title: Anaesthetic management of a patient with familial amyloid polyneuropathy of the Portuguese type.
abstract: Although familial amyloid polyneuropathy of the Portuguese type (FAP-PT) was first described in 1952, there is little in the medical literature detailing the anaesthetic management of such patients. FAP-PT is a disease with multiple clinical manifestations which include disturbances of sensibility, progressive paresis starting in the lower extremities, autonomic dysfunction, cardiac conduction disturbances, gastro-intestinal disorders, nephrotic syndrome, sexual and sphincter disorders, extreme emotionalism and apprehension. Several intermingling problems have to be considered in the anaesthetic management of each individual case. In our patient a sinus dysrhythmia resolved after isoflurane and this seems to be a good choice for general anaesthesia in patients with FAP-PT, if they are in an early stage of heart involvement.
authors.affiliation: Department of Anaesthesia, Hospital de S. João, Porto, Portugal.
authors.forename: J
authors.initials: J
authors.lastname: Castro Tavares
authors.forename: L
authors.initials: L
authors.lastname: Maciel
mesh_terms: D000328:Adult; D000686:Amyloidosis / Q000235:genetics* / Q000601:surgery; D000766:Anesthesia, Dental; D000769:Anesthesia, Inhalation; D005260:Female; D006801:Humans; D010523:Peripheral Nervous System Diseases / Q000235:genetics* / Q000601:surgery; D011174:Portugal; D013577:Syndrome; D014081:Tooth Extraction