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A Pilot Intervention Combining Assessment And Feedback With Communication Training And Behavioral Nudges To Increase Hpv Vaccine Uptake., Andrea Bradley-Ewing, Brian R. Lee, Jason N. Doctor, Georgann Meredith, Kathy Goggin, Angela Myers Dec 2022

A Pilot Intervention Combining Assessment And Feedback With Communication Training And Behavioral Nudges To Increase Hpv Vaccine Uptake., Andrea Bradley-Ewing, Brian R. Lee, Jason N. Doctor, Georgann Meredith, Kathy Goggin, Angela Myers

Manuscripts, Articles, Book Chapters and Other Papers

Human papillomavirus (HPV) causes >40,000 cancer diagnoses each year, yet vaccination rates remain low because widespread implementation of strategies to increase vaccinations has not occurred. Behavioral nudges have demonstrated efficacy in improving uptake of desired behaviors in health care settings but have not been tested for increasing HPV vaccinations. We assessed the impact of an intervention combining behavioral nudges with other proven strategies (i.e., assessment and feedback, provider communication training) on HPV vaccination rates and parental satisfaction in four Midwestern pediatric, outpatient practices. Practices were randomly assigned to receive either assessment and feedback or assessment and feedback combined with vaccine …


Gender Dysphoria In Adolescents With Ehlers-Danlos Syndrome., Jordan T. Jones, William R. Black, Christine Moser, Eric T. Rush, Lindsey Malloy-Walton Dec 2022

Gender Dysphoria In Adolescents With Ehlers-Danlos Syndrome., Jordan T. Jones, William R. Black, Christine Moser, Eric T. Rush, Lindsey Malloy-Walton

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Ehlers-Danlos Syndrome represents a family of heritable connective tissue disorders that include joint hypermobility, tissue fragility, and skin hyperextensibility. Ehlers-Danlos Syndrome presents with clinical sequela across multiple body systems that require multidisciplinary care. Little is known about adolescents with Ehlers-Danlos Syndrome who are transgender and gender diverse. To date, there have been no reports of transgender and gender diverse youth in pediatric patients with Ehlers-Danlos Syndrome. The objective of this study was to characterize transgender and gender diverse adolescents with Ehlers-Danlos Syndrome seen in a pediatric multidisciplinary specialty clinic.

METHODS: A retrospective chart review was performed and it was …


Characteristics Of Antifungal Utilization For Hospitalized Children In The United States., Lourdes Eguiguren, Brian R. Lee, Jason G. Newland, Matthew P. Kronman, Adam L. Hersh, Jeffrey S. Gerber, Grace M. Lee, Hayden T. Schwenk Dec 2022

Characteristics Of Antifungal Utilization For Hospitalized Children In The United States., Lourdes Eguiguren, Brian R. Lee, Jason G. Newland, Matthew P. Kronman, Adam L. Hersh, Jeffrey S. Gerber, Grace M. Lee, Hayden T. Schwenk

Manuscripts, Articles, Book Chapters and Other Papers

Objective: To characterize antifungal prescribing patterns, including the indication for antifungal use, in hospitalized children across the United States.

Design: We analyzed antifungal prescribing data from 32 hospitals that participated in the SHARPS Antibiotic Resistance, Prescribing, and Efficacy among Children (SHARPEC) study, a cross-sectional point-prevalence survey conducted between June 2016 and December 2017.

Methods: Inpatients aged < 18 years with an active systemic antifungal order were included in the analysis. We classified antifungal prescribing by indication (ie, prophylaxis, empiric, targeted), and we compared the proportion of patients in each category based on patient and antifungal characteristics.

Results: Among 34,927 surveyed patients, 2,095 (6%) received at least 1 systemic antifungal and there were 2,207 antifungal prescriptions. Most patients had an underlying oncology or bone marrow transplant diagnosis (57%) or were premature (13%). The most prescribed antifungal was fluconazole (48%) and the …


A Step-By-Step Guide For Mentors To Facilitate Team Building And Communication In Virtual Teams., Julia F. Aquino, Robert Riss, Sara M. Multerer, Leora N. Mogilner, Teri L. Turner Dec 2022

A Step-By-Step Guide For Mentors To Facilitate Team Building And Communication In Virtual Teams., Julia F. Aquino, Robert Riss, Sara M. Multerer, Leora N. Mogilner, Teri L. Turner

Manuscripts, Articles, Book Chapters and Other Papers

As collaborative work in medical education has increasingly moved online, team mentors have had to adapt their practices into the virtual environment. Fostering connection, communication and productivity on virtual teams requires specific skills and deliberate practice that differ from in-person teamwork. Drawing from best practices in business, education and medicine and also from our own experience as a virtual team, we present a guide for mentors to create and sustain successful virtual teams. Grounded in Tuckman's Five Stage Model of Team Development, we offer specific strategies for virtual team mentors to promote team cohesion, mitigate conflict, maintain productivity and leverage …


Impact Of Diabetes Status And Related Factors On Covid-19-Associated Hospitalization: A Nationwide Retrospective Cohort Study Of 116,370 Adults With Sars-Cov-2 Infection., Erin M. Tallon, Osagie Ebekozien, Janine Sanchez, Vincent S. Staggs, Diana Ferro, Ryan Mcdonough, Carla Demeterco-Berggren, Sarit Polsky, Patricia Gomez, Neha Patel, Priya Prahalad, Ori Odugbesan, Priyanka Mathias, Joyce M. Lee, Chelsey Smith, Chi-Ren Shyu, Mark A. Clements Dec 2022

Impact Of Diabetes Status And Related Factors On Covid-19-Associated Hospitalization: A Nationwide Retrospective Cohort Study Of 116,370 Adults With Sars-Cov-2 Infection., Erin M. Tallon, Osagie Ebekozien, Janine Sanchez, Vincent S. Staggs, Diana Ferro, Ryan Mcdonough, Carla Demeterco-Berggren, Sarit Polsky, Patricia Gomez, Neha Patel, Priya Prahalad, Ori Odugbesan, Priyanka Mathias, Joyce M. Lee, Chelsey Smith, Chi-Ren Shyu, Mark A. Clements

Manuscripts, Articles, Book Chapters and Other Papers

Aims: We examined diabetes status (no diabetes; type 1 diabetes [T1D]; type 2 diabetes [T2D]) and other demographic and clinical factors as correlates of coronavirus disease 2019 (COVID-19)-related hospitalization. Further, we evaluated predictors of COVID-19-related hospitalization in T1D and T2D.

Methods: We analyzed electronic health record data from the de-identified COVID-19 database (December 2019 through mid-September 2020; 87 US health systems). Logistic mixed models were used to examine predictors of hospitalization at index encounters associated with confirmed SARS-CoV-2 infection.

Results: In 116,370 adults ( >=18 years old) with COVID-19 (93,098 no diabetes; 802 T1D; 22,470 T2D), factors that independently increased …


Tonsillectomy In Children With 22q11.2 Deletion Syndrome., Jill M. Arganbright, Paul Bryan Hankey, Meghan Tracy, Srivats Narayanan, Janelle R. Noel-Macdonnell Phd, David G. Ingram Md Nov 2022

Tonsillectomy In Children With 22q11.2 Deletion Syndrome., Jill M. Arganbright, Paul Bryan Hankey, Meghan Tracy, Srivats Narayanan, Janelle R. Noel-Macdonnell Phd, David G. Ingram Md

Manuscripts, Articles, Book Chapters and Other Papers

Tonsillectomy is one of the most common procedures performed in children, however there are currently no published studies evaluating tonsillectomy in children with 22q11.2 deletion syndrome (22q11DS). With this study, our goal was to investigate the indications, efficacy, and complications of tonsillectomy in a pediatric cohort of patients with 22q11DS. This is a retrospective chart review of patients in our 22q Center's repository. Inclusion criteria were a diagnosis of 22q11DS and a history of tonsillectomy or adenotonsillectomy. Data collected included: indications for tonsillectomy, preoperative and postoperative polysomnography (PSG) results, and surgical complications. In total, 33 patients were included. Most common …


Frosted Branch Angiitis Presenting After A Sars-Cov-2 Infection., Akhila Alapati, Nathaniel Cameron, Sean Gratton, Erin Stahl, Mary Champion Nov 2022

Frosted Branch Angiitis Presenting After A Sars-Cov-2 Infection., Akhila Alapati, Nathaniel Cameron, Sean Gratton, Erin Stahl, Mary Champion

Manuscripts, Articles, Book Chapters and Other Papers

PURPOSE: To report a case of frosted branch angiitis presenting in a pediatric patient with unremarkable laboratory work-up apart from SARS-CoV-2 IgG antibodies.

OBSERVATIONS: Less than four weeks after a SARS-CoV-2 infection, a 10 year-old female presented to the emergency department with severe headache and intermittent fevers. During her hospital admission, the ophthalmology service was consulted for blurry vision. Subsequent eye examination revealed frosted branch angiitis. The patient initially received intravenous corticosteroids but was escalated to plasmapheresis to achieve resolution of her symptoms. Outpatient maintenance therapy consisted of an oral Prednisone taper and Infliximab infusion.

CONCLUSION AND IMPORTANCE: This case …


Diabetic Ketoacidosis Continuous Glucose Monitors, Children's Mercy Kansas City Nov 2022

Diabetic Ketoacidosis Continuous Glucose Monitors, Children's Mercy Kansas City

Clinical Critically Appraised Topics

No abstract provided.


A Review Of Ferric Citrate Clinical Studies, And The Rationale And Design Of The Ferric Citrate And Chronic Kidney Disease In Children (Fit4kid) Trial., Mark R. Hanudel, Marciana L. Laster, Anthony A. Portale, Aditi Dokras, Raymond P. Quigley, German A Lozano Guzman, Joshua J. Zaritsky, Nicole A. Hayde, Frederick J. Kaskel, Mark M. Mitsnefes, Jorge A. Ramirez, Peace D. Imani, Poyyapakkam R. Srivaths, Amy J. Kogon, Michelle R. Denburg, Tom D. Blydt-Hansen, Loretta Z. Reyes, Larry A. Greenbaum, Darcy K. Weidemann, Bradley A. Warady, David A. Elashoff, Susan R. Mendley, Tamara Isakova, Isidro B. Salusky Nov 2022

A Review Of Ferric Citrate Clinical Studies, And The Rationale And Design Of The Ferric Citrate And Chronic Kidney Disease In Children (Fit4kid) Trial., Mark R. Hanudel, Marciana L. Laster, Anthony A. Portale, Aditi Dokras, Raymond P. Quigley, German A Lozano Guzman, Joshua J. Zaritsky, Nicole A. Hayde, Frederick J. Kaskel, Mark M. Mitsnefes, Jorge A. Ramirez, Peace D. Imani, Poyyapakkam R. Srivaths, Amy J. Kogon, Michelle R. Denburg, Tom D. Blydt-Hansen, Loretta Z. Reyes, Larry A. Greenbaum, Darcy K. Weidemann, Bradley A. Warady, David A. Elashoff, Susan R. Mendley, Tamara Isakova, Isidro B. Salusky

Manuscripts, Articles, Book Chapters and Other Papers

Pediatric chronic kidney disease (CKD) is characterized by many co-morbidities, including impaired growth and development, CKD-mineral and bone disorder, anemia, dysregulated iron metabolism, and cardiovascular disease. In pediatric CKD cohorts, higher circulating concentrations of fibroblast growth factor 23 (FGF23) are associated with some of these adverse clinical outcomes, including CKD progression and left ventricular hypertrophy. It is hypothesized that lowering FGF23 levels will reduce the risk of these events and improve clinical outcomes. Reducing FGF23 levels in CKD may be accomplished by targeting two key stimuli of FGF23 production-dietary phosphate absorption and iron deficiency. Ferric citrate is approved for use …


A Methodological And Clinical Approach To Measured Energy Expenditure In The Critically Ill Pediatric Patient., Jaime Silva-Gburek, Paola Hong Zhu, Marwa Mansour, David Walding, Jorge A. Coss-Bu Oct 2022

A Methodological And Clinical Approach To Measured Energy Expenditure In The Critically Ill Pediatric Patient., Jaime Silva-Gburek, Paola Hong Zhu, Marwa Mansour, David Walding, Jorge A. Coss-Bu

Manuscripts, Articles, Book Chapters and Other Papers

The metabolic response to injury and stress is characterized initially by a decreased energy expenditure (Ebb phase) followed by an increased metabolic expenditure (Flow phase). Indirect calorimetry is a methodology utilized to measure energy expenditure and substrate utilization by measuring gas exchange in exhaled air and urinary nitrogen. The use of indirect calorimetry in critically ill patients requires precise equipment to obtain accurate measurements. The most recent guidelines suggested that measured energy expenditure by indirect calorimetry be used to determine energy requirements. This article reviews the methodological and clinical use of indirect calorimetry in critically ill pediatric patients.


The Use Of Virtual Tools In Narrowing The Impact Of Health Disparities In Neurology., Jean-Baptist Lepichon, Stephanie Horton, Omar Abdelmoity, Mark A. Hoffman, Emily Cramer, Nirmeen Kishk, Salah Hamada, Ahmed Abdelmoity Oct 2022

The Use Of Virtual Tools In Narrowing The Impact Of Health Disparities In Neurology., Jean-Baptist Lepichon, Stephanie Horton, Omar Abdelmoity, Mark A. Hoffman, Emily Cramer, Nirmeen Kishk, Salah Hamada, Ahmed Abdelmoity

Manuscripts, Articles, Book Chapters and Other Papers

The concept of Epilepsy Treatment Gap (ETG) refers to the proportion of people with epilepsy who are not being appropriately treated. The ETG in the USA approaches 10%, with historically underserved populations and rural populations disproportionately affected. The ETG in Low-and Middle-Income Countries (LMIC) is reported to be 5-10 times higher than in high-income countries. The growing availability of reliable internet access offers a unique opportunity to provide better care to children and adults with epilepsy. In this paper we explore various telehealth (TH) initiatives that have leveraged the availability of easy and free access to an internet connection in …


Respiratory Virus Surveillance Among Children With Acute Respiratory Illnesses - New Vaccine Surveillance Network, United States, 2016-2021., Ariana Perez, Joana Y. Lively, Aaron Curns, Geoffrey A. Weinberg, Natasha B. Halasa, Mary Allen Staat, Peter G. Szilagyi, Laura S. Stewart, Monica M. Mcneal, Benjamin Clopper, Yingtao Zhou, Brett L. Whitaker, Elizabeth Lemasters, Elizabeth Harker, Janet A. Englund, Eileen J. Klein, Rangaraj Selvarangan, Christopher J. Harrison, Julie A. Boom, Leila C. Sahni, Marian G. Michaels, John V. Williams, Gayle E. Langley, Susan I. Gerber, Angela Campbell, Aron J. Hall, Brian Rha, Meredith Mcmorrow, New Vaccine Surveillance Network Collaborators Oct 2022

Respiratory Virus Surveillance Among Children With Acute Respiratory Illnesses - New Vaccine Surveillance Network, United States, 2016-2021., Ariana Perez, Joana Y. Lively, Aaron Curns, Geoffrey A. Weinberg, Natasha B. Halasa, Mary Allen Staat, Peter G. Szilagyi, Laura S. Stewart, Monica M. Mcneal, Benjamin Clopper, Yingtao Zhou, Brett L. Whitaker, Elizabeth Lemasters, Elizabeth Harker, Janet A. Englund, Eileen J. Klein, Rangaraj Selvarangan, Christopher J. Harrison, Julie A. Boom, Leila C. Sahni, Marian G. Michaels, John V. Williams, Gayle E. Langley, Susan I. Gerber, Angela Campbell, Aron J. Hall, Brian Rha, Meredith Mcmorrow, New Vaccine Surveillance Network Collaborators

Manuscripts, Articles, Book Chapters and Other Papers

The New Vaccine Surveillance Network (NVSN) is a prospective, active, population-based surveillance platform that enrolls children with acute respiratory illnesses (ARIs) at seven pediatric medical centers. ARIs are caused by respiratory viruses including influenza virus, respiratory syncytial virus (RSV), human metapneumovirus (HMPV), human parainfluenza viruses (HPIVs), and most recently SARS-CoV-2 (the virus that causes COVID-19), which result in morbidity among infants and young children (1-6). NVSN estimates the incidence of pathogen-specific pediatric ARIs and collects clinical data (e.g., underlying medical conditions and vaccination status) to assess risk factors for severe disease and calculate influenza and COVID-19 vaccine effectiveness. Current NVSN …


Acute Otitis Media: Low Dose Versus High Dose Amoxicillin, Children's Mercy Kansas City Oct 2022

Acute Otitis Media: Low Dose Versus High Dose Amoxicillin, Children's Mercy Kansas City

Clinical Critically Appraised Topics

No abstract provided.


Diabetic Ketoacidosis Intravenous Fluids, Children's Mercy Kansas City Oct 2022

Diabetic Ketoacidosis Intravenous Fluids, Children's Mercy Kansas City

Clinical Critically Appraised Topics

No abstract provided.


Croup: Observation Time Post Racemic Epinephrine Dosing In Children With Croup, Children's Mercy Kansas City Oct 2022

Croup: Observation Time Post Racemic Epinephrine Dosing In Children With Croup, Children's Mercy Kansas City

Clinical Critically Appraised Topics

No abstract provided.


Microrna Expression Levels Change In Neonatal Patients During And After Exposure To Cardiopulmonary Bypass., Lance Hsieh, Lan N. Tu, Alison Paquette, Quanhu Sheng, Shilin Zhao, Douglas C. Bittel, James O'Brien, Kasey Vickers, Peter Pastuszko, Vishal Nigam Sep 2022

Microrna Expression Levels Change In Neonatal Patients During And After Exposure To Cardiopulmonary Bypass., Lance Hsieh, Lan N. Tu, Alison Paquette, Quanhu Sheng, Shilin Zhao, Douglas C. Bittel, James O'Brien, Kasey Vickers, Peter Pastuszko, Vishal Nigam

Manuscripts, Articles, Book Chapters and Other Papers

Background The systemic inflammation that occurs after exposure to cardiopulmonary bypass (CPB), which is especially severe in neonatal patients, is associated with poorer outcomes and is not well understood. In order to gain deeper insight into how exposure to bypass activates inflammatory responses in circulating leukocytes, we studied changes in microRNA (miRNA) expression during and after exposure to bypass. miRNAs are small noncoding RNAs that have important roles in modulating protein levels and function of cells. Methods and Results We performed miRNA-sequencing on leukocytes isolated from neonatal patients with CPB (n=5) at 7 time points during the process of CPB, …


Pharmacokinetic Evaluation Of Intravenous Vitamin C: A Classic Pharmacokinetic Study., Ping Chen, Greg Reed, Joyce Jiang, Yaohui Wang, Jean Sunega, Ruochen Dong, Yan Ma, Anna E. Esparham, Ryan Ferrell, Mark Levine, Jeanne Drisko, Qi Chen Sep 2022

Pharmacokinetic Evaluation Of Intravenous Vitamin C: A Classic Pharmacokinetic Study., Ping Chen, Greg Reed, Joyce Jiang, Yaohui Wang, Jean Sunega, Ruochen Dong, Yan Ma, Anna E. Esparham, Ryan Ferrell, Mark Levine, Jeanne Drisko, Qi Chen

Manuscripts, Articles, Book Chapters and Other Papers

Purpose: Intravenous vitamin C (IVC) is used in a variety of disorders with limited supporting pharmacokinetic data. Herein we report a pharmacokinetic study in healthy volunteers and cancer participants with IVC doses in the range of 1-100 g.

Methods: A pharmacokinetic study was conducted in 21 healthy volunteers and 12 oncology participants. Healthy participants received IVC infusions of 1-100 g; oncology participants received IVC infusions of 25-100 g. Serial blood and complete urine samples were collected pre-infusion and for 24 h post-infusion. Pharmacokinetic parameters were computed using noncompartmental methods. Adverse events were monitored during the study.

Results: In both cohorts, …


Screening Considerations For Patients With Sexually Transmitted Infections, Children's Mercy Kansas City Sep 2022

Screening Considerations For Patients With Sexually Transmitted Infections, Children's Mercy Kansas City

Clinical Critically Appraised Topics

No abstract provided.


Chap-Child: An Open Source Method For Estimating Sit-To-Stand Transitions And Sedentary Bout Patterns From Hip Accelerometers Among Children., Jordan A. Carlson, Nicola D. Ridgers, Supun Nakandala, Rong Zablocki, Fatima Tuz-Zahra, John Bellettiere, Paul R Hibbing, Chelsea Steel, Marta M. Jankowska, Dori E. Rosenberg, Mikael Anne Greenwood-Hickman, Jingjing Zou, Andrea Z. Lacroix, Arun Kumar, Loki Natarajan Aug 2022

Chap-Child: An Open Source Method For Estimating Sit-To-Stand Transitions And Sedentary Bout Patterns From Hip Accelerometers Among Children., Jordan A. Carlson, Nicola D. Ridgers, Supun Nakandala, Rong Zablocki, Fatima Tuz-Zahra, John Bellettiere, Paul R Hibbing, Chelsea Steel, Marta M. Jankowska, Dori E. Rosenberg, Mikael Anne Greenwood-Hickman, Jingjing Zou, Andrea Z. Lacroix, Arun Kumar, Loki Natarajan

Manuscripts, Articles, Book Chapters and Other Papers

Background: Hip-worn accelerometer cut-points have poor validity for assessing children's sedentary time, which may partly explain the equivocal health associations shown in prior research. Improved processing/classification methods for these monitors would enrich the evidence base and inform the development of more effective public health guidelines. The present study aimed to develop and evaluate a novel computational method (CHAP-child) for classifying sedentary time from hip-worn accelerometer data.

Methods: Participants were 278, 8-11-year-olds recruited from nine primary schools in Melbourne, Australia with differing socioeconomic status. Participants concurrently wore a thigh-worn activPAL (ground truth) and hip-worn ActiGraph (test measure) during up to 4 …


Location-Specific Psychosocial And Environmental Correlates Of Physical Activity And Sedentary Time In Young Adolescents: Preliminary Evidence For Location-Specific Approaches From A Cross-Sectional Observational Study., Adrian Ortega, Carolina M Bejarano, Christopher C Cushing, Vincent S. Staggs, Amy Papa, Chelsea Steel, Robin P. Shook, Terry L. Conway, Brian E. Saelens, Karen Glanz, Kelli L. Cain, Lawrence D. Frank, Jacqueline Kerr, Jasper Schipperijn, James F. Sallis, Jordan A. Carlson Aug 2022

Location-Specific Psychosocial And Environmental Correlates Of Physical Activity And Sedentary Time In Young Adolescents: Preliminary Evidence For Location-Specific Approaches From A Cross-Sectional Observational Study., Adrian Ortega, Carolina M Bejarano, Christopher C Cushing, Vincent S. Staggs, Amy Papa, Chelsea Steel, Robin P. Shook, Terry L. Conway, Brian E. Saelens, Karen Glanz, Kelli L. Cain, Lawrence D. Frank, Jacqueline Kerr, Jasper Schipperijn, James F. Sallis, Jordan A. Carlson

Manuscripts, Articles, Book Chapters and Other Papers

Background: A better understanding of the extent to which psychosocial and environmental correlates of physical activity are specific to locations would inform intervention optimization.

Purpose: To investigate cross-sectional associations of location-general and location-specific variables with physical activity and sedentary time in three common locations adolescents spend time.

Methods: Adolescents (N = 472,Mage = 14.1,SD = 1.5) wore an accelerometer and global positioning systems (GPS) tracker and self-reported on psychosocial (e.g., self-efficacy) and environmental (e.g., equipment) factors relevant to physical activity and sedentary time. We categorized each survey item based on whether it was specific to a location to generate psychosocial …


Comparing Kidney Health Outcomes In Children, Adolescents, And Adults With Focal Segmental Glomerulosclerosis., Debbie S. Gipson, Jonathan P. Troost, Cathie Spino, Samara Attalla, Joshua Tarnoff, Susan Massengill, Richard Lafayette, Virginia Vega-Warner, Sharon Adler, Patrick Gipson, Matthew Elliott, Frederick Kaskel, Damian Fermin, Marva Moxey-Mims, Richard N. Fine, Elizabeth J. Brown, Kimberly Reidy, Katherine Tuttle, Keisha Gibson, Kevin V. Lemley, Larry A. Greenbaum, Meredith A. Atkinson, Sangeeta Hingorani, Tarak Srivastava, Christine B. Sethna, Kevin Meyers, Cheryl Tran, Katherine M. Dell, Chia-Shi Wang, Jennifer Lai Yee, Matthew G. Sampson, Rasheed Gbadegesin, J J Lin, Tammy Brady, Michelle Rheault, Howard Trachtman Aug 2022

Comparing Kidney Health Outcomes In Children, Adolescents, And Adults With Focal Segmental Glomerulosclerosis., Debbie S. Gipson, Jonathan P. Troost, Cathie Spino, Samara Attalla, Joshua Tarnoff, Susan Massengill, Richard Lafayette, Virginia Vega-Warner, Sharon Adler, Patrick Gipson, Matthew Elliott, Frederick Kaskel, Damian Fermin, Marva Moxey-Mims, Richard N. Fine, Elizabeth J. Brown, Kimberly Reidy, Katherine Tuttle, Keisha Gibson, Kevin V. Lemley, Larry A. Greenbaum, Meredith A. Atkinson, Sangeeta Hingorani, Tarak Srivastava, Christine B. Sethna, Kevin Meyers, Cheryl Tran, Katherine M. Dell, Chia-Shi Wang, Jennifer Lai Yee, Matthew G. Sampson, Rasheed Gbadegesin, J J Lin, Tammy Brady, Michelle Rheault, Howard Trachtman

Manuscripts, Articles, Book Chapters and Other Papers

Importance: Focal segmental glomerulosclerosis (FSGS) is a common cause of end-stage kidney disease (ESKD) across the lifespan. While 10% to 15% of children and 3% of adults who develop ESKD have FSGS, it remains uncertain whether the natural history differs in pediatric vs adult patients, and this uncertainty contributes to the exclusion of children and adolescents in clinical trials.

Objective: To examine whether there are differences in the kidney health outcomes among children, adolescents, and adults with FSGS.

Design, Setting, and Participants: This cohort study used pooled and parallel analyses, completed July 5, 2022, from 3 complimentary data sources: (1) …


The Use Of A Simultaneous Fascia Lata-Free Flap With Cochlear Implantation After Radical Mastoidectomy., Jason G. May, John Kerr, Jack A. May, Patrick Tassone, Arnaldo L. Rivera Aug 2022

The Use Of A Simultaneous Fascia Lata-Free Flap With Cochlear Implantation After Radical Mastoidectomy., Jason G. May, John Kerr, Jack A. May, Patrick Tassone, Arnaldo L. Rivera

Manuscripts, Articles, Book Chapters and Other Papers

Patients undergoing cochlear implant after prior radical mastoidectomy are at increased risk of device infection requiring device explant. Various techniques including two-stage operations have been used. We report the novel technique with use of a vascularized fascia lata free flap for a patient undergoing cochlear implantation with radical mastoidectomy.


Safety And Efficacy Of Voxelotor In Pediatric Patients With Sickle Cell Disease Aged 4 To 11 Years., Jeremie H. Estepp, Ram Kalpatthi, Gerald Woods, Sara Trompeter, Robert I. Liem, Kacie Sims, Adlette Inati, Baba P D Inusa, Andrew Campbell, Connie Piccone, Miguel R. Abboud, Kim Smith-Whitley, Sandra Dixon, Margaret Tonda, Carla Washington, Noelle M. Griffin, Clark Brown Aug 2022

Safety And Efficacy Of Voxelotor In Pediatric Patients With Sickle Cell Disease Aged 4 To 11 Years., Jeremie H. Estepp, Ram Kalpatthi, Gerald Woods, Sara Trompeter, Robert I. Liem, Kacie Sims, Adlette Inati, Baba P D Inusa, Andrew Campbell, Connie Piccone, Miguel R. Abboud, Kim Smith-Whitley, Sandra Dixon, Margaret Tonda, Carla Washington, Noelle M. Griffin, Clark Brown

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Sickle cell disease (SCD) is a devastating, multisystemic disorder that affects millions of people worldwide. The earliest clinical manifestations of SCD can affect infants as young as 6 months of age, and pediatric patients are at risk for acute and life-threatening complications. Early intervention with treatments that target the underlying pathophysiological mechanism of SCD, sickle hemoglobin (HbS) polymerization, are expected to slow disease progression and circumvent disease-associated morbidity and mortality.

PROCEDURE: The HOPE-KIDS 1 trial (NCT02850406) is an ongoing four-part, phase 2a, open-label, single- and multiple-dose study to evaluate the pharmacokinetics, efficacy, and safety of voxelotor-a first-in-class HbS polymerization …


Association Of Acute Anti-Inflammatory Treatment With Medium-Term Outcomes For Coronary Artery Aneurysms In Kawasaki Disease., Kevin G. Friedman, Brian W. Mccrindle, Kyle Runeckles, Nagib Dahdah, Ashraf S. Harahsheh, Michael Khoury, Sean Lang, Cedric Manlhiot, Adriana H. Tremoulet, Geetha Raghuveer, Elif Seda Selamet Tierney, Pei-Ni Jone, Jennifer S. Li, Jacqueline R. Szmuszkovicz, Kambiz Norozi, Supriya S. Jain, Angela T. Yetman, Jane W. Newburger, International Kawasaki Disease Registry Aug 2022

Association Of Acute Anti-Inflammatory Treatment With Medium-Term Outcomes For Coronary Artery Aneurysms In Kawasaki Disease., Kevin G. Friedman, Brian W. Mccrindle, Kyle Runeckles, Nagib Dahdah, Ashraf S. Harahsheh, Michael Khoury, Sean Lang, Cedric Manlhiot, Adriana H. Tremoulet, Geetha Raghuveer, Elif Seda Selamet Tierney, Pei-Ni Jone, Jennifer S. Li, Jacqueline R. Szmuszkovicz, Kambiz Norozi, Supriya S. Jain, Angela T. Yetman, Jane W. Newburger, International Kawasaki Disease Registry

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The impact of adjunctive anti-inflammatory treatment on outcomes for patients with Kawasaki disease (KD) and coronary artery aneurysms (CAAs) is unknown.

METHODS: Using data from the International KD Registry in patients with ≥ medium CAA we evaluate associations of treatment with outcomes and major adverse cardiac events (MACE).

RESULTS: Medium or large CAA was present in 527 (32%) patients. All were treated with intravenous immunoglobulin (IVIG), 70% were male, and the median age was 1.3 years (interquartile range: 0.4-4.0 years). The most common acute therapies included single IVIG alone in 243 (46%), multiple IVIG in 100 (19%), multiple IVIG …


Croup: Predictors Of Admission For Patients With Croup, Children's Mercy Kansas City Aug 2022

Croup: Predictors Of Admission For Patients With Croup, Children's Mercy Kansas City

Clinical Critically Appraised Topics

No abstract provided.


Receptor Tyrosine Kinase (Rtk) Targeting In Pediatric High-Grade Glioma And Diffuse Midline Glioma: Pre-Clinical Models And Precision Medicine., Kallen Schwark, Dana Messinger, Jessica R. Cummings, Joshua Bradin, Abed Kawakibi, Clarissa M. Babila, Samantha Lyons, Sunjong Ji, Rodrigo T. Cartaxo, Seongbae Kong, Evan Cantor, Carl Koschmann, Viveka Nand Yadav Aug 2022

Receptor Tyrosine Kinase (Rtk) Targeting In Pediatric High-Grade Glioma And Diffuse Midline Glioma: Pre-Clinical Models And Precision Medicine., Kallen Schwark, Dana Messinger, Jessica R. Cummings, Joshua Bradin, Abed Kawakibi, Clarissa M. Babila, Samantha Lyons, Sunjong Ji, Rodrigo T. Cartaxo, Seongbae Kong, Evan Cantor, Carl Koschmann, Viveka Nand Yadav

Manuscripts, Articles, Book Chapters and Other Papers

Pediatric high-grade glioma (pHGG), including both diffuse midline glioma (DMG) and non-midline tumors, continues to be one of the deadliest oncologic diagnoses (both henceforth referred to as "pHGG"). Targeted therapy options aimed at key oncogenic receptor tyrosine kinase (RTK) drivers using small-molecule RTK inhibitors has been extensively studied, but the absence of proper in vivo modeling that recapitulate pHGG biology has historically been a research challenge. Thankfully, there have been many recent advances in animal modeling, including Cre-inducible transgenic models, as well as intra-uterine electroporation (IUE) models, which closely recapitulate the salient features of human pHGG tumors. Over 20% of …


Real-World Use Of Tisagenlecleucel In Infant Acute Lymphoblastic Leukemia., Amy Moskop, Lauren Pommert, Christina Baggott, Snehit Prabhu, Holly L. Pacenta, Christine L. Phillips, Jenna Rossoff, Heather E. Stefanski, Julie-An Talano, Steve P. Margossian, Michael R. Verneris, Douglas Myers, Nicole A. Karras, Patrick A. Brown, Muna Qayed, Michelle L. Hermiston, Prakash Satwani, Christa Krupski, Amy K. Keating, Rachel Wilcox, Cara A. Rabik, Vanessa A. Fabrizio, Vasant Chinnabhandar, A Yasemin Goksenin, Kevin J. Curran, Crystal L. Mackall, Theodore W. Laetsch, Erin M. Guest, Erin H. Breese, Liora M. Schultz Jul 2022

Real-World Use Of Tisagenlecleucel In Infant Acute Lymphoblastic Leukemia., Amy Moskop, Lauren Pommert, Christina Baggott, Snehit Prabhu, Holly L. Pacenta, Christine L. Phillips, Jenna Rossoff, Heather E. Stefanski, Julie-An Talano, Steve P. Margossian, Michael R. Verneris, Douglas Myers, Nicole A. Karras, Patrick A. Brown, Muna Qayed, Michelle L. Hermiston, Prakash Satwani, Christa Krupski, Amy K. Keating, Rachel Wilcox, Cara A. Rabik, Vanessa A. Fabrizio, Vasant Chinnabhandar, A Yasemin Goksenin, Kevin J. Curran, Crystal L. Mackall, Theodore W. Laetsch, Erin M. Guest, Erin H. Breese, Liora M. Schultz

Manuscripts, Articles, Book Chapters and Other Papers

Infants with B-cell acute lymphoblastic leukemia (B-ALL) have poor outcomes because of chemotherapy resistance leading to high relapse rates. Tisagenlecleucel, a CD19-directed chimeric antigen receptor T-cell (CART) therapy, is US Food and Drug Administration approved for relapsed or refractory B-ALL in patients ≤25 years; however, the safety and efficacy of this therapy in young patients is largely unknown because children(n = 14). Sixty-four percent of patients (n = 9) achieved minimal residual disease-negative remission after CART and 50% of patients remain in remission at last follow-up. All patients with high disease burden at time of CART infusion (>M1 marrow) …


Integrated Persevere And Endothelial Biomarker Risk Model Predicts Death And Persistent Mods In Pediatric Septic Shock: A Secondary Analysis Of A Prospective Observational Study., Mihir R. Atreya, Natalie Z. Cvijanovich, Julie C. Fitzgerald, Scott L. Weiss, Michael T. Bigham, Parag N. Jain, Adam J. Schwarz, Riad Lutfi, Jeffrey Nowak, Geoffrey L. Allen, Neal J. Thomas, Jocelyn R. Grunwell, Torrey Baines, Michael Quasney, Bereketeab Haileselassie, Christopher J. Lindsell, Matthew N. Alder, Hector R. Wong Jul 2022

Integrated Persevere And Endothelial Biomarker Risk Model Predicts Death And Persistent Mods In Pediatric Septic Shock: A Secondary Analysis Of A Prospective Observational Study., Mihir R. Atreya, Natalie Z. Cvijanovich, Julie C. Fitzgerald, Scott L. Weiss, Michael T. Bigham, Parag N. Jain, Adam J. Schwarz, Riad Lutfi, Jeffrey Nowak, Geoffrey L. Allen, Neal J. Thomas, Jocelyn R. Grunwell, Torrey Baines, Michael Quasney, Bereketeab Haileselassie, Christopher J. Lindsell, Matthew N. Alder, Hector R. Wong

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Multiple organ dysfunction syndrome (MODS) is a critical driver of sepsis morbidity and mortality in children. Early identification of those at risk of death and persistent organ dysfunctions is necessary to enrich patients for future trials of sepsis therapeutics. Here, we sought to integrate endothelial and PERSEVERE biomarkers to estimate the composite risk of death or organ dysfunctions on day 7 of septic shock.

METHODS: We measured endothelial dysfunction markers from day 1 serum among those with existing PERSEVERE data. TreeNet® classification model was derived incorporating 22 clinical and biological variables to estimate risk. Based on relative variable importance, …


Challenges Of Diagnosing Pseudohypoaldosteronism (Pha) In An Infant., Ghufran Babar, Minah Tariq Jul 2022

Challenges Of Diagnosing Pseudohypoaldosteronism (Pha) In An Infant., Ghufran Babar, Minah Tariq

Manuscripts, Articles, Book Chapters and Other Papers

Background. Pseudohypoaldosteronism (PHA) is characterized by renal tubular resistance to aldosterone. As a result, the symptoms typically involve hyperkalemia and hyponatremia. The aim of this clinical case report is to highlight the severe electrolyte imbalance PHA can present within an infant, as well as difficulties in diagnosing the condition. Case Presentation. A 5-week-old male arrived at the ER with episodes of emesis, lethargy, and difficulty in feeding. He had significant electrolyte abnormalities and was being treated by his PCP for failure to thrive. He presented with urinary sodium wasting, indicated by hyponatremia, hyperkalemia, low chloride, and hypercalcemia. Patient …


Validation Of Remote Height And Weight Assessment In A Rural Randomized Clinical Trial., Bethany Forseth Hanson, Ann M. Davis, Dana Bakula, Megan Murray, Kelsey Dean, Rebecca E. Swinburne Romine, Kandace Fleming Jul 2022

Validation Of Remote Height And Weight Assessment In A Rural Randomized Clinical Trial., Bethany Forseth Hanson, Ann M. Davis, Dana Bakula, Megan Murray, Kelsey Dean, Rebecca E. Swinburne Romine, Kandace Fleming

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The purpose of this study is to describe and assess a remote height and weight protocol that was developed for an ongoing trial conducted during the SARS COV-2 pandemic.

METHODS: Thirty-eight rural families (children 8.3 ± 0.7 years; 68% female; and caregivers 38.2 ± 6.1 years) were provided detailed instructions on how to measure height and weight. Families obtained measures via remote data collection (caregiver weight, child height and weight) and also by trained staff. Differences between data collection methods were examined.

RESULTS: Per absolute mean difference analyses, slightly larger differences were found for child weight (0.21 ± 0.21 …