Danazol and stanozolol in long-term prophylactic treatment of hereditary angioedema

Agostoni, A; Cicardi, Marco; Martignoni, G.; Bergamaschini, Luigi; Marasini, Bianca

doi:10.1016/0091-6749(80)90181-5

Cited by 88 publications

(49 citation statements)

References 14 publications

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“…Frequent attacks of peripheral angio-oedema (extremities, trunk), although unpleasant and annoying, are not dangerous and may not require (contingent upon the patient's judgement) long-term prophylaxis. However, prophylactic administration of antifibrinolytic agents (tranexamic acid [39] or epsilon-aminocaproic acid (EACA; not licensed in the UK) [40]) and/or synthetic attenuated androgens (danazol [41][42][43] or stanozolol [43][44][45][46]), has proved useful in reducing the frequency or severity of attacks (evidence level 2/3). Other androgens (methyltestosterone [47], fluoxymesterone [48] and oxymetholone [48,49]), can be used in adult males (evidence level 3).…”

Section: Long-term Prophylaxismentioning

confidence: 99%

C1 inhibitor deficiency: consensus document

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Abinun

et al. 2005

Clinical and Experimental Immunology

380

460

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SummaryWe present a consensus document on the diagnosis and management of C1 inhibitor deficiency, a syndrome characterized clinically by recurrent episodes of angio-oedema. In hereditary angio-oedema, a rare autosomal dominant condition, C1 inhibitor function is reduced due to impaired transcription or production of non-functional protein. The diagnosis is confirmed by the presence of a low serum C4 and absent or greatly reduced C1 inhibitor level or function. The condition can cause fatal laryngeal oedema and features indistinguishable from gastrointestinal tract obstruction. Attacks can be precipitated by trauma, infection and other stimulants. Treatment is graded according to response and the clinical site of swelling. Acute treatment for severe attack is by infusion of C1 inhibitor concentrate and for minor attack attenuated androgens and/or tranexamic acid. Prophylactic treatment is by attenuated androgens and/or tranexamic acid. There are a number of new products in trial, including genetically engineered C1 esterase inhibitor, kallikrein inhibitor and bradykinin B2 receptor antagonist. Individual sections provide special advice with respect to diagnosis, management (prophylaxis and emergency care), special situations (childhood, pregnancy, contraception, travel and dental care) and service specification.

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Section: Long-term Prophylaxismentioning

confidence: 99%

C1 inhibitor deficiency: consensus document

Gompels

Lock

Abinun

et al. 2005

Clinical and Experimental Immunology

380

460

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show abstract

“…Frequent attacks of peripheral angio-oedema (extremities, trunk), although unpleasant and annoying, are not dangerous and do not require long term prophylaxis. However, prophylactic administration of antifibrinolytic agents (e-aminocaproic acid 29 and tranexamic acid 30 ), androgens (methyltestosterone, 31 fluoxymesterone, and oxymetholone 32 33 ), or synthetic, attenuated androgens (danazol [34][35][36] or stanazolol [36][37][38][39] has proved useful in reducing the frequency or severity of attacks.…”

Section: Long Term Prophylaxismentioning

confidence: 99%

Current management of hereditary angio-oedema (C'1 esterase inhibitor deficiency)

Fay¹,

Abinun²

2002

Journal of Clinical Pathology

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“…Abbreviations used: ACHA, anti-cholesterol autoantibodies; HAE, hereditary angioedema; HDL, high-density lipoprotein; LDL, low-density lipoprotein. [6,7,25]. Nevertheless, patients receiving continuous danazol prophylaxis suffer from various side effects [26], most frequently weight gain, acne, hirsuitism, menstrual abnormalities and elevation of liver enzyme activity.…”

Section: Discussionmentioning

confidence: 99%

“…Danazol is effective for the prophylaxis of recurrent attacks of mucocutaneous and visceral swelling that occur in patients with HAE. Danazol apparently increases the synthesis of C1-INH, which is partially deficient in these heterozygous individuals [6,7]. Our previous findings indicated that the long-term use of danazol has an adverse effect on serum lipid profile: serum concentrations of highdensity lipoprotein (HDL) cholesterol and apolipoprotein A-I were significantly lower, whereas low-density lipoprotein (LDL) cholesterol and apolipoprotein B-100 were higher in danazol-treated patients, compared with untreated HAE controls [8].…”

Section: Introductionmentioning

confidence: 99%