Polycystic Kidney Disease is a genetic disease, and develops progressively,
some patients may concurrently have polycystic liver and renal calculus.
Infection is an important factor that may suddenly aggravate the disease and
make the renal function decline rapidly.
Because of gene inherited from the parents, the epithelial cells proliferate
abnormally, yet apoptosis of this kind of cells is inhibited. So, when
proliferating to certain extent (saying the diameter exceeds some milimeters),
this proliferated part will be separated from the renal tubule or collecting
tubule, so an isolated cyst filled with liquid will form.
At this time, the phenotype of renal tubular epithelial cells changes, and
renal tubular epithelial cells turns into cyst-lining epithelial cells, which
can secrete more and more cystic fluid;
however, the cystic fluid become more and more and can not be discharged
normally. With large amount of cystic fluid accumulated, the cyst become larger
and larger With growth of cysts, normal renal structure will be changed by these
cysts.
Normal kidney size is your kidneys are about 10-12cm and 30cm kidney are
enlarged greatly. And PKD leads to insufficient blood supply in kidneys, which
activates the Renin-angiotensin-aldosteron System, which cuases your high blood
pressure.
Indeed, many PKD patients were told that there was not a treatment for PKD
and let them wait until they could do dialysis. However, with 20-30% renal
function left, if you take effective treatment now to shrink your cysts and
control your blood pressure, you are probably to delay dialysis, even avoid
dialysis for your whole life.
As you can see from the above explanation, it is the cyst-lining epithelial
cells’ abnormal proliferation and apoptosis, as well as its secreation of cystic
liquid that make the cysts grow. So, we can do now is to change or inhibit the
cyst-lining epithelial cells’ function so as to prevent the cysts from growing
and to shrink the cysts at the same time. In that case, dialysis and kidney
transplant will be avoided.
