Patient-reported outcomes and functional assessments of patients with Alkaptonuria in a 3-year Nitisinone treatment trial.

Alkaptonuria is a rare disorder of tyrosine catabolism caused by deficiency of homogentisate 1,2-dioxygenase that leads to accumulation of homogentisic acid (HGA). Deposition of HGA-derived polymers in connective tissue causes progressive arthropathy of the spine and large joints, cardiac valvular disease, and genitourinary stones beginning in the fourth decade of life. Nitisinone, a potent inhibitor of the upstream enzyme, 4-hydroxyphenylpyruvate dioxygenase, dramatically reduces HGA production. As such, nitisinone is a proposed treatment for alkaptonuria. A randomized clinical trial of nitisinone in alkaptonuria confirmed the biochemical efficacy and tolerability of nitisinone for patients with alkaptonuria but the selected primary outcome did not demonstrate significant clinical benefit. Given that alkaptonuria is a rare disease with slow progression and variable presentation, identifying outcome parameters that can detect significant change during a time-limited clinical trial is challenging. To gain insight into patient-perceived improvements in quality of life and corresponding changes in physical function associated with nitisinone use, we conducted a post-hoc per protocol analysis of patient-reported outcomes and a functional assessment. Analysis revealed that nitisinone-treated patients showed significant improvements in complementary domains of the 36-Item Short-Form Survey (SF-36) and 6-min walk test (6MWT). Together, these findings suggest that nitisinone improves both quality of life and function of patients with alkaptonuria. The observed trends support nitisinone as a therapy for alkaptonuria.

Perception of Medical Student Mistreatment: Does Specialty Matter?

PURPOSE: Medical student mistreatment is pervasive, yet whether all physicians have a shared understanding of the problem is unclear. The authors presented professionally designed trigger videos to physicians from 6 different specialties to determine if they perceive mistreatment and its severity similarly. METHOD: From October 2016 to August 2018, resident and attending physicians from 10 U.S. medical schools viewed 5 trigger videos showing behaviors that could be perceived as mistreatment. They completed a survey exploring their perceptions. The authors compared perceptions of mistreatment across specialties and, for each scenario, evaluated the relationship between specialty and perception of mistreatment. RESULTS: Six-hundred fifty resident and attending physicians participated. There were statistically significant differences in perception of mistreatment across specialties for 3 of the 5 scenarios: aggressive questioning (range, 74.1%-91.2%), negative feedback (range, 25.4%-63.7%), and assignment of inappropriate tasks (range, 5.5%-25.5%) (P ≤ .001, for all). After adjusting for gender, race, professional role, and prior mistreatment, physicians in surgery viewed 3 scenarios (aggressive questioning, negative feedback, and inappropriate tasks) as less likely to represent mistreatment compared with internal medicine physicians. Physicians from obstetrics-gynecology and "other" specialties perceived less mistreatment in 2 scenarios (aggressive questioning and negative feedback), while family physicians perceived more mistreatment in 1 scenario (negative feedback) compared with internal medicine physicians. The mean severity of perceived mistreatment on a 1 to 7 scale (7 most serious) also varied statistically significantly across the specialties for 3 scenarios: aggressive questioning (range, 4.4-5.4; P < .001), ethnic insensitivity (range, 5.1-6.1; P = .001), and sexual harassment (range, 5.5-6.3; P = .004). CONCLUSIONS: Specialty was associated with differences in the perception of mistreatment and rating of its severity. Further investigation is needed to understand why these perceptions of mistreatment vary among specialties and how to address these differences.

Exploring Perspectives from Internal Medicine Clerkship Directors in the USA on Effective Narrative Evaluation: Results from the CDIM National Survey.

PURPOSE: Clinical performance evaluations play a critical role in determining medical school clerkship grades. This study aimed to provide clarification from clerkship directors in internal medicine on what constitutes an effective and informative narrative description of student performance. METHODS: In September 2016, the Clerkship Directors in Internal Medicine (CDIM) electronically administered its annual, voluntary, and confidential cross-sectional survey of its US membership. One section of the survey asked six questions regarding the helpful components of an effective narrative evaluation. Respondents were asked to rate the effectiveness of elements contained within narrative evaluations of students. RESULTS: Ninety-five CDIM members responded to the survey with an overall response rate of 74.2%. Descriptions of skills and behaviors were felt to be the most important, followed by a description of the overall synthetic or global assessment level of the student. Descriptions of personality and attitude were the next highest rated feature followed by adjectives describing performance. Length was felt to be the least important component. In free-text comments, several respondents indicated that direct observation of performance and specific examples of skills and behaviors are also desirable. CONCLUSIONS: Narrative evaluations of students that explicitly comment on skills, behaviors, and an overarching performance level of the learner are strongly preferred by clerkship directors. Direct observation of clinical performance and giving specific examples of such behaviors give evaluations even more importance. Faculty development on evaluation and assessment should include instruction on these narrative assessment characteristics.

Medical Student Mistreatment-an Obstetrics and Gynecology Perspective: a Pilot Study.

OBJECTIVE: To determine if perception of mistreatment and severity in each of the professional videotaped vignettes was similar between participants of differing professional status (Ob/Gyn attending physicians, resident physicians, and medical students), previous mistreatment status, ethnic minority status, and gender. METHODS: Three video vignettes were filmed portraying possible medical student mistreatment during an obstetrics and gynecology clerkship. Prior to watching the videos, all participants were asked to complete a questionnaire related to their prior experience with mistreatment as a medical student along with other demographic details. After viewing each video, participants were asked to rate the video as representing mistreatment (yes/no) and, if yes, the severity of medical student mistreatment. RESULTS: Eight attending physicians, ten resident physicians, and ten medical students participated in this study. Professional status, previous mistreatment status, ethnic minority status, and gender did not affect how participants perceived mistreatment or the severity of the video vignettes. Fifty percent (14/28) of participants reported previous mistreatment as a medical student, all of which occurred during their third year of medical school. CONCLUSION: Medical students, resident physicians, and attending physicians generally agreed which video vignettes represented medical student mistreatment and the level of severity of the event.

Faculty development for the evaluation system: a dual agenda.

Faculty development for the evaluation process serves two distinct goals. The first goal is to improve the quality of the evaluations submitted by the faculty. Providing an accurate assessment of a learner's capabilities is a skill and, similar to other skills, can be developed with training. Frame-of-reference training serves to calibrate the faculty's standard of performance and build a uniform language of the evaluation. Second, areas for faculty professional growth can be identified from data generated from learners' evaluations of the faculty using narrative comments, item-level comparison reports, and comparative rank list information. This paper presents an innovative model, grounded in institutional experience and review of the literature, to provide feedback to faculty evaluators, thereby improving the reliability of the evaluation process, and motivating the professional growth of faculty as educators.

A 3-year randomized therapeutic trial of nitisinone in alkaptonuria.

Alkaptonuria is a rare, autosomal recessive disorder of tyrosine degradation due to deficiency of the third enzyme in the catabolic pathway. As a result, homogentisic acid (HGA) accumulates and is excreted in gram quantities in the urine, which turns dark upon alkalization. The first symptoms, occurring in early adulthood, involve a painful, progressively debilitating arthritis of the spine and large joints. Cardiac valvular disease and renal and prostate stones occur later. Previously suggested therapies have failed to show benefit, and management remains symptomatic. Nitisinone, a potent inhibitor of the second enzyme in the tyrosine catabolic pathway, is considered a potential therapy; proof-of-principle studies showed 95% reduction in urinary HGA. Based on those findings, a prospective, randomized clinical trial was initiated in 2005 to evaluate 40 patients over a 36-month period. The primary outcome parameter was hip total range of motion with measures of musculoskeletal function serving as secondary parameters. Biochemically, this study consistently demonstrated 95% reduction of HGA in urine and plasma over the course of 3 years. Clinically, primary and secondary parameters did not prove benefit from the medication. Side effects were infrequent. This trial illustrates the remarkable tolerability of nitisinone, its biochemical efficacy, and the need to investigate its use in younger individuals prior to development of debilitating arthritis.

Patient safety education at U.S. and Canadian medical schools: results from the 2006 Clerkship Directors in Internal Medicine survey.

PURPOSE: To describe current patient safety curricula at U.S. and Canadian medical schools and identify factors associated with adoption of these programs. METHOD: A survey was mailed to institutional members of the Clerkship Directors in Internal Medicine at U.S. and Canadian academic medical schools in 2006. Respondents self-reported implementation of patient safety curricula and associated methods of instruction at the institution level. RESULTS: The survey had a 76% response rate (83/110). Only 25% of institutional members reported that their schools had explicit patient safety curricula. All respondents that reported having curricula use lectures and small-group instruction, and these were more likely to occur in preclinical settings. Topics and methods of instruction included reporting adverse incidents and analysis of medical errors; improvement of physician order writing to prevent medication errors; core measures; national patient safety goals; and standardization of medical care through the use of clinical guidelines and order set templates. Although only 25% of respondents reported having explicit curricula, 72% agreed that patient safety instruction should occur during medical school. CONCLUSIONS: Despite calls from regulatory, medical, and educational organizations to increase patient safety training of medical students, internal medicine clerkship directors report that few schools in the United States and Canada have implemented specific patient safety curricula. Most existing patient safety curricula use lecture and small-group discussion as preferred methods of instruction.

Use of electronic medical records by physicians and students in academic internal medicine settings.

PURPOSE: Electronic medical records (EMRs) have been touted as one method to improve quality and safety in medical care, and their use has recently increased. The purpose of this study is to describe current use of EMRs by medical students at U.S. and Canadian medical schools. METHOD: In 2006 the authors performed a cross-sectional survey of the Clerkship Directors in Internal Medicine institutional members at U.S. and Canadian academic health centers. Outcome measures included implementation of EHRs, EHR use by students, and the challenges of having students use EMRs. RESULTS: Of 110 members, 82 (74.5%) responded. Of those 82, 48 (58%) reported using an EMR in the ambulatory setting (excluding Veterans' Affairs medical centers) of their institutions, and only 21 of those 48 (44%) had policies regarding medical student documentation of progress notes in the EMR during the ambulatory internal medicine (IM) clerkship. Schools were dichotomously split; about half (23/48, 48%) required and about half (25/48, 52%) prohibited allowing students to document in the EMR. The programs that prohibited medical students from documenting in the EMR primarily cited billing concerns. Other issues regarding student use of EMRs included student access, faculty concerns, and note quality. CONCLUSIONS: Use of EMRs by IM clerkship students is common, yet many institutions do not have policies regarding student use. Where policies do exist, they vary, and many prohibit students from using EMRs. Concerns about documentation as it relates to billing seem to be a significant factor in prohibiting students' use of EMRs.

Training and assessment of ECG interpretation skills: results from the 2005 CDIM survey.

BACKGROUND: Despite published consensus-based statements on assessment of ECG interpretation skills, studies and curricula regarding the training needed to obtain basic ECG interpretation skills are lacking. These consensus statements have focused on attaining competency in ECG interpretation during postgraduate training; however, recommendations regarding assessment of competency in the undergraduate curriculum are not discussed. PURPOSE: The purpose is to describe the current methods of teaching and assessing ECG interpretation skills across institutions on the 3rd-year internal medicine (IM) clerkship. METHOD: In 2005, the Clerkship Directors in Internal Medicine surveyed its institutional members. Twelve questions on the survey dealt with ECG interpretation. Descriptive statistics, chi-square, and Mann-Whitney U were used for analysis. RESULTS: Eighty-eight of 109 members (81%) responded to the survey. Overall, 89% of institutional respondents feel that ECG interpretation is an important clinical skill for medical students with 92% indicating that instruction occurs on the IM clerkship. Lectures (75%) and teaching rounds (44%) were the most cited methods of instruction. Most schools spend 1 to 6 hr during the IM clerkship on formal ECG instruction. Over 63% indicated that ECG interpretation skills are assessed during the clerkship. The most common assessment methods were written exam (40%) and OSCE (23%). CONCLUSIONS: Objective data regarding attainment and assessment of basic ECG interpretation competency in the undergraduate curriculum are lacking; our report provides preliminary descriptive data regarding ECG teaching and assessment on the 3rd-year IM clerkship. Further studies are needed to determine the ideal method of instruction and evaluation of this important clinical skill.

Training and assessment of CXR/basic radiology interpretation skills: results from the 2005 CDIM Survey.

BACKGROUND: Despite published literature demonstrating deficiencies in chest radiograph (CXR)/basic radiology interpretation skills of 4th-year medical students, studies and subsequent curricula regarding the training needed to obtain these skills are lacking. Terms such as clinical exposure and radiology teaching have been used to describe the experience for these basic interpretive skills, but best practice methods of delivery, let alone common methods, have yet to be defined. PURPOSE: The objective is to describe the current methods of teaching and assessing CXR/basic radiology interpretation skills across institutions on the 3rd-year internal medicine (IM) clerkship. METHODS: In 2005, the Clerkship Directors in Internal Medicine (CDIM), an international organization representing U.S. and Canadian medical schools, surveyed its institutional members. Twelve questions on the survey dealt with X-ray interpretation. RESULTS: Eighty-eight of 109 members (81%) responded to the survey. Overall, 81% of respondents felt that CXR interpretation is an important clinical skill for medical students. Seventy-six percent indicated that instruction in these skills occurs on the IM clerkship. The most cited methods of instruction were lectures (56%) and teaching rounds (48%). Most schools spent on average of 2 to 4 hr during the IM clerkship on formal radiology instruction. Only 33% indicated that radiology interpretation skills are assessed during the clerkship. The most common assessment methods were written examination (19%) and OSCE (19%). CONCLUSION: Substantive data regarding attainment and assessment of CXR/basic radiology interpretation skills in the undergraduate curriculum are lacking. Our study provides preliminary descriptive data regarding CXR instruction and assessment on the 3rd-year IM clerkship.

Diffuse T-wave inversions associated with electroconvulsive therapy.

Electroconvulsive therapy (ECT) can lead to ST depression and arrhythmias in patients with coronary artery disease, most likely secondary to increased myocardial oxygen demand. This report describes a case of ECT-associated, global T-wave inversions in a patient with chronic atrial fibrillation treated with digoxin. Laboratory evaluation and echocardiography were normal. These T-wave changes may result from increased sympathetic activity associated with ECT. Prospective studies suggest that this finding may be seen in up to 4% of patients undergoing ECT.

Effect of a physician-directed educational campaign on performance of proper diabetic foot exams in an outpatient setting.

BACKGROUND: The established guidelines for a diabetes foot examination include assessing circulatory, skin, and neurological status to detect problems early and reduce the likelihood of amputation. Physician adherence to the guidelines for proper examination is less than optimal. OBJECTIVE: Our objective was to increase compliance with the performance of a proper foot examination through a predominantly physician-directed interventional campaign. METHODS: The study consisted of 3 parts: a retrospective chart review to estimate background compliance, an educational intervention, and prospective chart review at 3 and 6 months. A properly documented foot examination was defined as assessing at least 2 of the 3 necessary components. The educational intervention consisted of 2 lectures directed at resident physicians and a quality assurance announcement at a general internal medicine staff meeting. Clinic support staff were instructed to remove the shoes and socks of all diabetic patients when they were placed in exam rooms, and signs reminding diabetics were placed in each exam room. RESULTS: There was a significant increase in the performance of proper foot examination over the course of the study (baseline 14.0%, 3 months 58.0%, 6 months 62.1%; P <.001). Documentation of any component of a proper foot examination also increased substantially (32.6%, 67.3%, 72.5%; P <.001). Additionally, performance of each component of a proper exam increased dramatically during the study: neurological (13.5%, 35.8%, 38.5%; P <.001), skin (23.0%, 64.2%, 69.2%; P <.001), and vascular (14.0%, 51.2%, 50.5%; P <.001). CONCLUSIONS: Patients with diabetes are unlikely to have foot examinations in their primary medical care. A simple, low-cost educational intervention significantly improved the adherence to foot examination guidelines for patients with diabetes.

Paget's disease of bone in an Indian patient: genetic and environmental factors.

Epidemiologic data have shown that Paget's disease of bone is common among people of Anglo-Saxon descent, but it is believed to be rare in India and Asia. The worldwide prevalence of the disease shows pronounced geographic and ethnic heterogeneity, and can vary in populations through migration or loss of geographic isolation. The risk to an individual who changes geographic locations, however, is difficult to quantify because the specific genetic or environmental factors responsible for Paget's disease have yet to be completely determined. We describe a case of monostotic Paget's disease in a 59-year-old Asian Indian man, and consider the potential epidemiologic and etiologic influences (moving to an area of higher disease prevalence, role of genetics, viral exposures) that may have increased his risk for the disorder. The patient presented with several months of right anterior leg pain, and was found on examination to have anterior bowing of the tibia as well as warmth and an audible bruit. Radiographs of the tibia demonstrated a V-shaped lucency, enhanced trabecular pattern, and cortical thickening. Whole-body bone scan confirmed monostotic disease with expansile uptake in the tibia, and laboratory data revealed an elevated urinary collagen N-telopeptide that normalized after bisphosphonate treatment, as did his clinical symptoms. In light of the environmental factors that appear significantly to influence the development of Paget's disease, the diagnosis should be considered in patients traditionally considered at low risk for the disorder.