Nephrology Department, Enfermedades Renales Hereditarias, Fundació Puigvert, Spain .
Nefrologia. 2011;31(1):35-43. doi: 10.3265/Nefrologia.pre2010.Nov.10727.
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disorder. Its estimated prevalence is 1 per 800 individuals. ADPKD patients constitute 8% of the population on dialysis or kidney transplantation. The disease can be diagnosed using radiological or genetic procedures. Direct genetic diagnosis of the disease can now be performed in Spain; however, it is not an easy or cheap test. This is why every case should be considered individually to determine whether genetic testing is appropriate, and to determine which genetic test is most adequate. Genetic testing in ADPKD is of special interest for living donors and neonatal and sporadic cases. Genetic testing offers the chance of performing prenatal or pre-implantation testing of embryos in families with severe cases of the disease. Also, this will enable the disease to be treated, when specific treatment becomes available, in cases that would not be candidates for treatment without genetic confirmation.
常染色体显性多囊肾病(ADPKD)是最常见的遗传性肾脏疾病。据估计,其患病率为每 800 人中有 1 人。ADPKD 患者占透析或肾移植患者的 8%。该疾病可通过放射学或遗传学程序进行诊断。目前在西班牙可以直接进行该疾病的基因诊断;然而,这不是一项简单或廉价的检测。因此,每个病例都应单独考虑,以确定是否进行基因检测,以及确定哪种基因检测最合适。ADPKD 的基因检测对活体供者以及新生儿和散发性病例具有特殊意义。基因检测为疾病严重的家庭提供了对胚胎进行产前或植入前检测的机会。此外,一旦有了特定的治疗方法,在没有基因确认就无法进行治疗的情况下,也可以对这些病例进行治疗。