Frequently Asked Questions

Diabetes Insipidus (DI)

The questions below are regarding Diabetes Insipidus (DI) specifically. Please click on a question to view the answer.

  1. What is the difference in diabetes insipidus and diabetes mellitus?
  2. What are the chances my child will develop diabetes insipidus?
  3. Why is it important that the testing for DI be done in a clinic/hospital?
  4. How is a water deprivation test done?
  5. Can diabetes insipidus be reversed?
  6. Can diabetes insipidus occur before the diagnosis of LCH?
  7. Can diabetes insipidus due to LCH occur when there is no known involvement anywhere else?
  8. Can diabetes insipidus in LCH be prevented?

Histiocytic Disorders and Orphan Diseases

The questions below are regarding Histiocytic Disorders and Orphan Diseases in general. Please click on a question to view the answer.

  1. What are histiocytic disorders, and how are they classified?
  2. Why are all of these diseases with different names considered to be related to each other?
  3. Where can I find reliable information about histiocytosis?
  4. How can I explain histiocytosis to family and friends?
  5. What is an orphan disease?
  6. How many orphan diseases are there?
  7. Where can I learn more about rare diseases in general?

Diabetes Insipidus (DI)

  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.

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  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.

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Histiocytic Disorders and Orphan Diseases

 

  1. What are histiocytic disorders, and how are they classified?
    Histiocytic disorders are a diverse group of diseases caused by over-production of white blood cells known as histiocytes, which can lead to organ damage and tumor formation. They include a wide variety of conditions that can affect both children and adults.

    The disorders are classified into three groups based on the types of histiocyte cells involved.
    • The first group is called a dendritic cell disorder, and the most common disease in this group is Langerhans cell histiocytosis. Also included in this group are more rare diseases, juvenile xanthogranuloma (JXG) and Erdheim Chester.
    • The second group is called a macrophage cell disorder, and includes primarily hemophagocytic lymphohistiocytosis (HLH) and Rosai-Dorfman.
    • The third group is called malignant histiocytosis and includes certain kinds of leukemia and tumors.
  2. Why are all of these diseases with different names considered to be related to each other?
    All of the diseases are caused by the over-production of white blood cells called histiocytes. Their different classifications depend on the type of histiocyte involved

  3. Where can I find reliable information about histiocytosis?
    The Histiocytosis Association’s online community provides a number of informational documents and articles, as well as links to medical articles about the histiocytic disorders. While the Internet does provide a significant volume of information about histiocytic disorders, some of this information is not accurate. It is important to look for documents that are current, are free of grammatical and spelling errors, appear to be objective, are free of advertisements, and clearly state their sources.

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  4. How can I explain histiocytosis to family and friends?
    Histiocytosis is a rare disease that is caused by the over-production of a type of white cell that can lead to organ damage and the formation of tumors. The Histiocytosis Association’s Disease Fact Sheets are also a great way to help explain these complicated diseases to family and friends.

  5. What is an orphan disease?
    According to the Rare Disease Act of 2002, an orphan disease, also known as a rare disease, affects less than 200,000 persons in the U.S., or less than 1 in 1500 people. The criteria may vary in other countries. For example in Europe, an orphan disease is defined to occur in less than 1 in 2000.

  6. How many orphan diseases are there?
    According to the National Institutes of Health there are approximately 6800 such diseases. Combined, they affect nearly 30 million Americans.

  7. Where can I learn more about rare diseases in general?

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