Prognosis and Prevention of Polycystic Kidney Disease

Polycystic Kidney Disease (PKD) is a hereditary renal disorder and it can be divided into two categories---autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) according to different hereditary modes.

ARPKD, also known as infantile PKD, is rare in clinic and both the parents have no illness history. Most diseased babies will die shorty after birth and only a few light cases can survive until childhood or even adulthood.

ADPKD, also known as adult PKD is much more common. It has the feature of familial aggregation and male and female have the same possibility of inheriting the disease and there will be patients in continuous generations. The prognosis has been greatly improved with more and more understanding of it.

The prognosis of PKD has great individual difference and the duration of the disease is closely related to complications. The prognosis is poor if the patients have many complications and the illness gradually progresses into renal failure. In some cases, the prognosis is good because the progress is very slow and patients have no complications though their renal function has been impaired. Therefore the handling of complications is the determining factor of prognosis. And the younger the patients have clinical manifestations, the worse the prognosis is. Generally speaking, the survival time is 10 years after the symptoms occur. The main death causes of PKD are kidney failure, heart failure, intracranial hemorrhage, lung infection, etc.

Because of its heredity and it will accompany the patients for lifelong time, it is very important to know the effective measures for preventing the birth of diseased babies. From the perspective of eugenics and based on the the hereditary rule of ADPKD, the following can be done.

1. Premarital B ultrasound (this is especially important if parents have PKD). Try to avoid the marriage if both sides have the disease to lower the incidence of next generation.

2. Cystic gene detection. At the 10th week of pregnancy, the cystic gene detection of the amniotic fluid or hair cells should be done. Since the baby has 50% chance inheriting the disease if one parent has it, the molecular genetic inspection can help parents give birth to a healthy baby and the gene will not affect the following generations.