{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T18:03:44Z","timestamp":1793037824400,"version":"4.5.0"},"reference-count":13,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2018,11,1]],"date-time":"2018-11-01T00:00:00Z","timestamp":1541030400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2018,11,1]],"date-time":"2018-11-01T00:00:00Z","timestamp":1541030400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2019,4]]},"abstract":"<jats:p>Pulmonary arterial hypertension (PAH) can be found in patients suffering from a loss\u2010of\u2010function mutation of the gene encoding for the activin receptor\u2010like kinase 1 (ALK\u20101), a bone morphogenetic protein (BMP) type 1 receptor. Interestingly, ALK\u20101\u00a0mutations also lead to hereditary hemorrhagic telangiectasia (HHT), an autosomal dominant disease characterized by arteriovenous malformations (AVMs) leading to potentially life\u2010threatening bleeding complications such as epistaxis. Current therapeutic options for both diseases are limited and often only temporary or accompanied by severe side effects. Here, we report of a patient with a mutation of the ALK\u20101 gene suffering from both HHT and PAH. Recently, it was shown that tacrolimus increased ALK\u20101 signaling and had beneficial effects in selected end\u2010stage PAH patients. We thus hypothesized that treatment with tacrolimus may prevent disease progression in this patient. Surprisingly, treatment with low\u2010dose tacrolimus dramatically improved his HHT\u2010associated epistaxis but did not attenuate progression of PAH.<\/jats:p>","DOI":"10.1177\/2045894018805406","type":"journal-article","created":{"date-parts":[[2018,9,27]],"date-time":"2018-09-27T16:18:09Z","timestamp":1538065089000},"page":"1-3","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":37,"title":["Treatment with low\u2010dose tacrolimus inhibits bleeding complications in a patient with hereditary hemorrhagic telangiectasia and pulmonary arterial hypertension"],"prefix":"10.1002","volume":"9","author":[{"given":"N","family":"Sommer","sequence":"first","affiliation":[{"name":"Excellence Cluster Cardiopulmonary System University of Giessen and Marburg Lung Center (UGMLC) member of the German Center for Lung Research (DZL) Justus\u2010Liebig\u2010University Giessen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F","family":"Droege","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology Head and Neck Surgery University Hospital Essen University Duisburg\u2010Essen Essen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"KE","family":"Gamen","sequence":"additional","affiliation":[{"name":"Max Planck Institute for Heart and Lung Research Bad Nauheim Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"U","family":"Geisthoff","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology Head and Neck Surgery University Hospital Giessen and Marburg Philipps Universit\u00f0t Marburg Marburg"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H","family":"Gall","sequence":"additional","affiliation":[{"name":"Excellence Cluster Cardiopulmonary System University of Giessen and Marburg Lung Center (UGMLC) member of the German Center for Lung Research (DZL) Justus\u2010Liebig\u2010University Giessen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K","family":"Tello","sequence":"additional","affiliation":[{"name":"Excellence Cluster Cardiopulmonary System University of Giessen and Marburg Lung Center (UGMLC) member of the German Center for Lung Research (DZL) Justus\u2010Liebig\u2010University Giessen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"MJ","family":"Richter","sequence":"additional","affiliation":[{"name":"Excellence Cluster Cardiopulmonary System University of Giessen and Marburg Lung Center (UGMLC) member of the German Center for Lung Research (DZL) Justus\u2010Liebig\u2010University Giessen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"LM","family":"Deubner","sequence":"additional","affiliation":[{"name":"Excellence Cluster Cardiopulmonary System University of Giessen and Marburg Lung Center (UGMLC) member of the German Center for Lung Research (DZL) Justus\u2010Liebig\u2010University Giessen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2694-8444","authenticated-orcid":false,"given":"R","family":"Schmiedel","sequence":"additional","affiliation":[{"name":"Morbus Osler Selbsthilfe Hattingen"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Hecker","sequence":"additional","affiliation":[{"name":"Excellence Cluster Cardiopulmonary System University of Giessen and Marburg Lung Center (UGMLC) member of the German Center for Lung Research (DZL) Justus\u2010Liebig\u2010University Giessen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E","family":"Spiekerkoetter","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine Dept. of Medicine Stanford University Stanford CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K","family":"Wirsching","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology University Medical Center Regensburg Regensburg"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W","family":"Seeger","sequence":"additional","affiliation":[{"name":"Excellence Cluster Cardiopulmonary System University of Giessen and Marburg Lung Center (UGMLC) member of the German Center for Lung Research (DZL) Justus\u2010Liebig\u2010University Giessen Germany"},{"name":"Max Planck Institute for Heart and Lung Research Bad Nauheim Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"HA","family":"Ghofrani","sequence":"additional","affiliation":[{"name":"Department of Medicine Imperial College London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S","family":"Pullamsetti","sequence":"additional","affiliation":[{"name":"Max Planck Institute for Heart and Lung Research Bad Nauheim Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,11]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.3389\/fgene.2015.00001"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.3389\/fgene.2015.00115"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddx358"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI65592"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201411-2061LE"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.20818"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200212060"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/nm.2131"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.2500\/ar.2014.5.0091"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13606"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/FPC.0b013e328364db84"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2012-0470OC"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.coph.2008.03.003"}],"container-title":["Pulmonary Circulation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1177\/2045894018805406","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1177\/2045894018805406","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1177\/2045894018805406","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,28]],"date-time":"2023-08-28T16:08:33Z","timestamp":1693238913000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1177\/2045894018805406"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,11]]},"references-count":13,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2019,4]]}},"alternative-id":["10.1177\/2045894018805406"],"URL":"https:\/\/doi.org\/10.1177\/2045894018805406","archive":["Portico"],"relation":{},"ISSN":["2045-8940","2045-8940"],"issn-type":[{"value":"2045-8940","type":"print"},{"value":"2045-8940","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,11]]}}}