Advertisement

Mukoviszidose (Zystische Fibrose)

  • E. M. App

Zusammenfassung

Die Mukoviszidose oder zystische Fibrose („cystic fibrosis“; CF) ist eine autosomal rezessiv vererbte, progrediente Multiorganerkrankung aller exokrinen Drüsen. Sie ist die häufigste angeborene, frühletale Stoffwechselerkrankung der weißen Rasse mit einer Häufigkeit von 1: 2500 Geburten. In Deutschland gibt es derzeit etwa 8000 Mukoviszidosepatienten.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Literatur

  1. Andersen DH (1938) Cystic fibrosis of the pancreas and its relation to celiac disease. A clinical and pathological study. Am J Dis Child 56: 344–399.Google Scholar
  2. App EM, King M, Helfesrieder R, Köhler D, Matthys H (1990) Acute and long term amiloride inhalation in cystic fibrosis lung disease. Am Rev Respir Dis 141: 605–612.PubMedCrossRefGoogle Scholar
  3. App EM, Kieselmann R, Reinhardt D, Lindemann H, Dasgupta B, King M, Brand P (1998) Sputum rheology changes in cystic fibrosis lung disease following two different types of physiotherapy — Flutter vs autogenic drainage. Chest 114: 171–177.PubMedCrossRefGoogle Scholar
  4. App EM, Wunderlich MO, Lohse P, King M, Matthys H (1999) Oszillierende Physiotherapie bei Bronchialerkrankungen — rheolo-gischer und antientzündlicher Effekt. Pneumologie 53: 348–359.PubMedGoogle Scholar
  5. Bauernfeind A, Bertele RM, Harms K et al. (1987) Qualitative and quantitative microbiological analysis of sputa of 102 patients with cystic fibrosis. Infection 15: 270–277.PubMedCrossRefGoogle Scholar
  6. Fabel H, Smaczny CH (1993) Pulmonaler Verlauf der Mukoviszi-dose beim Erwachsenen. Atemw Lungenkr 19: 510–515.Google Scholar
  7. Faber S (1944) Pancreatic function and diseases in early life, V. Pathologic changes associated with pancreatic insufficiency in early life. Arch Path 37: 238–250.Google Scholar
  8. Fanconi G, Ühlinger E, Knauer C (1936) Das Coeliakiesyndrom bei angeborener zystischer Pankreasfibromatose und Bronchiek-tasien. Wiener Med Wochenschr 86: 753–756.Google Scholar
  9. Fitz-Simmons SC (1993) The changing epidemiology of cystic fibrosis. J Pediatr 122: 1–9.Google Scholar
  10. Fuchs HJ, Borowitz DS, Christiansen DH et al. (1994) Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group. N Engl J Med 331: 637–642.PubMedCrossRefGoogle Scholar
  11. Hardin DS (1997) Insulin resistance is associated with decreased clinical status in cystic fibrosis. J Pediatr 130: 948–956.PubMedCrossRefGoogle Scholar
  12. Høiby N, Koch C, Frederiksen B (1998) Cystic fibrosis. Nord Med 113: 328–330.PubMedGoogle Scholar
  13. Keicher U, Koletzko B, Reinhardt D (1995) Omega-3 fatty acids suppress the enhanced production of 5-lipoxygenase products from polymorph neutrophil granulocytes in cystic fibrosis. Eur J Clin Invest 25: 915–919.PubMedCrossRefGoogle Scholar
  14. Knowles MR, Gatzy J, Boucher RC (1981) Increased bioelectric potential differences across respiratory epithelial in cystic fibrosis. N Engl J Med 305: 1489–1498.PubMedCrossRefGoogle Scholar
  15. Köhler D, App EM, Egelseder A, Matthys H (1986) Unterschiede in der mukoziliären und Hustenclearance bei chronischer Bronchitis mit und ohne Mukoviszidose. Atemw Lungenkr 12: 358–361.Google Scholar
  16. Lanng S, Thorsteinsson B, Nerup J, Koch C (1994) Diabetes mellit-us in cystic fibrosis: effect of insulin therapy on lung function and infections. Acta Paediatr 83: 849–853.PubMedCrossRefGoogle Scholar
  17. Orenstein DM (1996) Cystic Fibrosis: a guide for patient and family. Lippincott-Raven, Philadelphia.Google Scholar
  18. Pavia D, Thomson ML, Clarke SW (1976) Enhanced clearance of secretions from the human lung after the administration of hypertonic saline aerosol. Am Rev Respir Dis 117: 199–203.Google Scholar
  19. Puchelle E, de Bentzmann S, Zahm JM (1995) Physical and functional properties of airway secretions in cystic fibrosis — therapeutic approaches. Respiration 62: 2–12.PubMedCrossRefGoogle Scholar
  20. Riedler J, Reade T, Button B, Robertson CF (1996) Inhaled hypertonic saline increases sputum expectoration in cystic fibrosis. J Paediatr Child Health 32: 48–50.PubMedCrossRefGoogle Scholar
  21. Rosenstein BJ, Cutting GR (1998) The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel. J Pediatr 132: 589–595.PubMedCrossRefGoogle Scholar
  22. Stern M, Sens B, Wiedemann B (1998) Qualitätssicherung Mukoviszidose — Probleme, Erfolge und Perspektiven 1995–1998. Z Ärztl Fortbild Qualitätssich 92: 513–519.PubMedGoogle Scholar
  23. Tsui LP (1999) (Selected Proceedings from the thirteenth Annual North America Cystic Fibrosis Conference, Seattle, October 7–10).Google Scholar
  24. Valerins NH, Koch C, Høiby N (1991) Prevention of chronic Pseu-domonas aeruginose colonistation in cystic fibrosis by early treatment. Lancet 338: 725–726.CrossRefGoogle Scholar
  25. Vic P, Ategbo S, Turck D et al. (1998) Efficacy, tolerance, and phar-macokinetics of once daily tobramycin for pseudomonas exacerbations in cystic fibrosis. Arch Dis Child 78: 536–539.PubMedCrossRefGoogle Scholar
  26. Welsh MJ (1999) (Selected Proceedings from the thirteenth Annual North America Cystic Fibrosis Conference, Seattle, October 7–10).Google Scholar
  27. Wood RE, Boat TF, Doershuk CF (1976) Cystic fibrosis. Am Rev Re-spir Dis 113: 833–878.Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2002

Authors and Affiliations

  • E. M. App

There are no affiliations available

Personalised recommendations