Recent progress in the treatment of cancer in children.

Although significant improvements have been made in the outcomes of children with cancer, the pace of improvement has slowed in recent years as the limits of therapy intensification may have been reached for many pediatric cancers. Furthermore, with increasing numbers of pediatric cancer survivors, the long-term side effects of treatment have become increasingly apparent. Therefore, attention has shifted to the use of molecularly targeted agents and immunotherapies to improve the outcomes of children who are not cured by traditional cytotoxic chemotherapies and to decrease exposure to cytotoxic chemotherapy and reduce late effects.

Assessing comfort level with pediatric skin specimens among dermatopathologists and pediatric pathologists: A national cross-sectional survey.

Background: Cutaneous histopathologic diagnoses in children often differ from those in adults. Depending on practice setting, these specimens may be evaluated by dermatopathologists or pediatric pathologists. We sought to determine whether comfort level with pediatric dermatopathology is associated with prior training, pediatric dermatopathology exposure during fellowship, career duration, or specimen subtype.

Appendectomy Versus Observation for Appendicitis in Neutropenic Children With Cancer.

Background: Optimal management of neutropenic appendicitis (NA) in children undergoing cancer therapy remains undefined. Management strategies include upfront appendectomy or initial nonoperative management. We aimed to characterize the effect of management strategy on complications and length of stay (LOS) and describe implications for chemotherapy delay or alteration.

Practical guidelines for monitoring and management of coagulopathy following tisagenlecleucel CAR T-cell therapy.

Cytokine release syndrome (CRS) is a systemic inflammatory response associated with chimeric antigen receptor T-cell (CAR-T) therapies. In severe cases, CRS can be associated with coagulopathy and hypofibrinogenemia. We present our global multicenter experience with CRS-associated coagulopathy after tisagenlecleucel therapy in 137 patients with relapsed or refractory B-cell acute lymphoblastic leukemia from the ELIANA and ENSIGN trials.

Heart Failure After Cavopulmonary Connection: Conversion to Biventricular Circulatory Support.

Mechanical support of patients with superior cavopulmonary connection is challenging; multiple factors contribute to failure: elevated pulmonary vascular resistance, aortopulmonary collateral flow, venovenous collaterals, ventricular dysfunction, and atrioventricular valve regurgitation. We report 2 cases of conversion from a single ventricle circulation to biventricular mechanical support by reestablishing caval continuity. Both patients have demonstrated recovery of end-organ function and participation in rehabilitation.

Arteritis and Large Vessel Occlusive Strokes in Children After COVID-19 Infection.

We describe 2 previously healthy children who suffered disabling arterial ischemic strokes because of acute intracranial large vessel occlusion within 3 to 4 weeks of coronavirus disease 2019 (COVID-19) infection. Both children presented from communities with high COVID-19 case rates in the Southwest United States. An 8-year-old American Indian girl experienced severe iron deficiency anemia requiring blood transfusion and presented with bilateral middle cerebral artery (MCA) distribution strokes 3 weeks later.

Basic Histopathologic Assessment of Germ Cell Tumors for Clinic and Research.

This chapter introduces the macroscopic and light microscopic features of testicular germ cell tumors (GCT) commonly encountered in clinical practice. Accurate diagnosis of these histologically diverse neoplasms is essential not only for clinical management but also for serving as the basis for interpretation of research findings. We will focus on general histopathologic concepts and discuss the use of immunohistochemistry (IHC) as an aid to the diagnosis.

Comparison of the platelet activation status of single-donor platelets obtained with two different cell separator technologies.

Background: The microparticle content (MP%) of apheresis platelets-a marker of platelet activation-is influenced by donor factors and by external stressors during collection and storage. This study assessed the impact of apheresis technology and other factors on the activation status (MP%) of single-donor apheresis platelets.

Study Design And Methods: Data from six US hospitals that screened platelets by measuring MP% through dynamic light scattering (ThromboLUX) were retrospectively analyzed.

Developing Content for Pediatric Hospital Medicine Certification Examination Using Practice Analysis.

Objectives: The American Board of Pediatrics (ABP) and the Pediatric Hospital Medicine (PHM) subboard developed a content outline to serve as a blueprint for the inaugural certification examination through practice analysis. The systematic approach of practice analyses process is described in the study.

Methods: A diverse, representative panel of 12 pediatric hospitalists developed the draft content outline using multiple resources (publications, textbooks, PHM Core Competencies, PHM fellow's curriculum, etc).

Perfusion-guided sonopermeation of neuroblastoma: a novel strategy for monitoring and predicting liposomal doxorubicin uptake .

Neuroblastoma (NB) is the most common extracranial solid tumor in infants and children, and imposes significant morbidity and mortality in this population. The aggressive chemoradiotherapy required to treat high-risk NB results in survival of less than 50%, yet is associated with significant long-term adverse effects in survivors. Boosting efficacy and reducing morbidity are therefore key goals of treatment for affected children.

DICER1-associated central nervous system sarcoma in children: comprehensive clinicopathologic and genetic analysis of a newly described rare tumor.

The spectrum of neoplasms associated with DICER1 variants continues to expand, with the recent addition of primary "DICER1-associated central nervous system sarcoma" (DCS). DCS is a high-grade malignancy predominantly affecting pediatric patients. Six pediatric DCS were identified through a combination of clinical diagnostic studies, archival inquiry, and interinstitutional collaboration.

Pediatric Airway Management in COVID-19 Patients: Consensus Guidelines From the Society for Pediatric Anesthesia's Pediatric Difficult Intubation Collaborative and the Canadian Pediatric Anesthesia Society.

The severe acute respiratory syndrome coronavirus 2 (coronavirus disease 2019 [COVID-19]) pandemic has challenged medical systems and clinicians globally to unforeseen levels. Rapid spread of COVID-19 has forced clinicians to care for patients with a highly contagious disease without evidence-based guidelines. Using a virtual modified nominal group technique, the Pediatric Difficult Intubation Collaborative (PeDI-C), which currently includes 35 hospitals from 6 countries, generated consensus guidelines on airway management in pediatric anesthesia based on expert opinion and early data about the disease.

Long-Term Neurobehavioral and Quality of Life Outcomes of Critically Ill Children after Glycemic Control.

Objectives: To investigate adaptive skills, behavior, and quality health-related quality of life in children from 32 centers enrolling in the Heart And Lung Failure-Pediatric INsulin Titration randomized controlled trial.

Study Design: This prospective longitudinal cohort study compared the effect of 2 tight glycemic control ranges (lower target, 80-100 mg/dL vs higher target, 150-180 mg/dL) 1-year neurobehavioral and health-related quality of life outcomes. Subjects had confirmed hyperglycemia and cardiac and/or respiratory failure.

Obstructive Sleep Apnea in Children With Autism.

Study Objectives: To describe the demographic and clinical characteristics of children with autism spectrum disorder (ASD) referred for polysomnography (PSG) and to look for predictors of obstructive sleep apnea (OSA) and severe OSA in these children.

Methods: This is a retrospective case series of children ages 2 to 18 years who underwent PSG between January 2009 and February 2015. Children were excluded if they had major comorbidities, prior tonsillectomy, or missing data.

Achievable Benchmarks of Care for Pediatric Readmissions.

Background: Most inpatient care for children occurs outside tertiary children's hospitals, yet these facilities often dictate quality metrics. Our objective was to calculate the mean readmission rates and the Achievable Benchmarks of Care (ABCs) for pediatric diagnoses by different hospital types: metropolitan teaching, metropolitan nonteaching, and nonmetropolitan hospitals.

Methods: We used a cross-sectional retrospective study of 30-day, all-cause, same-hospital readmission of patients less than 18 years old using the 2014 Healthcare Utilization Project National Readmission Database.

Clinical utilization of Chimeric Antigen Receptor T-cells (CAR-T) in B-cell acute lymphoblastic leukemia (ALL)-an expert opinion from the European Society for Blood and Marrow Transplantation (EBMT) and the American Society for Blood and Marrow Transplantation (ASBMT).

On August 30, 2017, the U.S. Food and Drug Administration (US-FDA) approved tisagenlecleucel (KYMRIAH, Novartis, Basel, Switzerland), a synthetic bioimmune product of anti-CD19 chimeric antigen receptor-T cells (CAR-T), for the treatment of children and young adults with relapsed/refractory B-cell acute lymphoblastic leukemia (B-ALL).

Clinical Utilization of Chimeric Antigen Receptor T Cells in B Cell Acute Lymphoblastic Leukemia: An Expert Opinion from the European Society for Blood and Marrow Transplantation and the American Society for Blood and Marrow Transplantation.

On August 30, 2017 the US Food and Drug Administration approved tisagenlecleucel (Kymriah; Novartis, Basel, Switzerland), a synthetic bioimmune product of anti-CD19 chimeric antigen receptor T cells (CAR-T), for the treatment of children and young adults with relapsed/refractory B cell acute lymphoblastic leukemia (B-ALL). With this new era of personalized cancer immunotherapy, multiple challenges are present, ranging from implementation of a CAR-T program to safe delivery of the drug, long-term toxicity monitoring, and disease assessments. To address these issues experts representing the American Society for Blood and Marrow Transplant, the European Society for Blood and Marrow Transplantation, the International Society of Cell and Gene Therapy, and the Foundation for the Accreditation of Cellular Therapy formed a global CAR-T task force to identify and address key questions pertinent for hematologists and transplant physicians regarding the clinical use of anti CD19 CAR-T therapy in patients with B-ALL.

Algorithm to detect pediatric provider attention to high BMI and associated medical risk.

We developed and validated an algorithm that uses combinations of extractable electronic-health-record (EHR) indicators (diagnosis codes, orders for laboratories, medications, and referrals) that denote widely-recommended clinician practice behaviors: attention to overweight/obesity/body mass index alone (BMI Alone), with attention to hypertension/other comorbidities (BMI/Medical Risk), or neither (No Attention). Data inputs used for each EHR indicator were refined through iterative chart review to identify and resolve modifiable coding errors. Validation was performed through manual review of randomly selected visit encounters (n = 308) coded by the refined algorithm.

Lenticulostriate vasculopathy in preterm infants: a new classification, clinical associations and neurodevelopmental outcome.

Objective: To examine the inter-rater reliability for the diagnosis of LSV on cranial ultrasound (cUS), determine the risk factors associated with LSV and its progression, and examine neurodevelopmental outcome.

Study Design: Prospective case-control study of neonates ≤32wks of gestation assessed for LSV by serial cUS (n = 1351) between 2012 and 2014 and their neurodevelopment at 18-36mon-corrected age compared to controls.

Results: Agreement for LSV on cUS improved from Κappa 0.

Craniofacial Flash: Minimizing Radiation Dose in Pediatric Craniofacial Computed Tomography.

Background And Purpose: The routine use of computed tomography (CT) in evaluation of pediatric craniofacial abnormalities is controversial. While there are benefits to preoperative imaging, there are risks related to ionizing radiation and sedation/anesthesia. In this study, the authors describe their experience with a new craniofacial CT protocol (Craniofacial-Flash) that provides bone detail equivalent to conventional CT, while delivering a substantially lower radiation dose and reducing the scan time to obviate the requirement of sedation/anesthesia.

Genomic Privacy.

Background: Genetic information is unique among all laboratory data because it not only informs the current health of the specific person tested but may also be predictive of the future health of the individual and, to varying degrees, all biological relatives.

Content: As DNA sequencing has become ubiquitous with decreasing cost, large repositories of genomic data have emerged from the domains of research, healthcare, law enforcement, international security, and recreational consumer interest (i.e.

Treatment of chronic rhinosinusitis with dornase alfa in patients with cystic fibrosis: a systematic review.

Background: A major component of sputum in cystic fibrosis (CF) patients is polymerized DNA, a byproduct of degraded neutrophils. Dornase alfa (dornase) selectively cleaves extracellular DNA and reduces the viscosity of sputum. It improves mucociliary clearance and pulmonary function.

Luc's Abscess and Temporomandibular Joint Septic Arthritis: Two Rare Sequelae of Acute Otitis Media.

A 5-year-old previously healthy child presented with right-sided otalgia, right facial and temporal swelling, and right jaw pain in the setting of 6 days of low-grade fever. The child had no trauma, vomiting, or prior dental treatments. On physical examination, the patient had facial swelling, erythema, and tenderness over the right temporal region along with trismus, as well as pain on palpation of the right temporomandibular joint (TMJ).