{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,4]],"date-time":"2026-03-04T03:08:04Z","timestamp":1772593684570,"version":"3.50.1"},"reference-count":13,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2026,3,4]],"date-time":"2026-03-04T00:00:00Z","timestamp":1772582400000},"content-version":"vor","delay-in-days":2010,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["www.atsjournals.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2020,9,15]]},"DOI":"10.1164\/rccm.202006-2142ed","type":"journal-article","created":{"date-parts":[[2020,7,15]],"date-time":"2020-07-15T17:52:06Z","timestamp":1594835526000},"page":"789-791","update-policy":"https:\/\/doi.org\/10.1164\/crossmarkpolicies","source":"Crossref","is-referenced-by-count":0,"title":["The Elephant Man Meets Pulmonary Hypertension. A Cautionary Tale"],"prefix":"10.1093","volume":"202","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6045-1979","authenticated-orcid":false,"given":"Nicole F.","family":"Ruopp","sequence":"first","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Brigham and Women\u2019s Hospital, Boston, Massachusetts, and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Harrison W.","family":"Farber","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, and Sleep Medicine, Tufts Medical Center, Boston, Massachusetts"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2020,9,1]]},"reference":[{"key":"2026030321470864500_bib1","volume-title":"The true history of the elephant man","author":"Howell","year":"1992","edition":"3rd ed"},{"key":"2026030321470864500_bib2","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1093\/oxfordjournals.aje.a010118","article-title":"NF1 gene and neurofibromatosis 1","volume":"151","author":"Rasmussen","year":"2000","journal-title":"Am J Epidemiol"},{"key":"2026030321470864500_bib3","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1164\/rccm.202001-0105OC","article-title":"Phenotype and outcomes of pulmonary hypertension associated with neurofibromatosis type 1","volume":"202","author":"Jutant","year":"2020","journal-title":"Am J Respir Crit Care Med"},{"key":"2026030321470864500_bib4","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1002\/ajmg.1320250315","article-title":"Pulmonary hypertension and interstitial fibrosis in von Recklinghausen neurofibromatosis","volume":"25","author":"Porterfield","year":"1986","journal-title":"Am J Med Genet"},{"key":"2026030321470864500_bib5","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1097\/MD.0b013e31821be2b7","article-title":"Pulmonary hypertension in patients with neurofibromatosis type I","volume":"90","author":"Montani","year":"2011","journal-title":"Medicine (Baltimore)"},{"key":"2026030321470864500_bib6","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.1164\/rccm.200510-1668OC","article-title":"Pulmonary arterial hypertension in France: results from a national registry","volume":"173","author":"Humbert","year":"2006","journal-title":"Am J Respir Crit Care Med"},{"key":"2026030321470864500_bib7","doi-asserted-by":"crossref","first-page":"1093","DOI":"10.1183\/09031936.00010409","article-title":"Alterations in oestrogen metabolism: implications for higher penetrance of familial pulmonary arterial hypertension in females","volume":"34","author":"Austin","year":"2009","journal-title":"Eur Respir J"},{"key":"2026030321470864500_bib8","doi-asserted-by":"crossref","first-page":"1801913","DOI":"10.1183\/13993003.01913-2018","article-title":"Haemodynamic definitions and updated clinical classification of pulmonary hypertension","volume":"53","author":"Simonneau","year":"2019","journal-title":"Eur Respir J"},{"key":"2026030321470864500_bib9","doi-asserted-by":"crossref","first-page":"180053","DOI":"10.1183\/16000617.0053-2018","article-title":"Pulmonary hypertension associated with neurofibromatosis type 1","volume":"27","author":"Jutant","year":"2018","journal-title":"Eur Respir Rev"},{"key":"2026030321470864500_bib10","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1183\/09031936.00090608","article-title":"Pulmonary veno-occlusive disease","volume":"33","author":"Montani","year":"2009","journal-title":"Eur Respir J"},{"key":"2026030321470864500_bib11","doi-asserted-by":"crossref","first-page":"1681","DOI":"10.1164\/ajrccm.157.5.9708065","article-title":"Pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary hemangiomatosis","volume":"157","author":"Humbert","year":"1998","journal-title":"Am J Respir Crit Care Med"},{"key":"2026030321470864500_bib12","doi-asserted-by":"crossref","first-page":"1043","DOI":"10.1378\/chest.15-0300","article-title":"Five-year outcomes of patients enrolled in the REVEAL registry","volume":"148","author":"Farber","year":"2015","journal-title":"Chest"},{"key":"2026030321470864500_bib13","doi-asserted-by":"crossref","first-page":"683","DOI":"10.1136\/bmj.293.6548.683","article-title":"The proteus syndrome: the elephant man diagnosed","volume":"293","author":"Tibbles","year":"1986","journal-title":"Br Med J (Clin Res Ed)"}],"container-title":["American Journal of Respiratory and Critical Care Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ajrccm\/article-pdf\/202\/6\/789\/67175862\/ajrccm_202_6_789.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ajrccm\/article-pdf\/202\/6\/789\/67175862\/ajrccm_202_6_789.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,4]],"date-time":"2026-03-04T02:47:16Z","timestamp":1772592436000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ajrccm\/article\/202\/6\/789\/8495225"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,9,1]]},"references-count":13,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2020,9,1]]},"published-print":{"date-parts":[[2020,9,15]]}},"URL":"https:\/\/doi.org\/10.1164\/rccm.202006-2142ed","relation":{},"ISSN":["1073-449X","1535-4970"],"issn-type":[{"value":"1073-449X","type":"print"},{"value":"1535-4970","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2020,9]]},"published":{"date-parts":[[2020,9,1]]},"assertion":[{"value":"2020-09-15","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}