Implementation of a Provider's Asthma-specific Note to Optimize Billing in the Pediatric Emergency Department.

Unlabelled: There is broad variability in provider documentation for asthma encounters within the pediatric emergency department. Inadequate provider documentation leads to discrepancies between the ideal current procedural terminology (CPT) code and the assigned CPT code based on the care provided. Multiple studies demonstrate improvement in medical provider documentation after implementing standardized documentation templates and educational programs.

Rheumatic Heart Disease in the United States: Forgotten But Not Gone: Results of a 10 Year Multicenter Review.

Background Recent evaluation of rheumatic heart disease (RHD) mortality demonstrates disproportionate disease burden within the United States. However, there are few contemporary data on US children living with acute rheumatic fever (ARF) and RHD. Methods and Results Twenty-two US pediatric institutions participated in a 10-year review (2008-2018) of electronic medical records and echocardiographic databases of children 4 to 17 years diagnosed with ARF/RHD to determine demographics, diagnosis, and management.

Current management of button battery injuries.

Unlabelled: Button batteries (BB) are found in common household items and can lead to significant morbidity and mortality in the pediatric population when ingested. BBs are made of various chemistries and have a unique size and shape that yield significant injury when lodged in the pediatric esophagus. BBs create a local tissue pH environment of 10 to 13 and can induce liquefactive necrosis at the negative pole.

Research environment and resources to support pediatric emergency medicine fellow research.

Background: There is a need for pediatric emergency medicine (PEM) researchers, but the current state of PEM fellow research training is not well described. We sought to (1) describe resources and gaps in PEM fellowship research training and (2) assess agreement between fellow and program director (PD) perceptions of these in fellow research experience.

Methods: Surveys were distributed electronically to U.

Development of a newborn screening tool for mucopolysaccharidosis type I based on bivariate normal limits: Using glycosaminoglycan and alpha-L-iduronidase determinations on dried blood spots to predict symptoms.

Purpose: Current newborn screening (NBS) for mucopolysaccharidosis type I (MPSI) has very high false positive rates and low positive predictive values (PPVs). To improve the accuracy of presymptomatic prediction for MPSI, we propose an NBS tool based on known biomarkers, alpha-L-iduronidase enzyme activity (IDUA) and level of the glycosaminoglycan (GAG) heparan sulfate (HS).

Methods: We developed the NBS tool using measures from dried blood spots (DBS) of 5000 normal newborns from Gifu Prefecture, Japan.

Spontaneous brain arteriovenous malformation rupture with atrioventricular block in a pediatric patient.

This case reports 9 year-old-female with atrioventricular block and seizure. Careful evaluation, including electrocardiogram (ECG) and computerized tomography (CT) revealed a high-grade atrioventricular block and spontaneous brain arteriovenous malformation (AVM) rupture. The patient had complete resolution of her bradycardia and AV block following atropine.

The Utility of DXA Assessment at the Forearm, Proximal Femur, and Lateral Distal Femur, and Vertebral Fracture Assessment in the Pediatric Population: 2019 ISCD Official Position.

Dual-energy X-ray absorptiometry (DXA) is widely used in the evaluation of bone fragility in children. Previous recommendations emphasized total body less head and lumbar spine DXA scans for clinical bone health assessment. However, these scan sites may not be possible or optimal for all groups of children with conditions that threaten bone health.

Gamma Glutamyltransferase Reduction Is Associated With Favorable Outcomes in Pediatric Primary Sclerosing Cholangitis.

Adverse clinical events in primary sclerosing cholangitis (PSC) happen too slowly to capture during clinical trials. Surrogate endpoints are needed, but no such validated endpoints exist for children with PSC. We evaluated the association between gamma glutamyltransferase (GGT) reduction and long-term outcomes in pediatric PSC patients.

Improving pediatric resident laryngoscopy training through the use of a video laryngoscope.

Importance: Opportunities for pediatric residents to perform direct laryngoscopy and tracheal intubation (DLTI) are few and the success rate is low.

Objective: We hypothesize that incorporation of video laryngoscope (McGrath MAC) into pediatric residents DLTI simulation course will improve the simulated DLTI success rate.

Methods: Residents were given 3 attempts at DLTI: (1) baseline using a conventional laryngoscope (CL); (2) using a video laryngoscope (VL); and (3) again using the CL.

ASMBS pediatric metabolic and bariatric surgery guidelines, 2018.

The American Society for Metabolic and Bariatric Surgery Pediatric Committee updated their evidence-based guidelines published in 2012, performing a comprehensive literature search (2009-2017) with 1387 articles and other supporting evidence through February 2018. The significant increase in data supporting the use of metabolic and bariatric surgery (MBS) in adolescents since 2012 strengthens these guidelines from prior reports. Obesity is recognized as a disease; treatment of severe obesity requires a life-long multidisciplinary approach with combinations of lifestyle changes, nutrition, medications, and MBS.

Intraoperative Traction May Be a Viable Alternative to Anterior Surgery in Cerebral Palsy Scoliosis ≥100 Degrees.

Background: For large scoliosis, 2 strategies to maximize correction include intraoperative traction and/or anterior release. It is unclear which patients will benefit the most form either approach. The purpose of our study is to compare the radiographic, perioperative clinical outcomes, and health-related quality of life (HRQoL) outcomes of 2 approaches when used in severe neuromuscular scoliosis in the setting of cerebral palsy (CP).

Reduced arterial elasticity due to surgical skeletonization is ameliorated by abluminal PEG hydrogel.

Arteries for bypass grafting are harvested either with neighboring tissue attached or as skeletonized vessels that are free of surrounding tissue. There are significant benefits to skeletonization, but reports suggest that skeletonized vessels may develop structural defects and are at risk for atherosclerosis. We investigated the specific short-term effects of skeletonization on carotid artery biomechanics and microanatomy in a rabbit model.

Transcriptional regulators of Na,K-ATPase subunits.

The Na,K-ATPase classically serves as an ion pump creating an electrochemical gradient across the plasma membrane that is essential for transepithelial transport, nutrient uptake and membrane potential. In addition, Na,K-ATPase also functions as a receptor, a signal transducer and a cell adhesion molecule. With such diverse roles, it is understandable that the Na,K-ATPase subunits, the catalytic α-subunit, the β-subunit and the FXYD proteins, are controlled extensively during development and to accommodate physiological needs.

SMN1 and SMN2 copy numbers in cell lines derived from patients with spinal muscular atrophy as measured by array digital PCR.

Proximal spinal muscular atrophy (SMA) is an early-onset motor neuron disease characterized by loss of α-motor neurons and associated muscle atrophy. SMA is caused by deletion or other disabling mutation of survival motor neuron 1 (SMN1). In the human genome, a large duplication of the SMN-containing region gives rise to a second copy of this gene (SMN2) that is distinguishable by a single nucleotide change in exon 7.

An integrated approach for analyzing clinical genomic variant data from next-generation sequencing.

Next-generation sequencing (NGS) technologies provide the potential for developing high-throughput and low-cost platforms for clinical diagnostics. A limiting factor to clinical applications of genomic NGS is downstream bioinformatics analysis for data interpretation. We have developed an integrated approach for end-to-end clinical NGS data analysis from variant detection to functional profiling.

A homozygous double mutation in SMN1: a complicated genetic diagnosis of SMA.

Spinal muscular atrophy (SMA), the most common autosomal recessive cause of infant death, is typically diagnosed by determination of SMN1 copy number. Approximately 3-5% of patients with SMA retain at least one copy of the SMN1 gene carrying pathogenic insertions, deletions, or point mutations. We report a patient with SMA who is homozygous for two mutations carried in cis: an 8 bp duplication (c.