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Renal-pancreatic-hepatic dysplasia in siblings |
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| Authors: | Douglas L. Blowey Bradley A. Warady David L. Zwick Catherine Ong |
| Affiliation: | (1) Division of Nephrology, The Children's Mercy Hospital, University of Missouri at Kansas City, 2401 Gillham Road, 64108 Kansas City, Missouri, USA;(2) Division of Pathology, The Children's Mercy Hospital, University of Missouri at Kansas City, 2401 Gillham Road, 64108 Kansas City, Missouri, USA;(3) Division of Cardiology, The Children's Mercy Hospital, University of Missouri at Kansas City, 2401 Gillham Road, 64108 Kansas City, Missouri, USA |
| Abstract: | Fewer than 20 children with complete renal-pancreatic-hepatic dysplasia have been reported since first described in 1959. We report two brothers with renal-pancreatic-hepatic dysplasia, one of whom had hypertrophic cardiomyopathy and pancreatic exocrine insufficiency, previously unreported associated findings. |
| Keywords: | Renal-pancreatic-hepatic dysplasia Cystic kidneys Biliary dysgenesis Pancreatic cysts Hypertrophic cardiomyopathy |
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