Survival rates in cystic fibrosis 835 Survival rates in cystic fibrosis

SUMMARY Life tables were calculated for 273 British children with cystic fibrosis for the period 1974-9. There was a marked improvement in survival rates in the meconium ileus group com-pared with the 1969-73 data, but there was little improvement in patients presenting later with other symptoms. Cystic fibrosis (CF) is the most common lethal inherited disease that affects white populations. Several studies have used actuarial life table analysis, and it has been noted that survival rates have improved markedly in the last 20-30 years.1 2 3 We compared recent survival rates with those in our earlier life tables. ' 4 We also examined whether mortality rates were greater among girls and patients with meconium ileus (MI). Patients and methods We studied the 273 patients attending the CF clinic at this hospital between 1 January 1974 and 31 December 1979. These patients had typical symp-toms of CF such as recurrent pulmonary infection, failure to thrive, nasal polyps, rectal prolapse, and MI, together with at least two sweat sodium con-centrations greater than 60 mmol (mEq)/l on samples weighing 100 mg or more. An exception was made for babies with MI who died before having a sweat test. They were included in the analysis if the pathological findings at necropsy were con-sistent with CF. Surgical admission records and necropsy records for the period of study were examined to ensure that no such patients were omitted. Data obtained by reviewing the patients ' medical records were recorded on punched cards for com-puter analysis. Life tables were constructed and survival curves plotted with the Survival procedure of the Statistical Package for the Social Science