Renal-hepatic-pancreatic dysplasia

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Synonyms: Ivemark II syndrome | Renohepaticopancreatic dysplasia

Renal-hepatic-pancreatic dysplasia is 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 tendancy 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-dependent diabetes.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version June 2024

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Help Hope Live supports community-based fundraising for people with unmet medical expenses and related costs due to cell and organ transplants or catastrophic injuries and illnesses. These efforts play a critical role in helping clients access the care and equipment they need to heal, live, and thrive. Unlock a powerful and safe way to raise funds for medical needs with Help Hope Live. They provide one-on-one fundraising guidance, a customizable Campaign Page, bill pay support, and additional benefits based on their 501(c)(3) nonprofit status.

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Clinical Trials

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