Monday, December 4, 2023

Omani parents save their sixth child in India after losing five children to Propionic Acidemia

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Eleven-month-old boy from Oman, suffering from Propionic Acidemia, a metabolic liver disorder leading to toxicity in the blood, was treated in India after four successful surgeries. The parents of Qusai earlier had lost five children with the same disease and required a special kind of liver transplant. In a time span of two months, the child underwent four surgeries that include a liver transplant, mesh placement, mesh replacement and mesh removal. The Liver transplant has been done under Dr Subhash Gupta, Max Hospital, Saket.

Dr Vikram Kumar, Principal Consultant, Pediatric Hepatology, CLBS, Max, Saket told ANI, “We did the liver transplant in October. After the transplant we came across a few issues, one among these is we found that the liver was getting compressed because the liver size was of a three-year-old child. We performed another surgery to put in a mesh. Later, the child underwent the third surgery to remove the mesh.” “Some of the medicines are extremely costly and are not available in India and had to get these medicines from Oman,” said the doctor.

Managing the metabolic disorder was one of the major challenges the doctors faced while treating the 11-month old.
Propionic acidemia metabolic liver disorder is a kind of genetic problem that happens due to a deficiency in enzymes. Here, the body is unable to process certain parts of proteins and lipids (fats) properly.

This condition is classified as an organic acid disorder which leads to an abnormal buildup of particular acids known as organic acids. These toxic substances cause severe neurological issues like unconsciousness, seizure, developmental delay. PA is treated by strictly restricting the intake of BCAA in the diet. This is achieved by giving BCAA deficient formulas. Propionic acidemia is rarely reported in India.

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