Effect of sex hormones on the complement‐related clinical disorder of hereditary angioedema

Frank, Michael M.

doi:10.1002/art.1780221118

Cited by 34 publications

(10 citation statements)

References 8 publications

(2 reference statements)

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“…A number of papers have been published on the relationship between fluctuations in sex hormone levels (e.g. during puberty, menstruation, pregnancy, oral contraceptive use or the menopause) and the frequency of HANO attacks (Donaldson & Rosen, 1966; Blohmé et al ., 1972; Frank et al ., 1976; Frank, 1979; Bockers & Bork, 1987; Agostoni et al ., 1992; Yip & Cunliffe, 1992; Bork et al ., 2000; McGlinchey & McCluskey, 2000). These reports, however, were based on clinical observations and historical data.…”

Section: Introductionmentioning

confidence: 99%

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Sex hormones in hereditary angioneurotic oedema

Visy

Füst²,

Varga³

et al. 2004

Clinical Endocrinology

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Section: Introductionmentioning

confidence: 99%

“…These reports, however, were based on clinical observations and historical data. Sex hormone levels were measured only in patients undergoing treatment with anabolic steroids (Frank, 1979; Schwarz et al ., 1981).…”

Section: Introductionmentioning

confidence: 99%

Sex hormones in hereditary angioneurotic oedema

Visy

Füst²,

Varga³

et al. 2004

Clinical Endocrinology

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“…It is likely that this reflects the influence of estrogen on disease severity. [4] Typically, attacks grow more severe in the first 24-36 hours and then gradually subside over the next 48-72 hours. In some patients, abdominal pain can last 4-5 days and peripheral swelling can last up to 9 days.…”

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confidence: 99%

An Overview of Novel Therapies for Acute Hereditary Angioedema

Firszt

Frank

2010

American Journal of Clinical Dermatology

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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 at times lead to asphyxiation. The disease is caused by a mutation in the gene encoding the complement C1-inhibitor protein, which leads to unregulated production of bradykinin. Long-term therapy has depended on the use of attenuated androgens or plasmin inhibitors but in the US there was, until recently, no specific therapy for acute attacks. As well, many patients with hereditary angioedema in the US were either not adequately controlled on previously available therapies or required doses of medications that exposed them to the risk of serious adverse effects. Five companies have completed or are currently conducting phase III clinical trials in the development of specific therapies to terminate acute attacks or to be used as prophylaxis. These products are based on either replacement therapy with purified plasma-derived or recombinant C1-inhibitor, or inhibition of the kinin-generating pathways with a recombinant plasma kallikrein inhibitor or bradykinin type 2 receptor antagonist. Published studies thus far suggest that all of these products are likely to be effective. These new therapies will likely lead to a totally new approach in treating hereditary angioedema.

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“…In the late 1880s, Osler described the hereditary feature of angioedema for the irst time [2]. In the 1960s, a deiciency in a type of serine proteinase inhibitor, C1 esterase inhibitor (C1-inh), was discovered as the cause of HAE, and a few years later, the second form was and minor medical procedures like tooth extraction [21][22][23]. Sometimes, the usage of ACEI or estrogen-containing drugs can trigger the symptoms in patients with a silent disease [14].…”

Section: Introductionmentioning

confidence: 99%

Hereditary Angioedema

Gelincik¹,

Demir²

2017

A Comprehensive Review of Urticaria and Angioedema

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Hereditary angioedema (HAE) is an autosomal dominantly inherited orphan disease manifested by recurrent unpredictable nonpiting and nonpruritic swelling atacks without urticarial plaques. HAE is caused by a deiciency of the C1 esterase inhibitor (C1-inh) or decreased function of C1-inh. Type 1 HAE, the most common form, occurs due to C1-inh deiciency and is seen with low-serum C1-inh levels. In type 2 HAE, the function of C1-inh is impaired, and in HAE with normal C1-inh serum levels, the function of C1-inh is normal. HAE episodes can afect various sites in the body such as the larynx, face, extremities, gastrointestinal tract, and urogenital area. Acute episodes can be treated with C1-inh concentrates, a kallikrein inhibitor, called ecallantide and bradykinin B2 receptor antagonist, icatibant. Depending on the frequency, severity, and location of the episodes, long-term prophylaxis regimens with plasma-derived C1-inh concentrates, antiibrinolytics, or 17α-alkylated androgens can be used. C1-inh concentrates or 17α-alkylated androgens should be administered before dental procedures and minor or major surgical interventions to provide short-term prophylaxis. In conclusion, HAE is a rare life-threatening disease of which clinical presentation is highly variable and early accurate diagnosis signiicantly prevents mortality and morbidity.

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Effect of sex hormones on the complement‐related clinical disorder of hereditary angioedema

Cited by 34 publications

References 8 publications

Sex hormones in hereditary angioneurotic oedema

Sex hormones in hereditary angioneurotic oedema

An Overview of Novel Therapies for Acute Hereditary Angioedema

Hereditary Angioedema

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