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Exophiala dermatitidis pneumonia in cystic fibrosis
G Kusenbach1, H Skopnik, G Haase
1Department of Paediatrics, Technical University, Aachen, Federal Republic of Germany.
Insights
A rare fungal pneumonia caused by Exophiala dermatitidis was identified in a child with cystic fibrosis (CF). This marks the first reported isolation of this fungus in CF patients, highlighting the need for broader diagnostic considerations.
Area of Science:
- Medical Mycology
- Pulmonology
- Pediatrics
Background:
- Cystic Fibrosis (CF) patients are susceptible to pulmonary infections.
- Fungal pathogens are increasingly recognized in CF pulmonary disease.
- Diagnostic challenges exist for identifying specific fungal etiologies in CF.
Observation:
- A pediatric patient with CF presented with progressive pulmonary symptoms and radiographic findings.
- Despite standard treatments, the patient's condition worsened.
- Multiple fungi, including Candida albicans, Aspergillus fumigatus, and Exophiala dermatitidis, were isolated from sputum.
Findings:
- Serological tests for Candida albicans and Aspergillus fumigatus were negative or normal.
- Antibodies against Exophiala dermatitidis were detected using a novel immunofluorescence assay.
- Exophiala dermatitidis was identified as the likely cause of fungal pneumonia.
- Antifungal therapy with amphotericin B, flucytosine, and itraconazole led to clinical improvement and fungal eradication.
Implications:
- This case represents the first isolation of Exophiala dermatitidis from a cystic fibrosis patient.
- It underscores the importance of considering less common fungal pathogens in CF-related lung disease.
- The findings suggest a need for expanded diagnostic approaches for fungal infections in CF.
Abstract:
The chest X-ray film of a girl with cystic fibrosis (CF) showed slowly increasing mottled densities during the 6th and 7th year of her life. Pulmonary symptoms and distress proceeded fast in spite of intensive treatment with antibiotics, corticosteroids, and physiotherapy. Three different fungal organisms were repeatedly cultured from the sputum: Candida albicans, Aspergillus fumigatus, and Exophiala dermatitidis. Antibodies against C. albicans were in the normal range. Candida antigen in blood and antibodies against A. fumigatus were absent. Antibodies against E. dermatitidis were detected by a recently developed indirect immunofluorescence assay. It seems most probable that E. dermatitidis was the causal agent for fungal pneumonia in this case. Under therapy with amphotericin B and flucytosine the clinical course and radiological appearance improved but definitive eradication of E. dermatitidis was only achieved after treatment with itraconazole. The isolation of this fungus from the sputum of a CF patient is reported for the first time. The significance of fungal infections in CF is discussed.