HNF1B nephropathy has a slow-progressive phenotype in childhood —with the exception of very early onset cases: results of the German Multicenter HNF1B Childhood Registry

ConclusionsIn most children,HNF1B nephropathy has a non-progressive course of cyst development and a slow-progressive course of kidney function. A subgroup of patients developed ESRD at very young age<  2 years requiring special medical attention. The parent-of-origin effect suggests an influence of epigenetic modifiers inHNF1B disease.
Source: Pediatric Nephrology - Category: Urology & Nephrology Source Type: research