title: [Hereditary pancreatitis].
abstract: Hereditary pancreatitis (HP) is a rare, heterogeneous familial disease and should be suspected in any patient who has suffered at least two attacks of acute pancreatitis for which there is no underlying cause and unexplained chronic pancreatitis with a family history in a first- or second degree relative. with an early onset, mostly during childhood. Genetic factors have been implied in cases of familial chronic pancreatitis. The most common are mutations of the PRSS1 gene on the long arm of the chromosome 7, encoding for the cationic trypsinogen. The inheritance pattern is autosomal dominant with an incomplete penetrance (80%). The inflammation results in repeated DNA damage, error-prone repair mechanisms and the progressive accumulation of genetic mutations. Risk of pancreatic adenocarcinoma is a major concern of many patients with hereditary chronic pancreatitis, but the individual risk is poorly defined. Better risk models of pancreatic cancer in individual patients based on etiology of pancreatitis, family history, genetics, smoking, alcohol, diabetes and the patient's age are needed.
authors.affiliation: Military Institute of Medicine in Warsaw, Poland, Department of Gastroenterology, Central Clinical Hospital of the Ministry of National Defense.
authors.forename: Przemysław
authors.initials: P
authors.lastname: Dyrla
authors.affiliation: 7 Navy Hospital in Gdańsk, Poland, Department of Gastroenterology.
authors.forename: Tomasz
authors.initials: T
authors.lastname: Nowak
authors.affiliation: Military Institute of Medicine in Warsaw, Poland, Department of Gastroenterology, Central Clinical Hospital of the Ministry of National Defense.
authors.forename: Jerzy
authors.initials: J
authors.lastname: Gil
authors.affiliation: Military Institute of Medicine in Warsaw, Poland, Department of Gastroenterology, Central Clinical Hospital of the Ministry of National Defense.
authors.forename: Cezary
authors.initials: C
authors.lastname: Adamiec
authors.affiliation: Military Institute of Medicine in Warsaw, Poland, Department of Gastroenterology, Central Clinical Hospital of the Ministry of National Defense.
authors.forename: Mariusz
authors.initials: M
authors.lastname: Bobula
authors.affiliation: Military Institute of Medicine in Warsaw, Poland, Department of Endocrinology and Isotope Therapy, Central Clinical Hospital of the Ministry of National Defense.
authors.forename: Marek
authors.initials: M
authors.lastname: Saracyn
mesh_terms: D000368:Aged; D002648:Child; D020022:Genetic Predisposition to Disease; D006801:Humans; D008875:Middle Aged; D009154:Mutation; D010190:Pancreatic Neoplasms / Q000209:etiology; D050500:Pancreatitis, Chronic / Q000150:complications / Q000235:genetics* / Q000473:pathology; D014357:Trypsin / Q000235:genetics*
chemical_list: C489303:PRSS1 protein, human; D014357:Trypsin
keywords: alcoholic chronic pancreatitis (ACP); chronic pancreatitis; hereditary pancreatitis (HP); idiopathic chronic pancreatitis (ICP); pancreatic cancer