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Zygomycosis in children: a systematic review and analysis of reported cases
Theoklis E Zaoutis1, Emmanuel Roilides, Christine C Chiou
1Department of Pediatrics and the Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania School of Medicine, Philadelphia, PA, USA. zaoutis@email.chop.edu
Insights
Pediatric zygomycosis is a severe fungal infection with high mortality, particularly in infants and those with underlying conditions like neutropenia or diabetes. Early treatment with amphotericin B and surgery significantly improves survival rates.
Area of Science:
- Mycology
- Pediatric Infectious Diseases
- Epidemiology
Background:
- Zygomycosis presents a significant and increasing threat, especially to immunocompromised individuals, carrying a high mortality rate.
- Comprehensive analyses of pediatric zygomycosis cases remain scarce in published literature.
Purpose of the Study:
- To conduct a thorough analysis of pediatric zygomycosis cases.
- To identify risk factors, common clinical presentations, and outcomes in children with zygomycosis.
Main Methods:
- A systematic PubMed search was performed for English-language publications on pediatric zygomycosis (ages 0-18).
- References from major textbooks and individual case reports/series were reviewed to identify additional cases.
- Data were compiled in a database and analyzed using logistic regression.
Main Results:
- 157 pediatric cases were identified, with neutropenia, prematurity, and diabetes mellitus as common predisposing factors.
- Cutaneous, gastrointestinal, rhinocerebral, and pulmonary zygomycosis were the most frequent forms; Rhizopus and Mucor species were the most common pathogens.
- Mortality was significantly higher in untreated patients (88% vs. 36%), with disseminated infection and age under 1 year identified as independent risk factors for death. Antifungal therapy and surgery substantially reduced mortality.
Conclusions:
- Zygomycosis is a life-threatening pediatric infection, often associated with neutropenia, diabetes mellitus, and prematurity.
- Disseminated disease, lack of treatment, and infancy (<1 year) are linked to poor outcomes.
- Amphotericin B treatment and surgical intervention are critical for improving survival in pediatric zygomycosis.
Background:
Zygomycosis has emerged as an increasingly important infection with a high mortality especially in immunocompromised patients. No comprehensive analysis of pediatric zygomycosis cases has been published to date.
Methods:
We used a PUBMED search for English publications of pediatric (0-18 years) zygomycosis cases and references from major books as well as single case reports or case series. Individual references were reviewed for additional cases. Data were entered into Filemaker-pro database and analyzed by logistic regression analysis.
Results:
One hundred fifty-seven cases (64% male) were found with median age 5 years (range, 0.16-13). Underlying conditions included neutropenia (18%), prematurity (17%), diabetes mellitus (15%), ketoacidosis (10%), and no apparent underlying condition (14%). The most common patterns of zygomycosis were cutaneous (27%), gastrointestinal (21%), rhinocerebral (18%), and pulmonary (16%). Among 77 culture-confirmed cases, Rhizopus spp. (44%) and Mucor spp. (15%) were most commonly identified. Of 81 patients who were given antifungal therapy, 73% received an amphotericin B formulation only. The remaining patients received mostly amphotericin B in combination with other antifungal agents. Mortality in patients without antifungal therapy was higher than in those with therapy (88% versus 36%, P < 0.0001). Ninety-two (59%) patients underwent surgery. Cerebral, gastrointestinal, disseminated and cutaneous zygomycosis were associated with mortality rates of 100, 100, 88, and 0%, respectively. Independent risk factors for death were disseminated infection (OR: 7.18; 95% CI: 3.02-36.59) and age <1 year (OR: 3.85; 95% CI: 1.05-7.43). Antifungal therapy and particularly surgery reduced risk of death by 92% (OR: 0.07; 95% CI: 0.04-0.25) and 84% (OR: 0.16; 95% CI: 0.09-0.61), respectively.
Conclusions:
Zygomycosis is a life-threatening infection in children with neutropenia, diabetes mellitus, and prematurity as common predisposing factors, and there is high mortality in untreated disease, disseminated infection, and age <1 year. Amphotericin B and surgery significantly improve outcome.
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