Abstract:
BACKGROUND:Hereditary 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. METHODS:Target 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. RESULTS:Two 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. CONCLUSIONS:HP 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.
journal_name
J Gastroenteroljournal_title
Journal of gastroenterologyauthors
Masamune A,Kikuta K,Hamada S,Nakano E,Kume K,Inui A,Shimizu T,Takeyama Y,Nio M,Shimosegawa Tdoi
10.1007/s00535-017-1388-0subject
Has Abstractpub_date
2018-01-01 00:00:00pages
152-160issue
1eissn
0944-1174issn
1435-5922pii
10.1007/s00535-017-1388-0journal_volume
53pub_type
杂志文章abstract::We report a 27-year-old male with Crohn's disease (CD) of the small and large intestine, whose peripheral blood lymphocytes (PBL) showed increased cell-mediated cytotoxicity (CTL). Autologous and allogeneic effector cells from PBL and intestinal lymph nodes (LN) were isolated on a Ficoll-Hypaque gradient. Colonic cell...
journal_title:Journal of gastroenterology
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pub_type: 杂志文章,多中心研究,随机对照试验
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