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<!--
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UID=3036
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ConceptID=C0008313
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-->
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<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Sclerosing cholangitis</div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>3036</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C0008313</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>Synonyms:</td>
|
||
<td>Cholangiitides, Sclerosing; Cholangiitis, Sclerosing; Cholangitides, Sclerosing; Cholangitis, Sclerosing; Sclerosing Cholangiitides; Sclerosing Cholangiitis; Sclerosing Cholangitides; Sclerosing Cholangitis</td></tr>
|
||
<tr><td><span class="bold">SNOMED CT: </span></td>
|
||
<td>Sclerosing cholangitis (235917005)</td></tr>
|
||
<tr><td colspan="2" class="small"> </td></tr><tr><td>HPO:</td>
|
||
<td><a target="_blank" title="Human Phenotype Ontology" href="https://hpo.jax.org/app/browse/term/HP:0030991">HP:0030991</a></td></tr>
|
||
<tr><td>Monarch Initiative:</td>
|
||
<td><a href="https://monarchinitiative.org/disease/MONDO:0018646" target="_blank">MONDO:0018646</a></td></tr>
|
||
<tr><td>Orphanet:</td>
|
||
<td><a target="_blank" title="Orphanet: The portal for rare diseases and orphan drugs" href="http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=447771">ORPHA447771</a></td></tr></tbody></table></div><div class="rprt-body jig-ncbiinpagenav" data-jigconfig="smoothScroll: false, gotoTopLink: true, gotoTopLinkText: '', gotoTopLinkAttrs: {'title': 'Go to the top of the page'},allHeadingLevels: ['h1'], topOfPageTOC: true, tocId: 'my-toc'"><div id="rprt-tabs-1" class="rprt-tab"><div id="tb-termsProp-1"><div class="leftCol mgCol"><div>
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<div class="portlet mgSection" id="ID_100">
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Definition">Definition</h1><a sid="100" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
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||
<div class="portlet_content ln">Cholangitis associated with evident ductal fibrosis that develops as a consequence of long-standing bile duct inflammatory, obstruction, or ischemic injury; it can be obliterative or nonobliterative. [from <a title="Human Phenotype Ontology" href="http://www.human-phenotype-ontology.org" class="defSource" target="_blank">HPO</a>]</div>
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Term_Hierarchy">Term Hierarchy</h1><a sid="118" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
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<div class="portlet_content ln HierarchyGTR"><div class="jig-ncbitabs"><ul><li><a href="#tabGTR">GTR</a></li><li><a href="#tabMGEN">MeSH</a></li></ul><div id="tabGTR"><div class="search_result"><div class="rprts"><div class="chiclet_legend"><span class="chiclet_list" style="position:static;"><span title="Clinical test" class="chiclet Ccolor round">C</span><span>Clinical test, </span><span title="Research test" class="chiclet Rcolor round">R</span><span>Research test, </span><span title="OMIM" class="chiclet Ocolor ">O</span><span>OMIM, </span><span title="GeneReview" class="chiclet Gcolor">G</span><span><em>GeneReviews</em>, </span><span title="ClinVar" class="chiclet Vcolor">V</span><span>ClinVar </span></span></div><div id="hierarchy" class="margin_t1"><div class="ds_tree"><ul><li class="matched_ds"><span class="chiclet_list"><span class="chiclet unavailable round" title="Clinical test">C</span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet unavailable" title="OMIM">O</span><span class="chiclet unavailable" title="GeneReviews">G</span><span class="chiclet unavailable" title="ClinVar">V</span></span><span class="TLline">Sclerosing cholangitis</span></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><ul><li><span class="TLline"><a href="/medgen/232130" ref="tree=MeSH" title="MedGen record for Disorder by Site">Disorder by Site</a></span><ul><li><span class="TLline"><a href="/medgen/8970" ref="tree=MeSH" title="MedGen record for Disorder of gastrointestinal tract">Disorder of gastrointestinal tract</a></span><ul><li><span class="TLline"><a href="/medgen/82758" ref="tree=MeSH" title="MedGen record for Hepatobiliary disorder">Hepatobiliary disorder</a></span><ul><li><span class="TLline"><a href="/medgen/476757" ref="tree=MeSH" title="MedGen record for Biliary System Disorder">Biliary System Disorder</a></span><ul><li><span class="TLline"><a href="/medgen/2240" ref="tree=MeSH" title="MedGen record for Bile duct disorder">Bile duct disorder</a></span><ul><li><span class="TLline"><a href="/medgen/40258" ref="tree=MeSH" title="MedGen record for Cholangitis">Cholangitis</a></span><ul><li><span class="matched_ds">Sclerosing cholangitis</span><ul><li><span class="TLline"><a href="/medgen/927778" ref="tree=MeSH" title="MedGen record for IgG4-related sclerosing cholangitis">IgG4-related sclerosing cholangitis</a></span></li><li><span class="TLline"><a href="/medgen/1393230" ref="tree=MeSH" title="MedGen record for Isolated neonatal sclerosing cholangitis">Isolated neonatal sclerosing cholangitis</a></span></li><li><span class="TLline"><a href="/medgen/334382" ref="tree=MeSH" title="MedGen record for Neonatal ichthyosis-sclerosing cholangitis syndrome">Neonatal ichthyosis-sclerosing cholangitis syndrome</a></span></li><li><span class="TLline"><a href="/medgen/107565" ref="tree=MeSH" title="MedGen record for Primary sclerosing cholangitis">Primary sclerosing cholangitis</a></span></li><li><span class="TLline"><a href="/medgen/586530" ref="tree=MeSH" title="MedGen record for Secondary sclerosing cholangitis">Secondary sclerosing cholangitis</a></span></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></div></div></div></div>
|
||
</div>
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||
<div class="portlet mgSection" id="ID_112">
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||
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Conditions_with_this_feature">Conditions with this feature</h1><a sid="112" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln clinfeat">
|
||
<div class="divPopper rprt" id="rdis_96019"><div><strong>Hyper-IgM syndrome type 1</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>96019</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information."><span class="highlight" style="background-color:">C0398689</span></a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">X-linked hyper IgM syndrome (HIGM1), a disorder of abnormal T- and B-cell function, is characterized by low serum concentrations of IgG, IgA, and IgE with normal or elevated serum concentrations of IgM. Mitogen proliferation may be normal, but NK- and T-cell cytotoxicity can be impaired. Antigen-specific responses are usually decreased or absent. Total numbers of B cells are normal but there is a marked reduction of class-switched memory B cells. Defective oxidative burst of both neutrophils and macrophages has been reported. The range of clinical findings varies, even within the same family. More than 50% of males with HIGM1 develop symptoms by age one year, and more than 90% are symptomatic by age four years. HIGM1 usually presents in infancy with recurrent upper- and lower-respiratory tract bacterial infections, opportunistic infections including Pneumocystis jirovecii pneumonia, and recurrent or protracted diarrhea that can be infectious or noninfectious and is associated with failure to thrive. Neutropenia is common; thrombocytopenia and anemia are less commonly seen. Autoimmune and/or inflammatory disorders (such as sclerosing cholangitis) as well as increased risk for neoplasms have been reported as medical complications of this disorder. Significant neurologic complications, often the result of a CNS infection, are seen in 5%-15% of affected males. Liver disease, a serious complication of HIGM1 once observed in more than 80% of affected males by age 20 years, may be decreasing with adequate screening and treatment of Cryptosporidium infection.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/96019">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_334382"><div><strong>Neonatal ichthyosis-sclerosing cholangitis syndrome</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>334382</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C1843355</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Ichthyosis, leukocyte vacuoles, alopecia, and sclerosing cholangitis (ILVASC) is a rare autosomal recessive syndrome characterized by scalp hypotrichosis, scarring alopecia, ichthyosis, and sclerosing cholangitis (summary by Feldmeyer et al., 2006).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/334382">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_347904"><div><strong>MHC class II deficiency 2</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>347904</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C1859535</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/347904">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_1393230"><div><strong>Isolated neonatal sclerosing cholangitis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1393230</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C4479344</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Neonatal sclerosing cholangitis (NSC) is a rare autosomal recessive form of severe liver disease with onset in infancy. Affected infants have jaundice, cholestasis, acholic stools, and progressive liver dysfunction resulting in fibrosis and cirrhosis; most require liver transplantation in the first few decades of life. Cholangiography shows patent biliary ducts, but there are bile duct irregularities (summary by Girard et al., 2016; Grammatikopoulos et al., 2016).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/1393230">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_1648410"><div><strong>Combined immunodeficiency due to DOCK8 deficiency</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1648410</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C4722305</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Hyper-IgE syndrome-2 with recurrent infections (HIES2) is an autosomal recessive immunologic disorder characterized by recurrent staphylococcal infections of the skin and respiratory tract, eczema, elevated serum immunoglobulin E, and hypereosinophilia. It is distinguished from autosomal dominant HIES1 (147060) by the lack of connective tissue and skeletal involvement (Renner et al., 2004). For a discussion of genetic heterogeneity of hyper-IgE syndrome, see 147060. See also TYK2 deficiency (611521), a clinically distinct disease entity that includes characteristic features of both autosomal recessive HIES2 and mendelian susceptibility to mycobacterial disease (MSMD; 209950) (Minegishi et al., 2006).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/1648410">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_1794249"><div><strong>Immunodeficiency 92</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1794249</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C5562039</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Immunodeficiency-92 (IMD92) is an autosomal recessive primary immunodeficiency characterized by the onset of recurrent infections in infancy or early childhood. Infectious agents are broad, including bacterial, viral, fungal, and parasitic, including Cryptosporidium and Mycobacteria. Patient lymphocytes show defects in both T- and B-cell proliferation, cytokine secretion, and overall function, and there is also evidence of dysfunction of NK, certain antigen-presenting cells, and myeloid subsets. Hematopoietic stem cell transplantation may be curative (summary by Beaussant-Cohen et al., 2019 and Levy et al., 2021).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/1794249">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_1794255"><div><strong>Cholestasis, progressive familial intrahepatic, 8</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1794255</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C5562045</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Progressive familial intrahepatic cholestasis-8 (PFIC8) is an autosomal recessive disorder characterized by cholestasis and high gamma-glutamyltransferase presenting in the infantile period (summary by Unlusoy Aksu et al., 2019). For a general phenotypic description and a discussion of genetic heterogeneity of PFIC, see PFIC1 (211600).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/1794255">Condition Record</a></div></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1794255" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Cholestasis, progressive familial intrahepatic, 8</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1648410" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Combined immunodeficiency due to DOCK8 deficiency</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_96019" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Hyper-IgM syndrome type 1</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1794249" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Immunodeficiency 92</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1393230" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Isolated neonatal sclerosing cholangitis</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_347904" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">MHC class II deficiency 2</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_334382" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Neonatal ichthyosis-sclerosing cholangitis syndrome</a></div></div>
|
||
</div>
|
||
|
||
<div class="portlet mgSection" id="ID_105">
|
||
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Professional_guidelines">Professional guidelines</h1><a sid="105" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln"><h3 class="subhead">PubMed<a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#guidelinesHelpPM"><img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a></h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/34358489">Extraintestinal Manifestations of Inflammatory Bowel Disease: Current Concepts, Treatment, and Implications for Disease Management.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Rogler G,
|
||
Singh A,
|
||
Kavanaugh A,
|
||
Rubin DT</span><br />
|
||
<span class="medgenPMjournal">Gastroenterology</span>
|
||
2021 Oct;161(4):1118-1132.
|
||
Epub 2021 Aug 3
|
||
doi: 10.1053/j.gastro.2021.07.042.
|
||
<span class="bold">PMID: </span><a href="/pubmed/34358489" target="_blank">34358489</a><a href="/pmc/articles/PMC8564770" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/27995906">ACG Clinical Guideline: Evaluation of Abnormal Liver Chemistries.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Kwo PY,
|
||
Cohen SM,
|
||
Lim JK</span><br />
|
||
<span class="medgenPMjournal">Am J Gastroenterol</span>
|
||
2017 Jan;112(1):18-35.
|
||
Epub 2016 Dec 20
|
||
doi: 10.1038/ajg.2016.517.
|
||
<span class="bold">PMID: </span><a href="/pubmed/27995906" target="_blank">27995906</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/24140396">Pathogenesis, diagnosis, and management of cholangiocarcinoma.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Ilyas SI,
|
||
Gores GJ</span><br />
|
||
<span class="medgenPMjournal">Gastroenterology</span>
|
||
2013 Dec;145(6):1215-29.
|
||
Epub 2013 Oct 15
|
||
doi: 10.1053/j.gastro.2013.10.013.
|
||
<span class="bold">PMID: </span><a href="/pubmed/24140396" target="_blank">24140396</a><a href="/pmc/articles/PMC3862291" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22sclerosing%20cholangitis%22%5Btiab%3A~0%5D)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">See all (457)</a></div></div>
|
||
</div>
|
||
<div class="display-none help-popup" id="guidelinesHelpPM">These guidelines are articles in PubMed that match specific search criteria developed by MedGen to capture the most relevant practice guidelines. This list may not be comprehensive and may include broader topics as well. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div><div class="display-none help-popup" id="guidelinesHelpCurated">These guidelines are manually curated by the MedGen team
|
||
to supplement articles available in PubMed. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div>
|
||
<div class="portlet mgSection" id="ID_103">
|
||
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_clinical_studies">Recent clinical studies</h1><a sid="103" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln"><h3 class="subhead">Etiology</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/37084929">Recent Advances in the Management of Primary Sclerosing Cholangitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Assis DN,
|
||
Bowlus CL</span><br />
|
||
<span class="medgenPMjournal">Clin Gastroenterol Hepatol</span>
|
||
2023 Jul;21(8):2065-2075.
|
||
Epub 2023 Apr 19
|
||
doi: 10.1016/j.cgh.2023.04.004.
|
||
<span class="bold">PMID: </span><a href="/pubmed/37084929" target="_blank">37084929</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/31154395">British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Chapman MH,
|
||
Thorburn D,
|
||
Hirschfield GM,
|
||
Webster GGJ,
|
||
Rushbrook SM,
|
||
Alexander G,
|
||
Collier J,
|
||
Dyson JK,
|
||
Jones DE,
|
||
Patanwala I,
|
||
Thain C,
|
||
Walmsley M,
|
||
Pereira SP</span><br />
|
||
<span class="medgenPMjournal">Gut</span>
|
||
2019 Aug;68(8):1356-1378.
|
||
Epub 2019 Jun 1
|
||
doi: 10.1136/gutjnl-2018-317993.
|
||
<span class="bold">PMID: </span><a href="/pubmed/31154395" target="_blank">31154395</a><a href="/pmc/articles/PMC6691863" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/29452711">Primary sclerosing cholangitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Dyson JK,
|
||
Beuers U,
|
||
Jones DEJ,
|
||
Lohse AW,
|
||
Hudson M</span><br />
|
||
<span class="medgenPMjournal">Lancet</span>
|
||
2018 Jun 23;391(10139):2547-2559.
|
||
Epub 2018 Feb 13
|
||
doi: 10.1016/S0140-6736(18)30300-3.
|
||
<span class="bold">PMID: </span><a href="/pubmed/29452711" target="_blank">29452711</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/28802875">Primary sclerosing cholangitis - a comprehensive review.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Karlsen TH,
|
||
Folseraas T,
|
||
Thorburn D,
|
||
Vesterhus M</span><br />
|
||
<span class="medgenPMjournal">J Hepatol</span>
|
||
2017 Dec;67(6):1298-1323.
|
||
Epub 2017 Aug 10
|
||
doi: 10.1016/j.jhep.2017.07.022.
|
||
<span class="bold">PMID: </span><a href="/pubmed/28802875" target="_blank">28802875</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/20955968">Primary sclerosing cholangitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Karlsen TH,
|
||
Schrumpf E,
|
||
Boberg KM</span><br />
|
||
<span class="medgenPMjournal">Best Pract Res Clin Gastroenterol</span>
|
||
2010 Oct;24(5):655-66.
|
||
doi: 10.1016/j.bpg.2010.07.005.
|
||
<span class="bold">PMID: </span><a href="/pubmed/20955968" target="_blank">20955968</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Sclerosing%20cholangitis%22%20AND%20Etiology%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (2965)</a></div><h3 class="subhead">Diagnosis</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/38250951">Primary Sclerosing Cholangitis: Diagnostic Criteria.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Cazzagon N,
|
||
Sarcognato S,
|
||
Catanzaro E,
|
||
Bonaiuto E,
|
||
Peviani M,
|
||
Pezzato F,
|
||
Motta R</span><br />
|
||
<span class="medgenPMjournal">Tomography</span>
|
||
2024 Jan 7;10(1):47-65.
|
||
doi: 10.3390/tomography10010005.
|
||
<span class="bold">PMID: </span><a href="/pubmed/38250951" target="_blank">38250951</a><a href="/pmc/articles/PMC10820917" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/33038000">Prednisolone-responsive primary sclerosing cholangitis with autoimmune hemolytic anemia: a case report and review of the literature.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Kawaguchi T,
|
||
Arinaga-Hino T,
|
||
Morishige S,
|
||
Mizuochi S,
|
||
Abe M,
|
||
Kunitake K,
|
||
Sano T,
|
||
Amano K,
|
||
Kuwahara R,
|
||
Ide T,
|
||
Nagafuji K,
|
||
Torimura T</span><br />
|
||
<span class="medgenPMjournal">Clin J Gastroenterol</span>
|
||
2021 Feb;14(1):330-335.
|
||
Epub 2020 Oct 10
|
||
doi: 10.1007/s12328-020-01256-8.
|
||
<span class="bold">PMID: </span><a href="/pubmed/33038000" target="_blank">33038000</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/31154395">British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Chapman MH,
|
||
Thorburn D,
|
||
Hirschfield GM,
|
||
Webster GGJ,
|
||
Rushbrook SM,
|
||
Alexander G,
|
||
Collier J,
|
||
Dyson JK,
|
||
Jones DE,
|
||
Patanwala I,
|
||
Thain C,
|
||
Walmsley M,
|
||
Pereira SP</span><br />
|
||
<span class="medgenPMjournal">Gut</span>
|
||
2019 Aug;68(8):1356-1378.
|
||
Epub 2019 Jun 1
|
||
doi: 10.1136/gutjnl-2018-317993.
|
||
<span class="bold">PMID: </span><a href="/pubmed/31154395" target="_blank">31154395</a><a href="/pmc/articles/PMC6691863" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/29452711">Primary sclerosing cholangitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Dyson JK,
|
||
Beuers U,
|
||
Jones DEJ,
|
||
Lohse AW,
|
||
Hudson M</span><br />
|
||
<span class="medgenPMjournal">Lancet</span>
|
||
2018 Jun 23;391(10139):2547-2559.
|
||
Epub 2018 Feb 13
|
||
doi: 10.1016/S0140-6736(18)30300-3.
|
||
<span class="bold">PMID: </span><a href="/pubmed/29452711" target="_blank">29452711</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/28802875">Primary sclerosing cholangitis - a comprehensive review.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Karlsen TH,
|
||
Folseraas T,
|
||
Thorburn D,
|
||
Vesterhus M</span><br />
|
||
<span class="medgenPMjournal">J Hepatol</span>
|
||
2017 Dec;67(6):1298-1323.
|
||
Epub 2017 Aug 10
|
||
doi: 10.1016/j.jhep.2017.07.022.
|
||
<span class="bold">PMID: </span><a href="/pubmed/28802875" target="_blank">28802875</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Sclerosing%20cholangitis%22%20AND%20Diagnosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (3369)</a></div><h3 class="subhead">Therapy</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/38943194">Causal relationship between primary sclerosing cholangitis and systemic lupus erythematosus: a bidirectional Mendelian randomization study.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Pan Z,
|
||
Zhang W</span><br />
|
||
<span class="medgenPMjournal">Eur J Med Res</span>
|
||
2024 Jun 28;29(1):351.
|
||
doi: 10.1186/s40001-024-01941-1.
|
||
<span class="bold">PMID: </span><a href="/pubmed/38943194" target="_blank">38943194</a><a href="/pmc/articles/PMC11212221" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
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<div class="portlet_content ln"><span class="medgenPMauthor">Tahboub Amawi AD,
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<div class="nl"><a target="_blank" href="/pubmed/22032524">Transplantation for cholangiocarcinoma.</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/34358489">Extraintestinal Manifestations of Inflammatory Bowel Disease: Current Concepts, Treatment, and Implications for Disease Management.</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/33038000">Prednisolone-responsive primary sclerosing cholangitis with autoimmune hemolytic anemia: a case report and review of the literature.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Kawaguchi T,
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Arinaga-Hino T,
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Morishige S,
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Mizuochi S,
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Abe M,
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Kunitake K,
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Sano T,
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Amano K,
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Kuwahara R,
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Ide T,
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Nagafuji K,
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<div class="nl"><a target="_blank" href="/pubmed/25469007">Ulcerative colitis-associated colorectal cancer.</a></div>
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<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Sclerosing%20cholangitis%22%20AND%20Clinical%20prediction%20guides%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (1599)</a></div></div>
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</div>
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<div class="portlet mgSection" id="ID_104">
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_systematic_reviews">Recent systematic reviews</h1><a sid="104" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln">
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<div class="nl"><a target="_blank" href="/pubmed/38052097">Incidence and Prevalence of Primary Sclerosing Cholangitis: A Meta-analysis of Population-based Studies.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Cooper J,
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Markovinovic A,
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Coward S,
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Herauf M,
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Shaheen AA,
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Swain M,
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Panaccione R,
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Ma C,
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Lu C,
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Novak K,
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Kroeker KI,
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Ng SC,
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Kaplan GG</span><br />
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<span class="bold">PMID: </span><a href="/pubmed/38052097" target="_blank">38052097</a><a href="/pmc/articles/PMC11532590" target="_blank" class="PubMedFree">Free PMC Article</a></div>
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||
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<div class="nl"><a target="_blank" href="/pubmed/34425093">Prevalence of Primary Sclerosing Cholangitis in Patients With Inflammatory Bowel Disease: A Systematic Review and Meta-analysis.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Barberio B,
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Massimi D,
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Cazzagon N,
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Zingone F,
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Ford AC,
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Savarino EV</span><br />
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<span class="medgenPMjournal">Gastroenterology</span>
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2021 Dec;161(6):1865-1877.
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Epub 2021 Aug 20
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<span class="bold">PMID: </span><a href="/pubmed/34425093" target="_blank">34425093</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/33385426">Prognostic Factors for Advanced Colorectal Neoplasia in Inflammatory Bowel Disease: Systematic Review and Meta-analysis.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Wijnands AM,
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de Jong ME,
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Lutgens MWMD,
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Hoentjen F,
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Elias SG,
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Oldenburg B;
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Dutch Initiative on Crohn and Colitis (ICC)</span><br />
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2021 Apr;160(5):1584-1598.
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Epub 2020 Dec 29
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<span class="bold">PMID: </span><a href="/pubmed/33385426" target="_blank">33385426</a></div>
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||
<div class="nl"><a target="_blank" href="/pubmed/29356770">Diagnosis and Management of Pediatric Autoimmune Liver Disease: ESPGHAN Hepatology Committee Position Statement.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Mieli-Vergani G,
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Vergani D,
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Debray D,
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Fischler B,
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Gupte G,
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Indolfi G,
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<span class="bold">PMID: </span><a href="/pubmed/29356770" target="_blank">29356770</a></div>
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||
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||
<div class="nl"><a target="_blank" href="/pubmed/28478443">Immunosuppressive Agents for the Treatment of Primary Sclerosing Cholangitis: A Systematic Review and Meta-Analysis.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Peng X,
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Luo X,
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Hou JY,
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Li LZ,
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Zheng MH,
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<span class="medgenPMjournal">Dig Dis</span>
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<span class="bold">PMID: </span><a href="/pubmed/28478443" target="_blank">28478443</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Sclerosing%20cholangitis%22%20AND%20systematic%5Bsb%5D%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (89)</a></div></div>
|
||
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|
||
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<h2 class="offscreen_noflow">Supplemental Content</h2>
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<div class="portlet_content ln"><ul><li><a href="http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Expert=447771" target="_blank">Orphanet</a></li><li><a href="https://clinicaltrials.gov/search?cond=Sclerosing%20cholangitis" target="_blank">ClinicalTrials.gov</a></li></ul></div>
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<div class="portlet_content ln"><ul class="a_poppers"><li><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22sclerosing%20cholangitis%22%5Btiab%3A~0%5D)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">PubMed</a><div class="help-popup">See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div></li><li><a target="_blank" href="/books/?term=((%22clinical%20guidelines%22%5BResource%20Type%5D)%20OR%20%22practice%20guideline%22%5BPublication%20Type%5D)%20AND%20(%22Sclerosing%20cholangitis%22)">Bookshelf</a><div class="help-popup">See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div></li></ul></div>
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