Skip to main content
SpringerLink
Log in

Zystische Fibrose

  • Chapter
Praxis der Hepatologie

Zusammenfassung

Bei der zystischen Fibrose (CF, auch Mukoviszidose genannt) handelt es sich um eine autosomalrezessiv vererbte Multiorganerkrankung, bei der die Epithelzellmembranen defekte Chloridkanäle aufweisen. Ursächlich ist ein Gendefekt im CF-Transmembran-Regulator-(CFTR-)Protein.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 79.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 99.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Similar content being viewed by others

Literatur

  • Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein RC, Uluer AZ, Aitken ML, Freedman SD, Rose LM, Mayer-Hamblett N, Dong Q, Zha J, Stone AJ, Olson ER, Ordoñez CL, Campbell PW, Ashlock MA, Ramsey BW (2010) Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med 363(21):1991–2003

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  • Adler FR, Aurora P, Barker DH, Barr ML, Blackwell LS, Bosma OH, Brown S, Cox DR, Jensen JL, Kurland G, Nossent GD, Quittner AL, Robinson WM, Romero SL, Spencer H, Sweet SC, van der Bij W, Vermeulen J, Verschuuren EA, Vrijlandt EJ, Walsh W, Woo MS, Liou TG (2009) Lung transplantation for cystic fibrosis. Proc Am Thorac Soc 6(8):619–633

    Article  PubMed  PubMed Central  Google Scholar 

  • Boyle MP (2010) Cystic fibrosis: year in review. Curr Opin Pulm Med 16(6):583

    Article  PubMed  Google Scholar 

  • Davies JC, Alton EW, Bush A (2007) Cystic fibrosis. BMJ 335(7632):1255–1259

    Article  PubMed  PubMed Central  Google Scholar 

  • Davis PB (2006) Cystic fibrosis since 1938. Am J Respir Crit Care Med 173:475

    Article  PubMed  Google Scholar 

  • De Boeck K, Wilschanski M, Castellani C, Taylor C, Cuppens H, Dodge J, Sinaasappel M, Diagnostic Working Group (2006) Cystic fibrosis: terminology and diagnostic algorithms. Thorax 61(7):627–635

    Article  PubMed  PubMed Central  Google Scholar 

  • De Meester J, Smits JM, Persijn GG, Haverich A (2001) Listing for lung transplantation: life expectancy and transplant effect, stratified by type of end-stage lung disease, the Eurotransplant experience. J Heart Lung Transplant 20(5):518–524

    Article  PubMed  Google Scholar 

  • Elkins MR, Robinson M, Rose BR, Harbour C, Moriarty CP, Marks GB, Belousova EG, Xuan W, Bye PT, National Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group (2006) A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 354(3):229–240

    Article  PubMed  CAS  Google Scholar 

  • Gelfond D, Borowitz D (2012) Gastrointestinal complications of cystic fibrosis. Clin Gastroenterol Hepatol. pii: S 1542–3565(12)01308–0

    Google Scholar 

  • Gibson RL, Burns JL, Ramsey BW (2003) Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med 168(8): 918–951

    Article  PubMed  Google Scholar 

  • Ooi CY, Dupuis A, Ellis L, Jarvi K, Martin S, Gonska T, Dorfman R, Kortan P, Solomon M, Tullis E, Durie PR (2012) Comparing the American and European diagnostic guidelines for cystic fibrosis: same disease, different language? Thorax 67(7):618–624. doi:10.1136/thoraxjnl-2011-201454

    Article  PubMed  Google Scholar 

  • Pletz MW, Sauer-Heilborn A, Köhnlein T, Seidler U, Lamprecht G (2010) Cystic fibrosis in adults. Internist (Berl) 51(Suppl 1):277–288. doi:10.1007/s00108-009-2527-3

    Article  Google Scholar 

  • S1-Leitlinie Mukoviszidose (Cystische Fibrose): Ernährung und exokrine Pankreasinsuffizienz der Gesellschaft für Pädiatrische Gastroenterologie und Ernährung (GPGE). In: AWMF online (Stand 2011)

    Google Scholar 

  • Scaparrotta A, Di Pillo S, Attanasi M, Consilvio NP, Cingolani A, Rapino D, Mohn A, Chiarelli F (2012) Growth failure in children with cystic fibrosis. J Pediatr Endocrinol Metab 25(5–6):393–405

    PubMed  Google Scholar 

  • Scheid P, Kempster L, Griesenbach U, Davies JC, Dewar A, Weber PP, Colledge WH, Evans MJ, Geddes DM, Alton EW (2001) Inflammation in cystic fibrosis airways: relationship to increased bacterial adherence. Eur Respir J 17(1):27–35

    Article  PubMed  CAS  Google Scholar 

  • Sharp JK, Rock MJ (2008) Newborn screening for cystic fibrosis. Clin Rev Allergy Immunol 35(3):107–115. doi:10.1007/s12016-008-8082-1

    Article  PubMed  Google Scholar 

  • Smaczny C, Born T, Wagner TO (2012) Emergencies in adult mucoviscidosis patients. Internist (Berl) 53(5):575–584

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Klinik für Gastroenterologie, Hepatologie und Endokrinologie, Medizinische Hochschule Hannover, Hannover, Deutschland

    Andrej Potthoff

Authors
  1. Andrej Potthoff
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Andrej Potthoff .

Editor information

Editors and Affiliations

  1. Abt. Gastroenterologie Hepatologie und Endokrinologie Medizinische Hochschule Hannover (MHH), Zentrum Innere Medizin, Hannover, Deutschland

    Michael P. Manns

  2. Klinik für Gastroenterologie Hepatologie und Endokrinologie, Medizinische Hochschule Hannover, Hannover, Deutschland

    Sabine Schneidewind

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Potthoff, A. (2016). Zystische Fibrose. In: Manns, M., Schneidewind, S. (eds) Praxis der Hepatologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-41620-0_23

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-41620-0_23

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-41619-4

  • Online ISBN: 978-3-642-41620-0

  • eBook Packages: Medicine (German Language)

Publish with us

Policies and ethics

Search

Navigation