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$Pretitle{}
$Title{Gordon Syndrome}
$Subject{Gordon Syndrome Arthrogryposis Multiplex Congenita, Distal, Type IIA
Distal Arthrogryposis, Type IIA Camptodactyly-Cleft Palate-Clubfoot
Arthrogryposis Multiplex Congenita}
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Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.

507:
Gordon Syndrome

** IMPORTANT **
It is possible the main title of the article (Gordon Syndrome) is not the
name you expected.  Please check the SYNONYMS listing on the next page to
find alternate names and disorder subdivisions covered by this article.

Synonyms

     Arthrogryposis Multiplex Congenita, Distal, Type IIA
     Distal Arthrogryposis, Type IIA
     Camptodactyly-Cleft Palate-Clubfoot

Information on the following disease may be found in the Related
Disorders section of this report:

     Arthrogryposis Multiplex Congenita

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.

Gordon Syndrome belongs to a group of genetic musculoskeletal disorders
called the Distal Arthrogryposes.  This disorder is characterized by permanent
flexion of one or more fingers (camptodactyly), a cleft palate, and clubfeet.
Other developmental abnormalities may also occur.

Symptoms

Gordon Syndrome is characterized by one or two permanently bent (flexed)
fingers, a cleft palate and clubfeet.  During pregnancy, a fetus with this
disorder usually has limited movement.  The intestine sometimes protrudes
through the navel at birth (omphalocele).  The skin between fingers grows
together (cutaneous syndactyly) and there are abnormalities in the
fingerprints (dermatoglyphics).  Fertility of adults with this disorder can
be diminished or be absent during later life.

Causes

Gordon Syndrome is 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.)

Affected Population

Gordon Syndrome is a very rare disorder beginning before birth that affects
females and males in equal numbers.

Related Disorders

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

Arthrogryposis Multiplex Congenita is a congenital disorder characterized
by reduced mobility of multiple joints at birth due to proliferation of
fibrous tissue (fibrous ankylosis).  (For more information on this disorder,
choose "Arthrogryposis" as your search term in the Rare Disease Database.)

Therapies:  Standard

Gordon Syndrome can be diagnosed before birth.  Abnormalities in babies born
with this disorder can often be corrected through surgery and physical
therapy.

Therapies:  Investigational

This disease entry is based upon medical information available through June
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 Gordon Syndrome, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     Forward Face
     560 First Ave.
     New York, NY  10016
     (212) 263-5205
     (800) 422-FACE

     AVENUES, a National Support Group for Arthrogryposis
     P.O. Box 5192
     Sonora, CA  95370-5192
     (209) 533-1468

     The National Arthritis and Musculoskeletal and Skin Diseases Information
     Clearinghouse
     Box AMS
     Bethesda, MD  20892
     (301) 495-4484

For genetic information and genetic counseling referrals, please contact:

     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

This report in the Rare Disease Database is based on outlines prepared by
medical and dental students (1984-1986) at the Medical College of Virginia
for their course in human genetics.

THREE DISTINCT TYPES OF X-LINKED ARTHROGRYPOSIS SEEN IN 6 FAMILIES:  J.G.
Hall, et al.;  Clin Genet (February 1982:  issue 21(2)).  Pp. 81-97.

THE GORDON SYNDROME:  AUTOSOMAL DOMINANT CLEFT PALATE, CAMPTODACTYLY, AND
CLUB FEET:  M. Robinow, et al.;  American Journal Med Genet (1981:  issue
9(2)).  Pp. 139-146.