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Angioedema urticaria [keywords]
- Neprilysin inhibition in chronic kidney disease. [REVIEW]
- Nephrol Dial Transplant 2014 Aug 18.
Despite current practice, patients with chronic kidney disease (CKD) are at increased risk of progression to end-stage renal disease and cardiovascular events. Neprilysin inhibition (NEPi) is a new therapeutic strategy with potential to improve outcomes for patients with CKD. NEPi enhances the activity of natriuretic peptide systems leading to natriuresis, diuresis and inhibition of the renin-angiotensin system (RAS), which could act as a potentially beneficial counter-regulatory system in states of RAS activation such as chronic heart failure (HF) and CKD. Early NEPi drugs were combined with angiotensin-converting enzyme inhibitors but were associated with unacceptable rates of angioedema and, therefore, withdrawn. However, one such agent (omapatrilat) showed promise of NEP/RAS inhibition in treating CKD in animal models, producing greater reductions in proteinuria, glomerulosclerosis and tubulointerstitial fibrosis compared with isolated RAS inhibition. A new class of drug called angiotensin receptor neprilysin inhibitor (ARNi) has been developed. One such drug, LCZ696, has shown substantial benefits in trials in hypertension and HF. In CKD, HF is common due to a range of mechanisms including hypertension and structural heart disease (including left ventricular hypertrophy), suggesting that ARNi could benefit patients with CKD by both retarding the progression of CKD (hence delaying the need for renal replacement therapy) and reducing the risk of cardiovascular disease. LCZ696 is now being studied in a CKD population.
- Immediate hypersensitivity reaction following liposomal amphotericin-B (AmBisome) infusion. [JOURNAL ARTICLE]
- Trop Doct 2014 Aug 19.
Liposomal amphotericin-B (AmBisome) is now becoming first choice for the treatment of visceral leishmaniasis (kala-azar) patients due to high efficacy and less toxicity. The reported incidence of hypersensitivity reactions to liposomal amphotericin-B (AmBisome), especially during therapy, is very rare. We report two patients with kala-azar: one developed breathing difficulties and hypotension followed by shock and the other had facial angioedema with chest tightness during treatment. Both patients were managed with immediate action of injection: adrenaline, diphenhydramine and hydrocortisone. In our experience, AmBisome can cause severe hypersensitivity reactions that warrant proper support and close supervision.
- Hereditary angioedema with F12 mutation: factors modifying the clinical phenotype. [JOURNAL ARTICLE]
- Allergy 2014 Aug 18.
Hereditary angioedema (HAE) with normal C1 inhibitor (C1Inh) associated with the c.983C>A and c.983C>G mutations of the F12 gene (HAE-FXII) is a rare condition, and presents with highly variable clinical expression. Based on data gathered from a large carrier cohort, we assessed the modifiers affecting the clinical phenotype.We analyzed clinical and biological data recorded from 118 mutation carriers (80 symptomatic and 38 asymptomatic), 58 non-carrier relatives from 40 families, and 200 healthy donors. Disease severity was scored in relation to frequency and location of edema, as well as age at disease onset. In order to predict HAE-FXII disease severity, we analyzed the biological phenotype (C1Inh, C4, spontaneous amidase, angiotensin-I converting enzyme [ACE], aminopeptidase P [APP], and carboxypeptidase N/M [CPN]) by means of logistic regression (Akaike information criterion) and odds ratio (OR).Meaningful variables contributed to HAE-FXII, with the kinin catabolism enzymes ACE and CPN exhibiting a significant inverse relationship with disease severity (OR=0.36, 95% CI 0.23-0.59, P<0.001; OR=0.58, 95% CI 0.36-0.91, P<0.05, respectively). CPN activities was 37.5 [28.5-41.3] nmol•mL(-1) •min(-1) and 38.5 [32.8-45.6] for HAE-FXII asymptomatic and symptomatic carriers respectively and 37.9 [30.5-43.7] nmol•mL(-1) •min(-1) for non-carriers. ACE activities was 58 [44-76] and 49 [35-59] nmol•mL(-1) •min(-1) for HAE-FXII asymptomatic and symptomatic carriers respectively, and 56 [49-66] nmol•mL(-1) •min(-1) for non-carriers.The HAE-FXII is associated with modifiers, e.g. kinin catabolism enzymes, ACE and CPN, different from those recognized in HAE with C1Inh deficiency. This article is protected by copyright. All rights reserved.
- [Exercise-induced urticaria and angioedema - case report.] [JOURNAL ARTICLE]
- Pneumonol Alergol Pol 2014; 82(5):467-471.
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.
- An allergic transfusion reaction with angioedema. [Journal Article]
- Transfusion 2014 Aug; 54(8):1916.
- Angioedema Related to Angiotensin Inhibitors. [JOURNAL ARTICLE]
- J Pharm Pract 2014 Aug 14.
Angiotensin inhibitors have been extensively evaluated in clinical trials and have demonstrated significant reductions in morbidity and mortality following myocardial infarction and stroke, as well as in patients with heart failure or who are at risk of cardiovascular disease. Further, both angiotensin-converting enzyme inhibitors (ACEIs) and angiotensin receptor blockers (ARBs) are frequently prescribed for the treatment of hypertension and to preserve renal function in patients with diabetes mellitus and chronic kidney disease. Angioedema is a known, but rare, adverse effect of ACEIs and ARBs. Therefore, it is important for clinicians to have a thorough understanding of risks and benefits of prescribing these medications, particularly in patients with a history of angioedema. This review describes the literature evaluating the incidence and cross-reactivity of angioedema with ACEIs and ARBs in order to provide guidance for clinical decision making.
- Another case of the rare complications of chronic lymphocytic leukemia: angioedema. [JOURNAL ARTICLE]
- Ann Biol Clin (Paris) 2014 Aug 1; 72(4):479-481.
Angioedema is a rare but may be serious (laryngeal edema). This is a recurrent edema, subcutaneous and/or submucosal, whose cause is a hereditary or acquired deficiency in C1 inhibiteur (C1 inhibitor fraction of complement). We present the case of a 56 years old patient who showed recurrent episodes of swelling of the face and hands in association with chronic lymphocytic leukemia stage A. The exploration of the complement pathway has allowed retaining the diagnosis of acquired angioedema type I. The association of angioedema and lymphoproliferative syndrome is rare; we present this interesting case to discuss it from the literature data.
- Transient angioedema of small bowel secondary to intravenous iodinated contrast medium. [Journal Article]
- Indian J Radiol Imaging 2014 Jul; 24(3):303-5.
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.
- Paediatric Hereditary Angioedema: A survey of UK service provision and patient experience. [JOURNAL ARTICLE]
- Clin Exp Immunol 2014 Aug 12.
Hereditary angioedema (HAE) is a rare disease characterised by episodes of potentially life-threatening angioedema. For affected children in the UK, there are relatively few data regarding disease prevalence, service organisation and the humanistic burden of the disease. To improve knowledge in these areas, we surveyed major providers of care for children with HAE. A questionnaire was sent to major paediatric centres, to determine patient numbers, symptoms, diagnostic difficulties, management and available services. In addition all patients at a single centre were given a questionnaire to determine the experiences of children and their families. 16 of 28 centres responded, caring for a total of 101 UK children. 7 children had experienced airway compromise. One third of patients were on long-term prophylactic medication, including C1 inhibitor prophylaxis in four children. 8 centres reported patients who were initially misdiagnosed. Broad differences in management were noted, particularly regarding indications for long-term prophylaxis and treatment monitoring. We also noted substantial variation in the organisation of services between centres, including the number of consultants contributing to patient care, the availability of specialist nurses, the availability of home therapy training and the provision of patient information. 10 of 12 patient/carer questionnaires were returned identifying three common themes: the need to access specialist knowledge, the importance of home therapy and concerns around the direct effect of angioedema on their life. To our knowledge this study represents the first dedicated survey of paediatric HAE services in the UK and provides useful information to inform the optimisation of services.