Expanding etiology of progressive familial intrahepatic cholestasis

  title={Expanding etiology of progressive familial intrahepatic cholestasis},
  author={Sarah A F Henkel and Judy H. Squires and M. Ayers and A. Ganoza and Patrick Mckiernan and J. Squires},
  journal={World Journal of Hepatology},
  pages={450 - 463}
  • Sarah A F Henkel, Judy H. Squires, +3 authors J. Squires
  • Published 2019
  • Medicine
  • World Journal of Hepatology
  • BACKGROUND Progressive familial intrahepatic cholestasis (PFIC) refers to a disparate group of autosomal recessive disorders that are linked by the inability to appropriately form and excrete bile from hepatocytes, resulting in a hepatocellular form of cholestasis. While the diagnosis of such disorders had historically been based on pattern recognition of unremitting cholestasis without other identified molecular or anatomic cause, recent scientific advancements have uncovered multiple specific… CONTINUE READING
    14 Citations
    Phenotypic and Molecular Characteristics of Children with Progressive Familial Intrahepatic Cholestasis in South China
    • PDF
    New paradigms of USP53 disease: normal GGT cholestasis, BRIC, cholangiopathy, and responsiveness to rifampicin
    • Highly Influenced
    Genetic risk factors in the development of idiosyncratic drug-induced liver injury.
    Cholestasis in the Premature Infant.
    Obeticholic acid-a new therapy in PBC and NASH.
    • 3


    Progressive familial intrahepatic cholestasis.
    • 222
    • PDF
    New Insights in Genetic Cholestasis: From Molecular Mechanisms to Clinical Implications
    • 21
    • PDF
    Histologic Pathology of the Liver in Progressive Familial Intrahepatic Cholestasis
    • 111
    Clinical Variability After Partial External Biliary Diversion in Familial Intrahepatic Cholestasis 1 Deficiency
    • 6
    • PDF