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Published on: March 9, 2018
Invasive mold infections in chronic granulomatous disease: a 25-year retrospective survey
Sophie Blumental1, Richard Mouy, Nizar Mahlaoui
1Immunology and Haematology Unit, Hôpital Necker Enfants Malades, Paris, France. sophie.blumental@ulb.ac.be
Background:
Invasive fungal infection (IFI) represents a life-threatening condition for patients with chronic granulomatous disease (CGD) and causes one-third of deaths in this population. This study offers a descriptive review of invasive mold infection (mIFI) in children with CGD over an extended period of time.
Methods:
In a cohort of patients with CGD registered in the French National database for Primary Immunodeficiency, we performed a retrospective review of proven mIFI episodes (European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group 2008 criteria) occurring from 1984 through 2009.
Results:
Twenty-nine proven mIFIs were identified in 24 patients. Thirteen (54%) of 24 children were receiving itraconazole prophylaxis. Seven episodes were caused by Aspergillus fumigatus, 10 by Aspergillus nidulans, 2 by Aspergillus species, and 6 by other opportunistic molds (4 patients only had positive pathological examination findings). First proven mIFI occurred later in the group that received itraconazole than in the group without (median time to mIFI, 10 vs 4 years; P < .01), with a higher proportion of infections due to A. nidulans and other opportunistic molds (P < .05). Course of IFI was complex, with the median duration of therapy and hospitalization reaching 446 and 153 days, respectively. Combined antifungal therapy was commonly used. Four patients received geno-identical hematopoietic stem cell transplantation as salvage therapy. Global cure rate among the cohort reached 75%, but sequelae were frequent. Prognosis has improved over time (43% mortality during 1985-1990 vs 6% thereafter; P = .06). Mortality tended to be lower in the group that recieved itraconazole prophylaxis but at the cost of a longer duration of therapy among cured patients.
Conclusions:
Management of mIFI remains challenging in patients with CGD, but significant improvements have been made over the past decade.
Insights
Invasive fungal infections (IFIs) are a major threat in chronic granulomatous disease (CGD). While management has improved, invasive mold infections (mIFIs) in children with CGD remain challenging.
Area of Science:
- Immunology
- Infectious Diseases
- Pediatrics
Background:
- Invasive fungal infections (IFIs) are a leading cause of mortality in patients with chronic granulomatous disease (CGD).
- Invasive mold infections (mIFIs) pose a significant threat, contributing to one-third of deaths in this vulnerable population.
- This study reviews mIFI in children with CGD over an extended timeframe.
Purpose of the Study:
- To describe the epidemiology and outcomes of proven invasive mold infections (mIFIs) in children with chronic granulomatous disease (CGD).
- To analyze the impact of antifungal prophylaxis on mIFI incidence and characteristics.
- To evaluate trends in management and prognosis of mIFI in pediatric CGD patients.
Main Methods:
- Retrospective review of proven mIFI episodes in a French national cohort of CGD patients (1984-2009).
- Utilized European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and National Institute of Allergy and Infectious Diseases Mycoses Study Group criteria for diagnosis.
- Analyzed patient demographics, mIFI characteristics, treatment strategies, and outcomes.
Main Results:
- Twenty-nine mIFI episodes were identified in 24 pediatric CGD patients.
- Itraconazole prophylaxis was associated with a later median time to first mIFI (10 vs. 4 years) and a higher proportion of infections due to Aspergillus nidulans and other molds.
- Treatment courses were prolonged (median 446 days therapy, 153 days hospitalization), with 75% global cure rate but frequent sequelae. Mortality improved significantly over time.
Conclusions:
- Management of mIFI in pediatric CGD patients remains complex.
- Significant improvements in outcomes for mIFI in CGD patients have been observed over the past decade.
- Antifungal prophylaxis may alter mIFI patterns but requires careful consideration of treatment duration and outcomes.
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