aHUS atypical Hemolytic Uremic Syndrome (aHUS) website

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aHUS glossary

ADAMTS13:
a type of protein that occurs naturally in the body. It helps break up a different protein that can help produce clots. An ADAMTS13 test can identify patients with TTP because they have low levels of ADAMTS13. aHUS patients have normal or only slightly reduced levels.

Atypical:
something that is irregular; an unusual type atypical Haemolytic Uraemic Syndrome (aHUS): a disease of the blood that causes low red blood cell and platelet counts, kidney failure, and damage to other vital organs, such as the heart and brain.

Complement system:
a network of proteins and enzymes that interact with each other to protect the body against foreign substances, like bacteria and other invading organisms. aHUS is a disease in which the complement system has trouble figuring out who is “one of us” and who is “one of them”.

Creatinine:
a chemical excreted by the kidneys, which when measured, shows whether the kidneys are functioning properly.

Colon:
the lower part of the large intestine.

Dialysis:
a treatment for kidney failure. Normally, the kidneys work to filter the blood and remove waste, excess salt, and water. Kidney failure, also called “end-stage renal disease,” occurs when the kidneys stop working completely. During haemodialysis, a machine takes over the job of the kidney by filtering the blood outside of the body, and then returning the filtered blood back to the body.

E coli:
bacteria that normally exist in the lower intestines of humans and other animals, which can cause illness.

Enzyme:
a protein that starts a chemical reaction within the body.

Immune system:
a complex group of cells, proteins, and other molecules that work together to identify foreign organisms and substances, such as bacteria; the main role of the system is to protect the body against these foreign organisms.

Infusion:
a process during which fluid is introduced into the body through a vein.

Microangiopathy:
a disease of very small blood vessels.

Mutation:
a permanent change in genetic material, usually in a single gene.

Oedema:
swelling of certain parts of the body due to the presence of abnormally large amounts of fluid.

Plasma:
the pale yellow liquid part of whole blood, in which the red and white blood cells and various other elements are suspended.

Plasma exchange:
a process of removing, treating, and returning plasma to the body.

Purpura:
any condition characterised by bleeding in the skin and mucous membranes, which can appear as bruises on the surface of the skin.

Shiga-toxin:
a poison produced by E coli bacteria that causes severe diarrhoea.

Shiga-toxin-production E coli haemolytic uraemic syndrome (STEC-HUS):
a syndrome triggered by Shiga-toxin–producing E coli. The disease is characterised by diarrhoea that is often bloody and followed by acute renal failure.

Stroke:
damage to the brain. Strokes can happen when an artery in the brain becomes clogged or starts bleeding and cuts off the blood supply to that portion of the brain.

Syndrome:
a set of symptoms that occur together in a pattern.

Systemic:
spread throughout the body.

Thrombotic:
producing blood clots.

Thrombocytopenia:
decreased numbers of platelets.

Thrombotic Microangiopathy (TMA):
clotting and inflammation that damages small blood vessels throughout the body; this is a symptom or a result of diseases such as aHUS and TTP.

Thrombotic Thrombocytopenic Purpura (TTP):
a rare condition that causes blood clots to form in small blood vessels throughout the body, leading to widespread thrombotic microangiopathy.

Uraemia:
signs and symptoms of kidney failure; signs and symptoms of uraemia can include nausea, vomiting, metallic taste in the mouth, muscle pain, and swelling.