Diabetes Insipidus

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AVP-D is the new name for the condition of diabetes insipidus, which is a condition faced my many hypothalamic-pituitary brain tumor patients.

Diabetes insipidus (DI) is a rare disorder characterized by excessive thirst and the production of large volumes of diluted urine. Because diabetes insipidus is different from the more commonly known condition, diabetes mellitus, which involves issues with insulin and blood sugar regulation, and often was confused by medical professionals, a name change was proposed in 2022 by group of representatives from national and international endocrine, nephrology and pediatric societies.

DI occurs when there is a deficiency in the production of vasopressin (also called antidiuretic hormone or ADH), which is produced by the hypothalamus and released by the pituitary gland. Vasopressin helps regulate the body’s water balance by controlling the reabsorption of water in the kidneys. In DI, the brain doesn’t produce enough vasopressin leading to the inability to concentrate urine and excessive fluid loss. It can result from various factors such as head injuries, tumors affecting the hypothalamus or pituitary gland, infections, or other underlying conditions.

Currently there is a movement to rename central diabetes insipidus to arginine vasopressin deficiency (AVP-D) to avoid confusion with diabetes mellitus.

Symptoms of Diabetes Insipidus

The hallmark symptoms of DI include extreme thirst (polydipsia) and excessive urine production (polyuria). Individuals with diabetes insipidus may need to urinate frequently and may produce large amounts of pale, diluted urine, leading to dehydration if water intake doesn’t match fluid loss. To diagnose DI, various tests such as water deprivation tests, blood tests, and urine concentration tests are performed to determine the underlying cause and type of the condition.

Patients with hypothalamic damage may also experience adipsia. Adipsia is a medical condition characterized by the absence or significant reduction of the sensation of thirst. This condition can result in a person not feeling the natural urge to drink fluids, even when their body is in need of hydration. Adipsia can lead to severe dehydration and electrolyte imbalances if not properly managed.

It’s important to seek medical attention for proper diagnosis and management, as this condition can lead to serious dehydration and electrolyte imbalances if not treated appropriately.

Treatment

Treatment of diabetes insipidus usually involves addressing the underlying cause if possible. For central diabetes insipidus, synthetic vasopressin or desmopressin (a synthetic form of vasopressin) can be prescribed to help regulate water balance.

For patients with adipsia or are newly diagnosed, routine blood sodium testing may be required to ensure sodium levels are within a safe range and to reach a proper dosage of desmopressin.

Desmopressin is available in various forms, including:

  1. Oral Tablets: Desmopressin is available in tablet form, which can be taken orally. This form is often used to treat conditions like diabetes insipidus and nocturnal enuresis. The tablets are usually taken with water.
  2. Nasal Spray: This is one of the more common forms of desmopressin. The medication is administered by spraying it into the nostrils. It is often used to treat conditions such as diabetes insipidus and nocturnal enuresis. The nasal spray is absorbed through the nasal mucosa and enters the bloodstream.
  3. Nasal Solution: Similar to the nasal spray, the nasal solution is also administered through the nostrils. It’s usually used for similar indications as the nasal spray, and the liquid form allows for controlled dosing.
  4. Injectable Form: Desmopressin can also be administered via injection, either intramuscularly or subcutaneously. Injectable forms are often used in cases where rapid onset of action is needed, such as in emergency situations or medical procedures.

The choice of the appropriate form of desmopressin depends on the patient’s condition, the severity of the condition, the desired speed of onset, and individual preferences. The prescribing healthcare professional will determine the most suitable form and dosage based on the patient’s medical history and needs. It’s important for patients to follow their healthcare provider’s instructions carefully and to monitor their response to the medication.


Breaking Through the Issues of Diabetes Insipidus Management

Joseph A. Majzoub, M.D. Vice Chair for Research, Department of Pediatrics Emeritus Chief, Division of Endocrinology, Boston Children’s Hospital shares many insights on Diabetes Insipidus and answers many questions about the various issues around medication management and dosing, urine output and breakthroughs, adipsia, sodium intake and diet.

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