primary complement deficiencies are rare and two related patients are reported here. the first patient is a 41- year- old man with eighteen episodes of pneumo¬coccal meningitis and other purulent infections. the serum c3 level was checked at three separate times, showing that this was a primary c3 deficient case; other immunological tests were however normal. this patient now takes prophylactic...

Hereditary and acquired angioedema are potentially life-threatening diseases characterized by spontaneous episodes of subcutaneous and submucosal swelling of face, lips, oral cavity, larynx, and GI tract. Hereditary angioedema (HAE) usually presents within the first and second decades of life, whereas acquired angioedema presents in adults after 40 years of age. These clinical symptoms together...

angioedema is characterized by recurrent, circumscribed, solitary or multiple subcutaneous and mucosal swelling, involving the extremities, face, larynx, bowel wall. angioedema is due to hereditary or acquired varieties of c1 esterase inhibitor (c1inh) deficiency. a case of atypical acquired angioedema in a 49-year old man, responding favourably to cinnarizine and alcohol abstinence, is present...

Background In classic hereditary angioedema, inadequate C1-inhibitor (C1-INH) failing to restrict factor-XII activity leads to increased production of bradykinin, a potent vasodilator and mediator of angioedema. Hereditary angioedema with normal C1-INH (Type-III) manifests with sporadic recurrent angioedema but normal C1-INH concentration and activity. Here, bradykinin accumulation appears depe...

This article focuses specifically on angioedema. Chronic angioedema represents a wide range of diseases and can be categorized into several forms including hereditary, acquired, drug induced, and idiopathic. Hereditary and acquired angioedema are known to be a result of abnormalities in C1 inhibitor protein while the mechanism of drug-induced and idiopathic angioedema is less clear. Significant...

INTRODUCTION Hereditary angioedema is the commonest inherited disorder of the complement system and has been associated with several immune glomerular diseases. A case of nephrotic syndrome and renal impairment due to idiopathic membranous glomerulonephritis in a patient with hereditary angioedema has not been described before. CASE PRESENTATION We present the first reported case of the assoc...

Hereditary angioedema is a serious medical condition caused by a deficiency of C1-inhibitor. The condition is the result of a defect in the gene controlling the synthesis of C1-inhibitor, which regulates the activity of a number of plasma cascade systems. Although the prevalence of hereditary angioedema is low - between 1:10,000 to 1:50,000 - the condition can result in considerable pain, debil...