Medical evaluation and genetic testing led to the diagnosis of Citrullinemia (Urea Cycle disorder).The team at the Liver Diseases and Transplantation Centre at Kauvery Hospital advised Liver Transplantation as the cure for the condition.

TN hospital performs successful liver transplant on 2-year-oldKauvery Hospital
Product Wednesday, January 12, 2022 - 10:50

Kauvery Hospital Trichy, a unit of Kauvery Group of Hospitals announced the successful treatment of an infant with genetic condition called Citrullinemia (Urea Cycle disorder).

The girl baby named Rafeeda Fathima aged 2 years, was brought to the hospital for a seizure and severe vomiting. Several tests including Endoscopy and CT Scan did not help to make a diagnosis.

Further evaluation and genetic testing led to the diagnosis of Citrullinemia (Urea Cycle disorder), which causes an accumulation of ammonia (hyperammonemia) and other toxic substances.   

The expert medical team at the Liver Diseases and Transplantation Centre at Kauvery Hospital advised Liver Transplantation as the cure for the condition.

The mother of the baby, Mrs. Sabana Parveen, came forward to be the donor.

The transplantation surgery was done by Dr. Elankumaran K, Head – Liver Diseases and Transplantation, Kauvery Group of Hospitals, and his highly experienced team.

A portion from the left side of the mother’s liver was surgically removed, after carefully adjusting for the weight and the size of the baby.

Speaking on the case Dr. Elankumaran, Kauvery Hospital said, “As the baby was weighing only 9 kgs, this was a quite complex situation where many underlying factors were involved. Citrullinemia is a genetic condition the diagnosis of which is usually missed at birth. New-borns are often diagnosed with birth defects but rarely with genetic defects. The onset of this particular genetic disorder can occur randomly in the later stages of life (mutation) without any warning. In this instance the onset of the disease occurred around the time when the baby was 6 months old but was diagnosed only after a year and a half or so. Had it been delayed for some more months, the ammonia levels in the baby’s blood would have increased causing brain damage, and ultimately, a state of coma. The mother of the baby volunteered to be the liver donor.”

The surgery was a success, and the liver started functioning immediately after the transplantation.

Dr. Kumaragurubaran S, Consultant – Hepatology, Kauvery Hospital, Trichy added “Metabolic liver disorders are second most common indication for pediatric liver transplant. Many children with metabolic liver disease remain undiagnosed. What we see is only tip of the iceberg. In the past children with end stage liver disease could rarely receive a liver transplant. Now, with present medical advancements, any child with end stage liver disease has a chance of receiving transplantation. With living donor liver transplant and split liver transplant, death while waiting for a transplant has become nil in pediatric liver transplants. Normal development and growth are possible after liver transplantation”.

Commenting on the success of the surgery, Dr. D. Senguttuvan, Co-founder, Executive Director and HOD – Dept. of Pediatrics, Kauvery Hospital said, “We congratulate Dr. Elankumaran, Dr.Kumaragurubaran and their excellent team for taking on this challenge, in handling the child-patient and the mother-donor with utmost care, and achieving a successful liver transplant. The expertise of the transplant surgeon and team is very important in such a highly complex procedure. We are blessed to have such a highly qualified and dedicated team of medical experts. This situation was dense with display of several emotions; the family was from a humble background and approached us after consulting another private hospital whose cost estimates were unaffordable to the family. We empathized with their situation and guided them to get their funds from Project Office of the Tamil Nadu Chief Minister's Comprehensive Health Insurance Scheme. The state-of-the-art medical technologies available at our facility and the medical expertise did the rest by ensuring that the baby was bought back to good health.

We also got to witness the strong love of the mother for her child; despite many objections from the family, the mother herself volunteered to make the liver donation. The baby also grew fond of the staff and felt at home during her treatment at the hospital. Overall, this was a wholesome experience for the entire medical team.”