Abstract
Background
The implementation of competency-based medical education and utilization of competence committees (CC) represents a paradigm shift in residency education. This qualitative study ...aimed to explore the methods used by two operational CC and their members to make decisions about progression and competence of their residents.
Methods
An instrumental case study methodology was used to study the CC of two postgraduate training programs. Transcripts from observed CC meetings, member interviews, and guiding documents were analyzed using a constructivist grounded theory approach to reveal themes explaining the decision-making process.
Results
Our study found that the CC followed a process that began within a social decision schema model and evolved to a discussion that invoked social influence theory, shared mental models, and social judgment scheme to clarify the points of contention. We identified that the CC decision-making was at risk of bias, primarily influenced by the group composition, the group orientation and individual members’ mindset, as well as their personal experiences with the trainees.
Conclusions
Increased awareness of the sources of bias in CC functioning and familiarity with the CC role in competency-based medical education would enable committees to provide valuable feedback to all trainees regardless of their trajectory.
Beta-propeller protein-associated neurodegeneration (BPAN) is a subtype of neurodegeneration with brain iron accumulation (NBIA) that presents with childhood developmental delay (especially speech ...delay), occasionally associated with epileptic encephalopathy, autism, or Rett-like syndrome. The majority of children described to date have been severely affected, with little to no expressive speech function, severe developmental delay, and cognitive impairment. Herein, five additional patients with BPAN identified in the same center in Canada are described, four with the typical severe phenotype and one with a milder phenotype. Our findings provide further evidence that a spectrum of severity exists for this rare and newly described condition. Challenges in identifying iron accumulation on brain MRI are also addressed. Additionally, the importance of including the WDR45 gene on epilepsy and Rett-like syndrome genetic panels is highlighted.
Perinatal stroke causes cerebral palsy and lifelong disability. Specific diseases are definable, but mechanisms are poorly understood. Evidence suggests possible associations between arterial ...perinatal stroke and prothrombotic disorders, but population-based, controlled, disease-specific studies are limited. Understanding thrombophilia in perinatal stroke informs pathogenesis models and clinical management. We conducted a population-based, prospective, case-control study to determine the association of specific perinatal stroke diseases with known thrombophilias. Children with idiopathic magnetic resonance imaging–classified neonatal arterial ischemic stroke (NAIS), arterial presumed perinatal ischemic stroke (APPIS), or fetal periventricular venous infarction (PVI) were recruited. Standardized thrombophilia evaluations were performed after 12 months of age on stroke cases and controls, including quantified proteins C and S, antithrombin, factors VIII/IX/XI, fibrinogen, lipoprotein(a), homocysteine, lupus anticoagulant, anticardiolipin antibodies and genotyping of factor V Leiden (FVL), factor II G20210A (FII), and methylenetetrahydrofolate reductase C677T. A total of 212 children were studied: 46 with NAIS, 34 with APPIS, 55 with PVI, and 77 controls (male, 53%; median age, 4.8 years). Of 14 parameters, no differences were observed in 12, including all common thrombophilias. Mean prothrombin time was shorter in arterial strokes (P < .001). Rates of antiphospholipid antibodies were low, comparable to those in controls, and resolved on repeat testing. FVL and FII rates were comparable to population norms. Total number of possible abnormalities did not differ between cases and controls. Our prospective, population-based, controlled, disease-specific study suggests minimal association between perinatal stroke and thrombophilia. This does not exclude the possibility of disordered coagulation at the time of stroke but suggests testing in childhood is not indicated.
•Thrombophilia in children with perinatal stroke is rare, with rates similar to those in the normal population.•Routine testing in childhood is not indicated.
Rapid onset Obesity, Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) is a rare syndrome whose underlying pathophysiology and etiology remain elusive. We present the ...case of a 36-month-old boy with the classic symptoms of ROHHAD and a neuroendocrine tumor, who progressed rapidly and subsequently succumbed to cardiorespiratory arrest because of hypoventilation. His magnetic resonance imaging findings at the initial diagnosis and the brain autopsy results are detailed. The literature was reviewed to summarize the current understanding of the underlying mechanism of this rare disorder.
The Royal College Comprehensive Objective Examination in Neurology provides certification for Canadian neurologists and consists of a written examination and the Observed Structured Clinical ...Encounter (OSCE). The OSCE portion of the certification involves residents visiting several patient stations where they address case scenarios with an examiner. Unfortunately, residents lack exam preparation time due to demanding work hours. In response to resident needs, we created a novel, virtual preparation OSCE program – “prepOSCE” – and evaluated its efficacy. The prepOSCE program employed a proprietary virtual solution from CTC Communications Corp. Ten virtual sessions accommodated 70 residents totally. Seven Canadian physicians and two co-chairs created case scenarios for the stations. On session day, seven residents arrived in a virtual plenary room for briefing followed by assignment to a station by CTC. Residents then moved virtually through prepOSCE stations a different examiner and case scenario in each. Following their session, residents and evaluators were surveyed to capture experiences. The average program rating was 4.22 out of 5 (n = 36 residents of 70 residents who participated in the program) and 4.35 (n = 17 evaluators). Ninety-two percent of residents agreed or strongly agreed that they would recommend this program to their peers; they would like prepOSCE to continue next year; and the program was relevant and added value to their studies. The positive feedback received from prepOSCE participants indicates there is a need for a program like prepOSCE. This model has potential for expansion and it is hoped that specialties outside of neurology could benefit from a similar program.
•Epilepsy in response to bathing can have varied triggers and semiologies•MDB5-associated neurological disorder, MAND, can result in reflex epilepsy•The genotypes associated with bathing epilepsy ...should expand to include 2q22.3q23.2•A modified EEG montage can safely capture an ictal reflex bathing seizure•Untreated bathing related reflex epilepsy with autonomic involvement has the potential for significant injury.
Humans with obstructive sleep apnea (OSA) experience multiple obstructive apneas every hour during sleep. During each apnea, patients are exposed to hypoxia, hypercapnia and subatmospheric ...intrathoracic pressure swings. Hypoxia and hypercapnia increase sympathetic activity, causing daytime hypertension in patients with OSA. The swings in intrathoracic pressure increase cardiac transmural pressure, decrease preload, and increase afterload, augmenting metabolic demand at the same time as arterial oxygen content decreases. The acute effects of OSA on cardiac function are not well known, although some patients are apparently susceptible to them because there is an increased incidence of nocturnal death from sudden cardiac causes among OSA patients. We conducted this investigation to determine if repeated inspiratory efforts against an occluded airway acutely impair cardiac contractile function. Using a small animal model, the anesthetised spontaneously breathing rat, we explored, at a whole muscle and molecular level, the cardiac contractile changes that occur during a 3 hour period of inspiratory occlusions, at a whole muscle and molecular level. We hypothesized that cardiac contractility would be impaired and associated with myocardial necrosis and a depression in myofibril ATPase activity. We measured a fall in MAP from baseline to the end of 3 h of occlusions, becoming significant after 2.7 h (91 ± 3 mmHg vs 106 ± 4 mmHg (control), p < 0.05, repeated measures ANOVA, Holm-Sidak post-hoc). This was accompanied by a prolongation of the MAP recovery following each occlusion at the end of the protocol (time to 90% of baseline MAP, 49.4 ± 6.5 s vs. 39.2 ± 4.6 s, p = 0.042, paired t-test). There was no change in LV dP/dt or LV dP/dt40, however we observed a reduction in -dP/dt and an increase in tau that indicate diastolic dysfunction. The contractile reserve of the left ventricle was also reduced following 3h of occlusions, measured as a blunting of the contractile response to dobutamine infusion (increase of 170% (sham) vs. 66% (occluded), p < 0.01, t-test). Molecular level dysfunction was demonstrated by a decrease in Ca2+-activated myofibril ATPase activity (p < 0.0001, t-test, n = 9 sham, 9 occluded). There was an increase in the serum cTnT concentration in 5 of 12 animals, indicating myocardial necrosis (p=0.002, fisher exact test). We found cardiovascular dysfunction as evidenced by impaired myocardial contractility, impaired myofibril ATPase activity, and myocardial necrosis. The finding that repeated inspiratory occlusions cause myocardial dysfunction should be considered and further investigated. Collectively, these results may account for the acute pathological effects of OSA.
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