Kolestaz ve Kaşıntı: Progresif Familyal İntrahepatik Kolestaz?

Yıl 2016, Cilt: 14 Sayı: 2, 88 - 95, 01.09.2016

Gökhan Tümgör

https://doi.org/10.4274/jcp.05706

Öz

Progresif familyal intrahepatik kolestaz PFİK , çocukluk çağı kolestazları içinde önemli bir yer tutmaktadır. Bazı karaciğer nakil merkezlerinde en sık karşılaşılan ikinci karaciğer nakil endikasyonudur. Tüm kolestazlı ve karaciğer nakli yapılan olguların yaklaşık %10-15’ini oluşturmaktadır. PFİK, otozomal resesif geçişlidir ve hepatosellüler kaynaklı kolestaza yol açmaktadır. Süt çocukluğundan erişkin döneme kadar olan dönemde karaciğer yetmezliğine yol açarak ölüme neden olabilmektedir. PFİK’nin üç tipi vardır ve tüm tiplerde en sık gözlenen klinik bulgular kaşıntı, sarılık, büyüme geriliği, hepatomegali, splenomegalidir. PFİK’nin üç tipinin de prognozu farklıdır. PFİK’nin komplikasyonları portal hipertansiyon, karaciğer yetmezliği, siroz, hepatosellüler karsinom ve ekstrahepatik bulgulardır. Olguların hayat kalitesini yükseltecek medikal ve cerrahi tedaviler uygulanmakla birlikte çoğu olguya erken dönemde karaciğer nakli gerekmektedir

Anahtar Kelimeler

Çocukluk çağı, kolestaz, kaşıntı

Cholestasis and Pruritus: Progressive Familial Intrahepatic Cholestasis?

Öz

Progressive familial intrahepatic cholestasis PFIC has a significant place among the childhood cholestasis. It is the second most frequent liver transplant indication met in some liver transplant institutions. It constitutes approximately 10-15% of all cases of cholestasis and liver transplant. PFIC is an autosomal recessively inherited disorder and causes hepatocellular-originated cholestasis. In the period from infancy to adulthood it may cause death due to liver failure. PFIC has three types and the most frequently observed clinical symptoms of all three types are; pruritus, hepatitis, growth retardation, hepatomegaly, and splenomegaly. The prognosis of these three types of PFIC is different. The complications of PFIC are; portal hypertension, liver failure, cirrhosis, hepatocellular carcinoma, and extrahepatic symptoms. Although medical and surgical treatments are applied in order to improve the patients’ life quality, most of the cases require liver transplantation in the early stage

Anahtar Kelimeler

Childhood, cholestasis, pruritus

Kaynakça

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