<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>1130-0108</journal-id>
<journal-title><![CDATA[Revista Española de Enfermedades Digestivas]]></journal-title>
<abbrev-journal-title><![CDATA[Rev. esp. enferm. dig.]]></abbrev-journal-title>
<issn>1130-0108</issn>
<publisher>
<publisher-name><![CDATA[Sociedad Española de Patología Digestiva]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1130-01082018000800010</article-id>
<article-id pub-id-type="doi">10.17235/reed.2018.5204/2017</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Trasplante hepático en hemofilia A y enfermedad de von Willebrand tipo 3. Manejo perioperatorio y evolución postrasplante]]></article-title>
<article-title xml:lang="en"><![CDATA[Liver transplantation in hemophilia A and von Willebrand disease type 3: perioperative management and post-transplant outcome]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Alonso-Madrigal]]></surname>
<given-names><![CDATA[Cristina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Dobón-Rebollo]]></surname>
<given-names><![CDATA[Manuela]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Laredo-de-la-Torre]]></surname>
<given-names><![CDATA[Viviana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Palomera-Bernal]]></surname>
<given-names><![CDATA[Luis]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[García-Gil]]></surname>
<given-names><![CDATA[Francisco-Agustín]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital Clínico Universitario Lozano Blesa Servicios de Hematología y Hemoterapia ]]></institution>
<addr-line><![CDATA[Zaragoza ]]></addr-line>
<country>Spain</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Hospital Clínico Universitario Lozano Blesa Servicios de Aparato Digestivo ]]></institution>
<addr-line><![CDATA[Zaragoza ]]></addr-line>
<country>Spain</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Hospital Clínico Universitario Lozano Blesa Servicios de Cirugía General y del Aparato Digestivo ]]></institution>
<addr-line><![CDATA[Zaragoza ]]></addr-line>
<country>Spain</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>08</month>
<year>2018</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>08</month>
<year>2018</year>
</pub-date>
<volume>110</volume>
<numero>8</numero>
<fpage>522</fpage>
<lpage>526</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_arttext&amp;pid=S1130-01082018000800010&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_abstract&amp;pid=S1130-01082018000800010&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_pdf&amp;pid=S1130-01082018000800010&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  la infección por virus hepatitis C (VHC) ha sido causa de importante morbi-mortalidad en hemofilia, planteándose el trasplante hepático (TH) por cirrosis y/o carcinoma hepatocelular (CHC).  Caso clínico:  presentamos los casos con coagulopatías congénitas e infección por VHC sometidos a TH en nuestro centro: tres pacientes con hemofilia A y uno con enfermedad de von Willebrand (EvW) tipo 3. Evaluamos el curso de la coagulopatía, el manejo perioperatorio, el consumo de factor y componentes sanguíneos y la supervivencia postrasplante. El factor deficitario se comenzó a administrar en bolo iv directo una hora antes del inicio de la cirugía para alcanzar un nivel deseado de dicho factor de 100 UI/dl, mantenido hasta conseguir el control estable de la hemostasia. Los tres pacientes con hemofilia A curaron su coagulopatía postrasplante. El factor VIII (FVIII) fue 93 UI/dl a los once años, 59 UI/dl a los 13 meses y 109 UI/dl a los nueve meses postrasplante en cada uno de los casos. El consumo medio perioperatorio de concentrados de FVIII fue 175 UI/kg, infundido hasta 36 h postrasplante de media. El paciente con EvW tipo 3 consiguió atenuar el curso natural de su sintomatología hemorrágica sin que se detectaran niveles hemostáticos del antígeno del factor von Willebrand (FVW:Ag) postrasplante.  Discusión:  tras el trasplante hepático, se produce la curación de la hemofilia A y la mejoría del fenotipo hemorrágico en la EvW tipo 3.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction:  infection with the hepatitis C virus (HCV) causes significant morbidity and mortality in patients with hemophilia. Finally, patients are considered for a liver transplantation (LT) due to cirrhosis and/or hepatocellular carcinoma (HCC).  Case report:  we report the cases of congenital coagulopathy and HCV infection that underwent LT in our institution. There were three patients with hemophilia A and one patient with von Willebrand disease (vWD) type 3. The coagulopathy outcome, perioperative management, factor and blood product usage and post-transplant survival were assessed. The deficient factor was initially administered in a direct bolus one hour before surgery with a target level of 100 IU/dl, which was sustained until stable hemostasis was reached. All three patients with hemophilia A were cured of their coagulopathy following transplantation. Factor VIII (FVIII) was 93 IU/dl at eleven years, 59 IU/dl at 13 months and 109 IU/dl at nine months post-transplant, in each case. The mean perioperative usage of FVIII concentrates was 175 IU/kg; concentrates were infused for an average of 36 hours post-transplant. The natural course of the bleeding symptoms of the patient with type-3 vWD was attenuated, with no detectable hemostatic levels of von Willebrand factor antigen (vWF:Ag) after transplantation.  Discussion:  after transplantation, hemophilia A cure and improved bleeding phenotype of type-3 vWD reduced morbidity and mortality. However, potential graft reinfection with HCV and relapsing HCC cast a shadow over these optimum results.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Hemofilia]]></kwd>
<kwd lng="es"><![CDATA[Enfermedad de von Willebrand]]></kwd>
<kwd lng="es"><![CDATA[Hepatitis C]]></kwd>
<kwd lng="es"><![CDATA[Trasplante hepático]]></kwd>
<kwd lng="en"><![CDATA[Hemophilia]]></kwd>
<kwd lng="en"><![CDATA[Von Willebrand disease]]></kwd>
<kwd lng="en"><![CDATA[Hepatitis C]]></kwd>
<kwd lng="en"><![CDATA[Liver transplant]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Hepatocellular carcinoma]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Llovet]]></surname>
<given-names><![CDATA[JM]]></given-names>
</name>
<name>
<surname><![CDATA[Burroughs]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Bruix]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<source><![CDATA[Lancet]]></source>
<year>2003</year>
<volume>362</volume>
<page-range>1907-17</page-range></nlm-citation>
</ref>
<ref id="B2">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Liver transplantation in a hemophiliac]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lewis]]></surname>
<given-names><![CDATA[JH]]></given-names>
</name>
<name>
<surname><![CDATA[Bontempo]]></surname>
<given-names><![CDATA[FA]]></given-names>
</name>
<name>
<surname><![CDATA[Spero]]></surname>
<given-names><![CDATA[JA]]></given-names>
</name>
</person-group>
<source><![CDATA[N Engl J Med]]></source>
<year>1985</year>
<volume>312</volume>
<numero>18</numero>
<issue>18</issue>
<page-range>1189-90</page-range></nlm-citation>
</ref>
<ref id="B3">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Haemophilia A and chronic hepatopathy caused by extrahepatic biliary atresia two congenital diseases cured by orthotopic liver transplantation]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Jiménez-Yuste]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Canales]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Jara]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<source><![CDATA[Haemophilia]]></source>
<year>1997</year>
<volume>3</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>145-8</page-range></nlm-citation>
</ref>
<ref id="B4">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Liver transplantation in pigs with von Willebrand's disease]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Bowie]]></surname>
<given-names><![CDATA[EJ]]></given-names>
</name>
<name>
<surname><![CDATA[Woods]]></surname>
<given-names><![CDATA[JE]]></given-names>
</name>
<name>
<surname><![CDATA[Fass]]></surname>
<given-names><![CDATA[DN]]></given-names>
</name>
</person-group>
<source><![CDATA[Br J Haematol]]></source>
<year>1975</year>
<volume>31</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>37-44</page-range></nlm-citation>
</ref>
<ref id="B5">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Liver transplantation in severe von Willebrand's disease]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Mannucci]]></surname>
<given-names><![CDATA[PM]]></given-names>
</name>
<name>
<surname><![CDATA[Federici]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Cattaneo]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<source><![CDATA[Lancet]]></source>
<year>1991</year>
<volume>337</volume>
<page-range>1105</page-range></nlm-citation>
</ref>
<ref id="B6">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Outcome of liver transplantation for haemophilia]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Yokoyama]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Bartlett]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Dar]]></surname>
<given-names><![CDATA[FS]]></given-names>
</name>
</person-group>
<source><![CDATA[HPB (Oxf)]]></source>
<year>2011</year>
<volume>13</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>40-5</page-range></nlm-citation>
</ref>
<ref id="B7">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Liver transplantation in Spanish haemophiliacs]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Aznar]]></surname>
<given-names><![CDATA[JA]]></given-names>
</name>
<name>
<surname><![CDATA[Marco]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Parra]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<source><![CDATA[Haemophilia]]></source>
<year>2012</year>
<volume>18</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>e15-6</page-range></nlm-citation>
</ref>
<ref id="B8">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Liver transplantation in the haemophilia patient]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lambing]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Kuriakose]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Kachalsky]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
</person-group>
<source><![CDATA[Haemophilia]]></source>
<year>2012</year>
<volume>18</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>300-3</page-range></nlm-citation>
</ref>
<ref id="B9">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Intermittent injections vs continuous infusion of factor VIII in haemophilia patients undergoing major surgery]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Batorova]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Martinowitz]]></surname>
<given-names><![CDATA[U]]></given-names>
</name>
</person-group>
<source><![CDATA[Br J Haematol]]></source>
<year>2000</year>
<volume>110</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>715-20</page-range></nlm-citation>
</ref>
<ref id="B10">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[European Haemophilia Therapy Standardisation Board Replacement therapy for invasive procedures in patients with haemophilia: literature review, European survey and recommendations]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hermans]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Altisent]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Batorova]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<source><![CDATA[Haemophilia]]></source>
<year>2009</year>
<volume>15</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>639-58</page-range></nlm-citation>
</ref>
<ref id="B11">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[HCV-related liver cancer in people with haemophilia]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Meijer]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Haagsma]]></surname>
<given-names><![CDATA[EB]]></given-names>
</name>
</person-group>
<source><![CDATA[Haemophilia]]></source>
<year>2012</year>
<volume>18</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>17-24</page-range></nlm-citation>
</ref>
<ref id="B12">
<nlm-citation citation-type="confpro">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lechler]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Stippel]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Scharrer]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Schramm]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
</person-group>
<source><![CDATA[Liver transplantation in a patient with severe von Willebrand disease type 3: levels of von Willebrand factor following transplantation]]></source>
<year>2002</year>
<conf-name><![CDATA[ 31st Hemophilia Symposium Hamburg 2000]]></conf-name>
<conf-loc>Berlín, Heidelberg </conf-loc>
<page-range>210-5</page-range></nlm-citation>
</ref>
<ref id="B13">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Modification of von Willebrand disease after liver transplantation]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Schulman]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Ericzon]]></surname>
<given-names><![CDATA[BG]]></given-names>
</name>
<name>
<surname><![CDATA[Eleborg]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
</person-group>
<source><![CDATA[Thromb Haemost]]></source>
<year>2001</year>
<volume>86</volume>
<page-range>1588-9</page-range></nlm-citation>
</ref>
<ref id="B14">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Liver transplantation in type III von Willebrand disease]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Ghinolfi]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Cecconi]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Molinari]]></surname>
<given-names><![CDATA[AC]]></given-names>
</name>
</person-group>
<source><![CDATA[Am J Transplant]]></source>
<year>2016</year>
<volume>16</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>1936-7</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
