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$Unique_ID{BRK03576}
$Pretitle{}
$Title{Celiac Sprue}
$Subject{Celiac Sprue Celiac disease Nontropical Sprue Gluten Enteropathy
Gee-Herter Disease Gee-Thaysen Disease Huebner-Herter Disease Idiopathic
Steatorrhea}
$Volume{}
$Log{}
Copyright (C) 1986, 1989, 1992 National Organization for Rare Disorders,
Inc.
222:
Celiac Sprue
** IMPORTANT **
It is possible the main title of the article (Celiac Sprue) is not the
name you expected. Please check the SYNONYMS listing to find the alternate
names and disorder subdivisions covered by this article.
Synonyms
Celiac disease
Nontropical Sprue
Gluten Enteropathy
Gee-Herter Disease
Gee-Thaysen Disease
Huebner-Herter Disease
Idiopathic Steatorrhea
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.
Celiac Sprue is a chronic, hereditary, intestinal malabsorption disorder
caused by intolerance to gluten. The illness is characterized by a flat
jejunal (part of the intestine) mucosa. Clinical and/or histologic
improvement of symptoms follow withdrawal of dietary gluten.
Symptoms
Celiac Sprue may be either symptomatic or asymptomatic. Family studies show
that typical mucosal abnormalities can appear in healthy siblings of affected
patients. Genetic markers HLA-B8 and HLA-DW3, have been shown to occur in
80% of persons with Celiac Sprue.
The disease may be manifested for the first time in infancy or adulthood.
Children, from 6 months to 3 years of age, usually show growth failure and
may or may not have diarrhea, projectile vomiting and a bloated abdomen.
The most common clinical symptoms for adults usually include some of the
following:
1. Weight loss
2. Chronic diarrhea
3. Abdominal cramping and bloating
4. Intestinal gas and abdominal distention
5. Muscle wasting.
Appetite is often increased to the point of craving food. Weakness, lack
of energy and fatigue are also common. Other symptoms may include:
1. Changes in the mucous membrane of the mouth and of other tissues due
to a vitamin deficiency (such as a smooth tongue and cracks in the corners of
the mouth
2. Mineral deficiency symptoms such as anemia, muscle cramping
(typically leg cramps from calcium deficiency)
3. Edema due to low blood protein level.
There is a great deal of variation in the combination of clinical
symptoms exhibited by persons with Celiac Sprue. This is thought to be
related to the variation in the amount of intestinal damage and the length of
time nutrient absorption has been abnormal.
A less common problem is Dermatitis herpetiformis, which may be present
in some patients with Celiac Sprue. Small itchy blisters appear on the skin
surface, most commonly on body pressure points such as elbows, knees and
feet.
When the absorptive lining of the small intestine is damaged, fats cannot
be absorbed normally. This results in fatty diarrhea, weight loss and
malabsorption of the fat-soluble vitamins A, D, and K. A decrease of these
vitamins may lead to some of the following problems:
1. Vitamin A deficiency may result in night blindness (follicular
hyperkeratosis)
2. Vitamin D deficiency may cause a softening of the bones
(osteomalacia) and muscle cramps, bone pain, fractures, or tetany.
3. Vitamin K deficiency may lead to decreased blood clotting.
The malabsorption of other nutrients, salt, and water may result in
dehydration, electrolyte depletion, growth retardation, edema, and anemia.
Milk sugar (lactose) intolerance, numbness and tingling in fingers and toes
(peripheral neuropathy), central nervous system and spinal cord lesions can
also occur.
Personality changes are especially common in children with Celiac Sprue;
concentration may become difficult and they may be irritable, cranky, and
have difficulties with mental alertness and memory function. These symptoms
can also occur in adults.
Further complications may include a gradual softening and bending of the
bones (osteomalacia) and loss of bone substance (osteoporosis). Bone
fractures may also occur more easily.
A characteristic symptom of Celiac Sprue involves the normal finger-like
projections (villi) in the intestine. These villi increase the absorptive
surface area of the small intestine. In Celiac Sprue, the villi are
partially or totally absent, and the brush border which normally appears on
the surface of the villi is substantially flattened or reduced.
Causes
Celiac Sprue is a hereditary congenital disorder. Gluten is a protein which
is present in wheat, oats, rye, barley and probably millet. Gliadin which is
a part of gluten cannot be absorbed properly by patients with Celiac Sprue.
This causes physiological deficiencies as well as intestinal abnormalities.
Affected Population
Celiac Sprue affects males and females equally. Although the disorder begins
in infancy, it is sometimes not diagnosed until the patient reaches
adulthood.
Related Disorders
Whipple's Disease or Intestinal Lipodystrophy is an uncommon illness occuring
predominantly in males aged 30 to 60 years. It is characterized by anemia,
skin pigmentation, joint symptoms (joint pain or arthralgia and arthritis),
weight loss, and severe malabsorption.
Tropical Sprue is a disease of unknown cause characterized by
malabsorption, multiple nutritional deficiencies, and abnormalities in the
small bowel mucosa.
(For more information on Whipple's Disease and Tropical Sprue, please
choose Whipple and Tropical Sprue as your search terms in the Rare Disease
Database.)
Celiac Sprue can be differentiated from Whipple's Disease and Tropical
Sprue by the pathological examination of a biopsy from the small intestines
which show flat or absent villi.
Therapies: Standard
Gluten must be completely excluded from the diet of people with Celiac Sprue.
Advice from a dietitian is needed.
Supplementary vitamins, minerals, and agents which improve blood
formation (hematinics) may be prescribed depending upon the degree of the
deficiency. Children, and rarely adults, who are seriously ill when they are
first diagnosed may require a period of intravenous feeding.
A few patients who do not respond adequately at first to gluten
withdrawal may respond to a period of treatment with oral steroids such as
prednisone.
If, after eight weeks of a gluten-free diet the symptoms still persist,
other tests are indicated to see if diseases such as lymphoma or carcinoma
are present.
Therapies: Investigational
This disease entry is based upon medical information available through
January 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 Celiac Sprue, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
Gluten Intolerance Group
P.O. Box 23053
Seattle, WA 98102
(206) 325-6980
National Digestive 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. 742-3.
THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme
Research Laboratories, 1987. Pp. 789, 792, 1832.
CELIAC SPRUE, Trier, Jerry S., N Eng J Med, December 12 1991, (issue 325
(24)). Pp. 1709-1719.