Showing papers by "Montreal Children's Hospital published in 2017"
University of Toronto1, German Cancer Research Center2, University of Düsseldorf3, University of Pittsburgh4, Ontario Institute for Cancer Research5, Seoul National University6, University of Warsaw7, University of Lyon8, Mayo Clinic9, The Chinese University of Hong Kong10, Johns Hopkins University11, University of Alabama at Birmingham12, University of Washington13, Fred Hutchinson Cancer Research Center14, University of California, San Francisco15, Hamilton Health Sciences16, McMaster University17, Vanderbilt University18, University of Colorado Denver19, Semmelweis University20, Erasmus University Rotterdam21, University of Ulsan22, Kitasato University23, Mexican Social Security Institute24, Masaryk University25, Emory University26, University of Debrecen27, University of Naples Federico II28, Washington University in St. Louis29, McGill University30, Montreal Children's Hospital31, Virginia Commonwealth University32, Chonnam National University33, University of Queensland34, University of Calgary35, University of São Paulo36, University of Cincinnati37, University of Arkansas for Medical Sciences38, The Catholic University of America39, University of California, Los Angeles40, University of Sydney41, Kumamoto University42, Saint Louis University43, Case Western Reserve University44
TL;DR: Similarity network fusion (SNF) applied to genome-wide DNA methylation and gene expression data across 763 primary samples identifies very homogeneous clusters of patients, supporting the presence of medulloblastoma subtypes.
737 citations
Children's Hospital of Eastern Ontario1, University of Ottawa2, University of British Columbia3, McGill University4, Queen's University5, University of Strathclyde6, Public Health Agency of Canada7, University of Alberta8, Montreal Children's Hospital9, University of Wollongong10, McMaster University11
TL;DR: These guidelines represent a sensible evolution of public health guidelines whereby optimal health is framed within the balance of movement behaviours across the whole day, while respecting preferences of end-users.
Abstract: The Canadian Society for Exercise Physiology convened representatives of national organizations, research experts, methodologists, stakeholders, and end-users who followed rigorous and transparent guideline development procedures to create the Canadian 24-Hour Movement Guidelines for the Early Years (0–4 years): An Integration of Physical Activity, Sedentary Behaviour, and Sleep. These novel guidelines for children of the early years embrace the natural and intuitive integration of movement behaviours across the whole day (24-h period). The development process was guided by the Appraisal of Guidelines for Research and Evaluation (AGREE) II instrument. Four systematic reviews (physical activity, sedentary behaviour, sleep, combined behaviours) examining the relationships within and among movement behaviours and several health indicators were completed and interpreted by a Guideline Development Panel. The systematic reviews that were conducted to inform the development of the guidelines, and the framework that was applied to develop the recommendations, followed the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology. Complementary compositional analyses were performed using data from the Canadian Health Measures Survey to examine the relationships between movement behaviours and indicators of adiposity. A review of the evidence on the cost effectiveness and resource use associated with the implementation of the proposed guidelines was also undertaken. A stakeholder survey (n = 546), 10 key informant interviews, and 14 focus groups (n = 92 participants) were completed to gather feedback on draft guidelines and their dissemination. The guidelines provide evidence-informed recommendations as to the combinations of light-, moderate- and vigorous-intensity physical activity, sedentary behaviours, and sleep that infants (<1 year), toddlers (1–2 years) and preschoolers (3–4 years) should achieve for a healthy day (24 h). Proactive dissemination, promotion, implementation, and evaluation plans were prepared to optimize uptake and activation of the new guidelines. These guidelines represent a sensible evolution of public health guidelines whereby optimal health is framed within the balance of movement behaviours across the whole day, while respecting preferences of end-users. Future research should consider the integrated relationships among movement behaviours, and similar integrated guidelines for other age groups should be developed.
419 citations
Baylor College of Medicine1, Boston Children's Hospital2, University of California, San Francisco3, Ain Shams University4, University of Arkansas at Little Rock5, Montreal Children's Hospital6, Indiana University7, Wayne State University8, University of California, Los Angeles9, Cedars-Sinai Medical Center10, University of South Florida11
TL;DR: This work provides an update of the evidence‐based guideline on immunoglobulin therapy, last published in 2006, and suggests that careful consideration of its indications and administration is warranted.
Abstract: Human immunoglobulin preparations for intravenous or subcutaneous administration are the cornerstone of treatment in patients with primary immunodeficiency diseases affecting the humoral immune system. Intravenous preparations have a number of important uses in the treatment of other diseases in humans as well, some for which acceptable treatment alternatives do not exist. We provide an update of the evidence-based guideline on immunoglobulin therapy, last published in 2006. Given the potential risks and inherent scarcity of human immunoglobulin, careful consideration of its indications and administration is warranted.
408 citations
University of Calgary1, McGill University Health Centre2, Libin Cardiovascular Institute of Alberta3, University of British Columbia4, Montreal Children's Hospital5, Université du Québec à Trois-Rivières6, Université de Montréal7, Laval University8, McMaster University9, University of Alberta10, University of Toronto11, Ottawa Hospital Research Institute12, Hôpital Maisonneuve-Rosemont13, University of Western Ontario14, Memorial University of Newfoundland15, Centre for Addiction and Mental Health16, University of Ottawa17, McGill University18, University Health Network19, University of Saskatchewan20, University of Manitoba21, Concordia University Wisconsin22, St. Michael's Hospital23, Montreal General Hospital24, Heart and Stroke Foundation of Canada25, Dalhousie University26, Université de Sherbrooke27, Université du Québec à Montréal28, Montreal Heart Institute29, Population Health Research Institute30, Simon Fraser University31, St George's, University of London32, Centre Hospitalier Universitaire Sainte-Justine33, Children's Hospital of Eastern Ontario34
TL;DR: Hypertension Canada provides annually updated, evidence-based guidelines for the diagnosis, assessment, prevention, and treatment of hypertension, including 10 new guidelines for individuals with non-AOBP readings ≥ 140 mm Hg.
256 citations
University of California, Irvine1, Ohio State University2, University of Pittsburgh3, Florida International University4, Montreal Children's Hospital5, University of California, Berkeley6, New York University7, Duke University8, University of Vermont9, Carleton University10, Columbia University11, Icahn School of Medicine at Mount Sinai12, University of Arkansas for Medical Sciences13, National Institutes of Health14, Cincinnati Children's Hospital Medical Center15, Pennsylvania State University16, University of North Carolina at Chapel Hill17, Del Rosario University18, Stanford University19
TL;DR: In the MTA follow-up into adulthood, the ADHD group showed symptom persistence compared to local norms from the LNCG, and within naturalistic subgroups of ADHD cases, extended use of medication was associated with suppression of adult height but not with reduction of symptom severity.
Abstract: Background
The Multimodal Treatment Study (MTA) began as a 14-month randomized clinical trial of behavioral and pharmacological treatments of 579 children (7–10 years of age) diagnosed with attention-deficit/hyperactivity disorder (ADHD)-combined type. It transitioned into an observational long-term follow-up of 515 cases consented for continuation and 289 classmates (258 without ADHD) added as a local normative comparison group (LNCG), with assessments 2–16 years after baseline.
Methods
Primary (symptom severity) and secondary (adult height) outcomes in adulthood were specified. Treatment was monitored to age 18, and naturalistic subgroups were formed based on three patterns of long-term use of stimulant medication (Consistent, Inconsistent, and Negligible). For the follow-up, hypothesis-generating analyses were performed on outcomes in early adulthood (at 25 years of age). Planned comparisons were used to estimate ADHD-LNCG differences reflecting persistence of symptoms and naturalistic subgroup differences reflecting benefit (symptom reduction) and cost (height suppression) associated with extended use of medication.
Results
For ratings of symptom severity, the ADHD-LNCG comparison was statistically significant for the parent/self-report average (0.51 ± 0.04, p < .0001, d = 1.11), documenting symptom persistence, and for the parent/self-report difference (0.21 ± 0.04, p < .0001, d = .60), documenting source discrepancy, but the comparisons of naturalistic subgroups reflecting medication effects were not significant. For adult height, the ADHD group was 1.29 ± 0.55 cm shorter than the LNCG (p < .01, d = .21), and the comparisons of the naturalistic subgroups were significant: the treated group with the Consistent or Inconsistent pattern was 2.55 ± 0.73 cm shorter than the subgroup with the Negligible pattern (p < .0005, d = .42), and within the treated group, the subgroup with the Consistent pattern was 2.36 ± 1.13 cm shorter than the subgroup with the Inconsistent pattern (p < .04, d = .38).
Conclusions
In the MTA follow-up into adulthood, the ADHD group showed symptom persistence compared to local norms from the LNCG. Within naturalistic subgroups of ADHD cases, extended use of medication was associated with suppression of adult height but not with reduction of symptom severity.
204 citations
TL;DR: The interview format optimizes young adult self-reporting when parent reports are not available, and the combination of parent and self-reports from rating scales, using an 'or' rule and a NB threshold optimized the balance between sensitivity and specificity.
Abstract: Objective
Longitudinal studies of children diagnosed with ADHD report widely ranging ADHD persistence rates in adulthood (5–75%). This study documents how information source (parent vs. self-report), method (rating scale vs. interview), and symptom threshold (DSM vs. norm-based) influence reported ADHD persistence rates in adulthood.
Method
Five hundred seventy-nine children were diagnosed with DSM-IV ADHD-Combined Type at baseline (ages 7.0–9.9 years) 289 classmates served as a local normative comparison group (LNCG), 476 and 241 of whom respectively were evaluated in adulthood (Mean Age = 24.7). Parent and self-reports of symptoms and impairment on rating scales and structured interviews were used to investigate ADHD persistence in adulthood.
Results
Persistence rates were higher when using parent rather than self-reports, structured interviews rather than rating scales (for self-report but not parent report), and a norm-based (NB) threshold of 4 symptoms rather than DSM criteria. Receiver-Operating Characteristics (ROC) analyses revealed that sensitivity and specificity were optimized by combining parent and self-reports on a rating scale and applying a NB threshold.
Conclusion
The interview format optimizes young adult self-reporting when parent reports are not available. However, the combination of parent and self-reports from rating scales, using an ‘or’ rule and a NB threshold optimized the balance between sensitivity and specificity. With this definition, 60% of the ADHD group demonstrated symptom persistence and 41% met both symptom and impairment criteria in adulthood.
155 citations
TL;DR: Psychiatric assessments administered longitudinally to the local normative comparison group of the Multimodal Treatment Study of ADHD found no evidence for adult-onset ADHD independent of a complex psychiatric history.
Abstract: Objective:Adolescents and young adults without childhood attention deficit hyperactivity disorder (ADHD) often present to clinics seeking stimulant medication for late-onset ADHD symptoms. Recent birth-cohort studies support the notion of late-onset ADHD, but these investigations are limited by relying on screening instruments to assess ADHD, not considering alternative causes of symptoms, or failing to obtain complete psychiatric histories. The authors address these limitations by examining psychiatric assessments administered longitudinally to the local normative comparison group of the Multimodal Treatment Study of ADHD.Method:Individuals without childhood ADHD (N=239) were administered eight assessments from comparison baseline (mean age=9.89 years) to young adulthood (mean age=24.40 years). Diagnostic procedures utilized parent, teacher, and self-reports of ADHD symptoms, impairment, substance use, and other mental disorders, with consideration of symptom context and timing.Results:Approximately 95% ...
110 citations
TL;DR: E coli and GBS remain the most common causes of bacterial meningitis in the first 90 days of life, and one should consider a third-generation cephalosporin (plus ampicillin for at least the first month) for empirical therapy of suspected Meningitis if there is evidence for Gram-negativeMeningitis.
Abstract: OBJECTIVES: The pathogens that cause bacterial meningitis in infants and their antimicrobial susceptibilities may have changed in this era of increasing antimicrobial resistance, use of conjugated vaccines, and maternal antibiotic prophylaxis for group B Streptococcus (GBS). The objective was to determine the optimal empirical antibiotics for bacterial meningitis in early infancy. METHODS: This was a cohort study of infants RESULTS: There were 113 patients diagnosed with proven meningitis (n = 63) or suspected meningitis (n = 50) presented at median 19 days of age, with 63 patients (56%) presenting a diagnosis from home. Predominant pathogens were Escherichia coli (n = 37; 33%) and GBS (n = 35; 31%). Two of 15 patients presenting meningitis on day 0 to 6 had isolates resistant to both ampicillin and gentamicin (E coli and Haemophilus influenzae type B). Six of 60 infants presenting a diagnosis of meningitis from home from day 7 to 90 had isolates, for which cefotaxime would be a poor choice (Listeria monocytogenes [n = 3], Enterobacter cloacae, Cronobacter sakazakii, and Pseudomonas stutzeri). Sequelae were documented in 84 infants (74%), including 8 deaths (7%). CONCLUSIONS: E coli and GBS remain the most common causes of bacterial meningitis in the first 90 days of life. For empirical therapy of suspected bacterial meningitis, one should consider a third-generation cephalosporin (plus ampicillin for at least the first month), potentially substituting a carbapenem for the cephalosporin if there is evidence for Gram-negative meningitis.
104 citations
TL;DR: The incidence of adverse sedation outcomes varied significantly with type of sedation medication, and use of ketamine only was associated with the best outcomes, resulting in significantly fewer SAEs and interventions than ketamine combined with propofol or fentanyl.
Abstract: Importance Procedural sedation for children undergoing painful procedures is standard practice in emergency departments worldwide. Previous studies of emergency department sedation are limited by their single-center design and are underpowered to identify risk factors for serious adverse events (SAEs), thereby limiting their influence on sedation practice and patient outcomes. Objective To examine the incidence and risk factors associated with sedation-related SAEs. Design, Setting, and Participants This prospective, multicenter, observational cohort study was conducted in 6 pediatric emergency departments in Canada between July 10, 2010, and February 28, 2015. Children 18 years or younger who received sedation for a painful emergency department procedure were enrolled in the study. Of the 9657 patients eligible for inclusion, 6760 (70.0%) were enrolled and 6295 (65.1%) were included in the final analysis. Exposures The primary risk factor was receipt of sedation medication. The secondary risk factors were demographic characteristics, preprocedural medications and fasting status, current or underlying health risks, and procedure type. Main Outcomes and Measures Four outcomes were examined: SAEs, significant interventions performed in response to an adverse event, oxygen desaturation, and vomiting. Results Of the 6295 children included in this study, 4190 (66.6%) were male and the mean (SD) age was 8.0 (4.6) years. Adverse events occurred in 736 patients (11.7%; 95% CI, 6.4%-16.9%). Oxygen desaturation (353 patients [5.6%]) and vomiting (328 [5.2%]) were the most common of these adverse events. There were 69 SAEs (1.1%; 95% CI, 0.5%-1.7%), and 86 patients (1.4%; 95% CI, 0.7%-2.1%) had a significant intervention. Use of ketamine hydrochloride alone resulted in the lowest incidence of SAEs (17 [0.4%]) and significant interventions (37 [0.9%]). The incidence of adverse sedation outcomes varied significantly with the type of sedation medication. Compared with ketamine alone, propofol alone (3.7%; odds ratio [OR], 5.6; 95% CI, 2.3-13.1) and the combinations of ketamine and fentanyl citrate (3.2%; OR, 6.5; 95% CI, 2.5-15.2) and ketamine and propofol (2.1%; OR, 4.4; 95% CI, 2.3-8.7) had the highest incidence of SAEs. The combinations of ketamine and fentanyl (4.1%; OR, 4.0; 95% CI, 1.8-8.1) and ketamine and propofol (2.5%; OR, 2.2; 95% CI, 1.2-3.8) had the highest incidence of significant interventions. Conclusions and Relevance The incidence of adverse sedation outcomes varied significantly with type of sedation medication. Use of ketamine only was associated with the best outcomes, resulting in significantly fewer SAEs and interventions than ketamine combined with propofol or fentanyl.
82 citations
Johns Hopkins University1, Harvard University2, Duke University3, University of Louisville4, University of Michigan5, Children's Hospitals and Clinics of Minnesota6, University of Missouri–Kansas City7, Boston Children's Hospital8, Alfred I. duPont Hospital for Children9, Society of Thoracic Surgeons10, Montreal Children's Hospital11, Northwestern University12, Baylor College of Medicine13, Cincinnati Children's Hospital Medical Center14, University College London15, Wayne State University16, Children's National Medical Center17
TL;DR: This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency.
74 citations
University of Melbourne1, Royal Children's Hospital2, Pierre-and-Marie-Curie University3, University of Queensland4, University of Strasbourg5, Monash University6, University of Paris7, ICM Partners8, Walter and Eliza Hall Institute of Medical Research9, Florey Institute of Neuroscience and Mental Health10, University of Rennes11, Monash University, Clayton campus12, McGill University13, Montreal Children's Hospital14, Aston University15, Montreal Neurological Institute and Hospital16, Boston Children's Hospital17
TL;DR: This work identified DCC mutations in four families and five sporadic individuals with isolated agenesis of the corpus callosum without intellectual disability that result in variable dominant phenotypes with decreased penetrance, including mirror movements and ACC associated with a favorable developmental prognosis.
Abstract: Brain malformations involving the corpus callosum are common in children with developmental disabilities. We identified DCC mutations in four families and five sporadic individuals with isolated agenesis of the corpus callosum (ACC) without intellectual disability. DCC mutations result in variable dominant phenotypes with decreased penetrance, including mirror movements and ACC associated with a favorable developmental prognosis. Possible phenotypic modifiers include the type and location of mutation and the sex of the individual.
TL;DR: The Optimal Resources document has been produced by surgical providers from LMICs who have the greatest insight into the needs and priorities in their population and will be refined further through online GICS Working Groups and the World Health Organization for broad application to ensure all children have timely access to safe surgical care.
Abstract: Background The Lancet Commission on Global Surgery reported that 5 billion people lack access to safe, affordable surgical care. The majority of these people live in low-resource settings, where up to 50% of the population is children. The Disease Control Priorities (Debas HTP, Donkor A, Gawande DT, Jamison ME, Kruk, and Mock CN, editors. Essential Surgery. Disease Control Priorities. Third Edition, vol 1. Essential Surgery. Washington, DC: World Bank; 2015) on surgery included guidelines for the improvement of access to surgical care; however, these lack detail for children's surgery. Aim To produce guidance for low- and middle-income countries (LMICs) on the resources required for children's surgery at each level of hospital care. Methods The Global Initiative for Children's Surgery (GICS) held an inaugural meeting at the Royal College of Surgeons in London in May 2016, with 52 surgical providers from 21 countries, including 27 providers from 18 LMICs. Delegates engaged in working groups over 2 days to prioritize needs and solutions for optimizing children's surgical care; these were categorized into infrastructure, service delivery, training, and research. At a second GICS meeting in Washington in October 2016, 94 surgical care providers, half from LMICs, defined the optimal resources required at primary, secondary, tertiary, and national referral level through a series of working group engagements. Results Consensus solutions for optimizing children's surgical care included the following: An “Optimal Resources” document was produced detailing the facilities and resources required at each level of care. Conclusion The Optimal Resources document has been produced by surgical providers from LMICs who have the greatest insight into the needs and priorities in their population. The document will be refined further through online GICS Working Groups and the World Health Organization for broad application to ensure all children have timely access to safe surgical care.
University of Louisville1, Medical College of Wisconsin2, University of Tennessee Health Science Center3, Albany Medical College4, Children's Memorial Hospital5, Cincinnati Children's Hospital Medical Center6, Montreal Children's Hospital7, University of Texas MD Anderson Cancer Center8, University of Michigan9, University of Washington10, University of Iowa11, University of Florida12
TL;DR: This review addresses six clinically applicable questions using available evidence to provide recommendations for the treatment of infants with a congenital pulmonary airway malformation (CPAM) and concludes very few articles provided definitive recommendations for care of the patient with a CPAM.
Abstract: Variation in management characterizes treatment of infants with a congenital pulmonary airway malformation (CPAM). This review addresses six clinically applicable questions using available evidence to provide recommendations for the treatment of these patients. Questions regarding the management of a pediatric patient with a CPAM were generated. English language articles published between 1960 and 2014 were compiled after searching Medline and OvidSP. The articles were divided by subject area and by the question asked, then reviewed and included if they specifically addressed the proposed question. 1040 articles were identified on initial search. After screening abstracts per eligibility criteria, 130 articles were used to answer the proposed questions. Based on the available literature, resection of an asymptomatic CPAM is controversial, and when performed is usually completed within the first six months of life. Lobectomy remains the standard resection method for CPAM, and can be performed thoracoscopically or via thoracotomy. There is no consensus regarding a monitoring protocol for observing asymptomatic lesions, although at least one chest computerized tomogram (CT) should be performed postnatally for lesion characterization. An antenatally identified CPAM can be evaluated with MRI if fetal intervention is being considered, but is not required for the fetus with a lesion not at risk for hydrops. Prenatal consultation should be offered for infants with CPAM and encouraged for those infants in whom characteristics indicate risk of hydrops. Very few articles provided definitive recommendations for care of the patient with a CPAM and none reported Level I or II evidence. Based on available information, CPAMs are usually resected early in life if at all. A prenatally diagnosed congenital lung lesion should be evaluated postnatally with CT, and prenatal counseling should be undertaken in patients at risk for hydrops.
Cincinnati Children's Hospital Medical Center1, Albany Medical College2, Seattle Children's3, Montreal Children's Hospital4, Boston Children's Hospital5, Nationwide Children's Hospital6, University of Tennessee Health Science Center7, Children's Memorial Hospital8, University of California, Irvine9, University of Texas Health Science Center at Houston10, Medical College of Wisconsin11, Children's Mercy Hospital12, Naval Medical Center Portsmouth13, University of Colorado Boulder14, University of Louisville15
TL;DR: There appears to be overwhelming evidence supporting ovarian detorsion rather than oopherectomy for the management of ovarian torsion in pediatric patients and warnings of thromboembolism and malignancy are overstated.
Cincinnati Children's Hospital Medical Center1, Beth Israel Deaconess Medical Center2, Hebrew University of Jerusalem3, Harvard University4, University of Texas Southwestern Medical Center5, Baylor College of Medicine6, Nationwide Children's Hospital7, Seattle Children's8, University of California, San Francisco9, Boston Children's Hospital10, Children's Hospital Los Angeles11, Children's Hospital of Philadelphia12, Montreal Children's Hospital13, University of New South Wales14, University of Utah15, University of Minnesota16, Medical College of Wisconsin17, Roy J. and Lucille A. Carver College of Medicine18
TL;DR: Pediatric AIP has a distinct presentation with features similar to type 2 AIP in adults, and is expected to help with the diagnosis and management of this disease and pave the way for future research studies.
TL;DR: The literature generally supports the intuitive expectations that compassionate and respectful care for the deceased and family, listening for and addressing family concerns, and an attitude to donation that is positive is best for both consent rates and family well-being.
Abstract: BackgroundFamilies are often asked to consent to the donation of their deceased relative’s organs or tissues. These end-of-life conversations are important because they affect consent rates as well as the psychological impact of the decision for families. This scoping review of the literature on req
TL;DR: The CC deficit identified here in newborns may support early identification of a range of midline structural impairments in fetal alcohol syndrome and provide an early indicator of fetal alcohol-related cognitive deficits that have been linked to this critically important brain structure in childhood and adolescence.
Abstract: BACKGROUND Magnetic resonance imaging (MRI) studies have consistently demonstrated disproportionately smaller corpus callosa in individuals with a history of prenatal alcohol exposure (PAE) but have not previously examined the feasibility of detecting this effect in infants. Tissue segmentation of the newborn brain is challenging because analysis techniques developed for the adult brain are not directly transferable, and segmentation for cerebral morphometry is difficult in neonates, due to the latter's incomplete myelination. This study is the first to use volumetric structural MRI to investigate PAE effects in newborns using manual tracing and to examine the cross-sectional area of the corpus callosum (CC). METHODS Forty-three nonsedated infants born to 32 Cape Coloured heavy drinkers and 11 controls recruited prospectively during pregnancy were scanned using a custom-designed birdcage coil for infants, which increases signal-to-noise ratio almost 2-fold compared to the standard head coil. Alcohol use was ascertained prospectively during pregnancy, and fetal alcohol spectrum disorders diagnosis was conducted by expert dysmorphologists. Data were acquired using a multi-echo FLASH protocol adapted for newborns, and a knowledge-based procedure was used to hand-segment the neonatal brains. RESULTS CC was disproportionately smaller in alcohol-exposed neonates than controls after controlling for intracranial volume. By contrast, CC area was unrelated to infant sex, gestational age, age at scan, or maternal smoking, marijuana, or methamphetamine use during pregnancy. CONCLUSIONS Given that midline craniofacial anomalies have been recognized as a hallmark of fetal alcohol syndrome in humans and animal models since this syndrome was first identified, the CC deficit identified here in newborns may support early identification of a range of midline structural impairments. Smaller CC during the newborn period may provide an early indicator of fetal alcohol-related cognitive deficits that have been linked to this critically important brain structure in childhood and adolescence.
Seattle Children's1, Boston Children's Hospital2, Medical College of Wisconsin3, University of Iowa4, Hebrew University of Jerusalem5, University of Texas Southwestern Medical Center6, University of Minnesota7, University of Utah8, Cincinnati Children's Hospital Medical Center9, University of New South Wales10, Montreal Children's Hospital11, Baylor College of Medicine12, University of California, San Francisco13, Nationwide Children's Hospital14, Harvard University15
TL;DR: Early‐onset pancreatitis is associated strongly with PRSS1 or CTRC mutations and family history of pancreatitis, and children with later‐ONSet disease are more likely to have nongenetic risk factors.
TL;DR: In light of the infrequent occurrence of LGEA and the technically demanding techniques involved to achieve esophageal continuity, it is strongly advised to develop regional or national centers of expertise for the management and follow-up of these very complex patients.
Abstract: INoEA is the International Network of Esophageal Atresia and consists of a broad spectrum of pediatric specialties and patient societies. The working group on long gap esophageal atresia set out to develop guidelines regarding the definition of long gap esophageal atresia, the best diagnostic and treatment strategies, and highlight the necessity of experience and communication in the management of these challenging patients. Review of the literature and expert discussion concluded that long gap esophageal atresia should be defined as any esophageal atresia that has no intra-abdominal air, realizing that this defines esophageal atresia with no distal trachea-esophageal fistula. Long gap esophageal atresia is considerably more complex than esophageal atresia with distal tracheo-esophageal fistulas, and should be referred to a center of expertise. The first choice is to preserve the native esophagus and pursue primary repair, delayed primary anastomosis or traction/growth techniques to achieve anastomosis. A cervical esophagostomy should be avoided if possible. Only if primary anastomosis is not possible, replacement techniques should be used. Jejunal interposition is proposed as the best option among the major esophageal atresia centers. In light of the infrequent occurrence of long gap esophageal atresia and the technically demanding techniques involved to achieve esophageal continuity, it is strongly advised to develop regional or national centers of expertise for the management and follow-up of these very complex patients.
TL;DR: For neonates with critical LVOTO undergoing single‐ventricle palliation, NW‐RVPA was associated with the best overall survival, and the impact of lower birth weight on survival may be mitigated after hybrid procedures compared with Norwood operations.
TL;DR: Teeth treated with REPs presented resolution of symptoms and, although clinical meaningful change was not achieved in all cases, increased root thickness, root length, and apical closure were observed at 30 months.
TL;DR: A rich description of how parents experience their grief in the first year after the death of their child, and how various bereavement follow-up and support services helped them during this time, is developed with the aim of informing follow- up andSupport services offered to bereaved parents.
Abstract: Our objective was to develop a rich description of how parents experience their grief in the first year after the death of their child, and how various bereavement follow-up and support services helped them during this time, with the aim of informing follow-up and support services offered to bereaved parents. Our findings situated parents' individual experiences of coping within the social and institutional contexts in which they grieved. In the first year after the death of their child, parents regulated their intense feelings of grief through loss-oriented, restoration-oriented, and/or meaning reconstruction strategies. Often, parents' relationships with others and many of the bereavement follow-up and support services helped them in this regard. This article also explores how the results may aid service providers in accompanying parents in a way that optimizes outcomes for these parents.
TL;DR: The results support the safety, tolerability, and potential efficacy of active rehabilitation for adolescents with persistent postconcussion symptoms and linear mixed modeling shows active rehabilitation was associated with a greater reduction on the Post-Concussion Symptom Scale than TAU only.
University of Toronto1, Children's Hospital of Eastern Ontario2, Montreal Children's Hospital3, University of British Columbia4, Boston Children's Hospital5, Holland Bloorview Kids Rehabilitation Hospital6, Laval University7, London Health Sciences Centre8, University of Calgary9, University Malaya Medical Centre10, University of Alberta11
TL;DR: This document provides a disease-specific review of illnesses associated with the necessity for long-term ventilation in children, including children with chronic lung disease, spinal muscle atrophy, muscular dystrophies, kyphoscoliosis, obesity hypoventilation syndrome, and central hypovENTilation syndromes.
Abstract: Over the last 30 to 40 years, improvements in technology, as well as changing clinical practice regarding the appropriateness of long-term ventilation in patients with “non-curable” disorders, have resulted in increasing numbers of children surviving what were previously considered fatal conditions. This has come but at the expense of requiring ongoing, long-term prolonged mechanical ventilation (both invasive and noninvasive). Although there are many publications pertaining to specific aspects of home mechanical ventilation (HMV) in children, there are few comprehensive guidelines that bring together all of the current literature. In 2011 the Canadian Thoracic Society HMV Guideline Committee published a review of the available English literature on topics related to HMV in adults, and completed a detailed guideline that will help standardize and improve the assessment and management of individuals requiring noninvasive or invasive HMV. This current document is intended to be a companion to the 20...
Catholic University of Leuven1, University of Barcelona2, French Institute of Health and Medical Research3, Montreal Children's Hospital4, University of Florence5, University Hospitals Bristol NHS Foundation Trust6, University of Buenos Aires7, University Hospital Complex Of Vigo8, Lille University of Science and Technology9, University of Zagreb10, Jaslok Hospital11, Federal University of Rio de Janeiro12, Hacettepe University13, Bristol Royal Hospital for Children14, University of Florida15, Centre Hospitalier de Luxembourg16, GlaxoSmithKline17, Alfred I. duPont Hospital for Children18
TL;DR: The frequency and severity of Blau uveitis highlight the need for close ophthalmologic surveillance as well as a search for more effective therapies, despite continuous systemic and local immunomodulatory therapy.
University of Toronto1, University of Alberta2, Alberta Children's Hospital3, University of Western Ontario4, Children's Hospital of Eastern Ontario5, McMaster Children's Hospital6, University of British Columbia7, McMaster University8, Public Health Agency of Canada9, University of Manitoba10, McGill University11, Montreal Children's Hospital12
TL;DR: Fourteen of 16 patients who were followed beyond 12 months post onset had neurologic deficits but showed ongoing clinical improvement and motor recovery.
Abstract: We describe the presenting features and long-term outcome of an unusual cluster of pediatric acute flaccid paralysis cases that occurred in Canada during the 2014 enterovirus D68 outbreak. Children (n = 25; median age 7.8 years) presenting to Canadian centers between July 1 and October 31, 2014, and who met diagnostic criteria for acute flaccid paralysis were evaluated retrospectively. The predominant presenting features included prodromal respiratory illness (n = 22), cerebrospinal fluid lymphocytic pleocytosis (n = 18), pain in neck/back (n = 14) and extremities (n = 10), bowel/bladder dysfunction (n = 9), focal central gray matter lesions found in all regions of the spinal cord within the cohort (n = 16), brain stem lesions (n = 8), and bulbar symptoms (n = 5). Enterovirus D68 was detectable in nasopharyngeal specimens (n = 7) but not in cerebrospinal fluid. Acute therapies (corticosteroids, intravenous immunoglobulins, plasmapheresis) were well tolerated with few side effects. Fourteen of 16 patients who were followed beyond 12 months post onset had neurologic deficits but showed ongoing clinical improvement and motor recovery.
TL;DR: There is a paucity of objective polysomnography data to definitively assess postoperative airway outcomes for PRS, and MDO appears to be the most effective technique based on the available PSG data.
Abstract: Background: Tongue-lip adhesion (TLA), mandibular distraction osteogenesis (MDO), and subperiosteal release of the floor of the mouth (SPRFM) are the most commonly performed surgical procedures to treat severe airway obstruction in infants born with Pierre Robin sequence (PRS). Objectives: To determine the rate of failure of each type of procedure, in terms of mortality and the need for tracheostomy, and to determine what proportion of infants have significant airway obstruction postoperatively as determined by polysomnography (PSG) and compare the data by procedure type. Method: A comprehensive literature search (1981 through June 2015) was done of the National Library of Medicine database using PubMed. Extracted data included diagnosis, type of surgery, and outcome including mortality, need for postoperative tracheostomy and details of PSG. Persistence of significant airway obstruction was defined as an apnea-hypopnea index >15 events/h on PSG. Results: Both mortality rate and need for tracheostomy ...
TL;DR: These data provide contemporary insights regarding the CHS workforce in North America and should help facilitate rational plans to address the needs of an expanding patient population.
TL;DR: Cost-effectiveness analyses, when done rigorously, can be very useful for the development of efficient surgical systems in LMICs, and a checklist to avoid these pitfalls is developed.
Abstract: Cost-effectiveness analysis can be a powerful policy-making tool. In the two decades since the first cost-effectiveness analyses in global surgery, the methodology has established the cost-effectiveness of many types of surgery in low- and middle-income countries (LMICs). However, with the crescendo of cost-effectiveness analyses in global surgery has come vast disparities in methodology, with only 15% of studies adhering to published guidelines. This has led to results that have varied up to 150-fold. The theoretical basis, common pitfalls, and guidelines-based recommendations for cost-effectiveness analyses are reviewed, and a checklist to be used for cost-effectiveness analyses in global surgery is created. Common pitfalls in global surgery cost-effectiveness analyses fall into five categories: the analytic perspective, cost measurement, effectiveness measurement, probability estimation, valuation of the counterfactual, and heterogeneity and uncertainty. These are reviewed in turn, and a checklist to avoid these pitfalls is developed. Cost-effectiveness analyses, when done rigorously, can be very useful for the development of efficient surgical systems in LMICs. This review highlights the common pitfalls in these analyses and methods to avoid these pitfalls.
TL;DR: Children and adolescents participating in an active rehabilitation program displayed improved post-concussion symptom severity at follow-up compared to pre-intervention and demonstrated improved physical, cognitive, emotional and sleep-related post- Concussion symptoms scores.
Abstract: Objective: To estimate the extent to which post-concussion symptoms were influenced by participation in an Active Rehabilitation (AR) program (aerobic exercise, coordination drills, visualization a...