Renal-hepatic-pancreatic dysplasia syndrome (Ivemark's syndrome)
- Diagn Pathol. 2007 Jul 2;2:24. doi: 10.1186/1746-1596-2-24.
- 1. Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. [email protected]
Background: Renal-Hepatic-Pancreatic dysplasia syndrome described by Ivemark in 1959 constitutes a triad pancreatic fibrosis, renal dysplasia and hepatic dysgenesis.
Case presentation: We describe two unrelated cases of Renal-Hepatic-Pancreatic dysplasia syndrome in stillborn babies. The characteristic microscopic features were present in both the cases. The second case illustrates the unique association lymphangiectasia with Renal-Hepatic-Pancreatic dysplasia syndrome. Both cases are unrelated and there is no history of any consanguineous marriage.
Conclusion: These two cases are unrelated and are rare. In the developmental research, the perinatal autopsy needs to be utilized as a major tool and an Ad hoc committee formation is required to formulate the approach towards syndromic diseases.