Search

Abstract Prematurity is associated with lower exercise capacity, which relies on the integrity of the cardiovascular, pulmonary, and skeletal muscle systems. Our animal model mimicking prematurity-associated conditions showed altered muscle composition and atrophy in adulthood. This study aimed to compare muscle composition and strength in adults born preterm versus full-term controls. This observational cohort study recruited 55 adults born preterm, ≤ 29 weeks’ of gestation and 53 full-term controls who underwent musculoskeletal ultrasound imaging to assess morphology of the rectus femoris at rest and during a maximal voluntary contraction. Maximal voluntary contraction of the hands and legs were measured by manual dynamometry. In adults born preterm, there was lower muscle strength (handgrip: − 4.8 kg, 95% CI − 9.1, − 0.6; knee extensor: − 44.6 N/m, 95% CI − 63.4, − 25.8) and smaller muscle area (− 130 mm2, 95% CI − 207, − 53), which was more pronounced with a history of bronchopulmonary dysplasia. Muscle stiffness was increased in the preterm versus term group (0.4 m/s, 95% CI 0.04, 0.7). Prematurity is associated with alterations in skeletal muscle composition, area, and function in adulthood. These findings highlight the necessity to implement preventive and/or curative approaches to improve muscle development and function following preterm birth to enhance overall health in this population.

AimTo co-create parental presence practice recommendations across Canadian NICUs during pandemics caused by respiratory pathogens such as COVID-19.MethodsRecommendations were developed through evidence, context, Delphi and Values and Preferences methods. For Delphi 1 and 2, participants rated 50 items and 20 items respectively on a scale from 1 (very low importance) to 5 (very high). To determine consensus, evidence and context of benefits and harms were presented and discussed within the Values and Preference framework for the top-ranked items. An agreement of 80% or more was deemed consensus.ResultsAfter two Delphi rounds (n = 59 participants), 13 recommendations with the highest rated importance were identified. Consensus recommendations included 6 strong recommendations (parents as essential caregivers, providing skin-to-skin contact, direct or mothers' own expressed milk feeding, attending medical rounds, mental health and psychosocial services access, and inclusion of parent partners in pandemic response planning) and 7 conditional recommendations (providing hands-on care tasks, providing touch, two parents present at the same time, food and drink access, use of communication devices, and in-person access to medical rounds and mental health and psychosocial services).ConclusionThese recommendations can guide institutions in developing strategies for parental presence during pandemics caused by respiratory pathogens like COVID-19

IntroductionCycling increased in popularity during the COVID-19 pandemic, but the impact on cycling injuries is not known. We examined the effect of lockdowns on cycling injury hospitalizations. MethodsWe identified hospitalizations for cycling injuries in Quebec, Canada, between April 2006 and March 2021. We used rate ratios (RR) and 95% confidence intervals (CI) to compare hospitalization rates by type of cycling injury and anatomical site during two waves of the pandemic. We performed interrupted time series regression to assess the effect of lockdowns on monthly cycling injury hospitalization rates, according to age, sex and other characteristics. ResultsThere were 2020 hospitalizations for cycling injuries between March 2020 and March 2021, including 617 during the first lockdown and 67 during the second lockdown. Compared with the period before the pandemic, risk of cycling-related injuries during the first lockdown increased the most for fractures (RR = 1.44; 95% CI: 1.26–1.64) and head and neck injuries (RR = 1.59; 95% CI: 1.19–2.12). Cycling injury hospitalization rates increased significantly among adults, adolescents and individuals from socioeconomically advantaged neighbourhoods or those with low concentrations of racialized people every month of the first lockdown. The second lockdown was not associated with cycling injuries. ConclusionThe first lockdown triggered a sharp increase in cycling injury hospitalizations, especially among adults, adolescents and individuals from socioeconomically advantaged and less racialized neighbourhoods.

IntroductionLe vélo a gagné en popularité au cours de la pandémie de COVID-19, sans que l’on connaisse l’impact de cette situation sur les blessures chez les cyclistes. Nous avons étudié l’effet des confinements sur les hospitalisations associées à des blessures à vélo. MéthodologieNous avons relevé les cas d’hospitalisation pour blessure à vélo au Québec (Canada) entre avril 2006 et mars 2021. Nous avons utilisé des rapports de taux (RT) et des intervalles de confiance (IC) à 95 % pour comparer les taux d’hospitalisation par type de blessure subie à vélo et par site anatomique au cours de deux vagues de la pandémie. Nous avons appliqué un modèle de régression de type séries chronologiques interrompues afin d’évaluer l’effet des confinements sur les taux mensuels d’hospitalisation associés aux blessures à vélo, en fonction de l’âge, du sexe et d’autres caractéristiques. RésultatsIl y a eu 2 020 hospitalisations pour blessure à vélo entre mars 2020 et mars 2021, dont 617 pendant le premier confinement et 67 pendant le deuxième confinement. Par rapport à la période prépandémique, la plus grande augmentation du risque de blessures liées aux déplacements à vélo pendant le premier confinement a concerné les fractures (RT = 1,44; IC à 95 % : 1,26 à 1,64) et les lésions traumatiques à la tête et au cou (RT = 1,59; IC à 95 % : 1,19 à 2,12). Lors de chaque mois durant le premier confinement, les taux d’hospitalisation pour blessure à vélo ont été considérablement plus élevés qu’en période prépandémique chez les adultes, les adolescents et les personnes vivant dans des quartiers favorisés sur le plan socio-économique ainsi que dans des quartiers à faible densité de population dite racisée. Aucune association avec les blessures à vélo n’a été constatée lors du deuxième confinement. ConclusionLe premier confinement a déclenché une augmentation marquée des hospitalisations pour blessures à vélo, en particulier chez les adultes, les adolescents et les personnes vivant dans des quartiers plus favorisés sur le plan socio-économique et à plus faible densité de population dite racisée.

Abstract Introduction Executive function deficits and adverse psychological outcomes are common in youth with congenital heart disease (CHD) or born preterm. Association white matter bundles play a critical role in higher order cognitive and emotional functions and alterations to their microstructural organization may result in adverse neuropsychological functioning. This study aimed to examine the relationship of myelination and axon density and orientation alterations within association bundles with executive functioning, psychosocial well‐being, and resilience in youth with CHD or born preterm. Methods Youth aged 16 to 26 years born with complex CHD or preterm at ≤33 weeks of gestational age and healthy controls completed a brain MRI and self‐report assessments of executive functioning, psychosocial well‐being, and resilience. Multicomponent driven equilibrium single‐pulse observation of T1 and T2 and neurite orientation dispersion and density imaging were used to calculate average myelin water fraction (MWF), neurite density index (NDI), and orientation dispersion index values for eight bilateral association bundles. The relationships of bundle‐average metrics with neuropsychological outcomes were explored with linear regression and mediation analyses. Results In the CHD group, lower MWF in several bundles was associated with poorer working memory and behavioral self‐monitoring and mediated self‐monitoring deficits relative to controls. In the preterm group, lower NDI in several bundles was associated with poorer emotional control and lower MWF in the left superior longitudinal fasciculus III mediated planning/organizing deficits relative to controls. No significant relationships were observed for psychosocial well‐being or resilience. Conclusion The findings of this study suggest that microstructural alterations to association bundles, including lower myelination and axon density, have different relationships with executive functioning in youth with CHD and youth born preterm. Future studies should aim to characterize other neurobiological, social, and environmental influences that may interact with white matter microstructure and neuropsychological functioning in these at‐risk individuals.

Abstract Background Preterm birth may affect maternal mental health, yet most studies focus on postpartum mental disorders only. We explored the relationship between preterm delivery and the long-term risk of maternal hospitalization for mental illness after pregnancy. Methods We performed a longitudinal cohort study of 1,381,300 women who delivered between 1989 and 2021 in Quebec, Canada, and had no prior history of mental illness. The exposure was preterm birth, including extreme (

IntroductionAlterations to white matter microstructure as detected by diffusion tensor imaging have been documented in both individuals born with congenital heart disease (CHD) and individuals born preterm. However, it remains unclear if these disturbances are the consequence of similar underlying microstructural disruptions. This study used multicomponent driven equilibrium single pulse observation of T1 and T2 (mcDESPOT) and neurite orientation dispersion and density imaging (NODDI) to characterize and compare alterations to three specific microstructural elements of white matter – myelination, axon density, and axon orientation – in youth born with CHD or born preterm.MethodsParticipants aged 16 to 26 years with operated CHD or born ≤33 weeks gestational age and a group of healthy peers of the same age underwent a brain MRI including mcDESPOT and high angular resolution diffusion imaging acquisitions. Using tractometry, average values of myelin water fraction (MWF), neurite density index (NDI), and orientation dispersion index (ODI) were first calculated and compared between groups for 30 white matter bundles. Afterwards, bundle profiling was performed to further characterize the topology of the detected microstructural alterations.ResultsThe CHD and preterm groups both presented with widespread bundles and bundle segments with lower MWF, accompanied by some occurrences of lower NDI, relative to controls. While there were no differences in ODI between the CHD and control groups, the preterm group presented with both higher and lower ODI compared to the control group and lower ODI compared to the CHD group.DiscussionWhile youth born with CHD or born preterm both presented with apparent deficits in white matter myelination and axon density, youth born preterm presented with a unique profile of altered axonal organization. Future longitudinal studies should aim to better understand the emergence of these common and distinct microstructural alterations, which could orient the development of novel therapeutic approaches.

BackgroundInvasive fungal infection (IFI) is associated with significant mortality and morbidity among preterm infants but there has been no population-based study of long-term neurodevelopmental outcomes. The objective of this study was to examine population-based incidence trends as well as mortality, short term in-hospital morbidity and long-term neurodevelopmental outcomes among preterm infants with IFI, non-fungal infections (NFI) and no infections in Canada.MethodsWe conducted a retrospective cohort study of 8,408 infants born at 24 h, maternal antibiotic treatment, antenatal steroid use, cesarean section), infants with IFI had significantly higher odds of sNDI than NFI (aOR: 2.19; 95% CI: 1.23, 3.91) or no infections (aOR: 2.97; 95% CI: 1.55, 5.71), and higher odds of mortality than NFI (aOR: 1.55; 95% CI: 1.07, 2.26) or no infections (aOR: 1.45; 95% CI: 0.97, 2.17).ConclusionsPreterm infants with invasive fungal infections have significantly higher incidence of mortality and adverse neurodevelopmental outcomes than those with non-invasive fungal infections and no infections.

Introduction Therapeutic hypothermia (TH) became the standard of care treatment for neonates with moderate and severe neonatal encephalopathy (NE) in most industrialized countries about 10 years ago. Although TH is effective in reducing mortality and the incidence of severe developmental disabilities, the recent literature converges in reporting frequent cognitive and behavioural difficulties at school entry in children with NE-TH. Although these challenges are deemed minor compared with cerebral palsy and intellectual disability, their impacts on a child’s self-determination and family’s well-being are quite significant. Therefore, the nature and extent of these difficulties need to be comprehensively described so that appropriate care can be offered.Methods and analysis The current study will be the largest follow-up study of neonates with NE treated with TH to characterize their developmental outcomes and associated brain structural profiles at 9 years of age. Specifically, we will compare executive function, attention, social cognition, behaviour, anxiety, self-esteem, peer problems, brain volume, cortical features, white matter microstructure and myelination between children with NE-TH and matched peers without NE. Associations of perinatal risk factors and structural brain integrity with cognitive, behavioural and psycho-emotional deficits will be evaluated to inform about the potential aggravating and protective factors associated with function.Ethics and dissemination This study is supported by the Canadian Institute of Health Research (202203PJT-480065-CHI-CFAC-168509), and received approval from the Pediatric Ethical Review Board of the McGill University Health Center (MP-37-2023-9320). The study findings will be disseminated in scientific journals and conferences and presented to parental associations and healthcare providers to inform best practices.Trial registration number NCT05756296.

BackgroundInfants born at 29–36 weeks gestational age (GA) are at risk of experiencing neurodevelopmental challenges. We hypothesize that cerebral hemodynamics and oxygen metabolism measured by bedside optical brain monitoring are potential biomarkers of brain development and are associated with neurological examination at term-equivalent age (TEA).MethodsPreterm infants (N = 133) born 29–36 weeks GA and admitted in the neonatal intensive care unit were enrolled in this prospective cohort study. Combined frequency-domain near infrared spectroscopy (FDNIRS) and diffuse correlation spectroscopy (DCS) were used from birth to TEA to measure cerebral hemoglobin oxygen saturation and an index of microvascular cerebral blood flow (CBFi) along with peripheral arterial oxygen saturation (SpO2). In combination with hemoglobin concentration in the blood, these parameters were used to derive cerebral oxygen extraction fraction (OEF) and an index of cerebral oxygen metabolism (CMRO2i). The Amiel-Tison and Gosselin Neurological Assessment was performed at TEA. Linear regression models were used to assess the associations between changes in FDNIRS-DCS parameters from birth to TEA and GA at birth. Logistic regression models were used to assess the associations between changes in FDNIRS-DCS parameters from birth to TEA and neurological examination at TEA.ResultsSteeper increases in CBFi (p < 0.0001) and CMRO2i (p = 0.0003) were associated with higher GA at birth. Changes in OEF, CBFi, and CMRO2i from birth to TEA were not associated with neurological examination at TEA.ConclusionIn this population, cerebral FDNIRS-DCS parameters were not associated with neurological examination at TEA. Larger increases in CBFi and CMRO2i from birth to TEA were associated with higher GA. Non-invasive bedside FDNIRS-DCS monitoring provides cerebral hemodynamic and metabolic parameters that may complement neurological examination to assess brain development in preterm infants.

Introduction: Support groups in neonatal intensive care units (NICUs) are beneficial to parents. The usefulness of prenatal support groups for prospective parents who will have a newborn requiring admission to the NICU has never been investigated. Methods: We assessed the needs of NICU parents regarding topics they would have wished to discuss prenatally and developed the content of a prenatal support workshop. A standardized survey prospectively evaluated the perspectives of pregnant women admitted to a high-risk pregnancy unit who participated in the resulting workshops. Results: During needs assessment, 295 parents invoked themes they would have wished to discuss antenatally: parental guilt, future parental role, normalizing their experience/emotions, coping with many losses, adapting to their new reality, control and trust, information about the NICU, technology around the baby, common neonatal interventions, the NICU clinical team, and the role of parents in the team. These findings were used to develop the workshop, including a moderator checklist and a visual presentation. Practical aspects of the meetings were tested/finalized during a pre-pilot phase. Among 21 pregnant women who answered the survey (average gestational age 29.3 weeks), all agreed that the workshop was useful, that it made them feel less lonely (95%), that exchanges with other women were beneficial (95%) and gave them a certain amount of control over their situation (89%). All answers to open-ended questions were positive. Conclusion: Prenatal educational/support workshops provide a unique and useful means to support future NICU parents. Future investigations will explore whether these prenatal interventions improve clinical outcomes.

Introduction Brief Resolved Unexplained Events (BRUEs) are a common presentation among infants. While most of these events are benign and self-limited, guidelines published by the American Academy of Pediatrics inaccurately identify many patients as higher-risk of a serious underlying aetiology (positive predictive value 5%). Recently, new clinical prediction rules have been derived to more accurately stratify patients. This data were however geographically limited to the USA, with no large studies to date assessing the BRUE population in a different healthcare setting. The study’s aim is to describe the clinical management and outcomes of infants presenting to Canadian hospitals with BRUEs and to externally validate the BRUE clinical prediction rules in identified cases.Methods and analysis This is a multicentre retrospective study, conducted within the Canadian Paediatric Inpatient Research Network (PIRN). Infants (

Abstract Background In the NICU, interventions intended to enhance maternal sensitivity are indicated in order to optimize preterm infant development and long-term mother-infant attachment. A novel nursing intervention was developed following a theory-oriented methodology and is based upon the guided participation theory for mothers to participate in their preterm infant’s therapeutic POSITioning and care (GP_Posit). The primary objective of this study is to evaluate the feasibility and acceptability of (i) the study design; and (ii) the experimental GP_Posit nursing intervention during NICU hospitalization. The secondary objective is to estimate the preliminary effects of GP_Posit on maternal and preterm infant outcomes. Methods A pilot parallel-group randomized clinical trial (RCT) was designed where mother-preterm infant dyads are being recruited and randomized to a control group (usual care) or experimental group (GP_Posit intervention). Data collection includes feasibility and acceptability data as well as preliminary effects on maternal sensitivity and infant neurodevelopment. Ethical approval from the University Hospital ethical board was obtained in January 2018 (2017–1540). Discussion Data collection for this pilot study is expected to end in 2020. Results of this pilot study will inform about the feasibility and acceptability of the study design and GP_Posit intervention, a nursing intervention having the potential to favor maternal sensitivity and infant neurodevelopment in the NICU and guide the elaboration of a large-scale RCT. Trial registration clinicaltrial.gov, NCT03677752 . Registered 19 September 2018.

Children born very preterm are at risk of severe neurodevelopmental impairment, a composite endpoint that includes cerebral palsy, developmental delay, and hearing and visual impairment defined by medical professionals. We aimed to describe preterm birth stakeholders’ perspectives on this classification. Ten clinical scenarios describing 18-month-old children with different components of severe neurodevelopmental impairment and one scenario of a typically developing child (control) were distributed to parents and stakeholders using a snowball sampling technique. For each scenario, participants rated health on a scale from 0 to 10 and whether the scenario represented a severe condition. Results were analyzed descriptively and mean differences from the control scenario were compared using a linear mixed-effects model. Stakeholders (number = 827) completed 4553 scenarios. Median health scores for each scenario varied from 6 to 10. The rating for the cerebral palsy and language delay scenario was significantly lower (mean difference −4.3; 95% confidence interval: −4.4, −4.1) than the control. The proportion of respondents rating a scenario as “severe” ranged from 5% for cognitive delay to 55% for cerebral palsy and language delay. Most participants disagreed with the rating used in research to describe severe neurodevelopmental impairment in preterm children. The term should be redefined to align with stakeholder perceptions.

Neurodevelopmental challenges in children born very preterm are common and not improving. This study tested the feasibility of using Evidence-based Practice to Improve Quality (EPIQ), a proven quality improvement technique that incorporates scientific evidence to target improving language abilities in very preterm populations in 10 Canadian neonatal follow-up programs. Feasibility was defined as at least 70% of sites completing four intervention cycles and 75% of cycles meeting targeted aims. Systematic reviews were reviewed and performed, an online quality improvement educational tool was developed, multidisciplinary teams that included parents were created and trained, and sites provided virtual support to implement and audit locally at least four intervention cycles of approximately 6 months in duration. Eight of ten sites implemented at least four intervention cycles. Of the 48 cycles completed, audits showed 41 (85%) met their aim. Though COVID-19 was a barrier, parent involvement, champions, and institutional support facilitated success. EPIQ is a feasible quality improvement methodology to implement family-integrated evidence-informed interventions to support language interventions in neonatal follow-up programs. Further studies are required to identify potential benefits of service outcomes, patients, and families and to evaluate sustainability.

Objective: To determine the association between postnatal age (PNA) at first administration of systemic postnatal steroids (sPNS) for bronchopulmonary dysplasia (BPD) and mortality or significant neurodevelopmental impairment (sNDI) at 18–24 months corrected age (CA) in infants < 29 weeks’ gestation. Methods: Data from the Canadian Neonatal Network and Canadian Neonatal Follow-up Network databases were used to conduct this retrospective cohort study. Infants exposed to sPNS for BPD after the 1st week of age were included and categorized into 8 groups based on the postnatal week of the exposure. The primary outcome was a composite of mortality or sNDI. A multivariable logistic regression model adjusting for potential confounders was used to determine the association between the sPNS and ND outcomes. Results: Of the 10,448 eligible infants, follow-up data were available for 6200 (59.3%) infants. The proportion of infants at first sPNS administration was: 8%, 17.5%, 23.1%, 18.7%, 12.6%, 8.3%, 5.8%, and 6% in the 2nd, 3rd, 4th, 5th, 6th, 7th, 8–9th, and ≥10th week of PNA respectively. No significant association between the timing of sPNS administration and the composite outcome of mortality or sNDI was observed. The odds of sNDI and Bayley-III motor composite < 70 increased by 1.5% (95% CI 0.4, 2.9%) and 2.6% (95% CI 0.9, 4.4%), respectively, with each one-week delay in the age of initiation of sPNS. Conclusions: No significant association was observed between the composite outcome of mortality or sNDI and PNA of sPNS. Among survivors, each week’s delay in initiation of sPNS may increase the odds of sNDI and motor delay.

Preterm infants are at risk for adverse neurodevelopmental outcomes, especially language delay. Preterm infants < 29 weeks’ gestational age, cared for in Canadian Neonatal Follow-Up Network affiliated hospitals, were assessed between 18 to 21 months corrected age using the Bayley-III. Bayley-III Language Composite Scores were compared using univariate and multivariate analyses for children in three primary language groups: English, French and other. 6146 children were included. The primary language at home was English, French or another language for 3708 children (60%), 1312 children (21%) and 1126 children (18%), respectively, and overall, 44% were exposed to two or more languages at home. Univariate analysis showed that primary language was associated with lower Bayley-III Language scores; however, multivariate analyses demonstrated that neither primary language nor language of administration were significantly associated with lower language scores when adjusted for gestational age, other developmental delays and sociodemographic factors, but multiple language exposure was. Sociodemographic and other factors are more important in determining language development than primary language at home. Further studies are needed to examine the association between exposure to multiple languages and lower Bayley-III language scores in preterm infants.

Introduction: Preterm infants are at increased risk of osteopenia of prematurity due to insufficient bone mineral accretion. Data on long term effects of prematurity on bone health are conflicting. This study aimed to compare bone mineral density (BMD) in young adults born very preterm and full-term controls and to examine factors associated with long-term bone health. Methods: This observational cross-sectional study enrolled 101 young adults (18–29 years) born

IntroductionRisk of developing a malignancy when born premature is unknown. We hypothesised that risk of certain cancers might be increased in youth born preterm versus term. We therefore performed a systematic review and meta-analysis to evaluate the incidence of malignancy in the context of preterm birth, according to various cancer types.MethodsThe study was designed per MOOSE and PRISMA guidelines. Articles were identified through November 2015. Observational studies exploring the association between childhood malignancy and birth characteristics were included. Of the 1658 records identified, 109 full text articles were evaluated for eligibility. Random effects meta-analyses were conducted on 10/26 studies retained; 95% confidence intervals were computed and adjusted following sensitivity analysis. Publication bias was evaluated using funnel plots, Begg's and Egger's tests.ResultsNo differences in risk of primary central nervous system tumor [OR 1.05; 95% CI 0.93-1.17, 5 studies, 580 cases] and neuroblastoma [OR 1.09; 95% CI 0.90-1.32, 5 studies, 211 cases] were observed in individuals born ConclusionsChildren born premature may be at increased risk for hepatoblastoma but there is no strong evidence of an increased risk of primary central nervous system tumours or neuroblastoma. There is insufficient evidence to conclude whether prematurity modulates the risk of other childhood cancers.

Background Preterm birth is linked to cardiovascular risks and diseases. Endothelial progenitor cells play a critical role in vascular development and repair. Cord blood endothelial progenitor cells of preterm‐born infants, especially endothelial colony‐forming cells (ECFC), show enhanced susceptibility to prematurity‐related pro‐oxidant stress. Whether ECFC dysfunction is present in adulthood following preterm birth is unknown. Methods and Results This cross‐sectional observational study includes 55 preterm‐born (≤29 gestational weeks) young adults (18–29 years old, 38% male) and 55 sex‐ and age‐matched full‐term controls. ECFC were isolated from peripheral blood; cell proliferative and vascular cord formation capacities were assessed in vitro. Daytime systolic blood pressure was higher, whereas glucose tolerance and body mass index were lower in preterm‐born subjects. ECFC colonies grew in culture for 62% of full‐term‐ and 58% of preterm‐born participants. Preterm‐born participants have formed ECFC colonies later in culture and have reduced proliferation compared with controls. Only in preterm‐born individuals, we observed that the later the ECFC colony grows in culture, the worse was overall ECFC function. In addition, in preterms, elevated systolic blood pressure significantly correlated with reduced ECFC proliferation (rS=−0.463; P=0.030) and numbers of branches formed on matrigel (rS=−0.443; P=0.039). In preterm‐born subjects, bronchopulmonary dysplasia was associated with impaired ECFC function, whereas exposure to antenatal steroids related to better ECFC function. Conclusions This study is the first to examine ECFC in preterm‐born adults and to demonstrate ECFC dysfunction compared with full‐term controls. In the preterm‐born group, ECFC dysfunction was associated with bronchopulmonary dysplasia, the major prematurity‐related neonatal morbidity, and with increased systolic blood pressure into adulthood.

Chronic granulomatous disease (CGD) is a rare primary immune deficiency caused by mutations in genes coding for components of the nicotinamide adenine dinucleotide phosphate oxidase, characterized by severe and recurrent bacterial and fungal infections, together with inflammatory complications. Dysregulation of inflammatory responses are often present in this disease and may lead to granulomatous lesions, most often affecting the gastrointestinal (GI) and urinary tracts. Treatment of inflammatory complications usually includes corticosteroids, whereas antimicrobial prophylaxis is used for infection prevention. Curative treatment of both infectious susceptibility and inflammatory disease can be achieved by hematopoietic stem cell transplantation. We report herein three patients with the same mutation of the CYBB gene who presented with very early-onset and severe GI manifestations of X-linked CGD. The most severely affected patient had evidence of antenatal inflammatory involvement of the GI and urinary tracts. Extreme hyperleukocytosis with eosinophilia and high inflammatory markers were observed in all three patients. A Mycobacterium avium lung infection and an unidentified fungal lung infection occurred in two patients both during their first year of life, which is indicative of the severity of the disease. All three patients underwent bone marrow transplantation and recovered fully from their initial symptoms. To our knowledge, these are the first reports of patients with such an early-onset and severe inflammatory manifestations of CGD.

Bronchopulmonary dysplasia (BPD) is an emerging risk factor for chronic obstructive pulmonary disease. For BPD survivors, there are no guidelines for the management of lung disease that is often misdiagnosed as asthma. Pulmonary magnetic resonsance imaging (MRI) provides clinically-relevant lung biomarkers of ventilation abnormalities and emphysema. Here our objective was to quantify lung MRI biomarkers in adults with BPD to understand the underlying pathophysiologies responsible for their symptoms and abnormal pulmonary-function. We hypothesized that MRI measurements would be abnormal and reflect emphysema, not airways disease. Patients aged 20–29 year and born ≤32 weeks gestational age were included and those with MRI contraindications were excluded. A 25-year-old female never-smoker born

Aim: Parents of children born preterm have identified outcomes to be measured for audit and research at 18-24 months of age: child well-being, quality of life/function, socio-emotional/behavioural outcomes, respiratory, feeding, sleeping, and caregiver mental health. The aim was to identify the best tools to measure these seven domains. Methods: Seven working groups completed literature reviews and evaluated potential tools to measure these outcomes in children aged 18-24 months. A group of experts and parents voted on the preferred tools in a workshop and by questionnaire. Consensus was 80% agreement. Results: Consensus was obtained for seven brief, inexpensive, parent friendly valid measures available in English or French for use in a minimum dataset and potential alternative measures for use in funded research. Conclusion: Valid questionnaires and tools to measure parent-identified outcomes in young preterm children exist. This study will facilitate research and collection of data important to families. [ABSTRACT FROM AUTHOR]

Copyright of Promotion de la Santé et Prévention des Maladies Chroniques au Canada is the property of Public Health Agency of Canada and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)