Learn About Hemolytic-Uremic Syndrome

What is the definition of Hemolytic-Uremic Syndrome?

Shiga-like toxin producing E coli hemolytic-uremic syndrome (STEC-HUS) is a disorder that most often occurs when an infection in the digestive system produces toxic substances. These substances destroy red blood cells and cause kidney injury.

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What are the alternative names for Hemolytic-Uremic Syndrome?

HUS; STEC-HUS; Hemolytic-uremic syndrome

What are the causes of Hemolytic-Uremic Syndrome?

Hemolytic-uremic syndrome (HUS) often occurs after a gastrointestinal infection with E coli bacteria (Escherichia coli O157:H7). However, the condition has also been linked to other gastrointestinal infections, including shigella and salmonella. It has also been linked to non-gastrointestinal infections.

HUS is most common in children. It is the most common cause of acute kidney failure in children. Several large outbreaks have been linked to undercooked hamburger meat contaminated with E coli.

E coli can be transmitted through:

  • Contact from one person to another
  • Consuming uncooked food, such as milk products or beef

STEC-HUS is not to be confused with atypical HUS (aHUS) which is not infection-related. It is similar to another disease called thrombotic thrombocytopenic purpura (TTP).

What are the symptoms of Hemolytic-Uremic Syndrome?

STEC-HUS often begins with vomiting and diarrhea, which may be bloody. Within a week, the person may become weak and irritable. People with this condition may urinate less than normal. Urine output may almost stop.

Red blood cell destruction leads to symptoms of anemia.

Early symptoms:

  • Blood in the stools
  • Irritability
  • Fever
  • Lethargy
  • Vomiting and diarrhea
  • Weakness

Later symptoms:

  • Bruising
  • Decreased consciousness
  • Low urine output
  • No urine output
  • Pallor
  • Seizures -- rare
  • Skin rash that looks like fine red spots (petechiae)
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What are the current treatments for Hemolytic-Uremic Syndrome?

Treatment may involve:

  • Dialysis
  • Medicines, such as corticosteroids
  • Management of fluids and electrolytes
  • Transfusions of packed red blood cells and platelets
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What is the outlook (prognosis) for Hemolytic-Uremic Syndrome?

This is a serious illness in both children and adults, and it can cause death. With proper treatment, more than half of people will recover. The outcome is better in children than adults.

What are the possible complications of Hemolytic-Uremic Syndrome?

Complications may include:

  • Blood clotting problems
  • Hemolytic anemia
  • Kidney failure
  • Hypertension leading to seizures, irritability, and other nervous system problems
  • Too few platelets (thrombocytopenia)
  • Uremia
When should I contact a medical professional for Hemolytic-Uremic Syndrome?

Contact your provider if you develop symptoms of HUS. Emergency symptoms include:

  • Blood in the stool
  • No urination
  • Reduced alertness (consciousness)

Contact your provider if you have had an episode of HUS and your urine output decreases, or you develop other new symptoms.

How do I prevent Hemolytic-Uremic Syndrome?

You can prevent the known cause, E coli, by cooking hamburger and other meats well. You should also avoid contact with unclean water and follow proper hand washing methods.

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What are the latest Hemolytic-Uremic Syndrome Clinical Trials?
Eculizumab in Hypertensive Emergency-associated Hemolytic Uremic Syndrome: a Randomized Multicenter Controlled Trial

Summary: Hemolytic and uremic syndrome (HUS) is a clinic-biological syndrome related to thrombotic microangiopathy affecting predominantly the kidney. Atypical HUS (aHUS) has been historically defined as HUS occurring in the absence of infectious event. The role of complement dysregulation in aHUS pathophysiology has been largely demonstrated, since C genetic rare variants are present in 60-70% aHUS patien...

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A Phase III, Multicenter, Single-Arm Study Evaluating the Efficacy, Safety, Pharmacokinetics, and Pharmacodynamics of Crovalimab in Pediatric Patients With Atypical Hemolytic Uremic Syndrome (aHUS)

Summary: This study aims to evaluate the efficacy and safety of crovalimab in pediatric participants with aHUS.

What are the Latest Advances for Hemolytic-Uremic Syndrome?
The treatment of atypical hemolytic uremic syndrome with eculizumab in pediatric patients: a systematic review.
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Oxaliplatin-induced thrombotic microangiopathy: a case report.
Who are the sources who wrote this article ?

Published Date: January 25, 2022
Published By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Alexander T, Licht C, Smoyer WE, Rosenblum ND. Diseases of the kidney and upper urinary tract in children. In: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. Brenner and Rector's The Kidney. 11th ed. Philadelphia, PA: Elsevier; 2020:chap: 72.

Mele C, Noris M, Remuzzi G. Hemolytic uremic syndrome. In: Ronco C, Bellomo R, Kellum JA, Ricci Z, eds. Critical Care Nephrology. 3rd ed. Philadelphia, PA: Elsevier; 2019:chap 50.

Schneidewend R, Epperla N, Friedman KD. Thrombotic thrombocytopenic purpura and the hemolytic uremic syndromes. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 134.