Anaesthesiological management of thalassemias

Thalassemias are the most common genetic disorders. Although primarily foun d in the mediterranean countries and in south-east Asia their importance in our region is increasing because of greater mobility and migration. Therefore the probability of treating thalassemic patients is increasing. Thalassemias show a great variety of clinical symptoms, from intauterine de ath to mild, asymptomatic anemias, Clinically most important are severe for ms of beta-thalassemia, Depending on the degree of anaemia a substitution t herapy must be initiated with respect to the severity of surgery. Chronic t ransfusion therapy may significantly influence perioperative anaesthesiolog ical management of thalassemic patients. Heart failure, liver cirrhosis and diabetes mellitus caused by transfusion related hemosiderosis may occur. C ephalofacial deformation may result in endotracheal intubation difficulties .