KDIGO met again to progress, through controversial means, knowledge of aHUS, or what ever it is called these days.
There were 61 questions asked on 14 aspects of the so called aHUS.
The first set are about the name.
The answers to these might be
1. The classification are important and there are many versions out there. Even if agreed and codified in the ICD, nomenclature is left to individuals to determine and use what they want as has always appeared to the case for our disease.
a. Primary and Secondary TMA or HUS should be dropped it has rarely, if ever, been used by patients. It was brought into explain what atypical meant. But it didn’t achieve that and confusion grew and hence these questions..
b. Using the NKF road map, is there any other road map? Would it be best to kick the can down the road and see if something better comes along.
c. Good question and should be answered by those expert in those etiologies and their patients, Giving time to focus on complement TMA patients.
d. Indeed but will knowing them still put “Primary aHUS” patients at the bottom of it of it all?
e. Feel? About 60% knew about potential name change years ago. No thanks to the way in which it ahs been approached. Patients think too and are not driven by emotions NKF route excluded them. They felt disappointed about being shut out . Is this too little too late question means. Patients will be the ones to think though the practical issues of the mistakes that will be made.
The second set are about clinical tests and measures.
2. For the minimum find the shortest workable current list and for optimum add better ones to it.
a. The shortest used.
b. Others used in longer lists until all suggestions are exhausted. Is this list ideal but sub optimal or over optimal?
c. Came out of left field a bit with the switch from diagnosis. Anyone’s guess? For all transplants?
3. At last an important question for Primary aHUS patients. Its a complement disease where complement is doing something really bad so knowing how much or how little is in the blood should help. Shouldn’t it? Or are Goldilocks amounts of complement still just as bad.
a. Which, when, how see how the questions mount up but its back to all TMAs.
b. As previously said what is the Goldilocks amount and what isn’t? And if a thing can be bad even with the Goldilocks levels how can they say anything?
c. Swift switch to autoantibodies which are measurable. But are all autoantibodies bodies measured? Why is it when autoantibodies could be created for every component in complement is it just one that has an impact and is talked about?
d. Depends on what is good or bad however measured.
Well that 3 out of 14 aspects and 20 out 61 questions.
Article No.793
