Group Description
Renal Tubular Acidosis is a scary diagnosis for any parent who hears it, but once understood, with support, ideas and help from other parents, it can make the road less worrisome. That is what this group is designed to do.
This is a support eGroup open to patients of RTA, parents of kids with RTA or anyone interested in making friends, sharing information and providing support for kids affected by "Renal Tubular Acidosis."
Affected individuals, family members, friends, medical professionals and rare disease researchers are all encouraged to join this open forum.
At one time, doctors divided RTA into four types.
This disorder may be inherited as a primary disorder or may be one symptom of a disease that affects many parts of the body. Researchers have now discovered the abnormal gene responsible for the inherited form. More often, however, classic distal RTA is a complication of diseases that affect many organ systems (systemic diseases), like the autoimmune disorders Sjögren's syndrome and lupus.
Other diseases and conditions associated with distal RTA include hyperparathyroidism, a hereditary form of deafness, analgesic nephropathy, rejection of a transplanted kidney, renal medullary cystic disease, obstructive uropathy, and chronic urinary tract infections.
A major consequence of classic distal RTA is low blood-potassium. The level drops if the kidneys excrete potassium into urine instead of returning it to the blood supply. Since potassium helps regulate nerve and muscle health and heart rate, low levels can cause extreme weakness, cardiac arrhythmias, paralysis, and even death.
Untreated distal RTA causes growth retardation in children and progressive renal and bone disease in adults. Restoring normal growth and preventing kidney stones, another common problem in this disorder, are the major goals of therapy. If acidosis is corrected with sodium bicarbonate or sodium citrate, then low blood-potassium, salt depletion, and calcium leakage into urine will be corrected. Alkali therapy also helps decrease the development of kidney stones. Potassium supplements are rarely needed except in infants, since alkali therapy prevents the kidney from excreting potassium into the urine.
source: http://kidney.niddk.nih.gov/kudiseases/pubs/tubularacidosis/index.htm
This form of RTA occurs most frequently in children as part of a disorder called Fanconi's syndrome. The symptoms of Fanconi's syndrome include high levels of glucose, amino acids, citrate, and phosphate in the urine, as well as vitamin D deficiency and low blood-potassium.
Proximal RTA can also result from inherited disorders that disrupt the body's normal breakdown and use of nutrients. Examples include the rare disease cystinosis (in which cystine crystals are deposited in bones and other tissues), hereditary fructose intolerance, and Wilson's disease.
Proximal RTA also occurs in patients treated with ifosfamide, a drug used in chemotherapy. A few older drugs--such as acetazolamide or outdated tetracycline--can also cause proximal RTA. In adults, proximal RTA may complicate diseases like multiple myeloma, or it may occur in people who experience chronic rejection of a transplanted kidney.
When possible, identifying and correcting the underlying causes are important steps in treating the acquired forms of proximal RTA. The diagnosis is based on the chemical analysis of blood and urine samples. Children with this disorder would likely receive large doses of oral alkali, such as sodium bicarbonate or potassium citrate, to treat acidosis and prevent bone disorders, kidney stones, and growth failure. Correcting acidosis and low potassium levels restores normal growth patterns, allowing bone to mature while preventing further renal disease. Vitamin D supplements may also be needed to help prevent bone problems.
source: http://kidney.niddk.nih.gov/kudiseases/pubs/tubularacidosis/index.htm
This form of RTA is most often referred to as type 4. It occurs when blood levels of the hormone aldosterone are low or when the kidneys do not respond to it. Aldosterone directs the kidneys to regulate the levels of sodium, potassium, and chloride in the blood. Type 4 RTA is distinguished by a high blood-potassium level.
Hyperkalemic distal RTA may result from sickle cell disease, urinary tract obstruction, lupus, amyloidosis, or transplantation.
Aldosterone's action may be impeded by drugs, including
For people who produce aldosterone but cannot use it, researchers have now identified the genetic basis for their body's resistance to the hormone. To treat type 4 RTA successfully, patients may require alkaline agents to correct acidosis as well as medication to lower the potassium in their blood.
If treated early, most people with RTA will not develop permanent kidney failure. Therefore, the goal is early recognition and adequate therapy, which will need to be maintained and monitored throughout the patient's lifetime.
source: http://kidney.niddk.nih.gov/kudiseases/pubs/tubularacidosis/index.htm
American Association of Kidney Patients
3505 East Frontage Road
Suite 315
Tampa, FL 33607
Phone: 1-800-749-2257 or (813) 636-8100
Email: info@aakp.org
Internet: www.aakp.org
American Kidney Fund
6110 Executive Boulevard
Suite 1010
Rockville, MD 20852
Phone: 1-800-638-8299 or (301) 881-3052
Email: helpline@akfinc.org
Internet: www.kidneyfund.org
National Kidney Foundation
30 East 33rd Street
New York, NY 10016
Phone: 1-800-622-9010 or (212) 889-2210
Email: info@kidney.org
Internet: www.kidney.org
source: http://kidney.niddk.nih.gov/kudiseases/pubs/tubularacidosis/index.htm