1826 Survival Rate of Disseminated Bcgitis in Children with Primary Immunodeficiency - Single Center Experience

Background and Aims Overall prevalence of primary immunodeficiency (PID) is 1:2,000 live births. PID characterized by increased susceptibility to severe infections with distinctive susceptibility to various types of pathogens, like mycobacterium bovis in BCG (Bacille Calmette-Guerin) vaccine. Methods We conducted retrospective study (demographics, investigations and treatments) over 5-years period, between 2007 and 2011, at King Abdulaziz Medical City-WR, Saudi Arabia for patients diagnosed with disseminated BCGitis. Results Out of 16 susceptible immunodeficiency patients given BCG vaccine at birth, 6 patients (37.5%); one chronic granulomatous disease, one ectodermal dysplasia/cytokines deficiency and 4-severe combined immunodeficiency were diagnosed to have disseminated BCGitis. The average age at time of diagnosis was 10½ - months (range: 3 to 30 months), one patient was diagnosed post-bone marrow transplantation. The diagnosis confirmed by microbiologic stain and TB cultures from lungs, gastrointestinal tract and skin biopsies. All patients received 4 anti-tuberculous (TB) drugs, except one patient still on five anti-TB and IFN-γ treatments for a year. In compression to studies from nonindustrial countries (their survival rates ranges: zero to 50%), we achieved an excellent survival rate (100%). However, one patient developed chronic lung disease. Conclusion Our results (100% survival rate) highlight the importance of high index of suspicion, early diagnosis of PID and BCGitis and promote intervention with anti-TB drugs. However, because of prolonged anti-TB therapies and high fatality rates of BCGitis reported from nonindustrial countries where also BCG vaccine is compulsory at birth, we recommend suspending BCG vaccine for susceptible PID newborns till such diagnosis ruled out.