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Background: Peutz-Jeghers syndrome (PJS) is associated with an increased cancer risk. As the determination of optimal surveillance strategies is hampered by wide ranges in cancer risk estimates and lack of data on cancer-related mortality, we assessed cancer risks and mortality in a large cohort of patients with PJS., Methods: Dutch PJS patients were included in this cohort study. Patients were followed prospectively between January 1995 and July 2009, and clinical data from the period before 1995 were collected retrospectively. Data were obtained by interview and chart review. Cumulative cancer risks were calculated by Kaplan-Meier analysis and relative cancer and mortality risks by Poisson regression analysis., Results: We included 133 PJS patients (48% males) from 54 families, contributing 5004 person-years of follow-up. 49 cancers were diagnosed in 42 patients (32%), including 25 gastrointestinal (GI) cancers. The median age at first cancer diagnosis was 45 years. The cumulative cancer risk was 20% at age 40 (GI cancer 12%), increasing to 76% at age 70 (GI cancer 51%). Cumulative cancer risks were higher for females than for males (p=0.005). The relative cancer risk was higher in PJS patients than in the general population (HR 8.96; 95% CI 6.46 to 12.42), and higher among female (HR 20.40; 95% CI 13.43 to 30.99) than among male patients (HR 4.76; 95% CI 2.82 to 8.04). 42 patients had died at a median age of 45 years, including 28 cancer-related deaths (67%). Mortality was increased in our cohort compared to the general population (HR 3.50; 95% CI 2.57 to 4.75)., Conclusions: PJS patients carry high cancer risks, leading to increased mortality. The malignancies occur particularly in the GI tract and develop at young age. These results justify surveillance in order to detect malignancies in an early phase to improve outcome.

Background and Study Aim: Double-balloon enteroscopy (DBE) has proven to be a relatively safe method for small-bowel evaluation, with a complication rate of 1 %. The main concern after diagnostic DBE is acute pancreatitis. Single-balloon enteroscopy (SBE) has emerged as a viable alternative to DBE. Until now, no incidence of pancreatitis has been reported for SBE. The aims were to evaluate complication rate and occurrence of hyperamylasemia and to identify the risk factors for hyperamylasemia after SBE., Patients and Methods: Prospectively, consecutive patients undergoing peroral ("proximal") or combined approach SBE were included. Complications were assessed at 1 and 30 days afterwards. Serum amylase and C-reactive protein (CRP) were assessed immediately before and 2 - 3 hours after SBE., Results: 166 SBE procedures were performed in 105 patients (53-male; mean age 51 years, range 9 - 87). The indications for SBE were: anemia (n = 55), Crohn's disease (n = 31) and abdominal complaints suspicious for inflammatory bowel disease (n = 5), Peutz-Jeghers syndrome (n = 1) and other (n = 13). Therapeutic interventions were performed during 21 procedures (13 %). One perforation (1 / 21 therapeutic interventions, 4.8 %) occurred after dilation of a benign stricture. While 13 patients (16 %) had post-SBE hyperamylasemia, none had complaints suggesting acute pancreatitis. Factors such as sex, indication, procedure duration, number of passes, route of SBE, findings, and/or treatment showed no significant correlation with presence of hyperamylasemia., Conclusions: SBE appears to be a safe diagnostic endoscopic procedure. The incidence of hyperamylasemia and pancreatitis after peroral SBE seems comparable to that after DBE., (Georg Thieme Verlag KG Stuttgart.New York.)