Medical Specialties Commons^™

Plasma Microbial Cell-Free Dna Sequencing For The Detection Of Kingella Kingae Pediatric Spinal Infections, Asim A Ahmed, Sarah Y Park, Matthew J Smollin, Martin S Lindner, Adriana Sarmiento Clemente, Ana Del Valle Penella, P Marcelo Laufer, Carolina Sanchez-Vegas, Manuel R Cotilla, Marian E Melish, Connie Trieu, Nazia A Kabani, Joshua Cooper, Audrey R Lloyd, David W Kimberlin, John C Arnold, Alejandro Jordan-Villegas, Lori E Patterson, Catherine E Foster, Pablo Yagupsky

Faculty, Staff and Students Publications

Background: Diagnosis of Kingella kingae skeletal system infections is made challenging by the microbe's fastidious nature. Detection and quantification of circulating microbial cell-free DNA (mcfDNA) in plasma by the Karius Test, a commercial metagenomic sequencing test, may offer promise in diagnosing pediatric spinal infections caused by difficult-to-culture organisms such as K. kingae .

Methods: Plasma mcfDNA sequencing detections of K. kingae from April 2018 to December 2020 were reviewed to identify pediatric (age < 18 years) patients. Medical charts of those with spinal infections were reviewed, and mcfDNA sequencing diagnostic performance was compared with usual care tests (ie, cultures, polymerase chain reaction).

Results: Ten children with K. kingae spinal infections were identified across 7 institutions. The median age was 16.5 months (range 11-23 months). All case-patients had vertebral osteomyelitis …

Ophthalmology Examinations In Children With Skull Fractures And Underlying Focal Hemorrhage, Karli Breeden, Cindy W Christian, Joanne N Wood, Gil Binenbaum, Daniel M Lindberg, Angela Bachim, Colleen J Bressler, Lori Frasier, Terra Frazier, Nicole R Johnson, Natalie Laub, Megan M Letson, Tagrid Ruiz-Maldonado, Matthew Valente, Jenna Kiely, Jan Leonard, M Katherine Henry

Faculty, Staff and Students Publications

Objectives: To assess the frequency and yield of retinal examination in children below 2 years old undergoing abuse evaluations in the setting of skull fracture(s) and small underlying intracranial hemorrhage.

Methods: This cross-sectional study used CAPNET, a multicenter child physical abuse network, to identify children below 2 years with a skull fracture(s) and intracranial injury limited to an underlying small focal intracranial hemorrhage undergoing subspeciality child abuse evaluations. Our outcomes of interest were (1) the performance of a retinal examination, (2) the identification of retinal hemorrhages, and (3) associations of clinical factors and CAPNET site with the performance of retinal …

Neuroanatomical Features Of Naa10 And Naa15-Related Neurodevelopmental Syndromes, Rahi Patel, Rikhil Makwana, Carolina Christ, Elaine Marchi, Christina Y Miyake, Fabricio Guimaraes Goncalves, Gholson J Lyon, Matthew T Whitehead

Faculty, Staff and Students Publications

Background: NAA10-related and NAA15-related neurodevelopmental (ND) syndromes present with intellectual disability, hypotonia, cardiac abnormalities, and delayed development. While data exists on the clinical manifestations of these conditions, there are few reports describing the neuroanatomical abnormalities present on imaging.

Objective: We aim to provide neuroimaging analyses for a subset of probands with NAA10- and NAA15-related neurodevelopmental symptoms and assess the severity and number of neuroanatomical anomalies and their associated functional impairments to better understand the pathophysiology of these disease processes.

Materials and methods: Neuroimaging studies were obtained from 26 probands (18 with pathogenic variants in NAA10, 8 with pathogenic variants in …

Measles, Mumps, Rubella Immunization And The Resurgence Of Measles In America, Peter J Hotez

Faculty, Staff and Students Publications

No abstract provided.

Diabetes-Related Distress Over Time And Its Associations With Glucose Levels In School-Aged Children., Susana R. Patton, Nicole Kahhan, Amy Milkes, Ryan J. Mcdonough, Matthew Benson, Mark A. Clements, Jessica S. Pierce

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: In a cohort of families of school-age children (8-12.99 years old) with type 1 diabetes, we examined the stability of parent and child diabetes-related distress (DRD) over 6 months and the associations between parent and child DRD and child glycated hemoglobin (HbA1c) over time.

RESEARCH DESIGN AND METHODS: We recruited families from two large pediatric hospital systems in the USA and used validated measures of parent (Parent Problem Areas in Diabetes-Child, PPAID-C) and child (Problem Areas in Diabetes-Child, PAID-C) DRD and children's HbA1c. We collected data at baseline and 6 months. We calculated minimal clinically important differences in PPAID-C …

Surveying Management Strategies For Pediatric Autoimmune Hepatitis, Lillie Baca

University Honors Theses

Pediatric autoimmune hepatitis (PAIH) is a long-term condition that requires ongoing treatment and regular monitoring. It is characterized by its elevation of liver enzymes, interface hepatitis, and the specific presence of circulating autoantibodies. There is a general lack of consensus on appropriate measures of diagnostic criteria, treatment medication, and beneficial practices for monitoring the disease. The existing literature contains several subtle discrepancies that challenge a practitioner's judgement for care management of children with autoimmune hepatitis. This study is a retrospective chart review aimed at understanding the current diagnostics, treatment, and long-term management strategies of PAIH at a single center. Also, …

Early Life Exposure To Fine Particulate Matter And Fine Motor Function, Attentional Function, And Working Memory Among Spanish School-Aged Children, Kristina W Whitworth, Aitana Lertxundi, Mingze Yuan, Alison Rector-Houze, Wei-Jen Chen, Mònica Guxens, Jordi Julvez, Michael Swartz, Elaine Symanski, Antonia Valentin, Carmen Iniguez, Llúcia L González-Safont, Jesús Ibarluzea

Faculty, Staff and Student Publications

Background: Evidence of the association between fine particulate matter (PM2.5) exposure and child neuropsychological function is equivocal. We examined early life PM2.5 exposure in relation to fine motor function, attention, and working memory in early childhood.

Methods: We used data from the Spanish INfancia y Medio Ambiente Project, 2003-2008. Exposure to PM2.5 (μg/m3) was assessed using spatiotemporal land-use random forest models and assigned based on residential address histories. Around age six, children completed the finger tapping test, attentional network test (ANT), and n-back task to evaluate fine motor speed, attention, and working memory, respectively. A total of 1,310 children had …

Reliability Of Physician Estimation Of Pelvic Free Fluid Volume On The Pediatric Focused Assessment With Sonography For Trauma., Ashkon Shaahinfar, Jennifer Rosin Wiebelhaus, Newton Addo, Ronald A. Cohen, Pinar Karakas-Rothey, Aaron E. Kornblith

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: To evaluate the reliability of physician gestalt estimation of pelvic free fluid volume on pediatric Focused Assessment with Sonography for Trauma (FAST). To determine a reliable cut-off volume and characteristics associated with small pelvic free fluid.

METHODS: Our study assessed the ability of 2 ultrasound-trained pediatric emergency medicine (PEM) physicians and 2 pediatric radiologists to characterize pelvic free fluid in a retrospective convenience sample of archived FAST from a Level 1 pediatric trauma center, April 2018-June 2020. Inter- and intra-rater reliability were measured to determine the most reliable volume cut-off. Chi-squared and Fisher's exact tests determined characteristics associated with …

Validity And Reliability Of The Tampa Scale For Kinesiophobia For Adolescents With Heart Disease., David A. White, William R. Black, Emily Cramer, Lindsey Malloy-Walton, Mollie Walton, Laura Martis, Brandy Enneking, Kelli M. Teson, Jessica S. Watson, Jami Gross-Toalson

Manuscripts, Articles, Book Chapters and Other Papers

PURPOSE: To assess the validity and reliability of a newly adapted Tampa Scale for KP for adolescents with HD (TSK-Heart-A).

METHODS: The TSK-Heart-A survey consists of 17 Likert scale items, producing a summary score (SS) ranging from 17 (low KP) to 68 (high KP). Following content and face validity, adolescents (age 12-18 yr) with arrhythmia disorders or Fontan palliation were recruited. The PROMIS pediatric anxiety and depression short forms, Pediatric Quality of Life Inventory (PedsQL) generic core and cardiac module, and a PA questionnaire for adolescents (PAQ-A) were used for criterion validity. The TSK-Heart-A was completed twice (20.4 ± 6.3 …

Glp-1 Agonists For Pediatric Psychopharmacology: Opportunities And Cautions., Michael Bartkoski, Ethan Poweleit, Stephani L. Stancil

Manuscripts, Articles, Book Chapters and Other Papers

Glucagon-like peptide-1 agonists (GLP-1A) are increasingly prescribed to treat obesity and type 2 diabetes mellitus in children and adolescents. Emerging evidence suggesting neuropsychiatric effects presents an opportunity for pediatric psychopharmacology but requires careful consideration of potential adverse effects and risk of misuse. This perspective examines potential applications and cautions surrounding GLP-1As for pediatric mental health, particularly substance use, depression, and eating disorders, and advocates for research and clinical monitoring to ensure their safety and efficacy.

"Sex Out Of Boredom": Key Stakeholders' Perspectives On Teen Pregnancy Prevention In Emerging Immigrant Latino Rural Communities., Romina Barral, J Dennis Fortenberry, Astrid Guerrero Avitia, Mariana Ramirez, Abbey R. Masonbrink, Claire D. Brindis

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Teen pregnancy (TP) rates are 1.5 times higher among Latina youth than the United States national average and one-third times higher in rural counties. The Socio-Ecological framework recognizes the myriad of issues that impact TP, including four bidirectional levels of influence on teenagers' behaviors: macro, community, institutional, and interpersonal levels. We aim to fill critical knowledge gaps regarding the influence of key community stakeholders living in rural, Latino-majority communities shaping Latino/a, immigrant adolescents' TP-related environments.

METHODS: A purposive sample of 48 key stakeholders was drawn from three rural counties (Finney, Ford, and Seward) in southwestern Kansas from 2016 to …

Covid-19 Pandemic Impacts On Behavioral And Emotional Health Of Young Children With Autism., Olivia M. Pokoski, Hayley M. Crain, Sarah M. Furnier, Ronald E. Gangnon, Cy Nadler, Eric J. Moody, Karen Pazol, Maria A. Stanley, Lisa D. Wiggins, Maureen S. Durkin

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: To test initial hypotheses that the coronavirus disease 2019 (COVID-19) pandemic was associated with decreases in adaptive behavior and increases in behavioral and emotional problems of children with autism; greater impacts for children who lost specialty services; and greater behavioral and emotional problems for children with autism vs control participants.

METHOD: Eligible participants (N = 1,158) enrolled in phase 3 of the multisite, case-control Study to Explore Early Development (SEED) before March 31, 2020, were between 2 and 5 years old, and completed follow-up assessments between January and July 2021. Caregivers completed a COVID-19 Impact Assessment Questionnaire, Vineland Adaptive …

Cardiovascular And Thromboembolic Events In Children And Adults With Glomerular Disease: Findings From The Cure Glomerulonephropathy (Curegn) Network., Shikha Wadhwani, Sarah A. Mansfield, Abigail R. Smith, Bruce M. Robinson, Eman Abdelghani, Amira Al-Uzri, Isa F. Ashoor, Sharon M. Bartosh, Aftab S. Chishti, Salim S. Hayek, Michelle A. Hladunewich, Bryce A. Kerlin, Siddharth S. Madapoosi, Laura H. Mariani, Amy K. Mottl, Michelle N. Rheault, Michelle M. O'Shaughnessy, Christopher John Sperati, Tarak Srivastava, David T. Selewski, Chia-Shi Wang, Craig S. Wong, Donald J. Weaver, Myda Khalid, Glomerulonephropathy (Curegn) Study Consortium

Manuscripts, Articles, Book Chapters and Other Papers

RATIONALE & OBJECTIVE: Cardiovascular (CV) and thromboembolic (TE) events are known complications of glomerular disease (GD), but their incidence and risk factors are poorly characterized. This analysis describes CV and TE outcomes in the Cure GlomeruloNephropathy (CureGN) Network.

STUDY DESIGN: Prospective cohort study.

SETTING & PARTICIPANTS: CureGN is a prospective cohort study of children and adults with biopsy-proven minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), membranous nephropathy (MN), or IgA nephropathy (IgAN)/vasculitis (IgAV). Data from 2,545 children and adults (23% MCD, 23% MN, 25% FSGS, 29% IgAN/IgAV) was analyzed.

EXPOSURE: Estimated glomerular filtration rate (eGFR), proteinuria, serum albumin, tobacco …

Clinical Manifestations, Disease Penetrance, And Treatment In Individuals With Socs1 Insufficiency: A Registry-Based And Population-Based Study, Jerome Hadjadj, Anna Wolfers, Oleg Borisov, Derek Hazard, Ronan Leahy, Marie Jeanpierre, Alexandre Belot, Shahrzad Bakhtiar, Fabian Hauck, Pui Y Lee, Stefano Volpi, Serena Palmeri, Vincent Barlogis, Nathalie Aladjidi, Georg Ebetsberger-Dachs, Jerome Avouac, Fabienne Charbit-Henrion, Morgane Cheminant, Jean Donadieu, Sujal Ghosh, David P Hoytema Van Konijnenburg, Julia Körholz, Jacinta Bustamante, Jeremie Rosain, Lisa Forbes Satter, Ingrid Selmeryd, Georgios Sogkas, Benedicte Neven, Frederic Rieux-Laucat, Stephan Ehl

Faculty, Staff and Students Publications

Background: Suppressor of cytokine signalling 1 (SOCS1) insufficiency is an inborn error of immunity affecting the negative regulation of cytokine and growth factor signalling. We aimed to enhance the understanding of clinical manifestations, disease trajectories, disease penetrance, and the effect of Janus kinase (JAK) inhibition in individuals with SOCS1 insufficiency.

Methods: This study used data from two independent cohorts: the European Society for Immunodeficiencies (ESID) registry and the UK Biobank. Participants from the ESID registry were from nine European countries (Austria, Belgium, France, Germany, Ireland, Italy, Portugal, Sweden, and Ukraine), China, Taiwan, and the USA. Participants from the ESID registry …

Postoperative Caregiver Decision Regret Following Velopharyngeal Dysfunction Surgery., Rohit Nallani, Nathan Farrokhian, Meghan Tracy, Kevin J. Sykes, Jill M. Arganbright

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Decision regret (DR) is a powerful factor in medical decision-making, particularly for surgical decisions which carry multiple risks, impact quality of life (QOL), and have uncertain outcomes. While prior studies have investigated caregiver DR after tonsillectomy and otologic surgery, there is a paucity of data assessing DR following pediatric otolaryngology procedures. The aim of this study is to measure DR following velopharyngeal dysfunction (VPD) surgery.

METHODS: A cross-sectional survey study with chart review was performed of caregivers of children who had VPD surgery performed by a single surgeon at a tertiary children's hospital. Demographic, medical, and surgical history were …

New Frontiers: The Intern Experience In A Newly Established Integrated Plastic Surgery Program, Hendrik Lintel, Imran Rizvi, Michael Haydon, Erik S Marques, Matthew R Greives, Mohin A Bhadkamkar

Faculty, Staff and Student Publications

The plastic surgery residency pathway has undergone many changes since its inception. The advent of integrated training has largely taken over as the predominant residency model with the addition of a few new programs every year. There is currently a paucity of literature describing the experiences of first-year interns in a new integrated residency program transitioning from the independent model. In this article, we seek to elucidate some of the advantages and difficulties encountered by the initial residents of a newly established integrated program and the steps taken to pave the way for future matched integrated trainees.

Measuring And Interpreting Individual Differences In Fetal, Infant, And Toddler Neurodevelopment, Halie A Olson, M Catalina Camacho, Gavkhar Abdurokhmonova, Sahar Ahmad, Emily M Chen, Haerin Chung, Renata Di Lorenzo, Áine T Dineen, Melanie Ganz, Roxane Licandro, Caroline Magnain, Natasha Marrus, Sarah A Mccormick, Tara M Rutter, Lauren Wagner, Kali Woodruff Carr, Lilla Zöllei, Kelly A Vaughn, Kathrine Skak Madsen

Faculty, Staff and Student Publications

As scientists interested in fetal, infant, and toddler (FIT) neurodevelopment, our research questions often focus on how individual children differ in their neurodevelopment and the predictive value of those individual differences for long-term neural and behavioral outcomes. Measuring and interpreting individual differences in neurodevelopment can present challenges: Is there a "standard" way for the human brain to develop? How do the semantic, practical, or theoretical constraints that we place on studying "development" influence how we measure and interpret individual differences? While it is important to consider these questions across the lifespan, they are particularly relevant for conducting and interpreting research …

Enhancing Seizure Control In Ultra-Refractory Postencephalitic Epilepsies Using Multinodal Network Neuromodulation, Subhiksha Srinivasan, Surya Suresh, Ganne Chaitanya, Manoj Saranathan, Nitin Tandon, Sandipan Pati

Faculty, Staff and Student Publications

This case series reports the formidable challenge posed by postencephalitic epilepsies, characterized by frequent drug-resistant seizures and neuropsychiatric and cognitive comorbidities. Polypharmacy is frequently required, and surgical resection may not be feasible due to multifocality. Neuromodulation therapies, including Deep Brain Stimulation (DBS) and Responsive Neurostimulation (RNS), offer a potential lifeline. In this case series, we shed light on the intricate landscape of seizure management and neuropsychiatric comorbidities in five individuals with frequent seizures (often weekly) and ultra-refractory epilepsy (defined as resistance to more than six different antiseizure medications, including failed epilepsy surgery) following catastrophic encephalitis. Four out of five patients …

An Electronic, Preclinic, Social Determinants Survey Shows Pediatric Atherosclerotic Risk Factor Referral Identifies At-Risk Families, Justin R Rahman, Bethany S Zachariah, Christina Y Miyake, Deidra Ansah, Justin P Zachariah

Faculty, Staff and Students Publications

Objective: Atherosclerotic risk factors are prevalent in childhood. Risk factors are associated with social determinants of health (SDoH), but have never been demonstrated in a clinical pediatric cardiology cohort. This report details the first use of a remotely deployed, previsit, self-administered electronic survey for the ascertainment of SDoH in youth referred to pediatric cardiology. We hypothesized that patients referred for atherosclerotic risk factors would have higher risk of SDoH factors.

Study design: SDoH were assessed using validated instruments from the PRAPARE toolkit and the Accountable Health Communities Health-Related Social Needs Screening Tool automatically deployed days before a scheduled visit in …

Functional Mobility And Pain Are Improved For 6 Years After Adolescent Bariatric Surgery, Neil Thivalapill, Todd M Jenkins, Thomas H Inge, Changchun Xie, Anita P Courcoulas, Carroll M Harmon, Michael A Helmrath, Stephanie Sisley, Marc P Michalsky, Justin R Ryder, Teen‐Longitudinal Assessment Of Bariatric Surgery (Teen‐Labs) Consortium

Faculty, Staff and Students Publications

Objective: The long-term durability of improvements in functional mobility and musculoskeletal pain for adolescents after metabolic and bariatric surgery (MBS) is unknown.

Methods: We used the Teen-Longitudinal Assessment of Bariatric Surgery (Teen-LABS) study to determine the change in mobility and pain among adolescents who underwent MBS. From standardized 400-m walk tests, we analyzed walk time, heart rate (HR) parameters, and musculoskeletal pain.

Results: The mean walk time improved from 383 s (95% CI: 368-399) prior to surgery to 351 s (95% CI: 330-372) by 6 years. The mean resting HR was 90 beats per minute (bpm; 95% CI: 87-93) preoperatively …

Understanding And Addressing Mental Health Challenges Of Families Admitted To The Neonatal Intensive Care Unit., Ashley D. Osborne, Daphna Yasova Barbeau, Tiffany Gladdis, Kara Hansen, Tonia Branche, Emily R. Miller, Christine C. Pazandak, Margaret K. Hoge, Michelle Spencer, Diana Montoya-Williams, Ryan Barbeau, Heather Padratzik, Stephen Lassen

Manuscripts, Articles, Book Chapters and Other Papers

This article reviews the psychological distress experienced by NICU families, including anxiety, postpartum depression (PPD), and post-traumatic stress disorder (PTSD), in addition to providing recommendations for clinicians at the individual, institutional, and national level. Currently, mental health screenings, specialized evaluations, and treatment options are not routinely offered to NICU families and are frequently under-utilized when offered. Here we provide expert opinion recommendations to address challenges in supporting universal screening, offering bedside interventions, including trained mental health professionals in care plans, updating neonatology training competencies, and advocating for policies that support the mental health of NICU families. We advocate that mental …

Epidemiology Of Shigella Species And Serotypes In Children: A Retrospective Substudy Of The Mal-Ed Observational Birth Cohort Study, Elizabeth T. Rogawski Mcquade, Jie Liu, Mustafa Mahfuz, Alexandre Havt, Furqan Kabir, Adil Kalam, Fatima Aziz, Sahrish Muneer, Najeeha Talat Iqbal, Zulfiqar Ahmed Bhutta

Department of Paediatrics and Child Health

Background: Shigellosis is a leading cause of diarrhoea in children globally. We aimed to assess the burden of specific Shigella species and Shigella flexneri serotypes, characterise their clinical syndromes and natural immunity, and evaluate their relevance as causes of diarrhoea and linear growth faltering.
Methods: In the MAL-ED birth cohort study, children younger than 17 days were enrolled from Nov 3, 2009, to Feb 29, 2012, in Bangladesh, Brazil, India, Nepal, Pakistan, Peru, Tanzania, and South Africa and were followed up for 2 years. In this retrospective substudy, we retested Shigella quantitative PCR-positive diarrhoeal and non-diarrhoeal stool samples with molecular …

Protocol Optimization For Out-Of-Hospital Cardiac Arrest: A Quality Initiative To Decrease Epinephrine Administration, Jessica Aird Do, Aaron Singer Md, David Rayburn Md, Mph, Meg Marino Md

Quality Improvement & Patient Safety Forum

No abstract provided.

American Society Of Hematology/International Society On Thrombosis And Haemostasis 2024 Updated Guidelines For Treatment Of Venous Thromboembolism In Pediatric Patients, Paul Monagle, Muayad Azzam, Rachel Bercovitz, Marisol Betensky, Rukhmi Bhat, Tina Biss, Brian Branchford, Leonardo R. Brandão, Anthony K.C. Chan, Vincent E.S. Faustino, Julie Jaffray, Sophie Jones, Hassan Kawtharany, Bryce A. Kerlin, Nicole Kucine, Riten Kumar, Christoph Male, Marie Claude Pelland-Marcotte, Leslie Raffini, Chittalsinh Raulji, Sarah E. Sartain, Clifford M. Takemoto, Cristina Tarango, C. Heleen Van Ommen, Maria C. Velez, Sara K. Vesely, John Wiernikowski, Suzan Williams, Hope P. Wilson, Et Al

School of Medicine Faculty Publications

Background: The American Society of Hematology (ASH) guidelines on treatment of pediatric venous thromboembolism (VTE) were published in 2018. In the last 6 years, there has been a 10-fold increase in the number of children involved in VTE treatment trials. Objective: The ASH Committee on Quality and Guidelines agreed to update the pediatric guidelines in conjunction with the International Society on Thrombosis and Haemostasis (ISTH). These ASH/ISTH evidence-based guidelines are intended to support patients, clinicians, and other health care professionals in the management of pediatric patients with VTE. Methods: ASH/ISTH formed a multidisciplinary guideline panel to minimize potential bias from …

Extracorporeal Membrane Oxygenation For Systemic Lupus Erythematosus: An Elso Registry Analysis, Maria Pereira, Danielle Guffey, Katherine Doane, Eyal Muscal, Carla Levin, Peter Rycus, Marc Anders, Andrea Ontaneda

Faculty, Staff and Students Publications

The success of extracorporeal membrane oxygenation (ECMO) in treating Systemic Lupus Erythematosus (SLE) and the risk factors associated with mortality remain uncertain. Methods: We describe the survival outcomes at discharge of the largest SLE cohort on ECMO support. We performed a retrospective cohort study of the Extracorporeal Life Support Organization registry database from 2012 to 2022. Pediatric and adult survivor groups were analyzed using descriptive statistics for the primary study outcome of survival to hospital discharge. Risk predictors for survival were determined by logistic regression.Results: We included 48 children and 368 adults with SLE. Overall, 198 patients (54%) survived …

Portal Vein Obstruction In Pediatric Liver Transplant Patients: An Evaluation Of Self-Reported Management Practices., Lydia Sieben, Bader A. Alfares, Ruben H. De Kleine, Barbara E. Wildhaber, Thomas Casswall, Greg Nowak, Martin Delle, Denise Aldrian, Valeria Berchtold, Georg F. Vogel, Piotr Kaliciński, Malgorzata Markiewicz-Kijewska, Adam Kolesnik, Jesús Quintero, María Mercadal Hally, Mauricio Larrarte King, Paolo Marra, Michela Bravi, Domenico Pinelli, Mureo Kasahara, Seisuke Sakamoto, Hajime Uchida, Vidyadhar Mali, Marion Aw, Stéphanie Franchi-Abella, Emmanuel Gonzales, Florent Guérin, Guillermo Cervio, Julia Minetto, Sergio Sierre, Martín De Santibañes, Victoria Ardiles, Jimmy Walker Uño, Helen Evans, David Duncan, John Mccall, Steffen Hartleif, Ekkehard Sturm, Jai V. Patel, Marumbo Mtegha, Raj Prasad, Cristina T. Ferreira, Luiza S. Nader, Marco Farina, Catalina Jaramillo, Manuel I. Rodriguez-Davalos, Peter Feola, Amit A. Shah, Phoebe M. Wood, Michael R. Acord, Ryan T. Fischer, Bhargava Mullapudi, Richard J. Hendrickson, Rajeev Khanna, Viniyendra Pamecha, Amar Mukund, Khalid Sharif, Girish Gupte, Simon Mcguirk, Gilda Porta, Winita Hardikar, Marco Spada, Tommaso Alterio, Giuseppe Maggiore, Marisa Beretta, Rudi A J Dierckx, Reinoud P H Bokkers, Hubert P J Van Der Doef, Portal Registry Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND AND AIMS: Portal vein obstruction (PVO) is a known complication after pediatric liver transplantation (pLT). Effective management strategies are crucial in improving patient outcomes. This study investigated the various practice patterns related to PVO management to clarify the degree of consensus on the diverse facets of care involved in addressing PVO after pLT.

METHODS: A self-reported evaluation was conducted using a scanned, paper-based survey among specialized pLT centers participating in the Portal vein Obstruction Revascularization Therapy After Liver transplantation (PORTAL) registry. The survey consisted of 30 questions covering the current practices regarding PVO, including experience, team composition, follow-up and …

Implementation Of A Health Care Transition Pathway For Children And Adults With Inborn Errors Of Metabolism: A Pilot Project, Brittany M. Murray

Doctor of Nursing Practice Scholarly Projects

Background: Children with medically complex diseases require highly specialized care by a highly specialized team. Those with rare disease, including inborn errors of metabolism (IEMs), are a medically complex population and face significant challenges undergoing the health care transition from a child/family-centered model to an adult/patient-centered model of care. The project site follows patients with IEMs in the Metabolism Program, but previously did not have a coordinated process and had insufficient resources for patients/families for the health care transition.

Methods: The Plan-Do-Study-Act (PDSA) method was utilized as the model for improvement. Due to the complexity of the population and time …

Identification Of Surrogate Biomarkers For Mucopolysaccharidosis Type Iva, Yasuhiko Ago, Shaukat Khan, Kimberly Klipner, Allison Bradford, Shunji Tomatsu

Department of Pediatrics Faculty Papers

Mucopolysaccharidosis type IVA (MPS IVA, Morquio A syndrome) is a rare inherited disorder characterized by skeletal dysplasia due to deficient N-acetylgalactosamine-6-sulfate sulfatase activity, resulting in glycosaminoglycan (GAG) accumulation. Identifying accurate biomarkers reflecting clinical severity and therapeutic response remains challenging. This study evaluated potential surrogate biomarkers, including N-terminal pro-C-type natriuretic peptide (NT-proCNP), collagen types I and II, mono-sulfated keratan sulfate (KS), di-sulfated KS, and chondroitin-6-sulfate (C6S), in blood and urine samples from 60 patients ranging from 1 to 62 years of age. NT-proCNP levels were significantly elevated in patients of all ages and negatively correlated with growth impairment, especially after 8 …

Reducing Surgery For Pediatric Posttonsillectomy Hemorrhage Using Tranexamic Acid: A Quality Improvement Initiative, Laura A. Petrauskas, Janavi Sethurathnam, Ansley J. Kunnath, Rahul K. Sharma, John Ceremsak, Ryan H. Belcher, James D. Phillips, Jay A. Werkhaven, Amy S. Whigham, Lyndy J. Wilcox, Christopher T. Wootten, Frank W. Virgin, Jason S. Park

School of Medicine Faculty Publications

Objective: Evaluate the use of tranexamic acid (TXA) and observation as a management option for pediatric patients presenting with posttonsillectomy hemorrhage (PTH). Study Design: Retrospective analysis of a prospectively implemented quality improvement initiative with a historical control comparison group. Setting: Tertiary children's hospital. Methods: Patients < 18 years of age who underwent adenotonsillectomy (AT) and returned to the Emergency Department for PTH were included. Patients who were stable without large volume or active bleeding were given intravenous TXA and admitted for overnight observation. Data were compared in a before-and-after analysis: preprotocol (April 2022 to March 2023) versus postprotocol (April 2023 to March 2024). For cost-effectiveness analysis, we analyzed aggregated claims data from a commercial claims database. Results: Preprotocol 1800 adenotonsillectomies were performed, and 40 procedures were performed for control of hemorrhage (2.2 per 100 AT). Postprotocol 2356 adenotonsillectomies were performed, and 30 procedures were performed to control hemorrhage (1.3 per 100 AT) showing a significant reduction in return to the operating room (relative risk [RR] = 0.59, 95% confidence interval [CI] [0.358, 0.916], P-value.020). There were no reported adverse events attributable to TXA. An estimated 21 surgeries were avoided, and 26 additional patients were observed in the hospital during the postprotocol period, for an estimated net cost savings of $174,970. Conclusion: The implementation of a standardized TXA protocol significantly reduced the need for return to the operating room for PTH in pediatric patients, without complications and with net cost savings to the health care system.

Rethinking Congenital Cytomegalovirus: A Narrative Review Of The Clinical, Public Health, And Ethical Challenges Of A Preventable Cause Of Childhood Hearing Loss, M. Peyton Simons, Gauri Mankekar

School of Medicine Faculty Publications

Congenital cytomegalovirus (cCMV) is the most common non-genetic cause of pediatric sensorineural hearing loss, affecting approximately tens of thousands of infants annually in the United States. While cCMV may present symptomatically at birth, many infants are asymptomatic initially and later develop progressive or fluctuating hearing loss, which is often undiagnosed without early screening. Despite advances in diagnostic methods such as polymerase chain reaction (PCR) testing, the absence of a national screening policy in the U.S. results in significant variability in early detection and follow-up care. This narrative review provides an overview of the clinical manifestations, diagnostic strategies, and treatment options …