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Sample records for angioedema hae attacks

  1. The hereditary angioedema burden of illness study in Europe (HAE-BOIS- Europe)

    DEFF Research Database (Denmark)

    Bygum, Anette; Aygören-Pürsün, Emel; Caballero, Teresa

    2012-01-01

    ABSTRACT: BACKGROUND: Hereditary angioedema (HAE) is a rare but serious disease marked by swelling attacks in the extremities, face, trunk, airway, or abdominal areas that can be spontaneous or the result of trauma and other triggers. It can be life-threatening due to the risk of asphyxiation...... of HAE-I or HAE-II. Data collection includes: (i) a survey on individuals' health care resource use, direct and indirect medical costs, impact on work and school, treatment satisfaction, and emotional functioning (via the Hospital Anxiety and Depression Scale); and (ii) one-on-one interviews to collect...

  2. Angioedema attacks in patients with hereditary angioedema : Local manifestations of a systemic activation process

    NARCIS (Netherlands)

    Hofman, Zonne L M; Relan, Anurag; Zeerleeder, Sacha; Drouet, Christian; Zuraw, Bruce; Hack, C. Erik

    Hereditary angioedema (HAE) caused by a deficiency of functional C1-inhibitor (C1INH) becomes clinically manifest as attacks of angioedema. C1INH is the main inhibitor of the contact system. Poor control of a local activation process of this system at the site of the attack is believed to lead to

  3. Angioedema attacks in patients with hereditary angioedema: Local manifestations of a systemic activation process

    NARCIS (Netherlands)

    Hofman, Zonne L. M.; Relan, Anurag; Zeerleder, Sacha; Drouet, Christian; Zuraw, Bruce; Hack, C. Erik

    2016-01-01

    Hereditary angioedema (HAE) caused by a deficiency of functional C1-inhibitor (C1INH) becomes clinically manifest as attacks of angioedema. C1INH is the main inhibitor of the contact system. Poor control of a local activation process of this system at the site of the attack is believed to lead to

  4. Risk of angioedema following invasive or surgical procedures in HAE type I and II – the natural history

    Science.gov (United States)

    Aygören-Pürsün, E; Martinez Saguer, I; Kreuz, W; Klingebiel, T; Schwabe, D

    2013-01-01

    Background Hereditary angioedema (HAE), caused by deficiency in C1-inhibitor (C1-INH), leads to unpredictable edema of subcutaneous tissues with potentially fatal complications. As surgery can be a trigger for edema episodes, current guidelines recommend preoperative prophylaxis with C1-INH or attenuated androgens in patients with HAE undergoing surgery. However, the risk of an HAE attack in patients without prophylaxis has not been quantified. Objectives This analysis examined rates of perioperative edema in patients with HAE not receiving prophylaxis. Methods This was a retrospective analysis of records of randomly selected patients with HAE type I or II treated at the Frankfurt Comprehensive Care Centre. These were examined for information about surgical procedures and the presence of perioperative angioedema. Results A total of 331 patients were included; 247 underwent 700 invasive procedures. Of these procedures, 335 were conducted in 144 patients who had not received prophylaxis at the time of surgery. Categories representing significant numbers of procedures were abdominal (n = 113), ENT (n = 71), and gynecological (n = 58) procedures. The rate of documented angioedema without prophylaxis across all procedures was 5.7%; in 24.8% of procedures, the presence of perioperative angioedema could not be excluded, leading to a maximum potential risk of 30.5%. Predictors of perioperative angioedema could not be identified. Conclusion The risk of perioperative angioedema in patients with HAE type I or II without prophylaxis undergoing surgical procedures ranged from 5.7% to 30.5% (CI 3.5–35.7%). The unpredictability of HAE episodes supports current international treatment recommendations to consider short-term prophylaxis for all HAE patients undergoing surgery. PMID:23968383

  5. The hereditary angioedema burden of illness study in Europe (HAE-BOIS-Europe: background and methodology

    Directory of Open Access Journals (Sweden)

    Bygum Anette

    2012-04-01

    Full Text Available Abstract Background Hereditary angioedema (HAE is a rare but serious disease marked by swelling attacks in the extremities, face, trunk, airway, or abdominal areas that can be spontaneous or the result of trauma and other triggers. It can be life-threatening due to the risk of asphyxiation. While there have been major advancements in our understanding of the immunogenetics of HAE, there are significant gaps in the literature regarding understanding of the humanistic and economic impact of the disease, particularly in Europe. The purpose of the HAE Burden of Illness Study-Europe (HAE-BOIS-Europe, the development and methodology of which is described here, is to better understand the management and impact of HAE from the patient perspective in Europe. Methods/Design This is a cross-sectional study in which retrospective data were also collected being conducted in Denmark, Germany and Spain. The study is open to patients ages 12 and older with a diagnosis of HAE-I or HAE-II. Data collection includes: (i a survey on individuals’ health care resource use, direct and indirect medical costs, impact on work and school, treatment satisfaction, and emotional functioning (via the Hospital Anxiety and Depression Scale; and (ii one-on-one interviews to collect detailed descriptive data and patient testimonials on the impact of HAE on patients’ health-related quality of life. Discussion The present manuscript describes the development and plans for implementing a multi-country European study with the aim of characterizing the humanistic and economic burden of HAE from the patient perspective. This study will help raise awareness of HAE as a rare but debilitating condition with wide-ranging impacts.

  6. The hereditary angioedema burden of illness study in Europe (HAE-BOIS-Europe): background and methodology.

    Science.gov (United States)

    Bygum, Anette; Aygören-Pürsün, Emel; Caballero, Teresa; Beusterien, Kathleen; Gholizadeh, Shadi; Musingarimi, Patience; Wait, Suzanne; Boysen, Henrik

    2012-04-26

    Hereditary angioedema (HAE) is a rare but serious disease marked by swelling attacks in the extremities, face, trunk, airway, or abdominal areas that can be spontaneous or the result of trauma and other triggers. It can be life-threatening due to the risk of asphyxiation. While there have been major advancements in our understanding of the immunogenetics of HAE, there are significant gaps in the literature regarding understanding of the humanistic and economic impact of the disease, particularly in Europe. The purpose of the HAE Burden of Illness Study-Europe (HAE-BOIS-Europe), the development and methodology of which is described here, is to better understand the management and impact of HAE from the patient perspective in Europe. This is a cross-sectional study in which retrospective data were also collected being conducted in Denmark, Germany and Spain. The study is open to patients ages 12 and older with a diagnosis of HAE-I or HAE-II. Data collection includes: (i) a survey on individuals' health care resource use, direct and indirect medical costs, impact on work and school, treatment satisfaction, and emotional functioning (via the Hospital Anxiety and Depression Scale); and (ii) one-on-one interviews to collect detailed descriptive data and patient testimonials on the impact of HAE on patients' health-related quality of life. The present manuscript describes the development and plans for implementing a multi-country European study with the aim of characterizing the humanistic and economic burden of HAE from the patient perspective. This study will help raise awareness of HAE as a rare but debilitating condition with wide-ranging impacts.

  7. Open-label, multicenter study of self-administered icatibant for attacks of hereditary angioedema

    DEFF Research Database (Denmark)

    Aberer, W; Maurer, M; Reshef, A

    2014-01-01

    Historically, treatment for hereditary angioedema (HAE) attacks has been administered by healthcare professionals (HCPs). Patient self-administration could reduce delays between symptom onset and treatment, and attack burden. The primary objective was to assess the safety of self-administered ica......Historically, treatment for hereditary angioedema (HAE) attacks has been administered by healthcare professionals (HCPs). Patient self-administration could reduce delays between symptom onset and treatment, and attack burden. The primary objective was to assess the safety of self...

  8. Angioedema attacks in patients with hereditary angioedema: Local manifestations of a systemic activation process.

    Science.gov (United States)

    Hofman, Zonne L M; Relan, Anurag; Zeerleder, Sacha; Drouet, Christian; Zuraw, Bruce; Hack, C Erik

    2016-08-01

    Hereditary angioedema (HAE) caused by a deficiency of functional C1-inhibitor (C1INH) becomes clinically manifest as attacks of angioedema. C1INH is the main inhibitor of the contact system. Poor control of a local activation process of this system at the site of the attack is believed to lead to the formation of bradykinin (BK), which increases local vasopermeability and mediates angioedema on interaction with BK receptor 2 on the endothelium. However, several observations in patients with HAE are difficult to explain from a pathogenic model claiming a local activation process at the site of the angioedema attack. Therefore we postulate an alternative model for angioedema attacks in patients with HAE, which assumes a systemic, fluid-phase activation of the contact system to generate BK and its breakdown products. Interaction of these peptides with endothelial receptors that are locally expressed in the affected tissues rather than with receptors constitutively expressed by the endothelium throughout the whole body explains that such a systemic activation process results in local manifestations of an attack. In particular, BK receptor 1, which is induced on the endothelium by inflammatory stimuli, such as kinins and cytokines, meets the specifications of the involved receptor. The pathogenic model discussed here also provides an explanation for why angioedema can occur at multiple sites during an attack and why HAE attacks respond well to modest increases of circulating C1INH activity levels because inhibition of fluid-phase Factor XIIa and kallikrein requires lower C1INH levels than inhibition of activator-bound factors. Copyright © 2016 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  9. Hereditary Angioedema Attacks: Local Swelling at Multiple Sites.

    Science.gov (United States)

    Hofman, Zonne L M; Relan, Anurag; Hack, C Erik

    2016-02-01

    Hereditary angioedema (HAE) patients experience recurrent local swelling in various parts of the body including painful swelling of the intestine and life-threatening laryngeal oedema. Most HAE literature is about attacks located in one anatomical site, though it is mentioned that HAE attacks may also involve multiple anatomical sites simultaneously. A detailed description of such multi-location attacks is currently lacking. This study investigated the occurrence, severity and clinical course of HAE attacks with multiple anatomical locations. HAE patients included in a clinical database of recombinant human C1-inhibitor (rhC1INH) studies were evaluated. Visual analog scale scores filled out by the patients for various symptoms at various locations and investigator symptoms scores during the attack were analysed. Data of 219 eligible attacks in 119 patients was analysed. Thirty-three patients (28%) had symptoms at multiple locations in anatomically unrelated regions at the same time during their first attack. Up to five simultaneously affected locations were reported. The observation that severe HAE attacks often affect multiple sites in the body suggests that HAE symptoms result from a systemic rather than from a local process as is currently believed.

  10. The Icatibant Outcome Survey: treatment of laryngeal angioedema attacks

    Science.gov (United States)

    Aberer, Werner; Bouillet, Laurence; Caballero, Teresa; Maurer, Marcus; Fabien, Vincent; Zanichelli, Andrea

    2016-01-01

    Objective To characterize the management and outcomes of life-threatening laryngeal attacks of hereditary angioedema (HAE) treated with icatibant in the observational Icatibant Outcome Survey (NCT01034969) registry. Methods This retrospective analysis was based on data from patients with HAE type I/II who received healthcare professional-administered or self-administered icatibant to treat laryngeal attacks between September 2008 and May 2013. Results Twenty centers in seven countries contributed data. Overall, 42 patients with HAE experienced 67 icatibant-treated laryngeal attacks. Icatibant was self-administered for 62.3% of attacks (healthcare professional-administered, 37.7%). One icatibant injection was used for 87.9% of attacks, with rescue or concomitant medication used for 9.0%. The median time to treatment was 2.0 h (n=31 attacks) and the median time to resolution was 6.0 h (n=35 attacks). Conclusions This analysis describes successful use of icatibant for the treatment of laryngeal HAE attacks in a real-world setting. PMID:27116379

  11. Treatment of HAE Attacks in the Icatibant Outcome Survey

    DEFF Research Database (Denmark)

    Hernández Fernandez de Rojas, Dolores; Ibañez, Ethel; Longhurst, Hilary

    2015-01-01

    were performed (February 2008 to December 2012). RESULTS: Icatibant was used in 652 attacks in 170 patients with HAE type I/II. Most icatibant injections were self-administered (431/652, 68.5%). The proportion of self-treated attacks increased over time (40.3% in 2009 vs. 89.7% in 2012). The median......-administration of icatibant provides a complementary option to HCP administration, enabling optimization of patient care. © 2015 S. Karger AG, Basel....

  12. Breakthrough attacks in patients with hereditary angioedema receiving long-term prophylaxis are responsive to icatibant

    DEFF Research Database (Denmark)

    Aberer, Werner; Maurer, Marcus; Bouillet, Laurence

    2017-01-01

    BACKGROUND: Patients with hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE) experience recurrent attacks of cutaneous or submucosal edema that may be frequent and severe; prophylactic treatments can be prescribed to prevent attacks. However, despite the use of long......-term prophylaxis (LTP), breakthrough attacks are known to occur. We used data from the Icatibant Outcome Survey (IOS) to evaluate the characteristics of breakthrough attacks and the effectiveness of icatibant as a treatment option. METHODS: Data on LTP use, attacks, and treatments were recorded. Attack...... characteristics, treatment characteristics, and outcomes (time to treatment, time to resolution, and duration of attack) were compared for attacks that occurred with versus without LTP. RESULTS: Data on 3228 icatibant-treated attacks from 448 patients with C1-INH-HAE were analyzed; 30.1% of attacks occurred while...

  13. Psychometric Field Study of Hereditary Angioedema Quality of Life Questionnaire for Adults: HAE-QoL.

    Science.gov (United States)

    Prior, Nieves; Remor, Eduardo; Pérez-Fernández, Elia; Caminoa, Magdalena; Gómez-Traseira, Carmen; Gayá, Francisco; Aabom, Anne; Aberer, Werner; Betschel, Stephen; Boccon-Gibod, Isabelle; Bouillet, Laurence; Bygum, Anette; Csuka, Dorottya; Farkas, Henriette; Gomide, Maria; Grumach, Anete; Leibovich, Iris; Malbran, Alejandro; Moldovan, Dumitru; Mihaly, Eniko; Obtulowicz, Krystyna; Perpén, Cecilia; Peveling-Oberhag, Adriane; Porebski, Grzegorz; Chavannes, Celine Rayonne; Reshef, Avner; Staubach, Petra; Wiednig, Michaela; Caballero, Teresa

    2016-01-01

    Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) may affect health-related quality of life (HRQoL). A specific HRQoL questionnaire for adult patients with C1-INH-HAE, the HAE-QoL, has recently been developed in Spain. The objective of this study was to perform a cross-cultural validation and psychometric study of the HAE-QoL in an international setting. Cross-cultural adaptation of the Spanish HAE-QoL draft version and an international rating phase with experts were performed. The resultant version of the HAE-QoL, a clinical questionnaire, and Short Form 36-item Health Survey Version 2.0 (SF-36v2) were pilot tested internationally. Item reduction was based on both descriptive and exploratory factor analysis. Psychometric properties were assessed. Cross-cultural adaptation of the HAE-QoL was performed in 18 countries. The draft version of the HAE-QoL was pilot tested in 332 patients, and accurate data were obtained from 290 patients from 11 countries. The reduction process resulted in a new version with 25 items and 7 dimensions (treatment difficulties, physical functioning and health, disease-related stigma, emotional role and social functioning, concern about offspring, perceived control over illness, and mental health). Strong psychometric properties were observed (Cronbach's α 0.92; test-retest reliability 0.87). Convergent validity showed mild to moderate correlations with SF-36v2 physical and mental component summaries (0.45 and 0.64, respectively) and with SF-36v2 dimensions (P < .004). HAE-QoL scores discriminated significantly among severity groups (median: asymptomatic 133.5 vs severe 84.0; P < .001); between patients with and without long-term prophylaxis (median: 101 vs 90; P = .001); and between patients with and without psychiatric and/or psychological care (median: 74 vs 103; P ≤ .001). The HAE-QoL, currently available in 18 languages, showed good reliability and validity evidence. Copyright © 2016 American Academy of Allergy

  14. Hereditary angioedema

    Science.gov (United States)

    ... disease; HAE- Hereditary angioedema; Kallikrein inhibitor-HAE: bradykinin receptor antagonist-HAE; C1-inhibitors-HAE; Hives-HAE ... aunt, uncle, or grandparent. Dental procedures, sickness (including colds and the flu), and surgery may trigger HAE ...

  15. Activation of the ficolin-lectin pathway during attacks of hereditary angioedema

    DEFF Research Database (Denmark)

    Csuka, Dorottya; Munthe-Fog, Lea; Hein, Estrid

    2014-01-01

    BACKGROUND: The activation of plasma enzyme systems is insufficiently controlled in hereditary angioedema due to the deficiency of C1-inhibitor (C1-INH) (HAE-C1-INH). Recently, it was suggested that the ficolin-lectin pathway (ficolin-LP) might play a more dominant role than the mannose-binding l......BACKGROUND: The activation of plasma enzyme systems is insufficiently controlled in hereditary angioedema due to the deficiency of C1-inhibitor (C1-INH) (HAE-C1-INH). Recently, it was suggested that the ficolin-lectin pathway (ficolin-LP) might play a more dominant role than the mannose......: There is a marked heterogeneity of the pathomechanism and development of hereditary angioedema attacks in different patients. Our results suggest that the activation of the ficolin-LP may deplete the innately low level of C1-INH and thus, it may contribute to the uncontrolled activation of plasma cascade systems...

  16. Helicobacter pylori infection as a triggering factor of attacks in patients with hereditary angioedema

    DEFF Research Database (Denmark)

    Visy, Beáta; Füst, George; Bygum, Anette

    2007-01-01

    BACKGROUND: Helicobacter pylori infection is considered among the causative factors of urticaria and angioedema. Having conducted a study on 65 patients, Hungarian authors reported in 2001 that successful eradication of H. pylori is followed by a significant reduction in the number of attacks...... in patients with hereditary angioedema (HAE). The present study aimed to reinvestigate the relationship between H. pylori infection and the attack rate in the framework of an international collaborative study. MATERIALS AND METHODS: Within the framework of the PREHAEAT project launched by the European Union......, further 152 patients were studied in seven collaborating centers, and participants of the earlier study were followed up in order to detect any relationship between H. pylori infection and the occurrence of attacks in patients suffered from HAE. RESULTS: The proportion of patients experiencing frequent...

  17. Hereditary angioedema

    DEFF Research Database (Denmark)

    Peterson, M P; Bygum, A

    2016-01-01

    We report a 64-year-old man who suffered from recurrent visible swelling attacks since the age of 20 as well as episodes with severe upper airway edema, resulting in 4 emergency tracheotomies. Eventually after 44 years he was diagnosed with hereditary angioedema (HAE) type II. The aims of this re......We report a 64-year-old man who suffered from recurrent visible swelling attacks since the age of 20 as well as episodes with severe upper airway edema, resulting in 4 emergency tracheotomies. Eventually after 44 years he was diagnosed with hereditary angioedema (HAE) type II. The aims...

  18. Disease Severity, Activity, Impact, and Control and How to Assess Them in Patients with Hereditary Angioedema

    DEFF Research Database (Denmark)

    Bygum, Anette; Busse, Paula; Caballero, Teresa

    2017-01-01

    Hereditary angioedema (HAE) is a group of rare, potentially life-threatening, and frequently debilitating diseases characterized by recurrent, and often with an unpredictable onset, of swelling attacks. HAE is heterogeneous, with considerable differences between its subtypes, patients, and even...

  19. Before and after, the impact of available on-demand treatment for HAE

    DEFF Research Database (Denmark)

    Christiansen, Sandra C; Bygum, Anette; Banerji, Aleena

    2015-01-01

    Availability of effective treatment for acute attacks is expected to transform the care of hereditary angioedema (HAE) patients. We felt that it would be of interest to test these assumptions by examining the perceptions of HAE patients regarding the impact that these therapies have had on their ......Availability of effective treatment for acute attacks is expected to transform the care of hereditary angioedema (HAE) patients. We felt that it would be of interest to test these assumptions by examining the perceptions of HAE patients regarding the impact that these therapies have had...

  20. Preventing Hereditary Angioedema Attacks in Children Using Cinryze®: Interim Efficacy and Safety Phase 3 Findings

    Science.gov (United States)

    Aygören-Pürsün, Emel; Soteres, Daniel; Moldovan, Dumitru; Christensen, Jim; Van Leerberghe, Arthur; Hao, James; Schranz, Jennifer; Jacobson, Kraig W.; Martinez-Saguer, Inmaculada

    2017-01-01

    Background Hereditary angioedema (HAE) is a rare genetic disease causing unpredictable and potentially life-threatening subcutaneous and submucosal edematous attacks. Cinryze® (Shire ViroPharma Inc., Lexington, MA, USA), a nanofiltered C1 inhibitor (C1-INH), is approved in Europe for the treatment, preprocedure prevention, and routine prophylaxis of HAE attacks, and for the routine prophylaxis of attacks in the USA. This phase 3 study assessed the safety and efficacy of 2 C1-INH doses in preventing attacks in children aged 6–11 years. Methods A randomized single-blind crossover study was initiated in March 2014. Results for the first 6 patients completing the study are reported here. After a 12-week qualifying observation period, patients were randomly assigned to 1 of 2 C1-INH doses, 500 or 1,000 U, every 3–4 days for 12 weeks and crossed over to the alternative dose for a second 12-week period. The primary efficacy endpoint was the number of angioedema attacks per month. Results Six females with HAE type I and a median age of 10.5 years received 2 doses of C1-INH (500 and 1,000 U). The mean (SD) difference in the number of monthly angioedema attacks between the baseline observation period and the treatment period was −1.89 (1.31) with 500 U and −1.89 (1.11) with 1,000 U. During the treatment periods, cumulative attack severity, cumulative daily severity, and the number of attacks needing acute treatment were lower. No serious adverse events or study drug discontinuations occurred. Conclusions Interim findings from this study indicate that routine prevention with intravenous administration of C1-INH is efficacious, safe, and well tolerated in children ≥6 years of age. PMID:28662509

  1. Ecallantide is a novel treatment for attacks of hereditary angioedema due to C1 inhibitor deficiency

    Directory of Open Access Journals (Sweden)

    Farkas H

    2011-05-01

    Full Text Available Henriette Farkas, Lilian Varga3rd Department of Internal Medicine, Semmelweis University, Budapest, HungaryAbstract: Hereditary angioedema (HAE resulting from the deficiency of the C1 inhibitor protein is a rare disease, characterized by paroxysms of edema formation in the subcutis and in the submucosa. Edema can cause obstruction of the upper airway, which may lead to suffocation. Prompt elimination of edema is necessary to save patients from this life-threatening condition. Essentially, these edematous attacks are related to the activation of the kinin-kallikrein system and the consequent release of bradykinin. Ecallantide (known as DX-88 previously, a potent and specific inhibitor of plasma kallikrein is an innovative medicinal product. This is the only agent approved recently by the FDA for all localizations of edematous HAE attacks. Its advantages include no risk of viral contamination, high selectivity, very rapid onset of action, good tolerability, and straightforward subcutaneous administration. Owing to the risk of anaphylaxis, ecallantide should be administered by a health care professional. A postmarketing survey to improve risk-assessment and risk-minimization has been launched. The results of these studies may lead to the approval of ecallantide for self-administration.Keywords: hereditary angioedema, C1-inhibitor deficiency, treatment, bradykinin, kallikrein inhibitor, subcutaneous administration

  2. In Vitro Fertilization Using Luteinizing Hormone-Releasing Hormone Injections Resulted in Healthy Triplets without Increased Attack Rates in a Hereditary Angioedema Case

    Directory of Open Access Journals (Sweden)

    Ceyda Tunakan Dalgıç

    2018-01-01

    Full Text Available Hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE is a rare, autosomal dominant disorder. The management of pregnant patients with C1-INH-HAE is a challenge for the physician. Intravenous plasma-derived nanofiltered C1-INH (pdC1INH is the only recommended option throughout pregnancy, postpartum, and breastfeeding period. In order to increase pregnancy rates, physicians use fertilization therapies increasing endogen levels of estrogens. Therefore, these techniques can provoke an increase in the number and severity of edema attacks in C1-INH-HAE. Our patient is a 32-year-old female, diagnosed with C1-INH-HAE type 1 since 2004. She had been taking danazol 50–200 mg/day for 9 years. Due to her pregnancy plans in 2013, danazol was discontinued. PdC1INH was prescribed regularly for prophylactic purpose. Triplet pregnancy occurred by in vitro fertilization using luteinizing hormone-releasing hormone (LHRH injections. In our patient, LHRH injections were done four times without causing any severe attack during in vitro fertilization. Angioedema did not worsen during pregnancy and delivery due to the prophylactic use of intravenous pdC1INH in our patient. According to the attack frequency and severity, there was no difference between the three pregnancy trimesters. To our knowledge, this is the first published case of C1-INH-HAE receiving in vitro fertilization therapies without any angioedema attacks during pregnancy and delivery and eventually having healthy triplets with the prophylactic use of intravenous pdC1INH.

  3. Icatibant, an inhibitor of bradykinin receptor 2, for hereditary angioedema attacks: prospective experimental single-cohort study

    Directory of Open Access Journals (Sweden)

    Regis Albuquerque Campos

    Full Text Available CONTEXT AND OBJECTIVE: Hereditary angioedema (HAE with C1 inhibitor deficiency manifests as recurrent episodes of edema involving the skin, upper respiratory tract and gastrointestinal tract. It can be lethal due to asphyxia. The aim here was to evaluate the response to therapy for these attacks using icatibant, an inhibitor of the bradykinin receptor, which was recently introduced into Brazil.DESIGN AND SETTING: Prospective experimental single-cohort study on the efficacy and safety of icatibant for HAE patients.METHODS: Patients with a confirmed HAE diagnosis were enrolled according to symptoms and regardless of the time since onset of the attack. Icatibant was administered in accordance with the protocol that has been approved in Brazil. Symptom severity was assessed continuously and adverse events were monitored.RESULTS: 24 attacks in 20 HAE patients were treated (female/male 19:1; 19-55 years; median 29 years of age. The symptoms were: subcutaneous edema (22/24; abdominal pain (15/24 and upper airway obstruction (10/24. The time taken until onset of relief was: 5-10 minutes (5/24; 20.8%; 10-20 (5/24; 20.8%; 20-30 (8/24; 33.4%; 30-60 (5/24; 20.8%; and 2 hours (1/24; 4.3%. The time taken for complete resolution of symptoms ranged from 4.3 to 33.4 hours. Adverse effects were only reported at injection sites. Mild to moderate erythema and/or feelings of burning were reported by 15/24 patients, itching by 3 and no adverse effects in 6.CONCLUSION: HAE type I patients who received icatibant responded promptly; most achieved improved symptom severity within 30 minutes. Local adverse events occurred in 75% of the patients.

  4. The humanistic burden of hereditary angioedema

    DEFF Research Database (Denmark)

    Caballero, Teresa; Aygören-Pürsün, Emel; Bygum, Anette

    2014-01-01

    Hereditary angioedema (HAE) is a rare but potentially life-threatening disease marked by spontaneous, recurrent attacks of swelling. The broad range of consequences of HAE on patients? lives is not well understood. The study objective was to comprehensively characterize the burden of illness......, traveling, and passing HAE to their children. Based on Hospital Anxiety and Depression Scale scores, 38 and 14% had clinically meaningful anxiety and depression, respectively. Despite standard of care, HAE patients still have frequent and painful attacks. Patients experience substantial impairment...

  5. Health-Related Quality of Life with Subcutaneous C1-Inhibitor for Prevention of Attacks of Hereditary Angioedema.

    Science.gov (United States)

    Lumry, William R; Craig, Timothy; Zuraw, Bruce; Longhurst, Hilary; Baker, James; Li, H Henry; Bernstein, Jonathan A; Anderson, John; Riedl, Marc A; Manning, Michael E; Keith, Paul K; Levy, Donald S; Caballero, Teresa; Banerji, Aleena; Gower, Richard G; Farkas, Henriette; Lawo, John-Philip; Pragst, Ingo; Machnig, Thomas; Watson, Douglas J

    2018-01-31

    Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) impairs health-related quality of life (HRQoL). The objective of this study was to assess HRQoL outcomes in patients self-administering subcutaneous C1-INH (C1-INH[SC]; HAEGARDA) for routine prevention of HAE attacks. Post hoc analysis of data from the placebo-controlled, crossover phase III COMPACT study (Clinical Studies for Optimal Management of Preventing Angioedema with Low-Volume Subcutaneous C1-Inhibitor Replacement Therapy). Ninety patients with C1-INH-HAE were randomized to 1 of 4 treatment sequences: C1-INH(SC) 40 or 60 IU/kg twice weekly for 16 weeks, preceded or followed by 16 weeks of twice weekly placebo injections. All HAE attacks were treated with open-label on-demand treatment as necessary. HRQoL assessments at week 14 (last visit) included the European Quality of Life-5 Dimensions Questionnaire (EQ-5D-3L), the Hospital Anxiety and Depression Scale (HADS), the Work Productivity and Activity Impairment Questionnaire (WPAI), and the Treatment Satisfaction Questionnaire for Medication (TSQM). Compared with placebo (on-demand treatment alone), treatment with twice weekly C1-INH(SC) (both doses combined) was associated with better EQ-5D visual analog scale general health, less HADS anxiety, less WPAI presenteeism, work productivity loss, and activity impairment, and greater TSQM effectiveness and overall treatment satisfaction. More patients self-reported a "good/excellent" response during routine prevention with C1-INH(SC) compared with on-demand only (placebo prophylaxis) management. For each HRQoL measure, a greater proportion of patients had a clinically meaningful improvement during C1-INH(SC) treatment compared with placebo. In patients with frequent HAE attacks, a treatment strategy of routine prevention with self-administered twice weekly C1-INH(SC) had a greater impact on improving multiple HAE-related HRQoL impairments, most notably anxiety and work productivity, compared with on

  6. Understanding Hereditary Angioedema

    Science.gov (United States)

    ... Allergy Library ▸ Understanding Hereditary Angioedema Share | Understanding Hereditary Angioedema This article has been reviewed by Thanai Pongdee, MD, FAAAAI Hereditary Angioedema (HAE) is a rare genetic condition. People with ...

  7. Current state of hereditary angioedema management: A patient survey

    Science.gov (United States)

    Busse, Paula; Christiansen, Sandra C.; Li, Henry; Lumry, William; Davis-Lorton, Mark; Bernstein, Jonathan A.; Frank, Michael; Castaldo, Anthony; Long, Janet F.; Zuraw, Bruce L.; Riedl, Marc

    2015-01-01

    Hereditary angioedema (HAE) is a chronic disease with a high burden of disease that is poorly understood and often misdiagnosed. Availability of treatments, including C1 esterase inhibitor (C1INH) replacement, ecallantide, and icatibant, marks a significant advance for HAE patients. We aimed to better understand the current state of HAE care, from a patient perspective, after the introduction of several novel therapies. One session of the United States Hereditary Angioedema Association 2013 patient summit was devoted to data collection for this study. Patients attending the summit were self-selected, and HAE diagnosis was self-reported. Survey questions assessed patient characteristics, burden of disease, and treatment. Participant responses were captured using an audience response system. We surveyed 149 (80%) type I and II HAE (HAE-C1INH) and 37 (20%) HAE with normal C1INH (HAE-nlC1INH) patients. HAE-C1INH (72%) and HAE-nlCINH patients (76%) equally reported that HAE had a significant impact on quality of life (QOL). A third of HAE-C1INH patients were diagnosed within one year of their first HAE attack, but another third reported a delay of more than 10 years. Most HAE-C1INH (88%) and HAE-nlC1INH (76%) patients had on-demand treatment available. HAE-C1INH patients frequently had an individual treatment plan (76%) compared with 50% of HAE-nlC1INH patients. Most HAE-C1INH patients went to the emergency department (ED) or were hospitalized less than once every six months (80%). Our findings show that HAE management is improving with good access to on-demand and prophylactic treatment options. However, HAE patients still have a significant burden of disease and continued research and educational efforts are needed. PMID:25976438

  8. Angioedema

    Science.gov (United States)

    Angioneurotic edema; Welts; Allergic reaction - angioedema; Hives - angioedema ... symptoms may include: Abdominal cramping Breathing difficulty Swollen eyes and mouth Swollen lining of the eyes ( chemosis )

  9. Hereditary Angioedema: a Challenging Diagnosis for the Gastroenterologist

    Directory of Open Access Journals (Sweden)

    Gábos Gabriella

    2016-12-01

    Full Text Available Hereditary angioedema (HAE caused by a deficiency of C1 esterase inhibitor enzyme (C1-INH is a very rare, autosomal dominantly inherited genetic disorder, characterized by recurrent peripheral angioedema, painful abdominal attacks and episodes of laryngeal edema. Abdominal attacks are frequent symptoms in adult HAE patients, occurring in more than 90% of the cases. Angioedema in the bowel or abdomen can occur in the absence of cutaneous manifestations and may be easily misdiagnosed unless the clinician has a high degree of awareness to include HAE in the differential diagnosis. Misdiagnosis is associated with inadequate treatments, including unnecessary surgical procedures. Any patient who presents recurrent episodes of swelling should be evaluated for HAE caused by C1-INH deficiency. New therapies could save lives and dramatically improve their quality of life.

  10. Recombinant C1-Inhibitor Effects on Coagulation and Fibrinolysis in Patients with Hereditary Angioedema

    NARCIS (Netherlands)

    Relan, Anurag; Bakhtiari, Kamran; van Amersfoort, Edwin S.; Meijers, Joost C. M.; Hack, C. Erik

    2012-01-01

    Background: Recombinant human C1-inhibitor (rhC1INH; Ruconest (R)) has been developed for treatment of acute angioedema attacks in patients with hereditary angioedema (HAE) due to heterozygous deficiency of C1INH. Previous reports suggest that administration of plasma-derived C1INH products may be

  11. Management of hereditary angioedema: 2010 Canadian approach

    Science.gov (United States)

    2010-01-01

    C1-inhibitor (C1-INH) deficiency is a rare blood disorder resulting in angioedema attacks that are debilitating and may be life-threatening. Prophylaxis and therapy of events has changed since our first Canadian Consensus Conference on the diagnosis, therapy and management of HAE. We have formed the Canadian Hereditary Angioedema Network (CHAEN)/Réseau Canadien d'Angioédème Héréditaire (RCAH) - http://www.haecanada.com to advance care of patients with this disorder in Canada. We here present a review of management of HAE in Canada. PMID:20667123

  12. Estimation of EuroQol 5-Dimensions health status utility values in hereditary angioedema

    DEFF Research Database (Denmark)

    Aygören-Pürsün, Emel; Bygum, Anette; Beusterien, Kathleen

    2016-01-01

    OBJECTIVE: To estimate health status utility (preference) weights for hereditary angioedema (HAE) during an attack and between attacks using data from the Hereditary Angioedema Burden of Illness Study in Europe (HAE-BOIS-Europe) survey. Utility measures quantitatively describe the net impact...... conceptually with the EuroQol 5-Dimensions (EQ-5D) domains (pain/discomfort, mobility, self-care, usual activities, and anxiety/depression) were manually crosswalked to the corresponding UK population-based EQ-5D utility weights. EQ-5D utilities were computed for each respondent in the HAE-BOIS-Europe survey...

  13. Self-administration of intravenous C1-inhibitor therapy for hereditary angioedema and associated quality of life benefits

    DEFF Research Database (Denmark)

    Bygum, Anette; Andersen, Klaus Ejner; Mikkelsen, Carsten Sauer

    2009-01-01

    Hereditary angioedema (HAE) is often debilitating with a serious effect on quality of life (QOL). Treatment of acute HAE attacks is usually with C1 esterase inhibitor (C1-INH) concentrates; however, treatment can be delayed by patients' travel time for attending emergency units. We assessed...

  14. [Angioedema].

    Science.gov (United States)

    2015-05-11

    Angioedema may be an overlooked common disease. Angioedema comprises the idiopathic, the allergic, pseudo allergic, the physical form as well as the hereditary form. Diagnosis is simple when symptoms are classical (lips, eyes and tongue) but might be missed if symptoms are nonspecific such as dizziness or dyspnoea. However, the most striking observation reviewing the literature is the absence of original research on angioedema considering the high prevalence of the disease. From a patient and physician perspective more information and research on angioedema is needed.

  15. Hereditary Angioedema in Childhood

    DEFF Research Database (Denmark)

    Kjaer, Line; Bygum, Anette

    2012-01-01

      Hereditary angioedema (HAE) is a rare inherited disease that is often difficult to diagnose. We report a case of a 9-year-old boy with a spontaneous mutation causing HAE, diagnosed after a life-threatening episode of angioedema of the head and upper respiratory tract after a 5-year history...

  16. Hereditary angioedema: what the gastroenterologist needs to know

    Directory of Open Access Journals (Sweden)

    Ali MA

    2014-11-01

    Full Text Available M Aamir Ali, Marie L Borum Division of Gastroenterology and Liver Diseases, George Washington University, Washington, DC, USA Abstract: Up to 93% of patients with hereditary angioedema (HAE experience recurrent abdominal pain. Many of these patients, who often present to emergency departments, primary care physicians, general surgeons, or gastroenterologists, are misdiagnosed for years and undergo unnecessary testing and surgical procedures. Making the diagnosis of HAE can be challenging because symptoms and attack locations are often inconsistent from one episode to the next. Abdominal attacks are common and can occur without other attack locations. An early, accurate diagnosis is central to managing HAE. Unexplained abdominal pain, particularly when accompanied by swelling of the face and extremities, suggests the diagnosis of HAE. A family history and radiologic imaging demonstrating edematous bowel also support an HAE diagnosis. Once HAE is suspected, C4 and C1 esterase inhibitor (C1-INH laboratory studies are usually diagnostic. Patients with HAE may benefit from recently approved specific treatments, including plasma-derived C1-INH or recombinant C1-INH, a bradykinin B2-receptor antagonist, or a kallikrein inhibitor as first-line therapy and solvent/detergent-treated or fresh frozen plasma as second-line therapy for acute episodes. Short-term or long-term prophylaxis with nanofiltered C1-INH or attenuated androgens will prevent or reduce the frequency and severity of episodes. Gastroenterologists can play a critical role in identifying and treating patients with HAE, and should have a high index of suspicion when encountering patients with recurrent, unexplained bouts of abdominal pain. Given the high rate of abdominal attacks in HAE, it is important for gastroenterologists to appropriately diagnose and promptly recognize and treat HAE, or refer patients with HAE to an allergist. Keywords: hereditary angioedema, abdominal pain, diagnosis

  17. Lesson of the month 2: The limitations of steroid therapy in bradykinin-mediated angioedema attacks.

    Science.gov (United States)

    Ismail, Sharif; Cheng, Leo; Grigoriadou, Sofia; Laffan, James; Menon, Manoj

    2015-02-01

    Acute angioedema attacks are conventionally treated with antihistamines and steroids, in line with a presumed mechanism of disease involving overwhelming mast-cell degranulation. This approach overlooks a small but important minority of cases in which attacks are bradykinin driven and exhibit poor responsiveness to steroid or anti-histamine therapy. These patients may have a family history of angioedema (hereditary angioedema), or a past medical history including B-cell lymphoproliferative disorders or autoimmune disease (acquired angioedema). Rather than steroid therapy, they respond to administration of a bradykinin inhibitor, or more commonly, a C1 esterase inhibitor substitute, to control acute symptoms and reduce the probability of invasive airway insertion. In the long-term, they require C1 esterase inhibitor sparing therapy and a treat-the-cause approach to reduce the risk of recurrent attacks. We present here a case of a middle-aged woman who presented with recurrent angioedema of initially uncertain aetiology. © 2015 Royal College of Physicians.

  18. Recombinant human C1-inhibitor in the treatment of acute angioedema attacks

    NARCIS (Netherlands)

    Choi, Goda; Soeters, Maarten R.; Farkas, Henriette; Varga, Lilian; Obtulowicz, Krystyna; Bilo, Barbara; Porebski, Greg; Hack, C. Erik; Verdonk, Rene; Nuijens, Jan; Levi, Marcel

    2007-01-01

    BACKGROUND: Patients with hereditary C1-inhibitor deficiency have recurrent attacks of angioedema, preferably treated with C1-inhibitor concentrate. A recombinant human C1-inhibitor (rHuC1INH) was developed, derived from milk from transgenic rabbits. This study was undertaken to investigate the

  19. Hereditary angioedema in women

    Directory of Open Access Journals (Sweden)

    Bouillet Laurence

    2010-07-01

    Full Text Available Abstract Women with hereditary angioedema (HAE are more likely to be symptomatic that men. Hormonal factors (puberty, contraception, pregnancy,.... play a significant role in the precipitation or worsening of the condition in women. So, combined contraceptive pills are not indicated and progestogen pill must be preferred. During pregnancy, attack rate can increase (38-48% of women. C1Inhibitor concentrate and tranexamic acid can be used during pregnancy. Attenuated androgens for long term prophylaxis are effective but side effects appear more often in female patients. These side effects are dose dependant and can be attenuated by titrating the dose down the lowest effective level.

  20. Benefits and risks of danazol in hereditary angioedema

    DEFF Research Database (Denmark)

    Bork, Konrad; Bygum, Anette; Hardt, Jochen

    2008-01-01

    BACKGROUND: Hereditary angioedema (HAE) due to C1 inhibitor deficiency is clinically characterized by relapsing skin swellings, abdominal pain attacks, and life-threatening upper airway obstruction. Treatment with androgens prevents attacks for those with this condition. OBJECTIVE: To examine....... In the other patients, hereditary angioedema ran a mild course. The frequency of acute attacks during danazol treatment was reduced to 16.2%, and the attacks were considerably milder than before treatment. Laryngeal edema was reduced to 4.8%. Adverse effects (weight gain, virilization, menstrual irregularities......, headache, depression, and/or liver adenomas) occurred in 93 of the 118 patients and led to discontinuation of danazol therapy in 30 patients. CONCLUSIONS: Danazol is highly beneficial in patients with frequent and severe attacks of HAE. Because the risk of adverse effects is high, close monitoring...

  1. Overview of hereditary angioedema caused by C1-inhibitor deficiency: assessment and clinical management.

    Science.gov (United States)

    Bork, K; Davis-Lorton, M

    2013-02-01

    Hereditary angioedema due to C1-inhibitor deficiency (HAE-C1-INH) is a rare, autosomal-dominant disease. HAE-C1-INH is characterized by recurrent attacks of marked, diffuse, nonpitting and nonpruritic skin swellings, painful abdominal attacks, and laryngeal edema. The extremities and the gastrointestinal tract are most commonly affected. Swelling of the upper respiratory mucosa poses the greatest risk because death from asphyxiation can result from laryngealedema. HAE-C1-INH attacks are variable, unpredictable, and may be induced by a variety of stimuli, including stress or physical trauma. Because the clinical presentation of HAE-C1-INH is similar to other types of angioedema, the condition may be a challenge to diagnose. Accurate identification of HAE-C1-INH is critical in order to avoid asphyxiation by laryngeal edema and to improve the burden of disease. Based on an understanding of the underlying pathophysiology of IHAE-C1-INH, drugs targeted specifically to the disease, such as C1-inhibitor therapy, bradykinin B2-receptor antagonists, and kallikrein-inhibitors, have become available for both treatment and prevention of angioedema attacks. This article reviews the clinical features, differential diagnosis, and current approaches to management of HAE-C1-INH.

  2. Presentation, diagnosis and treatment of angioedema without wheals: a retrospective analysis of a cohort of 1058 patients.

    Science.gov (United States)

    Mansi, M; Zanichelli, A; Coerezza, A; Suffritti, C; Wu, M A; Vacchini, R; Stieber, C; Cichon, S; Cicardi, M

    2015-05-01

    The first classification of angioedema without wheals was recently reported and comprises different forms of the disease distinguished by aetiology, mediator of oedema and inheritance. In total, 1725 consecutive patients with angioedema without wheals were examined at our centre between 1993 and 2012. We excluded from the analysis 667 patients because of incomplete data or because angioedema was related to a specific factor. According to the new classification of angioedema, the 1058 patients included in this analysis were diagnosed with hereditary (HAE; n = 377) or acquired angioedema (AAE; n = 681). The former group included HAE with C1-inhibitor (C1-INH) deficiency (C1-INH-HAE; n = 353) and HAE with normal C1-INH levels (n = 24), of which six had a factor XII mutation (FXII-HAE) and 18 had disease of unknown origin (U-HAE). The AAE group included disease with C1-INH deficiency (C1-INH-AAE; n = 49), AAE related to angiotensin-converting enzyme inhibitor treatment (n = 183), idiopathic histaminergic (IH-AAE; n = 379) and idiopathic nonhistaminergic angioedema (InH-AAE; n = 70). We compared hereditary and AAE with uncertain aetiopathogenesis: the FXII-HAE and U-HAE groups pooled (FXII/U-HAE) versus InH-AAE. The median age at onset of FXII/U-HAE and InH-AAE was 26 and 38 years, respectively. In addition, 56% of patients with FXII/U-HAE and 81% of those with InH-AAE reported more than five attacks per year (median duration of 48 h). The location of angioedema in patients with FXII/U-HAE versus those with InH-AAE was the following: face, 70% versus 86%; tongue, oral cavity or larynx, 55% versus 68%; limbs, 70% versus 56%; and gastrointestinal mucosa, 50% versus 20%. Prophylaxis with tranexamic acid was effective in all six patients with U-HAE and in 37 of 38 with InH-AAE who were started on this treatment. Our findings in this cohort of patients with angioedema provide new information on the clinical characteristics, diagnosis and treatment of this

  3. The burden of illness in patients with hereditary angioedema.

    Science.gov (United States)

    Banerji, Aleena

    2013-11-01

    Hereditary angioedema (HAE) is a rare genetic disease characterized by long-term recurrent attacks of subcutaneous or submucosal edema in different parts of the body. A comprehensive review of the literature on burden of illness for patients with HAE is presented. A Boolean search was performed using MEDLINE and EMBASE databases and the Internet. Articles discussing aspects of the burden of illness in HAE were selected. Topics focused on the course of the disease, nature of attacks, treatment, quality of life, and costs. Hereditary angioedema is associated with a significant and multifaceted disease burden. Diagnosis is often delayed for years, with patients receiving ineffective treatment and unnecessary medical procedures before diagnosis. HAE attacks are painful, unpredictable, and debilitating and often require emergency medical attention. Attacks can affect a patient's daily activities, including work or schooling. Depression and anxiety are prevalent in patients with HAE. Recent advances in treatment provide patients with effective and well-tolerated prophylactic and on-demand therapeutic options. However, end points specific to HAE that better measure the impact of treatment on disease burden are lacking. Furthermore, there is a notable paucity of literature directed toward physicians who are instrumental in diagnosing and treating patients with HAE (eg, emergency department). More publications are broadening the understanding of HAE. However, important gaps remain. Effective management of HAE requires a more comprehensive understanding of the disease burden so that disease management can be individualized to meet specific patient needs. Copyright © 2013 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  4. Safety and efficacy of icatibant self-administration for acute hereditary angioedema

    Science.gov (United States)

    Boccon-Gibod, I; Bouillet, L

    2012-01-01

    We evaluated the efficacy and safety of icatibant self-administration in 15 patients with hereditary angioedema (HAE) types I or III, for 55 acute attacks (mostly severe or very severe). Icatibant self-administration was generally effective: first symptom improvement occurred in 5 min–2 h (HAE type I; n = 17) and 8 min–1 h (HAE type III; n = 9) for abdominal attacks and 5–30 min (HAE type I; n = 4) and 10 min–12 h (HAE type III; n = 6) for laryngeal attacks. Complete symptom resolution occurred in 15 min–19 h (HAE type I; n = 8) and 15 min–48 h (HAE type III; n = 9) for abdominal attacks and 5–48 h (HAE type I; n = 3) and 8–48 h (HAE type III; n = 5) for laryngeal attacks. No patient required emergency hospitalization. The only adverse events were mild, spontaneously resolving injection site reactions. Patients reported that carrying icatibant with them gave them greater confidence in managing their condition. PMID:22519593

  5. The humanistic burden of hereditary angioedema: results from the Burden of Illness Study in Europe.

    Science.gov (United States)

    Caballero, Teresa; Aygören-Pürsün, Emel; Bygum, Anette; Beusterien, Kathleen; Hautamaki, Emily; Sisic, Zlatko; Wait, Suzanne; Boysen, Henrik B

    2014-01-01

    Hereditary angioedema (HAE) is a rare but potentially life-threatening disease marked by spontaneous, recurrent attacks of swelling. The broad range of consequences of HAE on patients' lives is not well understood. The study objective was to comprehensively characterize the burden of illness and impact of HAE types I and II from the patient perspective. The HAE Burden of Illness Study in Europe was conducted in Spain, Germany, and Denmark to assess the real-world experience of HAE from the patient perspective via a one-time survey, which included items on clinical characteristics and physical and emotional impacts. One hundred eighty-six patients participated; 59% reported having an attack at least once a month, 67% reported moderate-to-severe pain during their last attack, and 74% reported moderate-to-severe swelling. The most common sites of the last attack were the abdomen and extremities; 24% experienced an attack in more than one site. The impact of HAE on daily activities was high during attacks and did not vary significantly by body site affected; patients also reported that HAE impacted their daily activities between attacks. Patients reported substantial anxiety about future attacks, traveling, and passing HAE to their children. Based on Hospital Anxiety and Depression Scale scores, 38 and 14% had clinically meaningful anxiety and depression, respectively. Despite standard of care, HAE patients still have frequent and painful attacks. Patients experience substantial impairment physically and emotionally both during and between attacks. A better understanding of these effects may help in the clinical management of HAE patients.

  6. Comparing acquired angioedema with hereditary angioedema (types I/II): findings from the Icatibant Outcome Survey.

    Science.gov (United States)

    Longhurst, H J; Zanichelli, A; Caballero, T; Bouillet, L; Aberer, W; Maurer, M; Fain, O; Fabien, V; Andresen, I

    2017-04-01

    Icatibant is used to treat acute hereditary angioedema with C1 inhibitor deficiency types I/II (C1-INH-HAE types I/II) and has shown promise in angioedema due to acquired C1 inhibitor deficiency (C1-INH-AAE). Data from the Icatibant Outcome Survey (IOS) were analysed to evaluate the effectiveness of icatibant in the treatment of patients with C1-INH-AAE and compare disease characteristics with those with C1-INH-HAE types I/II. Key medical history (including prior occurrence of attacks) was recorded upon IOS enrolment. Thereafter, data were recorded retrospectively at approximately 6-month intervals during patient follow-up visits. In the icatibant-treated population, 16 patients with C1-INH-AAE had 287 attacks and 415 patients with C1-INH-HAE types I/II had 2245 attacks. Patients with C1-INH-AAE versus C1-INH-HAE types I/II were more often male (69 versus 42%; P = 0·035) and had a significantly later mean (95% confidence interval) age of symptom onset [57·9 (51·33-64·53) versus 14·0 (12·70-15·26) years]. Time from symptom onset to diagnosis was significantly shorter in patients with C1-INH-AAE versus C1-INH-HAE types I/II (mean 12·3 months versus 118·1 months; P = 0·006). Patients with C1-INH-AAE showed a trend for higher occurrence of attacks involving the face (35 versus 21% of attacks; P = 0·064). Overall, angioedema attacks were more severe in patients with C1-INH-HAE types I/II versus C1-INH-AAE (61 versus 40% of attacks were classified as severe to very severe; P < 0·001). Median total attack duration was 5·0 h and 9·0 h for patients with C1-INH-AAE versus C1-INH-HAE types I/II, respectively. © 2016 British Society for Immunology.

  7. Bradykinin-mediated angioedema.

    Science.gov (United States)

    Obtułowicz, Krystyna

    2016-01-01

    Angioedema and urticaria often constitute a challenge in daily clinical practice. They may either co- -occur or present as independent conditions. They are characterized by a complex pathomechanism, and their symptoms may be triggered by diverse factors. These differences are crucial for developing a successful treatment regimen. Both conditions may have an allergic origin (immunoglobulin [Ig] E and non-IgE-related), usually induced by histamine, or a nonallergic one, such as bradykinin-mediated angioedema in patients with C1 inhibitor (C1-INH) deficiency or angioedema induced by certain drugs (eg, angiotensin-converting enzyme inhibitors). Currently, we distinguish 5 types of nonallergic angioedema: hereditary angioedema (HAE) due to C1-INH deficiency, acquired angioedema (AAE), and angioedema induced by the renin-angiotensin-aldosterone system, all of which are mediated by bradykinin, as well as pseudoallergic angioedema and idiopathic angioedema. Bradykinin-mediated angioedema (eg, laryngeal angioedema) may be life-threatening because of resistance to corticosteroids and antihistamine drugs. C1-INH concentrates are the drugs of choice in the treatment of HAE and AAE. In recent years, some new drugs have been introduced in the treatment of bradykinin-mediated angioedema, such as bradykinin B2-receptor antagonist, icatibant, and kallikrein inhibitor, ecallantide, which allow to improve treatment outcomes.

  8. The relationship between anxiety and quality of life in children with hereditary angioedema.

    Science.gov (United States)

    Kessel, Aharon; Farkas, Henriette; Kivity, Shmuel; Veszeli, Nóra; Kőhalmi, Kinga V; Engel-Yeger, Batya

    2017-11-01

    The severe life-threatening characteristics of hereditary angioedema (HAE) with C1-inhibitor deficiency (C1-INH-HAE) can affect anxiety levels among pediatric patients. This emotional burden together with the physical restrictions of C1-INH-HAE may decrease children's health-related quality of life (HRQoL). (i) To compare anxiety state and trait between children with C1-INH-HAE and healthy controls; (ii) to examine the relationship between the level of anxiety of children with C1-INH-HAE, their disease activity/affected sites and their HRQoL; and (iii) to predict the HRQoL of children with C1-INH-HAE based on their anxiety level and disease activity/affected sites METHODS: Thirty-three children with C1-INH-HAE (aged 5-18 years) and 52 healthy controls were recruited from Israel and Hungary. All children completed the State-Trait Anxiety Inventory for Children (STAIC), the Pediatric Quality of Life Inventory (Peds-QL) demographic questionnaire and a disease activity and site questionnaire . Disease activity was defined as the number of attacks in last year. Both anxiety state and trait were significantly higher among children with C1-INH-HAE as compared to the controls (44.74±10.56 vs 38.76±10.67, Panxiety state (F 56,2 =4.69, P=.001) and trait (F 56,2 =9.06, Panxiety trait was correlated with the number of angioedema-affected sites (r=.52, P=.003). The presence of HAE attacks and higher anxiety trait predicted a lower HRQoL in children with C1-INH-HAE. C1-INH-HAE children have higher anxiety trait and state, which correlate with reduced HRQoL domains. © 2017 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.

  9. Hereditary angioedema with normal C1 inhibitor in a French cohort: Clinical characteristics and response to treatment with icatibant

    Science.gov (United States)

    Boccon‐Gibod, Isabelle; Launay, David; Gompel, Anne; Kanny, Gisele; Fabien, Vincent; Fain, Oliver

    2017-01-01

    Abstract Introduction The clinical characteristics and icatibant‐treatment outcomes of patients with hereditary angioedema with normal C1 inhibitor (HAE‐nC1 INH) are limited. Methods We retrospectively analyzed data from French HAE patients enrolled in the Icatibant Outcome Survey registry (from July 2009 to September 2013) to compare disease characteristics and the effectiveness and safety of acute icatibant‐treated angioedema attacks in patients with HAE‐nC1 INH, HAE with C1 INH deficiency (type I), or dysfunction (type II). Results One center in Grenoble contributed 22 patients with HAE‐nC1 INH and a family history of HAE while 15 centers across France contributed 153 patients with HAE type I and seven patients with HAE type II. Patients with HAE‐nC1 INH compared to HAE type I, respectively, were more likely to be female (88.1% vs. 63.4%), older at median age of disease onset (21 years vs. 15 years), and have a greater rate of abdominal (80% vs. 61%) and laryngeal (23% vs. 14%) attacks. Icatibant was effective in both groups though the median time to resolution of attack was significantly longer in the HAE‐nC1 INH group (20.0 h, 37 attacks) versus the HAE type I group (14.0 h, 67 attacks). Icatibant was self‐administered for 96.1% of attacks in patients with HAE‐nC1 INH and 75.8% in patients with HAE type I. No serious adverse side effects related to icatibant were reported. Conclusions These data help further define the disease characteristics of HAE‐nC1 INH in the French population and extend the limited data reporting the safe and effective use of icatibant in acute treatment of angioedema in French patients diagnosed with HAE‐nC1 INH. PMID:28250922

  10. Socioeconomic burden of hereditary angioedema: results from the hereditary angioedema burden of illness study in Europe

    Science.gov (United States)

    2014-01-01

    Background Hereditary angioedema (HAE) due to C1 inhibitor deficiency is a rare but serious and potentially life-threatening disease marked by spontaneous, recurrent attacks of swelling. The study objective was to characterize direct and indirect resource utilization associated with HAE from the patient perspective in Europe. Methods The study was conducted in Spain, Germany, and Denmark to assess the real-world experience of HAE via a cross-sectional survey of HAE patients, including direct and indirect resource utilization during and between attacks for patients and their caregivers over the past 6 months. A regression model examined predictors of medical resource utilization. Results Overall, 164 patients had an attack in the past 6 months and were included in the analysis. The most significant predictor of medical resource utilization was the severity of the last attack (OR 2.6; p < 0.001). Among patients who sought medical care during the last attack (23%), more than half utilized the emergency department. The last attack prevented patients from their normal activities an average of 4–12 hours. Patient and caregiver absenteeism increased with attack severity and frequency. Among patients who were working or in school (n = 120), 72 provided work/school absenteeism data, resulting in an estimated 20 days missing from work/school on average per year; 51% (n = 84) indicated that HAE has hindered their career/educational advancement. Conclusion HAE poses a considerable burden on patients and their families in terms of direct medical costs and indirect costs related to lost productivity. This burden is substantial at the time of attacks and in between attacks. PMID:24996814

  11. Towards a specific marker for acute bradykinin-mediated angioedema attacks: a literature review.

    Science.gov (United States)

    Deroux, Alban; Vilgrain, Isabelle; Dumestre-Pérard, Chantal; Boccon-Gibod, Isabelle; Bouillet, Laurence

    2015-01-01

    Bradykinin-mediated angioedema (AE) is a rare disease characterised by recurrent angioedema linked to acquired (e.g. angiotensin converting enzyme inhibitor induced AE) or hereditary disorders (e.g. AE type I or II). As the clinical picture can be misleading, diagnosis of this disease is sometimes difficult. A bradykinin-mediated AE attack may be a therapeutic emergency which requires access to effective, but expensive, treatments. Their prescription must therefore be justified. No specific marker of acute bradykinin-mediated AE attacks has yet been identified to facilitate the therapeutic decision but it has been sought in many studies. This article reviews the literature on this type of biomarker, comparing candidate bradykinin-mediated AE markers to candidate markers of mast cell activation. The most interesting biomarkers are those linked to endothelial stress (VE cadherin, E-selectin, endothelin-1, von Willebrand factor and its activity) which is significantly increased during an AE attack. All these markers must now be validated by prospective studies to determine their specificity and utility in diagnosis.

  12. Smjernice za dijagnostiku i liječenje hereditarnog angioedema [Guidelines for the diagnosis and treatment of hereditary angioedema

    OpenAIRE

    Stipić Marković, Asja; Rožmanić, Vojko; Anić, Branimir; Aberle, Neda; Račić, Goran; Novak, Srđan; Sunara, Davor; Grdinić, Boris; Karadža-Lapić, Ljerka; Ražov Radas, Melanija; Karanović, Boris; Kvenić, Barbara

    2014-01-01

    Hereditary angioedema (HAE) is a rare but potentially fatal genetic disorder with nonpitting, nonerythematous, and not pruritic swelling which can affect the hands, feet, face, genitals and visceral mucosa. The type, frequency, and severity of the attacks vary between patients, and over the lifetime of an individual patient. Efforts in Croatian counties have identified approximately 100 patients (but there must be more undiagnosed patients). The first global guideline for the management of HA...

  13. Diagnosis and screening of patients with hereditary angioedema in primary care

    Science.gov (United States)

    Henao, Maria Paula; Kraschnewski, Jennifer L; Kelbel, Theodore; Craig, Timothy J

    2016-01-01

    Hereditary angioedema (HAE) is a rare autosomal dominant disease that commonly manifests with episodes of cutaneous or submucosal angioedema and intense abdominal pain. The condition usually presents due to a deficiency of C1 esterase inhibitor (C1-INH) that leads to the overproduction of bradykinin, causing an abrupt increase in vascular permeability. A less-understood and less-common form of the disease presents with normal C1-INH levels. Symptoms of angioedema may be confused initially with mast cell-mediated angioedema, such as allergic reactions, and may perplex physicians when epinephrine, antihistamine, or glucocorticoid therapies do not provide relief. Similarly, abdominal attacks may lead to unnecessary surgeries or opiate dependence. All affected individuals are at risk for a life-threatening episode of laryngeal angioedema, which continues to be a source of fatalities due to asphyxiation. Unfortunately, the diagnosis is delayed on average by almost a decade due to a misunderstanding of symptoms and general lack of awareness of the disease. Once physicians suspect HAE, however, diagnostic methods are reliable and available at most laboratories, and include testing for C4, C1-INH protein, and C1-INH functional levels. In patients with HAE, management consists of acute treatment of an attack as well as possible short- or long-term prophylaxis. Plasma-derived C1-INH, ecallantide, icatibant, and recombinant human C1-INH are new treatments that have been shown to be safe and effective in the treatment of HAE attacks. The current understanding of HAE has greatly improved in recent decades, leading to growing awareness, new treatments, improved management strategies, and better outcomes for patients. PMID:27194914

  14. Hereditary angioedema: epidemiology, management, and role of icatibant

    Directory of Open Access Journals (Sweden)

    Ghazi A

    2013-05-01

    Full Text Available Aasia Ghazi, J Andrew GrantUniversity of Texas Medical Branch, Division of Allergy and Clinical Immunology, Galveston, TX, USAAbstract: Hereditary angioedema (HAE is an autosomal dominant, potentially life-threatening condition, manifesting as recurrent and self-limiting episodes of facial, laryngeal, genital, or peripheral swelling with abdominal pain secondary to intra-abdominal edema. The estimated prevalence of HAE in the general population is one individual per 50,000, with reported ranges from 1:10,000 to 1:150,000, without major sex or ethnic differences. Various treatment options for acute attacks and prophylaxis of HAE are authorized and available in the market, including plasma-derived (Berinert®, Cinryze®, and Cetor® and recombinant (Rhucin® and Ruconest™ C1 inhibitors, kallikrein inhibitor-ecallantide (Kalbitor®, and bradykinin B2 receptor antagonist-icatibant (Firazyr®. Some of these drugs are used only to treat HAE attacks, whereas others are only approved for prophylactic therapies and all of them have improved disease outcomes due to their different mechanisms of action. Bradykinin and its binding to B2 receptor have been demonstrated to be responsible for most of the symptoms of HAE. Thus icatibant (Firazyr®, a bradykinin B2 receptor antagonist, has proven to be an effective and more targeted treatment option and has been approved for the treatment of acute attacks of HAE. Rapid and stable relief from symptoms of cutaneous, abdominal, or laryngeal HAE attacks has been demonstrated by 30 mg of icatibant in Phase III clinical trials. Self-resolving mild to moderate local site reactions after subcutaneous injection of icatibant were observed. Icatibant is a new, safe, and effective treatment for acute attacks of HAE. HAE has been reported to result in enormous humanistic burden to patients, affecting both physical and mental health, with a negative impact on education, career, and work productivity, and with substantial

  15. Estimation of EuroQol 5-Dimensions health status utility values in hereditary angioedema.

    Science.gov (United States)

    Aygören-Pürsün, Emel; Bygum, Anette; Beusterien, Kathleen; Hautamaki, Emily; Sisic, Zlatko; Boysen, Henrik B; Caballero, Teresa

    2016-01-01

    To estimate health status utility (preference) weights for hereditary angioedema (HAE) during an attack and between attacks using data from the Hereditary Angioedema Burden of Illness Study in Europe (HAE-BOIS-Europe) survey. Utility measures quantitatively describe the net impact of a condition on a patient's life; a score of 0.0 reflects death and 1.0 reflects full health. The HAE-BOIS-Europe was a cross-sectional survey conducted in Spain, Germany, and Denmark to assess the real-world experience of HAE from the patient perspective. Survey items that overlapped conceptually with the EuroQol 5-Dimensions (EQ-5D) domains (pain/discomfort, mobility, self-care, usual activities, and anxiety/depression) were manually crosswalked to the corresponding UK population-based EQ-5D utility weights. EQ-5D utilities were computed for each respondent in the HAE-BOIS-Europe survey for acute attacks and between attacks. Overall, a total of 111 HAE-BOIS-Europe participants completed all selected survey items and thus allowed for computation of EQ-5D-based utilities. The mean utilities for an HAE attack and between attacks were 0.44 and 0.72, respectively. Utilities for an acute attack were dependent on the severity of pain of the last attack (0.61 for no pain or mild pain, 0.47 for moderate pain, and 0.08 for severe pain). There were no significant differences across countries. Mean utilities derived from the study approach compare sensibly with other disease states for both acute attacks and between attacks. The impacts of HAE translate into substantial health status disutilities associated with acute attacks as well as between attacks, documenting that the detrimental effects of HAE are meaningful from the patient perspective. Results were consistent across countries with regard to pain severity and in comparison to similar disease states. The results can be used to raise awareness of HAE as a serious disease with wide-ranging personal and social impacts.

  16. Estimation of EuroQol 5-Dimensions health status utility values in hereditary angioedema

    Science.gov (United States)

    Aygören-Pürsün, Emel; Bygum, Anette; Beusterien, Kathleen; Hautamaki, Emily; Sisic, Zlatko; Boysen, Henrik B; Caballero, Teresa

    2016-01-01

    Objective To estimate health status utility (preference) weights for hereditary angioedema (HAE) during an attack and between attacks using data from the Hereditary Angioedema Burden of Illness Study in Europe (HAE-BOIS-Europe) survey. Utility measures quantitatively describe the net impact of a condition on a patient’s life; a score of 0.0 reflects death and 1.0 reflects full health. Study design and methods The HAE-BOIS-Europe was a cross-sectional survey conducted in Spain, Germany, and Denmark to assess the real-world experience of HAE from the patient perspective. Survey items that overlapped conceptually with the EuroQol 5-Dimensions (EQ-5D) domains (pain/discomfort, mobility, self-care, usual activities, and anxiety/depression) were manually crosswalked to the corresponding UK population-based EQ-5D utility weights. EQ-5D utilities were computed for each respondent in the HAE-BOIS-Europe survey for acute attacks and between attacks. Results Overall, a total of 111 HAE-BOIS-Europe participants completed all selected survey items and thus allowed for computation of EQ-5D-based utilities. The mean utilities for an HAE attack and between attacks were 0.44 and 0.72, respectively. Utilities for an acute attack were dependent on the severity of pain of the last attack (0.61 for no pain or mild pain, 0.47 for moderate pain, and 0.08 for severe pain). There were no significant differences across countries. Mean utilities derived from the study approach compare sensibly with other disease states for both acute attacks and between attacks. Conclusion The impacts of HAE translate into substantial health status disutilities associated with acute attacks as well as between attacks, documenting that the detrimental effects of HAE are meaningful from the patient perspective. Results were consistent across countries with regard to pain severity and in comparison to similar disease states. The results can be used to raise awareness of HAE as a serious disease with wide

  17. The Levels of the Lectin Pathway Serine Protease MASP-1 and Its Complex Formation with C1 Inhibitor Are Linked to the Severity of Hereditary Angioedema

    DEFF Research Database (Denmark)

    Hansen, Cecilie Bo; Csuka, Dorottya; Munthe-Fog, Lea

    2015-01-01

    C1 inhibitor (C1-INH) is known to form complexes with the lectin complement pathway serine proteases MASP-1 and MASP-2. Deficiency of C1-INH is associated with hereditary angioedema (HAE), an autosomal inherited disease characterized by swelling attacks caused by elevated levels of bradykinin. MASP...

  18. The humanistic burden of hereditary angioedema: Impact on health-related quality of life, productivity, and depression.

    Science.gov (United States)

    Lumry, William R; Castaldo, Anthony J; Vernon, Margaret K; Blaustein, Marc B; Wilson, David A; Horn, Patrick T

    2010-01-01

    Hereditary angioedema (HAE) is a rare, autosomal dominant disorder characterized by recurrent acute attacks of swelling of the larynx, abdomen, and periphery. This study was designed to assess the humanistic burden of illness associated with HAE. HAE burden was assessed via a web-based survey of patients that solicited information on attack characterization, treatment, side effects, pain, and functional and emotional burden of disease management. In addition to HAE-specific sections, the survey used three standardized instruments to compare HAE patient data to normative (healthy) and chronic disease populations: the 12-Item Short Form (SF-12) Health Survey, the Work Productivity and Activity Impairment-General Health (WPAI-GH) questionnaire, and the Hamilton Depression Inventory-Short Form (HDI-SF). A total of 457 HAE patients responded to the survey (response rate, ∼19%). Patients reported significantly poorer health-related quality of life versus population norms, based on the SF-12 Physical Component Summary (mean, 43.7 versus 49.6; p 8.5, indicative of depressive symptomatology. Productivity was also markedly impaired in all WPAI-GH categories, including 34% overall work impairment. Because of their most recent HAE attack, workers lost a mean of 3.3 days; students lost a mean of 1.9 days. HAE results in considerable humanistic burden to patients across physical and mental health domains; negatively impacts education, career, and work productivity; and compounds the substantial economic burdens that are reported separately.

  19. Estimation of EuroQol 5-Dimensions health status utility values in hereditary angioedema

    Directory of Open Access Journals (Sweden)

    Aygören-Pürsün E

    2016-09-01

    Full Text Available Emel Aygören-Pürsün,1 Anette Bygum,2 Kathleen Beusterien,3 Emily Hautamaki,4 Zlatko Sisic,5 Henrik B Boysen,6 Teresa Caballero7 1Angioedema Centre, Department for Children and Adolescents, University Hospital Frankfurt, Goethe University, Frankfurt, Germany; 2Hereditary Angioedema Centre Denmark, Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark; 3Outcomes Research Strategies in Health, Washington, DC, 4Patient Reported Outcomes, Oxford Outcomes Inc., an ICON plc company, Bethesda, MD, USA; 5ViroPharma Incorporated, Chatsworth House, Maidenhead, UK; 6HAEi – Hereditary Angioedema International Patient Organization for C1 Inhibitor Deficiencies, Skanderborg, Denmark; 7Allergy Department, Hospital La Paz Institute for Health Research (IdiPaz, Biomedical Research Network on Rare Diseases U754 (CIBERER, University Hospital La Paz, Madrid, Spain Objective: To estimate health status utility (preference weights for hereditary angioedema (HAE during an attack and between attacks using data from the Hereditary Angioedema Burden of Illness Study in Europe (HAE-BOIS-Europe survey. Utility measures quantitatively describe the net impact of a condition on a patient’s life; a score of 0.0 reflects death and 1.0 reflects full health.Study design and methods: The HAE-BOIS-Europe was a cross-sectional survey conducted in Spain, Germany, and Denmark to assess the real-world experience of HAE from the patient perspective. Survey items that overlapped conceptually with the EuroQol 5-Dimensions (EQ-5D domains (pain/discomfort, mobility, self-care, usual activities, and anxiety/depression were manually crosswalked to the corresponding UK population-based EQ-5D utility weights. EQ-5D utilities were computed for each respondent in the HAE-BOIS-Europe survey for acute attacks and between attacks.Results: Overall, a total of 111 HAE-BOIS-Europe participants completed all selected survey items and thus allowed for computation

  20. [Perioperative Management of a Patient with Hereditary Angioedema: A Case Report].

    Science.gov (United States)

    Teranishi, Rie; Makino, Yumi; Amano, Eizou; Shibuya, Hiromi; Okada, Toshiki

    2015-04-01

    Hereditary angioedema (HAE) is a very rare disease that occurs in about 1 in 50,000 to 150,000 people. HAE is caused by low levels or inproper function of the plasma protein C1 inhibitor (C1-INH) which regulates activation of the complement system and the coagulation system. The typical symptom of HAE is regional swellings without pain nor itching, usually triggered by physical trauma or emotional stress. Unlike allergic edema, HAE attacks do not respond to antihistamines, corticosteroids noradrenaline. The swelling attacks against face and throat are potentially life-threatening, and should be treated as a medical emergency. We report a patient with HAE who underwent radical cystectomy of the upper gum under general anesthesia. Because the oral surgery with tracheal intubation is known to be a risk factor of laryngeal edema in a patient with HAE, she was given C1-INH before operation to prevent laryngeal edema according to HAE Guideline 2010 by the Japanese Association for Complement Research. Her pharynx and larynx were checked with Airwayscope before intubation and with bronchofiberscope before extubation, but no edema was recognized. Postoperatively, she was given C1-INH on the next morning again. She was discharged seven days after operation without any complications.

  1. Urticaria and angioedema.

    Science.gov (United States)

    Spickett, G

    2014-01-01

    Urticaria, also known as hives, and angioedema, where the swelling occurs below the skin instead of on the skin, are extremely common but there is a misconception that the most likely cause is an allergic reaction. Chronic urticaria in particular is rarely due to allergy. Equally for angioedema, many will consider the exceptionally rare hereditary angioedema (HAE), but in fact other medical causes are the most likely, in particular the use of angiotensin-converting enzyme inhibitor (ACE-I) drugs. Approximately 3-5% of patients receiving ACE-I will develop angioedema at some time in the course of their treatment.1 Stress is a major contributor to both chronic urticaria and recurrent angioedema. Treatment needs to focus on the use of long-acting, non-sedating, antihistamines. Corticosteroids may be used acutely but not long term.

  2. Hereditary Angioedema

    DEFF Research Database (Denmark)

    Abdel-Karim, Omar; Dizdarevic, Adis; Bygum, Anette

    2014-01-01

    of life. Most studies have been conducted in adults. We report a 13-year-old boy who quickly learned self-administration, which resulted in reduced frequency and severity of attacks. The aim of this report is to emphasize that children should be considered for self-administration training......Hereditary angioedema is an inherited disease that causes periodic swelling attacks, which can be life threatening and have a major effect on a patient's life. Studies have shown that home therapy for angioedema reduces disease severity, leads to faster relief of symptoms, and improves quality...

  3. Self-administered C1 esterase inhibitor concentrates for the management of hereditary angioedema: usability and patient acceptance

    Directory of Open Access Journals (Sweden)

    Li HH

    2016-09-01

    Full Text Available Huamin Henry Li Institute for Asthma and Allergy, Chevy Chase, MD, USA Abstract: Hereditary angioedema (HAE is a rare genetic disease characterized by episodic subcutaneous or submucosal swelling. The primary cause for the most common form of HAE is a deficiency in functional C1 esterase inhibitor (C1-INH. The swelling caused by HAE can be painful, disfiguring, and life-threatening. It reduces daily function and compromises the quality of life of affected individuals and their caregivers. Among different treatment strategies, replacement with C1-INH concentrates is employed for on-demand treatment of acute attacks and long-term prophylaxis. Three human plasma-derived C1-INH preparations are approved for HAE treatment in the US, the European Union, or both regions: Cinryze®, Berinert®, and Cetor®; however, only Cinryze is approved for long-term prophylaxis. Postmarketing studies have shown that home therapy (self-administered or administered by a caregiver is a convenient and safe option preferred by many HAE patients. In this review, we summarize the role of self-administered plasma-derived C1-INH concentrate therapy with Cinryze at home in the prophylaxis of HAE. Keywords: C1-INH concentrate, hereditary angioedema, disease management, first line, prophylaxis, self-administration 

  4. Pediatric hereditary angioedema due to C1-inhibitor deficiency

    Directory of Open Access Journals (Sweden)

    Farkas Henriette

    2010-07-01

    Full Text Available Abstract Hereditary angioedema (HAE resulting from the deficiency of the C1 inhibitor (C1-INH is a rare, life-threatening disorder. It is characterized by attacks of angioedema involving the skin and/or the mucosa of the upper airways, as well as the intestinal mucosa. In approximately 50 per cent of cases, clinical manifestations may appear during childhood. The complex management of HAE in pediatric patients is in many respects different from the management of adults. Establishing the diagnosis early, preferably before the onset of clinical symptoms, is essential in cases with a positive family history. Complement studies usually afford accurate diagnosis, whereas molecular genetics tests may prove helpful in uncertain cases. Appropriate therapy, supported by counselling, suitable modification of lifestyle, and avoidance of triggering factors (which primarily include mechanical trauma, mental stress and airway infections in children may spare the patient unnecessary surgery and may prevent mortality. Prompt control of edematous attacks, short-term prophylaxis and intermittent therapy are recommended as the primary means for the management of pediatric cases. Medicinal products currently used for the treatment of children with hereditary angioedema include antifibrinolytics, attenuated androgens, and C1-INH replacement therapy. Current guidelines favour antifibrinolytics for long-term prophylaxis because of their favorable safety profile but efficacy may be lacking. Attenuated androgens administered in the lowest effective dose are another option. C1-INH replacement therapy is also an effective and safe agent for children. Regular monitoring and follow-up of patients are necessary.

  5. Treatment of Hereditary Angioedema: items that need to be addressed in practice parameter

    Directory of Open Access Journals (Sweden)

    Dagen Callie

    2010-05-01

    Full Text Available Abstract Background Hereditary Angioedema (HAE is a rare, autosomal dominant (AD disorder caused by a C1 esterase inhibitor (C1-inh deficiency or qualitative defect. Treatment of HAE in many parts of the world fall short and certain items need to be addressed in future guidelines. Objective To identify those individuals who should be on long-term prophylaxis for HAE. Additionally, to determine if prodromal symptoms are sensitive and specific enough to start treatment with C-1 INH and possibly other newly approved therapies. Also, to discuss who is appropriate to self-administer medications at home and to discuss training of such patients. Methods A literature review (PubMed and Google was performed and articles published in peer-reviewed journals, which addressed HAE prophylaxis, current HAE treatments, prodromal symptoms of HAE and self-administration of injected home medications were selected, reviewed and summarized. Results Individuals whom have a significant decrease in QOL or have frequent or severe attacks and who fail or are intolerant to androgens should be considered for long-term prophylaxis with C1INH. Prodromal symptoms are sensitive, but non-specific, and precede acute HAE attacks in the majority of patients. Although the treatment of prodromal symptoms could lead to occasional overtreatment, it could be a viable option for those patients able to adequately predict their attacks. Finally, self-administration, has been shown to be feasible, safe and effective for patients who require IV therapy for multiple other diseases to include, but not limited to, hemophilia. Conclusions Prophylactic therapy, treatment at the time of prodromal symptoms and self-administration at home all should allow a reduction in morbidity and mortality associated with HAE.

  6. Depression and anxiety in patients with hereditary angioedema.

    Science.gov (United States)

    Fouche, Andrew S; Saunders, Erika F H; Craig, Timothy

    2014-04-01

    Hereditary angioedema (HAE) is characterized by edematous swelling attacks of the face, extremities, abdomen, genitalia, and upper airway. The potential for laryngeal swelling makes the disease life-threatening, and the swelling elsewhere contributes to the significant burden of illness. The increased risk for mental health disorders in HAE is due to the burden of disease and possibly associated activation of the immune system. To determine the prevalence of depression and anxiety in HAE patients and the most high-yield features of depression to target in a clinical encounter. Depression and anxiety symptoms were evaluated using the 29 items of the Hamilton Depression Rating Scale along with the 14-item Hamilton Anxiety Rating Scale. The sample size was 26 participants with a diagnosis of type 1 or 2 HAE drawn from a cohort of 60 adult patients. In addition, a literature search was performed regarding how immune modulation affects depression and anxiety. A total of 39% of participants were identified as experiencing depression of mild (50%), moderate (40%), or severe (10%) levels. Fifteen percent of participants displayed prominent anxiety, half of whom had mild anxiety, 25% moderate anxiety, and 25% severe anxiety. The literature on inflammation and depression suggests a possible link between HAE and depression. Our data and the literature support that depression and anxiety symptoms are common in patients with HAE and may be secondary to chronic disease burden, associated pathophysiologic features, or both. Treatment that addresses the psychosocial and mental health of HAE patients is critical for best practice. Copyright © 2014 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  7. Clinical Pattern and Acute and Long-term Management of Hereditary Angioedema Due to C1-Esterase Inhibitor Deficiency.

    Science.gov (United States)

    Gómez-Traseira, C; Pérez-Fernández, E; López-Serrano, M C; García-Ara, M C; Pedrosa, M; López-Trascasa, M; Caballero, T

    2015-01-01

    Hereditary angioedema due to C1-esterase inhibitor deficiency (HAE-C1-INH) is a life-threatening disease. To describe the clinical characteristics and management of patients with HAE-C1-INH during routine clinical practice. An observational, retrospective study was performed in patients with HAE-C1-INH. Demographic, clinical, and analytical data were collected from 2 periods: period A (October 2009-September 2010) and period B (October 2007-September 2009). We studied 112 patients with HAE-C1-INH (57.1% females). Age at onset of symptoms was 14.4 years (lower in patients who had experienced attacks in the previous year). In period B (n=87), 62.1% of patients presented at least 1 edema attack (median, 3.5 attacks/patient/2 years), and 19.1% of attacks were treated. In period A (n=77), 58.4% of patients were on maintenance therapy. Stanozolol was the most widely used drug (48.9%), with a mean weekly dose of 6.7 mg. At least 1 attack was recorded in 72.7% of patients (median, 3.0 attacks/patient/year), and 31.5% of the attacks were treated. Treatment of acute attacks increased by 12.4%. Age at onset of symptoms is associated with clinical expression of disease. The higher age at onset of symptoms, the fewer number of attacks per patient and year, and the lower dose of attenuated androgens necessary to control the disease than in other series lead us to hypothesize that HAE-C1-INH could have a less severe expression in Spain. Acute attacks seem to be treated increasingly often.

  8. Hereditary angioedema type I in a female patient: a case report

    OpenAIRE

    PRKAČIN, INGRID; BAN, ANA; CAVRIĆ, GORDANA; BARTOLEK HAMP, DUBRAVKA

    2014-01-01

    Hereditary angioedema (HAE) is rare autosomal dominant disease, characterised by spontaneous and recurrent swellings in various parts of the body. The main inflammatory factor in HAE is bradykinin (a key mediator of non-allergic angioedema) and it is responsible for capillary leak. C1 esterase inhibitor (C1-INH) is a protease inhibitor that blocks the activation of the classic complement pathway, but there are also many others biochemical pathways, including kinin. Type I HAE is defi...

  9. Effectiveness of icatibant for treatment of hereditary angioedema attacks is not affected by body weight

    DEFF Research Database (Denmark)

    Caballero, Teresa; Zanichelli, Andrea; Aberer, Werner

    2018-01-01

    in the literature. We examined disease characteristics and icatibant treatment effectiveness in patients stratified by BMI in the Icatibant Outcome Survey, an ongoing, international, observational study monitoring the real-world safety and effectiveness of icatibant. Methods: Attack and treatment characteristics......) were analyzed. There was no significant difference in the frequency and severity of attacks across BMI groups, although obese patients tended to have more attacks of high severity. There was no impact of BMI on the frequency of laryngeal attacks, but patients with normal BMI had fewer cutaneous attacks......) and treated attacks earlier than patients with normal BMI (P = 0.007). Furthermore, time to resolution and duration of attack were shorter for patients with high BMI (P normal). Conclusion: Overall, icatibant was comparatively effective in treating attacks...

  10. Clinical characteristics and real-life diagnostic approaches in all Danish children with hereditary angioedema

    DEFF Research Database (Denmark)

    Aabom, Anne; Andersen, Klaus E; Fagerberg, Christina

    2017-01-01

    BACKGROUND: With a potentially early onset, hereditary angioedema (HAE) requires special knowledge also in infancy and early childhood. In children from families with HAE, the diagnosis should be confirmed or refuted early, which can be difficult. Studies of childhood HAE and the diagnostic...

  11. Perioperative course in patients with hereditary or acquired angioedema.

    Science.gov (United States)

    MacBeth, Lisa S; Volcheck, Gerald W; Sprung, Juraj; Weingarten, Toby N

    2016-11-01

    Two types of bradykinin-mediated angioedema, hereditary angioedema (HAE) and acquired angioedema (AAE), result from deficiency or dysfunction of C1 esterase inhibitor, leading to an overproduction of bradykinin, which can lead to vascular permeability and life-threatening angioedema of the airway. The objective of this study was to review perioperative outcomes in a series of patients with HAE and AAE and to review current knowledge about anesthetic complications in patients with HAE or AAE. Medical records were retrospectively reviewed for perioperative complications in patients with HAE or AAE who underwent general anesthesia from January 1, 2000, to December 31, 2014, at our institution. Twenty-four patients (13 with HAE, 10 with AAE, and 1 with unspecified angioedema) underwent 38 instances of general anesthesia with airway manipulation. All except 4 received prophylactic therapy. One patient, a 67-year-old woman who was pretreated with stanozolol and fresh frozen plasma required reintubation after postoperative airway edema developed. Life-threatening episodes of angioedema of the airway occur infrequently, but they can occur in patients who received pretreatment and in patients who have previously undergone anesthesia uneventfully. Anesthesiologists must be ready to emergently manage a difficult airway and must be familiar with recommendations provided in consensus guidelines for the treatment of HAE and AAE patients. Copyright © 2016 Elsevier Inc. All rights reserved.

  12. HAE international home therapy consensus document

    DEFF Research Database (Denmark)

    Longhurst, Hilary J; Farkas, Henriette; Craig, Timothy

    2010-01-01

    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....

  13. Hereditary and acquired C1-inhibitor-dependent angioedema: from pathophysiology to treatment.

    Science.gov (United States)

    Zeerleder, Sacha; Levi, Marcel

    2016-01-01

    Uncontrolled generation of bradykinin (BK) due to insufficient levels of protease inhibitors controlling contact phase (CP) activation, increased activity of CP proteins, and/or inadequate degradation of BK into inactive peptides increases vascular permeability via BK-receptor 2 (BKR2) and results in subcutaneous and submucosal edema formation. Hereditary and acquired angioedema due to C1-inhibitor deficiency (C1-INH-HAE and -AAE) are diseases characterized by serious and potentially fatal attacks of subcutaneous and submucosal edemas of upper airways, facial structures, abdomen, and extremities, due to inadequate control of BK generation. A decreased activity of C1-inhibitor is the hallmark of C1-INH-HAE (types 1 and 2) due to a mutation in the C1-inhibitor gene, whereas the deficiency in C1-inhibitor in C1-INH-AAE is the result of autoimmune phenomena. In HAE with normal C1-inhibitor, a significant percentage of patients have an increased activity of factor XIIa due to a FXII mutation (FXII-HAE). Treatment of C1-inhibitor-dependent angioedema focuses on restoring control of BK generation by inhibition of CP proteases by correcting the balance between CP inhibitors and BK breakdown or by inhibition of BK-mediated effects at the BKR2 on endothelial cells. This review will address the pathophysiology, clinical picture, diagnosis and available treatment in C1-inhibitor-dependent angioedema focusing on BK-release and its regulation. Key Messages Inadequate control of bradykinin formation results in the formation of characteristic subcutaneous and submucosal edemas of the skin, upper airways, facial structures, abdomen and extremities as seen in hereditary and acquired C1-inhibitor-dependent angioedema. Diagnosis of hereditary and acquired C1-inhibitor-dependent angioedema may be troublesome as illustrated by the fact that there is a significant delay in diagnosis; a certain grade of suspicion is therefore crucial for quick diagnosis. Submucosal edema formation in

  14. Angioedema: Clinical and Etiological Aspects

    Directory of Open Access Journals (Sweden)

    Kanokvalai Kulthanan

    2007-01-01

    Full Text Available Angioedema is an abrupt swelling of the skin, mucous membrane, or both including respiratory and gastrointestinal tracts. This study aimed to report an experience of angioedema in a university hospital with respect to etiologies, clinical features, treatment, and outcome. One hundred and five patients were enrolled. About half had angioedema without urticaria. The common sites of involvement were periorbital area and lips. Forty five patients (49% had systemic symptoms. The most common cause of angioedema was allergic angioedema. Nonsteroidal anti-inflammatory drug-induced angioedema and idiopathic angioedema were detected in 20% and 18%, respectively. Among patients with allergic angioedema, 41.7% were caused by food, 39.6% by drugs. Thirty seven patients (39% had recurrent attacks of angioedema. Mean standard deviation (SD number of attacks in patients with recurrent angioedema was 3.9 (2.7 (ranging from 2 to 10 times. Patients who had older age and multiple sites of skin involvement had tendency to have systemic symptoms.

  15. Histaminergic Angioedema.

    Science.gov (United States)

    Busse, Paula J; Smith, Tukisa

    2017-08-01

    Angioedema is frequently categorized into histamine- or bradykinin-mediated disease. It is critical to determine the underlying mediator of symptoms as it directs treatment. Histaminergic angioedema is the most frequent cause of angioedema. It is classified as either acute (lasting 6 weeks). It is further classified into angioedema presenting with or without urticaria. Some patients with acute angioedema may have disease that becomes chronic. Mast cells and basophils are central to the underlying pathophysiology of histamine-mediated angioedema. The underlying treatments of histamine-mediated angioedema are antihistamines, corticosteroids, and epinephrine. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    DEFF Research Database (Denmark)

    Andersen, Michelle Fog; Bygum, Anette

    2015-01-01

    Hereditary angioedema is a rare, but potentially life-threatening genetic disorder that results from an autosomal dominant trait. It is characterized by acute, recurrent attacks of severe local edema, most commonly affecting the skin and mucosa. Swelling in hereditary angioedema patients does...... however not always have to be caused by angioedema but can relate to other concomitant disorders. In this report we are focusing on misdiagnosis in a patient with known hereditary angioedema, whose bleeding episode caused by idiopathic thrombocytopenic purpura was mistaken for an acute attack...... of hereditary angioedema. The case illustrates how clinicians can have difficulties in handling patients with rare diseases, especially in the emergency care setting....

  17. Burden of Illness in Hereditary Angioedema

    DEFF Research Database (Denmark)

    Bygum, Anette; Aygören-Pürsün, Emel; Beusterien, Kathleen

    2015-01-01

    The objective of the Hereditary Angioedema Burden of Illness Study in Europe was to assess the real-world experience of HAE from the patient perspective. Based on open-ended qualitative interviews with 30 patients from Spain, Germany and Denmark, 5 key themes emerged characterizing the impact...

  18. Patients perception of self-administrated medication in the treatment of hereditary angioedema.

    Science.gov (United States)

    Wang, Adrian; Fouche, Andrew; Craig, Timothy J

    2015-08-01

    Early therapy of hereditary angioedema (HAE) decreases morbidity, improves outcomes, decreases absenteeism, and possibly decreases mortality. This can be accomplished best with self-therapy. Previously, the authors examined barriers to self-therapy from the perspective of the nurse and the physician, but data are lacking on what patients perceive as major barriers to self-administered therapy for HAE. To identify those barriers in a prospective fashion by patient interview. After approval from the institutional review board, a telephone survey was performed of patients with HAE from a database of patients who were recently seen in the clinic. The survey focused on anxiety, depression, stress, concerns regarding method of administration, the ability to inject themselves, and what they perceived as barriers to providing self-care. Ninety-two patients were contacted and 59 agreed to participate. With 69% of those patients currently undergoing self-administered treatment, the results showed minimal depression and anxiety, a high satisfaction with treatment, and significant compliance with treatment. Most of those not yet on self-administered therapy wanted to start despite being satisfied with the care received in the emergency department. They also believed care at home would be optimal. The main concern of the 2 groups was not being able to treat themselves in the event of an HAE attack. From these data, it is obvious that most patients are willing to self-treat. This suggests that physicians should encourage self-treatment of HAE to improve outcomes and quality of life of patients with HAE. Copyright © 2015 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  19. Presence of C1-inhibitor polymers in a subset of patients suffering from hereditary angioedema.

    Directory of Open Access Journals (Sweden)

    Daniel Elenius Madsen

    Full Text Available Hereditary angioedema (HAE is a potentially life-threatening disease caused by mutations in the gene encoding the serine protease inhibitor (serpin C1 inhibitor (C1-inh. The mutations cause decreased functional plasma levels of C1-inh, which triggers unpredictable recurrent edema attacks. Subjects suffering from HAE have been classified in type I patients with decreased functional and antigenic levels of C1-inh, and type II patients with decreased functional but normal antigenic C1-inh levels. However, a few reports have demonstrated that some mutations cause C1-inh polymerization in vitro, and it is speculated that C1-inh polymers may exist in patient plasma, challenging the current classification of HAE patients. To investigate the presence of C1-inh polymers in patient plasma samples, we developed an immunological method, where monoclonal antibodies produced against polymerized C1-inh were applied in native PAGE western blotting. Using this approach we analyzed genuine plasma samples from 31 Danish HAE families, and found that plasma samples from three genotypically distinct HAE type I families (classified upon C1-inh plasma concentrations contained C1-inh polymers. Identical C1-inh polymerization phenotypes were observed in four affected family members from one of these families. Genotyping of the families revealed that the polymerogenic mutations of two families were located in proximity to the reactive center loop insertion site in C1-inh (p.Ile271Thr and p.Ser258_Pro260del,and one mutation affected helix C (p.Thr167Asn. In conclusion, we demonstrate that C1-inh polymers are present in the plasma of a subgroup of HAE type I patients.

  20. Tranexamic acid as maintenance treatment for non-histaminergic angioedema: analysis of efficacy and safety in 37 patients

    Science.gov (United States)

    Wintenberger, C; Boccon-Gibod, I; Launay, D; Fain, O; Kanny, G; Jeandel, P Y; Martin, L; Gompel, A; Bouillet, L

    2014-01-01

    Angioedema (AE) is a clinical syndrome characterized by localised swelling lasting several hours. The swelling is often recurring and can be lethal if it is located in the laryngeal region. Much progress has been made recently in the treatment of acute episodes, but no consensus has been reached on maintenance treatment. We have performed a national retrospective observational study to assess the use of tranexamic acid (TA) as maintenance treatment for non-histaminergic AE [hereditary AE (HAE) or idiopathic non-histaminergic AE]. Records for 64 cases were collected from 1 October 2012 to 31 August 2013; 37 of these were included (12 HAE with C1-inhibitor deficiency, six with HAE with normal C1-inhibitor and 19 idiopathic non-histaminergic AE). When treated with TA over six months, the number of attacks was reduced by 75% in 17 patients, 10 patients showed a lower level of reduction and 10 had the same number of attacks. In no instances were symptoms increased. No thromboembolic events were observed, and the main side effects were digestive in nature. Thus, TA, which is well tolerated and inexpensive, appears to be an effective maintenance treatment for some patients with HAE or idiopathic non-histaminergic AE. PMID:24827773

  1. Angioedema hereditario: Historia familiar y manifestaciones clínicas en 58 pacientes Hereditary angioedema: Family history and clinical manifestations in 58 patients

    Directory of Open Access Journals (Sweden)

    Diego S. Fernández Romero

    2009-12-01

    Full Text Available El angioedema hereditario (AEH es una enfermedad rara, autosómica dominante, caracterizada por episodios de angioedema que comprometen la piel, el tracto gastrointestinal y la laringe. Analizamos las características epidemiológicas y clínicas en una serie de 58 pacientes, 53 (91% con diagnóstico de AEH tipo I y 5 (9% con tipo II. La edad media al inicio fue de 10.8 ± 9.5 años (0.1 a 59 y de 25.8 ± 16.2 años (2 a 77 en el momento del diagnóstico, con un retraso diagnóstico de 15.3 ± 14.3 años. El promedio de ataques en los 6 meses previos a la consulta fue de 7.4 ± 7.6 (0 a 40. Cincuenta y cuatro (93% presentaron ataques cutáneos, 50 (86% abdominales, 24 (41% laríngeos y 24 (41% cutáneos y abdominales combinados. Veintisiete (46.5% nunca utilizaron medicación preventiva para la enfermedad y 17 (29% recibieron danazol en diferentes dosis por diferentes periodos de tiempo. Durante los ataques, 15 (26% pacientes recibieron C1 inhibidor endovenoso alguna vez, 7 (12% recibieron plasma fresco y 40 (69% tratamiento sintomático. Ansiedad o situaciones de estrés y traumatismos fueron los desencadenantes más frecuentes. Identificamos a 6 (10% pacientes como primera mutación y a 52 (90% con historia familiar previa. Analizamos 20 troncos familiares identificando 205 individuos en riesgo de heredar la enfermedad, 109 (53% de ellos con síntomas o diagnóstico AEH. El total de individuos con síntomas de AEH fue de 145, de los cuales 19 (13% murieron por asfixia. Disminuir el retraso diagnóstico y ofrecer una terapéutica adecuada son desafíos a afrontar en el AEH.Hereditary angioedema (HAE is a rare autosomal dominant disease, characterized by episodes of edema typically involving the skin, gastrointestinal tract and larynx. We here describe the epidemiologic and clinical characteristic of a series of 58 patients with diagnosis of HAE, 53 (91% type I and 5 (9% type II. The mean age at first symptom was 10.8 ± 9.5 years and the mean

  2. Hereditary angioedema: Assessing the hypothesis for underlying autonomic dysfunction.

    Directory of Open Access Journals (Sweden)

    Maddalena A Wu

    Full Text Available Attacks of Hereditary Angioedema due to C1-inhibitor deficiency (C1-INH-HAEare often triggered by stressful events/hormonal changes.Our study evaluates the relationship between autonomic nervous system (ANS and contact/complement system activation.Twenty-three HAE patients (6 males, mean age 47.5±11.4 years during remission and 24 healthy controls (8 males, mean age 45.3±10.6 years were studied. ECG, beat-by-beat blood pressure, respiratory activity were continuously recorded during rest (10' and 75-degrees-head-up tilt (10'. C1-INH, C4, cleaved high molecular weight kininogen (cHK were assessed; in 16 patients and 11 controls plasma catecholamines were also evaluated. Spectral analysis of heart rate variability allowed extraction of low-(LF and high-(HF frequency components, markers of sympathetic and vagal modulation respectively.HAE patients showed higher mean systolic arterial pressure (SAP than controls during both rest and tilt. Tilt induced a significant increase in SAP and its variability only in controls. Although sympathetic modulation (LFnu increased significantly with tilt in both groups, LF/HF ratio, index of sympathovagal balance, increased significantly only in controls. At rest HAE patients showed higher noradrenaline values (301.4±132.9 pg/ml vs 210.5±89.6pg/ml, p = 0.05. Moreover, in patients tilt was associated with a significant increase in cHK, marker of contact system activation (49.5 ± 7.5% after T vs 47.1 ± 7.8% at R, p = 0.01.Our data are consistent with altered ANS modulation in HAE patients, i.e. increased sympathetic activation at rest and blunted response to orthostatic challenge. Tilt test-induced increased HK cleavage suggests a link between stress and bradykinin production.

  3. Hereditary Angioedema

    Directory of Open Access Journals (Sweden)

    A.P. Volokha

    2014-03-01

    Full Text Available Hereditary angioedema — a rare disease caused by a congenital deficiency of C1-inhibitor. Clinical manifestations of the disease — recurrent episodes of angioedema, which manifest in childhood or adolescence, more often localized in the subcutaneous tissue (limbs, face, trunk, genitals or submucosa (intestine, larynx. Swellings of the larynx are potentially harmful to the patient’s life. Early detection enables to carry out timely appropriate treatment and prevention of angioedema. The paper presents a case of hereditary angioedema with manifestation in early childhood. The case study demonstrates the complexity of diagnosis and treatment of hereditary angioedema.

  4. The role of ficolins and MASPs in hereditary angioedema due to C1-inhibitor deficiency

    DEFF Research Database (Denmark)

    Csuka, Dorottya; Munthe-Fog, Lea; Skjoedt, Mikkel-Ole

    2013-01-01

    Hereditary angioedema due to C1-inhibitor deficiency (HAE-C1-INH) causes disturbances in the complement system. However, the influence of HAE-C1-INH on the lectin pathway of complement is unresolved. Thus, we studied the main initiator molecules, enzymes and regulators in the lectin pathway...

  5. Mutational spectrum and phenotypes in Danish families with hereditary angioedema because of C1 inhibitor deficiency

    DEFF Research Database (Denmark)

    Bygum, A; Fagerberg, C R; Ponard, D

    2011-01-01

    Hereditary angioedema (HAE), type I and II, is an autosomal dominant disease with deficiency of functional C1 inhibitor protein causing episodic swellings of skin, mucosa and viscera. HAE is a genetically heterogeneous disease with more than 200 different mutations in the SERPING1 gene. A genotype...

  6. Pediatric Angioedema.

    Science.gov (United States)

    Pattanaik, Debendra; Lieberman, Jay Adam

    2017-08-08

    The aims of this study are to update the clinician on current understanding of angioedema as it presents in the pediatric population and to review proper diagnostic techniques and treatment modalities for various types of angioedema. Angioedema is still best classified by whether it is likely histaminergic or kinin-mediated. New guidelines have been published around the world to help diagnose and treat both forms (urticaria/angioedema and hereditary angioedema). The vast majority of the studies on treatment have been conducted in the adult population; however, there are data available in the pediatric population. In the realm of hereditary angioedema, there are multiple new therapies that have been studied in the pediatric population (down to 2 years in some studies) in recent years and offer the clinician options for treatment. Angioedema (whether occurring with or without urticaria) is common in the pediatric population. The majority of the recent studies has been conducted in hereditary angioedema, and now, the clinician should have various options to treat all forms of angioedema. Many treatment options, especially for hereditary angioedema, are further being examined specifically in the pediatric population.

  7. Functional C1-inhibitor diagnostics in hereditary angioedema: assay evaluation and recommendations

    DEFF Research Database (Denmark)

    Wagenaar-Bos, Ineke G A; Drouet, Christian; Aygören-Pursun, Emel

    2008-01-01

    Hereditary angioedema (HAE) is an autosomal dominant disease characterized by recurrent episodes of potentially life-threatening angioedema. The most widespread underlying genetic deficiency is a heterozygous deficiency of the serine protease inhibitor C1 esterase inhibitor (C1-Inh). In addition ...

  8. Misdiagnosis trends in patients with hereditary angioedema from the real-world clinical setting

    DEFF Research Database (Denmark)

    Zanichelli, Andrea; Longhurst, Hilary J; Maurer, Marcus

    2016-01-01

    BACKGROUND: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) causes swelling in the skin and upper airways and pain in the abdomen because of mucosal swelling. C1-INH-HAE is frequently misdiagnosed, leading to delays in diagnosis, inadequate treatment, and unnecessary procedures...... being diagnosed as having C1-INH-HAE. RESULTS: In January 2016, a total of 418 of 633 IOS patients with C1-INH-HAE type I or II had provided misdiagnosis data. Of these, 185 of 418 (44.3%) received 1 or more prior misdiagnoses. The most common misdiagnoses were allergic angioedema (103 of 185...... patients without (1.7 years; P angioedema or appendicitis. Misdiagnosis results in marked delays in receiving the correct...

  9. Gastrointestinal Manifestations of Hereditary Angioedema Diagnosed by Ultrasound in the Emergency Department

    Directory of Open Access Journals (Sweden)

    Christine Riguzzi

    2014-11-01

    Full Text Available Abdominal angioedema is a less recognized type of angioedema, which can occur in patients with hereditary angioedema (HAE. The clinical signs may range from subtle, diffuse abdominal pain and nausea, to overt peritonitis. We describe two cases of abdominal angioedema in patients with known HAE that were diagnosed in the emergency department by point-of-care (POC ultrasound. In each case, the patient presented with isolated abdominal complaints and no signs of oropharyngeal edema. Findings on POC ultrasound included intraperitoneal free fluid and bowel wall edema. Both patients recovered uneventfully after receiving treatment. Because it can be performed rapidly, requires no ionizing radiation,and can rule out alternative diagnoses, POC ultrasound holds promise as a valuable tool in the evaluation and management of patients with HAE. [West J Emerg Med. 2014;15(7:-0.

  10. Disease expression in women with hereditary angioedema

    DEFF Research Database (Denmark)

    Bouillet, Laurence; Longhurst, Hilary; Boccon-Gibod, Isabelle

    2008-01-01

    OBJECTIVE: Fluctuations in sex hormones can trigger angioedema attacks in women with hereditary angioedema. Combined oral contraceptive therapies, as well as pregnancy, can induce severe attacks. The course of angioedema may be very variable in different women. STUDY DESIGN: Within the PREHAEAT...... project launched by the European Union, data on 150 postpubertal women with hereditary angioedema were collected in 8 countries, using a patient-based questionnaire. RESULTS: Puberty worsened the disease for 62%. Combined oral contraceptives worsened the disease for 79%, whereas progestogen-only pills......-sensitive phenotype for some patients. CONCLUSION: The course of angioedema in women with C1 inhibitor deficiency is affected by physiologic hormonal changes; consequently, physicians should take these into account when advising on management....

  11. Socioeconomic burden of hereditary angioedema

    DEFF Research Database (Denmark)

    Aygören-Pürsün, Emel; Bygum, Anette; Beusterien, Kathleen

    2014-01-01

    .001). Among patients who sought medical care during the last attack (23%), more than half utilized the emergency department. The last attack prevented patients from their normal activities an average of 4-12 hours. Patient and caregiver absenteeism increased with attack severity and frequency. Among patients...... who were working or in school (n = 120), 72 provided work/school absenteeism data, resulting in an estimated 20 days missing from work/school on average per year; 51% (n = 84) indicated that HAE has hindered their career/educational advancement. CONCLUSION: HAE poses a considerable burden on patients...... and their families in terms of direct medical costs and indirect costs related to lost productivity. This burden is substantial at the time of attacks and in between attacks....

  12. Treatment of hereditary angioedema due to C1 inhibitor deficiency in Argentina

    Directory of Open Access Journals (Sweden)

    Eloisa Malbrán

    2017-08-01

    Full Text Available The benefits of the worldwide approval of new drugs for the treatment of acute C1-INH-HAE attacks may still not reach all patients. Identifying the current barriers in the access to medication, as well as conducting a detailed assessment of the progress in this area, is essential to achieve universal treatment. Two hundred and twenty five patients registered in the Argentina Hereditary Angioedema Patient Association (AHAEPA were randomly selected and invited to participate in a web based questionnaire on accessibility to icatibant and pdC1-INH, self-treatment, delay to treatment, and coverage. The data retrieved was compared to our previous reports in 2008 and 2013. We collected 156/225 answers. One hundred and eighteen (76% patients have either pdC1-INH (n = 86, icatibant (n = 10 or both (n = 22, while 38 (24% do not have access to treatment. In 2008, 26% had access while 82% had it in 2013. Thirty-two subjects (22% self-inject themselves, similar to 29% in 2013, even though between studies, widespread self-injection training activities have taken place. However, considering injections by proxy, home treatment reached 56%. Only half of the patients decide to receive treatment early during the attack. Ninety-nine patients (63% have full coverage, thirty (19% have no coverage at all and the rest only obtain partial reimbursement. Twenty-nine families (31% share a single treatment dose of the medication, better than 36% in 2013. Argentina's C1-INH-HAE patients had a sustained improvement in their access to medication. Efforts should continue to further improve accessibility and optimal management of HAE acute attacks to all patients in the country.

  13. The Humanistic, Societal, and Pharmaco-economic Burden of Angioedema.

    Science.gov (United States)

    Longhurst, Hilary; Bygum, Anette

    2016-10-01

    Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare disorder characterized by intermittent and unpredictable episodes of swelling which cause disfigurement, disability, pain, or, in case of laryngeal swelling, risk of death. Historical factors, including the intermittent nature of the disorder, the lack of awareness of this ultra-rare condition amongst medical personnel, lack of specialist centers, and limited treatment options have contributed to under-diagnosis and under-treatment of the condition. Incorrect treatment of attacks has been common, even when medical help is sought. This has lead to reduced health-seeking behavior and alternative coping strategies, sometimes even denial, in many families, while a minority of HAE-affected patients have become serial emergency room attenders with chronic pain and ongoing requirement for opiate-based painkillers. Both strategies have incurred not only physical but also psychological and economic consequences.In the last 10 years, new and effective acute therapies have been made available, some of which have also provided short-term and long-term prophylaxis options, together with a better understanding of older prophylactic drugs. Improved awareness of HAE amongst the general public, family members, and physicians has reduced the long delay in diagnosis and increased the number of patients receiving effective and up-to-date therapies to improve the physical impact of the disorder.Data on the impact of treatment on the psychological outcomes is scarce, but the limited information available suggests that access to specialist advice and treatment leads to psychological as well as physical improvement.HAE also has profound effects on individual and family economic output, directly via absenteeism from school or work and indirectly via lost opportunities. Economic improvements associated with better treatments are offset by the high cost of new acute treatments, resulting in difficult pharmaco

  14. [Bradykinin-induced angioedema: Definition, pathogenesis, clinical presentation, diagnosis and therapy].

    Science.gov (United States)

    Hahn, J; Bas, M; Hoffmann, T K; Greve, J

    2015-12-01

    The incidence of bradykinin-induced angioedema is considerably lower than that of histamine-induced forms; however, the same is true for the clinician's knowledge of this condition. Bradykinin-induced angioedemas include hereditary angioedema (HAE), as well as acquired forms induced by drugs or antibody formation, e.g., during the course of oncologic disease. Drug-induced forms affect almost exclusively the head and neck region, and are thus important for the otorhinolaryngologist. Clear differentiation between histamine-induced angioedema (e. g., connected to allergy/urticaria) and bradykinin-induced angioedema is essential for selection of the specific treatment and may be lifesaving. Antihistamines and cortisone derivatives have no relevant effect in bradykinin induced-angioedema, whereas blood-derived C1 esterase inhibitor and bradykinin receptor 2 antagonists represent effective therapeutic options--both for acute and prophylactic treatment.

  15. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema

    DEFF Research Database (Denmark)

    Bowen, Tom; Cicardi, Marco; Farkas, Henriette

    2010-01-01

    ABSTRACT: BACKGROUND: We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) and updated this as Hereditary angioedema: a current state-of-the-art review: Canadian Hungarian 2007...... International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. OBJECTIVE: To update the International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema (circa 2010). METHODS: The Canadian Hereditary Angioedema Network (CHAEN...... approach. The Consensus document was reviewed at the meeting and then circulated for review. RESULTS: This manuscript is the 2010 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema that resulted from that conference. CONCLUSIONS: Consensus approach is only...

  16. Hereditary angioedema.

    Science.gov (United States)

    Bracho, Francisco A

    2005-11-01

    Hereditary angioedema is an autosomal-dominant deficiency of C1 inhibitor--a serpin inhibitor of kallikrein, C1r, C1s, factor XII, and plasmin. Quantitative or qualitative deficiency of C1 inhibitor leads to the generation of vasoactive mediators, most likely bradykinin. The clinical syndrome is repeated bouts of nonpruritic, nonpitting edema of the face, larynx, extermities, and intestinal viscera. Recently, investigators, physicians, and industry have demonstrated a renewed interest in the biology and treatment of hereditary angioedema. Investigators have generated a C1INH-/- mouse model that has demonstrated the importance of the contact activation system for hereditary angioedema-related vascular permeability. An interactive database of mutations is available electronically. Investigators have continued exploration into mRNA/protein levels. The proceedings of a recent workshop have been impressive in the scope and depth. Clinicians have produced consensus documents and expert reviews. The pharmaceutical industry has initiated clinical trails with novel agents. Hereditary angioedema is often misdiagnosed and poorly treated. Diagnosis requires careful medical and family history and the measurement of functional C1 inhibitor and C4 levels. Attenuated androgens, anti-fibrinolytics, and C1 inhibitor concentrates are used for long-term and preprocedure prophylaxis, but have significant drawbacks. C1 inhibitor concentrates and fresh frozen plasma are available for acute intervention. The mainstays of supportive care are airway monitoring, pain relief, hydration, and control of nausea. New agents such as recombinant C1 inhibitor, kallikrein inhibitors, and bradykinin inhibitors may offer safer and more tolerable treatments.

  17. Hereditary angioedema type III (estrogen-dependent) report of three cases and literature review*

    OpenAIRE

    Miranda, Amanda Rodrigues; de Ue, Ana Paula Fusel; Sabbag, Dominique Vilarinho; Furlani, Wellington de Jesus; de Souza, Patr?cia Karla; Rotta, Osmar

    2013-01-01

    In this article, three cases of hereditary angioedema (HAE) type III (estrogen-dependent or with normal C1 inhibitor) are reported. The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy. There is no change in the C1 inhibitor as happens in other types of hereditary angioedema, and mutations are observed in the encoding gene of the XII factor of coagulation in several patients. The...

  18. Angioedema hereditario: Guía de tratamiento Hereditary angioedema: A therapeutic guide

    Directory of Open Access Journals (Sweden)

    Alejandro Malbrán

    2012-04-01

    Full Text Available El angioedema hereditario (HAE es una enfermedad rara, autosómica dominante, caracterizada por episodios que comprometen la piel, el tracto gastrointestinal y la laringe. Tiene una mortalidad histórica por asfixia del 15 al 50%. Es producida por la deficiencia funcional del C1 inhibidor. La identificación de la bradiquinina como mediador principal ha estimulado el desarrollo de nuevos medicamentos para tratar la enfermedad. El tratamiento del HAE se establece en consensos internacionales. El desarrollo de guías para el tratamiento de la enfermedad permite ordenar el uso de procedimientos diagnósticos y drogas. Describimos aquí algunas características farmacológicas de los medicamentos utilizados en el tratamiento del HAE en la Argentina: el concentrado plasmático de C1 inhibidor, el antagonista de la bradiquinina, icatibant, el andrógeno atenuado danazol y los agentes anti-fibrinolíticos ácidos épsilon aminocaproico (EACA y tranexámico. Asimismo, se describe su forma de uso y del control de los eventos adversos más frecuentes, así como las recomendaciones del último consenso internacional, aplicables para conformar una primera guía de tratamiento del HAE en la Argentina.Hereditary angioedema (HAE is a rare autosomal dominant disease, characterized by episodes of edema involving the skin, gastrointestinal tract and larynx. HAE has a historical asphyxia mortality of 15% to 50%. It is the consequence of functional C1 inhibitor deficiency. The identification of bradykinin as the principal mediator of the disease has lead to the development of new drugs for its treatment. HAE management and treatment are agreed by international consensus decision. A therapeutic guide for the treatment of the disease is important to improve diagnosis and treatment. We here describe the pharmacology of drugs available for the treatment of HAE in Argentina: plasma derived C1 Inhibitor, the bradykinin antagonist: icatibant, the attenuated androgen

  19. Emerging concepts in the diagnosis and treatment of patients with undifferentiated angioedema

    Science.gov (United States)

    2012-01-01

    Angioedema is a sudden, transient swelling of well-demarcated areas of the dermis, subcutaneous tissue, mucosa, and submucosal tissues that can occur with or without urticaria. Up to 25% of people in the US will experience an episode of urticaria or angioedema during their lifetime, and many will present to the emergency department with an acute attack. Most cases of angioedema are attributable to the vasoactive mediators histamine and bradykinin. Histamine-mediated (allergic) angioedema occurs through a type I hypersensitivity reaction, whereas bradykinin-mediated (non-allergic) angioedema is iatrogenic or hereditary in origin. Although their clinical presentations bear similarities, the treatment algorithm for histamine-mediated angioedema differs significantly from that for bradykinin-mediated angioedema. Corticosteroids, and epinephrine are effective in the management of histamine-mediated angioedema but are ineffective in the management of bradykinin-mediated angioedema. Recent advancements in the understanding of angioedema have yielded pharmacologic treatment options for hereditary angioedema, a rare hereditary form of bradykinin-mediated angioedema. These novel therapies include a kallikrein inhibitor (ecallantide) and a bradykinin β2 receptor antagonist (icatibant). The physician’s ability to distinguish between these types of angioedema is critical in optimizing outcomes in the acute care setting with appropriate treatment. This article reviews the pathophysiologic mechanisms, clinical presentations, and diagnostic laboratory evaluation of angioedema, along with acute management strategies for attacks. PMID:23131076

  20. An ABC of the Warning Signs of Hereditary Angioedema

    DEFF Research Database (Denmark)

    Grumach, Anete Sevciovic; Ferraroni, Natasha; Olivares, Maria Margarita

    2017-01-01

    are common. HAE attacks may be fatal when upper-airway edema occurs, if proper treatment with a C1 inhibitor concentrate or BK receptor antagonist is not administered or an emergency tracheostomy is not performed. We propose a mnemonic method for the warning signs of HAE for the use as a diagnostic tool, i...

  1. Shortened Activated Partial Thromboplastin Time May Help in Diagnosing Hereditary and Acquired Angioedema.

    Science.gov (United States)

    Bork, Konrad; Witzke, Günther

    2016-01-01

    To evaluate whether activated partial thromboplastin time (APTT) could be used in the laboratory diagnosis of hereditary or acquired angioedema (HAE or AAE) with and without C1 inhibitor (C1-INH) deficiency. In a prospective investigation, APTT and other coagulation parameters were determined in 149 adult patients with various types of angioedema and in 26 healthy participants (HP). Mean APTT was significantly shortened in HAE-C1-INH type I (p angioedema, 4/30 (13.3%) patients with nonhistaminergic angioedema and in 2/26 (7.7%) HP. Thus, a shortened APTT was obtained in 8-9 times more patients with angioedema due to C1-INH deficiency when compared to patients with various forms of angioedema with normal C1-INH and also to HP. A shortened APTT may help to diagnose HAE-C1-INH and AAE-C1-INH when determination of C1-INH is not yet available. © 2016 S. Karger AG, Basel.

  2. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema

    Science.gov (United States)

    2010-01-01

    Background We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) and updated this as Hereditary angioedema: a current state-of-the-art review: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. Objective To update the International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema (circa 2010). Methods The Canadian Hereditary Angioedema Network (CHAEN)/Réseau Canadien d'angioédème héréditaire (RCAH) http://www.haecanada.com and cosponsors University of Calgary and the Canadian Society of Allergy and Clinical Immunology (with an unrestricted educational grant from CSL Behring) held our third Conference May 15th to 16th, 2010 in Toronto Canada to update our consensus approach. The Consensus document was reviewed at the meeting and then circulated for review. Results This manuscript is the 2010 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema that resulted from that conference. Conclusions Consensus approach is only an interim guide to a complex disorder such as HAE and should be replaced as soon as possible with large phase III and IV clinical trials, meta analyses, and using data base registry validation of approaches including quality of life and cost benefit analyses, followed by large head-to-head clinical trials and then evidence-based guidelines and standards for HAE disease management. PMID:20667127

  3. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema

    Directory of Open Access Journals (Sweden)

    Bowen Tom

    2010-07-01

    Full Text Available Abstract Background We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency and updated this as Hereditary angioedema: a current state-of-the-art review: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. Objective To update the International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema (circa 2010. Methods The Canadian Hereditary Angioedema Network (CHAEN/Réseau Canadien d'angioédème héréditaire (RCAH http://www.haecanada.com and cosponsors University of Calgary and the Canadian Society of Allergy and Clinical Immunology (with an unrestricted educational grant from CSL Behring held our third Conference May 15th to 16th, 2010 in Toronto Canada to update our consensus approach. The Consensus document was reviewed at the meeting and then circulated for review. Results This manuscript is the 2010 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema that resulted from that conference. Conclusions Consensus approach is only an interim guide to a complex disorder such as HAE and should be replaced as soon as possible with large phase III and IV clinical trials, meta analyses, and using data base registry validation of approaches including quality of life and cost benefit analyses, followed by large head-to-head clinical trials and then evidence-based guidelines and standards for HAE disease management.

  4. Acute dystonia mimicking angioedema of the tongue

    DEFF Research Database (Denmark)

    Rasmussen, Eva Rye; Pallesen, Kristine A U; Bygum, Anette

    2013-01-01

    We report a case of acute dystonia of the face, jaw and tongue caused by metoclopramide and mimicking angioedema. The patient had attacks for several years before the correct diagnosis was made and we present the first ever published video footage of an attack. This adverse drug reaction is known...

  5. The Story of Angioedema: from Quincke to Bradykinin.

    Science.gov (United States)

    Reshef, Avner; Kidon, Mona; Leibovich, Iris

    2016-10-01

    The term "swelling" has been used in the old scriptures to illustrate a change of normal figure and, as such, an expression of illness. It should be noted that in ancient times, human diseases were very often regarded a punishment from God. Hence, it is not surprising that one of the oldest tests for infidelity involved swelling as an inflicted punishment. The great Greek physician Hippocrates (377-460 BC), considered one of the most outstanding figures in the history of medicine and "Father of the Western Medicine," already used the term oídēma to describe swelling of organs. It took many centuries later until the first description of angioedema as a distinct medical entity was minted by Quinke in 1882. The historical progression in angioedema research has been characterized by intermittent "leaps" in interest and scientific achievements. As an example, it took 75 years from the accurate description of hereditary angioedema (HAE) by Osler (1888), until a group of researchers headed by Donaldson (1963) disclosed the central role of C1 inhibitor in angioedema pathophysiology. What followed was a result of a collective effort by many researchers and scientific groups who were able to elucidate the intricate connections between the implicated biochemical pathways. Still, scientific progress was hardly translated into effective therapy, and another 45 years had to elapse until the renewed interest in HAE was boosted by studies on the efficacy and safety of novel therapies about 10 years ago. In the twenty-first century, HAE ceased to be an "orphan disease" and its future is far more optimistic. It is better managed now by specialized angioedema centers, harmonized clinical guidelines, educational programs, laboratory services, and continued basic and clinical research. Patient associations worldwide are offering support and guidance, and governments and healthcare systems are gradually addressing patient and family needs.

  6. Urticaria and Prodromal Symptoms Including Erythema Marginatum in Danish Patients with Hereditary Angioedema

    DEFF Research Database (Denmark)

    Rasmussen, Eva R; Valente de Freitas, Priscila; Bygum, Anette

    2016-01-01

    Erythema marginatum is a characteristic skin rash seen in patients with hereditary angioedema (HAE); however, it can be confused with urticaria, leading to delay in correct diagnosis. The aim of this study was to clarify how often erythema marginatum is misinterpreted as urticaria, potentially...

  7. Safety of C1-Esterase Inhibitor in Acute and Prophylactic Therapy of Hereditary Angioedema

    DEFF Research Database (Denmark)

    Busse, Paula; Bygum, Anette; Edelman, Jonathan

    2014-01-01

    BACKGROUND: The plasma-derived, pasteurized C1-inhibitor (C1-INH) concentrate, Berinert has a 4-decade history of use in hereditary angioedema (HAE), with a substantial literature base that demonstrates safety and efficacy. Thromboembolic events have rarely been reported with C1-INH products, typ...

  8. International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

    OpenAIRE

    Farkas, H.; Martinez?Saguer, I.; Bork, K.; Bowen, T.; Craig, T.; Frank, M.; Germenis, A. E.; Grumach, A. S.; Luczay, A.; Varga, L.; Zanichelli, A.; Aberer, Werner; Andrejevic, Sladjana; Aygoeren?P?rs?n, Emel; Banerji, Alena

    2016-01-01

    BACKGROUND: The consensus documents published to date on hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients. We intended to produce consensus recommendations for the diagnosis and management of pediatric patients with C1-INH-HAE.METHODS: During an expert panel meeting that took place during the 9th C1 Inhibitor Deficiency Workshop in Budapest, 2015 (www.haenet.hu), ped...

  9. Use of a C1 Inhibitor Concentrate in Adults ≥65 Years of Age with Hereditary Angioedema

    DEFF Research Database (Denmark)

    Bygum, Anette; Martinez-Saguer, Inmaculada; Bas, Murat

    2016-01-01

    BACKGROUND: Treatment of hereditary angioedema (HAE) in 'older adults' (those aged ≥65 years) has not been well studied. The international Berinert Patient Registry collected data on the use of intravenous plasma-derived, pasteurized, nanofiltered C1-inhibitor concentrate (pnfC1-INH; Berinert......(®)/CSL Behring) in patients of any age, including many older adults. METHODS: This observational registry, conducted from 2010 to 2014 at 30 US and seven European sites, gathered prospective (post-enrollment) and retrospective (pre-enrollment) usage and adverse event (AE) data on subjects treated with pnfC1-INH....... RESULTS: The registry documented 1701 pnfC1-INH infusions in 27 older adults. A total of 1511 HAE attacks treated with pnfC1-INH administration were reported among 25 of the 27 (92.6 %) older adults. Among the older adults, mean (standard deviation [SD]) (8.8 [4.1] IU/kg) and median (6.4 IU/kg) pnfC1-INH...

  10. Evidence of impaired sense of smell in hereditary angioedema.

    Science.gov (United States)

    Perricone, C; Agmon-Levin, N; Shoenfeld, N; de Carolis, C; Guarino, M D; Gigliucci, G; Milana, I; Novelli, L; Valesini, G; Perricone, R; Shoenfeld, Y

    2011-01-01

    Hereditary angioedema (HAE) is an autosomal-dominant disorder resulting from C1-inhibitor (C1INH) deficiency. Smell impairments were found in patients affected with systemic lupus erythematosus, that, similarly to HAE, is characterized by the activation of the classical complement pathway with C4 consumption. In this study, we aimed at evaluating the sense of smell in patients with HAE. Thirty patients with HAE and 30 healthy age- and sex-matched controls were evaluated for olfactory functions using the 3-stages Sniffin'-Sticks kit (threshold, discrimination, and identification [TDI]). TDI scores were analyzed according to complement levels (C1INH, C3, C4 and CH50), Beck depression inventory (BDI-II) and danazol treatment. A significant decrease in olfactory function was observed in patients affected with HAE compared with controls in total TDI score (P < 0.001), and in the discrimination (P < 0.001) and identification scores (P = 0.012). Anosmia was present only in patients with HAE (3.3%) who also exhibited more frequently hyposmia (53.3%vs 3.3%, P < 0.0001). Complement levels were reduced in patients with HAE. C4 serum levels showed positive correlation with total TDI score (P < 0.001), and with discrimination (P = 0.002) and identification (P = 0.011) scores. CH50 complement levels showed positive correlation with total TDI score (P < 0.001), and with threshold (P = 0.002) and discrimination (P = 0.011) scores. Sex, age, danazol treatment, BDI-II scores were not different between the patients and controls and did not influence TDI scores significantly. Evidence for an impaired sense of smell was found in patients with HAE. The reduction in olfactory function in these cases seems to correlate with complement C4 and CH50 levels. Immune and genetic mechanisms might play a role in this defect. © 2010 John Wiley & Sons A/S.

  11. Angioedema: etiology, pathophysiology, current and emerging therapies.

    Science.gov (United States)

    Lewis, Lawrence M

    2013-11-01

    Angioedema (AE) is characterized by nonpitting edema of the dermis and subcutaneous layers. The most common sites of involvement are the tongue, lips, face, and throat; however, swelling can also occur in the extremities, genitalia, and viscera. Life-threatening airway swelling can also occur. AE may be allergic or nonallergic. The overall lifetime incidence of AE is reported to be as high as 15%. This article summarizes the etiology, pathophysiology, and current treatment of several forms of nonallergic AE (including hereditary, acquired, and idiopathic AE) and focuses on angiotensin-converting enzyme inhibitor-induced angioedema (ACEi-AE), which is responsible for 30%-40% of all AE seen in United States emergency departments. Although the triggers, which are primary biologic mechanisms, and treatments for ACEi-AE may differ from those of the hereditary and acquired forms of AE, the clinical effects of ACEi-AE are mediated through a shared pathway, the kallikrein-kinin system. Thus, although current therapeutic options for ACEi-AE are limited, recent advances in the treatment of hereditary AE (HAE) appear promising for improving the outcomes of patients with ACEi-AE. New HAE medications that correct imbalances in the kallikrein-kinin system may prove safe and efficacious in the treatment of ACEi-AE. Copyright © 2013 Elsevier Inc. All rights reserved.

  12. Hereditary Angioedema - Consequences of a New Treatment Paradigm in Denmark

    DEFF Research Database (Denmark)

    Bygum, Anette

    2014-01-01

    Experiences from a Danish patient cohort with hereditary angioedema are reported with focus on home therapy and burden of illness. Eighty patients have been prospectively followed over 11 years, having experienced a total of 7,809 attacks over 469 patient years. More than half of the patients...... stopped long-term prophylaxis with danazol or tranexamic acid and changed treatment regimen to on-demand treatment with C1 inhibitor concentrate or icatibant. At least 10% of the attacks remained un-treated. More than half of the patients felt that hereditary angioedema had a significant psychological...... therapy has profoundly improved the lives of hereditary angioedema patients....

  13. Angiotensin Converting Enzyme Inhibitor-related Angioedema: A Case of an Unexpected Death.

    Science.gov (United States)

    Atalay, Eray; Özdemir, Mehmet Tamer; Çiğsar, Gülşen; Omurca, Ferhat; Aslan, Nurullah; Yildiz, Mehmet; Gey, Zehra Bahar

    2015-12-01

    Angioedema is an asymmetric non-pitting oedema on face, lips, tongue and mucous membranes; any delay in diagnosis and treatment can be fatal. Treatment with lisinopril as an angiotensin converting enzyme (ACE) inhibitor, can be a reason of angioedema. Here we report a case who developed oral-facial edema four years after using lisinopril/hydrochlorothiazide. Laryngeal oedema is a main cause of death in angioedema. The treatment of choice in angioedema including fresh frozen plasma, C1 inhibitor concentrations and BRK-2 antagonists (bradykinin B2 receptor antagonists) were used. In this case; a 77 years old female patient suffering from hypertension was considered. This patient was suffering two days from swelling on her face and neck. Non- allergic angioedema was distinguished in five major forms; acquired (AAO), hereditary (HAE), renin-angiotensin-aldosterone system (RAAS) blocker-dependent, pseudoallergic angioedema (PAS) and an idiopathic angioedema (IAO). She was admitted to our clinic with the diagnosis of hereditary angioedema. Patient had skin edema and life threatening laryngeal edema. In emergency department treatment was started using intravenous methylprednisolone, diphenydramine as well as inhaled and subcutaneous epinephrine simultaneously. Despite the initial treatment, the patient died due to the insufficient respiration and cardiac arrest. The patient has no history of kidney disease.

  14. International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency

    DEFF Research Database (Denmark)

    Caballero, Teresa; Farkas, Henriette; Bouillet, Laurence

    2012-01-01

    BACKGROUND: There are a limited number of publications on the management of gynecologic/obstetric events in female patients with hereditary angioedema caused by C1 inhibitor deficiency (HAE-C1-INH). OBJECTIVE: We sought to elaborate guidelines for optimizing the management of gynecologic/obstetri......BACKGROUND: There are a limited number of publications on the management of gynecologic/obstetric events in female patients with hereditary angioedema caused by C1 inhibitor deficiency (HAE-C1-INH). OBJECTIVE: We sought to elaborate guidelines for optimizing the management of gynecologic...... section. Regional anesthesia is preferred to endotracheal intubation. Breast cancer: Attenuated androgens should be avoided. Antiestrogens can worsen angioedema symptoms. In these cases anastrozole might be an alternative. Other issues addressed include special features of HAE-C1-INH treatment in female...

  15. Genetics Home Reference: hereditary angioedema

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Hereditary angioedema Hereditary angioedema Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Hereditary angioedema is a disorder characterized by recurrent episodes of ...

  16. International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

    DEFF Research Database (Denmark)

    Farkas, H; Martinez-Saguer, I; Bork, K

    2017-01-01

    BACKGROUND: The consensus documents published to date on hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients. We intended to produce consensus recommendations for the diagn...

  17. [Hereditary angioedema: strange cause of abdominal pain].

    Science.gov (United States)

    Salas-Lozano, Nereo Guillermo; Meza-Cardona, Javier; González-Fernández, Coty; Pineda-Figueroa, Laura; de Ariño-Suárez, Mauricio

    2014-01-01

    Hereditary angioedema is an episodic swelling disorder with autosomal dominant inheritance characterized by sudden attacks of peripheral swelling. Patients also commonly have episodic swelling of the wall of hollow viscera, including the bowel. We present a 33-year-old previously healthy male with a complaint of acute-onset intense abdominal pain localized in the epigastrium. Pain irradiated to the right lower quadrant and was associated with five episodes of vomiting. Computed tomography showed thickening of the duodenal wall with liquid in the subphrenic space. Complementary laboratory tests showed low C4 complement levels (5.5 mg/dl) and 30% complement C1 inhibitor activity. Hereditary angioedema is caused by a deficiency (type I) or dysfunction (type II) in complement C1 inhibitor. Abdominal associated with angioedema may manifest as severe acute-onset abdominal pain or as moderately severe chronic recurrent abdominal pain. Two medications are currently FDA-approved for the treatment of these patients.

  18. Immunosafety of recombinant human C1-inhibitor in hereditary angioedema: evaluation of ige antibodies.

    Science.gov (United States)

    Hack, C Erik; Relan, Anurag; Baboeram, Aartie; Oortwijn, Beatrijs; Versteeg, Serge; van Ree, Ronald; Pijpstra, Rienk

    2013-04-01

    Recombinant human C1-inhibitor (rhC1INH) purified from milk of transgenic rabbits is used for the treatment of acute attacks in patients with hereditary angioedema (HAE) due to C1-inhibitor (C1INH) deficiency. The objective was to investigate the risk of rhC1INH inducing IgE antibodies or eliciting anaphylactic reactions. In subjects treated with rhC1INH, we retrospectively analysed the frequency and clinical relevance of pre-exposure and potentially newly induced IgE antibodies against rabbit and other animal allergens including cow's milk by the ImmunoCAP(®) Specific IgE blood test system. 130 HAE patients and 14 healthy subjects received 300 administrations of rhC1INH, 65 subjects (47.4 %) on one occasion; 72 (52.6 %) on at least two occasions (range 2-12; median 2). Five subjects had pre-existing anti-rabbit epithelium IgE; the subject with the highest levels and a previously undisclosed rabbit allergy developed an anaphylactic reaction upon first exposure to rhC1INH, whereas the other four subjects with lower pre-existing IgE levels (Class 1-3), did not. No other anaphylactic reactions were identified in any of the subjects exposed to rhC1INH. Analysis of post-exposure samples revealed that the risk of inducing new or boosting existing IgE responses to rabbit or cow's milk allergens was negligible. The propensity of rhC1INH to induce IgE antibodies following repeated administration of rhC1INH is low. Subjects with substantially elevated anti-rabbit epithelium IgE antibodies and/or clinical allergy to rabbits may have an increased risk for an allergic reaction. No other risk factors for allergic reactions to rhC1INH have been identified.

  19. Depressed activation of the lectin pathway of complement in hereditary angioedema

    DEFF Research Database (Denmark)

    Varga, L; Széplaki, G; Laki, J

    2008-01-01

    ) in three complement activation pathways. Functional activity of the CP, LP and AP were measured in the sera of 68 adult patients with hereditary angioedema (HAE) and 64 healthy controls. In addition, the level of C1q, MBL, MBL-associated serine protease-2 (MASP-2), C4-, C3- and C1INH was measured...... by standard laboratory methods. MBL-2 genotypes were determined by polymerase chain reaction. Besides the complement alterations (low CP and C1INH activity, low C4-, C1INH concentrations), which characterize HAE, the level of MASP-2 was also lower (P = 0.0001) in patients compared with controls. Depressed LP...

  20. Drug-induced angioedema: experience of Italian emergency departments.

    Science.gov (United States)

    Bertazzoni, G; Spina, M T; Scarpellini, M G; Buccelletti, F; De Simone, M; Gregori, M; Valeriano, V; Pugliese, F R; Ruggieri, M P; Magnanti, M; Susi, B; Minetola, L; Zulli, L; D'Ambrogio, F

    2014-06-01

    Acute angioedema represents a cause of admission to the emergency department requiring rapid diagnosis and appropriate management to prevent airway obstruction. Several drugs, including angiotensin-converting enzyme inhibitors (ACE-I), nonsteroidal anti-inflammatory drugs (NSAIDs) and oral antidiabetics, have been reported to induce angioedema. The aim of this prospective observational study conducted in a setting of routine emergency care was to evaluate the incidence and extent of drug-induced non-histaminergic angioedema in this specific clinical setting, and to identify the class of drugs possibly associated with angioedema. Patients admitted to seven different emergency departments (EDs) in Rome with the diagnosis of angioedema and urticaria were enrolled during a 6-month period. Of the 120,000 patients admitted at the EDs, 447 (0.37 %) were coded as having angioedema and 655 (0.5 %) as having urticaria. After accurate clinical review, 62 cases were defined as drug-induced, non-histaminergic angioedema. NSAIDs were the most frequent drugs (taken by 22 out of 62 patients) associated with the angioedema attack. Of the remaining patients, 15 received antibiotic treatment and 10 antihypertensive treatment. In addition, we observed in our series some cases of angioedema associated with drugs (such as antiasthmatics, antidiarrheal and antiepileptics) of which there are few descriptions in the literature. The present data, which add much needed information to the existing limited literature on drug-induced angioedema in the clinical emergency department setting, will provide more appropriate diagnosis and management of this potentially life-threatening adverse event.

  1. Angioedema hereditario: Guía de tratamiento

    Directory of Open Access Journals (Sweden)

    Alejandro Malbrán

    2012-04-01

    Full Text Available El angioedema hereditario (HAE es una enfermedad rara, autosómica dominante, caracterizada por episodios que comprometen la piel, el tracto gastrointestinal y la laringe. Tiene una mortalidad histórica por asfixia del 15 al 50%. Es producida por la deficiencia funcional del C1 inhibidor. La identificación de la bradiquinina como mediador principal ha estimulado el desarrollo de nuevos medicamentos para tratar la enfermedad. El tratamiento del HAE se establece en consensos internacionales. El desarrollo de guías para el tratamiento de la enfermedad permite ordenar el uso de procedimientos diagnósticos y drogas. Describimos aquí algunas características farmacológicas de los medicamentos utilizados en el tratamiento del HAE en la Argentina: el concentrado plasmático de C1 inhibidor, el antagonista de la bradiquinina, icatibant, el andrógeno atenuado danazol y los agentes anti-fibrinolíticos ácidos épsilon aminocaproico (EACA y tranexámico. Asimismo, se describe su forma de uso y del control de los eventos adversos más frecuentes, así como las recomendaciones del último consenso internacional, aplicables para conformar una primera guía de tratamiento del HAE en la Argentina.

  2. Complement factor C4 activation in patients with hereditary angioedema

    DEFF Research Database (Denmark)

    Åbom, Anne; Bygum, Anette; Koch, Claus

    2017-01-01

    , that C1-INH-HAE may potentially be overlooked, if screening is performed only by measurement of C4. It has been suggested that measurement of C4 activation products is better suited to avoid false negative results. Our aim was to investigate whether total antigenic C4 or non-functional C4c is a better......Objectives: Low complement factor C4 is usually considered a valuable screening tool for patients with the potentially life-threatening hereditary angioedema with C1-inhibitor (C1-INH) deficiency (C1-INH-HAE). However, there are patients with C1-INH-HAE presenting with normal C4 levels. This means...... measure of the increased C4 activation in C1-INH-HAE patients. Design and methods: Two different monoclonal antibodies (mAb) to human C4 were produced: one had specificity for the β-chain of C4 and would thus react with both functional and non-functional C4, and the other was developed against the factor...

  3. Brazilian guidelines for the diagnosis and treatment of hereditary angioedema

    Directory of Open Access Journals (Sweden)

    Pedro Giavina-Bianchi

    2011-01-01

    Full Text Available Hereditary angioedema is an autosomal dominant disease characterized by edema attacks with multiple organ involvement. It is caused by a quantitative or functional deficiency of the C1 inhibitor, which is a member of the serine protease inhibitor family. Hereditary angioedema is unknown to many health professionals and is therefore an underdiagnosed disease. The causes of death from hereditary angioedema include laryngeal edema with asphyxia. The estimated mortality rate in patients in whom the disease goes undetected and who are therefore incorrectly treated is 25-40%. In addition to edema of the glottis, hereditary angioedema often results in edema of the gastrointestinal tract, which can be incapacitating. Patients with hereditary angioedema may undergo unnecessary surgical interventions because the digestive tract can be the primary or only organ system involved, thus mimicking acute surgical abdomen. It is estimated that patients with hereditary angioedema experience some degree of disability 20-100 days per year. The Experts in Clinical Immunology and Allergy of the "Associação Brasileira de Alergia e Imunopatologia -ASBAI" developed these guidelines for the diagnosis, therapy, and management of hereditary angioedema.

  4. Hereditary angioedema as a metabolic liver disorder: novel therapeutic options and prospects for cure

    Directory of Open Access Journals (Sweden)

    Rohan Ameratuga

    2016-11-01

    Full Text Available Hereditary angioedema (HAE is a rare autosomal dominant disorder caused by mutations of the SERPING1 or the Factor 12 genes. It is potentially fatal, particularly if not identified at an early stage. Apart from androgens, which are contraindicated in children and in pregnant women, a range of effective, albeit very expensive treatments have recently become available for HAE patients. The cost of these new treatments is beyond the reach of most developing countries. At this time, there is no cure for the disorder. In spite of mutations of the SERPING1 gene, autoimmunity and infections are not prominent features of the condition. Here we present the argument that HAE should be viewed primarily as a metabolic liver disorder. This conceptual paradigm shift will stimulate basic research and may facilitate new therapeutic approaches to HAE outlined in this paper. We suggest several novel potential treatment options for HAE from the perspectives of clinical immunology, molecular biology and liver transplantation. Many of these offer the prospect of curing the disorder. The effectiveness of these options are rapidly improving in many cases and their risks are decreasing. Given the very high costs of treating HAE, some of these curative options may become feasible in the next decade.

  5. Safety and Usage of C1-Inhibitor in Hereditary Angioedema

    DEFF Research Database (Denmark)

    Riedl, Marc A; Bygum, Anette; Lumry, William

    2016-01-01

    of this study was to describe safety and usage patterns of pnfC1-INH. METHODS: A multicenter, observational, registry was conducted between 2010 and 2014 at 30 United States and 7 European sites to obtain both prospective (occurring after enrollment) and retrospective (occurring before enrollment) safety...... and usage data on subjects receiving pnfC1-INH for any reason. RESULTS: Of 343 enrolled patients, 318 received 1 or more doses of pnfC1-INH for HAE attacks (11,848 infusions) or for prophylaxis (3142 infusions), comprising the safety population. Median dosages per infusion were 10.8 IU/kg (attack treatment......, international patient registry documented widespread implementation of pnfC1-INH self-administration outside of a health care setting consistent with current HAE guidelines. These real-world data revealed pnfC1-INH usage for a variety of reasons in patients with HAE and showed a high level of safety regardless...

  6. Successful treatment of hereditary angioedema with bradykinin B2-receptor antagonist icatibant.

    Science.gov (United States)

    Krause, Karoline; Metz, Martin; Zuberbier, Torsten; Maurer, Marcus; Magerl, Markus

    2010-04-01

    The bradykinin B2 receptor antagonist icatibant has recently become available for treating hereditary angioedema. Our observations demonstrate icatibant to be effective and safe for the treatment of both, abdominal and cutaneous attacks in a practice setting beyond clinical studies.

  7. Angioedema hereditario: Tratamiento del ataque agudo en la Argentina

    Directory of Open Access Journals (Sweden)

    Alejandro Malbrán

    2014-06-01

    Full Text Available En el mundo, el angioedema hereditario (HAE afecta a 1 de cada 50 000 personas. Produce episodios de angioedema cutáneo, abdominal y laríngeos que generan gran incapacidad. La mortalidad por la enfermedad oscila entre 15 y 50%. Aunque en Argentina un concentrado plasmático de C1 inhibidor (pdC1INH ha estado aprobado y disponible por décadas para el tratamiento del ataque agudo, solo 15 (26% de 58 pacientes había recibido pdC1INH alguna vez hasta el año 2008, y solo 2(3.4% lo usaban regularmente. Luego de la aprobación de los nuevos medicamentos para HAE, incluido el icatibant en Argentina y de la publicación de las guías terapéuticas, 42 (82% de 51 pacientes del grupo original tienen pdC1INH para tratar el próximo ataque. Sin embargo, 16 (18% de estos pacientes continúan sin acceso a la medicación y otros 15 (35.7% acceden a través de otro enfermo en forma espuria. Solo 12 (28.6% de los pacientes con el medicamento puede auto tratarse en su domicilio. La mejora en el acceso a la medicación es importante pero debe extenderse a todos los afectados y facilitarse el auto-tratamiento.

  8. Risk factors associated with severe and recurrent angioedema: an epidemic linked to ACE-inhibitors.

    Science.gov (United States)

    Loftus, Patricia A; Tan, Melin; Patel, Gunj; Lin, Juan; Helman, Sam; Badhey, Arvind; Du, Eugenie; Smith, Richard V; Fried, Marvin P; Ow, Thomas J

    2014-11-01

    To evaluate the etiology and risk factors for severe manifestation and recurrent episodes of angioedema; to evaluate efficacy of short-term and long-term management strategies for angioedema among a high-risk population. Institutional review board-approved retrospective review of a large, urban population. Data from 875 adult patients treated from January 2008 to December 2013 with the diagnosis of angioedema were obtained using the Clinical Looking Glass utility and review of medical records. Demographic and clinicopathologic risk factors were recorded. The major outcomes evaluated were hospital admission, need for airway intervention, and recurrent episodes of angioedema following the first presentation. Initial treatment strategy and follow-up recommendations were also recorded. The most common cause of angioedema was angiotensin converting enzyme inhibitor (ACEi)-induced (496 [56.6%]). Significant risk factors for severe cases of angioedema included older age, Hispanic race, ACEi-induced angioedema type, American Society of Anesthesiologists class III or above, coexistent cardiopulmonary disease, and a positive smoking history. A total of 17.2% of patients experienced recurrent attacks of angioedema; of those patients, 25.9% were still taking an ACEi at subsequent presentation. Risk factors for recurrent episodes included older age, idiopathic angioedema type, and coexistent cardiopulmonary disease. Only 54.1% of patients who experienced ACEi-induced angioedema had electronic medical record documentation of these allergies. Knowledge of risk factors for severe and recurrent episodes of angioedema and improved education for both healthcare providers and patients, specifically related to ACEi use and allergy documentation, may significantly decrease the burden and morbidity of angioedema among high risk populations. 2b. © 2014 The American Laryngological, Rhinological and Otological Society, Inc.

  9. Bradykinin-mediated angioedema: factors associated with admission to an intensive care unit, a multicenter study.

    Science.gov (United States)

    Javaud, Nicolas; Floccard, Bernard; Gontier, Florian; Lapostolle, Frédéric; Boccon-Gibod, Isabelle; Martin, Ludovic; Amarger, Stéphanie; Boumedienne, Abdalia; Boubaya, Marouane; Asfar, Pierre; Coppere, Brigitte; Ollivier, Yann; Bouillet, Laurence; Adnet, Frédéric; Fain, Olivier

    2016-06-01

    Bradykinin-mediated angioedema is characterized by transient attacks of localized edema of subcutaneous or submucosal tissues and can be life-threatening when involving the upper airways. The aim of this study was to determine the features of acute attacks that might be associated with admission to an ICU. We carried out a retrospective, multicenter, observational study in consecutive patients attending one of six reference centers in France for acute bradykinin-mediated angioedema attacks. Patients had been hospitalized for an acute episode at least once previously. Acute attacks requiring ICU admission were compared with acute attacks that had not required ICU admission. Overall, 118 acute attacks in 31 patients were analyzed (10 patients with hereditary angioedema, 19 patients with angiotensin-converting enzyme inhibitor-induced angioedema, and two patients with acquired C1-inhibitor deficiency angioedema). In multivariate analysis, upper airway involvement, corticosteroid, and C1-inhibitor concentrate administration were associated with ICU admission. Seven episodes (18%) needed airway protection. The evolution was favorable in 38 of 39 attacks warranting ICU admission: patients were able to get out of the service (mean ICU stay 4±5 days). One death was observed by asphyxiation because of laryngeal swelling. Upper airway involvement is an independent risk factor for ICU admission. Corticosteroid use, which is an ineffective treatment, and C1-inhibitor concentrate use are factors for ICU admission. The presence of upper airway involvement should be a warning signal that the attack may be severe.

  10. New treatments addressing the pathophysiology of hereditary angioedema

    Directory of Open Access Journals (Sweden)

    Davis Alvin E

    2008-04-01

    Full Text Available Abstract Hereditary angioedema is a serious medical condition caused by a deficiency of C1-inhibitor. The condition is the result of a defect in the gene controlling the synthesis of C1-inhibitor, which regulates the activity of a number of plasma cascade systems. Although the prevalence of hereditary angioedema is low – between 1:10,000 to 1:50,000 – the condition can result in considerable pain, debilitation, reduced quality of life, and even death in those afflicted. Hereditary angioedema presents clinically as cutaneous swelling of the extremities, face, genitals, and trunk, or painful swelling of the gastrointestinal mucosa. Angioedema of the upper airways is extremely serious and has resulted in death by asphyxiation. Subnormal levels of C1-inhibitor are associated with the inappropriate activation of a number of pathways – including, in particular, the complement and contact systems, and to some extent, the fibrinolysis and coagulation systems. Current findings indicate bradykinin, a product of contact system activation, as the primary mediator of angioedema in patients with C1-inhibitor deficiency. However, other systems may play a role in bradykinin's rapid and excessive generation by depleting available levels of C1-inhibitor. There are currently no effective therapies in the United States to treat acute attacks of hereditary angioedema, and currently available agents used to treat hereditary angioedema prophylactically are suboptimal. Five new agents are, however, in Phase III development. Three of these agents replace C1-inhibitor, directly addressing the underlying cause of hereditary angioedema and re-establishing regulatory control of all pathways and proteases involved in its pathogenesis. These agents include a nano-filtered C1-inhibitor replacement therapy, a pasteurized C1-inhibitor, and a recombinant C1-inhibitor isolated from the milk of transgenic rabbits. All C1-inhibitors are being investigated for acute angioedema

  11. Trifluoperazine-Induced Angioedema

    Directory of Open Access Journals (Sweden)

    Mugtaba Osman

    2014-01-01

    Full Text Available Angioedema is a serious adverse drug reaction that can rarely be associated with trifluoperazine treatment. We present the case of a 44-year-old male with an established diagnosis of schizoaffective disorder, for which trifluoperazine therapy was considered. He presented to the emergency department with bilateral lower limb oedematous painful erythematous swelling that eased off completely when trifluoperazine was stopped. The possibility of allergic reaction, such as angioedema, should always be kept in mind by psychiatrists and mental health professionals when prescribing trifluoperazine antipsychotic.

  12. [Angioedema and urticaria].

    Science.gov (United States)

    Boccon-Gibod, I; Bouillet, L

    2014-11-01

    Angiœdema (AE) is the clinical expression of urticaria (U) which occurs when urticaria is located within the subcutis. It is a syndrome characterized by a sudden and limited subcutaneous and/or submucous swelling. The updated classification of urticaria distinguishes acute and chronic urticaria. Chronic urticaria is spontaneous (CSU) or inducible (CIU). Angioedema in chronic urticaria is rarely allergic, but most of the time caused by a non-specific histamine release from activated mast-cell (non IgE mediated reaction). Angioedemas are recurrent, concomitant or not with wheals. They appear skin-coloured, sometimes slightly rosy, non-inflammatory, and more painful than itchy. They are transient, ephemeral, migrant, last most of the time a few hours (Angioedema can be elicited or worsened by physical factors (cold urticaria, exercise, heat, solar, vibratory, aquagenic, delayed pressure urticaria…) and /or drugs (as aspirin, nonsteroid anti-inflammatory drugs, morphine, antibiotics…). The treatment of histaminergic angioedemas of chronic urticaria is based on modern second generation antihistamines (anti H1). In allergic acute urticaria only, additional treatment for anaphylaxis can be used if needed (grade 2 to 4). In chronic urticaria, steroids should be avoided : they can make symptoms worse and long-lasting because of corticosteroid dependence. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  13. Anaesthetic management of a patient with hereditary angioedema

    Directory of Open Access Journals (Sweden)

    Nergis Ataol

    2015-12-01

    Full Text Available Hereditary angioedema is a rare autosomal dominant disorder caused by reduced activity of the C1 esterase inhibitor. Patients with hereditary angioedema are clinically characterized by recurrent episodes of swelling of the extremities, face, trunk, airways and abdominal organs. Attacks may occur either spontaneously or following trauma, stress, surgery, infections and hormonal fluctuations. The most common cause of death is asphyxia related to laryngeal edema. Giving C1 esterase inhibitor is the most effective method of treatment. Also fresh frozen plasma, androgen steroids, quinine pathway inhibitors, antifibrinolytics and bradykinin receptor antagonists can be used as treatment. In this paper, the anesthetic management of a patient with hereditary angioedema undergoing inguinal hernia repair surgery is reported.

  14. Recurrent angioedema - a case report

    OpenAIRE

    Martins, Sandrina; Salgado, Miguel; Raposo, Filipa; Pinto, Diana; Martinho, Isabel; Araújo, Ana Rita

    2016-01-01

    Introduction: Hereditary angioedema (HA) is a rare cause of recurrent angioedema caused by a default in the gene that encodes the C1 esterase inhibitor (C1-INH). The oedema involves predominantly the face, limbs and genital and gastrointestinal tract. The involvement of the larynx, although less frequent, is the most severe clinical expression of HA and is potentially fatal. Case report: Clinical report of an eight-year-old female with multiple episodes of angioedema. The...

  15. Icatibant, a new bradykinin-receptor antagonist, in hereditary angioedema.

    Science.gov (United States)

    Cicardi, Marco; Banerji, Aleena; Bracho, Francisco; Malbrán, Alejandro; Rosenkranz, Bernd; Riedl, Marc; Bork, Konrad; Lumry, William; Aberer, Werner; Bier, Henning; Bas, Murat; Greve, Jens; Hoffmann, Thomas K; Farkas, Henriette; Reshef, Avner; Ritchie, Bruce; Yang, William; Grabbe, Jürgen; Kivity, Shmuel; Kreuz, Wolfhart; Levy, Robyn J; Luger, Thomas; Obtulowicz, Krystyna; Schmid-Grendelmeier, Peter; Bull, Christian; Sitkauskiene, Brigita; Smith, William B; Toubi, Elias; Werner, Sonja; Anné, Suresh; Björkander, Janne; Bouillet, Laurence; Cillari, Enrico; Hurewitz, David; Jacobson, Kraig W; Katelaris, Constance H; Maurer, Marcus; Merk, Hans; Bernstein, Jonathan A; Feighery, Conleth; Floccard, Bernard; Gleich, Gerald; Hébert, Jacques; Kaatz, Martin; Keith, Paul; Kirkpatrick, Charles H; Langton, David; Martin, Ludovic; Pichler, Christiane; Resnick, David; Wombolt, Duane; Fernández Romero, Diego S; Zanichelli, Andrea; Arcoleo, Francesco; Knolle, Jochen; Kravec, Irina; Dong, Liying; Zimmermann, Jens; Rosen, Kimberly; Fan, Wing-Tze

    2010-08-05

    Hereditary angioedema is characterized by recurrent attacks of angioedema of the skin, larynx, and gastrointestinal tract. Bradykinin is the key mediator of symptoms. Icatibant is a selective bradykinin B2 receptor antagonist. In two double-blind, randomized, multicenter trials, we evaluated the effect of icatibant in patients with hereditary angioedema presenting with cutaneous or abdominal attacks. In the For Angioedema Subcutaneous Treatment (FAST) 1 trial, patients received either icatibant or placebo; in FAST-2, patients received either icatibant or oral tranexamic acid, at a dose of 3 g daily for 2 days. Icatibant was given once, subcutaneously, at a dose of 30 mg. The primary end point was the median time to clinically significant relief of symptoms. A total of 56 and 74 patients underwent randomization in the FAST-1 and FAST-2 trials, respectively. The primary end point was reached in 2.5 hours with icatibant versus 4.6 hours with placebo in the FAST-1 trial (P=0.14) and in 2.0 hours with icatibant versus 12.0 hours with tranexamic acid in the FAST-2 trial (P<0.001). In the FAST-1 study, 3 recipients of icatibant and 13 recipients of placebo needed treatment with rescue medication. The median time to first improvement of symptoms, as assessed by patients and by investigators, was significantly shorter with icatibant in both trials. No icatibant-related serious adverse events were reported. In patients with hereditary angioedema having acute attacks, we found a significant benefit of icatibant as compared with tranexamic acid in one trial and a nonsignificant benefit of icatibant as compared with placebo in the other trial with regard to the primary end point. The early use of rescue medication may have obscured the benefit of icatibant in the placebo trial. (Funded by Jerini; ClinicalTrials.gov numbers, NCT00097695 and NCT00500656.)

  16. Health-related quality of life in relation to disease activity in adults with hereditary angioedema in Sweden.

    Science.gov (United States)

    Nordenfelt, Patrik; Nilsson, Mats; Lindfors, Anders; Wahlgren, Carl-Fredrik; Björkander, Janne

    2017-11-30

    Health-related quality of life (HR-QoL) is impaired in patients with hereditary angioedema (HAE) but has not yet been satisfactorily described. To study HR-QoL in patients with HAE by combining different HR-QoL instruments with disease activity assessment. All adults in the Swedish HAE registry were invited to take part in this questionnaire study, which used the generic HR-QoL instruments, EuroQol 5 Dimensions 5 Level (EQ-5D-5L) and the RAND Corporation Short Form 36 (RAND-36), the disease-specific Angioedema Quality of Life instrument (AE-QoL), the recently introduced Angioedema Activity Score (AAS) form, and questionnaires on sick leave and prophylactic medication. Sixty-four of 133 adults (26 men, 38 women) between 18 and 91 years old responded. The most affected HR-QoL dimensions in the EQ-5D-5L were pain/discomfort and anxiety/depression; in the RAND-36, energy/fatigue, general health, pain; and, in the AE-QoL, fears/shame and fatigue/mood. Women had lower HR-QoL in the RAND-36 for general health and energy/fatigue (p 0 had significantly impaired HR-QoL. There were significant associations (p depression, and fatigue/mood are important aspects of HAE but the AE-QoL disregards pain. HR-QoL was not significantly affected by prophylaxis. Increased disease activity was associated with impaired HR-QoL, which justifies more active disease management.

  17. [Management of bradykinin-mediated angioedema].

    Science.gov (United States)

    Floccard, B; Crozon, J; Rimmelé, T; Vulliez, A; Coppere, B; Chamouard, V; Boccon-Gibod, I; Bouillet, L; Allaouchiche, B

    2011-01-01

    Present the clinical signs of bradykinin-mediated angioedema, a disease little known to intensive care anaesthesiologists, and develop their scientific basis with recent data on management in emergency and perioperative care. International recommendations and recent general reviews. Data collection was performed using the Medline database with the keyword: angioedema. Research studies published during the last 10 years were reviewed. Relevant clinical information was extracted and discussed. Angioedema is a clinical syndrome characterized by episodes of transitory recurrent submucosal and subcutaneous oedema, called attacks. During an attack, the oedema may be localized at the level of the skin and/or ENT and digestive tract mucosa. This syndrome is not due to an allergic reaction. It is related to a C1 complement inhibitor deficiency or an increase in factor XII resulting in the excessive release of bradykinin, which leads to capillary permeability. There are hereditary and acquired forms, notably associated with the use of ACE inhibitors and sartans. This rare disease should be recognized by anaesthesiologists and intensive care and emergency physicians because, in the absence of specific treatment, it can be life-threatening due to the appearance of laryngeal oedema. In addition, there is a risk that the patient may have an attack during the perioperatory period, due to surgical trauma. International recommendations exist, and there are new molecules available in France. For moderate attacks, treatment is based on tranexamic acid. For hereditary forms, according to the localization and gravity of the attacks, emergency treatment is based on the use of Icatibant, a bradykinin B2 receptor inhibitor, and C1 inhibitor concentrate. For pregnant women and acquired forms, C1 inhibitor concentrate is the treatment of reference. Antalgic and perfusion treatments should not be neglected, and should be modified as a function of clinical signs. High-risk situations

  18. The Janus faces of acquired angioedema: C1-inhibitor deficiency, lymphoproliferation and autoimmunity.

    Science.gov (United States)

    Wu, Maddalena Alessandra; Castelli, Roberto

    2016-02-01

    Several clinical and biological features of lymphoproliferative diseases have been associated with an increased risk of developing autoimmune manifestations. Acquired deficiency of C1-inhibitor (C1-INH) (AAE) is a rare syndrome clinically similar to hereditary angioedema (HAE) characterized by local increase in vascular permeability (angioedema) of the skin and the gastrointestinal and oro-pharyngo-laryngeal mucosa. Bradykinin, a potent vasoactive peptide, released from high molecular weight kininogen when it is cleaved by plasma kallikrein (a serine protease controlled by C1-INH), is the mediator of symptoms. In total 46% of AAE patients carry an underlying hematological disorder including monoclonal gammopathy of uncertain significance (MGUS) or B cell malignancies. However, 74% of AAE patients have anti-C1-INH autoantibodies without hematological, clinical or instrumental evidence of lymphoproliferative disease. Unlike HAE patients, AAE patients usually have late-onset symptoms, do not have a family history of angioedema and present variable response to treatment due to the hypercatabolism of C1-INH. Experiments show that C1-INH and/or the classical complement pathway were consumed by the neoplastic lymphatic tissues and/or anti-C1-INH neutralizing autoantibodies. Therapy of AAE follows two directions: 1) prevention/reversal of the symptoms of angioedema; and 2) treatment of the associated disease. Different forms of B cell disorders coexist and/or evolve into each other in AAE and seem to be dominated by an altered control of B cell proliferation, thus AAE represents an example of the strict link between autoimmunity and lymphoproliferation.

  19. The Clinical Evaluation of Angioedema.

    Science.gov (United States)

    Gill, Parwinder; Betschel, Stephen D

    2017-08-01

    The clinical evaluation of angioedema is reliant on obtaining a thorough patient and family history with an assessment of risk factors and presenting symptoms unique to each subtype. It is important to distinguish between angioedema with and without urticaria as a primary step in the evaluation; thereafter, laboratory parameters and investigations allow for subsequent stratification. There is a significant disease burden associated with angioedema and thus it is essential for health care practitioners to establish a prompt and accurate diagnosis, and a comprehensive care plan that addresses the patient's physical and mental well-being alike. Crown Copyright © 2017. Published by Elsevier Inc. All rights reserved.

  20. ACE inhibitor-induced angioedema.

    Science.gov (United States)

    Baram, Michael; Kommuri, Anand; Sellers, Subhashini A; Cohn, John R

    2013-01-01

    Angiotensin-converting enzyme inhibitors (ACEI) are commonly prescribed for blood pressure control and renal protection. ACEI angioedema is a common problem in patients who are taking ACEI, although, in most cases, the disorder is self-limited, and spontaneous episodes of apparently unprovoked angioedema stop with the discontinuation of the medication. In a subset of patients, hospitalization and even intubation are required for airway protection. The diagnosis is made clinically. There are no laboratory studies that establish the diagnosis. However, such investigations help exclude alternative diagnoses as the cause for the patient's presentation. Conventional treatment with regimens used to control allergic angioedema is ineffective in this condition. The mechanism of ACEI-induced angioedema is thought to be related to its effect on the kallikrein-kinin system. Kallikrein is a protease that converts high-molecular-weight kininogens into kinins, primarily bradykinin. Medications recently developed, primarily icatibant and ecallantide, to control hereditary angioedema, a disorder also associated with kallikrein-kinin activation, have been used to treat ACEI angioedema with some success. The efficacy of these agents and their optimal use remains to be established by randomized and placebo controlled trials. Copyright © 2013 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  1. Icatibant in angiotensin-converting enzyme (ACE) inhibitor-associated angioedema.

    Science.gov (United States)

    Fok, J S; Katelaris, C H; Brown, A F; Smith, W B

    2015-08-01

    Angioedema occurs in up to 2% of those taking angiotensin-converting enzyme (ACE) inhibitors. Upper airway angioedema may potentially require endotracheal intubation or cricothyrotomy, and is usually unresponsive to adrenaline. The bradykinin receptor antagonist icatibant is proven to be effective in the treatment of acute attacks of hereditary angioedema, and has also been reported effective in the treatment of angioedema associated with ACE inhibitors. To describe the use of icatibant for ACE inhibitor-associated airway angioedema. We treated 13 consecutive emergency department (ED) patients, who had not improved with adrenaline and/or corticosteroids, with icatibant 30 mg subcutaneously for ACE inhibitor-associated upper respiratory tract angioedema according to an agreed protocol. Four patients were intubated in the ED either before or after receiving icatibant; three of these were extubated within 24 h of treatment. Eight patients received early icatibant and did not require intubation. The time from onset of airway angioedema to ED presentation ranged from 1 h to 3 days (median 4 h); from ED presentation to receiving icatibant, from 30 minutes to 3 days (median 3 h); and to onset of symptom improvement after icatibant, 15 minutes to 7 h (median 2 h). One patient received a second dose of icatibant. All patients improved after receiving icatibant, consistent with its bradykinin receptor blocking mechanism. Icatibant rapidly reversed symptoms, and appeared to avert the need for intubation or expedite extubation. Timely use of icatibant in ACE inhibitor-associated angioedema may avert the need for invasive airway procedures and intensive care unit admission. © 2015 Royal Australasian College of Physicians.

  2. Health-related quality of life in Danish children with hereditary angioedema

    DEFF Research Database (Denmark)

    Aabom, Anne; Nguyen, Dan; Fisker, Niels

    2017-01-01

    -INH-HAE, including possible correlations to disease severity and attack frequency. Methods: All Danish children ages 2-18 years with C1-INH-HAE were invited to complete questionnaires regarding HRQoL; 14 (93%) agreed. Child self-report forms were used for children ages ≥5 years. The instruments used...... scores for healthy children and better than the scores in the only other study dedicated to HRQoL in children. Children with recent attacks had lower scores, whereas HRQoL scores were not correlated to overall disease severity or age. Surprisingly, home therapy was associated with lower HRQoL; however......, home therapy was also correlated to a higher overall severity score and more frequent attacks. There was a strong child-parent agreement in the PedsQL forms, but scores were independent of whether the child had a family history of C1-INH-HAE or sporadic C1-INH-HAE and whether the parent completing...

  3. Angioedema related to Angiotensin inhibitors.

    Science.gov (United States)

    Knecht, Stephanie E; Dunn, Steven P; Macaulay, Tracy E

    2014-10-01

    Angiotensin inhibitors have been extensively evaluated in clinical trials and have demonstrated significant reductions in morbidity and mortality following myocardial infarction and stroke, as well as in patients with heart failure or who are at risk of cardiovascular disease. Further, both angiotensin-converting enzyme inhibitors (ACEIs) and angiotensin receptor blockers (ARBs) are frequently prescribed for the treatment of hypertension and to preserve renal function in patients with diabetes mellitus and chronic kidney disease. Angioedema is a known, but rare, adverse effect of ACEIs and ARBs. Therefore, it is important for clinicians to have a thorough understanding of risks and benefits of prescribing these medications, particularly in patients with a history of angioedema. This review describes the literature evaluating the incidence and cross-reactivity of angioedema with ACEIs and ARBs in order to provide guidance for clinical decision making. © The Author(s) 2014.

  4. Population pharmacokinetics of recombinant human C1 inhibitor in patients with hereditary angioedema.

    Science.gov (United States)

    Farrell, Colm; Hayes, Siobhan; Relan, Anurag; van Amersfoort, Edwin S; Pijpstra, Rienk; Hack, C Erik

    2013-12-01

    To characterize the pharmacokinetics (PK) of recombinant human C1 inhibitor (rhC1INH) in healthy volunteers and hereditary angioedema (HAE) patients. Plasma levels of C1INH following 294 administrations of rhC1INH in 133 subjects were fitted using nonlinear mixed-effects modelling. The model was used to simulate maximal C1INH levels for the proposed dosing scheme. A one-compartment model with Michaelis-Menten elimination kinetics described the data. Baseline C1INH levels were 0.901 [95% confidence interval (CI): 0.839-0.968] and 0.176 U ml(-1) (95% CI: 0.154-0.200) in healthy volunteers and HAE patients, respectively. The volume of distribution of rhC1INH was 2.86 l (95% CI: 2.68-3.03). The maximal rate of elimination and the concentration corresponding to half this maximal rate were 1.63 U ml(-1) h(-1) (95% CI: 1.41-1.88) and 1.60 U ml(-1) (95% CI: 1.14-2.24), respectively, for healthy volunteers and symptomatic HAE patients. The maximal elimination rate was 36% lower in asymptomatic HAE patients. Peak C1INH levels did not change upon repeated administration of rhC1INH. Bodyweight was found to be an important predictor of the volume of distribution. Simulations of the proposed dosing scheme predicted peak C1INH concentrations above the lower level of the normal range (0.7 U ml(-1)) for at least 94% of all patients. The population PK model for C1INH supports a dosing scheme on a 50 U kg(-1) basis up to 84 kg, with a fixed dose of 4200 U above 84 kg. The PK of rhC1INH following repeat administration are consistent with the PK following the first administration. © 2013 The British Pharmacological Society.

  5. Genetic Determinants of C1 Inhibitor Deficiency Angioedema Age of Onset.

    Science.gov (United States)

    Gianni, Panagiota; Loules, Gedeon; Zamanakou, Maria; Kompoti, Maria; Csuka, Dorottya; Psarros, Fotis; Magerl, Markus; Moldovan, Dimitru; Maurer, Marcus; Speletas, Matthaios G; Farkas, Henriette; Germenis, Anastasios E

    2017-01-01

    In view of the large heterogeneity in the clinical presentation of hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE), great efforts are being made towards detecting measurable biological determinants of disease severity that can help to improve the management of the disease. Considering the central role that plasma kallikrein plays in bradykinin production, we investigated the contribution of the functional polymorphism KLKB1-428G/A to the disease phenotype. We studied 249 C1-INH-HAE patients from 114 European families, and we explored possible associations of C1-INH-HAE clinical features with carriage of KLKB1-428G/A, combined or not with that of the functional F12-46C/T polymorphism. Carriers of the G allele of the KLKB1-428G/A polymorphism exhibited a significantly delayed disease onset (i.e., by 4.1 years [p < 0.001], depending on the zygocity status), while carriers of both the KLKB1-428G/A and the F12-46C/T polymorphism displayed an 8.8-year delay in disease onset (p < 0.001) and a 64% lower probability of needing long-term prophylactic treatment (p = 0.019). These findings support our initial hypothesis that functional alterations in genes of proteins involved in bradykinin metabolism and function affect the clinical phenotype and possibly contribute to the pathogenesis of C1-INH-HAE. Given that an earlier onset of symptoms is inversely correlated with the subsequent course of the disease and, eventually, the need for long-term prophylaxis, these polymorphisms may be helpful prognostic biomarkers of disease severity. © 2017 S. Karger AG, Basel.

  6. Recent Advances in Drug-Induced Angioedema

    Directory of Open Access Journals (Sweden)

    Naoko Inomata

    2012-01-01

    Full Text Available Angioedema is the end result of deep dermal, subcutaneous and/or mucosal swelling, and is potentially a life- threatening condition in cases where the pharynx or larynx is involved. Drug-induced angioedema has been reported to occur in response to a wide range of drugs and vaccines. Drug-induced angioedema, like other cutaneous drug reactions, has been reported to be most frequently elicited by beta-lactam antibiotics and nonsteroidal anti-inflammatory drugs, although reliable data from epidemiologic studies are scarce. Recent reports suggested an increasing role of angiotensin-converting enzyme inhibitors (ACEIs in the causation of life- threatening angioedema. ACEI-related angioedema is never accompanied by urticaria and occurs via a kinin- dependent mechanism. ACEI-related angioedema not only can start years after beginning the treatment, but it can then recur irregularly while under that treatment. Furthermore, allergy tests are unreliable for the diagnosis of ACEI-related angioedema, and so the relationship between angioedema and ACEIs is often missed and consequently quite underestimated. Accordingly, better understanding of the kinin-dependent mechanism, which is particular to angioedema, is necessary for the appropriate management of drug-induced angioedema.

  7. Angioedema Due to Lamiaceae Allergy.

    Science.gov (United States)

    Yazıcı, Selçuk; Nacaroglu, Hikmet Tekin; Bahçeci Erdem, Semiha; Karaman, Sait; Can, Demet

    2018-02-01

    We present a 13-year-old male childallergic to three different plants (Salvia officinalis, Mentha piperita and Origanum onites L.) of Lamiaceae family. The patient developed angioedema 20-30 minutes after eating chicken meat with cheddar cheese. There was no history of allergy. Oral food challenge (OFC) with both cheddar cheese and chicken meat was negative. Skin tests for inhalant allergens were negative. 3 weeks later, the patient was admitted with angioedema after drinking sage tea. OFC with sage was applied and angioedema was observed. It was recognized that the first trigger, chicken meat with cheddar cheese, included oregano (Origanum onites L.). OFC for oregano was positive. Prick to prick test for Lamiaceae herbs (oregano, sage, mint) was performed. A positive reaction was observed only to mint. OFC was repeated with fresh mint and angioedema developed after 16 hours. Diagnose of Lamiaceae allergy is complicated and cross-sensitivity is common. Skin prick test (prick to prick)revealed a positive response only to mint but not to oregano and sage. Commercial radioallergosorbent (RAST) tests are available only for a few members of the family. Finally, thediagnose is based mainly on OFC. Spices from Lamiaceae group should be considered as potential triggers of allergic reactions.

  8. Chapter 21: Urticaria and angioedema.

    Science.gov (United States)

    Carr, Tara F; Saltoun, Carol A

    2012-01-01

    Urticaria, also known as hives, may affect up to 20% of the population at some time in their lives. Urticaria is characterized by extreme pruritus and described as erythematous, raised, circumscribed lesions with central pallor that blanch with pressure. The pathogenesis of urticaria involves mast cell activation, with subsequent release of histamine and other vasoactive mediators, leading to increased vascular permeability of postcapillary venules and development of edema, erythema, and pruritus. Urticaria is closely associated with angioedema in 40% of individuals; ∼10% of patients experience angioedema without urticaria. Urticarial lesions often are generalized with multiple lesions in no specific distribution; angioedema tends to be localized, commonly affecting the face (periorbital and perioral regions), tongue, uvula, soft palate or larynx, extremities, and genitalia. Urticaria is subdivided into acute and chronic urticaria based on duration of symptoms. Acute urticaria lasts products, medications (aspirin, nonsteroidal anti-inflammatory drugs, and antibiotics), or insect stings. Urticaria lasting >6 weeks is designated as chronic urticaria, and an etiology is seldom identified and thus considered idiopathic. Chronic urticaria may have an autoimmune basis. There is a well-documented association between autoimmune hypothyroidism (Hashimoto's disease) and urticaria and angioedema with higher incidence of antithyroid (antithyroglobulin and antiperoxidase) antibodies in these usually euthyroid patients. Furthermore, studies have revealed a circulating IgG antibody directed against the IgE receptor (F(Cε)RIα) or IgE in 40-60% of patients with chronic urticaria. Histamine 1-receptor antagonists (antihistamines) are initial therapy.

  9. Recurrent angioedema associated with hypogonadism or anti-androgen therapy.

    Science.gov (United States)

    Pichler, W J; Lehner, R; Späth, P J

    1989-10-01

    Two male patients with hypogonadism and four female patients who received an anti-androgen as contraceptive (cyproteronacetate) and who had recurrent angioedema are described. In one male patient, augmentation of the plasma androgen level resulted in disappearance of symptoms. In the four female patients, recurrent angioedema and urticaria developed after initiation of the anti-androgen treatment. Cessation of cyproteronacetate and a change to another contraceptive resulted in complete resolution of the previously frequent angioedematous attacks. The women are still symptom free after more than 60 patient's months. These cases suggest that an androgen deficit due to either hypogonadism or to anti-androgen treatment may be another cause of angioedema. One of the two male patients was untreated and presented with 40% normal value of C1-INH. Androgen therapy normalized C1-INH concentration in this male patient. Functional C1-INH in the same patient, studied before and after the beginning of androgen therapy, clearly increased when assessed by inhibition of amidolytic activity of C1-esterase. The other male patient with hypogonadism had already been under androgen treatment for 4 years and had C1-INH levels in the normal range. In the female patients, complement profiles were normal before and after cessation of anti-androgen contraception; however, the C1-INH plasma levels were higher after cessation of anti-androgen anticonception. These results indicate an effect of androgen deficit on the level of C1-INH in circulating plasma but do not prove a role of C1-INH in angioedema associated with diminished androgen plasma levels.

  10. Angioedema without urticaria in childhood.

    Science.gov (United States)

    Ertoy Karagol, Hacer I; Yilmaz, Ozlem; Bakirtas, Arzu; Topal, Erdem; Demirsoy, Mehmet S; Turktas, Ipek

    2013-11-01

    There has been no separate study investigating angioedema without urticaria (Aw/oU) exclusively in children so far. The purpose of this study was to investigate the frequency, clinical presentation, etiology, management and follow-up of Aw/oU in children. This is a prospective study that included all consecutive patients with a history of Aw/oU referred to our clinic between January 2011 and May 2012. A standard diagnostic and therapeutic algorithm was applied to all patients. The frequency of Aw/oU was found to be 1.6% during the study period. An etiological factor could be found in only 45 patients (49%). The causes of Aw/oU were infection (21%), allergy (14%), thyroid autoimmunity (TA)-related (8%) and nonsteroid anti-inflammatory drug hypersensitivity (6%), and idiopathic angioedema (51%). There was no hereditary type I, II or acquired type of angioedema or rare syndromes associated with Aw/oU. The median follow-up was 16 months (range: 12-30 months). Antihistamine prophylaxis was initiated at therapeutic doses in 20 patients with frequently recurrent angioedema due to idiopathic and euthyroid TA-related Aw/oU for 3 months. These patients responded to antihistamine prophylaxis for 3 months. Four patients relapsed after cessation of prophylaxis at the end of 3 months. Antihistamine prophylaxis was prolonged to 6 months in three patients and to 9 months in one patient. Angioedema without urticaria in children is a rare condition and no etiology can be identified in half of them. Antihistamine treatment alone is sufficient, and prognosis is good in recurrent non hereditary cases in a short-term follow-up period. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  11. Angioedema Phenotypes: Disease Expression and Classification.

    Science.gov (United States)

    Wu, Maddalena Alessandra; Perego, Francesca; Zanichelli, Andrea; Cicardi, Marco

    2016-10-01

    Due to marked heterogeneity of clinical presentations, comprehensive knowledge of angioedema phenotypes is crucial for correct diagnosis and choosing the appropriate therapeutic approach. One of the ways to a meaningful clinical distinction can be made between forms of angioedema occurring "with or without wheals." Angioedema with wheals (rash) is a hallmark of urticaria, either acute or chronic, spontaneous or inducible. Angioedema without wheals may still be manifested in about 10 % of patients with urticaria, but it may also occur as a separate entity. Several classifications of angioedema as part of urticaria were published over time, while a latest one, released in 2014 (HAWK group consensus, see below), provided a classification of all forms of "angioedema without wheals" distinct from urticaria, which will be the focus of the present review. At this time, the HAWK consensus classification is the best in terms of covering the pathophysiology, mediators involved, angioedema triggers, and clinical expression. According to this classification, three types of hereditary angioedema (genetic C1-INH deficiency, normal C1-INH with factor XII mutations, and unknown origin) and four types of acquired angioedema (C1-INH deficiency, related to ACE inhibitors intake, idiopathic histaminergic, and idiopathic non-histaminergic) are presented. We will review the distinctive clinical features of each phenotype in details.

  12. Novelties in the Diagnosis and Treatment of Angioedema.

    Science.gov (United States)

    Cicardi, M; Suffritti, C; Perego, F; Caccia, S

    2016-01-01

    Angioedema is defined as local, noninflammatory, self-limiting edema that is circumscribed owing to increased leakage of plasma from the capillaries located in the deep layers of the skin and the mucosae. Two mediators, histamine and bradykinin, account for most cases of angioedema. Angioedema can occur with wheals as a manifestation of urticaria, and this form is frequently allergic. In the present review, we discuss nonallergic angioedema without wheals, which can be divided into 3 acquired and 4 hereditary forms. Histamine is the mediator in acquired angioedema of unknown etiology (idiopathic histaminergic acquired angioedema), whereas in other forms the main mediator is bradykinin. Angioedema can be caused by C1-inhibitor deficiency (C1-INH-hereditary angioedema and C1-INH-acquired angioedema), mutations in coagulation factor XII (FXII-hereditary angioedema), and treatment with angiotensin-converting enzyme inhibitors (ACEI-acquired angioedema). Etiology remains unclear in acquired angioedema (idiopathic nonhistaminergic acquired angioedema) and in 1 type of hereditary angioedema (hereditary angioedema of unknown origin). Several treatments are licensed for hereditary C1-INH deficiency. Plasma-derived and recombinant C1-INHs, the bradykinin receptor blocker icatibant, and the plasma kallikrein inhibitor ecallantide have been approved for on-demand treatment to reverse angioedema symptoms. Attenuated androgen and plasma-derived C1-INH are approved for prophylaxis.

  13. Chronic idiopathic angioedema: a single center experience.

    Science.gov (United States)

    Eli, Magen; Joseph, Mishal; Kuznik, Boris; Menachem, Schlesinger

    2014-10-01

    Chronic idiopathic angioedema (CIA) is defined as three or more episodes of angioedema in a period of > 6 months without a clear etiology. In the study, we tried to explore clinical and laboratory characteristics of patients with CIA unaccompanied by urticaria. We retrospectively reviewed clinical and laboratory characteristics of 1238 patients with chronic urticaria and/or angioedema referred to our allergy clinic. Eight hundred and forty-one (67.9%) subjects had chronic urticaria without angioedema (CU Group), 323 (26.1%) had both urticaria and angioedema (CU + CA group), and 74 (5.9%) had chronic angioedema without urticaria (CA). In 29 (39.2%) cases of CA, no etiologic factor of angioedema was discovered, thus the patients were defined as having chronic idiopathic angioedema (CIA Group). Twenty-two (75.8%) subjects had antihistamine-responsive CIA and seven (24.1%) had antihistamine-unresponsive CIA. There were no statistically significant differences in clinical (except of urticarial eruptions) and laboratory characteristics between CU, CA + CU, and CIA groups. Antihistamine responsive and antihistamine-unresponsive CIA groups had no distinguishable clinical or laboratory features. We suppose that CIA, at least its antihistamine-responsive form, represents a rare form of chronic spontaneous urticaria. The reasons why in CIA there are no other clinical signs of mast cell/basophil activation, such as pruritus, urticarial, and dermatographism, are largely unknown and have to be elucidated in future studies. © 2014 The International Society of Dermatology.

  14. Current treatment options for idiopathic angioedema.

    Science.gov (United States)

    Shroba, Jodi; Hanson, Jill; Portnoy, Jay

    2015-11-01

    Idiopathic angioedema is defined as localized swelling of the cutaneous and mucosal tissue that occurs in episodes without a clear etiology. It can be problematic to treat when the underlying pathophysiology is not well understood. To identify successful treatments of idiopathic angioedema reported in the literature. A literature search was performed using PubMed. Published case reports and articles discussing treatment of idiopathic angioedema were used in the formulation of this review. In addition, 2 case reports are provided. Although there are no approved treatments for idiopathic angioedema, several medications used for the treatment of hereditary angioedema, such as bradykinin receptor antagonists (icatibant), kallikrein inhibitors (ecallantide), and C1 inhibitors, were successful in 10 patients. Anti-IgE monoclonal antibody (omalizumab) proved successful in 5 patients. The most widely used and successful medication was tranexamic acid (154 patients). Despite an unknown etiology, this article highlights viable treatment options for idiopathic angioedema. More clinical trials and better markers identifying the cause of angioedema are needed. Copyright © 2015 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  15. [Comparison of Two Symptom-Triggered Treatments for Alcohol Withdrawal: HAES vs. SAB-P].

    Science.gov (United States)

    Holzbach, R; Ihlow, C; Takla, T; Kemper, U; Naber, D

    2016-02-01

    For alcohol withdrawal during hospitalization, often a medication as means for withdrawal needs to be chosen. Modern, score-controlled processes that can be used by the nursing staff after instruction by physicians are frequently not used and even unknown in hospitals. One reason for this is that some of the scores require checking several criteria and are therefore more time-consuming and complicated than use of a fixed-dosage strategy. The SAB-P and HAES are short with only 6 items that can be checked by the nursing staff. Safety of the Hamburg Alcohol Withdrawal Scale (Hamburger Alkoholentzugs-Skala (HAES)) was analyzed retrospectively and prospectively with regard to score-controlled alcohol-withdrawal treatment after rating by the nurse staff (Scoregesteuerte Alkoholentzugsbehandlung nach Rating durch das Pflegepersonal (SAB-P)). Incidence of complications in patients treated with SAB-P and HAES was nearly similar with 1% start of delirium and 3% seizures (SAB-P) and 0.5 to 1.5% start of delirium and 0 to 0.5% seizures in the HAES group. With both scales it was possible to start medical treatment while still under falling alcohol levels (0.93 and 0.91%, respectively). Medication dosage was initially higher using the HAES, so that the time needed to monitor withdrawal symptoms could be reduced (3.8 vs. 3.1 days). Using a score-controlled strategy for alcohol withdrawal leads to a lower complication rate than found in literature. The structured procedure was helpful for the nursing staff as well as for the physicians. SAB-P as well as HAES made withdrawal for the patients more comfortable and led to fewer complaints. Because of rapid reaction and faster symptom reduction of HAES, there was less time necessary for monitoring. Simple handling, clomethiazol, oxazepam or diazepam as applicable medication and clear documentation are the advantages of HAES. © Georg Thieme Verlag KG Stuttgart · New York.

  16. Nuevas obras de Carlos de Haes y de Aureliano de Beruete

    Directory of Open Access Journals (Sweden)

    Arias Anglés, Enrique

    2005-09-01

    Full Text Available El objeto de estas líneas es el de dar a conocer y catalogar cuatro nuevas obras de Carlos de Haes (Bruselas, 1826-Madrid, 1898 y otras dos de Aureliano de Beruete (Madrid, 1845- Madrid, 1912. Tres de las de Haes pertenecen a colecciones particulares madrileñas y la otra ha aparecido en el comercio alicantino; mientras que las dos de Aureliano de Beruete son de colección particular madrileña. Comenzaremos este análisis por las de Haes, tanto por razones cronológicas como por haber sido este pintor maestro de Beruete. La primera de ellas pertenece a la colección Rudolf Gerstenmaier (Madrid, y es un óleo sobre lienzo, de 0,35 x 0,65 m., firmado en rojo en el ángulo inferior izquierdo "C. de Haes", y que por representar un bello paisaje de los llamados de alta montaña, que no podemos identificar, lo titularíamos Vista de cordillera (Fig. 1...

  17. Idiopathic histaminergic angioedema without wheals: a case series of 31 patients

    Science.gov (United States)

    Boccon‐Gibod, I.; Mansard, C.; Dumestre Perard, C.; Pralong, P.; Chatain, C.; Deroux, A.; Bouillet, L.

    2016-01-01

    Summary Idiopathic histaminergic acquired angioedema (IH‐AAE) is a common cause of recurrent angioedema without wheals. It is a mast cell‐mediated disease thought to belong to the same clinical entity as chronic urticaria (CU). The objective of this study was to describe the clinical and epidemiological characteristics of IH‐AAE patients. From 2014 to 2015, 534 patients were seen at our national reference centre for angioedema and/or urticaria. Among them, we identified 31 patients with idiopathic histaminergic acquired angioedema without wheals (IH‐AAE). Thirty‐one patients (15 men and 16 women) with a mean age of 50 years met the criteria for IH‐AAE. The average delay in diagnosis was 6·3 years. A history of allergy was found in 12 patients (38·7%), nine suffering from allergic rhinitis. The mean duration of attacks was 28·1 h. The AE attack was located in the upper respiratory tract in 54·8% of cases (17 patients). A lingual location was found in 29% of patients. Men were more likely than women to have an upper airway involvement. No intubations or admissions to intensive care units were reported. The dosage of anti‐histamines to control the symptoms was onefold the recommended dose in 51·6% of patients (16 patients), twofold in 32% (10 patients) and three–fourfold in 16·1% (five patients). IH‐AAE is characterized by an important delay in diagnosis, a frequent involvement of the upper airway and a benign course during attacks. As in CU, a trial of up to fourfold dose of H1‐anti‐histamines may be necessary to control symptoms. PMID:26969870

  18. Idiopathic histaminergic angioedema without wheals: a case series of 31 patients.

    Science.gov (United States)

    Faisant, C; Boccon-Gibod, I; Mansard, C; Dumestre Perard, C; Pralong, P; Chatain, C; Deroux, A; Bouillet, L

    2016-07-01

    Idiopathic histaminergic acquired angioedema (IH-AAE) is a common cause of recurrent angioedema without wheals. It is a mast cell-mediated disease thought to belong to the same clinical entity as chronic urticaria (CU). The objective of this study was to describe the clinical and epidemiological characteristics of IH-AAE patients. From 2014 to 2015, 534 patients were seen at our national reference centre for angioedema and/or urticaria. Among them, we identified 31 patients with idiopathic histaminergic acquired angioedema without wheals (IH-AAE). Thirty-one patients (15 men and 16 women) with a mean age of 50 years met the criteria for IH-AAE. The average delay in diagnosis was 6·3 years. A history of allergy was found in 12 patients (38·7%), nine suffering from allergic rhinitis. The mean duration of attacks was 28·1 h. The AE attack was located in the upper respiratory tract in 54·8% of cases (17 patients). A lingual location was found in 29% of patients. Men were more likely than women to have an upper airway involvement. No intubations or admissions to intensive care units were reported. The dosage of anti-histamines to control the symptoms was onefold the recommended dose in 51·6% of patients (16 patients), twofold in 32% (10 patients) and three-fourfold in 16·1% (five patients). IH-AAE is characterized by an important delay in diagnosis, a frequent involvement of the upper airway and a benign course during attacks. As in CU, a trial of up to fourfold dose of H1-anti-histamines may be necessary to control symptoms. © 2016 British Society for Immunology.

  19. Angioedema hereditario en pediatría Hereditary pediatric angioedema

    OpenAIRE

    A. Calvo Gómez-Rodulfo; J.E. García López; J.D. Herrero-Morín; G. Rodríguez García; F. González Guerra

    2009-01-01

    Introducción: El angioedema hereditario es una patología de origen genético causada por la alteración del gen que codifica la proteína inhibidora de la C1 esterasa activada (C1-INH). La prevalencia de esta entidad es baja, lo que dificulta su diagnóstico y manejo adecuado.
    Caso clínico: Se presenta el caso de una paciente con episodios repetidos de edema subcutáneo localizado en las extremidades desde los tres años de vida, añadiendo disfagia y disfonía a partir de la pu...

  20. Angioedema Related to Angiotensin-Converting Enzyme Inhibitors

    Science.gov (United States)

    Javaud, Nicolas; Achamlal, Jallal; Reuter, Paul-George; Lapostolle, Frédéric; Lekouara, Akim; Youssef, Mustapha; Hamza, Lilia; Karami, Ahmed; Adnet, Frédéric; Fain, Olivier

    2015-01-01

    Abstract The number of cases of acquired angioedema related to angiotensin converting enzyme inhibitors induced (ACEI-AAE) is on the increase, with a potential concomitant increase in life-threatening attacks of laryngeal edema. Our objective was to determine the main characteristics of ACEI-AAE attacks and, in doing so, the factors associated with likelihood of hospital admission from the emergency department (ED) after a visit for an attack. A prospective, multicenter, observational study (April 2012–December 2014) was conducted in EDs of 4 French hospitals in collaboration with emergency services (SAMU 93) and a reference center for bradykinin-mediated angioedema. For each patient presenting with an attack, emergency physicians collected demographic and clinical presentation data, treatments, and clinical course. They recorded time intervals from symptom onset to ED arrival and to treatment decision, from ED arrival to specific treatment with plasma-derived C1-inhibitor (C1-INH) or icatibant, and from specific treatment to onset of symptom relief. Attacks requiring hospital admission were compared with those not requiring admission. Sixty-two eligible patients with ACEI-AAE (56% men, median age 63 years) were included. Symptom relief occurred significantly earlier in patients receiving specific treatment than in untreated patients (0.5 [0.5–1.0] versus 3.9 [2.5–7.0] hours; P < 0.0001). Even though icatibant was injected more promptly than plasma-derived C1-INH, there, however, was no significant difference in median time to onset of symptom relief between the 2 drugs (0.5 [0.5–1.3] versus 0.5 [0.4–1.0] hours for C1-INH and icatibant, respectively, P = 0.49). Of the 62 patients, 27 (44%) were admitted to hospital from the ED. In multivariate analysis, laryngeal involvement and progressive swelling at ED arrival were independently associated with admission (Odds ratio [95% confidence interval] = 6.2 [1.3–28.2] and 5.9 [1.3–26

  1. Life Threatening Idiopathic Recurrent Angioedema Responding to Cannabis

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    Amit Frenkel

    2015-01-01

    Full Text Available We present a case of a 27-year-old man with recurrent episodes of angioedema since he was 19, who responded well to treatment with medical grade cannabis. Initially, he responded to steroids and antihistamines, but several attempts to withdraw treatment resulted in recurrence. In the last few months before prescribing cannabis, the frequency and severity of the attacks worsened and included several presyncope events, associated with scrotal and neck swelling. No predisposing factors were identified, and extensive workup was negative. The patient reported that he was periodically using cannabis socially and that during these periods he was free of attacks. Recent data suggest that cannabis derivatives are involved in the control of mast cell activation. Consequently, we decided to try a course of inhaled cannabis as modulators of immune cell functions. The use of inhaled cannabis resulted in a complete response, and he has been free of symptoms for 2 years. An attempt to withhold the inhaled cannabis led to a recurrent attack within a week, and resuming cannabis maintained the remission, suggesting a cause and effect relationship.

  2. Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency

    NARCIS (Netherlands)

    Levi, Marcel; Choi, Goda; Picavet, Charles; Hack, C. Erik

    2006-01-01

    BACKGROUND: Administration of C1-inhibitor concentrate is effective for prophylaxis and treatment of severe angioedema attacks caused by C1-inhibitor deficiency. The concentrate should be administered intravenously and hence needs to be administered by health care professionals, which might cause

  3. A 31-year-old pregnant woman with angioedema.

    Science.gov (United States)

    Speck, Aimee L; Killen, Paul D; Greenhawt, Matthew J

    2015-01-01

    Angioedema is swelling of the deep layers of the dermis and subcutaneous tissue due to an increase in vascular permeability. Angioedema sometimes occurs concomitantly with urticaria and represents an allergic disease. In other cases, angioedema is not associated with an allergic condition. We present the case of a 31-year-old woman with new-onset angioedema in the setting of her first pregnancy. After detailed history, physical examination, and laboratory evaluation, a cause for her angioedema was found that had not been considered previously and had significant implications for future management, particularly in light of her current pregnancy. Because allergists are commonly called on to evaluate and treat angioedema, we should be aware of the many disease processes that can present with this symptom and be well-versed in the workup of new-onset angioedema.

  4. Classification, diagnosis, and approach to treatment for angioedema

    DEFF Research Database (Denmark)

    Cicardi, M; Aberer, W; Banerji, A

    2014-01-01

    Angioedema is defined as localized and self-limiting edema of the subcutaneous and submucosal tissue, due to a temporary increase in vascular permeability caused by the release of vasoactive mediator(s). When angioedema recurs without significant wheals, the patient should be diagnosed to have...... angioedema as a distinct disease. In the absence of accepted classification, different types of angioedema are not uniquely identified. For this reason, the European Academy of Allergy and Clinical Immunology gave its patronage to a consensus conference aimed at classifying angioedema. Four types of acquired...... and three types of hereditary angioedema were identified as separate forms from the analysis of the literature and were presented in detail at the meeting. Here, we summarize the analysis of the data and the resulting classification of angioedema....

  5. Prognostic factors in outcome of angioedema in the emergency department.

    Science.gov (United States)

    Felder, Sarah; Curtis, R Mason; Ball, Ian; Borici-Mazi, Rozita

    2014-01-01

    Angioedema is a transient, localized swelling caused by two distinct mechanisms, mediated by histamine and bradykinin, respectively, although a proportion of cases remain idiopathic. Studies that characterize undifferentiated angioedema presenting in emergency departments (EDs) are limited. This study investigates the presentation patterns of undifferentiated angioedema in the ED based on the presumed mechanism of swelling. Medical records from all ED visits to two tertiary care hospitals from July 2007 to March 2012 were electronically reviewed. Records with documented visible swelling on general inspection and/or fiberoptic laryngoscopy and a diagnostic code for anaphylactic shock, angioneurotic edema, allergy unspecified, defects in the complement system, or unspecified drug adverse effects were included. Demographic, clinical, and outcome data were collected via a standardized form. Data were analyzed descriptively, including frequencies and percentages for categorical data and means and SDs for continuous data. Predictors for admission were identified using multivariate logistic regression models. ED records from 527 visits for angioedema by 455 patients were included in the study. Annual rate of angioedema was 1 per 1000 ED visits. Urticaria was associated with peripheral (p = 0.008) and lip angioedema (p = 0.001), and the absence of urticaria correlated with tongue angioedema (p = 0.001) and trended toward correlation with pharyngeal angioedema (p = 0.056). Significant predictors of admission included nonsteroidal anti-inflammatory drug-induced angioedema (odds ratio [OR], 15.3), epinephrine treatment (OR, 8.34), hypotension (OR, 15.7), multiple-site angioedema (OR, 4.25), and pharyngeal (OR, 1.23) and tongue angioedema (OR, 4.62). Concomitant urticaria was associated with a significant longer stay in the ED (p angioedema, need for airway management, length of ED visit, and recurrence. A detailed drug and family history, screening blood work for C1 esterase

  6. Angioedema deaths in the United States, 1979-2010.

    Science.gov (United States)

    Kim, Susan J; Brooks, Jordan C; Sheikh, Javed; Kaplan, Michael S; Goldberg, Bruce J

    2014-12-01

    Hospital admission data indicate that the angioedema incidence has increased during the past several decades. Little is known about mortality trends. To count the number of deaths associated with angioedema in the United States, investigate correlations with age, sex, race, and other contributory causes, and analyze trends from 1979 to 2010. All US death certificates in which angioedema was listed as an underlying or contributing cause of death during 1979 to 2010 were analyzed. Age-adjusted mortality rates were analyzed by age, sex, and race. Other conditions designated as the underlying cause of death were investigated. From 1979 to 2010, there were 5,758 deaths in which angioedema was listed as a contributing cause. The age-adjusted death rate for hereditary angioedema decreased from 0.28 (95% confidence interval [CI] 0.25-0.32) to 0.06 (95% CI 0.05-0.08) per million persons per year. Conversely, mortality for angioedema increased from 0.24 (95% CI 0.21-0.27) to 0.34 (95% CI 0.31-0.37) per million. Blacks constituted 55% of angioedema deaths that were associated with use of angiotensin-converting enzyme inhibitors. On death certificates that listed hereditary angioedema as the underlying cause of death, cancer (frequently lymphoma or leukemia) was the second most commonly listed cause. Angioedema-associated deaths were very rare from 1979 to 2010. Hereditary angioedema deaths became even more so, whereas nonhereditary angioedema deaths increased. Risks associated with angiotensin-converting enzyme inhibitors were higher in blacks. Lack of specific coding for acquired angioedema most likely explains the observed association between cancer and hereditary angioedema. In the future, more granular coding systems may help distinguish hereditary from acquired angioedema. Copyright © 2014 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  7. Angioedema due to Systemic Isotretinoin Therapy

    Directory of Open Access Journals (Sweden)

    Pelin Üstüner

    2014-01-01

    Full Text Available Angioedema is the swelling of the mucosal membranes as a variant of urticaria induced by hereditary C1 esterase inhibitor enzyme deficiency, certain foods, or drugs. Herein, we report the case of a 23-year-old woman, with mild-moderate acne presenting with widespread facial angioedema on the 2nd day of systemic isotretinoin treatment. The patient had taken no drugs other than isotretinoin in the preceding days and had no known food allergy. Her angioedema was resolved after the isotretinoin was discontinued. We want to draw the attention of dermatologists to this rare adverse allergic effect of isotretinoin which is frequently used in the treatment of acne vulgaris.

  8. Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group.

    Science.gov (United States)

    Cicardi, M; Aberer, W; Banerji, A; Bas, M; Bernstein, J A; Bork, K; Caballero, T; Farkas, H; Grumach, A; Kaplan, A P; Riedl, M A; Triggiani, M; Zanichelli, A; Zuraw, B

    2014-05-01

    Angioedema is defined as localized and self-limiting edema of the subcutaneous and submucosal tissue, due to a temporary increase in vascular permeability caused by the release of vasoactive mediator(s). When angioedema recurs without significant wheals, the patient should be diagnosed to have angioedema as a distinct disease. In the absence of accepted classification, different types of angioedema are not uniquely identified. For this reason, the European Academy of Allergy and Clinical Immunology gave its patronage to a consensus conference aimed at classifying angioedema. Four types of acquired and three types of hereditary angioedema were identified as separate forms from the analysis of the literature and were presented in detail at the meeting. Here, we summarize the analysis of the data and the resulting classification of angioedema. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  9. The Angiotensin-Converting-Enzyme-Induced Angioedema.

    Science.gov (United States)

    Bas, Murat

    2017-02-01

    The bradykinin B2 receptor antagonist icatibant is effective in angiotensin-converting enzyme inhibitor-induced angioedema. The drug is not approved officially for this indication and has to be administered in an emergency situation off-label. Corticosteroids or antihistamines do not seem to work in this condition. The effectiveness of C1-esterase-inhibitor in angiotensin-converting enzyme-induced angioedema must be verified in a double-blind study. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Exploring the HME and HAE1 efflux systems in the genus Burkholderia

    Directory of Open Access Journals (Sweden)

    Pasca Maria

    2010-06-01

    Full Text Available Abstract Background The genus Burkholderia includes a variety of species with opportunistic human pathogenic strains, whose increasing global resistance to antibiotics has become a public health problem. In this context a major role could be played by multidrug efflux pumps belonging to Resistance Nodulation Cell-Division (RND family, which allow bacterial cells to extrude a wide range of different substrates, including antibiotics. This study aims to i identify rnd genes in the 21 available completely sequenced Burkholderia genomes, ii analyze their phylogenetic distribution, iii define the putative function(s that RND proteins perform within the Burkholderia genus and iv try tracing the evolutionary history of some of these genes in Burkholderia. Results BLAST analysis of the 21 Burkholderia sequenced genomes, using experimentally characterized ceoB sequence (one of the RND family counterpart in the genus Burkholderia as probe, allowed the assembly of a dataset comprising 254 putative RND proteins. An extensive phylogenetic analysis revealed the occurrence of several independent events of gene loss and duplication across the different lineages of the genus Burkholderia, leading to notable differences in the number of paralogs between different genomes. A putative substrate [antibiotics (HAE1 proteins/heavy-metal (HME proteins] was also assigned to the majority of these proteins. No correlation was found between the ecological niche and the lifestyle of Burkholderia strains and the number/type of efflux pumps they possessed, while a relation can be found with genome size and taxonomy. Remarkably, we observed that only HAE1 proteins are mainly responsible for the different number of proteins observed in strains of the same species. Data concerning both the distribution and the phylogenetic analysis of the HAE1 and HME in the Burkholderia genus allowed depicting a likely evolutionary model accounting for the evolution and spreading of HME and HAE

  11. "Nuts and Bolts" of Laboratory Evaluation of Angioedema.

    Science.gov (United States)

    Farkas, Henriette; Veszeli, Nóra; Kajdácsi, Erika; Cervenak, László; Varga, Lilian

    2016-10-01

    Angioedema, as a distinct disease entity, often becomes a clinical challenge for physicians, because it may cause a life-threatening condition, whereas prompt and accurate laboratory diagnostics may not be available. Although the bedside diagnosis needs to be established based on clinical symptoms and signs, family history, and the therapeutic response, later, laboratory tests are available. Currently, only for five out of the nine different types of angioedema can be diagnosed by laboratory testing, and these occur only in a minority of the patient population. Hereditary angioedema with C1-inhibitor (C1-INH) deficiency type I can be diagnosed by the low C1-INH function and concentration, whereas in type II, C1-INH function is low, but its concentration is normal or even elevated. C1q concentration is normal in both forms. Acquired angioedema with C1-INH deficiency type I is characterized by the low C1-INH function and concentration; however, C1q concentration is also low, and autoantibodies against C1-INH cannot be detected. Complement profile of acquired angioedema with C1-INH deficiency type II is similar to that of type I, but in this form, autoantibodies against C1-INH are present. Hereditary angioedema due to a mutation of the coagulation factor XII can be diagnosed exclusively by mutation analysis of FXII gene. Diagnostic metrics are not available for idiopathic histaminergic acquired angioedema, idiopathic non-histaminergic acquired angioedema, acquired angioedema related to angiotensin-converting enzyme inhibitor, and hereditary angioedema of unknown origin; these angioedemas can be diagnosed by medical and family history, clinical symptoms, and therapeutic response and by excluding the forms previously described. Several potential biomarkers of angioedema are used to date only in research. In the future, they could be utilized into the clinical practice to improve the differential diagnosis, therapy, as well as the prognosis of angioedema.

  12. [Biological investigation of kinin-mediated angioedema].

    Science.gov (United States)

    Defendi, F; Charignon, D; Ghannam, A; Ponard, D; Drouet, C

    2015-03-01

    Kinin-mediated angioedema results from accumulation of kinins, vasoactive and vasopermeant peptides, on the vascular endothelium. The disease is characterized by sudden episodes of swelling in the subcutaneous and submucosal tissues; the edema may occur spontaneously or it may be precipitated by triggering factors such as physical or emotional stress, or certain medicines. The characterization of kinin formation and catabolism systems helps improve knowledge of the aetiopathogenic mechanisms involved and provides the basis for classification of kinin-mediated angioedema conditions; thus, we may distinguish between angioedema with C1 inhibitor deficiency, whether inherited or acquired, and angioedema with normal C1 inhibitor activity, associated with increased kinin-forming activity or deficiency in kinin catabolism enzymes. In support of the clinical diagnosis, the physician may request laboratory investigation for a functional and molecular definition of the disease. Laboratory diagnosis is based on the characterization of: (1) kinin production control by C1 inhibitor investigation (function, antigen levels and circulating species); (2) kinin production (kininogenase activity, kininogen cleavage species); and (3) kinin catabolism enzymes (aminopeptidase P, carboxypeptidase N, angiotensin-I converting enzyme and dipeptidyl peptidase IV). An abnormal biological phenotype is supported by examination of susceptibility genes (SERPING1, F12 and XPNPEP2) and mutation segregation in the families. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  13. Epidemiology of Non-hereditary Angioedema

    DEFF Research Database (Denmark)

    Madsen, Flemming; Attermann, Jorn; Linneberg, Allan

    2012-01-01

    The prevalence of non-hereditary angioedema was investigated in a general population sample (n = 7,931) and in a sample of Danish patients (n = 7,433) tested for deficiency of functional complement C1 esterase inhibitor protein (functional C1 INH). The general population sample (44% response rate...

  14. Epidemiology of Non-hereditary Angioedema

    DEFF Research Database (Denmark)

    Madsen, Flemming; Attermann, Jørn; Linneberg, Allan

    2012-01-01

    The prevalence of non-hereditary angioedema was investigated in a general population sample (n¿=¿7,931) and in a sample of Danish patients (n¿=¿7,433) tested for deficiency of functional complement C1 esterase inhibitor protein (functional C1 INH). The general population sample (44% response rate...

  15. Idiopathic Non-histaminergic Angioedema: Successful Treatment with Omalizumab in Five Patients.

    Science.gov (United States)

    Faisant, Charles; Du Thanh, Aurélie; Mansard, Catherine; Deroux, Alban; Boccon-Gibod, Isabelle; Bouillet, Laurence

    2017-01-01

    Idiopathic non-histaminergic acquired angioedema (InH-AAE) is a rare disease characterized by AE resistant to antihistamines and a chronic course. We report five new cases of InH-AAE (two women and three men) with a rapid and dramatic response to the anti-immunoglobulin-E antibody omalizumab. In our literature review, we found 13 other relevant cases with a good response to this treatment. Overall, in 6 out of 18 patients, the doses of omalizumab required to prevent recurrences of attacks were higher than the licensed dose for chronic urticaria. No significant adverse effects have been reported.

  16. Antihistamine-resistant angioedema in women with negative family history: estrogens and F12 gene mutations.

    Science.gov (United States)

    Bork, Konrad; Wulff, Karin; Witzke, Günther; Stanger, Christian; Lohse, Peter; Hardt, Jochen

    2013-12-01

    In women with sporadic recurrent angioedema with an unknown cause who are unresponsive to antihistamines and have normal C1 inhibitor activity and a negative family history of angioedema, it is unclear whether they have idiopathic angioedema or hereditary angioedema with normal C1 inhibitor, and what impact exogenous estrogens have on their angioedema. A cohort of 147 women was analyzed for F12 exon 9 mutations and for the influence of oral contraceptives, hormonal replacement therapy, and pregnancy on their angioedema. A total of 142 women had idiopathic angioedema unresponsive to antihistamines. Five women had an F12 mutation and thereby hereditary angioedema with F12 mutations. Among the women with idiopathic angioedema, 63 had never taken estrogens. There was no estrogen impact in 42 women, a moderate impact in 15 women, and a severe impact in 22 women. The type and dose of estrogens did not differ in women with and without an estrogen impact. In 5 women, idiopathic angioedema disappeared after desogestrel use. Among the 5 women with hereditary angioedema with F12 mutations, angioedema symptoms occurred during 4 pregnancies, whereas no symptoms occurred during any of the 58 pregnancies in women with idiopathic angioedema. Women with recurrent angioedema unresponsive to antihistamines may have idiopathic angioedema or, more rarely, hereditary angioedema with F12 mutations. Both conditions may be provoked or aggravated by exogenous estrogens. In idiopathic angioedema, treatment with progestins may be helpful. Copyright © 2013. Published by Elsevier Inc.

  17. Angiotensin Converting Enzyme-induced Angioedema - A Dangerous New Epidemic

    DEFF Research Database (Denmark)

    Rasmussen, Eva Rye; Mey, Kristianna; Bygum, Anette

    2013-01-01

    Angioedema is a sudden localised and often asymmetric swelling of the skin or mucous membranes caused by transient increased endothelial permeability causing plasma extravasation. In the last decades the incidence of severe angioedema involving the upper airways and even fatal outcome due....... The diagnosis is often delayed and traditional treatment usually ineffective. Complement C1 inhibitor concentrate and bradykinin receptor antagonists, normally used to treat patients with hereditary angioedema, have shown good results when used in patients with bradykinin-mediated angioedema. This review...

  18. [Facial angioedema after filler injections. Description of five cases].

    Science.gov (United States)

    Cosatti, Micaela; Fernández Romero, Diego S; Juri, María Cecilia; Malbrán, Alejandro

    2010-01-01

    The use of fillers for cosmetic purposes is becoming increasingly frequent. Although initially considered inert, these products produce adverse reactions around the injection site. We present 5 cases of women with a history of filler injections who presented a hard and persistent angioedema followed by local subcutaneous nodules. They were referred to the allergist for suspected allergy related angioedema without response to usual antihistamine treatment. The angioedema episodes initiated 27.6 months (range 1 to 48) after the fillers treatment. The patients underwent exacerbations and remissions of angioedema, partially relieved with oral steroids and, in 2 cases, local triamcinolone injections. Mean time from onset of symptoms to remission of angioedema was 8.75 months (range 1 to 24). Until October 2009 four patients continued into remission after 24.5 months (range 7 to 36) free of symptoms. One patient continued with exacerbations 11 months after the initial symptoms. Fillers may cause angioedema as an adverse event and should be considered in the differential diagnosis of persistent angioedema. They are only sensitive to steroid treatment and in some steroid dependent cases they respond to ciclosporin. The frequency of angioedema after filler injections among patients with angioedema in the Unit of Asthma Allergy and Clinical Immunology was 0.5%.

  19. Fatal laryngeal angioedema: a case report and a workup of angioedema in a forensic setting.

    Science.gov (United States)

    Krizova, Adriana; Gardner, Taylor; Little, D'Arcy L; Arcieri-Piersanti, V; Pollanen, Michael S

    2015-12-01

    Angioedema is an episodic swelling of the deep dermis, subcutis, and/or submucosal tissue due to an increase in local vascular permeability. Swelling may involve skin, respiratory, and gastrointestinal tracts. The most commonly involved areas are the periorbital region and the lips. Here we report a case of a fatal laryngeal obstruction due to angioedema likely caused by an angiotensin-converting-enzyme inhibitor. The decedent, a 58-year-old man, was witnessed developing sudden facial swelling and acute respiratory difficulties quickly followed by unresponsiveness. His past medical history suggested that this was his second episode of angioedema without urticaria. Postmortem examination revealed a complete laryngeal obstruction in the absence of infection, neoplasm, or autoimmune disease. Postmortem computed tomography of the head and neck showed a complete obstruction of the upper airway. Based on the current understanding of the pathophysiology of different types of angioedema, we will suggest a workup of angioedema without urticaria in the forensic setting and offer readers resources they can use in their practice.

  20. Angioedema Triggered by Medication Blocking the Renin/Angiotensin System: Retrospective Study Using the French National Pharmacovigilance Database.

    Science.gov (United States)

    Faisant, Charles; Armengol, Guillaume; Bouillet, Laurence; Boccon-Gibod, Isabelle; Villier, Céline; Lévesque, Hervé; Cottin, Judith; Massy, Nathalie; Benhamou, Ygal

    2016-01-01

    Bradykinin-mediated angioedema (AE) is a rare side effect of some medications, including angiotensin converting enzyme inhibitors (ACEi) and angiotensin receptor blockers (ARB). In France, side-effects to treatments are reported to the national pharmacovigilance database. The national MedDRA database was searched using the term "angioedema". Patients were included if they met the clinical criteria corresponding to bradykinin-mediated AE, if their C1-inhibitor levels were normal, and if they were treated with an ACEi or an ARB. 7998 cases of AE were reported between 1994 and 2013. Among these, 112 met the criteria for bradykinin-mediated AE with normal C1-inhibitor levels. On the 112 drug-AE, patients were treated with an ARB in 21% of cases (24 patients), or an ACEi in 77% of cases (88 patients), in combination with another treatment in 17 cases (mTORi for 3 patients, iDPP-4 for 1 patient, hormonal treatment for 7 patients). ENT involvement was reported in 90% of cases (tongue: 48.2%, larynx: 23.2%). The median duration of treatment before the first attack was 720 days, and the mean duration of attacks was 36.6 h. Forty-one percent (19/46) of patients relapsed after discontinuing treatment. Angioedema triggered by medication blocking the renin/angiotensin system is rare but potentially severe, with a high risk of recurrence despite cessation of the causative drug.

  1. Angioedema Due to Pear: Medical Image

    Directory of Open Access Journals (Sweden)

    Cemil Kavalci

    2014-03-01

    Acute allergic angioedema is an abrupt-onset, unpredictable inflammatory reaction of the skin and mucous membranes. A 60-years-old female patient presented to our emergency department with dispne and edema in her mouth and lips. It was learned from the history that her symptoms were begun 15 minutes after eating a pear. 40 mg methylprednisolone and 50 mg diphenhydramine were administered intravenously.

  2. Angioedema due to Pomegranate: Original Image

    OpenAIRE

    Meliha Findik

    2014-01-01

     Acute allergic angioedema is an abrupt-onset, unpredictable inflammatory reaction of the skin and mucous membranes. A 35-year-old female patient presented to our emergency department with redness on the cheeks and edema in her mouth and eyelids. It was learned from the history that her symptoms were begun 15 minutes after eating a pomegranate. 40 mg methylprednisolone and 50 mg diphenhydramine were administered intravenously.

  3. Angioedema due to Pomegranate: Original Image

    Directory of Open Access Journals (Sweden)

    Meliha Findik

    2014-03-01

    Full Text Available  Acute allergic angioedema is an abrupt-onset, unpredictable inflammatory reaction of the skin and mucous membranes. A 35-year-old female patient presented to our emergency department with redness on the cheeks and edema in her mouth and eyelids. It was learned from the history that her symptoms were begun 15 minutes after eating a pomegranate. 40 mg methylprednisolone and 50 mg diphenhydramine were administered intravenously.

  4. Angioedema Due to Pear: Medical Image

    OpenAIRE

    Cemil Kavalci

    2014-01-01

      Acute allergic angioedema is an abrupt-onset, unpredictable inflammatory reaction of the skin and mucous membranes. A 60-years-old female patient presented to our emergency department with dispne and edema in her mouth and lips. It was learned from the history that her symptoms were begun 15 minutes after eating a pear. 40 mg methylprednisolone and 50 mg diphenhydramine were administered intravenously.

  5. Hereditary angioedema type I: a case report

    Directory of Open Access Journals (Sweden)

    Francisca Muñoz Peralta

    2016-03-01

    Full Text Available El angioedema hereditario es una enfermedad rara, de gran heterogeneidad en los síntomas, manifestándose con edema a nivel cutáneo, mucosa gastrointestinal y de laringe/faringe. Aunque existen tres variedades, el tipo I es el más frecuente y es provocado por una deficiencia en la síntesis del complemento C1 inhibidor. La gravedad de la clínica, junto a la baja prevalencia de la enfermedad y la necesidad de un tratamiento específico, hacen que el diagnóstico y tratamiento de dicha patología sea aún una asignatura pendiente para el médico de familia en atención primaria. Presentamos el caso de un adolescente varón con déficit de α-1 antitripsina desde los seis meses de edad, con aparición de angioedemas en piernas y brazos a los 11 años, diagnosticado de angioedema hereditario tipo I un año después. El diagnóstico definitivo de la enfermedad permitió instaurar un tratamiento adecuado a su patología, que consiste en la prevención de brotes que puedan comprometer la vida del paciente y, en el caso de que aparezcan, en la administración del complemento C1 inhibidor.

  6. Cardiovascular and Diabetic Medications That Cause Bradykinin-Mediated Angioedema.

    Science.gov (United States)

    Hudey, Stephanie N; Westermann-Clark, Emma; Lockey, Richard F

    Medication-induced angioedema is a bradykinin-mediated process that results from increased production or decreased degradation of bradykinin. These reactions are documented for several cardiac medications including blockers of the renin-angiotensin-aldosterone system (RAAS). Other cardiovascular and diabetes medications further increase the risk of medication-induced angioedema, particularly with concomitant use of RAAS inhibitors. Dipeptidyl peptidase IV inhibitors are a class of oral diabetic agents that affect bradykinin and substance P degradation and therefore can lead to angioedema. Neprilysin inhibitors are a separate class of cardiac medications, which includes sacubitril, and can lead to drug-induced angioedema especially when used in combination with RAAS inhibitors. This article discusses the proposed mechanisms by which these medications cause angioedema and how medication-induced angioedema differs from mast cell-mediated angioedema. It also details how to recognize medication-induced angioedema and the treatment options available. Copyright © 2017 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  7. Methicillin-resistant Staphylococcus Aureus Lip Infection Mimicking Angioedema.

    Science.gov (United States)

    Lucerna, Alan R; Espinosa, James; Darlington, Anne M

    2015-07-01

    It is rare for angioedema to be misidentified by the experienced clinician or for it to mimic another disease process. As an Emergency Physician, it is important to recognize and treat angioedema immediately. Of equal importance is the recognition and initiation of treatment of facial cellulitis. A case report follows that illustrates methicillin-resistant Staphylococcus aureus (MRSA) lip infection mimicking angioedema. Here, we describe a case of a 21-year-old man who presented with a swollen lower lip, initially diagnosed as angioedema. Further investigation revealed the cause of his lip swelling was actually a MRSA abscess and surrounding cellulitis, an unusual presentation for lip infection, which we discuss below. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Misidentifying MRSA lip infection for angioedema, with a delay in proper treatment, could result in serious morbidity or mortality. Copyright © 2015 Elsevier Inc. All rights reserved.

  8. C1 Inhibitor Deficiency and Angioedema of the Small Intestine Masquerading as Crohn’s Disease

    Directory of Open Access Journals (Sweden)

    Kelly W Burak

    2000-01-01

    Full Text Available A case of C1 inhibitor deficiency presenting as localized edema of the small intestine is described. A 16-year-old, previously healthy woman presented with recurrent attacks of abdominal pain and vomiting following minor abdominal trauma. Investigations including computed tomography scan and barium studies confirmed localized edema of the jejunum. At laparoscopy, Crohn’s disease was suspected; however, a subsequent enteroscopy was normal. Complement levels revealed a low C4 level, and C1 inhibitor deficiency was later confirmed. Attacks of abdominal pain began after starting oral contraceptives and have not returned since stopping the birth control pill. This rare cause of abdominal pain is examined, and C1 inhibitor deficiency and angioedema are reviewed.

  9. SMJERNICE ZA DIJAGNOSTIKU I LIJEČENJE HEREDITARNOG ANGIOEDEMA

    OpenAIRE

    Stipić Marković, Asja; Rožmanić,, Vojko; Anić, Branimir; Aberle, Neda; Račić, Goran; Novak, Srđan; Sunara, Davor; Grdinić, Boris; Karadža-Lapić, Ljerka; Ražov Radas, Melanija; Karanović, Boris; Kvenić, Barbara

    2014-01-01

    Hereditarni angioedem (HAE) rijetka je, ali potencijalno za život opasna bolest zbog nepredvidivih napadaja bezbolnih, ograničenih, recidivirajućih otoka supkutanog ili submukoznog, intersticijskog tkiva u trajanju od nekoliko sati do nekoliko dana. Oboljelih od HAE u RH ima oko 100 (ali vjerojatno ima više nedijagnosticiranih). Poseban kvalitativni napredak u usklađivanju dogovora o liječenju HAE jesu Smjernice Svjetske alergološke organizacije koje su donesene 2012. Oslanjajući se na taj do...

  10. Prevalence of autoantibodies in a group of hereditary angioedema patients Prevalência de autoanticorpos em uma população com angioedema hereditário

    OpenAIRE

    Sergio Duarte Dortas Junior; Solange Oliveira Rodrigues Valle; Soloni Afra Pires Levy; Rosangela P. Tortora; Augusto Tiaqui Abe; Gisele Viana Pires; José Angelo de Souza Papi; Alfeu Tavares França

    2012-01-01

    Hereditary Angioedema is a dominantly inherited disease. Routine screening of autoantibodies (AAB) is not recommended for individuals with Hereditary Angioedema; however, prevalence of these antibodies in Hereditary Angioedema patients is not well documented. We aim to determine the prevalence of AAB so that individuals at risk of developing autoimmune diseases can be identified. Fifteen patients with Hereditary Angioedema attended at Clementino Fraga Filho University Hospital accepted to par...

  11. Angiotensin-converting enzyme inhibitor-induced angioedema.

    Science.gov (United States)

    Bezalel, Shira; Mahlab-Guri, Keren; Asher, Ilan; Werner, Ben; Sthoeger, Zev Moshe

    2015-02-01

    Angiotensin-converting enzyme inhibitors (ACE-I) are widely used, effective, and well-tolerated antihypertensive agents. The mechanisms by which those agents act can cause side effects such as decreased blood pressure, hyperkalemia, and impaired renal function. ACE-I can induce cough in 5%-35% and angioedema in up to 0.7% of treated patients. Because cough and angioedema are considered class adverse effects, switching treatment to other ACE-I agents is not recommended. Angioedema due to ACE-I has a low fatality rate, although deaths have been reported when the angioedema involves the airways. Here, we review the role of bradykinin in the development of angioedema in patients treated with ACE-I, as well as the incidence, risk factors, clinical presentation, and available treatments for ACE-I-induced angioedema. We also discuss the risk for recurrence of angioedema after switching from ACE-I to angiotensin receptor blockers treatment. Copyright © 2015 Elsevier Inc. All rights reserved.

  12. Management of angioedema without urticaria in the emergency department.

    Science.gov (United States)

    Pedrosa, Maria; Prieto-García, Alicia; Sala-Cunill, Anna

    2014-12-01

    Angioedema refers to a localized, transient swelling of the deep skin layers or the upper respiratory or gastrointestinal mucosa. It develops as a result of mainly two different vasoactive peptides, histamine or bradykinin. Pathophysiology, as well as treatment, is different in each case; nevertheless, the resulting signs and symptoms may be similar and difficult to distinguish. Angioedema may occur at any location. When the affected area involves the upper respiratory tract, both forms of angioedema can lead to an imminent upper airway obstruction and a life-threatening emergency. Emergency physicians must have a basic understanding of the pathophysiology underlying this process. Angioedema evaluation in the emergency department (ED) should aim to distinguish between histamine- and bradykinin-induced angioedema, in order to provide appropriate treatment to patients. However, diagnostic methods are not available at the ED setting, neither to confirm one mechanism or the other, nor to identify a cause. For this reason, the management of angioedema should rely on clinical data depending on the particular features of the episode and the patient in each case. The history-taking should be addressed to identify a possible etiology or triggering agent, recording complete information for an ulterior diagnostic study in the outpatient clinic. It is mandatory quickly to recognize and treat a potential life-threatening upper airway obstruction or anaphylaxis. This review focuses on the underlying mechanisms and management of histamine- and bradykinin-induced angioedema at the emergency department and provides an update on the currently available treatments.

  13. Adverse events reported for hereditary angioedema medications: a retrospective study of spontaneous reports submitted to the EudraVigilance database, 2007-2013

    Directory of Open Access Journals (Sweden)

    Aagaard L

    2016-05-01

    Full Text Available Lise Aagaard,1 Anette Bygum,2 1Section for Clinical Pharmacology, Institute of Public Health, Faculty of Health Sciences, University of Southern Denmark, 2Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark Abstract: Information about long-term safety issues from use of orphan drugs in treatment of hereditary angioedema (HAE is limited and must be studied further. As clinical trials in patients with rare diseases are limited, prescribers and patients have to rely on spontaneous adverse drug reaction (ADR reports for obtaining major information about the serious, rarely occurring, and unknown ADRs. In this study, we aimed to characterize ADRs reported for HAE medications in Europe from 2007 to 2013. ADR reports submitted for C1-inibitors and bradykinin receptor antagonists to the European ADR database, EudraVigilance (EV, were included in this study. The ADR reports were categorized with respect to age and sex of the patients, category of the reporter, type and seriousness of the reported ADRs, and medications. The unit of analysis was one adverse event (AE. Totally, 187 AEs were located in EV, and of these, 138 AEs were reported for Cinryze® (C1-inhibitor (73% of the total and 49 AEs for Firazyr® (icatibant (26% of the total AEs. Approximately 60% of all AEs were serious, including three fatal cases. Less than 5% of AEs were reported in children. In total, 62% of AEs were reported for women and 38% for men. For both Cinryze® and Firazyr®, the majority of reported AEs were of the type “general disorders and administration site conditions”. For Cinryze®, a large number of AEs of the type “HAE” and “drug ineffective” was reported, but only few of these were serious. For Firazyr®, several nonserious reports on injection site reactions were reported. In conclusion, this study showed that in EV, several ADR reports from use of HAE medications were identified, and a large number of these were

  14. Heart Attack

    Science.gov (United States)

    Each year almost 800,000 Americans have a heart attack. A heart attack happens when blood flow to the heart suddenly ... it's important to know the symptoms of a heart attack and call 9-1-1 if you or ...

  15. Posthuman Difference: Traveling to Utopia with Young-Hae Chang Heavy Industries

    Directory of Open Access Journals (Sweden)

    Warren Liu

    2012-06-01

    Full Text Available This essay puts into conversation two rarely conjunctive discourses: posthumanism, which focuses on how technological mediation forces a reconsideration of the very categories of “subject,” “object,” and “literature”; and Asian American literary criticism, which seeks to continually interrogate how Asian American subjects are produced, reproduced, and represented. Putting these two discourses into conversation yields several important results: for one, posthumanist theory allows for a more complex understanding of the shift, within Asian American criticism, from nation-bound models to transnational frameworks. Moreover, posthumanism’s emphasis on technological mediation provides an important new theoretical framework for Asian American literary criticism, particularly in terms of the way that subjects are produced and reproduced in conjunction with technological objects. At the same time, Asian American literary criticism’s focus on the material effects of cultural productions pinpoints and illuminates a critical aporia in posthuman theory: its uncertain and equivocal treatment of race and ethnicity. The essay concludes with a reading of Young-Hae Chang Heavy Industries’ Traveling to Utopia: With a Brief History of the Technology. Considered together, the form and content of the piece enact an aesthetics of “posthuman difference,” which both highlights the limits, and requires the strengths, of posthumanist and Asian Americanist discourse.

  16. Angioedema por rellenos faciales: Descripción de cinco casos Facial angioedema after filler injections: Description of five cases

    Directory of Open Access Journals (Sweden)

    Micaela A. Cosatti

    2010-12-01

    Full Text Available En los últimos años se ha incrementado la utilización de sustancias de relleno facial con fines estéticos. Estos productos, originalmente considerados inertes, se asocian con diversos efectos adversos localizados alrededor del sitio de la aplicación. Describimos a 5 mujeres con antecedentes de inyecciones de sustancia de relleno facial que presentaron como síntoma inicial angioedema facial duro y persistente seguido por la aparición de nódulos subcutáneos. Todas las pacientes fueron derivadas al servicio de alergia por sospecha de angioedema de causa alérgica sin respuesta al tratamiento con antihistamínicos. El angioedema inició 27.6 meses (1 a 48 luego de la inyección del producto, y las pacientes evolucionaron con brotes y remisiones que fueron tratados con corticoides orales y en 2 oportunidades con inyecciones locales. El tiempo medio desde el inicio de los síntomas hasta la remisión del angioedema fue 8.75 meses (1 a 24. A octubre de 2009 cuatro pacientes se mantuvieron en remisión persistente, luego de un seguimiento clínico de 24.5 meses (7 a 36. Una paciente continúa con exacerbaciones luego de 11 meses de iniciados los síntomas. Las sustancias de relleno facial pueden producir angioedema como evento adverso y deben ser consideradas en el diagnóstico diferencial del angioedema persistente. Sólo responden al tratamiento con esteroides y en algunos casos esteroides dependientes, con ciclosporina. La frecuencia de angioedema por rellenos faciales entre pacientes con angioedema asistidos en la Unidad de Asma, Alergia e Inmunología Clínica fue del 0.5%.The use of fillers for cosmetic purposes is becoming increasingly frequent. Although initially considered inert, these products produce adverse reactions around the injection site. We present 5 cases of women with a history of filler injections who presented a hard and persistent angioedema followed by local subcutaneous nodules . They were referred to the allergist for

  17. Refractory Angioedema in a Patient with Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Zahra Habibagahi

    2015-07-01

    Full Text Available Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless edema, but edema of the genital area, gastrointestinal and laryngeal tracts have also been reported. In lupus patients, angioedema may be the result of an acquired type of C1 inhibitor deficiency, most probably due to antibody formation directed against the C1 inhibitor molecule. Herein we report a new case of lupus nephritis that developed angioedema and a rapid course of disease progression with acute renal failure and alveolar hemorrhage without response to high dose steroid and plasmapheresis.

  18. [Hereditary angioedema by C1 inhibitor-deficit: Diagnostic and therapeutic challenges. Case report].

    Science.gov (United States)

    Mayorga, Álvaro José; Ayestas-Moreno, Gerardo José

    2017-01-01

    Hereditary angioedema is a disease which manifests itself with episodes of spontaneous edema on skin, mucosal and airway. Treatment includes acute and prophylactic approach to minimize the attacks and severity. In many parts of the world, androgen derivatives, antifibrinolytic and fresh frozen plasma are the therapies available for prophylaxis. 16 years old teenager of without history of immune decease, has in the course of 1-year repetitive episodes of painless, non-pruritic angioedema, does not respond to antihistamine therapy, corticosteroids or adrenaline; fresh frozen plasma is applied in 1 occasion exacerbating episode with severity. The diagnosis is delayed because of the unavailability of the study in the country, so it is shipped abroad confirming the deficit of C1 Inhibitor (7.1 μg/mL). Initiating prophylactic therapy with Danazol, with subsequent episodes decreased. The delay diagnosis involves considerable risk in these patients; the importance of long-term prophylactic treatment is ratified in the use of androgens, being as an available option in developing countries.

  19. Ticagrelor-Induced Angioedema: A Rare and Unexpected Phenomenon

    Directory of Open Access Journals (Sweden)

    Rajeev Seecheran

    2017-01-01

    Full Text Available Angioedema can cause potentially life-threatening airway obstruction. This case report describes an exceedingly rare episode of ticagrelor-induced hypersensitivity reaction, manifesting as angioedema with periorbital and likely respiratory involvement. The heart team should be vigilant for this precarious condition which may require emergent airway management. Desensitization protocols and alternative regimens (e.g., clopidogrel, prasugrel, and addition of an adjunctive anticoagulant should be considered when there is an absolute indication for antiplatelet therapy.

  20. Ticagrelor-Induced Angioedema: A Rare and Unexpected Phenomenon.

    Science.gov (United States)

    Seecheran, Rajeev; Seecheran, Valmiki; Persad, Sangeeta; Lalla, Sasha; Seecheran, Naveen Anand

    2017-01-01

    Angioedema can cause potentially life-threatening airway obstruction. This case report describes an exceedingly rare episode of ticagrelor-induced hypersensitivity reaction, manifesting as angioedema with periorbital and likely respiratory involvement. The heart team should be vigilant for this precarious condition which may require emergent airway management. Desensitization protocols and alternative regimens (e.g., clopidogrel, prasugrel, and addition of an adjunctive anticoagulant) should be considered when there is an absolute indication for antiplatelet therapy.

  1. Emergency department management of patients with ACE-inhibitor angioedema.

    Science.gov (United States)

    Winters, Michael E; Rosenbaum, Steven; Vilke, Gary M; Almazroua, Faisal Y

    2013-11-01

    Angiotensin-converting-enzyme inhibitors (ACEI) are one of the most prescribed medications worldwide. Angioedema is a well-recognized adverse effect of this class of medications, with a reported incidence of ACEI angioedema of up to 1.0%. Of importance to note, ACEI angioedema is a class effect and is not dose dependent. The primary goal of this literature search was to determine the appropriate Emergency Department management of patients with ACEI angioedema. A MEDLINE literature search from January 1990 to August 2012 and limited to human studies written in English for articles with keywords of ACEI angioedema. Guideline statements and non-systematic reviews were excluded. Studies identified then underwent a structured review from which results could be evaluated. Five hundred sixty-two papers on ACEI angioedema were screened and 27 appropriate articles were rigorously reviewed in detail and recommendations given. The literature search did not support any specific treatment protocol with a high level of evidence due to the limited--and limitations of the--available studies. Copyright © 2013 Elsevier Inc. All rights reserved.

  2. Floral organ abscission peptide IDA and its HAE/HSL2 receptors control cell separation during lateral root emergence

    OpenAIRE

    Kumpf, Robert P.; Shi, Chun-Lin; Larrieu, Antoine; Stø, Ida Myhrer; Butenko, Melinka A.; Péret, Benjamin; Riiser, Even Sannes; Bennett, Malcolm J.; Aalen, Reidunn B.

    2013-01-01

    Throughout their life cycle, plants produce new organs, such as leaves, flowers, and lateral roots. Organs that have served their purpose may be shed after breakdown of primary cell walls between adjacent cell files at the site of detachment. In Arabidopsis, floral organs abscise after pollination, and this cell separation event is controlled by the peptide INFLORESCENCE DEFICIENT IN ABSCISSION (IDA), which signals through the leucine-rich repeat receptor-like kinases HAESA (HAE) and HAESA-LI...

  3. GHRH|HaeIII Gene Polymorphism in Dairy and Beef Cattle at National Livestock Breeding Centers

    Directory of Open Access Journals (Sweden)

    A. O. Rini

    2013-12-01

    Full Text Available This study was aimed to identify polymorphism of growth hormone releasing hormone (GHRH gene in 89 heads of Holstein-Friesian (HF dairy cattle from Lembang Artificial Insemination Center/LAIC (17 bulls, Singosari Artificial Insemination Center/SAIC (32 bulls, and Cipelang Livestock Embryo Center/CLEC (40 cows; as well as in 4 breeds of female beef cattle from CLEC for comparison, providing Simmental (13 cows, Limousin (14 cows, Brahman (5 cows, and Angus (5 cows. This study used PCR-RFLP method by using HaeIII restriction enzyme. The enzyme cut the GHRH gene at nucleotides of GG|CC at the base positions of 118, 312, and 406 and produced 4 fragments of 118, 194, 94, and 45 bp respectively. Genotyping the GHRH gene produced two types of allele, namely A (312, 94, and 45 bp and B (194, 118, 94, and 45 bp. These two alleles resulted in three types of genotype, namely AA (312, 94, and 45 bp, AB (312, 194, 118, 94, and 45 bp, and BB (194, 118, 94, and 45 bp. Frequency of the B allele was dominant to the A allele. Chi-Square analysis showed that all of HF dairy and beef cattle observed were in Hardy-Weinberg equilibrium (X2 < X2(0.05. The highest heterozygosity value was 0.471 for HF bulls in LAIC, while the lowest one was for HF bulls in SAIC. Heterozygosity values in Simmental and Limousin cattles were higher than that of in HF cattle. The GHRH gene in HF and beef cattle was polymorphic, the exception was for Brahman with the only B allele. This result will improve the understanding of the polymorphism of GHRH gene in dairy and beef cattle.

  4. Attack surfaces

    DEFF Research Database (Denmark)

    Gruschka, Nils; Jensen, Meiko

    2010-01-01

    The new paradigm of cloud computing poses severe security risks to its adopters. In order to cope with these risks, appropriate taxonomies and classification criteria for attacks on cloud computing are required. In this work-in-progress paper we present one such taxonomy based on the notion...... of attack surfaces of the cloud computing scenario participants....

  5. Positive impact of omalizumab on angioedema and quality of life in patients with refractory chronic idiopathic/spontaneous urticaria: analyses according to the presence or absence of angioedema.

    Science.gov (United States)

    Maurer, M; Sofen, H; Ortiz, B; Kianifard, F; Gabriel, S; Bernstein, J A

    2017-06-01

    Approximately 50% of patients with chronic idiopathic/spontaneous urticaria (CIU/CSU) report hives and angioedema; some experience hives/angioedema only. Assess omalizumab's effect on angioedema and quality of life (QoL) in subgroups with refractory CIU/CSU: those with and without angioedema. Patients received omalizumab (75, 150 or 300 mg) or placebo every 4 weeks for 12/24 weeks. Angioedema and QoL were assessed [Urticaria Patient Daily Diary and Dermatology Quality of Life Index (DLQI)]. Subgroups were based on the presence/absence of baseline angioedema 7 days prior to randomization. Patients with baseline angioedema randomized to omalizumab 300 mg had a greater reduction in mean weekly incidence of angioedema and mean number of days/week with angioedema vs. placebo at 12 and 24 weeks. A 3.3- to 4.5-point greater mean reduction in DLQI score was achieved with omalizumab 300 mg treatment vs. placebo, above the minimal clinically important difference threshold. Results with lower doses vs. placebo were variable. Compared with placebo, omalizumab 300 mg treatment over 12-24 weeks resulted in marked reduction in incidence and number of days/week with angioedema accompanied by clinically relevant improvement in QoL. © 2016 European Academy of Dermatology and Venereology.

  6. Epidemiology of angioedema without wheals in an allergy and immunology center.

    Science.gov (United States)

    Malbrán, Eloisa; Fernández Romero, Diego; Juri, Maria Cecilia; Larrauri, Blas J; Malbrán, Alejandro

    2015-01-01

    We describe the diagnostic epidemiology, the clinical course, the family history and the response to treatment of patients with angioedema without wheals (AWW) at an Allergy and Immunology Clinical Center. We reviewed the case records of all patients at our office from January 1997 to April 2013. We recorded sex, age, age at onset of symptoms, family history of angioedema, number of visits to the office, type of angioedema, and response to treatment from those patients with angioedema without wheals. We classified angioedema according to its pathophysiology. We also describe those patients with angioedema mimics. From a total of 17,823 new patients, 303 had a presumptive diagnosis of angioedema without wheals. Twenty-three patients had an angioedema mimic. Forty percent were male and 60% were female. Average age at first visit was 40.6. Average number of visits was 2.4. Fifty-seven patients referred a family history. We attributed idiopathic angioedema to 55.7% of patients, 24.3% were drug related, 15.7% were due to C1 inhibitor deficiency, 2.1% were drug related+idiopathic angioedema, 1.4% were type III and 0.7% had exercise-induced angioedema. Ninety six percent of 53 evaluable idiopathic angioedema patients referred a benefit with anti-histamine therapy. AWW was a rare cause of consultation. Most of our patients had anti H1 responsive idiopathic angioedema and none had allergic angioedema. Women cases prevailed over men's. Family history and average age of onset of symptoms were different among the different types of angioedema.

  7. Angioedema induced by cardiovascular drugs: new players join old friends.

    Science.gov (United States)

    Bas, M; Greve, J; Strassen, U; Khosravani, F; Hoffmann, T K; Kojda, G

    2015-10-01

    During the last years, two new cardiovascular drug classes, namely inhibitors of DPP IV or neprilysin, have been developed. In both cases, there is clinical evidence for their potential to induce angioedema as known already from blockers of the renin-angiotensin-aldosterone system (RAAS). The majority of angioedema induced by DPP IV inhibitors occurs during concomitant treatment with ACEi and is therefore likely mediated by overactivation of bradykinin type 2 receptors (B2). In striking contrast, the molecular pathways causing angioedema induced by neprilysin inhibitors, that is, sacubitril, are unclear, although a contribution of bradykinin appears likely. Nevertheless, there is no clinical evidence suggesting that inhibition of B2 might relieve the symptoms and/or prevent invasive treatment including coniotomy or tracheotomy in angioedema caused by these drugs. Therefore, the risk of angioedema should always be considered, especially in ambulatory care situations where patients have no rapid access to intensive care. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  8. Multicentre audit of ACE-inhibitor associated angioedema (MAAAA).

    Science.gov (United States)

    Gandhi, Jimit; Jones, Rachel; Teubner, David; Gabb, Genevieve

    2015-08-01

    Angiotensin-converting enzyme (ACE) inhibitors are a commonly used class of medications that are generally well tolerated. However, angioedema, a rare but potentially life-threatening adverse effect, may occur. A retrospective audit was performed on patients who presented with angioedema to two emergency departments in Adelaide, Australia. Case notes of patients presenting with angioedema who were using an ACE inhibitor were reviewed. This study examined the clinical features of presentation, treatment and outcomes of the patients. A total of 164 patients were identified as having angioedema across the two emergency departments. Fifty-one (31%; 95% CI = 24-39) were found to be on an ACE inhibitor. The two main presenting symptoms were soft tissue swelling in the head and neck (98%), and respiratory distress (33%), both of which usually developed after several hours. Patients were commonly treated with steroids (70%), antihistamines (65%) and adrenaline (35%). Two patients developed airway obstruction. A substantial proportion of emergency department encounters with angioedema in South Australia are from patients who also use an ACE inhibitor. It is important that general practitioners are aware of this problem, to enable rapid recognition and appropriate patient education when prescribing these medications.

  9. How Not to Be Misled by Disorders Mimicking Angioedema

    DEFF Research Database (Denmark)

    Andersen, Michelle Fog; Longhurst, Hilary J; Rasmussen, Eva Rye

    2016-01-01

    BACKGROUND: Angioedema is a vascular reaction involving the lower dermis, subcutis and/or submucosal tissue and causing a temporary localized swelling in any part of the body. For many health care professionals, the diagnosis presents an ongoing challenge; several disorders may manifest with subc......BACKGROUND: Angioedema is a vascular reaction involving the lower dermis, subcutis and/or submucosal tissue and causing a temporary localized swelling in any part of the body. For many health care professionals, the diagnosis presents an ongoing challenge; several disorders may manifest...... with subcutaneous or submucosal swelling and falsely be assumed to be angioedema. The clinicians at the emergency department and in the immunology/allergy clinics must be skilled at recognizing the features of angioedema and its differential diagnosis. METHODS: The review is based on a literature search...... with specific indexing terms in PubMed, a review of bibliographies and the authors' clinical experience. RESULTS: The most essential diseases that mimic angioedema, the so-called pseudoangioedemas, will each be discussed and illustrated by clinical photos, pointing out key features that help clarify...

  10. Heart Attack

    Science.gov (United States)

    ... pain Fatigue Heart attack Symptoms & causes Diagnosis & treatment Advertisement Mayo Clinic does not endorse companies or products. ... a Job Site Map About This Site Twitter Facebook Google YouTube Pinterest Mayo Clinic is a not- ...

  11. Assessment of 105 Patients with Angiotensin Converting Enzyme-Inhibitor Induced Angioedema

    DEFF Research Database (Denmark)

    Rasmussen, Eva Rye; von Buchwald, Christian; Wadelius, Mia

    2017-01-01

    Objective. To asses a cohort of 105 consecutive patients with angiotensin converting enzyme-inhibitor induced angioedema with regard to demographics, risk factors, family history of angioedema, hospitalization, airway management, outcome, and use of diagnostic codes used for the condition. Study...... Design. Cohort study. Methods. This was a retrospective cohort study of 105 patients with angiotensin converting enzyme-inhibitor induced angioedema in the period 1995-2014. Results. The cohort consisted of 67 females and 38 males (F : M ratio 1.8), with a mean age of 63 [range 26-86] years. Female...... gender was associated with a significantly higher risk of angiotensin converting enzyme-inhibitor induced angioedema. 6.7% had a positive family history of angioedema. Diabetes seemed to be a protective factor with regard to angioedema. 95% experienced angioedema of the head and neck. 4.7% needed...

  12. Prevalence of autoantibodies in a group of hereditary angioedema patients Prevalência de autoanticorpos em uma população com angioedema hereditário

    Directory of Open Access Journals (Sweden)

    Sergio Duarte Dortas Junior

    2012-04-01

    Full Text Available Hereditary Angioedema is a dominantly inherited disease. Routine screening of autoantibodies (AAB is not recommended for individuals with Hereditary Angioedema; however, prevalence of these antibodies in Hereditary Angioedema patients is not well documented. We aim to determine the prevalence of AAB so that individuals at risk of developing autoimmune diseases can be identified. Fifteen patients with Hereditary Angioedema attended at Clementino Fraga Filho University Hospital accepted to participate in this study. Prevalence of AAB was 40%. Our data indicate high prevalence of AAB in patients with Hereditary Angioedema. Large-scale studies should be considered to determine the significance of these AAB in the follow-up care of patients with Hereditary Angioedema.O Angioedema Hereditário é uma doença autossômica dominante. A pesquisa de rotina para autoanticorpos não é recomendada para pacientes com Angioedema Hereditário; entretanto, a prevalência desses anticorpos em pacientes com Angioedema Hereditário não está bem documentada. Objetivamos determinar a prevalência de autoanticorpos para identificar indivíduos sob risco de desenvolver doenças autoimunes. Quinze pacientes com Angioedema Hereditário atendidos no Hospital Universitário Clementino Fraga Filho aceitaram participar do estudo. A prevalência de autoanticorpos foi de 40%. Nossos dados indicam alta prevalência de autoanticorpos em pacientes com Angioedema Hereditário. Estudos de maior escala deveriam ser considerados para determinar a significância desses autoanticorpos no acompanhamento clínico de pacientes com Angioedema Hereditário.

  13. Floral organ abscission peptide IDA and its HAE/HSL2 receptors control cell separation during lateral root emergence.

    Science.gov (United States)

    Kumpf, Robert P; Shi, Chun-Lin; Larrieu, Antoine; Stø, Ida Myhrer; Butenko, Melinka A; Péret, Benjamin; Riiser, Even Sannes; Bennett, Malcolm J; Aalen, Reidunn B

    2013-03-26

    Throughout their life cycle, plants produce new organs, such as leaves, flowers, and lateral roots. Organs that have served their purpose may be shed after breakdown of primary cell walls between adjacent cell files at the site of detachment. In Arabidopsis, floral organs abscise after pollination, and this cell separation event is controlled by the peptide INFLORESCENCE DEFICIENT IN ABSCISSION (IDA), which signals through the leucine-rich repeat receptor-like kinases HAESA (HAE) and HAESA-LIKE2 (HSL2). Emergence of new lateral root primordia, initiated deep inside the root under the influence of auxin, is similarly dependent on cell wall dissolution between cells in the overlaying endodermal, cortical, and epidermal tissues. Here we show that this process requires IDA, HAE, and HSL2. Mutation in these genes constrains the passage of the growing lateral root primordia through the overlaying layers, resulting in altered shapes of the lateral root primordia and of the overlaying cells. The HAE and HSL2 receptors are redundant in function during floral organ abscission, but during lateral root emergence they are differentially involved in regulating cell wall remodeling genes. In the root, IDA is strongly auxin-inducible and dependent on key regulators of lateral root emergence--the auxin influx carrier LIKE AUX1-3 and AUXIN RESPONSE FACTOR7. The expression levels of the receptor genes are only transiently induced by auxin, suggesting they are limiting factors for cell separation. We conclude that elements of the same cell separation signaling module have been adapted to function in different developmental programs.

  14. Epidemiology of ACE Inhibitor Angioedema Utilizing a Large Electronic Health Record.

    Science.gov (United States)

    Banerji, Aleena; Blumenthal, Kimberly G; Lai, Kenneth H; Zhou, Li

    Angiotensin-converting enzyme inhibitors (ACEIs) are a common cause of drug-induced angioedema in the United States. Most epidemiologic ACEI angioedema data are from large multicenter clinical trials. The objective of this study was to identify the incidence of and risk factors for ACEI angioedema using a large integrated electronic health record (EHR). We conducted a retrospective cohort study of all ACEI prescriptions in the outpatient setting of a large academic center between January 1, 2000, and September 30, 2008. We determined frequency, timing, and risk factors for ACEI angioedema within 5 years of prescription. All data were derived from EHR sources, with angioedema defined by EHR reactions of angioedema, swelling, edema, or lip, eye, face, tongue, throat or mouth swelling. Among 134,945 patients prescribed an ACEI, 0.7% (n = 888) developed angioedema during the subsequent 5 years. Sex was similar but patients who developed ACEI angioedema were younger (61.5 vs 62.7 years, P = .007). Patients with ACEI angioedema were more likely to have a history of nonsteroidal anti-inflammatory drug allergy compared with patients who did not develop angioedema (7.1% vs 4.2%, P angioedema within 1 month of prescription and a 0.23% incidence during the first year. Incidence of angioedema was relatively constant annually over the subsequent 4 years (0.10% to 0.12%). The incidence of ACEI angioedema within a large EHR is consistent with large clinical trial data. We observed a persistent and relatively constant annual risk; however, angioedema risk factors and underlying genetic and pathophysiological mechanisms require further study. Copyright © 2017 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  15. Acute dystonic reaction leading to lingual hematoma mimicking angioedema

    Science.gov (United States)

    Sezer, Özgür; Aydin, Ali Attila; Bilge, Sedat; Arslan, Fatih; Arslan, Hasan

    2017-01-01

    Lingual hematoma is a severe situation, which is rare and endangers the airway. It can develop due to trauma, vascular abnormalities, and coagulopathy. Due to its sudden development, it can be clinically confused with angioedema. In patients who applied to the doctor with complaints of a swollen tongue, lingual hematoma can be confused with angioedema, in particular, at the beginning if the symptoms occurred after drug use. It should especially be considered that dystonia in the jaw can present as drug-induced hyperkinetic movement disorder. Early recognition of this rare clinical condition and taking precautions for providing airway patency are essential. In this case report, we will discuss mimicking angioedema and caused by a bite due to dystonia and separation of the tongue from the base of the mouth developing concurrently with lingual hematoma. PMID:29326495

  16. Angioedema after ovine hyaluronidase injection for treating hyaluronic acid overcorrection.

    Science.gov (United States)

    Andre, Pierre; Fléchet, Marie Laure

    2008-06-01

    Hyaluronic acid injections are becoming popular in aesthetic dermatology, and, sometimes, misplacements and very rarely adverse events have been reported. Hyaluronidase, an enzyme that hydrolyzes hyaluronic acid, is used to treat overcorrection or granulomatous reactions. Allergic reactions are well known except for how frequent they occur. This paper aims to confirm the efficacy of hyaluronidase injections to dissolve hyaluronic acid, but insists on the risk of hypersensitivity with animal-derived products. A case of angioedema due to ovine hyaluronidase is reported, and treatment is discussed. Hyaluronidase is highly effective, but skin test must be done before injection to avoid risk of angioedema and/or Quincke's edema.

  17. The Humanistic, Societal, and Pharmaco-economic Burden of Angioedema

    DEFF Research Database (Denmark)

    Longhurst, Hilary; Bygum, Anette

    2016-01-01

    on the psychological outcomes is scarce, but the limited information available suggests that access to specialist advice and treatment leads to psychological as well as physical improvement.HAE also has profound effects on individual and family economic output, directly via absenteeism from school or work...

  18. Development and validation of the angiotensin-converting enzyme inhibitor (ACEI) induced angioedema investigator rating scale and proposed discharge criteria.

    Science.gov (United States)

    Bonner, Nicola; Panter, Charlotte; Kimura, Alan; Sinert, Rich; Moellman, Joseph; Bernstein, Jonathan A

    2017-05-22

    The use of angiotensin-converting enzyme inhibitors (ACEI) has been associated with the development of bradykinin-mediated angioedema. With ever-widening indications for ACEI in diseases including hypertension, congestive heart failure and diabetic nephropathy, a concomitant increase in ACEI-Angioedema (ACEI-A) has been reported. At present there is no validated severity scoring or discharge criteria for ACEI-A. We sought to develop and validate an investigator rating scale with corresponding discharge criteria using clinicians experienced in treating ACEI-A. In-depth, 60-min qualitative telephone interviews were conducted with 12 US-based emergency physicians. Beforehand, clinicians were sent four case studies describing patients experiencing different severities of angioedema attacks. Clinicians were initially asked open-ended questions about their experience of patients' symptoms, treatment and discharge decisions. Clinicians then rated each patient case study and discussed patient diagnoses, ratings of symptom severity and discharge evaluation. The ratings were used to assess inter-rater reliability of the scale using the intra-class correlation coefficient (ICC) using IBM SPSS analysis Version 19 software. The findings provide support focusing on four key symptoms of airway compromise scored on a 0-4 scale: 1) Difficulty Breathing, 2) Difficulty Swallowing, 3) Voice Changes and 4) Tongue Swelling and the corresponding discharge criteria of a score of 0 or 'No symptoms' for Difficulty Breathing and Difficulty Swallowing and a score of 0 or 1 indicating mild or absence of symptoms for Voice Change and Tongue Swelling. Eleven clinicians agreed the absence of standardized discharge criteria supported the use of this scale. All physicians concurred with the recommended discharge criteria. The clinician ratings provided evidence of strong inter-rater reliability for the rating scale (ICC > 0.80). The investigator rating scale and discharge criteria are

  19. About Heart Attacks

    Science.gov (United States)

    ... Artery Disease Venous Thromboembolism Aortic Aneurysm More About Heart Attacks Updated:Jan 11,2018 A heart attack is ... coronary artery damage leads to a heart attack . Heart Attack Questions and Answers What is a heart attack? ...

  20. Heart Attack

    Science.gov (United States)

    ... Pressure, tightness, pain, or a squeezing or aching sensation in your chest or arms that may spread to your neck, jaw or back Nausea, indigestion, heartburn or abdominal pain Shortness of breath Cold sweat Fatigue Lightheadedness or sudden dizziness Heart attack ...

  1. Heart attack

    Science.gov (United States)

    ... part in support groups for people with heart disease . Outlook (Prognosis) After a heart attack, you have a higher ... P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine . 10th ed. Philadelphia, PA: Elsevier Saunders; 2014: ...

  2. Shark attack.

    Science.gov (United States)

    Guidera, K J; Ogden, J A; Highhouse, K; Pugh, L; Beatty, E

    1991-01-01

    Shark attacks are rare but devastating. This case had major injuries that included an open femoral fracture, massive hemorrhage, sciatic nerve laceration, and significant skin and muscle damage. The patient required 15 operative procedures, extensive physical therapy, and orthotic assistance. A review of the literature pertaining to shark bites is included.

  3. Urticaria and Angioedema – More than just Skin Deep!

    African Journals Online (AJOL)

    Hereditary or acquired deficiency, or functional impairment, of the C1 esterase enzyme should be also considered, especially if there is a family history of swelling, airway compromise or surgical complications indicating possible hereditary angioedema. .... Depression, functional and sleep impairment are common and it.

  4. a case report of suspected angioedema in a child after ...

    African Journals Online (AJOL)

    consecutive days.16 Common side effects include: nausea, vomiting while rare side effects include: rash, alopecia, urticaria and angioedema. Adverse effects appear to occur more frequently when higher doses are used.17. A compiled information from Food and Drug administration (FDA) and Facts Med users submissions.

  5. Urticaria and angioedema: a practical approach | Muller | South ...

    African Journals Online (AJOL)

    Urticaria (i.e., pruritic, raised wheals) and angioedema (i.e., deep mucocutaneous swelling) occur in up to 25 percent of the U.S. population. Vasoactive mediators released from mast cells and basophils produce the classic wheal and flare reaction. Diagnosis can be challenging, especially if symptoms are chronic or ...

  6. Fatal angioedema induced by angiotensin conversion enzyme (ACE ...

    African Journals Online (AJOL)

    ACE inhibitors are often prescribed in the treatment of hypertension, heart failure and kidney disease. These drugs are on the Essential Drugs List, and are therefore used at primary to tertiary health care levels in South Africa. Angioedema is considered a rare, but potentially fatal side-effect of this agent, with a reported ...

  7. Angiotensin converting enzyme induced angioedema: The need for ...

    African Journals Online (AJOL)

    Background: Angiotensin converting enzyme (ACE) inhibitor related angioneurotic edema or simply angioedema is a rare but common condition not well noticed in health facilities especially in developing countries. The complication can be life threatening with serious morbidity and mortality if not promptly diagnosed from ...

  8. Urticaria and angioedema – more than just skin deep! | Holtzhausen ...

    African Journals Online (AJOL)

    Urticaria and angioedema are characterized by pruritic hives and sometimes swelling of deeper mucocutaneous layers. Urticaria is caused by release of histamine and other mediators from mast cells. A cut-off of six weeks distinguishes acute and chronic forms, as these seem to differ regarding etiological and response ...

  9. A case report of suspected angioedema in a child after ...

    African Journals Online (AJOL)

    Children are at a higher risk of developing adverse drug reactions as they seldom express their own drug therapy experiences. Factors that have been implicated include polypharmacy ... Cutaneous drug allergy is a common manifestation of adverse drug reactions. Keywords: Angioedema, Mebendazole, Co-trimoxazole, ...

  10. Lactobacillus plantarum LG42 Isolated from Gajami Sik-Hae Inhibits Adipogenesis in 3T3-L1 Adipocyte

    Directory of Open Access Journals (Sweden)

    Jeong-Eun Park

    2013-01-01

    Full Text Available We investigated whether lactic acid bacteria isolated from gajami sik-hae (GLAB are capable of reducing the intracellular lipid accumulation by downregulating the expression of adipogenesis-related genes in differentiated 3T3-L1 cells. The GLAB, Lactobacillus plantarum LG42, significantly decreased the intracellular triglyceride storage and the glycerol-3-phosphate dehydrogenase (GPDH activity in a dose-dependent manner. mRNA expression of transcription factors like peroxisome proliferator-activated receptor (PPAR γ and CCAAT/enhancer-binding protein (C/EBP α involved in adipogenesis was markedly decreased by the GLAB treatment. Moreover, the GLAB also decreased the expression level of adipogenic markers like adipocyte fatty acid binding protein (aP2, leptin, GPDH, and fatty acid translocase (CD36 significantly. These results suggest that the GLAB inhibits lipid accumulation in the differentiated adipocyte through downregulating the expression of adipogenic transcription factors and other specific genes involved in lipid metabolism.

  11. Angiotensin-converting Enzyme Inhibitor Angioedema Requiring Admission to an Intensive Care Unit.

    Science.gov (United States)

    Soo Hoo, Guy W; Lin, Henry K; Junaid, Imran; Klaustermeyer, William B

    2015-07-01

    The purpose of this study was to review consecutive cases of angiotensin-converting enzyme (ACE) inhibitor angioedema admitted to an intensive care unit. Fifty subjects with ACE-inhibitor angioedema admitted from 1998-2011 were reviewed. All 50 subjects were men, 62.8 ± 8.4 years of age, 76% African Americans. Fifteen (30%) required ventilatory support and 2 (4%) required tracheostomy. Over half (56%) had taken ACE inhibitors for over a year. Logistic regression identified dyspnea and tongue involvement with the need for ventilatory support (P angioedema. Angioedema can occur even after extended use. Dyspnea and tongue involvement identified patients requiring ventilatory support. Published by Elsevier Inc.

  12. Angiotensin-converting Enzyme Inhibitor and Other Drug-associated Angioedema.

    Science.gov (United States)

    Stone, Cosby; Brown, Nancy J

    2017-08-01

    Nonsteroidal antiinflammatory agents, β-lactam antibiotics, non-β lactam antibiotics, and angiotensin-converting enzyme inhibitors are the most common classes of drugs that cause angioedema. Drug-induced angioedema is known to occur via mechanisms mediated by histamine, bradykinin, or leukotriene, and an understanding of these mechanisms is crucial in guiding therapeutic decisions. Nonallergic angioedema occurs in patients with genetic variants that affect metabolism or synthesis of bradykinin, substance P, prostaglandins, or leukotrienes, or when patients are taking drugs that have synergistic mechanisms. The mainstay in treatment of nonallergic drug-induced angioedema is cessation of the offending agents. Copyright © 2017 Elsevier Inc. All rights reserved.

  13. Optimum Use of Acute Treatments for Hereditary Angioedema: Evidence-Based Expert Consensus

    Science.gov (United States)

    Longhurst, Hilary

    2018-01-01

    Acute treatment of hereditary angioedema due to C1 inhibitor deficiency has become available in the last 10 years and has greatly improved patients’ quality of life. Two plasma-derived C1 inhibitors (Berinert and Cinryze), a recombinant C1 inhibitor (Ruconest/Conestat alpha), a kallikrein inhibitor (Ecallantide), and a bradykinin B2 receptor inhibitor (Icatibant) are all effective. Durably good response is maintained over repeated treatments and several years. All currently available prophylactic agents are associated with breakthrough attacks, therefore an acute treatment plan is essential for every patient. Experience has shown that higher doses of C1 inhibitor than previously recommended may be desirable, although only recombinant C1 inhibitor has been subject to full dose–response evaluation. Treatment of early symptoms of an attack, with any licensed therapy, results in milder symptoms, more rapid resolution and shorter duration of attack, compared with later treatment. All therapies have been shown to be well-tolerated, with low risk of serious adverse events. Plasma-derived C1 inhibitors have a reassuring safety record regarding lack of transmission of virus or other infection. Thrombosis has been reported in association with plasma-derived C1 inhibitor in some case series. Ruconest was associated with anaphylaxis in a single rabbit-allergic volunteer, but no further anaphylaxis has been reported in those not allergic to rabbits despite, in a few cases, prior IgE sensitization to rabbit or milk protein. Icatibant is associated with high incidence of local reactions but not with systemic effects. Ecallantide may cause anaphylactoid reactions and is given under supervision. For children and pregnant women, plasma-derived C1 inhibitor has the best evidence of safety and currently remains first-line treatment. PMID:29594115

  14. Angioedema triggered by pulp extirpation--a case report.

    Science.gov (United States)

    Baliga, Mohan; Ramanathan, Arvind; Bhambar, Rohan S

    2011-12-01

    C1-esterase inhibitor deficiency results in episodes of non-allergic edema of parts of the body. Edema of the face may be triggered by dental therapy. We report a case of C1-esterase inhibitor deficiency which was detected in a 42-year-old woman. The patient was completely unaware that she had this disorder or of any related family history, and the patient developed an intense facial angioedema after pulp extirpation of lower premolar tooth. In this case, the diagnosis of angioedema due to C1-esterase inhibitor deficiency was established at a later stage. The differing causes of C1-esterase inhibitor deficiency are briefly discussed and the treatment modalities outlined.

  15. Hereditary Angioedema: The Economics of Treatment of an Orphan Disease.

    Science.gov (United States)

    Lumry, William Raymond

    2018-01-01

    This review will discuss the cost burden of hereditary angioedema on patients, healthcare systems, and society. The impact of availability of and access to novel and specific therapies on morbidity, mortality, and the overall burden of disease will be explored along with potential changes in treatment paradigms to improve effectiveness and reduce cost of treatment. The prevalence of orphan diseases, legislative incentives to encourage development of orphan disease therapies and the impact of orphan disease treatment on healthcare payment systems will be discussed.

  16. Association between thyroid autoimmunity and recurrent angioedema in children.

    Science.gov (United States)

    Karagol, Hacer Ilbilge Ertoy; Yilmaz, Ozlem; Topal, Erdem; Bideci, Aysun; Bakirtas, Arzu

    2015-01-01

    The association between thyroid autoimmunity (TA) and idiopathic isolated angioedema (or angioedema without urticaria) has not been evaluated in either children or in adults up until now. We, therefore, aimed to investigate underlying or concomitant TA and/or autoimmune thyroid disease in children diagnosed with recurrent idiopathic angioedema. Children who were consecutively diagnosed with recurrent idiopathic histaminergic acquired angioedema (IH-AAE) between January 2011 and January 2014 constituted the case group. A standard diagnostic and therapeutic algorithm was applied to all the patients with recurrent IH-AAE. Thyroid autoantibodies and thyroid function tests were measured in all the patients with recurrent IH-AAE and in healthy control groups. Prophylaxis with an antihistamine was started for patients with frequently recurrent IH-AAE. Eighty consecutive children with recurrent IH-AAE (49 boys; median age, 8.3 years) and 80 healthy children (39 boys; median, 8 years) were enrolled in this prospective, case-control study (p > 0.05 for age and sex). The IH-AAE group was significantly different than the control group with respect to TA (13.7% versus 2.5%, respectively; p = 0.009) but was similar with respect to autoimmune thyroid disease (3.7% versus 0%, respectively; p = 0.2). The median follow-up of the recurrent IH-AAE group was 34 months (range, 12-45 months). Patients with recurrent IH-AAE with and those without TA were not different with respect to either the need or the duration of antihistamine prophylaxis (p > 0.05 for both). Recurrent IH-AAE may be related to or associated with TA and/or autoimmune thyroid diseases in some children. However, exploring to see whether this association is a causal link or just an epiphenomenon deserves further investigation and longer follow-ups.

  17. Colorectal intussusception: an unusual gastrointestinal complication of hereditary angioedema.

    Science.gov (United States)

    Witschi, A; Krähenbühl, L; Frei, E; Saltzman, J; Späth, P J; Müller, U R

    1996-09-01

    A 21-year-old man with a history of hereditary angioedema presented with protracted abdominal pain which failed to respond to infusion of C1 inhibitor concentrate. Evaluation by CT scan revealed extensive colorectal intussusception requiring surgical intervention. Under replacement therapy with C1 inhibitor concentrate, both the operation under general anesthesia and the postoperative phase were uneventful. The intraoperative examination suggested initiation of intussusception by local mucosal edema in the transverse colon.

  18. [Exercise-induced urticaria and angioedema - case report].

    Science.gov (United States)

    Stelmach, Iwona; Sztafińska, Anna; Lechańka, Joanna; Balcerak, Joanna; Jerzyńska, Joanna

    2014-01-01

    Urticaria is a heterogeneous group of disorders, with various clinical manifestations and intensity of symptoms. Urticaria can be induced with a wide variety of environmental stimuli, such as cold, pressure, vibration, sunlight, exercise, temperature changes, heat, and water. In a select group of patients, exercise can induce a spectrum of urticaria symptoms, ranging from cutaneous pruritus and warmth, generalised urticaria, angioedema, and the appearance of such additional manifestations as collapse, upper respiratory distress, and anaphylaxis. Specific provocation tests should be carried out on an individual basis to investigate the suspected cause and proper diagnosis. Modification of activities and behaviour is the mainstay of treatment in patients with physical urticaria. The aim of this study was to emphasise that primary care paediatricians should be able to recognise physical urticaria, supply a patient with rescue medications, and refer him/her to a specialist. In the article, the authors present a 13-year-old girl with typical urticaria lesions and angioedema after exercise. According to the history, physical examination, and provocation test, exercise-induced urticaria and angioedema were diagnosed.

  19. Diagnosis and Treatment of Urticaria and Angioedema: A Worldwide Perspective

    Science.gov (United States)

    2012-01-01

    Urticaria and angioedema are common clinical conditions representing a major concern for physicians and patients alike. The World Allergy Organization (WAO), recognizing the importance of these diseases, has contributed to previous guidelines for the diagnosis and management of urticaria. The Scientific and Clinical Issues Council of WAO proposed the development of this global Position Paper to further enhance the clinical management of these disorders through the participation of renowned experts from all WAO regions of the world. Sections on definition and classification, prevalence, etiology and pathogenesis, diagnosis, treatment, and prognosis are based on the best scientific evidence presently available. Additional sections devoted to urticaria and angioedema in children and pregnant women, quality of life and patient-reported outcomes, and physical urticarias have been incorporated into this document. It is expected that this article will supplement recent international guidelines with the contribution of an expert panel designated by the WAO, increasing awareness of the importance of urticaria and angioedema in medical practice and will become a useful source of information for optimum patient management worldwide. PMID:23282382

  20. Angioedema recorrente – caso clínico

    Directory of Open Access Journals (Sweden)

    Sandrina Martins

    2016-02-01

    Full Text Available Introdução: O Angioedema hereditário (AEH é uma causa rara de angioedema recorrente, resultante de um defeito a nível do gene que codifica o inibidor do C1 esterase (C1 -INH. O edema envolve predominantemente os tecidos da face, membros, trato gastrointestinal e área genital. O envolvimento da laringe, apesar de menos frequente, constitui a expressão clínica mais grave, sendo potencialmente fatal. Caso clínico: Descreve -se o caso clínico de uma criança do sexo feminino de oito anos de idade referenciada à consulta de pediatria por episódios recorrentes de angioedema. O estudo efetuado revelou tratar -se de um caso de AEH. Discussão: O diagnóstico, estabelecido com base no quadro clínico, estudo do complemento e história familiar, é de importância fundamental considerando que o AEH é potencialmente fatal e exige uma terapêutica específica.

  1. Pharmacogenetics of ACE inhibitor-induced angioedema and cough: a systematic review and meta-analysis

    NARCIS (Netherlands)

    Mahmoudpour, Seyed Hamidreza; Leusink, Maarten; van der Putten, Lisa; Terreehorst, Ingrid; Asselbergs, Folkert W.; de Boer, Anthonius; Maitland-van der Zee, Anke H.

    2013-01-01

    Angioedema and cough are the two most important adverse effects of ACE inhibitors (ACEIs). Evidence exists that ACEI-related angioedema/cough is partly genetically determined and several genes have been identified to play a role in the development of ACEI-related adverse effects. This study was

  2. Non-myeloablative allogeneic stem cell transplantation: a new treatment option for acquired angioedema?

    NARCIS (Netherlands)

    Zegers, I.H.A.; Aaldering, K.N.; Nieuwhof, C.M.; Schouten, H.C.

    2015-01-01

    INTRODUCTION: Acquired angioedema is a rare disorder causing recurrent life-threatening angioedema, due to decreased activity of C1 esterase inhibitor. CASE REPORT: A 57-year-old man presented to our hospital with recurrent swelling of the hands, lips, tongue, scrotum and throat. Lab examination

  3. Assessment of 105 Patients with Angiotensin Converting Enzyme-Inhibitor Induced Angioedema

    Science.gov (United States)

    von Buchwald, Christian; Prasad, Sumangali Chandra; Kamaleswaran, Shailajah; Ajgeiy, Kawa Khaled; Authried, Georg; Pallesen, Kristine Appel U.

    2017-01-01

    Objective. To asses a cohort of 105 consecutive patients with angiotensin converting enzyme-inhibitor induced angioedema with regard to demographics, risk factors, family history of angioedema, hospitalization, airway management, outcome, and use of diagnostic codes used for the condition. Study Design. Cohort study. Methods. This was a retrospective cohort study of 105 patients with angiotensin converting enzyme-inhibitor induced angioedema in the period 1995–2014. Results. The cohort consisted of 67 females and 38 males (F : M ratio 1.8), with a mean age of 63 [range 26–86] years. Female gender was associated with a significantly higher risk of angiotensin converting enzyme-inhibitor induced angioedema. 6.7% had a positive family history of angioedema. Diabetes seemed to be a protective factor with regard to angioedema. 95% experienced angioedema of the head and neck. 4.7% needed intubation or tracheostomy. 74 admissions took place during the study period with a total of 143 days spent in the hospital. The diagnosis codes most often used for this condition were “DT783 Quincke's oedema” and “DT78.4 Allergy unspecified”. Complement C1 inhibitor was normal in all tested patients. Conclusion. Female gender predisposes to angiotensin converting enzyme-inhibitor induced angioedema, whereas diabetes seems to be a protective factor. PMID:28286522

  4. Clinical characteristics and treatment of chronic urticaria and angioedema : Dwelling on swelling, dealing with whealing

    NARCIS (Netherlands)

    van den Elzen, M.T.

    2017-01-01

    The occurrence of wheals, angioedema or both for at least 6 weeks is diagnosed as chronic spontaneous urticaria in (inter) national guidelines - after excluding other illnesses. The underlying mechanism of angioedema without wheals is not entirely known. The objective of this thesis is to increase

  5. Dipeptidyl peptidase-4 inhibitor induced angioedema - an overlooked and potentially lethal adverse drug reaction?

    DEFF Research Database (Denmark)

    Scott, Susanne Irene; Andersen, Michelle Fog; Aagaard, Lise

    2018-01-01

    Introduction Angioedema is a potentially fatal adverse drug reaction of some medications, as swellings of the upper airways can cause death by asphyxiation. Angiotensin converting enzyme-inhibitors are widely known to cause angioedema but less is known about the association between dipeptidyl...

  6. Burden of Illness and Quality-of-Life Measures in Angioedema Conditions.

    Science.gov (United States)

    Caballero, Teresa; Prior, Nieves

    2017-08-01

    Burden of illness studies and evaluation of health-related quality of life using validated questionnaires have become an important task in the comprehensive management of angioedema conditions, mainly angioedema associated with chronic spontaneous urticaria and hereditary angioedema caused by C1-inhibitor deficiency. A review of the principal tools and studies is presented. Both diseases present a higher proportion of psychiatric disorders, impair work and studies productivity, and produce high direct and indirect costs. These assessments also have been useful to evaluate the positive impact of new drugs and interventions. More studies are desirable, especially in other types of angioedema disorders, such as hereditary angioedema with normal C1 inhibitor. Copyright © 2017 Elsevier Inc. All rights reserved.

  7. [Angioedema and the role of bradykinins: new treatments and implications in patients with heart failure].

    Science.gov (United States)

    Mansi, Marta; Wu, Maddalena Alessandra; Zanichelli, Andrea; Cicardi, Marco

    2016-12-01

    The definition of angioedema is an edema of subcutaneous and submucosal tissues due to increased vascular permeability and fluid extravasation. It can affect different areas, including extremities, genitals, upper airways and intestinal mucosa. The symptoms are disabling and this condition can be fatal if it involves the larynx. We can distinguish different forms of angioedema (hereditary and acquired) with different pathogenetic mechanisms, therefore responding to different treatments. Bradykinin-mediated angioedema (such as hereditary angioedema due to C1-inhibitor deficiency) does not respond to the standard therapy used for histamine-mediated angioedema. These forms should be immediately recognized and specific treatment should be used. In addition, when a patient manifests hypotension not responding to fluid replacement and associated with diffuse edema, hypoalbuminemia and hemoconcentration, we should consider the diagnosis of idiopathic systemic capillary leak syndrome, a very rare but fatal condition.

  8. Angioedema in progressive muscular dystrophy: a case report.

    Science.gov (United States)

    Dermendzhiev, Svetlan M; Simeonova, Radostina; Murdjeva, Marianna A

    2012-01-01

    Systemic allergic reactions, which include angioedema, are very common in clinical practice. There is great diversity in the etiological factors known to trigger angioedema, and in the pathogenetic mechanisms defining this condition. Beside the broad spectrum of immuno-allergic reactions involved in the angioedemic pathogenesis, this condition is known to also develop on the background of other disorders. These disorders may be of different etiology and have different pathogenesis (either non-immune or immune) but have one common feature referred to as "serological overlap". From research and clinical perspective, it is interesting to explore the combination of some rare neurological diseases, such as myopathies and in particular muscular dystrophies with systemic allergic reactions such as angioedema, urticaria and others. It is known that progressive muscular dystrophies (PMD) are hereditary diseases with different types of inheritance--X-chromosome recessive, X-chromosome dominant, autosomal dominant and others. In some forms, such as Duchenne muscular dystrophy (DMD), an increased expression of perforin in muscle is found which is evidence for involvement of the cellular immune response in the pathogenesis of myopathy. It is in this sense that it is interesting to explore and discuss a clinical case diagnosed as a facioscapulohumeral form of PMD, which also manifests angioedema with urticaria. We present a 41-year-old male hospitalized in the Division of Occupational Diseases and Allergology at St. George University Hospital in Plovdiv who suffered two incidents of massive angioedema on the face, back and chest, accompanied by an itchy urticarial rash. In 1985, after hospitalization to the Clinic of Neurology, he was diagnosed with PMD of facioscapulohumeral type. The medical history could not reveal any of the most common etiologic factors such as drugs, food, insects and other allergens that may be associated with the systemic allergic reactions. The

  9. [Prophylactic use of icatibant before tracheal intubation of a patient with hereditary angioedema type III. (A literature review of perioperative management of patients with hereditary angioedema type III)].

    Science.gov (United States)

    Iturri Clavero, F; González Uriarte, A; Tamayo Medel, G; Gamboa Setién, P M

    2014-01-01

    Type III hereditary angioedema is a rare familial disorder that has recently been described as a separate condition. Triggers for episodes of angioedema include surgery, dental procedures, and tracheal intubation maneuvers. Since episodes affecting the upper airway are potentially life-threatening, prophylactic treatment is recommended in these situations. The use of icatibant (Firazyr(®)), for prevention of angioedema prior to tracheal intubation, is reported in a patient with type iii hereditary angioedema. A literature review on the anesthetic management of this condition was conducted. Copyright © 2013 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Published by Elsevier España. All rights reserved.

  10. Pollen count and presentation of angiotensin-converting enzyme inhibitor-associated angioedema.

    Science.gov (United States)

    Straka, Brittany; Nian, Hui; Sloan, Chantel; Byrd, James Brian; Woodard-Grice, Alencia; Yu, Chang; Stone, Elizabeth; Steven, Gary; Hartert, Tina; Teo, Koon K; Pare, Guillaume; McCarty, Catherine A; Brown, Nancy J

    2013-01-01

    The incidence of angiotensin-converting enzyme (ACE) inhibitor-associated angioedema is increased in patients with seasonal allergies. We tested the hypothesis that patients with ACE inhibitor-associated angioedema present during months when pollen counts are increased. Cohort analysis examined the month of presentation of ACE inhibitor-associated angioedema and pollen counts in the ambulatory and hospital setting. Patients with ACE inhibitor-associated angioedema were ascertained through (1) an observational study of patients presenting to Vanderbilt University Medical Center, (2) patients presenting to the Marshfield Clinic and participating in the Marshfield Clinic Personalized Medicine Research Project, and (3) patients enrolled in The Ongoing Telmisartan Alone and in Combination with Ramipril Global Endpoint Trial (ONTARGET). Measurements include date of presentation of ACE inhibitor-associated angioedema, population exposure to ACE inhibitor by date, and local pollen counts by date. At Vanderbilt, the rate of angioedema was significantly associated with tree pollen months (P = .01 from χ(2) test). When separate analyses were conducted in patients with a history of seasonal allergies and patients without, the rate of ACE inhibitor-associated angioedema was increased during tree pollen months only in patients with a history of seasonal allergies (P = .002). In Marshfield, the rate of angioedema was significantly associated with ragweed pollen months (P = .025). In ONTARGET, a positive trend was observed between the ACE inhibitor-associated angioedema rate and grass season, although it was not statistically significant (P = .057). Patients with ACE inhibitor-associated angioedema are more likely to present with this adverse drug event during months when pollen counts are increased. Copyright © 2013 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  11. Kininogen Cleavage Assay: Diagnostic Assistance for Kinin-Mediated Angioedema Conditions

    Science.gov (United States)

    Defendi, Federica; Charignon, Delphine; Ghannam, Arije; Habib, Mohammed; Drouet, Christian

    2016-01-01

    Background Angioedema without wheals (AE) is a symptom characterised by localised episodes of oedema presumably caused by kinin release from kininogen cleavage. It can result from a hereditary deficiency in C1 Inhibitor (C1Inh), but it can present with normal level of C1Inh. These forms are typically difficult to diagnose although enhanced kinin production is suspected or demonstrated in some cases. Objectives We wanted to investigate bradykinin overproduction in all AE condition with normal C1Inh, excluding cases with enhanced kinin catabolism, and to propose this parameter as a disease biomarker. Methods We retrospectively investigated high molecular weight kininogen (HK) cleavage pattern, using gel electrophoresis and immunorevelation. Plasma samples were drawn using the same standardised procedure from blood donors or AE patients with normal C1Inh conditions, normal kinin catabolism, and without prophylaxis. Results Circulating native HK plasma concentrations were similar in the healthy men (interquartile range: 98–175μg/mL, n = 51) and in healthy women (90–176μg/mL, n = 74), while HK cleavage was lower (p14.4% HK cleavage for men; 33.0% HK cleavage for women, with >98% specificity achieved for all parameters. In plasma from patients undergoing recovery two months after oestrogen/progestin combination withdrawal (n = 13) or two weeks after AE attack (n = 2), HK cleavage was not fully restored, suggesting its use as a post-attack assay. Conclusion As a diagnostic tool, HK cleavage can offer physicians supportive arguments for kinin production in suspected AE cases and improve patient follow-up in clinical trials or prophylactic management. PMID:27685806

  12. Kininogen Cleavage Assay: Diagnostic Assistance for Kinin-Mediated Angioedema Conditions.

    Directory of Open Access Journals (Sweden)

    Rémi Baroso

    Full Text Available Angioedema without wheals (AE is a symptom characterised by localised episodes of oedema presumably caused by kinin release from kininogen cleavage. It can result from a hereditary deficiency in C1 Inhibitor (C1Inh, but it can present with normal level of C1Inh. These forms are typically difficult to diagnose although enhanced kinin production is suspected or demonstrated in some cases.We wanted to investigate bradykinin overproduction in all AE condition with normal C1Inh, excluding cases with enhanced kinin catabolism, and to propose this parameter as a disease biomarker.We retrospectively investigated high molecular weight kininogen (HK cleavage pattern, using gel electrophoresis and immunorevelation. Plasma samples were drawn using the same standardised procedure from blood donors or AE patients with normal C1Inh conditions, normal kinin catabolism, and without prophylaxis.Circulating native HK plasma concentrations were similar in the healthy men (interquartile range: 98-175μg/mL, n = 51 and in healthy women (90-176μg/mL, n = 74, while HK cleavage was lower (p14.4% HK cleavage for men; 33.0% HK cleavage for women, with >98% specificity achieved for all parameters. In plasma from patients undergoing recovery two months after oestrogen/progestin combination withdrawal (n = 13 or two weeks after AE attack (n = 2, HK cleavage was not fully restored, suggesting its use as a post-attack assay.As a diagnostic tool, HK cleavage can offer physicians supportive arguments for kinin production in suspected AE cases and improve patient follow-up in clinical trials or prophylactic management.

  13. How Not to Be Misled by Disorders Mimicking Angioedema: A Review of Pseudoangioedema.

    Science.gov (United States)

    Andersen, Michelle Fog; Longhurst, Hilary J; Rasmussen, Eva Rye; Bygum, Anette

    2016-01-01

    Angioedema is a vascular reaction involving the lower dermis, subcutis and/or submucosal tissue and causing a temporary localized swelling in any part of the body. For many health care professionals, the diagnosis presents an ongoing challenge; several disorders may manifest with subcutaneous or submucosal swelling and falsely be assumed to be angioedema. The clinicians at the emergency department and in the immunology/allergy clinics must be skilled at recognizing the features of angioedema and its differential diagnosis. The review is based on a literature search with specific indexing terms in PubMed, a review of bibliographies and the authors' clinical experience. The most essential diseases that mimic angioedema, the so-called pseudoangioedemas, will each be discussed and illustrated by clinical photos, pointing out key features that help clarify the diagnoses and differentiate these from classic angioedema. A variety of dermatologic conditions can cause swelling that resembles angioedema, some with a potentially fatal outcome if misdiagnosed. Knowledge of pseudoangioedema is fundamental in the emergency setting when handling patients with edema and should be kept in mind when assessing an atypical angioedema case. © 2016 S. Karger AG, Basel.

  14. Current and future therapies for the treatment of histamine-induced angioedema.

    Science.gov (United States)

    James, Christine; Bernstein, Jonathan A

    2017-02-01

    Angioedema, a sudden, self-limited swelling of localized areas of any part of the body that may or may not be associated with urticaria, is thought to be the result of a mast-cell mediated process versus a bradykinin etiology. Understanding the mechanism is key in determining the proper treatment. Areas Covered: Clinical presentation of varying angioedema types may be similar; however, the appropriate treatment algorithm is dependent upon clinicians' knowledge of the underlying pathophysiology and classification of angioedema. Literature review of recent guidelines, available medications, and alternative therapies was completed to provide an overview of options. There are no formal guidelines for treatment of acute or chronic histamine-mediated angioedema, and therefore, algorithms for the treatment of acute and chronic urticaria should be followed until such information becomes available. Differentiating histamine-mediated versus bradykinin mediated angioedema is essential, as treatments and treatment responses are quite different. Further research is needed to better understand idiopathic angioedema that is unresponsive to H1/H2 antagonists, LTMAs, or medications designed to treat bradykinin-mediated angioedema.

  15. Angioedema Spotlight: A Closer Examination of Sacubitril/Valsartan Safety Results.

    Science.gov (United States)

    Owens, Ryan E; Oliphant, Carrie S

    2017-01-01

    Incorporation of neprilysin inhibition into heart failure pharmacotherapy regimens has recently been recommended by U.S. guidelines, based on results from the PARADIGM-HF trial comparing sacubitril/valsartan to enalapril. While most of the discussion has focused on efficacy, a closer examination of the safety results, particularly the incidence of angioedema during the run-in and double-blind periods, is also warranted. Although no major safety concerns were identified, an angioedema risk comparable to enalapril was found, primarily in the black population. Therefore, despite combination with an angiotensin receptor blocker, which historically has a lower incidence of angioedema, the addition of neprilysin inhibition yields an angioedema risk profile comparable to angiotensin converting enzyme (ACE) inhibitors. Clinicians should recognize this safety risk when prescribing sacubitril/valsartan and remain vigilant in counseling patients regarding the signs and symptoms of angioedema. As recommended by the guidelines, avoiding sacubitril/valsartan use concurrently or within 36 hours of the last dose of an ACE inhibitor or in patients with a history of angioedema is also crucial to minimize angioedema risk and prevent patient harm. © Copyright 2017 by the American Board of Family Medicine.

  16. Genetic variants associated with angiotensin-converting enzyme inhibitor-associated angioedema

    Science.gov (United States)

    Pare, Guillaume; Kubo, Michiaki; Byrd, James B.; McCarty, Catherine A.; Woodard-Grice, Alencia; Teo, Koon K.; Anand, Sonia S.; Zuvich, Rebecca L.; Bradford, Yuki; Ross, Stephanie; Nakamura, Yusuke; Ritchie, Marylyn; Brown, Nancy J.

    2013-01-01

    Objective The objective of this study was to identify genetic variants associated with angiotensin-converting enzyme (ACE) inhibitor-associated angioedema. Participants and methods We carried out a genome-wide association study in 175 individuals with ACE inhibitor-associated angioedema and 489 ACE inhibitor-exposed controls from Nashville (Tennessee) and Marshfield (Wisconsin). We tested for replication in 19 cases and 57 controls who participated in Ongoing Telmisartan Alone and in Combination with Ramipril Global Endpoint Trial (ONTARGET). Results There were no genome-wide significant associations of any single-nucleotide polymorphism (SNP) with angioedema. Sixteen SNPs in African Americans and 41 SNPs in European Americans were associated moderately with angioedema (Pangioedema in the Nashville/Marshfield sample and ONTARGET. In a candidate gene analysis, rs989692 in the gene encoding neprilysin (MME), an enzyme that degrades bradykinin and substance P, was significantly associated with angioedema in ONTARGET and Nashville/Marshfield African Americans. Conclusion Unlike other serious adverse drug effects, ACE inhibitor-associated angioedema is not associated with a variant with a large effect size. Variants in MME and genes involved in immune regulation may be associated with ACE inhibitor-associated angioedema. PMID:23838604

  17. Treatment of angiotensin receptor blocker-induced angioedema: A case series.

    Science.gov (United States)

    Strassen, Ulrich; Bas, Murat; Hoffmann, Thomas K; Knopf, Andreas; Greve, Jens

    2015-07-01

    Angiotensin II receptor antagonists have been proposed as a replacement therapy after the occurrence of either an angiotensin converting enzyme (ACE) inhibitor-induced angioedema or cough. However, recent studies indicate that angioedema is associated with elevated bradykinin levels in a small fraction of patients treated with angiotensin-II-receptor blockers, suggesting a common pathophysiological mechanism. To date, a standard treatment for angiotensin II receptor blocker-induced angioedema does not exist. We present a case series of patients admitted to our hospital due to angioedema induced by an angiotensin II receptor blocker. The patients were either treated with either icatibant (n = 3) or prednisolone-21-hydrogen succinate/clemastine (n = 5). Both patient groups were compared with an untreated patient cohort (n = 3). All patients were previously diagnosed with essential hypertonia. Icatibant was an effective therapy for angiotensin II receptor blocker-induced angioedema. Full symptom recovery was achieved after 5 to 7 hours, whereas symptom remission occurred within 27 to 52 and 24 to 54 hours in patients treated with Solu-Decortin prednisolone/clemastine and untreated patients, respectively. The recovery time for icatibant was similar to that described in previous studies regarding the therapeutic efficacy of icatibant for the treatment of hereditary angioedema and patients suffering from angiotensin converting enzyme inhibitor-induced angioedema. Icatibant is a safe and effective substance for the treatment of angiotensin II receptor blocker-induced angioedema. Although the pathophysiology of angiotensin II receptor blocker-induced angioedema remains unclear, it appears to be associated with the bradykinin pathway. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

  18. Hereditary Angioedema: The Economics of Treatment of an Orphan Disease

    Directory of Open Access Journals (Sweden)

    William Raymond Lumry

    2018-02-01

    Full Text Available This review will discuss the cost burden of hereditary angioedema on patients, healthcare systems, and society. The impact of availability of and access to novel and specific therapies on morbidity, mortality, and the overall burden of disease will be explored along with potential changes in treatment paradigms to improve effectiveness and reduce cost of treatment. The prevalence of orphan diseases, legislative incentives to encourage development of orphan disease therapies and the impact of orphan disease treatment on healthcare payment systems will be discussed.

  19. Psychometric Field Study of Hereditary Angioedema Quality of Life Questionnaire for Adults

    DEFF Research Database (Denmark)

    Prior, Nieves; Remor, Eduardo; Pérez-Fernández, Elia

    2016-01-01

    was to perform a cross-cultural validation and psychometric study of the HAE-QoL in an international setting. METHODS: Cross-cultural adaptation of the Spanish HAE-QoL draft version and an international rating phase with experts were performed. The resultant version of the HAE-QoL, a clinical questionnaire......, and Short Form 36-item Health Survey Version 2.0 (SF-36v2) were pilot tested internationally. Item reduction was based on both descriptive and exploratory factor analysis. Psychometric properties were assessed. RESULTS: Cross-cultural adaptation of the HAE-QoL was performed in 18 countries. The draft...... and social functioning, concern about offspring, perceived control over illness, and mental health). Strong psychometric properties were observed (Cronbach's α 0.92; test-retest reliability 0.87). Convergent validity showed mild to moderate correlations with SF-36v2 physical and mental component summaries (0...

  20. Fibronectin gene polymorphisms and clinical manifestations of mixed cryoglobulinemic syndrome: increased risk of lymphoma associated to MspI DD and HaeIII AA genotypes

    Directory of Open Access Journals (Sweden)

    C. Fabro

    2011-09-01

    Full Text Available Objective: To analyse FN gene polymorphisms in type II mixed cryoglobulinemic syndrome (MCsn, an immune-complex mediated systemic vasculitis linked to hepatitis C virus (HCV infection and characterized by rheumatoid factor (RF positive B-cell proliferation at high risk for the progression into non Hodgkin’s lymphoma (NHL. Methods: Samples from eighty-one patients, with MCsn (type II serum cryoglobulins and clinical signs of vasculitis were studied. Sixthy-five (65/81, 80.3% patients were HCV-positive. Twenty-one (25.9% patients had developed a B-cell NHL during the course of MCsn. Seventy-two patients with HCV-negative and MC-unrelated NHL and 110 healthy blood donors (HBDs were taken as controls. HaeIIIb and MspI FN gene polymorphisms were analysed by PCR and specific restriction enzyme digestions, following reported procedures. Plasma FN levels were analysed by ELISA, whenever possible. Results: HaeIIIb and MspI allele and genotype frequencies did not differ between MCsn patients and HBDs. Of note, the DD-MspI (OR=5.56; CI=1.67-18.51, p=0.0046 and the AA-HaeIIIb (OR=5.54; CI=1.64-18.76, p=0.0066 homozygosis appeared significantly and independently associated with the development of B-cell NHL in MCsn patients, with the HaeIIIb A allele possibly conferring an increased risk of NHL in the general population (OR=1.72, CI=1.128- 2.635, p=0.0133. In contrast, the major vasculitic manifestations, such as peripheral neuropathy, skin ulcers and glomerulonephritis tended to be associated with the counterpart MspI C allele. No association between FN plasma levels and FN genotypes was found. Conclusion: Genotyping for MspI and HaeIIIb FN gene polymorphisms may be clinically relevant to define the predisposition to the major clinical manifestations in MCsn.

  1. Angioedema: Classification, management and emerging therapies for the perioperative physician

    Science.gov (United States)

    Misra, Lopa; Khurmi, Narjeet; Trentman, Terrence L

    2016-01-01

    Angioedema is a rare condition which manifests as sudden localised, non-pitting swelling of certain body parts including skin and mucous membranes. It is vital that anaesthesiologists understand this condition, as it may present suddenly in the perioperative period with airway compromise. To identify literature for this review, the authors searched the PubMed, Medline, Embase, Scopus and Web of Science databases for English language articles covering a 10-year period, 2006 through 2016. Angioedema can be either mast-cell mediated or bradykinin-induced. Older therapies for histaminergic symptoms are well known to anaesthesiologists (e.g., adrenaline, anti-histamines and steroids), whereas older therapies for bradykinin-induced symptoms include plasma and attenuated androgens. New classes of drugs for bradykinin-induced symptoms are now available, including anti-bradykinin, plasma kallikrein inhibitor and C1 esterase inhibitors. These can be used prophylactically or as rescue medications. Anaesthesiologists are in a unique position to coordinate perioperative care for this complex group of patients. PMID:27601734

  2. Angioedema: Classification, management and emerging therapies for the perioperative physician

    Directory of Open Access Journals (Sweden)

    Lopa Misra

    2016-01-01

    Full Text Available Angioedema is a rare condition which manifests as sudden localised, non-pitting swelling of certain body parts including skin and mucous membranes. It is vital that anaesthesiologists understand this condition, as it may present suddenly in the perioperative period with airway compromise. To identify literature for this review, the authors searched the PubMed, Medline, Embase, Scopus and Web of Science databases for English language articles covering a 10-year period, 2006 through 2016. Angioedema can be either mast-cell mediated or bradykinin-induced. Older therapies for histaminergic symptoms are well known to anaesthesiologists (e.g., adrenaline, anti-histamines and steroids, whereas older therapies for bradykinin-induced symptoms include plasma and attenuated androgens. New classes of drugs for bradykinin-induced symptoms are now available, including anti-bradykinin, plasma kallikrein inhibitor and C1 esterase inhibitors. These can be used prophylactically or as rescue medications. Anaesthesiologists are in a unique position to coordinate perioperative care for this complex group of patients.

  3. Identification and characterization of CbeI, a novel thermostable restriction enzyme from Caldicellulosiruptor bescii DSM 6725 and a member of a new subfamily of HaeIII-like enzymes.

    Science.gov (United States)

    Chung, Dae-Hwan; Huddleston, Jennifer R; Farkas, Joel; Westpheling, Janet

    2011-11-01

    Potent HaeIII-like DNA restriction activity was detected in cell-free extracts of Caldicellulosiruptor bescii DSM 6725 using plasmid DNA isolated from Escherichia coli as substrate. Incubation of the plasmid DNA in vitro with HaeIII methyltransferase protected it from cleavage by HaeIII nuclease as well as cell-free extracts of C. bescii. The gene encoding the putative restriction enzyme was cloned and expressed in E. coli with a His-tag at the C-terminus. The purified protein was 38 kDa as predicted by the 981-bp nucleic acid sequence, was optimally active at temperatures between 75°C and 85°C, and was stable for more than 1 week when stored at 35°C. The cleavage sequence was determined to be 5'-GG/CC-3', indicating that CbeI is an isoschizomer of HaeIII. A search of the C. bescii genome sequence revealed the presence of both a HaeIII-like restriction endonuclease (Athe 2438) and DNA methyltransferase (Athe 2437). Preliminary analysis of other Caldicellulosiruptor species suggested that this restriction/modification activity is widespread in this genus. A phylogenetic analysis based on sequence alignment and conserved motif searches identified features of CbeI distinct from other members of this group and classified CbeI as a member of a novel subfamily of HaeIII-like enzymes.

  4. Amitriptyline and bromazepam in the treatment of vibratory angioedema: which role for neuroinflammation?

    Science.gov (United States)

    Guarneri, Fabrizio; Guarneri, Claudio; Marini, Herbert Ryan

    2014-01-01

    Vibratory angioedema is a rare form of physical urticaria, hereditary or acquired, which occurs at body sites exposed to vibrations. Pathogenic mechanisms of disease are not completely clear and, consequently, current pharmacological treatment is sometimes unsatisfactory. We report the case of a horn player affected by acquired vibratory angioedema, relapsing after prolonged use of the instrument and resistant to systemic antihistamines and corticosteroids, which successfully responded to therapy with low doses of amitriptyline and bromazepam. A neuroinflammatory mechanism can be likely implicated in the pathogenesis of vibratory angioedema, in line with many different cutaneous/mucosal diseases involving a complex interplay of homeostatic/allostatic systems. Furthermore, in mucosal diseases, such as vibratory angioedema, physical/psychological stressors have a relevant role. In such cases, because of the complex interplay between nervous and immune system, the pharmacological activity of benzodiazepines and typical antidepressants may downregulate neuroinflammation. © 2014 Wiley Periodicals, Inc.

  5. Bowel Angioedema Associated With Iodinated Contrast Media: Incidence and Predisposing Factors.

    Science.gov (United States)

    Seo, Nieun; Chung, Yong Eun; Lim, Joon Seok; Song, Mi Kyung; Kim, Myeong-Jin; Kim, Ki Whang

    2017-09-01

    Bowel angioedema is an acute adverse reaction to iodinated contrast media (CM) that involves the gastrointestinal tract. We aimed to investigate the incidence and predisposing factors of iodinated CM-associated bowel angioedema during computed tomography (CT) examinations. This study was approved by our institutional review board, and informed consent was waived due to its retrospective design. From July 2013 to July 2015, adult patients with a history of adverse reactions to iodinated CM during CT (group A, n = 427) and patients without adverse reactions matched for age and sex with the propensity-score matching method (group B, n = 427) were studied. Contrast media-associated bowel angioedema was determined when bowel wall thickness increased after contrast enhancement compared with the precontrast scan. Potential predisposing factors including patient demographics, symptoms and time of adverse reactions, and CM-related factors were compared between patients with and without angioedema in group A. In addition, the incidence of bowel angioedema was compared between groups A and B. The incidence of CM-associated bowel angioedema in group A was 3.3% (14/427) in the per-patient analysis and 2.6% (15/578) in the per-examination analysis. The CM-associated bowel angioedema involved the distal duodenum and/or proximal jejunum and showed long-segmental circumferential bowel wall thickening on CT. None of the studied predisposing factors was different between patients with and without bowel angioedema (P > 0.05). The incidence of CM-associated bowel angioedema in group B was 1.9% (8/427) and 1.7% (8/458) for per-patient and per-examination analyses, respectively, and these rates were not significantly different between groups A and B (P = 0.346 and P = 0.370, respectively). The incidence of CM-associated bowel angioedema during CT was 1.7% to 3.3%, and none of the studied predisposing factors was associated with bowel angioedema.

  6. Rituximab therapy in a patient with low grade B-cell lymphoproliferative disease and concomitant acquired angioedema

    Directory of Open Access Journals (Sweden)

    Kaur R

    2014-12-01

    Full Text Available Ravdeep Kaur, Aerik Anthony Williams, Catherine Baker Swift, Jason W Caldwell Wake Forest University School of Medicine, Wake Forest University, Winston-Salem, NC, USA Abstract: Acquired angioedema is often associated with significant morbidity. An underlying lymphatic malignancy, autoimmune disorder, adenocarcinoma, or other malignancy may be present. Screening for these disorders should occur in all patients with acquired angioedema as treatment may result in resolution of angioedema. Keywords: complement, C1-INH deficiency, ecallantide, hemopathy

  7. ACE-I Angioedema: Accurate Clinical Diagnosis May Prevent Epinephrine-Induced Harm

    Directory of Open Access Journals (Sweden)

    R. Mason Curtis

    2016-06-01

    Full Text Available Introduction: Upper airway angioedema is a life-threatening emergency department (ED presentation with increasing incidence. Angiotensin-converting enzyme inhibitor induced angioedema (AAE is a non-mast cell mediated etiology of angioedema. Accurate diagnosis by clinical examination can optimize patient management and reduce morbidity from inappropriate treatment with epinephrine. The aim of this study is to describe the incidence of angioedema subtypes and the management of AAE. We evaluate the appropriateness of treatments and highlight preventable iatrogenic morbidity. Methods: We conducted a retrospective chart review of consecutive angioedema patients presenting to two tertiary care EDs between July 2007 and March 2012. Results: Of 1,702 medical records screened, 527 were included. The cause of angioedema was identified in 48.8% (n=257 of cases. The most common identifiable etiology was AAE (33.1%, n=85, with a 60.0% male predominance. The most common AAE management strategies included diphenhydramine (63.5%, n=54, corticosteroids (50.6%, n=43 and ranitidine (31.8%, n=27. Epinephrine was administered in 21.2% (n=18 of AAE patients, five of whom received repeated doses. Four AAE patients required admission (4.7% and one required endotracheal intubation. Epinephrine induced morbidity in two patients, causing myocardial ischemia or dysrhythmia shortly after administration. Conclusion: AAE is the most common identifiable etiology of angioedema and can be accurately diagnosed by physical examination. It is easily confused with anaphylaxis and mismanaged with antihistamines, corticosteroids and epinephrine. There is little physiologic rationale for epinephrine use in AAE and much risk. Improved clinical differentiation of mast cell and non-mast cell mediated angioedema can optimize patient management.

  8. A Novel Approach to the Treatment of Orolingual Angioedema After Tissue Plasminogen Activator Administration.

    Science.gov (United States)

    Pahs, Lesley; Droege, Chris; Kneale, Hilary; Pancioli, Arthur

    2016-09-01

    Orolingual angioedema is a rare adverse effect of tissue plasminogen activator (tPA), with an incidence of 1% to 5%. There are currently no published reports describing resolution of tPA-induced orolingual angioedema with complement inhibitor therapy. A 72-year-old man receiving home angiotensin-converting enzyme inhibitor therapy presented to the emergency department with newly developed orolingual angioedema after treatment with tPA for acute ischemic stroke. Therapy was initiated with intravenous methylprednisolone 125 mg, famotidine 20 mg, and diphenhydramine 50 mg, without significant improvement. Because of increased concern for airway protection, plasma-derived C1 esterase inhibitor was administered. Concerns about progressive and airway-threatening orolingual angioedema subsided 2 hours after administration, and invasive airway maneuvers were avoided. Orolingual angioedema is an infrequent, severe adverse effect of tPA for treatment of acute ischemic stroke. Complement inhibitors may be an additional therapeutic option for patients presenting with orolingual angioedema with potential airway compromise that is refractory to standard anaphylactic therapies. Copyright © 2016 American College of Emergency Physicians. Published by Elsevier Inc. All rights reserved.

  9. Angioedema of the upper aerodigestive tract: risk factors associated with airway intervention and management algorithm.

    Science.gov (United States)

    Brook, Christopher D; Devaiah, Anand K; Davis, Elizabeth M

    2014-03-01

    Angioedema of the upper aerodigestive tract can lead to significant airway obstruction. To date no articles have delineated risk factors for progression after initial evaluation. This article presents the results of a retrospective study of patients with angioedema at a single institution. Patients included were consecutive otolaryngology consultations for angioedema in the emergency department (ED) from 1999 to 2003. All patients were evaluated by an otolaryngologist and underwent fiber-optic laryngoscopy, which was repeated serially depending on findings. Data was collected on demographics, comorbidities, intubation, disposition, and progression of angioedema. A total of 177 patients were included in the study: 32 (18%) patients required intubation; 25 (14%) on initial presentation and 7 (4%) who progressed from an initially stable airway to requiring intervention after reevaluation. Analysis of variance (ANOVA) demonstrated a statistically significant variance between location of edema and rate of intubation, with higher rates in the pharynx and larynx vs the lip and face. Patients who required intubation after progression between serial evaluations were statistically more likely to have edema that involved deeper portions of the aerodigestive tract. Patients who required intubation were statistically more likely to be older (average age 61.8 vs 55.1 years, p = 0.03). In this large series of patients managed for aerodigestive angioedema we demonstrate risk factors associated with airway intervention, and risk factors associated with clinical progression on serial examination to airway intervention. In addition, we demonstrate a successful management algorithm for patients with aerodigestive angioedema. © 2014 ARS-AAOA, LLC.

  10. [Hereditary angioedema in Medellín (Colombia): Clinical evaluation and quality of life appraisal].

    Science.gov (United States)

    Sánchez, María Dulfary; Cuervo, Julián; Rave, Deisi; Clemen, Gustavo; Yepes-Núñez, Juan José; Ortiz-Reyes, Blanca; Sus, Sara; Cardona, Ricardo

    2015-01-01

    Hereditary angioedema is an autosomal dominant primary immunodeficiency caused by a deficiency of the C1 inhibitor protein and characterized by recurrent episodes of subcutaneous and mucosal edema. Unpredictable and frequent crisis of angioedema affect the quality of life of individuals suffering this kind of disorder. To analyze the clinical characteristics of a family with an index case of hereditary angioedema and to determine the impact of this disease on their quality of life. Twenty six members of the family were included in the trial; 25 of them were analyzed for C4 complement and antigenic and functional C1 inhibitor blood levels. Two instruments (SF-365 and KIDSCREEN-27) were used to evaluate adult health quality and children and teenagers quality of life, respectively. Eighty three percent (83%) of individuals reporting symptoms of the condition exhibited serological criteria of hereditary angioedema type I: low levels of both C4 complement and quantitative (antigenic) and qualitative (functional) C1 inhibitor. In relation to patients' psychological and emotional performance, their quality of life was significantly affected by the symptoms of hereditary angioedema. This study provides evidence of the first family in Valle de Aburrá (Colombia) characterized as having hereditary angioedema type I. Despite the use of a generic instrument, the negative impact on the quality of life of individuals suffering this disease was also confirmed.

  11. Disease: H01006 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H01006 Hereditary angioedema Hereditary angioedema (HAE) is a rare genetic disorde...r, manifested by recurrent episodes of angioedema localized to the skin or mucosa of the gastrointestinal tr...act or larynx. The laryngeal angioedema is potentially lethal. The classic forms, HAE types I and II, result...6100 PMID:14572810 (description, gene) ... AUTHORS ... Davis AE 3rd ... TITLE ... The pathogenesis of hereditary angioedema... (drug) ... AUTHORS ... Antoniu SA ... TITLE ... Therapeutic approaches in hereditary angioedema. ... JOURNAL ... Clin

  12. Conservation of the abscission signaling peptide IDA during Angiosperm evolution: withstanding genome duplications and gain and loss of the receptors HAE/HSL2

    Directory of Open Access Journals (Sweden)

    Ida M. Stø

    2015-10-01

    Full Text Available The peptide INFLORESCENCE DEFICIENT IN ABSCISSION (IDA, which signals through the leucine-rich repeat receptor-like kinases HAESA (HAE and HAESA-LIKE2 (HSL2, controls different cell separation events in Arabidopsis thaliana. We hypothesize the involvement of this signaling module in abscission processes in other plant species even though they may shed other organs than A. thaliana. As the first step towards testing this hypothesis from an evolutionarily perspective we have identified genes encoding putative orthologues of IDA and its receptors by BLAST searches of publically available protein, nucleotide and genome databases for angiosperms. Genes encoding IDA or IDA-LIKE (IDL peptides and HSL proteins were found in all investigated species, which were selected as to represent each angiosperm order with available genomic sequences. The 12 amino acids representing the bioactive peptide in A. thaliana have virtually been unchanged throughout the evolution of the angiosperms; however, the number of IDL and HSL genes varies between different orders and species. The phylogenetic analyses suggest that IDA, HSL2 and the related HSL1 gene, were present in the species that gave rise to the angiosperms. HAE has arisen from HSL1 after a genome duplication that took place after the monocot - eudicots split. HSL1 has also independently been duplicated in the monocots, while HSL2 has been lost in gingers (Zingiberales and grasses (Poales. IDA has been duplicated in eudicots to give rise to functionally divergent IDL peptides. We postulate that the high number of IDL homologs present in the core eudicots is a result of multiple whole genome duplications. We substantiate the involvement of IDA and HAE/HSL2 homologs in abscission by providing gene expression data of different organ separation events from various species.

  13. Per una retorica intermediale: corpo femminile, pubblicità e ideologia nelle opere digitali di Young-Hae Chang Heavy Industries

    Directory of Open Access Journals (Sweden)

    Beatrice Seligardi

    2014-06-01

    Full Text Available L'articolo si propone di analizzare, in prospettiva intermediale, due opere digitali di Young-Hae Chang Heavy Industries: "Cunnilingus in North Korea" e "Samsung Means To Come". Si tratta di animazioni in Adobe Flash che combinano testi e musica jazz, e sono visibili da parte di qualunque utente sul sito web del gruppo http://www.yhchang.com/. L’attenzione si concentrerà sulle scelte retoriche e stilistiche degli artisti attraverso cui viene problematizzato in maniera controversa il rapporto fra corpo femminile, tecnologia e poteri dominanti.

  14. BSACI guideline for the management of chronic urticaria and angioedema.

    Science.gov (United States)

    Powell, R J; Leech, S C; Till, S; Huber, P A J; Nasser, S M; Clark, A T

    2015-03-01

    This guidance for the management of patients with chronic urticaria and angioedema has been prepared by the Standards of Care Committee of the British Society for Allergy and Clinical Immunology (BSACI). The guideline is based on evidence as well as on expert opinion and is aimed at both adult physicians and paediatricians practising in allergy. The recommendations are evidence graded. During the development of these guidelines, all BSACI members were included in the consultation process using a Web-based system. Their comments and suggestions were carefully considered by the Standards of Care Committee. Where evidence was lacking, a consensus was reached by the experts on the committee. Included in this management guideline are clinical classification, aetiology, diagnosis, investigations, treatment guidance with special sections on children with urticaria and the use of antihistamines in women who are pregnant or breastfeeding. Finally, we have made recommendations for potential areas of future research. © 2015 John Wiley & Sons Ltd.

  15. Pericarditis - after heart attack

    Science.gov (United States)

    ... medlineplus.gov/ency/article/000166.htm Pericarditis - after heart attack To use the sharing features on this page, ... occur in the days or weeks following a heart attack . Causes Two types of pericarditis can occur after ...

  16. Heart attack first aid

    Science.gov (United States)

    First aid - heart attack; First aid - cardiopulmonary arrest; First aid - cardiac arrest ... A heart attack occurs when the blood flow that carries oxygen to the heart is blocked. The heart muscle becomes ...

  17. Social engineering attack framework

    CSIR Research Space (South Africa)

    Mouton, F

    2014-07-01

    Full Text Available link. A social engineering attack targets this weakness by; using various manipulation techniques in order to elicit sensitive; information. The field of social engineering is still in its infancy; stages with regards to formal definitions and attack...

  18. Terrorists and Suicide Attacks

    National Research Council Canada - National Science Library

    Cronin, Audrey K

    2003-01-01

    Suicide attacks by terrorist organizations have become more prevalent globally, and assessing the threat of suicide attacks against the United States and its interests at home and abroad has therefore...

  19. Solidarity under Attack

    DEFF Research Database (Denmark)

    Meret, Susi; Goffredo, Sergio

    2017-01-01

    https://www.opendemocracy.net/can-europe-make-it/susi-meret-sergio-goffredo/solidarity-under-attack......https://www.opendemocracy.net/can-europe-make-it/susi-meret-sergio-goffredo/solidarity-under-attack...

  20. Transient Ischemic Attack

    Medline Plus

    Full Text Available ... Ischemic Attack TIA , or transient ischemic attack, is a "mini stroke" that occurs when a blood clot blocks an artery for a short time. The only difference between a stroke ...

  1. Andrógenos en el tratamiento de mantenimiento a largo plazo del angioedema hereditario. ¿Es el momento de pensar en otras opciones?

    OpenAIRE

    Frías Iniesta, Jesús

    2016-01-01

    El angioedema hereditario (AEH) es una enfermedad transmitida con un patrón autosómico dominante, caracterizada por la presencia de angioedema recurrente y ocasionada por un defecto de la enzima conocida como inhibidor de C1. El principal mediador involucrado en el desarrollo del angioedema es la bradicinina

  2. Angioedema in the omalizumab chronic idiopathic/spontaneous urticaria pivotal studies.

    Science.gov (United States)

    Zazzali, James L; Kaplan, Allen; Maurer, Marcus; Raimundo, Karina; Trzaskoma, Benjamin; Solari, Paul G; Antonova, Evgeniya; Mendelson, Meryl; Rosén, Karin E

    2016-10-01

    Angioedema, present in some patients with chronic idiopathic/spontaneous urticaria (CIU/CSU), may have a negative effect on patient quality of life. To describe patient-reported angioedema and its management in the pivotal omalizumab studies (ASTERIA I, ASTERIA II, GLACIAL). Enrolled patients with CIU/CSU remained symptomatic despite treatment with histamine 1 (H 1 )-antihistamines at licensed doses (ASTERIA I, ASTERIA II) or H 1 -antihistamines at up to 4 times the approved dose plus H 2 -antihistamines and/or a leukotriene receptor antagonist (GLACIAL). All studies administered omalizumab (75, 150, or 300 mg in ASTERIA I and ASTERIA II; 300 mg in GLACIAL) or placebo subcutaneously every 4 weeks for at least 12 weeks. Urticaria Patient Daily Diary entries were completed by patients and summarized. At baseline, angioedema prevalence was higher in GLACIAL (53.1%) than in ASTERIA I (47.5%) or ASTERIA II (40.7%). The mean proportion of angioedema-free days during weeks 4 to 12 was greater for patients treated with 300 mg of omalizumab than placebo in ASTERIA I (96.1% vs 88.2%, P angioedema was managed by low-intensity interventions (doing nothing or taking medication). Treatment with 300 mg of omalizumab was efficacious in reducing patient-reported angioedema. Low-intensity interventions were generally used to manage angioedema episodes. clinicaltrials.gov Identifiers: NCT01287117 (ASTERIA I), NCT01292473 (ASTERIA II), and NCT01264939 (GLACIAL). Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

  3. Pharmacologic management of angioedema induced by angiotensin-converting enzyme inhibitors.

    Science.gov (United States)

    Scalese, Michael J; Reinaker, Travis S

    2016-06-15

    The published evidence on pharmacologic approaches to the management of angiotensin-converting enzyme inhibitor (ACEI)-induced angioedema is reviewed. Angioedema is a serious, potentially life-threatening adverse effect of ACEI use. Although the underlying mechanism is not fully understood, excess bradykinin produced through a complex interplay between the kallikrein-kinin and renin-angiotensin-aldosterone systems is thought to play a major role. The nonallergic nature of the reaction renders traditional therapies (corticosteroids and antihistamines) ineffective because those agents do not modify the proposed pathophysiology. Fresh frozen plasma (FFP) provides kinase II, a protein that breaks down bradykinin. Case reports support FFP as a treatment for ACEI-induced angioedema, but no formal evaluations have been completed to date. Both ecallantide and complement 1 esterase (C1) inhibitor concentrate reduce bradykinin production through upstream inhibition of kallikrein. C1 inhibitor concentrate has been used successfully to manage ACEI-induced angioedema in a few reported cases, but robust supportive studies are lacking. Conversely, ecallantide has been evaluated in multiple randomized trials but has not been shown to offer advantages over traditional therapies. The use of icatibant, a direct antagonist of bradykinin B2 receptors, was reported to be beneficial in several case reports and in a small Phase II study, safely and rapidly reducing symptoms of ACEI-induced angioedema. An ongoing Phase III trial (NCT01919801) will better define the role of icatibant in the management of ACEI-induced angioedema. FFP, C1 inhibitor, and icatibant appear to be safe and effective therapeutic options for the management of ACEI-induced angioedema, whereas it appears ecallantide should be avoided. Copyright © 2016 by the American Society of Health-System Pharmacists, Inc. All rights reserved.

  4. Factors associated with hospitalization of patients with angiotensin-converting enzyme inhibitor–induced angioedema

    Science.gov (United States)

    Gang, Cheng; Lindsell, Christopher J.; Moellman, Joseph; Sublett, Wesley; Hart, Kim; Collins, Sean

    2013-01-01

    Angiotensin-converting enzyme inhibitor (ACE-I)–induced angioedema can be life-threatening without emergent intervention. The putative mediator is believed to be bradykinin, similar to hereditary angioedema, so these patients respond poorly to corticosteroids and antihistamines. This study was designed to determine characteristics and clinical outcomes of patients presenting to an emergency department (ED) with ACE-I angioedema. This was a retrospective chart review of 100 patients presenting to the ED from 2007 to 2008 with an ICD-9 code of 995.1 (angioedema) or 995.2 (drug-induced angioedema). Two hundred fifty-two patients with these ICD-9 codes were identified and placed in random order, and the first 100 meeting inclusion criteria were included. Statistical analysis was primarily descriptive. All 100 patients had an ICD-9 code of 995.1 (angioedema). Patients presented in every month, with spring months (April–June) having the most presentations (32%). The median age was 59 years, 75% were African American, and 66% were admitted to the hospital. Two patients (2%) required endotracheal intubation. Lisinopril was the most commonly prescribed ACE-I (84%). The most common symptom was moderate lip and tongue swelling (89%) followed by mild difficulty breathing (12%). Tongue swelling was significantly associated with admission. Time from symptom onset to ED presentation was not associated with need for admission. Concomitant medications did not differ between admitted and discharged patients. ACE-I angioedema is associated with significant morbidity and health care use because many patients require hospitalization, suggesting an unmet need for novel therapies targeted to treat this condition. PMID:23676576

  5. Composite Dos Attack Model

    Directory of Open Access Journals (Sweden)

    Simona Ramanauskaitė

    2012-04-01

    Full Text Available Preparation for potential threats is one of the most important phases ensuring system security. It allows evaluating possible losses, changes in the attack process, the effectiveness of used countermeasures, optimal system settings, etc. In cyber-attack cases, executing real experiments can be difficult for many reasons. However, mathematical or programming models can be used instead of conducting experiments in a real environment. This work proposes a composite denial of service attack model that combines bandwidth exhaustion, filtering and memory depletion models for a more real representation of similar cyber-attacks. On the basis of the introduced model, different experiments were done. They showed the main dependencies of the influence of attacker and victim’s properties on the success probability of denial of service attack. In the future, this model can be used for the denial of service attack or countermeasure optimization.

  6. Associação entre os polimorfismos HaeIII e MspI do gene para o receptor alfa de estrogênio e densidade mamográfica em mulheres após a menopausa Association between HaeIII and MspI polymorphisms of estrogen receptor alpha gene and mammographic density in post-menopausal women

    Directory of Open Access Journals (Sweden)

    Eduardo Henrique de Moura Ramos

    2006-10-01

    Full Text Available OBJETIVO: Avaliar a presença dos polimorfismos HaeIII e MspI do gene para o receptor de estrogênio alfa, bem como fatores clínicos e suas possíveis associações com a densidade mamográfica em mulheres após a menopausa. MÉTODOS: Foram avaliadas 115 mulheres após a menopausa, não usuárias de terapia hormonal e sem lesão mamária clínica ou mamograficamente identificada. A densidade mamográfica foi determinada por três observadores independentes, tomando-se como base a classificação dos padrões mamográficos do ACR-BIRADS®, 2003 (duas avaliações subjetivas e uma computadorizada - Adobe Photoshop® 7.0. Amostras de raspado bucal foram obtidas para extração de DNA e em seguida foi realizada uma PCR-RFLP (Polymerase Chain Reation - Restriction Fragment Length Polymorphism para análise de polimorfismos presentes no íntron 1 e éxon 1 do gene do REalfa (HaeIII e MspI. RESULTADOS: O polimorfismo HaeIII foi encontrado em 43 (37,4% das 115 mulheres, ao passo que o MspI estava presente em 96 (83,5% das mesmas. Houve alto grau de concordância entre os três observadores na determinação da densidade mamográfica. Trinta e quatro (29,6% mulheres tinham mamas densas, e 81 (70,4%, mamas lipossubstituídas. CONCLUSÃO: Não houve associação entre o polimorfismo HaeIII do gene para o receptor de estrogênio alfa e densidade mamográfica (Fisher = 0,712. Houve associação próxima à significância estatística entre o polimorfismo MspI e densidade (Fisher = 0,098. Idade, paridade e índice de massa corporal mostraram-se associados com densidade mamográfica.PURPOSE: To assess the presence of estrogen receptor gene polymorphisms HaeIII and MspI as well as clinical factors, and their possible associations with high mammographic density in post-menopausal women. METHODS: One hundred and fifteen post-menopausal women, not in use of hormonal therapy and without clinical or mammographic lesions were evaluated. Three independent observers

  7. What Is a Heart Attack?

    Science.gov (United States)

    ... Research Home / Heart Attack Heart Attack Also known as Myocardial infarction Leer en español ... or years after the procedure. Other Treatments for Heart Attack Other treatments for heart attack include: Medicines Medical ...

  8. Non-myeloablative allogeneic stem cell transplantation: a new treatment option for acquired angioedema?

    Science.gov (United States)

    Zegers, I H A; Aaldering, K N A; Nieuwhof, C M G; Schouten, H C

    2015-10-01

    Acquired angioedema is a rare disorder causing recurrent life-threatening angioedema, due to decreased activity of C1 esterase inhibitor. A 57-year-old man presented to our hospital with recurrent swelling of the hands, lips, tongue, scrotum and throat. Lab examination showed the presence of an IgM kappa monoclonal antibody. Additional analysis showed that in the IgM fraction autoantibody activity against C1 esterase inhibitor was present. This confirmed the diagnosis of acquired angioedema in the presence of lymphoplasmacytic lymphoma. Despite standard therapy, there was an increase in the episodes of laryngeal oedema. Therefore it was decided to perform a non-myeloablative allogeneic haematopoietic stem cell transplantation, with his HLA-identical brother as donor. The post-transplantation course was without complications. Five years following alloSCT he is in complete remission without symptoms and with increased C1 esterase inhibitor activity. In this case all other known treatment options for severe acquired angioedema failed. This is the first case describing treatment of severe acquired angioedema, caused by lymphoplasmacytic lymphoma, with an alloSCT.

  9. [A Case of Life-Threatening Angioedema Occurred During Prolonged Angiotensin-Converting Enzyme Inhibitor Treatment].

    Science.gov (United States)

    Nakamura, Rintaro; Nihei, Shun-Ichi; Arai, Hideaki; Nagata, Keiji; Isa, Yasuki; Harayama, Nobuya; Aibara, Keiji; Kamochi, Msayuki

    2016-03-01

    Although angiotensin-converting enzyme (ACE) inhibitors are widely used as the first choice drug for treating hypertension, we have only a superficial understanding of their relationship to angioedema. We report a case of life-threatening angioedema. The case was a 60-year-old man who had been taking an ACE inhibitor for hypertension for 11 years. He visited his home doctor for dyspnea, and tongue and neck swelling. He was transported to our hospital because of the possibility of airway obstruction. On admission, his tongue and neck swelling became more severe. We performed an intubation using an endoscope and started airway management. We also stopped his ACE inhibitor. The severe tongue and neck swelling improved gradually and he was extubated on day 3. On the fifth day he was discharged. We diagnosed angioedema caused by an ACE inhibitor. Although the risk of airway obstruction with ACE inhibitors is acknowledged, we have only a superficial understanding of how prolonged ACE inhibitor treatment induces angioedema. So we should consider angioedema in cases of taking ACE inhibitors, especially in cases of prolonged treatment.

  10. Visceral angioedema: an under-recognized complication of angiotensin-converting enzyme inhibitors.

    Science.gov (United States)

    Mutnuri, Sangeeta; Khan, Adnan; Variyam, Easwaran P

    2015-03-01

    Cough and upper airway angioedema are well-recognized adverse reactions of angiotensin-converting enzyme inhibitor (ACEI) therapy. Visceral angioedema is an infrequent and often unrecognized complication of ACEI therapy. We describe a patient in whom the diagnosis was delayed for > 2 years. A 60-year-old woman with hypertension on treatment with enalapril presented with complaints of abdominal pain and diarrhea for 2 days. Physical examination was significant for diffuse abdominal tenderness and hypotension. Laboratory data were normal except for leukocytosis and elevated creatinine. Computed tomography (CT) of the abdomen showed diffuse small bowel wall thickening. ACEI-induced visceral angioedema was considered, enalapril was discontinued and supportive care was provided. Patient's symptoms and CT appearance improved 48 and 72 hours, respectively, after stopping enalapril. She remained symptom-free 1 year after discharge.

  11. Kleptographic Attacks on ECDSA

    Directory of Open Access Journals (Sweden)

    Nadezhda Anatolievna Chepick

    2014-12-01

    Full Text Available This paper presents secretly trapdoor with universal protection (SETUP attacks on the elliptic curve digital signature algorithm ECDSA. It allows a malicious manufacturer of black-box cryptosystems to implement these attacks to get access to user’s private key. The attacker can obtain user’s private key. The way ECDSA can be used for encryption and key exchange is also described.

  12. Transient Ischemic Attack

    Medline Plus

    Full Text Available ... stroke symptoms. Popular Topics TIA Cardiac Catheter Cholesterol Heart Attack Stent © 2018, American Heart Association, Inc. All rights reserved. Unauthorized use prohibited. ...

  13. Seven deadliest USB attacks

    CERN Document Server

    Anderson, Brian

    2010-01-01

    Do you need to keep up with the latest hacks, attacks, and exploits effecting USB technology? Then you need Seven Deadliest USB Attacks. This book pinpoints the most dangerous hacks and exploits specific to USB, laying out the anatomy of these attacks including how to make your system more secure. You will discover the best ways to defend against these vicious hacks with step-by-step instruction and learn techniques to make your computer and network impenetrable. Attacks detailed in this book include: USB Hacksaw USB Switchblade USB Based Virus/Malicous Code Launch USB Device Overflow RAMdum

  14. Angiodema due to oral acitretin and isotretinoin Angioedema por acitretina e isotretinoína oral

    Directory of Open Access Journals (Sweden)

    Roberto Rheingantz da Cunha Filho

    2011-08-01

    Full Text Available Angioedema may be caused by nonsteroidal antiinflammatory drugs, angiotensin- converting enzyme inhibitors, radiocontrast media, antibiotics, sea food etc. It can involve an allergic (IgE-mediated or non-allergic hypersensitivity reaction, both with a similar clinical presentation. While angioedema due to isotretionin has been described previously, this is the first description of angiodema due to acitretin. We report two uncommon cases of palpebral and labial angiodema due to retinoids, by acitretin and oral isotretinoin respectively: a 48-year-old man with psoriasis and a 24-year-old woman with severe acne resistant to antibiotics and topical drugs. In both cases the reaction persisted through-out treatment with these drugs, but resolved quickly after discontinuation. Reintroduction of the drugs brought on angioedema againAngioedema pode ser causado por diversos fármacos como : antiinflamatórios não-esteroidais, inibidores da ECA, contrastes, antibióticos e frutos do mar, entre outras causas. Pode ser uma reação alérgica, mediada por IgE, ou não-alérgica, com apresentações clínicas semelhantes. Angioedema por isotretinoína já foi relatado, mas não por acitretina. Relatamos dois casos, uma com angioedema palpebral e um labial, por acitretina e isotretinoína, respectivamente: um paciente de 48 anos com psoríase e uma paciente de 24 anos com acne resistente à terapia convencional. Em ambos casos a afecção persistiu durante o tratamento, resolveu com a interrupção e recidivou com reexposição

  15. Mucosal-dominant pemphigus vulgaris in a captopril-taking woman with angioedema.

    Science.gov (United States)

    Gornowicz-Porowska, Justyna; Dmochowski, Marian; Pietkiewicz, Pawel; Bowszyc-Dmochowska, Monika

    2015-01-01

    We describe a 39-year-old woman with an apparent captopril-induced, contact mucosal-dominant pemphigus vulgaris and angioedema, who took captopril during a bout of arterial hypertension. This exposure suggests that captopril and pathophysiology of angioedema stimulated the development of pemphigus vulgaris, which was diagnosed using the novel, indirect immunofluorescence BIOCHIP mosaic, with the modification to detect serum IgG4 autoantibodies. We discuss the patient, who experienced a chain of events leading to the active stage of pemphigus vulgaris, and review concepts of pemphigus vulgaris inducible by drugs and pathological immunity.

  16. Management of upper airway edema caused by hereditary angioedema

    Directory of Open Access Journals (Sweden)

    Farkas Henriette

    2010-07-01

    Full Text Available Abstract Hereditary angioedema is a rare disorder with a genetic background involving mutations in the genes encoding C1-INH and of factor XII. Its etiology is unknown in a proportion of cases. Recurrent edema formation may involve the subcutis and the submucosa - the latter can produce obstruction in the upper airways and thereby lead to life-threatening asphyxia. This is the reason for the high, 30-to 50-per-cent mortality of undiagnosed or improperly managed cases. Airway obstruction can be prevented through early diagnosis, meaningful patient information, timely recognition of initial symptoms, state-of-the-art emergency therapy, and close monitoring of the patient. Prophylaxis can substantially mitigate the risk of upper airway edema and also improve the patients' quality of life. Notwithstanding the foregoing, any form of upper airway edema should be regarded as a potentially life-threatening condition. None of the currently available prophylactic modalities is capable of preventing UAE with absolute certainty.

  17. Risk of angioedema associated with levetiracetam compared with phenytoin: Findings of the observational health data sciences and informatics research network.

    Science.gov (United States)

    Duke, Jon D; Ryan, Patrick B; Suchard, Marc A; Hripcsak, George; Jin, Peng; Reich, Christian; Schwalm, Marie-Sophie; Khoma, Yuriy; Wu, Yonghui; Xu, Hua; Shah, Nigam H; Banda, Juan M; Schuemie, Martijn J

    2017-08-01

    Recent adverse event reports have raised the question of increased angioedema risk associated with exposure to levetiracetam. To help address this question, the Observational Health Data Sciences and Informatics research network conducted a retrospective observational new-user cohort study of seizure patients exposed to levetiracetam (n = 276,665) across 10 databases. With phenytoin users (n = 74,682) as a comparator group, propensity score-matching was conducted and hazard ratios computed for angioedema events by per-protocol and intent-to-treat analyses. Angioedema events were rare in both the levetiracetam and phenytoin groups (54 vs. 71 in per-protocol and 248 vs. 435 in intent-to-treat). No significant increase in angioedema risk with levetiracetam was seen in any individual database (hazard ratios ranging from 0.43 to 1.31). Meta-analysis showed a summary hazard ratio of 0.72 (95% confidence interval [CI] 0.39-1.31) and 0.64 (95% CI 0.52-0.79) for the per-protocol and intent-to-treat analyses, respectively. The results suggest that levetiracetam has the same or lower risk for angioedema than phenytoin, which does not currently carry a labeled warning for angioedema. Further studies are warranted to evaluate angioedema risk across all antiepileptic drugs. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

  18. Plants under dual attack

    NARCIS (Netherlands)

    Ponzio, C.A.M.

    2016-01-01

    Though immobile, plants are members of complex environments, and are under constant threat from a wide range of attackers, which includes organisms such as insect herbivores or plant pathogens. Plants have developed sophisticated defenses against these attackers, and include chemical responses such

  19. Heart attack - discharge

    Science.gov (United States)

    ... and lifestyle Cholesterol - drug treatment Controlling your high blood pressure Deep vein thrombosis - discharge Dietary fats explained Fast food tips Heart attack - discharge Heart attack - what to ask your doctor Heart bypass ... pacemaker - discharge High blood pressure - what to ask your doctor How to read ...

  20. [Analysis of 76 patients with urticaria and angioedema induced by non-steroidal anti-inflammatory drugs (NSAIDs) in Japan].

    Science.gov (United States)

    Moriya, Maki; Aihara, Michiko; Hirota, Rie; Hirata, Yuko; Ikinaga, Naoko; Takamura, Naoko; Kunimi, Yuko; Uchida, Takahisa; Ikezawa, Zenro

    2011-06-01

    The pathogenesis of urticaria and angioedema induced by non-steroidal anti-inflammatory drugs (NSAIDs) is still obscure. We analyzed the clinical characteristics of patients with NSAIDs-induced urticaria and angioedema without asthma in Japan. We retrospectively collected the cases of NSAIDs-induced urticaria and angioedema from Japanese medical journals in 2000-2009. Seventy-six patients were analyzed. The male/female ratio was 1:2.5 and the mean age was 38.1 years. Urticaria was most frequent clinical manifestation in 3 groups; urticaria alone, urticaria and angioedema, and angioedema alone. Time interval from drug administration to onset was 5 minutes to 48 hours by aspirin at a dose of 25-1000 mg. Skin prick test was performed with aspirin in 33 patients, and the results were negative in all patients. Meloxicam, a selective cyclooxygenase-2 (COX-2) inhibitor, and celecoxib, a new selective COX-2 inhibitor, were administered safely in 4 of 6 patients and in 2 of 3 patients with NSAIDs-induced urticaria, respectively. These drugs were administered safely in all administered patients with NSAIDs-induced angioedema. Tiaramidehydrochroride (a basic COX-1 inhibitor) was safely used in 23 administered patients with NSAIDs-induced angioedema. Leukotriene receptor antagonists were effective in 2 of 5 patients administered, but aggravated symptoms in the others. Diversity of NSAIDs-induced urticaria and angioedema was shown in this study. Pathogenesis of NSAIDs-induced urticaria and angioedema without asthma seems to be different from that of NSAIDs-induced asthma.

  1. The role of icatibant-the B2 bradykinin receptor antagonist-in life-threatening laryngeal angioedema in the ED.

    Science.gov (United States)

    Bartal, Carmi; Zeldetz, Vladimir; Stavi, Vered; Barski, Leonid

    2015-03-01

    Angioedema is a localized, sudden, transient, and often recurrent swelling of the deeper layers of the skin or mucosa with no epidermal component. It is caused by vasoactive substances that produce a transient increase in endothelial permeability. Angioedema involving the laryngeal components is a life-threatening situation for the patient,and it is a challenge for the emergency medicine physician to rapidly achieve a safety airway. Most cases of laryngeal angioedema are induced by histamine release; but 10% are bradykinin induced, which does not respond to the conventional algorithm of treating allergic induced angioedema. We present a case report of an angiotensin converting enzyme (ACE) inhibitor–induced laryngeal angioedema alleviated only after treatment with the new bradykinin receptor inhibitor medication icatibant which was licensed only for use in hereditary angioedema. We reviewed the literature for the use of icatibant in acquired drug-induced angioedema; and because of the similar pathogenesis between the hereditary angioedema and the ACE inhibitor–induced angioedema,we propose an algorithm for careful use of icatibantin life-threatening angioedema in the emergency department.

  2. MR imaging of intestinal angioedema related to angiotensin-converting enzyme inhibitors: Report of three cases and review of literature.

    Science.gov (United States)

    Savino, Michael R; Mittal, Pardeep K; Miller, Frank H

    Angioedema is a condition in which an increase in vascular permeability leads to the swelling of body tissues. There are both hereditary and acquired forms of the disease, with the latter often associated with the administration of angiotensin-converting enzyme inhibitor medication. Involvement of the intestinal tract is a rare manifestation of angioedema, and can present with abdominal pain, nausea, and vomiting. It is critical for radiologists to be aware of this entity, as they may have the only opportunity to make the diagnosis. We present three cases of intestinal angioedema diagnosed on MRI with discussion of the imaging findings. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Abordagem anestésica de doente com angioedema hereditário proposto para cirurgia electiva

    OpenAIRE

    Conceição, Luís; Martinho, Hélder; Azenha, Marta

    2014-01-01

    O angioedema hereditário é uma entidade rara, com transmissão autossómica dominante, causada por deficiência no inibidor de C1. Esta condiciona uma ativação descontrolada da via clássica do complemento e da cascata das cininas, sendo responsável por episódios de angioedema com possível comprometimento da via aérea. Os autores descrevem um caso clínico de um doente com 36 anos com angioedema hereditário tipo I proposto para colecistectomia electiva sob anestesia geral, tendo sido tomadas algum...

  4. A novel assay to diagnose hereditary angioedema utilizing inhibition of bradykinin-forming enzymes

    DEFF Research Database (Denmark)

    Joseph, Kusumam; Bains, Sonia; Tholanikunnel, Baby G

    2015-01-01

    BACKGROUND: Hereditary angioedema types I and II are caused by a functional deficiency of C1 inhibitor (C1-INH) leading to overproduction of bradykinin. The current functional diagnostic assays employ inhibition of activated C1s, however, an alternative, more physiologic method, is desirable...

  5. Postanesthetic Severe Oral Angioedema in Patient’s Taking Angiotensin-Converting Enzyme Inhibitor

    Directory of Open Access Journals (Sweden)

    Acílio Marques

    2014-01-01

    Full Text Available Angiotensin-converting enzyme (ACE inhibitors are the leading cause of a drug-induced angioedema. This occurrence is frequently underdiagnosed, but its relapse can be life-threatening. The authors’ intention in reporting this clinical case is to sound a warning about reviewing attitudes and surveillance to try to improve patient perioperative safety.

  6. The effectiveness of a history-based diagnostic approach in chronic urticaria and angioedema

    NARCIS (Netherlands)

    Kozel, M. M.; Mekkes, J. R.; Bossuyt, P. M.; Bos, J. D.

    1998-01-01

    OBJECTIVE: To assess the value of extensive laboratory screening for the identification of causes in patients with chronic urticaria and/or angioedema. DESIGN: In a prospective study involving 220 patients, 2 diagnostic strategies were compared: the combination of detailed history taking and limited

  7. Nocturnal panic attacks

    Directory of Open Access Journals (Sweden)

    Lopes Fabiana L.

    2002-01-01

    Full Text Available The panic-respiration connection has been presented with increasing evidences in the literature. We report three panic disorder patients with nocturnal panic attacks with prominent respiratory symptoms, the overlapping of the symptoms with the sleep apnea syndrome and a change of the diurnal panic attacks, from spontaneous to situational pattern. The implication of these findings and awareness to the distinct core of the nocturnal panic attacks symptoms may help to differentiate them from sleep disorders and the search for specific treatment.

  8. Heart Attack Payment - State

    Data.gov (United States)

    U.S. Department of Health & Human Services — Payment for heart attack patients measure – state data. This data set includes state-level data for payments associated with a 30-day episode of care for heart...

  9. Heart Attack Payment - Hospital

    Data.gov (United States)

    U.S. Department of Health & Human Services — Payment for heart attack patients measure – provider data. This data set includes provider data for payments associated with a 30-day episode of care for heart...

  10. Heart Attack Payment - National

    Data.gov (United States)

    U.S. Department of Health & Human Services — Payment for heart attack patients measure – national data. This data set includes national-level data for payments associated with a 30-day episode of care for heart...

  11. Transient Ischemic Attack

    Medline Plus

    Full Text Available ... TIA , or transient ischemic attack, is a "mini stroke" that occurs when a blood clot blocks an ... a short time. The only difference between a stroke and TIA is that with TIA the blockage ...

  12. Case-control study evaluating competing risk factors for angioedema in a high-risk population.

    Science.gov (United States)

    Kamil, Rebecca J; Jerschow, Elina; Loftus, Patricia A; Tan, Melin; Fried, Marvin P; Smith, Richard V; Foster, David; Ow, Thomas J

    2016-08-01

    Black race is a risk factor for angioedema. The primary aim was to examine the relationship between race-ethnicity and risk factors for angioedema. Using a retrospective case-control study design, data was extracted with the Clinical Looking Glass utility, a data collection and management tool that captures data from electronic medical record systems within the Montefiore Healthcare System. Cases were emergency department (ED) visits with primary or secondary International Classification of Diseases, Ninth Revision, code diagnoses of angioedema in adults aged ≥ 18 years from January 2008 to December 2013 at three Montefiore centers in Bronx, New York. Controls were a random sampling of adult ED visits during the same period. In primary analyses, angiotensin-converting enzyme inhibitor (ACE-I) and black race were evaluated for synergy. The influence of different risk factors in the development of angioedema was evaluated using logistic regression models. Finally, race-ethnicity was further explored by evaluating for effect modification by stratification of models by race-ethnicity categories. There were 1,247 cases and 6,500 controls randomly selected from a larger control pool. ACE-I use (odds ratio [OR] 3.70, 95% confidence interval [CI] 2.98, 4.60), hypertension (OR 1.88, 95% CI 1.55, 2.29), and black race (OR 2.25, 95% CI 1.86, 2.72) were the strongest risk factors. ACE-I use and black race were not synergistic (OR 1.10, 95% CI 0.80, 1.51). Race-ethnicity was an effect modifier for certain risk factors. Race-ethnicity acts as an effect modifier for particular angioedema risk factors. The two strongest risk factors, ACE-I use and black race, were not synergistic. 3b. Laryngoscope, 126:1823-1830, 2016. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  13. Effect of C1-Esterase-inhibitor in angiotensin-converting enzyme inhibitor-induced angioedema.

    Science.gov (United States)

    Greve, Jens; Bas, Murat; Hoffmann, Thomas K; Schuler, Patrick J; Weller, Patrick; Kojda, Georg; Strassen, Ulrich

    2015-06-01

    The study objective was to generate pilot data to evaluate the effectiveness and safety of C1-esterase-inhibitor concentrate (C1-INH) compared to standard treatment in patients with angiotensin-converting enzyme inhibitor (ACEi)-induced angioedema affecting the upper aerodigestive tract. Proof-of-concept case series with historical control. Adult patients with angioedema in the upper aerodigestive tract presenting to the emergency department were included. After establishing the diagnosis of ACEi-induced angioedema based on patient history and thorough clinical examination, all patients were administered 1,000 international units (IU) of C1-INH intravenously. A historical control group consisting of adult patients with ACEi-induced angioedema who had been treated with intravenous corticosteroids and antihistamines at the same institution over the past 8 years was used for comparison. The most important parameters assessed were the time to complete resolution of symptoms and the need for intubation or tracheotomy. Ten patients were included in the C1-INH group and 47 in the corticosteroid/antihistamine group. The time to complete resolution of symptoms was considerably longer in the historical control group (33.1 ± 19.4 hours) than in the C1-INH group (10.1 ± 3.0 hours). No intubation or tracheotomy was needed in the C1-INH group (0/10 patients), whereas three out of the 47 historical controls required tracheotomy and two were intubated (5/47). The results suggest a role for C1-INH as an effective and safe therapeutic option in patients with ACEi-induced angioedema, which needs to be confirmed by further larger and double-blinded studies. 4. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

  14. Facial Dog Attack Injuries

    OpenAIRE

    Lin, Wei; Patil, Pavan Manohar

    2013-01-01

    The exposed position of the face makes it vulnerable to dog bite injuries. This fact combined with the short stature of children makes them a high-risk group for such attacks. In contrast to wounds inflicted by assaults and accidents, dog bite wounds are deep puncture type wounds compounded by the presence of pathologic bacteria from the saliva of the attacking dog. This, combined with the presence of crushed, devitalized tissue makes these wounds highly susceptible to infection. Key to succe...

  15. Consensus statement on the diagnosis, management, and treatment of angioedema mediated by Bradykinin. Part. II: treatment, follow-up, and special situations

    OpenAIRE

    Caballero, T.; Baeza, M. L.; Cabañas, R.; Campos, A.; Cimbollek, S.; Gómez-Traseira, C.; González-Quevedo, T.; Guilarte, M.; Jurado-Palomo, J.; Larco, J. I.; López-Serrano, M. C.; López-Trascasa, M.; Marcos, C.; Muñoz- Caro, J. M.; Pedrosa, M.

    2011-01-01

    Background: There are no previous Spanish guidelines or consensus statements on bradykinin-induced angioedema. Aim: To draft a consensus statement on the management and treatment of angioedema mediated by bradykinin in light of currently available scientifi c evidence and the experience of experts. This statement will serve as a guideline to health professionals. Methods: The consensus was led by the Spanish Study Group on Bradykinin-Induced Angioedema, a working group of the Spanish...

  16. Phenotype Standardization of Angioedema in the Head and Neck Region Caused by Agents Acting on the Angiotensin System

    Science.gov (United States)

    Wadelius, M; Marshall, S E; Islander, G; Nordang, L; Karawajczyk, M; Yue, Q-Y; Terreehorst, I; Baranova, E V; Hugosson, S; Sköldefors, K; Pirmohamed, M; Maitland-van der Zee, A-H; Alfirevic, A; Hallberg, P; Palmer, C N A

    2014-01-01

    Angioedema is a potentially life-threatening adverse reaction to angiotensin-converting enzyme inhibitors and angiotensin receptor blockers. To study the genetic etiology of this rare adverse event, international consortia and multicenter recruitment of patients are needed. To reduce patient heterogeneity, we have standardized the phenotype. In brief, it comprises swelling in the head and neck region that first occurs during treatment. It should not coincide with urticaria or have another likely cause such as hereditary angioedema. PMID:24960520

  17. Comparison of Clinical Factors Between Patients With Angiotensin-Converting Enzyme Inhibitor-Induced Angioedema and Cough.

    Science.gov (United States)

    Hallberg, Pär; Nagy, Julia; Karawajczyk, Malgorzata; Nordang, Leif; Islander, Gunilla; Norling, Pia; Johansson, Hans-Erik; Kämpe, Mary; Hugosson, Svante; Yue, Qun-Ying; Wadelius, Mia

    2017-04-01

    Angioedema is a rare and serious adverse drug reaction (ADR) to angiotensin-converting enzyme (ACE) inhibitor treatment. Dry cough is a common side effect of ACE inhibitors and has been identified as a possible risk factor for angioedema. We compared characteristics between patients with ACE inhibitor-induced angioedema and cough with the aim of identifying risk factors that differ between these adverse events. Data on patients with angioedema or cough induced by ACE inhibitors were collected from the Swedish database of spontaneously reported ADRs or from collaborating clinicians. Wilcoxon rank sum test, Fisher's exact test, and odds ratios (ORs) with 95% CIs were used to test for between-group differences. The significance threshold was set to P angioedema and 121 with cough only. Smoking and concomitant selective calcium channel blocker treatment were more frequent among patients with angioedema than cough: OR = 4.3, 95% CI = 2.1-8.9, P = 2.2 × 10 -5 , and OR = 3.7, 95% CI = 2.0-7.0, P = 1.7 × 10 -5 . Angioedema cases were seen more often in male patients (OR = 2.2, 95% CI = 1.4-3.6, P = 1.3 × 10 -4 ) and had longer time to onset and higher doses than those with cough ( P = 3.2 × 10 -10 and P = 2.6 × 10 -4 ). A multiple model containing the variables smoking, concurrent calcium channel blocker treatment, male sex, and time to onset accounted for 26% of the variance between the groups. Smoking, comedication with selective calcium channel blockers, male sex, and longer treatment time were associated with ACE inhibitor-induced angioedema rather than cough.

  18. Pediatric Hereditary Angioedema as a Cause of Acute Compartment Syndrome of the Hand and Forearm: A Case Report.

    Science.gov (United States)

    Venditto, Chelsea; Jager, Zachary; LoGiudice, John; Matloub, Hani

    2017-05-01

    Compartment syndrome of the upper extremity is a surgical emergency that, when left untreated, can have dire consequences. Its causes are numerous, one of which is the uncommon entity hereditary angioedema, an autosomal dominant disease resulting in edema in a variety of potential locations, including the extremities. This is only the second time hereditary angioedema has been mentioned in the literature as a cause of compartment syndrome. We present a case of hereditary angioedema leading to hand and forearm compartment syndrome in a 13-year-old pediatric patient. Diagnosis of hereditary angioedema was made by our Rheumatology colleagues with physical exam and a thorough history, and confirmed by laboratory studies. Our patient presented with compartment syndrome of the hand and forearm and underwent hand and volar forearm fasciotomies. She was subsequently worked up for hereditary angioedema with laboratory results confirming the diagnosis. She was discharged after a 5-day hospitalization with prophylactic C1-inhibitor therapy. Hereditary angioedema is a rare but known cause of compartment syndrome of the upper extremity, and must be considered when patients present with compartment syndrome of unknown etiology. This disease can be diagnosed by laboratory studies and symptoms can be controlled with medical therapy.

  19. Cyber Attacks, Information Attacks, and Postmodern Warfare

    Directory of Open Access Journals (Sweden)

    Valuch Jozef

    2017-06-01

    Full Text Available The aim of this paper is to evaluate and differentiate between the phenomena of cyberwarfare and information warfare, as manifestations of what we perceive as postmodern warfare. We describe and analyse the current examples of the use the postmodern warfare and the reactions of states and international bodies to these phenomena. The subject matter of this paper is the relationship between new types of postmodern conflicts and the law of armed conflicts (law of war. Based on ICJ case law, it is clear that under current legal rules of international law of war, cyber attacks as well as information attacks (often performed in the cyberspace as well can only be perceived as “war” if executed in addition to classical kinetic warfare, which is often not the case. In most cases perceived “only” as a non-linear warfare (postmodern conflict, this practice nevertheless must be condemned as conduct contrary to the principles of international law and (possibly a crime under national laws, unless this type of conduct will be recognized by the international community as a “war” proper, in its new, postmodern sense.

  20. Collaborative Attack vs. Collaborative Defense

    Science.gov (United States)

    Xu, Shouhuai

    We have witnessed many attacks in the cyberspace. However, most attacks are launched by individual attackers even though an attack may involve many compromised computers. In this paper, we envision what we believe to be the next generation cyber attacks — collaborative attacks. Collaborative attacks can be launched by multiple attackers (i.e., human attackers or criminal organizations), each of which may have some specialized expertise. This is possible because cyber attacks can become very sophisticated and specialization of attack expertise naturally becomes relevant. To counter collaborative attacks, we might need collaborative defense because each “chain” in a collaborative attack may be only adequately dealt with by a different defender. In order to understand collaborative attack and collaborative defense, we present a high-level abstracted framework for evaluating the effectiveness of collaborative defense against collaborative attacks. As a first step towards realizing and instantiating the framework, we explore a characterization of collaborative attacks and collaborative defense from the relevant perspectives.

  1. Seven Deadliest Wireless Technologies Attacks

    CERN Document Server

    Haines, Brad

    2010-01-01

    How can an information security professional keep up with all of the hacks, attacks, and exploits? One way to find out what the worst of the worst are is to read the seven books in our Seven Deadliest Attacks Series. Not only do we let you in on the anatomy of these attacks but we also tell you how to get rid of them and how to defend against them in the future. Countermeasures are detailed so that you can fight against similar attacks as they evolve. Attacks featured in this book include:Bluetooth AttacksCredit Card, Access Card, and Passport AttacksBad Encryption

  2. Analysis of characteristics associated with reinjection of icatibant

    DEFF Research Database (Denmark)

    Longhurst, Hilary J; Aberer, Werner; Bouillet, Laurence

    2015-01-01

    PURPOSE: Phase 3 icatibant trials showed that most hereditary angioedema (HAE) (C1 inhibitor deficiency) acute attacks were treated successfully with one injection of icatibant, a selective bradykinin B2 receptor antagonist. We conducted a post hoc analysis of icatibant reinjection for HAE type I...... and II attacks in a real-world setting by using data from the Icatibant Outcome Survey, an ongoing observational study that monitors the safety and effectiveness of icatibant treatment. METHODS: Descriptive retrospective analyses of icatibant reinjection were performed on Icatibant Outcome Survey data...... (February 2008 to December 2012). New attacks were defined as the onset of new symptoms after full resolution of the previous attack. Potential associations between the patient and attack characteristics and reinjection were explored by using logistic regression analysis. RESULTS: Icatibant was administered...

  3. Shark attack in Natal.

    Science.gov (United States)

    White, J A

    1975-02-01

    The injuries in 5 cases of shark attack in Natal during 1973-74 are reviewed. Experience in shark attacks in South Africa during this period is discussed (1965-73), and the value of protecting heavily utilized beaches in Natal with nets is assessed. The surgical applications of elasmobranch research at the Oceanographic Research Institute (Durban) and at the Headquarters of the Natal Anti-Shark Measures Board (Umhlanga Rocks) are described. Modern trends in the training of surf life-guards, the provision of basic equipment for primary resuscitation of casualties on the beaches, and the policy of general and local care of these patients in Natal are discussed.

  4. Angiotensin-converting enzyme inhibitor-induced angioedema may not be a class-related event

    Directory of Open Access Journals (Sweden)

    Hassan A Farhat

    2012-09-01

    Full Text Available Angioedema is a rare but potentially life threatening condition commonly associated with angiotensin-converting enzyme inhibitors (ACEIs. The incidence is approximately 0.1- 0.2% and may occur within the first week to several years of taking an ACEI. We present a case of a 37-year-old African-American male who was uneventfully taking a drug combination of quinapril and hydrochlorothiazide. When his medication was changed to lisinopril he developed an acute swelling of his lower lip and chin on fifth dose. The angioedema subsided within 24 hours after discontinuation of lisinopril. Therefore, this suggests that future treatment with ACEIs, as well as angiotensin receptor blockers (ARBs, is not recommended in this type of patient.

  5. Depression After Heart Attack

    Science.gov (United States)

    ... Heart Attack? Redford B. Williams Download PDF https://doi.org/10.1161/CIRCULATIONAHA.110.017285 Circulation. 2011; 123: ... e639-e640 , originally published June 27, 2011 https://doi.org/10.1161/CIRCULATIONAHA.110.017285 Citation Manager Formats ...

  6. Temporal Cyber Attack Detection.

    Energy Technology Data Exchange (ETDEWEB)

    Ingram, Joey Burton [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Draelos, Timothy J. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Galiardi, Meghan [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Doak, Justin E. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

    2017-11-01

    Rigorous characterization of the performance and generalization ability of cyber defense systems is extremely difficult, making it hard to gauge uncertainty, and thus, confidence. This difficulty largely stems from a lack of labeled attack data that fully explores the potential adversarial space. Currently, performance of cyber defense systems is typically evaluated in a qualitative manner by manually inspecting the results of the system on live data and adjusting as needed. Additionally, machine learning has shown promise in deriving models that automatically learn indicators of compromise that are more robust than analyst-derived detectors. However, to generate these models, most algorithms require large amounts of labeled data (i.e., examples of attacks). Algorithms that do not require annotated data to derive models are similarly at a disadvantage, because labeled data is still necessary when evaluating performance. In this work, we explore the use of temporal generative models to learn cyber attack graph representations and automatically generate data for experimentation and evaluation. Training and evaluating cyber systems and machine learning models requires significant, annotated data, which is typically collected and labeled by hand for one-off experiments. Automatically generating such data helps derive/evaluate detection models and ensures reproducibility of results. Experimentally, we demonstrate the efficacy of generative sequence analysis techniques on learning the structure of attack graphs, based on a realistic example. These derived models can then be used to generate more data. Additionally, we provide a roadmap for future research efforts in this area.

  7. Fatal crocodile attack.

    Science.gov (United States)

    Chattopadhyay, Saurabh; Shee, Biplab; Sukul, Biswajit

    2013-11-01

    Attacks on human beings by various animals leading to varied types of injuries and even death in some cases are not uncommon. Crocodile attacks on humans have been reported from a number of countries across the globe. Deaths in such attacks are mostly due to mechanical injuries or drowning. Bites by the crocodiles often cause the limbs to be separated from the body. The present case refers to an incident of a fatal attack by a crocodile on a 35 years old female where only the mutilated head of the female was recovered. Multiple lacerated wounds over the face and scalp along with fracture of the cranial bones was detected on autopsy. Two distinct bite marks in the form of punched in holes were noted over the parietal and frontal bones. Injuries on the head with its traumatic amputation from the body were sufficient to cause death. However, the presence of other fatal injuries on the unrecovered body parts could not be ruled out. Copyright © 2013 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.

  8. Episodic angioedema with eosinophilia (Gleich syndrome) is a multilineage cell cycling disorder.

    Science.gov (United States)

    Khoury, Paneez; Herold, Jacqueline; Alpaugh, Alexandra; Dinerman, Ellen; Holland-Thomas, Nicole; Stoddard, Jennifer; Gurprasad, Shakuntala; Maric, Irina; Simakova, Olga; Schwartz, Lawrence B; Fong, Juelia; Lee, Chyi-Chia Richard; Xi, Liqiang; Wang, Zengfeng; Raffeld, Mark; Klion, Amy D

    2015-03-01

    Episodic angioedema with eosinophilia (Gleich syndrome) is a rare disorder characterized by episodes of angioedema and eosinophilia that occur at monthly intervals and resolve spontaneously without therapy. Despite the striking periodicity of this disorder, its similarity to other cyclic hematopoietic disorders with multilineage involvement has not been assessed. To characterize the involvement of cell lineages in the etiology and pathogenesis of episodic angioedema with eosinophilia, four subjects were evaluated by blood counts and other analyses over the course of 1-2 months. Surface marker expression was assessed on T cells by flow cytometry and clonality by polymerase chain reaction. Intracellular cytokine evaluation, bone marrow and skin biopsies were performed during different parts of the cycle. Cycling of multiple cell lineages, including neutrophils, lymphocytes and eosinophils, was observed in the four subjects with the disorder with a periodicity of 25-35 days. An aberrant CD3(-)CD4(+) T-cell population was detected in all four subjects, and T-cell receptor rearrangement studies showed a clonal pattern in three subjects. A peak of type II cytokines was detected in the serum of subjects prior to the onset of symptoms and eosinophil cycling and corresponded to ex-vivo type II cytokines detected intracellularly in CD3(+)CD4(+)CD154(+) T cells. Although the etiology of episodic angioedema with eosinophilia is not yet known, multiple lineages, including lymphocytes, neutrophils and mast cells, are involved and may be related to disease pathogenesis. Whether these cells act directly or promote eosinophilia and eosinophil activation remains to be elucidated. All subjects gave informed consent and were evaluated under an Institutional Review Board-approved protocol (NCT00001406). Copyright© Ferrata Storti Foundation.

  9. BIRD ATTACK OCULAR INJURIES.

    Science.gov (United States)

    Tabatabaei, Seyed Ali; Soleimani, Mohammad; Behrouz, Mahmoud Jabbarvand

    2017-03-29

    To report 30 patients with bird attack-related eye injuries. This study was performed among patients coming to Farabi Eye Hospital, Tehran, Iran, from 2010 to 2015 with a history of bird attack causing eye injury. The inclusion criteria were a history of bird attack by pecking causing eye injury and having treatment and follow-up record for at least 6 months after treatment. The primary eye examinations included a full ophthalmic examination including evaluation of uncorrected visual acuity and best-corrected visual acuity (BCVA), anterior segment slit lamp biomicroscopy, and photography. For all patients with penetrating injury, primary repair was undertaken. Thirty patients (10 females and 20 males) with a mean age of 23.3 ± 18.5 years entered the study. The most common zone of injury was zone 1 (P < 0.001), and lensectomy was not needed in majority of patients (P < 0.001). The most common bird causing the injury was mynah (P < 0.001). Those patients with baseline BCVA of less than 20/200 or those with endophthalmitis had statistically worse final BCVA after treatment. Patients attacked by mynah bird had significantly better pretreatment uncorrected visual acuity and BCVA. The most common bird causing the eye injury among the sample of patients from Iran was mynah, which differs with previous studies indicating the rooster attack as the most common cause of eye injury. The authors also found that the most common zone of injury was zone 1, and the presence of endophthalmitis and lower baseline BCVA were significant risk factors for worse visual outcomes.

  10. Blocking of Brute Force Attack

    OpenAIRE

    M.Venkata Krishna Reddy

    2012-01-01

    A common threat Web developers face is a password-guessing attack known as a brute-force attack. A brute-force attack is an attempt to discover a password by systematically trying every possible combination of letters, numbers, and symbols until you discover the one correct combination that works. If your Web site requires user authentication, you are a good target for a brute-force attack. An attacker can always discover a password through a brute-force attack, but the downside is that it co...

  11. Acetyl salicylic acid induced-urticaria and/or angioedema in atopic children.

    Science.gov (United States)

    Botey, J; Navarro, C; Aulesa, C; Marín, A; Eseverri, J L

    1988-01-01

    From the report of Hirschberg, only 3 years after aspirin synthesis, there have been numerous works dedicated to showing the different types of adverse reactions found following aspirin administration. However, there are few publications on the process of urticaria and/or acute angioedema induced by ASA and few reported cases were found in children. Thus, we present 6 atopic children with urticaria and/or angioedema related with ASA. A carefully detailed history, oral provocation with ASA, oral provocation with other NSAI and HBDT with ASA were done to all of them. The oral provocation with ASA was positive in 5 of the 6 cases. The provocations with the rest of the NSAI and tartrazine and sodium benzoate were negative in all of the patients. The HBDT was positive in 5 of the cases. In conclusion, we insist that aspirin intolerance is not infrequent in infancy and it is not rare to see urticaria and or angioedema, in spite of the fact that asthmatics, atopics or non atopics, usually present as bronchospasm. We also believe that the HBDT can be a method of diagnosis used in these cases.

  12. Angiotensin converting enzyme inhibitor-related angioedema: onset, presentation, and management.

    Science.gov (United States)

    Chan, Norman J; Soliman, Ahmed M S

    2015-02-01

    This study aimed to determine the duration of use, presentation, and management of angiotensin converting enzyme (ACE) inhibitor-related angioedema patients at an urban academic medical center. Retrospective chart review. Eighty-eight patients who presented with ACE inhibitor-related angioedema between January 1, 2012, and December 31, 2012, were identified. They presented anywhere from 1 day to 20 years after starting an ACE inhibitor. About half the patients (50.7%) presented after taking an ACE inhibitor for at least 1 year. Fifty-five patients were female (62.5%). Twenty-eight patients (31.8%) had an airway intervention with 27 intubated and 1 requiring cricothyroidotomy. Six patients were intubated after more than 1 flexible laryngoscopy. The percentage of patients with involvement of the face, lips, tongue, floor of mouth, soft palate/uvula, and larynx were 12.5%, 60.2%, 39.7%, 6.8%, 17.0%, and 29.5%, respectively. Sixty-eight percent of patients with laryngeal edema were intubated. The majority of patients were treated with a corticosteroid and H1 and H2 receptor antagonists. Angioedema can occur at any time after starting ACE inhibitor use, with nearly half occurring after 1 year of use. Laryngeal involvement occurred in a minority of patients, but most of these patients were felt to require airway protection. © The Author(s) 2014.

  13. ACE Inhibitor-Induced Angioedema of the Intestine: Case Report, Incidence, Pathophysiology, Diagnosis and Management

    Directory of Open Access Journals (Sweden)

    Gavin Oudit

    2001-01-01

    Full Text Available A case report of fosinopril-induced angioedema of the intestine with a chronic course accompanied by multiple acute exacerbations is described. Angiotensin-converting enzyme (ACE inhibitor-induced angioedema of the intestine (AIAI occurs in a minority of patients taking an ACE inhibitor. The clinical presentation encompasses acute abdominal symptoms, pronounced bowel edema and ascites with occasional facial and/or oropharyngeal swelling. AIAI is diagnosed based on the temporal relationship between the symptomatic presentation and drug use, absence of alternative diagnoses including other causes of angioedema, and the prompt resolution of symptoms upon discontinuation of the ACE inhibitor. Prompt radiological investigation (abdominal computerized tomography and/or ultrasound is critical in making an early diagnosis and in preventing unnecessary surgical intervention. There is a female predominance of AIAI, which may reflect the interaction of estradiol with the various pathways involved in the pathophysiology of AIAI. Management of AIAI consists mainly of conservative measures and discontinuation of the ACE inhibitor. Angiotensin II receptor antagonists should not be considered as appropriate alternatives. Awareness and knowledge of AIAI are important because of the increasing use of ACE inhibitors, current delays in making the diagnosis, obvious management strategies once the diagnosis is made and the dysutility of alternative diagnoses, which may lead to considerable morbidity. AIAI must be considered in patients taking ACE inhibitors who develop gastrointestinal complaints irrespective of the duration of the therapy.

  14. Suspected tartrazine-induced acute urticaria/angioedema is only rarely reproducible by oral rechallenge.

    Science.gov (United States)

    Nettis, E; Colanardi, M C; Ferrannini, A; Tursi, A

    2003-12-01

    Tartrazine has been frequently linked to several diseases. However, a cause-and-effect role for tartrazine in these illnesses, especially in urticaria, has not always been established. The aim of this study is to determine the incidence of intolerance to tartrazine among subjects who experienced an acute episode of urticaria/angioedema following the ingestion of a meal or a product containing this substance. This was a retrospective study based on analysis of data of patients reported to have experienced episodes of urticaria and/or angioedema after ingesting meals or products containing tartrazine. At the first visit to the outpatients clinic, a careful anamnesis had been taken. Patients had then been submitted to the following diagnostic tests: IgE tests to common inhalant allergens and food allergens and a double-blind placebo-controlled challenge with tartrazine. A total of 102 subjects were enrolled in the study: 19 (18.6%) showed at least one relevant positive reaction to an IgE test for food allergy. Only one subject (1%) had reactions after ingestion of 5 mg of tartrazine, given on day 5. She did not have adverse reactions to placebo. This study shows that the percentage of acute urticaria and/or angioedema induced by tartrazine is very low (1%). In view of our results, we suggest that all physicians with patients who have suffered adverse reactions that could be attributed to tartrazine should also carefully evaluate other possible causes.

  15. Bluetooth security attacks comparative analysis, attacks, and countermeasures

    CERN Document Server

    Haataja, Keijo; Pasanen, Sanna; Toivanen, Pekka

    2013-01-01

    This overview of Bluetooth security examines network vulnerabilities and offers a comparative analysis of recent security attacks. It also examines related countermeasures and proposes a novel attack that works against all existing Bluetooth versions.

  16. Use of C1 Inhibitor for Angiotensin-Converting Enzyme (ACE) Inhibitor-Induced Angioedema Decreases Mechanical Ventilation Time.

    Science.gov (United States)

    Urnoski, Eric; Grillo, Angelo; Rosini, Jamie M

    2015-12-01

    Angiotensin-converting enzyme (ACE) inhibitor-induced angioedema is a rare, albeit serious emergency that can result in airway compromise and potentially death if not treated promptly. Currently, there are no agents approved by the Food and Drug Administration to target ACE inhibitor angioedema and to prevent intubation. C1 inhibitors are approved for hereditary angioedema but may show promise in alleviating inflammation associated with ACE inhibitor angioedema. A 41-year-old man presented to the emergency department with swelling of his lips a few days after starting lisinopril for hypertension. Despite receiving diphenhydramine, ranitidine, and methylprednisolone, the swelling progressed to the patient's tongue. A C1 inhibitor was ordered in an effort to prevent intubation. Before the arrival of the medication, the patient was intubated emergently for airway protection. After receipt of the C1 inhibitor, the swelling dramatically improved, and the patient was successfully extubated after less than 18 hours from presentation. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case illustrates a potential role for C1 inhibitors in the emergency setting for treating drug-induced angioedema, which may prevent or minimize mechanical ventilation time. Copyright © 2015 Elsevier Inc. All rights reserved.

  17. The role of serial physical examinations in the management of angioedema involving the head and neck: A prospective observational study

    Directory of Open Access Journals (Sweden)

    Gary Linkov

    2016-03-01

    Full Text Available Objective: To elucidate the progression of angioedema of the head and neck with routine management and to assess the utility of serial physical exams and fiberoptic laryngoscopy in its management. Methods: This study was a prospective observational research. From 2013 to 2014, a prospective observational study was conducted at a tertiary referral center. Forty patient were approached, 7 refused, 33 (18–90 years old were enrolled. Patients presented with angioedema involving the head and neck over a 12 month period were asked to participate in the study. Physical examination and fiberoptic laryngoscopy were performed at presentation and then repeated at least 1 h later. Results: Thirty-three patients with head and neck angioedema from any cause were enrolled (mean age 58, range 23–89 years. The upper lip was the most commonly involved site (58%. On reevaluation, 82% of patients reported subjective improvement in symptoms. The association between subjective improvement and the physical exam, including fiberoptic laryngoscopy findings, was statistically significant (P < 0.001. Conclusion: In stable patients with angioedema of any head and neck subsite, self-reported symptoms are associated with clinical stability or improvement as assessed by physical signs and fiberoptic laryngoscopy. Patients' symptoms may be an appropriate surrogate to monitor clinical status without the need for routine serial physical examinations or fiberoptic laryngoscopy, though further study is needed. Keywords: Angioedema, Physical examination, Fiberoptic laryngoscopy

  18. Heart Attack Coronary Artery Disease

    Science.gov (United States)

    ... our e-newsletter! Aging & Health A to Z Heart Attack Coronary Artery Disease, Angina Basic Facts & Information What ... and oxygen supply; this is what causes a heart attack. If the damaged area is small, however, your ...

  19. Thrombolytic drugs for heart attack

    Science.gov (United States)

    ... gov/ency/article/007488.htm Thrombolytic drugs for heart attack To use the sharing features on this page, ... supply blood and oxygen to the heart. A heart attack can occur if a blood clot stops the ...

  20. What Is a Heart Attack?

    Science.gov (United States)

    ... to help prevent your first heart attack. Heart-Healthy Lifestyle Changes A heart-healthy lifestyle can help prevent ... blood to flow to the heart muscle. Heart-Healthy Lifestyle Changes Treatment for a heart attack usually includes ...

  1. Automated Discovery of Mimicry Attacks

    National Research Council Canada - National Science Library

    Giffin, Jonathon T; Jha, Somesh; Miller, Barton P

    2006-01-01

    .... These systems are useful only if they detect actual attacks. Previous research developed manually-constructed mimicry and evasion attacks that avoided detection by hiding a malicious series of system calls within a valid sequence allowed by the model...

  2. Attack Trees with Sequential Conjunction

    NARCIS (Netherlands)

    Jhawar, Ravi; Kordy, Barbara; Mauw, Sjouke; Radomirović, Sasa; Trujillo-Rasua, Rolando

    2015-01-01

    We provide the first formal foundation of SAND attack trees which are a popular extension of the well-known attack trees. The SAND at- tack tree formalism increases the expressivity of attack trees by intro- ducing the sequential conjunctive operator SAND. This operator enables the modeling of

  3. Structural Learning of Attack Vectors for Generating Mutated XSS Attacks

    Directory of Open Access Journals (Sweden)

    Yi-Hsun Wang

    2010-09-01

    Full Text Available Web applications suffer from cross-site scripting (XSS attacks that resulting from incomplete or incorrect input sanitization. Learning the structure of attack vectors could enrich the variety of manifestations in generated XSS attacks. In this study, we focus on generating more threatening XSS attacks for the state-of-the-art detection approaches that can find potential XSS vulnerabilities in Web applications, and propose a mechanism for structural learning of attack vectors with the aim of generating mutated XSS attacks in a fully automatic way. Mutated XSS attack generation depends on the analysis of attack vectors and the structural learning mechanism. For the kernel of the learning mechanism, we use a Hidden Markov model (HMM as the structure of the attack vector model to capture the implicit manner of the attack vector, and this manner is benefited from the syntax meanings that are labeled by the proposed tokenizing mechanism. Bayes theorem is used to determine the number of hidden states in the model for generalizing the structure model. The paper has the contributions as following: (1 automatically learn the structure of attack vectors from practical data analysis to modeling a structure model of attack vectors, (2 mimic the manners and the elements of attack vectors to extend the ability of testing tool for identifying XSS vulnerabilities, (3 be helpful to verify the flaws of blacklist sanitization procedures of Web applications. We evaluated the proposed mechanism by Burp Intruder with a dataset collected from public XSS archives. The results show that mutated XSS attack generation can identify potential vulnerabilities.

  4. Seven Deadliest Unified Communications Attacks

    CERN Document Server

    York, Dan

    2010-01-01

    Do you need to keep up with the latest hacks, attacks, and exploits effecting Unified Communications technology? Then you need Seven Deadliest Unified Communication Attacks. This book pinpoints the most dangerous hacks and exploits specific to Unified Communications, laying out the anatomy of these attacks including how to make your system more secure. You will discover the best ways to defend against these vicious hacks with step-by-step instruction and learn techniques to make your computer and network impenetrable. Attacks featured in this book include: UC Ecosystem Attacks Insecure Endpo

  5. Emergency Department Airway Management of Severe Angioedema: A Video Review of 45 Intubations.

    Science.gov (United States)

    Driver, Brian E; McGill, John W

    2017-05-01

    Angioedema is an uncommon but important cause of airway obstruction. Emergency airway management of angioedema is difficult. We seek to describe the course and outcomes of emergency airway management for severe angioedema in our institution. We performed a retrospective, observational study of all intubations for angioedema performed in an urban academic emergency department (ED) between November 2007 and June 2015. We performed a structured review of video recordings of each intubation. We identified the methods of airway management, the success of each method, and the outcomes and complications of the effort. We identified 52 patients with angioedema who were intubated in the ED; 7 were excluded because of missing videos, leaving 45 patients in the analysis. Median time from arrival to the ED to the first intubation attempt was 33 minutes (interquartile range 17 to 79 minutes). Nasotracheal intubation was the most common first method (33/45; 73%), followed by video laryngoscopy (7/45; 16%). Two patients required attempts at more invasive airway procedures (retrograde intubation and cricothyrotomy). The intubating laryngeal mask airway was used as a rescue method 5 times after failure of multiple methods, with successful oxygenation, ventilation, and intubation through the laryngeal mask airway in all 5 patients. All patients were successfully intubated. In this series of ED patients who were intubated because of angioedema, emergency physicians used a range of methods to successfully manage the airway. These observations provide key lessons for the emergency airway management of these critical patients. Copyright © 2016 American College of Emergency Physicians. Published by Elsevier Inc. All rights reserved.

  6. The Angioedema Quality of Life Questionnaire (AE-QoL) - assessment of sensitivity to change and minimal clinically important difference.

    Science.gov (United States)

    Weller, K; Magerl, M; Peveling-Oberhag, A; Martus, P; Staubach, P; Maurer, M

    2016-08-01

    The Angioedema Quality of Life Questionnaire (AE-QoL) has recently been developed and validated as the first specific patient-reported outcome tool to assess quality of life (QoL) impairment in recurrent angioedema patients. As of yet, its sensitivity to change and minimal clinically important difference (MCID) have not been established. Recurrent angioedema patients with chronic spontaneous urticaria or hereditary angioedema were repeatedly asked to complete the AE-QoL along with the SF-12 and other anchors for QoL impairment and disease activity during routine care visits. The sensitivity to change of AE-QoL was determined by correlating changes in its scores over time with changes in the applied anchors. In addition, the MCID was determined using anchor-based and distributional criterion-based approaches. Two hundred and seventy-eight patients contributed data sets for analysis. Baseline AE-QoL values were found to correlate well with SF-12 results as well as all other applied anchors for angioedema-related QoL impairment and disease activity. In addition, AE-QoL score changes over time correlated significantly with changes in the above anchors, thus demonstrating its sensitivity to change. The MCID of the AE-QoL total score was found to be six points. The AE-QoL is a valuable tool to assess changes of QoL impairment in recurrent angioedema patients over time, including changes due to treatment. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  7. Improving Attack Graph Visualization through Data Reduction and Attack Grouping

    Energy Technology Data Exchange (ETDEWEB)

    John Homer; Ashok Varikuti; Xinming Ou; Miles A. McQueen

    2008-09-01

    Various tools exist to analyze enterprise network systems and to produce attack graphs detailing how attackers might penetrate into the system. These attack graphs, however, are often complex and difficult to comprehend fully, and a human user may find it problematic to reach appropriate configuration decisions. This paper presents methodologies that can 1) automatically identify portions of an attack graph that do not help a user to understand the core security problems and so can be trimmed, and 2) automatically group similar attack steps as virtual nodes in a model of the network topology, to immediately increase the understandability of the data. We believe both methods are important steps toward improving visualization of attack graphs to make them more useful in configuration management for large enterprise networks. We implemented our methods using one of the existing attack-graph toolkits. Initial experimentation shows that the proposed approaches can 1) significantly reduce the complexity of attack graphs by trimming a large portion of the graph that is not needed for a user to understand the security problem, and 2) significantly increase the accessibility and understandability of the data presented in the attack graph by clearly showing, within a generated visualization of the network topology, the number and type of potential attacks to which each host is exposed.

  8. Attacks on computer systems

    Directory of Open Access Journals (Sweden)

    Dejan V. Vuletić

    2012-01-01

    Full Text Available Computer systems are a critical component of the human society in the 21st century. Economic sector, defense, security, energy, telecommunications, industrial production, finance and other vital infrastructure depend on computer systems that operate at local, national or global scales. A particular problem is that, due to the rapid development of ICT and the unstoppable growth of its application in all spheres of the human society, their vulnerability and exposure to very serious potential dangers increase. This paper analyzes some typical attacks on computer systems.

  9. Presence of C1-Inhibitor Polymers in a Subset of Patients Suffering from Hereditary Angioedema

    DEFF Research Database (Denmark)

    Elenius Madsen, Daniel; Hansen, Søren Werner Karlskov; Gram, Jørgen Brodersen

    2014-01-01

    phenotypes were observed in four affected family members from one of these families. Genotyping of the families revealed that the polymerogenic mutations of two families were located in proximity to the reactive center loop insertion site in C1-inh (p.Ile271Thr and p.Ser258_Pro260del),and one mutation...... affected helix C (p.Thr167Asn). In conclusion, we demonstrate that C1-inh polymers are present in the plasma of a subgroup of HAE type I patients....

  10. Recent "phishing" attacks

    CERN Multimedia

    IT Department

    2009-01-01

    Over the last few weeks there has been a marked increase in the number of attacks on CERN made by cybercriminals. Typical attacks arrive in the form of e-mail messages purporting to come from the CERN Help Desk, Mail Service, or some similarly official-sounding entity and suggest that there is a problem with your account, such as it being over-quota. They then ask you to click on a link or to reply and give your password. Please don’t! Be cautious of any unexpected messages containing web links even if they appear to come from known contacts. If you happen to click on such a link and if your permission is requested to run or install software, always decline it. NEVER provide your password or other details if these are requested. These messages try to trick you into clicking on Web links which will help them to install malicious software on your computer, and anti-virus software cannot be relied on to detect all cases. In case of questions on this topic, you may contact mailto:helpdesk@cern.ch. CERN Comput...

  11. Drug-Induced Inhibition of Angiotensin Converting Enzyme and Dipeptidyl Peptidase 4 Results in Nearly Therapy Resistant Bradykinin Induced Angioedema: A Case Report.

    Science.gov (United States)

    Hahn, Janina; Trainotti, Susanne; Hoffmann, Thomas K; Greve, Jens

    2017-05-25

    BACKGROUND Bradykinin is an underestimated mediator of angioedema. One subgroup of bradykinin induced angioedema is angioedema triggered by treatment with angiotensin converting enzyme (ACE) inhibitors. Due to its localization in the head and neck region and its unpredictable course, it is a possibly life-threatening condition. There is not an officially approved treatment for ACE inhibitor induced angioedema. CASE REPORT We present a case of an 83-year-old woman, who presented to our ENT department because of acute swelling of the tongue. On admission, there was no pharyngeal or laryngeal edema and no dyspnea. Treatment with glucocorticoids and antihistamines had no response. The patient had ramipril as regular medication, so we assumed ACE inhibitor induced angioedema and treated consequently with C1-inhibitor (human) 1,500 IU. Nevertheless, swelling was progressive and required intubation. Even after the second specific treatment with icatibant, her angioedema subsided extremely slowly. The patient also had regular treatment with saxagliptin, a dipeptidyl peptidase 4 inhibitor, so we assumed that the simultaneous inhibition of two bradykinin degrading enzymes led to a treatment-refractory course of angioedema. CONCLUSIONS General awareness for bradykinin induced angioedema due to regular medication is limited. Our case demonstrated the importance of improving awareness and knowledge about this side effect. We need a better understanding of the pathomechanism to aid in more precise clinical diagnosis. Securing the patient's airway as well as administration of an officially approved therapy is of utmost importance. As the number of patients simultaneously treated with antihypertensive and antidiabetic drugs is likely to increase, the incidence of bradykinin mediated drug induced angioedema is likely to increase as well.

  12. Effect of omalizumab on angioedema in H1 -antihistamine-resistant chronic spontaneous urticaria patients: results from X-ACT, a randomized controlled trial.

    Science.gov (United States)

    Staubach, P; Metz, M; Chapman-Rothe, N; Sieder, C; Bräutigam, M; Canvin, J; Maurer, M

    2016-08-01

    Chronic spontaneous urticaria (CSU) severely impacts quality of life (QoL), especially in patients with wheals and angioedema. Omalizumab is approved as add-on therapy for CSU patients; however, its effect on patients who are double-positive for wheals and angioedema has not been systematically studied. The primary objective was to evaluate the efficacy of omalizumab vs placebo at week 28 using the Chronic Urticaria Quality of Life (CU-Q2oL) questionnaire. Number of angioedema-burdened days, time interval between successive angioedema episodes, disease activity, angioedema-specific and overall QoL impairment were secondary objectives. X-ACT was a phase III, randomized, double-blind study conducted in 24 centres (Germany), which selectively included CSU patients with angioedema and wheals. Patients were randomized (1 : 1) to omalizumab 300 mg or placebo (every 4 weeks up to week 24) (ClinicalTrials.gov number: NCT01723072). Of the 91 patients randomized to omalizumab (n = 44) or placebo (n = 47) at baseline, 68 completed the 28-week treatment phase (omalizumab, 35; placebo, 33). Omalizumab was superior to placebo in improving CU-Q2oL scores at week 28 (P omalizumab (0.3) vs placebo (1.1). The median time to first recurrence of angioedema was 57-63 days with omalizumab and Omalizumab significantly improved angioedema-specific QoL (P omalizumab. Omalizumab was an effective treatment option for patients with moderate-to-severe CSU symptoms and angioedema unresponsive to high doses of antihistamine treatment. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  13. Transient angioedema of small bowel secondary to intravenous iodinated contrast medium

    Directory of Open Access Journals (Sweden)

    Kirankumar N Kulkarni

    2014-01-01

    Full Text Available We report the clinical details and imaging findings of a case of transient angioedema of the small bowel following intravenous administration of non-ionic iodinated contrast material in a 17 year old female with no predisposing risk factors. Findings included long segment, symmetric, circumferential, low-density, bowel wall thickening involving the duodenum, jejunum, and most of the ileum on computed tomography scan obtained at 7 min following intravenous contrast material injection. This entity is self-limiting with a favourable clinical outcome and requires no specific treatment but only aggressive clinical monitoring.

  14. Allergic contact dermatitis mimicking angioedema due to paraphenylendiamine hypersensitivity: a case report.

    Science.gov (United States)

    Tukenmez Demirci, Gulsen; Kivanc Altunay, Ilknur; Atis, Guldehan; Kucukunal, Asli

    2012-09-01

    Active sensitization to paraphenylendiamine (PPD) and related compounds from temporary black henna tattoos has become an epidemic in the recent years. Hair dyes also include PPD like black henna tatoos which cause allergic contact dermatitis. Skin lesions of allergic contact dermatitis from PPD are mostly seen as an exudative erythema, an erythema multiforme-like eruption or a bullous contact dermatitis. We, herein, report a 27 year-old woman with an angioedema-like reaction occurring after the first exposure to hair dye who was unaware of being previously sensitized to PPD from black henna tattoo.

  15. [Facial and oropharyngeal angioedema in patient with alimentary fish allergy. Diagnosis and treatment].

    Science.gov (United States)

    Pino Rivero, V; Rodríguez Carmona, M; Iglesias González, R J; del Castillo Beneyto, F

    2007-01-01

    Vegetal or animal food can produce hipersensibility reactions IgE mediated of diverse intensity. We report the case of a 54 years old woman without previous allergic antecedents who after eating frozen fish had to go to Emergencies due to angioedema especially in face and oropharynx. The ENT exploration by fibroscopia descarted laryngeal edema but the patient showed initially respiratory symptoms so she was treated with SC adrenalina and then steroids during her admission. The diagnosis of alimentary alergia would be confirmed after by Allergology with cutaneous test prick type.

  16. Acquired Form of Angioedema of the Head and Neck Related to a Deficiency in C1-Inhibitor: A Case Report with a Review of the Literature

    Directory of Open Access Journals (Sweden)

    Bassel Hallak

    2012-01-01

    Full Text Available Angioedema related to a deficiency in the C1-inhibitor protein is characterized by its lack of response to therapies including antihistamine, steroids, and epinephrine. In the case of laryngeal edema, mortality rate is approximately 30 percent. The first case of the acquired form of angioedema related to a deficiency in C1-inhibitor was published in 1972. In our paper, we present a case of an acquired form of angioedema of the oropharyngeal region secondary to the simultaneous occurrence of two causative factors: neutralization of C1-inhibitor by an autoantibody and the use of an angiotensin convertin enzyme inhibitor.

  17. The Use of Plasma-Derived Complement C1-Esterase Inhibitor Concentrate (Berinert®) in the Treatment of Angiotensin Converting Enzyme-Inhibitor Related Angioedema

    DEFF Research Database (Denmark)

    Hermanrud, Thorbjørn; Duus, Nicolaj; Bygum, Anette

    2016-01-01

    Angioedema of the upper airways is a severe and potentially life-threatening condition. The incidence has been increasing in the past two decades, primarily due to pharmaceuticals influencing the generation or degradation of the vasoactive molecule bradykinin. Plasma-derived C1-esterase inhibitor...... concentrate is a well-established treatment option of hereditary and acquired complement C1-esterase inhibitor deficiency, which are also mediated by an increased level of bradykinin resulting in recurrent angioedema. We here present a case of severe angiotensin converting enzyme-inhibitor related angioedema...

  18. Cyber Attacks and Combat Behavior

    Directory of Open Access Journals (Sweden)

    Carataș Maria Alina

    2017-01-01

    Full Text Available Cyber terrorism is an intangible danger, a real over the corner threat in the life of individuals,organizations, and governments and is getting harder to deal with its damages. The motivations forthe cyber-attacks are different, depending on the terrorist group, from cybercrime to hacktivism,attacks over the authorities’ servers. Organizations constantly need to find new ways ofstrengthening protection against cyber-attacks, assess their cyber readiness, expand the resiliencecapacity and adopts international security regulations.

  19. Attacks on RFID Identification Systems

    Directory of Open Access Journals (Sweden)

    D. M. Mikhaylov

    2010-09-01

    Full Text Available This article is about attacks on RFID systems. Currently antivirus developers are not developing systems that protect from viruses that could exist on RFID tags. Such viruses are considered as not existing because the RFID tag memory is very small. Unfortunately such viruses exist. This article is concerned to such viruses and attacks that hackers could do using such viruses. Based on this article methods to prevent RFID-viruses attacks could be developed.

  20. Seven Deadliest Social Network Attacks

    CERN Document Server

    Timm, Carl

    2010-01-01

    Do you need to keep up with the latest hacks, attacks, and exploits effecting social networks? Then you need Seven Deadliest Social Network Attacks. This book pinpoints the most dangerous hacks and exploits specific to social networks like Facebook, Twitter, and MySpace, laying out the anatomy of these attacks including how to make your system more secure. You will discover the best ways to defend against these vicious hacks with step-by-step instruction and learn techniques to make your computer and network impenetrable. Attacks detailed in this book include: Social Networking Infrastruct

  1. Seven Deadliest Web Application Attacks

    CERN Document Server

    Shema, Mike

    2010-01-01

    Do you need to keep up with the latest hacks, attacks, and exploits effecting web applications? Then you need Seven Deadliest Web Application Attacks. This book pinpoints the most dangerous hacks and exploits specific to web applications, laying out the anatomy of these attacks including how to make your system more secure. You will discover the best ways to defend against these vicious hacks with step-by-step instruction and learn techniques to make your computer and network impenetrable. .. .. Attacks detailed in this book include: ..: ..; Cross-Site Scripting (XSS) ..; Cross-Site Request Fo

  2. The Timing of Terrorist Attacks

    DEFF Research Database (Denmark)

    Jensen, Thomas

    2016-01-01

    I use a simple optimal stopping model to derive policy relevant insights on the timing of one-shot attacks by small autonomous terrorist units or “lone wolf” individuals. A main insight is that an increase in proactive counterterrorism measures can lead to a short term increase in the number...... of attempted terrorist attacks because it makes it more risky for existing terrorist units to pursue further development of capabilities. This is consistent with the events in London in 2005 where a terrorist attack on 7 July was followed by a similar but unsuccessful attack two weeks later....

  3. Unilateral angioedema of the tongue and the floor of the mouth as a side-effect of ACE-inhibitors].

    NARCIS (Netherlands)

    de Lange, Jan; de Ruiter, M.H.T.; Smeele, L. E.

    2014-01-01

    Abstract An 80-year-old woman who was medically compromised had recurrent diffuse unilateral swelling of the tongue and the floor of the mouth. The clinical working diagnosis of angioedema as a result of the use of ACE-inhibitors (lisinopril) was made. In consultation with the cardiologist in charge

  4. Phenotype standardization of angioedema in the head and neck region caused by agents acting on the angiotensin system

    NARCIS (Netherlands)

    Wadelius, M.; Marshall, S. E.; Islander, G.; Nordang, L.; Karawajczyk, M.; Yue, Q.-Y.; Terreehorst, I.; Baranova, E. V.; Hugosson, S.; Sköldefors, K.; Pirmohamed, M.; Maitland-van der Zee, A.-H.; Alfirevic, A.; Hallberg, P.; Palmer, C. N. A.

    2014-01-01

    Angioedema is a potentially life-threatening adverse reaction to angiotensin-converting enzyme inhibitors and angiotensin receptor blockers. To study the genetic etiology of this rare adverse event, international consortia and multicenter recruitment of patients are needed. To reduce patient

  5. Angioedema adquirido autoimune de difícil controle em paciente com lúpus eritematoso sistêmico Intractable acquired autoimmune angioedema in a patient with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Vilson Furlanetto Junior

    2010-02-01

    Full Text Available O angioedema adquirido é causado por diferentes medicamentos e doenças linfoproliferativas, e tem sido raramente relacionado com a presença de doenças autoimunes. Descrevemos aqui uma paciente de 47 anos com lúpus eritematoso sistêmico (LES com envolvimento cutâneo importante que desenvolveu angioedema recorrente localizado em face incluindo lábios e pálpebras, membros superiores e tórax, não acompanhado de urticária e com dosagem do inibidor de C1 esterase reduzida. A utilização de antimaláricos, glicocorticoides e pulsoterapia com metilprednisolona associada ao uso de azatioprina não determinou melhora. A paciente utilizou também danazol sem sucesso, e apresentou resposta clínica somente após ter sido submetida a múltiplas sessões de plasmaferese, ocorrendo inclusive resolução de extenso angioedema na mucosa do trato gastrointestinal.Acquired angioedema is caused by different drugs and lymphoproliferative diseases, and rarely it has also been related to the presence of auto-immune disorders. We report the case of a 47 year old female with systemic lupus erythematosus (SLE and severe cutaneous involvement who developed recurrent localized angioedema of the face, including lips and eye lids, upper limbs, and thorax, not associated with urticaria, and with reduced levels of C1 esterase inhibitor. Treatment with antimalarials, glucocorticoids, and pulse therapy with methylprednisolone associated with azathioprine did not improve her condition. The patient was also unsuccessfully treated with danazol, and she only showed clinical response after several sessions of plasmapheresis, including resolution of the extensive edema of the gastrointestinal tract.

  6. Utility of routine laboratory testing in management of chronic urticaria/angioedema.

    Science.gov (United States)

    Tarbox, James A; Gutta, Ravi C; Radojicic, Cristine; Lang, David M

    2011-09-01

    Laboratory tests are routinely ordered to identify or rule out a cause in patients with chronic urticaria/angioedema (CUA). The results of these tests are usually within normal limits or unremarkable. To investigate the proportion of abnormal test results in patients with CUA leading to a change in management and in outcomes of care. Retrospective analysis of a random sample of adult patients with CUA from 2001-2009. Cases totaled 356: 166 with urticaria and angioedema (AE), 187 with urticaria, and 3 with only AE. Patients were predominately women (69.1%) and white (75.6%), with a mean age of 48 ± 15 years. Abnormalities were commonly seen in complete blood counts (34%) and in complete metabolic panels (9.4%). Among the 1,872 tests that were ordered, results of 319 (17%) were abnormal. Of 356 patients, 30 underwent further testing because of abnormalities in laboratory work. This represented 30 of 1,872 tests (1.60%). Only 1 patient benefited from a subsequent change in management. Laboratory testing in CUA patients referred for an Allergy and Immunology evaluation rarely lead to changes in management resulting in improved outcomes of care. Copyright © 2011 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  7. A Case of Miller Fisher Syndrome, Thromboembolic Disease, and Angioedema: Association or Coincidence?

    Science.gov (United States)

    Salehi, Nooshin; Choi, Eric D; Garrison, Roger C

    2017-01-16

    BACKGROUND Miller Fisher Syndrome is characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia, and is considered to be a variant of Guillain-Barre Syndrome. Miller Fisher Syndrome is observed in approximately 1-5% of all Guillain-Barre cases in Western countries. Patients with Miller Fisher Syndrome usually have good recovery without residual deficits. Venous thromboembolism is a common complication of Guillain-Barre Syndrome and has also been reported in Miller Fisher Syndrome, but it has generally been reported in the presence of at least one prothrombotic risk factor such as immobility. A direct correlation between venous thromboembolism and Miller Fisher Syndrome or Guillain-Barre Syndrome has not been previously described. CASE REPORT We report the case of a 32-year-old Hispanic male who presented with acute, severe thromboembolic disease and concurrently demonstrated characteristic clinical features of Miller Fisher Syndrome including ophthalmoplegia, ataxia, and areflexia. Past medical and family history were negative for thromboembolic disease, and subsequent hypercoagulability workup was unremarkable. During the course of hospitalization, the patient also developed angioedema. CONCLUSIONS We describe a possible association between Miller Fisher Syndrome, thromboembolic disease, and angioedema.

  8. Global Mapping of Cyber Attacks

    Science.gov (United States)

    2014-01-01

    Identifying factors behind countries weakness to cyber - attacks is an important step towards addressing these weaknesses at the root level. For...the lowest rates of cyber - attacks . This is surprising given the bad cyber reputation of some African countries such as Nigeria. Our research has many policy implications.

  9. [Heart-attack in pregnancy].

    Science.gov (United States)

    Výtisková, T; Suchá, D; Fučíková, Z

    To describe hear-attack on crystal meth addicted pregnant woman. Case report. Acute heart-attack during pregnancy means unexpected obstetric complication. The consequences could be fatal for the mother and the fetus. Although good delivery management and treatment could reduce morbidity and mortality to a minimum.

  10. Superposition Attacks on Cryptographic Protocols

    DEFF Research Database (Denmark)

    Damgård, Ivan Bjerre; Funder, Jakob Løvstad; Nielsen, Jesper Buus

    2011-01-01

    string model. While our protocol is classical, it is sound against a cheating unbounded quantum prover and computational zero-knowledge even if the verifier is allowed a superposition attack. Finally, we consider multiparty computation and show that for the most general type of attack, simulation based...

  11. Multiculturalism & The Charlie Hebdo Attack

    DEFF Research Database (Denmark)

    Lægaard, Sune

    2016-01-01

    The attack on Charlie Hebdo has by many been linked to multiculturalism. But it is unclear exactly how the connection between multiculturalism and the attack should be understood and whether there indeed is such a connection. The article discusses this by distinguishing between different senses o...

  12. Automated Generation of Attack Trees

    DEFF Research Database (Denmark)

    Vigo, Roberto; Nielson, Flemming; Nielson, Hanne Riis

    2014-01-01

    -prone and impracticable for large systems. Nonetheless, the automated generation of attack trees has only been explored in connection to computer networks and levering rich models, whose analysis typically leads to an exponential blow-up of the state space. We propose a static analysis approach where attack trees...

  13. Renin angiotensin system blockers-associated angioedema in the Thai population: analysis from Thai National Pharmacovigilance Database.

    Science.gov (United States)

    Win, Thet Su Zin; Chaiyakunapruk, Nathorn; Suwankesawong, Wimon; Dilokthornsakul, Piyameth; Nathisuwan, Surakit

    2015-09-01

    Renin-angiotensin-aldosterone system (RAS) blockers are commonly used for cardiovascular diseases. Currently, little information exists for the Asian population on angioedema, a rare yet serious adverse event. This study aimed to describe characteristics of RAS blockers-associated angioedema (RASBA) in Thai patients. A retrospective study using the national pharmacovigilance database of Thailand was undertaken. Cases indicating the presence of angioedema with RAS blockers uses from 1984-2011 were identified. Patient demographics, co-morbidities, concomitant drugs, information for the RAS blockers and angioedema were obtained as well as causality assessment and quality of reports. A total of 895 cases were identified. Mean age was 59.9+12.8 years and 66.5% being female. Most angioedema events (48.6%) occurred during the first week of treatment. Angiotensin converting enzyme inhibitors (87.7%) were the most commonly implicated agents followed by angiotensin receptor blockers (10.5%), aldosterone antagonist (2.1%) and direct renin inhibitor (0.2%). Out of the 895 cases incorporated in this study, 165 (18.4%) were classified as serious events and resulted in hospitalization. The overall case fatality rate was 0.4%. Respiratory disturbance occurred in 46 cases (5.1%). Patients with respiratory complications tended to be younger (53.4+13.9 vs 60.3+12.7 years old; p=0.002) and with higher frequency of allergy history (26.1% vs 14.7%; p=0.032) compared to those without respiratory complications. Based on multivariate logistic regression, the adjusted OR for history of allergy was 2.23 (95%CI: 1.04 - 4.78, p = 0.041). RASBA in Thai population occurred mostly in elderly female patients and often led to hospitalization. Since large number of patients is regularly exposed to RAS-blockers, a nationwide attempt to raise awareness of clinicians when prescribing RAS-blockers is prudent.

  14. WILD PIG ATTACKS ON HUMANS

    Energy Technology Data Exchange (ETDEWEB)

    Mayer, J.

    2013-04-12

    Attacks on humans by wild pigs (Sus scrofa) have been documented since ancient times. However, studies characterizing these incidents are lacking. In an effort to better understand this phenomenon, information was collected from 412 wild pig attacks on humans. Similar to studies of large predator attacks on humans, data came from a variety of sources. The various attacks compiled occurred in seven zoogeographic realms. Most attacks occurred within the species native range, and specifically in rural areas. The occurrence was highest during the winter months and daylight hours. Most happened under non-hunting circumstances and appeared to be unprovoked. Wounded animals were the chief cause of these attacks in hunting situations. The animals involved were typically solitary, male and large in size. The fate of the wild pigs involved in these attacks varied depending upon the circumstances, however, most escaped uninjured. Most human victims were adult males traveling on foot and alone. The most frequent outcome for these victims was physical contact/mauling. The severity of resulting injuries ranged from minor to fatal. Most of the mauled victims had injuries to only one part of their bodies, with legs/feet being the most frequent body part injured. Injuries were primarily in the form of lacerations and punctures. Fatalities were typically due to blood loss. In some cases, serious infections or toxemia resulted from the injuries. Other species (i.e., pets and livestock) were also accompanying some of the humans during these attacks. The fates of these animals varied from escaping uninjured to being killed. Frequency data on both non-hunting and hunting incidents of wild pig attacks on humans at the Savannah River Site, South Carolina, showed quantitatively that such incidents are rare.

  15. Shark Attack Project - Marine Attack at Towed Hydrophone Arrays

    National Research Council Canada - National Science Library

    Kalmijn, Adrianus J

    2005-01-01

    The original objective of the SIO Marine Attack project was to identify the electric and magnetic fields causing sharks to inflict serious damage upon the towed hydrophone arrays of US Navy submarines...

  16. The Cyber-Physical Attacker

    DEFF Research Database (Denmark)

    Vigo, Roberto

    2012-01-01

    the security properties of CPSs, as a system cannot be secured without defining the threats it is subject to. In this work an attacker scenario is presented which addresses the peculiarities of a cyber-physical adversary, and we discuss how this scenario relates to other attacker models popular in the security......The world of Cyber-Physical Systems ranges from industrial to national interest applications. Even though these systems are pervading our everyday life, we are still far from fully understanding their security properties. Devising a suitable attacker model is a crucial element when studying...

  17. Gas in Attack and Gas in Defense

    National Research Council Canada - National Science Library

    Fries, Amos A

    1919-01-01

    Carrying out a gas attack is the most technical and dangerous of war's attacks, not only to those on board the airplane that is initiating the attack, but also to those friendly troops on the ground for miles around...

  18. Social Engineering Attack Detection Model: SEADMv2

    CSIR Research Space (South Africa)

    Mouton, F

    2015-10-01

    Full Text Available and is only able to cater for social engineering attacks that use bidirectional communication. Previous research discovered that social engineering attacks can be classified into three different categories, namely attacks that utilise bidirectional...

  19. Forensics Investigation of Web Application Security Attacks

    OpenAIRE

    Amor Lazzez; Thabet Slimani

    2015-01-01

    Nowadays, web applications are popular targets for security attackers. Using specific security mechanisms, we can prevent or detect a security attack on a web application, but we cannot find out the criminal who has carried out the security attack. Being unable to trace back an attack, encourages hackers to launch new attacks on the same system. Web application forensics aims to trace back and attribute a web application security attack to its originator. This may significantly reduce the sec...

  20. Genetic attack on neural cryptography.

    Science.gov (United States)

    Ruttor, Andreas; Kinzel, Wolfgang; Naeh, Rivka; Kanter, Ido

    2006-03-01

    Different scaling properties for the complexity of bidirectional synchronization and unidirectional learning are essential for the security of neural cryptography. Incrementing the synaptic depth of the networks increases the synchronization time only polynomially, but the success of the geometric attack is reduced exponentially and it clearly fails in the limit of infinite synaptic depth. This method is improved by adding a genetic algorithm, which selects the fittest neural networks. The probability of a successful genetic attack is calculated for different model parameters using numerical simulations. The results show that scaling laws observed in the case of other attacks hold for the improved algorithm, too. The number of networks needed for an effective attack grows exponentially with increasing synaptic depth. In addition, finite-size effects caused by Hebbian and anti-Hebbian learning are analyzed. These learning rules converge to the random walk rule if the synaptic depth is small compared to the square root of the system size.

  1. Genetic attack on neural cryptography

    International Nuclear Information System (INIS)

    Ruttor, Andreas; Kinzel, Wolfgang; Naeh, Rivka; Kanter, Ido

    2006-01-01

    Different scaling properties for the complexity of bidirectional synchronization and unidirectional learning are essential for the security of neural cryptography. Incrementing the synaptic depth of the networks increases the synchronization time only polynomially, but the success of the geometric attack is reduced exponentially and it clearly fails in the limit of infinite synaptic depth. This method is improved by adding a genetic algorithm, which selects the fittest neural networks. The probability of a successful genetic attack is calculated for different model parameters using numerical simulations. The results show that scaling laws observed in the case of other attacks hold for the improved algorithm, too. The number of networks needed for an effective attack grows exponentially with increasing synaptic depth. In addition, finite-size effects caused by Hebbian and anti-Hebbian learning are analyzed. These learning rules converge to the random walk rule if the synaptic depth is small compared to the square root of the system size

  2. Panic Attacks and Panic Disorder

    Science.gov (United States)

    ... in panic attacks. For example, if a grizzly bear came after you, your body would react instinctively. ... panic disorder Major life stress, such as the death or serious illness of a loved one A ...

  3. Acutely Onset Amiodarone-Induced Angioedema in a Patient with New Atrial Fibrillation

    Directory of Open Access Journals (Sweden)

    Hossein Vakili

    2014-01-01

    Full Text Available A 50-year-old man was admitted to our emergency department due to new episode of palpitation. He had history of angioplasty of right coronary artery (RCA with drug eluting stent 2 years ago. His electrocardiogram revealed atrial fibrillation (AF. Intravenous amiodarone 150 mg during 10 minutes and then 1 mg/min infusion were started to achieve rate control and pharmacologic conversion to sinus rhythm. After 60 minutes of starting amiodarone infusion, he developed swelling of the skin around his mouth and eyes, and also mucosa of the mouth, eyes and tongue. To conclude, angioedema should be considered a rare side effect of amiodarone which is used broadly in cardiovascular field.

  4. NSAID-induced urticaria/angioedema does not evolve into chronic urticaria: a 12-year follow-up study.

    Science.gov (United States)

    Doña, I; Blanca-López, N; Torres, M J; Gómez, F; Fernández, J; Zambonino, M A; Monteseirín, F J; Canto, G; Blanca, M; Cornejo-García, J A

    2014-04-01

    Nonsteroidal anti-inflammatory drugs (NSAIDs) are the most frequent medicaments involved in drug hypersensitivity reactions, with NSAID-induced urticaria/angioedema (NIUA) being the most frequent clinical entity. The natural evolution of NIUA has been suggested to lead to chronic urticaria (CU) in an important proportion of patients, such that NIUA may therefore precede CU. Our aim was to verify whether these entities are related by following up a large cohort of patients with NIUA as well as a control group over a long period of time. The study comprised three groups: (i) patients with a confirmed history of NIUA (more than two episodes with at least two different NSAIDs or positive drug provocation tests), (ii) patients with more than two episodes of urticaria/angioedema to a single NSAID with good tolerance to a strong COX-1 inhibitor and/or evidence by in vivo tests supporting specific IgE antibodies to the drug (single NSAID-induced urticaria/angioedema, SNIUA), and (iii) controls who tolerated NSAIDs. All cases in the three groups were followed up over a period of 12 years. There were 190 patients with NIUA (64.6% female; mean age 43.71 ± 15.82 years, 110 with SNIUA, and 152 controls. At the 12-year evaluation, 12 patients with NIUA (6.15%) had developed CU over a 1- to 8-year period. Similar proportions were seen in SNIUA and controls. Nonsteroidal anti-inflammatory drugs-induced urticaria/angioedema does not seem to precede the onset of CU over the medium term. Further research including a longer follow-up is necessary to verify this observation. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  5. Analytical Characterization of Internet Security Attacks

    Science.gov (United States)

    Sellke, Sarah H.

    2010-01-01

    Internet security attacks have drawn significant attention due to their enormously adverse impact. These attacks includes Malware (Viruses, Worms, Trojan Horse), Denial of Service, Packet Sniffer, and Password Attacks. There is an increasing need to provide adequate defense mechanisms against these attacks. My thesis proposal deals with analytical…

  6. Recurrent spontaneous attacks of dizziness.

    Science.gov (United States)

    Lempert, Thomas

    2012-10-01

    This article describes the common causes of recurrent vertigo and dizziness that can be diagnosed largely on the basis of history. Ninety percent of spontaneous recurrent vertigo and dizziness can be explained by six disorders: (1) Ménière disease is characterized by vertigo attacks, lasting 20 minutes to several hours, with concomitant hearing loss, tinnitus, and aural fullness. Aural symptoms become permanent during the course of the disease. (2) Attacks of vestibular migraine may last anywhere from minutes to days. Most patients have a previous history of migraine headaches, and many experience migraine symptoms during the attack. (3) Vertebrobasilar TIAs affect older adults with vascular risk factors. Most attacks last less than 1 hour and are accompanied by other symptoms from the posterior circulation territory. (4) Vestibular paroxysmia is caused by vascular compression of the eighth cranial nerve. It manifests itself with brief attacks of vertigo that recur many times per day, sometimes with concomitant cochlear symptoms. (5) Orthostatic hypotension causes brief episodes of dizziness lasting seconds to a few minutes after standing up and is relieved by sitting or lying down. In older adults, it may be accompanied by supine hypertension. (6) Panic attacks usually last minutes, occur in specific situations, and are accompanied by choking, palpitations, tremor, heat, and anxiety. Less common causes of spontaneous recurrent vertigo and dizziness include perilymph fistula, superior canal dehiscence, autoimmune inner ear disease, otosclerosis, cardiac arrhythmia, and medication side effects. Neurologists need to venture into otolaryngology, internal medicine, and psychiatry to master the differential diagnosis of recurrent dizziness.

  7. Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency

    Directory of Open Access Journals (Sweden)

    Abdullateef Abdulkareem

    2018-01-01

    Full Text Available Acquired angioedema due to C1 inhibitor deficiency (C1INH-AAE is a rare and potentially fatal syndrome of bradykinin-mediated angioedema characterized by episodes of angioedema without urticaria. It typically manifests with nonpitting edema of the skin and edema in the gastrointestinal (GI tract mucosa or upper airway. Edema of the upper airway and tongue may lead to life-threatening asphyxiation. C1INH-AAE is typically under-diagnosed because of its rarity and its propensity to mimic more common abdominal conditions and allergic reactions. In this article, we present the case of a 62-year-old male with a history of recently diagnosed chronic lymphocytic leukemia (CLL who presented to our hospital with recurrent abdominal pain, initially suspected to have Clostridium difficile colitis and diverticulitis. He received a final diagnosis of acquired angioedema due to C1 esterase inhibitor deficiency due to concomitant symptoms of lip swelling, cutaneous nonpitting edema of his lower extremities, and complement level deficiencies. He received acute treatment with C1 esterase replacement and icatibant and was maintained on C1 esterase infusions. He also underwent chemotherapy for his underlying CLL and did not experience further recurrence of his angioedema.

  8. Approaches to the diagnosis and management of patients with a history of nonsteroidal anti-inflammatory drug-related urticaria and angioedema.

    Science.gov (United States)

    Kowalski, Marek L; Woessner, Katharine; Sanak, Marek

    2015-08-01

    Nonsteroidal anti-inflammatory drug (NSAID)-induced urticarial and angioedema reactions are among the most commonly encountered drug hypersensitivity reactions in clinical practice. Three major clinical phenotypes of NSAID-induced acute skin reactions manifesting with angioedema, urticaria, or both have been distinguished: NSAID-exacerbated cutaneous disease, nonsteroidal anti-inflammatory drug-induced urticaria/angioedema (NIUA), and single NSAID-induced urticaria and angioedema. In some patients clinical history alone might be sufficient to establish the diagnosis of a specific type of NSAID hypersensitivity, whereas in other cases oral provocation challenges are necessary to confirm the diagnosis. Moreover, classification of the type of cutaneous reaction is critical for proper management. For example, in patients with single NSAID-induced reactions, chemically nonrelated COX-1 inhibitors can be safely used. However, there is cross-reactivity between the NSAIDs in patients with NSAID-exacerbated cutaneous disease and NIUA, and thus only use of selective COX-2 inhibitors can replace the culprit drug if the chronic treatment is necessary, although aspirin desensitization will allow for chronic treatment with NSAIDs in some patients with NIUA. In this review we present a practical clinical approach to the patient with NSAID-induced urticaria and angioedema. Copyright © 2015 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  9. Angioedema as the first presentation of B-cell non-Hodgkin lymphoma--an unusual case with normal C1 esterase inhibitor level: a case report.

    Science.gov (United States)

    Gunatilake, Sonali Sihindi Chapa; Wimalaratna, Harith

    2014-08-07

    Acquired angioedema is a rare but recognized manifestation of lymphoproliferative disorders due to deficiency in C1 esterase inhibitor. Normal level of C1 esterase inhibitor proteins in association with angioedema due to lymphoproliferative disease is a rare and an uncommon finding caused by antibodies produced from the underlying disease. Antibodies cause inactivation of C1 esterase inhibitor, thus resulting in C1 esterase inhibitor dysfunction despite of normal quantity of C1 esterase inhibitor. A 50-year-old Sri Lankan male presented with first episode of angioedema without any family history. Physical examination revealed mild pallor with swelling of tongue, lips and perioral region. On investigations, erythrocyte sedimentation rate was persistently high and bone marrow with immunohistochemistry revealed infiltration with B-cell type low grade non-Hodgkin lymphoma. Computed tomography scan of the chest and abdomen showed paratracheal and subcarinal lymphadenopathy and splenomegaly, with the findings being compatible with lymphoma. He had normal C1 esterase inhibitor protein level with reduced activity and low C1q, C4 levels indicating antibodies against C1 esterase inhibitor causing dysfunctional C1 esterase inhibitor. Adult onset angioedema should prompt physicians to suspect underlying lymphoproliferative disorder despite of C1 esterase inhibitor protein level being normal. Though uncommon, presence of antibodies against C1 esterase inhibitor secondary to lymphoproliferative disorder should be considered in the presence of normal C1 esterase inhibitor protein levels with low functional capacity in the background of acquired angioedema.

  10. Detection of complex cyber attacks

    Science.gov (United States)

    Gregorio-de Souza, Ian; Berk, Vincent H.; Giani, Annarita; Bakos, George; Bates, Marion; Cybenko, George; Madory, Doug

    2006-05-01

    One significant drawback to currently available security products is their inabilty to correlate diverse sensor input. For instance, by only using network intrusion detection data, a root kit installed through a weak username-password combination may go unnoticed. Similarly, an administrator may never make the link between deteriorating response times from the database server and an attacker exfiltrating trusted data, if these facts aren't presented together. Current Security Information Management Systems (SIMS) can collect and represent diverse data but lack sufficient correlation algorithms. By using a Process Query System, we were able to quickly bring together data flowing from many sources, including NIDS, HIDS, server logs, CPU load and memory usage, etc. We constructed PQS models that describe dynamic behavior of complicated attacks and failures, allowing us to detect and differentiate simultaneous sophisticated attacks on a target network. In this paper, we discuss the benefits of implementing such a multistage cyber attack detection system using PQS. We focus on how data from multiple sources can be combined and used to detect and track comprehensive network security events that go unnoticed using conventional tools.

  11. Mitigating Higher Ed Cyber Attacks

    Science.gov (United States)

    Rogers, Gary; Ashford, Tina

    2015-01-01

    In this presentation we will discuss the many and varied cyber attacks that have recently occurred in the higher ed community. We will discuss the perpetrators, the victims, the impact and how these institutions have evolved to meet this threat. Mitigation techniques and defense strategies will be covered as will a discussion of effective security…

  12. Terrorist attacks escalate in frequency and fatalities preceding highly lethal attacks.

    Science.gov (United States)

    Martens, Andy; Sainudiin, Raazesh; Sibley, Chris G; Schimel, Jeff; Webber, David

    2014-01-01

    Highly lethal terrorist attacks, which we define as those killing 21 or more people, account for 50% of the total number of people killed in all terrorist attacks combined, yet comprise only 3.5% of terrorist attacks. Given the disproportionate influence of these incidents, uncovering systematic patterns in attacks that precede and anticipate these highly lethal attacks may be of value for understanding attacks that exact a heavy toll on life. Here we examined whether the activity of terrorist groups escalates--both in the number of people killed per attack and in the frequency of attacks--leading up to highly lethal attacks. Analyses of terrorist attacks drawn from a state-of-the-art international terrorism database (The Global Terrorism Database) showed evidence for both types of escalation leading up to highly lethal attacks, though complexities to the patterns emerged as well. These patterns of escalation do not emerge among terrorist groups that never commit a highly lethal attack.

  13. Analysis of related factors of orolingual angioedema after rt-PA intravenous thrombolytic therapy.

    Science.gov (United States)

    Wang, Y-X; Li, Y-Q; Chen, Y; Zhang, C-H; Dong, Z; Wang, Z; Zhao, S-N; Li, C-H; Zhang, P-L

    2018-03-01

    Orolingual angioedema (OA) is a rare clinical complication with a potentially fatal risk that occurs after the intravenous application of alteplase (rt-PA) in patients with acute ischemic stroke. The purpose of this work is to investigate the related factors of OA in patients with acute ischemic stroke after the administration of intravenous thrombolytic therapy, to improve the predictive ability of OA during intravenous thrombolytic therapy, and to reduce the prevalence of complications. We recruited 1223 cases of patients with acute ischemic stroke that were treated in the Department of Neurology No. 4 of the Tianjin Huanhu Hospital from June 2014 to April 2015. The clinical manifestations of rt-PA related OA were recorded, the clinical prevalence was counted, related factors of OA after intravenous thrombolytic therapy were analyzed, and the risk assessment of rt-PA related OA was conducted. 14 cases of patients developed OA, with a prevalence rate of 1.14%. Among them, 5 had a history of urticaria, 4 of drug allergy, and 3 of food allergy. Among the 14 cases of patients, 10 developed OA in the process of intravenous thrombolysis and 4 after intravenous thrombolysis, 12 showed lip edema, 9 showed extensive swelling of tongue, 3 showed swelling of lateral tongue, 3 were complicated by respiratory distress, 10 showed infarction in the middle cerebral artery territory, and 6 had previously been given oral ACEI drugs. Orolingual angioedema is a rare complication that occurs after rt-PA intravenous thrombolytic therapy; when serious, it may endanger a patient's life. If patients take an oral hypotension such as ACEI drugs before the onset of OA, they have a history of allergies, or the lesion is an infraction in the dominated area of the middle cerebral artery, the risk of OA after rt-PA intravenous thrombolytic therapy will be increased. The prevalence of OA should be monitored during the rt-PA intravenous thrombolytic therapy process; timely detection and early

  14. Effectiveness of omalizumab in a patient with a life-threatening episode of bronchospasm and larynx angioedema after exposure to house dust.

    Science.gov (United States)

    Kupryś-Lipińska, Izabela; Korczyńska, Paulina; Tworek, Damian; Kuna, Piotr

    2014-02-01

    Omalizumab is a monoclonal antibody against IgE, nowadays approved for the treatment of persistent severe (EU) or moderate-to severe (USA) IgE-mediated asthma but there is also some evidence (case reports and four published clinical trials) on the effectiveness of this medication in urticaria and angioedema. The case of a 42-year-old woman suffering from severe allergic asthma and severe chronic urticaria with concomitant angioedema is presented in the article. She had a life-threatening episode of bronchospasm and larynx edema after exposure to house dust recorded in her medical history. The patient did not respond to standard therapy. The improvement in asthma control and remission of chronic urticaria and angioedema was achieved after introducing the therapy with omalizumab.

  15. Type I anaphylactic reaction due to contrast induced angioedema causing neck swelling: the role of sitting fiberoptic bronchoscopy in emergent intubation

    Directory of Open Access Journals (Sweden)

    Ali Dabbagh

    2016-07-01

    Full Text Available Contrast induced angioedema is a rapidly progressive state involving a number of organ systems including the upper airway tract; which is usually a type I anaphylactic reaction also known as immediate hypersensitivity reaction. Prompt preservation of the respiratory tract is the cornerstone of this situation. The use of fiberoptic bronchoscope for tracheal intubation though very helpful, has some special considerations due to the anatomic distortions created by edema.This manuscript describes a patient with contrast induced angioedema managed successfully. Serum levels of IgE were highly increased during the first hours after the event; while serum levels of complement were normal. However, rapid airway management and prophylactic intubation saved the patient and prevented the possible aftermath of airway obstruction.Keywords: airway management; type I anaphylactic reaction, angioedema; fiberoptic bronchoscope.Conflict of interest: none of the authors has any conflict of interest.

  16. A computer network attack taxonomy and ontology

    CSIR Research Space (South Africa)

    Van Heerden, RP

    2012-01-01

    Full Text Available taxonomy and ontology RP van Heerden1,2, B Irwin2, ID Burke1, L Leenen1 1CSIR, Pretoria, South Africa 2Rhodes University, Grahamstown, South Africa Keywords/ Key Phrases: Network Attack, Network Attack Classification, Taxonomy, Ontology, Attack... Scenario rvheerden@csir.co.za b.irwin@ru.ac.za iburke@csir.co.za lleenen@csir.co.za Abstract: Computer network attacks differ in the motivation of the entity behind the attack, the execution and the end result. The diversity of attacks has a...

  17. Protecting Cryptographic Memory against Tampering Attack

    DEFF Research Database (Denmark)

    Mukherjee, Pratyay

    . In practice such attacks can be executed easily, e.g. by heating the device, as substantiated by numerous works in the past decade. Tampering attacks are a class of such physical attacks where the attacker can change the memory/computation, gains additional (non-black-box) knowledge by interacting...... with the faulty device and then tries to break the security. Prior works show that generically approaching such problem is notoriously difficult. So, in this dissertation we attempt to solve an easier question, known as memory-tampering, where the attacker is allowed tamper only with the memory of the device......In this dissertation we investigate the question of protecting cryptographic devices from tampering attacks. Traditional theoretical analysis of cryptographic devices is based on black-box models which do not take into account the attacks on the implementations, known as physical attacks...

  18. Being active after a heart attack (image)

    Science.gov (United States)

    ... best activity when you start exercising after a heart attack. Start slowly, and increase the amount of time ... best activity when you start exercising after a heart attack. Start slowly, and increase the amount of time ...

  19. Using an ontology for network attack planning

    CSIR Research Space (South Africa)

    Van Heerden, R

    2016-09-01

    Full Text Available The modern complexity of network attacks and their counter-measures (cyber operations) requires detailed planning. This paper presents a Network Attack Planning ontology which is aimed at providing support for planning such network operations within...

  20. Social engineering attack examples, templates and scenarios

    CSIR Research Space (South Africa)

    Mouton, Francois

    2016-06-01

    Full Text Available link. A social engineering attack targets this weakness by using various manipulation techniques to elicit sensitive information. The field of social engineering is still in its early stages with regard to formal definitions, attack frameworks...

  1. Understand Your Risk of Heart Attack

    Science.gov (United States)

    ... Heart-Health Basics Reducing your risk starts with smart choices. If you smoke, stop. The American Heart ... a Second Heart Attack | Spanish Cardiac Rehab Referral Card | Spanish Heart Attack Warning Signs: Patient sheet | Infographic | ...

  2. Can Vitamins Help Prevent a Heart Attack?

    Science.gov (United States)

    ... vitamins help prevent a heart attack? Can taking vitamins help prevent heart disease or a heart attack? Answers ... M.D. It's not yet clear if taking vitamins can reduce your risk of developing heart disease ...

  3. Peacetime Use of Computer Network Attack

    National Research Council Canada - National Science Library

    Busby, Daniel

    2000-01-01

    .... PDD-63 alerts the nation to prepare for impending cyber attacks. This paper examines the nature, scale, and likelihood of cyber attacks posited in PDD-63 and finds that the country does not face an imminent "electronic Pearl Harbor...

  4. Quantifying Shannon's work function for cryptanalytic attacks

    NARCIS (Netherlands)

    van Son, R.J.J.H.

    2010-01-01

    Attacks on cryptographic systems are limited by the available computational resources. A theoretical understanding of these resource limitations is needed to evaluate the security of cryptographic primitives and procedures. This study uses an Attacker versus Environment game formalism based on

  5. Stochastic Model of TCP SYN Attacks

    Directory of Open Access Journals (Sweden)

    Simona Ramanauskaitė

    2011-08-01

    Full Text Available A great proportion of essential services are moving into internet space making the threat of DoS attacks even more actual. To estimate the real risk of some kind of denial of service (DoS attack in real world is difficult, but mathematical and software models make this task easier. In this paper we overview the ways of implementing DoS attack models and offer a stochastic model of SYN flooding attack. It allows evaluating the potential threat of SYN flooding attacks, taking into account both the legitimate system flow as well as the possible attack power. At the same time we can assess the effect of such parameters as buffer capacity, open connection storage in the buffer or filte­ring efficiency on the success of different SYN flooding attacks. This model can be used for other type of memory depletion denial of service attacks.Article in Lithuanian

  6. Computational modeling of tracheal angioedema due to swelling of the submucous tissue layer.

    Science.gov (United States)

    Gou, Kun; Pence, Thomas J

    2017-10-01

    Angioedema is a tissue-swelling pathology due to rapid change in soft tissue fluid content. Its occurrence in the trachea is predominantly localized to the soft mucous tissue that forms the innermost tracheal layer. The biomechanical consequences, such as airway constriction, are dependent upon the ensuing mechanical interactions between all of the various tissues that comprise the tracheal tube. We model the stress interactions by treating the trachea organ as a three-tissue system consisting of swellable mucous in conjunction with nonswelling cartilage and nonswelling trachealis musculature. Hyperelastic constitutive modeling is used by generalizing the standard anisotropic, incompressible soft tissue framework to incorporate the swelling effect. Finite element stress analysis then proceeds with swelling of the mucous layer providing the driving factor for the mechanical analysis. The amount of airway constriction is governed by the mechanical interaction between the three predominant tissue types. The detailed stress analysis indicates the presence of stress concentrations near the various tissue junctions. Because of the tissue's nonlinear mechanical behavior, this can lead to material stiffness fluctuations as a function of location on the trachea. Patient specific modeling is presented. The role of the modeling in the interpretation of diagnostic procedures and the assessment of therapies is discussed. Copyright © 2017 John Wiley & Sons, Ltd.

  7. Defective glycosylation of coagulation factor XII underlies hereditary angioedema type III

    Science.gov (United States)

    Björkqvist, Jenny; de Maat, Steven; Lewandrowski, Urs; Di Gennaro, Antonio; Oschatz, Chris; Schönig, Kai; Nöthen, Markus M.; Drouet, Christian; Braley, Hal; Nolte, Marc W.; Sickmann, Albert; Panousis, Con; Maas, Coen; Renné, Thomas

    2015-01-01

    Hereditary angioedema type III (HAEIII) is a rare inherited swelling disorder that is associated with point mutations in the gene encoding the plasma protease factor XII (FXII). Here, we demonstrate that HAEIII-associated mutant FXII, derived either from HAEIII patients or recombinantly produced, is defective in mucin-type Thr309-linked glycosylation. Loss of glycosylation led to increased contact-mediated autoactivation of zymogen FXII, resulting in excessive activation of the bradykinin-forming kallikrein-kinin pathway. In contrast, both FXII-driven coagulation and the ability of C1-esterase inhibitor to bind and inhibit activated FXII were not affected by the mutation. Intravital laser-scanning microscopy revealed that, compared with control animals, both F12–/– mice reconstituted with recombinant mutant forms of FXII and humanized HAEIII mouse models with inducible liver-specific expression of Thr309Lys-mutated FXII exhibited increased contact-driven microvascular leakage. An FXII-neutralizing antibody abolished bradykinin generation in HAEIII patient plasma and blunted edema in HAEIII mice. Together, the results of this study characterize the mechanism of HAEIII and establish FXII inhibition as a potential therapeutic strategy to interfere with excessive vascular leakage in HAEIII and potentially alleviate edema due to other causes. PMID:26193639

  8. Network Attack Reference Data Set

    Science.gov (United States)

    2004-12-01

    fingerprinting tools include QueSO [10] (literally translates to “what OS”) and nmap [11], however there are a number of additional tools available for...Network Attack Reference Data Set J. McKenna and J. Treurniet Defence R&D Canada √ Ottawa TECHNICAL...collection of information is estimated to average 1 hour per response, including the time for reviewing instructions, searching existing data sources

  9. Biometrics Evaluation under Spoofing Attacks

    OpenAIRE

    Chingovska, Ivana; Anjos, André; Marcel, Sébastien

    2014-01-01

    While more accurate and reliable than ever, the trustworthiness of biometric verification systems is compromised by the emergence of spoofing attacks. Responding to this threat, numerous research publications address isolated spoofing detection, resulting in efficient counter-measures for many biometric modes. However, an important, but often overlooked issue regards their engagement into a verification task and how to measure their impact on the verification systems themselves. A novel evalu...

  10. Survey of Sybil Attacks in Social Networks

    OpenAIRE

    Gunturu, Rupesh

    2015-01-01

    This paper reviews the Sybil attack in social networks, which has the potential to compromise the whole distributed network. In the Sybil attack, the malicious user claims multiple identities to compromise the network. Sybil attacks can be used to change the overall ranking in voting applications, bad-mouth an opinion, access resources or to break the trust mechanism behind a P2P network. In this paper, different defense mechanisms used to mitigate Sybil attacks are also reviewed.

  11. Cyberprints: Identifying Cyber Attackers by Feature Analysis

    Science.gov (United States)

    Blakely, Benjamin A.

    2012-01-01

    The problem of attributing cyber attacks is one of increasing importance. Without a solid method of demonstrating the origin of a cyber attack, any attempts to deter would-be cyber attackers are wasted. Existing methods of attribution make unfounded assumptions about the environment in which they will operate: omniscience (the ability to gather,…

  12. Attacks and countermeasures on AES and ECC

    DEFF Research Database (Denmark)

    Tange, Henrik; Andersen, Birger

    2013-01-01

    is foreseeable while the rounds are performed. ECC (Elliptic Curve Cryptography) is used as a public key crypto system with the key purpose of creating a private shared between two participants in a communication network. Attacks on ECC include the Pohlig-Hellman attack and the Pollard's rho attack. Furthermore...

  13. Attacks and countermeasures on AES and ECC

    DEFF Research Database (Denmark)

    Tange, Henrik; Andersen, Birger

    2013-01-01

    AES (Advanced Encryption Standard) is widely used in LTE and Wi-Fi communication systems. AES has recently been exposed to new attacks which have questioned the overall security of AES. The newest attack is a so called biclique attack, which is using the fact that the content of the state array...

  14. The Value of Attack-Defence Diagrams

    NARCIS (Netherlands)

    Hermanns, H.; Krämer, Julia; Krčál, Jan; Stoelinga, Mariëlle Ida Antoinette; Piessens, Frank; Viganò, Luca

    Success or failure of attacks on high-security systems, such as hacker attacks on sensitive data, depend on various situational conditions, including the timing and success chances of single attack steps, and concurrent countermeasures of the defender. With the existing state-of-the-art modelling

  15. Automated classification of computer network attacks

    CSIR Research Space (South Africa)

    Van Heerden, R

    2013-11-01

    Full Text Available In this paper we demonstrate how an automated reasoner, HermiT, is used to classify instances of computer network based attacks in conjunction with a network attack ontology. The ontology describes different types of network attacks through classes...

  16. Angiotensin-Converting Enzyme Inhibitor-Induced Angioedema of the Small Bowel-A Surgical Abdomen Mimic.

    Science.gov (United States)

    Bloom, Adam S; Schranz, Craig

    2015-06-01

    Angioedema is an infrequent complication of the use of angiotensin-converting enzyme inhibitors (ACEi) that has an incidence of up to 0.5%. The oropharynx is most commonly affected. Angioedema of the small bowel is a much rarer occurrence; it uniformly presents with abdominal pain of variable duration. A 51-year-old man presented to the emergency department (ED) with generalized abdominal pain, emesis, diarrhea, and bloating. Medical history was significant for hypertension and medications included captopril, metoprolol and aspirin. Vital signs and laboratory tests were unremarkable. Due to the presence of significant abdominal tenderness with guarding on examination, a FAST (focused assessment with ultrasound in trauma) examination was performed and revealed free fluid in the abdomen. A computed tomography scan of the abdomen was quickly obtained, which revealed a large amount of simple-appearing free fluid within the abdomen and mucosal edema throughout the small bowel. The patient underwent an emergent diagnostic laparoscopy and was ultimately diagnosed with angioedema of the small bowel, deemed secondary to captopril usage. Captopril was discontinued and symptoms gradually resolved with supportive care. When imaging is obtained in cases such as this one, small bowel submucosal edema and ascites are often present. Supportive care and cessation of ACEi usage are the cornerstones of treatment. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: In patients on ACEi, it is important to keep this diagnosis in mind to potentially avoid an unneeded surgical intervention, as the condition is self-limiting and can be treated primarily with supportive measures. Published by Elsevier Inc.

  17. Calculating Adversarial Risk from Attack Trees: Control Strength and Probabilistic Attackers

    NARCIS (Netherlands)

    Pieters, Wolter; Davarynejad, Mohsen

    2015-01-01

    Attack trees are a well-known formalism for quantitative analysis of cyber attacks consisting of multiple steps and alternative paths. It is possible to derive properties of the overall attacks from properties of individual steps, such as cost for the attacker and probability of success. However, in

  18. Facial Oedema Is Not Always Angioedema: A Case of Spontaneous Pneumomediastinum with Subcutaneous Emphysema during COPD Exacerbation

    Directory of Open Access Journals (Sweden)

    Sarah Damanti

    2015-10-01

    Full Text Available We report a case of acute facial oedema in an elderly hospitalized patient which was initially misdiagnosed as angioedema secondary to antibiotics in a patient with an allergic diathesis. We describe the differential aetiologies and then the true cause of the oedema, which was an uncommon complication of a very common condition in the elderly: a pneumomediastinum with subcutaneous emphysema probably due to rupture of an emphysematous lung bulla during chronic obstructive pulmonary disease (COPD exacerbation. Lastly, we focus on the therapeutic procedures instituted for the treatment of the pneumomediastinum.

  19. Whispering through DDoS attack

    OpenAIRE

    Miralem Mehic; Jiri Slachta; Miroslav Voznak

    2016-01-01

    Denial of service (DoS) attack is an attempt of the attacker to disable victim's machine by depleting network or computing resources. If this attack is performed with more than one machine, it is called distributed denial of service (DDoS) attack. Covert channels are those channels which are used for information transmission even though they are neither designed nor intended to transfer information at all. In this article, we investigated the possibility of using of DDoS attack for purposes o...

  20. Script-viruses Attacks on UNIX OS

    Directory of Open Access Journals (Sweden)

    D. M. Mikhaylov

    2010-06-01

    Full Text Available In this article attacks on UNIX OS are considered. Currently antivirus developers are concentrated on protecting systems from viruses that are most common and attack popular operating systems. If the system or its components are not often attacked then the antivirus products are not protecting these components as it is not profitable. The same situation is with script-viruses for UNIX OS as most experts consider that it is impossible for such viruses to get enough rights to attack. Nevertheless the main conclusion of this article is the fact that such viruses can be very powerful and can attack systems and get enough rights.

  1. Stochastic Model of TCP SYN Attacks

    OpenAIRE

    Simona Ramanauskaitė; Antanas Čenys

    2011-01-01

    A great proportion of essential services are moving into internet space making the threat of DoS attacks even more actual. To estimate the real risk of some kind of denial of service (DoS) attack in real world is difficult, but mathematical and software models make this task easier. In this paper we overview the ways of implementing DoS attack models and offer a stochastic model of SYN flooding attack. It allows evaluating the potential threat of SYN flooding attacks, taking into account both...

  2. NETWORK SECURITY ATTACKS. ARP POISONING CASE STUDY

    Directory of Open Access Journals (Sweden)

    Luminiţa DEFTA

    2010-12-01

    Full Text Available Arp poisoning is one of the most common attacks in a switched network. A switch is a network device that limits the ability of attackers that use a packet sniffer to gain access to information from internal network traffic. However, using ARP poisoning the traffic between two computers can be intercepted even in a network that uses switches. This method is known as man in the middle attack. With this type of attack the affected stations from a network will have invalid entries in the ARP table. Thus, it will contain only the correspondence between the IP addresses of the stations from the same network and a single MAC address (the station that initiated the attack. In this paper we present step by step the initiation of such an attack in a network with three computers. We will intercept the traffic between two stations using the third one (the attacker.

  3. Whispering through DDoS attack

    Directory of Open Access Journals (Sweden)

    Miralem Mehic

    2016-03-01

    Full Text Available Denial of service (DoS attack is an attempt of the attacker to disable victim's machine by depleting network or computing resources. If this attack is performed with more than one machine, it is called distributed denial of service (DDoS attack. Covert channels are those channels which are used for information transmission even though they are neither designed nor intended to transfer information at all. In this article, we investigated the possibility of using of DDoS attack for purposes of hiding data or concealing the existing covert channel. In addition, in this paper we analyzed the possibility of detection of such covert communication with the well-known statistical method. Also, we proposed the coordination mechanisms of the attack which may be used. A lot of research has been done in order to describe and prevent DDoS attacks, yet research on steganography on this field is still scarce.

  4. Attack Tree Generation by Policy Invalidation

    DEFF Research Database (Denmark)

    Ivanova, Marieta Georgieva; Probst, Christian W.; Hansen, Rene Rydhof

    2015-01-01

    Attacks on systems and organisations increasingly exploit human actors, for example through social engineering, complicating their formal treatment and automatic identification. Formalisation of human behaviour is difficult at best, and attacks on socio-technical systems are still mostly identified...... through brainstorming of experts. In this work we formalize attack tree generation including human factors; based on recent advances in system models we develop a technique to identify possible attacks analytically, including technical and human factors. Our systematic attack generation is based...... on invalidating policies in the system model by identifying possible sequences of actions that lead to an attack. The generated attacks are precise enough to illustrate the threat, and they are general enough to hide the details of individual steps....

  5. Network Protection Against DDoS Attacks

    Directory of Open Access Journals (Sweden)

    Petr Dzurenda

    2015-03-01

    Full Text Available The paper deals with possibilities of the network protection against Distributed Denial of Service attacks (DDoS. The basic types of DDoS attacks and their impact on the protected network are presented here. Furthermore, we present basic detection and defense techniques thanks to which it is possible to increase resistance of the protected network or device against DDoS attacks. Moreover, we tested the ability of current commercial Intrusion Prevention Systems (IPS, especially Radware DefensePro 6.10.00 product against the most common types of DDoS attacks. We create five scenarios that are varied in type and strength of the DDoS attacks. The attacks intensity was much greater than the normal intensity of the current DDoS attacks.

  6. SQL Injection Attacks and Defense

    CERN Document Server

    Clarke, Justin

    2012-01-01

    SQL Injection Attacks and Defense, First Edition: Winner of the Best Book Bejtlich Read Award "SQL injection is probably the number one problem for any server-side application, and this book unequaled in its coverage." -Richard Bejtlich, Tao Security blog SQL injection represents one of the most dangerous and well-known, yet misunderstood, security vulnerabilities on the Internet, largely because there is no central repository of information available for penetration testers, IT security consultants and practitioners, and web/software developers to turn to for help. SQL Injection Att

  7. The impact of age and gender on reporting of cough and angioedema with RAS inhibitors: A case/non-case in VigiBase

    NARCIS (Netherlands)

    Alharbi, Fawaz F.; Kholod, Anzhelika A.V.; Souverein, Patrick C.; Meyboom, Ron H.; de Groot, Mark; De Boer, Anthonius; Klungel, Olaf H.

    2016-01-01

    Background: Little is known about the effect of age and gender on reporting of cough/ angioedema with renin angiotensin system (RAS) inhibitors (angiotensin- converting enzyme (ACE) inhibitors, angiotensin receptor blockers (ARBs) and aliskiren, a direct renin inhibitor (DRI). Objectives: To assess

  8. Coronary Artery Dissection: Not Just a Heart Attack

    Science.gov (United States)

    ... Aneurysm More Coronary Artery Dissection: Not Just a Heart Attack Updated:Mar 15,2018 Sometimes a heart attack ... Disease Go Red For Women Types of aneurysms Heart Attack • Home • About Heart Attacks Acute Coronary Syndrome (ACS) ...

  9. The impact of age and sex on the reporting of cough and angioedema with renin-angiotensin system inhibitors: a case/noncase study in VigiBase.

    Science.gov (United States)

    Alharbi, Fawaz F; Kholod, Anzhelika A V; Souverein, Patrick C; Meyboom, Ronald H; de Groot, Mark C H; de Boer, Anthonius; Klungel, Olaf H

    2017-12-01

    The purpose of this study was to assess the impact of age and sex on the reporting of cough and angioedema related to renin-angiotensin system (RAS) inhibitors. A case/noncase study was performed in VigiBase. Two case groups were identified, reports of cough and reports of angioedema, and noncases were all reports of all other adverse events. Logistic regression analysis was used to assess the association between reporting of cough and angioedema with each class of RAS inhibitors stratified by age/sex and to control for confounding. The reporting of cough with angiotensin-converting enzyme (ACE) inhibitors was significantly higher in women than in men [adjusted reporting odds ratio (ROR): 44.0, 95% CI (43.2-44.8) for women vs. 29.2, 95% CI (28.5-29.9) for men]. There was no difference in reporting of cough linked to angiotensin receptor blockers (ARBs) and aliskiren between men and women. In contrast, the reporting of angioedema with ACE inhibitors and ARBs was significantly higher in men than in women, but for aliskiren, women had a significantly higher ROR than men [adjusted ROR: 5.20, 95% CI (4.18-6.46) for women vs. 3.04, 95% CI (2.30-4.02) for men]. The reporting of cough with ACE inhibitors was increased with age until reaching a plateau at middle adulthood (40-59 years) and the reporting of angioedema with ACE inhibitors was increased with age until elderly (60-79 years). Age had only a slight effect on the reporting of cough and angioedema with ARBs and aliskiren. Both age and sex have substantial effects on the reporting of cough and angioedema with RAS inhibitors and in particular ACE inhibitors. Further study is needed to determine whether these differences mainly express different adverse drug reaction risks in subgroups or also can be explained by factors influencing reporting. © 2017 The Authors. Fundamental & Clinical Pharmacology published by John Wiley & Sons Ltd on behalf of Société Française de Pharmacologie et de Thérapeutique.

  10. The burden of angioedema on United States emergency departments: 2006-2010.

    Science.gov (United States)

    Smith, Aaron; Ray, Meredith; Jain, Nikhita; Zhang, Hongmei; Sebelik, Merry

    2017-04-01

    Angioedema (AE) is a condition that may prompt a visit to an emergency department (ED), and can quickly progress to airway obstruction. To optimize treatment of AE, it is necessary to understand epidemiology and practice patterns. This study measured the magnitude of AE ED visits and characterized demographics, management, frequency of airway interventions, and mortality. Analysis of two national data sets. From the Nationwide Emergency Department Sample and National Hospital Ambulatory Medical Care Survey, we identified all patients presenting from 2006 to 2010 with a primary diagnosis of AE, characterized by the International Classification of Diseases, Ninth Edition, Clinical Modification code 995.1. The discharges were weighted and stratified by comorbidities, age, treatments, and region. χ 2 , t test, and linear regression were employed for comparisons. Total discharges increased from 87,481 (29.3 of 100,000 people) to 111,116 (35.8 of 100,000 people). More females were afflicted (57%), and 41.1% were African American. The majority (83%) of patients were discharged from the ED. Twelve percent of cases were attributed to antihypertensive adverse reaction, and these patients were older (P < .0001, odds ratio [OR] = 1.02), and had more comorbidities (P < .0001, OR = 5.66), hospital admissions (P < .0001, OR = 4.83), and intubations (P < .03, OR = 2.07). Overall, patients required intubation infrequently (<1%) and mortality was low (0.08%). The AE burden on EDs has increased over time. Patients with adverse reactions to antihypertensives are older, have more comorbidities, and require admission and intubation more frequently. Further investigation is needed to better delineate causation and outcome predictors, and to understand regional practice variance. 2c. Laryngoscope, 127:828-834, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  11. Acute phase inflammatory markers in patients with non-steroidal anti-inflammatory drugs (NSAIDs)-induced acute urticaria/angioedema and after aspirin challenge.

    Science.gov (United States)

    Kasperska-Zając, A; Grzanka, A; Czecior, E; Misiolek, M; Rogala, B; Machura, E

    2013-08-01

    Active chronic urticaria, identified as a mast cell- and basophil-dependent inflammatory disorder of the skin is able to elicit acute phase response (APR). However, systemic inflammatory response in different types of urticaria is poorly characterized. To determine APR pattern in a clearly defined group of patients with acute urticaria and/or angioedema - induced by NSAIDs. Plasma IL-6 and serum C-reactive protein (CRP) concentrations were studied in 17 patients with NSAIDs-induced acute urticaria/angioedema (NSAIDsAU) and in 20 healthy controls. Eleven patients who used NSAIDs were presented at the emergency room with acute urticaria/angioedema while the remaining six manifested the symptoms during the aspirin challenge test. Patients were examined in a dynamic manner: during the acute phase, and next, after subsidence of the symptoms. CRP and IL-6 concentrations increased significantly in patients with NSAIDsAU as compared with their asymptomatic period and the healthy subjects. In addition, NSAIDsAU patients showed elevated concentration of the biomarkers following aspirin provocation with the baseline values recovered in the asymptomatic period. These results indicate that an acute systemic inflammatory response is activated in patients with NSAIDs-induced urticaria and/or angioedema. The study supports the evidence proving that up-regulation of CRP and IL-6 in urticaria/angioedema does not necessarily reflect any concomitant infection or other inflammatory processes, but may be due to the disease itself. © 2012 The Authors. Journal of the European Academy of Dermatology and Venereology © 2012 European Academy of Dermatology and Venereology.

  12. Model checking exact cost for attack scenarios

    DEFF Research Database (Denmark)

    Aslanyan, Zaruhi; Nielson, Flemming

    2017-01-01

    . However, current model checking does not encompass the exact cost analysis of an attack, which is standard for attack trees. Our first contribution is the logic erPCTL with cost-related operators. The extended logic allows to analyse the probability of an event satisfying given cost bounds and to compute......Attack trees constitute a powerful tool for modelling security threats. Many security analyses of attack trees can be seamlessly expressed as model checking of Markov Decision Processes obtained from the attack trees, thus reaping the benefits of a coherent framework and a mature tool support...... the exact cost of an event. Our second contribution is the model checking algorithm for erPCTL. Finally, we apply our framework to the analysis of attack trees....

  13. Securing internet by eliminating DDOS attacks

    Science.gov (United States)

    Niranchana, R.; Gayathri Devi, N.; Santhi, H.; Gayathri, P.

    2017-11-01

    The major threat caused to the authorised usage of Internet is Distributed Denial of Service attack. The mechanisms used to prevent the DDoS attacks are said to overcome the attack’s ability in spoofing the IP packets source addresses. By utilising Internet Protocol spoofing, the attackers cause a consequential load over the networks destination for policing attack packets. To overcome the IP Spoofing level on the Internet, We propose an Inter domain Packet Filter (IPF) architecture. The proposed scheme is not based on global routing information. The packets with reliable source addresses are not rejected, the IPF frame work works in such a manner. The spoofing capability of attackers is confined by IPF, and also the filter identifies the source of an attack packet by minimal number of candidate network.

  14. DDOS ATTACK DETECTION SIMULATION AND HANDLING MECHANISM

    Directory of Open Access Journals (Sweden)

    Ahmad Sanmorino

    2013-11-01

    Full Text Available In this study we discuss how to handle DDoS attack that coming from the attacker by using detection method and handling mechanism. Detection perform by comparing number of packets and number of flow. Whereas handling mechanism perform by limiting or drop the packets that detected as a DDoS attack. The study begins with simulation on real network, which aims to get the real traffic data. Then, dump traffic data obtained from the simulation used for detection method on our prototype system called DASHM (DDoS Attack Simulation and Handling Mechanism. From the result of experiment that has been conducted, the proposed method successfully detect DDoS attack and handle the incoming packet sent by attacker.

  15. Transient ischemic attack: diagnostic evaluation.

    Science.gov (United States)

    Messé, Steven R; Jauch, Edward C

    2008-08-01

    A transient ischemic attack portends significant risk of a stroke. Consequently, the diagnostic evaluation in the emergency department is focused on identifying high-risk causes so that preventive strategies can be implemented. The evaluation consists of a facilitated evaluation of the patient's metabolic, cardiac, and neurovascular systems. At a minimum, the following tests are recommended: fingerstick glucose level, electrolyte levels, CBC count, urinalysis, and coagulation studies; noncontrast computed tomography (CT) of the head; electrocardiography; and continuous telemetry monitoring. Vascular imaging studies, such as carotid ultrasonography, CT angiography, or magnetic resonance angiography, should be performed on an urgent basis and prioritized according to the patient's risk stratification for disease. Consideration should be given for echocardiography if no large vessel abnormality is identified.

  16. Where can an Insider attack?

    DEFF Research Database (Denmark)

    Probst, Christian W.; Hansen, René Rydhof; Nielson, Flemming

    2006-01-01

    By definition, an insider has better access, is more trusted, and has better information about internal procedures, high-value targets, and potential weak spots in the security, than an outsider. Consequently, an insider attack has the potential to cause significant, even catastrophic, damage...... to the targeted organisation. While the problem is well recognised in the security community as well as in law-enforcement and intelligence communities, the main resort still is to audit log files \\$\\backslash\\$emph{after the fact}. There has been little research into developing models, automated tools...... of the modelled systems. Our analysis of processes identifies which actions may be performed by whom, at which locations, accessing which data. This allows to compute a superset of audit results---before an incident occurs....

  17. Security under Uncertainty: Adaptive Attackers Are More Challenging to Human Defenders than Random Attackers

    Directory of Open Access Journals (Sweden)

    Frédéric Moisan

    2017-06-01

    Full Text Available Game Theory is a common approach used to understand attacker and defender motives, strategies, and allocation of limited security resources. For example, many defense algorithms are based on game-theoretic solutions that conclude that randomization of defense actions assures unpredictability, creating difficulties for a human attacker. However, many game-theoretic solutions often rely on idealized assumptions of decision making that underplay the role of human cognition and information uncertainty. The consequence is that we know little about how effective these algorithms are against human players. Using a simplified security game, we study the type of attack strategy and the uncertainty about an attacker's strategy in a laboratory experiment where participants play the role of defenders against a simulated attacker. Our goal is to compare a human defender's behavior in three levels of uncertainty (Information Level: Certain, Risky, Uncertain and three types of attacker's strategy (Attacker's strategy: Minimax, Random, Adaptive in a between-subjects experimental design. Best defense performance is achieved when defenders play against a minimax and a random attack strategy compared to an adaptive strategy. Furthermore, when payoffs are certain, defenders are as efficient against random attack strategy as they are against an adaptive strategy, but when payoffs are uncertain, defenders have most difficulties defending against an adaptive attacker compared to a random attacker. We conclude that given conditions of uncertainty in many security problems, defense algorithms would be more efficient if they are adaptive to the attacker actions, taking advantage of the attacker's human inefficiencies.

  18. Cache timing attacks on recent microarchitectures

    DEFF Research Database (Denmark)

    Andreou, Alexandres; Bogdanov, Andrey; Tischhauser, Elmar Wolfgang

    2017-01-01

    Cache timing attacks have been known for a long time, however since the rise of cloud computing and shared hardware resources, such attacks found new potentially devastating applications. One prominent example is S$A (presented by Irazoqui et al at S&P 2015) which is a cache timing attack against...... engineered as part of this work. This is the first time CSSAs for the Skylake architecture are reported. Our attacks demonstrate that cryptographic applications in cloud computing environments using key-dependent tables for acceleration are still vulnerable even on recent architectures, including Skylake...

  19. Religion and support for suicide attacks.

    Science.gov (United States)

    Ginges, Jeremy; Hansen, Ian; Norenzayan, Ara

    2009-02-01

    In four studies carried out across different cultural, religious, and political contexts, we investigated the association between religion and popular support for suicide attacks. In two surveys of Palestinians and one cognitive priming experiment with Israeli settlers, prayer to God, an index of religious devotion, was unrelated to support for suicide attacks. Instead, attendance at religious services, thought to enhance coalitional commitment, positively predicted support for suicide attacks. In a survey of six religions in six nations, regular attendance at religious services positively predicted a combination of willing martyrdom and out-group hostility, but regular prayer did not. Implications for understanding the role of religion in suicide attacks are discussed.

  20. Integrating cyber attacks within fault trees

    International Nuclear Information System (INIS)

    Nai Fovino, Igor; Masera, Marcelo; De Cian, Alessio

    2009-01-01

    In this paper, a new method for quantitative security risk assessment of complex systems is presented, combining fault-tree analysis, traditionally used in reliability analysis, with the recently introduced Attack-tree analysis, proposed for the study of malicious attack patterns. The combined use of fault trees and attack trees helps the analyst to effectively face the security challenges posed by the introduction of modern ICT technologies in the control systems of critical infrastructures. The proposed approach allows considering the interaction of malicious deliberate acts with random failures. Formal definitions of fault tree and attack tree are provided and a mathematical model for the calculation of system fault probabilities is presented.

  1. Visualizing Risks: Icons for Information Attack Scenarios

    National Research Council Canada - National Science Library

    Hosmer, Hilary

    2000-01-01

    .... Visual attack scenarios help defenders see system ambiguities, imprecision, vulnerabilities and omissions, thus speeding up risk analysis, requirements gathering, safeguard selection, cryptographic...

  2. Validation Method of a Telecommunications Blackout Attack

    National Research Council Canada - National Science Library

    Amado, Joao; Nunes, Paulo

    2005-01-01

    This paper presents an evaluation method of telecommunications infrastructure vulnerabilities, allowing the identification of components that can be attacked in order to achieve a communications blackout...

  3. Cued Panic Attacks in Body Dysmorphic Disorder

    Science.gov (United States)

    Phillips, Katharine A.; Menard, William; Bjornsson, Andri S.

    2013-01-01

    Background Body dysmorphic disorder (BDD) is a common and often severe disorder. Clinical observations suggest that panic attacks triggered by BDD symptoms may be common. However, to our knowledge, no study has examined such panic attacks in BDD. We investigated the prevalence, clinical features, and correlates of BDD-triggered panic attacks in individuals with this disorder. Methods Panic attacks and other variables were assessed using reliable and valid measures in 76 individuals with lifetime DSM-IV BDD. Results 28.9% (95% CI, 18.5%–39.4%) of participants reported lifetime panic attacks triggered by BDD symptoms. The most common triggers of such attacks were feeling that others were looking at or scrutinizing the perceived appearance defects (61.9%), looking in the mirror at perceived defects (38.1%), and being in bright light where perceived defects would be more visible (23.8%). The most common panic attack symptoms were palpitations (86.4%), sweating (66.7%), shortness of breath (63.6%), trembling or shaking (63.6%), and fear of losing control or going crazy (63.6%). Compared to participants without such panic attacks, those with BDD-triggered panic attacks had more severe lifetime BDD, social anxiety, and depressive symptoms, as well as poorer functioning and quality of life on a number of measures. They were also less likely to be employed and more likely to have been psychiatrically hospitalized and to have had suicidal ideation due to BDD. Conclusions Panic attacks triggered by BDD-related situations appear common in individuals with this disorder. BDD-triggered panic attacks were associated with greater symptom severity and morbidity. PMID:23653076

  4. Attack Trees for Practical Security Assessment: Ranking of Attack Scenarios with ADTool 2.0

    NARCIS (Netherlands)

    Gadyatskaya, Olga; Jhawar, Ravi; Kordy, P.T.; Lounis, Karim; Mauw, Sjouke; Trujillo-Rasua, Rolando

    2016-01-01

    In this tool demonstration paper we present the ADTool2.0: an open-source software tool for design, manipulation and analysis of attack trees. The tool supports ranking of attack scenarios based on quantitative attributes entered by the user; it is scriptable; and it incorporates attack trees with

  5. Evaluation of Crosstalk Attacks in Access Networks

    DEFF Research Database (Denmark)

    Wagner, Christoph; Eiselt, Michael; Grobe, Klaus

    2016-01-01

    WDM-PON systems regained interest as low-cost solution for metro and access networks. We present a comparative analysis of resilience of wavelength-selective and wavelength-routed architectures against crosstalk attackers. We compare the vulnerability of these architectures against attacks...

  6. Rotational Rebound Attacks on Reduced Skein

    DEFF Research Database (Denmark)

    Khovratovich, Dmitry; Nikolic, Ivica; Rechberger, Christian

    2010-01-01

    In this paper we combine a recent rotational cryptanalysis with the rebound attack, which results in the best cryptanalysis of Skein, a candidate for the SHA-3 competition. The rebound attack approach was so far only applied to AES-like constructions. For the first time, we show that this approach...

  7. Attack Tree Generation by Policy Invalidation

    NARCIS (Netherlands)

    Ivanova, Marieta Georgieva; Probst, Christian W.; Hansen, René Rydhof; Kammüller, Florian; Naeem Akram, R.; Jajodia, S.

    2015-01-01

    Attacks on systems and organisations increasingly exploit human actors, for example through social engineering, complicating their formal treatment and automatic identi﬿cation. Formalisation of human behaviour is difficult at best, and attacks on socio-technical systems are still mostly identi﬿ed

  8. Rotational Rebound Attacks on Reduced Skein

    DEFF Research Database (Denmark)

    Khovratovich, Dmitry; Nikolić, Ivica; Rechberger, Christian

    2014-01-01

    In this paper we combine two powerful methods of symmetric cryptanalysis: rotational cryptanalysis and the rebound attack. Rotational cryptanalysis was designed for the analysis of bit-oriented designs like ARX (Addition-Rotation-XOR) schemes. It has been applied to several hash functions and block...... ciphers, including the new standard SHA-3 (Keccak). The rebound attack is a start-from-the-middle approach for finding differential paths and conforming pairs in byte-oriented designs like Substitution-Permutation networks and AES. We apply our new compositional attack to the reduced version of the hash...... function Skein, a finalist of the SHA-3 competition. Our attack penetrates more than two thirds of the Skein core—the cipher Threefish, and made the designers to change the submission in order to prevent it. The rebound part of our attack has been significantly enhanced to deliver results on the largest...

  9. Use of Attack Graphs in Security Systems

    Directory of Open Access Journals (Sweden)

    Vivek Shandilya

    2014-01-01

    Full Text Available Attack graphs have been used to model the vulnerabilities of the systems and their potential exploits. The successful exploits leading to the partial/total failure of the systems are subject of keen security interest. Considerable effort has been expended in exhaustive modeling, analyses, detection, and mitigation of attacks. One prominent methodology involves constructing attack graphs of the pertinent system for analysis and response strategies. This not only gives the simplified representation of the system, but also allows prioritizing the security properties whose violations are of greater concern, for both detection and repair. We present a survey and critical study of state-of-the-art technologies in attack graph generation and use in security system. Based on our research, we identify the potential, challenges, and direction of the current research in using attack graphs.

  10. Automatic Classification of Attacks on IP Telephony

    Directory of Open Access Journals (Sweden)

    Jakub Safarik

    2013-01-01

    Full Text Available This article proposes an algorithm for automatic analysis of attack data in IP telephony network with a neural network. Data for the analysis is gathered from variable monitoring application running in the network. These monitoring systems are a typical part of nowadays network. Information from them is usually used after attack. It is possible to use an automatic classification of IP telephony attacks for nearly real-time classification and counter attack or mitigation of potential attacks. The classification use proposed neural network, and the article covers design of a neural network and its practical implementation. It contains also methods for neural network learning and data gathering functions from honeypot application.

  11. Two Improved Multiple-Differential Collision Attacks

    Directory of Open Access Journals (Sweden)

    An Wang

    2014-01-01

    Full Text Available In CHES 2008, Bogdanov proposed multiple-differential collision attacks which could be applied to the power analysis attacks on practical cryptographic systems. However, due to the effect of countermeasures on FPGA, there are some difficulties during the collision detection, such as local high noise and the lack of sampling points. In this paper, keypoints voting test is proposed for solving these problems, which can increase the success ratio from 35% to 95% on the example of one implementation. Furthermore, we improve the ternary voting test of Bogdanov, which can improve the experiment efficiency markedly. Our experiments show that the number of power traces required in our attack is only a quarter of the requirement of traditional attack. Finally, some alternative countermeasures against our attacks are discussed.

  12. Hill-Climbing Attacks and Robust Online Signature Verification Algorithm against Hill-Climbing Attacks

    Science.gov (United States)

    Muramatsu, Daigo

    Attacks using hill-climbing methods have been reported as a vulnerability of biometric authentication systems. In this paper, we propose a robust online signature verification algorithm against such attacks. Specifically, the attack considered in this paper is a hill-climbing forged data attack. Artificial forgeries are generated offline by using the hill-climbing method, and the forgeries are input to a target system to be attacked. In this paper, we analyze the menace of hill-climbing forged data attacks using six types of hill-climbing forged data and propose a robust algorithm by incorporating the hill-climbing method into an online signature verification algorithm. Experiments to evaluate the proposed system were performed using a public online signature database. The proposed algorithm showed improved performance against this kind of attack.

  13. Protecting complex infrastructures against multiple strategic attackers

    Science.gov (United States)

    Hausken, Kjell

    2011-01-01

    Infrastructures are analysed subject to defence by a strategic defender and attack by multiple strategic attackers. A framework is developed where each agent determines how much to invest in defending versus attacking each of multiple targets. A target can have economic, human and symbolic values, which generally vary across agents. Investment expenditure functions for each agent can be linear in the investment effort, concave, convex, logistic, can increase incrementally, or can be subject to budget constraints. Contest success functions (e.g., ratio and difference forms) determine the probability of a successful attack on each target, dependent on the relative investments of the defender and attackers on each target, and on characteristics of the contest. Targets can be in parallel, in series, interlinked, interdependent or independent. The defender minimises the expected damage plus the defence expenditures. Each attacker maximises the expected damage minus the attack expenditures. The number of free choice variables equals the number of agents times the number of targets, or lower if there are budget constraints. Each agent is interested in how his investments vary across the targets, and the impact on his utilities. Alternative optimisation programmes are discussed, together with repeated games, dynamic games and incomplete information. An example is provided for illustration.

  14. Radiological attacks and accidents. Medical consequences

    International Nuclear Information System (INIS)

    Sakuta, Hidenari

    2007-01-01

    Probability of the occurrence of radiological attacks appears to be elevated after the terrorist attacks against the United States on September 11 in 2001. There are a lot of scenarios of radiological attack: simple radiological device, radiological disperse device (RDD or dirty bomb), attacks against nuclear reactor, improvised nuclear device, and nuclear weapons. Of these, RDD attack is the most probable scenario, because it can be easily made and can generate enormous psychological and economic damages. Radiological incidents are occurring to and fro in the world, including several cases of theft to nuclear facilities and unsuccessful terrorist attacks against them. Recently, a former Russian spy has allegedly been killed using polonium-210. In addition, serious radiological accidents have occurred in Chernobyl, Goiania, and Tokai-mura. Planning, preparation, education, and training exercise appear to be essential factors to cope with radiological attacks and accidents effectively without feeling much anxiety. Triage and psychological first aid are prerequisite to manage and provide effective medial care for mass casualties without inducing panic. (author)

  15. Rotational Rebound Attacks on Reduced Skein

    DEFF Research Database (Denmark)

    Khovratovich, Dmitry; Nikolic, Ivica; Rechberger, Christian

    2010-01-01

    In this paper we combine a recent rotational cryptanalysis with the rebound attack, which results in the best cryptanalysis of Skein, a candidate for the SHA-3 competition. The rebound attack approach was so far only applied to AES-like constructions. For the first time, we show that this approac...... inside-out computations and neutral bits in the inbound phase of the rebound attack, and give well-defined rotational distinguishers as certificates of weaknesses for the compression functions and block ciphers.......In this paper we combine a recent rotational cryptanalysis with the rebound attack, which results in the best cryptanalysis of Skein, a candidate for the SHA-3 competition. The rebound attack approach was so far only applied to AES-like constructions. For the first time, we show that this approach...... and the Threefish cipher. The new techniques include an analytical search for optimal input values in the rotational cryptanalysis, which allows to extend the outbound phase of the attack with a precomputation phase, an approach never used in any rebound-style attack before. Further we show how to combine multiple...

  16. Smart Grid Integrity Attacks: Characterizations and Countermeasures

    Energy Technology Data Exchange (ETDEWEB)

    Annarita Giani; Eilyan Bitar; Miles McQueen; Pramod Khargonekar; Kameshwar Poolla

    2011-10-01

    Real power injections at loads and generators, and real power flows on selected lines in a transmission network are monitored, transmitted over a SCADA network to the system operator, and used in state estimation algorithms to make dispatch, re-balance and other energy management system [EMS] decisions. Coordinated cyber attacks of power meter readings can be arranged to be undetectable by any bad data detection algorithm. These unobservable attacks present a serious threat to grid operations. Of particular interest are sparse attacks that involve the compromise of a modest number of meter readings. An efficient algorithm to find all unobservable attacks [under standard DC load flow approximations] involving the compromise of exactly two power injection meters and an arbitrary number of power meters on lines is presented. This requires O(n2m) flops for a power system with n buses and m line meters. If all lines are metered, there exist canonical forms that characterize all 3, 4, and 5-sparse unobservable attacks. These can be quickly detected in power systems using standard graph algorithms. Known secure phase measurement units [PMUs] can be used as countermeasures against an arbitrary collection of cyber attacks. Finding the minimum number of necessary PMUs is NP-hard. It is shown that p + 1 PMUs at carefully chosen buses are sufficient to neutralize a collection of p cyber attacks.

  17. Situational awareness of a coordinated cyber attack

    Science.gov (United States)

    Sudit, Moises; Stotz, Adam; Holender, Michael

    2005-03-01

    As technology continues to advance, services and capabilities become computerized, and an ever increasing amount of business is conducted electronically the threat of cyber attacks gets compounded by the complexity of such attacks and the criticality of the information which must be secured. A new age of virtual warfare has dawned in which seconds can differentiate between the protection of vital information and/or services and a malicious attacker attaining their goal. In this paper we present a novel approach in the real-time detection of multistage coordinated cyber attacks and the promising initial testing results we have obtained. We introduce INFERD (INformation Fusion Engine for Real-time Decision-making), an adaptable information fusion engine which performs fusion at levels zero, one, and two to provide real-time situational assessment and its application to the cyber domain in the ECCARS (Event Correlation for Cyber Attack Recognition System) system. The advantages to our approach are fourfold: (1) The complexity of the attacks which we consider, (2) the level of abstraction in which the analyst interacts with the attack scenarios, (3) the speed at which the information fusion is presented and performed, and (4) our disregard for ad-hoc rules or a priori parameters.

  18. Angioedema hereditario en Medellín, Colombia: evaluación clínica y de la calidad de vida

    Directory of Open Access Journals (Sweden)

    María Dulfary Sánchez

    2015-09-01

    Conclusión. Este estudio provee información sobre la primera familia caracterizada con angioedema hereditario de tipo 1 en el Valle de Aburrá, Colombia. Aunque para ello se usó un instrumento genérico, se confirmó, además, el efecto negativo de la enfermedad en la calidad de vida de los individuos que la padecen.

  19. Calcium Supplements: A Risk Factor for Heart Attack?

    Science.gov (United States)

    ... for heart attack? I've read that calcium supplements may increase the risk of heart attack. Is ... Some doctors think it's possible that taking calcium supplements may increase your risk of a heart attack. ...

  20. Detecting Pulsing Denial-of-Service Attacks with Nondeterministic Attack Intervals

    Directory of Open Access Journals (Sweden)

    Xiapu Luo

    2009-01-01

    Full Text Available This paper addresses the important problem of detecting pulsing denial of service (PDoS attacks which send a sequence of attack pulses to reduce TCP throughput. Unlike previous works which focused on a restricted form of attacks, we consider a very broad class of attacks. In particular, our attack model admits any attack interval between two adjacent pulses, whether deterministic or not. It also includes the traditional flooding-based attacks as a limiting case (i.e., zero attack interval. Our main contribution is Vanguard, a new anomaly-based detection scheme for this class of PDoS attacks. The Vanguard detection is based on three traffic anomalies induced by the attacks, and it detects them using a CUSUM algorithm. We have prototyped Vanguard and evaluated it on a testbed. The experiment results show that Vanguard is more effective than the previous methods that are based on other traffic anomalies (after a transformation using wavelet transform, Fourier transform, and autocorrelation and detection algorithms (e.g., dynamic time warping.

  1. Detecting Pulsing Denial-of-Service Attacks with Nondeterministic Attack Intervals

    Science.gov (United States)

    Luo, Xiapu; Chan, Edmond W. W.; Chang, Rocky K. C.

    2009-12-01

    This paper addresses the important problem of detecting pulsing denial of service (PDoS) attacks which send a sequence of attack pulses to reduce TCP throughput. Unlike previous works which focused on a restricted form of attacks, we consider a very broad class of attacks. In particular, our attack model admits any attack interval between two adjacent pulses, whether deterministic or not. It also includes the traditional flooding-based attacks as a limiting case (i.e., zero attack interval). Our main contribution is Vanguard, a new anomaly-based detection scheme for this class of PDoS attacks. The Vanguard detection is based on three traffic anomalies induced by the attacks, and it detects them using a CUSUM algorithm. We have prototyped Vanguard and evaluated it on a testbed. The experiment results show that Vanguard is more effective than the previous methods that are based on other traffic anomalies (after a transformation using wavelet transform, Fourier transform, and autocorrelation) and detection algorithms (e.g., dynamic time warping).

  2. Off-Label Use of Agents for Management of Serious or Life-threatening Angiotensin Converting Enzyme Inhibitor-Induced Angioedema.

    Science.gov (United States)

    Culley, Colleen M; DiBridge, Julie N; Wilson, Gregory L

    2016-01-01

    To evaluate the place in therapy of fresh frozen plasma (FFP), C1 esterase concentrate (C1-INH), ecallantide, and icatibant in the management of angiotensin-converting enzyme inhibitor-induced angioedema (ACEI-IA). A literature search was performed using PubMed (1946 through August 2015) and Embase (angioedema, another bradykinin-mediated event, may be effective for use in ACEI-IA. Positive efficacy results were reported with FFP and C1-INH while mixed results have been seen with ecallantide. Off-label icatibant has the most evidence supporting its use in ACEI-IA with rapid symptom resolution (10 minutes to 6 hours) and avoidance of intubation and tracheotomy in several cases. These agents were well-tolerated in ACEI-IA. ACEI-IA is typically a self-limiting event. First-line therapies include ACEI discontinuation, observation, and supportive medications (eg, corticosteroids, antihistamines, and epinephrine). Symptom progression can be life-threatening and may require interventions such as tracheotomy and intubation. Off-label use of FFP and medications approved for hereditary angioedema have resulted in rapid resolution of symptoms and avoidance of intubation. Among these agents, icatibant has the most supporting evidence and has been incorporated into practice guidelines and algorithms as a second-line agent for serious life-threatening ACE-IA. © The Author(s) 2015.

  3. Whole Exome Sequencing and Molecular Modeling of a Missense Variant in TNFAIP3 That Segregates with Disease in a Family with Chronic Urticaria and Angioedema

    Directory of Open Access Journals (Sweden)

    Antoneicka L. Harris

    2018-01-01

    Full Text Available Chronic urticaria is a common condition characterized by recurrent hives lasting several weeks or months and is usually idiopathic. Approximately half of the individuals with chronic urticaria will present with episodes of angioedema that can be severe and debilitating. In this report, we describe a 47-year-old Hispanic male who presented initially for an evaluation of chronic hives following hospitalization due to hive-induced anaphylaxis. The individual had a history significant for urticaria and angioedema beginning in his early 30s. Interestingly, both the individual’s 41-year-old sister and 12-year-old daughter were also affected with chronic urticaria and severe angioedema. Whole exome sequencing of the proband and several family members revealed a heterozygous variant of uncertain significance in exon 2 of TNFAIP3, denoted as c.65G>A (p.R22Q, in all affected members. Variants in TNFAIP3 have been associated with multiple autoimmune diseases, susceptibility to allergy and asthma, and periodic fever syndromes, suggesting that this variant could potentially play a role in disease.

  4. The role of sleep in migraine attacks

    Directory of Open Access Journals (Sweden)

    Elaine Inamorato

    1993-11-01

    Full Text Available Migraine attacks may be precipitated by sleep deprivation or excessive sleep and sleep is also associated with relief of migraine attacks. In view of this variable relationship we studied the records of 159 consecutive outpatients of our Headache Unit. In 121 records there was reference to sleep involvement, in 55% by a single form and in 45% by more than one form. When only one form was related, relief was most common (70%. 30% of that group of patients had the migraine attack precipitated by sleep, 24% by deprivation and 6% by sleep excess. When the effects of sleep were multiple, these effects were as expected logically in 65%: «in accordance» group (e.g attack precipitated by sleep deprivation and relieved by sleep onset. In a second group, («conflicting» where the involvement was not logical, there were three different combinations of sleep involvement, possibly due to more than one pathophysiological mechanism.

  5. On localization attacks against cloud infrastructure

    Science.gov (United States)

    Ge, Linqiang; Yu, Wei; Sistani, Mohammad Ali

    2013-05-01

    One of the key characteristics of cloud computing is the device and location independence that enables the user to access systems regardless of their location. Because cloud computing is heavily based on sharing resource, it is vulnerable to cyber attacks. In this paper, we investigate a localization attack that enables the adversary to leverage central processing unit (CPU) resources to localize the physical location of server used by victims. By increasing and reducing CPU usage through the malicious virtual machine (VM), the response time from the victim VM will increase and decrease correspondingly. In this way, by embedding the probing signal into the CPU usage and correlating the same pattern in the response time from the victim VM, the adversary can find the location of victim VM. To determine attack accuracy, we investigate features in both the time and frequency domains. We conduct both theoretical and experimental study to demonstrate the effectiveness of such an attack.

  6. Marine Attack on Towed Hydrophone Arrays

    National Research Council Canada - National Science Library

    Kalmijn, Ad

    2002-01-01

    The original objective of the SIO Marine Attack project was to identify the electric and magnetic fields causing sharks to inflict serious damage upon the towed hydrophone arrays of US Navy submarines...

  7. Diabetes - preventing heart attack and stroke

    Science.gov (United States)

    Diabetes complications - heart; Coronary artery disease - diabetes; CAD - diabetes; Cerebrovascular disease - diabetes ... People with diabetes have a higher chance of having heart attacks and strokes. Smoking and having high blood pressure and high ...

  8. Outdoor Air Pollution, Heart Attack and Stroke

    Science.gov (United States)

    Elevated outdoor ambient air particle pollution triggers heart attacks, strokes, and abnormal heart rhythms and worsens heart failure in individuals at high risk due to underlying medical conditions. Emergency Medical Services in communities are the first responders to these eme...

  9. Heuristic attacks against graphical password generators

    CSIR Research Space (South Africa)

    Peach, S

    2010-05-01

    Full Text Available In this paper the authors explore heuristic attacks against graphical password generators. A new trend is emerging to use user clickable pictures to generate passwords. This technique of authentication can be successfully used for - for example...

  10. Twisted Polynomials and Forgery Attacks on GCM

    DEFF Research Database (Denmark)

    Abdelraheem, Mohamed Ahmed A. M. A.; Beelen, Peter; Bogdanov, Andrey

    2015-01-01

    nonce misuse resistance, such as POET. The algebraic structure of polynomial hashing has given rise to security concerns: At CRYPTO 2008, Handschuh and Preneel describe key recovery attacks, and at FSE 2013, Procter and Cid provide a comprehensive framework for forgery attacks. Both approaches rely...... heavily on the ability to construct forgery polynomials having disjoint sets of roots, with many roots (“weak keys”) each. Constructing such polynomials beyond naïve approaches is crucial for these attacks, but still an open problem. In this paper, we comprehensively address this issue. We propose to use...... in an improved key recovery algorithm. As cryptanalytic applications of our twisted polynomials, we develop the first universal forgery attacks on GCM in the weak-key model that do not require nonce reuse. Moreover, we present universal weak-key forgeries for the nonce-misuse resistant AE scheme POET, which...

  11. Using agility to combat cyber attacks.

    Science.gov (United States)

    Anderson, Kerry

    2017-06-01

    Some incident response practitioners feel that they have been locked in a battle with cyber criminals since the popular adoption of the internet. Initially, organisations made great inroads in preventing and containing cyber attacks. In the last few years, however, cyber criminals have become adept at eluding defence security technologies and rapidly modifying their exploit strategies for financial or political gains. Similar to changes in military combat tactics, cyber criminals utilise distributed attack cells, real-time communications, and rapidly mutating exploits to minimise the potential for detection. Cyber criminals have changed their attack paradigm. This paper describes a new incident response paradigm aimed at combating the new model of cyber attacks with an emphasis on agility to increase the organisation's ability to respond rapidly to these new challenges.

  12. Joint Warfighting: Attacking Time-Critical Targets

    National Research Council Canada - National Science Library

    Lewis, Jerry

    2001-01-01

    .... While DOD has developed and fielded considerable capability to detect, assess, and attack most fixed enemy targets, experiences in the Persian Gulf and more recently in Kosovo revealed that DOD...

  13. ATTACK WARNING: Costs to Modernize NORAD's Computer System Significantly Understated

    National Research Council Canada - National Science Library

    Cross, F

    1991-01-01

    ...) Integrated Tactical Warning and Attack Assessment (ITW/AA) system. These subsystems provide critical strategic surveillance and attack warning and assessment information to United States and Canadian leaders...

  14. Consciousness in Non-Epileptic Attack Disorder

    OpenAIRE

    Reuber, M.; Kurthen, M.

    2011-01-01

    Non-epileptic attack disorder (NEAD) is one of the most important differential diagnoses of epilepsy. Impairment of\\ud consciousness is the key feature of non-epileptic attacks (NEAs). The first half of this review summarises the clinical research\\ud literature featuring observations relating to consciousness in NEAD. The second half places this evidence in the wider context\\ud of the recent discourse on consciousness in neuroscience and the philosophy of mind. We argue that studies of consci...

  15. Semantic Identification Attacks on Web Browsing

    OpenAIRE

    Guha, Neel

    2016-01-01

    We introduce a Semantic Identification Attack, in which an adversary uses semantic signals about the pages visited in one browsing session to identify other browsing sessions launched by the same user. This attack allows an adver- sary to determine if two browsing sessions originate from the same user regardless of any measures taken by the user to disguise their browser or network. We use the MSNBC Anonymous Browsing data set, which contains a large set of user visits (labeled by category) t...

  16. Panic Attack History and Smoking Topography

    Science.gov (United States)

    Farris, Samantha G.; Brown, Lily A.; Goodwin, Renee D.; Zvolensky, Michael J.

    2016-01-01

    Background Little is known about panic attacks and puffing topography, a behavioral index of the value of smoking reinforcement. This study examined smoking style during the course of smoking of a single cigarette among adult daily smokers with and without a history of panic attacks. Method Participants (n = 124, Mage = 43.9, SD = 9.7; 44.4% female) were non-treatment seeking daily smokers. Lifetime panic attack history was assessed via diagnostic assessment; 28.2% (n = 35) of the sample had a panic attack history. Participants smoked one cigarette during an ad libitum smoking trial. Puff volume, duration, and inter-puff interval were measured using the Clinical Research Support System (CReSS) pocket device. Results Regression analyses revealed that panic attack status was not associated with significant differences in average puff volume, duration, or inter-puff interval. Multi-level modeling was used to examine puffing trajectories. Puff-level data revealed that there was a significant quadratic time x panic effect for puff volume and duration. Those with a panic attack history demonstrated relatively sustained levels of both puff volume and duration over time, whereas those without a history of panic attacks demonstrated an increase followed by a decrease in volume and duration over time. These effects were not accounted for by the presence of general psychopathology. Discussion Smokers with a panic attack history demonstrate more persistent efforts to self-regulate the delivery of nicotine, and thus may be at risk for continued smoking and dependence. Tailored treatment may be needed to address unique vulnerabilities among this group. PMID:28033542

  17. Attack by Pyemotes johnmoseri (Acari: Pyemotidae)

    Science.gov (United States)

    Tulin Askit; Ibrahim Cakmak; John Moser

    2007-01-01

    The Aegean Region of Turkey is one of the largest dried fig producers in the world. A Turkish cultivar sarilop (Ficus carica cv. Calimyrna L.) possesses good qualities for drying process, and has been grown extensively for many years in Turkey. Hypoborus ficus is the most common xylophagous insect attacking fig trees in Aydin (Aks¸it et al. 2003). This pest attacks...

  18. Cyber Security Audit and Attack Detection Toolkit

    Energy Technology Data Exchange (ETDEWEB)

    Peterson, Dale

    2012-05-31

    This goal of this project was to develop cyber security audit and attack detection tools for industrial control systems (ICS). Digital Bond developed and released a tool named Bandolier that audits ICS components commonly used in the energy sector against an optimal security configuration. The Portaledge Project developed a capability for the PI Historian, the most widely used Historian in the energy sector, to aggregate security events and detect cyber attacks.

  19. Combined Heuristic Attack Strategy on Complex Networks

    Directory of Open Access Journals (Sweden)

    Marek Šimon

    2017-01-01

    Full Text Available Usually, the existence of a complex network is considered an advantage feature and efforts are made to increase its robustness against an attack. However, there exist also harmful and/or malicious networks, from social ones like spreading hoax, corruption, phishing, extremist ideology, and terrorist support up to computer networks spreading computer viruses or DDoS attack software or even biological networks of carriers or transport centers spreading disease among the population. New attack strategy can be therefore used against malicious networks, as well as in a worst-case scenario test for robustness of a useful network. A common measure of robustness of networks is their disintegration level after removal of a fraction of nodes. This robustness can be calculated as a ratio of the number of nodes of the greatest remaining network component against the number of nodes in the original network. Our paper presents a combination of heuristics optimized for an attack on a complex network to achieve its greatest disintegration. Nodes are deleted sequentially based on a heuristic criterion. Efficiency of classical attack approaches is compared to the proposed approach on Barabási-Albert, scale-free with tunable power-law exponent, and Erdős-Rényi models of complex networks and on real-world networks. Our attack strategy results in a faster disintegration, which is counterbalanced by its slightly increased computational demands.

  20. On the anatomy of social engineering attacks: A literature-based dissection of successful attacks

    OpenAIRE

    Bullée, Jan Willem Hendrik; Montoya, Lorena; Pieters, W.; Junger, M.; Hartel, P.H.

    2018-01-01

    The aim of this study was to explore the extent to which persuasion principles are used in successful social engineering attacks. Seventy-four scenarios were extracted from 4 books on social engineering (written by social engineers) and analysed. Each scenario was split into attack steps, containing single interactions between offender and target. For each attack step, persuasion principles were identified. The main findings are that (a) persuasion principles are often used in social engineer...

  1. Association study of genetic variants in PLA2G4A, PLCG1, LAT, SYK, and TNFRS11A genes in NSAIDs-induced urticaria and/or angioedema patients.

    Science.gov (United States)

    Ayuso, Pedro; Plaza-Serón, María del Carmen; Doña, Inmaculada; Blanca-López, Natalia; Campo, Paloma; Cornejo-García, José A; Perkins, James R; Torres, Maria J; Blanca, Miguel; Canto, Gabriela

    2015-12-01

    NSAIDs-induced urticaria and/or angioedema (NIUA) is the most frequent entity of hypersensitivity reactions to NSAIDs. The underlying cause is considered to be because of a nonspecific immunological mechanism in which mast cells are key players. We studied the association of nine single nucleotide polymorphisms in five genes involved in mast cell activation (SYK, LAT1, PLCG1, PLA2G4A, and TNFRSF11A) in 450 NIUA patients and 500 controls. We identified several statistically significant associations when stratifying patients by symptoms: PLA2G4A rs12746200 (urticaria vs. controls, Pc=0.005). PLCG1 rs2228246 (angioedema vs. controls; Pc=0.044), and TNFRS11A rs1805034 (urticaria+angioedema vs. controls; Pc=0.041). The frequency of haplotype PLCG1 rs753381-rs2228246 (C-G) in angioedema-NIUA patients was lower than that in controls (Pc=0.040). In addition, the haplotype frequency of TNFRS11A rs1805034-rs35211496 (C-T) was higher among urticaria-NIUA and urticaria+angioedema-NIUA patients than the controls (Pc=0.045 and 0.046). Our results shed light on the involvement of variants in genes related to non-immunological mast cell activation in NIUA.

  2. Exploiting Hardware Vulnerabilities to Attack Embedded System Devices: a Survey of Potent Microarchitectural Attacks

    Directory of Open Access Journals (Sweden)

    Apostolos P. Fournaris

    2017-07-01

    Full Text Available Cyber-Physical system devices nowadays constitute a mixture of Information Technology (IT and Operational Technology (OT systems that are meant to operate harmonically under a security critical framework. As security IT countermeasures are gradually been installed in many embedded system nodes, thus securing them from many well-know cyber attacks there is a lurking danger that is still overlooked. Apart from the software vulnerabilities that typical malicious programs use, there are some very interesting hardware vulnerabilities that can be exploited in order to mount devastating software or hardware attacks (typically undetected by software countermeasures capable of fully compromising any embedded system device. Real-time microarchitecture attacks such as the cache side-channel attacks are such case but also the newly discovered Rowhammer fault injection attack that can be mounted even remotely to gain full access to a device DRAM (Dynamic Random Access Memory. Under the light of the above dangers that are focused on the device hardware structure, in this paper, an overview of this attack field is provided including attacks, threat directives and countermeasures. The goal of this paper is not to exhaustively overview attacks and countermeasures but rather to survey the various, possible, existing attack directions and highlight the security risks that they can pose to security critical embedded systems as well as indicate their strength on compromising the Quality of Service (QoS such systems are designed to provide.

  3. Predicting Factors of Zone 4 Attack in Volleyball.

    Science.gov (United States)

    Costa, Gustavo C; Castro, Henrique O; Evangelista, Breno F; Malheiros, Laura M; Greco, Pablo J; Ugrinowitsch, Herbert

    2017-06-01

    This study examined 142 volleyball games of the Men's Super League 2014/2015 seasons in Brazil from which we analyzed 24-26 games of each participating team, identifying 5,267 Zone 4 attacks for further analysis. Within these Zone 4 attacks, we analyzed the association between the effect of the attack carried out and the separate effects of serve reception, tempo and type of attack. We found that the reception, tempo of attack, second tempo of attack, and power of diagonal attack were predictors of the attack effect in Zone 4. Moreover, placed attacks showed a tendency to not yield a score. In conclusion, winning points in high-level men's volleyball requires excellent receptions, a fast attack tempo and powerfully executed of attacks.

  4. Pathologic features of fatal shark attacks.

    Science.gov (United States)

    Byard, R W; Gilbert, J D; Brown, K

    2000-09-01

    To examine the pattern of injuries in cases of fatal shark attack in South Australian waters, the authors examined the files of their institution for all cases of shark attack in which full autopsies had been performed over the past 25 years, from 1974 to 1998. Of the seven deaths attributed to shark attack during this period, full autopsies were performed in only two cases. In the remaining five cases, bodies either had not been found or were incomplete. Case 1 was a 27-year-old male surfer who had been attacked by a shark. At autopsy, the main areas of injury involved the right thigh, which displayed characteristic teeth marks, extensive soft tissue damage, and incision of the femoral artery. There were also incised wounds of the right wrist. Bony injury was minimal, and no shark teeth were recovered. Case 2 was a 26-year-old male diver who had been attacked by a shark. At autopsy, the main areas of injury involved the left thigh and lower leg, which displayed characteristic teeth marks, extensive soft tissue damage, and incised wounds of the femoral artery and vein. There was also soft tissue trauma to the left wrist, with transection of the radial artery and vein. Bony injury was minimal, and no shark teeth were recovered. In both cases, death resulted from exsanguination following a similar pattern of soft tissue and vascular damage to a leg and arm. This type of injury is in keeping with predator attack from underneath or behind, with the most severe injuries involving one leg. Less severe injuries to the arms may have occurred during the ensuing struggle. Reconstruction of the damaged limb in case 2 by sewing together skin, soft tissue, and muscle bundles not only revealed that no soft tissue was missing but also gave a clearer picture of the pattern of teeth marks, direction of the attack, and species of predator.

  5. Know the Warning Signs of a Heart Attack

    Science.gov (United States)

    ... No. 22 Know the Warning Signs of a Heart Attack What is a heart attack? Aheart attack happens when the blood vessels that ... hurting your heart muscle. Another name for a heart attack is myocardial infarction, or MI. If you have ...

  6. 12 CFR 263.17 - Collateral attacks on adjudicatory proceeding.

    Science.gov (United States)

    2010-01-01

    ... 12 Banks and Banking 3 2010-01-01 2010-01-01 false Collateral attacks on adjudicatory proceeding... Collateral attacks on adjudicatory proceeding. If an interlocutory appeal or collateral attack is brought in... shall be excused based on the pendency before any court of any interlocutory appeal or collateral attack. ...

  7. 12 CFR 509.17 - Collateral attacks on adjudicatory proceeding.

    Science.gov (United States)

    2010-01-01

    ... 12 Banks and Banking 5 2010-01-01 2010-01-01 false Collateral attacks on adjudicatory proceeding....17 Collateral attacks on adjudicatory proceeding. If an interlocutory appeal or collateral attack is... shall be excused based on the pendency before any court of any interlocutory appeal or collateral attack. ...

  8. A Game Theoretic Approach to Cyber Attack Prediction

    Energy Technology Data Exchange (ETDEWEB)

    Peng Liu

    2005-11-28

    The area investigated by this project is cyber attack prediction. With a focus on correlation-based prediction, current attack prediction methodologies overlook the strategic nature of cyber attack-defense scenarios. As a result, current cyber attack prediction methodologies are very limited in predicting strategic behaviors of attackers in enforcing nontrivial cyber attacks such as DDoS attacks, and may result in low accuracy in correlation-based predictions. This project develops a game theoretic framework for cyber attack prediction, where an automatic game-theory-based attack prediction method is proposed. Being able to quantitatively predict the likelihood of (sequences of) attack actions, our attack prediction methodology can predict fine-grained strategic behaviors of attackers and may greatly improve the accuracy of correlation-based prediction. To our best knowledge, this project develops the first comprehensive framework for incentive-based modeling and inference of attack intent, objectives, and strategies; and this project develops the first method that can predict fine-grained strategic behaviors of attackers. The significance of this research and the benefit to the public can be demonstrated to certain extent by (a) the severe threat of cyber attacks to the critical infrastructures of the nation, including many infrastructures overseen by the Department of Energy, (b) the importance of cyber security to critical infrastructure protection, and (c) the importance of cyber attack prediction to achieving cyber security.

  9. Trace Attack against Biometric Mobile Applications

    Directory of Open Access Journals (Sweden)

    Sanaa Ghouzali

    2016-01-01

    Full Text Available With the exponential increase in the dependence on mobile devices in everyday life, there is a growing concern related to privacy and security issues in the Gulf countries; therefore, it is imperative that security threats should be analyzed in detail. Mobile devices store enormous amounts of personal and financial information, unfortunately without any security. In order to secure mobile devices against different threats, biometrics has been applied and shown to be effective. However, biometric mobile applications are also vulnerable to several types of attacks that can decrease their security. Biometric information itself is considered sensitive data; for example, fingerprints can leave traces in touched objects and facial images can be captured everywhere or accessed by the attacker if the facial image is stored in the mobile device (lost or stolen. Hence, an attacker can easily forge the identity of a legitimate user and access data on a device. In this paper, the effects of a trace attack on the sensitivity of biometric mobile applications are investigated in terms of security and user privacy. Experimental results carried out on facial and fingerprint mobile authentication applications using different databases have shown that these mobile applications are vulnerable to the proposed attack, which poses a serious threat to the overall system security and user privacy.

  10. Panic Attack during Elective Gastrointestinal Endoscopy

    Directory of Open Access Journals (Sweden)

    Charalampos Mitsonis

    2011-01-01

    Full Text Available Background. Esophagogastroduodenoscopy (EGD and colonoscopy (CS can evoke anxiety, embarrassment, and discomfort. These concerns can culminate in panic attacks, which may traumatize patients and significantly decrease their compliance to the procedure. The objective of this study was to evaluate the relationship between preendoscopic anxiety and the possibility of a panic attack during an elective gastrointestinal endoscopy (EGE. Methods. The study population comprised of 79 Greek outpatients. The examination was carried out without the use of conscious sedation. Patients' anxiety levels were assessed before the procedure using the Greek version of the Spielberger State-Trait Anxiety Inventory (STAI-Y. Results. Seventy-nine patients were enrolled: 45 EGD and 34 CS. Females had higher state and trait anxiety levels than males (48.14 ± 7.94 versus 44.17 ± 7.43, <0.05; and 43.68 ± 6.95 versus 39.86 ± 7.46, <0.05. Patients who experienced panic attack had significantly higher levels of both trait and state anxiety, compared to those who were panic-free. There was no significant relationship between panic attacks and sex or type of procedure. Conclusions. Patients who experience panic attacks during endoscopic procedures appear to have significantly higher anxiety levels before the procedure. Administering the STAI questionnaire prior to the endoscopy seems to be a useful screening method for vulnerable patients.

  11. On the anatomy of social engineering attacks : A literature-based dissection of successful attacks

    NARCIS (Netherlands)

    Bullée, Jan Willem Hendrik; Montoya, Lorena; Pieters, W.; Junger, M.; Hartel, P.H.

    2018-01-01

    The aim of this study was to explore the extent to which persuasion principles are used in successful social engineering attacks. Seventy-four scenarios were extracted from 4 books on social engineering (written by social engineers) and analysed. Each scenario was split into attack steps,

  12. On the anatomy of social engineering attacks : A literature-based dissection of successful attacks

    NARCIS (Netherlands)

    Bullee, Jan-Willem; Montoya, L.; Pieters, Wolter; Junger, Marianne; Hartel, Pieter H.

    The aim of this studywas to explore the extent towhich persuasion principles are used in successful social engineering attacks. Seventy-four scenarioswere extracted from 4 books on social engineering (written by social engineers) and analysed. Each scenariowas split into attack steps, containing

  13. Overview of DOS attacks on wireless sensor networks and experimental results for simulation of interference attacks

    Directory of Open Access Journals (Sweden)

    Željko Gavrić

    2018-01-01

    Full Text Available Wireless sensor networks are now used in various fields. The information transmitted in the wireless sensor networks is very sensitive, so the security issue is very important. DOS (denial of service attacks are a fundamental threat to the functioning of wireless sensor networks. This paper describes some of the most common DOS attacks and potential methods of protection against them. The case study shows one of the most frequent attacks on wireless sensor networks – the interference attack. In the introduction of this paper authors assume that the attack interference can cause significant obstruction of wireless sensor networks. This assumption has been proved in the case study through simulation scenario and simulation results.

  14. Attack-tolerant networked control system: an approach for detection the controller stealthy hijacking attack

    Science.gov (United States)

    Atta Yaseen, Amer; Bayart, Mireille

    2017-01-01

    In this work, a new approach will be introduced as a development for the attack-tolerant scheme in the Networked Control System (NCS). The objective is to be able to detect an attack such as the Stuxnet case where the controller is reprogrammed and hijacked. Besides the ability to detect the stealthy controller hijacking attack, the advantage of this approach is that there is no need for a priori mathematical model of the controller. In order to implement the proposed scheme, a specific detector for the controller hijacking attack is designed. The performance of this scheme is evaluated be connected the detector to NCS with basic security elements such as Data Encryption Standard (DES), Message Digest (MD5), and timestamp. The detector is tested along with networked PI controller under stealthy hijacking attack. The test results of the proposed method show that the hijacked controller can be significantly detected and recovered.

  15. A Traceability Attack against e-Passports

    Science.gov (United States)

    Chothia, Tom; Smirnov, Vitaliy

    Since 2004, many nations have started issuing "e-passports" containing an RFID tag that, when powered, broadcasts information. It is claimed that these passports are more secure and that our data will be protected from any possible unauthorised attempts to read it. In this paper we show that there is a flaw in one of the passport's protocols that makes it possible to trace the movements of a particular passport, without having to break the passport's cryptographic key. All an attacker has to do is to record one session between the passport and a legitimate reader, then by replaying a particular message, the attacker can distinguish that passport from any other. We have implemented our attack and tested it successfully against passports issued by a range of nations.

  16. Liability for damage caused by terrorist attacks

    International Nuclear Information System (INIS)

    Heller, W.

    2004-01-01

    After the terrorist attacks on September 11, 2001, one of the questions raised was about the potential liability of the operator of a nuclear power plant for damage sustained by a third party as a result of a comparable terrorist attack on a nuclear power plant. Internationally, this situation is regulated by the Convention on Third-Party Liability in Nuclear Power, the so-called Paris Liability Convention, of 1960, 1964, 1982. Among other things, that Convention excludes liability in cases directly resulting form 'actions of armed conflict..'. The problem arises, among other things, from the absence of an internationally acknowledged definition of terrorism or terrorist attack, and from the idea that, according to the Paris Convention, the legal entities assumed to be involved in such actions are states and weapons. National and international agreements and laws about the liability of the operator of nuclear facility for damage to third parties as a result of terrorist actions are analyzed and discussed. (orig.)

  17. Key Recovery Attacks on Recent Authenticated Ciphers

    DEFF Research Database (Denmark)

    Bogdanov, Andrey; Dobraunig, Christoph; Eichlseder, Maria

    2014-01-01

    and wireless networks. All these schemes use well-established and secure components such as the AES, Grain-like NFSRs, ChaCha and SipHash as their building blocks. However, we discover key recovery attacks for all three designs, featuring square-root complexities. Using a key collision technique, we can...... recover the secret key of AVALANCHE in 2n/2, where n 2∈ {28; 192; 256} is the key length. This technique also applies to the authentication part of Calico whose 128-bit key can be recovered in 264 time. For RBS, we can recover its full 132-bit key in 265 time with a guess-and-determine attack. All attacks...

  18. Discovering Collaborative Cyber Attack Patterns Using Social Network Analysis

    Science.gov (United States)

    Du, Haitao; Yang, Shanchieh Jay

    This paper investigates collaborative cyber attacks based on social network analysis. An Attack Social Graph (ASG) is defined to represent cyber attacks on the Internet. Features are extracted from ASGs to analyze collaborative patterns. We use principle component analysis to reduce the feature space, and hierarchical clustering to group attack sources that exhibit similar behavior. Experiments with real world data illustrate that our framework can effectively reduce from large dataset to clusters of attack sources exhibiting critical collaborative patterns.

  19. Playing Attack and Defense with Trusted Storage

    DEFF Research Database (Denmark)

    Gonzalez, Javier; Bonnet, Philippe; Bouganim, Luc

    2014-01-01

    It is often convenient to assume in a data management platform that one or several computing devices are trusted, specially when the goal is to provide privacy guarantees over personal data. But what does it take for a computing device to be trusted? More specifically, how can a personal device...... provide trusted storage? This is the question we tackle in this demonstration. We describe how secure devices, equipped with a trusted execution environment, differ from general purpose devices. We illustrate with our demonstration scenario, that it is much more difficult to attack a storage service...... running on a secure device, than to attack the same service running on a general purpose device....

  20. Attacks and infections in percolation processes

    International Nuclear Information System (INIS)

    Janssen, Hans-Karl; Stenull, Olaf

    2017-01-01

    We discuss attacks and infections at propagating fronts of percolation processes based on the extended general epidemic process. The scaling behavior of the number of the attacked and infected sites in the long time limit at the ordinary and tricritical percolation transitions is governed by specific composite operators of the field-theoretic representation of this process. We calculate corresponding critical exponents for tricritical percolation in mean-field theory and for ordinary percolation to 1-loop order. Our results agree well with the available numerical data. (paper)