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Objectives: The Accreditation Council for Graduate Medical Education requires residency training programs to teach and assess professionalism in residents; however, programs may struggle to successfully remediate residents not meeting professionalism standards. To assist programs with this complex issue, a Professionalism Remediation Task Force was formed by the Council of Emergency Medicine Residency Directors (CORD-EM), which surveyed program directors (PDs) concerning their experiences. The purpose of this study is to report survey results regarding the identification and rating of unprofessional behaviors and challenges in the evaluation and remediation of professionalism. Methods: In June 2010, the task force sent an anonymous survey via the CORD-EM listserv to PDs with active EM programs. Results: Fifty percent (77 ⁄ 154) of eligible PDs responded to the survey. Most PDs rated the unprofessional behaviors of interpersonal ⁄ communication conflicts, lack of responsibility during patient care, lack of respect of coworkers, and reports of impairment as ‘‘critical’’; repeated tardiness, incomplete work, poor ability to accept feedback, poor attitude, and repetitive unresponsiveness to aid colleagues were rated as ‘‘very serious’’; frequent missed deadlines were ‘‘serious’’; and repetitive failure to complete medical records was rated as ‘‘mildly serious.’’ A resident with ‘‘less serious’’ professionalism issues was also felt to be likely to have ‘‘serious’’ or ‘‘critical’’ issues ‘‘often’’ (33.8% of respondents) or ‘‘always’’ (6.5%). The most common methods of assessment were clinical ⁄ advisor evaluations. However, existing assessment methods were described as inadequate in identifying serious professionalism issues by 50.7% of responding PDs. Unprofessionalism was most commonly discovered by unofficial faculty complaint (54.5%). Eighty percent report that professionalism is more difficult to remediate than other core competencies. Resident ownership of the problem was reported as most critical to remediation success (84.4%). PDs perceived the greatest challenges in residency remediation to be lack of resident insight or responsibility for the problem (45.2%) and personality ⁄ behavioral issues (32.9%).

Juvenile dermatomyositis (JDM) is a systemic vasculopathy characterized by skin and muscle involvement. Diagnostic criteria include muscle weakness and rash, electromyographic evidence of myopathy, muscle biopsy demonstrating inflammatory infiltrate with or without atrophy, and elevated muscle enzymes, although muscle enzymes may be normal (1). The etiology of JDM has not been established, but evidence suggests that environmental triggers such as an antecedent infection may play a role (2). Many children with JDM have a TNFα–308A allele which is associated with vascular occlusion(3) and increased TNF-α production (4). In addition, JDM disease activity has been associated with decreased absolute numbers of CD3−CD56+/16+ NK cells and elevated von Willebrand factor antigen (vWF:Ag) levels (5). Genes involved in immune responses, including those related to dendritic cell maturation and vasculature remodeling, are expressed at higher levels in muscle biopsies from children with greater duration of untreated disease (6). One particular manifestation of JDM is the presence of abnormal nailfold capillaries, evidenced by capillary dropout, capillary dilatation, and bushy loops (7). Nailfold capillaroscopy (NFC) is a noninvasive, reproducible technique that provides information about abnormalities in periungual microvasculature. It takes into account quantitative measurements of capillary density or end-row loop (ERL) loss as well as the presence of avascularity and abnormal capillaries represented by “bushy” or “bizarre” loops (7,8). ERL scores between 7.0 and 11.5 have been observed in healthy children without JDM or other recognized autoimmune diseases (9). Serial nailfold capillary observations have demonstrated progression of nailfold changes over time in children with JDM (6), 10- 12) and their degree of morphologic changes may correlate with the clinical course of the disease (10). While abnormal NFC are not part of the Bohan and Peter criteria for dermatomyositis, they may reflect systemic vasculopathy, and the data can be used to evaluate patients with JDM (13). ERL regeneration is associated with a shorter duration of untreated disease, a unicyclic disease course resulting in discontinuation of all immunosuppressive therapy before 36 months (versus non-unicyclic in which continuous or repeated immunosuppressive therapy is required), and a lower skin disease activity score (DAS), (11), (12). Lower numbers of ERL in children with JDM are also associated with decreased bioavailability of oral prednisolone compared with intravenous methylprednisolone, suggesting a relationship between systemic vasculopathy and decreased absorption of oral prednisolone (14). Another recent study also showed that six months after diagnosis, the presence of abnormal nailfold capillaries with Gottron’s papules predicted a longer time to remission (15). Given the potential uses of NFC in providing valuable clinical information with regard to JDM, we studied untreated children fulfilling criteria for definite/probable JDM for the following: 1) the association of normal ERL at the time of diagnosis with clinical and laboratory data, and 2) predictors of decreased number of ERL over time.

Hispanic ethnicity is an independent risk factor for increased morbidity and mortality in cystic fibrosis (CF) patients. In order to compare the prevalence of risk factors for morbidity and mortality between the Hispanic CF population and the non-Hispanic CF population, we performed a cross-sectional study of patients in the 2004 Cystic Fibrosis Foundation Patient Registry. Among 22,714 CF patients, 1,511 were identified as ethnic Hispanic. Hispanic patients were diagnosed earlier (2.8 vs. 3.3 years, P = 0.005) and acquired Pseudomonas aeruginosa at a younger age (6.6 years vs. 10 years, P < 0.001). FEV(1) was lower for Hispanic patients (81.5% vs. 87% predicted for those under 18 years old [P < 0.001] and 2.1 L vs. 2.3 L for those 18 years and older [P = 0.01]). Hispanic patients had similar or better nutritional status. Hispanic patients were more likely to be diagnosed with liver disease (OR 1.31 [1.1, 1.56]) but less likely to be diagnosed with depression (OR 0.53 [0.39, 0.68]), bone and joint disease (OR 0.55 [0.41, 0.71]), or CF-related diabetes (OR 0.53 [0.43, 0.62]). Hispanic patients had lower median income by zip code ($41,930 vs. $47,341; P < 0.001), a higher rate of government insurance (55.2% vs. 32.0%; P < 0.001), and greater percentage of mothers with less than a high school education (26.7% vs. 6.5%; P < 0.001). We conclude that there is an increased prevalence of important risk factors for morbidity and mortality in the Hispanic CF population.

ADV has emerged as an important pathogen in children undergoing allogeneic HPCT. A prospective study of the epidemiology of ADV infection and preemptive therapy of high risk ADV infections in children undergoing HPCT was undertaken. Cultures of throat, urine, and stool for viral pathogens and plasma for ADV PCR were obtained prior to transplantation, weekly for the first 100 days, and then monthly for one yr. Children developing high-risk ADV infections were treated preemptively with cidofovir 1 mg/kg/day given three times weekly for three wk. A case-controlled study was performed to identify risk factors for high-risk ADV infections. Seven (18%) of the 38 subjects developed high-risk ADV infections usually within 100 days of HPCT and were preemptively treated with i.v. cidofovir at a dose of 1 mg/kg/dose three times weekly for nine doses. High-risk ADV infections resolved in all seven patients without renal toxicity. CMV viremia occurred in two of seven patients during or shortly after therapy with cidofovir. A case-control study did not identify any risk factors that achieved statistical significance. Treatment with a modified dosing regimen of cidofovir was well-tolerated and high-risk ADV infections resolved in all patients.