C1 esterase inhibitor concentrate in the management of hereditary angio-oedema

Judge,; Km, Watson; Mw, Greaves

doi:10.3109/09546639309084523

Cited by 3 publications

(4 citation statements)

References 5 publications

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“…The mainstay of emergency medical treatment of HAE is intravenous fresh frozen plasma or C1 inhibitor concentrate or lyophilized vapor-heated C1 inhibitor concentrate [ 19 , 20 ]. For patients in whom attacks are infrequent and not life-threatening, the treatment may be restricted to avoidance of provocative factors, that is, trauma, estrogens, and ACEI administration [ 1 ].…”

Section: Discussionmentioning

confidence: 99%

Angioedema: Clinical and Etiological Aspects

Kulthanan

Jiamton

Boochangkool

et al. 2007

Clinical and Developmental Immunology

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Angioedema is an abrupt swelling of the skin, mucous membrane, or both including respiratory and gastrointestinal tracts. This study aimed to report an experience of angioedema in a university hospital with respect to etiologies, clinical features, treatment, and outcome. One hundred and five patients were enrolled. About half had angioedema without urticaria. The common sites of involvement were periorbital area and lips. Forty five patients (49%) had systemic symptoms. The most common cause of angioedema was allergic angioedema. Nonsteroidal anti-inflammatory drug-induced angioedema and idiopathic angioedema were detected in 20% and 18%, respectively. Among patients with allergic angioedema, 41.7% were caused by food, 39.6% by drugs. Thirty seven patients (39%) had recurrent attacks of angioedema. Mean standard deviation (SD) number of attacks in patients with recurrent angioedema was 3.9 (2.7) (ranging from 2 to 10 times). Patients who had older age and multiple sites of skin involvement had tendency to have systemic symptoms.

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Section: Discussionmentioning

confidence: 99%

Angioedema: Clinical and Etiological Aspects

Kulthanan

Jiamton

Boochangkool

et al. 2007

Clinical and Developmental Immunology

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“…However, most acute episodes are nonlife-threatening and the mainstay of emergency medical treatment is intravenous fresh frozen plasma or C1 inhibitor concentrate. 93 The recent availability of lyophilized vapor-heated C1 inhibitor concentrate 94 has largely removed concerns about transmission of HIV, viral hepatitis, and other infections. In this study, the product used (Immuno; Aventis Behring, Vienna, Austria) after reconstitution with saline contained 550 plasma U in a 10-mL vial and was administered at a dose of 25 plasma U/kg body weight to a total of 1000 plasma U repeated once if necessary.…”

Section: Treatmentmentioning

confidence: 99%

Angioedema

Kaplan

Greaves

2005

Journal of the American Academy of Dermatology

308

235

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“…These include glomerulonephritis, Sjögren syndrome, thyroiditis, and systemic lupus erythematosus [70]. Associated coagulopathies have also been reported[71,72] due, at least in some cases, to abnormally high levels of C4-binding protein, which also binds and inactivates proteins S, [71] a cofactor for the protein Ca inactivation of activated coagulation factors V and VIII.…”

Section: Hereditary Angioedemamentioning

confidence: 99%

“…If the patient has serious respiratory obstruction, intubation or tracheostomy should be carried out and may be lifesaving. However, most acute episodes are non-life threatening, and the mainstay of emergency medical treatment is intravenous fresh frozen plasma or C1 INH concentrate [72]. The recent availability of lyophilized vapor-heated C1 INH concentrate[73] has largely removed concerns about transmission of human immunodeficiency virus, viral hepatitis, and other infections.…”

Section: Hereditary Angioedemamentioning

confidence: 99%

Angioedema

Kaplan

2008

World Allergy Organization Journal

107

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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 particular role as a mediator of the swelling. Reactions to contrast agents resemble allergy with basophil and mast cell degranulation in the absence of specific IgE antibody and can be generalized, that is, anaphylactoid. Angioedema accompanies chronic urticaria in 40% of patients, and approximately half have an autoimmune mechanism in which there is IgG antibody directed to the subunit of the IgE receptor (40%) or to IgE itself (5%-10%). Bradykinin is the mediator of angioedema in hereditary angioedema types I and II (C1 inhibitor [INH] deficiency) and the newly described type III disorder some of which are caused by a mutation involving factor XII. Acquired C1 INH deficiency presents in a similar fashion to the hereditary disorder and is due either to C1 INH depletion by circulating immune complexes or to an IgG antibody directed to C1 INH. Although each of these causes excessive bradykinin formation because of activation of the plasma bradykinin-forming pathway, the angioedema due to angiotensin-converting enzyme inhibitors is caused by excessive bradykinin levels due to inhibition of bradykinin degradation. Idiopathic angioedema (ie, pathogenesis unknown) may be histaminergic, that is, caused by mast cell degranulation with histamine release, or nonhistaminergic. The mediator pathways in the latter case are yet to be defined. A minority may be associated with the same autoantibodies associated with chronic urticaria. Angioedema that is likely to be life threatening (laryngeal edema or tongue/pharyngeal edema that obstructs the airway) is seen in anaphylactic/anaphylactoid reactions and the disorders mediated by bradykinin.

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C1 esterase inhibitor concentrate in the management of hereditary angio-oedema

Cited by 3 publications

References 5 publications

Angioedema: Clinical and Etiological Aspects

Angioedema: Clinical and Etiological Aspects

Angioedema

Angioedema

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