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The critical first six months in cystic fibrosis: A syndrome of severe bronchiolitis

✍ Scribed by D. Katznelson; A. Szeinberg; A. Augarten; Y. Yahav

Publisher: John Wiley and Sons
Year: 1997
Tongue: English
Weight: 18 KB
Volume: 24
Category: Article
ISSN: 8755-6863
DOI: 10.1002/(sici)1099-0496(199708)24:2<134::aid-ppul12>3.0.co;2-3

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✦ Synopsis

The syndrome of infantile bronchiolitis in cystic fibrosis (CF) carries a high mortality. Fifteen cases of CF encountered over the past 19 years with severe bronchiolitis with onset during the first 6 months of life are described. Treatment include steroids in high doses. All patients recovered. Further progress resembled the usual natural course of CF and showed no evidence of persisting lung damage. The mechanism of this syndrome is not clear and is probably dependent on many factors involved in early lung disease in CF. The frequency of severe bronchiolitis in cystic fibrosis may not be high, but it continues to be seen in clinical practice today.

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We report the clinical features of 21 unrelated cystic fibrosis (CF) patients from Portugal and Spain, who carry the mutation R1066C in the CFTR gene. The current age of the patients was higher in the R1066C/any mutation group (P < 0.01), as compared to the aF508/aF508 group. Poor values for lung ra