Very high risk of cancer in familial Peutz-Jeghers syndrome.

  title={Very high risk of cancer in familial Peutz-Jeghers syndrome.},
  author={Frank M Giardiello and Jill D. Brensinger and Anne C. Tersmette and Steven N. Goodman and Gloria M. Petersen and Susan V. Booker and Marcia Roxana Cruz-Correa and Johan G. A. Offerhaus},
  volume={119 6},
BACKGROUND & AIMS The Peutz-Jeghers syndrome (PJS) is an autosomal dominant polyposis disorder with increased risk of multiple cancers, but literature estimates of risk vary. METHODS We performed an individual patient meta-analysis to determine the relative risk (RR) of cancer in patients with PJS compared with the general population based on 210 individuals described in 6 publications. RESULTS For patients with PJS, the RR for all cancers was 15.2 (95% confidence limits [CL], 2, 19). A… 

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