Langerhans cell histiocytosis: NACHO update on progress, chaos, and opportunity on the path to rational cures.

Langerhans cell histiocytosis (LCH) is a myeloid neoplastic disorder characterized by lesions with CD1a-positive/Langerin (CD207)-positive histiocytes and inflammatory infiltrate that can cause local tissue damage and systemic inflammation. Clinical presentations range from single lesions with minimal impact to life-threatening disseminated disease. Therapy for systemic LCH has been established through serial trials empirically testing different chemotherapy agents and durations of therapy.

Racism and Resistance: A Qualitative Study of Bias As a Barrier to Breastfeeding.

Nearly 75% of Black non-Hispanic babies born in 2016 ever breastfed. However, Black mothers still experience barriers to breastfeeding, perpetuating disparities in exclusivity and duration. Using data collected from five focus groups with Black mothers ( = 30) in Washington, District of Columbia during summer 2019, we critically examine the influence of institutionalized and personally mediated racism on breastfeeding.

Cost-Effectiveness of Pharmacomechanical Catheter-Directed Thrombolysis Versus Standard Anticoagulation in Patients With Proximal Deep Vein Thrombosis: Results From the ATTRACT Trial.

Background: In patients with acute deep vein thrombosis (DVT), pharmacomechanical catheter-directed thrombolysis (PCDT) in conjunction with anticoagulation therapy is increasingly used with the goal of preventing postthrombotic syndrome. Long-term costs and cost-effectiveness of these 2 treatment strategies from the perspective of the US healthcare system have not been compared.

Methods And Results: Between 2009 and 2014, the ATTRACT trial (Acute Venous Thrombosis: Thrombus Removal With Adjunctive Catheter-Directed Thrombolysis) randomized 692 patients with acute proximal DVT to PCDT plus anticoagulation (n=337) or standard treatment with anticoagulation alone (n=355).

Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO).

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, often associated with genetic defects of lymphocyte cytotoxicity. Though a distinctive constellation of features has been described for HLH, diagnosis remains challenging as patients have diverse presentations associated with a variety of triggers. We propose two concepts to clarify how HLH is diagnosed and treated: within the broader syndrome of HLH, "HLH disease" should be distinguished from "HLH disease mimics" and HLH subtypes should be categorized by specific etiologic associations, not the ambiguous dichotomy of "primary" and "secondary.

Clinical Metagenomic Sequencing for Diagnosis of Meningitis and Encephalitis.

Background: Metagenomic next-generation sequencing (NGS) of cerebrospinal fluid (CSF) has the potential to identify a broad range of pathogens in a single test.

Methods: In a 1-year, multicenter, prospective study, we investigated the usefulness of metagenomic NGS of CSF for the diagnosis of infectious meningitis and encephalitis in hospitalized patients. All positive tests for pathogens on metagenomic NGS were confirmed by orthogonal laboratory testing.

Disseminability of computerized cognitive training: Performance across coaches.

Cogmed is a computerized cognitive intervention utilizing coaches who receive standardized instruction in analyzing training indices and tailoring feedback to remotely monitor participant's performance. The goal of this study was to examine adherence, satisfaction, and efficacy of Cogmed across coaches. Survivors of pediatric brain tumors and acute lymphoblastic leukemia (N = 68) were randomized to intervention (Cogmed) or waitlist control.

Safety, Tolerability, and Pharmacokinetics of Liposomal Amphotericin B in Immunocompromised Pediatric Patients.

The safety, tolerability, and pharmacokinetics of the liposomal formulation of amphotericin B (L-AMB) were evaluated in 40 immunocompromised children and adolescents. The protocol was an open-label, sequential-dose-escalation, multidose pharmacokinetic study with 10 to 13 patients in each of the four dosage cohorts. Each cohort received daily dosages of 2.

Population Pharmacokinetics of Liposomal Amphotericin B in Immunocompromised Children.

Liposomal amphotericin B (LAmB) is widely used in the treatment of invasive fungal disease (IFD) in adults and children. There are relatively limited pharmacokinetic (PK) data to inform optimal dosing in children that achieves systemic drug exposures comparable to those of adults. Our objective was to describe the pharmacokinetics of LAmB in children aged 1 to 17 years with suspected or documented IFD.

Computerized Cognitive Training for Amelioration of Cognitive Late Effects Among Childhood Cancer Survivors: A Randomized Controlled Trial.

Purpose: Children receiving CNS-directed therapy for cancer are at risk for cognitive problems, with few available empirically supported interventions. Cognitive problems indicate neurodevelopmental disruption that may be modifiable with intervention. This study evaluated short-term efficacy of a computerized cognitive training program and neural correlates of cognitive change.

Disrupting NOTCH Slows Diffuse Intrinsic Pontine Glioma Growth, Enhances Radiation Sensitivity, and Shows Combinatorial Efficacy With Bromodomain Inhibition.

NOTCH regulates stem cells during normal development and stemlike cells in cancer, but the roles of NOTCH in the lethal pediatric brain tumor diffuse intrinsic pontine glioma (DIPG) remain unknown. Because DIPGs express stem cell factors such as SOX2 and MYCN, we hypothesized that NOTCH activity would be critical for DIPG growth. We determined that primary DIPGs expressed high levels of NOTCH receptors, ligands, and downstream effectors.

Seizures, encephalopathy, and vaccines: experience in the National Vaccine Injury Compensation Program.

Objectives: To describe the demographic and clinical characteristics of children for whom claims were filed with the National Vaccine Injury Compensation Program (VICP) alleging seizure disorder and/or encephalopathy as a vaccine injury.

Study Design: The National VICP within the Department of Health and Human Services compensates individuals who develop medical problems associated with a covered immunization. We retrospectively reviewed medical records of children younger than 2 years of age with seizures and/or encephalopathy allegedly caused by an immunization, where a claim was filed in the VICP between 1995 through 2005.

Incidental renal mass found on focused assessment with sonography in trauma.

We present the case of a 9-year-old female who presented after minor blunt abdominal trauma during a sporting event. On focused assessment with sonography for trauma, she was found to have an incidental renal mass, which was ultimately diagnosed as a renal cell carcinoma. In this report, we focus on the use of bedside ultrasound as a screening test and the management of incidental findings.

Educating pediatric residents about breastfeeding: evaluation of 3 time-efficient teaching strategies.

Background: Previously reported breastfeeding curricula for residents have combined different teaching methods, have focused on knowledge and attitudes, and have been time-intensive.

Objective: This study aimed to evaluate 3 time-efficient breastfeeding curricula for effectiveness in regard to pediatric residents' knowledge, confidence, and skills in managing a simulated breastfeeding scenario.

Methods: First-year pediatric residents during their 4-week community hospital newborn nursery rotation were consecutively assigned to 1 of 3 groups.

Impact of inpatient bronchiolitis clinical practice guideline implementation on testing and treatment.

Objective: To determine the association between institutional inpatient clinical practice guidelines (CPGs) for bronchiolitis and the use of diagnostic tests and treatments.

Study Design: A multicenter retrospective cohort study of infants aged 29 days to 24 months with a discharge diagnosis of bronchiolitis was conducted between July 2011 and June 2012. An electronic survey was sent to quality improvement leaders to determine the presence, duration, and method of CPG implementation at participating hospitals.

Critical role for death-receptor mediated apoptotic signaling in viral myocarditis.

Background: Apoptosis of cardiac myocytes plays a key role in the pathogenesis of many cardiac diseases, including viral myocarditis. The apoptotic signaling pathways that are activated during viral myocarditis and the role that these pathways play in disease pathogenesis have not been clearly delineated.

Methods And Results: We investigated the role of apoptotic signaling pathways after virus infection of primary cardiac myocytes.

Headache in a national sample of American children: prevalence and comorbidity.

The purpose of this study was to determine the prevalence, sociodemographic correlates, and comorbidity of recurrent headache in children in the United States. Participants were individuals aged 4 to 18 years (n = 10,198) who participated in the National Health and Nutrition Examination Surveys. Data on recurrent and other health conditions were analyzed.

Diagnostic value of lumbar puncture in afebrile infants with suspected new-onset seizures.

No established guidelines address the need for lumbar puncture in fever-free infants younger than 6 months of age with a first seizure. We analyzed cerebrospinal fluid results in infants and found that lumbar puncture adds little diagnostic value to the evaluation of young, well-appearing infants presenting with possible new-onset seizures.

Early postinduction intensification therapy improves survival for children and adolescents with high-risk acute lymphoblastic leukemia: a report from the Children's Oncology Group.

Longer and more intensive postinduction intensification (PII) improved the outcome of children and adolescents with "higher risk" acute lymphoblastic leukemia (ALL) and a slow marrow response to induction therapy. In the Children's Cancer Group study (CCG-1961), we tested longer versus more intensive PII, using a 2 x 2 factorial design for children with higher risk ALL and a rapid marrow response to induction therapy. Between November 1996 and May 2002, 2078 children and adolescents with newly diagnosed ALL (1 to 9 years old with white blood count 50 000/mm3 or more, or 10 years of age or older with any white blood count) were enrolled.

Sudden infant death syndrome.

Despite declines in prevalence during the past two decades, sudden infant death syndrome (SIDS) continues to be the leading cause of death for infants aged between 1 month and 1 year in developed countries. Behavioural risk factors identified in epidemiological studies include prone and side positions for infant sleep, smoke exposure, soft bedding and sleep surfaces, and overheating. Evidence also suggests that pacifier use at sleep time and room sharing without bed sharing are associated with decreased risk of SIDS.

Steroid-induced glaucoma in an infant.

A 3-week-old infant developed buphthalmos and glaucoma after a 1-week course of topical steroid drops. The glaucoma resolved after cessation of the medication. This report underscores the sensitivity of infants and very young children to steroid-related intraocular pressure rises.

Transition to adulthood in congenital heart disease: missed adolescent milestones.

Purpose: To determine whether adults with congenital heart disease (CHD) are better adjusted than adults with acquired heart disease (AHD).

Methods: Twenty-five adults with CHD completed the Patient Adjustment to Illness Self Report (PAIS-SR).

Results: CHD patients had good adjustment for Heath Care Orientation and Family Relations, but poorer adjustment for Vocational, Social and Domestic Environment, Sexual Relations, and Psychological Distress.