This article is taken from an extensive feature on the 2023 HAEi Regional Conference APAC, published in Global Perspectives #1 2023

By Deborah Corcoran, Chief, Special Projects and Research

The 2023 HAEi Regional Conference APAC track for healthcare professionals was planned by the Co-Chairs, Dr. Hilary Longhurst from Auckland City Hospital, Auckland, New Zealand, and Dr. Hiroshi Chantaphakul from Bumrungrad International Hospital, Bangkok, Thailand.

Friday 17 March 2023
Following the Conference opening, welcome and Dr. Lis’s presentation in the main room, all healthcare professionals and researchers left the main auditorium for the Scientific Poster Session, which was open to all research conducted in the Asia Pacific region on the topic of HAE (summary of posters on page 33).

Saturday 18 March 2023
The second day commenced with a welcome to attendees given by the Regional Conference Co-Chairs, Dr. Hilary Longhurst of Auckland City Hospital in New Zealand, and Dr. Hiroshi Chantaphakul from Bumrungrad International Hospital, Thailand.

As the recipient was unfortunately unable to attend the Regional Conference in person, Mr. Xiaogang Qi of HAE China received the award on behalf of Dr. Zeijan Zhang.

Following the Co-Chairs’ introduction, a plenary talk was given by Dr. Sira Nanthapisal of Thammasat University, Thailand. Dr. Nanthapisal gave an update on the progress made in how HAE is managed in Thailand by outlining the strategic plan being followed, and key initiatives undertaken, including developing Thai HAE Guidelines and a social media campaign to improve public and healthcare professional awareness of the disease.

The Co-Chairs then presented a series of fictitious clinical cases for discussion. Four cases were shown, which followed patients of different ages and gender over a number of years. Delegates were invited to vote on the best management course at key points, such as an increase in attacks, switching of prophylaxis, and patients attending the emergency room. The cases promoted much discussion and were well received by delegates.

After a short coffee break, delegates returned to a further plenary talk delivered by Dr. Ankur Jindal of the Post Graduate Institute of Medical Education and Research, Chandigarh, India. Dr. Jindal spoke on progress with HAE management in India. Key achievements, in collaboration with the HAE Society of India (HAESI), included the increased availability of facilities for diagnosis and genetic testing, a dedicated society for healthcare professionals, and the availability of plasma-derived C1-INH. Dr. Jindal concluded that while awareness had increased, more could be done; and that a national registry and further dedicated care centers for patients with HAE are expected soon.

At this point, delegates heard from oral presenters of selected abstracts.

The first abstract (‘N-glycome signatures are potential biomarkers for hereditary angioedema diagnosis and stratification’) was delivered by Dr. Zeijan Zhang, winner of the Young Researcher/Investigator Award, via a pre-recorded video presentation. Dr. Zhang’s research focused on discovering new biomarkers of HAE to improve options for diagnosis, especially in people with HAE with normal C1-INH. Dr. Zhang indicated that her research showed the full plasma N-glycomic signature of HAE for the first time and that changes in N-glycosylation that are specific to HAE could be used for diagnosis, prediction of disease severity, and monitoring.

Dr. Sanghamitra Macchua of the Postgraduate Institute of Medical Education and Research, Chandigarh, India, delivered the next oral presentation. The research, entitled ‘Transmission patterns in C1-INH deficiency hereditary angioedema favors a wild-type male offspring: our experience at Chandigarh, India’, looked at the risk of transmission of SERPING 1 gene mutation (which causes deficiency of C1-INH protein) from either father or mother to children. The researchers found no statistical significance between the transmission rates between fathers to female children or mothers to either male or female children. Dr. Macchua concluded that the transmission patterns indicated that normal genes were slightly more likely to come from the father and that further research is needed to explain why this is so.

Professor Marc Riedl of the US HAEA Angioedema Center in San Diego, United States, spoke next. He presented data from a study entitled ‘Efficacy And Safety Of Bradykinin B2 Receptor Inhibition With Oral PHVS416 In Treating Hereditary Angioedema Attacks: Results Of RAPIDe-1 Phase 2 Trial’. The research investigated a new bradykinin B2 receptor antagonist under development to treat and prevent HAE attacks. Prof. Riedl indicated that this study is of an investigational oral, soft-gel capsule formulation of the medicine called PHVS416 for use in treating attacks in people with HAE due to C1-INH deficiency. The presented data demonstrated a significant reduction in attack symptoms in patients taking the trial medicine compared to those taking a dummy pill or placebo. In those taking the investigational medication, there were three treatment-related adverse events. In the placebo group, one treatment-related adverse event was recorded. Prof. Riedl concluded that results from this research provided evidence to support the efficacy and safety of PHVS416 in treating HAE attacks and as a potential on-demand therapy.

The final oral presentation (‘Genetic profile of patients with hereditary angioedema at a tertiary care referral hospital in North India’) was given by Ms. Sanchi Chawla of the Postgraduate Institute of Medical Education and Research in Chandigarh, India. Ms. Chawla told delegates that the objective of her research was studying the genetic landscape of HAE within a tertiary referral hospital in India, as there is a lack of literature on the genetics of HAE in the country. The research conducted genetic studies of patients suspected to have HAE. The results led Ms. Chawla to conclude that the genetic profile of people with HAE in India may be different from that seen in other countries, as the majority of genetic variants were observed on the later parts of the SERPING1 gene. Ms. Chawla suggested that these mutations may influence the presentation of the disease.

The morning session ended with questions from assembled delegates, with responses from the Scientific Committee and other participants.