Results 31 to 40 of about 144,374 (198)

Successful treatment of post-pericardiotomy syndrome via C1 inhibitor replacement therapy in a hereditary angioedema patient with Marfan syndrome

The Turkish Journal of Pediatrics, 2023
Background. Hereditary angioedema with C1 inhibitor deficiency (HAE-C1INH) is caused by dysfunctional C1-INH protein due to mutations in the SERPING1 gene encoding C1-INH.
Ezgi Topyıldız, Handan Duman Şenol, Figen Gülen, Esen Demir, Nihal Mete Gökmen   +4 more
doaj   +1 more source

HAE international home therapy consensus document [PDF]

, 2010
Hereditary angioedema (C1 inhibitor deficiency, HAE) is associated with intermittent swellings which are disabling and may be fatal. Effective treatments are available and these are most useful when given early in the course of the swelling.
Bowen, Tom, Craig, Timothy, Boysen, Henrik, E. Aygören Pürsün, Janet Long, Zuraw Bruce, Avner Reshef, Longhurst, Hilary J., Cicardi Marco, Bygum, Anette, Hilary J Longhurst, Bruce Zuraw, Anette Bygum, Martinez-Saguer, Inmaculada, Bork Konrad, Inmaculada Martinez-Saguer, Sofia Grigoriadou, Caballero, Teresa, Claire Bethune, I. Martinez Saguer, Reshef Avner, Martinez Saguer, Inmaculada, M. Raguet, H. J. Longhurst, J. Bjorkander, Laurence Bouillet, Levi Marcel M, Raguet Michel, Timothy Craig, Bethune Claire, T. Bowen, Michel Raguet, L. Bouillet, Marco Cicardi, Gompels Mark, Bork, Konrad, C. Bethune, Dempster, John, Huffer Ursula, Marcel M Levi, Longhurst, Hilary, Aygören-Pürsün Emel, M. Cicardi, Farkas Henriette, Henriette Farkas, Raguet, Michel, Bygum Anette, Bjorkander Janne, Levi, Marcel M, Longhurst Hilary J, Martinez-Saguer Inmaculada, Teresa Caballero, Ursula Huffer, Bouillet, Laurence, H. Farkas, Boysen Henrik, Huffer, Ursula, M. M. Levi, M. Gompels, Gompels, Mark, S. Grigoriadou, Kreuz, Wolfhart, Janne Bjorkander, A. Bygum, Craig Timothy, Longhurst, Hilary J, Farkas, Henriette, Bygum, Anette; id_orcid, Bowen Tom, Gooi Jimmy, Bouillet Laurence, Boysen, Henrik B., A. Reshef, J. Gooi, J. Dempster, Konrad Bork, Henrik Boysen, Gooi, Jimmy, Jimmy Gooi, Cicardi, Marco, T. Craig, B. Zuraw, Caballero Teresa, Bethune, Claire, U. Huffer, Levi, Marcel M., Dempster John, John Dempster, Grigoriadou Sofia, Reshef, Avner, Long Janet, H. Boysen, Grigoriadou, Sofia, Bjorkander, Janne, T. Caballero, Tom Bowen, Zuraw, Bruce, J. Long, W. Kreuz, Mark Gompels, Emel Aygören-Pürsün, Aygören-Pürsün, Emel, K. Bork, Kreuz Wolfhart, Long, Janet, Wolfhart Kreuz   +105 more
core   +1 more source

HAE-AS: A Specific Disease Activity Scale for Hereditary Angioedema With C1-Inhibitor Deficiency [PDF]

Journal of Investigational Allergology and Clinical Immunology, 2021
Background: The activity of hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE) varies between patients and within individual patients. Objective: This study aims to develop a disease activity scale for C1-INH-HAE (HAE-AS) with sound measurement properties.
M J, Forjaz, A, Ayala, M, Caminoa, N, Prior, E, Pérez-Fernández, T, Caballero   +5 more
openaire   +2 more sources

Hereditary angioedema: beyond international consensus - circa December 2010 - The Canadian Society of Allergy and Clinical Immunology Dr. David McCourtie Lecture

Allergy, Asthma & Clinical Immunology, 2011
Background The 2010 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema was published earlier this year in this Journal (Bowen et al.
Bowen Tom
doaj   +1 more source

Clinical features of hereditary angioedema and warning signs (H4AE) for its identification

Clinics, 2022
Objectives The study describes a case series of hereditary angioedema with C1 Inhibitor Deficiency (C1INH-HAE) in order to corroborate six clinical warning signs “HAAAAE (H4AE)” to enable early identification of this disease.
Pedro Giavina-Bianchi, Marcelo Vivolo Aun, Juliana Fóes Bianchini Garcia, Laís Souza Gomes, Ana Júlia Ribeiro, Priscila Takejima, Rosana Câmara Agondi, Jorge Kalil, Antonio Abilio Motta   +8 more
doaj   +1 more source

Psychometric study of the SF-36v2 in hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) [PDF]

, 2022
BACKGROUND: The generic 36-item Short-Form Health Survey (SF-36v2) has been used to assess health related quality of life in adult patients with hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE) even though it has not yet been validated ...
Palao-Ocharan, Paola, Prior, Nieves, Caminoa, Magdalena, Caballero, Teresa, DV-HAE-QoL Study Group, Pérez-Fernández, Elia   +5 more
core   +1 more source

Hereditary angioedema: what the gastroenterologist needs to know [PDF]

, 2014
M Aamir Ali, Marie L Borum Division of Gastroenterology and Liver Diseases, George Washington University, Washington, DC, USA Abstract: Up to 93% of patients with hereditary angioedema (HAE) experience recurrent abdominal pain.
Borum, Marie L., Ali MA, Ali, Aamir M., Borum ML, Ali, M. Aamir   +4 more
core   +1 more source

An update on the genetics and pathogenesis of hereditary angioedema

Genes and Diseases, 2020
Hereditary angioedema (HAE) is an uncommon genetic disorder characterized by recurrent episodes of edema involving subcutaneous tissue and submucosa. The pathogenesis of HAE reflects an intricate coordinated regulation of components of complement, kinin ...
Aaqib Zaffar Banday, Anit Kaur, Ankur Kumar Jindal, Amit Rawat, Surjit Singh   +4 more
doaj   +1 more source

Genetic alterations and pathways in patients with Hereditary Angioedema of Unknown Cause (U-HAE)

, 2021
Objective: Hereditary angioedema ( HAE) with normal C1 inhibitor (HAE-nC1-INH), is a genetically complex, rare disease and mutations in F12, ANGPT1, PLG, MYOF genes are found in some families with HAE-nC1-INH. However, often a specific mutation cannot be
Gülbahar, Okan, Nikerel, İlkem Emrah, Alp, Hande, Kaymakçalan Çelebiler, Hande, Çağlayan, Ahmet Okay, Mete Gökmen, Emine Nihal   +5 more
core   +1 more source

Recurrent angioedema in childhood: hereditary angioedema or histaminergic angioedema?

, 2021
Background Recurrent angioedema is a rare entity during childhood. This study aimed to clarify differences between hereditary angioedema (HAE) and histaminergic angioedema (HA) in ...
ŞAHİNER, ÜMİT MURAT, Ercan Nain, Akin, Mustafa Senol, UYSAL SOYER, ÖZGE, Bülent Enis Şekerel, Ocak, Melike, Nain, Ercan, Melike Ocak, Esra Karabiber, Karabiber, Esra, Özge Soyer, Mustafa Şenol Akin, Ümit Murat Şahiner, ŞEKEREL, BÜLENT ENİS   +13 more
core   +1 more source