Summary Defects in telomere maintenance genes cause pathological telomere shortening, and manifest in syndromes which have prominent phenotypes in tissues of high turnover: the skin and bone marrow. Because the gastrointestinal (GI) epithelium has rapid turnover, we sought to determine whether telomere syndromes cause GI disease, and to define its prevalence, spectrum and natural history. We queried subjects in the Johns Hopkins Telomere Syndrome Registry for evidence of luminal GI disease. In sixteen percent of Registry subjects (6 of 38), there was a history of significant GI pathology, and 43 additional cases were identified in the literature. Esophageal stenosis, enteropathy and enterocolitis were the recurrent findings. In the intestinal mucosa, there was striking villous atrophy, extensive apoptosis, and anaphase bridging pointing to regenerative defects in the epithelial compartment. GI disease was often the first and most severe manifestation of telomere disease in young children. These findings indicate that telomere dysfunction disrupts the epithelial integrity in the human GI tract manifesting in recognizable disease processes. A high index of suspicion should facilitate diagnosis and management.
Providers generally focused on perceived patient-level barriers to HCV treatment initiation and adherence, as well as provider-level facilitators; PCPs additionally noted patient preferences and system-level issues that guide decision making regarding treatment initiation. While HPs focused almost exclusively on provider-level facilitators, PCPs additionally focused on patient-level facilitators of treatment. These data provide novel insights and suggest focusing on patient, provider, and system-level strategies to further improve HCV treatment initiation and adherence.
Depression after liver transplantation has been associated with decreased survival, but the effects of pre-transplant depression on early and late post-transplant outcomes remain incompletely evaluated. We assessed all patients who had undergone single-organ liver transplantation at a single center over the prior 10 years. A diagnosis of pre-transplant depression, covariates, and the outcomes of interest were extracted from the electronic medical record. Potential covariates included demographics, etiology and severity of liver disease, comorbidities, donor age, graft type, immunosuppression, and ischemic times. In multivariable models adjusting for these factors, we evaluated the effect of pre-transplant depression on transplant length of stay (LOS), discharge disposition (home vs. facility) and long-term survival. Among 1115 transplant recipients with a median follow-up time of 5 years, the average age was 56±11 and MELD was 12±9. Nineteen percent of the study population had a history of pre-transplant depression. Pre-transplant depression was associated with longer LOS (median = 19 vs. 14 days, IRR = 1.25, CI = 1.13,1.39), discharge to a facility (36% vs. 25%, OR 1.70,CI = 1.18,2.45), and decreased survival (HR = 1.54,CI = 1.14,2.08) in this cohort, accounting for other potential confounders. In conclusion, pre-transplant depression was significantly associated with longer transplant length of stay, discharge to a facility, and mortality in this cohort.
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In the United States, race-based disparities in cardiovascular disease care have proven to be pervasive, deadly, and expensive. African American/Black, Hispanic/Latinx, and Native/Indigenous American individuals are at an increased risk of cardiovascular disease and are less likely to receive high-quality, evidence-based medical care as compared with their White American counterparts. Although the United States population is diverse, the cardiovascular workforce that provides its much-needed care lacks diversity. The available data show that care provided by physicians from racially diverse backgrounds is associated with better quality, both for minoritized patients and for majority patients. Not only is cardiovascular workforce diversity associated with improvements in health care quality, but racial diversity among academic teams and research scientists is linked with research quality. We outline documented barriers to achieving workforce diversity and suggest evidence-based strategies to overcome these barriers. Key strategies to enhance racial diversity in cardiology include improving recruitment and retention of racially diverse members of the cardiology workforce and focusing on cardiovascular health equity for patients. This review draws attention to academic institutions, but the implications should be considered relevant for nonacademic and community settings as well.
IMPORTANCEBlack faculty and trainees remain underrepresented in academic medicine because of systemic racism. Years of diversity and inclusion efforts have not succeeded in eliminating the unique challenges faced by Black faculty in academic medicine. OBJECTIVES To elicit expert faculty perspectives on anti-Black racism in academic medicine based on lived and/or professional experience and to solicit recommendations for an intervention for faculty to dismantle anti-Black racism within academic medical centers. DESIGN, SETTING, AND PARTICIPANTS This qualitative study included semistructured interviews with experts in understanding and dismantling anti-Black racism within academic medical centers. Participants had expertise in anti-Black racism through their lived experience as a Black faculty member and/or professional experience in diversity, equity, and inclusion efforts. Participants were recruited from academic medical centers from around the United States. Interviews were conducted through an online meeting platform, audio recorded, transcribed verbatim, and subsequently coded following an inductive qualitative description approach. Interviews were completed between October 2020 and March 2021. MAIN OUTCOMES AND MEASURES Outcomes include the experiences of Black faculty and trainees in academic medicine and recommendations for developing an intervention to dismantle anti-Black racism within academic medicine. RESULTS A total of 16 participants completed this study; most identified as Black or African American (9 [56%]) and female (10 [63%]). Results were sorted into 2 content domains, with several themes within those domains: (1) barriers faced by Black faculty and trainees and potential solutions and (2) recommendations for an intervention directed at faculty to dismantle anti-Black racism in academic medicine. Barriers faced by Black faculty and trainees included lack of representation; challenges with the recruitment, retention, and promotion of Black faculty; and experiences of microaggressions and overt racism. Participants suggested that an intervention should have a comprehensive learning objective; be mandatory for all faculty, with the exception of Black faculty; draw from outside expertise; and receive allocation of resources and funding equal to other important training modules. CONCLUSIONS AND RELEVANCE The findings of this study affirm prior work about the unique challenges faced by Black faculty and trainees in academic medicine because of interpersonal and institutional racism and build on this prior work by soliciting recommendations to guide intervention development. An intervention to dismantle anti-Black racism within academic medicine is urgently needed and will require leadership buy-in and financial commitments from institutions for effective development and implementation.
The aim of this study was to assess the independent association between pre-transplant prescription opioid use and readmission following liver transplantation. We reviewed the medical records of all patients at a single medical center undergoing primary, single-organ, liver transplantation from 2004 to 2014. We assessed factors associated with hospital readmission 30 days and 1 year after hospital discharge using multivariable competing risk regression models. Among 1056 transplant recipients, 49 (4.6%) were prescribed pre-transplant prescription opioids. Readmission occurred in 421 (40%) patients within 30 days and 689 (65%) within 1 year. Patients with pre-transplant opioid use had a significantly higher risk of readmission at 30 days (HR 1.7; 95% CI 1.1-2.5) and a non-significantly elevated risk at 1 year (HR 1.4; 95% CI 1.0-1.9) when controlling for other potential confounders. Although pain was the major reason for readmission in only 12 (3%) patients at 30 days and 33 (6%) patients at 1 year, pre-transplant opioid use was significantly associated with pain-related readmission at both time points. In conclusion, prescription opioid use pre-transplantation was significantly associated with all-cause 30-day readmissions and pain-related readmissions at 30 days and 1 year.
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