Lungenveränderungen bei Cystischer Fibrose

Zusammenfassung

Die Cystische Fibrose (CF) ist die häufigste letal verlaufende autosomal rezessive Erkrankung unter Kaukasiern. Obwohl CF eine Multiorganerkrankung ist, ist der lebenslimitierende Faktor in mehr als 90% der Fälle die Lungenbeteiligung. In den letzten drei Jahrzehnten hat sich unter gewandeltem Therapieregime das Bild der pulmonalen Beteiligung bei CF im Kindesalter erheblich verändert. Das Ergebnis der intensivierten, auf frühe Behandlung bedachten Therapie ist u. a., dass heute mehr als 50% der Patienten älter als 18 Jahre sind. Die diagnostischen und therapeutischen Möglichkeiten sind weit gefächert und einer ständigen Weiterentwicklung unterworfen. Die bereits in Entwicklung stehenden diagnostischen und therapeutischen Optionen versprechen eine noch wesentlich weitere Verbesserung der Lebenserwartung und Lebensqualität für Menschen mit CF.

Summary

CF is the most frequent lethal autosomal recessive disease in Caucasians. CF is a multi-organ disease. Nevertheless more than 90% of deaths are related to pulmonary involvement. During the last three decades the new treatment regimes resulted in a dramatically changed clinical picture of CF in childhood. The earlier and more intensive treatment was accompanied by an enormous increase in survival. Today more than 50% of all CF patients are older than 18 years. The diagnostic and therapeutic possibilities are in a continuous process of development. That way we can expect that the life expectancy and quality of life will increase over the next years.

Schlüsselwörter

Lungenbeteiligung - Überleben - Therapieintensität

Keywords

Lung involvement - survival - intensity of treatment

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