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Angioedema urticaria [keywords]
- Management of hereditary angioedema in pregnant women: a review. [Journal Article, Review]
- Int J Womens Health 2014.:839-48.
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.
- Tissue factor expression on the surface of monocytes from a patient with hereditary angioedema. [JOURNAL ARTICLE]
- J Dermatol 2014 Sep 16.
Hereditary angioedema (HAE) presents as severe angioedema, which is mostly due to the C1 inhibitor (C1-INH) gene mutations. Environmental factors, minor trauma and oral contraceptives have been reported to induce angioedema attack, but the trigger may often be uncertain. Activated factor XII controlled by C1-INH facilitates bradykinin generation and also regulates coagulation cascade, but the relationship between edema formation and coagulation is still unclear. We have described a 35-year-old female patient with HAE, presenting with frequent angioedema attacks in the absence of an apparent triggering factor. She showed higher levels of FDP and D-dimer during angioedema than those in remission. In addition, tissue factor (TF), an initiator of the extrinsic coagulation cascade, was expressed on the surface of monocytes. It was significantly higher than that of monocytes from healthy controls and tends to further increase during attacks. The expression of TF on monocytes may play a role in the induction of angioedema attacks in HAE by activating the coagulation pathway in association with reduced functions of C1-INH.
- Hereditary angioedema: A rare cause of recurrent abdominal pain. [Journal Article]
- Pak J Med Sci 2014 Sep; 30(5):1147-9.
Hereditary angioedema is a rare autosomal dominant inherited disease which is characterized by an episodic, self-limiting increase in vascular permeability. Symptoms commonly involve in nonpitting, nonpruritic skin swellings. We present a case of hereditary angioedema. The patinets complained of a recurrent abdominal pain without accompanying skin swelling whose diagnosis was delayed nearly 20 years and accepted an unnecessary surgery. According to the decreased serum C1-inhibitor and C4 concentration, the patient was finally diagnosed with hereditary angioedema type I. After treatment with danazole, the patient reported a significant decrease in the frequency of attacks and the severity of pain. HAE is a rare cause of abdominal pain, however it needs to be taken as one of the differential diagnosis of various acute abdomens in order to avoid unnecessary surgeries.
- Effects of a plasma-derived C1 esterase inhibitor on hemostatic activation, clot formation, and thrombin generation. [JOURNAL ARTICLE]
- Blood Coagul Fibrinolysis 2014 Sep 12.
Hereditary angioedema (HAE) is a rare, autosomal dominant disease in which C1 esterase inhibitor (C1 INH) is deficient or dysfunctional. Package inserts for nanofiltered C1 esterase inhibitor (C1 INH-nf) products contain warnings about thrombotic events. The objective of this study was to evaluate the effect of C1 INH-nf on hemostatic activation, clot formation, and thrombin generation. Ten healthy volunteers provided blood samples for thromboelastometry using the ROTEM system. Platelet-poor samples were prepared for thrombin generation studies. C1 INH-nf was added to samples at final concentrations of 0.14, 0.7, 1.4, 2.8, and 7.0 U/ml. Recombinant factor VIIa and prothrombin complex concentrate were used as procoagulant controls, and antithrombin and desirudin were used as anticoagulant controls. C1 INH-nf had no procoagulant effect on hemostasis based on thromboelastometry, regardless of the final concentration or activating reagent used (P > 0.05 for all comparisons of C1 INH-nf versus negative control). C1 INH-nf 2.8 and 7.0 U/ml concentrations had a statistically significant anticoagulant effect on maximum clot firmness (P < 0.05 for all comparisons of C1 INH-nf versus negative control), with a magnitude similar to that observed with desirudin. C1 INH-nf had no effect on thrombin generation lag time, peak thrombin generation, or thrombin generation rate, regardless of the final concentration or activating reagent used (P < 0.05 for all comparisons of C1 INH-nf versus negative controls). We found no evidence of a procoagulant effect of C1 INH-nf when studied ex vivo at concentrations up to 10-fold higher than those achieved with clinical dosing in patients with HAE.
- Bradykinin-mediated hereditary angioedema (non-estrogen-dependent) without C1 inhibitor deficiency. [Journal Article]
- J Investig Allergol Clin Immunol 2014; 24(4):280-1.
- Adalimumab desensitization protocol in a patient with a generalized urticarial reaction and angioedema following adalimumab administration. [Journal Article]
- J Investig Allergol Clin Immunol 2014; 24(4):273-5.
- Long-term prophylaxis with C1-inhibitor concentrate in patients with hereditary angioedema. [Journal Article]
- J Investig Allergol Clin Immunol 2014; 24(4):271-3.
- Clinical comparison of scorpion envenomation by Androctonus mauritanicus and Buthus occitanus in children. [JOURNAL ARTICLE]
- Toxicon 2014 Sep 8.
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 < 0.05) in patients envenomed by Am than Bo, and in all population in comparison to control group. The correlation among these biological analyzes and clinical status showed that higher levels of LDH and value of leukocytes ≥19 x10(3)/mm(3) were indices of cardiac dysfunction with APE. Pallor sign was correlated with a state of shock and/or low level of hemoglobin, associated or not to bilirubin increase. Fatalities (7.3%), presenting toxic myocarditis, had lowest count of lymphocytes (≤4.2%) in comparison to survivors. This is the first report on lymphopenia which may be useful for forecast the fatal outcome in scorpion envenomation.
- Omalizumab: A Review of Its Use in Patients with Chronic Spontaneous Urticaria. [JOURNAL ARTICLE]
- Drugs 2014 Sep 13.
Omalizumab (Xolair(®)) is a humanized, recombinant, IgG, anti-IgE monoclonal antibody that binds to the Fc region of free IgE and prevents it from binding to its high-affinity receptor (FcεR1) on mast cells and basophils. This reduction in free IgE leads to a reduction in mast cell/basophil degranulation and the release of histamine, and to the down-regulation of FcεR1 receptors on these cells. Omalizumab does not bind to cell-bound IgE or to IgG. In well-controlled clinical trials in patients with chronic spontaneous urticaria and persistent symptoms despite background treatment with antihistamines, add-on therapy with subcutaneous omalizumab 300 mg every 4 weeks for 12 or 24 weeks significantly reduced the severity of itching, and the number and size of hives, and increased patients' health-related quality of life and the proportion of days free from angioedema compared with placebo. Subcutaneous omalizumab was generally well tolerated; the incidence and severity of adverse events in omalizumab recipients were similar to those in placebo recipients, and most adverse events were of mild or moderate severity. The only adverse events occurring more frequently with omalizumab than with placebo during treatment in a safety study were headache and upper respiratory tract infection. Thus, omalizumab is an effective and well-tolerated add-on therapy in patients with chronic spontaneous urticaria who are symptomatic despite background therapy with H1 antihistamines.
- Pharmaceutical approval update. [Journal Article]
- P T 2014 Sep; 39(9):619-26.
Belinostat (Beleodaq) for peripheral T-cell lymphoma; C1-esterase inhibitor (Ruconest) for acute attacks in hereditary angioedema; and tedizolid phosphate (Sivextro) for acute bacterial skin and skin structure infections.