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KMID : 0605920060120010024
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Journal of Korean Association of Pediatric Surgeons
2006 Volume.12 No. 1 p.24 ~ p.31
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Hereditary Pancreatitis: Report of a Kindred
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Kim Hyoung-Ran
Chung Jae-Hee
Song Young-Tack
Yoon Won-Jae
Ryu Ji-Kon
Kim Yong-Tae
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Abstract
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Hereditary pancreatitis (HP) appears as an autosomal dominant trait. If the patient has (1) more than 2 affected relatives in different generations and (2) no known etiological factors such as alcohol or gallstones, or has R122H or N29I mutation in the cationic trypsinogen (CT) gene, the diagnosis of HP can be applied. Risk of pancreatic cancer is estimated to be 53-fold higher than in a general population after the age of 50 years. We report a kindredof HP, involving three of its family together; two siblings (14 years old, 13 years old) and cousin (26 years old). The patient had complicating chronic pancreatitis and pancreatic stone, and was treated with amodified Puestow-Gillesby procedure. Her sisters showed chronic pancreatitis. Her cousin underwent a drainage procedure of the pancreatic duct for chronic pancreatitis during the high school period. All the three members showed the R122H mutation of the CT gene.
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KEYWORD
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Hereditary pancreatitis, Pancreatic stone, Surgery, Cationic trypsinogen gene
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