Polycystic Kidney Disease (PKD) is the most common genetic disorder which
threatening people’s lives, and the morbidity is 4/10 around the world. It is
characterized by fluid-filled cysts filled in the nephron of both kidneys,
unfortunately, many patients lead to Kidney Failure in the end. Polycystic
Kidney Disease is the fourth most common cause of Kidney Failure.
Cysts usually occur as an out pouching (like a blister), Approximately 70%
cysts detach from the nephron when they are small and proceed to enlarge as they
fill with either clear fluid or fluid that may contain blood cells. In patients
with PKD, hundreds to thousands of cysts can develop that range from the size of
a pinhead to as large as a grapefruit (10-20 cm.) in diameter. Each kidney may
expand to as large as a football and may weigh up to 38 pounds. The continual
enlargement of cysts crowds out healthy kidney tissue. The kidneys may quadruple
in size before kidney function is affected at which point renal function rapidly
declines.
There are at least three factors related to cyst formation in the kidney, but
the exact cause which gives rise to the process of cyst formation is not clear.
These three factors include:
· Increased production of cells lining the cyst wall. These cells continue to
reproduce all their lives and enable the cyst to enlarge.
· Increased production of fluid by the cells forming the cyst.
· Abnormal basement membrane structure and function. The basement membrane is
a thin layer of tissue upon which the cysts rest. In PKD the makeup of the
basement membrane is abnormal, the tissue is also thicker than usual, and
attachment to the cyst wall is increased.
Hypertension (high blood pressure) is closely associated with PKD due to the
increased burden on the blood vessels from inadequately filtered blood. It is
often one of the earliest symptoms of PKD and more patients at the age of 20 or
30 present these symptoms.
Polycystic kidney disease (PKD) affects approximately 600,000-700,000 people
in the United States. Approximately 12.5 million people around the world are
suffering from PKD. It is also thought that account for 7-10% people who are on
dialysis and require mechanical filtration of blood.
Additional important facts about PKD include:· Approximately 5,000 to 6,000
new cases of PKD are diagnosed each year in the U.S.
40% of the cases are diagnosed in the U.S. every year, more patients at the
age of 45, even younger
5-10% people with PKD don’t have the family history of PKD
Approximately 50% patients are diagnosed with Autosomal Dominant Polycystic
Kidney Disease (ADPKD) which is easy to develop into end-stage renal disease at
the age of 60.
Polycystic Kidney Disease is considered to be the fourth leading cause of
Kidney Failure.
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