Renal-pancreatic-hepatic dysplasia in siblings
- 14 Downloads
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.
Key wordsRenal-pancreatic-hepatic dysplasia Cystic kidneys Biliary dysgenesis Pancreatic cysts Hypertrophic cardiomyopathy
Unable to display preview. Download preview PDF.
- 1.Ivemark BI, Oldfelt V, Zetterstrom R (1959) Familial dysplasia of kidneys, liver and pancreas. Acta Paediatr 48: 1–11Google Scholar
- 9.Plauchu H, Bouvier R, Rebaud MF, Rebaud A, Rebert JM, Salle B, Mellier G (1990) Ivemark's renal-hepatic-pancreatic dysplasia. Variation of pathological manifestations in a family. Proceedings of the First European Meeting of Dysmorphology. Zaragosa I: p 18Google Scholar
- 12.Krick J (1986) Using the Z score as a descriptor of discrete changes in growth. Nutrition support services 6: 14–21Google Scholar