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Pol. Merkur. Lek (Pol. Med. J.), 2016, XL/236: 113-116 Maximize

Pol. Merkur. Lek (Pol. Med. J.), 2016, XL/236: 113-116

Title: Hereditary pancreatitis 

Authors: Dyrla P, Nowak T, Gil J, Adamiec C, Bobula M, Saracyn M. 

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SUMMARY IN POLISH & ENGLISH. FULL ARTICLE ONLY IN POLISH.

Hereditary pancreatitis


Dyrla P1, Nowak T2, Gil J1, Adamiec C1, Bobula M1, Saracyn M3.

1Military Institute of Medicine in Warsaw, Poland, Department of Gastroenterology, Central Clinical Hospital of the Ministry of National Defense; 27 Navy Hospital in Gdańsk, Poland, Department of Gastroenterology; 3Military Institute of Medicine in Warsaw, Poland, Department of Endocrinology and Isotope Therapy, Central Clinical Hospital of the Ministry of National Defense

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 seconddegree 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 repairmechanisms 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.

Key words: hereditary pancreatitis (HP), chronic pancreatitis, idiopathic chronic pancreatitis (ICP), alcoholic chronic pancreatitis (ACP), pancreatic cancer

Pol Med J, 2016; XL (236); 113–116