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Renal, pancreatic and hepatic dysplasia sequence
D Carles1, F Serville, J P Dubecq
1Service d'Anatomie Pathologique, UERSM III, Université de Bordeaux II, France.
European Journal of Pediatrics
|May 1, 1988
Summary
A rare renal, pancreatic, and hepatic dysplasia sequence was identified in a premature infant. This case highlights a severe congenital condition affecting multiple organs, emphasizing the importance of recognizing Ivemark syndrome.
Area of Science:
- Pediatric Pathology
- Medical Genetics
- Developmental Biology
Background:
- The renal, pancreatic, and hepatic dysplasia sequence (RPHD sequence) is a rare congenital disorder.
- Historically documented as Ivemark syndrome, it presents a distinct triad of organ abnormalities.
- Potential confusion exists with Ivemark's asplenia-cardiac anomaly syndrome.