After losing 5 children to propionic acid, a liver disease, Omani parents save their sixth child in India

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New Delhi: Eleven-month-old boy from Oman suffering from propionic acid, a metabolic disease of the liver that causes blood poisoning, was treated in India after four successful operations.

Qusai’s parents had previously lost five children with the same disease and needed a special type of liver transplant.

Over a period of two months, the child underwent four operations that included a liver transplant, mesh placement, mesh replacement, and mesh removal.

Dr. Vikram Kumar, Senior Advisor, Pediatric Hepatology, Center for Liver and Biliary Science (CLBS) told ANI, “We performed the liver transplant in October. After the transplant, we encountered some problems, including finding that the liver was compressed because the liver was the size of a three year old child. We performed another operation to set a net. The child later underwent the third operation to remove the mesh. “

“Some of the drugs are extremely expensive and not available in India and had to be procured from Oman,” said the doctor.

Treating the metabolic disorder was one of the major challenges doctors faced in treating the 11-month-old child.

The metabolic liver disease propionic acidemia is a type of genetic problem that results from a lack of enzymes. Here the body cannot process certain parts of proteins and lipids (fats) properly.

This condition is classified as an organic acid disorder, which leads to the abnormal accumulation of certain acids known as organic acids.

These toxic substances cause serious neurological problems such as loss of consciousness, seizures, developmental delay.

PA is treated by severely restricting the dietary intake of BCAA. This is achieved by giving BCAA-deficient formulas.

Propionic acidemia is rarely reported in India.