Text 14. Cerebral Dominance. Left Brain/Right Brain. Hemolytic Uremic Syndrome

Hemolytic uremic syndrome (HUS) is a systemic disease marked by renal failure, hemolytic anemia, thrombocytopenia (platelet deficiency), coagulation defects, and variable neurological signs (MedlinePlus, 2002). This disorder is most common in children. It frequently occurs after a gastrointestinal (enteric) infection, often one caused by a strain of specific E. colibacteria (Escherichia coli O157: H7). It has also been associated with other enteric infections, including shigella and salmonella, and with some nonenteric infections (MedlinePlus, 2002; Rothenberg & Chapman, 1994).

HUS often begins with vomiting and diarrhea (which may be bloody). Within a week the patient develops weakness and irritability. Urine output decreases dramatically and may almost cease. Because red blood cells are being destroyed (a process called hemolysis), the patient rapidly becomes anemic and pale (MedlinePlus, 2002). The incidence of HUS is 1–3 per 100,000, with the highest incidence occurring in the summer and fall. The age of onset is most common under the age of 4 years. HUS is the most common cause of acute renal failure in children (Pedlynx, 2002). There are two forms of HUS: the typical form (idiopathic) and the atypical or sporadic form. The typical form usually affects children 3 months to 6 years of age (80 percent < 3 years) and is caused by an E. coli serotype 0157:H7 that can produce specific enterocytotoxins. The risk factors for E. coli acquisition are undercooked ground beef and contact with a person with diarrhea within 2 weeks prior to disease onset. One in 10 children who have E. coli 0157:H7 will go on to develop HUS (Pedlynx, 2002). The atypical or sporadic form may be associated with an inherited autosomal recessive or dominant form and with scleroderma, radiation of kidneys, and essential or malignant hypertension. There is also a pregnancy or oral contraceptive association related to preeclampsia or postpartum renal failure (Pedlynx, 2002). Characteristics of hemolytic uremic syndrome may include:

1. Gastroenteritis

• Usually precedes illness by 5–10 days

• Diarrhea, bloody stool, severe colitis

• Fever, nausea, and vomiting

• Rectal prolapse

2. Renal manifestations

• Microscopic or gross hematuria

• Proteinuria that can progress to the nephritic level

• Complications such as nephritic syndrome (edema, hypertension, azotemia, oliguria), nephrotic syndrome (edema, hypoalbuminemia, hyperlipidemia), and renal failure that can range from mild renal insuffi ciency to acute renal failure (ARF).

3. Hematological manifestations

• Anemia

• Sudden onset of pallor, irritability, lethargy, weakness

• Hepatomegaly-hepatosplenomegaly

• Thrombocytopenia (90 percent)

4. Complications

• Irritability, seizures, coma

• Colitis with melena and perforation

• Acidosis, congestive heart failure, diabetes mellitus, fluid overload, hyperkalemia, rhabdomyolysis

Treatment usually includes transfusions of packed red cells, and platelets are given as needed. Kidney dialysis may be indicated. Medications prescribed include corticosteroids and aspirin. Plasmapheresis, also called plasma exchange (or passage of the plasma through a Protein A filter) may be performed, although its role is much less well documented than in TTP (thrombotic thrombocytopenic purpura). The blood plasma (the portion that does not contain cells, but does contain antibodies) is removed and replaced with fresh (donated) or filtered plasma to remove antibodies from the circulation (MedlinePlus, 2002). In addition, medical management of the complications such as nephritic syndrome, nephrotic syndrome, and chronic renal failure may include dialysis, kidney transplant, or both (Pedlynx, 2002). Special education may or may not be needed for children with HUS. The course and outcome of the disease are highly variable, and individual educational needs will vary as well. Chronic renal problems and medications may well create the need for typical chronic illness counseling and supportive assistance. Ninety percent of patients survive the acute phase with no renal impairment if aggressive management of acute renal failure (ARF) is instituted. A positive prognosis is associated with the age of the child, typical form, and summer months for diagnosis. A poor prognosis is associated with shock, signifi cant renal involvement, neurological signs and symptoms, and atypical form. The prognosis for children with HUS includes a mortality rate of 7–10 percent and renal dysfunction of 20 percent (Pedlynx, 2002). The known cause of HUS, E. coli in hamburger and ground meats, can be prevented by adequate cooking. Other unrecognized causes may not be preventable at this time.

(Source: Encyclopedia of Special Education, THIRD EDITION Cecil R. Reynolds and Elaine Fletcher-Janzen, Editors, 2007)

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