aHUS is….

Article No. 345

20 May 2020

COVID 19 lockdown does seem to give more time to think about things, so the question “What is aHUS?” has been pondered to fill in some time!
It is question that is frequently asked, and is answered in different ways depending on who is asking and the perception of those responding.
Take as an example the first two pages of Google search engine results on 16 May 2020  for the search term “What is aHUS”. Then looking at each for the first sentences of the answers beginning with “aHUS is..”  There were 16 websites found (n=16) and their first explanatory sentences starting “aHUS is…” are shown below. Only two of those websites (2/16) use the same explanatory words ( GARD and Patient Services)
The majority  (13/16) start by using the words represented by the abbreviation , most  10/13 use “Atypical hemolytic uremic syndrome”  ( sometimes, (2/10) ,  with a hyphen between the hemolytic and the uremic) and frequently switching the cases  so  the “a” in a HUS  starts with  “A”  and the rest is lower case “hemolytic etc”. Logically aHUS should  become “Ahus” , but “aHUS” in brackets  is used  in 10/16 sentences.
Although it would be unusual to start a sentence with out a capital letter,  would it offend the grammar police  if “atypical” or “a”  could be allowed to be the exception that proves the rule here?  iPad could not be IPad.
To confuse matters even more the “e” becomes “ae” depending on whether the Greek or Latin root  of the terms is preferred.(3/13).
Most (10/16) claim it is rare, qualified by some as “extremely” rare (2/10)  , “very” rare (2/10) and “ultra”  rare (1/10).
It is referred to as a “disease” by most sites (11/16 )” and a condition by some (4/16).
A syndrome is a collection of signs and symptoms which correlate and are associated with a particular disease and the remainder of the explanations to a greater or lesser extent describe the symptoms and signs of the syndrome.
Kidneys are mentioned in 10/16 explanations.
Blood clots are referred to in  7/16 explanations in which  thrombotic microangiopathy is mentioned in  2/7 occasions. Those blood clots are described  also as  “abnormal”  (2/7)  or “too many” (1/7). The clots are said to form in blood vessels (5/16) but  2/5 refer to the fact that the vessels are “small”.
On only 1/16 occasion is the destruction of red blood cells ( hemolytic anemia) mentioned or low platelets (thrombocytopenia) or uremia as clinical symptoms. But those appear in a sentence containing 59 words (NORD).
The cause of the disease is described as genetic ( 5/16) or due to Complement (3/16).
In a small number of instances (2/16)  aHUS is  described life threatening,  as well as “chronic” (2/16) and “progressive” (2/16)  . Only on one site does it mention that it affects adults and children (1/16).
The above analysis is based on the first sentence beginning “aHUS is….”,  in fairness each of the sites goes on to explain more aspects of aHUS in the sentences that follow and the core elements above within the all the first sentences are likely to be covered.
It is difficult to say clearly and succinctly ” what aHUS is “. Calling it aHUS as “something that is not HUS” risked being confusing from the outset for both medics and patients. Maybe this analysis merely confuses an already confusing situation.
Blame that on  COVID19 and too much time in lock down, or perhaps the time is approaching when a clear consistent and comprehensive answer should be available is one short sentence.
atypical Hemolytic Uremic Syndrome is a very rare, life threatening thrombotic microangiopathy which damages the kidneys as a result of uncontrolled activation of  genetically defective, or hampered, Complement.
Or is it just something that is NOT HUS.
Meanwhile a word cloud created from the sentence below exemplifies where we are now.

The sentences from the first two pages of website results resulting from a Google search for an answer to “What is aHUS”
Atypical hemolytic uremic syndrome (aHUS) is a disease that causes abnormal blood clots to form in small blood vessels in the kidneys. (GARD)
 aHUS (atypical hemolytic uremic syndrome) is a rare disease that causes too many blood clots to form in your blood vessels. ( AKF)
Atypical hemolytic uremic syndrome (aHUS) is an extremely rare disease characterized by low levels of circulating red blood cells due to their destruction (hemolytic anemia), low platelet count (thrombocytopenia) due to their consumption and inability of the kidneys to process waste products from the blood and excrete them into the urine (acute kidney failure), a condition known as uremia.  (NORD)
Atypical hemolytic-uremic syndrome is a disease that primarily affects kidney function (NIH)
Atypical hemolytic uremic syndrome (aHUS) is an extremely rare, life-threatening, progressive disease that frequently has a genetic component. (Wikipedia)
aHUS is an ultra-rare disease caused by a fault in the complement system. (NRCTC)
According to the National Center for Advancing Translational Sciences, Atypical hemolytic uremic syndrome (aHUS) is a disease that causes abnormal blood clots to form in small blood vessels in the kidneys. (Patient services)
aHUS is a rare genetic disease characterized by the formation of blood clots in the small blood vessels of the kidneys. (aHUS News)
Atypical haemolytic uraemic syndrome (aHUS) is a very rare kidney condition. (KRUK)
Atypical HUS (Hemolytic Uremic Syndrome) is a genetic disease in origin and has historically fallen under the category of “kidney disease” as the kidney was the main organ system involved.
aHUS is a rare, genetic, chronic disease that can damage vital organs such as the kidneys (Soliris.net)
Atypical hemolytic uremic syndrome is a rare condition caused by the uncontrolled activation of complement, and subsequently leads to complement-mediated thrombotic microangiopathy. (Haelio)
Atypical haemolytic uraemic syndrome (aHUS) is a chronic, rare, progressive condition
that causes severe inflammation of blood vessels and the formation of blood clots in
small blood vessels throughout the body, a process known as systemic thrombotic
microangiopathy. ( NHS England)
Atypical hemolytic-uremic syndrome (aHUS), unlike typical HUS, is not due to bacteria but rather to an idiopathic or genetic cause that promotes dysregulation of the alternative complement pathway.  (Hindawi).
Atypical Hemolytic Uremic Syndrome (aHUS) is a very rare, chronic and life-threatening genetic condition ( aHUS alliance)
Atypical Haemolytic Uraemic Syndrome (aHUS) is a very rare disease affecting the kidneys and other organs and can affect both adults and children in a similar way.  (Renal Association)

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