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Fibrillin\u20101 interacts with transforming growth factor\u2010\u03b2 (<jats:styled-content style=\"fixed-case\">TGF\u2010<\/jats:styled-content>\u03b2), and dysregulated <jats:styled-content style=\"fixed-case\">TGF\u2010<\/jats:styled-content>\u03b2 signaling plays a major role in the development of connective tissue disease and familial aortic aneurysm and dissection, including <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>arfan syndrome. Losartan, an angiotensin <jats:styled-content style=\"fixed-case\">II<\/jats:styled-content> blocker, has the potential to reduce <jats:styled-content style=\"fixed-case\">TGF\u2010<\/jats:styled-content>\u03b2 signaling and is expected to be an additional therapeutic option. Clinical diagnosis is made using the <jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>hent nosology, which requires comprehensive patient assessment and has been proven to work well, but evaluation of some of the diagnostic criteria by a single physician is difficult and time\u2010consuming. A <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>arfan clinic was established at the <jats:styled-content style=\"fixed-case\">U<\/jats:styled-content>niversity of <jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>okyo <jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>ospital in 2005, together with cardiologists, cardiac surgeons, pediatricians, orthopedists, and ophthalmologists in one place, for the purpose of speedy and accurate evaluation and diagnosis of <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>arfan syndrome. In this review, we discuss the recent progress in diagnosis and treatment of <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>arfan syndrome, and the characteristics of Japanese patients with <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>arfan syndrome.<\/jats:p>","DOI":"10.1111\/ped.12423","type":"journal-article","created":{"date-parts":[[2014,6,17]],"date-time":"2014-06-17T00:20:13Z","timestamp":1402964413000},"page":"484-491","source":"Crossref","is-referenced-by-count":7,"title":["Pathophysiology and <scp>J<\/scp>apanese clinical characteristics in <scp>M<\/scp>arfan syndrome"],"prefix":"10.1111","volume":"56","author":[{"given":"Daishi","family":"Fujita","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Medicine University of Tokyo Hospital  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Norifumi","family":"Takeda","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine University of Tokyo Hospital  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasushi","family":"Imai","sequence":"additional","affiliation":[{"name":"Division of Cardiovascular Medicine Jichi Medical University  Tochigi Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryo","family":"Inuzuka","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Tokyo Hospital  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Issei","family":"Komuro","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine University of Tokyo Hospital  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasunobu","family":"Hirata","sequence":"additional","affiliation":[{"name":"Tokyo Teishin Hospital  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,9,24]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.107.693523"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOP.0b013e3283557d4c"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19960424)62:4<417::AID-AJMG15>3.0.CO;2-R"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2009.072785"},{"key":"e_1_2_10_6_1","first-page":"220","article-title":"Un cas de deformation congenitale des quatre membres, plus prononcee aux extremites, caracterisee par l'allongement des os avec un certain degre d'amincissement","volume":"13","author":"Marfan M\u2010B","year":"1896","journal-title":"Bull. 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