PFIC3 Liver Transplant: An 8-Year-Old, Her Father and a New Liver

The case: eighteen months of worsening jaundice

For about a year and a half before she reached Kolkata, the girl had been getting steadily sicker. Her parents saw her jaundice deepen, her abdomen swell, and her appetite fade. She itched constantly, vomited often and had stopped growing as she should. Fluid kept building up in her abdomen (ascites) and had to be drained again and again at local hospitals.

Tests at Narayana RN Tagore Hospital, Mukundapur, showed end-stage liver disease caused by progressive familial intrahepatic cholestasis type 3 (PFIC3). Her bilirubin had climbed to about 20 mg/dL — many times the normal level — and her blood was not clotting properly, a sign that the liver could no longer make the proteins the body needs. At this stage medicines could not reverse the damage. The team advised a liver transplant.

What is PFIC3?

Progressive familial intrahepatic cholestasis (PFIC) is a group of rare genetic diseases, affecting roughly 1 in 100,000 people, in which the liver cannot move bile out of its cells properly. Bile builds up inside the liver, causing jaundice and intense itching, and over years it scars the liver into cirrhosis.

In PFIC3 the faulty gene is ABCB4, which makes a transporter (MDR3) that adds a protective fat to bile. Without it, bile becomes harsh on the small bile ducts and damages them. A child inherits one faulty copy from each parent; the parents are usually healthy carriers. Unlike some other PFIC types, PFIC3 typically shows a raised GGT on blood tests, which helps doctors tell the types apart. Some children respond for a time to a medicine called ursodeoxycholic acid, but many progress to liver failure in childhood or adolescence.

• Jaundice that does not go away, or keeps coming back
• Severe itching that disturbs sleep
• A swollen abdomen, often from fluid or an enlarged spleen
• Dark urine and pale stools
• Poor feeding, vomiting and failure to grow
• Easy bruising or bleeding

Diagnosis rests on blood tests, an ultrasound, sometimes a liver biopsy, and genetic testing, which confirms the type. PFIC sits alongside other autoimmune and genetic liver diseases and is one of the commonest reasons, after biliary atresia, that children need a liver transplant.

Why a transplant, and why a living donor

Once PFIC3 has caused liver failure, no medicine can restore the liver. A transplant replaces the diseased organ with a healthy one that makes the MDR3 transporter normally, so the underlying problem does not come back in the new liver. That is what the team meant when they said her disease had been "treated at its root".

Waiting for a deceased donor liver can take months, and a child with a bilirubin of 20 and failing clotting may not have months. In a living donor liver transplant, part of a healthy relative's liver is removed and transplanted; the donor's remaining liver and the transplanted portion both grow back to near-normal size within weeks. For children, a parent is often the natural donor — and here her father came forward at once.

Before any donation, the donor goes through a full evaluation: blood group, liver function, scans of the liver's size and blood vessels, heart and lung checks and a psychological assessment. Because a parent of a child with PFIC3 is usually a carrier of one faulty gene copy, the team also looks carefully at the donor's own liver health. He was confirmed as a suitable donor. Read more about who can be a liver donor and donor surgery.

The surgery: about 14 hours

On 28 September 2026 two operations ran in step. In one theatre, surgeons removed the left lobe of the father's liver — 410 grams. In the other, the team removed the girl's failing liver and implanted her father's graft, joining its artery, veins and bile duct to hers. The whole procedure took about 14 hours.

Paediatric transplants carry particular challenges. The blood vessels and bile ducts being joined are only a few millimetres across, so every stitch has to be exact. This child was also gravely ill going in, with fluid in the abdomen and a clotting abnormality that raised the risk of bleeding. As the transplant anaesthesia lead put it to Medical Dialogues, children are not small adults — the team had to bring a very sick child safely through a long operation while keeping her father, a healthy donor, completely safe.

1. 1

   Donor operation

   The left lobe of the father's liver is removed and prepared, with its vessels kept intact for joining.

2. 2

   Recipient operation

   The child's diseased liver is removed while blood loss and clotting are carefully managed.

3. 3

   Implantation

   The graft's veins, artery and bile duct are joined to the child's — the most delicate part in a small child.

4. 4

   Intensive care

   Both move to intensive care, where the new liver's function, blood flow and early signs of rejection are watched closely.

Recovery: home on day 18

The girl was breathing on her own, off the ventilator, within a day of surgery. She recovered in the liver ICU, started her immunosuppressive (anti-rejection) medicines, and was discharged in stable condition on the 18th day after the transplant. Follow-up tests have shown normal liver function.

Her father went home 10 days after donating and has returned to his normal life and his work as a police officer. After an initial period of protective care, she is expected to go back to school and grow like other children her age. She will need lifelong follow-up and medicines to protect her new liver — see recovery after a liver transplant and life after a liver transplant for what this involves.

The transplant team

A paediatric transplant is never one person's work. This one was carried out by a multidisciplinary team of transplant surgeons, hepatologists, paediatricians, intensivists and transplant anaesthetists at Narayana RN Tagore Hospital, Mukundapur. As reported, the team included Dr Sanjay Goja, Dr Bappaditya Har, Dr Anupam Purkayastha (transplant anaesthesia), Dr Sandip Pal, Dr Rahul Roy, Dr Hirakdyuti Mondal, Dr Jiban Basak, Dr Indranil Roychowdhury and the paediatric ICU team.

Dr Har, who trained in paediatric liver transplantation at NCCHD in Tokyo, has said the aim is to make paediatric liver transplantation more widely available across eastern India, so that children who need a transplant do not have to travel far from home to get one.

If your child has PFIC or long-standing jaundice

Many families reach a transplant centre late, after months of repeated admissions and fluid drainage. Earlier referral gives the team time to assess the child, evaluate a family donor and plan the operation before the child becomes critically ill. Ask your child's doctor about referral to a transplant team if your child has:

• Jaundice lasting more than a few weeks, or a rising bilirubin
• Itching that medicines no longer control
• Fluid in the abdomen that keeps coming back
• Poor growth despite good feeding
• A diagnosis of PFIC, biliary atresia or another genetic liver disease