Retrospective analysis of clinical status and lung function of adult patients with cystic fibrosis during 1993— 1997

Abstract

The aim of study was to describe clinical and functional characteristics of adult patients with cystic fibrosis, who underwent hospital and outpatient treatment in 1994— 1997. Seven subjects (2 males and 5 fem ales, aged 24.0±1.9 years) participated in the study. The diagnosis of cystic fibrosis was confirmed by positive sweat test in all the patients, 6 of them were heterozygous for DF 508 mutation (the most common disease-causing mutation in Russia). All of them underwent regular treatment in accordance with internertional guidlines. Body mass index (BM I) and lung function were evaluated during 1994— 1997. The evaluation of Schwachman score demonstrated mild to moderate clinical state of the patients in 1997 (the mean value was 57.4±1.5). There was a trend toward lower BMI in 1997 (17.5±1.2 kg/m at 1994 versus 17.2±1.4 k g /m² at 1997). The differences in lung function during 1994— 1997 years were not significant. The retrospective analysis of lung function and BMI in 1994 did not reveal any statistically significant changes the result, that could be attributed to regular treatment during 1994— 1997 years.