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Tytuł:
Long-term follow-up including extensive complement analysis of a pediatric C3 glomerulopathy cohort.
Autorzy:
Michels MAHM; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands. .
Wijnsma KL; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands.
Kurvers RAJ; Department of Pediatric Nephrology, Maastricht University Medical Center, Maastricht, The Netherlands.
Westra D; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands.
Schreuder MF; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands.
van Wijk JAE; Department of Pediatric Nephrology, Emma Children's Hospital, Amsterdam University Medical Centers, Amsterdam, The Netherlands.
Bouts AHM; Department of Pediatric Nephrology, Emma Children's Hospital, Amsterdam University Medical Centers, Amsterdam, The Netherlands.
Gracchi V; Department of Pediatric Nephrology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Engels FAPT; Department of Pediatric Nephrology, Maastricht University Medical Center, Maastricht, The Netherlands.
Keijzer-Veen MG; Department of Pediatric Nephrology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.
Dorresteijn EM; Department of Pediatric Nephrology, Sophia Children's Hospital, Erasmus Medical Center, Rotterdam, The Netherlands.
Volokhina EB; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands.; Department of Laboratory Medicine, Radboud University Medical Center, Nijmegen, The Netherlands.
van den Heuvel LPWJ; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands.; Department of Laboratory Medicine, Radboud University Medical Center, Nijmegen, The Netherlands.; Department of Pediatrics/Pediatric Nephrology and Department of Development and Regeneration, University Hospitals Leuven, Leuven, Belgium.
van de Kar NCAJ; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands.
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Źródło:
Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2022 Mar; Vol. 37 (3), pp. 601-612. Date of Electronic Publication: 2021 Sep 02.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Glomerulonephritis*
Glomerulonephritis, Membranoproliferative*/pathology
Kidney Diseases*
Child ; Complement C3 ; Complement C3 Nephritic Factor ; Complement Pathway, Alternative ; Female ; Follow-Up Studies ; Humans ; Male ; Properdin
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Genetic and pathological findings in a boy with psoriasis and C3 glomerulonephritis: A case report and literature review.
Autorzy:
Wei L; Department of Nephrology and Rheumatology, Children's Hospital of Zhengzhou University, Henan Children's Hospital, Zhengzhou Children's Hospital, Zhengzhou, Henan, China.
Fang Y; Department of Nephrology, Children's Hospital of Fudan University, National Pediatric Medical Center of China, Shanghai, China.; Shanghai Kidney Development and Pediatric Kidney Disease Research Center, Shanghai, China.
Cao G; Department of Nephrology and Rheumatology, Children's Hospital of Zhengzhou University, Henan Children's Hospital, Zhengzhou Children's Hospital, Zhengzhou, Henan, China.
Zhang S; Department of Nephrology and Rheumatology, Children's Hospital of Zhengzhou University, Henan Children's Hospital, Zhengzhou Children's Hospital, Zhengzhou, Henan, China.
Tian M; Department of Nephrology and Rheumatology, Children's Hospital of Zhengzhou University, Henan Children's Hospital, Zhengzhou Children's Hospital, Zhengzhou, Henan, China.
Shen Q; Department of Nephrology, Children's Hospital of Fudan University, National Pediatric Medical Center of China, Shanghai, China.; Shanghai Kidney Development and Pediatric Kidney Disease Research Center, Shanghai, China.
Xu H; Department of Nephrology, Children's Hospital of Fudan University, National Pediatric Medical Center of China, Shanghai, China.; Shanghai Kidney Development and Pediatric Kidney Disease Research Center, Shanghai, China.
Liu C; Department of Nephrology and Rheumatology, Children's Hospital of Zhengzhou University, Henan Children's Hospital, Zhengzhou Children's Hospital, Zhengzhou, Henan, China.
Rao J; Department of Nephrology, Children's Hospital of Fudan University, National Pediatric Medical Center of China, Shanghai, China.; Shanghai Kidney Development and Pediatric Kidney Disease Research Center, Shanghai, China.; Shanghai Key Lab of Birth Defect, Children's Hospital of Fudan University, Shanghai, China.
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Źródło:
Molecular genetics & genomic medicine [Mol Genet Genomic Med] 2020 Oct; Vol. 8 (10), pp. e1430. Date of Electronic Publication: 2020 Jul 28.
Typ publikacji:
Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
MeSH Terms:
CARD Signaling Adaptor Proteins/*genetics
Chloride Channels/*genetics
Dent Disease/*genetics
Glomerulonephritis/*genetics
Guanylate Cyclase/*genetics
Membrane Proteins/*genetics
Psoriasis/*genetics
Child ; Complement C3 Nephritic Factor/metabolism ; Dent Disease/pathology ; Glomerulonephritis/pathology ; Hemizygote ; Humans ; Male ; Mesangial Cells/metabolism ; Mesangial Cells/pathology ; Mutation, Missense ; Podocytes/metabolism ; Podocytes/ultrastructure ; Polymorphism, Single Nucleotide ; Psoriasis/pathology
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Immunological features of patients affected by Barraquer-Simons syndrome.
Autorzy:
Corvillo F; Complement Research Group, Hospital La Paz Institute for Health Research (IdiPAZ), La Paz University Hospital, Paseo de la Castellana, 261, 28046, Madrid, Spain. .; Center for Biomedical Network Research on Rare Diseases (CIBERER U754), Madrid, Spain. .
Ceccarini G; Obesity and Lipodystrophy Centre at the Endocrinology Unit, University Hospital of Pisa, Pisa, Italy.
Nozal P; Complement Research Group, Hospital La Paz Institute for Health Research (IdiPAZ), La Paz University Hospital, Paseo de la Castellana, 261, 28046, Madrid, Spain.; Center for Biomedical Network Research on Rare Diseases (CIBERER U754), Madrid, Spain.; Unit of Immunology, La Paz University Hospital, Madrid, Spain.
Magno S; Obesity and Lipodystrophy Centre at the Endocrinology Unit, University Hospital of Pisa, Pisa, Italy.
Pelosini C; Obesity and Lipodystrophy Centre at the Endocrinology Unit, University Hospital of Pisa, Pisa, Italy.
Garrido S; Complement Research Group, Hospital La Paz Institute for Health Research (IdiPAZ), La Paz University Hospital, Paseo de la Castellana, 261, 28046, Madrid, Spain.; Center for Biomedical Network Research on Rare Diseases (CIBERER U754), Madrid, Spain.; Unit of Immunology, La Paz University Hospital, Madrid, Spain.
López-Lera A; Complement Research Group, Hospital La Paz Institute for Health Research (IdiPAZ), La Paz University Hospital, Paseo de la Castellana, 261, 28046, Madrid, Spain.; Center for Biomedical Network Research on Rare Diseases (CIBERER U754), Madrid, Spain.
Moraru M; Immunogenetics and Histocompatibility, Instituto de Investigación Sanitaria Puerta de Hierro, Madrid, Spain.
Vilches C; Immunogenetics and Histocompatibility, Instituto de Investigación Sanitaria Puerta de Hierro, Madrid, Spain.
Fornaciari S; Immunogenetics laboratory, University Hospital of Pisa, Pisa, Italy.
Gabbriellini S; Immunogenetics laboratory, University Hospital of Pisa, Pisa, Italy.
Santini F; Obesity and Lipodystrophy Centre at the Endocrinology Unit, University Hospital of Pisa, Pisa, Italy.
Araújo-Vilar D; Thyroid and Metabolic Diseases Unit (U.E.T.eM.), Centro Singular de Investigación en Medicina Molecular e Enfermidades Crónicas (CIMUS-IDIS), School of Medicine, Universidad de Santiago de Compostela, Santiago de Compostela, Spain.
López-Trascasa M; Complement Research Group, Hospital La Paz Institute for Health Research (IdiPAZ), La Paz University Hospital, Paseo de la Castellana, 261, 28046, Madrid, Spain.; Universidad Autónoma de Madrid, Madrid, Spain.
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Źródło:
Orphanet journal of rare diseases [Orphanet J Rare Dis] 2020 Jan 10; Vol. 15 (1), pp. 9. Date of Electronic Publication: 2020 Jan 10.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Lipodystrophy/*immunology
Lipodystrophy/*metabolism
Adolescent ; Adult ; Aged ; Autoimmunity/physiology ; Child ; Complement C3/metabolism ; Complement C3 Nephritic Factor/metabolism ; Complement C4/metabolism ; Complement Factor B/metabolism ; Female ; Humans ; Male ; Middle Aged ; Properdin/metabolism ; Young Adult
SCR Disease Name:
Lipodystrophy, Partial, Acquired
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy.
Autorzy:
Garam N; Research Laboratory, 3rd Department of Internal Medicine, and MTA-SE Research Group of Immunology and Hematology, Hungarian Academy of Sciences and Semmelweis University, Kútvölgyi St 4, Budapest, H-1125, Hungary.
Prohászka Z; Research Laboratory, 3rd Department of Internal Medicine, and MTA-SE Research Group of Immunology and Hematology, Hungarian Academy of Sciences and Semmelweis University, Kútvölgyi St 4, Budapest, H-1125, Hungary. .
Szilágyi Á; Research Laboratory, 3rd Department of Internal Medicine, and MTA-SE Research Group of Immunology and Hematology, Hungarian Academy of Sciences and Semmelweis University, Kútvölgyi St 4, Budapest, H-1125, Hungary.
Aigner C; Division of Nephrology and Dialysis, Department of Medicine III, Medical University of Vienna, Vienna, Austria.
Schmidt A; Division of Nephrology and Dialysis, Department of Medicine III, Medical University of Vienna, Vienna, Austria.
Gaggl M; Division of Nephrology and Dialysis, Department of Medicine III, Medical University of Vienna, Vienna, Austria.
Sunder-Plassmann G; Division of Nephrology and Dialysis, Department of Medicine III, Medical University of Vienna, Vienna, Austria.
Bajcsi D; 1st Department of Internal Medicine, University of Szeged, Szeged, Hungary.
Brunner J; Department of Pediatrics, Medical University of Innsbruck, Innsbruck, Austria.
Dumfarth A; Department of Medicine III: Nephrology, Transplant Medicine and Rheumatology, Geriatric Department, Ordensklinikum Linz - Elisabethinen, Linz, Austria.
Cejka D; Department of Medicine III: Nephrology, Transplant Medicine and Rheumatology, Geriatric Department, Ordensklinikum Linz - Elisabethinen, Linz, Austria.
Flaschberger S; Hospital of Klagenfurt, Klagenfurt, Austria.
Flögelova H; Division of Nephrology, Department of Pediatrics, Faculty of Medicine, Palacky University and Faculty Hospital in Olomouc, Moravia, Czech Republic.
Haris Á; Department of Nephrology, Szent Margit Hospital, Budapest, Hungary.
Hartmann Á; Department of Pediatrics, University of Pécs, Pécs, Hungary.
Heilos A; Department of Pediatrics and Adolescent Medicine, Division of Pediatric Nephrology and Gastroenterology, Medical University of Vienna, Vienna, Austria.
Mueller T; Department of Pediatrics and Adolescent Medicine, Division of Pediatric Nephrology and Gastroenterology, Medical University of Vienna, Vienna, Austria.
Rusai K; Department of Pediatrics and Adolescent Medicine, Division of Pediatric Nephrology and Gastroenterology, Medical University of Vienna, Vienna, Austria.
Arbeiter K; Department of Pediatrics and Adolescent Medicine, Division of Pediatric Nephrology and Gastroenterology, Medical University of Vienna, Vienna, Austria.
Hofer J; Department of Pediatrics, Medical University of Innsbruck, Innsbruck, Austria.; Institute of Neurology of Senses and Language, Hospital of St John of God, Linz, Austria.; Research Institute for Developmental Medicine, Johannes Kepler University Linz, Linz, Austria.
Jakab D; Department of Pediatrics, University of Szeged, Szeged, Hungary.
Sinkó M; Department of Pediatrics, University of Szeged, Szeged, Hungary.
Szigeti E; Department of Pediatrics, University of Szeged, Szeged, Hungary.
Bereczki C; Department of Pediatrics, University of Szeged, Szeged, Hungary.
Janko V; Medimpax, Bratislava, Slovakia.
Kelen K; 1st Department of Pediatrics, Semmelweis University, Budapest, Hungary.
Reusz GS; 1st Department of Pediatrics, Semmelweis University, Budapest, Hungary.
Szabó AJ; 1st Department of Pediatrics, Semmelweis University, Budapest, Hungary.
Klenk N; FMC Center of Dialysis, Miskolc, Hungary.
Kóbor K; FMC Center of Dialysis, Miskolc, Hungary.
Kojc N; Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
Knechtelsdorfer M; 6th Department of Medicine, Nephrology and Dialysis, Wilhelminenspital, Vienna, Austria.
Laganovic M; Department of Nephrology, Arterial Hypertension, Dialysis and Transplantation, University Hopital Center Zagreb, School of Medicine University of Zagreb, Zagreb, Croatia.
Lungu AC; Fundeni Clinical Institute, Pediatric Nephrology Department, Bucharest, Romania.
Meglic A; Department of Pediatric Nephrology, Division of Pediatrics, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Rus R; Department of Pediatric Nephrology, Division of Pediatrics, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Kersnik-Levart T; Department of Pediatric Nephrology, Division of Pediatrics, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Macioniene E; Nephrology Center, Santaros Klinikos, Medical Faculty, Vilnius University, Vilnius, Lithuania.
Miglinas M; Nephrology Center, Santaros Klinikos, Medical Faculty, Vilnius University, Vilnius, Lithuania.
Pawłowska A; Department of Nephrology, Hypertension and Internal Medicine, School of Medicine, Collegium Medicum, University of Warmia and Mazury, Olsztyn, Poland.
Stompór T; Department of Nephrology, Hypertension and Internal Medicine, School of Medicine, Collegium Medicum, University of Warmia and Mazury, Olsztyn, Poland.
Podracka L; Dept. of Pediatrics, Comenius University, Bratislava, Slovakia.
Rudnicki M; Dept. of Internal Medicine IV - Nephrology and Hypertension, Medical University Innsbruck, Innsbruck, Austria.
Mayer G; Dept. of Internal Medicine IV - Nephrology and Hypertension, Medical University Innsbruck, Innsbruck, Austria.
Romana Rysava; Nephrology Clinic, 1st Faculty of Medicine, Charles University, Prague, Czech Republic.
Reiterova J; Nephrology Clinic, 1st Faculty of Medicine, Charles University, Prague, Czech Republic.
Saraga M; Department of Pathology, University Hospital Split University of Split, School of Medicine, Split, Croatia.
Tomáš Seeman; Department of Pediatrics, 2nd Faculty of Medicine, Charles University Prague, University Hospital Motol, Prague, Czech Republic.
Zieg J; Department of Pediatrics, 2nd Faculty of Medicine, Charles University Prague, University Hospital Motol, Prague, Czech Republic.
Sládková E; Department of Pediatrics, Charles University in Prague, Faculty of Medicine in Pilsen, Prague, Czech Republic.
Szabó T; Department of Pediatrics, University of Debrecen, Debrecen, Hungary.
Capitanescu A; Carol Davila Nephrology Hospital, Bucharest, Romania.
Stancu S; Carol Davila Nephrology Hospital, Bucharest, Romania.
Tisljar M; Department of Nephrology, Dubrava University Hospital, Zagreb, Croatia.
Galesic K; Department of Nephrology, Dubrava University Hospital, Zagreb, Croatia.
Tislér A; 1st Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
Vainumäe I; Department of Pathology of Tartu University Hospital, Tartu, Estonia.
Windpessl M; Internal Medicine IV, Section of Nephrology, Klinikum Wels-Grieskirchen, Wels, Austria.
Zaoral T; Department of Pediatrics, University Hospital and Faculty of Medicine Ostrava, Ostrava, Czech Republic.
Zlatanova G; University Children's Hospital Medical University, Sofia, Bulgaria.
Csuka D; Research Laboratory, 3rd Department of Internal Medicine, and MTA-SE Research Group of Immunology and Hematology, Hungarian Academy of Sciences and Semmelweis University, Kútvölgyi St 4, Budapest, H-1125, Hungary.
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Źródło:
Orphanet journal of rare diseases [Orphanet J Rare Dis] 2019 Nov 08; Vol. 14 (1), pp. 247. Date of Electronic Publication: 2019 Nov 08.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Autoantibodies/*metabolism
Complement C3 Nephritic Factor/*metabolism
Complement System Proteins/*metabolism
Glomerulonephritis, Membranoproliferative/*metabolism
Adolescent ; Adult ; Autoantibodies/immunology ; Female ; Glomerulonephritis, Membranoproliferative/immunology ; Humans ; Kidney Diseases/immunology ; Kidney Diseases/metabolism ; Male ; Young Adult
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Autoimmune abnormalities of the alternative complement pathway in membranoproliferative glomerulonephritis and C3 glomerulopathy.
Autorzy:
Noris M; IRCCS - Istituto di Ricerche Farmacologiche 'Mario Negri', Clinical Research Center for Rare Diseases 'Aldo e Cele Daccò', Ranica, Bergamo, Italy. .
Donadelli R; IRCCS - Istituto di Ricerche Farmacologiche 'Mario Negri', Clinical Research Center for Rare Diseases 'Aldo e Cele Daccò', Ranica, Bergamo, Italy.
Remuzzi G; IRCCS - Istituto di Ricerche Farmacologiche 'Mario Negri', Clinical Research Center for Rare Diseases 'Aldo e Cele Daccò', Ranica, Bergamo, Italy.; Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy.; Unit of Nephrology and Dialysis, Azienda Ospedaliera Papa Giovanni XXIII, Bergamo, Italy.
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Źródło:
Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2019 Aug; Vol. 34 (8), pp. 1311-1323. Date of Electronic Publication: 2018 Jun 09.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't; Review
MeSH Terms:
Complement C3/*immunology
Complement C3 Nephritic Factor/*immunology
Complement Pathway, Alternative/*genetics
Glomerulonephritis, Membranoproliferative/*immunology
Complement C3/genetics ; Complement C3-C5 Convertases, Alternative Pathway/immunology ; Complement Factor B/immunology ; Complement Factor H/immunology ; Glomerular Basement Membrane/immunology ; Glomerular Basement Membrane/pathology ; Glomerular Mesangium/immunology ; Glomerular Mesangium/pathology ; Glomerulonephritis, Membranoproliferative/genetics ; Glomerulonephritis, Membranoproliferative/pathology ; Humans
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
The properdin pathway: an "alternative activation pathway" or a "critical amplification loop" for C3 and C5 activation?
Autorzy:
Harrison RA; Institute of Infection and Immunity, School of Medicine, Cardiff University, Henry Wellcome Building, Heath Park, Cardiff, CF14 4XN, UK. .
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Źródło:
Seminars in immunopathology [Semin Immunopathol] 2018 Jan; Vol. 40 (1), pp. 15-35. Date of Electronic Publication: 2017 Nov 22.
Typ publikacji:
Journal Article; Review
MeSH Terms:
Complement Pathway, Alternative*
Signal Transduction*
Properdin/*metabolism
Animals ; Carrier Proteins/metabolism ; Cell Membrane/immunology ; Cell Membrane/metabolism ; Complement Activation/immunology ; Complement C3 Nephritic Factor/immunology ; Complement C3 Nephritic Factor/metabolism ; Complement C3-C5 Convertases/chemistry ; Complement C3-C5 Convertases/immunology ; Complement C3-C5 Convertases/metabolism ; Complement C3b Inactivator Proteins/immunology ; Complement C3b Inactivator Proteins/metabolism ; Elapid Venoms/immunology ; Elapid Venoms/metabolism ; Host-Pathogen Interactions/immunology ; Humans ; Properdin/immunology ; Protein Binding
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
C3 glomerulopathy and eculizumab: a report on four paediatric cases.
Autorzy:
Lebreton C; Service de Néphrologie Rhumatologie Dermatologie Pédiatriques, Centre de Référence des Maladies Rénales Rares Hôpital Femme Mère Enfant, Hospices Civils de Lyon, 59 boulevard Pinel, 69677, Bron, France.
Bacchetta J; Service de Néphrologie Rhumatologie Dermatologie Pédiatriques, Centre de Référence des Maladies Rénales Rares Hôpital Femme Mère Enfant, Hospices Civils de Lyon, 59 boulevard Pinel, 69677, Bron, France.; Faculté de Médecine Lyon Est, Université Lyon 1, Lyon, France.
Dijoud F; Faculté de Médecine Lyon Est, Université Lyon 1, Lyon, France.; Laboratoire d'Anatomopathologie, Groupe Hospitalier Est, Hospices Civils de Lyon, Bron, France.
Bessenay L; Service de Pédiatrie, CHU Clermont Ferrand, Clermont Ferrand, France.
Fremeaux-Bacchi V; Laboratoire d'Immunologie, Hôpital Georges Pompidou, Assistance publique-Hôpitaux de Paris (AP-HP), Paris, France.
Sellier-Leclerc AL; Service de Néphrologie Rhumatologie Dermatologie Pédiatriques, Centre de Référence des Maladies Rénales Rares Hôpital Femme Mère Enfant, Hospices Civils de Lyon, 59 boulevard Pinel, 69677, Bron, France. .
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Źródło:
Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2017 Jun; Vol. 32 (6), pp. 1023-1028. Date of Electronic Publication: 2017 Feb 24.
Typ publikacji:
Case Reports; Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Antibodies, Monoclonal, Humanized/*therapeutic use
Glomerulonephritis, Membranoproliferative/*drug therapy
Kidney Failure, Chronic/*therapy
Proteinuria/*drug therapy
Adolescent ; Child ; Complement C3/antagonists & inhibitors ; Complement C3/immunology ; Complement C3 Nephritic Factor ; Complement Pathway, Alternative/drug effects ; Female ; Glomerulonephritis, Membranoproliferative/complications ; Glomerulonephritis, Membranoproliferative/immunology ; Glomerulonephritis, Membranoproliferative/urine ; Graft Rejection/drug therapy ; Graft Rejection/immunology ; Humans ; Immunity, Humoral/drug effects ; Kidney/pathology ; Kidney Failure, Chronic/etiology ; Kidney Transplantation/adverse effects ; Male ; Off-Label Use ; Recurrence ; Treatment Outcome
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
C3 Glomerulopathy.
Autorzy:
Riedl M; Cell Biology Program of the Research Institute, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.; Department of Paediatrics, Innsbruck Medical University, Innsbruck, Austria.
Thorner P; Department of Pediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, ON, Canada.; Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada.
Licht C; Cell Biology Program of the Research Institute, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada. .; Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada. .; Department of Paediatrics, University of Toronto, Toronto, ON, Canada. .
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Źródło:
Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2017 Jan; Vol. 32 (1), pp. 43-57. Date of Electronic Publication: 2016 Apr 07.
Typ publikacji:
Journal Article; Review
MeSH Terms:
Complement C3*
Glomerulonephritis, Membranoproliferative/*therapy
Animals ; Complement C3 Nephritic Factor ; Disease Models, Animal ; Glomerulonephritis, Membranoproliferative/diagnosis ; Glomerulonephritis, Membranoproliferative/pathology ; Humans ; Treatment Outcome
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy.
Autorzy:
Corvillo F; Unidad de Inmunología, IdiPAZ, Hospital Universitario La Paz, Madrid, Spain.; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER U754), Madrid, Spain.
Bravo García-Morato M; Unidad de Inmunología, IdiPAZ, Hospital Universitario La Paz, Madrid, Spain.
Nozal P; Unidad de Inmunología, IdiPAZ, Hospital Universitario La Paz, Madrid, Spain.; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER U754), Madrid, Spain.
Garrido S; Unidad de Inmunología, IdiPAZ, Hospital Universitario La Paz, Madrid, Spain.; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER U754), Madrid, Spain.
Tortajada A; Centro De Investigaciones Biológicas, Consejo Superior de Investigaciones Científicas, Madrid, Spain (CIB-CSIC), Centro de Investigación Biomédica en Red de Enfermedades Raras (CBERER U738), Madrid, Spain.
Rodríguez de Córdoba S; Centro De Investigaciones Biológicas, Consejo Superior de Investigaciones Científicas, Madrid, Spain (CIB-CSIC), Centro de Investigación Biomédica en Red de Enfermedades Raras (CBERER U738), Madrid, Spain.
López-Trascasa M; Unidad de Inmunología, IdiPAZ, Hospital Universitario La Paz, Madrid, Spain.; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER U754), Madrid, Spain.
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Źródło:
Clinical and experimental immunology [Clin Exp Immunol] 2016 Apr; Vol. 184 (1), pp. 118-25. Date of Electronic Publication: 2016 Jan 22.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Complement Pathway, Alternative*
Complement C3-C5 Convertases/*immunology
Glomerulonephritis/*immunology
Properdin/*immunology
Proteinuria/*immunology
Adolescent ; Adult ; Biomarkers/blood ; Child ; Complement C3/genetics ; Complement C3/immunology ; Complement C3 Nephritic Factor/genetics ; Complement C3 Nephritic Factor/immunology ; Complement C3-C5 Convertases/genetics ; Complement C5/genetics ; Complement C5/immunology ; Complement Factor B/genetics ; Complement Factor B/immunology ; Complement Inactivating Agents/blood ; Complement Membrane Attack Complex/genetics ; Complement Membrane Attack Complex/immunology ; Female ; Gene Expression Regulation ; Glomerulonephritis/blood ; Glomerulonephritis/genetics ; Glomerulonephritis/pathology ; Humans ; Male ; Middle Aged ; Properdin/genetics ; Proteinuria/blood ; Proteinuria/genetics ; Proteinuria/pathology ; Retrospective Studies ; Severity of Illness Index ; Signal Transduction
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Successful therapy of C3Nef-positive C3 glomerulopathy with plasma therapy and immunosuppression.
Autorzy:
Häffner K; Center for Pediatrics and Adolescent Medicine, University Hospital Freiburg, Mathildenstr. 1, 79106, Freiburg, Germany. .
Michelfelder S; Center for Pediatrics and Adolescent Medicine, University Hospital Freiburg, Mathildenstr. 1, 79106, Freiburg, Germany.
Pohl M; Center for Pediatrics and Adolescent Medicine, University Hospital Freiburg, Mathildenstr. 1, 79106, Freiburg, Germany.
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Źródło:
Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2015 Nov; Vol. 30 (11), pp. 1951-9. Date of Electronic Publication: 2015 May 19.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Plasma Exchange*
Complement C3 Nephritic Factor/*metabolism
Glomerulonephritis, Membranoproliferative/*blood
Glomerulonephritis, Membranoproliferative/*therapy
Immunosuppressive Agents/*therapeutic use
Adolescent ; Adrenal Cortex Hormones/therapeutic use ; Child ; Combined Modality Therapy ; Female ; Humans ; Mycophenolic Acid/analogs & derivatives ; Mycophenolic Acid/therapeutic use
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
Rituximab fails where eculizumab restores renal function in C3nef-related DDD.
Autorzy:
Rousset-Rouvière C; Unité de Néphrologie Pédiatrique, Hôpital La Timone, AP-HM, Université de la Méditerranée, Marseille, France, .
Cailliez M
Garaix F
Bruno D
Laurent D
Tsimaratos M
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Źródło:
Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2014 Jun; Vol. 29 (6), pp. 1107-11. Date of Electronic Publication: 2014 Jan 10.
Typ publikacji:
Case Reports; Journal Article
MeSH Terms:
Antibodies, Monoclonal, Humanized/*therapeutic use
Glomerulonephritis, Membranoproliferative/*drug therapy
Antibodies, Monoclonal, Murine-Derived/therapeutic use ; Child ; Complement C3 Nephritic Factor/analysis ; Complement C3 Nephritic Factor/immunology ; Glomerulonephritis, Membranoproliferative/immunology ; Glomerulonephritis, Membranoproliferative/pathology ; Humans ; Male ; Rituximab
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
C3 nephritic factor can be associated with membranous glomerulonephritis.
Autorzy:
Niel O; Inserm U1091, Genetics of Renal Development and Diseases, UNS Université Nice Sophia Antipolis, 28, avenue de Valrose, 06000, Nice, France, .
Dallocchio A
Thouret MC
Guigonis V
Cassuto É
Frémeaux-Bacchi V
Bérard É
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Źródło:
Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2015 Feb; Vol. 30 (2), pp. 353-5. Date of Electronic Publication: 2014 Nov 14.
Typ publikacji:
Case Reports; Journal Article
MeSH Terms:
Complement C3 Nephritic Factor/*immunology
Glomerulonephritis, Membranous/*immunology
Adolescent ; Child ; Female ; Humans
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
A novel method for direct measurement of complement convertases activity in human serum.
Autorzy:
Blom AM; Department of Laboratory Medicine Malmö, Lund University, Malmö, Sweden.
Volokhina EB
Fransson V
Strömberg P
Berghard L
Viktorelius M
Mollnes TE
López-Trascasa M
van den Heuvel LP
Goodship TH
Marchbank KJ
Okroj M
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Źródło:
Clinical and experimental immunology [Clin Exp Immunol] 2014 Oct; Vol. 178 (1), pp. 142-53.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Complement C3-C5 Convertases/*analysis
Erythrocytes/*enzymology
Immunoassay/*methods
Animals ; Autoantibodies/immunology ; Complement C3 Nephritic Factor/immunology ; Complement C3-C5 Convertases/immunology ; Complement Pathway, Alternative/immunology ; Complement System Proteins/immunology ; Erythrocytes/immunology ; Guinea Pigs ; Half-Life ; Humans ; Rabbits ; Sheep
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Eculizumab therapy in a patient with dense-deposit disease associated with partial lipodystropy.
Autorzy:
Ozkaya O; Pediatric Nephrology Department, Ondokuz Mayis University Faculty of Medicine, Kurupelit, Samsun, Turkey.
Nalcacioglu H
Tekcan D
Genc G
Meydan BC
Ozdemir BH
Baysal MK
Keceligil HT
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Źródło:
Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2014 Jul; Vol. 29 (7), pp. 1283-7. Date of Electronic Publication: 2014 Jan 26.
Typ publikacji:
Case Reports; Journal Article
MeSH Terms:
Antibodies, Monoclonal, Humanized/*therapeutic use
Glomerulonephritis, Membranoproliferative/*drug therapy
Lipodystrophy/*complications
Adolescent ; Complement C3/analysis ; Complement C3 Nephritic Factor/deficiency ; Complement Factor H/deficiency ; Complement Pathway, Alternative ; Female ; Glomerulonephritis, Membranoproliferative/immunology ; Humans
SCR Disease Name:
Lipodystrophy, Partial, Acquired
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
Streptococcal infection as possible trigger for dense deposit disease (C3 glomerulopathy).
Autorzy:
Prasto J; General Pediatrics, Children's Hospital of Philadelphia, 34th Street and Civic Center Blvd, Philadelphia, PA, 19104, USA, .
Kaplan BS
Russo P
Chan E
Smith RJ
Meyers KE
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Źródło:
European journal of pediatrics [Eur J Pediatr] 2014 Jun; Vol. 173 (6), pp. 767-72. Date of Electronic Publication: 2014 Jan 03.
Typ publikacji:
Case Reports; Journal Article
MeSH Terms:
Glomerulonephritis, Membranoproliferative/*microbiology
Kidney/*pathology
Streptococcal Infections/*complications
Child ; Complement C3/analysis ; Complement C3 Nephritic Factor/analysis ; Complement Factor H/analysis ; Female ; Genetic Predisposition to Disease ; Glomerulonephritis, Membranoproliferative/genetics ; Glomerulonephritis, Membranoproliferative/pathology ; Humans ; Phenotype
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
Membranoproliferative glomerulonephritis with C3NeF and genetic complement dysregulation.
Autorzy:
Leroy V; Pediatric Nephrology Department, Université Paris 7, Hôpital Robert Debré, Paris, France. />Fremeaux-Bacchi V
Peuchmaur M
Baudouin V
Deschênes G
Macher MA
Loirat C
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Źródło:
Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2011 Mar; Vol. 26 (3), pp. 419-24. Date of Electronic Publication: 2010 Dec 25.
Typ publikacji:
Case Reports; Journal Article
MeSH Terms:
Complement C3 Nephritic Factor/*analysis
Complement Factor I/*genetics
Glomerulonephritis, Membranoproliferative/*genetics
Glomerulonephritis, Membranoproliferative/*immunology
Kidney/*immunology
Adolescent ; Biopsy ; Child ; Child, Preschool ; Complement Factor H/genetics ; Genetic Predisposition to Disease ; Glomerulonephritis, Membranoproliferative/drug therapy ; Glomerulonephritis, Membranoproliferative/pathology ; Heterozygote ; Humans ; Immunosuppressive Agents/therapeutic use ; Kidney/drug effects ; Kidney/pathology ; Male ; Mutation ; Phenotype ; Time Factors ; Treatment Outcome
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
Membranoproliferative glomerulonephritis.
Autorzy:
Alchi B; Renal Unit, Addenbrooke's Hospital, Cambridge, CB2 0QQ, UK.
Jayne D
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Źródło:
Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2010 Aug; Vol. 25 (8), pp. 1409-18. Date of Electronic Publication: 2009 Nov 12.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't; Review
MeSH Terms:
Glomerulonephritis, Membranoproliferative/*drug therapy
Glomerulonephritis, Membranoproliferative/*pathology
Adult ; Child ; Complement Activation ; Complement C3 Nephritic Factor ; Glomerulonephritis/pathology ; Glomerulonephritis, Membranoproliferative/diagnosis ; Humans ; Kidney/pathology ; Kidney Diseases/pathology ; Kidney Failure, Chronic/pathology ; Kidney Glomerulus/pathology ; Kidney Transplantation
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
A case of dense deposit disease associated with a group A streptococcal infection without the involvement of C3NeF or complement factor H deficiency.
Autorzy:
Suga K; Department of Pediatrics, The Institute of Health Bioscience, The University of Tokushima Graduate School, Tokushima University, Tokushima city, Tokushima, 770-8503, Japan. />Kondo S
Matsuura S
Kinoshita Y
Kitano E
Hatanaka M
Kitamura H
Hidaka Y
Oda T
Kagami S
Pokaż więcej
Źródło:
Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2010 Aug; Vol. 25 (8), pp. 1547-50. Date of Electronic Publication: 2010 Mar 11.
Typ publikacji:
Case Reports; Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Glomerulonephritis, Membranoproliferative/*pathology
Adolescent ; Biopsy/adverse effects ; Complement C3 Nephritic Factor/metabolism ; Complement Factor H/metabolism ; Female ; Follow-Up Studies ; Glomerulonephritis/complications ; Glomerulonephritis/etiology ; Glomerulonephritis/pathology ; Glomerulonephritis, Membranoproliferative/complications ; Humans ; Immunologic Deficiency Syndromes/complications ; Immunologic Deficiency Syndromes/metabolism ; Immunologic Deficiency Syndromes/pathology ; Immunomodulation ; Kidney Glomerulus/metabolism ; Kidney Glomerulus/pathology ; Nephritis/complications ; Nephritis/metabolism ; Nephritis/pathology ; Prednisolone/metabolism ; Proteinuria/complications ; Proteinuria/metabolism ; Proteinuria/pathology ; Receptors, Peptide ; Streptococcal Infections/complications ; Streptococcal Infections/immunology ; Time Factors
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Wyświetlanie 1-20 z 44

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