![]() ![]() Our case describes the very rare yet possible risk of such a devastating complication of BCG, especially important for countries where it is given routinely it also emphasis the need for screening of newborns for SCID in similar regions. We present an infant with SCID and BCGiosis. ![]() It is aptly called as a pediatric emergency as it invariably leads to fatality in infancy without early aggressive therapy and Hematopoietic stem cell transplantation (HSCT) or other specific therapy. SCID is arguably the most severe and lethal inherited primary immunodeficiency with a prevalence of approximately 1 in 50,000 live births. Several primary immunodeficiency syndromes has been associated with disseminated BCG disease (BCGiosis), such as Mendelian susceptibility to Mycobacterial disease (MSMD), hyper-IgM syndrome, Di George syndrome, chronic granulomatous disease, Severe combined Immunodeficiency (SCID), IL (Interleukin)-12/23 receptor β1 chain deficiency, IL-12p40 deficiency, STAT1 (Signal transducer and activator of transcription 1) deficiency and NEMO (Nuclear factor kappa-beta essential modulator) deficiency. Having only local complications in general, BCG is associated with lethal disseminated disease in those with immunodeficiency. Primary immunodeficiency, adverse event following immunization, hematopoietic stem cell transplantation, neonatal screening Introductionīacillus Calmette Guerin (BCG) vaccine, made from an attenuated strain of Mycobacterium bovis, is administered routinely in the neonatal period in TB endemic countries including India, as a part of the Universal Immunization schedule. The case highlights the possible risk of such rare yet lethal complication of BCG especially where it is given routinely at birth or in the neonatal period and also emphasizes the need for neonatal screening for SCID in such regions. We report an infant presenting persistent fever, weight loss, anemia, hepatosplenomegaly, skin rash and multiple osteolytic lesions subsequently diagnosed with SCID and BCGiosis. Patients with severe combined immunodeficiency (SCID) having defect in both cellular and humoral immunity are predisposed to a host of live vaccine related complications, especially BCG. BCG (Bacillus Calmette Guerin) being a live attenuated vaccine may cause disseminated disease (BCGiosis) in patients with impaired immunity. ![]()
0 Comments
Leave a Reply. |