{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,26]],"date-time":"2025-12-26T22:26:18Z","timestamp":1766787978999},"reference-count":7,"publisher":"American Dental Society of Anesthesiology (ADSA)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020]]},"abstract":"Cardiofaciocutaneous (CFC) syndrome is a rare condition characterized by congenital heart disease, craniofacial dysmorphology, and dermatological abnormalities. CFC syndrome is one of the RASopathies, a family of syndromes that also includes Noonan and Costello syndromes, all with underlying gene mutations involving the Ras\/mitogen-activated protein kinase pathways. Important considerations for anesthesiologists caring for these patients include the need to evaluate for possible cardiac defects, anticipating and planning for potentially difficult airway management, and the consideration of potential weakness of the respiratory muscles. Musculoskeletal abnormalities, such as muscle weakness and decreased muscle mass, are observed in all RASopathies, but are particularly prominent in CFC syndrome. In patients with CFC syndrome who experience respiratory muscle weakness, the use of desflurane and remifentanil may aid in a faster recovery and effectively help reduce the risk of respiratory complications, such as respiratory depression, following general anesthesia because of their rapid metabolism or elimination.<\/jats:p>","DOI":"10.2344\/anpr-67-01-07","type":"journal-article","created":{"date-parts":[[2020,3,19]],"date-time":"2020-03-19T18:40:56Z","timestamp":1584643256000},"page":"45-47","source":"Crossref","is-referenced-by-count":2,"title":["Anesthetic Management of a Pediatric Patient With Cardiofaciocutaneous Syndrome"],"prefix":"10.2344","volume":"67","author":[{"given":"Yoshiki","family":"Shionoya","sequence":"first","affiliation":[{"name":"Department of Dental Anesthesia, The Nippon Dental University Hospital, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maki","family":"Yamamoto","sequence":"additional","affiliation":[{"name":"Department of Dental Anesthesia, The Nippon Dental University Hospital, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katsuhisa","family":"Sunada","sequence":"additional","affiliation":[{"name":"Department of Dental Anesthesiology, The Nippon Dental University School of Life Dentistry, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kiminari","family":"Nakamura","sequence":"additional","affiliation":[{"name":"Department of Dental Anesthesia, The Nippon Dental University Hospital, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1191","reference":[{"key":"i0003-3006-67-1-45-b01","series-title":"Pediatrics","first-page":"1149","article-title":"Cardio-facio-cutaneous syndrome: clinical features, diagnosis, and management guidelines","volume":"134","author":"Pierpont \n MEM,","year":"2014","unstructured":"Pierpont \n MEM, \n \n \n Magoulas \n PL, \n \n \n Adi \n S, \n \n et al. \n Cardio-facio-cutaneous syndrome: clinical features, diagnosis, and management guidelines. Pediatrics. 2014;134:1149\u20131162."},{"key":"i0003-3006-67-1-45-b02","series-title":"Am J Med Genet","first-page":"413","article-title":"New multiple congenital anomalies\/mental retardation syndrome with cardio-facio-cutaneous involvement-the CFC syndrome","volume":"25","author":"Reynolds \n JF,","year":"1986","unstructured":"Reynolds \n JF, \n \n \n Neri \n G, \n \n \n Herrmann \n JP, \n \n \n Blumberg \n B, \n \n \n Coldwell \n JG, \n \n \n Miles \n PV, \n \n \n Opitz \n JM. \n \n \n New multiple congenital anomalies\/mental retardation syndrome with cardio-facio-cutaneous involvement-the CFC syndrome. Am J Med Genet. 1986;25:413\u2013427."},{"key":"i0003-3006-67-1-45-b03","series-title":"J Med Genet","first-page":"161","article-title":"Noonan-like short stature syndrome with sparse hair","volume":"23","author":"Baraitser \n M,","year":"1986","unstructured":"Baraitser \n M, \n \n \n Patton \n MA. A\n \n \n Noonan-like short stature syndrome with sparse hair. J Med Genet. 1986;23:161\u2013164."},{"key":"i0003-3006-67-1-45-b04","series-title":"Am J Med Genet C Semin Med Genet","first-page":"104","article-title":"Skeletal muscle pathology in Costello and cardio-facio-cutaneous syndromes: developmental consequences of germline Ras\/MAPK activation on myogenesis","volume":"157","author":"Tidyman \n WE,","year":"2011","unstructured":"Tidyman \n WE, \n \n \n Lee \n HS, \n \n \n Rauen \n KA. \n \n \n Skeletal muscle pathology in Costello and cardio-facio-cutaneous syndromes: developmental consequences of germline Ras\/MAPK activation on myogenesis. Am J Med Genet C Semin Med Genet. 2011;157:104\u2013114."},{"key":"i0003-3006-67-1-45-b05","series-title":"Arch Neurol","first-page":"1254","article-title":"Treatment of refractory status epilepticus with inhalational anesthetic agents isoflurane and desflurane","volume":"61","author":"Mirsattari \n SM,","year":"2004","unstructured":"Mirsattari \n SM, \n \n \n Sharpe \n MD, \n \n \n Young \n GB. \n \n \n Treatment of refractory status epilepticus with inhalational anesthetic agents isoflurane and desflurane. Arch Neurol. 2004;61:1254\u20131259."},{"key":"i0003-3006-67-1-45-b06","series-title":"A A Pract","first-page":"95","article-title":"Anesthesia for a pediatric patient with cardiofaciocutaneous syndrome","volume":"4","author":"Lam \n H,","year":"2015","unstructured":"Lam \n H, \n \n \n Dare \n S, \n \n \n Nguyen \n T, \n \n \n Austin \n T. \n \n \n Anesthesia for a pediatric patient with cardiofaciocutaneous syndrome. A A Pract. 2015;4:95\u201396."},{"key":"i0003-3006-67-1-45-b07","series-title":"J Med Genet","first-page":"833","article-title":"The cardiofaciocutaneous syndrome","volume":"43","author":"Roberts \n A,","year":"2006","unstructured":"Roberts \n A, \n \n \n Allanson \n J, \n \n \n Jadico \n SK, \n \n et al. \n The cardiofaciocutaneous syndrome. J Med Genet. 2006;43:833\u2013842."}],"container-title":["Anesthesia Progress"],"original-title":[],"link":[{"URL":"https:\/\/anesthesiaprogress.kglmeridian.com\/view\/journals\/anpr\/67\/1\/article-p45.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/meridian.allenpress.com\/anesthesia-progress\/article-pdf\/67\/1\/45\/2436438\/anpr-67-01-07.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/anesthesiaprogress.kglmeridian.com\/downloadpdf\/journals\/anpr\/67\/1\/article-p45.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/anesthesiaprogress.kglmeridian.com\/downloadpdf\/journals\/anpr\/67\/1\/article-p45.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,10,7]],"date-time":"2024-10-07T12:41:44Z","timestamp":1728304904000},"score":1,"resource":{"primary":{"URL":"https:\/\/anesthesiaprogress.kglmeridian.com\/view\/journals\/anpr\/67\/1\/article-p45.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020]]},"references-count":7,"journal-issue":{"issue":"1"},"URL":"https:\/\/doi.org\/10.2344\/anpr-67-01-07","relation":{},"ISSN":["0003-3006","1878-7177"],"issn-type":[{"type":"print","value":"0003-3006"},{"type":"electronic","value":"1878-7177"}],"subject":[],"published":{"date-parts":[[2020]]}}}