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$Unique_ID{BRK04072}
$Pretitle{}
$Title{Osteonecrosis}
$Subject{Osteonecrosis Avascular Necrosis Vasculitis Osteopetrosis Rheumatoid
Arthritis Lupus Erytheomatosus Legg-Calve-Perthes Syndrome Sickle Cell Disease
Gaucher's Disease Polycythemia Vera Caisson Disease}
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Copyright (C) 1990, 1992 National Organization for Rare Disorders, Inc.

756:
Osteonecrosis

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

Synonyms

     Avascular Necrosis

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

     Vasculitis
     Osteopetrosis
     Rheumatoid Arthritis
     Lupus Erytheomatosus
     Legg-Calve-Perthes Syndrome
     Sickle Cell Disease
     Gaucher's Disease
     Polycythemia Vera
     Caisson Disease

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.

Osteonecrosis is the destruction of a bone (necrosis) often due to an
inadequate supply of blood to a bone.  It most commonly affects the joints
and bones of the hips, knees and shoulder.  It often occurs as a result of
bone injuries or in conjunction with other diseases and conditions.

Symptoms

Osteonecrosis is a prevalent slowly progressive prevalent disease frequently
caused by a reduction of blood flow to an area that involves bones and joints
causing the bones to crack and eventually collapse.  This condition usually
occurs as the result of other diseases, injuries or conditions.  Pain is the
primary symptom.  It is a chronic and mild pain, usually occurring when
standing, walking or lifting.  The pain becomes worse when weight bearing
activities exert pressure on the bones or joints.  The pain may progress,
eventually occurring while at rest or even disturbing sleep.  Other symptoms
include muscle spasms, joint stiffness and limitation of motion.
Osteonecrosis most commonly affects the head of the femoral bone but may also
involve the head of the humerus bone, the round protruding area at the end of
the femur bone (condyles), the shin bone (distal tibia) and ankle (talus).

Causes

Osteonecrosis is the death of bone tissue associated with various diseases.
A common cause is trauma that can cause a dislocation or fracture of the neck
of the femur bone.  Bones may also be affected by the use of certain drugs
such as corticosteroids (glucocorticoids), or radiation and chemotherapy used
in treating cancer patients.  Osteonecrosis may also be a complication of
kidney transplantation, sickle cell disease, alcoholism and other disorders.

Affected Population

Osteonecrosis is a common progressive disorder that can occur at any age, but
is more frequently seen in people between 30 and 60 years of age.
Osteonecrosis of the hip is slightly more common in men while Osteonecrosis
that affects the knees is seen three times more often in women.  It is also
more common in those people with rheumatic diseases (e.g., rheumatoid
arthritis, systemic lupus erythematosus), steroid users (such as cortisone),
alcoholics, diabetics, and skin divers who have experienced a rapid reduction
of air pressure (bends).

Related Disorders

Symptoms of the following disorder may be similar to those of Osteonecrosis.
Comparisons may be useful for a differential diagnosis:

Osteopetrosis is a combination of several rare genetically caused
symptoms grouped together as one disorder.  It can be inherited as either a
dominant or recessive trait and is marked by increased bone density, brittle
bones, and in some cases skeletal abnormalities.  Although symptoms may not
initially be apparent to people with mild forms of this disorder, trivial
injuries may cause bone fractures due to abnormalities of the bone.  The
dominantly transmitted form is milder than the recessive form and may not be
diagnosed until adolescence or adulthood when symptoms first appear.  More
serious complications occur in the recessive form which may be diagnosed from
examination of skeletal x-rays during infancy or childhood.  (For more
information on this disorder, choose "Osteopetrosis " as your search term in
the Rare Disease Database).

The following disorders may be associated with the development of
Osteonecrosis.  They are not necessary for a differential diagnosis:

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 (ischemia).  The
lack of blood may cause damage to the tissues (necrosis), possible formation
of blood clots (thrombosis), or a weakening or ballooning which can possibly
cause a rupture of the vessel wall (aneurysm).  Osteonecrosis can occur in
people with vasculitis when blood flow has been obstructed to the bone or
joint.  (For more information on this disorder, choose "Vasculitis" as your
search term in the Rare Disease Database.)

Rheumatoid Arthritis is an inflammatory autoimmune disease in which the
body's natural defenses against foreign agents (antibodies & lymphocytes)
attack healthy joints.  This disorder is characterized by a lack of appetite
(anorexia), fatigue, painful and deformed joints, early morning stiffness
chiefly in the hands, knees, feet, jaw, and spine.  People with Arthritis are
at increased risk of also developing Osteonecrosis.  (For more information on
this disorder, choose "Arthritis" as your search term in the Rare Disease
Database).

Lupus (also known as SLE or Systemic Lupus Erythematosus) is an
inflammatory connective tissue disease that can affect many parts of the body
including the joints, skin and internal organs.  Lupus is a disease of the
body's immune system, most often striking young women between the ages of 15
and 35 years.  People who have Lupus are also at increased risk of developing
Osteonecrosis.  (For more information on this disorder, choose "Lupus" as
your search term in the Rare Disease Database).

Legg-Calve-Perthes Syndrome is a rare disease affecting the hip joint.
Abnormalities in bone growth early in life may result in permanent deformity
of the hip joint several years later.  Osteonecrosis of the femoral head is
often seen in those people with Legg-Calve-Perthes Disease.  (For more
information on his disorder, choose "Legg-Calve-Perthes" as your search term
in the Rare Disease Database.)

Gaucher's Disease is an inherited disease of lipid metabolism caused by
the failure to produce the enzyme glucocerebrosidase.  It is the most common
of the 14 known lipid storage disorders which includes Tay-Sachs, Fabry's
Disease, and Niemann-Pick Disease.  There are three types of Gaucher's
Disease Type I, II and III.  All three are characterized by the presence of
Gaucher (lipid-laden) cells in the bone marrow and other organs such as the
spleen and liver.  Bone deterioration (Osteonecrosis) is a major symptom of
this disease and can affect any part of the body.  (For more information on
this disorder, choose "Gaucher" as your search term in the Rare Disease
Database.)

Sickle Cell Disease is an inherited blood disease.  Symptomatic of this
disease are the "crisis periods" which often occur in conjunction with other
infections.  It is characterized by joint pain (arthralgia), fever, severe
abdominal pain, vomiting, and damage to the head of the femur bone
(osteonecrosis).  (For more information on this disorder, choose "Sickle
Cell" as your search term in the Rare Disease Database).

Polycythemia Vera is a chronic proliferative disorder of the bone marrow.
It is characterized by an increase in the number of red blood cells
(erythrocytosis) and hemoglobin concentration in the blood.  Osteonecrosis
can occur in Polycythemia Vera.  (For more information on this disorder,
choose "Polycythemia Vera" as your search term in the Rare Disease Database.)

Caisson Disease, also called "Decompression Sickness" or the "Bends", is
a disorder caused by the formation of nitrogen bubbles in the tissues and
blood.  This occurs from a very rapid reduction of air pressure after rising
quickly from deep water with high atmospheric pressure, to normal air
pressure.  It is characterized by painful joints, bone deterioration
(Osteonecrosis), chest tightness, giddiness, abdominal pain, vomiting and
visual difficulties.  In some with this disorder there may also be
convulsions and paralysis.

Therapies:  Standard

Treatment of Osteonecrosis consists of diagnosing, treating or eliminating
the underlying cause.  Bones damaged or weakened by this disorder will usually
heal and regenerate with appropriate treatment.  Limiting or avoiding
alcohol, weight bearing activities, standing or walking may help in the
recovery process.  Bed rest and reducing stress to the affected area are
helpful.  The pain associated with this disorder can be relieved with aspirin
or non-steroidal anti-inflammatory drugs such as ibuprofen.  Warm baths,
heating pads and electric blankets may also be helpful in relieving the
muscle spasms and pain associated with Osteonecrosis.  X-Ray's can be helpful
in diagnosing Osteonecrosis and determining the extent of bone damage.
Surgery may be necessary when there is a dislocation, fracture or if the bone
has collapsed.  Other treatment is symptomatic and supportive.  The most
often used method of treatment for advanced Osteonecrosis of either the knee
or hip is replacement of the affected area with a prosthetic joint.

Therapies:  Investigational

This disease entry is based upon medical information available through June
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 Osteonecrosis, please contact;

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

     Arthritis Foundation
     1314 Spring Street, NW
     Atlanta, GA  30309
     [phone]

     NIH/National Arthritis, Musculoskeletal and Skin Diseases Information
     Clearinghouse
     Box AMS
     Bethesda, MD 20892
     [phone]

References

TEXTBOOK OF MEDICINE, 18th ED.:  James B. Wynngaarden, M.D., et al.; ed.-in-
chief; W.B. Saunders Co. 1988.  Pp. 1517

OSTEONECROSIS OF THE HIP IN THE SICKLE-CELL DISEASES.  TREATMENT AND
COMPLICATIONS.  G. Hanker, et al.; J BONE SURG [AM] (April 1988, issue 70
(4)).  Pp. 499-506.

INFLUENCE OF ALCOHOL INTAKE, CIGARETTE SMOKING, AND OCCUPATIONAL STATUS
ON IDIOPATHIC OSTEONECROSIS OF THE FEMORAL HEAD.  K. Matsuo, et al.; CLIN
ORTHOP (September 1988, (234)).  Pp. 115-123.

OSTEONECROSIS OF THE FEMORAL HEAD.  PATHOGENIS AND LONG-TERM RESULTS OF
TREATMENT.  M. Meyers; CLIN ORTHOP (June 1988 (231)).  Pp. 51-61.

SURVEY OF THE LONG TERM INCIDENCE OF OSTEONECROSIS OF THE HIP AND ADVERSE
MEDICAL EVENTS IN RHEUMATOID ARTHRITIS AFTER HIGH DOSE INTRAVENOUS
METHYLPREDNISOLONE.  I. Williams, et al.; ANN RHEUM DIS (November 1988, issue
47(11)).  Pp. 930-933.