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Sample records for angioedema

  1. Angioedema

    OpenAIRE

    Kaplan, Allen P

    2008-01-01

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

  2. Hereditary angioedema

    DEFF Research Database (Denmark)

    Peterson, M P; Bygum, A

    2016-01-01

    We report a 64-year-old man who suffered from recurrent visible swelling attacks since the age of 20 as well as episodes with severe upper airway edema, resulting in 4 emergency tracheotomies. Eventually after 44 years he was diagnosed with hereditary angioedema (HAE) type II. The aims of this re...... of this report is to emphasize the importance of awareness concerning HAE, which does not respond to traditional anti-allergic therapy, and remind physicians to test for functional C1-INH deficiency....

  3. Radiologic manifestations of angioedema

    OpenAIRE

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

    2014-01-01

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

  4. Urticaria and angioedema

    Directory of Open Access Journals (Sweden)

    Kanani Amin

    2011-11-01

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

  5. Recurrent angioedema and urticaria.

    OpenAIRE

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

    1993-01-01

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

  6. Recurrent angioedema and urticaria.

    Science.gov (United States)

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

    1993-11-01

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

  7. 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...

  8. [Histaminergic angioedema and chronic urticaria].

    Science.gov (United States)

    Hacard, Florence; Nosbaum, Audrey; Bensaid, Benoit; Nicolas, Jean-François; Augey, Frédéric; Goujon, Catherine; Bérard, Frédéric

    2015-01-01

    Most angioedemas are histaminergic and correspond to deep urticarial swelling. Recurrent histaminergic angioedema led to the diagnosis of chronic urticaria, even when there are no superficial associated hives. Chronic urticaria is a benign disease, and autoimmune in 40 % of cases. The occurrence of angioedema in chronic urticaria is not a sign of severity. The occurrence of angioedema in chronic urticaria is associated with a longer duration of urticarial disease. NSAIDs and/or systemic corticotherapy are classic triggers of angioedema in chronic urticaria. In the absence of clinical endpoints, there is no need to make further assessment in chronic urticaria good responders to antihistamines.

  9. 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.

  10. 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.

  11. Acebrophylline-induced angioedema

    Directory of Open Access Journals (Sweden)

    Sanitha Kuriachan

    2015-01-01

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

  12. Genetics Home Reference: hereditary angioedema

    Science.gov (United States)

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

  13. 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.

  14. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    DEFF Research Database (Denmark)

    Andersen, Michelle Fog; Bygum, Anette

    2015-01-01

    Hereditary angioedema is a rare, but potentially life-threatening genetic disorder that results from an autosomal dominant trait. It is characterized by acute, recurrent attacks of severe local edema, most commonly affecting the skin and mucosa. Swelling in hereditary angioedema patients does...... of hereditary angioedema. The case illustrates how clinicians can have difficulties in handling patients with rare diseases, especially in the emergency care setting....

  15. Angioedema-Urticaria Due to Acitretin.

    Science.gov (United States)

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

    2016-01-01

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

  16. Disease expression in women with hereditary angioedema

    DEFF Research Database (Denmark)

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

    2008-01-01

    OBJECTIVE: Fluctuations in sex hormones can trigger angioedema attacks in women with hereditary angioedema. Combined oral contraceptive therapies, as well as pregnancy, can induce severe attacks. The course of angioedema may be very variable in different women. STUDY DESIGN: Within the PREHAEAT...... project launched by the European Union, data on 150 postpubertal women with hereditary angioedema were collected in 8 countries, using a patient-based questionnaire. RESULTS: Puberty worsened the disease for 62%. Combined oral contraceptives worsened the disease for 79%, whereas progestogen-only pills...... improved it for 64%. During pregnancies, 38% of women had more attacks, but 30% had fewer attacks. Vaginal delivery was usually uncomplicated. Attacks occurred within 48 hours in only 6% of cases. Those more severely affected during menses had more symptoms during pregnancies, suggesting a hormone...

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

    OpenAIRE

    Fletcher, A; Weetman, A P

    1998-01-01

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

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

    Science.gov (United States)

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

    2006-11-01

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

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

    DEFF Research Database (Denmark)

    Cicardi, M; Aberer, W; Banerji, A;

    2014-01-01

    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...

  20. 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.

  1. 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...

  2. 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...

  3. Acute dystonia mimicking angioedema of the tongue

    DEFF Research Database (Denmark)

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

    2013-01-01

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

  4. Acute allergic angioedema of upper lip.

    Science.gov (United States)

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

    2016-01-01

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

  5. Acute allergic angioedema of upper lip

    Science.gov (United States)

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

    2016-01-01

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

  6. Angioedema: Clinical Presentations and Pharmacological Management.

    Science.gov (United States)

    Collins-Yoder, Angela Smith

    2016-01-01

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

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

    Science.gov (United States)

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

    2016-08-01

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

  8. Angioedema due to Pomegranate: Original Image

    Directory of Open Access Journals (Sweden)

    Meliha Findik

    2014-03-01

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

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

    Science.gov (United States)

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

    2014-01-01

    Antecedentes: el angioedema hereditario es un trastorno inflamatorio episódico, que se hereda de manera autosómica dominante y se caracteriza por episodios de edema periférico. Los pacientes pueden tener edema de la pared de cualquier víscera hueca, incluido el intestino. Caso clínico: se comunica el caso de un paciente masculino de 33 años de edad, sin antecedentes de importancia, con dolor abdominal, localizado en el epigastrio, irradiado al cuadrante inferior derecho, acompañado de 5 vómitos. La tomografía abdominal mostró engrosamiento de la pared de la segunda y tercera porción del duodeno, con infiltración de grasa y líquido libre. Los exámenes de laboratorio mostraron: concentraciones bajas del complemento C4 (5.5 mg/dL) y actividad del inhibidor de C1 del complemento de 30%. Conclusiones: el angioedema hereditario es consecuencia de la deficiencia (tipo I) o disfunción (tipo II) del inhibidor C1 del complemento. El dolor abdominal asociado con angioedema es de inicio súbito, como dolor cólico, recurrente y de intensidad moderada. En la actualidad existen dos medicamentos aprobados por la Food and Drug Administration para el tratamiento de pacientes con esta afección.

  10. 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.

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

    DEFF Research Database (Denmark)

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

    2013-01-01

    Angioedema is a sudden localised and often asymmetric swelling of the skin or mucous membranes caused by transient increased endothelial permeability causing plasma extravasation. In the last decades the incidence of severe angioedema involving the upper airways and even fatal outcome due to asph...

  12. 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.

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

    Science.gov (United States)

    Banerji, Aleena

    2011-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Micaela A. Cosatti

    2010-12-01

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

  15. How Not to Be Misled by Disorders Mimicking Angioedema

    DEFF Research Database (Denmark)

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

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Beatriz Frutuoso

    2016-05-01

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

  17. Refractory Angioedema in a Patient with Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Zahra Habibagahi

    2015-07-01

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

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

    Institute of Scientific and Technical Information of China (English)

    Flavio Habal; Vivian Huang

    2012-01-01

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

  19. Burden of Illness in Hereditary Angioedema

    DEFF Research Database (Denmark)

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

    2015-01-01

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

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

    OpenAIRE

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

    2016-01-01

    Introduction: Upper airway angioedema is a life-threatening emergency department (ED) presentation with increasing incidence. Angiotensin-converting enzyme inhibitor induced angioedema (AAE) is a non-mast cell mediated etiology of angioedema. Accurate diagnosis by clinical examination can optimize patient management and reduce morbidity from inappropriate treatment with epinephrine. The aim of this study is to describe the incidence of angioedema subtypes and the management of AAE. We evaluat...

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

    Directory of Open Access Journals (Sweden)

    Aleksić Aleksandra

    2015-01-01

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

  2. 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.

  3. Anaesthetic management of a patient with hereditary angioedema

    Directory of Open Access Journals (Sweden)

    Nergis Ataol

    2015-12-01

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

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

    Directory of Open Access Journals (Sweden)

    Sergio Duarte Dortas Junior

    2012-04-01

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

  5. Isotretinoin induced rash, urticaria, and angioedema: a case report

    Directory of Open Access Journals (Sweden)

    Zonunsanga

    2015-10-01

    Full Text Available Isotretinoin is a vitamin A analogue, which is readily isomerized to tretinoin. It causes normalization of abnormal keratinisation. It also reduces sebum secretion. It also has anti-inflammatory as well as antibacterial properties. It has some adverse effects like teratogenecity, hypertriglyceridemia, pancreatitis, dryness of skin, cheilitis, altered liver functions etc. A 25 years old unmarried lady presented with acne vulgaris, who did not showed improvements with conventional (antibiotics therapy was given isotretinoin. She developed maculopapular rash, urticaria and angioedema Isotretinoin induced urticarial rashes and angioedema is rarely reported as far as our knowledge is concerned.

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

    OpenAIRE

    Gokalp, Hilal; Kaya, Kismet

    2014-01-01

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

  7. 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...

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

    OpenAIRE

    Akinci, Emine; OĞUZTÜRK, Oğuzhan; Coşkun, Figen

    2012-01-01

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

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

    Science.gov (United States)

    Alonso Lebrero, E

    1999-01-01

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

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

    OpenAIRE

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

    2012-01-01

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

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

    OpenAIRE

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

    2016-01-01

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

  12. Angioedema Related to Angiotensin-Converting Enzyme Inhibitors

    OpenAIRE

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

    2015-01-01

    Abstract The number of cases of acquired angioedema related to angiotensin converting enzyme inhibitors induced (ACEI-AAE) is on the increase, with a potential concomitant increase in life-threatening attacks of laryngeal edema. Our objective was to determine the main characteristics of ACEI-AAE attacks and, in doing so, the factors associated with likelihood of hospital admission from the emergency department (ED) after a visit for an attack. A prospective, multicenter, observational study (...

  13. Not all ACE inhibitor related angioedema is always evident: a case which is misdiagnosed as panic attack and speech disorder.

    Science.gov (United States)

    Gulec, M; Caliskaner, Z; Kartal, O; Erel, F; Karaayvaz, M

    2007-01-01

    Angiotensin-converting enzyme (ACE) inhibitors are the most common medications responsible for angioedema. Angioedema is a potentially life threatening conditions especially in geriatric age patients that they have take a several medications include ACE inhibitors and non steroidal anti inflammatory drugs. We present a case an ACE inhibitor induced angioedema that confused many clinical events. PMID:18047821

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

    Science.gov (United States)

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

    2014-01-01

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

  15. Pediatric hereditary angioedema due to C1-inhibitor deficiency

    Directory of Open Access Journals (Sweden)

    Farkas Henriette

    2010-07-01

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

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

    Directory of Open Access Journals (Sweden)

    Caballero T

    2014-09-01

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

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

    Science.gov (United States)

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

    2014-01-01

    Urticaria is a heterogeneous group of disorders, with various clinical manifestations and intensity of symptoms. Urticaria can be induced with a wide variety of environmental stimuli, such as cold, pressure, vibration, sunlight, exercise, temperature changes, heat, and water. In a select group of patients, exercise can induce a spectrum of urticaria symptoms, ranging from cutaneous pruritus and warmth, generalised urticaria, angioedema, and the appearance of such additional manifestations as collapse, upper respiratory distress, and anaphylaxis. Specific provocation tests should be carried out on an individual basis to investigate the suspected cause and proper diagnosis. Modification of activities and behaviour is the mainstay of treatment in patients with physical urticaria. The aim of this study was to emphasise that primary care paediatricians should be able to recognise physical urticaria, supply a patient with rescue medications, and refer him/her to a specialist. In the article, the authors present a 13-year-old girl with typical urticaria lesions and angioedema after exercise. According to the history, physical examination, and provocation test, exercise-induced urticaria and angioedema were diagnosed. PMID:25133816

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

    NARCIS (Netherlands)

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

    2013-01-01

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

  19. 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...

  20. [Hereditary angioedema. Treatment of acute attacks in Argentina].

    Science.gov (United States)

    Malbrán, Alejandro; Malbrán, Eloisa; Menéndez, Alejandra; Fernández Romero, Diego S

    2014-01-01

    In the world, hereditary angioedema (HAE) affects 1 every 50000 persons. It is characterized by highly disabling and recurrent episodes of cutaneous, abdominal and laryngeal episodes of angioedema. Asphyxia related mortality ranges from 15 to 50%. In Argentina a plasma derived C1 inhibitor concentrate (pdC1INH) has been available for the treatment of acute attacks for many decades, however, only15 (26%) out of 58 patients had received pdC1INH at least once until 2008, and only2 (3.4%) had used it regularly. After worldwide approval of the new drugs for the treatment of acute HAE attacks, adding icatibant to pdC1INH in Argentina, and after publication of the therapeutic guide for the country, 42 (82%) out of 51 patients from the original group has pdC1INH available to treat their next attack. However, 16 (18%) patients continue without access to medication and other 15 (35.7%) obtain their therapy spuriously through some other affected relative in their environment. Only 12 (28.6%) patients of the group self-treated at home. Access to treatment has greatly improved, but needs to be extended to all patients and self-treatment at home should be encouraged.

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

    Directory of Open Access Journals (Sweden)

    Alejandro Malbrán

    2014-06-01

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

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

    Directory of Open Access Journals (Sweden)

    Lopa Misra

    2016-01-01

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

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

    Science.gov (United States)

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

    2016-08-01

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

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

    Science.gov (United States)

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

    2014-01-01

    Vibratory angioedema is a rare form of physical urticaria, hereditary or acquired, which occurs at body sites exposed to vibrations. Pathogenic mechanisms of disease are not completely clear and, consequently, current pharmacological treatment is sometimes unsatisfactory. We report the case of a horn player affected by acquired vibratory angioedema, relapsing after prolonged use of the instrument and resistant to systemic antihistamines and corticosteroids, which successfully responded to therapy with low doses of amitriptyline and bromazepam. A neuroinflammatory mechanism can be likely implicated in the pathogenesis of vibratory angioedema, in line with many different cutaneous/mucosal diseases involving a complex interplay of homeostatic/allostatic systems. Furthermore, in mucosal diseases, such as vibratory angioedema, physical/psychological stressors have a relevant role. In such cases, because of the complex interplay between nervous and immune system, the pharmacological activity of benzodiazepines and typical antidepressants may downregulate neuroinflammation. PMID:25052839

  5. 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.

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

    OpenAIRE

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

    2011-01-01

    Angioedema may be caused by nonsteroidal antiinflammatory drugs, angiotensin- converting enzyme inhibitors, radiocontrast media, antibiotics, sea food etc. It can involve an allergic (IgE-mediated) or non-allergic hypersensitivity reaction, both with a similar clinical presentation. While angioedema due to isotretionin has been described previously, this is the first description of angiodema due to acitretin. We report two uncommon cases of palpebral and labial angiodema due to retinoids, by ...

  7. Angioedema por rellenos faciales: Descripción de cinco casos

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    Micaela A. Cosatti

    2010-12-01

    Full Text Available En los últimos años se ha incrementado la utilización de sustancias de relleno facial con fines estéticos. Estos productos, originalmente considerados inertes, se asocian con diversos efectos adversos localizados alrededor del sitio de la aplicación. Describimos a 5 mujeres con antecedentes de inyecciones de sustancia de relleno facial que presentaron como síntoma inicial angioedema facial duro y persistente seguido por la aparición de nódulos subcutáneos. Todas las pacientes fueron derivadas al servicio de alergia por sospecha de angioedema de causa alérgica sin respuesta al tratamiento con antihistamínicos. El angioedema inició 27.6 meses (1 a 48 luego de la inyección del producto, y las pacientes evolucionaron con brotes y remisiones que fueron tratados con corticoides orales y en 2 oportunidades con inyecciones locales. El tiempo medio desde el inicio de los síntomas hasta la remisión del angioedema fue 8.75 meses (1 a 24. A octubre de 2009 cuatro pacientes se mantuvieron en remisión persistente, luego de un seguimiento clínico de 24.5 meses (7 a 36. Una paciente continúa con exacerbaciones luego de 11 meses de iniciados los síntomas. Las sustancias de relleno facial pueden producir angioedema como evento adverso y deben ser consideradas en el diagnóstico diferencial del angioedema persistente. Sólo responden al tratamiento con esteroides y en algunos casos esteroides dependientes, con ciclosporina. La frecuencia de angioedema por rellenos faciales entre pacientes con angioedema asistidos en la Unidad de Asma, Alergia e Inmunología Clínica fue del 0.5%.

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

    OpenAIRE

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

    2000-01-01

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

  9. 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.

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

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    Kaur R

    2014-12-01

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

  11. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema

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    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.

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

    Science.gov (United States)

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

    2015-03-01

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

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

    Science.gov (United States)

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

    2016-09-01

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

  14. C-reactive protein levels in hereditary angioedema.

    Science.gov (United States)

    Hofman, Z L M; Relan, A; Hack, C E

    2014-07-01

    Hereditary angioedema (HAE) patients experience recurrent episodes of angioedema attacks that can be painful, disfiguring and even life-threatening. The disorder results from a mutation in the gene that controls the synthesis of C1-inhibitor (C1INH). C1INH is a major regulator of activation of the contact system. It is often assumed that attacks results from uncontrolled local activation of the contact system with subsequent formation of bradykinin. To evaluate the involvement of inflammatory reactions in HAE, we analysed C-reactive protein (CRP) levels. HAE patients included in a clinical database of recombinant human C1-inhibitor (rhC1INH) studies were evaluated. For the current study we analysed CRP levels when patients were asymptomatic, during a clinical attack and in a follow-up period, and correlated these with the clinical manifestations of the attack. Data from 68 HAE patients were analysed and included CRP levels on 273 occasions. While asymptomatic, 20% of the patients analysed had increased CRP. At the onset of the attack (P = 0·049) and during the next 24 h CRP rose significantly (P = 0·002) in patients with an abdominal location, and post-attack levels were significantly higher in these patients than in patients with attacks at other locations (P = 0·034). In conclusion, CRP levels are elevated in a substantial proportion of asymptomatic HAE patients. Levels of CRP increase significantly during an abdominal attack. These data suggest low-grade systemic inflammatory reactions in HAE patients as well as a triggering event for attacks that starts prior to symptom onset.

  15. 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

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

    Science.gov (United States)

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

    2016-03-01

    Although angiotensin-converting enzyme (ACE) inhibitors are widely used as the first choice drug for treating hypertension, we have only a superficial understanding of their relationship to angioedema. We report a case of life-threatening angioedema. The case was a 60-year-old man who had been taking an ACE inhibitor for hypertension for 11 years. He visited his home doctor for dyspnea, and tongue and neck swelling. He was transported to our hospital because of the possibility of airway obstruction. On admission, his tongue and neck swelling became more severe. We performed an intubation using an endoscope and started airway management. We also stopped his ACE inhibitor. The severe tongue and neck swelling improved gradually and he was extubated on day 3. On the fifth day he was discharged. We diagnosed angioedema caused by an ACE inhibitor. Although the risk of airway obstruction with ACE inhibitors is acknowledged, we have only a superficial understanding of how prolonged ACE inhibitor treatment induces angioedema. So we should consider angioedema in cases of taking ACE inhibitors, especially in cases of prolonged treatment. PMID:26972946

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

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    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

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

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    Ghazi A

    2013-05-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

  20. New therapies for hereditary angioedema: disease outlook changes dramatically.

    Science.gov (United States)

    Frank, Michael M; Jiang, Haixiang

    2008-01-01

    Hereditary angioedema (HAE) is an autosomal dominant disease associated with episodic attacks of nonpitting edema that may affect any external or mucosal body surface. Attacks most often affect the extremities, causing local swelling, the GI tract, leading to severe abdominal pain, and the mouth and throat, at times causing asphyxiation. Most patients with HAE have low levels of the plasma serine protease inhibitor C1 inhibitor. The edema in these patients is caused by unregulated generation of bradykinin. Effective chronic therapy of patients with impeded androgens or plasmin inhibitors has been available for decades, but in the United States, we do not have therapy for acute attacks. Five companies have completed or are in the process of conducting phase 3 clinical trials, double-blind, placebo-controlled studies of products designed to terminate acute attacks or to be used in prophylaxis. Two companies, Lev Pharmaceuticals and CSL Behring, have preparations of C1 inhibitor purified from plasma that have been used in Europe for decades (trade names Cinryze and Berinert P, respectively). One company, Pharming, has developed a recombinant C1 inhibitor preparation. One company, Dyax, is testing a kallikrein inhibitor (ecallantide), and one company, Jerini, is completing testing of a bradykinin type 2 receptor antagonist (Icatibant). Although little has been published thus far, all of these products may prove effective. It is likely that HAE treatment will change dramatically within the next few years. PMID:18206518

  1. New therapies for hereditary angioedema: disease outlook changes dramatically.

    Science.gov (United States)

    Frank, Michael M; Jiang, Haixiang

    2008-01-01

    Hereditary angioedema (HAE) is an autosomal dominant disease associated with episodic attacks of nonpitting edema that may affect any external or mucosal body surface. Attacks most often affect the extremities, causing local swelling, the GI tract, leading to severe abdominal pain, and the mouth and throat, at times causing asphyxiation. Most patients with HAE have low levels of the plasma serine protease inhibitor C1 inhibitor. The edema in these patients is caused by unregulated generation of bradykinin. Effective chronic therapy of patients with impeded androgens or plasmin inhibitors has been available for decades, but in the United States, we do not have therapy for acute attacks. Five companies have completed or are in the process of conducting phase 3 clinical trials, double-blind, placebo-controlled studies of products designed to terminate acute attacks or to be used in prophylaxis. Two companies, Lev Pharmaceuticals and CSL Behring, have preparations of C1 inhibitor purified from plasma that have been used in Europe for decades (trade names Cinryze and Berinert P, respectively). One company, Pharming, has developed a recombinant C1 inhibitor preparation. One company, Dyax, is testing a kallikrein inhibitor (ecallantide), and one company, Jerini, is completing testing of a bradykinin type 2 receptor antagonist (Icatibant). Although little has been published thus far, all of these products may prove effective. It is likely that HAE treatment will change dramatically within the next few years.

  2. 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.

  3. Management of upper airway edema caused by hereditary angioedema

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    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.

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

    Science.gov (United States)

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

    2016-02-01

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

  5. Generation of plasmin during acute attacks of hereditary angioedema.

    Science.gov (United States)

    Cugno, M; Hack, C E; de Boer, J P; Eerenberg, A J; Agostoni, A; Cicardi, M

    1993-01-01

    Hereditary angioedema is caused by a genetic deficiency of C1-inhibitor, a serine protease inhibitor that regulates activation of complement, contact, and fibrinolytic systems. Symptoms (bouts of subcutaneous and mucous swelling) depend on the release of a vasoactive mediator, probably through activation of these three systems. We studied the interrelationship among complement, contact, and fibrinolytic activation in 23 patients with hereditary angiodema, 18 during remission and five during an attack, by measuring plasma levels of C1-C1 inhibitor, factor XIIa-C1 inhibitor, kallikrein-C1 inhibitor, and plasmin-alpha 2-antiplasmin complexes, tissue plasminogen activator, and urokinase plasminogen activator. In addition, cleavage of high-molecular weight kininogen was detected by sodium dodecyl sulfate polyacrylamide gel electrophoresis analysis and quantified by densitometry. During remission, plasma levels of C1-C1 inhibitor complexes were elevated (p = 0.0002), whereas the other parameters were within the normal range. During acute attacks, not only plasma levels of C1-C1 inhibitor complexes but also those of plasmin-alpha 2-antiplasmin complexes (P = 0.0009) and cleaved high-molecular weight kininogen were elevated. A positive correlation between plasmin-alpha 2-antiplasmin complexes and cleaved high-molecular weight kininogen was observed (r = 0.75, p attacks is associated with the activation of the fibrinolytic system.

  6. Life Threatening Idiopathic Recurrent Angioedema Responding to Cannabis

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    Amit Frenkel

    2015-01-01

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

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

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    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

  8. [Edematous syndromes caused by capillary hyperpermeability. Diffuse angioedema].

    Science.gov (United States)

    Lagrue, G; Behar, A; Maurel, A

    1989-01-01

    Edema due to increased capillary permeability (ICP) may be diffuse or localized. Local edemas (Quincke edema, angioneurotic edema) are most often allergic or very rarely due to a defect in C1-inhibitor. Generalized edemas due to ICP share the following clinical features: Fluid retention (subcutaneous edema and diffused swelling) is predominant in lower limbs; it is worsened by orthostatism and warmth and alleviated by decubitus and cold, with important weight variations between morning and evening. It is associated with enhanced thirst, hypotension, oliguria, headaches and blood volume reduction; secondary hyperaldosteronism is the main mechanism. These troubles are due to ICP, associated with lymphatic drainage abnormalities; ICP is measured by the isotopic Landis Test. This abnormality is present in several diseases. Idiopathic orthostatic edema (IOE) is frequent and often unrecognized, occurring mainly in women, often associated with luteal insufficiency. Iatrogenic complications (diuretic and laxative abuses) are frequently superimposed. ICP may be corrected by vitamins P (rutin, anthocyanosides, diosmin, Ginkgo biloba extracts...) Cyclic shock due to ICP is rare. It is characterized by cyclic edema and shock with hypovolemia, hypoproteinemia; the mechanism of shock is a severe loss of fluid and protein from the vascular bed. It is often associated with monoclonal gammapathy and complement activation. In our personal case, the trouble in CP was present all along the disease with permanent edema and low blood pressure (especially in orthostatism). Vit "P" and Ginkgo biloba extracts were able to partially improve CP and the clinical troubles. However, in spite of this treatment a fatal shock occurred after ten years follow-up. Episodic angioedema associated with eosinophilia was first described by Gleich.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:2778397

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

    Science.gov (United States)

    Longhurst, Hilary

    2008-03-01

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

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

    Directory of Open Access Journals (Sweden)

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

    2013-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Acílio Marques

    2014-01-01

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

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

    Science.gov (United States)

    Longhurst, Hilary

    2008-03-01

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

  13. Asymmetric oro-facial angioedema following alteplase for acute ischaemic stroke.

    Science.gov (United States)

    Wu, Teddy Y; Wei, Diana Y; Baker, Yvette; Sharkey, Brendan; Frith, Richard W

    2014-11-01

    We present a patient with asymmetric oro-facial angioedema following thrombolysis for acute ischaemic stroke with serial photographs of this phenomenon. We discuss the mechanism for the development of asymmetric oro-facial oedema following thrombolysis and suggest a management plan. PMID:24974191

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

    NARCIS (Netherlands)

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

    2007-01-01

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

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

    DEFF Research Database (Denmark)

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

    2015-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    Rui Tang; Hong-yu Zhang; Jia Gan

    2009-01-01

    HEREDITARY angioedema (HAE) is an autosomal dominant inherited condition which was initially described by Osier in 1888.~1 Patients with HAE can develop rapid subcutaneous or submucosal edema involving the hands, feet, limbs, face, intestinal tract, even larynx and trachea.

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

    OpenAIRE

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

    2014-01-01

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

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

    Science.gov (United States)

    Varga, Lilian; Farkas, Henriette

    2008-11-01

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

  19. Recombinant replacement therapy for hereditary angioedema due to C1 inhibitor deficiency.

    Science.gov (United States)

    Moldovan, Dumitru; Bernstein, Jonathan A; Cicardi, Marco

    2015-01-01

    Hereditary angioedema is a rare genetic condition transmitted as an autosomal dominant trait and characterized most commonly by the production of either inadequate or nonfunctioning C1 esterase inhibitor (C1-INH), a blood protein that regulates proteases in the complement, fibrinolytic and contact systems. Patients with hereditary angioedema suffer from episodic, unpredictable manifestations of edema affecting multiple anatomical locations, including the GI tract, facial tissue, the upper airway, oropharynx, urogenital region and/or the arms and legs. A rational approach to treatment is replacement of C1-INH protein, to normalize the levels of C1-INH activity and halt the progression of the biochemical activation processes underlying the edema formation. Ruconest is a highly purified recombinant human C1-INH. This article will focus on the results of ten clinical studies demonstrating the efficacy and safety of Ruconest(®) (Pharming Group NV, Leiden, the Netherlands), which is now approved for use in Europe, Israel and the USA. PMID:26250409

  20. Recombinant replacement therapy for hereditary angioedema due to C1 inhibitor deficiency.

    Science.gov (United States)

    Moldovan, Dumitru; Bernstein, Jonathan A; Cicardi, Marco

    2015-01-01

    Hereditary angioedema is a rare genetic condition transmitted as an autosomal dominant trait and characterized most commonly by the production of either inadequate or nonfunctioning C1 esterase inhibitor (C1-INH), a blood protein that regulates proteases in the complement, fibrinolytic and contact systems. Patients with hereditary angioedema suffer from episodic, unpredictable manifestations of edema affecting multiple anatomical locations, including the GI tract, facial tissue, the upper airway, oropharynx, urogenital region and/or the arms and legs. A rational approach to treatment is replacement of C1-INH protein, to normalize the levels of C1-INH activity and halt the progression of the biochemical activation processes underlying the edema formation. Ruconest is a highly purified recombinant human C1-INH. This article will focus on the results of ten clinical studies demonstrating the efficacy and safety of Ruconest(®) (Pharming Group NV, Leiden, the Netherlands), which is now approved for use in Europe, Israel and the USA.

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

    Science.gov (United States)

    Varga, Lilian; Farkas, Henriette

    2008-11-01

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

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

    DEFF Research Database (Denmark)

    Visy, Beáta; Füst, George; Bygum, Anette;

    2007-01-01

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

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

    Science.gov (United States)

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

    2016-07-01

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

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

    Science.gov (United States)

    Wu, Maddalena Alessandra; Castelli, Roberto

    2016-02-01

    Several clinical and biological features of lymphoproliferative diseases have been associated with an increased risk of developing autoimmune manifestations. Acquired deficiency of C1-inhibitor (C1-INH) (AAE) is a rare syndrome clinically similar to hereditary angioedema (HAE) characterized by local increase in vascular permeability (angioedema) of the skin and the gastrointestinal and oro-pharyngo-laryngeal mucosa. Bradykinin, a potent vasoactive peptide, released from high molecular weight kininogen when it is cleaved by plasma kallikrein (a serine protease controlled by C1-INH), is the mediator of symptoms. In total 46% of AAE patients carry an underlying hematological disorder including monoclonal gammopathy of uncertain significance (MGUS) or B cell malignancies. However, 74% of AAE patients have anti-C1-INH autoantibodies without hematological, clinical or instrumental evidence of lymphoproliferative disease. Unlike HAE patients, AAE patients usually have late-onset symptoms, do not have a family history of angioedema and present variable response to treatment due to the hypercatabolism of C1-INH. Experiments show that C1-INH and/or the classical complement pathway were consumed by the neoplastic lymphatic tissues and/or anti-C1-INH neutralizing autoantibodies. Therapy of AAE follows two directions: 1) prevention/reversal of the symptoms of angioedema; and 2) treatment of the associated disease. Different forms of B cell disorders coexist and/or evolve into each other in AAE and seem to be dominated by an altered control of B cell proliferation, thus AAE represents an example of the strict link between autoimmunity and lymphoproliferation. PMID:26068904

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2004-09-01

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

  7. 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

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

    Science.gov (United States)

    Timmis, Christopher; Epstein, Elliot; Salim, Mohmad

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Kirankumar N Kulkarni

    2014-01-01

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

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

    DEFF Research Database (Denmark)

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

    2015-01-01

    BACKGROUND: Hereditary angioedema types I and II are caused by a functional deficiency of C1 inhibitor (C1-INH) leading to overproduction of bradykinin. The current functional diagnostic assays employ inhibition of activated C1s, however, an alternative, more physiologic method, is desirable...... samples were considered equivocal (4 controls and 38 patients). CONCLUSIONS: Diagnosis of HAE types I and II can be ascertained by inhibition of enzymes of the bradykinin-forming cascade; namely, factor XIIa and kallikrein. Either method yields functional C1-INH levels in HAE patients (types I & II...

  11. Clinical similarities among bradykinin-mediated and mast cell-mediated subtypes of non-hereditary angioedema : a retrospective study

    NARCIS (Netherlands)

    Schulkes, Karlijn J G; van den Elzen, Mignon T.; Hack, Erik C.; Otten, Henderikus G; Bruijnzeel-Koomen, Carla A.F.M.; Knulst, André C.

    2015-01-01

    BACKGROUND: Non-hereditary angioedema (non-HAE) is characterized by local swelling due to self-limiting, subcutaneous or submucosal extravasation of fluid, and can be divided into three subtypes. These subtypes are believed to have different pathophysiological backgrounds and are referred to in rece

  12. 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;

    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 diagn...

  13. 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 perf......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...

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

    Directory of Open Access Journals (Sweden)

    Hossein Vakili

    2014-01-01

    Full Text Available A 50-year-old man was admitted to our emergency department due to new episode of palpitation. He had history of angioplasty of right coronary artery (RCA with drug eluting stent 2 years ago. His electrocardiogram revealed atrial fibrillation (AF. Intravenous amiodarone 150 mg during 10 minutes and then 1 mg/min infusion were started to achieve rate control and pharmacologic conversion to sinus rhythm. After 60 minutes of starting amiodarone infusion, he developed swelling of the skin around his mouth and eyes, and also mucosa of the mouth, eyes and tongue. To conclude, angioedema should be considered a rare side effect of amiodarone which is used broadly in cardiovascular field.

  15. 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

    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......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...... detailed descriptive data and patient testimonials on the impact of HAE on patients' health-related quality of life. CONCLUSION: The present manuscript describes the development and plans for implementing a multi-country European study with the aim of characterizing the humanistic and economic burden...

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

    Institute of Scientific and Technical Information of China (English)

    Rui Tang; Shi Chen; Hong-yu Zhang

    2012-01-01

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

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

    OpenAIRE

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

    2013-01-01

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

  18. [Acquired angioedema with C1-INH deficiency and accompanying chronic spontaneous urticaria in a patient with chronic lymphatic B cell leukemia].

    Science.gov (United States)

    Klossowski, N; Braun, S A; von Gruben, V; Losem, C; Plewe, D; Homey, B; Meller, S

    2015-10-01

    Acquired angioedema due to C1 inhibitor deficiency (C1-INH-AAE) is characterized by recurrent edema of the subcutaneous and/or submucosal tissue without wheals and negative family history of angioedema. Here, we present the case of a patient with a chronic lymphatic B cell leukemia who suffered from both C1-INH-AAE and chronic spontaneous urticaria. Oral corticosteroids, antihistamines, and the anti-IgE antibody omalizumab were applied to treat the chronic urticaria in combination with the plasma-derived C1 esterase inhibitor concentrate Berinert® and the bradykinin B2 receptor antagonist icatibant, but the symptoms did not improved significantly. Thus, polychemotherapy targeting the slow-growing lymphoproliferative disease including rituximab was initiated, which resulted in remission of both the urticaria and the angioedema.

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

    Science.gov (United States)

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

    2016-01-01

    Angioedema of the upper airways is a severe and potentially life-threatening condition. The incidence has been increasing in the past two decades, primarily due to pharmaceuticals influencing the generation or degradation of the vasoactive molecule bradykinin. Plasma-derived C1-esterase inhibitor concentrate is a well-established treatment option of hereditary and acquired complement C1-esterase inhibitor deficiency, which are also mediated by an increased level of bradykinin resulting in recurrent angioedema. We here present a case of severe angiotensin converting enzyme-inhibitor related angioedema (ACEi-AE) of the hypopharynx that completely resolved rapidly after the infusion of plasma-derived C1-inhibitor concentrate adding to the sparse reports in the existing literature. PMID:27123347

  20. Defective glycosylation of coagulation factor XII underlies hereditary angioedema type III

    Science.gov (United States)

    Björkqvist, Jenny; de Maat, Steven; Lewandrowski, Urs; Di Gennaro, Antonio; Oschatz, Chris; Schönig, Kai; Nöthen, Markus M.; Drouet, Christian; Braley, Hal; Nolte, Marc W.; Sickmann, Albert; Panousis, Con; Maas, Coen; Renné, Thomas

    2015-01-01

    Hereditary angioedema type III (HAEIII) is a rare inherited swelling disorder that is associated with point mutations in the gene encoding the plasma protease factor XII (FXII). Here, we demonstrate that HAEIII-associated mutant FXII, derived either from HAEIII patients or recombinantly produced, is defective in mucin-type Thr309-linked glycosylation. Loss of glycosylation led to increased contact-mediated autoactivation of zymogen FXII, resulting in excessive activation of the bradykinin-forming kallikrein-kinin pathway. In contrast, both FXII-driven coagulation and the ability of C1-esterase inhibitor to bind and inhibit activated FXII were not affected by the mutation. Intravital laser-scanning microscopy revealed that, compared with control animals, both F12–/– mice reconstituted with recombinant mutant forms of FXII and humanized HAEIII mouse models with inducible liver-specific expression of Thr309Lys-mutated FXII exhibited increased contact-driven microvascular leakage. An FXII-neutralizing antibody abolished bradykinin generation in HAEIII patient plasma and blunted edema in HAEIII mice. Together, the results of this study characterize the mechanism of HAEIII and establish FXII inhibition as a potential therapeutic strategy to interfere with excessive vascular leakage in HAEIII and potentially alleviate edema due to other causes. PMID:26193639

  1. 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...... doses were lower than those reported for 252 'younger adults' (those aged

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

    Science.gov (United States)

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

    2016-02-01

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

  3. Current characteristics associated with hereditary angioedema attacks and treatment: the home infusion based patient experience.

    Science.gov (United States)

    Tachdjian, Raffi; Banerji, Aleena; Guyer, Autumn; Morphew, Tricia

    2015-01-01

    This article presents a current perspective on the characteristics of hereditary angioedema (HAE) attacks and treatment as captured by a home infusion service. Retrospective data on 158 HAE patients who were enrolled in this acute treatment program were analyzed for factors surrounding an attack. The majority of patients had a high level of disease severity at baseline (88%), with a higher than expected likelihood of having a positive family history (87.8%). The most likely times for patients to call for home treatment were just before and during working hours (6:00 A.M.-5:00 P.M.). Eighty-three percent had more than one alternate mode of medication. Factors associated with a severe attack included an overall severe rating of HAE attacks in the previous year, an abdominal attack alone or a combination of peripheral and abdominal attacks versus a peripheral attack alone, and the use of two doses rather than one for treatment of the current attack. Average time to relief onset was 43.5 minutes. One dose of ecallantide was sufficient to treat the majority of attacks, and a second dose was needed in 23.6% of patients experiencing a severe attack. However, patients who reported both a severe attack rating during the previous year and experiencing only a peripheral current attack were more likely to experience a severe current attack. Acute treatment paradigms for HAE remain diverse. Understanding factors driving these decisions could help alleviate the overall burden of this disease and help overcome some of the challenges faced by the patients and their caretakers and improve their quality of life. Enhanced capture and analysis of prodromal factors in future studies should help us further alleviate the burden of this disease.

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

    Directory of Open Access Journals (Sweden)

    Dagen Callie

    2010-05-01

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

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

    NARCIS (Netherlands)

    M. Levi; G. Choi; C. Picavet; C.E. Hack

    2006-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Li HH

    2016-09-01

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

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

    Science.gov (United States)

    Lösch, Barbara; Wank, Rudolf

    2014-01-01

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

  8. Elevated D-dimers in attacks of hereditary angioedema are not associated with increased thrombotic risk

    Science.gov (United States)

    Reshef, A; Zanichelli, A; Longhurst, H; Relan, A; Hack, C E

    2015-01-01

    Background Recommended management of attacks of hereditary angioedema (HAE) due to C1 esterase inhibitor (C1-INH) deficiency (C1-INH-HAE) includes therapy with exogenous C1INH. Thrombotic/thromboembolic events (TEE) have been reported with plasma-derived C1INH, but so far none with recombinant human C1INH (rhC1INH). This phase III, randomized, placebo (saline)-controlled study evaluated the safety of rhC1INH 50 IU/kg for the treatment of acute attacks in 74 patients with C1-INH-HAE. Methods Monitoring for TEE and assessment of risk of deep vein thrombosis (DVT) by the Wells prediction rule were performed, and levels of fibrin degradation products (plasma D-dimers) were assessed before study drug administration (baseline), 2 h, and 7 days posttreatment. Results Plasma D-dimer levels were elevated in 80% of the patients (median [25th–75th percentiles]: 2149 [480–5105] μg/l; normal ≤250 μg/l) and were higher in patients with submucosal (abdominal, oropharyngeal–laryngeal) attacks (3095 [890–10000] μg/l; n = 29) compared with subcutaneous (peripheral, facial) attacks (960 [450–4060] μg/l; n = 35). Median plasma D-dimer levels were comparable across treatment groups at baseline (1874 [475–4568] μg/l rhC1INH; 2259 [586–7533] μg/l saline) and 2 h postinfusion (2389 [760–4974] μg/l rhC1INH; 2550 [310–8410] μg/l saline); median plasma D-dimer levels were decreased by Day 7 in both groups (425 [232–3240] μg/l rhC1INH; 418 [246–2318] μg/l saline). No increased risk of DVT was identified, nor any TEE reported in rhC1INH treated or controls. Conclusion Elevated plasma D-dimer levels were associated with acute C1-INH-HAE attacks, particularly with submucosal involvement. However, rhC1INH therapy was not associated with thrombotic events. PMID:25640891

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

    Directory of Open Access Journals (Sweden)

    Bowen Tom

    2011-02-01

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

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

    DEFF Research Database (Denmark)

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

    2009-01-01

    Hereditary angioedema (HAE) is often debilitating with a serious effect on quality of life (QOL). Treatment of acute HAE attacks is usually with C1 esterase inhibitor (C1-INH) concentrates; however, treatment can be delayed by patients' travel time for attending emergency units. We assessed...... the Dermatology Life Quality Index (DLQI) and 36-Item Short Form Survey (SF-36) questionnaires. Seven patients were recruited into the study. QOL was assessed at baseline and after 3 to 48 months of home therapy. The mean DLQI score fell from 12.6 +/- 4.65 to 2.7 +/- 1.38 (P ... for the individual and combined components also improved significantly. No serious complications were documented during a follow-up period of 27 to 72 months. Self-administration of C1-INH improved QOL on both physical and psychological parameters. Patients were able to resume a normal life without restrictions...

  11. rhC1INH: a new drug for the treatment of attacks in hereditary angioedema caused by C1-inhibitor deficiency.

    Science.gov (United States)

    Varga, Lilian; Farkas, Henriette

    2011-03-01

    Recombinant human C1 esterase inhibitor (rhC1INH) (Ruconest(®), Pharming) is a new drug developed for the relief of symptoms occurring in patients with angioedema due to C1-inhibitor deficiency. Pertinent results have already been published elsewhere; this article summarizes the progress made since then. Similar to the purified C1-inhibitor derived from human plasma, the therapeutic efficacy of rhC1INH results from its ability to block the actions of enzymes belonging to the overactivated bradykinin-forming pathway, at multiple locations. During clinical trials into the management of acute edema, a total of 190 subjects received recombinant C1-inhibitor by intravenous infusion on 714 occasions altogether. Dose-ranging efficacy studies established 50 U/kg as the recommended dose, and demonstrated the effectiveness of this agent in all localizations of hereditary angioedema attacks. Studies into the safety of rhC1INH based on 300 administrations to healthy subjects or hereditary angioedema patients followed-up for 90 days have not detected the formation of autoantibodies against rhC1INH or IgE antibodies directed against rabbit proteins, even after repeated administration on multiple occasions. These findings met favorable appraisal by the EMA, which granted European marketing authorization for rhC1INH. Pharming is expected to file a biological licence with the US FDA by the end of 2010 to obtain marketing approval in the USA. The launch of rhC1INH onto the pharmaceutical market may represent an important progress in the management of hereditary angioedema patients. PMID:21426252

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

    DEFF Research Database (Denmark)

    Caballero, Teresa; Farkas, Henriette; Bouillet, Laurence;

    2012-01-01

    BACKGROUND: There are a limited number of publications on the management of gynecologic/obstetric events in female patients with hereditary angioedema caused by C1 inhibitor deficiency (HAE-C1-INH). OBJECTIVE: We sought to elaborate guidelines for optimizing the management of gynecologic/obstetri...... patients, genetic counseling, infertility, abortion, lactation, menopause treatment, and endometrial cancer. CONCLUSIONS: A consensus for the management of female patients with HAE-C1-INH is presented....

  13. rhC1INH: a new drug for the treatment of attacks in hereditary angioedema caused by C1-inhibitor deficiency.

    Science.gov (United States)

    Varga, Lilian; Farkas, Henriette

    2011-03-01

    Recombinant human C1 esterase inhibitor (rhC1INH) (Ruconest(®), Pharming) is a new drug developed for the relief of symptoms occurring in patients with angioedema due to C1-inhibitor deficiency. Pertinent results have already been published elsewhere; this article summarizes the progress made since then. Similar to the purified C1-inhibitor derived from human plasma, the therapeutic efficacy of rhC1INH results from its ability to block the actions of enzymes belonging to the overactivated bradykinin-forming pathway, at multiple locations. During clinical trials into the management of acute edema, a total of 190 subjects received recombinant C1-inhibitor by intravenous infusion on 714 occasions altogether. Dose-ranging efficacy studies established 50 U/kg as the recommended dose, and demonstrated the effectiveness of this agent in all localizations of hereditary angioedema attacks. Studies into the safety of rhC1INH based on 300 administrations to healthy subjects or hereditary angioedema patients followed-up for 90 days have not detected the formation of autoantibodies against rhC1INH or IgE antibodies directed against rabbit proteins, even after repeated administration on multiple occasions. These findings met favorable appraisal by the EMA, which granted European marketing authorization for rhC1INH. Pharming is expected to file a biological licence with the US FDA by the end of 2010 to obtain marketing approval in the USA. The launch of rhC1INH onto the pharmaceutical market may represent an important progress in the management of hereditary angioedema patients.

  14. 84 Immuno-Safety of Recombinant Human C1 Inhibitor in Patients With Hereditary Angioedema: An Integrated Analysis

    Science.gov (United States)

    Hack, Erik; Relan, Anurag; Kaufman, Leonard; Pijpstra, Rienk

    2012-01-01

    Background Recombinant C1 inhibitor (rhC1INH) is a novel therapeutic option for the treatment of acute angioedema attacks in patients with hereditary angioedema (HAE). The amino acid sequence of rhC1INH is identical to that of endogenous C1INH. However, any recombinant protein may elicit antibodies against the protein and/or host related impurities (HRI). Clinical consequences of these antibodies can theoretically range from no clinical symptoms to allergic reactions and reduced C1INH activity due to neutralizing antibodies. Objective To analyze the immuno-safety of rhC1INH in symptomatic patients with HAE. Methods Plasma samples were collected pre-treatment and 22 and 90 days post-treatment of an acute angioedema attack. Plasma samples were tested for the presence of antibodies against plasma-derived C1INH and rhC1INH using 6 different, validated enzyme-linked immunosorbent assays (ELISAs), to detect IgM, IgG and IgA antibodies against plasma-derived C1INH or rhC1INH. Antibodies against HRI in plasma samples were measured in an ELISA testing for all antibody classes. Plasma samples from normal healthy controls and HAE patients, never exposed to rhC1INH, were used to estimate cut off levels of the assays. Plasma samples with antibody levels above the cut-off level in the screening assays were tested in confirmatory displacement assay in case of anti-HRI antibodies and in an assay for neutralizing antibodies in case of antibodies against C1INH. Results Data from 155 symptomatic HAE patients having received a total of 424 administrations of rhC1INH were analyzed. The frequency of anti-C1INH antibody levels above the assay cut-off was low and similar in pre- and post-exposure samples (1.7 and 1.8%, respectively). Results above the assay cut-off were sporadic and transient. Occurrence of anti-C1INH antibodies did not correlate with repeated treatment or time since last treatment. No neutralizing antibodies were detected. A total of 5/155 (3%) rhC1INH-treated patients

  15. Angioedema Related to Angiotensin-Converting Enzyme Inhibitors: Attack Severity, Treatment, and Hospital Admission in a Prospective Multicenter Study.

    Science.gov (United States)

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

    2015-11-01

    The number of cases of acquired angioedema related to angiotensin converting enzyme inhibitors induced (ACEI-AAE) is on the increase, with a potential concomitant increase in life-threatening attacks of laryngeal edema. Our objective was to determine the main characteristics of ACEI-AAE attacks and, in doing so, the factors associated with likelihood of hospital admission from the emergency department (ED) after a visit for an attack.A prospective, multicenter, observational study (April 2012-December 2014) was conducted in EDs of 4 French hospitals in collaboration with emergency services (SAMU 93) and a reference center for bradykinin-mediated angioedema. For each patient presenting with an attack, emergency physicians collected demographic and clinical presentation data, treatments, and clinical course. They recorded time intervals from symptom onset to ED arrival and to treatment decision, from ED arrival to specific treatment with plasma-derived C1-inhibitor (C1-INH) or icatibant, and from specific treatment to onset of symptom relief. Attacks requiring hospital admission were compared with those not requiring admission.Sixty-two eligible patients with ACEI-AAE (56% men, median age 63 years) were included. Symptom relief occurred significantly earlier in patients receiving specific treatment than in untreated patients (0.5 [0.5-1.0] versus 3.9 [2.5-7.0] hours; P patients, 27 (44%) were admitted to hospital from the ED. In multivariate analysis, laryngeal involvement and progressive swelling at ED arrival were independently associated with admission (Odds ratio [95% confidence interval] = 6.2 [1.3-28.2] and 5.9 [1.3-26.5], respectively). A favorable course was observed in all patients. Three patients (5%) experienced a recurrence after angiotensin-converting enzyme inhibitor discontinuation after a median follow-up of 18 (11-30) months.Two severity criteria-laryngeal edema and the progression of the edema-were independent factors associated with

  16. C1-inhibitor therapy for hereditary angioedema attacks: prospective patient assessments of health-related quality of life.

    Science.gov (United States)

    Bewtra, Againdra K; Levy, Robyn J; Jacobson, Kraig W; Wasserman, Richard L; Machnig, Thomas; Craig, Timothy J

    2012-01-01

    C1-inhibitor (INH) concentrate, which is recommended as first-line treatment for acute hereditary angioedema (HAE) attacks in many countries, was recently approved in the United States. We sought to solicit patients' feedback about their health-related quality of life (HRQoL) while being treated with C1-INH concentrate for acute HAE attacks under real-world conditions, as well as the personal impact of the availability of C1-INH on lifestyle and mental health domains. Subjects enrolled in an open-label study of C1-INH at 20 U/kg for acute HAE attacks were invited to participate in a prospectively designed survey to solicit "real-time" patient responses that were collected via an interactive voice response service or online with a personal computer. Eighteen subjects submitted 60 quarterly HRQoL and treatment impact survey responses over 29 months. Seventeen of 18 patients responding reported mean short form 12 HRQoL scores that were within a normal range. More than one-half indicated that C1-INH availability made them feel somewhat or much better, and >80% reported having a better outlook on the future and feeling more secure about the danger of life-threatening attacks. These data confirm a high level of HRQoL and a positive impact in lifestyle and emotional domains among patients who were treated for acute attacks of HAE with C1-INH concentrate.

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

    Directory of Open Access Journals (Sweden)

    Aya Saeb

    2016-01-01

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

  18. 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.

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

    Institute of Scientific and Technical Information of China (English)

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

    2011-01-01

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

  20. Ace Inhibitors and Angioedema

    NARCIS (Netherlands)

    Vleeming W; van Amsterdam JGC; de Wildt DJ; Stricker B; TOX

    1995-01-01

    Dit rapport beschrijft de risico's die verbonden zijn aan het gebruik van angiotensine converting enzym (ACE) remmers. Hierbij staat de bijwerking angio-oedeem centraal. De benodigde literatuur is verzameld aan de hand van een zoekaktie middels MEDLINE. ACE-remmers zijn in gebruik ter behand

  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...... the past 6 months. A regression model examined predictors of medical resource utilization. RESULTS: Overall, 164 patients had an attack in the past 6 months and were included in the analysis. The most significant predictor of medical resource utilization was the severity of the last attack (OR 2.6; p ....001). Among patients who sought medical care during the last attack (23%), more than half utilized the emergency department. The last attack prevented patients from their normal activities an average of 4-12 hours. Patient and caregiver absenteeism increased with attack severity and frequency. Among patients...

  2. The humanistic burden of hereditary angioedema

    DEFF Research Database (Denmark)

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

    2014-01-01

    extremities; 24% experienced an attack in more than one site. The impact of HAE on daily activities was high during attacks and did not vary significantly by body site affected; patients also reported that HAE impacted their daily activities between attacks. Patients reported substantial anxiety about future...... attacks, traveling, and passing HAE to their children. Based on Hospital Anxiety and Depression Scale scores, 38 and 14% had clinically meaningful anxiety and depression, respectively. Despite standard of care, HAE patients still have frequent and painful attacks. Patients experience substantial...

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

    DEFF Research Database (Denmark)

    Longhurst, Hilary; Bygum, Anette

    2016-01-01

    nature of the disorder, the lack of awareness of this ultra-rare condition amongst medical personnel, lack of specialist centers, and limited treatment options have contributed to under-diagnosis and under-treatment of the condition. Incorrect treatment of attacks has been common, even when medical help...

  4. 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...

  5. Management of urticaria and angioedema in children : new trends

    NARCIS (Netherlands)

    Oranje, A. P.

    2010-01-01

    Urticaria in childhood is a common problem. History of development of urticaria should be carefully taken from a written history/information list. For urticaria, the EAACI/GALEN/EDF consensus guidelines on definition, classification, diagnosis and management of urticaria should be considered. Soon a

  6. 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.

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

    DEFF Research Database (Denmark)

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

    2016-01-01

    of this study was to describe safety and usage patterns of pnfC1-INH. METHODS: A multicenter, observational, registry was conducted between 2010 and 2014 at 30 United States and 7 European sites to obtain both prospective (occurring after enrollment) and retrospective (occurring before enrollment) safety...... and usage data on subjects receiving pnfC1-INH for any reason. RESULTS: Of 343 enrolled patients, 318 received 1 or more doses of pnfC1-INH for HAE attacks (11,848 infusions) or for prophylaxis (3142 infusions), comprising the safety population. Median dosages per infusion were 10.8 IU/kg (attack treatment......) and 16.6 IU/kg (prophylaxis). Approximately 95% of infusions were administered outside of a health care setting. No adverse events (AEs) were reported in retrospective data. Among prospective data (n = 296 subjects; 9148 infusions), 252 AEs were reported in 85 (28.7%) subjects (rate of 0.03 events...

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

    DEFF Research Database (Denmark)

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

    2014-01-01

    , and it is speculated that C1-inh polymers may exist in patient plasma, challenging the current classification of HAE patients. To investigate the presence of C1-inh polymers in patient plasma samples, we developed an immunological method, where monoclonal antibodies produced against polymerized C1-inh were applied...... in native PAGE western blotting. Using this approach we analyzed genuine plasma samples from 31 Danish HAE families, and found that plasma samples from three genotypically distinct HAE type I families (classified upon C1-inh plasma concentrations) contained C1-inh polymers. Identical C1-inh polymerization....... Subjects suffering from HAE have been classified in type I patients with decreased functional and antigenic levels of C1-inh, and type II patients with decreased functional but normal antigenic C1-inh levels. However, a few reports have demonstrated that some mutations cause C1-inh polymerization in vitro...

  9. 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

    their own assays and standards. A double-blind survey was conducted using plasma/serum samples from healthy donors and HAE patients and the uniformity of HAE diagnosis was evaluated. It can be concluded that the diagnosis of fC1-Inh deficiency was made correctly in most cases in this survey. We can...... especially useful when a laboratory first starts to offer assays on fC1-Inh for HAE diagnosis. In the present study we evaluated the performance of fC1-Inh assays in the 15 different laboratories that are specialised in HAE diagnostics and assessed inter-laboratory variation with each laboratory using...

  10. 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...

  11. Galactose-α-1,3-galactose and Delayed Anaphylaxis, Angioedema, and Urticaria in Children

    Science.gov (United States)

    Kennedy, Joshua L.; Stallings, Amy P.; Platts-Mills, Thomas A.E.; Oliveira, Walter M.; Workman, Lisa; James, Haley R.; Tripathi, Anubha; Lane, Charles J.; Matos, Luis; Heymann, Peter W.

    2013-01-01

    BACKGROUND AND OBJECTIVE: Despite a thorough history and comprehensive testing, many children who present with recurrent symptoms consistent with allergic reactions elude diagnosis. Recent research has identified a novel cause for “idiopathic” allergic reactions; immunoglobulin E (IgE) antibody specific for the carbohydrate galactose-α-1,3-galactose (α-Gal) has been associated with delayed urticaria and anaphylaxis that occurs 3 to 6 hours after eating beef, pork, or lamb. We sought to determine whether IgE antibody to α-Gal was present in sera of pediatric patients who reported idiopathic anaphylaxis or urticaria. METHODS: Patients aged 4 to 17 were enrolled in an institutional review board–approved protocol at the University of Virginia and private practice allergy offices in Lynchburg, VA. Sera was obtained and analyzed by ImmunoCAP for total IgE and specific IgE to α-Gal, beef, pork, cat epithelium and dander, Fel d 1, dog dander, and milk. RESULTS: Forty-five pediatric patients were identified who had both clinical histories supporting delayed anaphylaxis or urticaria to mammalian meat and IgE antibody specific for α-Gal. In addition, most of these cases had a history of tick bites within the past year, which itched and persisted. CONCLUSIONS: A novel form of anaphylaxis and urticaria that occurs 3 to 6 hours after eating mammalian meat is not uncommon among children in our area. Identification of these cases may not be straightforward and diagnosis is best confirmed by specific testing, which should certainly be considered for children living in the area where the Lone Star tick is common. PMID:23569097

  12. 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

    )/Reseau Canadien d'angioedeme hereditaire (RCAH) (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...

  13. Hypersensitivity to aldesleukin (interleukin-2 and proleukin) presenting as facial angioedema and erythema.

    Science.gov (United States)

    Abraham, Daryn; McGrath, Kris G

    2003-01-01

    Aldesleukin is a human recombinant interleukin-2 product. It also is known as interlukin-2 and Proleukin in the United States. It is indicated for the treatment of adults with metastatic renal cell carcinoma as well as for adults with metastatic melanoma. However, its use has been limited because of severe systemic toxicity. There have been no reports of aldesleukin producing a hypersensitivity reaction. This is the first reported case of an immediate systemic hypersensitivity reaction occurring after aldesleukin administration confirmed by enzyme-linked immunosorbent assay for specific immunoglobulin E against aldesleukin. PMID:12974198

  14. Helicobacter pylori: A significant and treatable cause of chronic urticaria and angioedema.

    Science.gov (United States)

    Rasooly, Marjohn M; Moye, Nancy A; Kirshenbaum, Arnold S

    2015-10-16

    Two outpatient medical offices evaluated 204 patients with chronic urticaria during 2012. This article presents a retrospective study showing that 10% of patients with chronic urticaria may be infected with H. pylori. Furthermore, eradication of infection can be followed by remission of urticaria, reduced morbidity from gastric ulcers, and cancer.

  15. Disease: H01006 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H01006 Hereditary angioedema Hereditary angioedema (HAE) is a rare genetic disorder...l 41:114-22 (2011) PMID:19160940 (drug) Reshef A, Leibovich I, Goren A Hereditary angioedema: new hopes for

  16. A 47-year-old man with tongue swelling.

    Science.gov (United States)

    Rodríguez-Roa, Maristely; Nazario, Sylvette; Ramos, Cristina

    2016-07-01

    Intermittent tongue angioedema can be the initial presentation of several disorders including angiotensin-converting-enzyme inhibitor induced angioedema and hereditary angioedema. Persistent angioedema on the other hand, can be associated with amyloidosis, tumors, thyroid disorders and acromegaly. We present a case of intermittent episodes of tongue swelling progressing to macroglossia. PMID:27401321

  17. Icatibant er en ny behandlingsmulighed ved livstruende angiotensinkonverterende enzym-inhibitor-udløst angioødem

    DEFF Research Database (Denmark)

    Fast, Søren; Henningsen, Emil; Bygum, Anette

    2011-01-01

    A 78 year-old woman with life-threatening angiotensin-converting enzyme inhibitor (ACE-i) induced angioedema was unresponsive to conventional treatment with corticosteroids, antihistamines and epinephrine. She was successfully treated with icatibant licensed for treatment of hereditary angioedema...... knowing that both conditions involve bradykinin induced activation of bradykinin B2 receptors. Randomised, controlled trials are warranted to document the efficacy of icatibant in ACE-i angioedema....

  18. Hereditary angioderma: an uncommon cause of acute abdomen. Abdominal computed tomography and ultrasound findings; Angioedema hereditario: una causa infrecuente de abdomen agudo. Hallazgos en la TC e ecografia abdominal

    Energy Technology Data Exchange (ETDEWEB)

    Cruz, R.A. de la; Oliver, J. M.; Bueno, A.; Albillos, J. C. [Fundacion Hospital Alcorcon. Madrid (Spain)

    2002-07-01

    We present an uncommon case of acute abdomen in a patient with hereditary angioderma. The ultrasound and CT findings described may suggest this diagnosis, thus avoiding useless surgical interventions in patients in whom the disease has not been previously diagnosed. (Author) 19 refs.

  19. Drug: D04492 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available ATOLOGICAL AGENTS B06A OTHER HEMATOLOGICAL AGENTS B06AC Drugs used in hereditary angioedema B06AC02 Icatiban...t D04492 Icatibant acetate (USAN) USP drug classification [BR:br08302] Immunological Agents Angioedema

  20. 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....

  1. Serum sickness

    Science.gov (United States)

    ... the problem should be stopped. Avoid using that medicine or antiserum in the future. ... antiserum that caused serum sickness again in the future, your ... blood vessels Swelling of the face, arms, and legs ( angioedema )

  2. 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

  3. 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

  4. Genetics Home Reference: vibratory urticaria

    Science.gov (United States)

    ... Torsten]. Citation on PubMed Aloyouny A, Stoopler ET. Vibrational angioedema: considerations for oral health care providers. Spec ... Services National Institutes of Health National Library of Medicine Lister Hill National Center for Biomedical Communications 8600 ...

  5. Analysis on treating hereditary angioedema acute attack with fresh frozen plasma in 16 case-times%新鲜冰冻血浆治疗遗传性血管性水肿急性发作16例次分析

    Institute of Scientific and Technical Information of China (English)

    汤蕊; 陈适; 张宏誉

    2012-01-01

    目的 探讨新鲜冰冻血浆(FFP)治疗遗传性血管性水肿(HAE)的适应证、安全性和有效性.方法 回顾性分析北京协和医院4例次及文献中12例次共16例次FFP治疗HAE急性发作的病例.结果 共13例HAE患者急性期16次输注FFP,输注量平均(586±337)mL,输注后15例次病情得到改善,1例次因输血反应中断用药.症状开始缓解时间为(49±19)min,输注后完全缓解时间由输注前的(6.7±27.0)h缩短为(2.0±12.0)h.除2例次患者出现腹痛加重、1例次患者出现皮疹外,其他患者均未出现不良反应.结论 FFP用于HAE急性发作期治疗相对安全有效.%Objective To discuss the indication, safety and efficiency of FFP for the treatment of HAE. Methods Retrospective analyze the clinical data of 2 patients with HAE in Peking Union medical college hospital and 11 patients in the medical literatures. Results Thirteen cases of patients were injected with FFP totally 16 times, and the average dosage of FFP is ( 586 ± 337 ) mL. Only one patient failed to be cured because of transfusion reaction. The symptoms begin to ease ( 49 ± 19 ) minutes after injection. And the time of complete remission reduced from ( 61. 7 ± 27.0 ) hours to( 2. 0 ± 12.0 )hours after FFP injection. There were 2 cases of abdominal pain and 1 case of skin rash, and others have no adverse reaction. Conclusion FFP is safe and effective for acute attacks of HAE.

  6. Idiopathic anaphylaxis.

    Science.gov (United States)

    Greenberger, Paul A

    2007-05-01

    Idiopathic anaphylaxis is a prednisone-responsive condition without external cause, but it can coexist with food-, medication-, or exercise-induced anaphylaxis. Mast cell activation may occur at night or after foods that have been eaten with impunity many times previously. Idiopathic anaphylaxis can be classified into frequent (if there are six or more episodes per year or two episodes in the last 2 months) or infrequent (if episodes occur less often). Idiopathic anaphylaxis-generalized consists of urticaria or angioedema associated with severe respiratory distress, syncope or hypotension, and gastrointestinal symptoms. Idiopathic anaphylaxis-angioedema consists of massive tongue enlargement or severe pharyngeal or laryngeal swelling with urticaria or peripheral angioedema. The differential diagnosis of idiopathic anaphylaxis is reviewed, and treatment approaches are presented. PMID:17493503

  7. Angioneurotisk ødem i forbindelse med behandling med angiotensinkonverterende enzym-haemmer

    DEFF Research Database (Denmark)

    Johansen, E C; Johansen, J B; Døssing, H

    1996-01-01

    Angioneurotic oedema secondary to angiotensin-converting enzyme (ACE) inhibitors is a rare condition, but it is a side effect which is likely to be seen more frequently because of the increased use of these drugs in the treatment of heart failure and hypertension. We report two cases which...... illustrate problems in the diagnosis and management of this life-threatening condition, and also demonstrate that angioedema re-occurs if the ACE inhibitor is not discontinued. If angioedema is suspected, therapy with any angiotensin converting-enzyme inhibitor should be discontinued promptly, respiratory...

  8. 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.

  9. Adverse Reaction to Omalizumab in Patients with Chronic Urticaria: Flare Up or Ineffectiveness?

    Science.gov (United States)

    Ertaş, Ragıp; Özyurt, Kemal; Yıldız, Sinem; Ulaş, Yılmaz; Turasan, Abdullah; Avcı, Atıl

    2016-02-01

    Omalizumab is a recombinant humanized anti-Ig E monoclonal antibody used as the third line treatment of chronic spontaneous urticaria (CSU). We report four patients with severe antihistamine-resistant CSU, who developed angioedema, anaphylaxis and/or flare up of urticaria at different times following omalizumab therapy. PMID:26996116

  10. Disease: H00106 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available nd angioedema: molecular mechanisms and clinical progress. Trends Mol Med 15:69-78 (2009) PMID:18374984 (description, gene) Ponce-Cas...tro IM, Gonzalez-Rubio C, Delgado-Cervino EM, Abarrategui-Garrido C, Fontan G, Sanc

  11. Isolated oedema of the uvula induced by intense snoring and ACE inhibitor

    DEFF Research Database (Denmark)

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

    2014-01-01

    A case of snoring-induced angioedema of uvula is described in a patient who was treated with ACE inhibitor. The patient partially responded to complement C1-inhibitor concentrate and did not suffer any recurrences after the medication was withdrawn. When encountering a patient suffering from...

  12. Nomenclatuur en scoresystemen chronische urticaria

    NARCIS (Netherlands)

    Urgert, M. C.; Van Den Elzen, M. T.; Tupker, R. A.; Franken, S. M.; Van Zuuren, E. J.; Knulst, A. C.

    2015-01-01

    The Dutch Guideline on urticaria is currently being developed. Whilst awaiting the guideline, this article already discusses the classification and score system. Chronic urticaria is defined as the occurrence of spontaneous wheals, angioedema or both for a period of at least six weeks. In the curren

  13. Asthma and anaphylactoid reactions to food additives.

    OpenAIRE

    Tarlo, S. M.; Sussman, G L

    1993-01-01

    Presumed allergic reactions to hidden food additives are both controversial and important. Clinical manifestations include asthma, urticaria, angioedema, and anaphylactic-anaphylactoid events. Most adverse reactions are caused by just a few additives, such as sulfites and monosodium glutamate. Diagnosis is suspected from the history and confirmed by specific challenge. The treatment is specific avoidance.

  14. Drug: D03931 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available CTRD (Disulfide bridge: 7-57; 16-40; 32-53) Peptide Treatment of hereditary angioedema; Reduction of blood l...TOLOGICAL AGENTS B06A OTHER HEMATOLOGICAL AGENTS B06AC Drugs used in hereditary a

  15. 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.

  16. 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...

  17. Complement

    Science.gov (United States)

    ... may have lower-than-normal levels of the complement proteins C3 and C4 . Complement activity varies throughout the body. ... Elsevier Saunders; 2013:chap 8. Read More Cirrhosis Complement component 3 (C3) Complement component 4 Glomerulonephritis Hepatitis Hereditary angioedema Kidney ...

  18. Complement component 4

    Science.gov (United States)

    ... may have lower-than-normal levels of the complement proteins C3 and C4 . Complement activity varies throughout the body. ... Saunders; 2013:chap 6. Read More Cirrhosis Complement Complement component 3 (C3) Glomerulonephritis Hepatitis Hereditary angioedema Kidney transplant Lupus nephritis ...

  19. A not so healthy muesli: a case report.

    Science.gov (United States)

    Meysman, M; Schelfaut, D; Vincken, W

    2009-01-01

    We present a patient with severe anaphylaxis, angioedema, hypotension and shock. The near fatal allergic reaction was caused by eating yogurt with muesli containing pine nuts. The patient developed an acute infero-posterolateral myocardial infarction due to systemic hypotension and shock. Food allergy to pine nut was demonstrated by dosage of specific IgE to pine nut.

  20. Eradication of Blastocystis hominis prevents the development of symptomatic Hashimoto's thyroiditis: a case report.

    Science.gov (United States)

    Rajič, Borko; Arapović, Jurica; Raguž, Kazimir; Bošković, Mladen; Babić, Senaida Marina; Maslać, Suzana

    2015-07-01

    In this case report we describe a 49 year-old man who presented with chronic urticaria, angioedema and soft stool consistency. During diagnostic examinations Hashimoto's thyroiditis was found even though the patient never had clear symptoms of this disease. Blastocystis hominis was isolated through a stool microbiologic examination, implicating that this parasite can cause the development of Hashimoto's thyroiditis and chronic urticaria. After two-weeks treatment with metronidazole the Blastocystis hominis was eradicated, then urticaria and angioedema disappeared. During the four years of follow-up, the patient presented without any symptoms, whereas thyroid hormones were normalized and anti-thyroid antibodies declined. For the first time in the literature we show that eradication of Blastocystis hominis can prevent the development of both symptomatic Hashimoto's thyroiditis and chronic urticaria. PMID:26230132

  1. Anaphylaxis to annatto dye: a case report.

    Science.gov (United States)

    Nish, W A; Whisman, B A; Goetz, D W; Ramirez, D A

    1991-02-01

    Annatto dye is an orange-yellow food coloring extracted from the seeds of the tree Bixa orellana. It is commonly used in cheeses, snack foods, beverages, and cereals. Previously reported adverse reactions associated with annatto dye have included urticaria and angioedema. We present a patient who developed urticaria, angioedema, and severe hypotension within 20 minutes following ingestion of milk and Fiber One cereal, which contained annatto dye. Subsequent skin tests to milk, wheat, and corn were negative. The patient had a strong positive skin test to annatto dye, while controls had no response. The nondialyzable fraction of annatto dye on SDS-PAGE demonstrated two protein staining bands in the range of 50 kD. Immunoblotting demonstrated patient IgE-specific for one of these bands, while controls showed no binding. Annatto dye may contain contaminating or residual seed proteins to which our patient developed IgE hypersensitivity. Annatto dye is a potential rare cause of anaphylaxis. PMID:1994783

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

    OpenAIRE

    Mrinal Gupta; Mahajan, Vikram K.; Mehta, Karaninder S.; Pushpinder S Chauhan

    2015-01-01

    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 Ind...

  3. A case of labio-facial necrotizing fasciitis complicating acne

    OpenAIRE

    Eltayeb, Amel Salah; Ahmad, Abdelnasir Gafar; Elbeshir, Elnour Ibrahim

    2016-01-01

    Background Facial necrotizing fasciitis is extremely rare. Most of the cases reported in literature are related to dental, sinus, tonsillar and salivary glands causes, but rarely as consequence of skin infection. We report a unique case of lower lip cellulitis, which was initially misdiagnosed as angioedema and subsequently progressed into lower lip necrotizing fasciitis. Case presentation This is a case report of necrotizing fasciitis involving the lower lip as a consequence of infected skin...

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

    OpenAIRE

    Damla TUNCER-BUDANUR; Murat Cengizhan YAŞ; 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 w...

  5. 420 Lentil Allergy: First Report from Venezuela

    OpenAIRE

    Albarran, Carlos; Hulett, Arnaldo Capriles

    2012-01-01

    Background Allergy to lentils is infrequent in Latin America: this a first case report from Venezuela. A 5 year old female preschooler attended our allergology clinic with chief complaint of generalized giant urticaria inmediately after ingestion of cooked lentils; clinical history revealed frequent (>3) emergency visits, since the age of one year, with facial angioedema and generalized urticaria even from inhalation of vapors while cooking of lentils at home; moreover, also symptoms describe...

  6. Anaphylaxis to husband's seminal plasma and treatment by local desensitization

    OpenAIRE

    Park Chul; Huh Jung-Sik; Chung Young-Bae; Kim Miok; Kim Sohyung; Lee Jaechun; Lee Keun; Kim Jeong

    2008-01-01

    Abstract Hypersensitivity to human seminal fluid is rare but can be life threatening. We report a case of IgE-mediated anaphylaxis to seminal plasma that was diagnosed by skin prick tests and successfully treated by local desensitization. A 32-year-old woman suffering from angioedema and hypotension after exposure to semen was treated with epinephrine upon admission. Skin prick tests and immunoblotting for IgE binding components showed that she was sensitized to her husband's seminal plasma. ...

  7. Accompanying conditions in patients with chronic spontaneous urticaria and urticarial vasculitis: Results of a retrospective study

    OpenAIRE

    Sevgi Akarsu; Turna İlknur; Özlem Özbağçıvan; Emel Fetil

    2015-01-01

    Background and Design: Chronic spontaneous urticaria (CSU), the most common form of chronic urticaria, is characterized by spontaneous wheals and/or angioedema lasting longer than six weeks. Urticarial vasculitis (UV) is a small vessel vasculitis; but is also included in the various classification systems of chronic urticaria by some authors. The aim of our study was to evaluate the frequency of accompanying conditions, and to compare the demographic, clinical and laboratory features of patie...

  8. Effector Cells and Mechanisms in Chronic Spontaneous Urticaria

    OpenAIRE

    Borzova, Elena

    2014-01-01

    Background: Chronic spontaneous urticaria (CSU) is characterised by weals, angioedema, or both, which occur for six weeks or more. Itchy, red and raised weals in CSU are thought to occur as a result of skin mast cell activation, local vasodilatation and increased vascular permeability which are the cardinal features of the disease. Serum histamine-releasing activity and abnormal basophil function were implicated in the pathophysiology of CSU. We hypothesized that severe and/or persistent CSU ...

  9. A RARE CASE OF CEFTRIAXONE INDUCED CORONARY SPASM WITH ACUTE MYOCARDIAL ISCHEMIA

    OpenAIRE

    Basavaraj; Ravikumar; Sandeep; Shreyas

    2014-01-01

    : Kounis syndrome has been known as allergic angina and/or allergic myocardial infarction. Allergic insults usually may include drugs, latex, and food. Although ceftriaxone administration has been associated with various allergic reactions such as urticaria, angioedema, erythema, rash and anaphylactic shock, there are very few published report that has shown an association between ceftriaxone use and Kounis syndrome. Here, we describe a case report of allergic vasospasm, l...

  10. 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.

  11. Blood cells and endothelial barrier function

    OpenAIRE

    Rodrigues, Stephen F.; Granger, D Neil

    2015-01-01

    The barrier properties of endothelial cells are critical for the maintenance of water and protein balance between the intravascular and extravascular compartments. An impairment of endothelial barrier function has been implicated in the genesis and/or progression of a variety of pathological conditions, including pulmonary edema, ischemic stroke, neurodegenerative disorders, angioedema, sepsis and cancer. The altered barrier function in these conditions is often linked to the release of solub...

  12. Adverse reactions to acetylcysteine and effects of overdose.

    OpenAIRE

    Mant, T. G.; Tempowski, J H; Volans, G N; Talbot, J. C.

    1984-01-01

    Since the introduction in 1979 of intravenous acetylcysteine (Parvolex) as an antidote for overdosage of paracetamol the National Poisons Information Service and the manufacturer have been notified of 38 adverse reactions that were anaphylactoid in nature and 19 accidental overdoses. The most common feature of the anaphylactoid reaction to normal dosage was rash; other features reported included angioedema, hypotension, and bronchospasm; all the patients recovered. The features associated wit...

  13. Tako-tsubo-like cardiomyopathy after EpiPen administration.

    Science.gov (United States)

    Zubrinich, C M; Farouque, H M Omar; Rochford, S E; Sutherland, M F

    2008-11-01

    Tako-tsubo-like cardiomyopathy is characterized by acute chest pain, electrocardiographic changes and increased cardiac enzymes in the absence of obstructive coronary vessel disease. We describe the development of tako-tsubo-like cardiomyopathy in an elderly woman after the use of an EpiPen for generalized urticaria and angioedema. As adrenaline may participate in the pathogenesis of this condition, the need for careful patient selection and education in the use of adrenaline self-injectors remains imperative.

  14. Pattern of drug eruptions in a tertiary care hospital

    International Nuclear Information System (INIS)

    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)

  15. Relationship between AQP4 expression and structural damage to the blood-brain barrier at early stages of traumatic brain injury in rats

    Institute of Scientific and Technical Information of China (English)

    LU Hong; LEI Xiao-yan; HU Hui; HE Zhan-ping

    2013-01-01

    Background Although some studies have reported that aquaporin-4 (AQP4) plays an important role in the brain edema after traumatic brain injury (TBI),little is known about the AQP4 expression in the early stage of TBI,or about the correlation between the structural damage to the blood-brain barrier (BBB) and angioedema.The aim of this project was to investigate the relationship between AQP4 expression and damage to the BBB at early stages of TBI.Methods One hundred and twenty healthy adult Wistar rats were randomly divided into two greups:sham operation group (SO) and TBI group.The TBI group was divided into five sub-groups according to the different time intervals:1,3,6,12,and 24 hours.The brains of the animals were taken out at different time points after TBI to measure brain water content.The cerebral edema and BBB changes in structure were examined with an optical microscopy (OM) and transmission electron microscopy (TEM),and the IgG content and AQP4 protein expression in traumatic brain tissue were determined by means of immunohistochemistry and Western blotting.The data were analyzed with SPSS 13.0statistical software.Results In the SO greup,tissue was negative for IgG,and there were no abnormalities in brain water content or AQP4 expression.In the TBI group,brain water content significantly increased at 6 hours and peaked at 24 hours following injury.IgG expression significantly increased from 1 to 6 hours following injury,and remained at a high level at 24 hours.Pathological observation revealed BBB damage at 1 hour following injury.Angioedema appeared at 1 hour,was gradually aggravated,and became obvious at 6 hours.Intracellular edema occurred at 3 hours,with the presence of large glial cell bodies and mitochondrial swelling.These phenomena were aggravated with time and became obvious at 12 hours.In addition,microglial proliferation was visible at 24 hours.AQP4 protein expression were reduced at 1 hour,lowest at 6 hours,and began to increase at 12 hours

  16. 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

  17. 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

  18. Kinins, airway obstruction, and anaphylaxis.

    Science.gov (United States)

    Kaplan, Allen P

    2010-01-01

    Anaphylaxis is a term that implies symptoms that are present in many organs, some of which are potentially fatal. The pathogenic process can either be IgE-dependent or non-IgE-dependent; the latter circumstance may be referred to as anaphylactoid. Bradykinin is frequently responsible for the manifestations of IgE-independent reactions. Blood levels may increase because of overproduction; diseases such as the various forms of C1 inhibitor deficiency (hereditary or acquired) or hereditary angioedema with normal C1 inhibitor are examples in this category. Blood levels may also increase because of an abnormality in bradykinin metabolism; the angioedema due to ACE inhibitors is a commonly encountered example. Angioedema due to bradykinin has the potential to cause airway obstruction and asphyxia as well as severe gastrointestinal symptoms simulating an acute abdomen. Formation of bradykinin in plasma is a result of a complex interaction among proteins such as factor XII, prekallikrein, and high molecular weight kininogen (HK) resulting in HK cleavage and liberation of bradykinin. These proteins also assemble along the surface of endothelial cells via zinc-dependent interactions with gC1qR, cytokeratin 1, and u-PAR. Endothelial cell expression (or secretion) of heat-shock protein 90 or prolylcarboxypeptidase can activate the prekallikrein-HK complex to generate bradykinin in the absence of factor XII, however factor XII is then secondarily activated by the kallikrein that results. Bradykinin is destroyed by carboxypeptidase N and angiotensin-converting enzyme. The hypotension associated with IgE-dependent anaphylaxis maybe mediated, in part, by massive proteolytic digestion of HK by kallikreins (tissue or plasma-derived) or other cell-derived kininogenases. PMID:20519882

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

    Directory of Open Access Journals (Sweden)

    Ervin Ç. Mingomataj

    2009-05-01

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

  20. 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...

  1. Urticaria and anaphilaxis in a child after inhalation of lentils vapours: a case report and literature review

    OpenAIRE

    Vitaliti Giovanna; Morselli Ignazio; Di Stefano Valeria; Lanzafame Angela; La Rosa Mario; Leonardi Salvatore

    2012-01-01

    Abstract Background Among legumes, lentils seem to be the most common legume implicated in pediatric allergic reactions in the Mediterranean area and India, and usually they start early in life, below 4 years of age. Case report A 22 -month-old child was admitted to our Pediatric Department for anaphylaxis and urticaria. At the age of 9 months she presented a first episode of angioedema and laryngeal obstruction, due to a second assumption of lentils in her diet. At admission we performed rou...

  2. Anaphylaxis to husband's seminal plasma and treatment by local desensitization

    Directory of Open Access Journals (Sweden)

    Park Chul

    2008-12-01

    Full Text Available Abstract Hypersensitivity to human seminal fluid is rare but can be life threatening. We report a case of IgE-mediated anaphylaxis to seminal plasma that was diagnosed by skin prick tests and successfully treated by local desensitization. A 32-year-old woman suffering from angioedema and hypotension after exposure to semen was treated with epinephrine upon admission. Skin prick tests and immunoblotting for IgE binding components showed that she was sensitized to her husband's seminal plasma. Local desensitization, which persisted for six months, was achieved by intravaginal administration of serial dilutions of her husband's seminal plasma.

  3. Cold-induced anaphylaxis - case report

    OpenAIRE

    Gomes, S; Viegas, V.; Pinheiro, A.; Gaspar, A.

    2010-01-01

    Introdução: A urticária ao frio, rara em idade pediátrica, caracteriza-se pela ocorrência de urticária e/ou angioedema após exposição ao frio. Na maioria dos casos é idiopática; causas secundárias incluem crioglobulinémia, défices do complemento, vasculites, neoplasias e doenças infecciosas. Habitualmente é benigna e auto-limitada, no entanto reacções sistémicas potencialmente fatais podem ocorrer. Caso Clínico: Os autores apresentam o caso de um adolescent...

  4. Urticaria Vasculitis: Estudio retrospectivo de 20 casos y protocolo diagn??stico-terap??utico

    OpenAIRE

    Soriano Hern??ndez, Isabel; Orgaz Molina, Jacinto; Arias Santiago, Salvador; El-Ahmed, Husein; Ortego Centeno, Norberto; Callejas, Jos?? L.; Fern??ndez Pugnaire, M. Antonia; Naranjo Sintes, Ram??n Jos??

    2011-01-01

    La urticaria vasculitis es una enfermedad cr??nica que se caracteriza por episodios urticariales o de angioedema que puede asociarse a niveles bajos de complemento. Hemos realizado un estudio descriptivo retrospectivo que incluye a 20 pacientes con urticaria vasculitis diagnosticados en los ??ltimos 5 a??os donde analizamos los s??ntomas m??s frecuentes y los tratamientos que se han empleado. A continuaci??n se ha dise??ado un protocolo diagn??stico-terap??utico de Urticaria Vascu...

  5. A case of precocious emphysema and lung cancer in a woman with a history of hypocomplementemic urticarial vasculitis.

    Science.gov (United States)

    Jamison, Suzanna C; Brierre, Stephen; Sweet, Jon; de Boisblanc, Ben

    2008-03-01

    Severe emphysema developed in a white woman with a 26-pack-year history of tobacco use. Serum alpha(1)-antitrypsin levels were normal. A history of autoimmune hemolytic anemia, angioedema, low complement, and recurrent urticaria prompted an immunologic workup that ultimately led to a diagnosis of hypocomplementemic urticarial vasculitis syndrome. Treatment with oral prednisone and inhaled bronchodilators improved symptoms, but 4 months after diagnosis non-small cell lung cancer was discovered and she ultimately died. Hypocomplementemic urticarial vasculitis is an uncommon cause of precocious emphysema and has not previously been reported in a patient with bronchogenic carcinoma. PMID:18321906

  6. The EAACI/GA(2) LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria

    DEFF Research Database (Denmark)

    Zuberbier, T; Aberer, W; Asero, R;

    2014-01-01

    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...

  7. Hair dyes and temporary tattoos are a real hazard for adolescents?

    Science.gov (United States)

    Haluk Akar, H; Adatepe, S; Tahan, F; Solmaz, I

    2014-01-01

    Temporary tattoos, especially those that contain black dyes, have become rampant among teenagers in recent years. Most of these tattoos, in addition to hair dyes include paraphenylenediamine (PPD). PPD is a well-known skin sensitizer, which causes allergic contact dermatitis. Allergic contact dermatitis skin lesions from PPD are mostly seen as erythema multiforme-like eruption, a bullous contact dermatitis or as an exudative erythema. Herein, we report on our finding on a 15 year-old adolescent female who had been unaware of being previously sensitized to PPD from a black henna tattoo, and angioedema-like reaction which occurred after her first exposure to hair dye.

  8. [Occupational immediate allergy to latex. I. Introduction, epidemiology and clinical findings].

    Science.gov (United States)

    Pałczyński, C; Walusiak, J

    1997-01-01

    For the 10 recent years the incidence of natural rubber latex (NRL) immediate allergy has dramatically increased. Allergy to latex is observed mostly among persons who regularly use rubber devices (particularly rubber gloves) or their mucous membrane is exposed to frequent contact with rubber products. The health service and the rubber industry workers are at the highest risk of latex allergy (10%). Urticaria, angioedema, bronchial asthma, conjunctivitis, rhinitis and anaphylactic shock are most common symptoms of allergy to latex. Severe systemic reactions are frequently first clinical manifestations of latex hypersensitivity. PMID:9312658

  9. Laboratory tests for disorders of complement and complement regulatory proteins.

    Science.gov (United States)

    Shih, Angela R; Murali, Mandakolathur R

    2015-12-01

    The complement pathway is a cascade of proteases that is involved in immune surveillance and innate immunity, as well as adaptive immunity. Dysfunction of the complement cascade may be mediated by aberrations in the pathways of activation, complement regulatory proteins, or complement deficiencies, and has been linked to a number of hematologic disorders, including paroxysmal noctural hemoglobinuria (PNH), hereditary angioedema (HAE), and atypical hemolytic-uremic syndrome (aHUS). Here, current laboratory tests for disorders of the complement pathway are reviewed, and their utility and limitations in hematologic disorders and systemic diseases are discussed. Current therapeutic advances targeting the complement pathway in treatment of complement-mediated hematologic disorders are also reviewed.

  10. C1-inhibitor polymers activate the FXII-dependent kallikrein-kinin system

    DEFF Research Database (Denmark)

    Elenius Madsen, Daniel; Sidelmann, Johannes Jakobsen; Biltoft, Daniel;

    2015-01-01

    BACKGROUND: The FXII-dependent kallikrein-kinin system (KKS) is tightly regulated by the serine protease inhibitor (serpin) C1-inhibitor (C1-inh). When regulation of the FXII-dependent KKS fails, which is the case in hereditary angioedema (HAE), patients consequently experience invalidating edema......-inh polymers in vivo. CONCLUSION: Polymerization of the C1-inh transforms the major inhibitor of the FXII-dependent KKS, into a potent activator of the very same system. GENERAL SIGNIFICANCE: The C1-inh polymers might play a role in the pathophysiology of HAE, but several diseases are characterized...

  11. 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.

  12. Clinical Evaluation of Inpatients with Acute Urticaria

    Directory of Open Access Journals (Sweden)

    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

  13. [Inducible urticaria and chronic spontaneous urticaria].

    Science.gov (United States)

    Du Thanh, A

    2014-11-01

    In the recently published 2013 revision of the guidelines of urticaria, chronic urticaria (CU) gathers chronic spontaneous urticaria (CSU) and inducible urticaria (IU), and excludes pseudourticarial rashes with more than 24h-lasting rash or more than 72h-lasting angiœdema. Activity and psychosocial impact of the disease must be measured with validated scores such as Urticaria and Angioedema Activity Scores, Urticaria Control Test, CU-Q2OL, AE-QOL. Although an allergic cause is generaly absent in CU, pathomecanisms remain elusive even since the well-known role of mast cell degranulation and the presence of autoantibodies anti-FcRεI or anti-IgE. Coagulation pathways may be involved, at least as an amplifying phenomenon. Mean duration of CU is 1 to 4 years, but many patients still have symptoms after 10 years, some predictive factors being known as severity, angioedema, a positive autologous serum test, inducible urticaria. Recommended routine diagnosic tests are validated provocation tests for IU (and cryoproteins for cold urticaria), blood cell count and CRP for CSU, since a thorough history and a normal detailed physical examination should avoid unnecessary tests. Management of CU has been improved by the off-label use of increased dosages of second generation anti- H1 antihistamines, but a subsequent therapeutic intensification may be necessary in some cases. Educational program may prevent this intensification. Independent studies evaluating available molecules are needed, along with more fundamental research studies.

  14. Clinicoepidemiologic features of chronic urticaria in patients having positive versus negative autologous serum skin test: A study of 100 Indian patients

    Directory of Open Access Journals (Sweden)

    Surbhi Vohra

    2011-01-01

    Full Text Available Background: Chronic urticaria patients who demonstrate autoantibodies against the high-affinity receptor of IgE (FceRI or IgE itself tend to have a high itch and wheal score, and systemic symptoms may have a significant bearing on their management in terms of super pharmacologic doses of antihistamines needed or use of immunomodulators. Most studies have used histamine release assays rather than autologous serum skin tests (ASSTs for correlating urticaria severity and histamine releasing activity. Methods: An ASST was performed in 100 (M:F, 31:69 chronic urticaria patients aged between 14 and 63 (mean, 32.69 ± 13 years with an objective to study the clinicoepidemiologic features like age, sex, age of onset and duration, frequency and distribution of wheals, urticaria severity, angioedema and systemic manifestations in ASST-positive and ASST-negative patients. Results: ASST was positive in 46% of the patients and negative in 54% of the patients, respectively. Both groups showed no statistically significant difference for epidemiological details. However, the ASST-positive patients had a higher mean urticaria activity score, frequent involvement of more body sites, particularly palms and soles, presence of throat angioedema and general constitutional, respiratory or gastrointestinal symptoms in comparison with the ASST-negative patients. Conclusions: Apparently, ASST-positive patients have more severe clinical manifestations of chronic urticaria. The knowledge will be useful for the treating dermatologists and patients alike in view of its therapeutic implications.

  15. Unusual urticarias.

    Science.gov (United States)

    Black, A K

    2001-11-01

    Typical urticarial lesions are transient cutaneous swellings of sudden onset, often itchy, persisting for less than 24 hours and resolving to leave normal appearing skin. Angioedema lesions are similar subcutaneous lesions. Atypical urticarias persist for longer than 24 hours, may be painful and bruised in appearance and accompanied with severe systemic symptoms. Conditions where prolonged weals are present include delayed pressure urticaria and urticarial vasculitis. These conditions do not respond well to antihistamine therapy. In delayed pressure urticaria, weals appear after a delay of hours at sites of sustained pressure on the skin and occur in association with ordinary chronic 'idiopathic' urticaria. Weals of urticarial vasculitis show histological features of venulitis, and can be accompanied by arthralgia and abdominal pain. Rarely, the condition is due to infective or autoimmune disease. Urticarial diseases, sometimes with features of urticarial vasculitis, and with associated systemic features include Schnitzler's Syndrome, Still's disease and Muckle-Wells syndrome. The latter syndrome is linked with chromosome 1q44, as is autosomal dominant cold urticaria, an unusual physical urticaria. Persistent cholinergic erythema, a variant of cholinergic urticaria, has been mistaken for a drug eruption or cutaneous mastocytosis. Rarely, food and exercise induced urticaria and anaphylaxis occur when exercise follows a specific food or any meal within a few hours. The early stages of inflammatory disease may be mistaken for urticaria and angioedema, but lesions usually persist for longer than 48 hours and are accompanied by epidermal changes. PMID:11770721

  16. Paraphenylenediamine Containing Hair Dye: An Emerging Household Poisoning.

    Science.gov (United States)

    Patra, Ambika Prasad; Shaha, Kusa Kumar; Rayamane, Anand P; Dash, Shreemanta Kumar; Mohanty, Manoj Kumar; Mohanty, Sachidananda

    2015-09-01

    Paraphenylenediamine poisoning is among one of the emerging causes of poisoning in Asian countries, because it is a constituent of hair dye formulations and is easily available in market at low cost. Hair dyes are rampantly used in Asian households compared with the western world. Locally, hair dye constituents may have allergic adverse effects, and acute systemic poisoning presents with characteristic angioedema, upper airway obstruction, rhabdomyolysis, methemoglobinemia, myoglobinuria, and acute renal failure. This study reports about the death of a 24-year-old Indian housewife who committed suicide by taking hair dye emulsion. She had an argument with her husband, and because of fit of rage, took a bowlful (80 mL) of hair dye emulsion kept prepared for the use by husband. She developed angioedema, cervical swelling, and rhabdomyolysis and died of acute renal failure within 24 hours. Toxicological analysis of viscera and blood revealed varying levels of paraphenylenediamine. Histopathological samples of kidney showed features of acute tubular necrosis and myoglobin casts in renal tubules. The aim of the study is to create awareness about the adverse effects of the hair dye, its poisoning outcome, and possible preventive measures. PMID:26056768

  17. Itch in Urticaria Management.

    Science.gov (United States)

    Deza, Gustavo; Giménez-Arnau, Ana M

    2016-01-01

    Urticaria is a common skin disorder defined by the occurrence of itchy and even painful wheals, angioedema, or both. The lifetime prevalence for its acute and chronic form is 20 and 1%, respectively. The patients' quality of life is impaired because of itch, disfigurement, and high associated comorbidity. To understand the pathophysiology of the wheal in order to ensure a correct therapeutic approach is critical. Mast cells are the primary effector cells in urticaria, which produce and secrete a variety of inflammatory mediators, mainly histamine. Their peripheral effects are responsible for the signs and symptoms of the disease, such as cutaneous swelling and pruritus. Management of itch in urticaria includes both nonpharmacological (avoidance or minimization of aggravating factors) and pharmacological treatments. The main therapeutic objective is to obtain complete relief of signs (hives and angioedema) and symptoms (pruritus) as quickly as possible. Licensed and up-dosed nonsedating H1-antihistamines are currently the first- and second-line therapies according to the European guidelines. When antihistamines are not enough, other treatments include anti-IgE antibodies, mast cell modulators, mast cell mediator blockers, and immunomodulators. As the knowledge of the pathogenesis of urticaria improves, the development of alternative therapies targeting these pathways may improve the patient's quality of life through the control of the pruritus, its main symptom. PMID:27578075

  18. IgE antibodies against snake venoms.

    Science.gov (United States)

    Alonso, A; Scavini, L M; Marino, G A; Rodríguez, S M

    1995-01-01

    A similar event was detected in the clinical records of a small group of atopic patients living in the northern provinces of Argentina, i.e., they were bitten by a snake of the Bothrops species (or yarará) during their rural activities (woodcutters, cattle-drivers and farmers). Those who were bitten twice suffered an acute episode of hives and angioedema within 15 minutes after the snake bite. The presence of specific antibodies against Bothrops alternata (Ba) extract was detected by means of RAST for IgE and Ouchterlony and Boyden for IgG. The Ouchterlony also demonstrated crossreactivity among the venoms of the Bothrops species and the positivity of the six fractions obtained by DEAE-cellulose column fractionation against the horse anti-Ba serum. The Ba antigen induced a definite inhibition of the RAST. We presume that hives and angioedema in atopic patients immediately after a second snake bite could be attributed to the presence of a specific IgE antibody against the venom, and must not be misinterpreted with the toxic effects that appear later. PMID:7551202

  19. Therapeutic imaging window of cerebral infarction revealed by multisequence magnetic resonance imaging An animal and clinical study

    Institute of Scientific and Technical Information of China (English)

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

    2012-01-01

    In this study, we established a Wistar rat model of right middle cerebral artery occlusion and observed pathological imaging changes (T2-weighted imaging [T2WI], T2FLAIR, and diffusion-weighted imaging [DWI]) following cerebral infarction. The pathological changes were divided into three phases: early cerebral infarction, middle cerebral infarction, and late cerebral infarction. In the early cerebral infarction phase (less than 2 hours post-infarction), there was evidence of intracellular edema, which improved after reperfusion. This improvement was defined as the ischemic penumbra. In this phase, a high DWI signal and a low apparent diffusion coefficient were observed in the right basal ganglia region. By contrast, there were no abnormal T2WI and T2FLAIR signals. For the middle cerebral infarction phase (2–4 hours post-infarction), a mixed edema was observed. After reperfusion, there was a mild improvement in cell edema, while the angioedema became more serious. A high DWI signal and a low apparent diffusion coefficient signal were observed, and some rats showed high T2WI and T2FLAIR signals. For the late cerebral infarction phase (4–6 hours post-infarction), significant angioedema was visible in the infarction site. After reperfusion, there was a significant increase in angioedema, while there was evidence of hemorrhage and necrosis. A mixed signal was observed on DWI, while a high apparent diffusion coefficient signal, a high T2WI signal, and a high T2FLAIR signal were also observed. All 86 cerebral infarction patients were subjected to T2WI, T2FLAIR, and DWI. MRI results of clinic data similar to the early infarction phase of animal experiments were found in 51 patients, for which 10 patients (10/51) had an onset time greater than 6 hours. A total of 35 patients had MRI results similar to the middle and late infarction phase of animal experiments, of which eight patients (8/35) had an onset time less than 6 hours. These data suggest that defining the

  20. Severe anaphylactic reaction to bovine serum albumin at the first attempt of artificial insemination.

    Science.gov (United States)

    Wüthrich, B; Stern, A; Johansson, S G

    1995-02-01

    A 33-year-old woman without history of previous atopic diseases or drug allergies developed a severe anaphylactic reaction with asthma, vomiting, itching, generalized urticaria, and angioedema during artificial insemination with her husband's sperm. The sperm-processing medium contained bovine serum albumin (BSA). Skin prick test and RAST demonstrated an IgE-mediated hypersensitivity to BSA as well as a polyvalent atopic sensitization to pollens, animal danders, cow's milk, beef, pork, and mutton. SDS-PAGE studies indicated serum albumin to be the appropriate allergen with a high degree of cross-reactivity between serum albumin from different animal species. Artificial insemination with fluid containing potential allergens can, therefore, represent an unnecessary risk for atopic females, even in the absence of prior clinical symptoms of allergic diseases. Preoperative testing with the medium is recommended. PMID:7604943

  1. Applying complement therapeutics to rare diseases.

    Science.gov (United States)

    Reis, Edimara S; Mastellos, Dimitrios C; Yancopoulou, Despina; Risitano, Antonio M; Ricklin, Daniel; Lambris, John D

    2015-12-01

    Around 350 million people worldwide suffer from rare diseases. These may have a genetic, infectious, or autoimmune basis, and several include an inflammatory component. Launching of effective treatments can be very challenging when there is a low disease prevalence and limited scientific insights into the disease mechanisms. As a key trigger of inflammatory processes, complement has been associated with a variety of diseases and has become an attractive therapeutic target for conditions involving inflammation. In view of the clinical experience acquired with drugs licensed for the treatment of rare diseases such as hereditary angioedema and paroxysmal nocturnal hemoglobinuria, growing evidence supports the safety and efficacy of complement therapeutics in restoring immune balance and preventing aggravation of clinical outcomes. This review provides an overview of the candidates currently in the pharmaceutical pipeline with potential to treat orphan diseases and discusses the molecular mechanisms triggered by complement involved with the disease pathogenesis.

  2. A subtle mimicker in emergency department

    Science.gov (United States)

    Angelis, Maria Vittoria De; Giacomo, Roberta Di; Muzio, Antonio Di; Onofrj, Marco; Bonanni, Laura

    2016-01-01

    Abstract Background: Movement disorder emergencies include any movement disorder which develops over hours to days, in which failure to appropriately diagnose and manage can result in patient morbidity or mortality. Movement disorder emergencies include acute dystonia: sustained or intermittent muscle contractions causing abnormal, often repetitive, movements. Acute dystonia is a serious challenge for emergency room doctors and neurologists, because of the high probability of misdiagnosis, due to the presence of several mimickers including partial seizures, meningitis, localized tetanus, serum electrolyte level abnormalities, strychnine poisoning, angioedema, malingering, catatonia, and conversion. Methods: We describe 2 examples, accompanied by videos, of acute drug-induced oro-mandibular dystonia, both subsequent to occasional haloperidol intake. Results: Management and treatment of this movement disorder are often difficult: neuroleptics withdrawal, treatment with benzodiazepines, and anticholinergics are recommended. Conclusion: Alternative treatment options are also discussed. PMID:27741141

  3. [Angiotensin-receptor- and neprilysin-inhibition: a new option against heart failure].

    Science.gov (United States)

    Bruhn, Claudia

    2016-01-01

    The molecular combination of sacubitril and valsartan (Entresto) is a new drug for reducing the risk of cardiovascular death and hospitalization for heart failure in patients with chronic heart failure (NYHA Class II-IV) and reduced ejection fraction. It is usually administered in conjunction with other heart failure therapies, instead of an ACE inhibitor or an angiotensin-receptor blocker (ARB). In studies, sacubitril/ valsartan was superior to enalapril in reducing the risks of death and hospitalization for heart failure. Possible side effects of sacubitril/valsartan are hypotension, angioedema, impaired renal function and elevation in serum potassium levels. The drug should not be used in times of pregnancy and breast feeding, in patients with servere hepatic impairment (Child-Pugh C) and in combination with aliskiren in patients with diabetes. PMID:26975167

  4. [Biological roles of complement and recent topics in clinical medicine].

    Science.gov (United States)

    Wakamiya, Nobutaka

    2012-08-01

    The complement has been identified as a complementation factor to compensate for the function of an antibody. The complement consists of C1-C9, a complement-related molecule, and its regulating molecules. Three major biological roles of the complement have been classified: First: opsonization following phagocytosis and the elimination of microbes; second: direct destruction of bacteria due to membrane attack complex (MAC); third: complement activation following the induction of anaphylactoid factors and local recruitment and activation of neutrophilic leukocytes. In this review, the basic findings and recent treatments of paroxysmal nocturnal hemoglobinuria (PNH) and hereditary angioedema (HAE) are summarized. Finally, there is a short review of a rare autosomal recessive disorder of 3MC syndrome and new biological functions of complement factors except for that of innate immunity are proposed.

  5. 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 

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

    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.

  7. Aspirin Sensitivity and Chronic Rhinosinusitis with Polyps: A Fatal Combination

    Directory of Open Access Journals (Sweden)

    Hendrik Graefe

    2012-01-01

    Full Text Available Aspirin-exacerbated respiratory disease (AERD refers to aspirin sensitivity, chronic rhinosinusitis (CRS, nasal polyposis, asthma, eosinophil inflammation in the upper and lower airways, urticaria, angioedema, and anaphylaxis following the ingestion of NSAIDs. Epidemiologic and pathophysiological links between these diseases are established. The precise pathogenesis remains less defined, even though there is some progress in the understanding of several molecular mechanisms. Nevertheless, these combinations of diseases in patients classified by AERD constitute a fatal combination and may be difficult to treat with standard medical and surgical interventions. This paper reviews in brief the epidemiology, clinical features, diagnosis, molecular pathogenesis, and specific therapies of patients classified by AERD and postulates future attempts to gain new insights into this disease.

  8. Angioimmunoblastic T Cell Lymphoma Mimicking Chronic Urticaria

    Directory of Open Access Journals (Sweden)

    Mohleen Kang

    2016-01-01

    Full Text Available Angioimmunoblastic T cell lymphoma (AITL is a rare but distinct type of T cell lymphoma with an aggressive course and high mortality. Most patients are diagnosed late in the disease and usually present with generalized lymphadenopathy. A minority have skin lesions at the time of diagnosis, more commonly in the form of nonspecific maculopapular rash with or without pruritus. We report a rare case of AITL presenting with chronic, recurrent angioedema and urticaria-like lesions and no palpable peripheral adenopathy. Primary Care physicians, dermatologists, and allergists must maintain a high index of suspicion for cutaneous manifestations of lymphoma, especially if the skin lesions are refractory to standard treatment. Timely diagnosis is essential to improve survival.

  9. 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.

  10. Inducible urticaria: Case series and literature review.

    Science.gov (United States)

    Amaya, Daniel; Sánchez, Andrés; Sánchez, Jorge

    2016-01-01

    Inducible urticaria is a heterogeneous group of skin disorders characterized by the appearance of wheals, pruritus and/or angioedema, sometimes accompanied by systemic symptoms caused by innocuous stimuli (cold, heat, pressure, etc.). This group of disorders compromises people's quality of life and most of the literature in this regard comes from case reports and case series since its epidemiology has been poorly studied and some cases are very rare. The aim of this review is to show an up-to-date overview of the available literature for various types of inducible urticarias, always beginning with an illustrative case and then describing their pathophysiological mechanisms, clinical manifestations, and treatment. PMID:27622434

  11. 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...... 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...... occupational IgE-mediated chlorhexidine allergy. AIMS: To identify cases of chlorhexidine allergy among health care workers. METHODS: A questionnaire was distributed among HCWs in wards and operating theatres at a UK district general hospital to raise awareness of potential chlorhexidine allergy and to invite...

  12. Clinical comparison of scorpion envenomation by Androctonus mauritanicus and Buthus occitanus in children.

    Science.gov (United States)

    Aboumaâd, Bouchra; Lahssaini, Mohammed; Tiger, Abdelaziz; Benhassain, Sidi Mohammed

    2014-11-01

    The clinical results of scorpion stings by Androctonus mauritanicus (Am) and Buthus occitanus (Bo) (main sources of scorpionism in Morocco) were evaluated in this work. The objective was to compare the clinical manifestations of envenoming from these species by investigating possible correlations among symptoms/signs and laboratory abnormalities of envenomed patients. 41 children (25 males, 18 months - 11 years) were admitted at the Provincial Hospital of El Jadida-Morocco. Their minor (18 children) or severe (23 children) systemic signs such as pallor (48.8%), pulmonary edema (APE) (36.6%), convulsion (26.8%), coma (7.3%) were more frequent in children envenomed by Am than Bo, but angioedema (Quincke's edema) (4.9%) was particularly developed in the latter group. The laboratory blood abnormalities (hyperglycemia, high levels of aspartate aminotransferase (AST), lactate dehydrogenase (LDH), creatinine, bilirubin, leukocytes, neutrophils, monocytes, platelets and low levels of lymphocytes and hemoglobin) were significantly higher (p scorpion envenomation. PMID:25218169

  13. 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.

  14. [Latex allergy--report on two cases].

    Science.gov (United States)

    Lukesová, S; Krcmová, I; Kopecký, O

    2005-01-01

    In the last few years latex allergy has been increasingly recognised as a potential medical problem because of the increase in frequency and potential severity of latex-mediated reactions. Latex allergy is an IgE-mediated hypersensitivity response to natural rubber latex protein with a variety of clinical signs ranging from contact urticaria, angioedema, asthma, and anaphylaxis. Also IV. type of immunology response can participate in it. In highest risk for latex allergy are patients with spina bifida, but health care workers, latex industry workers, patients with multiple surgical procedures and others who wear gloves are also at risk. Patients with history of atopy belong to the high risk group. Diagnosis is done by positive in vitro tests (EAST, CAP-FEIA, immunoblott etc.) and skin prick test. Allergen avoidance and substitution and the use of latex-safe devices including synthetic gloves are essential for the affected patient. PMID:16193945

  15. Cutaneous reactions to analgesic-antipyretics and nonsteroidal anti-inflammatory drugs. Analysis of reports to the spontaneous reporting system of the Gruppo Italiano Studi Epidemiologici in Dermatologia.

    Science.gov (United States)

    1993-01-01

    We analyzed the cutaneous reactions to systemic analgesic-antipyretics and non-steroidal anti-inflammatory drugs reported to the spontaneous reporting system of the Gruppo Italiano Studi Epidemiologici in Dermatologia (GISED). The system has been active since 1988, with periodic intensive surveillance exercises, and 202 dermatologists have collaborated. Up to December 1991, 2,137 reactions had been collected, of which 713 were reactions to systemic analgesic-antipyretics and nonsteroidal anti-inflammatory drugs. A general profile of the reactions was identifiable. It included, in order of frequency, urticaria/angioedema, fixed eruptions, exanthemas, erythema multiforme and Stevens Johnson syndrome. Fixed eruptions and Stevens Johnson syndrome were reported with exceedingly high frequency in association with feprazone. Our system also revealed previously unreported reactions, including fixed eruption to nimesulide, fixed eruption to piroxicam and fixed eruption to flurbiprofen.

  16. [Allergic reaction after contact with Hura crepitans (sandbox tree)].

    Science.gov (United States)

    Thumm, E J; Bayerl, C; Goerdt, S

    2002-03-01

    We describe a 36-year-old female patient with angioedema-like swellings and rhinoconjunctivitis for 1 year occurring exclusively at her home. The clinical history revealed no correlation with foods, food additives, drugs, or aeroallergens. The complaints always started immediately after contact with the sandbox tree (Hura crepitans) placed in her apartment. Scratch testing resulted in a two-fold positive reaction towards leaves and stem, while five controls remained negative. Thus we suggest the reaction of the patient to be allergic in nature. Hura crepitans belongs to the family of Euphorbiaceae, whose largest genera are Euphorbia and Croton. The toxic reactions to the milky sap, the so-called latex, of these plants are caused by ingredients such as phorbol esters, croton oil, lectins, and terpens. Various terpens are also well known as allergens. Phytotoxic and phytoallergic reactions are growing increasingly important and should therefore be included in the differential diagnosis in dermatology.

  17. A challenge for pediatrician: non allergic urticaria

    Directory of Open Access Journals (Sweden)

    M. Mandelli

    2013-12-01

    Full Text Available In general population about 15-20% of subjects have suffered from one episode of urticaria-angioedema syndrome in their life. The etiology of his condition is various and multifactorial. In children the principal cause of acute urticaria is infection, while physical factors are the main agents of chronic urticaria. All those conditions which lack an etiology are named chronic idiopathic urticaria, but in reality a considerable number of these patients is affected by a chronic autoimmune urticaria. For this reason, screening out the most frequent causes of chronic urticaria, it’s useful to know when it’s possible to apply specific diagnostic tests for this condition and which therapies are employable.

  18. 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.

  19. 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. PMID:26661797

  20. 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.

  1. A study of drug eruptions by provocative tests

    Directory of Open Access Journals (Sweden)

    Das J

    2001-09-01

    Full Text Available Sixty cases of drug eruptions were observed during the period of one year. The incidence of drug eruption was 0.47% amongst all Dermatology O.P.D. attendances. Male to female ratio was 7:3. The highest number of cases were seen in the age group of 21-30 years. Fixed drug eruptions were the most frequent (58.3%, followed by urticaria and angioedema (20%. The drug sulphonamides (including co-trimoxazole accounted for the highest number of eruptions (35%. The other drugs which were responsible for the eruptions, in order of frequency, were oxyphenbutazone, ampicillin, analgin, penicillin, tetracycline, ibuprofen, paracetamol, phenylbutazone, acetaminophen and phenobarbitone. The causative drug (s were confirmed by provocation tests in 42 (70% cases.

  2. Spondylarthritis presenting with an allergic immediate systemic reaction to adalimumab in a woman: a case report

    Directory of Open Access Journals (Sweden)

    Valentini Maurizio

    2011-04-01

    Full Text Available Abstract Introduction The efficacy of adalimumab, a fully human anti-tumor necrosis factor α recombinant antibody, has dramatically improved the quality of life of patients with rheumatoid and psoriatic arthritis and Crohn's disease. Because it is fully human, one should not expect immune reactions to this molecule. Adverse reactions to adalimumab are limited mainly to injection site reactions and are very common. Immediate systemic reactions are rarely reported. Case presentation We report the case of a 61-year-old Caucasian woman who was treated with adalimumab for spondylarthritis and developed injection site reactions after the sixth dose. After a two-month suspension, she recommenced therapy and experienced two systemic reactions. The first occurred after one hour with itching of the palms and soles and angioedema of the tongue and lips. Thirty minutes after the next dose the patient had itching of the palms and soles with diffusion to her whole body, angioedema of the lips, dizziness and visual disturbances. A skin-prick test and intra-dermal tests with adalimumab gave strong positive results at the immediate reading. However, serum-specific immunoglobulin E (IgE to adalimumab were not detectable by using Phadia solid phase, especially harvested for this case, in collaboration with our Immunology and Allergy Laboratory Unit. Her total IgE concentration was 6.4 kU/L. Conclusion We describe what is, to the best of our knowledge, the first reported case of immediate systemic reaction to adalimumab studied with a skin test giving positive results and a serum-specific IgE assay giving negative results. The mechanism of the reaction must be immunologic but not IgE-mediated.

  3. Accompanying conditions in patients with chronic spontaneous urticaria and urticarial vasculitis: Results of a retrospective study

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    Sevgi Akarsu

    2015-03-01

    Full Text Available Background and Design: Chronic spontaneous urticaria (CSU, the most common form of chronic urticaria, is characterized by spontaneous wheals and/or angioedema lasting longer than six weeks. Urticarial vasculitis (UV is a small vessel vasculitis; but is also included in the various classification systems of chronic urticaria by some authors. The aim of our study was to evaluate the frequency of accompanying conditions, and to compare the demographic, clinical and laboratory features of patients with CSU and UV. Materials and Methods: In this study, the files of CSU (n=146 and UV (n=43 patients who had been hospitalized between January 2010-December 2013 were retrospectively reviewed in terms of demographic data, disease-specific history, accompanying diseases, medications and laboratory findings. Results: When comparing between the two groups for statistical differences, it was found that the frequencies of personal atopy and angioedema were significantly higher in patients with CSU; however, the patients with UV had significantly higher rates for female gender and the presence of systemic symptoms compared to those with CSU. It was determined that the mean values for erythrocyte sedimentation rate and C-reactive protein, as well as the rates of antinuclear antibody positivity and hypocomplementemia were significantly higher in the UV group, compared with those in the CSU group. Accompanying factors were determined as infections, medications, autoimmune diseases and malignities in order of frequency in both CSU and UV groups, but only the frequency of autoimmune diseases was significantly higher in patients with UV group. Conclusion: Although various authors have reported that a large number (up to 40-60% of patients with UV may present only with wheals (clinically indistinguishable from CSU; we assumed that it would be more appropriate to be considered UV as a priority in patients especially who had systemic symptom, autoimmune disease

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

    Institute of Scientific and Technical Information of China (English)

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

    2012-01-01

    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 < 0.01). These findings suggest that upregulated aquaporin-4 expression is likely to be the main molecular mechanism of intracellular edema and may be the molecular basis for decreased relative apparent diffusion coefficient. Aquaporin-4 gene interference can effectively inhibit the upregulation of aquaporin-4 expression during the stage of intracellular edema with time

  5. Drug eruptions: An 8-year study including 106 inpatients at a dermatology clinic in Turkey

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    Fatma Akpinar

    2012-01-01

    Full Text Available Background: Few clinical studies are found in the literature about patients hospitalized with a diagnosis of cutaneous drug eruption. Aims: To determine the clinical types of drug eruptions and their causative agents in a hospital-based population. Materials and Methods: This retrospective study was performed in the Dermatology Department of Haseki General Hospital. Through 1751 patients hospitalized in this department between 2002 and 2009, inpatients diagnosed as drug eruption were evaluated according to WHO causality definitions. 106 patients composed of probable and possible cases of cutaneous drug eruptions were included in this study. Results: Seventy one females and 35 males were evolved. Mean age was 44.03±15.14. Duration between drug intake and onset of reaction varied from 5 minutes to 3 months. The most common clinical type was urticaria and/or angioedema in 48.1% of the patients, followed by maculopapular rash in 13.2%, and drug rash with eosinophilia and systemic symptoms in 8.5%. Drugs most frequently associated with cutaneous drug eruptions were antimicrobial agents in 40.5% of the patients, followed by antipyretic/anti-inflammatory analgesics in 31.1%, and antiepileptics in 11.3%. Conclusion: Urticaria and/or angioedema and maculopapular rash comprised majority of the drug eruptions. Rare reactions such as acute generalized exanthematous pustulosis, sweet syndrome, oral ulceration were also found. Antimicrobial agents and antipyretic/anti-inflammatory analgesics were the most commonly implicated drugs. Infrequently reported adverse reactions to myorelaxant agents, newer cephalosporins and fluoroquinolones were also detected. We suppose that studies on drug eruptions should continue, because the pattern of consumption of drugs is changing in every country at different periods and many new drugs are introduced on the market continuously.

  6. AUTOLOGOUS SERUM SKIN TESTING (ASST IN CHRONIC IDIOPATHIC URTICARIA

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    Arun

    2014-01-01

    Full Text Available Chronic idiopathic urticaria (CIU is a form of urticaria , in which there appears to be persistent activation of mast cells , but the mechanism of mast cell triggering is unknown. The Autologous serum skin test (ASST is an in vivo test which assesses auto reactivity. ASST could be good screening test for Autoreactive urticaria a subset of chronic idiopathic urticaria. AIMS : To study the clinical profile of chronic idiopathic urticaria and pattern of ASST among p atients of chronic idiopathic urticaria. METHODS : Study included 200 patients of CIU that were recruited from outpatient department (OPD. Patient s with a history of urticaria for more than 6 weeks were included in the study. The detailed history , cutaneou s and systemic examination was done. ASST was performed under strict aseptic precaution. RESULTS : Out of 200 patients 100 were male and 100 were female. Mean age of patients was 31.9 yrs. Most common age group was 25 - 34yrs. ASST was found positive in 36(18 % patients. In most of the patients duration of disease was less than 11months (42% with the mean duration of disease 21.74 months. Mean duration of wheal was 64.7 minutes , in which most of the patients (112 having duration of wheal less than 59 min. Hi story of angioedema was positive in 109(54.5% patients. Aggravating factors were positive in 50(25% patients in which drugs and cold were the most common. Family history of urticaria and angioedema was present in 35 patients. None of patient complained a ny adverse reactions during and after ASST. CONCLUSION : ASST is the easily available bed side test for the diagnosis of autoreactive urticaria. It is a simple , inexpensive , semi invasive and easy - to - perform test which can be done and recorded by the dermat ologist himself. ASST may help in diagnosis and management of chronic idiopathic urticaria.

  7. A case of isocyanate-induced asthma possibly complicated by food allergy after peanut consumption: a case report

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    Xhixha Fatmira

    2008-11-01

    Full Text Available Abstract Background Isocyanates are extensively used in the manufacture of polyurethane foams, plastics, coatings or adhesives. They are a major cause of occupational asthma in a proportion of exposed workers. Recent findings in animal models have demonstrated that isocyanate-induced asthma does not always represent an IgE-mediated sensitization, but still a mixed profile of CD4+ Th1 and TH2, as well as a CD8+ immune response. Despite immunologic similarities between this pathology and IgE-mediated food allergies, this co-morbidity is rarely reported. Case presentation A 50-year old man employed as vehicle body painter, for 8 years complained about breathlessness, wheezing, sneezing, nasal obstruction and excessive production of mucus during the use of DuPont Refinish Centari Tintings – an acrylic enamel tint. Symptoms occurred 15–20 minutes after workplace exposure and usually persisted until evening, or at times, up to two consecutive days. The above mentioned symptoms were associated with a decrease of lung functions parameters. The use of inhaled adrenergic bronchio-dilatators and steroids relived the symptoms. In addition, three years ago he developed an anaphylactic reaction due to peanut consumption, experiencing urticaria, angioedema and airway obstruction. He was successfully treated in the hospital. Later, the subject exhibited labial itching, as well as orbital and perioral angioedema, 20 minutes after stationary performance of challenge test with peanuts. Evaluating the reported data, this process might be developed rather due to induction of a TH2 profile, because in both cases have occurred IgE-mediated symptoms. A less plausible mechanism could be the presence of isocyanates in peanuts due to a probable contamination by pesticides resulting in an allergic reaction after "consumption" of di-isocyanate as long as the isocyanate contamination of peanuts has not been proven. Conclusion Despite the lack of relevant laboratory

  8. 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

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

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    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

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

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    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.

  11. Unmet clinical needs in chronic spontaneous urticaria. A GA²LEN task force report.

    Science.gov (United States)

    Maurer, M; Weller, K; Bindslev-Jensen, C; Giménez-Arnau, A; Bousquet, P J; Bousquet, J; Canonica, G W; Church, M K; Godse, K V; Grattan, C E H; Greaves, M W; Hide, M; Kalogeromitros, D; Kaplan, A P; Saini, S S; Zhu, X J; Zuberbier, T

    2011-03-01

    Chronic spontaneous urticaria, formerly also known as chronic idiopathic urticaria and chronic urticaria (CU), is more common than previously thought. At any time, 0.5-1% of the population suffers from the disease (point prevalence). Although all age groups can be affected, the peak incidence is seen between 20 and 40 years of age. The duration of the disease is generally 1-5 years but is likely to be longer in more severe cases, cases with concurrent angioedema, in combination with physical urticaria or with a positive autologous serum skin test (autoreactivity). Chronic spontaneous urticaria has major detrimental effects on quality of life, with sleep deprivation and psychiatric comorbidity being frequent. It also has a large impact on society in terms of direct and indirect health care costs as well as reduced performance at work and in private life. In the majority of patients, an underlying cause cannot be identified making a causal and/or curative treatment difficult. Nonsedating H₁-antihistamines are the mainstay of symptomatic therapy, but treatment with licensed doses relieves symptoms effectively in < 50% of patients. Although guideline-recommended updosing up to fourfold increases symptom control in many patients, a substantial number of patients have only little benefit from H₁ -antihistamines. Consequently, there is a great need for new therapeutic strategies.

  12. 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.

  13. Echinacea purpurea: Pharmacology, phytochemistry and analysis methods

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    Azadeh Manayi

    2015-01-01

    Full Text Available Echinacea purpurea (Asteraceae is a perennial medicinal herb with important immunostimulatory and anti-inflammatory properties, especially the alleviation of cold symptoms. The plant also attracted scientists′ attention to assess other aspects of its beneficial effects. For instance, antianxiety, antidepression, cytotoxicity, and antimutagenicity as induced by the plant have been revealed in various studies. The findings of the clinical trials are controversial in terms of side effects. While some studies revealed the beneficial effects of the plant on the patients and no severe adverse effects, some others have reported serious side effects including abdominal pain, angioedema, dyspnea, nausea, pruritus, rash, erythema, and urticaria. Other biological activities of the plant such as antioxidant, antibacterial, antiviral, and larvicidal activities have been reported in previous experimental studies. Different classes of secondary metabolites of the plant such as alkamides, caffeic acid derivatives, polysaccharides, and glycoproteins are believed to be biologically and pharmacologically active. Actually, concurrent determination and single analysis of cichoric acid and alkamides have been successfully developed mainly by using high-performance liquid chromatography (HPLC coupled with different detectors including UV spectrophotometric, coulometric electrochemical, and electrospray ionization mass spectrometric detectors. The results of the studies which were controversial revealed that in spite of major experiments successfully accomplished using E. purpurea, many questions remain unanswered and future investigations may aim for complete recognition of the plant′s mechanism of action using new, complementary methods.

  14. [Histamine intolerance--possible dermatologic sequences].

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    Lugović-Mihić, Liborija; Seserko, Ana; Duvancić, Tomislav; Situm, Mirna; Mihić, Josip

    2012-12-01

    Although histamine intolerance (HIT) is not very frequently encountered, it can have serious consequences. Food intolerance is a non allergic hypersensitivity to food that does not include the immune system even though the symptoms are similar to those of IgE-mediated allergic reactions. HIT apparently develops as a result of an impaired diamine oxidase (DAO) activity due to gastrointestinal disease or through DAO inhibition, as well as through a genetic predisposition which was proven in a number of patients. The intake of histamine-rich foods as well as alcohol or drugs which cause either the release of histamine or the blocking of DAO can lead to various disorders in many organs (gastrointestinal system, skin, lungs, cardiovascular system and brain), depending on the expression of histamine receptors. Dermatologic sequels can be rashes, itch, urticaria, angioedema, dermatitis, eczema and even acne, rosacea, psoriasis, and other. Recognizing the symptoms due to HIT is especially important in treating such patients. The significance of HIT in patients with atopic dermatitis in whom the benefit of a low histamine diet has been proven is becoming increasingly understood recently. Because of the possibility of symptoms affecting numerous organs, a detailed history of symptoms following the intake of histamine-rich foods or drugs that interfere with histamine metabolism is essential for making the diagnosis of HIT. Considering that such symptoms can be the result of multiple factors, the existence of HIT is usually underestimated, but considerable expectations are being made from future studies. PMID:23814966

  15. IgE-mediated food hypersensitivity disorders.

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    Gotua, M; Lomidze, N; Dolidze, N; Gotua, T

    2008-04-01

    Food allergy has become a serious health concern especially in developed countries in the past two decades. In general population approximately 4-6% of children and 1-3% of adults experience food allergy. The article reviews IgE-mediated food hypersensitivity disorders. Epidemiology, Mechanism, Clinical manifestations, Genetically modified crops (GMOs), Diagnosis, Prevention and Treatment of IgE-mediated food allergies are discussed. The investigations show that over 90% of IgE-mediated food allergies in childhood are caused by: cow's milk, hen's egg, soy, peanuts, tree nuts, wheat, fish and shellfish. Also the causes of food allergy are food additives, genetically modified crops. Risk factors for food-dependent exercise-induced anaphylaxis include asthma and previous allergic reactions to the causative food. Food allergy is one of the most common causes of systematic anaphylaxis and anaphylactoid reactions, with an annual incidence of four cases per million populations and estimated 500 deaths annually. In addition to gastrointestinal symptoms, individuals may experience urticaria, angioedema, atopic dermatitis, oral syndrome, asthma, rhinitis, conjunctivitis, hypotension, shock and cardiac arrhythmias, caused by the massive release of mediators from mast cells and basophiles. Diagnosis of food allergy is based on history, detailed dietary analysis, skin testing, measuring specific IgE in blood serum and challenge tests. Treatment and prevention includes: avoidance diet, application of auto-injectable epinephrine, H1 and H2 antihistamines, corticosteroids, antileukotrienes, prostaglandin synthetase inhibitors, cromolyn sodium, etc. PMID:18487689

  16. The contact activation and kallikrein/kinin systems: pathophysiologic and physiologic activities.

    Science.gov (United States)

    Schmaier, A H

    2016-01-01

    The contact activation system (CAS) and kallikrein/kinin system (KKS) are older recognized biochemical pathways that include several proteins that skirt the fringes of the blood coagulation, fibrinolytic, complement and renin-angiotensin fields. These proteins initially were proposed as part of the hemostatic pathways because their deficiencies are associated with prolonged clinical assays. However, the absence of bleeding states with deficiencies of factor XII (FXII), prekallikrein (PK) and high-molecular-weight kininogen indicates that the CAS and KKS do not contribute to hemostasis. Since the discovery of the Hageman factor 60 years ago much has been learned about the biochemistry, cell biology and animal physiology of these proteins. The CAS is a pathophysiologic surface defense mechanism against foreign proteins, organisms and artificial materials. The KKS is an inflammatory response mechanism. Targeting their activation through FXIIa or plasma kallikrein inhibition when blood interacts with the artificial surfaces of modern interventional medicine or in acute attacks of hereditary angioedema restores vascular homeostasis. FXII/FXIIa and products that arise with PK deficiency also offer novel ways to reduce arterial and venous thrombosis without an effect on hemostasis. In summary, there is revived interest in the CAS and KKS due to better understanding of their activities. The new appreciation of these systems will lead to several new therapies for a variety of medical disorders. PMID:26565070

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

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    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.

  18. Perforation of the Colon During Imatinib Mesylate (Gleevec) Treatment in a Patient with Chronic Myeloid Leukemia (CML).

    Science.gov (United States)

    El Jurdi, Najla; Bankoff, Mark; Klein, Andreas; Saif, Muhammad W

    2016-01-01

    Imatinib (Gleevec; STI-571) is a tyrosine-kinase inhibitor (TKI) used in the treatment of multiple cancers, most notably Philadelphia chromosome-positive (Ph+) chronic myelogenous leukemia (CML) as well as gastrointestinal stromal tumor (GIST). The most common adverse effects with imatinib include superficial edema, muscle cramps, musculoskeletal pain, rash, fatigue, headache, and gastrointestinal side effects. Less frequent side effects include pancytopenia, febrile neutropenia, flushing, and liver function test abnormalities. Very rare side effects include secondary malignancies, Sweet's syndrome, angioedema, or cardiac arrest. We report the first case report of gastrointestinal perforation complicating imatinib treatment for CML. Unlike other antiangiogenic TKIs such as sunitinib or sorafenib that target vascular endothelial growth factor (VEGF) and known to cause gastrointestinal perforation, imatininib is a TKI with no known anti-VEGF activity, and so it remains unclear how imatinib would be associated with developing this life threatening complication. However, physicians caring for patients of imatinib should be aware of this potential toxicity. We suggest that careful attention and an appropriate clinical evaluation are required for patients presenting with gastrointestinal symptoms during imatinib treatment. PMID:27489753

  19. 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.

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

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    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.

  1. Omalizumab for the treatment of chronic urticaria.

    Science.gov (United States)

    Zuberbier, Torsten; Maurer, Marcus

    2015-02-01

    Urticaria is a common and often debilitating dermatological condition defined by the sudden appearance of wheals, angioedema or both. It is further classified into specific subtypes based on duration and specific triggers. Awareness and understanding of urticaria are important to ensure a correct initial diagnosis and initiate appropriate guideline-based treatment outlining a stepwise approach. However, in chronic urticaria, approximately 50% of patients are refractory to the first step, the use of licensed doses of second-generation H1-antihistamines. If the second step, an increase in the dose of the second-generation H1-antihistamines, is also not successful, in the third step omalizumab (Xolair™, Novartis Pharma AG(©)/Genentech, Inc.(©)), an anti-IgE therapy, is recommended as an add-on. Of all alternative treatments mentioned in the guidelines, omalizumab is currently the only licensed treatment for H1-antihistamine-refractory chronic spontaneous urticaria, has a favorable risk/benefit ratio and was well tolerated in clinical studies.

  2. Subtypes of chronic urticaria in patients attending allergy clinics in Venezuela.

    Science.gov (United States)

    Sánchez-Borges, M; Caballero-Fonseca, F; Capriles-Hulett, A

    2014-11-01

    Chronic urticaria (CU) is one of the most puzzling clinical entities confronted by the medical profession. It is a common motive for consultation, and in a sizable proportion of patients no identifiable cause is evident. Since there are relatively few publications regarding CU in developing countries, we performed a prospective 3-year study on the demographic and clinical features of patients with CU. Four hundred and twenty-three subjects were studied, 52 children and 371 adults, 295 females (69.7%), with a mean age of 38.4 ± 17.8 years. More often, wheals and angioedema (AE) were present on the head, upper and lower limbs and the trunk. AE was present in 162 patients (38.4%). The most frequent subtypes were chronic spontaneous urticaria, aspirin-exacerbated cutaneous disease, dermographic urticaria, and combinations of various subtypes. A better understanding of the characteristics of patients suffering CU is helpful for clinicians dealing with this ailment, and provides guidance for new investigations on its pathogenesis, which will hopefully result in a better management of this vexing condition.

  3. Controversies and challenges in the management of chronic urticaria.

    Science.gov (United States)

    Staubach, P; Zuberbier, T; Vestergaard, C; Siebenhaar, F; Toubi, E; Sussman, G

    2016-07-01

    This supplement reports proceedings of the second international Global Urticaria Forum, which was held in Berlin, Germany in November 2015. Despite the clear international guideline, there remain a number of controversies and challenges in the management of patients with chronic urticaria (CU). As a result of major advancements in urticaria over the past 4 years, the current EAACI/GA(2) LEN/EDF/WAO urticaria guideline treatment algorithm requires updating. Case studies from patients with chronic spontaneous urticaria (CSU) [also called chronic idiopathic urticaria (CIU)], chronic inducible urticaria (CIndU) or diseases and syndromes related to CU are useful in describing and exploring challenges in disease management. Case studies of specific CSU patient populations such as children with CU or patients with angio-edema but no hives also require consideration as potentially challenging groups with unmet needs. The current EAACI/GA(2) LEN/EDF/WAO urticaria guideline provides a general framework for the management of patients with CU but, as these cases highlight, a personalized approach based on the expert knowledge of the physician may be required.

  4. Urticaria and anaphilaxis in a child after inhalation of lentils vapours: a case report and literature review

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    Vitaliti Giovanna

    2012-12-01

    Full Text Available Abstract Background Among legumes, lentils seem to be the most common legume implicated in pediatric allergic reactions in the Mediterranean area and India, and usually they start early in life, below 4 years of age. Case report A 22 -month-old child was admitted to our Pediatric Department for anaphylaxis and urticaria. At the age of 9 months she presented a first episode of angioedema and laryngeal obstruction, due to a second assumption of lentils in her diet. At admission we performed routine analyses that were all in the normal range, except for the dosage of specific IgE, that revealed a positive result for lentils. Prick tests too were positive for lentils, while they were all negative for other main food allergens. The child also performed a prick by prick that gave the same positive result (with a wheal of 8 mm of diameter. The child had not previously eaten lentils and other legumes, but her pathological anamnesis highlighted that the allergic reaction appeared soon after the inhalation of cooking lentil vapours when the child entered the kitchen Therefore a diagnosis of lentils vapours allergy was made. Conclusions Our case shows the peculiarity of a very early onset. In literature there are no data on episodes of anaphylaxis in so young children, considering that our child was already on lentils exclusion diet. Therefore a diet of exclusion does not absolutely preserve patients from allergic reactions, that can develop also after their cooking steams inhalation.

  5. Oral allergy syndrome--the need of a multidisciplinary approach.

    Science.gov (United States)

    Kelava, Nikolina; Lugović-Mihić, Liborija; Duvancić, Tomislav; Romić, Renata; Situm, Mirna

    2014-06-01

    Oral allergy syndrome (OAS) is one of the most common types of food allergy. The syndrome includes itching and swelling of the lips, palate and tongue, usually after consuming fresh fruits and vegetables. The underlying pathogenic mechanism is cross-reactivity between IgE antibodies specific to pollen, and antigens in food, such as fresh fruits, vegetables and nuts that are structurally similar to pollen. Both pollen and food antigens can bind to IgE and trigger type I immune reaction. Diagnosis is primarily based on the patient's history, and confirmed by skin tests, in vitro tests, and oral provocation tests. Differential diagnoses include many diseases (such as burning mouth syndrome, angioedema, hay fever, various other oral diseases, etc.), and for this reason a multidisciplinary approach is necessary, as different specialists need to be involved in the diagnostic procedure. Therapy includes avoiding, or thermal processing of, fruit and vegetables known to trigger a reaction, and antihistamine medications. If a more severe anaphylactic reaction develops, more aggressive therapy is required. The goal of this article is to present OAS, its etiopathogenesis, clinical picture, and symptoms, diagnostic approach and therapy for OAS.

  6. Serum IgG antibodies to C1q in hypocomplementemic urticarial vasculitis syndrome.

    Science.gov (United States)

    Wisnieski, J J; Naff, G B

    1989-09-01

    Urticaria, angioedema, and arthritis are cardinal features of hypocomplementemic urticarial vasculitis syndrome (HUVS). Considered to be an immune complex-mediated disorder, HUVS has been differentiated from systemic lupus erythematosus (SLE), based on its clinical manifestations and the C1q precipitin (C1q-p) reaction, which is manifested as gel precipitation of C1q by a small percentage of HUVS IgG molecules. This phenomenon has been attributed to an Fc region abnormality, and the responsible IgG molecules are said to possess C1q-p activity. We purified IgG from 4 HUVS patients and confirmed that HUVS IgG contains C1q binding activity. F(ab')2 fragments from these patients also bound to C1q, as measured by 2 different C1q binding methods at physiologic ionic strength; HUVS IgG Fc fragments did not bind to C1q. Preincubation of HUVS F(ab')2 fragments with antibody to human F(ab')2 prevented subsequent binding to C1q. We conclude that IgG antibodies to C1q are present in HUVS serum, and it is likely that these antibodies are C1q-p. Because the clinical manifestations of HUVS and the presence of anti-C1q antibodies have been described in patients with SLE, our findings support the concept that HUVS is an autoimmune syndrome related to SLE. PMID:2528353

  7. Safety and Tolerability of the Direct Renin Inhibitor Aliskiren in Combination with Angiotensin Receptor Blockers and Thiazide Diuretics: A Pooled Analysis of Clinical Experience of 12,942 Patients

    Science.gov (United States)

    White, William B.; Bresalier, Robert; Kaplan, Allen P.; Palmer, Biff F.; Riddell, Robert H.; Lesogor, Anastasia; Chang, William; Keefe, Deborah L.

    2011-01-01

    Combinations of the direct renin inhibitor aliskiren with angiotensin receptor blockers (ARBs) or diuretics are effective therapeutic regimens for the treatment of hypertension. A large database of safety information has become available during the past several years with aliskiren in combination trials. Data were pooled from nine short-term (8-week) and four longer-term (26–52-week) randomized, controlled trials of aliskiren in patients with hypertension. Adverse event (AE) rates were assessed for aliskiren combination therapy compared to component monotherapies. In short-term studies, overall AE rates were similar for those receiving aliskiren/valsartan or aliskiren/diuretic combinations (32.2–39.8%) and those receiving the component monotherapies (30.0–39.6%). In longer-term studies, AE rates with aliskiren/losartan (55.5%) and aliskiren/diuretic (45.0%) combination therapy were similar to those with losartan (53.9%) and diuretic (48.9%) alone. Angioedema and hyperkalemia occurred in similar proportions of patients on combination therapies versus monotherapy. In conclusion, the safety and tolerability profile of aliskiren in combination with the ARBs valsartan or losartan, or diuretic is similar to aliskiren, ARBs or diuretic alone. PMID:21029339

  8. Manicurists and pedicurists – Occupation group at high risk of work-related dermatoses

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    Marta Kieć-Świerczyńska

    2013-08-01

    Full Text Available In recent years occupational skin and respiratory diseases have been more and more frequently diagnosed in small production and service enterprises. The awareness of occupational exposure and its possible health effects among their workers and employers is not sufficient. Beauty salons, in addition to hairdressers and beauticians, frequently employ manicurists and pedicurists. The workers often happen to perform various activities interchangeably. The health status of beauty salons workers has rarely been assessed. The most numerous reports concern hairdressers. In this occupational group, the occurrence of skin lesions induced by wet work and frequent allergy to metals, hair dyes and bleaches and perm solutions has been emphasized, while information about health hazards for being a manicurist or pedicurist in beauty salons is seldom reported. The aim of this paper is to present professional activities (manicure and pedicure, methods of nail stylization, occupational exposure and literature data on work-related adverse health effects in manicurists and pedicurists. Wet work and exposure to solvents, fragrances, resins, metals, gum, detergents may cause skin disorders (contact dermatitis, urticaria, angioedema, photodermatoses, conjunctivitis, anaphylaxis, respiratory tract diseases, including asthma. The discussed occupations are also associated with the increased incidence of bacterial (particularly purulent, viral and fungal infections and cancer. Med Pr 2013;64(4:579–591

  9. Aquaporin-4 gene silencing protects injured neurons after early cerebral infarction

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    Zhan-ping He

    2015-01-01

    Full Text Available Aquaporin-4 regulates water molecule channels and is important in tissue regulation and water transportation in the brain. Upregulation of aquaporin-4 expression is closely related to cellular edema after early cerebral infarction. Cellular edema and aquaporin-4 expression can be determined by measuring cerebral infarct area and apparent diffusion coefficient using diffusion-weighted imaging (DWI. We examined the effects of silencing aquaporin-4 on cerebral infarction. Rat models of cerebral infarction were established by occlusion of the right middle cerebral artery and siRNA-aquaporin-4 was immediately injected via the right basal ganglia. In control animals, the area of high signal intensity and relative apparent diffusion coefficient value on T2-weighted imaging (T2WI and DWI gradually increased within 0.5-6 hours after cerebral infarction. After aquaporin-4 gene silencing, the area of high signal intensity on T2WI and DWI reduced, relative apparent diffusion coefficient value was increased, and cellular edema was obviously alleviated. At 6 hours after cerebral infarction, the apparent diffusion coefficient value was similar between treatment and model groups, but angioedema was still obvious in the treatment group. These results indicate that aquaporin-4 gene silencing can effectively relieve cellular edema after early cerebral infarction; and when conducted accurately and on time, the diffusion coefficient value and the area of high signal intensity on T2WI and DWI can reflect therapeutic effects of aquaporin-4 gene silencing on cellular edema.

  10. Angiotensin receptor neprilysin inhibition in heart failure: mechanistic action and clinical impact.

    Science.gov (United States)

    Buggey, Jonathan; Mentz, Robert J; DeVore, Adam D; Velazquez, Eric J

    2015-09-01

    Heart failure (HF) is an increasingly common syndrome associated with high mortality and economic burden, and there has been a paucity over the past decade of new pharmacotherapies that improve outcomes. However, recent data from a large randomized controlled trial compared the novel agent LCZ696, a dual-acting angiotensin receptor blocker and neprilysin inhibitor (ARNi), with the well established angiotensin-converting enzyme (ACE) inhibitor enalapril and found significant reduction in mortality among the chronic reduced ejection fraction HF population. Preclinical and clinical data suggest that neprilysin inhibition provides beneficial outcomes in HF patients by preventing the degradation of natriuretic peptides and thereby promoting natriuresis and vasodilatation and counteracting the negative cardiorenal effects of the up-regulated renin-angiotensin-aldosterone system. Agents such as omapatrilat combined neprilysin and ACE inhibition but had increased rates of angioedema. Goals of an improved safety profile provided the rationale for the development of the ARNi LCZ696. Along with significant reductions in mortality and hospitalizations, clinical trials suggest that LCZ696 may improve surrogate markers of HF severity. In this paper, we review the preclinical and clinical data that led to the development of LCZ696, the understanding of the underlying mechanistic action, and the robust clinical impact that LCZ696 may have in the near future.

  11. Blood cells and endothelial barrier function.

    Science.gov (United States)

    Rodrigues, Stephen F; Granger, D Neil

    2015-01-01

    The barrier properties of endothelial cells are critical for the maintenance of water and protein balance between the intravascular and extravascular compartments. An impairment of endothelial barrier function has been implicated in the genesis and/or progression of a variety of pathological conditions, including pulmonary edema, ischemic stroke, neurodegenerative disorders, angioedema, sepsis and cancer. The altered barrier function in these conditions is often linked to the release of soluble mediators from resident cells (e.g., mast cells, macrophages) and/or recruited blood cells. The interaction of the mediators with receptors expressed on the surface of endothelial cells diminishes barrier function either by altering the expression of adhesive proteins in the inter-endothelial junctions, by altering the organization of the cytoskeleton, or both. Reactive oxygen species (ROS), proteolytic enzymes (e.g., matrix metalloproteinase, elastase), oncostatin M, and VEGF are part of a long list of mediators that have been implicated in endothelial barrier failure. In this review, we address the role of blood borne cells, including, neutrophils, lymphocytes, monocytes, and platelets, in the regulation of endothelial barrier function in health and disease. Attention is also devoted to new targets for therapeutic intervention in disease states with morbidity and mortality related to endothelial barrier dysfunction. PMID:25838983

  12. 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.

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

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    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.

  14. A STUDY OF CLINICAL, PATHOLOGICAL AND BIOCHEMICAL FINDINGS IN PATIENTS WHO ATTEMPTED SUICIDE BY HAIR DYE SUPERVASMOL 33 INGESTION

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    Sudheer Babu

    2015-06-01

    Full Text Available BACKGROUND : All the patient in the study consumed the hair dye available in the market with the trade name Supervasmol 33. The prevalence of Super vasmol 33 hair dye poisoning has been on a surge for the past 2 - 3 years as has been observed by the increase in number of cases being admitted into the hospitals. It was observed that the tendency of poisoning by Super vasmol 33 hair dye was more in females than in males and was more in the age group of 15 - 35 years, as with any other poisoning. METERIALS AND METHODS: Study of Clinical, pathological and biochemical findings in patients who attempted suicide by hair dye ingestion (Supervasmol 3 who were admitted in Government General Hospital/Guntur medical college, Guntur during June 2013 to March 2015 brought to emergency department and those who were admitted into the HDU, ICU and medical wards of the hospital, after the following exclusion criteria were ruled out. RESULTS: A significant statistical correlation was found to exist between development of AKI and the levels of CPK in blood, rhabdomyolysis and hyperkalemia. The morbidity rates were 100% for angioedema, 58% for rhabdomyolysis and 32% for acute kidney injury (AKI. Institutional mortality rate was about 8% due to refractory hemodynamic shock. CONCLUSION: As the burden of handling Supervasmol 33 hair dye poisoning cases has been recently increasing, primary care physicians, intensive care physicians and nephrologists need to be aware of its clinical manifestation and management

  15. Clinical characteristics and treatment of Melkersson-Rosenthal syndrome - overview of six patients

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    Rašković Sanvila

    2015-01-01

    Full Text Available Introduction. Melkersson-Rosenthal syndrome is a rare disease of unknown etiology. Histopathologically, it presents as granulomatous cheilitis. From laboratory aspect, it is a nonspecific, differential diagnostically and therapeutically complex condition. Case Report. This is a report of six cases treated at the Department of Allergology and Immunology of the Clinical Center of Serbia, who had presented with the referral diagnosis of recurring or persistent lip edema, and who were diagnosed with Melkersson-Rosenthal syndrome upon detailed evaluation. Three patients had complete triad of symptoms, two had the oligosymptomatic form and one manifested the monosymptomatic form of the disease. Histopathological findings of the oral mucosa specimens verified the presence of non-necrotic epithelioid granulomas in all patients. The patients were treated with the H1 and H2 antihistamines, corticosteroids, followed by anabolic drugs and antibiotics, resulting in transient and unfavorable effects. Conclusion. In differential diagnosis, Melkersson-Rosenthal syndrome diagnosis primarily refers to conditions of angioneurotic edema and hereditary angioedema, as well as granulomatous diseases such as sarcoidosis, tuberculosis and Chron’s disease. It is necessary to follow-up these patients in view of monitoring the effects of the therapy and possible development of systemic granulomatous diseases.

  16. Aquaporin-4 gene silencing protects injured neurons after early cerebral infarction

    Institute of Scientific and Technical Information of China (English)

    Zhan-ping He; Hong Lu

    2015-01-01

    Aquaporin-4 regulates water molecule channels and is important in tissue regulation and water transportation in the brain. Upregulation of aquaporin-4 expression is closely related to cel-lular edema after early cerebral infarction. Cellular edema and aquaporin-4 expression can be determined by measuring cerebral infarct area and apparent diffusion coefficient using diffu-sion-weighted imaging (DWI). We examined the effects of silencing aquaporin-4 on cerebral infarction. Rat models of cerebral infarction were established by occlusion of the right middle cerebral artery and siRNA-aquaporin-4 was immediately injectedvia the right basal ganglia. In control animals, the area of high signal intensity and relative apparent diffusion coefifcient value on T2-weighted imaging (T2WI) and DWI gradually increased within 0.5–6 hours after cerebral infarction. After aquaporin-4 gene silencing, the area of high signal intensity on T2WI and DWI reduced, relative apparent diffusion coefifcient value was increased, and cellular edema was ob-viously alleviated. At 6 hours after cerebral infarction, the apparent diffusion coefifcient value was similar between treatment and model groups, but angioedema was still obvious in the treat-ment group. These results indicate that aquaporin-4 gene silencing can effectively relieve cellular edema after early cerebral infarction; and when conducted accurately and on time, the diffusion coefifcient value and the area of high signal intensity on T2WI and DWI can relfect therapeutic effects of aquaporin-4 gene silencing on cellular edema.

  17. Wheat-dependent exercise-induced anaphylaxis sensitized with hydrolyzed wheat protein in soap.

    Science.gov (United States)

    Chinuki, Yuko; Morita, Eishin

    2012-12-01

    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.

  18. Anaphylaxis to protamine masquerading as an insulin allergy.

    Science.gov (United States)

    Kim, R

    1993-01-01

    This is the case of a 62-year-old man referred for the evaluation of insulin allergy. This patient had reacted to the subcutaneous injection of Novolin 70/30 (Squibb, Princeton, N.J.) and Humulin NPH (Eli Lilly, Indianapolis, Ind.). These reactions were characterized by the immediate onset of diffuse pruritic urticaria and angioedema with progression to hypotension as well as a local reaction. Past history also included anaphylactic shock after intravenous administration of protamine sulfate used for heparin reversal during arterial bypass surgery. Immediate hypersensitivty skin testing to protamine containing (NPH) insulin and protamine sulfate USP were strongly positive, while Lente insulin (Eli Lilly, Indianapolis, Ind.) and controls were negative. RAST tests revealed the titers > 24 ng/ml of protamine specific IgE with 98 percent inhibition and 1163 ng/ml of protamine specific IgG with 29 percent inhibition, while levels of insulin specific antibodies were negligible. Subsequently, the patient was treated with non-protamine containing insulin preparation, Lente insulin, without further incident. This study confirms the diagnosis of Type I hypersensitivity to protamine sulfate masquerading as insulin allergy. PMID:8454092

  19. [Use of antihistamines in a physician's clinical practice].

    Science.gov (United States)

    Luss, L V

    2014-01-01

    Histamine that belongs to one of the most important mediators involved in the regulation of the body's vital functions plays a great role in the pathogenesis of different diseases. Histamine is released during inflammatory and allergic reactions, anaphylactic and anaphylactoid shock, pseudoallergic reactions, and others. Acting through histamine receptors, it leads to increased intracellular concentration of cyclic guanosine monophosphate, enhanced chemotaxis of eosinophils and neutrophils, production of prostaglandins and thromboxane B, suppressed synthesis of lymphokines, etc. and causes contraction of smooth muscles of particularly the bronchi and intestine, dilation of vessels and their increased permeability, mucus hypersecretion in the upper airways, lower blood pressure, angioedema and itch, etc. In this connection, antihistamines that block histamine-induced reactions in various ways: by inhibiting its biosynthesis, enhancing its neutralization, blocking the access to receptors, and suppressing the release from mast cells, occupy a prominent place in clinical practice. The review covers the classification, main mechanisms of pharmacological action, and indications for the use of antihistamines that not only have the well-known antihistamine properties, but have also a broad spectrum of anti-inflammatory activity. There are data on the benefits of a group of antihistamines, the quinuclidine derivatives (quifenadine, sequifenadine) that were designed by Academician M.D. Mashkovsky and are one of the first examples of designing new classes of multifunctional non-sedating antihistamines, which combines a high selective activity to block histamine type 1 receptors and an ability to block serotonin and to break down histamine directly in tissues. PMID:25306755

  20. Anaphylaxis and Anaphylactoid Reactions: Diagnosis and Management.

    Science.gov (United States)

    Luskin, Allan T.; Luskin, Susan S.

    1996-07-01

    Anaphylaxis is an acute fatal or potentially fatal hypersensitivity reaction. Anaphylaxis represent a clinical diagnosis based on history and physical examination and includes symptoms of airway obstruction, generalized skin reactions, particularly flushing, itching, urticaria, angioedema cardiovascular symptoms including hypotension and gastrointestinal symptoms. These symptoms result from the action of mast cell mediators, especially histamine and lipid mediators such as leukotrienes and platelet activating factor on shock tissue. The shock tissue includes blood vessels, mucous glands, smooth muscle, and nerve endings. Anaphylaxis follows the typical immediate hypersensitivity time course, with a reaction beginning within minutes of antigen exposure. A late-phase reaction hours after the initial reaction may occur. Mast cell mediator release can be triggered by both IgE and non--IgE-mediated factors. Therefore, anaphylaxis may be termed anaphylaxis (IgE mediated) or anaphylactoid (non--IgE mediated). The most common IgE-mediated triggers are drugs, typically penicillin or other beta-lactam antibiotics, foods, most commonly nuts, peanuts, fish and shellfish, or hymenoptera stings. Non-IgE-mediated causes include factors causing marked complement activation such as plasma proteins or compounds which act directly on the mast cell membrane, such as vancomycin, quinolone antibiotics, or radiographic contrast media. The pathophysiology of some trigger factors, such as aspirin, remains unclear. Therapy of anaphylaxis revolves around patient education, avoidance, desensitization or pharmacologic pretreatment when agents causing anaphylaxis need to be readministered, and early recognition and prompt therapy of reactions should they occur. PMID:11862283

  1. Medical image of the week: bilateral pneumothorax, pneumomediastinum, and massive subcutaneous emphysema

    Directory of Open Access Journals (Sweden)

    Ahmed Z

    2014-03-01

    Full Text Available No abstract available. Article truncated at 150 words. An 80 year old man with chronic obstructive pulmonary disease (COPD presented to the emergency department with respiratory distress and poor oxygen saturation. Physical exam revealed an obese male in respiratory distress with poor air entry bilaterally and scattered wheezing. His chest, neck, tongue, and lips were swollen. The patient was intubated for respiratory failure, felt to be due to angioedema. His oxygen saturation immediately improved, however the patient developed progressive swelling throughout his body including his eyelids, fingers and toes. Diffuse crepitus was felt on palpation. Chest radiography and computed tomography (CT of the chest revealed large bilateral pneumothoraces, pneumomediastinum, and subcutaneous emphysema (Figure 1. Bilateral chest tubes were inserted with re-expansion of both lungs. Over the next several days his respiratory parameters improved, with full re-expansion of the lungs and reabsorption of the pneumomediastinum and subcutaneous emphysema. The patient was extubated successfully and was discharged in good health. ...

  2. Natural rubber latex allergy: spectrum, diagnostic approach, and therapy.

    Science.gov (United States)

    Woods, J A; Lambert, S; Platts-Mills, T A; Drake, D B; Edlich, R F

    1997-01-01

    Latex allergy has reached epidemic proportions in the United States and is increasingly recognized as a significant contributor to morbidity and mortality during medical and surgical procedures. Ultimately, many of the affected patients with recognized latex sensitivity and those who are not yet diagnosed will receive treatment for their allergic reactions to latex in emergency departments. Consequently, emergency physicians must have a comprehensive understanding of the etiology, epidemiology, pathogenesis, treatment, and management of these challenging patients. Groups at high risk include spina bifida cystica patients, health care workers, latex industry workers, specific food-allergy patients, and patients with a history of atopy or multiple surgical procedures. Sensitization to latex antigens is commonly encountered in health care workers wearing latex gloves with high latex allergen concentrations and in workers using powdered latex surgical gloves. Exposure to air-borne allergens and water-soluble IgE reactive latex antigens from natural rubber latex products in sensitized individuals can result in type I (immediate) hypersensitivity reactions. Clinical manifestations include contact urticaria, dermatitis, allergic rhinitis, conjunctivitis, asthma, angioedema, and anaphylaxis. Diagnostic tools include serological assays and skin prick testing. At present, latex avoidance is the only available treatment and is the key to preventing allergic reactions in latex-sensitized individuals. Health care worker sensitization to latex antigens in natural rubber products is becoming an increasing contributor to workers' liability and disability claims. Specific action can be taken to reduce occupational and patient exposure to latex antigens. PMID:9017491

  3. 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.

  4. Penicillin induced toxic epidermal necrolysis with secondary impetiginization: a rare case

    Directory of Open Access Journals (Sweden)

    Hari Babu Ramineni

    2015-02-01

    Full Text Available Drug induced allergic reactions can be categorized into IgE-mediated and non-IgE mediated hypersensitivity reactions. Symptoms of IgE-mediated reactions are angioedema, bronchospasm, anaphylaxis, and urticaria that appears within 72 hours and those which are Non-IgE mediated hypersensitivity reactions include morbilliform eruptions, interstitial nephritis, hemolytic anemia, serum sickness, thrombocytopenia, and erythema multiforme, after 72 hours. TEN is defined as an extensive detachment of full-thickness epidermis most often related to an adverse drug reaction. We report a rare case of penicillin induced toxic epidermal necrolysis with Secondary Impetigination in a 38-year-old male patient with complaints of rashes all over the body, chest pain and dry tongue since seven days. Based on history and clinical examination patient was diagnosed as of penicillin induced toxic epidermal necrolysis with secondary impetigination and was successfully treated with antihistamines, parenteral antibiotics and corticosteroids. [Int J Res Med Sci 2015; 3(2.000: 511-512

  5. The modified Atkins diet for intractable epilepsy may be associated with late-onset egg-induced anaphylactic reaction: a case report.

    Science.gov (United States)

    Levy, Yael; Peleg-Weiss, Lilach; Goldberg-Stern, Hadassa

    2011-03-01

    The modified Atkins diet is a therapeutic option for children with intractable epilepsy. It is less restrictive than the traditional ketogenic diet, with ≈60% of calories from fat sources. We describe a 6-y-old boy with intractable epilepsy treated with the modified Atkins diet who presented to the emergency department with first-time anaphylactic reaction to egg. Symptoms of urticaria and angioedema, shortness of breath, wheezing, and cyanosis developed several minutes after he ate a hard-boiled egg. His history was remarkable for asthma, but no food allergies were documented. The anaphylactic reaction appeared after 6 mo of treatment with the modified Atkins diet (including 10-15 eggs daily), which ameliorated his seizures, and was preceded by streptococcal pharyngitis. Laboratory workup revealed specific immunoglobulin E antibodies to egg. This is the first report of new-onset egg allergy in a child, probably triggered by the high egg content of the modified Atkins diet. The risk of egg allergy should be kept in mind when treating epileptic children with the modified Atkins diet, especially those with comorbid asthma.

  6. Epinephrine autoinjector refill history in an HMO.

    Science.gov (United States)

    Kaplan, Michael S; Jung, Sandy Y; Chiang, Matthew L

    2011-02-01

    Epinephrine can be lifesaving in episodes of anaphylaxis, yet it is underprescribed and underused. Tracking of epinephrine refills over time for patients with a diagnosis of anaphylaxis has not been reported. This study reports on the refill history of 14,677 patients in a large HMO who received an initial dispensing of EpiPen (Dey Pharma, Basking Ridge, NJ) or EpiPen Jr between 2000 and 2006. A total of 6,776 (46%) refilled at least once. Twenty-five percent of the patients who were in the cohort for 5 years or more refilled multiple times, and 11% refilled consistently at all expected refill times. Infants through children 12 years of age were more likely to receive a refill dispensing (63%) compared with teenagers and adults (40%). The most common ICD-9 codes that were linked to the initial epinephrine dispensing were allergic disorder (37%), miscellaneous anaphylaxis/angioedema (23%), hymenoptera/insect bite or sting (14%), and specific or nonspecific food allergy (11%). A total of 79% of patients with a food-related ICD-9 code and 59% of patients with an insect sting-related ICD-9 code refilled epinephrine at least once. An opportunity exists to identify system-based as well as personal barriers in an ongoing effort to provide patients at risk with the tools and empowerment that could reduce their risk during life-threatening anaphylactic reactions.

  7. Exercise-induced anaphylaxis.

    Science.gov (United States)

    Sheffer, A L; Austen, K F

    1980-08-01

    Sixteen patients were seen because of possibly life-threatening exercise-associated symptoms similar to anaphylactic reactions. Asthma attacks, cholinergic urticaria and angioedema, and cardiac arrythmias are recognized as exertion-related phenomena in predisposed patients but are distinct from the syndrome described here. A syndrome characterized by the exertion-related onset of cutaneous pruritus and warmth, the development of generalized urticaria, and the appearance of such additional manifestations as collapse in 12 patients, gastrointestinal tract symptoms in five patients, and upper respiratory distress in 10 patients has been designated exercise-induced anaphylaxis, because of the striking similarity of this symptom complex to the anaphylactic syndrome elicited by ingestion or injection of a foreign antigenic substance. There is a family history of atopic desease for 11 patients and cold urticaria for two others and a personal history of atopy in six. The size of the wheals, the failure to develop an attack with a warm bath or shower or a fever, and the prominence of syncope rule against the diagnosis of conventional cholinergic urticaria. There is no history or evidence of an encounter with an environmental source of antigen during the exercise period. PMID:7400473

  8. A fatal adverse effect of cefazolin administration: severe brain edema in a patient with multiple meningiomas

    Directory of Open Access Journals (Sweden)

    Tribuddharat S

    2016-02-01

    Full Text Available Sirirat Tribuddharat,1 Thepakorn Sathitkarnmanee,1 Amnat Kitkhuandee,2 Sunchai Theerapongpakdee,1 Kriangsak Ngamsaengsirisup,1 Sarinya Chanthawong,11Department of Anesthesiology, 2Department of Surgery, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand Abstract: Cefazolin is commonly administered before surgery as a prophylactic antibiotic. Hypersensitivity to cefazolin is not uncommon, and the symptoms mostly include urticaria, skin reaction, diarrhea, vomiting, and transient neutropenia, which are rarely life threatening. We present a rare case of fatal cefazolin hypersensitivity in a female who was diagnosed with multiple meningiomas and scheduled for craniotomy and tumor removal. Immediately after cefazolin IV administration, the patient developed acute hypertensive crisis, which resolved within 10 minutes after the treatment. This was followed by unexplained metabolic acidosis. The patient then developed severe brain edema 100 minutes later. The patient had facial edema when her face was exposed for the next 30 minutes. A computed tomography scan revealed global brain edema with herniation. She was admitted to the intensive care unit for symptomatic treatment and died 10 days after surgery from multiorgan failure. The serum IgE level was very high (734 IU/mL. Single-dose administration of cefazolin for surgical prophylaxis may lead to rare, fatal adverse reaction. The warning signs are sudden, unexplained metabolic acidosis, hypertensive crisis, tachycardia, and facial angioedema predominating with or without cutaneous symptoms like urticaria. Keywords: cefazolin, adverse effect, drug hypersensitivity, brain edema, hypertension

  9. [Mansonella perstans filariasis].

    Science.gov (United States)

    Bregani, E R; Tantardini, F; Rovellini, A

    2007-06-01

    Mansonella perstans filariasis is widely present in Africa and equatorial America and its pathogenicity has been recently reconsidered. Although M. perstans infection has been considered a minor filariasis, remaining asymptomatic in most of infected subjects, more recent studies have shown that M. perstans is capable of inducing a variety of clinical features, including angioedemas, swellings like the "Calabar swellings" of loiasis, pruritus, fever, headache, pain in bursae and/or joint synovia, or in serous cavities. It is likely that some of the pathological changes observed are induced by the immune response to the infection. Eosinophilia is present in many cases of infection. Moreover M. perstans filariasis is difficult to be treated. Effective treatment is lacking and there is no consensus on optimal therapeutic approach. The most commonly used drug is diethylcarbamazine (DEC) that is however often ineffective. Although other drugs have been tried (e.g. praziquantel, ivermectin), none has proven to be reliably and rapidly effective. Mebendazole seemed more active than DEC in eliminating the infection, with a comparable rate of overall responses. Thiabendazole evidenced a small, but significant activity against the infection. Combination treatments (DEC plus mebendazole) resulted in a significantly higher activity compared with the single drugs. PMID:18416002

  10. [Manicurists and pedicurists - occupation group at high risk of work-related dermatoses].

    Science.gov (United States)

    Kieć-Swierczyńska, Marta; Chomiczewska-Skóra, Dorota; Swierczyńska-Machura, Dominika; Krecisz, Beata

    2013-01-01

    In recent years occupational skin and respiratory diseases have been more and more frequently diagnosed in small production and service enterprises. The awareness of occupational exposure and its possible health effects among their workers and employers is not sufficient. Beauty salons, in addition to hairdressers and beauticians, frequently employ manicurists and pedicurists. The workers often happen to perform various activities interchangeably. the health status of beauty salons workers has rarely been assessed. The most numerous reports concern hairdressers. In this occupational group, the occurrence of skin lesions induced by wet work and frequent allergy to metals, hair dyes and bleaches and perm solutions has been emphasized, while information about health hazards for being a manicurist or pedicurist in beauty salons is seldom reported. The aim of this paper is to present professional activities (manicure and pedicure, methods of nail stylization), occupational exposure and literature data on work-related adverse health effects in manicurists and pedicurists. Wet work and exposure to solvents, fragrances, resins, metals, gum, detergents may cause skin disorders (contact dermatitis, urticaria, angioedema, photodermatoses), conjunctivitis, anaphylaxis, respiratory tract diseases, including asthma. The discussed occupations are also associated with the increased incidence of bacterial (particularly purulent), viral and fungal infections and cancer. PMID:24502122

  11. [Histamine intolerance--possible dermatologic sequences].

    Science.gov (United States)

    Lugović-Mihić, Liborija; Seserko, Ana; Duvancić, Tomislav; Situm, Mirna; Mihić, Josip

    2012-12-01

    Although histamine intolerance (HIT) is not very frequently encountered, it can have serious consequences. Food intolerance is a non allergic hypersensitivity to food that does not include the immune system even though the symptoms are similar to those of IgE-mediated allergic reactions. HIT apparently develops as a result of an impaired diamine oxidase (DAO) activity due to gastrointestinal disease or through DAO inhibition, as well as through a genetic predisposition which was proven in a number of patients. The intake of histamine-rich foods as well as alcohol or drugs which cause either the release of histamine or the blocking of DAO can lead to various disorders in many organs (gastrointestinal system, skin, lungs, cardiovascular system and brain), depending on the expression of histamine receptors. Dermatologic sequels can be rashes, itch, urticaria, angioedema, dermatitis, eczema and even acne, rosacea, psoriasis, and other. Recognizing the symptoms due to HIT is especially important in treating such patients. The significance of HIT in patients with atopic dermatitis in whom the benefit of a low histamine diet has been proven is becoming increasingly understood recently. Because of the possibility of symptoms affecting numerous organs, a detailed history of symptoms following the intake of histamine-rich foods or drugs that interfere with histamine metabolism is essential for making the diagnosis of HIT. Considering that such symptoms can be the result of multiple factors, the existence of HIT is usually underestimated, but considerable expectations are being made from future studies.

  12. Signal detection for Thai traditional medicine: examination of national pharmacovigilance data using reporting odds ratio and reported population attributable risk.

    Science.gov (United States)

    Wechwithan, Sareeya; Suwankesawong, Wimon; Sornsrivichai, Vorasith; McNeil, Edward B; Jiraphongsa, Chuleeporn; Chongsuvivatwong, Virasakdi

    2014-10-01

    Herbal containing medicine consumption has increased while the awareness of adverse drug reaction (ADR) was less than conventional medicine. Early detection of unexpected numbers of ADRs from herbal medicines' reports which are abnormal from the whole database needs quantification. Disproportionality analysis has been performed for signal detection by using reporting odds ratio (ROR) as measurement. The impact of having medicine as exposures in each ADR should be measured by using reported population attributable risks (RPAR). This study aimed to quantify the contribution of Thai traditional medicine (TTM) to ADR reports and to assess the association between TTMs and serious adverse drug reactions. Data were retrieved from the adverse drug reaction surveillance database, Thai-Food and Drug Administration from 2002 to 2013. Crude and adjusted RORs for each drug-ADR pair and RPARs were computed. TTM contributed only 0.001% of all serious ADRs reported. Out of 4208 TTM-ADR pairs were examined, three had the statistically significant RORs, namely Andrographis paniculata and anaphylactic shock (ROR 2.32, 95% CI 1.03, 5.21); green traditional medicine and Stevens-Johnson syndrome (ROR 13.04, 95% CI 5.4-31.51) and Derris scandens Benth and angioedema (ROR 2.71, 95% CI 1.05-6.95). Their RPARs ranged from 0.05% to 0.16%. We conclude that TTMs need more intensive surveillance. PMID:24945744

  13. Avocado hypersensitivity.

    Science.gov (United States)

    Blanco, C; Carrillo, T; Castillo, R; Quiralte, J; Cuevas, M

    1994-07-01

    The avocado (Av) is a fruit that belongs to the Lauraceae family. We report 17 patients with immediate hypersensitivity to avocado. Clinical manifestations in relation to avocado ingestion were as follows: systemic anaphylaxis in seven patients, angioedema/urticaria in six, vomiting in two, bronchial asthma in one, and rhinoconjunctivitis in one. Skin prick test (SPT) with fresh avocado was positive in all patients with the Strong avocado variety (SAv) and in 14 patients with the Hass avocado variety (HAv). Our patient-associated sensitizations were as follows: 10 to latex, eight to chestnut, eight to banana, four to kiwi, and four to walnut. Avocado-sensitized patients with latex allergy were typically middle-aged women, professionally exposed to latex, who also exhibited frequent associated sensitizations to chestnut, banana, and other fruits. Specific IgE against avocado was demonstrated in 11 of our patients, by both commercial CAP and RAST with avocado extract coupled to nitrocellulose disks. Despite its lower protein content, SAv seems to be more allergenic than HAv, both in vivo and in vitro. On incubating a pool of sera from our patients with avocado, latex, chestnut, and banana extracts, a progressive RAST inhibition was obtained, with SAv- and chestnut-marked disks. This suggests the existence of common antigenic determinants among these allergens.

  14. An update on the clinical pharmacology of the dipeptidyl peptidase 4 inhibitor alogliptin used for the treatment of type 2 diabetes mellitus.

    Science.gov (United States)

    Chen, Xiao-Wu; He, Zhi-Xu; Zhou, Zhi-Wei; Yang, Tianxin; Zhang, Xueji; Yang, Yin-Xue; Duan, Wei; Zhou, Shu-Feng

    2015-12-01

    Alogliptin, a dipeptidyl peptidase-4 (DPP-4) inhibitor that is a class of relatively new oral hypoglycaemic drugs used in patients with type 2 diabetes (T2DM), can be used as monotherapy or in combination with other anti-diabetic agents, including metformin, pioglitazone, sulfonylureas and insulin with a considerable therapeutic effect. Alogliptin exhibits favorable pharmacokinetic and pharmacodynamic profiles in humans. Alogliptin is mainly metabolized by cytochrome P450 (CYP2D6) and CYP3A4. Dose reduction is recommended for patients with moderate or worse renal impairment. Side effects of alogliptin include nasopharyngitis, upper-respiratory tract infections and headache. Hypoglycaemia is seen in about 1.5% of the T2DM patients. Rare but severe adverse reactions such as acute pancreatitis, serious hypersensitivity including anaphylaxis, angioedema and severe cutaneous reactions such as Stevens-Johnson syndrome have been reported from post-marketing monitoring. Pharmacokinetic interactions have not been observed between alogliptin and other drugs including glyburide, metformin, pioglitazone, insulin and warfarin. The present review aimed to update the clinical information on pharmacodynamics, pharmacokinetics, adverse effects and drug interactions, and to discuss the future directions of alogliptin. PMID:26218204

  15. 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

    Directory of Open Access Journals (Sweden)

    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

  16. The case for a national service for primary immune deficiency disorders in New Zealand.

    Science.gov (United States)

    Ameratunga, Rohan; Steele, Richard; Jordan, Anthony; Preece, Kahn; Barker, Russell; Brewerton, Maia; Lindsay, Karen; Sinclair, Jan; Storey, Peter; Woon, See-Tarn

    2016-01-01

    Primary immune deficiency disorders (PIDs) are rare conditions for which effective treatment is available. It is critical these patients are identified at an early stage to prevent unnecessary morbidity and mortality. Treatment of these disorders is expensive and expert evaluation and ongoing management by a clinical immunologist is essential. Until recently there has been a major shortage of clinical immunologists in New Zealand. While the numbers of trained immunologists have increased in recent years, most are located in Auckland. The majority of symptomatic PID patients require life-long immunoglobulin replacement. Currently there is a shortage of subcutaneous and intravenous immunoglobulin (SCIG/IVIG) in New Zealand. A recent audit by the New Zealand Blood Service (NZBS) showed that compliance with indications for SCIG/IVIG treatment was poor in District Health Boards (DHBs) without an immunology service. The NZBS audit has shown that approximately 20% of annual prescriptions for SCIG/IVIG, costing $6M, do not comply with UK or Australian guidelines. Inappropriate use may have contributed to the present shortage of SCIG/IVIG necessitating importation of the product. This is likely to have resulted in a major unnecessary financial burden to each DHB. Here we present the case for a national service responsible for the tertiary care of PID patients and oversight for immunoglobulin use for primary and non-haematological secondary immunodeficiencies. We propose that other PIDs, including hereditary angioedema, are integrated into a national PID service. Ancillary services, including the customised genetic testing service, and research are also an essential component of an integrated national PID service and are described in this review. As we show here, a hub-and-spoke model for a national service for PIDs would result in major cost savings, as well as improved patient care. It would also allow seamless transition from paediatric to adult services.

  17. Anaphylaxis to honey in pollinosis to mugwort: a case report.

    Science.gov (United States)

    Fuiano, N; Incorvaia, C; Riario-Sforza, G G; Casino, G

    2006-12-01

    A case of anaphylaxis to honey in a 19 year old female sensitized to Compositae pollen is described. The patient suffered from summer rhinoconjunctivitis since seven years; in January 2006, ten minutes after eating bread and honey she developed angioedema of the lips and tongue, runny nose, cough, dyspnoea, and collapse, requiring hospitalization and treatment with high dose corticosteroids and anti-histamines. After two weeks, skin prick tests (SPT) with a standard panel of inhalant allergens and prick + prick with a number of kinds of honey were performed. SPTs were positive to mugwort, ragweed, dandelion, and goldenrod. Concerning honey, the prick + prick was positive to "Millefiori" (obtained from bees foraging on Compositae) and also to sunflower, limetree, and gum tree honey, while was negative for other kinds of honey, including the frequently used chestnut honey and acacia honey. The allergenic component responsible of anaphylaxis in this case seems to be a molecule occurring in Compositae pollens, as previously reported for other three reports, but also in pollen from plants of different families. Honey contains a large number of components derived from bees, such as gland secretions and wax, as well as from substances related to their foraging activity, such flower nectar and pollens (1, 2). Honey as a food has been associated to allergic reactions and particularly to anaphylaxis (3-6). Among the pollens, the role of Compositae is somewhat controversial, since its responsibility is clear in some studies (3, 5, 6) but considered negligible in others (7). Here we present the case of a patient sensitized to Compositae pollen who had an anaphylactic reaction to the ingestion of honey obtained from bees foraging on Compositae flowers and was tested with a number of different varieties of honey. PMID:17274522

  18. Chronic spontaneous urticaria and internal parasites--a systematic review.

    Science.gov (United States)

    Kolkhir, P; Balakirski, G; Merk, H F; Olisova, O; Maurer, M

    2016-03-01

    Chronic spontaneous urticaria (CSU) is defined as persistent wheals, angioedema, or both lasting for >6 weeks due to known or unknown causes. Some epidemiological studies and case reports suggest that internal parasite infections (PI) can cause CSU. Here, we provide a systematic overview of published findings on the prevalence and relevance of PI in CSU and we discuss possible pathomechanisms. The prevalence of PI in CSU was investigated by 39 independent studies and comorbidity reportedly ranged from 0 to 75.4% (two-thirds of these studies reported infection rates of 10% or less). The prevalence of PI in adult and pediatric CSU patients ranged from 0% to 75.4% and from 0% to 37.8%, respectively. CSU patients were more often diagnosed with protozoa and had a significantly higher risk of toxocariasis seropositivity and Anisakis simplex sensitization when compared to healthy controls. Patients with chronic urticaria more frequently had seropositivity of fasciolosis, Anisakis simplex sensitization, and the presence of Blastocystis hominis allele 34 (ST3) as compared with control subjects. In 21 studies, efficacy of treatment with antiparasitic drugs ranged from 0 to 100% (35.7% of 269 CSU patients benefitted). In 9 (42.8%) of 21 studies, more than 50% of efficacy was observed. The reported rate of urticaria comorbidity in PI patients in 18 independent studies is 1-66.7%. Urticaria including CSU might be a quite common symptom of strongyloidiasis and blastocystosis. Pathogenic mechanisms in CSU due to PI may include specific IgE, Th2 cytokine skewing, eosinophils, activation of the complement, and the coagulation systems. PMID:26648083

  19. The persian version of the chronic urticaria quality of life questionnaire: factor analysis, validation, and initial clinical findings.

    Directory of Open Access Journals (Sweden)

    Marzieh Tavakol

    2014-08-01

    Full Text Available Chronic urticaria (CU also known as chronic idiopathic urticaria results in a lowered quality of life (QoL. Disease specific questionnaires are necessary to assess QoL in CU patients. Chronic Urticaria Quality of Life Questionnaire (CU-Q2oL is the only available and validated disease specific questionnaire in the assessment of QoL in CU patients originally developed in Italian language. The aim of the current study was to develop the Persian version of the CU-Q2oL with an acceptable reliability and validity.Using the standard methods provided by guidelines, CU-Q2oL was translated into Persian. A total number of 110 patients with confirmed diagnosis of CU were asked to fill the questionnaire. Determination of scales was performed in addition to checking the data for internal consistency and known group validity. Urticaria activity score 7 (UAS7 was used to assess the severity of the CU in the population study. The 6 dimensional scale of Persian CU-Q2oL was determined using the Exploratory Factor Analysis. About 68% of the variance was explained by these 6 factor structure higher than 59.9% of the original Italian version.All 6 factors showed acceptable internal consistency as measured by Cronbach α coefficient. There was a significant correlation between UAS7 and total CU-Q2oL score. UAS7 and the presence of angioedema were predictors of CU-Q2oL score.The Persian version of CU-Q2oL was shown to be a valid and reliable tool to be used in the future clinical studies. Cultural considerations must be kept in mind in adoption of CU-Q2oL to other languages.

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

    Directory of Open Access Journals (Sweden)

    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.

  1. Questions and answers in chronic urticaria: where do we stand and where do we go?

    Science.gov (United States)

    Maurer, M; Church, M K; Marsland, A M; Sussman, G; Siebenhaar, F; Vestergaard, C; Broom, B

    2016-07-01

    This supplement reports proceedings of the second international Global Urticaria Forum, which was held in Berlin, Germany in November 2015. In 2011, a report of the GA(2) LEN task force on urticaria outlined important and unanswered questions in chronic urticaria (CU). These included, but were not limited to, questions on the epidemiology and course of chronic spontaneous urticaria (CSU) [also called chronic idiopathic urticaria (CIU)], the resources allocated for the diagnosis and treatment of CSU, whether patients with angioedema as an isolated symptom can be regarded as a subgroup of CSU, and the efficacy and long-term safety of therapies. Many of these questions have been addressed by recent studies. Some of the answers obtained raise new questions. Here, we summarize some of the key insights on CU obtained over recent years, and we discuss old and new unmet needs and how to address them with future studies. We need to analyze the influence of recent advances in understanding of the burden of CU on patients and society, disease management and the CU patient journey. Our increased understanding of urticarial pathophysiology and consideration of the patient as a whole will need to be translated to better treatment algorithms and protocols. Actions to address these challenges include the 5th International Consensus Meeting on Urticaria, which will take place later this year. The formation of a global network of Urticaria Centers of Reference and Excellence over the next few years has also been proposed, with the aim of providing consistent excellence in urticaria management and a clear referral route, furthering knowledge of urticaria through additional research and educating/promoting awareness of urticaria.

  2. Antihypertensive agent-induced drug eruptions:a literature review%抗高血压药引起药疹的文献分析

    Institute of Scientific and Technical Information of China (English)

    苏惠春; 姚煦; 王宝玺

    2016-01-01

    抗高血压药种类繁多,结构复杂,常用的有利尿剂、钙离子拮抗剂、血管紧张素转化酶抑制剂、血管紧张素Ⅱ受体阻滞剂、β受体阻滞剂5大类,可引起各种不同类型的药疹。通过文献回顾,对抗高血压药引起的血管性水肿、红斑狼疮样、银屑病样、湿疹样、疱疹样、扁平苔藓样等药疹的临床特征、发病机制、治疗及转归进行归纳总结,实现抗高血压药引起药疹的早发现、早诊断、早停药、早治疗,并为抗高血压药引起的药疹的临床和基础研究提供信息和思路。%There are various kinds of antihypertensive agents with complex chemical structures. Common antihypertensive agents are divided into 5 classes, including diuretics, calcium antagonists, angiotensin-converting enzyme inhibitors, angiotensin Ⅱ receptor blockers and β-blockers, and can cause various types of drug eruptions. This review summarizes clinical characteristics, possible pathogenesis, treatment and consequences of antihypertensive agent-induced drug eruptions, including angioedema, and lupus erythematosus-like, psoriasis-like, eczematoid, herpetiform or lichen planus-like drug eruptions, in hope to facilitate their early detection, diagnosis and treatment, and to provide information and ideas for clinical and basic researches into them.

  3. Urticarial vasculitis and urticarial autoinflammatory syndromes.

    Science.gov (United States)

    Marzano, A V; Tavecchio, S; Venturini, M; Sala, R; Calzavara-Pinton, P; Gattorno, M

    2015-02-01

    Urticaria is a frequent disorder classified as acute and chronic forms, which presents with wheals that can be associated with angioedema. Several entities may manifest with urticarial skin lesions, encompassing a heterogeneous group of conditions that have to be differentiated from ordinary urticaria. This review is focused on two of these urticarial syndromes: urticarial vasculitis (UV), which represents the most important differential diagnosis with common urticaria, and autoinflammatory diseases such as cryopyrin-associated periodic syndromes (CAPS) and Schnitzler's Syndrome, both rare multisystem forms that may masquerade as common urticaria. UV is a small-vessel vasculitis with predominant skin involvement, characterized by wheals persisting for more than 24 hours, burning rather than itching and resolving with hyperpigmentation as well as by other cutaneous manifestations including purpura, papules, vesicles, bullae and necrotic-ulcerative lesions. Histology shows a classic pattern of leukocytoclastic vasculitis, with possible presence of upper dermal edema. CAPS are classified as three distinct entities: familial cold autoinflammatory syndrome, Muckle-Wells Syndrome and chronic infantile neurological cutaneous and articular syndrome, which represent a spectrum of disorders caused by different mutations in a single gene, NLRP3 (NOD-like receptor 3). This gene encodes for cryopyrin, an inflammasome protein that activates interleukin-1β, leading to an overproduction of this pivotal proinflammatory cytokine. Histologically, urticarial lesions are generally characterized by a perivascular neutrophilic infiltrate. Unlike urticaria, neither UV nor urticarial autoinflammatory syndromes do respond to antihistamines: thus, it is important not to misdiagnose such conditions in order to give the patients specific treatments, potentially preventing serious systemic complications. PMID:25586657

  4. Comorbidity and pathogenic links of chronic spontaneous urticaria and systemic lupus erythematosus--a systematic review.

    Science.gov (United States)

    Kolkhir, P; Pogorelov, D; Olisova, O; Maurer, M

    2016-02-01

    Chronic spontaneous urticaria (CSU) is a common mast cell-driven disease characterized by the development of wheals (hives), angioedema (AE), or both for > 6 weeks. It is thought that autoimmunity is a common cause of CSU, which is often associated with autoimmune thyroiditis, whereas the link to other autoimmune disorders such as systemic lupus erythematosus (SLE) has not been carefully explored. Here, we systematically reviewed the existing literature for information on the prevalence of CSU in SLE (and vice versa) and we examined the possible clinical and pathogenetic relationship between CSU and SLE. The prevalence of CSU and CSU-like rash in SLE was investigated by 42 independent studies and comorbidity in adult patients reportedly ranged from 0% to 21.9% and 0.4% to 27.5%, respectively (urticarial vasculitis: 0-20%). In children with SLE, CSU was reported in 0-1.2% and CSU-like rash in 4.5-12% (urticarial vasculitis: 0-2.2%). In contrast, little information is available on the prevalence of SLE in patients with CSU, and more studies are needed to determine the rate of comorbidity. Recent insights on IgG- and IgE-mediated autoreactivity suggest similarities in the pathogenesis of CSU and SLE linking inflammation and autoimmunity with the activation of the complement and coagulation system. Future studies of patients with either or both conditions could help to better define common pathomechanisms in CSU and SLE and to develop novel targeted treatment options for patients with CSU and SLE. PMID:26545308

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

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    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.

  6. 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.

  7. Potential Roles for C1 Inhibitor in Transplantation.

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    Berger, Mel; Baldwin, William M; Jordan, Stanley C

    2016-07-01

    Complement is a major contributor to inflammation and graft injury. This system is especially important in ischemia-reperfusion injury/delayed graft function as well as in acute and chronic antibody-mediated rejection (AMR). The latter is increasingly recognized as a major cause of late graft loss, for which we have few effective therapies. C1 inhibitor (C1-INH) regulates several pathways which contribute to both acute and chronic graft injuries. However, C1-INH spares the alternative pathway and the membrane attack complex (C5-9) so innate antibacterial defenses remain intact. Plasma-derived C1-INH has been used to treat hereditary angioedema for more than 30 years with excellent safety. Studies with C1-INH in transplant recipients are limited, but have not revealed any unique toxicity or serious adverse events attributed to the protein. Extensive data from animal and ex vivo models suggest that C1-INH ameliorates ischemia-reperfusion injury. Initial clinical studies suggest this effect may allow transplantation of donor organs which are now discarded because the risk of primary graft dysfunction is considered too great. Although the incidence of severe early AMR is declining, accumulating evidence strongly suggests that complement is an important mediator of chronic AMR, a major cause of late graft loss. Thus, C1-INH may also be helpful in preserving function of established grafts. Early clinical studies in transplantation suggest significant beneficial effects of C1-INH with minimal toxicity. Recent results encourage continued investigation of this already-available therapeutic agent.

  8. C1-esterase inhibitor treatment: preclinical safety aspects on the potential prothrombotic risk.

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    Schürmann, Daniel; Herzog, Eva; Raquet, Elmar; Nolte, Marc W; May, Frauke; Müller-Cohrs, Jochen; Björkqvist, Jenny; Dickneite, Gerhard; Pragst, Ingo

    2014-11-01

    Human plasma-derived C1-esterase inhibitor (C1-INH) is an efficacious and safe treatment for hereditary angioedema. However, thrombotic events in subjects treated with C1-INH at recommended or off-label, high doses have been reported. In this study, we addressed the potential prothrombotic risk of C1-INH treatment in high doses using a non-clinical rabbit model. Following intravenous infusion of C1-INH to rabbits at doses up to 800 IU/kg, the exposure and the pharmacodynamic efficacy of C1-INH in rabbits were confirmed by activity measurements of C1-esterase, and coagulation factors XIa and XIIa, respectively. Potential prothrombotic effects were assessed following induction of venous and arterial thrombosis using in vivo models of venous and arterial stasis, complemented by various in vitro assays of coagulation markers. Administration of C1-INH at doses up to 800 IU/kg did not potentiate thrombus formation during venous stasis. In contrast, inhibition of arterial occlusion was observed upon C1-INH administration when compared with isotonic saline treatment, indicating antithrombotic rather than prothrombotic activity of high dose C1-INH treatment in vivo. This was further confirmed in vitro by decreased thrombin generation, increased activated partial thromboplastin time, clotting time and clot formation time, and inhibition of platelet aggregation. No relevant changes in fibrinolysis or in the levels of thrombin-antithrombin complexes, and prothrombin fragment 1+2 were observed upon high dose C1-INH treatment. The data suggest that treatment of healthy rabbits with high doses of C1-INH could potentially inhibit coagulation and thrombus formation rather than induce a prothrombotic risk.

  9. Complexity of case mix in a regional allergy service

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    Kaminski Edward R

    2012-02-01

    Full Text Available Abstract Background Currently in the United Kingdom (UK, there is a mismatch between limited financial resources and the large proportion of patients with suspected allergies actually being referred to specialist allergy clinics. To better understand the case mix of patients being referred, we audited referrals to a regional allergy service over an 8 year period. The main source of data was consultant letters to General Practitioners (GP summarising the diagnosis of patients, archived from January 2002 to September 2009. Letters were reviewed, extracting the clinic date, doctor seen, gender, date of birth, postcode, GP, and diagnoses. Diagnoses were classified into seven groups and illustrative cases for each group noted. Findings Data from 2,028 new referrals with suspected allergy were analysed. The largest group of patients (43% were diagnosed with a type I hypersensitivity. The other diagnostic groups were chronic idiopathic (spontaneous urticaria (35%, suspected type I hypersensitivity but no allergen identified (8%, idiopathic (spontaneous angioedema (8%, physical urticaria (2.5%, non-allergic symptoms (1.6%, type IV hypersensitivity (0.8% and ACE inhibitor sensitivity (0.5%. Two thirds of patients seen were female with a higher percentage of female patients in the non type-I hypersensitivity group (71% than the type 1 hypersensitivity (66% (χ2 = 5.1, 1df, p = 0.024. The type 1 hypersensitivity patients were younger than other patients (38 Vs 46 years, t = -10.8, p Conclusions This study highlights the complexity of specialist allergy practice and the large proportion of patients referred with non-type I hypersensitivities, chronic idiopathic (spontaneous urticaria being by far the largest group. Such information is critical to inform commissioning decisions, define referral pathways and in primary care education.

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

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    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.

  11. Structure function analysis of serpin super-family: "a computational approach".

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    Singh, Poonam; Jairajpuri, Mohamad Aman

    2014-01-01

    Serine Protease inhibitors (serpins) are a super-family of proteins that controls the proteinases involved in the inflammation, complementation, coagulation and fibrinolytic pathways. Serpins are prone to conformational diseases due to a complex inhibition mechanism that involves large scale conformational change, and their susceptibility to undergo point mutations might lead to functional defects. Serpins are associated with diseases like emphysema/cirrhosis, angioedema, familial dementia, chronic obstructive bronchitis and thrombosis. Serpin polymerization based pathologies are fairly widespread and devising a cure has been difficult due to lack of clarity regarding its mechanism. Serpin can exist in various conformational states and has a variable cofactor binding ability. It has a large genome and proteome database which can be utilized to gain critical insight into their structure, mechanism and defects. Comprehensive computational studies on the serpin family is lacking, most of the work done till date is limited and deals mostly with few individual serpins. We have tried to analyze few aspect of this family using diverse computational biology tools and have shown the following: a) the importance of residue burial linked shift in the conformational stability as a major factor in increasing the polymer propensity in serpins. b) Amino acids involved in the polymerization are in general completely buried in the native conformation. c) An isozyme specific antithrombin study showed the structural basis of improved heparin binding to beta antithrombin as compared to alpha-antithrombin. d) A comprehensive cavity analysis showed its importance in inhibition and polymerizaiton and finally e) an interface analysis of various serpin protease complexes identified critical evolutionary conserved residues in exosite that determines its protease specificity. This work introduces the problem and emphasizes on the need for in-depth computational studies of serpin superfamily

  12. Current understanding of contrast media reactions and implications for clinical management.

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    Meth, Marc J; Maibach, Howard I

    2006-01-01

    Iodinated contrast media (CM) are an integral part of modern diagnostic medicine. Although these agents are considered to be relatively safe, adverse effects in the form of allergy-like reactions occur in a significant number of exposed patients. These reactions may be divided into immediate and delayed responses. Immediate (within 1 hour of administration) anaphylactic reactions range from urticaria and angioedema to laryngeal oedema, hypotension and even death. Delayed reactions to CM occur from 1 hour to 1 week after administration and usually have mostly cutaneous manifestations. History of prior CM reactions and atopy predispose patients to CM reactions. Despite intense research into the pathogenesis of the immediate anaphylactoid responses, new evidence shows that true IgE type I hypersensitivity mediation occurs only in rare, severe cases. The aetiology appears to be multifactorial in most individuals. There is strong evidence to conclude that type IV hypersensitivity is responsible for the delayed reactions to CM. Although switching to non-ionic agents significantly reduces the incidence of immediate reactions to CM, there is little consensus regarding corticosteroid prophylaxis in high-risk individuals. Skin testing and provocative challenges also provide little security. Therefore, physicians must be better prepared to treat immediate anaphylactoid responses. Preventing delayed CM reactions is best performed with patch and delayed intradermal testing in those with a history of prior reactions, although false-negative results have been reported. Corticosteroids and antihistamines may be required for treatment. Until newer agents are developed that negate these issues, healthcare providers must strive to better understand the risk factors associated with CM reactions, as well as the available prophylactic and treatment options. PMID:16454540

  13. Papain Induced Occupational Asthma with Kiwi and Fig Allergy.

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    Jiang, Nannan; Yin, Jia; Wen, Liping

    2016-03-01

    Papain is a proteolytic enzyme which is widely used in food industry, pharmaceuticals, and cosmetics. Occupational and non-occupational papain allergies have previously been documented; however, there are limited publications about papain allergy with its relative fruit allergy. Here, we present a case of occupational, IgE-mediated papain allergy with kiwi fruit and fig fruit allergy. A 53-year-old man suffered from rhinitis for several years, with the onset of his symptoms coinciding with the time he started to work at a sausage processing plant where papain is often used as a meat tenderizer. He began to experience symptoms of chest tightness, shortness of breath and wheezing shortly after starting work 5 years ago. Furthermore, he experienced several episodes of oral itching, and tongue and oropharyngeal angioedema after injestion of kiwi fruit and fig fruit. The patient had a lifelong history of allergic conjunctivitis, allergic rhinitis, and childhood asthma. Specific IgE was positive to kiwi fruit, papain and chymopapain (2.95 kUA/L, >100 kUA/L, and 95.0 kUA/L, respectively). Similar bands at 10-15 kDa in blotting with papain and kiwi fruit extracts were found. This patient showed a potential association between papain allergy and sensitization to kiwi fruit. We also reviewed 13 patients with papain allergy published in the literature, with 85% (11/13) of the patients sensitized through the respiratory tract, and 40% (4/11) having atopy. Further studies should focus on the determination of cross-reactive allergens between papain and its fruit relatives, and the prevalence of food allergy in patients with papain allergy should be investigated in a relatively large cohort. PMID:26739411

  14. Peptide inhibitor of complement C1 (PIC1, a novel suppressor of classical pathway activation: mechanistic studies and clinical potential

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    Julia A Sharp

    2014-08-01

    Full Text Available The classical pathway of complement plays multiple physiological roles including modulating immunological effectors initiated by adaptive immune responses as well as an essential homeostatic role in the clearance of damaged self-antigens. However, dysregulated classical pathway activation is associated with antibody-initiated, inflammatory diseases processes like cold agglutinin disease (CAD, acute intravascular hemolytic transfusion reaction (AIHTR and acute/hyperacute transplantation rejection. To date, only one putative classical pathway inhibitor, C1 esterase inhibitor (C1-INH, is currently commercially available and its only approved indication is for replacement treatment in hereditary angioedema (HAE, which is predominantly a kinin pathway disease. Given the variety of disease conditions in which the classical pathway is implicated, development of therapeutics that specifically inhibit complement initiation represents a major unmet medical need. Our laboratory has identified a peptide that specifically inhibits the classical and lectin pathways of complement. In vitro studies have demonstrated that these Peptide Inhibitors of Complement C1 (PIC1 bind to the collagen-like region of the initiator molecule of the classical pathway, C1q. PIC1 binding to C1q blocks activation of the associated serine proteases (C1s-C1r-C1r-C1s and subsequent downstream complement activation. Rational design optimization of PIC1 has resulted in the generation of a highly potent derivative of fifteen amino acids. PIC1 inhibits classical pathway mediated complement activation in ABO incompatibility in vitro as well as inhibiting classical pathway activation in vivo in rats. This review will focus on the pre-clinical development of PIC1 and discuss its potential as a therapeutic in antibody-mediated classical pathway disease, specifically AIHTR.

  15. The case for a national service for primary immune deficiency disorders in New Zealand.

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    Ameratunga, Rohan; Steele, Richard; Jordan, Anthony; Preece, Kahn; Barker, Russell; Brewerton, Maia; Lindsay, Karen; Sinclair, Jan; Storey, Peter; Woon, See-Tarn

    2016-01-01

    Primary immune deficiency disorders (PIDs) are rare conditions for which effective treatment is available. It is critical these patients are identified at an early stage to prevent unnecessary morbidity and mortality. Treatment of these disorders is expensive and expert evaluation and ongoing management by a clinical immunologist is essential. Until recently there has been a major shortage of clinical immunologists in New Zealand. While the numbers of trained immunologists have increased in recent years, most are located in Auckland. The majority of symptomatic PID patients require life-long immunoglobulin replacement. Currently there is a shortage of subcutaneous and intravenous immunoglobulin (SCIG/IVIG) in New Zealand. A recent audit by the New Zealand Blood Service (NZBS) showed that compliance with indications for SCIG/IVIG treatment was poor in District Health Boards (DHBs) without an immunology service. The NZBS audit has shown that approximately 20% of annual prescriptions for SCIG/IVIG, costing $6M, do not comply with UK or Australian guidelines. Inappropriate use may have contributed to the present shortage of SCIG/IVIG necessitating importation of the product. This is likely to have resulted in a major unnecessary financial burden to each DHB. Here we present the case for a national service responsible for the tertiary care of PID patients and oversight for immunoglobulin use for primary and non-haematological secondary immunodeficiencies. We propose that other PIDs, including hereditary angioedema, are integrated into a national PID service. Ancillary services, including the customised genetic testing service, and research are also an essential component of an integrated national PID service and are described in this review. As we show here, a hub-and-spoke model for a national service for PIDs would result in major cost savings, as well as improved patient care. It would also allow seamless transition from paediatric to adult services. PMID:27355232

  16. 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

  17. 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.

  18. ASTHMA AND RHINITIS INDUCED BY SELECTIVE IMMEDIATE REACTIONS TO PARACETAMOL AND NON-STEROIDAL ANTI-INFLAMMATORY DRUGS IN ASPIRIN TOLERANT SUBJECTS

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    Diana Pérez-Alzate

    2016-07-01

    Full Text Available In subjects with non-steroidal anti-inflammatory drugs (NSAIDs- exacerbated respiratory disease (NERD symptoms are triggered by acetyl salicylic acid (ASA and other strong COX-1 inhibitors, and in some cases by weak COX-1 or by selective COX-2 inhibitors. The mechanism involved is related to prostaglandin pathway inhibition and leukotriene release. Subjects who react to a single NSAID and tolerate others are considered selective responders, and often present urticaria and/or angioedema and anaphylaxis (SNIUAA. An immunological mechanism is implicated in these reactions. However, anecdotal evidence suggests that selective responders who present respiratory airway symptoms may also exist.Our objective was to determine if subjects might develop selective responses to NSAIDs/paracetamol that manifest as upper/lower airways respiratory symptoms. For this purpose we studied patients reporting asthma and/or rhinitis induced by paracetamol or a single NSAID that tolerated ASA. An allergological evaluation plus controlled challenge with ASA was carried out. If ASA tolerance was found, we proceeded with an oral challenge with the culprit drug. The appearance of symptoms was monitored by a clinical questionnaire and by measuring FEV1 and/or nasal airways volume changes pre and post challenge. From a total of 21 initial cases, we confirmed the appearance of nasal and/or bronchial manifestations in ten, characterised by a significant decrease in FEV1% and/or a decrease in nasal volume cavity after drug administration. All cases tolerated ASA.This shows that ASA tolerant subjects with asthma and/or rhinitis induced by paracetamol or a single NSAID without skin/systemic manifestations exist. Whether these patients represent a new clinical phenotype to be included within the current classification of hypersensitivity reactions to NSAIDs requires further investigation.

  19. Asthma and Rhinitis Induced by Selective Immediate Reactions to Paracetamol and Non-steroidal Anti-inflammatory Drugs in Aspirin Tolerant Subjects

    Science.gov (United States)

    Pérez-Alzate, Diana; Blanca-López, Natalia; Doña, Inmaculada; Agúndez, José A.; García-Martín, Elena; Cornejo-García, José A.; Perkins, James R.; Blanca, Miguel; Canto, Gabriela

    2016-01-01

    In subjects with non-steroidal anti-inflammatory drugs (NSAIDs)- exacerbated respiratory disease (NERD) symptoms are triggered by acetyl salicylic acid (ASA) and other strong COX-1 inhibitors, and in some cases by weak COX-1 or by selective COX-2 inhibitors. The mechanism involved is related to prostaglandin pathway inhibition and leukotriene release. Subjects who react to a single NSAID and tolerate others are considered selective responders, and often present urticaria and/or angioedema and anaphylaxis (SNIUAA). An immunological mechanism is implicated in these reactions. However, anecdotal evidence suggests that selective responders who present respiratory airway symptoms may also exist. Our objective was to determine if subjects might develop selective responses to NSAIDs/paracetamol that manifest as upper/lower airways respiratory symptoms. For this purpose, we studied patients reporting asthma and/or rhinitis induced by paracetamol or a single NSAID that tolerated ASA. An allergological evaluation plus controlled challenge with ASA was carried out. If ASA tolerance was found, we proceeded with an oral challenge with the culprit drug. The appearance of symptoms was monitored by a clinical questionnaire and by measuring FEV1 and/or nasal airways volume changes pre and post challenge. From a total of 21 initial cases, we confirmed the appearance of nasal and/or bronchial manifestations in ten, characterized by a significant decrease in FEV1% and/or a decrease in nasal volume cavity after drug administration. All cases tolerated ASA. This shows that ASA tolerant subjects with asthma and/or rhinitis induced by paracetamol or a single NSAID without skin/systemic manifestations exist. Whether these patients represent a new clinical phenotype to be included within the current classification of hypersensitivity reactions to NSAIDs requires further investigation. PMID:27489545

  20. Alergia al látex: Manifestaciones clínicas en la población general y reactividad cruzada con alimentos Latex allergy: Clinical manifestations in the general population and reactivity crossed with foodstuffs

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    M. Anda

    2003-01-01

    Full Text Available La alergia al látex en los últimos años ha adquirido una gran importancia médica, no sólo para el personal sanitario sino también, aunque en menor medida, para la población general, debido a su ubicuidad. Son múltiples los objetos que manejamos en nuestra vida cotidiana que están fabricados con látex, y que por tanto pueden suponer un riesgo para los pacientes alérgicos a este material. Además del personal sanitario, existe otro subgrupo de pacientes en los que la alergia al látex tiene una mayor incidencia que en la población general; se trata de los pacientes multintervenidos, y especialmente los niños con espina bífida y mielomeningocele. Las manifestaciones clínicas de alergia al látex, abarcan un amplio abanico: dermatitis de contacto, urticaria, angioedema, rinoconjuntivitis, asma y en ocasiones anafilaxia, dependiendo la presencia de una u otra clínica de la vía de contacto con el látex y también del tiempo de exposición al mismo. Además como ocurre con otros aeroalergenos, existen múltiples reactividades cruzadas con alimentos, por lo que los pacientes alérgicos al látex en muchas ocasiones asocian alergias alimentarias que se deben diagnosticar, ya que su desconocimiento puede suponer un riesgo añadido para el paciente.Because of widespread latex manufacturing in the last decades, latex allergy has become an important clinical problem, not only in high-risk groups (health-workers but also among the general population. Latex is used to produce a large variety of natural rubber products (medical equipment, household gloves, condoms, balls and balloons,footwear, baby pacifiers… employed in the ordinary life, with high risk for patients allergic to latex. Among general population, children affected by myelomeningocele or spina bifida, have a higher risk to develop latex allergy. Clinical manifestations range from local reactions(contact dermatitis, urticaria, rhino-conjunctivitis, asthma, pharyngeal edema to

  1. Cheilitis glandularis in an African-American woman: response to antibiotic therapy.

    Science.gov (United States)

    Bender, Matthew M; Rubenstein, Melissa; Rosen, Ted

    2005-01-01

    A 52-year-old black woman presented with a 2-day history of lower lip swelling 5 days after starting a new medication, lisinopril. She had never experienced similar episodes in the past. She denied shortness of breath, tightening of the throat, swelling of the tongue, generalized cutaneous eruption, urticaria, or pruritus. She also denied symptoms consistent with facial paresis. Her past medical history was significant for hepatitis C infection, coronary artery disease, and hypertriglyceridemia. She had a 15 pack-year smoking history and denied both alcohol and drug abuse. She had never received a blood transfusion and was HIV negative. Physical examination disclosed a tender, swollen, and erythematous lower lip with induration, oozing, and crusting (Figure 1). Pinpoint openings evident throughout the lip surface exuded a clear, sticky, mucoid secretion. Tongue, parotid glands, and regional lymph nodes were normal. The working diagnosis was angioedema secondary to lisinopril. The presumptive offending drug was discontinued, and conservative therapy (topical clobetasol ointment, oral ranitidine, and oral fexofenadine) was initiated. Despite treatment, signs and symptoms persisted unabated. One week after initial presentation, a punch biopsy of her lower lip was taken to rule out granulomatous cheilitis and sarcoidosis. Histopathology included diffuse lymphohistiocytic infiltrate, minimal microabscess formation, and notable absence of granulomata. There was neither hypertrophy nor detectable abnormality of the salivary glands, with the exception of infiltrating mononuclear cells. Based on the clinical history and compatible pathologic findings, a diagnosis of cheilitis glandularis was made. Specifically, crusting and erosion clinically suggested a diagnosis of the superficial suppurative subtype of cheilitis glandularis. The patient received oral penicillin (dicloxacillin, 1.0 g/d) combined with oral fluoroquinolone (ciprofloxacin, 1.0 g/d). Within 2 weeks of

  2. Adverse drug reactions to ibuprofen: a case report

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    Khobragade Yadneshwar

    2016-02-01

    Full Text Available Ibuprofen is a commonly used drug available by prescription and over the counter for treatment of fever, joint pain, headache, migraine, inflammatory states. It is available in combination with paracetamol and various other drugs. Side effects associated with aspirin and non-steroidal anti-inflammatory drugs (NSAIDs are rash, gastrointestinal ulcers, hepatic toxicity, Steven Johnson syndrome, respiratory skin rashes, acute exacerbation of asthma and anaphylaxis. We have reported here severe distress hypersensitive reaction with ibuprofen induced hypersensitivity syndrome. Within two hours of consumption of ibuprofen patient developed severe bronchospasm, throat and laryngeal oedema leading to respiratory distress. He was treated with salbutamol, hydrocortisone, deriphylline and supportive oxygen, but did not respond and went into coma. Unlike acetaminophen, ibuprofen does not have any antidote hence managing adverse drug reactions (ADR due to ibuprofen is big challenge. Therefore understanding pathophysiology of ADR to Ibuprofen is necessary to manage the patient. Literature in the field of allergic drug reaction shows that epinephrine, a physiological antagonist of histamine is the first drug of choice for the treatment of allergic or drug induced angioedema, laryngeal oedema and bronchospasm due to its direct action on target organs. Such reactions should therefore be managed by epinephrine without loss of time. ADR due to ibuprofen could be prevented by (a avoiding unnecessary intake of drug, (b educating patients / families and public about adverse drug reactions (c surveillance and monitoring of drug reactions (d record keeping (e drug audit and (f reporting of ADR to state/central pharmacovigilance agency. We do observe doctors having misconception about adrenaline, its actions, usage and side effects especially cardio-vascular, hence are reluctant to use. But in severe violent adverse drug reaction we have to use our wisdom and judgement

  3. Clinical Protests Food Allergy in Children

    Directory of Open Access Journals (Sweden)

    P Ataei

    2014-04-01

    immediate reactions to food antigens, such as rash, urticaria, angioedema, or anaphylaxis. However, many children suffer enteropathy without immediate reactions, and it is important to recognize that food allergic entropathy often occurs in the absence of systemic signs of food allergy. Thus, skin prick tests may be negative and specific IgE undetectable.  Review of growth records in classic CMSE often shows a period of good weight gain prior to the onset of symptoms, followed by downward drift against the centiles until antigen exclusion is adequate. This presentation probably represents the loss of initially established oral tolerance.  

  4. Severe asthma and the omalizumab option

    Directory of Open Access Journals (Sweden)

    Johnston Chambless

    2008-05-01

    Full Text Available Abstract Atopic diseases and asthma are increasing at a remarkable rate on a global scale. It is now well recognized that asthma is a chronic inflammatory disease of the airways. The inflammatory process in many patients is driven by an immunoglobulin E (IgE-dependent process. Mast cell activation and release of mediators, in response to allergen and IgE, results in a cascade response, culminating in B lymphocyte, T lymphocyte, eosinophil, fibroblast, smooth muscle cell and endothelial activation. This complex cellular interaction, release of cytokines, chemokines and growth factors and inflammatory remodeling of the airways leads to chronic asthma. A subset of patients develops severe airway disease which can be extremely morbid and even fatal. While many treatments are available for asthma, it is still a chronic and incurable disease, characterized by exacerbation, hospitalizations and associated adverse effects of medications. Omalizumab is a new option for chronic asthma that acts by binding to and inhibiting the effects of IgE, thereby interfering with one aspect of the asthma cascade reviewed earlier. This is a humanized monoclonal antibody against IgE that has been shown to have many beneficial effects in asthma. Use of omalizumab may be influenced by the cost of the medication and some reported adverse effects including the rare possibility of anaphylaxis. When used in selected cases and carefully, omalizumab provides a very important tool in disease management. It has been shown to have additional effects in urticaria, angioedema, latex allergy and food allergy, but the data is limited and the indications far from clear. In addition to decreasing exacerbations, it has a steroid sparing role and hence may decrease adverse effects in some patients on high-dose glucocorticoids. Studies have shown improvement in quality of life measures in asthma following the administration of omalizumab, but the effects on pulmonary function are

  5. A STUDY ON THE CLINICO-EPIDEMIOLOGICAL ASPECTS AND EVIDENCE OF AUTOIMMUNITY IN PATIENTS WITH MULTIPLE DRUG ALLERGY SYNDROME

    Directory of Open Access Journals (Sweden)

    Sukumarakurup

    2015-11-01

    Full Text Available Though multiple drug allergy syndrome or multiple drug hypersensitivity syndrome has been described as a distinct entity, not much data is available on the same, especially in Indian literature. AIMS To study the incidence of multiple drug allergy syndrome among patients with cutaneous adverse drug reaction (CADR attending a referral centre and to study its clinical and epidemiological features and evidence of autoimmunity. METHODS All patients admitted in Dermatology ward of our tertiary care hospital for a 2- year period from 1st August 2012 to 31st July 2014 with CADR were studied for documented evidence of CADR to two or more unrelated drugs. The subjects were included in this prospective study after clearance from institutional ethics committee and written informed consent from study subjects. The cases identified as multiple drug allergy syndrome were studied in a more detailed manner and were evaluated for comorbidities with special reference to autoimmune diseases and human immunodeficiency virus infection. The clinical patterns and the common offending drugs were studied. RESULTS During the two-year study period, 10 out of the 94 patients with CADR included in the study were found to be belonging to the category of multiple drug allergy syndrome (After strict scrutiny with a clear female predilection (70%. The different reaction patterns in patients with multiple drug allergy syndrome were fixed drug eruption, urticaria, erythema multiforme, angioedema, drug reaction with eosinophilia and systemic symptoms (DRESS and exfoliative dermatitis. Six out of ten patients with multiple drug allergy syndrome showed positivity on antinuclear antibody profile and two were diagnosed to have systemic lupus erythematosus. CONCLUSIONS A 10.6% of those admitted with CADR have multiple drug allergy syndrome, which exists as a separate entity. Female predominance and the evidence of autoimmune manifestations in the majority underscores the need to

  6. Complement analysis 2016: Clinical indications, laboratory diagnostics and quality control.

    Science.gov (United States)

    Prohászka, Zoltán; Nilsson, Bo; Frazer-Abel, Ashley; Kirschfink, Michael

    2016-11-01

    In recent years, complement analysis of body fluids and biopsies, going far beyond C3 and C4, has significantly enhanced our understanding of the disease process. Such expanded complement analysis allows for a more precise differential diagnosis and for critical monitoring of complement-targeted therapy. These changes are a result of the growing understanding of the involvement of complement in a diverse set of disorders. To appreciate the importance of proper complement analysis, it is important to understand the role it plays in disease. Historically, it was the absence of complement as manifested in severe infection that was noted. Since then complement has been connected to a variety of inflammatory disorders, such as autoimmune diseases and hereditary angioedema. While the role of complement in the rejection of renal grafts has been known longer, the significant impact of complement. In certain nephropathies has now led to the reclassification of some rare kidney diseases and an increased role for complement analysis in diagnosis. Even more unexpected is that complement has also been implicated in neural, ophtalmological and dermatological disorders. With this level of involvement in some varied and impactful health issues proper complement testing is clearly important; however, analysis of the complement system varies widely among laboratories. Except for a few proteins, such as C3 and C4, there are neither well-characterized standard preparations nor calibrated assays available. This is especially true for the inter-laboratory variation of tests which assess classical, alternative, or lectin pathway function. In addition, there is a need for the standardization of the measurement of complement activation products that are so critical in determining whether clinically relevant complement activation has occurred in vivo. Finally, autoantibodies to complement proteins (e.g. anti-C1q), C3 and C4 convertases (C3 and C4 nephritic factor) or to regulatory proteins

  7. Complement analysis 2016: Clinical indications, laboratory diagnostics and quality control.

    Science.gov (United States)

    Prohászka, Zoltán; Nilsson, Bo; Frazer-Abel, Ashley; Kirschfink, Michael

    2016-11-01

    In recent years, complement analysis of body fluids and biopsies, going far beyond C3 and C4, has significantly enhanced our understanding of the disease process. Such expanded complement analysis allows for a more precise differential diagnosis and for critical monitoring of complement-targeted therapy. These changes are a result of the growing understanding of the involvement of complement in a diverse set of disorders. To appreciate the importance of proper complement analysis, it is important to understand the role it plays in disease. Historically, it was the absence of complement as manifested in severe infection that was noted. Since then complement has been connected to a variety of inflammatory disorders, such as autoimmune diseases and hereditary angioedema. While the role of complement in the rejection of renal grafts has been known longer, the significant impact of complement. In certain nephropathies has now led to the reclassification of some rare kidney diseases and an increased role for complement analysis in diagnosis. Even more unexpected is that complement has also been implicated in neural, ophtalmological and dermatological disorders. With this level of involvement in some varied and impactful health issues proper complement testing is clearly important; however, analysis of the complement system varies widely among laboratories. Except for a few proteins, such as C3 and C4, there are neither well-characterized standard preparations nor calibrated assays available. This is especially true for the inter-laboratory variation of tests which assess classical, alternative, or lectin pathway function. In addition, there is a need for the standardization of the measurement of complement activation products that are so critical in determining whether clinically relevant complement activation has occurred in vivo. Finally, autoantibodies to complement proteins (e.g. anti-C1q), C3 and C4 convertases (C3 and C4 nephritic factor) or to regulatory proteins

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

    Directory of Open Access Journals (Sweden)

    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

  9. Clinical epidemiological characteristics of chronic urticaria: report of 535 cases%535例慢性荨麻疹临床流行病学特征分析

    Institute of Scientific and Technical Information of China (English)

    罗婕; 郝飞; 宋志强; 钟华; 尹锐; 黄秀英; 李垚莹; 程良金; 向明明; 王莉

    2011-01-01

    % ), and others 54 (10. 0% ), whereas 263 (49. 2%) of the participants could not identify any trigger. The most common type was chronic spontaneous urticaria (CSU) , accounting for 294 (55. 0% ), followed by demographic urticaria factitia 120 (22. 4% ). While the rarest type was delayed pressure urticaria, vibratory urticaria and exercise induced urticaria type. An-gioedema in CU accounted for 105 (19.6% ), 70 (21.1% ) were female, and 76 (25.9% ) were CSU with it. Positive autologous serum skin test (ASST) was accounted for 49. 6%. Conclusion The high prevalence of CU is in the young populations, with various clinical manifestations. Coexisting with angioedema is not uncommon. Investigating CU clinical epidemiological characteristics is helpful to guide the therapy strategies.

  10. [Health lips beautiful smile: Conditions to know].

    Science.gov (United States)

    Czerninski, R

    2016-04-01

    Diagnosis and management of lip pathologies should be part of the dentists' responsibilities. Lip pathologies might affect patient's function, appearance and quality of life. The routine clinical examination of the oral cavity includes lip examination--changes in lip color, size or texture should be recognized during the examination, as well as noticing any habit such as lip licking or mouth breathing.The aim of the review is to present common external lip lesions. Color changes--oral melanotic macule is a localized benign pigmented lesion. It might be similar to early melanoma therefore if it is a new lesion, growing or large it should be excised. White and red changes are usually seen at the lower lip which is exposed to the sun light mainly among light skin individuals. The color changes are showing epithelial and subepithlial permanent changes and is called Solar cheilitis. As a potentially malignant disorder the patient should be carefully examined and under follow- ups. These patients are also advised to reduce sun exposure and use sun screen lipsticks. Lichen planus of the lip can be seen as red and white changes the lips are less prevalent site of the disease and usually the patients will have in intra oral involvement as well. Size changes--angioedema is a quick enlargement of the lip sometimes the face as well. It is an allergic reaction to food drugs and other antigens and resolves spontaneously with 72 hours, but when the upper airway is involved an immediate emergency treatment should be given. A chronic swelling of the lip/lips may be a sign of orofacial granulomatosis--a complicated condition with an unknown etiology, diagnosed by a biopsy and elimination of other diagnoses. Treatment includes steroids in various forms or a combination of surgery. Changes of the epithelial surface of the lip can be a result of dry lips in a hot or dry environment or due to mouth breathing. Lip licking might worsen the situation by secondary infection. Angular

  11. Clinical Observation of Diphenhydramine Combined with Prednisone in the Prevention of Iodine-containing Contrast Agent Allergic Reactions%苯海拉明联合泼尼松预防碘造影剂过敏反应的临床观察

    Institute of Scientific and Technical Information of China (English)

    胡立禄; 高全清; 刘振良

    2016-01-01

    OBJECTIVE:To observe the success rate and safety of diphenhydramine combined with prednisone in the preven-tion of iodine-containing contrast agent allergic reactions. METHODS:1 day before surgery,42 patients with positive iodine aller-gy test was given 40 mg Prednisone tablet,orally,3 times a day,50 mg diphenhydramine was given by intramuscular injection and 1 ml iodine contrast by intravenous injection(allergy test performed again)1 h before surgery. After all patients used iodine con-trast in 15 min,vascular interventional treatment was conducted if there was no bronchospasm,angioedema,leather ball sample itchy rash,hypotension,itching and other allergic reactions. Prevention success rate were observed,and the incidence of adverse re-actions was recorded. RESULTS:Prevention success rate was 90.48%,the incidence of adverse reactions was 7.14%,and it self-improved after stopping drugs. CONCLUSIONS:Diphenhydramine combined with prednisone has high success rate in the pre-vention of iodine-containing contrast agent allergic reactions,with good safety.%目的:观察苯海拉明联合泼尼松预防碘造影剂过敏反应的成功率和安全性。方法:42例碘过敏试验呈阳性患者于术前1 d给予泼尼松片40 mg,口服,每日3次,术前1 h给予苯海拉明50 mg,肌内注射,并同时静脉注射碘造影剂1 ml(再次进行过敏试验)。所有患者使用碘造影剂15 min后若不存在支气管痉挛、血管性水肿、皮团样皮疹、低血压、瘙痒等过敏反应,方可行血管介入治疗。观察42例患者预防成功率及不良反应发生情况。结果:预防成功率为90.48%;不良反应发生率为7.14%,经停药后症状均自行好转。结论:苯海拉明联合泼尼松预防碘造影剂过敏反应具有较高的成功率,安全性较好。

  12. 对错误溶栓患者临床转归及安全性的观察%Misdiagnosis of stroke in intravenous rt-PA treated patients: safety and outcomes

    Institute of Scientific and Technical Information of China (English)

    周颖奇; 单培佳; 单连标

    2013-01-01

    Objective To determine the incidence, causes, safety and the outcome of patients with stroke mimic ( SM ) who received i. v recombinant tissue plasminogen activator ( rt-PA ). Methods A retrospective analysis was conducted of 91 consecutive patients in the hospital over 2 years period whom were treated with IV rt-PA ( IVT ) within 4. 5 h after stroke symptom on set. The frequency, clinical characteristics, safety, and outcome of stroke mimic patients were determined. All available previously published studies in which the incidence, causes, safety and the clinical outcome of IVT in SMs being investigated were also evaluated together. Results Three of 91 patients( 3. 3% )had final diagnose of SM which include Todds paralysis, isolated cranial nerve palsy and drug intoxication. These stroke mimic patients had no intracranial hemorrhage and had more excellent functional outcomes than patients with " real" acute ischemic stroke ( AIS ) after IVT. A pooled analysis of previously published data showed that 252 SMs ( 6. 0% ) were found in 4200 AIS patients with IVT and only one angioedema was observed. Not any intracerebral hemorrhage had occurred in these SMs. Patients with SM had a good safety profile and outcomes when treated with rt PA. Seizures( 31.7% ,80/252 ), conversion disorderr (24. 2% ,60/252 ) and migraine( 21. 0% , 53/252 ) were the 3 most common final diagnoses in SMs. Conclusion SMs were infrequent among AIS patients with IVT, and their treatment had favorable safety and outcomes.%目的 了解重组组织纤溶酶原激活物(rt-PA)静脉溶栓患者中貌似卒中(SM)患者的发生率、安全性、临床转归及原因.方法 对2010年4月-2012年3月发病4.5 h内采用静脉溶栓治疗的急性脑梗死患者资料进行回顾性分析.对其中诊断为貌似卒中的发生率、病因、治疗安全性及临床转归情况进行了统计分析.同时收集了2003-2012年全球公开发表的同类研究文献,对其中的数

  13. 常见预防接种异常反应及监测分析%Monitoring Analysis of Abnormal Reaction and Common Inoculation

    Institute of Scientific and Technical Information of China (English)

    余东

    2015-01-01

    Objective To analysis the common abnormal vaccination reaction and response to these anomalies reasonable monitor-ing, promote the scientific and reasonable getting vaccinated. Methods In April 2012 to December, the region of vaccination vac-cine were retrospectively analyzed. Results The region between Apr 2012 and Dec 14387 cases was preventive vaccination, 106 cases suspected immunization abnormal reaction occurred, was 0.74%. With the general reaction was 85 cases, was 80.2%, abnor-mal reaction 15 cases, was 14.2%. No quality accident of vaccine, vaccination accidents. The general reaction, mainly was the fever/red/induration 73 cases (85.9%), followed by allergic skin rashes 5 cases, was 5.9%;Abnormal reaction angioedema was most 10 cases, was 66.7%.Conclusion The region of inoculation against common abnormal reaction and monitoring analysis can reduce the adverse reaction appeared in the process of vaccination, improve the success rate of vaccination.%目的:分析常见预防接种异常反应并对这些异常反应进行合理的监测,促进科学合理的进行预防接种。方法于2012年2~12月对该地区预防接种的疫苗进行了回顾性分析。结果该地区2012年2~12月共预防接种疫苗14387例,发生疑似预防接种异常反应106例,发生率为0.74%。其中以一般反应为多见,共85例,占80.2%,异常反应15例,占14.2%。无疫苗质量事故、预防接种事故发生。发生不良反应的106例中,85例为一般反应,主要是发热/红肿/硬结73例,占85.9%,其次是过敏性皮疹5例,占5.9%;异常反应中最多的是血管性水肿为10例,占异常反应总数的66.7%。结论对该地区的常见预防接种异常反应及监测分析可以减少预防接种过程中出现的不良反应,提高接种的成功率。

  14. 国内流脑疫苗预防接种不良反应评价%A Review on Adverse Events Following Immunization of Meningococcal Vaccines

    Institute of Scientific and Technical Information of China (English)

    杨声坪; 李婧媛; 闫先侠; 杨克虎; 刘雅莉

    2011-01-01

    Objective To evaluate the adverse events following immunization (AEFI) of meningococcal vaccines, so as to provide references for the appraisal and treatment of AEFI. Methods The Chinese Bio-medicine Database (1978 to April, 2010), China Journal Full-text Database (1994 to April, 2010), VIP Database (1989 to April, 2010) and WangFang database (1988 to April, 2010) were fully searched, and the references listed in original studies were searched manually as well. Then two reviewers independently screened studies and abstracted relevant data. Results A total of 52 articles involving 61 cases were included. Among the AEFI cases, 72.13% were hypersensitive response, including henoch-schonlein purpura (accompanied with or without nephritis), anaphylactic shock, allergic eruption, angioedema, local allergic reaction and so on. Conclusion The results of this study show that meningococcal vaccines may result in AEFI. However, most AEFI are temporary and can be cured after treatment in time. In general, meningococcal vaccine is safe, but monitoring and treatment for AEFI are necessary.%目的 评价流脑疫苗预防接种不良反应(adverse events following immunization,AEFI),以了解流脑疫苗AEFI发生的情况,为AEFI的鉴定和处理提供参考.方法 全面检索中国生物医学文献数据库(1978~2010.4)、中国期刊全文数据库(1979~2010.4)、维普数据库(1989~2010.4)和万方数据知识服务平台(1988~2010.4),并追索纳入研究的参考文献,由两位评价员独立筛选合格文献并提取相关信息进行分析.结果 共纳入52篇文献,合计61例患者.其所发生的流脑疫苗AEFI中,72.13%为过敏反应,包括过敏性紫癜(合并或不合并肾炎)、过敏性休克、过敏性皮疹、血管性水肿、局部过敏性反应等.结论 本研究结果提示,虽然流脑疫苗预防接种可能发生AEFI,但其大多为一过性损害,经及时抢救和适当治疗后可恢复或痊愈.流脑疫苗的免疫接种总

  15. SP 04-1 THE ROLE OF NATRIURETIC PEPTIDES IN THE PATHOGENESIS OF CARDIOVASCULAR DISEASES.

    Science.gov (United States)

    Kobalava, Zhanna

    2016-09-01

    NEPi yields broader effects than anticipated and explains why NEPi is best combined with the inhibition of other vasoactive compounds. Angiotensin receptor blockers (ARB) do not disrupt bradykinin metabolism as much as ACE-inhibitors, and some patients with ACE-inhibitor-associated angioedema can be switched over to an ARB without the occurrence of angioedema. A novel class of drugs that combines the actions of NEP inhibitors and ARB, known as angiotensin receptor blockade with neutral endopeptidase inhibition (ARNi) was developed. LCZ696 is the first compound of this category. Novel ARNi-based therapeutic strategies are expected contribute to optimize control of CVD and of their outcomes. PMID:27643143

  16. Immediate hypersensitivity reactions induced by iodinated contrast media%碘造影剂所致速发型过敏反应

    Institute of Scientific and Technical Information of China (English)

    周细平; 李宏

    2011-01-01

    Iodinated contrast media (ICM) are the most commonly used drugs in diagnostic visualisation technique.ICM may be classified as ionic and nonionic according to their chemical structure or high-osmolar, low-osmolar.and iso-osmolar according to their osmoWity.ICM are generally considered to be relatively safe.but serious adverse reactions may occur, such as severe immediate hypersensitivity reactions.Severe immediate hypersensitivity reactions may lead to angioedema, unconsciousness, profound hypotension, arrhythmias, respiratory arrest and cardiac arrest, and so on.The incidence rates of severe immediate hypensensitivity reactions to ionic and nonionic ICM are O.1%- 0.4% and 0.02%- 0.04% , respectively.The most significant risk factor for an immediate hypersensitivity reaction is a history of a hypersensitivity reaction to contrast media.Other risk factors are the history of asthma and allergic history to drug or food, and so on.The mechanism of ICM-induced immediate hypersensitivity reactions may be reWed to histamine release from basophil and mast cells.Histamine release might be due to a direct membrane effect associated with the solution osmolarity or the chemical structure of ICM, an activation of the complement system, as well as the formation of bradykinin and the activation of antigen-antibody reactions mediated by IgE.The preventive and therapeutic measures are as follows: (1) application of Iow-osmolar or iso-osmolar and noruonic ICM as far as possible; (2) the skin test and the premedication such as glucocorticoid and antihistamine to the patients with risk factors should be recommended; (3) the patients with moderate or severe hypersensitivity reactions to ICM should receive the symptomatic and supportive treatments.%碘造影剂是影像学诊断中最常用的药物,根据分子结构可分为离子和非离子型,根据渗透压可分为高渗、低渗和等渗型.碘造影剂通常较为安全,但也可发生严重不良反

  17. Cloning and prokaryotic expression of parvalbumin from silver carp (Hypophthalmichthy molitrix) skeletal muscle%鲢小清蛋白的cDNA克隆及在大肠杆菌中的原核表达

    Institute of Scientific and Technical Information of China (English)

    王慈; 曹敏杰; 郑晓江; 詹春兰; 刘光明; 蔡秋凤

    2014-01-01

    Parvalbumin (PV) is a major fish allergen that is involved in IgE-mediated food hypersensitivity. Sensitized individuals can develop some clinical symptoms including urticaria, angioedema, asthma, and even fatal anaphylaxis after ingestion of trace quantitiesof fish. As the largest producer and consumer of fresh water fish in the world, a high number of Chinese people suffer from allergies associated with consumption of fresh water fish. Despite this, little is known about the allergens in freshwater fish products that are available in China. We extracted total RNA from silver carp (Hypophthalmichthys molitrix) skeletal muscle, and synthesized first-strand cDNA by reverse transcriptase with an oligo (dT)18 primer. Some specific primers were designed based on the sequences of silver carp PV mRNA (GenBank nos. FJ216937 and FJ216938). Using these primers and the synthesized cDNA, two PV isoform genes (PVI and PVII) were cloned. The full-length coding region of both PVs was 330 bp, which encoded a protein of 109 amino acid resi-dues. The PCR products were cloned into a pMD18-T vector for sequencing. Both the positive plasmid and the plasmid pET28a were digested by Nde I and BamH I.The target genes were subcloned into pET28a for expression in [E.coli BL21 (DE3)] by 1 mmol/LIPTG induction at 37℃ for 4 h. The two target protein bands were~13 kD, which was con-sistent with the predicted size. SDS-PAGE analysis indicated that the recombinant PVI and PVII both existed in the soluble fraction of the proteins. The recombinant PVI and PVII were further purified by Ni-NTA agarose affinity chromatography, and the target proteins were eluted by 100 mmol/L imidazol. Both purified proteins yielded a single band on SDS-PAGE. Similar to the native PV, the recombinant proteins reacted strongly with anti-silver carp PV monoclonal antibody in the western blot analysis, suggesting that the recombinant PVI and PVII have strong IgG binding activity. Thus, we obtained two isoforms of

  18. Surveillance on adverse events following immuhization in Yuexiu district of Guangzhou city%广州市越秀区预防接种副反应监测分析

    Institute of Scientific and Technical Information of China (English)

    辜洁妮; 湛柳华; 刘淑勤

    2011-01-01

    Guangzhou city from 2006 to 2009 and analyzed. Results Totally 286 AEFI cases were reported from 2006 to 2009 by the surveillance system. The estimated incidence of AEFI was 12.12/100 000 dose vaccination and most of the AEFI were reported during March to August. The AEFI reports involved 21 kinds of vaccine. Among all AEFI reported, there were 134 (5.67/100 000 doses) general response, 87 (3.68/100 000 doses) abnormal reaction, and 65 (2. 75/100 000 doses) coincidental reaction. The vaccines with first three frequent AEFI were Areinfluenza H1N1 influenza vaccine(75/100 000 doses) ,MR vaccine( 67.11/100 000 doses) and DTwP vaccine(66. 99/100 000 doses). The general reactions were fever (49. 25% ), local swelling and induration (29. 1% ), and fever with local swelling and induration (11. 94% ). The first three abnormal reactions were allergic skin rash, aseptic abscess, angioedema. The main coincidental reaction was upper respiratory infection. There were significant differences in the incidence of AEFI between vaccination with and without dilution^2 =5. 68 ,P = 0. 017 ) , and between different injection way (x2 = 4. 7, P = 0. 03). The children with allergy history were more likely to have abnormal reaction than general response with an odds ratio of 2.1. Conclusion No vaccine quality problem and mistake vaccination were found in AEFI surveillance. The vaccines are used safely and the incidence of AEFI is within the expected range in Yuexiu district of Guangzhou city.

  19. 2009至2012年江苏省扬州市广陵区疑似预防接种异常反应监测分析%Analysis of adverse events following immunization from 2009 to 2011 in Guangling District,Yangzhou City, Jiangsu Province

    Institute of Scientific and Technical Information of China (English)

    钱颖

    2013-01-01

    Objective To evaluate the monitoring quality of adverse events following immunization (AEFI) from 2 0 0 9 to 2 0 1 1 in Guangling District,Yangzhou City,Jiangsu Province preliminarily.Methods The data of AEFI in Guangling District from 2009 to 2011 were collected through the AEFI information system of China and analyzed by descriptive epidemiological method.Results A total of 24 kinds of vaccine were inoculated from 2009 to 2012 in Guangling District,the accumulative total inoculation doses were 732 005,the accumulative total AEFI cases were 388,the reporting rate was 53.7 per 100 000 doses.In seven state indicators of AEFI monitoring system,reporting rate within 48 hours,investigation rate within 48 hours,and classification rate were 100.0%.The input rate of questionnaire within 3 days,upload rate of investigation report within 7 days,integrity rate of key project in questionnaire,and report coverage rate in districts were not up to the standard of AEFI during 2009.The input rate of questionnaire within 3 days and upload rate of investigation report within 7 days were increased greatly during 2010,but were not up to the standard of AEFI yet.The completion rate of all indicators of AEFI monitoring system reached 100.0% since 2011.Of 388 cases of AEFI,the common adverse reactions were found in 330 cases (85.0%),the main clinical manifestations were fever,red swelling and induration in injection area.The abnormal reactions were found in 50 cases (12.9%),the clinical manifestations were allergic skin rashes (40 cases,80.0%),angioedema (3 cases,6.0%),BCG abscess (2 cases,4.0%),morbilliform rash (2 cases,4.0%),allergic purpura (1 case,2.0%),febrile convulsion (1 case,2.0%),and diarrhea (1 case,2.0%).The coincidental events occurred in 8 cases (2.1%).The top five vaccines associated with reporting rate of AEFI were pneumococcal 7-valent conjugate vaccine (PCV-7,507.9/100000),23-valent pneumococcal polysaccharide vaccine (343.2/100 000

  20. 中国2010年疑似预防接种异常反应监测数据分析%Analysis on Adverse Events Following Immunization Surveillance in China, 2010

    Institute of Scientific and Technical Information of China (English)

    武文娣; 刘大卫; 李克莉; 许涤沙; 郑景山; 曹玲生; 曹雷; 袁平; 王华庆

    2012-01-01

    Objective This study is to analyze the occurrence features of adverse event following immunization (AEFI) in China, and to evaluate the implementation of AEFI surveillance system, and the immunization safety related to the national immunization program (NIP) vaccines. Methods 2010 AEFI data were collected through the national AEFI information system, which reported before March 13, 2012. The descriptive methodology was used in the study. Results 56 001 AEFI cases were reported, of which 1368 cases were serious AEFIs, 54 633 cases were non-serious AEFIs. 2561 counties (82.88% ) in all provinces but Tibet autonomous region reported AEFI cases. The reported rate within 48h was 95.35%, and the investigated rate within 48h was 98.72%. Cases ≤1 year old accounted for 63.43%, and the sex ratio (male to female) was 1.41:1. The cases often occurred in the third quarter of the year. The classification rate of AEFI was 99.72%. The different vaccines' estimated reported serious AEFI incidence rates were between 0.54 per million doses and 31.08 per million doses, and the estimated non-serious AEFI incidence rates were between 11.45 per million doses and 1494.38 per million doses. The common, minor vaccine reactions were mainly fever, local swelling and induration. The main diagnosis of rare, more serious vaccine reactions were anaphylactic rash, sterile abscess, lymphadenitis of Bacilli Calmette-Guerin vaccine (BCG), henoch-schonlein purpura and angioedema. The vaccine reactions often occurred in the vaccine day and Id after. In all rare, more serious vaccine reactions, the estimated reported rates of main vaccines were as follows: 0.24 per million doses for disseminated BCG infection, 0.21 per million for vaccine-associated paralytic poliomyelitis, 6.55 per million doses for sterile abscess of diphtheria, tetanus pertussis combined vaccine, 0.25 per million doses for henoch-schonlein purpura of measles attenuated live vaccine, 0.03 per million doses for therombocytopenic

  1. Evaluation of safety of haemophilus influenza type b(Hib) conjugate vaccine in postmarketing based on the immunization information management system%基于预防接种信息管理系统的b型流感嗜血杆菌结合疫苗上市后安全性评价

    Institute of Scientific and Technical Information of China (English)

    汪志国; 马福宝; 张晋琳; 于静; 康国栋; 高君

    2015-01-01

    合疫苗具有良好的安全性,但需关注预防接种后过敏性休克、喉头水肿等急性过敏性反应的发生.%Objective 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.Methods 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 x2 tests.Results 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