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

NCBI: db=pubmed; Term=diabetes insipidus

Related Articles

Evaluation of pharmacochaperone-mediated rescue of mutant V2 receptor proteins.

Eur J Pharmacol. 2019 Nov 15;:172803

Authors: Tuncdemir BE, Mergen H, Ozer ES

Abstract
Nephrogenic Diabetes Insipidus is a rare disorder which is characterized by severe water imbalance in the body. The disease can be acquired or inherited. AVPR2 (arginine vasopressin type 2 receptor) mutations are responsible for genetical type of the disorder. Mutations in the AVPR2 gene may cause loss-of-function due to conformational defects. According to the mutation type, the three-dimensional structure of AVPR2 may be affected even if it is functional and therefore it may not reach the plasma membrane where it is functional. Consequently, it is generally trapped in the Endoplasmic reticulum or Golgi apparatus, which are the quality control systems of the cell. Pharmacological chaperones have been used to retrieve these mutant AVPR2s from these quality control systems of the cell and take them to the plasma membrane for therapeutic purposes. In this study, in order to analyze the effects of two pharmacological chaperones, SR121463B and SR49059, we performed total ELISA and surface ELISA studies and cAMP accumulation assays on mutant receptors (G12E, R68W, V88M, ΔR67_G69/G107W, R106C, V162A and T273M). We observed that pharmacological chaperones may act differently on mutated AVPR2s. Cell surface expression of the mutant receptors and cAMP accumulation response, after stimulation with AVP, were mostly improved by these pharmacological chaperones. We believe that, this study presents important results with respect to the process of the variable type of mutated proteins in the cell and may help in developing a process of new types of chaperones.

PMID: 31738937 [PubMed - as supplied by publisher]

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