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by Drs.
Aaron Walfish & Allen Sapadin
(from
Volume 6, Issue 3, 1998)
The
gastrointestinal (GI) manifestations of PXE are thought to
result from the calcification of elastic tissue in the
gastrointestinal arterial wall which can lead to thinning,
aneurysm formation, and rupture. Hemorrhage, specifically
in the stomach, is the most common GI presentation in
patients with PXE, although jejunal and rectal bleeding have
also been described. Although most cases of GI hemorrhage
occur between the ages of 20 and 30 years, bleeding has also
been seen in pediatric patients under age 10.
GI bleeding develops in approximately 13% of patients with
PXE. There is an increased incidence during pregnancy and
it frequently occurs before the eye and skin changes have
fully emerged. Patients are predisposed to recurrent
episodes of acute GI hemorrhage due to continued elastic
fiber deterioration in arterial walls and possibly because
of failure of vascular constriction. Frequently the
bleeding is not serious and stops spontaneously so that the
problem can go undetected. Occasionally when severe stomach
bleeding does occur endoscopic or even surgical intervention
may become necessary.
Although severe bleeding is rare in pregnant patients, it is
still important for PXE patients to be aware of this risk.
The obstetrician who is following the pregnant patient
should periodically perform stool examinations for the
detection of occult blood.
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