CYSTIC-FIBROSIS IN ADOLESCENTS AND ADULTS

Abstract

Forty-five patients (25 male and 20 female) over 12 yr of age with cystic fibrosis were studied clinically, radiologically and physiologically. Their mean age at the 1st visit was 17 yr; they were followed for a mean period of 4 yr and attended at least every 6 mo. The 1st symptom which developed before the age of 5 in 42 of the 45 patients was respiratory. Of the 45 patients, 32 had severe lung disease (Group III) at the start of the study and 7 patients died during the study. Cough and sputum were almost universal; 23 had hemoptyses and 8 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 1 patient the symptoms were mild and 5 patients abandoned dietary restriction and pancreatin without ill effect. Seven patients had symptoms of partial bowel obstruction (meconium ileus equivalent) but only 1 required surgical relief. The liver was enlarged in 7 patients and the spleen was felt in 3. 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.