<?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>0872-0169</journal-id>
<journal-title><![CDATA[Portuguese Journal of Nephrology & Hypertension]]></journal-title>
<abbrev-journal-title><![CDATA[Port J Nephrol Hypert]]></abbrev-journal-title>
<issn>0872-0169</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Nefrologia]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0872-01692021000300203</article-id>
<article-id pub-id-type="doi">10.32932/pjnh.2021.10.148</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Alagille Syndrome: An ultrafiltration dilema]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Abrantes]]></surname>
<given-names><![CDATA[Catarina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Furtado]]></surname>
<given-names><![CDATA[Teresa]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Domingues]]></surname>
<given-names><![CDATA[Patrícia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Valério]]></surname>
<given-names><![CDATA[Patrícia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Felgueiras]]></surname>
<given-names><![CDATA[Joana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Assunção]]></surname>
<given-names><![CDATA[José]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Vaz]]></surname>
<given-names><![CDATA[Álvaro]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Setubal Hospital Center Nephrology Department ]]></institution>
<addr-line><![CDATA[Setubal ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>30</day>
<month>09</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>30</day>
<month>09</month>
<year>2021</year>
</pub-date>
<volume>35</volume>
<numero>3</numero>
<fpage>203</fpage>
<lpage>204</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S0872-01692021000300203&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S0872-01692021000300203&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S0872-01692021000300203&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT Alagille syndrome (AGLS) is a rare genetic disorder caused by mutations in the Notch signaling pathway. Multiple organ dysfunction is frequent despite phenotypic variability. We report the case of an AGLS patient with right heart failure and persistent fluid overload who started peritoneal ultrafiltration, without initial benefit. Possible pathophysiological mechanisms related to the underlying genetic condition, namely vascular abnormalities, that could help explain the poor ultrafiltration are provided, while other ultrafiltration failure causes are briefly discussed. New evidence is necessary for a better understanding of this syndrome in PD modality.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Alagille syndrome]]></kwd>
<kwd lng="en"><![CDATA[JAG1 mutation]]></kwd>
<kwd lng="en"><![CDATA[ultrafiltration]]></kwd>
<kwd lng="en"><![CDATA[peritoneal dialysis.]]></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[Turnpenny]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Ellard]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Alagille Syndrome: pathogenesis, diagnosis and management]]></article-title>
<source><![CDATA[Eur J Med Genet]]></source>
<year>2012</year>
<volume>20</volume>
<page-range>251-7</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[Mitchell]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Gilbert]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Loomes]]></surname>
<given-names><![CDATA[KM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Alagille Syndrome]]></article-title>
<source><![CDATA[Clin Liver Dis]]></source>
<year>2018</year>
<volume>22</volume>
<page-range>625-41</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[Kamath]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Podkameni]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Hutchinson]]></surname>
<given-names><![CDATA[AL]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Renal anomalies in Alagille Syndrome: a diseasedefining feature]]></article-title>
<source><![CDATA[Am J Med Genet A]]></source>
<year>2012</year>
<volume>158A</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>85-9</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[Kamath]]></surname>
<given-names><![CDATA[BM]]></given-names>
</name>
<name>
<surname><![CDATA[Spinner]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Rosenblum]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Renal involvement and the role of Notch signalling in Alagille Syndrome]]></article-title>
<source><![CDATA[Nat Rev Nephrol]]></source>
<year>2013</year>
<volume>9</volume>
<page-range>409-18</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[Bissonnette]]></surname>
<given-names><![CDATA[MZ]]></given-names>
</name>
<name>
<surname><![CDATA[Lane]]></surname>
<given-names><![CDATA[JC]]></given-names>
</name>
<name>
<surname><![CDATA[Chang]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Extreme renal pathology in Alagille Syndrome]]></article-title>
<source><![CDATA[Kidney Int Rep]]></source>
<year>2017</year>
<volume>2</volume>
<page-range>493-7</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[Berta Sousa]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Medeira]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Kamath]]></surname>
<given-names><![CDATA[BM]]></given-names>
</name>
<name>
<surname><![CDATA[Spinner]]></surname>
<given-names><![CDATA[NB]]></given-names>
</name>
<name>
<surname><![CDATA[Cordeiro]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Familial stenosis of the pulmonar artery branches with a JAG1 mutation]]></article-title>
<source><![CDATA[Rev Port Cardiol]]></source>
<year>2006</year>
<volume>25</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>447-52</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[Lu]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Muciño-Bermejo]]></surname>
<given-names><![CDATA[MJ]]></given-names>
</name>
<name>
<surname><![CDATA[Ribeiro]]></surname>
<given-names><![CDATA[LC]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Peritoneal dialysis in patients with refractory congestive heart failure: a systematic review]]></article-title>
<source><![CDATA[Cardiorenal Med]]></source>
<year>2015</year>
<volume>5</volume>
<page-range>145-56</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[Fröhlich]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Katus]]></surname>
<given-names><![CDATA[HA]]></given-names>
</name>
<name>
<surname><![CDATA[Täger]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Peritoneal ultrafiltration in end-stage chronic heart failure]]></article-title>
<source><![CDATA[Clin Kidney J]]></source>
<year>2015</year>
<volume>8</volume>
<page-range>219-25</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Silver]]></surname>
<given-names><![CDATA[SA]]></given-names>
</name>
<name>
<surname><![CDATA[Harel]]></surname>
<given-names><![CDATA[Z]]></given-names>
</name>
<name>
<surname><![CDATA[Perl]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Practical considerations when prescribing icodextrin: a narrative review]]></article-title>
<source><![CDATA[Am J Nephrol]]></source>
<year>2014</year>
<volume>39</volume>
<page-range>515-27</page-range></nlm-citation>
</ref>
<ref id="B10">
<label>10</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Teitelbaum]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Ultrafiltration failure in Peritoneal dialysis: a pathophysiologic approach]]></article-title>
<source><![CDATA[Blood Purif]]></source>
<year>2015</year>
<volume>39</volume>
<page-range>70-3</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[Kamath]]></surname>
<given-names><![CDATA[BM]]></given-names>
</name>
<name>
<surname><![CDATA[Spinner]]></surname>
<given-names><![CDATA[NB]]></given-names>
</name>
<name>
<surname><![CDATA[Emerick]]></surname>
<given-names><![CDATA[KM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Vascular anomalies in Alagille Syndrome - a significant cause of morbidity and mortality]]></article-title>
<source><![CDATA[Circulation]]></source>
<year>2004</year>
<volume>109</volume>
<page-range>1354-8</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[Zavadil]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Cermak]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Soto-Nieves]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Böttinger]]></surname>
<given-names><![CDATA[EP]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Integration of TGF-&#946;/Smad and Jagged1/Notch signalling in epithelial-to-mesenchymal transition]]></article-title>
<source><![CDATA[EMBO J]]></source>
<year>2004</year>
<volume>23</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>1155-65</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
