Many people with TSC live healthy lives with few complications from the disorder's renal manifestations. However, because of the risks these abnormalities pose, and because kidney lesions can progress rapidly, it is important for people with TSC to undergo regular renal examinations. Aggressive treatments can be very effective in combating the debilitating effects of TSC, but only if problems are identified early in their development.
Specialists recommend that people with TSC undergo renal examinations every two to three years prior to puberty and every year after that. Because large lesions (greater than 4 cm in diameter) tend to grow faster than small lesions and pose a higher risk of hemorrhage, a person with such a lesion may require more frequent examinations.
Physicians and radiologists typically use ultrasound imaging equipment to examine the kidneys of people with TSC. They may also use computed tomography (CT) scans or magnetic resonance imaging (MRI) in cases where higher resolution images are required to determine, for example, whether a lesion is benign or malignant.
For most people with TSC, regular kidney examinations are all that is required to ensure renal health. However, in cases where TSC-related abnormalities affect kidney function more significantly, other steps may be required.
Doctors commonly prescribe antihypertensive medications to help control blood pressure. Controlling blood pressure is particularly important in patients with impaired kidney function. Low-protein diets may play a role as well, minimizing the amount of work an already taxed renal system has to perform.
Doctors may also attempt to slow or stop the growth of progressive kidney lesions, while preserving existing kidney function. One way to do this is through a process called selective embolization. This involves cutting off the flow of blood that feeds a growing tumor, causing the tumor to regress. In cases where embolization is impossible or ineffective, it is sometimes necessary to remove part or all of a kidney to stop the progression of renal tumors.
Though it is uncommon, some people with TSC who develop progressive kidney failure may require regular dialysis treatments to make up for their loss of kidney function. This process cleans toxins from the blood that the damaged or missing kidneys would normally remove. For people with such severe kidney dysfunction, transplantation offers the only alternative to dialysis. Physicians have found kidney transplantation to be a useful option in a select group of patients.
It is important to remember:
- Many kidney manifestations of TSC have no adverse effects on kidney function, but some do, and their effects can be life threatening.
- Angiomyolipomas (AMLs) are benign tumors of the kidney.
- Renal cell carcinomas are malignant tumors of the kidney that may be more prevalent in people with TSC than in people without TSC.
- Renal cysts are typically small and few in number.
- Large, numerous renal cysts suggest a more serious condition: polycystic kidney disease.
- TSC specialists recommend kidney screenings every two to three years before puberty and annually after puberty.
- Make sure your physician is familiar with TSC-related kidney abnormalities.
- Make sure your physician has access to radiologists experienced in distinguishing between benign and malignant renal lesions.
A nephrologist is a physician who has been educated and trained in kidney diseases, kidney transplantation, and dialysis therapy. Nephrologists are trained to recognize various renal lesions, including AMLs, renal cysts, and renal cell carcinomas. Children with TSC may see a pediatric nephrologist.
A radiologist is a person who creates and interprets images, including x-rays, ultrasounds, MRIs, and CT scans. A radiologist is trained to distinguish between AMLs and renal cysts as well as between benign and malignant tumors.
A urologist is a physician who has specialized knowledge and skill regarding problems of the male and female urinary tract and the male reproductive organs.
You can find a list of on-staff physicians on the Herscot Center for Children and Adults with TSC Web site.
American Kidney Fund
The American Kidney Fund provides education about kidney disease and financial assistance to kidney patients in need.
National Kidney Foundation
The National Kidney Foundation provides information about kidney disease and kidney transplantation. (There may also be a regional office in your state.)