A city hospital here has successfully carried out an auxiliary liver transplant on a 14-yr-old patient, suffering from Crigler Najjar Syndrome (CNS) Type 1, a rare genetic disorder inherited by children from parents. The auxiliary liver transplant, also known as auxiliary partial orthotopic liver transplant(APOLT) of the young boy named Yash, was performed by an expert team of doctors at the Global Hospitals, Mumbai.
Patients suffering from CNS are unable to produce an enzyme called, UDP Glucuronyl Transferase, which helps in detoxification of bilirubin produced by the breakdown of RBCs. Bilirubin is a toxic metabolite, which can get accumulated in body and lead to jaundice and cause damage to the brain, muscles, nerves and loss of life in extreme conditions.
Till date 12 Auxiliary Liver Transplants have been done in the country; out of these 11 have been done in Southern India and Yash’s was the first one in Central, Western and Northern India. Light treatment (photo therapy), blood transfusion and liver transplant are used for CNS treatment. Photo therapy, however, has a limitation beyond an age, as human skin thickens,its efficacy comes down.
Auxiliary liver transplant is an effective modality for treating CNS. The surgery entails attaching a part of donor liver to the failing liver in the recipient, where it supports the patient, clears toxins, and prevents brain injury during recovery. Prof. Mohamed Rela, Group Director, Institute of Liver Diseases, HPB Surgery andTransplantation, Global Hospital said, “Auxiliary liver transplant is a complex and technically demanding procedure, unlike a normal liver transplant. It may be possible for an Auxiliary transplant patient to stop immunosuppression medicines that are generally given life-long after transplant and avoid long term consequences.”
Auxiliary partial orthotopic liver transplant (APOLT) is technically more challenging because the blood flow preferentially goes towards native liver and the small sized artery for anastomosis, posses’ high risk of technical complications and very few centres in the country conduct it. Dr. Ravi Mohanka – Chief Surgeon and HOD, HPB Surgery and Liver Transplantation Global Hospitals, Mumbai said, “Only 1-2% of liver tissue is sufficient to provide the required enzyme for bilirubin conjugation and auxiliary liver transplant is the best option for this. With the successful transplant, the patient has recovered quickly and has potential for gene therapy in the future.”
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