Agenesis of the dorsal pancreas  

作　　者：Wolfgang J Schnedl Claudia Piswanger-Soelkner Sandra J Wallner Robert Krause Rainer W Lipp 

机构地区：[1]Practice for General Internal Medicine,Hauptstrasse 5,A-8940 Liezen,Austria [2]Department of Internal Medicine,Medical University,Auenbruggerplatz 15,A-8036 Graz,Austria [3]Institute of Pathophysiology,Center for Molecular Medicine,Heinrichstrasse 31a,A-8010 Graz,Austria

出　　处：《World Journal of Gastroenterology》2009年第3期376-377,共2页世界胃肠病学杂志（英文版）

摘　　要：During the last 100 years in medical literature,there are only 54 reports,including the report of Pasaoglu et al(World J Gastroenterol 2008;14:2915-2916),with clinical descriptions of agenesis of the dorsal pancreas in humans.Agenesis of the dorsal pancreas,a rare congenital pancreatic malformation,is associated with some other medical conditions such as hyperglycemia,abdominal pain,pancreatitis and a few other diseases.In approximately 50% of reported patients with this congenital malformation,hyperglycemia was demonstrated.Evaluation of hyperglycemia and diabetes mellitus in all patients with agenesis of the dorsal pancreas including description of fasting blood glucose,oral glucose tolerance test,glycated hemoglobin and medical treatment would be a future goal.Since autosomal dominant transmission has been suggested in single families,more family studies including imaging technologies with demonstration of the pancreatic duct system are needed for evaluation of this disease.With this letter to the editor,we aim to increase available information for the better understanding of this rare disease.During the last 100 years in medical literature, there are only 54 reports, including the report of Pasaoglu et al （World J Gastroenterol 2008; 14： 2915-2916）, with clinical descriptions of agenesis of the dorsal pancreas in humans. Agenesis of the dorsal pancreas, a rare congenital pancreatic malformation, is associated with some other medical conditions such as hyperglycemia, abdominal pain, pancreatitis and a few other diseases. In approximately 50% of reported patients with this congenital malformation, hyperglycemia was demonstrated. Evaluation of hyperglycemia and diabetes mellitus in all patients with agenesis of the dorsal pancreas including description of fasting blood glucose, oral glucose tolerance test, glycated hemoglobin and medical treatment would be a future goal. Since autosomal dominant transmission has been suggested in single families, more family studies including imaging technologies with demonstration of the pancreatic duct system are needed for evaluation of this disease. With this letter to the editor, we aim to increase available information for the better understanding of this rare disease.

关 键 词：Agenesis of dorsal pancreas DIABETESMELLITUS Glucose intolerance Abdominal pain PANCREATITIS 

分 类 号：R657.5[医药卫生—外科学]