Renal, pancreatic and hepatic dysplasia sequence

✍ Scribed by D. Carles; F. Serville; J. P. Dubecq; J. M. Gonnet

• Book ID: 104786999
• Publisher: Springer
• Year: 1988
• Tongue: English
• Weight: 450 KB
• Volume: 147
• Category: Article
• ISSN: 0340-6997
• DOI: 10.1007/bf00496429

✦ Synopsis

A renal, pancreatic and hepatic dysplasia sequence (RPHD sequence) was found in a male premature baby who died a few minutes after birth. Autopsy documented multicystic dysplastic kidneys, a dysplastic pancreas with dilated ducts, cysts, fibrosis and inflammatory infiltrates, prominent portal tracts containing dilated bile ducts and hypoplastic lungs. Other organs were normal. This triad constitutes a "dysplastic sequence" and was first reported by Ivemark et al. as "familial dysplasia of kidneys, liver and pancreas". Since then, this combination of abnormalities has been named "polycystic dysplasia" and "renal-hepatic-pancreatic dysplasia", but mostly "Ivemark syndrome", at the risk of being confused with asplenia-cardiac anomaly syndrome, which was reviewed by Ivemark et al. and also bears Ivemark's name.