Background: Childhood obesity can result in adverse health outcomes. The objectives of this study were to describe the prevalence of obesity and determine the association between obesity at cancer diagnosis and event-free survival (EFS) and overall survival (OS) in children diagnosed with cancer in Canada.
Methods: The authors conducted a retrospective cohort study using the Cancer in Young People in Canada database, including all children with newly diagnosed cancer aged 2-18 years across Canada from 2001 to 2020.
Background: Little is known about the influence of interprofessional education (IPE) on interprofessional collaboration (IPC), particularly following the COVID 19 pandemic.
Aim: To examine nursing students' perceived ability to practice IPC after exposure to virtual lectures and immersive clinical IPE activities founded on the competencies from the National Interprofessional Competency Framework.
Design: A cross-sectional design was used.
Sickle cell disease (SCD) is a group of inherited blood disorders caused by a mutation in the beta subunit of hemoglobin (HbS). SCD will hereafter be referred to as sickle cell anemia (SCA) as this is the term our patients and their families prefer. There are approximately 5000 Canadians living with SCA including children.
View Article and Find Full Text PDFClassification of inherited bone marrow failure syndromes (IBMFSs) according to clinical and genetic diagnoses enables proper adjustment of treatment. Unfortunately, 30% of patients enrolled in the Canadian Inherited Marrow Failure Registry (CIMFR) with features suggesting hereditability could not be classified with a specific syndromic diagnosis. We analyzed the outcome of hematopoietic stem cell transplantation (HSCT) in unclassified IBMFSs (uIBMFSs) and the factors associated with outcome.
View Article and Find Full Text PDFBackground: This study examined the structural outcomes for joints of boys with severe hemophilia A receiving frequency/dose-escalated primary prophylaxis using magnetic resonance imaging (MRI), and the importance of interval MRI changes.
Methods: Forty-six subjects (27 with interval studies) were evaluated by radiographs (X-rays) and mid- and end-of-study MRIs (using the International Prophylaxis Study Group scale), as part of the Canadian Hemophilia Prophylaxis Study. The primary outcome was the presence of MRI osteochondral findings.
Objective: The primary objective of this study was to assess whether there are different patterns (classes) of joint health in young boys with severe haemophilia A (SHA) prescribed primary tailored prophylaxis. We also assessed whether age at first index joint bleed, blood group, FVIII gene abnormality variant, factor VIII trough level, first-year bleeding rate and adherence to the prescribed prophylaxis regimen significantly predicted joint damage trajectory, and thus class membership.
Methods: Using data collected prospectively as part of the Canadian Hemophilia Primary Prophylaxis Study (CHPS), we implemented a latent class growth mixture model technique to determine how many joint damage classes existed within the cohort.
Background: Globally, many infants and children are diagnosed with illnesses that impose limitations on their well-being and life course trajectory. Children's care becomes the central focus of family life. Inadequate support for parents is detrimental to their well-being and management of their child's care and support needs.
View Article and Find Full Text PDFPrimary CNS tumors are the leading cause of cancer-related death in pediatrics. It is essential to understand treatment trends to interpret national survival data. In Canada, children with CNS tumors are treated at one of 16 tertiary care centers.
View Article and Find Full Text PDFThroughout their careers, doctors and other healthcare professionals experience numerous transitions. When supporting transitions, opportunities for development and learning should be maximized, while stressors having negative impacts on well-being should be minimized. Building on our international data, this study aimed to develop a conceptual model of the trainee-trained transition (i.
View Article and Find Full Text PDFProgressive cytopenia is a serious complication among paediatric patients with inherited bone marrow failure syndromes (IBMFS). Androgens have been used to improve blood counts in different bone marrow failure conditions. Little is known about efficacy and toxicity with new androgens (i.
View Article and Find Full Text PDFBackground: Standard of care for persons with severe hemophilia A includes regular replacement of factor VIII (FVIII). Prophylaxis regimens using standard half-life (SHL) FVIII concentrates, while effective, are costly and require frequent intravenous infusions.
Aim: This study evaluated the adherence of 56 boys with severe hemophilia A to tailored, frequency-escalated prophylaxis with an SHL recombinant FVIII concentrate.
Inherited bone marrow failure syndromes (IBMFSs) are genetically heterogeneous disorders with cytopenia. Many IBMFSs also feature physical malformations and an increased risk of cancer. Point mutations can be identified in about half of patients.
View Article and Find Full Text PDFIKBKB immune deficiency is a rare but life-threatening primary immunodeficiency disorder, involving activation defects in adaptive and innate immunity. We present sixteen cases of a homozygous IKBKB mutation (c.1292dupG) in infants characterized by early-onset bacterial, viral, fungal and Mycobacterial infections.
View Article and Find Full Text PDFInherited bone marrow failure syndromes (IBMFSs) comprise a genetically heterogeneous group of diseases with hematopoietic failure and a wide array of physical malformations. Copy number variants (CNVs) were reported in some IBMFSs. It is unclear what impact CNVs play in patients evaluated for a suspected diagnosis of IBMFS.
View Article and Find Full Text PDFBackground: In 2016, the Food and Drug Administration (FDA) proposed to enhance performance expectations for point-of-care testing (POCT) international normalized ratio (INR) devices relative to International Organization for Standardization (ISO) 17593:2007. The objective of the study was to estimate the frequency of warfarin dosing errors associated with a central laboratory INR method, a POCT INR method, and the proposed FDA performance goals.
Methods: A data set of INR results (n = 51912) from adult patients with INR ≤4 was used to assess the influence of adding assay imprecision and bias on warfarin dose decisions.
Background: Phenotypic overlap among the inherited bone marrow failure syndromes (IBMFSs) frequently limits the ability to establish a diagnosis based solely on clinical features. >70 IBMFS genes have been identified, which often renders genetic testing prolonged and costly. Since correct diagnosis, treatment and cancer surveillance often depend on identifying the mutated gene, strategies that enable timely genotyping are essential.
View Article and Find Full Text PDFInherited bone marrow failure syndromes are a group of rare, heterogeneous genetic disorders with a risk of clonal and malignant myeloid transformation including clonal marrow cytogenetic abnormalities, myelodysplastic syndrome and acute myeloid leukemia. The clinical characteristics, risk classification, prognostic factors and outcome of clonal and malignant myeloid transformation associated with inherited bone marrow failure syndromes are largely unknown. The aims of this study were to determine the impact of category, cytopathology and cytogenetics, the three components of the "Category Cytology Cytogenetics" classification of pediatric myelodysplastic syndrome, on the outcome of clonal and malignant myeloid transformation associated with inherited bone marrow failure.
View Article and Find Full Text PDFBackground: Adolescents have their own views about the cancer care they receive and how they feel they are treated, but their opinions are rarely solicited.
Objective: To determine whether the 56-item Give Youth a Voice (GYV-56), its subscales and its 20-item short-form, are clinically meaningful and psychometrically sound instruments that can be used to measure teen-centred care (TCC) in paediatric oncology.
Design: Qualitative interviews and a questionnaire survey.
Background: Few Canadian studies have examined stress in residency and none have included a large sample of resident physicians. Previous studies have also not examined well-being resources nor found significant concerns with perceived stress levels in residency. The goal of "The Happy Docs Study" was to increase knowledge of current stressors affecting the health of residents and to gather information regarding the well-being resources available to them.
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