Clinical Infectious Diseases Advance Access published December 23, 2014 1

COMMON SEVERE INFECTIONS IN CHRONIC GRANULOMATOUS

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DISEASE

Beatriz E. Marciano1, Christine Spalding1, Alan Fitzgerald1, Daphne Mann2 Thomas Brown2, Sharon Osgood1, Lynne Yockey1, Dirk N. Darnell1, Lisa

Barnhart1, Janine Daub1, Lisa Boris2, Amy P. Rump2, Anderson Victoria1, Carissa

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Tamika Mason1, Suk See DeRavin5, Elizabeth Kang5, John I. Gallin5, Harry L.

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Malech4, Kenneth N. Olivier1, Gulbu Uzel1, Alexandra F. Freeman1, Theo Heller6, Christa S. Zerbe1, Steven M. Holland1

Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious

Diseases, NIH 2

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Clinical Research Directorate/Clinical Monitoring Research Program, Leidos

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Biomedical Research, Inc., Frederick National Laboratory for Cancer Research, Frederick, Maryland 21702 Leidos, Frederick, MD

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Department of Laboratory Medicine, Clinical Center, NIH

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Laboratory of Host Defenses, NIAID, NIH

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Liver Disease Section, NIDDK

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Correspondence to: Steven M. Holland, MD, CRC B3-4141 MSC 1684, Bethesda, MD 20892-1684, 301-402-7684 voice, 301-480-4807 fax, [email protected] email

Published by Oxford University Press on behalf of the Infectious Diseases Society of America 2014. This work is written by (a) US Government employee(s) and is in the public domain in the US.

Downloaded from http://cid.oxfordjournals.org/ at University of California, Davis on January 21, 2015

Haney1, Douglas B Kuhns3, Sergio D. Rosenzweig4, Corin Kelly5, Adrian Zelazny4,

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Summary: Chronic Granulomatous Disease is a genetic immunodeficiency characterized by a

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limited spectrum of recurrent bacterial and fungal infections. Genetically determined superoxide production is linked to overall survival as well as severity of infections.

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This project has been funded in whole or in part with federal funds from the National

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HHSN261200800001E. The content of this publication does not necessarily reflect the

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views or policies of the Department of Health and Human Services, nor does mention of trade names, commercial products, or organizations imply endorsement by the U.S.

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Government.

This research was supported [in part] by the Intramural Research Program of the NIH,

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National Cancer Institute, Center for Cancer Research

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Cancer Institute, National Institutes of Health, under Contract No.

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ABSTRACT: Chronic Granulomatous Disease (CGD) is due to defective NADPH oxidase activity and

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characterized by recurrent infections with a limited spectrum of bacteria and fungi as well as inflammatory complications. In order to understand the impact of common severe

infections in CGD, we examined the records of 268 patients followed at a single center

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Results: Aspergillus incidence was estimated at 2.6 cases per 100 patient/years;

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Burkholderia incidence was 1.06 per 100 patient/years; Nocardia incidence was 0.81 per 100 patient/years; Serratia infection incidence was 0.98 per 100 patients /years and severe Staphylococcus infections were 1.44 per 100 patient/years. Lung infection

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occurred in 87% of patients, while liver abscess occurred in 32%. Aspergillus incidence was 55% in the lower superoxide producing quartiles (quartiles 1 and 2) but only 41% in the higher quartiles (rate ratio=

Common severe infections in chronic granulomatous disease.

Chronic granulomatous disease (CGD) is due to defective nicotinamide adenine dinucleotide phosphate oxidase activity and characterized by recurrent in...
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