Mukoviszidose - eine multidisziplinäre Herausforderung

Zusammenfassung

Die zystische Fibrose (CF) als eine der häufigsten autosomal rezessiv vererbbaren Erkrankungen stellt nach dem enormen Wissenszuwachs über die molekularen und pathophysiologischen Hintergründe dieser Stoffwechselkrankheit in den letzten 15 Jahren mittlerweile eine Herausforderung für viele medizinische, klinische und wissenschaftliche Disziplinen dar. Neben dem Pädiater sind der Pneumologe, Gastroenterologe, Chirurg, Orthopäde, Gynäkologe und HNO-Arzt für eine adäquate medizinische Versorgung der Patienten notwendig. Einen ebenso hohen Stellenwert in der umfassenden Betreuung der Patienten spielen die Disziplinen Ernährungsberatung, Physiotherapie, Psychologie und Sozialarbeit. Die Grundlagenforschung bezieht Mediziner, Biologen und Physiologen mit ein. Eine optimale Betreuung von CF-Patienten sollte von spezialisierten Zentren übernommen werden. Im vorliegenden Artikel wird der derzeitige Stand der Erforschung, medizinischen Behandlung und Betreuung von Betroffenen der zystischen Fibrose zusammengefasst.

Cystic Fibrosis - A Multidisciplinary Challenge

Cystic fibrosis, one of the most common autosomal recessive diseases, poses a formidable challenge to praetitioners in the clinical and scientific disciplines, in part due to the complexity of the advances made in molecular biology and pathophysiology over the past fifteen years. Patients with CF require a multidisciplinary medical team including paediatricians, pulmonologists, gastroenterologists, surgeons, orthopedists, gynaecologists and otolaryngologists. Comprehensive care of these patients also requires input from nutritionists, physical therapists, psychologists and social workers. Basic research in cystic fibrosis is promoted by clinicians, biologists and physiologists. The care for patients with cystic fibrosis should be performed at specialised centres. This article reviews the current state of research on CF and medical treatment and psychosocial care of these patients.

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