Forty patients with cystic fibrosis and chronic broncho-pulmonary Pseudomonas aeruginosa infection entered a prospective double-blind placebo-controlled study of colistin inhalation. Active treatment consisted of inhalation of colistin one million units twice daily for three months and was compared to placebo inhalations of isotonic saline. Significantly more patients in the colistin inhalation group completed the study as compared to the placebo group (18 versus 11). Colistin treatment was superior to placebo treatment in terms of a significantly better clinical symptom score, maintenance of pulmonary function and inflammatory parameters. We recommend colistin inhalation therapy for cystic fibrosis patients with chronic P. aeruginosa lung infection as a supplementary treatment to frequent courses of intravenous anti-pseudomonas chemotherapy.

In order to compare the efficacy of pancreatic enzyme supplementation as pH-sensitive enteric-coated microspheres Pancrease to that of conventional supplementation with enteric-coated Pancreatin in cystic fibrosis, a double blind cross-over study was conducted. Eleven patients under 12 years of age received each of the enzyme preparations for four weeks. Treatment efficacy was evaluated by means of a symptom score card recording stool frequency, consistency, colour, odour, abdominal cramps and appetite as well as a 3 days fat absorption test. Weight increments were recorded 3 months before the study when patients were on Pancreatin, and 3 months after the study when patients were on Pancrease. In eight of the patients fat absorption was improved on Pancrease, but the difference did not reach statistical significance. However, the patients experienced significantly less dyspeptic symptoms, decreased stool frequency, better appetite and increments in weight were significantly higher on Pancrease compared to Pancreatin.

Islet cell cytoplasmic antibodies were determined in 85 individuals 60 to 74 years old with fasting hyperglycaemia, in 65 patients with cystic fibrosis, in 113 patients with pancreatitis, in 21 patients with Turner's phenotype, and in 135 first-degree relatives of patients with Type 1 (insulin-dependent) diabetes. Islet cell antibodies were absent in all 60 to 74-year-old subjects with fasting hyperglycaemia detected by screening, and who did not require insulin treatment within 3 years. Islet cell antibodies were also absent in all patients with pancreatitis, cystic fibrosis, or Turner's phenotype. Islet cell antibodies were detected in 2 out of 135 (1.5%) first-degree relatives of new Type 1 diabetic patients, and in 1 out of 371 (0.3%) non-diabetic control subjects. During 12 years of follow-up 1 of the 2 first-degree relatives with islet cell antibodies and the only positive control developed Type 1 diabetes. It is suggested that islet cell antibodies are primarily associated with Type 1 diabetes and not with other disorders of glucose tolerance.

The effect of peroral N-acetylcysteine (NAC) in patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) was investigated. 41 CF patients and 13 PCD patients completed the study which was a double-blind, placebo-controlled, cross-over trial. The patients received either NAC or placebo for two periods of three months followed by a three month follow-up period. Active treatment consisted of NAC, either 200 mg x 3 daily (patients weighing less than 30 kg) or 400 mg x 2 daily (greater than 30 kg). The effect was evaluated in terms of a subjective clinical score, weight, sputum bacteriology, blood leucocyte count, sedimentation rate, titres of specific antimicrobial antibodies, lung function parameters and measurement of the ciliary function. No effect was seen in PCD patients, but in CF patients an improved lung function was seen in the period when the patients suffer most from lower airway infections.