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Diagnosis Of B-Cell Lymphoproliferative Disorder Through Concomitant Management Of Medication-Related Osteonecrosis Of The Jaw: A Case Report, Robert Diecidue, Allen F. Champion, Ruifeng Zhou, James G. Buckley Mar 2019

Diagnosis Of B-Cell Lymphoproliferative Disorder Through Concomitant Management Of Medication-Related Osteonecrosis Of The Jaw: A Case Report, Robert Diecidue, Allen F. Champion, Ruifeng Zhou, James G. Buckley

Department of Oral and Maxillofacial Surgery Faculty Papers

No abstract provided.


New Synthetic Cannabinoids Metabolism And Strategies To Best Identify Optimal Marker Metabolites., Xingxing Diao, Marilyn A. Huestis Mar 2019

New Synthetic Cannabinoids Metabolism And Strategies To Best Identify Optimal Marker Metabolites., Xingxing Diao, Marilyn A. Huestis

Institute of Emerging Health Professions Faculty Papers

Synthetic cannabinoids (SCs) were initially developed as pharmacological tools to probe the endocannabinoid system and as novel pharmacotherapies, but are now highly abused. This is a serious public health and social problem throughout the world and it is highly challenging to identify which SC was consumed by the drug abusers, a necessary step to tie adverse health effects to the new drug's toxicity. Two intrinsic properties complicate SC identification, their often rapid and extensive metabolism, and their generally high potency relative to the natural psychoactive Δ 9 -tetrahydrocannabinol in cannabis. Additional challenges are the lack of reference standards for the …


Study Of The Cytological Features Of Bone Marrow Mesenchymal Stem Cells From Patients With Neuromyelitis Optica., Chunsheng Yang, Yang Yang, Li Ma, Guang-Xian Zhang, Fu-Dong Shi, Yaping Yan, Guoqiang Chang Mar 2019

Study Of The Cytological Features Of Bone Marrow Mesenchymal Stem Cells From Patients With Neuromyelitis Optica., Chunsheng Yang, Yang Yang, Li Ma, Guang-Xian Zhang, Fu-Dong Shi, Yaping Yan, Guoqiang Chang

Department of Neurology Faculty Papers

Neuromyelitis optica (NMO) is a refractory autoimmune inflammatory disease of the central nervous system without an effective cure. Autologous bone marrow‑derived mesenchymal stem cells (BM‑MSCs) are considered to be promising therapeutic agents for this disease due to their potential regenerative, immune regulatory and neurotrophic effects. However, little is known about the cytological features of BM‑MSCs from patients with NMO, which may influence any therapeutic effects. The present study aimed to compare the proliferation, differentiation and senescence of BM‑MSCs from patients with NMO with that of age‑ and sex‑matched healthy subjects. It was revealed that there were no significant differences in …


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 Mar 2019

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.


Ripk1 Can Mediate Apoptosis In Addition To Necroptosis During Embryonic Development., Xuhua Zhang, John P. Dowling, Jianke Zhang Mar 2019

Ripk1 Can Mediate Apoptosis In Addition To Necroptosis During Embryonic Development., Xuhua Zhang, John P. Dowling, Jianke Zhang

Department of Microbiology and Immunology Faculty Papers

RIPK1 has emerged as a key effector in programmed necrosis or necroptosis. This function of RIPK1 is mediated by its protein serine/threonine kinase activity and through the downstream kinase RIPK3. Deletion of RIPK1 prevents embryonic lethality in mice lacking FADD, a signaling adaptor protein required for activation of Caspase 8 in extrinsic apoptotic pathways. This indicates that FADD-mediated apoptosis inhibits RIPK1-dependent necroptosis to ensure successful embryogenesis. However, the molecular mechanism for this critical regulation remains unclear. In the current study, a novel mouse model has been generated, by disrupting a potential caspase cleavage site at aspartic residue (D)324 in RIPK1. …


An Atypical Presentation Of A Rare Disease, Elizabeth Schoenberg, Kathleen P. Mcguinn, Deirdre Connolly, Sherry Yang Mar 2019

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 Mar 2019

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 Mar 2019

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 …


Probable Donor-Derived Human Adenovirus Type 34 Infection In 2 Kidney Transplant Recipients From The Same Donor., Matthew A. Pettengill, Tara M. Babu, Paritosh Prasad, Sally Chuang, Michael G. Drage, Marilyn Menegus, Daryl M. Lamson, Xiaoyan Lu, Dean Erdman, Nicole Pecora Mar 2019

Probable Donor-Derived Human Adenovirus Type 34 Infection In 2 Kidney Transplant Recipients From The Same Donor., Matthew A. Pettengill, Tara M. Babu, Paritosh Prasad, Sally Chuang, Michael G. Drage, Marilyn Menegus, Daryl M. Lamson, Xiaoyan Lu, Dean Erdman, Nicole Pecora

Department of Pathology, Anatomy, and Cell Biology Faculty Papers

Human adenovirus type 34 (HAdV-34) infection is a recognized cause of transplant-associated hemorrhagic cystitis and, in rare cases, tubulointerstitial nephritis. The source of such infections is often difficult to assess, that is, whether acquired as a primary infection, exposure to a pathogen in the transplanted organ, or reactivation of an endogenous latent infection. We present here 2 cases of likely transplant-acquired HAdV-34 infection from the same organ donor, manifesting as tubulointerstitial nephritis in 1.


Application Of Tasman Technique For Congenital Absence Of Nasal Cartilage: A Case Report, Dominick Gadaleta, Ryan N. Heffelfinger Mar 2019

Application Of Tasman Technique For Congenital Absence Of Nasal Cartilage: A Case Report, Dominick Gadaleta, Ryan N. Heffelfinger

Department of Otolaryngology - Head and Neck Surgery Faculty Papers

No abstract provided.


Intravenous Meloxicam For The Treatment Of Moderate To Severe Acute Pain: A Pooled Analysis Of Safety And Opioid-Reducing Effects., Eugene R. Viscusi, Tong J. Gan, Sergio Bergese, Neil Singla, Randall J. Mack, Stewart W. Mccallum, Wei Du, Sue Hobson Mar 2019

Intravenous Meloxicam For The Treatment Of Moderate To Severe Acute Pain: A Pooled Analysis Of Safety And Opioid-Reducing Effects., Eugene R. Viscusi, Tong J. Gan, Sergio Bergese, Neil Singla, Randall J. Mack, Stewart W. Mccallum, Wei Du, Sue Hobson

Department of Anesthesiology Faculty Papers

BACKGROUND AND OBJECTIVES: To describe the safety and tolerability of intravenous meloxicam compared with placebo across all phase II/III clinical trials.

METHODS: Safety data and opioid use from subjects with moderate to severe postoperative pain who received ≥1 dose of intravenous meloxicam (5-60 mg) or placebo in 1 of 7 studies (4 phase II; 3 phase III) were pooled. Data from intravenous meloxicam 5 mg, 7.5 mg and 15 mg groups were combined (low-dose subset).

RESULTS: A total of 1426 adults (86.6% white; mean age: 45.8 years) received ≥1 dose of meloxicam IV; 517 (77.6% white; mean age: 46.7 years) …


Genetic Susceptibility To Alopecia., Jouni Uitto Feb 2019

Genetic Susceptibility To Alopecia., Jouni Uitto

Department of Dermatology and Cutaneous Biology Faculty Papers

No abstract provided.


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 Feb 2019

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 …


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 Feb 2019

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 Feb 2019

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, …


Ephrin-B3 Controls Excitatory Synapse Density Through Cell-Cell Competition For Ephbs., Nathan T. Henderson, Sylvain J. Le Marchand, Martin Hruska, Simon Hippenmeyer, Liqun Luo, Matthew B. Dalva Feb 2019

Ephrin-B3 Controls Excitatory Synapse Density Through Cell-Cell Competition For Ephbs., Nathan T. Henderson, Sylvain J. Le Marchand, Martin Hruska, Simon Hippenmeyer, Liqun Luo, Matthew B. Dalva

Department of Neuroscience Faculty Papers

Cortical networks are characterized by sparse connectivity, with synapses found at only a subset of axo-dendritic contacts. Yet within these networks, neurons can exhibit high connection probabilities, suggesting that cell-intrinsic factors, not proximity, determine connectivity. Here, we identify ephrin-B3 (eB3) as a factor that determines synapse density by mediating a cell-cell competition that requires ephrin-B-EphB signaling. In a microisland culture system designed to isolate cell-cell competition, we find that eB3 determines winning and losing neurons in a contest for synapses. In a Mosaic Analysis with Double Markers (MADM) genetic mouse model system in vivo the relative levels of eB3 control …


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 Feb 2019

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 …


Maternal And Gestational Influences On Childhood Blood Pressure., Bonita Falkner Feb 2019

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 …


The Population Health Template: A Roadmap For Successful Health Improvement Initiatives, Michael Kobernick, Md, Ms-Hsa, Ms-Poph, Faafp Feb 2019

The Population Health Template: A Roadmap For Successful Health Improvement Initiatives, Michael Kobernick, Md, Ms-Hsa, Ms-Poph, Faafp

Population Health Leadership Series: PopTalk Webinars

Learning Objectives

  • Describe the population health template as a tool to achieve and report on Quintuple Aim objectives of health improvement initiatives
  • Identify gaps in current health initiatives illustrating the needs for the template's more organized approach
  • Apply the template to health improvement opportunities in health improvement initiatives
  • Understand the role of social determinants of health in health improvement initiatives

Presentation: 50:31


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 Feb 2019

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 …


The Voices Of Graduates: Informing Faculty Practices To Establish Best Practices For Readying Nclex-Rn Applicants, Mary Hanson-Zalot, Angela Gerolamo, Julia Ward Feb 2019

The Voices Of Graduates: Informing Faculty Practices To Establish Best Practices For Readying Nclex-Rn Applicants, Mary Hanson-Zalot, Angela Gerolamo, Julia Ward

College of Nursing Faculty Papers & Presentations

Changes in the National Council of State Boards of Nursing along with other factors influence graduates’ successful completion of a nursing program and the licensing examination. Literature is scarce in the area of examining stu-dent perceptions of preparing for and taking the NCLEX-RN examination. Our study sought to fill this gap in knowledge by conducting a focus group and interviews with individuals who passed the NCLEX-RN on their first at-tempt and those who did not. This was a descriptive qualitative study which used semi-structured interviews and a focus group to examine graduates’ perceptions related to preparing for and taking the …


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 Feb 2019

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 Feb 2019

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 Feb 2019

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 …


Endoscopic Laser-Ablation For The Treatment Of Orthotopic And Ectopic Ureteroceles In Dogs: 13 Cases (2008-2017)., Cleo P. Rogatko, Allyson C. Berent, Larry G. Adams, Chick W. Weisse, Demetrius H. Bagley Feb 2019

Endoscopic Laser-Ablation For The Treatment Of Orthotopic And Ectopic Ureteroceles In Dogs: 13 Cases (2008-2017)., Cleo P. Rogatko, Allyson C. Berent, Larry G. Adams, Chick W. Weisse, Demetrius H. Bagley

Department of Urology Faculty Papers

BACKGROUND: Ureteroceles are a rare condition in dogs in which conventional treatments can result in substantial morbidity. Cystoscopic and fluoroscopic-guided laser ablation (CLA) of ureteroceles can successfully relieve obstruction.

OBJECTIVES: To describe the technique and outcomes of attempting CLA for treatment of ureteroceles in dogs.

ANIMALS: Thirteen client-owned dogs that underwent CLA for treatment of ureteroceles.

METHODS: Retrospective multicentered study. Medical records were reviewed in all dogs that underwent CLA for ureterocele(s). A laser was used to extend the opening of the ureteral orifice (UO) unless surgical conversion was necessary. Data collected included signalment, clinicopathologic data, imaging, procedural findings, complications, …


Is Retinal Metabolic Dysfunction At The Center Of The Pathogenesis Of Age-Related Macular Degeneration?, Thierry Léveillard, Nancy J. Philp, Florian Sennlaub Feb 2019

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 …


Tradd Regulates Perinatal Development And Adulthood Survival In Mice Lacking Ripk1 And Ripk3., John P. Dowling, Mohamed Alsabbagh, Christina Del Casale, Zheng-Gang Liu, Jianke Zhang Feb 2019

Tradd Regulates Perinatal Development And Adulthood Survival In Mice Lacking Ripk1 And Ripk3., John P. Dowling, Mohamed Alsabbagh, Christina Del Casale, Zheng-Gang Liu, Jianke Zhang

Department of Microbiology and Immunology Faculty Papers

TRADD is an adaptor for TNFR1-induced apoptosis and NFκB activation. However, TRADD-deficient mice undergo normal development and contain normal lymphoid populations, which contrasts with an embryonic defect in mice lacking FADD, the shared adaptor mediating apoptosis. Recent studies indicate FADD suppresses embryonic necroptosis mediated by RIPK1. TRADD was suggested to also mediate necroptosis. Here we report that targeting TRADD fails to rescue Fadd −/− embryos from necroptosis, and ablation of TRADD rescues Ripk1 −/− mice from perinatal lethality when RIPK3-mediated necroptosis is disabled. The resulting Ripk1 −/− Ripk3 −/− Tradd −/− mice survive until early adulthood, but die thereafter. A …


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 Feb 2019

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 Feb 2019

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 Feb 2019

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 …