Pruritus due to progressive familial intrahepatic cholestasis

Pruritus due to progressive familial intrahepatic cholestasis (PFIC) is a severe, debilitating symptom characterized by intractable itching arising from a group of rare, inherited childhood hepatobiliary disorders It primarily affects pediatric patients and stems from genetic mutations that disrupt hepatocellular transport systems responsible for bile acid secretion and formation This impaired canalicular transport causes intrahepatic cholestasis, systemic accumulation of bile acids, and resulta...

Pruritus due to progressive familial intrahepatic cholestasis (PFIC) is a severe, debilitating symptom characterized by intractable itching arising from a group of rare, inherited childhood hepatobiliary disorders It primarily affects pediatric patients and stems from genetic mutations that disrupt hepatocellular transport systems responsible for bile acid secretion and formation

This impaired canalicular transport causes intrahepatic cholestasis, systemic accumulation of bile acids, and resultant activation of pruritogenic sensory pathways The symptom is notoriously refractory to conventional therapy and can lead to skin excoriation, sleep disturbances, impaired quality of life, and eventual liver failure requiring surgical diversion or transplantation

The symptom is notoriously refractory to conventional therapy and can lead to skin excoriation, sleep disturbances, impaired quality of life, and eventual liver failure requiring surgical diversion or transplantation