A 'de novo' arisen case of angioedema C1-inhibitor deficiency dependent: possible mutagenic effect of azathioprine?

Abstract

It is reported that a C1-inhibitor (CI-INH) deficiency dependent angiodema case arose 'de novo' in a child without a family history of this disease. His mother was undergoing immunosuppressive therapy (50 mg of azathioprine plus 8 mg of methyl-prednisolone daily) during pregnancy, uninterrupted for seven years because of a kidney transplant. All the other known causes of acquired C1-INH deficiency were excluded. An involvement of an azathioprine-induced C1-INH gene mutation is hypothised.