Susceptibility to mycobacterial infections in children with X-linked chronic granulomatous disease: a review of 17 patients living in a region endemic for tuberculosis.

التفاصيل البيبلوغرافية
العنوان:	Susceptibility to mycobacterial infections in children with X-linked chronic granulomatous disease: a review of 17 patients living in a region endemic for tuberculosis.
المؤلفون:	Lee PP; Department of Paediatrics and Adolescent Medicine, The University of Hong Kong, HKSAR, China., Chan KW, Jiang L, Chen T, Li C, Lee TL, Mak PH, Fok SF, Yang X, Lau YL
المصدر:	The Pediatric infectious disease journal [Pediatr Infect Dis J] 2008 Mar; Vol. 27 (3), pp. 224-30.
نوع المنشور:	Journal Article; Research Support, Non-U.S. Gov't
اللغة:	English
بيانات الدورية:	Publisher: Williams & Wilkins Country of Publication: United States NLM ID: 8701858 Publication Model: Print Cited Medium: Print ISSN: 0891-3668 (Print) Linking ISSN: 08913668 NLM ISO Abbreviation: Pediatr Infect Dis J Subsets: MEDLINE
أسماء مطبوعة:	Original Publication: [Baltimore, Md. : Williams & Wilkins, c1987-
مواضيع طبية MeSH:	Granulomatous Disease, Chronic/complications , Granulomatous Disease, Chronic/genetics , Tuberculosis/epidemiology , Tuberculosis/genetics, Abscess/epidemiology ; Adolescent ; Animals ; Child ; Child, Preschool ; China/epidemiology ; Disease Susceptibility ; Endemic Diseases ; Humans ; Infant ; Lymphadenitis/epidemiology ; Male ; Mutation ; Mycobacterium bovis/isolation & purification ; Mycobacterium tuberculosis/isolation & purification ; Pneumonia/epidemiology
مستخلص:	Background: Chronic granulomatous disease (CGD) is a rare disorder of phagocytic oxidative bursts leading to recurrent pyogenic infections. Affected individuals are most prone to infections caused by staphylococci, Salmonella, Candida, and Aspergillus, but previously we observed a high incidence of Mycobacterium tuberculosis infection in Chinese children with CGD. Objective: To determine the spectrum of infections in patients with X-linked CGD, with an emphasis on mycobacterial infections, and to review all CYBB gene mutations identified in our center. Results: From 1988 to 2005, 17 Chinese male children were diagnosed to have X-linked CGD. Fifteen mutations were identified, including 3 splice site defects (IVS1-1G>C, 266G>A, IVS3-1G>A), 5 missense mutations (591T>C, 627T>A, 949T>A, 1039T>A, 1512G>C), 3 nonsense mutations (882C>T, 1451C>A, 1569G>T), 1 insertion (756_757insA), and 3 deletions (660_662delTTC, 727delT, 1341delT). Eight of these were novel mutations. Recurrent pneumonia, lymphadenitis, and bacterial skin abscess were the commonest types of infection. Seven patients had tuberculosis (TB). Seven patients had prolonged scarring or abscess formation at the Calmette-Guérin bacillus (BCG) injection site, and 1 had disseminated BCG infection. Three patients had pulmonary aspergillosis. Four patients underwent hemopoietic stem cell transplantation, but 2 died of complications. Conclusions: Patients with CGD are susceptible to TB and BCG complications. Our observation suggests that oxidative burst is probably important in host defense against mycobacterial infections. Because interferon-gamma is the key cytokine involved in mycobacterial immunity, there may be a stronger indication for its use in CGD patients living in areas endemic for TB.
تواريخ الأحداث:	Date Created: 20080219 Date Completed: 20080612 Latest Revision: 20220321
رمز التحديث:	20231215
DOI:	10.1097/INF.0b013e31815b494c
PMID:	18277931
قاعدة البيانات:	MEDLINE

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تدمد:	0891-3668
DOI:	10.1097/INF.0b013e31815b494c