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2021

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The Intranasal Dexmedetomidine Plus Ketamine For Procedural Sedation In Children, Adaptive Randomized Controlled Non-Inferiority Multicenter Trial (Ketodex): A Statistical Analysis Plan, Anna Heath, Juan David Rios, Eleanor Pullenayegum, Petros Pechlivanoglou, Martin Offringa, Maryna Yaskina, Rick Watts, Shana Rimmer, Terry P. Klassen, Kamary Coriolano, Naveen Poonai, Darcy Beer, Scott Sawyer, Maala Bhatt, April Kam, Quynh Doan, Vikram Sabhaney, Serena Hickes, Samina Ali, Karly Stillwell, Tannis Erickson, Chelsea Bowkett, Chelsea Bowkett, Carolyn Shimmin, Brendon Foot, Candace Mcgahern, Redjana Carciurmaruj, Jeannine Schellenberg Dec 2021

The Intranasal Dexmedetomidine Plus Ketamine For Procedural Sedation In Children, Adaptive Randomized Controlled Non-Inferiority Multicenter Trial (Ketodex): A Statistical Analysis Plan, Anna Heath, Juan David Rios, Eleanor Pullenayegum, Petros Pechlivanoglou, Martin Offringa, Maryna Yaskina, Rick Watts, Shana Rimmer, Terry P. Klassen, Kamary Coriolano, Naveen Poonai, Darcy Beer, Scott Sawyer, Maala Bhatt, April Kam, Quynh Doan, Vikram Sabhaney, Serena Hickes, Samina Ali, Karly Stillwell, Tannis Erickson, Chelsea Bowkett, Chelsea Bowkett, Carolyn Shimmin, Brendon Foot, Candace Mcgahern, Redjana Carciurmaruj, Jeannine Schellenberg

Paediatrics Publications

Background: Procedural sedation and analgesia (PSA) is frequently required to perform closed reductions for fractures and dislocations in children. Intravenous (IV) ketamine is the most commonly used sedative agent for closed reductions. However, as children find IV insertion a distressing and painful procedure, there is need to identify a feasible alternative route of administration. There is evidence that a combination of dexmedetomidine and ketamine (ketodex), administered intranasally (IN), could provide adequate sedation for closed reductions while avoiding the need for IV insertion. However, there is uncertainty about the optimal combination dose for the two agents and whether it can provide …

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School's Out: Parenting Stress And Screen Time Use In School-Age Children During The Covid-19 Pandemic, Diane Seguin, Elizabeth Kuenzel, J. Bruce Morton, Emma G. Duerden Dec 2021

School's Out: Parenting Stress And Screen Time Use In School-Age Children During The Covid-19 Pandemic, Diane Seguin, Elizabeth Kuenzel, J. Bruce Morton, Emma G. Duerden

Paediatrics Publications

Background During the COVID-19 pandemic, millions of children abruptly moved to online schooling, which required high levels of parental involvement. Family routines were disrupted, potentially increasing parental stress, and may be reflected in greater media screen time use in children. Objectives To determine whether (1) parenting styles and (2) parenting stress were associated with children's screen time use during the pandemic compared to the pre-pandemic period. Methods Parents (> 18 years of age) were recruited to complete an online survey regarding changes in their children's (6–12 years) screen time use and daily activities before and during the pandemic. Stress and …

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Animal, Human, And 23Na Mri Imaging Evidence For The Negative Impact Of High Dietary Salt In Children, Guido Filler, Fabio Salerno, Christopher William Mcintyre, Maria E.Díaz González De Ferris Dec 2021

Animal, Human, And 23Na Mri Imaging Evidence For The Negative Impact Of High Dietary Salt In Children, Guido Filler, Fabio Salerno, Christopher William Mcintyre, Maria E.Díaz González De Ferris

Paediatrics Publications

Purpose of the Review: Conditions typically prevalent in adults such as hypertension, kidney stones, osteoporosis, and chronic kidney disease are increasing among adolescents and young adults (AYA). The purpose of this review is to describe the association of these conditions to a high salt diet among pediatric patients. Recent Findings: We present animal, human, and 23Na MRI evidence associated with the negative impact of high dietary salt in children. Special focus is placed on novel 23Na MRI imaging which reveals the important concept of a third compartment for sodium storage in soft tissue. Finally, we make recommendations on who should …

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Adolescent And Caregiver Attitudes Towards Telemedicine Use In Pediatric Nephrology, Yi Qiu, Sherry Coulson, Christopher William Mcintyre, Brooke Wile, Guido Filler Dec 2021

Adolescent And Caregiver Attitudes Towards Telemedicine Use In Pediatric Nephrology, Yi Qiu, Sherry Coulson, Christopher William Mcintyre, Brooke Wile, Guido Filler

Paediatrics Publications

Background: Telemedicine is increasingly utilized as an alternative to in person consultation. Current pandemic conditions are providing additional impetus to virtual care delivery. We compared both adolescent and caregiver (parent or guardian) attitudes towards telemedicine (here as tertiary center to remote health care location) as a crucial determinant of longer-term effectiveness. Methods: This qualitative research study analyzed transcribed structured telephone interviews with both 11–18 year-old pediatric nephrology patients and their caregivers and performed a quantitative analysis of patient demographics, disease factors and distance to tertiary center vs. telemedicine center. Results: The study was conducted in a medium-sized tertiary pediatric nephrology …

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Evaluating The Association Between Extreme Heat And Mortality In Urban Southwestern Ontario Using Different Temperature Data Sources, Kristin K. Clemens, Alexandra M. Ouédraogo, Lihua Li, James A. Voogt, Jason Gilliland, E. Scott Krayenhoff, Sylvie Leroyer, Salimah Z. Shariff Dec 2021

Evaluating The Association Between Extreme Heat And Mortality In Urban Southwestern Ontario Using Different Temperature Data Sources, Kristin K. Clemens, Alexandra M. Ouédraogo, Lihua Li, James A. Voogt, Jason Gilliland, E. Scott Krayenhoff, Sylvie Leroyer, Salimah Z. Shariff

Paediatrics Publications

Urban areas have complex thermal distribution. We examined the association between extreme temperature and mortality in urban Ontario, using two temperature data sources: high-resolution and weather station data. We used distributed lag non-linear Poisson models to examine census division-specific temperature–mortality associations between May and September 2005–2012. We used random-effect multivariate meta-analysis to pool results, adjusted for air pollution and temporal trends, and presented risks at the 99th percentile compared to minimum mortality temperature. As additional analyses, we varied knots, examined associations using different temperature metrics (humidex and minimum temperature), and explored relationships using different referent values (most frequent temperature, 75th …

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Correction To: Providing Dental Insurance Can Positively Impact Oral Health Outcomes In Ontario (Bmc Health Services Research, (2020), 20, 1, (124), 10.1186/S12913-020-4967-3), Nevena Zivkovic, Musfer Aldossri, Noha Gomaa, Julie W. Farmer, Sonica Singhal, Carlos Quiñonez, Vahid Ravaghi Dec 2021

Correction To: Providing Dental Insurance Can Positively Impact Oral Health Outcomes In Ontario (Bmc Health Services Research, (2020), 20, 1, (124), 10.1186/S12913-020-4967-3), Nevena Zivkovic, Musfer Aldossri, Noha Gomaa, Julie W. Farmer, Sonica Singhal, Carlos Quiñonez, Vahid Ravaghi

Paediatrics Publications

Following publication of the original article [1], the authors would like to add some information in the Competing interests section. The updated content in the Competing interests is shown below: Carlos Quiñonez receives consulting income for dental care related issues from Green Shield Canada. All other authors declare no competing interests. The original article has been corrected.

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A Crossroad Between Placental And Tumor Biology: What Have We Learnt?, Peeyush K. Lala, Pinki Nandi, Ali Hadi, Chidambra Halari Dec 2021

A Crossroad Between Placental And Tumor Biology: What Have We Learnt?, Peeyush K. Lala, Pinki Nandi, Ali Hadi, Chidambra Halari

Paediatrics Publications

Placenta in certain species including the human has evolved as a highly invasive tumor-like organ invading the uterus aned its vasculature to derive oxygen and nutrients for the fetus and exchange waste products. While several excellent reviews have been written comparing hemochorial placentation with tumors, no comprehensive review is available dealing with mechanistic insights into what makes them different, and what tumor biologists can learn from placental biologists, and vice versa. In this review, we analyze the structure-function relationship of the human placenta, emphasizing the functional need of the spatio-temporally orchestrated trophoblast invasiveness for fetal development and growth, and pathological …

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A National Study Of Choanal Atresia In Tertiary Care Centers In Canada – Part I: Clinical Presentation, Josee Paradis, Agnieszka Dzioba, Hamdy El-Hakim, Paul Hong, Frederick K. Kozak, Lily H.P. Nguyen, Demitri Perera, Evan Jon Propst Dec 2021

A National Study Of Choanal Atresia In Tertiary Care Centers In Canada – Part I: Clinical Presentation, Josee Paradis, Agnieszka Dzioba, Hamdy El-Hakim, Paul Hong, Frederick K. Kozak, Lily H.P. Nguyen, Demitri Perera, Evan Jon Propst

Paediatrics Publications

Background: To evaluate the clinical presentation of choanal atresia (CA) in tertiary centers across Canada. Methods: Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with CA at a participating center. Results: The health charts of 215 patients (59.6% female) with CA were reviewed and included in this study. The mean age of patients at time of CA presentation was 0.4 months (range 0.1 to 7.2 months) for bilateral CA and 37.8 months (range 0.1 to 164.1 months) for unilateral cases. The most common presenting symptoms for …

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A National Study Of Choanal Atresia In Tertiary Care Centers In Canada – Part Ii: Clinical Management, Josee Paradis, Agnieszka Dzioba, Hamdy El-Hakim, Paul Hong, Frederick K. Kozak, Lily H.P. Nguyen, Demitri Perera, Evan Jon Propst Dec 2021

A National Study Of Choanal Atresia In Tertiary Care Centers In Canada – Part Ii: Clinical Management, Josee Paradis, Agnieszka Dzioba, Hamdy El-Hakim, Paul Hong, Frederick K. Kozak, Lily H.P. Nguyen, Demitri Perera, Evan Jon Propst

Paediatrics Publications

Background: To evaluate the clinical management of choanal atresia (CA) in tertiary centers across Canada. Methods: Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with choanal atresia to a participating center. Results: The health charts of 215 patients (59.6% female) with choanal atresia (CA) were reviewed. Mean age of initial surgical repair was 0.8 months for bilateral CA, and 48.6 months for unilateral CA. Approaches of surgical repair consisted of endoscopic transnasal (31.7%), non-endoscopic transnasal (42.6%), and transpalatal (25.2%). Stents were used on 70.7% of patients. …

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A Novel Preference-Informed Complementary Trial (Pict) Design For Clinical Trial Research Influenced By Strong Patient Preferences, Samina Ali, Gareth Hopkin, Naveen Poonai, Lawrence Richer, Maryna Yaskina, Anna Heath, Terry Paul Klassen, Chris Mccabe, Amy Drendel, Jeff Round, Jeff Round, Martin Offringa, Petros Pechlivanoglou, Petros Pechlivanoglou, Eleanor Pullenayegum, Eleanor Pullenayegum, David Rios, Marie Christine Auclair, Kelly Kim, Lise Bourrier, Lauren Dawson, Kamary Coriolano Dasilva, Pamela Marples, Rick Watts, Jennifer Thull-Freedman, Patrick Mcgrath Dec 2021

A Novel Preference-Informed Complementary Trial (Pict) Design For Clinical Trial Research Influenced By Strong Patient Preferences, Samina Ali, Gareth Hopkin, Naveen Poonai, Lawrence Richer, Maryna Yaskina, Anna Heath, Terry Paul Klassen, Chris Mccabe, Amy Drendel, Jeff Round, Jeff Round, Martin Offringa, Petros Pechlivanoglou, Petros Pechlivanoglou, Eleanor Pullenayegum, Eleanor Pullenayegum, David Rios, Marie Christine Auclair, Kelly Kim, Lise Bourrier, Lauren Dawson, Kamary Coriolano Dasilva, Pamela Marples, Rick Watts, Jennifer Thull-Freedman, Patrick Mcgrath

Paediatrics Publications

Background: Patients and their families often have preferences for medical care that relate to wider considerations beyond the clinical effectiveness of the proposed interventions. Traditionally, these preferences have not been adequately considered in research. Research questions where patients and families have strong preferences may not be appropriate for traditional randomized controlled trials (RCTs) due to threats to internal and external validity, as there may be high levels of drop-out and non-adherence or recruitment of a sample that is not representative of the treatment population. Several preference-informed designs have been developed to address problems with traditional RCTs, but these designs have …

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Canadian Urological Association Guideline: Management Of Ureteral Calculi - Abridged Version, Jason Y. Lee, Sero Andonian, Naeem Bhojani, Jennifer Bjazevic, Ben H. Chew, Shubha De, Hazem Elmansy, Andrea G. Lantz-Powers, Kenneth T. Pace, Trevor D. Schuler, Rajiv K. Singal, Peter Wang, Michael Ordon Dec 2021

Canadian Urological Association Guideline: Management Of Ureteral Calculi - Abridged Version, Jason Y. Lee, Sero Andonian, Naeem Bhojani, Jennifer Bjazevic, Ben H. Chew, Shubha De, Hazem Elmansy, Andrea G. Lantz-Powers, Kenneth T. Pace, Trevor D. Schuler, Rajiv K. Singal, Peter Wang, Michael Ordon

Paediatrics Publications

No abstract provided.

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Childhood-Onset Dystonia-Causing Kmt2b Variants Result In A Distinctive Genomic Hypermethylation Profile, Andrea Ciolfi, Aidin Foroutan, Alessandro Capuano, Lucia Pedace, Lorena Travaglini, Simone Pizzi, Marco Andreani, Evelina Miele, Federica Invernizzi, Chiara Reale, Celeste Panteghini, Maria Iascone, Marcello Niceta, Ralitza H. Gavrilova, Laura Schultz-Rogers, Emanuele Agolini, Maria Francesca Bedeschi, Paolo Prontera, Matteo Garibaldi, Serena Galosi, Vincenzo Leuzzi, Paola Soliveri, Rory J. Olson, Giovanna S. Zorzi, Barbara M. Garavaglia, Marco Tartaglia Dec 2021

Childhood-Onset Dystonia-Causing Kmt2b Variants Result In A Distinctive Genomic Hypermethylation Profile, Andrea Ciolfi, Aidin Foroutan, Alessandro Capuano, Lucia Pedace, Lorena Travaglini, Simone Pizzi, Marco Andreani, Evelina Miele, Federica Invernizzi, Chiara Reale, Celeste Panteghini, Maria Iascone, Marcello Niceta, Ralitza H. Gavrilova, Laura Schultz-Rogers, Emanuele Agolini, Maria Francesca Bedeschi, Paolo Prontera, Matteo Garibaldi, Serena Galosi, Vincenzo Leuzzi, Paola Soliveri, Rory J. Olson, Giovanna S. Zorzi, Barbara M. Garavaglia, Marco Tartaglia

Paediatrics Publications

Background: Dystonia is a clinically and genetically heterogeneous movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements and/or postures. Heterozygous variants in lysine methyltransferase 2B (KMT2B), encoding a histone H3 methyltransferase, have been associated with a childhood-onset, progressive and complex form of dystonia (dystonia 28, DYT28). Since 2016, more than one hundred rare KMT2B variants have been reported, including frameshift, nonsense, splice site, missense and other in-frame changes, many having an uncertain clinical impact. Results: We characterize the genome-wide peripheral blood DNA methylation profiles of a cohort of 18 patients with pathogenic and unclassified KMT2B …

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Choosing Wisely Canada – Pediatric Otolaryngology Recommendations, Mitchell Mcdonough, Kalpesh Hathi, Gerard Corsten, Christopher J. Chin, Paolo Campisi, Jonathan Cavanagh, Neil Chadha, M. Elise Graham, Murad Husein, Liane B. Johnson, Jodi Jones, Bruce Korman, John Manoukian, Lily H.P. Nguyen, Doron D. Sommer, Julie Strychowsky, Trina Uwiera, Warren Yunker, Paul Hong Dec 2021

Choosing Wisely Canada – Pediatric Otolaryngology Recommendations, Mitchell Mcdonough, Kalpesh Hathi, Gerard Corsten, Christopher J. Chin, Paolo Campisi, Jonathan Cavanagh, Neil Chadha, M. Elise Graham, Murad Husein, Liane B. Johnson, Jodi Jones, Bruce Korman, John Manoukian, Lily H.P. Nguyen, Doron D. Sommer, Julie Strychowsky, Trina Uwiera, Warren Yunker, Paul Hong

Paediatrics Publications

The Choosing Wisely Canada campaign raises awareness amongst physicians and patients regarding unnecessary or inappropriate tests and treatments. Using an online survey, members of the Pediatric Otolaryngology Subspecialty Group within the Canadian Society of Otolaryngology – Head & Neck Surgery developed a list of nine evidence based recommendations to help physicians and patients make treatment decisions regarding common pediatric otolaryngology presentations: (1) Don’t routinely order a plain film x-ray in the evaluation of nasal fractures; (2) Don’t order imaging to distinguish acute bacterial sinusitis from an upper respiratory infection; (3) Don’t place tympanostomy tubes in most children for a single …

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Correction To: A Novel Preference-Informed Complementary Trial (Pict) Design For Clinical Trial Research Influenced By Strong Patient Preferences (Trials, (2021), 22, 1, (206), 10.1186/S13063-021-05164-1), Samina Ali, Gareth Hopkin, Naveen Poonai, Lawrence Richer, Maryna Yaskina, Anna Heath, Terry Paul Klassen, Chris Mccabe Dec 2021

Correction To: A Novel Preference-Informed Complementary Trial (Pict) Design For Clinical Trial Research Influenced By Strong Patient Preferences (Trials, (2021), 22, 1, (206), 10.1186/S13063-021-05164-1), Samina Ali, Gareth Hopkin, Naveen Poonai, Lawrence Richer, Maryna Yaskina, Anna Heath, Terry Paul Klassen, Chris Mccabe

Paediatrics Publications

Following the publication of the original article [1], we were notified that the Acknowledgements section needs to accommodate additional collaborators. The original article has been corrected.

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Differential And Synergistic Effects Of Low Birth Weight And Western Diet On Skeletal Muscle Vasculature, Mitochondrial Lipid Metabolism And Insulin Signaling In Male Guinea Pigs, Kristyn Dunlop, Ousseynou Sarr, Nicole Stachura, Lin Zhao, Karen Nygard, Jennifer A. Thompson, Jennifer Hadway, Bryan S. Richardson, Yves Bureau, Nica Borradaile, Ting Yim Lee, Timothy R.H. Regnault Dec 2021

Differential And Synergistic Effects Of Low Birth Weight And Western Diet On Skeletal Muscle Vasculature, Mitochondrial Lipid Metabolism And Insulin Signaling In Male Guinea Pigs, Kristyn Dunlop, Ousseynou Sarr, Nicole Stachura, Lin Zhao, Karen Nygard, Jennifer A. Thompson, Jennifer Hadway, Bryan S. Richardson, Yves Bureau, Nica Borradaile, Ting Yim Lee, Timothy R.H. Regnault

Paediatrics Publications

Low birth weight (LBW) offspring are at increased risk for developing insulin resistance, a key precursor in metabolic syndrome and type 2 diabetes mellitus. Altered skeletal muscle vasculature, extracellular matrix, amino acid and mitochondrial lipid metabolism, and insulin signaling are implicated in this pathogenesis. Using uteroplacental insufficiency (UPI) to induce intrauterine growth restriction (IUGR) and LBW in the guinea pig, we investigated the relationship between UPI-induced IUGR/LBW and later life skeletal muscle arteriole density, fibrosis, amino acid and mitochondrial lipid metabolism, markers of insulin signaling and glucose uptake, and how a postnatal high-fat, high-sugar “Western” diet (WD) modulates these changes. …

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Discovery Of An Exosite On The Socs2-Sh2 Domain That Enhances Sh2 Binding To Phosphorylated Ligands, Edmond M. Linossi, Kunlun Li, Gianluca Veggiani, Cyrus Tan, Farhad Dehkhoda, Colin Hockings, Dale J. Calleja, Narelle Keating, Rebecca Siu, Monika Wojtera, Murad Husein Dec 2021

Discovery Of An Exosite On The Socs2-Sh2 Domain That Enhances Sh2 Binding To Phosphorylated Ligands, Edmond M. Linossi, Kunlun Li, Gianluca Veggiani, Cyrus Tan, Farhad Dehkhoda, Colin Hockings, Dale J. Calleja, Narelle Keating, Rebecca Siu, Monika Wojtera, Murad Husein

Paediatrics Publications

Suppressor of cytokine signaling (SOCS)2 protein is a key negative regulator of the growth hormone (GH) and Janus kinase (JAK)-Signal Transducers and Activators of Transcription (STAT) signaling cascade. The central SOCS2-Src homology 2 (SH2) domain is characteristic of the SOCS family proteins and is an important module that facilitates recognition of targets bearing phosphorylated tyrosine (pTyr) residues. Here we identify an exosite on the SOCS2-SH2 domain which, when bound to a non-phosphorylated peptide (F3), enhances SH2 affinity for canonical phosphorylated ligands. Solution of the SOCS2/F3 crystal structure reveals F3 as an α-helix which binds on the opposite side of the …

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Disentangling Language Status And Country-Of-Origin Explanations Of The Bilingual Advantage In Preschoolers, Isu Cho, Jewan Park, Hyun Joo Song, J. Bruce Morton Dec 2021

Disentangling Language Status And Country-Of-Origin Explanations Of The Bilingual Advantage In Preschoolers, Isu Cho, Jewan Park, Hyun Joo Song, J. Bruce Morton

Paediatrics Publications

Bilingual preschoolers from East Asia outperform monolingual preschoolers from North America or Europe in executive functioning tasks, which has been interpreted as evidence of a bilingual advantage in executive functioning. This study tested whether these differences actually reflect country-of-origin effects given that East Asian preschoolers frequently outperform North American or European children in executive functioning tasks. Consistent with previous findings, Korean–English bilingual preschoolers made fewer errors in an age-appropriate executive functioning task than did English monolingual children in Canada. However, Korean–English bilingual preschoolers performed comparably to Korean monolingual preschoolers in Korea. Differences between Korean and Canadian children's executive functioning were …

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Dna Methylation Epi-Signature Is Associated With Two Molecularly And Phenotypically Distinct Clinical Subtypes Of Phelan-Mcdermid Syndrome, L. C. Schenkel, E. Aref-Eshghi, K. Rooney, J. Kerkhof, M. A. Levy, H. Mcconkey, R. C. Rogers, K. Phelan, S. M. Sarasua, L. Jain, R. Pauly, L. Boccuto, B. Dupont, G. Cappuccio, N. Brunetti-Pierri, C. E. Schwartz, B. Sadikovic Dec 2021

Dna Methylation Epi-Signature Is Associated With Two Molecularly And Phenotypically Distinct Clinical Subtypes Of Phelan-Mcdermid Syndrome, L. C. Schenkel, E. Aref-Eshghi, K. Rooney, J. Kerkhof, M. A. Levy, H. Mcconkey, R. C. Rogers, K. Phelan, S. M. Sarasua, L. Jain, R. Pauly, L. Boccuto, B. Dupont, G. Cappuccio, N. Brunetti-Pierri, C. E. Schwartz, B. Sadikovic

Paediatrics Publications

Background: Phelan-McDermid syndrome is characterized by a range of neurodevelopmental phenotypes with incomplete penetrance and variable expressivity. It is caused by a variable size and breakpoint microdeletions in the distal long arm of chromosome 22, referred to as 22q13.3 deletion syndrome, including the SHANK3 gene. Genetic defects in a growing number of neurodevelopmental genes have been shown to cause genome-wide disruptions in epigenomic profiles referred to as epi-signatures in affected individuals. Results: In this study we assessed genome-wide DNA methylation profiles in a cohort of 22 individuals with Phelan-McDermid syndrome, including 11 individuals with large (2 to 5.8 Mb) 22q13.3 …

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Dress Induced By Amoxicillin-Clavulanate In Two Pediatric Patients Confirmed By Lymphocyte Toxicity Assay, Arun Dhir, Hasandeep Kular, Abdelbaset A. Elzagallaai, Bruce Carleton, Michael J. Rieder, Raymond Mak, Tiffany Wong Dec 2021

Dress Induced By Amoxicillin-Clavulanate In Two Pediatric Patients Confirmed By Lymphocyte Toxicity Assay, Arun Dhir, Hasandeep Kular, Abdelbaset A. Elzagallaai, Bruce Carleton, Michael J. Rieder, Raymond Mak, Tiffany Wong

Paediatrics Publications

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare but serious delayed hypersensitivity reaction that can be caused by antibiotic exposure. The reaction typically develops in 2 to 6 weeks. The pathophysiology is thought to involve toxic drug metabolites acting as a hapten, triggering a systemic response. The diagnosis is made clinically but can be confirmed using assays such as the lymphocyte toxicity assay (LTA), which correlates cell death upon exposure to drug metabolites with susceptibility to hypersensitivity reactions. Case presentations: Case 1 involves a previously healthy 11-month-old male with first exposure to amoxicillin-clavulanate, prescribed for seven …

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Dynamic Tracking Of Microvascular Hemoglobin Content For Continuous Perfusion Monitoring In The Intensive Care Unit: Pilot Feasibility Study, Asher A. Mendelson, Ajay Rajaram, Daniel Bainbridge, Keith St Lawrence, Tracey Bentall, Michael Sharpe, Mamadou Diop, Christopher G. Ellis Dec 2021

Dynamic Tracking Of Microvascular Hemoglobin Content For Continuous Perfusion Monitoring In The Intensive Care Unit: Pilot Feasibility Study, Asher A. Mendelson, Ajay Rajaram, Daniel Bainbridge, Keith St Lawrence, Tracey Bentall, Michael Sharpe, Mamadou Diop, Christopher G. Ellis

Paediatrics Publications

Purpose: There is a need for bedside methods to monitor oxygen delivery in the microcirculation. Near-infrared spectroscopy commonly measures tissue oxygen saturation, but does not reflect the time-dependent variability of microvascular hemoglobin content (MHC) that attempts to match oxygen supply with demand. The objective of this study is to determine the feasibility of MHC monitoring in critically ill patients using high-resolution near-infrared spectroscopy to assess perfusion in the peripheral microcirculation. Methods: Prospective observational cohort of 36 patients admitted within 48 h at a tertiary intensive care unit. Perfusion was measured on the quadriceps, biceps, and/or deltoid, using the temporal change …

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Ectopic Mineralisation Of The Mandibular Symphysis In Ent1 Knockout Mice: A Model Of Dystrophic Calcification, Dale E. Fournier, Kim L. Beaucage, Ryan J. Beach, Patti K. Kiser, Cheryle A. Séguin, S. Jeffrey Dixon Dec 2021

Ectopic Mineralisation Of The Mandibular Symphysis In Ent1 Knockout Mice: A Model Of Dystrophic Calcification, Dale E. Fournier, Kim L. Beaucage, Ryan J. Beach, Patti K. Kiser, Cheryle A. Séguin, S. Jeffrey Dixon

Paediatrics Publications

Equilibrative nucleoside transporter 1 (ENT1) transfers nucleosides, such as adenosine, across plasma membranes. We reported previously that mice lacking ENT1 (ENT1−/−) exhibit progressive ectopic calcification of spinal tissues—a phenotype resembling diffuse idiopathic skeletal hyperostosis (DISH) in humans. Our objective was to investigate potential calcification of orofacial tissues in ENT1−/− mice. Heads of wild-type mice and ENT1−/− mice from 3 to 17 months were evaluated using microcomputed tomography (μCT). Some heads were decalcified and processed for histological assessment. Other heads were examined using energy dispersive X-ray spectroscopy and micro X-ray diffraction. Using μCT, ENT1−/− mice showed extensive radiopaque lesions within the …

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Efficacy Of Mobile Phone Use On Adherence To Nevirapine Prophylaxis And Retention In Care Among The Hiv-Exposed Infants In Prevention Of Mother To Child Transmission Of Hiv: A Randomized Controlled Trial, Lilian M.N. Kebaya, Dalton Wamalwa, Nyambura Kariuki, Bashir Admani, Philip Ayieko, Ruth Nduati Dec 2021

Efficacy Of Mobile Phone Use On Adherence To Nevirapine Prophylaxis And Retention In Care Among The Hiv-Exposed Infants In Prevention Of Mother To Child Transmission Of Hiv: A Randomized Controlled Trial, Lilian M.N. Kebaya, Dalton Wamalwa, Nyambura Kariuki, Bashir Admani, Philip Ayieko, Ruth Nduati

Paediatrics Publications

Background: HIV is a major contributor to infant mortality. A significant gap remains between the uptake of infant and maternal antiretroviral regimens and only a minority of HIV-exposed infants receives prophylaxis and safe infant feeding. Losses to follow-up of HIV-exposed infants are associated with shortcomings of facility-based PMTCT models with weak community support of linkages. Use of mobile phones offers an opportunity for improving care and promoting retention assessed by timely attendance of scheduled appointments for the mother-baby pairs and achievement of an HIV-free generation. The objective of this study was to compare self-reported adherence to infant Nevirapine (NVP) prophylaxis …

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Exploring Sensory Phenotypes In Autism Spectrum Disorder, Nichole E. Scheerer, Kristina Curcin, Bobby Stojanoski, Evdokia Anagnostou, Rob Nicolson, Elizabeth Kelley, Stelios Georgiades, Xudong Liu Dec 2021

Exploring Sensory Phenotypes In Autism Spectrum Disorder, Nichole E. Scheerer, Kristina Curcin, Bobby Stojanoski, Evdokia Anagnostou, Rob Nicolson, Elizabeth Kelley, Stelios Georgiades, Xudong Liu

Paediatrics Publications

Background: Atypical reactions to the sensory environment are often reported in autistic individuals, with a high degree of variability across the sensory modalities. These sensory differences have been shown to promote challenging behaviours and distress in autistic individuals and are predictive of other functions including motor, social, and cognitive abilities. Preliminary research suggests that specific sensory differences may cluster together within individuals creating discrete sensory phenotypes. However, the manner in which these sensory differences cluster, and whether the resulting phenotypes are associated with specific cognitive and social challenges is unclear. Methods: Short sensory profile data from 599 autistic children and …

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Factors Associated With Parents’ Experiences Using A Knowledge Translation Tool For Vaccination Pain Management: A Qualitative Study, Nicole E. Mackenzie, Perri R. Tutelman, Christine T. Chambers, Jennifer A. Parker, Noni E. Macdonald, C. Meghan Mcmurtry, Pierre Pluye, Vera Granikov Dec 2021

Factors Associated With Parents’ Experiences Using A Knowledge Translation Tool For Vaccination Pain Management: A Qualitative Study, Nicole E. Mackenzie, Perri R. Tutelman, Christine T. Chambers, Jennifer A. Parker, Noni E. Macdonald, C. Meghan Mcmurtry, Pierre Pluye, Vera Granikov

Paediatrics Publications

Background: Vaccination is a common painful procedure for children. Parents’ concern regarding vaccination pain is a significant driver of vaccine hesitancy. Despite the wealth of evidence-based practices available for managing vaccination pain, parents lack knowledge of, and access to, these strategies. Knowledge translation (KT) tools can communicate evidence-based information to parents, however little is known about what factors influence parents’ use of these tools. A two-page, electronic KT tool on psychological, physical, and pharmacological vaccination pain management strategies for children, was shared with parents as part of a larger mixed methods study, using explanatory sequential design, exploring factors related to …

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Feasibility Of Implementation Of Card™ For School-Based Immunizations In Calgary, Alberta: A Cluster Trial, Anna Taddio, Joanne Coldham, Charlotte Logeman, C. Meghan Mcmurtry, Cheri Little, Tracy Samborn, Lucie M. Bucci, Noni E. Macdonald, Vibhuti Khan, Guang Liang Dec 2021

Feasibility Of Implementation Of Card™ For School-Based Immunizations In Calgary, Alberta: A Cluster Trial, Anna Taddio, Joanne Coldham, Charlotte Logeman, C. Meghan Mcmurtry, Cheri Little, Tracy Samborn, Lucie M. Bucci, Noni E. Macdonald, Vibhuti Khan, Guang Liang

Paediatrics Publications

Background: Negative experiences with school-based immunizations can contribute to vaccine hesitancy in youth and adulthood. We developed an evidence-based, multifaceted and customizable intervention to improve the immunization experience at school called the CARD™ (C-Comfort, A-Ask, R-Relax, D-Distract) system. We evaluated the feasibility of CARD™ implementation for school-based immunizations in Calgary, Canada. Methods: In a mixed methods study, two Community Health Centres providing immunization services, including 5 schools each with grade 9 students (aged approximately 14 years), were randomized to CARD™ or control (usual care). In the CARD™ group, public health staff and students were educated about coping strategies prior to …

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Haploinsufficiency Of Sf3b2 Causes Craniofacial Microsomia, Andrew T. Timberlake, Casey Griffin, Carrie L. Heike, Anne V. Hing, Michael L. Cunningham, David Chitayat, Mark R. Davis, Soghra J. Doust Dec 2021

Haploinsufficiency Of Sf3b2 Causes Craniofacial Microsomia, Andrew T. Timberlake, Casey Griffin, Carrie L. Heike, Anne V. Hing, Michael L. Cunningham, David Chitayat, Mark R. Davis, Soghra J. Doust

Paediatrics Publications

Craniofacial microsomia (CFM) is the second most common congenital facial anomaly, yet its genetic etiology remains unknown. We perform whole-exome or genome sequencing of 146 kindreds with sporadic (n = 138) or familial (n = 8) CFM, identifying a highly significant burden of loss of function variants in SF3B2 (P = 3.8 × 10−10), a component of the U2 small nuclear ribonucleoprotein complex, in probands. We describe twenty individuals from seven kindreds harboring de novo or transmitted haploinsufficient variants in SF3B2. Probands display mandibular hypoplasia, microtia, facial and preauricular tags, epibulbar dermoids, lateral oral clefts in addition to skeletal and …

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High Infliximab Trough Concentrations Are Associated With Sustained Histologic Remission In Inflammatory Bowel Disease: A Prospective Cohort Study, Aze Wilson, Bethany Choi, Michael Sey, Terry Ponich, Melanie Beaton, Richard B. Kim Dec 2021

High Infliximab Trough Concentrations Are Associated With Sustained Histologic Remission In Inflammatory Bowel Disease: A Prospective Cohort Study, Aze Wilson, Bethany Choi, Michael Sey, Terry Ponich, Melanie Beaton, Richard B. Kim

Paediatrics Publications

Background: The threshold concentration of infliximab during maintenance therapy has not been well-defined in relation to histologic remission. The aim of the study is to dentify the maintenance-phase infliximab concentration associated with histologic remission in inflammatory bowel disease patients (IBD). Methods: A prospective cohort study was carried out in 104 IBD patients seen at a tertiary care centre in London, Canada. Infliximab trough concentrations were collected during the maintenance phase of treatment and compared between participants with and without evidence of histologic remission. Participants were additionally evaluated for sustained histologic remission, and relapse to active disease. Results: Participants in histologic …

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How High? Trends In Cannabis Use Prior To First Admission To Inpatient Psychiatry In Ontario, Canada, Between 2007 And 2017, Taylor Mcguckin, Mark A. Ferro, David Hammond, Shannon Stewart, Bridget Maloney-Hall, Nawaf Madi, Amy Porath, Christopher M. Perlman Dec 2021

How High? Trends In Cannabis Use Prior To First Admission To Inpatient Psychiatry In Ontario, Canada, Between 2007 And 2017, Taylor Mcguckin, Mark A. Ferro, David Hammond, Shannon Stewart, Bridget Maloney-Hall, Nawaf Madi, Amy Porath, Christopher M. Perlman

Paediatrics Publications

Objectives: To examine the trends in cannabis use within 30 days of first admission to inpatient psychiatry in Ontario, Canada, between 2007 and 2017, and the characteristics of persons reporting cannabis use. Methods: A retrospective cross-sectional analysis was conducted for first-time admissions to nonforensic inpatient psychiatric beds in Ontario, Canada, between January 1, 2007, and December 31, 2017, using data from the Ontario Mental Health Reporting System (N = 81,809). Results: Across all years, 20.1% of patients reported cannabis use within 30 days of first admission. Use increased from 16.7% in 2007 to 25.9% in 2017, and the proportion with …

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Incidental Findings From Cancer Next Generation Sequencing Panels, Nika Maani, Karen Panabaker, Jeanna M. Mccuaig, Kathleen Buckley, Kara Semotiuk, Kirsten M. Farncombe, Peter Ainsworth, Seema Panchal, Bekim Sadikovic, Susan Randall Armel, Hanxin Lin, Raymond H. Kim Dec 2021

Incidental Findings From Cancer Next Generation Sequencing Panels, Nika Maani, Karen Panabaker, Jeanna M. Mccuaig, Kathleen Buckley, Kara Semotiuk, Kirsten M. Farncombe, Peter Ainsworth, Seema Panchal, Bekim Sadikovic, Susan Randall Armel, Hanxin Lin, Raymond H. Kim

Paediatrics Publications

Next-generation sequencing (NGS) technologies have facilitated multi-gene panel (MGP) testing to detect germline DNA variants in hereditary cancer patients. This sensitive technique can uncover unexpected, non-germline incidental findings indicative of mosaicism, clonal hematopoiesis (CH), or hematologic malignancies. A retrospective chart review was conducted to identify cases of incidental findings from NGS-MGP testing. Inclusion criteria included: 1) multiple pathogenic variants in the same patient; 2) pathogenic variants at a low allele fraction; and/or 3) the presence of pathogenic variants not consistent with family history. Secondary tissue analysis, complete blood count (CBC) and medical record review were conducted to further delineate the …

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Lentivirus-Mediated Gene Therapy For Fabry Disease, Aneal Khan, Dwayne L. Barber, Ju Huang, C. Anthony Rupar, Jack W. Rip, Christiane Auray-Blais, Michel Boutin, Pamela O’Hoski, Kristy Gargulak, William M. Mckillop, Graeme Fraser, Syed Wasim, Kaye Lemoine, Shelly Jelinski, Ahsan Chaudhry, Nicole Prokopishyn, Chantal F. Morel, Stephen Couban, Peter R. Duggan, Daniel H. Fowler, Armand Keating, Michael L. West, Ronan Foley, Jeffrey A. Medin Dec 2021

Lentivirus-Mediated Gene Therapy For Fabry Disease, Aneal Khan, Dwayne L. Barber, Ju Huang, C. Anthony Rupar, Jack W. Rip, Christiane Auray-Blais, Michel Boutin, Pamela O’Hoski, Kristy Gargulak, William M. Mckillop, Graeme Fraser, Syed Wasim, Kaye Lemoine, Shelly Jelinski, Ahsan Chaudhry, Nicole Prokopishyn, Chantal F. Morel, Stephen Couban, Peter R. Duggan, Daniel H. Fowler, Armand Keating, Michael L. West, Ronan Foley, Jeffrey A. Medin

Paediatrics Publications

Enzyme and chaperone therapies are used to treat Fabry disease. Such treatments are expensive and require intrusive biweekly infusions; they are also not particularly efficacious. In this pilot, single-arm study (NCT02800070), five adult males with Type 1 (classical) phenotype Fabry disease were infused with autologous lentivirus-transduced, CD34+-selected, hematopoietic stem/progenitor cells engineered to express alpha-galactosidase A (α-gal A). Safety and toxicity are the primary endpoints. The non-myeloablative preparative regimen consisted of intravenous melphalan. No serious adverse events (AEs) are attributable to the investigational product. All patients produced α-gal A to near normal levels within one week. Vector is detected in peripheral …

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