Endoscopic cystostomy and biliary sphincterotomy for choledochoceles: A pediatric case series.

Management of choledochoceles (type III choledochal cysts) in children varies. We highlight the potential role of endoscopic management of choledochoceles with cystostomy and biliary sphincterotomy through a series of three successfully treated pediatric patients aged 12-13 at our tertiary center. Patients presented with symptoms including abdominal pain and pancreatitis.

and variants are the underlying cause of Perrault-like syndrome and cardiac anomalies in a patient.

Recent studies have established an association between variants and Perrault syndrome. In this case report, we present a female patient with Perrault syndrome and cardiomyopathy, resulting from variants in and , respectively.

Infant versus noninfant formulas and cow's milk: Transition based on age or weight?

Infant formulas are meant to be used until 1 year of age, at which point children are transitioned to non-infant formulas or cow's milk, depending on their remaining dietary intake. Noninfant formulas and cow's milk are appropriate for children who have an average weight at that 1-year mark (9-9.5 kg); however, can contribute significant protein and/or electrolytes to children who are underweight for age, particularly if they still rely heavily on formula feeding for their caloric intake.

Celiac disease in North America: What is the current practice of pediatric gastroenterology providers?

Objectives: While guidelines exist for the diagnosis and management of pediatric celiac disease (CeD), current practices in North America are not well-described. This study aimed to explore current practice patterns to identify gaps and direct future clinical, training and research initiatives.

Methods: A 23-item survey designed by the Celiac Disease Special Interest Group was distributed electronically to its members.

A regional learning health system of congregate care facilities for COVID-19 response.

Introduction: The COVID-19 pandemic disproportionately affected congregate care (CC) facilities due to communal living, presence of vulnerable populations, inadequate preventive resources, and limited ability to respond to the pandemic's rapidly evolving phases. Most facilities function independently and are not organized for collaborative learning and operations.

Methods: We formed a learning health system of CC facilities in our 14-county metropolitan region, coordinated with public health and health care sectors, to address challenges driven by COVID-19.

Eculizumab for Shiga-toxin-induced hemolytic uremic syndrome in adults with neurological involvement.

The role of eculizumab in treating Shiga-toxin-producing (STEC) hemolytic uremic syndrome (HUS) patients with neurological involvement remains unclear. We describe two distinctly different STEC-HUS patients with neurologic involvement successfully managed with eculizumab, and perform a literature review of all published cases. Both patients had complete resolution of neurological symptoms after initiation of eculizumab.

Validity evidence for a team-leading assessment tool in pediatric emergency resuscitations using video review.

Background: Effective leadership of health care action teams has demonstrated positive influence on team performance and patient care, but there is no consensus on how to assess these skills. We developed a novel team leadership assessment tool for leaders of interprofessional pediatric resuscitation teams and collected validity evidence for this tool using video review.

Methods: This was a prospective cohort study from November 2021 to October 2022.

Bias between capnometry and venous carbon dioxide during initial assessment of pediatric emergency department patients: A video-based study.

Objective: The bias of capnometry (ETCO) and venous carbon dioxide (vpCO) among pediatric emergency department (PED) patients triaged to critical care areas is unknown. We aimed to explore correlations and bias between ETCO and vpCO¸and identify predictors of bias.

Methods: This was an observational, video-based, retrospective study comparing ETCO and vpCO.

Systematic online academic resource (SOAR) review: Pediatric respiratory infectious disease.

Background: Free open access medical education (FOAM) resources have become increasingly popular in graduate medical education. Despite their accessibility, the assessment of FOAM resources' quality is challenging due to their decentralized nature and the diverse qualifications of their authors and distribution platforms. In this first pediatric systematic online academic resource (SOAR) review, we utilized a systematic methodology to aggregate and assess the quality of FOAM resources on pediatric respiratory infectious disease topics.

The effect of transfusion on survival in head and neck cancer after free tissue reconstruction.

Objective: To examine if perioperative blood transfusion affects overall survival (OS) and recurrence-free survival (RFS) in head and neck cancer patients who undergo free tissue reconstruction.

Design: Retrospective cohort study.

Methods: The medical records of free tissue flaps between 2007 and 2010 were reviewed.

Learning from an equitable, data-informed response to COVID-19: Translating knowledge into future action and preparation.

Introduction: The COVID-19 pandemic revealed numerous barriers to effectively managing public health crises, including difficulties in using publicly available, community-level data to create learning systems in support of local public health decision responses. Early in the COVID-19 pandemic, a group of health care partners began meeting to learn from their collective experiences. We identified key tools and processes for using data and learning system structures to drive equitable public health decision making throughout different phases of the pandemic.

Conceptualizing and redefining successful patient engagement in patient advisory councils in learning health networks.

Introduction: Patient engagement has historically referenced engagement in one's healthcare, with more recent definitions expanding patient engagement to encompass patient advocacy work in Learning Health Networks (LHNs). Efforts to conceptualize and define what patient engagement means-and what patient engagement means-are, however, lacking and a barrier to meaningful and sustainable patient engagement via patient advisory councils (PACs) across LHNs.

Methods: Several co-authors (Madeleine Huwe, Becky Woolf, Jennie David) are former ImproveCareNow (ICN) PAC members, and we integrate a narrative review of the extant literature and a case study of our lived experiences as former ICN PAC members.

Rapid cycle deliberate practice to improve airway skills and performance of trainees in a pediatric emergency department.

Objective: The study objective was to determine the effect of a rapid cycle deliberate practice (RCDP) program on simulated and actual airway skills by pediatric emergency medicine (PEM) fellows.

Methods: We designed and implemented a 12-month RCDP airway skills curriculum for PEM fellows at an academic pediatric institution. The curriculum was designed using airway training literature, RCDP principals, and internal quality assurance airway video review program.

Sync for Genes Phase 5: Computable artifacts for sharing dynamically annotated FHIR-formatted genomic variants.

Introduction: Variant annotation is a critical component in next-generation sequencing, enabling a sequencing lab to comb through a sea of variants in order to hone in on those likely to be most significant, and providing clinicians with necessary context for decision-making. But with the rapid evolution of genomics knowledge, reported annotations can quickly become out-of-date. Under the ONC Sync for Genes program, our team sought to standardize the sharing of dynamically annotated variants (e.

Examining bidirectional associations between sleep and behavior among children with attention-deficit/hyperactivity disorder.

Background: Children with attention-deficit/hyperactivity disorder (ADHD) have more sleep problems than their peers which contribute to behavioral and functional impairments. This study examines the bidirectional relationship between nightly sleep (i.e.

Challenges and lessons learned birthing during the COVID-19 pandemic: A scoping review.

Background And Aims: The impact of the COVID-19 pandemic on the healthcare system facilitated a change in policies to redress the consequences of increased demand and fear of disease transmission. Restrictive measures throughout the healthcare system limiting access to accompanying partners of birthing people in addition to fears of contracting COVID-19, an increasing number of birthing people chose to have an out-of-hospital birth. Out-of-hospital births are not prevalent in the United States.

Establishing a Cystic Fibrosis Learning Network: Interventions to promote collaboration and data-driven improvement at scale.

Introduction: A learning health network is a type of learning health system in which stakeholders use network organization to improve health and health care. Building on existing resources in the cystic fibrosis (CF) community, the Cystic Fibrosis Learning Network (CFLN) was designed to improve medical outcomes and quality of life through an intentional focus on achieving reliable evidence-based chronic care delivery and creating a system for data-driven collaborative learning.

Methods: We describe the development and growth of the CFLN considering six domains of a Network Maturity Grid: system leadership; governance and policy management; quality improvement (QI); engagement and community building; data and analytics; and research.

Factors leading to supranormal cardiac index in pediatric pulmonary hypertension patients treated with parenteral prostanoid therapy.

Parenteral prostanoid therapy (PPT) can result in supranormal cardiac index (SCI; >4 L/min/m) in pediatric pulmonary hypertension (PPH) patients. We evaluated the incidence, hemodynamic factors, and outcomes associated with SCI in PPH. This retrospective cohort study included 22 PPH patients on PPT from 2005 to 2020.

What to know about 2.0: Revisions to the pediatric emergency medicine milestones.

Introduction: The Accreditation Council for Graduate Medical Education (ACGME) adopted educational milestones for trainee assessment in 2013, as a key component of the Next Accreditation System. Two years later, the ACGME, American Board of Pediatrics (ABP), and American Board of Emergency Medicine (ABEM) collaborated to create specialty-specific subcompetencies in pediatric emergency medicine (PEM). Since that time, emerging data have demonstrated the need to revise specialty milestones.

Spectrum of Pediatric to Early Adulthood -Associated Movement Disorders.

Background: pathogenic variants are associated with hypomyelination, hypodontia, hypogonadism, and movement disorders.

Cases: We describe the range of movement disorders seen in six patients (four female, two male) with variants [three novel (c.2214del, c.

Lifetime Burden of Morbidity in Patients With Isolated Congenital Ventricular Septal Defect.

Background The lifetime burden of morbidity in patients with isolated congenital ventricular septal defect (VSD) is not completely described. Methods and Results In a population-based cohort study in Denmark using nationwide medical registries, we included 8006 patients diagnosed with a congenital VSD before 2018 along with 79 568 randomly selected controls from the general Danish population matched by birth year and sex. Concomitant congenital cardiac malformations and chromosomal abnormalities were excluded.

Psychosocial predictors of problematic eating in young adults who underwent adolescent bariatric surgery.

Introduction: This study examined problematic eating and eating-related psychopathology among young adults who underwent adolescent bariatric surgery including concurrent and prospective associations with psychosocial factors and weight change.

Methods: VIEW point is a 6-year follow-up study within a prospective observational study series observing adolescents with severe obesity who had bariatric surgery ( = 139) or who presented to nonsurgical lifestyle modification programs ( = 83). Participants completed height/weight measurements, questionnaires, and diagnostic interviews.

Pre-existing humoral immune comebacks control the development of the severe form of coronavirus disease 2019 in Gaucher patients.

Coronavirus disease 2019 (COVID-19) and the Gaucher disease (GD) exhibit lot of resemblances in induction of innate and adaptive immune inflammation that include the immune cells activation and the massive generation of pro-inflammatory cytokines, chemokines, and growth factors, which are all critical for propagation of the disease process and the multiple organ damage. However, majority of the GD patients have not revealed the expansion of severe form of the COVID-19. This study suggests that the pre-existing humoral immunity influence the devlopment of strong network of antibodies to different structural proteins of SARS-CoV2 in GD patients with COVID-19.

Toward an ontology of collaborative learning healthcare systems.

Objective: To establish a basis for a domain ontology - a formal, explicit specification of a shared conceptualization - of collaborative learning healthcare systems (CLHSs) in order to facilitate measurement, explanation, and improvement.

Methods: We adapted the "Methontology" approach to begin building an ontology of CLHSs. We specified the purpose of an ontology, acquired domain knowledge via literature review, conceptualized a common framework of CLHSs using a grounded approach, refined these concepts based on expert panel input, and illustrated concept application via four cases.