Hereditary angio-oedema: Long-term prophylaxis with danazol

Long-term prophylaxis in hereditary angio-oedema: a systematic review

OBJECTIVE To systematically review the evidence regarding long-term prophylaxis in the prevention or reduction of attacks in hereditary angio-oedema (HAE). DESIGN Systematic review and meta-analysis. DATA SOURCES Electronic databases were searched up to April 2011. Two reviewers selected the studies and extracted the study data, patient characteristics and outcomes of interest. ELIGIBILIT...

Management of hereditary angio-oedema with low-dose danazol.

Successful use of danazol for hereditary angio-oedema.

A 10-year-old boy with severe hereditary angio-oedema was given danazol, 200 mg each day. There was a pronounced reduction in the frequency and severity of oedema.

Hepatocellular carcinoma after danazol treatment for hereditary angio-oedema.

Hereditary angio-oedema is characterised by recurrent episodes of laryngeal, intra-abdominal, facial or peripheral oedema. Danazol can be used as prophylaxis for recurrent attacks. Hepatotoxicity is a recognised adverse effect of danazol. We report an exceptional case of a danazol-induced hepatocellular carcinoma in a 75-year-old patient with hereditary angio-oedema.

Hereditary angio-oedema

Hereditary angio-oedema is caused by a heterozygous deficiency of C1 inhibitor. This inhibitor regulates several inflammatory pathways, and patients with hereditary angio-oedema have intermittent cutaneous or mucosal swellings because of a failure to control local production of bradykinin. Swellings typically evolve in several hours and persist for a few days. In addition to orofacial angio-oed...