Once again, the American Society of Nephrology’s (ASN) Kidney Week adds considerably to the knowledge of kidney disease in all its facets
During Kidney Week, this year held in New Orleans, there are hundreds of talks and presentations, and thousands of posters from clinicians from many countries around the world.
As has been the case for several years now, aHUS is a topic which features and now increasingly associated with other complement related thrombotic microangiopathies (TMAs).
The following is a list of posters presented at Kidney Weeks found with an “aHUS” term search of the conference poster database. Click on the title to see a more detailed abstract of each.
This is the long awaited multi-national report on the study of the those aHUS patients involved in the early trials of the use of Eculizumab for treating aHUS. It works when needed to stop the TMA. The report provides data on the TMA incidents when on or off treatment.
In this report by the Spanish Group for the study of Complement and Renal Disease the results of a study of Spanish aHUS patients whose precipitating event was pregnancy was presented. Among the characteristics was a suggestion that there was a high association with cesarian delivery.
In this review of 306 Brazillian patients 12 were found to have had post transplant TMA and of these 9 were found to have had aHUS and all had higher than needed levels of tacrolimis. There were variable outcomes with use of Eculizumab and half recovered well.
In this case study from Kentucky included in the ASN publication, a patient who had a renal-pancreas transplant experienced idiopathic aHUS post transplant.
In this published abstract from Boston some deposits were found in a post transplant biopsy of an aHUS patient who had experienced recurrent TMA. The significance of such deposits is as yet unknown.
The characteristics of patients in the aHUS Registry for this recruiting site in Virginia are reported with some differences found to the “norm” in terms of age ethnicity predisposing genetic mutations and other factors.
This study from Germany of over 200 patients with TMA found that over half were due to aHUS.
In this case study from Dallas USA ,the challenge of diagnosing the cause of TMA in pregnancy is discussed. A patient presenting with HELLP is found to have aHUS because of complement involvement.
In this paper from Canada a possible trigger for TMA with evidence of CFH involvement is postulated through the use of Calcineurin Inhibitor drugs.
Although the link is not clear in this case study a patient presenting with a TMA in Pennsylvania was found to have Parvovirus infection which is thought to have triggered aHUS.
This abstract which made the publication for Kidney week is from Saudi Arabia and is about how children with aHUS there have a high level of genetic mutations, there is a high level of consanguinity marriages, but they respond well to eculizumab.
This study group in Germany found a novel significant variant (mutation) in Complement Factor H .
In this case study from New York a Secondary TMA trigeered by a catastpohic episode of antiphospholipid syndrom was found to be a likely effect of alternative pathway of Complement . eculizumab was effective as a treatment in this instance.
This study reports on a case study of a patient experiencing aHUS as a result of TMA brought on by Acute Pulmonary-Renal Syndrome with extra renal manifestation.
This oral poster was presented by a group from Maasrict in The Netherlands and reveals that some patients thought to have hypertension related kidney diseases were found to have TMA mediated by Complement and with complement genetic mutations.
In this case study from Alabama a diagnosis of aHUS followed from initially thinking the patient had malignant hypertension TMA. It claims there is an overlap with aHUS for some MH patients.
In this case study from New York a patient presenting with heart failure is found to have a TMA by the use of a skin biopsy which is found to have been complement mediated aHUS.
In this case study from Oklahoma a double hit from complement mutations and a coagulation mutation triggered both an aHUS onset and Lupus flare illustrating again an overlap between primary and secondary aHUS/TMAs
Another example in this study from Chicago of the overlap between complement mediated TMA and other secondary triggers causing renal disease.
In this poster from the Mayo clinic another TMA was studied and because of the complement mediated causes , an overlap between C3G/MPGN and aHUS can result.
In this case study from Japan the possible trigger of aHUS by IgaN is reported.
Whilst it is appreciated that all the clinicians and researchers have had to make a considerable effort to collect evidence and write papers about their findings , recognition needs also to be given to all the aHUS and other patients who have allowed their experiences to be described for the benefit of others.
As has been the case for several years now, the atypical HUS Foundation (USA patient organisation) has had a booth in the Exhibition Hall.