TITLE

New treatment options for acute edema attacks caused by hereditary angioedema

AUTHOR(S)
THOMAS, MICHAEL C.; SHAH, SAMIT
PUB. DATE
November 2011
SOURCE
American Journal of Health-System Pharmacy;11/15/2011, Vol. 68 Issue 22, p2129
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Purpose. New treatment options for acute edema attacks caused by hereditary angioedema (HAE) are reviewed. Summary. HAE is characterized by mutations in the C1 inhibitor gene leading to either a reduced expression of C1 inhibitor in the plasma or expression of a functionally impaired C1 inhibitor. HAE is classified into two major types based on the cause of the C1 inhibitor deficiency. Type I HAE is defined by a reduced expression of C1 inhibitor in the plasma, whereas type II HAE is characterized by the expression of a dysfunctional C1 inhibitor protein. Clinical data were reviewed for C1 inhibitor, ecallantide, and icatibant in the treatment of acute edema attacks caused by HAE. C1 inhibitor leads to a faster onset of edema relief and is effective in decreasing the duration of edema. Dosing strategies include fixed dosing and weight-based dosing. Optimal dosing strategies have not been established, but fixed dosing (500-1000 units) or 20 units/kg has been effective in clinical trials and reports. No comparative trials suggest that one strategy is superior to another; however, the approved labeling for acute treatment is based on weight. Ecallantide is also efficacious for treating acute episodes; however, the available evidence is limited to a single published trial. Icatibant has shown variable effects in two trials with placebo and active controls. Conclusion. In patients with HAE, most edema episodes only involve the skin and gastrointestinal tract, though airway obstruction caused by laryngeal angioedema is the most common cause of death. I.V. C1 inhibitor should be considered first-line treatment for acute edema attacks because of its fast onset of action and effectiveness, though it is not clear whether fixed or weight-based dosing is preferred. Ecallantide can be considered as a second-line treatment option.
ACCESSION #
67225219

 

Related Articles

  • Ecallantide for treatment of acute attacks of hereditary angioedema. Martello, Jay L.; Woytowish, Melanie R.; Chambers, Hannah // American Journal of Health-System Pharmacy;4/15/2012, Vol. 69 Issue 8, p651 

    Purpose. The pharmacology, pharmacokinetics, efficacy, safety, dosage, administration, adverse effects, and place in therapy of ecallantide, a kallikrein inhibitor for the treatment of hereditary angioedema (HAE), are reviewed. Summary. Ecallantide is the first member of the kallikrein inhibitor...

  • An Overview of Novel Therapies for Acute Hereditary Angioedema. Firszt, Rafael; Frank, Michael M. // American Journal of Clinical Dermatology;2010, Vol. 11 Issue 6, p383 

    Hereditary angioedema is an episodic swelling disorder with autosomal dominant inheritance. Attacks are characterized by nonpitting edema of external or mucosal body surfaces. Patients often present with swelling of the extremities, abdominal pain, and swelling of the mouth and throat, which can...

  • CASE 1 DIAGNOSIS: HEREDITARY ANGIOEDEMA. Silverman, Jason A; Yeung, Alfred K; Jain, Sangeeta; Kavadas, Fotini D // Paediatrics & Child Health (1205-7088);May2014, Vol. 19 Issue 5, p234 

    The article presents a case study of a person diagnosed with hereditary angioedema (HAE). The patient's abdominal pain improved a lot within 24 hours. Complement assays showed normal C3, low normal C4, and low Cl esterase inhibitor functional activity. HAE is a multisystem disease which features...

  • Diagnosis and treatment of hereditary angio-oedema attacks. Longhurst, Hilary J.; Nzeako, Ugo C. // British Journal of Hospital Medicine (17508460);Mar2012, Vol. 73 Issue 3, p148 

    Patients with hereditary angio-oedema will often present to the emergency department or hospital with cutaneous swelling, abdominal pain or laryngeal oedema. This article reviews the diagnosis and acute management of patients with hereditary angio-oedema.

  • Management of hereditary angioedema. Dempster, John // Nursing Standard;5/15/2013, Vol. 27 Issue 37, p35 

    Hereditary angioedema is characterised by unpredictable, painful and potentially life-threatening oedema. Recently, some C1 inhibitors have been approved for self-administration and/or routine prevention, enabling patients to be proactive in managing their disease and reducing the burden of...

  • Hereditary angioedema: A clinical review for the otolaryngologist. Anon, Jack B. // ENT: Ear, Nose & Throat Journal;Jan2011, Vol. 90 Issue 1, p32 

    The article offers clinical information on hereditary angioedema (HAE), a rare genetic disorder characterized with either low levels of C1 esterase inhibitor (C1-INH) or the presence of dysfunctional C1-INH. The pathophysiology and genetic defects associated with HAE are described. Differential...

  • Clinical Management of Hereditary Angioedema. Weitzel, Rhonda // International Student Journal of Nurse Anesthesia;Summer2013, Vol. 12 Issue 2, p28 

    The article presents a case study of a 57-year-old Caucasian male who underwent an outpatient colonoscopy and drainage of rectal abscess. It states that the anesthesia assessment was performed with a special focus on the patient's history of hereditary angioedema, an autosomal dominant disorder...

  • Ä°Diyopatik anafilaksi olgusu: Ayırıcı tanıda herediter anjiyodem. YILMAZ, Insu; TUTAR, Nuri; BÜYÜKOĞLAN, Hakan; GÜLMEZ, Inci; OYMAK, Sema; DEMİR, Ramazan // Asthma Allergy Immunology / Astim Allerji Immunoloji;May2013, Vol. 11 Issue 1, p53 

    Anaphylaxis is potentially fatal immediate-type reaction due to the release of bioactive mediators from mast cells and basophils. Anaphylaxis typically occurs through an IgE-dependent immunologic mechanism, most commonly triggered by foods, stinging insect venoms, or medications. Medications,...

  • C1 Esterase Inhibitor (Human) for the Treatment of Acute Hereditary Angioedema. Valle, Solange Oliveira Rodrigues; Fran‡a, Alfeu Tavares; Campos, Regis A.; Grumach, Anete Sevciovic // Clinical Medicine Insights: Blood Disorders;2011, Issue 4, p21 

    Hereditary angioedema (HAE) is a relatively rare disease characterized by acute episodes of swelling. These swellings can be disfiguring, painful and life-threatening. Since the symptoms occur in different areas and most patients experience a delay in their diagnosis, resulting in unnecessary...

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics