背侧胰腺发育不全(agenesis of the dorsal pancreas,ADP)是一种先天性胰腺发育畸形,其特征性表现为胰腺颈体尾部未发育或发育不全,起病隐匿,多数患者无临床症状,部分患者表现为腹部疼痛[1]。既往基因检测研究显示,胰腺发育不全可能与P...背侧胰腺发育不全(agenesis of the dorsal pancreas,ADP)是一种先天性胰腺发育畸形,其特征性表现为胰腺颈体尾部未发育或发育不全,起病隐匿,多数患者无临床症状,部分患者表现为腹部疼痛[1]。既往基因检测研究显示,胰腺发育不全可能与PDX1、PTF1A、GATA4、GATA6、HNF1A和HNF1B等基因突变相关[2]。展开更多
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 i...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.展开更多
文摘背侧胰腺发育不全(agenesis of the dorsal pancreas,ADP)是一种先天性胰腺发育畸形,其特征性表现为胰腺颈体尾部未发育或发育不全,起病隐匿,多数患者无临床症状,部分患者表现为腹部疼痛[1]。既往基因检测研究显示,胰腺发育不全可能与PDX1、PTF1A、GATA4、GATA6、HNF1A和HNF1B等基因突变相关[2]。
文摘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.
