Hemolytic Uremic Syndrome
Overview
Hemolytic uremic syndrome (HUS) is a condition that disrupts the small blood vessels in the kidneys, leading to the destruction of red blood cells and a decrease in platelet count, which is crucial for clotting. While it can affect individuals of any age, it predominantly impacts young children.
The primary cause of HUS is specific strains of Escherichia coli (E. coli) bacteria. Symptoms commonly include diarrhea, abdominal pain, elevated blood pressure, and reduced urine output. Although the kidneys are primarily affected, other organs may also be involved.
HUS can potentially lead to kidney failure, posing a serious threat. However, timely treatment often results in full recovery, especially in children. Treatment typically involves intravenous fluids, blood plasma, and medication administration.
Symptoms
Generally, hemolytic uremic syndrome damages blood vessels, leading to the breakdown of red blood cells and the formation of blood clots, ultimately resulting in kidney damage.
Acute kidney damage symptoms include:
- Oliguria, or reduced production of urine
- Blood in the urine
- Feeling ill as a result of elevated blood toxin levels
- Severe fatigue
- Bruised easily
- Elevated blood pressure, or hypertension
- Edema, or swelling, in the hands, face, or ankles or less frequently, in other parts of the body
- Dyspnea, or breathing difficulties
- Dizziness, convulsions, or stroke
- The skin or the mucous membranes in the nose and mouth appear pale
Hemolytic uremic syndrome symptoms triggered by E. coli bacteria may involve bloody diarrhea, stomach pain, bloating, fever, and vomiting.
Immediate emergency care is necessary if there is no urine output for 12 hours or longer. Generally, consult a healthcare provider if bloody diarrhea or prolonged diarrhea accompanied by reduced urination, swelling, bruising, unusual bleeding, or extreme fatigue are experienced.
Causes
The majority of the numerous E. coli variants are usual and pose no harm. However, certain strains of E. coli have the potential to cause hemolytic uremic syndrome.
Hemolytic uremic syndrome is primarily caused by specific strains of E. coli bacteria, with the most common being E. coli O157:H7 in North America. These strains, also known as Shiga toxin-producing E. coli (STEC), release a toxin called Shiga, which damages the lining of the small intestines, leading to diarrhea.
Atypical hemolytic uremic syndrome (aHUS) is a rare form of the condition that can be inherited within families. While not everyone who carries the gene for aHUS develops the syndrome, certain factors such as infections, specific medications, or underlying health issues can trigger its manifestation.
Hemolytic uremic syndrome can also be caused by:
- Other infections: Such as influenza virus, HIV infection, and pneumococcal infection.
- Complications of existing conditions: Such as pregnancy or illnesses like cancer or autoimmune diseases.
- Certain medications: Such as those used to prevent organ rejection in recipients of donated organs and certain cancer treatments.
Risk factors
E. coli-caused hemolytic uremic syndrome is often contracted by consuming undercooked or contaminated food and drinks, such as ground beef, unpasteurized milk or juice, and unwashed raw fruits and vegetables.
E. coli can also spread through person-to-person contact via an oral-fecal route, such as swimming in pools or lakes contaminated with fecal matter containing the bacteria.
Individuals at the highest risk of developing hemolytic uremic syndrome include children aged 5 or younger, individuals with weakened immune systems, and those with specific genetic alterations.
Diagnosis
Diagnosing hemolytic uremic syndrome typically requires a series of tests and a thorough physical examination. Each test is designed to detect different markers indicative of the syndrome. When the cause of HUS is unclear, further tests may be performed to pinpoint the underlying factors contributing to the condition.
Tests that may be ordered include:
- Blood tests: During the procedure, a tiny needle is used to take a blood sample from an arm vein. Blood tests provide information on red blood cell and platelet levels, offering insights into kidney and liver functionality. The test can detect damage to the red blood cells.
- Urine test: This test searches for the presence of blood or protein in the urine. A urine sample is often collected through a container. A dipstick is then inserted into the jar. If there is blood or protein in the urine, the color of the dipstick will change.
- Fecal test: This test is conducted to identify the existence of E. coli O157, and a kidney biopsy is performed to reveal any damage to the kidneys.
Treatment
Hemolytic uremic syndrome is treatable. Individuals diagnosed with HUS can achieve recovery without enduring lasting health consequences. Children usually recover better than adults, and more than 85% of people with HUS regain full kidney function.
To compensate for the kidneys’ inability to eliminate waste and fluids as well as they should, treatment entails replenishing lost minerals and fluids. It may also entail receiving nourishment via a vein. Hospitalization is required for treatment of hemolytic uremic syndrome.
- Medicines: For individuals with atypical hemolytic uremic syndrome, healthcare providers may recommend eculizumab or ravulizumab to prevent blood clots that could harm blood vessels, blood cells, and kidneys. A blood pressure-lowering medication may be used to treat hemolytic uremic syndrome-related kidney damage that lasts for a while.
However, taking these medications increases the risk of meningococcal and pneumococcal diseases, so vaccinations and antibiotics may be prescribed. - Transfusions: Red blood cell transfusions can be beneficial in alleviating symptoms of anemia, while platelet transfusions can improve blood clotting in individuals experiencing easy bruising or bleeding. This is often done intravenously while in the hospital.
- Surgery and other procedures: Certain procedures may help with treating hemolytic uremic syndrome, such as:
- Plasma exchange: This procedure involves using a machine to eliminate the fluid part of blood, known as plasma, and replacing it with fresh or frozen donor plasma to facilitate the circulation of blood cells and platelets.
- Kidney transplant: This may be necessary for individuals with severe kidney damage.
- Kidney dialysis: Although dialysis is usually temporary, individuals with substantial kidney damage may require extended dialysis. The process requires removal of waste and excess fluid from the blood, typically performed until the kidneys regain normal function.
