aimed to determine the prevalence and predictors of poor 3 and 12 month quality of life outcomes in a cohort of pediatric patients with isolated mild TBI. We conducted a prospective cohort study of children and adolescents <18 years of age treated for an isolated mild TBI, defined as "no radiographically apparent intracranial injury" or "an isolated skull fracture, and no other clinically significant non-brain injuries." The main outcome measure was the change in quality of life from baseline at 3 and 12 months following injury, as measured by the Pediatric Quality of Life index (PedsQL). Poor functioning was defined as a decrease in total PedsQL score of >15 points between baseline and follow-up scores (at 3 and 12 months). Of the 329 patients who met inclusion criteria, 11.3% (95% CI 8.3-15.3%) at 3 months and 12.9% (95% CI 9.6-17.2%) at 12 months following injury had relatively poor functioning. Significant predictors of poor functioning included less parental education, Hispanic ethnicity (at 3 months following injury, but not at 12 months); low household income (at 3 and 12 months), and Medicaid insurance (at 12 months only). Children and adolescents sustaining a mild TBI who are socioeconomically disadvantaged may require additional intervention to mitigate the effects of mild TBI on their functioning.

Unlabelled: Traumatic brain injury (TBI) is a leading cause of pediatric disability. Although persistent pain has been recognized as a significant postinjury complication, there is a paucity of data concerning the postinjury pain experience of youth. This study aimed to examine the prevalence of persistent pain in adolescents after TBI, identify risk factors for pain, and evaluate the impact of pain on adolescent health-related quality of life. Participants included 144 adolescents with mild to severe TBI who were followed over 36 months after injury. At 3-, 12-, 24-, and 36-month assessments, measures of pain intensity, depression, posttraumatic stress disorder, and health-related quality of life were completed by adolescents. Findings demonstrated that 24.3% of adolescents reported persistent pain (defined as usual pain intensity ≥3/10) at all assessment points after TBI. Female sex (odds ratio = 2.73, 95% confidence interval = 1.12-6.63) and higher levels of depressive symptoms at 3 months after injury (odds ratio = 1.26, 95% confidence interval = 1.12-1.43) were predictors of persistent pain at 36 months. Furthermore, mixed linear models indicated that early pain experience at 3 months following TBI was associated with a significantly poorer long-term health-related quality of life., Perspective: This is the first study to examine the prevalence of persistent pain over long-term follow-up in adolescents after TBI and its impact on health-related quality of life. These findings indicate that adolescents with TBI may benefit from timely evaluation and intervention to minimize the development and impact of pain., (Copyright © 2013 American Pain Society. Published by Elsevier Inc. All rights reserved.)

Objective: To compare the extent of disability in multiple areas of functioning after mild, moderate, and severe traumatic brain injury (TBI) between Hispanic and non-Hispanic white (NHW) children., Methods: This was a prospective cohort study of children aged <18 years treated for a TBI between March 1, 2007, and September 30, 2008. Hispanic (n = 74) and NHW (n = 457) children were included in the study. Outcome measures were disability in health-related quality of life, adaptive skills, and participation in activities 3, 12, 24, and 36 months after injury compared with preinjury functioning. We compared change in outcome scores between Hispanic and NHW children at each follow-up time. All analyses were adjusted for age, gender, severity and intent of injury, insurance, family function at baseline, parental education, and income., Results: The health-related quality of life for all children was lower at all follow-up times compared with baseline. Although NHW children showed some improvement during the first 3 years after injury, Hispanic children remained significantly impaired. Significant differences were also observed in the domains of communication and self-care abilities after TBI. Differences between groups in scores for participation in activities were also present but were only significant 3 months after injury., Conclusions: Hispanic children with TBI report larger and long-term reductions in their quality of life, participation in activities, communication, and self-care abilities compared with NHW children. The reasons for these differences need to be better understood and interventions implemented to improve the outcomes of these children.

Objective: To examine variations in processes of paediatric inpatient rehabilitation care related to school re-entry and management of cognitive and communication impairments after traumatic brain injury., Design: Retrospective cohort study., Methods: Adherence to care processes recommended for children (aged 0-17) with moderate-to-severe traumatic brain injury and admitted for inpatient rehabilitation was assessed. Quality-of-care indicators for processes supporting school re-entry and cognitive and communication rehabilitation were applied to measure variations in care delivered to 174 children across nine facilities using medical record review., Main Outcomes and Results: Adherence rates (the number of times recommended care was delivered or attempted divided by the number of times care was indicated) were calculated, revealing substantial variations in care within and between facilities. Overall, children received 51.3% (95% CI = 31.9-70.7) and 72.3% (95% CI = 61.1-83.5), of the care recommended for school re-entry and cognitive and communication rehabilitation, respectively., Conclusion: Substantial variations exist in the delivery of paediatric inpatient rehabilitation care processes for managing school re-entry and cognitive and communication impairments after traumatic brain injury. Measures of association of these care processes with patient outcomes are necessary. Reduction in this variation is essential to improving quality of care.

Objectives: We examined the burden of disability resulting from traumatic brain injuries (TBIs) among children younger than 18 years., Methods: We derived our data from a cohort study of children residing in King County, Washington, who were treated in an emergency department for a TBI or for an arm injury during 2007-2008. Disabilities 12 months after injury were assessed according to need for specialized educational and community-based services and scores on standardized measures of adaptive functioning and social-community participation., Results: The incidence of children receiving new services at 12 months was about 10-fold higher among those with a mild TBI than among those with a moderate or severe TBI. The population incidence of disability (defined according to scores below the norm means on the outcome measures included) was also consistently much larger (2.8-fold to 28-fold) for mild TBIs than for severe TBIs., Conclusions: The burden of disability caused by TBIs among children is primarily accounted for by mild injuries. Efforts to prevent these injuries as well as to decrease levels of disability following TBIs are warranted.

This study examined the outcome of 0- to 17-year-old children 36 months after traumatic brain injury (TBI), and ascertained if there was any improvement in function between 24 and 36 months. Controls were children treated in the emergency department for an arm injury. Functional outcome 36 months after injury was measured by the Pediatric Quality of Life Inventory (PedsQL), the self-care and communication subscales of the Adaptive Behavior Assessment Scale-2nd edition (ABAS-II), and the Child and Adolescent Scale of Participation (CASP). At 36 months after TBI, those with moderate or severe TBI continued to have PedsQL scores that were 16.1 and 17.9 points, respectively, lower than at baseline, compared to the change seen among arm injury controls. Compared to the baseline assessment, children with moderate or severe TBI had significantly poorer functioning on the ABAS-II and poorer participation in activities (CASP). There was no significant improvement in any group on any outcomes between 24 and 36 months. Post-injury interventions that decrease the impact of these deficits on function and quality of life, as well as preventive interventions that reduce the likelihood of TBI, should be developed and tested.

A growing number of transcriptional regulatory proteins are known to be modified by the small ubiquitin-like protein, SUMO. Posttranslational modification by SUMO may be one means by which transcriptional regulatory factors that play context-dependent roles in multiple processes can be regulated such that they direct the appropriate cellular and developmental outcomes. In early vertebrate embryos, SUMOylation of SoxE transcription factors profoundly affects their function, inhibiting their neural crest-inducing activity and promoting ear formation. In this paper, we provide mechanistic insight into how SUMO modification modulates SoxE function. We show that SUMOylation dramatically altered recruitment of transcriptional coregulator factors by SoxE proteins, displacing coactivators CREB-binding protein/p300 while promoting the recruitment of a corepressor, Grg4. These data demonstrate that SoxE proteins can function as transcriptional repressors in a SUMO-dependent manner. They further suggest a novel multivalent mechanism for SUMO-mediated recruitment of transcriptional coregulatory factors.

Background: Traumatic brain injury (TBI) is a leading cause of acquired disability in children and adolescents., Objective: To demonstrate the association between specific findings on initial noncontrast head CT and long-term outcomes in children who have suffered TBI., Materials and Methods: This was an IRB-approved prospective study of children ages 2-17 years treated in emergency departments for TBI and who underwent a head CT as part of the initial work-up (n = 347). The change in quality of life at 12 months after injury was measured by the PedsQL scale., Results: Children with TBI who had intracranial injuries identified on the initial head CT had a significantly lower quality-of-life scores compared to children with TBI whose initial head CTs were normal. In multivariate analysis, children whose initial head CT scans demonstrated intraventricular hemorrhage, parenchymal injury, midline shift ≥ 5 mm, hemorrhagic shear injury, abnormal cisterns or subdural hematomas ≥ 3 mm had lower quality of life scores 1 year after injury than children whose initial CTs did not have these same injuries., Conclusion: Associations exist between findings from the initial noncontrast head CT and quality of life score 12 months after injury in children with TBI.

Objective: To determine variations in care provided by 9 inpatient rehabilitation units for children with traumatic brain injury (TBI) using newly developed quality indicators., Design: Retrospective cohort study., Setting: Nine inpatient rehabilitation units., Participants: Children (N=174; age range, 0-17y) admitted for the inpatient rehabilitation of moderate to severe TBI., Interventions: Not applicable., Main Outcome Measures: Adherence to 119 newly developed quality-of-care indicators in 7 different domains: general care, family-centered care, cognitive-communication, motor, neuropsychological, school, and community integration., Results: There was substantial variation both within and between institutions in the percentage of patients receiving recommended care in the 7 domains. The lowest scores were found for the school domain. Only 5 institutions scored above 50% for all quality indicators, and only 1 institution scored above 70% overall. Greater adherence to quality indicators was found for facilities with a higher proportion of therapists with pediatric training and for facilities that only admitted children. Patient volume was not associated with adherence to quality indicators., Conclusions: The results indicate a tremendous variability and opportunity for improvement in the care of children with TBI., (Copyright © 2012 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.)

The degree to which postinjury posttraumatic stress disorder (PTSD) and/or depressive symptoms in adolescents are associated with cognitive and functional impairments at 12 and 24 months after traumatic brain injury (TBI) is not yet known. The current study used a prospective cohort design, with baseline assessment and 3-, 12-, and 24-month followup, and recruited a cohort of 228 adolescents ages 14-17 years who sustained either a TBI (n = 189) or an isolated arm injury (n = 39). Linear mixed-effects regression was used to assess differences in depressive and PTSD symptoms between TBI and arm-injured patients and to assess the association between 3-month PTSD and depressive symptoms and cognitive and functional outcomes. Results indicated that patients who sustained a mild TBI without intracranial hemorrhage reported significantly worse PTSD (Hedges g = 0.49, p = .01; Model R(2) = .38) symptoms across time as compared to the arm injured control group. Greater levels of PTSD symptoms were associated with poorer school (η(2) = .07, p = .03; Model R(2) = .36) and physical (η(2) = .11, p = .01; Model R(2) = .23) functioning, whereas greater depressive symptoms were associated with poorer school (η(2) = .06, p = .05; Model R(2) = .39) functioning., (Copyright © 2012 International Society for Traumatic Stress Studies.)

Objectives: To develop evidence-based and expert-driven quality indicators for measuring variations in the structure and organization of acute inpatient rehabilitation for children after traumatic brain injury (TBI) and to survey centers across the United States to determine the degree of variation in care., Design: Quality indicators were developed using the RAND/UCLA modified Delphi method. Adherence to these indicators was determined from a survey of rehabilitation facilities., Setting: Inpatient rehabilitation units in the United States., Participants: A sample of rehabilitation programs identified using data from the National Association of Children's Hospitals and Related Institutions, Uniform Data System for Medical Rehabilitation, and the Commission on Accreditation of Rehabilitation Facilities yielded 74 inpatient units treating children with TBI. Survey respondents comprised 31 pediatric and 28 all age units., Interventions: Not applicable., Main Outcome Measures: Variations in structure and organization of care among institutions providing acute inpatient rehabilitation for children with TBI., Results: Twelve indicators were developed. Pediatric inpatient rehabilitation units and units with higher volumes of children with TBI were more likely to have: a census of at least 1 child admitted with a TBI for at least 90% of the time; adequate specialized equipment; a classroom; a pediatric subspecialty trained medical director; and more than 75% of therapists with pediatric training., Conclusions: There were clinically and statistically significant variations in the structure and organization of acute pediatric rehabilitation based on the pediatric focus of the unit and volume of children with TBI., (Copyright © 2012 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.)

Objective: To develop measurement tools for assessing compliance with identifiable processes of inpatient care for children with traumatic brain injury (TBI) that are reliable, valid, and amenable to implementation., Design: Literature review and expert panel using the RAND/UCLA Appropriateness Method and a Delphi technique., Setting: Not applicable., Participants: Children with TBI., Interventions: Not applicable., Main Outcome Measure: Quality of care indicators., Results: A total of 119 indicators were developed across the domains of general management; family-centered care; cognitive-communication, speech, language, and swallowing impairments; gross and fine motor skill impairments; neuropsychologic, social, and behavioral impairments; school reentry; and community integration. There was a high degree of agreement on these indicators as valid and feasible quality measures for children with TBI., Conclusions: These indicators are an important step toward building a better base of evidence about the effectiveness and efficiency of the components of acute inpatient rehabilitation for pediatric patients with TBI., (Copyright © 2012 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.)

This study aimed to examine the prevalence and trajectory of sleep disturbances and their associated risk factors in children up to 24 months following a traumatic brain injury (TBI). In addition, the longitudinal association between sleep disturbances and children's functional outcomes was assessed. This was a prospective study of a cohort of children with TBI and a comparison cohort of children with orthopedic injury (OI). Parental reports of pre-injury sleep disturbances were compared to reports of post-injury changes at 3, 12, and 24 months. Risk factors for sleep disturbances were examined, including severity of TBI, presence of psychosocial problems, and pain. Sleep disturbances were also examined as a predictor of children's functional outcomes in the areas of adaptive behavior skills and activity participation. Both cohorts (children with TBI and OI) displayed increased sleep disturbances after injury. However, children with TBI experienced higher severity and more prolonged duration of sleep disturbances compared to children with OI. Risk factors for disturbed sleep included mild TBI, psychosocial problems, and frequent pain. Sleep disturbances emerged as significant predictors of poorer functional outcomes in children with moderate or severe TBI. Children with TBI experienced persistent sleep disturbances over 24 months. Findings suggest a potential negative impact of disturbed sleep on children's functional outcomes, highlighting the need for further research on sleep in children with TBI.

Objective: To determine the prevalence of headache 3 and 12 months after pediatric traumatic brain injury (TBI)., Methods: This is a prospective cohort study of children ages 5 to 17 years in which we analyzed the prevalence of headache 3 and 12 months after mild TBI (mTBI; n = 402) and moderate/severe TBI (n = 60) compared with controls with arm injury (AI; n = 122)., Results: The prevalence of headache 3 months after injury was significantly higher after mTBI than after AI overall (43% vs 26%, relative risk [RR]: 1.7 [95% confidence interval (CI): 1.2-2.3]), in adolescents (13-17 years; 46% vs 25%, RR: 1.8 [95% CI: 1.1-3.1]), and in girls (59% vs 24%, RR: 2.4 [95% CI: 1.4-4.2]). The prevalence of headache at 3 months was also higher after moderate/severe TBI than AI in younger children (5-12 years; 60% vs 27%; RR: 2.0 [95% CI: 1.2-3.4]). Twelve months after injury, TBI was not associated with a significantly increased frequency of headache. However, girls with mTBI reported serious headache (≥ 5 of 10 pain scale rating) more often than controls (27% vs 10%, RR: 2.2 [95% CI: 0.9-5.6])., Conclusions: Pediatric TBI is associated with headache. A substantial number of children suffer from headaches months after their head injury. The prevalence of headache during the year after injury is related to injury severity, time after injury, age, and gender. Girls and adolescents appear to be at highest risk of headache in the months after TBI.

Objective: Traumatic brain injury (TBI) is a major cause of disability among US children. Our goal was to obtain population-based data on TBI incidence rates., Methods: We conducted surveillance through a stratified random sample of hospital emergency departments in King County, Washington, to identify children 0 to 17 years of age with medically treated TBIs during an 18-month study period in 2007-2008. Additional cases were identified through hospital admission logs and the medical examiner's office. For a sample of nonfatal cases, parents were interviewed to verify TBIs, and medical record data on severity and mechanisms were obtained., Results: The estimated incidence of TBIs in this setting was 304 cases per 100,000 child-years. The incidence was highest for preschool-aged children and lowest for children aged 5 to 9 years. Rates were uniformly higher for boys than for girls; there was a larger gender gap at older ages. Falls were the main mechanism of injury, especially among preschool-aged children, whereas being struck by or against an object and motor vehicle-related trauma were important contributors for older children. Approximately 97% of TBI cases were mild, although moderate/severe TBI incidence increased with age., Conclusions: TBIs led to many emergency department visits involving children, but a large majority of the cases were clinically mild. Incidence rates for King County were well below recent national estimates but within the range reported in previous US studies. Because mechanisms of injury varied greatly according to age, prevention strategies almost certainly must be customized to each age group for greatest impact.

Objective: To examine disability in children and adolescents after traumatic brain injury (TBI) across the spectrum of injury severity., Methods: This was a prospective cohort study of children younger than 18 years treated for a TBI (n = 729) or an arm injury (n = 197) between March 1, 2007, and September 30, 2008. The main outcome measures were disability in health-related quality of life, adaptive skills, and participation in social and community activities 3, 12, and 24 months after injury compared with preinjury functioning., Results: The health-related quality of life for children with moderate or severe TBI was lower at all follow-up times compared with baseline, but there was some improvement during the first 2 years after injury. Three months after injury, there was a substantial decrease in the level of activities in which children with moderate and severe TBI were able to participate; these activities improved at 12 and 24 months but were still significantly impaired. Communication and self-care abilities in children with moderate and severe TBI were lower at 3 months than at baseline and did not improve by 24 months. Children who met the definition of mild TBI but had an intracranial hemorrhage had lower quality-of-life scores at 3 months., Conclusions: Children with moderate or severe TBI and children with mild TBI who had intracranial hemorrhage had substantial long-term reduction in their quality of life, participation in activities with others, and ability to communicate and care for themselves.

Objective: To identify sociodemographic factors associated with completing a follow-up survey about health status on the web versus by telephone, and to examine differences in reported health-related quality of life by method of response., Data Sources/study Settings: Survey about child health status of 896 parents of children aged 0-17 years treated in a hospital emergency department or admitted for a traumatic brain injury or arm injury, and 227 injured adolescents aged 14-17 years., Study Design: The main outcomes were characteristics of those who completed a follow-up survey on the web versus by telephone and health-related quality of life by method of response., Principal Findings: Email addresses were provided by 76.9 percent of parents and 56.5 percent of adolescents at baseline. The survey was completed on the web by 64.9 percent of parents and 40.2 percent of adolescents through email. Parents with email access who were Blacks, Hispanics, had lower incomes, and those who were not working were less likely to choose the web mode for completing the survey. Unlike adolescents, the amount of time for parents to complete the survey online was significantly shorter than completion by telephone. Differences by survey mode were small but statistically significant in some of the six functional outcome measures examined., Conclusions: Survey mode was associated with several sociodemographic characteristics. Sole use of web surveys could provide biased data., (© Health Research and Educational Trust.)

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by formation of renal cysts that destroy the kidney. Mutations in PKD1 and PKD2, encoding polycystins-1 and -2, cause ADPKD. Polycystins are thought to function in primary cilia, but it is not well understood how these and other proteins are targeted to cilia. Here, we provide the first genetic and biochemical link between polycystins and the exocyst, a highly-conserved eight-protein membrane trafficking complex. We show that knockdown of exocyst component Sec10 yields cellular phenotypes associated with ADPKD, including loss of flow-generated calcium increases, hyperproliferation, and abnormal activation of MAPK. Sec10 knockdown in zebrafish phenocopies many aspects of polycystin-2 knockdown-including curly tail up, left-right patterning defects, glomerular expansion, and MAPK activation-suggesting that the exocyst is required for pkd2 function in vivo. We observe a synergistic genetic interaction between zebrafish sec10 and pkd2 for many of these cilia-related phenotypes. Importantly, we demonstrate a biochemical interaction between Sec10 and the ciliary proteins polycystin-2, IFT88, and IFT20 and co-localization of the exocyst and polycystin-2 at the primary cilium. Our work supports a model in which the exocyst is required for the ciliary localization of polycystin-2, thus allowing for polycystin-2 function in cellular processes., Competing Interests: The authors have declared that no competing interests exist.

Research focused on cilia as extremely important cellular organelles has flourished in recent years. A thorough understanding of cilia regulation and function is critical, as disruptions of cilia structure and/or function have been linked to numerous human diseases and disorders. The tropical freshwater zebrafish is an excellent model organism in which to study cilia structure and function. We can readily image cilia and their motility in embryonic structures including Kupffer's vesicle during somite stages and the pronephros from 1 day postfertilization onward. Here, we describe how to image cilia by whole-mount immunofluorescence, transverse cryosection/immunohistochemistry, and transmission electron microscopy. We also describe how to obtain videos of cilia motility in living embryos., (2010 Elsevier Inc. All rights reserved.)

To examine the prevalence and characteristics of pain in children with neuromuscular disease (NMD), 42 youths with NMD underwent a comprehensive evaluation including a detailed intake interview and structured questionnaire that included demographic and functional data. Youths who reported chronic pain were further queried about pain characteristics, locations, and intensity using an 11-point numerical rating scale and a modified Brief Pain Inventory (BPI). The sample consisted of 24 males (57%) and 18 females (43%), ages ranging from 9 to 20 years (M = 14.8, SD = 2.96). Participants included 14 (37%) with Duchenne muscular dystrophy, 6 (14%) with myotonic dystrophy, 2 (5%) with Becker dystrophy, 2 (5%) with limb-girdle dystrophy, 2 (5%) with congenital muscular dystrophy, 1 (2%) facioscapulohumeral, and 15 (36%) were classified as ''other NMD.'' Twenty-one (50%) were ambulatory; 26 (62%) used power wheelchairs/scooters, 9 (2%) used manual wheelchairs, 3 (.07%) used crutches/canes, and 1 (2%) used a walker. A total of 23 (55%) of the youths reported having chronic pain. Current pain intensity was 1.30 (range = 0-6), mean pain intensity over the past week was 2.39 (range = 0-7), mean pain duration was 8.75 hours (SD = 12.84). Pain in the legs was most commonly reported and 83% reported using pain medications. This study indicates that chronic pain is a significant problem in youths with NMD. These data strongly support making comprehensive pain assessment and management an integral part of the standard of care for youths with NMD.

Cilia defects have been implicated in a variety of human diseases and genetic disorders, but how cilia motility contributes to these phenotypes is still unknown. To further our understanding of how cilia function in development, we have cloned and characterized two alleles of seahorse, a zebrafish mutation that results in pronephric cysts. seahorse encodes Lrrc6l, a leucine-rich repeat-containing protein that is highly conserved in organisms that have motile cilia. seahorse is expressed in zebrafish tissues known to contain motile cilia. Although mutants do not affect cilia structure and retain the ability to interact with Disheveled, both alleles of seahorse strongly affect cilia motility in the zebrafish pronephros and neural tube. Intriguingly, although seahorse mutations variably affect fluid flow in Kupffer's vesicle, they can have very weak effects on left-right patterning. Combined with recently published results, our alleles suggest that the function of seahorse in cilia motility is separable from its function in other cilia-related phenotypes.

Objective: Traumatic brain injury has a substantial impact on caregivers. This study describes the burden experienced by caregivers of children with traumatic brain injury and examines the relationship between child functioning and family burden during the first year after injury., Patients and Methods: Children aged 5 to 15 years hospitalized for traumatic brain injury at 4 participating trauma centers were eligible. Caregivers completed baseline and 3- and 12-month telephone interviews measuring the child's health-related quality of life using the Pediatric Quality of Life Inventory. The emotional impact scale of the Child Health Questionnaire was used to identify caregivers with substantial distress, including general worry or interference with family routine. Caregiver perceptions of whether health care needs were met or unmet and days missed from work were also measured., Results: A total of 330 subjects enrolled; follow-up was conducted with 312 at 3 months and 288 at 12 months. Most subjects were white (68%) and male (69%). Abnormal Pediatric Quality of Life Inventory subscores were related to substantial caregiver burden (either general worry or interference in routine). These abnormalities were reported by >75% of patients at 3 months and persisted to 1 year in some patients. Parental perception of unmet health care needs was strongly related to family burden outcomes, with up to 69% of this subset of parents reporting substantial worry, and nearly one quarter reporting interference with daily routine/concentration 1 year after injury. Child dysfunction predicted parental burden at 3 and 12 months. Burden was greater when health care need was unmet. Abnormalities on the Pediatric Quality of Life Inventory predicted the amount of work missed by parents, especially in the presence of unmet needs., Conclusions: Caregivers are more likely to report family burden problems when child functioning is poorer and health care needs are unmet. Improved identification and provision of services is a potentially modifiable factor that may decrease family burden after pediatric traumatic brain injury.

The objective of this 12-month study was to describe the clinical features of late-onset Pompe disease and identify appropriate outcome measures for use in clinical trials. Assessments included quantitative muscle testing (QMT), functional activities (FAA), 6-min walk test (6MWT), and pulmonary function testing (PFT). Percent predicted values indicated quantifiable upper and lower extremity weakness, impaired walking ability, and respiratory muscle weakness. Significant declines in arm and leg strength and pulmonary function were observed during the study period. The outcome measures were demonstrated to be safe and reliable. Symptom duration was identified as the best predictor of the extent of skeletal and respiratory muscle weakness.

This article reviews the literature on the effectiveness of rehabilitation after pediatric trauma. The characteristics of comprehensive pediatric trauma rehabilitation services during the initial acute care hospitalization, as well as subsequent inpatient and outpatient care during the first 12 months after injury are discussed. Important research questions and the challenges associated with conducting such research are identified. For many children, rehabilitation is the final common pathway that may define the success or failure of their treatment-their ultimate outcome. Large, prospective, multicenter studies will be needed so that seriously injured children can receive standardized and effective rehabilitative care.

Objectives: To document health-related quality of life (HRQOL) of children with traumatic brain injury (TBI) and to examine the relationship between TBI severity and HRQOL during the first year after injury., Design: Prospective cohort study., Setting: Four pediatric level I trauma centers., Patients: Children with TBI (n = 330)., Main Exposure: Traumatic brain injury., Main Outcome Measures: A primary caregiver completed telephone interviews at baseline, 3 months, and 12 months to measure the child's HRQOL using the Pediatric Quality of Life Inventory. The HRQOL outcomes were modeled as a function of injury, patient characteristics, and family characteristics using longitudinal, multivariable regression., Results: A considerable proportion of children had impaired HRQOL at 3 months (42% of children) and 12 months (40% of children) after injury. Multiple dimensions of HRQOL were negatively affected among children with moderate or severe TBI (decrease of 3.7 to 17.6) (P<.05) and did not improve significantly over time. Concomitant lower extremity fractures and spinal injuries resulted in large declines in overall HRQOL, particularly at 3 months after injury (decrease of 12.9 and 8.1, respectively) (P<.05). The HRQOL scores were also reduced by preexisting psychosocial conditions (decrease of 2.9 to 12.3), impaired family functioning (decrease of 5.1 to 6.8), having Medicaid coverage or being uninsured (decrease of 3.1 to 5.5), and single-parent households (decrease of 3.2 to 3.4) (P<.05)., Conclusions: Moderate or severe TBI resulted in measurable declines in children's HRQOL after injury. Injury-related factors impacted HRQOL more compared with patient and family characteristics during the first year after injury.

Objectives: To assess the reliability and validity of the Pediatric Quality of Life Inventory, version 4.0 (PedsQL), and to compare it with that of the Behavior Rating Inventory of Executive Function (BRIEF) among children with traumatic brain injury (TBI)., Design: Prospective cohort study that documented the health-related quality of life of 391 children at 3 and 12 months postinjury., Setting: Four level I pediatric trauma centers., Participants: Children (age range, 5-15 y) hospitalized with a TBI or an extremity fracture., Interventions: Not applicable., Main Outcome Measures: Parent-reported PedsQL and BRIEF scale scores., Results: Both the PedsQL and BRIEF scales showed good internal consistency (PedsQL alpha range, .74-.93; BRIEF alpha range, .82-.98) and test-retest reliability (PedsQL r range, .75-.90; BRIEF r range, .82-.92), respectively. Factor analysis revealed that most PedsQL items loaded most highly on their conceptually derived scale. The PedsQL cognitive function scale detected the largest differences among groups of children with varying severities of TBI as well as parents' assessment of change in cognition postinjury., Conclusions: Although the reliability of the 2 instruments is comparable, the PedsQL discriminates better among children with TBI. The PedsQL is a promising instrument for measuring the health of children after TBI.

Objective: To determine the incidence of psychiatric illness 3 years after mild traumatic brain injury (TBI) in children., Design: Prospective cohort study with 3-year follow-up., Setting: Emergency department, hospital, and outpatient clinics in a large health maintenance organization., Participants: Children, 14 years old or less (n=490), who sustained a mild TBI in 1993. Three TBI unexposed subjects per TBI exposed patient were matched by sex, age, and enrollment at the time of injury (n=1470)., Interventions: Not applicable., Main Outcome Measures: Computerized records were examined to identify psychiatric diagnoses, psychiatric medication prescription, and utilization of psychiatric services for the year before TBI and 3 years after. Adjusted relative risks for incidence of psychiatric illness were estimated for those with and without a premorbid psychiatric disorder., Results: The cumulative incidence estimates for any psychiatric illness in the 3 years after mild TBI were 30% in children exposed to mild TBI and 20% in unexposed children (P=.0001). Cumulative incidence estimates were particularly high in both TBI exposed (55%) and unexposed children (63%) who had psychiatric illness in the year before the index TBI (psychiatric history). The exposed and unexposed children with psychiatric history did not have significantly different estimates of incidence during follow-up for any of the studied indicators of psychiatric illness. In those with no psychiatric history, 26% of exposed and 16% of unexposed children (P<.0001) had evidence of a psychiatric illness in the 3 years after mild TBI. For those with no psychiatric history, the adjusted relative risk estimate of any psychiatric illness in TBI exposed versus unexposed children, in the first year after TBI, was 2.03 (95% confidence interval [CI], 1.4-2.9). Children with mild TBI but no psychiatric history were at higher risk for hyperactivity (diagnosis of hyperkinetic syndrome of childhood or prescription of psychostimulants) in the first year after injury (incidence, 3%; first year relative risk, 7.59; 95% CI, 2.7-21.6)., Conclusions: In the 3 years after mild TBI, children with no evidence of prior-year psychiatric history were at significantly increased risk for psychiatric illness, particularly hyperactivity in the first year after injury. Prior-year psychiatric history conferred a significant independent risk for subsequent psychiatric illness. There was no evidence for an additional increase in risk in the 3-year follow-up that is attributable to mild TBI in children with prior psychiatric history.

Fibrocartilaginous embolism is a rare cause of spinal cord infarction. It is postulated that an acute vertical disk herniation of the nucleus pulposus material can lead to spinal cord infarction by a retrograde embolization to the central artery. An increased intradiskal pressure resulting from axial loading of the vertebral column with a concomitant Valsalva maneuver is thought to be the initiating event for the embolus. We present a previously healthy 16-year-old boy with sudden onset of back pain and progressive paraparesis within 36 hours after lifting exercises in a squat position. His clinical presentation and neuroimaging studies were consistent with spinal cord infarction resulting from a central artery embolus at the T8 spinal cord level. Laboratory investigation showed no evidence of infectious, autoimmune, inflammatory, or neoplastic causes. Although no histologic confirmation was obtained, lack of evidence for other plausible diagnoses in the setting of his clinical presentation and in the magnetic resonance imaging findings made fibrocartilaginous embolism myelopathy the most likely diagnosis. We postulated that some cases of transverse myelitis might actually be fibrocartilaginous embolism, making it a more prevalent cause of an acute myelopathy than commonly recognized. Relevant literature and current theories regarding the pathogenesis of fibrocartilaginous embolism myelopathy are reviewed.

Objective: To assess functional outcome and describe disability at discharge in children who have had trauma without significant head injury., Design: Retrospective cohort., Setting: National Pediatric Trauma Registry, 1988-1994., Participants: Patients of ages 7 to 18 years with Glasgow Coma Scale (GCS) 13 to 15 without significant anatomic head inJury., Results: Functional Independence Measure (FIM) at discharge was used to assess patient outcome. There were 13,649 children meeting study criteria who had sustained 34,254 injuries. Fractures constituted 30% of all injuries. As measured by FIM, 1,522 (11.2%) patients had mild disability at discharge; 1,983 (14.5%) had moderate disability. After adjustment for age and injury severity, children with lower extremity fractures were more likely to be discharged with functional limitations than those without (relative risk, 5.43; 95% confidence interval: 5.06, 5.84). Of children with moderate disability at discharge, less than 50% were referred for rehabilitation evaluation and less than 25% for physical therapy., Conclusion: Functional dependence is present in a large proportion of injured children, even without significant head injury. Rehabilitation and other services may be underused in this population. Further study is required to fully assess the degree and duration of disability in these patients.