COVID-19 associated Multisystem Inflammatory Syndrome in Children (MIS-C) guidelines; revisiting the Western New York approach as the pandemic evolves.

Multisystem inflammatory syndrome of children (MIS-C) continues to be a highly concerning diagnosis in those recently infected with SARS-CoV-2. The diagnosis of MIS-C cases will likely become even more challenging as vaccine uptake and natural immunity in previously infected persons leads to lower circulating rates of SARS-CoV-2 infection and will make cases sporadic. Febrile children presenting with cardiac dysfunction, symptoms overlapping Kawasaki disease or significant gastrointestinal complaints warrant a thorough screen in emergency departments, urgent care centers, and outpatient pediatric or family medicine practices. An increased index of suspicion and discussion regarding higher level of care (transferring to pediatric tertiary care centers or to intensive care) continues to be recommended. Herein we outline a broad approach with a multidisciplinary team for those meeting the case definition and believe such an approach is crucial for successful outcomes.

Outcome of Heart Disease in Syrian Refugee Children: Insights into Crisis.

BACKGROUND: There are many challenges facing Syrian refugee children with heart disease. In this report, we present the spectrum, management, and outcome of heart disease in Syrian refugee children over six-year period, highlighting challenges in management and availability of funding. METHODS: Data on Syrian refugee children with heart disease diagnosed between 2012 and 2017 were collected. Patients were followed until January 2019. Data reported included age, diagnosis, recommended treatment, types of procedures done, mortality, cost, financial sources for procedures, and outcome. RESULTS: 415 Syrian refugee children were diagnosed with heart disease at our institution. Median age was 1·9 years (0·4-6·05) years. Children were either born in Syria and fled to Jordan with their families (224, 54%), or born in Jordan to refugee parents (191, 46%). Follow-up was established for 335 patients (81%). Of 196 patients needing surgery, 130 (72%) underwent Surgery, and of 97 patients needing interventional catheterization, 95 underwent the procedure. Waiting time was 222(± 272) days for surgery and 67(± 75) days for catheterizations. Overall mortality was 17% (56 patients), of which 28 died while waiting for surgery. Cost of surgical and interventional catheterization procedures was $7820 (± $4790) and $2920 (± $2140), respectively. Funding was obtained mainly from non-government organizations, private donors, and United Nations fund. CONCLUSION: Despite local and international efforts to manage Syrian refugee children with heart disease, there is significant shortage in providing treatment resulting in delays and mortality. More organized efforts are needed to help with this ongoing crisis.

Necrotizing Enterocolitis Complicating Severe RSV Bronchiolitis in PICU Settings.

This retrospective study aims to analyze the baseline characteristics and factors associated with poor outcomes in patients with necrotizing enterocolitis (NEC) complicating respiratory syncytial virus (RSV) infection. Using the Virtual Pediatric Systems data registry, patients under 2 years admitted to the pediatric intensive care unit (PICU) were screened. Patients with documented RSV infection and NEC, intestinal perforation, noninfectious gastroenteritis/colitis, or pneumatosis intestinalis occurring around the timing of RSV bronchiolitis diagnosis were included. Out of the screened patients, 41 were analyzed. Most patients (93%) were aged 30 days to 2 years, one-third had baseline anatomical cardiac defects, and 20% history of prematurity. Median PICU length of stay was 11.7 days. Seven patients died before hospital discharge. While not statistically significant, nonsurvivors tended to exhibit higher PRISM-3 scores, more acidemia, and lower systolic blood pressure. These findings emphasize the need for cautious assessment of gastrointestinal symptoms in critically ill patients with RSV infection.

Negative-Pressure Ventilation in the Pediatric ICU.

Negative-pressure ventilation (NPV) is a form of noninvasive ventilation that has been recently utilized in pediatric acute respiratory failure. Negative-pressure ventilators apply negative pressure onto the chest wall via a cuirass to recruit areas of atelectasis. Continuous negative extrathoracic pressure, the most common mode, is similar to CPAP, where negative pressure is maintained at a constant level throughout the respiratory cycle while patients initiate their own breaths and continue to breathe spontaneously throughout. Control mode, which is similar to bi-level positive airway pressure, alternates negative pressure with positive pressure and controls both phases of breathing at a mandatory frequency set higher than the patient's spontaneous frequency. Supplemental oxygen is provided through a nasal cannula or face mask due of the lack of NPV devices' interface with the mouth or nose. NPV can improve preload to the heart and cardiac output (CO) in patients with restrictive right-ventricular physiology requiring CO augmentation and those with Fontan physiology. The purpose of this article is to review the physiological principles of spontaneous and NPV, examine the evidence supporting the use of NPV, give practical and meaningful guidance on its clinical application in the pediatric ICU, and summarize areas for future studies on its uses.

Mechanical Ventilation during ECMO: Best Practices.

Adults and children who require extracorporeal membrane oxygenation for respiratory failure remain at risk for ongoing lung injury if ventilator management is not optimized. This review serves as a guide to assist the bedside clinician in ventilator titration for patients on extracorporeal membrane oxygenation, with a focus on lung-protective strategies. Existing data and guidelines for extracorporeal membrane oxygenation ventilator management are reviewed, including non-conventional ventilation modes and adjunct therapies.

COVID-19 and Severe Rhabdomyolysis Causing Acute Kidney Injury and Life-Threatening Hyperkalemia in a Pediatric Patient: a Case Report.

Though initially believed to primarily be a respiratory pathogen, the SARS-CoV-2 virus has manifested as a virus that has the potential to affect multiple organ systems causing a wide variety of disease and symptomatology. Children have been largely spared in comparison to adult morbidity and mortality; however, acute pediatric illness secondary to COVID-19 infection has become both more common and more serious. Here, we present a teenager with acute COVID-19 who presented to the hospital with profound weakness and oliguria and was discovered to have severe rhabdomyolysis causing life-threatening hyperkalemia and acute kidney injury. He required treatment with emergent renal replacement therapy in the intensive care unit. His initial CK was 584,886 U/L. Creatinine was 14.1 mg/dL and potassium was 9.9 mmol/L. He was successfully treated with CRRT and was discharged on hospital day 13 with normal kidney function on follow-up. Rhabdomyolysis and acute kidney injury are increasingly recognized as complications of acute SARS-CoV-2 infection and require vigilance given the potentially fatal complications and long-standing morbidity associated with these conditions.

Mechanisms to expedite pediatric clinical trial site activation: The DOSE trial experience.

BACKGROUND: Timely trial start-up is a key determinant of trial success; however, delays during start-up are common and costly. Moreover, data on start-up metrics in pediatric clinical trials are sparse. To expedite trial start-up, the Trial Innovation Network piloted three novel mechanisms in the trial titled Dexmedetomidine Opioid Sparing Effect in Mechanically Ventilated Children (DOSE), a multi-site, randomized, double-blind, placebo-controlled trial in the pediatric intensive care setting. METHODS: The three novel start-up mechanisms included: 1) competitive activation; 2) use of trial start-up experts, called site navigators; and 3) supplemental funds earned for achieving pre-determined milestones. After sites were activated, they received a web-based survey to report perceptions of the DOSE start-up process. In addition to perceptions, metrics analyzed included milestones met, time to start-up, and subsequent enrollment of subjects. RESULTS: Twenty sites were selected for participation, with 19 sites being fully activated. Across activated sites, the median (quartile 1, quartile 3) time from receipt of regulatory documents to site activation was 82 days (68, 113). Sites reported that of the three novel mechanisms, the most motivating factor for expeditious activation was additional funding available for achieving start-up milestones, followed by site navigator assistance and then competitive site activation. CONCLUSION: Study start-up is a critical time for the success of clinical trials, and innovative methods to minimize delays during start-up are needed. Milestone-based funds and site navigators were preferred mechanisms by sites participating in the DOSE study and may have contributed to the expeditious start-up timeline achieved. CLINICALTRIALS: gov #: NCT03938857.

Mechanical Ventilation and Respiratory Support in the Pediatric Intensive Care Unit.

Children admitted to the pediatric intensive care unit often require respiratory support for the treatment of respiratory distress and failure. Respiratory support comprises both noninvasive modalities (ie, heated humidified high-flow nasal cannula, continuous positive airway pressure, bilevel positive airway pressure, negative pressure ventilation) and invasive mechanical ventilation. In this article, we review the various essential elements and considerations involved in the planning and application of respiratory support in the treatment of the critically ill children.

Extracorporeal Membrane Oxygenation for Pediatric Toxin Exposures: Review of the Extracorporeal Life Support Organization Registry.

Evidence for the use of extracorporeal membrane oxygenation (ECMO) in children with refractory respiratory failure or shock following ingestion or toxin exposure, has been confined to single-center experiences, individual case reports, and extrapolated from adult cohorts; no pediatric multicenter cohorts exist. The objective of this retrospective review of the Extracorporeal Life Support Organization registry is to describe pediatric ECMO use following ingestion or toxin exposure, and define factors associated with mortality. Twenty-eight children between the ages of 30 days and 18 years met inclusion criteria between January 1, 2008 and December 31, 2017. The primary outcome measure was mortality before hospital discharge, which occurred in 32% of patients. Factors associated with in-hospital mortality included pre-ECMO use of inhaled nitric oxide (44.4% vs. 5.3%, p = 0.026), lower pre-ECMO arterial blood gas pH (6.97 [6.80-7.17] vs. 7.20 [7.15-7.32], p = 0.034), and higher pre-ECMO PaCO2 (79 [57-85] vs. 49 [38-63], p = 0.014). Receipt of inotropic support during ECMO was more common in nonsurvivors (66.7% vs. 21.1%, p = 0.035). Extracorporeal membrane oxygenation should be considered in the most severe pediatric toxin exposures as a bridge to recovery, providing time for both toxin elimination and end-organ recovery.

A Rare Presentation of Multi-System Inflammatory Disease in Children Associated With Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2).

Management of multi-system inflammatory disease in children (MIS-C) remains a challenge due to the evolving nature of the coronavirus disease 2019 (COVID-19) pandemic. This article reports a rare presentation of multi-system inflammatory disease in a previously healthy 16-month-old male who fully recovered with minimal residual cardiac insufficiency upon discharge. Our case is unique due to patient's young age, cardiac findings, and his response to our treatment protocol. A multi-disciplinary team in a tertiary center was involved with care.

Extracorporeal Life Support: Four Decades and Counting.

Extracorporeal membrane oxygenation (ECMO) or extracorporeal life support (ECLS) is a form of heart lung bypass that is used to support neonates, pediatrics, and adult patients with cardiorespiratory failure for days or weeks till organ recovery or transplantation. Venoarterial (VA) and venovenous (VV) ECLS are the most common modes of support. ECLS circuit components and monitoring have been evolving over the last 40 years. The technology is safer, simpler, and more durable with fewer complications. The use of neonatal respiratory ECLS use has been declining over the last two decades, while adult respiratory ECLS is growing especially since the H1N1 influenza pandemic in 2009. This review provides an overview of ECLS evolution over the last four decades, its use in neonatal, pediatric and adults, description of basic principles, circuit components, complications, and outcomes as well as a quick look into the future.