OBJECTIVE To evaluate whether activated partial thromboplastin time (APTT) could be used in the laboratory diagnosis of hereditary or acquired angioedema (HAE or AAE) with and without C1 inhibitor (C1-INH) deficiency. METHODS In a prospective investigation, APTT and other coagulation parameters were determined in 149 adult patients with various types of angioedema and in 26 healthy participan...

Hereditary angioedema secondary to C1-inhibitor deficiency is a rare autosomal dominant disorder characterized by a deficiency of C1 esterase inhibitor. An eight-year-old girl showed periorbital painless swelling, diagnosed as ethmoiditis. A craniofacial scan did not evidence a paranasal sinus involvement, C1INH levels were undetectable, with low C4 levels: 7.6 mg/dl and C1INH: <8.46 mg/dl. The...

Hereditary (HAE) and acquired (AAE) angioedema are vascular reactions involving the sub mucosal tissues, representing localized edema caused by dilatation and increased permeability of the capillaries. HAE and AAE are clinical disorders characterized by angioedema that require prompt differentiation from other causes of angioedema in order to receive the most pertinent and effective therapeutic...

p p t ngioedema is a pathologic condition first described y Quincke and Osler. It can be genetically deterined or acquired, and it is caused by a vascular eaction induced by deficiency or functional altertion of the C1 inhibitor (C1-INH), an enzyme inolved in the regulation of complement, contact, firinolytic, and coagulation systems. Two forms of angioedema have been described in he literature...

A patient with a lymphoproliferative disorder, angioedema, and an acquired deficiency of the inhibitor of the activated first component of complement was studied. The patient's complement profile revealed depletion of the first component of complement, which has not been seen in angioedema of the hereditary type. There was no evidence for C1-depleting activity in the patient's plasma. The major...

Hereditary angioedema (HAE) is an inherited autosomal dominant trait and symptoms of HAE include sudden onset of edema in various organs, such as subcutaneous edema, laryngeal edema and gastrointestinal tracts mucosa. We have experienced a case of a pregnant woman diagnosed with HAE and successfully managed her pregnancy. The patient was 37 years old, gravida 1, primiparous. She had experienced...

Background Hereditary Angioedema (HAE) is a rare disease characterized by recurrent angioedema attacks involving larynx, abdomen, extremities and various body parts. The reactions are by and large self-limited, but potentially, could be fatal. Until recently, the only approved treatment in Canada is an intravenous C1-esterase inhibitor infusion. However, intravenous therapy can be challenging f...