A5018930918- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Hemophilia Treatment and Research
- Blood Coagulation and Thrombosis Mechanisms
- Complement system in diseases
- Mast cells and histamine
- Autoimmune Bullous Skin Diseases
- Urticaria and Related Conditions
- Vitamin K Research Studies
- Enzyme function and inhibition
- Platelet Disorders and Treatments
Semmelweis University
2020-2024
Angiotensin-converting enzyme inhibitors may cause angioedema. Currently, no laboratory method is available for identifying acquired angioedema related to angiotensin-converting inhibitors. However, establishing the diagnosis possible from medical history and preexisting inhibitor therapy, as well by excluding other types.To evaluate results of complement testing in patients experiencing while taking inhibitors.Between 2005 2019, a total 149 were referred our Angioedema Center diagnostic...
Autoantibodies against C1-inhibitor (C1-INH-Ab) have a diagnostic value in acquired angioedema due to deficiency (C1-INH-AAE), even though antibodies can circulate complexes, which be undetectable by proven methods. Our aim was measure C1-INH/C1-INH-Ab complexes (CAC) and investigate their connection C1-INH-Ab the changes titer over time.19 patients were diagnosed with C1-INH-AAE Hungarian Angioedema Center of Reference Excellence; 79% them had an underlying disease. Samples examined newly...
Background: Acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a rare disease that can be diagnosed by complement testing. It often accompanies lymphoproliferative underlying diseases. Methods: In the last 30 years, from 3938 patients who were sent Angioedema Center with symptoms, we have C1-INH-AAE in 19 cases, and monitored them regularly. The connections between disease, its treatment, symptoms parameters investigated. Results: followed up for 6 years on average....
C1 inhibitor (C1INH) functions as a key endogenous of contact pathway coagulation factors XII, XI and plasma kallikrein (PKa). Patients with congenital deficiency in C1INH suffer from rare swelling disorder called hereditary angioedema (HAE) caused by excess bradykinin generation. HAE also have elevated circulating levels markers activation an increased risk venous thromboembolism, phenotypes recapitulated C1INH-deficient mice. We sought to evaluate the molecular mechanism which leads basal...
Abstract As many aspects of hereditary angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency (C1-INH-HAE) cannot be explained with elevated bradykinin level alone, it has recently become clear that other factors also play an important role in the pathogenesis. One these could neutrophil granulocyte (NG) counts, which are associated increased NG activation C1-INH-HAE patients; however, their origin not been elucidated so far. Here, we aimed investigate whether excess NGs is disturbed...
Acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a rare disease that can be diagnosed via complement testing. It often accompanies lymphoproliferative underlying diseases. Our study aimed examine if there connection between parameters and the clinical symptoms of C1-INH-AAE, and, in case known disease, its activity. The other question how connection, proven, could help development therapeutic strategy C1-INH-AAE patients. In past 30 years, out 3938 patients sent Angioedema...
Acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a rare disease, which often appears with hematological underlying diseases. It can be diagnosed by complement testing and its treatment consists of the management disease attacks. The patient (59 year-old male) who was taking angiotensin-converting-enzyme inhibitor, referred Hungarian Angioedema Center Reference Excellence an upper airway edema did not respond conventional required tracheotomy. At our Center, C1-INH-AAE...