Atypical Hemolytic Uremic Syndrome Essay
Atypical Hemolytic Uremic Syndrome (aHUS) is a rare chronic disease, different from typical
hemolytic uremic syndrome (HUS). Typical HUS is caused by bacteria, specifically E. Coli (
Hemolytic uremic syndrome (HUS) , 2016; Noris Remuzzi, 2010). It can be treated, and most
children will have a full recovery ( Hemolytic uremic syndrome (HUS) , 2016). aHUS can develop
in two forms: familial or sporadic, that affect both children and adults, making it difficult to
diagnose (Noris Remuzzi, 2010; NORD, 2016). Atypical Hemolytic Uremic Syndrome is
distinguished by three main manifestations: hemolytic anemia (destruction of RBC),
thrombocytopenia(low platelets), and uremia NORD, 2016; Loirat Fremeaux Bacci, 2011;
Biermann, 2017; Kaplan,... Show more content on Helpwriting.net ...
The main trigger for sporadic aHUS derives from an infection. Other common triggers include
pregnancy, cancer, transplants or certain medications. According to Norris and Remuzzi,
Approximately 50% of sporadic cases appear to be idiopathic . Approximately 10 20% of patients
with sporadic aHUS also display a mutation of the CFH gene. (Noris Remuzzi, 2010)
Mutation of the CFH gene causes a disruption of the activation pathway in the body, therefore it
begins to attack cells in the body specifically on the kidney or liver (Bu, Borsa, Gianluigi, Smith,
2012). The attack on the kidney can lead to thrombotic microangiopathy (TMA), small vessel
clots. Platelets will help minimize any leaking of vessels done by the attack of the disrupted
activation pathway but after several clots form oxygen is cut off from certain parts of the kidney.
When the kidney no longer receives oxygen, those parts begin to die off leading to
thrombocytopenia, anemia and renal failure. When the kidney begins to fail, erythropoietin levels
decrease. EPO binds to a receptor in the bone marrow to inhibit erythropoiesis. Therefore, RBC
production in the bone marrow decreases when EPO levels decrease. (Bu, Borsa, Gianluigi, Smith,
2012)
In the United States approximately 2 per million children are affected by aHUS according to
ncbi.gov. Although genetically complement protein mutations have been more frequent in
European descendants rather than from the USA 5:1. There is no
... Get more on HelpWriting.net ...