Diabetes Insipidus Causes, Symptoms, and Treatment .

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Diabetes insipidus facts*

*Diabetes insipidus facts Medically Edited by: Melissa Conrad Stöppler, MD

  • Diabetes insipidus is not related to diabetes mellitus (type 1 and type 2 diabetes).
  • Diabetes insipidus is caused by problems related to the antidiuretic hormone (ADH) or its receptor and causes frequent urination.
  • There are four types of diabetes insipidus; 1) central diabetes insipidus, 2) nephrogenic diabetes insipidus, 3) dipsogenic diabetes insipidus, and 4) gestational diabetes insipidus.
  • The most common symptom of diabetes insipidus is frequent urination.
  • The diagnosis for diabetes insipidus is based on a series of tests (for example, urinalysis and fluid deprivation test).
  • The treatment for diabetes insipidus depends on the type of diabetes insipidus.
  • Diabetes can lead to chronic kidney disease.
  • Diabetes is the most common cause of kidney failure in the US.

Diabetes Insipidus Definition

Excessive urination and extreme thirst as a result of inadequate output of the pituitary hormone ADH (antidiuretic hormone, also called vasopressin) or the lack of the normal response by the kidney to ADH.


There are two types of diabetes insipidus — central and nephrogenic. Central diabetes insipidus is a lack of ADH production and is due to damage to the pituitary gland or hypothalamus where ADH is produced. Nephrogenic diabetes insipidus is lack of response of the kidney to the fluid-conserving action of ADH. Nephrogenic diabetes insipidus can be due to diseases of the kidney (such as polycystic kidney disease), certain drugs (such as lithium), and can also occur an inherited disorder.


In both central and nephrogenic diabetes insipidus, patients excrete extraordinarily large volumes of very dilute urine. They feel thirsty and drink very large amounts of water to compensate for the water they lose in the urine. The main danger with diabetes insipidus comes when fluid intake does not keep pace with urine output, resulting in dehydration and high blood sodium.

What is diabetes insipidus?

Nephrogenic diabetes insipidus is a disorder of water balance. The body normally balances fluid intake with the excretion of fluid in urine. However, people with nephrogenic diabetes insipidus produce too much urine (polyuria), which causes them to be excessively thirsty (polydipsia). Affected individuals can quickly become dehydrated if they do not drink enough water, especially in hot weather or when they are sick.

Nephrogenic diabetes insipidus can be either acquired or hereditary. The acquired form is brought on by certain drugs and chronic diseases and can occur at any time during life. The hereditary form is caused by genetic mutations, and its signs and symptoms usually become apparent within the first few months of life.

What are the symptoms of diabetes insipidus in infants?

Infants with hereditary nephrogenic diabetes insipidus may eat poorly and fail to gain weight and grow at the expected rate (failure to thrive). They may also be irritable and experience fevers, diarrhea, and vomiting. Recurrent episodes of dehydration can lead to slow growth and delayed development. If the condition is not well-managed, over time it can damage the bladder and kidneys leading to pain, infections, and kidney failure. With appropriate treatment, affected individuals usually have few complications and a normal lifespan.

Are diabetes insipidus and diabetes mellitus the same condition?

Nephrogenic diabetes insipidus should not be confused with diabetes mellitus, which is much more common. Diabetes mellitus is characterized by high blood sugar levels resulting from a shortage of the hormone insulin or an insensitivity to this hormone. Although nephrogenic diabetes insipidus and diabetes mellitus have some features in common, they are separate disorders with different causes.



QUESTION


Diabetes is defined best as…
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Is diabetes insipidus inherited?

When nephrogenic diabetes insipidus results from mutations in the AVPR2 gene, the condition has an X-linked recessive pattern of inheritance. The AVPR2gene is located on the X chromosome, which is one of the two sex chromosomes. In males (who have only one X chromosome), one altered copy of the gene in each cell is sufficient to cause the condition. In females (who have two X chromosomes), a mutation usually has to occur in both copies of the gene to cause the disorder. However, some females who carry a single mutated copy of the AVPR2 gene have features of nephrogenic diabetes insipidus, including polyuria and polydipsia. A characteristic of X-linked inheritance is that fathers cannot pass X-linked traits to their sons.

When nephrogenic diabetes insipidus is caused by mutations in the AQP2 gene, it can have either an autosomal recessive or, less commonly, an autosomal dominant pattern of inheritance. In autosomal recessive inheritance, both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition. In autosomal dominant inheritance, one mutated copy of the AQP2 gene in each cell is sufficient to cause the disorder.
 

What are other names for diabetes insipdus?

ADH-resistant diabetes insipidus, congenital nephrogenic diabetes insipidus, diabetes insipidus renalis, diabetes insipidus, nephrogenic, NDI, vasopressin-resistant diabetes insipidus.
 

For more information about diabetes insipidus

The Diabetes Insipidus Foundation, Inc.
Patient Support and Information
Mary Evans-Lee
3742 Woodland Drive
Columbus, GA 31907
Phone: 706-323-7576
Email: [email protected]
Internet: www.diabetesinsipidus.org

The Diabetes Insipidus and Related Disorders Network
535 Echo Court
Saline, MI 48176-1270
Email: [email protected]
Internet: web.archive.org/web/20021201133912/members.aol.com/ruudh/dipage1.htm?mtbrand=AOL_US

National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
P.O. Box 1968
Danbury, CT 06813-1968
Phone: 1-800-999-6673 (voicemail) or 203-744-0100
Email: [email protected]
Internet: www.rarediseases.org

Nephrogenic Diabetes Insipidus Foundation
Main Street
P.O. Box 1390
Eastsound, WA 98245
Phone: 1-888-376-6343
Fax: 1-888-376-6356
Email: [email protected]
Internet: www.ndif.org




SLIDESHOW


Type 2 Diabetes Diagnosis, Treatment, Medication
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Medically Reviewed on 8/28/2019

References


SOURCE: National Kidney and Urologic Diseases Information
Clearinghouse, National Institutes of Health. Diabetes Insipidus. Aug 20, 2019.





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