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$Unique_ID{BRK03997}
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$Title{Meningococcemia}
$Subject{Meningococcemia Meningococcal Disease Meningococcemia-Meningitis
Fulminant Meningococcemia Waterhouse-Friderichsen Syndrome Chronic
Meningococcemia Rocky Mountain Spotted Fever Henoch-Schonlein Purpura Acute
Vasculitis Rheumatic Fever Toxic Shock Syndrome Bacterial Endocarditis}
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Copyright (C) 1992 National Organization for Rare Disorders, Inc.

916:
Meningococcemia

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

Synonyms

     Meningococcal Disease
     Meningococcemia-Meningitis

Disorder Subdivisions:

     Fulminant Meningococcemia (also known as Waterhouse-Friderichsen
Syndrome)
     Chronic Meningococcemia

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

     Rocky Mountain Spotted Fever
     Henoch-Schonlein Purpura
     Acute Vasculitis
     Rheumatic Fever
     Toxic Shock Syndrome
     Bacterial Endocarditis

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.

Meningococcemia is an infectious disease that occurs rarely.  However,
there are years when epidemics of the illness occur.  Major symptoms may
include upper respiratory tract infection, fever, skin rash and lesions, eye
and ear problems and possibly shock (a sudden state of extreme physical
depression which could cause a life-threatening situation).

Symptoms

Meningococcemia is characterized by sudden intense headache, nausea, fever,
vomiting, skin rash and in cases associated with meningitis, a stiff neck.
The patient may first complain of an upper respiratory infection.  Chills may
develop, then skin rash on the arms or legs and the trunk.  Diarrhea may also
be present.  Later the rash may become widespread or develop into bleeding
spots under the skin (petechiae, ecchymoses, or purpura).  There may be
associated swelling, muscle pain, skin deterioration or gangrene in the arms
and legs.  Pneumonia may also develop along with the other symptoms if the
person has a suppressed immune system.

In cases where meningitis occurs along with meningococcemia, the patient
may have the symptoms listed above along with the combination of headache,
confusion, stiff neck, and muscle pain from irritation of membranes
surrounding the brain and spinal cord (meningismus).  (For more information
on this disorder, choose "Meningitis" as your search term in the Rare Disease
Database).

Disorder Subdivision

Fulminant Meningococcemia is also known as Waterhouse-Friderichsen
Syndrome and is the most severe form of the disorder.  The disease comes on
very suddenly and the progression of the symptoms is very rapid.  In less
than a few hours the patient has very high fever, chills, weakness, vomiting
and severe headache.  A red rash appears on the arms and legs and spreads
very quickly over the body including the eyes and nose.  The patient's blood
pressure may drop dangerously and the fever may also drop dramatically.  The
patient may go into shock.  Without immediate medical treatment this disorder
can be life-threatening.

Chronic Meningococcemia is a rarer form of the disease.  It is
characterized by fever that comes and goes over a period of weeks or months.
Muscle and joint pain with headache as well as a skin rash may also come and
go.  This form of the disorder may also include an enlarged spleen.

Causes

Meningococcemia is caused by infection with the meningococci bacteria
(Neisseria memingitidis) which are gram-negative diplococci bacteria.  There
are various groups of this bacteria that cause different forms of the disease
and they are grouped by strains A,B,C,D,X,Y,Z, 29E and W135.  These groups
can be identified by testing the blood, scrapings of the skin rash and
samples of the cerebrospinal fluid of the patient.  Testing may take up to
five days as the cultures are very slow growing.

Infection with the bacteria is usually caused by a carrier.  The natural
place for the bacteria to be located is in either the nose or throat of the
carrier, and they can be spread by airborne or close contact methods.  The
carrier may spread the infection for weeks or months if they are not
diagnosed and treated.

Affected Population

Meningococcemia affects males and females in equal numbers.  However, most
cases develop in persons twenty years of age or younger and half of these
cases are in children under five years of age.  In the United States 1.2
cases per 100,000 occur annually.  Winter and spring are the most common
seasons of the year when cases are reported.  Epidemics occur under crowded
conditions and tend to occur at 20 to 30 year intervals.  In other parts of
the world epidemics are usually caused by the Group A strain of the bacteria.
During epidemics, rates of 5 to 24 cases per 100,000 persons have occurred.
In Sao Paulo, Brazil, during 1974 the epidemic rate was 370 per 100,000
persons infected with Meningococcemia.  In the United States, the most
prevalent Group strains of the bacteria are B,C,Y, and W135.

Related Disorders

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

Rocky Mountain Spotted Fever is a tick-borne disease that begins with an
incubation period of from two to twelve days.  A gradually or abruptly
beginning fever may be followed after three to five days by a pink or
purplish colored rash on the wrists and ankles.  The fever and rash usually
become more severe for seven to fourteen days.  The rash may not develop in
all cases, possibly making diagnosis difficult.  A blood test is necessary to
confirm the diagnosis.  (For more information on this disorder, choose "Rocky
Mountain" as your search term in the Rare Disease Database).

Shoenlein-Henoch Purpura is one of a group of disorders characterized by
purplish or brownish red discolorations on the skin.  These spots may be
large or small.  Internal bleeding may occur in various areas of the body.
This blood vessel disorder may affect the skin, joints, gastrointestinal
system, kidneys, and in a very few cases the central nervous system.  (For
more information on this disorder, choose "Shoenlein-Henoch" as your search
term in the Rare Disease Database).

Vasculitis is a common disorder characterized by an inflammation of the
blood vessel walls.  This inflammation causes a narrowing of the inside of
the vessel and can obstruct the flow of blood to the tissues.  Red or purple
patches of discoloration may develop under the skin.  Arteries and veins of
all sizes and in all parts of the body may be affected.  It may be localized
or affect multiple areas of the body with inflammatory and destructive
lesions.  There may be muscle pain, joint pain, fever, weight loss and loss
of appetite, headache, and generalized weakness.  (For more information on
this disorder, choose "Vasculitis" as your search term in the Rare Disease
Database).

Rheumatic Fever is an inflammatory syndrome that can occur following a
streptococcal infection.  Patients initially experience moderate fever, a
general feeling of ill health, a sore throat, fatigue and a red rash.  Major
complications can include heart disease, joint pain and arthritis,
involuntary abrupt limb movements with characteristic grimaces and skin
symptoms.  (For more information on this disorder, choose "Rheumatic" as your
search term in the Rare Disease Database).

Toxic Shock Syndrome symptoms appear very suddenly.  Initially, there is
a fever of 102 to 105 degrees F, headache, sore throat, and conjunctivitis.
Other early symptoms include profound lethargy, periods of disorientation,
vomiting, severe diarrhea, and a diffuse sunburn-like rash leading to
sloughing of skin after several days.  In severe cases, the syndrome may
progress to shock (dangerously low blood pressure and circulatory collapse)
within forty-eight hours.  (For more information on this disorder, choose
"Toxic Shock" as your search term in the Rare Disease Database).

Infective Endocarditis usually has a very sudden onset.  Complaints of
low back pain, pain in the joints (arthralgia) or in one or more muscles
(myalgia) are common.  These symptoms usually appear early in the disease,
occasionally as the only initial symptoms.  Fever, night sweats, chills,
headache and loss of appetite may also occur.  Blood or blood cells may be
present in the urine (hematuria), small red or purple spots composed of blood
(petechiae) may cover the skin of the upper trunk and there may also be pale,
oval spots on the retina of the eye.  (For more information on this disorder,
choose "Endocarditis" as your search term in the Rare Disease Database).

Therapies:  Standard

Meningococcemia is usually treated with Penicillin or Ampicillin.  In adults
the method of treatment is often through intravenous Penicillin G.  In
children penicillin is still the treatment of choice, however, other
organisms must be ruled out before treatment is begun.  For persons who are
unable to take penicillin, other antibiotics are used such as:  cefuroxime,
cefotaxime or ceftriaxone.

In persons who survive severe meningococcal septicemia there may be
ongoing problems with veins and arteries.  There are usually serious
orthopedic problems.  If gangrene occurs amputation may be necessary.  These
patients should have continuing medical evaluations as a precaution against
other conditions that can arise in later years.

During times of epidemics, chemoprophylaxis (Rifampin) is used to protect
persons exposed to or in close contact with infected patients.

Therapies:  Investigational

This disease entry is based upon medical information available through May
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 Meningococcemia, please contact:

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

     NIH/National Institute of Allergy & Infectious Diseases (NIAID)
     9000 Rockville Pike
     Bethesda, MD  20892
     [phone]

     Centers for Disease Control (CDC)
     1600 Clifton Road, NE
     Atlanta, GA 30333
     [phone]

References

CECIL TEXTBOOK OF MEDICINE, 19th Ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1990.  Pp. 1611-1617.

CLINICAL DERMATOLOGY, 2nd Ed.; Thomas P. Habif, M.D., Editor:  The C.V.
Mosby Company, 1990.  Pp. 210-211.

CHONDRO-OSSEOUS GROWTH ABNORMALITIES AFTER MENINGOCOCCEMIA, A CLINICAL
AND HISTOPATHOLOGICAL STUDY., Grogan, D.P., et al.; J Bone Joint Surg (AM),
July, 1989, (issue 71 (6)).  Pp. 920-928.