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...

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...

A patient with apparent X-linked agammaglobulinaemia was found to be inordinately susceptible to anaphylactoid reactions to intramuscular injections of gammaglobulin. The patient was found also to have low levels of C1 esterase inhibitor (C1 INH). The possibility that the C1 INH deficiency and in this patient, whether genetic or acquired, fostered the susceptibility to the production of anaphyl...

The activated first component of complement (CI) possesses an esterase activity in vitro which will hydrolyse an ester of tyrosine to release H+. The activity of the serum inhibitor of C1 esterase may be measured by monitoring its ability to prevent H+ release under standard conditions. This paper describes a method of measuring such activity, monitoring H+ release by the use of either an acid ...

BACKGROUND Plasma-derived C1 inhibitor (C1-INH) concentrate is a treatment option for acute hereditary angioedema (HAE) attacks and is considered the standard-of-care in many countries, although it is not yet available in the United States. Studies are still being conducted to establish its safety and efficacy as required by the FDA. OBJECTIVE To review the medical literature to determine if ...

INTRODUCTION Administration of human C1 esterase inhibitor (Berinert(®) P) from target import is the most widespread treatment strategy for patients with hereditary angioedema (HAE). However, a therapeutic health program including Ruconest(®) (conestat alfa) could shorten a patient's expectancy for a life-saving treatment. AIM To evaluate the cost-utility of Ruconest(®) (conestat alfa) financ...

Case presentation We report a case of Waldenstrom ́s macroglobulinemia causing an acquired deficiency of C1 esterase inhibitor in a 40-year-old woman. She initially presented with an episode of angioedema followed by many episodes of abdominal distention associated with pain, vomiting, and diarrhea for 1.5 years. Work-up revealed low C1 esterase inhibitor levels, normal C3, and nonexistent C4. A...

Hereditary angioedema is characterized by sudden episodes of nonpitting edema that cause discomfort and pain. Typically the extremities, genitalia, trunk, gastrointestinal tract, face, and larynx are affected by attacks of swelling. Laryngeal swelling carries significant risk for asphyxiation. The disease results from mutations in the C1 esterase inhibitor gene that cause C1 esterase inhibitor ...

Hereditary angioedema (HAE), is an autosomal immunological disease, that is characterized by repetitive diffuse non-pitting painless edema without urticaria or pruritus. HAE is a rare disease with wide range of manifestation, thus diagnosis can be easily missed. Screening for HAE should be warranted in any patient with following manifestations; recurrent self-limiting unexplained abdominal pain...