Osmania doctors successfully perform liver transplant on 8-month-old infant with rare syndrome

Hyderabad: Liver transplantation for an eight-month-old baby who was diagnosed with an extremely rare genetic syndrome called NISCH was successfully performed by doctors at the Osmania General Hospital here.

NISCH or Neonatal Ichthyosis Scalp alopecia Cholangio Hepatitis syndrome is a rare genetic syndrome. Due to this, the baby had dry scaly skin, no scalp hair, jaundice since birth and water had also formed in his abdomen.

It is an extremely rare syndrome. Till now, only 18 such cases have been reported across the world, out of which only four underwent liver transplantation. Doctors said this baby is the first patient to undergo liver transplantation for NISCH syndrome in India.

The eight-month-old baby was diagnosed with NISCH syndrome while admitted to Niloufer Hospital. However, the baby was referred to Osmania General Hospital for liver transplantation due to progressive liver failure (deep jaundice, Ascites and coagulopathy).

"NISCH is a genetic syndrome common in consanguineous marriage (marriage between relatives). The baby's parents were close relatives. The mother, an Anganwadi worker, had a child before who was born with a similar syndrome and died immediately after birth," Dr. B. Nagendra, the superintendent of Osmania General Hospital, said.

He added, "We initially treated the baby with drugs but he did not respond and so decided to do liver transplantation."

The baby's mother donated a portion of her liver for her child. The baby hails from Jagitial, Telangana.

"We did living donor liver transplantation on 17 June at Osmania General Hospital, Hyderabad, with paediatric support from Niloufer hospital. The operation went on for almost 18 hours," the doctor said.