WorldWideScience

Sample records for angioedema hae attacks

  1. Hereditary angioedema with normal C1-INH (HAE type III).

    Science.gov (United States)

    Riedl, Marc A

    2013-01-01

    Hereditary angioedema (HAE) with normal C1 inhibitor (C1-INH), also known as HAE type III, is a familial condition only clinically recognized within the past three decades. Similar to HAE from C1-INH deficiency (HAE types I and II), affected individuals experience unpredictable angioedema episodes of the skin, gastrointestinal tract, and airway. Unique clinical features of HAE with normal C1-INH include the predominance of affected women, frequent exacerbation by estrogen, and a prominence of angioedema that involves the face and oropharynx. The underlying pathophysiology of HAE with normal C1-INH is poorly understood, but indirect evidence points to contact pathway dysregulation with bradykinin-mediated angioedema. Currently, evaluation is complicated by a lack of confirmatory laboratory testing such that clinical criteria must often be used to make the diagnosis of HAE with normal C1-INH. Factor XII mutations have been identified in only a minority of persons affected by HAE with normal C1-INH, limiting the utility of such analysis. To date, no controlled clinical studies have examined the efficacy of therapeutic agents for HAE with normal C1-INH, although published evidence supports frequent clinical benefit with medications shown effective in HAE due to C1-INH deficiency. PMID:24565612

  2. Prophylaxis in hereditary angioedema (HAE) with C1 inhibitor deficiency.

    Science.gov (United States)

    Greve, Jens; Strassen, Ulrich; Gorczyza, Marina; Dominas, Nina; Frahm, Uta-Marie; Mühlberg, Heike; Wiednig, Michaela; Zampeli, Vasiliki; Magerl, Markus

    2016-03-01

    Hereditary angioedema (HAE) is a rare congenital disorder characterized by recurrent episodes of subcutaneous or submucosal edema. Laryngeal manifestations can be life-threatening. In the majority of cases, the disease can be adequately treated with an on-demand approach - in some cases, however, short- or long-term prophylaxis is indicated. Attenuated androgens used to be the drugs of choice, but they are associated with considerable side effects and no longer commercially available in the German-speaking countries of the EU. They are currently being replaced by more effective and more tolerable agents such C1-inhibitors, the kallikrein inhibitor ecallantide, and the B2 receptor antagonist icatibant, which have recently obtained market authorization. These new drugs have had a major impact, especially on the indications and procedures for long-term prophylaxis. According to the most recent international consensus papers and our own experience, self-administered C1-inhibitors are now the first option for long-term prophylactic therapy. The decision for prophylaxis should no longer be based on single parameters such as the frequency of attacks but on adequate overall disease control including quality of life. More drugs are currently being developed, which may lead to further changes in the treatment algorithms of HAE. PMID:26972189

  3. 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.

  4. 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......-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: This is a cross-sectional study being conducted in Denmark, Germany and Spain. The study is open to patients ages 12 and older with a...... collect detailed descriptive data and patient testimonials on the impact of HAE on patients' health-related quality of life. CONCLUSION: The present manuscript describes the development and plans for implementing a multi-country European study with the aim of characterizing the humanistic and economic...

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

    DEFF Research Database (Denmark)

    Aberer, W; Maurer, M; Reshef, A; Longhurst, H; Kivity, S; Bygum, Anette; Caballero, T; Bloom, B; Nair, N; Malbrán, 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...

  6. Management of acute attacks of hereditary angioedema: role of ecallantide

    Directory of Open Access Journals (Sweden)

    Duffey H

    2015-04-01

    Full Text Available Hannah Duffey,1 Rafael Firszt1,2 1Department of Pediatrics, 2Division of Allergy, Immunology and Rheumatology, University of Utah, Salt Lake City, UT, USA Abstract: Hereditary angioedema (HAE is characterized as an episodic swelling disorder with autosomal dominant inheritance. Clinical features include nonpitting edema of external or mucosal body surfaces, and patients often present with swelling of the extremities, abdominal pain, and swelling of the mouth and throat, which can lead to asphyxiation. Patients with HAE classically have no associated urticaria, which is often referred to as nonhistaminergic angioedema. Treatment for HAE involves long-term prophylaxis, short-term prophylaxis, and management of acute attacks. Up until the past few years, acute HAE episodes were predominately treated with supportive measures. Three classes of medications have recently been approved by the US Food and Drug Administration (FDA for the management of acute HAE attacks. Ecallantide, a recombinant protein that acts as a reversible inhibitor of kallikrein, is currently indicated for acute attacks of HAE in those aged 12 years. In two randomized, double-blind, placebo-controlled, multicenter trials, EDEMA3 and EDEMA4, patients treated with 30 mg of ecallantide demonstrated statistically significant improvement in symptoms compared to those on placebo. In addition to its use as treatment for HAE, ecallantide has been used off label in the management of nonhistaminergic angioedema, not due to HAE. Ecallantide has shown promise in the treatment of these other forms; however, data are limited to mainly case reports at this time. Ecallantide is generally a safe and well-tolerated medication; however, based on reports of anaphylaxis, ecallantide does contain a black box warning. Due to the risk of anaphylaxis, ecallantide cannot be self-administered and must be given by a health care professional. Overall, ecallantide is a safe and effective medication for the

  7. Management of acute attacks of hereditary angioedema: role of ecallantide

    Science.gov (United States)

    Duffey, Hannah; Firszt, Rafael

    2015-01-01

    Hereditary angioedema (HAE) is characterized as an episodic swelling disorder with autosomal dominant inheritance. Clinical features include nonpitting edema of external or mucosal body surfaces, and patients often present with swelling of the extremities, abdominal pain, and swelling of the mouth and throat, which can lead to asphyxiation. Patients with HAE classically have no associated urticaria, which is often referred to as nonhistaminergic angioedema. Treatment for HAE involves long-term prophylaxis, short-term prophylaxis, and management of acute attacks. Up until the past few years, acute HAE episodes were predominately treated with supportive measures. Three classes of medications have recently been approved by the US Food and Drug Administration (FDA) for the management of acute HAE attacks. Ecallantide, a recombinant protein that acts as a reversible inhibitor of kallikrein, is currently indicated for acute attacks of HAE in those aged ≥12 years. In two randomized, double-blind, placebo-controlled, multicenter trials, EDEMA3 and EDEMA4, patients treated with 30 mg of ecallantide demonstrated statistically significant improvement in symptoms compared to those on placebo. In addition to its use as treatment for HAE, ecallantide has been used off label in the management of nonhistaminergic angioedema, not due to HAE. Ecallantide has shown promise in the treatment of these other forms; however, data are limited to mainly case reports at this time. Ecallantide is generally a safe and well-tolerated medication; however, based on reports of anaphylaxis, ecallantide does contain a black box warning. Due to the risk of anaphylaxis, ecallantide cannot be self-administered and must be given by a health care professional. Overall, ecallantide is a safe and effective medication for the treatment of acute attacks of HAE. PMID:25931832

  8. 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. PMID:25527240

  9. Conestat alfa for the treatment of angioedema attacks

    Directory of Open Access Journals (Sweden)

    Davis B

    2011-07-01

    Full Text Available Benjamin Davis, Jonathan A BernsteinUniversity of Cincinnati College of Medicine, Department of Internal Medicine, Division of Immunology/Allergy Section, Cincinnati, OH, USAAbstract: Recently, multiple C1 inhibitor (C1-INH replacement products have been approved for the treatment of hereditary angioedema (HAE. This review summarizes HAE and its current treatment modalities and focuses on findings from bench to bedside trials of a new C1-INH replacement, conestat alfa. Conestat alfa is unique among the other C1-INH replacement products because it is produced from transgenic rabbits rather than derived from human plasma donors, which can potentially allow an unlimited source of drug without any concern of infectious transmission. The clinical trial data generated to date indicate that conestat alfa is safe and effective for the treatment of acute HAE attacks.Keywords: androgens, adverse events, patients, HAE attacks 

  10. HAE update: special considerations in the female patient with hereditary angioedema.

    Science.gov (United States)

    Geng, Bob; Riedl, Marc A

    2013-01-01

    This review on hereditary angioedema (HAE) focuses on special topics regarding HAE in female patients. HAE is a bradykinin-mediated disorder, and the role of hormonal regulation of disease expression will be discussed focusing on the effect of estrogen on disease mechanism. The impact of exogenous estrogen on symptom exacerbation leads to special consideration regarding choice of contraceptives and safety of hormone replacement therapy. The effects of pregnancy and childbirth will be examined on the course of disease control. Unique considerations regarding therapeutic management for female HAE patients will be addressed, including the role of C1 inhibitor (C1-INH), ecallantide, and icatibant. Finally, this review will provide an overview of the more recently characterized HAE with normal C1-INH (HAE type III) that predominantly affects women and is in some cases associated with factor XII gene mutations. PMID:23406930

  11. Prospective, double-blind, placebo-controlled trials of ecallantide for acute attacks of hereditary angioedema.

    Science.gov (United States)

    Stolz, Leslie E; Sheffer, Albert L

    2012-01-01

    Hereditary angioedema (HAE) is a rare genetic disorder characterized by unpredictable, episodic, incapacitating attacks of well-demarcated angioedema in the absence of urticaria and pruritus. HAE is due to deficient or dysfunctional C1-esterase inhibitor activity, which results in unopposed activation of plasma kallikrein, resulting in increased levels of bradykinin. Ecallantide is a potent and specific plasma kallikrein inhibitor approved for the treatment of acute attacks of HAE affecting any anatomic site. In Phase III clinical trials, subcutaneously administered ecallantide demonstrated significant, rapid and durable symptom relief. Ecallantide was effective for all attack types, including potentially life-threatening laryngeal attacks. The main safety concern is potentially serious hypersensitivity reactions, including anaphylaxis. Ecallantide represents an important treatment option for the management of acute attacks of HAE. PMID:22149337

  12. Treatment of HAE Attacks in the Icatibant Outcome Survey

    DEFF Research Database (Denmark)

    Hernández Fernandez de Rojas, Dolores; Ibañez, Ethel; Longhurst, Hilary; Maurer, Marcus; Fabien, Vincent; Aberer, Werner; Bouillet, Laurence; Zanichelli, Andrea; Caballero, Teresa; Bygum, Anette

    2015-01-01

    treated with self-administered and HCP-administered icatibant in a real-world setting. METHODS: The Icatibant Outcome Survey (Shire, Zug, Switzerland; NCT01034969) is an international observational study monitoring the safety and effectiveness of icatibant treatment. Descriptive retrospective analyses......-administration. CONCLUSIONS: The proportion of HAE attacks treated with self-administered icatibant increased over time. Patients successfully self-administered icatibant for a wide variety of HAE attacks, demonstrating that icatibant is generally well tolerated and effective for self-administration. Self-administration of...

  13. Treatment response after repeated administration of C1 esterase inhibitor for successive acute hereditary angioedema attacks.

    Science.gov (United States)

    Craig, Timothy J; Bewtra, Againdra K; Hurewitz, David; Levy, Robyn; Janss, Gerti; Jacobson, Kraig W; Packer, Flint; Bernstein, Jonathan A; Rojavin, Mikhail A; Machnig, Thomas; Keinecke, Heinz-Otto; Wasserman, Richard L

    2012-01-01

    Placebo-controlled studies established the efficacy of replacement therapy with C1 esterase inhibitor (C1-INH) concentrate for treating single acute hereditary angioedema (HAE) attacks, but only limited data from prospective studies are available on repeated treatment of successive HAE attacks. This study evaluates the association between repeated treatments with 20 U/kg of C1-INH concentrate (Berinert; CSL Behring, Marburg, Germany) for HAE attacks at any body location and treatment response. In a post hoc analysis of an open-label extension study (International Multicenter Prospective Angioedema C1-INH Trial [I.M.P.A.C.T.2]), the association between repeated treatment with C1-INH and times to onset of symptom relief and complete resolution of HAE symptoms was assessed in patients who were treated for at least 15 attacks by linear regression on the ordinal attack number. Eighteen patients received C1-INH concentrate for at least 15 HAE attacks over a mean duration of 34 months. Demographic and baseline characteristics of these patients were similar to those of all patients in the study. The distribution of body locations and the intensity of HAE attacks were similar for each of the first 15 attacks and subsequent attacks. The extent of previous use of C1-INH concentrate had no effect on the time to onset of symptom relief, the time to complete resolution of HAE symptoms, or the time between attacks treated with C1-INH concentrate; the median of individual linear regression coefficients was not statistically significantly different from 0. Treatment with 20 U/kg of C1-INH concentrate provided consistent treatment response in patients treated for multiple successive HAE attacks at any body location. (Clinicaltrials.gov identifier: NCT00292981). PMID:22856636

  14. 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...... (> or = 5 per year) abdominal attacks was higher (p = .002) among the H. pylori-infected participants of the international study who underwent eradication as compared to the rest of patients. Successful eradication of H. pylori significantly (p = .0006) reduced the number of attacks in these patients as...... of frequent, edematous abdominal attacks may decrease substantially following the eradication of H. pylori from HAE patients infected with this pathogen. Therefore, screening of patients with HAE for H. pylori infection seems warranted. Eradication of H. pylori may lead to a marked reduction in...

  15. Ecallantide: a plasma kallikrein inhibitor for the treatment of acute attacks of hereditary angioedema.

    Science.gov (United States)

    Stolz, L E; Horn, P T

    2010-08-01

    Hereditary angioedema (HAE) is a debilitating, potentially fatal disease characterized by variable and unpredictable acute attacks of swelling affecting the subcutaneous tissue and mucosa. It is an autosomal dominant disorder resulting from a genetic deficiency of functional C1-esterase inhibitor. Available treatments include long-term prophylaxis, short-term prophylaxis and treatment of acute attacks. Ecallantide is a novel, specific and potent inhibitor of plasma kallikrein that was recently approved in the United States for the treatment of acute attacks of HAE in patients aged 16 years and older. In two phase III clinical trials, the subcutaneous administration of 30 mg ecallantide resulted in significantly greater symptom improvement than placebo for acute attacks of HAE. Ecallantide was generally well tolerated throughout the clinical development program. The main safety concern following ecallantide treatment is hypersensitivity reactions, including anaphylaxis. A Risk Evaluation and Management Strategy (REMS) has been implemented to minimize this risk and a long-term observational safety study is currently under way to collect more information about hypersensitivity and immunogenicity. Ecallantide represents a novel treatment option for patients with HAE. PMID:20830315

  16. Fresh Frozen Plasma for the Treatment of Hereditary Angioedema Acute Attacks

    Institute of Scientific and Technical Information of China (English)

    Rui Tang; Shi Chen; Hong-yu Zhang

    2012-01-01

    Objective To determine the safety and efficacy of fresh frozen plasma (FFP) infusion for the treatment of hereditary angioedema (HAE).Methods The medical records of patients with HAE admitted to Peking Union Medical College Hospital who had received FFP infusion during 2004 and 2010 were reviewed and PubMed database from 1966 to the present were searched using the following key words:hereditary angioedema and fresh frozen plasma.The patient's age,sex,body location of HAE attacks,the dose of FFP infusion,time of beginning to improvement,time to complete remission,complication,C1 inhibitor activity,and outcome were analyzed.Results A total of 13 enrolled patients (7 male and 6 female) received 16 times of FFP infusion,including 2 patients undergoing FFP infusion in Peking Union Medical College Hospital and 11 patients reported in the literature.The mean dosage of FFP infusion was 586±337 mL.Two cases suffered from worsening abdominal pain and one case experienced skin rash.Only 1 patient had no improvement in symptom owing to transfusion related reaction.There was a defimite improvement in symptom 49± 19 minutes after beginning FFP infusion.The remission time decreased from 61.7±27.0 hours to 3.3 (2.0,12.0) hours after FFP infusion.FFP infusion was effective for both type Ⅰ and type Ⅱ HAE.Conclusion FFP seems to be safe and effective for acute attacks of HAE.

  17. Urticaria and angioedema

    Directory of Open Access Journals (Sweden)

    Kanani Amin

    2011-11-01

    Full Text Available Abstract Urticaria (hives is a common disorder that often presents with angioedema (swelling that occurs beneath the skin. It is generally classified as acute, chronic or physical. Second-generation, non-sedating H1-receptor antihistamines represent the mainstay of therapy for both acute and chronic urticaria. Angioedema can occur in the absence of urticaria, with angiotensin-converting enzyme (ACE inhibitor-induced angioedema and idiopathic angioedema being the more common causes. Rarer causes are hereditary angioedema (HAE or acquired angioedema (AAE. Although the angioedema associated with these disorders is often self-limited, laryngeal involvement can lead to fatal asphyxiation in some cases. The management of HAE and AAE involves both prophylactic strategies to prevent attacks of angioedema (i.e., trigger avoidance, attenuated androgens, tranexamic acid, and plasma-derived C1 inhibitor replacement therapy as well as pharmacological interventions for the treatment of acute attacks (i.e., C1 inhibitor replacement therapy, ecallantide and icatibant. In this article, the authors review the causes, diagnosis and management of urticaria (with or without angioedema as well as the work-up and management of isolated angioedema, which vary considerably from that of angioedema that occurs in the presence of urticaria.

  18. Socioeconomic burden of hereditary angioedema

    DEFF Research Database (Denmark)

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

    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...

  19. 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

  20. Icatibant for hereditary angioedema.

    Science.gov (United States)

    Gras, Jordi

    2009-12-01

    Hereditary angioedema (HAE) is an autosomal dominant, potentially life-threatening disease, characterized by recurrent self-limiting bouts of edema mainly involving the extremities, genitalia, face, intestines and airways. The prevalence of HAE in the general population has been estimated to be in the range of 1:10,000 to 1:150,000. Currently, acute attacks of HAE are treated mainly symptomatically, with poor outcomes. Recently, it has been demonstrated that bradykinin (BK) is responsible for most of the symptoms of HAE. Icatibant (Firazyr, HOE 140, JE049) is a potent, specific and selective B2 BK receptor antagonist that has recently been approved by the EMEA for the treatment of HAE. In phase III clinical trials, 30 mg of subcutaneous icatibant demonstrated rapid and stable relief from symptoms in cutaneous, abdominal or laryngeal HAE attacks. Local site reactions after subcutaneous injection of icatibant were observed, however, these reactions were mild to moderate in severity and resolved spontaneously and quickly. Icatibant is a new, safe and effective treatment for acute attacks of HAE. PMID:20135020

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

    DEFF Research Database (Denmark)

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

    2015-01-01

    current treatments has substantially improved the quality of life for HAE patients in the United States, similar to a survey of Danish HAE patients regarding the introduction of home treatment. Nevertheless, our study shows that a substantial burden of illness remains for HAE patients.......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...... lives. Patients at a United States HAE Association summit meeting were asked to rate the burden of HAE currently and compare by recall with 2009 when these therapies were not available. Questions covered five domains: psychological/emotional status, ability to carry out daily activities, fear of...

  2. The prophylactic use of C1 inhibitor in hereditary angioedema patients undergoing invasive surgical procedures: a retrospective study

    OpenAIRE

    Gavigan, Geneviève; Yang, William H; Santucci, Stephanie; Harrison, Rachel; Karsh, Jacob

    2014-01-01

    Background Hereditary Angioedema (HAE) is a rare autosomal dominant condition characterized by episodic angioedema, which may be triggered by invasive procedures and surgery. C1 inhibitor (C1 INH) was approved in the United States and Canada in 2009 and 2010, respectively, for the treatment of acute attacks. Most recently in April 2013, it was approved in Europe for short-term prophylaxis (STP), prior to medical, dental, or surgical procedures, to prevent HAE attacks in both children and adul...

  3. 86 The Efficacy and Safety of Human Plasma-derived C1-Inhibitor Concentrate Administered for the Treatment of Attacks in Pediatric Patients with Hereditary Angioedema Due to C1-Inhibitor Deficiency

    OpenAIRE

    Farkas, Henriette; Csuka, Dorottya; Zotter, Zsuzsanna; Szabó, Erika; Kelemen, Zsuzsanna; Varga, Lilian; Fejes, János; Harmat, George

    2012-01-01

    Background Hereditary angioedema due to C1-inhibitor deficiency (HAE-C1-INH) is a life-threatening, rare disease characterized by recurrent edematous attacks. In 50% of cases, the initial onset of symptoms occurs between 5 and 11 years of age. There are limited data on the emergency treatment of acute episodes in pediatric patients. Our aim was to analyze the efficacy and safety of human plasma-derived C1-INH concentrate in our pediatric patient population with HAE-C1-INH. Methods 50 pediatri...

  4. Recent developments in the treatment of acute abdominal and facial attacks of hereditary angioedema: focus on human C1 esterase inhibitor.

    Science.gov (United States)

    Cardona, Lourdes Pastó; Bellfill, Ramon Lleonart; Caus, Joaquim Marcoval

    2010-01-01

    Hereditary angioedema (HAE) is a potentially fatal genetic disorder typified by a deficiency (type I) or dysfunction (type II) of the C1-inhibitor (C1-INH) and characterized by swelling of the extremities, face, trunk, abdominal viscera, and upper airway. Type III is normal estrogen-sensitive C1-INH HAE. Bradykinin, the main mediator of HAE, binds to endothelial B2 receptors, increasing vascular permeability and resulting in edema. HAE management includes short- and long-term prophylaxis. For treating acute episodes, C1-INH concentrate is recommended with regression of symptoms achieved in 30-90 min. Infusions of 500-1000 U have been used in Europe for years. Two plasma-derived C1-INH concentrates have been licensed recently in the United States: Berinert(®) for treating acute attacks and Cinryze(®) for prophylaxis in adolescent/adult patients. A recombinant C1-INH that is being considered for approval (conestat alfa) exhibited significant superiority versus placebo. Ecallantide (Kalbitor(®)) is a selective kallikrein inhibitor recently licensed in the United States for treating acute attacks in patients aged >16 years. It is administered in three 10-mg subcutaneous injections with the risk of anaphylactic reactions. Icatibant (Firazyr(®)) is a bradykinin B2 receptor competitor. It is administered subcutaneously as a 30-mg injection and approved in Europe but not in the United States. PMID:23776358

  5. Cardiac arrest due to airway obstruction in hereditary angioedema.

    Science.gov (United States)

    Fuse, Takashi; Nakada, Taka-aki; Taniguchi, Masashi; Mizushima, Yasuaki; Matsuoka, Tetsuya

    2015-12-01

    Hereditary angioedema (HAE) is a rare genetic disease caused by a deficiency of functional C1 esterase inhibitor that causes swelling attacks in various body tissues. We hereby report a case of out-of-hospital cardiac arrest due to airway obstruction in HAE. Cutaneous swelling and abdominal pain attacks caused by gastrointestinal wall swelling are common symptoms in HAE, whereas laryngeal swelling is rare. Emergency physicians may have few chances to experience cases of life-threatening laryngeal edema resulting in a delay from symptom onset to the diagnosis of HAE. Hereditary angioedema is diagnosed by performing complement blood tests. Because safe and effective treatment options are available for the life-threatening swellings in HAE, the diagnosis potentially reduces the risk of asphyxiation in patients and their blood relatives. PMID:25913082

  6. Management of hereditary angioedema: 2010 Canadian approach

    Directory of Open Access Journals (Sweden)

    Bowen Tom

    2010-07-01

    Full Text Available Abstract 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.

  7. Angioedema

    OpenAIRE

    Kaplan, Allen P

    2008-01-01

    Angioedema can be caused by either mast cell degranulation or activation of the kallikrein-kinin cascade. In the former case, angioedema can be caused by allergic reactions caused by immunoglobulin E (IgE)-mediated hypersensitivity to foods or drugs that can also result in acute urticaria or a more generalized anaphylactic reaction. Nonsteroidal anti-inflammatory drugs (cyclooxygenase 1 inhibitors, in particular) may cause angioedema with or without urticaria, and leukotrienes may have a part...

  8. HAE therapies: past present and future

    Directory of Open Access Journals (Sweden)

    Zuraw Bruce L

    2010-07-01

    Full Text Available Abstract Advances in understanding the pathophysiology and mechanism of swelling in hereditary angioedema (HAE has resulted in the development of multiple new drugs for the acute and prophylactic treatment of patients with HAE. This review will recap the past treatment options, review the new current treatment options, and discuss potential future treatment options for patients with HAE.

  9. 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 the...

  10. Hereditary angioedema: classification, pathogenesis, and diagnosis.

    Science.gov (United States)

    Banerji, Aleena

    2011-01-01

    Hereditary angioedema (HAE) is a rare autosomal dominant genetic disorder associated with a deficiency in C1 inhibitor. More than 200 mutations in this gene, located on chromosome 11, have been identified. Although HAE is often inherited, 20-25% of cases are from new spontaneous mutations and they have no family history of swelling. Decreased C1 inhibitor activity leads to inappropriate activation of multiple pathways, including the complement and contact systems and the fibrinolysis and coagulation systems. Reduced C1 inhibitor activity results in increased activation of plasma kallikrein-kinin system proteases and increased bradykinin levels. Bradykinin is felt to be the main mediator of symptoms in HAE. Patients with HAE have recurrent episodes of swelling of the extremities, abdomen, face, and upper airway. Angioedema involving the gastrointestinal tract can lead to intestinal wall edema, which results in abdominal pain, nausea, vomiting, and diarrhea. Laryngeal swelling is life-threatening and may lead to asphyxia. Common triggers of an attack include trauma, stress, infection, menstruation, oral contraceptives, hormone replacement therapy, and angiotensin-converting enzyme inhibitors. Laboratory testing including C4, C1 inhibitor level, and function is needed to confirm or rule out the diagnosis of HAE. The treatment of HAE has improved significantly in recent years with the availability of several safe and effective therapies. Several consensus guidelines have been created to further assist in the management of HAE patients. This review will provide an update on the classification, pathophysiology, clinical presentation, and diagnosis of HAE. PMID:22221432

  11. HAE international home therapy consensus document

    Directory of Open Access Journals (Sweden)

    Longhurst Hilary J

    2010-07-01

    Full Text Available Abstract Hereditary angioedema (C1 inhibitor deficiency, HAE is associated with intermittent swellings which are disabling and may be fatal. Effective treatments are available and these are most useful when given early in the course of the swelling. The requirement to attend a medical facility 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.

  12. Diagnosis and treatment of hereditary angioedema.

    Science.gov (United States)

    Canonica, G W; Rossi, O

    2012-09-01

    Hereditary angioedema (HAE) is a rare autosomal dominant disorder affecting approximately 1 in 50000 persons. It causes frequent attacks of non-pitting, non-pruritic edema without urticaria, usually of the skin of the extremities, gastrointestinal tract, and upper airways. Gastrointestinal attacks may cause severe pain, and attacks in the laryngeal region may lead to asphyxiation and death. HAE usually begins in childhood or adolescence and persists throughout life. The majority of HAE cases are caused by mutations that result in low levels of functional C1-inhibitor (C1-INH), a serine protease inhibitor that plays regulatory roles in the contact, complement, and fibrinolytic systems. Low C1-INH function results in overproduction of bradykinin, the primary cause of HAE symptoms. Type I HAE is characterized by low levels of functional C1-INH, whereas type II HAE is characterized by normal levels of dysfunctional C1-INH. A third type of HAE has a similar presentation, but is not due to C1-INH deficiency or impairment. Some patients with type III HAE carry mutations in the coagulation factor XII gene that do not alter factor XII plasma levels but markedly increase its activity. HAE is often undiagnosed or misdiagnosed, sometimes leading to inappropriate treatment that may include surgery. HAE should be suspected in any patient who presents with repeated attacks of cutaneous edema without urticaria or recurrent unexplained abdominal pain. Diagnosis requires laboratory testing of complement levels. HAE requires disease-specific treatment with agents that increase functional C1-INH levels and/or reduce the production or activity of bradykinin. These treatments include C1-INH concentrates, icatibant, ecallantide, and attenuated androgens. HAE severely reduces patients' quality of life, which makes supportive care an essential part of the treatment program. PMID:22801442

  13. 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 of...

  14. Angioedema.

    Science.gov (United States)

    Kaplan, Allen P

    2008-06-01

    Angioedema can be caused by either mast cell degranulation or activation of the kallikrein-kinin cascade. In the former case, angioedema can be caused by allergic reactions caused by immunoglobulin E (IgE)-mediated hypersensitivity to foods or drugs that can also result in acute urticaria or a more generalized anaphylactic reaction. Nonsteroidal anti-inflammatory drugs (cyclooxygenase 1 inhibitors, in particular) may cause angioedema with or without urticaria, and leukotrienes may have a particular role as a mediator of the swelling. Reactions to contrast agents resemble allergy with basophil and mast cell degranulation in the absence of specific IgE antibody and can be generalized, that is, anaphylactoid. Angioedema accompanies chronic urticaria in 40% of patients, and approximately half have an autoimmune mechanism in which there is IgG antibody directed to the subunit of the IgE receptor (40%) or to IgE itself (5%-10%). Bradykinin is the mediator of angioedema in hereditary angioedema types I and II (C1 inhibitor [INH] deficiency) and the newly described type III disorder some of which are caused bya mutation involving factor XII. Acquired C1 INH deficiency presents in a similar fashion to the hereditary disorder and is due either toC1 INH depletion by circulating immune complexes or to an IgG antibody directed to C1 INH. Although each of these causes excessive bradykinin formation because of activation of the plasma bradykinin-forming pathway, the angioedema due to angiotensin-converting enzyme inhibitors is caused by excessive bradykinin levels due to inhibition of bradykinin degradation. Idiopathic angioedema (ie, pathogenesis unknown) may be histaminergic, that is, caused by mast cell degranulation with histamine release, or nonhistaminergic. The mediator pathways in the latter case are yet to be defined. A minority may be associated with the same autoantibodies associated with chronic urticaria. Angioedema that is likely to be life threatening (laryngeal

  15. Development of a disease-specific quality of life questionnaire for adult patients with hereditary angioedema due to C1 inhibitor deficiency (HAE-QoL: Spanish multi-centre research project

    Directory of Open Access Journals (Sweden)

    Prior Nieves

    2012-07-01

    Full Text Available Abstract Background There is a need for a disease-specific instrument for assessing health-related quality of life in adults with hereditary angioedema due to C1 inhibitor deficiency, a rare, disabling and life-threatening disease. In this paper we report the protocol for the development and validation of a specific questionnaire, with details on the results of the process of item generation, domain selection, and the expert and patient rating phase. Methods/Design Semi-structured interviews were completed by 45 patients with hereditary angioedema and 8 experts from 8 regions in Spain. A qualitative content analysis of the responses was carried out. Issues raised by respondents were grouped into categories. Content analysis identified 240 different responses, which were grouped into 10 conceptual domains. Sixty- four items were generated. A total of 8 experts and 16 patients assessed the items for clarity, relevance to the disease, and correct dimension assignment. The preliminary version of the specific health-related quality of life questionnaire for hereditary angioedema (HAE-QoL v 1.1 contained 44 items grouped into 9 domains. Discussion To the best of our knowledge, this is the first multi-centre research project that aims to develop a specific health-related quality of life questionnaire for adult patients with hereditary angioedema due to C1 inhibitor deficiency. A preliminary version of the specific HAE-QoL questionnaire was obtained. The qualitative analysis of interviews together with the expert and patient rating phase helped to ensure content validity. A pilot study will be performed to assess the psychometric properties of the questionnaire and to decide on the final version.

  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. 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 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...

  18. 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.

  19. Management of acute attacks of hereditary angioedema: potential role of icatibant

    Directory of Open Access Journals (Sweden)

    Hilary J Longhurst

    2010-09-01

    Full Text Available Hilary J LonghurstDepartment of Immunology, Barts and The London NHS Trust, London, UKAbstract: Icatibant (Firazyr® is a novel subcutaneous treatment recently licensed in the European Union for acute hereditary angioedema. Hereditary angioedema, resulting from inherited partial C1 inhibitor deficiency, is a disabling condition characterized by intermittent episodes of bradykinin-mediated angioedema. Icatibant blocks bradykinin B2 receptors, attenutating the episode. Randomized double-blind, placebo-controlled trials of icatibant, showed significant superiority over oral tranexamic acid in 74 European patients and a trend to improvement in a similar US trial comparing icatibant with placebo in 55 patients. Outcomes for several endpoints did not reach significance in the US trial, perhaps because of low participant numbers and confounding factors: a further trial is planned. Open label studies have shown benefit in multiple treatments for attacks at all sites. Approximately 10% of patients require a second dose for re-emergent symptoms, usually 10 to 27 hours after the initial treatment. Its subcutaneous route of administration, good tolerability and novel mode of action make icatibant a promising addition to the limited repertoire of treatments for hereditary angioedema.Keywords: hereditary angioedema, bradykinin, icatibant, C1 inhibitor deficiency

  20. Pediatric Hereditary Angioedema: Onset, Diagnostic Delay, and Disease Severity.

    Science.gov (United States)

    Christiansen, Sandra C; Davis, Donna K; Castaldo, Anthony J; Zuraw, Bruce L

    2016-09-01

    Hereditary angioedema (HAE) typically presents in childhood. Large gaps remain in our understanding of the natural history of HAE during childhood. We examined age of onset, delay in diagnosis, androgen exposure, and their influence on ultimate disease severity in a large cohort of patients with HAE. Median age of first swelling was 11 years with a median age at diagnosis of 19 years. Earlier onset of symptoms correlated with longer delays in diagnosis (P < .001) and predicted a more severe disease course, including increased number of attacks per year (P = .0009) and hospital admissions (P = .009). Earlier age of onset also significantly correlated with increased perceived HAE severity (P = .0002), negative overall life impact (P < .0001), and use of anabolic androgen. Our observations highlight the importance of early HAE diagnosis and suggest the necessity of a disease management plan once the diagnosis has been made. PMID:26581355

  1. Novel Vasoregulatory Aspects of Hereditary Angioedema: the Role of Arginine Vasopressin, Adrenomedullin and Endothelin-1.

    Science.gov (United States)

    Kajdácsi, Erika; Jani, Péter K; Csuka, Dorottya; Varga, Lilian; Prohászka, Zoltán; Farkas, Henriette; Cervenak, László

    2016-02-01

    The elevation of bradykinin (BK) level during attacks of hereditary angioedema due to C1-Inhibitor deficiency (C1-INH-HAE) is well known. We previously demonstrated that endothelin-1 (ET-1) level also increases during C1-INH-HAE attacks. Although BK and ET-1 are both potent vasoactive peptides, the vasoregulatory aspect of the pathomechanism of C1-INH-HAE has not yet been investigated. Hence we studied the levels of vasoactive peptides in controls and in C1-INH-HAE patients, as well as evaluated their changes during C1-INH-HAE attacks. The levels of arginine vasopressin (AVP), adrenomedullin (ADM) and ET-1 were measured in the plasma of 100 C1-INH-HAE patients in inter-attack periods and of 111 control subjects, using BRAHMS Kryptor technologies. In 18 of the 100 C1-INH-HAE patients, the levels of vasoactive peptides were compared in blood samples obtained during attacks, or in inter-attack periods. AVP, ADM and ET-1 levels were similar in inter-attack samples from C1-INH-HAE patients and in the samples of controls, although cardiovascular risk has an effect on the levels of vasoactive peptides in both groups. The levels of all three vasoactive peptides increased during C1-INH-HAE attacks. Moreover, the levels of ET-1 and ADM as well as their changes during attacks were significantly correlated. This study demonstrated that vascular regulation by vasoactive peptides is affected during C1-INH-HAE attacks. Our results suggest that the cooperation of several vasoactive peptides may be necessary to counterbalance the actions of excess BK, and to terminate the attacks. This may reveal a novel pathophysiological aspect of C1-INH-HAE. PMID:26873707

  2. Icatibant in hereditary angioedema: news and challenges.

    Science.gov (United States)

    Bouillet, Laurence

    2011-05-01

    Hereditary angioedema (HAE) is a rare condition. Its prognosis depends on whether there is laryngeal involvement with a risk of asphyxia, which is present in 25% of such cases. Improved understanding of the pathophysiology of this disease has resulted in the development of targeted therapies including icatibant, which acts as an antagonist at bradykinin B2 receptors. This agent has been shown to be effective in the treatment of attacks of HAE in three Phase III randomized double-blind published studies. Efficacy data have been collected in all types of attack: cutaneous, abdominal and laryngeal. Safety data are also encouraging. Icatibant is administered subcutaneously, with the potential for patients to self-administer. In the future, this therapy may offer increased independence for HAE patients. PMID:21595592

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

    Science.gov (United States)

    Boccon-Gibod, I; Bouillet, L

    2012-06-01

    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

  4. 86 The Efficacy and Safety of Human Plasma-derived C1-Inhibitor Concentrate Administered for the Treatment of Attacks in Pediatric Patients with Hereditary Angioedema Due to C1-Inhibitor Deficiency

    Science.gov (United States)

    Farkas, Henriette; Csuka, Dorottya; Zotter, Zsuzsanna; Szabó, Erika; Kelemen, Zsuzsanna; Varga, Lilian; Fejes, János; Harmat, George

    2012-01-01

    Background Hereditary angioedema due to C1-inhibitor deficiency (HAE-C1-INH) is a life-threatening, rare disease characterized by recurrent edematous attacks. In 50% of cases, the initial onset of symptoms occurs between 5 and 11 years of age. There are limited data on the emergency treatment of acute episodes in pediatric patients. Our aim was to analyze the efficacy and safety of human plasma-derived C1-INH concentrate in our pediatric patient population with HAE-C1-INH. Methods 50 pediatric patients (23 boys, 27 girls; 45 HAE type I, 5 HAE type II patients) were enrolled. The follow-up period began at the time of diagnosis and ended when the patient turned 18 years old. The indications for the use of C1-INH concentrate were upper airway oedema of any severity; moderate-to-severe abdominal edema; edema of face, neck, or lips and severe edema of the extremities and trunk. Clinical and laboratory data were entered into the Hungarian HAE Registry. Results 152 attacks out of 1392 experienced by 42 patients were treated with C1-INH concentrate (28% of attacks at home and 72% at the clinic). The distribution of C1-INH-treated attacks by location was as follows: 38% subcutaneous, 32% abdominal, 30% upper airway. In all locations, the clinical symptoms were consistently relieved by 500 IU C1-INH concentrate. An additional 500 IU dose of C1-INH concentrate was required in 4 cases only. The symptoms improved within 15 to 60 minutes of drug administration. Time to complete resolution was 24 to 48 hours in subcutaneous edema, 12 to 24 hours in abdominal attacks, and less than 12 hours when the edema involved the upper airways. No progression or recurrence of the attack was observed. Repeated administration did not reduce therapeutic efficacy of the drug. Adverse events did not occur. Transmission of viral infections (HIV, HBV, HBC, Parvo virus B19) was not detected. Comparing the first and last year of follow-up, anti-C1-INH antibodies (IgA, IgG, IgM types) did not show any

  5. Bradykinin-mediated angioedema.

    Science.gov (United States)

    Obtułowicz, Krystyna

    2016-02-01

    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. PMID:26842379

  6. The therapeutic potential of a kallikrein inhibitor for treating hereditary angioedema.

    Science.gov (United States)

    Levy, Jerrold H; O'Donnell, Penrose S

    2006-09-01

    Hereditary angioedema (HAE) manifests as intermittent, painful attacks of submucosal oedema affecting the larynx, gastrointestinal tract or limbs. Currently, acute treatment is available in Europe but not USA, and requires intravenous administration of a pooled blood product. HAE is most likely caused by dysinhibition of the contact cascade, resulting in overproduction of bradykinin. DX-88 (ecallantide, Dyax Corp.) is a highly specific recombinant plasma kallikrein inhibitor that halts the production of bradykinin and can be dosed subcutaneously. In a placebo-controlled Phase II trial in patients with HAE, DX-88 resulted in significant improvement in symptoms compared with placebo. A Phase III trial is ongoing. This review explains the pathophysiology of HAE and the mechanism by which DX-88, a non-intravenous, nonplasma-derived therapy, might improve the disease, and discusses the clinical course of HAE and available treatments. Finally, it explores the potential value and efficacy of DX-88 in treating HAE. PMID:16916274

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

    DEFF Research Database (Denmark)

    Elenius Madsen, Daniel; Hansen, Søren; Gram, Jørgen Brodersen; Bygum, Anette; Drouet, Christian; Sidelmann, Johannes Jakobsen

    2014-01-01

    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...

  8. [Hereditary angioedema].

    Science.gov (United States)

    Bouchard, Laura J; Hyry, Heli; Meri, Seppo

    2012-01-01

    Hereditary angioedema (HAE) is a rare autosomal dominant disease characterized by episodic swelling of the face, extremities, larynx, gastrointestinal tract or genitals. Three different subtypes have been identified so far. Type I and II HAE are caused by mutations in the C1 inhibitor gene leading to decreased or dysfunctional C1 inhibitor, respectively. Type III is caused by a mutation in the coagulation factor XII. In addition, acquired forms or forms with no known etiology exist. Increased bradykinin production leading to increased vessel permeability is common to all HAE types. Treatment of HAE has evolved dramatically during the last years as self-administration of C1 inhibitor concentrate and bradykinin-2 receptor antagonist icatibant have become available. PMID:23393928

  9. Non-histaminergic angioedema: focus on bradykinin-mediated angioedema.

    Science.gov (United States)

    Busse, P J; Buckland, M S

    2013-04-01

    Angioedema is a result of increased vascular permeability, with subsequent extravasation of intravascular fluid into the surrounding tissues. Angioedema may be mediated by histamine, bradykinin or other mediators. Histaminergic angioedema generally presents with urticaria and/or pruritus and will respond to conventional treatment with antihistamines, corticosteroids or epinephrine. Bradykinin-mediated angioedema, which includes hereditary angioedema (HAE types I, II and III), acquired C1-INH deficiency, and angiotensin-converting enzyme inhibitor-induced angioedema does not typically present with urticaria/weals and does not respond to conventional agents such as antihistamines or corticosteroids. In recent years, several agents that prevent the generation or activity of bradykinin have been developed for the treatment of HAE types I and II and are also being evaluated in other types of bradykinin-mediated angioedema. These agents have the potential to improve outcomes for patients with different forms of bradykinin-mediated angioedema. PMID:23517034

  10. Hereditary Angioedema

    DEFF Research Database (Denmark)

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

    2014-01-01

    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 of...

  11. Burden of Illness in Hereditary Angioedema: A Conceptual Model.

    Science.gov (United States)

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

    2015-07-01

    The objective of the Hereditary Angioedema Burden of Illness Study in Europe was to assess the real-world experience of hereditary angioedema (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 of HAE on health-related quality of life (HRQoL): (i) unnecessary treatments and procedures, (ii) symptom triggers, (iii) attack impacts, (iv) caregiver impacts, and (v) long-term impacts. Patients for example experience unnecessary medical procedures due to diagnostic delays; anxiety and fear about attacks, and passing HAE to children; reduced work/school productivity; and limited career/educational achievement. Patient caregivers also experience worry and work/activity interruption during the attacks. In conclusion, a conceptual model was developed illustrating the hypothesized relationships among the wide-ranging short- and long-term HRQoL impacts of HAE. These findings can be used to highlight important issues in clinical management, raise awareness of the patients' experience among policymakers and help guide measurement of HRQoL outcomes in future studies in HAE. PMID:25394853

  12. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond.

    Science.gov (United States)

    Agostoni, Angelo; Aygören-Pürsün, Emel; Binkley, Karen E; Blanch, Alvaro; Bork, Konrad; Bouillet, Laurence; Bucher, Christoph; Castaldo, Anthony J; Cicardi, Marco; Davis, Alvin E; De Carolis, Caterina; Drouet, Christian; Duponchel, Christiane; Farkas, Henriette; Fáy, Kálmán; Fekete, Béla; Fischer, Bettina; Fontana, Luigi; Füst, George; Giacomelli, Roberto; Gröner, Albrecht; Hack, C Erik; Harmat, George; Jakenfelds, John; Juers, Mathias; Kalmár, Lajos; Kaposi, Pál N; Karádi, István; Kitzinger, Arianna; Kollár, Tímea; Kreuz, Wolfhart; Lakatos, Peter; Longhurst, Hilary J; Lopez-Trascasa, Margarita; Martinez-Saguer, Inmaculada; Monnier, Nicole; Nagy, István; Németh, Eva; Nielsen, Erik Waage; Nuijens, Jan H; O'grady, Caroline; Pappalardo, Emanuela; Penna, Vincenzo; Perricone, Carlo; Perricone, Roberto; Rauch, Ursula; Roche, Olga; Rusicke, Eva; Späth, Peter J; Szendei, George; Takács, Edit; Tordai, Attila; Truedsson, Lennart; Varga, Lilian; Visy, Beáta; Williams, Kayla; Zanichelli, Andrea; Zingale, Lorenza

    2004-09-01

    Hereditary angioedema (HAE), a rare but life-threatening condition, manifests as acute attacks of facial, laryngeal, genital, or peripheral swelling or abdominal pain secondary to intra-abdominal edema. Resulting from mutations affecting C1 esterase inhibitor (C1-INH), inhibitor of the first complement system component, attacks are not histamine-mediated and do not respond to antihistamines or corticosteroids. Low awareness and resemblance to other disorders often delay diagnosis; despite availability of C1-INH replacement in some countries, no approved, safe acute attack therapy exists in the United States. The biennial C1 Esterase Inhibitor Deficiency Workshops resulted from a European initiative for better knowledge and treatment of HAE and related diseases. This supplement contains work presented at the third workshop and expanded content toward a definitive picture of angioedema in the absence of allergy. Most notably, it includes cumulative genetic investigations; multinational laboratory diagnosis recommendations; current pathogenesis hypotheses; suggested prophylaxis and acute attack treatment, including home treatment; future treatment options; and analysis of patient subpopulations, including pediatric patients and patients whose angioedema worsened during pregnancy or hormone administration. Causes and management of acquired angioedema and a new type of angioedema with normal C1-INH are also discussed. Collaborative patient and physician efforts, crucial in rare diseases, are emphasized. This supplement seeks to raise awareness and aid diagnosis of HAE, optimize treatment for all patients, and provide a platform for further research in this rare, partially understood disorder. PMID:15356535

  13. Hereditary angioedema in childhood: an approach to management.

    Science.gov (United States)

    Ebo, Didier G; Verweij, Marjoke M; De Knop, Kathleen J; Hagendorens, Margo M; Bridts, Chris H; De Clerck, Luc S; Stevens, Wim J

    2010-08-01

    Hereditary angioedema (HAE) is an inherited disorder characterized by recurrent, circumscribed, non-pitting, non-pruritic, and rather painful subepithelial swelling of sudden onset, which fades during the course of 48-72 hours, but can persist for up to 1 week. Lesions can be solitary or multiple, and primarily involve the extremities, larynx, face, esophagus, and bowel wall. Patients with HAE experience angioedema because of a defective control of the plasma kinin-forming cascade that is activated through contact with negatively charged endothelial macromolecules leading to binding and auto-activation of coagulation factor XII, activation of prekallikrein to kallikrein by factor XIIa, and cleavage of high-molecular-weight kininogen by kallikrein to release the highly potent vasodilator bradykinin. Three forms of HAE have currently been described. Type I and type II HAE are rare autosomal dominant diseases due to mutations in the C1-inhibitor gene (SERPING1). C1-inhibitor mutations that cause type I HAE occur throughout the gene and result in truncated or misfolded proteins with a deficiency in the levels of antigenic and functional C1-inhibitor. Mutations that cause type II HAE generally involve exon 8 at or adjacent to the active site, resulting in an antigenically intact but dysfunctional mutant protein. In contrast, type III HAE (also called estrogen-dependent HAE) is characterized by normal C1-inhibitor activity. The diagnosis of HAE is suggested by a positive family history, the absence of accompanying pruritus or urticaria, the presence of recurrent gastrointestinal attacks of colic, and episodes of laryngeal edema. Estrogens may exacerbate attacks, and in some patients attacks are precipitated by trauma, inflammation, or psychological stress. For type I and type II HAE, diminished C4 concentrations are highly suggestive for the diagnosis. Further laboratory diagnosis depends on demonstrating a deficiency of C1-inhibitor antigen (type I) in most kindreds

  14. Treatment of hereditary angioedema with plasma-derived C1 inhibitor

    Directory of Open Access Journals (Sweden)

    Michael J Prematta

    2008-08-01

    Full Text Available Michael J Prematta, Tracy Prematta, Timothy J CraigSection of Allergy and Immunology, Penn State University, Milton S. Hershey Medical Center, PA, USABackground: Plasma-derived C1 inhibitor (C1-INH concentrate is a treatment option for acute hereditary angioedema (HAE attacks and is considered the standard-of-care in many countries, although it is not yet available in the United States. Studies are still being conducted to establish its safety and efficacy as required by the FDA.Objective: To review the medical literature to determine if C1-INH concentrate is a safe and effective treatment for acute HAE attacks.Methods: The following keywords were searched in PubMed and OVID: C1 esterase inhibitor, C1-inhibitor, C1 inhibitor, and hereditary angioedema treatment. English-language articles were searched from 1966 to the present to look for studies demonstrating the efficacy and the safety of C1-INH concentrate.Results: The English-language literature search revealed several studies showing significantly improved relief of HAE symptoms with the administration of C1-INH concentrate – many studies demonstrated some improvement of symptoms within 30 minutes. Side effects have been similar to placebo, and no proven cases of viral transmission have occurred in over 20 years.Conclusion: C1-INH concentrate appears to be a very safe and effective treatment option for HAE.Keywords: hereditary angioedema, c1 inhibitor, c1 esterase inhibitor, hereditary angioedema treatment

  15. Ecallantide: in acute hereditary angioedema.

    Science.gov (United States)

    Garnock-Jones, Karly P

    2010-07-30

    Ecallantide, a recombinant protein that is a selective, highly potent and reversible inhibitor of human plasma kallikrein, is indicated for the treatment of acute attacks of hereditary angioedema (HAE) in patients aged >or=16 years. In the randomized, double-blind, placebo-controlled, multicentre, phase III trial EDEMA3, mean symptom response to treatment at 4 hours (assessed using the Treatment Outcome Score [TOS]; primary endpoint) was significantly greater with a single subcutaneous dose of ecallantide 30 mg than with placebo in patients with acute, moderate to severe attacks of HAE. In addition, the mean change from baseline in symptom severity at 4 hours (assessed using the Mean Symptom Complex Severity [MSCS] scale) was significantly greater with ecallantide than with placebo. At 4 hours in the similarly designed EDEMA4 trial, the mean change from baseline in MSCS score (primary endpoint) and mean TOS were both significantly greater in recipients of a single subcutaneous dose of ecallantide 30 mg than in placebo recipients. Subcutaneous ecallantide 30 mg was generally well tolerated in patients with acute attacks of HAE in the EDEMA3 and EDEMA4 trials. Adverse events were mostly of mild to moderate severity, and no event that was more common in ecallantide than placebo recipients occurred in >10% of patients. PMID:20614949

  16. Coexistence of hereditary angioedema in a case of familial Mediterranean fever with partial response to colchicine

    Science.gov (United States)

    Bahceci, Semiha Erdem; Genel, Ferah; Gulez, Nesrin

    2015-01-01

    Hereditary angioedema (HAE) is a very rare and potentially life-threatening genetic disease characterised by episodes of edema in various parts of the body, including the extremities, face, and airway. The disease is usually associated with attacks of abdominal pain. On the other hand, familial Mediterranean fever (FMF) is an inherited condition characterised by recurrent episodes of painful inflammation in the abdomen, chest, or joints. In this report, we present a child with FMF and undiagnosed HAE, which made him a partial responder to colchicine treatment. Consequently, HAE must be considered in differential diagnosis of cases in which a partial response is obtained from FMF treatment, particularly in countries where FMF is frequently encountered, because early diagnosis of HAE can facilitate prevention of life-threatening complications, such as upper airway obstruction. To our knowledge, our patient is the first patient reported in the literature with the diagnosis of HAE and FMF together. PMID:26155193

  17. 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

  18. 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

  19. Hereditary angioedema: special consideration in children, women of childbearing age, and the elderly.

    Science.gov (United States)

    Kuhlen, James L; Banerji, Aleena

    2015-01-01

    This review on hereditary angioedema (HAE) focused on special topics regarding HAE in children, women of childbearing age, and the elderly. HAE is a rare autosomal dominant bradykinin-mediated disorder characterized by recurrent attacks of subcutaneous or submucosal swelling that usually affects the face, upper airway, extremities, gastrointestinal tract, or genitalia. These recurrent attacks cause significant morbidity and can be life threatening, especially when the swelling affects the airway. Our objective was to summarize the published data available on the disease epidemiology, pathophysiology, clinical presentation, on demand and prophylactic therapy, and focus on management considerations for these special patient populations. Unique aspects of HAE in women with regard to contraception, hormone replacement therapy, pregnancy, lactation, and menopause were also reviewed. PMID:26534748

  20. 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; Varga, Lilian; Farkas, Henriette; Hansen, Karin Møller; Koch, Claus; Skjødt, Karsten; Garred, Peter; Skjoedt, Mikkel-Ole

    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...

  1. Cinryze as the first approved C1 inhibitor in the USA for the treatment of hereditary angioedema: approval, efficacy and safety.

    Science.gov (United States)

    Lunn, Michael; Santos, Carah; Craig, Timothy

    2010-01-01

    Hereditary angioedema (HAE) is a clinical disorder characterized by a deficiency of C1 esterase inhibitor (C1-INH). HAE has traditionally been divided into two subtypes. Unique among the inherited deficiencies of the complement system, HAE Types I and II are inherited as an autosomal dominant disorder. The generation of an HAE attack is caused by the depletion and/or consumption of C1-inhibitor manifested as subcutaneous or submucosal edema of the upper airway, face, extremities, or gastrointestinal tract. Attacks can be severe and potentially life-threatening, particularly with laryngeal involvement. Despite the availability of C1-INH for the treatment of HAE since the 1980s in Europe and other countries, HAE treatment in the United States was limited to androgen therapy. The human plasma-derived C1 esterase inhibitor (Cinryze™), distributed by Lev Pharmaceuticals, was approved in October 2008 for the prevention of HAE attacks based on the results of a phase III clinical trial. This review aims to describe the history of C1-INH replacement in HAE as well as the pharmacology, efficacy and safety of C1-INH, concentrating on Cinryze as the first approved chronic replacement treatment for the prophylaxis of HAE attacks. PMID:22282695

  2. Hereditary Angioedema in Swedish Adults: Report From the National Cohort.

    Science.gov (United States)

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

    2016-04-12

    Hereditary angioedema (HAE) is rare, disabling and sometimes life-threatening. The aim of this study is to describe its prevalence, symptomatology and treatment in Sweden. A total of 146 patients were identified; 110 adults and 36 children with HAE type I (n = 136) or II (n = 10), giving a minimal HAE prevalence of 1.54/100,000. All patients received a written questionnaire followed by a structured telephone interview. This report focuses on the 102 adults who responded. Females reported 19 attacks in the previous year vs. 9 for males (p < 0.01), and females reported 10 days of sick leave vs. 4 days for males (p < 0.05). For all treated acute attacks, plasma-derived C1-inhibitor concentrate (pdC1INH) (used in 27% of patients) had a good effect. For maintenance treatment, 43% used attenuated androgens and 8% used pdC1INH, which reduced their attack rate by more than 50%. In conclusion, the minimal HAE prevalence in Sweden was 1.54/100,000. HAE affected females more severely. Attenuated androgens and pdC1INH had a good effect on preventing attacks. PMID:26540175

  3. Growth factors and IL-17 in hereditary angioedema.

    Science.gov (United States)

    Salemi, M; Mandalà, V; Muggeo, V; Misiano, G; Milano, S; Colonna-Romano, G; Arcoleo, F; Cillari, E

    2016-05-01

    Hereditary angioedema (HAE) is a rare autosomal dominant disorder, due to C1-inhibitor deficiency, which causes episodic swellings of subcutaneous tissues, bowel walls and upper airways which are disabling and potentially life-threatening. We evaluated n = 17 patients with confirmed HAE diagnosis in basal and crisis state and n = 19 healthy subjects. The samples were tested for IL-17, FGFb, G-CSF and GM-CSF, using Bio-plex kit. Data analysis was performed via nonparametric Spearman's correlations and two sets of linear mixed models. When comparing HAE subjects during basal and crisis states, we found out significantly (i.e., p value <0.05) higher values in crisis states rather than in basal states for the three growth factors and cytokine IL-17. When comparing healthy subjects versus HAE patients at basal state, we found out significantly higher values in HAE subjects only for GM-CSF, FGFb and IL-17, but not for G-CSF. In HAE patients, there is a connection between IL-17 and growth factors. The low-grade inflammation in absence of attacks is demonstrated by constant higher amount of IL-17, FGFb and GM-CSF with respect to healthy patients. This could indicate that in this disease there is a level of activation that maintains the system in a "tick-over state," that can be activate by several stimuli that are able to induce a increase in inflammatory mediators during the acute attack. PMID:25773165

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

    Directory of Open Access Journals (Sweden)

    Henao MP

    2016-05-01

    Full Text Available Maria Paula Henao,1 Jennifer L Kraschnewski,1 Theodore Kelbel,2 Timothy J Craig3 1Department of Medicine, 2Division of Allergy and Immunology, 3Department of Medicine and Pediatrics, Pennsylvania State University College of Medicine at Hershey Medical Center, Hershey, PA, USA Abstract: 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

  5. Diagnosis and treatment of bradykinin-mediated angioedema: outcomes from an angioedema expert consensus meeting.

    Science.gov (United States)

    Craig, Timothy J; Bernstein, Jonathan A; Farkas, Henriette; Bouillet, Laurence; Boccon-Gibod, Isabelle

    2014-01-01

    Several types of angioedema exist beyond hereditary angioedema (HAE) types I/II; however, the diagnostic and treatment needs of these conditions are not well understood. Noticeably, there are no licensed treatments available for other forms of angioedema beyond HAE types I/II, and similarly they are unresponsive to conventional antihistamine/glucocorticoid treatment. A group of angioedema experts met in Budapest in May 2013 to discuss such issues, presenting their experience, reviewing available literature and identifying unmet diagnostic and treatment needs in three different angioedema types: HAE with normal C1-inhibitor (C1-INH; previously referred to as type III HAE); nonallergic angiotensin-converting enzyme inhibitor (ACEI)-induced angioedema (ACEI-AAE), and acquired angioedema due to C1-INH deficiency (C1-INH-AAE). The group identified unmet diagnostic and treatment needs in HAE-nC1-INH, C1-INH-AAE and ACEI-AAE, explored remedies and made recommendations on how to diagnose and treat these forms of angioedema. The group discussed the difficulties associated with using diagnostic markers, such as the level and function of C1-INH, C1q and C4 to reliably diagnose the angioedema type, and considered the use of genetic testing to identify mutations in FXII or XPNPEP2 that have been associated with HAE-nC1-INH and ACEI-AAE, respectively. Due to the lack of approved treatments for HAE-nC1-INH, ACEI-AAE and C1-INH-AAE, the group presented several case studies in which therapies approved for treatment of HAE types I/II, such as icatibant, ecallantide and pasteurized, nanofiltered C1-INH, were successful. It was uniformly agreed that further studies are needed to improve the diagnosis and treatment of angioedema other than HAE types I/II. PMID:25401373

  6. On demand treatment and home therapy of hereditary angioedema in Germany - the Frankfurt experience

    Directory of Open Access Journals (Sweden)

    Aygören-Pürsün Emel

    2010-07-01

    Full Text Available Abstract Background Manifestation of acute edema in hereditary angioedema (HAE is characterized by interindividual and intraindividual variability in symptom expression over time. Flexible therapy options are needed. Methods We describe and report on the outcomes of the highly individualized approach to HAE therapy practiced at our HAE center in Frankfurt (Germany. Results The HAE center at the Frankfurt University Hospital currently treats 450 adults with HAE or AAE and 107 pediatric HAE patients with highly individualized therapeutic approaches. 73.9% of the adult patients treat HAE attacks by on-demand therapy with pasteurized pd C1-INH concentrate, 9.8% use additional prophylaxis with attenuated androgens, 1% of the total patient population in Frankfurt has been treated with Icatibant up to now. In addition adult and selected pediatric patients with a high frequency of severe attacks are instructed to apply individual replacement therapy (IRT with pasteurized pd C1-INH concentrate. Improvement on Quality of Life items was shown for these patients compared to previous long-term danazol prophylaxis. Home treatment of HAE patients was developed in the Frankfurt HAE center in line with experiences in hemophilia therapy and has so far been implemented over a period of 28 years. At present 248 (55% of the adult patients and 26 (24% of the pediatric patients are practicing home treatment either as on demand or IRT treatment. Conclusions In conclusion, the individualized home therapies provided by our HAE center, aim to limit the disruption to normal daily activities that occurs for many HAE patients. Furthermore, we seek to optimize the economic burden of the disease while offering a maximum quality of life to our patients.

  7. Hereditary Angioedema due to C1 Inhibitor Deficiency: C1-INH Replacement Therapy

    Directory of Open Access Journals (Sweden)

    Mauro Cancian

    2014-04-01

    Full Text Available Hereditary angioedema (HAE is a rare condition affecting about 1 in 50.000 individuals and caused by a mutation in the gene encoding the C1-esterase inhibitor (C1-INH, which is involved in the control of complement, clotting, fibrinolytic and kinin pathways. HAE is characterized by plasma outflow from blood vessels, leading to fluid collecting (edema in the deep tissue layers of the face, larynx, abdomen, and extremities. Three different types of HAE have been identified: in type I the mutation leads to the lack of production of C1-INH, in type II the mutation leads to the production of dysfunctional C1-INH, while type III is extremely rare and still not fully understood. Therapeutic approaches for HAE include on-demand treatments to stop angioedema attacks and prophylactic treatment to prevent attacks both by pre-procedural (short-term and routine (long-term prophylaxis. Aim of the present review is to present an overview of C1-INH replacement therapy with the plasma-derived concentrate of C1-INH Berinert® (CSL Behring GmbH in the treatment of type I and II HAE.http://dx.doi.org/10.7175/rhc.v5i2.913

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

    Science.gov (United States)

    Ghazi, Aasia; Grant, J Andrew

    2013-01-01

    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 economic burdens. The timely and proper use of disease-specific treatments could improve patients' quality of life, reduce the disease

  9. Treatment of hereditary angioedema with icatibant: efficacy in clinical trials versus effectiveness in the real-world setting.

    Science.gov (United States)

    Maurer, Marcus; Longhurst, Hilary J; Fabien, Vincent; Li, H Henry; Lumry, William R

    2014-01-01

    Icatibant was efficacious and generally well tolerated for type I or II hereditary angioedema (HAE) attacks in adults in the phase III, randomized, placebo-controlled For Angioedema Subcutaneous Treatment (FAST)-3 trial. The Icatibant Outcome Survey (IOS) is an international, observational study assessing icatibant treatment of HAE attacks. We conducted a posthoc analysis to compare for the first time the treatment of HAE type I or II attacks in patients prescribed icatibant in real-world (IOS) versus controlled trial settings (FAST-3). In FAST-3, patients received icatibant administered by health care professionals (HCPs). In IOS, patients self-administered icatibant or were treated by HCPs. Median time to treatment, time to resolution (almost complete resolution [FAST-3] or complete resolution [IOS]), and attack duration in patients who were treated by an HCP were compared between IOS and FAST-3. Descriptive statistical methods compared nonlaryngeal attacks treated less than 12 hours from attack onset. Analysis included 102 patients (376 attacks) from IOS and 43 patients (43 attacks) from FAST-3 (controlled phase). All endpoints were significantly longer for patients in FAST-3 (HCP administration) versus IOS (HCP administration) (p III setting, with a shortened time to symptom resolution and attack duration. ClinicalTrials.gov NCT00912093 (FAST-3); NCT01034969 (IOS). PMID:25198193

  10. [Diagnosis of hereditary angioedema].

    Science.gov (United States)

    Bouillet, Laurence

    2015-01-01

    Hereditary angioedema is a rare disease, potentially life-threatening. It requires a specific treatment. Angioedema without wheals associated with abdominal attacks are very specific of this disease. Antigenemy and functional C1Inhibitor assays are necessary for the diagnosis. The hereditary angioedema with normal C1Inh (type III) is a diagnostic challenge. Bradykinin, secondary to kallikrein-kinin system activation is the key mediator of hereditary angioedema. Female are more symptomatic. Attacks can be induced by menstruations, pregnancies or contraceptive pills. PMID:25511656

  11. 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.

  12. Optimizing hereditary angioedema management through tailored treatment approaches.

    Science.gov (United States)

    Nasr, Iman H; Manson, Ania L; Al Wahshi, Humaid A; Longhurst, Hilary J

    2016-01-01

    Hereditary angioedema (HAE) is a rare but serious and potentially life threatening autosomal dominant condition caused by low or dysfunctional C1 esterase inhibitor (C1-INH) or uncontrolled contact pathway activation. Symptoms are characterized by spontaneous, recurrent attacks of subcutaneous or submucosal swellings typically involving the face, tongue, larynx, extremities, genitalia or bowel. The prevalence of HAE is estimated to be 1:50,000 without known racial differences. It causes psychological stress as well as significant socioeconomic burden. Early treatment and prevention of attacks are associated with better patient outcome and lower socioeconomic burden. New treatments and a better evidence base for management are emerging which, together with a move from hospital-centered to patient-centered care, will enable individualized, tailored treatment approaches. PMID:26496459

  13. Current and emerging management options for hereditary angioedema in the US.

    Science.gov (United States)

    Epstein, Tolly G; Bernstein, Jonathan A

    2008-01-01

    Hereditary angioedema (HAE) is a rare disorder characterized by recurrent attacks of swelling that may involve multiple anatomical locations. In the majority of patients, it is caused by a functional or quantitative defect in the C1 inhibitor (C1-INH), which is an important regulator of the complement, fibrinolytic, kallikrein-kinin and coagulation systems. Standard treatments used for other types of angioedema are ineffective for HAE. Traditional therapies for HAE, including fresh frozen plasma, epsilon-aminocaproic acid and danazol, may be well tolerated and effective in some patients; however, there are limitations both in their safety and efficacy. Several novel therapies have completed phase III trials in the US, including: (i) plasma-derived C1-INH replacement therapies (Berinert P and Cinryze); (ii) a recombinant C1-INH replacement therapy (conestat alfa; Rhucin); (iii) a kallikrein inhibitor (ecallantide [DX-88]); and (iv) a bradykinin-2-receptor antagonist (icatibant). Both Berinert P and Cinryze are reported to have excellent efficacy and safety data from phase III trials. Currently, only Cinryze has been approved for prophylactic use in the US. US FDA approval for other novel agents to treat HAE and for the use of Cinryze in the treatment of acute attacks is pending. PMID:19093699

  14. 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.

  15. Hereditary angioedema: New therapeutic options for a potentially deadly disorder

    Directory of Open Access Journals (Sweden)

    Eidelman Frank J

    2010-05-01

    Full Text Available Abstract Although the biochemistry of hereditary angioedema (HAE is fairly well understood today, the lag in diagnosis of a decade or more suggests that clinicians have low awareness of this disease. This lag in diagnosis and hence treatment certainly stems from the rarity and complexity of the presentation which can be easily mistaken for allergic and non-allergic reactions alike. The symptoms of the disease include acute swelling of any or multiple parts of the body. The attacks may be frequent or rare, and they may vary substantially in severity, causing stomach discomfort or periorbital swelling in mild cases and hypovolemic shock due to abdominal fluid shift or asphyxiation in the most severe cases. Given that these patients are at significant risk for poor quality of life and death, greater awareness of this disease is needed to ensure that newly available, effective medications are used in these patients. These new medications represent significant advances in HAE therapy because they are targeted at the plasma cascades implicated in the pathophysiology of this disease. The clinical presentation of HAE, overlapping symptoms with other angioedemas, and available therapies are reviewed.

  16. Hereditary Angioedema and Gastrointestinal Complications: An Extensive Review of the Literature

    Science.gov (United States)

    Patel, Napoleon; Suarez, Lisbet D.; Kapur, Sakshi; Bielory, Leonard

    2015-01-01

    Hereditary Angioedema (HAE) is a rare autosomal dominant (AD) disease characterized by deficient (type 1) or nonfunctional (type 2) C1 inhibitor protein. The disorder is associated with episodes of angioedema of the face, larynx, lips, abdomen, or extremities. The angioedema is caused by the activation of the kallikrein-kinin system that leads to the release of vasoactive peptides, followed by edema, which in severe cases can be life threatening. The disease is usually not diagnosed until late adolescence and patients tend to have frequent episodes that can be severely impairing and have a high incidence of morbidity. Gastrointestinal involvement represents up to 80% of clinical presentations that are commonly confused with other gastrointestinal disorders such as appendicitis, cholecystitis, pancreatitis, and ischemic bower. We present a case of an HAE attack presenting as colonic intussusception managed conservatively with a C1 esterase inhibitor. Very few cases have been reported in the literature of HAE presentation in this manner, and there are no reports of any nonsurgical management of these cases. PMID:26339513

  17. Intestinal Angioedema Misdiagnosed as Recurrent Episodes of Gastroenteritis

    Directory of Open Access Journals (Sweden)

    LoCascio, Edward J

    2010-09-01

    Full Text Available Emergency physicians (EP frequently encounter angioedema involving the lips and tongue. However, angioedema from Angiotensin Converting Enzyme inhibitors or hereditary angioedema (HAE can present with gastrointestinal symptoms due to bowel wall involvement. EPs should begin to consider this clinical entity as a potential cause for abdominal pain and associated gastrointestinal symptoms given the common use of medications that can precipitate angioedema. We report a case of a 34-year-old woman who presented with abdominal cramping, vomiting and diarrhea due to an acute exacerbation of HAE. [West J Emerg Med. 2010; 11(4:391-394.

  18. An evidence-based review of the potential role of icatibant in the treatment of acute attacks in hereditary angioedema type I and II

    Directory of Open Access Journals (Sweden)

    Floccard B

    2012-09-01

    Full Text Available Bernard Floccard,1 Etienne Hautin,1 Laurence Bouillet,2 Brigitte Coppere,3 Bernard Allaouchiche11Département d'Anesthésie Réanimation, Centre de Référence des Angiœdèmes à Bradykinine, Hospices Civils de Lyon, Hôpital Edouard Herriot, Lyon, 2Clinique Universitaire de Médecine Interne, Centre National de Référence des Angiœdèmes à Bradykinine, CHU de Grenoble, Grenoble, 3Service de Médecine Interne, Centre de Référence des Angiœdèmes à Bradykinine, Hospices Civils de Lyon, Hôpital Edouard Herriot, Lyon, FranceIntroduction: Icatibant, a first-in-class B2 bradykinin receptor antagonist, appears to have a favorable efficacy and safety profile for the treatment of acute attacks of hereditary angioedema in adults.Aims: To update the evidence and provide an overview of the available data on icatibant.Evidence review: Peer reviewed articles published and listed in Medline Search and published updated guidelines for the treatment of acute attacks in hereditary angioedema type I and II in adults were reviewed. The validity and quality of evidence were evaluated.Place in therapy: Clinical evidence for the treatment of acute hereditary angioedema attacks with icatibant is strong. Approximately 10% of the patients require a second dose. No serious adverse reactions have been reported. The only significant side effects consistently registered by 90% of patients are transient local pain, swelling, and erythema at the local injection site.Conclusion: Subcutaneously administered 30 mg icatibant has been shown to be a safe and efficacious treatment in clinical trials. It is the only specific treatment authorized for self-administration by the subcutaneous route offering increased patient independence.Keywords: icatibant, hereditary angioedema, self-administration, acute attacks

  19. Angioedema: Diagnosis and treatment approaches

    Directory of Open Access Journals (Sweden)

    Ali Tahsin Güneş

    2013-03-01

    Full Text Available Angioedema (AE is defined as sudden, localized and transient swelling of the skin and/or mucous membranes. This swelling condition is a result of interstitial edema from vasoactive mediators increasing the permeability of postcapillary venules of the subcutaneous and submucosal tissues. When localized to the skin, it presents as asymmetric, nonpitting, nondependent, and occasionally painful edema. However, mucosal attacks, such as laryngeal edema and bowel involvement can produce severe discomfort and life-threatening symptoms. There are several forms including those involving dysfunction or depletion of the C1-inhibitor gene (classical hereditary AE types and acquired AE, allergic AE, drug-induced AE (nonsteroidal anti-inflammatory drug-induced AE, angiotensin converting enzyme inhibitor-induced AE, idiopathic and a recently described form, HAE type 3. These various forms of AE have overlapping symptoms, but some unique clinical and historical features as well as presence of accompanying urticaria can aid in the differential diagnosis. The key to successful management is to rule out conditions that masquerade as AE, detection and avoidance of triggers, early recognition of attacks, and aggressive airway management when warranted. In this article, common and rare forms as well as clinical symptoms, differential diagnosis, and treatment approaches for AE are reviewed.

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

    Science.gov (United States)

    Zeerleder, Sacha; Levi, Marcel

    2016-06-01

    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

  1. The humanistic burden of hereditary angioedema

    DEFF Research Database (Denmark)

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

    2014-01-01

    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...

  2. 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

    enrolled. We analyzed blood samples drawn during attacks, and obtained 35 samples from the same patients during symptom-free periods. The serum levels of ficolin-2, ficolin-3, MASP-2, ficolin-3/MASP-2 complex, C1-INH, and C4, as well as the extent of ficolin-3-mediated terminal complement complex (FCN3-TCC......) deposition, were measured using ELISA-based methods. RESULTS: Levels of MASP-2 and of the ficolin-3/MASP-2 complex were elevated (P < .0001 and .033, respectively), whereas that of FCN3-TCC was lower (P < .0001) during attacks than during the symptom-free period. During symptom-free periods, FCN3-TCC...

  3. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    Science.gov (United States)

    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. PMID:26819784

  4. 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....

  5. Erythema Marginatum as an Early Symptom of Hereditary Angioedema: Case Report of 2 Newborns.

    Science.gov (United States)

    Martinez-Saguer, Inmaculada; Farkas, Henriette

    2016-02-01

    Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare genetic disease that causes recurrent swelling attacks that may affect various body tissues. Angioedematous attacks can be fatal in the case of upper airway edema and are often preceded by prodromal symptoms like erythema marginatum. Initial symptoms usually occur in the first decade of life. We report on manifestation of profound and recurrent erythema marginatum in 2 newborns. In both cases, prodromal symptoms could help determine the diagnosis of C1-INH-HAE such that, at a later time, angioedematous attacks could be treated promptly and effectively. Awareness of C1-INH-HAE is low among physicians and even lower among the general public. This report aims at raising the level of awareness and shows that initial symptoms of the potentially life-threatening condition can manifest in newborns and that erythema marginatum can even be present at birth. Recognition of early symptoms and timely diagnosis of the disease along with adequate education of the pediatrician and parents are a prerequisite for prompt and effective treatment of attacks and the successful management of the disease. PMID:26759410

  6. Pathogenesis and laboratory diagnosis of hereditary angioedema.

    Science.gov (United States)

    Zuraw, Bruce L; Christiansen, Sandra C

    2009-01-01

    Hereditary angioedema (HAE) was first described in the 19th century. Over the past 50 years, many details of the pathophysiology and molecular biology of HAE have been elucidated. Two types of HAE, type I and type II, result from mutations in the gene for the broad-spectrum protease inhibitor C1 inhibitor (C1INH). Type I HAE is characterized by low antigenic and functional C1INH levels and type II HAE has normal antigenic but low functional C1INH levels. Type III HAE, by contrast, has normal antigenic and functional C1INH levels. In some families, type III HAE has been linked to mutations in Hageman factor. C1INH is the primary inhibitor of the complement proteases C1r and C1s as well as the contact system proteases activated Hageman factor (coagulation factor XIIa and XIIf) and plasma kallikrein. It is also an inhibitor of plasmin and coagulation factor XIa. The primary mediator of swelling in HAE has now been unequivocally shown to be bradykinin, generated from activation of the plasma contact system. The knowledge gained concerning the underlying mechanisms of the different types of HAE allow the clinician to approach the laboratory diagnosis with confidence and provides opportunities for novel therapeutic strategies. PMID:19843402

  7. Clinical efficacy of icatibant in the treatment of acute hereditary angioedema during the FAST-3 trial.

    Science.gov (United States)

    Baş, Murat

    2012-11-01

    Bradykinin is the key mediator of symptoms of hereditary angioedema (HAE), a rare genetic disorder characterized by recurrent episodes of edema of the skin, mucosa and muscle. Icatibant, a bradykinin B(2) receptor antagonist, is an effective and generally well-tolerated treatment option for acute attacks of type I and II HAE. A Phase III randomized, double-blind, placebo-controlled study, FAST-3 (NCT00912093), was designed to further evaluate the efficacy and safety of icatibant in patients presenting with moderate to very severe cutaneous and/or abdominal or mild-to-moderate laryngeal symptoms. Severe laryngeal attacks were treated with open-label icatibant. The controlled phase of FAST-3, completed in October 2010 with results published in December 2011, demonstrated that compared with placebo, icatibant evoked clinically meaningful and statistically significant efficacy across multiple end points in the treatment of type I and II HAE attacks. In addition, icatibant was generally well tolerated and no drug-related serious adverse events were experienced. PMID:23167682

  8. Hereditary angioedema: Historical aspects, classification, pathophysiology, clinical presentation, and laboratory diagnosis.

    Science.gov (United States)

    Khan, David A

    2011-01-01

    Hereditary angioedema (HAE) is a rare disorder first described in 1888 by Sir William Osler. Since then, our understanding of this condition has increased tremendously. This article reviews the historical aspects, classification, pathophysiology, clinical presentation, and laboratory diagnosis of HAE. A review was performed of historical and current literature of HAE. HAE I and II are related to insufficient production of C1-esterase inhibitor (C1-INH) or production of a dysfunctional C1-INH protein, respectively. HAE III is not related to C1-INH deficiency and the pathogenesis is unknown. Bradykinin appears to be the main mediator responsible for angioedema in patients with C1-INH deficiencies. Angioedema of the extremities, face, and upper airway along with gastrointestinal angioedema are the most common clinical features in HAE. The laboratory tests that are most commonly used in the diagnosis of HAE include C4, C1-INH concentration, and C1-INH function. Advances in our understanding of the pathogenesis of HAE have led to several advances in the therapy of this disease. Despite our more thorough understanding of the genetics and pathophysiology of HAE, many questions remain unanswered. PMID:21262092

  9. 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

  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...

  11. Enzymatic pathways in the pathogenesis of hereditary angioedema: the role of C1 inhibitor therapy.

    Science.gov (United States)

    Kaplan, Allen P

    2010-11-01

    A functional abnormality of C1 inhibitor (C1INH) is present in types I and II hereditary angioedema (HAE), and normal C1INH may be rendered ineffective in the newly described type III HAE. C1INH inhibits factor XIIa, factor XII fragment (XIIf), kallikrein, and plasmin. Thus, in its absence, there is marked activation of the bradykinin-forming cascade resulting in severe angioedema. Factor XII may autoactivate on binding to endothelial cell surface gC1qR (receptor for the globular heads of C1q) thus initiating the cascade. Alternatively, stimuli that activate endothelial cells may liberate (or express at the cell surface) heat shock protein 90 or the enzyme prolylcarboxypeptidase, either of which can interact with the prekallikrein-high-molecular-weight kininogen complex to convert prekallikrein to kallikrein stoichiometrically. The kallikrein produced can cleave high-molecular-weight kininogen to produce bradykinin and also recruit factor XII by enzymatically activating it. Patients with type I or II HAE have mutant C1INH so that control of C1 activation is lost. Autoactivation of C1r in the absence of C1INH leads to C1s activation followed by C4 cleavage and depletion. An attack of swelling is accompanied by conversion of factor XIIa to factor XIIf and further enzymatic activation of C1r so that C4 levels drop further and C2 is depleted. New therapies for HAE focus on the bradykinin-forming cascade and include a kallikrein inhibitor and a bradykinin B-2 receptor antagonist in addition to administration of purified C1INH. PMID:20889195

  12. Angioedema hereditário: considerações sobre terapia Therapeutic approach of hereditary angioedema

    Directory of Open Access Journals (Sweden)

    Kélem de Nardi Chagas

    2004-09-01

    HAE ser causado pelo mesmo defeito e acometer membros da mesma família, diferentes critérios têm sido estabelecidos para o tratamento desses pacientes. Foram indicados diferentes esquemas terapêuticos para HAE e alguns dos pacientes puderam ser seguidos sem terapia medicamentosa.PURPOSE: Hereditary Angioedema was first described by William Osler in 1888 and it is caused by a hereditary or acquired deficiency of C1 esterase inhibitor (C1-INH. Treatment is indicated for acute attacks or prophylaxis of angioedema which occur in the subcutaneous tissue respiratory or gastrointestinal tracts. Treatment includes attenuated androgens, inhibitors of kininogen or plasminogen, like tranexamic acid or e-aminocaproic acid and the administration of C1-INH concentrate. We describe the peculiarities of the treatment chosen for 10 patients (4 families with HAE and their evolution. METHODS: Ten patients (1-38 years old with HAE were diagnosed by clinical history and laboratory evaluation. The following tests were performed for the complement system: C1-INH, C4 and C3 levels and hemolytic assay (CH50 and APH50 for the classic and alternative pathways. Treatment was initiated considering severity of symptoms, age, gender and therapeutic response of the patient. RESULTS: Clinical evaluation showed: 4/10 patients with recurrent subcutaneous edema; 3/10 with previous laryngeal edema and 3/10 with sporadic symptoms. Different severity of symptoms was verified in the same family. The laboratory evaluation detected: low C1-INH levels (10/10; low serum C4 level (8/10; undetectable CH50 (3/10 and low CH50 levels (6/10; low APH50 levels (2/10. Six out of ten patients did not receive any specific treatment and 2 of them had high risk of asphyxia. One adolescent had been controlled with e-aminocaproic acid, one child had been changed from danazol to tranexamic acid, a 30 year old female patient had received oxandrolone and a 38 year old man had been treated with danazol. CONCLUSIONS: Although

  13. Hereditary Angioedema: Report of Three Cases and Approach to Diagnosis and Management

    Directory of Open Access Journals (Sweden)

    Sadiye Kuş

    2009-06-01

    Full Text Available Hereditary angioedema (HAE is a distinctive form of recurrent angioedema with life threatening consequences. Type I is defined with quantitative C1 esterase inhibitor (C1 INH deficiency, type II with functional C1 INH deficency and type III with normal quantity and function of C1 INH respectively. Here in, We present three cases with HAE and discuss diagnostic and therapeutic issues.

  14. 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 ...

  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 ...

  16. 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; Kocsis, Andrea; Zotter, Zsuzsanna; Gál, Péter; Varga, Lilian; Farkas, Henriette; Füst, George; Garred, Peter

    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 in...

  17. 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; Monnier, N; Lunardi, J; Drouet, C

    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...

  18. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema

    OpenAIRE

    Bowen Tom; Cicardi Marco; Farkas Henriette; Bork Konrad; Longhurst Hilary J; Zuraw Bruce; Aygoeren-Pürsün Emel; Craig Timothy; Binkley Karen; Hebert Jacques; Ritchie Bruce; Bouillet Laurence; Betschel Stephen; Cogar Della; Dean John

    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...

  19. 2010 international consensus algorithm for the diagnosis, therapy and management of hereditary angioedema

    OpenAIRE

    Bowen, Tom; Cicardi, Marco; Farkas, Henriette; Bork, Konrad; Longhurst, Hilary J; Zuraw, Bruce; Aygören-Pürsün, Emel; Craig, Timothy; Binkley, Karen; Hebert, Jacques; Ritchie, Bruce; Bouillet, Laurence; Betschel, Stephen; Cogar, Della; Dean, John

    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 Hered...

  20. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema

    OpenAIRE

    Bowen, Tom; Cicardi, Marco; Farkas, Henriette; Bork, Konrad; Longhurst, Hilary J; Zuraw, Bruce; Aygoeren-Pürsün, Emel; Craig, Timothy; Binkley, Karen; Hebert, Jacques; Ritchie, Bruce; Bouillet, Laurence; Betschel, Stephen; Cogar, Della; Dean, John

    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 Hered...

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

    OpenAIRE

    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 wit...

  2. Hereditary Angioedema Presenting as Recurrent Acute Pancreatitis.

    Science.gov (United States)

    Berger, Tal D; Garty, Ben-Zion

    2016-02-01

    Hereditary angioedema (HAE) may manifest with swelling of the face, extremities, and upper airways. Gastrointestinal symptoms are also common and may include abdominal pain, vomiting, and diarrhea. However, pancreatic involvement is rare and has been reported only in a few adults with previously diagnosed HAE. We describe a 6-year-old boy who presented with recurrent severe abdominal pain accompanied by an elevation in pancreatic enzyme levels, without subcutaneous or cutaneous angioedema. His symptoms had begun 18 months earlier, and he was hospitalized several times before the present admission with a diagnosis of acute pancreatitis. More comprehensive analysis yielded low levels of C2, C4, CH50, and C1 esterase inhibitor, establishing the diagnosis of HAE. One year after diagnosis, swelling of the extremities appeared for the first time. This is the first report of a child in whom pancreatic disease was the presenting symptom of HAE. HAE should be included in the differential diagnosis of recurrent pancreatitis in children. PMID:26812927

  3. 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.

    Science.gov (United States)

    Hansen, Cecilie Bo; Csuka, Dorottya; Munthe-Fog, Lea; Varga, Lilian; Farkas, Henriette; Hansen, Karin Møller; Koch, Claus; Skjødt, Karsten; Garred, Peter; Skjoedt, Mikkel-Ole

    2015-10-15

    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-1 was shown to cleave high m.w. kininogen into bradykinin; therefore, we hypothesized that MASP-1 levels and the quantity of MASP-1/C1-INH complexes might be associated with different paraclinical and clinical outcomes of HAE. We measured MASP-1 serum concentrations and endogenous MASP-1/C1-INH complex levels in 128 HAE patients and 100 controls. Relatively high levels of pre-existing MASP-1/C1-INH complexes were observed in normal serum, and we found that both the serum levels of MASP-1 and the complex formation between MASP-1 and C1-INH were significantly reduced in HAE patients compared with matched controls (p < 0.0001). The level of MASP-1 and MASP-1/C1-INH complexes in HE patients correlated with the level of C1-INH (p = 0.0009 and p = 0.0047, respectively), the level of C4 (p = 0.0084 and p < 0.0001, respectively), and the number of attacks in the year of blood sampling (p = 0.0075 and p = 0.0058, respectively). In conclusion, we show that MASP-1/C1-INH complexes circulate in normal human blood. The levels of MASP-1 and MASP-1/C1-INH complexes are reduced in HAE patients compared with controls. Both MASP-1 and MASP-1/C1-INH complexes are related to the degree of complement C4 consumption, as well as the severity of disease. These results suggest that MASP-1 may exert a previously unrecognized role in the pathophysiology of HAE. PMID:26371246

  4. Update on laboratory tests for the diagnosis and differentiation of hereditary angioedema and acquired angioedema.

    Science.gov (United States)

    Frazer-Abel, Ashley; Giclas, Patricia C

    2011-01-01

    The importance of laboratory testing in the diagnosis of hereditary angioedema (HAE) has increased with the advent of new treatment options in recent years. It has been 50 years since HAE was linked to a decrease of C1INH (the inhibitor of complement enzyme, C1 esterase), a link that provided for the first laboratory test available for this disorder. HAE is subdivided into types that can be differentiated only by laboratory testing. The Type I form is characterized by low levels and function of C1INH in the circulation. The Type II form is characterized by normal levels of C1INH, but low function. Sample collection and handling is critical for the functional assays. The serum samples for the functional analysis must be collected, separated, and frozen at less than -60°C within 2 hours of the blood draw. Additionally some suspected Type II patients may benefit from looking closely at what method is used for the functional testing. The acquired forms of angioedema (AAE) can benefit from the same clinical testing, because most are ultimately due to decreased C1INH. Measurement of C1q levels and testing for anti-C1INH autoantibodies can help differentiate AAE from HAE. Diagnostic testing for the third hereditary form, alternately called estrogen-dependent HAE, HAE with Normal C1INH or HAE Type III, still presents challenges, and definitive testing may have to wait until there is a more complete understanding of this mixed group of patients. The next steps will include genetic analysis of C1INH and other proteins involved in HAE. PMID:22195757

  5. Hereditary Angioedema: Three Cases Report, Members of the Same Family

    Directory of Open Access Journals (Sweden)

    Alexandros Kolokotronis

    2010-01-01

    Full Text Available Background: This current clinical case report highlights three cases of Hereditary angioedema (HAE patients who are all members of the same family (father and his two daughters. The father has C1–INH deficiency, while his daughters have low C1–INH levels: the first possesses only 10% function and the second has low C1–INH level with 0% function. Of note, the second daughter was discovered to have HAE at the age of 2, thus making her the youngest known HAE case report in the English literature.Methods: Assess the efficacy of administration of C1-INH before dental operation as regards the prevention of HAE episode, when total or partial C1-INH deficiency exists.Results: Acute angioedema leading to laryngeal oedema is a possibly fatal complication for HAE patients undergoing dental procedures. Use of both short-term and long-term HAE prophylaxis prior to dental operations might be life saving for those patients.Conclusions: Prevention and early recognition of potential laryngeal oedema that can occur as a complication of dental procedures may be lifesaving for HAE patients.

  6. Hereditary angioedema presenting as irritable bowel syndrome: a case of early closure

    Directory of Open Access Journals (Sweden)

    Karim M. Benrajab

    2015-10-01

    Full Text Available Abdominal pain is one of the most common reasons for outpatient and emergency department visits. We present one such case of early closure in a 32-year-old male with recurrent abdominal pain who was diagnosed with irritable bowel syndrome (IBS. Family history was suspicious for hereditary angioedema (HAE. The HAE workup came back positive, and the patient was started on prophylactic therapy, which led to an improvement in symptoms and quality of life. The purpose of this case is to create awareness among physicians to test for HAE in patients diagnosed with IBS who, based on their history or physical examination, have clinical suspicion for HAE.

  7. Hereditary angioedema presenting as irritable bowel syndrome: a case of early closure

    Science.gov (United States)

    Benrajab, Karim M.; Singh, Gurkeerat; Obah, Eugene

    2015-01-01

    Abdominal pain is one of the most common reasons for outpatient and emergency department visits. We present one such case of early closure in a 32-year-old male with recurrent abdominal pain who was diagnosed with irritable bowel syndrome (IBS). Family history was suspicious for hereditary angioedema (HAE). The HAE workup came back positive, and the patient was started on prophylactic therapy, which led to an improvement in symptoms and quality of life. The purpose of this case is to create awareness among physicians to test for HAE in patients diagnosed with IBS who, based on their history or physical examination, have clinical suspicion for HAE. PMID:26486119

  8. New mutations in SERPING1 gene of Brazilian patients with hereditary angioedema.

    Science.gov (United States)

    Cagini, Nathália; Veronez, C L; Constantino-Silva, R N; Buzolin, Márcia; Martin, Renan Paulo; Grumach, A S; Velloso, Lício Augusto; Mansour, Eli; Pesquero, João Bosco

    2016-04-01

    Hereditary Angioedema is an autosomal dominant inherited disease leading to oedema attacks with variable severity and localization predominantly caused by C1-INH deficit. More than 400 mutations have been already identified, however no genetic analysis of a Brazilian cohort of HAE patients with C1-INH deficiency has been published. Our aim was to perform genetic analysis of C1-INH gene (SERPING1) in Brazilian HAE patients. We screened the whole SERPING1 coding region from 30 subjects out of 16 unrelated families with confirmed diagnosis of HAE due to C1-INH deficiency. Clinical diagnosis was based on symptoms and quantitative and/or functional analysis of C1-INH. We identified fifteen different mutations among which eight were not previously described according to databases. We found five small deletions (c.97_115del19; c.553delG; c.776_782del7; c.1075_1089del15 and c.1353_1354delGA), producing frameshifts leading to premature stop codons; seven missense mutations (c.498C>A; c.550G>C; c.752T>C; c.889G>A; c.1376C>A; c.1396C>T; c.1431C>A); one nonsense mutation (c.1480C>T), and two intronic alterations (c.51+1G>T; c.51+2T>C). Despite the small number of participants in this study, our results show mutations not previously identified in SERPING1 gene. This study represents the first Brazilian HAE cohort evaluated for mutations and it introduces the possibility to perform genetic analysis in case of need for differential diagnosis. PMID:26812872

  9. Obstetrical Complications and Outcome in Two Families with Hereditary Angioedema due to Mutation in the F12 Gene.

    Science.gov (United States)

    Picone, Olivier; Donnadieu, Anne-Claire; Brivet, François G; Boyer-Neumann, Catherine; Frémeaux-Bacchi, Véronique; Frydman, René

    2010-01-01

    Backgroud. Hereditary angioedema (HAE) is characterized by recurrent swelling of the skin, the abdomen (causing severe acute pain), and the airways. A recently discovered type caused by mutations in the factor XII gene (designated as HAE type III) occurs mainly in women. Estrogens may play an important role, but few obstetrical complications have been reported. Case. We report the symptoms and obstetrical complications of women in two families with HAE attributable to the p. Thr328Lys mutation in the F12 gene. Clinical manifestations included acute and severe maternal abdominal pain, with transient ascites, laryngeal edema, and fetal and neonatal deaths. Patients had normal C4 levels and a normal C1 inhibitor gene. Administration of C1-inhibitor concentration twice monthly decreased the attack rate in one mother, and its predelivery administration (1000 U) led to the delivery of healthy girls. Conclusions. Obstetricians and anesthesiologists should be aware of this rare cause of unexplained maternal ascites and in utero or fetal death associated with edema. PMID:20490261

  10. [Bradykinin mediated angioedema].

    Science.gov (United States)

    Bouillet, L; Boccon-Gibod, I; Massot, C

    2011-04-01

    Bradykinin angioedema (AE) are characterized by acute recurrent episodes of localized swelling. They are not associated with pruritus or erythema, and are short-lived (24 to 72 hours), disappearing without any sequelae. Corticosteroids are useless. Skin or mucous membranes (upper respiratory and intestinal) could be affected. Bradykinin AE can be secondary to: (1) AE associated with C1 inhibitor deficiency (hereditary or acquired); (2) drug-induced AE (converting enzyme inhibitors…); (3) type III AE type (oestrogen dependant) without C1 inhibitor deficiency. These type III AE can be associated with a gain of function mutation that markedly increases factor XII activity. Prognosis depends on the laryngeal attacks (resulting in 25 % of death in the absence of specific treatment). In case of severe attacks, icatibant (bradykinin receptor antagonist) or C1 inhibitor concentrate can be used. In case of frequent attacks, long-term therapy with danazol or tranexamic acid is effective. PMID:20538389

  11. [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. PMID:26597136

  12. Angioedema hereditario en pediatría Hereditary pediatric angioedema

    Directory of Open Access Journals (Sweden)

    A. Calvo Gómez-Rodulfo

    2009-01-01

    Full Text Available

    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 pubertad. Su madre y un hermano presentaban sintomatología similar. En los tres casos se demostró deficiencia de C1-INH, siendo diagnosticados de angioedema hereditario.
    Conclusiones: El angioedema hereditario es una entidad poco frecuente y potencialmente grave. Aunque la sintomatología puede ser similar a cuadros alérgicos y anafilácticos, el manejo es muy diferente, siendo la administración intravenosa de C1-INH de elección en el tratamiento del episodio agudo grave. Debe considerarse también la necesidad de tratamiento profiláctico a largo plazo ante el antecedente de episodio de angioedema grave o cuando los episodios se repitan frecuentemente. En este artículo se revisan los distintos aspectos diagnósticos y terapéuticos del angioedema hereditario.

    Introduction: The hereditary angioedema is a rare genetical disease caused by deficiency of C1 esterase inhibitor (C1-INH. The diagnosis is difficult because the low prevalence f it.
    Clinical report: We show a female with recurrent episodes of edema in extremities since 3 years old, with dysphagia and voice change since puberty. Her mother and brother had similar manifestations. All they were diagnosed of hereditary angioedema with C1-INH deficiency.
    Conclusions: The hereditary angioedema is a rare and potential severe disease. Its manifestations may be similar to the allergy or anaphylaxis, but its treatment is different. Emergency therapy of acute oedematous attacks with C1

  13. Management of hereditary angioedema in pregnant women: a review

    Directory of Open Access Journals (Sweden)

    Caballero T

    2014-09-01

    Full Text Available Teresa Caballero,1,2 Julio Canabal,1 Daniela Rivero-Paparoni,1 Rosario Cabañas1 1Hospital La Paz Institute for Health Research, (IdiPaz 2Biomedical Research Network on Rare Diseases-U754 (CIBERER, Madrid, Spain Abstract: Three types of hereditary angioedema (HAE have been described: two are due to C1 inhibitor (C1-INH deficiency (C1-INH-HAE types I and II and one is characterized by normal C1-INH (nC1-INH-HAE. The management of pregnancy in patients with HAE is often a clinical challenge owing to potential worsening of the disease in relation to the physiological increase in estrogens and the limited treatment options. This review addresses the potential influence of pregnancy on the clinical severity of hereditary angioedema and the management of this disease during pregnancy with currently available treatments. Keywords: hereditary angioedema, pregnancy, female, treatment, C1 inhibitor concentrate, tranexamic acid

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

    OpenAIRE

    Henao, Maria Paula

    2016-01-01

    Maria Paula Henao,1 Jennifer L Kraschnewski,1 Theodore Kelbel,2 Timothy J Craig3 1Department of Medicine, 2Division of Allergy and Immunology, 3Department of Medicine and Pediatrics, Pennsylvania State University College of Medicine at Hershey Medical Center, Hershey, PA, USA Abstract: 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...

  15. 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

  16. 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.

  17. Burden of Illness in Hereditary Angioedema

    DEFF Research Database (Denmark)

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

    2015-01-01

    attacks, and passing HAE to children, reduced work/school productivity, and limited career/educational achievement. Patient caregivers also experienced worry and work/activity interruption during the attacks. In conclusion, a conceptual model was developed illustrating the hypothesized relationships among...

  18. A Nationwide Study of Norwegian Patients with Hereditary Angioedema with C1 Inhibitor Deficiency Identified Six Novel Mutations in SERPING1

    Science.gov (United States)

    Johnsrud, Irene; Kulseth, Mari Ann; Rødningen, Olaug Kristin; Landrø, Linn; Helsing, Per; Waage Nielsen, Erik; Heimdal, Ketil

    2015-01-01

    Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is characterized by relapsing, non-pruritic swelling in skin and submucosal tissue. Symptoms can appear in early infancy when diagnosis is more difficult. In the absence of a correct diagnosis, treatment of abdominal attacks often lead to unnecessary surgery, and laryngeal edema can cause asphyxiation. A cohort study of 52 patients from 25 unrelated families in Norway was studied. Diagnosis of C1-INH-HAE was based on international consensus criteria including low functional and/or antigenic C1-INH values and antigenic C4. As SERPING1 mutations in Norwegian patients with C1-INH-HAE are largely undescribed and could help in diagnosis, we aimed to find and describe these mutations. Mutation analysis of the SERPING1 gene was performed by Sanger sequencing of all protein coding exons and exon-intron boundaries. Samples without detected mutation were further analyzed by multiplex ligation-dependent probe amplification to detect deletions and duplications. Novel mutations suspected to lead to splice defects were analyzed on the mRNA level. Fifty-two patients from 25 families were included. Forty-four (84,6%) suffered from C1-INH-HAE type I and eight (15,4%) suffered from C1-INH-HAE type II. Pathogenic or likely pathogenic mutations were found in 22/25 families (88%). Thirteen unique mutations were detected, including six previously undescribed. There were three missense mutations including one mutation affecting the reactive center loop at codon 466, three nonsense mutations, three small deletions/duplications, three gross deletions, and one splice mutation. PMID:26154504

  19. 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...... abdominal area, 17% had diarrhoea, 11% had vomiting and 6% fainted during attacks. Non-hereditary angioedema has high lifetime prevalence and becomes chronic in approximately 50% of affected patients. Symptoms in the larynx and throat, as well as non-specific symptoms, such as dizziness and abdominal pain......) reported a lifetime prevalence of 7.4% for angioedema. In both groups symptoms were most frequent in the lips, head, neck, eyes and tongue. In the C1 INH test normal group angioedema was still active at the time of the study in 53% of the patients, and 36% reported symptoms in the throat, 23% in the...

  20. Increased activity of coagulation factor XII (Hageman factor) causes hereditary angioedema type III.

    Science.gov (United States)

    Cichon, Sven; Martin, Ludovic; Hennies, Hans Christian; Müller, Felicitas; Van Driessche, Karen; Karpushova, Anna; Stevens, Wim; Colombo, Roberto; Renné, Thomas; Drouet, Christian; Bork, Konrad; Nöthen, Markus M

    2006-12-01

    Hereditary angioedema (HAE) is characterized clinically by recurrent acute skin swelling, abdominal pain, and potentially life-threatening laryngeal edema. Three forms of HAE have been described. The classic forms, HAE types I and II, occur as a consequence of mutations in the C1-inhibitor gene. In contrast to HAE types I and II, HAE type III has been observed exclusively in women, where it appears to be correlated with conditions of high estrogen levels--for example, pregnancy or the use of oral contraceptives. A recent report proposed two missense mutations (c.1032C-->A and c.1032C-->G) in F12, the gene encoding human coagulation factor XII (FXII, or Hageman factor) as a possible cause of HAE type III. Here, we report the occurrence of the c.1032C-->A (p.Thr328Lys) mutation in an HAE type III-affected family of French origin. Investigation of the F12 gene in a large German family did not reveal a coding mutation. Haplotype analysis with use of microsatellite markers is compatible with locus heterogeneity in HAE type III. To shed more light on the pathogenic relevance of the HAE type III-associated p.Thr328Lys mutation, we compared FXII activity and plasma levels in patients carrying the mutation with that of healthy control individuals. Our data strongly suggest that p.Thr328Lys is a gain-of-function mutation that markedly increases FXII amidolytic activity but that does not alter FXII plasma levels. We conclude that enhanced FXII enzymatic plasma activity in female mutation carriers leads to enhanced kinin production, which results in angioedema. Transcription of F12 is positively regulated by estrogens, which may explain why only women are affected with HAE type III. The results of our study represent an important step toward an understanding of the molecular processes involved in HAE type III and provide diagnostic and possibly new therapeutic opportunities. PMID:17186468

  1. Treatment of hereditary angioedema with nanofiltered C1-esterase inhibitor concentrate (Cetor®): multi-center phase II and III studies to assess pharmacokinetics, clinical efficacy and safety.

    Science.gov (United States)

    Hofstra, J J; Kleine Budde, I; van Twuyver, E; Choi, G; Levi, M; Leebeek, F W G; de Monchy, J G R; Ypma, P F; Keizer, R J; Huitema, A D R; Strengers, P F W

    2012-03-01

    From 1997, plasma-derived C1-inhibitor concentrate (Cetor®) has been available to HAE and AAE patients. Recently, a virus reducing 15 nm nanofiltration step has been introduced in the production process. A randomized, double-blind controlled cross-over study was performed to compare the pharmacokinetics (PK) of nanofiltered (C1-INH-NF) with conventional C1-inhibitor (C1-INH). Efficacy and safety were investigated in an open-label, on-demand and a prophylactic study. No differences in pharmacokinetic parameters between C1-INH and C1-INH-NF were found (13 non-symptomatic HAE patients). Both C1-inhibitor products equally increased plasma C4 levels. In the on-demand study, 14 acute angioedema attacks in 8 patients were analyzed. In the prophylactic study, 1 AAE and 5 HAE patients experienced in total 31 attacks during 748 observation days. In total 180,000 units of C1-INH-NF were administered. No product-related adverse events occurred, and no anti-C1-antibodies were induced. Nanofiltration in the production process of C1-inhibitor did not affect the pharmacokinetics, efficacy, and safety. PMID:22197071

  2. Radiologic manifestations of angioedema

    OpenAIRE

    Ishigami, Kousei; Averill, Sarah L.; Pollard, Janet H.; McDonald, Joshua M.; Sato, Yutaka

    2014-01-01

    Objectives The purpose of this pictorial review is to present imaging findings of angioedema involving the various organs. Conclusion The role of imaging for patients with angioedema includes the evaluation of the upper airway for obstruction and the exclusion of other possible aetiologies, such as neoplastic or infectious processes. Glossomegaly is a common finding of head and neck angioedema. Angioedema may involve organ systems beyond the superficial regions and the head and neck including...

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

    Science.gov (United States)

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

    2016-03-01

    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 leading physicians to refrain from testing for HAE. Few studies have been published on urticaria and prodromal symptoms in HAE, thus the incidence of these parameters were also investigated. A total of 87 patients affiliated to the national HAE Centre were included. Retrospective and prospective data on skin eruptions and prodromal symptoms were collected. Fifty-six percent of 87 patients had a positive history of erythema marginatum. Half of the patients had experienced erythema marginatum being misinterpreted as urticaria. The most prevalent other prodromal symptoms were other skin symptoms, malaise, psychological changes, fatigue and gastrointestinal symptoms. HAE patients with erythema marginatum have a longer diagnostic delay, presumably caused by misinterpretation of the rash as urticaria. PMID:26336842

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

    DEFF Research Database (Denmark)

    Busse, Paula; Bygum, Anette; Edelman, Jonathan; Lumry, William; Machnig, Thomas; Martinez-Saguer, Inmaculada; Rojavin, Mikhail

    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...

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

    DEFF Research Database (Denmark)

    Varga, L; Széplaki, G; Laki, J; Kocsis, A; Kristóf, K; Gál, P; Bajtay, Z; Wieslander, J; Daha, M R; Garred, P; Madsen, H O; Füst, G; Farkas, H

    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...

  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

    2015-01-01

    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. 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....

  8. C1-inhibitor (C1-INH) autoantibodies in hereditary angioedema. Strong correlation with the severity of disease in C1-INH concentrate naïve patients.

    Science.gov (United States)

    Varga, Lilian; Széplaki, Gábor; Visy, Beáta; Füst, George; Harmat, George; Miklós, Katalin; Németh, Julianna; Cervenak, László; Karádi, István; Farkas, Henriette

    2007-02-01

    The presence of autoantibodies to C1-inhibitor (C1-INH-Abs) is a hallmark of acquired C1-inhibitor deficiency. However, only scarce data are available on their prevalence in hereditary angioedema (HAE). In a prospective study performed between 2001 and 2004 in 95 patients with Type I or II HAE, serum samples were taken one to three times a year and clinical status of the patients was registered. Serum samples were tested for total activity of the classical pathway, C1q, C3, C4 and C1-inhibitor (C1-INH) concentration and activity levels, as well as the presence of IgG, IgA and IgM type anti-C1-inhibitor antibodies (C1-INH-Ab). Fifty-four healthy age and gender matched persons served as control. Significant differences between the patients and controls in the occurrence of elevated (2S.D. higher than mean of control) C1-INH-Abs titers was found only in the case of IgM type C1-INH-Abs. Elevated (>4.22AU/ml) IgM C1-INH-Abs levels were found in 31 and 4% of the patients and controls, respectively (p<0.001). Surprisingly, high titer IgM C1-INH-Abs were present with equal frequency in the 41 HAE patients ever treated with C1-INH concentrate and in the 54 C1-INH treatment naïve patients. In the latter group, strong positive correlation between the levels of the IgM C1-INH-Abs and the most severe disease (score 1) (p=0.0021) and the yearly attack rate (p=0.0173) were obtained. In addition, the levels of the IgM C1-INH-Abs exhibited strong negative correlation to the C1-inhibitor concentration and functional activity, total classical complement pathway activity, and a positive correlation to total IgM concentration. Taken together, these data indicate that IgM type C1-INH-Abs are present with highly elevated frequency in HAE patients irrespectively of the previous treatment with C1-INH concentrate. Most probable production of these autoantibodies is the consequence of the activation of complement and other plasma enzyme systems during HAE attacks. Determination of IgM C1

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

    Directory of Open Access Journals (Sweden)

    Eray Atalay

    2015-11-01

    Full Text Available 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.

  10. 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

    2015-11-01

    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. PMID:26725563

  11. Canadian 2003 International Consensus Algorithm For the Diagnosis, Therapy, and Management of Hereditary Angioedema.

    Science.gov (United States)

    Bowen, Tom; Cicardi, Marco; Farkas, Henriette; Bork, Konrad; Kreuz, Wolfhart; Zingale, Lorenza; Varga, Lilian; Martinez-Saguer, Inmaculada; Aygören-Pürsün, Emel; Binkley, Karen; Zuraw, Bruce; Davis, Alvin; Hebert, Jacques; Ritchie, Bruce; Burnham, Jeanne; Castaldo, Anthony; Menendez, Alejandra; Nagy, Istvan; Harmat, George; Bucher, Christoph; Lacuesta, Gina; Issekutz, Andrew; Warrington, Richard; Yang, William; Dean, John; Kanani, Amin; Stark, Donald; McCusker, Christine; Wagner, Eric; Rivard, Georges-Etienne; Leith, Eric; Tsai, Ellie; MacSween, Michael; Lyanga, John; Serushago, Bazir; Leznoff, Art; Waserman, Susan; de Serres, Jean

    2004-09-01

    C1 inhibitor deficiency (hereditary angioedema [HAE]) is a rare disorder for which there is a lack of consensus concerning diagnosis, therapy, and management, particularly in Canada. European initiatives have driven the approach to managing HAE with 3 C1-INH Deficiency Workshops held every 2 years in Hungary starting in 1999, with the third Workshop having recently been held in May 2003. The European Contact Board has established a European HAE Registry that will hopefully advance our knowledge of this disorder. The Canadian Hereditary Angioedema Society/Société d'Angioédème Héréditaire du Canada organized a Canadian International Consensus Conference held in Toronto, Ontario, Canada, on October 24 to 26, 2003, to foster consensus between major European and North American HAE treatment centers. Papers were presented by investigators from Europe and North America, and this consensus algorithm approach was discussed. There is a paucity of double-blind placebo-controlled trials in the treatment of HAE, making levels of evidence to support the algorithm less than optimal. Enclosed is the consensus algorithm approach recommended for the diagnosis, therapy, and management of HAE and agreed to by the authors of this article. This document is only a consensus algorithm approach and requires validation. As such, participants agreed to make this a living 2003 algorithm (ie, a work in progress) and agreed to review its content at future international HAE meetings. The consensus, however, has strength in that it was arrived at by the meeting of patient-care providers along with patient group representatives and individual patients reviewing information available to date and reaching agreement on how to approach the diagnosis, therapy, and management of HAE circa 2003. Hopefully evidence to support approaches to the management of HAE will approach the level of meta-analysis of randomized controlled trials in the near future. PMID:15356569

  12. Recurrent angioedema and urticaria.

    OpenAIRE

    Bishop, P C; Wisnieski, J J; Christensen, J

    1993-01-01

    The case reported here illustrates the life-threatening aspects of angioedema and the need to thoroughly investigate the possible causes of this clinical finding. As discussed, the causes of angioedema are numerous. Commonly implicated in drug-induced angioedema are antihypertensive ACE inhibitor drugs, as was originally thought with this patient. Because of her skin lesions and macrocytic anemia, further studies were done. These studies led to a diagnosis of hypocomplementemic urticarial vas...

  13. Angioedema Associated with Haloperidol

    OpenAIRE

    AlMadhyan, Abdulmajeed Brahim

    2015-01-01

    Haloperidol is one of the antipsychotic medications which are widely used in the emergency department and its association with angioedema which is very rarely reported in scientific literature. One of the serious situations in the emergency department is angioedema and it bends airway obstruction which is need early attention and treatment. A case was reported which was observed at a governmental hospital in Riyadh. An adult female developed angioedema after single dose of Haloperidol intramu...

  14. Hereditary angioedema: Validation of the end point time to onset of relief by correlation with symptom intensity.

    Science.gov (United States)

    Bernstein, Jonathan A; Ritchie, Bruce; Levy, Robyn J; Wasserman, Richard L; Bewtra, Againdra K; Hurewitz, David S; Obtułowicz, Krystyna; Reshef, Avner; Moldovan, Dumitru; Shirov, Todor; Grivcheva-Panovska, Vesna; Kiessling, Peter C; Keinecke, Heinz-Otto; Craig, Timothy J

    2011-01-01

    Time to onset of symptom relief in hereditary angioedema (HAE) is a common primary end point in clinical studies but it has never been validated by correlation with the course of HAE symptoms. This study was designed as a retrospective validation of the primary end point for a placebo-controlled phase II/III study in patients with HAE. Ninety-eight abdominal attacks were treated with 10 or 20 U/kg of a highly purified C1 esterase inhibitor (C1-INH) concentrate or placebo. The primary end point was the time to onset of symptom relief, as determined by the patients. Patients assessed the intensity of the symptoms of pain, nausea, vomiting, cramps, and diarrhea over time. By Spearman rank correlation, the primary end point was compared with the time to first reduction of (1) any symptom intensity, (2) the sum of symptom intensity scores, and (3) the intensity of the last symptom present at baseline. The C1-INH, 20 U/kg, and placebo groups were compared by one-sided two-sample Wilcoxon tests. The time to first reduction in intensity of the last symptom present at baseline had the highest correlation with the primary end point (r = 0.77). The time to onset of symptom relief and the time to the first reduction in intensity of the last symptom were significantly shorter for the C1-INH, 20 U/kg, group compared with placebo (p = 0.009 and p = 0.0036, respectively). The association with the intensity of single symptoms confirmed that the time to onset of symptom relief is an appropriate end point for assessing the efficacy of C1-INH therapy. PMID:21262096

  15. Recurrent angioedema and urticaria.

    Science.gov (United States)

    Bishop, P C; Wisnieski, J J; Christensen, J

    1993-11-01

    The case reported here illustrates the life-threatening aspects of angioedema and the need to thoroughly investigate the possible causes of this clinical finding. As discussed, the causes of angioedema are numerous. Commonly implicated in drug-induced angioedema are antihypertensive ACE inhibitor drugs, as was originally thought with this patient. Because of her skin lesions and macrocytic anemia, further studies were done. These studies led to a diagnosis of hypocomplementemic urticarial vasculitis syndrome, an uncommon to rare form of acquired angioedema, urticarial vasculitis, arthritis, and obstructive airway disease associated with the production of autoantibodies to C1q. It is an autoimmune disorder related to but separate from SLE. PMID:8279170

  16. A Rare Cause of Abdominal Pain in Children: Hereditary Angioedema

    Directory of Open Access Journals (Sweden)

    Deniz Özçeker

    2015-03-01

    Full Text Available Hereditary angioedema (HA is a rare, autosomal-dominant genetic disorder presenting with recurrent attacks of angioedema. The most commonly involved organs include the extremites, face, neck, upper respiratory tract, genital region and the gastrointestinal tract. Edema of the intestinal mucosa can cause temporary obstruction and severe abdominal pain that can be confused with acute abdomen. Pediatricians and emergency physicians should keep in mind this rare disease in the differential diagnosis of severe abdominal pain.

  17. 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.

  18. [A case report of hereditary angioedema and studies on the serum components of complement, C1-inactivator and proteinase inhibitors during edema attack].

    Science.gov (United States)

    Mikami, A; Kohno, M

    1987-05-01

    Sixteen years old girl was admitted because of for the past ten years' frequent edema attack and abdominal pain. Laboratory examination revealed hypocomplementemia, marked depletion of the fourth component of complement and low level of C1-inactivator. Familial studies revealed that her mother was also hypocomplementemic and in low level of C1-inactivator. Serial studies performed on the alterlation of components of complement, C1-inactivator, alpha 1-antitrypsin, antithrombin III, and alpha 2-macroglobulin during edema attack. The fourth component of complement and C1-inactivator were markedly depleted in remission and attack. Remarkable depletion was found in antithrombin III and esterase inhibition activity of C1-inactivator during attack. In contrast, alpha 1-antitrypsin and alpha 2-macroglobulin did not change. The present study may explain that Hageman factor fragments, activated by C1s, promotes kinin generation via kalikrein activation. And the condition that complete functional deficiency of C1-inactivator was main role in this circuit. Fibrynolysis and late components of complement was less influence on edema attack. PMID:3610041

  19. 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.

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

    Science.gov (United States)

    Miranda, Amanda Rodrigues; Ue, Ana Paula Fusel de; Sabbag, Dominique Vilarinho; Furlani, Wellington de Jesus; Souza, Patrícia Karla de; 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 current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms. A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented. PMID:24068129

  1. Epidemiology of Non-hereditary Angioedema

    DEFF Research Database (Denmark)

    Madsen, Flemming; Attermann, Jørn; Linneberg, Allan

    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...... abdominal area, 17% had diarrhoea, 11% had vomiting and 6% fainted during attacks. Non-hereditary angioedema has high lifetime prevalence and becomes chronic in approximately 50% of affected patients. Symptoms in the larynx and throat, as well as non-specific symptoms, such as dizziness and abdominal pain......) reported a lifetime prevalence of 7.4% for angioedema. In both groups symptoms were most frequent in the lips, head, neck, eyes and tongue. In the C1 INH test normal group angioedema was still active at the time of the study in 53% of the patients, and 36% reported symptoms in the throat, 23% in the...

  2. Angioedema from instant coffee

    OpenAIRE

    Larkin, Kelly J.; Dvoretzky, Toban; Solomos, Nicholas J.

    2012-01-01

    Acute allergic angioedema is an abrupt-onset, unpredictable inflammatory reaction of the skin and mucous membranes. Without treatment, the condition may resolve within hours; however, when swallowing or breathing is affected, emergent medical attention is required. We report an atypical presentation of this condition, with a unique dietary cause. A 50-year-old man with no relevant medical history emergently presented with acute angioedema of the lower lip, without urticaria. The inflammation ...

  3. Genetic analysis of hereditary angioedema in a Brazilian family by targeted next generation sequencing.

    Science.gov (United States)

    Veronez, Camila Lopes; da Silva, Elton Dias; Lima Teixeira, Patrícia Varela; Cagini, Nathália; Constantino-Silva, Rosemeire Navickas; Grumach, Anete Sevciovic; Mansour, Eli; Velloso, Lício A; Pesquero, João Bosco

    2016-04-01

    Hereditary angioedema (HAE) is accompanied by an overproduction of bradykinin (BK) as the primary mediator of swelling. Although many proteins may be involved in regulating the wide spectrum of HAE symptoms, most studies have only focused on C1-INH and FXII. For the first time, a next generation sequencing (NGS) method was applied to develop a robust, time- and cost-effective diagnostic and research tool to analyze selected genes related to HAE. The entire coding region and the exon-intron boundaries of 15 genes from 23 subjects of a Brazilian family, nine of whom were symptomatic, were analyzed by NGS. One new mutation found uniquely in the nine symptomatic patients, p.Ala457Pro in the SERPING1 gene, was estimated as likely to be pathogenic (PolyPhen-2 software analysis) and is the main candidate to be responsible for HAE in these patients. Alterations identified in a few asymptomatic individuals but also found in almost all symptomatic patients, such as p.Ile197Met (HMWK), p.Glu298Asp (NOS3) and p.Gly354Glu (B2R), may also be involved in modulating patient-specific symptoms. This NGS gene panel has proven to be a valuable tool for a quick and accurate molecular diagnosis of HAE and efficient to indicate modulators of HAE symptoms. PMID:26751894

  4. 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.

  5. 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

  6. New treatments addressing the pathophysiology of hereditary angioedema.

    Science.gov (United States)

    Davis, Alvin E

    2008-01-01

    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 attacks; the nano-filtered C1

  7. Hereditary angio-edema involving the gastrointestinal tract: CT findings

    International Nuclear Information System (INIS)

    We report a case of hereditary angio-edema in a young man presenting with recurrent abdominal pain for many years. The diagnosis was suspected on the basis of abdominal CT performed during an abdominal attack and was then confirmed by the measurement of serum concentration of C1 esterase inhibitor (C1-INH). To our knowledge, this is the first case reported of the hereditary form of angio-edema with isolated abdominal pain and in which the diagnosis was suggested by abdominal CT findings. (orig.)

  8. [Hereditary angioedema in childhood. Diagnosis and therapeutic challenges].

    Science.gov (United States)

    Pagnier, Anne

    2015-01-01

    Hereditary angioedema is a rare disease. In case of laryngeal edema or chronic abdominal pains, diagnosis is difficult in childhood because numerous differential diagnoses possibilities are to be considered. The diagnosis of hereditary angioedema with normal C1Inh (type III) is also a challenge because it is based only on clinical features. Important school absenteeism can be due to recurrent abdominal attacks. Early diagnosis, specific management, and therapeutic education are necessary for improvement of quality of life. Actually, subcutaneous treatment is not yet available for children. Studies are going on. In the meantime, C1Inh concentrate intravenous administration must be available for children quickly and safely. PMID:25511651

  9. [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 (< 24 or 48h) and disappear without after-effects. They are considered "deep urticaria" and wheals "superficial urticaria". When AE or wheals last more than 6 weeks (with or without free intermission), it is called chronic urticaria. 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. PMID:25539680

  10. Hereditary angioedema: beyond international consensus - circa December 2010 - The Canadian Society of Allergy and Clinical Immunology Dr. David McCourtie Lecture

    Directory of Open Access Journals (Sweden)

    Bowen Tom

    2011-02-01

    Full Text Available Abstract Background The 2010 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema was published earlier this year in this Journal (Bowen et al. Allergy, Asthma & Clinical Immunology 2010, 6:24 - http://www.aacijournal.com/content/6/1/24. Since that publication, there have been multiple phase III clinical trials published on either prophylaxis or therapy of hereditary angioedema and some of these products have changed approval status in various countries. This manuscript was prepared to review and update the management of hereditary angioedema. Objective To review approaches for the diagnosis and management of hereditary angioedema (HAE circa December 2010 and present thoughts on moving from HAE management from international evidence-based consensus to facilitate more local health unit considerations balancing costs, efficacies of treatments, and risk benefits. Thoughts will reflect Canadian and international experiences. Methods PubMed searches including hereditary angioedema and diagnosis, therapy, management and consensus were reviewed as well as press releases from various pharmaceutical companies to early December 2010. Results The 2010 International Consensus Algorithms for the Diagnosis, Therapy and Management of Hereditary Angioedema is reviewed in light of the newly published phase III Clinical trials for prevention and therapy of HAE. Management approaches and models are discussed. Conclusions Consensus approach and double-blind placebo controlled trials are only interim guides to a complex disorder such as HAE and should be replaced as soon as possible with large phase IV clinical trials, meta analyses, data base registry validation of approaches including quality of life and cost benefit analyses, safety, and head-to-head clinical trials investigating superiority or non-inferiority comparisons of available approaches. Since not all therapeutic products are available in all jurisdictions

  11. 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.

  12. Social costs of icatibant self-administration vs. health professional-administration in the treatment of hereditary angioedema in Spain

    OpenAIRE

    Blasco, Antonio J.; Lázaro, Pablo; Caballero, Teresa; Guilarte, Mar

    2013-01-01

    Background: Icatibant is the only subcutaneous treatment for acute Type I and Type II hereditary angioedema with C1-esterase inhibitor deficiency (HAE-C1-INH) licensed for self-administration in Europe. Aim: To compare the economic impact of two icatibant administration strategies: health professional-administration only (strategy 1) versus including the patient self-administration option (strategy 2). Methods:Economic evaluation model based on the building of a decision tree. Both strategies...

  13. Hereditary angioedema: Not an allergy

    Directory of Open Access Journals (Sweden)

    Sanjay Bhivgade

    2012-01-01

    Full Text Available Hereditary angioedema is a genetic disorder due to a deficiency or malfunction of C1 esterase inhibitor. We herein describe a case of 25-year-old male who presented with swelling over face since one day. There was history of similar episodes since two years with gradual subsidence of swelling without any treatment. Investigations revealed grossly reduced complement C4 and C1 esterase inhibitor level. Patient was diagnosed to have hereditary angioedema type 1 and started on stanozolol 2 mg three times a day with no recurrence in one year of follow-up. Hereditary angioedema resembles angioedema of an allergic reaction. However, the cause is different.

  14. Prophylaxe beim hereditären Angioödem (HAE) mit C1-Inhibitormangel.

    Science.gov (United States)

    Greve, Jens; Strassen, Ulrich; Gorczyza, Marina; Dominas, Nina; Frahm, Uta-Marie; Mühlberg, Heike; Wiednig, Michaela; Zampeli, Vasiliki; Magerl, Markus

    2016-03-01

    Das hereditäre Angioödem (engl.: hereditary angioedema, HAE) ist eine seltene angeborene Erkrankung, die durch wiederkehrende Episoden subkutaner oder submuköser Ödeme charakterisiert ist. Kehlkopf-Manifestationen können lebensbedrohlich sein. In den meisten Fällen kann die Erkrankung mit einem On-Demand-Ansatz angemessen behandelt werden - in einigen Fällen ist jedoch eine Kurz- oder Langzeitprophylaxe angebracht. Attenuierte Androgene waren einmal das Standardmedikament; sie werden jedoch mit erheblichen Nebenwirkungen in Verbindung gebracht und sind in den deutschsprachigen Ländern der EU nicht mehr kommerziell erhältlich. Zurzeit werden sie von wirksameren und besser verträglichen Therapien wie C1-Esterase-Inhibitoren, dem Kallikrein-Inhibitor Ecallantid und dem B2-Rezeptorantagonisten Icatibant verdrängt, welche kürzlich auf dem Markt zugelassen wurden. Diese neuen Medikamente hatten einen erheblichen Einfluss, insbesondere auf die Indikationsstellung und das Vorgehen bei einer Langzeitprophylaxe. Nach den neuesten internationalen Konsenspapieren und unserer eigenen Erfahrung sind selbstverabreichte C1-Inhibitoren nun die erste Option bei der Langzeitprophylaxe. Die Entscheidung für eine Prophylaxe sollte nicht länger auf der Grundlage einzelner Parameter wie der Häufigkeit der Anfälle getroffen werden, sondern auf einer adäquaten allgemeinen Krankheitskontrolle, einschließlich der Lebensqualität. Zurzeit werden weitere Medikamente entwickelt, welche zu weiteren Veränderungen bei den Behandlungsalgorithmen des HAE führen könnten. PMID:26972190

  15. 'Epinephrine-resistant' angioedema.

    Science.gov (United States)

    Ange, Nikhita; Rabbolini, David J; Pidcock, Michael; Randall, Katrina L

    2016-01-01

    A man in his 60s was brought to the emergency department, with airway compromise and dysarthria due to a grossly enlarged tongue. As he was on a current course of antibiotics, he was treated for a likely antibiotic-associated allergic reaction. However, as he failed to improve with intramuscular and nebulised epinephrine, another cause of his symptoms was sought. Further discussion revealed a history of chronic lymphocytic leukaemia (CLL), which had recently relapsed. Investigations were ordered to confirm that the symptoms were due to acquired angioedema, and the patient was managed for this diagnosis based on the presence of an undetectable C4 level. This diagnosis was later confirmed when the results of specialist tests became available. The patient was treated for his relapsed CLL with good effect, and has had no further episodes of angioedema and an improvement in the level of his C1 esterase protein level and function. PMID:26823364

  16. HAE international home therapy consensus document

    DEFF Research Database (Denmark)

    Longhurst, Hilary J; Farkas, Henriette; Craig, Timothy; Aygoren-Pursun, Emel; Bethune, Claire; Bjorkander, Janne; Bork, Konrad; Bouillet, Laurence; Boysen, Henrik; Bygum, Anette; Caballero, Teresa; Cicardi, Marco; Dempster, John; Gompels, Mark; Gooi, Jimmy; Grigoriadou, Sofia; Huffer, Ursula; Kreuz, Wolfhart; Levi, Marcel M; Long, Janet; Martinez-Saguer, Inmaculada; Raguet, Michel; Reshef, Avner; Bowen, Tom; Zuraw, Bruce

    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...

  17. Acebrophylline-induced angioedema

    Directory of Open Access Journals (Sweden)

    Sanitha Kuriachan

    2015-01-01

    Full Text Available A 53-year-old woman visited her physician complaining of acute breathlessness and productive cough. Her medications included budesonide and formoterol for asthma, fixed-dose combination aspirin 150 mg + clopidogrel 75 mg + atorvastatin 20 mg for ischemic heart disease. History revealed that she had allergic rhinitis and was hypersensitive to penicillins. The patient was prescribed acebrophylline (ABP. Six hours after ABP therapy she presented with generalized urticarial lesions, swelling of hands, feet, lips and face, suggestive of angioedema. ABP was stopped immediately, and the patient was treated symptomatically. This case was categorized as probable as per standard causality assessment scale.

  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. PMID:26068904

  19. Hereditary angioedema in a Jordanian family with a novel missense mutation in the C1-inhibitor N-terminal domain.

    Science.gov (United States)

    Jaradat, Saied A; Caccia, Sonia; Rawashdeh, Rifaat; Melhem, Motasem; Al-Hawamdeh, Ali; Carzaniga, Thomas; Haddad, Hazem

    2016-03-01

    Hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE) is an autosomal dominant disease caused by mutations in the SERPING1 gene. A Jordanian family, including 14 individuals with C1-INH-HAE clinical symptoms, was studied. In the propositus and his parents, SERPING1 had four mutations leading to amino acid substitutions. Two are known polymorphic variants (c.167T>C; p.Val34Ala and c.1438G>A; p.Val458Met), the others are newly described. One (c.203C>T; p.Thr46Ile) is located in the N-terminal domain of the C1-inhibitor protein and segregates with angioedema symptoms in the family. The other (c.800C>T; p.Ala245Val) belongs to the serpin domain, and derives from the unaffected father. DNA from additional 24 family members were screened for c.203C>T mutation in the target gene. All individuals heterozygous for the c.203C>T mutation had antigenic and functional plasma levels of C1-inhibitor below 50% of normal, confirming the diagnosis of type I C1-INH-HAE. Angioedema symptoms were present in 14 of 16 subjects carrier for the c.203T allele. Among these subjects, those carrying the c.800T variation had more severe and frequent symptoms than subjects without this mutation. This family-based study provides the first evidence that multiple amino acid substitutions in SERPING1 could influence C1-INH-HAE phenotype. PMID:26895475

  20. An overview of novel therapies for acute hereditary angioedema.

    Science.gov (United States)

    Firszt, Rafael; Frank, Michael M

    2010-12-01

    Hereditary angioedema is an episodic swelling disorder with autosomal dominant inheritance. Attacks are characterized by nonpitting edema of external or mucosal body surfaces. Patients often present with swelling of the extremities, abdominal pain, and swelling of the mouth and throat, which can at times lead to asphyxiation. The disease is caused by a mutation in the gene encoding the complement C1-inhibitor protein, which leads to unregulated production of bradykinin. Long-term therapy has depended on the use of attenuated androgens or plasmin inhibitors but in the US there was, until recently, no specific therapy for acute attacks. As well, many patients with hereditary angioedema in the US were either not adequately controlled on previously available therapies or required doses of medications that exposed them to the risk of serious adverse effects. Five companies have completed or are currently conducting phase III clinical trials in the development of specific therapies to terminate acute attacks or to be used as prophylaxis. These products are based on either replacement therapy with purified plasma-derived or recombinant C1-inhibitor, or inhibition of the kinin-generating pathways with a recombinant plasma kallikrein inhibitor or bradykinin type 2 receptor antagonist. Published studies thus far suggest that all of these products are likely to be effective. These new therapies will likely lead to a totally new approach in treating hereditary angioedema. PMID:20866113

  1. Genetics Home Reference: hereditary angioedema

    Science.gov (United States)

    ... Cicardi M. C1-inhibitor deficiency and angioedema: molecular mechanisms and clinical progress. Trends Mol Med. 2009 Feb; ... with a qualified healthcare professional . About Genetics Home Reference Site Map Contact Us Selection Criteria for Links ...

  2. Complements Are Not Always a Good Thing: Novel Therapies for Angioedema.

    Science.gov (United States)

    Bailey, Abby Mynatt; Reed, Brittany S; Weant, Kyle A; Justice, Stephanie Baker

    2016-01-01

    Hereditary angioedema attacks are rare, but emergency care providers must be aware of the clinical presentation and treatment of these patients because the emergency department remains the most common setting where these patients seek treatment. If providers are not aware of the past medical history of these patients, they are likely to receive standard therapies for respiratory distress and anaphylaxis including antihistamines, corticosteroids, and epinephrine. However, these medications may not work in these patients, given the pathophysiology of their underlying disease. Since 2009, several new therapies have been approved for the treatment of acute hereditary angioedema attacks. This article discusses pathophysiology, clinical presentation, and use of novel therapies for the management of angioedema. PMID:27139131

  3. A Family with Hereditary Angioedema Having Been Followed as Familial Mediterranean Fever

    Directory of Open Access Journals (Sweden)

    Gülben Sarıcı

    2009-03-01

    Full Text Available Hereditary angioedema is a rare autosomal dominant disorder resulting from the congenital deficiency of functional C1 esterase inhibitor protein. Patients with hereditary angioedema are clinically characterized by recurrent episodes of swelling of the extremities, face, trunk, airways and abdominal viscera. Attacks may occur either spontaneously or following stress or trauma. The disease is usually associated with attacks of abdominal pain. So, patients may apply for this complaint to other clinics rather than dermatology, and may be misdiagnosed and followed for a long time. Therefore hereditary angioedema should be thought in differential diagnosis of patients suffering from abdominal pain. Here in this writing, we describe a family with hereditary angioedema who has been followed as Familial Mediterranean Fever for a long time. The family members complained from swellings which have been occuring in various regions of the body and disappearing spontaneously, and complained from severe abdominal pain, since childhood. These patients have been followed and tried to be treated with the misdiagnosis of Familial Mediterranean Fever for many years. These patients were diagnosed as hereditary angioedema in our clinic, and benefited from danazol treatment

  4. Successful C1 inhibitor short-term prophylaxis during redo mitral valve replacement in a patient with hereditary angioedema

    Directory of Open Access Journals (Sweden)

    Coleman Suzanne

    2010-10-01

    Full Text Available Abstract Hereditary angioedema is characterized by sudden episodes of nonpitting edema that cause discomfort and pain. Typically the extremities, genitalia, trunk, gastrointestinal tract, face, and larynx are affected by attacks of swelling. Laryngeal swelling carries significant risk for asphyxiation. The disease results from mutations in the C1 esterase inhibitor gene that cause C1 esterase inhibitor deficiency. Attacks of hereditary angioedema result from contact, complement, and fibrinolytic plasma cascade activation, where C1 esterase inhibitor irreversibly binds substrates. Patients with hereditary angioedema cannot replenish C1 esterase inhibitor levels on pace with its binding. When C1 esterase inhibitor is depleted in these patients, vasoactive plasma cascade products cause swelling attacks. Trauma is a known trigger for hereditary angioedema attacks, and patients have been denied surgical procedures because of this risk. However, uncomplicated surgeries have been reported. Appropriate prophylaxis can reduce peri-operative morbidity in these patients, despite proteolytic cascade and complement activation during surgical trauma. We report a case of successful short-term prophylaxis with C1 esterase inhibitor in a 51-year-old man with hereditary angioedema who underwent redo mitral valve reconstructive surgery.

  5. The establishment and utility of Sweha-Reg: a Swedish population-based registry to understand hereditary angioedema

    Directory of Open Access Journals (Sweden)

    Werner Sonja

    2007-11-01

    Full Text Available Abstract Background The importance of acquiring comprehensive epidemiological and clinical data on hereditary angioedema has increasingly caught the attention of physicians and scientists around the world. The development of networks and creation of comprehensive policies to improve care of people suffering from rare diseases, such as hereditary angioedema, is a stated top priority of the European Union. Hereditary angioedema is a rare disease, that it may be life-threatening. Although the exact prevalence is unknown, current estimates suggest that it is 1/10,000–1/150,000 individuals. The low prevalence requires combined efforts to gain accurate epidemiological data on the disease and so give us tools to reduce morbidity and mortality, and improve quality of life of sufferers. Methods Sweha-Reg is a population-based registry of hereditary angioedema in Sweden with the objectives of providing epidemiological data, and so creates a framework for the study of this disease. The registry contains individual-based data on diagnoses, treatments and outcomes. Conclusion The present manuscript seeks to raise awareness of the existence of Sweha-Reg to stimulate the international collaboration of registries. A synthesis of data from similar registries across several countries is required to approach an inclusive course understanding of HAE.

  6. Transcriptional profiling of the Arabidopsis abscission mutant hae hsl2 by RNA-Seq

    Directory of Open Access Journals (Sweden)

    Niederhuth Chad E

    2013-01-01

    Full Text Available Abstract Background Abscission is a mechanism by which plants shed entire organs in response to both developmental and environmental signals. Arabidopsis thaliana, in which only the floral organs abscise, has been used extensively to study the genetic, molecular and cellular processes controlling abscission. Abscission in Arabidopsis requires two genes that encode functionally redundant receptor-like protein kinases, HAESA (HAE and HAESA-LIKE 2 (HSL2. Double hae hsl2 mutant plants fail to abscise their floral organs at any stage of floral development and maturation. Results Using RNA-Seq, we compare the transcriptomes of wild-type and hae hsl2 stage 15 flowers, using the floral receptacle which is enriched for abscission zone cells. 2034 genes were differentially expressed with a False Discovery Rate adjusted p INFLORESCENCE DEFICIENT IN ABSCISSION (ida mutants shows that many of the same genes are co-regulated by IDA and HAE HSL2 and support the role of IDA in the HAE and HSL2 signaling pathway. Comparison to microarray data from stamen abscission zones show distinct patterns of expression of genes that are dependent on HAE HSL2 and reveal HAE HSL2- independent pathways. Conclusion HAE HSL2-dependent and HAE HSL2-independent changes in genes expression are required for abscission. HAE and HSL2 affect the expression of cell wall modifying and defense related genes necessary for abscission. The HAE HSL2-independent genes also appear to have roles in abscission and additionally are involved in processes such as hormonal signaling, senescence and callose deposition.

  7. Life-threatening angioedema of the tongue: the detection of the RNA of B henselae in the saliva of a male patient and his dog as well as of the DNA of three Bartonella species in the blood of the patient.

    Science.gov (United States)

    Lösch, Barbara; Wank, Rudolf

    2014-01-01

    Non-hereditary angioedema is a common disease with a prevalence between 5% and 19% and approximately half of the patients experience a swelling of the tongue. We report a case of a 49-year-old Caucasian man with a gross life-threatening angioedema of the tongue, whose attacks occurred every 4 weeks. The most frequent causes of angioedema were excluded. We detected DNA and RNA from Bartonella henselae in the blood and saliva of the patient and in the saliva of the patient's hunting dog. Treatment with azithromycin plus minocycline cleared the blood and saliva of RNA and DNA of Bartonella species, and the patient has been free from angioedema for 1 year. None of the therapy modalities used to treat the hereditary form or ACE or allergy-induced angioedema affect the detrimental course caused by Bartonella species. We therefore suggest that a molecular Bartonella test be included in the analysis of angioedema. PMID:24654245

  8. 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

    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...... patients, genetic counseling, infertility, abortion, lactation, menopause treatment, and endometrial cancer. CONCLUSIONS: A consensus for the management of female patients with HAE-C1-INH is presented....... devices, and progestins can be used. Pregnancy: Attenuated androgens are contraindicated and should be discontinued before attempting conception. Plasma-derived human C1 inhibitor concentrate (pdhC1INH) is preferred for acute treatment, short-term prophylaxis, or long-term prophylaxis. Tranexamic acid...

  9. F12-46C/T polymorphism as modifier of the clinical phenotype of hereditary angioedema.

    Science.gov (United States)

    Speletas, M; Szilágyi, Á; Csuka, D; Koutsostathis, N; Psarros, F; Moldovan, D; Magerl, M; Kompoti, M; Varga, L; Maurer, M; Farkas, H; Germenis, A E

    2015-12-01

    The factors influencing the heterogeneous clinical manifestation of hereditary angioedema due to C1-INH deficiency (C1-INH-HAE) represent one of the oldest unsolved problems of the disease. Considering that factor XII (FXII) levels may affect bradykinin production, we investigated the contribution of the functional promoter polymorphism F12-46C/T in disease phenotype. We studied 258 C1-INH-HAE patients from 113 European families, and we explored possible associations of F12-46C/T with clinical features and the SERPING1 mutational status. Given that our cohort consisted of related subjects, we implemented generalized estimating equations (GEEs), an extension of the generalized linear model accounting for the within-subject correlation. F12-46C/T carriers exhibited a significantly delayed disease onset (P < 0.001) and did not need long-term treatment (P = 0.02). In a GEE linear regression model, the presence of F12-46C/T was significantly associated with a 7-year delay in disease onset (P < 0.0001) regardless of SERPING1 mutational status. It is concluded that F12-46C/T carriage acts as an independent modifier of C1-INH-HAE severity. PMID:26248961

  10. Angioedema-Urticaria Due to Acitretin.

    Science.gov (United States)

    Solak, Berna; Metin, Nurcan; Erdem, Mustafa Teoman

    2016-01-01

    Acitretin is a synthetic oral retinoid that has been used for a number of dermatological diseases. Several side effects of acitretin have been reported such as teratogenicity, cheilitis, xerosis, dyslipidemia, and photosensitivity. Many drugs, mainly antibiotics and nonsteroidal anti-inflammatory drugs, can cause angioedema-urticaria. We present the case of angioedema-urticaria due to acitretin, confirmed by oral provocation test, in a 61-year-old man with psoriasis. To the best of our knowledge, only 1 case of angioedema due to oral acitretin has been reported in the literature so far. We report this case to draw attention that acitretin may cause angioedema-urticaria and to inform patients about this risk besides other side effects due to acitretin. PMID:26820109

  11. Acquired angioedema secondary to hormone replacement therapy

    Directory of Open Access Journals (Sweden)

    Malani Kumar

    2005-10-01

    Full Text Available Angioedema is a potentially life threatening condition and may be either inherited or acquired. The latter is rare with only a handful of cases reported in the world literature. Presenting complaints are often vague. Those most commonly described include swelling in the subcutaneous and submucosal tissues. Patients presenting with laryngeal edema have high mortality, and high clinical suspicion is necessary to avoid instrumentation, which can precipitate laryngeal spasm. We present a review of reported cases of hormonally induced hereditary angioedema, along with a report of a patient with acquired angioedema secondary to hormone replacement therapy. To the best of our knowledge, this case probably represents the first reported case of acquired angioedema secondary to hormone replacement therapy.

  12. Coexistence of hereditary angioedema and Turner's syndrome.

    OpenAIRE

    Fletcher, A; Weetman, A P

    1998-01-01

    A 34-year-old woman presented to the out-patient clinic with angioedema and type II hereditary angioedema was confirmed immunologically. She also volunteered she had never had a menstrual period and physical examination identified several features of Turner's syndrome. A mosaic karyotype with XY and XO was found on chromosomal analysis and gonadectomy was performed in view of the high risk of gonadoblastoma. After commencing oestrogen at physiological replacement doses, the patient experience...

  13. Cytokine-associated angioedema syndromes including episodic angioedema with eosinophilia (Gleich's Syndrome).

    Science.gov (United States)

    Banerji, Aleena; Weller, Peter F; Sheikh, Javed

    2006-11-01

    Angioedema can be associated with many disorders and the presentation can be variable. Subsets of the angioedema syndromes are thought to be cytokine mediated (Table 1). Of these, the best described are the episodic angioedema with eosinophilia syndrome (Gleich's syndrome) and non-episodic angioedema with eosinophilia, which share some common features, but appear to have differences in pathophysiology. NERDS (nodules, eosinophilia, rheumatism, dermatitis and swelling), Clarkson syndrome (idiopathic capillary leak syndrome), and angioedema associated with aldesleukin (human recombinant IL-2) and IFN-alpha have also been reported in the literature, and have been discussed in this review. There is still much to be learned about the pathophysiology, diagnosis, and treatment of patients with these disorders. Our hope is that this review will be of help to those readers who care for patients with these disorders, and will stimulate interest in further research into the pathophysiology of these conditions. PMID:17085290

  14. 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. PMID:26969870

  15. Clinical and Laboratory Characteristics That Differentiate Hereditary Angioedema in 72 Patients with Angioedema

    Directory of Open Access Journals (Sweden)

    Isao Ohsawa

    2014-01-01

    Conclusions: Early onset of AE, positive family history, recurrent AE in the extremities and GI tract, and suffocation are distinctive characteristics of HAE. A low serum level of C4 is a useful marker for making a differential diagnosis of HAE.

  16. Factor XII mutations, estrogen-dependent inherited angioedema, and related conditions

    Directory of Open Access Journals (Sweden)

    Binkley Karen E

    2010-07-01

    Full Text Available Abstract The clinical, biochemical and genetic features of the conditions known as estrogen-dependent inherited angioedema, estrogen-associated angioedema, hereditary angioedema with normal C-1 inhibitor, type III angioedema, or factor XII angioedema are reviewed. Discussion emphasizes pathogenesis, diagnosis, and management.

  17. Factor XII mutations, estrogen-dependent inherited angioedema, and related conditions.

    Science.gov (United States)

    Binkley, Karen E

    2010-01-01

    The clinical, biochemical and genetic features of the conditions known as estrogen-dependent inherited angioedema, estrogen-associated angioedema, hereditary angioedema with normal C-1 inhibitor, type III angioedema, or factor XII angioedema are reviewed. Discussion emphasizes pathogenesis, diagnosis, and management. PMID:20667119

  18. Treatment of type I and II hereditary angioedema with Rhucin, a recombinant human C1 inhibitor.

    Science.gov (United States)

    Varga, Lilian; Farkas, Henriette

    2008-11-01

    Hereditary and acquired angioedema are of outstanding clinical importance, as edematous attacks associated with these conditions can thrust afflicted patients into mortal danger. Currently, C1 inhibitor concentrate - a human blood product - is available as a replacement therapy. In view of the limited number of donors, as well as the risk of transmission of blood-borne infections, it is a reasonable expectation to develop a therapeutic alternative based on recombinant technology, which would eliminate all these shortcomings. Pharming (Leiden, The Netherlands) has developed Rhucin, a recombinant human C1 inhibitor, as a proprietary product, which is currently being evaluated in Phase III clinical trials. Ongoing studies conducted within the framework of the development program are almost complete and their interim findings are reassuring. This should facilitate successful regulatory approval in the near future, which is indispensable in order to make Rhucin available for patients with hereditary angioedema or other disorders amenable to C1 inhibitor replacement. PMID:20477114

  19. 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.

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

    NARCIS (Netherlands)

    Levi, M; Choi, G; Picavet, C; Hack, CE

    2006-01-01

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

  1. Differentiation of Neisseria gonorrhoeae from other Neisseria species by use of the restriction endonuclease HaeIII.

    OpenAIRE

    Torres, A. R. de; Li, M K; Ward, D C; Edberg, S C

    1984-01-01

    We used the restriction endonuclease HaeIII to differentiate Neisseria gonorrhoeae from other Neisseria species and Branhamella catarrhalis. A total of 16 clinical isolates and four American Type Culture Collection strains of N. gonorrhoeae were resistant to HaeIII digestion, whereas 17 isolates and four American Type Culture Collection strains from eight different bacterial species were susceptible. This resistance was not caused by an enzyme inhibitor. We propose that protection of the HaeI...

  2. Hereditary Angioedema Due to C1 Inhibitor Deficiency in Serbia: Two Novel Mutations and Evidence of Genotype-Phenotype Association

    Science.gov (United States)

    Andrejević, Slađana; Korošec, Peter; Šilar, Mira; Košnik, Mitja; Mijanović, Radovan; Bonači-Nikolić, Branka; Rijavec, Matija

    2015-01-01

    Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare autosomal dominant disease characterized by recurrent life-threatening oedemas and/or abdominal pain and caused by mutations affecting the C1 inhibitor gene, SERPING1. We sought to investigate the spectrum of SERPING1 mutations in Serbia and the possible genotype-phenotype association. C1-INH-HAE was diagnosed on the basis of clinical and laboratory criteria in 40 patients from 27 families; four were asymptomatic. Mutational analysis of the SERPING1 gene was performed by sequencing and multiplex ligation-dependent probe amplification. Disease-causing mutations in SERPING1 were identified in all patients. In C1-INH-HAE type I, we identified 19 different mutations, including 6 missense mutations, 6 nonsense mutations, 2 small deletions, 1 small insertion, 2 splicing defects and 2 large deletions. Two of the mutations (c.300C>T and c.1184_1185insTA) are reported here for the first time. All C1-INH-HAE type II patients from three families harboured the same substitution (c.1396C>T). Based on the type of mutation identified in the SERPING1 gene, patients were divided into two groups: group 1 (nonsense, frameshift, large deletions/insertions, splicing defect, and mutations at Arg444) or group 2 (missense, excluding mutations at Arg444). Significant differences were found in the clinical severity score (P = 0.005), prevalence of laryngeal (P = 0.040) and facial (P = 0.013) oedema, and long-term prophylaxis (P = 0.023) between the groups with different types of mutations. Because our population consisted of related subjects, differences in the severity score between mutation groups were further confirmed using the generalized estimating equation (P = 0.038). Our study identified 20 different disease-causing mutations, including two novel mutations, in all C1-INH-HAE patients, highlighting the heterogeneity of mutations in the SERPING1 gene. Furthermore, it appears that mutations with a clear effect

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

    DEFF Research Database (Denmark)

    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; Bygum, Anette

    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 an...... 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.......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...

  4. Neurofeedback in Hereditary Angioedema: A Single Case Study of Symptom Reduction.

    Science.gov (United States)

    Burns, Stephanie T

    2015-09-01

    Neurofeedback training was performed consisting of rewarding and encouraging 12-15 Hz brainwaves (SMR), while simultaneously discouraging 4-7 Hz brainwaves (theta) and 22-30 Hz brainwaves (high beta) in the right dorsal posterior quadrant of the brain (T4, P4) for 20 half-hour NFB sessions to determine the impact on cortisol levels, DHEA-S levels, scores on the Symptom Checklist-90-R (SCL-90-R), the quality of life inventory, and acute attack medication usage for a Hereditary Angioedema patient. PMID:25958076

  5. Angioedema: Clinical Presentations and Pharmacological Management.

    Science.gov (United States)

    Collins-Yoder, Angela Smith

    2016-01-01

    Angioedema (AE) is a unique clinical presentation of an unchecked release of bradykinin. The origin of this clinical presentation can be either genetic or acquired. The outcome within the patient is subcutaneous swelling of the lower layers of the epidermis. Symptoms are most often localized to the upper airway or the gastrointestinal tract. A typical course resolves in 5 to 7 days, but in some patients, the clinical manifestations exist up to 6 weeks. Hereditary AE is rare and genetically linked, and typically, the patient has episodes for many years before diagnosis. Episodes of acquired AE may be drug induced, triggered by a specific allergen, or idiopathic. Angioedema can elicit the need for critical care interventions, for advanced airway management, or unnecessary abdominal surgery. The treatment for these patients is evolving as new pharmacological agents are developed. This article addresses subtypes of AE, triggers, pharmacology, and information for interdisciplinary team planning of individualized case management. PMID:27258954

  6. Acute allergic angioedema of upper lip.

    Science.gov (United States)

    Mahendran, Kavitha; Padmini, Govindasway; Murugesan, Ramesh; Srikumar, Arthiseethalakshmi

    2016-01-01

    Mishaps can occur during dental procedures, some owing to inattention to detail and others are totally unpredictable. They usually include anaphylaxis or allergic reactions to materials used for restorative purposes or drugs such as local anesthetics. A patient reported to our department with moderate dental fluorosis, and the treatment was planned with indirect composite veneering. During the procedure while cementation acute allergic reaction occurred, the specific cause could not be identified after allergic testing. During the procedure while cementationacute allergic angioedema of upper lip. Anaphylaxis, urticaria, allergy, hereditary atopic eczema, cellulitis, cheilitis granulomatosa, and cheilitis glandularis. The patient was reassured and given prednisolone 10 mg and cetirizine 10 mg orally, once daily for 3 days after which the symptoms subsided. This paper will discuss the pathogenesis, classification, identification, and management of angioedema during dental procedures. PMID:27217646

  7. Acute allergic angioedema of upper lip

    Science.gov (United States)

    Mahendran, Kavitha; Padmini, Govindasway; Murugesan, Ramesh; Srikumar, Arthiseethalakshmi

    2016-01-01

    Mishaps can occur during dental procedures, some owing to inattention to detail and others are totally unpredictable. They usually include anaphylaxis or allergic reactions to materials used for restorative purposes or drugs such as local anesthetics. A patient reported to our department with moderate dental fluorosis, and the treatment was planned with indirect composite veneering. During the procedure while cementation acute allergic reaction occurred, the specific cause could not be identified after allergic testing. During the procedure while cementationacute allergic angioedema of upper lip. Anaphylaxis, urticaria, allergy, hereditary atopic eczema, cellulitis, cheilitis granulomatosa, and cheilitis glandularis. The patient was reassured and given prednisolone 10 mg and cetirizine 10 mg orally, once daily for 3 days after which the symptoms subsided. This paper will discuss the pathogenesis, classification, identification, and management of angioedema during dental procedures. PMID:27217646

  8. Acute allergic angioedema of upper lip

    Directory of Open Access Journals (Sweden)

    Kavitha Mahendran

    2016-01-01

    Full Text Available Mishaps can occur during dental procedures, some owing to inattention to detail and others are totally unpredictable. They usually include anaphylaxis or allergic reactions to materials used for restorative purposes or drugs such as local anesthetics. A patient reported to our department with moderate dental fluorosis, and the treatment was planned with indirect composite veneering. During the procedure while cementation acute allergic reaction occurred, the specific cause could not be identified after allergic testing. During the procedure while cementationacute allergic angioedema of upper lip. Anaphylaxis, urticaria, allergy, hereditary atopic eczema, cellulitis, cheilitis granulomatosa, and cheilitis glandularis. The patient was reassured and given prednisolone 10 mg and cetirizine 10 mg orally, once daily for 3 days after which the symptoms subsided. This paper will discuss the pathogenesis, classification, identification, and management of angioedema during dental procedures.

  9. 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.

  10. 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.

  11. 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.

  12. Analysis of characteristics associated with reinjection of icatibant

    DEFF Research Database (Denmark)

    Longhurst, Hilary J; Aberer, Werner; Bouillet, Laurence; Caballero, Teresa; Fabien, Vincent; Zanichelli, Andrea; Maurer, Marcus; Bygum, Anette

    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...

  13. 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

  14. Panic Attack or Heart Attack?

    Science.gov (United States)

    Panic Attack or Heart Attack? Diagnosing heart disease in women A Healthy Hearts Guide ® Heart disease in women is often mistaken for panic attack with shortness of breath, anxiety, palpitations and indigestion. ...

  15. 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.

  16. Angioedema Due to use of ACE-Inhibitor

    Directory of Open Access Journals (Sweden)

    Hulya Eyigor

    2014-03-01

    Full Text Available       Angioedema; which may be hereditary or non-hereditary, is defined as a sudden, severe, often in awkward, temporary swelling of skin, subcutaneous and mucous membranes of the face, tongue, lip, larynx, and gastrointestinal areas. Angiotensin Converting Enzyme (ACE inhibitor drugs are widely used in essential hypertension and congestive heart diseases and effective and safe drugs. Angioedema is quite rare due to the use of ACE inhibitors, the rate changes from 0.1 to 0.7% reported in the literature. The pathophysiology of angioedema induced by ACE inhibitors are not completely understood, this situation has been tought to be associated with an increased activity of bradykinin related vasodilatation, increased vascular permeability and interstitial edema. In this study, a case of 65-year-old male patient presented angioedema induced by lisinopril was presented and a very rare side effect of ACE inhibitor drugs was reviewed with the relevant literature.

  17. Visceral Angioedema Induced by Angiotensin Converting Enzyme Inhibitor: Case Report

    Directory of Open Access Journals (Sweden)

    Beatriz Frutuoso

    2016-05-01

    Conclusion: The diagnosis of intestinal angioedema induced by angiotensin converting enzyme inhibitor can be challenging and time consuming due to its rarity and nonspecific symptoms, which may lead to underdiagnosis of this entity.

  18. 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...... 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 the...

  19. Hereditary angioedema: quality of life in Brazilian patients

    OpenAIRE

    Gomide, Maria Abadia Consuelo M S; Eliana Toledo; Solange Oliveira Rodrigues Valle; Campos, Regis A.; Alfeu T. França; Nieves Prior Gomez; Heitor Franco Andrade Jr.; Teresa Caballero; Grumach, Anete S.

    2013-01-01

    OBJECTIVE: Hereditary angioedema is a serious medical condition caused by a rare autosomal dominant genetic disorder and it is associated with deficient production or dysfunction of the C1 esterase inhibitor. In most cases, affected patients experience unexpected and recurrent crises of subcutaneous, gastrointestinal and laryngeal edema. The unpredictability, intensity and other factors associated with the disease impact the quality of life of hereditary angioedema patients. We evaluated the ...

  20. Angioedema associated with Crohn's disease: Response to biologics

    Institute of Scientific and Technical Information of China (English)

    Flavio Habal; Vivian Huang

    2012-01-01

    A 46-year-old female patient with terminal ileum Crohn's disease and ankylosing spondylitis presented with recurrent angioedema and urticaria.Investigations ruled out hereditary angioedema,and environmental or food allergen triggers.She was diagnosed with chronic idiopathic urticaria with angioedema,and was treated with a trial of intravenous immunoglobulin immunotherapy,danazol,prednisone and hydroxyzine.Due to ongoing bowel and arthritic complaints,she was started on infliximab infusions and within 2 treatments,she had complete resolution of the angioedema and urticaria,as well as of the bowel and arthritic symptoms.Unfortunately she developed allergic reactions to the infliximab and was switched to another anti-tumor necrosis factor (TNF)-α agent,adalimumab.Since then,she has had no further angioedema or urticaria,and her Crohn's disease has been quiescent.This is the first known case report of chronic idiopathic urticaria with angioedema coexistent with Crohn's disease that was successfully treated with anti-TNF-α agents.

  1. 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

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

    OpenAIRE

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

    2016-01-01

    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 evaluat...

  3. Pathophysiology, clinical manifestation and management of angioedema - our experience

    Directory of Open Access Journals (Sweden)

    Aleksić Aleksandra

    2015-01-01

    Full Text Available Introduction. Angioedema is characterized by subcutaneous and/or submucosal swelling usually localized to the lips, eyelids, tongue, oral cavity, larynx and pharynx. Various types of angioedema, caused by different pathophysiologic mechanisms, can have the same or very similar clinical picture and require different diagnostic and therapeutic procedures. The immediate threat to life as a result of rapidly developed edema of the pharynx and larynx with airway obstruction requires endotracheal intubation or emergency tracheotomy. Standard therapy, which includes epinephrine, second-generation antihistamines and steroids, is not effective in the treatment of all types of angioedema. Objective. On the basis of the clinical presentation and course of angioedema, this retrospective study was aimed at contributing to a better understanding of the etiopathogenesis of the disease and at helping determine the most effective available treatment modalities. Methods. This retrospective study included patients treated under the diagnosis of angioedema of the upper aerodigestive tract between 2000 and 2012 in the Department of Otorhinolaryngology, Clinical Center of Banja Luka. Results. A total of 76 subjects were included in the study. The average age was 62.8 years. There were 40 (52.6% male and 36 (47.4% female patients. The largest number of patients (44.7% had type II angioedema. Almost half of the patients or 36 patients (47.4% were on treatment with an angiotensinconverting enzyme inhibitor (ACEi, but there was no statistically significant difference under the total number of patients (p=0.678. Conclusion. Better understanding of pathophysiologic mechanisms and the adoption of diagnostic protocols contributes to more effective treatment of angioedema.

  4. 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.

  5. [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. PMID:24931134

  6. 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 to...... asphyxia has increased. This is mainly due to pharmaceuticals such as angiotensin converting enzyme-inhibitors, which are extensively used worldwide. Some aspects of the pathophysiology have been elucidated and the vasoactive molecule bradykinin is shown to be one of the main causative agents. The...

  7. Aniseed-induced nocturnal tongue angioedema.

    Science.gov (United States)

    Gázquez García, V; Gaig Jané, P; Bartolomé Zavala, B

    2007-01-01

    Aniseed is a spice native to the eastern Mediterranean region. Cases of simultaneous hypersensitivity to celery, mugwort pollen, and spices of the Umbelliferae family have been described as the celery-mugwort-spices syndrome. We report a case of aniseed-induced tongue angioedema. Skin prick tests to foods proved positive only to aniseed. Serum-specific immunoglobulin (Ig) E determination by enzyme allergosorbent test was 0.4 kU/L to aniseed extract and 0.6 kU/L to tare and cumin seeds. The molecular mass of the IgE-binding proteins studied by sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE) immunoblotting revealed a broad IgE-binding band of 12.9-13.7 kd in aniseed and tare extract assays and a broad band of 15-17.5 kd in cumin extract. This is the first case of type I hypersensitivity due to aniseed liqueur ingestion reported. SDS-PAGE immunoblotting study showed a broad specific IgE-binding band of 12.9-13.7 kd when aniseed extract was incubated with the patient's serum; this band might correspond to the protein responsible for the described symptoms. PMID:18088024

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

    DEFF Research Database (Denmark)

    Longhurst, Hilary; Bygum, Anette

    2016-01-01

    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...... 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...

  9. Attack surfaces

    DEFF Research Database (Denmark)

    Gruschka, Nils; Jensen, Meiko

    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 at...... attack surfaces of the cloud computing scenario participants. © 2010 IEEE....

  10. Heart Attack

    Science.gov (United States)

    ... a million people in the U.S. have a heart attack. About half of them die. Many people have permanent heart damage or die because they don't get ... It's important to know the symptoms of a heart attack and call 9-1-1 if someone ...

  11. Angioedema-like allergic contact dermatitis related to black henna

    OpenAIRE

    Gokalp, Hilal; Kaya, Kismet

    2014-01-01

    Allergic contact dermatitis related to para-phenylendiamine (PPD) from temporary black henna tattoos and hair dyes has become an epidemic in recent years. Several cases of adverse skin reactions to PPD have been reported in the literature. Herein, we present a case of angioedema-like allergic contact dermatitis related to hair coloring with henna.

  12. Angioedema in a Patient with C1 Esterase Inhibitor Deficiency

    Directory of Open Access Journals (Sweden)

    Antonino Murinello

    2005-09-01

    A case of atypical acquired angioedema in a 49-year old man, responding favourably to cinnarizine and alcohol abstinence, is presented in this article. Cinnarizine was prescribed due to presumed alcoholic liver disease. The clinical significant amelioration was not associated with concomitant good laboratory result, which is a relatively common occurrence.

  13. Heart Attack

    Science.gov (United States)

    ... having another heart attack. These medicines include: aspirin, beta blockers, statins, ACE inhibitors and fish oil. Your doctor ... have had a stent placed in your heart. Beta blockers are a group of drugs that lower the ...

  14. Panic Attack

    OpenAIRE

    Scantamburlo, Gabrielle; Ansseau, Marc

    2004-01-01

    Panic attacks can occur unexpectedly and in almost any situation. The present article discusses the main features of anxiety states, the approach, the management and the practice guidelines for the treatment of panic disorder. Peer reviewed

  15. Acute urticaria and angioedema caused by horse-chestnut (aesculus hippocastanum) ingestion: a case report

    OpenAIRE

    Akinci, Emine; OĞUZTÜRK, Oğuzhan; Coşkun, Figen

    2012-01-01

    Acute urticaria and angioedema, which can develop due to various causes, are common life threatening condition seen in emergency departments (EDs). The literature includes reports of angioedema cases developing after contacting various plants and seeds. We present the case of a 47-year-old male patient who developed acute urticaria and angioedema after eating horse-chestnut (aesculus hippocastanum). The patient presented to the ED with redness and irritation spread around the body and swellin...

  16. Immunomodulatory Effects of Balneotherapy with Hae-Un-Dae Thermal Water on Imiquimod-Induced Psoriasis-Like Murine Model

    OpenAIRE

    Lee, Young Bok; Lee, Jun Young; Lee, Hye Jin; Yun, Seong Taek; Lee, Jong Tae; Kim, Hong Jig; Yu, Dong Soo; Woo, So Youn; Kim, Jin-Wou

    2014-01-01

    Background Balneotherapy, although not a well-established dermatological treatment, is thought to have therapeutic properties for psoriasis and is used as an alternative treatment modality throughout the world. Objective To evaluate the mechanism underlying the therapeutic immunologic effects of thermomineral water. Methods A murine model of imiquimod-induced psoriasis-like skin inflammation was used for evaluating the therapeutic effects of balneotherapy with Hae-Un-Dae hot spring mineral wa...

  17. 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.

  18. [Round Table: Urticaria and angioedema: introduction and classification].

    Science.gov (United States)

    Alonso Lebrero, E

    1999-01-01

    Urticaria and angioedema are common diseases in children and adults. Approximately 15-25% of the population will have urticaria or angioedema at least once in their life-time. Urticaria is characterized as the appearance of erythematous, circumscribed, elevated, pruritic, edematous swelling of the upper dermal tissue. Erythematous swelling of the deeper cutaneous and subcutaneous tissue is called angioedema. In angioedema lesions are less pruritic but pain and burning are common. Urticaria may occur in any part of the body, whereas angioedema often involves face, extremities or genitalia. In contrast to other forms of edema there are not symmetric distribution. Urticaria and angioedema are often associated. Urticaria is considered acute if symptoms are present for less than 6 weeks, but usually in childhood lesions disappear in a few days. In chronic urticaria symptoms are longer than 6 weeks; if the episodes were of shorter duration than the symptoms-free periods urticaria is considered recurrent. Acute urticaria has been reported to be the common type in childhood and chronic urticaria is more frequent in adults. Acute urticaria is usually a self-limited benign disease in young children. Nevertheless it is an uncomfortable nuisance, interfering daily activities and sleep, and produces psychosocial impact in patients and parents (an altered self-image is always an alarming situation). Urticaria is a frequent cause of emergency room visit but few patients need to be admitted. Urticaria has long been believed to be an allergic disease but clinically it has rare been proved to be so. The basic mechanism involves the release of diverse vasoactive mediators that arise from the activation of cells or enzymatic pathways. Histamine is the best known of these substances, and elicits the classic triple response consisting of vasodilatation (erythema), increased vascular permeability (edema) and an axon reflex that increases reaction. In contrast to simple symptoms and easy

  19. Visceral Angioedema Induced by Angiotensin Converting Enzyme Inhibitor: Case Report

    OpenAIRE

    Beatriz Frutuoso; Joana Esteves; Mafalda Silva; Pedro Gil; Ana Cristina Carneiro; Sílvio Vale

    2016-01-01

    Introduction: Intestinal angioedema is a rare adverse effect of angiotensin converting enzyme inhibitors. Clinical case: A 42-year old woman presented to the Emergency Department complaining of diffuse abdominal pain, predominantly in the right quadrants, with no other associated symptoms. She had been started on perindopril plus indapamide 72 h before the admission for arterial hypertension. There was no other relevant background. Physical examination suggested peritoneal irritation...

  20. Hereditary angioedema: quality of life in Brazilian patients

    Directory of Open Access Journals (Sweden)

    Maria Abadia Consuelo M. S. Gomide

    2013-01-01

    Full Text Available OBJECTIVE: Hereditary angioedema is a serious medical condition caused by a rare autosomal dominant genetic disorder and it is associated with deficient production or dysfunction of the C1 esterase inhibitor. In most cases, affected patients experience unexpected and recurrent crises of subcutaneous, gastrointestinal and laryngeal edema. The unpredictability, intensity and other factors associated with the disease impact the quality of life of hereditary angioedema patients. We evaluated the quality of life in Brazilian hereditary angioedema patients. METHODS: Patients older than 15 years with any severity of hereditary angioedema and laboratory confirmation of C1 inhibitor deficiency were included. Two questionnaires were used: a clinical questionnaire and the SF-36 (a generic questionnaire. This protocol was approved by the Ethics Committee of Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. RESULTS: The SF-36 showed that 90.4% (mean of all the patients had a score below 70 and 9.6% had scores equal to or higher than 70. The scores of the eight dimensions ranged from 51.03 to 75.95; vitality and social aspects were more affected than other arenas. The internal consistency of the evaluation was demonstrated by a Cronbach's alpha value above 0.7 in seven of the eight domains. CONCLUSIONS: In this study, Brazilian patients demonstrated an impaired quality of life, as measured by the SF-36. The most affected domains were those related to vitality and social characteristics. The generic SF-36 questionnaire was relevant to the evaluation of quality of life; however, there is a need for more specific instruments for better evaluation.

  1. Transient small bowel angioedema due to intravenous iodinated contrast media

    OpenAIRE

    Hu, Xiu-Hua; Gong, Xiang-Yang; Hu, Peng

    2012-01-01

    Three cases of transient proximal small bowel angioedema induced by intravenous administration of nonionic iodinated contrast media (CM) are presented. Computed tomography (CT) images in the venous phase displayed the proximal small bowel with circumferential thickening of the wall including the duodenum and proximal segment of the jejunum. The bowel wall was normal in non-enhanced images, and normal or inconspicuous in arterial phase enhanced images. In one of the three cases, the bowel wall...

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

    DEFF Research Database (Denmark)

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

    2016-01-01

    ) and 16.6 IU/kg (prophylaxis). Approximately 95% of infusions were administered outside of a health care setting. No adverse events (AEs) were reported in retrospective data. Among prospective data (n = 296 subjects; 9148 infusions), 252 AEs were reported in 85 (28.7%) subjects (rate of 0.03 events....../infusion); 9 events were considered related to pnfC1-INH. Two thromboembolic events were reported in subjects with thrombotic risk factors. No patient was noted to have undergone viral testing for suspected blood-borne infection during registry participation. CONCLUSIONS: The findings from this large......, 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...

  3. 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

    Aim: 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. Materials & me

  4. Heart Attack Risk Assessment

    Science.gov (United States)

    ... Pressure Tools & Resources Stroke More Heart Attack Risk Assessment Updated:May 31,2016 We're sorry, but ... Can You Recognize a Heart Attack? Quiz Risk Assessment Patient Information Sheets: Heart Attack Heart Attack Personal ...

  5. Attack warning

    Energy Technology Data Exchange (ETDEWEB)

    1988-12-01

    The North American Aerospace Defense Command, located at Cheyenne Mountain Air Force Station, is responsible for warning the United States and Canadian leaders that North America is under air, missile, or space attack. The Air Force has been developing a replacement for NORAD's communications computer system, and it expects to spend about $281 million on this program-about $72 million for block I and $209 million for block II. Block I of the replacement system has experienced significant schedule and performance problems, such as the inability to meet the requirement for the unit to restore full mission capability within 267 minutes after a total loss of power, and the incompatibility of the unit with other computer equipment due to the wiring standard. These problems have pushed the planned installation data from 1986 to 1999. The Air Force intends to have the contractor correct the deficiencies during block II development. This report discusses how the replacement program should be reassessed.

  6. [Treatment of drugs-associated non-hereditary angioedema mediated by bradykinin].

    Science.gov (United States)

    Muller, Yannick; Harr, Thomas

    2016-01-13

    Angioedema is a deep intradermal or sub-cutaneous edema, which can be mediated by histamine, bradykinin or mixture of both components. The aims of this review are to describe the clinical approach and diagnosis of non-hereditary bradykinin-mediated angioedema induced by drugs such as: angiotensin-converting inhibitor, sartan, gliptins, rapamycin or some thrombolytic reagents and renin inhibitors. Furthermore, we will discuss the drug management of these angioedema, which is mainly based on C1 inhibitor concentrate or icatibant administration. PMID:26946694

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

    Science.gov (United States)

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

    2016-08-01

    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

  8. 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.

  9. 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. PMID:25052839

  10. 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

  11. Angioedema por rellenos faciales: Descripción de cinco casos

    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%.

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

    OpenAIRE

    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 ...

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

    Science.gov (United States)

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

    2016-01-01

    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. PMID:27330660

  14. Anaphylactoid reactions and angioedema during alteplase treatment of acute ischemic stroke

    OpenAIRE

    Hill, M D; Barber, P.A.; Takahashi, J.; Demchuk, A.M.; Feasby, T E; Buchan, A M

    2000-01-01

    Among 105 patients given recombinant tissue plasminogen activator (rt-PA, alteplase) intravenously for acute stroke, 2 (1.9%) had lingual angioedema, which progressed to a fatal anaphylactoid reaction in 1. The authors review the 2 cases and possible mechanisms responsible. They warn that patients who are taking an angiotensin-converting-enzyme inhibitor may be at increased risk for angioedema with concomitant alteplase therapy.

  15. 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.

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

    OpenAIRE

    Roberto Rheingantz da Cunha Filho; Hiram Larangeira de Almeida Jr.; Juliano de Avelar Breunig

    2011-01-01

    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 ...

  17. Heart Attack Recovery FAQs

    Science.gov (United States)

    ... Pressure High Blood Pressure Tools & Resources Stroke More Heart Attack Recovery FAQs Updated:Aug 24,2016 Most people ... recovery. View an animation of a heart attack . Heart Attack Recovery Questions and Answers What treatments will I ...

  18. The effect of long-term danazol prophylaxis on liver function in hereditary angioedema?a longitudinal study

    OpenAIRE

    Farkas, Henriette; Czaller, Ibolya; Csuka, Dorottya; Vas, Anikó; Valentin, Szilvia; Varga, Lilian; Széplaki, Gábor; Jakab, László; Füst, George; Prohászka, Zoltán; Harmat, George; Visy, Beata; Karádi, István

    2009-01-01

    Abstract Background Danazol is a drug most widely used for the prophylaxis of hereditary angioedema resulting from the deficiency of the C1-inhibitor. Potential hepatotoxic or liver tumor-inducing side effects of long-term danazol prophylaxis have been investigated during the follow-up of hereditary angioedema patients. Methods Characteristic parameters of liver function (including bilirubin, GOT, GPT, ?GT...

  19. Active Linkability Attacks

    OpenAIRE

    Schnoor, Henning; Woizekowski, Oliver

    2013-01-01

    We study linking attacks on communication protocols. We show that an active attacker is strictly more powerful in this setting than previously-considered passive attackers. We introduce a formal model to reason about active linkability attacks, formally define security against these attacks and give very general conditions for both security and insecurity of protocols. In addition, we introduce a composition-like technique that allows to obtain security proofs by only studying small component...

  20. [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. PMID:26972946

  1. Membranous nephropathy in a patient with hereditary angioedema: a case report

    Directory of Open Access Journals (Sweden)

    Majoni Sandawana W

    2008-10-01

    Full Text Available Abstract Introduction Hereditary angioedema is the commonest inherited disorder of the complement system and has been associated with several immune glomerular diseases. A case of nephrotic syndrome and renal impairment due to idiopathic membranous glomerulonephritis in a patient with hereditary angioedema has not been described before. Case presentation We present the first reported case of the association of membranous nephropathy and hereditary angioedema in a 43-year-old male Caucasian patient who presented with acute intestinal angioedema, hypertension, acute pancreatitis, renal impairment and generalised body swelling due to severe nephrotic syndrome. We present the challenges involved in the clinical management of the patient. Conclusion This patient's presentation with severe nephrotic syndrome, renal impairment and hypertension required aggressive treatment of the membranous nephropathy given the high risk for progression to end stage renal failure. The contraindication to angiotensin converting enzyme inhibitors and angiotensin II receptor blockers in this patient, the lack of published evidence on the use of alkylating agents and other immunosuppressive agents in patients with hereditary angioedema and the lack of published data on the management of similar cases presented a clinical challenge in this patient's management.

  2. Generating IDS Attack Pattern Automatically Based on Attack Tree

    Institute of Scientific and Technical Information of China (English)

    向尕; 曹元大

    2003-01-01

    Generating attack pattern automatically based on attack tree is studied. The extending definition of attack tree is proposed. And the algorithm of generating attack tree is presented. The method of generating attack pattern automatically based on attack tree is shown, which is tested by concrete attack instances. The results show that the algorithm is effective and efficient. In doing so, the efficiency of generating attack pattern is improved and the attack trees can be reused.

  3. 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

  4. 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 ...

  5. 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 ...

  6. 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 ...

  7. 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. PMID:26560224

  8. Hereditary angioedema type 2 presented as an orbital complication of acute rhinosinusitis.

    Science.gov (United States)

    Somuk, Battal Tahsin; Göktas, Göksel; Özer, Samet; Sapmaz, Emrah; Bas, Yalcın

    2016-03-01

    Hereditary angioedema is an autosomal dominant and life-threatening disorder characterized by recurrent episodes of non-pitting edema affecting the skin, respiratory system and digestive tracts and caused by a congenital deficiency or function defect of the C1 esterase inhibitor. Preseptal cellulitis is defined as an infection of the tissues of the anterior orbital septum. It is generally caused by complications from an upper respiratory tract infection, dacryocystitis, dermal infection, and, rarely, sinusitis. The disease presents with orbital pain, edema on the eyelids, erythema, and fever. In this case, a child with hereditary angioedema type 2 who presented as mimicking a complication of acute sinusitis is discussed. PMID:26857308

  9. Diagnosis and treatment of hereditary angioedema with normal C1 inhibitor

    Directory of Open Access Journals (Sweden)

    Bork Konrad

    2010-07-01

    Full Text Available Abstract Until recently it was assumed that hereditary angioedema is a disease that results exclusively from a genetic deficiency of the C1 inhibitor. In 2000, families with hereditary angioedema, normal C1 inhibitor activity and protein in plasma were described. Since then numerous patients and families with that condition have been reported. Most of the patients by far were women. In many of the affected women, oral contraceptives, hormone replacement therapy containing estrogens, and pregnancies triggered the clinical symptoms. Recently, in some families mutations in the coagulation factor XII (Hageman factor gene were detected in the affected persons.

  10. 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.

  11. 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.

  12. Cutaneous necrosis in pregnancy secondary to activated protein C resistance in hereditary angioedema.

    Science.gov (United States)

    Perkins, W; Downie, I; Keefe, M; Chisholm, M

    1995-04-01

    A 26-year-old woman with hereditary angineurotic oedema (HAE) presented at 22 weeks gestation with severe cutaneous necrosis similar to that seen in coumarin skin necrosis. Protein S deficiency secondary to HAE and pregnancy was postulated. Treatment with heparin, C1-inhibitor concentrates, systemic steroids and surgical debridement resulted in a successful outcome for both mother and child. Subsequent investigations revealed normal levels of protein C, antithrombin III, total protein S, free protein S but reduced function protein S activity with evidence of activated protein C resistance. Cutaneous necrosis has not been reported in associated with activated protein C resistance previously and the possible mechanisms are discussed. PMID:7745572

  13. Seven Deadliest Network Attacks

    Energy Technology Data Exchange (ETDEWEB)

    Prowell, Stacy J [ORNL; Borkin, Michael [None; Kraus, Robert [Solutionary, Inc.

    2010-05-01

    Do you need to keep up with the latest hacks, attacks, and exploits effecting networks? Then you need "Seven Deadliest Network Attacks". This book pinpoints the most dangerous hacks and exploits specific to networks, 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: Denial of Service; War Dialing; Penetration 'Testing'; Protocol Tunneling; Spanning Tree Attacks; Man-in-the-Middle; and, Password Replay. Knowledge is power, find out about the most dominant attacks currently waging war on computers and networks globally. Discover the best ways to defend against these vicious attacks; step-by-step instruction shows you how. Institute countermeasures, don't be caught defenseless again, learn techniques to make your computer and network impenetrable.

  14. What Is a Heart Attack?

    Science.gov (United States)

    ... from the NHLBI on Twitter. What Is a Heart Attack? Español A heart attack happens when the flow ... This Content: NEXT >> Featured Video What is a heart attack? 05/22/2014 Describes how a heart attack ...

  15. What Causes a Heart Attack?

    Science.gov (United States)

    ... from the NHLBI on Twitter. What Causes a Heart Attack? Coronary Heart Disease A heart attack happens if ... This Content: NEXT >> Featured Video What is a heart attack? 05/22/2014 Describes how a heart attack ...

  16. Life After a Heart Attack

    Science.gov (United States)

    ... from the NHLBI on Twitter. Life After a Heart Attack Many people survive heart attacks and live active, ... a few weeks. Anxiety and Depression After a Heart Attack After a heart attack, many people worry about ...

  17. Cricothyroidotomy in a angiotensin-converting enzyme (ACE Inhibitor tongue´s angioedema.

    Directory of Open Access Journals (Sweden)

    Acle-Cervera L, Morales-Angulo C, García-Zornoza R, Rubio Suárez A

    2013-01-01

    Full Text Available Hereditary angioedema by inhibitors of Angiotensin Converting Enzyme(ACE is a very rare disorder. It usually affects the upper airway mucosa andproduce rapidly evolving acute exacerbations requiring urgent treatment.We repost the case of a patient being treated with ACE inhibitors and anreview of prevalence, pathophysiology and management of angioedemawith ACE inhibitors for treatment and the latest treatments.

  18. 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.

  19. Rhucin, a recombinant C1 inhibitor for the treatment of hereditary angioedema and cerebral ischemia.

    Science.gov (United States)

    Longhurst, Hilary

    2008-03-01

    Pharming NV and Esteve are developing Rhucin, a recombinant human C1 esterase inhibitor. Rhucin is currently undergoing phase III clinical trials in North America and is awaiting regulatory approval in Western Europe for the treatment of prophylactic and acute hereditary angioedema. Pharming is also investigating Rhucin for the potential treatment of cerebral ischemic injury. PMID:18311668

  20. Nocturnal panic attacks

    OpenAIRE

    Lopes Fabiana L; Nardi Antonio E; Nascimento Isabella; Valença Alexandre M.; Zin Walter A.

    2002-01-01

    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 sle...

  1. 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.

  2. 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

  3. Seven Deadliest Microsoft Attacks

    CERN Document Server

    Kraus, Rob; Borkin, Mike; Alpern, Naomi

    2010-01-01

    Do you need to keep up with the latest hacks, attacks, and exploits effecting Microsoft products? Then you need Seven Deadliest Microsoft Attacks. This book pinpoints the most dangerous hacks and exploits specific to Microsoft 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. Windows Operating System-Password AttacksActive Directory-Escalat

  4. Seven Deadliest Network Attacks

    CERN Document Server

    Prowell, Stacy; Borkin, Mike

    2010-01-01

    Do you need to keep up with the latest hacks, attacks, and exploits effecting networks? Then you need Seven Deadliest Network Attacks. This book pinpoints the most dangerous hacks and exploits specific to networks, 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: Denial of Service War Dialing Penetration "Testing" Protocol Tunneling Spanning Tree At

  5. Mathematical Attacks on RSA Cryptosystem

    Directory of Open Access Journals (Sweden)

    Imad K. Salah

    2006-01-01

    Full Text Available In this paper some of the most common attacks against Rivest, Shamir, and Adleman (RSA cryptosystem are presented. We describe the integer factoring attacks, attacks on the underlying mathematical function, as well as attacks that exploit details in implementations of the algorithm. Algorithms for each type of attacks are developed and analyzed by their complexity, memory requirements and area of usage.

  6. 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.

  7. Signs of a Heart Attack

    Science.gov (United States)

    ... attack Heart Health and Stroke Signs of a heart attack Related information Make the Call. Don't Miss ... to top More information on Signs of a heart attack Read more from womenshealth.gov Make the Call, ...

  8. 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 ...

  9. 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...

  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...

  11. 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...

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

    OpenAIRE

    Yi-Hsun Wang; Ching-Hao Mao; Hahn-Ming Lee

    2010-01-01

    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...

  13. XQuery Injection Attack and Countermeasures

    Institute of Scientific and Technical Information of China (English)

    谭玉森

    2014-01-01

    As a database that allows data to be stored in XML format, XML database suffers from some similar attacks as traditional relational database does. These attacks include injection attack by XQuey function in application software. These include BaseX, eXist and MarkLogic. In order to defeat these attacks, countermeasures are proposed.

  14. 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

  15. 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. PMID:24237838

  16. 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.

  17. Cell Counting Attack and Browser Attack against TOR Network

    OpenAIRE

    Swati

    2014-01-01

    The onion router (TOR) allows to hide your identity various software under this categories are available that allows online anonymity network, supporting TCP applications over the Internet. It doesn't allow network surveillance or traffic analysis to get tracked but most of these software used equal size of cells (512B). In this paper we are comparing cell-counting attacks and browser attacks against TOR. Different from cell-counting attacks, these attacks can confirm anonymou...

  18. Orolingual angioedema to alteplase. Identify, counsel and monitor at risk patients.

    Science.gov (United States)

    Timmis, Christopher; Epstein, Elliot; Salim, Mohmad

    2016-01-01

    Orolingual angioedema (OLA) is a known complication of intravenous alteplase used to treat ischaemic stroke. The incidence may be as high as 5.1%. ACE inhibitors are thought to increase the risk of developing this potentially life-threatening complication. This case report demonstrates how we may improve in the identification of risk factors in the history; the counselling of patients appropriately; in seeking alternative therapies such as mechanical thrombectomy; and in the monitoring of patients for signs of OLA once alteplase has been given. PMID:27591036

  19. Blocking of Brute Force Attack

    Directory of Open Access Journals (Sweden)

    M.Venkata Krishna Reddy

    2012-08-01

    Full Text Available 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 could take years to find it. Depending on the password's length and complexity, there could be trillions of possible combinations. To speed things up a bit, a brute-force attack could start with dictionary words or slightly modified dictionary words because most people will use those rather than a completely random password. These attacks are called dictionary attacks or hybrid brute-force attacks. Brute-force attacks put user accounts at risk and flood your site with unnecessary traffic. Hackers launch brute-force attacks using widely available tools that utilize wordlists and smart rule set to intelligently and automatically guess user passwords. Although such attacks are easy to detect, they are not so easy to prevent

  20. ATTACK PATTERNS FOR DETECTING AND PREVENTING DDOS AND REPLAY ATTACKS

    Directory of Open Access Journals (Sweden)

    A.MADHURI,

    2010-09-01

    Full Text Available In this paper, we discuss the methods for detecting and preventing the DDoS Attacks and Replay Attacks, which have been posing the problems for the Internet. We explained a scheme AMFDR (AttackPatterns for Marking Filtering DoS and Replay attacks that identifies the attack packets from the packets that are sent by legitimate users and filters the attack packets. A Denial of service attack is generally launched to make a service unavailable even to an unauthorized user. If this attack uses many computers across the world, it is called Distributed Denial of service attack. Replay attack is retransmission of a data transmission which used to gain authentication in a fraudulent manner. These replayed packets or attack packets are identified. This scheme is less expensive and the implementation of this scheme needs minimal interaction with routers. The scheme is like firewall system, so that the occurrence of an attack is recognized quickly and a punitive action is taken without any loss genuine packets.

  1. 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; Rasmussen, Eva Rye

    2016-01-01

    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......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...

  2. Simultaneous Attacks by Terrorist Organisations

    Directory of Open Access Journals (Sweden)

    Kathleen Deloughery

    2013-12-01

    Full Text Available While terror attacks that are a part of a coordinated effort receive attention in the popular media, they have not received much attention in the academic literature. The decision to carry out simultaneous attacks should be examined as one of the choices a terrorist organisation makes about the method of attack. Determining the impact of simultaneous attacks vis-à-vis a single attack can explain why groups would use this method. Up to one quarter of all attacks coded in two major databases, GTD and ITERATE, may be part of a simultaneous campaign. Empirical analysis shows simultaneous attacks are more likely to be successful and cause more fatalities, though not in a one-to-one fashion. These results underline the importance of considering simultaneous attacks in empirical analysis.

  3. When women attack.

    Science.gov (United States)

    McLaughlin, Bryan; Davis, Catasha; Coppini, David; Kim, Young Mie; Knisely, Sandra; McLeod, Douglas

    2015-01-01

    The common assumption that female candidates on the campaign trail should not go on the attack, because such tactics contradict gender stereotypes, has not received consistent support. We argue that in some circumstances gender stereotypes will favor female politicians going negative. To test this proposition, this study examines how gender cues affect voter reactions to negative ads in the context of a political sex scandal, a context that should prime gender stereotypes that favor females. Using an online experiment involving a national sample of U.S. adults (N = 599), we manipulate the gender and partisan affiliation of a politician who attacks a male opponent caught in a sex scandal involving sexually suggestive texting to a female intern. Results show that in the context of a sex scandal, a female candidate going on the attack is evaluated more positively than a male. Moreover, while female participants viewed the female sponsor more favorably, sponsor gender had no effect on male participants. Partisanship also influenced candidate evaluations: the Democratic female candidate was evaluated more favorably than her Republican female counterpart. PMID:26399945

  4. LITERATURE SURVEY ON WORMHOLE ATTACK

    OpenAIRE

    Avinash S. Bundela

    2015-01-01

    Security plays an important role in Mobile Ad Hoc Network when data transmission is performed within un - trusted wireless scenario. Various attacks like Black hole, Wormhole, Gray hole and many more have been identified & corresponding solutions have been proposed. These attacks are caused by the malicious node hence ad hoc wireless network is unprotected from the attacks of the malicious node. Between all these attacks the wormhole att ...

  5. 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

    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...... 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...

  6. 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.

  7. 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

  8. 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.

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

    CERN Document Server

    Wang, Yi-Hsun; Lee, Hahn-Ming; 10.4204/EPTCS.35.2

    2010-01-01

    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...

  10. The attack navigator

    DEFF Research Database (Denmark)

    Probst, Christian W.; Willemson, Jan; Pieters, Wolter

    2016-01-01

    The need to assess security and take protection decisions is at least as old as our civilisation. However, the complexity and development speed of our interconnected technical systems have surpassed our capacity to imagine and evaluate risk scenarios. This holds in particular for risks that are...... caused by the strategic behaviour of adversaries. Therefore, technology-supported methods are needed to help us identify and manage these risks. In this paper, we describe the attack navigator: a graph-based approach to security risk assessment inspired by navigation systems. Based on maps of a socio...

  11. 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.

  12. How Is a Heart Attack Treated?

    Science.gov (United States)

    ... from the NHLBI on Twitter. How Is a Heart Attack Treated? Early treatment for a heart attack can ... or years after the procedure. Other Treatments for Heart Attack Other treatments for heart attack include: Medicines Medical ...

  13. How Is a Heart Attack Diagnosed?

    Science.gov (United States)

    ... from the NHLBI on Twitter. How Is a Heart Attack Diagnosed? Your doctor will diagnose a heart attack ... This Content: NEXT >> Featured Video What is a heart attack? 05/22/2014 Describes how a heart attack ...

  14. 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...

  15. FLOODING ATTACK AWARE SECURE AODV

    OpenAIRE

    Madhavi, S; K. Duraiswamy

    2013-01-01

    Providing security in a Mobile Ad hoc Network (MANET) is a challenging task due to its inherent nature. Flooding is a type of Denial of Service (DoS) attack in MANET. Intentional flooding may lead to disturbances in the networking operation. This kind of attack consumes battery power, storage space and bandwidth. Flooding the excessive number of packets may degrade the performance of the network. This study considers hello flooding attack. As the hello packets are continuously flooded by the ...

  16. Sulfate attack expansion mechanisms

    Energy Technology Data Exchange (ETDEWEB)

    Müllauer, Wolfram, E-mail: wolf_m@gmx.at; Beddoe, Robin E.; Heinz, Detlef

    2013-10-15

    A specially constructed stress cell was used to measure the stress generated in thin-walled Portland cement mortar cylinders caused by external sulfate attack. The effects of sulfate concentration of the storage solution and C{sub 3}A content of the cement were studied. Changes in mineralogical composition and pore size distribution were investigated by X-ray diffraction and mercury intrusion porosimetry, respectively. Damage is due to the formation of ettringite in small pores (10–50 nm) which generates stresses up to 8 MPa exceeding the tensile strength of the binder matrix. Higher sulfate concentrations and C{sub 3}A contents result in higher stresses. The results can be understood in terms of the effect of crystal surface energy and size on supersaturation and crystal growth pressure.

  17. 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

  18. 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

  19. 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.

  20. Aspirin-Exacerbated Diseases: Advances in Asthma with Nasal Polyposis, Urticaria, Angioedema, and Anaphylaxis.

    Science.gov (United States)

    Stevens, Whitney; Buchheit, Kathleen; Cahill, Katherine N

    2015-12-01

    Aspirin-exacerbated diseases are important examples of drug hypersensitivities and include aspirin-exacerbated respiratory disease (AERD), aspirin- or non-steroidal anti-inflammatory drug (NSAID)-induced urticaria/angioedema, and aspirin- or NSAID-induced anaphylaxis. While each disease subtype may be distinguished by unique clinical features, the underlying mechanisms that contribute to these phenotypes are not fully understood. However, the inhibition of the cyclooxygenase-1 enzyme is thought to play a significant role. Additionally, eosinophils, mast cells, and their products, prostaglandins and leukotrienes, have been identified in the pathogenesis of AERD. Current diagnostic and treatment strategies for aspirin-exacerbated diseases remain limited, and continued research focusing on each of the unique hypersensitivity reactions to aspirin is essential. This will not only advance the understanding of these disease processes, but also lead to the subsequent development of novel therapeutics that patients who suffer from aspirin-induced reactions desperately need. PMID:26475526

  1. Thrombolytic drugs for heart attack

    Science.gov (United States)

    ... attack URL of this page: //medlineplus.gov/ency/article/007488.htm Thrombolytic drugs for heart attack To use the sharing features on this page, ... the management of patients with non-ST-elevation acute coronary syndromes: a report ... myocardial infarction: a report of the American College of Cardiology ...

  2. Defense mechanisms against attacks on reputation systems

    OpenAIRE

    Qian, Yuchen

    2013-01-01

    Vain tiivistelmÀ. OpinnÀytteiden arkistokappaleet ovat luettavissa Helsingin yliopiston kirjastossa. Hae HELKA-tietokannasta (http://www.helsinki.fi/helka/index.htm). Abstract only. The paper copy of the whole thesis is available for reading room use at the Helsinki University Library. Search HELKA online catalog (http://www.helsinki.fi/helka/index.htm). Endast avhandlingens sammandrag. Pappersexemplaret av hela avhandlingen finns för lÀsesalsbruk i Helsingfors universitets bibliote...

  3. Superposition Attacks on Cryptographic Protocols

    DEFF Research Database (Denmark)

    Damgård, Ivan Bjerre; Funder, Jakob Løvstad; Nielsen, Jesper Buus;

    2011-01-01

    information. In this paper, we introduce a fundamentally new model of quantum attacks on classical cryptographic protocols, where the adversary is allowed to ask several classical queries in quantum superposition. This is a strictly stronger attack than the standard one, and we consider the security of...... 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...... several primitives in this model. We show that a secret-sharing scheme that is secure with threshold $t$ in the standard model is secure against superposition attacks if and only if the threshold is lowered to $t/2$. We use this result to give zero-knowledge proofs for all of NP in the common reference...

  4. 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.

  5. The Cyber-Physical Attacker

    DEFF Research Database (Denmark)

    Vigo, Roberto

    2012-01-01

    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 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...

  6. Suicide Attacks on the Rise

    OpenAIRE

    CCS Research Staff

    2008-01-01

    This article was published in Culture and Conflict Review (March 2008), v.2 no.2 "The last six weeks has brought some of the worst violence in Afghanistan since 2001. In 2007, there were more than 230 Improvised Explosive Device (IED) attacks and 145 suicide attacks. Casualty rates were at least 25 percent higher in 2007 than the previous year. In the past 18 months, IED attacks have targeted numerous police and army busses, a group of legislators outside a factory at Baghlan, a five-star ...

  7. Lifestyle Changes for Heart Attack Prevention

    Science.gov (United States)

    ... from the NHLBI on Twitter. How Can a Heart Attack Be Prevented? Lowering your risk factors for coronary ... This Content: NEXT >> Featured Video What is a heart attack? 05/22/2014 Describes how a heart attack ...

  8. Social Engineering:A Partial Technical attack

    OpenAIRE

    P.S.Maan; Manish Sharma

    2012-01-01

    This paper suggests the crystal clear concept behind the social engineering attack. Basically social engineering is a non technical attack. But social engineering attack is an attack on human psychology to get the information, but using what? Basically it is an attack on human psychology by using some technical skills or technology. Social engineering attack has many types like fake mail, telephonic cheat etc. which are impossible without any technical skills, so in this paper we suggest that...

  9. 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.

  10. Genetic attack on neural cryptography

    International Nuclear Information System (INIS)

    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

  11. Acute ischemic cerebral attack

    Directory of Open Access Journals (Sweden)

    Franco-Garcia Samir

    2010-12-01

    Full Text Available The decrease of the cerebral blood flow below the threshold of autoregulation led to changes of cerebral ischemia and necrosis that traduce in signs and symtoms of focal neurologic dysfunction called acute cerebrovascular symdrome (ACS or stroke. Two big groups according to its etiology are included in this category the hemorragic that constitue a 20% and the ischemic a 80% of cases. Great interest has wom the ischemic ACS because of its high social burden, being the third cause of no violent death in the world and the first of disability. Many risk factors favor the presentation of these events and some of them are susceptible of modification and therfore are objetives of primary prevention just as the control of diabetes, hypertension and the practice of healthy habits of life. The advances in the knowledge of the physiopatology, had taken to sustantial change in the nomenclature and management of ischemic ACS. Within these changes it was substituted the term cerebrovascular accident fo acute stroke, making emphasis in the key rol of a timely management with goals of time similiar to the acute coronary syndrome. It was redefined the time of acute ischemic attack to a one hour. Once stablished the cerebrovascular attack the semiology of symtoms with frecuency will led us make a topographic diagnosis of the in injury that joined to the cerebral TAC will allow us to exclude an hemorragic event and to start the treatment. In the management of these patients its essential the coordination of the differents teams of work, from the early recognition of symtoms on the part of patients andthe family, the rapid activation and response of emergency systems and the gearing of health care institutions. Are pillars of treatment: the abcde of reanimatiion, to avoid the hiperpirexis, the seizures, the hipoglicemy, the hiperglicemy, to achieve the thrombolysis in the first three hours of the begining of symtoms, to use antiplatelets, antithrombotic profilaxis

  12. Panic Attacks and Panic Disorders

    OpenAIRE

    Fontaine, Rejean; Beaudry, Paul

    1984-01-01

    The diagnosis and treatment of panic attacks and panic disorders have progressed markedly during the last decade. Unlike phobic disorders, the key feature of panic disorders is the many panic attacks that are mostly spontaneous or not caused by a particular situation. Recent studies linking its pathogenesis with lactate infusion tests are reviewed. For treatment, psychotherapy combined with in vivo exposure and pharmacotherapy is more efficacious than either treatment alone. In most cases, ps...

  13. Impersonation Attack on EKE Protocol

    OpenAIRE

    Shirisha Tallapally

    2010-01-01

    The key exchange protocol is one of the most elegant ways of establishing secure communication between pair of users by using a session key. The passwords are of low entropy, hence the protocol should resist all types of password guessing attacks. Recently ECC-3PEKE protocol has been proposed by Chang and Chang. They claimed the protocol is secure, efficient and practical. Unless their claims Yoon and Yoo presented an Undetectable online password guessing attack on the above protocol. A key r...

  14. Migraine attacks the Basal Ganglia

    OpenAIRE

    Bigal Marcelo; Brawn Jennifer; Pendse Gautam; Nutile Lauren; Becerra Lino; Maleki Nasim; Burstein Rami; Borsook David

    2011-01-01

    Abstract Background With time, episodes of migraine headache afflict patients with increased frequency, longer duration and more intense pain. While episodic migraine may be defined as 1-14 attacks per month, there are no clear-cut phases defined, and those patients with low frequency may progress to high frequency episodic migraine and the latter may progress into chronic daily headache (> 15 attacks per month). The pathophysiology of this progression is completely unknown. Attempting to unr...

  15. Social Engineering:A Partial Technical attack

    Directory of Open Access Journals (Sweden)

    P. S. Maan

    2012-03-01

    Full Text Available This paper suggests the crystal clear concept behind the social engineering attack. Basically social engineering is a non technical attack. But social engineering attack is an attack on human psychology to get the information, but using what? Basically it is an attack on human psychology by using some technical skills or technology. Social engineering attack has many types like fake mail, telephonic cheat etc. which are impossible without any technical skills, so in this paper we suggest that , it is a partial technical attack and can be divided in human based and typical computer based social engineering attack.

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

    Science.gov (United States)

    Hermanrud, Thorbjørn; Duus, Nicolaj; Bygum, Anette; Rasmussen, Eva Rye

    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 (ACEi-AE) of the hypopharynx that completely resolved rapidly after the infusion of plasma-derived C1-inhibitor concentrate adding to the sparse reports in the existing literature. PMID:27123347

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

    Science.gov (United States)

    Hermanrud, Thorbjørn; Duus, Nicolaj; Bygum, Anette; Rasmussen, Eva Rye

    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 (ACEi-AE) of the hypopharynx that completely resolved rapidly after the infusion of plasma-derived C1-inhibitor concentrate adding to the sparse reports in the existing literature. PMID:27123347

  18. 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

  19. 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

    2015-08-01

    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

  20. VoIP Malware: Attack Tool & Attack Scenarios

    OpenAIRE

    Nassar, Mohamed; State, Radu; Festor, Olivier

    2009-01-01

    With the appearance of new Internet services like Voice over IP and IP television, malwares are in the way to update and extend their targets. In this paper, we discuss the emergence of a new generation of malwares attacking VoIP infrastructures and services. Such malwares constitute a real threat to the currently deployed VoIP architectures without strong security measures in place. We present one implemented environment that can be used to evaluate such attacks. Our ``VoIP bots'' support a ...

  1. RESIST SRP AGAINST WORMHOLE ATTACK

    Directory of Open Access Journals (Sweden)

    Marjan Kuchaki Rafsanjani

    2013-06-01

    Full Text Available Ad-hoc networks refer to temporary or interim networks which form for special purposes. Actually they are wireless networks with mobile nodes. These networks use no network assisting element for path routing and in these networks available nodes are responsible for path routing. Therefore when malicious nodes want to find a way to interfere with the path routing then the existence of a secure route protocol (SRP can prevent the interference. SRP protocol is one of the secure algorithms of path routing protocol but it is notresistant against wormhole attack. Wormhole attack is considered as a subtle attack in which two malicious nodes make a short connection in network's topology through private or implicit connection and represent two non neighbor nodes as neighbors and prevent the correctoperation of path routing protocol by using this method. One of the methods of preventing wormhole attack is by using packet leashes. We try to decrease the wormhole attack occurrence in this routing protocol by a kind of packet leashes called temporal leashes. We alsowill minimize problems resulting from using temporal leashes by different methods and modifications in its structure.

  2. Hereditary angioedema. Long-term follow-up of 88 patients. Experience of the Argentine Allergy and Immunology Institute.

    Science.gov (United States)

    Fabiani, J E; Avigliano, A; Dupont, J C; Fabiana, J E

    2000-01-01

    Since the detection of the first patient with hereditary angioedema (HA) in 1978, 88 new patients belonging to 16 families have been referred to our clinic. Eighty patients had Type I disease, 5 Type II, and 3 Type III (secondary). We describe the clinical onset, frequent complications, diagnostic tests of the complement system, and abnormalities of the coagulation pathway linked to complement activation. Particular attention was paid to family members who could present succedaneum symptoms. The results of danazole and other therapies and protective and preventive treatment for surgery also are discussed. PMID:11270087

  3. Automated Generation of Attack Trees

    DEFF Research Database (Denmark)

    Vigo, Roberto; Nielson, Flemming; Nielson, Hanne Riis

    2014-01-01

    Attack trees are widely used to represent threat scenarios in a succinct and intuitive manner, suitable for conveying security information to non-experts. The manual construction of such objects relies on the creativity and experience of specialists, and therefore it is error-prone and impractica...

  4. Television Journalism During Terror Attacks

    DEFF Research Database (Denmark)

    Mogensen, Kirsten

    This article views television news coverage of ongoing terrorist attacks and their immediate aftermath as a special genre within journalism, and describes norms connected with the genre. The description is based on qualitative analyses of the coverage on the major American networks in the fi rst ...

  5. Television journalism during terror attacks

    DEFF Research Database (Denmark)

    Mogensen, Kirsten

    2008-01-01

    This article views television news coverage of ongoing terrorist attacks and their immediate aftermath as a special genre within journalism, and describes norms connected with the genre. The description is based on qualitative analyses of the coverage on major American networks the first 24 hours...

  6. FLOODING ATTACK AWARE SECURE AODV

    Directory of Open Access Journals (Sweden)

    S. Madhavi

    2013-01-01

    Full Text Available Providing security in a Mobile Ad hoc Network (MANET is a challenging task due to its inherent nature. Flooding is a type of Denial of Service (DoS attack in MANET. Intentional flooding may lead to disturbances in the networking operation. This kind of attack consumes battery power, storage space and bandwidth. Flooding the excessive number of packets may degrade the performance of the network. This study considers hello flooding attack. As the hello packets are continuously flooded by the malicious node, the neighbor node is not able to process other packets. The functioning of the legitimate node is diverted and destroys the networking operation. Absence of hello packet during the periodical hello interval may lead to wrong assumption that the neighbor node has moved away. So one of the intermediate neighbor nodes sends Route Error (RERR message and the source node reinitiates the route discovery process. In a random fashion the hello interval values are changed and convey this information to other nodes in the network in a secured manner. This study identifies and prevents the flooding attack. This methodology considers the performance parameters such as packet delivery ratio, delay and throughput. This algorithm is implemented in Secure AODV and tested in ad hoc environment. The result of the proposed algorithm decreases the control overhead by 2%.

  7. 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 a...

  8. Performance of attack strategies on modular networks

    CERN Document Server

    da Cunha, Bruno Requião

    2016-01-01

    Vulnerabilities of complex networks have became a trend topic in complex systems recently due to its real world applications. Most real networks tend to be very fragile to high betweenness adaptive attacks. However, recent contributions have shown the importance of interconnected nodes in the integrity of networks and module-based attacks have appeared promising when compared to traditional malicious non-adaptive attacks. In the present work we deeply explore the trade-off associated with attack procedures, introducing a generalized robustness measure and presenting an attack performance index that takes into account both robustness of the network against the attack and the run-time needed to obtained the list of targeted nodes for the attack. Besides, we introduce the concept of deactivation point aimed to mark the point at which the network stops to function properly. We then show empirically that non-adaptive module-based attacks perform better than high degree and betweenness adaptive attacks in networks ...

  9. Network robustness under large-scale attacks

    CERN Document Server

    Zhou, Qing; Liu, Ruifang

    2012-01-01

    Network Robustness under Large-Scale Attacks provides the analysis of network robustness under attacks, with a focus on large-scale correlated physical attacks. The book begins with a thorough overview of the latest research and techniques to analyze the network responses to different types of attacks over various network topologies and connection models. It then introduces a new large-scale physical attack model coined as area attack, under which a new network robustness measure is introduced and applied to study the network responses. With this book, readers will learn the necessary tools to

  10. Generic attack approaches for industrial control systems.

    Energy Technology Data Exchange (ETDEWEB)

    Duggan, David P.

    2006-01-01

    This report suggests a generic set of attack approaches that are expected to be used against Industrial Control Systems that have been built according to a specific reference model for control systems. The posed attack approaches are ordered by the most desirable, based upon the goal of an attacker. Each attack approach is then graded by the category of adversary that would be capable of utilizing that attack approach. The goal of this report is to identify necessary levels of security required to prevent certain types of attacks against Industrial Control Systems.

  11. The inhibition of kallikrein-bradykinin pathway may be useful in the reduction of allergic reactions during honeybee venom immunotherapy

    Directory of Open Access Journals (Sweden)

    Ervin Ç. Mingomataj

    2009-05-01

    Full Text Available "nVenom immunotherapy (VIT protects patients with hymenoptera venom anaphylaxis from subsequent potentially life-threatening reactions. The most important side effects during VIT are systemic anaphylactic reactions (SAR, which are more prevalent during honeybee VIT. Despite the demonstrated diversity with regard to venom compounds, previous publications did not mention the plausible reason that can justify the difference of SAR frequency between honeybee and wasps. On the other hand, pre-treatment with H1-blocking antihistamines reduces the frequency and intensity of local and mild systemic anaphylactic reactions during VIT, but not appropriately moderate adverse reactions such as abdominal pain or angioedematous reactions, which can occur more prevalently also during honeybee VIT. In contrast to hymenoptera venom (HV anaphylaxis, these symptoms are very common during hereditary angioedema (HAE. In addition, in some patients who repeatedly experienced anaphylactic reactions during hyposensitization with HV are reported significantly lower renin, angiotensinogen I, and angiotensinogen II plasma levels. These facts may indicate that during honeybee VIT could be occurred a defective implication of renin-angiotensin system. This may be possible, because among hymenoptera, only the HV contains the antigen melittin, a potent kallikrein activator. These effects during honeybee VIT are similar to the HAE, because melittin-induced kallikrein activation on the first hand, as well as the implication of complement classical pathway during HAE on the second one, can lead both to increased bradykinin (BK secretion, plasma extravasation, and therefore to the occurrence of angioedema and abdominal symptoms. Consequently, the clinical effectiveness of BK receptor and generator blockers such as icatibant, ecallantide or NPC 18884, shown recently during the treatment of HAE attacks and acetic acid-induced abdominal constrictions in mice, may lead to the hypothesis

  12. Hidden Attacks on Power Grid: Optimal Attack Strategies and Mitigation

    OpenAIRE

    Deka, Deepjyoti; Baldick, Ross; Vishwanath, Sriram

    2014-01-01

    Real time operation of the power grid and synchronism of its different elements require accurate estimation of its state variables. Errors in state estimation will lead to sub-optimal Optimal Power Flow (OPF) solutions and subsequent increase in the price of electricity in the market or, potentially overload and create line outages. This paper studies hidden data attacks on power systems by an adversary trying to manipulate state estimators. The adversary gains control of a few meters, and is...

  13. Perfection of Recent Attacks using IP

    Directory of Open Access Journals (Sweden)

    A. RENGARAJAN

    2012-02-01

    Full Text Available The Internet threat monitoring (ITM systems have been deployed to detect widespread attacks on the Internet in recent years. However, the effectiveness of ITM systems critically depends on the confidentiality of the location of their monitors. If adversaries learn the monitor locations of an ITM system, they can bypass the monitors and focus on the uncovered IP address space without being detected. In this paper, we study a new class of attacks, the invisible LOCalization (iLOC attack. The iLOC attack can accurately and invisibly localize monitors of ITM systems. In the iLOC attack, the attacker launches low-rate port-scan traffic, encoded with a selected pseudo noise code (PN-code, to targeted networks. While the secret PN-code is invisible to others, the attacker can accurately determine the existence of monitors in the targeted networks based on whether the PN-code is embedded in the report data queried from the data center of the ITM system. We formally analyze the impact of various parameters on attack effectiveness. We implement the iLOC attack and conduct the performance evaluation on a real-world ITM system to demonstrate the possibility of such attacks. We also conduct extensive simulations on the iLOC attack using real-world traces. Our data show that the iLOC attack can accurately identify monitors while being invisible to ITM systems. Finally, we present a set of guidelines to counteract the iLOC attack.

  14. Intermittent hypoendorphinaemia in migraine attack.

    Science.gov (United States)

    Baldi, E; Salmon, S; Anselmi, B; Spillantini, M G; Cappelli, G; Brocchi, A; Sicuteri, F

    1982-06-01

    Beta-endorphin (RIA method, previous chromatographic extraction) was evaluated in plasma of migraine sufferers in free periods and during attacks. Decreased levels of the endogenous opioid peptide were found in plasma sampled during the attacks but not in free periods. Even chronic headache sufferers exhibited significantly lowered levels of beta-endorphin, when compared with control subjects with a negative personal and family history of head pains. The mechanism of the hypoendorphinaemia is unknown: lowered levels of the neuropeptide, which controls nociception, vegetative functions and hedonia, could be important in a syndrome such as migraine, characterized by pain, dysautonomia and anhedonia. The impairment of monoaminergic synapses ("empty neuron" condition) constantly present in sufferers from serious headaches, could be due to the fact that opioid neuropeptides, because of a receptoral or metabolic impairment, poorly modulate the respective monoaminergic neurons, resulting in imbalance of synaptic neurotransmission. PMID:6290072

  15. Diabetes Ups Risk of Heart Attack Death

    Science.gov (United States)

    ... 159557.html Diabetes Ups Risk of Heart Attack Death Study points to need for better coordinated care, ... people with diabetes have a higher risk of death after a heart attack. "We knew that following ...

  16. Diabetes - preventing heart attack and stroke

    Science.gov (United States)

    ... medlineplus.gov/ency/patientinstructions/000080.htm Diabetes - preventing heart attack and stroke To use the sharing features on ... with diabetes have a higher chance of having heart attacks and strokes. Smoking and having high blood pressure ...

  17. Heart attack - what to ask your doctor

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/patientinstructions/000231.htm Heart attack - what to ask your doctor To use the ... features on this page, please enable JavaScript. A heart attack occurs when blood flow to a part of ...

  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. DULOXETINE-RELATED PANIC ATTACKS

    OpenAIRE

    Sabljić, Vladimir; Rakun, Radmir; Ružić, Klementina; Grahovac, Tanja

    2011-01-01

    Side-effects arising on the grounds of antidepressant administration pose as a substantial obstacle hindering successful depressive disorder treatment. Side-effects, especially those severe or those manifested through dramatic clinical presentations such as panic attacks, make the treatment far more difficult and shake patients’ trust in both the treatment and the treating physician. This case report deals with a patient experiencing a moderately severe depressive episode, who respon...

  20. Coyote Attacks: An Increasing Suburban Problem

    OpenAIRE

    Timm, Robert M.; Baker, Rex O.; Bennett, Joe R.; Coolahan, Craig C.

    2004-01-01

    Coyote attacks on humans and pets have increased within the past 5 years in California. We discuss documented occurrences of coyote aggression and attacks on people, using data from USDA Wildlife Services, the California Department of Fish & Game, and other sources. Forty-eight such attacks on children and adults were verified from 1998 through 2003, compared to 41 attacks during the period 1988 through 1997; most incidents occurred in Southern California near the suburban-wildland interfac...

  1. Sybil attack in Wireless Sensor Network

    OpenAIRE

    Abirami.K; Santhi.B

    2013-01-01

    Wireless network is very susceptible to different types of attack. The main attack is Sybil attack, which allows forming other attacks on the network. Security is very important to the wireless network. In wireless sensor network, to verify node identities by cryptographic authentication but this is not easy because sensor node which contains limited resources. Therefore the current research is going on how to handling the situation of different traffic levels and transmission power for secur...

  2. Faked states attack and quantum cryptography protocols

    CERN Document Server

    Denny, Travis

    2011-01-01

    Leveraging quantum mechanics, cryptographers have devised provably secure key sharing protocols. Despite proving the security in theory, real-world application falls short of the ideal. Last year, cryptanalysts completed an experiment demonstrating a successful eavesdropping attack on commercial quantum key distribution (QKD) systems. This attack exploits a weakness in the typical real-world implementation of quantum cryptosystems. Cryptanalysts have successfully attacked several protocols. In this paper, we examine the Kak quantum cryptography protocol and how it may perform under such attacks.

  3. Panic attacks simulate presence of somatic illnesses

    OpenAIRE

    Latas Milan; Soldatović Ivan; Stamenović Marko; Starčević Vladan

    2009-01-01

    Introduction. Panic attacks are characterized with sudden attacks of anxiety with numerous somatic symptoms, such as palpitations, tachycardia, tachypnea, nausea, vertigo. The objective of this study was to analyze symptoms of panic attacks in patients with panic disorder, especially, to determine the specific relationship of somatic and neurological symptoms of panic attacks in boundaries of somatic systems. Material and methods. The study sample consisted of 97 patients with primary diagnos...

  4. 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...

  5. Understand Your Risk of Heart Attack

    Science.gov (United States)

    ... Stroke More Understand Your Risks to Prevent a Heart Attack Updated:Aug 2,2016 Knowledge is power, so ... medication. This content was last reviewed June 2016. Heart Attack • Home • About Heart Attacks Acute Coronary Syndrome (ACS) • ...

  6. Attacks and countermeasures on AES and ECC

    DEFF Research Database (Denmark)

    Tange, Henrik; Andersen, Birger

    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...

  7. A Novel Attack against Android Phones

    CERN Document Server

    Backes, Michael; von Styp-Rekowsky, Philipp

    2011-01-01

    In the first quarter of 2011, Android has become the top-selling operating system for smartphones. In this paper, we present a novel, highly critical attack that allows unprompted installation of arbitrary applications from the Android Market. Our attack is based on a single malicious application, which, in contrast to previously known attacks, does not require the user to grant it any permissions.

  8. Methods of Identifying and Preventing SQL Attacks

    Directory of Open Access Journals (Sweden)

    Bojken Shehu

    2012-11-01

    Full Text Available The paper begins by identifying the organizations which are vulnerable to the SQL attack referred to as an SQL injection attack. The term SQL injection attack is defined and a diagram is used to illustrate the way that attack occurs. In another section, the paper identifies the methods used to detect an attack to SQL, whereby the techniques are discussed extensively using relevant diagrams for illustration. The other sections cover the preventive methods, where the methods are also discussed with an illustration using diagrams.

  9. 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...

  10. Attacking Embedded Systems through Power Analysis

    Directory of Open Access Journals (Sweden)

    Dr. Sastry JKR

    2011-03-01

    Full Text Available Embedded Systems are being used for the development and implementation of Safety and Mission Critical Systems. Malfunctions of such type of embedded systems will lead to disasters at times. The embedded systems must be fully secured from outside intervention in order to have effective functioning as well as to provide protective environment to these mission critical systems. There are several attacking systems discussed in the literature each requiring a kind of counter attacking system. Power Analysis and variations of power analysis are the significant attacking mechanisms discussed in the literature. Crypto servers are the main areas of attacking as they deal with securing the data that flow in-between several components of the embedded systems. Most of the attacking systems suggested in the litterer suffer from lack of experimental models to emulate the attacking system. An attacking system could be amply proved when several samples of data are used for attacking and the samples of data provides for knowledge base. In this paper an experimental setup is proposed which is an embedded system itself for creation of a Knowledgebase which shall form the basis for attacking. The experimental setup required for undertaking the actual attacking with the usage of the knowledgebase is also presented. Further the proposed attacking system is applied for mission critical system and the experimental results obtained through the simulation are also presented.

  11. 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.

  12. 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

  13. What Are the Symptoms of a Heart Attack?

    Science.gov (United States)

    ... on Twitter. What Are the Symptoms of a Heart Attack? Not all heart attacks begin with the sudden, ... This Content: NEXT >> Featured Video What is a heart attack? 05/22/2014 Describes how a heart attack ...

  14. Migraine attacks the Basal Ganglia

    Directory of Open Access Journals (Sweden)

    Bigal Marcelo

    2011-09-01

    Full Text Available Abstract Background With time, episodes of migraine headache afflict patients with increased frequency, longer duration and more intense pain. While episodic migraine may be defined as 1-14 attacks per month, there are no clear-cut phases defined, and those patients with low frequency may progress to high frequency episodic migraine and the latter may progress into chronic daily headache (> 15 attacks per month. The pathophysiology of this progression is completely unknown. Attempting to unravel this phenomenon, we used high field (human brain imaging to compare functional responses, functional connectivity and brain morphology in patients whose migraine episodes did not progress (LF to a matched (gender, age, age of onset and type of medication group of patients whose migraine episodes progressed (HF. Results In comparison to LF patients, responses to pain in HF patients were significantly lower in the caudate, putamen and pallidum. Paradoxically, associated with these lower responses in HF patients, gray matter volume of the right and left caudate nuclei were significantly larger than in the LF patients. Functional connectivity analysis revealed additional differences between the two groups in regard to response to pain. Conclusions Supported by current understanding of basal ganglia role in pain processing, the findings suggest a significant role of the basal ganglia in the pathophysiology of the episodic migraine.

  15. To mitigate Black-hole attack with CBDS in MANET

    OpenAIRE

    Navjot; Er.Pooja Ran

    2015-01-01

    Mobile ad-hoc network is self configured network that consist of mobile nodes which communicate with each other. Distributed self-organized nature of this network makes it venerable to various attacks likes DOS attack, Black hole attack, wormhole attack and jamming attack etc. Blackhole attack is one of the serious attack in network in which information loss occur which degrades the performance of network. In this work black hole attack is detected with the help of CBDS (cooperati...

  16. Anger attacks in obsessive compulsive disorder

    Directory of Open Access Journals (Sweden)

    Nitesh Prakash Painuly

    2011-01-01

    Full Text Available Background: Research on anger attacks has been mostly limited to depression, and only a few studies have focused on anger attacks in obsessive compulsive disorder. Materials and Methods: In a cross-sectional study all new obsessive compulsive disorder patients aged 20-60 years attending an outpatient clinic were assessed using the anger attack questionnaire, irritability, depression and anxiety scale (for the direction of the aggressive behavior and quality of life (QOL. Results: The sample consisted of 42 consecutive subjects with obsessive compulsive disorder, out of which 21 (50% had anger attacks. The obsessive compulsive disorder subjects with and without anger attacks did not show significant differences in terms of sociodemographic variables, duration of illness, treatment, and family history. However, subjects with anger attacks had significantly higher prevalence of panic attacks and comorbid depression. Significantly more subjects with anger attacks exhibited aggressive acts toward spouse, parents, children, and other relatives in the form of yelling and threatening to hurt, trying to hurt, and threatening to leave. However, the two groups did not differ significantly in terms of QOL, except for the psychological domain being worse in the subjects with anger attacks. Conclusion: Anger attacks are present in half of the patients with obsessive compulsive disorder, and they correlate with the presence of comorbid depression.

  17. Bradykinin-mediated diseases.

    Science.gov (United States)

    Kaplan, Allen P

    2014-01-01

    Diseases which have been demonstrated to be caused by increased plasma levels of bradykinin all have angioedema as the common major clinical manifestation. Angioedema due to therapy with angiotensin-converting enzyme (ACE) inhibitors is caused by suppressed bradykinin degradation so that it accumulates. This occurs because ACE metabolizes bradykinin by removal of Phe-Arg from the C-terminus, which inactivates it. By contrast, angioedema due to C1 inhibitor deficiency (either hereditary types I and II, or acquired) is caused by bradykinin overproduction. C1 inhibitor inhibits factor XIIa, kallikrein and activity associated with the prekallikrein-HK (high-molecular-weight kininogen) complex. In its absence, uncontrolled activation of the plasma bradykinin cascade is seen once there has been an initiating stimulus. C4 levels are low in all types of C1 inhibitor deficiency due to the instability of C1 (C1r, in particular) such that some activated C1 always circulates and depletes C4. In the hereditary disorder, formation of factor XIIf (factor XII fragment) during attacks of swelling causes C4 levels to drop toward zero, and C2 levels decline. A kinin-like molecule, once thought to be a cleavage product derived from C2 that contributes to the increased vascular permeability seen in hereditary angioedema (HAE), is now thought to be an artifact, i.e. no such molecule is demonstrable. The acquired C1 inhibitor deficiency is associated with clonal disorders of B cell hyperreactivity, including lymphoma and monoclonal gammopathy. Most cases have an IgG autoantibody to C1 inhibitor which inactivates it so that the presentation is strikingly similar to type I HAE. New therapies for types I and II HAE include C1 inhibitor replacement therapy, ecallantide, a kallikrein antagonist, and icatibant, a B2 receptor antagonist. A newly described type III HAE has normal C1 inhibitor, although it is thought to be mediated by bradykinin, as is an antihistamine-resistant subpopulation of

  18. Percolation of localized attack on complex networks

    CERN Document Server

    Shao, Shuai; Stanley, H Eugene; Havlin, Shlomo

    2014-01-01

    The robustness of complex networks against node failure and malicious attack has been of interest for decades, while most of the research has focused on random attack or hub-targeted attack. In many real-world scenarios, however, attacks are neither random nor hub-targeted, but localized, where a group of neighboring nodes in a network are attacked and fail. In this paper we develop a percolation framework to analytically and numerically study the robustness of complex networks against such localized attack. In particular, we investigate this robustness in Erd\\H{o}s-R\\'{e}nyi networks, random-regular networks, and scale-free networks. Our results provide insight into how to better protect networks, enhance cybersecurity, and facilitate the design of more robust infrastructures.

  19. Classification of Attacks in Wireless Sensor Networks

    OpenAIRE

    Messai, Mohamed-Lamine

    2014-01-01

    In wireless sensor networks (WSNs), security has a vital importance. Recently, there was a huge interest to propose security solutions in WSNs because of their applications in both civilian and military domains. Adversaries can launch different types of attacks, and cryptography is used to countering these attacks. This paper presents challenges of security and a classification of the different possible attacks in WSNs. The problems of security in each layer of the network's OSI model are dis...

  20. Detection Block Model for SQL Injection Attacks

    OpenAIRE

    Diksha G. Kumar; Madhumita Chatterjee

    2014-01-01

    With the rapid development of Internet, more and more organizations connect their databases to the Internet for resource sharing. However, due to developers' lack of knowledge of all possible attacks, web applications become vulnerable to multiple attacks. Thus the network databases could face multiple threats. Web applications generally consist of a three tier architecture where database is in the third pole, which is the most valuable asset in any organization. SQL injection is an attack te...

  1. Where can an Insider attack?

    DEFF Research Database (Denmark)

    Probst, Christian W.; Hansen, René Rydhof; Nielson, Flemming

    2006-01-01

    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......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...... properties 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....

  2. Detecting Denial of Service Attacks in Tor

    Science.gov (United States)

    Danner, Norman; Krizanc, Danny; Liberatore, Marc

    Tor is currently one of the more popular systems for anonymizing near real-time communications on the Internet. Recently, Borisov et al. proposed a denial of service based attack on Tor (and related systems) that significantly increases the probability of compromising the anonymity provided. In this paper, we propose an algorithm for detecting such attacks and examine the effectiveness of the obvious approach to evading such detection. We implement a simplified version of the detection algorithm and study whether the attack may be in progress on the current Tor network. Our preliminary measurements indicate that the attack was probably not implemented during the period we observed the network.

  3. Off-Path Attacking the Web

    CERN Document Server

    Gilad, Yossi

    2012-01-01

    We show how an off-path (spoofing-only) attacker can perform cross-site scripting (XSS), cross-site request forgery (CSRF) and site spoofing/defacement attacks, without requiring vulnerabilities in either web-browser or server and circumventing known defenses. Attacker can also launch devastating denial of service (DoS) attacks, even when the connection between the client and the server is secured with SSL/TLS. The attacks are practical and require a puppet (malicious script in browser sandbox) running on a the victim client machine, and attacker capable of IP-spoofing on the Internet. Our attacks use a technique allowing an off-path attacker to learn the sequence numbers of both client and server in a TCP connection. The technique exploits the fact that many computers, in particular those running Windows, use a global IP-ID counter, which provides a side channel allowing efficient exposure of the connection sequence numbers. We present results of experiments evaluating the learning technique and the attacks ...

  4. Integrating cyber attacks within fault trees

    International Nuclear Information System (INIS)

    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.

  5. Agent Based Preventive Measure for UDP Flood Attack in DDoS Attacks

    Directory of Open Access Journals (Sweden)

    AARTI SINGH,

    2010-08-01

    Full Text Available Distributed Denial-of-Service (DDoS attack is an attack which makes victim resources and services unavailable to its intended users. In particular, User Datagram Protocol (UDP flood attack in DDoS attacks is a method causing host based denial of service. It occurs when attacker sends UDP packets to a random port on the victim system,causing responses to be sent to forged IP address. The basic thrust of this paper is agent based solution for UDP lood attack because software agent technology seems to be a strong candidate for defending DDoS attacks and very few researchers have thought of deploying agents towards providing solution for UDP attack earlier.

  6. Spatially localized attacks on interdependent networks: the existence of a finite critical attack size

    CERN Document Server

    Berezin, Yehiel; Danziger, Michael M; Li, Daqing; Havlin, Shlomo

    2013-01-01

    Many real world complex systems such as infrastructure, communication and transportation networks are embedded in space, where entities of one system may depend on entities of other systems. These systems are subject to geographically localized failures due to malicious attacks or natural disasters. Here we study the resilience of a system composed of two interdependent spatially embedded networks to localized geographical attacks. We find that if an attack is larger than a finite (zero fraction of the system) critical size, it will spread through the entire system and lead to its complete collapse. If the attack is below the critical size, it will remain localized. In contrast, under random attack a finite fraction of the system needs to be removed to initiate system collapse. We present both numerical simulations and a theoretical approach to analyze and predict the effect of local attacks and the critical attack size. Our results demonstrate the high risk of local attacks on interdependent spatially embedd...

  7. Terrorist attacks escalate in frequency and fatalities preceding highly lethal attacks.

    Directory of Open Access Journals (Sweden)

    Andy Martens

    Full Text Available 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.

  8. A Efficient Approach for Password Attacks

    Directory of Open Access Journals (Sweden)

    I. Naga Geethika

    2014-03-01

    Full Text Available Online password guessing attacks has emerged as a major problem in password based systems. To avoid this we implemented many solutions day by day to restrict bruteforce attacks and password guessing attacks and dictionary attacks. The approach in this paper is that using of mobile alert messages and giving options to the mobile user for more security that will intimate admin that someone is trying to hack his email. The legal users/attackers are challenged to answer the code that is sent to the mobile to retrieve the login page when the number of failed login attempts from a single machine crosses certain threshold value. This protocol uses either the IP addresses of the machines or the browser cookies, or both to identify the machines from which successful logins are made previously. A machine is said to be known when a successful login is made from it and its IP address is added to whitelist. The main goal of this protocol is to limit the attackers with a few failed login attempts made from each unknown machine and forcing them to challenge the security options. Attackers can have a threshold value (For ex 5 which is Whenever a user/attacker types an invalid username.

  9. Panic attacks simulate presence of somatic illnesses

    Directory of Open Access Journals (Sweden)

    Latas Milan

    2009-01-01

    Full Text Available Introduction. Panic attacks are characterized with sudden attacks of anxiety with numerous somatic symptoms, such as palpitations, tachycardia, tachypnea, nausea, vertigo. The objective of this study was to analyze symptoms of panic attacks in patients with panic disorder, especially, to determine the specific relationship of somatic and neurological symptoms of panic attacks in boundaries of somatic systems. Material and methods. The study sample consisted of 97 patients with primary diagnosis of panic disorder, without any acute, severe and unstable somatic illness. The presence and frequency of symptoms of panic attacks were estimated by the Panic Disorder Questionnaire. Results. The study results indicate that the most frequent symptoms of panic attacks were cardiological signs (heart pounding or racing and trembling, followed by unsteady and fainting feeling symptoms, sweating, respiratory symptoms and gastroenterological symptoms. The results of correlation analyses indicate that symptoms of panic attacks classified into cardio-vascular, gastro-enterological, respiratory and neuro-otological systems show statistically significant correlations. Conclusion. The results of analyses of symptoms of panic attacks point to their intercorrelation. This specific association of the symptoms, if they are examined on their own in the patients, could lead to false clinical manifestation of some somatic illness. Therefore, it is necessary to analyze adequately and make the proper differential diagnosis of patients with panic disorder.

  10. Fast Collision Attack on MD5

    NARCIS (Netherlands)

    Stevens, M.M.J.

    2006-01-01

    In this paper, we present an improved attack algorithm to find two-block collisions of the hash function MD5. The attack uses the same differential path of MD5 and the set of sufficient conditions that was presented by Wang et al. We present a new technique which allows us to deterministically fulfi

  11. Rotational Rebound Attacks on Reduced Skein

    DEFF Research Database (Denmark)

    Khovratovich, Dmitry; Nikolic, Ivica; Rechberger, Christian

    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...

  12. The cost of attack in competing networks.

    Science.gov (United States)

    Podobnik, B; Horvatic, D; Lipic, T; Perc, M; Buldú, J M; Stanley, H E

    2015-11-01

    Real-world attacks can be interpreted as the result of competitive interactions between networks, ranging from predator-prey networks to networks of countries under economic sanctions. Although the purpose of an attack is to damage a target network, it also curtails the ability of the attacker, which must choose the duration and magnitude of an attack to avoid negative impacts on its own functioning. Nevertheless, despite the large number of studies on interconnected networks, the consequences of initiating an attack have never been studied. Here, we address this issue by introducing a model of network competition where a resilient network is willing to partially weaken its own resilience in order to more severely damage a less resilient competitor. The attacking network can take over the competitor's nodes after their long inactivity. However, owing to a feedback mechanism the takeovers weaken the resilience of the attacking network. We define a conservation law that relates the feedback mechanism to the resilience dynamics for two competing networks. Within this formalism, we determine the cost and optimal duration of an attack, allowing a network to evaluate the risk of initiating hostilities. PMID:26490628

  13. Automated Detection System for SQL Injection Attacks

    Directory of Open Access Journals (Sweden)

    Dr K.V.N.Sunitha

    2010-10-01

    Full Text Available Many software systems have evolved to include a Web-based component that makes them available to the public via the Internet and can expose them to a variety of Web-based attacks. One of these attacks is SQL Injection vulnerability (SQLIV, which can give attackers unrestricted access to the databases that underlie Web applications and has become increasingly frequent and serious. The intent is that Web applications will limit the kinds of queries that can be generated to a safe subset of all possible queries, regardless of what input users provide. SQL Injection attacks are possible due to the design drawbacks of the web sites, which interact with back-end databases. Successful attacks may damage more. We introduce a system that deals with new automated technique for preventing SQLIA based on the novel concept of regular expressions is to detect SQL Injection attacks. The proposed system can detect the attacks that are from Internet and Insider Attacks, by analyzing the packets of the network servers.

  14. Evaluating Deterioration of Concrete by Sulfate Attack

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Effects of factors such as water to cement ratio, fly ash and silica fume on the resistance of concrete to sulfate attack were investigated by dry-wet cycles and immersion method. The index of the resistance to sulfate attack was used to evaluate the deterioration degree of concrete damaged by sulfate. The relationship between the resistance of concrete to sulfate attack and its permeability/porosity were analyzed as well as its responding mechanism. Results show that the depth of sulfate crystal attack from surface to inner of concrete can be reduced by decreasing w/c and addition of combining fly ash with silica fume. The variation of relative elastic modulus ratio and relative flexural strength ratio of various specimens before and after being subjected to sulfate attack was compared.

  15. 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.

  16. Combating Memory Corruption Attacks On Scada Devices

    Science.gov (United States)

    Bellettini, Carlo; Rrushi, Julian

    Memory corruption attacks on SCADA devices can cause significant disruptions to control systems and the industrial processes they operate. However, despite the presence of numerous memory corruption vulnerabilities, few, if any, techniques have been proposed for addressing the vulnerabilities or for combating memory corruption attacks. This paper describes a technique for defending against memory corruption attacks by enforcing logical boundaries between potentially hostile data and safe data in protected processes. The technique encrypts all input data using random keys; the encrypted data is stored in main memory and is decrypted according to the principle of least privilege just before it is processed by the CPU. The defensive technique affects the precision with which attackers can corrupt control data and pure data, protecting against code injection and arc injection attacks, and alleviating problems posed by the incomparability of mitigation techniques. An experimental evaluation involving the popular Modbus protocol demonstrates the feasibility and efficiency of the defensive technique.

  17. 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...... 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...... 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....

  18. New Multi-step Worm Attack Model

    CERN Document Server

    Robiah, Y; Shahrin, S; Faizal, M A; Zaki, M Mohd; Marliza, R

    2010-01-01

    The traditional worms such as Blaster, Code Red, Slammer and Sasser, are still infecting vulnerable machines on the internet. They will remain as significant threats due to their fast spreading nature on the internet. Various traditional worms attack pattern has been analyzed from various logs at different OSI layers such as victim logs, attacker logs and IDS alert log. These worms attack pattern can be abstracted to form worms' attack model which describes the process of worms' infection. For the purpose of this paper, only Blaster variants were used during the experiment. This paper proposes a multi-step worm attack model which can be extended into research areas in alert correlation and computer forensic investigation.

  19. SURVEY OF PACKET DROPPING ATTACK IN MANET

    Directory of Open Access Journals (Sweden)

    A.Janani

    2014-03-01

    Full Text Available Mobile Ad-hoc NETwork (MANET is an application of wireless network with self-configuring mobile nodes. MANET does not require any fixed infrastructure. Its development never has any threshold range. Nodes in MANET can communicate with each other if and only if all the nodes are in the same range. This wide distribution of nodes makes MANET vulnerable to various attacks, packet dropping attack or black hole attack is one of the possible attack. It is very hard to detect and prevent. To prevent from packet dropping attack, detection of misbehavior links and selfish nodes plays a vital role in MANETs. In this paper, a omprehensive investigation on detection of misbehavior links and malicious nodes is carried out.

  20. 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.

  1. Detection Block Model for SQL Injection Attacks

    Directory of Open Access Journals (Sweden)

    Diksha G. Kumar

    2014-10-01

    Full Text Available With the rapid development of Internet, more and more organizations connect their databases to the Internet for resource sharing. However, due to developers' lack of knowledge of all possible attacks, web applications become vulnerable to multiple attacks. Thus the network databases could face multiple threats. Web applications generally consist of a three tier architecture where database is in the third pole, which is the most valuable asset in any organization. SQL injection is an attack technique in which specially crafted input string is entered in user input field. It is submitted to server and result is returned to the user. In SQL injection vulnerability, the database server is forced to execute malicious operations which may cause the data loss or corruption, denial of access, and unauthentic access to sensitive data by crafting specific inputs. An attacker can directly compromise the database, and that is why this is a most threatening web attack. SQL injection attack occupies first position in top ten vulnerabilities as specified by Open Web Application Security Project. It is probably the most common Website vulnerability today. Current scenarios which provide solutions to SQL injection attack either have limited scope i.e. can’t be implemented across all platforms, or do not cover all types of SQL injection attacks. In this work we implement Message Authentication Code (MAC based solution against SQL injection attacks. The model works both on client and server side. Client side implements a filter function and server side is based on information theory. MAC of static and dynamic queries is compared to detect SQL injection attack.

  2. Research on First Attack Probability Model of an Attacker Level Bombing Anti-air Radars

    Institute of Scientific and Technical Information of China (English)

    LUO Peng-cheng; MU Fu-ling; ZHOU Jing-lun

    2007-01-01

    The paper mainly studies the first attack probability (FAP) of an attacker level bombing anti-air radars through analyzing the radiant point orientation and attack (RPOA) process of attackers. Firstly, the searching target process is analyzed, and a corresponding target finding model is brought forward. Secondly, the target approaching process is concretely analyzed when the attacker levelly bombs the anti-air radar, and a corresponding target approaching model is presented. Finally, a demonstration is given to analyze the major factors in the model and prove its validity.

  3. Utilizing Probabilistic Linear Equations in Cube Attacks

    Institute of Scientific and Technical Information of China (English)

    Yuan Yao; Bin Zhang; Wen-Ling Wu

    2016-01-01

    Cube attacks, proposed by Dinur and Shamir at EUROCRYPT 2009, have shown huge power against stream ciphers. In the original cube attacks, a linear system of secret key bits is exploited for key recovery attacks. However, we find a number of equations claimed linear in previous literature actually nonlinear and not fit into the theoretical framework of cube attacks. Moreover, cube attacks are hard to apply if linear equations are rare. Therefore, it is of significance to make use of probabilistic linear equations, namely nonlinear superpolys that can be approximated by linear expressions effectively. In this paper, we suggest a way to test out and utilize these probabilistic linear equations, thus extending cube attacks to a wider scope. Concretely, we employ the standard parameter estimation approach and the sequential probability ratio test (SPRT) for linearity test in the preprocessing phase, and use maximum likelihood decoding (MLD) for solving the probabilistic linear equations in the online phase. As an application, we exhibit our new attack against 672 rounds of Trivium and reduce the number of key bits to search by 7.

  4. Radiological attacks and accidents. Medical consequences

    International Nuclear Information System (INIS)

    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)

  5. Forensics Investigation of Web Application Security Attacks

    Directory of Open Access Journals (Sweden)

    Amor Lazzez

    2015-02-01

    Full Text Available 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 security attacks targeting a web application every day, and hence improve its security. The aim of this paper is to carry out a detailed overview about the web application forensics. First, we define the web applications forensics, and we present a taxonomic structure of the digital forensics. Then, we present the methodology of a web application forensics investigation. After that, we illustrate the forensics supportive tools for a web application forensics investigation. After that, we present a detailed presentation of a set of the main considered web application forensics tools. Finally, we provide a comparison of the main considered web application forensics tools.

  6. 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.

  7. 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.

  8. FAST CORRELATION ATTACKS ON BLUETOOTH COMBINER

    Institute of Scientific and Technical Information of China (English)

    Ma Weiju; Feng Dengguo

    2006-01-01

    A simple fast correlation attack is used to analysis the security of Bluetooth combiner in this paper.This attack solves the tradeoff between the length of the keystream and the computing complexity needed to recover the secret key. We give the computing complexities of the attack algorithm according to different lengths of the known keystream. The result is less time-consuming than before. It is also shown that the security of the modified Bluetooth combiner by Hermelin and Nyberg is not significantly enhanced.

  9. Review Paper on Flooding Attack in MANET

    Directory of Open Access Journals (Sweden)

    Ruchita Meher,

    2014-01-01

    Full Text Available Mobile ad-hoc network (MANET is widely applicable in various areas like military services, civilian environments and emergency operations. The issues in MANET are broadcasting, clustering, mobility management, bandwidth management and power management. Broadcasting becomes an important issue in MANET for route information discovery. The different routing attacks in MANET are flooding, black hole, link spoofing and wormhole attack. In this paper we are representing works proposed by various author on flooding attack. Our contribution in this paper is that we have presented details comparison of various counter based schemes.

  10. RAPTOR: Routing Attacks on Privacy in Tor

    OpenAIRE

    Sun, Yixin; Edmundson, Anne; Vanbever, Laurent; Li, Oscar; Rexford, Jennifer; Chiang, Mung; Mittal, Prateek

    2015-01-01

    The Tor network is a widely used system for anonymous communication. However, Tor is known to be vulnerable to attackers who can observe traffic at both ends of the communication path. In this paper, we show that prior attacks are just the tip of the iceberg. We present a suite of new attacks, called Raptor, that can be launched by Autonomous Systems (ASes) to compromise user anonymity. First, AS-level adversaries can exploit the asymmetric nature of Internet routing to increase the chance of...

  11. Security Attacks On MANET Routing Protocols

    Directory of Open Access Journals (Sweden)

    Chiragkumar V. Patel

    2013-10-01

    Full Text Available The paper gives idea about various routing protocols for Mobile Ad-Hoc Network against security issues. MANET is vulnerable to various security attacks due to its dynamically changing topology, self configurable nature and lack of centralized control. Malicious node can significantly degrade the performance and reliability of Ad-hoc Network routing protocols. From the survey it has been made quite clear that basic MANET protocols are vulnerable to various routing attacks. While Secure AODV (SAODV routing protocol performs quite well to improve performance in presence of security attack in MANET.

  12. Who Is at Risk for a Heart Attack?

    Science.gov (United States)

    ... on Twitter. Who Is at Risk for a Heart Attack? Certain risk factors make it more likely that ... This Content: NEXT >> Featured Video What is a heart attack? 05/22/2014 Describes how a heart attack ...

  13. Using Fresh Frozen Plasma for Acute Airway Angioedema to Prevent Intubation in the Emergency Department: A Retrospective Cohort Study

    Directory of Open Access Journals (Sweden)

    Aya Saeb

    2016-01-01

    Full Text Available Background. Angioedema (AE is a common condition which can be complicated by laryngeal edema, having up to 40% mortality. Although sporadic case reports attest to the benefits of fresh frozen plasma (FFP in treating severe acute bouts of AE, little evidence-based support for this practice is available at present. Study Objectives. To compare the frequency, duration of intubation, and length of intensive care unit (ICU stay in patients with acute airway AE, with and without the use of FFP. Methods. A retrospective cohort study was conducted, investigating adults admitted to large community hospital ICU with a diagnosis of AE during the years of 2007–2012. Altogether, 128 charts were reviewed for demographics, comorbidities, hospital courses, and outcomes. A total of 20 patients received FFP (108 did not. Results. Demographics and comorbidities did not differ by treatment group. However, nontreated controls did worse in terms of intubation frequency (60% versus 35%; p=0.05 and ICU stay (3.5 days versus 1.5 days; p<0.001. Group outcomes were otherwise similar. Conclusion. In an emergency department setting, the use of FFP should be considered in managing acute airway nonhereditary AE (refractory to steroid, antihistamine, and epinephrine. Larger prospective, better controlled studies are needed to devise appropriate treatment guidelines.

  14. Proposed Methodology for Securing Wireless LANs from Wormhole Attack

    OpenAIRE

    Patel, A K; Jaypalsinh. A. Gohil; D.T. Meva

    2010-01-01

    Wormhole attacks enable an attacker with limited Resources and no cryptographic material to wreak havoc on wireless networks. Initial research focused that this attack is possible only on Adhoc networks, but in present condition wormhole attack is possible on infrastructure based wireless LANs also. We propose architecture and analyze the possibility of wormhole attack along with a countermeasure to avoid such an attack. The proposed mechanism involves the shared information between commun...

  15. 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.

  16. Recovery of infrastructure networks after localised attacks

    Science.gov (United States)

    Hu, Fuyu; Yeung, Chi Ho; Yang, Saini; Wang, Weiping; Zeng, An

    2016-04-01

    The stability of infrastructure network is always a critical issue studied by researchers in different fields. A lot of works have been devoted to reveal the robustness of the infrastructure networks against random and malicious attacks. However, real attack scenarios such as earthquakes and typhoons are instead localised attacks which are investigated only recently. Unlike previous studies, we examine in this paper the resilience of infrastructure networks by focusing on the recovery process from localised attacks. We introduce various preferential repair strategies and found that they facilitate and improve network recovery compared to that of random repairs, especially when population size is uneven at different locations. Moreover, our strategic repair methods show similar effectiveness as the greedy repair. The validations are conducted on simulated networks, and on real networks with real disasters. Our method is meaningful in practice as it can largely enhance network resilience and contribute to network risk reduction.

  17. SECURING MANET FROM BLACKHOLE AND WORMHOLE ATTACKS

    Directory of Open Access Journals (Sweden)

    C.M.Vidhyapathi

    2013-06-01

    Full Text Available Mobile Ad-Hoc networks are self-configuring and self-organizing multi-hop wireless networks. They do not have any fixed infrastructure or centralized management. Due to this, the ad hoc networks are vulnerable to attacks. The routing protocol for MANET considered in this paper is AODV(Ad hoc On-demand Distance Vector Routing Protocol. Blackhole and Wormhole nodes are malicious nodeswhich degrade the performance of the network. They actively participate in the network and conform to forward packets to the destination. The Watchdog Mechanism is used to correct the network from both blackhole and wormhole attacks. The networks originally, with the attacks and after being prevented from attacks are compared on the basis of packets received, throughput, end-to-end delay and packet delivery ratio. ns2 software is used for the simulation.

  18. Women's Heart Disease: Heart Attack Symptoms

    Science.gov (United States)

    ... this page please turn JavaScript on. Feature: Women's Heart Disease Heart Attack Symptoms Past Issues / Winter 2014 Table ... NHLBI has uncovered some of the causes of heart diseases and conditions, as well as ways to prevent ...

  19. Karachi Commander Escapes Attack, 10 Dead

    Institute of Scientific and Technical Information of China (English)

    潘志强

    2004-01-01

    KARACHI: The army commander in the Pakistani city of Karachi narrowly① escaped assassination② yesterday when gunmen attacked his convoy③ with gunfire and a bomb, killing at least 10 and wounding his driver, officials said.

  20. Compiling symbolic attacks to protocol implementation tests

    Directory of Open Access Journals (Sweden)

    Michael Rusinowitch

    2013-07-01

    Full Text Available Recently efficient model-checking tools have been developed to find flaws in security protocols specifications. These flaws can be interpreted as potential attacks scenarios but the feasability of these scenarios need to be confirmed at the implementation level. However, bridging the gap between an abstract attack scenario derived from a specification and a penetration test on real implementations of a protocol is still an open issue. This work investigates an architecture for automatically generating abstract attacks and converting them to concrete tests on protocol implementations. In particular we aim to improve previously proposed blackbox testing methods in order to discover automatically new attacks and vulnerabilities. As a proof of concept we have experimented our proposed architecture to detect a renegotiation vulnerability on some implementations of SSL/TLS, a protocol widely used for securing electronic transactions.

  1. AN APPROACH OF DEFENDING AGAINST DDOS ATTACK

    Institute of Scientific and Technical Information of China (English)

    Wu Zhijun; Duan Haixin; Li Xing

    2006-01-01

    An approach of defending against Distributed Denial of Service (DDoS) attack based on flow model and flow detection is presented. The proposed approach can protect targets from DDoS attacking, and allow targets to provide good service to legitimate traffic under DDoS attacking, with fast reaction. This approach adopts the technique of dynamic comb filter, yields a low level of false positives of less than 1.5%,drops similar percentage of good traffic, about 1%, and passes neglectable percentage of attack bandwidth to the victim, less than 1.5%. The prototype of commercial product, D-fighter, is developed by implementing this proposed approach on Intel network processor platform IXP 1200.

  2. A NEW METHOD FOR RESYNCHRONIZATION ATTACK

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    This paper presents a new method for resynchronization attack, which is the combination of the differential cryptanalysis and algebraic attack. By using the new method one gets a system of linear equations or low-degree equations about initial keys, and the solution of the system of equations results in the recovery of the initial keys. This method has a lower computational complexity and better performance of attack in contrast to the known methods. Accordingly, the design of the resynchronization stream generators should be reconsidered to make them strong enough to avoid our attacks. When implemented to the Toyocrypt, our method gains the computational complexity of O(217), and that of O(267) for LILI-128.

  3. Desynchronization Attacks on RFID Security Protocols

    Directory of Open Access Journals (Sweden)

    Miaolei Deng

    2013-02-01

    Full Text Available The characteristics of radio frequency identification (RFID systems introduce growing security and privacy concerns. RFID systems need security protocols to provide confidentiality, user privacy, mutual authentication and etc. Many security protocols for the RFID system have been presented. This paper analyze several of the newest RFID security protocols which proposed by Niu et al., Fu et al. and Habibi et al. respectively form the security viewpoint of data desynchronization attack. These lightweight protocols were expected to proposed security protections for the RFID system and safeguard against almost all major attacks. However, we found that these RFID security protocols were vulnerable to the attack of data desynchronization. Based on strand spaces model, data desynchronization attacks on these protocols were analyzed and described. Furthermore, improvements to overcome the security vulnerabilities of two protocols presented by Niu et al. and Fu et al. were given.

  4. Dengue Virus May Bolster Zika's Attack

    Science.gov (United States)

    ... page: https://medlineplus.gov/news/fullstory_159534.html Dengue Virus May Bolster Zika's Attack Prior exposure to ... 23, 2016 (HealthDay News) -- Prior exposure to the dengue fever virus may increase the severity of Zika ...

  5. Fault attacks on two software countermeasures

    OpenAIRE

    Moro, Nicolas; Heydemann, Karine; Dehbaoui, Amine; Robisson, Bruno; Encrenaz, Emmanuelle

    2014-01-01

    Short version of the article "Experimental evaluation of two software countermeasures against fault attacks" presented at the 2014 IEEE Symposium on Hardware-Oriented Security and Trust (HOST) in May 2014.

  6. CASE STUDY OF SQL INJECTION ATTACKS

    OpenAIRE

    Sonakshi*, Rakesh Kumar, Girdhar Gopal

    2016-01-01

    Today, most of the web applications are associated with database at back-end so there are possibilities of SQL injection attacks (SQLIA) on it. A number of preventive measures have also been discovered by various researchers to overcome this attack, but which measure is more convenient and provides fast access to application without compromising the security is also a major concern nowadays. This paper provides a clear distinction among different types of SQLIAs and how these can be performed...

  7. Compiling symbolic attacks to protocol implementation tests

    OpenAIRE

    Michael Rusinowitch; Ghazi Maatoug; Hatem Ghabri

    2013-01-01

    Recently efficient model-checking tools have been developed to find flaws in security protocols specifications. These flaws can be interpreted as potential attacks scenarios but the feasability of these scenarios need to be confirmed at the implementation level. However, bridging the gap between an abstract attack scenario derived from a specification and a penetration test on real implementations of a protocol is still an open issue. This work investigates an architecture for automatically g...

  8. Panic Attack during Elective Gastrointestinal Endoscopy

    OpenAIRE

    Charalampos Mitsonis; Nikolaos Dimopoulos; Marianna Zavrou; Vassiliki Psarra; Christos Giofkos; Christos Fiorakis; Athanasios Dimitriadis; Dimitrios Valavanis; Eleni Vousoura; Iannis Zervas; Efstathios Papavassiliou

    2011-01-01

    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 outpatie...

  9. Attacks in WEB Based Embedded Applications

    OpenAIRE

    C. Yaashuwanth; Ramesh, R.

    2010-01-01

    This paper deals with the issues related to embedded applications when they are implemented in internet. There are various attacks in embedded systems when implemented in the internet. These attacks have a negligible effect in the operating system which results in the decrease in the system performance. But in embedded system case, it has life and death consequence attached to it. Many of these embedded systems work in hazardous environment where a system failure results to catastrophic effec...

  10. Wireless Sensor Networks Attacks and Solutions

    OpenAIRE

    Alajmi, Naser

    2014-01-01

    A few years ago, wireless sensor networks (WSNs) used by only military. Now, we have seen many of organizations use WSNs for some purposes such as weather, pollution, traffic control, and healthcare. Security is becoming on these days a major concern for wireless sensor network. In this paper I focus on the security types of attacks and their detection. This paper anatomizes the security requirements and security attacks in wireless sensor networks. Also, indicate to the benchmarks for the se...

  11. Cluster headache attacks and multiple sclerosis

    OpenAIRE

    Gentile, Salvatore; Ferrero, Margherita; Vaula, Giovanna; Rainero, Innocenzo; Pinessi, Lorenzo

    2007-01-01

    We report the case of a patient who developed typical cluster headache attacks and was diagnosed as having multiple sclerosis (MS) at the same time. The headache attacks resolved after i.v. treatment with methylprednisolone. MR imaging showed a pontine demyelinating lesion involving the trigeminal nerve root inlet area, on the same side as the pain. The association between cluster headache and MS has been rarely described before. This case suggests that in patients with cluster headache neuro...

  12. 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.

  13. Two Improved Multiple-Differential Collision Attacks

    OpenAIRE

    An Wang; Liji Wu; Zongyue Wang; Xuexin Zheng; Man Chen; Jing Ma

    2014-01-01

    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 implementat...

  14. Various Database Attacks and its Prevention Techniques

    OpenAIRE

    K.A.VarunKumar; M.Prabakaran; Ajay Kaurav; S.Sibi Chakkaravarthy; Thiyagarajan, S; Pokala Venkatesh

    2014-01-01

    Increasing in the popularity of internet, the application of database also widely spread. There are some serious threats because of hackers done various attempts to steal the data in the database. Various attacks like Sql injection, Cross site scripting may change the information in the databases which decreases the truthfulness of the database. Intrusion detection system is used to detect whether the attack is carried on the database. In this paper we surveyed different types of database att...

  15. Private Circuits II versus Fault Injection Attacks

    OpenAIRE

    Rakotomalala, Henitsoa; Ngo, Xuan Thuy; Najm, Zakaria; Danger, Jean-Luc; Guilley, Sylvain

    2015-01-01

    —Cryptographic implementations are subject to physical attacks. Private circuits II is a proven protection against a strong attacker, able to read and write on a finite number of chosen internal nodes. In practice, side-channel analyses and fault injections are less accurate: typically, classical injection techniques (clock and power glitches, electromagnetic pulses, etc.) can be reproducible, but they do not allow to choose the targeted nodes (the situation is different for software dual-rai...

  16. Logical Modes of Attack in Argumentation Networks

    OpenAIRE

    Gabbay, Dov M.; Garcez, A. S. D. Avila

    2009-01-01

    This paper studies methodologically robust options for giving logical contents to nodes in abstract argumentation networks. It defines a variety of notions of attack in terms of the logical contents of the nodes in a network. General properties of logics are refined both in the object level and in the meta level to suit the needs of the application. The network-based system improves upon some of the attempts in the literature to define attacks in terms of defeasible proofs, the...

  17. Session Initiation Protocol Attacks and Challenges

    OpenAIRE

    Keshavarz, Hassan; Sattari, Mohammad Reza Jabbarpour; Noor, Rafidah Md

    2012-01-01

    In recent years, Session Initiation Protocol (SIP) has become widely used in current internet protocols. It is a text-based protocol much like Hyper Text Transport Protocol (HTTP) and Simple Mail Transport Protocol (SMTP). SIP is a strong enough signaling protocol on the internet for establishing, maintaining, and terminating session. In this paper the areas of security and attacks in SIP are discussed. We consider attacks from diverse related perspectives. The authentication schemes are comp...

  18. To mitigate Black-hole attack with CBDS in MANET

    Directory of Open Access Journals (Sweden)

    Navjot

    2015-06-01

    Full Text Available Mobile ad-hoc network is self configured network that consist of mobile nodes which communicate with each other. Distributed self-organized nature of this network makes it venerable to various attacks likes DOS attack, Black hole attack, wormhole attack and jamming attack etc. Blackhole attack is one of the serious attack in network in which information loss occur which degrades the performance of network. In this work black hole attack is detected with the help of CBDS (cooperative Bait Detection Algorithm and MD5 is used for the security purpose. This work is implemented in Network simulator and performance is checked on the bases of network parameters.

  19. Noncombatants and liability to be attacked in wars

    DEFF Research Database (Denmark)

    Lippert-Rasmussen, Kasper

    2013-01-01

    attack," I mean that S has forfeited his or her right not to be attacked by armed forces. Here, "liable" is used in a normative-cum-legal sense, not descriptively as it is when one says "some colors are liable to darken in perpetual shade," and "attack" is used passively, as an equivalent of be attacked...... mean to focus on intended, as opposed to incidental, attacks. Finally, although "attack" often implies initiation of a conflict ("I didn't attack him! He started it!"), it does not do so here. An unjust aggressor who initiates a conflict can be, and indeed very often is, liable to attack. All...

  20. Distinguishing attack and second-preimage attack on encrypted message authentication codes (EMAC)

    Science.gov (United States)

    Ariwibowo, Sigit; Windarta, Susila

    2016-02-01

    In this paper we show that distinguisher on CBC-MAC can be applied to Encrypted Message Authentication Code (EMAC) scheme. EMAC scheme in general is vulnerable to distinguishing attack and second preimage attack. Distinguishing attack simulation on AES-EMAC using 225 message modifications, no collision have been found. According to second preimage attack simulation on AES-EMAC no collision found between EMAC value of S1 and S2, i.e. no second preimage found for messages that have been tested. Based on distinguishing attack simulation on truncated AES-EMAC we found collision in every message therefore we cannot distinguish truncated AES-EMAC with random function. Second-preimage attack is successfully performed on truncated AES-EMAC.

  1. 韩国东海天然地奖水天津市场现状研究%The Market Situation of Dong Hae Di Jang Soo, a Bottled Mineral Water from Korea

    Institute of Scientific and Technical Information of China (English)

    罗明薇; 周璇

    2014-01-01

    As the brand of Dong Hae Di Jang Soo is less popular and the bottled mineral water prices high now, it is difficult to increase its market share in Tianjin. In general, the market of high-end water is immature in Tianjin, all brands of the water not selling well. However, the market potential is great with an increasing demand of the water by an impressive amount of high-income target consumers in the city. Given that, brand operators of the water shall seize the opportunity to promote the brand by building brand awareness and reputation and expand its market share while breaking the monopoly of other foreign brands.%目前,韩国东海天然地奖水由于品牌知名度低,产品价格偏高,提升其在天津市场占有率还有一定难度。总体上,天津高端水市场发育尚不成熟,各品牌销售情况不佳,但天津高收入目标消费者数量可观,对高端水的需求不断增长,消费潜力很大,市场前景非常乐观。韩国东海天然地奖水经营者应该认识到天津高端水市场尚未被充分开发的现状,顺应消费趋势,抓住市场上缺少领导品牌的有利时机,强化品牌推广,努力提升品牌知名度、美誉度,打破国外其他品牌的独占和垄断格局,扩大其市场份额。

  2. A case of mimicking angioedema: chin silicone granulomatous reaction spreading all over the face after receiving liquid silicone injection forty years previously

    Institute of Scientific and Technical Information of China (English)

    Yu-cheng Chen; Mei-ling Chen; Ying-ming Chiu

    2011-01-01

    Liquid injectable silicone has been used for soft tissue augmentation for five decades. Many complications following liquid silicone injection have been reported. To diagnose and manage silicone granuloma remains difficult. Silicone granuloma must be diagnosed with the history of liquid silicone injection and the histology of tissue biopsy. We presented a case of granulomatous reaction after the injection of liquid silicone for chin augmentation forty years ago, causing total facial swelling, which mimicking angioedema initially. We administered methylprednisolone to the patient. Initial response to methylprednisolone was favorable.

  3. 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.

  4. Detecting and Localizing Wireless Network Attacks Techniques

    Directory of Open Access Journals (Sweden)

    Iyad Aldasouqi

    2010-03-01

    Full Text Available In order to increase employee productivity within a feasible budget, we have to track newtechnologies, investigate and choose the best plan and implementation of these technologies.WLAN is vulnerable to malicious attacks due to their shared medium in unlicensed frequencyspectrum, thus requiring security features for a variety of applications.This paper will discuss some techniques and approaches which can help to detect, localize andidentify wireless network attacks, which present a unique set of challenges to IT and securityprofessionals. All efforts were focusing on the ability to identity based attacks in which amalicious device uses forged MAC addresses to masquerade as a specific client or to createmultiple illegitimate identities. Also, to be sure that the network is able to robustly identify eachtransmitter independently of packet contents, allowing detection of a large class of identity-basedattacks with high probability.The attacker can listen to all wireless traffic, compromise encryption and Use attenuators,amplifiers, directional antennas, software radios, but he cannot be at the location of user or at thelocation of access points. However, we have to choose the best design, implementation, andevaluation techniques in order to secure our network from attackers, where our choice willdepend on a technical implementation to mitigate the risk on the enterprise network infrastructure.

  5. Power analysis attacks revealing the secrets of smart cards

    CERN Document Server

    Mangard, Stefan; Popp, Thomas

    2008-01-01

    A comprehensive treatment of power analysis attacks and countermeasures. Based on the principle that the only way to defend against power analysis attacks is to understand them, this book explains how power analysis attacks work. It discusses simple and differential power analysis as well as advanced techniques like template attacks.

  6. Coronary Artery Dissection: Not Just a Heart Attack

    Science.gov (United States)

    ... Stroke More Coronary Artery Dissection: Not Just a Heart Attack Updated:May 24,2016 Sometimes a heart attack ... Disease Go Red For Women Types of aneurysms Heart Attack • Home • About Heart Attacks Acute Coronary Syndrome (ACS) • ...

  7. 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.

  8. Transforming Graphical System Models to Graphical Attack Models

    DEFF Research Database (Denmark)

    Ivanova, Marieta Georgieva; Probst, Christian W.; Hansen, Rene Rydhof;

    2016-01-01

    approach to transforming graphical system models to graphical attack models in the form of attack trees. Based on an asset in the model, our transformations result in an attack tree that represents attacks by all possible actors in the model, after which the actor in question has obtained the asset....

  9. Proposed Methodology for Securing Wireless LANs from Wormhole Attack

    Directory of Open Access Journals (Sweden)

    A.K. Patel

    2010-05-01

    Full Text Available Wormhole attacks enable an attacker with limited Resources and no cryptographic material to wreak havoc on wireless networks. Initial research focused that this attack is possible only on Adhoc networks, but in present condition wormhole attack is possible on infrastructure based wireless LANs also. We propose architecture and analyze the possibility of wormhole attack along with a countermeasure to avoid such an attack. The proposed mechanism involves the shared information between communicating Access Points to prevent Rouge Access Points from masquerading as false neighbours. Our defense greatly diminishes the threat of wormhole attacks and requires no location information or clock synchronization.

  10. Attack Prevention for Collaborative Spectrum Sensing in Cognitive Radio Networks

    CERN Document Server

    Duan, Lingjie; Huang, Jianwei; Shin, Kang G

    2011-01-01

    Collaborative spectrum sensing can significantly improve the detection performance of secondary unlicensed users (SUs). However, the performance of collaborative sensing is vulnerable to sensing data falsification attacks, where malicious SUs (attackers) submit manipulated sensing reports to mislead the fusion center's decision on spectrum occupancy. Moreover, attackers may not follow the fusion center's decision regarding their spectrum access. This paper considers a challenging attack scenario where multiple rational attackers overhear all honest SUs' sensing reports and cooperatively maximize attackers' aggregate spectrum utilization. We show that, without attack-prevention mechanisms, honest SUs are unable to transmit over the licensed spectrum, and they may further be penalized by the primary user for collisions due to attackers' aggressive transmissions. To prevent such attacks, we propose two novel attack-prevention mechanisms with direct and indirect punishments. The key idea is to identify collisions...

  11. Liability for damage caused by terrorist attacks

    International Nuclear Information System (INIS)

    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.)

  12. Quantifying Mixed Uncertainties in Cyber Attacker Payoffs

    Energy Technology Data Exchange (ETDEWEB)

    Chatterjee, Samrat; Halappanavar, Mahantesh; Tipireddy, Ramakrishna; Oster, Matthew R.; Saha, Sudip

    2015-04-15

    Representation and propagation of uncertainty in cyber attacker payoffs is a key aspect of security games. Past research has primarily focused on representing the defender’s beliefs about attacker payoffs as point utility estimates. More recently, within the physical security domain, attacker payoff uncertainties have been represented as Uniform and Gaussian probability distributions, and intervals. Within cyber-settings, continuous probability distributions may still be appropriate for addressing statistical (aleatory) uncertainties where the defender may assume that the attacker’s payoffs differ over time. However, systematic (epistemic) uncertainties may exist, where the defender may not have sufficient knowledge or there is insufficient information about the attacker’s payoff generation mechanism. Such epistemic uncertainties are more suitably represented as probability boxes with intervals. In this study, we explore the mathematical treatment of such mixed payoff uncertainties.

  13. Counteracting Power Analysis Attacks by Masking

    Science.gov (United States)

    Oswald, Elisabeth; Mangard, Stefan

    The publication of power analysis attacks [12] has triggered a lot of research activities. On the one hand these activities have been dedicated toward the development of secure and efficient countermeasures. On the other hand also new and improved attacks have been developed. In fact, there has been a continuous arms race between designers of countermeasures and attackers. This chapter provides a brief overview of the state-of-the art in the arms race in the context of a countermeasure called masking. Masking is a popular countermeasure that has been extensively discussed in the scientific community. Numerous articles have been published that explain different types of masking and that analyze weaknesses of this countermeasure.

  14. Early Detection of SQL Injection Attacks

    Directory of Open Access Journals (Sweden)

    Hossain Shahriar

    2013-07-01

    Full Text Available SQL Injection (SQLI is a common vulnerability found in web applications.The starting point of SQLIattack is the client-side (browser. If attack inputs can be detected early at the browse side, then it could bethwarted early by not forwarding the malicious inputs to the server-side for further processing.This paperpresents a client-side approach to detect SQLI attacks1. The client-side accepts shadow SQL queries fromthe server-side and checks any deviation betweenshadow queries with dynamic queries generated withuser supplied inputs. We measure the deviation of shadow query and dynamic query based on conditionalentropy metrics and propose four metrics in this direction. We evaluate the approach with three PHPapplications containing SQLI vulnerabilities. The evaluation results indicate that our approach can detectwell-known SQLI attacks early at the client-side and impose negligible overhead.

  15. Quantifying Public Response towards Islam on Twitter after Paris Attacks

    OpenAIRE

    Magdy, Walid; Darwish, Kareem; Abokhodair, Norah

    2015-01-01

    The Paris terrorist attacks occurred on November 13, 2015 prompted a massive response on social media including Twitter, with millions of posted tweets in the first few hours after the attacks. Most of the tweets were condemning the attacks and showing support to Parisians. One of the trending debates related to the attacks concerned possible association between terrorism and Islam and Muslims in general. This created a global discussion between those attacking and those defending Islam and M...

  16. Effect of migraine attacks on paracetamol absorption.

    OpenAIRE

    Tokola, R A; Neuvonen, P J

    1984-01-01

    The absorption of effervescent paracetamol (1000 mg) was investigated in nine female patients during a migraine attack and in the same patients when headache free. Migraine attack decreased (P less than 0.05) the areas under the serum paracetamol concentration-time curves (AUC) of 0-2 h, 0-4 h and 0-6 h and the peak serum concentration. The severity of nausea correlated significantly with the decrease in the AUC values. Our results support findings of delayed gastric emptying in migraine atta...

  17. Attack strategies on quantum cryptographic protocols

    International Nuclear Information System (INIS)

    Full text: Quantum key distribution (QKD) and quantum authentication (QA) have been a topic of extensive research in the last 20 years. In course of that many attacks on QKD and QA protocols have been studied. Among these, Zhang, Lee and Guo presented an attack on a QKD protocol using entanglement swapping. Based on that strategy we take a look at other protocols to inspect how much information an adversary may get if he shares entanglement with either one or both parties. We will present some protocols where an adversary can even get full information about the key using entanglement. (author)

  18. Session Initiation Protocol Attacks and Challenges

    CERN Document Server

    Keshavarz, Hassan; Noor, Rafidah Md

    2012-01-01

    In recent years, Session Initiation Protocol (SIP) has become widely used in current internet protocols. It is a text-based protocol much like Hyper Text Transport Protocol (HTTP) and Simple Mail Transport Protocol (SMTP). SIP is a strong enough signaling protocol on the internet for establishing, maintaining, and terminating session. In this paper the areas of security and attacks in SIP are discussed. We consider attacks from diverse related perspectives. The authentication schemes are compared, the representative existing solutions are highlighted, and several remaining research challenges are identified. Finally, the taxonomy of SIP threat will be presented.

  19. Fast Collision Attack on MD5

    OpenAIRE

    Stevens, Marc

    2006-01-01

    In this paper, we present an improved attack algorithm to find two-block collisions of the hash function MD5. The attack uses the same differential path of MD5 and the set of sufficient conditions that was presented by Wang et al. We present a new technique which allows us to deterministically fulfill restrictions to properly rotate the differentials in the first round. We will present a new algorithm to find the first block and we will use an algorithm of Klima to find the second block. To o...

  20. Textual Manipulation for SQL Injection Attacks

    Directory of Open Access Journals (Sweden)

    Hussein AlNabulsi

    2013-11-01

    Full Text Available SQL injection attacks try to use string or text manipulations to access illegally websites and their databases. This is since using some symbols or characters in SQL statements may trick the authentication system to incorrectly allow such SQL statements to be processed or executed. In this paper, we highlighted several examples of such text manipulations that can be successfully used in SQL injection attacks. We evaluated the usage of those strings on several websites and web pages using SNORT open source. We also conducted an extensive comparison study of some relevant papers.

  1. Defending Against Wormhole Attack in OLSR

    Institute of Scientific and Technical Information of China (English)

    HONG Liang; HONG Fan; FU Cai

    2006-01-01

    OLSR (optimal link state routing) is one of the four basic routing protocols used in mobile ad hoc Networks by the MANET working group of IETF(Internet engineering task force). OLSR, a proactive routing protocol, is based on a multipoint relaying flooding technique to reduce the number of topology broadcast. OLSR uses periodic HELLO packets to neighbor detection. As introduced in Reference [1], the wormhole attack can form a serious threat in wireless Networks, especially against many ad hoc Network routing protocols and location-based wireless security systems. Here, a trust model to handle this attack in OLSR is provided and simulated in NS2.

  2. A novel proposed network security management approach for cyber attacks

    International Nuclear Information System (INIS)

    Network security is a discipline that focuses on securing networks from unauthorized access. Given the Escalating threats of malicious cyber attacks, modern enterprises employ multiple lines of defense. A comprehensive defense strategy against such attacks should include (I) an attack detection component that deter- mines the fact that a program is compromised, (2) an attack identification and prevention component that identifies attack packets so that one can block such packets in the future and prevents the attack from further propagation. Over the last decade, a significant amount of research has been vested in the systems that can detect cyber attacks either statically at compile time or dynamically at run time, However, not much effort is spent on automated attack packet identification or attack prevention. In this paper we present a unified solution to the problems mentioned above. We implemented this solution after the forward engineering of Open Source Security Information Management (OSSIM) system called Preventive Information Security management (PrISM) system that correlates input from different sensors so that the resulting product can automatically detect any cyber attack against it and prevents by identifying the actual attack packet(s). The PrISM was always able to detect the attacks, identify the attack packets and most often prevent by blocking the attacker's IP address to continue normal execution. There is no additional run-time performance overhead for attack prevention. (author)

  3. Managing burn victims of suicide bombing attacks: outcomes, lessons learnt, and changes made from three attacks in Indonesia

    OpenAIRE

    Chim, Harvey; Yew, Woon Si; Song, Colin

    2007-01-01

    Introduction Terror attacks in Southeast Asia were almost nonexistent until the 2002 Bali bomb blast, considered the deadliest attack in Indonesian history. Further attacks in 2003 (Jakarta), 2004 (Jakarta), and 2005 (Bali) have turned terrorist attacks into an ever-present reality. Methods The authors reviewed medical charts of victims evacuated to the Singapore General Hospital (SGH) Burns Centre during three suicide attacks involving Bali (2002 and 2005) and the Jakarta Marriott hotel (200...

  4. Cryptographic Attacks and Countermeasures - A Mathematical View

    OpenAIRE

    Canright, David; Stanica, Pantelimon

    2009-01-01

    2009 CyberSecurity Summit, Naval Postgraduate School 1st NPS Cyber Summit was held at the Naval Postgraduate School in Monterey, California on October 29, 2009. Sessions were held on the following topics: security requirements, Kerchkoffs' principle, mathematics of cybersecurity, the data encryption standard, nonlinear equations, Cyber attacks on Bluetooth EQ, Crypto properties of S-boxes.

  5. Modified localized attack on complex network

    Science.gov (United States)

    Dong, Gaogao; Du, Ruijin; Hao, Huifang; Tian, Lixin

    2016-01-01

    Since a shell structure contains a wealth of information, it is not only very important for understanding the transport properties of the network, but also essential to identify influential spreaders in complex networks. Nodes within each shell can be classified into two categories: protected nodes and unprotected nodes. In this paper, we propose a generalization of the localized attack, modified localized attack, which means that when a randomly chosen node (root node) is under attack, protected nodes will not be removed, but unprotected nodes in the nearest shells will fail. We numerically and analytically study the system robustness under this attack by taking an Erdös-Rényi (ER) network, a regular random (RR) network and a scale-free (SF) network as examples. Moreover, a fraction of nodes belonging to giant component S and a critical threshold q c , where S approaches to zero, are given. The result implies that increasing connection density has been found to be useful to significantly improve network robustness.

  6. Chemical Attack of Malaysian Pozzolans Concrete

    Directory of Open Access Journals (Sweden)

    Mohd Hilton Ahmad

    2011-09-01

    Full Text Available Malaysia produces more than 10 million tonne of by-product from industrial sector per year. As the environmental impact concerns, more than half of the by-product can be recycled to be used as construction materials. One of them is pozzolan, a cement replacing material that can be used to enhance the properties of the concrete. This paper studies the chemical attack to local prozzolans concrete. The parameters studied include weight loss, length change, and residual strength of local pozzolans concrete after been exposed to severe environment. The specimen were tested under normal room temperature, exposed to magnesium sulfate, MgSO4. 2H2O where both sulfate attack and acid attack take place. Two series of pozzolans< which is Pulverized fly ash (PFA and Palm oil fuel ash (POFA were identified. Another series of admixture, Quarry Dust (QD from quarry waste that contain high amount of silica content also been carried out. Each series will study the effect of cement replacement percentage of 5%, 10% and 15%. The parameters were compared to conventional ordinary Portland cement (OPC concrete as control mix. Accelerated testing was conducted at 3, 7, 28, 56 and 90 days. The result shows that the local pozzolans concrete were found to be in good resistance against sulfate attack compared to conventional concrete. Compared to all series conducted, series of PFA replacement gave the best resistance followed by POFA and QD replacement series.

  7. Adversarial Feature Selection Against Evasion Attacks.

    Science.gov (United States)

    Zhang, Fei; Chan, Patrick P K; Biggio, Battista; Yeung, Daniel S; Roli, Fabio

    2016-03-01

    Pattern recognition and machine learning techniques have been increasingly adopted in adversarial settings such as spam, intrusion, and malware detection, although their security against well-crafted attacks that aim to evade detection by manipulating data at test time has not yet been thoroughly assessed. While previous work has been mainly focused on devising adversary-aware classification algorithms to counter evasion attempts, only few authors have considered the impact of using reduced feature sets on classifier security against the same attacks. An interesting, preliminary result is that classifier security to evasion may be even worsened by the application of feature selection. In this paper, we provide a more detailed investigation of this aspect, shedding some light on the security properties of feature selection against evasion attacks. Inspired by previous work on adversary-aware classifiers, we propose a novel adversary-aware feature selection model that can improve classifier security against evasion attacks, by incorporating specific assumptions on the adversary's data manipulation strategy. We focus on an efficient, wrapper-based implementation of our approach, and experimentally validate its soundness on different application examples, including spam and malware detection. PMID:25910268

  8. Why Does Asthma Attack Severely at Night?

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    @@ The rhythmic changes of human physiological activities by day and at night may cause regular change of the patient's condition within one day. The following is the explanation with the theory of Chinese medicine for the reason why asthma attacks severely at night.

  9. Rhode Island School Terrorist Attack Preparedness

    Science.gov (United States)

    Dube, Michael W. M.

    2012-01-01

    This study examined the state of safety and terrorist attack preparedness in Rhode Island Schools as determined by Rhode Island school leader perceptions. The study is descriptive in nature as it gathers data to describe a particular event or situation. Using a researcher generated survey based on terrorist preparedness guidelines and suggestions…

  10. Association between Terror Attacks and Suicide Attempts

    Science.gov (United States)

    Weizman, Tal; Yagil, Yaron; Schreiber, Shaul

    2009-01-01

    Based on Durkheim's "Control theory," we explored the association between frequency of terror attacks in Israel and the frequency of suicide attempts admitted to the Emergency Room of a major general hospital in Tel-Aviv (1999-2004). Analysis of the six-year study period as a whole revealed no significant correlation between the variables, with…

  11. After Heart Attack, New Threat: Heart Failure

    Science.gov (United States)

    ... nih.gov/medlineplus/news/fullstory_159007.html After Heart Attack, New Threat: Heart Failure 1 in 4 survivors develops this serious ... TUESDAY, May 24, 2016 (HealthDay News) -- Risk of heart failure appears high within a few years of ...

  12. Nonepileptic attack disorder among married women

    Directory of Open Access Journals (Sweden)

    Dhanaraj M

    2005-01-01

    Full Text Available Aim : To study the clinical features, precipitating stressful life events and prognosis of nonepileptic attack disorder (NEAD among married women. Design: Prospective cohort study with 1-year follow-up. Setting: A tertiary care teaching hospital. Subjects: Of the 1020 patients with epilepsy referred to the epilepsy clinic during 2002-2003, 30 were married women with NEAD. Materials and Methods: The diagnostic criteria for NEAD included normal EEG during ictal and post-ictal phase of the generalized ′attack.′ The data collected included clinical characteristics, semiology of the attacks, precipitating stressful events, and co-morbid psychiatric disorders. The control group included 30 age-matched married women with generalized tonic-clonic seizures. The long-term outcome and factors influencing the outcomes were analyzed. Results: The mean duration of illness was 18 months, and the pattern of the attack was ′fall and lying still′ in 53% and ′fall with generalized motor movements′ in 47%. The frequency was one or more per week in 57% and occasionally in 43%. The important stressful events were matrimonial discord following illegal relationship of the husband with another woman (c2 = 9.02, P = 0.003 and constant quarrel with other family members (c2 = 5.19, P = 0.02. The prevalence of sexual abuse was low (7%. Co-morbid psychiatric disorder was observed in 70%. At the end of 1 year, 39% were free from the attack. Resolution of the stressful life events (c2 = 4.52, P = 0.03 and lower frequency of attack at the time of reporting (c2 = 3.88, P = 0.05 correlated with good outcomes. Conclusion : Among patients with NEAD in India, the major precipitating factors were matrimonial discord following illegal relationship of the husband with another woman and constant quarrel with other family members and not sexual abuse. Women with low frequency of attack at the time of reporting and the remission of the stressful events had better outcomes.

  13. Assessing Terrorist Motivations for Attacking Critical Infrastructure

    Energy Technology Data Exchange (ETDEWEB)

    Ackerman, G; Abhayaratne, P; Bale, J; Bhattacharjee, A; Blair, C; Hansell, L; Jayne, A; Kosal, M; Lucas, S; Moran, K; Seroki, L; Vadlamudi, S

    2006-12-04

    Certain types of infrastructure--critical infrastructure (CI)--play vital roles in underpinning our economy, security and way of life. These complex and often interconnected systems have become so ubiquitous and essential to day-to-day life that they are easily taken for granted. Often it is only when the important services provided by such infrastructure are interrupted--when we lose easy access to electricity, health care, telecommunications, transportation or water, for example--that we are conscious of our great dependence on these networks and of the vulnerabilities that stem from such dependence. Unfortunately, it must be assumed that many terrorists are all too aware that CI facilities pose high-value targets that, if successfully attacked, have the potential to dramatically disrupt the normal rhythm of society, cause public fear and intimidation, and generate significant publicity. Indeed, revelations emerging at the time of this writing about Al Qaida's efforts to prepare for possible attacks on major financial facilities in New York, New Jersey, and the District of Columbia remind us just how real and immediate such threats to CI may be. Simply being aware that our nation's critical infrastructure presents terrorists with a plethora of targets, however, does little to mitigate the dangers of CI attacks. In order to prevent and preempt such terrorist acts, better understanding of the threats and vulnerabilities relating to critical infrastructure is required. The Center for Nonproliferation Studies (CNS) presents this document as both a contribution to the understanding of such threats and an initial effort at ''operationalizing'' its findings for use by analysts who work on issues of critical infrastructure protection. Specifically, this study focuses on a subsidiary aspect of CI threat assessment that has thus far remained largely unaddressed by contemporary terrorism research: the motivations and related factors that

  14. A graph based system for multi-stage attacks recognition

    Institute of Scientific and Technical Information of China (English)

    Safaa O. Al-Mamory; Zhai Jianhong; Zhang Hongli

    2008-01-01

    Building attack scenario is one of the most important aspects in network security. This paper proposed a system which collects intrusion alerts, clusters them as sub-attacks using alerts abstraction, aggregates the similar sub-attacks, and then correlates and generates correlation graphs. The scenarios were represented by alert classes instead of alerts themselves so as to reduce the required rules and have the ability of detecting new variations of attacks. The proposed system is capable of passing some of the missed attacks. To evaluate system effectiveness, it was tested with different datasets which contain multi-step attacks. Compressed and easily understandable correlation graphs which reflect attack scenarios were generated. The proposed system can correlate related alerts, uncover the attack strategies, and detect new variations of attacks.

  15. Recovery of human remains after shark attack.

    Science.gov (United States)

    Byard, Roger W; James, Ross A; Heath, Karen J

    2006-09-01

    Two cases of fatal shark attack are reported where the only tissues recovered were fragments of lung. Case 1: An 18-year-old male who was in the sea behind a boat was observed by friends to be taken by a great white shark (Carcharodon carcharias). The shark dragged him under the water and then, with a second shark, dismembered the body. Witnesses noted a large amount of blood and unrecognizable body parts coming to the surface. The only tissues recovered despite an intensive beach and sea search were 2 fragments of lung. Case 2: A 19-year-old male was attacked by a great white shark while diving. A witness saw the shark swim away with the victim's body in its mouth. Again, despite intensive beach and sea searches, the only tissue recovered was a single piece of lung, along with pieces of wetsuit and diving equipment. These cases indicate that the only tissue to escape being consumed or lost in fatal shark attacks, where there is a significant attack with dismemberment and disruption of the integrity of the body, may be lung. The buoyancy of aerated pulmonary tissue ensures that it rises quickly to the surface, where it may be recovered by searchers soon after the attack. Aeration of the lung would be in keeping with death from trauma rather than from drowning and may be a useful marker in unwitnessed deaths to separate ante- from postmortem injury, using only relatively small amounts of tissues. Early organ recovery enhances the identification of human tissues as the extent of morphologic alterations by putrefactive processes and sea scavengers will have been minimized. DNA testing is also possible on such recovered fragments, enabling confirmation of the identity of the victim. PMID:16936505

  16. Analysis of the SYN Flood DoS Attack

    Directory of Open Access Journals (Sweden)

    Mitko Bogdanoski

    2013-06-01

    Full Text Available The paper analyzes systems vulnerability targeted by TCP (Transmission Control Protocol segments when SYN flag is ON, which gives space for a DoS (Denial of Service attack called SYN flooding attack or more often referred as a SYN flood attack. The effects of this type of attack are analyzed and presented in OPNET simulation environment. Furthermore, the paper presents two anomaly detection algorithms as an effective mechanism against this type of attack. Finally, practical approaches against SYN flood attack for Linux and Windows environment which are followed by are shown.

  17. A Survey of Mobile Ad Hoc Network Attacks

    Directory of Open Access Journals (Sweden)

    PRADIP M. JAWANDHIYA,

    2010-09-01

    Full Text Available Security is an essential requirement in mobile ad hoc network (MANETs. Compared to wired networks, MANETs are more vulnerable to security attacks due to the lack of a trusted centralized authority and limited resources. Attacks on ad hoc networks can be classified as passive and active attacks, depending on whether the normal operation of the network is disrupted or not. In this paper, we are describing the all prominent attacks described in literature in a consistent manner to provide a concise comparison on attack types. To the best of our knowledge, this is the first paper that studies all the existing attacks on MANETs.

  18. Defense and attack of complex and dependent systems

    International Nuclear Information System (INIS)

    A framework is constructed for how to analyze the strategic defense of an infrastructure subject to attack by a strategic attacker. Merging operations research, reliability theory, and game theory for optimal analytical impact, the optimization program for the defender and attacker is specified. Targets can be in parallel, series, combined series-parallel, complex, k-out-of-n redundancy, independent, interdependent, and dependent. The defender and attacker determine how much to invest in defending versus attacking each of multiple targets. A target can have economic, human, and symbolic values, subjectively assessed by the defender and attacker. A contest success function determines the probability of a successful attack on each target, dependent on the investments by the defender and attacker into each target, and on characteristics of the contest. The defender minimizes the expected damage plus the defense costs. The attacker maximizes the expected damage minus the attack costs. Each agent is concerned about how his investments vary across the targets, and the impact on his utilities. Interdependent systems are analyzed where the defense and attack on one target impacts all targets. Dependent systems are analyzed applying Markov analysis and repeated games where a successful attack on one target in the first period impacts the unit costs of defense and attack, and the contest intensity, for the other target in the second period.

  19. Satellite Threat Warning and Attack Reporting

    Energy Technology Data Exchange (ETDEWEB)

    Hilland, D. [Kirkland AFB, NM (United States). Air Force Research Lab.; Phipps, G. [Sandia National Labs., Albuquerque, NM (United States). Optics & Technologies Dept.; Jingle, C.; Newton, G. [Schafer Corp., Albuquerque, NM (United States)

    1997-12-31

    The Air Force Research Laboratory`s Satellite Threat Warning and Attack Reporting (STW/AR) program will provide technologies for advanced threat warning and reporting of radio frequency (RF) and laser threats. The STW/AR program objectives are: (a) develop cost- effective technologies to detect, identify, locate, characterize, and report attacks or interference against U.S. and Allied satellites. (b) demonstrate innovative, light-weight, low-power, laser and RF sensors. The program focuses on the demonstration of RF and laser sensors. The RF sensor effort includes the investigation of interferometric antenna arrays, multi-arm spiral and butler matrix antennas, wideband receivers, adaptive processors, and improved processing algorithms. The laser sensor effort includes the investigation of alternative detectors, broadband grating and optical designs, active pixel sensing, and improved processing algorithms.

  20. GNSS-based positioning: Attacks and Countermeasures

    CERN Document Server

    Papadimitratos, P

    2010-01-01

    Increasing numbers of mobile computing devices, user-portable, or embedded in vehicles, cargo containers, or the physical space, need to be aware of their location in order to provide a wide range of commercial services. Most often, mobile devices obtain their own location with the help of Global Navigation Satellite Systems (GNSS), integrating, for example, a Global Positioning System (GPS) receiver. Nonetheless, an adversary can compromise location-aware applications by attacking the GNSS-based positioning: It can forge navigation messages and mislead the receiver into calculating a fake location. In this paper, we analyze this vulnerability and propose and evaluate the effectiveness of countermeasures. First, we consider replay attacks, which can be effective even in the presence of future cryptographic GNSS protection mechanisms. Then, we propose and analyze methods that allow GNSS receivers to detect the reception of signals generated by an adversary, and then reject fake locations calculated because of ...

  1. Two fatal tiger attacks in zoos.

    Science.gov (United States)

    Tantius, Britta; Wittschieber, Daniel; Schmidt, Sven; Rothschild, Markus A; Banaschak, Sibylle

    2016-01-01

    Two captive tiger attacks are presented that took place in Cologne and Münster zoos. Both attacks occurred when the handlers, intent on cleaning the enclosures, entered whilst the tigers accidently retained access to the location, and thus defended their territory against the perceived intruders. Both victims suffered fatal neck injuries from the bites. At Münster, colleagues managed to lure the tiger away from its victim to enable treatment, whilst the Cologne zoo tiger had to be shot in order to allow access to be gained. Whilst it was judged that human error led to the deaths of the experienced zookeepers, the investigation in Münster was closed as no third party was found to be at fault, whereas the Cologne zoo director was initially charged with being negligent. These charges were subsequently dismissed as safety regulations were found to be up to date. PMID:26115644

  2. Quantum Communication Attacks on Classical Cryptographic Protocols

    DEFF Research Database (Denmark)

    Damgård, Ivan Bjerre

    , one can show that the protocol remains secure even under such an attack. However, there are also cases where the honest players are quantum as well, even if the protocol uses classical communication. For instance, this is the case when classical multiparty computation is used as a “subroutine......” in quantum multiparty computation. Furthermore, in the future, players in a protocol may employ quantum computing simply to improve efficiency of their local computation, even if the communication is supposed to be classical. In such cases, it no longer seems clear that a quantum adversary must be limited......In the literature on cryptographic protocols, it has been studied several times what happens if a classical protocol is attacked by a quantum adversary. Usually, this is taken to mean that the adversary runs a quantum algorithm, but communicates classically with the honest players. In several cases...

  3. Mitigation of Malicious Attacks on Networks

    CERN Document Server

    Schneider, Christian M; Andrade, Jose S; Havlin, Shlomo; Herrmann, Hans J; 10.1073/pnas.1009440108

    2011-01-01

    Terrorist attacks on transportation networks have traumatized modern societies. With a single blast, it has become possible to paralyze airline traffic, electric power supply, ground transportation or Internet communication. How and at which cost can one restructure the network such that it will become more robust against a malicious attack? We introduce a unique measure for robustness and use it to devise a method to mitigate economically and efficiently this risk. We demonstrate its efficiency on the European electricity system and on the Internet as well as on complex networks models. We show that with small changes in the network structure (low cost) the robustness of diverse networks can be improved dramatically while their functionality remains unchanged. Our results are useful not only for improving significantly with low cost the robustness of existing infrastructures but also for designing economically robust network systems.

  4. Modelling Social-Technical Attacks with Timed Automata

    DEFF Research Database (Denmark)

    David, Nicolas; David, Alexandre; Hansen, Rene Rydhof;

    2015-01-01

    Attacks on a system often exploit vulnerabilities that arise from human behaviour or other human activity. Attacks of this type, so-called socio-technical attacks, cover everything from social engineering to insider attacks, and they can have a devastating impact on an unprepared organisation. In...... this paper we develop an approach towards modelling socio-technical systems in general and socio-technical attacks in particular, using timed automata and illustrate its application by a complex case study. Thanks to automated model checking and automata theory, we can automatically generate possible...... attacks in our model and perform analysis and simulation of both model and attack, revealing details about the specific interaction between attacker and victim. Using timed automata also allows for intuitive modelling of systems, in which quantities like time and cost can be easily added and analysed....

  5. Heart Attack Before 50 Ups Early Death Risk

    Science.gov (United States)

    ... 160692.html Heart Attack Before 50 Ups Early Death Risk But healthy living can improve those odds, ... 30, 2016 (HealthDay News) -- The risk of early death after a heart attack has lessened over the ...

  6. Nonlinear optical cryptosystem resistant to standard and hybrid attacks

    Science.gov (United States)

    Sinha, Aloka

    2016-06-01

    We propose a nonlinear optical cryptosystem that is resistant to amplitude-phase retrieval attacks, known-plaintext attack and chosen-plaintext attack. A squaring operation is introduced in the encryption path, which thwarts the iterative attacks. This nonlinear operation tends to amplify the error in the estimation during an iterative attack. The decryption process requires the use of a square-root operation. Thus, in the reverse path also, the attacks encounter the nonlinear square-root operation. These two nonlinearities make the iterative attacks unstable, thereby leading to non-convergence of the mean square error (MSE). Our technique is also resistant to hybrid attacks. The technique is general and is shown to work on a variety of images of the type grayscale and binary. Numerical simulation results corroborate the effectiveness of the proposed cryptosystem.

  7. Concrete Deterioration under Alternate Action of Carbonation and Sulfate Attack

    Directory of Open Access Journals (Sweden)

    Fenglan Li

    2013-11-01

    Full Text Available By micro- and macro-observations, the study reveals the deterioration mechanisms of concrete under alternate action of carbonation and sodium sulfate solution attack (C-SA comparied with the single sodium sulfate solution attack (SA. The studies indicate that in both cases, the main attack product is ettringite, some gypsum is checked only in the first layer of case SA, the thaumasite form of sulfate attack is not initiated in case C-SA. Compared with the single sodium sulfate solution attack, although the carbonation layer mitigates the extent of sulfate attack in limited exposure period, the alternate action of carbonation and sulfate attack still leads to strength degradation to some extent. The effect of carbonation on sulfate attack for longer exposure period deserves further research.

  8. Sex Suffers for Younger Adults After Heart Attack

    Science.gov (United States)

    ... gov/news/fullstory_160722.html Sex Suffers for Younger Adults After Heart Attack Lack of interest a ... 2016 (HealthDay News) -- After a heart attack, many younger adults experience sexual difficulties -- and women more so ...

  9. Heart Attack Help? There's an App for That

    Science.gov (United States)

    ... page: https://medlineplus.gov/news/fullstory_159504.html Heart Attack Help? There's an App for That Emergency medicine ... American Heart Association. When someone is suffering a heart attack, stroke or cardiac arrest, quick action can be ...

  10. Extensions of the Cube Attack Based on Low Degree Annihilators

    Science.gov (United States)

    Zhang, Aileen; Lim, Chu-Wee; Khoo, Khoongming; Wei, Lei; Pieprzyk, Josef

    At Crypto 2008, Shamir introduced a new algebraic attack called the cube attack, which allows us to solve black-box polynomials if we are able to tweak the inputs by varying an initialization vector. In a stream cipher setting where the filter function is known, we can extend it to the cube attack with annihilators: By applying the cube attack to Boolean functions for which we can find low-degree multiples (equivalently annihilators), the attack complexity can be improved. When the size of the filter function is smaller than the LFSR, we can improve the attack complexity further by considering a sliding window version of the cube attack with annihilators. Finally, we extend the cube attack to vectorial Boolean functions by finding implicit relations with low-degree polynomials.

  11. Heart Attack: Tips for Recovering and Staying Well

    Science.gov (United States)

    ... version Heart Attack | Tips for Recovering and Staying Well If you have had a heart attack, you ... healthy diet. The food you eat affects how well your blood flows through your heart and arteries. ...

  12. Robustness of Complex Networks against Attacks Guided by Damage

    CERN Document Server

    Wang, Hui; Xu, Xiaomin; Xiao, Yanghua; Wang, Wei

    2011-01-01

    Extensive researches have been dedicated to investigating the performance of real networks and synthetic networks against random failures or intentional attack guided by degree (degree attack). Degree is one of straightforward measures to characterize the vitality of a vertex in maintaining the integrity of the network but not the only one. Damage, the decrease of the largest component size that was caused by the removal of a vertex, intuitively is a more destructive guide for intentional attack on networks since the network functionality is usually measured by the largest component size. However, it is surprising to find that little is known about behaviors of real networks or synthetic networks against intentional attack guided by damage (damage attack), in which adversaries always choose the vertex with the largest damage to attack. In this article, we dedicate our efforts to understanding damage attack and behaviors of real networks as well as synthetic networks against this attack. To this end, existing ...

  13. Securing iris recognition systems against masquerade attacks

    OpenAIRE

    Galbally Herrero, Javier; Gómez-Barrero, Marta; Ross, Arun A.; Fiérrez, Julián; Ortega-García, Javier

    2013-01-01

    Javier Galbally ; Marta Gomez-Barrero ; Arun Ross ; Julian Fierrez ; Javier Ortega-Garcia; "Securing iris recognition systems against masquerade attacks", Biometric and Surveillance Technology for Human and Activity Identification X, Proc. SPIE 8712 (May 31, 2013); doi:10.1117/12.2015690. Copyright 2013 Society of Photo‑Optical Instrumentation Engineers. One print or electronic copy may be made for personal use only. Systematic reproduction and distribution, duplication of any material in...

  14. Incidence of Fungal attack on Aircraft Fuselage

    Directory of Open Access Journals (Sweden)

    H. M. Dayal

    1968-10-01

    Full Text Available Incidence of fungal attack on the fuselage of a few Vampire aircraft has been observed. The fungus isolated from the infected regions has been tentatively indentified as TorulaSp. Laboratory experiments have revealed that within four weeks this fungus causes about 44 percent loss in the tensile strength of the brich plywood used in the manufacture of the fuselage of the aircraft.

  15. Transient ischemic attack after Mesobuthus gibbosus envenomation

    OpenAIRE

    Golcuk, Yalcin; Ozsarac, Murat; Bilge, Adnan; Golcuk, Burcu; Cinar, Bilge Piri; Hayran, Mustafa

    2014-01-01

    Endometriosis Mesobuthus gibbosus is a medically important venomous scorpion in western of Turkey which causes significant morbidity. Envenoming of this species scorpion results with varying degrees of local symptoms and clinically important systemic effects. This case describes an authenticated first case of transient ischemic attack in a 68 year-old-woman following envenoming by M. gibbosus in Turkey.Keywords: Scorpion, Envenomation, Emergency department

  16. Visualizing Endpoint Security Technologies using Attack Trees

    OpenAIRE

    Pettersson , Stefan

    2008-01-01

    Software vulnerabilities in programs and malware deployments have been increasing almost every year since we started measuring them. Information about how to program securely, how malware shall be avoided and technological countermeasures for this are more available than ever. Still, the trend seems to favor the attacker. This thesis tries to visualize the effects of a selection of technological countermeasures that have been proposed by researchers. These countermeasures: non-executable memo...

  17. Towards a translational model of panic attacks

    OpenAIRE

    Luiz Carlos Schenberg

    2010-01-01

    About 20 years ago, Deakin and Graeff proposed that whereas generalized anxiety disorder is produced by the overactivity of 5-HT excitatory projections from dorsal raphe nucleus to the areas of prefrontal cortex and amygdala which process distal threat, panic attacks are a dysfunction of 5-HT inhibitory projections from dorsal raphe nucleus to the dorsal periaqueductal gray matter, thereby releasing the responses to proximal threat, innate fear or anoxia. Besides, they suggested that the decr...

  18. Sharkonomics How to attack market leaders

    CERN Document Server

    Engeseth, Stefan

    2012-01-01

    Sharks are nature's most revered and feared killing machines. But if you study the behaviour of sharks, you will learn they are also highly strategic and efficient in the way they survive and thrive in nature's competitive environment. Inspired by the shark's evolved (over 420 million years) instincts and strategic moves, this book provides businesses with 10 ways on how to attack the market leaders, and take market share, in your sector.

  19. An Analysis of Various Attacks in MANET

    OpenAIRE

    M.Udhayamoorthi; C. Senthilkumar; Dr S. Karthik; Dr. T.Kalaikumaran

    2014-01-01

    An Ad-hoc network is a self-organized network, without a central coordinator, and which frequently changes its topology. According to the perspective of our paper, we try to connect the current status of computers era to adhoc networking. As adhoc networks has been unleashing several updated technologies, it is the most growing area in the field of networks in information and communication Engineering has ever seen .we start with area introduction, deeply reviewed about the various attacks es...

  20. Attacks and Counterattacks on Wireless Sensor Networks

    Directory of Open Access Journals (Sweden)

    Nusrat Fatema

    2013-12-01

    Full Text Available WSN is formed by autonomous nodes with partial memo ry, communication range, power, and bandwidth. Their occupation depends on inspecting corporal and environmental conditions and communing through a system and performing data processing. The applicat ion field is vast, comprising military, ecology, healthcare, home or commercial and require a highly secured communication. The paper analyses different types of attacks and counterattacks and provides so lutions for the WSN threats.

  1. Quantum Key Distribution against Trojan Horse Attacks

    Institute of Scientific and Technical Information of China (English)

    CAI Qing-Yu; LV Hua

    2007-01-01

    Realistic experimental apparatus of quantum cryptography are imperfect, which may be utilized by a potential eavesdropper to eavesdrop on the communication. We show that quantum communication may be improved with quantum teleportation and entanglement swapping, which is robustly secure against the most general Trojan horse attacks. Our scheme is not an improvement of the communication apparatus, but the improvement of quantum communication protocol itself. We show that our modified schemes may be implemented with current technology.

  2. Nonepileptic attack disorder among married women

    OpenAIRE

    Dhanaraj M; Rangaraj R; Arulmozhi T; Vengatesan A

    2005-01-01

    Aim : To study the clinical features, precipitating stressful life events and prognosis of nonepileptic attack disorder (NEAD) among married women. Design: Prospective cohort study with 1-year follow-up. Setting: A tertiary care teaching hospital. Subjects: Of the 1020 patients with epilepsy referred to the epilepsy clinic during 2002-2003, 30 were married women with NEAD. Materials and Methods: The diagnostic criteria for NEAD included normal EEG during ictal and post-ictal phase of the...

  3. Securing iris recognition systems against masquerade attacks

    Science.gov (United States)

    Galbally, Javier; Gomez-Barrero, Marta; Ross, Arun; Fierrez, Julian; Ortega-Garcia, Javier

    2013-05-01

    A novel two-stage protection scheme for automatic iris recognition systems against masquerade attacks carried out with synthetically reconstructed iris images is presented. The method uses different characteristics of real iris images to differentiate them from the synthetic ones, thereby addressing important security flaws detected in state-of-the-art commercial systems. Experiments are carried out on the publicly available Biosecure Database and demonstrate the efficacy of the proposed security enhancing approach.

  4. NETWORK SECURITY: ATTACKS, TOOLS AND TECHNIQUES

    OpenAIRE

    Mhatre Suvidnya Shashikant; Potadar Devangi; Salvi Bhageshree

    2015-01-01

    Network security is main issue of this generation of computing because many types of attacks are increasing day by day. Establishing a network is not a big issue for network admini st rators but protecting the entire network is a big issue. There are various methods and tools are available today for destroying the existing network. In this paper we mainly emphasize on the network security also we present some major issues that can affect our network.

  5. Attack Detection By Clustering And Classification Approach

    Directory of Open Access Journals (Sweden)

    Priyanka J. Pathak, Prof. Snehlata Dongre

    2012-04-01

    Full Text Available Intrusion detection is a software application that monitors network and/or system activities for malicious activities or policy violations and produces reports to a Management Station. Security is becoming big issue for all networks. Hackers and intruders have made many successful attempts to bring down high profile company networks and web services. Intrusion Detection System (IDS is an important detection that is used as a countermeasure to preserve data integrity and system availability from attacks. The work is implemented in two phases, in first phase clustering by K-means is done and in next step of classification is done with k-nearest neighbours and decision trees. The objects are clustered or grouped based on the principle of maximizing the intra-class similarity and minimizing the interclass similarity. This paper proposes an approach which make the clusters of similar attacks and in next step of classification with K nearest neighbours and Decision trees it detect the attack types. This method is advantageous over single classifier as it detect better class than single classifier system.

  6. A Secure Localization Approach against Wormhole Attacks Using Distance Consistency

    OpenAIRE

    Honglong Chen; Wei Lou; Xice Sun; Zhi Wang

    2010-01-01

    Wormhole attacks can negatively affect the localization in wireless sensor networks. A typical wormhole attack can be launched by two colluding attackers, one of which sniffs packets at one point in the network and tunnels them through a wired or wireless link to another point, and the other relays them within its vicinity. In this paper, we investigate the impact of the wormhole attack on the localization and propose a novel distance-consistency-based secure localization scheme against worm...

  7. A Structured Multi-signature Scheme Against Forgery Attack

    Directory of Open Access Journals (Sweden)

    Wenjun Luo

    2011-12-01

    Full Text Available There are some classic structured multi-signature programs, such as Burmester’s, Harn’s and Lin’s schemes that can not resist inside attack and outside attack. In this paper, we briefly review Burmester’s program and relate safety analysis, Burmester’s scheme vulnerable to forgery attack. Then we propose a structured multi-signature scheme against forgery attack. In the new scheme, we increase the signature parameter verification to improve security.

  8. Migraineurs with exercise-triggered attacks have a distinct migraine

    OpenAIRE

    Koppen, Hille; van Veldhoven, Peter LJ

    2013-01-01

    Background Sport as a migraine trigger has been reported, but extensive information on these triggered attacks and the patients experiencing these attacks is lacking. Goal of this study was to investigate the lifetime prevalence of exercise triggered migraine attacks in migraine patients and if patients with exercise triggered attacks experience specific prodromal or ictal migraine symptoms. Methods 103 consecutive migraine patients seen during their first visit at a Dutch headache clinic wer...

  9. Machine Learning Methods for Attack Detection in the Smart Grid

    OpenAIRE

    Ozay, Mete; Esnaola, Inaki; Vural, Fatos T. Yarman; Kulkarni, Sanjeev R.; Poor, H. Vincent

    2015-01-01

    Attack detection problems in the smart grid are posed as statistical learning problems for different attack scenarios in which the measurements are observed in batch or online settings. In this approach, machine learning algorithms are used to classify measurements as being either secure or attacked. An attack detection framework is provided to exploit any available prior knowledge about the system and surmount constraints arising from the sparse structure of the problem in the proposed appro...

  10. Detecting man-in-the-middle attacks by precise timing

    OpenAIRE

    Aziz, Benjamin; Hamilton, G.

    2009-01-01

    Man-in-the-middle attacks are one of the most popular and fundamental attacks on distributed systems that have evolved with advances in distributed computing technologies and have assumed several shapes ranging from simple IP spoofing to complicated attacks on wireless communications, which have safety-critical applications such as remote wireless passport verification. This paper proposes a static analysis algorithm for the detection of man-in-the-middle attacks in mobile processes using a s...

  11. A Formal Classification of Internet Banking Attacks and Vulnerabilities

    Directory of Open Access Journals (Sweden)

    Laerte Peotta

    2011-02-01

    Full Text Available A formal classification of attacks and vulnerabilities that affect current internet banking systems is presented along with two attacks which demonstrate the insecurity of such systems. Based ona thoroughanalysis of current security models, we propose a guidelines for designing secure internet banking systems which are not affected by the presented attacks and vulnerabilities.

  12. Attacks Prevention and Detection Techniques In MANET: A Survey

    Directory of Open Access Journals (Sweden)

    Pranjali D. Nikam,

    2014-11-01

    Full Text Available Wireless sensor network is a set of distributed sensor nodes. Which are randomly deployed in geographical area to capture climatic changes like temperature, humidity and pressure. In Wireless Network MANET is a Mobile Ad-Hoc Networks which is a one self-configurable network. MANET is a collection of Wireless mobile node which is dynamically moves from one location to another location. Both attacks Active as well as Passive attacks is in MANET. It doesn’t have a static structure. Security for wireless network is much difficult as compare to wired networks. In last few years many security and attacks issue are face many researchers in MANET. Attacks like Packet dropping attack, Black-Hole attack, Denial of Service attack, wormhole attacks and Packet modification attacks found in MANET. At the time of data communication all the above mentioned attacks access data easily without permission. To solve the problem of attacks in MANET and secure data communication use Intrusion Detection System. In This paper propose the survey of different kinds of attacks on MANET and Wireless sensor networks. This paper helps to young researcher for implement new hybrid algorithm for secure intrusion detection in MANET.

  13. A Review of Man-in-the-Middle Attacks

    OpenAIRE

    Gangan, Subodh

    2015-01-01

    This paper presents a survey of man-in-the-middle (MIM) attacks in communication networks and methods of protection against them. In real time communication, the attack can in many situations be discovered by the use of timing information. The most common attacks occur due to Address Resolution Protocol (ARP) cache poisoning, DNS spoofing, session hijacking, and SSL hijacking.

  14. Vulnerability Assessment by Learning Attack Specifications in Graphs

    OpenAIRE

    Franqueira, Virginia N L; Lopes, Raul H. C.

    2007-01-01

    This paper presents an evolutionary approach for learning attack specifications that describe attack scenarios. The objective is to find vulnerabilities in computer networks which minimise the cost of an attack with maximum impact. Although we focus on Insider Threat, the proposed approach applies to networks in general, including social networks and computer grid.

  15. Assessing Terrorist Motivations for Attacking Critical Infrastructure

    Energy Technology Data Exchange (ETDEWEB)

    Ackerman, G; Abhayaratne, P; Bale, J; Bhattacharjee, A; Blair, C; Hansell, L; Jayne, A; Kosal, M; Lucas, S; Moran, K; Seroki, L; Vadlamudi, S

    2006-12-04

    Certain types of infrastructure--critical infrastructure (CI)--play vital roles in underpinning our economy, security and way of life. These complex and often interconnected systems have become so ubiquitous and essential to day-to-day life that they are easily taken for granted. Often it is only when the important services provided by such infrastructure are interrupted--when we lose easy access to electricity, health care, telecommunications, transportation or water, for example--that we are conscious of our great dependence on these networks and of the vulnerabilities that stem from such dependence. Unfortunately, it must be assumed that many terrorists are all too aware that CI facilities pose high-value targets that, if successfully attacked, have the potential to dramatically disrupt the normal rhythm of society, cause public fear and intimidation, and generate significant publicity. Indeed, revelations emerging at the time of this writing about Al Qaida's efforts to prepare for possible attacks on major financial facilities in New York, New Jersey, and the District of Columbia remind us just how real and immediate such threats to CI may be. Simply being aware that our nation's critical infrastructure presents terrorists with a plethora of targets, however, does little to mitigate the dangers of CI attacks. In order to prevent and preempt such terrorist acts, better understanding of the threats and vulnerabilities relating to critical infrastructure is required. The Center for Nonproliferation Studies (CNS) presents this document as both a contribution to the understanding of such threats and an initial effort at ''operationalizing'' its findings for use by analysts who work on issues of critical infrastructure protection. Specifically, this study focuses on a subsidiary aspect of CI threat assessment that has thus far remained largely unaddressed by contemporary terrorism research: the motivations and related factors that

  16. Unified communications forensics anatomy of common UC attacks

    CERN Document Server

    Grant, Nicholas Mr

    2013-01-01

    Unified Communications Forensics: Anatomy of Common UC Attacks is the first book to explain the issues and vulnerabilities and demonstrate the attacks, forensic artifacts, and countermeasures required to establish a secure (UC) environment. This book is written by leading UC experts Nicholas Grant and Joseph W. Shaw II and provides material never before found on the market, including: analysis of forensic artifacts in common UC attacks an in-depth look at established UC technologies and attack exploits hands-on understanding of UC attack vectors and associated countermeasures

  17. Machine Learning Methods for Attack Detection in the Smart Grid.

    Science.gov (United States)

    Ozay, Mete; Esnaola, Inaki; Yarman Vural, Fatos Tunay; Kulkarni, Sanjeev R; Poor, H Vincent

    2016-08-01

    Attack detection problems in the smart grid are posed as statistical learning problems for different attack scenarios in which the measurements are observed in batch or online settings. In this approach, machine learning algorithms are used to classify measurements as being either secure or attacked. An attack detection framework is provided to exploit any available prior knowledge about the system and surmount constraints arising from the sparse structure of the problem in the proposed approach. Well-known batch and online learning algorithms (supervised and semisupervised) are employed with decision- and feature-level fusion to model the attack detection problem. The relationships between statistical and geometric properties of attack vectors employed in the attack scenarios and learning algorithms are analyzed to detect unobservable attacks using statistical learning methods. The proposed algorithms are examined on various IEEE test systems. Experimental analyses show that machine learning algorithms can detect attacks with performances higher than attack detection algorithms that employ state vector estimation methods in the proposed attack detection framework. PMID:25807571

  18. PERFORMANCE EVALUATION OF WIRELESS SENSOR NETWORK UNDER HELLO FLOOD ATTACK

    Directory of Open Access Journals (Sweden)

    Mohammad Abdus Salam

    2016-03-01

    Full Text Available Wireless sensor network (WSN is highly used in many fields. The network consists of tiny lightweight sensor nodes and is largely used to scan or detect or monitor environments. Since these sensor nodes are tiny and lightweight, they put some limitations on resources such as usage of power, processing given task, radio frequency range. These limitations allow network vulnerable to many different types of attacks such as hello flood attack, black hole, Sybil attack, sinkhole, and many more. Among these attacks, hello flood is one of the most important attacks. In this paper,we have analyzed the performance of hello flood attack and compared the network performance as number of attackers increases. Network performance is evaluated by modifying the ad-hoc on demand distance vector (AODV routing protocol by using NS2 simulator. It has been tested under different scenarios like no attacker, single attacker, and multiple attackers to know how the network performance changes. The simulation results show that as the number of attackers increases the performance in terms of throughput and delay changes.

  19. WIRELESS AD-HOC NETWORK UNDER BLACK-HOLE ATTACK

    Directory of Open Access Journals (Sweden)

    Shree Om

    2011-01-01

    Full Text Available Wireless Ad-hoc Network is a temporary and decentralized type of wireless network. Due to security vulnerabilities in the routing protocol currently, this type of network is unprotected to network layer attacks. Black-hole attack is such a type of attack and is a Denial-of-Service (DoS attack. Due to its nature, the attack makes the source node send all the data packets to a Black-hole node that ends up dropping all the packets. The aim of this paper is to reflect light on the severe effects of a Black-hole attack in a Wireless Ad-hoc network and the drawbacks of the security mechanisms being used for the mitigation of this attack.

  20. Attack Graph-Based Risk Assessment and Optimisation Approach

    Directory of Open Access Journals (Sweden)

    Mohammed Alhomidi

    2014-06-01

    Full Text Available Attack graphs are models that offer significant capabilities to analyse security in network systems. An attack graph allows the representation of vulnerabilities, exploits and conditions for each attack in a single unifying model. This paper proposes a methodology to explore the graph using a genetic algorithm (GA. Each attack path is considered as an independent attack scenario from the source of attack to the target. Many such paths form the individuals in the evolutionary GA solution. The population-based strategy of a GA provides a natural way of exploring a large number of possible attack paths to find the paths that are most important. Thus unlike many other optimization solutions a range of solutions can be presented to a user of the methodology.