Aldosterone in cystic fibrosis: measurement in saliva and correlation with disease severity.

Aldosterone was measured in the saliva of 20 patients with cystic fibrosis and a group of 20 normal children matched for age and sex. Mean levels were higher in the patient group but overall differences were small and statistically not significant. For the first time a link between aldosterone level and disease severity in patients with cystic fibrosis was established using a simple scoring system to assess disease activity. Urinary and salivary electrolytes were similar in the two groups. The results do not support the proposed hypothesis that the salivary glands of patients with cystic fibrosis are insensitive to aldosterone.

Measurement of osmolality and sodium concentration in heated-cup sweat collections for the investigation of cystic fibrosis.

A new system (Wescor) for sweat collection and analysis was examined with respect to its suitability for the investigation of children suspected to have cystic fibrosis. The effects of iontophoresis current, sweat collection time, sweat storage and analysis were examined, and as a result the technique was modified to allow collection and storage of sufficient sweat for sodium and potassium as well as osmolality assays in 10-20 minutes. The small electrodes and speed of the procedure make it practical for use with small children, with a reproducibility of 13-24% (coefficient of variation for whole procedure).

Ceftazidime in cystic fibrosis: clinical, microbiological and immunological studies.

We have investigated the use of the new cephalosporin ceftazidime for the treatment of pseudomonas infection in cystic fibrosis, using 100 to 240 mg/kg intravenously daily. The clinical and microbiological results of 18 courses of therapy, lasting from 1 to 4 weeks have been satisfactory, particularly since the patients had previously proved refractory to treatment with most other appropriate antibiotics. However, in common with other anti-pseudomonal antibiotics, a first course of ceftazidime proved the most successful, subsequent courses being less effective. Three patients died because of chronic respiratory infection. In cystic fibrosis pseudomonal infections rapidly relapse after therapy and therefore we have been examining the interactions of antibiotics with relevant host defence mechanisms. In one patient who received a 4 g infusion of ceftazidime there was enhancement of neutrophil phagocytosis-triggered chemiluminescence. We conclude that, used in high doses, ceftazidime is a useful addition to the available antibiotics for therapy of pseudomonas infection in cystic fibrosis.

Serum alphafetoprotein in cystic fibrosis of the pancreas.

Serum alphafetoprotein concentrations were measured by three different types of radioimmunoassay in 30 patients with cystic fibrosis of the pancreas and in 55 controls. The highest value obtained in cystic patient was 10.2 ng/ml and in a control 10.8 ng/ml. These are within published normal limits. Previously reported large increases in serum AFP in patients with cystic fibrosis and in heterozygote carriers have not been confirmed.

Tobramycin therapy of infections due to Pseudomonas aeruginosa in patients with cystic fibrosis: effect of dosage and concentration of antibiotic in sputum.

Established respiratory infections with mucoid Pseudomonas aeruginosa in patients suffering from cystic fibrosis were treated with conventional as well as larger doses of tobramycin. The infection was eradicated in four of the 17 patients treated, but the duration of follow-up study of one patient was short. It appeared that treatment was most successful in those patients in whom the highest peak concentrations of tobramycin in sputum were obtained. No side effects were noted, even when tobramycin was given in large doses for two weeks.

Leucocyte function in a case of chronic benign neutropenia of infancy associated with circulating leucoagglutinins.

A case of chronic benign neutropenia is described in association with circulating leucoagglutinins. IgG and IgM leucoagglutinins demonstrated by Sephadex G-200 chromatography agglutinated neutrophils and monocyte enriched leucocyte preparations. Total IgG, IgA, IgM and IgE, total haemolytic complement, C3 and C4 complement components were all within normal limits. Lymphocyte populations and transformation were normal apart from a relative increase in the number of B lymphocytes. In vitro studies showed that the patient's monocytes responded in chemotaxis, phagocytosis and intracellular killing of S. aureus in a comparable fashion to that of control neutrophils. Although the patient's serum also agglutinated monocytes these findings do not exclude the possibility that leucoagglutinins may have aetiological significance in this disease. The study also emphasizes the versatility of the monocyte in benign neutropenia.

Neglected coeliac disease.

A review has been carried out of patients diagnosed as having coeliac disease some years previously and subsequently lost to follow-up. Most were unaware of the need for continuing treatment and had returned to a normal diet. The resulting morbidity was slight, although one patient had died of a small-bowel lymphoma. If untreated coeliac disease is indeed a pre-malignant condition, then it is suggested that there must be a large population at risk, with no motivation to return to treatment other than the risk of malignancy itself.