CD-ROM Today 1996 January/CD-ROM Today 1996 January.iso/dp/0366/03663.txt

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$Unique_ID{BRK03663}
$Pretitle{}
$Title{Diabetes, Insulin-Dependent}
$Subject{Diabetes Insulin-Dependent Type I Diabetes Juvenile Diabetes
Diabetes Mellitus}
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Copyright (C) 1986, 1987, 1990, 1991, 1992 National Organization for Rare
Disorders, Inc.

220:
Diabetes, Insulin-Dependent

** IMPORTANT **
It is possible the main title of the article (Insulin-Dependent Diabetes)
is not the name you expected.  Please check the SYNONYMS listing to find the
alternate names and disorder subdivisions covered by this article.

Synonyms

     Type I Diabetes
     Juvenile Diabetes
     Diabetes Mellitus

General Discussion

** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.


Insulin-dependent Diabetes is a disorder in which the body does not
produce enough insulin and is, therefore, unable to convert nutrients into
the energy necessary for daily activity.  The disorder affects females and
males approximately equally.  Although the causes of insulin-dependent
diabetes are not known, genetic factors seem to play a role.

Symptoms

Normally, sugars and starches (carbohydrates) in the foods we eat are
processed by digestive juices into glucose.  Glucose circulates in the blood
as a major energy source for body functions.  Its use is regulated primarily
by insulin, a hormone produced by the pancreas gland (located behind the
stomach).  In the person with diabetes, there is a malfunction in the
production of insulin.  There are two main types of diabetes:  Type I or
Insulin-Dependent and Type II or Noninsulin-Dependent.

The insulin-dependent type of diabetes generally has onset during
childhood or adolescence, though it can occur at any age.  Because the
pancreas supplies little or no insulin in this disease, daily injections of
the hormone and a controlled diet are necessary to regulate blood sugar
levels.  Insulin is generally effective in preventing glucose buildup, but it
is a treatment and not a cure for diabetes.

The onset of Insulin-Dependent Diabetes begins with frequent urination,
extreme thirst, constant hunger, and unexplained weight loss.  Because people
with Type I Diabetes lack sufficient insulin, glucose accumulates in the
blood to levels too high for the kidneys to excrete.  In an effort to remove
the excess sugar, the kidneys excrete large amounts of water as well as
essential body elements resulting in frequent urination, thirst, and
weakness.  Hunger and fatigue are caused by the body's inability to utilize
foods properly for nourishment and energy.  To find alternate sources of
energy, the body turns to its stores of fat and protein, causing weight loss
and the accumulation of fat breakdown products (acetone and related acids) in
the blood.  These metabolites of fat produce increased acidity of the blood,
and a potentially fatal condition (ketoacidosis) can result if treatment is
not prompt.

A child with Type I Diabetes may also fail to grow and develop normally.
Diabetics of all ages may experience itching of the skin, changes in vision,
and slow healing of cuts and bruises.  Medical attention should be sought if
any of these symptoms occur.

The diabetic condition can result in certain long-term complications
which may involve many organs of the body.  The blood vessels, nervous
system, kidneys and eyes are particularly affected.  While successful control
of blood glucose levels may reduce the risk of complications, the exact
relationship between these factors is not fully understood.  Studies are
being conducted to determine whether strict blood glucose control plays a
significant role in preventing or delaying the onset of complications
resulting from diabetes.

1.  Cardiovascular Complications.
     Heart and blood vessel diseases such as heart attack, hardening of the
arteries (arteriosclerosis), and stroke are the leading causes of illness,
disability and death among diabetics.  Persons with diabetes are twice as
likely to suffer from coronary heart disease and stroke and five times as
likely to suffer from arterial disease of the limbs than the non-diabetic
population.  Exactly how diabetes damages the cardiovascular system is not
yet clear.

2.  Diabetic Nephropathy (Kidney Disease).
     Kidney (renal) disease, or diabetic nephropathy, can be a serious
complication of diabetes.  Normally, the kidneys cleanse impurities from the
blood, but diabetes can cause damage to the blood vessels in the kidney and
interfere with this vital process.  A procedure called hemodialysis is
frequently used to remove waste products from the blood when the kidneys can
no longer perform this function adequately.  Diabetics with serious renal
disease may also be candidates for a kidney transplant if a suitable donor
organ is available.

3.  Diabetic Neuropathy (damage to the nerves).
     Diabetes can also cause a complication called Diabetic Neuropathy which
is damage to the peripheral nerves.  These nerves run throughout the body,
connecting the spinal cord to muscles, skin, blood vessels, and all other
organs.  Most importantly, they serve as the primary link between the central
nervous system and the entire body.  Diabetes is a common cause of peripheral
neuropathy; however, this condition can also result from injury, alcoholism,
or other factors.  Almost all people with diabetes eventually develop some
peripheral nerve involvement, but for many, it is slight and produces no
symptoms.  For the 10 to 25 percent who suffer from serious neuropathy, it
can produce problems such as tingling and numbness in the feet, dizziness,
impotence, leg pain and double vision.

4.  Diabetic Retinopathy (damaged retina).
     Diabetes is the leading cause of partial loss of vision and new blindness
in the United States today.  Generally, diabetes affects the retina of the
eye and produces a variety of changes referred to as diabetic retinopathy.
Less frequent, but no less serious, are several other eye problems caused by
diabetes including cataracts, glaucoma, and optic nerve disease.  While many
persons with diabetes undergo some changes in the retina, only about 3
percent are seriously threatened with blindness.  Today, there are
approximately 150,000 Americans who suffer significant visual impairment due
to diabetic retinopathy.  (For more information on this disorder, choose
"Diabetic Retinopathy" as your search term in the Rare Disease Database.)

Although the way in which diabetes damages eye tissue is not known, two
important treatments have been developed in recent years.  The first, laser
photocoagulation, uses finely focused beams of laser light to seal off and
destroy abnormal retinal vessels and diseased tissues.  While this treatment
does not prevent diabetic changes from taking place, it has proven beneficial
in reducing the risk of severe vision loss in many cases.

The second technique, vitrectomy, involves surgical removal of cloudy eye
fluids that result from blood vessel hemorrhage.  This procedure offers hope
to people with severely impaired vision resulting from this form of diabetic
retinopathy.  Scientists continue to explore better use of these techniques
as well as the basic causes of eye damage resulting from diabetes.

5.  Complications of Pregnancy.
     Diabetic women run a greater risk of having babies who are stillborn, die
in infancy, and suffer from congenital defects than do non-diabetics.  It is
not uncommon for infants of diabetic mothers to be larger than normal at
birth if maternal blood glucose level is elevated.  In fact, this is
sometimes a warning sign of diabetes in a woman who has not yet been
diagnosed with the disorder.  Strict attention to control of diabetes is
essential during pregnancy to help reduce risks to both mother and baby.

Diabetic emergencies which require prompt medical attention include
hypoglycemia and ketoacidosis.  Hypoglycemia, also called "insulin reaction"
or "insulin shock" can occur if the blood sugar level of the person with
diabetes falls too low.  This results from too much insulin in the system
caused by too large a dose of insulin, overly strenuous exercise, or failure
to eat shortly after insulin is taken.  Although each person may react
differently, common symptoms of insulin reaction include trembling, sweating
and nervousness.  Some persons with diabetes may experience hunger, headache,
nausea, drowsiness, or symptoms similar to inebriation.  In severe cases of
insulin shock, the person with diabetes may even become unconscious.  A
careful blend of correct amounts of insulin, exercise and food can usually
prevent insulin reaction.  When hypoglycemia does occur, however, most people
with diabetes sense early warning signals and eat or drink something sweet to
raise the amount of sugar in the blood.  If a person is unconscious, an
injection of glucose solution or the hormone glucagon (which stimulates the
production of glucose), should be administered.

Ketoacidosis, or Diabetic Coma, results from too little insulin in the
system.  When the body is unable to use glucose for fuel, it draws on its own
stores of protein and fat for energy.  Acids, or ketones, produced by the
excessive breakdown of fat then accumulate in the blood stream quicker than
the kidneys can excrete them.  Unlike hypoglycemia, the symptoms of
ketoacidosis develop slowly over a period of days.  The person with diabetes
may begin to experience abdominal pain, nausea, vomiting, rapid breathing and
drowsiness.  If left untreated, ketoacidosis can progress to coma and death.

Ketoacidosis can be prevented by careful daily evaluation of insulin
needs.  Particularly stressful situations such as illness or surgery may
require increased amounts of insulin.  Most importantly, a person with
diabetes should never skip or delay an insulin injection and should pay
careful attention to his/her diet.

A survey by the U.S. Department of Health, Education and Welfare during
1960 to 1962 on forty-four million adults showed that men and women with
diabetes ran a higher than average risk of periodontal disease.

Causes

The exact causes of Insulin-Dependent Diabetes are not known although most
types of diabetes are known to have hereditary factors.  Scientists believe
that both heredity and environment may play important roles in the
development of diabetes.

Research suggests that certain viruses, in combination with genetic
susceptibility and other unknown environmental factors, may trigger the onset
of some types of this disorder.  High levels of growth hormone in adolescents
and adults under stress may also stimulate the production of glucose by the
liver.

Affected Population

Onset of Insulin-Dependent Diabetes usually occurs before 40 years of age;
onset of this type of Diabetes in late adulthood is less common, but it can
occur.  The disorder affects females and males approximately equally.  Two
million Americans may be affected by this disorder.

Related Disorders

Type II Diabetes (Non-Insulin Dependent Diabetes Mellitus) is the more common
form of the disorder.  Also known as Adult Onset Diabetes, it usually occurs
after the age of 40 years.  This type of diabetes is not secondary to other
diseases or conditions.  In many cases, the disorder can be controlled
through diet, regular exercise, and sometimes, with oral medications (e.g.,
chlorpropamide, glypizide, or glyburide).  Some scientists believe that
people with non-insulin dependent diabetes do not convert glucose (sugar)
into its starch-like storage form, glycogen, at the same rate as normal
people.  Glucose is converted in the muscles.  This means that there may be
two defects in people with non-insulin dependent diabetes.  Glucose may not
get into muscle cells normally.  The beta cells in the pancreas, which
respond to blood glucose levels, work overtime to increase their secretion of
insulin.  Over a long period of time the beta cells can't keep up their high
pace, and they begin to fail.  This can cause insulin-dependent diabetes.

Therapies:  Standard

A daily routine of insulin-injection, controlled diet, exercise to burn off
glucose, and testing for blood sugar level is vital in achieving and
maintaining good blood sugar control in patients with Insulin-Dependent
Diabetes.  Urine testing for glucose spillage had been a standard
recommendation in past years, but has now been replaced with self blood
glucose testing.  Self monitoring of blood glucose levels uses a single drop
of blood which is obtained with a finger stick, and placed on a chemically
treated pad on a plastic strip; the color change of the chemically treated
pad is compared to a color chart or "read" by a battery operated portable
meter.

Insulin must be given by injection, usually two or more times each day.
Recently portable "insulin pumps" have been developed, which permit
continuous administration of insulin, as well as additional amounts of
insulin when needed to control the changes in blood sugar level that occurs
after meals.

Therapies:  Investigational

In recent years, research supported by the National Institute of Diabetes,
Digestive and Kidney Diseases (NIDDK), and other components of the National
Institutes of Health, and non profit agencies (See Resources) that fund
scientific research on diabetes has yielded new and exciting information on
the possible causes and improved management of diabetes and its
complications.  Scientists have now identified genetic factors that appear to
be associated with diabetes - a finding that could lead to methods of
prevention of the disorder in genetically susceptible persons.  In related
studies, the discovery that the insulin-producing beta cells can be infected
and destroyed by common viruses could eventually result in the development of
a vaccine to prevent diabetes.

Pancreas transplantation has had limited success, primarily due to the
problem of rejection.  However, recent advances in immunology have raised
hopes that the problem of rejection reaction common in organ transplantation
may be altered or prevented.  These findings increase the possibility of
transplanting healthy insulin-producing cells to correct the diabetic
condition.  Recently, a clinical study to assess the effectiveness of a
programmable implantable insulin pump for unstable diabetes has been funded
by FDA's Orphan Product Grant program.  This orphan device is being studied
at the University of California at Irvine, CA by Dr. M. Arthur Charles.

Although these advances hold great promise for the future, it is
important to recognize that they are still in the research phase and are not
part of the routine treatment of diabetes.

Exciting research on all aspects of diabetes is now being conducted at
medical institutions throughout the United States and abroad.  The
association of heredity, viral infections, the presence of high levels of
growth hormone, and immunology has provided new leads in the treatment and
gives hope for ultimate prevention of diabetes.

New information, based on experiments on mice, has shed new light on the
understanding in insulin secretion.  The signaling process between glucose
stimulation and insulin secretion seems to be under the direct influence of
calcium in the cell.  How and why insulin secretion is disrupted in diabetes
and how this disruption can be prevented or repaired could help lead to
finding a cure for diabetes.

Scientists are investigating how precise control of diabetes compares to
the usual therapies for diabetes in order to learn how best to prevent or
delay the serious complications of the disorder.  To participate in the
Diabetes Control and Complications Trial (DCCT), a person must be between 13
and 39 years of age; had Insulin-Dependent Diabetes for at least one year but
not more than 15 years; not have any severe complications such as eye or
kidney disease; and not be taking more than 2 insulin injections per day or
using an insulin pump.  For more information on this study, call:  (800) 522-
DCCT.  This research project is sponsored by the National Institute of
Diabetes, Digestive and Kidney Diseases (NIDDK).

Transplanting a whole pancreas or a segment of one shows promise in
restoring normal insulin production in some Diabetes patients.  More than
1,000 pancreas transplants have been performed worldwide, with varying
degrees of success.  Immunosuppressive drug treatment following
transplantation may consists of a combination of cyclosporine, prednisone,
and azathioprine (Aza), although these drugs may be slightly toxic in large
doses.  Because transplant patients will have to undergo a lifetime of
immunosuppression, this procedure is recommended only for individuals whose
complications are more severe than those caused by immunosuppression.

A new therapy aimed at treating persons who may become diabetic in a few
years by giving them the immunosuppression drug Imuran is under
investigation.  More study is necessary to determine the long-term safety and
effectiveness of this form of treatment.

Clinical trials are underway to study pancreas transplantation and the
monitoring of graft function.  Interested persons may wish to contact:

     Dr. A. Osama Gaber
     University of Tennessee - Memphis
     956 Court Ave., Suite G212
     Memphis, TN  38163
     [phone]

to see if further patients are needed for this research.

Clinical trials are underway to study molecular genetics of heritable
insulin resistance (A).  Interested persons may wish to contact:

     Louis J. Elsas, II, M.D.
     Emory University, Dept. of Pediatrics
     2040 Rigewood Dr.
     Atlanta, GA  30322
     [phone]

to see if further patients are needed for this research.

Clinical trials are underway to study the genetic location causing non-insulin
diabetes and especially neonatal diabetes.  Interested persons may wish to
contact:

     Dr. M. Alan Permutt
     Washington University School of Medicine
     Metabolic Division
     660 S. Euclid
     St. Louis, MO  63110
     [phone]

to see if further patients are needed for this research.

This disease entry is based upon medical information available through
January 1992.  Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate.  Please check with the agencies listed in the Resources section for
the most current information about this disorder.

Resources

For more information on Insulin-Dependent Diabetes, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     American Diabetes Association
     National Service Center
     1660 Duke St.
     Alexandria, VA  22314
     [phone]
     (800) ADA-DISC [phone])

     Juvenile Diabetes Foundation International
     60 Madison Avenue, 4th Floor
     New York, NY  10010
     [phone]

     National Diabetes Information Clearinghouse
     Box NDIC
     Bethesda, MD  20892
     [phone]

For information on genetics and genetic counseling referrals, please
contact:

     March of Dimes Birth Defects Foundation
     1275 Mamaroneck Avenue
     White Plains, NY  10605
     [phone]

     Alliance of Genetic Support Groups
     35 Wisconsin Circle, Suite 440
     Chevy Chase, MD  20815
     (800) 336-GENE
     [phone]

References

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W. B. Saunders Co., 1988.  Pp. 1360-81.

THE MERCK MANUAL 15th ed:  R. Berkow, et al:  eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  Pp. 1069-71.

MUSCLES PINPOINTED AS SITE OF DIABETIC DEFECT, Collins, J., Research
Resources Reporter, (September 1990, issue 14 (9)).  Pp. 1-3.