Complement deficiencies, diagnosis and management. Contents
|
|
- Byron Scott
- 5 years ago
- Views:
Transcription
1 Complement deficiencies, diagnosis and management Classification: Protocol Lead Author: Dr Hana Alachkar Additional author(s): Victoria Blakeley Authors Division: Tertiary Medicine Unique ID: D5 Issue number: 4 Expiry Date: June 2019 Contents Section Page Who should read this document 2 Key points 2 Background/ Scope 2 What is new in this version 2 Policy/Procedure/Guideline 2 1 Use clear section headings 2 Include hyperlinks if you think they will help navigation 3 etc Standards 5 Explanation of terms/ Definitions 5 References and Supporting Documents 6 Roles and Responsibilities Appendix Document control information (Published as separate document) Document Control 7 Policy Implementation Plan 7 Monitoring and Review 8 Endorsement 8 Equality analysis 9 Page 1 of 6
2 Who should read this document? Immunology medical team Immunology Nurses Other teams in the trust as a reference/guideline Key Messages This protocol describes the evaluation and management of patients with suspected complement deficiency to ensure that diagnostic tests and treatment meets the needs and requirements of such patients and conforms to accepted national guidelines Background & Scope This protocol describes the evaluation and management of patients with suspected complement deficiency to ensure that diagnostic tests and treatment meets the needs and requirements of such patients and conforms to accepted national guidelines What is new in this version? This is an updated version but there are no important changes to the content Policy/ Guideline/ Protocol The complement system is part of the innate immune system. The complement system plays an important part in defence against pyogenic organisms. It promotes the inflammatory response, eliminates pathogens, and enhances the immune response. The complement system is also important for the clearance of immune complexes and apoptotic cells. Deficiencies of the complement components result in susceptibility to infections either specifically to neisserial species in some cases or to bacterial infections generally. There is also a marked susceptibility to autoimmune disease in early complement component defects. Deficiencies in complement predispose patients to infection via 2 mechanisms: (1) ineffective opsonization and (2) defects in lytic activity (defects in MAC). Deficiency of complement components is most commonly secondary to consumption in inflammatory disease processes. Genetically determined primary deficiencies of all the complement cascade factors can occur, but are rare. Page 2 of 6
3 Complement deficiency and disease association C1, C4 & C2 Deficiency: Autoimmune disease, especially Systemic Lupus Erythematosous (SLE), is the most common presentation in patients with early component deficiency. The incidence rates of SLE in individuals with C1q and C4 are reported to be around 90% and 75%, respectively. Patients with C2 deficiency develop SLE with lesser frequency (around 15%). The proposed mechanisms of high incidence of autoimmune diseases include impaired clearance of immune complex and apoptotic cells and loss of complement-dependent B-cell tolerance. Recurrent bacterial infection is common in patients with C2 deficiency. Mannose-Binding lectin (MBL) pathway including MASP-2 deficiency: Frequent pyogenic infection, including pneumococcal infection in infants and young children. Alternative pathway (properdin, factor B, factor D): Severe fulminant neisserial infections with a high mortality rate. C3, factor H, and factor I: Deficiency of these factors predisposes individuals to severe pyogenic bacterial infections. Factor H and factor I deficiencies cause secondary C3 deficiency with C3 consumption and impose the same infectious risk as primary C3 deficiency. Factor H deficiency is also associated with atypical (diarrhea-negative) hemolytic-uremic syndrome (HUS) and glomerulonephritis. C3 deficiency is associated with membranoproliferative glomerulonephritis. Terminal pathway (C5-C9) Deficiency: The lack of Membrane Attack Complex (MAC) formation results in severe recurrent infection with Neisseria gonorrhoeae or Neisseria meningitidis. C1INH: (hereditary or acquired) leads to uninhibited cleavage of C4 by C1s, results in recurrent episodes of angioedema Decay Accelerating Factor (DAF) and CD59 deficiency: Paroxysmal nocturnal hemoglobinuria (PNH) This protocol does not include C1INH deficiency or DAF and CD59 deficiency management. Differential Diagnosis Hypogammaglobulinemia, SLE with complement consumption, serum sickness. Investigations: Consider complement Deficiency in the following Recurrent meningococcal disease (40% incidence of complement deficiency) Single episode of meningococcal disease caused by an unusual sero-group organism eg W135, X, Y (20-50% incidence of complement deficiency) Family history of meningococcal disease (10% incidence of complement deficiency) Recurrent pneumococcal, streptococcal or haemophilus infections (Consideration should be given to excluding other primary Page 3 of 6
4 immunodeficiencies, in particular antibody deficiency, before extensive complement investigations are undertaken) Recurrent minor infections in preschool children with normal Igs (MBL pathway) Autoimmune disease: o Severe, familial, early onset or atypical SLE and glomerulonephritis o Antinuclear Antibodies (ANA) negative SLE How to investigate complement defects 1. Check classical and alternative haemolytic pathways (CH50 and AP50) Classical Pathway defect Alternative pathway defect Terminal pathway defect (+factor H and I deficiencies) CH50 Normal AP50 Normal 2. Individual complement components can then be measured 3. Low C3 and C4 levels suggest activation of the classical pathway 4. Low C3 and normal C4 levels suggest activation of the alternate pathway NB: It should be remembered that many of the complement factors are labile so that serum samples need to be separated and frozen at 70 0 C within 3 hours of collection. If transport to the laboratory may be delayed, specimens should be kept and transported on ice. Management Autoimmune disease Management is as for non-complement deficient disease (by Rheumatologists or Nephrologists), although the disease may be more severe and require more aggressive treatment. Infections -Supportive 1. Antibiotic prophylaxis. Penicillin V if susceptibility restricted to neisserial disease. Azithromycin, Augmentin, Cefixime or Septrin if broader susceptibility 2. Vaccinate against as many bacterial pathogens as possible including Hib, meningococcal A, C, W135, and Y conjugate vaccine (2 doses-2 months apart), and conjugate pneumococcal (prevenar) followed by polysaccharide pneumococcal vaccines. Page 4 of 6
5 3. Conjugate vaccines are preferred over pure polysaccharide vaccines 4. Patients with complement deficiency may receive all routine vaccines safely and are not at increased risk for adverse reactions to live viral vaccines. Responses should be checked and re-vaccination given as necessary. 5. Fresh frozen plasma infusion can be given to replace factors in the context of severe infection but no good evidence that these help. If found to be of benefit in an individual patient and long term or repeated plasma infusions is to be used, use of a detergent treated plasma product should be considered. 6. Policy Implementation Plan It is the responsibility of the Consultant Immunologist in consultation with Specialist registrar and Immunology Specialist nurse to implement this protocol The Clinical immunology team will be responsible for reviewing progress in implementation The protocol will be implemented within Immunology clinic/ team meetings by the all team members Standards United Kingdom Primary Immunodeficiency Network (UKPIN) guidelines European Society for Immunodeficiency (ESID) diagnostic Criteria for Primary Immune Deficiency (PID). Explanation of terms & Definitions Terms explained in document References and Supporting Documents Adapted from UKPIN guidelines Figueroa JE, Densen P (1991) Infectious diseases associated with complement deficiencies. Clin Microbiol Rev;4: Fijen CAP, Kuijper EJ te BulteMT et al (1999) Assessment of complement deficiency in patients with meningococcal disease in the Netherlands. Clin Inf Dis ;28: Sullivan KE, Winkelstein J (1999). Primary Immunodeficiency Diseases. Eds Ochs H, Smith CIE, Puck J. Oxford University Press Page 5 of 6
6 Roles and responsibilities Consultant Immunologist/ Specialist Registrar: Initial clinic assessment of patient with possible complement deficiency Investigations and diagnosis Discuss management and treatment options with patient/ family/ specialist Nurse Initiate treatment, obtain consent if applicable Follow up patient in clinic Specialist Immunology Nurse: Initiate blood sampling/ investigations Give immunisations if applicable/ follow up samples Discuss treatment options with patient and family Page 6 of 6
Complement: History. Discovered in 1894 by Bordet. It represents lytic activity of fresh serum
Complement: History Discovered in 1894 by Bordet It represents lytic activity of fresh serum Its lytic activity destroyed when heated at 56C for 30 min Complement functions Host benefit: opsonization to
More informationComplement. History. Chapter 7. Complement Components. Complement Pathways. Pathways of complement activation
History Chapter 7 Complement Jules Border in 1890 s discovered complement Paul Ehrlich coined the term complement The activity of blood serum that completes the action of antibody Now: Set of serum proteins
More informationHistory. Chapter 13. Complement Components. Complement Pathways
History Chapter 13 Complement Jules Border in 1890 s discovered complement Paul Ehrlich coined the term complement The activity of blood serum that completes the action of antibody Now: Set of serum proteins
More informationImmunology and the middle ear Andrew Riordan
Immunology and the middle ear Andrew Riordan The Immune system is NOT there; To baffle medical students To keep Immunologists in a job To encourage experiments on mice The Immune system IS there as a defence
More informationTHE COMPLEMENT SYSTEM OBJECTIVES:
Dr Mohammed Al- ani THE COMPLEMENT SYSTEM OBJECTIVES: When you finish this section, you should be able to: 1. Describe the effects of complement activation. 2. Outline the Classical, Mannan-Binding (MB)
More informationComplement pathways: Classical pathway Alternative pathway Lectin pathway
Complement Complement pathways: Classical pathway Alternative pathway Lectin pathway Complement proteins Classical pathway C1q C1r C1s C4 C2 Alternative pathway D C3 B Lectin pathway MBL MASP-1 MASP-2
More informationM1 - Immunology, Winter 2008
University of Michigan Deep Blue deepblue.lib.umich.edu 2008-09 M1 - Immunology, Winter 2008 Fantone, J.; Pietropaolo, M. T. Fantone, J., Pietropaolo, M. T. (2008, August 13). Immunology. Retrieved from
More informationAttribution: University of Michigan Medical School, Department of Microbiology and Immunology
Attribution: University of Michigan Medical School, Department of Microbiology and Immunology License: Unless otherwise noted, this material is made available under the terms of the Creative Commons Attribution
More informationAnastasios E. Germenis
Anastasios E. Germenis Professor and Chairman Department of Immunology & Histocompatibility School of Medicine University of Thessaly University Hospital of Larissa Greece agermen@med.uth.gr The Complement
More informationTarget cell lysis Opsonization Activation of the inflammatory response (e.g. degranulation, extravasation) Clearance of immune complexes
Immunology Dr. John J. Haddad Chapter 13 Complement Major roles of complement (Figure 13-1): Target cell lysis Opsonization Activation of the inflammatory response (e.g. degranulation, extravasation) Clearance
More informationInflammation. (4 of 5)
Inflammation (4 of 5) What will we discuss today? Plasma protein derived mediators Anti-inflammatory mediators Morphologic patterns of acute inflammation Plasma protein derived mediators 3 systems: -Complement
More informationIntroduction. A system of soluble enzymes and proteins. Complement components: C1 to C9, B, D and P
Complement Introduction A system of soluble enzymes and proteins Complement components: C1 to C9, B, D and P When activated, each component is split into small and large (major) fragments a b *A horizontal
More informationThe Biology of Fc γ Receptors and Complement
Discovery consists of seeing what everybody has seen, and thinking what nobody has thought --Albert Szent-György Nobel prize in Physiology or Medicine, 1937 The Biology of Fc γ Receptors and Complement
More informationBiology of Fc γ Receptors. Selected Functions of Ig Isotypes
Biology of Fc γ Receptors Selected Functions of Ig Isotypes Biology of Fc γ Receptors 1 Functional Sites on the IgG Molecule V H V L C1q binding site FcγR binding site Glycosylation site Selected Functions
More informationThe Biology of Fc γ Receptors and Complement. Biology of Fc γ Receptors. Discovery consists of seeing what everybody
Discovery consists of seeing what everybody has seen, and thinking what nobody has thought The Biology of Fc γ Receptors and Complement --Albert Szent-György Nobel prize in Physiology or Medicine, 1937
More informationCD B T NK NKT!! 1
CD B T NK NKT!! 1 2 !! 3 4 5 6 7 8 9 10 11 Biological effects of C5a 12 13 Opsonization and phagocytosis 14 15 http://www.med.sc.edu:85/book/wel come.htm 16 http://www.med.sc.edu:85/book/im munol-sta.htm
More informationHistory. Chapter 13. Complement Components. Complement Pathways
History Chapter 13 Complement Jules Border in 1890 s discovered complement Paul Ehrlich coined the term complement The activity of blood serum that completes the action of antibody Now: Set of serum proteins
More information3/31/2014 PNH. Jack Goldberg MD FACP. Clinical Professor of Medicine University of Pennsylvania
PNH Jack Goldberg MD FACP Clinical Professor of Medicine University of Pennsylvania 1 2 3 4 1 5 6 Historically Viewed as a Hemolytic Anemia Normal red blood cells are protected from complement attack by
More informationTHE COMPLEMENT SYSTEM OBJECTIVES:
THE COMPLEMENT SYSTEM OBJECTIVES: When you finish this section, you should be able to: 1. Describe the effects of complement activation. 2. Outline the Classical, Mannan-Binding (MB) Lectin and Alternative
More informationPrimary Immunodeficiency Disease: Underdiagnosed at any age. Anne L Sherwood, PhD Director of Scientific Affairs The Binding Site, Inc.
Primary Immunodeficiency Disease: Underdiagnosed at any age Anne L Sherwood, PhD Director of Scientific Affairs The Binding Site, Inc. Learning Objectives Identify the difference between primary and secondary
More informationBasic Immunology. Lecture 16th. Complement system
Basic Immunology Lecture 16th Complement system Components: Inactive factors in the serum and body fluids which can activate each other in an enzyme cascade Cell surface receptors (CR) for binding the
More informationComplement Elizabeth Repasky, PhD Fall, 2015
Complement Elizabeth Repasky, PhD Fall, 2015 Complement pathways: Classical pathway Alternative pathway Lectin pathway White Board Schematic C3 plays a central role in complement activation Complement
More information7/14/2014. SOLIRIS (eculizumab) SOLIRIS PNH Clinical Studies. SOLIRIS Blocks Terminal Complement. 86% Reduction in LDH: TRIUMPH and SHEPHERD
Proximal Terminal Lactate Dehydrogenase (U/L) 7/1/1 SOLIRIS (eculizumab) Humanized First in Class Anti - C5 Antibody SOLIRIS (eculizumab) Human Framework Regions No mutations Germline SOLIRIS is a Complement
More informationIgG subclass deficiencies
IgG subclass deficiencies hello@piduk.org 0800 987 8986 www.piduk.org About this booklet This booklet provides information on IgG subclass deficiencies. It has been produced by the PID UK Medical Advisory
More informationComplement: Deficiency Diseases
Complement: Deficiency Diseases Jerry A Winkelstein, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA Kathleen E Sullivan, University of Pennsylvania School of Medicine, Philadelphia,
More informationTopic (6): The Complement System
Topic (6): The Complement System Introduction The complement system is a complex system of many different glycoproteins. It comprises several plasma proteins that sequentially activate each other by proteolytic
More informationCellular & Molecular Immunology 2009
Cellular & Molecular Immunology 2009 Complement Nicholas M. Ponzio, Ph.D. Department of Pathology & Laboratory Medicine March 4, 2009 Innate and adaptive immunity FAMOUS BELGIANS Jules Jean Baptiste Vincent
More informationThe term complement refers to the ability of a system of some nonspecific proteins in normal human serum to complement, i.e., augment the effects of
COMPLEMENT SYSTEM The term complement refers to the ability of a system of some nonspecific proteins in normal human serum to complement, i.e., augment the effects of other components of immune system,
More informationInnate Immunity: (I) Molecules & (II) Cells
Innate Immunity: (I) Molecules & (II) Cells Stephanie Eisenbarth, M.D., Ph.D. FOCIS Advanced Course 2/19/18 Department of Laboratory Medicine Yale School of Medicine Department of Immunobiology Yale School
More informationComplement. Definition : series of heat-labile serum proteins. : serum and all tissue fluids except urine and CSF
Complement Complement Definition : series of heat-labile serum proteins Site : serum and all tissue fluids except urine and CSF Synthesis : in liver appear in fetal circulation during 1 st 13 W Function
More informationSOLIRIS is a Complement Inhibitor Indicated for the Treatment of Patients With PNH to Reduce Hemolysis
SOLIRIS (eculizumab) SOLIRIS is a Complement Inhibitor Indicated for the Treatment of Patients With PNH to Reduce Hemolysis SOLIRIS is the First and Only Approved Therapy for PNH SOLIRIS (eculizumab) [package
More informationاالستاذ المساعد الدكتور خالد ياسين الزاملي \مناعة \المرحلة الثانية \ التحليالت المرضية \ المعهد التقني كوت
Complement System The term complement refers to the ability of a system of some nonspecific proteins in normal human serum to complement, i.e., augment the effects of other components of immune system,
More informationVaccination and prophylaxis for asplenia: Guideline for clinicians
Vaccination and prophylaxis for asplenia: Guideline for clinicians Adults better health * better care * better value Acknowledgements The Western Australian Committee for Antimicrobials (WACA) would like
More informationWhen should a Primary Immunodeficiency be Suspected?
When should a Primary Immunodeficiency be Suspected? Ricardo U Sorensen. MD Head, Jeffrey Modell Diagnostic Center for Primary Immunodeficiencies. LSUHSC, New Orleans Learning Objectives Define Primary
More informationIf these vaccines haven t been given, please follow guidelines below for emergency procedures.
MANAGEMENT OF PATIIENTS POST SPLENECTOMY & HYPOSPLENIIC PATIIENTS Splenectomised and hyposplenic patients are at increased risk of life-threatening infections due to encapsulated micro-organisms such as
More informationSuvasini Modi Complement System Activation of Membrane attacking complex (MAC) and its effect and regulation
Figure- 1 https://en.wikipedia.org/wiki/complement_system Suvasini Modi Complement System Activation of Membrane attacking complex (MAC) and its effect and regulation Content Introduction Activation of
More informationProposal form for the evaluation of a genetic test for NHS Service Gene Dossier
Proposal form for the evaluation of a genetic test for NHS Service Gene Dossier Test Disease Population Triad Disease name OMIM number for disease 312060 Disease alternative names please provide any alternative
More information3/29/2011. Algorithms for Diagnosis of Suspected Immunodeficiency. Overview. Case #1. Case #2. Primary Immunodeficiency (PID) Case #3
Overview Algorithms for Diagnosis of Immuno Susan M. Orton, PhD, D(ABMLI), MT(ASCP) Associate Professor Four major categories of primary immuno (PID) Clinical conditions associated with PID and organisms
More informationPrimary Immunodeficiency
Primary Immunodeficiency DiGeorge Syndrome Severe Combined Immunodeficiency SCID X-Linked Agammaglobulinemia Common variable immunodeficiency (CVID) IgA deficiency Hyper- IgM Syndrome Wiskott-Aldrich syndrome
More informationLecture 17: Attack by Complement and Counterattack by Microbes
Lecture 17: Attack by Complement and Counterattack by Microbes 2 Review Concepts of Complement Complement was addressed in Lecture 3 Major first line of defense (innate immunity) Major functions: Opsonization
More informationIndications for the Immunological Evaluation of Patients with Meningitis
INVITED ARTICLE CLINICAL PRACTICE Ellie J. C. Goldstein, Section Editor Indications for the Immunological Evaluation of Patients with Meningitis Gary D. Overturf Department of Pediatrics and Pathology,
More informationCatalog Number: A114 Sizes Available: 250 µg/vial Concentration: 1.0 mg/ml (see Certificate of Analysis for actual concentration)
Name: C3b Catalog Number: A114 Sizes Available: 250 µg/vial Concentration: 1.0 mg/ml (see Certificate of Analysis for actual concentration) Form: Liquid Purity: >90% by SDS-PAGE Buffer: 10 mm sodium phosphate,
More informationApproach to a child with recurrent infections. Dave le Roux 9 March 2012
Approach to a child with recurrent infections Dave le Roux 9 March 2012 Jeffrey Modell Foundation http://www.info4pi.org Primary immune deficiencies: myths PID s are very very rare Selective IgA 1:333
More informationLiving with PNH 7/3/2013. Paroxysmal Nocturnal Hemoglobinuria (PNH): A Chronic, Systemic, and Life- Threatening Disease
Living with PNH Laurence A. Boxer, MD University of Michigan Case Study 15 year old awakened in the morning with chest pain and a sore throat. She experienced chest pain all day accompanied with coughing
More informationS404- Meningococcal Vaccines: Updated Policy Statement 2014
S404- Meningococcal Vaccines: Updated Policy Statement 2014 Michael T. Brady, MD Associate Medical Director Nationwide Children s Hospital Columbus, Ohio Disclosure of Relevant Relationship Dr. Brady (or
More informationImmunization of Specific Populations (Immunosuppressed and Chronic Health Conditions)
Immunization of Specific Populations (Immunosuppressed and Chronic Health Conditions) Revision Date: January 4, 2018 Health Conditions Requiring Special Considerations for Immunization General Principles...
More informationPNH ahus. Dosing and Administration. For Paroxysmal Nocturnal Hemoglobinuria (PNH) and atypical Hemolytic Uremic Syndrome (ahus) patients
For Paroxysmal Nocturnal Hemoglobinuria (PNH) and atypical Hemolytic Uremic Syndrome (ahus) patients PNH ahus Dosing and Administration Soliris is indicated for the treatment of patients with paroxysmal
More informationSAMPLE. PGD Reviewed by: Chris Faldon, John Maloney, Tim Patterson, Adrian MacKenzie, Claire Stein
Patient Group Direction for the supply or administration of haemophilus influenzae type B and meningococcal conjugate vaccine to children requiring immunisation as part of the national childhood vaccination
More informationComplement disorders and hereditary angioedema
Complement disorders and hereditary angioedema Michael M. Frank, MD Durham, NC The term complement was introduced more than 100 years ago to refer to a group of plasma factors important in host defense
More informationWhat is PNH? PNH: What it is Not 9/11/2015. What is Paroxysmal Nocturnal Hemoglobinuria?
9/11/15 PNH: Current Thinking on the Disease, Diagnosis, and Treatment Joseph H. Antin, MD Professor of Medicine Harvard Medical School Jock and Bunny Adams Chair in Hematology Dana-Farber/Brigham and
More informationPNH ahus gmg. Dosing and Administration Guide
Injection for Intravenous Use PNH ahus gmg For Paroxysmal Nocturnal Hemoglobinuria (PNH) and atypical Hemolytic Uremic Syndrome (ahus), and generalized Myasthenia Gravis (gmg) patients Dosing and Administration
More information3/6/2017. Prevention of Complement Activation and Antibody Development: Results from the Duet Trial
Prevention of Complement Activation and Antibody Development: Results from the Duet Trial Jignesh Patel MD PhD FACC FRCP Medical Director, Heart Transplant Cedars-Sinai Heart Institute Disclosures Name:
More informationSoliris Medical Policy Prior Authorization Program Summary
Soliris Medical Policy Prior Authorization Program Summary Precertification/Prior Authorization may be required under certain plans. Please verify each member s benefits. OBJECTIVE The intent of the Soliris
More informationPathogenetic mechanisms in atypical HUS
Pathogenetic mechanisms in atypical HUS Seppo Meri University & University Hospital Helsinki, Finland Antalya, Turkey Photo: Markku Kallio 21.10.2017 HUS after eating Kebab - 22 yo female - watery diarrhea
More informationSoliris. Soliris (eculizumab) Description
Federal Employee Program 1310 G Street, N.W. Washington, D.C. 20005 202.942.1000 Fax 202.942.1125 5.85.11 Subject: Soliris Page: 1 of 5 Last Review Date: September 20, 2018 Soliris Description Soliris
More informationESCMID Online Lecture Library. by author
Therapies or diseases predisposing to infection Congenital immunodeficiences Acquired conditions Common variable immunedeficiency particular diseases (e.g. HIV, cancer,leukemia, lymphoma, diabetes, cystic
More informationMacrophage Activation & Cytokine Release. Dendritic Cells & Antigen Presentation. Neutrophils & Innate Defense
Macrophage Activation & Cytokine Release Dendritic Cells & Antigen Presentation Neutrophils & Innate Defense Neutrophils Polymorphonuclear cells (PMNs) are recruited to the site of infection where they
More informationParoxysmal Nocturnal Hemoglobinuria
Paroxysmal Nocturnal Hemoglobinuria Barry Skikne MD, FACP, FCP(SA) Professor of Hematology Division of Hematologic Malignancies and Cellular Therapeutics Cardinal Clinical Manifestations PNH Clonal disease
More informationImmunodeficiency: Recognizing Subtle Signs, Diagnosis & Referral
Immunodeficiency: Recognizing Subtle Signs, Diagnosis & Referral Michael Daines, M.D. Associate Professor, Pediatric Allergy and Immunology Division director, Pediatric Allergy, Immunology, and Rheumatology
More informationSCID:failing the final exam on day 1
SCID:failing the final exam on day 1 Michael Daines, M.D. Associate Professor, Pediatric Allergy and Immunology Division director, Pediatric Allergy, Immunology, and Rheumatology Disclosures Nothing relevant
More informationImmunity and how vaccines work
Immunity and how vaccines work Dr Mary O Meara National Immunisation Office Objectives of session An understanding of the following principles Overview of immunity Different types of vaccines and vaccine
More informationNot So Benign Hematology Aplastic anemia, Paroxysmal Nocturnal Hemoglobinuria, atypical Hemolytic Uremic Syndrome, Thrombotic Thrombocytopenic Purpura
Not So Benign Hematology Aplastic anemia, Paroxysmal Nocturnal Hemoglobinuria, atypical Hemolytic Uremic Syndrome, Thrombotic Thrombocytopenic Purpura Robert A. Brodsky, MD Johns Hopkins Family Professor
More information1. The barriers of the innate immune system to infection
Section 3.qxd 16/06/05 2:11 PM Page 12 12 SECTION THREE: Fleshed out 1. The barriers of the innate immune system to infection Questions What are the three characteristics of the innate immune system? What
More informationI have no disclosures
Disclosures Streptococcal Pharyngitis: Update and Current Guidelines Richard A. Jacobs, MD, PhD Emeritus Professor of Medicine Division of Infectious Diseases I have no disclosures CID 2012:55;e 86-102
More informationSupplementary Appendix
Supplementary Appendix This appendix has been provided by the authors to give readers additional information about their work. Supplement to: Lapeyraque A-L, Malina M, Fremeaux-Bacchi V, et al. Eculizumab
More informationPost-Transplant Vaccination and Re-Immunisation Procedure
Post-Transplant Vaccination and Re-Immunisation Procedure Table of Contents Purpose... 1 Scope/Audience... 1 Associated documents and forms... 1 Definitions... 2 Background... 2 Vaccination Recommendations...
More informationPRIMARY IMMUNODEFICIENCIES CVID MANAGEMENT CVID MANAGEMENT
PRIMARY IMMUNODEFICIENCIES CVID MANAGEMENT CVID MANAGEMENT 1 PRIMARY IMMUNODEFICIENCIES KEY ABBREVIATIONS CVID CT IgA IgG IgM IPOPI IVIG SCIG PID Common Variable Immune Deficiency Computerised tomography
More informationHths 2231 Laboratory 7 Infection
Watch Movie: Meningitis Answer the movie questions on the worksheet. Complete activities 1-3. Activity #1: Go to the patho web page and click on activity 1. Click on Tutorials Click on Immunopathology
More informationPrinciples of Vaccination
Immunology and Vaccine-Preventable Diseases Immunology is a complicated subject, and a detailed discussion of it is beyond the scope of this text. However, an understanding of the basic function of the
More informationSoliris (eculizumab): Physician s guide to prescribing for patients with paroxysmal nocturnal haemoglobinuria (PNH)
Soliris (eculizumab): Physician s guide to prescribing for patients with paroxysmal nocturnal haemoglobinuria (PNH) CONTENTS PAGE WHAT IS SOLIRIS? SOLIRIS INDICATIONS IMPORTANT SAFETY INFORMATION SOLIRIS
More informationDescription of the evidence collection method. (1). Each recommendation was discussed by the committee and a consensus
Special Article Guidelines on the treatment of primary immune thrombocytopenia in children and adolescents: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular Sandra Regina Loggetto 1
More informationCONJUGATE MENINGOCOCCAL (ACWY) VACCINE
Cumbria & Lancashire Vaccine PGD Group ADVISORY INFORMATION FOR USE WITH PATIENT SPECIFIC DIRECTION OR INDIVIDUAL PRESCRIPTION SIGNED BY GP OR NON-MEDICAL PRESCRIBER. CONJUGATE MENINGOCOCCAL (ACWY) VACCINE
More informationUnderstanding the Complement Cascade and Its Role in Cold Agglutinin Disease. 1 M-CAgD-US-3006 February 2018
Understanding the Complement Cascade and Its Role in Cold Agglutinin Disease 1 February 2018 Instructions This information is provided as an educational resource for healthcare providers. It is not intended
More informationNEMO deficiency syndrome
NEMO NEMO deficiency syndrome hello@piduk.org 0800 987 8986 www.piduk.org About this leaflet This leaflet has been produced jointly between PID UK, Great Ormond Street Hospital (GOSH) and the Great North
More informationComplement aberrations and autoantibodies to complement proteins in relation to disease mechanisms.
Complement aberrations and autoantibodies to complement proteins in relation to disease mechanisms. Skattum, Lillemor Melander 2008 Link to publication Citation for published version (APA): Melander Skattum,
More informationCover Page. Author: Kotimaa, Juha Title: Analysis of systemic complement in experimental renal injury and disease Issue Date:
Cover Page The handle http://hdl.handle.net/1887/48207 holds various files of this Leiden University dissertation Author: Kotimaa, Juha Title: Analysis of systemic complement in experimental renal injury
More informationImmunisation Update for Occupational Health
Immunisation Update for Occupational Health Dr Gayatri Amirthalingam Immunisation, Hepatitis & Blood Safety department Public Health England 29 th April 2016 Session Outline Epidemiology of vaccine preventable
More informationPRESCRIBER SAFETY BROCHURE; IMPORTANT SAFETY INFORMATION FOR THE HEALTHCARE PROVIDER
PRESCRIBER SAFETY BROCHURE; IMPORTANT SAFETY INFORMATION FOR THE HEALTHCARE PROVIDER BEFORE STARTING YOUR PATIENTS ON SOLIRIS Important safety information for the healthcare provider Prior to initiating
More informationTest Name Results Units Bio. Ref. Interval
135091662 Age 45 Years Gender Male 29/8/2017 120000AM 29/8/2017 100215AM 29/8/2017 110825AM Ref By Final RHEUMATOID AUTOIMMUNE COMREHENSIVE ANEL ANTI NUCLEAR ANTIBODY / FACTOR (ANA/ANF), SERUM ----- 20-60
More informationPATIENT SAFETY BROCHURE; IMPORTANT SAFETY INFORMATION FOR PATIENTS. Before starting on Soliris Important safety information for patients
PATIENT SAFETY BROCHURE; IMPORTANT SAFETY INFORMATION FOR PATIENTS Before starting on Soliris Important safety information for patients Before you begin Soliris (eculizumab) treatment, your physician will
More informationComplement System. Jil Schrader 16 th of May 2018 Immunology Lecture
Source: https://bestprac.dk/2017/05/30/diagnosticering-af-almindelig-variabel-immundefekt-cvid-2/, letzter Zugriff: 14.05.2018 Complement System Jil Schrader 16 th of May 2018 Immunology Lecture Contents
More informationName: C3 Protein Concentrated Catalog Number: A113c Sizes Available: Concentration: Form: Activity: Purity: Buffer: Extinction Coeff.
Name: C3 Protein Concentrated Catalog Number: A113c Sizes Available: 1000 µg/vial Concentration: 5.0 mg/ml (see Certificate of Analysis for actual concentration) Form: Frozen liquid Activity: >70% versus
More informationLecture 07. Complement
Lecture 07 Complement Science is a social process. It happens on a time scale longer than a human life. If I die, someone takes my place. You die; someone takes your place. What's important is to get it
More informationDISCLOSURE. Relevant relationships with commercial entities none. Potential for conflicts of interest within this presentation none
AUTOIMMUNITY DISCLOSURE Relevant relationships with commercial entities none Potential for conflicts of interest within this presentation none Steps taken to review and mitigate potential bias N/A MODULE
More informationIF YOU HAVE PNH, YOU ARE NOT ALONE. Take a closer look and take control of PNH US/SOL-P/0005
IF YOU HAVE PNH, YOU ARE NOT ALONE Take a closer look and take control of PNH INDICATION & IMPORTANT SAFETY INFORMATION FOR SOLIRIS (eculizumab) INDICATION What is SOLIRIS? SOLIRIS is a prescription medicine
More informationAcknowledgements. Introduction. Structure of the video
Educators Guide Acknowledgements The Ministry of Health would like to thank Blue Bicycle Flicks. Thanks also to the staff and students from Evans Bay Intermediate School who contributed to the shooting
More informationImmunodeficiency and Skin (September 21, 2018) By (Arti Nanda, MD, DNBE [Kuwait])
Immunodeficiency and Skin (September 21, 2018) By (Arti Nanda, MD, DNBE [Kuwait]) Immune deficiency refers to a state in which part of immune system is missing or defective resulting into an inability
More informationSoliris (eculizumab): Physician s guide to prescribing for patients with refractory generalised myasthenia gravis (gmg)
Soliris (eculizumab): Physician s guide to prescribing for patients with refractory generalised myasthenia gravis (gmg) CONTENTS PAGE WHAT IS SOLIRIS? SOLIRIS INDICATIONS IMPORTANT SAFETY INFORMATION SOLIRIS
More informationWhat are the new active vaccine recommendations in the Canadian Immunization Guide?
154 CCDR 17 April 2014 Volume 40-8 https://doi.org/10.14745/ccdr.v40i08a03 1 What are the new active vaccine recommendations in the Canadian Immunization Guide? Warshawsky B 1 and Gemmill I 2 on behalf
More informationProblem 7 Unit 6 Clinical: Primary immunodeficiency
Problem 7 Unit 6 Clinical: Primary immunodeficiency THE IMMUNE SYSTEM - Function: recognizing pathogens (foreign non-self antigens) and organizing a defense response against them by facilitating destruction
More information9/10/2018. Principles of Vaccination. Immunity. Antigen. September 2018
Centers for Disease Control and Prevention National Center for Immunization and Respiratory Diseases Principles of Vaccination September 2018 Chapter 1 September 2018 Photographs and images included in
More informationImmunodeficiency. (1 of 2)
Immunodeficiency (1 of 2) Primary immunodeficiency diseases Innate or adaptive Most are detected in infancy (6 months 2 years) Rare but some mild genetic forms exist in many individuals Defects in Innate
More informationHemolytic uremic syndrome: Investigations and management
Hemolytic uremic syndrome: Investigations and management SAWAI Toshihiro M.D., Ph.D. Department of Pediatrics, Shiga University of Medical Science Otsu, JAPAN AGENDA TMA; Thrombotic micro angiopathy STEC-HUS;
More informationNot So Benign Hematology. Robert A. Brodsky, MD Johns Hopkins Family Professor Director of Adult Hematology
Not So Benign Hematology Robert A. Brodsky, MD Johns Hopkins Family Professor Director of Adult Hematology Disclosures Dr. Brodsky serves as a Scientific Advisory Board member to: Alexion Pharmaceuticals
More informationClinical Commissioning Policy Proposition: Rituximab for cytopaenia complicating primary immunodeficiency
Clinical Commissioning Policy Proposition: Rituximab for cytopaenia complicating primary immunodeficiency Reference: NHS England F06X02/01 Information Reader Box (IRB) to be inserted on inside front cover
More informationBefore starting on Soliris.
Before starting on Soliris. Important safety information for patients Before you begin Soliris (eculizumab) treatment, your physician will give you a: Medication Guide Soliris Patient Safety Information
More informationCorporate Medical Policy
Corporate Medical Policy File Name: Origination: Last CAP Review: Next CAP Review: Last Review: eculizumab_soliris 8/2014 4/2018 4/2019 4/2018 Description of Procedure or Service Paroxysmal nocturnal hemoglobinuria
More information2016 Vaccine Preventable Disease Summary
2016 Vaccine Preventable Disease Summary 12251 James Street Holland, MI 49424 www.miottawa.org/healthdata Prepared October 2017 2016 Summary of Vaccine Preventable Diseases (VPDs) Reported to Ottawa County
More information