DOCSLIB.ORG

UK National Haemophilia Database Bleeding Disorder

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
UK National Haemophilia Database Bleeding Disorder UKHCDO Annual Report 2016 & Bleeding Disorder Statistics for 2015/2016 Published by United Kingdom Haemophilia Centre Doctors’ Organisation 2016 © UKHCDO 2016 Copyright of the United Kingdom Haemophilia Centre Doctors’ Organisation. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system or transmitted in any other form by any means, electronic, mechanical, photocopying or otherwise without the prior permission in writing of the Chairman, c/o Secretariat, UKHCDO, City View House, Union Street, Ardwick, Manchester, M12 4JD. ISBN: 978-1-901787-20-7 UKHCDO Annual Report 2016 & Bleeding Disorder Statistics for 2015/2016 Contents 1. Chairman’s Report 1 2. Bleeding Disorder Statistics for 2015 / 2016 Contents 4 2.0. Comments on the Bleeding Disorder Statistics for 2015 / 2016 7 2.1 Haemophilia A 10 2.2 Haemophilia B 39 2.3 Von Willebrand Disease 56 2.4 Inhibitors Congenital and Acquired 61 2.5 Rarer Bleeding Disorders 67 2.6 Adverse Events 71 2.7 Morbidity and Mortality 74 3. Data Management Working Party 81 4. Haemtrack Group Report 84 5. Genetics Working Party 86 6. Genetic Laboratory Network 87 7. Inhibitor Working Party 89 8. Musculoskeletal Working Party 91 9. Paediatric Working Party 92 10. von Willebrand Disease Working Party 94 11. Obstetric Task Force 95 12. Therapeutic Task Force on Enhanced Half-Life Products 96 13. UK Haemophilia Data Managers’ Forum Group 97 14. Haemophilia Nurses’ Association 98 15. Haemophilia Chartered Physiotherapists’ Association 99 16. BCSH Haemostasis and Thrombosis Task Force 100 17. Haemophilia Society 102 18. The Macfarlane Trust 110 UKHCDO Annual Report 2016 & Bleeding Disorder Statistics for 2015/2016 1. Chairman’s Report Welcome to the QEII centre in Central London and the 2016 Annual General Meeting of the UKHCDO. I hope you will find it a useful, informative and enjoyable meeting. We have made some alterations to the format of the meeting this year; on the Thursday we are concentrating on the progress made by the UKHCDO Working Parties and these sessions will be open to everybody to listen and feedback on findings and ideas for future work. Two of the working parties are due to re-form later this year and there will be opportunity for newer members to apply to participate. On the Thursday we will also hear about some other completed and on-going research studies including a presentation from the Dutch group on the OPTI-CLOT project, a collaborative UK/Dutch study looking at pharmacokinetic dosing in haemophilia B. On the Friday morning we will have two ‘hot topic’ lectures on monitoring enhanced half-life (EHL) products and a gene therapy update followed by the standard AGM business and presentation of the annual statistics. Please do feedback after this meeting as this will help planning of future meetings. As we all know we are living in difficult times within the NHS with enormous shortfalls in the funding required to pay for existing and innovative services and treatments. The National procurement exercises that we have undertaken over the last few years have enabled us to make considerable savings and we now have very favourable factor concentrate prices when compared to other prices globally. During the last year the UKHCDO has continued to be very closely involved with the Commercial Medicines Unit (CMU) in the latest factor concentrate procurement exercise. The previous tender was a collaborative project between the UKHCDO and the NHS England appointed Clinical Reference Group (CRG) for Haemophilia. The latter was disbanded in early 2016 and reformed some months later but despite this disruption we were able to provide clinical input and oversight of the procurement process. The frameworks are now in place and we are able to purchase the majority of these factor concentrates for our patients; details have been communicated with all Centres. I would like to extend our gratitude for the continued support we receive from Alison Greenwood and Wendy Roach from the CMU. The recent tender will enable us to treat some of our patients with the new extended half-life products. The UKHCDO guideline on the use of ‘extended half-life’ products, written by a Task Force set-up in 2015, was published earlier on this year and is an informative document to refer to when discussing the use of these products. In England there has been recent commissioning guidance on how we can use these concentrates in a cost-neutral or cost- saving way; again this was circulated to members recently. The NHSE CRG has not met since the last AGM due to its dissolution but it has now been reformed and we will have a CRG update session during the AGM. Despite the hiatus in CRG status we were pleased to hear in mid-2016 that the UKHCDO protocol for immune tolerance was adopted as an NHSE official policy. This means that ITI for children in England should only be done according to this protocol as this is the only ITI that is commissioned. We are planning detailed collection of outcome data using this protocol which will be co-ordinated by the Inhibitor Working Party. The UKHCDO has a good track record at peer review and audit but moving forward on this has been somewhat on the back burner over the last year or so. I am delighted to welcome John Hanley as the new Audit Lead for the UKHCDO and look forward to working with him and a multi-disciplinary ‘audit team’ (yet to be formed) to develop a new audit tool to use for 2017. It is likely that future audits will have more credibility with commissioners if they include Page | 1 UKHCDO Annual Report 2016 & Bleeding Disorder Statistics for 2015/2016 assessments of outcome measures and evidence of adherence to national protocols and guidelines. During the AGM we will as usual hear about the work and projects being done by the NHD and updates to HCIS and Haemtrack. Haemtrack usage uptake has continued to increase across the country and it has benefits that improve patient care. NHS England has adopted Haemtrack usage as a CQUIN for 2016/17; the development of this was time-consuming and it is a complicated CQUIN which has been adopted by the majority – but not all – of the English centres. It is being refined for 2017/18 and may well become mandatory for all Centres. My first year has Chairman (‘Chairperson’) has gone very quickly and required more hours of input than I envisaged; there are still a number of tasks I have not yet addressed but hope to be able to in the coming months. I am very grateful for the support of the rest of the Executive and particularly want to thank David Keeling who will be stepping down as Vice-Chair at the AGM and I am delighted that he will be replaced by Professor Peter Collins who we welcome onto the Executive Committee. I would like to encourage all members to come to me and the other Executive Committee members with queries, suggestions and feedback on any bleeding disorder issues. In the coming year the UKHCDO will continue to work closely with the newly formed NHSE Clinical Reference Group (and NHS Scotland, Wales and N.I) on moving forward with improving haemophilia care. Lastly I would like to thank all members of the NHD staff and their hard work in supporting the UKHCDO and the challenging times that we are working in. Dr Ri Liesner UKHCDO Chair October 2016 Page | 2 UKHCDO Annual Report 2016 & Bleeding Disorder Statistics for 2015/2016 Bleeding Disorder Statistics for April 2015 to March 2016 A report from the National Haemophilia Database November 2016 Page | 3 UKHCDO Annual Report 2016 & Bleeding Disorder Statistics for 2015/2016 2. Bleeding Disorder Statistics for 2015 / 2016 Contents Appendix 1: Glossary .......................................................................................................................... 7 2.0. Comments on the Bleeding Disorder Statistics for 2015 / 2016 ................ 9 2.1 Haemophilia A ........................................................................................ 10 Table 1 Patients with congenital Haemophilia A registered and treated April 2015 ‐ March 2016 ........................................................................................................ 11 Figure 1 Carriers of Haemophilia A currently registered and newly registered, by baseline factor VIII level ....................................................................................... 12 Table 2 New registrations of Haemophilia A between April 2015 & March 2016, by age at mid‐year and severity ........................................................................... 13 Table 3 New registrations of patients with Severe Haemophilia A aged over 5 years old, and subsequent treatment by year ..................................................... 14 Figure 2 Trend in numbers of Severe Haemophilia A patients aged 60 years and above, 1975 – 2015/16 (including HIV +ve pts).................................................... 15 Figure 3 Trend in numbers of non‐Severe Haemophilia A patients aged 60 years and above, 1975 – 2015/16 (including HIV +ve pts)............................................. 15 Figure 4 Factor VIII units used by UK Haemophilia Centres 1992 ‐ 2015/16 all diagnoses .............................................................................................................. 17 Figure 5 Factor VIII units used by UK Haemophilia Centres to treat Severe Haemophilia A – 2010/11 ‐ 2015/16 .................................................................... 18 Table 4 Treatment intensity of patients with Severe Haemophilia A – 2010/11 ‐ 2015/16 ...............................................................................................................
Load more

Recommended publications
  • Genetic and Epigenetic Determinants of Thrombin Generation Potential : an Epidemiological Approach Maria-Ares Rocanin-Arjo
    Genetic and Epigenetic Determinants of Thrombin Generation Potential : an epidemiological approach Maria-Ares Rocanin-Arjo To cite this version: Maria-Ares Rocanin-Arjo. Genetic and Epigenetic Determinants of Thrombin Generation Potential : an epidemiological approach. Génétique humaine. Université Paris Sud - Paris XI, 2014. Français. NNT : 2014PA11T067. tel-01231859 HAL Id: tel-01231859 https://tel.archives-ouvertes.fr/tel-01231859 Submitted on 21 Nov 2015 HAL is a multi-disciplinary open access L’archive ouverte pluridisciplinaire HAL, est archive for the deposit and dissemination of sci- destinée au dépôt et à la diffusion de documents entific research documents, whether they are pub- scientifiques de niveau recherche, publiés ou non, lished or not. The documents may come from émanant des établissements d’enseignement et de teaching and research institutions in France or recherche français ou étrangers, des laboratoires abroad, or from public or private research centers. publics ou privés. UNIVERSITÉ PARIS-SUD ÉCOLE DOCTORALE 420 : SANTÉ PUBLIQUE PARIS SUD 11, PARIS DESCARTES Laboratoire : Equip 1 de Unité INSERM UMR_S1166 Genomics & Pathophysiology of Cardiovascular Diseases THÈSE DE DOCTORAT SANTÉ PUBLIQUE - GÉNÉTIQUE STATISTIQUE par Ares ROCAÑIN ARJO Genetic and Epigenetic Determinants of Thrombin Generation Potential: an epidemiological approach. Date de soutenance : 20/11/2014 Composition du jury : Directeur de thèse : David Alexandre TREGOUET DR, INSERM U1166, Université Paris 6, Jussieu Rapporteurs : Guy MEYER PU_PH, Service de pneumologie. Hôpital européen Georges Pompidou Richard REDON DR, Institut thorax, UMR 1087 / CNRS UMR 6291 , Université de Nantes Examinateurs : Laurent ABEL DR, INSERM U980, Institut Imagine Marie Aline CHARLES DR, INSERM U1018, CESP Al meu pare (to my father /à mon père) Your genetics load the gun.
    [Show full text]
  • Haemostatic Effects of Recombinant Coagulation Factor Viia
    Haemostatic effects of recombinant coagulation factor VIIa Ton Lisman Lay-out: Pre Press, Baarn Cover illustration by Janine Marie¨n Inside illustrations by Geert Donker ISBN: 90-393-3192-8 Haemostatic effects of recombinant coagulation factor VIIa Bloedstelping door recombinant stollingsfactor VIIa (Met een samenvatting in het Nederlands) PROEFSCHIFT Ter verkrijging van de graad van doctor aan de Universiteit Utrecht op gezag van de Rector Magnificus, Prof. Dr. W.H. Gispen, in gevolge het besluit van het College voor Promoties in het openbaar te verdedigen op dinsdag 17 december 2002 des ochtends te 10.30 uur. door Johannes Antonius Lisman Geboren op 10 Maart 1976, te Arnhem Promotor: Prof. Dr. Ph.G. de Groot Faculteit geneeskunde, Universiteit Utrecht Co-Promotor: Dr. H.K. Nieuwenhuis Faculteit geneeskunde, Universiteit Utrecht The studies described in this thesis were supported in part by an unrestricted educational grant from Novo Nordisk. Financial support by Novo Nordisk for the publication of this thesis is gratefully acknowledged. I’ve got a pen in my pocket does that make me a writer Standing on the mountain doesn’t make me no higher Putting on gloves don’t make you a fighter And all the study in the world Doesn’t make it science Paul Weller Contents Chapter 1. General Introduction 9 Haemophilia Chapter 2. Inhibition of fibrinolysis by recombinant factor VIIa in plasma from patients with severe haemophilia A 41 Appendix to chapter 2. Enhanced procoagulant and antifibrinolytic potential of superactive variants of recombinant factor VIIa in plasma from patients with severe haemophilia A 55 Cirrhosis and liver transplantation Chapter 3.
    [Show full text]
  • Acquired Bleeding Disorders Tiede, Andreas; Zieger, Barbara; Lisman, Ton
    University of Groningen Acquired bleeding disorders Tiede, Andreas; Zieger, Barbara; Lisman, Ton Published in: Haemophilia DOI: 10.1111/hae.14033 IMPORTANT NOTE: You are advised to consult the publisher's version (publisher's PDF) if you wish to cite from it. Please check the document version below. Document Version Publisher's PDF, also known as Version of record Publication date: 2021 Link to publication in University of Groningen/UMCG research database Citation for published version (APA): Tiede, A., Zieger, B., & Lisman, T. (2021). Acquired bleeding disorders. Haemophilia, 27(S3), 5-13. https://doi.org/10.1111/hae.14033 Copyright Other than for strictly personal use, it is not permitted to download or to forward/distribute the text or part of it without the consent of the author(s) and/or copyright holder(s), unless the work is under an open content license (like Creative Commons). The publication may also be distributed here under the terms of Article 25fa of the Dutch Copyright Act, indicated by the “Taverne” license. More information can be found on the University of Groningen website: https://www.rug.nl/library/open-access/self-archiving-pure/taverne- amendment. Take-down policy If you believe that this document breaches copyright please contact us providing details, and we will remove access to the work immediately and investigate your claim. Downloaded from the University of Groningen/UMCG research database (Pure): http://www.rug.nl/research/portal. For technical reasons the number of authors shown on this cover page is limited to 10 maximum. Download date: 30-09-2021 Received: 24 February 2020 | Accepted: 23 April 2020 DOI: 10.1111/hae.14033 SUPPLEMENT ARTICLE Acquired bleeding disorders Andreas Tiede1 | Barbara Zieger2 | Ton Lisman3 1Hematology, Hemostasis, Oncology and Stem Cell Transplantation, Hannover Abstract Medical School, Hannover, Germany Acquired bleeding disorders can accompany hematological, neoplastic, autoimmune, 2 Division of Pediatric Hematology and cardiovascular or liver diseases, but can sometimes also arise spontaneously.
    [Show full text]
  • Haemostasis & Thrombosis Laboratory User Guide
    Haemostasis and Thrombosis Laboratory User Guide HAEMOSTASIS & THROMBOSIS LABORATORY USER GUIDE Document number: HPA-FO-002 WARNING: This is a controlled Page: 1 of 38 Author: Sarah Clarke document Date issued: 08/06/2020 Approved by: Head BMS Revision: 13.0 Haemostasis and Thrombosis Laboratory User Guide CONTENTS 1 INTRODUCTION ....................................................................................................... 4 2 LOCATION ................................................................................................................ 4 3 OPENING HOURS .................................................................................................... 5 3.1 Routine Coagulation Screening Service ................................................................ 5 3.2 Specialist Coagulation Service: ............................................................................. 5 4 CONTACT NUMBERS AND KEY PERSONNEL ...................................................... 6 5 CONFIDENTIALITY AND THE PROTECTION OF PERSONAL INFORMATION ..... 6 6 COMPLAINTS AND COMPLIMENTS……………………………………………………..7 7 CLINICAL INFORMATION ........................................................................................ 8 8 CLINICAL ADVICE AND INTERPRETATION ........................................................... 8 9 LABELLING OF REQUEST FORMS ........................................................................ 8 10 LABELLING OF SPECIMENS .................................................................................. 9 11 SAMPLE REQUIREMENTS
    [Show full text]
  • Hemostasis: Definition
    Bleeding disorders in children prof. Mariusz Wysocki, Katedra i Klinika Pediatrii, Hematologii i Onkologii Collegium Medicum Bydgoszcz UMK Toruń Hemostasis: definition • the sum total of those specialized functions within the circulating blood and its vessels that are intended to stop hemorrhage Hemostasis: players and phases • plasma factors • Vascular phase • inhibitors • Platelet phase • fibrinolisis • Plasma phase • platelets • vessels The coagulation system Bleeding disorders: clinical approach • history • physical examination • laboratory tests Bleeding diosrders: signs and symptoms Site Within normal May be abnormal Usually due to a limits and due to a bleeding disorder number of causes Nose Finger – induced Unilateral Recurrent, requiring medical intervention or causing anemia Oral Blood on brush Gum ooze < 30 min Gum ooze > 30 min Gut Rectal fissure, blood Haematemesis, in nappy Melaena Bleeding diorders: signs and symptoms Site Within normal May be abnormal Usually due to a limits and due to a bleeding disorder number of causes Menstrual loss 4 – 7 days „same as Mum” Loss leading to anemia or transfusion Skin Shins don’t count Bony prominences Spontaneous bruising over soft areas, laceration bleeding > 30 min Joints and muscles Trauma induced Spontaneous Intracranial Neonatal , trauma Spontaneous induced History: neonates (Sharathkumar A, 2008, Bowman M, 2009) • prolonged bleeding at the circumcision site, • cephalohematomas, • prolonged umbilical stump bleeding Warning signgs and symptoms: Epistaxis: (Sharathkumar A, 2008,
    [Show full text]
  • Spontaneous Intraocular Bleeding As an Initial Presentation of Factor XI Deficiency
    Hematocase Spontaneous intraocular bleeding as an initial presentation of Factor XI deficiency L. Duquenne, MD1, V. Schlesser, PhD2, Z. Jedidi, MD3, L. Plawny, MD4 Haemophilia C, also known as Rosenthal syndrome, is a rare disorder affecting essentially persons of Ashkenazi Jewish ascendancy. We report the case of a 79 year old patient without previous bleeding history, except for slight bruising, who presented with a severe retinal bleeding in the absence of trauma. Biology showed elevated activated partial thromboplastin time (77,7 seconds vs. normal range 30-36 seconds). Factor analysis showed isolated decreased Factor XI of 1%. No Factor XI inhibitor could be found. The patient was subsequently treated with Factor XI infusions which allowed a reduction of the bleeding and normal eyesight within four days. Spontaneous bleeding in old age is a rare inaugural sign of Factor XI deficiency, such episodes mostly occur after haemostatic challenge such as surgery or trauma leading to blood analysis and coagulation tests. Intraocular haemorrhage is an uncommon presentation of mild bleeding disorders.1-5 (Belg J Hematol 2014;5(1):22-24) Introduction most prevalent genetic defects among the Ashkenazy Haemophilia C is defined by a dysfunction of the Factor Jewish population, eight to nine percent of them presents XI activity. It can result from an impairment of secre- a heterozygous mutation while homozygosity varies tion, dimerisation or by a mechanism of heterodimer from 0.22% - 0.53%.1-3 trapping in heterozygous subjects. A bleeding score has been computed by the Interna- Factor XI (FXI) is a plasma glycoprotein that partici- tional Multicentre Study initially described for von pates in the intrinsic phase of blood coagulation.6,7 Un- Willebrand disease, defining grades of bleeding severity like other contact factors, FXI plays an important role in coagulation disorders.
    [Show full text]
  • National Bloood Authority Annual Report 2010–11
    NATIONAL BLOOD AUTHORITY BLOOD NATIONAL NATIONAL BLOOD AUTHORITY AUSTRALIA ANNUAL REPORT 2010–2011 AUSTRALIA ANNUAL REPORT ANNUAL 2010–2011 NATIONAL BLOOD AUTHORITY AUSTRALIA ANNUAL REPORT 2010–2011 Our mission is to secure a quality blood supply through world leading contractual arrangements; promote safe, high quality management and use of blood and blood products in Australia; and drive continual performance improvement across the sector. NBA 38977 Annual Report 2010-11_Text_AW4.indd i 14/10/11 5:23 PM ii 2010–11 With the exception of any logos and registered trademarks, and where otherwise noted, all material presented in this document is provided under a Creative Commons Attribution 3.0 Australia (http://creativecommons.org/licenses/by/3.0/au/) licence. ANNUAL REPORT The details of the relevant licence conditions are available on the Creative Commons NBA website (accessible using the links provided) as is the full legal code for the CC BY 3.0 AU licence (http://creativecommons.org/licenses/by/3.0/au/legalcode). The content obtained from this document or derivative of this work must be attributed as the National Blood Authority annual report 2010–11. ISSN 1832–1909 This report is available online at: www.nba.gov.au/pubs/annual-report.html Printed by: Paragon Printers Australasia Designed by: Swell Design Group Contact offi cer: Stephanie Gunn Acting CEO and General Manager Locked Bag 8430 Canberra ACT 2601 Telephone: 02 6211 8300 Facsimile: 02 6211 8330 Email: [email protected] Website: www.nba.gov.au Images of blood and blood products contained in graphics are courtesy of NBA’s suppliers.
    [Show full text]
  • Bypassing Agents Review
    National Essential Medicines List Tertiary/Quaternary Medication Review Process Bypassing Agents Date: September 2017 Medication: Haemophilia bypassing agents Recombinant Factor VIIa (rVIIa) Activated Prothrombin Complex Concentrate (aPCC) Indication: Haemophilia with inhibitors (on demand, when presenting with a significant bleed) Introduction Haemophilia refers to inherited bleeding disorders caused by deficiency of specific coagulation factors. Haemophilia A is caused by coagulation factor VIII (FVIII) deficiency, haemophilia B by deficiency of coagulation factor IX (FIX), and haemophilia C by deficiency of coagulation factor XI. These clotting factor deficiencies are caused by recessive mutations of the respective clotting factor genes. Both diseases have the same clinical presentation, so their specific diagnosis must be established by factor assay. Haemophilia A has a prevalence of about 1 in 10 000 males, while haemophilia B is less common, with a prevalence of about 1 in 35 000 males. Haemophilia A and B is treated using FVIII-replacement or Factor FIX therapy, but administration of the factor can lead to the development of anti-FVIII or FIX antibodies, commonly known as inhibitors. These inhibitors interfere with the factor function and prevent coagulation. Inhibitors are usually IgG antibodies that neutralise the procoagulant activity of FVIII or FIX. About 10 - 15% of haemophilia A patients and 1 - 3% of haemophilia B patients may develop persistent inhibitors, which make treatment with factor concentrates difficult.1 Haemophilia patients with inhibitors are at a higher risk of experiencing a bleeding episode and are more difficult to treat. Treatment of acute bleeding episodes in patients with high-responding inhibitors most often involves the use of bypassing haemostatic agents, such as plasma-derived activated prothrombin complex concentrates (aPCC) or recombinant activated Factor VII (rFVIIa).
    [Show full text]
  • Factor XI Deficiency
    Factor XI deficiency Description Factor XI deficiency is a disorder that can cause abnormal bleeding due to a shortage ( deficiency) of the factor XI protein, which is involved in blood clotting. This condition is classified as either partial or severe based on the degree of deficiency of the factor XI protein. However, regardless of the severity of the protein deficiency, most affected individuals have relatively mild bleeding problems, and some people with this disorder have few if any symptoms. The most common feature of factor XI deficiency is prolonged bleeding after trauma or surgery, especially involving the inside of the mouth and nose (oral and nasal cavities) or the urinary tract. If the bleeding is left untreated after surgery, solid swellings consisting of congealed blood (hematomas) can develop in the surgical area. Other signs and symptoms of this disorder can include frequent nosebleeds, easy bruising, bleeding under the skin, and bleeding of the gums. Women with this disorder can have heavy or prolonged menstrual bleeding (menorrhagia) or prolonged bleeding after childbirth. In contrast to some other bleeding disorders, spontaneous bleeding into the urine (hematuria), gastrointestinal tract, or skull cavity are not common in factor XI deficiency, although they can occur in severely affected individuals. Bleeding into the muscles or joints, which can cause long-term disability in other bleeding disorders, generally does not occur in this condition. Frequency Factor XI deficiency is estimated to affect approximately 1 in 1 million people worldwide. The severe deficiency disorder is much more common in people with central and eastern European (Ashkenazi) Jewish ancestry, occurring in about 1 in 450 individuals in that population.
    [Show full text]
  • Overview of the Coagulation System
    Review Article Overview of the coagulation system Address for correspondence: Sanjeev Palta, Richa Saroa, Anshu Palta1 Dr. Sanjeev Palta, Departments of Anaesthesiology and Intensive Care and 1Pathology, Government Medical College and Department of Anaesthesiology Hospital, Chandigarh, India and Intensive Care, Government Medical College and Hospital, Chandigarh, India. ABSTRACT E-mail: sanjeev_palta@yahoo. com Coagulation is a dynamic process and the understanding of the blood coagulation system has evolved over the recent years in anaesthetic practice. Although the traditional classification of the coagulation system into extrinsic and intrinsic pathway is still valid, the newer insights into coagulation provide more authentic description of the same. Normal coagulation pathway represents a balance between the pro coagulant pathway that is responsible for clot formation and the Access this article online mechanisms that inhibit the same beyond the injury site. Imbalance of the coagulation system may Website: www.ijaweb.org occur in the perioperative period or during critical illness, which may be secondary to numerous factors leading to a tendency of either thrombosis or bleeding. A systematic search of literature on DOI: 10.4103/0019-5049.144643 PubMed with MeSH terms ‘coagulation system, haemostasis and anaesthesia revealed twenty Quick response code eight related clinical trials and review articles in last 10 years. Since the balance of the coagulation system may tilt towards bleeding and thrombosis in many situations, it is mandatory
    [Show full text]
  • Open Research Online Oro.Open.Ac.Uk
    Open Research Online The Open University’s repository of research publications and other research outputs Development of a Liver Gene Therapy Strategy for Haemophilia B With Lentiviral Vectors Thesis How to cite: Cantore, Alessio (2013). Development of a Liver Gene Therapy Strategy for Haemophilia B With Lentiviral Vectors. PhD thesis The Open University. For guidance on citations see FAQs. c 2013 The Author https://creativecommons.org/licenses/by-nc-nd/4.0/ Version: Version of Record Link(s) to article on publisher’s website: http://dx.doi.org/doi:10.21954/ou.ro.0000f0bd Copyright and Moral Rights for the articles on this site are retained by the individual authors and/or other copyright owners. For more information on Open Research Online’s data policy on reuse of materials please consult the policies page. oro.open.ac.uk (v£s-nS\ creD Alessio Cantore, M.Sc. Development of a Liver Gene Therapy Strategy for Haemophilia B with Lentiviral Vectors PhD thesis in fulfilment o f the requirements o f the Open University for the degree ofDoctor o f Philosophy in Molecular and Cellular Biology 17th December 2012 Director of Studies External Supervisor Prof. Luiai Naldini Prof. Thierry VandenDriessche Additional Supervisor Prof. Maria Grazia Roncarolo Vita-Salute San Raffaele University Telethon Institute for Gene Therapy (TIGET) DIBIT, San Raffaele Scientific Institute, Milan, Italy The Open University, UK Dtfvce _s,o m\ ss vdA Z IR CL £ o 12 ProQuest Number: 13835931 All rights reserved INFORMATION TO ALL USERS The quality of this reproduction is dependent upon the quality of the copy submitted.
    [Show full text]
  • The Bleeding Child; Is It NAI? a E Thomas
    1163 PERSONAL PRACTICE Arch Dis Child: first published as 10.1136/adc.2003.034538 on 19 November 2004. Downloaded from The bleeding child; is it NAI? A E Thomas ............................................................................................................................... Arch Dis Child 2004;89:1163–1167. doi: 10.1136/adc.2003.034538 As a paediatric haematologist, the question of whether a Presenting complaint The history of the presenting complaint will child has been abused or whether they might have a include questions as to how the injury occurred bleeding diathesis is a question that I am regularly asked. and an assessment made as to whether this When I first became a consultant, I would often find that not explains the injuries or bleeding seen. Guidance is given that abuse should be suspected if there is enough information was available; for example, significant bruising or bleeding with no history incomplete histories had been taken or investigations were of trauma or a history inconsistent with the incomplete and difficult to interpret. This inevitably led to severity of the injury,3 but in a child with a bleeding diathesis, this may be precisely how the delays in confirming the cause of the bleeding and meant child presents. Where a child has bruising in a that if parents or carers contested a diagnosis of abuse, recognisable pattern such as a belt or hand, then excluding a bleeding disorder was extremely difficult. I was suspected abuse must be reported regardless of the results of laboratory tests.4 However, finger- also aware that carers of several of my patients with tip bruising can be seen in children with a haemophilia or other bleeding disorders had initially been bleeding diathesis from normal physical interac- under suspicion of abuse, most usually at the time of the tion.
    [Show full text]