Hepatic Function and Fibrinolysis in Patients with Hereditary Angioedema Undergoing Long-Term Treatment with Tranexamic Acid

Prophylactic treatment with antifibrinolytic agents, epsilon-aminocapriod and tranexamic acid, reduces the incidence and severits of attacks in patients with hereditary angioedema. Long-term ellectivenessor risk of antifibrinolytic agents has not been established. Sixteen patients needing continuous prophylaxis because of frequency and severity of attacks were treated with tranexamic acid. In four patients this treatment was ineffective and the drug was withdrawn after 2 months. A remission or reduction in the frequency or serverity of attacks was observed in 12 patients treated for a period ranging from 8 to 34 months. Hepatic tests and blood fibrinolytic activity were not influenced by long term oral treatment with tranexamic acid.     

Impact of anxiety,stress and depression related to COVID-19 pandemic on the course of hereditary angioedema with C1-inhibitor deficiency

Background

Hereditary angioedema (HAE) attacks can be provoked with psychological factors. The aim of this study was to assess the effects of anxiety, depression and stress related to COVID-19 pandemic on disease activity of HAE patients during the quarantine period (QP) and the return to normal period (RTNP).

Methods

This study was conducted between March 2020 and September 2020 in four allergy centres. Demographic, clinical features and mental health status were evaluated in QP (from March to the beginning of June) and RTNP (from June to the beginning of September) applied by the government. The 10-point visual analogue scale (VAS10) was used to define the severity of HAE attacks. Depression, Anxiety and Stress Scales-21 (DASS-21) and Fear of COVID-19 (FC-19) scale were performed to assess mental health status.

Results

139 HAE patients were included in the study. In QP, median attack numbers and median VAS10 scores were 5 (min-max: 0–45) and 6 (min-max: 0–10), respectively. HAE attack numbers, DASS-21 stress, anxiety, depression and total DASS-21 scores, and FC-19 scores were higher in QP than RTNP (p = 0.001, p < 0.001, p = 0.001, p < 0.001, p < 0.001, p < 0.001, respectively). However, there was no difference in attack severity scores between the two periods (p > 0.05).

Conclusions

This study revealed that the restriction measures during COVID-19 outbreak cause an increase in the number of HAE attacks in relation to anxiety, depression, stress and fear of COVID-19 pandemic. Therefore, it is important to provide psychological support to HAE patients during the pandemic.

     

Morphologic evaluation of the liver in hereditary angioedema patients on long-term treatment with androgen derivatives

17 alpha-Alkylated androgens are highly effective in preventing attacks in HAE patients. These drugs, however, seem to be implicated in the development of cholestatic jaundice, peliosis hepatis, and liver tumors. In order to assess the risk-benefit balance of the long-term therapy with androgen derivatives, a follow-up investigation was performed in 13 HAE patients. The results of this study indicate that long-term treatment (15 to 47 mo) with low doses of danazol or stanozolol does not induce significant hepatic damage detectable by laboratory tests or liver biopsy. However, the limited number of patients, although in a rather long period of observation, still suggests a careful control and the use of minimal effective doses.     

Angiotensin-converting enzyme inhibitor-related angioedema: how to deal with it

Angiotensin-converting enzyme inhibitor-related angioedema is a well documented condition, which seems to occur in up to 1% of treated patients. It represents a problem for both the clinician and the patient: for the clinician, the diagnosis may be difficult due to its peculiar clinical characteristics, whereas for the misdiagnosed patient the delay prolongs a potentially dangerous situation. If the drug is not discontinued, the attacks tend to become worse and even life-threatening. There are now evidences that increased levels of bradykinin have an important role in the pathophysiology of attacks and, moreover, there are genetic factors that render certain individuals susceptible to angiotensin-converting enzyme inhibitor-related angioedema. In this review, the authors analyse the pathogenetic mechanism, the clinical presentation, the management and future perspectives of research on this condition.     

C1 Esterase Inhibitor for Ace-Inhibitor Angioedema: A Case Series and Literature Review

BackgroundAngiotensin-converting enzyme (ACE) inhibitors are a commonly prescribed and effective medication to treat hypertension. Although generally well tolerated, about 1% of patients will experience angioedema, a potentially life-threatening adverse drug reaction. This reaction is thought to be mediated via a buildup of bradykinin and does not typically respond to epinephrine, corticosteroids, or antihistamines. Alternative treatment strategies have been investigated, the bulk of which surround the use of therapies approved for hereditary angioedema.ObjectiveUtilization of C1 esterase inhibitors at our institution was reviewed between 2016 and 2018 for treatment of ACE inhibitor–induced angioedema. We describe the clinical course of case series, along with a review of current literature.DiscussionUtilization of C1 esterase inhibitors for treating ACE inhibitor–induced angioedema is an uncommon therapy and has limited efficacy in our case series. Treatment did not result in rapid improvement of swelling, prevention of intubation, or prevention of intensive care unit admission.ConclusionsBased on our case series, C1 esterase therapy should not be utilized routinely for ACE inhibitor–induced angioedema and is not expected to prevent intubation in severe cases.     

乙酰半胱氨酸注射液致血管性水肿1例分析

目的探讨乙酰半胱氨酸注射液致血管性水肿的原因及治疗方法。方法分析吉林大学第一医院血管外科2018年9月收治的1例动脉硬化闭塞症患者因注射乙酰半胱氨酸注射液致血管性水肿的可能性及治疗方法。结果 1例65岁女性患者因左下肢发凉、麻木、疼痛1年入院。入院第1天给予乙酰半胱氨酸注射液8 g+5%葡萄糖溶液250 mL静脉滴注,注射该药后约1小时20分钟,患者出现面部水肿、全身皮疹等血管性水肿症状,立即停药并给予抗过敏治疗,对症治疗后1 h患者上述症状消失,入院第14天病情好转出院。结论乙酰半胱氨酸注射液很可能引起该患者血管性水肿。注射该药物时应注意减小滴速,谨慎用于有药物过敏史、哮喘的患者,同时注射时应有专人陪护并注意观察。