Recently KMC Hospital witnessed two rare cases of 2 year and 6 year old children suffering from tuberculosis due to genetic defect in immunity. Two year old Shreya (name changed) was brought to the Hospital in a critical condition. She had multiple lymph glands swelling with discharging sinuses and fever. She has had developed lymphadenitis due BCG vaccination and was treated with ant tubercular medicines for 6 months during her first year of life. Few months later the lymph node reappeared with high fever. Lymph node biopsy showed multiple acid fast bacilli suggestive of tubercular infection. She was diagnosed with Mendelian Susceptibility to Mycobacterial Disease (MSMD that is an inherited condition due to defect in T lymphocytes, a type of white blood cell. Patients with MSMD have increased susceptibility to systemic infections with weakly virulent non-tuberculosis mycobacteria including the Bacillus Calmette-Guérin (BCG) vaccine strain. These conditions are common in children born to parents who married within the family.
Another 6 year old girl was presented with tubercular infection in brain and in the abdomen along with oral thrush and fungal infection of the nails. She was also found out to have low number of T-lymphocytes which is responsible for body defense against infection. Further tests revealed that she had a rare condition called ROR gamma T mutation. In this condition there is a defect in the body immunity against tubercular infection and Candida.
Speaking on the matter Dr. Harsha Prasada L, Consultant, Pediatric Hematologist and Oncologist, who looks after children with immunodeficiency at KMC Hospital, said “Children who develop recurrent or multifocal tubercular or atypical mycobacterial infection the primary reason can be immunodeficiency and treatment must be planned accordingly. Errors in genes weaken child’s immunity. Inherited problems with T lymphocytes cause reduced ability to produce interferon gamma in response to mycobacteria and render the patients vulnerable to tubercular infections including live bacilli calmetter-Guerin (BCG) vaccines used to inoculate against tuberculosis. In these children BCG vaccine can cause severe even fatal illness. He further added, “these above two cases highlight the genetic vulnerability to tuberculosis infection, this means affected children require prolonged treatment, some require preventive treatment, while a few benefit from addition of interferon gamma injections.”
Tuberculosis (TB) is a bacterial infection spread through inhaling tiny droplets from the coughs or sneezes of an infected person. It mainly affects the lungs, but it can affect any part of the body, including the tummy (abdomen), glands, bones and nervous system. All though it is a serious condition, but it can be cured if treated with the right antibiotics. Currently around a million children suffer from TB worldwide and that more than 136,000 die each year. Inherited defect in the immunity leading tuberculosis infection is not well known.