Source: Annual Congress 2009 - Cystic fibrosis: advances in clinical research

Abstract

Introduction: Cystic fibrosis (CF) is the most common inherited cause of bronchiectasis in white populations. Progress in diagnostic testing has expanded the spectrum of disease phenotypes, including less severe dysfunctions manifestations that only becoming evident in adulthood. Few studies have compared these two different CF populations.
Objective and Methods: The aim of this retrospective study was to describe clinical and functional characteristics and the microbiological profile of patients with CF, comparing differences between patients whose diagnosis was made in childhood (less than 16 years old) or adulthood (more than 16 years old).
Results: Nineteen patients had CF diagnosis before 16 years old and 17 after. Data of both groups are quite similar (Table 1).

Differences between CF early and late diagnosis

	Early diagnosis	Late diagnosis
Cough	73.7%	88.2%
Wheezing	21.1%	31.3%
Hemoptysis	15.8%	5.9%
Pancreatic disorder	57.9%	55.6%
Diabetes melittus	26.3%	16.7%
Domiciliary oxygen therapy	36.8%	0*
Decrease in FEV1	0.11 ± 0.4 mL/year	-0.08 ± 0.3 mL/year
Pseudomonas aeruginosa	83.3%	81.3%
Staphylococcus aureus	77.8%	50.0%

*p<0.05; FEV1 = forced expiratory volume in the first second

Conclusions: In disagreement with literature data, our late diagnosis patients do not have a milder lung or pancreatic involvement, nor have less frequent pseudomonal colonization. This may be related to delayed diagnosis or even genotype variations. Cystic fibrosis should be considered as a potential diagnosis in any adult patient with bronchiectasis.

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Citations should be made in the following way:
R. Athanazio, S. Rached, S. Azevedo, R. Carvalho-Pinto, A. Cuckier, R. Stelmach (São Paulo, Brazil). Comparison of clinical features in patients with cystic fibrosis according to the age at diagnosis. Eur Respir J 2009; 34: Suppl. 53, 1260

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