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- $Unique_ID{BRK03922}
- $Pretitle{}
- $Title{Legg-Calve-Perthes Syndrome}
- $Subject{Legg-Calve-Perthes Syndrome Coxa Plana Osteochondrosis Deformans
- Juvenilis Capital Femoral Epiphysis Perthes Disease Pseudocarolgia Quiet Hip
- Ankylosing Spondylitis }
- $Volume{}
- $Log{}
-
- Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.
-
- 563:
- Legg-Calve-Perthes Syndrome
-
- ** IMPORTANT **
- It is possible that the main title of the article (Legg-Calve-Perthes
- Syndrome) is not the name you expected. Please check the synonym list to
- find the alternate names and disorder subdivisions covered by this article.
-
- Synonyms
-
- Coxa Plana
- Osteochondrosis Deformans Juvenilis
- Capital Femoral Epiphysis
- Perthes Disease
- Pseudocarolgia
- Quiet Hip
-
- Information on the following diseases can be found in the Related
- Disorders section of this report:
-
- Ankylosing Spondylitis
-
- 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.
-
- 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.
-
- Symptoms
-
- Legg-Calve-Perthes symptoms may appear without warning. Onset is usually
- between the ages of 6 and 12 years with mild aching in the hip followed by
- inability to move the affected leg normally. Pain in the hip may eventually
- become more intense and muscle spasms can develop. While the pain usually
- resolves itself, the bone may become shorter than normal, causing a
- noticeable limp.
-
- Causes
-
- Legg-Calve-Perthes is thought to be a genetic disorder inherited through
- autosomal dominant genes. (Human traits including the classic genetic
- diseases, are the product of the interaction of two genes for that condition,
- one received from the father and one from the mother. In dominant disorders,
- a single copy of the disease gene (received from either the mother or father)
- will be expressed "dominating" the normal gene and resulting in appearance of
- the disease. The risk of transmitting the disorder from affected parent to
- offspring is 50% for each pregnancy regardless of the sex of the resulting
- child.) Legg-Calve-Perthes is characterized by atrophy of the hip joint and
- shortening of the long bone in the leg from the hip to knee (femur). Damage
- to the developing bone is caused by reduced blood supply to the joint part of
- the femur. The bone does not grow at a normal rate.
-
- Affected Population
-
- Legg-Calve-Perthes Syndrome is a rare bone disorder affecting males more
- often than females.
-
- Related Disorders
-
- Symptoms of the following disorders can be similar to those of Legg-Calve-
- Perthes Syndrome. Comparisons may be useful for a differential diagnosis:
-
- Ankylosing Spondylitis usually begins gradually with episodes of low back
- pain, especially in the tailbone and hips (sacroiliac) and lower back
- (lumbar) regions. Pain may occur around the sciatic nerve, radiating from
- the back into the buttock and down a lower extremity. Well-defined morning
- back stiffness often occurs. Symptoms commonly become progressively worse,
- spreading from the low back frequently into the mid-back and occasionally the
- neck. The hips and shoulders may be affected at any stage of the disease.
- Symptoms usually begin between the ages of 10 and 35 years. It is now
- believed that the incidence of the disease is probably equal in males and
- females. However, some sources indicate that males may be affected three
- times as often as females. (For more information on this disorder, choose
- "Ankylosing Spondylitis" as your search term in the Rare Disease Database).
-
- Therapies: Standard
-
- Patients with Legg-Calve-Perthes Syndrome usually find that pain can be
- reduced with anti-inflammatory drugs and analgesics. Other treatment is
- symptomatic and supportive.
-
- Therapies: Investigational
-
- This disease entry is based upon medical information available through
- November 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 Legg-Calve-Perthes Syndrome, please contact:
-
- National Organization for Rare Disorders (NORD)
- P.O. Box 8923
- New Fairfield, CT 06812-1783
- (203) 746-6518
-
- Arthritis Foundation
- 1314 Spring St, NW
- Atlanta, Ga. 30309
- (404) 872-7100
-
- The National Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
- Information Clearinghouse
- Box AMS
- Bethesda, MD 20892
- (301) 495-4484
-
- For genetic information and genetic counseling referrals:
-
- March of Dimes Birth Defects Foundation
- 1275 Mamaroneck Avenue
- White Plains, NY 10605
- (914) 428-7100
-
- Alliance of Genetic Support Groups
- 35 Wisconsin Circle, Suite 440
- Chevy Chase, MD 20815
- (800) 336-GENE
- (301) 652-5553
-
- References
-
- MENDELIAN INHERITANCE IN MAN, 7th ed.: Victor A. McKusick; Johns Hopkins
- University Press, 1986. Pp. 453.
-
- LONG-TERM FOLLOW-UP OF LEGG-CALVE-PERTHES DISEASE; M.P. McAndrew, et al;
- J Bone Joint Surg [AM] (July, 1984, issue 66 (6)). Pp. 860-869.
-
- LEGG-CALVE-PERTHES DISEASE IN A FAMILY: GENETIC OR ENVIRONMENTAL: M.
- O'Sullivan, et al.; Clin Orthop (October, 1985, issue 199). Pp. 179-181.
-
- LESIONS OF THE FEMORAL NECK IN LEGG-PERTHES DISEASE: F.N. Silverman, AJR
- (June, 1985, issue 144 (6)). Pp. 1249-1254.
-
-