Clinical features as predictors of functional status in children with cystic fibrosis.

Previous clinical studies in patients with cystic fibrosis have demonstrated substantial variability in the symptoms present at diagnosis and in subsequent survival rates. In this study we assessed the association between features present at diagnosis and the clinical course of cystic fibrosis in 89 patients. The 5- and 10-year outcomes for children with cystic fibrosis were better than has been generally appreciated. Overall, two thirds of the patients had either improved or remained at the same level of morbidity 5 years after diagnosis. Children who presented with isolated gastrointestinal symptoms had a good clinical course; some actually improved clinically during the first 5 to 10 years after diagnosis. In contrast, children who presented with respiratory disease frequently had clinical deterioration during the follow-up period. Neither age at presentation nor the initial level of morbidity was significantly related to subsequent outcome. We conclude that clinical features apparent at diagnosis are valuable prognostic indicators in children with cystic fibrosis.