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Renal Tubular

Acidosis
National Kidney and Urologic Diseases Information Clearinghouse

What is renal tubular sodium bicarbonate and sodium citrate, alka­
line agents to neutralize acidic blood. The
acidosis (RTA)? good news is that medical treatment can
Renal tubular acidosis (RTA) is a disease indeed reverse the effects of RTA.
U.S. Department
that occurs when the kidneys fail to excrete
acids into the urine, which causes a person’s
of Health and
Human Services blood to remain too acidic. Without proper How is RTA diagnosed?
treatment, chronic acidity of the blood leads To diagnose RTA, doctors check the acid-base
NATIONAL to growth retardation, kidney stones, bone balance in blood and urine samples. If the
INSTITUTES
OF HEALTH disease, chronic kidney disease, and possibly blood is more acidic than it should be and the
total kidney failure. urine less acidic than it should be, RTA may
be the reason, but additional information is
The body’s cells use chemical reactions to needed to rule out other causes. If RTA is
carry out tasks such as turning food into the reason, additional information about the
energy and repairing tissue. These chemical sodium, potassium, and chloride levels in the
reactions generate acids. Some acid in urine and the potassium level in the blood will
the blood is normal, but too much acid— help identify which type of RTA a person has.
acidosis—can disturb many bodily functions. In all cases, the first goal of therapy is to neu­
Healthy kidneys help maintain acid-base tralize acid in the blood, but different treat­
balance by excreting acids into the urine and ments may be needed to address the different
returning bicarbonate—an alkaline, or base, underlying causes of acidosis.
substance—to the blood. This “reclaimed”
bicarbonate neutralizes much of the acid
that is created when food is broken down in What are the types of RTA?
the body. The movement of substances like Type 1: Classical Distal RTA
bicarbonate between the blood and structures
in the kidneys is called transport. Type 1 is also called classical distal RTA.
“Distal,” which means distant, refers to the
One researcher has theorized that Charles point in the urine-forming tube of the kidney
Dickens may have been describing a child where the defect occurs—relatively distant
with RTA in the character of Tiny Tim from from the point where fluid from the blood
A Christmas Carol. Tiny Tim’s small stature, enters the tiny tube, or tubule, that collects
malformed limbs, and periods of weakness fluid and wastes to form urine.
are all possible consequences of the chemi­
cal imbalance caused by RTA.1 In the story, This disorder may be inherited as a primary
Tiny Tim recovers when he receives medical disorder or may be one symptom of a
treatment, which would likely have included disease that affects many parts of the body.
Researchers have discovered abnormal genes
responsible for the inherited forms of the
1
Lewis D. What was wrong with Tiny Tim? disease. More often, however, classical distal
American Journal of Diseases of Children. RTA occurs as a result of systemic diseases—
1992;146(12):1403–1407.

calcium deposits to build up in the kidney and Proximal RTA also occurs in patients treated impair distal tubule function. which also attack the distal tubule. Type 2 is also called proximal RTA. The level drops or outdated tetracycline—can also cause if the kidneys excrete too much potassium into proximal RTA. In adults. paralysis. proximal RTA may urine instead of returning it to the blood sup­ complicate diseases like multiple myeloma. other tissues. primary biliary cir­ ited disorders that disrupt the body’s normal rhosis.” which means near. and children as part of a disorder called Fanconi’s potassium that normally occurs in the distal 2 Renal Tubular Acidosis . The word “proximal. renal medullary cystic disease. a hereditary form of deafness. low levels rejection of a transplanted kidney. and growth calcium leakage into urine will be corrected. Proximal RTA can also result from inher­ chronic active hepatitis. analgesic breakdown and use of nutrients. amino acids. hereditary fructose intolerance. identifying and correcting the beat. hyperthyroidism. A major consequence of classical distal RTA A few older drugs—such as acetazolamide is a low blood potassium level. Children with this growth and preventing kidney stones are the disorder would likely receive large doses of major goals of therapy. to treat acidosis and prevent then low blood-potassium. allow­ development of kidney stones and stabilizes ing bone to mature while preventing further kidney function so kidney failure does not renal disease. Correcting acidosis and low potassium This alkali therapy also helps decrease the levels restores normal growth patterns. and chronic urinary tract infections. but older children and adults rarely do because alkali therapy prevents the kidney Type 3 from excreting potassium into the urine. Infants may need potassium supple­ also be needed to help prevent bone problems. The features of Fanconi’s syn­ the autoimmune disorders Sjögren’s syndrome drome include the abnormal excretion of glu­ and lupus. classical distal RTA include sickle cell ane­ mia. as well as vitamin D deficiency and Other diseases and conditions associated with low blood-potassium. Examples nephropathy. Because potassium helps regulate nerve it may occur in people who experience chronic and muscle health and heart rate. failure. or ply. Type 3 is rarely used as a classification because it is now thought to be a combination Type 2: Proximal RTA of type 1 and type 2. caused by a generalized transport abnormality of the distal tubule. kidney stones. obstructive cystine crystals are deposited in bones and uropathy. such as sodium bicarbonate or with sodium bicarbonate or sodium citrate. The retardation in children and progressive kidney diagnosis is based on the chemical analysis of and bone disease in adults. cose. salt depletion. If acidosis is corrected an oral alkali. citrate. potassium citrate. ments. indicates that Type 4: Hyperkalemic RTA the defect is closer to the point where fluid and Type 4 is also called hyperkalemic RTA and is wastes from the blood enter the tubule. with ifosfamide. underlying causes are important steps in treat­ Untreated classical distal RTA causes growth ing the acquired forms of proximal RTA. Many of these conditions cause abnormal and Wilson disease. and bone disorders. a drug used in chemotherapy. in which ney. rejection of a transplanted kid­ include the rare disease cystinosis.diseases that affect many organ systems—like syndrome. irregular heart­ When possible. can cause extreme weakness. Restoring normal blood and urine samples. hyperparathyroidism. and phosphate into the urine. chloride. Vitamin D supplements may progress. The transport of This form of RTA occurs most frequently in electrolytes such as sodium. and even death.

disease. urine and the potassium level in the Drugs that may cause type 4 RTA include blood will help identify which type of RTA a person has. Therefore. To treat type 4 RTA successfully. • an agent called heparin that keeps blood patients may require alkaline agents to correct from clotting acidosis and medication to lower the potas­ sium in their blood. and potassium. Addi­ son’s disease. the first goal of therapy eplerenone is to neutralize acid in the blood. because potassium is important in regulating which causes a person’s blood to heart rate. and kidney tive and Kidney Diseases (NIDDK) conducts transplant rejection.tubule is impaired. additional of electrolytes such as sodium. • diuretics used to treat congestive heart failure such as spironolactone or • In all cases. amyloidosis. which is used genetic basis for their body’s resistance to the to treat pneumonia hormone. the goal is early recogni­ prevent rejection tion and adequate therapy. to address the different underlying converting enzyme (ACE) inhibitors and causes of acidosis. • a class of painkillers called nonsteroidal anti-inflammatory drugs (NSAIDs) If treated early. but different treatments may be needed • blood pressure drugs called angiotensin. and supports research into many kinds of kid­ For people who produce aldosterone but ney disease. sickle cell disease. This form is distinguished from classical distal RTA and proximal RTA because it results in high levels of potassium Points to Remember in the blood instead of low levels. Type 4 RTA occurs when blood levels of the • Without proper treatment. RTA also occurs when the tubule transport • If RTA is suspected. potassium is impaired due to an inherited dis­ potassium. removal or The National Institute of Diabetes and Diges­ destruction of both adrenal glands. chronic hormone aldosterone are low or when the kid­ acidity of the blood leads to growth neys do not respond to it. including renal tubular acidosis. remain too acidic. and chloride in the blood. kidney stones. and chloride levels in the order or the use of certain drugs. Type 4 possibly total kidney failure. Either • Renal tubular acidosis (RTA) is a low potassium—hypokalemia—or high disease that occurs when the kidneys potassium—hyperkalemia—can be a problem fail to excrete acids into the urine. HIV/AIDS. chronic kidney disease. bone the kidneys to regulate the levels of sodium. urinary tract Hope through Research obstruction. angiotensin receptor blockers (ARBs) • the antibiotic trimethoprim • the antibiotic pentamidine. most people with any type of RTA will not develop permanent kidney • some immunosuppressive drugs used to failure. Aldosterone directs retardation. chloride. cannot use it. lupus. as diabetic nephropathy. researchers have identified the NIDDK-supported researchers are exploring 3 Renal Tubular Acidosis . which will need to Type 4 RTA may also result from diseases be maintained and monitored throughout the that alter kidney structure and function such person’s lifetime. and information about the sodium.

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