02527nas a2200337   4500000000100000008004100001260001200042653001700054653001600071653001200087653003000099653001400129100001200143700001200155700001200167700001300179700001300192700001200205700001500217700001100232700001300243700001200256700001500268700001100283700001200294700001600306245012800322300001100450520171400461022001402175       2020                            d  c12/202010aBCG adenitis10aBCG vaccine10aBCGosis10aInborn errors of immunity10ascreening1 aYadav R1 aDalvi A1 aGupta M1 aBargir U1 aMhatre S1 aAluri J1 aKulkarni M1 aHule G1 aKambli P1 aSetia P1 aJodhawat N1 aTaur P1 aDesai M1 aMadkaikar M00aSpectrum of Inborn errors of immunity in a cohort of 90 patients presenting with complications to BCG vaccination in India.  ae130103 a<p>World Health Organisation recommends the practice of BCG vaccination at birth in countries which have a high incidence of tuberculosis and/or high leprosy burden. The BCG vaccination is considered safe for a competent immune system. However, in children with weakened immune systems cause of which can be primary or secondary, the vaccine may lead to side effects which can be localized or disseminated. In this study, we report a spectrum of Inborn Errors of Immunity (IEI) commonly referred to as primary immunodeficiency disorders (PIDs) diagnosed in a large cohort of patients presenting with complications to BCG vaccination from India. Retrospective data analysis of patients referred to ICMR- National Institute of Immunohematology (ICMR-NIIH) for IEI workup between 2007-2019 was done. IEI were identified in n=52/90 (57.7%) patients presenting with BCG complications. Of these, n=13(14.4%) patients were diagnosed with Severe Combined Immune-deficiency, n=15(16.7%) with Chronic Granulomatous disease, n=19(21.1%) with Inborn errors of IFN-γ immunity, n=4(4.4%) with Combined immunodeficiency and n=1(1.1%) with Leucocyte Adhesion Deficiency type1. Majority of cases with BCGosis (88%) had an underlying IEI. This study strongly highlights the need for evaluation of patients with BCG complications for underlying IEI. While disseminated BCGosis strongly predicts underlying IEI, even localized persistent adenitis may be a warning sign of underlying IEI. It is also strongly recommended to record a family history of previous sibling death prior to administration of this live vaccine and deferring live vaccine till the diagnosis of IEI is ruled out in cases with a positive family history.</p>  a1365-3083