{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,25]],"date-time":"2026-06-25T05:10:48Z","timestamp":1782364248534,"version":"3.54.5"},"reference-count":11,"publisher":"Wiley","issue":"S1","license":[{"start":{"date-parts":[[2009,5,4]],"date-time":"2009-05-04T00:00:00Z","timestamp":1241395200000},"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":[[2009,12]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>We report a Japanese girl affected with a neonatal\u2010onset form of propionic acidaemia (PA). She developed severe metabolic crisis after dehydration at 2 years of age. Bradycardia with complete atrioventricular block responded to haemodiafiltration, but severe cardiac failure was refractory to inotropic treatment. She was diagnosed with acute cardiac dysfunction caused by PA\u2010induced metabolic crisis. Extracorporeal membrane oxygenation (ECMO), a technique for providing mechanical circulatory support, was required. This is the first case report of a PA patient who recovered from a life\u2010threatening metabolic crisis with cardiac failure by ECMO. Cardiac failure may be a cause of death, but it is occasionally an under\u2010recognized complication. Mitochondrial dysfunction in the myocardium due to propionyl\u2010CoA could contribute to the pathomechanism of cardiac complications of PA. We believe that ECMO should be attempted in PA patients with cardiac failure, in addition to haemodiafiltration and other therapeutic measures, because doing so may lead to the recovery of cardiac dysfunction, as was evident in our patient. In conclusion, prompt investigations and management of cardiac complications should be performed immediately during PA\u2010induced metabolic crises.<\/jats:p>","DOI":"10.1007\/s10545-009-1029-8","type":"journal-article","created":{"date-parts":[[2009,5,4]],"date-time":"2009-05-04T18:53:29Z","timestamp":1241463209000},"page":"37-40","source":"Crossref","is-referenced-by-count":7,"title":["Extracorporeal membrane oxygenation in a patient with propionic acidaemia: a therapeutic option for cardiac failure"],"prefix":"10.1002","volume":"32","author":[{"given":"K.","family":"Mizuguchi","sequence":"first","affiliation":[{"name":"Division of Child Neurology, National Center of Child Health and Development Okura 2\u201310\u20131 Setagaya\u2010ku Tokyo 157\u20138504 Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"Hoshino","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, National Center of Child Health and Development Okura 2\u201310\u20131 Setagaya\u2010ku Tokyo 157\u20138504 Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.","family":"Nagasawa","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, National Center of Child Health and Development Okura 2\u201310\u20131 Setagaya\u2010ku Tokyo 157\u20138504 Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Kubota","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, National Center of Child Health and Development Okura 2\u201310\u20131 Setagaya\u2010ku Tokyo 157\u20138504 Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2009,5,4]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2006.11.043"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.c.30090"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2005.04.004"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01955907"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(96)70162-2"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/0885-4505(91)90027-I"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.1177\/000992280404300908"},{"key":"e_1_2_4_9_1","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20060221"},{"key":"e_1_2_4_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/0887-8994(92)90085-D"},{"key":"e_1_2_4_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01953939"},{"key":"e_1_2_4_12_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2000.108391"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-009-1029-8","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1007\/s10545-009-1029-8","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-009-1029-8","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T03:19:04Z","timestamp":1694747944000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-009-1029-8"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,5,4]]},"references-count":11,"journal-issue":{"issue":"S1","published-print":{"date-parts":[[2009,12]]}},"alternative-id":["10.1007\/s10545-009-1029-8"],"URL":"https:\/\/doi.org\/10.1007\/s10545-009-1029-8","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,5,4]]}}}