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Diabetes insipidus

 

Diabetes insipidus is a rare disorder where the system the body uses to regulate its water levels becomes disrupted. This disruption leads to the two most common symptoms of diabetes insipidus:

  • excessive and prolonged thirst
  • passing large amounts of urine and needing to urinate frequently

Left untreated, diabetes insipidus can result in severe dehydration, shock (a sudden drop in blood pressure) and, in particularly vulnerable people, death.

Antidiuretic hormone (ADH)

The amount of water in the body is regulated by a hormone called antidiuretic hormone (ADH), also known as vasopressin. ADH is made by a part of the brain called the hypothalamus and is stored just below the brain, in the pituitary gland, until it is needed.

When the amount of water in the body becomes too low, ADH is released from the pituitary gland.

This helps to retain water in the body by stopping the kidneys from producing urine.

However, in diabetes insipidus, ADH does not stop the kidneys from producing urine and allows too much water to be passed from the body. This results in symptoms such as needing to pass large quantities of urine often and feeling extremely thirsty all the time. See Diabetes insipidus - symptoms for more information.

Types of diabetes insipidus

There are two main types of diabetes insipidus, which are described below.

Cranial diabetes insipidus

Cranial diabetes insipidus occurs when there is not enough ADH in the body to regulate the amount of urine that is produced.

This is the most common type of diabetes insipidus and can be caused by damage to the hypothalamus or pituitary gland, for example after an infection, operation or head injury.

In an estimated 30% of cases of cranial diabetes insipidus, the cause of the condition is unknown.

Nephrogenic diabetes insipidus

Nephrogenic diabetes insipidus occurs when there is enough ADH in the body but the kidneys fail to respond to it. It can run in families or it can be caused by kidney damage.

Some medications, especially lithium (used in the treatment of a number of serious mental health conditions, such as bipolar disorder), have been known to cause nephrogenic diabetes insipidus.

How common is diabetes insipidus?

Diabetes insipidus is a rare condition, affecting an estimated 1 in 25,000 people. As the condition is usually acquired through injury or infection, it can affect people of all ages.

Outlook

The outlook for diabetes insipidus can vary widely depending on the underlying cause. 

If it is diagnosed quickly before dehydration occurs, the outlook is moderate to good.

The symptoms of cranial central diabetes insipidus can usually be controlled with medication, while changes to diet are used to help treat nephrogenic diabetes. For more information, see Diabetes insipidus - treatment.

Different types of diabetes

It is important not to confuse diabetes insipidus with diabetes mellitus, which most people just know as ‘diabetes’. There are two types of diabetes mellitus: type 1 diabetes and type 2 diabetes.

Diabetes mellitus is far more common and occurs when there is too much glucose (sugar) in the blood.

The word ‘diabetes’ relates to the Greek verb for urination. It is used to refer to both diabetes insipidus and diabetes mellitus as they affect the chemical make-up of urine (although the treatments for each condition are very different).

The two main symptoms of diabetes insipidus are:

  • needing to pass large amounts of urine often
  • feeling thirsty all the time

These and other symptoms you may experience if you have diabetes insipidus are outlined below.

Passing excess urine

You may pass pale, watery urine as often as every 15-20 minutes. The amount of urine passed can range from 2.5 litres (4.4 pints) in mild cases to up to 15 litres (26 pints) in severe cases. 

Constant thirst

You may be constantly thirsty and have a 'dry' feeling that is always present, no matter how much water you drink.

Trouble sleeping and carrying out daily activities

If you need to pass urine often and always feel thirsty, your sleeping patterns and daily activities may be disrupted as a result. This can cause tiredness, irritability and difficulty concentrating, which can affect your daily life further.

Generally feeling unwell

If you have diabetes insipidus, you may feel generally unwell and 'run down' much of the time for no apparent reason.

Additional symptoms in children

In very young children who are unable to speak, it may be difficult to realise that they are excessively thirsty. Signs and symptoms that could suggest diabetes insipidus include:

  • excessive crying
  • irritability
  • slower than expected growth
  • high body temperature (hyperthermia)
  • unexplained weight loss

In older children, symptoms of diabetes insipidus include:wetting the bed

  • loss of appetite
  • fatigue

Toilet card

Constantly needing to pass urine can make it difficult to be out and about if you don't know where the nearest toilet is. If you have diabetes insipidus, the Pituitary Foundation can provide a toilet facility card, which will allow you to use toilets in public places if they are not easily available.

Diabetes insipidus is related to a hormone known as antidiuretic hormone (ADH). ADH is sometimes called vasopressin.

ADH is produced by the hypothalamus, a section in your brain that also controls mood and appetite. Until it is needed, ADH is stored in the pituitary gland, which is found just below your brain, behind the bridge of your nose.

The function of ADH is to regulate the level of water in your body by controlling the amount of urine that your kidneys produce. When the level of water in your body drops, your pituitary gland releases ADH to conserve water and stop the production of urine.

However, if you have diabetes insipidus, ADH fails to properly regulate your body's level of water, and allows too much urine to be produced and passed from your body.

In cranial diabetes insipidus, the body does not produce enough ADH, so excessive amounts of water are lost during urination.

In nephrogenic diabetes insipidus, ADH is produced at the right levels but, for a variety of possible reasons, the kidneys fail to respond to the ADH in the normal way.

Possible underlying causes for both types of diabetes insipidus are described below.

Causes of cranial diabetes insipidus

The three most common causes of cranial diabetes insipidus are: brain tumour

  • that damages the hypothalamus or pituitary gland, which accounts for 25% of cases
  • severe head injury that damages the hypothalamus or pituitary gland, which accounts for 16% of cases
  • complications that occur during brain surgery, which account for 20% of cases

In about a third of cases, no apparent cause can be found for development of the condition. These types of cases, known as idiopathic cases, appear to be related to the immune system attacking healthy brain tissue by mistake. What causes the immune system to do this is unclear.

Less common causes of cranial diabetic insipidus include:

  • cancers that spread from another part of the body, such as the lungs or the bone marrow, to the brain
  • Wolfram syndrome, a rare genetic disorder that also causes sight and vision loss
  • brain damage caused by a sudden loss of oxygen, which can occur during a stroke or drowning
  • infections, such as meningitis and encephalitis, that can damage the brain

Causes of nephrogenic diabetes insipidus

Your kidneys contain tubules called nephrons, which control how much water is reabsorbed into your body and how much is passed as urine.

In a healthy person, ADH signals to the nephrons to reabsorb water into the body. This does not occur in cases of nephrogenic diabetes insipidus, leading to the symptoms of constant thirst and excessive urine production.

The possible causes of nephrogenic diabetes insipidus are classed as either:

  • congenital, which means the condition is present at birth
  • acquired, which means the condition develops later in life due to an external factor

Congenital nephrogenic diabetes insipidus

Two genetic mutations (abnormal changes to the genes) have been identified that cause nephrogenic diabetes insipidus present at birth.

The first is known as the AVRP2 gene mutation and is responsible for 90% of all cases of congenital diabetes insipidus (although it is still very rare, occurring in an estimated 1 in every 250,000 births). This gene mutation can only be passed down by mothers (who are not affected) to their sons (who are affected).

The remaining 10% of cases of congenital nephrogenic diabetes insipidus are caused by the AQP2 gene mutation, which can affect both males and females.

Acquired nephrogenic diabetes insipidus

The most common cause of acquired nephrogenic diabetes insipidus is the medication lithium.

Lithium is widely used to treat bipolar disorder (manic depression), which is a mental health condition that can cause sudden and severe mood swings from a state of depression (feeling very sad) to a state of mania (feeling very happy and full of energy but acting irrationally).

Long-term use of lithium can damage the cells of the kidneys, meaning they no longer respond to ADH.

Just over half of all people on long-term lithium therapy will develop nephrogenic diabetes insipidus. Stopping treatment with lithium will often restore normal kidney function, though in many cases the damage to the kidneys is permanent.

Many people with bipolar disorder are willing to accept the risks associated with long-term lithium use as they feel they are outweighed by the improvement they experience with their mental health symptoms.

Due to the risks above, it is recommended that you have kidney function tests every three months if you are taking lithium. See the Health A-Z page on Bipolar disorder - treatment for more information.

Other causes of acquired nephrogenic diabetes insipidus include:

  • hypercalcaemia, a condition where there is too much calcium in the blood (high calcium levels can damage the kidneys)
  • hypokalemia, a condition where there is not enough potassium in the blood (all the cells in the body, including kidney cells, require potassium to function properly)
  • the kidneys becoming damaged by infection (the medical term for kidney infection is pyelonephritis)
  • ureteral obstruction, when one or both of the tubes that connect the kidneys to the bladder (the ureters) become blocked by an object such as a kidney stone, which can then damage the kidneys
GlossaryBrain
The brain controls thought, memory and emotion. It sends messages to the body controlling movement, speech and senses.
Genetic
Genetic is a term that refers to genes, the characteristics inherited from a family member.
Kidney
Kidneys are a pair of bean-shaped organs located at the back of the abdomen, which remove waste and extra fluid from the blood and pass them out of the body as urine.
Pituitary gland
The pituitary gland is a pea-sized gland in the centre of the head, which hangs below the brain and produces hormones.

See your GP if you think you may have diabetes insipidus. They will ask you about your symptoms and will carry out several tests. Your GP may refer you to an endocrinologist (a specialist in hormone conditions) for these tests.

As symptoms such as producing lots of urine can be linked to other conditions, including diabetes mellitus, tests are needed to confirm or rule out a diagnosis of diabetes insipidus. The tests can also identify which of the two types of diabetes insipidus you have: cranial diabetes insipidus or nephrogenic diabetes insipidus.

The tests you may have to confirm a diagnosis of diabetes insipidus are described below.

Water deprivation test

If you have a water deprivation test, you will not be allowed to drink any liquid for several hours to see how your body responds. If you have diabetes insipidus, you will continue to pass large amounts of dilute urine, when normally you would only pass a small amount of concentrated urine.

During the water deprivation test, your GP or endocrinologist will take urine samples to measure the amount of urine you are producing. They may also carry out a blood test to assess the levels of antidiuretic hormone (ADH) in your blood.

Your blood and urine may also be tested for substances such as blood sugar (glucose), calcium and potassium. If you have diabetes insipidus, your urine will be very diluted, with low levels of other substances. However, if there is a high amount of sugar in your urine, you may have diabetes mellitus, not diabetes insipidus.

Antidiuretic hormone (ADH) test

After the water deprivation test, your GP or endocrinologist will give you a small dose of ADH, usually as an injection. This will show how your body reacts to the hormone and can help identify which type of diabetes insipidus you have.

If the dose of ADH causes you to stop producing urine, it is likely your condition is due to a shortage of ADH. If this is the case, you may be diagnosed with cranial diabetes insipidus.

However, if you continue to produce lots of urine despite the dose of ADH, it suggests that there is already enough ADH in your body, but that your kidneys are not responding to it. In this case, you may be diagnosed with nephrogenic diabetes insipidus.

MRI scan

Magnetic resonance imaging (MRI) is a scan that uses a strong magnetic field and radio waves to produce images of the inside of your body, including your brain.

You may need an MRI scan if your GP or endocrinologist thinks you have cranial diabetes insipidus as a result of damage to your hypothalamus or pituitary gland.

If your condition is due to an abnormality in your hypothalamus or pituitary gland, such as a tumour, it will need to be treated before you can receive treatment for diabetes insipidus.

GlossaryDehydrated
Dehydration is an excessive loss of fluids and minerals from the body.
Glucose
Glucose (or dextrose) is a type of sugar that is used by the body to produce energy.
Kidneys
Kidneys are a pair of bean-shaped organs located at the back of the abdomen, which remove waste and extra fluid from the blood and pass them out of the body as urine.
MRI
MRI stands for magnetic resonance imaging. It is the use of magnets and radio waves to take detailed pictures of inside the body.
Pituitary gland
The pituitary gland is a pea-sized gland in the centre of the head, which hangs below the brain and produces hormones.
Urinalysis
Urinalysis/UA is when a urine sample is tested, commonly to check for any signs of infection or protein or sugar levels.

Treatment for diabetes insipidus aims to reduce the amount of urine that your body produces. Depending on the type of diabetes insipidus you have, there are several ways of treating your condition and controlling your symptoms.

Treating cranial diabetes insipidus

If you have mild cranial diabetes insipidus, you may not need any medical treatment for your condition.

Cranial diabetes insipidus is considered to be mild if you produce approximately three to four litres of urine over 24 hours.

If this is the case, you may be able to ease your symptoms by increasing the amount of water you drink, to avoid dehydration. Your GP or endocrinologist (specialist in hormone conditions) may advise you to drink a certain amount of water every day, usually at least 2.5 litres.

However, if your cranial diabetes insipidus is more severe, drinking water may not be enough to keep your symptoms under control. As your condition is due to a shortage of antidiuretic hormone (ADH), your GP or endocrinologist may prescribe a treatment that takes the place of ADH, known as desmopressin.

Desmopressin

Desmopressin is a manufactured version of ADH, and is more powerful than the ADH naturally produced by your body. It works just like natural ADH, stopping your kidneys producing urine when the level of water in your body is low.

Desmopressin is usually taken as a tablet known as DDAVP or nordurine or it may be given as a nasal spray. If you are prescribed DDAVP tablets, you may need to take more than twice a day.  If you are prescribed a Desmospray or DDAVP nasal spray, you will need to spray it inside your nose once or twice a day, where it is quickly absorbed into your bloodstream.  Your GP or endocrinologist may suggest switching your treatment to DDAVP tablets if you develop a cold that prevents you from using the nasal spray.

Desmopressin is very safe to use. There are very few side effects, but they can include:

  • headache
  • stomach pain
  • nausea
  • blocked or runny nose
  • nosebleeds

If you take too much desmopressin or drink too much fluid while taking it, it can cause your body to retain too much water. This can result in:

  • headaches
  • dizziness
  • feeling bloated
  • a dangerously low level of sodium (salt) in your blood (known as hyponatraemia)

Symptoms of hyponatraemia include:

  • an unusually bad or prolonged headache
  • confusion
  • unexplained weight gain
  • nausea
  • vomiting

If you think you may have hyponatraemia, immediately stop taking the medication and call your GP for advice. If this is not possible, go to your local accident and emergency department.

Treating nephrogenic diabetes insipidus

If your nephrogenic diabetes insipidus is caused by taking a particular medication, such as lithium or tetracycline, your GP or endocrinologist may stop your treatment and suggest an alternative medication. Do not stop taking any medication unless you have been told to do so by a healthcare professional.

As nephrogenic diabetes insipidus results from your kidneys not responding to ADH, rather than a shortage of ADH, it cannot be treated with desmopressin. However, it is still important to drink plenty of water to avoid dehydration.

If your condition is mild, your GP or endocrinologist may suggest changing your diet so it is very low in salt and protein, which will help your kidneys produce less urine. This may mean eating less salt and less protein-rich food, such as processed foods, meat, eggs and nuts. Do not alter your diet without medical advice. Your GP or endocrinologist can advise you about which foods to cut down on.

If your nephrogenic diabetes insipidus is more severe, you may be prescribed a combination of a thiazide diuretic and an NSAID (such as ibuprofen) which may help reduce the amount of urine your kidneys produce

GlossaryAnti-inflammatories
Anti-inflammatory medicines reduce swelling and inflammation.
Diuretics
Diuretic medicine increases the production and flow of urine from the body, used to remove excess fluid from the body.

 

In some cases, diabetes insipidus can cause complications, particularly if it is undiagnosed or poorly controlled.

Dehydration

If you have diabetes insipidus, your body finds it difficult to retain enough water, even if you drink fluid constantly. This can lead to dehydration, which is a severe lack of water in your body.

If you or someone you know has diabetes insipidus, it is important to look out for the signs and symptoms of dehydration, which may include:

  • dry mouth and lips
  • sunken features (particularly the eyes)
  • headaches
  • dizziness
  • confusion and irritability

Dehydration can be treated by rebalancing the level of water in your body. If you are very dehydrated, it is better to drink a rehydration fluid rather than plain water, as this will replace lost minerals, salts and sugars as well as lost water.

If you are severely dehydrated, you may need treatment in hospital, where your fluids are replaced intravenously (through a drip into a vein).

Electrolyte imbalance

Diabetes insipidus can also cause an electrolyte imbalance. Electrolytes are minerals in your blood that have a tiny electric charge, such as sodium, calcium, potassium, chlorine, magnesium and bicarbonate. Along with antidiuretic hormone (ADH), electrolytes help maintain the balance of water in your body.

If you have diabetes insipidus, these electrolytes can become unbalanced and the amount of water in your body is affected. This can cause dehydration and disrupt other body functions, such as the way muscles work, which can lead to headache, fatigue, irritability and muscle pain.

An electrolyte imbalance can be treated in the same way as dehydration, with a rehydration fluid containing replacement electrolytes.

GlossaryDehydrated
Dehydration is an excessive loss of fluids and minerals from the body.
Fatigue
Fatigue is extreme tiredness and lack of energy.
Fever
A high temperature, also known as a fever, is when someone's body temperature is 38C (100.4F) or above.
Vomiting
Vomiting is when you bring up the contents of your stomach through your mouth.

Content provided by NHS Choices www.nhs.uk and adapted for Ireland by the Health A-Z.