Medical Alert: Stomach Problems Can Signal
Serious Illness
Previously published in The Gathered View,
March-April 1998
We have recently recognized and reported* an
important medical condition in individuals with Prader-Willi syndrome which
families and other care providers should know more about. Although the condition
is not common in individuals with PWS, it is much more common in these
individuals than in anyone else. It is important to recognize the condition
because it can cause severe medical problems when diagnosis and treatment are
delayed. The condition can be successfully managed, however, when recognized in
a timely fashion.
We have called the condition acute idiopathic
gastric dilatation. The condition often begins suddenly in individuals
in their 20s or 30s. There is generally no known cause. The first symptoms of
illness are vague central abdominal discomfort or pain and vomiting. Bloating of
the abdomen, caused by swelling or distention of the stomach, may also appear at
this time. The person's temperature may also begin to become elevated at this
point In addition, the individual often begins to look and feels quite ill.
Individuals in whom these symptoms appear should
receive immediate medical attention:
A simple X-ray or CT scan of the abdomen should be taken to look for
abdominal distentioa If abdominal distention is present and the individual has
pain but is relatively well appearing, a test called an endoscopy should next be
performed to test the person's stomach lining for signs of inflammation. If the
individual has distention on X-ray and is quite ill, emergency surgery might be
necessary to more closely examine the person's stomach for signs of inflammation
and necrosis [death or decay] of the tissue lining the stomach wall. When severe
distention and necrosis is present, treatment consists of surgical removal of a
significant portion of the stomach.
Anyone who has knowledge of other individuals who have had severe stomach
problems or would like more information can get in touch with PWSA (USA)
1-800-926-4797
* Wharton RH et al. (1997) Acute idiopathic gastric dilation with gastric
necrosis in individuals with Prader-Willi syndrome. American Journal of
Medical Genetics, Dec. 31; Vol. 73(4): page 437-441.
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