Clinical features of individuals with cystic fibrosis in New Zealand.

AIMS

To determine the status of cystic fibrosis patients, their mode of diagnosis and source of medical care in New Zealand.

METHODS

A database was established in 1988 to record clinical information on individuals with cystic fibrosis in New Zealand. Three hundred and two cystic fibrosis patients were identified and clinical information on 248 obtained.

RESULTS

Thirty five per cent of patients at diagnosis had gastrointestinal and pulmonary and 33% had gastrointestinal symptoms alone. Ninety three infants were diagnosed after a positive screening test and confirmatory sweat test. The mean age at diagnosis overall was 1.8 years, while 61% of children were diagnosed before one year of age. 74.2% of patients were said to be pancreatic insufficient, 4% pancreatic sufficient and in 22% the results were unavailable or unknown. Medical care was provided by 28 sources comprising paediatricians, physicians or combined clinics. One cystic fibrosis clinic cared for more than 40 patients. Sixty-one per cent of cystic fibrosis subjects were colonised with Staphylococcus aureus at some time, and 44% were reported to be colonised with Pseudomonas aeruginosa.

CONCLUSIONS

Common clinical features of cystic fibrosis have been characterised for New Zealand patients. It is unknown to what extent the clinical features of the disease and source of medical care in New Zealand are responsible for previously reported survival for the condition but it is planned to examine serial measurements of growth, nutrition, pulmonary function and survival and it is hoped that these measurements will be the subject of a further report.