In argininevasopressin resistance (formerly known as nephrogenic diabetes insipidus), the kidneys produce a large volume of dilute urine because the kidney tubules fail to respond to vasopressin (antidiuretic hormone) and are unable to reabsorb filtered water back into the body.
Often argininevasopressin resistance is hereditary, but it can be caused by medications or disorders that affect the kidneys.
Symptoms include excessive thirst and excretion of large amounts of urine.
Diagnosis of argininevasopressin resistance is based on tests of blood and urine.
Drinking increased amounts of water helps prevent dehydration.
To treat argininevasopressin resistance, people restrict salt in their diet and sometimes take medications to reduce the amount of urine excreted.
(See also Introduction to Disorders of Kidney Tubules.)
In argininevasopressin resistance, the kidneys do not respond to vasopressin (antidiuretic hormone), so they continue to excrete a large amount of dilute urine.
In (previously called central diabetes insipidus), which is more common, the pituitary gland fails to secrete vasopressin.
Causes of ArginineVasopressin Resistance
Normally, the kidneys adjust the concentration and amount of urine according to the body’s needs. The kidneys make this adjustment in response to the level of vasopressin in the blood. Vasopressin, which is secreted by the pituitary gland, signals the kidneys to conserve water and concentrate the urine. In argininevasopressin resistance, the kidneys fail to respond to the signal.
Argininevasopressin resistance may be
Hereditary
Acquired
Hereditary argininevasopressin resistance
In hereditary argininevasopressin resistance, the gene that typically causes the disorder is recessive and carried on the X chromosome, 1 of the 2 sex chromosomes, so usually only males develop symptoms. However, females who carry the gene can transmit the disease to their sons. Rarely, another abnormal gene can cause argininevasopressin resistance in both males and females.
Acquired argininevasopressin resistance
In the acquired form, argininevasopressin resistance may be caused by certain medications that block the action of vasopressin
Argininevasopressin resistance can also occur if the kidney is affected by disorders such as polycystic kidney disease, sickle cell anemia, medullary sponge kidney, infections (pyelonephritis) that are severe, amyloidosis, Sjögren syndrome, and certain cancers (for example, sarcoma or myeloma).
Also, high levels of calcium or low levels of potassium in the blood, especially if they persist, partially block the action of vasopressin.
Sometimes the cause is unknown.
Symptoms of ArginineVasopressin Resistance
The symptoms of argininevasopressin resistance are
Excessive thirst (polydipsia)
Excretion of large volumes of dilute urine (polyuria)
People may pass from 1 to 6 gallons (3 to 20 liters) of urine per day.
When argininevasopressin resistance is hereditary, symptoms usually start soon after birth. Because infants cannot communicate thirst, they may become very dehydrated. They may develop a fever accompanied by vomiting and seizures.
Older adults with dementia are also more likely to develop dehydration because they too may be unable to communicate thirst.
Diagnosis of ArginineVasopressin Resistance
Blood tests
Urine tests
Laboratory tests reveal high sodium levels in the blood and very dilute urine. A doctor may use a water deprivation test to help make the diagnosis.
Treatment of Arginine Vasopressin Resistance
Adequate amounts of water to drink
Diet and medications to decrease the volume of urine
To prevent dehydration, people with argininevasopressin resistance must drink adequate amounts of water as soon as they feel thirsty. Infants, young children, and very sick older adults must be given water often. People who drink enough water are not likely to become dehydrated, but several hours without water can lead to severe dehydration.
A diet low in salt and protein may help.
Nonsteroidal anti-inflammatory drugs (NSAIDs) and thiazide diuretics are sometimes used to treat this disorder. NSAIDs and thiazide diuretics act by different mechanisms to increase the amounts of sodium and water that are reabsorbed by the kidney. These changes decrease the volume of urine.
Prognosis for ArginineVasopressin Resistance
The prognosis is good if argininevasopressin resistance is diagnosed before the person suffers severe episodes of dehydration.
With treatment, an infant who has this disorder is likely to develop normally. However, if hereditary argininevasopressin resistance is not quickly diagnosed and treated, the brain may be damaged, leaving the infant with permanent intellectual disability. Frequent episodes of dehydration can also slow physical development.
In cases in which the disorder is not inherited, correction of the underlying abnormality usually helps kidney function return to normal.
More Information
The following English-language resource may be useful. Please note that THE MANUAL is not responsible for the content of this resource.
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK): Provides insight into ongoing research, consumer health information in English and Spanish, a blog, and community health and outreach programs.
