Hemolytic uremic syndrome (HUS)
Overview
Hemolytic uremic syndrome (HUS) is a serious condition characterized by the inflammation and damage of small blood vessels, leading to blood clots that can harm the kidneys and other organs, potentially causing life-threatening kidney failure. While anyone can develop HUS, it primarily affects young children and is most commonly triggered by infections with certain strains of Escherichia coli (E. coli) bacteria. Other potential causes include different infections, certain medications, and conditions like pregnancy, cancer, or autoimmune diseases, as well as specific genetic mutations. Despite its severity, timely treatment often results in complete recovery, especially in young children.
Symptoms
Hemolytic uremic syndrome (HUS) is a complex condition that can vary in symptoms depending on its cause. When HUS is triggered by E. coli infection, initial symptoms often include:
- Gastrointestinal issues:
- Diarrhea (potentially bloody)
- Stomach pain, cramping, or bloating
- Vomiting
- Fever
As HUS progresses, it causes significant damage to blood vessels, leading to various severe symptoms due to anemia, blood clots, and kidney damage:
- Anemia and blood clots:
- Pale skin
- Fatigue
- Easy bruising
- Unusual bleeding (e.g., from the nose or mouth)
- Kidney damage:
- Reduced urine output or blood in the urine
- Swelling (edema) in legs, feet, ankles, and less commonly in the face, hands, or entire body
- High blood pressure
- Neurological symptoms: confusion, seizures, or stroke
Classic triad of HUS
The classic medical triad indicating HUS consists of:
- Microangiopathic hemolytic anemia: Damage to red blood cells.
- Thrombocytopenia: A steady decrease in platelet count.
- Acute kidney injury: Sudden impairment of kidney function.
Immediate medical consultation is advised if you or your child experiences:
- Bloody diarrhea
- Persistent diarrhea leading to symptoms such as:
- Reduced urination
- Swelling
- Bruising or unusual bleeding
- Extreme tiredness
Seek immediate medical attention if there is an absence of urination for 12 hours or more.
Causes
Hemolytic uremic syndrome (HUS) is a serious condition, most commonly triggered by infection with specific strains of E. coli bacteria, particularly in children under the age of 5. These strains produce a dangerous toxin known as Shiga toxin, making them referred to as Shiga toxin-producing E. coli (STEC).
While many of the hundreds of E. coli types are harmless, certain strains can lead to HUS. Beyond E. coli, other factors can cause this syndrome:
- Other infections: These can include infections from pneumococcal bacteria, the human immunodeficiency virus (HIV), or influenza viruses.
- Complications from other conditions: In rare cases, conditions such as pregnancy, autoimmune diseases, or cancer may lead to HUS.
- Certain medications: This category includes some cancer treatments and medications designed to prevent organ rejection in transplant recipients.
Additionally, an atypical form of HUS exists, which is inherited. Individuals with the gene for this form do not always develop the condition; however, factors such as infections, specific medications, or ongoing health issues can trigger HUS in those genetically predisposed.
Risk factors
Hemolytic uremic syndrome (HUS), caused by E. coli, can occur through various exposure routes including consuming contaminated meat, fruits, or vegetables, swimming in feces-contaminated pools or lakes, or having close contact with someone infected. The risk of developing HUS is particularly high among children under five, individuals with weakened immune systems, and those with specific genetic predispositions. E. coli infections often stem from eating undercooked meat, especially ground beef, or consuming unpasteurized milk, fruit juices, or contaminated raw produce. Additionally, E. coli can spread via the oral-fecal route, highlighting the importance of thorough hand washing after using the bathroom, changing diapers, or touching animals, as failing to do so can lead to the bacteria entering the system through consumed food or mouth contact.
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
Fluid and mineral replacement
Treatment aims to mitigate reduced kidney function by replenishing lost fluids and minerals, a consequence of the kidneys’ diminished capacity to filter waste and excess fluids properly. Additionally, nutritional support is often administered intravenously to ensure the patient receives necessary sustenance.
Transfusions
- Red blood cells: To alleviate anemia symptoms, patients may receive red blood cell transfusions.
- Platelets: For those experiencing easy bleeding or bruising, platelet transfusions can enhance blood clotting.
Medications
- Blood pressure medication: To address potential lasting kidney damage, medications that lower blood pressure may be used to help prevent or slow further harm.
- Eculizumab: For atypical hemolytic uremic syndrome or complications, eculizumab can prevent additional blood vessel damage. Patients on eculizumab must be vaccinated against meningitis due to increased risk.
Surgical and other procedures
- Kidney dialysis: Temporarily replaces kidney function by removing waste and extra fluid from the blood. It’s used until the kidneys recover, though some may require long-term dialysis.
- Plasma exchange: This process involves replacing the patient’s plasma (the liquid part of blood) with fresh or frozen donor plasma to help circulate blood cells and platelets more effectively.
- Kidney transplant: In cases of severe kidney damage, a kidney transplant may be necessary.
