Severe allergic bronchopulmonary aspergillosis in an infant with cystic fibrosis and her asthmatic father

An infant with cystic fibrosis and her asthmatic father were diagnosed as s uffering from allergic bronchopulmonary aspergillosis (ABPA). Cystic fibros is was diagnosed in the infant at 6 weeks of age, and gene mutations were W 1282X/G542X. She was diagnosed definitively as suffering from ABPA at age 3 .5 years, but had suggestive symptoms from age 11 months, This may be the y oungest age described to date for ABPA, The child responded well to systemi c steroid therapy, but remained steroid-dependent over the next 4 years, Tr eatment with itraconazole enabled a marked reduction in steroid dosage. The father was an asthmatic, and a heterozygote for the cystic fibrosis transm embrane regulator (CFTR) mutation W1282X. He had a normal sweat test, atopy , and moderate reversible airway obstruction. There was no proven exposure to Aspergillus in the home environment. The importance of considering the d iagnosis of ABPA even in infancy, the therapeutic dilemmas, and the possibl e role of abnormal CFTR function in the development of ABPA are discussed. Pediatr Pulmonol, 2000; 29:155-159, (C) 2000 Wiiey-Liss, Inc.