BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuNephrogenic diabetes insipidusNephrogenic diabetes insipidus; Acquired nephrogenic diabetes insipidus; Congenital nephrogenic diabetes insipidus; NDINephrogenic diabetes insipidus (NDI) is a disorder in which a defect in the small tubes (tubules) in the kidneys causes a person to pass a large amount of urine and lose too much water. Causes Normally, the kidney tubules allow most water in the blood to be filtered and returned to the blood.NDI occurs when the kidney tubules do not respond to a hormone in the body called antidiuretic hormone (ADH), also called vasopressin. ADH normally causes the kidneys to make the urine more concentrated.Antidiuretic hormoneAntidiuretic blood test measures the level of antidiuretic hormone (ADH) in blood.Read Article Now Book Mark Article As a result of not responding to the ADH signal, the kidneys release too much water into the urine. This causes the body to produce a large quantity of very dilute urine.NDI is very rare. Congenital nephrogenic diabetes insipidus means it is present at birth. It is a result of a genetic variant passed down through families. Men are usually affected, though women can pass this gene on to their children.Most commonly, NDI develops because of other reasons. This is called an acquired disorder. Factors that can trigger the acquired form of this condition include:Blockage in the urinary tract High blood calcium levels Low blood potassium levels Use of certain medicines (lithium, demeclocycline, amphotericin B) Symptoms You may have intense or uncontrollable thirst, and crave ice water. You will produce large amounts of urine, usually more than 3 liters, and up to 15 liters per day. The urine is very dilute and looks almost like water. You may need to urinate every hour or even more, even during the night when you are not eating or drinking as much.If you do not drink enough fluids, dehydration can result. Symptoms may include:Dry mucous membranes Dry skin Sunken appearance to eyes Sunken fontanelles (soft spot) in infants Changes in memory or balanceOther symptoms that can occur due to lack of fluids, causing dehydration, include:Fatigue, feeling weak Headache HeadacheA headache is pain or discomfort in the head, scalp, or neck. Serious causes of headaches are rare. Most people with headaches can feel much better...ImageRead Article Now Book Mark Article Irritability Low body temperature Muscle pain Rapid heart rate Weight loss A change in alertness, and even comaComaDecreased alertness is a state of reduced awareness and is often a serious condition. A coma is the most severe state of decreased alertness in which...Read Article Now Book Mark Article Exams and Tests Your health care provider will examine you and ask about your or your child's symptoms.A physical exam may reveal: Low blood pressure Rapid pulse Shock ShockShock is a life-threatening condition that occurs when the body is not getting enough blood flow. Lack of blood flow means the cells and organs do n...ImageRead Article Now Book Mark Article Signs of dehydration Tests may reveal:High serum osmolality High serum osmolalityOsmolality blood test is a test that measures the concentration of all chemical particles found in the fluid part of blood. Osmolality in the urine c...ImageRead Article Now Book Mark Article High urine output, regardless of how much fluid you drink Kidneys do not concentrate urine when you are given ADH (usually a medicine called desmopressin) Low urine osmolality Urine osmolalityThe osmolality urine test measures the concentration of chemicals in urine. Osmolality in the blood can be measured using a blood test.ImageRead Article Now Book Mark Article Normal or high blood ADH levels Other tests that may be done include:Blood electrolyte test Blood electrolyte testThe sodium blood test measures the concentration of sodium in the blood. Sodium can also be measured using a urine test.ImageRead Article Now Book Mark Article Urine 24-hour volume Urine 24-hour volumeThe urine 24-hour volume test measures the amount of urine produced in a day. The amounts of creatinine, protein, and other chemicals released into ...ImageRead Article Now Book Mark Article Urine concentration test Urine concentration testA urine concentration test measures the ability of the kidneys to conserve or excrete water.ImageRead Article Now Book Mark Article Urine specific gravity Urine specific gravityUrine specific gravity is a laboratory test that shows the total concentration of all chemical particles in the urine.ImageRead Article Now Book Mark Article Supervised water deprivation testWater deprivation testA urine concentration test measures the ability of the kidneys to conserve or excrete water.ImageRead Article Now Book Mark Article Treatment The goal of treatment is to control the body's fluid levels. A large amount of fluids will be given. The amount should be about equal to the amount of water being lost in the urine.If the condition is due to a certain medicine, stopping the medicine may improve symptoms. But, do not stop taking any medicine without first talking to your provider.Medicines may be given to improve symptoms by reducing urine output. Outlook (Prognosis) If a person drinks enough water, this condition will not have much effect on the fluid or electrolyte balance of the body. Sometimes, passing a lot of urine for a long time can cause other electrolyte problems. ElectrolyteElectrolytes are minerals in your blood and other body fluids that carry an electric charge. Electrolytes affect how your body functions in many ways...Read Article Now Book Mark Article If the person does not drink enough fluids, high urine output may cause dehydration and high levels of sodium in the blood. NDI that is present at birth is a long-term condition requiring lifelong treatment. Possible Complications Untreated, NDI may cause any of the following:Dilation of the ureters and bladder High blood sodium (hypernatremia) HypernatremiaThe sodium blood test measures the concentration of sodium in the blood. Sodium can also be measured using a urine test.ImageRead Article Now Book Mark Article Severe dehydration Shock Coma When to Contact a Medical Professional Contact your provider if you or your child has symptoms of this disorder. Prevention Congenital NDI cannot be prevented.Treating the disorders that can lead to the acquired form of the condition may prevent it from developing in some cases. Open ReferencesReferencesAl-Awqati Q, Radhakrishnan J. Disorders of sodium and water. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 102.Bock ME, Dixon BP. Diabetes insipidus. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds. Nelson Textbook of Pediatrics. 22nd ed. Philadelphia, PA: Elsevier; 2025:chap 570.Bockenhauer D. Fluid, electrolyte, and acid-base disorders in children. In: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. Brenner and Rector's The Kidney. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 73.Scheinman SJ. Genetically based kidney transport disorders. In: Gilbert SJ, Weiner DE, eds. National Kidney Foundation Primer on Kidney Diseases. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 37.Thompson CJ, Garrah A, Disorders of sodium, diabetes insipidus and hyponatremia. In: Robertson RP, eds. DeGroot's Endocrinology. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 12.AllVideoImagesTogKidney anatomy - illustration The kidneys are responsible for removing wastes from the body, regulating electrolyte balance and blood pressure, and the stimulation of red blood cell production.Kidney anatomyillustrationKidney anatomy - illustration The kidneys are responsible for removing wastes from the body, regulating electrolyte balance and blood pressure, and the stimulation of red blood cell production.Kidney anatomyillustration Tests for Nephrogenic diabetes insipidus Urine concentration testRelated Information Central diabetes insipidus(Condition)Antidiuretic hormone blood test(Medical Test)Hypothalamus(Special Topic)Thirst - excessive(Symptoms)Electrolytes(Special Topic)Urination - excessive amount(Symptoms)Dehydration(Condition)Diabetes insipidus(Condition)Sodium blood test(Medical Test) Review Date: 3/31/2024 Reviewed By: Walead Latif, MD, Nephrologist and Clinical Associate Professor, Rutgers Medical School, Newark, NJ. 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Nephrogenic diabetes insipidusNephrogenic diabetes insipidus; Acquired nephrogenic diabetes insipidus; Congenital nephrogenic diabetes insipidus; NDINephrogenic diabetes insipidus (NDI) is a disorder in which a defect in the small tubes (tubules) in the kidneys causes a person to pass a large amount of urine and lose too much water. Causes Normally, the kidney tubules allow most water in the blood to be filtered and returned to the blood.NDI occurs when the kidney tubules do not respond to a hormone in the body called antidiuretic hormone (ADH), also called vasopressin. ADH normally causes the kidneys to make the urine more concentrated.Antidiuretic hormoneAntidiuretic blood test measures the level of antidiuretic hormone (ADH) in blood.Read Article Now Book Mark Article As a result of not responding to the ADH signal, the kidneys release too much water into the urine. This causes the body to produce a large quantity of very dilute urine.NDI is very rare. Congenital nephrogenic diabetes insipidus means it is present at birth. It is a result of a genetic variant passed down through families. Men are usually affected, though women can pass this gene on to their children.Most commonly, NDI develops because of other reasons. This is called an acquired disorder. Factors that can trigger the acquired form of this condition include:Blockage in the urinary tract High blood calcium levels Low blood potassium levels Use of certain medicines (lithium, demeclocycline, amphotericin B) Symptoms You may have intense or uncontrollable thirst, and crave ice water. You will produce large amounts of urine, usually more than 3 liters, and up to 15 liters per day. The urine is very dilute and looks almost like water. You may need to urinate every hour or even more, even during the night when you are not eating or drinking as much.If you do not drink enough fluids, dehydration can result. Symptoms may include:Dry mucous membranes Dry skin Sunken appearance to eyes Sunken fontanelles (soft spot) in infants Changes in memory or balanceOther symptoms that can occur due to lack of fluids, causing dehydration, include:Fatigue, feeling weak Headache HeadacheA headache is pain or discomfort in the head, scalp, or neck. Serious causes of headaches are rare. Most people with headaches can feel much better...ImageRead Article Now Book Mark Article Irritability Low body temperature Muscle pain Rapid heart rate Weight loss A change in alertness, and even comaComaDecreased alertness is a state of reduced awareness and is often a serious condition. A coma is the most severe state of decreased alertness in which...Read Article Now Book Mark Article Exams and Tests Your health care provider will examine you and ask about your or your child's symptoms.A physical exam may reveal: Low blood pressure Rapid pulse Shock ShockShock is a life-threatening condition that occurs when the body is not getting enough blood flow. Lack of blood flow means the cells and organs do n...ImageRead Article Now Book Mark Article Signs of dehydration Tests may reveal:High serum osmolality High serum osmolalityOsmolality blood test is a test that measures the concentration of all chemical particles found in the fluid part of blood. Osmolality in the urine c...ImageRead Article Now Book Mark Article High urine output, regardless of how much fluid you drink Kidneys do not concentrate urine when you are given ADH (usually a medicine called desmopressin) Low urine osmolality Urine osmolalityThe osmolality urine test measures the concentration of chemicals in urine. Osmolality in the blood can be measured using a blood test.ImageRead Article Now Book Mark Article Normal or high blood ADH levels Other tests that may be done include:Blood electrolyte test Blood electrolyte testThe sodium blood test measures the concentration of sodium in the blood. Sodium can also be measured using a urine test.ImageRead Article Now Book Mark Article Urine 24-hour volume Urine 24-hour volumeThe urine 24-hour volume test measures the amount of urine produced in a day. The amounts of creatinine, protein, and other chemicals released into ...ImageRead Article Now Book Mark Article Urine concentration test Urine concentration testA urine concentration test measures the ability of the kidneys to conserve or excrete water.ImageRead Article Now Book Mark Article Urine specific gravity Urine specific gravityUrine specific gravity is a laboratory test that shows the total concentration of all chemical particles in the urine.ImageRead Article Now Book Mark Article Supervised water deprivation testWater deprivation testA urine concentration test measures the ability of the kidneys to conserve or excrete water.ImageRead Article Now Book Mark Article Treatment The goal of treatment is to control the body's fluid levels. A large amount of fluids will be given. The amount should be about equal to the amount of water being lost in the urine.If the condition is due to a certain medicine, stopping the medicine may improve symptoms. But, do not stop taking any medicine without first talking to your provider.Medicines may be given to improve symptoms by reducing urine output. Outlook (Prognosis) If a person drinks enough water, this condition will not have much effect on the fluid or electrolyte balance of the body. Sometimes, passing a lot of urine for a long time can cause other electrolyte problems. ElectrolyteElectrolytes are minerals in your blood and other body fluids that carry an electric charge. Electrolytes affect how your body functions in many ways...Read Article Now Book Mark Article If the person does not drink enough fluids, high urine output may cause dehydration and high levels of sodium in the blood. NDI that is present at birth is a long-term condition requiring lifelong treatment. Possible Complications Untreated, NDI may cause any of the following:Dilation of the ureters and bladder High blood sodium (hypernatremia) HypernatremiaThe sodium blood test measures the concentration of sodium in the blood. Sodium can also be measured using a urine test.ImageRead Article Now Book Mark Article Severe dehydration Shock Coma When to Contact a Medical Professional Contact your provider if you or your child has symptoms of this disorder. Prevention Congenital NDI cannot be prevented.Treating the disorders that can lead to the acquired form of the condition may prevent it from developing in some cases. Open ReferencesReferencesAl-Awqati Q, Radhakrishnan J. Disorders of sodium and water. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 102.Bock ME, Dixon BP. Diabetes insipidus. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds. Nelson Textbook of Pediatrics. 22nd ed. Philadelphia, PA: Elsevier; 2025:chap 570.Bockenhauer D. Fluid, electrolyte, and acid-base disorders in children. In: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. Brenner and Rector's The Kidney. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 73.Scheinman SJ. Genetically based kidney transport disorders. In: Gilbert SJ, Weiner DE, eds. National Kidney Foundation Primer on Kidney Diseases. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 37.Thompson CJ, Garrah A, Disorders of sodium, diabetes insipidus and hyponatremia. In: Robertson RP, eds. DeGroot's Endocrinology. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 12.
