Long-Term Study of One Hundred Five Patients with Cystic Fibrosis

TLDR

Survival of cystic fibrosis patients beyond childhood is increasing due to earlier diagnosis and effective therapies, yet long‑term outcomes for large cohorts have not been reported. The study introduces a clinical evaluation system to compare patients. The authors developed a clinical evaluation tool that quantifies patient status for inter‑patient comparison. No long‑term outcome data exist for large cystic fibrosis cohorts, despite the largest survivor cohort reported by McIntosh. Other: The abstract mentions additional points but provides no further details.

Abstract

The survival beyond childhood of patients with cystic fibrosis is being noted with increasing frequency. This is explained by improved diagnostic methods, with the early recognition of mild cases, and by the application of effective therapeutic measures. The largest number of survivors beyond the age of 10 included in one report is in that of McIntosh.¹However, there have been no reports dealing with the results of long-term care provided to a large group of patients with cystic fibrosis. It is obvious that the end-results of such a study would reflect diagnostic acumen, for the earlier the case is recognized and the milder the disease the better should be the end-result. It is for this reason that we have introduced a system of clinical evaluation which can be used as a measuring device to compare one patient with the next. It is well, also, to point out some of