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Chronic pancreatitis and cystic fibrosis
  1. H Witt
  1. Correspondence to:
    Dr H Witt, Kinderklinik, Charité-Campus Virchow-Klinikum, Humboldt-Universität, Augustenburger Platz 1, D-13353 Berlin, Germany;
    heiko.witt{at}charite.de

Abstract

Recent discoveries of trypsinogen and trypsin inhibitor mutations in patients with chronic pancreatitis (CP) support the hypothesis that an inappropriate activation of pancreatic zymogens to active enzymes within the pancreatic parenchyma starts the inflammatory process. Current data suggest that CP may be inherited dominant, recessive, or complex as a result of mutations in the above mentioned or yet unidentified genes. Evaluation of patients with CP should include genetic testing. Cystic fibrosis (CF) is an autosomal recessive inherited disorder caused by mutations in the CF transmembrane conductance regulator (CFTR) gene and is characterised by pancreatic insufficiency and chronic bronchopulmonary infection. The progression and severity of pulmonary disease differs considerably between people with identical CFTR mutations and does not seem to correlate with the type or class of the CFTR mutation. The identification of further disease modifying genetic factors will increase the pathophysiological understanding and may help to identify new therapeutic targets.

  • pancreatitis
  • cystic fibrosis
  • CP, chronic pancreatitis
  • CF, cystic fibrosis
  • CFTR, cystic fibrosis transmembrane conductance regulator
  • MHC, major histocompatibility complex
  • TNFα, tumour necrosis factor α
  • TGFβ1, transforming growth factor β1
  • GSTM1, glutathione S transferase M1
  • NO, nitric oxide
  • MBL, mannose binding lectin
  • CBAVD; congenital bilateral absence of the vas deferens
  • ABPA, allergic bronchopulmonary aspergillosis

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Footnotes

  • This paper is dedicated to Michael Becker on his 59th anniversary.