Often nephrogenic diabetes insipidus is hereditary, but it can be caused by drugs or disorders that affect the kidneys.
Symptoms include excessive thirst and excretion of large amounts of urine.
Diagnosis of nephrogenic diabetes insipidus is based on tests of blood and urine.
Drinking increased amounts of water helps prevent dehydration.
To treat nephrogenic diabetes insipidus, people restrict salt in their diet and sometimes take drugs to reduce the amount of urine excreted.
(See also Introduction to Disorders of Kidney Tubules Introduction to Disorders of Kidney Tubules The kidneys filter and cleanse the blood. They also maintain the body’s balance of water, electrolytes (such as sodium, potassium, bicarbonate, and chloride), and nutrients in the blood. The... read more .)
Both diabetes insipidus and the better-known type of diabetes, diabetes mellitus Diabetes Mellitus (DM) Diabetes mellitus is a disorder in which the body does not produce enough or respond normally to insulin, causing blood sugar (glucose) levels to be abnormally high. Urination and thirst are... read more , result in the excretion of large volumes of urine. Otherwise, the two types of diabetes are very different.
Two types of diabetes insipidus exist:
In nephrogenic diabetes insipidus, the kidneys do not respond to vasopressin (antidiuretic hormone), so they continue to excrete a large amount of dilute urine.
In central diabetes insipidus Central Diabetes Insipidus Central diabetes insipidus is a lack of the hormone vasopressin (antidiuretic hormone) that causes excessive production of very dilute urine (polyuria). Central diabetes insipidus has several... read more , which is more common, the pituitary gland fails to secrete vasopressin.
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 nephrogenic diabetes insipidus, the kidneys fail to respond to the signal.
Nephrogenic diabetes insipidus may be
In hereditary nephrogenic diabetes insipidus, the gene that typically causes the disorder is recessive and carried on the X chromosome, one of the two 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 nephrogenic insipidus in both males and females.
In the acquired form, nephrogenic diabetes insipidus may be caused by certain drugs that block the action of vasopressin, such as lithium.
Nephrogenic diabetes insipidus can also occur if the kidney is affected by disorders such as polycystic kidney disease Polycystic Kidney Disease(PKD) Polycystic kidney disease is a hereditary disorder in which many fluid-filled sacs (cysts) form in both kidneys. The kidneys grow larger but have less functioning tissue. Polycystic kidney disease... read more , sickle cell anemia Sickle Cell Disease Sickle cell disease is an inherited genetic abnormality of hemoglobin (the oxygen-carrying protein found in red blood cells) characterized by sickle (crescent)-shaped red blood cells and chronic... read more , medullary sponge kidney Medullary Sponge Kidney Medullary sponge kidney is an uncommon disorder in which the urine-containing tubules of the kidneys are dilated. Medullary sponge kidney is usually caused by a nongenetic abnormality that occurs... read more , infections (pyelonephritis Kidney Infection Pyelonephritis is a bacterial infection of one or both kidneys. Infection can spread up the urinary tract to the kidneys, or uncommonly the kidneys may become infected through bacteria in the... read more ) that are severe, amyloidosis Amyloidosis Amyloidosis is a rare disease in which abnormally folded proteins form amyloid fibrils that accumulate in various tissues and organs, sometimes leading to organ dysfunction, organ failure, and... read more , Sjögren syndrome Sjögren Syndrome Sjögren syndrome is a common autoimmune rheumatic disorder and is characterized by excessive dryness of the eyes, mouth, and other mucous membranes. White blood cells can infiltrate and damage... read more , 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.
The symptoms of nephrogenic diabetes insipidus are
People may pass from 1 to 6 gallons (3 to 20 liters) of urine per day.
When nephrogenic diabetes insipidus 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 people with dementia Dementia Dementia is a slow, progressive decline in mental function including memory, thinking, judgment, and the ability to learn. Typically, symptoms include memory loss, problems using language and... read more are also more likely to develop dehydration because they too may be unable to communicate thirst.
Laboratory tests reveal high sodium levels in the blood and very dilute urine. A doctor may use a water deprivation test Central diabetes insipidus is a lack of the hormone vasopressin (antidiuretic hormone) that causes excessive production of very dilute urine (polyuria). Central diabetes insipidus has several... read more to help make the diagnosis.
The prognosis is good if nephrogenic diabetes insipidus 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 nephrogenic diabetes insipidus 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.
To prevent dehydration, people with nephrogenic diabetes insipidus must drink adequate amounts of water as soon as they feel thirsty. Infants, young children, and very sick older people must be given water often. People who drink enough water are not likely to become dehydrated, but several hours without water can lead to serious 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.
The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.
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