Document Detail

Severe allergic bronchopulmonary aspergillosis in an infant with cystic fibrosis and her asthmatic father.
MedLine Citation:
PMID:  10639207     Owner:  NLM     Status:  MEDLINE    
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.
H Mussaffi; J Greif; L Kornreich; S Ashkenazi; Y Levy; T Schonfeld; H Blau
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatric pulmonology     Volume:  29     ISSN:  8755-6863     ISO Abbreviation:  Pediatr. Pulmonol.     Publication Date:  2000 Feb 
Date Detail:
Created Date:  2000-02-15     Completed Date:  2000-02-15     Revised Date:  2006-03-28    
Medline Journal Info:
Nlm Unique ID:  8510590     Medline TA:  Pediatr Pulmonol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  155-9     Citation Subset:  IM    
Copyright Information:
Copyright 2000 Wiley-Liss, Inc.
Sackler School of Medicine, Tel Aviv University, Ramat Aviv, Israel.
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MeSH Terms
Aspergillosis, Allergic Bronchopulmonary / complications*,  diagnosis,  drug therapy
Asthma / complications*
Child, Preschool
Cystic Fibrosis / complications*,  drug therapy,  genetics
Cystic Fibrosis Transmembrane Conductance Regulator / genetics
Reg. No./Substance:
0/CFTR protein, human; 126880-72-6/Cystic Fibrosis Transmembrane Conductance Regulator

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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