title: [ADPKD: predictors of Renal Disease progression].
abstract: Factors predicting rapid progression of kidney disease in ADPKD can be divided into genetic (non-modifiable) and clinical (modifiable) risk factors. Patients harbouring PKD1 mutations, in particular if truncating, have a more severe form of ADPKD. Clinical risk factors include decrease in glomerular filtration rate and renal blood flow at a young age; high total kidney volume; hypertension and urological complications <35 years; albuminuria/proteinuria. The renal disease is also more severe in males and in subjects with family history of ESRD <55 years. In recent years, two models for predicting progression in ADPKD have been published: the Mayo model, based on height-adjusted TKV, age and eGFR, and the Brest model, based on PKD gene mutation type, gender, and early onset of hypertension and urological complications. With the emergence of new disease-modifying therapies, prediction tools are essential. However, the high variability in ADPKD makes the predicting models difficult to apply on an individual patient basis. Thus, the above-mentioned predicting models should be viewed as complimentary to clinical evaluation and follow-up. In the future, an individual risk score linking genetic, imaging and clinical data might prove the most accurate way of predicting long-term outcome.
authors: Francesco
authors: F
authors: Scolari
authors: Nadia
authors: N
authors: Dallera
authors: Arianna
authors: A
authors: Saletti
authors: Vincenzo
authors: V
authors: Terlizzi
authors: Claudia
authors: C
authors: Izzi
mesh_terms: D018450:Disease Progression; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology*; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications*; D011379:Prognosis; D012307:Risk Factors