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<!--
|
||
UID=1825946
|
||
ConceptID=C5679820
|
||
-->
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||
<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Idiopathic and/or familial pulmonary arterial hypertension</div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1825946</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.">C5679820</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>Synonym:</td>
|
||
<td>Idiopathic/heritable pulmonary arterial hypertension</td></tr>
|
||
<tr><td>Modes of inheritance:</td>
|
||
<td>
|
||
<div class="divPopper rprt" id="moi_141025"><div><strong>Autosomal recessive inheritance</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>141025</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.">C0441748</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Intellectual Product</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Source: Orphanet</div>
|
||
<div class="spaceAbove">A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).</div></div>
|
||
<div class="divPopper rprt" id="moi_141047"><div><strong>Autosomal dominant inheritance</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>141047</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.">C0443147</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Intellectual Product</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Source: Orphanet</div>
|
||
<div class="spaceAbove">A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.</div></div>
|
||
<div class="divPopper rprt" id="moi_988794"><div><strong>Not genetically inherited</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>988794</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier assigned by MedGen (starting with CN) for terms that cannot be identified in NLM's Unified Medical Language system (UMLS) Click for more information.">CN307044</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Finding</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Source: Orphanet</div>
|
||
<div class="spaceAbove">clinical entity without genetic inheritance.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/988794">This record</a></div></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#moi_141025" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Autosomal recessive inheritance</a><span> (Orphanet)</span></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#moi_141047" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Autosomal dominant inheritance</a><span> (Orphanet)</span></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#moi_988794" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Not genetically inherited</a><span> (Orphanet)</span></div></td></tr>
|
||
<tr><td colspan="2" class="small"> </td></tr><tr><td><a class="help" data-jig="ncbipopper" href="#target-gene-related">Related genes:<img src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a><div id="target-gene-related" class="display-none">
|
||
Gene(s) associated with related conditions. For conditions<br />
|
||
in a hierarchy, the parent condition will list the genes<br />
|
||
associated with the children conditions.</div></td>
|
||
<td><a target="_blank" href="/gene/79572">ATP13A3</a>, <a target="_blank" href="/gene/4093">SMAD9</a>, <a target="_blank" href="/gene/3777">KCNK3</a>, <a target="_blank" href="/gene/857">CAV1</a>, <a target="_blank" href="/gene/659">BMPR2</a></td></tr><tr><td colspan="2" class="small"> </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=422">ORPHA422</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">
|
||
<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>
|
||
<div class="portlet_content ln">A form of pulmonary arterial hypertension (PAH) characterized by elevated pulmonary arterial resistance leading to right heart failure; it is progressive and potentially fatal. The majority cases have an identifiable genetic cause, but a significant proportion are idiopathic. [from <a title="Orphanet Rare Disease Ontology (ORDO)" href="http://www.orphadata.org/cgi-bin/inc/ordo_orphanet.inc.php" class="defSource" target="_blank">ORDO</a>]</div>
|
||
</div>
|
||
|
||
<div class="portlet mgSection" id="ID_118">
|
||
<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>
|
||
<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="TLclosed"><span class="chiclet_list"><span class="chiclet Ccolor round" title="Clinical test"><a target="_blank" href="/gtr/tests/?term=C0152171[DISCUI]&test_type=Clinical" ref="ncbi_uid=57749">C</a></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 Vcolor" title="ClinVar"><a target="_blank" href="/clinvar?LinkName=medgen_clinvar&from_uid=57749" ref="ncbi_uid=57749">V</a></span></span><span class="TLline"><a href="/medgen/57749" ref="tree=GTR&ncbi_uid=57749&link_uid=57749" title="View MedGen record for 'Primary pulmonary hypertension'">Primary pulmonary hypertension</a></span><ul><li class="TLclosed"><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 Ocolor" title="OMIM"><a ref="ncbi_uid=468368" target="_blank" href="/omim/178600">O</a></span><span class="chiclet unavailable" title="GeneReviews">G</span><span class="chiclet Vcolor" title="ClinVar"><a target="_blank" href="/clinvar?LinkName=medgen_clinvar&from_uid=468368" ref="ncbi_uid=468368">V</a></span></span><span class="TLline"><a href="/medgen/468368" ref="tree=GTR&ncbi_uid=468368&link_uid=468368" title="View MedGen record for 'Idiopathic pulmonary arterial hypertension'">Idiopathic pulmonary arterial hypertension</a></span><ul><li class="matched_ds"><span class="chiclet_list"><span class="chiclet Ccolor round" title="Clinical test"><a target="_blank" href="/gtr/tests/?term=C5679820[DISCUI]&test_type=Clinical&redirect=true" ref="ncbi_uid=1825946">C</a></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 Vcolor" title="ClinVar"><a target="_blank" href="/clinvar?LinkName=medgen_clinvar&from_uid=1825946" ref="ncbi_uid=1825946">V</a></span></span><span class="TLline">Idiopathic and/or familial pulmonary arterial hypertension</span><ul><li class="TLclosed"><span class="TLline"><a href="/medgen/90953" ref="tree=GTR&ncbi_uid=90953&link_uid=90953" title="View MedGen record for 'Heritable pulmonary arterial hypertension'">Heritable pulmonary arterial hypertension</a></span></li><li class="TLclosed"><span class="chiclet_list"><span class="chiclet Ccolor round" title="Clinical test"><a target="_blank" href="/gtr/tests/?term=C5676877[DISCUI]&test_type=Clinical&redirect=true" ref="ncbi_uid=1802382">C</a></span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet Ocolor" title="OMIM"><a ref="ncbi_uid=1802382" target="_blank" href="/omim/265400">O</a></span><span class="chiclet unavailable" title="GeneReviews">G</span><span class="chiclet Vcolor" title="ClinVar"><a target="_blank" href="/clinvar?LinkName=medgen_clinvar&from_uid=1802382" ref="ncbi_uid=1802382">V</a></span></span><span class="TLline"><a href="/medgen/1802382" ref="tree=GTR&ncbi_uid=1802382&link_uid=1802382" title="View MedGen record for 'Pulmonary hypertension, primary, autosomal recessive'">Pulmonary hypertension, primary, autosomal recessive</a></span></li></ul></li></ul></li></ul></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><ul><li><span class="matched_ds">Idiopathic and/or familial pulmonary arterial hypertension</span><ul><li><span class="TLline"><a href="/medgen/468368" ref="tree=MeSH" title="MedGen record for Idiopathic pulmonary arterial hypertension">Idiopathic pulmonary arterial hypertension</a></span><ul><li><span class="matched_ds">Idiopathic and/or familial pulmonary arterial hypertension</span><ul><li><span class="TLline"><a href="/medgen/468368" ref="tree=MeSH" title="MedGen record for Idiopathic pulmonary arterial hypertension">Idiopathic pulmonary arterial hypertension</a></span><ul><li><span class="matched_ds">Idiopathic and/or familial pulmonary arterial hypertension</span><ul><li><span class="TLline"><a href="/medgen/468368" ref="tree=MeSH" title="MedGen record for Idiopathic pulmonary arterial hypertension">Idiopathic pulmonary arterial hypertension</a></span><ul><li><span class="matched_ds">Idiopathic and/or familial pulmonary arterial hypertension</span><ul><li><span class="TLline"><a href="/medgen/90953" ref="tree=MeSH" title="MedGen record for Heritable pulmonary arterial hypertension">Heritable pulmonary arterial hypertension</a></span><ul><li><span class="TLline"><a href="/medgen/1643124" ref="tree=MeSH" title="MedGen record for Pulmonary hypertension, primary, 1">Pulmonary hypertension, primary, 1</a></span><ul><li><span class="TLline"><a href="/medgen/942109" ref="tree=MeSH" title="MedGen record for Pulmonary hypertension, primary, fenfluramine or dexfenfluramine-associated">Pulmonary hypertension, primary, fenfluramine or dexfenfluramine-associated</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/854709" ref="tree=MeSH" title="MedGen record for Pulmonary hypertension, primary, 2">Pulmonary hypertension, primary, 2</a></span></li><li><span class="TLline"><a href="/medgen/815522" ref="tree=MeSH" title="MedGen record for Pulmonary hypertension, primary, 3">Pulmonary hypertension, primary, 3</a></span></li><li><span class="TLline"><a href="/medgen/815528" ref="tree=MeSH" title="MedGen record for Pulmonary hypertension, primary, 4">Pulmonary hypertension, primary, 4</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/468368" ref="tree=MeSH" title="MedGen record for Idiopathic pulmonary arterial hypertension">Idiopathic pulmonary arterial hypertension</a></span></li><li><span class="TLline"><a href="/medgen/1802382" ref="tree=MeSH" title="MedGen record for Pulmonary hypertension, primary, autosomal recessive">Pulmonary hypertension, primary, autosomal recessive</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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||
|
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<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/36517177">Progressive pulmonary fibrosis: an expert group consensus statement.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Rajan SK,
|
||
Cottin V,
|
||
Dhar R,
|
||
Danoff S,
|
||
Flaherty KR,
|
||
Brown KK,
|
||
Mohan A,
|
||
Renzoni E,
|
||
Mohan M,
|
||
Udwadia Z,
|
||
Shenoy P,
|
||
Currow D,
|
||
Devraj A,
|
||
Jankharia B,
|
||
Kulshrestha R,
|
||
Jones S,
|
||
Ravaglia C,
|
||
Quadrelli S,
|
||
Iyer R,
|
||
Dhooria S,
|
||
Kolb M,
|
||
Wells AU</span><br />
|
||
<span class="medgenPMjournal">Eur Respir J</span>
|
||
2023 Mar;61(3)
|
||
Epub 2023 Mar 30
|
||
doi: 10.1183/13993003.03187-2021.
|
||
<span class="bold">PMID: </span><a href="/pubmed/36517177" target="_blank">36517177</a><a href="/pmc/articles/PMC10060665" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/33620586">Evidence-based clinical practice guidelines for peptic ulcer disease 2020.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Kamada T,
|
||
Satoh K,
|
||
Itoh T,
|
||
Ito M,
|
||
Iwamoto J,
|
||
Okimoto T,
|
||
Kanno T,
|
||
Sugimoto M,
|
||
Chiba T,
|
||
Nomura S,
|
||
Mieda M,
|
||
Hiraishi H,
|
||
Yoshino J,
|
||
Takagi A,
|
||
Watanabe S,
|
||
Koike K</span><br />
|
||
<span class="medgenPMjournal">J Gastroenterol</span>
|
||
2021 Apr;56(4):303-322.
|
||
Epub 2021 Feb 23
|
||
doi: 10.1007/s00535-021-01769-0.
|
||
<span class="bold">PMID: </span><a href="/pubmed/33620586" target="_blank">33620586</a><a href="/pmc/articles/PMC8005399" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/29610508">ACG Clinical Guideline: Management of Crohn's Disease in Adults.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Lichtenstein GR,
|
||
Loftus EV,
|
||
Isaacs KL,
|
||
Regueiro MD,
|
||
Gerson LB,
|
||
Sands BE</span><br />
|
||
<span class="medgenPMjournal">Am J Gastroenterol</span>
|
||
2018 Apr;113(4):481-517.
|
||
Epub 2018 Mar 27
|
||
doi: 10.1038/ajg.2018.27.
|
||
<span class="bold">PMID: </span><a href="/pubmed/29610508" target="_blank">29610508</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(idiopathic%20and%2For%20familial%20pulmonary%20arterial%20hypertension)%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 (8227)</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/38232988">Pulmonary veno-occlusive disease: illustrative cases and literature review.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Lechartier B,
|
||
Boucly A,
|
||
Solinas S,
|
||
Gopalan D,
|
||
Dorfmüller P,
|
||
Radonic T,
|
||
Sitbon O,
|
||
Montani D</span><br />
|
||
<span class="medgenPMjournal">Eur Respir Rev</span>
|
||
2024 Jan 31;33(171)
|
||
Epub 2024 Jan 17
|
||
doi: 10.1183/16000617.0156-2023.
|
||
<span class="bold">PMID: </span><a href="/pubmed/38232988" target="_blank">38232988</a><a href="/pmc/articles/PMC10792437" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/37591298">Treatment of pulmonary arterial hypertension: recent progress and a look to the future.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Humbert M,
|
||
Sitbon O,
|
||
Guignabert C,
|
||
Savale L,
|
||
Boucly A,
|
||
Gallant-Dewavrin M,
|
||
McLaughlin V,
|
||
Hoeper MM,
|
||
Weatherald J</span><br />
|
||
<span class="medgenPMjournal">Lancet Respir Med</span>
|
||
2023 Sep;11(9):804-819.
|
||
Epub 2023 Aug 14
|
||
doi: 10.1016/S2213-2600(23)00264-3.
|
||
<span class="bold">PMID: </span><a href="/pubmed/37591298" target="_blank">37591298</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/36649754">Portopulmonary Hypertension: Management and Liver Transplantation Evaluation.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">DuBrock HM</span><br />
|
||
<span class="medgenPMjournal">Chest</span>
|
||
2023 Jul;164(1):206-214.
|
||
Epub 2023 Jan 14
|
||
doi: 10.1016/j.chest.2023.01.009.
|
||
<span class="bold">PMID: </span><a href="/pubmed/36649754" target="_blank">36649754</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/36041750">Sotatercept for the treatment of pulmonary arterial hypertension: PULSAR open-label extension.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Humbert M,
|
||
McLaughlin V,
|
||
Gibbs JSR,
|
||
Gomberg-Maitland M,
|
||
Hoeper MM,
|
||
Preston IR,
|
||
Souza R,
|
||
Waxman AB,
|
||
Ghofrani HA,
|
||
Escribano Subias P,
|
||
Feldman J,
|
||
Meyer G,
|
||
Montani D,
|
||
Olsson KM,
|
||
Manimaran S,
|
||
de Oliveira Pena J,
|
||
Badesch DB</span><br />
|
||
<span class="medgenPMjournal">Eur Respir J</span>
|
||
2023 Jan;61(1)
|
||
Epub 2023 Jan 6
|
||
doi: 10.1183/13993003.01347-2022.
|
||
<span class="bold">PMID: </span><a href="/pubmed/36041750" target="_blank">36041750</a><a href="/pmc/articles/PMC9816418" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/24134901">Targeted therapies in pulmonary arterial hypertension.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Montani D,
|
||
Chaumais MC,
|
||
Guignabert C,
|
||
Günther S,
|
||
Girerd B,
|
||
Jaïs X,
|
||
Algalarrondo V,
|
||
Price LC,
|
||
Savale L,
|
||
Sitbon O,
|
||
Simonneau G,
|
||
Humbert M</span><br />
|
||
<span class="medgenPMjournal">Pharmacol Ther</span>
|
||
2014 Feb;141(2):172-91.
|
||
Epub 2013 Oct 14
|
||
doi: 10.1016/j.pharmthera.2013.10.002.
|
||
<span class="bold">PMID: </span><a href="/pubmed/24134901" target="_blank">24134901</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Idiopathic%20and%2For%20familial%20pulmonary%20arterial%20hypertension%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 (53397)</a></div><h3 class="subhead">Diagnosis</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/38461018">Managing Pulmonary Arterial Hypertension With Cardiopulmonary Comorbidities.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Provencher S,
|
||
Mai V,
|
||
Bonnet S</span><br />
|
||
<span class="medgenPMjournal">Chest</span>
|
||
2024 Mar;165(3):682-691.
|
||
doi: 10.1016/j.chest.2023.08.023.
|
||
<span class="bold">PMID: </span><a href="/pubmed/38461018" target="_blank">38461018</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/37450768">Pulmonary Hypertension: A Contemporary Review.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Johnson S,
|
||
Sommer N,
|
||
Cox-Flaherty K,
|
||
Weissmann N,
|
||
Ventetuolo CE,
|
||
Maron BA</span><br />
|
||
<span class="medgenPMjournal">Am J Respir Crit Care Med</span>
|
||
2023 Sep 1;208(5):528-548.
|
||
doi: 10.1164/rccm.202302-0327SO.
|
||
<span class="bold">PMID: </span><a href="/pubmed/37450768" target="_blank">37450768</a><a href="/pmc/articles/PMC10492255" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/36982160">Systemic Lupus Erythematosus and Pulmonary Hypertension.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Parperis K,
|
||
Velidakis N,
|
||
Khattab E,
|
||
Gkougkoudi E,
|
||
Kadoglou NPE</span><br />
|
||
<span class="medgenPMjournal">Int J Mol Sci</span>
|
||
2023 Mar 7;24(6)
|
||
doi: 10.3390/ijms24065085.
|
||
<span class="bold">PMID: </span><a href="/pubmed/36982160" target="_blank">36982160</a><a href="/pmc/articles/PMC10049584" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/36649754">Portopulmonary Hypertension: Management and Liver Transplantation Evaluation.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">DuBrock HM</span><br />
|
||
<span class="medgenPMjournal">Chest</span>
|
||
2023 Jul;164(1):206-214.
|
||
Epub 2023 Jan 14
|
||
doi: 10.1016/j.chest.2023.01.009.
|
||
<span class="bold">PMID: </span><a href="/pubmed/36649754" target="_blank">36649754</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/34737219">The physiological basis of pulmonary arterial hypertension.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Naeije R,
|
||
Richter MJ,
|
||
Rubin LJ</span><br />
|
||
<span class="medgenPMjournal">Eur Respir J</span>
|
||
2022 Jun;59(6)
|
||
Epub 2022 Jun 16
|
||
doi: 10.1183/13993003.02334-2021.
|
||
<span class="bold">PMID: </span><a href="/pubmed/34737219" target="_blank">34737219</a><a href="/pmc/articles/PMC9203839" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Idiopathic%20and%2For%20familial%20pulmonary%20arterial%20hypertension%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 (49909)</a></div><h3 class="subhead">Therapy</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/39209475">Definition, classification and diagnosis of pulmonary hypertension.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Kovacs G,
|
||
Bartolome S,
|
||
Denton CP,
|
||
Gatzoulis MA,
|
||
Gu S,
|
||
Khanna D,
|
||
Badesch D,
|
||
Montani D</span><br />
|
||
<span class="medgenPMjournal">Eur Respir J</span>
|
||
2024 Oct;64(4)
|
||
Epub 2024 Oct 31
|
||
doi: 10.1183/13993003.01324-2024.
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<div class="nl"><a target="_blank" href="/pubmed/37591298">Treatment of pulmonary arterial hypertension: recent progress and a look to the future.</a></div>
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<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Idiopathic%20and%2For%20familial%20pulmonary%20arterial%20hypertension%22%20AND%20Prognosis%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 (30290)</a></div><h3 class="subhead">Clinical prediction guides</h3>
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<div class="nl"><a target="_blank" href="/pubmed/37945977">A Systematic Review of Novel Therapies of Pulmonary Arterial Hypertension.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Nabeh OA,
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Saud AI,
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Amin B,
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Khedr AS,
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Amr A,
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Faoosa AM,
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Esmat E,
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Mahmoud YM,
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Hatem A,
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Osama A,
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Soliman YMA,
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Elkorashy RI,
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Epub 2023 Nov 9
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<span class="bold">PMID: </span><a href="/pubmed/37945977" target="_blank">37945977</a><a href="/pmc/articles/PMC10805839" target="_blank" class="PubMedFree">Free PMC Article</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/37989727">Reduced FOXF1 links unrepaired DNA damage to pulmonary arterial hypertension.</a></div>
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<span class="bold">PMID: </span><a href="/pubmed/35551212" target="_blank">35551212</a><a href="/pmc/articles/PMC9098455" target="_blank" class="PubMedFree">Free PMC Article</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/33082079">Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Hoeper MM,
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Pausch C,
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Grünig E,
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Klose H,
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Staehler G,
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Huscher D,
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Pittrow D,
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Olsson KM,
|
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Vizza CD,
|
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Gall H,
|
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Benjamin N,
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Distler O,
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Opitz C,
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Gibbs JSR,
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Delcroix M,
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Ghofrani HA,
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Rosenkranz S,
|
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Ewert R,
|
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Kaemmerer H,
|
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Lange TJ,
|
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Kabitz HJ,
|
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Skowasch D,
|
||
Skride A,
|
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Jureviciene E,
|
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Paleviciute E,
|
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Miliauskas S,
|
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Claussen M,
|
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Behr J,
|
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Milger K,
|
||
Halank M,
|
||
Wilkens H,
|
||
Wirtz H,
|
||
Pfeuffer-Jovic E,
|
||
Harbaum L,
|
||
Scholtz W,
|
||
Dumitrescu D,
|
||
Bruch L,
|
||
Coghlan G,
|
||
Neurohr C,
|
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Tsangaris I,
|
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Gorenflo M,
|
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Scelsi L,
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Vonk-Noordegraaf A,
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Ulrich S,
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Held M</span><br />
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<span class="medgenPMjournal">J Heart Lung Transplant</span>
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2020 Dec;39(12):1435-1444.
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Epub 2020 Sep 30
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doi: 10.1016/j.healun.2020.09.011.
|
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<span class="bold">PMID: </span><a href="/pubmed/33082079" target="_blank">33082079</a></div>
|
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<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Idiopathic%20and%2For%20familial%20pulmonary%20arterial%20hypertension%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 (33176)</a></div></div>
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</div>
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||
<div class="portlet mgSection" id="ID_104">
|
||
<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>
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<div class="portlet_content ln">
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<div class="nl"><a target="_blank" href="/pubmed/36554613">Application of the Schroth Method in the Treatment of Idiopathic Scoliosis: A Systematic Review and Meta-Analysis.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Dimitrijević V,
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Šćepanović T,
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Jevtić N,
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Rašković B,
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Milankov V,
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Milosević Z,
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Ninković SS,
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Chockalingam N,
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Obradović B,
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Drid P</span><br />
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<span class="medgenPMjournal">Int J Environ Res Public Health</span>
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2022 Dec 13;19(24)
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<span class="bold">PMID: </span><a href="/pubmed/36554613" target="_blank">36554613</a><a href="/pmc/articles/PMC9779560" target="_blank" class="PubMedFree">Free PMC Article</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/34962437">Corrective exercise-based therapy for adolescent idiopathic scoliosis: Systematic review and meta-analysis.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Gámiz-Bermúdez F,
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Obrero-Gaitán E,
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Zagalaz-Anula N,
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Lomas-Vega R</span><br />
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<span class="medgenPMjournal">Clin Rehabil</span>
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2022 May;36(5):597-608.
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Epub 2021 Dec 28
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<span class="bold">PMID: </span><a href="/pubmed/34962437" target="_blank">34962437</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/32740208">Treatments of Periorbital Hyperpigmentation: A Systematic Review.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Michelle L,
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Pouldar Foulad D,
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Ekelem C,
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Saedi N,
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Mesinkovska NA</span><br />
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<span class="medgenPMjournal">Dermatol Surg</span>
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<span class="bold">PMID: </span><a href="/pubmed/32740208" target="_blank">32740208</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/30016036">Effects of corrective, therapeutic exercise techniques on adolescent idiopathic scoliosis. A systematic review.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Ceballos Laita L,
|
||
Tejedor Cubillo C,
|
||
Mingo Gómez T,
|
||
Jiménez Del Barrio S</span><br />
|
||
<span class="medgenPMjournal">Arch Argent Pediatr</span>
|
||
2018 Aug 1;116(4):e582-e589.
|
||
doi: 10.5546/aap.2018.eng.e582.
|
||
<span class="bold">PMID: </span><a href="/pubmed/30016036" target="_blank">30016036</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/25414441">Drug-induced thrombotic microangiopathy: a systematic review of published reports.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Al-Nouri ZL,
|
||
Reese JA,
|
||
Terrell DR,
|
||
Vesely SK,
|
||
George JN</span><br />
|
||
<span class="medgenPMjournal">Blood</span>
|
||
2015 Jan 22;125(4):616-8.
|
||
Epub 2014 Nov 20
|
||
doi: 10.1182/blood-2014-11-611335.
|
||
<span class="bold">PMID: </span><a href="/pubmed/25414441" target="_blank">25414441</a><a href="/pmc/articles/PMC4304106" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Idiopathic%20and%2For%20familial%20pulmonary%20arterial%20hypertension%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 (1949)</a></div></div>
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<div class="portlet_content ln"><ul><li><a href="/gtr/tests?term=C5679820%5bDISCUI%5d&filter=method%3A2%5F8" target="_blank">Deletion/duplication analysis (1)</a></li>
|
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<li><a href="/gtr/tests?term=C5679820%5bDISCUI%5d&filter=method%3A2%5F7" target="_blank">Sequence analysis of the entire coding region (1)</a></li>
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