Medical Specialties Commons^™

A Pediatric Patient With Sickle Cell Disease Presenting With Severe Anemia And Splenic Sequestration In The Setting Of Covid-19, S. Jacob, A. Dworkin, E. Romanos-Sirakis

Journal Articles

No abstract provided.

Immunotherapies For Covid-19: Lessons Learned From Sepsis, Ke Remy, Sc Brakenridge, B Francois, T Daix, Cs Deutschman, G Monneret, R Jeannet, Pf Laterre, Rs Hotchkiss, Ll Moldawer

Journal Articles

No abstract provided.

Pediatric Covid-19-Associated Rhabdomyolysis: A Case Report, Am Gefen, N Palumbo, Sk Nathan, Ps Singer, Lj Castellanos-Reyes, Cb Sethna

Journal Articles

No abstract provided.

Altered Monocyte And Langerhans Cell Innate Immunity In Patients With Recurrent Respiratory Papillomatosis (Rrp), M. Israr, J. A. Devoti, F. Lam, A. L. Abramson, B. M. Steinberg, V. R. Bonagura

Journal Articles

No abstract provided.

Cd4 T Follicular Helper Cells Prevent Depletion Of Follicular B Cells In Response To Cecal Ligation And Puncture, M. D. Taylor, M. R. Brewer, A. Nedeljkovic-Kurepa, Y. Yang, K. S. Reddy, M. N. Abraham, B. J. Barnes, C. S. Deutschman

Journal Articles

No abstract provided.

Comparison Of Artifacts Between Paste And Collodion Method Of Electrode Application In Pediatric Eeg, D. Brigham, Y. Shah, K. Singh, I. Pavkovic, S. Karkare, S. V. Kothare

Journal Articles

© 2019 International Federation of Clinical Neurophysiology Objectives: Children pose challenges to obtain quality EEG data due to excessive artifact. Collodion is used in EEG electrodes due to its water resistance and strong adhesive qualities. This study was done to evaluate differences in artifacts between the collodion and paste method. Methods: 115 subjects (children age >3 years) were randomized into paste and collodion groups and artifacts evaluated at baseline and every hour over 30 s increments. Age, sleep state, and number of electrodes with artifact were also documented. T-test was performed to determine differences in the various parameters between the …

Cost Of Survivorship Care And Adherence To Screening-Aligning The Priorities Of Health Care Systems And Survivors., C. Benedict, J. Wang, M. Reppucci, C. L. Schleien, J. D. Fish

Journal Articles

Childhood cancer survivors (CCS) experience significant morbidity due to treatment- related late effects and benefit from late-effects surveillance. Adherence to screening recommendations is suboptimal. Survivorship care programs often struggle with resource limitations and may benefit from understanding institution-level financial outcomes associated with patient adherence to justify programmatic development and growth. The purpose of this study is to examine how CCS adherence to screening recommendations relates to the cost of care, insurance status, and institution-level financial outcomes. A retrospective chart review of 286 patients, followed in a structured survivorship program, assessed adherence to the Children's Oncology Group follow-up guidelines by comparing …

Demographics And Outcomes Of Patients With Eating Disorders Treated In Residential Care, M. Fisher, J. R. Henretty, S. A. Cox, R. Feinstein, V. Fornari, L. Moskowitz, M. Schneider, S. Levine, J. Malizio, J. Fishbein

Journal Articles

No abstract provided.

Early Oral Colostrum Administration In Preterm Infants, D. Maffei, M. Brewer, C. Codipilly, B. Weinberger, R. J. Schanler

Journal Articles

No abstract provided.

Evaluation And Management Of Overweight And Obesity In Children And Adolescents – Current Problems In Pediatric And Adolescent Health Care, J. Kerns, M. Fisher

Journal Articles

No abstract provided.

Gonococcal Osteomyelitis In A Pediatric Patient With Disseminated Gonococcal Infection: Implications For Antimicrobial Management, W. Liakos, B. Schaffler, S. Rajan, S. H. Hagmann

Journal Articles

© 2020 The Authors We report a case of a female teenager with gonococcal septic arthritis of the right shoulder that also caused osteomyelitis of the humeral head. Infection with Neisseria gonorrhoeae is a frequently diagnosed sexually transmitted infection in the sexually active teenage population and disseminated gonococcal infection (DGI) is the most common systemic manifestation of acute gonorrhea. DGI commonly involves acute arthritis, tenosynovitis and dermatitis with less common complications of endocarditis, hepatitis and meningitis. In contrast, osteomyelitis has only rarely been reported as a result of gonococcal infection. Clinicians need to be aware of this unusual manifestation of …

Management Of Pediatric Systemic Lupus Erythematosus: Focus On Belimumab, M. Guzman, J. S. Hui-Yuen

Journal Articles

No abstract provided.

Newborn Screening Protocols And Positive Predictive Value For Congenital Adrenal Hyperplasia Vary Across The United States, P. W. Speiser, R. Chawla, M. Chen, A. Diaz-Thomas, C. Finlayson, M. M. Rutter, D. E. Sandberg, K. Shimy, J. Cerise, E. C. Délot, +2 Additional Authors

Journal Articles

© 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). Newborn screening for congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency is mandated throughout the US. Filter paper blood specimens are assayed for 17-hydroxyprogesterone (17OHP). Prematurity, low birth weight, or critical illness cause falsely elevated results. The purpose of this report is to highlight differences in protocols among US state laboratories. We circulated a survey to state laboratory directors requesting qualitative and quantitative information about individual screening programs. Qualitative …

Use Of Organ Dysfunction As A Primary Outcome Variable Following Cecal Ligation And Puncture: Recommendations For Future Studies, M. N. Abraham, A. P. Kelly, A. B. Brandwein, T. D. Fernandes, D. E. Leisman, M. D. Taylor, M. R. Brewer, C. A. Capone, C. S. Deutschman

Journal Articles

Outcomes variables for research on sepsis have centered on mortality and changes in the host immune response. However, a recent task force (Sepsis-3) revised the definition of sepsis to "life-threatening organ dysfunction caused by a dysregulated host response to infection." This new definition suggests that human studies should focus on organ dysfunction. The appropriate criteria for organ dysfunction in either human sepsis or animal models are, however, poorly delineated, limiting the potential for translation. Further, in many systems, the difference between "dysfunction" and "injury" may not be clear. In this review, we identify criteria for organ dysfunction and/or injury in …

Characteristics And Risk Factors Associated With Critical Illness In Pediatric Covid-19, G. Fisler, S. M. Izard, S. Shah, D. Lewis, M. K. Kainth, S. H. F. Hagmann, J. A. Belfer, L. M. Feld, F. Mastroianni, C. L. Kvasnovsky, C. A. Capone, J. Schneider, T. Sweberg, C. Schleien, M. D. Taylor

Journal Articles

© 2020, The Author(s). Background: While much has been reported regarding the clinical course of COVID-19 in children, little is known regarding factors associated with organ dysfunction in pediatric COVID-19. We describe critical illness in pediatric patients with active COVID-19 and identify factors associated with PICU admission and organ dysfunction. This is a retrospective chart review of 77 pediatric patients age 1 day to 21 years admitted to two New York City pediatric hospitals within the Northwell Health system between February 1 and April 24, 2020 with PCR + SARS-CoV-2. Descriptive statistics were used to describe the hospital course and …

The Role Of Rassf5 On Cell Growth And Hippo Signaling In Rhabdomyosarcoma, Megan Garver, Lee Helman, Md, Katrina Slemmons, Phd

Phase 1

Introduction: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood. Dysregulation of the Hippo pathway, a signaling cascade that regulates many biological processes, is associated with many human cancers. The goal of my project was to delete RASSF5, a regulator of the Hippo pathway, in RMS cells utilizing CRISPR/Cas9 and then to evaluate how the absence of RASSF5 affects RMS cell growth and Hippo signaling, with and without DNMTi Tx.

Methods: A lipofectamine transfection was performed in two different RMS cell lines, Rh30 & RD, in which two different CRIPSR/Cas9 vectors with RASSF5 guide RNA were introduced. …

Graduate Medical Education Annual Report 2019-2020, 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.

Allergic Reaction In Hem/Onc Patient, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Growth Hormone Deficiency In Megalencephaly-Capillary Malformation Syndrome: An Association With Activating Mutations In Pik3ca, Shanlee Davis, Meredith A Ware, Jordan Zeiger, Matthew A Deardorff, Katheryn Grand, Adda Grimberg, Stephanie Hsu, Megan Kelsey, Shideh Majidi, Revi P Matthew, Melanie Napier, Natalie Nokoff, Chitra Prasad, Andrew C Riggs, Margaret L Mckinnon, Ghayda Mirzaa

Paediatrics Publications

Megalencephaly-capillary malformation syndrome (MCAP) is a brain overgrowth disorder characterized by cortical malformations (specifically polymicrogyria), vascular anomalies, and segmental overgrowth secondary to somatic activating mutations in the PI3K-AKT-MTOR pathway (PIK3CA). Cases of growth failure and hypoglycemia have been reported in patients with MCAP, raising the suspicion for unappreciated growth hormone (GH) deficiency. Here we report an observational multicenter study of children with MCAP and GH deficiency. Eleven participants were confirmed to have GH deficiency, all with very low or undetectable circulating concentrations of insulin-like growth factor-1 and insulin-like growth factor binding protein-3. Seven underwent GH stimulation testing and all had …

The Pancreas-Specific Form Of Secretory Pathway Calcium Atpase 2 Regulates Multiple Pathways Involved In Calcium Homeostasis, Melissa Fenech, Mckenzie M Carter, Peter B Stathopulos, Christopher Pin

Paediatrics Publications

Acinar cell exocytosis requires spatiotemporal Ca²⁺ signals regulated through endoplasmic reticulum (ER) stores, Ca²⁺ATPases, and store-operated Ca²⁺ entry (SOCE). The secretory pathway Ca²⁺ATPase 2 (SPCA2) interacts with Orai1, which is involved in SOCE and store independent Ca²⁺ entry (SICE). However, in the pancreas, only a C-terminally truncated form of SPCA2 (termed SPAC2C) exists. The goal of this study was to determine if SPCA2C effects Ca²⁺ homeostasis in a similar fashion to the full-length SPCA2. Using epitope-tagged SPCA2C (SPCA2C^FLAG) expressed in HEK293A cells and Fura2 imaging, cytosolic [Ca²⁺] was …

Intranasal Dexmedetomidine For Procedural Distress In Children: A Systematic Review., Naveen Poonai, Joseph Spohn, Ben Vandermeer, Samina Ali, Maala Bhatt, Shawn Hendrikx, Evelyne D Trottier, Vikram Sabhaney, Amit Shah, Gary Joubert, Lisa Hartling

Paediatrics Publications

CONTEXT: Intranasal dexmedetomidine (IND) is an emerging agent for procedural distress in children.

OBJECTIVE: To explore the effectiveness of IND for procedural distress in children.

DATA SOURCES: We performed electronic searches of Medline (1946-2019), Embase (1980-2019), Google Scholar (2019), Cumulative Index to Nursing and Allied Health Literature (1981-2019), and Cochrane Central Register.

STUDY SELECTION: We included randomized trials of IND for procedures in children.

DATA EXTRACTION: Methodologic quality of evidence was evaluated by using the Cochrane Collaboration's risk of bias tool and the Grading of Recommendations Assessment, Development, and Evaluation system, respectively. The primary outcome was the proportion of participants …

Brain Metabolite Levels In Sedentary Women And Non-Contact Athletes Differ From Contact Athletes., Amy L Schranz, Gregory A Dekaban, Lisa Fischer, Kevin Blackney, Christy Barreira, Timothy J Doherty, Douglas Fraser Md, Phd, Frcpc, Arthur Brown, Jeff Holmes, Ravi S Menon, Robert Bartha

Paediatrics Publications

White matter tracts are known to be susceptible to injury following concussion. The objective of this study was to determine whether contact play in sport could alter white matter metabolite levels in female varsity athletes independent of changes induced by long-term exercise. Metabolite levels were measured by single voxel proton magnetic resonance spectroscopy (MRS) in the prefrontal white matter at the beginning (In-Season) and end (Off-Season) of season in contact (

Mtorc1 Transcriptional Regulation Of Ribosome Subunits, Protein Synthesis, And Molecular Transport In Primary Human Trophoblast Cells., Fredrick J Rosario, Theresa L Powell, Madhulika B Gupta, Laura Cox, Thomas Jansson

Paediatrics Publications

Mechanistic Target of Rapamycin Complex 1 (mTORC1) serves as positive regulator of placental nutrient transport and mitochondrial respiration. The role of mTORC1 signaling in modulating other placental functions is largely unexplored. We used gene array following silencing of raptor to identify genes regulated by mTORC1 in primary human trophoblast (PHT) cells. Seven hundred and thirty-nine genes were differentially expressed; 487 genes were down-regulated and 252 up-regulated. Bioinformatic analyses demonstrated that inhibition of mTORC1 resulted in decreased expression of genes encoding ribosomal proteins in the 60S and 40S ribosome subunits. Furthermore, down-regulated genes were functionally enriched in genes involved in eIF2, …

The Cost-Utility Of Measles-Mumps-Rubella Immunization Strategies During A Mumps Outbreak, Kikanwa Anyiwe, David Naimark, Oliver Baclic, Vinita Dubey, Marina Salvadori, Man Wah Yeung, Matthew Tunis, Beate Sander

Paediatrics Publications

No abstract provided.

Improving Access And Mental Health For Youth Through Virtual Models Of Care, Cheryl Forchuk, Sandra Fisman, Jeffrey P. Reiss, Kerry Collins, Julie Eichstedt, Abraham Rudnick, Wanrudee Isaranuwatchai, Jeffrey S. Hoch, Xianbin Wang, Daniel Lizotte, Shona Macpherson, Richard Booth

Paediatrics Publications

The overall objective of this research is to evaluate the use of a mobile health smartphone application (app) to improve the mental health of youth between the ages of 14–25 years, with symptoms of anxiety/depression. This project includes 115 youth who are accessing outpatient mental health services at one of three hospitals and two community agencies. The youth and care providers are using eHealth technology to enhance care. The technology uses mobile questionnaires to help promote self-assessment and track changes to support the plan of care. The technology also allows secure virtual treatment visits that youth can participate in through …

Nox2 Is Critical To Endocardial To Mesenchymal Transition And Heart Development, Hoda Moazzen, Yan Wu, Anish Engineer, Xiangru Lu, Simran Aulakh, Qingping Feng

Paediatrics Publications

NADPH oxidases (NOX) are a major source of reactive oxygen species (ROS) production in the heart. ROS signaling regulates gene expression, cell proliferation, apoptosis, and migration. However, the role of NOX2 in embryonic heart development remains elusive. We hypothesized that deficiency of Nox2 disrupts endocardial to mesenchymal transition (EndMT) and results in congenital septal and valvular defects. Our data show that 34% of Nox2-/- neonatal mice had various congenital heart defects (CHDs) including atrial septal defects (ASD), ventricular septal defects (VSD), atrioventricular canal defects (AVCD), and malformation of atrioventricular and aortic valves. Notably, Nox2-/- embryonic hearts show abnormal development of …

Estimation Of Gfr In Patients With Cystic Fibrosis: A Cross-Sectional Study, Andrea Wallace, April Price, Erin Fleischer, Michael Khoury, Guido Filler

Paediatrics Publications

Background: Patients with cystic fibrosis (CF) have frequent infectious complications requiring nephrotoxic medications, necessitating monitoring of renal function. Although adult studies have suggested that cystatin C (CysC)-based estimated glomerular filtration rate (eGFR) may be preferable due to reduced muscle mass of patients with CF, pediatric patients remain understudied. Objective: Our objective was to determine which eGFR formula is best for estimating glomerular filtration rate (GFR) in pediatric patients with CF. Methods: A total of 17 patients with CF treated with nephrotoxic antibiotics were recruited from the Children’s Hospital at London Health Sciences Centre, London, Ontario, Canada. 99Tc DTPA GFR (measured …

Presence Of Benznidazole Conjugated Metabolites In Urine Identified By Β-Glucuronidase Treatment, María Elena Marson, Facundo García Bournissen, Jaime Altcheh, Guillermo Moscatelli, Samantha Moroni, Guido Enrique Mastrantonio

Paediatrics Publications

Chagas disease is a serious public health problem in Latin America and, due to migration, in other non-endemic regions. Benznidazole (BNZ) is first choice drug in pediatric therapeutics. However, little is known regarding its metabolism in humans. The aim of the study was to isolate and identify products of human BZN metabolism in urine samples obtained from a pediatric Chagas patient and a healthy adult volunteer both treated with BZN. Urine samples were collected after dose of BNZ. Urine was treated with β-glucuronidase followed by an extraction procedure under two different pH conditions and a HPLC/UV and MS/MS identification of …

Composite Outcomes For Clinical Trials In Critical Care: The Devil Is In The Detail, Saptharishi Lalgudi Ganesan, Narayanan Parameswaran

Data and Test Instruments

No abstract provided.

Office Of Equity Diversity Report Annual Report Fy2020, 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 Leadership Structure, Employee Demographics, Patient Demographics, lasting impressions from FY20, and the future of healthcare providers.