| Hemolytic (HEE-mo-LIT-ik) uremic (yoo-REE-mik)
syndrome is a rare condition affecting mostly children under the age of
10. Hemolytic uremic syndrome (HUS) is characterized by destruction of red
blood cells, damage to the lining of blood vessel walls, and, in severe
cases, kidney failure.
Most cases of HUS occur after an infection in the digestive system
caused by the Escherichia colibacterium found on contaminated food
like meat, dairy products, and juice. The first stages of HUS frequently
present with gastrointestinal symptoms such as abdominal pain, vomiting,
and bloody diarrhea. This stage lasts from 1 to 15 days. Recovery from
this acute colitic phase is the rule. However, more severe problems in the
bowel and colon may develop in some cases. (The most vulnerable and
severely stricken of children with HUS can die during the first acute
stage of the disease--between 5 percent and 10 percent.)
For most children with HUS, the vomiting and diarrhea stop after 2 or 3
days. Even when the gastroenteritis has stopped, the child does not
recover full health, remaining pale, tired, and irritable. The bacteria
originally lodged in the digestive system make toxins that enter the
bloodstream, and these toxins destroy red blood cells. Small, unexplained
bruises or small clot-sized hemorrhages visible in the mucosal lining of
the mouth may occur because the toxins also destroy the platelets, cells
that normally contribute to the clotting system.
The damaged red blood cells and other factors may clog the tiny blood
vessels in the kidneys or cause lesions in the kidneys, making the kidneys
work harder to remove wastes and extra fluid from the blood. The child may
produce little urine as a result. The body's inability to rid itself of
excess fluid and waste may in turn cause hypertension or swelling of the
hands and feet or may cause generalized fluid accumulation (edema).
Once HUS develops, there is no known treatment that can stop the
progress of the syndrome. Unfortunately, it must run its course. Most
treatments are supportive in nature and aimed at easing the immediate
symptoms and signs of this disease and at preventing further
complications. Supportive therapy consists of maintaining specific levels
of fluids and salts, which are replaced only as needed. Blood transfusions
(packed red blood cells) are sometimes needed. Treatment of high blood
pressure is often necessary. Dialysis is also needed at times to help the
kidneys perform their function. Other complications, such as neurologic
complications, necessitate additional medications.
Ninety percent of children with HUS who receive careful supportive care
survive past the initial harrowing stages of the condition, and most of
those will have no long-term effects. But between 10 percent and 30
percent of the survivors will have kidney damage that leads to permanent
kidney failure either immediately or over the course of several years.
Most children with kidney failure will require peritoneal dialysis or
hemodialysis to wash the wastes and extra fluid from their blood or may
require a kidney transplant.
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