A kidney disorder in which the body no longer responds to a natural hormone known as antidiuretic hormone (ADH) is called nephrogenic diabetes insipidus. Thus, it produces too much urine that often causes quick and dangerous (sometimes) dehydration. This condition is often inherited from biological parents, but it may also occur due to certain medicines or other health conditions.
In other words, a health condition that occurs when the kidneys cannot properly balance fluids in the body because they do not respond to ADH (also known as vasopressin) is called nephrogenic diabetes insipidus. Without treatment, it may lead to a serious form of dehydration.
Healthcare providers do not know how many people have nephrogenic diabetes insipidus, but they think it happens quite rarely.
Types of Diabetes Insipidus
In general, there are 4 types of diabetes insipidus. For example:
- Central diabetes insipidus – This type of the condition causes damage to the pituitary gland or hypothalamus. This damage affects the body’s ability to produce, store, and release ADH.
- Nephrogenic diabetes insipidus – In such cases, the kidneys do not respond properly to ADH, causing overproduction of urine.
- Dipsogenic diabetes insipidus – This form of diabetes insipidus is also known as primary polydipsia, and it occurs when the hypothalamus has a defective thirst mechanism. As a result, it may lead to severe thirst that makes you drink excessive amounts of liquids.
- Gestational diabetes insipidus – This form of condition occurs during pregnancy.
What is the Difference between Nephrogenic Diabetes Insipidus and Diabetes Mellitus?
While both disorders have some similar symptoms, they are totally different. For instance, diabetes mellitus occurs more commonly, and it involves high glucose levels in the blood caused by a lack or improper use of the hormone Insulin.
Symptoms
In most cases, people with nephrogenic diabetes insipidus experience the following symptoms. For example:
- Dehydration symptoms (including dry mouth, fatigue or extreme tiredness, dizziness, and others)
- Excessive thirst
- Large amounts of urine output (more than 3 liters per day)
This condition may also occur in infants. However, they can experience other symptoms. These include:
If you or your child experiences any of the previous symptoms, do not hesitate to see a doctor for a diagnosis and treatment. Otherwise, it may lead to dangerous dehydration.
Causes
Normally, the kidneys filter the blood to remove water and excess fluid from the body. They leave the body through the urine. A hormone that helps the body keep the balance between fluid intake and urine output is called vasopressin (ADH). In a healthy person, the body understands when to produce less urine. For instance, if you experience moderate to severe sweating or drink less fluid, the body produces less urine due to an increased level of vasopressin hormone. When the body produces more urine, the levels of ADH are low.
However, in people with nephrogenic diabetes insipidus, the body does not respond to ADH, and it may produce too much urine when it is not needed. Furthermore, the acquired form of this disorder is more common, and it causes damage to the body’s ability to respond to vasopressin. Check some disorders that may cause damage to the kidneys below:
- Amyloidosis
- Chronic kidney disease
- Some types of cancer
- Polycystic kidney disease
- Medullary sponge kidney
- Pyelonephritis (infection of the kidneys)
In addition, the following things may also cause this condition. Examples include:
- Some medicines (including Lithium)
- Reduced levels of Potassium in the blood (also called hypokalemia)
- Hypercalcemia (increased levels of Calcium in the blood)
- Sjogren’s syndrome
- Sickle cell anemia
- Urinary tract obstruction
All people who develop an inherited nephrogenic diabetes insipidus have a mutation in the AVPR2 or AQP2 genes. Normally, these genes provide information about how much water to include in urine. Mostly, the symptoms of this form of nephrogenic diabetes insipidus often appear in the first 30 days of life.
What Are The Potential Complications of Nephrogenic Diabetes Insipidus?
People with this condition may also experience some complications, especially if they do not get treatment. Examples include:
- Dehydration
- Infections (including urinary tract infections and kidney infections)
- Developmental delays, damage to the brain, or intellectual disability (in infants)
- Kidney failure
- Electrolyte imbalances
- Coma
- Blockages in the urinary tract
- Muscle pain
- Fast heart rate
This document does not contain a complete list of complications, but you can consult with your doctor about measures to reduce the risk or even prevent them. In addition, there is no way to prevent this condition. However, you can reduce the risk of the acquired form by managing chronic health conditions (such as hypertension, high cholesterol, and others), quitting smoking, stopping the use of certain medicines, limiting or avoiding alcoholic drinks, and others. For more details, discuss it with your doctor.
Diagnosis
Commonly, physicians perform the following tests to confirm or rule out nephrogenic diabetes insipidus. Check some tests below:
- Blood tests
- Urine tests (urinalysis)
- Water deprivation test – During this test, you should not drink any liquid for a few hours. Thereafter, assess urine output.
- MRI (magnetic resonance imaging) – This is an imaging test used to produce detailed images of different parts of the body. In such cases, doctors will examine the pituitary gland, hypothalamus, and the kidneys.
- Genetic tests – To perform this test, doctors will take a sample of blood to check for the abnormal genes that cause this disease.
Treatment
When this condition is chronic (long-term), the treatment goal is to prevent dehydration. For example:
- Dietary changes
- Frequent drinks of water throughout the day (whenever you feel thirsty)
- Medicines to reduce the volume of urine. These include Thiazide diuretics, prescription nonsteroidal anti-inflammatory drugs (NSAIDs)
Healthcare professionals may also recommend limiting alcohol, caffeine, salt, and protein, along with the previous treatments.
Frequently Asked Questions
Is there a cure for nephrogenic diabetes insipidus?
Unfortunately, this disorder cannot be cured. However, the acquired form of the disease can be reversed by treating the underlying condition.
When should I seek medical attention for nephrogenic diabetes insipidus?
If you experience increased or reduced urine output, pain, or other concerning symptoms, do not hesitate to see a doctor for a diagnosis. If you are urinating more than 2 liters per day or frequently (more than 7 times per day), it may indicate a problem.
What causes diabetes insipidus?
This disorder occurs due to a problem with a chemical called arginine vasopressin (AVP). It is also called antidiuretic hormone (ADH). Moreover, in people with nephrogenic diabetes insipidus, the kidneys do not respond to ADH, causing an overproduction of urine. If you have any other questions, ask your healthcare provider.
