Learn About Agenesis of the Dorsal Pancreas

What is the definition of Agenesis of the Dorsal Pancreas?
Agenesis of the dorsal pancreas describes a congenital malformation of the pancreas in which either the entire dorsal pancreas or part of the dorsal pancreas fails to develop (complete agenesis or partial agenesis, respectively). Some individuals experience no symptoms, while others may develop hyperglycemia, diabetes mellitus, bile duct obstruction, abdominal pain, pancreatitis, or other conditions. Hyperglycemia has been shown to be present in approximately 50% of affected individuals. The cause of Agenesis of the dorsal pancreas is currently not well understood. It may occur in individuals with no history of the condition in the family (sporadically) and in some cases, autosomal dominant or X-linked dominant inheritance has been suggested. It has also been reported to occur with very rare conditions including polysplenia and polysplenia/heterotaxy syndrome.
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What are the alternative names for Agenesis of the Dorsal Pancreas?
  • Agenesis of the dorsal pancreas
  • Complete agenesis of the dorsal pancreas
  • Congenital pancreatic agenesis
  • Congenital short pancreas
  • Pancreas agenesis, dorsal
  • Pancreas, dorsal, agenesis of
  • Partial agenesis of the dorsal pancreas
  • Partial pancreatic agenesis
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Published Date: May 02, 2022
Published By: Genetic and Rare Diseases Informnation Center

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