Purpose: Patients with neurofibromatosis 1 (NF1) are at increased risk for a variety of cardiovascular disorders, but the natural history and pathogenesis of these abnormalities are poorly understood. Methods: The National Neurofibromatosis Foundation convened an expert task force to review current knowledge about cardiovascular manifestations of NF1 and to make recommendations regarding clinical management and research priorities related to these features of the disease. Results: This report summarizes the NF1 Cardiovascular Task Force's current understanding of vasculopathy, hypertension, and congenital heart defects that occur in association with Key Words: neurofibromatosis 1, vasculopathy, hypertension, congenital heart defects Neurofibromatosis 1 (NF1), an autosomal dominant disease, is one of the most common mendelian disorders. 1 It is characterized by extremely variable expressivity, but most patients have café-au-lait spots, intertriginous freckling, dermal and plexiform neurofibromas, and learning disabilities. 2 People with NF1 may also develop cardiac and vascular disease, but the frequency, natural history, and pathogenesis of these abnormalities are uncertain. The National Neurofibromatosis Foundation convened an expert panel, the NF1 Cardiovascular Task Force, to review current knowledge about the cardiovascular manifestations of NF1 and to make recommendations regarding clinical management and research priorities. This is the report of that Task Force.Neurofibromas, the characteristic tumors of NF1, can develop within the heart, obstruct blood flow in the heart or major vessels by compression or invasion, or erode a vessel and cause hemorrhage. Fortunately, these are rare complications. This report will concentrate on the three most common cardiovascular manifestations of NF1, viz., vasculopathy, hypertension, and congenital heart defects.People with NF1 constitute a substantial fraction of all patients with dysplastic renal artery stenosis, 3 early-onset cerebrovascular disease, 4 or pheochromocytomas, 5 and cardiovascular disease is a frequent cause of premature death in individuals with NF1. Sørensen and associates 6 found that myocardial infarction and cerebrovascular accidents often occurred at a younger than expected age among NF1 patients. Zöller et al. 7 reported that cardiovascular disease, hemorrhage, and embolism were frequent causes of death in 70 adult NF1 patients who were followed for 12 years. Hypertension was significantly associated with mortality, and the mean age at death among the NF1 patients was approximately 14 years younger than expected. The median age of death reported on death certificates of 3,253 individuals with probable NF1 was approximately 15 years less than expected in another study. 8 Diagnoses suggestive of NF1 vasculopathy were listed 7.2 times more often than expected among NF1 patients Ͻ 30 years old and 2.2 times more often than expected among those who were 30 to 40 years old at the time of death. NF1 VASCULOPATHY Epidemiology and clinical featuresNF1 c...

Context Physicians depend on the medical literature to keep current with clinical information. Little is known about residents' ability to understand statistical methods or how to appropriately interpret research outcomes. Objective To evaluate residents' understanding of biostatistics and interpretation of research results. Design, Setting, and Participants Multiprogram cross-sectional survey of internal medicine residents. Main Outcome Measure Percentage of questions correct on a biostatistics/study design multiple-choice knowledge test. Results The survey was completed by 277 of 367 residents (75.5%) in 11 residency programs. The overall mean percentage correct on statistical knowledge and interpretation of results was 41.4% (95% confidence interval [CI], 39.7%-43.3%) vs 71.5% (95% CI, 57.5%-85.5%) for fellows and general medicine faculty with research training (PϽ.001). Higher scores in residents were associated with additional advanced degrees (50.0% [95% CI, 44.5%-55.5%] vs 40.1% [95% CI, 38.3%-42.0%]; PϽ.001); prior biostatistics training (45.2% [95% CI, 42.7%-47.8%] vs 37.9% [95% CI, 35.4%-40.3%]; P=.001); enrollment in a university-based training program (43.0% [95% CI, 41.0%-45.1%] vs 36.3% [95% CI, 32.6%-40.0%]; P=.002); and male sex (44.0% [95% CI, 41.4%-46.7%] vs 38.8% [95% CI, 36.4%-41.1%]; P = .004). On individual knowledge questions, 81.6% correctly interpreted a relative risk. Residents were less likely to know how to interpret an adjusted odds ratio from a multivariate regression analysis (37.4%) or the results of a Kaplan-Meier analysis (10.5%). Seventy-five percent indicated they did not understand all of the statistics they encountered in journal articles, but 95% felt it was important to understand these concepts to be an intelligent reader of the literature. Conclusions Most residents in this study lacked the knowledge in biostatistics needed to interpret many of the results in published clinical research. Residency programs should include more effective biostatistics training in their curricula to successfully prepare residents for this important lifelong learning skill.

Key Points Question How do minority resident physicians view the role of race/ethnicity in their training experiences? Findings This qualitative study of 27 minority resident physicians found that participants described 3 major themes: a daily barrage of microaggressions and bias, minority residents tasked as race/ethnicity ambassadors, and challenges negotiating professional and personal identity while seen as “other.” Meaning Results of this study suggest that minority residents face extra workplace burdens during a period already characterized by substantial stress, warranting further attention from educators, institutions, and accreditation bodies.

Direct observation of competence training, a new multifaceted approach to faculty development, leads to meaningful changes in rating behaviors and in faculty comfort with evaluation of clinical skills.