[Non-CF bronchiectasis of adults: short review for clinical practice Position paper of Board of disease with bronchial obstruction Czech Pulmonological and Phthiseological Society Czech Medical Association of J. E. Purkyne]. / Non-CF bronchiektázie dospelých: strucný prehled pro praxi Dokument Sekce chorob s bronchiální obstrukcí Ceské pneumologické a ftizeologické spolecnosti Ceské lékarské spolecnosti J. E. Purkyne.

Bronchiectasis is a clinically important, but poorly understood, pulmonary condition characterized by dilated and thick-walled bronchi. Bronchiectasis remains a significant cause of morbidity and mortality around the world. Targeted effort to early high-resolution computed tomography diagnosis and detailed confirmation of causation are in the spotlight of respiratory physicians in the developed countries. The risk population consists of subjects with persistent and/or productive cough, where another clear diagnosis has not been performed. Specific treatment tailored on underlying diseases and non-specific airway clearance techniques are able to improve symptoms, and reduce lung impairment. Evidence-based treatment algorithms for anti-inflammatory, and antibiotic treatment of stable non-CF BE will have to await large-scale, long-term controlled studies. Surgery should be reserved for individuals with highly symptomatic, localized bronchiectasis who have failed medical management. Unfortunately, there have been few well designed longitudinal or cross-sectional studies in the field of bronchiectasis. To give truly meaningful and generalizable results, a longitudinal observational study of bronchiectasis would require to enrol several thousand patients, more than any one center can enrol. The European Bronchiectasis Registry will create an open, pan-European registry of patients with non-CF bronchiectasis. The authors emphatically recommend that all respiratory specialist managed non-CF BE subjects should be actively involved in the European Bronchiectasis Registry.Key words: bronchiectasis - diagnosis - registry - treatment.

[Diagnostics of cystic fibrosis in adults]. / Diagnostika cystické fibrózy u dospelých.

INTRODUCTION: There is an increasing number of cystic fibrosis (CF) patients with the diagnosis established in adulthood worldwide. AIM: To give an overview of our experience with the diagnostics of CF in adulthood in the Czech Republic. METHODS: CF patients with the diagnosis determined at the age 18 years during 2000-2014 period were selected from the Czech Registry of CF (www.cfregistr.cz). Demographic and clinical data were reported from medi-cal records at the time of diagnosis and as of 31st December 2014. Only those with two CF causing mutation or with one CF causing mutation together with sweat chloride concentration > 60 mmol/l were included in the study. The clinical presentation was compared with a control group consisting of homozygous F508del patients with the diagnosis established in childhood. RESULTS: 23 patients (16 men and 7 women) with the diagnosis determined at a mean age of 32.9 ± 8.5 years were included in the study. Presenting symptoms included bronchiectasis and/or haemoptysis in 12 cases, obstructive azoospermia in 7 cases and recurrent pancreatitis in 4 cases. When compared with the control group, the patients had higher age (38.6 ± 8.3 vs. 28.3 ± 4.7 years; p < 0.001), a lower concentration of sweat chloride (62 ± 23 vs. 90 ± 12 mmol/l; p < 0.001), less frequent airway infections with Pseudomonas aeruginosa and/or Burkholderia cepacia complex (4 vs. 12; p = 0.029), bronchiectasis (14 vs. 23; p = 0.001), exocrine pancreatic insufficiency (1 vs. 23; p < 0.001) and therapy with insulin (1 vs. 9; p = 0.01); on the contrary, pancreatitis was more frequent (6 vs. 0; p = 0.022). CONCLUSION: Diagnosis of CF in adults should be considered in those with corresponding symptoms in respiratory, digestive and reproductive tract. Clinical presentation differs from classical CF in many parameters. KEY WORDS: adults - cystic fibrosis - diagnostics.