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Basic Informations About ARPKD

2013-04-03 16:40

ARPKD stands for autosomal recessive polycystic kidney disease and it is one of the two main types of PKD, the other being ADPKD. Since ARPKD is much more rare in clinic and patients often die within days or even hours after birth, there are relatively few information on ARPKD. This article will cover some basic informations about this rare but serious kidney problem.

Before ARPKD is often called infantile polycystic kidney disease since patients will have obvious clinical symptoms in infantile period and a minority can occur to children and adolescents. The incidence of ARPKD is 1/10000-1/40000. Only f both parents carry the abnormal genes can the children inherit the disease and the genetic rate is 25%. Often the liver will be involved and the manifestation is cysts in the liver.

The incidence of ARPKD is related to the gene of PKHD1 and the prognosis is not good. About 50% children will die of respiratory failure or heart failure after birth within several hours or days. Even if the diseased child can survive to adulthood, he will progress into renal failure. Besides, there will many other healthy problems such as renal collecting duct fusiform expansion, cholangiectasis, congenital liver fibrosis, high blood pressure, etc.

Since it is due to gene mutation, there is at present no treatment for ARPKD. Blood pressure control, dialysis and kidney transplant can help prolong the patient’s life span to adulthood. Ideal blood pressure for ARPKD is 130/85 or even lower. Controlling high blood pressure can significantly slow down the decline rate of kidney functions.

Even though the life expectancy can be improved, the patients will suffer a lot even with proper treatments. Therefore a prenatal gene diagnosis is recommended to see if the children will have the disease in advance. If both parents are the carrier of the virulence gene but none of them develop the disease, they still have 75% chance to give birth to a healthy baby(25% healthy baby with no abnormal gene and 50% healthy baby but the baby carries one abnormal gene and will pass it to the following generations).

Tag: PKD Overview

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