CANAVAN HASTALIĞINDA ANESTEZİ YÖNETİMİ: İKİ OLGU SUNUMU
Canavan hastalığı, aspartoaçilaz enzim eksikliği ile karakterize, otozomal resesif kalıtılan ilerleyici bir lökodisitrofidir. Canavanhastalığında makrosefali, kas güçsüzlüğü, yutma bozukluğu, gastroözefageal reflü ve epilepsi görülebilecek bulgulardandır. Tedavisisemptomatiktir. Bu yazıda Canavan hastalığı olan iki olguda uyguladığımız anestezi deneyiminin tartışılması amaçlanmıştır
ANESTHESIA MANAGEMENT IN CANAVAN DISEASE: TWO CASE REPORTS
Canavan disease is an autosomal recessive, progressive leucodistrophy characterized by aspartoacylase enzyme deficiency.Macrocephaly, muscle weakness, swallowing disorder, gastroesophageal reflux and epilepsy can be seen in Canavan disease.Treatment is symptomatic. In this article we aimed to discuss the anesthesia experience that we have performed in two Canavandisease cases
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