Medicine and Health Sciences Commons^™

Mice Deficient In Involucrin, Envoplakin, And Periplakin Have A Defective Epidermal Barrier., Lisa M Sevilla, Rachida Nachat, Karen R Groot, John F Klement, Jouni Uitto, Philippe Djian, Arto Määttä, Fiona M Watt

Department of Dermatology and Cutaneous Biology Faculty Papers

The cornified envelope is assembled from transglutaminase cross-linked proteins and lipids in the outermost epidermal layers and is essential for skin barrier function. Involucrin, envoplakin, and periplakin form the protein scaffold on which the envelope assembles. To examine their combined function, we generated mice deficient in all three genes. The triple knockouts have delayed embryonic barrier formation and postnatal hyperkeratosis (abnormal accumulation of cornified cells) resulting from impaired desquamation. Cornified envelopes form but are ultrastructurally abnormal, with reduced lipid content and decreased mechanical integrity. Expression of proteases is reduced and the protease inhibitor, serpina1b, is highly upregulated, resulting in defective …

Editorial Board

Jefferson Journal of Psychiatry

No abstract provided.

Short-Wavelength Light Sensitivity Of Circadian, Pupillary, And Visual Awareness In Humans Lacking An Outer Retina, Farhan H. Zaidi, Joseph T. Hull, Stuart N. Peirson, Katharina Wulff, Daniel Aeschbach, Joshua J. Gooley, George C. Brainard, Kevin Gregory-Evans, Joseph F. Rizzo Iii, Charles A. Czeisler, Russell G. Foster, Merrick J. Moseley, Steven W. Lockley

Department of Neurology Faculty Papers

As the ear has dual functions for audition and balance, the eye has a dual role in detecting light for a wide range of behavioral and physiological functions separate from sight. These responses are driven primarily by stimulation of photosensitive retinal ganglion cells (pRGCs) that are most sensitive to short-wavelength ( approximately 480 nm) blue light and remain functional in the absence of rods and cones. We examined the spectral sensitivity of non-image-forming responses in two profoundly blind subjects lacking functional rods and cones (one male, 56 yr old; one female, 87 yr old). In the male subject, we found …

Evidence-B(I)Ased Medicine: Limitations And Non-Superstition-Based Alternatives, Joshua S. Green M.D., Ph.D. (Pgy4)

Jefferson Journal of Psychiatry

The Evidence-Based Medicine (EBM) movement crystallized in the early 1990's at McMaster University in Canada. The movement originally challenged practitioners to validate their treatments based on reasoning and clinical studies rather than personal authority. In this essay, I will argue that, contrary to this wholesome intention, EBM is authoritarian in spirit and actually constricts discourse about how to make clinical decisions.

Diphenhydramine-Dependence Resulting From Self-Medication Of Antipsychotic- Induced Extrapyramidal Symptoms, Faisal S. Shaikh M.B.B.S., Ann M. Mortimer M.D.

Jefferson Journal of Psychiatry

Antipsychotic-induced extrapyramidal symptoms should be recognised and adequately treated. We report an unusual complication of not doing so: a patient’s self-medicating to control these symptoms, which produced diphenhydramine-dependence.

Dissociative-Like Spells In A Child With Neurofibromatosis (Type 1), Gregory M. Singleton M.D. (Pgy2), Ann M. Lagges Ph.D., Karen G. Meighen M.D.

Jefferson Journal of Psychiatry

Neurofibromatosis, type 1, (NF1) is a common neurocutaneous disorder of childhood. Little is known about the psychiatric aspects of the condition. We present the case of a 10-year-old male with NF1 and complex spells. For two years he had been experiencing self-limited paroxysms of auditory and visual hallucination, assaultiveness, excited undressing, and amnesia. The spells have been refractory to multiple treatments, including antipsychotic medication. The question remains whether this episodic amnestic disorder is comorbid with NF1 or is caused by it.

A Major Event Of Self-Mutilation In A Patient With Fetal Alcohol Syndrome, Joel P. Johnson M.D. (Pgy4), Steven Sugden M.D., James A. Bourgeois O.D., M.D.

Jefferson Journal of Psychiatry

We describe a young adult male with fetal alcohol syndrome (FAS) who presented with a non-repetitive, major, genital self-mutilation necessitating urological intervention. On formal psychiatric evaluation, he did not have psychosis or suicidality. Most commonly, self-injury in developmental disability is less severe and more stereotypic; major-class self-mutilation is confined, generally, to psychosis. It is this discrepancy that is the unusual feature of our case.

Autocastration And Autoamputation Of The Penis In A Patient With Delusions Of Sexual Guilt, Craig B. Franke M.D. (Pgy4), James A. Rush M.D. (Pgy3)

Jefferson Journal of Psychiatry

Genital self-mutilation (GSM) is a rare event that is commonly associated with psychotic disorders; we report an occurrence in the context of psychosis and drug use. We also review the etiologies of this phenomenon and how these etiologies differ across gender.

Clozapine, Fluoxetine, And Benztropine- Associated Ileus: Case Report, Pegah Pajouhi M.D. (Pgy1), James A. Bourgeois O.D., M.D.

Jefferson Journal of Psychiatry

Gastrointestinal complications of anticholinergic medications are prevalent, potentially life-threatening, and could be more actively prevented. We present a case report of an ileus that required surgical intervention and developed in the context of clozapine, benztropine, and fluoxetine use. The case exemplifies the potential anticholinergic toxicities of clozapine and benztropine as well as possible pharmacokinetic interactions between fluoxetine, clozapine, and benztropine. We discuss ways to minimize the likelihood of anticholinergic complications with these medications.

Oral Immunization Of Rhesus Macaques With Adenoviral Hiv Vaccines Using Enteric-Coated Capsules, George T. Mercier, Pramod N. Nehete, Marco F. Passeri, Bharti N. Nehete, Eric A. Weaver, Nancy Smyth Templeton, Kimberly Schluns, Stephanie S. Buchl, K. Buchl, Michael A. Barry

Nebraska Center for Virology: Faculty Publications

Targeted delivery of vaccine candidates to the gastrointestinal (GI) tract holds potential for mucosal immunization, particularly against mucosal pathogens like the human immunodeficiency virus (HIV). Among the different strategies for achieving targeted release in the GI tract, namely the small intestine, pH sensitive enteric coating polymers have been shown to protect solid oral dosage forms from the harsh digestive environment of the stomach and dissolve relatively rapidly in the small intestine by taking advantage of the luminal pH gradient. We developed an enteric polymethacrylate formulation for coating hydroxy-propyl-methyl-cellulose (HPMC) capsules containing lyophilized Adenoviral type 5 (Ad5) vectors expressing HIV-1 gag …

Characterization Of The Anti-Angiogenic Properties Of Kdr-Ig4-7 And Its Variants As Potential Anti-Cancer Therapeutics, Jang Pyo Park

All Dissertations

Beginning with the development of an embryo and throughout one's adult life angiogenesis plays an essential role for organ growth and repair. The balancing of pro-angiogenic factors to anti-angiogenic factors is what regulates the process of angiogenesis. When this balance is disrupted in one direction or the other, numerous disorders can occur including ocular and inflammatory diseases and, in particular, tumor growth and metastasis. Therefore, inhibition of angiogenesis has become an effective therapy for arresting the growth of tumors.
A key regulator of angiogenesis involves vascular endothelial growth factor (VEGF) and its receptor, VEGFR-2 (KDR). The binding of VEGF to …

Evaluation Of Human Prolactin, Its Antagonist, And Antagonist-Based Fusion Proteins As Chemopreventative And Therapeutic Agents, Seth Tomblyn

All Dissertations

Cancer is a collection of diseases with many different manifestations and is the second leading cause of death in the United States. Breast cancer accounts for nearly one third of cancer diagnosis in women. Prolactin (PRL) functions as a lactogen and as a mammary gland differentiation factor. PRL acts in an autocrine/paracrine manner within the mammary gland and in breast tumors which implies PRL may be involved in breast cancer progression. This is corroborated by the PRLR over-expression in breast cancer cells lines and the majority of patient biopsies. These reasons make PRL and PRLR attractive targets for breast cancer …

How To Prevent Perioperative Myocardial Injury: The Conundrum Continues, Jian-Zhong Sun, David Maguire

Department of Anesthesiology Faculty Papers

BACKGROUND: Perioperative myocardial injury (PMI) remains a major cause of perioperative morbidity and mortality but clinical strategies to prevent PMI are still uncertain.

METHODS AND RESULTS: We comprehensively searched PubMed for major research articles concerning clinical strategies to prevent PMI. The key findings are as follows: (1) the American College of Cardiology/American Heart Association guideline update for perioperative cardiovascular evaluation for noncardiac surgery is very useful to stratify cardiac risk preoperatively; (2) cardiac troponin has emerged as a biomarker to diagnose postoperative PMI and to predict clinical outcomes; (3) coronary revascularization before noncardiac surgery probably would provide cardiac protection in …

Targeted Therapies In The Management Of Metastatic Bladder Cancer., Matteo Fassan, Edouard J. Trabulsi, Leonard G Gomella, Raffaele Baffa

Department of Urology Faculty Papers

The management of metastatic urothelial carcinoma (UC) of the bladder is a common and complex clinical challenge. Despite the fact that UC is one of the most frequent tumors in the population, long term survival for metastatic disease remains low, and chemotherapy is curative for only a small minority of patients. UC is genetically heterogeneous, and it is surrounded by a complex tissue microenvironment. The problems of clinical practice in the field of metastatic bladder cancer have begun to stimulate translational research. Advances in the understanding of the molecular biology of urothelial cancer continue to contribute to the identification of …

Interventional Options For Malignant Upper Gi Obstruction, 2nd Ed, James R. Ouellette, Lisa Patterson, Paula M. Termuhlen

Department of Surgery Faculty Publications

Background

Patients with unresectable cancers of the upper gastrointestinal tract often suffer severe symptoms due to pain, nausea and vomiting, weight loss, cachexia, and poor food tolerance. This can be related to gastric and duodenal cancers causing intrinsic obstruction of the intestinal lumen or pancreatic and biliary cancers causing extrinsic biliary compression. Management options vary depending on the site of obstruction, the patient’s functional status, the patient-defined goals of care, and estimated prognosis. Fast Fact #45 discussed medical management options. This Fact Fact reviews interventional approaches for upper GI obstructions, especially when further radiation, chemotherapy, medical management, or curative surgical …

Renal Cell Carcinoma Metastatic To The Duodenum: Treatment By Classic Pancreaticoduodenectomy And Review Of The Literature, Robert Adamo, Patrick J. Greaney Jr., Agnieszka Witkiewicz, Eugene P. Kennedy, Charles J. Yeo

Department of Surgery Faculty Papers

Renal cell cancer (RCC) most commonly metastasizes to the lungs, bones, liver, renal fossa, and brain, although metastases can occur elsewhere. RCC metastatic to the duodenum is especially rare, with only a small number of cases reported in the literature. Herein, we describe a case of an 86-year-old woman with a history of RCC treated by radical nephrectomy 13 years previously. The patient presented with duodenal obstruction and anemia from a solitary duodenal mass invading into the pancreas and was treated via classic pancreaticoduodenectomy. Preoperative imaging and intra-operative assessment showed no evidence of other disease. Pathology confirmed metastatic RCC without …

Traumatic Carotid-Cavernous Fistula:Clinical Presentation And Outcome, Atta A. Bhatti, S. Soulat Raza, Ehsan Bari, S. Ather Enam, Tanveer Ul Haq, Zafar Sajjad

Pakistan Journal of Neurological Sciences (PJNS)

No abstract provided.

Special Article: Physical Activity, Physical Fitness, And Cardiovascular Risk Factors In Childhood, Samuel S. Gidding

Department of Pediatrics Faculty Papers

In adults, physical activity and exercise training are associated with reduced cardiovascular morbidity and mortality, a reduced likelihood of developing adverse cardiovascular risk factors, and improved insulin sensitivity. In childhood, participation in appropriate physical activity may prevent the development of cardiovascular risk factors in the future and complement treatment of existing cardiovascular risk factors, including hypertension, dyslipidemia, and overweight. Exercise in children can also significantly improve insulin sensitivity independent of weight loss. These e fects are mediated in overweight children by increases in lean body mass relative to fat mass and associated improvements in inflammatory mediators, endothelial function, and the …

Breast Cancer In Pakistan - Awareness And Early Detection, Saba Sohail, Shams Nadeem Alam

Department of Radiology

No abstract provided.

Outcome Of Triple Procedure In Older Children With Developmental Dysplasia Of Hip (Ddh), Masood Umer, Haq Nawaz, Pashtoon Murtaza Kasi, Mahmood Ahmed

Department of Surgery

Objective: To evaluate the radiographic and functional results of the triple procedure (open reduction, femoral shortening and Salter's Osteotomy) in the treatment of Developmental Dysplasia of the Hip (DDH) disease in older children. Methods: This case-series comprising 23 patients (29 hips) underwent the triple procedure of open reduction, femoral shortening and Salter osteotomy, at Aga Khan University Hospital between June 1995 and June 2005. Patients were classified pre-operatively according to the Tonnis class. Postoperative functional evaluation was performed using Modified MacKay's scoring system and radiographic assessment using Severin's scoring method. Results: The mean age of patients at presentation was 6.84 …

Bullet-Induced Synovitis As A Cause Of Secondary Osteoarthritis Of The Hip Joint: A Case Report And Review Of Literature, A Rehman, Masood Umer, Yasir J Sepah, Muhammad A Wajid

Department of Surgery

Abstract

BACKGROUND:

With increasing prevalence of gunshot injuries we are seeing more patients with retained bullet fragments lodged in their bodies. Embedded lead bullets are usually considered inert after their kinetic energy has dissipated hence these are not removed routinely. However, exposure of any foreign body to synovial fluid may lead to rapid degradation and hence result in systemic absorption, causing local and systemic symptoms. We present the case of a thirty year old man who came to our out patient department with a history of progressive, severe hip pain ten years after a gun shot injury to his right …

Aoa Symposium. Orthopaedists Partnering With Other Physicians To Provide Musculoskeletal Care., Alan Hilibrand, Kurt P Spindler, Scott D Boden

Rothman Institute Faculty Papers

No abstract provided.

Prognosis Of Hospitalized New-Onset Systolic Heart Failure In Indo-Asians--A Lethal Problem, Fahim H. Jafary, Mahesh Kumar, Irfan E. Chandna

Section of Cardiology

Background: Systolic heart failure (SHF), particularly when requiring hospital admission carries a poor prognosis. There is a paucity of data in Indo-Asians on outcomes of SHF, among whom the burden of cardiovascular disease is consistently rising. The purpose of this study was to determine the frequency and predictors of mortality and morbidity amongst patients admitted with new-onset SHF at a tertiary care hospital in Pakistan.

Methods and Results: Hospital charts of 196 patients with a diagnosis of new or recent onset (!3 months) SHF (ejection fraction [EF] !40%) were reviewed. Patients who died during the admission, those with life-limiting concomitant …

Cardiopulmonary Predicators Of Dysfunctional Ventilator Weaning Response After Coronary Artery Bypass Graft, Annapoorna Mary

Theses and Dissertations (ETD)

Although the majority of coronary artery bypass graft (CABG) surgery patients are extubated within 6 to 8 hours following surgery, 20% to 40% of patients remain intubated 12 hours after surgery due to dysfunctional ventilator weaning response (DVWR). DVWR associated with increased morbidity and mortality (30% to 43%) following CABG surgery. Finding significant antecedence to predict DVWR could help to identify and prevent the complications from DVWR after CABG surgery. Literature review revealed that there is an association between cardiopulmonary indicators (CPI) and DVWR after CABG surgery. Cardiopulmonary indicators are the selected hemodynamic parameters that have an association with DVWR. …

Comparison Of Eight Prosthetic Aortic Valves In A Cadaver Model., Benjamin A. Youdelman, Hitoshi Hirose, Harsh Jain, J Yasha Kresh, John W.C. Entwistle, Andrew S. Wechsler

Department of Surgery Faculty Papers

OBJECTIVES: Proper valve selection is critical to ensure appropriate valve replacement for patients, because implantation of a small valve might place the patient at risk for persistent gradients. Labeled valve size is not the same as millimeter measure of prosthetic valve diameters or the annulus into which it will fit. Studies that use the labeled valve size in lieu of actual measured diameter in millimeters to compare different valves might be misleading. Using human cadaver hearts, we sized the aortic annulus with 8 commonly used prosthetic aortic valve sizers and compared the valves using geometric orifice area. This novel method …

Perforated Appendicitis: Accuracy Of Ct Diagnosis And Correlation Of Ct Findings With The Length Of Hospital Stay, Asma Hina Siddiqui, Shaista Afzal

Department of Radiology

Objective: To determine the sensitivity and specificity of CT findings in the differentiation of perforated from nonperforated appendicitis and correlate CT diagnosis with the length of hospital stay.STUDY Design: An analytical study.PLACE AND DURATION OF STUDY: This study was conducted at Radiology Department of Aga Khan University Hospital, Karachi, from January 2004 to June 2005.PATIENTS AND Methods: The study included 70 patients who presented with right lower quadrant abdominal pain and underwent pre-operative CT scan followed by appendectomy. Patients were divided into two groups of having perforated and nonperforated appendicitis on the basis of CT scan findings. The surgical and …

Neurosurgical Training In Pakistan, Syed Ather Enam

Pakistan Journal of Neurological Sciences (PJNS)

No abstract provided.

Role Of Video-Eeg Monitoring In Themanagement Of Intractable Seizures Andnon-Epileptic Spells, Mughis Sheerani, Ali Hassan, Ameer Jan, Royala Zaka

Pakistan Journal of Neurological Sciences (PJNS)

No abstract provided.

Etiology And Disturbance Of Consciousnessin Stroke: A Hospital-Based Study, Hamzullah Khan, Muhammad Zarif

Pakistan Journal of Neurological Sciences (PJNS)

No abstract provided.

Fluorosis: A Rare Cause Of Spinal Cordcompression, Maimoona Siddiqui, Azra Zafar, M. Salman, Yaseen Pathan

Pakistan Journal of Neurological Sciences (PJNS)

No abstract provided.