Determinants of renal disease variability in ADPKD.

@article{Harris2010DeterminantsOR,
  title={Determinants of renal disease variability in ADPKD.},
  author={Peter C. Harris and Sandro Rossetti},
  journal={Advances in chronic kidney disease},
  year={2010},
  volume={17 2},
  pages={131-9}
}
In common with other Mendelian diseases, the presentation and progression of autosomal dominant polycystic kidney disease (ADPKD) vary widely in the population. The typical course is of adult-onset disease with ESRD in the 6th decade. However, a small proportion has adequate renal function into the 9th decade, whereas others present with enlarged kidneys as neonates. ADPKD is genetically heterogeneous, and the disease gene is a major determinant of severity; PKD1 on average is associated with… CONTINUE READING

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