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Sample records for angioedema

  1. Angioedema

    Directory of Open Access Journals (Sweden)

    Luisa María Holguín-Gómez

    2016-10-01

    Full Text Available Angioedema is defined as edema of the skin or mucosa, including the respiratory and the gastrointestinal mucosa, which is self-limiting, and in most cases is completely resolved in less than 72 hours. It occurs due to increased permeability of the mucosal and submucosal capillaries and postcapillary venules, with resulting plasma extravasation. There are different types of angioedema: histaminergic (which may be mediated by immunoglobulin E, hereditary, from acquired C1 inhibitor deficiency, from angiotensin converting enzyme inhibitor, bradykinin-mediated, and non-histaminergic idiopathic angioedema. Treatment depends on the cause of angioedema, age, and the frequency and severity of manifestations. The main measures are avoiding external triggers or causes, giving antihistamines, steroids, or adrenaline for histaminergic angioedema; replacing the deficient protein or blocking the action of bradykinin in C1 inhibitor deficiency and angioedema from angiotensin converting enzyme inhibitor.

  2. Hereditary angioedema

    Science.gov (United States)

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

  3. Hereditary angioedema.

    Science.gov (United States)

    Bracho, Francisco A

    2005-11-01

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

  4. Hives and Angioedema

    Science.gov (United States)

    ... is swelling or if you're having trouble breathing. Causes Hives and angioedema can be caused by: Foods. Many foods can trigger reactions in people with sensitivities. Shellfish, fish, peanuts, tree nuts, eggs and milk are frequent ...

  5. 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 r...... of recurrent skin swellings and abdominal pain leading to several hospital admissions. The aim of this report is to direct focus on this rare disease, which can be treated effectively, to diminish morbidity and mortality of children suffering from undiagnosed HAE....

  6. Trifluoperazine-Induced Angioedema

    Directory of Open Access Journals (Sweden)

    Mugtaba Osman

    2014-01-01

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

  7. 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.

  8. Recent Advances in Drug-Induced Angioedema

    Directory of Open Access Journals (Sweden)

    Naoko Inomata

    2012-01-01

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

  9. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    DEFF Research Database (Denmark)

    Andersen, Michelle Fog; Bygum, Anette

    2015-01-01

    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...

  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 p......-sensitive phenotype for some patients. CONCLUSION: The course of angioedema in women with C1 inhibitor deficiency is affected by physiologic hormonal changes; consequently, physicians should take these into account when advising on management.......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. Prophylactic Therapy for Hereditary Angioedema.

    Science.gov (United States)

    Longhurst, Hilary; Zinser, Emily

    2017-08-01

    Long-term prophylaxis is needed in many patients with hereditary angioedema and poses many challenges. Attenuated androgens are effective in many but are limited by side effect profiles. There is less evidence for efficacy of tranexamic acid and progestagens; however, the small side effect profile makes tranexamic acid an option for prophylaxis in children and progestagens an option for women. C1 inhibitor is beneficial, but at present requires intravenous delivery and may need dose titration for maximum efficacy. Short-term prophylaxis should be considered for all procedures. New therapies are promising in overcoming many problems encountered with current options for long-term prophylaxis. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. Angioedema

    Science.gov (United States)

    ... cold or heat Foods (such as berries, shellfish, fish, nuts, eggs, and milk) Insect bites Medicines ( drug ... be painful. Other symptoms may include: Abdominal cramping Breathing difficulty Swollen eyes and mouth Swollen lining of ...

  13. 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.

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

    DEFF Research Database (Denmark)

    Cicardi, M; Aberer, W; Banerji, A

    2014-01-01

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

  15. 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...

  16. Epidemiology of Non-hereditary Angioedema

    DEFF Research Database (Denmark)

    Madsen, Flemming; Attermann, Jørn; Linneberg, Allan

    2012-01-01

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

  17. 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.

  18. An ABC of the Warning Signs of Hereditary Angioedema

    DEFF Research Database (Denmark)

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

    2017-01-01

    Hereditary angioedema (HAE) with C1 inhibitor deficiency is a genetic disorder that clinically manifests with attacks of angioedema in the subcutaneous and submucosal tissues, mainly in the extremities, abdomen, and upper airway. During attacks, vascular permeability is increased due to increased...

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

    OpenAIRE

    van den Elzen, M.T.

    2017-01-01

    The occurrence of wheals, angioedema or both for at least 6 weeks is diagnosed as chronic spontaneous urticaria in (inter) national guidelines - after excluding other illnesses. The underlying mechanism of angioedema without wheals is not entirely known. The objective of this thesis is to increase insight in the treatment of adult patients with angioedema with or without urticaria, and with urticaria with or without angioedema. We first studied patients with angioedema: we show that bradykini...

  20. 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.

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

    Science.gov (United States)

    Giavina-Bianchi, Pedro; França, Alfeu T; Grumach, Anete S; Motta, Abílio A; Fernandes, Fátima R; Campos, Regis A; Valle, Solange O; Rosário, Nelson A; Sole, Dirceu

    2011-01-01

    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.

  2. 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.

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

    Directory of Open Access Journals (Sweden)

    Rajeev Seecheran

    2017-01-01

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

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

    Science.gov (United States)

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

    2017-06-01

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

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

    Science.gov (United States)

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

    2017-04-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

  7. Acute dystonic reaction leading to lingual hematoma mimicking angioedema

    Science.gov (United States)

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

    2017-01-01

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

  8. Inhalant abuse of computer cleaner manifested as angioedema.

    Science.gov (United States)

    Kurniali, Peter C; Henry, Letitia; Kurl, Rita; Meharg, Joseph V

    2012-01-01

    Inhalant abuse is the intentional inhalation of chemical vapors or volatile substance to achieve a euphoric effect. Although no statistical data are reported yet, inhalant abuse is potentially life-threatening and has resulted in a wide range of toxic effects such as central nervous system depression, seizures, aspiration, cardiac arrhythmia, asphyxiation, hypoxia, metabolic acidosis, and sudden death among others. We are reporting a 25-year-old white man who was brought to the emergency department after inhaling aerosolized computer-cleaning spray composed of difluoroethane. He was found to have marked upper and lower lip facial swelling consistent with angioedema. The patient also had a prolonged QT interval, mild inspiratory stridor, but no urticaria. In this case, we believe the difluoroethane-related angioedema represents either idiopathic or bradykinin-induced angioedema.

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

    DEFF Research Database (Denmark)

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

    2017-01-01

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

  10. How Not to Be Misled by Disorders Mimicking Angioedema

    DEFF Research Database (Denmark)

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

    2016-01-01

    BACKGROUND: Angioedema is a vascular reaction involving the lower dermis, subcutis and/or submucosal tissue and causing a temporary localized swelling in any part of the body. For many health care professionals, the diagnosis presents an ongoing challenge; several disorders may manifest with subc...

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

    African Journals Online (AJOL)

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

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

    DEFF Research Database (Denmark)

    Bygum, Anette

    2014-01-01

    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...

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

    African Journals Online (AJOL)

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

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

    African Journals Online (AJOL)

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

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

    Science.gov (United States)

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

    2010-08-05

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

  16. Brazilian Guidelines for Hereditary Angioedema Management - 2017 Update Part 1: Definition, Classification and Diagnosis.

    Science.gov (United States)

    Giavina-Bianchi, Pedro; Arruda, Luisa Karla; Aun, Marcelo V; Campos, Regis A; Chong-Neto, Herberto J; Constantino-Silva, Rosemeire N; Fernandes, Fátima R; Ferraro, Maria F; Ferriani, Mariana P L; França, Alfeu T; Fusaro, Gustavo; Garcia, Juliana F B; Komninakis, Shirley; Maia, Luana S M; Mansour, Eli; Moreno, Adriana S; Motta, Antonio A; Pesquero, João B; Portilho, Nathalia; Rosário, Nelson A; Serpa, Faradiba S; Solé, Dirceu; Takejima, Priscila; Toledo, Eliana; Valle, Solange O.R; Veronez, Camila L; Grumach, Anete S

    2018-01-01

    Hereditary angioedema is an autosomal dominant disease characterized by recurrent angioedema attacks with the involvement of multiple organs. The disease is unknown to many health professionals and is therefore underdiagnosed. Patients who are not adequately diagnosed and treated have an estimated mortality rate ranging from 25% to 40% due to asphyxiation by laryngeal angioedema. Intestinal angioedema is another important and incapacitating presentation that may be the main or only manifestation during an attack. In this article, a group of experts from the "Associação Brasileira de Alergia e Imunologia (ASBAI)" and the "Grupo de Estudos Brasileiro em Angioedema Hereditário (GEBRAEH)" has updated the Brazilian guidelines for the diagnosis and treatment of hereditary angioedema.

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

    Science.gov (United States)

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

    2014-07-04

    Hereditary angioedema (HAE) due to C1 inhibitor deficiency is a rare but serious and potentially life-threatening disease marked by spontaneous, recurrent attacks of swelling. The study objective was to characterize direct and indirect resource utilization associated with HAE from the patient perspective in Europe. The study was conducted in Spain, Germany, and Denmark to assess the real-world experience of HAE via a cross-sectional survey of HAE patients, including direct and indirect resource utilization during and between attacks for patients and their caregivers over the past 6 months. A regression model examined predictors of medical resource utilization. Overall, 164 patients had an attack in the past 6 months and were included in the analysis. The most significant predictor of medical resource utilization was the severity of the last attack (OR 2.6; p career/educational advancement. HAE poses a considerable burden on patients and their families in terms of direct medical costs and indirect costs related to lost productivity. This burden is substantial at the time of attacks and in between attacks.

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

    Directory of Open Access Journals (Sweden)

    Kélem de Nardi Chagas

    2004-09-01

    Full Text Available OBJETIVOS: A primeira descrição clínica completa do angioedema hereditário (HAE foi relatada por William Osler, em 1888. As formas de angioedema com deficiência de C1-INH são divididas em hereditárias e adquiridas. A terapêutica pode ser direcionada aos ataques agudos ou profilaxia de novos episódios. O tratamento de escolha é feito através de hormônios masculinizantes, podendo também ser indicado os inibidores da ativação do cininogênio e do plasminogênio como o ácido tranexâmico ou o ácido e-aminocapróico e a reposição de concentrado de C1-INH. O presente estudo relata a evolução de 10 pacientes (quatro famílias acometidos por HAE e as peculiaridades do tratamento utilizado em cada caso. MÉTODOS: Dez pacientes (1-38 anos com HAE foram diagnosticados através de história clínica e exames laboratoriais. Os testes realizados para avaliação do sistema complemento foram: dosagem sérica de C1-INH, C4 e C3 e ensaio hemolítico (CH50 e APH50 para as vias clássica e alternativa. O tratamento foi indicado de acordo com a gravidade dos sintomas, idade, sexo e resposta terapêutica. RESULTADOS: A avaliação clínica evidenciou 4/10 pacientes com edema subcutâneo recorrente; 3/10 pacientes com edema de laringe prévio e 3/10 pacientes com sintomas esporádicos. Sintomas de gravidade diferentes foram evidenciados na mesma família. A avaliação laboratorial (dosagem sérica demonstrou: níveis de C1-INH diminuídos em 10/10; níveis diminuídos de C4 8/10; níveis indetectáveis de CH50 em 3/10 e diminuídos em 6/10; níveis diminuídos de APH50 em 2/10. 6/10 pacientes não receberam tratamento específico, sendo que dois deles apresentam alto risco para asfixia; uma adolescente tem sido controlada com ácido e-aminocapróico, uma criança que fazia uso de danazol passou a receber ácido tranexâmico, uma paciente de 30 anos recebe oxandrolona e um homem de 38 anos está em tratamento com danazol. CONCLUSÃO: Apesar do

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

    Science.gov (United States)

    Lumry, William Raymond

    2018-01-01

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

  20. Angioedema recorrente – caso clínico

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    Sandrina Martins

    2016-02-01

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

  1. Caffeine as a cause of urticaria-angioedema

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    Linda Tognetti

    2014-01-01

    Full Text Available We report the case of a young woman presenting with recurrent urticaria. The episodes occurred both in and out of the workplace. On three occasions it presented as urticaria-angioedema, requiring emergency care on one occassion. A thorough clinical history along with serological and allergological tests allowed a diagnosis of caffeine-induced urticaria-angioedema. We advised the patient to follow a caffeine-free diet and to avoid all caffeine or methylxanthine-containing drugs. After two years of caffeine abstinence, she had not experienced any further episodes of urticaria-angioedema. Only a few cases of caffeine-induced urticaria and/or anaphylaxis have been reported till date, with varying outcomes in allergologic investigations. Moreover, several cases are probably undiagnosed or misdiagnosed as idiopathic urticaria or as occupational allergy. We speculate that hypersensitivity to caffeine rather than autoimmine reaction may be the probable cause of urticaria. Caffeine should considered as a potential urticaria-inducing agent and should be included in the allergological test series.

  2. Functional C1-inhibitor diagnostics in hereditary angioedema: Assay evaluation and recommendations

    NARCIS (Netherlands)

    Wagenaar-Bos, Ineke G. A.; Drouet, Christian; Aygoeren-Pursun, Emel; Bork, Konrad; Bucher, Christoph; Bygum, Anette; Farkas, Henriette; Fust, George; Gregorek, Hanna; Hack, C. Erik; Hickey, Alaco; Joller-Jemelka, Helen I.; Kapusta, Maria; Kreuz, Wolfhart; Longhurst, Hilary; Lopez-Trascasa, Margarita; Madalinski, Kazimierz; Naskalski, Jerzy; Nieuwenhuys, Ed; Ponard, Denise; Truedsson, Lennart; Varga, Lilian; Nielsen, Erik Waage; Wagner, Eric; Zingale, Lorenza; Cicardi, Marco; van Ham, S. Marieke

    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 Cl esterase inhibitor (C1-Inh). In addition to

  3. 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 ...

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

    NARCIS (Netherlands)

    van den Elzen, M.T.

    2017-01-01

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

  5. 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 ...

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

    DEFF Research Database (Denmark)

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

    2016-01-01

    OBJECTIVE: To estimate health status utility (preference) weights for hereditary angioedema (HAE) during an attack and between attacks using data from the Hereditary Angioedema Burden of Illness Study in Europe (HAE-BOIS-Europe) survey. Utility measures quantitatively describe the net impact of a...

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

    Science.gov (United States)

    Owens, Ryan E; Oliphant, Carrie S

    2017-01-01

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

  8. Gastrointestinal manifestations of hereditary angioedema diagnosed by ultrasound in the emergency department.

    Science.gov (United States)

    Riguzzi, Christine; Losonczy, Lia; Teismann, Nathan; Herring, Andrew A; Nagdev, Arun

    2014-11-01

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

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

    Directory of Open Access Journals (Sweden)

    Christine Riguzzi

    2014-11-01

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

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

    Science.gov (United States)

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

    2010-04-01

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

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

    Science.gov (United States)

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

    2017-09-01

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

  12. Dipeptidyl Peptidase IV in Angiotensin-Converting Enzyme Inhibitor–Associated Angioedema

    OpenAIRE

    Byrd, James Brian; Touzin, Karine; Sile, Saba; Gainer, James V.; Yu, Chang; Nadeau, John; Adam, Albert; Brown, Nancy J.

    2007-01-01

    Angioedema is a potentially life-threatening adverse effect of angiotensin-converting enzyme inhibitors. Bradykinin and substance P, substrates of angiotensin-converting enzyme, increase vascular permeability and cause tissue edema in animals. Studies indicate that amino-terminal degradation of these peptides, by aminopeptidase P and dipeptidyl peptidase IV, may be impaired in individuals with angiotensin-converting enzyme inhibitor–associated angioedema. This case-control study tested the hy...

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

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    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.

  14. 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

  15. 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.

  16. Hereditary angioedema: a bradykinin-mediated swelling disorder.

    Science.gov (United States)

    Björkqvist, Jenny; Sala-Cunill, Anna; Renné, Thomas

    2013-03-01

    Edema is tissue swelling and is a common symptom in a variety of diseases. Edema form due to accumulation of fluids, either through reduced drainage or increased vascular permeability. There are multiple vascular signalling pathways that regulate vessel permeability. An important mediator that increases vascular leak is the peptide hormone bradykinin, which is the principal agent in the swelling disorder hereditary angioedema. The disease is autosomal dominant inherited and presents clinically with recurrent episodes of acute swelling that can be life-threatening involving the skin, the oropharyngeal, laryngeal, and gastrointestinal mucosa. Three different types of hereditary angiodema exist in patients. The review summarises current knowledge on the pathophysiology of hereditary angiodema and focuses on recent experimental and pharmacological findings that have led to a better understanding and new treatments for the disease.

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

    Science.gov (United States)

    Banerji, Aleena

    2013-11-01

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

  18. Hereditary angioedema: what the gastroenterologist needs to know

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    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

  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. Enzymatic assays for the diagnosis of bradykinin-dependent angioedema.

    Directory of Open Access Journals (Sweden)

    Federica Defendi

    Full Text Available BACKGROUND: The kinins (primarily bradykinin, BK represent the mediators responsible for local increase of vascular permeability in hereditary angioedema (HAE, HAE I-II associated with alterations of the SERPING1 gene and HAE with normal C1-Inhibitor function (HAE-nC1INH. Besides C1-Inhibitor function and concentration, no biological assay of kinin metabolism is actually available to help physicians for the diagnosis of angioedema (AE. We describe enzymatic tests on the plasma for diagnosis of BK-dependent AE. METHODS: The plasma amidase assays are performed using the Pro-Phe-Arg-p-nitroanilide peptide substrate to evaluate the spontaneous amidase activity and the proenzyme activation. We analyzed data of 872 patients presenting with BK-dependent AE or BK-unrelated diseases, compared to 303 controls. Anti-high MW kininogen (HK immunoblot was achieved to confirm HK cleavage in exemplary samples. Reproducibility, repeatability, limit of blank, limit of detection, precision, linearity and receiver operating characteristics (ROC were used to calculate the diagnostic performance of the assays. RESULTS: Spontaneous amidase activity was significantly increased in all BK-dependent AE, associated with the acute phase of disease in HAE-nC1INH, but preserved in BK-unrelated disorders. The increase of the amidase activity was associated to HK proteolysis, indicating its relevance to identify kininogenase activity. The oestrogens, known for precipitating AE episodes, were found as triggers of enzymatic activity. Calculations from ROC curves gave the optimum diagnostic cut-off for women (9.3 nmol⋅min(-1⋅mL(-1, area under curve [AUC] 92.1%, sensitivity 80.0%, and specificity 90.1% and for men (6.6 nmol·min(-1⋅mL(-1, AUC 91.0%, sensitivity 87.0% and specificity 81.2%. CONCLUSION: The amidase assay represents a diagnostic tool to help physicians in the decision to distinguish between BK-related and -unrelated AE.

  1. 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

  2. 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.

  3. Recent advances in management and treatment of hereditary angioedema.

    Science.gov (United States)

    Sardana, Niti; Craig, Timothy J

    2011-12-01

    Hereditary angioedema (HAE) is a rare autosomal-dominant disease characterized by recurrent self-limiting episodes of skin and mucosal edema. Morbidity and mortality are significant, and new and pending therapies are now available to reduce the risk associated with the disease. To update the reader on new advances in HAE to improve patient care. We performed a literature search of Ovid, PubMed, and Google to develop this review. Articles that are necessary for the understanding and use of the new therapeutic options for HAE were chosen, and studies of high quality were used to support the use of therapies, and in most cases, results from phase III studies were used. Until recently, therapy for HAE attacks in the United States consisted of symptom relief with narcotics, hydration, and fresh-frozen plasma, which contains active C1 inhibitor. Therapy to prevent HAE attacks has been confined to androgens and, occasionally, antifibrinolytic agents; however, both drug groups have significant adverse effects. The approval of C1-inhibitor concentrate for prevention and acute therapy has improved efficacy and safety. Ecallantide has also been approved for therapy of attacks, and icatibant is expected to be approved in the next few months for attacks. Recombinant C1 inhibitor is presently in phase III studies and should be available for attacks in the near future. In this article we review the changing therapeutic options available for patients in 2011 and beyond.

  4. 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.

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

    DEFF Research Database (Denmark)

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

    2018-01-01

    to vasodilatation and increase in vascular permeability in the capillaries. Objective To assess the risk and pathomechanism of angioedema due to inhibition of dipeptidyl peptidase-4 inhibitors when used as monotherapy and in combination with angiotensin converting enzyme-inhibitors. Method PubMed, Embase......, the Cochrane Library, PubMed Central, Web of Science, Google Scholar and clinicaltrials.gov were searched using different combinations of keywords "angioedema", "dipeptidyl peptidase 4", "dipeptidyl peptidase 4 inhibitors", "gliptins", "bradykinin", "substance P" and "angiotensin converting enzyme...

  6. Angioedema in heart failure patients treated with sacubitril/valsartan (LCZ696) or enalapril in the PARADIGM-HF study.

    Science.gov (United States)

    Shi, Victor; Senni, Michele; Streefkerk, Hendrik; Modgill, Vikas; Zhou, Wenchun; Kaplan, Allen

    2018-08-01

    PARADIGM-HF demonstrated significant clinical benefits for sacubitril/valsartan (LCZ696, an angiotensin receptor neprilysin inhibitor) versus the angiotensin-converting enzyme inhibitor (ACEI) enalapril in patients with heart failure with reduced ejection fraction. As inhibition of ACE, and co-inhibition of ACE and neprilysin, may increase the risk of angioedema, this was an adverse event of special interest. Following sequential enalapril and sacubitril/valsartan run-ins, patients were randomized to twice-daily sacubitril/valsartan 200 mg or enalapril 10 mg. The study design incorporated two wash-out periods (~36 h each) to minimize any potential risk of angioedema due to overlapping ACE and neprilysin inhibition. Suspected cases of angioedema were reported to, and blindly adjudicated by, an independent angioedema adjudication committee (AAC). Of the 10,513 patients entering the enalapril run-in, 9419 entered the sacubitril/valsartan run-in and 8432 received double-blind treatment. Overall, 148 suspected angioedema events occurring in 144 patients were reported to AAC, with one event reported during screening period. Of the remaining 147 events, 54 were confirmed as angioedema by AAC. A confirmed event was experienced by 15 (0.14%) and 10 (0.11%) patients, during the enalapril and sacubitril/valsartan run-ins, respectively, and by 10 (0.24%) and 19 (0.45%) patients in the corresponding randomized arms during the double-blind phase. The frequency of confirmed angioedema was higher in black patients. Most events were mild. Only five patients required hospitalization and none required mechanical airway support. The number of confirmed angioedema events in PARADIGM-HF was low and there was no-marked excess risk of angioedema with sacubitril/valsartan versus enalapril. Copyright © 2018 The Authors. Published by Elsevier B.V. All rights reserved.

  7. Depression and anxiety in patients with hereditary angioedema.

    Science.gov (United States)

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

    2014-04-01

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

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

    Science.gov (United States)

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

    2011-01-01

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

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

    DEFF Research Database (Denmark)

    Caballero, Teresa; Zanichelli, Andrea; Aberer, Werner

    2018-01-01

    Background: Icatibant is a bradykinin B2-receptor antagonist used for the treatment of hereditary angioedema attacks resulting from C1-inhibitor deficiency. Treatment is not adjusted by body weight however the impact of body mass index (BMI) on the effectiveness of icatibant is not documented in ...

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

    Science.gov (United States)

    Longhurst, Hilary J

    2010-09-07

    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.

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

    DEFF Research Database (Denmark)

    Aberer, W; Maurer, M; Reshef, A

    2014-01-01

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

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

    DEFF Research Database (Denmark)

    Aberer, Werner; Maurer, Marcus; Bouillet, Laurence

    2017-01-01

    BACKGROUND: Patients with hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE) experience recurrent attacks of cutaneous or submucosal edema that may be frequent and severe; prophylactic treatments can be prescribed to prevent attacks. However, despite the use of long-term prop...

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

    Science.gov (United States)

    Longhurst, Hilary J

    2010-01-01

    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. PMID:20859548

  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 ...

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

    OpenAIRE

    Hilary Longhurst

    2018-01-01

    Acute treatment of hereditary angioedema due to C1 inhibitor deficiency has become available in the last 10 years and has greatly improved patients’ quality of life. Two plasma-derived C1 inhibitors (Berinert and Cinryze), a recombinant C1 inhibitor (Ruconest/Conestat alpha), a kallikrein inhibitor (Ecallantide), and a bradykinin B2 receptor inhibitor (Icatibant) are all effective. Durably good response is maintained over repeated treatments and several years. All currently available prophyla...

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

    Science.gov (United States)

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

    1988-01-01

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

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

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    Gavin Oudit

    2001-01-01

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

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

    Science.gov (United States)

    Bork, K; Davis-Lorton, M

    2013-02-01

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

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

    Science.gov (United States)

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

    2003-12-01

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

  20. Hereditary angioedema with C1 inhibitor deficiency: delay in diagnosis in Europe.

    Science.gov (United States)

    Zanichelli, Andrea; Magerl, Markus; Longhurst, Hilary; Fabien, Vincent; Maurer, Marcus

    2013-08-12

    Hereditary angioedema (HAE) is a rare, debilitating, and potentially life-threatening disease characterized by recurrent edema attacks. Important advances in HAE treatment have been made, including the development of new therapies for treating or preventing attacks. Nevertheless, the disease is still frequently misdiagnosed and inappropriately treated, potentially exposing patients with laryngeal attacks to the risk of asphyxiation. The Icatibant Outcome Survey (IOS) is an international, observational study that documents the clinical outcome of HAE patients eligible for treatment with icatibant. Patient ages at first symptoms and at diagnosis were recorded at enrolment, and the delay between first symptoms and diagnosis was calculated. The median [range] diagnostic delay in HAE type I and II patients across eight countries was 8.5 years [0-62.0]. The median delay in diagnosis was longer for HAE type II versus type I (21 versus 8 years, respectively), although this did not quite reach statistical significance. Although it can be difficult to differentiate HAE symptoms from those of more common angioedema sub-types (e.g. idiopathic or acquired angioedema), our results show that HAE type I and II patients have an unacceptable delay in diagnosis, even those with a family history of the disease. Raising physician awareness of this disabling and potentially fatal disease may lead to a more accurate diagnosis and timely treatment.

  1. [Acquired angioedema – clinical characteristic of the patients diagnosed in 2012-2016 with acquired C1 inhibitor deficiency].

    Science.gov (United States)

    Stobiecki, Marcin; Czarnobilska, Ewa; Obtułowicz, Krystyna

    Acquired angioedema is a rare disease caused by a deficiency of C1 esterase inhibitor with recurrent swelling symptoms. It may occur in the course of lymphoproliferative disorders or autoimmune diseases. Symptoms resemble hereditary angioedema, and the only differentiating features is negative family history, late onset of symptoms and accompanying lymphoproliferative disorder. The aim of the study was to analyze the cases of acquired angioedema. The retrospective analysis of 341 patients from the registry of patients with C1 inhibitor deficiency. Results: We identified 4 patients among 119 with HAE (3.57%) diagnosed in this same period of time 2012-2016 who fulfilled the criteria of acquired edema. In two cases the primary reason of angioedema was lymphoproliferive disease, in two monoclonal gammapathy of unknown reason. We analyzed also the results of laboratory tests C4, C1 inhibitor, C1q. In all cases the face was dominated localization. After the treatment of primary lymphoproliferive disease, in two cases, we observed total remission of angioedema. Only one patient with gammapathy require treatment with C1 inhibitor during the attacks. In these case we observed both plasma deriver, and recombinant C1 inhibitor were effective.

  2. Autoimmune thyroid disease as a risk factor for angioedema in patients with chronic idiopathic urticaria: a case-control study

    Directory of Open Access Journals (Sweden)

    Ruy Felippe Brito Gonçalves Missaka

    Full Text Available CONTEXT AND OBJECTIVE: An association between chronic idiopathic urticaria (CIU and autoimmune thyroid disease (ATD has been reported. However, there have not been any reports on whether ATD raises the risk of angioedema, which is a more severe clinical presentation of CIU. Thus, the aim of the present study was to evaluate whether the risk of angioedema is increased in patients with CIU and ATD. DESIGN AND SETTING: Case-control study including 115 patients with CIU at a tertiary public institution. METHODS: The patients were evaluated with regard to occurrence of angioedema and presence of ATD, hypothyroidism or hyperthyroidism. RESULTS: Angioedema was detected in 70 patients (60.9%. There were 22 cases (19.1% of ATD, 19 (16.5% of hypothyroidism and nine (7.8% of hyperthyroidism. The risk among patients with ATD was 16.2 times greater than among those without this thyroid abnormality (confidence interval, CI = 2.07-126.86. The odds ratio for hypothyroidism was 4.6 (CI = 1.00-21.54 and, for hyperthyroidism, 3.3 (CI = 0.38-28.36. CONCLUSIONS: Patients with CIU and ATD presented greater risk of angioedema, which reinforces the idea that a relationship exists between this allergic condition and thyroid autoimmunity. This finding could imply that such patients require specifically directed therapy.

  3. Isolated angioedema of the bowel due to C1 esterase inhibitor deficiency: a case report and review of literature

    Directory of Open Access Journals (Sweden)

    Kothari Shivangi T

    2011-02-01

    Full Text Available Abstract Introduction We report a rare, classic case of isolated angioedema of the bowel due to C1-esterase inhibitor deficiency. It is a rare presentation and very few cases have been reported worldwide. Angioedema has been classified into three categories. Case presentation A 66-year-old Caucasian man presented with a ten-month history of episodic severe cramping abdominal pain, associated with loose stools. A colonoscopy performed during an acute attack revealed nonspecific colitis. Computed tomography of the abdomen performed at the same time showed a thickened small bowel and ascending colon with a moderate amount of free fluid in the abdomen. Levels of C4 ( Conclusion In addition to a detailed comprehensive medical history, laboratory data and imaging studies are required to confirm a diagnosis of angioedema due to C1 esterase inhibitor deficiency.

  4. [Tranexamic acid as first-line emergency treatment for episodes of bradykinin-mediated angioedema induced by ACE inhibitors].

    Science.gov (United States)

    Beauchêne, C; Martins-Héricher, J; Denis, D; Martin, L; Maillard, H

    2018-05-04

    Episodes of acquired bradykinin-mediated angioedema due to angiotensin-converting enzyme (ACE) inhibitors may result in fatal outcomes. There is no consensus regarding emergency pharmacological management of these episodes. Treatment options include icatibant and C1INH concentrate. Tranexamic acid is administered for moderate episodes. Its efficacy in the treatment of ACE inhibitor-induced episodes of angioedema is not established. The aim of this retrospective study is to assess the benefits of emergency tranexamic acid administration in the management of ACE inhibitor-induced episodes of angioedema. Retrospective analysis of the medical files of patients who consulted between 2010 and 2016 in two French tertiary care hospitals for a bradykinic angioedema attributed to an ACE treatment. All of them had received tranexamic acid as a first line treatment. Thirty three patients who had experienced severe episode of angioedema were included. Twenty seven patients showed significant improvement when treated with tranexamic acid alone. The six remaining patients were treated with icatibant (5/33) or C1INH concentrate (1/33), due to partial improvement after tranexamic acid therapy. None of the patients were intubated, no fatalities were recorded and no side effects were reported. Tranexamic acid is an easily accessible and affordable therapy that may provide effective treatment for ACE inhibitor-induced episodes of angioedema. It may help while waiting for a more specific treatment (icatibant and C1INH concentrate) that is at times unavailable in emergency departments. Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    Aygören-Pürsün E

    2016-09-01

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

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

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    Kirankumar N Kulkarni

    2014-01-01

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

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

    DEFF Research Database (Denmark)

    Varga, L; Széplaki, G; Laki, J

    2008-01-01

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

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

    DEFF Research Database (Denmark)

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

    2012-01-01

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

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

    Science.gov (United States)

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

    2017-01-01

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

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

    Science.gov (United States)

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

    2007-01-01

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

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

    Science.gov (United States)

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

    2012-09-01

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

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

    DEFF Research Database (Denmark)

    Busse, Paula; Bygum, Anette; Edelman, Jonathan

    2014-01-01

    BACKGROUND: The plasma-derived, pasteurized C1-inhibitor (C1-INH) concentrate, Berinert has a 4-decade history of use in hereditary angioedema (HAE), with a substantial literature base that demonstrates safety and efficacy. Thromboembolic events have rarely been reported with C1-INH products......, typically with off-label use or at supratherapeutic doses. OBJECTIVES: Active surveillance of safety and clinical usage patterns of pasteurized C1-inhibitor concentrate and the more recent pasteurized, nanofiltered C1-INH, with a particular interest in thromboembolic events. METHODS: A registry...

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

    Science.gov (United States)

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

    2016-08-01

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

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

    DEFF Research Database (Denmark)

    Hermanrud, Thorbjørn; Duus, Nicolaj; Bygum, Anette

    2016-01-01

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

  15. 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.

  16. Determinants of angiotensin-converting enzyme inhibitor (ACEI) intolerance and angioedema in the UK Clinical Practice Research Datalink

    NARCIS (Netherlands)

    Mahmoudpour, Seyed Hamidreza; Baranova, Ekaterina Vitalievna; Souverein, Patrick C.; Asselbergs, Folkert W.; de Boer, Anthonius; Maitland-van der Zee, Anke Hilse

    2016-01-01

    AimThe aim of the present study was to describe the occurrence and determinants of angiotensin-converting enzyme (ACE) inhibitor (ACEI) intolerance and angioedema (AE) among patients initiating ACEI therapy in a real-world primary care population. MethodsTwo nested case-control studies were

  17. ELISA to measure neutralizing capacity of anti-C1-inhibitor antibodies in plasma of angioedema patients

    NARCIS (Netherlands)

    Engel, Ruchira; Rensink, Irma; Roem, Dorina; Brouwer, Mieke; Kalei, Asma; Perry, Dawn; Zeerleder, Sacha; Wouters, Diana; Hamann, Dörte

    2015-01-01

    Neutralizing autoantibodies (NAbs) against plasma serpin C1-inhibitor (C1-inh) are implicated in the rare disorder, acquired angioedema (AAE). There is insufficient understanding of the process of antibody formation and its correlation with disease progression and severity. We have developed an

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

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    Vilson Furlanetto Junior

    2010-02-01

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

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

    Science.gov (United States)

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

    2017-01-16

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

  20. Recurrent Laryngeal Edema Imitating Angioedema Caused by Dislocated Screw after Anterior Spine Surgery

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    Piotr Wójtowicz

    2015-01-01

    Full Text Available The anterior cervical spine surgery is a common procedure to stabilize vertebrae damaged by various diseases. The plates and screws are usually used in the spine fixation. This kind of instrumentation may detach from the bones which is a rare but well-known complication. A 77-year-old male presented to the otorhinolaryngology department with throat pain, choking, and dysphagia. At first the angioedema was diagnosed and he was treated conservatively. The endoscopy revealed laryngeal edema, being more defined on the right side with right vocal fold paresis. CT scans showed the stabilizing plate with two screws attached tightly and the back-out of the third screw toward soft tissue of the neck. In the meantime, his condition deteriorated and he needed tracheotomy. In few days the surgical removal of the dislocated screw was performed successfully. Although two-month follow-up reported no obstruction of the larynx, the vocal folds paresis with gradual functional improvement was observed. Long-term complication of anterior spine surgery sometimes may suggest laryngeal angioedema at first. If the conservative treatment is ineffective and there is a history of anterior spine surgery, the clinicians should consider the displacement of the plate or screws in differential diagnosis.

  1. 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

    BACKGROUND: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) may affect health-related quality of life (HRQoL). A specific HRQoL questionnaire for adult patients with C1-INH-HAE, the HAE-QoL, has recently been developed in Spain. OBJECTIVE: The objective of this study...... was to perform a cross-cultural validation and psychometric study of the HAE-QoL in an international setting. METHODS: Cross-cultural adaptation of the Spanish HAE-QoL draft version and an international rating phase with experts were performed. The resultant version of the HAE-QoL, a clinical questionnaire...... with and without psychiatric and/or psychological care (median: 74 vs 103; P ≤ .001). CONCLUSIONS: The HAE-QoL, currently available in 18 languages, showed good reliability and validity evidence....

  2. Acutely Onset Amiodarone-Induced Angioedema in a Patient with New Atrial Fibrillation

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    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.

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

    Science.gov (United States)

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

    2017-11-01

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

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

    OpenAIRE

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

    2016-01-01

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

  5. Approaches to the diagnosis and management of patients with a history of nonsteroidal anti-inflammatory drug-related urticaria and angioedema.

    Science.gov (United States)

    Kowalski, Marek L; Woessner, Katharine; Sanak, Marek

    2015-08-01

    Nonsteroidal anti-inflammatory drug (NSAID)-induced urticarial and angioedema reactions are among the most commonly encountered drug hypersensitivity reactions in clinical practice. Three major clinical phenotypes of NSAID-induced acute skin reactions manifesting with angioedema, urticaria, or both have been distinguished: NSAID-exacerbated cutaneous disease, nonsteroidal anti-inflammatory drug-induced urticaria/angioedema (NIUA), and single NSAID-induced urticaria and angioedema. In some patients clinical history alone might be sufficient to establish the diagnosis of a specific type of NSAID hypersensitivity, whereas in other cases oral provocation challenges are necessary to confirm the diagnosis. Moreover, classification of the type of cutaneous reaction is critical for proper management. For example, in patients with single NSAID-induced reactions, chemically nonrelated COX-1 inhibitors can be safely used. However, there is cross-reactivity between the NSAIDs in patients with NSAID-exacerbated cutaneous disease and NIUA, and thus only use of selective COX-2 inhibitors can replace the culprit drug if the chronic treatment is necessary, although aspirin desensitization will allow for chronic treatment with NSAIDs in some patients with NIUA. In this review we present a practical clinical approach to the patient with NSAID-induced urticaria and angioedema. Copyright © 2015 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  6. Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency

    Directory of Open Access Journals (Sweden)

    Abdullateef Abdulkareem

    2018-01-01

    Full Text Available Acquired angioedema due to C1 inhibitor deficiency (C1INH-AAE is a rare and potentially fatal syndrome of bradykinin-mediated angioedema characterized by episodes of angioedema without urticaria. It typically manifests with nonpitting edema of the skin and edema in the gastrointestinal (GI tract mucosa or upper airway. Edema of the upper airway and tongue may lead to life-threatening asphyxiation. C1INH-AAE is typically under-diagnosed because of its rarity and its propensity to mimic more common abdominal conditions and allergic reactions. In this article, we present the case of a 62-year-old male with a history of recently diagnosed chronic lymphocytic leukemia (CLL who presented to our hospital with recurrent abdominal pain, initially suspected to have Clostridium difficile colitis and diverticulitis. He received a final diagnosis of acquired angioedema due to C1 esterase inhibitor deficiency due to concomitant symptoms of lip swelling, cutaneous nonpitting edema of his lower extremities, and complement level deficiencies. He received acute treatment with C1 esterase replacement and icatibant and was maintained on C1 esterase infusions. He also underwent chemotherapy for his underlying CLL and did not experience further recurrence of his angioedema.

  7. Hypovolemic Shock Caused by Angiotensin-Converting Enzyme Inhibitor-Induced Visceral Angioedema: A Case Series and A Simple Method to Diagnose this Complication in the Emergency Department.

    Science.gov (United States)

    Myslinski, Joseph; Heiser, Andrew; Kinney, Ashley

    2018-03-01

    Visceral angioedema is a rarely reported side effect of angiotensin-converting-enzyme inhibitors (ACEI). Because signs and symptoms tend to be nonspecific, the diagnosis is difficult to make, especially in the emergency department (ED). We describe 2 patients presenting with signs of hypovolemic shock, in which the diagnosis of ACEI-induced visceral angioedema was made in the ED. We surmise that patients with abdominal pain, who present with hypovolemic shock and are taking medications that can predispose to angioedema, may have this complication if their hemoglobin level is elevated compared with their previous levels. An abdominal computed tomography scan, if it does not identify any other significant etiology, will increase the probability that ACEI-induced visceral angioedema is the diagnosis when there is nonspecific bowel wall thickening or edema. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Identification of ACEI-induced visceral angioedema in the ED will avoid prolonged admissions, unnecessary procedures, and future recurrences. Copyright © 2017 Elsevier Inc. All rights reserved.

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

    Science.gov (United States)

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

    2016-01-01

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

  9. Type I anaphylactic reaction due to contrast induced angioedema causing neck swelling: the role of sitting fiberoptic bronchoscopy in emergent intubation

    Directory of Open Access Journals (Sweden)

    Ali Dabbagh

    2016-07-01

    Full Text Available Contrast induced angioedema is a rapidly progressive state involving a number of organ systems including the upper airway tract; which is usually a type I anaphylactic reaction also known as immediate hypersensitivity reaction. Prompt preservation of the respiratory tract is the cornerstone of this situation. The use of fiberoptic bronchoscope for tracheal intubation though very helpful, has some special considerations due to the anatomic distortions created by edema.This manuscript describes a patient with contrast induced angioedema managed successfully. Serum levels of IgE were highly increased during the first hours after the event; while serum levels of complement were normal. However, rapid airway management and prophylactic intubation saved the patient and prevented the possible aftermath of airway obstruction.Keywords: airway management; type I anaphylactic reaction, angioedema; fiberoptic bronchoscope.Conflict of interest: none of the authors has any conflict of interest.

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

    Directory of Open Access Journals (Sweden)

    Rohan Ameratuga

    2016-11-01

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

  11. Severe angioedema in myxedema coma: a difficult airway in a rare endocrine emergency.

    Science.gov (United States)

    Lee, Christopher H; Wira, Charles R

    2009-10-01

    Myxedema coma is the most lethal manifestation of hypothyroidism. It is a true medical emergency and can result in profound hemodynamic instability and airway compromise. Myxedema coma currently remains a diagnostic challenge due to the rarity of cases seen today, and failure to promptly initiate therapy with replacement thyroid hormone can be fatal. As thyroid hormone therapy can take days or weeks to reverse the manifestations of myxedema coma, interim supportive therapy is critical while awaiting clinical improvement. Some patients will require endotracheal intubation in the emergency department (ED), and physicians should be aware that unanticipated posterior pharyngeal edema in myxedema coma could severely complicate airway management. Although mechanical ventilation is a well-described adjunctive therapy for myxedema coma, reports of the potential difficulty in securing a definitive airway in these patients are rare. We describe a case of an unidentified woman who presented to the ED with myxedema coma requiring urgent endotracheal intubation and was found to have extensive posterior pharyngeal angioedema inconsistent with her relatively benign external examination. This case highlights the typical features of myxedema coma and discusses our necessity for a rescue device in definitive endotracheal tube placement. Emergency physicians should anticipate a potentially difficult airway in all myxedema coma patients regardless of the degree of external facial edema present.

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

    Science.gov (United States)

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

    2017-11-30

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

  13. Facial Oedema Is Not Always Angioedema: A Case of Spontaneous Pneumomediastinum with Subcutaneous Emphysema during COPD Exacerbation

    Directory of Open Access Journals (Sweden)

    Sarah Damanti

    2015-10-01

    Full Text Available We report a case of acute facial oedema in an elderly hospitalized patient which was initially misdiagnosed as angioedema secondary to antibiotics in a patient with an allergic diathesis. We describe the differential aetiologies and then the true cause of the oedema, which was an uncommon complication of a very common condition in the elderly: a pneumomediastinum with subcutaneous emphysema probably due to rupture of an emphysematous lung bulla during chronic obstructive pulmonary disease (COPD exacerbation. Lastly, we focus on the therapeutic procedures instituted for the treatment of the pneumomediastinum.

  14. Hereditary angioedema attacks resolve faster and are shorter after early icatibant treatment.

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    Marcus Maurer

    Full Text Available BACKGROUND: Attacks of hereditary angioedema (HAE are unpredictable and, if affecting the upper airway, can be lethal. Icatibant is used for physician- or patient self-administered symptomatic treatment of HAE attacks in adults. Its mode of action includes disruption of the bradykinin pathway via blockade of the bradykinin B(2 receptor. Early treatment is believed to shorten attack duration and prevent severe outcomes; however, evidence to support these benefits is lacking. OBJECTIVE: To examine the impact of timing of icatibant administration on the duration and resolution of HAE type I and II attacks. METHODS: The Icatibant Outcome Survey is an international, prospective, observational study for patients treated with icatibant. Data on timings and outcomes of icatibant treatment for HAE attacks were collected between July 2009-February 2012. A mixed-model of repeated measures was performed for 426 attacks in 136 HAE type I and II patients. RESULTS: Attack duration was significantly shorter in patients treated <1 hour of attack onset compared with those treated ≥ 1 hour (6.1 hours versus 16.8 hours [p<0.001]. Similar significant effects were observed for <2 hours versus ≥ 2 hours (7.2 hours versus 20.2 hours [p<0.001] and <5 hours versus ≥ 5 hours (8.0 hours versus 23.5 hours [p<0.001]. Treatment within 1 hour of attack onset also significantly reduced time to attack resolution (5.8 hours versus 8.8 hours [p<0.05]. Self-administrators were more likely to treat early and experience shorter attacks than those treated by a healthcare professional. CONCLUSION: Early blockade of the bradykinin B(2 receptor with icatibant, particularly within the first hour of attack onset, significantly reduced attack duration and time to attack resolution.

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

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    Hilary Longhurst

    2018-03-01

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

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

    Science.gov (United States)

    Longhurst, Hilary

    2017-01-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

  18. 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.

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

    Science.gov (United States)

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

    2012-04-26

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

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

    Science.gov (United States)

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

    2015-08-01

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

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

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    Rémi Baroso

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

  2. Audit report and systematic review of orolingual angioedema in post-acute stroke thrombolysis.

    Science.gov (United States)

    Lekoubou, Alain; Philippeau, Frédéric; Derex, Laurent; Olaru, Angel; Gouttard, Michel; Vieillart, Anne; Kengne, Andre Pascal

    2014-07-01

    Post-intravenous recombinant tissue plasminogen activator (r-tPA) orolingual angioedema (PIROLA), including the life-threatening form, is an underappreciated complication of ischaemic stroke treatment. We present an audit report and a systematic review of published observational studies on PIROLA occurrence in acute ischaemic stroke patients. Clinical files of patients treated in the stroke unit of Bourg-en-Bresse General Hospital (France) from January 2010 to December 2012 were reviewed, and MEDLINE (inception to May 2013) were searched and bibliographies/citations of retrieved articles examined for evidence of PIROLA. Of the 129 acute ischaemic stroke patients treated at Bourg-en-Bresse between 2010 and 2012, four patients, all receiving angiotensin converting enzyme inhibitor (ACEI), developed a PIROLA (cumulative incidence rate: 32‰). The complication started within an hour of receiving r-tPA and integrally resolved within 3-24 hours, with antihistamines/steroid treatment in two patients. The systematic review identified 27 studies, totalising with ours, over 9050 acute ischaemic stroke patients from 12 countries, among whom 100 (cumulative incidence rate: 17‰; 95% confidence intervals: 8-26), developed a PIROLA within 6-240 minutes of receiving r-tPA, 0-100% of them occurring among patients on ACEI. The complication was contralateral to the stroke location in 47% cases, ipsilateral in 14%, and bilateral in 39%; and resolved within 24 hours with treatment in 90%. No related death was recorded. About 17‰ acute ischaemic stroke patients receiving r-tPA develop PIROLA, occurring essentially among those on concomitant ACEI. PIROLA occurrence should be actively monitored, particularly within the first few hours as some may require urgent lifesaving procedures.

  3. Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group

    NARCIS (Netherlands)

    Cicardi, M.; Bork, K.; Caballero, T.; Craig, T.; Li, H. H.; Longhurst, H.; Reshef, A.; Zuraw, B.; Werner, Aberer; Aygören-Pürsün, Emel; Banerji, Aleena; Bjorkander, Janne; Boccon-Gibod, Isabelle; Konrad, Bork; Bouillet, Laurence; Bova, Maria; Bowen, Tom; Branco Ferreira, Manuel; Bygum, Anette; Caballero, Teresa; Cancian, Mauro; Castel-Branco, Maria Graça; Cicardi, Marco; Craig, Timothy; de Carolis, Caterina; Mihály, Enikö; Josè, Fabiani; Farkas, Henriette; Gompels, Mark; Gower, Richard; Groffik, Adriane; Grumach, Anete; Guillarte, Mar; Hernandez Landeros, Maria Esthela; Kaplan, Allen; Leibovich, Iris; Li, Henry; Lock, Bob; Longhurst, Hilary; Lumry, William; Malbran, Alejandro; Martinez-Saguer, Immaculada; Campos, Matta; Maurer, Marcus; Moldovan, Dumitru; Montinaro, Vincenzo; Nieto, Sandra; Nordenfelt, Patrik; Obtulovicz, Krystana; Zeerleder, Sacha

    2012-01-01

    Angioedema owing to hereditary deficiency of C1 inhibitor (HAE) is a rare, life-threatening, disabling disease. In the last 2 years, the results of well-designed and controlled trials with existing and new therapies for this condition have been published, and new treatments reached the market.

  4. The Complex Interaction Between Polycystic Ovary Syndrome and Hereditary Angioedema: Case Reports and Review of the Literature.

    Science.gov (United States)

    Iahn-Aun, Marina; Aun, Marcelo Vivolo; Motta, Antonio Abílio; Kalil, Jorge; Giavina-Bianchi, Pedro; Hayashida, Sylvia Asaka; Baracat, Edmund Chada; Maciel, Gustavo Arantes

    2017-07-01

    Hereditary angioedema (HAE) is a rare but severe disease, with high risk of death, and attacks have been associated to high estrogen levels. Polycystic ovary syndrome (PCOS) is a common hyperandrogenic condition, which is frequently treated with combined oral contraceptives. The aim of this study was to describe 2 clinical cases of young women diagnosed as having PCOS who developed HAE attacks after the introduction of combined estrogen-progestin pills to treat PCOS symptoms. Literature review of sex hormones' role in genesis of HAE attacks and possible mechanisms involved. In the cases reported, after initiation of combined contraceptives, patients presented with facial swelling with airway involvement (laryngeal edema) and abdominal pain. They had a familial history of angioedema and normal C1 inhibitor (C1-INH) levels, leading to the diagnosis of HAE with normal C1-INH (HAEnC1-INH) or HAE type III. After suspension of exogenous estrogen, patients remained asymptomatic from HAE. HAEnC1-INH is an estrogen-dependent form of HAE. It is well established that exogenous estrogen triggers attacks of all types of HAE. However, this is the first description of the association between PCOS and HAE, in which PCOS could be masking HAE symptoms. We propose that PCOS might have a protective role regarding HAE attacks, because of its particular hormonal features, that is, hyperandrogenism and relative stable levels of estradiol. The use of combined estrogen-progestin compounds in women with PCOS and HAE must be avoided, and treatment must be individualized.

  5. Clinical Features of Hereditary and Mast Cell-mediated Angioedema Focusing on the Differential Diagnosis in Japanese Patients.

    Science.gov (United States)

    Ohsawa, Isao; Honda, Daisuke; Hisada, Atsuko; Inoshita, Hiroyuki; Onda-Tsueshita, Kisara; Mano, Satoshi; Sato, Nobuyuki; Nakamura, Yuya; Shimizu, Tatsuo; Gotoh, Hiromichi; Goto, Yoshikazu; Suzuki, Yusuke; Tomino, Yasuhiko

    2018-02-01

    Objective The present study was designed to identify the clinical characteristics that permit the differential diagnosis of hereditary angioedema (HAE) and mast cell-mediated angioedema (Mast-AE) during the first consultation. Methods The medical histories and laboratory data of 46 patients with HAE and 41 patients with Mast-AE were compared. Results The average age of onset in the HAE group (19.8±9.0 years) was significantly lower than that in the Mast-AE group (35.2±12.0 years). The incidence of familial angioedema (AE) in the HAE group (73.9%) was significantly higher than that in the Mast-AE group (9.7%). The frequency of history of AE in the extremities, larynx, or gastrointestinal tract was significantly higher in the HAE group. The frequency of AE episodes of the lips and eyelids was significantly lower in the HAE group. The serum C4 concentration and CH50 titer were lower than the normal limit in 91.3% and 45.6% of the patients in the HAE group, respectively; in Mast-AE group the serum C4 concentration and CH50 titer were significantly lower than the normal limit in 4.8% and 0% of the patients, the difference between the two groups was statistically significant. A C1-inhibitor (C1-INH) activity level of <50% was observed in all of the HAE patients, but none of the Mast-AE patients. The mean serum IgE titer in the HAE group (120.8±130.5 IU/mL) was significantly lower than that in the Mast-AE group (262.2±314.9 IU/mL). Conclusion The parameters within the patients' medical histories, such as the age at the onset of AE, a family history of AE, and the locations of past AE episodes are critical for the successful diagnosis of the disease. Measurements of the C4 and C1-INH activity are very useful for differential diagnosis of HAE from Mast-AE.

  6. Angioedema hereditario en Medellín, Colombia: evaluación clínica y de la calidad de vida

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    María Dulfary Sánchez

    2015-09-01

    Conclusión. Este estudio provee información sobre la primera familia caracterizada con angioedema hereditario de tipo 1 en el Valle de Aburrá, Colombia. Aunque para ello se usó un instrumento genérico, se confirmó, además, el efecto negativo de la enfermedad en la calidad de vida de los individuos que la padecen.

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

    Science.gov (United States)

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

    2018-01-31

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

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

    Science.gov (United States)

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

    2010-01-01

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

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

    DEFF Research Database (Denmark)

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

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Olivier Picone

    2010-01-01

    Full Text Available 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.

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

    Directory of Open Access Journals (Sweden)

    Ceyda Tunakan Dalgıç

    2018-01-01

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

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

    Science.gov (United States)

    Campos, Regis Albuquerque; Valle, Solange Oliveira Rodrigues; França, Alfeu Tavares; Cordeiro, Elisabete; Serpa, Faradiba Sarquis; Mello, Yara Ferreira; Malheiros, Teresinha; Toledo, Eliana; Mansour, Elie; Fusaro, Gustavo; Grumach, Anete Sevciovic

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Regis Albuquerque Campos

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

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

    Science.gov (United States)

    Farkas, H; Martinez-Saguer, I; Bork, K; Bowen, T; Craig, T; Frank, M; Germenis, A E; Grumach, A S; Luczay, A; Varga, L; Zanichelli, A

    2017-02-01

    The consensus documents published to date on hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients. We intended to produce consensus recommendations for the diagnosis and management of pediatric patients with C1-INH-HAE. During an expert panel meeting that took place during the 9th C1 Inhibitor Deficiency Workshop in Budapest, 2015 (www.haenet.hu), pediatric data were presented and discussed and a consensus was developed by voting. The symptoms of C1-INH-HAE often present in childhood. Differential diagnosis can be difficult as abdominal pain is common in pediatric C1-INH-HAE, but also commonly occurs in the general pediatric population. The early onset of symptoms may predict a more severe subsequent course of the disease. Before the age of 1 year, C1-INH levels may be lower than in adults; therefore, it is advisable to confirm the diagnosis after the age of one year. All neonates/infants with an affected C1-INH-HAE family member should be screened for C1-INH deficiency. Pediatric patients should always carry a C1-INH-HAE information card and medicine for emergency use. The regulatory approval status of the drugs for prophylaxis and for acute treatment is different in each country. Plasma-derived C1-INH, recombinant C1-INH, and ecallantide are the only agents licensed for the acute treatment of pediatric patients. Clinical trials are underway with additional drugs. It is recommended to follow up patients in an HAE comprehensive care center. The pediatric-focused international consensus for the diagnosis and management of C1-INH-HAE patients was created. © 2016 The Authors. Allergy Published by John Wiley & Sons Ltd.

  15. Exposure‐Response Model of Subcutaneous C1‐Inhibitor Concentrate to Estimate the Risk of Attacks in Patients With Hereditary Angioedema

    Science.gov (United States)

    Tortorici, Michael A.; Pawaskar, Dipti; Pragst, Ingo; Machnig, Thomas; Hutmacher, Matthew; Zuraw, Bruce; Cicardi, Marco; Craig, Timothy; Longhurst, Hilary; Sidhu, Jagdev

    2018-01-01

    Subcutaneous C1‐inhibitor (HAEGARDA, CSL Behring), is a US Food and Drug Administration (FDA)‐approved, highly concentrated formulation of a plasma‐derived C1‐esterase inhibitor (C1‐INH), which, in the phase III Clinical Studies for Optimal Management in Preventing Angioedema with Low‐Volume Subcutaneous C1‐inhibitor Replacement Therapy (COMPACT) trial, reduced the incidence of hereditary angioedema (HAE) attacks when given prophylactically. Data from the COMPACT trial were used to develop a repeated time‐to‐event model to characterize the timing and frequency of HAE attacks as a function of C1‐INH activity, and then develop an exposure–response model to assess the relationship between C1‐INH functional activity levels (C1‐INH(f)) and the risk of an attack. The C1‐INH(f) values of 33.1%, 40.3%, and 63.1% were predicted to correspond with 50%, 70%, and 90% reductions in the HAE attack risk, respectively, relative to no therapy. Based on trough C1‐INH(f) values for the 40 IU/kg (40.2%) and 60 IU/kg (48.0%) C1‐INH (SC) doses, the model predicted that 50% and 67% of the population, respectively, would see at least a 70% decrease in the risk of an attack. PMID:29316335

  16. 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.

    Science.gov (United States)

    Prior, Nieves; Remor, Eduardo; Gómez-Traseira, Carmen; López-Serrano, Concepción; Cabañas, Rosario; Contreras, Javier; Campos, Ángel; Cardona, Victoria; Cimbollek, Stefan; González-Quevedo, Teresa; Guilarte, Mar; de Rojas, Dolores Hernández Fernández; Marcos, Carmen; Rubio, María; Tejedor-Alonso, Miguel Ángel; Caballero, Teresa

    2012-07-20

    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. 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. 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.

  17. Understanding Hereditary Angioedema

    Science.gov (United States)

    American Academy of Allergy Asthma & Immunology Menu Search Main navigation Skip to content Conditions & Treatments Allergies Asthma Primary Immunodeficiency Disease Related Conditions Drug Guide Conditions Dictionary Just ...

  18. Development and content validity testing of a patient-reported outcomes questionnaire for the assessment of hereditary angioedema in observational studies.

    Science.gov (United States)

    Bonner, Nicola; Abetz-Webb, Linda; Renault, Lydie; Caballero, Teresa; Longhurst, Hilary; Maurer, Marcus; Christiansen, Sandra; Zuraw, Bruce

    2015-07-01

    Hereditary Angioedema (HAE), a rare genetic disease, manifests as intermittent, painful attacks of angioedema. Attacks vary in frequency and severity and include skin, abdominal and life-threatening laryngeal swellings. This study aimed to develop a patient reported outcome (PRO) tool for the assessment of HAE attacks, including their management and impact on patients' lives, for use in clinical studies, or by physicians in general practice. The results of open-ended face to face concept elicitation interviews with HAE patients in Argentina (n = 10) and the US (n = 33) were used to develop the first draft questionnaire of the HAE patient reported outcomes questionnaire (HAE PRO). Subsequently, in-depth cognitive debriefing interviews were performed with HAE patients in the UK (n = 10), Brazil (n = 10), Germany (n = 11) and France (n = 12). Following input from eight multinational clinical experts further cognitive interviews were conducted in the US (n = 12) and Germany (n = 12). Patients who experienced abdominal, cutaneous or laryngeal attacks of varying severity levels were included in all rounds of interviews. Across the rounds of interviews patients discussed their HAE attack symptoms, impacts and treatments. Cognitive debriefing interviews explored patient understanding and relevance of questionnaire items. All interviews were conducted face to face following a pre-defined semi-structured interview guide in the patient's native language. Patients reported a variety of HAE symptoms, attack triggers, warning signs, attack impacts and treatment options which were used to develop the HAE PRO. The HAE PRO was revised and refined following input from patients and clinical experts. The final 18-item HAE PRO provides an assessment of the HAE attack experience including symptoms, impacts, treatment requirements, healthcare resource use and loss of productivity caused by HAE attacks. Patient and expert input has contributed to the

  19. Effectiveness of icatibant for treatment of hereditary angioedema attacks is not affected by body weight: findings from the Icatibant Outcome Survey, a cohort observational study.

    Science.gov (United States)

    Caballero, Teresa; Zanichelli, Andrea; Aberer, Werner; Maurer, Marcus; Longhurst, Hilary J; Bouillet, Laurence; Andresen, Irmgard

    2018-01-01

    Icatibant is a bradykinin B2-receptor antagonist used for the treatment of hereditary angioedema attacks resulting from C1-inhibitor deficiency. Treatment is not adjusted by body weight however the impact of body mass index (BMI) on the effectiveness of icatibant is not documented in the literature. We examined disease characteristics and icatibant treatment effectiveness in patients stratified by BMI in the Icatibant Outcome Survey, an ongoing, international, observational study monitoring the real-world safety and effectiveness of icatibant. Attack and treatment characteristics as well as outcomes following treatment with icatibant were compared among patients with underweight, normal, overweight, and obese BMI. Data from 2697 icatibant-treated attacks in 342 patients (3.5, 44.7, 34.8, and 17.0% patients of underweight, normal, overweight, and obese BMI, respectively) were analyzed. There was no significant difference in the frequency and severity of attacks across BMI groups, although obese patients tended to have more attacks of high severity. There was no impact of BMI on the frequency of laryngeal attacks, but patients with normal BMI had fewer cutaneous attacks and more abdominal attacks. Most attacks (71.9-83.8%) were treated with a single icatibant injection without the need for rescue with plasma-derived C1-inhibitor (pdC1-INH), regardless of BMI. Patients with obese BMI used pdC1-INH as rescue treatment more often (P < 0.0001; P = 0.0232 excluding 2 outliers) and treated attacks earlier than patients with normal BMI (P = 0.007). Furthermore, time to resolution and duration of attack were shorter for patients with high BMI (P < 0.001 for overweight and P < 0.05 for obese versus normal). Overall, icatibant was comparatively effective in treating attacks in patients across all BMI groups. Trial registration NCT01034969.

  20. Availability of and access to orphan drugs: an international comparison of pharmaceutical treatments for pulmonary arterial hypertension, Fabry disease, hereditary angioedema and chronic myeloid leukaemia.

    Science.gov (United States)

    Blankart, Carl Rudolf; Stargardt, Tom; Schreyögg, Jonas

    2011-01-01

    Market authorization does not guarantee patient access to any given drug. This is particularly true for costly orphan drugs because access depends primarily on co-payments, reimbursement policies and prices. The objective of this article is to identify differences in the availability of orphan drugs and in patient access to them in 11 pharmaceutical markets: Australia, Canada, England, France, Germany, Hungary, the Netherlands, Poland, Slovakia, Switzerland and the US. Four rare diseases were selected for analysis: pulmonary arterial hypertension (PAH), Fabry disease (FD), hereditary angioedema (HAE) and chronic myeloid leukaemia (CML). Indicators for availability were defined as (i) the indications for which orphan drugs had been authorized in the treatment of these diseases; (ii) the application date; and (iii) the date upon which these drugs received market authorization in each country. Indicators of patient access were defined as (i) the outcomes of technology appraisals; (ii) the extent of coverage provided by healthcare payers; and (iii) the price of the drugs in each country. For PAH we analysed bosentan, iloprost, sildenafil, treprostinil (intravenous and inhaled) as well as sitaxentan and ambrisentan; for FD we analysed agalsidase alfa and agalsidase beta; for HAE we analysed icatibant, ecallantide and two complement C1s inhibitors; for CML we analysed imatinib, dasatinib and nilotinib. Most drugs included in this study had received market authorization in all countries, but the range of indications for which they had been authorized differed by country. The broadest range of indications was found in Australia, and the largest variations in indications were found for PAH drugs. Authorization process speed (the time between application and market authorization) was fastest in the US, with an average of 362 days, followed by the EU (394 days). The highest prices for the included drugs were found in Germany and the US, and the lowest in Canada, Australia and

  1. Socioeconomic burden of hereditary angioedema

    DEFF Research Database (Denmark)

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

    2014-01-01

    who were working or in school (n = 120), 72 provided work/school absenteeism data, resulting in an estimated 20 days missing from work/school on average per year; 51% (n = 84) indicated that HAE has hindered their career/educational advancement. CONCLUSION: HAE poses a considerable burden on patients...... and their families in terms of direct medical costs and indirect costs related to lost productivity. This burden is substantial at the time of attacks and in between attacks....

  2. Photos & Graphics: Urticaria (Hives) and Angioedema

    Science.gov (United States)

    ... Conditions Drug Guide Conditions Dictionary Just for Kids Library School Tools Videos Virtual Allergist Education & Training Careers in ... Support the AAAAI Foundation Donate Utility navigation Español Journals Annual Meeting Member Login / My Membership Search navigation ...

  3. Burden of Illness in Hereditary Angioedema

    DEFF Research Database (Denmark)

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

    2015-01-01

    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 experienced 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 experienced worry and work/activity interruption during the attacks. In conclusion, a conceptual model was developed illustrating the hypothesized relationships among...

  4. The humanistic burden of hereditary angioedema

    DEFF Research Database (Denmark)

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

    2014-01-01

    and impact of HAE types I and II from the patient perspective. The HAE Burden of Illness Study in Europe was conducted in Spain, Germany, and Denmark to assess the real-world experience of HAE from the patient perspective via a one-time survey, which included items on clinical characteristics and physical......, traveling, and passing HAE to their children. Based on Hospital Anxiety and Depression Scale scores, 38 and 14% had clinically meaningful anxiety and depression, respectively. Despite standard of care, HAE patients still have frequent and painful attacks. Patients experience substantial impairment...

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

    DEFF Research Database (Denmark)

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

    2016-01-01

    , 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...... of this study was to describe safety and usage patterns of pnfC1-INH. METHODS: A multicenter, observational, registry was conducted between 2010 and 2014 at 30 United States and 7 European sites to obtain both prospective (occurring after enrollment) and retrospective (occurring before enrollment) safety...... and usage data on subjects receiving pnfC1-INH for any reason. RESULTS: Of 343 enrolled patients, 318 received 1 or more doses of pnfC1-INH for HAE attacks (11,848 infusions) or for prophylaxis (3142 infusions), comprising the safety population. Median dosages per infusion were 10.8 IU/kg (attack treatment...

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

    African Journals Online (AJOL)

    approach, and then touch on current guidelines and recent developments ... as is evident in the family name of the Urticaceae stinging .... patients respond to treatment.1,2 A single cause is seldom found .... desloratidine, fexofenadine or rupatidine, as first line therapy.16 ... Cancer Risk in Patients with Chronic Urticaria: A.

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

    DEFF Research Database (Denmark)

    Longhurst, Hilary; Bygum, Anette

    2016-01-01

    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...

  8. Benefits and risks of danazol in hereditary angioedema

    DEFF Research Database (Denmark)

    Bork, Konrad; Bygum, Anette; Hardt, Jochen

    2008-01-01

    the benefits and risks of long-term treatment with danazol. METHODS: Data were generated retrospectively from 118 German and Danish patients who had HAE due to C1 inhibitor deficiency and were treated with danazol from 2 months to 30 years. The frequency and severity of acute attacks were registered before......, headache, depression, and/or liver adenomas) occurred in 93 of the 118 patients and led to discontinuation of danazol therapy in 30 patients. CONCLUSIONS: Danazol is highly beneficial in patients with frequent and severe attacks of HAE. Because the risk of adverse effects is high, close monitoring...

  9. Guía Argentina de urticaria y angioedema

    Directory of Open Access Journals (Sweden)

    Jorge Máspero

    2014-08-01

    Full Text Available Se actualiza el diagnóstico de la urticaria crónica (UC y los conceptos, definiciones y sugerencias basados en la evidencia para su tratamiento. La urticaria ocurre en al menos 20% de la población en algún momento de la vida. Su etiología difiere en la forma aguda (menos de 6 semanas, y en la crónica. No es posible pronosticar si las formas agudas evolucionarán a UC, ya que todas son agudas al comienzo. La UC ocurre como espontánea (UCE o inducible (UCI. El diagnóstico es sencillo, pero incluye un minucioso estudio para descartar diagnósticos diferenciales; para UCI son útiles las pruebas de provocación en la caracterización y manejo. Los estudios complementarios se deben limitar y orientar según sospecha clínica. El tratamiento se divide en tres enfoques: evitación, eliminación o tratamiento del estímulo desencadenante o de la causa, y tratamiento farmacológico. Recientemente éste se modificó, con empleo de antihistamínicos de segunda generación como primera línea y aumento de dosis de antihistamínicos H1 no sedantes, hasta 4 veces, como segunda línea. Los antihistamínicos son fundamentales para tratar la UC; sin embargo, un 40% de los pacientes no logra un buen control pese al aumento de dosis y requiere otro medicamento adicional. La evidencia más reciente considera que un grupo de fármacos puede utilizarse como tercera línea en estos casos, para mejorar la calidad de vida y limitar la toxicidad por el uso frecuente o crónico de esteroides sistémicos. Se recomiendan para esta tercera línea solo 3 fármacos: omalizumab, ciclosporina A o antileucotrienos.

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

    African Journals Online (AJOL)

    The complication can be life threatening with serious morbidity and mortality if not promptly diagnosed from drug history and properly handled within the emergency unit. Apart from taking drug history concerning ACE inhibitor use in patients with heart failure, coronary heart disease and hypertension, a history of angiotensin ...

  11. Hereditary angioedema by C1 inhibitor-deficit: Diagnostic and therapeutic challenges. Case report

    Directory of Open Access Journals (Sweden)

    Álvaro José Mayorga

    2017-12-01

    Conclusion: The delay diagnosis involves considerable risk in these patients; the importance of long-term prophylactic treatment is ratified in the use of androgens, being as an available option in developing countries.

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

    DEFF Research Database (Denmark)

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

    2015-01-01

    . This was evident regardless whether we measured factor XIIa-C1-INH or kallikrein-C1-INH complexes, and the two assays were in close agreement. By contrast, testing the same samples utilizing the commercial method (complex ELISA, Quidel Corp.) revealed levels of C1-INH between 0 and 57% of normal (mean, 38%) and 42...

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

    DEFF Research Database (Denmark)

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

    2017-01-01

    at onset was 4 [1-11] years. The first attack was peripheral in 8/14 children and abdominal in 6/14 children, i.e. no one had their first attacks in the upper airways. Most children had less than one attack per month. All of the symptomatic children had been treated with tranexamic acid and/or C1 inhibitor...

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

    DEFF Research Database (Denmark)

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

    2016-01-01

    . OBJECTIVE: To evaluate the history of misdiagnosis in patients participating in the Icatibant Outcome Survey (IOS). METHODS: The IOS is an observational study in which safety and effectiveness of icatibant have been evaluated since 2009. As part of the IOS, patients record any misdiagnoses received before...

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

    DEFF Research Database (Denmark)

    Aabom, Anne; Nguyen, Dan; Fisker, Niels

    2017-01-01

    have considerable impact on the health-related quality of life (HRQoL) in adult patients. Half the patients with C1-INH-HAE develop symptoms before the age of 10 years. However, the HRQoL in children with C1-INH-HAE is almost unexplored. Objective: To investigate HRQoL in Danish children with C1...... were the PedsQL (Child Self-Report and Parent Proxy-Report forms); the Children's Dermatology Life Quality Index; a nonvalidated, diseasespecific quality-of-life questionnaire; and two visual analog scales that rated general health. Results: The HRQoL scores in our study were comparable with the normal...... the Parent Proxy-Report form carried the disease. Conclusion: Overall, the children assessed on average had a normal HRQoL and better than those with other common skin disorders. However, according to our findings, health care providers should be especially attentive to HRQoL when children with C1-INH...

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

    OpenAIRE

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

    2017-01-01

    Patient: Male, 32 Final Diagnosis: Miller Fisher syndrome Symptoms: Ataxia ? headache ? ophthalmoplegia Medication: ? Clinical Procedure: Plasmapheresis Specialty: Neurology Objective: Rare co-existance of disease or pathology Background: Miller Fisher Syndrome is characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia, and is considered to be a variant of Guillain-Barre Syndrome. Miller Fisher Syndrome is observed in approximately 1?5% of all Guillain-Barre cases in Wes...

  17. 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 TCC was lower (P TCC...

  18. Disease: H01006 [KEGG MEDICUS

    Lifescience Database Archive (English)

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

  19. Elucidating the Mechanism of Gain of Toxic Function From Mutant C1 Inhibitor Proteins in Hereditary Angioedema

    Science.gov (United States)

    2017-10-01

    antibodies to 5 specifically blot wild-type C1INH in the pathologic polymers.. A FLAG tag was placed into the wild-type C1INH cDNA located immediately...resulted in decreased secretion of the 3x-FLAG-WT-C1INH when cotransfected with the mutant cDNA . This was an important confirmation of our...C1INH plus mutant C1INH cDNA in the presence or absence of a lactacystin, a proteasome inhibitor. As shown in figure 2, blocking degradation of

  20. Long-term safety of icatibant treatment of patients with angioedema in real-world clinical practice

    DEFF Research Database (Denmark)

    Zanichelli, A; Maurer, M; Aberer, W

    2017-01-01

    . A total of 143 SAEs occurred in 59 (10.6%) patients; only three events (drug inefficacy, gastritis, and reflux esophagitis) in two patients were considered related to icatibant. Notably, no SAEs related to icatibant occurred in patients with cardiovascular disease, nor in those using icatibant...

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

    DEFF Research Database (Denmark)

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

    2017-01-01

    : The symptoms of C1-INH-HAE often present in childhood. Differential diagnosis can be difficult as abdominal pain is common in pediatric C1-INH-HAE, but also commonly occurs in the general pediatric population. The early onset of symptoms may predict a more severe subsequent course of the disease. Before...

  2. Disease: H00106 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available : C1 inhibitor deficiency (hereditary angioedema); C4 binding protein alpha deficiency; C4 binding protein b... subcutaneous and submucosal layers, identified as angioedema (hereditary or acquired). Genetic deficiency o

  3. 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

    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...

  4. Anaphylaxis Due to Head Injury

    Directory of Open Access Journals (Sweden)

    Bruner, Heather C.

    2015-05-01

    Full Text Available Both anaphylaxis and head injury are often seen in the emergency department, but they are rarely seen in combination. We present a case of a 30-year-old woman who presented with anaphylaxis with urticaria and angioedema following a minor head injury. The patient responded well to intramuscular epinephrine without further complications or airway compromise. Prior case reports have reported angioedema from hereditary angioedema during dental procedures and maxillofacial surgery, but there have not been any cases of first-time angioedema or anaphylaxis due to head injury. [West J Emerg Med. 2015;16(3:435–437.

  5. Anaphylaxis due to head injury.

    Science.gov (United States)

    Bruner, Heather C; Bruner, David I

    2015-05-01

    Both anaphylaxis and head injury are often seen in the emergency department, but they are rarely seen in combination. We present a case of a 30-year-old woman who presented with anaphylaxis with urticaria and angioedema following a minor head injury. The patient responded well to intramuscular epinephrine without further complications or airway compromise. Prior case reports have reported angioedema from hereditary angioedema during dental procedures and maxillofacial surgery, but there have not been any cases of first-time angioedema or anaphylaxis due to head injury.

  6. Disease: H01799 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H01799 Vibratory urticaria; Vibratory angioedema Vibratory urticaria is a rare typ...e of physical skin reactivity characterized by the occurrence of local erythematous, edematous, cutaneous an...enship ML, Pruzansky JJ ... TITLE ... Vibratory angioedema: a hereditary type of ph

  7. Anaphylaxis in children

    African Journals Online (AJOL)

    EL-HAKIM

    radiocontrast dyes and dialysis membranes).4 ... proteins, affects as many as 6% of young children and 3% to 4% of adults and ... angioedema) caused by eating certain plant-derived foods. This is ... plasma histamine levels and non-allergic.

  8. Gene : CBRC-OANA-01-1559 [SEVENS

    Lifescience Database Archive (English)

    Full Text Available |Oan#S38831641 PREDICTED: Ornithorhynchus anatinus similar to serpin peptidase inhibitor, clade G (C1 inhibitor), member 1, (angioede...ma, hereditary), (LOC100079402), partial mRNA /cds=p(1,9

  9. Gene : CBRC-OANA-01-0477 [SEVENS

    Lifescience Database Archive (English)

    Full Text Available |Oan#S38831641 PREDICTED: Ornithorhynchus anatinus similar to serpin peptidase inhibitor, clade G (C1 inhibitor), member 1, (angioede...ma, hereditary), (LOC100079402), partial mRNA /cds=p(1,9

  10. Formaldehyd i tekstil som mulig årsag til arthritis og angioødem

    DEFF Research Database (Denmark)

    Jensen, O C; Bach, B

    1992-01-01

    A case of arthritis and angioedema which developed on occupational exposure to formaldehyde in textiles is described. Possible pathological mechanisms are discussed. The suspicion that an unknown immunological reaction may be the cause is raised....

  11. Safety of Oral Paracetamol – Analysis of Data from a Spontaneous ...

    African Journals Online (AJOL)

    (DOAJ), African Journal Online, Bioline International, Open-J-Gate and Pharmacy Abstracts ... pharmaceutical market in 1950 under the name .... reported 25 cases of hypersensitivity in children ... food allergy, idiopathic angioedema, acquired.

  12. Odynophagia following retained bee stinger

    Directory of Open Access Journals (Sweden)

    S Viswanathan

    2012-01-01

    Full Text Available Nearly half of Hymenoptera stings affect the head and neck region of victims, but reports on oropharyngeal bee stings are very few. We describe the case of a patient with odynophagia and suffocation in mass envenomation. He had a retained bee stinger whose removal was delayed for more than 24 hours following the sting, due to persisting angioedema. Odynophagia receded after removal of the stinger and treatment with paracetamol, steroids and metronidazole. The patient also developed rhabdomyolysis, renal failure and hepatitis that were treated with conservative therapy. Oropharyngeal stings can simulate symptoms of persisting angioedema in victims of mass envenomation.

  13. Cutaneous findings in five cases of malaria

    Directory of Open Access Journals (Sweden)

    Jignesh B Vaishnani

    2011-01-01

    Full Text Available Malaria is an infectious disease caused by protozoa of the genus Plasmodium. Cutaneous lesions in malaria are rarely reported and include urticaria, angioedema, petechiae, purpura, and disseminated intravascular coagulation (DIC. Here, five malaria cases associated with cutaneous lesions have been described. Out of the five cases of malaria, two were associated with urticaria and angioedema, one case was associated with urticaria, and other two were associated with reticulated blotchy erythema with petechiae. Most of the cutaneous lesions in malaria were nonspecific and reflected the different immunopathological mechanism in malarial infection.

  14. Autoimmune progesterone dermatitis in a patient with endometriosis: case report and review of the literature

    OpenAIRE

    Baptist, Alan P; Baldwin, James L

    2004-01-01

    Abstract Autoimmune progesterone dermatitis (APD) is a condition in which the menstrual cycle is associated with a number of skin findings such as urticaria, eczema, angioedema, and others. In affected women, it occurs 3–10 days prior to the onset of menstrual flow, and resolves 2 days into menses. Women with irregular menses may not have this clear correlation, and therefore may be missed. We present a case of APD in a woman with irregular menses and urticaria/angioedema for over 20 years, w...

  15. HAE international home therapy consensus document

    DEFF Research Database (Denmark)

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

    2010-01-01

    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 ...

  16. HAE international home therapy consensus document

    NARCIS (Netherlands)

    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

    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

  17. Analysis of characteristics associated with reinjection of icatibant

    DEFF Research Database (Denmark)

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

    2015-01-01

    PURPOSE: Phase 3 icatibant trials showed that most hereditary angioedema (HAE) (C1 inhibitor deficiency) acute attacks were treated successfully with one injection of icatibant, a selective bradykinin B2 receptor antagonist. We conducted a post hoc analysis of icatibant reinjection for HAE type I...

  18. Angiotensin-converting enzyme insertion/deletion polymorphism ...

    African Journals Online (AJOL)

    The clinical manifestations of KD include per- sistent fever, non-purulent conjunctivitis, diffuse muco- sal inflammation, polymorphous skin rashes, indurative angioedema of the hands and feet, and non-suppurative cervical lymphadenopathy3. In about 20% of patients vasculitis will lead to coronary artery lesions as detect-.

  19. Efficacy and safety of the angiotensin II receptor blocker losartan for hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Axelsson, Anna; Iversen, Kasper; Vejlstrup, Niels

    2015-01-01

    ·0001) confirmed drug compliance; blood pressure did not decrease in the placebo group. Two (2%) patients, both in the placebo group, died from sudden cardiac death during follow-up. In the losartan group, one (1%) patient had angioedema, one (1%) had deterioration of renal function, and one (1%) had hyperkalaemia...

  20. Quincke's oedema of the uvula associated with mucous retention cyst-a case report.

    Science.gov (United States)

    Arunachalam, P S; Ramachandra, C R

    2000-04-01

    We report a rare case of recurrent angioedema of uvula (Quincke's Oedema), causing air way obstruction. A brief review of literature and treatment options are alto included. The histoloigical specimen showed an associated mucous retention eyst, which in conjunction with Quinckc's oedema has not been previously reported.

  1. Quincke’s oedema of the uvula associated with mucous retention cyst-a case report

    OpenAIRE

    Arunachalam, P. S.; Ramachandra, C. R. S.

    2000-01-01

    We report a rare case of recurrent angioedema of uvula (Quincke’s Oedema), causing air way obstruction. A brief review of literature and treatment options are alto included. The histoloigical specimen showed an associated mucous retention eyst, which in conjunction with Quinckc’s oedema has not been previously reported.

  2. Early health technology assessments in pharmacogenomics: a case example in cardiovascular drugs

    NARCIS (Netherlands)

    Geenen, Joost W.; Baranova, Ekaterina V.; Asselbergs, Folkert W.; de Boer, Anthonius; Vreman, Rick A.; Palmer, Colin Na; Maitland-van der Zee, Anke H.; Hövels, Anke M.

    2017-01-01

    Aim: To assess the required characteristics (cost, sensitivity and specificity) of a pharmacogenomic test for being a cost-effective prevention of angiotensin-converting enzyme inhibitors induced angioedema. Furthermore, we assessed the influence of only testing high-risk populations. Materials &

  3. Omalizumab for Chronic Urticaria:

    DEFF Research Database (Denmark)

    Ghazanfar, Misbah Nasheela; Thomsen, Simon Francis

    2017-01-01

    Purpose of Review The purpose of this study was to review real-life studies on effectiveness and safety of omalizumab in chronic urticaria (CU). Recent Findings CU is an itching skin disease characterized by wheals, angioedema, or both (present >6 weeks). Omalizumab is a humanized anti...

  4. Role of tropomyosin as a cross-reacting allergen in sensitization to cockroach in patients from Martinique (French Caribbean island) with a respiratory allergy to mite and a food allergy to crab and shrimp

    NARCIS (Netherlands)

    Purohit, A.; Shao, J.; Degreef, J. M.; van Leeuwen, A.; van Ree, R.; Pauli, G.; de Blay, F.

    2007-01-01

    BACKGROUND: Tropomyosin has been described as cross-reacting allergen between mite, cockroach and shrimp. METHODS: In 13 patients with asthma and/or rhinitis sensitized to mite and/or German cockroach and presenting urticaria, oral allergy syndrome or angio-edema upon eating shrimp and/or crab, we

  5. The Sport’s Bar Grandpa: an unusual left temporo-mandibular and tongue pain

    Directory of Open Access Journals (Sweden)

    Paolo Leandri

    2014-09-01

    Full Text Available This case report describes the condition of an elderly patient admitted to hospital for a new onset headache and pain in the left temporo-mandibular joint, initially incorrectly interpreted as an angioedema, but that evolved into a tongue infarction.

  6. Anaphylaxis to Moringa oleifera: First description

    OpenAIRE

    Lucinda J. Berglund

    2018-01-01

    We describe the first reported case of repeated anaphylaxis after ingestion of Moringa oleifera, causing significant hypotension, angioedema and elevation of serum tryptase. Moringa oleifera seedpod was confirmed as the causative allergen by skin testing with the fresh pod. Moringa oleifera is widely consumed, both as a vegetable and in herbal medicines.

  7. Cold urticaria patients exhibit normal skin levels of functional mast cells and histamine after tolerance induction

    DEFF Research Database (Denmark)

    Kring Tannert, Line; Stahl Skov, Per; Bjerremann Jensen, Louise

    2012-01-01

    Cold urticaria is a skin condition characterized by rapid appearance of itchy wheals and occasionally angioedema in response to cold stimulation. Antihistamines do not sufficiently protect all patients from symptoms, even when used in higher than standard doses. In these patients, desensitization...

  8. Autoimmune progesterone dermatitis in a patient with endometriosis: case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Baldwin James L

    2004-08-01

    Full Text Available Abstract Autoimmune progesterone dermatitis (APD is a condition in which the menstrual cycle is associated with a number of skin findings such as urticaria, eczema, angioedema, and others. In affected women, it occurs 3–10 days prior to the onset of menstrual flow, and resolves 2 days into menses. Women with irregular menses may not have this clear correlation, and therefore may be missed. We present a case of APD in a woman with irregular menses and urticaria/angioedema for over 20 years, who had not been diagnosed or correctly treated due to the variable timing of skin manifestations and menses. In addition, we review the medical literature in regards to clinical features, pathogenesis, diagnosis, and treatment options.

  9. Clinical profile of HIV infection

    Directory of Open Access Journals (Sweden)

    Khopkar Uday

    1992-01-01

    Full Text Available HIV seropositivity rate of 14 percent was observed amongst STD cases. Heterosexual contact with prostitutes was the main risk factor. Fever, anorexia, weight loss, lymphadenopathy and tuberculosis were useful clinical leads. Genital ulcers, especially chancroid, were common in seropositivies. Alopecia of unknown cause, atypical pyoderma, seborrhea, zoster, eruptive mollusca and sulfa-induced erythema multiforme were viewed with suspicion in high risk groups. Purpura fulminans, fulminant chancroid, vegetating pyoderma and angioedema with purpura were unique features noted in this study.

  10. Dermatologic emergencies

    Directory of Open Access Journals (Sweden)

    M.P. Simón Díaz

    2016-01-01

    Full Text Available Dermatologic emergencies represent about 8–20% of the diseases seen in the Emergency Department of hospitals. It is often a challenge for primary care physicians to differentiate mundane skin ailments from more serious, life threatening conditions that require immediate intervention. In this review we included the following conditions: Stevens-Johnson syndrome/toxic epidermal necrosis, pemphigus vulgaris, toxic shock syndrome, fasciitis necrotising, angioedema/urticaria, meningococcemia, Lyme disease and Rocky Mountain spotted fever.

  11. AMINO ACID BLOOD POOL OF CHILDREN WITH ALLERGIC DISEASES

    Directory of Open Access Journals (Sweden)

    Shmulich O. V.

    2014-01-01

    Full Text Available The amino acid blood pool of children with atopic dermatitis, bronchial asthma, urticaria, angioedema was investigated. The variability of blood plasma amino acid content (tryptophan, histidine, tyrosine, cysteine, methionine was observed. The changes of histidine and tryptophan levels might be connected with the formation of biogenic amines, such as histamine, serotonine, with take part in the development of allergic reactions and inflammatory processes in organism.

  12. Hypersensitivity Reactions to Nonsteroidal Anti-Inflammatory Drugs: An Update

    OpenAIRE

    Sánchez-Borges, Mario; Caballero-Fonseca, Fernan; Capriles-Hulett, Arnaldo; González-Aveledo, Luis

    2010-01-01

    After beta lactam antibiotics, hypersensitivity reactions to nonsteroidal antiinflammatory drugs are the second cause of hypersensitivity to drugs. Acute manifestations affect the respiratory tract (aspirin exacerbated respiratory disease), the skin (urticaria and angioedema), or are generalized (anaphylaxis). Correct diagnosis and treatment in order to prevent unnecessary morbidity and the potential risk of death from these severe reactions, and to provide proper medical advice on future dru...

  13. Pattern of drug eruptions in a tertiary care hospital

    International Nuclear Information System (INIS)

    Tahir, Z.; Nadeem, N.; Aman, S.; Kazmi, A.H.

    2013-01-01

    Background: An adverse drug reaction is unintentional which occurs at doses used for prophylaxis, diagnosis or treatment. Objectives: To determine the frequency of various cutaneous drug eruptions that occur in patients in a tertiary care hospital setting. Patients and Methods: All patients with cutaneous drug eruptions seen at the Dermatology Department of Mayo Hospital, Lahore, over 6 months were enrolled and the pattern of drug eruptions like urticaria, angioedema, fixed drug eruption, maculopapular rash, erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis etc. were recorded, along with drugs that caused it. Results:A total of 160 patients (86 males, 74 females) were included in the study. Mean age of patients was 30.7+-15.4 years. Major eruptions were fixed drug eruption (21.3%) followed by urticaria without angioedema (10%), maculopapular rash (9.3%), lichenoid drug eruption (8.7%), acneiform drug eruption (7.5%), Stevens-Johnson syndrome (6.9%), vesiculobullous eruption (5.6%), erythema multiforme and eczematous eruption (5% each). Common drugs causing eruptions were sulfonamides (16.3%), followed by NSAIDs (14.4%), herbal and homeopathic medications (12.5%), penicillins (9.3%), tetracyclines (8.7%), antituberculous drugs, cephalosporins and antiepileptics (6.3% each). Conclusion: Fixed drug eruption and urticaria without angioedema were commonest eruptions while, sulfonamides and NSAIDs were the major causative drugs. Policy message: Reporting of adverse drug reactions is not done in Pakistan and needs to be done in each hospital. (author)

  14. Qualidade de vida em urticária crônica: inquérito em ambulatório público universitário, Botucatu (Brasil Quality of life in chronic urticaria: a survey at a public university outpatient clinic, Botucatu (Brazil

    Directory of Open Access Journals (Sweden)

    Maria Regina Cavariani Silvares

    2011-10-01

    Full Text Available OBJETIVO: Avaliar o impacto da urticária crônica na qualidade de vida dos pacientes de ambulatório universitário a partir do questionário DLQI (Dermatology Life Quality Index. MÉTODOS: Inquérito sobre o impacto na qualidade de vida infligido pela urticária crônica a partir do questionário DLQI validado para a língua portuguesa. Pacientes foram entrevistados durante suas consultas em ambulatório especializado, entre maio de 2009 e maio de 2010, em serviço público brasileiro (Botucatu-SP. Os escores do DLQI foram analisados segundo subgrupos: idade, gênero, escolaridade, tempo de doença e presença de angioedema. RESULTADOS: Foram entrevistados 100 pacientes com urticária crônica. Predominou o gênero feminino (86%, a idade média foi de 41,8 anos, duração média da doença foi de seis anos e angioedema ocorreu em 82% dos pacientes. O escore médio do DLQI foi de 13,5, caracterizando grave impacto à qualidade de vida, superior a hanseníase, psoríase, eczema atópico e carcinoma basocelular. Presença de angioedema se associou a maiores escores: 14,5 x 9,9 (p OBJECTIVE: To evaluate the impact of chronic urticaria on quality of life of outpatients through the university questionnaire Dermatology Life Quality Index (DLQI. METHODS: Survey of the impact on quality of life caused by chronic urticaria, using the DLQI questionnaire validated for the Portuguese language. Patients were interviewed during visits to a specialized outpatient clinic between May 2009 and May 2010 at a Brazilian public service (Botucatu-SP. DLQI scores were analyzed according to the following subgroups: age, gender, education, disease duration, and presence of angioedema. RESULTS: We interviewed 100 patients with chronic urticaria. There was a female predominance (86%, mean age 41.8 years, mean disease duration of 6 years, and angioedema occurrence in 82% of patients. The mean DLQI score was 13.5, characterized by serious impact on quality of life, higher

  15. Estudo da qualidade de vida nos pacientes com urticária crônica Quality of life assessment in patients with chronic urticaria

    Directory of Open Access Journals (Sweden)

    Ana Paula Fusel de Ue

    2011-10-01

    Full Text Available FUNDAMENTOS: A urticária crônica compromete o doente por interferir nas atividades diárias, prejudicar a autoestima e as relações interpessoais. Os profissionais de saúde subestimam seu impacto na qualidade de vida dos doentes. OBJETIVOS: Avaliar a qualidade de vida com questionário específico e genérico. Compará-la entre os tipos clínicos de urticária crônica e avaliar se o angioedema piora a qualidade de vida. MÉTODOS: Participaram 62 doentes com urticária crônica, com sinais e sintomas da doença até 7 dias da consulta, que foram divididos em urticária crônica comum, urticária crônica física e urticária crônica mista. RESULTADOS: Observou-se predominância de mulheres (72,6%, idade média de 39,8 anos, angioedema associado em 75,8% dos doentes. Apresentaram mais angioedema doentes com urticária crônica comum (p=0,011 e mulheres (p=0,024. Quanto aos tipos clínicos, 32,3% apresentaram urticária crônica comum, 27,4% urticária crônica física e 40,3% urticária crônica mista. O escore médio total do questionário específico foi 10,4. No questionário específico, os domínios mais comprometidos foram "Sintomas e sentimentos" e "Atividades diárias", e, no SF-36, "Aspectos físicos" e "Vitalidade". Houve comprometimento da qualidade de vida nas mulheres, nos doentes com até 30 anos, em primeira consulta, nos mais escolarizados, naqueles com até 1 ano de doença e naqueles com angioedema. CONCLUSÃO: A urticária crônica compromete a qualidade de vida medida pelos questionários específico e genérico. Não houve diferença estatisticamente significante na qualidade de vida entre os tipos clínicos. A presença do angioedema conferiu pior qualidade de vida aos doentes. Houve correlação estatisticamente significante entre os escores do questionário específico e do questionário genérico.BACKGROUND: Chronic urticaria affects patients by interfering with their daily activities, damaging their self-esteem and

  16. Effective treatment of different phenotypes of chronic urticaria with omalizumab: Case reports and review of literature.

    Science.gov (United States)

    Kasperska-Zajac, A; Jarząb, J; Żerdzińska, A; Bąk, K; Grzanka, A

    2016-06-01

    Despite the excellent efficacy and safety profile of omalizumab in chronic spontaneous urticaria (CSU), there are scarce data concerning its role in the treatment of refractory cases with different phenotypes of urticaria. We describe our experience with the therapy of nine patients with CSU co-existing with delayed pressure urticaria (DPU) or angioedema or both and refractory to treatment with high-dose antihistamines. The first patient, with severe CSU and recurrent angioedema, did not respond well to cyclosporine A or corticosteroids and suffered from numerous side effects of long-term corticosteroid therapy. The second patient presented with severe symptoms of DPU, which first of all prevented any daily activities of the professional routines. Both patients showed a complete remission of urticaria after the first injection of omalizumab. The third patient with CSU and severe DPU had been ineffectively treated for more than 20 years with various medications. Following the administration of omalizumab, the symptoms of CSU subsided but those of DPU intensified, and the drug was withdrawn after two cycles. In another four patients with refractory CSU and angioedema, the symptoms subsided after the first administration of omalizumab, and the patients have been in remission for about 5 weeks. In the remaining two patients, the symptoms did not resolve despite four 300 mg doses of omalizumab. It is important to establish a therapeutic regimen with omalizumab (150-300 mg; every 4-8 weeks) tailored to individual patient's needs and dependent on the type of urticaria; this may minimize unnecessary the medication exposure, adverse drug effects, and healthcare costs. © The Author(s) 2015.

  17. A case report: Nursing interventions on a patient with anaphylactic shock in ICU.

    Science.gov (United States)

    Zariquiey-Esteva, G; Santa-Candela, P

    The care plan of a 42-year-old woman with anaphylactic shock, secondary to ingestion of amoxicillin/clavulanic acid, with upper airway involvement due to laryngeal angioedema, is presented. Previously she had had two episodes of angioedema of unknown origin. The incidence of this phenomenon is between 3.2 and 10 cases/100,000 people/year. An evaluation was made and three altered necessities stood out: breathing and circulation (she needed mechanical ventilation and noradrenalin perfusion), elimination (she required furosemide to keep an acceptable diuresis time), and hygiene and skin protection (she presented generalised hyperaemia, lip, lingual and oropharyngeal oedema). The hospital's Clinical Research Ethics Committee requested the patient's informed consent to access her clinical history. According to the altered necessities, seven diagnoses were prioritised according to NANDA taxonomy: risk of allergic response, risk of infection, risk of ineffective renal perfusion, decreased cardiac output, impaired spontaneous ventilation, risk of unstable blood glucose level, and risk of dysfunctional gastrointestinal motility. Scores of outcome criteria showed a favourable evolution after 24hours. The development of a standardised NANDA-NOC-NIC language in the clinical case presented allowed us to organise the nursing work, facilitating recording and normalising clinical practice. As a limitation of this case, we didn't have serial plasma levels of histamine and tryptase to assess the evolution of anaphylactic symptoms. Highlight the importance of health education in a patient with a history of angioedema. Copyright © 2016 Sociedad Española de Enfermería Intensiva y Unidades Coronarias (SEEIUC). Publicado por Elsevier España, S.L.U. All rights reserved.

  18. Cetirizine-Induced atrial fibrillation

    Directory of Open Access Journals (Sweden)

    Altuğ Osken

    2016-01-01

    Full Text Available Atrial fibrillation (AF is the most common observed arrhythmia in clinical practice. In the literature, AF events associated with drug induction are available. Cetirizine is a second-generation histamine antagonist used in the treatment of allergies, angioedema, and urticaria. We wish to present an atypical case who took cetirizine medication for relieving symptoms of upper tract respiratory system infection, experienced rapid ventricular response AF and treated successfully. To best of our knowledge, this is the first case of cetirizine-induced AF.

  19. Factor XII-independent activation of the bradykinin-forming cascade

    DEFF Research Database (Denmark)

    Joseph, Kusumam; Tholanikunnel, Baby G; Bygum, Anette

    2013-01-01

    and assayed for kallikrein formation. C1-INH was removed from factor XII-deficient plasma by means of immunoadsorption. RESULTS: We demonstrate that prekallikrein-HK will activate to kallikrein in phosphate-containing buffers and that the rate is further accelerated on addition of heat shock protein 90...... the prekallikrein-HK complex to prevent HK cleavage either by prekallikrein or by prekallikrein-HK autoactivation to generate kallikrein. In patients with hereditary angioedema, kallikrein and bradykinin formation can occur without invoking factor XII activation, although the kallikrein formed can rapidly activate...

  20. Urticária e doenças sistêmicas

    Directory of Open Access Journals (Sweden)

    R. F. J. Criado

    1999-12-01

    Full Text Available Chronic urticaria and concurrent angioedema are disappoiting problems for both physicians and patients. The disease can result from multiple causes and probably does not have a single etiology. Several factors have been identified that appear to be important in the pathogenesis of individual cases, some drugs, food additives, physical factors and internal diseases. In some cases no pathogenesis are identified and those cases are classified as idiopathic. In recent years several articles has emphasized autoimmunity and infections due to Helicobacter pylori. Our article reviewed the etiology of chronic urticaria at current concepts.

  1. Successful and Safe Treatment of Chronic Spontaneous Urticaria with Omalizumab in a Woman during Two Consecutive Pregnancies

    DEFF Research Database (Denmark)

    Ghazanfar, Misbah Nasheela; Thomsen, Simon Francis

    2015-01-01

    Chronic spontaneous urticaria is an itching skin disease characterised by wheals, angioedema, or both present for more than six weeks. Omalizumab is a humanized anti-IgE monoclonal antibody recently approved for treatment of chronic urticaria. Several randomised controlled trials have investigated...... the safety, tolerability, and efficacy of omalizumab for chronic urticaria. The safety of omalizumab in pregnancy is not known. We describe a female patient with chronic spontaneous urticaria who was treated with omalizumab continuously through two consecutive pregnancies with convincing results...

  2. Successful and Safe Treatment of Chronic Spontaneous Urticaria with Omalizumab in a Woman during Two Consecutive Pregnancies

    Directory of Open Access Journals (Sweden)

    Misbah Nasheela Ghazanfar

    2015-01-01

    Full Text Available Chronic spontaneous urticaria is an itching skin disease characterised by wheals, angioedema, or both present for more than six weeks. Omalizumab is a humanized anti-IgE monoclonal antibody recently approved for treatment of chronic urticaria. Several randomised controlled trials have investigated the safety, tolerability, and efficacy of omalizumab for chronic urticaria. The safety of omalizumab in pregnancy is not known. We describe a female patient with chronic spontaneous urticaria who was treated with omalizumab continuously through two consecutive pregnancies with convincing results and no apparent toxicity.

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

    DEFF Research Database (Denmark)

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

    2015-01-01

    of suffocation, worry about their children inheriting HAE, and medication side effects. Data were analyzed using Wilcoxon signed-rank tests or analysis of variance. Responses were obtained from 134 self-identified HAE subjects: 85 type I, 21 type II, and 28 with normal C1 inhibitor (C1INH). Burden of disease......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...

  4. Blepharochalasis

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    Özlem Bilgiç

    2012-12-01

    Full Text Available Blepharochalasis is a disorder characterized by recurrent episodes of painless edema of the eyelid skin, which leads to atrophy and relaxation of the eyelid structures and ptosis. The condition typically manifests itself in childhood or adolescence. The author reported a 16-year-old girl who has been suffered from recurrent painless swelling episodes on the upper eyelids for three years and laxity and wrinkling predominantly on the right upper eyelid for one year. This case was presented for the disorder is a rarely encountered disease and may easily misdiagnosed as recurrent angioedema, especially in its early stage.

  5. Perioperative Management of a Patient with Cold Urticaria

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    Priscilla Agbenyefia

    2017-12-01

    Full Text Available Cold urticaria consists of an allergic immune response to cold temperatures with symptoms ranging from pruritic wheals to life-threatening angioedema, bronchospasm, or anaphylactic shock. Adequate planning to maintain normothermia perioperatively is vital due to impaired hypothalamic thermoregulation and overall depression of sympathetic outflow during deep sedation and general anesthesia. This case report describes the successful perioperative management of a 45-year-old female with a history of cold urticaria undergoing a laparoscopic Nissen fundoplication for refractory gastroesophageal reflux disease and discusses how to appropriately optimize the care of these patients.

  6. Bivirkninger ved N-acetylcystein-behandling af paracetamolforgiftede patienter

    DEFF Research Database (Denmark)

    Schmidt, L E; Dalhoff, K P

    1999-01-01

    number of patients will be treated with NAC than with previous regimens based on plasma concentrations of paracetamol. In this retrospective study we evaluated the incidence of side effects of NAC in 310 patients admitted to the Department of Hepatology, Rigshospitalet, Copenhagen, over a four-year...... period (1.1.1994-31.12.1997). Twenty-six (8.4%) patients developed side effects. Side effects were anaphylactoid, mainly from skin (25 rash, pruritus or flushing), in rare cases more serious (four bronchospasm, three angioedema, one hypotension). None were life-threatening and all patients received...

  7. Clinical Evaluation of Inpatients with Acute Urticaria

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    Ayşe Serap

    2011-12-01

    Full Text Available Background and Design: To determine the clinical and etiological features of inpatients with acute urticaria and angioedema and to assess the need for laboratory tests. Material and Methods: We recruited 105 patients with acute urticaria and angioedema who were admitted to our inpatient unit. The lesions and the characteristics of the patients were analyzed. Routine diagnostic tests including complete blood count, thyroid function tests, hepatitis panel, stool parasite, total IgE levels, cultures, erythrocyte sedimentation rate, C-reactive protein, anti-nuclear antibody, and posterior anterior lung X-ray were ordered. A psychiatric consultation was obtained, when needed. The results were analyzed with SPSS 15.0 statistical software.Results: Among 105 patients, 28 (26.7% had urticaria, 7 (6.7% had angioedema, and 70 (66.7% suffered from both urticaria and angioedema. The most common accompanying symptoms were itching (91.4% and burning (34.3%. The most common systemic symptoms were fatigue (15.2% and headache (12.4%. The lesions usually appeared in the evening hours (24.8%. Twenty-five patients were waking up due to itching during the night. Some lesions were associated with physical activities. Systemic diseases accompanied the lesions in 12 patients (11%. In terms of etiological factors, 33 patients (22.5% had infections. Food- related lesions were encountered in 14 (13% patients. Thirty patients (28.5% had history of medication use. Stress was detected in 37.1% of the patients; anxiety was diagnosed in 3% of patients. The stool was positive for parasites in 10 (9% patients. Conclusion: Acute urticaria is a benign disorder. Although the underlying cause of urticaria can not always be identified, infections and medications are the most common causes. A comprehensive and detailed history is very important to discover the underlying cause. The diagnostic tests should be ordered according to the patient’s history. Conducting diagnostic tests

  8. Different clinical allergological features of Taenia solium infestation.

    Science.gov (United States)

    Minciullo, Paola Lucia; Cascio, Antonio; Isola, Stefania; Gangemi, Sebastiano

    2016-01-01

    The tapeworm Taenia ( T. ) solium can be responsible for two different conditions: taeniasis and cysticercosis. Helminth infections in human host cause an immune response associated with elevated levels of IgE, tissue eosinophilia and mastocytosis, and with the presence of CD4+ T cells that preferentially produce IL-4, IL-5, and IL-13. Individuals exposed to helminth infections may have allergic inflammatory responses to parasites and parasite antigens. PubMed search of human cases of allergic reactions occurring during T. solium infestation was performed combining the terms (allergy, urticaria, angioedema, asthma, anaphylaxis) with T. solium . A study was considered eligible for inclusion in the review if it reported data on patients with T. solium infestation who had signs or symptoms of allergy. In literature we found six articles reporting the association between an allergic reaction and T. solium infestation: two cases of urticaria, two cases of relapsing angioedema, one case of asthma and two cases of anaphylaxis. Despite the large diffusion of T. solium infestation, we found only a few cases of concomitant allergic reaction and the presence of Taenia in the host. The association between T. solium infestation and allergic manifestations has never been clearly demonstrated, and in absence of a well-documented causality the hypotheses are merely speculative. Therefore, the association between Taenia infection and allergy needs to be thoroughly studied to better clarify if this association may really exist and which is the pathogenetic mechanism supported.

  9. Omalizumab for treating chronic spontaneous urticaria: an expert review on efficacy and safety.

    Science.gov (United States)

    Giménez-Arnau, Ana M

    2017-03-01

    Chronic spontaneous urticaria (CSU) is characterized by the recurrence of itchy hives and/or angioedema for greater than six weeks, with no known external trigger. Omalizumab, a humanized, recombinant, monoclonal anti-IgE antibody, is the only approved add-on therapy for H1-antihistamine refractory CSU patients. Areas covered: The objective of this article is to discuss the mechanism of action, pharmacokinetics and pharmacodynamics of omalizumab for the treatment of CSU. The review also summarizes efficacy and safety data from proof-of-concept, phase II (X-CUISITE, MYSTIQUE), and pivotal phase III omalizumab studies (ASTERIA I, ASTERIA II, and GLACIAL). Expert opinion: Omalizumab is a clinically effective and safe biological therapy for treating H1-antihistamine refractory CSU patients. It significantly reduces CSU symptoms (hives, itch and angioedema), and improves patient health-related quality of life. While omalizumab is already integral to the treatment of antihistamine refractory CSU, widespread use will depend on legal and economic factors, as well as improvements in the early and accurate diagnosis of CSU patients who would benefit from treatment.

  10. 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.

  11. Case of immediate hypersensitivity to beer.

    Science.gov (United States)

    Inoue, Tomoko; Yagami, Akiko; Shimojo, Naoshi; Hara, Kazuhiro; Nakamura, Masashi; Matsunaga, Kayoko

    2016-06-01

    We report here a case of immediate hypersensitivity to beer, in which a female patient developed angioedema of the eyelids shortly after consuming beer. In skin prick tests, the patient showed positive reactions to the base ingredients of beer, particularly malt and barley. The specific serum immunoglobulin E antibodies against barley and malt displayed weakly positive reactivity. To identify the immunoreactive antigens, malt and barley proteins were separated by 2-D polyacrylamide gel electrophoresis and immunoreacted with the patient's serum. The results of mass spectrometric analysis revealed that the main antigen was a protein with similarity to protein z-type serpin. Notably, the identified antigen had a molecular weight of 20-25 kDa, which is markedly smaller than that previously reported for protein Z4 (44 kDa). Taken together, these analyses indicate that a possible new antigen which belongs to the protein Z family elicits immediate hypersensitivity to beer. © 2015 Japanese Dermatological Association.

  12. Autoimmune Progesterone Dermatitis: A Diagnosis to Consider in a Patient With Cyclical Cutaneous Eruptions.

    Science.gov (United States)

    Steuer, Alexa B; Scherl, Sharon; Ashinoff, Robin

    2017-10-01

    Autoimmune progesterone dermatitis (APD) is a cyclical cutaneous reaction to progesterone, with symptoms that typically begin 3-10 days before the onset of menstrual flow and end 1-2 days into menses. The symptoms vary in severity from barely visible to anaphylaxis, but most often include an eczematous eruption, erythema multiforme, urticaria, folliculitis, and angioedema. This is a rare disorder with only a handful of documented cases. The pathogenesis of this condition remains unknown and significant variations in the presentation and severity of symptoms complicates its diagnosis. Treatment seeks to inhibit progesterone secretion through suppression of ovulation, but it may be unsuccessful. We present a case of autoimmune progesterone dermatitis that eluded diagnosis for several years, and subsequently the patient was completely controlled with oral contraceptive pills. J Drugs Dermatol. 2017;16(10):1040-1042..

  13. Potential hazards due to food additives in oral hygiene products.

    Science.gov (United States)

    Tuncer Budanur, Damla; Yas, Murat Cengizhan; Sepet, Elif

    2016-01-01

    Food additives used to preserve flavor or to enhance the taste and appearance of foods are also available in oral hygiene products. The aim of this review is to provide information concerning food additives in oral hygiene products and their adverse effects. A great many of food additives in oral hygiene products are potential allergens and they may lead to allergic reactions such as urticaria, contact dermatitis, rhinitis, and angioedema. Dental practitioners, as well as health care providers, must be aware of the possibility of allergic reactions due to food additives in oral hygiene products. Proper dosage levels, delivery vehicles, frequency, potential benefits, and adverse effects of oral health products should be explained completely to the patients. There is a necessity to raise the awareness among dental professionals on this subject and to develop a data gathering system for possible adverse reactions.

  14. Use of anti-TNFs for difficult-to-treat urticaria: response to Cooke et al

    Directory of Open Access Journals (Sweden)

    Thomsen SF

    2015-11-01

    Full Text Available Simon Francis Thomsen,1,2 Freja Lærke Sand1,2 1Department of Dermatology, Bispebjerg Hospital, Copenhagen, Denmark; 2Department of Biomedical Sciences, University of Copenhagen, Copenhagen, DenmarkWe read with interest the recent paper by Cooke et al about the use of biologic agents for intractable urticaria.1 Particularly, the authors reckon that the evidence supporting the use of anti-TNFs is limited by the small numbers of patients in non-controlled studies, often with urticarial disorders not typical of chronic urticaria such as vasculitis and delayed pressure urticaria. However, we want to draw the authors’ and readers’ attention to our report from 2013 about the use of adalimumab and etanercept in 20 patients with chronic urticaria with or without angioedema2 (updated in 2015 with an additional five patients.3View original article by Cooke et al 

  15. Adverse drug events in the oral cavity.

    Science.gov (United States)

    Yuan, Anna; Woo, Sook-Bin

    2015-01-01

    Adverse reactions to medications are common and may have a variety of clinical presentations in the oral cavity. Targeted therapies and the new biologic agents have revolutionized the treatment of cancers, autoimmune diseases, and inflammatory and rheumatologic diseases but have also been associated with adverse events in the oral cavity. Some examples include osteonecrosis, seen with not only bisphosphonates but also antiangiogenic agents, and the distinctive ulcers caused by mammalian target of rapamycin inhibitors. As newer therapeutic agents are approved, it is likely that more adverse drug events will be encountered. This review describes the most common clinical presentations of oral mucosal reactions to medications, namely, xerostomia, lichenoid reactions, ulcers, bullous disorders, pigmentation, fibrovascular hyperplasia, white lesions, dysesthesia, osteonecrosis, infection, angioedema, and malignancy. Oral health care providers should be familiar with such events, as they will encounter them in their practice. Copyright © 2015 Elsevier Inc. All rights reserved.

  16. Urticarial vasculitis induced by OTC diet pills: a case report.

    Science.gov (United States)

    Chérrez Ojeda, Iván; Loayza, Enrique; Greiding, Leonardo; Calderón, Juan Carlos; Cherrez, Annia; Adum, Farid

    2015-01-01

    Urticarial Vasculitis (UV) is in most of the cases idiopathic; however it has been associated with several conditions and drugs. Over the counter (OTC) diet pills are widely available, even on-line, but they are rarely regulated by pharmaceutical control. We present the case of a 35-year-old female patient suffering of pruriginous and painful wheals more than 1 cm in diameter, with a burning sensation. The eruption lasted more than 24 hours and was accompanied by angioedema, headache and myalgia. No remarkable medical history was found, except for previous intake of OTC diet pills. UV diagnosis was confirmed by the skin biopsy of a lesion. OTC diet pills are widely available worldwide, and due to its widespread use, allergologists and dermatologist should be able to recognize symptoms and lesions of cutaneous vasculitis, which may be under reported.

  17. Treatment of Severe Cold Contact Urticaria with Omalizumab: Case Reports

    Directory of Open Access Journals (Sweden)

    Petra Brodská

    2012-12-01

    Full Text Available We report 2 patients with cold urticaria with different response to treatment with omalizumab (Xolair®. Cold contact urticaria (CCU is a common subtype of physical urticaria. It is characterized by the development of wheal and/or angioedema within minutes after cold contact. Clinical manifestation of CCU can range from mild, localized whealing to life-threatening anaphylactic shock reactions. Omalizumab has been described to be useful in cases of chronic urticaria and may be an interesting option for treatment of CCU. We describe one patient with significant and long-lasting improvement of symptoms and one without any improvement after anti-immunoglobulin E therapy. In our case reports, we want to highlight that there is still a small group of patients without benefit from omalizumab treatment. It is necessary to identify this minor subgroup of patients where omalizumab does not represent an effective treatment possibility.

  18. Asthma and allergy in pregnancy.

    Science.gov (United States)

    Schatz, M; Zeiger, R S

    1997-06-01

    Rhinitis is extremely common during pregnancy, and asthma is one of the most common potentially serious medical problems to complicate pregnancy. Cutaneous allergy (urticaria/angioedema and eczema) also may occur during pregnancy. All of these entities may worsen with pregnancy in some patients and appear to improve in others. Uncontrolled asthma may directly threaten the fetus, and morbidity from the other illnesses may indirectly affect pregnancy through an effect on eating, sleeping, or emotional well-being. Appropriate diagnosis, avoiding triggering factors when possible; appropriate use of pharmacotherapy; and, when indicated, allergen immunotherapy usually allow these chronic conditions to be controlled during pregnancy so as to optimize both the health of the mother and that of her baby.

  19. The Journal of Allergy and Clinical Immunology: In Practice - 2016 Year in Review.

    Science.gov (United States)

    Schatz, Michael; Sicherer, Scott H; Zeiger, Robert S

    As editors, we concluded that it would be helpful to our readers to write a Year in Review article that highlights the Review, Original, and Clinical Communication articles published in 2016 in The Journal of Allergy and Clinical Immunology: In Practice. We summarized articles on the topics of asthma, rhinitis/rhinosinusitis, food allergy, anaphylaxis, drug allergy, urticarial/angioedema, eosinophilic disorders, and immunodeficiency. Within each topic, epidemiologic findings are presented, relevant aspects of prevention are described, and diagnostic and therapeutic advances are enumerated. Diagnostic tools described include history, skin tests, and in vitro tests. Treatments discussed include behavioral therapy, allergen avoidance therapy, positive and negative effects of pharmacologic therapy, and various forms of immunologic and desensitization management. We hope this review will help you, our readers, consolidate and use this extensive and practical knowledge for the benefit of your patients. Copyright © 2016 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  20. [Chronic urticaria in childhood : Rational diagnostics and treatment].

    Science.gov (United States)

    Ott, H

    2017-07-01

    Chronic urticaria (CU) is defined by episodes of urticaria with or without angioedema, which recur daily or nearly daily over more than 6 weeks. Sudden manifestations of CU with or without known causes are termed chronic spontaneous urticaria, which is differentiated from chronic inducible urticaria. The differential diagnoses of CU in childhood range from self-limiting dermatoses to severe systemic diseases. Further targeted steps are taken to detect potential trigger factors or underlying illnesses only if suspicion arises on anamnestic grounds and CU is best treated in accordance with international guidelines. First-line therapy consists of non-sedating H 1 -antihistamines at approved or even higher doses. If symptoms persist, additional treatment with omalizumab, cyclosporine or montelukast can be initiated after careful individual consideration.

  1. POTENTIAL HAZARDS DUE TO FOOD ADDITIVES IN ORAL HYGIENE PRODUCTS

    Directory of Open Access Journals (Sweden)

    Damla TUNCER-BUDANUR

    2016-04-01

    Full Text Available Food additives used to preserve flavor or to enhance the taste and appearance of foods are also available in oral hygiene products. The aim of this review is to provide information concerning food additives in oral hygiene products and their adverse effects. A great many of food additives in oral hygiene products are potential allergens and they may lead to allergic reactions such as urticaria, contact dermatitis, rhinitis, and angioedema. Dental practitioners, as well as health care providers, must be aware of the possibility of allergic reactions due to food additives in oral hygiene products. Proper dosage levels, delivery vehicles, frequency, potential benefits, and adverse effects of oral health products should be explained completely to the patients. There is a necessity to raise the awareness among dental professionals on this subject and to develop a data gathering system for possible adverse reactions.

  2. Interactions of carbon nanotubes and fullerenes with the immune system of the skin and the possible implications related to cutaneous nanotoxicity

    Directory of Open Access Journals (Sweden)

    Ana Luiza Castro Fernandes

    2013-11-01

    Full Text Available The understanding of the interaction of carbon nanotubes and fullerenes with the constituents of the skin, especially the skin immune unit, is relevant to the determina-tion of toxicological endpoints. A systematic review was done focused on such aspects. Considerable part of the found references concentrated in cytotoxicity and skin per-meation. On a smaller scale, there are articles on immunomodulation and activation of immune cells and other elements. Few of the found studies deal specifically with cutaneous immune response, limiting the related knowledge. The findings suggest that nanomaterials studied may be involved in skin problems such irritant contact dermatitis, anaphylactoid reactions, urticaria, angioedema, and raised the need for performing additional studies to confirm the findings. The standardization of the description and testing of nanomaterials characteristics used in experiments can facilitate comparison of results.

  3. IgE-mediated chlorhexidine allergy: a new occupational hazard?

    DEFF Research Database (Denmark)

    Nagendran, Vasantha; Wicking, Jennifer; Ekbote, Anjali

    2009-01-01

    BACKGROUND: Chlorhexidine is an effective antimicrobial agent commonly used in UK hospitals, primarily for skin decontamination. Recent UK infection control guidelines recommend the use of 2% chlorhexidine solution in specific clinical settings, thus increasing chlorhexidine use by health care...... those with possible clinical allergy to come forward for further testing. Diagnosis was based on an appropriate clinical history with positive serum-specific IgE to chlorhexidine and/or positive skin prick testing. RESULTS: Four cases of occupational IgE-mediated allergy to chlorhexidine were identified...... workers (HCWs). Chlorhexidine has been widely reported to cause IgE-mediated allergic reactions (from urticaria and angioedema to anaphylaxis) among patients undergoing surgery/invasive procedures. Despite its widespread use in health care settings, there are no reports of clinically confirmed...

  4. http://escholarship.org/uc/item/6sz1k5r3

    Directory of Open Access Journals (Sweden)

    Jonathan G. Wagner

    2015-12-01

    Full Text Available A 52-year-old African American male with a long history of poorly controlled hypertension presented to the emergency department (ED with two days of genital edema and pain. During ED work-up, the patient developed sudden onset of non-pitting, non-pruritic, and non-urticarial upper lip edema. Review of his antihypertensive medication list revealed that he normally took benazepril, highly suggestive of a diagnosis of angiotensin-converting-enzyme inhibitor-related angioedema (ACEIRA. We present the first reported case of penile ACEI-RA that progressed to involve the oropharynx. The ED management of the condition and some of the newer treatment options available for ACEIRA is also briefly discussed.

  5. Benazepril-Induced Agranulocytosis: A Case Report and Review of the Literature.

    Science.gov (United States)

    Hashmi, Hafiz Rizwan Talib; Jabbour, Rami; Schreiber, Zwi; Khaja, Misbahuddin

    2016-06-23

    Angiotensin-converting enzyme inhibitors are widely used drugs, and in appropriately selected patients, serious side effects are infrequent. Commonly seen side effects include cough, rash, hyperkalemia, renal dysfunction, and angioedema. Historically, dose-related agranulocytosis has been associated with captopril. Benazepril, a relatively more potent angiotensin-converting enzyme inhibitor, is rarely associated with agranulocytosis. Here, we report a case of drug-induced agranulocytosis due to benazepril, with complete recovery of white blood cell count upon discontinuation of the drug. All tests for other causes of agranulocytosis were unremarkable. This report highlights a serious and rare side effect associated with benazepril. Benazepril is a commonly employed anti-hypertensive medication, and we report an unusual condition associated with this medication in order to increase vigilance among caregivers. In such cases, prompt recognition and discontinuation of the causative drug can make the difference between a recovery and a fatal outcome associated with drug-induced agranulocytosis.

  6. Temporal Regulation by Innate Type 2 Cytokines in Food Allergies.

    Science.gov (United States)

    Graham, Michelle T; Andorf, Sandra; Spergel, Jonathan M; Chatila, Talal A; Nadeau, Kari C

    2016-10-01

    Food allergies (FAs) are a growing epidemic in western countries with poorly defined etiology. Defined as an adverse immune response to common food allergens, FAs present heterogeneously as a single- or multi-organ response that ranges in severity from localized hives and angioedema to systemic anaphylaxis. Current research focusing on epithelial-derived cytokines contends that temporal regulation by these factors impact initial sensitization and persistence of FA responses upon repeated food allergen exposure. Mechanistic understanding of FA draws insight from a myriad of atopic conditions studied in humans and modeled in mice. In this review, we will highlight how epithelial-derived cytokines initiate and then potentiate FAs. We will also review existing evidence of the contribution of other atopic diseases to FA pathogenesis and whether FA symptoms overlap with other atopic diseases.

  7. Beneficial Effect of Conversion to Belatacept in Kidney-Transplant Patients with a Low Glomerular-Filtration Rate

    Directory of Open Access Journals (Sweden)

    Julie Belliere

    2014-01-01

    Full Text Available Belatacept has been found to be efficient at preserving good kidney function in maintenance kidney-transplant patients. Herein, we report on the use of belatacept as a rescue therapy for two kidney-transplant patients presenting with severe adverse events after treatment with calcineurin inhibitors (CNIs and mammalian target-of-rapamycin (mTOR inhibitors. Two kidney-transplant patients developed severely impaired kidney function after receiving CNIs. The use of everolimus was associated with severe angioedema. Belatacept was then successfully used to improve kidney function in both cases, even though estimated glomerular-filtration rate before conversion was <20 mL/min. These case reports show that belatacept can be used as a rescue therapy, even if kidney function is very low in kidney-transplant patients who cannot tolerate CNIs and/or mTOR inhibitors.

  8. Chronic urticaria in the real-life clinical practice setting in Sweden, Norway and Denmark

    DEFF Research Database (Denmark)

    Thomsen, S F; Pritzier, E C; Anderson, C D

    2017-01-01

    ]; chronic urticaria quality of life questionnaire [CU-Q2 oL; Danish patients only]) and work productivity and activity impairment (WPAI) scores were also assessed. RESULTS: Overall, 158 CU patients from seven centres in Denmark (n = 80), Norway (n = 50) and Sweden (n = 28) were included in this baseline......BACKGROUND: Chronic urticaria (CU) is characterized by the recurrence of itchy hives and/or angioedema for more than 6 weeks. AWARE (A World-wide Antihistamine-Refractory Chronic Urticaria Patient Evaluation) is a multinational study designed to document the real-life treatment situation, burden...... (>2 months) that is refractory to H1-antihistamines. Baseline patient characteristics, disease activity (urticaria control test [UCT]), pharmacological treatment, comorbidities and healthcare usage were documented by the treating physician. Quality of life (QoL; dermatology life quality index [DLQI...

  9. Should the host reaction to anisakiasis influence the treatment?: Different clinical presentations in two cases

    Directory of Open Access Journals (Sweden)

    Stefano Pontone

    Full Text Available Gastrointestinal anisakiasis is a parasitic infection occurring in people that consume raw or inadequately cooked fish or squid. It is frequently characterized by severe epigastric pain, nausea and vomiting caused by the penetration of the larvae into the gastric wall. Acute gastric anisakiasis with severe chest discomfort is rarely reported in Italy. On the other hand, gastro-allergic anisakiasis with rash, urticaria and isolated angioedema or anaphylaxis is a clinical entity that has been described only recently. Also, if patients usually develop symptoms within 12 hours after raw seafood ingestion, not always endoscopic exploration can promptly identify the Anisakis larvae. Moreover, some authors consider the prevailing allergic reaction as a natural and effective defense against the parasitic attack. We report two cases of peculiar manifestations of anisakiasis in both acute and chronic forms (severe chest discomfort and anaphylactoid reaction.

  10. Should the host reaction to anisakiasis influence the treatment?: Different clinical presentations in two cases ¿Debe la reacción frente la anisakiasis influir sobre el tratamiento?: Presentación clínica

    Directory of Open Access Journals (Sweden)

    Stefano Pontone

    2012-12-01

    Full Text Available Gastrointestinal anisakiasis is a parasitic infection occurring in people that consume raw or inadequately cooked fish or squid. It is frequently characterized by severe epigastric pain, nausea and vomiting caused by the penetration of the larvae into the gastric wall. Acute gastric anisakiasis with severe chest discomfort is rarely reported in Italy. On the other hand, gastro-allergic anisakiasis with rash, urticaria and isolated angioedema or anaphylaxis is a clinical entity that has been described only recently. Also, if patients usually develop symptoms within 12 hours after raw seafood ingestion, not always endoscopic exploration can promptly identify the Anisakis larvae. Moreover, some authors consider the prevailing allergic reaction as a natural and effective defense against the parasitic attack. We report two cases of peculiar manifestations of anisakiasis in both acute and chronic forms (severe chest discomfort and anaphylactoid reaction.

  11. Endurance exercise after orange ingestion anaphylaxis

    Directory of Open Access Journals (Sweden)

    Manu Gupta

    2016-01-01

    Full Text Available Endurance exercise after orange ingestion cause anaphylaxis which is food-dependent exercise-induced anaphylaxis (FDEIA which is a form of exercise-induced anaphylaxis. In this article, an individual develops symptoms such as flushing, itching, urticaria, angioedema, and wheezing after eating a food allergen and proceeds to exercise. Neither the food alone nor exercise alone is sufficient to induce a reaction. This case report describes a 36-year-old asthmatic male athlete who experienced nausea, vomiting, flushing, urticaria, and facial swelling while exercising in a gymnasium after eating oranges. Neither oranges alone nor exercise alone induced the reaction. Total avoidance of suspected food allergens would be ideal. Persons with FDEIA should keep at hand an emergency kit with antihistamines, injectable rapid action corticoids, and adrenaline.

  12. Sacubitril/valsartan for heart failure with reduced left ventricular ejection fraction : A retrospective cohort study.

    Science.gov (United States)

    De Vecchis, R; Ariano, C; Di Biase, G; Noutsias, M

    2018-01-19

    The combination drug sacubitril/valsartan was reported to be superior to enalapril in reducing all-cause death, cardiovascular mortality, and heart failure (HF) hospitalizations in patients with cardiac insufficiency and reduced left ventricular ejection fraction (HFREF) with NYHA class II-IV. Our retrospective cohort study aimed to assess the effects of sacubitril/valsartan in addition to a beta-blocker and mineral receptor antagonist (MRA) in a group of HFREF patients with NYHA class II-III HF vs. conventional therapy (ACE inhibitor or angiotensin II receptor blocker added to a beta-blocker plus an MRA) administered to a control group of HFREF patients with comparable clinical features. In both groups, treatment was supplemented by a loop diuretic, usually furosemide, at variable doses. The primary outcomes were all-cause death and HF hospitalizations. Safety outcomes were symptomatic hypotension, angioedema, hyperkalemia, and worsening renal function. Mortality at 6 months was 6.8% in patients taking sacubitril/valsartan vs. 34% in those on conventional therapy (odds ratio [OR] = 0.14; 95% CI: 0.04-0.49). Moreover, there was a 4.5% rate of HF hospitalizations in the sacubitril/valsartan group vs. 59% in the control group (OR = 0.03; 95% CI: 0.01-0.14). Safety outcomes were comparable in the two groups, although hypotension (systolic blood pressure sacubitril/valsartan group vs. 5.7% in the control group (OR = 3.14; 95% CI: 0.94-10.55). Sacubitril/valsartan offered strong protection against all-cause death and HF hospitalizations at 6 months without any significant side effects. To validate this efficacious molecule, further postmarketing observational studies, focusing mainly on hypotension and angioedema are warranted.

  13. A modular, prospective, semi-automated drug safety monitoring system for use in a distributed data environment.

    Science.gov (United States)

    Gagne, Joshua J; Wang, Shirley V; Rassen, Jeremy A; Schneeweiss, Sebastian

    2014-06-01

    The aim of this study was to develop and test a semi-automated process for conducting routine active safety monitoring for new drugs in a network of electronic healthcare databases. We built a modular program that semi-automatically performs cohort identification, confounding adjustment, diagnostic checks, aggregation and effect estimation across multiple databases, and application of a sequential alerting algorithm. During beta-testing, we applied the system to five databases to evaluate nine examples emulating prospective monitoring with retrospective data (five pairs for which we expected signals, two negative controls, and two examples for which it was uncertain whether a signal would be expected): cerivastatin versus atorvastatin and rhabdomyolysis; paroxetine versus tricyclic antidepressants and gastrointestinal bleed; lisinopril versus angiotensin receptor blockers and angioedema; ciprofloxacin versus macrolide antibiotics and Achilles tendon rupture; rofecoxib versus non-selective non-steroidal anti-inflammatory drugs (ns-NSAIDs) and myocardial infarction; telithromycin versus azithromycin and hepatotoxicity; rosuvastatin versus atorvastatin and diabetes and rhabdomyolysis; and celecoxib versus ns-NSAIDs and myocardial infarction. We describe the program, the necessary inputs, and the assumed data environment. In beta-testing, the system generated four alerts, all among positive control examples (i.e., lisinopril and angioedema; rofecoxib and myocardial infarction; ciprofloxacin and tendon rupture; and cerivastatin and rhabdomyolysis). Sequential effect estimates for each example were consistent in direction and magnitude with existing literature. Beta-testing across nine drug-outcome examples demonstrated the feasibility of the proposed semi-automated prospective monitoring approach. In retrospective assessments, the system identified an increased risk of myocardial infarction with rofecoxib and an increased risk of rhabdomyolysis with cerivastatin years

  14. Gene interference regulates aquaporin-4 expression in swollen tissue of rats with cerebral ischemic edema: Correlation with variation in apparent diffusion coefficient.

    Science.gov (United States)

    Hu, Hui; Lu, Hong; He, Zhanping; Han, Xiangjun; Chen, Jing; Tu, Rong

    2012-07-25

    To investigate the effects of mRNA interference on aquaporin-4 expression in swollen tissue of rats with ischemic cerebral edema, and diagnose the significance of diffusion-weighted MRI, we injected 5 μL shRNA- aquaporin-4 (control group) or siRNA- aquaporin-4 solution (1:800) (RNA interference group) into the rat right basal ganglia immediately before occlusion of the middle cerebral artery. At 0.25 hours after occlusion of the middle cerebral artery, diffusion-weighted MRI displayed a high signal; within 2 hours, the relative apparent diffusion coefficient decreased markedly, aquaporin-4 expression increased rapidly, and intracellular edema was obviously aggravated; at 4 and 6 hours, the relative apparent diffusion coefficient slowly returned to control levels, aquaporin-4 expression slightly increased, and angioedema was observed. In the RNA interference group, during 0.25-6 hours after injection of siRNA- aquaporin-4 solution, the relative apparent diffusion coefficient slightly fluctuated and aquaporin-4 expression was upregulated; during 0.5-4 hours, the relative apparent diffusion coefficient was significantly higher, while aquaporin-4 expression was significantly lower when compared with the control group, and intracellular edema was markedly reduced; at 0.25 and 6 hours, the relative apparent diffusion coefficient and aquaporin-4 expression were similar when compared with the control group; obvious angioedema remained at 6 hours. Pearson's correlation test results showed that aquaporin-4 expression was negatively correlated with the apparent diffusion coefficient (r = -0.806, P coefficient. Aquaporin-4 gene interference can effectively inhibit the upregulation of aquaporin-4 expression during the stage of intracellular edema with time-effectiveness. Moreover, diffusion-weighted MRI can accurately detect intracellular edema.

  15. Safety of aspirin desensitization in patients with reported aspirin allergy and cardiovascular disease.

    Science.gov (United States)

    McMullan, Kathryn L; Wedner, H James

    2013-01-01

    Aspirin (ASA) is the drug of choice in patients with coronary artery disease for primary and secondary prevention. This poses a problem for those patients reporting hypersensitivity to this drug or class of drugs. Desensitization to ASA may be carried out safely and effectively in patients with reported ASA or nonsteroidal anti-inflammatory drug (NSAID) hypersensitivity needing ASA for cardiac indications. Our 7-step protocol is one choice for a rapid desensitization protocol. A retrospective chart review was conducted evaluating ASA desensitization in patients with reported ASA or NSAID hypersensitivity and a cardiac indication for ASA. In 160 evaluations over 15 years, 89 desensitizations were performed in both the inpatient and outpatient setting with only 16 reactions (18%). Eleven of these 16 patients (68.7%) were able to take daily ASA. Twenty-six desensitization procedures were performed with our 7-step rapid desensitization protocol in 10 inpatients and 16 outpatients with 3 reactions (18.75% of reactions). Initial reaction to ASA involving angioedema and reacting to ASA within the past year increased the risk of having a reaction to desensitization. Desensitization may be safely performed in patients with reported ASA or NSAID hypersensitivity and a cardiac indication for ASA. Our 7-step rapid protocol may be used in both the inpatient and outpatient setting to desensitize these patients. Patients who had angioedema with ASA ingestion or a reaction to ASA within the past year are at higher risk for reaction during the desensitization protocol. The authors have no funding, financial relationships, or conflicts of interest to disclose. © 2012 Wiley Periodicals, Inc.

  16. Neprilysin inhibition with sacubitril/valsartan in the treatment of heart failure: mortality bang for your buck.

    Science.gov (United States)

    Ansara, A J; Kolanczyk, D M; Koehler, J M

    2016-04-01

    Heart failure remains a leading cause of morbidity and mortality worldwide. Advanced therapies have prolonged survival in patients with advanced heart failure, but pharmacotherapeutic optimization remains the mainstay of treatment. It has been over 10 years since the last mortality-reducing medication has been approved by the Food and Drug Administration. This article reviews the background, current knowledge and data supporting the use of sacubitril/valsartan (Entresto(®) ), the newly FDA-approved medication that dually inhibits angiotensin and neprilysin, in the treatment of heart failure. A literature search was performed (January 1980 to August 2015) using PubMed and the search terms were as follows: neprilysin inhibitor, heart failure, endopeptidase, natriuretic peptides, angiotensin, omapatrilat, LCZ696, valsartan and sacubitril. Peer-reviewed, published clinical trials, review articles, relevant treatment guidelines and prescribing information documents were identified and reviewed for relevance. Additionally, reference citations from publications identified were reviewed. The inhibition of endopeptidases has been an area of extensive study for the treatment of heart failure. Previously published literature with the endopeptidase inhibitor omapatrilat failed to demonstrate a sufficient balance between clinical efficacy and safety to justify its approval. Omapatrilat blocked three pathways that break down bradykinin, leading to high rates of angioedema. Sacubitril, on the other hand, is metabolized to a form that is highly selective for neprilysin without possessing activity for the other two peptidases, ACE and APP. The combination of sacubitril with valsartan in a single formulation offers the benefit of concurrent blockade of the renin angiotensin aldosterone system and the inhibition of neprilysin while minimizing angioedema risk. When compared to ACE inhibitor therapy in systolic heart failure patients, sacubitril/valsartan demonstrated reductions in

  17. Consumo de aditivos alimentares e efeitos à saúde: desafios para a saúde pública brasileira Food additive intake and health effects: public health challenges in Brazil

    Directory of Open Access Journals (Sweden)

    Maria Lúcia Teixeira Polônio

    2009-08-01

    Full Text Available Este estudo visa a contextualizar por meio de uma revisão sistemática da literatura, os riscos acarretados pelo consumo de aditivos alimentares. Em relação aos resultados dos estudos associando o consumo de aditivos ao aparecimento do câncer, os efeitos adversos à saúde foram observados principalmente nos estudos em que a Ingestão Diária Aceitável (IDA foi excedida. Também apontou uma carência de pesquisas sobre o transtorno do déficit de atenção e hiperatividade. Já em relação à hipersensibilidade não específica, o número de estudos foi significativo e os resultados mais consistentes quanto às manifestações clínicas de rinite, urticária e angioedema provocadas pelos aditivos, em particular pelos os corantes artificiais. As crianças aparecem como grupo vulnerável, em razão do consumo potencial de alimentos com aditivos alimentares, particularmente corantes artificiais. Os resultados indicam que estudos de consumo de aditivos alimentares deveriam servir de base para a elaboração de estratégias de vigilância alimentar e nutricional, com a finalidade de promover hábitos alimentares saudáveis.This study uses a systematic literature review to contextualize the risks associated with food additive intake. Studies comparing food additive intake and cancer showed that adverse health effects appeared when Acceptable Daily Intake (ADI was exceeded. The review also detected a lack of studies on attention deficit-hyperactivity disorder. There were more studies on non-specific hypersensitivity, highlighting such clinical manifestations as rhinitis, urticaria, and angioedema, all associated with food additives, particularly artificial colorants. Children are a vulnerable group as potential consumers of food additives, particularly artificial colorants. Studies on food additive intake should provide the basis for effective food and nutritional surveillance strategies, aiming to promote healthy eating habits.

  18. Skin manifestations and immunological parameters in childhood food allergy.

    Science.gov (United States)

    Oehling, A; Fernández, M; Córdoba, H; Sanz, M L

    1997-01-01

    According to Hansen's contact rule, the digestive system should be considered as the main shock organ, yet in food allergy, this is not the case. Very often specific food triggers clinical manifestations not involving the digestive system; that is, reactions are manifested either in the respiratory system, as asthma or rhinitis, or in the skin. In these cases the BALT (broncho-alveolar lymphoid tissue) and GALT (gastrointestinal lymphoid tissue) units play a basic role in the sensitizations. The purpose of this study was to determine the most frequent skin manifestations of food allergy among children, and the most frequently involved foods. We also thought it interesting to evaluate the diagnostic reliability of the different standard immunological parameters utilized by the study team in food allergy. All patients underwent intracutaneous tests with 12 groups of the most frequent food allergens, as well as serum IgE, antigen-specific IgE against foods, and antigen-specific histamine release tests. Antigen-specific IgG4 determination was performed in some cases. The results obtained confirmed previous studies, the most common manifestations being: angioedema (48%), followed by urticaria (31%) and atopic dermatitis (21%). Regarding the frequency of sensitization to different food allergens, in mono- or polisensitization, fish and egg stand out in our environment. Certain food allergens are more frequently responsible for specific skin manifestations. Thus, for fish sensitization, the most frequent skin manifestation is atopic dermatitis (50%); for egg sensitization, angioedema is the most frequent skin manifestation (50%); and for milk, urticaria (50%). Finally, and in agreement with previous works regarding the diagnostic reliability of in vitro techniques, we found that the histamine release test offered the highest percentage of diagnostic reliability. Only for sensitization to milk proteins did antigen-specific IgE demonstrate higher reliability. Once again, we

  19. Alergia látex-fruta Latex-fruit allergy

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    Flávia Andréia MARIN

    2002-01-01

    Full Text Available O látex está sendo considerado o alergênico do ano 2000, tendo em vista que inúmeros indivíduos, principalmente profissionais da área de saúde e pacientes submetidos a várias intervenções diagnósticas e terapêuticas, estão freqüentemente expostos aos alérgenos do látex, presentes em produtos de borracha natural. As manifestações clínicas conseqüentes às reações alérgicas de hipersensibilidade imediata vão desde rinite, urticária, conjuntivite, angioedema, asma, até anafilaxia. Estudos recentes estão demonstrando que pacientes alérgicos ao látex desenvolvem concomitantemente sensibilização a certos alimentos de origem vegetal, especialmente frutas como papaia, figo, banana, abacate, kiwi, pêssego, abacaxi, melão e castanha, acreditando-se numa provável ocorrência de reações cruzadas entre os alérgenos do látex e destas frutas. Faz-se, então, uma revisão sobre a alergia ao látex, em particular sobre os grupos de risco, incluindo a presença de reatividade cruzada entre o látex e as frutas.The latex is being considered the allergenic agent of the year 2000, taking into account that several individuals, mainly health care professionals, and patients who had undergone many diagnostic and therapeutic interventions, are frequently exposed to latex allergens, which are present in natural rubber latex products. The clinical manifestations, derived from allergic reactions of immediate hypersensitivity vary from since rhinitis, conjunctivitis, urticaria, angioedema, asthma, to anaphylaxis. Recent researches are demonstrating that patients allergic to latex develop concomitantly sensitization to certain vegetable foods, especially fruits like papaya, fig, banana, avocado, kiwi, peach, pineapple, melon and chestnut, and a probable occurrence of cross reaction between allergens of latex and of these fruits is believed. A review is made about latex allergy, in particular about risk groups, including the presence of

  20. Aspirin Desensitization in Patients With Coronary Artery Disease: Results of the Multicenter ADAPTED Registry (Aspirin Desensitization in Patients With Coronary Artery Disease).

    Science.gov (United States)

    Rossini, Roberta; Iorio, Annamaria; Pozzi, Roberto; Bianco, Matteo; Musumeci, Giuseppe; Leonardi, Sergio; Lettieri, Corrado; Bossi, Irene; Colombo, Paola; Rigattieri, Stefano; Dossena, Cinzia; Anzuini, Angelo; Capodanno, Davide; Senni, Michele; Angiolillo, Dominick J

    2017-02-01

    There are limited data on aspirin (ASA) desensitization for patients with coronary artery disease. The aim of the present study was to assess the safety and efficacy of a standard rapid desensitization protocol in patients with ASA sensitivity undergoing coronary angiography. This is a prospective, multicenter, observational study including 7 Italian centers including patients with a history of ASA sensitivity undergoing coronary angiography with intent to undergo percutaneous coronary intervention. A total of 330 patients with history of ASA sensitivity with known/suspected stable coronary artery disease or presenting with an acute coronary syndrome, including ST-segment-elevation myocardial infarction were enrolled. Adverse effects to aspirin included urticaria (n=177, 53.6%), angioedema (n=69, 20.9%), asthma (n=65, 19.7%), and anaphylactic reaction (n=19, 5.8%). Among patients with urticaria/angioedema, 13 patients (3.9%) had a history of idiopathic chronic urticaria. All patients underwent a rapid ASA (5.5 hours) desensitization procedure. The desensitization procedure was performed before cardiac catheterization in all patients, except for those (n=78, 23.6%) presenting with ST-segment-elevation myocardial infarction who underwent the desensitization after primary percutaneous coronary intervention. Percutaneous coronary intervention was performed in 235 patients (71%) of the overall study population. The desensitization procedure was successful in 315 patients (95.4%) and in all patients with a history of anaphylactic reaction. Among the 15 patients (4.6%) who did not successfully respond to the desensitization protocol, adverse reactions were minor and responded to treatment with corticosteroids and antihistamines. Among patients with successful in-hospital ASA desensitization, 253 patients (80.3%) continued ASA for at least 12 months. Discontinuation of ASA in the 62 patients (19.7%) who had responded to the desensitization protocol was because of medical

  1. Severe adverse drug reaction following Crotalidae Polyvalent Immune Fab (Ovine) administration for copperhead snakebite.

    Science.gov (United States)

    Lepak, Maryjoy R; Bochenek, Samantha H; Bush, Sean P

    2015-01-01

    To present the case of a severe anaphylactic/anaphylactoid reaction to Crotalidae Polyvalent Immune Fab (Ovine) in a patient bitten by a copperhead snake. A 68-year-old man presented with progressive envenomation after receiving a copperhead snakebite on each hand. Crotalinae Fab antivenom was administered. While the initial and only dose was partially infusing, the patient developed an adverse drug reaction (ADR) of urticaria and hypotension, which resolved with cessation of the infusion, recurred with resumption of the infusion, and ultimately was completed with supportive care. An additional episode of hypotension, urticaria, and angioedema occurred shortly after antivenom therapy completion. Epinephrine was administered, resolving the reaction with complete patient recovery. The event received a Naranjo score of 10, indicating a definite ADR. Treating copperhead snakebites with antivenom is a matter of debate. Concern over adverse events and cost induce some physicians to manage copperhead bites without antivenom because they are generally milder in severity. As demonstrated in this case, severe ADR can occur with Crotalinae Fab antivenom, and its efficacy for copperhead envenoming needs to be better established via placebo-controlled, randomized trials. © The Author(s) 2014.

  2. [Acute blue urticaria following subcutaneous injection of patent blue dye].

    Science.gov (United States)

    Hamelin, A; Vial-Dupuy, A; Lebrun-Vignes, B; Francès, C; Soria, A; Barete, S

    2015-11-01

    Patent blue (PB) is a lymphatic vessel dye commonly used in France for sentinel lymph node detection in breast cancer, and less frequently in melanoma, and which may induce hypersensitivity reactions. We report a case of acute blue urticaria occurring within minutes of PB injection. Ten minutes after PB injection for sentinel lymph node detection during breast cancer surgery, a 49-year-old woman developed generalised acute blue urticaria and eyelid angioedema without bronchospasm or haemodynamic disturbance, but requiring discontinuation of surgery. Skin testing using PB and the anaesthetics given were run 6 weeks after the episode and confirmed PB allergy. PB was formally contra-indicated. Immediate hypersensitivity reactions to PB have been reported for between 0.24 and 2.2% of procedures. Such reactions are on occasion severe, chiefly involving anaphylactic shock. Two mechanisms are probably associated: non-specific histamine release and/or an IgE-mediated mechanism. Skin tests are helpful in confirming the diagnosis of PB allergy. Blue acute urticaria is one of the clinical manifestations of immediate hypersensitivity reactions to patent blue dye. Skin tests must be performed 6 weeks after the reaction in order to confirm the diagnosis and formally contra-indicate this substance. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  3. Cow's milk protein allergy in children: a practical guide

    Directory of Open Access Journals (Sweden)

    Calzone Luigi

    2010-01-01

    Full Text Available Abstract A joint study group on cow's milk allergy was convened by the Emilia-Romagna Working Group for Paediatric Allergy and by the Emilia-Romagna Working Group for Paediatric Gastroenterology to focus best practice for diagnosis, management and follow-up of cow's milk allergy in children and to offer a common approach for allergologists, gastroenterologists, general paediatricians and primary care physicians. The report prepared by the study group was discussed by members of Working Groups who met three times in Italy. This guide is the result of a consensus reached in the following areas. Cow's milk allergy should be suspected in children who have immediate symptoms such as acute urticaria/angioedema, wheezing, rhinitis, dry cough, vomiting, laryngeal edema, acute asthma with severe respiratory distress, anaphylaxis. Late reactions due to cow's milk allergy are atopic dermatitis, chronic diarrhoea, blood in the stools, iron deficiency anaemia, gastroesophageal reflux disease, constipation, chronic vomiting, colic, poor growth (food refusal, enterocolitis syndrome, protein-losing enteropathy with hypoalbuminemia, eosinophilic oesophagogastroenteropathy. An overview of acceptable means for diagnosis is included. According to symptoms and infant diet, three different algorithms for diagnosis and follow-up have been suggested.

  4. The importance of monitoring adverse drug reactions in pediatric patients: the results of a national surveillance program in Italy.

    Science.gov (United States)

    Carnovale, Carla; Brusadelli, Tatiana; Zuccotti, GianVincenzo; Beretta, Silvia; Sullo, Maria Giuseppa; Capuano, Annalisa; Rossi, Francesco; Moschini, Martina; Mugelli, Alessandro; Vannacci, Alfredo; Laterza, Marcella; Clementi, Emilio; Radice, Sonia

    2014-09-01

    To gain information on safety of drugs used in pediatrics through a 4-year post-marketing active pharmacovigilance program. The program sampled the Italian population and was termed 'Monitoring of the Adverse Effects in Pediatric population' (MEAP). Adverse drug reactions (ADRs) were collected for individuals aged 0 - 17 years treated in hospitals and territorial health services in Lombardy, Tuscany, Apulia and Campania; located to gain an appropriate sampling of the population. ADRs were evaluated using the Adverse Drug Reaction Probability Scale (Naranjo) and analyzed with respect to time, age, sex, category of ADR, seriousness, suspected medicines, type of reporter and off-label use. We collected and analyzed reports from 3539 ADRs. Vaccines, antineoplastic and psychotropic drugs were the most frequently pharmacotherapeutic subgroups involved. Seventeen percent of reported ADRs were serious; of them fever, vomiting and angioedema were the most frequently reported. Eight percent of ADRs were associated with off-label use, and 10% were unknown ADRs. Analysis of these revealed possible strategies of therapy optimization. The MEAP project demonstrated that active post-marketing pharmacovigilance programs are a valid strategy to increase awareness on pediatric pharmacology, reduce underreporting and provide information on drug actions in pediatrics. This information enhances drug therapy optimization in the pediatric patients.

  5. The sensitivity and clinical course of patients with wheat-dependent exercise-induced anaphylaxis sensitized to hydrolyzed wheat protein in facial soap - secondary publication.

    Science.gov (United States)

    Hiragun, Makiko; Ishii, Kaori; Hiragun, Takaaki; Shindo, Hajime; Mihara, Shoji; Matsuo, Hiroaki; Hide, Michihiro

    2013-09-01

    Recently, an increasing number of patients with wheat-dependent exercise-induced anaphylaxis (WDEIA) have been reported in Japan. Most of them had developed this condition during or after using hydrolyzed wheat protein (HWP)-containing soap (HWP-WDEIA). To clarify the relation between WDEIA and HWP-containing soap and their prognosis, we retrospectively studied the patients who visited Hiroshima University Hospital and were diagnosed as WDEIA from January 2010 to June 2011. We took detailed clinical histories, performed skin prick tests, serum immunoassays for antigen-specific IgE and basophil histamine release test, and followed up their clinical courses after the diagnosis. Among 36 patients with WDEIA, 30 patients had used only one type of HWP-soap. The patients with HWP-WDEIA were mainly women and had developed facial symptoms and angioedema. They suffered from blood pressure reductions less frequently than patients with conventional WDEIA. The levels of gluten-specific IgE were higher than those of omega-5 gliadin in patients with HWP-WDEIA (P soap. The development of HWP-WDEIA is associated with the use of HWP-soap. The sensitivity to HWP that cross reacts with non-processed wheat may be reduced or possibly cured after the discontinuation of HWP-soap.

  6. [The sensitivity and clinical course of patients with wheat-dependent exercise-induced anaphylaxis sensitized to hydrolyzed wheat protein in facial soap].

    Science.gov (United States)

    Hiragun, Makiko; Ishii, Kaori; Hiragun, Takaaki; Shindo, Hajime; Mihara, Shoji; Matsuo, Hiroaki; Hide, Michihiro

    2011-12-01

    Recently an increasing number of patients with wheat-dependent exercise-induced anaphylaxis (WDEIA), developed during or after using hydrolyzed wheat protein (HWP)-containing soap (HWP-WDEIA), were reported in Japan. To clarify the relation between WDEIA and HWP-containing soap and their prognosis, we investigated the patients who visited Hiroshima University Hospital and were diagnosed as WDEIA from January 2010 to June 2011. We took detailed clinical histories, performed skin prick tests, serum immunoassays for antigen-specific IgE and basophil histamine release test, and followed up their clinical courses after the diagnosis. Among 36 patients with WDEIA, 30 patients had used only one type of HWP-soap. The patients with HWP-WDEIA were mainly women and had developed facial symptoms and angioedema. They suffered from blood pressure reductions less frequently than patients with conventional WDEIA. The levels of glutens-specific IgE were higher than those of ω-5 gliadin in patients with HWP-WDEIA (psoap. The development of HWP-WDEIA is associated with the use of HWP-soap. The sensitivities to HWP that cross reacts with non-processed wheat may be reduced or possibly cured after the discontinuation of HWP-soap.

  7. A Report of Two Cases of Solid Facial Edema in Acne.

    Science.gov (United States)

    Kuhn-Régnier, Sarah; Mangana, Joanna; Kerl, Katrin; Kamarachev, Jivko; French, Lars E; Cozzio, Antonio; Navarini, Alexander A

    2017-03-01

    Solid facial edema (SFE) is a rare complication of acne vulgaris. To examine the clinical features of acne patients with solid facial edema, and to give an overview on the outcome of previous topical and systemic treatments in the cases so far published. We report two cases from Switzerland, both young men with initially papulopustular acne resistant to topical retinoids. Both cases responded to oral isotretinoin, in one case combined with oral steroids. Our cases show a strikingly similar clinical appearance to the cases described by Connelly and Winkelmann in 1985 (Connelly MG, Winkelmann RK. Solid facial edema as a complication of acne vulgaris. Arch Dermatol. 1985;121(1):87), as well as to cases of Morbihan's disease that occurs as a rare complication of rosacea. Even 30 years after, the cause of the edema remains unknown. In two of the original four cases, a potential triggering factor was identified such as facial trauma or insect bites; however, our two patients did not report such occurrencies. The rare cases of solid facial edema in both acne and rosacea might hold the key to understanding the specific inflammatory pattern that creates both persisting inflammation and disturbed fluid homeostasis which can occur as a slightly different presentation in dermatomyositis, angioedema, Heerfordt's syndrome and other conditions.

  8. Exantema fijo medicamentoso por paracetamol. Caso clínico con revisión bibliográfica

    Directory of Open Access Journals (Sweden)

    Justo Rueda López

    Full Text Available RESUMEN El exantema fijo medicamentoso es una toxicodermia caracterizada por la aparición de una lesión cutánea en forma de mácula de color rojizo o violáceo, redonda u oval, edematosa, bien delimitada, y que presenta recurrencias tras la administración del agente causal, generalmente un fármaco. Puede ser producido por una gran variedad de fármacos, entre ellos el paracetamol. El paracetamol es un medicamento con propiedades analgésicas y antipiréticas ampliamente conocido y utilizado por su amplio margen de seguridad y elevada biodisponibilidad. Sin embargo, se han descrito reacciones cutáneas adversas de diferentes tipos e intensidad tras su administración, fundamentalmente urticaria y angioedema, y con menor frecuencia eritema multiforme, necrólisis epidérmica tóxica, exantema fijo y púrpura de Henoch-Schönlein. Se presenta el caso de un usuario tipo con exantema fijo medicamentoso relacionado con el consumo de paracetamol. Se ha realizado una revisión bibliográfica sobre los diversos hallazgos clínicos y el diagnóstico diferencial entre las diferentes toxicodermias, así como el manejo y abordaje de las lesiones basado en el concepto TIME.

  9. Celiac Disease and Wheat Allergy: A Growing Association?

    Science.gov (United States)

    Micozzi, Sarah; Infante, Sonsoles; Fuentes-Aparicio, Victoria; Álvarez-Perea, Alberto; Zapatero, Lydia

    2018-05-30

    Celiac disease and wheat allergy (WA) are infrequent diseases in the general population, and a combination of the 2 is particularly rare. Celiac disease occurs in around 1% of the general population and WA in around 1% of all children. We report 2 patients with celiac disease and a gluten-free diet who developed WA consistent in anaphylaxis and an eyelid angioedema, respectively, through accidental wheat exposure. A serum study and an intestinal biopsy confirmed celiac disease. Both patients were studied with a skin prick test and serum-specific IgE, with a diagnosis of WA. In patients with celiac disease, the trace amounts of cereals present in gluten-free food could act as a sensitization factor, and probably patients with persistent symptoms (despite a gluten-free diet) are experiencing WA symptoms rather than celiac disease symptoms. The number of patients diagnosed with celiac disease has increased in the recent decades: the association between celiac disease and WA, exceedingly rare to date, could increase as well, prompting special attention to the possibility of inadvertent intake of cereals. © 2018 S. Karger AG, Basel.

  10. The Mast Cell, Contact, and Coagulation System Connection in Anaphylaxis

    Directory of Open Access Journals (Sweden)

    Mar Guilarte

    2017-07-01

    Full Text Available Anaphylaxis is the most severe form of allergic reaction, resulting from the effect of mediators and chemotactic substances released by activated cells. Mast cells and basophils are considered key players in IgE-mediated human anaphylaxis. Beyond IgE-mediated activation of mast cells/basophils, further mechanisms are involved in the occurrence of anaphylaxis. New insights into the potential relevance of pathways other than mast cell and basophil degranulation have been unraveled, such as the activation of the contact and the coagulation systems. Mast cell heparin released upon activation provides negatively charged surfaces for factor XII (FXII binding and auto-activation. Activated FXII, the initiating serine protease in both the contact and the intrinsic coagulation system, activates factor XI and prekallikrein, respectively. FXII-mediated bradykinin (BK formation has been proven in the human plasma of anaphylactic patients as well as in experimental models of anaphylaxis. Moreover, the severity of anaphylaxis is correlated with the increase in plasma heparin, BK formation and the intensity of contact system activation. FXII also activates plasminogen in the fibrinolysis system. Mast cell tryptase has been shown to participate in fibrinolysis through plasmin activation and by facilitating the degradation of fibrinogen. Some usual clinical manifestations in anaphylaxis, such as angioedema or hypotension, or other less common, such as metrorrhagia, may be explained by the direct effect of the activation of the coagulation and contact system driven by mast cell mediators.

  11. The safety of sacubitril-valsartan for the treatment of chronic heart failure.

    Science.gov (United States)

    Tyler, Jeffrey M; Teerlink, John R

    2017-02-01

    Sacubitril-valsartan is a combination drug that contains the neprilysin inhibitor sacubitril and angiotensin II receptor blocker valsartan. In 2015, the US Food and Drug Administration approved sacubitril-valsartan for treatment of heart failure patients with reduced ejection fraction and New York Heart Association class II-IV symptoms following a large, Phase III clinical trial (PARADIGM-HF) that demonstrated a 20% reduction in the combined primary end-point of death from cardiovascular cause or hospitalization for heart failure compared to enalapril. Areas covered: This review discusses the clinical efficacy and safety of angiotensin receptor neprilysin inhibitor sacubitril-valsartan in heart failure with reduced ejection fraction. Expert opinion: Based on the PARADIGM-HF trial, sacubitril-valsartan offers compelling reductions in meaningful clinical endpoints, independent of age or severity of disease. The rate of adverse events was comparable between the enalapril and sacubitril-valsartan groups, although the absolute rates are likely underestimated due to the entry criteria and run-in period. Future trials and post-market surveillance are critical to better understand the risk of angioedema in high risk populations, particularly African-Americans, as well as long-term theoretical risks including the potential for increased cerebral amyloid plaque deposition with possible development of neurocognitive disease. Current trials are underway to evaluate potential benefit in patients with heart failure with preserved ejection fraction.

  12. Anti-snake venom: use and adverse reaction in a snake bite study clinic in Bangladesh

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    MR Amin

    2008-01-01

    Full Text Available Snakebites can present local or systemic envenomation, while neurotoxicity and respiratory paralysis are the main cause of death. The mainstay of management is anti-snake venom (ASV, which is highly effective, but liable to cause severe adverse reactions including anaphylaxis. The types of adverse reaction to polyvalent anti-snake venom have not been previously studied in Bangladesh. In this prospective observational study carried out between 1999 and 2001, in the Snake Bite Study Clinic of Chittagong Medical College Hospital, 35 neurotoxic-snake-bite patients who had received polyvalent anti-snake venom were included while the ones sensitized to different antitoxins and suffering from atopy were excluded. The common neurotoxic features were ptosis (100%, external ophthalmoplegia (94.2%, dysphagia (77.1%, dysphonia (68.5% and broken neck sign (80%. The percentage of anti-snake venom reaction cases was 88.57%; pyrogenic reaction was 80.64%; and anaphylaxis was 64.51%. The common features of anaphylaxis were urticaria (80%; vomiting and wheezing (40%; and angioedema (10%. The anti-snake venom reaction was treated mainly with adrenaline for anaphylaxis and paracetamol suppository in pyrogenic reactions. The average recovery time was 4.5 hours. Due to the danger of reactions the anti-snake venom should not be withheld from a snakebite victim when indicated and appropriate guidelines should be followed for its administration.

  13. Off-Label Uses of Omalizumab.

    Science.gov (United States)

    El-Qutob, David

    2016-02-01

    The off-label use of medicines is a common and extensive clinical practice. Omalizumab has been licensed for use in severe allergic asthma and chronic urticaria. Omalizumab dosing was based on body weight and baseline serum IgE concentration. All patients are required to have a baseline IgE between 30 and 700 IU/ml and body weight not more than 150 kg. The use of off-label drugs may lead to several problems including adverse effects and an increased risk/benefit balance. In this article, there are summarized off-label uses of omalizumab in the last recent years in diseases in which IgE maybe or certainly has a corner role such as allergic rhinitis, allergic bronchopulmonary aspergillosis, anaphylaxis, keratoconjunctivitis, food allergy, drug allergy, urticaria, angioedema, non-atopic asthma, atopic dermatitis, nasal polyps, Churg-Strauss syndrome, eosinophilic otitis media, chronic rhinosinusitis, bullous pemphigoid, contact dermatitis, and others. Use in pregnancy asthmatic women and pre-co-administration with specific immunotherapy will also be revised.

  14. Omalizumab in the treatment of chronic spontaneous urticaria – literature review and own experience

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    Joanna Dawicka

    2016-02-01

    Full Text Available Chronic urticaria is a condition characterized by the appearance of wheals and/or angioedema that last for at least 6 weeks. According to the recent recommendations, if H1-antihistamines used in four times licensed doses remain ineffective, add-on treatment with omalizumab, cyclosporine A or montelukast should be applied. This paper offers a literature review of the studies on using omalizumab in chronic spontaneous urticaria (CSU treatment. Moreover, it presents our own experience with the use of omalizumab in the treatment of two patients with CSU. Both patients were treated with H1-antihistamines in four times licensed doses recommended by European guidelines, oral corticosteroids, montelukast, and methotrexate – all without satisfactory results. During the time of the omalizumab therapy both patients achieved a significant improvement measured by the decrease in the Average Urticaria Activity Score for 7 days (UAS7 – the first patient from 34 to 11.8 points, the second one from 41 to 5.76.

  15. T cell activity in successful treatment of chronic urticaria with omalizumab

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    Gonzalez Ruperto

    2011-07-01

    Full Text Available Abstract Omalizumab, a humanized monoclonal anti-IgE antibody has the potential to alter allergen processing. Recently, it has been postulated the assessment of PHA-stimulated adenosine triphosphate (ATP activity as maker of CD4+ T cells activity in peripheral blood cells. We present the case report of a 35-year-old woman with a history of chronic idiopathic urticaria and angioedema of 8 years of development with poor response to treatment. The patient was partially controlled with cyclosporine at doses of 100 mg/12 h. However, she was still developing hives daily. Finally treatment with omalizumab was started at dose of 300 mg every 2 weeks. The patient experienced a decrease in urticarial lesions 2 days after starting therapy. We also evaluated the effects of omalizumab therapy on the activity of peripheral blood CD4+ T cells from the patient, in order to determine the potential modification of anti-IgE therapy on the process of antigen presentation-recognition. Activity of CD4+ cells by ATP release was clearly increased demonstrating an enlarged CD4 activity. Omalizumab may be useful in the treatment of severe chronic urticaria. ATP activity of peripheral blood CD4+ T cells might be a non-subjective method to assess Omalizumab activity.

  16. Omalizumab, an anti-immunoglobulin E antibody: state of the art

    Science.gov (United States)

    Incorvaia, Cristoforo; Mauro, Marina; Russello, Marina; Formigoni, Chiara; Riario-Sforza, Gian Galeazzo; Ridolo, Erminia

    2014-01-01

    A large number of trials show that the anti-immunoglobulin (Ig) E antibody omalizumab is very effective in patients with severe allergic asthma. This is acknowledged in consensus documents. The drug also has a good safety profile and a pharmacoeconomic advantage due to a reduction in the number of hospitalizations for asthma attacks. In recent years, some studies have shown that omalizumab is effective also in nonallergic asthma. Effects on the complex signaling mechanisms leading to activation of effector cells and to mediator release may account for this outcome. Indeed, omalizumab has been reported to be effective in a number of IgE-mediated and non-IgE-mediated disorders. Concerning the former, clinical efficacy has been observed in rhinitis, allergic bronchopulmonary aspergillosis, latex allergy, atopic dermatitis, allergic urticaria, and anaphylaxis. In addition, omalizumab has been demonstrated to be able to prevent systemic reactions to allergen immunotherapy, thus enabling completion of treatment in patients who otherwise would have to stop it. Concerning non-IgE-mediated disorders, omalizumab has been reported to be effective in nasal polyposis, autoimmune urticaria, chronic idiopathic urticaria, physical urticaria, idiopathic angioedema, and mastocytosis. Current indications for treatment with omalizumab are confined to severe allergic asthma. Consequently, any other prescription can only be off-label. However, it is reasonable to expect that the use of omalizumab will be approved for particularly important indications, such as anaphylaxis, in the near future. PMID:24532966

  17. Profile of omalizumab in the treatment of chronic spontaneous urticaria.

    Science.gov (United States)

    Labrador-Horrillo, Moises; Ferrer, Marta

    2015-01-01

    Chronic spontaneous urticaria (CSU) is a disease with significant morbidity and relative prevalence that has important effects on the quality of life (QoL) of those who suffer from it. Omalizumab is a recombinant humanized anti-immunoglobulin E (IgE) antibody that binds to the Cε3 domain of the IgE heavy chain and prevents it from binding to its high-affinity receptor FcεRI. It has been largely studied in the field of asthma and is currently approved for the treatment of both adult and pediatric (children; >6-year-old) patients. In addition, in recent, well-controlled clinical trials in patients with CSU resistant to antihistamines, add-on therapy with subcutaneous omalizumab significantly reduced the severity of itching, and the number and size of hives, and increased patients' health-related QoL and the proportion of days free from angioedema compared with placebo, with an excellent tolerance. Thus, omalizumab is an effective and well-tolerated add-on therapy for patients with CSU who are symptomatic despite background therapy with H1 antihistamines. In this review, we cover the following points: epidemiology, pathogenesis, assessment of activity, impact on QoL, and treatment of CSU, and finally, we focus on omalizumab in the treatment of CSU including the pharmacokinetic properties and mechanism of action, and use in pregnant women, nursing infants, and children.

  18. Is it possible for chronic urticaria diagnostic approach to be simplified? A clinical data checklist

    Directory of Open Access Journals (Sweden)

    Iván Chérrez-Ojeda

    2017-10-01

    Full Text Available Background: Existing clinical guidelines do not offer an efficient alternative for the collection of data on relevant clinical traits during history and physical of the patient with chronic urticaria. Objective: Our aim was to provide a clinical data checklist together with its guide to allow for thorough information to be obtained and for a physical exam that identifies the main features and triggering factors of the disease to be carried out. Methods: A search was conducted for relevant literature on chronic urticaria in Medline, the Cochrane library and PubMed. Results: We developed an easy-to-use clinical data checklist with its corresponding clinical guide, comprised by 42 items based on two components: essential clues for history taking and chronic urticaria diagnosis (typical symptoms according to subgroups, etiology and laboratory results. Some components are the time of disease onset, wheals’ duration, shape, size, color and distribution, associated angioedema, atopy, triggering factors and others. Conclusions: The clinical data checklist and its guide constitute a tool to focus, guide and save time in medical consultation, with the main purpose to aid physicians in providing better diagnosis and management of the disease.

  19. The EAACI/GA(2) LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update.

    Science.gov (United States)

    Zuberbier, T; Aberer, W; Asero, R; Bindslev-Jensen, C; Brzoza, Z; Canonica, G W; Church, M K; Ensina, L F; Giménez-Arnau, A; Godse, K; Gonçalo, M; Grattan, C; Hebert, J; Hide, M; Kaplan, A; Kapp, A; Abdul Latiff, A H; Mathelier-Fusade, P; Metz, M; Nast, A; Saini, S S; Sánchez-Borges, M; Schmid-Grendelmeier, P; Simons, F E R; Staubach, P; Sussman, G; Toubi, E; Vena, G A; Wedi, B; Zhu, X J; Maurer, M

    2014-07-01

    This guideline is the result of a systematic literature review using the 'Grading of Recommendations Assessment, Development and Evaluation' (GRADE) methodology and a structured consensus conference held on 28 and 29 November 2012, in Berlin. It is a joint initiative of the Dermatology Section of the European Academy of Allergy and Clinical Immunology (EAACI), the EU-funded network of excellence, the Global Allergy and Asthma European Network (GA(2) LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) with the participation of delegates of 21 national and international societies. Urticaria is a frequent, mast cell-driven disease, presenting with wheals, angioedema, or both. The life-time prevalence for acute urticaria is approximately 20%. Chronic spontaneous urticaria and other chronic forms of urticaria do not only cause a decrease in quality of life, but also affect performance at work and school and, as such, are members of the group of severe allergic diseases. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  20. The EAACI/GA²LEN/EDF/WAO Guideline for the Definition, Classification, Diagnosis and Management of Urticaria. The 2017 Revision and Update.

    Science.gov (United States)

    Zuberbier, T; Aberer, W; Asero, R; Abdul Latiff, A H; Baker, D; Ballmer-Weber, B; Bernstein, J A; Bindslev-Jensen, C; Brzoza, Z; Buense Bedrikow, R; Canonica, G W; Church, M K; Craig, T; Danilycheva, I V; Dressler, C; Ensina, L F; Giménez-Arnau, A; Godse, K; Gonçalo, M; Grattan, C; Hebert, J; Hide, M; Kaplan, A; Kapp, A; Katelaris, C H; Kocatürk, E; Kulthanan, K; Larenas-Linnemann, D; Leslie, T A; Magerl, M; Mathelier-Fusade, P; Meshkova, R Y; Metz, M; Nast, A; Nettis, E; Oude-Elberink, H; Rosumeck, S; Saini, S S; Sánchez-Borges, M; Schmid-Grendelmeier, P; Staubach, P; Sussman, G; Toubi, E; Vena, G A; Vestergaard, C; Wedi, B; Werner, R N; Zhao, Z; Maurer, M

    2018-01-15

    This evidence and consensus-based guideline was developed following the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working group. The conference was held on December 1st, 2016. It is a joint initiative of the Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the EU-founded network of excellence, the Global Allergy and Asthma European Network (GA²LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) with the participation of 48 delegates of 42 national and international societies. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). Urticaria is a frequent, mast cell-driven disease, presenting with wheals, angioedema, or both. The lifetime prevalence for acute urticaria is approximately 20%. Chronic spontaneous urticaria and other chronic forms of urticaria are disabling, impair quality of life, and affect performance at work and school. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  1. Less travelled roads in clinical immunology and allergy: drug reactions and the environmental influence.

    Science.gov (United States)

    Selmi, Carlo; Crotti, Chiara; Meroni, Pier Luigi

    2013-08-01

    Allergy and clinical immunology are examples of areas of knowledge in which working hypotheses are dominant over mechanistic understanding. As such, sometimes scientific efforts follow major streams and overlook some epidemiologically prevalent conditions that thus become underestimated by the research community. For this reason, we welcome the present issue of Clinical Reviews in Allergy and Immunology that is dedicated to uncommon themes in clinical immunology and allergy. First, comprehensive discussions are provided for allergy phenomena of large potential impact in clinical practice such as reactions to cephalosporins or aspirin-induced asthma and in everyday life such as allergies to food additives or legumes. Further, the issue addresses other uncommon themes such as urticaria and angioedema, cercarial dermatitis, or late-onset inflammation to soft tissue fillers. Last, there will be discussion on transversal issues such as olfactory defects in autoimmunity, interleukin 1 beta pathway, and the search for new serological markers in chronic inflammation. As a result, we are convinced that this issue will be of help to clinicians involved in internal medicine as well as to allergists and clinical immunologists. More importantly, we are convinced that these discussions will be of interest also to basic scientists for the numerous translational implications.

  2. Structural and metabolic changes in the traumatically injured rat brain. High-resolution in vivo proton magnetic resonance spectroscopy at 7 T

    International Nuclear Information System (INIS)

    Li, Jing; Zhao, Can; Rao, Jia-Sheng; Yang, Fei-Xiang; Yang, Zhao-Yang; Wang, Zhan-Jing; Lei, Jian-Feng; Li, Xiao-Guang

    2017-01-01

    The understanding of microstructural and metabolic changes in the post-traumatic brain injury is the key to brain damage suppression and repair in clinics. Ten female Wistar rats were traumatically injured in the brain CA1 region and above the cortex. Next, diffusion tensor magnetic resonance imaging (DTI) and proton magnetic resonance spectroscopy ( 1 H MRS) were used to analyze the microstructural and metabolic changes in the brain within the following 2 weeks. Anisotropy fraction (FA) and axial diffusivity (AD) of the corpus callosum (CC) began to decrease significantly at day 1, whereas radial diffusivity (RD) significantly increased immediately after injury, reflecting the loss of white matter integrity. Compared with day 3, RD decreased significantly at day 7, implicating the angioedema reduction. In the hippocampus, FA significantly increased at day 7; the choline-containing compounds (Cho) and myo-inositol (MI) remarkably increased at day 7 compared with those at day 3, indicating the proliferation of astrocytes and radial glial cells after day 7. No significant differences between DTI and 1 H MRS parameters were observed between day 1 and day 3. Day 1-3 after traumatic brain injury (TBI) may serve as a relatively appropriate time window for treatment planning and the following nerve repair. (orig.)

  3. Mosquito bite anaphylaxis: immunotherapy with whole body extracts.

    Science.gov (United States)

    McCormack, D R; Salata, K F; Hershey, J N; Carpenter, G B; Engler, R J

    1995-01-01

    Adverse reactions to mosquito bites have been recognized for some time. These usually consist of large local swellings and redness, generalized urticaria, angioedema and less easily definable responses such as nausea, dizziness, headaches, and lethargy. We report two patients who experienced systemic anaphylaxis from mosquito bites. Both were skin tested and given immunotherapy using whole body mosquito extracts. Skin testing using whole body mosquito extracts was positive to Aedes aegypti at 1/1,000 weight/volume (wt/vol) in one patient and to Aedes aegypti at 1/100,000 wt/vol, and Culex pipiens at 1/10,000 wt/vol in the other. Skin testing of ten volunteers without a history of adverse reactions to mosquito bites was negative. Immunotherapy using these extracts resulted in resolution of adverse reactions to mosquito bites in one patient and a decrease in reactions in the other. Immunotherapy with whole body mosquito extracts is a viable treatment option that can play a role in patients with mosquito bite-induced anaphylaxis. It may also result in severe side effects and one must determine the benefit versus risks for each individual patient.

  4. H1-antihistamines in pregnancy and lactation.

    Science.gov (United States)

    Schatz, Michael

    2002-01-01

    Antihistamines may be used for the treatment of allergic rhinitis, upper respiratory infections, urticaria/angioedema, atopic dermatitis, and, rarely, as adjunctive treatment for anaphylaxis, during pregnancy. Because these illnesses may affect maternal comfort and safety as well as threaten the fetus directly (anaphylaxis) or indirectly, they often require therapy during pregnancy. Based on the information available to date, in this chapter we have attempted to provide rational guidelines for the gestational use of H1-receptor antagonists in a manner that will lead to the optimal well-being of both the mother and her infant. As more information becomes available, the recommendations herein may require modification. Although this chapter has dealt specifically with gestational management, a case can be made for considering this information when making therapeutic decisions in all women of childbearing potential. First, most pregnancies are unplanned, and the peak period of fetal vulnerability to drug-induced teratogenesis begins the day a woman's period is due. Second, during gestation, substantial alterations in a previously successful but not optimal-for-pregnancy chronic therapeutic regimen may be psychologically threatening to the patient and may lead to either uncontrolled disease or unanticipated side effects. Thus, pregnancy-appropriate regimens should ideally be discussed with all women of childbearing age as part of the informed therapeutic decision-making process.

  5. Prevalence and characteristics of reported penicillin allergy in an urban outpatient adult population.

    Science.gov (United States)

    Albin, Stephanie; Agarwal, Shradha

    2014-01-01

    Penicillin allergy remains the most common drug allergy, with a reported prevalence of 10% in the United States. Epidemiology of penicillin allergy in outpatient populations is relatively scarce. This study sought to determine the prevalence and characteristics of reported penicillin allergy in an urban outpatient population and to identify trends in clinical evaluation and management from a tertiary center serving a large inner-city population. A retrospective review of electronic medical records was performed of adult patients seen in the Internal Medicine Associates Clinic of Mount Sinai Hospital between January 31, 2012, and July 31, 2012. Medical records were selected based on the documentation of penicillin in patient's allergy section. Of the 11,761 patients seen in the clinic, 1348 patients (11.5%) reported a history of penicillin allergy. The most common allergic reactions were rash (37%), unknown/undocumented (20.2%), hives (18.9%), swelling/angioedema (11.8%), and anaphylaxis (6.8%). There was an increased prevalence of penicillin allergy in female patients compared with male patients (odds ratio [OR] = 1.82; 95% CI = 1.60, 2.08; p penicillin allergy compared with Caucasians (OR = 0.51; 95% CI = 0.32, 0.83; p = 0.007). However, only 78 (6%) of the patients reporting penicillin allergy had a referral to an allergy specialist. Overall, improved referral to an allergist will help to identify patients who have penicillin allergy requiring avoidance.

  6. Wheat-Dependent Exercise-Induced Anaphylaxis Sensitized with Hydrolyzed Wheat Protein in Soap

    Directory of Open Access Journals (Sweden)

    Yuko Chinuki

    2012-01-01

    Full Text Available Wheat-dependent exercise-induced anaphylaxis (WDEIA is a specific form of wheat allergy typically induced by exercise after ingestion of wheat products. Wheat ω-5 gliadin is a major allergen associated with conventional WDEIA, and detection of serum immunoglobulin E (IgE specific to recombinant ω-5 gliadin is a reliable method for its diagnosis. Recently, an increased incidence of a new subtype of WDEIA, which is likely to be sensitized via a percutaneous and/or rhinoconjunctival route to hydrolyzed wheat protein (HWP, has been observed. All of the patients with this new subtype had used the same brand of soap, which contained HWP. Approximately half of these patients developed contact allergy several months later and subsequently developed WDEIA. In each of these patients, contact allergy with soap exposure preceded food ingestion-induced reactions. Other patients directly developed generalized symptoms upon ingestion of wheat products. The predominant observed symptom of the new WDEIA subtype was angioedema of the eyelids; a number of patients developed anaphylaxis. This new subtype of WDEIA has little serum ω-5 gliadin-specific serum IgE.

  7. Polyetheretherketone (PEEK) intervertebral cage as a cause of chronic systemic allergy: a case report.

    Science.gov (United States)

    Maldonado-Naranjo, Andres L; Healy, Andrew T; Kalfas, Iain H

    2015-07-01

    Polyetheretherketone (PEEK) is an organic polymer thermoplastic with strong mechanical and chemical resistance properties. It has been used in industry to fabricate items for demanding applications such as bearings, piston parts, compressor plate valves, and cable insulation. Since the early 1980s, polyetheretherketone polymers have been increasingly used in orthopedic and spinal surgery applications. Numerous studies and years of clinical experience have confirmed the biocompatibility of this material. The purpose of the study was to report a case of chronic systemic allergy after anterior cervical decompression and fusion (ACDF) and implantation of an intervertebral PEEK cage, with resolution of symptoms after removal of PEEK cage. This study is a case report with clinical evidence for allergy to PEEK. The methods involve clinical findings and review of current literature. After ACDF and implantation of an intervertebral PEEK cage, the patient had developed an angioedema-like picture marked by severe redness, itching, swelling of his tongue, and skin thickening. A skin patch test was positive for PEEK. Removal of the implant resulted in the resolution of his allergy symptoms shortly after surgery. Tissue reactions to PEEK are extremely rare. Herein, we present the first report of a chronic allergic response to interbody PEEK material. Copyright © 2015 Elsevier Inc. All rights reserved.

  8. A Case of Apparent Contact Dermatitis Caused by Toxocara Infection

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    Rosanna Qualizza

    2014-01-01

    Full Text Available Infection from Toxocara species may give rise to a large array of clinical symptoms, including apparent manifestations of allergy such as asthma, urticaria/angioedema, and dermatitis. We report a case, thus far not described, of contact dermatitis attributed to nickel allergy but caused by Toxocara infection. The patient was a 53-year-old woman presenting from 10 years a dermatitis affecting head, neck, and thorax. Patch tests initially performed gave a positive result to nickel, but avoidance of contact with nickel did not result in recovery. The patient referred to our Allergy Service in 2010 because of dermatitis to feet. Patch testing confirmed the positive result for nickel, but expanding the investigation a positive result for IgG antibodies to Toxocara was detected by Western blotting and ELISA. Treatment with mebendazole achieved immediate efficacy on feet dermatitis. Then, two courses of treatment with albendazole resulted in complete regression of dermatitis accompanied by development of negative ELISA and Western blotting for Toxocara antibodies. This report adds another misleading presentation of Toxocara infection as apparent contact dermatitis caused by nickel and suggests bearing in mind, in cases of contact dermatitis not responding to avoidance of the responsible hapten and to medical treatment, the possible causative role of Toxocara.

  9. Autologous serum skin test as an indicator of chronic autoimmune urticaria in a tertiary care hospital in South India.

    Science.gov (United States)

    Vikramkumar, Adaikalampillai Ganapathy; Kuruvila, Sheela; Ganguly, Satyaki

    2014-12-01

    Autologous serum skin test (ASST) is a simple in-vivo clinical test for the detection of basophil histamine releasing activity and to diagnose chronic autoimmune urticaria (CAU) among chronic spontaneous urticaria (CSU) patients. Diagnosing these patients is also important as they may need high doses of antihistamines and systemic corticosteroids during acute exacerbations. The aim of this study is to study the prevalence of CAU among cases of CSU by using ASST. This was a cross-sectional study done among 48 patients presenting with CSU. Detailed history, physical examination and routine investigations were recorded for all patients. ASST was done on all the 48 patients. Of the 48 patients included in the study, 20 patients (41.6%) were ASST positive, while the remaining 28 (58%) were ASST negative. The median duration of disease in both ASST positive and negative patients was 1 year. ASST positivity was higher (66.6%) among patients with a history of round shaped weals, though not statistically significant. ASST positivity was seen in 5 (71.4%) out of seven patients with systemic involvement, which was again not statistically significant. Our study did not show any significant difference between patients with and without antibodies regarding mean age and sex distribution, clinical morphology of individual weals, duration, severity, systemic symptoms, angioedema, atopy, and association with other autoimmune conditions.

  10. Kiss-induced severe anaphylactic reactions

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    Atanasković-Marković Marina

    2010-01-01

    Full Text Available Introduction. Ingestion is the principal route for food allergens to trigger allergic reaction in atopic persons. However, in some highly sensitive patients severe symptoms may develop upon skin contact and by inhalation. The clinical spectrum ranges from mild facial urticaria and angioedema to life-threatening anaphylactic reactions. Outline of Cases. We describe cases of severe anaphylactic reactions by skin contact, induced by kissing in five children with prior history of severe anaphylaxis caused by food ingestion. These cases were found to have the medical history of IgE mediated food allergy, a very high total and specific serum IgE level and very strong family history of allergy. Conclusion. The presence of tiny particles of food on the kisser's lips was sufficient to trigger an anaphylactic reaction in sensitized children with prior history of severe allergic reaction caused by ingestion of food. Allergic reaction provoked with food allergens by skin contact can be a risk factor for generalized reactions. Therefore, extreme care has to be taken in avoiding kissing allergic children after eating foods to which they are highly allergic. Considering that kissing can be a cause of severe danger for the food allergic patient, such persons should inform their partners about the risk factor for causing their food hypersensitivity.

  11. Hair dye dermatitis and p-phenylenediamine contact sensitivity: A preliminary report

    Directory of Open Access Journals (Sweden)

    Mrinal Gupta

    2015-01-01

    Full Text Available Background: The contact allergic reactions from p-phenylenediamine (PPD in hair dyes vary from mild contact dermatitis to severe life- threatening events (angioedema, bronchospasm, asthma, renal impairment. Objectives: To study the clinical patterns and PPD contact sensitivity in patients with hair-dye dermatitis. Materials and Methods: Eighty (M: F 47:33 consecutive patients aged between 18 and 74 years suspected to have contact allergy from hair dye were studied by patch testing with Indian Standard Series including p-phenylenediamine (PPD, 1.0% pet. Results: 54 Fifty-four (M: F 21:33 patients showed positive patch tests from PPD. Eight of these patients also showed positive patch test reaction from fragrance mix, thiuram mix, paraben mix, or colophony. Fifty-seven (71% patients affected were aged older than 40 years. The duration of dermatitis varied from 1 year with exacerbation following hair coloring. Forty-nine patients had dermatitis of scalp and/or scalp margins and 23 patients had face and neck dermatitis. Periorbital dermatitis, chronic actinic dermatitis, and erythema multiforme-like lesions were seen in 4, 2, and 1 patients, respectively. Conclusions: Hair dyes and PPD constitute a significant cause of contact dermatitis. There is an urgent need for creating consumer awareness regarding hair-dyes contact sensitivity and the significance of performing sensitivity testing prior to actual use.

  12. Structural and metabolic changes in the traumatically injured rat brain. High-resolution in vivo proton magnetic resonance spectroscopy at 7 T

    Energy Technology Data Exchange (ETDEWEB)

    Li, Jing; Zhao, Can; Rao, Jia-Sheng [Beihang University, Beijing Key Laboratory for Biomaterials and Neural Regeneration, School of Biological Science and Medical Engineering, Beijing (China); Yang, Fei-Xiang; Yang, Zhao-Yang [Capital Medical University, Department of Neurobiology, School of Basic Medical Sciences, Beijing (China); Wang, Zhan-Jing; Lei, Jian-Feng [Capital Medical University, Medical Experiment and Test Center, Beijing (China); Li, Xiao-Guang [Beihang University, Beijing Key Laboratory for Biomaterials and Neural Regeneration, School of Biological Science and Medical Engineering, Beijing (China); Capital Medical University, Department of Neurobiology, School of Basic Medical Sciences, Beijing (China)

    2017-12-15

    The understanding of microstructural and metabolic changes in the post-traumatic brain injury is the key to brain damage suppression and repair in clinics. Ten female Wistar rats were traumatically injured in the brain CA1 region and above the cortex. Next, diffusion tensor magnetic resonance imaging (DTI) and proton magnetic resonance spectroscopy ({sup 1}H MRS) were used to analyze the microstructural and metabolic changes in the brain within the following 2 weeks. Anisotropy fraction (FA) and axial diffusivity (AD) of the corpus callosum (CC) began to decrease significantly at day 1, whereas radial diffusivity (RD) significantly increased immediately after injury, reflecting the loss of white matter integrity. Compared with day 3, RD decreased significantly at day 7, implicating the angioedema reduction. In the hippocampus, FA significantly increased at day 7; the choline-containing compounds (Cho) and myo-inositol (MI) remarkably increased at day 7 compared with those at day 3, indicating the proliferation of astrocytes and radial glial cells after day 7. No significant differences between DTI and {sup 1}H MRS parameters were observed between day 1 and day 3. Day 1-3 after traumatic brain injury (TBI) may serve as a relatively appropriate time window for treatment planning and the following nerve repair. (orig.)

  13. The EAACI/GA2LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update

    Directory of Open Access Journals (Sweden)

    T. Zuberbier

    2015-05-01

    Full Text Available This guideline is the result of a systematic literature review using the ‘Grading of Recommendations Assessment, Development and Evaluation’ (GRADE methodology and a structured consensus conference held on 28 and 29 November 2012, in Berlin. It is a joint initiative of the Dermatology Section of the European Academy of Allergy and Clinical Immunology (EAACI, the EU-funded network of excellence, the Global Allergy and Asthma European Network (GA2LEN, the European Dermatology Forum (EDF, and the World Allergy Organization (WAO with the participation of delegates of 21 national and international societies. Urticaria is a frequent, mast cell-driven disease, presenting with wheals, angioedema, or both. The life-time prevalence for acute urticaria is approximately 20%. Chronic spontaneous urticaria and other chronic forms of urticaria do not only cause a decrease in quality of life, but also affect performance at work and school and, as such, are members of the group of severe allergic diseases. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS.

  14. [Anaesthesic management of vaginal delivery in a parturient with C1 esterase deficiency].

    Science.gov (United States)

    Libert, N; Schérier, S; Dubost, C; Franck, L; Rouquette, I; Tortosa, J-C; Rousseau, J-M

    2009-04-01

    Hereditary and acquired angioedema (HAE/AAE) are the clinical translation of a qualitative or a quantitative deficit of C1 esterase inhibitor (C1 INH). The frequency and severity of clinical manifestations vary greatly, ranging from a moderate swelling of the extremities to obstruction of upper airway. Anaesthesiologists and intensivists must be prepared to manage acute manifestations of this disease in case of life-threatening laryngeal edema. Surgery, physical trauma and labour are classical triggers of the disease. The anaesthesiologists should be aware of the drugs used as prophylaxis and treatment of acute attacks when considering labour and caesarean section. Androgens are contraindicated during pregnancy. If prophylaxis is required, tranexamic acid may be used with caution. The safest obstetric approach appears to be to administer a predelivery infusion of C1 INH concentrate. It is important to avoid manipulation of the airway as much as possible by relying on regional techniques. We report the case of a patient suffering from an HAE discovered during pregnancy. The management included administration of C1 INH during labor and early epidural analgesia for pain relief. A short review of the pathophysiology and therapeutic options follows.

  15. Drug hypersensitivity in children: report from the pediatric task force of the EAACI Drug Allergy Interest Group.

    Science.gov (United States)

    Gomes, E R; Brockow, K; Kuyucu, S; Saretta, F; Mori, F; Blanca-Lopez, N; Ott, H; Atanaskovic-Markovic, M; Kidon, M; Caubet, J-C; Terreehorst, I

    2016-02-01

    When questioned, about 10% of the parents report suspected hypersensitivity to at least one drug in their children. However, only a few of these reactions can be confirmed as allergic after a diagnostic workup. There is still a lack of knowledge on drug hypersensitivity (DH) epidemiology, clinical spectrum, and appropriate diagnostic methods particularly in children. Meanwhile, the tools used for DH management in adults are applied also for children. Whereas this appears generally acceptable, some aspects of DH and management differ with age. Most reactions in children are still attributed to betalactams. Some manifestations, such as nonsteroidal anti-inflammatory drug-associated angioedema and serum sickness-like reactions, are more frequent among young patients as compared to adults. Risk factors such as viral infections are particularly frequent in children, making the diagnosis challenging. The practicability and validity of skin test and other diagnostic procedures need further assessment in children. This study presents an up-to-date review on epidemiology, clinical spectrum, diagnostic tools, and current management of DH in children. A new general algorithm for the study of these reactions in children is proposed. Data are presented focusing on reported differences between pediatric and adult patients, also identifying unmet needs to be addressed in further research. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  16. Human basophil degranulation test in drug allergy.

    Science.gov (United States)

    Sastre Domínguez, J; Sastre Castillo, A

    1986-01-01

    We have evaluated the usefulness of HBDT as an in vitro method for the diagnosis of drug allergy. Two hundred and thirty six patients with suspected drug sensitization to penicillin, streptomycin, sulfamides, pyrazolones and A.S.A. were analyzed. Seventy-nine of them were allergic; in 43 cases it was confirmed by in vivo methods. Other patients were diagnosed by clinical history only if they had more than two reactions to the same drug. In order to be included in this group patients with reactions to pyrazolones and A.S.A. had to have tolerated other NSAI, therefore these patients were allergic to one compound only. All patients were considered non-allergic were determined by a negative provocation test. In the group of allergic patients we obtained 63 (79%) positive degranulations and 16 (21%) negative. One hundred and thirty two (84%) negative degranulations and 25 (16%) positive were obtained in the group of non-allergic patients. Once having analyzed 10 statistical parameters with each drug, the HBOT appears to be a useful method for these drugs except for streptomycin. In 16 (80%) out of 20 aspirin sensitive asthmatic patients we found that their basophils were degranulated. In 7 patients with urticaria and/or angioedema by A.S.A. and other NSAI the degranulation was negative, confirming the absence of the involvement of basophils in this reactions.

  17. Interações de nanotubos de carbono e fulerenos com o sistema imune da pele e as possíveis implicações relacionadas à nanotoxicidade cutânea

    Directory of Open Access Journals (Sweden)

    Ana Luiza Castro Fernandes

    2013-11-01

    Full Text Available O entendimento da interação de nanotubos de carbonos e fulerenos com os constituintes da pele, em especial o sistema imune de pele, é relevante para a determinação de parâmetros toxicológicos. Realizou-se revisão voltada a tais aspectos. Parte considerável das referências encontradas concentrou-se nos efeitos citotóxicos e de permeação da pele. Em menor escala, há artigos sobre ativação e imunomodulação de células e outros elementos imunes. Poucos trabalhos encontrados tratam, especificamente, da resposta imunológica cutânea, limitando o conhecimento relacionado. Os achados sugerem que os nanomateriais analisados possam estar envolvidos em quadros dermatológicos, como dermatite de contato irritativa, reações anafilactoides, urticária e angioedema, e suscitam a necessidade de que se realizem estudos adicionais para confirmação dos achados. A padronização da descrição e testagem de características dos nanomateriais empregados em experimentos pode facilitar a comparação dos resultados.

  18. Potential immediate hypersensitivity reactions following immunization in preschool aged children in Victoria, Australia.

    Science.gov (United States)

    Baxter, C-M; Clothier, H J; Perrett, K P

    2018-04-06

    Immediate hypersensitivity reactions (IHR) are rare but potentially serious adverse events following immunization (AEFI). Surveillance of Adverse Events following Vaccination in the Community (SAEFVIC) is an enhanced passive surveillance system that collects, analyses and reports information about AEFI in Victoria, Australia. We describe the incidence, timing and type of potential IHR following vaccination in preschool children reported over an 8-year period. A total of 2110 AEFI were reported in 1620 children, of which 23.5% (496) were classified as potential IHR. Of these, 37.1% (184) were suspected to be IgE-mediated, (including anaphylaxis, angioedema and/or urticaria) and 83.5% (414) occurred within 15 minutes of vaccination. The incidence of potential IHR was 5.4 per 100,000 doses, with that of suspected IgE-mediated IHR being 2.0 per 100,000 doses. The incidence of anaphylaxis was extremely low (0.13 per 100,000 doses) and is consistent with other published studies. Potential IHR following immunization should be reported to appropriate local pharmacovigilance systems and patients reviewed by specialists able to evaluate, investigate and manage future vaccinations.

  19. Obstrucción aguda de la vía respiratoria superior y rabdomiolisis luego de intoxicación por tintura para el cabello Acute upper respiratory obstruction and rhabdomiolysis due to intoxication with a hair dye

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    Claudia Arroyave

    2004-09-01

    Full Text Available Las intoxicaciones por tinturas para el cabello son infrecuentes en Antioquia; sin embargo, su toxicidad es potencialmente letal cuando ingresan al organismo por vía oral al producir un compromiso multiorgánico que puede llevar a la muerte del paciente. Se presenta el caso de una paciente de 2 años que ingirió una dosis de 125 mg/kg de parafenilendiamina, asociada a ácido bórico y perborato de sodio, compuestos que hacen parte de una presentación comercial de una tintura para el cabello; la intoxicación por parafenilendiamina tuvo como consecuencia principal la obstrucción de la vía aérea por angioedema que requirió intubación endotraqueal, soporte ventilatorio y administración de esteroides y antihistamínicos; secundario al compromiso aéreo la paciente presentó edema agudo de pulmón y posteriormente un cuadro neumónico que requirió tratamiento con antibióticos. Adicionalmente presentó rabdomiolisis severa que se trató con líquidos endovenosos para que no se comprometiera la función renal. Luego de una terapia durante 10 días en el hospital, la niña fue dada de alta con resolución completa del cuadro tóxico. Se revisan los posibles mecanismos fisiopatológicos, las manifestaciones clínicas y el tratamiento de la intoxicación por parafenilendiamina, dado que este fue el compuesto tóxico clínicamente importante en el contexto de este caso. There have been few cases reported of intoxication by hair dyes in Antioquia; however, their toxicity may be potentially lethal when their components enter the organism by oral route inducing a multiorganic compromise that may lead to death. We report the case of a two year old girl who ingested 125mg/kg of paraphenylenediamine, with boric acid and sodium perborate; these compounds are part of the commercial presentation of a hair dye; the child suffered airway obstruction because of angioedema that required intubation, ventila tory support, steroids and antihistaminics; due to

  20. Efeitos adversos durante a infusão de infliximabe em crianças e adolescentes: estudo multicêntrico Adverse reactions during infliximab infusion in children and adolescents: multicenter study

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    Cássia Maria Passarelli Lupoli Barbosa

    2008-10-01

    Full Text Available OBJETIVO: Avaliar a freqüência e a gravidade dos efeitos adversos durante a infusão de infliximabe. MATERIAL E MÉTODOS: Avaliação retrospectiva de prontuários de 58 pacientes acompanhados em cinco serviços de reumatologia pediátrica. Todos apresentavam doença refratária ou eram intolerantes ao uso de uma ou mais drogas modificadoras de doença e receberam uma ou mais infusões de infliximabe. A análise estatística foi descritiva, levando-se em conta a freqüência e o tipo dos efeitos adversos, assim como sua relação com o número de infusões, a dose de infliximabe e a indicação. RESULTADOS: A média da idade na época da introdução do infliximabe foi de 12 anos e 9 meses e a média do tempo de evolução da doença foi de 7 anos e 7 meses. A principal indicação da medicação foi artrite idiopática juvenil (43. Dos 58 pacientes avaliados, 25 (43,1% apresentaram efeitos adversos durante a infusão e em 17 (29,3%, a medicação teve de ser suspensa. As reações que ocorreram foram: dispnéia (dez, náuseas e vômitos (oito, rash cutâneo (sete, choque anafilático (seis, rubor facial (cinco, angioedema (quatro, dor torácica (quatro, urticária e hipertensão, entre outras. A reação anafilática foi mais freqüente entre a quarta e a sexta infusão. CONCLUSÃO: A freqüência e a gravidade dos efeitos adversos ao infliximabe devem ser levadas em conta, não apenas por ocasião da sua indicação, mas principalmente por ocasião da escolha do local para a sua aplicação, que deve contar com estrutura para atendimento de urgência.OBJECTIVE: To evaluate the frequency and the severity of the adverse reactions during the infusion of infliximab. METHODS: We performed a retrospective chart review of 58 patients, followed up at 5 paediatric rheumatology centers. All patients presented refractory disease or were intolerant to one or more disease modifying drugs and received one or more infliximab infusions. The data analysis

  1. Acute adverse events associated with the administration of Crotalidae polyvalent immune Fab antivenom within the North American Snakebite Registry.

    Science.gov (United States)

    Kleinschmidt, Kurt; Ruha, Anne-Michelle; Campleman, Sharan; Brent, Jeffrey; Wax, Paul

    2018-04-24

    Crotalidae Polyvalent Immune Fab (Fab Antivenom) is the primary Viperid antivenom used in the United States since 2000. Adverse event data associated with its use are limited. The purpose of this study is to describe the prevalence of acute adverse events associated with the use of Fab antivenom. The American College of Medical Toxicology's Toxicology Investigators Consortium maintains a prospective case registry of poisoned and envenomated patients managed by medical toxicologists at the bedside. This registry includes the North American Snakebite sub-registry. We performed a review of 438 cases entered into the Snakebite sub-registry. A total of 373 (85.2%) received at least one vial of Fab Antivenom. Forty percent were children. Adverse events occurred in 10 patients (2.7%) of whom six were adults. Rash was the most common adverse event. More severe adverse events (hypotension, bronchospasm, and/or angioedema) occurred in four (1.1%) patients. Prophylaxis was administered prior to Fab antivenom in 4.0%. Eight patients received various treatments for their adverse events. Neither the initial number of Fab antivenom vials, atopic history, nor prior envenomation correlated with the prevalence of adverse events. This prevalence of adverse events was lower than in previous studies and in a meta-analysis of 11 studies. The types of adverse events and treatments used are consistent with those in previous reports. There were no prior reports of prophylaxis use with which to compare. The prevalence of Fab antivenom adverse events in the North American Snakebite Registry was 2.7%.

  2. Analysis of characteristics associated with reinjection of icatibant: Results from the icatibant outcome survey.

    Science.gov (United States)

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

    2015-01-01

    Phase 3 icatibant trials showed that most hereditary angioedema (HAE) (C1 inhibitor deficiency) acute attacks were treated successfully with one injection of icatibant, a selective bradykinin B2 receptor antagonist. We conducted a post hoc analysis of icatibant reinjection for HAE type I and II attacks in a real-world setting by using data from the Icatibant Outcome Survey, an ongoing observational study that monitors the safety and effectiveness of icatibant treatment. Descriptive retrospective analyses of icatibant reinjection were performed on Icatibant Outcome Survey data (February 2008 to December 2012). New attacks were defined as the onset of new symptoms after full resolution of the previous attack. Potential associations between the patient and attack characteristics and reinjection were explored by using logistic regression analysis. Icatibant was administered for 652 attacks in 170 patients with HAE type I or II. Most attacks (89.1%) were treated with a single icatibant injection. For attacks that required two or three injections, the second injection was given a median of 11.0 hours after the first injection, with 90.4% of second injections administered ≥6 hours after the first injection. Time to resolution and attack duration were significantly longer for two or three injections versus one icatibant injection (p < 0.0001 and p < 0.05, respectively). Multivariate logistic regression analysis identified sex, attack severity, and laryngeal attacks as significantly correlated with reinjection (all p ≤ 0.05). These factors did not remain predictors for reinjection when two outlier patients with distinct patterns of icatibant use were excluded. In this real-world setting, most HAE attacks resolved with one icatibant injection. There was no distinct profile for patients or attacks that required reinjection when outliers with substantially different patterns of use were excluded. Because new attacks were not distinguished from the recurrence of symptoms

  3. The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients.

    Science.gov (United States)

    Jachiet, Marie; Flageul, Béatrice; Deroux, Alban; Le Quellec, Alain; Maurier, François; Cordoliani, Florence; Godmer, Pascal; Abasq, Claire; Astudillo, Leonardo; Belenotti, Pauline; Bessis, Didier; Bigot, Adrien; Doutre, Marie-Sylvie; Ebbo, Mikaël; Guichard, Isabelle; Hachulla, Eric; Héron, Emmanuel; Jeudy, Géraldine; Jourde-Chiche, Noémie; Jullien, Denis; Lavigne, Christian; Machet, Laurent; Macher, Marie-Alice; Martel, Clotilde; Melboucy-Belkhir, Sara; Morice, Cécile; Petit, Antoine; Simorre, Bernard; Zenone, Thierry; Bouillet, Laurence; Bagot, Martine; Frémeaux-Bacchi, Véronique; Guillevin, Loïc; Mouthon, Luc; Dupin, Nicolas; Aractingi, Selim; Terrier, Benjamin

    2015-02-01

    Hypocomplementemic urticarial vasculitis (HUV) is an uncommon vasculitis of unknown etiology that is rarely described in the literature. We undertook this study to analyze the clinical spectrum and the therapeutic management of patients with HUV. We conducted a French nationwide retrospective study that included 57 patients with chronic urticaria, histologic leukocytoclastic vasculitis, and hypocomplementemia. We assessed clinical and laboratory data and evaluated the patients' cutaneous and immunologic responses to therapy. We evaluated treatment efficacy by measuring the time to treatment failure. Urticarial lesions were typically more pruritic than painful and were associated with angioedema in 51% of patients, purpura in 35%, and livedo reticularis in 14%. Extracutaneous manifestations included constitutional symptoms (in 56% of patients) as well as musculoskeletal involvement (in 82%), ocular involvement (in 56%), pulmonary involvement (in 19%), gastrointestinal involvement (in 18%), and kidney involvement (in 14%). Patients with HUV typically presented with low C1q levels and normal C1 inhibitor levels, in association with anti-C1q antibodies in 55% of patients. Hydroxychloroquine or colchicine seemed to be as effective as corticosteroids as first-line therapy. In patients with relapsing and/or refractory disease, rates of cutaneous and immunologic response to therapy seemed to be higher with conventional immunosuppressive agents, in particular, azathioprine, mycophenolate mofetil, or cyclophosphamide, while a rituximab-based regimen tended to have higher efficacy. Finally, a cutaneous response to therapy was strongly associated with an immunologic response to therapy. HUV represents an uncommon systemic and relapsing vasculitis with various manifestations, mainly, musculoskeletal and ocular involvement associated with anti-C1q antibodies, which were found in approximately half of the patients. The best strategy for treating HUV has yet to be defined

  4. Chloramine-induced anaphylaxis while showering: a case report.

    Science.gov (United States)

    D'Alò, Simona; De Pasquale, Tiziana; Incorvaia, Cristoforo; Illuminati, Ilenia; Mistrello, Gianni; Roncarolo, Daniela; Pucci, Stefano

    2012-09-25

    Sodium-N-chlorine-p-toluene sulfonamide, commonly known as chloramine-T, is a derivative of chlorine which is widely used as a disinfectant. For many years, chloramine-T has been described as a cause of immediate-type hypersensitivity, especially with regard to asthma and rhinitis, and as a cause of occupational dermatoses in cleaning personnel in hospitals, although no anaphylactic reaction has yet been reported. Hence, to the best of our knowledge we present the first case of anaphylaxis to chloramine-T with evidence of specific immunoglobulin E antibodies. We describe the case of a 25-year-old Caucasian woman who was in good health and with a negative history for atopy, including no respiratory symptoms of rhinitis or asthma, and with no professional exposure to chloramine-T. She, while showering, applied a chloramine-T solution to a skin area with folliculitis on her leg, and within a few minutes developed generalized urticaria and angioedema, followed by vomiting and collapse with loss of consciousness. A skin prick test with a chloramine-T solution at 10mg/mL concentration was positive, and specific immunoglobulin E to chloramine-T was quantified at a value of 2.9 optical density as measured by the enzyme allergosorbent test technique. The strict cause-effect relationship and the results of the skin test and the in vitro test make certain the causative role of chloramine-T in this case of anaphylaxis. This suggests that chloramine-T, based on its wide use as a disinfectant, should be considered a possible cause in anaphylaxis of unknown origin.

  5. Chloramine-induced anaphylaxis while showering: a case report

    Directory of Open Access Journals (Sweden)

    D’Alò Simona

    2012-09-01

    Full Text Available Abstract Introduction Sodium-N-chlorine-p-toluene sulfonamide, commonly known as chloramine-T, is a derivative of chlorine which is widely used as a disinfectant. For many years, chloramine-T has been described as a cause of immediate-type hypersensitivity, especially with regard to asthma and rhinitis, and as a cause of occupational dermatoses in cleaning personnel in hospitals, although no anaphylactic reaction has yet been reported. Hence, to the best of our knowledge we present the first case of anaphylaxis to chloramine-T with evidence of specific immunoglobulin E antibodies. Case presentation We describe the case of a 25-year-old Caucasian woman who was in good health and with a negative history for atopy, including no respiratory symptoms of rhinitis or asthma, and with no professional exposure to chloramine-T. She, while showering, applied a chloramine-T solution to a skin area with folliculitis on her leg, and within a few minutes developed generalized urticaria and angioedema, followed by vomiting and collapse with loss of consciousness. A skin prick test with a chloramine-T solution at 10mg/mL concentration was positive, and specific immunoglobulin E to chloramine-T was quantified at a value of 2.9 optical density as measured by the enzyme allergosorbent test technique. Conclusion The strict cause-effect relationship and the results of the skin test and the in vitro test make certain the causative role of chloramine-T in this case of anaphylaxis. This suggests that chloramine-T, based on its wide use as a disinfectant, should be considered a possible cause in anaphylaxis of unknown origin.

  6. Long-term (52-week) safety and efficacy of Sacubitril/valsartan in Asian patients with hypertension.

    Science.gov (United States)

    Supasyndh, Ouppatham; Sun, Ningling; Kario, Kazuomi; Hafeez, Kudsia; Zhang, Jack

    2017-05-01

    Sacubitril/valsartan (LCZ696), a first-in-class angiotensin receptor-neprilysin inhibitor, demonstrated significant reductions in office and 24 h ambulatory blood pressure (BP) over 8 weeks in Asian patients with hypertension. This 52-week extension to the 8-week core study was aimed at evaluating the long-term safety, tolerability and efficacy of sacubitril/valsartan. Patients who completed an 8-week randomized study (the core study) were enrolled in this 52-week open-label study and received sacubitril/valsartan 200 mg QD. The sacubitril/valsartan dose was uptitrated to 400 mg QD if BP was uncontrolled (>140/90 mm Hg) after 4 weeks. Subsequently, in patients with uncontrolled BP, treatment was intensified every 4 weeks with amlodipine 5-10 mg followed by hydrochlorothiazide 6.25-25 mg. Of the 341 patients enrolled, 7 (2.1%) discontinued the study drug due to adverse events (AEs). The incidence of AEs and serious AEs were 63.9 and 3.8%, respectively, and no deaths were reported in this study. The most frequent AEs were nasopharyngitis (18.2%) and dizziness (8.8%). Events that were potentially indicative of low BP were infrequent. One patient reported mild transient angioedema (lasting 2.5 h) that resolved without treatment but led to study drug discontinuation. The sacubitril/valsartan-based regimen provided clinically significant mean sitting systolic BP (msSBP) and mean sitting diastolic BP (msDBP) reductions from baseline (-24.7/-16.2 mm Hg). The overall BP control, msSBP and msDBP response rates were 75.3, 90.6 and 87.6%, respectively. Long-term use of sacubitril/valsartan was generally safe and well-tolerated in patients with hypertension and provided significant BP reductions from baseline.

  7. Rationale and design of the comParIson Of sacubitril/valsartaN versus Enalapril on Effect on nt-pRo-bnp in patients stabilized from an acute Heart Failure episode (PIONEER-HF) trial.

    Science.gov (United States)

    Velazquez, Eric J; Morrow, David A; DeVore, Adam D; Ambrosy, Andrew P; Duffy, Carol I; McCague, Kevin; Hernandez, Adrian F; Rocha, Ricardo A; Braunwald, Eugene

    2018-04-01

    The objective is to assess the safety, tolerability, and efficacy of sacubitril/valsartan compared with enalapril in patients with heart failure (HF) with a reduced ejection fraction (EF) stabilized during hospitalization for acute decompensated HF. Sacubitril/valsartan, a first-in-class angiotensin receptor-neprilysin inhibitor, improves survival among ambulatory HF patients with a reduced EF. However, there is very limited experience with the in-hospital initiation of sacubitril/valsartan in patients who have been stabilized following hospitalization for acute decompensated HF. PIONEER-HF is a 12-week, prospective, multicenter, double-blind, randomized controlled trial enrolling a planned 882 patients at more than 100 participating sites in the United States. Medically stable patients >18 years of age with an EF 1600 pg/mL or b-type natriuretic peptide >400 pg/mL are eligible for participation no earlier than 24 hours and up to 10 days from initial presentation while still hospitalized. Patients are randomly assigned 1:1 to in-hospital initiation of sacubitril/valsartan titrated to 97/103 mg by mouth twice daily versus enalapril titrated to 10 mg by mouth twice daily for 8 weeks. All patients receive open-label treatment with sacubitril/valsartan for the remaining 4 weeks of the study. The primary efficacy end point is the time-averaged proportional change in amino terminal-pro b-type natriuretic peptide from baseline through weeks 4 and 8. Secondary and exploratory end points include serum and urinary biomarkers as well as clinical outcomes. Safety end points include the incidence of angioedema, hypotension, renal insufficiency, and hyperkalemia. The PIONEER-HF trial will inform clinical practice by providing evidence on the safety, tolerability, and efficacy of in-hospital initiation of sacubitril/valsartan among patients who have been stabilized following an admission for acute decompensated HF with a reduced EF. Copyright © 2018 Elsevier Inc. All rights

  8. Pharmacokinetic, pharmacodynamic, and antihypertensive effects of the neprilysin inhibitor LCZ-696: sacubitril/valsartan.

    Science.gov (United States)

    Chrysant, Steven G

    2017-07-01

    LCZ-696, sacubitril/valsartan, is a dual-acting molecule consisting of the angiotensin II (Ang II) receptor blocker valsartan and the neprilysin (neutral endopeptidase) inhibitor AHU-377 with significant beneficial effects in patients with hypertension and heart failure (HF). Several recent studies have demonstrated a higher effectiveness of LCZ-696 compared to valsartan in the treatment of hypertension and HF. The rationale for the development and the Food and Drug Administration approval of LCZ-696 was based on the concept of an additive effect of the Ang II receptor blocker valsartan and the neutral endopeptidase (neprilysin) inhibitor AHU-377 for the treatment of hypertension and HF. The synergism from these drugs arises from the vasodilating effects of valsartan through its blockade of Ang II type 1 receptor and the action of natriuretic peptides atrial natriuretic peptide and B-type natriuretic peptide (BNP) by preventing their catabolism with neprilysin resulting in increase of cyclic guanosine monophosphate. This action of neprilysin is associated with increased natriuresis, diuresis, and systemic vasodilation, since these peptides have been shown to have potent diuretic, natriuretic, and vasodilating effects. In addition, it reduces the levels of N terminal pro-BNP. Therefore, administration of LCZ-696 results in significant reduction of wall stress from pressure and volume overload of the left ventricle as demonstrated by the reduction of N terminal pro-BNP, both significant constituents of hypertension and HF, and it is safe, well tolerated and is almost free of cough and angioedema. Copyright © 2017 American Society of Hypertension. Published by Elsevier Inc. All rights reserved.

  9. Sacubitril/Valsartan (LCZ696) in Heart Failure.

    Science.gov (United States)

    Khder, Yasser; Shi, Victor; McMurray, John J V; Lefkowitz, Martin P

    2017-01-01

    It has been known since the 1990s that long-term morbidity and mortality is improved in patients with heart failure with reduced ejection fraction (HFrEF) by treatments that target the renin-angiotensin-aldosterone system (RAAS). It has also long been thought that enhancement of the activity of natriuretic peptides (NPs) could potentially benefit patients with HFrEF, but multiple attempts to realize this benefit had failed over the years - until 2014, when a large, phase III, randomized, controlled clinical trial (PARADIGM-HF) was completed comparing sacubitril/valsartan with enalapril, a well-established treatment for HFrEF. Sacubitril/valsartan (formerly known as LCZ696) is a first-in-class angiotensin receptor neprilysin inhibitor (ARNI) that simultaneously suppresses RAAS activation through blockade of angiotensin II type 1 receptors and enhances vasoactive peptides including NPs through inhibition of neprilysin, the enzyme responsible for their degradation. In PARADIGM-HF, patients with HFrEF treated with sacubitril/valsartan had 20% less risk for cardiovascular death or hospitalization for heart failure (the primary endpoint), 20% less risk for cardiovascular death, 21% less risk for first hospitalization for heart failure, and 16% less risk for death from any cause, compared with enalapril (all p sacubitril/valsartan group had higher proportions of patients with hypotension and nonserious angioedema but lower proportions with renal impairment, hyperkalemia, and cough, compared with the enalapril group. The use of sacubitril/valsartan has been endorsed by the latest heart failure treatment guidelines in Europe and the USA. This chapter reviews the discoveries, scientific reasoning, and clinical evidence that led to the development of sacubitril/valsartan, the first novel therapy in a new drug class to improve survival in HFrEF in the last 15 years.

  10. Sacubitril/Valsartan: The Newest Addition to the Toolbox for Guideline-Directed Medical Therapy of Heart Failure.

    Science.gov (United States)

    Rodgers, Jo E

    2017-06-01

    Sacubitril/valsartan combines a neprilysin inhibitor with an angiotensin receptor blocker. As an inhibitor of neprilysin, an enzyme that degrades biologically active natriuretic peptides, this first-in-class therapy increases levels of circulating natriuretic peptides, resulting in natriuretic, diuretic, and vasodilatory effects. In patients with chronic New York Heart Association class II-IV heart failure with reduced ejection fraction, the PARADIGM-HF trial demonstrated that sacubitril/valsartan significantly reduced the primary endpoint of cardiovascular mortality and heart failure hospitalization, compared with enalapril. The rate of all-cause mortality was also significantly reduced. Subsequently, the American College of Cardiology/American Heart Association/Heart Failure Society of America recently updated guideline recommendations for Stage C patients with heart failure with reduced ejection fraction to recommend angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, or sacubitril/valsartan in conjunction with other evidence-based therapies to reduce morbidity and mortality. Several analyses have suggested the cost-effectiveness of this new therapy. To ensure tolerability, initiating the lower dosage form of sacubitril/valsartan is warranted in patients with severe renal impairment, moderate hepatic impairment, and low blood pressure, and close monitoring is warranted in such patients. A 36-hour washout period is recommended when switching patients from an angiotensin-converting enzyme inhibitor to sacubitril/valsartan. Similarly, sacubitril/valsartan is contraindicated in patients receiving concomitant angiotensin-converting enzyme inhibitor or angiotensin receptor blocker and those with a history of angioedema. Copyright © 2017 Elsevier Inc. All rights reserved.

  11. Psychiatric morbidity in dermatology patients: Frequency and results of consultations

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    Seyhan Muammer

    2006-01-01

    Full Text Available BACKGROUND: Dermatological patients quite commonly depict psychiatric morbidity. PURPOSES: To study the psychiatric morbidity among skin patients of our clinic. METHODS: In the present study, the patients who were treated in the Dermatology Clinic of Inonu University Medical Faculty were evaluated retrospectively. The age, gender, marital status, habits, dermatological and systemic diseases, previously used drugs, current therapy and psychiatric diagnosis of each patient were recorded. FINDINGS: Of 636 patients involved in the study, 15.3% had psychopathological problems, which were depression (32.0%, adjustment difficulty (15.5%, anxiety (13.4%, psychosomatic disorders (10.3%, obsessive-compulsive disorder and conversion (5.1%, dysthymic disorder (4.1%, attention deficit and hyperactivity disorder (2.1%, panic attack (1.0%, premenstrual syndrome, schizophrenia, somatization disorder, insomnia, alcohol dependency, bipolar affective disorder, mental retardation, agoraphobia, social phobia and dementia. The dermatological diseases defined for the patients with psychopathology diagnosis were chronic urticaria (25.8%; psoriasis (15.5%; alopecia areata, totalis and iniversalis (11.3%; acute urticaria, neurodermatitis and Behcet′s disease (5.1%; atopic dermatitis and drug eruptions (4.1%; pemphigus (3.1%; angioedema, contact dermatitis and generalized pruritus (2.1%; folliculitis and the others (1.0%. CONCLUSIONS: Psychiatric morbidity has an affect on the course of dermatological diseases. When required, psychiatric consultation should be sought by dermatology clinics and patients should be followed with the cooperation of dermatologists and psychiatrists. LIMITATION: The indoor-based study had not included any control group and any domicillary patient.

  12. A Retrospective Study of Epidemiological and Clinical Patterns of ACDRs in Goa Medical College over a 6 Year Period

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    Rakhi Ghodge

    2017-04-01

    Full Text Available Background: Adverse Cutaneous Drug Reactions (ACDRs account for 3% of all hospitalizations. The spectrum of drug reactions can be varied from mild to life threatening forms. Since the diagnosis of ACDR is purely clinical, early and prompt identification and withdrawal of drug (s is life saving for the patient. Aim and Objectives: To study the epidemiological and common clinical patterns and drugs causing ACDRs in tertiary care hospital of Goa Medical College, Goa over a 6 year period. Material and Methods:This was a retrospective study conducted over a period of 6 years. The medical records were analyzed for demographic profiles, morphology of drug eruptions, common groups of drugs involved, presence of co-morbid factors, systemic and mucosal involvement, common haematological abnormalities encountered, time interval between drug intake and onset of rash and mortality. Results: Our study population had 256 patients and the age group of 21-40 years was commonly affected. Maculopapular rash followed by angioedema were the commonest morphology of drug rash patterns encountered in our study. The time interval between consumption of drugs and onset of ACDR varied with interval of 1-7 days being the commonest group in having 158 (61.7% patients. Antibiotics followed by anticonvulsants and antiretrovirals were the commonest groups of drugs causing ACDR. We found that significant proportion of our patients had haematological, renal and hepatic system involvement. Conclusion: Early identification and withdrawal of the culprit drug remains the cornerstone in prevention of mortalities in ACDRs. A prior knowledge about the reaction patterns and common offending drugs in the population by the treating physician cannot be overemphasized.

  13. Clinical Practice Guidelines for Diagnosis and Management of Hypersensitivity Reactions to Contrast Media.

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    Rosado Ingelmo, A; Doña Diaz, I; Cabañas Moreno, R; Moya Quesada, M C; García-Avilés, C; García Nuñez, I; Martínez Tadeo, J I; Mielgo Ballesteros, R; Ortega-Rodríguez, N; Padial Vilchez, M A; Sánchez-Morillas, L; Vila Albelda, C; Moreno Rodilla, E; Torres Jaén, M J

    2016-01-01

    The objective of these guidelines is to ensure efficient and effective clinical practice. The panel of experts who produced this consensus document developed a research protocol based on a review of the literature. The prevalence of allergic reactions to iodinated contrast media (ICM) is estimated to be 1:170 000, that is, 0.05%-0.1% of patients undergoing radiologic studies with ICM (more than 75 million examinations per year worldwide). Hypersensitivity reactions can appear within the first hour after administration (immediate reactions) or from more than 1 hour to several days after administration (nonimmediate or delayed reactions). The risk factors for immediate reactions include poorly controlled bronchial asthma, concomitant medication (eg, angiotensin-converting enzyme inhibitors, ß-blockers, and proton-pump inhibitors), rapid administration of the ICM, mastocytosis, autoimmune diseases, and viral infections. The most common symptoms of immediate reactions are erythema and urticaria with or without angioedema, which appear in more than 70% of patients. Maculopapular rash is the most common skin feature of nonimmediate reactions (30%-90%). Skin and in vitro tests should be performed for diagnosis of both immediate and nonimmediate reactions. The ICM to be administered will therefore be chosen depending on the results of these tests, the ICM that induced the reaction (when known), the severity of the reaction, the availability of alternative ICM, and the information available on potential ICM cross-reactivity. Another type of contrast media, gadolinium derivatives, is used used for magnetic resonance imaging. Although rare, IgE-mediated reactions to gadolinium derivatives have been reported.

  14. A STUDY ON PAEDIATRIC DERMATOSES IN CHILDREN ATTENDING OPD OF GVR HOSPITAL, KURNOOL

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    M. Bhuvaneswari

    2018-02-01

    Full Text Available BACKGROUND Paediatric dermatoses include various diseases of neonates such as sebaceous hyperplasia, milia, sucking blisters etc., cutaneous infections such as impetigo, folliculitis, furuncles etc., arthropod bites and infestations such as scabies, pediculosis, nutritional dermatoses such as acrodermatitis enteropathica, essential fatty acid deficiency, kwashiorkor etc., eczematous disorders such as atopic dermatitis, seborrhoeic dermatitis, etc., vesiculobullous disorders such as erythema multiforme, SJS/TEN, hypersensitivity disorders like urticaria, papular urticaria, angioedema etc., photosensitivity diseases like acute sunburn reactions, phototoxic reactions, porphyrias etc., epidermal diseases like psoriasis, lichen planus etc., keratinisation disorders like ichthyosis vulgaris, palmoplantar keratosis, etc., The epidemiological aspects of various childhood dermatoses have been the subject of study by various researchers for over a century, but the accurate incidence or prevalence could not be made out because: lack of properly designed guidelines for evaluation, inadequate and improper compilation, lack of parental awareness and knowledge about the problems, home remedies, treatment by quacks not being reported. MATERIALS AND METHODS It is a hospital based observational study, which was conducted at Govt. general hospital, Kurnool and GVR Hospital, Kurnool. The study included 14,730 children who attended the above departments, of them, 5775 children were males and 5995 children are females. The study was conducted during the period of 1 year from March 2015-June 2016. RESULTS Infections are the commonest (30.44% followed by Infestations (14.40%. Secondary Infections were the commonest bacterial infections (25.37%. Among the infestations Scabies was the most prevalent (77.76% CONCLUSION In our study, it was observed that 28-30% of the children aged below 12 years attending paediatric outpatient department, GVR Hospital and 30-40% of the

  15. A STUDY ON ADVERSE DRUG REACTIONS INVOLVING CENTRAL NERVOUS SYSTEM, ITS SEVERITY AND CAUSALITY ASSESSMENT IN PEDIATRIC PATIENTS ADMITTED TO A TERTIARY CARE HOSPITAL

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    Arati

    2015-09-01

    Full Text Available A retrospective study was conducted in Department of pediatrics SCB Medical College and SVPPGIP for a period of 2 years i.e. September 2012 to August 2014 . All the patients from birth to 14 years admitted to the pediatric ward in this study were under ADR surveillance. Patients admitted to our hospital with adverse drug reaction o r patients developing adverse drug reaction in our hospital were studied; only those cases where the central nervous system was involved were taken in our study. The cases were compiled and the causality of offending drugs was found using WHO - UMC causality assessment score. The severity of drug reaction in every case was determined by using HARTWIG’s severity scoring scale. Total 350 Adverse reactions were reported in this period with prevalence rate of 2.04% i.e. 20 out of 1000 children faced ADR due to dr ugs, with annual incidence rate of 0.9% and 1.14% over two years. Out of total 350 cases dermatological system was most commonly involved i.e. 207 cases (59.14%. This is followed by involvement of central nervous system 46 number of cases (13.14%. The GI system was involved in 34 cases i.e. (9.71%. Life threatening reactions like anaphylaxis, angioedema and shock like immediate life threatening ADRs were reported in 16 cases. Our study group was the patient in whom the ADR involved the CNS. Out of 46 suc h cases, there were 25 female and 21 male. Various reaction due to drug were encephalopathy , eps, febrile seizure, tremor, head reeling, ototoxicity, persistant cry, pseudotumor cerebri, psychosis, seizure, status epilepticus, toxic amblyopia, tremor, atax ia etc. The most common CNS manifestation was Extra pyramidal side effects (EPS involving 21% of cases. The most common Drug causing CNS manifestation was ATT (HRZE causing blindness, Eps, psychosis , toxic amblyopia blindness etc.

  16. An improved Peltier effect-based instrument for critical temperature threshold measurement in cold- and heat-induced urticaria.

    Science.gov (United States)

    Magerl, M; Abajian, M; Krause, K; Altrichter, S; Siebenhaar, F; Church, M K

    2015-10-01

    Cold- and heat-induced urticaria are chronic physical urticaria conditions in which wheals, angioedema or both are evoked by skin exposure to cold and heat respectively. The diagnostic work up of both conditions should include skin provocation tests and accurate determination of critical temperature thresholds (CTT) for producing symptoms in order to be able to predict the potential risk that each individual patient faces and how this may be ameliorated by therapy. To develop and validate TempTest(®) 4, a simple and relatively inexpensive instrument for the accurate determination of CTT which may be used in clinical practice. TempTest(®) 4 has a single 2 mm wide 350 mm U-shaped Peltier element generating a temperature gradient from 4 °C to 44 °C along its length. Using a clear plastic guide placed over the skin after provocation, CTT values may be determined with an accuracy of ±1 °C. Here, TempTest(®) 4 was compared with its much more expensive predecessor, TempTest(®) 3, in inducing wheals in 30 cold urticaria patients. Both TempTest(®) 4 and TempTest(®) 3 induced wheals in all 30 patients between 8 ° and 28 °C. There was a highly significant (P < 0.0001) correlation between the instruments in the CTT values in individual patients. The TempTest(®) 4 is a simple, easy to use, licensed, commercially available and affordable instrument for the determination of CTTs in both cold- and heat-induced urticaria. © 2014 European Academy of Dermatology and Venereology.

  17. Omalizumab, an anti-immunoglobulin E antibody: state of the art

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    Incorvaia C

    2014-02-01

    Full Text Available Cristoforo Incorvaia,1 Marina Mauro,2 Marina Russello,2 Chiara Formigoni,3 Gian Galeazzo Riario-Sforza,1 Erminia Ridolo41Allergy/Pulmonary Rehabilitation, Istituti Clinici di Perfezionamento Hospital, Milan, Italy; 2Allergy Unit, 3Scientific Library, Sant'Anna Hospital, Como, Italy; 4Department of Clinical and Experimental Medicine, University of Parma, Parma, ItalyAbstract: A large number of trials show that the anti-immunoglobulin (Ig E antibody omalizumab is very effective in patients with severe allergic asthma. This is acknowledged in consensus documents. The drug also has a good safety profile and a pharmacoeconomic advantage due to a reduction in the number of hospitalizations for asthma attacks. In recent years, some studies have shown that omalizumab is effective also in nonallergic asthma. Effects on the complex signaling mechanisms leading to activation of effector cells and to mediator release may account for this outcome. Indeed, omalizumab has been reported to be effective in a number of IgE-mediated and non-IgE-mediated disorders. Concerning the former, clinical efficacy has been observed in rhinitis, allergic bronchopulmonary aspergillosis, latex allergy, atopic dermatitis, allergic urticaria, and anaphylaxis. In addition, omalizumab has been demonstrated to be able to prevent systemic reactions to allergen immunotherapy, thus enabling completion of treatment in patients who otherwise would have to stop it. Concerning non-IgE-mediated disorders, omalizumab has been reported to be effective in nasal polyposis, autoimmune urticaria, chronic idiopathic urticaria, physical urticaria, idiopathic angioedema, and mastocytosis. Current indications for treatment with omalizumab are confined to severe allergic asthma. Consequently, any other prescription can only be off-label. However, it is reasonable to expect that the use of omalizumab will be approved for particularly important indications, such as anaphylaxis, in the near future

  18. Omalizumab for the treatment of chronic spontaneous urticaria: A meta-analysis of randomized clinical trials.

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    Zhao, Zuo-Tao; Ji, Chun-Mei; Yu, Wen-Jun; Meng, Ling; Hawro, Tomasz; Wei, Ji-Fu; Maurer, Marcus

    2016-06-01

    Chronic spontaneous urticaria (CSU) is defined by itchy hives, angioedema, or both for at least 6 weeks. Omalizumab, an anti-IgE antibody that affects mast cell and basophil function, is a promising new treatment option. As of now, however, the efficacy and safety of different doses of omalizumab used in clinical trials for CSU have not been systematically analyzed and summarized. We sought to assess the efficacy and safety of different doses of omalizumab for the treatment of CSU in a meta-analysis of clinical trial results. Suitable trials were identified by searching PubMed, Medline, Embase, and Web of Science databases and with the help of omalizumab's manufacturers. Only double-blind, randomized, placebo-controlled studies with omalizumab-treated versus placebo-treated patients with CSU were included in this analysis. We identified 7 randomized, placebo-controlled studies with 1312 patients with CSU. Patients treated with omalizumab (75-600 mg every 4 weeks) had significantly reduced weekly itch and weekly wheal scores compared with the placebo group. Omalizumab's effects were dose dependent, with the strongest reduction in weekly itch and weekly wheal scores observed with 300 mg. Rates of complete response were significantly higher in the omalizumab group (relative risk, 4.55; P omalizumab and placebo groups. This meta-analysis provides high-quality evidence for the efficacy and safety of omalizumab in patients with CSU and for treating these patients with 300 mg of omalizumab every 4 weeks. Copyright © 2016 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  19. Latex allergy: an emerging

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    Fernando Samuel Sion

    2013-11-01

    Full Text Available RESUMO: A alergia ao látex é manifestação de hipersensibilidade imediata mediada por IgE à proteína do látex natural, com variedade de sinais clínicos como urticária de contato, dermatite de contato por irritação primária ou por mecanismo de hipersensibilidade tardia, angioedema, asma, até anafilaxia. Com maior risco para a alergia ao látex estão os trabalhadores da área de saúde e demais profissões que usam látex. Pacientes com hipersensibilidade ao látex podem apresentar reação cruzada a frutas / alimentos, especialmente banana, kiwi e abacate. O diagnóstico é feito por detecção de anticorpos IgE específicos, testes cutâneos de puntura e testes de contato. Prevenção à exposição, substituição e utilização de produtos livres de látex, como luvas sintéticas são essenciais para os afetados. Adaptações no local de trabalho devem ser feitas com uso de luvas sem talco, com baixo teor de alérgenos ou luvas sintéticas. Estas medidas preventivas reduziram significativamente a prevalência de reações. Descritores: Alergia ao látex, Alimentos e alergia ao látex, Prevenção a reação ao látex.

  20. [Food allergies in paediatrics: Current concepts].

    Science.gov (United States)

    Plaza-Martin, Ana María

    2016-07-01

    The concept of allergic reaction currently includes all those where an immunological reaction depends on a reaction mediated by IgE, as well as those that involve other immune mechanisms, such as T-cell regulators. There are many different clinical situations, like the classic immediate reactions (IgE mediated) such as urticaria, angioedema, immediate vomiting, abdominal pain, both upper respiratory (aphonia or rhinitis) and lower (wheezing or dyspnoea) symptom, and cardiovascular symptoms. The reactions that involve more than one organ, such as anaphylaxis, which could be an anaphylactic shock if there is cardiovascular involvement. The clinical signs and symptoms produced by non-IgE mediated reactions are usually more insidious in how they start, such as vomiting hours after the ingestion of food in enterocolitis, diarrhoea after days or weeks from starting food, dermatitis sometime after starting food. In these cases it is more difficult to associate these clinical symptoms directly with food. In this article, we attempt to clarify some concepts such as sensitisation/allergy, allergen/allergenic source, or the relationship of different clinical situations with food allergy, in order to help the paediatrician on the one hand, to prescribe strict diets in case of a suspicion based on the cause/effect relationship with the food, and on the other hand not to introduce unnecessary diets that very often have to last an excessively long time, and could lead to nutritional deficiencies in the children. Copyright © 2016 Asociación Española de Pediatría. Published by Elsevier España, S.L.U. All rights reserved.

  1. Profile of omalizumab in the treatment of chronic spontaneous urticaria

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    Labrador-Horrillo M

    2015-08-01

    Full Text Available Moises Labrador-Horrillo,1 Marta Ferrer2 1Allergy Section, Internal Medicine Department, Vall d’Hebron Hospital, Universitat Autònoma de Barcelona, Barcelona, 2Department of Allergy and Clinical Immunology, Clínica Universidad de Navarra, IDISNA, Instituto de Investigación de Navarra, Pamplona, Spain Abstract: Chronic spontaneous urticaria (CSU is a disease with significant morbidity and relative prevalence that has important effects on the quality of life (QoL of those who suffer from it. Omalizumab is a recombinant humanized anti-immunoglobulin E (IgE antibody that binds to the Cε3 domain of the IgE heavy chain and prevents it from binding to its high-affinity receptor FcεRI. It has been largely studied in the field of asthma and is currently approved for the treatment of both adult and pediatric (children; >6-year-old patients. In addition, in recent, well-controlled clinical trials in patients with CSU resistant to antihistamines, add-on therapy with subcutaneous omalizumab significantly reduced the severity of itching, and the number and size of hives, and increased patients’ health-related QoL and the proportion of days free from angioedema compared with placebo, with an excellent tolerance. Thus, omalizumab is an effective and well-tolerated add-on therapy for patients with CSU who are symptomatic despite background therapy with H1 antihistamines. In this review, we cover the following points: epidemiology, pathogenesis, assessment of activity, impact on QoL, and treatment of CSU, and finally, we focus on omalizumab in the treatment of CSU including the pharmacokinetic properties and mechanism of action, and use in pregnant women, nursing infants, and children. Keywords: omalizumab, chronic spontaneous urticaria, antihistamines, subcutaneous administration, add-on therapy

  2. Incidence and risk factors of major toxicity associated to first-line antituberculosis drugs for latent and active tuberculosis during a period of 10 years

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    Ana Tavares e Castro

    2015-05-01

    Full Text Available Introduction: Adverse drug reactions (ADR to first-line antituberculosis drugs are frequent and have important implications that may affect the effectiveness of treatment and course of tuberculosis (TB. Material and methods: Retrospective data analysis of clinical records and national registration forms from patients with ADR to first line antituberculosis that occurred between 2004 and 2013 at a Portuguese Pulmonology Diagnostic Centre, and from a case–control population matched by sex, age and year of initiation of treatment. Results: Of the 764 patients treated with antituberculosis drugs, 55 (52.7% male, 92.7% European, mean age 50.8 ± 19.5 years had at least one severe ADR and six had a second ADR, for a total of 61 events. The most frequent ADR were hepatotoxicity (86.9%, rash (8.2% and others, such as ocular toxicity, gastrointestinal intolerance and angioedema (4.9%. Isoniazid, alone or in combination, was the antituberculosis drug most associated to toxicity. Due to ADR, treatment time changed an average of 1.0 ± 2.6 months (range −3.4 to 10.6. There was no correlation between age or gender and the overall incidence of ADR although we found a significant association between younger age and an increased risk of hepatotoxicity (P = 0.035. There was also a statistically significant relationship between ADR and diabetes mellitus (P = 0.042 but not for other comorbidities or multi-resistant TB risk factors. Conclusions: This study found a high frequency of ADR with strong impact on subsequent therapeutic orientation. What seems to be of particular interest is the relationship between ADR and diabetes mellitus and the increased frequency of hepatotoxicity in younger patients. Keywords: Tuberculosis, Adverse reaction, Antituberculosis, Treatment

  3. Methylene blue for clinical anaphylaxis treatment: a case report

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    Janine Moreira Rodrigues

    Full Text Available CONTEXT AND OBJECTIVE: Nitric oxide has a pathophysiological role in modulating systemic changes associated with anaphylaxis. Nitric oxide synthase inhibitors may exacerbate bronchospasm in anaphylaxis and worsen clinical conditions, with limited roles in anaphylactic shock treatment. The aim here was to report an anaphylaxis case (not anaphylactic shock, reversed by methylene blue (MB, a guanylyl cyclase inhibitor. CASE REPORT: A 23-year-old female suddenly presented urticaria and pruritus, initially on her face and arms, then over her whole body. Oral antihistamine was administered initially, but without improvement in symptoms and signs until intravenous methylprednisolone 500 mg. Recurrence occurred after two hours, plus vomiting. Associated upper respiratory distress, pulmonary sibilance, laryngeal stridor and facial angioedema (including erythema and lip edema marked the evolution. At sites with severe pruritus, petechial lesions were observed. The clinical situation worsened, with dyspnea, tachypnea, peroral cyanosis, laryngeal edema with severe expiratory dyspnea and deepening unconsciousness. Conventional treatment was ineffective. Intubation and ventilatory support were then considered, because of severe hypoventilation. But, before doing that, based on our previous experience, 1.5 mg/kg (120 mg bolus of 4% MB was infused, followed by one hour of continuous infusion of another 120 mg diluted in dextrose 5% in water. Following the initial intravenous MB dose, the clinical situation reversed completely in less than 20 minutes, thereby avoiding tracheal intubation. CONCLUSION: Although the nitric oxide hypothesis for MB effectiveness discussed here remains unproven, our intention was to share our accumulated cohort experience, which strongly suggests MB is a lifesaving treatment for anaphylactic shock and/or anaphylaxis and other vasoplegic conditions.

  4. Clinical manifestations of gastrointestinal form of food allergy in children and approaches to its diagnosis

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    Yu.R. Chernysh

    2017-08-01

    Full Text Available Gastrointestinal food allergy is caused by the development of allergic inflammation in the mucosa of the gastrointestinal tract. The mechanisms of this inflammation are immunogflobulin E (IgE-mediated (oral allergic syndrome, immediate gastrointestinal hypersensitivity, non-IgE-mediated (protein-induced enterocolitis syndrome, protein-induced enteropathy, protein-induced allergic proctocolitis and mixed IgE- and non-IgE-mediated reactions (eosinophilic esophagitis, eosinophilic gastritis and eosinophilic gastroenteritis. Gastrointestinal manifestations of food allergy are also combined with symptoms of atopic diseases, more often with atopic dermatitis, urticaria and angioedema. Clinical manifestations of allergic lesions of the gastrointestinal tract are different and non-specific. Common signs of gastrointestinal allergy include: vomiting (occurs from a few minutes to 4–6 hours after eating; сolic (immediately or several hours after eating; constipation; diarrhea; refusal of food (from a specific product or complete refusal to eat; abdominal pain; flatulence, the presence of mucus and eosinophils in the stool; poor appetite; headache. Differential diagnosis of gastrointestinal food allergy should be carried out with diseases such as disease and abnormalities in the development of the digestive system, mental and metabolic disorders, intoxications, infectious diseases, pancreatic endocrine gland failure, celiac disease, cystic fibrosis, immunodeficiencies, disaccharidic insufficiency, side effects of medications, endocrine pathology, irritable bowel syndrome. Methods for diagnosing gastrointestinal allergy, which currently exist, are limited and imperfect. This requires further scientific researches aimed at timely detection of this pathology, prevention in genetically predisposed children, development of optimal diagnostic algorithms, prevention of the progression of clinical manifestations, the choice of individual diet therapy and

  5. Symptoms of pseudoallergy and histamine metabolism disorders

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    Joanna Kacik

    2016-09-01

    Full Text Available Histamine intolerance is a poorly investigated type of hypersensitivity responsible for a number of often serious symptoms, erroneously interpreted as food allergy. Endogenous histamine originates from the histidine amino acid with the help of the histidine decarboxylase enzyme. Apart from the endogenous production histamine may be supplied to the body with food. Slow-maturing and fermenting products are characterised by particularly high levels of histamine. Some food products stimulate excessive release of histamine from stores in the body as well as containing significant amounts of it. These products include spices, herbs, dried fruits and a large group of food additives. Histamine intolerance is considered to be a condition in which the amount of histamine in the body exceeds its tolerance threshold, which leads to the development of adverse reactions. These reactions primarily include skin symptoms (pruritus, urticaria, skin reddening, acne lesions, angioedema, respiratory symptoms (nasal obstruction and watery discharge, sneezing, coughing, wheezing, gastrointestinal symptoms (abdominal cramps, diarrhoea, bloating, nervous system symptoms (headaches, fatigue, irritability, anxiety, panic attacks, cardiovascular symptoms (tachycardia, hypotension, chest pain, primary dysmenorrhoea and many more. It is estimated that nearly 1% of society is susceptible to histamine intolerance. The diagnosis of this disorder is based on observing at least two characteristic symptoms and their disappearance or improvement following histamine-free diet. A new, although not easily accessible diagnostic tool is assay for serum diamine oxidase activity, which correlates to a significant extent with symptoms of histamine intolerance. Normal activity of diamine oxidase is considered to be the amount of >80 HDU/mL, decreased activity – 40–80 HDU/mL and severely decreased activity – <40 HDU/mL. Currently the option of diamine oxidase supplementation is

  6. Fish allergy in childhood.

    Science.gov (United States)

    Pascual, Cristina Y; Reche, Marta; Fiandor, Ana; Valbuena, Teresa; Cuevas, Teresa; Esteban, Manuel Martin

    2008-11-01

    Fish and its derived products play an important role in human nutrition, but they may also be a potent food allergen. Fish can be an ingested, contact, and inhalant allergen. Gad c I, a Parvalbumin, the major allergen in codfish, is considered as fish and amphibian pan-allergen. Prevalence of fish allergy appears to depend on the amount of fish eaten in the local diet. In Europe, the highest consumption occurs in Scandinavian countries, Spain and Portugal. In Spain, fish is the third most frequent allergen in children under 2 yr of age after egg and cow's milk. An adverse reaction to fish may be of non-allergic origin, due to food contamination or newly formed toxic products, but the most frequent type of adverse reactions to fish are immunologic-mediated reactions (allergic reactions). Such allergic reactions may be both IgE-mediated and non-IgE-mediated. Most cases are IgE-mediated, due to ingestion or contact with fish or as a result of inhalation of cooking vapors. Some children develop non-IgE-mediated type allergies such as food protein induced enterocolitis syndrome. The clinical symptoms related to IgE-mediated fish allergy are most frequently acute urticaria and angioedema as well as mild oral symptoms, worsening of atopic dermatitis, respiratory symptoms such as rhinitis or asthma, and gastrointestinal symptoms such as nausea and vomiting. Anaphylaxis may also occur. Among all the species studied, those from the Tunidae and Xiphiidae families appear to be the least allergenic.

  7. Rationale for selecting antihistamine drugs for the therapy of chronic urticaria in terms of efficacy and safety

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    O. V. Skorokhodkina

    2014-01-01

    Full Text Available Goal of the study. To compare the efficacy of antihistamine drugs for the therapy of chronic urticaria taking into consideration their effect on the patients’ cognitive functions. Materials and methods. The study involved 178 patients with chronic urticaria who were divided into six groups taking second generation antihistamine drugs: Cetirizine (n = 38, Levocetirizine (n = 27, Fexofenadine (n=26, Ebastine (n = 33, Loratadine (n = 26 and Desloratadine (n = 28. The patients recorded dynamic changes in clinical symptoms of the disease (number of urticarial components, skin itching intensity, availability or absence of urticarial derniographism, angioedema signs and signs of the shortness of breath and reduced blood pressure in their individual diaries. Baseline signs of the patients’ cognitive condition and those recorded during the treatment were studied using the Kraepelin’s arithmetic test (modified by Schulte, I.M. Lushchikhina’s verbal and visual thinking assessment method and method for memorizing ten words. The control group comprised 31 subjects without chronic urticaria. Results of the study. Ebastine and Fexofenadine are the most efficient antihistamine drugs for the treatment of chronic urticaria. At the same time, they do not have any negative effect on the patients’ cognitive functions so they can be recommended for long-term treatment of chronic urticaria. In spite of its evident positive therapeutic effect, Cetirizine reduces mental alertness and deteriorates thinking in patients with chronic urticaria. Because of this, the drug must be prescribed with care for long-term administration to those patients whose professional activities demand increased attention concentration. Loratadine has a positive effect on the patients’ attention and thinking. However, taking into consideration its low efficacy, the drug can be prescribed as the basis therapy for the treatment of light forms of chronic urticaria.

  8. The Prevalence of Contact Dermatitis Among Occupational and Work-related Diseases. Correlation between Atopy and Allergic or Irritative Contact Dermatitis

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    Codruta-Dana Pitis

    2015-12-01

    Full Text Available The prevalence of contact dermatitis (CD in Europe varies between 6.7% and 10.6% depending on the sector of activity. Professional CD (PCD has an important economic impact − 30% of the budget compensation for occupational disease. In Romania, the prevalence of PCD is underestimated, with an even distribution of cases with respect to the allergic or irritative mechanism. A retrospective clinical study was conducted; target population being the patients admitted in Occupational Medicine Clinic Cluj-Napoca between 2003 and 2011. Objectives of study were: specifying the prevalence range of allergic CD (ACD / irritative CD (ICD among occupational/work-related diseases, the distribution of allergic/irritative CD (A/ICD for different sectors of activity and establishing the correlation between atopy and A/ICD. We have applied allergy skin tests - prick (environmental allergens and patch (occupational allergens. Inclusion criteria were: -documented occupational exposure at skin allergens/irritants; -the atopy state; -diagnostic established at discharge. Patients with recurrent chronic urticaria, angioedema, hypereosinophilic syndrome have been excluded. Statistical analysis was done using SPSS software. The study indicated a similar prevalence for A/ICD, with similar distribution. Prevalence above average has been recorded in the metallurgy sector (A/ICD, in health care sector (ACD, respectively, textile industry (ICD. The correlation atopy-ACD has proved to be lower compared to previous reports. Regarding ICD, the diagnostic was confirmed frequently to non-atopic persons. We strongly recommend the compliance with a multidisciplinary protocol for the management of A/ICD, individualized for specific activity sectors or even work stations.

  9. Post-licensure safety surveillance for human papillomavirus-16/18-AS04-adjuvanted vaccine: more than 4 years of experience.

    Science.gov (United States)

    Angelo, Maria-Genalin; Zima, Julia; Tavares Da Silva, Fernanda; Baril, Laurence; Arellano, Felix

    2014-05-01

    To summarise post-licensure safety surveillance over more than 4 years of routine use of the human papillomavirus-16/18-AS04-adjuvanted vaccine (HPV-16/18 vaccine: Cervarix®, GlaxoSmithKline, Belgium). We describe global post-licensure passive surveillance data based on routine pharmacovigilance from 18 May 2007 until 17 November 2011 and enhanced surveillance implemented during the 2-year national immunisation programme in the UK (school years 2008-2010). Spontaneous reports from countries worldwide showed a similar pattern for the most frequently reported adverse events after HPV-16/18 vaccination. No patterns or trends were observed for potential immune-mediated diseases after vaccination. Observed incidences of Bell's palsy and confirmed Guillain-Barré syndrome were within the expected range in the general population. Outcomes of pregnancy in women who were inadvertently exposed to HPV-16/18 vaccine during pregnancy, were in line with published reports for similar populations. Enhanced surveillance of adverse events in the UK triggered a review of cases of anaphylaxis, angioedema and syncope reports, leading to an update to the prescribing information. Collaborative partnerships between industry and national regulatory agencies facilitated rapid notification and transfer of safety information, allowing for rapid responses in the event of a safety signal of adverse event of concern. More than 4 years of post-licensure experience may provide confidence to providers and the public about the safety profile of HPV-16/18 vaccine in routine use. The safety profile appears to be consistent with pre-licensure data reporting that HPV-16/18 vaccine has an acceptable benefit-risk profile in adolescent girls and women. © 2014 The Authors. Pharmacoepidemiology and Drug Safety published by John Wiley & Sons, Ltd.

  10. IgE-mediated food allergies in Swiss infants and children.

    Science.gov (United States)

    Ferrari, Giovanni Gaspare; Eng, Peter Andreas

    2011-10-12

    To determine the most frequent food allergens causing immediate hypersensitivity reactions in Swiss children of different age groups and to investigate the clinical manifestation of IgE-mediated food allergies in young patients. The study was a prospective analysis of children referred for assessment of immediate type I food hypersensitivity reactions. The diagnostic strategy included a careful history, skin prick tests with commercial extracts and native foods, in vitro determination of specific IgE to food proteins and food challenges when appropriate. A total of 278 food allergies were identified in 151 children with a median age of 1.9 years at diagnosis. Overall, the most frequent food allergens were hen's egg (23.7%), cow's milk (20.1%), peanut (14.0%), hazelnut (10.4%), wheat (6.1%), fish (4.3%), kiwi and soy (2.2% each). In infancy, cow's milk, hen's egg and wheat were the most common allergens. In the second and third year of life however, the top three food allergens were hen's egg, cow's milk and peanut, whereas above the age of 3 years, peanut was number one, followed by hen's egg and fish. Overall, urticaria (59.0%) and angioedema (30.2%) were the most frequent clinical manifestations. Gastrointestinal symptoms were found in 25.9% and respiratory involvement in 25.2%. There were 13 cases (4.7%) of anaphylaxis to peanut, fish, cow's milk, hen's egg, wheat and shrimps. A total of eight allergens account for 83% of IgE-mediated food allergies in Swiss infants and children, with differences in the distribution and order of the most frequently involved food allergens between paediatric age groups.

  11. Chronic urticaria in patients with autoimmune thyroiditis: Significance of severity of thyroid gland inflammation

    Directory of Open Access Journals (Sweden)

    Mustafa Gulec

    2011-01-01

    Full Text Available Background: There is a clear association between autoimmune thyroiditis (AT and chronic urticaria/angioedema (CUA. However, not all patients with AT demonstrate urticaria. Aims: The aim of the study was to investigate in which patients with AT did CUA become a problem. A sensitive inflammation marker, neopterine (NP was used to confirm whether the severity of inflammation in the thyroid gland was responsible for urticaria or not. Methods: Neopterine levels were assessed in patients with AT with urticaria and without urticaria. Furthermore, levels were compared in relation to pre and post levothyroxine treatment. Twenty-seven patients with urticaria (Group 1 and 28 patients without urticaria (Group 2 were enrolled in the study. A course of levothyroxine treatment was given to all patients, and urine neopterine levels before and after the trial were obtained. Results: All patients completed the trial. Mean age in Group 1 and Group 2 was similar (35.70 ± 10.86 years and 38.36 ± 10.38 years, respectively (P=0.358. Pre-treatment urine neopterine levels were significantly higher in Group 1 (P=0.012. Post-treatment levels decreased in each group, as expected. However, the decrease in the neopterine level was insignificant in the patients of Group 2 (P=0.282. In Group 1, a significant decrease in post-treatment neopterine levels (P=0.015 was associated with the remission of urticaria. Conclusion: In patients with CUA and AT, pre-treatment elevated levels of NP, and its decrease with levothyroxine treatment along with symptomatic relief in urticaria, may be evidence of the relationship between the degree of inflammation in thyroid and presence of urticaria.

  12. Clinical approach on challenge and desensitization procedures with aspirin in patients with ischemic heart disease and nonsteroidal anti-inflammatory drug hypersensitivity.

    Science.gov (United States)

    Cortellini, G; Romano, A; Santucci, A; Barbaud, A; Bavbek, S; Bignardi, D; Blanca, M; Bonadonna, P; Costantino, M T; Laguna, J J; Lombardo, C; Losappio, L M; Makowska, J; Nakonechna, A; Quercia, O; Pastorello, E A; Patella, V; Terreehorst, I; Testi, S; Cernadas, J R; Dionicio Elera, J; Lippolis, D; Voltolini, S; Grosseto, D

    2017-03-01

    Hypersensitivity to acetylsalicylic acid (ASA) constitutes a serious problem for subjects with coronary artery disease. In such subjects, physicians have to choose the more appropriate procedure between challenge and desensitization. As the literature on this issue is sparse, this study aimed to establish in these subjects clinical criteria for eligibility for an ASA challenge and/or desensitization. Collection and analysis of data on ASA challenges and desensitizations from 10 allergy centers, as well as consensus among the related physicians and an expert panel. Altogether, 310 subjects were assessed; 217 had histories of urticaria/angioedema, 50 of anaphylaxis, 26 of nonimmediate cutaneous eruptions, and 17 of bronchospasm related to ASA/nonsteroidal anti-inflammatory drugs (NSAID) intake. Specifically, 119 subjects had index reactions to ASA doses lower than 300 mg. Of the 310 subjects, 138 had an acute coronary syndrome (ACS), 101 of whom underwent desensitizations, whereas 172 suffered from a chronic ischemic heart disease (CIHD), 126 of whom underwent challenges. Overall, 163 subjects underwent challenges and 147 subjects underwent desensitizations; 86 of the latter had index reactions to ASA doses of 300 mg or less. Ten subjects reacted to challenges, seven at doses up to 500 mg, three at a cumulative dose of 110 mg. The desensitization failure rate was 1.4%. In patients with stable CIHD and histories of nonsevere hypersensitivity reactions to ASA/NSAIDs, an ASA challenge is advisable. Patients with an ACS and histories of hypersensitivity reactions to ASA, especially following doses lower than 100 mg, should directly undergo desensitization. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  13. Evaluation of autologous serum skin test and skin prick test reactivity to house dust mite in patients with chronic spontaneous urticaria.

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    Zhiqiang Song

    Full Text Available BACKGROUND: Chronic spontaneous urticaria (CSU is a common skin disorder with etiology that is not well understood. METHODS: In this study, we evaluated the prevalence of autologous serum skin test (ASST and skin prick testing (SPT to house dust mite (HDM in 862 CSU cases in China. Clinical features, courses and treatment responses were also recorded. RESULTS: The prevalence of positive ASST was 46.3%, and patients aged 30-39 years had the highest positive rate (52.1%. Positive SPT to HDM was seen in 153 patients (17.7% with the highest positive rate (34.2% in patients aged 20 or less. Patients with positive ASST had higher urticaria activity scores (UAS (4.18±0.65 vs. 3.67±0.53 but lower positive rates of HDM (24.6% vs. 37.6%, as compared with those with negative ASST (odds ratio (OR 1.84, 95% CI 1.38-2.47. Patients could be categorized into four groups based on the results of ASST and SPT to HDM and patients with positive ASST and positive SPT to HDM had the highest disease activity scores, experienced higher frequencies of angioedema, diseases duration, and required higher dosage of loratadine every month, compared with other subgroups (P<0.0001. CONCLUSIONS: Patients with CSU showed varied responses of positive ASST and varied sensitivity to HDM, Patients with positive ASST and/or positive SPT had more disease activity compared with patients with negative ASST and/or negative SPT. Further classification can be made based on the result of SPT and ASST.

  14. Anaphylactic Reactions After Discontinuation of Hymenoptera Venom Immunotherapy: A Clonal Mast Cell Disorder Should Be Suspected.

    Science.gov (United States)

    Bonadonna, Patrizia; Zanotti, Roberta; Pagani, Mauro; Bonifacio, Massimiliano; Scaffidi, Luigi; Olivieri, Elisa; Franchini, Maurizio; Reccardini, Federico; Costantino, Maria Teresa; Roncallo, Chiara; Mauro, Marina; Boni, Elisa; Rizzini, Fabio Lodi; Bilò, Maria Beatrice; Marcarelli, Anna Rosaria; Passalacqua, Giovanni

    2017-12-16

    Up to 75% of patients with severe anaphylactic reactions after Hymenoptera sting are at risk of further severe reactions if re-stung. Venom immunotherapy (VIT) is highly effective in protecting individuals with ascertained Hymenoptera venom allergy (HVA) and previous severe reactions. After a 3- to 5-year VIT course, most patients remain protected after VIT discontinuation. Otherwise, a lifelong treatment should be considered in high-risk patients (eg, in mastocytosis). Several case reports evidenced that patients with mastocytosis and HVA, although protected during VIT, can re-experience severe and sometimes fatal reactions after VIT discontinuation. To evaluate whether patients who lost protection after VIT discontinuation may suffer from clonal mast cell disorders. The survey describes the characteristics of patients who received a full course of VIT for previous severe reactions and who experienced another severe reaction at re-sting after VIT discontinuation. Those with a Red Española de Mastocitosis score of 2 or more or a serum basal tryptase level of more than 25 ng/mL underwent a hematological workup (bone marrow biopsy, KIT mutation, expression of aberrant CD25) and/or skin biopsy. Nineteen patients (mean age, 56.3 years; 89.5% males) were evaluated. All of them had received at least 4 years of VIT and were protected. After VIT discontinuation they were re-stung and developed, in all but 1 case, severe anaphylactic reactions (12 with loss of consciousness, in the absence of urticaria/angioedema). Eighteen patients (94.7%) had a clonal mast cell disorder, 8 of them with normal tryptase. Looking at this selected population, we suggest that mastocytosis should be considered in patients developing severe reactions at re-sting after VIT discontinuation and, as a speculation, patients with mastocytosis and HVA should be VIT-treated lifelong. Copyright © 2017. Published by Elsevier Inc.

  15. Adverse drug reaction reports for cardiometabolic drugs from sub-Saharan Africa: a study in VigiBase.

    Science.gov (United States)

    Berhe, Derbew Fikadu; Juhlin, Kristina; Star, Kristina; Beyene, Kidanemariam G M; Dheda, Mukesh; Haaijer-Ruskamp, Flora M; Taxis, Katja; Mol, Peter G M

    2015-06-01

    Identifying key features in individual case safety reports (ICSR) of suspected adverse drug reactions (ADRs) with cardiometabolic drugs from sub-Saharan Africa (SSA) compared with reports from the rest of the world (RoW). Reports on suspected ADRs of cardiometabolic drugs (ATC: A10[antidiabetic], B01[antithrombotics] and C[cardiovascular]) were extracted from WHO Global database, VigiBase(®) (1992-2013). We used vigiPoint, a logarithmic odds ratios (log2 OR)-based method to study disproportional reporting between SSA and RoW. Case-defining features were considered relevant if the lower limit of the 99% CI > 0.5. In SSA, 3773 (9%) of reported ADRs were for cardiometabolic drugs, in RoW for 18%. Of these, 79% originated from South Africa and 81% were received after 2007. Most reports were for drugs acting on the renin-angiotensin system (36% SSA & 14% RoW). Compared with RoW, reports were more often sent for patients 18-44 years old (log2 OR 0.95 [99 CI 0.80; 1.09]) or with non-fatal outcome (log2 OR 1.16 [99 CI 1.10; 1.22]). Eight ADRs (cough, angioedema, lip swelling, face oedema, swollen tongue, throat irritation, drug ineffective and blood glucose abnormal) and seven drugs (enalapril, rosuvastatin, perindopril, vildagliptin, insulin glulisine, nifedipine and insulin lispro) were disproportionally more reported in SSA than in the RoW. 'In recent years, the number of adverse drug reactions (ADRs) reported in Sub-Saharan Africa (SSA) has sharply increased. The data showed the well-known population-based differential ADR profile of ACE inhibitors in the SSA population.' © 2015 John Wiley & Sons Ltd.

  16. AUTOIMMUNE CYTOPENIAS IN CHRONIC LYMPHOCYTIC LEUKEMIA, FACTS AND MYTHS

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    Pavankumar Tandra

    2013-11-01

    Full Text Available CLL has been defined as presence of more than 5000 small mature appearing monoclonal B lymphocytes with a specific immunophenotype in peripheral blood. It is a well-known fact that CLL is associated with autoimmune cytopenias. CLL cells are CD5+ B lymphocytes, and usually are not the “guilty” cells which produce autoantibodies. T cell defect is another characteristic of CLL and the total number of T cells is increased, and there is inversion of the CD4/CD8 ratio. Autoimmune hemolytic anemia (AIHA is the most common autoimmune complication of CLL and has been reported in 10-25% of CLL patients. However, the stage-adjusted estimated rate of AIHA in CLL is about 5%. Conversely, CLL is three times more common in patients who present with AIHA. Direct agglutinin test (DAT is positive in 7-14% of CLL patients but AIHA may also occur in DAT negative patients. Autoimmune thrombocytopenia (AIT is the second most common complication of CLL and has been reported in 2-3% of patients. DAT is positive in AIT but presence of antiplatelet antibodies is neither diagnostic nor reliable. Autoimmune neutropenia (AIN and pure red cell aplasia (PRCA are very rare complications of CLL and like other autoimmune complications of CLL may occur at any clinical stage. It is believed that most case reports of AIN and PRCA in CLL actually belong to large granular lymphocytic leukemia (LGL. Non-hematologic autoimmune complications of CLL including cold agglutinin disease (CAD, paraneoplastic pemphigus (PNP, acquired angioedema, and anti-myelin associated globulin are rare. Before starting any treatment, clinicians should distinguish between autoimmune cytopenias and massive bone marrow infiltration since autoimmune complications of CLL are not necessarily equal to advanced disease with poor prognosis. According to IWCLL guideline, steroids are the mainstay of treatment of simple autoimmunity. Intravenous immunoglobulin (IVIg, cyclosporine, and rituximab are used in

  17. Characterization of hypersensitivity reactions reported among Andrographis paniculata users in Thailand using Health Product Vigilance Center (HPVC) database.

    Science.gov (United States)

    Suwankesawong, Wimon; Saokaew, Surasak; Permsuwan, Unchalee; Chaiyakunapruk, Nathorn

    2014-12-24

    reaction (n = 4) and angioedema (n = 4). Time to development of symptoms ranged from 5 minutes to 1 day. The doses of andrographis used varied from 352 mg to 1,750 mg. Causality assessment of 13 case reports were certain (n = 3), probable (n = 8) and possible (n = 2). Our findings suggested that hypersensitivity reactions have been reported among patients receiving Andrographis paniculata. Healthcare professionals should be aware of this potential risk. Further investigation of the causal relationship is needed; meanwhile including hypersensitivity reactions for andrographis product labeling should be considered.

  18. The F4/AS01B HIV-1 Vaccine Candidate Is Safe and Immunogenic, But Does Not Show Viral Efficacy in Antiretroviral Therapy-Naive, HIV-1-Infected Adults

    Science.gov (United States)

    Dinges, Warren; Girard, Pierre-Marie; Podzamczer, Daniel; Brockmeyer, Norbert H.; García, Felipe.; Harrer, Thomas; Lelievre, Jean-Daniel; Frank, Ian; Colin De Verdière, Nathalie; Yeni, Guy-Patrick; Ortega Gonzalez, Enrique; Rubio, Rafael; Clotet Sala, Bonaventura; DeJesus, Edwin; Pérez-Elias, Maria Jesus; Launay, Odile; Pialoux, Gilles; Slim, Jihad; Weiss, Laurence; Bouchaud, Olivier; Felizarta, Franco; Meurer, Anja; Raffi, François; Esser, Stefan; Katlama, Christine; Koletar, Susan L.; Mounzer, Karam; Swindells, Susan; Baxter, John D.; Schneider, Stefan; Chas, Julie; Molina, Jean-Michel; Koutsoukos, Marguerite; Collard, Alix; Bourguignon, Patricia; Roman, François

    2016-01-01

    Abstract The impact of the investigational human immunodeficiency virus type 1 (HIV-1) F4/AS01B vaccine on HIV-1 viral load (VL) was evaluated in antiretroviral therapy (ART)-naive HIV-1 infected adults. This phase IIb, observer-blind study (NCT01218113), included ART-naive HIV-1 infected adults aged 18 to 55 years. Participants were randomized to receive 2 (F4/AS01B_2 group, N = 64) or 3 (F4/AS01B_3 group, N = 62) doses of F4/AS01B or placebo (control group, N = 64) at weeks 0, 4, and 28. Efficacy (HIV-1 VL, CD4+ T-cell count, ART initiation, and HIV-related clinical events), safety, and immunogenicity (antibody and T-cell responses) were evaluated during 48 weeks. At week 48, based on a mixed model, no statistically significant difference in HIV-1 VL change from baseline was demonstrated between F4/AS01B_2 and control group (0.073 log10 copies/mL [97.5% confidence interval (CI): −0.088; 0.235]), or F4/AS01B_3 and control group (−0.096 log10 copies/mL [97.5% CI: −0.257; 0.065]). No differences between groups were observed in HIV-1 VL change, CD4+ T-cell count, ART initiation, or HIV-related clinical events at intermediate timepoints. Among F4/AS01B recipients, the most frequent solicited symptoms were pain at injection site (252/300 doses), fatigue (137/300 doses), myalgia (105/300 doses), and headache (90/300 doses). Twelve serious adverse events were reported in 6 participants; 1 was considered vaccine-related (F4/AS01B_2 group: angioedema). F4/AS01B induced polyfunctional F4-specific CD4+ T-cells, but had no significant impact on F4-specific CD8+ T-cell and anti-F4 antibody levels. F4/AS01B had a clinically acceptable safety profile, induced F4-specific CD4+ T-cell responses, but did not reduce HIV-1 VL, impact CD4+ T-cells count, delay ART initiation, or prevent HIV-1 related clinical events. PMID:26871794

  19. The F4/AS01B HIV-1 Vaccine Candidate Is Safe and Immunogenic, But Does Not Show Viral Efficacy in Antiretroviral Therapy-Naive, HIV-1-Infected Adults: A Randomized Controlled Trial.

    Science.gov (United States)

    Dinges, Warren; Girard, Pierre-Marie; Podzamczer, Daniel; Brockmeyer, Norbert H; García, Felipe; Harrer, Thomas; Lelievre, Jean-Daniel; Frank, Ian; Colin De Verdière, Nathalie; Yeni, Guy-Patrick; Ortega Gonzalez, Enrique; Rubio, Rafael; Clotet Sala, Bonaventura; DeJesus, Edwin; Pérez-Elias, Maria Jesus; Launay, Odile; Pialoux, Gilles; Slim, Jihad; Weiss, Laurence; Bouchaud, Olivier; Felizarta, Franco; Meurer, Anja; Raffi, François; Esser, Stefan; Katlama, Christine; Koletar, Susan L; Mounzer, Karam; Swindells, Susan; Baxter, John D; Schneider, Stefan; Chas, Julie; Molina, Jean-Michel; Koutsoukos, Marguerite; Collard, Alix; Bourguignon, Patricia; Roman, François

    2016-02-01

    The impact of the investigational human immunodeficiency virus type 1 (HIV-1) F4/AS01B vaccine on HIV-1 viral load (VL) was evaluated in antiretroviral therapy (ART)-naive HIV-1 infected adults.This phase IIb, observer-blind study (NCT01218113), included ART-naive HIV-1 infected adults aged 18 to 55 years. Participants were randomized to receive 2 (F4/AS01B_2 group, N = 64) or 3 (F4/AS01B_3 group, N = 62) doses of F4/AS01B or placebo (control group, N = 64) at weeks 0, 4, and 28. Efficacy (HIV-1 VL, CD4 T-cell count, ART initiation, and HIV-related clinical events), safety, and immunogenicity (antibody and T-cell responses) were evaluated during 48 weeks.At week 48, based on a mixed model, no statistically significant difference in HIV-1 VL change from baseline was demonstrated between F4/AS01B_2 and control group (0.073 log10 copies/mL [97.5% confidence interval (CI): -0.088; 0.235]), or F4/AS01B_3 and control group (-0.096 log10 copies/mL [97.5% CI: -0.257; 0.065]). No differences between groups were observed in HIV-1 VL change, CD4 T-cell count, ART initiation, or HIV-related clinical events at intermediate timepoints. Among F4/AS01B recipients, the most frequent solicited symptoms were pain at injection site (252/300 doses), fatigue (137/300 doses), myalgia (105/300 doses), and headache (90/300 doses). Twelve serious adverse events were reported in 6 participants; 1 was considered vaccine-related (F4/AS01B_2 group: angioedema). F4/AS01B induced polyfunctional F4-specific CD4 T-cells, but had no significant impact on F4-specific CD8 T-cell and anti-F4 antibody levels.F4/AS01B had a clinically acceptable safety profile, induced F4-specific CD4 T-cell responses, but did not reduce HIV-1 VL, impact CD4 T-cells count, delay ART initiation, or prevent HIV-1 related clinical events.

  20. Initiating sacubitril/valsartan (LCZ696) in heart failure: results of TITRATION, a double-blind, randomized comparison of two uptitration regimens.

    Science.gov (United States)

    Senni, Michele; McMurray, John J V; Wachter, Rolf; McIntyre, Hugh F; Reyes, Antonio; Majercak, Ivan; Andreka, Peter; Shehova-Yankova, Nina; Anand, Inder; Yilmaz, Mehmet B; Gogia, Harinder; Martinez-Selles, Manuel; Fischer, Steffen; Zilahi, Zsolt; Cosmi, Franco; Gelev, Valeri; Galve, Enrique; Gómez-Doblas, Juanjo J; Nociar, Jan; Radomska, Maria; Sokolova, Beata; Volterrani, Maurizio; Sarkar, Arnab; Reimund, Bernard; Chen, Fabian; Charney, Alan

    2016-09-01

    To assess the tolerability of initiating/uptitrating sacubitril/valsartan (LCZ696) from 50 to 200 mg twice daily (target dose) over 3 and 6 weeks in heart failure (HF) patients (ejection fraction ≤35%). A 5-day open-label run-in (sacubitril/valsartan 50 mg twice daily) preceded an 11-week, double-blind, randomization period [100 mg twice daily for 2 weeks followed by 200 mg twice daily ('condensed' regimen) vs. 50 mg twice daily for 2 weeks, 100 mg twice daily for 3 weeks, followed by 200 mg twice daily ('conservative' regimen)]. Patients were stratified by pre-study dose of angiotensin-converting enzyme inhibitor/angiotensin-receptor blocker (ACEI/ARB; low-dose stratum included ACEI/ARB-naïve patients). Of 540 patients entering run-in, 498 (92%) were randomized and 429 (86.1% of randomized) completed the study. Pre-defined tolerability criteria were hypotension, renal dysfunction and hyperkalaemia; and adjudicated angioedema, which occurred in ('condensed' vs. 'conservative') 9.7% vs. 8.4% (P = 0.570), 7.3% vs. 7.6% (P = 0.990), 7.7% vs. 4.4% (P = 0.114), and 0.0% vs. 0.8% of patients, respectively. Corresponding proportions for pre-defined systolic blood pressure 5.5 mmol/L, and serum creatinine >3.0 mg/dL were 8.9% vs. 5.2% (P = 0.102), 7.3% vs. 4.0% (P = 0.097), and 0.4% vs. 0%, respectively. In total, 378 (76%) patients achieved and maintained sacubitril/valsartan 200 mg twice daily without dose interruption/down-titration over 12 weeks (77.8% vs. 84.3% for 'condensed' vs. 'conservative'; P = 0.078). Rates by ACEI/ARB pre-study dose stratification were 82.6% vs. 83.8% (P = 0.783) for high-dose/'condensed' vs. high-dose/'conservative' and 84.9% vs. 73.6% (P = 0.030) for low-dose/'conservative' vs. low-dose/'condensed'. Initiation/uptitration of sacubitril/valsartan from 50 to 200 mg twice daily over 3 or 6 weeks had a tolerability profile in line with other HF treatments. More gradual initiation/uptitration maximized attainment of target dose in the low

  1. Insect venom hypersensitivity: experience in a clinical immunology/allergy service in Singapore.

    Science.gov (United States)

    Thong, B Y H; Leong, K P; Chng, H H

    2005-10-01

    To study the profile of patients with allergy to the venom of insect stings. 31 consecutive cases referred to our clinical immunology/allergy outpatient service from June 1, 1998 to June 30, 2002 were reviewed. These patients comprised 3.5 percent of 889 cases referred during the study period. Their mean age was 28.8 +/- 10.5 (range 19-57) years and the majority were males (90.3 percent). Of these, 20 (64.5 percent) were Chinese, four (12.9 percent) were Malays and seven (22.6 percent) were of other races. 19 patients (61.3 percent) were men from the uniformed services including 12 (63.2 percent) full-time National Servicemen. 71 percent (22 patients) were stung for the first time. Urticaria (22 cases, 71.0 percent), dyspnoea (13, 41.9 percent), angioedema (12, 38.7 percent) and syncope (ten, 32.3 percent) were the most common manifestations of insect allergy. Anaphylaxis occurred in 22 (71.0 percent) cases, constituting 30.1 percent of all cases of anaphylaxis referred to our service during the study period. Although the causative insect was identified as honeybee (12, 38.7 percent), ant (four, 12.9 percent), wasp (three, 9.7 percent), and fire ant (two, 6.5 percent) by the majority of patients, ten (32.2 percent) patients were unable to identify the causative insect. The two patients stung by fire ants were Americans working in Singapore who had been stung while in the United States. Among those with anaphylaxis, honeybee, wasp and fire ant venom, for which specific immunotherapy is available, were identified as the cause in 40.9 percent, 4.5 percent, and 4.5 percent, respectively. Insect venom hypersensitivity made up 3.5 percent of allergy/immunology referrals and 32.8 percent of cases of anaphylaxis referred to our institution. The majority were military servicemen who developed allergic reactions during the course of duty. The inability to identify the causative insect in 50 percent with sting anaphylaxis limits the role of specific immunotherapy in our

  2. Adverse reactions associated with acetylcysteine.

    Science.gov (United States)

    Sandilands, E A; Bateman, D N

    2009-02-01

    Paracetamol (acetaminophen) is one of the most common agents deliberately ingested in self-poisoning episodes and a leading cause of acute liver failure in the western world. Acetylcysteine is widely acknowledged as the antidote of choice for paracetamol poisoning, but its use is not without risk. Adverse reactions, often leading to treatment delay, are frequently associated with both intravenous and oral acetylcysteine and are a common source of concern among treating physicians. A systematic literature review investigating the incidence, clinical features, and mechanisms of adverse effects associated with acetylcysteine. A variety of adverse reactions to acetylcysteine have been described ranging from nausea to death, most of the latter due to incorrect dosing. The pattern of reactions differs with oral and intravenous dosing, but reported frequency is at least as high with oral as intravenous. The reactions to the intravenous preparation result in similar clinical features to true anaphylaxis, including rash, pruritus, angioedema, bronchospasm, and rarely hypotension, but are caused by nonimmunological mechanisms. The precise nature of this reaction remains unclear. Histamine now seems to be an important mediator of the response, and there is evidence of variability in patient susceptibility, with females, and those with a history of asthma or atopy are particularly susceptible. Quantity of paracetamol ingestion, measured through serum paracetamol concentration, is also important as higher paracetamol concentrations protect patients against anaphylactoid effects. Most anaphylactoid reactions occur at the start of acetylcysteine treatment when concentrations are highest. Acetylcysteine also affects clotting factor activity, and this affects the interpretation of minor disturbances in the International Normalized Ratio in the context of paracetamol overdose. This review discusses the incidence, clinical features, underlying pathophysiological mechanisms, and

  3. Update on the bird-egg syndrome and genuine poultry meat allergy.

    Science.gov (United States)

    Hemmer, Wolfgang; Klug, Christoph; Swoboda, Ines

    Allergy to poultry meat is rare and affects both children and adults. The prevalence of poultry meat allergy is unknown, but presumably is similar to that of red meat allergy. There is no close relationship between allergy to poultry meat and allergy to red meat. Poultry meat allergy may present as primary (genuine) food allergy or as secondary food allergy resulting from cross-reactivity. Secondary poultry meat allergy may arise in the context of bird-egg-syndrome, which is due to sensitization to serum albumins present in many tissues including muscle tissue and egg yolk (Gal d 5). Primary sensitization to serum albumin may happen via the respiratory tract through exposure to pet birds (mainly in adults) or within the context of egg allergy in early childhood. Due to the heat lability of serum albumins, reactions are often limited to the skin upon contact with raw meat. Symptoms from meat ingestion are rare and mostly mild, whereas systemic reactions are common after ingestion of raw or soft-boiled egg yolk. Primary poultry meat allergy is mainly seen in adolescents and young adults, though hypersensitivity may have started already at (pre)school age. Egg allergy is usually absent. Typical symptoms of primary poultry meat allergy include OAS (±dyspnea), gastrointestinal complaints, urticaria and angioedema. Severe anaphylaxis with cardiovascular symptoms is rare. Chicken and turkey meat are highly cross-reactive and responsible for most reactions, while duck and goose meat causes milder or no symptoms. Soups, sausages, and ham represent relevant allergen sources, too. Patients with poultry meat allergy unexpectedly often suffer from concomitant allergy to fish and possibly shrimp. Serum specific IgE against fish and shrimp is found in respectively 60 and 40 % of sera, suggestive of cross-reactive allergens in these foods. The allergens thus far recognized in genuine poultry meat are LMW proteins of 5-25 kDa. One of them has been identified as

  4. Cutaneous adverse drug reactions seen in a tertiary hospital in Johor, Malaysia.

    Science.gov (United States)

    Ding, Wen Yi; Lee, Chew Kek; Choon, Siew Eng

    2010-07-01

    Adverse drug reactions are most commonly cutaneous in nature. Patterns of cutaneous adverse drug reactions (ADRs) and their causative drugs vary among the different populations previously studied. Our aim is to determine the clinical pattern of drug eruptions and the common drugs implicated, particularly in severe cutaneous ADRs in our population. This study was done by analyzing the database established for all adverse cutaneous drug reactions seen from January 2001 until December 2008. A total of 281 cutaneous ADRs were seen in 280 patients. The most common reaction pattern was maculopapular eruption (111 cases, 39.5%) followed by Stevens-Johnson Syndrome (SJS: 79 cases, 28.1%), drug reaction with eosinophilia and systemic symptoms (DRESS: 19 cases, 6.8%), toxic epidermal necrolysis (TEN: 16 cases, 5.7 %), urticaria/angioedema (15 cases, 5.3%) and fixed drug eruptions (15 cases, 5.3%). Antibiotics (38.8%) and anticonvulsants (23.8%) accounted for 62.6% of the 281 cutaneous ADRs seen. Allopurinol was implicated in 39 (13.9%), carbamazepine in 29 (10.3%), phenytoin in 27 (9.6%) and cotrimoxazole in 26 (9.3%) cases. Carbamazepine, allopurinol and cotrimoxazole were the three main causative drugs of SJS/TEN accounting for 24.0%, 18.8% and 12.5% respectively of the 96 cases seen whereas DRESS was mainly caused by allopurinol (10 cases, 52.6%) and phenytoin (3 cases, 15.8%). The reaction patterns and drugs causing cutaneous ADRs in our population are similar to those seen in other countries although we have a much higher proportion of severe cutaneous ADRs probably due to referral bias, different prescribing habit and a higher prevalence of HLA-B*1502 and HLA-B*5801 which are genetic markers for carbamazepine-induced SJS/TEN and allopurinol-induced SJS/TEN/DRESS respectively. The most common reaction pattern seen in our study population was maculopapular eruptions. Antibiotics, anticonvulsants and NSAIDs were the most frequently implicated drug groups. Carbamazepine

  5. Chronic idiopathic urticaria and post-traumatic stress disorder (PTSD): an under-recognized comorbidity.

    Science.gov (United States)

    Gupta, Madhulika A; Gupta, Aditya K

    2012-01-01

    A large body of literature supports the role of psychologic stress in urticaria; however, the comorbidity between chronic idiopathic urticaria (CIU) and post-traumatic stress disorder (PTSD), a classic stress-mediated syndrome, has received little attention. The underlying etiology of urticaria is not identifiable in about 70% of patients, possibly because of difficulties with identification of a direct cause-and-effect relationship between a potential causative factor and the onset of urticaria. The core features of PTSD (Diagnostic and Statistical Manual of Mental Disorders, 4th Edition, Text Revision [DSMIV-TR]) that are important in urticaria include (1) autonomic nervous system reactivity and state of sympathetic hyperarousal that can manifest as CIU, and (2) the persistent re-experiencing of the traumatic events in PTSD, which can manifest as urticaria or angioedema, or both, affecting a previously traumatized body region (eg, urticarial wheals affecting the body region where the patient had been stabbed years earlier). The following features of PTSD make it difficult to use the cause-and-effect model for the determination of causation: (1) PTSD may first emerge years after the initial trauma and is classified as PTSD with Delayed Onset (DSMIV-TR); and (2) the traumatic triggers that precipitate the PTSD symptoms may be unique and idiosyncratic to the patient and not even qualify as stressful or traumatic by standard criteria (eg, precipitating events for the PTSD may include smell of a certain cologne that was used by the perpetrator or witnessing a scene in a movie that was reminiscent of the location where the abuse occurred). Finally, in PTSD with Delayed Onset, patients may not make a conscious association between their recurrent urticaria and their earlier traumas because they can develop classically conditioned associations between stimuli that are reminiscent of the original abuse situation and their somatic reactions such as urticaria. The clinician

  6. Positive serum specific IgE has a short half-life in patients with penicillin allergy and reversal does not always indicate tolerance.

    Science.gov (United States)

    Hjortlund, Janni; Mortz, Charlotte Gotthard; Stage, Tore Bjerregaard; Skov, Per Stahl; Dahl, Ronald; Bindslev-Jensen, Carsten

    2014-01-01

    The positive and negative predictive values of specific IgE to penicillins are not well established for penicillin hypersensitivity. One reason may be that serum IgE levels to penicillin diminish over time. The objective in this study was to investigate variations in serum half-life (T½) for specific IgE to penicillins (s-IgE) and to evaluate the outcome of penicillin challenges in patients with previous but not present specific IgE to penicillins. Two subgroups were investigated. All included patients had a history of penicillin allergy with reported symptoms such as urticaria/angioedema or unclassified cutaneous rash. T½ of specific IgE to penicillins was calculated based on sera from 29 patients with repeated measurements of s-IgE. Twenty-two patients with a previous positive s-IgE was followed and challenged with penicillin when IgE had become negative. The T½ for s-IgE varied between the 26 patients with decreasing s-IgE from 1.6 months to 76.4 months and 52% had a T½ of less than a year. The three patients with stable and increasing IgE-values showed T½ approaching infinity A total of 29 challenges with β-lactams were performed. Four different patterns were seen when evaluating the clinical reaction to challenge (positive/negative) and post-challenge boost of s-IgE (yes/no). Eight (36.4%) had negative challenge and negative post-challenge s-IgE, eight (36.4%) negative challenge, but positive post-challenge s-IgE levels. 3 (13.6%) had positive challenge and positive post-challenge s-IgE whereas 3 (13.6%) were challenge positive, but had negative post-challenge s-IgE. Specific IgE to penicillins declines over time stressing the importance of a close time relation between diagnostic work-up and clinical reaction. Reversal of previously positive s-IgE may still be associated with positive penicillin challenges and/or re-boostering of s-IgE to positivity.

  7. Intravenous recombinant tissue plasminogen activator for acute ischemic stroke: a feasibility and safety study

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    Sadeghi-Hokmabadi E

    2016-10-01

    Full Text Available Elyar Sadeghi-Hokmabadi, Mehdi Farhoudi, Aliakbar Taheraghdam, Mazyar Hashemilar, Daryous Savadi-Osguei, Reza Rikhtegar, Kaveh Mehrvar, Ehsan Sharifipour, Parisa Youhanaee, Reshad Mirnour Neurosciences Research Center, Neurology Department, Tabriz University of Medical Sciences, Tabriz, East Azerbaijan, Iran Background: In developing countries, intravenous thrombolysis (IVT is available at a limited number of centers. This study aimed to assess the feasibility and safety of IVT at Tabriz Imam Reza Hospital. Methods: In a prospective study, over a 55-month period, any patient at the hospital for whom stroke code had been activated was enrolled in the study. Data on demographic characteristics, stroke risk factors, admission blood pressure, blood tests, findings of brain computed tomography (CT scans, time of symtom onset, time of arrival to the emergency department, time of stroke code activation, time of CT scan examination, and the time of recombinant tissue plasminogen activator administration were recorded. National Institutes of Health Stroke Scale assessments were performed before IVT bolus, at 36 hours, at either 7 days or discharge (which ever one was earlier, and at 3-month follow-up. Brain CT scans were done for all patients before and 24 hours after the treatment. Results: Stroke code was activated for 407 patients and IVT was done in 168 patients. The rate of functional independence (modified Rankin Scale [mRS] 0–1 at 3 months was 39.2% (62/158. The mortality rate at day 7 was 6% (10/168. Hemorrhagic transformation was noted in 16 patients (9.5%. Symptomatic intracranial hemorrhage occurred in 5 (3%, all of which were fatal. One case of severe urinary bleeding and one other fatal case of severe angioedema were observed. Conclusion: During the first 4–5 years of administration of IVT in the hospital, it was found to be feasible and safe, but to increase the efficacy, poststroke care should be more organized and a stroke center

  8. Urinary metabolites of histamine and leukotrienes before and after placebo-controlled challenge with ASA and food additives in chronic urticaria patients.

    Science.gov (United States)

    Di Lorenzo, G; Pacor, M L; Vignola, A M; Profita, M; Esposito-Pellitteri, M; Biasi, D; Corrocher, R; Caruso, C

    2002-12-01

    The recovery of mediator metabolites from urine has the potential to provide a rapid, safe, and easily available index of release of mediators. We aimed to determine urinary metabolites of both histamine and leukotrienes (LTs) in patients affected by chronic urticaria (CU). Twenty patients with CU were studied. They were selected on the basis of double-blind placebo-controlled challenge (DBPC) with acetyl salicylic acid (ASA) and food additives. Ten patients (group B) were negative to both challenges. Ten patients (group C) presented urticaria and/or the appearance of angioedema during or 24 h after challenge, with reactions to ASA (five patients) or food additives (five patients). We recruited 15 healthy volunteers as controls (group A). During a second challenge, groups B and C were challenged double-blind with a single dose of ASA, or a specific food additive, or placebo. The healthy group was challenged only with a placebo (talc capsule). Patients in groups B and C were challenged twice: with placebo (as groups B1 and C1) and with ASA (groups B2 and C2) or food additives (C2). Four samples of urine were collected; one during the night before the specific or sham challenge (baseline), and three at 2, 6 and 24 h after the challenge. Urinary methylhistamine (N-MH) and LTE4 were analyzed and normalized for urinary creatinine. For urinary N-MH at baseline, there was a significant difference only between group A and groups B1, B2, C1 and C2 (A vs. B1, P < 0.0001; A vs. B2, P < 0.0001; A vs. C1, P < 0.0001; A vs. C2, P < 0.0001). We detected a significant variation in urinary methylhistamine excretion only in group C2 after 2 h, 6 h and 24 h (P < 0.0001). However, no variations were observed in N-MH excretion rate in the other groups (A, B1, C1) after challenge with placebo, and in B2 after challenge with ASA 20 mg. For urinary LTE4 at baseline no differences were found between the mean values for the different groups. After specific challenge, only C2 patients showed

  9. [Evaluation of safety of haemophilus influenza type b(Hib) conjugate vaccine in postmarketing based on the immunization information management system].

    Science.gov (United States)

    Wang, Zhiguo; Ma, Fubao; Zhang, Jinlin; Yu, Jing; Kang, Guodong; Gao, Jun

    2015-06-01

    To analyze the occurrence feature of adverse events following immunization (AEFI) of Hib conjugate vaccine (HibCV) and to evaluate the safety of HibCV in postmarketing. 2008-2013 HibCV AEFI data were collected through national AEFI information management system, which were downloaded in March 18, 2014.The demographic information and inoculation quantity of HibCV were from Immunization information management system in Jiangsu province. The incidence rate and 95% CI value of AEFI, common vaccine reaction and rare vaccine reaction following immunization of HibCV were calculated. The differences in the incidence rate of common vaccine reaction and rare vaccine reaction among sex, months of age, and number of injections were compared by means of (χ² tests. A total of 6.16 million doses of vaccine were administered in Jiangsu province during 2008-2013, and 4 718 vaccinees reported having adverse event, for a rate of 76.60/100 000 (95% CI: 74.42/100 000-78.79/100 000). The incidence rate of common vaccine reaction and rare vaccine reaction was 71.10/100 000 (95% CI: 68.99/100 000-73.20/100 000) and 5.16/100 000 (95% CI: 4.60/100 000-5.73/100 000), respectively. The main symptoms of common vaccine reactions were fever, swelling, indurations and gastrointestinal reactions. The incidence rates of them were 40.54/100 000, 35.09/100 000, 12.94/100 000 and 0.36/100 000 in turn. The main symptoms of rare vaccine reactions were anaphylactic rashes and angioedema, the incidence rates of which were 4.77/100 000 and 0.15/100 000 respectively. 91.39% (4 002/4 379) of common vaccine reactions and 88.36% (281/318) of rare vaccine reactions happened within 1 d after vaccination. Anaphylactic shock (3 cases) and laryngeal edema (1 case) all happened within 1 d after vaccination. The incidence rate of common vaccine reactions among boys (79.72/100 000, 2 641/3 313 071) was higher than that of girls (61.07/100 000, 1 738/2 846 001) (χ² = 74.92, P < 0.001). The incidence rate of common

  10. Latex allergy in the workplace.

    Science.gov (United States)

    Toraason, M; Sussman, G; Biagini, R; Meade, J; Beezhold, D; Germolec, D

    2000-11-01

    While less than 1% of the general population is sensitized to latex, the U.S. Occupational Safety and Health Administration estimates that 8-12% of health-care workers are sensitized. The major source of workplace exposure is powdered natural rubber latex (NRL) gloves. NRL is harvested from HEVEA: brasiliensis trees and ammoniated to prevent coagulation resulting in the hydrolysis of the latex proteins. Prior to use in manufacturing, the latex is formulated by the addition of multiple chemicals. Thus, human exposure is to a mixture of residual chemicals and hydrolyzed latex peptides. Clinical manifestations include irritant contact dermatitis, allergic contact dermatitis (type IV), and type I immediate hypersensitivity response. Type I (IgE-mediated) NRL allergy includes contact urticaria, systemic urticaria, angioedema, rhinitis, conjunctivitis, bronchospasm, and anaphylaxis. Taking an accurate history, including questions on atopic status, food allergy, and possible reactions to latex devices makes diagnosis of type-I latex allergy possible. To confirm a diagnosis, either in vivo skin prick testing (SPT) or in vitro assays for latex-specific IgE are performed. While the SPT is regarded as a primary confirmatory test for IgE-mediated disease, the absence of a U.S. Food and Drug Administration-licensed HEVEA: brasiliensis latex extract has restricted its use in diagnosis. Serological tests have, therefore, become critically important as alternative diagnostic tests. Three manufacturers currently have FDA clearance for in vitro tests, to detect NRL-specific IgE. The commercially available assays may disagree on the antibody status of an individual serum, which may be due to the assay's detecting anti-NRL IgEs to different allergenic NRL proteins. Sensitized individuals produce specific IgE antibody to at least 10 potent HEVEA: allergens, Hev b 1-Hev b 10, each of which differs in its structure, size, and net charge. The relative content and ratios of Hevs in the

  11. Patrón de reacciones adversas a medicamentos referidas por la población mayor de 15 años Drugs-adverse reactions pattern provoked mentioned by population aged older than 15

    Directory of Open Access Journals (Sweden)

    Liuba Alonso Carbonell

    2009-03-01

    reaction, being more evident in persons aged older than 40 (56,7 %, and in women (60 %. More frequent reported drugs as producing of adverse reactions include antimicrobial agents (42,3 % and anti-inflammatory ones (42,3 %, and non-steroid anti-inflammatory agents (18.1 %. Main adverse events reported are located in skin (35,6 %, and in central nervous system (CNS (17,8 %. More reported adverse reactions were the minor ones (91,2 %. From the severe ones (9,8 %, the more frequent were angioedema and consciousness loss with 10 and 8 cases, respectively. CONCLUSION: pattern of drugs adverse reactions mentioned by surveyed persons is similar to that of Cuban National Coordinator Unit of Pharmacy-surveillance; feed on spontaneous notifications of National Health System professionals.