Unique presentations and chronic complications in adult cystic fibrosis: do they teach us anything about CFTR?

Respir Res. 2000;1(3):133-5. doi: 10.1186/rr23. Epub 2000 Nov 16.

Abstract

The increase in numbers of adults with cystic fibrosis (CF) has allowed us to identify previously unrecognized chronic complications of CF, as well as appreciate unique presentations of cystic fibrosis-related diseases. Do these chronic complications and unique presentations provide us with new insight into cystic fibrosis transmembrane conductance regulator (CFTR) function? Current data suggest that the 'chronic complications' reveal mainly the effect of a long-term absence of previously recognized CFTR functions. In contrast, the 'unique presentations' provide new insight into the role of CFTR in different tissues.

Publication types

  • Review

MeSH terms

  • Adult
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / physiopathology
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism*
  • Humans
  • Male
  • Pancreatitis / etiology
  • Paranasal Sinus Diseases / etiology
  • Vas Deferens / abnormalities

Substances

  • CFTR protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator