[The effect of ambulatory nutritional intervention using defined enteral nutrition on the nutritional status of selected adult patients with cystic fibrosis]. / Vliv ambulantní nutricní intervence prostrednictvím definované enterální výzivy na nutricní stav vybraných dospelých nemocných s cystickou fibrózou.

The prevalence of mild and serious nutritional disorders in patients with cystic fibrosis is high. One of the possible ways how to supplement the necessary nutrients is the administration of defined supplementary enteral nutrition. The objective of the presented work is to evaluate the effect of supplementary domiciliary enteral nutrition in adult patients with cystic fibrosis. To eight patients with cystic fibrosis, mean body weight 51.77 +/- 10.63 kg, mean age 24.7 +/- 1.9 years for a period of 6 +/- 1 months enteral nutrition was administered (containing 40 and 55.1% energy as fat) with a caloric density of 10 kcal/kg as supplementary domiciliary nutrition. The mean body weight increased from 51.77 +/- 10.63 to 53.13 +/- 10.59 kg (p < 0.02), the mean skinfold thickness increased from 7.98 +/- 6.04 to 8.65 +/- 6.30 mm (p < 0.05) and the mean serum albumin concentration increased from 33.66 +/- 0.97 to 37.29 +/- 3.33 g/l (p < 0.05). No undesirable side-effect associated with the administration of enteral nutrition were observed. The nutritional intervention by domiciliary enteral nutrition produced no undesirable side-effects and led to a statistically significant improvement of parameters of the nutritional status.

[10 years' of care of adult patients with cystic fibrosis]. / Deset let péce o dospelé nemocné s cystickou fibrózou.

The authors summarized the results of ten years care of adult patients with cystic fibrosis (CF). They treated a total of 45 patients incl. 26 men and 19 women. Nineteen patients (42%) died. The most frequent cause of admission to hospital was respiratory infection (84%), planned antibiotic treatment (6.5%), pneumothorax (6%) and haemoptysis (4%). In all patients impaired ventilation of the obstructive type was found. The mean annual decline of FEV1 was 0.173 +/- 0.194 l, VC 0.303 +/- 0.323 l. The authors did not find a significant difference between live and dead patients. The mean annual increase of PaCO2 was 0.5 +/- 0.87 kPa, the mean annual decrease of PaO2 was 0.6 +/- 0.96 kPa. The difference of values in dead and live patients was significant. CF still remains an incurable disease. It is, however, possible by comprehensive treatment to prolong the patients life and to improve its quality.