Pediatrics Commons^™

Human Milk Polyunsaturated Fatty Acids Are Related To Neurodevelopmental, Anthropometric, And Allergic Outcomes In Early Life: A Systematic Review, Saori Mitguard, Olivia Doucette, John Miklavcic

Food Science Faculty Articles and Research

Polyunsaturated fatty acids are critically important for newborn nutrition and in the trajectory of growth and developmental processes throughout early life. This systematic review (PROSPERO ID: CRD42023400059) critically analyzes literature pertaining to how omega-3 and omega-6 fatty acids in human milk are related to health outcomes in early life. Literature selected for the review were published between 2005 and 2020 and included assessments in healthy term children between 0 and 5 years of age. The studies reported the relation between human milk fatty acids docosahexaenoic acid (C22:6n-3, DHA), eicosapentaenoic acid (C20:5n-3, EPA), alpha-linolenic acid (C18:3n-3, ALA), arachidonic acid (C20:4n-6, AA), …

Prenatal Cocaine Exposure And Its Influence On Pediatric Epigenetic Clocks And Epigenetic Scores In Humans, Thiago Wendt Viola, Christina Danzer, Victor Mardini, Claudia Szobot, João Henrique Chrusciel, Laura Stertz, Joy M Schmitz, Consuelo Walss-Bass, Gabriel R Fries, Rodrigo Grassi-Oliveira

Faculty, Staff and Student Publications

The investigation of the effects of prenatal cocaine exposure (PCE) on offspring has been inconsistent, with few studies investigating biological outcomes in humans. We profiled genome-wide DNA methylation (DNAm) of umbilical cord blood (UCB) from newborns with (n = 35) and without (n = 47) PCE. We used DNAm data to (1) assess pediatric epigenetic clocks at birth and (2) to estimate epigenetic scores (ES) for lifetime disorders. We generated gestational epigenetic age estimates (DNAmGA) based on Knight and Bohlin epigenetic clocks. We also investigated the association between DNAmGA and UCB serum brain-derived neurotrophic factor (BDNF) levels. Considering the large-scale …

Talking To Your Child About Their First Heartbreak, Maggie Mckay, Jami Gross-Toalson

Parent-ish Podcast

Our first love can be a highly emotional experience and the same goes for first heartbreak. It’s never easy to see your child hurting, but you can ease their struggle by remaining open and available to listen. Learn how to support your child from Dr. Jami Gross-Toalson, pediatric psychologist at Children's Mercy Kansas City.

Click here to stream the podcast episode.

Molecular Mechanisms In Pathophysiology Of Mucopolysaccharidosis And Prospects For Innovative Therapy, Yasuhiko Ago, Estera Rintz, Krishna Sai Musini, Zhengyu Ma, Shunji Tomatsu

Department of Pediatrics Faculty Papers

Mucopolysaccharidoses (MPSs) are a group of inborn errors of the metabolism caused by a deficiency in the lysosomal enzymes required to break down molecules called glycosaminoglycans (GAGs). These GAGs accumulate over time in various tissues and disrupt multiple biological systems, including catabolism of other substances, autophagy, and mitochondrial function. These pathological changes ultimately increase oxidative stress and activate innate immunity and inflammation. We have described the pathophysiology of MPS and activated inflammation in this paper, starting with accumulating the primary storage materials, GAGs. At the initial stage of GAG accumulation, affected tissues/cells are reversibly affected but progress irreversibly to: (1) …

Modeling The Health Impact And Cost-Effectiveness Of A Combined Schoolgirl Hpv Vaccination And Cervical Cancer Screening Program In Guangdong Province, China, Yating Huang, Dantao Zhang, Lihua Yin, Jianguo Zhao, Zhifeng Li, Jing Lu, Xiaoming Zhang, Chenggang Wu, Wei Wu

Faculty, Staff and Student Publications

Low human papillomavirus (HPV) vaccine uptake is a key barrier to cervical cancer elimination. We aimed to evaluate the health impact and cost-effectiveness of introducing different HPV vaccines into immunization programs and scaling up the screening program in Guangdong. We used a dynamic compartmental model to estimate the impact of intervention strategies during 2023-2100. We implemented the incremental cost-effectiveness ratio (ICER) in costs per averted disability-adjusted life year (DALY) as an indicator to assess the effectiveness of the intervention. We used an age-standardized incidence of 4 cases per 100,000 women as the threshold for the elimination of cervical cancer. Compared …

Armc5 Controls The Degradation Of Most Pol Ii Subunits, And Armc5 Mutation Increases Neural Tube Defect Risks In Mice And Humans, Hongyu Luo, Linjiang Lao, Kit Sing Au, Hope Northrup, Xiao He, Diane Forget, Marie-Soleil Gauthier, Benoit Coulombe, Isabelle Bourdeau, Wei Shi, Lucia Gagliardi, Maria Candida Barisson Villares Fragoso, Junzheng Peng, Jiangping Wu

Faculty, Staff and Student Publications

BACKGROUND: Neural tube defects (NTDs) are caused by genetic and environmental factors. ARMC5 is part of a novel ubiquitin ligase specific for POLR2A, the largest subunit of RNA polymerase II (Pol II).

RESULTS: We find that ARMC5 knockout mice have increased incidence of NTDs, such as spina bifida and exencephaly. Surprisingly, the absence of ARMC5 causes the accumulation of not only POLR2A but also most of the other 11 Pol II subunits, indicating that the degradation of the whole Pol II complex is compromised. The enlarged Pol II pool does not lead to generalized Pol II stalling or a generalized …

The Challenges Of Implementing And Documenting Ottawa Ankle Rules In The Pediatric Emergency Department: A Retrospective Study, Emmalee M. Kugler, Michael Gillman, Olivia Toner

Cooper Rowan Medical Journal

INTRODUCTION
Acute ankle sprains account for nearly 2% of visits to the pediatric emergency department (PED). The Ottawa Ankle Rules (OAR) were developed as a safe and effective clinical decision-making tool for detecting the need for radiographs in adults with acute ankle pain. OAR state radiographs are required with at least one of the following:

1. Inability to bear weight immediately following the injury and for four steps in the ED 2. Bony tenderness at the posterior edge of the lateral or medial malleolus

OBJECTIVE
Few prospective cohort studies have attempted to assess OAR pediatric populations. This study investigates the …

Effect Of Maternal Adverse Childhood Experiences (Aces) And Maternal Resiliency On Early Childhood Outcomes On Vaccination Adherence And Emergency Department Visits, Leslie Abraham, Hsiao-Wei Banks, John P. Gaughan, Alla Kushnir

Cooper Rowan Medical Journal

Introduction: Adverse Childhood Experiences (ACEs) are traumatic events that occurred to an individual during ages 0-17 years. The 1998 Adverse Childhood Experiences (ACE) suggested higher ACE scores contributed to negative future health outcomes. The tool is now used to screen at-risk populations for the development of chronic health conditions. Protective factors, such as resilience, were later studied and found to offset the negative health impacts of traumatic childhood events. The 7Cs questionnaire was a tool developed to measure resilience in adolescents and demonstrated better health outcomes despite the presence of higher ACE scores. This pilot study evaluated whether higher …

Clarification Of Adverse Drug Reactions By A Pharmacovigilance Team Results In Increased Antibiotic Re-Prescribing At A Freestanding United States Children's Hospital., Keith Feldman, Sarah Suppes, Jennifer Goldman

Manuscripts, Articles, Book Chapters and Other Papers

Documentation of adverse drug reactions (ADRs) is a key factor in guiding future prescribing. However, incomplete documentation is common and often fails to distinguish implicated drugs as true allergies. This in turn leads to unnecessary avoidance of implicated drug classes and may result in sub-optimal prescribing. Pharmacovigilance (PV) programs utilize a systematic approach to clarify ADR documentation and are known to improve patient safety. Yet it remains unclear if PV alters prescribing. Or, if the existence of the ADR documentation itself continues to prompt avoidance of implicated drugs. To address this, our work presents a retrospective cohort study assessing if …

Sepsis Epidemiology In Australian And New Zealand Children (Sentinel): Protocol For A Multicountry Prospective Observational Study, Elliot Long, Meredith L. Borland, Shane George, Shefali Jani, Eunicia Tan, Jocelyn Neutze, Natalie Phillips, Amit Kochar, Simon Craig, Anna Lithgow, Arjun Rao, Stuart Dalziel, Ed Oakley, Stephen Hearps, Sonia Singh, Ben Gelbart, Sarah Mcnab, Fran Balamuth, Scott Weiss, Nathan Kuppermann, Amanda Williams, Franz E. Babl

Department of Pediatrics Faculty Papers

INTRODUCTION: Sepsis affects 25.2 million children per year globally and causes 3.4 million deaths, with an annual cost of hospitalisation in the USA of US$7.3 billion. Despite being common, severe and expensive, therapies and outcomes from sepsis have not substantially changed in decades. Variable case definitions, lack of a reference standard for diagnosis and broad spectrum of disease hamper efforts to evaluate therapies that may improve sepsis outcomes. This landscape analysis of community-acquired childhood sepsis in Australia and New Zealand will characterise the burden of disease, including incidence, severity, outcomes and cost. Sepsis diagnostic criteria and risk stratification tools will …

Impact Of Genetic And Non-Genetic Factors On Phenotypic Diversity In Nbas-Associated Disease, Nicole Hammann, Dominic Lenz, Ivo Baric, Ellen Crushell, Carlo Dionisi Vici, Felix Distelmaier, Francois Feillet, Peter Freisinger, Maja Hempel, Anna L. Khoreva, Martin W. Laass, Yves Lacassie, Elke Lainka, Catherine Larson-Nath, Zhongdie Li, Patryk Lipiński, Eberhard Lurz, André Mégarbané, Susana Nobre, Giorgia Olivieri, Bianca Peters, Paolo Prontera, Lea D. Schlieben, Christine M. Seroogy, Cristina Sobacchi, Shigeru Suzuki, Christel Tran, Jerry Vockley

School of Medicine Faculty Publications

Biallelic pathogenic variants in neuroblastoma-amplified sequence (NBAS) cause a pleiotropic multisystem disorder. Three clinical subgroups have been defined correlating with the localisation of pathogenic variants in the NBAS gene; variants affecting the C-terminal region of NBAS result in SOPH syndrome (short stature, optic atrophy, Pelger-Huët anomaly), variants affecting the Sec 39 domain are associated with infantile liver failure syndrome type 2 (ILFS2) and variants affecting the ß-propeller domain give rise to a combined phenotype. However, there is still unexplained phenotypic diversity across the three subgroups, challenging the current concept of genotype-phenotype correlations in NBAS-associated disease. Therefore, besides examining the genetic …

Clinical Rarity: Simultaneous Choroid Plexus Papilloma And Extraventricular Neurocytoma Presenting As Intraventricular Hemorrhage In An Adolescent, Kaleb Derouen, Kierany Shelvin, Wesley Shoap, Randall Craver, Jerome Volk, Oritsejolomi A. Roberts

School of Medicine Faculty Publications

We present a patient with an intraventricular hemorrhage. Imaging identified a left atrial intraventricular mass and a vague adjacent second periventricular cystic lesion. A guided trans-sulcal approach via a left parietal craniotomy resulted in a gross total resection of both lesions. These represented two distinct lesions, the periventricular cystic lesion was an extraventricular neurocytoma (EVN) and a World Health Organization grade 1 choroid plexus papilloma (CPP). The neurocytoma required methylation studies for confirmatory diagnosis. The patient had an uneventful recovery with a normal neurological exam at 12-weeks. This documents the occurrence of two distinct central nervous system tumors, a CPP …

Barriers To Care For Children With Cerebral Palsy In The Rural State Of Maine: A Mixed-Methods Study, Rayne L. Whitten, Anya K. Cutler, Alexa K. Craig

Journal of Maine Medical Center

Introduction: Guidelines on orthopedic hip surveillance in children with cerebral palsy have been published to minimize the effects of cerebral palsy and maximize quality of life. Researchers aimed to identify barriers to cerebral palsy care and to assess adherence to national hip-surveillance guidelines among a small subset of children with cerebral palsy who live in a rural state.

Methods: Parents of children with cerebral palsy were interviewed, and thematic analyses were performed on the recorded transcripts. Patient-specific data about imaging and demographics were manually extracted from the electronic health record to perform a mixed-methods analysis.

Results: Twenty-one parents were interviewed, …

Development Of Right Ventricular Electromechanical Dyssynchrony Following Surgical Repair Of Tetralogy Of Fallot In Infants., Andrew W. Mccrary, Sydney D. Collins, Zebulon Z. Spector, P Andrea Kropf, Piers C A Barker, Joseph Kisslo, Daniel Forsha

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: In adolescents and adults with tetralogy of Fallot (TOF), right ventricle (RV) electromechanical dyssynchrony (EMD) due to right bundle branch block (RBBB) is associated with reduced exercise capacity and RV dysfunction. While the development of RBBB following surgical repair of tetralogy of Fallot (rTOF) is a frequent sequela, it is not known whether EMD is present in every patient immediately following rTOF. The specific timing of the onset of RBBB following rTOF therefore provides an opportunity to assess whether acute RBBB is associated with the simultaneous acquisition of EMD.

METHODS: Transthoracic echocardiography with speckle tracking analysis for RV global …

Synthetic Oct-A Blood Vessel Maps Using Fundus Images And Generative Adversarial Networks, Ashanul Haque, Mohd Wajid Ali Khan, Khalaf M Alenezi, Raoudha Soury, Muhammad S Khan, Shahzaib Ahamad, Md Mushtaque, Dinesh Gupta

Faculty, Staff and Student Publications

The design and development of new small-molecule glycation inhibitors are essential for preventing various chronic diseases, including diabetes mellitus, immunoinflammation, cardiovascular, and neurodegenerative diseases. 4-Thiazolidinone or thiazolidine-4-one is a well-known heterocyclic compound with the potential to inhibit the formation of advanced glycation end products. In the present work, we report the synthesis and characterization of four new 5-arylidene 3-cyclopropyl-2-(phenylimino)thiazolidin-4-one (1–4) compounds and their human serum albumin glycation inhibitory activity. One of the compounds 5-(2H-1,3-benzodioxol-5-ylmethylidene)-3-cyclopropyl-2-(phenylimino)-1,3-thiazolidin-4-one (3) showed potent inhibition in the synthesis of initial, intermediary, and final products of glycation reactions. Besides, conformational …

Achieving Reliable Patient Reported Outcomes Collection To Measure Health Care Improvement In A Learning Health Network: Lessons From Pediatric Rheumatology Care And Outcomes Improvement Network., Nancy Pan, Esi M. Morgan, Meghan Ryan, Beth Gottlieb, Julia G. Harris, Tzielan Lee, Y Ingrid Goh

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Data from the Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) registry suggests that reliable collection of patient-reported outcomes (PROs) varies across sites. The objective of this study was to better understand the practices of collecting PROs at PR-COIN sites.

METHODS: A REDCap survey was sent to the lead representative for each PR-COIN site. Registry data were analyzed to better understand the completion rates of PROs. Interviews of physician leaders of high performing sites were conducted by videoconference, audiotranscribed and themes were summarized. Quantitative data were analyzed using descriptive statistics and qualitative data were thematically analyzed.

RESULTS: All 23 …

Impact Of Elexacaftor/Tezacaftor/Ivacaftor On Lipid And Fat‐Soluble Vitamin Levels And Association With Body Mass Index, Tanvi Patel, Kimberly Mcbennett, Senthilkumar Sankararaman, Teresa Schindler, Nori Mercuri Minich, Katherine Kutney

Faculty Scholarship

Introduction:Cystic fibrosis transmembrane conductance regulator (CFTR) modulators improve gastrointestinal absorption of nutrients and may result in changes in body mass index (BMI), serum lipids, and fat-soluble vitamin levels. We hypothesized that serum lipids and vitamin levels would increase with CFTR modulator therapy and that greater increase in lipids and vitamin levels would be related to greater increase in BMI. Methods:A retrospective study was performed to evaluate the impact of elexacaftor/tezacaftor/ivacaftor (ETI) on nutritional parameters, serum lipids, and fat-soluble vitamin levels. Pre-ETI values (<2 years prior) and post-ETI values (>1 month after) were compared. Linear regression was used to evaluate whether change in BMI is associated …

Postprandial Glucose Variability Following Typical Meals In Youth Living With Type 1 Diabetes., Susana R. Patton, Simon Bergford, Jennifer L. Sherr, Robin L. Gal, Peter Calhoun, Mark A. Clements, Michael C. Riddell, Corby K. Martin

Manuscripts, Articles, Book Chapters and Other Papers

We explored the association between macronutrient intake and postprandial glucose variability in a large sample of youth living with T1D and consuming free-living meals. In the Type 1 Diabetes Exercise Initiative Pediatric (T1DEXIP) Study, youth took photographs before and after their meals on 3 days during a 10 day observation period. We used the remote food photograph method to obtain the macronutrient content of youth's meals. We also collected physical activity, continuous glucose monitoring, and insulin use data. We measured glycemic variability using standard deviation (SD) and coefficient of variation (CV) of glucose for up to 3 h after meals. …

Factors Associated With Blood Mercury Concentrations And Their Interactions With Three Glutathione S-Transferase Genes (Gstt1, Gstm1, And Gstp1): An Exposure Assessment Study Of Typically Developing Jamaican Children, Sheikh Farzana Zaman, Maureen Samms-Vaughan, Sepideh Saroukhani, Jan Bressler, Manouchehr Hessabi, Megan L Grove, Sydonnie Shakespeare Pellington, Katherine A Loveland, Mohammad H Rahbar

Faculty, Staff and Student Publications

BACKGROUND: Jamaican soil is abundant in heavy metals including mercury (Hg). Due to availability and ease of access, fish is a traditional dietary component in Jamaica and a significant source of Hg exposure. Mercury is a xenobiotic and known neuro-toxicant that affects children's neurodevelopment. Human glutathione S-transferase (GST) genes, including GSTT1, GSTM1, and GSTP1, affect Hg conjugation and elimination mechanisms.

METHODS: In this exposure assessment study we used data from 375 typically developing (TD) 2-8-year-old Jamaican children to explore the association between environmental Hg exposure, GST genes, and their interaction effects on blood Hg concentrations (BHgCs). We used multivariable general …

Infant Growth And Body Composition From Birth To 24 Months: Are Infants Developing The Same?, Shane A. Norris, Lukhanyo H. Nyati, Alexia Murphy Alford, Nishani Lucas, Ina S. Santos, Caroline S. Costa, Rebecca Kuriyan, V Pujitha Wickranasinghe, Shabina Ariff, Andrew P. Hills

Department of Paediatrics and Child Health

Background: Given the importance of infancy for establishing growth trajectories, with later-life health consequences, we investigated longitudinal body composition among infants from six economically and ethnically diverse countries.
Methods: We recruited mother-infant dyads using the WHO Multicenter Growth Reference Study criteria. We measured fat-free mass (FFM) in 1393 (49% female) infants from birth to 6 months of age (Australia, India, and South Africa; n = 468), 3-24 months of age (Brazil, Pakistan, South Africa, and Sri Lanka; n = 925), and derived fat mass (FM), fat mass index (FMI), and fat-free mass index (FFMI). Height-for-age (HAZ), weight-for-age (WAZ), and weight-for-length …