İSMAİL DEMİREL, ESEF BOLAT, AYSUN YILDIZ ALTUN, Erkan ATICI, TUGAY TARTAR

PROGRESİF FAMİLYAL İNTRAHEPATİK KOLESTAZ TİP I’DE (BYLER HASTALIĞI) ANESTEZİ YÖNETİMİ

Progresif familyal intrahepatik kolestaz Tip I (PFIC) ya da Byler hastalığı metabolik ve genetik orjinli intrahepatik kolestazınen yaygın formlarından birisidir. Otozomal resesif geçişli bir hastalık olup yaşamın ilk 6 ayında ortaya çıkar ve intrahepatikkolestaz ve ilerleyici fibrozis ile siroz gelişir. Olgular genellikle adölesan çağ öncesinde karaciğer yetmezliği sebebiyle hayatlarınıkaybederler. Olgumuz taşlı kolesistit nedeniyle genel anestezi altında laparoskopik kolesistektomi planlanan, 1.5 yaşında Bylerhastalığı teşhisi almış medikal tedaviyle izlenen 8 yaşındaki bir kız çocuğudur. Bu olguda karaciğer fonksiyonlarının bozulmasındankaçınmak için hepatotoksik etkileri olmayan ya da minimal olan anestezik ajanların, Byler hastalığı olan bir hastanın anesteziközellikleri tanımlanmıştır

ANESTHETIC MANAGEMENT IN PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS TYPE I (BYLER DISEASE)

Progressive familial intrahepatic cholestasis (PFIC) or Byler disease is one of the most common forms of intrahepatic cholestasisof metabolic and genetic origin. It's an autosomal recessive disease and appears in the first 6 months of life with intrahepaticcholestasis and progressive fibrosis resulting with cirrhosis. Patients usually die because of hepatic deficiency before adolescantperiod. In this study we would like to report about an eight year old girl diagnosed with Byler disease since 1,5 year of age plannedfor laparoscopic cholecystectomy due to calculous cholecystitis under general anasthesia. This case report describes the anestheticfeatures of a patient with Byler's disease in which anesthetic agents with no or minimal hepatotoxic effect should be used to avoiddeterioration of liver function

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