somal dominant disorder that results in episodes of acute edema in various organs, including the gastrointestinal tract, skin and larynx. It is estimated to affect about 1 in 50 000–100 000 people. The symptoms, including abdominal pain, laryngeal edema and subcutaneous edema, usually begin in childhood and persist throughout life with unpredictable severity. Hereditary angioedema is classified...

Keeney S, Halalau A. BMJ Case Rep 2017. doi:10.1136/bcr-2017-221027 Description A 43-year-old man with a history of idiopathic acute pancreatitis and pancreatic pseudocysts presented to the hospital with acute abdominal pain, vomiting and hypotension. He had been hospitalised on multiple occasions for recurrent, self-limited abdominal pain accompanied by hypotension. Given his history, the symp...

Icatibant is used to treat acute hereditary angioedema with C1 inhibitor deficiency types I/II (C1-INH-HAE types I/II) and has shown promise in angioedema due to acquired C1 inhibitor deficiency (C1-INH-AAE). Data from the Icatibant Outcome Survey (IOS) were analysed to evaluate the effectiveness of icatibant in the treatment of patients with C1-INH-AAE and compare disease characteristics with ...

The biologic activity of C1 esterase, activated forms of factor XII and kallikrein at sites of vascular inflammation may be regulated by C1 inhibitor (C1 INH) elaborated by endothelial cells. Therefore, we investigated whether human umbilical vein endothelial cells (HUVEC) in culture produce C1 INH. Passaged HUVEC contain 1.6 +/- 0.8 micrograms of C1 INH/10(8) cells (mean +/- S.D.; n = 7) which...

To the Editor: Horstick and colleagues1 recently reported detrimental side effects of C1-inhibitor (C1inh) in pigs with myocardial ischemia. Local complement activation contributes to ischemic myocardium injury, and complement inhibition constitutes a novel therapeutic approach for coronary syndromes. C1inh reduces reperfusion injury in various animal models of myocardial infarction (MI). In do...