title: A family history of serious complications due to BCG vaccination is a tool for the early diagnosis of severe primary immunodeficiency.
abstract: Severe Combined Immunodeficiency (SCID) is one of the most severe forms of primary immunodeficiency (PID). Complications of BCG vaccination, especially disseminated infection and its most severe forms, are known to occur in immunodeficient patients, particularly in SCID. A carefully taken family history before BCG injection as well as delaying vaccination if PID is suspected could be a simple and effective method to avoid inappropriate vaccination of an immunodeficient child in some cases until the prospect of newborn screening for SCID has been fully developed. We describe a patient with a very early diagnosis of SCID, which was suspected on the basis of the previous death of two siblings younger than one year due to severe complications secondary to the BCG vaccine. We suggest that a family history of severe or fatal reactions to BCG should be included as a warning sign for an early diagnosis of SCID.
authors.affiliation: Department of Pediatrics, Ribeirão Preto Medical School, University of São Paulo, Avenida Bandeirantes, 3900, Ribeirão Preto, São Paulo 14049-900, Brazil. persiorj@fmrp.usp.br.
authors.forename: Pérsio
authors.initials: P
authors.lastname: Roxo-Junior
authors.forename: Jorgete
authors.initials: J
authors.lastname: Silva
authors.forename: Mauro
authors.initials: M
authors.lastname: Andrea
authors.forename: Larissa
authors.initials: L
authors.lastname: Oliveira
authors.forename: Fernando
authors.initials: F
authors.lastname: Ramalho
authors.forename: Thiago
authors.initials: T
authors.lastname: Bezerra
authors.forename: Altacílio A
authors.initials: AA
authors.lastname: Nunes
mesh_terms: D001500:BCG Vaccine / Q000008:administration & dosage / Q000009:adverse effects* / Q000276:immunology*; D001938:Brazil; D002423:Cause of Death; D042241:Early Diagnosis; D005260:Female; D006579:Heterozygote; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008487:Medical History Taking; D009169:Mycobacterium tuberculosis / Q000235:genetics; D010375:Pedigree; D018570:Risk Assessment; D012494:Sampling Studies; D016511:Severe Combined Immunodeficiency / Q000175:diagnosis* / Q000235:genetics* / Q000401:mortality; D015996:Survival Rate; D014397:Tuberculosis, Pulmonary / Q000517:prevention & control; D014611:Vaccination / Q000009:adverse effects
chemical_list: D001500:BCG Vaccine
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