home
***
CD-ROM
|
disk
|
FTP
|
other
***
search
/
CD-ROM Today (UK) (Spanish) 15
/
CDRT.iso
/
dp
/
0398
/
03982.txt
< prev
next >
Wrap
Text File
|
1994-01-17
|
6KB
|
156 lines
$Unique_ID{BRK03982}
$Pretitle{}
$Title{Mediterranean Fever, Familial}
$Subject{Mediterranean Fever, Familial Siegel-Cattan-Mamou Syndrome Armenian
Syndrome Benign Paroxysmal Peritonitis Familial Paroxysmal Polyserositis
Mediterranean Fever Periodic Amyloid Syndrome Periodic Peritonitis Syndrome
Recurrent Polyserositis Reimann's Syndrome Reimann Periodic Disease }
$Volume{}
$Log{}
Copyright (C) 1986, 1990, 1992 National Organization for Rare Disorders,
Inc.
127:
Mediterranean Fever, Familial
** IMPORTANT **
It is possible that the main title of the article (Familial Mediterranean
Fever) is not the name you expected. Please check the SYNONYM listing to
find the alternate names and disorder subdivisions covered by this article.
Synonyms
Siegel-Cattan-Mamou Syndrome
Armenian Syndrome
Benign Paroxysmal Peritonitis
Familial Paroxysmal Polyserositis
Mediterranean Fever
Periodic Amyloid Syndrome
Periodic Peritonitis Syndrome
Recurrent Polyserositis
Reimann's Syndrome
Reimann Periodic 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.
Familial mediterranean fever (FMF) is a hereditary disorder characterized
by recurrent bouts of fever and inflammation of the serous membranes, which
line the inside of the body cavity and the various organs that protrude into
it. FMF occurs in persons of Armenian, Arabic, or Sephardic Jewish ancestry,
and affects males more often than females. The disorder has a good prognosis
in the absence of complications. The causes of Familial Mediterranean Fever
are not known.
Symptoms
The first symptoms of Familial Mediterranean Fever usually appear between the
ages of five and fifteen, although onset occasionally occurs in young
adulthood or early middle age. Attacks recur at intervals of weeks or
months, and usually last twenty-four to forty-eight hours, although sometimes
they can last as long as one week. In women, attacks may correspond with
menstrual periods.
Fever, sometimes severe abdominal and stabbing chest pain (due to
inflammation of the pleural and peritoneal membranes), and occasionally,
arthritis and skin lesions, characterize the attacks. The patient may feel
unable to breathe deeply because of pain (pleurisy). Pain occurs in the
larger joints and lasts 2 to 3 days. Skin lesions consist of painful,
reddish, swollen areas, usually on the lower legs.
A major complication of Familial Mediterranean Fever is amyloidosis
particularly affecting the kidneys. (For more information on Amyloidosis,
choose "amyloidosis" as your search term in the Rare Disease Database.) This
can lead to kidney failure. In the United States, drug addiction is an
important complication, usually resulting from the use of narcotics to
alleviate pain during attacks.
Causes
Familial Mediterranean Fever is inherited through an autosomal recessive
mechanism. (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 recessive disorders, the condition
does not appear unless a person inherits the same defective gene from each
parent. If one receives one normal gene and one gene for the disease, the
person will be a carrier for the disease, but usually will show no symptoms.
The risk of transmitting the disease to the children of a couple, both of
whom are carriers for a recessive disorder, is twenty-five percent. Fifty
percent of their children will be carriers, but healthy as described above.
Twenty-five percent of their children will receive both normal genes, one
from each parent and will be genetically normal.)
Scientists have located the gene that results in the development of
Familial Mediterranean Fever in non-Ashkanazi Jews on the short arm of
chromosome 16.
The biochemical or structural defect in this disease is not known.
Inherited metabolic or endocrine defects are possibilities.
Affected Population
Familial Mediterranean Fever preferentially affects persons of Armenian,
Arabic, or Sephardic Jewish ancestry. More males than females are affected.
Therapies: Standard
Colchicine prevents attacks for reasons that are not understood. In larger
quantities, it stops attacks that are already in progress. Narcotics should
not be used routinely to control pain because of the possibility of drug
addiction. Surgery is of no benefit. When amyloidosis has destroyed the
kidneys, renal transplantation or renal dialysis may be necessary.
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 Familial Mediterranean Fever, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
National Institute of Diabetes, Digestive & Kidney Diseases Information
Clearinghouse
Box NDIC
Bethesda, MD 20892
(301) 468-2162
For information on genetics 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
CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.: W. B. Saunders Co., 1988. Pp. 147, 795, 1196-9.
THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme
Research Laboratories, 1987. P. 255.