Cystic fibrosis: a cell culture study on an adult patient population

Abstract

Skin fibroblast cultures from an adult population of cystic fibrosis (CF) patients have been studied to determine any possible genetic or clinical significance of the two classes described for cultured CF skin fibroblasts (Danes 1973). On the basis of clinical course, the 46 adult patients from 43 unrelated families studied were divided into two groups (40 typical, 6 atypical). The cultured fibroblasts from 37 of the 40 patients with typical CF were Class I (metachromatic, cystic fibrosis factor activity (CFFA) in the culture medium, and metabolic cooperation with normal fibroblasts), and the cultures from the remaining three patients were Class II (ametachromatic, no CFFA in the culture medium and no metabolic cooperation with metachromatic CF fibroblasts). During the 4-year research period one of the 37 patients from Class I and all three Class II patients with typical CF succumbed. The cultured skin fibroblasts from two of the six patients who had an atypical course for CF were Class I and four were Class II. When the parents of three of these atypical CF Class II patients were studied, the cultured fibroblasts from one parent in each family in each family were Class I and the other Class II. The affected offspring from such Class I-II matings may represent genetic compounds.