CD-ROM Today 1996 January/CD-ROM Today 1996 January.iso/dp/0385/03850.txt

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$Unique_ID{BRK03850}
$Pretitle{}
$Title{Hypoglycemia}
$Subject{Hypoglycemia Exogenous Hypoglycemia Factitious Hypoglycemia Fasting
Hypoglycemia Iatrogenic Hypoglycemia Infantile Hypoglycemia Neonatal
Hypoglycemia Reactive Functional Hypoglycemia Reactive Hypoglycemia, Secondary
to Mild Diabetes Spontaneous Hypoglycemia Tachyalimentation Hypoglycemia Low
Blood Sugar Insulin-Dependent Diabetes Hereditary Fructose Intolerance
Galactosemia }
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Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.

574:
Hypoglycemia

** IMPORTANT **
It is possible that the main title of this article (Hypoglycemia) is not
the name you expected.  Please check the SYNONYM list to find the alternate
names and disorder subdivisions covered by this article.

Synonyms

     Exogenous Hypoglycemia
     Factitious Hypoglycemia
     Fasting Hypoglycemia
     Iatrogenic Hypoglycemia
     Infantile Hypoglycemia
     Neonatal Hypoglycemia
     Reactive Functional Hypoglycemia
     Reactive Hypoglycemia, Secondary to Mild Diabetes
     Spontaneous Hypoglycemia
     Tachyalimentation Hypoglycemia
     Low Blood Sugar

Information on the following disorder can be found in the Related
Disorders section of this report:

     Insulin-Dependent Diabetes
     Hereditary Fructose Intolerance
     Galactosemia

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 physician and/or the agencies listed in the "Resources" section
of this report.

Hypoglycemia is a common condition characterized by an abnormally low
blood sugar (glucose) level.  Glucose is essential for the functioning of
many organs and systems in the body, especially the central nervous system.

Symptoms

Hypoglycemia means an abnormally low blood sugar (glucose) level.  This level
is kept in a delicate balance by the passing of glucose through the blood
vessels into surrounding tissues.  When muscles, nerves or cells need glucose
for energy, this substance passes from the blood into the tissue.  Insulin
secreted by the pancreas normally causes the liver to pass stored glycogen
(which is subsequently converted into glucose) into the blood stream.
Hypoglycemia can be caused by excessive levels of insulin in the body, or
insufficient levels of glucose in the blood.

Symptoms of Hypoglycemia may be grouped into two categories:

1. Faintness, weakness, jitteriness, profuse perspiration, excessive
hunger, and nervousness.

2. Central nervous system symptoms including headaches, confusion, visual
disturbances, muscle weakness, paralysis, impaired muscle coordination
(ataxia), and marked personality changes.  Untreated, these central nervous
system disturbances may progress to loss of consciousness, convulsions, and
coma.  The pace and severity of Hypoglycemia attacks may vary over time.

Causes

There are two main types of Hypoglycemia:

A:  Fasting Hypoglycemia is caused by:

Too much insulin in the blood (Hyperinsulinism) which may result from
pancreas beta-cell tumors, an overgrowth (hyperplasia), or autoimmune
factors, which occurs when the body has produced antibodies to either its own
insulin or its insulin receptors.  These problems can produce too much
insulin and/or too much glucose in the blood.

Fasting (without food) Hypoglycemia can also be due to tumors outside the
pancreas.  Drugs such as insulin or sulfonylureas taken by diabetics, or the
prolonged abuse of alcohol without eating any food, are another cause of this
disorder.  Deficits of hormones such as glucocorticoids, growth hormone, and
possibly glucagon or epinephrine, may also result in Fasting Hypoglycemia.

Extensive impairment of liver function, chronic kidney failure, or
disorders characterized by severe tissue wasting may be associated with
Fasting Hypoglycemia as well.

B:  Reactive Hypoglycemia may result from:

Eating a high amount of carbohydrates or rapid absorption of glucose into
the circulation and subsequent outpouring of excessive amounts of insulin.
In this instance glucose is used very quickly by the body leading to lowering
of the blood sugar level again.  A similar reactive Hypoglycemia due to
delayed insulin-response occurs in some people with mild maturity-onset
diabetes after they eat carbohydrates.  Hypoglycemia may also occur after a
period of prolonged alcohol abuse.  The condition may also result from a
combination of starvation and impaired glucose production by the liver from
glycogen, the stored energy source of the body.  This type of hypoglycemia
can usually be controlled by proper diet and avoidance of alcohol.

Affected Population

Hypoglycemia affects males and females of all ages in equal numbers.  It
affects several million people.

Related Disorders

Symptoms of the following disorders can be similar to those of Hypoglycemia.
Comparisons may be useful for a differential diagnosis:

Galactosemia is a hereditary disorder of carbohydrate metabolism.  It is
a rare inability to convert galactose (a sugar contained in milk) to glucose
(a different type of sugar).  The disorder is caused by a deficiency of the
enzyme "galactose-1-phosphate uridyl transferase".  After an apparently
normal period, an infant may lose its appetite and start vomiting
excessively.  Yellow jaundice and liver enlargement may also occur.  Since
milk is the main staple of an infant's diet, early diagnosis and treatment of
this disorder is absolutely essential to avoid serious lifelong disability.
(For more information, choose "Galactosemia" as your search term in the Rare
Disease Database.)

Hereditary Fructose Intolerance is a hereditary inability to digest fruit
sugar (fructose) or sucrose (sugar, sorbitol and brown sugar).  This is
caused by a deficiency of the enzyme 1-phosphofructoa in the liver, kidney
cortex and small intestine.  After adding fructose to an affected infants
diet, prolonged vomiting, failure to thrive, occasional unconsciousness,
jaundice, enlargement of the liver and a tendency to bleed may occur.
Decreased levels of glucose and phosphate are found in the blood and
increased levels of fructose appear in the blood and urine.  (For more
information, choose "Fructose" as your search term in the Rare Disease
Database.)

Insulin-Dependent Diabetes is a common disorder in which the body does
not produce enough insulin and is therefore unable to convert nutrients into
the energy necessary for daily activity and proper functioning of the central
nervous system.  The disorder affects males and females approximately
equally.  Although the causes of Insulin-Dependent Diabetes are not known,
genetic factors seem to play a role.  (For more information, choose
"Diabetes" as your search term in the Rare Disease Database.)

Therapies:  Standard

Treatment of acute attacks of Hypoglycemia consists of either immediately
eating sugar or intravenous injection of glucose.  Glucagon, a natural body
substance which promotes release of glucose by the liver, can be injected
into the muscles in some cases.  The effect of glucagon lasts only a short
period of time so that it is imperative to provide glucose as soon as
possible.  The plasma glucose level should be monitored to prevent
Hypoglycemia from recurring.

After initial control of acute Hypoglycemia is established, the
underlying cause can be determined and treated.  In drug-induced cases, the
drug should be withdrawn.  Hypoglycemia associated with tumors outside the
pancreas may improve by removal or reduction in the tumor size.  Recurrent
fasting Hypoglycemia as in chronic kidney failure, may respond to a diet of
frequent high-calorie meals.

The preferred treatment of fasting Hypoglycemia due to hyperinsulinism is
complete surgical removal of pancreas tumors, and partial removal of multiple
tumors, leaving sufficient pancreas tissue to preserve function.   Diabetes
may follow removal of pancreas tissue in some cases.

When insulin-producing tumors have spread (metastatic insulinoma) from
the pancreas to other parts of the body, the chemotherapy drug streptozotocin
may be prescribed.  However, toxicity to the kidney may limit use of this
drug.  Responses to the cancer drugs doxorubicin (adriamycin) and mithramycin
have also been described in the medical literature.

Medical treatment of hyperinsulinism that is not caused by tumors
includes frequent meals without sugars and the avoidance of hunger.
Diazoxide, a drug that suppresses insulin secretion may limit glucose
utilization through other mechanisms.  However, side effects may include
swelling (edema), nausea and excessive growth of hair (hypertrichosis).  An
abnormally low blood pressure and lack of certain white blood cells
(granulocytopenia) have also occurred with use of this drug.

Treatment for reactive Hypoglycemia may not be necessary.  The plasma
glucose level will return to normal levels.  A diet that includes frequent
meals and avoidance of simple sugars is usually sufficient.

Therapies:  Investigational

This disease entry is based upon medical information available through
December 1988.  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 Hypoglycemia, please contact:

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

     National Hypoglycemia Association
     P.O. Box 120
     Ridgewood, NJ  07451
     [phone]

     American Diabetes Association
     1660 Duke Street
     Alexandria, VA  22314
     [phone]
     (800) ADA-DISC [phone])

     National Digestive Diseases Information Clearinghouse
     Box NDDIC
     Bethesda, MD  20892
     [phone]

References

INTERNAL MEDICINE, 2nd ed.:  Jay H. Stein, et al., eds; Little, Brown, 1987.
Pp. 1024-1030.

FACTITIOUS HYPOGLYCEMIA DUE TO SURREPTITIOUS ADMINISTRATION OF INSULIN.
DIAGNOSIS, TREATMENT, AND LONG-TERM FOLLOW-UP:  G. Grunberger, et al.; Ann
Intern Med (February 1988:  issue 108(2)).  Pp. 252-257.

PREDICTING NOCTURNAL HYPOGLYCEMIA IN PATIENTS WITH TYPE I DIABETES
TREATED WITH CONTINUOUS INSULIN INFUSION:  A. Schiffrin, et al.; American
Journal Med (June 1987:  issue 82(6)).  Pp. 1127-1132.

PERSISTENT NEONATAL HYPERINSULINISM:  P. M. Mathew, et al.; Clin Pediatr
(Phila) (March 1988:  issue 27(3)).  Pp. 148-151.