Polycystic Kidney Disease(PKD)

I was interested in the report on Hemochromatosis, well I am not afficlicted with that but in my branch of the Tully family we are susceptible to Polycystic Kidney Disease (PKD). My father died of this, as apparently did his father. Currently 3 of my 4 brothers have this disease (under control thank God). Some info on the disease follows.

PKD is an inherited disorder, the dominant form of the disease (ADPKD) is passed from one generation to the next by an affected parent. Each child of an ADPKD parent has a 50 percent chance of inheriting the disease. Scientists have also discovered that approximately 10% of the PKD patient community became infected through spontaneous mutation, and not through inheritance. ADPKD equally affects men and women, regardless of age, race, or ethnic origin.

This disease comes in two hereditary forms:

1. autosomal dominant (ADPKD), the most common of all life-threatening genetic diseases

2. autosomal recessive (ARPKD), a relatively rare disease that often
causes significant mortality in the first month of life.

A normal kidney is the size of a human fist. However, with the presence of PKD, cysts develop in both kidneys. There may be just a few cysts or many, and the cysts may range in size from a pinhead to the size of a grapefruit. When many cysts develop, the kidneys can grow to be the size of a football or larger and weigh as much as 38 pounds each.


In the 1700s and 1800s, PKD was often given the label of Bright's disease. This term encompassed any of several kidney diseases marked by high concentrations of protein in the urine. Today, we know that any of the cases of Bright's disease were actually cases of PKD. The first documented case of PKD dates back to mid 1500's.

While not an "IRISH" disease it is inherited, which in my family travels the Tully line.

Regards
Philip J. Tully
tullyph@bellatlantic.net

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