Autosomal dominant polycystic kidney disease: Light at the end of the tunnel?

作者：Melander Catherine; Joly Dominique; Knebelmann Bertrand^*

来源：Nephrologie & Therapeutique, 2010, 6(4): 226-231.

DOI：10.1016/j.nephro.2010.02.004

• Autosomal dominant polycystic kidney disease
• Vasopressin receptor antagonists
• MTOR inhibitors
• Somatostatin

Autosomal dominant polycystic kidney disease, characterized by numerous cysts in both kidneys, is the most frequent, potentially lethal monogenic disorder. Its prevalence is evaluated between 1/400 and 1/1000 live births and it accounts for 7 to 8% of end-stage renal disease in developped countries. As yet, the pathogenesis of this disease is not fully understood and there is no specific treatment available. Nevertheless, in the last few years, fundamental and clinical research has been highly efficient in these fields. The purpose of this review is to update the practical implications of this research in terms of clinical manifestations, diagnosis and treatment.

• 出版日期2010-7