Cystic fibrosis in adolescents and adults.

Forty-five patients (25 male and 20 female) over 12 years of age 
with cystic fibrosis have been studied clinically, radiologically and physiologically. Their 
mean age at the first visit was 17 years; they were 
followed for a mean period of 4 years and attended at 
least every six months. The first symptom which developed before the 
age of five in 42 of the 45 patients was respiratory. 
Thirty-two of the 45 patients had severe lung disease (Group III) 
at the start of the study of the seven patients died 
during the study. Cough and sputum were almost universal, 23 had 
haemoptyses and eight pneumothoraces. Staphylococcus pyogenes, Haemophilus influenzae and Pseudomonas aeruginosa 
were the common pathogens isolated from sputum and the increasing prevalence 
of the latter was again confirmed. Acquisition of the mucoid strain 
of pseudomonas signified poor prognosis. Established infection was never eradicated. Forty-three 
patients had evidence of pancreatic insufficiency; in all but one patient 
the symptoms were mild and five patients abandoned dietary restriction and 
pancreatin without ill effect. Seven patients had symptoms of partial bowel 
obstruction (meconium ileus equivalent) but only one required surgical relief. The 
liver was enlarged in seven patients and the spleen was felt 
in three. Three patients had diabetes mellitus. The influence of cystic 
fibrosis on growth and development is reported--the growth spurt is late 
in the majority but growth failure is not confined to those 
with severe lung infection or malabsorption and in these circumstances remains 
unexplained. Mean weight was low in relation to height and puberty 
was delayed in both sexes. 