Hemolytic-Uremic Syndrome (HUS)

February 7, 2017

Hemolytic Uremic Syndrome (HUS), commonly referred to as “Hamburger Disease”, is a disease that affects the kidneys and other organs. It poses a substantial threat to Canadian children as one of the leading causes of both acute and chronic kidney failure.

HUS is considered a syndrome because it is a combination of findings that may have different causes. In most cases, HUS occurs after a severe bowel infection with certain toxic strains of the bacteria called E. coli. It may also occur in response to certain medicines, but this is rare. Even more rarely, HUS occurs for unknown reasons. This fact sheet primarily focuses on the type of HUS that occurs in infants and children as a result of an E. coli infection.

It is a condition that affects the blood and blood vessels. It results in the destruction of blood platelets (cells involved in clotting), a low red blood cell count (anemia) and kidney failure due to damage to the very small blood vessels of the kidneys. Other organs, such as the brain or heart, may also be affected by damage to very small blood vessels.

HUS is a kidney condition that happens when red blood cells are destroyed and block the kidneys’ filtering system. Red blood cells contain hemoglobin—an iron-rich protein that gives blood its red color and carries oxygen from the lungs to all parts of the body. When the kidneys and glomeruli—the tiny units within the kidneys where blood is filtered—become clogged with the damaged red blood cells, they are unable to do their jobs. If the kidneys stop functioning, a child can develop acute kidney injury—the sudden and temporary loss of kidney function. Hemolytic uremic syndrome is the most common cause of acute kidney injury in children.

Though hemolytic uremic syndrome is a serious condition, getting timely and appropriate treatment leads to a full recovery for most people, especially young children.

Causes

HUS develops when Escherichia coli (E. coli) bacteria lodged in the digestive tract make toxins that enter the bloodstream and start to destroy red blood cells. Most cases of HUS occur after an infection of the digestive tract by the E. coli bacterium, which is found in foods like meat, dairy products, and juice when they are contaminated. Some people have contracted HUS after swimming in pools or lakes contaminated with feces.

Infection of the digestive tract is called gastroenteritis and may cause a child to vomit and have stomach cramps and bloody diarrhea. Most children who have gastroenteritis recover fully in 2 or 3 days and do not develop HUS.

E.coli O157:H7 can be found in –

  • Undercooked meat, most often ground beef
  • Unpasteurized, or raw, milk
  • Unwashed, contaminated raw fruits and vegetables
  • Contaminated juice
  • Contaminated swimming pools or lakes

Less common causes, sometimes called atypical hemolytic uremic syndrome, can include –

  • Taking certain medications, such as chemotherapy
  • Having other viral or bacterial infections
  • Inheriting a certain type of hemolytic uremicsyndrome that runs in families

More information about foodborne illnesses and the digestive system is provided in the NIDDK health topic, foodborne illnesses.

Risk Factors

Those most at risk of developing hemolytic uremic syndrome are –

  • Children under 5 years of age
  • People who have certain genetic changes that make them more susceptible

Young children and elderly adults are the most likely to be seriously ill from hemolytic uremic syndrome.

Symptoms

HUS often begins with vomiting and diarrhea, which may be bloody. Within a week, the person may become weak and irritable. Persons 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
  • 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)
  • Yellow skin (jaundice)

Complications

  • Blood clotting problems
  • Hemolytic anemia
  • Kidney failure
  • Nervous system problems
  • Too few platelets (thrombocytopenia)
  • Uremia

Treatment

  • Fluid replacement – Lost fluid and electrolytes need to be carefully replaced because the kidneys aren’t removing fluids and waste as efficiently as normal.
  • Red blood cell transfusions – If patients don’t have enough red blood cells, they may feel chilled, fatigued and short of breath. They may have a rapid heart rate, yellow skin and dark urine. Red blood cell transfusions, given through an intravenous (IV) needle, may help reverse these signs and symptoms.
  • Platelet transfusions – If the patient is bleeding or bruising easily, platelet transfusions can help your blood clot more normally. Like red blood cell transfusions, platelet transfusions are given through an IV needle.
  • Plasma exchange – Plasma is the part of blood that supports the circulation of blood cells and platelets. Sometimes a machine is used to clear the blood of its own plasma and replace it with fresh or frozen donor plasma. This process is called plasmapheresis.
  • Kidney dialysis – Sometimes dialysis is needed to filter waste and excess fluid from the blood. Dialysis is usually a temporary treatment until the kidneys begin functioning adequately again. If the kidney damage is significant, however, permanent kidney failure — requiring long-term dialysis or a kidney transplant — is possible.

Alternative Treatment

 

Reference –

https://www.nlm.nih.gov/medlineplus/ency/article/000510.htm

http://www.merckmanuals.com/professional/hematology-and-oncology/thrombocytopenia-and-platelet-dysfunction/thrombotic-thrombocytopenic-purpura-(ttp)-and-hemolytic-uremic-syndrome-(hus)

http://www.niddk.nih.gov/health-information/health-topics/kidney-disease/hemolytic-uremic-syndrome-in-children-hus/Pages/facts.aspx

http://patient.info/doctor/Haemolytic-Uraemic-Syndrome.htm

http://www.merckmanuals.com/professional/hematology-and-oncology/thrombocytopenia-and-platelet-dysfunction/thrombotic-thrombocytopenic-purpura-(ttp)-and-hemolytic-uremic-syndrome-(hus)

http://medicalxpress.com/news/2015-11-hemolytic-uremic-syndrome-treatment-regimen.html

http://medind.nic.in/ibv/t09/i12/ibvt09i12p1075.pdf

http://www.nejm.org/doi/full/10.1056/NEJMra0902814

http://www.aafp.org/afp/2006/0915/p991.html

http://library.med.utah.edu/ed/ahus.html

http://www.uptodate.com/contents/overview-of-hemolytic-uremic-syndrome-in-children

https://www.rareconnect.org/en/community/atypical-hemolytic-uremic-syn

Posted in NUTRITION