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Angioedema urticaria [keywords]
- Recurrent migratory angioedema as cutaneous manifestation in a familiar case of TRAPS: dramatic response to Anakinra. [Journal Article]
- Dermatol Online J 2013; 19(11):20405.
Background:Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) is a hereditary autoinflammatory syndrome characterized by recurrent episodes of fever and localized inflammation. Clinical presentation can be very variable in terms of duration of fever attacks, periodicity, and accompanying manifestations. One of the most characteristic symptoms is the occurrence of migrating skin rash with myalgia that is sustained by monocytic inflammation. Observations: We herein present the case of a family suffering from TRAPS who had been misdiagnosed for a long period of time and whose main symptom was migrating angioedema. Skin biopsy from one of the patients documented a monocytic panniculitis. All the living patients responded dramatically to anakinra treatment.
Conclusions:The classic symptom of migratory angioedema with myalgia in TRAPS can be produced by monocytic panniculitis.This manifestation is so characteristic of TRAPS that its occurrence, even in the absence of other manifestations, should prompt genetic analysis. Our patient's condition responded promptly to anakinra treatment.
- Pediatric hereditary angioedema. [JOURNAL ARTICLE]
- Pediatr Allergy Immunol 2013 Dec 9.
Hereditary angioedema (HAE) is a lifelong illness characterized by recurrent swelling of the skin, intestinal tract, and, ominously, the upper airway. It is caused by inadequate activity of the protein C1-inhibitor, with dysfunction in the kallikrein/bradykinin pathway underlying the clinical symptoms. In addition to the physical symptoms, patients experience significant decrements in vocational and school achievement as well as in overall quality of life. Symptoms often begin in childhood and occur by age 20 in most patients, but life-threatening attacks are uncommon in the pediatric population. The availability of new therapies has transformed the management of HAE.
- Q: Can an ARB be given to patients who have had angioedema on an ACE inhibitor? [Journal Article]
- Cleve Clin J Med 2013 Dec; 80(12):755-7.
- [Ultrasound assessment of acute abdominal pain in hereditary angioedema.] [LETTER]
- An Pediatr (Barc) 2013 Nov 28.
- Angiotensin Converting Enzyme-induced Angioedema - A Dangerous New Epidemic. [JOURNAL ARTICLE]
- Acta Derm Venereol 2013 Nov 21.
Angioedema is a sudden localised and often asymmetric swelling of the skin or mucous membranes caused by transient increased endothelial permeability causing plasma extravasation. In the last decades the incidence of severe angioedema involving the upper airways and even fatal outcome due to asphyxia has increased. This is mainly due to pharmaceuticals such as angiotensin converting enzyme-inhibitors, which are extensively used worldwide. Some aspects of the pathophysiology have been elucidated and the vasoactive molecule bradykinin is shown to be one of the main causative agents. The diagnosis is often delayed and traditional treatment usually ineffective. Complement C1 inhibitor concentrate and bradykinin receptor antagonists, normally used to treat patients with hereditary angioedema, have shown good results when used in patients with bradykinin-mediated angioedema. This review discusses the disease, prognosis and treatment options.
- Cost-utility analysis of Ruconest(®) (conestat alfa) compared to Berinert(®) P (human C1 esterase inhibitor) in the treatment of acute, life-threatening angioedema attacks in patients with hereditary angioedema. [JOURNAL ARTICLE]
- Postepy Dermatol Alergol 2013 Jun; 30(3):152-158.
Administration of human C1 esterase inhibitor (Berinert(®) P) from target import is the most widespread treatment strategy for patients with hereditary angioedema (HAE). However, a therapeutic health program including Ruconest(®) (conestat alfa) could shorten a patient's expectancy for a life-saving treatment.To evaluate the cost-utility of Ruconest(®) (conestat alfa) financed from public funds within the newly introduced therapeutic health program compared with Berinert(®) P (human C1 esterase inhibitor) in the treatment of acute angioedema attacks in adults with HAE.The cost-utility analysis from the Polish healthcare payer's perspective was performed for 1 year (2012). The costs and health outcomes were simulated for three pairs of eligible HAE patient groups (active treatment and corresponding placebo). The incremental costs of each intervention compared with placebo were listed together (direct or indirect comparisons between options were impossible due to limited clinical data available).The incremental cost-utility ratios (ICURs) for the evaluated interventions compared with placebo were as follows: EUR 15,226 per QALY (Ruconest(®)) and EUR 27,786 per QALY (Berinert(®) P). The probability of cost-utility (ICUR < EUR 24,279 per QALY) assessed for Ruconest(®) administered in the case of acute angioedema attack was 61% and 41% for Berinert(®) P.The administration of Ruconest(®) in acute life-threatening angioedema attacks is economically justified from the Polish healthcare payer's perspective, results in lower costs and is characterized by higher cost-utility probability compared with Berinert(®) P.
- Rifampin induced angioedema: a rare but serious side effect. [LETTER]
- Braz J Infect Dis 2013 Nov 22.
- Acute urticaria presenting in the emergency room of a general hospital. [JOURNAL ARTICLE]
- Eur J Intern Med 2013 Nov 22.
Acute urticaria is a common disorder that often prompts patients to seek treatment in the emergency room (ER). There are few data on acute urticaria presenting in ER.This study aimed to provide demographic and clinical data of patients presenting with acute urticaria at an ER of an Italian general hospital covering an area of about 90,000 inhabitants. The predictive factors of the length of stay in the ER had also been investigated.The database of ER patients was searched for urticaria by ICD-9 code and by keywords in the diagnosis description. All the medical records of the identified patients were reviewed and the length of stay in ER was noted.A total of 459 patients were admitted to ER with acute urticaria in a 1-year period corresponding to 1.01% of total ER visits and to 1.2 admission per day. Angioedema was present in 139 cases (30.3%), fever in 55 (12%). Twenty-nine patients fulfilled the criteria of anaphylaxis. Triggers could be identified in 193 cases (42%): drugs in 20.7%, insects bites (10.2%), foods (7.4%) and contact urticaria in 3.7%. Anaphylaxis (p<0.001), food (p<0.05) and drugs (p<0.05) as triggers were significant and independent predictive factors of the length of stay in ER.Patients with acute urticaria are frequently referred to the emergency room, but only in a few cases urticaria is associated with severe allergic manifestations. Drug and food hypersensitivity, together with anaphylaxis, are the best predictors of the length of stay in ER.
- Nanofiltered C1 esterase inhibitor for treatment of laryngeal attacks in patients with hereditary angioedema. [Journal Article]
- Am J Rhinol Allergy 2013 Nov; 27(6):517-21.
Laryngeal edema is a life-threatening manifestation of hereditary angioedema (HAE), an autosomal-dominant disorder caused by quantitative or functional C1 esterase inhibitor (C1 INH) deficiency. The preparation of nanofiltered C1 INH (C1 INH-nf) used in this study is indicated for routine prophylaxis against angioedema attacks in the United States and for treatment, preprocedure prevention, and routine prevention of HAE in Europe. The objective of this analysis was to evaluate the effectiveness and tolerability of C1 INH-nf when used for the treatment of laryngeal attacks.A post hoc analysis of an open-label treatment study evaluated the effectiveness of C1 INH-nf in the treatment of laryngeal attacks in patients with HAE. Outcomes included unequivocal or clinical relief rates and time from treatment to onset of relief. Data were compiled from this and three other studies for post hoc dosing and tolerability analyses. In all studies, C1 INH-nf at 1000 U was administered i.v., with a second 1000-U dose given after 60 minutes if indicated.In the open-label treatment study, 60 (50/84) and 77% (65/84) of attacks achieved unequivocal relief within 1 and 4 hours, respectively, after treatment. Time to unequivocal relief was shorter with prompt treatment. When C1 INH-nf was administered within 4 hours of symptom onset, clinical relief was achieved in 94% (45/48) of attacks within 4 hours after treatment. Of 265 attacks from the four studies, 62% received two 1000-U doses of C1 INH-nf. No serious adverse events occurring within 7 days after treatment were attributed to study drug, and only one patient required intubation after receiving C1 INH-nf (14.5 hours after symptom onset).This analysis supports that C1 INH-nf is an effective and well-tolerated therapy for laryngeal angioedema attacks.
- [Allergic adverse transfusion reactions in paediatrics, a 3-year study]. [English Abstract, Journal Article]
- Transfus Clin Biol 2013 Dec; 20(5-6):455-7.
In the transfused patients, in France, in 2011, allergy ranked as the third adverse transfusion reaction. In order to evaluate the incidence and symptomatology of allergic adverse transfusion reactions in the paediatric people, a study was performed.It was focused on patients under 18years of age cared for in hospitals of the Rhone-Alpes area. The national haemovigilance database (e-FIT) reports of allergic transfusion reactions were reviewed.From January 1st 2009 to December 31st 2011, among 2,165 reports, 141 (6.5%) adverse transfusion reaction reports were collected in paediatric patients. Sixty-eight (48.2%) indicated allergic reactions and corresponded to 64 recipients. As regards clinical manifestations, forty-eight (70.6%) indicated cutaneous signs only, 3 (4.4%) mentioned pulmonary signs only and 9 (13.2%) reported both. Urticaria was observed in 38 cases (55.9%). Bronchospasm was notified in 4 cases but there was no angioedema. As for the severity of reactions, one adverse transfusion reaction was severe (grade 2) and 2 were life-threatening (grade 3). The most involved blood component was the apheresis platelet concentrate (40 cases, 58.8%) followed by the red blood cell concentrate (17 cases, 25.0%) and the methylene blue-treated fresh-frozen plasma (11 cases, 16.2%).This study shows that among paediatric recipients, cutaneous signs are predominant in allergic adverse transfusion reactions and that the apheresis platelet concentrate is the most frequently involved blood component.