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<meta name="keywords" content="C0238399, finding, lymphangioleiomyomatosis, neoplastic process, pulmonary lymphangiomyomatosis, pulmonary myomatosis, 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="Infiltration of smooth muscle-like cells in lymph vessels as well as the lung (pleura, alveolar septa, bronchi, pulmonary vessels and lymphatics as well as lymph nodes, especially in posterior mediastinum and retroperitoneum). Focal emphysema can develop because of airway narrowing, and the thoracic duct may be obliterated. Pulmonary lymphangiomyomatosis may lead to multiple small cysts with a hamartomatous proliferation of smooth muscle in their walls." /><meta name="robots" content="index,nofollow,noarchive" />
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<title>Pulmonary lymphangiomyomatosis (Concept Id: C0238399)
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<!--
UID=852455
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-->
<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Pulmonary lymphangiomyomatosis</div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>852455</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)&#10;Click for more information.">C0238399</a></dd><dt><span class="dotprefix"></span></dt><dd>Finding; Neoplastic Process</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>Synonyms:</td>
<td>Lymphangioleiomyomatosis; Pulmonary myomatosis</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:0012798">HP:0012798</a></td></tr>
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<div class="portlet mgSection" id="ID_100">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Definition">Definition</h1><a sid="100" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln">Infiltration of smooth muscle-like cells in lymph vessels as well as the lung (pleura, alveolar septa, bronchi, pulmonary vessels and lymphatics as well as lymph nodes, especially in posterior mediastinum and retroperitoneum). Focal emphysema can develop because of airway narrowing, and the thoracic duct may be obliterated. Pulmonary lymphangiomyomatosis may lead to multiple small cysts with a hamartomatous proliferation of smooth muscle in their walls. [from <a title="Human Phenotype Ontology" href="http://www.human-phenotype-ontology.org" class="defSource" target="_blank">HPO</a>]</div>
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<div class="portlet mgSection" id="ID_118">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Term_Hierarchy">Term Hierarchy</h1><a sid="118" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln HierarchyGTR"><div class="jig-ncbitabs"><ul><li><a href="#tabGTR">GTR</a></li><li><a href="#tabMGEN">MeSH</a></li></ul><div id="tabGTR"><div class="search_result"><div class="rprts"><div class="chiclet_legend"><span class="chiclet_list" style="position:static;"><span title="Clinical test" class="chiclet Ccolor round">C</span><span>Clinical test,  </span><span title="Research test" class="chiclet Rcolor round">R</span><span>Research test,  </span><span title="OMIM" class="chiclet Ocolor ">O</span><span>OMIM,  </span><span title="GeneReview" class="chiclet Gcolor">G</span><span><em>GeneReviews</em>,  </span><span title="ClinVar" class="chiclet Vcolor">V</span><span>ClinVar  </span></span></div><div id="hierarchy" class="margin_t1"><div class="ds_tree"><ul><li class="matched_ds"><span class="chiclet_list"><span class="chiclet unavailable round" title="Clinical test">C</span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet unavailable" title="OMIM">O</span><span class="chiclet unavailable" title="GeneReviews">G</span><span class="chiclet unavailable" title="ClinVar">V</span></span><span class="TLline">Pulmonary lymphangiomyomatosis</span></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><ul><li><span class="TLline"><a href="/medgen/19750" ref="tree=MeSH" title="MedGen record for Disease, Respiratory Tract">Disease, Respiratory Tract</a></span><ul><li><span class="TLline"><a href="/medgen/7399" ref="tree=MeSH" title="MedGen record for Disorder of lung">Disorder of lung</a></span><ul><li><span class="TLline"><a href="/medgen/7400" ref="tree=MeSH" title="MedGen record for Neoplasm of lung">Neoplasm of lung</a></span><ul><li><span class="matched_ds">Pulmonary lymphangiomyomatosis</span></li></ul></li></ul></li></ul></li></ul></div></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_148366"><div><strong>Lymphangiomyomatosis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>148366</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)&#10;Click for more information."><span class="highlight" style="background-color:">C0751674</span></a></dd><dt><span class="dotprefix"></span></dt><dd>Neoplastic Process</dd></dl></div></div></div>
<div class="spaceAbove">Lymphangioleiomyomatosis (LAM) is a condition that affects the lungs, the kidneys, and the lymphatic system. The lymphatic system consists of a network of vessels that transport lymph fluid and immune cells throughout the body. Lymph fluid helps exchange immune cells, proteins, and other substances between the blood and tissues.\n\nLAM is found almost exclusively in women. It often occurs as a feature of an inherited syndrome called tuberous sclerosis complex. When LAM occurs alone it is called isolated or sporadic LAM.\n\nSigns and symptoms of LAM most often appear during a woman's thirties. Affected women have an overgrowth of abnormal smooth muscle-like cells (LAM cells) in the lungs, resulting in the formation of lung cysts and the destruction of normal lung tissue. They may also have an accumulation of fluid in the cavity around the lungs (chylothorax).\n\nThe lung abnormalities resulting from LAM may cause difficulty breathing (dyspnea), chest pain, and coughing, which may bring up blood (hemoptysis). Many women with this disorder have recurrent episodes of collapsed lung (spontaneous pneumothorax). The lung problems may be progressive and, without lung transplantation, may eventually lead to limitations in activities of daily living, the need for oxygen therapy, and respiratory failure. Although LAM cells are not considered cancerous, they may spread between tissues (metastasize). As a result, the condition may recur even after lung transplantation.\n\nWomen with LAM may develop cysts in the lymphatic vessels of the chest and abdomen. These cysts are called lymphangioleiomyomas. Affected women may also develop tumors called angiomyolipomas made up of LAM cells, fat cells, and blood vessels. Angiomyolipomas usually develop in the kidneys. Internal bleeding is a common complication of angiomyolipomas.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/148366">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_344288"><div><strong>Tuberous sclerosis 1</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>344288</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)&#10;Click for more information.">C1854465</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Tuberous sclerosis complex (TSC) involves abnormalities of the skin (hypomelanotic macules, confetti skin lesions, facial angiofibromas, shagreen patches, fibrous cephalic plaques, ungual fibromas); brain (subependymal nodules, cortical tubers, and subependymal giant cell astrocytomas [SEGAs], seizures, TSC-associated neuropsychiatric disorder [TAND]); kidneys (benign renal angiomyolipomas, epithelial cysts, oncocytoma, renal cell carcinoma); heart (rhabdomyomas, arrhythmias); and lungs (lymphangioleiomyomatosis [LAM], multifocal micronodular pneumonocyte hyperplasia). Central nervous system-related problems (including TAND) are the leading cause of morbidity, whereas kidney disease is the leading cause of mortality.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/344288">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_348170"><div><strong>Tuberous sclerosis 2</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>348170</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)&#10;Click for more information.">C1860707</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Tuberous sclerosis complex (TSC) involves abnormalities of the skin (hypomelanotic macules, confetti skin lesions, facial angiofibromas, shagreen patches, fibrous cephalic plaques, ungual fibromas); brain (subependymal nodules, cortical tubers, and subependymal giant cell astrocytomas [SEGAs], seizures, TSC-associated neuropsychiatric disorder [TAND]); kidneys (benign renal angiomyolipomas, epithelial cysts, oncocytoma, renal cell carcinoma); heart (rhabdomyomas, arrhythmias); and lungs (lymphangioleiomyomatosis [LAM], multifocal micronodular pneumonocyte hyperplasia). Central nervous system-related problems (including TAND) are the leading cause of morbidity, whereas kidney disease is the leading cause of mortality.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/348170">Condition Record</a></div></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_148366" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Lymphangiomyomatosis</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_344288" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Tuberous sclerosis 1</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_348170" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Tuberous sclerosis 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/36085075">Rapamycin nanoparticles improves drug bioavailability in PLAM treatment by interstitial injection.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Shi Y,
Jiao C,
Lu X,
Nie Y,
Li X,
Han D</span><br />
<span class="medgenPMjournal">Orphanet J Rare Dis</span>
2022 Sep 9;17(1):349.
doi: 10.1186/s13023-022-02511-6.
<span class="bold">PMID: </span><a href="/pubmed/36085075" target="_blank">36085075</a><a href="/pmc/articles/PMC9463820" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22pulmonary%20lymphangiomyomatosis%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 (1)</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/36206158">Diagnostic value and safety of medical thoracoscopy under local anesthesia for unexplained diffuse interstitial lung disease: A retrospective study.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Zhang L,
Xie T,
Li Y,
Zhang B,
Fu Y,
Ding Y,
Wu H</span><br />
<span class="medgenPMjournal">Chron Respir Dis</span>
2022 Jan-Dec;19:14799731221133389.
doi: 10.1177/14799731221133389.
<span class="bold">PMID: </span><a href="/pubmed/36206158" target="_blank">36206158</a><a href="/pmc/articles/PMC9549086" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26618803">RETINAL ASTROCYTIC HAMARTOMA: Optical Coherence Tomography Classification and Correlation With Tuberous Sclerosis Complex.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Pichi F,
Massaro D,
Serafino M,
Carrai P,
Giuliari GP,
Shields CL,
Veronese C,
Ciardella AP,
Nucci P</span><br />
<span class="medgenPMjournal">Retina</span>
2016 Jun;36(6):1199-208.
doi: 10.1097/IAE.0000000000000829.
<span class="bold">PMID: </span><a href="/pubmed/26618803" target="_blank">26618803</a></div>
<div class="nl"><a target="_blank" href="/pubmed/9609163">Accuracy of high-resolution CT in diagnosing lung diseases.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Bonelli FS,
Hartman TE,
Swensen SJ,
Sherrick A</span><br />
<span class="medgenPMjournal">AJR Am J Roentgenol</span>
1998 Jun;170(6):1507-12.
doi: 10.2214/ajr.170.6.9609163.
<span class="bold">PMID: </span><a href="/pubmed/9609163" target="_blank">9609163</a></div>
<div class="nl"><a target="_blank" href="/pubmed/3370906">Early onset of childhood pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Nussbaum E,
Groncy P,
Finklestein J,
Dickman P,
Harrell JH 3rd</span><br />
<span class="medgenPMjournal">Clin Pediatr (Phila)</span>
1988 Jun;27(6):279-84.
doi: 10.1177/000992288802700603.
<span class="bold">PMID: </span><a href="/pubmed/3370906" target="_blank">3370906</a></div>
<div class="nl"><a target="_blank" href="/pubmed/1146965">Pulmonary lymphangiomyomatosis. A review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Corrin B,
Liebow AA,
Friedman PJ</span><br />
<span class="medgenPMjournal">Am J Pathol</span>
1975 May;79(2):348-82.
<span class="bold">PMID: </span><a href="/pubmed/1146965" target="_blank">1146965</a><a href="/pmc/articles/PMC1912658" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pulmonary%20lymphangiomyomatosis%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 (11)</a></div><h3 class="subhead">Diagnosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/8214254">Transbronchial biopsy in lymphangiomyomatosis of the lung. HMB45 for diagnosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Bonetti F,
Chiodera PL,
Pea M,
Martignoni G,
Bosi F,
Zamboni G,
Mariuzzi GM</span><br />
<span class="medgenPMjournal">Am J Surg Pathol</span>
1993 Nov;17(11):1092-102.
doi: 10.1097/00000478-199311000-00002.
<span class="bold">PMID: </span><a href="/pubmed/8214254" target="_blank">8214254</a></div>
<div class="nl"><a target="_blank" href="/pubmed/1487466">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Aerts P,
Van Fraeyenhoven L,
Verschakelen J</span><br />
<span class="medgenPMjournal">J Belge Radiol</span>
1992 Oct;75(5):404-5.
<span class="bold">PMID: </span><a href="/pubmed/1487466" target="_blank">1487466</a></div>
<div class="nl"><a target="_blank" href="/pubmed/7413250">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Pieterse S,
Henderson DW</span><br />
<span class="medgenPMjournal">Pathology</span>
1980 Apr;12(2):168, 284-6.
doi: 10.3109/00313028009060069.
<span class="bold">PMID: </span><a href="/pubmed/7413250" target="_blank">7413250</a></div>
<div class="nl"><a target="_blank" href="/pubmed/453961">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Shuman RL,
Engelman R,
Kittle CF</span><br />
<span class="medgenPMjournal">Ann Thorac Surg</span>
1979 Jan;27(1):70-5.
doi: 10.1016/s0003-4975(10)62975-x.
<span class="bold">PMID: </span><a href="/pubmed/453961" target="_blank">453961</a></div>
<div class="nl"><a target="_blank" href="/pubmed/4719757">Pulmonary lymphangiomyomatosis syndrome.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Stovin PG</span><br />
<span class="medgenPMjournal">J Pathol</span>
1973 Jan;109(1):Pvii.
<span class="bold">PMID: </span><a href="/pubmed/4719757" target="_blank">4719757</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pulmonary%20lymphangiomyomatosis%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 (30)</a></div><h3 class="subhead">Therapy</h3>
<div class="nl"><a target="_blank" href="/pubmed/36543771">Targeting SPHK1/S1PR3-regulated S-1-P metabolic disorder triggers autophagic cell death in pulmonary lymphangiomyomatosis (LAM).</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Li F,
Zhang Y,
Lin Z,
Yan L,
Liu Q,
Li Y,
Pei X,
Feng Y,
Han X,
Yang J,
Zheng F,
Li T,
Zhang Y,
Fu Z,
Shao D,
Yu J,
Li C</span><br />
<span class="medgenPMjournal">Cell Death Dis</span>
2022 Dec 21;13(12):1065.
doi: 10.1038/s41419-022-05511-3.
<span class="bold">PMID: </span><a href="/pubmed/36543771" target="_blank">36543771</a><a href="/pmc/articles/PMC9772321" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/36085075">Rapamycin nanoparticles improves drug bioavailability in PLAM treatment by interstitial injection.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Shi Y,
Jiao C,
Lu X,
Nie Y,
Li X,
Han D</span><br />
<span class="medgenPMjournal">Orphanet J Rare Dis</span>
2022 Sep 9;17(1):349.
doi: 10.1186/s13023-022-02511-6.
<span class="bold">PMID: </span><a href="/pubmed/36085075" target="_blank">36085075</a><a href="/pmc/articles/PMC9463820" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/3184096">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Eisera NB,
Venegoni E,
Colombi R</span><br />
<span class="medgenPMjournal">J R Soc Med</span>
1988 Oct;81(10):607-9.
doi: 10.1177/014107688808101021.
<span class="bold">PMID: </span><a href="/pubmed/3184096" target="_blank">3184096</a><a href="/pmc/articles/PMC1291813" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/6723393">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Dishner W,
Cordasco EM,
Blackburn J,
Demeter S,
Levin H,
Carey WD</span><br />
<span class="medgenPMjournal">Chest</span>
1984 Jun;85(6):796-9.
doi: 10.1378/chest.85.6.796.
<span class="bold">PMID: </span><a href="/pubmed/6723393" target="_blank">6723393</a></div>
<div class="nl"><a target="_blank" href="/pubmed/7432404">Pulmonary lymphangiomyomatosis responsive to progesterone.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">McCarty KS Jr,
Mossler JA,
McLelland R,
Sieker HO</span><br />
<span class="medgenPMjournal">N Engl J Med</span>
1980 Dec 18;303(25):1461-5.
doi: 10.1056/NEJM198012183032506.
<span class="bold">PMID: </span><a href="/pubmed/7432404" target="_blank">7432404</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pulmonary%20lymphangiomyomatosis%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 (17)</a></div><h3 class="subhead">Prognosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/31847710">Renal angiomyolipoma (AML) harboring a missense mutation of TSC2 with copy-neutral loss of heterozygosity (CN-LOH).</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Idogawa M,
Hida T,
Tanaka T,
Ohira N,
Tange S,
Sasaki Y,
Uhara H,
Masumori N,
Tokino T,
Natori H</span><br />
<span class="medgenPMjournal">Cancer Biol Ther</span>
2020 Apr 2;21(4):315-319.
Epub 2019 Dec 17
doi: 10.1080/15384047.2019.1702406.
<span class="bold">PMID: </span><a href="/pubmed/31847710" target="_blank">31847710</a><a href="/pmc/articles/PMC7515489" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/11704609">Chromosome 16 loss of heterozygosity in tuberous sclerosis and sporadic lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Yu J,
Astrinidis A,
Henske EP</span><br />
<span class="medgenPMjournal">Am J Respir Crit Care Med</span>
2001 Oct 15;164(8 Pt 1):1537-40.
doi: 10.1164/ajrccm.164.8.2104095.
<span class="bold">PMID: </span><a href="/pubmed/11704609" target="_blank">11704609</a></div>
<div class="nl"><a target="_blank" href="/pubmed/7887359">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Buckley KM,
DeLuca SA</span><br />
<span class="medgenPMjournal">Am Fam Physician</span>
1995 Mar;51(4):831-3.
<span class="bold">PMID: </span><a href="/pubmed/7887359" target="_blank">7887359</a></div>
<div class="nl"><a target="_blank" href="/pubmed/1643935">Pulmonary lymphangiomyomatosis. Follow-up and long-term outcome with antiestrogen therapy; a report of eight cases.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Urban T,
Kuttenn F,
Gompel A,
Marsac J,
Lacronique J</span><br />
<span class="medgenPMjournal">Chest</span>
1992 Aug;102(2):472-6.
doi: 10.1378/chest.102.2.472.
<span class="bold">PMID: </span><a href="/pubmed/1643935" target="_blank">1643935</a></div>
<div class="nl"><a target="_blank" href="/pubmed/3370906">Early onset of childhood pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Nussbaum E,
Groncy P,
Finklestein J,
Dickman P,
Harrell JH 3rd</span><br />
<span class="medgenPMjournal">Clin Pediatr (Phila)</span>
1988 Jun;27(6):279-84.
doi: 10.1177/000992288802700603.
<span class="bold">PMID: </span><a href="/pubmed/3370906" target="_blank">3370906</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pulmonary%20lymphangiomyomatosis%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 (11)</a></div><h3 class="subhead">Clinical prediction guides</h3>
<div class="nl"><a target="_blank" href="/pubmed/35483879">Tuberous sclerosis complex: a complex case.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Powell RM,
Pattison S,
Moravec JC,
Bhat B,
Guirguis N,
Markie D,
Jones GT,
Copedo J,
Print CG,
Morison IM,
Gavryushkin A,
Gray B,
Wyeth LJ,
Eccles MR,
Macaulay EC</span><br />
<span class="medgenPMjournal">Cold Spring Harb Mol Case Stud</span>
2022 Apr;8(3)
Epub 2022 Apr 28
doi: 10.1101/mcs.a006182.
<span class="bold">PMID: </span><a href="/pubmed/35483879" target="_blank">35483879</a><a href="/pmc/articles/PMC9059781" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/10901911">Lymphangiomyomatosis: radiological and measured lung function deterioration after contrast-enhanced computed tomography.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Teo MS,
Tan LK,
Lim TK</span><br />
<span class="medgenPMjournal">Australas Radiol</span>
1999 May;43(2):249-52.
doi: 10.1046/j.1440-1673.1999.00642.x.
<span class="bold">PMID: </span><a href="/pubmed/10901911" target="_blank">10901911</a></div>
<div class="nl"><a target="_blank" href="/pubmed/1487466">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Aerts P,
Van Fraeyenhoven L,
Verschakelen J</span><br />
<span class="medgenPMjournal">J Belge Radiol</span>
1992 Oct;75(5):404-5.
<span class="bold">PMID: </span><a href="/pubmed/1487466" target="_blank">1487466</a></div>
<div class="nl"><a target="_blank" href="/pubmed/6698139">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">El Allaf D,
Borlee G,
Hadjoudj H,
Henrard L,
Marcelle R,
van Cauwenberge H</span><br />
<span class="medgenPMjournal">Eur J Respir Dis</span>
1984 Feb;65(2):147-52.
<span class="bold">PMID: </span><a href="/pubmed/6698139" target="_blank">6698139</a></div>
<div class="nl"><a target="_blank" href="/pubmed/1146965">Pulmonary lymphangiomyomatosis. A review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Corrin B,
Liebow AA,
Friedman PJ</span><br />
<span class="medgenPMjournal">Am J Pathol</span>
1975 May;79(2):348-82.
<span class="bold">PMID: </span><a href="/pubmed/1146965" target="_blank">1146965</a><a href="/pmc/articles/PMC1912658" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pulmonary%20lymphangiomyomatosis%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 (14)</a></div></div>
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