Distal renal tubular acidosis
Renal tubular acidosis - distal; Renal tubular acidosis type I; Type I RTA; RTA - distal; Classical RTA
Distal renal tubular acidosis is a disease that occurs when the kidneys do not properly remove acids from the blood into the urine. As a result, too much acid remains in the blood (called acidosis ).
Acidosis
Acidosis is a condition in which there is too much acid in the body fluids. It is the opposite of alkalosis (a condition in which there is too much ...
Causes
When the body performs its normal functions, it produces acid. If this acid is not removed or neutralized, the blood becomes too acidic. This can lead to electrolyte imbalances in the blood. It can also cause problems with normal function of some cells.
Electrolyte
Electrolytes are minerals in your blood and other body fluids that carry an electric charge. Electrolytes affect how your body functions in many ways...
The kidneys help control the body's acid level by removing acid from the blood and excreting it into the urine.
Distal renal tubular acidosis (Type I RTA) is caused by a defect in the kidney tubes that causes acid to build up in the blood.
Type I RTA is caused by a variety of conditions, including:
-
Amyloidosis
, a buildup of abnormal protein, called amyloid, in the tissues and organs
Amyloidosis
Primary amyloidosis is a disorder in which abnormal proteins build up in tissues and organs. Clumps of the abnormal proteins are called amyloid depo...
- Fabry disease, an abnormal buildup in the body of a certain type of fatty substance
-
High level of calcium in the blood
High level of calcium in the blood
Hypercalcemia means you have too much calcium in your blood.
-
Sickle cell disease
, red blood cells that are normally shaped like a disk take on a sickle or crescent shape
Sickle cell disease
Sickle cell anemia is a disease passed down through families. The red blood cells that are normally shaped like a disc take on a sickle or crescent ...
-
Sjögren syndrome
, an autoimmune disorder in which the glands that produce tears and saliva are destroyed
Sjögren syndrome
Sjögren syndrome is an autoimmune disorder in which the glands that produce tears and saliva are destroyed. This causes dry mouth and dry eyes. The...
-
Systemic lupus erythematosus
, an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue
Systemic lupus erythematosus
Systemic lupus erythematosus (SLE) is an autoimmune disease. In this disease, the body's immune system mistakenly attacks healthy tissue. It can af...
-
Wilson disease
, an inherited disorder in which there is too much copper in the body's tissues
Wilson disease
Wilson disease is an inherited disorder in which there is too much copper in the body's tissues. The excess copper damages the liver and nervous sys...
- Use of certain medicines, such as amphotericin B, lithium, and analgesics
Symptoms
Symptoms of distal renal tubular acidosis include any of the following:
-
Confusion
or
decreased alertness
Confusion
Confusion is the inability to think as clearly or quickly as you normally do. You may feel disoriented and have difficulty paying attention, remembe...
Decreased alertness
Decreased alertness is a state of reduced awareness. A coma is a state of decreased alertness from which a person cannot be awakened. A long-term co...
-
Fatigue
Fatigue
Fatigue is a feeling of weariness, tiredness, or lack of energy.
- Impaired growth in children
-
Increased breathing rate
Increased breathing rate
Hyperventilation is rapid and deep breathing. It is also called overbreathing, and it may leave you feeling breathless.
-
Kidney stones
Kidney stones
A kidney stone is a solid mass made up of tiny crystals. One or more stones can be in the kidney or ureter at the same time.
-
Nephrocalcinosis
(too much calcium deposited in the kidneys)
Nephrocalcinosis
Nephrocalcinosis is a disorder in which there is too much calcium deposited in the kidneys. It is common in premature babies.
-
Osteomalacia
(softening of the bones)
Osteomalacia
Osteomalacia is softening of the bones. It most often occurs because of a problem with vitamin D, which helps your body absorb calcium. Your body n...
- Muscle weakness
Other symptoms may include:
-
Bone pain
Bone pain
Bone pain or tenderness is aching or other discomfort in one or more bones.
-
Decreased urine output
Decreased urine output
Decreased urine output means that you produce less urine than normal. Most adults make at least 500 ml of urine in 24 hours (a little over 2 cups)....
-
Increased
heart rate
or
irregular heartbeat
Irregular heartbeat
Palpitations are feelings or sensations that your heart is pounding or racing. They can be felt in your chest, throat, or neck. You may:Have an unpl...
-
Muscle cramps
Muscle cramps
Muscle cramps are when a muscle gets tight (contracts) without you trying to tighten it, and it does not relax. Cramps may involve all or part of on...
- Pain in the back, flank, or abdomen
- Skeletal abnormalities
Exams and Tests
The doctor will perform a physical exam and ask about your symptoms.
Tests that may be ordered include:
-
Arterial blood gas
Arterial blood gas
Blood gases are a measurement of how much oxygen and carbon dioxide are in your blood. They also determine the acidity (pH) of your blood.
-
Blood chemistry
Blood chemistry
A comprehensive metabolic panel is a group of blood tests. They provide an overall picture of your body's chemical balance and metabolism. Metaboli...
-
Urine pH
and
acid-loading test
Acid-loading test
The acid loading test (pH) measures the ability of the kidneys to send acid to the urine when there is too much acid in the blood. This test involve...
-
Urinalysis
Urinalysis
Urinalysis is the physical, chemical, and microscopic examination of urine. It involves a number of tests to detect and measure various compounds th...
Treatment
The goal is to restore normal acid level and electrolyte balance in the body. This will help correct bone disorders and reduce calcium buildup in the kidneys (nephrocalcinosis) and kidney stones.
The underlying cause of distal renal tubular acidosis should be corrected if it can be identified.
Medicines that may be prescribed include potassium citrate and sodium bicarbonate. These are alkaline medicines that help correct the acidic condition of the body. Sodium bicarbonate may correct the loss of potassium and calcium.
Outlook (Prognosis)
The disorder must be treated to reduce its effects and complications, which can be permanent or life threatening. Most cases get better with treatment.
When to Contact a Medical Professional
Call your health care provider if you have symptoms of distal renal tubular acidosis.
Get medical help right away if you develop emergency symptoms such as:
-
Decreased consciousness
Decreased consciousness
Decreased alertness is a state of reduced awareness. A coma is a state of decreased alertness from which a person cannot be awakened. A long-term co...
-
Seizures
Seizures
A seizure is the physical findings or changes in behavior that occur after an episode of abnormal electrical activity in the brain. The term "seizure...
-
Severe
decrease in alertness
or
orientation
Decrease in alertness
Decreased alertness is a state of reduced awareness. A coma is a state of decreased alertness from which a person cannot be awakened. A long-term co...
Orientation
Mental status testing is done to check a person's thinking ability, and to determine if any problems are getting better or worse. It is also called ...
Prevention
There is no prevention for this disorder.
References
Krapf R, Seldin DW, Alpern RJ. Clinical syndromes of metabolic acidosis. In: Alpern RJ, Caplan M, Moe OW, eds. Seldin and Giebisch's The Kidney . 5th ed. Philadelphia, PA: Elsevier; 2013:chap 59.
Sreedharan R, Avner ED. Renal tubular acidosis. In: Kliegman RM, Stanton BF, St Geme, JW III, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, PA: Elsevier; 2015:chap 529.
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Kidney anatomy - illustration
The kidneys are responsible for removing wastes from the body, regulating electrolyte balance and blood pressure, and stimulating red blood cell production.
Kidney anatomy
illustration
-
Kidney - blood and urine flow - illustration
This is the typical appearance of the blood vessels (vasculature) and urine flow pattern in the kidney. The blood vessels are shown in red and the urine flow pattern in yellow.
Kidney - blood and urine flow
illustration
-
Kidney anatomy - illustration
The kidneys are responsible for removing wastes from the body, regulating electrolyte balance and blood pressure, and stimulating red blood cell production.
Kidney anatomy
illustration
-
Kidney - blood and urine flow - illustration
This is the typical appearance of the blood vessels (vasculature) and urine flow pattern in the kidney. The blood vessels are shown in red and the urine flow pattern in yellow.
Kidney - blood and urine flow
illustration
-
Kidney stones
(In-Depth)
Review Date: 10/13/2015
Reviewed By: Walead Latif DO, nephrologist, Medical Director of Fresenius Vascular Care, and Clinical Assistant Professor of Rutgers Medical School, Newark, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.