{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T22:00:48Z","timestamp":1775080848828,"version":"3.50.1"},"reference-count":41,"publisher":"BMJ","issue":"12","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Clin Pathol"],"published-print":{"date-parts":[[2000,12]]},"abstract":"<jats:p>Primary pulmonary hypertension (PPH) represents the end stage of a disruption of pulmonary vascular integrity, of unknown cause. Although PPH is associated with several systemic disorders, there have hitherto been few clues as to the aetiological factors responsible for the pathogenesis of this condition. As an example of the application of modern molecular genetics and positional cloning, this leader desribes the range of studies currently under way, which aim to find the gene that underlies PPH, and summarises the implications of the identification of such a gene.<\/jats:p>","DOI":"10.1136\/jcp.53.12.899","type":"journal-article","created":{"date-parts":[[2002,7,27]],"date-time":"2002-07-27T06:37:59Z","timestamp":1027751879000},"page":"899-903","source":"Crossref","is-referenced-by-count":13,"title":["Primary pulmonary hypertension: the pressure rises for a gene"],"prefix":"10.1136","volume":"53","author":[{"given":"J R","family":"Thomson","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R C","family":"Trembath","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2000,12,1]]},"reference":[{"key":"2025121219295974000_53.12.899.1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.104.1.236"},{"key":"2025121219295974000_53.12.899.2","unstructured":"Rich S. Primary pulmonary hypertension. Executive summary from the world symposium. Primary pulmonary hypertension. World Health Organisation Publications, 1998."},{"key":"2025121219295974000_53.12.899.3","doi-asserted-by":"crossref","first-page":"216","DOI":"10.7326\/0003-4819-107-2-216","volume":"107","year":"1987","journal-title":"Ann Intern Med"},{"key":"2025121219295974000_53.12.899.4","doi-asserted-by":"crossref","first-page":"343","DOI":"10.7326\/0003-4819-115-5-343","volume":"115","year":"1991","journal-title":"Ann Intern Med"},{"key":"2025121219295974000_53.12.899.5","doi-asserted-by":"crossref","first-page":"S29","DOI":"10.1378\/chest.105.2_Supplement.29S","volume":"105","year":"1994","journal-title":"Chest"},{"key":"2025121219295974000_53.12.899.6","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199207093270203"},{"key":"2025121219295974000_53.12.899.7","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.70.4.366"},{"key":"2025121219295974000_53.12.899.8","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(51)90020-4"},{"key":"2025121219295974000_53.12.899.9","first-page":"195","volume":"30","year":"1954","journal-title":"Bull N Y Acad Med"},{"key":"2025121219295974000_53.12.899.10","first-page":"194","volume":"129","year":"1984","journal-title":"Am Rev Respir Dis"},{"key":"2025121219295974000_53.12.899.11","unstructured":"Online mendelian inheritance in man: http:\/\/www.ncbi.nlm.nih.gov\/Omim\/searchomim.html"},{"key":"2025121219295974000_53.12.899.12","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1136\/hrt.21.1.81","volume":"21","year":"1959","journal-title":"Br Heart J"},{"key":"2025121219295974000_53.12.899.13","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.30.3.336"},{"key":"2025121219295974000_53.12.899.14","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1164\/ajrccm.152.1.7599869","volume":"152","year":"1995","journal-title":"Am J Respir Crit Care Med"},{"key":"2025121219295974000_53.12.899.15","doi-asserted-by":"publisher","DOI":"10.1378\/chest.111.6_Supplement.82S"},{"key":"2025121219295974000_53.12.899.16","doi-asserted-by":"publisher","DOI":"10.1097\/00001573-199905000-00006"},{"key":"2025121219295974000_53.12.899.17","doi-asserted-by":"publisher","DOI":"10.1038\/ng0397-277"},{"key":"2025121219295974000_53.12.899.18","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.95.12.2603"},{"key":"2025121219295974000_53.12.899.19","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199707173370314"},{"key":"2025121219295974000_53.12.899.20","doi-asserted-by":"crossref","first-page":"1055","DOI":"10.1164\/ajrccm.161.3.9906051","volume":"161","year":"2000","journal-title":"Am J Respir Crit Care Med"},{"key":"2025121219295974000_53.12.899.21","doi-asserted-by":"publisher","DOI":"10.1006\/geno.2000.6291"},{"key":"2025121219295974000_53.12.899.22","first-page":"286","volume":"144","year":"1994","journal-title":"Am J Pathol"},{"key":"2025121219295974000_53.12.899.23","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199207093270202"},{"key":"2025121219295974000_53.12.899.24","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.95.08122129"},{"key":"2025121219295974000_53.12.899.25","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.physiol.59.1.89"},{"key":"2025121219295974000_53.12.899.26","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199306173282402"},{"key":"2025121219295974000_53.12.899.27","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199507273330403"},{"key":"2025121219295974000_53.12.899.28","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.98.14.1400"},{"key":"2025121219295974000_53.12.899.29","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(05)78495-6"},{"key":"2025121219295974000_53.12.899.30","first-page":"160","volume":"27","year":"1985","journal-title":"Cor Vasa"},{"key":"2025121219295974000_53.12.899.31","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.70.6.537"},{"key":"2025121219295974000_53.12.899.32","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199608293350901"},{"key":"2025121219295974000_53.12.899.33","first-page":"290","volume":"352","year":"1998","journal-title":"Lancet"},{"key":"2025121219295974000_53.12.899.34","doi-asserted-by":"publisher","DOI":"10.1172\/JCI1910"},{"key":"2025121219295974000_53.12.899.35","doi-asserted-by":"publisher","DOI":"10.1378\/chest.114.3_Supplement.225S"},{"key":"2025121219295974000_53.12.899.36","doi-asserted-by":"crossref","first-page":"S125","DOI":"10.1016\/0021-9150(95)90080-2","volume":"118","year":"1995","journal-title":"Atherosclerosis"},{"key":"2025121219295974000_53.12.899.37","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.68.4.820"},{"key":"2025121219295974000_53.12.899.38","doi-asserted-by":"crossref","first-page":"1293","DOI":"10.1016\/S0046-8177(87)80417-3","volume":"18","year":"1987","journal-title":"Hum Pathol"},{"key":"2025121219295974000_53.12.899.39","doi-asserted-by":"publisher","DOI":"10.4065\/73.1.37"},{"key":"2025121219295974000_53.12.899.40","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.155.3.9117037"},{"key":"2025121219295974000_53.12.899.41","doi-asserted-by":"crossref","first-page":"1539","DOI":"10.1016\/0735-1097(91)90688-6","volume":"18","year":"1991","journal-title":"J Am Coll Cardiol"}],"container-title":["Journal of Clinical Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/jcp.53.12.899","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,13]],"date-time":"2025-12-13T03:30:10Z","timestamp":1765596610000},"score":1,"resource":{"primary":{"URL":"https:\/\/jcp.bmj.com\/lookup\/doi\/10.1136\/jcp.53.12.899"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2000,12]]},"references-count":41,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2000,12,1]]},"published-print":{"date-parts":[[2000,12]]}},"alternative-id":["10.1136\/jcp.53.12.899"],"URL":"https:\/\/doi.org\/10.1136\/jcp.53.12.899","relation":{},"ISSN":["0021-9746","1472-4146"],"issn-type":[{"value":"0021-9746","type":"print"},{"value":"1472-4146","type":"electronic"}],"subject":[],"published":{"date-parts":[[2000,12]]}}}