The Global Action website has been featuring the pioneers and first events of aHUS history in its series Before aHUS Endeavours Fade.
We have featured the work of Professor Conrad Von Gasser who gave the disease its name.
On this day, 20 September , in 1955 the disease name HUS appeared in public for the first time, albeit in its German form “hämolytisches urämisches syndrom”
It is no surprise that over nearly seventy years later, the actual article identifying our disease has disappeared from public view. Even from the Swiss Medical Weekly journal that published it.
Any citation in modern articles would bring the reader to a list in which only the title and authors’ names are shown. Not the article. Not even an abstract.
To the best of our knowledge that first article in German has never been published nor available in English.
Global Action has done something about that. It has had the original article translated into English and it is published for the first time today. (A copy of the seven page document follows this article below).
The article basically goes into the clinical and pathological detail about what happened, and was observed, in the health catastrophe befalling four little girls and one boy in a Zurich hospital in the 1950s.
They were Susi ( age 7 years) , Carmen ( 2 months) , Barbara ( 7 months) , Ruth ( 13 months) and Walter ( 14 months) and they were therefore the first aHUS patient cohort identified and included in aHUS research.
They shared a triad of symptoms :
- Haemolytic anaemia
- thrombocytopaenia
- kidney failure
The three symptoms that define our aHUS to this day.
Four of them had evidence of an infection triggers. Two with pneumonia , one with rhinitis and one with diarrhoea/vomiting . The other had no evident trigger at all but had a history of allergic reaction to strawberries!!
Barbara was the first to be officially diagnosed with Haemolytic Uraemic Syndromes, or aHUS as it is now known.
The official diagnosis of the others was revised after their deaths. Years later one them was thought by one of the authors to have actually been a childhood case of TTP.
aHUS alliance Global Action wishes thank Astra Zeneca Alexion for their help in providing us with a remaining pdf copy of the German version of the article.
Also a big thank you too to donors to our PayPal Giving account which allowed us to pay for a professional translation of this historic article into English.
So for the first time the Von Gasser et al HUSs article can be read in English.
Susi, Carmen , Barbara, Ruth and Walter , who, if the treatment technology of today had been available then, would be in their 70s now.
Instead they are remembered as the first aHUS patient cohort in research into aHUS, which with other patient research cohorts since, have provided essential insights to benefit aHUS patients both now and in the future.
aHUS Awareness Day is on the 24th September.
Article No. 688
