Risk of Pancreatic Adenocarcinoma in Patients With Hereditary Pancreatitis: A National Exhaustive Series View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2008-01-01

AUTHORS

Vinciane Rebours, Marie-Christine Boutron-Ruault, Matthieu Schnee, Claude Férec, Frédérique Maire, Pascal Hammel, Philippe Ruszniewski, Philippe Lévy

ABSTRACT

BACKGROUND AND AIMS: An increased risk of pancreatic adenocarcinoma (PA) in patients with hereditary pancreatitis (HP) was previously demonstrated in two multinational studies. The PA frequency in this setting is however unknown due to lack of exhaustive case collection. The aims of this study were to evaluate the standardized incidence ratio (SIR) of PA in an exhaustive national series of patients with HP and to search for risk factors. METHODS: All French genetic laboratories (response rate 100%), pediatricians, and gastroenterologists (response rate 84%) were contacted. INCLUSION CRITERIA: mutation in the PRSS1 gene or recurrent, acute, or chronic pancreatitis, with no precipitating factors in two first-degree relatives or >or=3 second-degree relatives in >or=2 generations. Diagnosis of PA was based on histological records. RESULTS: Seventy-eight families and 200 patients were included (181 alive, 6,673 person-years, median number of generations 3, men 53%, alcoholism 5%, and smoking 34%). PRSS1 mutations were searched for in 96% of the patients and were detected in 68% (maternal inheritance 54%, R122H 78%, N29I 12%, and others 10%). Ten PA were diagnosed (median age 55 yr). SIR of PA for the whole population, men, and women were 87 (95% CI 42-113), 69 (25-150), and 142 (38-225), respectively, with no influence of genetic mutation. At ages 50 and 75 yr, the cumulated risk of PA was 11% and 49% for men and 8% and 55% for women, respectively. Smoking and diabetes mellitus were the main associated risk factors. CONCLUSION: Patients with HP have a marked relative and absolute increased risk of PA as compared to the general population, especially in smokers. There is no correlation with the type of PRSS1 mutation. More... »

PAGES

ajg20085018

References to SciGraph publications

Identifiers

URI

http://scigraph.springernature.com/pub.10.1111/j.1572-0241.2007.01597.x

DOI

http://dx.doi.org/10.1111/j.1572-0241.2007.01597.x

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1041187812

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/18184119


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34 schema:description BACKGROUND AND AIMS: An increased risk of pancreatic adenocarcinoma (PA) in patients with hereditary pancreatitis (HP) was previously demonstrated in two multinational studies. The PA frequency in this setting is however unknown due to lack of exhaustive case collection. The aims of this study were to evaluate the standardized incidence ratio (SIR) of PA in an exhaustive national series of patients with HP and to search for risk factors. METHODS: All French genetic laboratories (response rate 100%), pediatricians, and gastroenterologists (response rate 84%) were contacted. INCLUSION CRITERIA: mutation in the PRSS1 gene or recurrent, acute, or chronic pancreatitis, with no precipitating factors in two first-degree relatives or >or=3 second-degree relatives in >or=2 generations. Diagnosis of PA was based on histological records. RESULTS: Seventy-eight families and 200 patients were included (181 alive, 6,673 person-years, median number of generations 3, men 53%, alcoholism 5%, and smoking 34%). PRSS1 mutations were searched for in 96% of the patients and were detected in 68% (maternal inheritance 54%, R122H 78%, N29I 12%, and others 10%). Ten PA were diagnosed (median age 55 yr). SIR of PA for the whole population, men, and women were 87 (95% CI 42-113), 69 (25-150), and 142 (38-225), respectively, with no influence of genetic mutation. At ages 50 and 75 yr, the cumulated risk of PA was 11% and 49% for men and 8% and 55% for women, respectively. Smoking and diabetes mellitus were the main associated risk factors. CONCLUSION: Patients with HP have a marked relative and absolute increased risk of PA as compared to the general population, especially in smokers. There is no correlation with the type of PRSS1 mutation.
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42 National Exhaustive Series
43 PA frequency
44 PRSS1 gene
45 PRSS1 mutations
46 SIR of PA
47 adenocarcinoma
48 age 50
49 aim
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51 case collection
52 chronic pancreatitis
53 collection
54 correlation
55 diabetes mellitus
56 diagnosis
57 diagnosis of PDAC
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61 factors
62 family
63 first-degree relatives
64 frequency
65 gastroenterologists
66 general population
67 generation
68 genes
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71 hereditary pancreatitis
72 histological records
73 incidence ratios
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78 men
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84 patients
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94 second-degree relatives
95 series
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