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$Unique_ID{BRK03634}
$Pretitle{}
$Title{Crohn's Disease}
$Subject{Crohn's Disease Regional Enteritis Ileitis Granulomatous Colitis CD
Granulomatous Ileitis Ileocolitis Gastritis Jejunitis Jejunoileitis
Enterocolitis Duodenitis Ulcerative Colitis Primary Sclerosing Choloangitis
Chronic Erosive Gastritis Glucose-Galactose Malabsorption Irritable Bowel
Syndrome}
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Copyright (C) 1876, 1988, 1990, 1991, 1993 National Organization for Rare
Disorders, Inc.

58:
Crohn's Disease

** IMPORTANT **
It is possible that the main title of the article (Crohn's Disease) is
not the name you expected.  Please check the SYNONYMS listing to find the
alternate name and disorder subdivisions covered by this article.

Synonyms

     Regional Enteritis
     Ileitis
     Granulomatous Colitis
     CD

Disorder Subdivisions:

     Granulomatous Ileitis
     Ileocolitis
     Gastritis
     Jejunitis
     Jejunoileitis
     Enterocolitis
     Duodenitis

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

     Ulcerative Colitis
     Primary Sclerosing Choloangitis
     Chronic Erosive Gastritis
     Glucose-Galactose Malabsorption
     Irritable Bowel Syndrome

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.


Crohn's Disease is an inflammatory bowel disease characterized by severe,
chronic inflammation of the intestinal wall or any portion of the
gastrointestinal tract.  The lower portion of the small intestine (ileum) and
the rectum are most commonly affected by this disorder.  The symptoms of
Crohn's Disease can be difficult to manage and diagnosis is often delayed.

Symptoms

Crohn's Disease typically affects the lower portion of the small intestine
(ileum) and/or the colon, especially the right colon.  Occasionally,
inflammation may occur in the middle and lower portions of the small
intestine (jeunoileitis).  In some cases, there is inflammation of the
membranes that line the mouth (mucous), the esophagus, and/or stomach.

The symptoms of Crohn's Disease may begin abruptly or appear slowly over
a long period of time.  Symptoms that may develop over time include nausea,
vomiting, fever, night sweats, loss of appetite, a general feeling of
weakness (malaise), waves of abdominal pain and discomfort, diarrhea and/or
bleeding from the rectum.  Weight loss is common in people with Crohn's
Disease.

Acute attacks of Crohn's Disease may cause fever, elevated white blood
cells counts, and/or severe pain in the lower right abdomen.  These symptoms
are frequently confused with appendicitis.

Crohn's Disease may cause lesions (pathological changes) in the
intestinal wall and the surrounding lymph nodes.  Abscesses in the anorectal
area may occur before the appearance of other symptoms.  Grooves on the inner
surface of the intestines (fissures) may also occur.  These may feel like a
solid mass in the abdomen and when the mucosal lining of the intestines
becomes thickened, it may feel like cobblestones.  Deep open abscesses
(fistulas), scarring, and some degree of intestinal obstruction may occur as
a result of chronic inflammation of the intestine.  In some cases, fistulas
and abscesses may create an opening through the intestinal wall and result in
infection by the bacteria that occur naturally in the intestines
(septicemia).  Massive, abnormal enlargement of the colon (toxic megacolon)
is a serious complication of Crohn's Disease and may result in intestinal
bleeding into the abdomen and septicemia.

When Crohn's Disease causes intestinal obstruction, the symptoms may
include pain, constipation, swelling of the abdomen, and/or vomiting.  This
may be due to the accumulation of fluid (edema) in the intestines or
thickening of the muscosal layers of the intestinal walls.  Inflammation and
obstruction may occur together and can impair digestion and the absorption of
food and may lead to malnutrition.

Crohn's Disease rarely occurs in children, and is characterized by
failure to thrive, fever, and/or abnormally low levels of hemoglobin in the
blood (anemia).  Children may also experience joint pain and stiffness
(arthritis).  Sexual development is often delayed.  Initially, children with
Crohn's Disease may not experience diarrhea or abdominal pain.

People with Crohn's Disease may have anemia, abnormally low levels of
albumin in the blood (hypoalbuminism), abnormally high white blood cell
counts, and/or a deficiency of vitamin B-12.  Other laboratory findings may
include abnormally low blood levels of sodium, potassium, calcium, and/or
magnesium.

Crohn's Disease may also have symptoms that are not related to intestinal
dysfunction.  These may include joint pain, or skin and eye problems.  A
fatty like substance (amyloid) may accumulate in various parts of the body.
Blood circulation may be impaired by abnormally thick blood, dehydration,
and/or lack of movement or exercise.  In some cases, arthritis may occur
resulting in swollen and painful joints.

In rare cases of Crohn's Disease, liver function may be impaired.  These
complications may include a fatty liver, inflammation of the liver and the
bile ducts, chronic hepatitis, and/or cirrhosis.   Kidney stones may also
occur.

The diagnosis of Crohn's Disease may be difficult because of the wide
range of symptoms.  Diagnosis may be made by a variety of procedures
including x-rays, the examination of the colon with special instruments
(sigmoidoscopy), and fiber-optic examination of the colon (colonoscopy).

Causes

The exact cause of Crohn's Disease is unknown.  The clustering of this
disease within some families may suggest a genetic or environmental
influence.

Scientists believe 10 to 30 percent of Crohn's Disease patients may have
inherited the disorder, but its genetic inheritance pattern is still unknown.

A study on Crohn's Disease and Ulcerative Colitis (Denmark, 1991)
suggested that relatives of people with either of these diseases are at an
increased risk for developing the disease.  The risk factor may be as great
as a ten fold increase.  The study also suggested that Crohn's Disease and
Ulcerative Colitis may be inherited.

Some of the complications of Crohn's Disease suggest that immunological
agents may be responsible in part for the disease.  Other research suggests
that infectious agents may play a role in causing Crohn's Disease.  Recent
studies have suggested that emotional conditions do not cause this disease,
although the psychological effects of the disease are recognized.

Affected Population

Crohn's Disease affects males and females in equal numbers.  People of Jewish
ancestry are affected by this disorder 3 to 6 times more frequently than
others.  Crohn's Disease typically affects people between the ages of 15 to
55 years.  This disorder may also occur in young children and the elderly.
Twenty-five percent of people with Crohn's Disease are likely to have a
relative with either Crohn's disease or Ulcerative Colitis.

Related Disorders

Symptoms of the following disorders can be similar to those of Crohn's
Disease.  Comparisons may be useful for a differential diagnosis:

Ulcerative Colitis is an acute inflammatory bowel disease characterized
by diarrhea and blood in the stools because of multiple, irregular
ulcerations of the bowel.  The initial symptoms of this disorder may include
a general feeling of weakness (malaise) and fatigue.  There may be abdominal
discomfort, along with a change in the frequency and consistency of stools.
Other symptoms may include abdominal pain, cramping, and urgency (tenesmus).
Weight loss and a decrease in appetite are also associated with Ulcerative
Colitis.  (For more information on this disorder, choose "Ulcerative Colitis"
as your search term in the Rare Disease Database.)

Primary Sclerosing Cholangitis is a rare collagen disorder involving
inflammation and blockage of the bile duct, liver ducts, and gallbladder.
Symptoms of this disorder include abdominal pain, loss of appetite, nausea,
vomiting, and/or weight loss.  Later symptoms may include a yellow
discoloration to the skin (jaundice), fever, chills, and/or itching of the
skin.  Bacterial infections resulting from Ulcerative Colitis, Crohn's
Disease, and/or Vasculitis may be associated with bile duct blockages of
Primary Sclerosing Cholangitis.  (For more information on this disorder,
choose "Primary Sclerosing Cholangitis" as your search term in the Rare
Disease Database.)

Chronic Erosive Gastritis is an inflammatory disorder characterized by
multiple lesions in the mucosal lining of the stomach.  Symptoms of this
disorder may include a burning or heavy feeling in the stomach, mild nausea,
vomiting, loss of appetite and general weakness.  In severe cases of Chronic
Erosive Gastritis there may be bleeding from the stomach that can result in
anemia.  (For more information on this disorder, choose "Chronic Erosive
Gastritis" as your search term in the Rare Disease Database.)

Glucose-Galactose Malabsorption (carbohydrate intolerance) is a rare
inherited disorder characterized by the inability of the small intestine to
transport and absorb glucose and galactose.  The symptoms of this disorder in
children may include diarrhea, dehydration, and failure to gain weight.  In
adults, symptoms of this disorder may include bloating, nausea, diarrhea,
abdominal cramps, rumbling sounds caused by gas in the intestine
(borborygmi), and/or excessive urination.  (For more information on this
disorder, choose "Glucose-Galactose Malabsorption" as your search term in the
Rare Disease Database.)

Irritable Bowel Syndrome, also known as Spastic Colon, is a common
digestive disorder that involves both the small intestine and the large
bowel.  This disorder is characterized by abdominal pain, constipation,
bloating, nausea, headache, and/or diarrhea.  The spastic colon type of this
syndrome is characterized by variable bowel movements and abdominal pain that
is associated with periodic constipation or diarrhea.  Those patients with
Irritable Bowel Syndrome who have painless diarrhea may experience an urgent
need to defecate upon arising.  (For more information on this disorder,
choose "Irritable Bowel Syndrome" as your search term in the Rare Disease
Database.)

Other digestive diseases with similar symptoms include infectious
diseases such as Yersinia Enterocolitica Infection, Amebiasis, chronic fungal
bowel infections, Intestinal Tuberculosis, Pseudomembranous Colitis that is
caused by excessive use of antibiotics, and certain venereal diseases.
Ischemic Colitis and certain cancers such as abdominal lymphoma may also have
symptoms that are similar to those of Crohn's Disease.

Therapies:  Standard

The treatment of Crohn's disease is aimed at relieving the symptoms and to
halt or slow the inflammation and destruction of the tissues.  Sulfasalazine
is a drug often used in the extended treatment of low grade intestinal
inflammation.  Acute inflammatory episodes respond to corticosteroid drugs;
however, these may provoke many side effects if used over long periods of
time.

People with Crohn's Disease may also be treated with diphenoxylate,
loperamide, opium tincture, or codeine to help relieve abdominal cramps and
diarrhea.  Hydrophilic mucilloids (methylcellulose or phyllium preparations)
may help prevent anal irritation by increasing stool firmness.  Broad
spectrum antibiotics that are effective against certain bacteria may be
helpful in reducing the symptoms of active Crohn's Disease but may be more
beneficial for those patients who have intestinal abscesses or fistulas.

Metronidazole is a drug that has been shown to be beneficial in the
treatment of Crohn's Disease.  This drug may reduce fever and diarrhea, and
relieve abdominal pain and tenderness.  Metronidazole is used primarily in
patients who do not respond to Sulfasalazine.

Corticosteroids may be useful in the treatment of acute stages of Crohn's
Disease.  These drugs (i.e., prednisone and hydrocortisone) may help improve
fever and relieve diarrhea.  Corticosteroids should be avoided when obvious
infections are present because they impair the function of the immune system.

Nutrition is an important consideration for people with Crohn's Disease,
especially in children and when obstructions and fistulas are present.
Vitamins, particularly B-12, and minerals must be added to the daily diet.
In cases of Crohn's Disease where there is impairment in the ability to
digest fats, people should maintain a low fat diet.  A medically prescribed
liquid diet (elemental supplementation) may be useful when eating is
difficult.  The ingestion of greater than normal amounts of food
(hyperalimentation) may help prepare patients for surgery, and to supplement
their diets after surgery.

Certain people with Crohn's Disease may be considered for surgery.  These
include those who have not received any relief for their symptoms
(intractable disease) through the use of pharmaceuticals, and cases of
intestinal obstruction such as fistula and/or abscess.  This disease recurs
in approximately 50 percent of cases after five years.  In half the cases
that recur, further surgery is eventually required.

Immunosuppressive drugs such as metronidazole, azathioprine, and 6-
mercaptopurine are also used to treat Crohn's Disease, especially when the
disease is limited to the colon.  Frequently these drugs to not produce an
improvement of symptoms for 3 to 6 months.  These drugs, when used along with
metronidazole, may promote the closure of fissures in patients with Crohn's
Disease.

Therapies:  Investigational

Studies are being conducted in the use of gammaglobulin as a treatment for
Crohn's Disease.  Further investigation is needed to determine the long-term
safety and effectiveness of this treatment.

In 1990 Dr. Sally Schuette of the University of Chicago, Chicago, IL, is
studying Mg Diglycin Chelate in Crohn's patients.  Dr. Walter Raymond Thayer
of Rhode Island Hospital, Providence, RI, is studying Rifabutin and
Streptomycin in severe Crohn's Disease.

Cyclosporine (Sandimmune) and methotrexate are also being investigated
for use in the treatment of Crohn's Disease, especially in those people who
do not respond to other therapies.  These are immunosuppressive drugs that are
normally used by organ transplant patients.  Further study is required to
determine the safety and long term effectiveness of these treatments.

This disease entry is based upon medical information available through
May 1993.  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 Crohn's Disease, please contact:

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

     Crohn's and Colitis Foundation of America, Inc.
     444 Park Ave. South
     New York, NY  10016
     [phone]

     National Digestive Diseases Information Clearinghouse
     Box NDDIC
     Bethesda, MD  20892
     [phone]

     United Ostomy Association, Inc.
     36 Executive Park, Suite 120
     Irvine, CA  97214
     [phone]

For Genetic Information and Genetic Counseling Referrals:

     March of Dimes Birth Defects Foundation
     1275 Mamaroneck Ave.
     White Plains, NY  10605
     (914)-428-7100

     Alliance of Genetic Support Groups
     35 Wisconsin Circle, Suite 440
     Chevy Chase, MD  20815
     (800) 336-GENE
     [phone]

References

MENDELIAN INHERITANCE IN MAN, 10th Ed.:  Victor A. McKusick, Editor:  Johns
Hopkins University Press, 1992.  Pp. 1675.

GASTROINTESTINAL DISEASE, 4TH ED.:  Marvin H. Sleisenger and John S.
Fordtran Editors; W.B. Saunders Co., 1989.  Pp. 1327-1358.

CECIL TEXTBOOK OF MEDICINE, 19th Ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1990.  Pp. 702-708.

THE MERCK MANUAL, 16th Ed.:  Robert Berkow Ed.; Merck Research
Laboratories, 1992.  Pp. 830-832.

BIRTH DEFECTS ENCYCLOPEDIA, Mary Louise Buyse, M.D., Editor-In-Chief;
Blackwell Scientific Publications, 1990.  Pp. 970-972.

FAMILIAL OCCURRENCE OF INFLAMMATORY BOWEL DISEASE, M. Orholm, MD, N Eng J
Med (January 10, 1991, issue 324 (2)).  Pp. 84-88.

DRUG THERAPY FOR INFLAMMATORY BOWEL DISEASE:  PART II.  F.V. Linn; Am J
Surg (Aug 1992; 164(2)).  Pp. 178-185.

MEDICAL TREATMENT OF INFLAMMATORY BOWEL DISEASE.  F. Shannon; Annu Rev Med
(1992:43).  Pp. 125-33.

USE OF AZATHIOPRINE OR 6-MERCAPTOPURINE IN THE TREATMENT OF CROHN'S
DISEASE.  J.J. O'Brien; Gastroenterology (July 1991: 101(1)).  Pp. 39-46.