Hepatocyte Nuclear Factor 1β-Associated Kidney Disease: More than Renal Cysts and Diabetes.

Abstract excerpt

Hepatocyte nuclear factor 1β (HNF1β)-associated disease is a recently recognized clinical entity with a variable multisystem phenotype. Early reports described an association between HNF1B mutations and maturity-onset diabetes of the young. These patients often presented with renal cysts and renal function decline that preceded the diabetes, hence it was initially referred to as renal cysts and diabetes syndrome....

Topics

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• Diabetic Nephropathies
• Hepatocyte Nuclear Factor 1-beta
• Kidney Diseases, Cystic
• Mutation
• Phenotype