Pediatrics Commons^™

Parental Psychosocial Outcomes After A Positive Newborn Screen For A Lysosomal Storage Disorder., Courtney D. Berrios, Randi N. Gadea, Meghan Strenk, Twisha Nadella, Jennifer L. Gannon, Janelle R. Noel-Macdonnell Phd

Manuscripts, Articles, Book Chapters and Other Papers

PURPOSE: This study explores the psychosocial impact of a positive newborn screen (NBS) result for four lysosomal storage disorders (LSDs) (Fabry disease, Krabbe disease, Mucopolysaccharidosis Type I, Pompe disease) across confirmatory results.

METHODS: Parents whose child who had a positive NBS for one of the included LSDs were recruited for a retrospective cohort (n = 80) or prospective, longitudinal cohorts (n = 50). Surveys assessed uncertainty, anxiety, intrusive or avoidant thoughts, and perceived vulnerability of their child's health. In-depth interviews explored the NBS experience and psychosocial response.

RESULTS: Participants experienced uncertainty and anxiety during confirmatory testing that improved as parents …

Preoperative Immobilization For Pediatric Femur Fractures., Connor Mathes, Leilani Mansy, Lisa Berglund, Caleb W. Grote

Manuscripts, Articles, Book Chapters and Other Papers

Guidelines for the definitive surgical management of pediatric femoral shaft fractures have recently been established. Initial preoperative immobilization of these patients varies by age and size; limited guidelines exist to guide caregivers in appropriate preoperative care. Here, we present our recommendations based on clinical experience and literature review. We prefer temporary splints for patients younger than 5 years and Bucks boot traction for skeletally immature patients older than 5 years. In small patients, the splint should extend past the iliac crest and down to the foot to ensure proper immobilization of the femur fracture. For Bucks boot traction, proper traction …

Bilateral Pleuropulmonary Blastoma In An Infant., R Guanà, E Zambaiti, Maria Grazia Sacco Casamassima, S Garofalo, L Lonati, E Stanton, F Gennari

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Improving Infant Continuity Of Care In An Academic Center Clinic: A Quality Improvement Project., Abiye Y. Okah, Morgen L. Govindan, Rachael D. Dameron, Kristi Williams

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Continuity of care (COC) with an identified provider is a key principle of the patient-centered medical home. COC may be impacted by other activities that providers practicing in an academic center must engage in. The disruption of care processes during the COVID-19 pandemic led to a decrease in COC for infants attending our academic center's primary care clinic. We implemented a quality improvement (QI) project to improve the rates of infant COC.

METHODS: In 2021, we implemented a QI project with the aim of increasing the percentage of 12-month-old infants who had seen the same provider for at least …

Implementation Of The Multicountry Who Covid-19 Pregnancy Cohort Study: Challenges And Lessons Learned During The Pandemic, Maria Costa, Renato Souza, Jose Cecatti, Sami Gottlieb, Marie Delnord, Soe Soe Thwin, Ndema Habib, Marleen Temmerman, Ingrid Gichere, Beth Maina

Obstetrics and Gynaecology, East Africa

Introduction A generic research protocol was developed for a prospective cohort study to allow systematic, harmonized data collection of the impact of SARS-CoV-2 infection and vaccination during pregnancy on maternal, obstetric, and neonatal outcomes across different settings. This article describes the study conception, development, implementation, challenges, and key lessons learned within study sites across the world.

Methods The protocol was implemented in 43 facilities in 10 countries during the pandemic, involving consecutive recruitment of over 16,000 pregnant or postpartum women. We evaluated selection of study sites, ethical approvals, staff recruitment and training, recruitment and follow-up, and incorporation of new elements …

Neonatal Unit Human Resources: Coverage For Six Cadres And Trends For Staff-To-Baby Ratios In 65 Neonatal Units Implementing With Nest360 In Kenya, Malawi, Nigeria, And Tanzania, Rebecca Penzias, Eric Ohuma, Opeyemi Odedere, Olabisi Dosunmu, George Okello, Hannah Mwaniki, Robert Tillya, Vincent Ochieng, Ekran Rashid, William Macharia

Paediatrics and Child Health, East Africa

Background Implementing small and sick newborn care (SSNC) requires skilled health workers; however, there is a shortage, adversely impacting patient outcomes and health worker well-being. There are limited data and no current World Health Organization (WHO) standards for staff-to-baby ratios in neonatal units in low- and middle-income countries (LMICs) to inform policy, planning, and investment.

Methods In 65 neonatal units (36 in Malawi, 13 in Kenya, 7 in Tanzania, and 9 in Nigeria), a health facility assessment (HFA) for SSNC and government-led quality improvement (QI) processes were implemented. Staffing data were collated from baseline HFA (Sept 2019–March 2021) and mid-2023 …

Intravenous Sotalol In Pediatric Cardiac Intensive Care Patients Compared To Other Antiarrhythmics: Insights From A Multicenter Database, Rohit S. Loomba, Emily R. Backes, Juan Farias Torres, Alejandro Borquez, Lindsey Malloy-Walton, Saul Flores

Posters

Background: Sotalol is an antiarrhythmic for which an intravenous (IV) form became available in 2015. Studies suggest it is safe and effective for treating pediatric arrhythmias, with fewer effects on physiologic parameters and cardiac output than amiodarone, and it does not appear to be associated with its long-term risks. However, data in pediatric patients remains limited. This study aimed to characterize the use of intravenous sotalol in pediatric intensive care admissions and compare its use and outcomes with intravenous amiodarone and procainamide. Methods: Data were drawn from the Pediatric Health Information System across six hospitals with the highest IV sotalol …

Small For Gestational Age Subgroups Have Differential Morbidity, Growth, And Neurodevelopment At Age 2: The Interbio-21st Newborn Study, Aris T. Papageorghiou, María C. Restrepo-Ménde, Rose Mcgready, Fernando C. Barros, Francois Nosten, Shama Munim, Roseline Ochieng, Rachel Craik, Hellen C. Barsosio, Zulfiqar Ahmed Bhutta

Department of Obstetrics & Gynaecology

Background: Small for gestational age is a complex perinatal syndrome associated with increased neonatal morbidity, mortality, and impaired childhood growth and neurodevelopment. Current classifications rely primarily on birthweight, which does not capture the heterogeneity of the condition nor predict long-term health outcomes. Here we aim to identify and characterize distinct small for gestational age subgroups and assess their neonatal and early childhood health trajectories.
Objective: To refine the classification of small for gestational age by identifying subgroups based on maternal, fetal, and environmental factors and evaluating their associations with neonatal morbidity, growth, and neurodevelopment at age 2.
Study design: Prospective …

Use Of An Electronic Medical Record Tool To Ensure Tertiary Survey Compliance In Pediatric Trauma Patients, Ryan T. Davis, Erica Surman, Begum Akay, Nathan Novotny, Anthony Stallion, Pavan Brahmamdam

Conference Presentation Abstracts

Introduction:
The purpose of the tertiary survey is to ensure that traumatic injuries that may be missed during the primary and secondary survey are identified and treated accordingly. After a review, our trauma program identified a quality gap where tertiary surveys were not being documented. The purpose of this study is to evaluate whether the use of an electronic medical record tool, in conjunction with a reporting process, improved documentation of the tertiary survey in pediatric trauma patients.

Methods:
A review of the pediatric trauma database was performed. Patients < 16 years old who were admitted after level 1 or 2 activations or trauma evaluations were included. The study period was between January 2023 and January 2024. An Epicâ„¢ SmartPhrase was developed with input from the pediatric surgery team, pediatric trauma nurse manager, and the general surgery residents. The residents and pediatric surgery attendings were educated and the smartphrase was implanted July 2023. Reviews were made on a month-to-month basis and if a patient did not have a tertiary survey documented, emails to the trauma attending and residents of record were sent, notifying them of missed documentation of a tertiary survey.

Results:
Tertiary survey documentation compliance in the six months prior to …

A Rare Case Of Ogilvie’S Syndrome In A Pediatric Patient With A Complex Medical History, Kirby Lampe, Victoria Sarata, Aileen Har

Posters

Acute colonic pseudo-obstruction (ACPO) or Ogilvie’s syndrome is an acute dilatation of the small or large bowel without an obstructive lesion. Symptoms include abdominal distension, nausea and vomiting, constipation and occasional dyspnea. The disorder most commonly involves the cecum and right colon and is most frequently seen in male patients above the age of 60 (1). It is rare for Ogilvie’s to occur in the pediatric population though some infrequent instances have been reported. Here, we report a case of a patient with significant abdominal distension and gaseous distension seen on x-ray, which was diagnosed and treated as Ogilvie’s syndrome. …

A Case Report: Ansa Pancreatica As A Cause Of Recurrent Pancreatitis, Chase Branstetter, Elizaveta Khenner, Victoria Sarata, Nadia Ibrahimi

Posters

No abstract provided.

Bleeding (Life-Threatening): Children’S Mercy Kansas, Children's Mercy Kansas City

Clinical Pathways

No abstract provided.

Hallucinations, Children's Mercy Kansas City

Clinical Pathways

No abstract provided.

Human Sex Trafficking, Children's Mercy Kansas City

Clinical Pathways

No abstract provided.

Bridging The Gap: An Educational Intervention For The Early Introduction Of Peanut Protein In Pediatric Care, Rachel Gilson Dnp, Rnc-Ob, C-Onqs, Fnp-C, Parkview Health

Nursing Publications, Posters, & Presentations

Presented at the 6th Annual Parkview Nursing Research and Evidence-Based Practice Symposium. Theme: “From Paper to Practice: The Art of Implementation Science,” at the Parkview Mirro Center for Research and Innovation.

The primary aim of this Doctor of Nursing Practice (DNP) project was to improve provider adherence to the NIAID guidelines for the early introduction of peanut protein in infants under one year of age. The project intended to address identified knowledge and practice gaps through a targeted educational intervention at Fort Wayne Pediatrics (FWP).

Ear Foreign Body, Children's Mercy Kansas City

Clinical Pathways

No abstract provided.

The 10-Year Status Of Pediatric Gastroenterologists' Perception Of Their Roles In The Management Of Childhood Obesity., Sara Karjoo, Senthilkumar Sankararaman, Fotini Tania Mitsinikos, Marwa Abu El Haija, Sheela Mahendra, Ayesha Baig, Ruba A. Abdelhadi, Ruben E. Quiros-Tejeira, Timothy Sentongo

Manuscripts, Articles, Book Chapters and Other Papers

In 2013, a survey of NASPGHAN members on their involvement in managing pediatric obesity revealed that most pediatric gastroenterologists viewed their roles as primarily supportive, focusing on the management of obesity-related gastrointestinal comorbidities. This 10-year follow-up survey, which targeted pediatric gastroenterology programs and practices, found similar perceptions, along with an increased interest in learning about obesity pharmacotherapy. Additionally, most programs and practices reported limited access to dedicated dietitians, a lack of funding, and a desire for greater institutional support for multidisciplinary programs addressing obesity. Notably, none of the funded programs cited philanthropy as a source of funding. These findings underscore …

Intraoperative Electrical Stimulation Can Identify Potential Ulnar Nerve Injury From Crossed Pinning Of A Supracondylar Humerus Fracture., Lucas A. Georger, Mckenna C. Noe, Richard M. Schwend

Manuscripts, Articles, Book Chapters and Other Papers

Treatment of Gartland type III supracondylar fractures using reduction and crossed Kirschner wire fixation is considered the most stable method; however, there is a risk of iatrogenic ulnar nerve injury during medial pin placement. We describe a technique for intraoperative detection of such nerve injury.An inexpensive disposable nerve stimulator is set at 2 mA and applied directly to the Kirschner wires after placement, alerting the surgeon intraoperatively if a wire contacts the ulnar nerve. A positive response involves any finger motion with Kirschner wire stimulation. We recommend this novel method of intraoperative Kirschner wire stimulation using an inexpensive disposable nerve …

Laceration, Children's Mercy Kansas City

Clinical Pathways

No abstract provided.

Cerumen Impaction, Children's Mercy Kansas City

Clinical Pathways

No abstract provided.

Skin Soft Tissue Infection: Antibiotics For Abscesses, Children's Mercy Kansas City

Clinical Critically Appraised Topics

No abstract provided.

Intravascular Hemolysis As A Converging Mechanism In Hemolytic Disorders, Justin A Courson, Arun Saini

Faculty, Staff and Students Publications

No abstract provided.

Pediatric Heart-Kidney Transplantation, Swati Choudhry, Kriti Puri, Vikas R Dharnidharka

Faculty, Staff and Students Publications

Heart transplantation is regarded as the definitive treatment for advanced pediatric heart failure. However, concomitant kidney dysfunction often complicates candidate selection for isolated heart transplantation. Prolonged venous congestion, nephrotoxic exposures, and recurrent episodes of acute kidney injury can result in varying degrees of renal impairment. Differentiating between reversible kidney injury secondary to cardiorenal syndrome and irreversible intrinsic renal disease remains a major challenge. Pediatric heart-kidney transplantation has shown survival benefits for patients with end-stage heart failure requiring chronic dialysis, yet its role in patients with less severe renal dysfunction is less clearly defined. This review summarizes the current evidence on …

Selective Agonism Of Liver And Gut Fxr Prevents Cholestasis And Intestinal Atrophy In Parenterally Fed Neonatal Pigs, Yanjun Jiang, Zhengfeng Fang, Gregory Guthrie, Barbara Stoll, Shaji Chacko, Sen Lin, Bolette Hartmann, Jens J Holst, Harry Dawson, Jose J Pastor, Ignacio R Ipharraguerre, Douglas G Burrin

Faculty, Staff and Students Publications

We aimed to investigate the relative efficacy of feeding different bile acids in preventing PNALD in neonatal pigs. Newborn pigs given total parenteral nutrition (TPN) combined with minimal enteral feeding of chenodeoxycholic acid (CDCA) or increasing doses of obeticholic acid (OCA) for 19 days. Enteral OCA (5 and 15 mg/kg), but not CDCA (30 mg/kg) reduced blood cholestasis markers compared to TPN controls and increased bile acids in the gallbladder and intestine. Major bile acids in the liver and distal intestine were CDCA, HCA, HDCA, and OCA, and their relative proportions were increased by the type of bile acid (CDCA …

Factors Influencing 25-Year Survival In Pediatric Liver Transplant Recipients, Garrett Wortham, Megan Crawford, John Miggins, Chun-Sing Huang, John A Goss, Abbas Rana, Nhu Thao Galván

Faculty, Staff and Students Publications

Background: This study assesses the impact of cholestatic liver disease, including biliary atresia, on 25-year survival post-transplantation and additional factors influencing long-term outcomes after pediatric liver transplantation.

Methods: We conducted a retrospective analysis of pediatric liver transplant recipients (1987-1998) using de-identified data from the OPTN 2023 Liver Database. After exclusions for multi-organ transplants (n = 222), prior transplants (n = 2256), and deaths within 1 year (n = 526), 2429 patients remained, including 645 with cholestatic disease. Univariate and multivariate analyses identified factors associated with 25-year survival.

Results: A primary diagnosis of cholestatic disease was associated with improved 25-year survival …

From Pachymeningitis Hemorrhagica To Abusive Head Trauma: A Journey Through The Centuries, Oliver Berthold, Flora Blangis, Christopher Spencer Greeley

Faculty, Staff and Students Publications

The development of medical knowledge follows specific principles. These also apply to abusive head trauma. The seminal literature from the early 20th century is based on earlier research from Europe, which is fundamental to understand the evolution of understanding and nomenclature of abusive head trauma. A review of original medical literature on intracranial injuries in children, published in German, English, and French, was conducted using PubMed and Google Scholar for articles before 1950. The primary search focused on subdural hemorrhages and pachymeningitis in children, followed by a snowball search of references. Full texts of available manuscripts were reviewed. Primary literature …

Differences In Patient And Parent Preferences For Prognostic Communication Across Disease Milestones In Advanced Childhood Cancer, Rachel A Kentor, Shoshana Mehler, Caroline Christianson, Harmony Farner, Tara M Brinkman, Justin N Baker, Pamela S Hinds, Jennifer W Mack, Erica C Kaye

Faculty, Staff and Students Publications

Background: Navigating prognostic communication is challenging for clinicians caring for adolescents and young adults (AYAs) with poor-prognosis cancer. Presently, little is known about how AYA and parent preferences for prognostic disclosure evolve over time. This study explored communication preferences at key illness milestones of disease progression to inform individualized, person-centered approaches to optimize prognostic disclosure.

Procedure: Sixty-five participants, including 25 AYAs aged 12-25 years and 40 parents, were recruited at distinct illness timepoints-diagnosis, relapse/progression, Phase I/II trial enrollment, and bereavement-to participate in semi-structured interviews about their preferences for timing, style, and content of prognostic communication. Rapid analysis was conducted to …

Beyond Seizures As An Outcome Measure: A Global Severity Scoring System For Cdkl5 Deficiency Disorder, Peter Jacoby, Eric D Marsh, Scott Demarest, Jacinta M Saldaris, Helen Leonard, Heather E Olson, Joni N Saby, Elia Pestana-Knight, Rajsekar Rajaraman, Dana Price, Judith Weisenberg, Bernhard Suter, Jenny Downs, Tim A Benke

Faculty, Staff and Students Publications

Background: CDKL5 deficiency disorder (CDD) is a rare developmental and epileptic encephalopathy (DEE) associated with multiple impairments and comorbidities. Outcome measures for disease-modifying clinical trials for DEEs should measurably capture a spectrum of caregiver priorities and be externally validated.

Methods: The International CDKL5 Clinical Research Network was the data source for this observational study. A Structural Equation Model was constructed with latent, exogenous variables related to observed clinical features to calculate a global severity score from the following assessments: the CDKL5 Clinical Severity Assessment-Clinician and -Caregiver, Communication and Symbolic Behavior Scales Developmental Profile Infant Toddler Checklist and the Sleep Disturbance …

A Rare Molecular Diagnosis In A Patient With Hepatocerebral Syndrome Contributes To The Expansion Of The Phenotypic Spectrum Of Polg2 -Related Mitochondrial Disorder, Vittoria Rossi, Dan Brooks, Hongzheng Dai, Elizabeth Mizerik, Karla Salazar, Daniel Davila-Williams, Yishay Ben-Moshe, Seema R Lalani, Sarah H Elsea, Charul Gijavanekar, Daryl A Scott, Keren Machol, Mir Reza Bekheirnia, Fernando Scaglia

Faculty, Staff and Students Publications

POLG2 encodes an accessory subunit in DNA polymerase gamma that is required for mitochondrial DNA synthesis. Monoallelic pathogenic variants in POLG2 are associated primarily with progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal dominant type 4 (PEOA4, MIM #610131). We report a rare case of severe infantile hepatocerebral syndrome associated with biallelic variants in POLG2. The proband, a 5-week-old female infant, presented with seizures and acute liver failure. Extensive metabolic workup, including untargeted metabolomics analysis and elevated plasma growth differentiation factor 15, was suggestive of mitochondrial dysfunction. Rapid trio genome sequencing identified compound heterozygous variants, a likely pathogenic variant and …

Indomethacin-Induced Ductus Arteriosus Closure And Aortopulmonary Collateral Formation In Fetal Ebstein Anomaly With Circular Shunt, Mounica Y Rao, Ruth M Vaughan, Shaine A Morris, Nancy A Ayres, Maggie J Nguyen, Jianhong Zhang, Betul Yilmaz Furtun

Faculty, Staff and Students Publications

• Indomethacin may improve hemodynamics in fetal Ebstein anomaly with circular shunt. • Ductal closure with indomethacin can impair pulmonary blood flow and growth. • Ductal closure may result in subsequent development of APCs. • Irreversible ductal closure may affect postnatal surgical palliation. • Fetal echocardiography is vital for early intervention and medication titration.