Sticking It Straight: Pediatric Procedure Curriculum Initiative.

OBJECTIVES: Literature demonstrates that pediatric residents are not graduating with procedural confidence and competency. This was confirmed with our own institution's Accreditation Council for Graduate Medical Education and internal surveys. Our primary objective was to improve procedural confidence among pediatric residents with the introduction of a mandatory longitudinal pediatric procedural curriculum, including simulation in combination with online modules. METHODS: We performed a quality improvement intervention to increase resident comfort level performing Accreditation Council for Graduate Medical Education-required procedures. This study involved pediatric residents, postgraduation year (PGY) 1-3, at an academic, tertiary care hospital. Between April 2015 and June 2017, the combination of online self-directed learning modules and hands-on simulation curriculum was implemented for pediatric residents. Surveys were administered at 1-year intervals to assess self-reported comfort level on 12 procedures using a Likert scale (1 for "strongly disagree" to 5 for "strongly agree, maximum score of 60 for all procedures). RESULTS: Forty (63%) of 63 participant presurveys and 45 (71%) of 63 postsurveys were available for analysis. The mean comfort level for all procedures demonstrated a statistically significant increase from 32.4 to 37.1, or 12.7% (P = 0.005). By PGY level, the score increased from 24.4 to 30.9 (21%) for PGY1, 34.4 to 37.5 (8.3%) for PGY2, and 38.6 to 42.8 (9.8%) for PGY3 (P < 0.005). Overall, pediatric residents rated the simulation experience very favorably. CONCLUSIONS: A mandatory longitudinal procedure curriculum improved procedural comfort level among pediatric residents. Iterative curriculum designs found the most productive combination to be deliberate practice within mastery learning simulation sessions with required precourse online modules.

Point-of-Care Ultrasound for the Evaluation of Neck Masses in the Pediatric Emergency Department: A Case Series.

ABSTRACT: Point-of-care ultrasound can be an effective tool for pediatric emergency medicine providers in the evaluation of soft tissue lesions. We present a series of 4 pediatric patients with neck lesions in whom point-of-care ultrasound identified the type of lesion, guided decision-making on the need for computed tomography imaging, and led to definitive management.

New Cluster of Acute Flaccid Myelitis in Western Pennsylvania.

Acute flaccid myelitis is a debilitating illness characterized by acute onset of limb weakness, with one or more spinal segments displaying magnetic resonance imaging-confirmed gray matter lesions. Since the first outbreak in 2014, tracking by the Centers for Disease Control and Prevention has demonstrated biennial epidemics in the United States, with a current outbreak occurring in 2018. The cases of 3 children with acute flaccid myelitis who were initially thought to have common nonneurologic diagnoses are presented. Emergency physicians need to be vigilant to recognize the subtleties of acute flaccid myelitis because the illness progression is rapid and therapy is nuanced.

Gene therapy: the promise of a permanent cure.

Gene therapy offers the possibility of a permanent cure for any of the more than 10,000 human diseases caused by a defect in a single gene. Among these diseases, the hemophilias represent an ideal target, and studies in both animals and humans have provided evidence that a permanent cure for hemophilia is within reach.

Application of dynamic air bronchograms on lung ultrasound to diagnose pneumonia in undifferentiated respiratory distress.

Patients presenting with undifferentiated respiratory distress remain a diagnostic dilemma. The use of point-of-care ultrasound (POCUS) to evaluate the lungs and pleural cavities can improve diagnostic uncertainty in these patients. When visualizing consolidated lung tissue with POCUS, one may encounter static or dynamic air bronchograms. Static air bronchograms are seen in atelectasis and pneumonia, whereas dynamic air bronchograms are highly specific for pneumonia. We describe a case of a critically ill patient where bedside thoracic ultrasound helped to narrow the differential diagnosis early in the resuscitation, while standard radiographs were non-diagnostic.

Effect of ketamine on transcranial Doppler Gosling pulsatility index in children undergoing procedural sedation: A pilot study.

Objectives: There has been controversy over whether ketamine affects intracranial pressure (ICP) in children. Transcranial Doppler ultrasound (TCD) is a validated technique used to assess ICP changes noninvasively. Gosling pulsatility index (PI) directly correlates with ICP changes. The objective of this study was to quantify PI changes as a surrogate marker for ICP changes in previously healthy children receiving intravenous ketamine for procedural sedation. Methods: We performed a prospective, observational study of patients 5-18 years old who underwent sedation with intravenous ketamine as monotherapy. ICP changes were assessed by surrogate PI at baseline, immediately after ketamine administration, and every 5 minutes until completion of the procedure. The primary outcome measure was PI change after ketamine administration compared to baseline (denoted ΔPI). Results: We enrolled 15 participants. Mean age was 9.9 ± 3.4 years. Most participants underwent sedation for fracture reduction (87%). Mean initial ketamine dose was 1.4 ± 0.3 mg/kg. PI decreased at all time points after ketamine administration. Mean ΔPI at sedation onset was -0.23 (95% confidence interval [CI] = -0.30 to -0.15), at 5 minutes was -0.23 (95% CI = -0.28 to -0.18), at 10 minutes was -0.14 (95% CI = -0.21 to -0.08), at 15 minutes was -0.18 (95% CI = -0.25 to -0.12), and at 20 minutes was -0.19 (95% CI = -0.26 to -0.12). Using a clinically relevant threshold of ΔPI set at +1 (+8 cm H2O), no elevation in ICP, based on the PI surrogate marker, was demonstrated with 95% confidence at all time points after ketamine administration. Conclusions: Ketamine did not significantly increase PI, which was used as a surrogate marker for ICP in this sample of previously healthy children. This pilot study demonstrates a model for evaluating ICP changes noninvasively in the emergency department.

Investigating Optimal Autologous Cellular Platforms for Prenatal or Perinatal Factor VIII Delivery to Treat Hemophilia A.

Patients with the severe form of hemophilia A (HA) present with a severe phenotype, and can suffer from life-threatening, spontaneous hemorrhaging. While prophylactic FVIII infusions have revolutionized the clinical management of HA, this treatment is short-lived, expensive, and it is not available to many A patients worldwide. In the present study, we evaluated a panel of readily available cell types for their suitability as cellular vehicles to deliver long-lasting FVIII replacement following transduction with a retroviral vector encoding a B domain-deleted human F8 transgene. Given the immune hurdles that currently plague factor replacement therapy, we focused our investigation on cell types that we deemed to be most relevant to either prenatal or very early postnatal treatment and that could, ideally, be autologously derived. Our findings identify several promising candidates for use as cell-based FVIII delivery vehicles and lay the groundwork for future mechanistic studies to delineate bottlenecks to efficient production and secretion of FVIII following genetic-modification.