Hereditary Oedema (Milroy's Disease)

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...

Therapy in hereditary angioneurotic oedema.

Gwynn, C. M. (1974). Archives of Disease in Childhood, 49, 636. Therapy in hereditary angioneurotic oedema. Two branches of a family suffering from hereditary angioneurotic oedema underwent trials of therapy of E-amino caproic-acid (EACA) to ascertain the optimum dosage required to alleviate symptoms without giving rise to unpleasant side effects. It was found that children under 11 years toler...

Hereditary angioneurotic oedema: a neglected diagnosis.

A case of hereditary angioneurotic oedema, which was only diagnosed after presentation to several hospital departments, is reported. It was then discovered that the mother of the patient had the same condition but that, unusually, it appeared to have been in remission for more than 20 years. This disease, due to C1 esterase inhibitor deficiency, is potentially fatal but easily treatable. The di...

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.