Dr. David Steele: Tuberous sclerosis affects the kidney largely by way of either infiltration with certain forms of tumors which have a long and difficult to pronounce name, called angiomyolipomas, and or by producing cysts within the kidney in a mechanism which is very similar to another disease, somewhat related, called polycystic kidney disease. Both of these abnormalities of the kidney generally have an onset at a fairly young age in tuberous sclerosis patients. They are slowly progressive over time. About 80 percent of patients who have tuberous sclerosis will develop angiomyolipomas during the course of their illness. And when angiomyolipomas occur, they generally occur involving both kidneys. Most tumors that are less than four centimeters in size, do not cause the patients problems. Tumors of greater than four centimeters in size are associated with an increased risk of bleeding, and certainly greater than ten centimeters in size, increased symptoms such as pain, abdominal discomfort. If the tumor in and of itself enlarges to the extent that it is painful in and of itself, we may need to intervene either surgically or angiographically. Surgical intervention may involve removal of the whole kidney, if there is significant enough damage to involve the whole kidney on that side, or removal of part of the kidney. Radiologically there are some options. These options involve what is called angiographic embolization. Basically what we are doing is cutting off the blood supply to this growing tumor, or this tumor, which is causing the patient pain. And in that way, the tumor regresses and the symptoms are relieved.
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