TY - JOUR
T1 - Severe allergic bronchopulmonary aspergillosis in an infant with cystic fibrosis and her asthmatic father
AU - Mussaffi, Huda
AU - Greif, Joel
AU - Kornreich, Liora
AU - Ashkenazi, Shai
AU - Levy, Yael
AU - Schonfeld, Tommy
AU - Blau, Hannah
PY - 2000/2
Y1 - 2000/2
N2 - An infant with cystic fibrosis and her asthmatic father were diagnosed as suffering from allergic bronchopulmonary aspergillosis (ABPA). Cystic fibrosis was diagnosed in the infant at 6 weeks of age, and gene mutations were W1282X/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 youngest age described to date for ABPA. The child responded well to systemic steroid therapy, but remained steroid-dependent over the next 4 years. Treatment with itraconazole enabled a marked reduction in steroid dosage. The father was an asthmatic, and a heterozygote for the cystic fibrosis transmembrane 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 diagnosis of ABPA even in infancy, the therapeutic dilemmas, and the possible role of abnormal CFTR function in the development of ABPA are discussed.
AB - An infant with cystic fibrosis and her asthmatic father were diagnosed as suffering from allergic bronchopulmonary aspergillosis (ABPA). Cystic fibrosis was diagnosed in the infant at 6 weeks of age, and gene mutations were W1282X/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 youngest age described to date for ABPA. The child responded well to systemic steroid therapy, but remained steroid-dependent over the next 4 years. Treatment with itraconazole enabled a marked reduction in steroid dosage. The father was an asthmatic, and a heterozygote for the cystic fibrosis transmembrane 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 diagnosis of ABPA even in infancy, the therapeutic dilemmas, and the possible role of abnormal CFTR function in the development of ABPA are discussed.
KW - Allergic bronchopulmonary aspergillosis
KW - Asthma
KW - CFTR
KW - Cystic fibrosis
KW - Familial
KW - Infant
UR - http://www.scopus.com/inward/record.url?scp=0033968462&partnerID=8YFLogxK
U2 - 10.1002/(SICI)1099-0496(200002)29:2<155::AID-PPUL11>3.0.CO;2-H
DO - 10.1002/(SICI)1099-0496(200002)29:2<155::AID-PPUL11>3.0.CO;2-H
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AN - SCOPUS:0033968462
SN - 8755-6863
VL - 29
SP - 155
EP - 159
JO - Pediatric Pulmonology
JF - Pediatric Pulmonology
IS - 2
ER -