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A UK website about atypical Haemolytic Uraemic Syndrome (aHUS)

KIDNEY WEEK 2015- American Society of Nephrology  (ASN)

KIDNEY WEEK 2015- American Society of Nephrology (ASN)

Each year a Kidney Week is held in the USA by the ASN.

It is the biggest of its kind and over thirteen thousand of delegates are attending Kidney Week 2015   to learn and network  with others from 110 countries  around the world with clinical , industrial and patient organisation backgrounds.

This year the event is being held in San Diego.

This will be the first attended by the aHUS alliance and although aHUS is but a tiny fraction of the kidney issues featured at the conference there is a lot of relevance. Click here for more information about the event

The aim of this blog is to give feedback on the sessions and meetings attended, within, and surrounding the conference; as well as some highlights from the associated Exhibiton Hall.

The opening address on 5th November  by ASN President Johnathon Himmelfarb can be seek by clicking here.

6 November

Early start and a first visit to the large Exhibition Hall which accompanies the conference.

A chance for a look at the Alexion Display close to the entrance and a short chat with a couple of its Marketing people . Then onto the Omeros Booth and quick chat to one of their medical delegates about their developments with existing trials. This is Omeros’s first Kidney Week.

The Foundation for atypical HUS has a booth too, and a chance to meet  the Foundation’s representatives Crystal  and Michelle, who both have sons with aHUS. Their enthusiasm and friendliness was very evident and attracted passing delegates to stop and chat. Some aHUS alliance material was also on display!

Crystal and Michelle

Crystal and Michelle at the Foundation Booth

(It is going to take some time to get around everything including the Poster section, adjacent to the Exhibitions area,which  is enormous. If you have ever been to an aHUS conference this is 100 times the size each day!)

Bumped into Gema Ariceta from Spain, who had talked at the Second aHUS alliance conference in Paris, on the way to the Translational Session  where the the opportunity was also taken to  say hello to Carla Nester from the  University of Iowa, who with Susan E Quaggin was moderator of the   session entitled “Complement Appreciated- A refined role in graft injury and an opportunity for novel therapies.

There were four talks during the session which were as follows:

Emerging Role of Complement in Regulating Alloimmune Responses by Paolo Cravedi, MD, PhD

After a quick overveiw  of the mechanics of complement Paulo demonstrated the part played by complement especially  C3a and C5a in causing T cell proliferation by destabilisation and affecting the T cell regulation, and so  becoming a cause of transplant rejection and requiring a specific therapy.

Complement and Ischemia-Reperfusion Injury by Steven H. Sacks, MD, PhD from Kings College London

Average kidney transplant life is adversely affected by delay in graft working as well as acute rejection. Complement has an injurious effect on not only the recipient, but also on the donated organ. Whilst it may be not surprising that the Alternative Pathway is implicated it was surprising that research into 560 patients studied that the Classical pathway has little part but the Lectin pathway has appeared as an interesting player in the injury causing process.

Inhibiting Terminal Complement Activation in Kidney Transplantation: Where Do We Stand?  by Christophe M. Legendre, MD

Christophe talked  about the effect of anti factor h antibodies and the outcome of four case  studies involving the aHUS, Antiphospholipids Syndrome and C3GN where eculizumab had been used with good results for the first two but not for C3GN.. 

Targetting the Complement Cacade :Novel Treatments coming down the Pike by Joshua M. Thurman, MD

In his talk Joshua looked at complement therapies in development comparing them  against three criteria

  • The balance between blocking C3 and the risk of infections
  • Cost of antobodies v molecules
  • Administrating the drug

The therapies included Purified Plasma Products of which C1 Inhihibtor is showing promise, Monoclonal Antibodies, which Eculizumab leads, Recombitant Proteins and Small Proteins.

These development may have implications for those with aHUS, but as this session showed that complement has role in many kidney diseases and solutions may be around the corner for others.

The next event visited was a lunchtime talk ( a Greek Salad provided! ) by Prof. Craig Shadur of University of Iowa, Desmoines . Craig in a talk titled “Distinguishing aHUS from other TMAs” attempted to show how to make a diagnosis of aHUS and “what clues you in”. After an introduction explaining the illness and why it is worthy of thought given its extensive organ impact not just kidney, he then illustarted with 3 case studies ( a 60 year old man, a 10 month child and a 32 year old pregnant female ) how the clinical manifestation of aHUS caused by as he put it complement amplifying conditions which trigger the aHUS.   He demonstrated the clinical signs and measures including ruling out of ecoli HUS and TTP as there was no simple test to make an aHUS diagnosis. And when all else may not help, how a patients family history can provide some clues. He made the point, often said, that it does not need a genetic test to think aHUS, but that information is very helpful for long term management.



       Prof Craig Shadur: “cluing us all in” on a diagnosis of aHUS

To show this world of aHUS is shrinking, even ended up talking with representatives from Alexion UK, John and Jane, about UK issues -the NHS Specialised Service Specification for aHUS and the SMC application!!!.

Attending the aHUS Investigator Symposium at the Marriot Marquis this evening .

The purpose of the Symposium is to

  • Provide a global update on the aHUS Registry
  • Share the current activities of the aHUS Registry International Scientific Advisory Board
  • Give an opportunity for all participants to discuss their experience with the aHUS Registry
  • Discuss future goals and objectives for the Global aHUS Registry.

The speakers were:

Dr Christophe Gastegar who welcomed investigators and announced that the Registry Numbers were approaching 1000 which was a sign of success and will be beneficial for a rare diseases like aHUS.

Dr Larry Greenbaum confirmed that 945 patients were now registered and then gave some figures about patient registrants and recruiting sites for different countries.He also give details of three publications which  have or are about to be  published.

Prof Eric Rondeau talked about the protocols developed for data entry mentioning how TMA had been defined. He said that some work was needed on the accuracy of genetic data entered.

Dr Franz Schaefer listed and went through the roles and responsibilities  of national coordinators and the purpose of local meetings which had been held ( two further planned for Belgium and USA)

Prof Johan Vande Walle reminded the investigators that although the Registry was run by Alexion its use was guided by the Scientific Advisory Board. He went through the roles and respionsibilities of the SAB and the work it had completed on Advice to Alexion ,Publications completed and the advocacy delivered . He concluded by saying the SAB welcomed and encouraged collaborative research in aHUS using the Registry.


7 November 2015

Return to the Exhibition Centre to walk the Posters area. Starting from No 1 and then passed over 1000 posters. There were 5 readily identifiable as about aHUS. The first two were about case studies of single patients who had onset with rare triggers including SLE. Then there was a poster by someone from the Redboud University in The Netherlands about the success with eculizumab treatment there.

At this point it was apparent that I was on a parallel course with someone else who was looking at aHUS posters specifically . Dr Lillian Pereira  from Brazil  introduced herself  and said that she was particularly interested in diseases which had a TMA event, which of course includes aHUS. A conversation followed about aHUS patients in Brazil and how some had been in contact with aHUS organisations in other countries  belonging to the aHUS alliance and how it is hoped from that a patient organisation might emerge in Brazil; something that Lillian thought was needed.

aHUS posters were now very rare when one appeared by Gianluigi Ardissino, from Milan, about a process for relieving  the stress of blood monitoring of young children, which had  led to more accurate readings and thus revealed those children who were in need of treatment for hypertension. Gianluigi is perhaps better known for his studies on tailoring treatment for aHUS patients, which had been the subject of  his poster on a previous day! He provide a copy of it  for this blog.  Click here to see Extension


Dr Gianluigi Ardissino

There was a concern  beginning about ever spotting another aHUS poster when Tess Harris appeared in the section devoted to posters related to Polycystic Kidney Disease. Tess  is Chief Executive of the PKD Charity in the UK and a member of the rare kidney disease group supported by KRUK which aHUSUK also belongs to.

Soon after luck changed another poster was found about the success story of eculizumab supported transplants in France.

Although not strictly  an aHUS related, a poster from the University of Utah provided insights into how the stress of living with chronic kidney failure affected adolescent’s self esteem and compliance with treatment following kidney transplant.Intervention with CBT counselling had proved beneficial to their adjustment. This could apply to any adolescents adjusting to life itself as well as a life including a chronic health condition.

It makes you think how a patient organisation “research poster” might fare in among the clinical ones some time in the future!

On leaving the poster exhibition another visit was made  to the Foundation’s booth for a chat. Crystal having gone home, Michelle was joined by her husband. Three days of “manning the stall” really is hard work but it clearly had been worthwhile in raising awareness, not just in the US, but, because of the nature of the conference, globally.

One final visit to the Alexion Exhibit and quite a long and interesting chat with another of their representatives, whose role is to give support to clinicians in the USA when making an aHUS diagnosis; something which has probably helped  many a patient in the US to get a better outcome from their encounter with aHUS.

The day ends with dinner with a group of clinicians from the UK- from Brighton, Manchester, Nottingham, Stoke  and Newcastle upon Tyne, including “Journey for Life walker” and aHUS Conference presenter  Edwin Wong, and Faieza Quasim . An interesting and insightful discussion ensued about aHUS issues in UK. During dinner some reports were being received about events in Puna in India where the first ever Indian  aHUS Conference was taking place. It is a small world!

8 November 2015

So onto the last day and the Sunday morning sessions before the Kidney Week 2015 comes to an end.

Pathollant Kidney by Zoltan G. Laszik, MD, PhD Pathology of TMA in the Transplant 

Zoltan addressed the damage caused by TMA ,rather than the triggers or even the resolution of it. He produced an extensive list of damage effects and illustrations of the features which can be found in the a damaged kidney by TMA. He went on say that with appropriate treatment that damage can clear up in a few days.

Infections in HUS  by Jeffrey C. Laurence, MD

Jeffrey focused on how post transplant TMA manifestations occurred as a result of viral and bacterial infection and illustrated it with a cmprehensive list of such agents and a few case studies  to show that the normal tests did not lead to the patients getting the best treatment and how he had intervened , sometimes using skin biopsies, in the cases to bring about a better outcome for the patients.

Generic Mutations in Hereditary HUS and the Transplant Kidney: New Insights and New Challenges by Peter F. Zipfel, PhD

Peter brought together all the known  genetic variants that are implicated in post- transplant TMA.. He said the challenge of a direct diagnosis test was still needing to be met but went through the diagnosis tests currently available including plasma tests , auto immune  antibody searches , genetic tests and kidney biopsies.He finished with a copy of an excellent illustration by Paul Morgan and Claire Harris in their review of complement inhibitors in progress.

Pathology and Pathogenesis of Antibody-Mediated Rejection–Associated TMA in the Transplant  by Mark Haas, MD, PhD

Mark was presenting the last talk of the last session of ASN 2015 and using the American Football draft analogy of the last person selected becoming known as “Mr Irrelevant” to introduce himself ; but he was far from and he  reinforced how much complement was implicated in Anti body Mediated Rejection through TMA. This talk underlined how important TMA was to diagnosis and it was a standout feature in all the complement and aHUS talks.


So  the take away message from KIDNEY WEEK 2015


THINK KIDNEY                               THINK AKI                         THINK TMA                             THINK aHUS

5 Responses to KIDNEY WEEK 2015- American Society of Nephrology (ASN)

  1. Linda Burke says:

    Thanks for providing a presence for the aHUS community at this event, looking forward to further information as it becomes available.

  2. Cheryl says:

    Fantastic overview Len. As always, it is a privilege reading your report. Thank you.

    • aHUSUK says:

      Thanks Cheryl now that I am not so sleep deprived i have even had a chance to remedy some of the typos and spelling mistakes in it! I guess that you might have experienced the overwhelming effect of a Kidney Week in the past. From what I have been told about similar events in the USA for cardiology I could not imagine what it would be like attending something which has delegates numbering 100,000 plus!!!!

  3. Phyllis Talbot says:

    Hey Len! Great update! (and on a side note isn’t San Diego a fabulous city! Been there for an ASN myself). Hope you are survived the crazy pace though and glad you got to see Dr. Greenbaum – we LOVE him – he’s Hyde’s main AHUS doc and also the bow-tie wearing juggling doc Hyde loves 😉

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