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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Agenesis of the dorsal pancreas


Other Names for this Disease
  • Complete agenesis of the dorsal pancreas
  • Congenital short pancreas
  • Pancreas agenesis, dorsal
  • Pancreas, dorsal, agenesis of
  • Partial agenesis of the dorsal pancreas
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Cause


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What causes agenesis of the dorsal pancreas?

Partial or complete agenesis of the dorsal pancreas results from the failure of the dorsal pancreatic bud to form the body and tail of the pancreas in the developing fetus.[1] It may occur from the absence, or regression of, the dorsal bud during fetal development. Heredity may play a role in the development of this condition, but further research is needed to clarify this.[1]

There have been reports in the literature of the condition being associated (rarely) with other congenital diseases, specifically a very rare disorder called polysplenia/heterotaxy syndrome. In this case, it may occur due to errors in development of the asymmetric organs and may be associated with benign to severe congenital cardiac (heart) malformations.[2]
Last updated: 7/22/2011

References
  1. Joo YE, Kang HC, Kim HS, Choi SK, Rew JS, Chung MY, Kim SJ. Agenesis of the dorsal pancreas: a case report and review of the literature. Korean J Intern Med. December 2006; 21(4):236-239. http://www.kjim.or.kr/upload/abstract/files/062104236.pdf. Accessed 7/22/2011.
  2. Suraj Kapa, Ferga C Gleeson, Santhi Swaroop Vege. Dorsal Pancreas Agenesis and Polysplenia/Heterotaxy Syndrome: A Novel Association with Aortic Coarctation and a Review of the Literature. JOP. J Pancreas (Online). 2007; 8(4):433-437. http://www.joplink.net/prev/200707/200707_02.pdf. Accessed 7/22/2011.