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renal-hepatic-pancreatic dysplasia - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

renal-hepatic-pancreatic dysplasia

MONDO:0017417

Also known as:Ivemark II syndromeIvemark's syndromeRenohepaticopancreatic dysplasia

Overview

A rare, genetic, developmental defect during embryogenesis syndrome characterized by the triad of pancreatic fibrosis (and cysts, with a reduction of parenchymal tissue), renal dysplasia (with peripheral cortical cysts, primitive collecting ducts, glomerular cysts and metaplastic cartilage) and hepatic dysgenesis (enlarged portal areas containing numerous elongated binary profiles with a tendency to perilobular fibrosis). Situs abnormalities, skeletal anomalies and anencephaly have also been associated. Patients that survive the neonatal period present renal insufficiency, chronic jaundice and insulin-dependant diabetes.

ResearchLimited published research (3 articles)

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for renal-hepatic-pancreatic dysplasia.

3 publications have been identified in PubMed for renal-hepatic-pancreatic dysplasia. Research spans Review / Meta-Analysis (33%), Case Report / Case Series (33%), and Basic Science / Preclinical (33%).

Recent Publications

Teng CT (2025). [PMID: 41149691](https://pubmed.ncbi.nlm.nih.gov/41149691/). *Pediatric reports*. [Case Report / Case Series]
Roig J (2025). [PMID: 40189576](https://pubmed.ncbi.nlm.nih.gov/40189576/). *Cell communication and signaling : CCS*. [Review / Meta-Analysis]
Thuy PX (2024). [PMID: 39408701](https://pubmed.ncbi.nlm.nih.gov/39408701/). *International journal of molecular sciences*. [Basic Science / Preclinical]

Data assembled from 3 of 12 sources · Last updated Sep 20, 2026, 1:42 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:294415

GARD

Genetic and Rare Diseases Info Center

GARD:17356

Questions for Your Care Team

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renal-hepatic-pancreatic dysplasia

Overview

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Subtypes

Related Diseases

Questions for Your Care Team

Questions for Your Care Team