Progressive Familial Intrahepatic Cholestasis

Also known as: PFIC.

What is progressive familial intrahepatic cholestasis?

Progressive familial intrahepatic cholestasis is one of many types of progressive liver diseases that ultimately lead to liver failure. The unique thing about progressive familial intrahepatic cholestasis is that, liver cells are less able to secrete a digestive fluid called bile. This buildup ultimately leads to many problems. It frequently begins in young infants.

What causes progressive familial intrahepatic cholestasis?

The disease is caused by genetic mutations. It’s also a hereditary condition that can be passed down from parents to their children.

What are the symptoms of progressive familial intrahepatic cholestasis?

Symptoms of progressive familial intrahepatic cholestasis can include jaundice (yellowing of the skin and eyes), itchy skin, fatigue, irritability, poor feeding, nausea, vomiting and failure to thrive.

What are progressive familial intrahepatic cholestasis care options?

Medications and supplements can help with the symptoms, but most children with progressive familial intrahepatic cholestasis will require surgery. Two procedures called partial cutaneous biliary diversion (PEBD) shunts the bile to a bag outside the skin or Partial internal biliary drainage (PIBD) is another technique that  connects  the gallbladder to colon. If unsuccessful a liver transplant is required.

Reviewed by: Shifra A Koyfman, MD

This page was last updated on: 5/30/2018 8:01:35 PM

From the Newsdesk

Pediatric Neurosurgeon and Chief of Surgery for Nicklaus Children’s Passes Away
05/24/2018 — The medical staff, employees and volunteers of Nicklaus Children’s Hospital mourn the passing of our esteemed Dr. Sanjiv Bhatia, a longstanding leader and dedicated champion for children with complex medical conditions and their families.
Medical Mission to Algeria Helps Children in Need of Spinal Surgeries
03/06/2018 — A group of children in Algeria who underwent complex surgeries as part of a 2016 U.S.-sponsored medical mission have many reasons to celebrate, and can do so with better movement of their limbs.


At just 17 years old, Tonaly was diagnosed with ulcerative colitis, a condition that kept her in the hospital for almost three months. Thanks to the treatment she received at Nicklaus Children’s, Tonaly no longer experiences the strong pains she once did, and with the help of Lisa, she developed the strength to face her condition head-on.