title: Contemporary perioperative management of adult familial dysautonomia (Riley-Day syndrome).
abstract: Familial dysautonomia (Riley-Day syndrome) is a rare multisystem disorder associated with an excess risk of perioperative morbidity and mortality. Because life expectancy is limited, few reports consider the perioperative management of familial dysautonomia in adults with advanced disease and end-organ dysfunction. Here, we report on the management of an adult patient with familial dysautonomia, highlighting recent developments in perioperative technology and pharmacology of special relevance to this challenging population.
authors.affiliation: From the University College London Hospitals, London, United Kingdom.
authors.forename: Andrew
authors.initials: A
authors.lastname: Milne
authors.forename: Wint Yu
authors.initials: WY
authors.lastname: Mon
authors.forename: James
authors.initials: J
authors.lastname: Down
authors.forename: Austin
authors.initials: A
authors.lastname: Obichere
authors.forename: Gareth L
authors.initials: GL
authors.lastname: Ackland
mesh_terms: D000328:Adult; D001794:Blood Pressure / Q000502:physiology; D002302:Cardiac Output / Q000502:physiology; D004402:Dysautonomia, Familial / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008297:Male; D016343:Monitoring, Intraoperative / Q000379:methods; D019990:Perioperative Care / Q000379:methods; D011182:Postoperative Care / Q000379:methods