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Severe Septicaemia in a Patient with Polychondritis and Sweet's
81 LETTERS Ann Rheum Dis: first published as 10.1136/ard.62.1.88 on 1 January 2003. Downloaded from Severe septicaemia in a patient with polychondritis and Sweet’s syndrome after initiation of treatment with infliximab F G Matzkies, B Manger, M Schmitt-Haendle, T Nagel, H-G Kraetsch, J R Kalden, H Schulze-Koops ............................................................................................................................. Ann Rheum Dis 2003;62:81–82 D Sweet first described an acute febrile neutrophilic dermatosis in 1964 characterised by acute onset, fever, Rleucocytosis, and erythematous plaques.1 Skin biopsy specimens show infiltrates consisting of mononuclear cells and neutrophils with leucocytoclasis, but without signs of vasculi- tis. Sweet’s syndrome is frequently associated with solid malig- nancies or haemoproliferative disorders, but associations with chronic autoimmune connective tissue disorders have also been reported.2 The aetiology of Sweet’s syndrome is unknown, but evidence suggests that an immunological reaction of unknown specificity is the underlying mechanism. CASE REPORT A 51 year old white man with relapsing polychondritis (first diagnosed in 1997) was admitted to our hospital in June 2001 with a five week history of general malaise, fever, recurrent arthritis, and complaints of morning stiffness. Besides Figure 1 autoimmune polychondritis, he had insulin dependent Manifestation of Sweet’s syndrome in a patient with relapsing polychondritis. diabetes mellitus that was diagnosed in 1989. On admission, he presented with multiple small to medium, sharply demarked, raised erythematous plaques on both fore- dose of glucocorticoids (80 mg) and a second application of http://ard.bmj.com/ arms and lower legs, multiple acne-like pustules on the face, infliximab (3 mg/kg body weight) were given. -
Clinical Classification of Caroli's Disease: an Analysis of 30 Patients
View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector DOI:10.1111/hpb.12330 HPB ORIGINAL ARTICLE Clinical classification of Caroli's disease: an analysis of 30 patients Zhong-Xia Wang1,2*, Yong-Gang Li2*, Rui-Lin Wang2, Yong-Wu Li3, Zhi-Yan Li3, Li-Fu Wang2, Hui-Ying Yang2, Yun Zhu2, Yao Wang2, Yun-Feng Bai2, Ting-Ting He2, Xiao-Feng Zhang2 & Xiao-He Xiao1,2 1Department of Graduate School, 301 Hospital, 2Integrative Medical Centre, and 3Imaging Centre, 302 Hospital, Beijing, China Abstract Background: Caroli's disease (CD) is a rare congenital disorder. The early diagnosis of the disease and differentiation of types I and II are of extreme importance to patient survival. This study was designed to review and discuss observations in 30 patients with CD and to clarify the clinical characteristics of the disease. Methods: The demographic and clinical features, laboratory indicators, imaging findings and pathology results for 30 patients with CD were reviewed retrospectively. Results: Caroli's disease can occur at any age. The average age of onset in the study cohort was 24 years. Patients who presented with symptoms before the age of 40 years were more likely to develop type II CD. Approximately one-third of patients presented without positive signs at original diagnosis and most of these patients were found to have type I CD on pathology. Anaemia, leucopoenia and thrombocytopoenia were more frequent in patients with type II than type I CD. Magnetic resonance cholangiopancreatography (MRCP) and computed tomography (CT) examinations were most useful in diagnosing CD. -
Biliary Tract
2016-06-16 The role of cytology in management of diseases of hepatobiliary ducts • Diagnosis in patients with radiologically/clinically detected lesions • Screening of dysplasia/CIS/cancer in risk groups biliary tract cytology • Preoperative evaluation of the candidates for liver transplantation (Patients with cytological low-grade and high-grade Mehmet Akif Demir, MD dysplasia/adenocarcinoma are currently referred for liver transplantation Sahlgrenska University Hospital in some institutions). Gothenburg Sweden Sarajevo 18th June 2016 • Diagnosis of the benign lesions and infestations False positive findings • majority of false positive cases have a Low sensitivity but high specificity! background of primary sclerosing cholangitis. – lymphoplasmacytic sclerosing pancreatitis and cholangitis, – primary sclerosing cholangitis, – granulomatous disease, – non-specific fibrosis/inflammation – stone disease. False negative findings • Repeat brushing increases the diagnostic yield and should be performed when sampling • Poor sampling biliary strictures with a cytology brush at ERCP. • Lack of diagnostic criteria for dysplasia-carcinoma in situ • Difficulties in recognition of special tumour types – well-differentiated cholangiocarcinoma with tubular architecture • Predictors of positive yield include – gastric foveolar type cholangiocarcinoma with mucin-producing – tumour cells. older age, •Underestimating the significance of the smear background – mass size >1 cm, and – stricture length of >1 cm. •The causes of false negative cytology –sampling -
OCSHCN-10G, Medical Eligibility List for Clinical and Case Management Services.Pdf
OCSHCN-10g (01 2019) (Rev 7-15-2017) Office for Children with Special Health Care Needs Medical Eligibility List for Clinical and Case Management Services BODY SYSTEM ELIGIBLE DISEASES/CONDITIONS ICD-10-CM CODES AFFECTED AUTISM SPECTRUM Autistic disorder, current or active state F84.0 Autistic disorder DISORDER (ASD) F84.3 Other childhood disintegrative disorder Autistic disorder, residual state F84.5 Asperger’s Syndrome F84.8 Other pervasive developmental disorder Other specified pervasive developmental disorders, current or active state Other specified pervasive developmental disorders, residual state Unspecified pervasive development disorder, current or active Unspecified pervasive development disorder, residual state CARDIOVASCULAR Cardiac Dysrhythmias I47.0 Ventricular/Arrhythmia SYSTEM I47.1 Supraventricular/Tachycardia I47.2 Ventricular/Tachycardia I47.9 Paroxysmal/Tachycardia I48.0 Paroxysmal atrial fibrillation I48.1 Persistent atrial fibrillationar I48.2 Chronic atrial fibrillation I48.3 Typical atrial flutter I48.4 Atypical atrial flutter I49.0 Ventricular fibrillation and flutter I49.1 Atrial premature depolarization I49.2 Junctional premature depolarization I49.3 Ventricular premature depolarization I49.49 Ectopic beats Extrasystoles Extrasystolic arrhythmias Premature contractions Page 1 of 28 OCSHCN-10g (01 2019) (Rev 7-15-2017) Office for Children with Special Health Care Needs Medical Eligibility List for Clinical and Case Management Services I49.5 Tachycardia-Bradycardia Syndrome CARDIOVASCULAR Chronic pericarditis -
The National Economic Burden of Rare Disease Study February 2021
Acknowledgements This study was sponsored by the EveryLife Foundation for Rare Diseases and made possible through the collaborative efforts of the national rare disease community and key stakeholders. The EveryLife Foundation thanks all those who shared their expertise and insights to provide invaluable input to the study including: the Lewin Group, the EveryLife Community Congress membership, the Technical Advisory Group for this study, leadership from the National Center for Advancing Translational Sciences (NCATS) at the National Institutes of Health (NIH), the Undiagnosed Diseases Network (UDN), the Little Hercules Foundation, the Rare Disease Legislative Advocates (RDLA) Advisory Committee, SmithSolve, and our study funders. Most especially, we thank the members of our rare disease patient and caregiver community who participated in this effort and have helped to transform their lived experience into quantifiable data. LEWIN GROUP PROJECT STAFF Grace Yang, MPA, MA, Vice President Inna Cintina, PhD, Senior Consultant Matt Zhou, BS, Research Consultant Daniel Emont, MPH, Research Consultant Janice Lin, BS, Consultant Samuel Kallman, BA, BS, Research Consultant EVERYLIFE FOUNDATION PROJECT STAFF Annie Kennedy, BS, Chief of Policy and Advocacy Julia Jenkins, BA, Executive Director Jamie Sullivan, MPH, Director of Policy TECHNICAL ADVISORY GROUP Annie Kennedy, BS, Chief of Policy & Advocacy, EveryLife Foundation for Rare Diseases Anne Pariser, MD, Director, Office of Rare Diseases Research, National Center for Advancing Translational Sciences (NCATS), National Institutes of Health Elisabeth M. Oehrlein, PhD, MS, Senior Director, Research and Programs, National Health Council Christina Hartman, Senior Director of Advocacy, The Assistance Fund Kathleen Stratton, National Academies of Science, Engineering and Medicine (NASEM) Steve Silvestri, Director, Government Affairs, Neurocrine Biosciences Inc. -
Cardiothoracic Fellowship Program
Cardiothoracic Fellowship Program Table of Contents Program Contact ............................................................................................ 3 Other contact numbers .................................................................................. 4 Introduction ........................................................................................................... 5 Goals and Objectives of Fellowship: ..................................................................... 6 Rotation Schedule: ........................................................................................ 7 Core Curriculum .................................................................................................... 8 Fellow’s Responsibilities ..................................................................................... 22 Resources ........................................................................................................... 23 Facilities ....................................................................................................... 23 Educational Program .......................................................................................... 26 Duty Hours .......................................................................................................... 29 Evaluation ........................................................................................................... 30 Table of Appendices .................................................................................... 31 Appendix A -
Screening Chest X-Ray Interpretations and Radiographic Techniques IOM GUIDELINES FIRST EDITION Iii
FIRST EDITION 2015 Screening Chest X-Ray Interpretations and Radiographic Techniques IOM GUIDELINES Global Radiology Coordination and Teleradiology Centre Migration Health Division International Organization for Migration (Manila Administrative Centre) 24th floor Citibank Tower, Paseo De Roxas 8741, Makati city 1226 Metro Manila, Philippines Email: [email protected] • [email protected] Tel: +632 230 1674 The opinions expressed in the report are those of the authors and do not necessarily reflect the views of the International Organization for Migration (IOM). The designations employed and the presentation of material throughout the report do not imply the expression of any opinion whatsoever on the part of IOM concerning the legal status of any country, territory, city or area, or of its authorities, or concerning its frontiers or boundaries. IOM is committed to the principle that humane and orderly migration benefits migrants and society. As an intergovernmental organization, IOM acts with its partners in the international community to: assist in meeting the operational challenges of migration; advance understanding of migration issues; encourage social and economic development through migration; and uphold the human dignity and well-being of migrants. Author Sifrash Meseret GELAW, MD Radiologist, MPH; Global Radiology Coordinator IOM, Manila Administrative Centre, Manila, Philippines Major Contributor Anthony MACDERMOTT, MD former Global HAP Quality Coordinator, IOM, Regional Office for Asia and the Pacific, Bangkok, Thailand Additional -
Orthopedic-Conditions-Treated.Pdf
Orthopedic and Orthopedic Surgery Conditions Treated Accessory navicular bone Achondroplasia ACL injury Acromioclavicular (AC) joint Acromioclavicular (AC) joint Adamantinoma arthritis sprain Aneurysmal bone cyst Angiosarcoma Ankle arthritis Apophysitis Arthrogryposis Aseptic necrosis Askin tumor Avascular necrosis Benign bone tumor Biceps tear Biceps tendinitis Blount’s disease Bone cancer Bone metastasis Bowlegged deformity Brachial plexus injury Brittle bone disease Broken ankle/broken foot Broken arm Broken collarbone Broken leg Broken wrist/broken hand Bunions Carpal tunnel syndrome Cavovarus foot deformity Cavus foot Cerebral palsy Cervical myelopathy Cervical radiculopathy Charcot-Marie-Tooth disease Chondrosarcoma Chordoma Chronic regional multifocal osteomyelitis Clubfoot Congenital hand deformities Congenital myasthenic syndromes Congenital pseudoarthrosis Contractures Desmoid tumors Discoid meniscus Dislocated elbow Dislocated shoulder Dislocation Dislocation – hip Dislocation – knee Dupuytren's contracture Early-onset scoliosis Ehlers-Danlos syndrome Elbow fracture Elbow impingement Elbow instability Elbow loose body Eosinophilic granuloma Epiphyseal dysplasia Ewing sarcoma Extra finger/toes Failed total hip replacement Failed total knee replacement Femoral nonunion Fibrosarcoma Fibrous dysplasia Fibular hemimelia Flatfeet Foot deformities Foot injuries Ganglion cyst Genu valgum Genu varum Giant cell tumor Golfer's elbow Gorham’s disease Growth plate arrest Growth plate fractures Hammertoe and mallet toe Heel cord contracture -
An Unusually Dry Story
555050 Case Report An unusually dry story Srinivas Rajagopala, Gurukiran Danigeti, Dharanipragada Subrahmanyan We present a middle-aged woman with a prior history of central nervous system (CNS) Access this article online demyelinating disorder who presented with an acute onset quadriparesis and respiratory Website: www.ijccm.org failure. The evaluation revealed distal renal tubular acidosis with hypokalemia and DOI: 10.4103/0972-5229.164808 medullary nephrocalcinosis. Weakness persisted despite potassium correction, and ongoing Quick Response Code: Abstract evaluation confi rmed recurrent CNS and long-segment spinal cord demyelination with anti-aquaporin-4 antibodies. There was no history of dry eyes or dry mouth. Anti-Sjogren’s syndrome A antigen antibodies were elevated, and there was reduced salivary fl ow on scintigraphy. Coexistent antiphospholipid antibody syndrome with inferior vena cava thrombosis was also found on evaluation. The index patient highlights several rare manifestations of primary Sjogren’s syndrome (pSS) as the presenting features and highlights the differential diagnosis of the clinical syndromes in which pSS should be considered in the Intensive Care Unit. Keywords: Acute demyelinating encephalomyelitis, distal renal tubular acidosis, hypokalemic paralysis, nephrocalcinosis, neuromyelitis optica, Sjogren’s syndrome Introduction was diagnosed with postviral encephalomyelitis 3 years ago and treated with steroids with complete resolution of Primary Sjogren’s syndrome (pSS) is a relatively symptoms and signs. She had normal menstrual cycles common autoimmune disease affecting 2–3% of the adult and had one spontaneous second trimester abortion population. It is characterized by lymphocyte infi ltration 8 years ago. She had one living child and had undergone and destruction of exocrine glands. -
A Resident's Guide to Pediatric Rheumatology
A RESIDENT’S GUIDE TO PEDIATRIC RHEUMATOLOGY 4th Revised Edition - 2019 A RESIDENT’S GUIDE TO PEDIATRIC RHEUMATOLOGY This guide is intended to provide a brief introduction to basic topics in pediatric rheumatology. Each topic is accompanied by at least one up-to-date reference that will allow you to explore the topic in greater depth. In addition, a list of several excellent textbooks and other resources for you to use to expand your knowledge is found in the Appendix. We are interested in your feedback on the guide! If you have comments or questions, please feel free to contact us via email at [email protected]. Supervising Editors: Dr. Ronald M. Laxer, SickKids Hospital, University of Toronto Dr. Tania Cellucci, McMaster Children’s Hospital, McMaster University Dr. Evelyn Rozenblyum, St. Michael’s Hospital, University of Toronto Section Editors: Dr. Michelle Batthish, McMaster Children’s Hospital, McMaster University Dr. Roberta Berard, Children’s Hospital – London Health Sciences Centre, Western University Dr. Liane Heale, McMaster Children’s Hospital, McMaster University Dr. Clare Hutchinson, North York General Hospital, University of Toronto Dr. Mehul Jariwala, Royal University Hospital, University of Saskatchewan Dr. Lillian Lim, Stollery Children’s Hospital, University of Alberta Dr. Nadia Luca, Alberta Children’s Hospital, University of Calgary Dr. Dax Rumsey, Stollery Children’s Hospital, University of Alberta Dr. Gordon Soon, North York General Hospital and SickKids Hospital Northern Clinic in Sudbury, University -
Diagnostic Imaging and Risk Factors Downloaded from by EERP/BIBLIOTECA CENTRAL User on 26 August 2019
ISSN 2472-1972 Nephrocalcinosis and Nephrolithiasis in X-Linked Hypophosphatemic Rickets: Diagnostic Imaging and Risk Factors Downloaded from https://academic.oup.com/jes/article-abstract/3/5/1053/5418933 by EERP/BIBLIOTECA CENTRAL user on 26 August 2019 Guido de Paula Colares Neto,1,2 Fernando Ide Yamauchi,3 Ronaldo Hueb Baroni,3 Marco de Andrade Bianchi,4 Andrea Cavalanti Gomes,4 Maria Cristina Chammas,4 and Regina Matsunaga Martin1,2 1Department of Internal Medicine, Division of Endocrinology, Osteometabolic Disorders Unit, Hospital das Cl´ınicas da Faculdade de Medicina da Universidade de S~ao Paulo, 05403-900 S~ao Paulo, SP, Brazil; 2Department of Internal Medicine, Division of Endocrinology, Laborat´orio de Hormoniosˆ e Gen´etica Molecular (LIM/42), Hospital das Cl´ınicas da Faculdade de Medicina da Universidade de S~ao Paulo, 05403-900 S~ao Paulo, SP, Brazil; 3Department of Radiology and Oncology, Division of Radiology, Computed Tomography Unit, Hospital das Cl´ınicas da Faculdade de Medicina da Universidade de S~ao Paulo, 05403-001 S~ao Paulo, SP, Brazil; and 4Department of Radiology and Oncology, Division of Radiology, Ultrasound Unit, Hospital das Cl´ınicas da Faculdade de Medicina da Universidade de S~ao Paulo, 05403-001 S~ao Paulo, SP, Brazil ORCiD numbers: 0000-0003-3355-0386 (G. P. Colares Neto); 0000-0002-4633-3711 (F. I. Yamauchi); 0000-0001-7041-3079 (M. C. Chammas). Context: Nephrocalcinosis (NC) and nephrolithiasis (NL) are described in hypophosphatemic rickets, but data regarding their prevalence rates and the presence of metabolic risk factors in X-linked hypophosphatemic rickets (XLH) are scarce. -
New Jersey Chapter American College of Physicians
NEW JERSEY CHAPTER AMERICAN COLLEGE OF PHYSICIANS ASSOCIATES ABSTRACT COMPETITION 2015 SUBMISSIONS 2015 Resident/Fellow Abstracts 1 1. ID CATEGORY NAME ADDITIONAL PROGRAM ABSTRACT AUTHORS 2. 295 Clinical Abed, Kareem Viren Vankawala MD Atlanticare Intrapulmonary Arteriovenous Malformation causing Recurrent Cerebral Emboli Vignette FACC; Qi Sun MD Regional Medical Ischemic strokes are mainly due to cardioembolic occlusion of small vessels, as well as large vessel thromboemboli. We describe a Center case of intrapulmonary A-V shunt as the etiology of an acute ischemic event. A 63 year old male with a past history of (Dominik supraventricular tachycardia and recurrent deep vein thrombosis; who has been non-compliant on Rivaroxaban, presents with Zampino) pleuritic chest pain and was found to have a right lower lobe pulmonary embolus. The deep vein thrombosis and pulmonary embolus were not significant enough to warrant ultrasound-enhanced thrombolysis by Ekosonic EndoWave Infusion Catheter System, and the patient was subsequently restarted on Rivaroxaban and discharged. The patient presented five days later with left arm tightness and was found to have multiple areas of punctuate infarction of both cerebellar hemispheres, more confluent within the right frontal lobe. Of note he was compliant at this time with Rivaroxaban. The patient was started on unfractionated heparin drip and subsequently admitted. On admission, his vital signs showed a blood pressure of 138/93, heart rate 65 bpm, and respiratory rate 16. Cardiopulmonary examination revealed regular rate and rhythm, without murmurs, rubs or gallops and his lungs were clear to auscultation. Neurologic examination revealed intact cranial nerves, preserved strength in all extremities, mild dysmetria in the left upper extremity and an NIH score of 1.