Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2023 Sep 5;11(9):2233.
doi: 10.3390/microorganisms11092233.

Chronic Granulomatous Disease (CGD): Commonly Associated Pathogens, Diagnosis and Treatment

Affiliations
Review

Chronic Granulomatous Disease (CGD): Commonly Associated Pathogens, Diagnosis and Treatment

Angel A Justiz-Vaillant et al. Microorganisms. .

Abstract

Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by a defect in the phagocytic function of the innate immune system owing to mutations in genes encoding the five subunits of the nicotinamide adenine dinucleotide phosphatase (NADPH) oxidase enzyme complex. This review aimed to provide a comprehensive approach to the pathogens associated with chronic granulomatous disease (CGD) and its management. Patients with CGD, often children, have recurrent life-threatening infections and may develop infectious or inflammatory complications. The most common microorganisms observed in the patients with CGD are Staphylococcus aureus, Aspergillus spp., Candida spp., Nocardia spp., Burkholderia spp., Serratia spp., and Salmonella spp. Antibacterial prophylaxis with trimethoprim-sulfamethoxazole, antifungal prophylaxis usually with itraconazole, and interferon gamma immunotherapy have been successfully used in reducing infection in CGD. Haematopoietic stem cell transplantation (HCT) have been successfully proven to be the treatment of choice in patients with CGD.

Keywords: antimicrobials; chronic granulomatous disease; microorganisms; neutrophils.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Schematic representation of normal phagocytic function reproduced from Kruger et al. (2015). Copyright: © 2015 Kruger et al. This is an open-access article distributed under the terms of the Creative Commons [8].
Figure 2
Figure 2
Illustration of the NADPH oxidase complex subunits (OEC). The membrane bound subunits gp22phos (CYBA) and gp91phox (CYBB) components also include the molecule for essential reactive oxygen species (EROS), which interacts with other proteins in the membrane. Adapted from Anjani et al. (2020) [4].
Figure 3
Figure 3
Summary of conclusions, including common microorganisms and clinical aspects.

References

    1. Bridges R.A., Berendes H., Good R.A. A Fatal Granulomatous Disease of Childhood; the Clinical, Pathological, and Laboratory Features of a New Syndrome. AMA J. Dis. Child. 1959;97:387–408. doi: 10.1001/archpedi.1959.02070010389004. - DOI - PubMed
    1. O’Shea P.A. Chronic Granulomatous Disease of Childhood. Perspect. Pediatr. Pathol. 1982;7:237–258. - PubMed
    1. Good R.A., Quie P.G., Windhorst D.B., Page A.R., Rodey G.E., White J., Wolfson J.J., Holmes B.H. Fatal (chronic) Granulomatous Disease of Childhood: A Hereditary Defect of Leukocyte Function. Semin. Hematol. 1968;5:215–254. - PubMed
    1. Anjani G., Vignesh P., Joshi V., Shandilya J.K., Bhattarai D., Sharma J., Rawat A. Recent Advances in Chronic Granulomatous Disease. Genes Dis. 2020;7:84–92. doi: 10.1016/j.gendis.2019.07.010. - DOI - PMC - PubMed
    1. Roos D. Chronic Granulomatous Disease. Br. Med. Bull. 2016;118:50–63. doi: 10.1093/bmb/ldw009. - DOI - PMC - PubMed

LinkOut - more resources