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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Perianal Langerhans cell histiocytosis: a rare presentation in an adult male.

Autops Case Rep. 2017 Jul-Sep;7(3):38-43

Authors: Abdou AG, MaherTaie D

Langerhans cell histiocytosis (LCH) is a rare disease characterized by a proliferation of cells that show immunophenotypic and ultrastructural similarities with antigen-presenting Langerhans cells of mucosal sites and skin. LCH in adults is rare, and there are still many undiagnosed/misdiagnosed patients. We describe LCH involvement of the perianal region of a 33-year-old male with a previous history of diabetes insipidus. The differential diagnosis and all the reported cases of LCH of the perianal skin involvement since its description in 1984 till 2016 are discussed. LCH should be considered in the differential diagnosis of perianal ulceration, especially in young patients where topical drug treatment has failed. The history of previous central diabetes insipidus of unknown etiology demands imaging studies in order to rule out central involvement of the disease.

PMID: 29043209 [PubMed]

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Central Diabetes Insipidus Linked to Rathke's Cleft Cyst, Polyuria in a 17-year-old Girl.

Electrolyte Blood Press. 2017 Sep;15(1):23-25

Authors: Kim HY, Lee SJ, Bae EH, Ma SK, Kim SW

A 17-year-old girl presented with polyuria (7 L/day) and polydipsia for one year. Initial urine osmolality was 113mOsm/kg H2O. Following 6 h of fluid restriction, serum plasma osmolality reached 300mOsm/kg H2O, whereas urine osmolality was 108mOsm/kg H2O. Urine osmolality was increased by 427% from 108 to 557mOsm/kg after vasopressin challenge. The patient was diagnosed with central diabetes insipidus, possibly derived from the atypical occupation of a Rathke's cleft cyst at the pituitary stalk following magnetic resonance imaging with enhancement. She was discharged with desmopressin nasal spray (10 µg); urine output was maintained at 2-3 L/day, and urine osmolality was >300 mOsm/kg. Additional pituitary image studies and evaluation of hypopituitarism should be included in the differential diagnosis of patients with central diabetes insipidus.

PMID: 29042904 [PubMed]

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The supraorbital eyebrow approach for removal of craniopharyngioma in children: a case series.

Childs Nerv Syst. 2017 Oct 16;:

Authors: de Oliveira RS, Viana DC, Augusto LP, Santos MV, Machado HR

OBJECTIVE: Craniopharyngiomas can be a surgical challenge for the pediatric neurosurgeon. Ideally, total removal must be achieved. However, the need to reduce surgical morbidity and preserve quality of life has led to a number of neurosurgical approaches in order to attain this goal. The aim of this article is to present an alternative surgical approach to these lesions and to provide the rationale for this technique.
MATERIAL AND METHODS: Medical charts and operative records of eight pediatric patients harboring craniopharyngiomas who underwent surgical treatment using a supraorbital eyebrow approach (SOA) were reviewed from 2014 to 2016. Only patients younger than 18 years with a minimum follow-up of 12 months were included in this study. Using pre-operative magnetic resonance (MRI) scans, tumors were classified according to their degree of hypothalamic involvement. The surgical technique is also described in detail.
RESULTS: The study group included six males and two females with a mean age of 10 years (range, 2-16 years). The SOA was used successfully in elective surgery of eight craniopharyngiomas. The hypothalamus was displaced by the tumor in three patients and severely involved in five patients. Subtotal resection was undertaken in six patients, whereas gross-total resection was achieved in two. Endoscopic assistance was used after standard microscopic visualization in two out of eight cases. Cosmetic outcomes were excellent, and the complication rate related to the surgical procedure was quite low, apart from diabetes insipidus (which occurred in three out of the eight patients). In one patient, a large subdural collection needed surgery for evacuation. Mean follow-up was 23.2 months (range, 12-36 months). Additionally, no CSF leak or wound infection was identified.
CONCLUSIONS: The supraorbital eyebrow approach is an alternative route to operate on craniopharyngiomas in properly selected cases of all pediatric age ranges, from infants to teenagers. There is sufficient working space for the endoscope and all instruments, allowing for endoscopic assistance and bimanual surgical technique. Cosmetic results are excellent, and complications related to the approach are minimal.

PMID: 29038894 [PubMed - as supplied by publisher]

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Hum Mol Genet. 2017 May 01;26(9):1599-1611

Authors: Rouzier C, Moore D, Delorme C, Lacas-Gervais S, Ait-El-Mkadem S, Fragaki K, Burté F, Serre V, Bannwarth S, Chaussenot A, Catala M, Yu-Wai-Man P, Paquis-Flucklinger V

Wolfram syndrome (WS) is a progressive neurodegenerative disease characterized by early-onset optic atrophy and diabetes mellitus, which can be associated with more extensive central nervous system and endocrine complications. The majority of patients harbour pathogenic WFS1 mutations, but recessive mutations in a second gene, CISD2, have been described in a small number of families with Wolfram syndrome type 2 (WFS2). The defining diagnostic criteria for WFS2 also consist of optic atrophy and diabetes mellitus, but unlike WFS1, this phenotypic subgroup has been associated with peptic ulcer disease and an increased bleeding tendency. Here, we report on a novel homozygous CISD2 mutation (c.215A > G; p.Asn72Ser) in a Moroccan patient with an overlapping phenotype suggesting that Wolfram syndrome type 1 and type 2 form a continuous clinical spectrum with genetic heterogeneity. The present study provides strong evidence that this particular CISD2 mutation disturbs cellular Ca2+ homeostasis with enhanced Ca2+ flux from the ER to mitochondria and cytosolic Ca2+ abnormalities in patient-derived fibroblasts. This Ca2+ dysregulation was associated with increased ER-mitochondria contact, a swollen ER lumen and a hyperfused mitochondrial network in the absence of overt ER stress. Although there was no marked alteration in mitochondrial bioenergetics under basal conditions, culture of patient-derived fibroblasts in glucose-free galactose medium revealed a respiratory chain defect in complexes I and II, and a trend towards decreased ATP levels. Our results provide important novel insight into the potential disease mechanisms underlying the neurodegenerative consequences of CISD2 mutations and the subsequent development of multisystemic disease.

PMID: 28335035 [PubMed - indexed for MEDLINE]

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