Medical Specialties Commons^™

Fatigue In Young People With Duchenne Muscular Dystrophy, Basmah El-Aloul, Kathy N Speechley, Yi Wei, Piotr Wilk, Craig Campbell

Paediatrics Publications

AIM: To describe fatigue in Duchenne muscular dystrophy (DMD) from patients' and parents' perspectives and to explore risk factors for fatigue in children and adolescents with DMD.

METHOD: A multicentre, cross-sectional study design was used. Seventy-one patients (all males; median age 12y, age range 5-17y) identified via the Canadian Neuromuscular Disease Registry, and their parents completed questionnaires. Subjective fatigue was assessed using the Pediatric Quality of Life Inventory Multidimensional Fatigue Scale by child self-report and parent proxy-report.

RESULTS: Patients with DMD across ages and disease stages experienced greater fatigue compared to typically developing controls from published data. Sleep disturbance symptoms …

Prevalence Of Sensorineural Hearing Loss In Children With Palliated Or Repaired Congenital Heart Disease., Lalitha Gopineti, Mane Paulpillai, Andrea Rosenquist, Andrew H. Van Bergen

Manuscripts, Articles, Book Chapters and Other Papers

Background Children with congenital heart disease (CHD) are at increased risk of neurodevelopmental deficits, and the presence of sensorineural hearing loss (SNHL) may further lead to poor language skills acquisition and speech delays. Prevalence of SNHL in the general pediatric population is estimated to be 0.2% at birth to 0.35% during adolescence. Very few studies have attempted to estimate SNHL prevalence in children who have undergone congenital heart surgery. Methods This retrospective study aimed to estimate SNHL prevalence in children who underwent congenital heart surgery in our institution and were followed up in our high-risk pediatric cardiology clinics for four …

Facing Covid-19 In The Icu: Vascular Dysfunction, Thrombosis, And Dysregulated Inflammation, De Leisman, Cs Deutschman, M Legrand

Journal Articles

No abstract provided.

Varying Presentations And Favourable Outcomes Of Covid-19 Infection In Children And Young Adults With Sickle Cell Disease: An Additional Case Series With Comparisons To Published Cases., A Appiah-Kubi, S Acharya, C Fein Levy, A Vlachos, G Ostovar, K Murphy, A Farrell, D Brower, Jm Lipton, B Aygun, +1 Additional Authors

Journal Articles

No abstract provided.

A Pediatric Infectious Disease Perspective Of Sars-Cov-2 And Covid-19 In Children., A. L. Shane, A. I. Sato, C. Kao, F. C. Adler-Shohet, S. B. Vora, J. Ery, J. Auletta, S. Nachman, M. K. Kainth, K. Kotloff, +15 Additional Authors

Journal Articles

Understanding the role that children play in the clinical burden and propagation of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) responsible for novel coronavirus (COVID-19) infections is emerging. While the severe manifestations and acute clinical burden of COVID-19 has largely spared children compared to adults, understanding the epidemiology, clinical presentation, diagnostics, management, and prevention opportunities as well as the social and behavioral impacts on child health is vital. Foremost is clarifying the contribution of asymptomatic and mild infections to transmission within the household and community and the clinical and epidemiologic significance of uncommon severe post-infectious complications. Herein we summarize the …

Management Of Neonates After Postpartum Discharge And All Children In The Ambulatory Setting During The Coronavirus Disease 2019 (Covid-19) Pandemic, K. L. Harriel, D. Nolt, S. Moore, S. Kressly, H. H. Bernstein

Journal Articles

PURPOSE OF REVIEW: The present coronavirus disease 2019 (COVID-19) pandemic has created additional challenges with an increased number of presumed healthy, full-term newborns being discharged at 24 h after delivery. Short lengths of stay raise the possibility of mother-infant dyads being less ready for discharge, defined as at least one of the three informants (i.e., mother, pediatrician, and obstetrician) believing that either the mother and/or infant should stay longer than the proposed time of discharge. This public health crisis has reduced the number of in-person well child visits, negatively impacting vaccine receipt, and anticipatory guidance. RECENT FINDINGS: Extra precautions should …

Cytokine Elevation In Severe And Critical Covid-19: A Rapid Systematic Review, Meta-Analysis, And Comparison With Other Inflammatory Syndromes., D. E. Leisman, L. Ronner, R. Pinotti, M. D. Taylor, P. Sinha, C. S. Calfee, A. V. Hirayama, F. Mastroiani, C. J. Turtle, C. S. Deutschman, +2 Additional Authors

Journal Articles

The description of a so-called cytokine storm in patients with COVID-19 has prompted consideration of anti-cytokine therapies, particularly interleukin-6 antagonists. However, direct systematic comparisons of COVID-19 with other critical illnesses associated with elevated cytokine concentrations have not been reported. In this Rapid Review, we report the results of a systematic review and meta-analysis of COVID-19 studies published or posted as preprints between Nov 1, 2019, and April 14, 2020, in which interleukin-6 concentrations in patients with severe or critical disease were recorded. 25 COVID-19 studies (n=1245 patients) were ultimately included. Comparator groups included four trials each in sepsis (n=5320), cytokine …

Clinical Characteristics And Outcomes Of Critically Ill Patients With Covid-19 Admitted To An Intensive Care Unit In London: A Prospective Observational Cohort Study, R. J. Thomson, J. Hunter, J. Dutton, J. Schneider, M. Khosravi, A. Casement, K. Dhadwal, D. Martin

Journal Articles

© 2020 Thomson et al. Background Cohorts of severely ill patients with COVID-19 have been described in several countries around the globe, but to date there have been few published reports from the United Kingdom (UK). Understanding the characteristics of the affected population admitted to intensive care units (ICUs) in the UK is crucial to inform clinical decision making, research and planning for future waves of infection. Methods We conducted a prospective observational cohort study of all patients with COVID-19 admitted to a large UK ICU from March to May 2020 with follow-up to June 2020. Data were collected from …

A Primer On A Comprehensive Genetic Approach To Vascular Anomalies, A. J. Borst, T. A. Nakano, F. Blei, D. M. Adams, J. Duis

Journal Articles

No abstract provided.

Sé Un Héroe Tú También, Samantha S. Rohe, Sophie Cemaj

Books: Pediatrics

This children's book explains COVID-19 and how to decrease the incidence of transmissions.

Quiet Please: The Effects Of Sleep Quality And Quantity As A Result Of A Qi Project To Minimize Nighttime Sleep Interruptions, Chari Stump, Ashlie Tseng, Jennifer Brigle, Clifton Lee

Graduate Medical Education (GME) Resident and Fellow Research Day Posters

Quiet Please! The effects on sleep quality and quantity as a result of QI project to minimize nighttime interruptions

INTRODUCTION: Sleep is a significant component of health that is often overlooked in hospitalized patients. We introduced a QI project in 2016 to minimize nighttime interruptions in clinically stable patients by performing “passive vitals” which eliminated temperature and blood pressure readings at 4am and allowed patients to sleep longer overnight. Despite this intervention, the effects on sleep quality and quantity are unknown.

OBJECTIVE: The objective was to compare total sleep time, sleep interruptions, and sleep quality between patients eligible for passive …

Improving Early Antibiotic Administration For Treatment Of Sepsis At Children’S Hospital Of Richmond At Vcu: 2012-2019, Hannah Miller Md, Ashlie Tseng Md, Perry Taylor Pharmd, Matthew Schefft Do Msha, Sheila Pedigo Pharmd Bcpps, Tracy Lowerre Bsn Ms, Jose Munoz Md, Mark Astoria Md, Jonathan Silverman Md Mph

Graduate Medical Education (GME) Resident and Fellow Research Day Posters

Background: The Surviving Sepsis Campaign recommends initiating IV antibiotic administration within one hour of recognition of severe sepsis. Several studies have shown that prompt blood culture collection, administration of broad-spectrum antibiotics, and fluid resuscitation following recognition improves child survival.

Objective: Our goal was to evaluate effectiveness of sepsis initiatives and institutional changes in the timing of early antibiotic administration at Children’s Hospital of Richmond at VCU.

Methods: We formed a Pediatric Sepsis Committee with representatives from each unit in 2013. In 2016, the committee began tracking time from the order of a first stat dose IV antibiotic to administration as …

Quiet A Puzzle: A Case Report Of 15 Year Old Female With Severe Anemia Due To Scurvy, Shaikha Alqahtani, Wang Zhihong

Graduate Medical Education (GME) Resident and Fellow Research Day Posters

Scurvy is historically thought of a disease affecting sailors in the seventeenth century. Vitamin C deficiency is rare in the developed world. It is mainly found in patients with poor nutrition and specifically those with developmental delay. The spectrum of scurvy is varied and includes dermatological, dental, bone and systemic manifestations. We present to you a case of a 15 year old female with a history of trisomy 21 presenting with severe anemia as a direct cause of vitamin C deficiency.

She presented with a one month history of progressive bilateral lower extremity bruising and inability to walk. Physical exam …

Cancer Care Annual Report 2019-2020, 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 year’s report focuses on tumors that arise in the kidney and illustrates three important areas of focus that a childhood cancer center must possess in this new age of medicine.

Prevalence Of Alcohol Use In Late Pregnancy, Amna Umer, Christa Lilly, Candice Hamilton, Aileen Baldwin, Janine Breyel, Amy Tolliver, Christina Mullins, Collin John, Stefan Maxwell

Faculty & Staff Scholarship

Background

Prenatal alcohol exposure (PAE) can result in detrimental developmental complications. The objective of this study was to estimate the most recent PAE prevalence data for the state of West Virginia (WV) and associated factors.

Method

In all, 1830 newborn residual dried blood spots (DBS) in the WV Newborn Screening Repository were analyzed for phosphatidylethanol (PETH). Data were matched with Project WATCH data (94% match, N = 1729).

Results

The prevalence of late pregnancy PAE was 8.10% (95%CI: 6.81, 9.38) for all births, 7.61% (95%CI: 6.26, 8.97) for WV residents only, and ranged from 2.27 to 17.11% by region. The …

Adolescent Sjogren's Syndrome Presenting As Psychosis: A Case Series, E. K. Hammett, C. Fernandez-Carbonell, C. Crayne, A. Boneparth, R. Q. Cron, S. M. Radhakrishna

Journal Articles

No abstract provided.

A Quality Improvement Project To Standardize Surfactant Delivery In The Era Of Noninvasive Ventilation., J. E. Kim, M. Brewer, R. Spinazzola, E. Wallace, J. Casatelli, J. Beachy, B. Weinberger, S. Perveen

Journal Articles

No abstract provided.

Diagnosis, Treatment, And Outcomes In Children With Congenital Nephrogenic Diabetes Insipidus: A Pediatric Nephrology Research Consortium Study, C. D'Alessandri-Silva, M. Carpenter, R. Ayoob, J. Barcia, A. Chishti, A. Constantinescu, K. M. Dell, J. Goodwin, C. Sethna, J. D. Mahan, +9 Additional Authors

Journal Articles

© Copyright © 2020 D'Alessandri-Silva, Carpenter, Ayoob, Barcia, Chishti, Constantinescu, Dell, Goodwin, Hashmat, Iragorri, Kaspar, Mason, Misurac, Muff-Luett, Sethna, Shah, Weng, Greenbaum and Mahan. Background and Objectives: Congenital or primary nephrogenic diabetes insipidus (NDI) is a rare genetic disorder that severely impairs renal concentrating ability, resulting in massive polyuria. There is limited information about prognosis or evidence guiding the management of these patients, either in the high-risk period after diagnosis, or long-term. We describe the clinical presentation, genetic etiology, treatment and renal outcomes in a large group of children (89%) and white (67%). Median age at diagnosis was 4.2 months …

Advisory Committee On Immunization Practices Recommended Immunization Schedule For Children And Adolescents Aged 18 Years Or Younger - United States, 2020, C. L. Robinson, H. Bernstein, K. Poehling, J. R. Romero, P. Szilagyi

Journal Articles

No abstract provided.

Epidemiological And Clinical Characteristics Of International Travelers With Enteric Fever And Antibiotic Resistance Profiles Of Their Isolates: A Geosentinel Analysis, S. H. Hagmann, K. M. Angelo, R. Huits, K. Plewes, G. Eperon, M. P. Grobusch, A. Mccarthy, M. Libman, E. Caumes, D. H. Hamer, +15 Additional Authors

Journal Articles

Copyright © 2020 American Society for Microbiology. All Rights Reserved. Enteric fever, caused by Salmonella enterica serovar Typhi (S. Typhi) and S. enterica serovar Paratyphi (S. Paratyphi), is a common travel-related illness. Limited data are available on the antimicrobial resistance (AMR) patterns of these serovars among travelers. Records of travelers with a culture-confirmed diagnosis seen during or after travel from January 2007 to December 2018 were obtained from GeoSentinel. Traveler demographics and antimicrobial susceptibility data were analyzed. Isolates were classified as nonsusceptible if intermediate or resistant or as susceptible in accordance with the participating site’s national guidelines. A total of …

Foreword: Evaluation And Management Of Overweight And Obesity In Children And Adolescents, M. Fisher

Journal Articles

No abstract provided.

Functional Biology Of The Steel Syndrome Founder Allele And Evidence For Clan Genomics Derivation Of Col27a1 Pathogenic Alleles Worldwide, C. Gonzaga-Jauregui, G. Yesil, H. Nistala, A. Gezdirici, Y. Bayram, K. C. Nannuru, D. Pehlivan, B. Yuan, D. H. Tegay, J. R. Lupski, +27 Additional Authors

Journal Articles

© 2020, The Author(s). Previously we reported the identification of a homozygous COL27A1 (c.2089G>C; p.Gly697Arg) missense variant and proposed it as a founder allele in Puerto Rico segregating with Steel syndrome (STLS, MIM #615155); a rare osteochondrodysplasia characterized by short stature, congenital bilateral hip dysplasia, carpal coalitions, and scoliosis. We now report segregation of this variant in five probands from the initial clinical report defining the syndrome and an additional family of Puerto Rican descent with multiple affected adult individuals. We modeled the orthologous variant in murine Col27a1 and found it recapitulates some of the major Steel syndrome associated …

Longitudinal Changes In Health-Related Quality Of Life In Primary Glomerular Disease: Results From The Curegn Study, S. L. Murphy, J. D. Mahan, J. P. Troost, T. Srivastava, A. J. Kogon, Y. Cai, T. K. Davis, H. Fernandez, C. B. Sethna, V. Royal, +90 Additional Authors

Journal Articles

© 2020 Introduction: Prior cross-sectional studies suggest that health-related quality of life (HRQOL) worsens with more severe glomerular disease. This longitudinal analysis was conducted to assess changes in HRQOL with changing disease status. Methods: Cure Glomerulonephropathy (CureGN) is a cohort of patients with minimal change disease, focal seNorthwell Healthntal glomerulosclerosis, membranous nephropathy, IgA vasculitis, or IgA nephropathy. HRQOL was assessed at enrollment and follow-up visits 1 to 3 times annually for up to 5 years with the Patient-Reported Outcomes Measurement Information System (PROMIS). Global health, anxiety, and fatigue domains were measured in all; mobility was measured in children; and sleep-related …

Long-Term Neurobehavioral And Quality Of Life Outcomes Of Critically Ill Children After Glycemic Control, K. V. Biagas, V. J. Hinton, N. R. Hasbani, P. M. Luckett, D. Wypij, V. M. Nadkarni, M. S. Agus, V. Srinivasan, J. Schneider, A. Natarajan, +35 Additional Authors

Journal Articles

© 2019 Elsevier Inc. Objectives: To investigate adaptive skills, behavior, and quality health-related quality of life in children from 32 centers enrolling in the Heart And Lung Failure-Pediatric INsulin Titration randomized controlled trial. Study design: This prospective longitudinal cohort study compared the effect of 2 tight glycemic control ranges (lower target, 80-100 mg/dL vs higher target, 150-180 mg/dL) 1-year neurobehavioral and health-related quality of life outcomes. Subjects had confirmed hyperglycemia and cardiac and/or respiratory failure. Patients aged 2-16 years old enrolled between April 2012 and September 2016 were studied at 1 year after intensive care discharge. The primary outcome, adaptive …

Meningococcal Vaccination: Recommendations Of The Advisory Committee On Immunization Practices, United States, 2020, S. A. Mbaeyi, C. H. Bozio, J. Duffy, L. G. Rubin, S. Hariri, D. S. Stephens, J. R. Macneil

Journal Articles

© 2020. This report compiles and summarizes all recommendations from CDC's Advisory Committee on Immunization Practices (ACIP) for use of meningococcal vaccines in the United States. As a comprehensive summary and update of previously published recommendations, it replaces all previously published reports and policy notes. This report also contains new recommendations for administration of booster doses of serogroup B meningococcal (MenB) vaccine for persons at increased risk for serogroup B meningococcal disease. These guidelines will be updated as needed on the basis of availability of new data or licensure of new meningococcal vaccines. ACIP recommends routine vaccination with a quadrivalent …

Physician Perceptions Of Adhd Stimulant Diversion And Misuse, N. M. Colaneri, S. A. Keim, A. Adesman

Journal Articles

© The Author(s) 2016. Objective: The recent rise in ADHD has prompted concerns about adolescents with ADHD diverting and/or misusing stimulants. This is the first study to assess physician perceptions of the pervasiveness of these issues. Method: Questionnaires were mailed to a national sample of pediatric subspecialists. Responses were analyzed (n = 826; 18% response rate) using descriptive statistics and regression analyses. Results: In the past year, 59% of physicians suspected ≥1 patient(s) with ADHD diverted stimulants. Seventy-four percent believed ≥1 patient(s) feigned symptoms to obtain an initial ADHD diagnosis; 66% believed ≥1 patient(s) wanted stimulants to improve academic performance. …

Pandemic-Related Submissions: The Challenge Of Discerning Signal Amidst Noise, L. J. Kaplan, T. P. Bleck, T. G. Buchman, R. P. Dellinger, C. S. Deutschman, J. C. Marshall, D. M. Maslove, H. Masur, M. M. Parker, J. J. Zimmerman, +4 Additional Authors

Journal Articles

No abstract provided.

Patient And Physician Discordance Of Global Disease Assessment In Juvenile Dermatomyositis: Findings From The Childhood Arthritis & Rheumatology Research Alliance Legacy Registry, H. Tory, D. Zurakowski, S. Kim, L. Abramson, E. Anderson, M. Andrew, N. Battle, M. Becker, B. Gottlieb, M. Simmons, +90 Additional Authors

Journal Articles

© 2020 The Author(s). Background: Global disease activity scores (gVAS) capture patient or family (PF) and physician (MD) assessments of disease. This study sought to measure discordance between PF and MD global activity scores in juvenile dermatomyositis (JDM), and determine factors associated with discordance. Methods: Patients with JDM were included from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry (N = 563). PF and MD gVAS were assessed for discordance, defined as a ≥ 2-point difference. Factors associated with discordant gVAS were compared in univariate analysis. Multivariable regression analysis was used to identify predictors of discordance. Results: Almost …

Outcome Measurement Instruments For Peripheral Vascular Malformations And An Assessment Of The Measurement Properties: A Systematic Review, S. E. Horbach, A. P. Rongen, R. G. Elbers, C. M. Van Der Horst, C. A. Prinsen, P. I. Spuls, F. Blei, C. J. Van Der Vleuten, I. J. Frieden, L. M. Boon, +4 Additional Authors

Journal Articles

© 2019, The Author(s). Purpose: The Outcome measures for vascular malformation (OVAMA) group reached consensus on the core outcome domains for the core outcome set (COS) for peripheral vascular malformations (venous, lymphatic and arteriovenous malformations). However, it is unclear which instruments should be used to measure these domains. Therefore, our aims were to identify all outcome measurement instruments available for vascular malformations, and to evaluate their measurement properties. Methods: With the first literature search, we identified outcomes and instruments previously used in prospective studies on vascular malformations. A second search yielded studies on measurement properties of patient- and physician-reported instruments …

Proceedings From The 2NdNext Gen Therapies For Systemic Juvenile Idiopathic Arthritis And Macrophage Activation Syndrome Symposium Held On October 3-4, 2019, S. W. Canna, G. S. Schulert, A. De Jesus, A. Pickering, H. Brunner, M. Gadina, S. Levine, R. Goldbach-Mansky, B. Gottlieb, V. Delgaizo, +20 Additional Authors

Journal Articles

© 2020 The Author(s). For reasons poorly understood, and despite the availability of biological medications blocking IL-1 and IL-6 that have markedly improved overall disease control, children with Systemic Juvenile Idiopathic Arthritis (SJIA) are now increasingly diagnosed with life-threatening chronic complications, including hepatitis and lung disease (SJIA-LD). On October 3-4, 2019, a two-day meeting, NextGen Therapies for Systemic Juvenile Idiopathic Arthritis (SJIA) & macrophage activation syndrome (MAS) organized by the Systemic JIA Foundation (www.systemicjia.org/) in Washington, DC brought together scientists, clinicians, parents and FDA representatives with the objectives (1) to integrate clinical and research findings in MAS and SJIA-LD, and …