Rapid identification and phenotyping of nonalcoholic fatty liver disease patients using a machine-based approach in diverse healthcare systems.

Nonalcoholic fatty liver disease (NAFLD) is the most common global cause of chronic liver disease and remains under-recognized within healthcare systems. Therapeutic interventions are rapidly advancing for its inflammatory phenotype, nonalcoholic steatohepatitis (NASH) at all stages of disease. Diagnosis codes alone fail to recognize and stratify at-risk patients accurately.

Malnutrition and protein energy wasting are associated with severity and progression of pediatric chronic kidney disease.

Background: Protein energy wasting (PEW), a state of deficient protein storage, is not well understood in children and adolescents with chronic kidney disease (CKD). We aimed to re-define PEW using pediatric malnutrition guidelines from the Academy of Nutrition and Dietetics/American Society for Parenteral and Enteral Nutrition (AND/ASPEN), and to describe the relationship between PEW, malnutrition, and kidney function.

Methods: This was a retrospective review of outpatients ≤ 20 years old with a diagnosis of CKD from January 1, 2013, to August 31, 2018.

Co-Occurring X-Linked Agammaglobulinemia and X-Linked Chronic Granulomatous Disease: Two Isolated Pathogenic Variants in One Patient.

We present a unique and unusual case of a male patient diagnosed with two coexisting and typically unassociated X-linked conditions: he was initially diagnosed with X-linked agammaglobulinemia (XLA) followed by a diagnosis of X-linked chronic granulomatous disease (XCGD) and an as of yet unpublished hypomorphic gp91phox variant in the gene. The latter was tested after the finding of granulomatous gingivitis. Hematopoietic stem cell transplant (HSCT) was performed due to severe colitis and nodular regenerative hyperplasia (NRH) of the liver.

Impact of Trainee Involvement on Pediatric ERCP Procedures: Results From the Pediatric ERCP Initiative.

This study examines the role of trainee involvement with pediatric endoscopic retrograde cholangiopancreatography (ERCP) and whether it affects the procedure's success, post-procedural adverse outcomes, and duration. A secondary analysis of the Pediatric ERCP Database Initiative, an international database, was performed. Consecutive ERCPs on children <19 years of age from 18 centers were entered prospectively into the database.

Pediatric ERCP in the Setting of Acute Pancreatitis: A Secondary Analysis of an International Multicenter Cohort Study.

Previous studies have demonstrated the safety of performing endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric population; however, few have addressed the outcomes of children undergoing ERCP during acute pancreatitis (AP). We hypothesize that ERCP performed in the setting of AP can be executed with similar technical success and adverse event profiles to those in pediatric patients without pancreatitis. Using the Pediatric ERCP Database Initiative, a multi-national and multi-institutional prospectively collected dataset, we analyzed 1124 ERCPs.

Genetic insights into childhood-onset schizophrenia: The yield of clinical exome sequencing.

Childhood-onset schizophrenia (COS) is a rare form of schizophrenia with an onset prior to 13 years of age. Although genetic factors play a role in COS etiology, only a few causal variants have been reported to date. This study presents a diagnostic exome sequencing (ES) in 37 Israeli Jewish families with a proband diagnosed with COS.

Technical Outcomes in Pediatric Endoscopic Retrograde Cholangiopancreatography: Data from an International Collaborative.

Objectives: Endoscopic retrograde cholangiopancreatography (ERCP) in adults has been extensively studied through multicenter prospective studies. Similar pediatric studies are lacking. The Pediatric ERCP Database Initiative (PEDI) is a multicenter collaborative aiming to evaluate the indications and technical outcomes in pediatric ERCPs.

High-impact rare genetic variants in severe schizophrenia.

Extreme phenotype sequencing has led to the identification of high-impact rare genetic variants for many complex disorders but has not been applied to studies of severe schizophrenia. We sequenced 112 individuals with severe, extremely treatment-resistant schizophrenia, 218 individuals with typical schizophrenia, and 4,929 controls. We compared the burden of rare, damaging missense and loss-of-function variants between severe, extremely treatment-resistant schizophrenia, typical schizophrenia, and controls across mutation intolerant genes.

Predictors of Prolonged Fluoroscopy Exposure in Pediatric Endoscopic Retrograde Cholangiopancreatography: Results From the Large Pediatric Endoscopic Retrograde Cholangiopancreatography Database Initiative Multicenter Cohort.

Background And Aims: Ionizing radiation exposure during endoscopic retrograde cholangiopancreatography (ERCP) is an important quality issue especially in children. We aim to identify factors associated with extended fluoroscopy time (FT) in children undergoing ERCP.

Methods: ERCP on children <18 years from 15 centers were entered prospectively into a REDCap database from May 2014 until May 2018.

COVID-19 Mortality in a Pediatric Patient with Hemoglobin SC Disease and Alpha-Thalassemia Trait.

As the pandemic continues to evolve, more cases of COVID-19 in pediatric patients are being detected. A 12-year-old boy with HbSC disease alpha-thalassemia trait presented to a pediatric emergency room with fever and weakness. His vital signs were notable for fever, tachypnea, and tachycardia.

Asthma among hospitalized patients with COVID-19 and related outcomes.

Background: Several underlying conditions have been associated with severe acute respiratory syndrome coronavirus 2 illness, but it remains unclear whether underlying asthma is associated with worse coronavirus disease 2019 (COVID-19) outcomes.

Objective: Given the high prevalence of asthma in the New York City area, our objective was to determine whether underlying asthma was associated with poor outcomes among hospitalized patients with severe COVID-19 compared with patients without asthma.

Methods: Electronic heath records were reviewed for 1298 sequential patients 65 years or younger without chronic obstructive pulmonary disease who were admitted to our hospital system with a confirmed positive severe acute respiratory syndrome coronavirus 2 test result.

The Bayley-III scale may underestimate neurodevelopmental disability after cardiac surgery in infants.

Objectives: Neurodevelopmental disability is the most common complication among congenital heart surgery survivors. The Bayley scales are standardized instruments to assess neurodevelopment. The most recent edition (Bayley Scales of Infant and Toddler Development 3rd Edition, Bayley-III) yields better-than-expected scores in typically developing and high-risk infants than the second edition (Bayley Scales of Infant Development 2nd Edition, BSID-II).

Preprocedural Ultrasound for Infant Lumbar Puncture: A Randomized Clinical Trial.

Background: Our purpose was to determine the potential effect of preprocedural ultrasound (US) to increase lumbar puncture (LP) success compared with standard palpation method. Further, we assessed feasibility of and clinician satisfaction with a standardized US protocol.

Methods: This prospective, two-arm, parallel-group randomized trial was conducted in a single-center pediatric emergency department.

An Easy-to-Make, Low-Cost Ultrasound Phantom for Simulation Training in Abscess Identification and Aspiration.

We have created a low-cost and easy to make phantom for abscess identification using point-of-care ultrasound. The phantom also allows needle tracking and abscess aspiration using ultrasound guidance.

Rapidly progressive mitral valve stenosis in patients with acromelic dysplasia.

Acromelic dysplasias are a group of skeletal dysplasias characterised by short-limbed short stature with other distinctive phenotypic features including small hands and feet and stiff joints. Geleophysic dysplasia is an acromelic dysplasia that is associated with characteristic facial features, progressive cardiac valvular thickening, and tracheal stenosis. Owing to overlapping clinical features with other types of short-limbed skeletal dysplasias, it is important to make a precise diagnosis as they have different cardiac morbidity and mortality.

The use of in situ simulation to detect latent safety threats in paediatrics: a cross-sectional survey.

Background: In situ simulation (ISS) has been reported as an innovative method to identify and mitigate latent safety threats (LSTs) in healthcare. Little is known about the current utilisation of ISS across academic simulation programmes.

Objective: This study aims to describe the use of ISS to identify LST across paediatric academic simulation programmes.

Disparities in Adherence to Pediatric Sepsis Guidelines across a Spectrum of Emergency Departments: A Multicenter, Cross-Sectional Observational In Situ Simulation Study.

Background: Each year in the United States, 72,000 pediatric patients develop septic shock, at a cost of $4.8 billion. Adherence to practice guidelines can significantly reduce mortality; however, few methods to compare performance across a spectrum of emergency departments (EDs) have been described.

Neurodevelopmental outcomes after cardiac surgery in infancy.

Background: Neurodevelopmental disability is the most common complication for survivors of surgery for congenital heart disease (CHD).

Methods: We analyzed individual participant data from studies of children evaluated with the Bayley Scales of Infant Development, second edition, after cardiac surgery between 1996 and 2009. The primary outcome was Psychomotor Development Index (PDI), and the secondary outcome was Mental Development Index (MDI).

Interobserver and intraobserver reliability of the modified Waldenström classification system for staging of Legg-Calvé-Perthes disease.

Background: The absence of a reliable classification system for Legg-Calvé-Perthes disease has contributed to difficulty in establishing consistent management strategies and in interpreting outcome studies. The purpose of this study was to assess interobserver and intraobserver reliability of the modified Waldenström classification system among a large and diverse group of pediatric orthopaedic surgeons.

Methods: Twenty surgeons independently completed the first two rounds of staging: two assessments of forty deidentified radiographs of patients with Legg-Calvé-Perthes disease in various stages.

Debriefing in the emergency department after clinical events: a practical guide.

One vital aspect of emergency medicine management is communication after episodes of care to improve future performance through group reflection on the shared experience. This reflective activity in teams is known as debriefing, and despite supportive evidence highlighting its benefits, many practitioners experience barriers to implementing debriefing in the clinical setting. The aim of this article is to review the current evidence supporting postevent debriefing and discuss practical approaches to implementing debriefing in the emergency department.