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<meta name="keywords" content="C5935600, capns1, disease or syndrome, pph6, pulmonary hypertension, primary, 6, autosomal dominant, autosomal recessive, birth defects, chromosomal disease, chromosome, clinical features, clinical findings, clinical genetics, clinical recommendations, clinvar, congenital chromosomal disease, consumer genetic resources, cytogenetic location, disease characteristics, disease definitions, disease descriptions, disease ontology, disease synonyms, disease vocabulary, dysmorphology, entrez, familial disease, gene, gene-disease relationship, genereviews, genetic disease, genetic disorder, genetic terminology, genetic testing registry, genetics home reference, genomic disease, gtr, hereditary disease, heritable disease, hpo, human phenotype ontology, inherited disease, management guidelines, maternal inheritance, medgen, medical genetics, medical subject headings, mesh, mitochondrial inheritance, mode of inheritance, national center for biotechnology information, national institutes of health, national library of medicine, ncbi, nih, nlm, omim, ordo, orphanet, paternal inheritance, phenome, position statements, professional practice guidelines, rare disease, reference sequence, refseq, snomed ct, syndrome, undiagnosed diseases, x-linked recessive" /><meta name="description" content="Primary pulmonary hypertension-6 (PPH6) is characterized by markedly elevated pulmonary arterial hypertension, associated with reduced oxygen saturation and diffuse ground-glass opacities on chest x-ray. Lung biopsy shows thickening of the alveolar septae and abnormally proliferating capillaries (Postma et al., 2023). For a general phenotypic description and a discussion of genetic heterogeneity of primary pulmonary hypertension, see PPH1 (178600)." /><meta name="robots" content="index,nofollow,noarchive" />
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<!--
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UID=1863339
|
||
ConceptID=C5935600
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-->
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<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Pulmonary hypertension, primary, 6<span class="h1sub">(PPH6)</span></div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1863339</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:">C5935600</span></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>PPH6</td></tr>
|
||
<tr><td colspan="2" class="small"> </td></tr><tr><td><a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#target-gene-loc">Gene (location):<img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a><div class="display-none" id="target-gene-loc">
|
||
Gene(s) directly associated with<br />
|
||
this condition or phenotype.</div></td>
|
||
<td><a target="_blank" title="CAPNS1 - ID: 826 - NCBI Gene" href="/gene/826" class="medgenPMinfo">CAPNS1</a> (19q13.12)</td></tr>
|
||
<tr><td colspan="2" class="small"> </td></tr><tr><td>Monarch Initiative:</td>
|
||
<td><a href="https://monarchinitiative.org/disease/MONDO:0958334" target="_blank">MONDO:0958334</a></td></tr>
|
||
<tr><td>OMIM<span class="superscript">®</span>:</td>
|
||
<td><a href="https://omim.org/entry/620777" target="_blank">620777</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>
|
||
<div class="portlet_content ln">Primary pulmonary hypertension-6 (PPH6) is characterized by markedly elevated pulmonary arterial hypertension, associated with reduced oxygen saturation and diffuse ground-glass opacities on chest x-ray. Lung biopsy shows thickening of the alveolar septae and abnormally proliferating capillaries (Postma et al., 2023). For a general phenotypic description and a discussion of genetic heterogeneity of primary pulmonary hypertension, see PPH1 (178600). [from <a title="Online Mendelian Inheritance in Man" href="http://www.omim.org" class="defSource" target="_blank">OMIM</a>]</div>
|
||
</div>
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||
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<div class="portlet mgSection" id="ID_102">
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Clinical_features">Clinical features</h1><a sid="102" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln clinfeat"><strong>From HPO</strong><br />
|
||
<div class="divPopper rprt" id="clin_87404"><div><strong>Pulmonary capillary hemangiomatosis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>87404</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.">C0340548</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/87404">Feature record</a> | <a href="/medgen?term=%22Pulmonary%20capillary%20hemangiomatosis%22%5BClinical%20Features%5D%20OR%2087404%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_13955"><div><strong>Atrial flutter</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>13955</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.">C0004239</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Pathologic Function</dd></dl></div></div></div>
|
||
<div class="spaceAbove">A type of atrial arrhythmia characterized by atrial rates of between 240 and 400 beats per minute and some degree of atrioventricular node conduction block. Typically, the ventricular rate is half the atrial rate. In the EKG; atrial flutter waves are observed as sawtooth-like atrial activity. Pathophysiologically, atrial flutter is a form of atrial reentry in which there is a premature electrical impulse creates a self-propagating circuit.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/13955">Feature record</a> | <a href="/medgen?term=%22Atrial%20flutter%22%5BClinical%20Features%5D%20OR%2013955%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_13956"><div><strong>Atrioventricular block</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>13956</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.">C0004245</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Delayed or lack of conduction of atrial depolarizations through the atrioventricular node to the ventricles.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/13956">Feature record</a> | <a href="/medgen?term=%22Atrioventricular%20block%22%5BClinical%20Features%5D%20OR%2013956%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_2881"><div><strong>Hypertrophic cardiomyopathy</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>2881</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.">C0007194</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Hypertrophic cardiomyopathy (HCM) is defined by the presence of increased ventricular wall thickness or mass in the absence of loading conditions (hypertension, valve disease) sufficient to cause the observed abnormality.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/2881">Feature record</a> | <a href="/medgen?term=%22Hypertrophic%20cardiomyopathy%22%5BClinical%20Features%5D%20OR%202881%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_57981"><div><strong>Right ventricular hypertrophy</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>57981</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.">C0162770</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">In this case the right ventricle is more muscular than normal, causing a characteristic boot-shaped (coeur-en-sabot) appearance as seen on anterior- posterior chest x-rays. Right ventricular hypertrophy is commonly associated with any form of right ventricular outflow obstruction or pulmonary hypertension, which may in turn owe its origin to left-sided disease. The echocardiographic signs are thickening of the anterior right ventricular wall and the septum. Cavity size is usually normal, or slightly enlarged. In many cases there is associated volume overload present due to tricuspid regurgitation, in the absence of this, septal motion is normal.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/57981">Feature record</a> | <a href="/medgen?term=%22Right%20ventricular%20hypertrophy%22%5BClinical%20Features%5D%20OR%2057981%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_65907"><div><strong>Right ventricular failure</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>65907</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.">C0235527</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Reduced ability of the right ventricle to perform its function (to receive blood from the right atrium and to eject blood into the pulmonary artery), often leading to pitting peripheral edema, ascites, and hepatomegaly.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/65907">Feature record</a> | <a href="/medgen?term=%22Right%20ventricular%20failure%22%5BClinical%20Features%5D%20OR%2065907%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_425404"><div><strong>Pulmonary arterial hypertension</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>425404</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.">C2973725</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Pulmonary hypertension is defined mean pulmonary artery pressure of 25mmHg or more and pulmonary capillary wedge pressure of 15mmHg or less when measured by right heart catheterisation at rest and in a supine position.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/425404">Feature record</a> | <a href="/medgen?term=%22Pulmonary%20arterial%20hypertension%22%5BClinical%20Features%5D%20OR%20425404%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_746019"><div><strong>Failure to thrive</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>746019</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.">C2315100</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Failure to thrive (FTT) refers to a child whose physical growth is substantially below the norm.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/746019">Feature record</a> | <a href="/medgen?term=%22Failure%20to%20thrive%22%5BClinical%20Features%5D%20OR%20746019%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_2009"><div><strong>Apnea</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>2009</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.">C0003578</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Sign or Symptom</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Lack of breathing with no movement of the respiratory muscles and no exchange of air in the lungs. This term refers to a disposition to have recurrent episodes of apnea rather than to a single event.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/2009">Feature record</a> | <a href="/medgen?term=%22Apnea%22%5BClinical%20Features%5D%20OR%202009%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_3938"><div><strong>Dyspnea</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>3938</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.">C0013404</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Sign or Symptom</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Difficult or labored breathing. Dyspnea is a subjective feeling only the patient can rate, e.g., on a Borg scale.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/3938">Feature record</a> | <a href="/medgen?term=%22Dyspnea%22%5BClinical%20Features%5D%20OR%203938%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_11197"><div><strong>Respiratory insufficiency</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>11197</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.">C0035229</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Pathologic Function</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Impairment of gas exchange within the lungs secondary to a disease process, neoplasm, or trauma, possibly resulting in hypoxia, hypercarbia, or both, but not requiring intubation or mechanical ventilation. Patients are normally managed with pharmaceutical therapy, supplemental oxygen, or both.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/11197">Feature record</a> | <a href="/medgen?term=%22Respiratory%20insufficiency%22%5BClinical%20Features%5D%20OR%2011197%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_1717924"><div><strong>Hemosiderin-laden macrophages in bronchoalveolar fluid</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1717924</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.">C5397971</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Finding</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Hemosiderin-laden macrophages (HLM) in bronchoalveolar lavage (BAL) fluid were originally known as adiagnostic biomarker of alveolar hemorrhage, but have also been observed in idiopathic pulmonary fibrosis (IPF) with histopathological pattern of usual interstitial pneumonia (UIP).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/1717924">Feature record</a> | <a href="/medgen?term=%22Hemosiderin-laden%20macrophages%20in%20bronchoalveolar%20fluid%22%5BClinical%20Features%5D%20OR%201717924%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_1779663"><div><strong>Ground-glass opacification</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1779663</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.">C5539411</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Finding</dd></dl></div></div></div>
|
||
<div class="spaceAbove">On chest radiographs, ground-glass opacity appears as an area of hazy increased lung opacity, usually extensive, within which margins of pulmonary vessels may be indistinct. On CT scans, it appears as hazy increased opacity of lung, with preservation of bronchial and vascular margins. It is caused by partial filling of airspaces, interstitial thickening (due to fluid, cells, and/or fibrosis), partial collapse of alveoli, increased capillary blood volume, or a combination of these, the common factor being the partial displacement of air. Ground-glass opacity is less opaque than consolidation, in which bronchovascular margins are obscured.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/1779663">Feature record</a> | <a href="/medgen?term=%22Ground-glass%20opacification%22%5BClinical%20Features%5D%20OR%201779663%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_1779532"><div><strong>Alveolar septal thickening</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1779532</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.">C5539545</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Finding</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Increased width of the alveolar septum, which is the structure that separates neighboring alveoli. This finding can be appreciated on histology.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/1779532">Feature record</a> | <a href="/medgen?term=%22Alveolar%20septal%20thickening%22%5BClinical%20Features%5D%20OR%201779532%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_1385064"><div><strong>Increased circulating NT-proBNP concentration</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1385064</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.">C4477024</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Finding</dd></dl></div></div></div>
|
||
<div class="spaceAbove">An elevated level of circulating N-terminal part of the prohormone of B-type natriuretic peptide (BNP).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/1385064">Feature record</a> | <a href="/medgen?term=%22Increased%20circulating%20NT-proBNP%20concentration%22%5BClinical%20Features%5D%20OR%201385064%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_1189"><div><strong>Cyanosis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1189</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.">C0010520</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Sign or Symptom</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Bluish discoloration of the skin and mucosa due to poor circulation or inadequate oxygenation of arterial or capillary blood.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/1189">Feature record</a> | <a href="/medgen?term=%22Cyanosis%22%5BClinical%20Features%5D%20OR%201189%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_57721"><div><strong>Premature birth</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>57721</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.">C0151526</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Pathologic Function</dd></dl></div></div></div>
|
||
<div class="spaceAbove">The birth of a baby of less than 37 weeks of gestational age.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/57721">Feature record</a> | <a href="/medgen?term=%22Premature%20birth%22%5BClinical%20Features%5D%20OR%2057721%5Buid%5D">Search on this feature</a></div></div><div class="TreeLite" data-jigconfig="closed: 1"><div class="concept-def"><a class="small" href="#" onclick="jQuery(".TreeLite","#ID_102").TreeLite().openAll(); return false;">Show all</a><a class="small" href="#" onclick="jQuery(".TreeLite","#ID_102").TreeLite().closeAll(); return false;">Hide all</a></div><ul><li><span class="TLline">Abnormality of metabolism/homeostasis</span><ul><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_1385064" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Increased circulating NT-proBNP concentration</a></span></li></ul></li><li><span class="TLline">Abnormality of prenatal development or birth</span><ul><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_57721" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Premature birth</a></span></li></ul></li><li><span class="TLline">Abnormality of the cardiovascular system</span><ul><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_13955" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Atrial flutter</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_13956" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Atrioventricular block</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_2881" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Hypertrophic cardiomyopathy</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_425404" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Pulmonary arterial hypertension</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_65907" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Right ventricular failure</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_57981" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Right ventricular hypertrophy</a></span></li></ul></li><li><span class="TLline">Abnormality of the integument</span><ul><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_1189" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Cyanosis</a></span></li></ul></li><li><span class="TLline">Abnormality of the respiratory system</span><ul><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_1779532" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Alveolar septal thickening</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_2009" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Apnea</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_3938" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Dyspnea</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_1779663" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Ground-glass opacification</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_1717924" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Hemosiderin-laden macrophages in bronchoalveolar fluid</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_11197" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Respiratory insufficiency</a></span></li></ul></li><li><span class="TLline">Growth abnormality</span><ul><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_746019" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Failure to thrive</a></span></li></ul></li><li><span class="TLline">Neoplasm</span><ul><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_87404" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Pulmonary capillary hemangiomatosis</a></span></li></ul></li></ul></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/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/34953525">Abiraterone acetate and prednisolone with or without enzalutamide for high-risk non-metastatic prostate cancer: a meta-analysis of primary results from two randomised controlled phase 3 trials of the STAMPEDE platform protocol.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Attard G,
|
||
Murphy L,
|
||
Clarke NW,
|
||
Cross W,
|
||
Jones RJ,
|
||
Parker CC,
|
||
Gillessen S,
|
||
Cook A,
|
||
Brawley C,
|
||
Amos CL,
|
||
Atako N,
|
||
Pugh C,
|
||
Buckner M,
|
||
Chowdhury S,
|
||
Malik Z,
|
||
Russell JM,
|
||
Gilson C,
|
||
Rush H,
|
||
Bowen J,
|
||
Lydon A,
|
||
Pedley I,
|
||
O'Sullivan JM,
|
||
Birtle A,
|
||
Gale J,
|
||
Srihari N,
|
||
Thomas C,
|
||
Tanguay J,
|
||
Wagstaff J,
|
||
Das P,
|
||
Gray E,
|
||
Alzoueb M,
|
||
Parikh O,
|
||
Robinson A,
|
||
Syndikus I,
|
||
Wylie J,
|
||
Zarkar A,
|
||
Thalmann G,
|
||
de Bono JS,
|
||
Dearnaley DP,
|
||
Mason MD,
|
||
Gilbert D,
|
||
Langley RE,
|
||
Millman R,
|
||
Matheson D,
|
||
Sydes MR,
|
||
Brown LC,
|
||
Parmar MKB,
|
||
James ND;
|
||
Systemic Therapy in Advancing or Metastatic Prostate cancer: Evaluation of Drug Efficacy (STAMPEDE) investigators</span><br />
|
||
<span class="medgenPMjournal">Lancet</span>
|
||
2022 Jan 29;399(10323):447-460.
|
||
Epub 2021 Dec 23
|
||
doi: 10.1016/S0140-6736(21)02437-5.
|
||
<span class="bold">PMID: </span><a href="/pubmed/34953525" target="_blank">34953525</a><a href="/pmc/articles/PMC8811484" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/34737226">COMPERA 2.0: a refined four-stratum risk assessment model for pulmonary arterial hypertension.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Hoeper MM,
|
||
Pausch C,
|
||
Olsson KM,
|
||
Huscher D,
|
||
Pittrow D,
|
||
Grünig E,
|
||
Staehler G,
|
||
Vizza CD,
|
||
Gall H,
|
||
Distler O,
|
||
Opitz C,
|
||
Gibbs JSR,
|
||
Delcroix M,
|
||
Ghofrani HA,
|
||
Park DH,
|
||
Ewert R,
|
||
Kaemmerer H,
|
||
Kabitz HJ,
|
||
Skowasch D,
|
||
Behr J,
|
||
Milger K,
|
||
Halank M,
|
||
Wilkens H,
|
||
Seyfarth HJ,
|
||
Held M,
|
||
Dumitrescu D,
|
||
Tsangaris I,
|
||
Vonk-Noordegraaf A,
|
||
Ulrich S,
|
||
Klose H,
|
||
Claussen M,
|
||
Lange TJ,
|
||
Rosenkranz S</span><br />
|
||
<span class="medgenPMjournal">Eur Respir J</span>
|
||
2022 Jul;60(1)
|
||
Epub 2022 Jul 7
|
||
doi: 10.1183/13993003.02311-2021.
|
||
<span class="bold">PMID: </span><a href="/pubmed/34737226" target="_blank">34737226</a><a href="/pmc/articles/PMC9260123" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(pulmonary%20hypertension%2C%20primary%2C%206)%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 (279)</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/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/35777416">Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Hoeper MM,
|
||
Dwivedi K,
|
||
Pausch C,
|
||
Lewis RA,
|
||
Olsson KM,
|
||
Huscher D,
|
||
Pittrow D,
|
||
Grünig E,
|
||
Staehler G,
|
||
Vizza CD,
|
||
Gall H,
|
||
Distler O,
|
||
Opitz C,
|
||
Gibbs JSR,
|
||
Delcroix M,
|
||
Park DH,
|
||
Ghofrani HA,
|
||
Ewert R,
|
||
Kaemmerer H,
|
||
Kabitz HJ,
|
||
Skowasch D,
|
||
Behr J,
|
||
Milger K,
|
||
Lange TJ,
|
||
Wilkens H,
|
||
Seyfarth HJ,
|
||
Held M,
|
||
Dumitrescu D,
|
||
Tsangaris I,
|
||
Vonk-Noordegraaf A,
|
||
Ulrich S,
|
||
Klose H,
|
||
Claussen M,
|
||
Eisenmann S,
|
||
Schmidt KH,
|
||
Swift AJ,
|
||
Thompson AAR,
|
||
Elliot CA,
|
||
Rosenkranz S,
|
||
Condliffe R,
|
||
Kiely DG,
|
||
Halank M</span><br />
|
||
<span class="medgenPMjournal">Lancet Respir Med</span>
|
||
2022 Oct;10(10):937-948.
|
||
Epub 2022 Jun 28
|
||
doi: 10.1016/S2213-2600(22)00097-2.
|
||
<span class="bold">PMID: </span><a href="/pubmed/35777416" target="_blank">35777416</a><a href="/pmc/articles/PMC9514996" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/34588193">Mendelian randomisation and experimental medicine approaches to interleukin-6 as a drug target in pulmonary arterial hypertension.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Toshner M,
|
||
Church C,
|
||
Harbaum L,
|
||
Rhodes C,
|
||
Villar Moreschi SS,
|
||
Liley J,
|
||
Jones R,
|
||
Arora A,
|
||
Batai K,
|
||
Desai AA,
|
||
Coghlan JG,
|
||
Gibbs JSR,
|
||
Gor D,
|
||
Gräf S,
|
||
Harlow L,
|
||
Hernandez-Sanchez J,
|
||
Howard LS,
|
||
Humbert M,
|
||
Karnes J,
|
||
Kiely DG,
|
||
Kittles R,
|
||
Knightbridge E,
|
||
Lam B,
|
||
Lutz KA,
|
||
Nichols WC,
|
||
Pauciulo MW,
|
||
Pepke-Zaba J,
|
||
Suntharalingam J,
|
||
Soubrier F,
|
||
Trembath RC,
|
||
Schwantes-An TL,
|
||
Wort SJ,
|
||
Wilkins MR,
|
||
Gaine S,
|
||
Morrell NW,
|
||
Corris PA;
|
||
Uniphy Clinical Trials Network</span><br />
|
||
<span class="medgenPMjournal">Eur Respir J</span>
|
||
2022 Mar;59(3)
|
||
Epub 2022 Mar 10
|
||
doi: 10.1183/13993003.02463-2020.
|
||
<span class="bold">PMID: </span><a href="/pubmed/34588193" target="_blank">34588193</a><a href="/pmc/articles/PMC8907935" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/33082079">Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Hoeper MM,
|
||
Pausch C,
|
||
Grünig E,
|
||
Klose H,
|
||
Staehler G,
|
||
Huscher D,
|
||
Pittrow D,
|
||
Olsson KM,
|
||
Vizza CD,
|
||
Gall H,
|
||
Benjamin N,
|
||
Distler O,
|
||
Opitz C,
|
||
Gibbs JSR,
|
||
Delcroix M,
|
||
Ghofrani HA,
|
||
Rosenkranz S,
|
||
Ewert R,
|
||
Kaemmerer H,
|
||
Lange TJ,
|
||
Kabitz HJ,
|
||
Skowasch D,
|
||
Skride A,
|
||
Jureviciene E,
|
||
Paleviciute E,
|
||
Miliauskas S,
|
||
Claussen M,
|
||
Behr J,
|
||
Milger K,
|
||
Halank M,
|
||
Wilkens H,
|
||
Wirtz H,
|
||
Pfeuffer-Jovic E,
|
||
Harbaum L,
|
||
Scholtz W,
|
||
Dumitrescu D,
|
||
Bruch L,
|
||
Coghlan G,
|
||
Neurohr C,
|
||
Tsangaris I,
|
||
Gorenflo M,
|
||
Scelsi L,
|
||
Vonk-Noordegraaf A,
|
||
Ulrich S,
|
||
Held M</span><br />
|
||
<span class="medgenPMjournal">J Heart Lung Transplant</span>
|
||
2020 Dec;39(12):1435-1444.
|
||
Epub 2020 Sep 30
|
||
doi: 10.1016/j.healun.2020.09.011.
|
||
<span class="bold">PMID: </span><a href="/pubmed/33082079" target="_blank">33082079</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pulmonary%20hypertension%2C%20primary%2C%206%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 (2352)</a></div><h3 class="subhead">Diagnosis</h3>
|
||
<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 class="nl"><a target="_blank" href="/pubmed/33082079">Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Hoeper MM,
|
||
Pausch C,
|
||
Grünig E,
|
||
Klose H,
|
||
Staehler G,
|
||
Huscher D,
|
||
Pittrow D,
|
||
Olsson KM,
|
||
Vizza CD,
|
||
Gall H,
|
||
Benjamin N,
|
||
Distler O,
|
||
Opitz C,
|
||
Gibbs JSR,
|
||
Delcroix M,
|
||
Ghofrani HA,
|
||
Rosenkranz S,
|
||
Ewert R,
|
||
Kaemmerer H,
|
||
Lange TJ,
|
||
Kabitz HJ,
|
||
Skowasch D,
|
||
Skride A,
|
||
Jureviciene E,
|
||
Paleviciute E,
|
||
Miliauskas S,
|
||
Claussen M,
|
||
Behr J,
|
||
Milger K,
|
||
Halank M,
|
||
Wilkens H,
|
||
Wirtz H,
|
||
Pfeuffer-Jovic E,
|
||
Harbaum L,
|
||
Scholtz W,
|
||
Dumitrescu D,
|
||
Bruch L,
|
||
Coghlan G,
|
||
Neurohr C,
|
||
Tsangaris I,
|
||
Gorenflo M,
|
||
Scelsi L,
|
||
Vonk-Noordegraaf A,
|
||
Ulrich S,
|
||
Held M</span><br />
|
||
<span class="medgenPMjournal">J Heart Lung Transplant</span>
|
||
2020 Dec;39(12):1435-1444.
|
||
Epub 2020 Sep 30
|
||
doi: 10.1016/j.healun.2020.09.011.
|
||
<span class="bold">PMID: </span><a href="/pubmed/33082079" target="_blank">33082079</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/1863023">Survival in patients with primary pulmonary hypertension. Results from a national prospective registry.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">D'Alonzo GE,
|
||
Barst RJ,
|
||
Ayres SM,
|
||
Bergofsky EH,
|
||
Brundage BH,
|
||
Detre KM,
|
||
Fishman AP,
|
||
Goldring RM,
|
||
Groves BM,
|
||
Kernis JT</span><br />
|
||
<span class="medgenPMjournal">Ann Intern Med</span>
|
||
1991 Sep 1;115(5):343-9.
|
||
doi: 10.7326/0003-4819-115-5-343.
|
||
<span class="bold">PMID: </span><a href="/pubmed/1863023" target="_blank">1863023</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pulmonary%20hypertension%2C%20primary%2C%206%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 (1391)</a></div><h3 class="subhead">Therapy</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/36982922">New Drugs and Therapies in Pulmonary Arterial Hypertension.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Shah AJ,
|
||
Beckmann T,
|
||
Vorla M,
|
||
Kalra DK</span><br />
|
||
<span class="medgenPMjournal">Int J Mol Sci</span>
|
||
2023 Mar 19;24(6)
|
||
doi: 10.3390/ijms24065850.
|
||
<span class="bold">PMID: </span><a href="/pubmed/36982922" target="_blank">36982922</a><a href="/pmc/articles/PMC10058689" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/36877098">Phase 3 Trial of Sotatercept for Treatment of Pulmonary Arterial Hypertension.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Hoeper MM,
|
||
Badesch DB,
|
||
Ghofrani HA,
|
||
Gibbs JSR,
|
||
Gomberg-Maitland M,
|
||
McLaughlin VV,
|
||
Preston IR,
|
||
Souza R,
|
||
Waxman AB,
|
||
Grünig E,
|
||
Kopeć G,
|
||
Meyer G,
|
||
Olsson KM,
|
||
Rosenkranz S,
|
||
Xu Y,
|
||
Miller B,
|
||
Fowler M,
|
||
Butler J,
|
||
Koglin J,
|
||
de Oliveira Pena J,
|
||
Humbert M;
|
||
STELLAR Trial Investigators</span><br />
|
||
<span class="medgenPMjournal">N Engl J Med</span>
|
||
2023 Apr 20;388(16):1478-1490.
|
||
Epub 2023 Mar 6
|
||
doi: 10.1056/NEJMoa2213558.
|
||
<span class="bold">PMID: </span><a href="/pubmed/36877098" target="_blank">36877098</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>
|
||
|
||
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<div class="portlet_content ln"><span class="medgenPMauthor">Waxman A,
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<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pulmonary%20hypertension%2C%20primary%2C%206%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 (1516)</a></div><h3 class="subhead">Clinical prediction guides</h3>
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Pausch C,
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Olsson KM,
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Huscher D,
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Pittrow D,
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Grünig E,
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Staehler G,
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Vizza CD,
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Gall H,
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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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Park DH,
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Ewert R,
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Kaemmerer H,
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Kabitz HJ,
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Skowasch D,
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Behr J,
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Milger K,
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Halank M,
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Wilkens H,
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Seyfarth HJ,
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Held M,
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Dumitrescu D,
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Tsangaris I,
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Vonk-Noordegraaf A,
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Ulrich S,
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Klose H,
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Lange TJ,
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Rosenkranz S</span><br />
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<span class="bold">PMID: </span><a href="/pubmed/34737226" target="_blank">34737226</a><a href="/pmc/articles/PMC9260123" target="_blank" class="PubMedFree">Free PMC Article</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/34588193">Mendelian randomisation and experimental medicine approaches to interleukin-6 as a drug target in pulmonary arterial hypertension.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Toshner M,
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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,
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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,
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Halank M,
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Wilkens H,
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Wirtz H,
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Pfeuffer-Jovic E,
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Harbaum L,
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Scholtz W,
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Dumitrescu D,
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Bruch L,
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Coghlan G,
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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="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=%22Pulmonary%20hypertension%2C%20primary%2C%206%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 (1418)</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>
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<div class="portlet_content ln">
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<div class="nl"><a target="_blank" href="/pubmed/36947725">Exercise-based rehabilitation programmes for pulmonary hypertension.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Morris NR,
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Kermeen FD,
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Jones AW,
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Lee JY,
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<div class="portlet_content ln"><span class="medgenPMauthor">Burgos LM,
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<div class="nl"><a target="_blank" href="/pubmed/34967848">Cancer Incidence, Mortality, Years of Life Lost, Years Lived With Disability, and Disability-Adjusted Life Years for 29 Cancer Groups From 2010 to 2019: A Systematic Analysis for the Global Burden of Disease Study 2019.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Global Burden of Disease 2019 Cancer Collaboration,
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<div class="nl"><a target="_blank" href="/pubmed/32862030">Pulmonary hypertension and pregnancy outcomes: Systematic Review and Meta-analysis.</a></div>
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|
||
Epub 2020 Aug 24
|
||
doi: 10.1016/j.ejogrb.2020.08.028.
|
||
<span class="bold">PMID: </span><a href="/pubmed/32862030" target="_blank">32862030</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/32619039">Techniques for preventing hypotension during spinal anaesthesia for caesarean section.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Chooi C,
|
||
Cox JJ,
|
||
Lumb RS,
|
||
Middleton P,
|
||
Chemali M,
|
||
Emmett RS,
|
||
Simmons SW,
|
||
Cyna AM</span><br />
|
||
<span class="medgenPMjournal">Cochrane Database Syst Rev</span>
|
||
2020 Jul 1;7(7):CD002251.
|
||
doi: 10.1002/14651858.CD002251.pub4.
|
||
<span class="bold">PMID: </span><a href="/pubmed/32619039" target="_blank">32619039</a><a href="/pmc/articles/PMC7387232" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
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