Article
Pancreatic hemi-agenesis in MEN1: A clinical report.
European journal of medical genetics - 1 Apr 2018
Vinck Wouter J, Van de Mierop Frank, Van Mieghem François, Vinck Maarten, Becq Herman, Michils Geneviève
Abstract excerpt
We first describe a patient with multiple endocrine neoplasia type 1 (MEN1) and dorsal pancreatic hemi-agenesis. Previously, pancreas divisum has been reported in MEN1. Recent data in mice have elucidated the molecular mechanisms of pancreatic endoderm specification. Disinhibition of hedgehog signaling appears to be important in how Gata4 and Gata6 variants cause pancreatic agenesis. Disinhibition of hedgehog...
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