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ABSTRACT
Patients with cystic fibrosis (CF) often are colonized by Aspergillus species in their respiratory tract, but invasive aspergillosis is a rare complication. We describe the autopsy findings of an infant with cystic fibrosis who had fatal disseminated aspergillosis. The causative agent was identified as A. penicillioides by molecular technique. To our knowledge, this is the first report of disseminated aspergillosis caused by A. penicillioides in any type of patient. The literature on invasive aspergillosis in patients with cystic fibrosis also is reviewed.
Key words: autopsy, cystic fibrosis, disseminated, invasive aspergillosis
INTRODUCTION
Cystic fibrosis (CF) is an autosomal recessive disorder due to mutation in the CF transmembrane conductor regulator (CFTR) protein gene on chromosome 7 [1]. It affects multiple organs and may present with varied clinical symptoms. The patients are predisposed to recurrent bacterial infections of the lungs due to defective mucociliary clearance. Fungal involvement of the lungs in cystic fibrosis usually is allergic bronchopulmonary aspergillosis (ABPA), with reported incidence in approximately 11% patients [2]. However, invasive aspergillosis is rare in CF and it has been described mainly in patients who receive immunosuppressive therapy after lung transplantation [2-6], where its incidence is reported to be 22.5% [5]. We report a rare case of a 3-month-old infant with CF, who suffered fatal disseminated aspergillosis due to Aspergillus penicillioides.
CASE REPORT
Clinical history
A 3-month-old infant boy presented with fever, respiratory distress, and cough for 1 month in duration, with worsening symptoms for last 2 days. He had been treated previously for pneumonia (oxygen, antibiotics, nebulization), but had persistent cough. At admission, he had respiratory distress and lethargy. He was third in birth order of a nonconsanguineous marriage, born through a normal vaginal delivery. His birth weight was 3.75 kg, and there were no perinatal complications. An older sibling had died at two years of age due to pneumonia but clinical details were unavailable. The patient was immunized with bacillus Calmette-Guerin (BCG), oral polio vaccine (OPV), and hepatitis but had not received diphtheria, pertussis, and tetanus (DPT), and was developmentally normal. At admission, he had tachycardia, tachypnea, shallow respiratory effort, and central cyanosis. Examination revealed failure to thrive (weight = 4 kg, length = 54 cm, head circumference = 36 cm), but there was no...