Competency-based medical education and continuing professional development: A conceptualization for change.

Competency-based medical education (CBME) is as important in continuing professional development (CPD) as at any other stage of a physician's career. Principles of CBME have the potential to revolutionize CPD. Transitioning to CBME-based CPD will require a cultural change to gain commitment from physicians, their employers and institutions, CPD providers, professional organizations, and medical regulators. It will require learning to be aligned with professional and workplace standards. Practitioners will need to develop the expertise to systematically examine their own clinical performance data, identify performance improvement opportunities and possibilities, and develop a plan to address areas of concern. Health care facilities and systems will need to produce data on a regular basis and to develop and train CPD educators who can work with physician groups. Stakeholders, such as medical regulatory authorities who are responsible for licensing physicians and other standard-setting bodies that credential and develop maintenance-of-certification systems, will need to change their paradigm of competency enhancement through CPD.

Familial idiopathic small-bowel and colonic varices in three siblings.

Idiopathic small-bowel and colonic varices are a rare source of bleeding from the gastrointestinal tract. To date there are only eight published case series of familial idiopathic small-bowel and colonic varices. We present a case series detailing three affected siblings who presented with significant lower gastrointestinal bleeding and had multiple varices on endoscopy and imaging. Though not confirmed, consanguinity in the parents suggests an autosomal recessive mode of inheritance. We summarize the literature to date and describe our institution's experience of endoscopy, diagnostic imaging, and treatment in these patients.

Adult siblings with homozygous G6PC3 mutations expand our understanding of the severe congenital neutropenia type 4 (SCN4) phenotype.

BACKGROUND: Severe congenital neutropenia type 4 (SCN4) is an autosomal recessive disorder caused by mutations in the third subunit of the enzyme glucose-6-phosphatase (G6PC3). Its core features are congenital neutropenia and a prominent venous skin pattern, and affected individuals have variable birth defects. Oculocutaneous albinism type 4 (OCA4) is caused by autosomal recessive mutations in SLC45A2. METHODS: We report a sister and brother from Newfoundland, Canada with complex phenotypes. The sister was previously reported by Cullinane et al., 2011. We performed homozygosity mapping, next generation sequencing and conventional Sanger sequencing to identify mutations that cause the phenotype in this family. We have also summarized clinical data from 49 previously reported SCN4 cases with overlapping phenotypes and interpret the medical histories of these siblings in the context of the literature. RESULTS: The siblings' phenotype is due in part to a homozygous mutation in G6PC3, [c.829C > T, p.Gln277X]. Their ages are 38 and 37 years respectively and they are the oldest SCN4 patients published to date. Both presented with congenital neutropenia and later developed Crohn disease. We suggest that the latter is a previously unrecognized SCN4 manifestation and that not all affected individuals have an intellectual disability. The sister also has a homozygous mutation in SLC45A2, which explains her severe oculocutaneous hypopigmentation. Her brother carried one SLC45A2 mutation and was diagnosed with "partial OCA" in childhood. CONCLUSIONS: This family highlights that apparently novel syndromes can in fact be caused by two known autosomal recessive disorders.

Hepatitis C virus replicates in the same immune cell subsets in chronic hepatitis C and occult infection.

BACKGROUND & AIMS: Infection of the lymphatic system by hepatitis C virus (HCV) appears to be an intrinsic characteristic of chronic hepatitis C (CHC) and low-level (occult) HCV infection, but the subsets of immune cells involved were not defined. The aim of this study was to characterize HCV replication status and to assess virus compartmentalization in CD4+ and CD8+ T lymphocytes, B cells, and monocytes in CHC, and silent infection persisting after resolution of hepatitis C. METHODS: Immune cell subtypes isolated from 7 patients with CHC and 7 individuals with occult infection were analyzed for HCV-RNA-positive and -negative strands and, in selected cases, nonstructural protein 5A display and HCV variants. RESULTS: All subtypes of immune cells investigated support HCV replication in both forms of infection, although significant differences were found between patients, and virus loads in the cells were greater in CHC than in occult infection. Although HCV RNA occurred at a comparable frequency in all cell subtypes in CHC, monocytes contained the greatest loads. In contrast, B cells tended to carry the highest virus quantities in occult infection, whereas monocytes appeared to be the least frequently infected. Detection of HCV nonstructural protein 5A and HCV variants that were not found in plasma confirmed virus replication in different immune cell types. CONCLUSIONS: This work documents that the immune system supports HCV replication regardless of clinical appearance of infection and identifies immune cells that are reservoirs of HCV in symptomatic and occult infections.

National programmes for validating physician competence and fitness for practice: a scoping review.

OBJECTIVE: To explore and categorise the state of existing literature for national programmes designed to affirm or establish the continuing competence of physicians. DESIGN: Scoping review. DATA SOURCES: MEDLINE, ERIC, Sociological Abstracts, web/grey literature (2000-2014). SELECTION: Included when a record described a (1) national-level physician validation system, (2) recognised as a system for affirming competence and (3) reported relevant data. DATA EXTRACTION: Using bibliographic software, title and abstracts were reviewed using an assessment matrix to ensure duplicate, paired screening. Dyads included both a methodologist and content expert on each assessment, reflective of evidence-informed best practices to decrease errors. RESULTS: 45 reports were included. Publication dates ranged from 2002 to 2014 with the majority of publications occurring in the previous six years (n=35). Country of origin--defined as that of the primary author--included the USA (N=32), the UK (N=8), Canada (N=3), Kuwait (N=1) and Australia (N=1). Three broad themes emerged from this heterogeneous data set: contemporary national programmes, contextual factors and terminological consistency. Four national physician validation systems emerged from the data: the American Board of Medical Specialties Maintenance of Certification Program, the Federation of State Medical Boards Maintenance of Licensure Program, the Canadian Revalidation Program and the UK Revalidation Program. Three contextual factors emerged as stimuli for the implementation of national validation systems: medical regulation, quality of care and professional competence. Finally, great variation among the definitions of key terms was identified. CONCLUSIONS: There is an emerging literature focusing on national physician validation systems. Four major systems have been implemented in recent years and it is anticipated that more will follow. Much of this work is descriptive, and gaps exist for the extent to which systems build on current evidence or theory. Terminology is highly variable across programmes for validating physician competence and fitness for practice.

Evidence-based recommendations for short- and long-term management of uninvestigated dyspepsia in primary care: an update of the Canadian Dyspepsia Working Group (CanDys) clinical management tool.

The present paper is an update to and extension of the previous systematic review on the primary care management of patients with uninvestigated dyspepsia (UD). The original publication of the clinical management tool focused on the initial four- to eight-week assessment of UD. This update is based on new data from systematic reviews and clinical trials relevant to UD. There is now direct clinical evidence supporting a test-and-treat approach in patients with nondominant heartburn dyspepsia symptoms, and head-to-head comparisons show that use of a proton pump inhibitor is superior to the use of H2-receptor antagonists (H2RAs) in the initial treatment of Helicobacter pylori-negative dyspepsia patients. Cisapride is no longer available as a treatment option and evidence for other prokinetic agents is lacking. In patients with long-standing heartburn-dominant (ie, gastroesophageal reflux disease) and nonheartburn-dominant dyspepsia, a once-in-a-lifetime endoscopy is recommended. Endoscopy should also be considered in patients with new-onset dyspepsia that develops after the age of 50 years. Conventional nonsteroidal anti-inflammatory drugs, acetylsalicylic acid and cyclooxygenase-2-selective inhibitors can all cause dyspepsia. If their use cannot be discontinued, cotherapy with either a proton pump inhibitor, misoprostol or high-dose H2RAs is recommended, although the evidence is based on ulcer data and not dyspepsia data. In patients with nonheartburn-dominant dyspepsia, noninvasive testing for H pylori should be performed and treatment given if positive. When starting nonsteroidal anti-inflammatory drugs for a prolonged course, testing and treatment with H2RAs are advised if patients have a history of previous ulcers or ulcer bleeding.

Canadian Helicobacter Study Group Consensus Conference: Update on the approach to Helicobacter pylori infection in children and adolescents--an evidence-based evaluation.

As an update to previously published recommendations for the management of Helicobacter pylori infection, an evidence-based appraisal of 14 topics was undertaken in a consensus conference sponsored by the Canadian Helicobacter Study Group. The goal was to update guidelines based on the best available evidence using an established and uniform methodology to address and formulate recommendations for each topic. The degree of consensus for each recommendation is also presented. The clinical issues addressed and recommendations made were: population-based screening for H. pylori in asymptomatic children to prevent gastric cancer is not warranted; testing for H. pylori in children should be considered if there is a family history of gastric cancer; the goal of diagnostic interventions should be to determine the cause of presenting gastrointestinal symptoms and not the presence of H. pylori infection; recurrent abdominal pain of childhood is not an indication to test for H. pylori infection; H. pylori testing is not required in patients with newly diagnosed gastroesophageal reflux disease; H. pylori testing may be considered before the use of long-term proton pump inhibitor therapy; testing for H. pylori infection should be considered in children with refractory iron deficiency anemia when no other cause has been found; when investigation of pediatric patients with persistent or severe upper abdominal symptoms is indicated, upper endoscopy with biopsy is the investigation of choice; the 13C-urea breath test is currently the best noninvasive diagnostic test for H. pylori infection in children; there is currently insufficient evidence to recommend stool antigen tests as acceptable diagnostic tools for H. pylori infection; serological antibody tests are not recommended as diagnostic tools for H. pylori infection in children; first-line therapy for H. pylori infection in children is a twice-daily, triple-drug regimen comprised of a proton pump inhibitor plus two antibiotics (clarithromycin plus amoxicillin or metronidazole); the optimal treatment period for H. pylori infection in children is 14 days; and H. pylori culture and antibiotic sensitivity testing should be made available to monitor population antibiotic resistance and manage treatment failures.

Recommendations for the appropriate use of anti-inflammatory drugs in the era of the coxibs: defining the role of gastroprotective agents.

Treatment with anti-inflammatory drugs and the analgesic efficacy of conventional nonsteroidal anti-inflammatory drugs (NSAIDs) are compromised by a two- to fourfold increased risk of gastrointestinal complications. This increased risk has resulted in an increasing use of the new selective cyclooxygenase-2 inhibitors or coxibs, which, in clinical trials and outcomes studies, reduced gastrointestinal adverse events by 50% to 65% compared with conventional NSAIDs. However, the coxibs are not available to all patients who need them, and NSAIDs are still widely used. Moreover, treatment with a coxib cannot heal pre-existing gastrointestinal lesions, and cotherapy with an anti-secretory drug or mucosal protective agent may be required. This paper addresses the management of patients with risk factors for gastrointestinal complications who are taking NSAIDs and makes recommendations for the appropriate use of 'gastroprotective' agents (GPAs) in patients who need to take an NSAID or a coxib. When economically possible, a coxib alone is preferable to a conventional NSAID plus a GPA to minimize exposure to potential gastrointestinal damage and avoid unnecessary dual therapy. Patients at high risk require a GPA in addition to a coxib.

Evaluation of Hemo Techt NS-Plus system for use in a province-wide colorectal cancer screening program.

OBJECTIVES: The NS-Plus automated analyzer and fecal immunochemical testing (FIT) testing system (Alfresa Pharma) was evaluated for use in Newfoundland and Labrador's provincial colorectal cancer (CRC) screening program. DESIGN AND METHODS: Various method performance characteristics were evaluated including the sample stability. The sensitivity for detecting neoplastic lesions was evaluated in 249 patients scheduled for colonoscopy. Each patient collected up to 2 samples for both guaiac based testing (Hemoccult SENSA; gFOBT) and FIT using the NS-plus system (cutoff=20 µg Hb/g feces or 100 µg Hb/L) over 2 days. Data was analyzed comparing 1- and 2-day testing strategies. RESULTS: The analyzer showed acceptable linearity, precision, and accuracy. The collection device maintained acceptable sample stability for at least 7 days at: 37 °C, room temperature (~23 °C), 4-8 °C, and -20 °C. The 2-day sampling strategy identified 30% (21 of 69) of all neoplastic lesions (low and high grade adenomas and CRC) including 2 of 4 high-grade adenomas and 2 of 2 CRCs. The single day strategy identified the same high-grade adenomas and CRCs but fewer low-grade adenomas (23% of all neoplasia). Reducing the screening cutoff to the estimated 95th percentile of FIT results in the healthy adult population (10 µg Hb/g feces), detected all high-grade adenomas in the 2-day strategy. CONCLUSIONS: The NS Plus automated analyzer system detects clinically significant neoplasms and shows acceptable performance for use in a CRC screening program with the potential for gains in sensitivity by modifying the number of days of screening or through lowering the cutoff.

Mitogen-induced upregulation of hepatitis C virus expression in human lymphoid cells.

Considering growing evidence indicating that hepatitis C virus (HCV) replicates in lymphoid cells, establishment of a reliable and sensitive method for detection of HCV in these cells may provide means for monitoring the infection and the efficacy of sterilizing antiviral therapy. In this study, conditions for ex vivo augmentation and detection of the HCV genome in peripheral blood mononuclear cells (PBMCs) from patients with chronic hepatitis C (CHC) or after a sustained virological response (SVR) to antiviral treatment were assessed. Following stimulation with combinations of mitogens and/or cytokines, PBMCs and, in certain cases, affinity-purified T and B cells were examined for HCV positive- and negative-strand RNA by using RT-PCR followed by nucleic acid hybridization, while the presence of viral NS3 protein was determined by flow cytometry. HCV RNA augmentation was assessed by quantification of Southern and dot-blot hybridization signals. The results showed that treatment of peripheral lymphoid cells with mitogens stimulating T- and B-cell proliferation and with cytokines supporting their growth significantly increased HCV RNA detection in patients with both CHC and SVR. This enhancement was up to 100-fold for the HCV genome and fivefold for the NS3 protein compared with untreated cells. In conclusion, HCV RNA can be readily detected in circulating lymphoid cells in progressing hepatitis C and following SVR after ex vivo cell stimulation. As such, this method offers a new investigative tool to study HCV lymphotropism and to monitor virus presence during the course of HCV infection.