Medicine and Health Sciences Commons^™

Enpp1 Variants In Patients With Gaci And Pxe Expand The Clinical And Genetic Heterogeneity Of Heritable Disorders Of Ectopic Calcification., Douglas Ralph, Yvonne Nitschke, Michael A Levine, Matthew Caffet, Tamara Wurst, Amir Hossein Saeidian, Leila Youssefian, Hassan Vahidnezhad, Sharon F Terry, Frank Rutsch, Jouni Uitto, Qiaoli Li

Department of Medicine Faculty Papers

Pseudoxanthoma elasticum (PXE) and generalized arterial calcification of infancy (GACI) are clinically distinct genetic entities of ectopic calcification associated with differentially reduced circulating levels of inorganic pyrophosphate (PPi), a potent endogenous inhibitor of calcification. Variants in ENPP1, the gene mutated in GACI, have not been associated with classic PXE. Here we report the clinical, laboratory, and molecular evaluations of ten GACI and two PXE patients from five and two unrelated families registered in GACI Global and PXE International databases, respectively. All patients were found to carry biallelic variants in ENPP1. Among ten ENPP1 variants, one homozygous variant demonstrated uniparental disomy …

Angiotensin Converting Enzyme Inhibitors And Angiotensin Receptor Blockers In Acute Heart Failure: Invasive Hemodynamic Parameters And Clinical Outcomes., Kevin Bryan Lo, Hesam Mostafavi Toroghi, Grace Salacup, Jiahui Jiang, Ruchika Bhargav, Eduardo Quintero, Kira Balestrini, Anum Shahzad, Roy O Mathew, Peter A Mccullough, Janani Rangaswami

Department of Medicine Faculty Papers

There are limited data regarding the use of angiotensin converting enzyme inhibitors/angiotensin receptor blockers (ACEi/ARBs) in acute heart failure (AHF). The purpose is to determine the patterns of ACEi/ARB use at the time of admission and discharge in relation to invasive hemodynamic data, mortality, and heart failure (HF) readmissions. This is a retrospective single-center study in patients with AHF who underwent right heart catheterization between January 2010 and December 2016. Patients on dialysis, evidence of shock, or incomplete follow up were excluded. Multivariate logistic regression analysis was used to analyze the factors associated with continuation of ACEi/ARB use on discharge …

Effects Of Combining Meditation Techniques On Short-Term Memory, Attention, And Affect In Healthy College Students., Samani Unnata Pragya, Neelam D Mehta, Bassam Abomoelak, Parvin Uddin, Pushya Veeramachaneni, Naina Mehta, Stephanie Moore, Melissa Jean-Francois, Stephanie Garcia, Samani Chaitanya Pragya, Devendra I Mehta

Department of Medicine Faculty Papers

Meditation refers to a family of self-regulation practices that focuses on training attention and awareness to foster psycho-emotional well-being and to develop specific capacities such as calmness, clarity, and concentration. We report a prospective convenience-controlled study in which we analyzed the effect of two components of Preksha Dhyāna – buzzing bee sound meditation (Mahapran dhvani) and color meditation (leśyā dhyāna) on healthy college students. Mahapran and leśya dhyāna are two Preksha Dhyāna practices that are based on sound and green color, respectively. The study population represents a suitable target as college students experience different stress factors during the school year. …

Cytokine Storms, Evolution And Covid-19, Joe Alcock, Alix Masters

Department of Medicine Faculty Papers

Since the identification of severe illness caused by the novel coronavirus SARS-CoV-2, the role of the host immune system in causing disease has attracted widespread attention, along with intense interest in medical interventions that target the host immune response. A wide variety of agents have been proposed to treat a cytokine storm in coronavirus disease 2019 (COVID-19), but so far, only one class of medications, corticosteroids, has proved useful. In recent decades, experimental therapies for cytokine storms have been tried and mostly failed to help patients with severe sepsis and other infections. We summarize this history in order to frame …

Post-Partum Skin-To-Skin Care And Infant Safety: Results Of A State-Wide Hospital Survey, Eileen Tyrala, Md, Michael H. Goodstein, Md, Erich Batra, Md, Barbara Kelly, Md, Judy Bannon, Mba, Ted Bell, Ms

Department of Medicine Faculty Papers

Objectives: Survey current experience with Skin to Skin care (SSC) in Pennsylvania Maternity Centers.

Study Design: The nursing director of each Maternity Center in PA (n = 95) was sent an on-line confidential survey querying SSC practices. Responses were compared by delivery size, location, and nature of affiliation. Statistics analyzed by chi-square and student t-test.

Results: Of these 64/95 MCs (67%) responded. All allowed SSC after vaginal deliveries, 55% after C-section, 73% mother’s room. Monitoring included delivery room nurse (94%) with support from other providers (61%), family members (37%), and electronic monitoring (5%). If SSC occurred in mother’s …

Biomolecules As Model Indicators Of In Vitro And In Vivo Cold Plasma Safety, Caitlin Heslin, Daniela Boehm, Julianne Megaw, Brendan F. Gilmore, Theresa A. Freeman, Noreen J. Hickok, P. J. Cullen, Paula Bourke

Department of Medicine Faculty Papers

No abstract provided.

Efficacy And Safety Of Bempedoic Acid For Prevention Of Cardiovascular Events And Diabetes: A Systematic Review And Meta-Analysis., Xing Wang, Yu Zhang, Huiwen Tan, Peng Wang, Xi Zha, Weelic Chong, Liangxue Zhou, Fang Fang

Department of Medicine Faculty Papers

BACKGROUND: Bempedoic acid is an oral, once-daily, first-in-class drug being developed for the treatment of hyperlipidemia. However, evidence of bempedoic acid use for the prevention of cardiovascular events and diabetes is lacking. Thus, we aim to evaluate the benefit and safety of bempedoic acid use for the prevention of cardiovascular events and diabetes.

METHODS: We searched Medline, Embase, and the Cochrane Central Register of Controlled Trials with no language restriction from inception until March 3, 2020. Pairs of reviewers independently identified randomized controlled trials comparing the use of bempedoic acid with placebo or no treatment for primary prevention of cardiovascular …

Fruit And Vegetable Consumption Is Associated With Lower Prevalence Of Asymptomatic Diverticulosis: A Cross-Sectional Colonoscopy-Based Study., Benjamin Maxner, Jessica Mcgoldrick, Danielle Bellavance, Po-Hong Liu, Ramnik J Xavier, Joseph C Yarze, Rocco Ricciardi, Kyle Staller, Daniel C Chung, Hamed Khalili

Department of Medicine Faculty Papers

BACKGROUND: Previous studies of the relationship between dietary factors and risk of diverticulosis have yielded inconsistent results. We therefore sought to investigate the association between consumption of fruit and vegetables and prevalent diverticulosis.

METHODS: Our study population included participants in the Gastrointestinal Disease and Endoscopy Registry (GIDER), a colonoscopy-based longitudinal cohort at the Massachusetts General Hospital, who provided comprehensive information on dietary intake and lifestyle factors using validated questionnaires prior to colonoscopy. Information on presence and location of diverticula was obtained from the endoscopist at the end of each procedure. We used Poisson regression modeling to calculate the prevalence ratios …

A Foldable Nonpenetrating Keratoprosthesis: Medium To Long-Term Clinical Results In Patients With Corneal Blindness Due To Noninflammatory Anterior Cornea Disease., Jose M Vargas, Eric Shiuey

Department of Medicine Faculty Papers

PURPOSE: To report the medium to long-term safety and performance outcomes of the KeraKlear nonpenetrating artificial cornea (KeraKlear) as the primary procedure in patients with corneal blindness due to noninflammatory anterior cornea disease.

METHODS: Fifteen patients with corneal blindness (preoperative visual acuity [VA] of ≥20/200) due to a non-inflammatory anterior corneal condition were included in this prospective, single-center study. Preoperative diagnoses included corneal scars, keratoconus, and corneal dystrophies. Diseased corneas were implanted with the KeraKlear (KeraMed Inc., Irvine, California, USA) by a single surgeon (JMV) using a femtosecond laser to create all incisions. Participants were followed up with for as …

Evaluating The Relationship Between Participation In Student-Run Free Clinics And Changes In Empathy In Medical Students., Anita Modi, Michele Fascelli, Zachary Daitch, Mohammadreza Hojat Professor

Department of Medicine Faculty Papers

PURPOSE: We explored differences in changes in medical student empathy in the third year of medical school between volunteers at JeffHOPE, a multisite medical student-run free clinic of Sidney Kimmel Medical College (SKMC), and nonvolunteers.

METHOD: Volunteerism and leadership experience at JeffHOPE were documented for medical students in the Class of 2015 (n = 272) across their medical educations. Students completed the Jefferson Scale of Empathy at the beginning of medical school and at the end of the third year. Students who reported participation in other Jefferson-affiliated clinics (n = 44) were excluded from this study. Complete data were available …

Postoperative Intussusception In 10-Year-Old Presenting As Decreased Intestinal Motility, Arielle Spellun, Loren Berman, Stephen Murphy

Department of Medicine Faculty Papers

No abstract provided.

A 22-Year-Old Man With Pleural Tuberculosis Associated Hydropneumothorax: Case Report And Literature Review., Lauren A Sharan, Thea Price, Md, Boyd Hehn, David Manoff, Md, Scott W. Cowan, Md

Department of Medicine Faculty Papers

A 22-year-old Asian male presented with fever, non-productive cough, right-sided pleuritic chest pain and was found to have a large right hydropneumothorax. A chest tube was placed. Pleural fluid analysis revealed a lymphocytic predominant exudate and he was subsequently started on four-drug daily anti-tuberculosis therapy (isoniazid, ethambutol, rifampin, pyrazinamide). Pleural biopsy revealed acid-fast bacilli. Given his persistent pleural effusion, he was given four doses of intrapleural tissue plasminogen activator (tPA) and dornase alpha (DNase) via his chest tube over a period of 6 days resulting in clinical and radiologic improvement. Pleural biopsy and pleural fluid culture specimens later revealed Mycobacterium …

Cannibalism, Kuru, And Mad Cows: Prion Disease As A "Choose-Your-Own-Experiment" Case Study To Simulate Scientific Inquiry In Large Lectures., Antonio Serrano, Jeffrey Liebner, Justin K Hines

Department of Medicine Faculty Papers

Despite significant efforts to reform undergraduate science education, students often perform worse on assessments of perceptions of science after introductory courses, demonstrating a need for new educational interventions to reverse this trend. To address this need, we created An Inexplicable Disease, an engaging, active-learning case study that is unusual because it aims to simulate scientific inquiry by allowing students to iteratively investigate the Kuru epidemic of 1957 in a choose-your-own-experiment format in large lectures. The case emphasizes the importance of specialization and communication in science and is broadly applicable to courses of any size and sub-discipline of the life sciences.

Anxiety And Depression In Patients With Advanced Macular Degeneration: Current Perspectives., Verena R Cimarolli, Robin J Casten, Barry W. Rovner, Vera Heyl, Silvia Sörensen, Amy Horowitz

Department of Medicine Faculty Papers

Age-related macular degeneration (AMD) - despite advances in prevention and medical treatment options - remains prevalent among older adults, often resulting in functional losses that negatively affect the mental health of older adults. In particular, the prevalence of both anxiety and depression in patients with AMD is high. Along with medical treatment options, low vision rehabilitation and AMD-specific behavioral and self-management programs have been developed and have demonstrated effectiveness in improving the mental health of AMD patients. This article reviews the prevalence of anxiety and depression in patients with advanced AMD, discusses potential mechanisms accounting for the development of depression …

The Effect Of Aspirin On Bleeding And Transfusion In Contemporary Cardiac Surgery., Jordan E Goldhammer, Gregary D. Marhefka, Constantine Daskalakis, Mark W Berguson, John E Bowen, James Diehl, Jianzhong Sun

Department of Medicine Faculty Papers

OBJECTIVE: Despite evidence that preoperative aspirin improves outcomes in cardiac surgery, recommendations for aspirin use are inconsistent due to aspirin's anti-platelet effect and concern for bleeding. The purpose of this study was to investigate preoperative aspirin use and its effect on bleeding and transfusion in cardiac surgery.

METHODS: This retrospective study involved consecutive patients (n=1571) who underwent CABG, valve, or combined CABG and valve surgery at a single center between March 2007 and July 2012. Of all patients, 728 met the inclusion criteria and were divided into two groups: those using (n=603) or not using (n=125) aspirin within 5 days …