Medicine and Health Sciences Commons^™

The Similarities And Differences Between Glomerular Vs. Non-Glomerular Diagnoses On Intelligence And Executive Functions In Pediatric Chronic Kidney Disease: A Brief Report., Stephen R. Hooper, Rebecca J. Johnson, Marc Lande, Matthew Matheson, Shlomo Shinnar, Amy J. Kogon, Lyndsay Harshman, Joann Spinale, Arlene C. Gerson, Bradley A. Warady, Susan L. Furth

Manuscripts, Articles, Book Chapters and Other Papers

Pediatric chronic kidney disease (CKD) appears to be a heterogeneous group of conditions, but this heterogeneity has not been explored with respect to its impact on neurocognitive functioning. This study investigated the neurocognitive functioning of those with glomerular (G) vs. non-glomerular (NG) diagnoses. Data from the North American CKiD Study were employed and the current study included 1,003 children and adolescents with mild to moderate CKD. The G Group included 260 participants (median age = 14.7 years) and the NG Group included 743 individuals (median age = 9.0 years). Neurocognitive measures assessed IQ, inhibitory control, attention regulation, problem solving, working …

Tisagenlecleucel Immunogenicity In Relapsed/Refractory Acute Lymphoblastic Leukemia And Diffuse Large B-Cell Lymphoma., Karen Thudium Mueller, Stephan A. Grupp, Shannon L. Maude, John E. Levine, Michael A. Pulsipher, Michael W. Boyer, Keith August, Douglas Myers, Constantine S. Tam, Ulrich Jaeger, Stephen Ronan Foley, Peter Borchmann, Stephen J. Schuster, Edmund K. Waller, Rakesh Awasthi, Bernd Potthoff, Andy Warren, Edward R. Waldron, Fraser Mcblane, Andrea Chassot-Agostinho, Theodore W. Laetsch

Manuscripts, Articles, Book Chapters and Other Papers

Tisagenlecleucel is indicated for pediatric and young adult patients with relapsed/refractory (r/r) B-cell acute lymphoblastic leukemia (B-ALL) and adult patients with r/r diffuse large B-cell lymphoma (DLBCL). The tisagenlecleucel chimeric antigen receptor (CAR) contains a murine single-chain variable fragment domain; we examined the effects of humoral and cellular immune responses to tisagenlecleucel on clinical outcomes using 2 validated assays. Data were pooled from the ELIANA (registered at www.clinicaltrials.gov as #NCT02435849) and ENSIGN (#NCT02228096) trials in r/r B-ALL (N = 143) and the JULIET trial (#NCT02445248) in r/r DLBCL (N = 115). Humoral responses were determined …

Attenuation Of Autoimmune Phenomena In A Patient With Autoimmune Polyglandular Syndrome Type 1., Jill Jacobson, Julia Broussard, Courtney Marsh, Brandon D. Newell

Manuscripts, Articles, Book Chapters and Other Papers

Autoimmune polyglandular syndrome type 1 (APS1) is a progressive life-threatening illness with no known cure. Current treatments involve replacement of the hormone deficiencies that result from autoimmune destruction of multiple endocrine organs. We report on a girl whose disease was progressing rapidly until she began on immunosuppressive agents. A healthy 6-year-old girl with no remarkable medical history presented with new onset hypocalcemic seizures and primary hypoparathyroidism. Howell-Jolly bodies consistent with autoimmune hyposplenism were also noted. Genetic testing revealed compound heterozygosity for 2 disease-associated variants in the autoimmune regulator (AIRE) gene. She later developed elevated liver enzymes, primary adrenal insufficiency, and …

Multiplex Pcr Pathogen Detection In Acute Gastroenteritis Among Hospitalized Us Children Compared With Healthy Controls During 2011-2016 In The Post-Rotavirus Vaccine Era., Christopher J. Harrison, Ferdaus Hassan, Brian R. Lee, Julie Boom, Leila C. Sahni, Coreen Johnson, James Dunn, Daniel C. Payne, Mary E. Wikswo, Umesh Parashar, Rangaraj Selvarangan

Manuscripts, Articles, Book Chapters and Other Papers

Background: Despite vaccine-induced decreases in US rotavirus (RV) disease, acute gastroenteritis (AGE) remains relatively common. We evaluated AGE pathogen distribution in hospitalized US children in the post-RV vaccine era.

Methods: From December 2011 to June 2016, the New Vaccine Surveillance Network (NVSN) conducted prospective, active, population-based surveillance in hospitalized children with AGE. We tested stools from 2 NVSN sites (Kansas City, Houston) with Luminex x-TAG Gastrointestinal Pathogen Panels (Luminex GPP) and analyzed selected signs and symptoms.

Results: For 660 pediatric AGE inpatients and 624 age-matched healthy controls (HCs), overall organism detection was 51.2% and 20.6%, respectively (P < .001). Among AGE subjects, GPP polymerase chain reaction detected >1 virus …

Enterovirus D68-Associated Acute Respiratory Illness ─ New Vaccine Surveillance Network, United States, July-November 2018-2020., Melisa M. Shah, Ariana Perez, Joana Y. Lively, Vasanthi Avadhanula, Julie A. Boom, James Chappell, Janet A. Englund, Wende Fregoe, Natasha B. Halasa, Christopher J. Harrison, Robert W. Hickey, Eileen J. Klein, Monica M. Mcneal, Marian G. Michaels, Mary Moffatt, Catherine Otten, Leila C. Sahni, Elizabeth Schlaudecker, Jennifer E. Schuster, Rangaraj Selvarangan, Mary A. Staat, Laura S. Stewart, Geoffrey A. Weinberg, John V. Williams, Terry Fan Fei Ng, Janell A. Routh, Susan I. Gerber, Meredith L. Mcmorrow, Brian Rha, Claire M. Midgley

Manuscripts, Articles, Book Chapters and Other Papers

Enterovirus D68 (EV-D68) is associated with a broad spectrum of illnesses, including mild to severe acute respiratory illness (ARI) and acute flaccid myelitis (AFM). Enteroviruses, including EV-D68, are typically detected in the United States during late summer through fall, with year-to-year fluctuations. Before 2014, EV-D68 was infrequently reported to CDC (1). However, numbers of EV-D68 detection have increased in recent years, with a biennial pattern observed during 2014-2018 in the United States, after the expansion of surveillance and wider availability of molecular testing. In 2014, a national outbreak of EV-D68 was detected (2). EV-D68 was also reported in 2016 via …

Rotavirus Genotype Trends And Gastrointestinal Pathogen Detection In The United States, 2014-2016: Results From The New Vaccine Surveillance Network., Mathew D. Esona, M Leanne Ward, Mary E. Wikswo, Slavica M. Rustempasic, Rashi Gautam, Charity Perkins, Rangaraj Selvarangan, Christopher J. Harrison, Julie A. Boom, Janet A. Englund, Eileen J. Klein, Mary Allen Staat, Monica M. Mcneal, Natasha Halasa, James Chappell, Geoffrey A. Weinberg, Daniel C. Payne, Umesh D. Parashar, Michael D. Bowen

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Following the implementation of rotavirus vaccination in 2006, severe acute gastroenteritis (AGE) due to group A rotavirus (RVA) has substantially declined in US children. We report the RVA genotype prevalence as well as coinfection data from 7 US New Vaccine Surveillance Network sites during 3 consecutive RVA seasons, 2014-2016.

METHODS: A total of 1041 stool samples that tested positive for RVA by Rotaclone enzyme immunoassay were submitted to the Centers for Disease Control and Prevention (CDC) for RVA genotyping and multipathogen testing.

RESULTS: A total of 795 (76%) samples contained detectable RVA when tested at the CDC. Rotavirus disease …

Adding Family Digital Supports To Classroom-Based Physical Activity Interventions To Target In-And Out-Of-School Activity: An Evaluation Of The Stay Active Intervention Duringthe Covid-19 Pandemic, Bethany Forseth Hanson, Adrian Ortega, Paul R. Hibbing, Mallory Moon, Chelsea Steel, Mehar Singh, Avinash Kollu, Bryce Miller, Maurice Miller, Vincent S. Staggs, Hannah Calvert, Ann M. Davis, Jordan A. Carlson

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Interventions targeting multiple levels of influence and settings may have a greater impact on children’s total daily physical activity than those targeting a single setting. This study evaluated the addition of family supports to a classroom-based physical activity intervention.

Methods: 6 schools were randomized to a remotely delivered classroom-based physical activity only (CBPA) or classroom-based physical activity plus family (CBPA+) arm. The latter added behavior change tools (e.g., self-regulation) delivered via text messages and newsletters to caregiver/child dyads. The intervention lasted for 7 weeks with a 1-week baseline and 1-week post-intervention assessment (9 weeks total). Children who enrolled in …

Demographic Characteristics Among Members Of Patient Family Advisory Councils At A Pediatric Health System., Amanda Montalbano, Sheryl Chadwick, Deejo Miller, Kathryn Taff, Evelyn Donis De Miranda, Kimberly Pina, Andrea Bradley-Ewing

Manuscripts, Articles, Book Chapters and Other Papers

Patient Family Advisory Councils (PFACs) are groups of patients, families, and clinical health system leaders collaborating to improve the quality, safety, and experience of care. Best practices encourage PFAC membership to reflect the diversity of the communities served. A cross-sectional survey was conducted from September 2019 to January 2020 collecting demographic characteristics of the members of a pediatric health system's 17 PFACs. Of the 93 respondents (71.5%), notable inequities were racial (4.8% adult PFAC members vs. 17.4% patients were Black) and financial (adult PFAC median annual income was >$100,000 compared to the community median $77,589). The governing family advisory board …

The Cnn Hip Accelerometer Posture (Chap) Method For Classifying Sitting Patterns From Hip Accelerometers: A Validation Study., Mikael Anne Greenwood-Hickman, Supun Nakandala, Marta M. Jankowska, Dori E Rosenberg, Fatima Tuz-Zahra, John Bellettiere, Jordan A. Carlson, Paul R. Hibbing, Jingjing Zou, Andrea Z. Lacroix, Arun Kumar, Loki Natarajan

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Sitting patterns predict several healthy aging outcomes. These patterns can potentially be measured using hip-worn accelerometers, but current methods are limited by an inability to detect postural transitions. To overcome these limitations, we developed the Convolutional Neural Network Hip Accelerometer Posture (CHAP) classification method.

METHODS: CHAP was developed on 709 older adults who wore an ActiGraph GT3X+ accelerometer on the hip, with ground-truth sit/stand labels derived from concurrently worn thigh-worn activPAL inclinometers for up to 7 d. The CHAP method was compared with traditional cut-point methods of sitting pattern classification as well as a previous machine-learned algorithm (two-level behavior …

Healthcare System-To-System Cost Variability In The Care Of Pediatric Abdominal Pain-Associated Functional Gastrointestinal Disorders., Michelle Livitz, Alec S. Friesen, Earl F. Glynn, Jennifer Verrill Schurman, Jennifer Colombo, Craig A. Friesen

Manuscripts, Articles, Book Chapters and Other Papers

The purpose of this study was to assess cost variability in the care of abdominal pain-associated functional gastrointestinal disorders (AP-FGIDS) in youth across health systems, races, and specific AP-FGID diagnoses. Patients, aged 8-17 years, with a priority 1 diagnosis corresponding to a Rome IV defined AP-FGID were identified within the Health Facts® database. Total costs were obtained across the continuum of care including outpatient clinics, emergency department, and inpatient or observation units. Cost variability was described comparing different health systems, races, and diagnoses. Thirteen thousand two hundred and fourteen patients were identified accounting for 17,287 encounters. Total costs were available …

Prenatal Delineation Of Coronary Anatomy In Dextro-Transposition Of Great Arteries, Geetha Haligheri, Chandrakant Patel, Rukmini Komarlu

Manuscripts, Articles, Book Chapters and Other Papers

Background: Dextro-transposition of the great arteries (D-TGA) is the second-most common cyanotic congenital heart disease with variable coronary artery anatomy. The arterial switch procedure has revolutionized outcomes for this defect, with coronary anatomy being a key determinant of both short- and long-term outcomes following surgical repair. The assessment of coronary anatomy is usually undertaken in the postnatal period by transthoracic echocardiography, with assessment prenatally not being well studied. We sought to assess the feasibility of delineating the coronary arteries on fetal echocardiograms in a small cohort of patients followed prenatally. Methods: This was a retrospective review of fetuses with D-TGA …

Effectiveness Of Pfizer-Biontech Mrna Vaccination Against Covid-19 Hospitalization Among Persons Aged 12-18 Years - United States, June-September 2021., Samantha M. Olson, Margaret M. Newhams, Natasha B. Halasa, Ashley M. Price, Julie A. Boom, Leila C. Sahni, Katherine Irby, Tracie C. Walker, Stephanie P. Schwartz, Pia S. Pannaraj, Aline B. Maddux, Tamara T. Bradford, Ryan A. Nofziger, Benjamin J. Boutselis, Melissa L. Cullimore, Elizabeth H. Mack, Jennifer E. Schuster, Shira J. Gertz, Natalie Z. Cvijanovich, Michele Kong, Melissa A. Cameron, Mary A. Staat, Emily R. Levy, Brandon M. Chatani, Kathleen Chiotos, Laura D. Zambrano, Angela P. Campbell, Manish M. Patel, Adrienne G. Randolph, Overcoming Covid-19 Investigators

Manuscripts, Articles, Book Chapters and Other Papers

Pfizer-BioNTech COVID-19 vaccine is authorized for use in children and adolescents aged 12-15 years and is licensed by the Food and Drug Administration (FDA) for persons aged ≥16 (1). A randomized placebo-controlled trial demonstrated an efficacy of 100% (95% confidence interval [CI] = 75.3%-100%) in preventing outpatient COVID-19 in persons aged 12-15 years (2); however, data among adolescents on vaccine effectiveness (VE) against COVID-19 in real-world settings are limited, especially among hospitalized patients. In early September 2021, U.S. pediatric COVID-19 hospitalizations reached the highest level during the pandemic (3,4). In a test-negative, case-control study at 19 pediatric hospitals in 16 …

Lipomatous Infiltration In Tuberous Sclerosis Complex - A Case Series And Literature Review., Mohammed Ilyas, Julio Quezada, Erin K. Opfer

Manuscripts, Articles, Book Chapters and Other Papers

Diffuse Lipomatosis is a dermatological lesion consisting of a poorly circumscribed, infiltrative overgrowth of mature adipose tissue that usually affects the trunk and the extremities. The lesions in the Tuberous Sclerosis Complex (TSC) are usually hamartomatous in nature, but lesions arising from adipocytes are rare. There are only three previous reports of association of TSC with diffuse lipomatosis. Herein we present a case series of diffuse lipomatosis in three subjects with TSC and proceed to review the literature for any other reported cases. On the basis of the three index cases and identification of three more cases in the literature, …

A Prospective Observational Study Of Antihemophilic Factor (Recombinant) Prophylaxis Related To Physical Activity Levels In Patients With Hemophilia A In The United States (Space)., Barbara A. Konkle, Doris V. Quon, Leslie Raffini, Michael Recht, Vlad C. Radulescu, Shannon L. Carpenter, Amy L. Dunn, Mei Lu, Maureen Watt

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: High collision-risk physical activity can increase bleeding risk in people with hemophilia A, as can increasing the time between factor VIII (FVIII) administration and physical activity. FVIII prophylaxis may be tailored to planned activities to prevent activity-related bleeding.

Aim: To explore the relationship between physical activity levels, FVIII infusion timing, and occurrence of bleeding in patients with severe/moderately severe hemophilia A without FVIII inhibitors receiving antihemophilic factor (recombinant) (rAHF; ADVATE®; Baxalta US Inc., a Takeda company, Lexington, MA, USA).

Methods: SPACE was a 6-month, prospective, multicenter, observational outcomes study (NCT02190149). Enrolled patients received an eDiary application and …

Knowledge About Juvenile Idiopathic Arthritis-Associated Uveitis: More Frequent Reminders Are Associated With Higher Patient And Family Uveitis Knowledge., Ashley M. Cooper, Elaine R. Flanagan, Tova Ronis, Baruch Goldberg, Ashley K. Sherman, Chelsey Smith, Gary N. Holland, Carra Uveitis Workgroup

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Chronic anterior uveitis is a sight-threatening complication of juvenile idiopathic arthritis (JIA) and a primary contributor to long-term morbidity in people with JIA. Levels of knowledge about uveitis among JIA patients and their parents are unknown. A survey of JIA patients and parents was conducted to assess knowledge about uveitis complications and recommended screening.

METHODS: A survey was developed consisting of six demographic questions, six arthritis/uveitis history questions, and nine uveitis knowledge questions. The survey was administered to JIA patients age 14 and older and parents of patients with JIA at three pediatric rheumatology practices and online through the …

Rare Variants In Kdr, Encoding Vegf Receptor 2, Are Associated With Tetralogy Of Fallot., Doris Škorić-Milosavljević, Najim Lahrouchi, Fernanda M. Bosada, Gregor Dombrowsky, Simon G. Williams, Robert Lesurf, Fleur V Y Tjong, Roddy Walsh, Ihssane El Bouchikhi, Jeroen Breckpot, Enrique Audain, Aho Ilgun, Leander Beekman, Ilham Ratbi, Alanna Strong, Maximilian Muenke, Solveig Heide, Alison M. Muir, Mariam Hababa, Laura A. Cross, Dihong Zhou, T Pastinen, German Competence Network For Congenital Heart Defects, Elaine Zackai, Samir Atmani, Karim Ouldim, Najlae Adadi, Katharina Steindl, Anita Rauch, David Brook, Anna Wilsdon, Irene Kuipers, Nico A. Blom, Barbara J. Mulder, Heather C. Mefford, Boris Keren, Pascal Joset, Paul Kruszka, Isabelle Thiffault, Sarah E. Sheppard, Amy Roberts, Elisabeth M. Lodder, Bernard D. Keavney, Sally-Ann B. Clur, Seema Mital, Marc-Philip Hitz, Vincent M. Christoffels, Alex V. Postma, Connie R. Bezzina

Manuscripts, Articles, Book Chapters and Other Papers

Purpose: Rare genetic variants in KDR, encoding the vascular endothelial growth factor receptor 2 (VEGFR2), have been reported in patients with tetralogy of Fallot (TOF). However, their role in disease causality and pathogenesis remains unclear.

Methods: We conducted exome sequencing in a familial case of TOF and large-scale genetic studies, including burden testing, in >1,500 patients with TOF. We studied gene-targeted mice and conducted cell-based assays to explore the role of KDR genetic variation in the etiology of TOF.

Results: Exome sequencing in a family with two siblings affected by TOF revealed biallelic missense variants in KDR. Studies in knock-in …

Organophosphate Pesticides And Progression Of Chronic Kidney Disease Among Children: A Prospective Cohort Study., Melanie H. Jacobson, Yinxiang Wu, Mengling Liu, Kurunthachalam Kannan, Adela Jing Li, Morgan Robinson, Bradley A. Warady, Susan Furth, Howard Trachtman, Leonardo Trasande

Manuscripts, Articles, Book Chapters and Other Papers

Background: Growing evidence suggests that exposure to environmental chemicals, such as pesticides, impacts renal function and chronic kidney disease (CKD). However, it is not clear if pesticides may affect CKD progression and no studies exist in children.

Objectives: The objective of this study was to examine associations between serially measured urinary OP pesticide metabolites and clinical and laboratory measures of kidney function over time among children with CKD.

Methods: This study used data on 618 participants enrolled in the CKD in Children study (CKiD), a cohort study of pediatric CKD patients from the US and Canada. Children were followed over …

Data-Driven Clustering Identifies Features Distinguishing Multisystem Inflammatory Syndrome From Acute Covid-19 In Children And Adolescents., Alon Geva, Manish M. Patel, Margaret M. Newhams, Cameron C. Young, Mary Beth F. Son, Michele Kong, Aline B. Maddux, Mark W. Hall, Becky J. Riggs, Aalok R. Singh, John S. Giuliano, Charlotte V. Hobbs, Laura L. Loftis, Gwenn E. Mclaughlin, Stephanie P. Schwartz, Jennifer E. Schuster, Christopher J. Babbitt, Natasha B. Halasa, Shira J. Gertz, Sule Doymaz, Janet R. Hume, Tamara T. Bradford, Katherine Irby, Christopher L. Carroll, John K. Mcguire, Keiko M. Tarquinio, Courtney M. Rowan, Elizabeth H. Mack, Natalie Z. Cvijanovich, Julie C. Fitzgerald, Philip C. Spinella, Mary A. Staat, Katharine N. Clouser, Vijaya L. Soma, Heda Dapul, Mia Maamari, Cindy Bowens, Kevin M. Havlin, Peter M. Mourani, Sabrina M. Heidemann, Steven M. Horwitz, Leora R. Feldstein, Mark W. Tenforde, Jane W. Newburger, Kenneth D. Mandl, Adrienne G. Randolph, Overcoming Covid-19 Investigators

Manuscripts, Articles, Book Chapters and Other Papers

Background: Multisystem inflammatory syndrome in children (MIS-C) consensus criteria were designed for maximal sensitivity and therefore capture patients with acute COVID-19 pneumonia.

Methods: We performed unsupervised clustering on data from 1,526 patients (684 labeled MIS-C by clinicians)clusters, followed by recursive feature elimination to identify characteristics of potentially misclassified MIS-C-labeled patients.

Findings: Of 94 clinical features tested, 46 were retained for clustering. Cluster 1 patients (N = 498; 92% labeled MIS-C) were mostly previously healthy (71%), with mean age 7·2 ± 0·4 years, predominant cardiovascular (77%) and/or mucocutaneous (82%) involvement, high inflammatory biomarkers, and mostly SARS-CoV-2 PCR negative (60%). Cluster …

Aberrantly Low Stat3 And Stat5 Responses Are Associated With Poor Outcome And An Inflammatory Gene Expression Signature In Pediatric Acute Myeloid Leukemia., P Narayanan, T-K Man, R B Gerbing, R Ries, A M Stevens, Y-C Wang, X Long, A S. Gamis, T Cooper, S Meshinchi, T A Alonzo, M S Redell

Manuscripts, Articles, Book Chapters and Other Papers

The relapse rate for children with acute myeloid leukemia is nearly 40% despite aggressive chemotherapy and often stem cell transplant. We sought to understand how environment-induced signaling responses are associated with clinical response to treatment. We previously reported that patients whose AML cells showed low G-CSF-induced STAT3 activation had inferior event-free survival compared to patients with stronger STAT3 responses. Here, we expanded the paradigm to evaluate multiple signaling parameters induced by a more physiological stimulus. We measured STAT3, STAT5 and ERK1/2 responses to G-CSF and to stromal cell-conditioned medium for 113 patients enrolled on COG trials AAML03P1 and AAML0531. Low …

Longitudinal Associations Between Family Conflict, Parent Engagement, And Metabolic Control In Children With Recent-Onset Type 1 Diabetes., Hannah Case, David D. Williams, Shideh Majidi, Diana Ferro, Mark A. Clements, Susana R. Patton

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: We prospectively investigated the associations between diabetes-related family conflict, parent engagement in child type 1 diabetes (T1D) care, and child glycated hemoglobin (HbA1c) in 127 families of school-age children who we recruited within the first year of their T1D diagnosis.

Research design and methods: Parents completed the Diabetes Family Conflict Scale-Revised (DFCS-R) to assess for diabetes-related family conflict and the Diabetes Self-Management Questionnaire-Brief (DSMQ-Brief) to assess parent engagement in child T1D care at the initial study visit (T1) and at 12 (T2) and 27 (T3) months later. We also collected child HbA1c at these time points. Our analyses included …

Initiative To Increase The Rate Of Emergency Department Physician Preprocedure Time-Out Documentation., Arjun Sarin, Nikita Sharma, Shobhit Jain

Manuscripts, Articles, Book Chapters and Other Papers

The preprocedure time-out is an important safety measure to verify patient identity and accuracy of a planned procedure. The time-out is an institutional and Joint Commission requirement. However, physicians in our emergency departments (EDs) document it inconsistently. We aimed to improve physician preprocedure time-out documentation for deep sedation (ketamine and/or propofol) from 75% to 90%, and separately for cutaneous abscess incision and drainage (I&D) from 94% to 98% by June 2020.

Methods: We analyzed 1 year of baseline data and weekly electronic medical record (EMR) reports from November 2019 through June 2020. Our outcome measures were the rate of physician …

Brief Musculoskeletal Screen And Patient Education For Down Syndrome-Associated Arthritis., Jordan T. Jones, Chelsey Smith, Nasreen Talib

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Mortality In Children Treated With Maintenance Peritoneal Dialysis: Findings From The International Pediatric Peritoneal Dialysis Network Registry., Sophie Ploos Van Amstel, Marlies Noordzij, Dagmara Borzych-Duzalka, Nicholas C. Chesnaye, Hong Xu, Lesley Rees, Il-Soo Ha, Zenaida L. Antonio, Nakysa Hooman, William Wong, Karel Vondrak, Yok Chin Yap, Hiren Patel, Maria Szczepanska, Sara Testa, Monica Galanti, Jameela A. Kari, Charlotte Samaille, Sevcan A. Bakkaloglu, Wai-Ming Lai, Luisa Fernanda Rojas, Mabel Sandoval Diaz, Biswanath Basu, Alicia Neu, Bradley A. Warady, Kitty J. Jager, Franz Schaefer

Manuscripts, Articles, Book Chapters and Other Papers

RATIONALE & OBJECTIVE: Research on pediatric kidney replacement therapy (KRT) has primarily focused on Europe and North America. In this study, we describe the mortality risk of children treated with maintenance peritoneal dialysis (MPD) in different parts of the world and characterize the associated demographic and macroeconomic factors.

STUDY DESIGN: Prospective cohort study.

SETTING & PARTICIPANTS: Patients younger than 19 years at inclusion into the International Pediatric Peritoneal Dialysis Network registry, who initiated MPD between 1996 and 2017.

EXPOSURE: Region as primary exposure (Asia, Western Europe, Eastern Europe, Latin America, North America, and Oceania). Other demographic, clinical, and macroeconomic (4 …

Are Body Surface Area Based Estimates Of Liver Volume Applicable To Children With Overweight Or Obesity? An In Vivo Validation Study., Chelsea Hosey-Cojocari, Sherwin S. Chan, Chance S. Friesen, Amie L. Robinson, Veronica Williams, Erica Swanson, Daniel O'Toole, Jansynn Radford, Neil J. Mardis, Trevor N. Johnson, J Steven Leeder, Valentina Shakhnovich

Manuscripts, Articles, Book Chapters and Other Papers

The liver is the primary organ responsible for clearing most drugs from the body and thus determines systemic drug concentrations over time. Drug clearance by the liver appears to be directly related to organ size. In children, organ size changes as children age and grow. Liver volume has been correlated with body surface area (BSA) in healthy children and adults and has been estimated by functions of BSA. However, these relationships were derived from "typical" populations and it is unknown whether they extend to estimations of liver volumes for population "outliers," such as children with overweight or obesity, who today …

Identifying The Content And Context Of Pain Within Paediatric Rheumatology Healthcare Professional Curricula In The Uk: A Summative Content Analysis., Rebecca Rachael Lee, Janet E. Mcdonagh, Mark Connelly, Sarah Peters, Lis Cordingley

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The curriculum for professionals working in paediatric rheumatology should include pain but it is unclear to what extent this currently occurs. The aim of this study was to identify pain-related curriculum content and the context in which pain is presented in educational and training documentation for healthcare professionals in this clinical speciality.

METHODS: Core curricula documents from UK based professional organisations were identified in partnership with healthcare professionals. Documents were analysed using a summative content analysis approach. Key pain terms were quantified and weighted frequencies were used to explore narrative pain themes. Latent content was interpreted qualitatively to explore …

Rumination Syndrome In Children And Adolescents: A Mini Review., Marc Martinez, Sandeep Rathod, Hunter J. Friesen, John Rosen, Craig A. Friesen, Jennifer Verrill Schurman

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Rumination syndrome involves recurrent regurgitation of food and is believed to be underdiagnosed with patients experiencing long delays in diagnosis. It can be associated with significant social consequences, high rates of school absenteeism, and medical complications such as weight loss. The primary aims of the current review are to assess the literature regarding prevalence, pathophysiology, and treatment outcomes with a focus on neurotypical children and adolescents.

Results: Population studies in children/adolescents, 5 years of age or older, range from 0 to 5.1%. There are fewer studies in clinical settings, but the prevalence appears to be higher in patients with …

Ability Of Video Telemetry To Predict Unplanned Hospital Admissions For Single Ventricle Infants., Doaa Aly, Lori Erickson, Hayley S. Hancock, Johnathan Apperson, Monica Gaddis, Girish S. Shirali, Suma Goudar

Manuscripts, Articles, Book Chapters and Other Papers

Background: Our Cardiac High Acuity Monitoring Program (CHAMP) uses home video telemetry (HVT) as an adjunct to monitor infants with single ventricle during the interstage period. This study describes the development of an objective early warning score using HVT, for identification of infants with single ventricle at risk for clinical deterioration and unplanned hospital admissions (UHA).

Methods and Results: Six candidate scoring parameters were selected to develop a pragmatic score for routine evaluation of HVT during the interstage period. We evaluated the individual and combined ability of these parameters to predict UHA. All infants with single ventricle monitored at home …

Ventricular Fibrillation Due To A Likely Pathogenic Sos1 Variant: An Unrecognized Etiology Of Infantile Sudden Death?, Christopher Follansbee, Lindsey Malloy-Walton

Manuscripts, Articles, Book Chapters and Other Papers

We present the case of a female infant presenting after a ventricular fibrillation arrest found to have ectopic atrial tachycardia (EAT). Evaluation revealed a likely pathogenic variant in SOS1 not previously reported in affected individuals. SOS1 variants are associated with Noonan syndrome, which belongs to a family of related genetic syndromes affecting the RAS/MAPK signaling pathway. To date, this is the first case reported of a ventricular fibrillation arrest in a patient with a RASopathy-related variant prior to development of the typically associated structural cardiac phenotype and may represent a previously unrecognized etiology of sudden death during infancy.

Atypical Presentation Of Systemic Arthritis In A Toddler With Down Syndrome., Emily Worley, Weijie Li, Jordan T. Jones

Manuscripts, Articles, Book Chapters and Other Papers

Systemic juvenile idiopathic arthritis (sJIA) is a chronic, inflammatory disease of childhood, which is characterized by the combination of arthritis, serositis, daily, high-spiking fevers, and evanescent macular rash and can present with the life-threatening complication of macrophage activation syndrome (MAS). Children with Down syndrome (DS) have complex medical challenges related to abnormalities in their immune system, which can cause a broad spectrum of disease manifestations, which can occur atypically. Children with DS are at increased risk for arthritis and interstitial lung disease (ILD) associated with sJIA that has high mortality. This case report outlines an atypical presentation of sJIA in …

An Update On The Assessment And Management Of Pediatric Abdominal Pain., Craig A. Friesen, Jennifer Colombo, Amanda D. Deacy, Jennifer Verrill Schurman

Manuscripts, Articles, Book Chapters and Other Papers

Chronic abdominal pain is very common in children and adolescent and results in high personal and social costs. Most youth with chronic abdominal pain fulfill criteria for a functional abdominal pain disorder (FAPD) as defined by Rome criteria. These are complex conditions with a wide array of biological, psychological, and social factors contributing to the experience of pain. The purpose of the current review is to provide an overview of the pathophysiology of FAPDs and an up-to-date summary of the literature related to FAPDs in children and adolescents, with additional focus on several areas (eg, diet and probiotics) where patients …