| The novel triad of dorsal agenesis of the pancreas with concurrent pancreatic ductal adenocarcinoma and nonalcoholic chronic calcific pancreatitis: a case series and review of the literature. | |
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MedLine Citation:
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PMID: 21512846 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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INTRODUCTION: Dorsal agenesis of the pancreas (DAP) is a rare congenital anomaly, with only 44 cases having been reported in the English literature since 1966. MATERIALS AND METHODS: A retrospective review of our IRB-approved pancreatic surgery database was performed from November 2005 to November 2010 searching for cases of DAP. DISCUSSION: Disorders in the retinoic acid (Raldh) and hedgehog (Hh) signaling pathways, which appear to play a role in the development of DAP, have been implicated in other diseases of the pancreas such as pancreatic ductal adenocarcinoma (PDA) and nonalcoholic chronic calcific pancreatitis (NCCP). CONCLUSION: In this report, we describe three cases of DAP in the setting of PDA, two of which include the third component of NCCP. We provide a discussion of the clinical features of this novel triad and address the molecular pathways that relate to these respective diseases. |
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Authors:
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David W Rittenhouse; Eugene P Kennedy; Andres A Mascaro; Jennifer L Brumbaugh; Louis H Stein; Laura H Rosenberger; Patricia K Sauter; Charles J Yeo; Harish Lavu |
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Publication Detail:
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Type: Case Reports; Journal Article; Review Date: 2011-04-22 |
Journal Detail:
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Title: Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract Volume: 15 ISSN: 1873-4626 ISO Abbreviation: J. Gastrointest. Surg. Publication Date: 2011 Sep |
Date Detail:
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Created Date: 2011-08-23 Completed Date: 2012-01-23 Revised Date: 2012-05-16 |
Medline Journal Info:
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Nlm Unique ID: 9706084 Medline TA: J Gastrointest Surg Country: United States |
Other Details:
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Languages: eng Pagination: 1643-9 Citation Subset: IM |
Affiliation:
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Department of Surgery, Thomas Jefferson University, Jefferson Pancreas, Biliary and Related Cancer Center, Philadelphia, PA 19107, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adenocarcinoma
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complications*,
diagnosis,
genetics,
surgery Adult Aged Animals Congenital Abnormalities / diagnosis*, genetics Female Hedgehog Proteins / genetics Humans Male Middle Aged Pancreas / abnormalities Pancreatic Neoplasms / complications*, diagnosis, genetics, surgery Pancreatitis, Chronic / complications*, diagnosis, genetics, surgery Retinal Dehydrogenase / genetics Signal Transduction / genetics |
| Grant Support | |
ID/Acronym/Agency:
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T32 AI078875/AI/NIAID NIH HHS; T32 AI078875-02/AI/NIAID NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Hedgehog Proteins; EC 1.2.1.36/ALDH1A2 protein, human; EC 1.2.1.36/Retinal Dehydrogenase |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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