Academic Editor: John Bose
Nikola
Bakovića, Ivana Pantica, Ivana Vasiljevica,
Marko Vojnovica, Milica Stojadinovicc, Tamara Milovanovicab
aClinic for
Gastroenterology and Hepatology, University Clinical Center of Serbia, 11000 Belgrade,
Serbia
bFaculty of Medicine, University of Belgrade, 11000 Belgrade,
Serbia
cCenter for Radiology, University Clinical
Center of Serbia, 11000 Belgrade, Serbia
Corresponding Author: Tamara Milovanovic, Clinic for
Gastroenterology and Hepatology, University Clinical Center of Serbia, 11000
Belgrade, Serbia.
Citation: Nikola Baković, Ivana Pantica, Ivana
Vasiljevic, Marko Vojnovic, Milica Stojadinovic, Tamara Milovanovic (2025) From
Device to Disease: Tricuspid Valve Replacement in a Patient with Copper T
associated Endocarditis and Thrombotic Thrombocytopenic Purpura. World J Case
Rep Clin Imag. 2025 May-June; 4(1)1-5.
Copyrights: © Tamara
Milovanovic, et al., 2025, This article is licensed
under the Creative Commons Attribution-NonCommercial-4.0-International-License-(CCBY-NC)
(https://worldjournalofcasereports.org/blogpage/copyright-policy). Usage and
distribution for commercial purposes require written permission.
Abstract
Background:
Dorsal agenesis of the pancreas is a rare congenital disorder characterized by
the absence of the body, tail and ventrosuperior part of the head of the
pancreas. Magnetic Resonance Cholangiopancreatography is the primary diagnostic
method to confirm diagnosis, and, in that case pancreatic duct is not visible. Summary:
We presented two cases of dorsal pancreatic agenesis associated with
cholestatic syndrome. Both cases are young adults with cholestatic type of the
liver function tests abnormalities, without symptoms and radiographically
confirmed with pancreatic agenesis. Viral markers and autoantibodies suggestive
of liver disease were negative, alpha-1 antitrypsin levels were normal, and
there were no criteria for the diagnosis of Wilson’s disease. Further
examination confirmed the presence of an HNF1B mutation in both patients. A
literature review revealed two cases where dorsal agenesis of pancreas was
associated with cholestatic syndrome, both had mutation of HNF1B. Besides liver
disease, such patients are prone to kidney disease and diabetes mellitus.
Key Messages:
The aim of the -article is to raise awareness about this rare syndrome, and
help identifying this frequently overlooked patient subpopulation.
Keywords: Dorsal Agenesis of the Pancreas, Cholestatic Syndrome, HNF1B Mutation
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