Hereditary angioedema: beyond international consensus - circa December 2010 - The Canadian Society of Allergy and Clinical Immunology Dr. David McCourtie Lecture

<p>Abstract</p> <p>Background</p> <p>The 2010 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema was published earlier this year in this Journal (Bowen et al. <it>Allergy, Asthma & Clinical Immunology </it>2010, 6:24 - <url>http://www.aacijournal.com/content/6/1/24</url>). Since that publication, there have been multiple phase III clinical trials published on either prophylaxis or therapy of hereditary angioedema and some of these products have changed approval status in various countries. This manuscript was prepared to review and update the management of hereditary angioedema.</p> <p>Objective</p> <p>To review approaches for the diagnosis and management of hereditary angioedema (HAE) circa December 2010 and present thoughts on moving from HAE management from international evidence-based consensus to facilitate more local health unit considerations balancing costs, efficacies of treatments, and risk benefits. Thoughts will reflect Canadian and international experiences.</p> <p>Methods</p> <p>PubMed searches including hereditary angioedema and diagnosis, therapy, management and consensus were reviewed as well as press releases from various pharmaceutical companies to early December 2010.</p> <p>Results</p> <p>The 2010 International Consensus Algorithms for the Diagnosis, Therapy and Management of Hereditary Angioedema is reviewed in light of the newly published phase III Clinical trials for prevention and therapy of HAE. Management approaches and models are discussed.</p> <p>Conclusions</p> <p>Consensus approach and double-blind placebo controlled trials are only interim guides to a complex disorder such as HAE and should be replaced as soon as possible with large phase IV clinical trials, meta analyses, data base registry validation of approaches including quality of life and cost benefit analyses, safety, and head-to-head clinical trials investigating superiority or non-inferiority comparisons of available approaches. Since not all therapeutic products are available in all jurisdictions and since health care delivery approaches and philosophy vary between countries, each health care delivery sector will likely devise their own algorithms based on local practicalities for implementing evidence-based guidelines and standards for HAE disease management. Quality-of-life and cost affordability benefit conclusions will likely vary between countries and health care units. Data base registries for rare disorders like HAE should be used to detect early adverse events for new therapies and to facilitate phase IV clinical trials and encourage superiority and non-inferiority comparisons of HAE management approaches.</p

HAE international home therapy consensus document

Hereditary angioedema (C1 inhibitor deficiency, HAE) is associated with intermittent swellings which are disabling and may be fatal. Effective treatments are available and these are most useful when given early in the course of the swelling. The requirement to attend a medical facility for parenteral treatment results in delays. Home therapy offers the possibility of earlier treatment and better symptom control, enabling patients to live more healthy, productive lives. This paper examines the evidence for patient-controlled home treatment of acute attacks ('self or assisted administration') and suggests a framework for patients and physicians interested in participating in home or self-administration programmes. It represents the opinion of the authors who have a wide range of expert experience in the management of HAE

Laparoscopic live donor nephrectomy: the preliminary experience

BackgroundRecent improvements in video technology and surgical instrumentation have resulted in the application of minimally invasive techniques to many surgical procedures including splenectomy and adrenalectomy. Nephrectomy requires a long flank incision with division of abdominal musculature and possible subcostal nerve damage. Severe postoperative pain and a prolonged recuperative period may result, and the cosmetic outcome may not be satisfactory. A new surgical approach utilizing laparoscopic dissection and delivery of the kidney through a small incision was performed to circumvent these problems. The aim of this paper is to describe the technique of laparoscopic live donor nephrectomy (LLDN) and present the preliminary outcome.MethodsOver the 12-month period between May 1997 and April 1998, 16 donors underwent donor nephrectomy by a laparoscopic approach. The procedure was assessed with regard to its safety, feasibility and advantages over the open method.ResultsAll the nephrectomies were completed without conversion to an open procedure. The average postoperative pain score on a visual analogue scale of 1-10 was 2 in LLDN. The donors required 36 mg morphine on average over 36 h postoperatively. Postoperative stay averaged 3 days. One donor developed an infective complication along the wound drain tract which settled with adequate drainage and antibiotics. All the removed donor kidneys were transplanted with immediate good function. There were no surgical complications or graft losses. The recipients' serum creatinine was in the range of 96-181 mmol/L 3 months after transplantation.ConclusionsSignificant potential advantages of LLDN include less postoperative pain, shorter hospitalization and decreased recuperative time. This preliminary experience indicates LLDN to be effective in terms of safety and feasibility.C. Hensman, G. Lionel, P. Hewett and M. Mohan Ra