Motor training for young children with cerebral palsy: A single-blind randomized controlled trial.

AIM: To compare the effect of iMOVE (Intensive Mobility training with Variability and Error) therapy with dose-matched conventional therapy on gross motor development and secondary outcomes in young children with cerebral palsy. METHOD: This single-blind, randomized controlled trial included repeated assessments of gross motor function (using the Gross Motor Function Measure) and secondary outcomes during a 12- to 24-week intervention phase and at three follow-up points after treatment. Treatment was delivered three times per week in both groups. Forty-two children aged 12 to 36 months were stratified by age and motor function to ensure equivalence between groups at baseline. RESULTS: Thirty-six children completed treatment and follow-up phases. Treatment fidelity was high and adherence was equivalent between groups (77.3% conventional therapy, 76.2% iMOVE). There were no group differences on the primary (gross motor function after 12 weeks p = 0.18; after 24 weeks p = 0.94) or any secondary (postural control p = 0.88, caregiver satisfaction p = 0.52, child engagement p = 0.98) measure after treatment or at the follow-up points. However, one-third of total participants exceeded predicted change after 12 weeks and 77% exceeded predicted change after 24 weeks of treatment. INTERPRETATION: Our observations indicate a potential dose-response effect of rehabilitation therapy. We further demonstrated that individual therapeutic ingredients can be manipulated. When delivered consistently, both iMOVE and conventional therapy interventions might both be more effective than standard care. WHAT THIS PAPER ADDS: Those receiving iMOVE therapy demonstrated more independent practice time, error, and child-initiation than those receiving the dose-matched control. iMOVE therapy was not superior to the control (conventional physical) therapy. Most participants exceeded predicted change after 24 weeks of treatment.

Development of a neurologic severity scale for Aicardi Goutières Syndrome.

BACKGROUND AND PURPOSE: Aicardi Goutières Syndrome (AGS) is a severe, autoinflammatory leukodystrophy characterized by global neurologic dysfunction. Our goal was to create an easy-to-apply scale relevant to the unique developmental challenges associated with AGS. METHODS: All individuals were recruited through our natural history study. Individuals were classified by AGS severity as mild, moderate, or severe, and clinical encounters were assigned a composite score for neurologic function calculated from the sum of three functional classification scales. Through expert consensus, we identified 11 key items to reflect the severity of AGS across gross motor, fine motor, and cognitive skills to create the AGS Scale. There was strong interrater reliability. The AGS scale was applied across available medical records to evaluate neurologic function over time. The AGS scale was compared to performance on a standard measure of gross motor function (Gross Motor Function Measure-88, GMFM-88) and a putative diagnostic biomarker of disease, the interferon signaling gene expression score (ISG). RESULTS: The AGS scale score correlated with severity classifications and the composite neurologic function scores. When retrospectively applied across our natural history study, the majority of individuals demonstrated an initial decline in function followed by stable scores. Within the first 6 months of disease, the AGS score was the most dynamic. The AGS scale correlated with performance by the GMFM-88, but did not correlate with ISG levels. CONCLUSIONS: This study demonstrates the utility of the AGS scale as a multimodal tool for the assessment of neurologic function in AGS. The AGS scale correlates with clinical severity and with a more labor-intensive tool, GMFM-88. This study underscores the limitations of the ISG score as a marker of disease severity. With the AGS scale, we found that AGS neurologic severity is the most dynamic early in disease. This novel AGS scale is a promising tool to longitudinally follow neurologic function in this unique population.

Orofacial Manifestations of Stickler Syndrome: An Analysis of Speech Outcome and Facial Growth After Cleft Palate Repair.

PURPOSE: The purpose of this study was to characterize airway problems, speech outcomes, and facial growth in patients with Stickler syndrome undergoing cleft palate repair. METHODS: A retrospective, longitudinal study was performed at the Children's Hospital of Philadelphia on 25 patients with Stickler syndrome and 53 nonsyndromic patients with clefts of the secondary palate repaired between 1977 and 2000. Airway problems were characterized by the incidence of Pierre Robin Sequence (PRS) and the necessity for surgical airway management. Speech was analyzed using the Pittsburgh weighted values for speech symptoms associated with velopharyngeal incompetence (VPI). Longitudinal anthropometric measurements represented up to 12 years of longitudinal cephalofacial growth. RESULTS: Seventy-two percent of patients with Stickler syndrome were diagnosed with PRS, 55.6% of whom required surgical airway management. Conversely, 20.8% of nonsyndromic patients were diagnosed with PRS (P < 0.0001), 18% of whom required surgical intervention (P < 0.05). Speech outcomes were poorer in patients with Stickler syndrome with 40% demonstrating borderline VPI and 13.3% demonstrating VPI, compared with 21.8% and 9.1%, respectively, in the nonsyndromic group. Both groups exhibited significantly shallower upper and mid facial depths and wider upper facial breadths when compared with normal standards of facial growth. Although there was a tendency toward decreased facial depths in patients with Stickler syndrome relative to nonsyndromic patients, the differences were nonsignificant. CONCLUSIONS: Patients with Stickler syndrome show significant potential for early airway compromise and a poorer prognosis for speech outcome after cleft palate repair. Their cephalofacial growth does not differ significantly from that of nonsyndromic cleft palate patients.

Decision-Making Involvement and Prediction of Adherence in Youth With Type 1 Diabetes: A Cohort Sequential Study.

Objective: To assess developmental trajectories of decision-making involvement (DMI), defined as the ways in which parents and children engage each other in decision-making about illness management, in youth with type 1 diabetes (T1D) and examine the effects of DMI on levels of and changes in adherence with age. Methods: Participants included 117 youth with T1D, enrolled at ages 8-16 years and assessed five times over 2 years. The cohort sequential design allowed for the approximation of the longitudinal curve from age 8 to 19 from overlapping cohort segments. Children and parents completed the Decision-Making Involvement Scale, which yields subscales for different aspects of DMI, and a self-report adherence questionnaire. Mixed-effects growth curve modeling was used for analysis, with longitudinal measures nested within participant and participants nested within cohort. Results: Most aspects of DMI (Parent Express, Parent Seek, Child Express, and Joint) increased with child age; scores on some child report subscales (Parent Express, Child Seek, and Joint) decreased after age 12-14 years. After accounting for age, Child Seek, Child Express, and Joint were associated with overall higher levels of adherence in both child (estimates = 0.08-0.13, p < .001) and parent (estimates = 0.07- 0.13, p < .01) report models, but they did not predict changes in adherence with age. Conclusion: These data suggest that helping children to be more proactive in T1D discussions, by encouraging them to express their opinions, share information, and solicit guidance from parents, is a potential target for interventions to enhance effective self-management.

Effect of blood T1 estimation strategy on arterial spin labeled cerebral blood flow quantification in children and young adults with kidney disease.

PURPOSE: To compare blood T1 estimation approaches used for quantifying cerebral blood flow (CBF) with arterial spin labeled (ASL) perfusion MRI in a developmental cohort of chronic kidney disease (CKD) patients with anemia and a control group. METHODS: 61 patients with CKD and 47 age-matched control subjects were studied. Blood T1 approaches included: (1) a fixed value, (2) estimation based on measured hematocrit (Hct), and (3) estimation based on Age+Sex using a published formula. Resulting T1 and CBF values were compared along with group, age and sex effects. RESULTS: Highly significant group differences in CBF using fixed blood T1 were reduced when Hct-corrected blood T1 was used, and were eliminated entirely when using the Age+Sex estimated approach. In the control cohort, fixed T1 method showed the strongest correlations of CBF with age and sex. Hct-corrected T1 preserved a significant correlation between CBF and age and sex, while Age+Sex estimated T1 produced a poor fit of CBF with age and sex. CONCLUSIONS: Blood T1 estimation method can confound the interpretation of CBF changes measured using ASL MRI in patients with CKD. Blood T1 should ideally be corrected for hematocrit effects in clinical populations with anemia.

Regional Cerebral Blood Flow in Children and Young Adults with Chronic Kidney Disease.

Purpose To investigate the pathophysiologic effects of chronic kidney disease (CKD) on brain function in children with CKD by correlating cerebral blood flow (CBF) with clinical and behavioral indexes. Materials and Methods In this prospective study, 73 pediatric patients with CKD (mean age, 15.80 years ± 3.63; range, 9-25 years) and 57 control subjects (mean age, 15.65 years ± 3.76; range, 9-25 years) were recruited. CBF measurements were acquired with an MRI arterial spin labeling scheme. Neurocognitive measurements were performed with traditional and computerized neurocognitive batteries. Clinical data were also collected. Group-level global and regional CBF differences between patients with CKD and control subjects were assessed. Regression analyses were conducted to evaluate the associations among regional CBF, clinical variables, and cognitive performance. Results Patients with CKD showed higher global CBF compared with control subjects that was attributable to reduced hematocrit level (mean, 60.2 mL/100 g/min ± 9.0 vs 56.5 mL/100 g/min ± 8.0, respectively). White matter CBF showed correlation with blood pressure (r = 0.244, P = .039), a finding suggestive of altered cerebrovascular autoregulation. Regional CBF differences between patients and control subjects included regions in the "default mode" network. In patients with CKD, positive extrema in the precuneus showed a strong correlation with executive function (ρ = 0.608, P = .001). Conclusion Systemic effects of estimated glomerular filtration rate, hematocrit level, and blood pressure on CBF and alterations in regional CBF may reflect impaired brain function underlying neurocognitive symptoms in CKD. These findings further characterize the nature of alterations in brain physiologic features in children, adolescents, and young adults with CKD.

Brain Magnetic Resonance Imaging Findings in Children and Young Adults With CKD.

BACKGROUND: The neuroanatomic basis for cognitive impairment in chronic kidney disease (CKD) is incompletely characterized. We performed advanced quantitative structural magnetic resonance imaging (MRI) to determine whether CKD affects brain structure and whether poorer neurocognitive performance in CKD is associated with structural brain differences. STUDY DESIGN: Cross-sectional. SETTING & PARTICIPANTS: 85 individuals with CKD stages 2 to 5 and 63 healthy controls, aged 8 to 25 years PREDICTORS: CKD versus control, estimated glomerular filtration rate (eGFR), and kidney transplant status were analyzed as predictors of MRI findings. MRI volumes in 19 prespecified regions of gray matter (GM), white matter (WM), and cerebrospinal fluid were analyzed as predictors of neurocognitive performance (median z scores) in 7 prespecified domains. OUTCOMES: 19 prespecified brain regions of interest (ROIs) in 7 prespecified domains. Neurocognitive performance in 7 prespecified domains. MEASUREMENTS: ROI volumes were compared in CKD versus controls using unadjusted t tests and analysis of covariance (ANCOVA). Associations of ROI volumes with eGFR and kidney transplant status in participants with CKD were analyzed using ANCOVA and linear regression. Associations of neurocognitive performance and ROI volumes were analyzed by linear regression. RESULTS: Participants with CKD had lower whole-brain, cortical, and left parietal GM volumes than controls in unadjusted analyses, but no differences were found in adjusted analysis. In participants with CKD, lower eGFR was associated with higher WM volume in whole-brain (P=0.05) and frontal (P=0.04) ROIs, but differences were not significant after multiple comparisons correction. Kidney transplant recipients had lower GM volumes in whole-brain (P=0.01; Q=0.06), frontal (P=0.02; Q=0.08), and left and right parietal (P=0.01; Q=0.06; and P=0.03; Q=0.1) ROIs and higher whole-brain WM volume (P=0.04; Q=0.1). Neurocognitive performance in the CKD group was not associated with ROI volumes. LIMITATIONS: Unable to assess changes in brain structure and kidney function over time; analysis limited to prespecified ROIs and neurocognitive domains. CONCLUSIONS: CKD in children and young adults may be associated with lower GM and higher WM volumes in some ROIs. Differences were relatively subtle in the CKD group as a whole, but were more prominent in recipients of a kidney transplant. However, neurocognitive performance was not explained by differences in brain ROI volumes, suggesting a functional rather than structural basis for neurocognitive impairment in CKD.

iMOVE: Intensive Mobility training with Variability and Error compared to conventional rehabilitation for young children with cerebral palsy: the protocol for a single blind randomized controlled trial.

BACKGROUND: Cerebral palsy (CP) is the most common cause of physical disability in children. The best opportunity to maximize lifelong independence is early in motor development when there is the most potential for neuroplastic change, but how best to optimize motor ability during this narrow window remains unknown. We have systematically developed and pilot-tested a novel intervention that incorporates overlapping principles of neurorehabilitation and infant motor learning in a context that promotes upright mobility skill and postural control development. The treatment, called iMOVE therapy, was designed to allow young children with CP to self-initiate motor learning experiences similar to their typically developing peers. This manuscript describes the protocol for a subsequent clinical trial to test the efficacy of iMOVE therapy compared to conventional therapy on gross motor development and other secondary outcomes in young children with CP. METHODS: The study is a single-blind randomized controlled trial. Forty-two participants with CP or suspected CP between the ages of 1-3 years will be randomized to receive either the iMOVE or conventional therapy group. Distinguishing characteristics of each group are detailed. Repeated measures of gross motor function will be collected throughout the 12-24 week intervention phase and at three follow-up points over one year post therapy. Secondary outcomes include measures of postural control, physical activity, participation and caregiver satisfaction. DISCUSSION: This clinical trial will add to a small, but growing, body of literature on early interventions to optimize the development of motor control in young children with CP. The information learned will inform clinical practice of early treatment strategies and may contribute to improving the trajectory of motor development and reducing lifelong physical disability in individuals with CP. TRIAL REGISTRATION: ClinicalTrials.gov identifier NCT02340026 . Registered January 16, 2015.

Intramuscular Fentanyl and Ketorolac Associated with Superior Pain Control After Pediatric Bilateral Myringotomy and Tube Placement Surgery: A Retrospective Cohort Study.

BACKGROUND: Bilateral myringotomy and pressure equalization tube insertion (BMT) is the most common surgery in children. Multiple anesthetic techniques for BMT have been proposed, but that which reliably promotes ideal recovery remains unclear. We sought to assess associations between anesthetic regimens that included single-agent (fentanyl or ketorolac) or dual-agent (fentanyl and ketorolac) analgesic therapy and the primary outcome of maximal postanesthesia care unit (PACU) pain score. Secondary outcomes included in-hospital rescue analgesic administration, recovery time, and emesis incidence. METHODS: Principal analysis was conducted on a retrospective cohort of 3669 children aged 6 months to <7 years who underwent BMT over a 16-month period and received intraoperative fentanyl and/or ketorolac. Routine anesthetic care included preoperative oral midazolam, general anesthesia via a mask maintained with sevoflurane and N2O or air in O2, and intramuscular analgesic administration. Multivariable analyses were performed examining relationships between analgesic regimen with the following outcomes: maximum PACU Face, Legs, Activity, Cry, and Consolability (FLACC) score = 0 or 7 to 10, oxycodone administration, and time to discharge readiness. Demographic variables, midazolam exposure, and location (main hospital vs ambulatory surgery center) were included in the multivariable analyses as potential confounders. Associations with postoperative vomiting were studied separately in 2725 children from a subsequent, nonoverlapping 12-month period using similar inclusion criteria. Fentanyl and ketorolac dose-response relationships were evaluated for selected outcome variables. RESULTS: Maximum FLACC = 0, maximum FLACC score of 7 to 10, and oxycodone rescue were most strongly associated with dual-agent therapy versus single-agent ketorolac: odds ratios 4.89 (95% confidence interval [CI], 4.04-5.93), 0.13 (95% CI, 0.10-0.16), and 0.11 (98.3% CI, 0.09-0.14), respectively, P < .001 for each). Minor associations were found for age, Hispanic ethnicity, midazolam, and location, and none for sex or race. For subjects managed with higher dose fentanyl (≥1.5 µg/kg) and ketorolac (≥0.75 mg/kg), 90% had no demonstrable pain, agitation, or distress. Mean discharge readiness times were 21 ± 11 minutes (ketorolac), 26 ± 16 minutes (fentanyl), and 24 ± 14 minutes (dual) (P < .0001). Postoperative emesis incidences associated with ketorolac (2.7%) versus dual therapy (4.5%) were not different (P = .08). CONCLUSIONS: In this large retrospective pediatric BMT study, combination intramuscular fentanyl/ketorolac was strongly associated with superior PACU analgesia and reduced need for oxycodone rescue without clinically significant increases in recovery time or emesis incidence. Combination fentanyl at 1.5 to 2 µg/kg and 1 mg/kg ketorolac was associated with optimal outcomes. Dual therapy appears similarly effective in children of either European Caucasian or African ancestry or of Hispanic ethnicity.

Seizure Detection by Critical Care Providers Using Amplitude-Integrated Electroencephalography and Color Density Spectral Array in Pediatric Cardiac Arrest Patients.

OBJECTIVES: Determine the accuracy and confidence of critical care medicine providers to identify seizures using amplitude-integrated electroencephalography versus amplitude-integrated electroencephalography combined with color density spectral array electroencephalography (aEEG + CDSA). DESIGN: Tutorial and questionnaire. SETTING: PICU. SUBJECTS: Pediatric critical care providers (attendings, fellows, and nurses). INTERVENTIONS: A standardized powerpoint tutorial on amplitude-integrated electroencephalography and color density spectral array followed by classification of 100 amplitude-integrated electroencephalography images and 100 amplitude-integrated electroencephalography combined with color density spectral array as displaying seizures or not displaying seizures. MEASUREMENTS AND MAIN RESULTS: Electroencephalography tracings were obtained from children monitored with continuous electroencephalography after cardiac arrest. The gold standard for seizure identification was continuous electroencephalography interpretation by a pediatric electroencephalographer. The same electroencephalography tracings were used to generate images containing only amplitude-integrated electroencephalography or aEEG + CDSA. Twenty-three critical care medicine providers underwent a 30-minute tutorial on amplitude-integrated electroencephalography and color density spectral array interpretation. They were then asked to determine if there were seizures on 100 amplitude-integrated electroencephalography images and 100 aEEG + CDSA. Amplitude-integrated electroencephalography seizure detection sensitivity was 77% (95% CI, 73%-80%), specificity of 65% (95% CI, 62%-67%), negative predictive value of 88% (95% CI, 86%-90%), and positive predictive value of 46% (95% CI, 43%-49%). For aEEG + CDSA, sensitivity was 77% (95% CI, 74%-81%), specificity of 68% (95% CI, 66%-71%), negative predictive value of 89% (95% CI, 87%-90%), and positive predictive value of 49% (95% CI, 46%-52%). Sensitivity for status epilepticus detection was 77% (95% CI, 71%-82%) with amplitude-integrated electroencephalography and 75% (95% CI, 69%-81%) with aEEG + CDSA. The addition of color density spectral array to amplitude-integrated electroencephalography did not improve seizure detection. However, 87% of critical care medicine providers qualitatively felt that combining both modalities increased their ability to detect seizures. CONCLUSIONS: Amplitude-integrated electroencephalography and aEEG + CDSA offer reasonable sensitivity and negative predictive value for seizure detection by critical care medicine providers. aEEG + CDSA did not improve seizure detection over amplitude-integrated electroencephalography alone although critical care medicine providers felt more confident using both tools combined. Amplitude-integrated electroencephalography and color density spectral array require further evaluation as a tool for screening for seizures and should only be used in conjunction with professional continuous electroencephalography review.

The timing and prevalence of intraoperative hypotension in infants undergoing laparoscopic pyloromyotomy at a tertiary pediatric hospital.

BACKGROUND: Intraoperative hypotension may be associated with adverse outcomes in children undergoing surgery. Infants and neonates under 6 months of age have less autoregulatory cerebral reserve than older infants, yet little information exists regarding when and how often intraoperative hypotension occurs in infants. AIMS: To better understand the epidemiology of intraoperative hypotension in infants, we aimed to determine the prevalence of intraoperative hypotension in a generally uniform population of infants undergoing laparoscopic pyloromyotomy. METHODS: Vital sign data from electronic records of infants who underwent laparoscopic pyloromyotomy with general anesthesia at a children's hospital between January 1, 1998 and October 4, 2013 were analyzed. Baseline blood pressure (BP) values and intraoperative BPs were identified during eight perioperative stages based on anesthesia event timestamps. We determined the occurrence of relative (systolic BP <20% below baseline) and absolute (mean arterial BP <35 mmHg) intraoperative hypotension within each stage. RESULTS: A total of 735 full-term infants and 82 preterm infants met the study criteria. Relative intraoperative hypotension occurred in 77%, 72%, and 58% of infants in the 1-30, 31-60, and 61-90 days age groups, respectively. Absolute intraoperative hypotension was seen in 21%, 12%, and 4% of infants in the 1-30, 31-60, and 61-90 days age groups, respectively. Intraoperative hypotension occurred primarily during surgical prep and throughout the surgical procedure. Preterm infants had higher rates of absolute intraoperative hypotension than full-term infants. CONCLUSIONS: Relative intraoperative hypotension was routine and absolute intraoperative hypotension was common in neonates and infants under 91 days of age. Preterm infants and infants under 61 days of age experienced the highest rates of absolute and relative intraoperative hypotension, particularly during surgical prep and throughout surgery.

A multivariate model exploring the predictive value of demographic, adolescent, and family factors on glycemic control in adolescents with type 1 diabetes.

OBJECTIVE: The current study examined how a comprehensive set of variables from multiple domains, including at the adolescent and family level, were predictive of glycemic control in adolescents with type 1 diabetes (T1D). METHODS: Participants included 100 adolescents with T1D ages 10-16 yrs and their parents. Participants were enrolled in a longitudinal study about youth decision-making involvement in chronic illness management of which the baseline data were available for analysis. Bivariate associations with glycemic control (HbA1C) were tested. Hierarchical linear regression was implemented to inform the predictive model. RESULTS: In bivariate analyses, race, family structure, household income, insulin regimen, adolescent-reported adherence to diabetes self-management, cognitive development, adolescent responsibility for T1D management, and parent behavior during the illness management discussion were associated with HbA1c. In the multivariate model, the only significant predictors of HbA1c were race and insulin regimen, accounting for 17% of the variance. Caucasians had better glycemic control than other racial groups. Participants using pre-mixed insulin therapy and basal-bolus insulin had worse glycemic control than those on insulin pumps. CONCLUSIONS: This study shows that despite associations of adolescent and family-level variables with glycemic control at the bivariate level, only race and insulin regimen are predictive of glycemic control in hierarchical multivariate analyses. This model offers an alternative way to examine the relationship of demographic and psychosocial factors on glycemic control in adolescents with T1D.

Pediatric perioperative adverse events requiring rapid response: a retrospective case-control study.

BACKGROUND: Perioperative pediatric adverse events have been challenging to study within and across institutions due to varying definitions, low event rates, and incomplete capture. AIM: The aim of this study was to determine perioperative adverse event prevalence and to evaluate associated case characteristics and potential contributing factors at an academic pediatric quaternary-care center. METHODS: At the Children's Hospital of Philadelphia (CHOP), perioperative adverse events requiring rapid response assistance are termed Anesthesia Now (AN!) events. They have been accurately captured and entered into a quality improvement database since 2010. Adverse events involving open heart and cardiac catheterization cases are managed separately and not included in this database. We conducted a retrospective case-control study utilizing Compurecord (Phillips Healthcare, Andover, MA, USA), EPIC (EPIC, Verona, WI, USA), and Chartmaxx (MedPlus, Mason, OH, USA) systems matching AN! event cases to noncardiac controls (1 : 2) based on surgical date. RESULTS: From April 16, 2010 to September 25, 2012, we documented 213 AN! events in the noncardiac perioperative complex and remote sites at our main hospital. AN! prevalence was 0.0043 (1 : 234) with a 95% confidence interval (CI) (0.0037, 0.0049). Respiratory events, primarily laryngospasm, were most common followed by events of cardiovascular etiology. Median age was lower in the AN! group than in controls, 2.86 years (interquartile range 0.94, 10.1) vs 6.20 (2.85, 13.1), P < 0.0001. Odds ratios (with 95% CI) for age, 0.969 (0.941, 0.997); American Society of Anesthesiologists physical status, 1.67 (1.32, 2.12); multiple (≥2) services, 2.27 (1.13, 4.55); nonoperating room vs operating room location, 0.240 (0.133, 0.431); and attending anesthesiologist's experience, 0.976 (0.959, 0.992) were all significant. CONCLUSIONS: Decreased age, increased comorbidities, multiple (vs single) surgical services, operating room (vs nonoperating room) location, and decreased staff experience were associated with increased risk of AN! events, which were predominantly respiratory in origin.

Detection of electrographic seizures by critical care providers using color density spectral array after cardiac arrest is feasible.

OBJECTIVE: To determine the accuracy and reliability of electroencephalographic seizure detection by critical care providers using color density spectral array electroencephalography. DESIGN: Tutorial and questionnaire. SUBJECTS: Critical care providers (attending physicians, fellow trainees, and nurses). INTERVENTIONS: A standardized powerpoint color density spectral array tutorial followed by classification of 200 color density spectral array images as displaying seizures or not displaying seizures. MEASUREMENTS AND MAIN RESULTS: Using conventional electroencephalography recordings obtained from patients who underwent electroencephalography monitoring after cardiac arrest, we created 100 color density spectral array images, 30% of which displayed seizures. The gold standard for seizure category was electroencephalographer determination from the full montage conventional electroencephalography. Participants did not have access to the conventional electroencephalography tracings. After completing a standardized color density spectral array tutorial, images were presented to participants in duplicate and in random order. Twenty critical care physicians (12 attendings and eight fellows) and 19 critical care nurses classified the color density spectral array images as having any seizure(s) or no seizures. The 39 critical care providers had a color density spectral array seizure detection sensitivity of 70% (95% CI, 67-73%), specificity of 68% (95% CI, 67-70%), positive predictive value of 46%, and negative predictive value of 86%. The sensitivity of color density spectral array detection of status epilepticus was 72% (95% CI, 69-74%). CONCLUSION: Determining which post-cardiac arrest patients experience electrographic seizures by critical care providers is feasible after a brief training. There is moderate sensitivity for seizure and status epilepticus detection and a high negative predictive value.

Design and methods of the NiCK study: neurocognitive assessment and magnetic resonance imaging analysis of children and young adults with chronic kidney disease.

BACKGROUND: Chronic kidney disease is strongly linked to neurocognitive deficits in adults and children, but the pathophysiologic processes leading to these deficits remain poorly understood. The NiCK study (Neurocognitive Assessment and Magnetic Resonance Imaging Analysis of Children and Young Adults with Chronic Kidney Disease) seeks to address critical gaps in our understanding of the biological basis for neurologic abnormalities in chronic kidney disease. In this report, we describe the objectives, design, and methods of the NiCK study. DESIGN/METHODS: The NiCK Study is a cross-sectional cohort study in which neurocognitive and neuroimaging phenotyping is performed in children and young adults, aged 8 to 25 years, with chronic kidney disease compared to healthy controls. Assessments include (1) comprehensive neurocognitive testing (using traditional and computerized methods); (2) detailed clinical phenotyping; and (3) multimodal magnetic resonance imaging (MRI) to assess brain structure (using T1-weighted MRI, T2-weighted MRI, and diffusion tensor imaging), functional connectivity (using functional MRI), and blood flow (using arterial spin labeled MRI). Primary analyses will examine group differences in neurocognitive testing and neuroimaging between subjects with chronic kidney disease and healthy controls. Mechanisms responsible for neurocognitive dysfunction resulting from kidney disease will be explored by examining associations between neurocognitive testing and regional changes in brain structure, functional connectivity, or blood flow. In addition, the neurologic impact of kidney disease comorbidities such as anemia and hypertension will be explored. We highlight aspects of our analytical approach that illustrate the challenges and opportunities posed by data of this scope. DISCUSSION: The NiCK study provides a unique opportunity to address key questions about the biological basis of neurocognitive deficits in chronic kidney disease. Understanding these mechanisms could have great public health impact by guiding screening strategies, delivery of health information, and targeted treatment strategies for chronic kidney disease and its related comorbidities.

Parent attendance and homework adherence predict response to a family-school intervention for children with ADHD.

This study examined the relative contribution of two dimensions of parent engagement, attendance and homework adherence, to parent and child treatment response and explored whether early engagement was a stronger predictor of outcomes than later engagement. The sample consisted of parents of participants (n = 92; M age = 9.4 years, SD = 1.27; 67% male, 69% White) in a 12-session evidence-based family-school intervention for children with attention-deficit/hyperactivity disorder. Attendance was assessed using clinician records, and homework adherence was measured by rating permanent products. Outcomes included parent and teacher ratings of family involvement in education, parenting practices, and child functioning. Accounting for the contributions of baseline scores and attendance, homework adherence was a significant predictor of parental self-efficacy, the parent-teacher relationship, parenting through positive involvement, and the child's inattention to homework and homework productivity. Accounting for the contribution of baseline scores and homework adherence, attendance was a significant predictor of one outcome, the child's academic productivity. Early homework adherence appeared to be more predictive of outcomes than later adherence, whereas attendance did not predict outcomes during either half of treatment. These results indicate that, even in the context of evidence-based practice, it is the extent to which parents actively engage with treatment, rather than the number of sessions they attend, that is most important in predicting intervention response. Because attendance is limited as an index of engagement and a predictor of outcomes, increased efforts to develop interventions to promote parent adherence to behavioral interventions for children are warranted.

A randomized multi-institutional crossover comparison of the GlideScope® Cobalt Video laryngoscope to the flexible fiberoptic bronchoscope in a Pierre Robin manikin.

BACKGROUND: The GlideScope Cobalt Video laryngoscope is being used more often in children with challenging laryngoscopy. There are, however, no pediatric trials comparing it to flexible fiberoptic bronchoscopy, the current accepted gold standard. This preliminary manikin study compares the first-attempt intubation success of the GlideScope Cobalt video laryngoscope to the flexible fiberoptic bronchoscope when performed by attending pediatric anesthesiologists at two major pediatric centers. METHODS: This prospective randomized, crossover study evaluated 120 attempts (60 with each study device) to intubate the AirSim Pierre Robin manikin (PRM) with fiberoptic bronchoscopy and video laryngoscopy (VL). Attending pediatric anesthesiologists from two quaternary pediatric centers were eligible to participate. Each attending anesthesiologist randomly performed a single tracheal intubation attempt with one of the study devices followed by the alternate method. The primary outcome was the first-attempt success rate of tracheal intubation. Blinding was not feasible. We hypothesized that first-attempt success would be higher with fiberoptic bronchoscopy. RESULTS: Thirty anesthesiologists from each center were randomized to use one of the study devices followed by the alternate method. We analyzed all participants' data. There was no overall difference in first-attempt success between VL and fiberoptic bronchoscopy (88.3% vs 85% respectively, P = 0.59). There were significant institutional differences in first-attempt success using VL (76.7% vs 100%). CONCLUSIONS: There was no difference in first-attempt success of tracheal intubation using VL vs fiberoptic bronchoscopy when performed by attending anesthesiologists at two large pediatric centers. However, institutional differences exist in success rates with VL across the two centers. Results from single-center device evaluations should be verified by multi-center evaluations. A significant proportion of attending anesthesiologists lack experience with advanced airway devices; targeted education may enhance intubation success and patient safety.

Relationship of youth involvement in diabetes-related decisions to treatment adherence.

The aim of this study was to examine the relationship of youth's involvement in diabetes-related decisions to adherence. Children and adolescents (8-19 years) and their parents (N = 89) completed the Decision Making Involvement Scale and the Self Care Inventory, a self-report measure of adherence. After controlling for youth age, the degree to which youth expressed an opinion and information to parents was associated with better parent- and youth-reported adherence. The degree to which parents expressed an opinion and information to youth was associated with worse parent-reported adherence. Joint decision-making behaviors (e.g., negotiation; provision of options) also were associated with better youth-reported adherence. Encouraging youth to express opinions and share illness-related information with parents during illness management discussions may improve adherence. Additional research is needed to identify mechanisms of effect and determine associations between decision making involvement and health behaviors and outcomes over time.

Characterization of Fine Motor and Visual Motor Skills in Aicardi-Goutières Syndrome.

Aicardi-Goutières syndrome is a genetic inflammatory disorder resulting in dispersed neurologic dysfunction. Despite a recognition of overall motor impairment, fine and visual motor skills are undercharacterized. We hypothesize that there is a spectrum of fine and visual motor skills in the Aicardi-Goutières syndrome population as captured by a standard outcome measure, the Peabody Developmental Motor Scales (PDMS-2), which will be proportional to overall disease severity.In a cohort of 74 subjects, the Peabody Developmental Motor Scales-2 grasping and visual-motor integration subtests were administered concurrently with the Aicardi-Goutières syndrome Severity Scale (severe [range 0-3], moderate [range 4-8], and attenuated [range 9-11]). The cohort was also compared by genotype and performance as defined by raw scores. The distribution of Peabody Developmental Motor Scales-2 scores within a genotype was assessed by interquartile ranges (IQRs).Peabody Developmental Motor Scales-2 grasping and visual-motor integration performance was the least variable in the TREX1-cohort (IQR: 10.00-12.00) versus the SAMHD1 and IFIH1 cohorts (IQR: 51.00-132.00 and 48.50-134.00, respectively). Neurologic severity highly correlated with both fine and visual motor skills (Spearman correlation: r = 0.87, 0.91, respectively). A floor effect (lowest 10% of possible scores) was observed within the severe cohort (n = 32/35), whereas a ceiling effect (top 10%) was observed in the attenuated cohort (n = 13/17).This study characterized the spectrum of fine and visual motor function in the Aicardi-Goutières syndrome population, which correlated with overall neurologic dysfunction. The Peabody Developmental Motor Scales-2 grasping and visual-motor integration showed promise as potential assessment tools in moderate and attenuated Aicardi-Goutières syndrome cohorts. A better understanding of fine and visual motor function in this population will benefit clinical care and clinical trial design.