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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 …

Sleep Spindles Promote The Restructuring Of Memory Representations In Ventromedial Prefrontal Cortex Through Enhanced Hippocampal-Cortical Functional Connectivity, E. Cowan, A. Liu, S. Henin, S. Kothare, O. Devinsky, L. Davachi

Journal Articles

No abstract provided.

The Longitudinal Relationship Between Patient-Reported Outcomes And Clinical Characteristics Among Patients With Focal Senorthwell Healthntal Glomerulosclerosis In The Nephrotic Syndrome Study Network, J. P. Troost, A. Waldo, N. E. Carlozzi, S. Murphy, F. Modersitzki, H. Trachtman, P. H. Nachman, C. B. Sethna, P. Singer, D. S. Gipson, +36 Additional Authors

Journal Articles

No abstract provided.

Turner Syndrome With Y Chromosome: Spontaneous Thelarche, Menarche, And Risk Of Malignancy, E. Dabrowski, E. K. Johnson, V. Patel, Y. Hsu, S. Davis, A. L. Goetsch, R. Habiby, W. J. Brickman, C. Finlayson

Journal Articles

No abstract provided.

The Surviving Sepsis Campaign: Basic/Translational Science Research Priorities∗, C. S. Deutschman, J. Hellman, R. Ferrer Roca, D. De Backer, C. M. Coopersmith

Journal Articles

© 2020 by the Society of Critical Care Medicine and Wolters Kluwer Health, Inc. All Rights Reserved. Objectives: Expound upon priorities for basic/translational science identified in a recent paper by a group of experts assigned by the Society of Critical Care Medicine and the European Society of Intensive Care Medicine. Data Sources: Original paper, search of the literature. Study Selection: By several members of the original task force with specific expertise in basic/translational science. Data Extraction: None. Data Synthesis: None. Conclusions: In the first of a series of follow-up reports to the original paper, several members of the original task …

Treatment Of Children And Adolescents Who Are Overweight Or Obese, S. Di Figlia-Peck, R. Feinstein, M. Fisher

Journal Articles

No abstract provided.

Urinary Epidermal Growth Factor As A Marker Of Disease Progression In Children With Nephrotic Syndrome, D. S. Gipson, H. Trachtman, A. Waldo, K. L. Gibson, S. Eddy, K. M. Dell, T. Srivastava, K. V. Lemley, C. B. Sethna, W. Ju, +18 Additional Authors

Journal Articles

© 2019 International Society of Nephrology Introduction: Childhood-onset nephrotic syndrome has a variable clinical course. Improved predictive markers of long-term outcomes in children with nephrotic syndrome are needed. This study tests the association between baseline urinary epidermal growth factor (uEGF) excretion and longitudinal kidney function in children with nephrotic syndrome. Methods: The study evaluated 191 participants younger than 18 years enrolled in the Nephrotic Syndrome Study Network, including 118 with their first clinically indicated kidney biopsy (68 minimal change disease; 50 focal seNorthwell Healthntal glomerulosclerosis) and 73 with incident nephrotic syndrome without a biopsy. uEGF was measured at baseline for …

Highly Sensitive Blocker Displacement Amplification And Droplet Digital Pcr Reveal Low-Level Parental Foxf1 Somatic Mosaicism In Families With Alveolar Capillary Dysplasia With Misalignment Of Pulmonary Veins., J. A. Karolak, Q. Liu, N. G. Xie, L. R. Wu, G. Rocha, E. K. Fiorino, M. Edelman, J. Fox, D. A. Hayes, P. Stankiewicz, +9 Additional Authors

Journal Articles

Detection of low-level somatic mosaicism [alternate allele fraction (AAF) ≤ 10%] in parents of affected individuals with the apparent de novo pathogenic variants enables more accurate estimate of recurrence risk. To date, only a few systematic analyses of low-level parental somatic mosaicism have been performed. Herein, highly sensitive blocker displacement amplification, droplet digital PCR, quantitative PCR, long-range PCR, and array comparative genomic hybridization were applied in families with alveolar capillary dysplasia with misalignment of pulmonary veins. We screened 18 unrelated families with the FOXF1 variant previously determined to be apparent de novo (n = 14), of unknown parental origin (n …

Clinical Practices For Measles-Mumps-Rubella Vaccination Among Us Pediatric International Travelers., E. P. Hyle, S. R. Rao, A. C. Bangs, P. Gastañaduy, A. P. Fiebelkorn, S. H. F. Hagmann, A. T. Walker, R. P. Walensky, E. T. Ryan, R. C. Larocque

Journal Articles

Importance: The US population is experiencing a resurgence of measles, with more than 1000 cases during the first 6 months of 2019. Imported measles cases among returning international travelers are the source of most US measles outbreaks, and these importations can be reduced with pretravel measles-mumps-rubella (MMR) vaccination of pediatric travelers. Although it is estimated that children account for less than 10% of US international travelers, pediatric travelers account for 47% of all known measles importations.

Objective: To examine clinical practice regarding MMR vaccination of pediatric international travelers and to identify reasons for nonvaccination of pediatric travelers identified as MMR …

Characteristics, Cardiac Involvement, And Outcomes Of Multisystem Inflammatory Disease Of Childhood (Mis-C) Associated With Sars-Cov-2 Infection., Ca Capone, A Subramony, T Sweberg, J Schneider, S Shah, L Rubin, C Schleien, S Epstein, Jc Johnson, A Kessel, N Misra, E Mitchell, N Palumbo, S Rajan, J Rocker, K Williamson, Kw Davidson

Journal Articles

No abstract provided.

Limiting Hospital Resources For Acute Appendicitis In Children: Lessons Learned From The U.S. Epicenter Of The Covid-19 Pandemic, Cl Kvasnovsky, Y Shi, B S. Rich, R D. Glick, S Z. Soffer, A M. Lipskar, S Dolgin, A Hong, J M. Prince, C Sathya, +2 Additional Authors

Journal Articles

No abstract provided.

Febrile Infant: Covid-19 In Addition To The Usual Suspects, E Robbins, Z Ilahi, P Roth

Journal Articles

No abstract provided.

Presentation Of Severe Acute Respiratory Syndrome-Coronavirus 2 Infection As Cholestatic Jaundice In Two Healthy Adolescents, A. Perez, D. Kogan-Liberman, S. Sheflin-Findling, A. Raizner, K. L. Ahuja, N. Ovchinsky

Journal Articles

© 2020 Elsevier Inc. Liver abnormalities in severe acute respiratory syndrome-coronavirus 2 infection, including hepatitis and cholestasis, have been observed in adults and are associated with worse outcomes. We describe 2 adolescents with cholestasis and hepatitis with mild presentation of severe acute respiratory syndrome-coronavirus 2 lacking typical symptoms. Our intention is to raise index of suspicion for testing and protective equipment use.

Severe Coronavirus Disease 2019 Infection In An Adolescent Patient After Hematopoietic Stem Cell Transplantation, G. Fisler, A. Haimed, C. F. Levy, J. Stiles, C. A. Capone, J. D. Fish, J. A. Brochstein, M. D. Taylor

Journal Articles

© 2020 American College of Chest Physicians Infection with the severe acute respiratory syndrome coronavirus 2 causes severe acute lung injury in approximately 5% of infected adults, but few reports have been made of severe pediatric disease. We present an adolescent patient who contracted severe acute respiratory syndrome coronavirus 2 one week after a paternal haplo-identical hematopoietic stem cell transplant, with development of severe hyperferritinemic acute lung injury and macrophage activation-like syndrome. We present her case and a comparison of her laboratory data with those of a cohort of pediatric patients with coronavirus disease 2019 without severe disease.