Navigating the complexities of progressive familial intrahepatic cholestasis-II: Genetic insights, patient outcomes, and nursing implications - A case report.

Abstract excerpt

INTRODUCTION: Progressive Familial Intrahepatic Cholestasis Type 2 (PFIC-2) is a rare autosomal recessive liver disorder caused by mutations in the ABCB11 gene encoding the bile salt export pump (BSEP). The condition is characterised by persistent cholestasis, low γ-glutamyl transferase (GGT) levels, severe pruritus, hepatosplenomegaly, and progressive liver dysfunction. Due to its rarity and clinical complexity,...

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