Abstracts from the 10th C1-inhibitor deficiency workshop
Bibliographic record
Abstract
The 10th C1-inhibitor deficiency workshop will be held between 18 and 21 May 2017 in Budapest (2017.haenetworkshop.hu),among the picturesque surroundings of Margaret Island.As indicated by the name of this event, most of the interest focused on angioedema due to C1-inhibitor deficiency in 1999, when it was first organized.The name is unchanged, but the range of angioedemas has expanded since to include all known varieties of hereditary and acquired angioedemas with a bradykinin-mediated pathomechanism.Looking back to the agenda of this biennial conference, many questions remained unanswered and new issues have arisen despite the enormous scientific progress made.On this occasion, 318 participants have registered from 42 countriesthis is the greatest attendance in the history of the Workshop since the start of the series.Eighty-six presentations have been submitted for this 4-day long scientific forum.The scientific program sounds interesting-it comprises novel achievements by leading scientific teams in basic research into bradykinin-mediated angioedema, the new findings of diagnostics and genetics, promising therapeutic solutions awaiting introduction, and the experience accumulated with the latest therapeutic procedures.Several presentations discuss the efforts related to improving the patients' quality of life.This time, we have invited five prominent experts-namely, Alvin Schmaier (Cleveland, OH, USA), Marco Cicardi (Milan, Italy), Avner Reshef (Tel-Hashomer, Israel), Dumitru Moldovan (Tirgu-Mures, Romania) and Attila Mócsai (Budapest, Hungary).Alvin Schmaier will show us that our knowledge about the underlying mechanisms of bradykinin-mediated angioedemas is still limited-this may be remedied by extending our interest to other forms of angioedema with different pathophysiological backgrounds.Marco Cicardi will expose the similarities and the differences between bradykinin-mediated edema formation, and the idiopathic systemic capillary leak syndrome.Avner Reshef will explore a similar issue in his presentation titled 'Angioedema-Histamine or Bradykinin?' .The lecture on neutrophil granulocytes by Attila Mócsai will take us closer to understanding the pathomechanism of angioedema.The agenda also contains the traditional roundtable session, an opportunity to develop consensus and international guidelines-this year, genetics will be in the limelight.In this session, the keynote lectures will be read by Margarita-Lopez Trascasa (Madrid, Spain) on the extended diagnostic approach integrating serological and genetic methodology; by Anastasios Germenis (Larissa, Greece) on the latest techniques for studying the SERPING1 gene; and by Nancy Brown (Nashville, TN, USA) on the pharmacogenetics of angiotensin-converting enzyme inhibitor-associated angioedema.Notwithstanding the remarkable progress made in South-America and in the former Soviet-bloc countries of Europe, state-of-the-art diagnostic and therapeutic modalities are still not available in many regions of the World.Dumitru Moldovan will review the stages along the way to making these accessible, and the experience accumulated in the effort to achieve high levels of patient care.The conference will be attended both by researchers and by clinicians-medical professionals and nurses, by the representatives of patient organizations, and by pharmaceutical industry experts involved in drug development, in order to assist the efforts of each other through joint thinking.Within the framework of this fruitful cooperation, the pharmaceutical companies also lent financial support to the conference-in addition to their scientific contribution.The travel grants, make it possible for an increasing number of professionals involved in the research or the management of patients with angioedema to attend the Workshop.The generous support by our Sponsors enabled us again to present the "For HAE Patients" award, as well as the "Grant for Young Investigators".The major donors to this event are CSL Behring and Shire.Pharming Group NV, Swedish Orphan Biovitrum, BioCryst Phamaceuticals, KininX SAS also contributed the sponsorship of the Workshop.On occasion of this tenth, jubilee event, the "For HAE Patients" award goes to Bruce Zuraw (San Diego, USA), who will present his lecture 'Let the Treatment Fit the Disease' on the festive session of the scientific section on Day 1.His achievements in bettering the management of patients will be recalled by Antony Castaldo, the chair of the International Patient Organization for C1 Inhibitor Deficiencies.The concluding event of the conference will be the awarding of the 'Grant for Young Investigators' to the top four young presenters.The support referred to above made it possible to publish the submitted abstracts of the Workshop in the journal Allergy, Asthma, and Clinical Immunology, in order to make them available to an even broader range of professionals interested in this subject.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.002 | 0.004 |
| Meta-epidemiology (narrow) | 0.002 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.004 | 0.002 |
| Open science | 0.001 | 0.003 |
| Research integrity | 0.002 | 0.003 |
| Insufficient payload (model declined to judge) | 0.157 | 0.098 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".