Impact of pre-transplant induction and consolidation cycles on AML allogeneic transplant outcomes: a CIBMTR analysis in 3113 AML patients.

We investigated the impact of the number of induction/consolidation cycles on outcomes of 3113 adult AML patients who received allogeneic hematopoietic cell transplantation (allo-HCT) between 2008 and 2019. Patients received allo-HCT using myeloablative (MAC) or reduced-intensity (RIC) conditioning in first complete remission (CR) or with primary induction failure (PIF). Patients who received MAC allo-HCT in CR after 1 induction cycle had 1.

Evolution and Revolution of Imaging Technologies in Neurosurgery.

We understand only a small fraction of the events happening in our brains; therefore, despite all the progress made thus far, a whole array of questions remains. Nonetheless, neurosurgeons invented new tools to circumvent the challenges that had plagued their predecessors. With the manufacturing boom of the 20th century, technological innovations blossomed enabling the neuroscientific community to study and operate upon the living brain in finer detail and with greater precision while avoiding harm to the nervous system.

Monogenic early-onset lymphoproliferation and autoimmunity: Natural history of STAT3 gain-of-function syndrome.

Background: In 2014, germline signal transducer and activator of transcription (STAT) 3 gain-of-function (GOF) mutations were first described to cause a novel multisystem disease of early-onset lymphoproliferation and autoimmunity.

Objective: This pivotal cohort study defines the scope, natural history, treatment, and overall survival of a large global cohort of patients with pathogenic STAT3 GOF variants.

Methods: We identified 191 patients from 33 countries with 72 unique mutations.

Prolonged hospital length of stay after pediatric heart transplantation: A machine learning and logistic regression predictive model from the Pediatric Heart Transplant Society.

Background: Heart transplantation (HT) is the gold standard for managing end-stage heart failure. Multiple quality metrics, including length of stay (LOS), have been used in solid organ transplantation. However, limited data are available regarding trends and factors influencing LOS after pediatric HT.

Management of pediatric appendicitis during the COVID-19 pandemic: A nationwide multicenter cohort study.

Background: The COVID-19 pandemic has impacted timely access to care for children, including patients with appendicitis. This study aimed to evaluate the effect of the COVID-19 pandemic on management of appendicitis and patient outcomes.

Methods: A multicenter retrospective study was performed including 19 children's hospitals from April 2019-October 2020 of children (age≤18 years) diagnosed with appendicitis.

Social determinants of health in pediatric scald burns: Is food access an issue?

Background: Burn injury risk, severity, and outcomes have been associated with socioeconomic status. Limited data exist to evaluate health access-related influences at a structural population level. This study evaluated factors at the Census-tract level, specifically evaluating food access and social vulnerability in pediatric scald burns.

Nitric Oxide on Extracorporeal Life Support-Circuit Modifications for a Safe Therapy.

Nitric oxide (NO) incorporation into the sweep gas of the extracorporeal life support (ECLS) circuit has been proposed as a strategy to ameliorate the insults caused by the systemic inflammatory response. This technical study describes circuit modifications allowing nitric oxide to be incorporated into the circuit and describing and validating the oxygenator sweep flow rates necessary to achieve consistent safe delivery of the therapy. For patients requiring sweep rates less than 2 L/min, a simplified setup, incorporating a pressure relief valve/low flow meter in the gas delivery line, was placed in line between the blender/NO injector module and the NO sampling port/oxygenator.

Vitamin D Supplementation: Association With Serum Cytokines in Pediatric Hematopoietic Stem Cell Transplantation.

Vitamin D deficiency is prevalent in pediatric patients presenting for hematopoietic stem cell transplantation (HSCT) and has been linked to poor clinical outcomes. Using the data from a randomized control trial, in this paper we explore the effects of vitamin D supplementation on circulating cytokine levels during pediatric HSCT (www.clinicaltrials.

Integrated PERSEVERE and endothelial biomarker risk model predicts death and persistent MODS in pediatric septic shock: a secondary analysis of a prospective observational study.

Background: Multiple organ dysfunction syndrome (MODS) is a critical driver of sepsis morbidity and mortality in children. Early identification of those at risk of death and persistent organ dysfunctions is necessary to enrich patients for future trials of sepsis therapeutics. Here, we sought to integrate endothelial and PERSEVERE biomarkers to estimate the composite risk of death or organ dysfunctions on day 7 of septic shock.

Use of REGEN-COV in children after heart transplantation for treatment and post-exposure prophylaxis of COVID-19.

COVID-19 pandemic continues to evolve and new variants like Delta and Omicron have been discovered. REGEN-COV is a recombinant human monoclonal antibody to the spike protein of SARS-CoV-2 which received emergency use authorisation for treatment and post-exposure prophylaxis in patients with high risk of progression to severe disease. We review our experience with use of REGEN-COV in paediatric heart transplant patients.

Multicenter Assessment of Cryoanalgesia Use in Minimally Invasive Repair of Pectus Excavatum: A 20-center Retrospective Cohort Study.

Objective: To assess the clinical implications of cryoanalgesia for pain management in children undergoing minimally invasive repair of pectus excavatum (MIRPE).

Background: MIRPE entails significant pain management challenges, often requiring high postoperative opioid use. Cryoanalgesia, which blocks pain signals by temporarily ablating intercostal nerves, has been recently utilized as an analgesic adjunct.

Mechanical Ventilation during ECMO: Lessons from Clinical Trials and Future Prospects.

Acute Respiratory Distress Syndrome (ARDS) accounts for 10% of ICU admissions and affects 3 million patients each year. Despite decades of research, it is still associated with one of the highest mortality rates in the critically ill. Advances in supportive care, innovations in technologies and insights from recent clinical trials have contributed to improved outcomes and a renewed interest in the scope and use of Extracorporeal life support (ECLS) as a treatment for severe ARDS, including high flow veno-venous Extracorporeal Membrane Oxygenation (VV-ECMO) and low flow Extracorporeal Carbon Dioxide Removal (ECCO2R).

Natural Killer Cell Alloreactivity Predicted By Killer Cell Immunoglobulin-Like Receptor Ligand Mismatch Does Not Impact Engraftment in Umbilical Cord Blood and Haploidentical Stem Cell Transplantation.

Natural killer cell alloreactivity is determined by killer cell immunoglobulin-like receptor (KIR) ligands in donor and recipient pairs. A small, single institution study suggested that the risk of primary graft failure after cord blood hematopoietic cell transplantation (CBT) can be predicted by host-versus-graft (HvG)-directed natural killer cell alloreactivity. In the haploidentical transplantation (Haplo HCT) cohort, graft failures were observed only in graft-versus-host (GvH) KIR ligand mismatched pairs.

Impact of Adequate Day 30 Post-Pediatric Hematopoietic Stem Cell Transplantation Vitamin D Level on Clinical Outcome: An Observational Cohort Study.

This prospective observational study evaluated the impact of adequate vitamin D levels by day +30 after vitamin D supplementation on early post-HSCT outcomes, including acute graft-versus-host disease (aGVHD), immune recovery, infection rates, and overall survival. Forty children (age 2 to 16 years) undergoing hematopoietic stem cell transplantation (HSCT) were given vitamin D supplementation, were followed prospectively from day +30 post-transplantation, and had day +30 vitamin D levels measured. Thirty patients with normal vitamin D levels (≥30 ng/mL) were compared with 10 patients with low day +30 vitamin D levels (<30 ng/mL).

IV Sotalol Use in Pediatric and Congenital Heart Patients: A Multicenter Registry Study.

Background There is limited information regarding the clinical use and effectiveness of IV sotalol in pediatric patients and patients with congenital heart disease, including those with severe myocardial dysfunction. A multicenter registry study was designed to evaluate the safety, efficacy, and dosing of IV sotalol. Methods and Results A total of 85 patients (age 1 day-36 years) received IV sotalol, of whom 45 (53%) had additional congenital cardiac diagnoses and 4 (5%) were greater than 18 years of age.

Directly observed therapy to promote medication adherence in adolescent heart transplant recipients.

Purpose: HT recipients experience high levels of medication non-adherence during adolescence. This pilot study examined the acceptability and feasibility of an asynchronous DOT mHealth application among adolescent HT recipients. The app facilitates tracking of patients' dose-by-dose adherence and enables transplant team members to engage patients.

Evaluation of the Neonatal Sequential Organ Failure Assessment and Mortality Risk in Preterm Infants with Necrotizing Enterocolitis.

Introduction: The neonatal sequential organ failure assessment (nSOFA) score is a tool for calculating mortality risk of infants in the neonatal intensive care unit. The utility of the nSOFA in determining the risk of mortality or the association with surgical intervention among infants with necrotizing enterocolitis (NEC) has not been investigated.

Methods: We performed a retrospective, cohort study of preterm (<37 weeks) infants with NEC Bell's stage ≥ IIA at six hospitals from 2008 to 2020.

Risk Factors for CMV Viremia and Treatment-Associated Adverse Events Among Pediatric Hematopoietic Stem Cell Transplant Recipients.

Background: Cytomegalovirus (CMV) causes substantial morbidity and mortality after hematopoietic stem cell transplantation (HSCT). There are limited data on risk factors for CMV viremia and the safety of antiviral medications used to treat CMV in children.

Methods: We conducted a single-center retrospective study of children who underwent HSCT between 2000 and 2016.

Assessment of Current Society of Thoracic Surgeons Data Elements for Adults With Congenital Heart Disease.

Background: To identify opportunities for enhanced data collection for adults with congenital heart disease (ACHD), a structured review of existing variables in The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database (CHSD) and the STS Adult Cardiac Surgery Database (ACSD) was conducted.

Methods: A working group was assembled representing multiple STS Workforces and Task Forces. The ACSD was reviewed systematically over eight 90-minute calls.

Bolus gastric feeds improve nutrition delivery to mechanically ventilated pediatric medical patients: Results of the COntinuous vs BOlus multicenter trial.

Background: Comparison of bolus gastric feeding (BGF) vs continuous gastric feeding (CGF) with respect to timing and delivery of energy and protein in mechanically ventilated (MV) pediatric patients has not been investigated. We hypothesized that bolus delivery would shorten time to goal nutrition and increase the percentage of goal feeds delivered.

Methods: Multicenter, prospective, randomized comparative effectiveness trial conducted in seven pediatric intensive care units (PICUs).

Human factors in ECLS - A keystone for safety and quality - A narrative review for ECLS providers.

Introduction: Although the technology used for extracorporeal life support (ECLS) has improved greatly in recent years, the application of these devices to the patient is quite complex and requires extensive training of team members both individually and together. Human factors is an area that addresses the activities, contexts, environments, and tools which interact with human behavior in determining overall system performance.

Hypothesis: Analyses of the cognitive behavior of ECLS teams and individual members of these teams with respect to the occurrence of human errors may identify additional opportunities to enhance safety in delivery of ECLS.