According to the newspaper, the phoenix trees and poplar trees on the streets of Hangzhou are just about to move, and the flock season is coming as scheduled, which is also the high incidence season of allergic dermatitis and skin redness.

Recently, the outpatient volume of allergy departments in major hospitals in Hangzhou has increased sharply.

　　"Repeated 'entanglement' of allergies and edema, and the ineffectiveness of conventional medicines, may not be a simple allergic disease." Professor Wang Huiying, deputy director of the Department of Allergy at the Second Affiliated Hospital of Zhejiang University School of Medicine, reminded that at this time, we need to be alert to an easy A rare disease that is misdiagnosed and mistreated—hereditary angioedema (HAE).

　　Dangerous diseases disguised under "masks"

　　Hereditary angioedema is good at "camouflage", and some patients show skin edema, which is often misdiagnosed as allergy; some patients show abdominal pain, which is easily misdiagnosed as gastroenteritis and appendicitis.

Professor Cao Qian, Director of the Department of Gastroenterology, Run Run Shaw Hospital Affiliated to Zhejiang University School of Medicine, said: "If hereditary angioedema affects the digestive system, it mainly manifests as severe abdominal pain, nausea, vomiting, and sometimes diarrhea and constipation. These symptoms are closely related to the digestive tract. It is easy to confuse patients with acute abdomen. However, when there is a long course of disease, repeated attacks, and other symptoms such as skin and mucous membrane edema, chest tightness, shortness of breath, and upper respiratory tract involvement, one should be alert to the possibility of HAE.”

　　Even more frightening is that although the global prevalence is only one in 50,000, hereditary angioedema is "deadly".

According to statistics, 58.9% of patients with hereditary angioedema in my country have experienced laryngeal edema. This process sometimes progresses very rapidly. If rescue is not timely, patients can die from asphyxiation within 4.6 hours, and the highest fatality rate can reach 40%.

　　"See through" HAE, "snipe" rare edema

　　In China, the diagnosis rate of hereditary angioedema is estimated to be less than 5%, and it takes an average of 13 years from the onset of the patient to a clear diagnosis.

Professor Wang Huiying emphasized: "On the one hand, it is necessary to strengthen the popular science education of HAE diseases to improve public awareness; The group's ability to differentially diagnose HAE, such as serological tests and genetic tests. In addition, it is necessary to focus on the patient's family history and track the progress of the patient's diagnosis and treatment."

　　Nowadays, it is not difficult to "see through" HAE and "snipe" rare edema.

　　For patients with high suspicion of HAE, preliminary screening can be performed by complement C4 detection, and then the level of C1 esterase inhibitor (C1-INH) can be detected, which can effectively reduce the rate of misdiagnosis and missed diagnosis.

At present, many hospitals in the province, including the Second Affiliated Hospital of Zhejiang University School of Medicine, have carried out C1-INH testing projects, which have perfect conditions for the diagnosis and treatment of HAE diseases.

　　For patients diagnosed with HAE, Professor Wang Huiying suggested: "Acute attacks of hereditary angioedema need the guidance of a doctor, rush to the emergency room as soon as possible, and take medication as soon as possible. In order to deal with the risk of acute edema, all HAE patients should always have at least two emergency medicines around them. , to relieve the pain in time.”

　　Making the "Key to Hope" more accessible

　　The ultimate goal of HAE treatment is to achieve complete control of the disease and normalization of the patient's life, meaning zero episodes.

　　"It is gratifying that my country has introduced innovative drugs for the long-term prevention and treatment of HAE and acute attacks." Xie Junming, member of the National Health Commission's Rare Disease Diagnosis and Protection Expert Committee and chairman-designate of the Rare Disease Branch of Zhejiang Medical Association According to the professor, lanarizumab is the only drug in the world for the prevention and treatment of hereditary angioedema, which can effectively reduce the number of repeated episodes of edema in patients and prevent fatal asphyxia.

In addition, icatibant acetate, a drug used to treat acute attacks of hereditary angioedema, has been successfully included in the National Medical Insurance List and will be officially implemented on January 1, 2022.

　　"In recent years, the state has paid great attention to rare diseases and patient groups. Many rare disease treatment drugs, especially high-value rare disease drugs, have been included in the national medical insurance, so that more rare disease patients have hope of regaining a healthy life. Emergency medicines for HAE patients Inclusion in the national medical insurance has effectively solved the urgent treatment needs of the patient group.” Professor Xie Junming believes that the next step is to focus on the implementation of medical insurance for drugs, and to ensure that hospitals and general hospitals specializing in the treatment of HAE can always stock HAE acute attack treatment drugs. A weapon in the hands of a doctor.