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Published on: March 9, 2018
Non-Aspergillus fungal infections in chronic granulomatous disease
John Dotis1, Zoe Dorothea Pana, Emmanuel Roilides
11st Department of Pediatrics, Aristotle University School of Medicine, Hippokration General Hospital, Thessaloniki, Greece.
Abstract:
Chronic granulomatous disease (CGD) is a congenital immunodeficiency, characterised by significant infections due to an inability of phagocyte to kill catalase-positive organisms including certain fungi such as Aspergillus spp. Nevertheless, other more rare fungi can cause significant diseases. This report is a systematic review of all published cases of non-Aspergillus fungal infections in CGD patients. Analysis of 68 cases of non-Aspergillus fungal infections in 65 CGD patients (10 females) published in the English literature. The median age of CGD patients was 15.2 years (range 0.1-69), 60% of whom had the X-linked recessive defect. The most prevalent non-Aspergillus fungal infections were associated with Rhizopus spp. and Trichosporon spp. found in nine cases each (13.2%). The most commonly affected organs were the lungs in 69.9%. In 63.2% of cases first line antifungal treatment was monotherapy, with amphotericin B formulations being the most frequently used antifungal agents in 45.6% of cases. The overall mortality rate was 26.2%. Clinicians should take into account the occurrence of non-Aspergillus infections in this patient group, as well as the possibility of a changing epidemiology in fungal pathogens. Better awareness and knowledge of these pathogens can optimise antifungal treatment and improve outcome in CGD patients.
Insights
Chronic granulomatous disease (CGD) patients face rare fungal infections beyond Aspergillus. This review highlights Rhizopus and Trichosporon as key pathogens, emphasizing improved clinical awareness for better outcomes.
Area of Science:
- Immunology
- Mycology
- Infectious Diseases
Background:
- Chronic granulomatous disease (CGD) is a primary immunodeficiency hindering phagocyte killing of catalase-positive organisms.
- While Aspergillus spp. are common fungal pathogens in CGD, rare fungal infections pose significant threats.
Purpose of the Study:
- To systematically review and analyze all published cases of non-Aspergillus fungal infections in CGD patients.
- To identify prevalent non-Aspergillus fungal pathogens, affected organs, treatment strategies, and outcomes in CGD.
Main Methods:
- Systematic literature review of English-language publications.
- Analysis of 68 cases of non-Aspergillus fungal infections in 65 CGD patients.
- Data extraction on patient demographics, fungal species, affected organs, treatments, and mortality.
Main Results:
- Rhizopus spp. and Trichosporon spp. were the most prevalent non-Aspergillus pathogens (9 cases each).
- Pulmonary involvement was most common (69.9%).
- Amphotericin B formulations were the most frequent first-line monotherapy (45.6%), with an overall mortality rate of 26.2%.
Conclusions:
- Non-Aspergillus fungal infections are significant in CGD patients and require clinical consideration.
- Awareness of emerging fungal pathogens and their epidemiology is crucial for optimizing antifungal treatment.
- Improved knowledge can enhance patient outcomes in CGD.
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