Nhibitor: Rhucin, a recombinant C1INH, is currently in development. It
Nhibitor: Rhucin, a recombinant C1INH, is currently in development. It is likely to be an alternative to pdC1INH for the treatment of acute attacks. It has a shorter half-life than pdC1INH, therefore its role in prophylaxis is uncertain[41].Patients with acquired C1INH deficiency may also benefit from home therapy. In some, but not all cases, higherConclusion Every patient with HAE should have the immediate means to control an acute attack quickly and effectively, in order to minimise PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/28388412 impact on physical, social andLonghurst et al. Allergy, Asthma Clinical Immunology 2010, 6:22 http://www.aacijournal.com/content/6/1/Page 6 ofeconomic wellbeing to themselves and their family. Similar to the haemophilia home care model that has worked so well for many years[35], the option of home and self-administration offers the prospect of achieving the aim of `each C1 inhibitor deficient (HAE) patient to be able to manage his/her symptoms proactively in such a way that they maintain personal safety and minimal disruption in living a healthy and productive life'[32].Appendix 1. Recommendations: summaryInclusionsHome therapy training should be inclusive. Extremes of age or lack of infusion partner are not necessarily contraindications. Patients with HAE, with acquired angioedema (acquired C1 inhibitor deficiency), and, where treatment is available, HAE3, should be included. Prophylaxis, if required, should be optimised while home therapy training is ongoing.Attack TreatmentPenn State University, Hershey, Pennsylvania, USA. 4Johann Wolfgang Goethe University, Frankfurt/Main, Germany. 5Department of Immunology, Plymouth Hospitals NHS Trust, UK. 6Dept of Internal Medicin, Ryhov County Hospital, SE-55185 J k ing, Sweden. 7Department of Dermatology, University Hospital of the Johannes Gutenberg-University of Mainz, Mainz, Germany. 8Department of Medicine, CHU de Grenoble, Grenoble, France. 9 Executive Director, HAE International, Denmark. 10Department of Dermatology and Allergy Centre, Odense University Hospital, Denmark. 11 Hospital La Paz Health Research Institute, Madrid, Spain. 12Department of Internal Medicine, Universita degli Studi di Milano, Ospedale L. Sacco, Milan, Italy. 13Department of Immunology, Barts and the London NHS Trust, London, UK. 14Department of Immunology, Southmead PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/27663262 Hospital, Bristol, UK. 15 Department of Immunology, St James’ Hospital, Leeds, UK. 16Department of Immunology, Barts and the London NHS Trust, London, UK. 17HAE association, Germany. 18Johann Wolfgang Goethe University, Frankfurt/Main, Germany. 19Academic Medical Center, University of Amsterdam, Amsterdam, Netherlands. 20US HAEA Executive Vice President; US HAEA Patient Registry, USA. 21Johann Wolfgang Goethe University, Frankfurt/Main, Germany. 22HAE Association, France. 23Tel Hashomer, and Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel. 24Departments of Medicine and Paediatrics, University of Calgary, Calgary, Alberta, Canada. 25University of California, San Diego, San Diego, California, USA. Authors’ contributions HL chaired home therapy debates and facilitated consensus at the Budapest workshop and Toronto CHAEN meetings. TC, JL, WK, BZ acted as faciliators/ expert panel at the debate at the Budapest workshop. HL Naramycin AMedChemExpress Cycloheximide prepared the manuscript. All authors have read, revised and approved the manuscript and have participated in one or more of the debates, or in the subsequent discussions. Competing interests Many of the authors have either entered consu.