File Download

There are no files associated with this item.

  Links for fulltext
     (May Require Subscription)
Supplementary

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

TitleSusceptibility to mycobacterial infections in children with x-linked chronic granulomatous disease: A review of 17 patients living in a region endemic for tuberculosis
Authors
KeywordsChildren
Chronic granulomatous disease
CYBB
Issue Date2008
PublisherLippincott Williams & Wilkins. The Journal's web site is located at http://www.pidj.com
Citation
Pediatric Infectious Disease Journal, 2008, v. 27 n. 3, p. 224-230 How to Cite?
AbstractBackground: 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-γ 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.
Persistent Identifierhttp://hdl.handle.net/10722/80030
ISSN
2015 Impact Factor: 2.587
2015 SCImago Journal Rankings: 1.416
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorLee, PPWen_HK
dc.contributor.authorChan, KWen_HK
dc.contributor.authorJiang, Len_HK
dc.contributor.authorChen, Ten_HK
dc.contributor.authorLi, Cen_HK
dc.contributor.authorLee, TLen_HK
dc.contributor.authorMak, PHSen_HK
dc.contributor.authorFok, SFSen_HK
dc.contributor.authorYang, Xen_HK
dc.contributor.authorLau, YLen_HK
dc.date.accessioned2010-09-06T08:01:33Z-
dc.date.available2010-09-06T08:01:33Z-
dc.date.issued2008en_HK
dc.identifier.citationPediatric Infectious Disease Journal, 2008, v. 27 n. 3, p. 224-230en_HK
dc.identifier.issn0891-3668en_HK
dc.identifier.urihttp://hdl.handle.net/10722/80030-
dc.description.abstractBackground: 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-γ 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.en_HK
dc.languageengen_HK
dc.publisherLippincott Williams & Wilkins. The Journal's web site is located at http://www.pidj.comen_HK
dc.relation.ispartofPediatric Infectious Disease Journalen_HK
dc.rightsThe Pediatric Infectious Disease Journal. Copyright © Lippincott Williams & Wilkins.en_HK
dc.subjectChildrenen_HK
dc.subjectChronic granulomatous diseaseen_HK
dc.subjectCYBBen_HK
dc.titleSusceptibility to mycobacterial infections in children with x-linked chronic granulomatous disease: A review of 17 patients living in a region endemic for tuberculosisen_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=0891-3668&volume=27&issue=3&spage=224&epage=230&date=2008&atitle=Susceptibility+to+Mycobacterial+Infections+in+Children+With+X-Linked+Chronic+Granulomatous+Diseaseen_HK
dc.identifier.emailLee, PPW:ppwlee@hku.hken_HK
dc.identifier.emailLau, YL:lauylung@hkucc.hku.hken_HK
dc.identifier.authorityLee, PPW=rp00462en_HK
dc.identifier.authorityLau, YL=rp00361en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1097/INF.0b013e31815b494cen_HK
dc.identifier.scopuseid_2-s2.0-44949109341en_HK
dc.identifier.hkuros142449en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-44949109341&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume27en_HK
dc.identifier.issue3en_HK
dc.identifier.spage224en_HK
dc.identifier.epage230en_HK
dc.identifier.isiWOS:000253661500006-
dc.publisher.placeUnited Statesen_HK
dc.identifier.scopusauthoridLee, PPW=14048822200en_HK
dc.identifier.scopusauthoridChan, KW=8587755300en_HK
dc.identifier.scopusauthoridJiang, L=35285772300en_HK
dc.identifier.scopusauthoridChen, T=35285506000en_HK
dc.identifier.scopusauthoridLi, C=11838894100en_HK
dc.identifier.scopusauthoridLee, TL=8508917400en_HK
dc.identifier.scopusauthoridMak, PHS=36877626000en_HK
dc.identifier.scopusauthoridFok, SFS=7005182792en_HK
dc.identifier.scopusauthoridYang, X=13606095400en_HK
dc.identifier.scopusauthoridLau, YL=7201403380en_HK

Export via OAI-PMH Interface in XML Formats


OR


Export to Other Non-XML Formats