A four year old boy Rishi suffering from Citrullinaemia was
successfully treated with a complex liver transplant procedure in India. His
mental and physical growth was retarded by Citrullinaemia a condition which is
rare in the world and seen in one only about 60,000 children across the globe. Citrullinaemia
is characterized by a deficiency of enzyme ASS1 in the liver, which metabolises
protein.
Rishi’s Father Shirish donated a portion of liver to perform
the liver transplant. According to Indian law only blood relation can donate
their organ. To stop illegal organ transplant, Indian Government has come up with a
1994 law that criminalizes organ sales. The Human Organs Transplant Act, 1994
has laid down certain rules and regulations that are to be followed while
conducting organ transplant.
“The child was
unable to metabolise proteins ingested in the daily diet. Rishi suffered from
mental slowness, recurrent vomiting, poor feeding and chest and intestinal
infections. For four years, the child survived on a low protein diet with
medicines and imported essential amino acids formulae which cost Rs 30,000 per
month,” Dr A S Soin, chief surgeon at the Medanta Liver Institute in Gurgaon
said.
“After the
transplant, the child is now on a normal diet and mentally sharper than before.
For the first time in his life, after transplant, he drank milk and ate a
chocolate”, said Dr Neelam Mohan, director, paediatric hepatology.
Doctors said that there is
no drug therapy which could be used for this disorder.