Atrial fibrillation in adults with congenital heart disease following cardiac surgery in a single center: Analysis of incidence and risk factors.

Objective: The primary aim of our work is to determine the incidence of atrial fibrillation following cardiac surgery in adults with congenital heart disease. Secondary aims include identifying risk factors predictive of developing early postoperative atrial fibrillation and morbidities associated with early postoperative atrial fibrillation.

Design: Retrospective analysis.

Procalcitonin Levels in Critically Ill Children With Status Asthmaticus.

Background: Children with status asthmaticus (SA) often present with fever and are evaluated with chest radiographs (CXRs). In the absence of a confirmatory test for bacterial infection, antibiotics are started whenever there are radiological infiltrates or if there is a suspicion of pneumonia. We undertook this study to determine if serum procalcitonin (PCT) levels at admission are altered in critically ill children with SA.

Impact of cytogenetic abnormalities on outcomes of adult Philadelphia-negative acute lymphoblastic leukemia after allogeneic hematopoietic stem cell transplantation: a study by the Acute Leukemia Working Committee of the Center for International Blood and Marrow Transplant Research.

Cytogenetic risk stratification at diagnosis has long been one of the most useful tools to assess prognosis in acute lymphoblastic leukemia (ALL). To examine the prognostic impact of cytogenetic abnormalities on outcomes after allogeneic hematopoietic cell transplantation, we studied 1731 adults with Philadelphia-negative ALL in complete remission who underwent myeloablative or reduced intensity/non-myeloablative conditioning transplant from unrelated or matched sibling donors reported to the Center for International Blood and Marrow Transplant Research. A total of 632 patients had abnormal conventional metaphase cytogenetics.

A case of sinus pause induced by swallowing in the setting of olanzapine and guanfacine overdose.

Olanzapine, a second-generation antipsychotic, is used in both adult and pediatric populations for schizophrenia, bipolar disorder, and depression and has been associated with autonomic dysregulation in the setting of overdose. Guanfacine is a sympatholytic drug used in the treatment of attention deficit hyperactivity disorder and has also been associated with autonomic dysfunction. We present a unique case of a 17-year-old male who overdosed on 340 mg of olanzapine and 189 mg of extended-release guanfacine with a previously unreported adverse event.

A single centre experience with an evolving approach for the repair of coarctation of the aorta.

Background: Isolated coarctation of the aorta can be repaired by either lateral thoracotomy or sternotomy approach with end-to-end anastomosis. Most commonly, neonates with coarctation of the aorta also have hypoplasia of the arch, requiring median sternotomy and extended end-to-side anastomosis with arch augmentation. The aim of this study was to describe our experience as the institution adopted the median sternotomy approach for repair, by reviewing complications, mortality, and reintervention.

Sofosbuvir and Ribavirin Therapy for Children Aged 3 to <12 Years With Hepatitis C Virus Genotype 2 or 3 Infection.

Currently, the only approved hepatitis C virus (HCV) treatment for children aged <12 years is pegylated interferon plus ribavirin. In an open-label study, we evaluated the safety and efficacy of sofosbuvir plus ribavirin for 12 weeks in children aged 3 to <12 years chronically infected with genotype 2 or for 24 weeks in patients with genotype 3. Patients aged 3 to <6 years weighing <17 kg received sofosbuvir 150 mg, and patients aged 3 to <6 years weighing ≥17 kg and all patients aged 6 to <12 years received sofosbuvir 200 mg once daily.

A genomics-informed computational biology platform prospectively predicts treatment responses in AML and MDS patients.

Patients with myelodysplastic syndromes (MDS) or acute myeloid leukemia (AML) are generally older and have more comorbidities. Therefore, identifying personalized treatment options for each patient early and accurately is essential. To address this, we developed a computational biology modeling (CBM) and digital drug simulation platform that relies on somatic gene mutations and gene CNVs found in malignant cells of individual patients.

Thenar Muscle Oxygen Saturation Using Vascular Occlusion Test: A Novel Technique to Study Microcirculatory Abnormalities in Pediatric Heart Failure Patients.

Heart failure (HF) is associated with microcirculatory changes secondary to neuro-humoral imbalance, vascular stiffness and increased sympathetic tone. Near Infra-Red Spectroscopy (NIRS) derived Thenar muscle tissue oxygenation levels (StO) can provide an estimate of the functional status of microcirculation. There is a paucity of literature regarding evaluation of microcirculation in pediatric subjects with HF.

Impact of T Cell Dose on Outcome of T Cell-Replete HLA-Matched Allogeneic Peripheral Blood Stem Cell Transplantation.

Data on whether the T cell dose of allogeneic peripheral blood stem cell (PBSC) products influences transplantation outcomes are conflicting. Using the Center for International Blood and Marrow Transplant Research database, we identified 2736 adult patients who underwent first allogeneic PBSC transplantation for acute leukemia or myelodysplastic syndrome between 2008 and 2014 using an HLA-matched sibling donor (MSD) or an 8/8-matched unrelated donor (MUD). We excluded ex vivo and in vivo T cell-depleted transplantations.

Real-World Effectiveness of Common Treatment Strategies for Juvenile Idiopathic Arthritis: Results From a Canadian Cohort.

Objective: Undervaluing the effectiveness of conventional treatments may lead to overtreatment with biologic medications in children with juvenile idiopathic arthritis (JIA). Using data from a nationwide inception cohort and strict methods to control bias, the aim of our study was to estimate the real-world effectiveness of simple JIA treatment strategies recommended in current guidelines.

Methods: Children with JIA who were recruited at 16 Canadian centers from 2005 to 2010 were followed for up to 5 years.

Infusion of Hematopoietic Stem Cell Products Using Pump Mechanism: An Approach Worth Consideration?

Hematopoietic stem cell transplantation using progenitor cells is a potentially curative treatment option for patients with high-risk malignancies and nonmalignant hematologic, immunologic, and genetic conditions. There is a need for evidence regarding safe practices and controlled infusion processes. Syringe and intravenous infusion pumps are not commonly used to deliver hematopoietic stem cell products (HPCs) due to a paradigm of thought that suggests that the pressure from the pump might damage the HPCs.

Thenar Muscle Oxygen Saturation Levels: A Surrogate for Central Venous Oxygen Saturation?

Purpose: Shock is associated with increased tissue oxygen extraction. Near-infrared spectroscopy-derived thenar muscle tissue oxygenation (StO) levels can provide an estimate of the oxygen supply-demand balance at the tissue level. We hypothesized that thenar StO levels would correlate with central venous oxygen saturation (ScvO) levels, the gold standard for global tissue oxygen extraction in the body.

Left Ventricular Aneurysm Following Blunt-Force Trauma in a Child.

• LV aneurysms are rare in the pediatric population. • Given different implications, it is key to distinguish aneurysm and pseudoaneurysm. • The authors describe multimodality imaging to manage a pediatric LV aneurysm.

Establishing an Updated Core Domain Set for Studies in Juvenile Idiopathic Arthritis: A Report from the OMERACT 2018 JIA Workshop.

Objective: The current Juvenile Idiopathic Arthritis (JIA) Core Set used in randomized controlled trials (RCT) and longitudinal observational studies (LOS) was developed without the input of patients/parents. At the Outcome Measures in Rheumatology (OMERACT) 2016, a special interest group voted to reconsider the core set, incorporating broader input. We describe subsequent work culminating in an OMERACT 2018 plenary and consensus voting.

Female low dose tip syringes-increased complexity of use may compromise dosing accuracy in paediatric patients.

What Is Known And Objective: The International Organization for Standardization (ISO) created enteral device specifications to reduce tubing misconnections. The Global Enteral Device Supplier Association (GEDSA) supports a female design: standard and low dose tip (LDT). Concerns include increased complexity of use with adapters, dosing accuracy and workflow.

Predicting Which Children with Juvenile Idiopathic Arthritis Will Not Attain Early Remission with Conventional Treatment: Results from the ReACCh-Out Cohort.

Objective: To estimate the probability of early remission with conventional treatment for each child with juvenile idiopathic arthritis (JIA). Children with a low chance of remission may be candidates for initial treatment with biologics or triple disease-modifying antirheumatic drugs (DMARD).

Methods: We used data from 1074 subjects in the Research in Arthritis in Canadian Children emphasizing Outcomes (ReACCh-Out) cohort.

Outcomes of Children With Critical Bronchiolitis Meeting at Risk for Pediatric Acute Respiratory Distress Syndrome Criteria.

Objectives: New definitions of pediatric acute respiratory distress syndrome include criteria to identify a subset of children "at risk for pediatric acute respiratory distress syndrome." We hypothesized that, among PICU patients with bronchiolitis not immediately requiring invasive mechanical ventilation, those meeting at risk for pediatric acute respiratory distress syndrome criteria would have worse clinical outcomes, including higher rates of pediatric acute respiratory distress syndrome development.

Design: Single-center, retrospective chart review.

Ombitasvir/Paritaprevir/Ritonavir With or Without Dasabuvir and With or Without Ribavirin for Adolescents With HCV Genotype 1 or 4.

In adults, treatment of hepatitis C virus (HCV) infection with ombitasvir (OBV)/paritaprevir (PTV)/ritonavir (r) with or without dasabuvir (DSV) and ±ribavirin (RBV) results in high rates of sustained virologic response (SVR). However, these regimens have not been investigated in adolescents. This ongoing, open-label, phase 2/3 study evaluated the pharmacokinetics, safety, and efficacy of OBV/PTV/r+DSV±RBV treatment for 12 weeks in adolescents infected with HCV genotype (GT) 1 without cirrhosis (part 1) and the safety and efficacy of OBV/PTV/r±DSV±RBV treatment for 12 or 24 weeks in adolescents infected with GT1 or GT4 without cirrhosis or with compensated cirrhosis (parts 1 and 2).

Prospective Determination of the Incidence and Risk Factors of New-Onset Uveitis in Juvenile Idiopathic Arthritis: The Research in Arthritis in Canadian Children Emphasizing Outcomes Cohort.

Objective: Identification of the incidence of juvenile idiopathic arthritis (JIA)-associated uveitis and its risk factors is essential to optimize early detection. Data from the Research in Arthritis in Canadian Children Emphasizing Outcomes inception cohort were used to estimate the annual incidence of new-onset uveitis following JIA diagnosis and to identify associated risk factors.

Methods: Data were reported every 6 months for 2 years, then yearly to 5 years.

Recurrent nonbacterial thrombotic endocarditis: A novel therapeutic approach.

We present the case of a 29-year-old male with a history of Mizuho hemolytic anemia, a rare form of unstable hemoglobinopathy, who presented with congestive heart failure secondary to recurrent valve thrombosis despite appropriate oral anticoagulation. He subsequently required mitral and aortic valve replacement. Pathologic examination revealed extensive nonbacterial thrombotic endocarditis.

Safety of a 2-Day Antibiotic Regimen After Delayed Chest Closure Post Pediatric Cardiac Surgery.

Background: There is no consensus for the length of prophylactic antibiotics after delayed chest closure (DCC) postcardiac surgery in pediatrics. In September 2014, our institution's pediatric cardiac intensive care unit changed the policy on length of prophylactic antibiotics after DCC from 5 days (control) to 2 days (study group). The objective of the study was to determine whether a 2-day course of antibiotics is as effective as a 5-day course in preventing blood stream and sternal wound infections in pediatric DCC.