title: International registries of families at high risk of pancreatic cancer.
abstract: OBJECTIVE
  To describe the need for multinational registries of families at high risk of pancreatic cancer and the issues surrounding identification of such families.
  
  
  RESULTS
  A consensus position was published describing surveillance of individuals at high risk of pancreatic cancer. Hereditary pancreatitis patients, people with Peutz-Jeghers syndrome, individuals with CDKN2A or BRCA1/2 mutations with a family history of pancreatic cancer and kindred with multiple pancreatic cancers were considered suitable for research-based screening. Mutations responsible for familial predisposition are mostly unknown, although BRCA2 mutations have been identified in some families and a mutation in the palladin gene has been shown to segregate with pancreatic cancer in one kindred. Specific autosomal dominant inheritance of pancreatic cancer risk seems to involve anticipation; this finding aids identification of families and determination of individual risk. Diabetes mellitus is an early symptom of pancreatic cancer, but recent publications suggest that it may not be a significant predisposing factor; this remains controversial. However, in the context of hereditary pancreatitis, diabetes probably does predispose to pancreatic cancer as shown in a recent description of French families.
  
  
  CONCLUSIONS
  Appropriate inclusion of patients within registries of high-risk families provides a framework for secondary screening and research on risk stratification and early tumorigenesis. and IAP.
authors.affiliation: Division of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. greenhaf@liv.ac.uk
authors.forename: William
authors.initials: W
authors.lastname: Greenhalf
authors.forename: Nuria
authors.initials: N
authors.lastname: Malats
authors.forename: Magnus
authors.initials: M
authors.lastname: Nilsson
authors.forename: Detlef
authors.initials: D
authors.lastname: Bartsch
authors.forename: John
authors.initials: J
authors.lastname: Neoptolemos
mesh_terms: D003920:Diabetes Mellitus; D005190:Family; D005819:Genetic Markers; D020022:Genetic Predisposition to Disease; D006801:Humans; D007391:International Cooperation; D009313:National Health Programs; D010190:Pancreatic Neoplasms / Q000150:complications / Q000235:genetics*; D010195:Pancreatitis / Q000150:complications / Q000235:genetics; D010375:Pedigree; D012042:Registries; D012306:Risk
chemical_list: D005819:Genetic Markers