Classes of skin fibroblast metachromasia in cystic fibrosis correlated with clinical parameters.

A retrospective study of clinical and radiological lung symptoms and intensity of 
treatment of 98 patients with cystic fibrosis was conducted. The results 
were correlated to class of skin fibroblast metachromasia. Fourteen patients belonged 
to class I (vesicular metachromasia), 52 to class II (generalized metachromasia), 
and 32 to class III (no metachromasia). All patients above 16 
years of age (six patients) were class II. Simple scoring systems 
for clinical lung symptoms and for treatment of lung disease were 
developed. When compared at the same ages, class I and III 
patients were more severely affected (judged by the clinical lung symptoms) 
than class II patients, and they had been more intensively treated. 
Comparing class I + II patients (metachromatic fibroblasts) with class III 
patients (ametachromatic fibroblasts), it was found that class III patients were 
more severely affected and had been more intensively treated than class 
I + II patients. As regards chest radiographs (judged by Norman's 
system) a similar pattern was seen, but no statistically significant differences 
were found. In 68 patients (9 of class I, 35 of 
class II, and 24 of class III) the serum protein electrophoretic 
fractions were studied. It was found that class III patients had 
more severe alterations than class II patients, and more than class 
I + II patients. The proportion of female patients in class 
III was greater than in the other two classes, but this 
could not explain the differences. Because of the small number of 
patients in class I, the differences between class I and class 
II patients are more doubtful than the differences between class III 
and class II patients, or between class III and class I 
+ II patients. 