Hereditary Angio-Oedema (HAO)

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...

Hereditary angio-oedema with mesangiocapillary glomerulonephritis.

A patient with hereditary angio-oedema (HAO) developed mesangiocapillary glomerulonephritis (MCGN) under observation. HAO is characterized by an inherited defect of complement-deficiency of C1 esterase. MCGN is often associated with another complement abnormality which leads to depression of serum C3 and there is some evidence that the complement abnormality precedes the nephritis. The coincide...

Recurrent abdominal pain due to hereditary angio-oedema.

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.