Medicine and Health Sciences Commons^™

A Case Of Hemoglobinopathy-Thalassemia Syndrome, Michele C. Khurana, B.S., Gene Gulati, Ph.D

Department of Pathology Honors Program Student Research Symposium

Patient Presentation

• 43 year-old African American female presents with left lower extremity pain
• Physical exam: unremarkable
• Past medical history:

Anemic since childhood

Avascular necrosis of left hip

DVT

Chronic illnesses: asthma, depression

Surgical history: appendectomy, tubal ligation, splenectomy, cholecystectomy, L hip replacement

• Family history: mother has lupus

Thoracic Dissecting Aneurysm And The Importance Of Genetics, Dan Kramer, Ba, Anthony Prestipino, M.D.

Department of Pathology Honors Program Student Research Symposium

Patient Presentation

• 28 year old white male with a past medical history significant for untreated hypertension
• Presents with 3 day history of chest discomfort, lower extremity weakness, bowel and bladder dysfunction, near-syncopal episodes
• Absent pulse in right femoral artery, weak pulse in left femoral artery
• Echosonography showed acute Type A dissecting aortic aneurysm
• Treatment

Immediately taken to surgery for repair of aorta with a graft and replacement of the aortic valve

• Outcome

Unstable after surgery

Biventricular heart failure

Persistent bleeding into mediastinum due to DIC

Required massive transfusion of RBCs, FFP, Cryoprecipitate, and Platelets

Became anuric and acidotic

Became fluid …

Adenoid Cystic Carcinoma In Unusual Locations – Differential Diagnosis Difficulties, Heather A. Mcmahon, B.S., Madalina Tuluc, Md

Department of Pathology Honors Program Student Research Symposium

Adenoid Cystic Carcinoma (ACC) is an uncommon malignant salivary gland tumor, representing 1% of all malignant tumors of the oral and maxillofacial region and 22% of all salivary gland malignancies. Although in the majority of cases ACC manifests in the major salivary glands, in rare instances it can be found in locations such as the esophagus, larynx, trachea, lacrimal gland, breast, prostate, lungs, or auditory canal. These unusual locations of ACC often pose a diagnostic challenge to the clinician and are associated with poorer outcomes for patients as compared to those ACC of a more typical presentation. In this study …

Correlations Between Gene Amplification And Protein Expression Of Topoisomerase 2a (Top2a) In Squamous Cell Carcinoma Of The Lung, Anita Modi, Msii, Charalambos C. Solomides, Md

Department of Pathology Honors Program Student Research Symposium

Background: While DNA topoisomerase 2A (TOP2A) plays an essential role in maintaining the structural integrity of the double helix during replication and recombination, excessive expression of this enzyme may promote malignant cell transformations. In fact, increased levels of TOP2A have been observed in various cancer cell lines including squamous cell carcinoma of the lung. This study sought to identify correlations between genotypic and phenotypic evidence of TOP2A obtained via in situ hybridization (ISH) and immunohistochemistry (IHC) techniques.

Methods Tissue microarrays created from 29 samples of Stage I Squamous Cell Carcinoma of the lung were stained with VENTANA BenchMark ULTRA platform …

Temporal Lobe Ganglioglioma In An Epilepsy Patient With Worsening Seizures: Case Study And Future Directions, Adam M. Olszewski, Mark Curtis, Md Phd.

Department of Pathology Honors Program Student Research Symposium

Gangliogliomas are rare brain tumors that have both neuronal and glial components. They hold a better prognosis than other infiltrative glial tumors, but differentiating between them can be challenging. While molecular markers are being studied, it is currently a histologic diagnosis.

In this case, the patient presented with epilepsy and worsening seizures secondary to a medial temporal lobe mass. Radiology report was inconclusive. Surgical resection was achieved and based on histologic examination the lesion was diagnosed as ganglioglioma, WHO grade I.

In this patient, pathological diagnosis of ganglioglioma offers a favorable prognosis and low risk of recurrence. In the future, …

Diagnostic Yield Of Endobronchial Ultrasound-Guided Fine Needle Aspiration (Ebus-Fna) In Lung Cancer Staging, Subtyping And Diagnosis Of Unexplained Mediastinal Lymphadenopathy, Rino Sato, Rossitza Draganova-Tacheva, Md

Department of Pathology Honors Program Student Research Symposium

INTRODUCTION

• Lung cancer (LC) is the most commonly diagnosed cancer worldwide and the most frequent cause of cancer death in both men and women in the US (more deaths than the next three most common cancers combined)¹
• Clinical staging of LC is an integral part of patient care because it directs therapy and has prognostic value

Patients are routinely investigated with a conventional workup (medical history, PE, lab tests, bronchoscopy), CT and integrated whole-body PET-CT, followed by mediastinal tissue staging for enlarged or PET-positive intrathoracic nodes²

• Mediastinal tissue staging has been classically performed by mediastinoscopy, but they can …

Melanocytoma Of The Cerebellopontine Angle, Pranay Soni, M.D, Lawrence C. Kenyon, Md, Phd

Department of Pathology Honors Program Student Research Symposium

Ms. M.G. is a 46-year-old woman with a history of hypertension and prior bilateral laser eye surgery. In 2009, she presented with vertigo, tinnitus, and decreased hearing in the left ear. An MRI scan revealed an enhancing mass in the posterior fossa that was thought to be an acoustic neuroma. In May of 2009, she underwent gamma-knife radiotherapy for the 2.7 cm mass.

In September of 2012, M.G. noted progressive change in her voice quality as well as a swallowing disturbance and left facial spasms. A subsequent MRI showed significant enlargement of the tumor to a maximal size of 3.7 …

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (Dipnech) To Carcinoid: Exploring The Link, Jacob D. Mcfadden Msii, Jeffrey P. Baliff Md

Department of Pathology Honors Program Student Research Symposium

Neuroendocrine cells (NECs) play important roles in normal lung development, autonomic regulation of lung function, and response to hypoxia or injury. They are located between the respiratory epithelium and the basement membrane of bronchi and bronchioles (below), often as single cells or small clusters.

NEC proliferation is a common pathologic occurrence, well established as a reactive response to chronic lung injury. Much less commonly, NEC proliferation is observed in the absence of apparent inciting factors. This neoplastic phenomenon is termed “diffuse idiopathic pulmonary neuroendocrine cell hyperplasia” (DIPNECH).

DIPNECH is recognized as a precursor lesion for carcinoid tumors. While the progression …

Ocular Manifestations Of Rosai-Dorfman Disease, Phoebe L. Mellen, B.S., Ralph C. Eagle Jr., M.D.

Department of Pathology Honors Program Student Research Symposium

History of Rosai-Dorfman Disease

In 1969, Dr. Juan Rosai and Dr. Ronald F. Dorfman reported four cases of an entity that previously had puzzled pathologists and clinicians. The four cases had failed to fit under any diagnosis, but shared a few common characteristics. Each patient presented with painless, massive lymphadenopathy, most commonly of the cervical lymph nodes¹. Other locations included the inguinal, intra-parotid, and axillary lymph nodes. The patients all presented with fever and leukocytosis¹. The differential diagnosis included malignant lymphoma, malignant histiocytosis, reticuloendotheliosis, and chronic inflammation. However, the histopathological characteristics of the cases did not …

Download Entire Issue- Jefferson Interprofessional Education And Care Newsletter, Spring, 2013, Volume 4, Issue 1

Collaborative Healthcare: Interprofessional Practice, Education and Evaluation (JCIPE)

Entire Spring 2013, Volume 4, No. 1 JCIPE newsletter

Upcoming Faculty And Staff Development Programs

Collaborative Healthcare: Interprofessional Practice, Education and Evaluation (JCIPE)

Interprofessional Geriatric Education and Practice Institute

Eastern Pennsylvania‐Delaware Geriatric Education Center : Interprofessional Geriatric Practice

Collaborating Across Borders (CAB IV) An American‐Canadian Dialogue on Interprofessional Health Education & Practice

From The Editors, Elizabeth Speakman, Edd, Rn, Anef, Christine A. Arenson, Md

Collaborative Healthcare: Interprofessional Practice, Education and Evaluation (JCIPE)

Welcome to the latest edition of the Jefferson Interprofessional Education and Care Newsletter. In our past newsletter we described a meeting that took place February 2011 that previewed the core competencies presented by IPEC. We are pleased to announce that TJU has adopted four IPE core competencies for Interprofessional Collaborative Practice, they are; Values/Ethics‐ Respect the unique cultures, values, roles/responsibilities and expertise of other health professionals; Roles/ Responsibilities‐ Explain the roles and responsibilities of other health/healthcare providers and how the team works together to provide care; Interprofessional Communication‐ Work to ensure common understanding of information, treatment, and health/healthcare decisions by …

Jeff-Chat Offers Students An Interprofessional Forum To Discuss Experiences, Danielle Elliot, Md, Scott Schmidt, Md, Claire Hoppenot, Md, Maria Hervada-Page, Mss

Collaborative Healthcare: Interprofessional Practice, Education and Evaluation (JCIPE)

Feelings of frustration, loss, and exhaustion are common among healthcare workers. Students placed among new experiences and in a dependent learning role are not shielded from these emotions but often experience them differently from other members of the healthcare team. In addition to the systematic and individual support available through TJUH, for the past year and a half Jeff‐ CHAT (Compassion, Humanism, Altruism, Trust) has provided a forum for students to discuss and reflect on difficult emotions and situations.

Six Sigma Methodology Applied To Immediate Use Sterilization At Thomas Jefferson University Hospital, Beth-Ann Schauer, Mba, Pmp, Ssgb, Lori Szymonowicz, Cpam, Ssgb, Precil Varghese, Pharmd

Collaborative Healthcare: Interprofessional Practice, Education and Evaluation (JCIPE)

Immediate use sterilization, also known as flash sterilization, is an expedited sterilization process used for surgical equipment needed in emergent clinical situations. Immediate use sterilization is a procedure used by Operating Room staff members to sterilize instrumentation on an as needed or just in time basis when instruments needed for a particular case are not immediately available or become contaminated in the course of the case. If instrumentation is not sterilized appropriately, the patient’s safety is possibly put at risk from the potential of surgical site infections. No specific literature data directly correlates immediate use sterilization with surgical site infection. …

Cavin1; A Regulator Of Lung Function And Macrophage Phenotype., Praveen Govender, Freddy Romero, Dilip Shah, Jesus Paez, Shi-Ying Ding, Libin Liu, Adam Gower, Elizabeth Baez, Sherif Shawky Aly, Paul Pilch, Ross Summer

Center for Translational Medicine Faculty Papers

Caveolae are cell membrane invaginations that are highly abundant in adipose tissue, endothelial cells and the lung. The formation of caveolae is dependent on the expression of various structural proteins that serve as scaffolding for these membrane invaginations. Cavin1 is a newly identified structural protein whose deficiency in mice leads to loss of caveolae formation and to development of a lipodystrophic phenotype. In this study, we sought to investigate the functional role of Cavin1 in the lung. Cavin1 deficient mice possessed dramatically altered distal lung morphology and exhibited significant physiological alterations, notably, increased lung elastance. The changes in distal lung …

The Nordinet® International Outcome Study And Novonet® Answer Program®: Rationale, Design, And Methodology Of Two International Pharmacoepidemiological Registry-Based Studies Monitoring Long-Term Clinical And Safety Outcomes Of Growth Hormone Therapy (Norditropin®)., Charlotte Höybye, Lars Sävendahl, Henrik Thybo Christesen, Peter Lee, Birgitte Tønnes Pedersen, Michael Schlumpf, John Germak, Judith Ross

Department of Pediatrics Faculty Papers

OBJECTIVE: Randomized controlled trials have shown that growth hormone (GH) therapy has effects on growth, metabolism, and body composition. GH therapy is prescribed for children with growth failure and adults with GH deficiency. Carefully conducted observational study of GH treatment affords the opportunity to assess long-term treatment outcomes and the clinical factors and variables affecting those outcomes, in patients receiving GH therapy in routine clinical practice.

DESIGN: The NordiNet® International Outcome Study (IOS) and the American Norditropin® Studies: Web Enabled Research (ANSWER Program®) are two complementary, non-interventional, observational studies that adhere to current guidelines for pharmacoepidemiological data.

PATIENTS: The studies …

Pharmacology And Clinical Potential Of Guanylyl Cyclase C Agonists In The Treatment Of Ulcerative Colitis., Giovanni M Pitari

Department of Pharmacology and Experimental Therapeutics Faculty Papers

Agonists of the transmembrane intestinal receptor guanylyl cyclase C (GCC) have recently attracted interest as promising human therapeutics. Peptide ligands that can specifically induce GCC signaling in the intestine include endogenous hormones guanylin and uroguanylin, diarrheagenic bacterial enterotoxins (ST), and synthetic drugs linaclotide, plecanatide, and SP-333. These agonists bind to GCC at intestinal epithelial surfaces and activate the receptor's intracellular catalytic domain, an event initiating discrete biological responses upon conversion of guanosine-5'-triphosphate to cyclic guanosine monophosphate. A principal action of GCC agonists in the colon is the promotion of mucosal homeostasis and its dependent barrier function. Herein, GCC agonists are …

Functional And Quality-Of-Life Outcomes In Geriatric Patients With Type-Ii Dens Fracture., Alexander R Vaccaro, Christopher K Kepler, Branko Kopjar, Jens Chapman, Christopher Shaffrey, Paul Arnold, Ziya Gokaslan, Darrel Brodke, John France, Mark Dekutoski, Rick Sasso, S Tim Yoon, Christopher Bono, James Harrop, Michael G Fehlings

Rothman Institute Papers

BACKGROUND: Dens fractures are relatively common in the elderly. The treatment of Type-II dens fractures remains controversial. The aim of this multicenter prospective cohort study was to compare outcomes (assessed with use of validated clinical measures) and complications of nonsurgical and surgical treatment of Type-II dens fractures in patients sixty-five years of age or older.

METHODS: One hundred and fifty-nine patients with a Type-II dens fracture were enrolled in a multicenter prospective study. Subjects were treated either surgically (n = 101) or nonsurgically (n = 58) as determined by the treatment preferences of the treating physicians and the patients. The …

Alcohol And The Heart: To Abstain Or Not To Abstain?, Rajesh Movva, Md, Mrcp, Vincent M. Figueredo, M.D.

Division of Cardiology Faculty Papers

Alcohol has been consumed by most societies over the last 7000 years. Abraham Lincoln said "It has long been recognized that the problems with alcohol relate not to the use of a bad thing, but to the abuse of a good thing." Light to moderate alcohol consumption reduces the incidence of coronary heart disease (CHD), ischemic stroke, peripheral arterial disease, CHD mortality, and all-cause mortality, especially in the western populations. However, heavy alcohol consumption is detrimental causing cardiomyopathy, cardiac arrhythmias, hepatic cirrhosis, pancreatitis, and hemorrhagic stroke. In this article, we review the effects of alcohol on CHD, individual cardiovascular risk …

Smooth Muscle Fascicular Reorientation Is Required For Esophageal Morphogenesis And Dependent On Cdo., Anthony I Romer, Jagmohan Singh, Satish Rattan, Robert S Krauss

Department of Medicine Faculty Papers

Postnatal maturation of esophageal musculature involves proximal-to-distal replacement of smooth muscle with skeletal muscle by elusive mechanisms. We report that this process is impaired in mice lacking the cell surface receptor Cdo and identify the underlying developmental mechanism. A myogenic transition zone containing proliferative skeletal muscle precursor cells migrated in a proximal-distal direction, leaving differentiated myofibers in its wake. Distal to the transition zone, smooth muscle fascicles underwent a morphogenetic process whereby they changed their orientation relative to each other and to the lumen. Consequently, a path was cleared for the transition zone, and smooth muscle ultimately occupied only the …

Comparison Study Of Airway Reactivity Outcomes Due To A Pharmacologic Challenge Test: Impulse Oscillometry Versus Least Mean Squared Analysis Techniques., Elena Rodriguez, Charrell M Bullard, Milena H Armani, Thomas L Miller, Thomas H Shaffer

Department of Medicine Faculty Papers

The technique of measuring transpulmonary pressure and respiratory airflow with manometry and pneumotachography using the least mean squared analysis (LMS) has been used broadly in both preclinical and clinical settings for the evaluation of neonatal respiratory function during tidal volume breathing for lung tissue and airway frictional mechanical properties measurements. Whereas the technique of measuring respiratory function using the impulse oscillation technique (IOS) involves the assessment of the relationship between pressure and flow using an impulse signal with a range of frequencies, requires less cooperation and provides more information on total respiratory system resistance (chest wall, lung tissue, and airways). …

Treatment Of Intractable Neurogenic Cough With Cricopharyngeal Myotomy, Anne Lee Durstenfeld, Bs, Joseph Spiegel, Md, Mauritis Boon, Md

Department of Otolaryngology - Head and Neck Surgery Presentations and Grand Rounds

Objectives: Neuropathic cough that is incompletely responsive to medical therapy may be due to cricopharyngeal hypertonicity. The objective was to describe the utility of cricopharyngeal myotomy in alleviating symptoms of intractable neuropathic cough.

Study Design: Retrospective review.

Methods: A retrospective chart review was performed for three patients who underwent cricopharyngeal myotomy for intractable cough. Trigger phenomena, previous evaluation and treatment, and outcomes after surgery, namely patient perception of improvement and medication use after surgery, were assessed and documented.

Results: After cricopharyngeal myotomy, all three patients noted symptomatic improvement and were weaned off medication. Continued improvement was noted at follow …

A Review Of Nutritional Factors In Hypertension Management., Ha Nguyen, Olaide A Odelola, Janani Rangaswami, Aman Amanullah

Department of Medicine Faculty Papers

Hypertension is a major health problem worldwide. Its attendant morbidity and mortality complications have a great impact on patient's quality of life and survival. Optimizing blood pressure control has been shown to improve overall health outcomes. In addition to pharmacological therapies, nonpharmacological approach such as dietary modification plays an important role in controlling blood pressure. Many dietary components such as sodium, potassium, calcium, and magnesium have been studied substantially in the past decades. While some of these nutrients have clear evidence for their recommendation, some remain controversial and are still of ongoing study. Dietary modification is often discussed with patients …

Bortezomib (Ps-341) Treatment Decreases Inflammation And Partially Rescues The Expression Of The Dystrophin-Glycoprotein Complex In Grmd Dogs., Karla P C Araujo, Gloria Bonuccelli, Caio N Duarte, Thais P Gaiad, Dayson F Moreira, David Feder, José E Belizario, Maria A Miglino, Michael P Lisanti, Carlos E Ambrosio

Department of Stem Cell Biology and Regenerative Medicine Faculty Papers & Presentations

Golden retriever muscular dystrophy (GRMD) is a genetic myopathy corresponding to Duchenne muscular dystrophy (DMD) in humans. Muscle atrophy is known to be associated with degradation of the dystrophin-glycoprotein complex (DGC) via the ubiquitin-proteasome pathway. In the present study, we investigated the effect of bortezomib treatment on the muscle fibers of GRMD dogs. Five GRMD dogs were examined; two were treated (TD- Treated dogs) with the proteasome inhibitor bortezomib, and three were control dogs (CD). Dogs were treated with bortezomib using the same treatment regimen used for multiple myeloma. Pharmacodynamics were evaluated by measuring the inhibition of 20S proteasome activity …

Converging Evidence For The Processing Costs Associated With Ambiguous Quantifier Comprehension., Corey T Mcmillan, Danielle Coleman, Robin Clark, Tsao-Wei Liang, Rachel G Gross, Murray Grossman

Department of Neurology Faculty Papers

Traditional neuroanatomic models of language comprehension have emphasized a core language network situated in peri-Sylvian cortex. More recent evidence appears to extend the neuroanatomic network beyond peri-Sylvian cortex to encompass other aspects of sentence processing. In this study, we evaluate the neuroanatomic basis for processing the ambiguity in doubly-quantified sentences. For example, a sentence like "All the dogs jumped in a lake" can be interpreted with a collective interpretation (e.g., several dogs jumping into a single lake) or a distributive interpretation (e.g., several dogs each jumping into a different lake). In Experiment 1, we used BOLD fMRI to investigate neuroanatomic …

Glucose Stimulation Induces Dynamic Change Of Mitochondrial Morphology To Promote Insulin Secretion In The Insulinoma Cell Line Ins-1e., Bong Sook Jhun, Hakjoo Lee, Zheng-Gen Jin, Yisang Yoon

Center for Translational Medicine Faculty Papers

Fission and fusion of mitochondrial tubules are the major processes regulating mitochondrial morphology. However, the physiological significance of mitochondrial shape change is poorly understood. Glucose-stimulated insulin secretion (GSIS) in pancreatic β-cells requires mitochondrial ATP production which evokes Ca(2+) influx through plasma membrane depolarization, triggering insulin vesicle exocytosis. Therefore, GSIS reflects mitochondrial function and can be used for evaluating functional changes associated with morphological alterations of mitochondria. Using the insulin-secreting cell line INS-1E, we found that glucose stimulation induced rapid mitochondrial shortening and recovery. Inhibition of mitochondrial fission through expression of the dominant-negative mutant DLP1-K38A eliminated this dynamic mitochondrial shape change …

Glucose Stimulation Induces Dynamic Change Of Mitochondrial Morphology To Promote Insulin Secretion In The Insulinoma Cell Line Ins-1e., Bong Sook Jhun, Hakjoo Lee, Zheng-Gen Jin, Yisang Yoon

Center for Translational Medicine Faculty Papers

Fission and fusion of mitochondrial tubules are the major processes regulating mitochondrial morphology. However, the physiological significance of mitochondrial shape change is poorly understood. Glucose-stimulated insulin secretion (GSIS) in pancreatic β-cells requires mitochondrial ATP production which evokes Ca(2+) influx through plasma membrane depolarization, triggering insulin vesicle exocytosis. Therefore, GSIS reflects mitochondrial function and can be used for evaluating functional changes associated with morphological alterations of mitochondria. Using the insulin-secreting cell line INS-1E, we found that glucose stimulation induced rapid mitochondrial shortening and recovery. Inhibition of mitochondrial fission through expression of the dominant-negative mutant DLP1-K38A eliminated this dynamic mitochondrial shape change …

Promoting Health Literacy Through Leading Edge Occupational Therapy Education, Susan Toth-Cohen, Phd, Otr/L, Annalisa Synnestvedt, Ba, Ots, Alyssa Frey Peachey, Ots

Department of Occupational Therapy Posters and Presentations

Synopsis:

Health literacy has become a vital focus of U.S. healthcare. By incorporating health literacy knowledge, attitudes, and skills into entry-­‐level education, occupational therapy professionals can enact a leading role in health and human services teams to address our national healthcare goals.

Poster presentation at the American Occupational Therapy Association Annual Conference, San Diego, CA, April 2013.

Jefferson Digital Commons Quarterly Report: January-March 2013, Daniel G. Kipnis, Msi

Jefferson Digital Commons Reports

Jefferson Digital Commons quarterly report includes statistics, new libguide for the Jefferson Digital Commons, new assets added to the collection.

The Tweak Receptor Fn14 Is A Therapeutic Target In Melanoma: Immunotoxins Targeting Fn14 Receptor For Malignant Melanoma Treatment., Hong Zhou, Suhendan Ekmekcioglu, John W Marks, Khalid A Mohamedali, Kaushal Asrani, Keeley K Phillips, Sharron A N Brown, Emily Cheng, Michele B Weiss, Walter N Hittelman, Nhan L Tran, Hideo Yagita, Jeffrey A Winkles, Michael G Rosenblum

Department of Cancer Biology Faculty Papers

Fibroblast growth factor-inducible protein 14 (Fn14), the cell surface receptor for tumor necrosis factor-like weak inducer of apoptosis (TWEAK), is overexpressed in various human solid tumor types and can be a negative prognostic indicator. We detected Fn14 expression in ∼60% of the melanoma cell lines we tested, including both B-Raf WT and B-Raf(V600E) lines. Tumor tissue microarray analysis indicated that Fn14 expression was low in normal skin, but elevated in 173/190 (92%) of primary melanoma specimens and in 86/150 (58%) of melanoma metastases tested. We generated both a chemical conjugate composed of the recombinant gelonin (rGel) toxin and the anti-Fn14 …