KIMSHEALTH successfully performed a liver transplant on a two-and-a-half-year-old child from Kollam, diagnosed with Propionic Acidemia, a rare genetic metabolic disorder caused by the absence of an essential enzyme needed to break down certain proteins and fats in the body. Propionic Acidemia affects only 1 in 100,000 people worldwide.
The child was brought to KIMSHEALTH after suffering multiple episodes of life-threatening drowsiness and poor responsiveness. As the child’s condition demanded intensive care, he was immediately admitted to the Liver ICU. The disorder's toxic accumulation had also caused neuro developmental delay in the child. Since birth, the child had been admitted more than 15 times with life-threatening crisis episodes in the Pediatric ICU and was saved multiple times with ventilator support.
Due to the recurrent metabolic crises threatening the life of the patient, the Pediatric Inborn Errors of Metabolism (IEM) team recommended a liver transplant, a viable long-term solution. At this juncture, the child’s mother stepped forward to donate a portion of her liver. Following the eight-hour-long surgery, the child made steady progress as the transplanted liver replenished the missing enzyme activity, protecting him from further metabolic and neurological damage. This signifies the state’s first reported liver transplant for Propionic Acidemia.
Propionic Acidemia is a rare genetic disorder in which the body is unable to break down certain proteins and fats, leading to toxic acid build up. As the liver is vital to this metabolic pathway, a healthy liver transplant can restore the deficient enzyme activity, said Dr Shiraz Ahmad Rather, Consultant and Head of Multivisceral Transplant, Hepatobiliary, Pancreatic & Liver Transplant Surgery, who led the transplant.
Dr. Shabeerali T. U, Chief Coordinator and Senior Consultant, Hepatobiliary, Pancreatic and Liver Transplant Surgery; Dr. Anu K. Vasu, Associate Consultant, Department of Pediatric Gastroenterology, Hepatology and Liver Transplant; Dr. Madhu Sasidharan, Co-ordinator and Senior Consultant, Department of Gastroenterology; Dr. Varghese Yeldho, Consultant, Department of Hepatobiliary, Pancreatic and Liver Transplant surgery; Dr. Hashir A, Consultant, Department of Anaesthesiology; Dr. Prijith R.S, Associate Consultant, Department of Transplant Anaesthesia & Critical Care, Hepatobiliary, Pancreatic & Liver Transplant Surgery; Dr. Abhijith Uthaman, Department of Transplant Anaesthesia & Critical Care; and Dr. Manoj K S, Senior Consultant, Imaging & Interventional Radiology, were also part of the treatment.