Clinical Characteristics and Immunogenetics of BCGosis/BCGitis in Chinese Children: A 6 Year Follow-Up Study

In this study, the clinical and immunogenetical features in a cohort of Chinese patients with BCGosis/BCGitis were investigated. For the patients with abnormal immunological functions, Sanger sequencing was used to identify the involved genes. There were 74 confirmed cases of BCGosis/BCGitis during 2007–2012. Classified by infected tissues and organs, no cases only had local infection, 39 patients had a regional infection, 21 patients had a distant infection and 14 patients had a disseminated infection. Thirty-two patients (43.2%) had definitive primary immunodeficiency diseases (PID) and chronic granulomatous disease (CGD) is the most common PID (n = 23, accounted for 71.9% of all PID patients). For CGD patients, based on the anti-tuberculosis treatment, administration of rhIFN-γ resulted in better control of BCGosis/BCGitis. The results indicate that PIDs are associated with susceptibility to BCG disease. For children with BCGosis/BCGitis, immune function evaluation is necessary, and IFN-γ treatment for BCGosis/BCGitis patients with CGD is effective.

[1]  Y. Wang,et al.  Prenatal Diagnosis of X‐Linked Chronic Granulomatous Disease by Percutaneous Umbilical Blood Sampling , 2012, Scandinavian journal of immunology.

[2]  S. Rosenzweig,et al.  Bacillus Calmette-Guérin (BCG) complications associated with primary immunodeficiency diseases. , 2012, The Journal of infection.

[3]  J. Casanova,et al.  Partial recessive IFN-γR1 deficiency: genetic, immunological and clinical features of 14 patients from 11 kindreds. , 2011, Human molecular genetics.

[4]  Smita Y. Patel,et al.  Revisiting Human IL-12R&bgr;1 Deficiency: A Survey of 141 Patients From 30 Countries , 2010, Medicine.

[5]  A. Towbin,et al.  Chronic granulomatous disease , 2010, Pediatric Radiology.

[6]  M. Chiriacò,et al.  Identification of deletion carriers in X-linked chronic granulomatous disease by real-time PCR. , 2009, Genetic testing and molecular biomarkers.

[7]  Xing Jun Li,et al.  A new genetic subgroup of chronic granulomatous disease with autosomal recessive mutations in p40 phox and selective defects in neutrophil NADPH oxidase activity. , 2009, Blood.

[8]  Dirk Roos,et al.  Chronic Granulomatous Disease: The European Experience , 2009, PloS one.

[9]  H. Xia,et al.  Prevalence of tuberculosis drug resistance in 10 provinces of China , 2008, BMC infectious diseases.

[10]  R. Gie,et al.  Bacille Calmette-Guérin vaccine-induced disease in HIV-infected and HIV-uninfected children. , 2006, Clinical infectious diseases : an official publication of the Infectious Diseases Society of America.

[11]  A. Muñoz,et al.  BCG vaccine effectiveness in preventing tuberculosis and its interaction with human immunodeficiency virus infection. , 2000, International journal of epidemiology.

[12]  J. Palmblad,et al.  Gamma Interferon Treatment of Patients with Chronic Granulomatous Disease Is Associated with Augmented Production of Nitric Oxide by Polymorphonuclear Neutrophils , 1999, Clinical Diagnostic Laboratory Immunology.

[13]  J. Grange Complications of bacille Calmette-Guérin (BCG) vaccination and immunotherapy and their management. , 1998, Communicable disease and public health.

[14]  R. Frothingham,et al.  PCR identification of Mycobacterium bovis BCG , 1997, Journal of clinical microbiology.

[15]  A. Fischer,et al.  Immunological conditions of children with BCG disseminated infection , 1995, The Lancet.

[16]  John I. Gallin,et al.  A controlled trial of interferon gamma to prevent infection in chronic granulomatous disease. The International Chronic Granulomatous Disease Cooperative Study Group. , 1991, The New England journal of medicine.