Tuğba DOĞU, Hasan Ali ŞAHİN, Mesut ERBAŞ

WİLSON HASTALIĞI OLAN OLGUDA ANESTEZİ YÖNETİMİ

Wilson hastalığı (WH) 13. kromozom üzerindeki ATP7B geni üzerindeki mutasyon ile oluşan otozomal resesif geçişli, bakırınbilier ekskresyonun bozukluğuna neden olan bir hastalıktır. Bu olgu sunumunda Wilson hastalığı olan ve apandisit nedeniyle acilcerrahi prosedür uygulanan bir hastada anestezi deneyimimizi tartışmayı amaçladık. Altı yıl önce Wilson hastalığı teşhisi konmuş,astım ve kardiyomiyopati tanıları mevcut 42 yaşındaki erkek hastaya apandisit nedeniyle acil cerrahi planlandı. Hastanın nörolojikmuayenesi normaldi. Genel anestezi uygulanan hastaya indüksiyonda 2 mg kg-1propofol, 1 µg kg-1fentanil, ve 0.5 mg kg-1roküronyumbromür intravenöz olarak uygulandı. Anestezi idamesinde %50 oksijen ve %50 hava ile %2 sevofluran kullanıldı. Sorunsuz bir şekildeekstübe edilen hastada postoperatif koreiform hareketler ortaya çıktı. Hastanın takibinde koreiform hareketlerinin postoperatif3-6 saat arasında azalarak kaybolduğu gözlendi. Hastanın anesteziden derlenmesinde bir gecikme gözlemlemedik. Sonuç olarak,Wilson hastalığı olgularında karaciğere en az toksik ajanlar tercih edilerek; koruyucu önlemler, perioperatif ve postopereatif sıkıtakip ile sorunsuz bir anestezinin uygulanabileceğini düşünüyoruz

ANESTHESIA MANAGEMENT OF A PATIENT WITH WILSON’S DISEASE

Wilson’s disease (WD) is an autosomal recessive disorder characterized by mutations in the ATP7B gene, which causes the disorderof copper excretion into bile. In this study we aimed to discuss our anesthesia experience in a Wilson's disease patient who underwentemergency surgical procedures for appendicitis. A 42-year-old male WD patient suffering the disease for 6 years with a diagnosisof cardiomyopathy and asthma, was planned an emergency surgery for appendicitis. The patient's neurological examination wasnormal. General anesthesia was preffered and for induction 2 mg kg-1propofol, 1 µg kg-1fentanyl, and 0.5 mg kg-1rocuronium wasintravenously administered. Anesthesia was maintained with 50% oxygen and 50% air and 2% sevoflurane. The patient wasextubated uneventfully, subsequently postoperative choreiform movements occurred. During the postoperative follow-up, the patient'schoreiform movements were observed to disappear and declined by between 3-6 hours. We did not observe a delay in the patient'sanesthesia recovery. Consequently, we think general anesthesia can be uneventually applied in Wilson’s disease patients by usingleast toxic agents to the liver, protective measures and with an attentive follow-up in the perioperative and postoperative periods

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