Kerala doctors successfully performed a liver transplant on a child diagnosed with a rare metabolic disorder.
The two-and-a-half-year-old child, who was diagnosed with Propionic Acidemia, was brought to the attention of experts after the guardians consulted them for poor responsiveness.
As the child’s condition demanded intensive care, he was immediately admitted to the Liver ICU at KIMSHEALTH. The disorder's toxic accumulation had also caused neurodevelopmental delay. As per the hospital, 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.
What is 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 buildup. "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.
The disorder is 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.
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As per the National Organisation for Rare Disorders, symptoms most commonly become apparent during the first weeks of life and may include abnormally diminished muscle tone (hypotonia), poor feeding, vomiting, listlessness (lethargy), dehydration and seizures.
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. The mother of the child stepped forward to donate a portion of her liver and after a gruesome 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, informed KIMSHEALTH.
