What is Diabetes Insipidus?

Diabetes insipidus (DI) is a rare problem where your body does not balance fluids well, making you pee a lot. Diabetes insipidus is affecting about 1 in 25,000 people worldwide. It is not the same as the more common diabetes (diabetes mellitus) that involves high sugar levels. Instead, diabetes insipidus makes your body produce a large amount of urine, sometimes up to 20 quarts a day, way more than the usual 1 to 3 quarts. This condition also makes you feel very, very thirsty all the time, known as polydipsia. The main issue in diabetes insipidus is a problem with a hormone called antidiuretic hormone (ADH or vasopressin). Your body either does not make enough of it, or your kidneys don’t use it properly. This leads to frequent urination.

If you do not drink enough water to replace what you are losing in urine, you can become dehydrated, which is not good for your health. Even though the name sounds similar, diabetes insipidus is not connected to the more common diabetes mellitus. They just share some symptoms like extreme thirst and peeing a lot. In severe cases, a person with diabetes insipidus can pee up to 20 liters in a day. Luckily, there are treatments to help with symptoms like managing thirst, reducing urine production, and preventing dehydration. There are different types of diabetes insipidus, including central, nephrogenic, and pregnancy-related, but there’s no complete cure yet. Medical interventions can, however, help make it more manageable.

Diabetes Insipidus vs. Diabetes Mellitus

Diabetes insipidus (DI) and diabetes mellitus (DM) might sound similar because they both involve increased thirst and urination, but they’re actually quite different. DI happens when the body lacks a hormone called antidiuretic hormone (ADH), which normally helps balance water in the body. This can be due to issues in the brain or kidneys. On the other hand, diabetes mellitus is a metabolic problem where blood sugar levels are too high, either because the body does not make enough insulin (Type 1) or because it can not use insulin effectively (Type 2).

In DI, the main hormone involved is ADH, while in DM, it is insulin. DI shows up with symptoms like extreme thirst and watery urine, while DM has signs like increased thirst, frequent urination, weight loss, tiredness, and slow wound healing. To figure out if someone has DI or DM, doctors do different tests. Treatment for DI involves replacing the missing ADH, while managing DM might include lifestyle changes, medications, or insulin. So, even though both conditions share some symptoms, they have different reasons and need different treatments.

Symptoms of Diabetes Insipidus

Signs of diabetes insipidus in adults can be:

  • Feeling very thirsty, especially liking cold water.
  • Peeing a lot with pale urine.
  • Waking up at night to pee and drink water.
  • Usually, adults pee about 1 to 3 quarts (1 to 3 liters) a day. But if someone has diabetes insipidus and drinks a ton of fluids, they might pee as much as 20 quarts (about 19 liters) a day.

For babies or young kids with diabetes insipidus, watch out for:

  • Lots of pale pee making diapers very wet.
  • Wetting the bed.
  • Being very thirsty and preferring water and cold drinks.
  • Losing weight.
  • Not growing well.
  • Throwing up.
  • Being cranky.
  • Having a fever.
  • Having trouble pooping.
  • Getting headaches.
  • Having sleep problems.
  • Having issues with vision.

Diabetes Insipidus Causes

Female urinary system

Your body has a special team called the urinary system, which includes the kidneys, ureters, bladder, and urethra. This team’s main job is to get rid of waste by making pee. The kidneys, located in the upper part of your belly, act like filters. They take out waste and extra liquid from your blood to create pee. The pee then travels through small tubes called ureters to reach the bladder, which is like a storage tank. When it’s time to go to the bathroom, the bladder lets the pee out through a tiny tube called the urethra. So, the urinary system works together to keep your body clean and healthy by getting rid of waste.

Pituitary gland and hypothalamus

The pituitary gland and the hypothalamus are in the brain. They control hormone production.

Diabetes insipidus is when the body has trouble keeping the right amount of water in it. Let’s break it down: our blood has water, and the kidneys filter this blood to clean out waste. Most of the cleaned water goes back into the blood, and a bit, along with waste, becomes urine, which eventually leaves our body.

Now, there’s a hormone called antidiuretic hormone (ADH) or vasopressin. This hormone is like a traffic controller for the filtered water. It’s made in the brain’s hypothalamus, stored in the pituitary gland (a small gland near the brain’s base), and its job is to guide the filtered water back into the bloodstream. If something goes wrong, like the brain making too little ADH or something blocking its effects, the body ends up making too much urine.

In simple terms, diabetes insipidus messes with this water-balancing act. What exactly goes wrong depends on the type of diabetes insipidus.

Types of Diabetes Insipidus

Central Diabetes Insipidus

Central diabetes insipidus (CDI) is a condition where the body doesn’t make enough of a hormone called antidiuretic hormone (ADH). ADH helps control how much water your body keeps or gets rid of. It’s made by the hypothalamus and released by the pituitary gland. When someone has central diabetes insipidus, they don’t have enough ADH. This makes it hard for their body to make urine concentrated enough. As a result, they end up making a lot of diluted urine, which makes them feel really thirsty, and they drink more fluids. The term “diabetes insipidus” is used because, like diabetes mellitus, it involves a lot of urination. However, the two conditions are different.

There are different reasons why someone might get central diabetes insipidus:

  1. Head Trauma: Injuries to the head, especially to the hypothalamus or pituitary gland, can affect the production or release of ADH.
  2. Tumors: Growths in or around the hypothalamus or pituitary gland can interfere with ADH production.
  3. Infections: Problems like inflammation, infections, or autoimmune disorders that affect the hypothalamus or pituitary gland can cause central diabetes insipidus.
  4. Surgery: Certain surgeries involving the pituitary gland or nearby areas might damage the tissues responsible for ADH production.
  5. Genetic Factors: In some rare cases, central diabetes insipidus can be inherited due to genetic mutations affecting ADH production.

The main signs of central diabetes insipidus include always feeling thirsty (polydipsia) and making a lot of watery urine (polyuria). If it’s not treated, it can lead to dehydration and problems with electrolytes. Doctors use tests, like a water deprivation test, to figure out if someone has central diabetes insipidus. Treatment often involves giving a synthetic form of ADH (desmopressin) through a nasal spray, oral medication, or injection.

Nephrogenic Diabetes Insipidus

Nephrogenic diabetes insipidus (NDI) is a condition where the kidneys don’t listen well to a hormone called antidiuretic hormone (ADH). Normally, ADH tells the kidneys to keep water, but in this condition, the message doesn’t get through properly. As a result, the body makes a lot of very watery urine.

Here’s why it happens:

  1. Genetic Factors: Sometimes, people inherit a problem with their kidneys that makes them not respond properly to ADH.
  2. Certain Medications: Some medicines, like lithium or certain diuretics, can cause nephrogenic DI.
  3. High Calcium Levels: Having too much calcium in the body, a condition called hypercalcemia, can lead to this issue.
  4. Chronic Kidney Disease: Problems with the kidneys, like chronic kidney disease, can affect how they respond to ADH.

The main signs of nephrogenic DI are feeling very thirsty all the time and making a lot of very diluted urine. These are similar to the symptoms of another condition called central diabetes insipidus. Doctors use different tests to figure out if someone has nephrogenic diabetes insipidus. Treatment may involve dealing with the underlying cause, adjusting medications, or using certain drugs to help. People with nephrogenic diabetes insipidus should work closely with their doctors to keep an eye on their condition. It’s important to drink enough water and manage how much fluid is taken in to avoid problems related to making too much urine.

Gestational diabetes insipidus

Gestational diabetes is a type of diabetes that develops during pregnancy. When a woman is pregnant, her body goes through hormonal changes, and sometimes these changes can make it harder for the body to use insulin effectively. Insulin is a hormone that helps control blood sugar levels.

  1. During Pregnancy: When a woman is pregnant, her body may need more insulin than usual to keep blood sugar levels in check.
  2. Gestational Diabetes: If the body can’t produce enough extra insulin during pregnancy, it can lead to gestational diabetes.
  3. Risk Factors: Certain factors increase the risk of gestational diabetes, such as being overweight, having a family history of diabetes, or being older than 25 during pregnancy.
  4. Effects on the Baby: Gestational diabetes can sometimes cause high blood sugar levels in the baby, which may lead to complications during birth.
  5. Testing and Management: Pregnant women are often screened for gestational diabetes, usually around the 24th to 28th week of pregnancy. If diagnosed, it can be managed through lifestyle changes, diet, and sometimes medication.
  6. Post-Pregnancy: Gestational diabetes usually goes away after the baby is born. However, women who have had gestational diabetes have a higher risk of developing type 2 diabetes later in life.

Pregnant women need to attend prenatal care and follow the advice of doctors to monitor and manage gestational diabetes. This helps ensure a healthy pregnancy and reduces the risk of complications for both the mother and the baby.

Primary Polydipsia

Primary polydipsia, also known as psychogenic polydipsia or compulsive water drinking, is a condition characterized by excessive and uncontrollable thirst, leading to the consumption of large amounts of water. The term “primary” indicates that the excessive thirst is not caused by another underlying medical condition.

  1. Excessive Thirst: People with primary polydipsia feel an intense and constant need to drink large quantities of water.
  2. Not Caused by Other Conditions: Unlike some other causes of excessive thirst, primary polydipsia is not linked to other medical issues like diabetes or kidney problems.
  3. Potential Impact on the Body: Drinking too much water can dilute the levels of sodium in the blood, leading to a condition called hyponatremia. This imbalance in electrolytes can have various effects on the body.
  4. Psychological Component: The excessive thirst in primary polydipsia often has a psychological or behavioral component. It may be associated with certain mental health conditions or habits.
  5. Diagnosis: Diagnosing primary polydipsia involves ruling out other potential causes of excessive thirst through medical tests.
  6. Treatment: Treatment may involve addressing any underlying psychological factors, managing water intake, and, in some cases, using medications to regulate thirst.

Risk Factors

Diabetes insipidus (DI) is a rare condition characterized by excessive thirst and the excretion of large amounts of diluted urine. There are different types of diabetes insipidus, and they can have different risk factors. Here are some general risk factors associated with diabetes insipidus:

  1. Damage to the Hypothalamus or Pituitary Gland: The most common cause of diabetes insipidus is damage to the hypothalamus or pituitary gland. This damage can result from head injuries, tumors, surgeries, or infections affecting these areas.
  2. Trauma or Surgery: Head injuries, particularly those affecting the hypothalamus or pituitary gland, can lead to diabetes insipidus. Additionally, surgical procedures in or around the brain may pose a risk.
  3. Infections: Infections such as meningitis or encephalitis can damage the hypothalamus or pituitary gland, increasing the risk of DI.
  4. Family History: While most cases of diabetes insipidus are not hereditary, there are rare cases where a genetic mutation can be involved. A family history of diabetes insipidus may be a risk factor in such instances.
  5. Certain Medications: Some medications, such as lithium (used in the treatment of bipolar disorder), can cause diabetes insipidus as a side effect.
  6. Pregnancy: In rare cases, pregnancy-related complications can lead to gestational diabetes insipidus. This is usually a temporary condition that resolves after childbirth.
  7. Autoimmune Disorders: In some cases, autoimmune diseases that affect the pituitary gland, such as lymphocytic hypophysitis, may increase the risk of diabetes insipidus.

DI is different from diabetes mellitus, which is the more common form of diabetes associated with problems in insulin production or utilization. Diabetes insipidus is related to problems with antidiuretic hormone (ADH), also known as vasopressin, which regulates the balance of water in the body.

Complications:

When someone has diabetes insipidus, it can lead to complications like dehydration. Dehydration occurs when the body loses too much fluid, resulting in various symptoms such as:

  1. Dry Mouth: Your mouth may feel dry.
  2. Thirst: You may feel an increased need to drink water.
  3. Extreme Tiredness: You might experience unusual fatigue.
  4. Dizziness: You may feel lightheaded or dizzy.
  5. Fainting: Dehydration can lead to a loss of consciousness.
  6. Nausea: You may feel queasy or nauseous.

Additionally, diabetes insipidus can disrupt the balance of minerals in the blood, known as electrolytes, which include sodium and potassium. An imbalance in electrolytes can result in the following symptoms:

  1. Weakness: You may feel generally weak or lack strength.
  2. Nausea: A feeling of sickness in the stomach.
  3. Vomiting: The act of forcefully expelling stomach contents.
  4. Loss of Appetite: A decrease in the desire to eat.
  5. Confusion: You may feel disoriented or have difficulty concentrating.

Diagnosing

To determine if someone has diabetes insipidus, healthcare providers use various tests:

  1. Water Deprivation Test:
    • You’ll be asked to stop drinking fluids for a few hours.
    • Changes in body weight, urine production, and the concentration of urine and blood are measured.
    • The healthcare provider may check the level of ADH (antidiuretic hormone) in your blood.
    • You might receive a synthetic form of ADH to see if your body responds appropriately.
  2. Urine Test:
    • Examining urine to detect excessive water content helps in identifying diabetes insipidus.
  3. Blood Tests:
    • Checking blood levels of substances like sodium, potassium, and calcium aids in diagnosis and can help identify the type of diabetes insipidus.
  4. Magnetic Resonance Imaging (MRI):
    • This imaging test investigates potential issues with the pituitary gland or hypothalamus.
    • It utilizes a strong magnetic field and radio waves to create detailed pictures of the brain.
  5. Genetic Testing:
    • If others in your family have experienced excessive urination or have been diagnosed with diabetes insipidus, your healthcare provider may suggest genetic testing.

These tests are important in understanding how your body handles fluids and whether there are underlying issues with hormones or genetic factors contributing to DI.

Diabetes Insipidus Treatment

If you have a mild case of diabetes insipidus, drinking more water can often prevent dehydration. However, the treatment approach varies depending on the type of diabetes insipidus.

  1. Central Diabetes Insipidus:
    • If a pituitary gland or hypothalamus disorder, like a tumor, causes central diabetes insipidus, addressing that disorder is the first step.
    • For ongoing treatment, a manufactured hormone called desmopressin (DDAVP, Nocdurna) is commonly used. This medication acts as a replacement for the lacking antidiuretic hormone (ADH), reducing urine production.
    • Desmopressin is available in pill, nasal spray, and injection forms.
    • Adjusting the dosage is crucial, as the body’s ADH production can vary. Taking too much desmopressin may lead to water retention and, in some cases, low sodium levels. Consult your healthcare provider for guidance on adjusting the dosage.
  2. Nephrogenic Diabetes Insipidus:
    • As the kidneys don’t respond well to ADH in this type, desmopressin is ineffective.
    • A low-salt diet may be recommended to decrease urine production.
    • Hydrochlorothiazide (Microzide), a diuretic, might be prescribed to help manage symptoms for some individuals with nephrogenic diabetes insipidus.
    • If your symptoms are medication-related, consult your healthcare provider before stopping any medication.
  3. Gestational Diabetes Insipidus:
    • Treatment involves using desmopressin, a manufactured hormone.
  4. Primary Polydipsia:
    • No specific medication treats this form of diabetes insipidus.
    • The primary approach is to reduce fluid intake.
    • If linked to a mental illness, addressing the mental health condition may alleviate symptoms.

Diabetes Insipidus ICD 10

The International Classification of Diseases, 10th Edition (ICD-10), provides a system for coding various diseases and conditions. The specific code for DI ICD-10 is:

E23.2 – Diabetes insipidus

This code is used to classify and document the diagnosis of DI in medical records and billing systems. It’s important to note that the ICD-10 coding system is regularly updated, so it’s advisable to check for any changes or revisions to the codes as needed. Additionally, healthcare professionals and coders should use the most recent edition of the ICD-10 for accurate coding.

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