Fresh Frozen Plasma for the Treatment of Hereditary Angioedema Acute Attacks

Objective To determine the safety and efficacy of fresh frozen plasma （FFP） iniusion for the treat- ment of hereditary angioedema （FIAE）. Methods The medical records of patients with HAE admitted to Peking Union Medical College Fiospital who had received FFP infusion during 2004 and 2010 were reviewed and PubMed database iFom 1966 to the present were searched using the following key words： hereditary angioedema and fresh frozen plasma. The patient＇s age, sex, body location of HAE attacks, the dose of FFP infusion, time of beginning to improvenaent, time to complete remission, complication, C 1 inhibitor activity, and outcome were analyzed. Results A total of 13 enrolled patients （7 male and 6 female） received 16 times of FFP infusion, in- cluding 2 patients undergoing FFP infusion in Peking Union Medical College Hospital and 11 patients re- ported in the literature. The mean dosage of FFP infusion was 586±337 mL. Two cases suffered from wors- ening abdominal pain and one case experienced skin rash. Only I patient had no improvement in symptom owing to transfusion related reaction. There was a definite improvement in symptom 49± 19 minutes after beginning FFP infusion. The remission time decreased from 61.7±27.0 hours to 3.3 （2.0, 12.0） hours after FFP infusion. FFP infusion was effective for both type I and type Ⅱ HAE. Conclusion FFP seems to be safe and effective for acute attacks of HAE.

Fresh Frozen Plasma for the Treatment of a Chinese Patient with Hereditary Angioedema

HEREDITARY angioedema （HAE） is an autosomal dominant inherited condition which was initially described by Osier in 1888.1 Patients with HAE can develop rapid subcutaneous or submucosal edema involving the hands, feet, limbs, face, intestinal tract, even larynx and trachea. The mortality of an acute attack of HAE without treatment was reported as high as 30%.2 HAE is caused by the deficiency of Cl esterase inhibitor （CIINH） which results in episodes of edema in parts of the human body,

Newly Found C1 Inhibitor Gene Mutation in Hereditary Angioedema Patients

Hereditary angioedema （HAE） is an autosomal dominant condition that affects one in about 50 000 persons, characterized by recurrent episodes of subcutaneous or submucosal swelling involving the hands, feet, limbs, face, intestinal tract, even larynx and trachea.

Hereditary Angioedema in Pregnancy and Management without Recombinant Human C1-INH

A pregnant woman diagnosed with type 1 angioedema seeks care at a public hospital for planning the delivery. This report presents ways to prevent and manage an acute HAE crisis during childbirth and early postpartum without the availability of first-line medications, such as plasma-derived human C1-INH concentrate.

Hypersensitivity to Aspirin as a Factor for Poor Control in Hereditary Angioedema

Background: Hereditary angioedema (HAE) is a primary immunodeficiency disorder characterized by C1 complement inhibitor deficiency and unregulated activation of complement. Aspirin hypersensitivity is related to an increase in the amount of leukotrienes with eosinophil and mast cell activation and increased levels of glandular kallikrein with upregulated local conversion of bradykinin. Both conditions can be present in the same patient. Objectives: We present five patients with HAE;they were all being treated in similar ways according to the therapeuthic options available in our institute (danazol). However, three of them had recurrent episodes of angioedema;in these cases, it was identified aspirin hypersensitivity as a cause of poor disease control. A review of the literature is included. Case Presentation: We present the cases of four females and one male (age range 21 - 58 years) with type I HAE. Subjects were all ISSSTE beneficiaries (state workers) treated at the National Medical Center “20 de Noviembre”. Aspirin hypersensitivity was identified in three patients. Elimination of NSAIDs along with dietary elimination of high salicylate-containing foods improved control of angioedema crisis (severe and/ or recurrent episodes). Discussion: Aspirin hypersensitivity was identified as a factor for poor control in our patients with HAE. Such cases improved with dietary elimination of high salicylate-containing foods and avoidance of NSAIDs. Conclusions: This is the first report of patients with HAE and aspirin hypersensitivity as a cause of poor control. We recommend a deliberate search of these comorbidities, especially in cases of poor disease control. Further studies are needed to continue the investigation on this topic.

Perioperative anesthetic management in pediatric hereditary angioedema;case report

Hereditary angioedema is a rare but life-threatening disease, usually resulting from upper respiratory tract traumas and stress. In this case report, we present the management of a 14-year-old female patient who was diagnosed with hereditary angioedema and scheduled to undergo transurethral resection of bladder (TURB) procedure for bladder tumor. She was on prophylactic danazol treatment and prior to the operation the dose of danazol was increased. On the day of the operation, patient was given C1-IHN concentrate and was sedated. In conclusion, hereditary angioedema is a rare disease in which multidisciplinary and aggressive approach during anesthesia would yield successful results.

An update on the genetics and pathogenesis of hereditary angioedema

Hereditary angioedema(HAE)is an uncommon genetic disorder characterized by recurrent episodes of edema involving subcutaneous tissue and submucosa.The pathogenesis of HAE reflects an intricate coordinated regulation of components of complement,kinin and hemostatic pathway.Till date,mutations in 4 different genes have been identified to cause HAE which includes serine protease inhibitor G1(SERPING1),factor XII(F12),plasminogen(PLG)and angiopoietin 1(ANGPT 1).These mutations lead to increased bradykinin 2 receptor mediated signalling via increased production of bradykinin except mutations in ANGPT1 gene that disturbs the cytoskeletal assembly of vascular endothelial cells.In this review we aim to summarize the recent advances in the pathogenesis and genetics of HAE.We also provide an overview of possible future prospects in the identification of new genetic defects in HAE.

以头颈部水肿为表现的遗传性血管性水肿分析

目的 分析以头颈部水肿为主要表现的遗传性血管性水肿的治疗方法,以期对其更加深入的了解。方法 回顾性分析2017年6月~2021年4月于聊城市人民医院耳鼻咽喉头颈外科住院的遗传性血管性水肿患者,其中男1例,女3例,年龄23~55岁。结果 4例患者均来自于同一家族,主要表现为头颈部组织水肿,2例主要以喉头水肿为主,其中1例因喉头水肿多次住院治疗,另2例主要以头颈部皮肤肿胀为主。患者住院后通过输注灭活血浆,病情得到改善。结论 遗传性血管性水肿是一种临床主要表现为皮肤及黏膜反复发作的水肿性疾病,全身多处皮肤黏膜均可发病。遗传性血管性水肿患者需详细询问病史及家族史,并掌握其治疗流程,避免误诊漏诊贻误患者病情。