الفهرس يوجد فقط 14 صفحة متاحة للعرض العام
 |  | from | 92 |  |  |
| | | from | 92 | | |
المستخلص
Background: Progressive familial intrahepatic cholestasis (PFIC) refers to heterogeneous group of autosomal recessive liver disorders of childhood in which cholestasis of hepatocellular origin often presents in the neonatal period or first year of life and leads to death from liver failure at ages usually ranging from infancy to adolescence . PFIC represents 10 to 15% of causes of cholestasis in children and 10 to 15% of liver transplantation indications in children. PFIC1 and PFIC2 represent 2/3 of cases of PFIC, and PFIC3 1/3 of cases . Methods: Screening of exons 13,14 and 15 of 30 patients with PFCI3 by direct gene sequancing.Detailed clinical and laboratoty history of the patients were carried out,to properly diagnose patients with PFIC3