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$Unique_ID{BRK03880}
$Pretitle{}
$Title{Intestinal Pseudoobstruction}
$Subject{Intestinal Pseudoobstruction Pseudointestinal Obstruction Syndrome
Hypomotility Disorder CIIP Congenital Short Bowel Syndrome Pseudoobstructive
Syndrome Chronic Idiopathic Intestinal Pseudoobstruction Irritable Bowel
Syndrome Megacystis Microcolon Intestinal Hypoperistalsis Syndrome Paralytic
Ileus Acute Colonic Pseudoobstruction (Ogilvie's Syndrome) Intestinal
Obstruction Scleroderma Myxedema Amyloidosis Muscular Dystrophy Hypokalemia
Renal Failure Diabetes Mellitus Anticholinergic Toxicity Opiate Toxicity }
$Volume{}
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Copyright (C) 1987, 1988, 1989 National Organization for Rare Disorders,
Inc.

452:
Intestinal Pseudoobstruction

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

Synonyms

     Pseudointestinal Obstruction Syndrome
     Hypomotility Disorder
     CIIP
     Congenital Short Bowel Syndrome
     Pseudoobstructive Syndrome
     Chronic Idiopathic Intestinal Pseudoobstruction

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

     Irritable Bowel Syndrome
     Megacystis Microcolon Intestinal Hypoperistalsis Syndrome
     Paralytic Ileus
     Acute Colonic Pseudoobstruction (Ogilvie's Syndrome)
     Intestinal Obstruction
     Scleroderma
     Myxedema
     Amyloidosis
     Muscular Dystrophy
     Hypokalemia
     Renal Failure
     Diabetes Mellitus
     Anticholinergic Toxicity
     Opiate Toxicity

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.


Intestinal Pseudoobstruction is a digestive disorder which may be present
at birth.  The intestinal walls are unable to contract normally in wave-like
(peristaltic) motions (hypomotility).  This condition resembles a true
obstruction, but no such blockage exists.  Abdominal pain, vomiting,
diarrhea, constipation, malabsorption of nutrients leading to weight loss
and/or failure to thrive, enlargement of various parts of the small intestine
or bowel also occur.

Symptoms

Intestinal Pseudoobstruction is characterized by a lack of wave-like motions
(peristalsis) of the intestinal walls which normally moves food through the
digestive tract.  Although symptoms appear to be caused by an intestinal
obstruction, no such blockage exists.  Enlargement of parts of the
gastrointestinal tract may be accompanied by pain, and accumulations of air
and/or fluid.  Additionally, malabsorption of nutrients can lead to diarrhea
or constipation, and eventually weight loss.  Babies with this disorder may
fail to thrive due to lack of nutrients.

Intestinal Pseudoobstruction is characterized primarily by "failure to
thrive" during infancy.  The intestine may be abnormally formed and the
involuntary wave-like contractions which propel food through the digestive
system (peristalsis) may be lacking.  Intermittent pain, a swollen abdomen
(abdominal distension), and forceful vomiting often occur as the esophagus
and small intestine dilate.  In some cases, central nervous system
deterioration may occur, possibly resulting in impaired walking coordination,
and an abnormal dilation of the pupils in the eyes.  Additionally, speech
disturbances, absent deep tendon reflexes, poor muscle sensation, and lack of
sweating during warm temperatures may also develop.

Causes

Intestinal Pseudoobstruction may occur sporadically with no known cause or as
a complication of various other disorders.  It may also be inherited as an
autosomal dominant trait.  (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.)

Disorders which may precede development of this disorder include
Scleroderma, Myxedema, Amyloidosis, Muscular Dystrophy, Hypokalemia, Chronic
Renal Failure, or Diabetes Mellitus.  Drug toxicity as a result of
anticholinergic drugs and opiate narcotics may also cause Intestinal
Pseudoobstruction.  Symptoms are thought to be caused by abnormalities of the
nerves in the intestinal wall which deter normal wave-like contractions of
the intestine.

Affected Population

Intestinal Pseudoobstruction is a rare disorder occurring worldwide which
affects males and females in equal numbers.

Related Disorders

Symptoms of the following disorder may be similar to those of Chronic
Idiopathic Intestinal Pseudoobstruction (CIIP).  Comparisons may be useful
for a differential diagnosis:

Irritable Bowel Syndrome, also known as spastic colon or mucous colitis,
is a digestive disorder which involves both the small intestine and the large
bowel.  It is characterized by varying degrees of abdominal pain,
constipation, diarrhea, and an apparent reaction to stress in susceptible
individuals.  However, this disorder usually appears in adults, and rarely in
infants.   (For more information on this disorder, choose "Irritable Bowel"
as your search term in the Rare Disease Database).

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is
characterized by a massively enlarged bladder (leading to displacement of
other internal organs), a smaller than normal colon, and lack of the normal
wave-like motions (peristalsis) of the intestines which aid absorption and
digestion of nutrients.  Bowel and bladder dysfunction can be treated with
catheterization and anticholinergic drugs.  Although there is no cure,
management of the disorder can avoid complications and lead to temporary
asymptomatic periods.  In severe cases, surgical removal of the colon and/or
ileum, catheterization, and parenteral or enteral nutrition may be
recommended.  Parenteral feeding is being fed through any pathway that does
not involve the gastrointestinal tract or lungs, i.e., feeding through a tube
directly into veins (intravenous), beneath the skin (subcutaneous), into a
muscle (intramuscular), or into the bone marrow of the spinal cord
(intramedullary).  Enteral feeding involves being fed through a tube directly
into the gastrointestinal tract.

Paralytic Ileus is caused by paralysis of the bowel wall.  The paralysis
is usually a result of localized or generalized inflammation of the
membranous sac surrounding the abdominal cavity known as the peritoneum
(peritonitis), or shock.  Symptoms are similar to those of Intestinal
Pseudoobstruction.

Acute Colonic Pseudoobstruction (Ogilvie's Syndrome) is characterized by lack
of wave-like motions (peristalsis) of the colon section of the large
intestine rather than the areas of the intestine affected by other forms of
pseudoobstruction.  Symptoms are similar to those of Intestinal
Pseudoobstruction although treatment is different.  Decompression of the
enlarged colon with the use of a colonoscopic overtube is generally an
effective treatment.

Intestinal Obstruction is a general term denoting any mechanical blockage
of the passage of food through the intestinal tract which is detectable upon
physical examination.  Symptoms of this condition include lack of absorption
of nutrients, diarrhea or constipation, and eventually, failure to thrive
and/or weight loss.  The obstruction can be a tumor, abscess or other mass
that blocks the intestines.

The following disorders may precede the development of Chronic Idiopathic
Intestinal Pseudoobstruction.  They can be useful in identifying an
underlying cause of some forms of this disorder:

Scleroderma is characterized by thickening and hardening of the skin and
fibrous tissue, which may eventually affect the internal organs.  This
disorder is also known as Progressive Systemic Sclerosis.  (For more
information on this disorder, choose "Scleroderma" as your search term in the
Rare Disease Database).

Myxedema is a combination of hypothyroidism manifested by a relatively
hard swelling (edema) of an inner layer of skin, dryness and loss of hair.
Additionally, body temperature may be below normal.  Hoarseness, muscle
weakness, and slow return of a muscle to it's normal position after a tendon
jerk may occur.  This disorder can be caused by removal of the thyroid or
loss of functioning of the thyroid gland.

Amyloidosis results from an excess accumulation of amyloid, a
glycoprotein, in almost any organ system.  Systemic amyloidosis occurs in
three forms distinguished by certain biochemical and pathological
characteristics.  Primary Amyloidosis arises either independently of other
disease, or in association with multiple myeloma.  (For more information on
this disorder, choose "Amyloidosis" as your search term in the Rare Disease
Database).

Batten Turner Muscular Dystrophy is a benign congenital form of muscular
dystrophy characterized by frequent stumbling and falling during early
childhood.  Unlike the Duchenne variety of muscular dystrophy which typically
is present in young boys, Batten Turner Syndrome affects both sexes.  (For
more information on this disorder, choose "Muscular Dystrophy" as your search
term in the Rare Disease Database).

Hypokalemia is characterized by an abnormally small concentration of
potassium in the circulating blood.   This condition can occur in conjunction
with Familial Periodic Paralysis and in potassium depletion due to excessive
loss from the gastrointestinal tract or kidneys.  Muscle weakness may result
from this deficiency.  (For more information on this disorder, choose
"Hypokalemia" as your search term in the Rare Disease Database).

Renal (kidney) Failure occurs as a result of a number of conditions or
disorders wherein the kidney function deteriorates to an extreme degree.
This has been known to cause some cases of Intestinal Pseudoobstruction.

Diabetes Mellitus (Insulin-dependent Diabetes) is a disorder in which the
body does not produce enough insulin and is, therefore, unable to convert
nutrients into the energy necessary for daily activity.  The disorder affects
females and males approximately equally.  Although the causes of Insulin-
dependent Diabetes are not known, genetic factors seem to play a role.  Some
cases of Intestinal Pseudoobstruction can be a complication of Diabetes
Mellitus.  (For more information on this disorder, choose "Diabetes" as your
search term in the Rare Disease Database).

Anticholinergic Toxicity is an adverse reaction to a drug which would
normally be administered to deter action of the cholinergic nerve fibers.
Intestinal Pseudoobstruction occurs when these nerve fibers in the intestinal
walls somehow become inactive and do not facilitate the wavelike motion
(peristalsis) which normally moves nutrients through the intestinal tract.
Obviously, if anticholinergic drugs are administered to an Intestinal
Pseudoobstruction patient, further problems would result.

Opiate Toxicity is an adverse reaction to pain relieving drugs derived
from the opium poppy such as morphine and similar compounds.  Intestinal
Pseudoobstruction is among the possible adverse side effects of these drugs.

Therapies:  Standard

Treatment for forms of Intestinal Pseudoobstruction which are secondary to
kidney (renal) failure and drug toxicity involve treatment of the underlying
disorder.  Broad spectrum antibiotics can be useful for treating
malabsorption or diarrhea caused by bacterial overgrowth which may occur when
ingested food remains stationary in the intestines.  Metroclopramide (Reglan)
may increase intestinal motility in some cases.  However, overall
effectiveness is limited by side effects.

Oral administration of liquid, low-residue, complete nutrition
preparations may provide and maintain adequate nutrition.  In severe cases,
patients may require long-term parenteral or enteral nutrition.  Parenteral
feeding is being fed through any pathway that does not involve the
gastrointestinal tract or lungs, i.e., feeding through a tube directly into
the veins (intravenous), beneath the skin (subcutaneous), into a muscle
(intramuscular), or into the bone marrow of the spinal cord (intramedullary).
Enteral feeding involves being fed through a tube directly into the
gastrointestinal tract.  Additionally, surgical removal of enlarged loops of
intestine may improve oral nutrition and relieve pain, but the effectiveness
of this measure tends to vary greatly between patients.

Genetic counseling may be of benefit for some patients with the
hereditary form of this disorder and their families.  Other treatment is
symptomatic and supportive.

Therapies:  Investigational

Testing of the Orphan Drug cisapride, which induces motility in the
intestines (peristalsis) in Intestinal Pseudoobstruction is being conducted.
Further testing is required since results have not been fully documented for
all but the most severe cases of Intestinal Pseudoobstruction.  For more
information, physicians can contact:

     Pediatric Gastrointestinal Motility Center
     Dr. Paul Hyman, Chief
     Harbor UCLA Medical Center
     1124 W. Carson St., Trailer C-1
     Torrance, CA  90502

     or

     Janssen Pharmaceutica, Inc.
     40 Kingsbridge Rd.
     Piscataway, NJ  08854

This disease entry is based upon medical information available through
April 1989.  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 Intestinal Pseudoobstruction, please contact:

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

     North American Pediatric Pseudoobstruction Society
     16 Mammmola Way
     Medford, MA  02155
     [phone]

     American Society of Adults with Pseudoobstruction
     19 Carrol Rd.
     Woburn, MA  01801
     [phone]

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

     Frances Harley, M.D.
     Department of Pediatrics, 4-120A, C.S.B.
     University of Alberta, Edmonton, Alberta  TG6 2G3
     Canada
     [phone]

For information on Parenteral or Enteral Nutrition, contact:

     PEN Parent Education Network
     203 Brookfield Dr.
     Straford, WI  54484
     [phone]

For genetic information and genetic counseling referrals, please contact:

     March of Dimes Birth Defects Foundation
     1275 Mamaroneck Avenue
     White Plains, NY  10605
     [phone]

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

References

MENDELIAN INHERITANCE IN MAN, 7th ed.; Victor A. McKusick; Johns Hopkins
University Press, 1986.  Pp.  154-155.

INTERNAL MEDICINE, 2nd Ed.:  Jay H. Stein, ed.-in-chief; Little, Brown
and Co., 1987.  Pp.  1065-1066.

PROBLEMS OF TRACE ELEMENTS AND VITAMINS DURING LONG-TERM PARENTERAL
NUTRITION:  A CASE REPORT OF IDIOPATHIC INTESTINAL PSEUDO-OBSTRUCTION.  H.
Kadowski, et al.; JPEN (May-June 1987, issue 11(3).  Pp. 322-325.

CHRONIC IDIOPATHIC INTESTINAL PSEUDO-OBSTRUCTION CAUSED BY VISCERAL
NEUROPATHY LOCALISED IN THE LEFT COLON:  REPORT OF TWO CASES.  H.  Suzuki, et
al.; Jpn J. Surg (July 1987, issue 17 (4).  Pp. 302-306.

FAMILIAL INTESTINAL PSEUDOOBSTRUCTION DOMINATED BY A PROGRESSIVE NEUROLOGIC
DISEASE AT A YOUNG AGE:  J. Faber, et al.; Gastroenterology (March 1987, issue
92(3)).  Pp. 786-790.

FAMILIAL VISCERAL NEUROPATHY WITH AUTOSOMAL DOMINANT TRANSMISSION:  E.A.
Mayer, et al.; Gastroenterology (December, 1986, issue 91(6)).  Pp. 1528-
1535.

CHRONIC IDIOPATHIC INTESTINAL PSEUDO-OBSTRUCTION:  CLINICAL AND
INTESTINAL MANOMETRIC FINDINGS:  V. Stanghellini, et al.; Gut (January 1987,
issue 28(1)).  Pp. 5-12.