Medical Specialties Commons^™

Enhanced Contribution Of Hla In Pediatric Onset Ulcerative Colitis, S. Venkateswaran, J. Prince, D. J. Cutler, U. M. Marigorta, D. T. Okou, S. Prahalad, D. Mack, B. Boyle, J. Markowitz, S. Kugathasan, +20 Additional Authors

Journal Articles

© 2018 Crohn's & Colitis Foundation. Published by Oxford University Press on behalf of Crohn's & Colitis Foundation. Background The genetic contributions to pediatric onset ulcerative colitis (UC), characterized by severe disease and extensive colonic involvement, are largely unknown. In adult onset UC, Genome Wide Association Study (GWAS) has identified numerous loci, most of which have a modest susceptibility risk (OR 0.84-1.14), with the exception of the human leukocyte antigen (HLA) region on Chromosome 6 (OR 3.59). Method To study the genetic contribution to exclusive pediatric onset UC, a GWAS was performed on 466 cases with 2099 healthy controls using …

Barriers To Adherence In Juvenile Idiopathic Arthritis: A Multicenter Collaborative Experience And Preliminary Results, L. A. Favier, J. Taylor, K. L. Rich, K. B. Jones, S. S. Vora, J. G. Harris, B. S. Gottlieb, L. Robbins, J. T. Lai, A. C. Modi, +7 Additional Authors

Journal Articles

© 2018. All rights reserved. Objective. Nonadherence is currently an underrecognized and potentially modifiable obstacle to care in juvenile idiopathic arthritis (JIA). The purpose of our study was to design and implement a standardized approach to identifying adherence barriers for youth with JIA across 7 pediatric rheumatology clinics through the Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) and to assess the frequency of adherence barriers in patients and their caregivers across treatment modalities. Methods. An iterative process using coproduction among parents and providers of patients with JIA was used to design the Barriers Assessment Tool to screen for adherence …

Increased Prevalence Of Congenital Heart Disease In Children With Diamond Blackfan Anemia Suggests Unrecognized Diamond Blackfan Anemia As A Cause Of Congenital Heart Disease In The General Population: A Report Of The Diamond Blackfan Anemia Registry., A. Vlachos, D. S. Osorio, E. Atsidaftos, J. Kang, M. L. Lababidi, H. S. Seiden, D. Gruber, B. E. Glader, K. Onel, J. M. Lipton, +6 Additional Authors

Journal Articles

No abstract provided.

A Functional Assay For The Clinical Annotation Of Genetic Variants Of Uncertain Significance In Diamond–Blackfan Anemia, A. Aspesi, M. Betti, M. Sculco, C. Actis, C. Olgasi, M. W. Wlodarski, A. Vlachos, J. M. Lipton, U. Ramenghi, I. Dianzani, +3 Additional Authors

Journal Articles

© 2018 The Authors. Human Mutation published by Wiley Periodicals, Inc. Diamond–Blackfan anemia (DBA) is a rare genetic hypoplasia of erythroid progenitors characterized by mild to severe anemia and associated with congenital malformations. Clinical manifestations in DBA patients are quite variable and genetic testing has become a critical factor in establishing a diagnosis of DBA. The majority of DBA cases are due to heterozygous loss-of-function mutations in ribosomal protein (RP) genes. Causative mutations are fairly straightforward to identify in the case of large deletions and frameshift and nonsense mutations found early in a protein coding sequence, but diagnosis becomes more …

Minimum Quality Threshold In Pre-Clinical Sepsis Studies (Mqtipss): An International Expert Consensus Initiative For Improvement Of Animal Modeling In Sepsis, M. F. Osuchowski, A. Ayala, S. Bahrami, M. Bauer, M. Boros, J. M. Cavaillon, I. H. Chaudry, C. Deutschman, P. Wang, B. Zingarelli, +21 Additional Authors

Journal Articles

© 2018, The Author(s). Purpose: Pre-clinical animal studies precede the majority of clinical trials. While the clinical sepsis definitions and recommended treatments are regularly updated, a systematic review of pre-clinical models of sepsis has not been done and clear modeling guidelines are lacking. To address this deficit, a Wiggers-Bernard Conference on pre-clinical sepsis modeling was held in Vienna in May, 2017. The conference goal was to identify limitations of pre-clinical sepsis models and to propose a set of guidelines, defined as the “Minimum Quality Threshold in Pre-Clinical Sepsis Studies” (MQTiPSS), to enhance translational value of these models. Methods: 31 experts …

Disparities In Utilization Of Social Determinants Of Health Referrals Among Children In Immigrant Families, O. T. Uwemedimo, H. May

Journal Articles

© 2018 Uwemedimo and May. Objective: Children in immigrant families (CIF) are at elevated risk of experiencing adverse social determinants of health (SDH), particularly material hardship, which contribute to disparate health outcomes. Previous studies have found that SDH screening programs integrated into pediatric practices have increased receipt of social service resources. Few studies have examined use of social services in these programs among ethnically-diverse patient populations and associations with caregiver immigrant status or limited English proficiency (LEP). Methods: Caregivers of children (<18 >years) were routinely screened in a practice-based, SDH screening program offering referral, assisted navigation and follow-up support. Information …

Minimum Quality Threshold In Pre-Clinical Sepsis Studies (Mqtipss): An International Expert Consensus Initiative For Improvement Of Animal Modeling In Sepsis., M. F. Osuchowski, A. Ayala, S. Bahrami, M. Bauer, M. Boros, J. Cavaillon, I. H. Chaudry, C. S. Deutschman, P. Wang, B. Zingarelli, +21 Additional Authors

Journal Articles

Preclinical animal studies precede the majority of clinical trials. While the clinical definitions of sepsis and recommended treatments are regularly updated, a systematic review of preclinical models of sepsis has not been done and clear modeling guidelines are lacking. To address this deficit, a Wiggers-Bernard Conference on preclinical sepsis modeling was held in Vienna in May, 2017. The goal of the conference was to identify limitations of preclinical sepsis models and to propose a set of guidelines, defined as the "Minimum Quality Threshold in Preclinical Sepsis Studies" (MQTiPSS), to enhance translational value of these models. A total of 31 experts …

Minimum Quality Threshold In Pre-Clinical Sepsis Studies (Mqtipss): An International Expert Consensus Initiative For Improvement Of Animal Modeling In Sepsis., M. F. Osuchowski, A. Ayala, S. Bahrami, M. Bauer, M. Boros, J. Cavaillon, I. H. Chaudry, C. Deutschman, P. Wang, B. Zingarelli, +21 Additional Authors

Journal Articles

BACKGROUND:Pre-clinical animal studies precede the majority of clinical trials. While the clinical definitions of sepsis and recommended treatments are regularly updated, a systematic review of pre-clinical models of sepsis has not been done and clear modeling guidelines are lacking. OBJECTIVE:To address this deficit, a Wiggers-Bernard Conference on pre-clinical sepsis modeling was held in Vienna in May 2017. The goal of the conference was to identify limitations of pre-clinical sepsis models and to propose a set of guidelines, defined as the "Minimum Quality Threshold in Pre-Clinical Sepsis Studies" (MQTiPSS), to enhance translational value of these models. METHODS:A total of 31 experts …

Identification, Management, And Transition Of Care For Patients With Opioid Use Disorder In The Emergency Department, H. C. Duber, I. A. Barata, E. Cioe-Pena, S. Y. Liang, E. Ketcham, W. Macias-Konstantopoulos, S. A. Ryan, M. Stavros, L. K. Whiteside

Journal Articles

No abstract provided.

Hyposialylated Igg Activates Endothelial Igg Receptor Fcγriib To Promote Obesity-Induced Insulin Resistance, K. Tanigaki, A. Sacharidou, J. Peng, K. L. Chambliss, I. S. Yuhanna, D. Ghosh, M. Ahmed, A. J. Szalai, W. Vongpatanasin, C. Mineo, +10 Additional Authors

Journal Articles

Type 2 diabetes mellitus (T2DM) is a common complication of obesity. Here, we have shown that activation of the IgG receptor FcγRIIB in endothelium by hyposialylated IgG plays an important role in obesity-induced insulin resistance. Despite becoming obese on a high-fat diet (HFD), mice lacking FcγRIIB globally or selectively in endothelium were protected from insulin resistance as a result of the preservation of insulin delivery to skeletal muscle and resulting maintenance of muscle glucose disposal. IgG transfer in IgG-deficient mice implicated IgG as the pathogenetic ligand for endothelial FcγRIIB in obesity-induced insulin resistance. Moreover, IgG transferred from patients with T2DM …

2017-2018 Pediatric Subspecialty Trainees, Children's Mercy Hospital

Residents and Fellows Portraits

Photo composites of the graduate pediatric trainees in allergy/immunology, child abuse pediatrics, child neurology, clinical child psychology, clinical pharmacology, developmental-behavioral pediatrics, hospice & palliative care, laboratory genetics & genomics, neonatal-perinatal medicine, pediatric adolescent gynecology, pediatric anesthesiology, pediatric bioethics, pediatric cardiology, pediatric critical care medicine, pediatric dentistry, pediatric emergency medicine, pediatric endocrinology, pediatric gastroenterology, pediatric hematology/oncology, pediatric hospital medicine, pediatric infectious diseases, pediatric nephrology, pediatric optometry, pediatric ophthalmology, pediatric otolaryngology, pediatric pulmonology, pediatric radiology, pediatric rehabilitation, pediatric sports medicine, pediatric surgery, pediatric residency and med/peds residency.

Developing Racial Literacy And Cultural Fluency In The Pediatric Healthcare Setting: The Use Of Staff Focus Groups To Address Implicit Bias And Cultural Competence When Working With A Culturally Diverse Patient Population, Natalie Kinsky

Graduate Student Independent Studies

Developing Racial Literacy and Cultural Fluency in the Pediatric Healthcare Setting: The use of staff focus groups to address implicit bias and cultural competence when working with a culturally diverse patient population by Natalie Kinsky.

The purpose of this evidence-based practice (EBP), quality improvement project was to explore how targeted education and discussion, delivered through healthcare staff focus groups pertaining to race, culture, and bias, affect staff members’ knowledge and awareness of how these issues and experiences affect pediatric patients/families.

The EBP project consisted of pediatric healthcare staff focus groups to discuss topics of race, bias, and culture. The self-selected …

Spontaneous Dna Damage To The Nuclear Genome Promotes Senescence, T Redox Imbalance And Aging, Andria R. Robinson, Matthew J. Yousefzadeh, Tania A. Rozgaja, Jin Wang, Xuesen Li, Jeremy S. Tilstra, Chelsea H. Feldman, Siobhan Q. Gregg, Caroline H. Johnson, Erin M. Skoda, Marie-Celine Frantz, Harris Bell-Temin, Hannah Pope-Varsalona, Aditi U. Gurkar, Luigi A. Nasto, Rena A.S. Robinson, Heike Fuhrmann-Stroissnigg, Jolanta Czerwinska, Sara J. Mcgowan, Nadiezhda Cantu-Madellin, Jamie B. Harris, Salony Maniar, Mark A. Ross, Christy E. Trussoni, Nicholas F. Larusso, Eugenia Cifuentes-Pagano, Patrick J. Pagano, Barbara Tudek, Nam V. Vo, Lora H. Rigatti, Patricia L. Opresko, Donna B. Stolz, Simon C. Watkins, Christin E. Burd, Claudette M. St, Croix, Gary Siuzdak, Nathan A. Yates, Paul D. Robbins, Yinsheng Wang, Peter Wipf, Eric E. Kelley, Laura J. Neidernhofer

Faculty & Staff Scholarship

Accumulation of senescent cells over time contributes to aging and age-related diseases. However, what drives senescence in vivo is not clear. Here we used a genetic approach to determine if spontaneous nuclear DNA damage is sufficient to initiate senescence in mammals. Ercc1-/Δ mice with reduced expression of ERCC1-XPF endonuclease have impaired capacity to repair the nuclear genome. Ercc1-/Δ mice accumulated spontaneous, oxidative DNA damage more rapidly than wild-type (WT) mice. As a consequence, senescent cells accumulated more rapidly in Ercc1-/Δ mice compared to repair-competent animals. However, the levels of DNA damage and senescent cells in Ercc1-/Δ mice never exceeded that …

Risk Perceptions Of Cellphone Use While Driving: Results From A Delphi Survey, Motao Zhu, Toni M. Rudisill, Kimberly J. Rauscher, Danielle M. Davidov, Jing Feng

Faculty & Staff Scholarship

Cellphone use while driving has been recognized as a growing and important public health issue by the World Health Organization and U.S. Center for Disease Control and Prevention. Surveys typically collect data on overall texting while driving, but do not differentiate between various forms of cellphone use. This study sought to improve the survey indicators when monitoring cellphone use among young drivers. Experts and young drivers were recruited to propose behavioral indicators (cellphone use while driving behaviors) and consequential indicators (safety consequences of cellphone use while driving) in 2016. Subsequently, experts and young drivers selected the top indicators using the …

Future Implications Of Using Registered Dietitians In Multidisciplinary Polycystic Ovary Syndrome Treatment, Wendy M. Wolf, Rachel A. Wattick, Pamela J. Murray, Melanie Clemmer, Melissa D. Olfert

Faculty & Staff Scholarship

Polycystic ovary syndrome (PCOS) is the most common reproductive endocrine disorder in females with insulin resistance playing a key role in pathogenesis. The objective of this study was to investigate current trends and future implications of multidisciplinary PCOS clinics with inclusion of dietitians. A two-phase, formative investigation on practitioners was conducted through an anonymous survey followed by focus groups. Survey respondents included 261 health care providers from around the world; the majority (59%) representing multidisciplinary teams. Focus group participants included four dietitians, three physicians, a health psychologist and a licensed nutritionist. Primary barriers for future multidisciplinary clinics included: money/resources, insurance …

Airway Pressure Release Ventilation In Pediatric Acute Respiratory Distress Syndrome. A Randomized Controlled Trial, Saptharishi Lalgudi Ganesan, Muralidharan Jayashree, Sunit Chandra Singhi, Arun Bansal

Data and Test Instruments

No abstract provided.

Graduate Medical Education Annual Report 2017-2018, Children's Mercy Hospital

GME Annual Reports

Annual report of the Children's Mercy Hospital Graduate Medical Education department, including pediatric residency, internal medicine/pediatrics residency, fellowship programs, medical students, continuing medical education, and library services.

Cancer Care Annual Report 2017-2018, Children's Mercy Hospital

Cancer Center Annual Reports

Annual report of cancer care and oncology services at the Children's Mercy Hospital, Kansas City MO, a pediatric medical center. This report has a focus on the HOPE Clinic (Clinica de Hematologia/Oncologia: Puente de Esperanza).

Learning The Hard Way: Clinical Trials In Juvenile Idiopathic Arthritis, Roberta A Berard, Ronald M Laxer

Paediatrics Publications

No abstract provided.

A Randomized Placebo-Controlled Phase 3 Trial Of An Antisense Oligonucleotide, Drisapersen, In Duchenne Muscular Dystrophy, Nathalie Goemans, Eugenio Mercuri, Elena Belousova, Hirofumi Komaki, Alberto Dubrovsky, Craig M Mcdonald, John E Kraus, Afrodite Lourbakos, Zhengning Lin, Giles Campion, Susanne X Wang, Craig Campbell, Demand Iii Study Group

Paediatrics Publications

This 48-week, randomized, placebo-controlled phase 3 study (DMD114044; NCT01254019) evaluated efficacy and safety of subcutaneous drisapersen 6 mg/kg/week in 186 ambulant boys aged ≥5 years, with Duchenne muscular dystrophy (DMD) resulting from an exon 51 skipping amenable mutation. Drisapersen was generally well tolerated, with injection-site reactions and renal events as most commonly reported adverse events. A nonsignificant treatment difference (P = 0.415) in the change from baseline in six-minute walk distance (6MWD; primary efficacy endpoint) of 10.3 meters in favor of drisapersen was observed at week 48. Key secondary efficacy endpoints (North Star Ambulatory Assessment, 4-stair climb ascent velocity, and …

Peripheral Blood Epi-Signature Of Claes-Jensen Syndrome Enables Sensitive And Specific Identification Of Patients And Healthy Carriers With Pathogenic Mutations In Kdm5c., Laila C Schenkel, Erfan Aref-Eshghi, Cindy Skinner, Peter Ainsworth, Hanxin Lin, Guillaume Paré, David I Rodenhiser, Charles Schwartz, Bekim Sadikovic

Paediatrics Publications

Background: Claes-Jensen syndrome is an X-linked inherited intellectual disability caused by mutations in the KDM5C gene. Kdm5c is a histone lysine demethylase involved in histone modifications and chromatin remodeling. Males with hemizygous mutations in KDM5C present with intellectual disability and facial dysmorphism, while most heterozygous female carriers are asymptomatic. We hypothesized that loss of KDM5C function may influence other components of the epigenomic machinery including DNA methylation in affected patients.

Results: Genome-wide DNA methylation analysis of 7 male patients affected with Claes-Jensen syndrome and 56 age- and sex-matched controls identified a specific DNA methylation defect (epi-signature) in the peripheral blood …

Genomic Dna Methylation-Derived Algorithm Enables Accurate Detection Of Malignant Prostate Tissues., Erfan Aref-Eshghi, Laila C Schenkel, Peter Ainsworth, Hanxin Lin, David I Rodenhiser, Jean-Claude Cutz, Bekim Sadikovic

Paediatrics Publications

Introduction: The current methodology involving diagnosis of prostate cancer (PCa) relies on the pathology examination of prostate needle biopsies, a method with high false negative rates partly due to temporospatial, molecular, and morphological heterogeneity of prostate adenocarcinoma. It is postulated that molecular markers have a potential to assign diagnosis to a considerable portion of undetected prostate tumors. This study examines the genome-wide DNA methylation changes in PCa in search of genomic markers for the development of a diagnostic algorithm for PCa screening.

Methods: Archival PCa and normal tissues were assessed using genomic DNA methylation arrays. Differentially methylated sites and regions …

Disruption To Functional Networks In Neonates With Perinatal Brain Injury Predicts Motor Skills At 8 Months, Annika C Linke, Conor Wild, Leire Zubiaurre-Elorza, Charlotte Herzmann, Hester Duffy, Victor K Han, David S C Lee, Rhodri Cusack

Paediatrics Publications

Objective

Functional connectivity magnetic resonance imaging (fcMRI) of neonates with perinatal brain injury could improve prediction of motor impairment before symptoms manifest, and establish how early brain organization relates to subsequent development. This cohort study is the first to describe and quantitatively assess functional brain networks and their relation to later motor skills in neonates with a diverse range of perinatal brain injuries.

Methods

Infants (n = 65, included in final analyses: n = 53) were recruited from the neonatal intensive care unit (NICU) and were stratified based on their age at birth (prematurevs. term), and on whether neuropathology …

Infants Born Large For Gestational Age And Developmental Attainment In Early Childhood, Cairina E Frank, Kathy N Speechley, Jennifer J Macnab, M Karen Campbell

Paediatrics Publications

Objectives

To investigate if an association exists between being born large for gestational age (LGA) and verbal ability or externalizing behaviour problems at ages 4-5 years.

Method

A secondary analysis was conducted using the National Longitudinal Survey of Children and Youth, including singleton births in 2004-2005 followed till 4-5 years (

Results

Infants born LGA were not found to be at increased risk for poor verbal ability (aOR: 1.16 [0.49,2.72] and aOR: 0.83 [0.37,1.87] for girls and boys, resp.) or externalizing behaviour problems (aOR: 1.24 [0.52,2.93] and aOR: 1.24 [0.66,2.36] for girls and boys, resp.). Social factors were found to …

Factors That Reduce The Accuracy Of Seizure Identification Using Quantitative Eeg Displays, Saptharishi Lalgudi Ganesan, Craig Stewart, Eshetu Atenafu, Ayako Ochi, Hiroshi Otsubo, Cristina Go, Cecil Hahn

Data and Test Instruments

No abstract provided.

All Ttg Positive Patient Do Not Have Celiac Disease, Pankaj Vohra, Kalyan Ray Parashette, Rajmohan Dharmaraj, Angela Deubel, Richardo Castillo, Jacqueline Fridge

Pediatrics Research and Scholarship

Tissue transglutaminase antibody IgA level (tTG) is an excellent screening test for celiac disease in the presence of adequate IgA levels. Most labs while screening for Celiac disease (CD) will do the IgA levels automatically. However the serological test is not a diagnostic test.

An Investigation Into The Relationship Between Fundamental Movement Skill Proficiency And Markers Of Health Among A Cohort Of Irish Primary School Children, Linda Bolger

PhDs

Background: Fundamental movement skills (FMS) are basic observable patterns of movement. Although FMS are positively associated with health, FMS levels among children worldwide are low. Interventions to improve FMS and markers of health among children are warranted. Thus, the purpose of this research was to firstly evaluate the FMS proficiency and markers of health of Irish primary school children, and to design and implement a programme of interventions to improve both.

Methods: Two interventions (1 Physical Activity [PA] and 1 FMS) were implemented and evaluated using children from 3 primary schools in Cork. Children (N=217, mean age: 7.98±2.00 years) …

The Effectiveness Of School-Based Interventions On The Fundamental Movement Skill Proficiency Among A Cohort Of Irish Primary School Children, Lisa Bolger

PhDs

Background: Fundamental movement skills (FMS) are basic observable patterns of movement such as running and jumping. FMS facilitate participation in physical activity and sport. The ability to perform FMS correctly (i.e. FMS proficiency) is associated with numerous health benefits and is important for the holistic development of children. FMS proficiency among primary school children worldwide is low. Thus, interventions aimed at improving FMS levels among children are warranted. Therefore, this thesis aimed to assess the FMS proficiency among a cohort of Irish primary school children and examine the effectiveness of a physical activity (PA) (Year 1) and a multicomponent FMS …

Children's Mercy Annual Report 2018, Children's Mercy Hospital

Children's Mercy Annual Reports

Annual report for The Children's Mercy Hospital in Kansas City MO, a pediatric medical center.

Equity & Diversity Report 2017, Children's Mercy Hospital

Equity & Diversity Annual Reports

A report on equity and diversity at Children's Mercy Hospitals & Clinics, in the care it provides, the people it serves, and in its workforce. Specifically discusses the Equity & Diversity Council, The Office of Equity and Diversity, Community Engagement, Care Delivery and Supportive Mechanisms, Patient-Provider Communication, Workforce Diversity and Inclusion, Leadership, Research, Public Accountability and Quality Improvement, and Education and Training.