全身麻酔下に智歯抜歯を行ったファブリー病患者の1例

抄録

Fabry disease is a congenital disorder of glycolipid metabolism caused by a reduction or depletion in activity of α-galactosidase A, an intercellular lysosomal enzyme. In this disease, accumulation of glycosphingolipid is observed in the heart, kidneys, blood vessels, skin, cornea, and nerves, which leads to multiple organ dysfunction. We report a case of a patient with typical Fabry disease who underwent extraction of bilateral lower third molars. The patient was a male in his thirties who requested that the operation be performed under general anesthesia. Preoperative assessment revealed mild renal dysfunction without other major problems, and careful and safe perioperative management was performed. A thorough preoperative investigation for patient care around the operation is strongly recommended to prevent cardiovascular, renal and cerebrovascular complications in patients suffering from Fabry disease.