Autosomal dominant polycystic kidney disease: the changing face of clinical management

Lancet. 2015 May 16;385(9981):1993-2002. doi: 10.1016/S0140-6736(15)60907-2.

Abstract

Autosomal dominant polycystic kidney disease is the most common inherited kidney disease and accounts for 7-10% of all patients on renal replacement therapy worldwide. Although first reported 500 years ago, this disorder is still regarded as untreatable and its pathogenesis is poorly understood despite much study. During the past 40 years, however, remarkable advances have transformed our understanding of how the disease develops and have led to rapid changes in diagnosis, prognosis, and treatment, especially during the past decade. This Review will summarise the key findings, highlight recent developments, and look ahead to the changes in clinical practice that will likely arise from the adoption of a new management framework for this major kidney disease.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Cyclic AMP / metabolism
  • Female
  • Forecasting
  • Genetic Testing
  • Humans
  • Male
  • Middle Aged
  • Mutation / genetics
  • Polycystic Kidney, Autosomal Dominant / diagnosis
  • Polycystic Kidney, Autosomal Dominant / etiology
  • Polycystic Kidney, Autosomal Dominant / therapy*
  • Prognosis
  • Protein-Serine-Threonine Kinases / genetics
  • Pyruvate Dehydrogenase (Acetyl-Transferring) Kinase
  • Signal Transduction / physiology
  • TOR Serine-Threonine Kinases / metabolism
  • TRPP Cation Channels / genetics
  • Young Adult

Substances

  • Pyruvate Dehydrogenase (Acetyl-Transferring) Kinase
  • TRPP Cation Channels
  • polycystic kidney disease 1 protein
  • Cyclic AMP
  • MTOR protein, human
  • TOR Serine-Threonine Kinases
  • Protein-Serine-Threonine Kinases