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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The multiple faces of Langerhans cell histiocytosis in childhood: A gentle reminder.

Mol Clin Oncol. 2018 Mar;8(3):489-492

Authors: Papadopoulou M, Panagopoulou P, Papadopoulou A, Hatzipantelis E, Efstratiou I, Galli-Tsinopoulou A, Papadopoulou-Alataki E

Abstract
Langerhans cell histiocytosis (LCH) is a rare hematologic disorder that results from the clonal multiplication and accumulation of immature dendritic Langerhans cells. Its reported incidence rate varies, but is considered to be 2.6-8.9 per million children who are <15 years of age each year. It may affect any system or organ. The present study reported 4 pediatric LCH cases in order to highlight the heterogeneity of the initial presentation, and the pitfalls that may mislead clinicians and delay diagnosis. The clinical features, as well as the pathognomonic imaging, pathology findings and treatment options were presented. LCH may be rare, but it should always be included in the differential diagnosis of persistent eczema, unexplained skin lesions, diabetes insipidus and persistent bone pain, among others. While the debate on pathogenesis and treatment is ongoing, high index of suspicion among pediatricians, pediatric oncologists and other specialists (pathologists, dermatologists, orthopaedic surgeons, general practitioners or family physicians) is essential for early diagnosis, and optimal outcome.

PMID: 29468064 [PubMed]

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Diabetes mellitus and optic atrophy: study of the Wolfram syndrome

Gac Med Mex. 2017;153(4):466-472

Authors: Rivas-Gómez B, Reza-Albarrán AA

Abstract
Background: Wolfram syndrome (WS), also known by the acronym DIDMOAD, is a rare and progresive hereditary disease of autosomal recessive inheritance which minimum ascertainment diagnostic criteria are the occurrence together of diabetes mellitus and optic atrophy before 15 years of age.
Objective: To describe the clinical, biochemical and molecular profile of WS in a tertiary care hospital in Mexico.
Materials and Methods: We reviewed patients records who fulfill the minimum ascertainment diagnostic criteria of WS presenting between January 1987 and May 2015 in a tertiary care hospital in Mexico.
Results: Five patients fulfill the inclusion criteria (three male and two female). Diabetes mellitus was the first manifestation of the syndrome in all of them, with a mean age at diagnosis of 5.8 ± 2.71 years, while the WS diagnosis was established at a mean age of 15.8 ± 8.37 years. All the patients had optic atrophy and two of them presented with the complete DIDMOAD spectrum. We found new associations with autoimmune hepatitis and testicular cancer.
Conclusions: This study shows the variability of clinical presentation of WS, as well as two new associations.

PMID: 28991271 [PubMed - indexed for MEDLINE]

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