In renal tubular acidosis, the kidney tubules cannot adequately remove acids from the blood to excrete them in the urine.

• The tubules of the kidneys that remove acid from the blood are damaged when a person takes certain drugs or has another disorder that affects the kidneys.
• Typically muscle weakness and diminished reflexes occur when the disorder has been present for a long time.
• Blood tests are done to detect high acid levels.
• Some people drink a solution of baking soda every day to neutralize the acid.

Normally, the breakdown of food produces acids that circulate in the blood. The kidneys remove acids from the blood and excrete them in the urine. This function is predominantly performed by the kidney tubules. In renal tubular acidosis, the ability of the kidneys to excrete acids is partially impaired, and acid levels build up in the blood (metabolic acidosis). The balance of electrolytes is also affected. Renal tubular acidosis may lead to the following problems:

• Low or high potassium levels in the blood
• Calcium deposits in the kidneys, which may lead to kidney stones
• Dehydration
• Painful softening and bending of the bones (osteomalacia or rickets)

Renal tubular acidosis may be a permanent, inherited disorder. However, it may be an intermittent problem in people who have other disorders, such as diabetes mellitus, sickle cell disease, or an autoimmune disorder (such as systemic lupus erythematosus). Renal tubular acidosis may also be a temporary condition brought on by an obstruction of the urinary tract or by drugs, such as acetazolamideSome Trade Names
DIAMOX
, amphotericin BSome Trade Names
FUNGIZONE
, angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers (ARBs), and diuretics that conserve the body's potassium (so-called potassium-sparing diuretics).

There are four types of renal tubular acidosis, types 1 through 4. The types are distinguished by the particular abnormality in kidney function that causes acidosis. All four types are uncommon, but type 3 is extremely rare.

Symptoms and Diagnosis

Many people have no symptoms. Most others develop symptoms only after the disorder has been present for a long time. Which symptoms eventually develop depend on the type of renal tubular acidosis. When potassium levels in the blood are low, as occurs in types 1 and 2, neurologic problems may develop, including muscle weakness, diminished reflexes, and even paralysis. In type 4, potassium levels typically increase, although it is uncommon for the level to rise high enough to cause symptoms. If the level becomes too high, irregular heartbeats and muscle paralysis may develop. In type 1, kidney stones may develop, causing damage to kidney cells and, in some cases, chronic kidney failure.

A doctor considers the diagnosis of type 1 or type 2 renal tubular acidosis when a person has certain characteristic symptoms (such as muscle weakness and diminished reflexes) and when tests reveal high levels of acid and low levels of bicarbonate and potassium in the blood. Type 4 renal tubular acidosis is usually suspected when high potassium levels accompany high acid levels and low bicarbonate levels in the blood. Special tests help to determine the type of renal tubular acidosis.

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Some Types of Renal Tubular Acidosis Type Cause Underlying Abnormality Resulting Symptoms and Metabolic Abnormalities 1 May be hereditary or may be triggered by an autoimmune disease or certain drugs. Cause usually not known, especially in women Inability to excrete acid into the urine High blood acidity Mild dehydration Low potassium levels in the blood, leading to muscle weakness and paralysis Fragile bones Bone pain Calcium deposits, leading to kidney stones Kidney failure 2 Usually caused by a hereditary disease such as Fanconi syndrome, hereditary fructose intolerance, Wilson's disease, or oculocerebrorenal syndrome (Lowe syndrome. May also be caused by heavy metal poisoning or certain drugs Inability to reabsorb bicarbonate from the urine, so too much bicarbonate is excreted High blood acidity Mild dehydration Low potassium levels in the blood 4 Not hereditary. Caused by diabetes, an autoimmune disease, sickle cell disease, or an obstruction in the urinary tract. Worsened by certain drugs, including potassium-sparing diuretics, angiotensin-converting enzyme inhibitors, and angiotensin II receptor blockers Deficiency of or inability to respond to aldosterone, a hormone that helps regulate potassium and sodium excretion by the kidneys High blood acidity and high potassium levels in the blood that rarely cause symptoms, unless the potassium level is unusually high (in that case, irregular heartbeats and muscle paralysis develop) Note: Type 3 is a mixture of Types 1 and 2 and is extremely rare.

Treatment

Treatment depends on the type. Types 1 and 2 are treated by drinking a solution of sodium bicarbonate (baking soda) every day to neutralize the acid that is produced from food. This treatment relieves the symptoms and prevents kidney failure and bone disease or keeps these problems from becoming worse. Other specially prepared solutions are available, and potassium supplements may also be required. In type 4, the acidosis is so mild that bicarbonate may not be needed. High potassium levels in the blood can usually be kept in check by restricting potassium intake, avoiding dehydration, and substituting different drugs or adjusting drug dosages.

Last full review/revision December 2006 by Peter C. Brazy, MD