Browsing by Author "Bjornson, Candice"

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    Delayed diagnosis of high proximal tracheoesophageal fistula in esophageal atresia and a novel approach to the treatment of tracheomalacia by submanubrial tracheopexy
    (Springer Plus, 2014-02-27) Bjornson, Candice; Brindle, Mary E.; Bailey, Michelle J.A.; Mitchell, Ian; Soles, Melissa
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    Immunoreactive trypsinogen levels in newborn screened infants with an inconclusive diagnosis of cystic fibrosis
    (2019-10-22) Ooi, Chee Y; Sutherland, Rosie; Castellani, Carlo; Keenan, Katherine; Boland, Margaret; Reisman, Joe; Bjornson, Candice; Chilvers, Mark A; van Wylick, Richard; Kent, Steven; Price, April; Mateos-Corral, Dimas; Hughes, Daniel; Solomon, Melinda; Zuberbuhler, Peter; Brusky, Janna; Durie, Peter R; Ratjen, Felix; Gonska, Tanja
    Abstract Background Newborn screening (NBS) for cystic fibrosis (CF) not only identifies infants with a diagnosis of CF, but also those with an uncertain diagnosis of cystic fibrosis (CF), i.e. CF transmembrane conductance regulator (CFTR)-related metabolic syndrome (CRMS) or CF screen positive inconclusive diagnosis (CFSPID). These infants have an uncertain long-term outcome and it is currently unclear around time of diagnosis, which infants are at higher risk of later fulfilling a CF diagnosis. In this study, we hypothesised that immunoreactive trypsinogen (IRT) levels, used in NBS as a marker of pancreatic disease and function, may reflect the degree of CFTR dysfunction in each individual and therefore would help to identify those with CRMS/CSPID who are later at risk for meeting the criteria of CF. Methods In this longitudinal, prospective study, infants with CRMS/CFSPID and CF were recruited and followed in 9 CF clinics (Canada and Italy). We compared NBS IRT levels between CF and CRMS/CFSPID, and between children with CRMS/CFSPID→CF and CRMS/CFSPID→CRMS/CFSPID during the period of June 2007 to April 2016. Results Ninety eight CRMS/CFSPID and 120 CF subjects were enrolled. During the study period, 14 (14.3%) CRMS/CFSPID subjects fulfilled the diagnostic criteria for CF (CRMS/CFSPID→CF), while the diagnosis remained uncertain (CRMS/CFSPID→ CRMS/CFSPID) in 84 (85.7%) subjects. Significantly higher NBS IRT concentrations (ng/ml) were present in CF than CRMS/CFPSID (median (interquartile range): 143.8 (99.8–206.2) vs. 75.0 (61.0–105.9); P < 0.0001). Infants with CRMS/CFSPID→CF (n = 14) had significantly higher NBS IRT concentrations (ng/ml) than CRMS/CFSPID→ CRMS/CFSPID (n = 83) (median (interquartile range): 108.9 (72.3–126.8) vs. 73.7(60.0–96.0); P = 0.02). Conclusions Amongst infants who tested positive on NBS for CF, there is a gradation of elevated NBS IRT concentrations. Infants with CF have higher NBS IRT levels than CRMS/CFPSID, and higher NBS IRT concentrations were present in infants with CRMS/CFSPID→CF than CRMS/CFSPID→ CRMS/CFSPID. NBS IRT concentrations, in concert with other factors, may have the potential to predict the likelihood of CF amongst infants with CRMS/CFSPID.
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    The Cost of Cystic Fibrosis Care in Canada: A Scoping Review
    (2024) Desai, Leena; Bomersback, Taryn; Galante, Gary; Bjornson, Candice; Hirota, Simon
    Background: Cystic fibrosis (CF) contributes a significant economic burden on individuals, health systems and society. This economic impact is highly relevant in a single-payer, publicly funded health system such as Canada. CFTR modulator therapy has been shown in large clinical trials to reduce the burden of disease in the pediatric population. Currently 17% of patients at the Alberta Children’s Hospital in Calgary, AB do not qualify for any CFTR modulator therapy, presumably resulting in an incremental cost to the system. Precision medicine tools such as organoid models may offer an opportunity for n-of-1 trials for individual patients who are currently not approved for therapy. Rationale: In order to better understand the health and economic impact of cystic fibrosis in Canada, we wish to identify direct, indirect and intangible costs of cystic fibrosis care in Alberta and Canada, and highlight the higher rate of heterogeneity amongst our southern Alberta CF population which predisposes to higher cost of care due to lack of approval to currently available therapies. Aim and Objective: This protocol describes the purpose of a scoping review which is to identify the most accurate estimation of the cost of cystic fibrosis care in Canada, and gather comprehensive data on how the costs of CF care (including direct, indirect and intangible costs) are defined and measured in Canada. Methodology: The protocol is developed using the JBI (Joanna Briggs Institute) guidance for scoping reviews and based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) Checklist, both of which will also guide its reporting. We will search PUBMED and OVID/MEDLINE for peer-reviewed English-language publications and grey literature search of relevant databases (ProQuest Dissertation and Theses, Canadian Health Research Collection) and organizational websites (Health Canada, Government of Canada, Cystic Fibrosis Foundation of Canda, Canadian Agency for Drugs and Technology in Health (CADTH)).