<?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>1137-6627</journal-id>
<journal-title><![CDATA[Anales del Sistema Sanitario de Navarra]]></journal-title>
<abbrev-journal-title><![CDATA[Anales Sis San Navarra]]></abbrev-journal-title>
<issn>1137-6627</issn>
<publisher>
<publisher-name><![CDATA[Gobierno de Navarra. Departamento de Salud]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1137-66272022000200015</article-id>
<article-id pub-id-type="doi">10.23938/assn.1006</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Claves diagnósticas en el síndrome hemolítico urémico atípico: a propósito de un caso]]></article-title>
<article-title xml:lang="en"><![CDATA[Diagnostic clues in atypical hemolytic uremic syndrome: a case report]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Luquin-Irigoyen]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Armendariz-Brugos]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Vallejo-Ruiz]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Servicio Navarro de Salud-Osasunbidea Hospital Universitario de Navarra Servicio de Análisis Clínicos]]></institution>
<addr-line><![CDATA[Pamplona ]]></addr-line>
<country>España</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>08</month>
<year>2022</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>08</month>
<year>2022</year>
</pub-date>
<volume>45</volume>
<numero>2</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_arttext&amp;pid=S1137-66272022000200015&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_abstract&amp;pid=S1137-66272022000200015&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_pdf&amp;pid=S1137-66272022000200015&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen El síndrome hemolítico urémico atípico (SHUa) es una entidad clínica caracterizada por anemia hemolítica no inmune, trombopenia y fallo renal, en la que las lesiones están mediadas por un proceso de microangiopatía trombótica (MAT) sistémica. Es una patología rara y cuyo origen es una desregulación del sistema del complemento debido a mutaciones en genes del mismo que llevan a una activación incontrolada de C5 y la formación del complejo de ataque de membrana. Su correcto diagnóstico permite prescribir el tratamiento basado en Eculizumab, inhibidor de C5. Se presenta el caso clínico de una paciente gestante con SHUa, con el objetivo de destacar la importancia del diagnóstico diferencial precoz para el establecimiento temprano de un tratamiento efectivo de esta patología. Se actualiza la fisiopatología, diagnóstico y estudio genético, así como el manejo terapéutico del SHUa.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract Atypical hemolytic uremic syndrome (aHUS) is a clinical entity characterized by non-immune hemolytic anemia, thrombocytopenia and renal failure, in which lesions are mediated by a systemic thrombotic microangiopathy. It is a rare pathology whose origin is a complement system deregulation due to mutations in its genes that lead to uncontrolled activation of C5 and the formation of the membrane attack complex.- Its correct diagnosis allows us to prescribe the treatment based on Eculizumab, a C5 inhibitor. We report the case of a pregnant patient with aHUS, with the aim of highlighting the importance of early differential diagnosis to establish an early and effective treatment of this pathology. The pathophysiology, diagnosis and genetic study are updated, as well as the therapeutic management of aHUS.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Síndrome hemolítico urémico atípico]]></kwd>
<kwd lng="es"><![CDATA[Anemia hemolítica microangiopática]]></kwd>
<kwd lng="es"><![CDATA[Vía alternativa del complemento]]></kwd>
<kwd lng="es"><![CDATA[Proteínas del sistema del complemento]]></kwd>
<kwd lng="es"><![CDATA[Eculizumab]]></kwd>
<kwd lng="en"><![CDATA[Atypical hemolytic-uremic síndrome]]></kwd>
<kwd lng="en"><![CDATA[Microangiopathic hemolytic anemia]]></kwd>
<kwd lng="en"><![CDATA[Complement pathway alternative]]></kwd>
<kwd lng="en"><![CDATA[Complement System Proteins]]></kwd>
<kwd lng="en"><![CDATA[Eculizumab]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<label>1</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Noris]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Remuzzi]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Atypical hemolytic-uremic syndrome]]></article-title>
<source><![CDATA[N Engl J Med]]></source>
<year>2009</year>
<volume>361</volume>
<page-range>1676-87</page-range></nlm-citation>
</ref>
<ref id="B2">
<label>2</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Timmermans]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Werion]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Spaanderman]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Reutelingsperger]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Damoiseaux]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Morelle]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The natural course of pregnancies in women with primary atypical haemolytic uraemic syndrome and asymptomatic relatives]]></article-title>
<source><![CDATA[Br J Haematol]]></source>
<year>2020</year>
<volume>190</volume>
<page-range>442-9</page-range></nlm-citation>
</ref>
<ref id="B3">
<label>3</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Campistol]]></surname>
<given-names><![CDATA[JM]]></given-names>
</name>
<name>
<surname><![CDATA[Arias]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Ariceta]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Blasco]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Espinosa]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Espinosa]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Actualización en síndrome hemolítico urémico atípico: diagnóstico y tratamiento. Documento de consenso]]></article-title>
<source><![CDATA[Nefrología]]></source>
<year>2015</year>
<volume>35</volume>
<page-range>421-47</page-range></nlm-citation>
</ref>
<ref id="B4">
<label>4</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Noris]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Caprioli]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Bresin]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Mossali]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Pianetti]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Gamba]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype]]></article-title>
<source><![CDATA[Clin J Am Soc Nephrol]]></source>
<year>2010</year>
<volume>5</volume>
<page-range>1844-59</page-range></nlm-citation>
</ref>
<ref id="B5">
<label>5</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Pereira-Victorio]]></surname>
<given-names><![CDATA[CJ]]></given-names>
</name>
<name>
<surname><![CDATA[Vázquez-Molinero]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Lizán-García]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Luis]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[González]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Síndrome de HELLP en atención primaria]]></article-title>
<source><![CDATA[Med Gen y Fam]]></source>
<year>2016</year>
<volume>5</volume>
<page-range>83-90</page-range></nlm-citation>
</ref>
<ref id="B6">
<label>6</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Fakhouri]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Roumenina]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Provot]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Sallée]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Caillard]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Couzi]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations]]></article-title>
<source><![CDATA[J Am Soc Nephrol]]></source>
<year>2010</year>
<volume>21</volume>
<page-range>859-67</page-range></nlm-citation>
</ref>
<ref id="B7">
<label>7</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Nester]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Barbour]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Rodriguez-de-Cordoba]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Dragon-Durey]]></surname>
<given-names><![CDATA[MA]]></given-names>
</name>
<name>
<surname><![CDATA[Fremeaux-Bacchi]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Goodship]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Atypical aHUS: state of the art]]></article-title>
<source><![CDATA[Mol Immunol]]></source>
<year>2015</year>
<volume>67</volume>
<page-range>31-42</page-range></nlm-citation>
</ref>
<ref id="B8">
<label>8</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Shah]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Rutherford]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Matevosyan]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Shen]]></surname>
<given-names><![CDATA[YM]]></given-names>
</name>
<name>
<surname><![CDATA[Sarode]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Role of ADAMTS13 in the management of thrombotic microangiopathies including thrombotic thrombocytopenic purpura (TTP)]]></article-title>
<source><![CDATA[Br J Haematol]]></source>
<year>2013</year>
<volume>163</volume>
<page-range>514-9</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9</label><nlm-citation citation-type="book">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Noris]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Bresin]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Mele]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Remuzzi]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Adam]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Ardinger]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Genetic atypical hemolytic-uremic syndrome]]></article-title>
<source><![CDATA[GeneReviews]]></source>
<year>2021</year>
<publisher-loc><![CDATA[Seattle(WA) ]]></publisher-loc>
<publisher-name><![CDATA[University of Washington]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B10">
<label>10</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Roumenina]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Frimat]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Miller]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Provot]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Dragon-Durey]]></surname>
<given-names><![CDATA[MA]]></given-names>
</name>
<name>
<surname><![CDATA[Bordereau]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[A prevalent C3 mutation in aHUS patients causes a direct C3 convertase gain of function]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2012</year>
<volume>119</volume>
<page-range>4182-91</page-range></nlm-citation>
</ref>
<ref id="B11">
<label>11</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Martinez-Barricarte]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Meike]]></surname>
<given-names><![CDATA[Heurich]]></given-names>
</name>
<name>
<surname><![CDATA[López-Perrote]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Tortajada]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Pinto]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[López-Trascasa]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The molecular and structural bases for the association of complement C3 mutations with atypical hemolytic uremic syndrome]]></article-title>
<source><![CDATA[Mol Immunol]]></source>
<year>2015</year>
<volume>66</volume>
<page-range>263-73</page-range></nlm-citation>
</ref>
<ref id="B12">
<label>12</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Bresin]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Rurali]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Caprioli]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Sanchez-Corral]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Fremeaux-Bacchi]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Rodriguez-de-Cordoba]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Combined complement gene mutations in atypical hemolytic uremic syndrome influence clinical phenotype]]></article-title>
<source><![CDATA[J Am Soc Nephrol]]></source>
<year>2013</year>
<volume>24</volume>
<page-range>475-86</page-range></nlm-citation>
</ref>
<ref id="B13">
<label>13</label><nlm-citation citation-type="">
<collab>Agencia Europea del Medicamento</collab>
<source><![CDATA[Soliris (eculizumab). Ficha técnica]]></source>
<year>2012</year>
</nlm-citation>
</ref>
<ref id="B14">
<label>14</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Fakhouri]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Fila]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Hummel]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Ribes]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Sellier-Leclerc]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Ville]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Eculizumab discontinuation in children and adults with atypical hemolytic-uremic syndrome: a prospective multicenter study]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2021</year>
<volume>137</volume>
<page-range>2438-49</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
