[Improved prognosis for patients with cystic fibrosis. A result of aggressive center-based treatment]. / Forbedret prognose for patienter med cystisk fibrose. Et resultat of aggressiv centerbehandling.

We report survival data for Danish centre-treated cystic fibrosis (CF) patients, covering the period 1974-1993 using cross-sectional cumulative survival probability based on annual age-specific mortality rates. No significant differences were noted in the survival probability when patients were grouped according to sex or absence/presence of meconium ileus. The annual mortality rate for 1989-1993 was 0-1.2%. Using the age-specific mortality rate for 1989-1993, we were unable to calculate the median survival probability because the curve did not fall below 50% (age up to 45 years). It was, however, possible to show that the survival probability for a CF child born after 1989 to reach his or hers 45th birthday was 80.4% (95% confidence interval 76.5-84.6%). The probability of surviving 40 years after the diagnosis of CF is made was 83.3% (95% confidence interval 80.1%-86.6%). This is considerably higher than any other published survival probability. An aggressive anti-Pseudomonas aeruginosa treatment regimen seemed important in achieving the observed improved survival.

[Glucose tolerance in patients with cystic fibrosis]. / Glukosetolerance hos patienter med cystisk fibrose.

In order to define prevalence and incidence of diabetes mellitus in cystic fibrosis, we followed 191 unselected patients above two years of age (median 13.6) in a five-year prospective study with annual oral glucose tolerance tests. The prevalence of diabetes increased from 11 to 24% during the study period with an annual age-dependent incidence rate of 4-9%. Diabetes was diagnosed at a median age of 21 years (range 3-40). At diagnosis of diabetes, hyperglycaemia, fasting hyperglycaemia (> or = 7.8 mmol/l), and increased haemoglobin Alc levels (> 6.4) were present in 33%, 16% and 16% of the diabetic patients, respectively. Impaired glucose tolerance implied a higher risk than normal glucose tolerance for the development of diabetes (odds ratio 5.6). In 58% of cases with impaired glucose tolerance, however, glucose tolerance was normalised at the next annual test. Normal glucose tolerance was found in only 37% of the patients at all five tests. Within this group of patients, median fasting and two-hour post-load plasma glucose concentrations and haemoglobin Alc levels increased by 6-8% during five years. Thus, the prevalence and incidence of diabetes in patients with cystic fibrosis is very high and increases with age. Since symptoms of hyperglycaemia and increased fasting plasma glucose and haemoglobin Alc levels are inconstant findings in newly diagnosed diabetic cystic fibrosis patients, we recommend annual oral glucose tolerance tests in all cystic fibrosis patients above the age of 10 years.

[Reduced concentration of insulin-like growth factor I in patients with cystic fibrosis]. / Nedsatte koncentrationer af insulin-like growth factor I hos patienter med cystisk fibrose.

Cystic fibrosis is frequently accompanied by a catabolic condition with low body mass index caused by a number of disease complications. Insulin-like growth factor I (IGF-I) is an anabolic hormone and an important marker of nutritional status, liver function and linear growth. Using radioimmunoassay we measured IGF-I once in 235 of our 240 patients (114 males, 121 females, median age 16.2 years, range 0.1-44.0 years). IGF-I was significantly reduced compared with a healthy Scandinavian control population: Mean (-2SD to +2SD) IGF-I-SD score -0.97 (-3.7 to 1.7) in males and -0.67 (-3.2 to 1.9) in females, both values were significantly different from zero (p < 0.001). Height SD score was -0.95 (-3.3 to 1.4) in males and -0.81 (-3.2 to 1.6) in females (p < 0.001). The low IGF-I concentrations may reflect the catabolic state of many patients with cystic fibrosis and play a part in their abnormal growth pattern.