Medical Specialties Commons^™

An Atypical Presentation Of A Rare Disease, Elizabeth Schoenberg, Kathleen P. Mcguinn, Deirdre Connolly, Sherry Yang

Department of Dermatology and Cutaneous Biology Faculty Papers

A 76-year-old white woman presented for evaluation of asymptomatic skin lesions on her right shin, right buttock, and left arm. All lesions initially underwent slow growth and plateaued and then remained stable in size. A complete review of systems revealed normal results. She had 3 well-demarcated erythematous round plaques ranging from 1.5 to 3 cm, all with a central depression, yellow hue, and prominent telangiectasias (Figs 1 and 2). An excisional biopsy was performed. Histologically, there were palisading granulomas within the papillary and reticular dermis, predominantly composed of a histiocytic cell population with multiple large giant cells (S100^-; …

Stressed Out With Sepsis., Robert Hilton, Michael Baram

Division of Pulmonary, Allergy, and Critical Care Medicine Faculty Papers

No abstract provided.

Survival Probability In Patients With Sickle Cell Anemia Using The Competitive Risk Statistical Model., Emilia Matos Do Nascimento, Clarisse Lopes De Castro Lobo, Basilio De Bragança Pereira, Samir K. Ballas

Cardeza Foundation for Hematologic Research

The clinical picture of patients with sickle cell anemia (SCA) is associated with several complications some of which could be fatal. The objective of this study is to analyze the causes of death and the effect of sex and age on survival of Brazilian patients with SCA. Data of patients with SCA who were seen and followed at HEMORIO for 15 years were retrospectively collected and analyzed. Statistical modeling was performed using survival analysis in the presence of competing risks estimating the covariate effects on a sub-distribution hazard function. Eight models were implemented, one for each cause of death. The …

Single-Subject Designs And Practice-Based Research In Palliative Care: A Letter To The Editor., James Gerhart, Dennis J. Hand, Michael Hoerger, Sean O'Mahony

Department of Obstetrics and Gynecology Faculty Papers

No abstract provided.

Improving The Quality Of Patient-Provider Interactions And Promoting Equitable, Accessible And Patient-Centered Cancer Care, C. Vaughan-Briggs, Lcsw, A. Petok, Lcsw, Mph, T. Dejesse, Amy Leader, Drph, Mph

Department of Medical Oncology Posters

• Philadelphia is among the top 10 cities in the US with populations over 500,000 with a high Community Need Index score (CNI- severity of Health disparity)
• Data from 2015 County Health Ranking, had Philadelphia last of all 67 counties in the state for social economic factors. (unemployment, income inequality, injury deaths, etc. )
• Cancer is a leading cause of mortality in the US
• The SKCC participated in a year-long, externally supported self study to improve the quality of patientprovider interactions and promote more equitable, accessible and patient- centered cancer care. This program aligned with internal initiatives.
• SKCC at Jefferson is …

Ultrasound-Triggered Antibiotic Release From Peek Clips To Prevent Spinal Fusion Infection: Initial Evaluations., Lauren J. Delaney, Daniel Macdonald, Jay Leung, Keith Fitzgerald, Alex M. Sevit, John R. Eisenbrey, Neil Patel, Flemming Forsberg, Christopher K. Kepler, Taolin Fang, Steven M. Kurtz, Noreen J. Hickok

Department of Orthopaedic Surgery Faculty Papers

Despite aggressive peri-operative antibiotic treatments, up to 10% of patients undergoing instrumented spinal surgery develop an infection. Like most implant-associated infections, spinal infections persist through colonization and biofilm formation on spinal instrumentation, which can include metal screws and rods for fixation and an intervertebral cage commonly comprised of polyether ether ketone (PEEK). We have designed a PEEK antibiotic reservoir that would clip to the metal fixation rod and that would achieve slow antibiotic release over several days, followed by a bolus release of antibiotics triggered by ultrasound (US) rupture of a reservoir membrane. We have found using human physiological fluid …

Genetic Susceptibility To Alopecia., Jouni Uitto

Department of Dermatology and Cutaneous Biology Faculty Papers

No abstract provided.

Osteoblasts Are "Educated" By Crosstalk With Metastatic Breast Cancer Cells In The Bone Tumor Microenvironment., Alexus D. Kolb, Alison B. Shupp, Dimpi Mukhopadhyay, Frank C. Marini, Karen M. Bussard

Department of Cancer Biology Faculty Papers

INTRODUCTION: In a cancer-free environment in the adult, the skeleton continuously undergoes remodeling. Bone-resorbing osteoclasts excavate erosion cavities, and bone-depositing osteoblasts synthesize osteoid matrix that forms new bone, with no net bone gain or loss. When metastatic breast cancer cells invade the bone, this balance is disrupted. Patients with bone metastatic breast cancer frequently suffer from osteolytic bone lesions that elicit severe bone pain and fractures. Bisphosphonate treatments are not curative. Under ideal circumstances, osteoblasts would synthesize new matrix to fill in erosion cavities caused by osteoclasts, but this is not what occurs. Our prior evidence demonstrated that osteoblasts are …

Guidelines Of The International Headache Society For Controlled Trials Of Acute Treatment Of Migraine Attacks In Adults: Fourth Edition., Hans-Christoph Diener, Cristina Tassorelli, David W. Dodick, Stephen Silberstein, Richard B. Lipton, Messoud Ashina, Werner J. Becker, Michel D. Ferrari, Peter J. Goadsby, Patricia Pozo-Rosich, Shuu-Jiun Wang, Jay Mandrekar

Department of Neurology Faculty Papers

The quality of clinical trials is an essential part of the evidence base for the treatment of headache disorders. In 1991, the International Headache Society Clinical Trials Standing Committee developed and published the first edition of the Guidelines for controlled trials of drugs in migraine. Scientific and clinical developments in headache medicine led to second and third editions in 2000 and 2012, respectively. The current, fourth edition of the Guidelines retains the structure and much content from previous editions. However, it also incorporates evidence from clinical trials published after the third edition as well as feedback from meetings with regulators, …

Maternal And Gestational Influences On Childhood Blood Pressure., Bonita Falkner

Department of Medicine Faculty Papers

Exposures that contribute to a sub-optimal intrauterine environment can have an effect on the developing fetus. Impaired fetal growth that results in low birth weight is an established risk factor for cardio-metabolic disorders later in life. Recent epidemiologic and prospective cohort studies that include the maternal and gestational period have identified maternal and gestational conditions that confer increased risk for subsequent cardio-metabolic disorders in the absence of low birth weight. Maternal pre-conception health status, including chronic obesity and type 2 diabetes, increase risk for childhood obesity and obesity-related higher blood pressure (BP) in child offspring. Maternal gestational exposures, including gestational …

Development And Validation Of The Fsiq-Rms: A New Patient-Reported Questionnaire To Assess Symptoms And Impacts Of Fatigue In Relapsing Multiple Sclerosis, Stacie Hudgens, René Schüler, Jonathan Stokes, Sonya Eremenco, Elke Hunsche, Thomas P. Leist

Department of Neurology Faculty Papers

Objectives: A new patient-reported outcome (PRO) instrument to measure fatigue symptoms and impacts in relapsing multiple sclerosis (RMS) was developed in a qualitative stage, followed by psychometric validation and migration from paper to an electronic format.

Methods: Adult patients with relapsing-remitting multiple sclerosis (RRMS) were interviewed to elicit fatigue-related symptoms and impacts. A draft questionnaire was debriefed in cognitive interviews with further RRMS patients, and revised. Content confirmation interviews were conducted with patients with progressive-relapsing multiple sclerosis (PRMS) and relapsing secondary-progressive multiple sclerosis (RSPMS). Psychometric analyses used data from adult patients with different RMS subtypes and matched non-RMS controls in …

A Closer Look At Arsa Activity In A Patient With Metachromatic Leukodystrophy., Kathleen Doherty, S. Barron Frazier, Matthew Clark, Anna Childers, Sumit Pruthi, David A. Wenger, Jessica Duis

Department of Neurology Faculty Papers

Metachromatic leukodystrophy (MLD) is an autosomal recessive lysosomal storage disease mainly caused by a deficiency of arylsulfatase A activity. The typical clinical course of patients with the late infantile form includes a regression in motor skills with progression to dysphagia, seizures, hypotonia and death. We present a case of a 4-year-old female with rapidly progressive developmental regression with loss of motor milestones, spasticity and dysphagia. MRI showed volume loss and markedly abnormal deep white matter. Enzymatic testing in one laboratory showed arylsulfatase A activity in their normal range. However, extraction of urine showed a large increase in sulfatide excretion in …

Early Progression Of Krabbe Disease In Patients With Symptom Onset Between 0 And 5 Months., Maria L. Beltran-Quintero, Nicholas A. Bascou, Michele D. Poe, David A. Wenger, Carlos A. Saavedra-Matiz, Matthew J. Nichols, Maria L. Escolar

Department of Neurology Faculty Papers

BACKGROUND: Krabbe disease is a rare neurological disorder caused by a deficiency in the lysosomal enzyme, β-galactocerebrosidase, resulting in demyelination of the central and peripheral nervous systems. If left without treatment, Krabbe disease results in progressive neurodegeneration with reduced quality of life and early death. The purpose of this prospective study was to describe the natural progression of early onset Krabbe disease in a large cohort of patients.

METHODS: Patients with early onset Krabbe disease were prospectively evaluated between 1999 and 2018. Data sources included diagnostic testing, parent questionnaires, standardized multidisciplinary neurodevelopmental assessments, and neuroradiological and neurophysiological tests.

RESULTS: We …

A Novel Handheld Robotic-Assisted System For Unicompartmental Knee Arthroplasty: Surgical Technique And Early Survivorship., Andrew K. Battenberg, Nathan A. Netravali, Jess H. Lonner

Department of Orthopaedic Surgery Faculty Papers

Technology, including robotics, has been developed for use in unicompartmental knee arthroplasty (UKA) to improve accuracy and precision of bone preparation, implant positioning, and soft tissue balance. The NAVIO™ System (Smith and Nephew, Pittsburgh, PA, United States) is a handheld robotic system that assists surgeons in planning implant positioning based on an individual patient's anatomy and then preparing the bone surface to accurately achieve the plan. The surgical technique is presented herein. In addition, initial results are presented for 128 patients (mean age 64.7 years; 57.8% male) undergoing UKA with NAVIO. After a mean of follow-up period of 2.3 years, …

Evaluation Of Cranial Base Repair Techniques Utilizing A Novel Cadaveric Cpap Model., Chandala Chitguppi, Ryan A. Rimmer, Hermes G. Garcia, Ian J. Koszewski, Judd H. Fastenberg, Gurston G. Nyquist, Marc R. Rosen, Colin Huntley, Mindy R. Rabinowitz, James J. Evans

Department of Otolaryngology - Head and Neck Surgery Faculty Papers

BACKGROUND: Although recent guidelines for obstructive sleep apnea recommend early postoperative use of continuous positive airway pressure (CPAP) after endonasal skull base surgery, the time of initiation of CPAP is unclear. In this study we used a novel, previously validated cadaveric model to analyze the pressures delivered to the cranial base and evaluate the effectiveness of various repair techniques to withstand positive pressure.

METHODS: Skull base defects were surgically created in 3 fresh human cadaver heads and repaired using 3 commonly used repair techniques: (1) Surgicel™ onlay; (2) dural substitute underlay with dural sealant onlay; and (3) dural substitute underlay …

Is Retinal Metabolic Dysfunction At The Center Of The Pathogenesis Of Age-Related Macular Degeneration?, Thierry Léveillard, Nancy J. Philp, Florian Sennlaub

Department of Pathology, Anatomy, and Cell Biology Faculty Papers

The retinal pigment epithelium (RPE) forms the outer blood⁻retina barrier and facilitates the transepithelial transport of glucose into the outer retina via GLUT1. Glucose is metabolized in photoreceptors via the tricarboxylic acid cycle (TCA) and oxidative phosphorylation (OXPHOS) but also by aerobic glycolysis to generate glycerol for the synthesis of phospholipids for the renewal of their outer segments. Aerobic glycolysis in the photoreceptors also leads to a high rate of production of lactate which is transported out of the subretinal space to the choroidal circulation by the RPE. Lactate taken up by the RPE is converted to pyruvate and metabolized …

Hemi-Eses Associated With Agenesis Of The Corpus Callosum And Normal Cognition., Mahmoud Mohammadi, Safoura Kowkabi, Ali Akbar Asadi-Pooya, Reza Azizi Malamiri, Reza Shervin Badv

Department of Neurology Faculty Papers

• Corpus callosum plays the important role in bilateral synchronous expression of focal discharges of ESES.
• Sparing dominant hemisphere form continuous spike and slow waves during sleep accounts for normal cognitive scores.
• Early detection and treatment of ESES have a great impact on cognitive and language scores and final prognosis.

Gitelman Syndrome: A Rare Cause Of Seizure Disorder And A Systematic Review., Muhammad Asim Shahzad, Maryam Mukhtar, Asrar Ahmed, Waqas Ullah, Rehan Saeed, Mohsin Hamid

Abington Jefferson Health Papers

Gitelman syndrome is one of the few inherited causes of metabolic alkalosis due to salt losing tubulopathy. It is caused by tubular defects at the level of distal convoluted tubules, mimicking a thiazide-like tumor. It usually presents in late childhood or in teenage as nonspecific weakness, fatigability, polyuria, and polydipsia but very rarely with seizures. It is classically associated with hypokalemia, hypomagnesemia, hypocalciuria, hyperreninemia, and hyperaldosteronism. However, less frequently, it can present with normal magnesium levels. It is even rarer to find normomagnesemic patients of GS who develop seizures as the main complication since hypomagnesemia is considered the principal etiology …

Pharmacological And Non-Pharmacological Treatments For The Neonatal Abstinence Syndrome (Nas)., A. K. Mangat, G. M. Schmölzer, W. K. Kraft

Department of Pharmacology and Experimental Therapeutics Faculty Papers

Neonatal abstinence syndrome is defined by signs and symptoms of withdrawal that infants develop after intrauterine maternal drug exposure. All infants with documented in utero opioid exposure, or a high pre-test probability of exposure should have monitoring with a standard assessment instrument such as a Finnegan Score. A Finnegan score of >8 is suggestive of opioid exposure, even in the absence of declared use during pregnancy. At least half of infants in most locales can be treated without the use of pharmacologic means. For this reason, symptom scores will drive the decision for pharmacologic therapy. Nevertheless, all infants, regardless of …

Combination Therapy With Fingolimod And Neural Stem Cells Promotes Functional Myelination, Yuan Zhang, Xin-Yu Lu, Ze-Qin Ye, Bogoljub Ciric, Cun-Gen Ma, A. M. Rostami, Xing Li, Guang-Xian Zhang

Department of Neurology Faculty Papers

Myelination, which occurs predominantly postnatally and continues throughout life, is important for proper neurologic function of the mammalian central nervous system (CNS). We have previously demonstrated that the combination therapy of fingolimod (FTY720) and transplanted neural stem cells (NSCs) had a significantly enhanced therapeutic effect on the chronic stage of experimental autoimmune encephalomyelitis, an animal model of CNS autoimmunity, compared to using either one of them alone. However, reduced disease severity may be secondary to the immunomodulatory effects of FTY720 and NSCs, while whether this therapy directly affects myelinogenesis remains unknown. To investigate this important question, we used three myelination …

Finding The 'Qr' To Patient Safety: Applying Gamification To Incorporate Patient Safety Priorities Through A Simulated 'Escape Room' Experience., Xiao Chi Zhang, Gretchen Diemer, Hyunjoo Lee, Rebecca Jaffe, Dimitrios Papanagnou

Department of Emergency Medicine Faculty Papers

Medical errors are the eighth leading cause of mortality in the United States and contribute to over one million preventable injuries. In an effort to prevent medical errors, reporting systems serve as invaluable tools to detect patient safety events and quality problems longitudinally. Historically, trainees (i.e., students and residents) rarely submit incident reports for encountered patient safety threats. The authors propose an immersive learning experience utilizing gamification theory and leveraging the increasingly popular ‘escape room’ to help resident trainees identify reportable patient safety priorities. All 130 incoming intern physicians at the Thomas Jefferson University (Jefferson) were enrolled in the Patient …

Medical Marijuana-Induced Tacrolimus Toxicity., Daniel Moadel, Keira Chism

Department of Psychiatry and Human Behavior Faculty Papers

As both recreational and therapeutic marijuana use increases in the US, more attention is being paid to its direct medical and psychoactive effects. One crucial dimension is the potential for marijuana or marijuana-derived therapies to interact with other prescribed medications. Tacrolimus is an immunosuppressant medication prescribed to prevent rejection in patients receiving solid organ and bone marrow transplants. Clinically, it is characterized by a narrow therapeutic index and multiple drug-drug interactions. Constituents in marijuana are known to inhibit cytochrome P-450 3A, which is normally responsible for metabolizing tacrolimus, leading to the potential for a dangerous interaction. Though this phenomenon has …

Eliciting Patient-Important Outcomes Through Group Brainstorming: When Is Saturation Reached?, Marianna Lanoue, Alexzandra Gentsch, Amy Cunningham, Geoffrey Mills, Amanda M.B. Doty, Judd E. Hollander, Brendan G. Carr, Larry Loebell, Gail Weingarten, Kristin L. Rising

Department of Emergency Medicine Faculty Papers

PURPOSE: Group brainstorming is a technique for the elicitation of patient input that has many potential uses, however no data demonstrate concept saturation. In this study we explore concept saturation in group brainstorming performed in a single session as compared to two or three sessions.

METHODS: Fifty-two predominately African American adults patients with moderately to poorly controlled Diabetes Mellitus participated in three separate group brainstorming sessions as part of a PCORI-funded group concept mapping study examining comparing methods for the elicitation of patient important outcomes (PIOs). Brainstorming was unstructured, in response to a prompt designed to elicit PIOs in diabetes …

Utilizing The Boston Syncope Observation Management Pathway To Reduce Hospital Admission And Decrease Adverse Outcomes, Oren J. Mechanic, Celine Y. Pascheles, Gregory J. Lopez, Alina M. Winans, Nathan I. Shapiro, Carrie Tibbles, Richard E. Wolfe, Shamai A. Grossman

Student Papers, Posters & Projects

Introduction: In an age of increasing scrutiny of each hospital admission, emergency department (ED) observation has been identified as a low-cost alternative. Prior studies have shown admission rates for syncope in the United States to be as high as 70%. However, the safety and utility of substituting ED observation unit (EDOU) syncope management has not been well studied. The objective of this study was to evaluate the safety of EDOU for the management of patients presenting to the ED with syncope and its efficacy in reducing hospital admissions.

Methods: This was a prospective before-and-after cohort study of consecutive patients presenting …

Association Between Hypertensive Disorders And Fetal Growth Abnormalities In Class Ii And Iii Obese Women, Mary Ferrante, Bs, Rebekah Mccurdy, Md, Mph

Phase 1

Introduction: Studies have shown that hypertension in pregnancy can lead to small for gestational age newborns while obesity can lead to large for gestational age newborns. However, little research has been done to study the influence of both disorders on birthweight.

Objective: To assess the effect of both maternal obesity and a hypertensive disorder on neonatal birthweight and to identify the specific growth abnormality(s) present if a discrepancy exists. We hypothesize that obese women diagnosed with hypertensive disorders are more likely to deliver neonates with growth abnormalities compared to obese women without hypertensive disorders.

Methods: This is a …

Bova Is Superior To Spesi In Identification Of High Risk Pulmonary Embolism Patients, J. Dibello, P. Khaing, E. Gupta, C. Au, T. Chengsupanimit, B. K. Awsare, M. Baram, F. M. West, G. C. Kane

Phase 1

Introduction: Prognostic models exist for the purpose of stratifying patients with acute pulmonary embolism. Of these, the Pulmonary Embolism Severity Index (PESI) and the simplified PESI (sPESI) are the most well-known, although more recent composite models, like the BOVA score, are now being studied and implemented. Comparative efficacy of these scores to predict long term mortality is not well established.

Methods: We performed a retrospective analysis of all consecutive patients diagnosed with PE using computed tomography scan from 2014-2016 at an urban tertiary-referral medical center. Cox proportional hazard analyses were performed to compare the performance of two prognostic …

Emergency Medicine Palliative Care Access (Empalla): Preliminary Data From A Multi-Center Randomized Controlled Trial, Abigail M. Schmucker, Ba, Deborah J. Shim, Bs, Corita R. Grudzen, Md, Mshs, Jeanne Cho, Mph, Keith Goldfeld, Drph

Phase 1

Introduction: Emergency department (ED)-initiated palliative care has been shown to improve patient-centered outcomes in older adults with serious illnesses, but the optimal modality for providing such interventions is unknown. The EMPallA trial compares nurse-led, telephonic case management with specialty, outpatient palliative care on: 1) patient quality of life (QOL); 2) healthcare utilization; 3) loneliness and symptom burden; and 4) caregiver strain, QOL, and bereavement.

Objective: Summarize preliminary demographic and QOL data for the EMPallA cohort.

Methods: A pragmatic, parallel, two-arm randomized controlled trial is enrolling 1350 ED patients across 9 EDs over 3 years to compare the effectiveness of palliative …

Opioid Consumption After Orthopedic Surgery: Prospective Randomized Trial On The Effects Of Preoperative Counseling, Sage Vincent, Taylor Paskey, Elizabeth Critchlow, Erica Mann, Talia Chapman, Md, Kristin Sandrowski, Md, Asif Ilyas, Md

Phase 1

Introduction: Recently, there have been increases in morbidity and mortality associated with opioid medications. Physician overprescribing, particularly within orthopedics, contributes to large quantities of leftover opioids, leading to an increased risk of non-medical opioid use and abuse.

Objective: To understand the role of preoperative patient counseling on postoperative opioid consumption, a prospective randomized study was done, with the idea that patients receiving counseling would take fewer opioids than their control counterparts.

Methods: Patients undergoing elective outpatient orthopedic surgery at Jefferson affiliate hospitals were enrolled over a 3-month period. Patients were randomized to a control group or to receive preoperative counseling …

Direct Anterior Approach Utilizing A Bikini Incision Has Less Wound Related Complications In Patients With High Bmi, Taylor Paskey, Bs, Jorge Manrique, Md, Majd Tarabichi, Md, Camilo Restrepo, Md, Carol Foltz, Phd, William J. Hozack, Md

Phase 1

Introduction: Direct anterior approach (DAA) total hip arthroplasty (THA) can be performed through a traditional vertical skin incision, situating the proximal incision at the hip flexion crease, or a horizontal (bikini) skin incision, situating the incision slightly distal and parallel to the hip flexion crease. The dissection beyond the subcutaneous layer is identical for both methods.

Objective: The purpose of this study was to compare these approaches, performed by an experienced single surgeon, in terms of overall wound complications and patient-reported esthetics 6-months post-operatively. It was hypothesized that the bikini incision would result in less wound complications and …

Minimally Invasive Surgery In Neonates With Congenital Anomalies: Experience From The Nsqip-P, Emily Sagalow, Katerina Dukleska, Md, Courtney Devin, Md, Erin Teeple, Md, Scott W. Cowan, Md, Charles D. Vincour, Md, Loren Berman, Md

Phase 1

Background: Congenital diaphragmatic hernias (CDH) and tracheoesophageal fistulas (TEF) are managed with minimally invasive surgery (MIS) or open surgery. Little is known about the patient populations and outcomes for those treated by each approach. Hypothesis/Specific Aims: We expect that there will be fewer complications, better outcomes, and longer operative times for the MIS group versus the open group.

Methods: National Surgical Quality Improvement Program-Pediatric Participant Use Files (NSQIP-P PUFs) from 2012-2015 were used to identify neonates (up to 30 days old) who underwent CDH and TEF repair. The patient characteristics, post-operative complications, and 30-day mortality were analyzed using multivariable logistic …