Strategies to Enable Transformation in Medical Education: Faculty and Trainee Development in Competence By Design.

Transformative changes in health professions education need to incorporate effective faculty development, but few very large-scale faculty development designs have been described. The Royal College of Physicians and Surgeons of Canada's Competence by Design project was launched to transform the delivery of postgraduate medical education in Canada using a competency-based model. In this paper we outline the goals, principles, and rationale of the Royal College's national strategy for faculty and resident development initiatives to support the implementation of Competence by Design. We describe the activities and resources for both faculty and trainees that facilitated the redesign of training programs for each specialty and subspecialty at the national level, as well as supporting the implementation of the redesign at the local level. This undertaking was not without its challenges: we thus reflect on those challenges, enablers, and the lessons learned, and discuss a continuous quality improvement approach that was taken to iteratively inform the implementation process moving forward.

Understanding the near-peer relationship: resident perspectives around a novel on-call workplace-based assessment.

Background: Workplace-based assessment (WBA) is a critical component of competency-based medical education (CBME), though literature on WBA for overnight call is limited. We evaluated a WBA tool completed by supervising subspecialty trainees on paediatric residents during subspecialty overnight call, for usefulness facilitating feedback/coaching in this setting. Methods: Web-based surveys were sent to residents pre- and post-WBA tool implementation monthly for four months (August-December 2018), exploring feedback frequency, Likert-scaled opinions of tool feasibility/usefulness facilitating feedback, and qualitative experiences. Assessor comments were categorized as actionable/non-actionable. Quantitative data was summarized using descriptive statistics. Qualitative data was coded to identify themes. Results: Total response rates averaged 41% (total 25 responses, average five respondents/12 residents on-call each month). Post-implementation (n = 16 responses), a non-sustained trend of increased Medical Expert feedback was observed. Residents were generally divided or disagreed on tool usefulness facilitating feedback and feasibility. Comments contained actionable feedback in < 10% of completed WBAs. Qualitative analysis revealed barriers to tool-facilitated coaching including: feedback quality and setting/environment, role of senior near-peer as assessor, interpersonal burden in encounters, and tool-specific issues. Conclusions: Increasing frequency of WBA tool completion is not sufficient to achieve CBME goals. Factors impacting feedback/coaching within the resident/near-peer dyad must be addressed.

Contexte: Tandis que l'évaluation en milieu de travail (EMT) est une composante essentielle de l'éducation médicale fondée sur les compétences (EMFC), il y a peu de recherches sur l'EMT en contexte de garde de nuit. Nous avons étudié un formulaire d'évaluation en milieu de travail rempli par des résidents en surspécialité supervisant des résidents en pédiatrie pendant la garde de nuit en surspécialité, afin de déterminer s'il facilite la rétroaction avec coaching dans ce contexte. Méthodes: Des questionnaires en ligne ont été envoyés aux résidents avant la mise en œuvre de l'outil d'EMT et à partir de celle-ci, tous les mois pendant quatre mois (d'août à décembre 2018). Ils exploraient la fréquence des rétroactions, les opinions des participants, exprimées sur une échelle de Likert, sur le caractère pratique et l'utilité de l'outil comme facilitateur de la rétroaction et leurs expériences qualitatives. Les commentaires des évaluateurs ont été catégorisés comme étant exploitables ou non exploitables. Les données quantitatives ont été résumées à l'aide de statistiques descriptives. Les données qualitatives ont été codées pour identifier les thèmes. Résultats: Le taux de réponse total était en moyenne de 41 % (total de 25 réponses, moyenne de 5 répondants/12 résidents de garde chaque mois). Après l'introduction de l'outil (n = 16 réponses), une tendance non soutenue à l'augmentation des commentaires des experts médicaux a été observée. Les résidents étaient généralement partagés ou en désaccord quant au caractère pratique de l'outil et à sa capacité à faciliter la rétroaction. Les commentaires contenaient des informations exploitables dans moins de 10 % des EMT remplies. L'analyse qualitative a révélé les obstacles suivants au fonctionnement de l'outil comme facilitateur du coaching : la qualité des commentaires et l'environnement, le rôle du presque pair senior en tant qu'évaluateur, la tension lors des rencontres de coaching et les problèmes spécifiques à l'outil. Conclusion: Pour atteindre les objectifs de l'EMFC, il ne suffit pas de remplir plus souvent l'outil d'EMT. Les facteurs qui influencent la rétroaction avec coaching au sein de la dyade résident-presque pair doivent également être pris en compte.

Assessing nephrology competence in general paediatrics-A survey of general paediatricians, paediatric nephrologists, residents, and program directors.

Objective: The objective of this study was to identify nephrology topics of lowest perceived competency and importance for general paediatricians. Methods: Surveys were distributed to general paediatricians, paediatric residents, paediatric residency program directors, and paediatric nephrologists. Perceived importance and competence were rated on a 5-point Likert scale. Means and 95% confidence intervals were calculated. Results: Mean perceived competency from general paediatricians across all nephrology domains was 3.0, 95%CI (2.9 to 3.1) and mean importance was 3.2, 95%CI (3.1 to 3.3). Domains scoring below the means for competence and importance, respectively were kidney stones (2.5, 95%CI [2.2 to 2.7]) and 2.6, 95%CI [2.3 to 2.8]), acute kidney injury (2.5, 95%CI [2.2 to 2.8] and 2.4, 95%CI [2.1 to 2.8]), chronic kidney disease (1.9, 95%CI [1.7 to 2.2] and 2.1, 95%CI [1.8 to 2.4]), tubular disorders (1.8, 95%CI [1.6 to 2.0] and 2.0, 95%CI [1.8 to 2.3]), and kidney transplant (1.6, 95%CI [1.4 to 1.8] and 1.7, 95%CI [1.4 to 1.9]). Residents, program directors, and paediatric nephrologists agreed that stones, chronic kidney disease, tubular disorders, and transplant were of lower importance. However, acute kidney injury was the domain with the largest discrepancy in perceived importance between residents (4.4, 95%CI [4.2 to 4.6]), nephrologists (4.2, 95%CI [3.8 to 4.6]), and program directors (4.2, 95%CI [3.7 to 4.7]) compared to general paediatricians ([2.4, 95%CI [2.1 to 2.8]; P<0.05). Conclusion: Paediatricians did not believe acute kidney injury was important to their practice, despite expert opinion and evidence of long-term consequences. Educational interventions must address deficits in crucial domains of renal health in paediatrics.

Progressive decline of T and B cell numbers and function in a patient with CDC42 deficiency.

Single allele mutations in the Cell Division Control protein 42 homolog (CDC42) gene were recently shown to cause Takenouchi-Kosaki syndrome with diverse manifestations. These include persistent mild thrombocytopenia with large platelet size, severe developmental delay, growth retardation, facial dysmorphism, and other neurodevelopmental and hematological anomalies. CDC42 deficiency might also cause myelofibrosis, myeloproliferation, and severe autoinflammation. CDC42 closely interacts with the Wiskott-Aldrich Syndrome Protein, but little is still known about the immune abnormalities associated with CDC42 deficiency. Detailed immune evaluations were performed in a patient diagnosed with a CDC42 Tyr64Cys mutation. The 19-year-old female suffered from recurrent pneumonia, otitis media, and bacteremia, which resolved at 10 years of age, concordant with the initiation of amoxicillin prophylaxis. In addition, the patient had frequent viral upper respiratory tract infections, which resolved without need for medical interventions. Immune evaluations demonstrated decreased immunoglobulin levels, inability to maintain antibody responses, progressive decline in the number of CD19+ B cells, and decreased switched memory B cells. There was also a decrease in CD4+ and CD8+ T cells, markedly reduced naïve T cells, and intermittent depressed proliferation of T cells to stimulation. Natural killer cells' number and functions were normal. However, no opportunistic infections were observed, nor was there evidence for autoinflammation. CDC42 deficiency might also be associated with decline in T and B cell function. Therefore, immunity in patients with CDC42 defects should be closely monitored, particularly among those with frequent infections or systemic autoinflammation.

Basophil activation test shows high accuracy in the diagnosis of peanut and tree nut allergy: The Markers of Nut Allergy Study.

BACKGROUND: Peanut and tree nut allergies are the most important causes of anaphylaxis. Co-reactivity to more than one nut is frequent, and co-sensitization in the absence of clinical data is often obtained. Confirmatory oral food challenges (OFCs) are inconsistently performed. OBJECTIVE: To investigate the utility of the basophil activation test (BAT) in diagnosing peanut and tree nut allergies. METHODS: The Markers Of Nut Allergy Study (MONAS) prospectively enrolled patients aged 0.5-17 years with confirmed peanut and/or tree nut (almond, cashew, hazelnut, pistachio, walnut) allergy or sensitization from Canadian (n = 150) and Austrian (n = 50) tertiary pediatric centers. BAT using %CD63+ basophils (SSClow/CCR3pos) as outcome was performed with whole blood samples stimulated with allergen extracts of each nut (0.001-1000 ng/mL protein). BAT results were assessed against confirmed allergic status in a blinded fashion to develop a generalizable statistical model for comparison to extract and marker allergen-specific IgE. RESULTS: A mixed effect model integrating BAT results for 10 and 100 ng/mL of peanut and individual tree nut extracts was optimal. The area under the ROC curve (AUROC) was 0.98 for peanut, 0.97 for cashew, 0.92 for hazelnut, 0.95 for pistachio, and 0.97 for walnut. The BAT outperformed sIgE testing for peanut or hazelnut and was comparable for walnut (AUROC 0.95, 0.94, 0.92) in a sub-analysis in sensitized patients undergoing OFC. CONCLUSIONS: Basophil activation test can predict allergic clinical status to peanut and tree nuts in multi-nut-sensitized children and may reduce the need for high-risk OFCs in patients.

Long-Term Outcome of Adenosine Deaminase-Deficient Patients-a Single-Center Experience.

PURPOSE: Inherited defects in the adenosine deaminase (ADA) enzyme can cause severe combined immune deficiency (SCID) and systemic abnormalities. Management options for ADA-deficient patients include enzyme replacement therapy (ERT), hematopoietic stem cell transplantation (HSCT), and gene therapy (GT). Here, we describe the long-term benefits of these treatments. METHODS: Survival, infections, systemic sequelae, and laboratory assessments were recorded for all ADA-deficient SCID patients, managed at a single center since 1985, who survived 5 or more years following treatment. RESULTS: Of 20 ADA-deficient patients, the 8 (40%) who survived 5 or more years (range 6-29.5 years, median 14 years) were included in the study. Among the long-term survivors, two patients were treated exclusively with ERT, five underwent HSCT (three from HLA-matched sibling donors, two from HLA-mismatched related donors), and one received GT. The long-term survivors often suffered from recurrent respiratory infections; however, opportunistic infections occurred in only one patient. Systemic sequelae included lung disease such as bronchiectasis and asthma (four patients), neurologic abnormalities (six patients), metabolic disturbances (two patients), allergy and autoimmunity (six patients), and neoplasms (three patients). Normal CD4+ T cell numbers and function, as well as antibody production, were usually observed after HSCT and GT, but not after ERT. Late deaths occurred in two patients at 15 and 25 years after HSCT, respectively, and were attributed to respiratory failure. CONCLUSIONS: ADA-deficient patients commonly suffer from long-term complications, emphasizing the need for improved management and for multi-disciplinary follow-up.

Antibiotic skin testing accompanied by provocative challenges in children is a useful clinical tool.

BACKGROUND: Diagnostic testing to antibiotics other than to penicillin has not been widely available, making the diagnosis of antibiotic allergy difficult and often erroneous. There is often reluctance in performing challenges to antibiotics when standardized testing is lacking. However, while the immunogenic determinants are not known for most antibiotics, a skin reaction at a non-irritating concentration (NIC) may mean that antibodies to the native form are present in the circulation. While the NIC's for many non penicillin antibiotics have been determined in adults, the use of these concentrations for skin testing pediatric subjects prior to provocative challenge has not been done. Our objective was to determine if we could successfully uncover the true nature of antibiotic allergy in children using these concentrations for testing. METHODS: Children were included between 2003-2009 upon being referred to the Drug and Adverse Reaction/Toxicology (DART) clinic of the Hospital for Sick Children in Toronto, Ontario Canada. The referral needed to demonstrate that clinical care was being compromised by the limitation in antibiotic options or there was a significant medical condition for which the label of antibiotic allergy may prove detrimental. Patients were not seen if there was a suggestion of serum like sickness, Stevens Johnson Syndrome or Toxic Epidermal Necrolysis. Patients were excluded from testing if there was objective evidence of anaphylaxis. All other patients were consented to receive testing and/or challenges. A retrospective chart review was then performed of the results. RESULTS: We were able to exclude an antibiotic allergy in the majority of our patients who had a negative intradermal test result and were then challenged (>90%). Only one patient was challenged with a positive intradermal test to Cotrimoxazole because of a questionable history and this patient failed the provocative challenge. While we did not challenge more patients with positive testing, we did note that 10/11 (91%) patients with positive intradermal testing had some aspect of a Type 1 reaction in their history. CONCLUSIONS: Through testing with NIC's of various antibiotics in children and providing provocative challenges based on negative skin testing results, we were able to advance the medical care of the majority of our patients by increasing their antibiotic options in order to successfully treat future infections. While challenging patients with positive testing was not deemed ethically appropriate at this stage of our study, it would be a useful future step to reaching statistical validity of testing to these antibiotics.

Safety of intravenous immunoglobulin in the treatment of juvenile dermatomyositis: adverse reactions are associated with immunoglobulin A content.

OBJECTIVE: Anecdotal reports have suggested differences in children's tolerance to different intravenous immunoglobulin products; however, there has been little research on this issue. We sought to determine whether different intravenous immunoglobulin products used in the treatment of juvenile dermatomyositis are equally well tolerated by patients and, if not, whether differences in tolerance are linked to immunoglobulin A content. PATIENTS AND METHODS: The intravenous immunoglobulin infusion history (product given and history of adverse events) of patients who were attending the juvenile dermatomyositis clinic at the Hospital for Sick Children from 1986 to 2005 was reviewed. Products with an immunoglobulin A content of >15 microg/mL were classified as "high immunoglobulin A." Data were analyzed by using logistic regression models adjusted for repeated measures. RESULTS: Thirty-eight patients with juvenile dermatomyositis received 1056 infusions at the Hospital for Sick Children. Adverse events were reported on 92 occasions (9%), affecting 25 patients (66%), a frequency that is higher than that usually reported in adult patients (<1%-5%). Adverse events were reported more often with products that contained high immunoglobulin A (15.0% vs 8.0%). These were accounted for specifically by fever (8.0% vs 1.0%), lethargy or malaise (2.0% vs 0.1%), and nausea or vomiting (5.0% vs 1.0%). Of the possible pharmacologic predictors, including dose, immunoglobulin G concentration, immunoglobulin A level, pH, glycine content, sugar content, sodium content, and osmolality, only immunoglobulin A level was significantly associated with adverse events. CONCLUSIONS: Intravenous immunoglobulin was found to be safe and well tolerated by most children with juvenile dermatomyositis. However, in contrast to adult studies, we found that significant differences existed in tolerance to different intravenous immunoglobulin products, most likely because of immunoglobulin A concentration. This study confirms anecdotal reports that a high level of immunoglobulin A in intravenous immunoglobulin is less well tolerated by children and provides evidence that product choice is important in pediatrics.

Low serum immunoglobulin G2 levels in infancy can be transient.

OBJECTIVE: The immunoglobulin G2 subclasses contain predominantly antipolysaccharide antibodies. It was therefore believed intuitively that low immunoglobulin G2 levels could predispose individuals to infections with encapsulated bacteria. Although many reports initially supported this notion, more recent studies challenged it. Regardless of the biological significance, the natural history of low immunoglobulin G2 levels has not been carefully studied. METHODS: We studied the outcome of low serum immunoglobulin G2 subclass levels in children. Thirteen patients who were referred because of recurrent infections were found to have low immunoglobulin G2 levels. Laboratory evaluation at presentation and follow-up visits included total serum immunoglobulins, immunoglobulin subclasses, and specific antibodies to protein antigens and to pneumococcal vaccine. RESULTS: Low immunoglobulin G2 levels resolved completely within 0.6 years to 6 years (median: 1.5 years) in all patients. All 13 patients responded adequately to vaccination with protein antigens such as tetanus toxoid and polio as well as to immunization with pneumococcal vaccine. Four of 13 patients had a previous history of transient hypogammaglobulinemia, raising the possibility that the other cases may simply represent the tail end of this condition. CONCLUSION: We have demonstrated that low immunoglobulin G2 detected in early infancy and childhood is likely to resolve completely within several months and up to 6 years from the time of presentation.