Overcoming the barriers to implementation of competence-based medical education in post-graduate medical education: a narrative literature review.

To ensure that residents are equipped with the necessary skills for practice, competence-based medical education (CBME) represents a transformative change in postgraduate medical education, which is being progressively introduced across Canadian specialty residency programs. Successful implementation will require adjustments to curriculum, assessment, and evaluation, with careful attention to the unique needs in the local context, including resident and faculty development. This narrative review of the literature aimed to determine the potential barriers to the successful implementation of CBME and the strategies by which they can be addressed, with a specific consideration of the author's program in pediatrics in Manitoba. Eleven articles were identified with a specific focus on the implementation of CBME in the post-graduate setting, and 10 were included in the review after critical appraisal. Three key themes emerged from the articles: the value of broad stakeholder engagement and leadership, the importance of faculty and resident development, and the development of specific support systems for the educational curriculum. Different strategies were considered and contrasted for addressing these important themes. This review provides important insights and practical approaches to the barriers that should be useful as programs prepare for the implementation of CBME.

Preferences and Health-Related Quality-of-Life Related to Disease and Treatment Features for Patients with Hemophilia A in a Canadian General Population Sample.

BACKGROUND: Current treatments for hemophilia A in Canada include on-demand treatment as bleeds occur and regular intravenous prophylactic factor VIII (FVIII) infusions. The subcutaneous therapy emicizumab was recently approved for treatment of hemophilia A. The objective of this study was to estimate utility values associated with hemophilia A health and treatment states from a Canadian societal perspective, including preferences related to treatment efficacy and frequency and route of administration. METHODS: A vignette-based time trade-off (TTO) utilities elicitation was undertaken in Canadian adults to compare preferences for six hemophilia health states describing prophylactic and on-demand treatment, with varying bleed rates and frequency of treatment administration. A convenience sample was recruited via market research panels and snowball sampling. Health state/vignette definitions were informed by clinical experience, clinical trial results regarding bleed frequency, and validated by qualitative interviews of hemophilia patients and caregivers (n=10). Utilities were estimated via an online, trained interviewer-guided, vignette-based TTO exercise, where respondents valuated health states describing hemophilia patients (adults or children) receiving subcutaneous prophylaxis, intravenous prophylaxis, and on-demand treatments. Analyses included a descriptive analysis by health state; a mixed-effects analysis of utility values adjusted for subcutaneous vs infusion-based therapies and number of bleeds; and for prophylactic regimens, an analysis of utilities by frequency of infusions or injections. RESULTS: TTO interviews were conducted with 82 respondents. Mean utilities [95% CI] were highest for subcutaneous prophylaxis (0.90 [0.87-0.93]), followed by intravenous prophylaxis (0.81 [0.78-0.85]), and on-demand treatment (0.70 [0.65-0.76]). In regression analysis, subcutaneous treatment health states were associated with a utility increment of +0.1112. Additional bleeds and more frequent infusions were associated with lower utility values (-0.0027 per bleed and -0.0003 per infusion). CONCLUSION: Subcutaneous prophylaxis is associated with higher utility values compared to intravenous prophylactic and on-demand treatment, while increased bleeds and infusions are associated with reduced utility.

Syrian Refugees and Their Impact on Health Service Delivery in the Pediatric Hematology/Oncology Clinics Across Canada.

This study examined the impact of Syrian refugees on 1 area of the Canadian health care sector. We predicted that pediatric hematology clinics across Canada would see a spike in their Syrian refugee patient population in proportion to their recent migration and, as a result, an increase in perceived workload. Data on the number of refugee patients, types of diseases, and perceived workload were gathered from hematology clinics across Canada using a clinical survey (Supplemental Digital Content 1, http://links.lww.com/JPHO/A315). The results showed that Ontario had the most Syrian refugee patients, followed by the Quebec, Western Canadian, and Atlantic regions. The results also showed that perceived workload ranged from "no increase" (4 programs) to "minimal increase" <25% (1 program), "moderate increase" 25% to 75% (4 programs), and "significant increase" >75% (3 programs, 2 of which had no transfusion-dependent thalassemia patients before the immigration).

From the voices of people with haemophilia A and their caregivers: Challenges with current treatment, their impact on quality of life and desired improvements in future therapies.

INTRODUCTION: Haemophilia A is a chronic disease requiring frequent intravenous infusions of recombinant factor VIII. Previous studies have shown that challenges associated with current treatments may have significant impacts on quality of life (QoL) that are as important as the health outcomes conferred by the therapy. Emerging therapeutic innovations offer the potential to mitigate treatment-related challenges, and it is therefore important to develop a better understanding of patient and caregiver experiences with existing haemophilia A treatments in order to characterize the full value of new treatments. AIM: To gather firsthand perspectives from people with haemophilia A (PWHA) and caregivers on the challenges with current treatment, their impact on QoL and desired improvements in future therapies. METHODS: Qualitative insights were gathered from 20 non-inhibitor PWHA or caregivers of PWHA across Canada through one-on-one interviews; insights were further explored through focus group sessions to uncover overarching themes and prioritize issues with current treatments. RESULTS: PWHA and caregivers identified several challenges, including administration of intravenous infusions, coordination of treatment schedules and ensuring adequate medication and supplies. Participants described how these challenges impact psychosocial well-being, physical health, personal/social life and work. Alternate modes of administration and longer-lasting treatment effects were identified as desired improvements over current treatments. CONCLUSION: This study emphasizes the impact that existing haemophilia A treatments have on psychological well-being, employment opportunities and adherence to treatment regimens. These considerations may help to inform decision-making for policymakers and health systems around the true value of new therapies entering the haemophilia market.

Two Cases of Hypercalcemia in Pediatric Ovarian Dysgerminoma.

OBJECTIVE: To discuss the finding of hypercalcemia in pediatric ovarian dysgerminoma. METHODS: Two cases of pediatric ovarian dysgerminoma that presented with hypercalcemia are discussed. RESULTS: Hypercalcemia is a rare finding in ovarian dysgerminoma. CONCLUSION: Ovarian dysgerminoma should be considered in pediatric patients presenting with signs of hypercalcemia. Parathyroid hormone, parathyroid hormone related protein, and 1,25 dihydroxyvitamin D may elucidate the cause of hypercalcemia.

The unusual pattern of hereditary bleeding disorders in the province of Newfoundland and Labrador-Canada's most Eastern Province.

Newfoundland and Labrador (NL), the most eastern province of Canada, is characterized by a unique topography and pattern of settlement. The current population is descended from a small founding population of indigenous Innu, Inuit and Mi'kmaq and an estimated 28,000 settlers. These settlers originated from Southwest England and Southeast Ireland and came to invest and work in one of the world's richest fisheries. They settled in bays, coves and islands off the coast, in small settlements called out-ports. These isolated communities developed unusual patterns of genetic disease including an unusual prevalence of some extremely rare Hereditary Bleeding Disorders (HBD). This study was designed to document the prevalence of these rare disorders, at a snapshot in time, using our provincial HBD registry. These diagnoses were verified by reviewing the original initial diagnostic coagulation results to confirm or refute each diagnosis. When available, we also recorded the underlying mutation. Population based prevalence rates were then compared with data published from the World Federation of Hemophilia (WFH) Global Registry. The results are striking. Using the WFH data the per capita prevalence in NL of Hemophilia A, Factors V, XI, and XIII Deficiency are higher than that of mainland Canada minus Labrador by a factor of 2.89, 4.54, 5.44 and 9.22, respectively. The increased prevalence of mild Hemophilia A is explained by a founder effect of the Val 2016 Ala mutation. All the severe FXIII deficient patients are homozygotes for c.691-1 G > A mutation. These results show that NL's unique geography and population distribution led to a genetic drift that increased the prevalence of some rare factor deficiencies. This comparatively high prevalence provides a potential pool of patients for genotype/phenotype research.

Prevalence of Peripheral Eosinophilia at Diagnosis in Children With Inflammatory Bowel Disease.

BACKGROUND AND OBJECTIVE: Inflammatory bowel disease (IBD) encompasses 2 disorders of unknown etiology: Crohn disease (CD) and ulcerative colitis (UC). There has been a continuous search for markers for disease activity. Eosinophils are granulocytic leukocytes that are implicated in the pathogenesis of IBD. The aim of this study was to examine the prevalence and significance of peripheral eosinophilia (PE) at diagnosis in children with IBD. METHODS: A comprehensive chart review of all children with diagnosed as having IBD between January 2006 and August 2014 was performed. Patients with PE at diagnosis were compared with those without in relation to disease clinical activity and disease course. RESULTS: A total of 109 children (mean age 14.6 ± 2.77, range 4.5-17.9 years, 55 boys) with IBD (68 with CD and 41 with UC) who were studied for a mean duration of 2.82 ± 1.89 (range 0.1-9.2 years) were identified. At diagnosis, 44 (40.4%) children had PE, which was more prevalent in patients with UC compared with those with CD (61.3% vs 36.3%, P < 0.05). At diagnosis, PE was more common in patients with high eosinophilic count in colonic biopsy samples (P < 0.01) and was significantly associated with disease activity as indicated by Pediatric CD Activity Index for children with CD (P < 0.05), Pediatric UC Activity Index for children with UC (P < 0.01). CONCLUSIONS: PE is a common finding at diagnosis in children with IBD especially in those with UC. Patients with PE at diagnosis are more likely to present with higher clinical activity indices. PE is associated with more eosinophils in colonic biopsy samples.

Neonatal renal vein thrombosis: review of the English-language literature between 1992 and 2006.

Renal vein thrombosis is a complication that occurs in neonates with various underlying risk factors. It carries a grave prognosis for affected kidneys. Anticoagulant and fibrinolytic therapies have been promoted in the past with anecdotal success in some circumstances. However, prospective controlled trials are still lacking, and to date there have been no evidence-based guidelines available for the treatment of neonates with renal vein thrombosis. We retrospectively reviewed all the available medical literature pertaining to renal vein thrombosis published in English during the past 15 years. A total of 271 patients from 13 case series were identified by using the terms "renal vein thrombosis" and "neonates" via PubMed and Cochrane Library searches. Data then were extracted from each of the studies for analysis. During the past 15 years, a male predominance (67.2%) in neonatal renal vein thrombosis has been reported. More than 70% of patients had unilateral renal vein thrombosis, which was more prevalent on the left side (63.6%). The thrombus involved the inferior vena cava and was associated with adrenal hemorrhage in 43.7% and 14.8% of neonates, respectively. Forty percent of the patients were treated conservatively with supportive care alone. Among those patients who received anticoagulation therapy, unfractionated heparin and low molecular weight heparin were used alone in 21.6% and 20.7% of the patients, respectively. Fibrinolytic treatment alone was used in 11.2% of the patients. Only a minority of patients were treated with antithrombin (1.7%), warfarin alone, (0.9%) or underwent surgical intervention (0.3%). The majority (70.6%) of the involved kidneys became atrophic. A total of 9 neonates died with non-renal vein thrombosis-related conditions during the study period. Evidence-based recommendations on treatment cannot be made at the present time. Cooperative prospective studies that involve multiple centers are needed to elucidate the optimal treatment for neonatal renal vein thrombosis.

Risk factors for early-onset group B streptococcal disease in neonates: a population-based case-control study.

BACKGROUND: Infection with group B streptococcus (GBS) is a major cause of neonatal illness and death. We examined the antenatal and perinatal risk factors for early-onset GBS disease among neonates. METHODS: We identified cases by population-based surveillance in all microbiology laboratories serving Alberta. A case was defined as any instance of a positive sterile-site GBS culture in an infant born between 1993 and 1997 who was either less than 7 days old or stillborn after 20 weeks' gestation. We randomly selected controls from a computer-compiled list of all hospital births, including stillbirths after 20 weeks' gestation, in Alberta during the study period. To increase power, we chose 5 or 6 control infants born in the same year as each case infant. We reviewed hospital, prenatal clinic and physician health records and, between 1997 and 1999, conducted maternal interviews by telephone. RESULTS: There were no differences between the 90 cases and 489 controls in sociodemographic variables or in many reproductive and behavioural variables. Case infants were more likely than control infants to be of low birth weight (odds ratio [OR] 3.60, 95% confidence interval [CI] 1.68-7.65), to have been delivered preterm (OR 3.89, 95% CI 2.08-7.27), or to have a mother with amnionitis (OR 15.03, 95% CI 5.58-41.89), intrapartum fever (OR 4.65, 95% CI 2.48-8.69) or premature rupture of the membranes (OR 2.39, 95% CI 1.38-4.14). After adjustment for potential confounders, intrauterine fetal monitoring was associated with a more than 2-fold increase in the risk of neonatal GBS disease (OR 2.24, 95% CI 1.22-4.13). INTERPRETATION: Intrauterine fetal monitoring should be added to the list of risk factors in risk-based screening. Since many of the cases had no identifiable maternal risk factors, universal screening for GBS may be appropriate.