Faculty Bibliography

Paraneoplastic cerebellar degeneration (PCD) is a rare disorder that is associated with lung or gynecological malignancies and Hodgkin lymphoma. Neurologic symptoms are commonly the initial presenting sign leading to the diagnosis of an underlying malignancy. We are presenting an Asian male with progressive lower extremity weakness with EBV-positive nasopharyngeal carcinoma (NPC) and anti-Yo antibodies. Peculiarly, transient diffuse leptomeningeal enhancement is seen on MR imaging. This is the first report of PCD associated with NPC and thus illustrates that PCD embodies a boarder set of disease than previously described.

The phase of entrainment (chronotype) is known to be associated with time perspective (TP), suggesting that the state of circadian system is involved in the long-term planning of human life. However, little is known regarding the influence of circadian misalignment on long-term planning ability. The aim of this study was to investigate the association between social jetlag (SJL) and TP. A total of 1064 schoolchildren and university students (mean age ± standard deviation, 19.2 ± 2.9 years; range, 15-25 years; females, 71.7%) from four cities in the Russian Federation located between 56.9 and 61.7 degrees North completed the Munich ChronoType Questionnaire, the Pittsburgh Sleep Quality Index, the Seasonal Pattern Assessment Questionnaire, and Zimbardo Time Perspective Inventory. Study participants also indicated personal data (age, sex, height weight, place of residence, and achievements). A multiple regression analysis with stepwise inclusion of predictors in the model was performed to evaluate associations between time perspective characteristics (dependent variables) and predictor variables. The change in R² was used as the measure of effect size. Chronotype was found to be a moderate predictor of future TP (B = 0.034; ΔR²Â = 0.037). In addition, sleep quality was found to be a moderate predictor of past negative (B = 0.043; ΔR²Â = 0.074), present fatalistic (B = 0.021; ΔR²Â = 0.035), and deviation from balanced TP (B = 0.034; ΔR²Â = 0.066). Mood seasonality was a moderate predictor of present hedonistic TP (B = 0.016; ΔR²Â = 0.038), and social jetlag was a weak predictor of present fatalistic (B = 0.052; ΔR²Â = 0.019), future (B = -0.033; ΔR²Â = 0.004), and deviation from balanced TP (B = 0.047; ΔR²Â = 0.012). In conclusion, this study found a weak but significant association between social jetlag and TP in adolescents and young adults.

BACKGROUND:Intraductal papillary mucinous neoplasm (IPMN) is premalignant pancreatic lesion. International guidelines offer limited predictors of individual risk. A nomogram to predict individual IPMN malignancy risk was released, with good diagnostic performance based on a large cohort of Asian patients with IPMN. The present study validated a nomogram to predict malignancy risk and invasiveness of IPMN using both Eastern and Western cohorts. METHODS:Clinicopathological and radiological data from patients who underwent pancreatic resection for IPMN at four centres each in Eastern and Western countries were collected. After excluding patients with missing data for at least one malignancy predictor in the nomogram (main pancreatic duct diameter, cyst size, presence of mural nodule, serum carcinoembryonic antigen and carbohydrate antigen (CA) 19-9 levels, and age). RESULTS:In total, data from 393 patients who fit the criteria were analysed, of whom 265 were from Eastern and 128 from Western institutions. Although mean age, sex, log value of serum CA19-9 level, tumour location, main duct diameter, cyst size and presence of mural nodule differed between the Korean/Japanese, Eastern and Western cohorts, rates of malignancy and invasive cancer did not differ significantly. Areas under the receiver operating characteristic (ROC) curve values for the nomogram predicting malignancy were 0·745 for Eastern, 0·856 for Western and 0·776 for combined cohorts; respective values for the nomogram predicting invasiveness were 0·736, 0·891 and 0·788. CONCLUSIONS:External validation of the nomogram showed good performance in predicting cancer in both Eastern and Western patients with IPMN lesions.

INTRODUCTION/BACKGROUND:Two developing forces have achieved prominence in medical education: the advent of competency-based assessments and a growing commitment to expand access to medicine for a broader range of learners with a wider array of preparation. Remediation is intended to support all learners to achieve sufficient competence. Therefore, it is timely to provide practical guidelines for remediation in medical education that clarify best practices, practices to avoid, and areas requiring further research, in order to guide work with both individual struggling learners and development of training program policies. METHODS:Collectively, we generated an initial list of Do's, Don'ts, and Don't Knows for remediation in medical education, which was then iteratively refined through discussions and additional evidence-gathering. The final guidelines were then graded for the strength of the evidence by consensus. RESULTS:We present 26 guidelines: two groupings of Do's (systems-level interventions and recommendations for individual learners), along with short lists of Don'ts and Don't Knows, and our interpretation of the strength of current evidence for each guideline. CONCLUSIONS:Remediation is a high-stakes, highly complex process involving learners, faculty, systems, and societal factors. Our synthesis resulted in a list of guidelines that summarize the current state of educational theory and empirical evidence that can improve remediation processes at individual and institutional levels. Important unanswered questions remain; ongoing research can further improve remediation practices to ensure the appropriate support for learners, institutions, and society.

Curricular revision efforts have resulted in learner-centered programs that value content integration and active learning. Yet, less attention has been placed on assessment methods that are learner-centered and promote assessment for learning. The use of context rich short answer question (CR-SAQ) exams in the preclinical years of medical school was evaluated to determine if this format aligns with the criteria for assessment for learning. Medical students and preclinical faculty members were sent a survey comprised of closed and open-ended questions about their experience using CR-SAQ exams. Data were analyzed using a mixed-method design. Open-ended responses were evaluated using thematic analysis within the framework of criteria for assessment for learning. A total of 274 students (94%) and 24 faculty (75%) completed the survey. Fifty four percent of students reported preferring a CR-SAQ exam format over multiple choice questions (MCQ) format. Quantitative data and qualitative comments by students supported that CR-SAQ exams aligned with criteria for assessment for learning, including acceptability, authenticity, educational effect, and the cueing effect. Student concerns included preparation for USMLE Step 1 exam, as well as the validity and reproducibility of CR-SAQ assessments. Faculty largely agreed with the benefits of the CR-SAQ, but were concerned about feasibility, acceptability and reproducibility. The CR-SAQ exam format assessment strategy supports assessment for learning in an undergraduate medical education setting. Both benefits and drawbacks of this method are presented, however students and faculty describe a broader impact that this assessment method has on their development as a physician.

OBJECTIVE:To determine if World Trade Center (WTC) exposure is associated with hearing loss. METHODS:Logistic regression to evaluate the immediate impact of WTC exposure and parametric survival analysis to assess longitudinal outcomes. RESULTS:Those arriving on the morning of 9/11/2001 had elevated odds of low-frequency (odds ratio [OR]: 1.24;95%CI:1.04-1.47) and high-frequency (OR:1.16;95%CI:1.02-1.31) hearing loss at their first post-9/11/2001 exam. Longitudinally, participants arriving before 9/13/2001 and spending ≥6 months at the WTC-site had greater risk of hearing loss in the low frequencies (risk ratio [RR]:1.31;95%CI:1.05-1.60) and high frequencies (RR:1.37;95%CI:1.22-1.54). By 2016, 3,194 (37%) had abnormal hearing sensitivity in either ear and 1,751 (20%) in both ears. CONCLUSIONS:More heavily WTC-exposed workers were at increased risk of hearing loss, and group differences persisted for at least 15 years. Those with abnormal hearing sensitivity may benefit from interventions such as hearing aids and other rehabilitation.

BACKGROUND:Conflicting reports exist regarding the racial and the gender distribution of rheumatoid arthritis-related interstitial lung disease (RA-ILD). In a major population study of predominately Whites, RA-ILD was reported mainly among smoker middle-aged men. However, recent data suggest that the disease is that of elderly women. Our study aimed to assess the prevalence and identify the gender differences and clinical characteristics of RA-ILD in a predominantly Black population. METHODS:Cross-sectional analysis of data obtained from the records of 1142 patients with RA diagnosis by ICD codes of which 503 cases met the inclusion criteria for the study. Eighty-six patients had chronic respiratory symptoms of cough and dyspnea and were further assessed by our multidisciplinary group of investigators. Thirty-two subjects with an established diagnosis of rheumatoid arthritis met the diagnostic criteria for interstitial lung disease. RESULTS:). Usual interstitial pneumonia (UIP) was found in 24/32 (75%) of the cases. Seventy-two percent of the RA-ILD patient had seropositive RA. Smoking history was reported in 31.3% of the cohort, gastroesophageal reflux disease (GERD) in 32.3%, and cardiovascular disease (CVD) risk factors in 65.6%. CONCLUSION/CONCLUSIONS:Our study indicates RA-ILD among Blacks is predominantly a disease of elderly females with higher rates of GERD and CVD risk factors. Further studies are needed to identify the pathogenetic differences accounting for the gender distribution of RA-ILD among Black and White populations.Key Points• First study to assess ILD among predominantly Black RA patients.• The prevalence of RA-associated ILD was 6.36%, affecting mostly women in their sixth decade with seropositive disease.• COPD was the most common airway disease among non-RA-ILD Black population.• GERD was found in approximately one-third of patients with RA-associated ILD versus one-fifth of those RA patients without any lung disease.

BACKGROUND:Coronary artery calcium (CAC) has been shown in multiple populations to predict atherosclerotic cardiovascular disease. However, its predictive value in Asian-Americans is poorly described. PATIENTS AND METHODS/METHODS:We studied 1621 asymptomatic Asian-Americans in the CAC Consortium, a large multicenter retrospective cohort. CAC was modeled in categorical (CAC = 0; CAC = 1-99; CAC = 100-399; CAC ≥ 400) and continuous [ln (CAC + 1)] forms. Participants were followed over a mean follow-up of 12 ± 4 years for coronary heart disease (CHD) death, cardiovascular disease (CVD) death, and all-cause mortality. The predictive value of CAC for individual outcomes was assessed using multivariable-adjusted Cox regression models adjusted for traditional cardiovascular risk factors and reported as hazard ratios (95% confidence interval). RESULTS:The mean (SD) age of the population was 54 (11.2) years and 64% were men. The mean 10-year atherosclerotic cardiovascular disease risk score was 8%. Approximately half had a CAC score of 0, whereas 22.5% had a CAC score of greater than 100. A total of 56 deaths (16 CVD and 8 CHD) were recorded, with no CVD or CHD deaths in the CAC = 0 group. We noted a significantly increased risk of CHD [hazard ratio (HR): 2.6 (1.5-4.3)] and CVD [HR: 2.3 (1.8-2.9)] mortality per unit increase in In (CAC + 1). Compared to those with CAC scores of 0, individuals with CAC scores of at least 400 had over a three-fold increased risk of all-cause mortality [HR: 3.3 (1.3-8.6)]. CONCLUSION/CONCLUSIONS:Although Asian-Americans are a relatively low-risk group, CAC strongly predicts CHD, CVD, and all-cause mortality beyond traditional risk factors. These findings may help address existing knowledge gaps in CVD risk prediction in Asian-Americans.

Minimal residual disease (MRD) tracking by next generation sequencing of immunoglobulin sequences is moving towards clinical implementation in multiple myeloma. However, there is only sparse information available to address whether clonal sequences remain stable for tracking over time, and to what extent light chain sequences are sufficiently unique for tracking. Here, we analyzed immunoglobulin repertoires from 905 plasma cell myeloma and healthy control samples, focusing on the third complementarity determining region (CDR3). Clonal heavy and/or light chain expression was identified in all patients at baseline, with one or more subclones related to the main clone in 3.2 %. In 45 patients with 101 sequential samples, the dominant clonal CDR3 sequences remained identical over time despite differential clonal evolution by whole exome sequencing in 49 % of patients. The low frequency of subclonal CDR3 variants and absence of evolution over time in active multiple myeloma indicates that tumor cells at this stage are not under selective pressure to undergo antibody affinity maturation. Next, we establish somatic hypermutation and non-templated insertions as the most important determinants of light chain clonal uniqueness, identifying a potentially trackable sequence in the majority of patients. Taken together, we show that dominant clonal sequences identified at baseline are reliable biomarkers for long-term tracking of the malignant clone, including both IGH and the majority of light chain clones. This article is protected by copyright. All rights reserved.

BACKGROUND:Gastrointestinal symptoms are prevalent in patients with cirrhosis. Cirrhotic patients have a known predilection to delayed gastric emptying compared to those without cirrhosis. However, the contributing factors have not been fully elucidated. Retained gastric food on esophagogastroduodenoscopy (EGD) has been used as a surrogate marker for delayed gastric emptying with reasonably high specificity. Therefore, we hypothesize that the frequency of retained gastric food contents at EGD will be higher in a cirrhotic population compared to a control population without liver disease. Additionally, we hypothesize that increased frequency of gastric food contents will be associated with increased severity of cirrhosis. AIM/OBJECTIVE:To determine the relative frequency of delayed gastric emptying among cirrhotics as compared to non-cirrhotics and to identify associated factors. METHODS:We performed a retrospective case-control study of cirrhotic subjects who underwent EGD at an academic medical center between 2000 and 2015. Three hundred sixty-four patients with confirmed cirrhosis, who underwent a total of 1044 EGDs for the indication of esophageal variceal screening or surveillance, were identified. During the same period, 519 control patients without liver disease, who underwent a total of 881 EGDs for the indication of anemia, were identified. The presence of retained food on EGD was used as a surrogate for delayed gastric emptying. The relative frequency of delayed gastric emptying among cirrhotics was compared to non-cirrhotics. Characteristics of patients with and without retained food on EGD were compared using univariable and multivariable logistic regression analysis to identify associated factors. RESULTS:0.01) were also associated with a higher likelihood of food retention on EGD. CONCLUSION/CONCLUSIONS:Cirrhotics have a higher frequency of retained food at EGD than non-cirrhotics. Decompensated cirrhosis, defined by Child-Pugh class C, is associated with a higher likelihood of delayed gastric emptying.