{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,9,16]],"date-time":"2023-09-16T10:15:47Z","timestamp":1694859347529},"reference-count":7,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2003,9,1]],"date-time":"2003-09-01T00:00:00Z","timestamp":1062374400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2003,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Inborn errors of metabolism have not previously been recognized as a risk factor for acute respiratory distress syndrome (ARDS). We report this complication in four patients with defects of the mitochondrial trifunctional protein (MTP). This enzyme catalyses three steps in the \u03b2\u2010oxidation of long\u2010chain fatty acids. Three of the patients were homozygous for the \u2018common\u2019 1528G&gt;C mutation in the \u03b1\u2010subunit of the MTP, giving rise to long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase deficiency. The fourth patient did not carry this mutation but had severely decreased activities of long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase and long\u2010chain 3\u2010ketoacyl\u2010CoA thiolase. One patient died and histology in this patient showed severe interstitial pulmonary fibrosis. The other three patients recovered after being ventilated for up to 6 months. The high frequency of ARDS in patients with MTP defects suggests that this inborn error may be a risk factor for ARDS.<\/jats:p>","DOI":"10.1023\/a:1025995813914","type":"journal-article","created":{"date-parts":[[2003,10,24]],"date-time":"2003-10-24T22:52:54Z","timestamp":1067035974000},"page":"537-541","source":"Crossref","is-referenced-by-count":12,"title":["Acute respiratory distress syndrome in long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase and mitochondrial trifunctional protein deficiencies"],"prefix":"10.1002","volume":"26","author":[{"given":"C. T.","family":"Lundy","sequence":"first","affiliation":[{"name":"Departments of Child Health and Clinical Biochemistry Royal Belfast Hospital for Sick Children  Belfast"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. P. H.","family":"Shield","sequence":"additional","affiliation":[{"name":"Department of Child Health Royal Hospital for Children  Bristol UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E. A.","family":"Kvittingen","sequence":"additional","affiliation":[{"name":"Institute of Biochemistry Rijkshospitalet  Oslo"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"O. J.","family":"Vinorum","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Ullevaal University Hospital  Oslo Norway"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E. R.","family":"Trimble","sequence":"additional","affiliation":[{"name":"Departments of Child Health and Clinical Biochemistry Royal Belfast Hospital for Sick Children  Belfast"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A. A. M.","family":"Morris","sequence":"additional","affiliation":[{"name":"Metabolic Unit  Great Ormond Street Hospital London UK"},{"name":"Willink Biochemical Genetics Unit Royal Manchester Children's Hospital  Hospital Road, Pendlebury Manchester M27 4HA UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2003,9]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/thorax.57.6.540"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.1950191152"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.79.3.256"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI118863"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/0960-8966(96)00352-5"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1999.tb01089.x"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799419"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1023%2FA%3A1025995813914","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1023\/A%3A1025995813914","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T12:58:30Z","timestamp":1694782710000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1023\/A%3A1025995813914"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2003,9]]},"references-count":7,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2003,9]]}},"alternative-id":["10.1023\/A:1025995813914"],"URL":"https:\/\/doi.org\/10.1023\/a:1025995813914","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2003,9]]}}}