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- An inherited disorder in which copper accumulates in the body and damages the liver – can cause liver failure
- 8-year-old Anishka was in a coma on admission at Apollo
- Mortality rate is high in children who develop coma due to Wilson disease
India
healthysoch
New Delhi, April 19, 2022 :
8 year old girl from Bihar, underwent a lifesaving liver transplant procedure at Indraprastha Apollo Hospitals after being diagnosed with rare Wilson’s disease- a genetic disorder in which excess copper builds up in the body. Treatment includes lifelong medication to bind copper. However, some children can develop coma due to liver failure. Mortality rate is 100 % in children who develop coma due to Wilson disease.
8 year old Anshika was admitted to Indraprastha Apollo Hospitals on 25th March evening in a state of coma. Previously she was being treated at another hospital where her condition had deteriorated.
At Apollo she was admitted under the supervision of Dr Anupam Sibal, Group Medical Director, Senior Pediatric Gastroenterologist, Apollo Hospitals Group along with his team including Dr Smita Malhotra and Dr Karunesh Kumar (Consultants, Paediatric Gastroenterology) and Dr Neerav Goyal, Senior Consultant, Liver Transplant, Indraprastha Apollo Hospitals along with Dr Arun V, Dr Varun M, Dr Pradeep Kumar (Consultants, Liver Transplant) and Dr Raman R (Anaesthetist).
The child was immediately put on a ventilator and advised a liver transplant, she was parallely put on dialysis and plasma exchange therapy to remove copper and buy time for a pre-transplant evaluation.
Due to non-availability of the same or compatible blood group donor, an ABO-incompatible emergency live donor liver transplant was planned, with the child’s mother as the donor. 31hours after admission, on 27th March, a liver transplant was started. Two days later she was removed from a ventilator, and she then regained consciousness
Speaking about this life saving transplantation, Dr Anupam Sibal, Group Medical Director and Senior Pediatric Gastroenterologist, Apollo Hospitals Groupsaid, “The complexity of the case required us to support the child on ventilator immediately post her admission. Both her pre-surgical management and the seamless administration of the liver transplant surgery (with her donor being ABO Compatible) were a challenge. This was an exceptionally rare case where a living related liver transplant was performed within 31 hours of admission. Of the 432 pediatric liver transplants performed at the Apollo Liver Transplant Program, this is the first case of an emergency ABO incompatible living related liver transplant.”
Dr Neerav Goyal, Senior Consultant, Liver Transplant, Indraprastha Apollo Hospitals, said, “A rare complication, in a case of Wilson disease children typically manifest deteriorating symptoms and if not treated within a specific duration can cause life threatening problems. This child had come to us with acute liver failure, swelling in the brain due to ammonia content, was unconscious and in a state of coma. At Apollo, we immediately counselled the family for a liver transplant. Her recovery has been uneventful and the child was discharged on 12th April, post-operative day 17.”
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