Congenital pancreatic hypoplasia: a syndrome of exocrine and endocrine pancreatic insufficiency |
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| Authors: | W E Winter N K Maclaren W J Riley P P Toskes J Andres A L Rosenbloom |
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| Affiliation: | 1. Department of Internal Medicine I, University Hospital “Carl Gustav Carus,” TU Dresden, Dresden, Germany;2. Clinical Trials Unit, German Bone Marrow Donor Center (DKMS), Dresden, Germany;3. Institute of Pathology, Faculty of Medicine and University Hospital “Carl Gustav Carus,” TU Dresden, Dresden, Germany;4. National Center for Tumor Diseases (NCT), Partner Site Dresden, Germany: German Cancer Research Center (DKFZ), Heidelberg, Germany |
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| Abstract: | We describe two brothers with small size at birth, early-onset insulin-dependent diabetes, and pancreatic exocrine insufficiency. In contrast to the findings in pancreatic aplasia, their serum C-peptide and glucagon levels were measurable. These findings, in concert with their clinical courses, are consistent with the diagnosis of congenital pancreatic hypoplasia. |
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