Experimental Hemarthrosis in Rhesus Monkeys: Light Microscopic, Scanning and Transmission Electron Microscopic, Biochemical, Metabolic and Morphometric Analyses
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WFH Treatment Guidelines 3Ed Chapter 7 Treatment Of
96 TREATMENT OF SPECIFIC 7 HEMORRHAGES Johnny Mahlangu1 | Gerard Dolan2 | Alison Dougall3 | Nicholas J. Goddard4 | Enrique D. Preza Hernández5 | Margaret V. Ragni6 | Bradley Rayner7 | Jerzy Windyga8 | Glenn F. Pierce9 | Alok Srivastava10 1 Department of Molecular Medicine and Haematology, University of the Witwatersrand, National Health Laboratory Service, Johannesburg, South Africa 2 Guy’s and St. Thomas’ Hospitals NHS Foundation Trust, London, UK 3 Special Care Dentistry Division of Child and Public Dental Health, School of Dental Science, Trinity College Dublin, Dublin Dental University Hospital, Dublin, Ireland 4 Department of Trauma and Orthopaedics, Royal Free Hospital, London, UK 5 Mexico City, Mexico 6 Division of Hematology/Oncology, Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA 7 Cape Town, South Africa 8 Department of Hemostasis Disorders and Internal Medicine, Laboratory of Hemostasis and Metabolic Diseases, Institute of Hematology and Transfusion Medicine, Warsaw, Poland 9 World Federation of Hemophilia, Montreal, QC, Canada 10 Department of Haematology, Christian Medical College, Vellore, India All statements identified as recommendations are • In general, the main treatment for bleeding episodes in consensus based, as denoted by CB. patients with severe hemophilia is prompt clotting factor replacement therapy and rehabilitation. However, different types of bleeds and bleeding at particular anatomical sites 7.1 Introduction may require more specific management with additional -
Updated Treatment for Calcium Pyrophosphate Deposition Disease: an Insight
Open Access Review Article DOI: 10.7759/cureus.3840 Updated Treatment for Calcium Pyrophosphate Deposition Disease: An Insight Shumaila M. Iqbal 1 , Sana Qadir 2 , Hafiz M. Aslam 3 , Madiha A. Qadir 4 1. Internal Medicine, University at Buffalo / Sisters of Charity Hospital, Buffalo, USA 2. Internal Medicine, S & A Pediatrics, Parsippany, USA 3. Internal Medicine, Seton Hall University / Hackensack Meridian School of Medicine, Trenton, USA 4. Internal Medicine, Jinnah Sindh Medical University, Karachi, PAK Corresponding author: Shumaila M. Iqbal, [email protected] Abstract Calcium pyrophosphate disease (CPPD) is caused by the deposition of calcium pyrophosphate (CPP) crystals in the joint tissues, particularly fibrocartilage and hyaline cartilage. CPP crystals trigger inflammation, causing local articular tissue damage. Our review article below covers different aspects of CPPD. It discusses how CPPD can manifest as different kinds of arthritis, which may be symptomatic or asymptomatic. The metabolic and endocrine disease associations and routine investigations used in the diagnostic workup are briefly reviewed. Conventional and newer therapies for the treatment of CPPD are outlined. Overall, this extensive review would provide an updated insight to clinicians for evidence-based treatment of CPPD. Categories: Rheumatology Keywords: calcium pyrophosphate deposition disease, calcium pyrophosphate crystal, arthritis, colchicine, immunomodulants, nsaid Introduction And Background Calcium pyrophosphate deposition disease (CPPD) is caused by the deposition of calcium pyrophosphate (CCP) crystals in the articular cartilage, resulting in inflammation and degenerative changes in the affected joint. CPPD is a collective term that comprises all the various forms of CPP crystal-induced arthropathies. The acute arthritis attack caused by CPP crystals deposition triggers the same inflammatory reaction in the joint tissues as do the monosodium urate crystals in gout patients. -
Familial Multiple Coagulation Factor Deficiencies
Journal of Clinical Medicine Article Familial Multiple Coagulation Factor Deficiencies (FMCFDs) in a Large Cohort of Patients—A Single-Center Experience in Genetic Diagnosis Barbara Preisler 1,†, Behnaz Pezeshkpoor 1,† , Atanas Banchev 2 , Ronald Fischer 3, Barbara Zieger 4, Ute Scholz 5, Heiko Rühl 1, Bettina Kemkes-Matthes 6, Ursula Schmitt 7, Antje Redlich 8 , Sule Unal 9 , Hans-Jürgen Laws 10, Martin Olivieri 11 , Johannes Oldenburg 1 and Anna Pavlova 1,* 1 Institute of Experimental Hematology and Transfusion Medicine, University Clinic Bonn, 53127 Bonn, Germany; [email protected] (B.P.); [email protected] (B.P.); [email protected] (H.R.); [email protected] (J.O.) 2 Department of Paediatric Haematology and Oncology, University Hospital “Tzaritza Giovanna—ISUL”, 1527 Sofia, Bulgaria; [email protected] 3 Hemophilia Care Center, SRH Kurpfalzkrankenhaus Heidelberg, 69123 Heidelberg, Germany; ronald.fi[email protected] 4 Department of Pediatrics and Adolescent Medicine, University Medical Center–University of Freiburg, 79106 Freiburg, Germany; [email protected] 5 Center of Hemostasis, MVZ Labor Leipzig, 04289 Leipzig, Germany; [email protected] 6 Hemostasis Center, Justus Liebig University Giessen, 35392 Giessen, Germany; [email protected] 7 Center of Hemostasis Berlin, 10789 Berlin-Schöneberg, Germany; [email protected] 8 Pediatric Oncology Department, Otto von Guericke University Children’s Hospital Magdeburg, 39120 Magdeburg, Germany; [email protected] 9 Division of Pediatric Hematology Ankara, Hacettepe University, 06100 Ankara, Turkey; Citation: Preisler, B.; Pezeshkpoor, [email protected] B.; Banchev, A.; Fischer, R.; Zieger, B.; 10 Department of Pediatric Oncology, Hematology and Clinical Immunology, University of Duesseldorf, Scholz, U.; Rühl, H.; Kemkes-Matthes, 40225 Duesseldorf, Germany; [email protected] B.; Schmitt, U.; Redlich, A.; et al. -
Immune-Pathophysiology and -Therapy of Childhood Purpura
Egypt J Pediatr Allergy Immunol 2009;7(1):3-13. Review article Immune-pathophysiology and -therapy of childhood purpura Safinaz A Elhabashy Professor of Pediatrics, Ain Shams University, Cairo Childhood purpura - Overview vasculitic disorders present with palpable Purpura (from the Latin, purpura, meaning purpura2. Purpura may be secondary to "purple") is the appearance of red or purple thrombocytopenia, platelet dysfunction, discolorations on the skin that do not blanch on coagulation factor deficiency or vascular defect as applying pressure. They are caused by bleeding shown in table 1. underneath the skin. Purpura measure 0.3-1cm, A thorough history (Table 2) and a careful while petechiae measure less than 3mm and physical examination (Table 3) are critical first ecchymoses greater than 1cm1. The integrity of steps in the evaluation of children with purpura3. the vascular system depends on three interacting When the history and physical examination elements: platelets, plasma coagulation factors suggest the presence of a bleeding disorder, and blood vessels. All three elements are required laboratory screening studies may include a for proper hemostasis, but the pattern of bleeding complete blood count, peripheral blood smear, depends to some extent on the specific defect. In prothrombin time (PT) and activated partial general, platelet disorders manifest petechiae, thromboplastin time (aPTT). With few exceptions, mucosal bleeding (wet purpura) or, rarely, central these studies should identify most hemostatic nervous system bleeding; -
ACR Appropriateness Criteria® Chronic Knee Pain
Revised 2018 American College of Radiology ACR Appropriateness Criteria® Chronic Knee Pain Variant 1: Adult or child greater than or equal to 5 years of age. Chronic knee pain. Initial imaging. Procedure Appropriateness Category Relative Radiation Level Radiography knee Usually Appropriate ☢ Image-guided aspiration knee Usually Not Appropriate Varies CT arthrography knee Usually Not Appropriate ☢ CT knee with IV contrast Usually Not Appropriate ☢ CT knee without and with IV contrast Usually Not Appropriate ☢ CT knee without IV contrast Usually Not Appropriate ☢ MR arthrography knee Usually Not Appropriate O MRI knee without and with IV contrast Usually Not Appropriate O MRI knee without IV contrast Usually Not Appropriate O Bone scan knee Usually Not Appropriate ☢☢☢ US knee Usually Not Appropriate O Radiography hip ipsilateral Usually Not Appropriate ☢☢☢ Variant 2: Adult or child greater than or equal to 5 years of age. Chronic knee pain. Initial knee radiograph negative or demonstrates joint effusion. Next imaging procedure. Procedure Appropriateness Category Relative Radiation Level MRI knee without IV contrast Usually Appropriate O Image-guided aspiration knee May Be Appropriate Varies CT arthrography knee May Be Appropriate ☢ CT knee without IV contrast May Be Appropriate ☢ US knee May Be Appropriate (Disagreement) O Radiography hip ipsilateral May Be Appropriate ☢☢☢ Radiography lumbar spine May Be Appropriate ☢☢☢ MR arthrography knee May Be Appropriate O MRI knee without and with IV contrast Usually Not Appropriate O CT knee with IV contrast Usually Not Appropriate ☢ CT knee without and with IV contrast Usually Not Appropriate ☢ Bone scan knee Usually Not Appropriate ☢☢☢ ACR Appropriateness Criteria® 1 Chronic Knee Pain Variant 3: Adult or child greater than or equal to 5 years of age. -
Imaging in Gout and Other Crystal-Related Arthropathies 625
ImaginginGoutand Other Crystal-Related Arthropathies a, b Patrick Omoumi, MD, MSc, PhD *, Pascal Zufferey, MD , c b Jacques Malghem, MD , Alexander So, FRCP, PhD KEYWORDS Gout Crystal arthropathy Calcification Imaging Radiography Ultrasound Dual-energy CT MRI KEY POINTS Crystal deposits in and around the joints are common and most often encountered as inci- dental imaging findings in asymptomatic patients. In the chronic setting, imaging features of crystal arthropathies are usually characteristic and allow the differentiation of the type of crystal arthropathy, whereas in the acute phase and in early stages, imaging signs are often nonspecific, and the final diagnosis still relies on the analysis of synovial fluid. Radiography remains the primary imaging tool in the workup of these conditions; ultra- sound has been playing an increasing role for superficially located crystal-induced ar- thropathies, and computerized tomography (CT) is a nice complement to radiography for deeper sites. When performed in the acute stage, MRI may show severe inflammatory changes that could be misleading; correlation to radiographs or CT should help to distinguish crystal arthropathies from infectious or tumoral conditions. Dual-energy CT is a promising tool for the characterization of crystal arthropathies, partic- ularly gout as it permits a quantitative assessment of deposits, and may help in the follow-up of patients. INTRODUCTION The deposition of microcrystals within and around the joint is a common phenomenon. Intra-articular microcrystals -
2004400 October 2004, Change Report and NCD Coding Policy
Medicare National Coverage Determinations (NCD) Coding Policy Manual and Change Report April 2012 Clinical Diagnostic Laboratory Services Health & Human Services Department Centers for Medicare & Medicaid Services 7500 Security Boulevard Baltimore, MD 21244 CMS Email Point of Contact: [email protected] TDD 410.786.0727 Fu Associates, Ltd. Medicare National Coverage Determinations (NCD) Coding Policy Manual and Change Report This is CMS Logo. NCD Manual Changes Date Reason Release Change Edit The following section represents NCD Manual updates for April 2012. *04/01/12 *ICD-9-cm code range *2012200 *376.21-376.22 *(190.15) Blood Counts and descriptors Endocrine exophthalmos revised 376.21-376.9 Disorders of the orbit, *376.40-376.47 except 376.3 Other Deformity of orbit exophthalmic conditions (underlining *376.50-376.52 in original) Enophthalmos *376.6 Retained (old) foreign body following penetrating wound of orbit *376.81-376.82 Orbital cysts; myopathy of extraocular muscles *376.89 Other orbital disorders *376.9 Unspecified disorder of orbit The following section represents NCD Manual updates for January 2012. 01/01/12 Per CR 7621 add ICD-9- 2012100 786.50 Chest pain, (190.17) Prothrombin Time CM codes 786.50 and unspecified (PT) 786.51 to the list of ICD- 9-CM codes that are 786.51 Precordial pain covered by Medicare for the Prothrombin Time (PT) (190.17) NCD. Transmittal # 2344 01/01/12 Per CR 7621 delete ICD- 2012100 425.11 Hypertrophic (190.25) Alpha-fetoprotein 9-CM codes 425.11 and obstructive cardiomyopathy 425.18 from the list of ICD-9-CM codes that are 425.18 Other hypertrophic covered by Medicare for cardiomyopathy the Alpha-fetoprotein (190.25) NCD. -
ARTICULAR BLEEDING (HEMARTHROSIS) in HEMOPHILIA an ORTHOPEDIST’S POINT of VIEW Second Edition
TREATMENT OF HEMOPHILIA April 2008 · No. 23 ARTICULAR BLEEDING (HEMARTHROSIS) IN HEMOPHILIA AN ORTHOPEDIST’S POINT OF VIEW Second edition E. C. Rodríguez-Merchán Consultant Orthopedic Surgeon La Paz University Hospital Madrid, Spain Associate Professor of Orthopedics University Autonoma Madrid, Spain Published by the World Federation of Hemophilia (WFH), 2000; revised 2008. © Copyright World Federation of Hemophilia, 2008 The WFH encourages redistribution of its publications for educational purposes by not-for-profit hemophilia organizations. In order to obtain permission to reprint, redistribute, or translate this publication, please contact the Programs and Education Department at the address below. This publication is accessible from the World Federation of Hemophilia’s eLearning Platform at eLearning.wfh.org Additional copies are also available from the WFH at: World Federation of Hemophilia 1425 René Lévesque Boulevard West, Suite 1010 Montréal, Québec H3G 1T7 CANADA Tel. : (514) 875-7944 Fax : (514) 875-8916 E-mail: [email protected] Internet: www.wfh.org The Treatment of Hemophilia series is intended to provide general information on the treatment and management of hemophilia. The World Federation of Hemophilia does not engage in the practice of medicine and under no circumstances recommends particular treatment for specific individuals. Dose schedules and other treatment regimes are continually revised and new side-effects recognized. WFH makes no representation, express or implied, that drug doses or other treatment recommendations in this publication are correct. For these reasons it is strongly recommended that individuals seek the advice of a medical adviser and/or to consult printed instructions provided by the pharmaceutical company before administering any of the drugs referred to in this monograph. -
21362 Arthritis Australia a to Z List
ARTHRITISINFORMATION SHEET Here is the A to Z of arthritis! A D Goodpasture’s syndrome Achilles tendonitis Degenerative joint disease Gout Achondroplasia Dermatomyositis Granulomatous arteritis Acromegalic arthropathy Diabetic finger sclerosis Adhesive capsulitis Diffuse idiopathic skeletal H Adult onset Still’s disease hyperostosis (DISH) Hemarthrosis Ankylosing spondylitis Discitis Hemochromatosis Anserine bursitis Discoid lupus erythematosus Henoch-Schonlein purpura Avascular necrosis Drug-induced lupus Hepatitis B surface antigen disease Duchenne’s muscular dystrophy Hip dysplasia B Dupuytren’s contracture Hurler syndrome Behcet’s syndrome Hypermobility syndrome Bicipital tendonitis E Hypersensitivity vasculitis Blount’s disease Ehlers-Danlos syndrome Hypertrophic osteoarthropathy Brucellar spondylitis Enteropathic arthritis Bursitis Epicondylitis I Erosive inflammatory osteoarthritis Immune complex disease C Exercise-induced compartment Impingement syndrome Calcaneal bursitis syndrome Calcium pyrophosphate dehydrate J (CPPD) F Jaccoud’s arthropathy Crystal deposition disease Fabry’s disease Juvenile ankylosing spondylitis Caplan’s syndrome Familial Mediterranean fever Juvenile dermatomyositis Carpal tunnel syndrome Farber’s lipogranulomatosis Juvenile rheumatoid arthritis Chondrocalcinosis Felty’s syndrome Chondromalacia patellae Fibromyalgia K Chronic synovitis Fifth’s disease Kawasaki disease Chronic recurrent multifocal Flat feet Kienbock’s disease osteomyelitis Foreign body synovitis Churg-Strauss syndrome Freiberg’s disease -
The Need for Comprehensive Care for Persons with Chronic Immune Thrombocytopenic Purpura
Children's Mercy Kansas City SHARE @ Children's Mercy Manuscripts, Articles, Book Chapters and Other Papers 12-2020 The Need for Comprehensive Care for Persons with Chronic Immune Thrombocytopenic Purpura. Kristin T. Ansteatt Chanel J. Unzicker Marsha L. Hurn Oluwaseun O. Olaiya Children's Mercy Hospital Diane J. Nugent See next page for additional authors Follow this and additional works at: https://scholarlyexchange.childrensmercy.org/papers Recommended Citation Ansteatt KT, Unzicker CJ, Hurn ML, Olaiya OO, Nugent DJ, Tarantino MD. The Need for Comprehensive Care for Persons with Chronic Immune Thrombocytopenic Purpura. J Blood Med. 2020;11:457-463. Published 2020 Dec 17. doi:10.2147/JBM.S289390 This Article is brought to you for free and open access by SHARE @ Children's Mercy. It has been accepted for inclusion in Manuscripts, Articles, Book Chapters and Other Papers by an authorized administrator of SHARE @ Children's Mercy. For more information, please contact [email protected]. Creator(s) Kristin T. Ansteatt, Chanel J. Unzicker, Marsha L. Hurn, Oluwaseun O. Olaiya, Diane J. Nugent, and Michael D. Tarantino This article is available at SHARE @ Children's Mercy: https://scholarlyexchange.childrensmercy.org/papers/2899 Journal of Blood Medicine Dovepress open access to scientific and medical research Open Access Full Text Article REVIEW The Need for Comprehensive Care for Persons with Chronic Immune Thrombocytopenic Purpura This article was published in the following Dove Press journal: Journal of Blood Medicine Kristin T Ansteatt 1 Abstract: Chronic platelet disorders (CPD), including chronic immune thrombocytopenic Chanel J Unzicker1 purpura (cITP), thrombotic thrombocytopenic purpura (TTP) and platelet function disorders Marsha L Hurn1 are among the most common bleeding disorders and are associated with morbidity and Oluwaseun O Olaiya2 mortality. -
Thrombophilia (Hypercoagulable State)
Thrombophilia (Hypercoagulable state) 435 medicine teamwork [ Important | Notes | Extra | Editing file ] lecture objectives: ⇨ Not given Done By: Khawla Alammari, Abdulrahman Alshammari & Rawan Aldhuwayhi Edited By: Rahaf Binabbad References: Slides + Davidson + 434 team Thrombophilia - Balance of homeostasis : - - Balance of bleeding and clotting. - Imbalance in one direction can lead to: 1. Hypocoagulable state → bleeding 2. Hypercoagulable state → thrombosis The major components of the hemostatic system are : 1. The vessel wall. 2. Platelets (and other blood elements). 3. Plasma proteins (coagulation and fibrinolytic factors). Stages of normal hemostasis Pre-injury Fibrin clot formation Limiting clot formation Fibrinolysis • The endothelium produces •The endothelium is •Once hemostasis has been •The insoluble clot needs to be broken anti-thrombotic agents. (thus injured. secured, the propagation of down for vessel recanalization. platelets & coagulation factors •Platelets are activated. clot is halted by natural •Plasminogen is activated by tissue circulate in non-activated •Then Coagulation cascade anticoagulants. plasminogen activator state) is activated. (t-PA)→plasmin. •Fibrin clot forms. •Plasmin hydrolyse the fibrin clot, producing fibrin degradation factors including D-dimer. Natural anticoagulants Antithrombin Tissue factor pathway inhibitor Protein C & S •Serine protease inhibitor synthesized by the •Binds to and inactivates VIIa & Xa. •Protein C is activated through binding of liver. thrombin to thrombomodulin (structure on the •Destroys activated factors such as XIa, Xa & endothelium), activated protein C binds to its thrombin (IIa). co-factor protein S → then cleaves Va & VIIIa. •Both protein C & S are K-dependent and are depleted by warfarin. ★ Take a deep breath & appreciate this graph while paying attention to the red & green dotted arrows. Antithrombotic functions of endothelium: 1 Prostacyclin (PGI2) Prevents formation of the platelet plug. -
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Leyens et al. Orphanet J Rare Dis (2021) 16:326 https://doi.org/10.1186/s13023-021-01945-8 RESEARCH Open Access The combined prevalence of classifed rare rheumatic diseases is almost double that of ankylosing spondylitis Judith Leyens1,2, Tim Th. A. Bender1,3, Martin Mücke1, Christiane Stieber4, Dmitrij Kravchenko1,5, Christian Dernbach6 and Matthias F. Seidel7* Abstract Background: Rare diseases (RDs) afect less than 5/10,000 people in Europe and fewer than 200,000 individuals in the United States. In rheumatology, RDs are heterogeneous and lack systemic classifcation. Clinical courses involve a variety of diverse symptoms, and patients may be misdiagnosed and not receive appropriate treatment. The objec- tive of this study was to identify and classify some of the most important RDs in rheumatology. We also attempted to determine their combined prevalence to more precisely defne this area of rheumatology and increase awareness of RDs in healthcare systems. We conducted a comprehensive literature search and analyzed each disease for the speci- fed criteria, such as clinical symptoms, treatment regimens, prognoses, and point prevalences. If no epidemiological data were available, we estimated the prevalence as 1/1,000,000. The total point prevalence for all RDs in rheumatol- ogy was estimated as the sum of the individually determined prevalences. Results: A total of 76 syndromes and diseases were identifed, including vasculitis/vasculopathy (n 15), arthritis/ arthropathy (n 11), autoinfammatory syndromes (n 11), myositis (n 9), bone disorders (n 11),= connective tissue diseases =(n 8), overgrowth syndromes (n 3), =and others (n 8).= Out of the 76 diseases,= 61 (80%) are clas- sifed as chronic, with= a remitting-relapsing course= in 27 cases (35%)= upon adequate treatment.