Diabetes insipidus is a rare disease characterized by excessive production of dilute urine and excessive thirst. There are four main types: central diabetes insipidus caused by problems with the pituitary gland; nephrogenic diabetes insipidus caused when the kidneys do not respond to vasopressin; dipsogenic diabetes insipidus caused by damage to the body's thirst regulation system; and gestational diabetes insipidus which occurs in some pregnant women when the placenta destroys vasopressin. Gestational diabetes insipidus is usually treated with desmopressin nasal spray to manage urine control, while dipsogenic diabetes insipidus has no specific treatment.
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Rare DIabetes Insipidus Explained
1. Diabetes insipidus is "Chronic
excretion of very large amounts of pale
urine of low specific gravity, causing
dehydration and extreme thirst;
ordinarily results from inadequate
output of pituitary antidiuretic hormone;
the urine abnormalities may be
mimicked as a result of excessive fluid
intake, as in psychogenic polydipsia.
Two types exist: central and
nephrogenic. Autosomal dominant
[MIM*125700, *125800, *192340], X-
linked [MIM*304800 and *304900], and
even autosomal recessive forms
[MIM*222000] have been described."
Diabetes insipidus (DI) is a rare disease
that causes frequent urination. The large
volume of urine is diluted, mostly water.
To make up for lost water, a person with
2. diabetes insipidus may feel the need to
drink large amounts of water .Milder
forms of diabetes insipidus can be
managed by drinking enough water,
usually between 2 and 2.5 liters a day.
Diabetes insipidus severe enough to
endanger a person's health is rare.
approximately 1 year every 25,000
people are suffered from Diabetes
Insipidus . DI caused by a lack of ADH is
called central diabetes insipidus. When DI is
caused by a failure of the kidneys to respond
to ADH, the condition is called nephrogenic
diabetes insipidus. ADH is a hormone
produced in a region of the brain called the
hypothalamus. It is then stored and released
from the pituitary gland, a small gland at the
base of the brain. The amount of water
conserved is controlled by antidiuretic
hormone (ADH), also called vasopressin.
3. Symptoms Diabetes insipidus
Cool hands and feet
Delayed growth (failure to thrive)
Diapers (UK: nappies) which are
excessively wet
Diarrhea
Dry skin
Fever
Inconsolable crying
Unexplained fussiness
Vomiting
Weight loss (failure to thrive)
Two types of Diabetes Insipidus
1) Central Diabetes Insipidus
2)Nephrogenic Diabetes Insipidus
Central Diabetes Insipidus
4. DI caused by a lack of ADH is called central
diabetes insipidus
Central diabetes insipidus (cranial
diabetes insipidus) - usually caused by
damage to the hypothalamus or the
pituitary gland, often as a result of
surgery, a tumor, meningitis,
inflammation or a head injury, which
disrupts ADH production, storage and
release. Central diabetes insipidus that
is caused by a head injury, or surgery to
your pituitary gland, is sometimes only a
temporary condition that lasts for just a
couple of weeks.
Causes
In central diabetes insipidus, the antidi-
uretic hormone vasopressin is either
5. missing or present at a low level. This low
level or lack of vasopressin is due to a
malfunction in the part of your brain, the
posterior pituitary gland, which releases the
hormone into your bloodstream. Injury to
the head, tumors, neurosurgical operations,
infections, or bleeding can affect your
brain's ability to release the right amount of
vasopressin.
BODY PART AFFECTED
Central diabetes insipidus can be caused by
damage to the hypothalamus or pituitary
gland as a result of:
Head injury
Infection
6. Loss of blood supply to the gland
Surgery
Tumor
What are the symptoms?
■ excessive urination
■ excessive thirst (polydipsia)
Patients with central diabetes
insipidus are often extremely tired
because they cannot get enough
sleep uninterrupted by the need to
urinate. Their urine is very clear and
odorless. These symptoms can
appear at any time. Because they
lose so much water from urination,
they also feel very thirsty. If this
disorder is untreated, they could
become seriously dehydrated, and
7. their bodies will not have enough
water to function properly
Therapy
central diabetes insipidus, you will
sniff a drug called DDAVP
(Desmopressin), a derivative of
vasopressin. You will be shown the
right way to use this drug by your
physician, nurse, or pharmacist.
Nephrogenic Diabetes Insipidus
When DI is caused by a failure of the
kidneys to respond to ADH, the condition is
called nephrogenic diabetes insipidus.
Nephrogenic diabetes insipidus may be
caused by kidney diseases that make the
kidneys unable to respond to vasopressin.
8. While there is enough vasopressin in the
body (unlike in central diabetes insipidus),
the kidneys cannot respond to the
hormone's signal to reabsorb water. The
disease may be acquired or inherited by
male children.
Symptoms
The symptoms of nephrogenic diabetes
insipidus are similar to central diabetes
insipidus; that is, excessive urination
(polyuria) followed by excessive thirst
(polydipsia)
Treatment
The first step in treating this disease is
correct diagnosis. In addition to the med-
ications available, balancing your water or
fluid intake with your urine output is also
9. part of treatment. If this disorder is
untreated, you could become seriously
dehydrated, and your body will not have
enough water to function.
Theary
nephrogenic diabetes insipidus, water pills
(thiazide diuretics) may be prescribed by
your doctor. You may be confused as to
why you need to take diuretics for this
disorder. Thiazide diuretics have been
shown to stimulate the production of a
hormone that helps your body retain salt.
This added amount of salt keeps you from
losing too much water.
Signs and tests
MRI of the head
10. There are four major types of diabetes
insipidus. Central diabetes insipidus is
caused by problems with the pituitary gland.
Nephrogenic diabetes insipidus can
develop when the kidneys do not respond
properly to vasopressin. In dipsogenic
diabetes insipidus, the body’s system for
regulating the sense of thirst is damaged.
Finally, gestational diabetes insipidus
occurs in pregnant women when the
placenta destroys the mother’s
vasopressin.
Dipsogenic Diabetes Insipidus: A Newly Recognized Syndrome Caused by a Selective Defect in
the Osmoregulation of Thirst
Gestational diabetes insipidus. Treatment for most cases of gestational diabetes insipidus is with the synthetic hormone desmopressin. In rare cases, this form
of the condition is caused by an abnormality in the thirst mechanism. In these rare cases, doctors don't prescribe desmopressin.
Dipsogenic diabetes insipidus. There is no specific treatment for this form of DI. However, if the condition is caused by mental illness, treating the mental illness
may relieve dipsogenic DI.
11. Dipsogenic
dipsogenic diabetes insipidus is a syndrome of
disordered thirst, in patients without
psychiatric disease . Therapy with antidiuretic
hormone in patients with dipsogenic diabetes
insipidus is thought to be contraindicated for
fear of inducing water intoxication.
Dipsogenic diabetes insipidus is an
uncommonly (and not universally) recognized
disorder, requiring monitored testing in order
to distinguish it from incomplete forms of
central diabetes insipidus. Though therapy
with desmopressin cannot be recommended
based on the results of a single case, the
outcome presented here is intriguing and
suggests that larger studies in such patients is
warranted to assess the broader application of
such an intervention.
Gestrational
12. This is a type of diabetes that some women get
during pregnancy. Between 2 and 10 percent of
expectant mothers develop this condition,
making it one of the most common health
problems of pregnancy.
When the woman is pregnant, hormonal
changes can make your cells less responsive to
insulin. When the body needs additional insulin,
the pancreas dutifully secretes more of it. But if
your pancreas can't keep up with the increased
insulin demand during pregnancy, your blood
glucose levels rise too high, resulting in
gestational diabetes.
Most women with gestational diabetes don't
remain diabetic after the baby is born. Once
you've had gestational diabetes, though, you're
at higher risk for getting it again during a future
pregnancy and for developing diabetes later in
life.
13. What causes gestagenic diabetes insipidus?
Vasopressin, also called antidiuretic hormone
(ADH), is a substance in the body that regulates
the body's water retention. Although it's rare,
sometimes during pregnancy, an enzyme
secreted by the placenta can destroy
vasopressin. When that happens, the lack of
ADH results in excessive thirst and frequent
urination. In some cases, gestagenic diabetes
insipidus is caused by a deficiency in the body's
thirst mechanism..
What are symptoms of gestagenic diabetes
insipidus?
Most pregnant women find themselves making
frequent trips to the bathroom at night due to
the increasing pressure of the growing fetus on
the bladder. But frequent urination can also be
the first sign of gestagenic diabetes insipidus. If
it is, it usually correlates with an intense thirst.
Symptoms of too much fluid in the body with
gestational DI may include nausea,dizziness,
and weakness. If this happens, call your doctor
for a medical evaluation.
14. How is gestagenic diabetes insipidus
diagnosed?
To make a correct diagnosis of gestagenic
diabetes insipidus, your doctor will first conduct
a urinalysis to see if the urine is diluted and not
concentrated or yellow. In addition, your doctor
may send your blood for laboratory tests.
Further testing may be necessary.
How is gestagenic diabetes insipidus
treated?
The doctor may prescribe no treatment for
gestagenic DI. In that case, your doctor will
likely ask that you come to the office frequently.
The purpose of the office visits is to make sure
your body does not become overloaded with
fluid. In addition, you'll need to always keep
liquids with you so you don't get dehydrated.
Your doctor may prescribe desmopressin, a
synthetic form of ADH. Desmopressin is usually
delivered in a nasal spray. This treatment for
gestagenic diabetes insipidus helps manage
the control of urine. Desmopressin is also used
to treat bedwetting, since it allows the body to
absorb and manage water from the kidney.
15. If the body's thirst mechanism is the cause of
gestational DI, other treatments may be
prescribed.