Source : http://epaperbeta.timesofindia.com/Article.aspx?eid=31806&articlexml=COU...

The high court on Monday directed the staterun Indira Gandhi Institute of Child Health (IGICH), Bengaluru, to provide free treatment to patients of Lysoso mal Storage Disorders (LSD), a group of 50odd genetic diseases caused by de ficiency of enzymes.

A division bench headed by acting Chief Justice Subhro Kamal Mukherjee gave the direction while hearing a PIL filed by Lysosomal Stor age Disorders Society of In dia. The court was informed that only seven of these dis orders can be treated in the form of enzyme replacement therapy (ERT), which is expensive. The petitioner said applications of 34 patients for treatment are pending with the authorities. This includes 18 patients of Gaucher's, 13 of mucopolysaccharidoses (MPS) and a patient each of Pompe, glycogen storage disease (GSD) and mucolipidosis.

Besides, the authorities have failed to establish a special unit at IGICH even a year after making budgetary allocation in February 2014, they alleged.

Since children are involved, we direct the medical authorities to provide free treatment to those suffering from Gaucher's, Pompe, Fabry and four types of MPS, the court said.

What are Lysosomal Storage Disorders

Lysosomal Storage Disorders (LSD) are genetically inherited diseases caused by deficiency of vital enzymes.

They occur due to defects in lysosomes, which are sacs of enzymes within cells that digest large molecules and pass on fragments to other parts of the cells for recycling.

Only seven -Gaucher's, Pompe, Fabry and four types of MPS (Hurler syndrome, Hunter syndrome, Morquio syndrome and Maroteaux-Lamy syndrome) -have definitive treatment in the form of enzyme replacement therapy.Symptoms: Developmental delay, movement disorders, seizures, dementia, deafness, blindness, enlarged livers, enlarged spleens, pulmonary and cardiac problems and abnormal growth of bones. LSD patients also show clinical symptoms owing to death of cells, resulting in early fatality o slow, painful death besides life threatening complications.