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Central diabetes insipidus


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Other Names:
Diabetes insipidus neurogenic; Neurohypophyseal diabetes insipidus; Pituitary diabetes insipidus; Diabetes insipidus neurogenic; Neurohypophyseal diabetes insipidus; Pituitary diabetes insipidus; Diabetes insipidus neurohypophyseal; Diabetes insipidus cranial type; Neurogenic diabetes insipidus See More
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Central diabetes insipidus (DI) is a form of DI that occurs when the body has lower than normal levels of antidiuretic hormone (vasopressin), which is characterized by frequent urination.[1][2] Diabetes insipidus is subdivided into central and nephrogenic DI. Two other forms are gestational DI and primary polydipsia (dipsogenic DI). Central DI results from damage to the pituitary gland, which disrupts the normal storage and release of antidiuretic hormone (ADH). When this hormone reaches the kidneys, it directs them to make less urine. The major symptoms of central diabetes insipidus (DI) include urinating too much (polyuria), getting up at night to urinate (nocturia), and drinking too much liquids (polydipsia).  Damage to the pituitary gland can be caused by different diseases as well as by head injuries, neurosurgery, or genetic disorders.[2] The three main options for the treatment of central DI include a synthetic hormone called desmopressin, which can be taken by as an injection, a nasal spray, or a pill; other drugs, such as chlorpropamide, carbamazepine, thiazide diuretics, and nonsteroidal anti-inflammatory drugs; and a low-solute (mostly low-sodium, low-protein) diet.[3]
Last updated: 12/27/2016

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Anorexia 0002039
Dehydration 0001944
Diabetes insipidus 0000873
Failure to thrive
Faltering weight
Weight faltering
[ more ]
0001508
Nocturia 0000017
Polydipsia
Extreme thirst
0001959
Weight loss 0001824
30%-79% of people have these symptoms
Anxiety
Excessive, persistent worry and fear
0000739
Depressivity
Depression
0000716
Excessive daytime somnolence
More than typical sleepiness during day
0001262
Fever 0001945
Headache
Headaches
0002315
Lethargy 0001254
5%-29% of people have these symptoms
Diarrhea
Watery stool
0002014
Hyponatremia
Low blood sodium levels
0002902
Nausea and vomiting 0002017
Seizure 0001250
Percent of people who have these symptoms is not available through HPO
Abnormality of metabolism/homeostasis
Laboratory abnormality
Metabolism abnormality
[ more ]
0001939
Autosomal dominant inheritance 0000006
Gliosis 0002171
Hypertelorism
Wide-set eyes
Widely spaced eyes
[ more ]
0000316
Long philtrum 0000343
Osteopenia 0000938
Short nose
Decreased length of nose
Shortened nose
[ more ]
0003196
Wide nose
Broad nose
Increased breadth of nose
Increased nasal breadth
Increased nasal width
Increased width of nose
[ more ]
0000445
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Last updated: 7/1/2020

The resources below provide information about treatment options for this condition. If you have questions about which treatment is right for you, talk to your healthcare professional.

Management Guidelines

  • Orphanet Emergency Guidelines is an article which is expert-authored and peer-reviewed that is intended to guide health care professionals in emergency situations involving this condition.  

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources


Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
The main differential diagnosis is NDI as the disorders share the same manifestations. Recently, the dosage of aquaporin 2 (AQP2) has been used in the differential diagnosis of CDI as the failure to increase AQP2 excretion after desmopressin administration indicates a nephrogenic form of diabetes insipidus. Wolfram syndrome (see this term) is another differential diagnosis.
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Central diabetes insipidus. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Central diabetes insipidus. Click on the link to view a sample search on this topic.

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  1. Diabetes insipidus - central. MedlinePlus. 2015; https://medlineplus.gov/ency/article/000460.htm.
  2. Diabetes Insipidus. National Kidney and Urologic Diseases Information Clearinghouse. 2015; http://kidney.niddk.nih.gov/kudiseases/pubs/insipidus/.
  3. Bichet DG. Treatment of central diabetes insipidus. UpToDate. 216; http://www.uptodate.com/contents/treatment-of-central-diabetes-insipidus?source=search_result&search=central+diabetes+insipidus&selectedTitle=1~54.