A disorder caused by mutations in the LARS gene is now referred to as ‘Infantile Liver Failure Syndrome type I’ and was discovered just recently here in Dublin and we coordinate the care for a number of patients with this condition. The LARS gene is responsible for an enzyme in a step in the body’s ability to make protein and this process is faulty in these children. Children with this condition can become very ill very quickly, especially in the first couple of years of life. Some (but not all) may suffer from acute liver failure and seizures, particularly when they have a fever or viral illness. We are now studying it further to try to understand the best ways of preventing severe illness and managing the complications. Further patients have also now been identified in America and Europe.
