Results 1 to 10 of about 61,741 (331)

C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency [PDF]

Orphanet Journal of Rare Diseases, 2023
Background Autoantibodies against C1-inhibitor (C1-INH-Ab) have a diagnostic value in acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE), even though antibodies can circulate in complexes, which can be undetectable by proven methods. Our aim
Zsofia Polai, Erika Kajdacsi, Laszlo Cervenak, Zsuzsanna Balla, Szabolcs Benedek, Lilian Varga, Henriette Farkas   +6 more
doaj   +4 more sources

C1 inhibitor deficiency: consensus document [PDF]

Clinical and Experimental Immunology, 2005
SummaryWe present a consensus document on the diagnosis and management of C1 inhibitor deficiency, a syndrome characterized clinically by recurrent episodes of angio-oedema. In hereditary angio-oedema, a rare autosomal dominant condition, C1 inhibitor function is reduced due to impaired transcription or production of non-functional protein.
M M, Gompels, R J, Lock, M, Abinun, C A, Bethune, G, Davies, C, Grattan, A C, Fay, H J, Longhurst, L, Morrison, A, Price, M, Price, D, Watters   +11 more
exaly   +6 more sources

Editorial: C1 inhibitor deficiency and angioedema [PDF]

Frontiers in Allergy, 2022
Henriette Farkas, Anastasios E. Germenis, Hilary Longhurst   +2 more
doaj   +4 more sources

13th C1-inhibitor deficiency and angioedema workshop—2023 [PDF]

Allergy, Asthma & Clinical Immunology, 2023
2023, I-
doaj   +4 more sources

Nine year follow-up of a rare case of angioedema due to acquired C1-inhibitor deficiency with late onset and good response to attenuated androgen [PDF]

Allergy, Asthma & Clinical Immunology, 2018
Background Angioedema due to acquired deficiency of C1-inhibitor (C1-INH-AAE) is a rare disease sharing some clinical and laboratory similarities with hereditary angioedema, but with late onset and no positive family history. The underlining cause may be
Polliana Mihaela Leru, Vlad Florin Anton, Horia Bumbea   +2 more
doaj   +2 more sources

Isolated angioedema of the bowel due to C1 esterase inhibitor deficiency: a case report and review of literature [PDF]

Journal of Medical Case Reports, 2011
Introduction We report a rare, classic case of isolated angioedema of the bowel due to C1-esterase inhibitor deficiency. It is a rare presentation and very few cases have been reported worldwide. Angioedema has been classified into three categories. Case
Kothari Shivangi T, Shah Anish M, Botu Deviprasad, Spira Robert, Greenblatt Robert, Depasquale Joseph   +5 more
doaj   +2 more sources

Secreted Phospholipases A2 in Hereditary Angioedema With C1-Inhibitor Deficiency [PDF]

Frontiers in Immunology, 2018
BackgroundHereditary angioedema (HAE) caused by deficiency (type I) or dysfunction (type II) of the C1 inhibitor protein (C1-INH-HAE) is a disabling, potentially fatal condition characterized by recurrent episodes of swelling. We have recently found that
Stefania Loffredo, Anne Lise Ferrara, Maria Bova, Francesco Borriello, Francesco Borriello, Chiara Suffritti, Nóra Veszeli, Angelica Petraroli, Maria Rosaria Galdiero, Gilda Varricchi, Francescopaolo Granata, Andrea Zanichelli, Henriette Farkas, Marco Cicardi, Gérard Lambeau, Gianni Marone, Gianni Marone   +16 more
doaj   +5 more sources

Searching for Genetic Biomarkers for Hereditary Angioedema Due to C1-Inhibitor Deficiency (C1-INH-HAE) [PDF]

Frontiers in Allergy, 2022
Existing evidence indicates that modifier genes could change the phenotypic outcome of the causal SERPING1 variant and thus explain the expression variability of hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE).
Faidra Parsopoulou, Gedeon Loules, Maria Zamanakou, Dorottya Csuka, Agnes Szilagyi, Maria Kompoti, Grzegorz Porebski, Fotis Psarros, Markus Magerl, Markus Magerl, Anna Valerieva, Maria Staevska, Krystyna Obtulowicz, Marcus Maurer, Marcus Maurer, Matthaios Speletas, Henriette Farkas, Anastasios E. Germenis, Anastasios E. Germenis   +18 more
doaj   +2 more sources

Daratumumab-based treatment of monoclonal gammopathy–associated angioedema due to acquired C1-inhibitor deficiency [PDF]

Journal of Allergy and Clinical Immunology: Global
Daratumumab-based treatment could control severe, treatment-refractory, life-threatening angioedema due to acquired C1-inhibitor deficiency associated with monoclonal gammopathy.
Remy S. Petersen, MD, Lauré M. Fijen, MD, PhD, Laurens E. Franssen, MD, PhD, Josephine M.I. Vos, MD, PhD, Danny M. Cohn, MD, PhD   +4 more
doaj   +2 more sources

Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate [PDF]

Orphanet Journal of Rare Diseases, 2019
Background Acquired angioedema due to C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is a serious condition that may result in life-threatening asphyxiation due to laryngeal edema. It is associated with malignant B-cell lymphoma and other disorders.
Konrad Bork, Petra Staubach-Renz, Jochen Hardt   +2 more
doaj   +4 more sources