Nationwide survey of hereditary pancreatitis in Japan View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2017-08-31

AUTHORS

Atsushi Masamune, Kazuhiro Kikuta, Shin Hamada, Eriko Nakano, Kiyoshi Kume, Ayano Inui, Toshiaki Shimizu, Yoshifumi Takeyama, Masaki Nio, Tooru Shimosegawa

ABSTRACT

BackgroundHereditary pancreatitis (HP) is a rare cause of chronic pancreatitis. We here report a nationwide survey to clarify the epidemiological, genetic, and clinical features of HP in Japan.MethodsTarget subjects were patients with HP and their family members who had visited selected hospitals between 2005 and 2014. This study consisted of two-stage surveys; patients with HP were identified by the first questionnaire and their clinical features were assessed by the second questionnaire.ResultsTwo hundred seventy-one patients (153 males and 118 females) in 100 families diagnosed based on the Japanese criteria or 231 patients (131 males and 100 females) patients in 80 families based on the EUROPAC criteria were reported. Of the families undertaking genetic tests, 41% had the PRSS1 mutations (p.R122H 33%, p.N29I 8%) and 36% had the SPINK1 mutations (p.N34S 22%, c.194+2T>C 14%, p.P45S 1%). The mean age at symptom onset was 17.8 years. The cumulative rates of pancreatic exocrine insufficiency and diabetes mellitus were 16.1 and 5.5% at 20 years old, and 45.3 and 28.2% at 40 years, respectively. Forty-four percent of the patients underwent endoscopic treatment and/or surgery. The cumulative rate of pancreatic cancer diagnosis was 2.8% at 40 years old, 10.8% at 60 years, and 22.8% at 70 years.ConclusionsHP was characterized by early disease onset, frequent development of pancreatic exocrine insufficiency and diabetes mellitus, requirement of endoscopic treatment and/or surgery, and increased risk of pancreatic cancer. PRSS1 and SPINK1 mutations serve as genetic background for HP in Japan. More... »

PAGES

152-160

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00535-017-1388-0

DOI

http://dx.doi.org/10.1007/s00535-017-1388-0

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1091407733

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/28861620


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37 schema:description BackgroundHereditary pancreatitis (HP) is a rare cause of chronic pancreatitis. We here report a nationwide survey to clarify the epidemiological, genetic, and clinical features of HP in Japan.MethodsTarget subjects were patients with HP and their family members who had visited selected hospitals between 2005 and 2014. This study consisted of two-stage surveys; patients with HP were identified by the first questionnaire and their clinical features were assessed by the second questionnaire.ResultsTwo hundred seventy-one patients (153 males and 118 females) in 100 families diagnosed based on the Japanese criteria or 231 patients (131 males and 100 females) patients in 80 families based on the EUROPAC criteria were reported. Of the families undertaking genetic tests, 41% had the PRSS1 mutations (p.R122H 33%, p.N29I 8%) and 36% had the SPINK1 mutations (p.N34S 22%, c.194+2T>C 14%, p.P45S 1%). The mean age at symptom onset was 17.8 years. The cumulative rates of pancreatic exocrine insufficiency and diabetes mellitus were 16.1 and 5.5% at 20 years old, and 45.3 and 28.2% at 40 years, respectively. Forty-four percent of the patients underwent endoscopic treatment and/or surgery. The cumulative rate of pancreatic cancer diagnosis was 2.8% at 40 years old, 10.8% at 60 years, and 22.8% at 70 years.ConclusionsHP was characterized by early disease onset, frequent development of pancreatic exocrine insufficiency and diabetes mellitus, requirement of endoscopic treatment and/or surgery, and increased risk of pancreatic cancer. PRSS1 and SPINK1 mutations serve as genetic background for HP in Japan.
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43 schema:keywords ConclusionsHPS
44 HP
45 Japan
46 Japanese criteria
47 PRSS1
48 PRSS1 mutations
49 ResultsTwo hundred seventy
50 SPINK1 mutations
51 age
52 background
53 cancer
54 cancer diagnosis
55 cause
56 chronic pancreatitis
57 clinical features
58 criteria
59 cumulative rate
60 development
61 diabetes mellitus
62 diagnosis
63 disease onset
64 early disease onset
65 endoscopic treatment
66 exocrine insufficiency
67 family
68 family members
69 features
70 first questionnaire
71 frequent development
72 genetic background
73 genetic tests
74 hereditary pancreatitis
75 hospital
76 insufficiency
77 mean age
78 mellitus
79 members
80 mutations
81 nationwide survey
82 onset
83 pancreatic cancer
84 pancreatic cancer diagnosis
85 pancreatic exocrine insufficiency
86 pancreatitis
87 patients
88 patients patients
89 percent
90 questionnaire
91 rare cause
92 rate
93 requirements
94 risk
95 second questionnaire
96 seventies
97 study
98 subjects
99 surgery
100 survey
101 symptom onset
102 test
103 treatment
104 two-stage survey
105 years
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