{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T02:19:49Z","timestamp":1792721989262,"version":"4.5.0"},"reference-count":18,"publisher":"European Respiratory Society (ERS)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2000,7,27]]},"published-print":{"date-parts":[[2001,3]]},"abstract":"<jats:p>In 1971 Keutel<jats:italic>et al<\/jats:italic>. described a new syndrome in two siblings presenting with peripheral pulmonary stenoses, brachytelephalangism, neural hearing loss and abnormal cartilage calcification. Recent investigations provided evidence that mutations in the gene encoding the human matrix GLA protein cause Keutel syndrome. With these new insights in the disease the symptomatology of Keutel syndrome was reassessed. The follow-up of the two siblings was studied by clinical and<jats:italic>post mortem<\/jats:italic>examination.<\/jats:p><jats:p>As a new feature of Keutel syndrome tracheobronchial stenosis and concentric calcification of pulmonary, coronary, hepatic, renal, meningeal and cerebral arteries were described.<\/jats:p><jats:p>Complementary to the results in molecular genetics the symptomatology of Keutel syndrome could be revised by clinical and<jats:italic>post mortem<\/jats:italic>examination.<\/jats:p>","DOI":"10.1183\/09031936.01.17305660","type":"journal-article","created":{"date-parts":[[2002,7,27]],"date-time":"2002-07-27T12:36:48Z","timestamp":1027773408000},"page":"566-569","source":"Crossref","is-referenced-by-count":96,"title":["Tracheobronchial stenosis in Keutel syndrome"],"prefix":"10.1183","volume":"17","author":[{"given":"M.","family":"Meier","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.P.","family":"Weng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Alexandrakis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"R\u00fcschoff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Goeckenjan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2001,3,1]]},"reference":[{"key":"2024101808394029000_17.3.566.1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0028-1110200"},{"key":"2024101808394029000_17.3.566.2","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.142.5.903"},{"key":"2024101808394029000_17.3.566.3","first-page":"1084","volume":"88","year":"1962","journal-title":"AJR Am J Roentgenol"},{"key":"2024101808394029000_17.3.566.4","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320240209"},{"key":"2024101808394029000_17.3.566.5","doi-asserted-by":"publisher","DOI":"10.1007\/BF00442449"},{"key":"2024101808394029000_17.3.566.6","doi-asserted-by":"publisher","DOI":"10.1016\/S0009-9260(05)81215-2"},{"key":"2024101808394029000_17.3.566.7","doi-asserted-by":"publisher","DOI":"10.1007\/BF00973228"},{"key":"2024101808394029000_17.3.566.8","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19980630)78:2<182::AID-AJMG18>3.0.CO;2-J"},{"key":"2024101808394029000_17.3.566.9","first-page":"461","volume":"51","year":"1975","journal-title":"Ann Pediatr"},{"key":"2024101808394029000_17.3.566.10","doi-asserted-by":"publisher","DOI":"10.1007\/BF02010925"},{"key":"2024101808394029000_17.3.566.11","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1055\/s-2007-996990","volume":"77","year":"1998","journal-title":"Laryngo-Rhino-Otol"},{"key":"2024101808394029000_17.3.566.12","doi-asserted-by":"publisher","DOI":"10.1007\/BF01958278"},{"key":"2024101808394029000_17.3.566.13","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19990319)83:3<209::AID-AJMG13>3.0.CO;2-1"},{"key":"2024101808394029000_17.3.566.14","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.153.7.765"},{"key":"2024101808394029000_17.3.566.15","first-page":"391","volume":"35","year":"1922","journal-title":"Wien klin Wschr"},{"key":"2024101808394029000_17.3.566.16","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9610(64)90023-6"},{"key":"2024101808394029000_17.3.566.17","doi-asserted-by":"publisher","DOI":"10.1038\/386078a0"},{"key":"2024101808394029000_17.3.566.18","doi-asserted-by":"publisher","DOI":"10.1038\/5102"}],"container-title":["European Respiratory Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1183\/09031936.01.17305660","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,24]],"date-time":"2025-02-24T07:36:19Z","timestamp":1740382579000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.ersnet.org\/lookup\/doi\/10.1183\/09031936.01.17305660"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2001,3]]},"references-count":18,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2001,3,1]]},"published-print":{"date-parts":[[2001,3]]}},"alternative-id":["10.1183\/09031936.01.17305660"],"URL":"https:\/\/doi.org\/10.1183\/09031936.01.17305660","relation":{},"ISSN":["0903-1936","1399-3003"],"issn-type":[{"value":"0903-1936","type":"print"},{"value":"1399-3003","type":"electronic"}],"subject":[],"published":{"date-parts":[[2001,3]]}}}