title: Paroxysmal nocturnal haemoglobinuria. Peri-operative management of a patient with Budd-Chiari syndrome.
abstract: The peri-operative management of a patient with paroxysmal nocturnal haemoglobinuria and associated hypoplastic anaemia, who underwent successful elective surgery for the complication of Budd-Chiari syndrome, is described. Anaesthesia in patients with paroxysmal nocturnal haemoglobinuria should be based on drugs and materials least likely to cause complement activation. The reasons for use of a technique based on benzodiazepines, opioids, isoflurane and the avoidance of nitrous oxide are discussed.
mesh_terms: D000328:Adult; D000743:Anemia, Hemolytic / Q000150:complications*; D000768:Anesthesia, General; D006502:Budd-Chiari Syndrome / Q000150:complications* / Q000601:surgery*; D002908:Chronic Disease; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications*; D006801:Humans; D008297:Male