OBJECTIVES: The aim of this study was to evaluate immunity in HIV-uninfected children with bacille Calmette-Guerin-induced disease (BCG-ID) over an 8-year period, with particular emphasis on underlying diseases. METHODS: Patient afflicted with BCG-ID proven by clinical courses, dermatologic features, pathology, specific polymerase chain reaction, and/or spoligotyping were enrolled between 2000 and 2007. Lymphocyte proliferation, polymorphonuclear function, interleukin (IL)-12/23-interferons (IFN)-gamma circuit, and Toll-like receptor 2-associated signaling were investigated. RESULTS: Of the 271,618 total live births who received the BCG vaccine, eight patients (seven males) with BCG-ID were enrolled during an 8-year period and presented as three disseminated, two distant, and three regional BCG-ID. Their age at onset ranged from 1 to 28 months. All had a vaccine-injection scar except for one with lower CD3 and natural killer cells, compatible with severe combined immunodeficiency (SCID) identified by IL-2 receptor common gamma chain (IL2RG) mutation (Arg226Lys). The other SCID patient with de novo IL2RG mutation (Trp74Gly) had more recurrent infections. The third patient with primary autoimmune neutropenia had disseminated BCG-ID extending to abdominal wall. The fourth patient with chronic mucocutaneous candidiasis had regional BCG-ID and impaired lymphocyte proliferation to Candida and BCG antigens. No defective evidence of polymorphonuclear functions, IL-12/23-IFN-gamma circuit, and Toll-like receptor 2-associated signaling was detected in the remaining four patients. CONCLUSION: Immunologic analysis in HIV-uninfected patients with BCG-ID reveals primary immunodeficiency diseases, especially in those with deficiencies in T-cell and neutrophil functions observed in our cohort, including primary autoimmune neutropenia and chronic mucocutaneous candidiasis.
OBJECTIVES: The aim of this study was to evaluate immunity in HIV-uninfectedchildren with bacille Calmette-Guerin-induced disease (BCG-ID) over an 8-year period, with particular emphasis on underlying diseases. METHODS:Patient afflicted with BCG-ID proven by clinical courses, dermatologic features, pathology, specific polymerase chain reaction, and/or spoligotyping were enrolled between 2000 and 2007. Lymphocyte proliferation, polymorphonuclear function, interleukin (IL)-12/23-interferons (IFN)-gamma circuit, and Toll-like receptor 2-associated signaling were investigated. RESULTS: Of the 271,618 total live births who received the BCG vaccine, eight patients (seven males) with BCG-ID were enrolled during an 8-year period and presented as three disseminated, two distant, and three regional BCG-ID. Their age at onset ranged from 1 to 28 months. All had a vaccine-injection scar except for one with lower CD3 and natural killer cells, compatible with severe combined immunodeficiency (SCID) identified by IL-2 receptor common gamma chain (IL2RG) mutation (Arg226Lys). The other SCIDpatient with de novo IL2RG mutation (Trp74Gly) had more recurrent infections. The third patient with primary autoimmune neutropenia had disseminated BCG-ID extending to abdominal wall. The fourth patient with chronic mucocutaneous candidiasis had regional BCG-ID and impaired lymphocyte proliferation to Candida and BCG antigens. No defective evidence of polymorphonuclear functions, IL-12/23-IFN-gamma circuit, and Toll-like receptor 2-associated signaling was detected in the remaining four patients. CONCLUSION: Immunologic analysis in HIV-uninfectedpatients with BCG-ID reveals primary immunodeficiency diseases, especially in those with deficiencies in T-cell and neutrophil functions observed in our cohort, including primary autoimmune neutropenia and chronic mucocutaneous candidiasis.
Authors: E Jouanguy; S Lamhamedi-Cherradi; F Altare; M C Fondanèche; D Tuerlinckx; S Blanche; J F Emile; J L Gaillard; R Schreiber; M Levin; A Fischer; C Hivroz; J L Casanova Journal: J Clin Invest Date: 1997-12-01 Impact factor: 14.808
Authors: A C Hesseling; H Rabie; B J Marais; M Manders; M Lips; H S Schaaf; R P Gie; M F Cotton; P D van Helden; R M Warren; N Beyers Journal: Clin Infect Dis Date: 2006-01-11 Impact factor: 9.079
Authors: Aileen M Cleary; Wenwei Tu; Andrea Enright; Thierry Giffon; Rene Dewaal-Malefyt; Kathleen Gutierrez; David B Lewis Journal: J Immunol Date: 2003-01-01 Impact factor: 5.422
Authors: Riny Janssen; Annelies van Wengen; Marieke A Hoeve; Monique ten Dam; Miriam van der Burg; Jacques van Dongen; Esther van de Vosse; Maarten van Tol; Robbert Bredius; Tom H Ottenhoff; Corry Weemaes; Jaap T van Dissel; Arjan Lankester Journal: J Exp Med Date: 2004-08-30 Impact factor: 14.307