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<meta name="keywords" content="C0238378, desquamative interstitial pneumonia, desquamative interstitial pneumonitis, dip, disease or syndrome, familial desquamative interstitial pneumonitis, filling of the alveoli with alveolar macrophages, filling of the alveoli with desquamated epithelial cells, ild, desquamative, interstitial lung disease, desquamative, interstitial pneumonitis, desquamative, familial, intra-alveolar accumulation of macrophages, pneumonia, desquamative interstitial, familial, pneumonitis, desquamative interstitial, familial, rbild, respiratory bronchiolitis interstitial lung disease, respiratory bronchiolitis-associated interstitial lung disease, 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="Interstitial lung disease (ILD), or pneumonitis, is a heterogeneous group of disorders characterized pathologically by expansion of the interstitial compartment of the lung by inflammatory cells. Fibrosis occurs in many cases (Visscher and Myers, 2006). See also interstitial lung disease-1 (ILD1; 619611). Desquamative interstitial pneumonitis (DIP) was originally described as a pathologic entity by Liebow et al. (1965). Lung biopsy shows diffuse and uniform filling of alveoli by clusters of cells which Liebow et al. (1965) speculated to be 'desquamated pneumocytes.' Since then, these cells have been shown primarily to be pigmented alveolar macrophages. Other features include thickened alveolar septa with an infiltrate of inflammatory cells and plump, cuboidal type II pneumocytes. Mild collagen deposition without architectural distortion or honeycombing may be present. Different forms of ILD represent pathologic classifications based on histologic patterns rather than clinical diagnoses and may occur in a variety of clinical contexts (Visscher and Myers, 2006). Although DIP occurs most often as a sporadic disorder in adults during the third to fifth decade of life and is highly associated with smoking (Carrington et al., 1978), reports of a familial form with onset in infancy and early death suggest a genetic basis (Sharief et al., 1994). Cases of DIP reported in infants are often more severe and refractory to treatment than those reported in adults (Nogee et al., 2001)." /><meta name="robots" content="index,nofollow,noarchive" />
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<!--
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||
UID=65962
|
||
ConceptID=C0238378
|
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-->
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<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Desquamative interstitial pneumonia<span class="h1sub">(DIP)</span></div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>65962</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.">C0238378</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>Synonyms:</td>
|
||
<td>Desquamative interstitial pneumonitis; DIP; ILD, DESQUAMATIVE; INTERSTITIAL LUNG DISEASE, DESQUAMATIVE; INTERSTITIAL PNEUMONITIS, DESQUAMATIVE, FAMILIAL; PNEUMONIA, DESQUAMATIVE INTERSTITIAL, FAMILIAL</td></tr>
|
||
<tr><td><span class="bold">SNOMED CT: </span></td>
|
||
<td>Desquamative interstitial pneumonia (8549006); Desquamative interstitial pneumonitis (8549006)</td></tr>
|
||
<tr><td colspan="2" class="small"> </td></tr><tr><td>HPO:</td>
|
||
<td><a target="_blank" title="Human Phenotype Ontology" href="https://hpo.jax.org/app/browse/term/HP:0005942">HP:0005942</a></td></tr>
|
||
<tr><td>Monarch Initiative:</td>
|
||
<td><a href="https://monarchinitiative.org/disease/MONDO:0009887" target="_blank">MONDO:0009887</a></td></tr>
|
||
<tr><td>OMIM<span class="superscript">®</span>:</td>
|
||
<td><a href="https://omim.org/entry/263000" target="_blank">263000</a></td></tr>
|
||
<tr><td>Orphanet:</td>
|
||
<td><a target="_blank" title="Orphanet: The portal for rare diseases and orphan drugs" href="http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=98852">ORPHA98852</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">Interstitial lung disease (ILD), or pneumonitis, is a heterogeneous group of disorders characterized pathologically by expansion of the interstitial compartment of the lung by inflammatory cells. Fibrosis occurs in many cases (Visscher and Myers, 2006). See also interstitial lung disease-1 (ILD1; 619611). Desquamative interstitial pneumonitis (DIP) was originally described as a pathologic entity by Liebow et al. (1965). Lung biopsy shows diffuse and uniform filling of alveoli by clusters of cells which Liebow et al. (1965) speculated to be 'desquamated pneumocytes.' Since then, these cells have been shown primarily to be pigmented alveolar macrophages. Other features include thickened alveolar septa with an infiltrate of inflammatory cells and plump, cuboidal type II pneumocytes. Mild collagen deposition without architectural distortion or honeycombing may be present. Different forms of ILD represent pathologic classifications based on histologic patterns rather than clinical diagnoses and may occur in a variety of clinical contexts (Visscher and Myers, 2006). Although DIP occurs most often as a sporadic disorder in adults during the third to fifth decade of life and is highly associated with smoking (Carrington et al., 1978), reports of a familial form with onset in infancy and early death suggest a genetic basis (Sharief et al., 1994). Cases of DIP reported in infants are often more severe and refractory to treatment than those reported in adults (Nogee et al., 2001). [from <a title="Online Mendelian Inheritance in Man" href="http://www.omim.org" class="defSource" target="_blank">OMIM</a>]</div>
|
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</div>
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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_370652"><div><strong>Tubulointerstitial fibrosis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>370652</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.">C1969372</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">A progressive detrimental connective tissue deposition (fibrosis) on the kidney parenchyma involving the tubules and interstitial tissue of the kidney. Tubulointerstitial injury in the kidney is complex, involving a number of independent and overlapping cellular and molecular pathways, with renal interstitial fibrosis and tubular atrophy (IF/TA) as the final common pathway. However, IF and TA are separable, as shown by the profound TA in renal artery stenosis, which characteristically has little or no fibrosis (or inflammation). For new annotations it is preferable to annotate to the specific HPO terms for Renal interstitial fibrosis and/or Renal tubular atrophy.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/370652">Feature record</a> | <a href="/medgen?term=%22Tubulointerstitial%20fibrosis%22%5BClinical%20Features%5D%20OR%20370652%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_18765"><div><strong>Cor pulmonale</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>18765</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.">C0034072</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Right-sided heart failure resulting from chronic hypertension in the pulmonary arteries and right ventricle.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/18765">Feature record</a> | <a href="/medgen?term=%22Cor%20pulmonale%22%5BClinical%20Features%5D%20OR%2018765%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_41325"><div><strong>Cough</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>41325</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.">C0010200</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Sign or Symptom</dd></dl></div></div></div>
|
||
<div class="spaceAbove">A sudden, audible expulsion of air from the lungs through a partially closed glottis, preceded by inhalation.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/41325">Feature record</a> | <a href="/medgen?term=%22Cough%22%5BClinical%20Features%5D%20OR%2041325%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_66669"><div><strong>Tachypnea</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>66669</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.">C0231835</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Finding</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Very rapid breathing.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/66669">Feature record</a> | <a href="/medgen?term=%22Tachypnea%22%5BClinical%20Features%5D%20OR%2066669%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_65962"><div><strong>Desquamative interstitial pneumonia</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>65962</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.">C0238378</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Interstitial lung disease (ILD), or pneumonitis, is a heterogeneous group of disorders characterized pathologically by expansion of the interstitial compartment of the lung by inflammatory cells. Fibrosis occurs in many cases (Visscher and Myers, 2006). See also interstitial lung disease-1 (ILD1; 619611). Desquamative interstitial pneumonitis (DIP) was originally described as a pathologic entity by Liebow et al. (1965). Lung biopsy shows diffuse and uniform filling of alveoli by clusters of cells which Liebow et al. (1965) speculated to be 'desquamated pneumocytes.' Since then, these cells have been shown primarily to be pigmented alveolar macrophages. Other features include thickened alveolar septa with an infiltrate of inflammatory cells and plump, cuboidal type II pneumocytes. Mild collagen deposition without architectural distortion or honeycombing may be present. Different forms of ILD represent pathologic classifications based on histologic patterns rather than clinical diagnoses and may occur in a variety of clinical contexts (Visscher and Myers, 2006). Although DIP occurs most often as a sporadic disorder in adults during the third to fifth decade of life and is highly associated with smoking (Carrington et al., 1978), reports of a familial form with onset in infancy and early death suggest a genetic basis (Sharief et al., 1994). Cases of DIP reported in infants are often more severe and refractory to treatment than those reported in adults (Nogee et al., 2001).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/65962">Feature record</a> | <a href="/medgen?term=%22Desquamative%20interstitial%20pneumonia%22%5BClinical%20Features%5D%20OR%2065962%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_96907"><div><strong>Respiratory distress</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>96907</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.">C0476273</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Sign or Symptom</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Respiratory distress is objectively observable as the physical or emotional consequences from the experience of dyspnea. The physical presentation of respiratory distress is generally referred to as labored breathing, while the sensation of respiratory distress is called shortness of breath or dyspnea.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/96907">Feature record</a> | <a href="/medgen?term=%22Respiratory%20distress%22%5BClinical%20Features%5D%20OR%2096907%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_154380"><div><strong>Recurrent upper respiratory tract infections</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>154380</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.">C0581381</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">An increased susceptibility to upper respiratory tract infections as manifested by a history of recurrent upper respiratory tract infections (running ears - otitis, sinusitis, pharyngitis, tonsillitis).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/154380">Feature record</a> | <a href="/medgen?term=%22Recurrent%20upper%20respiratory%20tract%20infections%22%5BClinical%20Features%5D%20OR%20154380%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_257837"><div><strong>Respiratory failure</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>257837</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.">C1145670</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">A severe form of respiratory insufficiency characterized by inadequate gas exchange such that the levels of oxygen or carbon dioxide cannot be maintained within normal limits.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/257837">Feature record</a> | <a href="/medgen?term=%22Respiratory%20failure%22%5BClinical%20Features%5D%20OR%20257837%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_1785168"><div><strong>Type II pneumocyte hypertrophy</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1785168</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.">C5539455</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Finding</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Increase in size of type II pneumocytes, characterized by qualitative morphologic alterations, including cuboidal shapes, increased nucleocytoplasmic ratio, enlarged nuclei, prominent nucleoli, and various alterations in their nuclear chromatin.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/1785168">Feature record</a> | <a href="/medgen?term=%22Type%20II%20pneumocyte%20hypertrophy%22%5BClinical%20Features%5D%20OR%201785168%5Buid%5D">Search on this feature</a></div></div>
|
||
<div class="divPopper rprt" id="clin_867398"><div><strong>Abnormality of metabolism/homeostasis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>867398</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.">C4021768</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Finding</dd></dl></div></div></div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/867398">Feature record</a> | <a href="/medgen?term=%22Abnormality%20of%20metabolism%2Fhomeostasis%22%5BClinical%20Features%5D%20OR%20867398%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="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_867398" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Abnormality of metabolism/homeostasis</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_18765" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Cor pulmonale</a></span></li></ul></li><li><span class="TLline">Abnormality of the genitourinary system</span><ul><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_370652" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Tubulointerstitial fibrosis</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_41325" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Cough</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_65962" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Desquamative interstitial pneumonia</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_154380" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Recurrent upper respiratory tract infections</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_96907" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Respiratory distress</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_257837" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Respiratory failure</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_66669" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Tachypnea</a></span></li><li class="TLline">
|
||
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_1785168" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Type II pneumocyte hypertrophy</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></ul></div></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><li><a href="#tabORDO">Orphanet</a></li></ul><div id="tabGTR"><div class="search_result"><div class="rprts"><div class="chiclet_legend"><span class="chiclet_list" style="position:static;"><span title="Clinical test" class="chiclet Ccolor round">C</span><span>Clinical test, </span><span title="Research test" class="chiclet Rcolor round">R</span><span>Research test, </span><span title="OMIM" class="chiclet Ocolor ">O</span><span>OMIM, </span><span title="GeneReview" class="chiclet Gcolor">G</span><span><em>GeneReviews</em>, </span><span title="ClinVar" class="chiclet Vcolor">V</span><span>ClinVar </span></span></div><div id="hierarchy" class="margin_t1"><div class="ds_tree"><ul><li class="matched_ds"><span class="chiclet_list"><span class="chiclet unavailable round" title="Clinical test">C</span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet Ocolor" title="OMIM"><a ref="ncbi_uid=65962" target="_blank" href="/omim/263000">O</a></span><span class="chiclet unavailable" title="GeneReviews">G</span><span class="chiclet unavailable" title="ClinVar">V</span></span><span class="TLline">Desquamative interstitial pneumonia</span></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><ul><li><span class="TLline"><a href="/medgen/867443" ref="tree=MeSH" title="MedGen record for Phenotypic abnormality">Phenotypic abnormality</a></span><ul><li><span class="TLline"><a href="/medgen/866322" ref="tree=MeSH" title="MedGen record for Abnormality of the respiratory system">Abnormality of the respiratory system</a></span><ul><li><span class="TLline"><a href="/medgen/220360" ref="tree=MeSH" title="MedGen record for Abnormal respiratory system physiology">Abnormal respiratory system physiology</a></span><ul><li><span class="TLline"><a href="/medgen/868593" ref="tree=MeSH" title="MedGen record for Abnormal respiratory system morphology">Abnormal respiratory system morphology</a></span><ul><li><span class="TLline"><a href="/medgen/892303" ref="tree=MeSH" title="MedGen record for Abnormality of the lung">Abnormality of the lung</a></span><ul><li><span class="TLline"><a href="/medgen/1788738" ref="tree=MeSH" title="MedGen record for Abnormal pulmonary interstitial morphology">Abnormal pulmonary interstitial morphology</a></span><ul><li><span class="matched_ds">Desquamative interstitial pneumonia</span></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></div></div><div id="tabORDO">Follow <a target="_blank" href="http://www.orpha.net/consor/cgi-bin/Disease_Classif.php?lng=EN&data_id=156&PatId=13869&search=Disease_Classif_Simple&new=1" class="ital bold">this link</a> to review classifications for <span class="ital">Desquamative interstitial pneumonia</span> in Orphanet.</div></div></div>
|
||
</div>
|
||
|
||
<div class="portlet mgSection" id="ID_112">
|
||
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Conditions_with_this_feature">Conditions with this feature</h1><a sid="112" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln clinfeat">
|
||
<div class="divPopper rprt" id="rdis_65962"><div><strong>Desquamative interstitial pneumonia</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>65962</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.">C0238378</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Interstitial lung disease (ILD), or pneumonitis, is a heterogeneous group of disorders characterized pathologically by expansion of the interstitial compartment of the lung by inflammatory cells. Fibrosis occurs in many cases (Visscher and Myers, 2006). See also interstitial lung disease-1 (ILD1; 619611). Desquamative interstitial pneumonitis (DIP) was originally described as a pathologic entity by Liebow et al. (1965). Lung biopsy shows diffuse and uniform filling of alveoli by clusters of cells which Liebow et al. (1965) speculated to be 'desquamated pneumocytes.' Since then, these cells have been shown primarily to be pigmented alveolar macrophages. Other features include thickened alveolar septa with an infiltrate of inflammatory cells and plump, cuboidal type II pneumocytes. Mild collagen deposition without architectural distortion or honeycombing may be present. Different forms of ILD represent pathologic classifications based on histologic patterns rather than clinical diagnoses and may occur in a variety of clinical contexts (Visscher and Myers, 2006). Although DIP occurs most often as a sporadic disorder in adults during the third to fifth decade of life and is highly associated with smoking (Carrington et al., 1978), reports of a familial form with onset in infancy and early death suggest a genetic basis (Sharief et al., 1994). Cases of DIP reported in infants are often more severe and refractory to treatment than those reported in adults (Nogee et al., 2001).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/65962">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_368844"><div><strong>Surfactant metabolism dysfunction, pulmonary, 1</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>368844</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.">C1968602</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Inborn errors of pulmonary surfactant metabolism are genetically heterogeneous disorders resulting in severe respiratory insufficiency or failure in full-term neonates or infants. These disorders are associated with various pathologic entities, including pulmonary alveolar proteinosis (PAP), desquamative interstitial pneumonitis (DIP), or cellular nonspecific interstitial pneumonitis (NSIP) (Clark and Clark, 2005). A clinically similar disorder characterized by respiratory distress (267450) can affect preterm infants, who show developmental deficiency of surfactant. Acquired PAP (610910) is an autoimmune disorder characterized by the presence of autoantibodies to CSF2 (138960). Genetic Heterogeneity of Pulmonary Surfactant Metabolism Dysfunction See also SMDP2 (610913), caused by mutation in the SPTPC gene (178620) on 8p21; SMDP3 (610921), caused by mutation in the ABCA3 gene (601615) on 16p13; SMDP4 (300770), caused by mutation in the CSF2RA gene (306250) on Xp22; and SMDP5 (614370), caused by mutation in the CSF2RB gene (138981) on 22q12.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/368844">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_410074"><div><strong>Interstitial lung disease due to ABCA3 deficiency</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>410074</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.">C1970456</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">For a general phenotypic description and a discussion of genetic heterogeneity of pulmonary surfactant metabolism dysfunction, see SMDP1 (265120).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/410074">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_410078"><div><strong>Surfactant metabolism dysfunction, pulmonary, 2</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>410078</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.">C1970470</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Pulmonary surfactant metabolism dysfunction-2 (SMDP2) is a rare autosomal dominant disease associated with progressive respiratory insufficiency and lung disease with a variable clinical course. The pathophysiology of the disorder is postulated to involve intracellular accumulation of a structurally defective SPC protein (Thomas et al., 2002). For a general phenotypic description and a discussion of genetic heterogeneity of pulmonary surfactant metabolism dysfunction, see SMDP1 (265120).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/410078">Condition Record</a></div></div>
|
||
<div class="divPopper rprt" id="rdis_863232"><div><strong>STAT3-related early-onset multisystem autoimmune disease</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>863232</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.">C4014795</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Infantile-onset multisystem autoimmune disease-1 is characterized by early childhood onset of a spectrum of autoimmune disorders affecting multiple organs. Common manifestations include insulin-dependent diabetes mellitus and autoimmune enteropathy, or celiac disease, and autoimmune hematologic disorders. Other features include short stature and nonspecific dermatitis. More variable features include hypothyroidism, autoimmune arthritis, and delayed puberty. Some patients may show recurrent infections. The disorder results from an inborn error of cytokine signaling (summary by Flanagan et al., 2014 and Milner et al., 2015). Genetic Heterogeneity of Infantile-Onset Multisystem Autoimmune Disease See also ADMIO2 (617006), caused by mutation in the ZAP70 gene (176947) on chromosome 2q12, and ADMIO3 (620430), caused by mutation in the CBLB gene (604491) on chromosome 3q13.</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/863232">Condition Record</a></div></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_65962" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Desquamative interstitial pneumonia</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_410074" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Interstitial lung disease due to ABCA3 deficiency</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_863232" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">STAT3-related early-onset multisystem autoimmune disease</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_368844" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Surfactant metabolism dysfunction, pulmonary, 1</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_410078" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Surfactant metabolism dysfunction, pulmonary, 2</a></div></div>
|
||
</div>
|
||
|
||
<div class="portlet mgSection" id="ID_105">
|
||
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Professional_guidelines">Professional guidelines</h1><a sid="105" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln"><h3 class="subhead">PubMed<a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#guidelinesHelpPM"><img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a></h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/29222007">Current Concepts in Pathogenesis, Diagnosis, and Management of Smoking-Related Interstitial Lung Diseases.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Kumar A,
|
||
Cherian SV,
|
||
Vassallo R,
|
||
Yi ES,
|
||
Ryu JH</span><br />
|
||
<span class="medgenPMjournal">Chest</span>
|
||
2018 Aug;154(2):394-408.
|
||
Epub 2017 Dec 5
|
||
doi: 10.1016/j.chest.2017.11.023.
|
||
<span class="bold">PMID: </span><a href="/pubmed/29222007" target="_blank">29222007</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/15103243">Advances in the treatment of rheumatic interstitial lung disease.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Vassallo R,
|
||
Thomas CF</span><br />
|
||
<span class="medgenPMjournal">Curr Opin Rheumatol</span>
|
||
2004 May;16(3):186-91.
|
||
doi: 10.1097/00002281-200405000-00004.
|
||
<span class="bold">PMID: </span><a href="/pubmed/15103243" target="_blank">15103243</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/4746546">Desquamative interstitial pneumonia. Case report of an infant unresponsive to treatment.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Howatt WF,
|
||
Heidelberger KP,
|
||
LeGlovan DP,
|
||
Schnitzer B</span><br />
|
||
<span class="medgenPMjournal">Am J Dis Child</span>
|
||
1973 Sep;126(3):346-8.
|
||
doi: 10.1001/archpedi.1973.02110190304011.
|
||
<span class="bold">PMID: </span><a href="/pubmed/4746546" target="_blank">4746546</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22desquamative%20interstitial%20pneumonia%22%5Btiab%3A~0%5D)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">See all (7)</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/37055089">Smoking-Related Interstitial Lung Diseases.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Alarcon-Calderon A,
|
||
Vassallo R,
|
||
Yi ES,
|
||
Ryu JH</span><br />
|
||
<span class="medgenPMjournal">Immunol Allergy Clin North Am</span>
|
||
2023 May;43(2):273-287.
|
||
Epub 2023 Mar 1
|
||
doi: 10.1016/j.iac.2023.01.007.
|
||
<span class="bold">PMID: </span><a href="/pubmed/37055089" target="_blank">37055089</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/36737166">Smoking-related interstitial lung disease.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Serrano Gotarredona MP,
|
||
Navarro Herrero S,
|
||
Gómez Izquierdo L,
|
||
Rodríguez Portal JA</span><br />
|
||
<span class="medgenPMjournal">Radiologia (Engl Ed)</span>
|
||
2022 Dec;64 Suppl 3:277-289.
|
||
doi: 10.1016/j.rxeng.2022.10.008.
|
||
<span class="bold">PMID: </span><a href="/pubmed/36737166" target="_blank">36737166</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/34445658">Fibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key Players.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Samarelli AV,
|
||
Tonelli R,
|
||
Marchioni A,
|
||
Bruzzi G,
|
||
Gozzi F,
|
||
Andrisani D,
|
||
Castaniere I,
|
||
Manicardi L,
|
||
Moretti A,
|
||
Tabbì L,
|
||
Cerri S,
|
||
Beghè B,
|
||
Dominici M,
|
||
Clini E</span><br />
|
||
<span class="medgenPMjournal">Int J Mol Sci</span>
|
||
2021 Aug 19;22(16)
|
||
doi: 10.3390/ijms22168952.
|
||
<span class="bold">PMID: </span><a href="/pubmed/34445658" target="_blank">34445658</a><a href="/pmc/articles/PMC8396471" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/20553056">Amiodarone: review of pulmonary effects and toxicity.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Papiris SA,
|
||
Triantafillidou C,
|
||
Kolilekas L,
|
||
Markoulaki D,
|
||
Manali ED</span><br />
|
||
<span class="medgenPMjournal">Drug Saf</span>
|
||
2010 Jul 1;33(7):539-58.
|
||
doi: 10.2165/11532320-000000000-00000.
|
||
<span class="bold">PMID: </span><a href="/pubmed/20553056" target="_blank">20553056</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/5902256">Desquamative interstitial pneumonia.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Gaensler EA,
|
||
Goff AM,
|
||
Prowse CM</span><br />
|
||
<span class="medgenPMjournal">N Engl J Med</span>
|
||
1966 Jan 20;274(3):113-28.
|
||
doi: 10.1056/NEJM196601202740301.
|
||
<span class="bold">PMID: </span><a href="/pubmed/5902256" target="_blank">5902256</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Desquamative%20interstitial%20pneumonia%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 (98)</a></div><h3 class="subhead">Diagnosis</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/37055089">Smoking-Related Interstitial Lung Diseases.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Alarcon-Calderon A,
|
||
Vassallo R,
|
||
Yi ES,
|
||
Ryu JH</span><br />
|
||
<span class="medgenPMjournal">Immunol Allergy Clin North Am</span>
|
||
2023 May;43(2):273-287.
|
||
Epub 2023 Mar 1
|
||
doi: 10.1016/j.iac.2023.01.007.
|
||
<span class="bold">PMID: </span><a href="/pubmed/37055089" target="_blank">37055089</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/36737166">Smoking-related interstitial lung disease.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Serrano Gotarredona MP,
|
||
Navarro Herrero S,
|
||
Gómez Izquierdo L,
|
||
Rodríguez Portal JA</span><br />
|
||
<span class="medgenPMjournal">Radiologia (Engl Ed)</span>
|
||
2022 Dec;64 Suppl 3:277-289.
|
||
doi: 10.1016/j.rxeng.2022.10.008.
|
||
<span class="bold">PMID: </span><a href="/pubmed/36737166" target="_blank">36737166</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/34445658">Fibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key Players.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Samarelli AV,
|
||
Tonelli R,
|
||
Marchioni A,
|
||
Bruzzi G,
|
||
Gozzi F,
|
||
Andrisani D,
|
||
Castaniere I,
|
||
Manicardi L,
|
||
Moretti A,
|
||
Tabbì L,
|
||
Cerri S,
|
||
Beghè B,
|
||
Dominici M,
|
||
Clini E</span><br />
|
||
<span class="medgenPMjournal">Int J Mol Sci</span>
|
||
2021 Aug 19;22(16)
|
||
doi: 10.3390/ijms22168952.
|
||
<span class="bold">PMID: </span><a href="/pubmed/34445658" target="_blank">34445658</a><a href="/pmc/articles/PMC8396471" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/27180915">Cystic Lung Diseases: Algorithmic Approach.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Raoof S,
|
||
Bondalapati P,
|
||
Vydyula R,
|
||
Ryu JH,
|
||
Gupta N,
|
||
Raoof S,
|
||
Galvin J,
|
||
Rosen MJ,
|
||
Lynch D,
|
||
Travis W,
|
||
Mehta S,
|
||
Lazzaro R,
|
||
Naidich D</span><br />
|
||
<span class="medgenPMjournal">Chest</span>
|
||
2016 Oct;150(4):945-965.
|
||
Epub 2016 May 13
|
||
doi: 10.1016/j.chest.2016.04.026.
|
||
<span class="bold">PMID: </span><a href="/pubmed/27180915" target="_blank">27180915</a><a href="/pmc/articles/PMC7534033" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/14338290">DESQUAMATIVE INTERSTITIAL PNEUMONIA.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">LIEBOW AA,
|
||
STEER A,
|
||
BILLINGSLEY JG</span><br />
|
||
<span class="medgenPMjournal">Am J Med</span>
|
||
1965 Sep;39:369-404.
|
||
doi: 10.1016/0002-9343(65)90206-8.
|
||
<span class="bold">PMID: </span><a href="/pubmed/14338290" target="_blank">14338290</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Desquamative%20interstitial%20pneumonia%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 (175)</a></div><h3 class="subhead">Therapy</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/37055089">Smoking-Related Interstitial Lung Diseases.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Alarcon-Calderon A,
|
||
Vassallo R,
|
||
Yi ES,
|
||
Ryu JH</span><br />
|
||
<span class="medgenPMjournal">Immunol Allergy Clin North Am</span>
|
||
2023 May;43(2):273-287.
|
||
Epub 2023 Mar 1
|
||
doi: 10.1016/j.iac.2023.01.007.
|
||
<span class="bold">PMID: </span><a href="/pubmed/37055089" target="_blank">37055089</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/36737166">Smoking-related interstitial lung disease.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Serrano Gotarredona MP,
|
||
Navarro Herrero S,
|
||
Gómez Izquierdo L,
|
||
Rodríguez Portal JA</span><br />
|
||
<span class="medgenPMjournal">Radiologia (Engl Ed)</span>
|
||
2022 Dec;64 Suppl 3:277-289.
|
||
doi: 10.1016/j.rxeng.2022.10.008.
|
||
<span class="bold">PMID: </span><a href="/pubmed/36737166" target="_blank">36737166</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/20553056">Amiodarone: review of pulmonary effects and toxicity.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Papiris SA,
|
||
Triantafillidou C,
|
||
Kolilekas L,
|
||
Markoulaki D,
|
||
Manali ED</span><br />
|
||
<span class="medgenPMjournal">Drug Saf</span>
|
||
2010 Jul 1;33(7):539-58.
|
||
doi: 10.2165/11532320-000000000-00000.
|
||
<span class="bold">PMID: </span><a href="/pubmed/20553056" target="_blank">20553056</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/6062495">Desquamative interstitial pneumonia.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Goff AM,
|
||
McNary WF Jr,
|
||
Gaensler EA</span><br />
|
||
<span class="medgenPMjournal">Med Thorac</span>
|
||
1967;24(5):317-29.
|
||
doi: 10.1159/000192536.
|
||
<span class="bold">PMID: </span><a href="/pubmed/6062495" target="_blank">6062495</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/5902256">Desquamative interstitial pneumonia.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Gaensler EA,
|
||
Goff AM,
|
||
Prowse CM</span><br />
|
||
<span class="medgenPMjournal">N Engl J Med</span>
|
||
1966 Jan 20;274(3):113-28.
|
||
doi: 10.1056/NEJM196601202740301.
|
||
<span class="bold">PMID: </span><a href="/pubmed/5902256" target="_blank">5902256</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Desquamative%20interstitial%20pneumonia%22%20AND%20Therapy%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 (124)</a></div><h3 class="subhead">Prognosis</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/37055089">Smoking-Related Interstitial Lung Diseases.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Alarcon-Calderon A,
|
||
Vassallo R,
|
||
Yi ES,
|
||
Ryu JH</span><br />
|
||
<span class="medgenPMjournal">Immunol Allergy Clin North Am</span>
|
||
2023 May;43(2):273-287.
|
||
Epub 2023 Mar 1
|
||
doi: 10.1016/j.iac.2023.01.007.
|
||
<span class="bold">PMID: </span><a href="/pubmed/37055089" target="_blank">37055089</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/32581141">Desquamative interstitial pneumonia: still orphan and not always benign.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Cottin V</span><br />
|
||
<span class="medgenPMjournal">Eur Respir Rev</span>
|
||
2020 Jun 30;29(156)
|
||
Epub 2020 Jun 23
|
||
doi: 10.1183/16000617.0183-2020.
|
||
<span class="bold">PMID: </span><a href="/pubmed/32581141" target="_blank">32581141</a><a href="/pmc/articles/PMC9489088" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/20854463">Desquamative interstitial pneumonia.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Tazelaar HD,
|
||
Wright JL,
|
||
Churg A</span><br />
|
||
<span class="medgenPMjournal">Histopathology</span>
|
||
2011 Mar;58(4):509-16.
|
||
Epub 2010 Sep 21
|
||
doi: 10.1111/j.1365-2559.2010.03649.x.
|
||
<span class="bold">PMID: </span><a href="/pubmed/20854463" target="_blank">20854463</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/369671">Fibrosing alveolitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Thomas P</span><br />
|
||
<span class="medgenPMjournal">Can Med Assoc J</span>
|
||
1978 Nov 18;119(10):1211-6.
|
||
<span class="bold">PMID: </span><a href="/pubmed/369671" target="_blank">369671</a><a href="/pmc/articles/PMC1818528" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/5902256">Desquamative interstitial pneumonia.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Gaensler EA,
|
||
Goff AM,
|
||
Prowse CM</span><br />
|
||
<span class="medgenPMjournal">N Engl J Med</span>
|
||
1966 Jan 20;274(3):113-28.
|
||
doi: 10.1056/NEJM196601202740301.
|
||
<span class="bold">PMID: </span><a href="/pubmed/5902256" target="_blank">5902256</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Desquamative%20interstitial%20pneumonia%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 (89)</a></div><h3 class="subhead">Clinical prediction guides</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/36808083">ABCA3-related interstitial lung disease beyond infancy.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Li Y,
|
||
Seidl E,
|
||
Knoflach K,
|
||
Gothe F,
|
||
Forstner ME,
|
||
Michel K,
|
||
Pawlita I,
|
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Gesenhues F,
|
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Sattler F,
|
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Yang X,
|
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Kroener C,
|
||
Reu-Hofer S,
|
||
Ley-Zaporozhan J,
|
||
Kammer B,
|
||
Krüger-Stollfuß I,
|
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Dinkel J,
|
||
Carlens J,
|
||
Wetzke M,
|
||
Moreno-Galdó A,
|
||
Torrent-Vernetta A,
|
||
Lange J,
|
||
Krenke K,
|
||
Rumman N,
|
||
Mayell S,
|
||
Sismanlar T,
|
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Aslan A,
|
||
Regamey N,
|
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Proesmans M,
|
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Stehling F,
|
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Naehrlich L,
|
||
Ayse K,
|
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Becker S,
|
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Koerner-Rettberg C,
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Plattner E,
|
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Manali ED,
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Papiris SA,
|
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Campo I,
|
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Kappler M,
|
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Schwerk N,
|
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Griese M</span><br />
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<span class="medgenPMjournal">Thorax</span>
|
||
2023 Jun;78(6):587-595.
|
||
Epub 2023 Feb 20
|
||
doi: 10.1136/thorax-2022-219434.
|
||
<span class="bold">PMID: </span><a href="/pubmed/36808083" target="_blank">36808083</a><a href="/pmc/articles/PMC10314027" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/32581141">Desquamative interstitial pneumonia: still orphan and not always benign.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Cottin V</span><br />
|
||
<span class="medgenPMjournal">Eur Respir Rev</span>
|
||
2020 Jun 30;29(156)
|
||
Epub 2020 Jun 23
|
||
doi: 10.1183/16000617.0183-2020.
|
||
<span class="bold">PMID: </span><a href="/pubmed/32581141" target="_blank">32581141</a><a href="/pmc/articles/PMC9489088" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/29138550">Overlaps and uncertainties of smoking-related idiopathic interstitial pneumonias.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Bak SH,
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Lee HY</span><br />
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<span class="medgenPMjournal">Int J Chron Obstruct Pulmon Dis</span>
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2017;12:3221-3229.
|
||
Epub 2017 Nov 1
|
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doi: 10.2147/COPD.S146899.
|
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<span class="bold">PMID: </span><a href="/pubmed/29138550" target="_blank">29138550</a><a href="/pmc/articles/PMC5677302" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/20553056">Amiodarone: review of pulmonary effects and toxicity.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Papiris SA,
|
||
Triantafillidou C,
|
||
Kolilekas L,
|
||
Markoulaki D,
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Manali ED</span><br />
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<span class="medgenPMjournal">Drug Saf</span>
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2010 Jul 1;33(7):539-58.
|
||
doi: 10.2165/11532320-000000000-00000.
|
||
<span class="bold">PMID: </span><a href="/pubmed/20553056" target="_blank">20553056</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/4096977">Observations on the relationship of desquamative interstitial pneumonia and pulmonary alveolar proteinosis in childhood: a pathologic and experimental study.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Bhagwat AG,
|
||
Wentworth P,
|
||
Conen PE</span><br />
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||
<span class="medgenPMjournal">Chest</span>
|
||
1970 Oct;58(4):326-32.
|
||
doi: 10.1378/chest.58.4.326.
|
||
<span class="bold">PMID: </span><a href="/pubmed/4096977" target="_blank">4096977</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Desquamative%20interstitial%20pneumonia%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 (64)</a></div></div>
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<div class="portlet_content ln">
|
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<div class="nl"><a target="_blank" href="/pubmed/33296607">COVID-19 mimics on chest CT: a pictorial review and radiologic guide.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Hochhegger B,
|
||
Zanon M,
|
||
Altmayer S,
|
||
Mandelli NS,
|
||
Stüker G,
|
||
Mohammed TL,
|
||
Verma N,
|
||
Meirelles GSP,
|
||
Marchiori E</span><br />
|
||
<span class="medgenPMjournal">Br J Radiol</span>
|
||
2021 Feb 1;94(1118):20200703.
|
||
Epub 2020 Dec 9
|
||
doi: 10.1259/bjr.20200703.
|
||
<span class="bold">PMID: </span><a href="/pubmed/33296607" target="_blank">33296607</a><a href="/pmc/articles/PMC7934294" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/32581140">Desquamative interstitial pneumonia: a systematic review of its features and outcomes.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Hellemons ME,
|
||
Moor CC,
|
||
von der Thüsen J,
|
||
Rossius M,
|
||
Odink A,
|
||
Thorgersen LH,
|
||
Verschakelen J,
|
||
Wuyts W,
|
||
Wijsenbeek MS,
|
||
Bendstrup E</span><br />
|
||
<span class="medgenPMjournal">Eur Respir Rev</span>
|
||
2020 Jun 30;29(156)
|
||
Epub 2020 Jun 23
|
||
doi: 10.1183/16000617.0181-2019.
|
||
<span class="bold">PMID: </span><a href="/pubmed/32581140" target="_blank">32581140</a><a href="/pmc/articles/PMC9488565" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/12137662">Nebulised morphine for severe interstitial lung disease.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Polosa R,
|
||
Simidchiev A,
|
||
Walters EH</span><br />
|
||
<span class="medgenPMjournal">Cochrane Database Syst Rev</span>
|
||
2002;2002(3):CD002872.
|
||
doi: 10.1002/14651858.CD002872.
|
||
<span class="bold">PMID: </span><a href="/pubmed/12137662" target="_blank">12137662</a><a href="/pmc/articles/PMC8406932" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Desquamative%20interstitial%20pneumonia%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 (3)</a></div></div>
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<h2 class="offscreen_noflow">Supplemental Content</h2>
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<div class="portlet_content ln"><ul><li><a href="https://www.omim.org/search?index=entry&start=1&limit=10&sort=score%20desc&field=number&search=263000" target="_blank">OMIM</a></li><li><a href="http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Expert=98852" target="_blank">Orphanet</a></li><li><a href="https://clinicaltrials.gov/search?cond=Desquamative%20interstitial%20pneumonia" target="_blank">ClinicalTrials.gov</a></li></ul></div>
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<div class="portlet_content ln"><ul><li><a href="https://catalog.coriell.org/Search?q=263000" target="_blank">Coriell Institute for Medical Research</a></li></ul></div>
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<div class="portlet_content ln"><ul><li><a href="https://www.malacards.org/card/interstitial_pneumonitis_desquamative_familial" target="_blank">MalaCards</a></li><li><a href="https://vsearch.nlm.nih.gov/vivisimo/cgi-bin/query-meta?v:project=medlineplus&query=Desquamative%20interstitial%20pneumonia" target="_blank">MedlinePlus</a></li><li><a href="https://rarediseases.info.nih.gov/diseases/16864/disease" target="_blank">NCATS Office of Rare Diseases Research (GARD)</a></li></ul></div>
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<a class="htb ralinkpopperctrl" ref="log$=activity&linkpos=1" href="/portal/utils/pageresolver.fcgi?recordid=67d52cf767c23b31e0f4fd9f">Desquamative interstitial pneumonia</a>
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<div class="tertiary">MedGen</div>
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