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Tytuł:
Hereditary angioedema caused by a novel intronic variant of SERPING1.
Autorzy:
López-Martínez R; Department of Immunology, Hospital Universitario Central de Asturias, Oviedo, Spain.
Martínez-Borra J; Department of Immunology, Hospital Universitario Central de Asturias, Oviedo, Spain.; Instituto de Investigación Sanitaria del Principado de Asturias, ISPA, Oviedo, Spain.
Fernández-González P; Department of Pediatrics, Hospital Universitario Central de Asturias, Oviedo, Spain.
Coto E; Instituto de Investigación Sanitaria del Principado de Asturias, ISPA, Oviedo, Spain.; Department of Genetics, Hospital Universitario Central de Asturias, Oviedo, Spain.; Universidad de Oviedo, Oviedo, Spain.
Toyos-González P; Department of Pediatrics, Hospital Universitario Central de Asturias, Oviedo, Spain.
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Źródło:
Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology [Pediatr Allergy Immunol] 2022 Jan; Vol. 33 (1), pp. e13681.
Typ publikacji:
Letter
MeSH Terms:
Angioedemas, Hereditary*/diagnosis
Angioedemas, Hereditary*/genetics
Complement C1 Inhibitor Protein*/genetics
Complement C1 Inactivator Proteins/genetics ; Humans ; Mutation ; Pedigree
Opinia redakcyjna
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Cerebrospinal fluid shotgun proteomics identifies distinct proteomic patterns in cerebral amyloid angiopathy rodent models and human patients.
Autorzy:
Vervuurt M; Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, 830 TML, Radboud University Medical Center, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands.
Schrader JM; Department of Biomedical and Pharmaceutical Sciences, George & Anne Institute for Neuroscience, University of Rhode Island, Kingston, RI, USA.
de Kort AM; Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, 830 TML, Radboud University Medical Center, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands.
Kersten I; Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, 830 TML, Radboud University Medical Center, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands.
Wessels HJCT; Department of Human Genetics, Translational Metabolic Laboratory, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, The Netherlands.
Klijn CJM; Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, 830 TML, Radboud University Medical Center, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands.
Schreuder FHBM; Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, 830 TML, Radboud University Medical Center, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands.
Kuiperij HB; Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, 830 TML, Radboud University Medical Center, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands.
Gloerich J; Department of Human Genetics, Translational Metabolic Laboratory, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, The Netherlands.
Van Nostrand WE; Department of Biomedical and Pharmaceutical Sciences, George & Anne Institute for Neuroscience, University of Rhode Island, Kingston, RI, USA.
Verbeek MM; Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, 830 TML, Radboud University Medical Center, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands. .; Department of Human Genetics, Translational Metabolic Laboratory, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, The Netherlands. .
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Źródło:
Acta neuropathologica communications [Acta Neuropathol Commun] 2024 Jan 08; Vol. 12 (1), pp. 6. Date of Electronic Publication: 2024 Jan 08.
Typ publikacji:
Journal Article
MeSH Terms:
Rodentia*
Cerebral Amyloid Angiopathy*
Humans ; Rats ; Animals ; Complement C1 Inhibitor Protein ; Amyloid beta-Peptides ; Proteomics ; Endopeptidases ; Biomarkers
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
The analysis of the effect of the COVID-19 pandemic on patients with hereditary angioedema type I and type II.
Autorzy:
Szilágyi D; Hungarian Angioedema Center of Reference and Excellence, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
Horváth HR; Hungarian Angioedema Center of Reference and Excellence, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
Andrási N; Hungarian Angioedema Center of Reference and Excellence, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.; Doctorate School, Semmelweis University, Budapest, Hungary.; Pediatric Center, Tűzoltó Street Department, Semmelweis University, Budapest, Hungary.
Kempler MS; Hungarian Angioedema Center of Reference and Excellence, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
Balla Z; Hungarian Angioedema Center of Reference and Excellence, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.; Doctorate School, Semmelweis University, Budapest, Hungary.
Farkas H; Hungarian Angioedema Center of Reference and Excellence, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary. .
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Źródło:
Scientific reports [Sci Rep] 2023 Nov 22; Vol. 13 (1), pp. 20446. Date of Electronic Publication: 2023 Nov 22.
Typ publikacji:
Journal Article
MeSH Terms:
Hereditary Angioedema Types I and II*/drug therapy
Hereditary Angioedema Types I and II*/epidemiology
COVID-19*/epidemiology
Angioedemas, Hereditary*/epidemiology
Angioedema*/epidemiology
Vaccines*/therapeutic use
Humans ; Quality of Life ; Pandemics ; SARS-CoV-2 ; Complement C1 Inhibitor Protein
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Mutation update of SERPING1 related to hereditary angioedema in the Chinese population.
Autorzy:
Wang X; Department of Allergy & Clinical Immunology, National Clinical Research Center for Immunologic Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, #1 Shuaifuyuan, Wangfujing, Beijing, 100730, P.R. China.
Lei S; Eighth-year Program of Clinical Medicine, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Xu Y; Department of Allergy & Clinical Immunology, National Clinical Research Center for Immunologic Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, #1 Shuaifuyuan, Wangfujing, Beijing, 100730, P.R. China.
Liu S; Department of Allergy & Clinical Immunology, National Clinical Research Center for Immunologic Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, #1 Shuaifuyuan, Wangfujing, Beijing, 100730, P.R. China.
Zhi Y; Department of Allergy & Clinical Immunology, National Clinical Research Center for Immunologic Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, #1 Shuaifuyuan, Wangfujing, Beijing, 100730, P.R. China. yuxiang_.
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Źródło:
Hereditas [Hereditas] 2022 Jul 11; Vol. 159 (1), pp. 28. Date of Electronic Publication: 2022 Jul 11.
Typ publikacji:
Journal Article
MeSH Terms:
Angioedemas, Hereditary*/diagnosis
Angioedemas, Hereditary*/epidemiology
Angioedemas, Hereditary*/genetics
Complement C1 Inhibitor Protein*/genetics
Asian People/genetics ; China/epidemiology ; Humans ; Mutation
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Acquired angioedema in B cell lymphoproliferative disease: A retrospective case series.
Autorzy:
Wonnaparhown A; Division of Pulmonary, Allergy, and Critical Care Medicine, Duke University Medical Center, Durham, North Carolina, USA.
Stefanovic A; Division of Hematologic Malignancies and Cellular Therapy, Duke University Medical Center, Durham, North Carolina, USA.
Lugar P; Division of Pulmonary, Allergy, and Critical Care Medicine, Duke University Medical Center, Durham, North Carolina, USA.
Hostetler HP; Division of Pulmonary, Allergy, and Critical Care Medicine, Duke University Medical Center, Durham, North Carolina, USA.
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Źródło:
Clinical and experimental immunology [Clin Exp Immunol] 2021 Dec; Vol. 206 (3), pp. 378-383. Date of Electronic Publication: 2021 Oct 11.
Typ publikacji:
Letter
MeSH Terms:
Angioedema/*pathology
Complement C1 Inhibitor Protein/*genetics
Hereditary Angioedema Types I and II/*genetics
Lymphoproliferative Disorders/*pathology
Aged ; Angioedema/genetics ; Anti-Inflammatory Agents, Non-Steroidal/therapeutic use ; Autoantibodies/immunology ; Bradykinin/analogs & derivatives ; Bradykinin/therapeutic use ; Bradykinin B2 Receptor Antagonists/therapeutic use ; Complement C1 Inhibitor Protein/immunology ; Complement C1q/antagonists & inhibitors ; Complement C1q/metabolism ; Female ; Humans ; Lymphoproliferative Disorders/genetics ; Male ; Middle Aged ; Peptides/therapeutic use ; Retrospective Studies
SCR Disease Name:
Acquired angioedema
Opinia redakcyjna
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
A novel murine in vivo model for acute hereditary angioedema attacks.
Autorzy:
Bupp S; Office of Biotechnology Products, Office of Pharmaceutical Quality, Center for Drug Evaluation and Research, US Food and Drug Administration, 10903 New Hampshire Avenue, Silver Spring, MD, 20993, USA.
Whittaker M; Office of New Drugs, Center for Drug Evaluation and Research, US Food and Drug Administration, Silver Spring, MD, 20993, USA.
Lehtimaki M; Office of Biotechnology Products, Office of Pharmaceutical Quality, Center for Drug Evaluation and Research, US Food and Drug Administration, 10903 New Hampshire Avenue, Silver Spring, MD, 20993, USA.
Park J; Office of Biotechnology Products, Office of Pharmaceutical Quality, Center for Drug Evaluation and Research, US Food and Drug Administration, 10903 New Hampshire Avenue, Silver Spring, MD, 20993, USA.
Dement-Brown J; Office of Biotechnology Products, Office of Pharmaceutical Quality, Center for Drug Evaluation and Research, US Food and Drug Administration, 10903 New Hampshire Avenue, Silver Spring, MD, 20993, USA.
Zhou ZH; Office of Biotechnology Products, Office of Pharmaceutical Quality, Center for Drug Evaluation and Research, US Food and Drug Administration, 10903 New Hampshire Avenue, Silver Spring, MD, 20993, USA.
Kozlowski S; Office of Biotechnology Products, Office of Pharmaceutical Quality, Center for Drug Evaluation and Research, US Food and Drug Administration, 10903 New Hampshire Avenue, Silver Spring, MD, 20993, USA. .
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Źródło:
Scientific reports [Sci Rep] 2021 Aug 05; Vol. 11 (1), pp. 15924. Date of Electronic Publication: 2021 Aug 05.
Typ publikacji:
Journal Article
MeSH Terms:
Disease Models, Animal*
Angioedemas, Hereditary/*physiopathology
Complement C1 Inhibitor Protein/*genetics
Animals ; Bradykinin/genetics ; Complement Activation/genetics ; Complement Activation/immunology ; Complement C1 Inhibitor Protein/metabolism ; Edema/drug therapy ; Female ; Fibrinolysis/genetics ; Hypotension/physiopathology ; Male ; Mice ; Mice, Inbred C57BL ; Peptides ; Serpins/genetics
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Recombinant human C1 esterase inhibitor (conestat alfa) in the prevention of severe SARS-CoV-2 infection in hospitalized patients with COVID-19: A structured summary of a study protocol for a randomized, parallel-group, open-label, multi-center pilot trial (PROTECT-COVID-19).
Autorzy:
Urwyler P; Division of Internal Medicine, University Hospital Basel, Basel, Switzerland.
Charitos P; Division of Internal Medicine, University Hospital Basel, Basel, Switzerland.
Moser S; Division of Internal Medicine, University Hospital Basel, Basel, Switzerland.
Heijnen IAFM; Division of Medical Immunology, Laboratory Medicine, University Hospital Basel, Basel, Switzerland.
Trendelenburg M; Division of Internal Medicine, University Hospital Basel, Basel, Switzerland.; Department of Clinical Research and Department of Biomedicine, University of Basel, Basel, Switzerland.
Thoma R; Division of Infectious Diseases and Hospital Epidemiology, Cantonal Hospital St. Gallen, St. Gallen, Switzerland.
Sumer J; Division of Infectious Diseases and Hospital Epidemiology, Cantonal Hospital St. Gallen, St. Gallen, Switzerland.
Camacho-Ortiz A; Hospital Universitario Dr. José Eleuterio González, Facultad de Medicina, Universidad Autónoma de Nuevo León, Monterrey, Mexico.
Bacci MR; Department of General Practice, Centro Universitário em Saúde do ABC, Santo André, SP, Brazil.
Huber LC; Clinic for Internal Medicine, City Hospital Triemli, Zurich, Switzerland.
Stüssi-Helbling M; Clinic for Internal Medicine, City Hospital Triemli, Zurich, Switzerland.
Albrich WC; Division of Infectious Diseases and Hospital Epidemiology, Cantonal Hospital St. Gallen, St. Gallen, Switzerland.
Sendi P; Department of Infectious Diseases and Hospital Epidemiology, University Hospital Basel, Basel, Switzerland.; Institute for Infectious Diseases, University of Bern, Bern, Switzerland.
Osthoff M; Division of Internal Medicine, University Hospital Basel, Basel, Switzerland. .; Department of Clinical Research and Department of Biomedicine, University of Basel, Basel, Switzerland. .
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Źródło:
Trials [Trials] 2021 Jan 04; Vol. 22 (1), pp. 1. Date of Electronic Publication: 2021 Jan 04.
Typ publikacji:
Clinical Trial Protocol; Letter
MeSH Terms:
COVID-19 Drug Treatment*
Complement C1 Inhibitor Protein/*administration & dosage
SARS-CoV-2/*isolation & purification
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Brazil ; COVID-19/blood ; COVID-19/diagnosis ; COVID-19/virology ; Clinical Trials, Phase II as Topic ; Complement C1 Inhibitor Protein/adverse effects ; Complement C1 Inhibitor Protein/pharmacokinetics ; Drug Administration Schedule ; Female ; Humans ; Injections, Intravenous/methods ; Male ; Mexico ; Middle Aged ; Multicenter Studies as Topic ; Pilot Projects ; Randomized Controlled Trials as Topic ; Recombinant Proteins/administration & dosage ; Recombinant Proteins/adverse effects ; Recombinant Proteins/pharmacokinetics ; Severity of Illness Index ; Switzerland ; Treatment Outcome ; Young Adult
Opinia redakcyjna
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Novel SERPING1 gene mutations and clinical experience of type 1 hereditary angioedema from North India.
Autorzy:
Jindal AK; Allergy Immunology Unit, Department of Paediatrics, Advanced Paediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Rawat A; Allergy Immunology Unit, Department of Paediatrics, Advanced Paediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Kaur A; Allergy Immunology Unit, Department of Paediatrics, Advanced Paediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Sharma D; Allergy Immunology Unit, Department of Paediatrics, Advanced Paediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Suri D; Allergy Immunology Unit, Department of Paediatrics, Advanced Paediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Gupta A; Allergy Immunology Unit, Department of Paediatrics, Advanced Paediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Garg R; Allergy Immunology Unit, Department of Paediatrics, Advanced Paediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Dogra S; Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Saikia B; Department of Immunopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Minz RW; Department of Immunopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Singh S; Allergy Immunology Unit, Department of Paediatrics, Advanced Paediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
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Źródło:
Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology [Pediatr Allergy Immunol] 2021 Apr; Vol. 32 (3), pp. 599-611. Date of Electronic Publication: 2020 Dec 05.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Angioedemas, Hereditary*/diagnosis
Angioedemas, Hereditary*/drug therapy
Angioedemas, Hereditary*/epidemiology
Complement C1 Inhibitor Protein*/genetics
Adolescent ; Adult ; Age of Onset ; Child ; Child, Preschool ; Complement C4 ; Female ; Humans ; Infant ; Male ; Mutation ; Young Adult
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
Gene variants of coagulation related proteins that interact with SARS-CoV-2.
Autorzy:
Holcomb D; Center for Biologics Evaluation and Research, Office of Tissues and Advanced Therapies, Division of Plasma Protein Therapeutics, Food and Drug Administration, Silver Spring, Maryland, United States of America.
Alexaki A; Center for Biologics Evaluation and Research, Office of Tissues and Advanced Therapies, Division of Plasma Protein Therapeutics, Food and Drug Administration, Silver Spring, Maryland, United States of America.
Hernandez N; Center for Biologics Evaluation and Research, Office of Tissues and Advanced Therapies, Division of Plasma Protein Therapeutics, Food and Drug Administration, Silver Spring, Maryland, United States of America.
Hunt R; Center for Biologics Evaluation and Research, Office of Tissues and Advanced Therapies, Division of Plasma Protein Therapeutics, Food and Drug Administration, Silver Spring, Maryland, United States of America.
Laurie K; Center for Biologics Evaluation and Research, Office of Tissues and Advanced Therapies, Division of Plasma Protein Therapeutics, Food and Drug Administration, Silver Spring, Maryland, United States of America.
Kames J; Center for Biologics Evaluation and Research, Office of Tissues and Advanced Therapies, Division of Plasma Protein Therapeutics, Food and Drug Administration, Silver Spring, Maryland, United States of America.
Hamasaki-Katagiri N; Center for Biologics Evaluation and Research, Office of Tissues and Advanced Therapies, Division of Plasma Protein Therapeutics, Food and Drug Administration, Silver Spring, Maryland, United States of America.
Komar AA; Center for Gene Regulation in Health and Disease, Department of Biological, Geological and Environmental Sciences, Cleveland State University, Cleveland, Ohio, United States of America.
DiCuccio M; National Center of Biotechnology Information, National Institutes of Health, Bethesda, Maryland, United States of America.
Kimchi-Sarfaty C; Center for Biologics Evaluation and Research, Office of Tissues and Advanced Therapies, Division of Plasma Protein Therapeutics, Food and Drug Administration, Silver Spring, Maryland, United States of America.
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Źródło:
PLoS computational biology [PLoS Comput Biol] 2021 Mar 17; Vol. 17 (3), pp. e1008805. Date of Electronic Publication: 2021 Mar 17 (Print Publication: 2021).
Typ publikacji:
Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
MeSH Terms:
Blood Coagulation*
Blood Proteins/*genetics
COVID-19/*metabolism
Complement C1 Inhibitor Protein/*genetics
Poly(A)-Binding Proteins/*genetics
SARS-CoV-2/*metabolism
Vitamin K Epoxide Reductases/*genetics
Anticoagulants/administration & dosage ; Blood Proteins/metabolism ; COVID-19/physiopathology ; COVID-19/virology ; Complement C1 Inhibitor Protein/metabolism ; Genome-Wide Association Study ; Humans ; Models, Molecular ; Mutation ; Poly(A)-Binding Proteins/metabolism ; Protein Binding ; SARS-CoV-2/genetics ; Severity of Illness Index ; Viral Proteins/metabolism ; Vitamin K Epoxide Reductases/metabolism ; Warfarin/administration & dosage
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
Heat-Inactivation of Human Serum Destroys C1 Inhibitor, Pro-motes Immune Complex Formation, and Improves Human T Cell Function.
Autorzy:
Fante MA; Department of Internal Medicine III, University Medical Center Regensburg, 93053 Regensburg, Germany.
Decking SM; Department of Internal Medicine III, University Medical Center Regensburg, 93053 Regensburg, Germany.; Regensburg Center for Interventional Immunology, 93053 Regensburg, Germany.
Bruss C; Department of Internal Medicine III, University Medical Center Regensburg, 93053 Regensburg, Germany.
Schreml S; Department of Dermatology, University Medical Center Regensburg, 93053 Regensburg, Germany.
Siska PJ; Department of Internal Medicine III, University Medical Center Regensburg, 93053 Regensburg, Germany.
Kreutz M; Department of Internal Medicine III, University Medical Center Regensburg, 93053 Regensburg, Germany.; Regensburg Center for Interventional Immunology, 93053 Regensburg, Germany.
Renner K; Department of Internal Medicine III, University Medical Center Regensburg, 93053 Regensburg, Germany.; Regensburg Center for Interventional Immunology, 93053 Regensburg, Germany.
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Źródło:
International journal of molecular sciences [Int J Mol Sci] 2021 Mar 05; Vol. 22 (5). Date of Electronic Publication: 2021 Mar 05.
Typ publikacji:
Journal Article
MeSH Terms:
Antigen-Antibody Complex/*immunology
CD4-Positive T-Lymphocytes/*immunology
Complement C1 Inhibitor Protein/*immunology
Antigen-Antibody Complex/blood ; Antigens, CD/blood ; Antigens, CD/immunology ; Antigens, Differentiation, T-Lymphocyte/blood ; Antigens, Differentiation, T-Lymphocyte/immunology ; CD28 Antigens/blood ; CD28 Antigens/immunology ; CD4-Positive T-Lymphocytes/metabolism ; Complement C1 Inhibitor Protein/metabolism ; Cytokines/blood ; Cytokines/immunology ; Hot Temperature ; Humans ; Lectins, C-Type/blood ; Lectins, C-Type/immunology ; Programmed Cell Death 1 Receptor/blood ; Programmed Cell Death 1 Receptor/immunology
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
Peptide Inhibitor of Complement C1 (PIC1) demonstrates antioxidant activity via single electron transport (SET) and hydrogen atom transfer (HAT).
Autorzy:
Gregory Rivera M; Department of Microbiology and Molecular Cell Biology, Eastern Virginia Medical School, Norfolk, Virginia, United States of America.
Hair PS; Department of Pediatrics, Eastern Virginia Medical School, Norfolk, Virginia, United States of America.
Cunnion KM; Department of Microbiology and Molecular Cell Biology, Eastern Virginia Medical School, Norfolk, Virginia, United States of America.; Department of Pediatrics, Eastern Virginia Medical School, Norfolk, Virginia, United States of America.; Children's Specialty Group, Norfolk, Virginia, United States of America.; Children's Hospital of The King's Daughters, Norfolk, Virginia, United States of America.
Krishna NK; Department of Microbiology and Molecular Cell Biology, Eastern Virginia Medical School, Norfolk, Virginia, United States of America.; Department of Pediatrics, Eastern Virginia Medical School, Norfolk, Virginia, United States of America.
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Źródło:
PloS one [PLoS One] 2018 Mar 02; Vol. 13 (3), pp. e0193931. Date of Electronic Publication: 2018 Mar 02 (Print Publication: 2018).
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Antioxidants/*metabolism
Complement C1 Inhibitor Protein/*metabolism
Electron Transport/*physiology
Hydrogen/*metabolism
Peptides/*metabolism
Complement C1/metabolism ; Glutathione/metabolism ; Hemoglobins/metabolism ; Humans ; Hydroxyl Radical/metabolism ; Oxidation-Reduction ; Peroxidase/metabolism ; Reactive Oxygen Species/metabolism
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Mutational spectrum of the SERPING1 gene in Swiss patients with hereditary angioedema.
Autorzy:
Steiner UC; Department of Clinical Immunology, University Hospital Zurich, Zurich, Switzerland.
Keller M; Division of Medical Microbiology, Kantonsspital Lucerne, Lucerne, Switzerland.; Division of Hematology and Central Hematology Laboratory, Department of Internal Medicine, Kantonsspital Lucerne, Lucerneand University of Berne, Berne, Switzerland.
Schmid P; Division of Hematology and Central Hematology Laboratory, Department of Internal Medicine, Kantonsspital Lucerne, Lucerneand University of Berne, Berne, Switzerland.
Cichon S; Division of Medical Genetics, University Hospital Basel, Basel, Switzerland.; Department of Biomedicine, University of Basel, Basel, Switzerland.
Wuillemin WA; Division of Hematology and Central Hematology Laboratory, Department of Internal Medicine, Kantonsspital Lucerne, Lucerneand University of Berne, Berne, Switzerland.
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Źródło:
Clinical and experimental immunology [Clin Exp Immunol] 2017 Jun; Vol. 188 (3), pp. 430-436. Date of Electronic Publication: 2017 Mar 19.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Angioedemas, Hereditary/*diagnosis
Angioedemas, Hereditary/*genetics
Complement C1 Inactivator Proteins/*genetics
Adult ; Aged ; Complement C1 Inhibitor Protein ; Family Health ; Female ; Genotype ; Humans ; Male ; Middle Aged ; Mutation ; Phenotype ; Switzerland ; Young Adult
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency.
Autorzy:
Polai Z; Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis University, Szentkiralyi u. 46, Budapest, 1088, Hungary.
Kajdacsi E; Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
Cervenak L; Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
Balla Z; Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis University, Szentkiralyi u. 46, Budapest, 1088, Hungary.
Benedek S; Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
Varga L; Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis University, Szentkiralyi u. 46, Budapest, 1088, Hungary.
Farkas H; Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis University, Szentkiralyi u. 46, Budapest, 1088, Hungary. .
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Źródło:
Orphanet journal of rare diseases [Orphanet J Rare Dis] 2023 Feb 01; Vol. 18 (1), pp. 24. Date of Electronic Publication: 2023 Feb 01.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Angioedema*/diagnosis
Angioedemas, Hereditary*/diagnosis
Humans ; Complement C1 Inhibitor Protein ; Autoantibodies ; Enzyme-Linked Immunosorbent Assay
SCR Disease Name:
Acquired angioedema
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Evaluation of the efficacy and safety of icatibant and C1 esterase/kallikrein inhibitor in severe COVID-19: study protocol for a three-armed randomized controlled trial.
Autorzy:
Mansour E; Department of Internal Medicine, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Bueno FF; Obesity and Comorbidities Research Center, University of Campinas, Campinas, São Paulo, Brazil.
de Lima-Júnior JC; Obesity and Comorbidities Research Center, University of Campinas, Campinas, São Paulo, Brazil.
Palma A; Department of Internal Medicine, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Monfort-Pires M; Obesity and Comorbidities Research Center, University of Campinas, Campinas, São Paulo, Brazil.
Bombassaro B; Obesity and Comorbidities Research Center, University of Campinas, Campinas, São Paulo, Brazil.
Araujo EP; Obesity and Comorbidities Research Center, University of Campinas, Campinas, São Paulo, Brazil.; School of Nursing, University of Campinas, Campinas, São Paulo, Brazil.
Bernardes AF; Department of Internal Medicine, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Ulaf RG; Department of Internal Medicine, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Nunes TA; Department of Internal Medicine, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Ribeiro LC; Department of Internal Medicine, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Falcão ALE; Department of Surgery, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Santos TM; Department of Internal Medicine, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Trabasso P; Department of Internal Medicine, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Dertkigil RP; Department of Radiology, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Dertkigil SS; Department of Radiology, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Maia RP; Department of Statistics, Institute of Mathematics, Statistics, and Scientific Computation, University of Campinas, Campinas, São Paulo, Brazil.
Benaglia T; Department of Statistics, Institute of Mathematics, Statistics, and Scientific Computation, University of Campinas, Campinas, São Paulo, Brazil.
Moretti ML; Department of Internal Medicine, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Velloso LA; Department of Internal Medicine, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil. .; Obesity and Comorbidities Research Center, University of Campinas, Campinas, São Paulo, Brazil. .
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Źródło:
Trials [Trials] 2021 Jan 20; Vol. 22 (1), pp. 71. Date of Electronic Publication: 2021 Jan 20.
Typ publikacji:
Clinical Trial Protocol; Journal Article
MeSH Terms:
COVID-19 Drug Treatment*
Bradykinin/*analogs & derivatives
Complement C1 Inhibitor Protein/*administration & dosage
Respiratory Insufficiency/*drug therapy
Adult ; Angiotensin-Converting Enzyme 2/metabolism ; Bradykinin/administration & dosage ; Bradykinin/adverse effects ; Bradykinin/antagonists & inhibitors ; Bradykinin/immunology ; Bradykinin/metabolism ; Bradykinin B2 Receptor Antagonists/administration & dosage ; Bradykinin B2 Receptor Antagonists/adverse effects ; Brazil ; COVID-19/complications ; COVID-19/immunology ; COVID-19/virology ; Clinical Trials, Phase II as Topic ; Complement C1 Inhibitor Protein/adverse effects ; Drug Administration Schedule ; Drug Therapy, Combination/adverse effects ; Drug Therapy, Combination/methods ; Humans ; Injections, Intravenous ; Injections, Subcutaneous ; Kallikreins/antagonists & inhibitors ; Kallikreins/metabolism ; Randomized Controlled Trials as Topic ; Respiratory Insufficiency/immunology ; Respiratory Insufficiency/virology ; SARS-CoV-2/isolation & purification ; SARS-CoV-2/pathogenicity ; Severity of Illness Index ; Treatment Outcome
Czasopismo naukowe
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Tytuł:
An analysis of the teaching of intravenous self-administration in patients with hereditary angio-oedema.
Autorzy:
Mühlberg H; Healthcare at Home Deutschland GmbH, Weinheim, Germany.
Ettl N; Healthcare at Home Deutschland GmbH, Weinheim, Germany.
Magerl M; Department of Dermatology and Allergy, Charité-Universitätsmedizin Berlin, Berlin, Germany.
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Źródło:
Clinical and experimental dermatology [Clin Exp Dermatol] 2016 Jun; Vol. 41 (4), pp. 366-71. Date of Electronic Publication: 2016 Feb 06.
Typ publikacji:
Journal Article
MeSH Terms:
Time-to-Treatment*
Angioedemas, Hereditary/*drug therapy
Complement C1 Inactivator Proteins/*administration & dosage
Patient Education as Topic/*methods
Complement C1 Inactivator Proteins/therapeutic use ; Complement C1 Inhibitor Protein ; Edema ; Infusions, Intravenous/methods ; Self Administration
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
A Novel C1-Esterase Inhibitor Oxygenator Coating Prevents FXII Activation in Human Blood.
Autorzy:
Gerling K; Clinic for Thoracic and Cardiovascular Surgery, University Hospital Tuebingen, Calwerstr, 7/1, 72076 Tuebingen, Germany.
Ölschläger S; Clinic for Thoracic and Cardiovascular Surgery, University Hospital Tuebingen, Calwerstr, 7/1, 72076 Tuebingen, Germany.
Avci-Adali M; Clinic for Thoracic and Cardiovascular Surgery, University Hospital Tuebingen, Calwerstr, 7/1, 72076 Tuebingen, Germany.
Neumann B; Clinic for Thoracic and Cardiovascular Surgery, University Hospital Tuebingen, Calwerstr, 7/1, 72076 Tuebingen, Germany.
Schweizer E; Section Medical Materials and Technology, University Hospital Tuebingen, Osianderstr, 2-8, 72076 Tuebingen, Germany.
Schlensak C; Clinic for Thoracic and Cardiovascular Surgery, University Hospital Tuebingen, Calwerstr, 7/1, 72076 Tuebingen, Germany.
Wendel HP; Clinic for Thoracic and Cardiovascular Surgery, University Hospital Tuebingen, Calwerstr, 7/1, 72076 Tuebingen, Germany.
Stoppelkamp S; Clinic for Thoracic and Cardiovascular Surgery, University Hospital Tuebingen, Calwerstr, 7/1, 72076 Tuebingen, Germany.
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Źródło:
Biomolecules [Biomolecules] 2020 Jul 13; Vol. 10 (7). Date of Electronic Publication: 2020 Jul 13.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Complement C1 Inhibitor Protein/*pharmacology
Factor XII/*drug effects
Factor XIIa/*drug effects
Heparin/*pharmacology
Anticoagulants ; Antithrombin III/metabolism ; Blood Platelets/drug effects ; Blood Platelets/physiology ; Cell Adhesion/drug effects ; Complement C1 Inhibitor Protein/chemistry ; Heparin/chemistry ; Humans ; Leukocytes, Mononuclear/drug effects ; Leukocytes, Mononuclear/physiology ; Oxygenators, Membrane ; Thrombin/metabolism
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Serum complexes between C1INH and C1INH autoantibodies for the diagnosis of acquired angioedema.
Autorzy:
López-Lera A; Instituto de Investigación Sanitaria del Hospital La Paz (IdiPaz), Madrid, Spain.; Centre for Biomedical Network Research on Rare Diseases (CIBERER) U-754, Hospital Universitario La Paz, Madrid, Spain.
Garrido S; Centre for Biomedical Network Research on Rare Diseases (CIBERER) U-754, Hospital Universitario La Paz, Madrid, Spain.; Immunology Unit, Hospital Universitario La Paz, Madrid, Spain.
Nozal P; Centre for Biomedical Network Research on Rare Diseases (CIBERER) U-754, Hospital Universitario La Paz, Madrid, Spain.; Immunology Unit, Hospital Universitario La Paz, Madrid, Spain.
Skatum L; Clinical Immunology and Transfusion Medicine, Office for Medical Services, Lund, Sweden.
Bygum A; National HAE Centre, Odense University Hospital, Denmark.
Caballero T; Instituto de Investigación Sanitaria del Hospital La Paz (IdiPaz), Madrid, Spain.; Centre for Biomedical Network Research on Rare Diseases (CIBERER) U-754, Hospital Universitario La Paz, Madrid, Spain.; Department of Allergy, Hospital Universitario La Paz, Madrid, Spain.
López Trascasa M; Instituto de Investigación Sanitaria del Hospital La Paz (IdiPaz), Madrid, Spain.; Departamento de Medicina, Universidad Autónoma de Madrid, Madrid, Spain.
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Źródło:
Clinical and experimental immunology [Clin Exp Immunol] 2019 Dec; Vol. 198 (3), pp. 341-350. Date of Electronic Publication: 2019 Sep 17.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Angioedema/*immunology
Angioedemas, Hereditary/*immunology
Autoantibodies/*immunology
Complement C1 Inhibitor Protein/*immunology
Multiprotein Complexes/*immunology
Adult ; Aged ; Aged, 80 and over ; Angioedema/blood ; Angioedema/diagnosis ; Angioedemas, Hereditary/blood ; Angioedemas, Hereditary/diagnosis ; Autoantibodies/blood ; Autoantibodies/metabolism ; Cohort Studies ; Complement C1 Inhibitor Protein/genetics ; Complement C1 Inhibitor Protein/metabolism ; Complement C1q/immunology ; Complement C1q/metabolism ; Enzyme-Linked Immunosorbent Assay ; Europe ; Female ; Humans ; Male ; Middle Aged ; Multiprotein Complexes/blood ; Multiprotein Complexes/metabolism ; Mutation ; Sensitivity and Specificity
SCR Disease Name:
Acquired angioedema
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Long-term Prophylaxis with Androgens in the management of Hereditary Angioedema (HAE) in emerging countries.
Autorzy:
Guo Y; Department of Allergy and Immunology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Zhang H; Department of Allergy Medicine, Shanxi Bethune Hospital, Third Hospital of Shanxi Medical University, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, Taiyuan, China.
Lai H; Department of Allergy, The Second Affiliated Hospital, Guangzhou Medical University, Guangzhou, China.
Wang H; Department of Allergy, The Second Affiliated Hospital,, Zhejiang University School of Medicine, Hangzhou, China.
Chong-Neto HJ; Department of Pediatrics, Hospital de Clínicas, Federal University of Paraná, Curitiba, Brazil.
Valle SOR; Department of Clinical Medicine, Hospital Universitário Clementino Fraga Filho, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
Zhu R; Department of Allergy, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, 430030, Wuhan, China. .
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Źródło:
Orphanet journal of rare diseases [Orphanet J Rare Dis] 2022 Nov 02; Vol. 17 (1), pp. 399. Date of Electronic Publication: 2022 Nov 02.
Typ publikacji:
Journal Article; Review; Research Support, Non-U.S. Gov't
MeSH Terms:
Angioedemas, Hereditary*/drug therapy
Angioedemas, Hereditary*/prevention & control
Child ; Humans ; Female ; Androgens/therapeutic use ; Plasma Kallikrein ; Quality of Life ; Complement C1 Inhibitor Protein/therapeutic use ; Bradykinin B2 Receptor Antagonists/therapeutic use
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Registry-based analysis of Icatibant and C1-inhibitor use in treatment of laryngeal attacks of hereditary angioedema.
Autorzy:
Hakl R; Department of Clinical Immunology and Allergology, St. Anne's University Hospital in Brno, Brno, Czech Republic.; Faculty of Medicine, Masaryk University, Brno, Czech Republic.
Kuklínek P; Department of Clinical Immunology and Allergology, St. Anne's University Hospital in Brno, Brno, Czech Republic.
Sobotková M; Department of Immunology, University Hospital in Motol, Charles University, Prague, Czech Republic.
Krčmová I; Institute of Clinical Immunology and Allergy, University Hospital Hradec Kralove, Charles University, Faculty of Medicine in Hradec Kralove, Hradec Kralove, Czech Republic.
Králíčková P; Institute of Clinical Immunology and Allergy, University Hospital Hradec Kralove, Charles University, Faculty of Medicine in Hradec Kralove, Hradec Kralove, Czech Republic.
Vachová M; Department of Immunology and Allergology, Teaching Hospital, Pilsen, Czech Republic.; Faculty of Medicine in Pilsen, Charles University, Pilsen, Czech Republic.
Hanzlíková J; Department of Immunology and Allergology, Teaching Hospital, Pilsen, Czech Republic.
Nováčková M; Institute of Biostatistics and Analyses, Ltd., Brno, Czech Republic.
Svoboda M; Institute of Biostatistics and Analyses, Ltd., Brno, Czech Republic.
Kováčová I; International Clinical Research Centre, St Anne's University Hospital, Brno, Czech Republic.
Litzman J; Department of Clinical Immunology and Allergology, St. Anne's University Hospital in Brno, Brno, Czech Republic.; Faculty of Medicine, Masaryk University, Brno, Czech Republic.
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Źródło:
Clinical and experimental allergy : journal of the British Society for Allergy and Clinical Immunology [Clin Exp Allergy] 2022 Aug; Vol. 52 (8), pp. 994-997. Date of Electronic Publication: 2022 Jun 13.
Typ publikacji:
Letter
MeSH Terms:
Angioedemas, Hereditary*/diagnosis
Angioedemas, Hereditary*/drug therapy
Bradykinin/analogs & derivatives ; Bradykinin/therapeutic use ; Complement C1 Inhibitor Protein/genetics ; Complement C1 Inhibitor Protein/therapeutic use ; Humans ; Registries
Opinia redakcyjna
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
Negative pressure flash pulmonary edema in a child with hereditary angioedema.
Autorzy:
Barman P; Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Basu S; Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Thakur I; Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Chawla S; Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Kaur A; Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Jafra A; Department of Anaesthesia, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Jindal AK; Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Singh S; Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
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Źródło:
Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology [Pediatr Allergy Immunol] 2022 Jul; Vol. 33 (7), pp. e13825.
Typ publikacji:
Letter
MeSH Terms:
Angioedema*
Angioedemas, Hereditary*/complications
Angioedemas, Hereditary*/diagnosis
Pulmonary Edema*/etiology
Child ; Complement C1 Inactivator Proteins ; Complement C1 Inhibitor Protein ; Humans
Opinia redakcyjna
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Wyświetlanie 1-20 z 520

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