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

Huda Mussaffi, Joel Greif, Liora Kornreich, Shai Ashkenazi, Yael Levy, Tommy Schonfeld, Hannah Blau*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

12 Scopus citations

Abstract

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.

Original languageEnglish
Pages (from-to)155-159
Number of pages5
JournalPediatric Pulmonology
Volume29
Issue number2
DOIs
StatePublished - Feb 2000

Keywords

  • Allergic bronchopulmonary aspergillosis
  • Asthma
  • CFTR
  • Cystic fibrosis
  • Familial
  • Infant

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