Related Experiment Video
Updated: Jul 14, 2026

Human Liver Microphysiological System for Assessing Drug-Induced Liver Toxicity In Vitro
Published on: January 31, 2022
Renal-hepatic-pancreatic dysplasia syndrome (Ivemark's syndrome)
Mahesha Vankalakunti1, Kirti Gupta, Nandita Kakkar
1Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. vkmahesh123@yahoo.co.in
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.
Related Concept Videos
Hepatic Encephalopathy
Chronic Pancreatitis II: Pathophysiology
Acute Pancreatitis II: Pathophysiology
Chronic Pancreatitis I: Introduction
Acute Pancreatitis II: Clinical Manifestations and Management
Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test
