Pediatric craniosynostosis computed tomography: an institutional experience in reducing radiation dose while maintaining diagnostic image quality.

Background: Children with craniosynostosis may undergo multiple computed tomography (CT) examinations for diagnosis and post-treatment follow-up, resulting in cumulative radiation exposure.

Objective: To reduce the risks associated with radiation exposure, we evaluated the compliance, radiation dose reduction and clinical image quality of a lower-dose CT protocol for pediatric craniosynostosis implemented at our institution.

Materials And Methods: The standard of care at our institution was modified to replace pediatric head CT protocols with a lower-dose CT protocol utilizing 100 kV, 5 mAs and iterative reconstruction.

Choosing Wisely in Pediatric Hospital Medicine: 5 New Recommendations to Improve Value.

Objectives: The health care system faces ongoing challenges due to low-value care. Building on the first pediatric hospital medicine contribution to the American Board of Internal Medicine Foundation Choosing Wisely Campaign, a working group was convened to identify additional priorities for improving health care value for hospitalized children.

Methods: A study team composed of nominees from national pediatric medical professional societies was convened, including pediatric hospitalists with expertise in clinical care, hospital leadership, and research.

Global leadership in paediatric and congenital cardiac care: "global health advocacy, lift as you rise - an interview with Liesl J. Zühlke, MBChB, MPH, PhD".

Professor Liesl Zühlke is the focus of our fifth in a series of interviews in Cardiology in the Young entitled, "Global Leadership in Paediatric and Congenital Cardiac Care". Professor Zühlke (nee Hendricks) was born in Cape Town, South Africa. She would attend medical school in her hometown at University of Cape Town, graduating in 1991.

An adapted European LeukemiaNet genetic risk stratification for acute myeloid leukemia patients undergoing allogeneic hematopoietic cell transplant. A CIBMTR analysis.

Cytogenetic and molecular abnormalities are known to influence post-transplant outcomes in acute myeloid leukemia (AML) but data assessing the prognostic value of combined genetic models in the HCT setting are limited. We developed an adapted European LeukemiaNet (aELN) risk classification based on available genetic data reported to the Center for International Blood and Marrow Transplant Research, to predict post-transplant outcomes in 2289 adult AML patients transplanted in first remission, between 2013 and 2017. Patients were stratified according to aELN into three groups: favorable (Fav, N = 181), intermediate (IM, N = 1185), and adverse (Adv, N = 923).

Risk classification at diagnosis predicts post-HCT outcomes in intermediate-, adverse-risk, and KMT2A-rearranged AML.

Little is known about whether risk classification at diagnosis predicts post-hematopoietic cell transplantation (HCT) outcomes in patients with acute myeloid leukemia (AML). We evaluated 8709 patients with AML from the CIBMTR database, and after selection and manual curation of the cytogenetics data, 3779 patients in first complete remission were included in the final analysis: 2384 with intermediate-risk, 969 with adverse-risk, and 426 with KMT2A-rearranged disease. An adjusted multivariable analysis detected an increased risk of relapse for patients with KMT2A-rearranged or adverse-risk AML as compared to those with intermediate-risk disease (hazards ratio [HR], 1.

Characterization and Outcomes of Hospitalized Children With Coronavirus Disease 2019: A Report From a Multicenter, Viral Infection and Respiratory Illness Universal Study (Coronavirus Disease 2019) Registry.

Objectives: Multicenter data on the characteristics and outcomes of children hospitalized with coronavirus disease 2019 are limited. Our objective was to describe the characteristics, ICU admissions, and outcomes among children hospitalized with coronavirus disease 2019 using Society of Critical Care Medicine Discovery Viral Infection and Respiratory Illness Universal Study: Coronavirus Disease 2019 registry.

Design: Retrospective study.

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.

Diagnostic work-up for severe aplastic anemia in children: Consensus of the North American Pediatric Aplastic Anemia Consortium.

The North American Pediatric Aplastic Anemia Consortium (NAPAAC) is a group of pediatric hematologist-oncologists, hematopathologists, and bone marrow transplant physicians from 46 institutions in North America with interest and expertise in aplastic anemia, inherited bone marrow failure syndromes, and myelodysplastic syndromes. The NAPAAC Bone Marrow Failure Diagnosis and Care Guidelines Working Group was established with the charge of harmonizing the approach to the diagnostic workup of aplastic anemia in an effort to standardize best practices in the field. This document outlines the rationale for initial evaluations in pediatric patients presenting with signs and symptoms concerning for severe aplastic anemia.

Pediatric Resuscitation.

This article describes evidence-based nursing practices for detecting pediatric decompensation and prevention of cardiopulmonary arrest and outlines the process for effective and high-quality pediatric resuscitation and postresuscitation care. Primary concepts include pediatric decompensation signs and symptoms, pediatric resuscitation essential practices, and postresuscitation care, monitoring, and outcomes. Pediatric-specific considerations for family presence during resuscitation, ensuring good outcomes for medically complex children in community settings, and the role of targeted temperature management, continuous electroencephalography, and the use of extracorporeal membrane oxygenation in pediatric resuscitation are also discussed.

Use of bivalirudin for anticoagulation in pediatric extracorporeal membrane oxygenation (ECMO).

This study describes the use of bivalirudin in children on extracorporeal membrane oxygenation (ECMO). Pediatric patients receiving bivalirudin were compared to patients receiving heparin as the anticoagulant on ECMO. Data was collected for children under 18 years of age supported by ECMO from January 2016 to December 2019.

Standardizing Definitions of Hematopoietic Recovery, Graft Rejection, Graft Failure, Poor Graft Function, and Donor Chimerism in Allogeneic Hematopoietic Cell Transplantation: A Report on Behalf of the American Society for Transplantation and Cellular Therapy.

Allogeneic hematopoietic cell transplantation (allo-HCT) is potentially curative for certain hematologic malignancies and nonmalignant diseases. The field of allo-HCT has witnessed significant advances, including broadening indications for transplantation, availability of alternative donor sources, less toxic preparative regimens, new cell manipulation techniques, and novel GVHD prevention methods, all of which have expanded the applicability of the procedure. These advances have led to clinical practice conundrums when applying traditional definitions of hematopoietic recovery, graft rejection, graft failure, poor graft function, and donor chimerism, because these may vary based on donor type, cell source, cell dose, primary disease, graft-versus-host disease (GVHD) prophylaxis, and conditioning intensity, among other variables.

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

Medication non-adherence causes poor outcomes in paediatric organ transplantation. COVID-19 pandemic has led to an exponential use of mobile health approaches for patient care. Herein, we describe a pilot intervention study using mobile video directly observed therapy building on emerging trends in research and clinical practice pertaining to medication adherence in paediatric organ transplantation.

Machine learning-based prediction of health outcomes in pediatric organ transplantation recipients.

Objectives: Prediction of post-transplant health outcomes and identification of key factors remain important issues for pediatric transplant teams and researchers. Outcomes research has generally relied on general linear modeling or similar techniques offering limited predictive validity. Thus far, data-driven modeling and machine learning (ML) approaches have had limited application and success in pediatric transplant outcomes research.

Global leadership in paediatric and congenital cardiac care: "Humility in Leadership - an interview with Katarina Hanséus, MD, PhD, President of the Association for European Paediatric and Congenital Cardiology (AEPC)".

Dr. Katarina Hanséus is the focus of our fourth in a series of interviews in Cardiology in the Young entitled, "Global Leadership in Paediatric and Congenital Cardiac Care". Dr.

Kawasaki Disease Complicated by Coinfection.

Kawasaki disease is a medium vessel vasculitis with a multisystem presentation affecting 9-20 per 100,000 children under 5 years of age in the United States. coinfection has not been previously described. We present a 12-month-old vaccinated male with Kawasaki disease in the setting of bacteremia.

Stoss therapy is safe for treatment of vitamin D deficiency in pediatric patients undergoing HSCT.

Vitamin D deficiency remains common among pediatric patients undergoing hematopoietic stem cell transplant (HSCT) despite both aggressive and standard of care strategies. This study examined the safety and efficacy of single high-dose oral vitamin D therapy (Stoss therapy) for treatment of vitamin D deficiency in HSCT recipients. Patients ages 1-21 years presenting for HSCT were randomized to receive either Stoss regimen plus weekly/daily supplementation or standard of care, per US Endocrine Society guidelines.

Impact of depth of clinical response on outcomes of acute myeloid leukemia patients in first complete remission who undergo allogeneic hematopoietic cell transplantation.

Acute myeloid leukemia (AML) patients often undergo allogeneic hematopoietic cell transplantation (alloHCT) in first complete remission (CR). We examined the effect of depth of clinical response, including incomplete count recovery (CRi) and/or measurable residual disease (MRD), in patients from the Center for International Blood and Marrow Transplantation Research (CIBMTR) registry. We identified 2492 adult patients (1799 CR and 693 CRi) who underwent alloHCT between January 1, 2007 and December 31, 2015.

Spring-assisted minimally invasive repair of sagittal craniosynostosis.

Craniosynostosis surgery is intended to repair cranial deformity, reduce the risk of increased intracranial pressure from cephalocranial disproportion, and reduce the risk of developmental delays. In recent years, minimally invasive surgical techniques have been developed to achieve these goals with less tissue disruption, lower rates of transfusion, and shorter recovery time. The operation focuses on unlocking the fused bones, while reshaping relies on an adjunct, most commonly a postoperative cranial molding helmet.

Posttransplant cyclophosphamide is associated with increased cytomegalovirus infection: a CIBMTR analysis.

Prior studies suggest increased cytomegalovirus (CMV) infection after haploidentical donor transplantation with posttransplant cyclophosphamide (HaploCy). The role of allograft source and posttransplant cyclophosphamide (PTCy) in CMV infection is unclear. We analyzed the effect of graft source and PTCy on incidence of CMV infection, and effects of serostatus and CMV infection on transplant outcomes.