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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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Overview

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.[1][2][3] Hyperglycemia has been shown to be present in approximately 50% of affected individuals.[3] 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.[4][5] It has also been reported to occur with very rare conditions including polysplenia and polysplenia/heterotaxy syndrome.[6]
Last updated: 7/22/2011

References

  1. Whitcomb DC, Lowe ME. Hereditary, Familial, and Genetic Disorders of the Pancreas and Pancreatic Disorders in Childhood. In: Feldman. Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 9th ed. Philadelphia, PA: Saunders; 2010;
  2. Schnedl WJ, Piswanger-Soelkner C, Wallner SJ, Reittner P, Krause R, Lipp RW, Hohmeier HE. Agenesis of the dorsal pancreas and associated diseases. Dig Dis Sci. 2009 Mar;
  3. Wolfgang J Schnedl, Claudia Piswanger-Soelkner, Sandra J Wallner, Robert Krause, Rainer W Lipp. Agenesis of the dorsal pancreas. World Journal of Gastroenterology. January 2009; 15(3):376-377. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2653338/pdf/WJG-15-376.pdf. Accessed 7/22/2011.
  4. Joanna S. Amberger . Pancreas, Dorsal, Agenesis of. OMIM. May 10, 2011; http://omim.org/entry/167755. Accessed 7/22/2011.
  5. 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.
  6. 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.
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Basic Information

In Depth Information

  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Agenesis of the dorsal pancreas. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

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
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.