hemolytic-uremic syndrome


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Hemolytic-Uremic Syndrome

 

Definition

Hemolytic-uremic syndrome (HUS) is a rare condition that affects mostly children under the age of 10, but also may affect the elderly as well as persons with other illnesses. HUS, which most commonly develops after a severe bowel infection with certain toxic strains of a bacteria, is characterized by destruction of red blood cells, damage to the lining of blood vessel walls, and in severe cases, kidney failure.

Description

Most cases of HUS occur after an infection in the digestive system that has been caused by toxin-producing strains of the bacterium Escherichia coli. About 75% of HUS cases in the United States are caused by the strain referred to as E. coli O157:H7, which is found in the intestinal tract of cattle, while the remaining cases are caused by non-O157 strains. Some children infected with E. coli O157:H7 will develop HUS. HUS also can follow respiratory infection episodes in young children. In the United States, there are about 20,000 infections and 250 deaths annually that are caused by E. coli O157:H7. HUS has also been known to occur in persons using drugs such as oral contraceptives, immunosuppressors, and antineoplastics, and in women during the postpartum period.
E. coli. O157:H7, first identified in 1982, and isolated with increasing frequency since then, is found in contaminated foods such as meat, dairy products, and juices. Infection with E. coli. O157:H7 causes severe gastroenteritis, which can include abdominal pain, vomiting, and bloody diarrhea. For most children, the vomiting and diarrhea stop within two to three days. However, about 5 to 10% of the children will develop HUS and will become pale, tired, and irritable. Toxins produced by the bacteria enter the blood stream, where they destroy red blood cells and platelets, which contribute to the clotting of blood. The damaged red blood cells and platelets clog tiny blood vessels in the kidneys, or form lesions to occur in the kidneys, making it difficult for the kidneys to remove wastes and extra fluid from the body, resulting in hypertension, fluid accumulation, and reduced production of urine.

Causes and symptoms

The most common way an E. coli O157:H7 infection is contracted is through the consumption of undercooked ground beef (e.g., eating hamburgers that are still pink inside). Healthy cattle carry E. coli within their intestines. During the slaughtering process, the meat can become contaminated with the E. coli from the intestines. When contaminated beef is ground up, the E. coli are spread throughout the meat. Additional ways to contract an E. coli infection include drinking contaminated water and unpasteurized milk and juices, eating contaminated fruits and vegetables, and working with cattle. The infection is also easily transmitted from an infected person to others in settings such as day care centers and nursing homes when improper sanitary practices are used.
Symptoms of an E. coli O157:H7 infection start about seven days after infection with the bacteria. The first symptom is sudden onset of severe abdominal cramps. After a few hours, watery diarrhea starts, causing loss of fluids and electrolytes (dehydration), which causes the person to feel tired and ill. The watery diarrhea lasts for about a day, and then changes to bright red bloody stools, as the infection causes sores to form in the intestines. The bloody diarrhea lasts for two to five days, with as many as ten bowel movements a day. Additional symptoms may include nausea and vomiting, without a fever, or with only a mild fever. After about five to ten days, HUS can develop, which is characterized by paleness, irritability, and fatigue, as well as reduced urine production.

Diagnosis

The diagnosis of an E. coli infection is made through a stool culture. The culture must be taken within the first 48 hours after the start of the bloody diarrhea. If a positive culture is obtained, the patient should be monitored for the development of HUS, with treatment initiated as required.
Children should not go to day care until they have had two negative stool cultures. Older people in nursing homes should stay in bed until two stool cultures are negative.

Treatment

Treatment of HUS is supportive, with particular attention to management of fluids and electrolytes. Treatment generally is provided in a hospital setting. Blood transfusions may be required. In about 50% of the cases, short term replacement of kidney function is required in the form of dialysis. Most patients will recover kidney function and be able to discontinue dialysis.
Some studies have shown that the use of antibiotics and antimotility agents during an E. coli infection may worsen the course of the infection and should be avoided. However, other studies have been less definitive. Physicians should stay informed so that clinical practices matches medical advances on this aspect of treatment.

Alternative treatment

Persons with HUS must be under the care of health care professionals skilled in the treatment of HUS.

Prognosis

Ninety percent of children with HUS who receive careful supportive care survive the initial acute stages of the condition, with most having no long-term effects. However, between 10 and 30 percent of the survivors will have kidney damage that will lead to kidney failure immediately or within several years. These children with kidney failure require on-going dialysis to remove wastes and extra fluids from their bodies, or may require a kidney transplant.

Key terms

Antineoplastics — Agents that inhibit or prevent the development, maturation, and proliferation of malignant cells.
Gastroenteritis — An acute inflammation of the lining of the stomach and intestines, characterized by nausea, diarrhea, abdominal pain and weakness, which has various causes, including food poisoning due to infection with such organisms as Escherichia coli, Staphylococcus aureus and Salmonella species, consumption of irritating food or drink, or psychological factors such as anger, stress and fear.

Prevention

Prevention of HUS caused by ingestion of foods contaminated with E. coli O157:H7 and other toxin-producing bacteria is accomplished through practicing hygienic food preparation techniques, including adequate handwashing, cooking of meat thoroughly, defrosting meats safely, vigorous washing of fruits and vegetables, and handling leftovers properly. Irradiation of meat has been approved by the United States Food and Drug Administration and the United States Department of Agriculture in order to decrease bacterial contamination of consumer meat supplies.

Resources

Other

National Kidney and Urologic Diseases Information Clearinghouse. Fact Sheet: Hemolytic Uremic Syndrome. NIH Publication No. 99-4570. March 2000. 〈http://www.niddk.nih.gov/health/kidney/summary/hus/〉.

hemolytic-uremic syndrome

Internal medicine A condition often accompanied by a prodrome of bloody diarrhea, more common in summer and microangiopathic hemolytic anemia, thrombocytopenia and platelet defects, not uncommon in infants < age 2 Lab Impaired aggregation, depleted platelet serotonin, ADP, β-thromboglobulin. See TTP-HUS.
hemolytic-uremic syndrome etiology
•  Prototypic or 'classic' form
•  Post-infectious, eg Shigella dysenteriae-1, S pneumoniae, Salmonella typhi, occasionally viruses
•  Hereditary forms: AD or AR associated with HTN
•  Immune-mediated forms
•  Associated with other diseases, eg HTN, connective tissue disease, immunosuppression, RT to kidneys
•  Related to pregnancy and oral contraceptives
References in periodicals archive ?
Lozano, "Efficacy of eculizumab in the treatment of recurrent atypical hemolytic-uremic syndrome after renal transplantation," Transplantation, vol.
ABBREVATIONS: ADAMTS = a disintegrin-like and metalloprotease domain with thrombospondin type motifs; HUS = hemolytic-uremic syndrome; LD = lactate dehydrogenase; MAHA = microangiopathic hemolytic anemia; PT = prothrombin times; PTT = partial thromboplastin times; TMA = thrombotic microangiopathy; TT = thrombin times; TTP = thrombotic thrombocytopenic purpura; ULVWF = ultralarge VWF; VTEC = verotoxin-producing Ecoli; VWF = Von Willebrand factor; VWF-cp = VWF-cleaving protease.
HUS, or Hemolytic-uremic syndrome, has so far been found in 520 people.
One metaanalysis did not show a higher risk of hemolytic-uremic syndrome associated with antibiotic use (JAMA 2002;288:996-1001), while a small study demonstrated safe treatment of carriers of shiga-producing non-0157 E.
No cases of hemolytic-uremic syndrome or deaths were reported, although one elderly man was in critical condition.
Escherichia coli O 157:H7 and the hemolytic-uremic syndrome. N Engl J Med 1995;333:364-8.
coli O157:H7 has emerged as the single greatest cause of hemolytic-uremic syndrome (HUS) and hemorrhagic colitis in the United States (1,2).
coli-caused hemolytic-uremic syndrome (HUS), a severe complication of a type of E.
Heterogeneity of Shiga toxin-producing Escherichia coli strains isolated from hemolytic-uremic syndrome patients, cattle, and food samples in central France.
coil isolated from stool specimens or hemolytic-uremic syndrome, and 16 patients were hospitalized (MMWR 50[151:293-97, 2001).
Pavia AT, Nichols CR, Green DP, et al, Hemolytic-uremic syndrome during an outbreak of