Diabetes Insipidus
What is Diabetes Insipidus?
Diabetes insipidus (DI) is a rare disorder that can occur as a consequence of histiocytosis involving the pituitary gland. It should not be confused with the more common diabetes mellitus, also known as sugar diabetes, which results from too much sugar in the blood. Although both disorders have similar symptoms, in every other way including the cause and treatment, they are completely unrelated diseases. The rate of occurrence for DI is not known, because there has been no organized method to count the number of patients.


Diabetes insipidus is a result of damage to the pituitary gland, a small gland at the base of the brain which stores and releases a hormone called ADH (antidiuretic hormone), also known as vasopressin. This hormone normally causes the kidney to control the amount of water released as urine from the body. When the pituitary is damaged, the kidneys lose too much water (increased urination), which then leads to increased thirst.

The connection between histiocytosis and diabetes insipidus was first reported in the late 1800s. Since then, DI has been recognized as a characteristic feature of LCH. It is known to also occur in other histiocytic disorders, such as Rosai Dorfman and JXG.

It is believed that approximately between 5% and 50% of LCH patients develop DI depending on the extent of disease. The risk of developing DI in patients with multisystem LCH is 4 to 6 times more than those with single-system disease. Patients with skull, facial, and/or eye bone lesions are at much higher risk of developing DI. This risk is increased further if LCH remains active for a longer period or if it recurs.

Diabetes insipidus is recognized by a great increase in the amount of urine passed (often several gallons per day) and an increased thirst. Any patient with known LCH with an increase in drinking habits or passing large amounts of urine should be tested for DI.

Diabetes insipidus is diagnosed with a water deprivation test, which measures changes in body weight, blood values, urine output, and urine composition when fluids are withheld over a several-hour period. It is very important that this test be supervised by a knowledgeable physician in a medical setting. An x-ray test called an MRI scan is sometimes performed to see if there is change in the brain and pituitary area, but this test alone cannot diagnose DI.

Diabetes insipidus is usually a permanent, lifelong condition and cannot be cured. However, the symptoms of constant thirst and urination can be well controlled with treatment with DDAVP, a synthetic kind of vasopressin, and normal, symptom-free quality of life can be restored.

Please be advised that all the information you read here is not a replacement for the advice you will get from your consultant and their team.

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1. What is the difference in diabetes insipidus and diabetes mellitus?
Two different types of hormones are involved: diabetes insipidus due to impaired production by the pituitary gland of a hormone called antidiuretic hormone and may occur as a consequence of histiocytosis. Diabetes mellitus, on the other hand, results from too much sugar in the blood, due to impaired insulin production by the pancreas. Although both disorders have similar symptoms of increased thirst and urination, in every other way including the cause and treatment, they are completely unrelated diseases.

2. What are the chances my child will develop diabetes insipidus?
DI occurs in as many as 25% of all patients and as many as 50% of patients with multisystem LCH.

3. Why is it important that the testing for DI be done in a clinic/hospital?
The water-deprivation test is a complicated procedure that requires highly trained medical professionals to perform specialized measurements. The body’s water balance must be carefully monitored during the procedure to prevent rapid and dangerous dehydration.

4. How is a water deprivation test done?
This test includes timed measurements (some done every hour and others done every other hour) of blood pressure, pulse, weight, urine, and blood. Fluid is withheld during testing. The test may take up to 8 hours to complete, but it may be stopped sooner, depending on lab results. Further information and instructions will be provided by your physician.

5. Can diabetes insipidus be reversed?
Once DI has been diagnosed, the chance of reversal is uncommon. However, it has been reported in some cases where treatment was started within a few days of symptom onset.

6. Can diabetes insipidus occur before the diagnosis of LCH?
DI can be the first presenting symptom, although one-half of these patients develop LCH lesions within 1 year after the onset of diabetes insipidus.

7. Can diabetes insipidus due to LCH occur when there is no known involvement anywhere else?
Yes. It is believed that this occurs in less than 10% of patients. The diagnosis is made from biopsy of the tumor in the pituitary stalk.

8. Can diabetes insipidus in LCH be prevented?
There is evidence that a rapid start of chemotherapy after onset of multisystem LCH may prevent DI.

Please be advised that all the information you read here is not a replacement for the advice you will get from your consultant and their team.

Help ensure that we can continue to bring you this vital informational material, make a donation today

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pubmed: diabetes insipidus

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Related Articles

Daily Sodium Monitoring and Fluid Intake Protocol: Preventing Recurrent Hospitalization in Adipsic Diabetes Insipidus.

J Endocr Soc. 2019 May 01;3(5):882-886

Authors: Pabich S, Flynn M, Pelley E

Management of diabetes insipidus (DI) is usually facilitated by an intact thirst mechanism prompting water ingestion in times of rising osmolality. Maintenance of eunatremia can be quite difficult in patients with DI and adipsia because of the absence of this homeostatic mechanism. Few published protocols for management of these complex cases exist. We report a case of a 16-year-old girl who had a diagnosis of craniopharyngioma with preoperative hypopituitarism and central DI. She underwent transsphenoidal resection in 2013 and additionally developed postoperative cognitive impairment and hypothalamic dysfunction, including adipsia. She subsequently experienced widely dysregulated sodium levels, necessitating inpatient care ∼30% of days in 2014 and 2015. We created a protocol for this patient that uses a fixed daily dose of subcutaneous DDAVP combined with daily modulation of fluid intake based on daily serum sodium measurement. The protocol provides guidance for the day's fluid intake based on both the current sodium result and the rate of change from the previous day. Since the adoption of the protocol in June 2016, the patient has had a dramatic reduction in hospitalizations. Use of a protocol for providing recommendations for fluid intake based on the sodium level and rate of change may help to maintain normal sodium levels in such patients, decreasing hospitalization and improving quality of life.

PMID: 31008421 [PubMed]

Related Articles

Lithium treatment, nephrogenic diabetes insipidus and the risk of hypernatraemia: a retrospective cohort study.

Ther Adv Psychopharmacol. 2019;9:2045125319836563

Authors: Ott M, Forssén B, Werneke U

Background: Hypernatraemia is a serious condition that can potentially become life threatening. It is known that lithium is associated with polyuria and nephrogenic diabetes insipidus, risk factors for hypernatraemia. In this study, we tested the hypothesis that lithium treatment was a risk factor for hypernatraemia.
Methods: We performed a retrospective cohort study in the Swedish region of Norrbotten into the effects and potential adverse effects of lithium treatment and other mood stabilizers (LiSIE). For this particular study, we included all patients who had experienced at least one episode with a sodium concentration ⩾150 mmol/L between 1997 and 2013. Medical records were reviewed regarding past or current lithium exposure, diabetes insipidus and other potential risk factors for hypernatraemia.
Results: Of 2463 patients included, 185 (7.5%) had experienced 204 episodes of hypernatraemia within the 17-year review period. In patients 65 years or older, infections dominated as the cause with 51%. In patients younger than 65 years, intoxications, particularly with alcohol, dominated as the cause with 35%. In the whole sample, dehydration accounted for 12% of episodes, 25% of which in the context of suspected or confirmed nephrogenic diabetes insipidus. Of all episodes, 25% resulted in death, with infection being the most common cause of death in 62% of cases.
Conclusions: In our sample, infections and harmful use of substances including alcohol were the most common causes of hypernatraemia. Both current and past use of lithium also led to episodes of hypernatraemia, when associated with nephrogenic diabetes insipidus. Clinicians should remain vigilant, have a low threshold for checking sodium concentrations and consider even risk factors for hypernatraemia beyond lithium.

PMID: 31007893 [PubMed]

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