ISSN:
1432-1076
Keywords:
Dysplasia, polycystic congenital
;
Kidneys Pancreas
;
Liver
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract A renal, pancreatic and hepatic dysplasia sequene (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. [6] as “familial dysplasia of kidneys, liver and pancreas”. Since then, this combination of abnormalities has been named “polycystic dysplasia” [4] and “renal-hepatic-pancreatic dysplasia” [1], but mostly “Ivemark syndrome” [8], at the risk of being confused with asplenia-cardiac anomaly syndrome, which was reviewed by Ivemark et al. [5] and also bears Ivemark's name.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00496429
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