Selective activation of FZD2 and FZD7 reveals non-redundant function during mesoderm differentiation.

During gastrulation, Wnt-β-catenin signaling dictates lineage bifurcation generating different mesoderm cell types. However, the specific role of Wnt receptors in mesoderm specification remains elusive. Using selective Frizzled (FZD) and LRP5/6 antibody-based agonists, we examined FZD receptors' function during directed mesoderm differentiation of human pluripotent stem cells (hPSCs).

ChatGPT Use Among Pediatric Health Care Providers: Cross-Sectional Survey Study.

Background: The public launch of OpenAI's ChatGPT platform generated immediate interest in the use of large language models (LLMs). Health care institutions are now grappling with establishing policies and guidelines for the use of these technologies, yet little is known about how health care providers view LLMs in medical settings. Moreover, there are no studies assessing how pediatric providers are adopting these readily accessible tools.

Severe kidney dysfunction in sialidosis mice reveals an essential role for neuraminidase 1 in reabsorption.

Sialidosis is an ultra-rare multisystemic lysosomal disease caused by mutations in the neuraminidase 1 (NEU1) gene. The severe type II form of the disease manifests with a prenatal/infantile or juvenile onset, bone abnormalities, severe neuropathology, and visceromegaly. A subset of these patients present with nephrosialidosis, characterized by abrupt onset of fulminant glomerular nephropathy.

Evaluation of Research Diagnostic Criteria in Craniofacial Microsomia.

Characteristics of patients with craniofacial microsomia (CFM) vary in type and severity. The diagnosis is based on phenotypical assessment and no consensus on standardized clinical diagnostic criteria is available. The use of diagnostic criteria could improve research and communication among patients and healthcare professionals.

Outcomes of SARS-CoV-2 infection in 126 children and adolescents with central nervous system tumors.

Background: The Global Registry of COVID-19 in Childhood Cancer (GRCCC) seeks to describe the natural history of SARS-CoV-2 in children with cancer across the world. Here, we report the disease course and management of coronavirus disease 2019 (COVID-19) infection in the subset of children and adolescents with central nervous system (CNS) tumors who were included in the GRCCC until February 2021, the first data freeze.

Procedure: The GRCCC is a deidentified web-based registry of patients less than 19 years of age with cancer or recipients of a hematopoietic stem cell transplant and laboratory-confirmed SARS-CoV-2 infection.

Heated humidified high flow nasal cannula therapy in children with obstructive sleep apnea: A randomized cross-over trial.

Objective/background: Moderate-to-severe obstructive sleep apnea (OSA) is highly prevalent in children with obesity and/or underlying medical complexity. The first line of therapy, adenotonsillectomy (AT), does not cure OSA in more than 50% of these children. Consequently, continuous positive airway pressure (CPAP) is the main therapeutic option but adherence is often poor.

Secondary impacts of the COVID-19 pandemic at a tertiary children's hospital in Canada: a mixed-methods study.

Objectives: Decisions to pause all non-essential paediatric hospital activities during the initial phase of the COVID-19 pandemic may have led to significant delays, deferrals and disruptions in medical care. This study explores clinical cases where the care of children was perceived by hospital clinicians to have been negatively impacted because of the changes in healthcare delivery attributing to the restrictions placed resulting from the COVID-19 pandemic.

Design And Setting: This study used a mixed-methods approach using the following: (1) a quantitative analysis of overall descriptive hospital activity between May and August 2020, and utilisation of data during the study period was performed, and (2) a qualitative multiple-case study design with descriptive thematic analysis of clinician-reported consequences of the COVID-19 pandemic on care provided at a tertiary children's hospital.

Respiratory Management of Patients With Neuromuscular Weakness: An American College of Chest Physicians Clinical Practice Guideline and Expert Panel Report.

Background: Respiratory failure is a significant concern in neuromuscular diseases (NMDs). This CHEST guideline examines the literature on the respiratory management of patients with NMD to provide evidence-based recommendations.

Study Design And Methods: An expert panel conducted a systematic review addressing the respiratory management of NMD and applied the Grading of Recommendations, Assessment, Development, and Evaluations approach for assessing the certainty of the evidence and formulating and grading recommendations.

Upper and Lower Limb Anomalies in Craniofacial Microsomia and Its Relation to the OMENS+ Classification: A Multicenter Study of 688 Patients.

Background: Craniofacial microsomia (CFM) is characterized by several malformations related to the first and second pharyngeal arch. Patients typically present with facial asymmetry, but extracraniofacial organ systems might be involved, including limb anomalies. The purpose of this study was to analyze the occurrence of upper and lower limb anomalies in CFM patients.

What Should Clinicians and Patients Know About the Clinical Gaze, Disability, and Iatrogenic Harm When Making Decisions?

While clinicians, ethicists, and policymakers are increasingly aware that race, ethnicity, sexuality, gender, and class biases interfere with care provision, disability is not always considered as a confounding factor. This article explores the way embodiment affects personal and professional values. When patients who live with bodies others might not fully comprehend or embrace refuse-or challenge-clinical interventions, they offer real opportunities for clinicians to grasp the central role that embodied experience plays in how patients make health decisions and thereby avoid harming patients or undermining their relationships with patients.

Adolescent and young adult (AYA) oncology: A credentialed area of focused competence in Canada.

Background: Adolescents and young adults (AYA, ages 15-39 years) affected by cancer have unique treatment, survivorship, and palliation concerns. Current oncology training does not focus on the distinctive needs of this demographic. Amid this recognition, the Canadian National AYA Cancer Task Force and Canadian Partnership Against Cancer have advocated the need for clinicians with formalized AYA experience.

Comprehensive literature review on the radiographic findings, imaging modalities, and the role of radiology in the COVID-19 pandemic.

Since the outbreak of the coronavirus disease 2019 (COVID-19) pandemic, over 103214008 cases have been reported, with more than 2231158 deaths as of January 31, 2021. Although the gold standard for diagnosis of this disease remains the reverse-transcription polymerase chain reaction of nasopharyngeal and oropharyngeal swabs, its false-negative rates have ignited the use of medical imaging as an important adjunct or alternative. Medical imaging assists in identifying the pathogenesis, the degree of pulmonary damage, and the characteristic features in each imaging modality.

Clinical Prediction Models for Suspected Pediatric Foreign Body Aspiration: A Systematic Review and Meta-analysis.

Importance: Although various clinical prediction models (CPMs) have been described for diagnosing pediatric foreign body aspiration (FBA), to our knowledge, there is still no consensus regarding indications for bronchoscopy, the criterion standard for identifying airway foreign bodies.

Objective: To evaluate currently available CPMs for diagnosing FBA in children.

Data Sources: Performed in Ovid MEDLINE, Ovid Embase, PubMed, Web of Science, and CINAHL database with citation searching of retrieved studies.

Compassion in pediatric oncology: A patient, parent and healthcare provider empirical model.

Objective: Compassion has long been considered a cornerstone of quality pediatric healthcare by patients, parents, healthcare providers and systems leaders. However, little dedicated research on the nature, components and delivery of compassion in pediatric settings has been conducted. This study aimed to define and develop a patient, parent, and healthcare provider informed empirical model of compassion in pediatric oncology in order to begin to delineate the key qualities, skills and behaviors of compassion within pediatric healthcare.

Update on pediatric testicular germ cell tumors.

Background: Testicular germ cell tumors are uncommon tumors that are encountered by pediatric surgeons and urologists and require a knowledge of appropriate contemporary evaluation and surgical and medical management.

Method: A review of the recommended diagnostic evaluation and current surgical and medical management of children and adolescents with testicular germ cell tumors based upon recently completed clinical trials was performed and summarized in this article.

Results: In this summary of childhood and adolescent testicular germ cell tumors, we review the initial clinical evaluation, surgical and medical management, risk stratification, results from recent prospective cooperative group studies, and clinical outcomes.

Extracutaneous involvement is common and associated with prolonged disease activity and greater impact in juvenile localized scleroderma.

Objective: The aim of this study was to evaluate factors associated with extracutaneous involvement (ECI) in juvenile localized scleroderma (jLS).

Methods: A prospective, multicentre, 6-month observational study was performed. The data collected included disease features, global assessments, and subject symptoms.

Genetic structure and forensic characteristics of the Korean population revealed by GoldenEye 20A.

Background: In China, most Koreans live in the Northeast, including Jilin (59.64%), Heilongjiang (20.21%), and Liaoning (12.

Accelerating drug development for neuroblastoma: Summary of the Second Neuroblastoma Drug Development Strategy forum from Innovative Therapies for Children with Cancer and International Society of Paediatric Oncology Europe Neuroblastoma.

Only one class of targeted agents (anti-GD2 antibodies) has been incorporated into front-line therapy for neuroblastoma since the 1980s. The Neuroblastoma New Drug Development Strategy (NDDS) initiative commenced in 2012 to accelerate the development of new drugs for neuroblastoma. Advances have occurred, with eight of nine high-priority targets being evaluated in paediatric trials including anaplastic lymphoma kinase inhibitors being investigated in front-line, but significant challenges remain.

Detecting metabolic differences in fetal and adult sheep adipose and skeletal muscle tissues.

The primary metabolic pathway required to produce ATP differs as a result of tissue type, developmental stage and substrate availability. We utilized molecular and histological techniques to define the metabolic status in foetal and adult, adipose and skeletal muscle tissues. Redox ratios of these tissues were also determined optically by two-photon microscopy.

A phase 3 study of tezacaftor in combination with ivacaftor in children aged 6 through 11 years with cystic fibrosis.

Background: Tezacaftor/ivacaftor is a new treatment option in many regions for patients aged ≥12 years who are homozygous (F/F) or heterozygous for the F508del-CFTR mutation and a residual function (F/RF) mutation. This Phase 3, 2-part, open-label study evaluated the pharmacokinetics (PK), safety, tolerability, and efficacy of tezacaftor/ivacaftor in children aged 6 through 11 years with these mutations.

Methods: Part A informed weight-based tezacaftor/ivacaftor dosages for part B.

Resident training and interrater agreements using the ACNS critical care EEG terminology.

Purpose: Electroencephalography (EEG) remains the gold standard for identifying rhythmic and periodic patterns in critically ill patients. Residents have frequent exposures to EEG and critically ill patients during their training. Our study aimed to assess resident competency in the use of the American Clinical Neurophysiology Society (ACNS) critical care EEG terminology.

A multicentre investigation of organ and tissue donation education for critical care residents.

Purpose: To describe critical care medicine residents' training, expertise, and skills regarding organ and tissue donation processes and procedures.

Methods: We undertook a qualitative multicentre study and employed a purposive sample of program directors, physicians, nurses, residents, and organ donation leaders from all nine academic intensive care unit (ICU) training centres (five adult, four pediatric) in Ontario (n = 71). Interviews, conducted by telephone between December 2015 and March 2016, were audio-recorded and transcribed verbatim.