Spectrum of Inborn errors of immunity in a cohort of 90 patients presenting with complications to BCG vaccination in India
Autor: | Neha Jodhawat, Manasi Kulkarni, Snehal Shabrish, Prasad Taur, Umair Ahmed Bargir, Mukesh Desai, Maya Gupta, Gouri Hule, Priyanka Kambli, Jahnavi Aluri, Manisha Madkaikar, Aparna Dalvi, Priyanka Setia, R Yadav |
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Rok vydání: | 2020 |
Předmět: |
0301 basic medicine
Male Pediatrics medicine.medical_specialty Tuberculosis Immunology India Granulomatous Disease Chronic 03 medical and health sciences 0302 clinical medicine medicine Humans Family history Tuberculosis Pulmonary Immunodeficiency business.industry Incidence (epidemiology) Vaccination Infant General Medicine Mycobacterium tuberculosis medicine.disease 030104 developmental biology Treatment Outcome Cohort Primary immunodeficiency BCG Vaccine Female Severe Combined Immunodeficiency business BCG vaccine 030215 immunology |
Zdroj: | Scandinavian journal of immunologyREFERENCES. 93(5) |
ISSN: | 1365-3083 |
Popis: | 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 and 2019 was done. IEI was 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. |
Databáze: | OpenAIRE |
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