Spectrum of Inborn errors of immunity in a cohort of 90 patients presenting with complications to BCG vaccination in India.
BCG adenitis
BCG vaccine
BCGosis
Inborn errors of immunity
screening
Journal
Scandinavian journal of immunology
ISSN: 1365-3083
Titre abrégé: Scand J Immunol
Pays: England
ID NLM: 0323767
Informations de publication
Date de publication:
May 2021
May 2021
Historique:
revised:
29
11
2020
received:
04
07
2020
accepted:
06
12
2020
pubmed:
17
12
2020
medline:
28
4
2021
entrez:
16
12
2020
Statut:
ppublish
Résumé
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.
Substances chimiques
BCG Vaccine
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e13010Subventions
Organisme : Indian Council of Medical Research
Informations de copyright
© 2020 The Scandinavian Foundation for Immunology.
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