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Tytuł :
[Cystic fibrosis prognosis in Europa : chronicle of an announced dilemma].
Autorzy :
Lebecque P; Unité de Pneumologie pédiatrique et Mucoviscidose, MontLégia, Liège, Belgique.
Thimmesch M; Unité de Pneumologie pédiatrique et Mucoviscidose, MontLégia, Liège, Belgique.
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Transliterated Title :
Modulateurs et pronostic de la mucoviscidose en Europe : chronique d’un dilemme annoncé.
Źródło :
Revue medicale de Liege [Rev Med Liege] 2021 Mar; Vol. 76 (3), pp. 202-207.
Typ publikacji :
Journal Article
MeSH Terms :
Cystic Fibrosis*/diagnosis
Cystic Fibrosis*/therapy
Quinolones*
Aminophenols ; Aminopyridines ; Benzodioxoles ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Europe ; Humans ; Mutation ; Prognosis
Czasopismo naukowe
Tytuł :
Current Treatment Options for Cystic Fibrosis-Related Liver Disease.
Autorzy :
Staufer K; Department of Visceral Surgery and Medicine, Inselspital, University Hospital Bern, 3010 Bern, Switzerland; ; Tel.: +41-31-63-2-74-88.
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Źródło :
International journal of molecular sciences [Int J Mol Sci] 2020 Nov 14; Vol. 21 (22). Date of Electronic Publication: 2020 Nov 14.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Cystic Fibrosis*/complications
Cystic Fibrosis*/genetics
Cystic Fibrosis*/metabolism
Cystic Fibrosis*/therapy
Liver Diseases*/etiology
Liver Diseases*/genetics
Liver Diseases*/metabolism
Liver Diseases*/pathology
Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Humans ; Ursodeoxycholic Acid/therapeutic use
Czasopismo naukowe
Tytuł :
A systematic review of the clinical and genetic characteristics of Chinese patients with cystic fibrosis.
Autorzy :
Shi R; Division of Pulmonology, Department of Pediatrics, Third Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Wang X; Division of Pulmonology, Department of Pediatrics, Third Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Lu X; Division of Pulmonology, Department of Pediatrics, Third Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Zhu Z; Division of Pulmonology, Department of Pediatrics, Third Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Xu Q; Division of Pulmonology, Department of Pediatrics, Third Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Wang H; Division of Pulmonology, Department of Pediatrics, Third Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Song L; Division of Pulmonology, Department of Pediatrics, Third Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Zhu C; Department of Neonatology, Third Affiliated Hospital and Institute of Neuroscience of Zhengzhou University, Zhengzhou, China.
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Źródło :
Pediatric pulmonology [Pediatr Pulmonol] 2020 Nov; Vol. 55 (11), pp. 3005-3011. Date of Electronic Publication: 2020 Jul 31.
Typ publikacji :
Journal Article; Systematic Review
MeSH Terms :
Asian Continental Ancestry Group*/genetics
Cystic Fibrosis*/diagnosis
Cystic Fibrosis*/genetics
Cystic Fibrosis*/microbiology
Bacterial Infections/diagnosis ; Bacterial Infections/genetics ; Bacterial Infections/microbiology ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Humans
Czasopismo naukowe
Tytuł :
Retroperitoneal Metastasis, with Marked Fibrosis, of Lung Adenocarcinoma after Afatinib Treatment: An Autopsy Case Report.
Autorzy :
Ota H; Department of Respiratory Medicine, Japanese Red Cross Society, Omori Red Cross Hospital, Japan.
Ota T; Department of Respiratory Medicine, Japanese Red Cross Society, Omori Red Cross Hospital, Japan.
Narisawa E; Department of Respiratory Medicine, Japanese Red Cross Society, Omori Red Cross Hospital, Japan.
Chiba H; Department of Gastroenterology, Japanese Red Cross Society, Omori Red Cross Hospital, Japan.
Nakayama H; Department of Surgery, Japanese Red Cross Society, Omori Red Cross Hospital, Japan.
Tsukumo Y; Department of Pathology, Japanese Red Cross Society, Omori Red Cross Hospital, Japan.
Sakamoto A; Department of Pathology, Japanese Red Cross Society, Omori Red Cross Hospital, Japan.
Honma N; Department of Pathology, Toho University School of Medicine, Japan.
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Źródło :
Internal medicine (Tokyo, Japan) [Intern Med] 2020 Nov 15; Vol. 59 (22), pp. 2915-2919. Date of Electronic Publication: 2020 Jul 28.
Typ publikacji :
Case Reports; Journal Article
MeSH Terms :
Adenocarcinoma of Lung*/drug therapy
Lung Neoplasms*/drug therapy
Retroperitoneal Fibrosis*
Retroperitoneal Neoplasms*
Afatinib ; Aged ; Autopsy ; ErbB Receptors ; Female ; Fibrosis ; Humans
Czasopismo naukowe
Tytuł :
[Rare diseases on the agenda for innovation in health: progress and challenges with cystic fibrosis].
Autorzy :
Amaral MB; Faculdade de Educação, Universidade Federal Fluminense, Niterói, Brasil.; Escola Nacional de Saúde Pública Sergio Arouca, Fundação Oswaldo Cruz, Rio de Janeiro, Brasil.
Rego S; Escola Nacional de Saúde Pública Sergio Arouca, Fundação Oswaldo Cruz, Rio de Janeiro, Brasil.
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Transliterated Title :
Doenças raras na agenda da inovação em saúde: avanços e desafios na fibrose cística.
Źródło :
Cadernos de saude publica [Cad Saude Publica] 2020 Dec 18; Vol. 36 (12), pp. e00115720. Date of Electronic Publication: 2020 Dec 18 (Print Publication: 2020).
Typ publikacji :
Journal Article
MeSH Terms :
Cystic Fibrosis*/therapy
Brazil ; Cystic Fibrosis Transmembrane Conductance Regulator ; Humans ; Precision Medicine ; Rare Diseases/therapy
Czasopismo naukowe
Tytuł :
Human Fibrosis: Is There Evidence for a Genetic Predisposition in Musculoskeletal Tissues?
Autorzy :
Dagneaux L; Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN.
Owen AR; Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN.
Bettencourt JW; Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN.
Barlow JD; Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN.
Amadio PC; Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN.
Kocher JP; Department of Bioinformatics, Mayo Clinic, Rochester, MN.
Morrey ME; Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN.
Sanchez-Sotelo J; Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN.
Berry DJ; Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN.
van Wijnen AJ; Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN.
Abdel MP; Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN.
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Źródło :
The Journal of arthroplasty [J Arthroplasty] 2020 Nov; Vol. 35 (11), pp. 3343-3352. Date of Electronic Publication: 2020 Jun 04.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
MeSH Terms :
Genetic Predisposition to Disease*
Pulmonary Fibrosis*/pathology
Fibrosis ; Humans ; Knee ; Knee Joint/pathology
Czasopismo naukowe
Tytuł :
Rats Race to Keep Pace in the Growing Cystic Fibrosis Model Space.
Autorzy :
Hisert KB; National Jewish Health, Denver, Colorado.
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Źródło :
American journal of respiratory and critical care medicine [Am J Respir Crit Care Med] 2020 Nov 01; Vol. 202 (9), pp. 1212-1214.
Typ publikacji :
Editorial; Comment
MeSH Terms :
Cystic Fibrosis*/genetics
Aminophenols ; Animals ; Cystic Fibrosis Transmembrane Conductance Regulator ; Mucus ; Quinolones ; Rats
Opinia redakcyjna
Tytuł :
Cystic fibrosis newborn screening: the importance of bloodspot sample quality.
Autorzy :
Doull I; Department of Paediatric Respiratory Medicine and Paediatric Cystic Fibrosis Centre, Children's Hospital for Wales, Cardiff, UK .
Course CW; Department of Paediatric Respiratory Medicine and Paediatric Cystic Fibrosis Centre, Children's Hospital for Wales, Cardiff, UK.
Hanks RE; Department of Paediatric Respiratory Medicine and Paediatric Cystic Fibrosis Centre, Children's Hospital for Wales, Cardiff, UK.
Southern KW; Department of Women's and Children's Health, University of Liverpool, Liverpool, UK.
Forton JT; Department of Paediatric Respiratory Medicine and Paediatric Cystic Fibrosis Centre, Children's Hospital for Wales, Cardiff, UK.
Thia LP; Department of Paediatric Respiratory Medicine and Paediatric Cystic Fibrosis Centre, Children's Hospital for Wales, Cardiff, UK.
Moat SJ; Department of Medical Biochemistry, Immunology & Toxicology, University Hospital of Wales, Cardiff, UK.; School of Medicine, Cardiff University, Cardiff, UK.
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Źródło :
Archives of disease in childhood [Arch Dis Child] 2021 Mar; Vol. 106 (3), pp. 253-257. Date of Electronic Publication: 2020 Aug 28.
Typ publikacji :
Journal Article
MeSH Terms :
Cystic Fibrosis/*diagnosis
Dried Blood Spot Testing/*statistics & numerical data
Neonatal Screening/*methods
Trypsinogen/*blood
Chlorides/analysis ; Cystic Fibrosis/blood ; Cystic Fibrosis/epidemiology ; Cystic Fibrosis/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Dried Blood Spot Testing/methods ; False Negative Reactions ; Humans ; Incidence ; Infant ; Infant, Newborn ; Meconium Ileus/epidemiology ; Meconium Ileus/etiology ; Predictive Value of Tests ; Retrospective Studies ; Selection Bias ; Sweat/chemistry ; Wales/epidemiology
Czasopismo naukowe
Tytuł :
Macrophage dysfunction in cystic fibrosis: Nature or nurture?
Autorzy :
Turton KB; Wellcome-Wolfson Institute for Experimental Medicine, Queen's University Belfast, Belfast, United Kingdom.
Ingram RJ; Wellcome-Wolfson Institute for Experimental Medicine, Queen's University Belfast, Belfast, United Kingdom.
Valvano MA; Wellcome-Wolfson Institute for Experimental Medicine, Queen's University Belfast, Belfast, United Kingdom.
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Źródło :
Journal of leukocyte biology [J Leukoc Biol] 2021 Mar; Vol. 109 (3), pp. 573-582. Date of Electronic Publication: 2020 Jul 17.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't; Review
MeSH Terms :
Cystic Fibrosis/*pathology
Cystic Fibrosis/*physiopathology
Macrophages/*pathology
Animals ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Humans ; Models, Biological ; Mutation/genetics ; Phagosomes/metabolism
Czasopismo naukowe
Tytuł :
Dysfunctional Inflammation in Cystic Fibrosis Airways: From Mechanisms to Novel Therapeutic Approaches.
Autorzy :
Ghigo A; Department of Molecular Biotechnology and Health Sciences, Molecular Biotechnology Center, University of Torino, 10126 Torino, Italy.
Prono G; Department of Molecular Biotechnology and Health Sciences, Molecular Biotechnology Center, University of Torino, 10126 Torino, Italy.
Riccardi E; Postgraduate School in Respiratory Medicine, University of Torino, 10126 Torino, Italy.
De Rose V; Department of Molecular Biotechnology and Health Sciences, Molecular Biotechnology Center, University of Torino, 10126 Torino, Italy.
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Źródło :
International journal of molecular sciences [Int J Mol Sci] 2021 Feb 16; Vol. 22 (4). Date of Electronic Publication: 2021 Feb 16.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Anti-Inflammatory Agents/*therapeutic use
Cystic Fibrosis/*drug therapy
Inflammation/*drug therapy
Animals ; Anti-Bacterial Agents/pharmacology ; Anti-Bacterial Agents/therapeutic use ; Anti-Inflammatory Agents/pharmacology ; Azithromycin/pharmacology ; Azithromycin/therapeutic use ; COVID-19/complications ; COVID-19/drug therapy ; COVID-19/metabolism ; Cannabinoids/pharmacology ; Cannabinoids/therapeutic use ; Cystic Fibrosis/complications ; Cystic Fibrosis/metabolism ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Eicosanoids/metabolism ; Humans ; Inflammation/complications ; Inflammation/metabolism ; Protein Kinase Inhibitors/pharmacology ; Protein Kinase Inhibitors/therapeutic use ; Roscovitine/pharmacology ; Roscovitine/therapeutic use ; Signal Transduction/drug effects ; Thymalfasin/pharmacology ; Thymalfasin/therapeutic use
Czasopismo naukowe
Tytuł :
Sustained recovery of exocrine pancreatic function in a teenager with cystic fibrosis treated with ivacaftor.
Autorzy :
Smith H; Faculty of Medicine, MD Undergraduate Program, University of British Columbia, Vancouver, British Columbia, Canada.
Rayment JH; Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada.; Division of Respiratory Medicine, British Columbia Children's Hospital, Vancouver, British Columbia, Canada.
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Źródło :
Pediatric pulmonology [Pediatr Pulmonol] 2020 Oct; Vol. 55 (10), pp. 2493-2494. Date of Electronic Publication: 2020 Jul 17.
Typ publikacji :
Letter; Comment
MeSH Terms :
Cystic Fibrosis*/drug therapy
Cystic Fibrosis*/genetics
Gastrointestinal Microbiome*
Adolescent ; Aminophenols/therapeutic use ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Humans ; Quinolones
Opinia redakcyjna
Tytuł :
Ivacaftor in Infants Aged 4 to <12 Months with Cystic Fibrosis and a Gating Mutation. Results of a Two-Part Phase 3 Clinical Trial.
Autorzy :
Davies JC; National Heart & Lung Institute, Imperial College London and Royal Brompton Hospital, London, United Kingdom.
Wainwright CE; Queensland Children's Hospital, University of Queensland, Brisbane, Queensland, Australia.
Sawicki GS; Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Higgins MN; Vertex Pharmaceuticals Incorporated, Boston, Massachusetts; and.
Campbell D; Vertex Pharmaceuticals Incorporated, Boston, Massachusetts; and.
Harris C; Vertex Pharmaceuticals Incorporated, Boston, Massachusetts; and.
Panorchan P; Vertex Pharmaceuticals Incorporated, Boston, Massachusetts; and.
Haseltine E; Vertex Pharmaceuticals Incorporated, Boston, Massachusetts; and.
Tian S; Vertex Pharmaceuticals Incorporated, Boston, Massachusetts; and.
Rosenfeld M; Seattle Children's Hospital, University of Washington School of Medicine, Seattle, Washington.
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Źródło :
American journal of respiratory and critical care medicine [Am J Respir Crit Care Med] 2021 Mar 01; Vol. 203 (5), pp. 585-593.
Typ publikacji :
Clinical Trial, Phase III; Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Aminophenols/*therapeutic use
Chloride Channel Agonists/*therapeutic use
Cystic Fibrosis/*drug therapy
Quinolones/*therapeutic use
Aminophenols/pharmacokinetics ; Chloride Channel Agonists/pharmacokinetics ; Chlorides/metabolism ; Cough/epidemiology ; Cystic Fibrosis/genetics ; Cystic Fibrosis/metabolism ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Exocrine Pancreatic Insufficiency/metabolism ; Female ; Fever/epidemiology ; Genotype ; Humans ; Infant ; Ion Channel Gating/genetics ; Male ; Mutation ; Otitis Media/epidemiology ; Pancreatic Elastase/metabolism ; Quinolones/pharmacokinetics ; Respiratory Tract Infections/epidemiology ; Rhinorrhea/epidemiology ; Sweat/metabolism ; Treatment Outcome ; Vomiting/epidemiology
Czasopismo naukowe
Tytuł :
Mechanisms of progressive fibrosis in connective tissue disease (CTD)-associated interstitial lung diseases (ILDs).
Autorzy :
Spagnolo P; Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova School of Medicine and Surgery, Padova, Italy .
Distler O; Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
Ryerson CJ; Department of Medicine, The University of British Columbia, Vancouver, British Columbia, Canada.
Tzouvelekis A; Department of Respiratory and Internal Medicine, University of Patras Faculty of Medicine, Patras, Greece.
Lee JS; School of Medicine, University of Colorado Denver - Anschutz Medical Campus, Aurora, Colorado, USA.
Bonella F; Center for Interstitial and Rare Lung Disease Unit, University of Duisburg-Essen, Ruhrlandklinik, Essen, Germany.
Bouros D; Department of Pneumonology, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Hoffmann-Vold AM; Department of Rheumatology, Oslo University Hospital, Oslo, Norway.
Crestani B; Inserm U1152, Université de Paris, F-75018, Paris, France.; Department of Pneumonology, Hôpital Bichat, Assistance Publique - Hôpitaux de Paris, F-75018, Paris, France.
Matteson EL; Division of Rheumatology and Department of Health Sciences Research, Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA.
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Źródło :
Annals of the rheumatic diseases [Ann Rheum Dis] 2021 Feb; Vol. 80 (2), pp. 143-150. Date of Electronic Publication: 2020 Oct 09.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Connective Tissue Diseases/*pathology
Lung/*pathology
Lung Diseases, Interstitial/*pathology
Pulmonary Fibrosis/*etiology
Connective Tissue Diseases/complications ; Disease Progression ; Fibrosis ; Humans ; Lung Diseases, Interstitial/complications ; Phenotype
Czasopismo naukowe
Tytuł :
The first five-year evaluation of cystic fibrosis neonatal screening program in São Paulo State, Brazil.
Autorzy :
Maciel LMZ; Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, Brasil.
Magalhães PKR; Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, Brasil.
Ciampo IRLD; Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, Brasil.
Sousa MLB; Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, Brasil.
Fernandes MIM; Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, Brasil.
Sawamura R; Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, Brasil.
Bittar RR; Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, Brasil.
Molfetta GA; Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, Brasil.
Silva Júnior WAD; Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, Brasil.
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Źródło :
Cadernos de saude publica [Cad Saude Publica] 2020 Oct 26; Vol. 36 (10), pp. e00049719. Date of Electronic Publication: 2020 Oct 26 (Print Publication: 2020).
Typ publikacji :
Journal Article
MeSH Terms :
Cystic Fibrosis*/diagnosis
Cystic Fibrosis*/epidemiology
Neonatal Screening*
Brazil/epidemiology ; Child ; Cystic Fibrosis Transmembrane Conductance Regulator ; Humans ; Infant ; Infant, Newborn ; Trypsinogen
Czasopismo naukowe
Tytuł :
GPRC5A reduction contributes to pollutant benzo[a]pyrene injury via aggravating murine fibrosis, leading to poor prognosis of IIP patients.
Autorzy :
Huang Z; Department of Pathology, Tongji University Affiliated Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China; Tongji University School of Medicine, Tongji University, Shanghai 200092, China.
Wang S; Department of Pathology, Tongji University Affiliated Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China.
Liu Y; Department of Pathology, Tongji University Affiliated Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China.
Fan L; Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China.
Zeng Y; Department of Pathology, Tongji University Affiliated Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China.
Han H; Department of Pathology, Tongji University Affiliated Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China.
Zhang H; Department of Pathology, Tongji University Affiliated Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China.
Yu X; Department of Pathology, Tongji University Affiliated Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China.
Zhang Y; Department of Pathology, Tongji University Affiliated Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China.
Huang D; Department of Pathology, Tongji University Affiliated Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China.
Wu Y; Department of Pathology, Tongji University Affiliated Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China.
Jiang W; Department of Pathology, Tongji University School of Medicine, Tongji University, Shanghai 200092, China.
Zhu P; Department of Pathology, Tongji University Affiliated Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China. Electronic address: .
Zhu X; Department of Pathology, Tongji University Affiliated Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China. Electronic address: .
Yi X; Department of Pathology, Tongji University Affiliated Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China; Tongji University School of Medicine, Tongji University, Shanghai 200092, China. Electronic address: .
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Źródło :
The Science of the total environment [Sci Total Environ] 2020 Oct 15; Vol. 739, pp. 139923. Date of Electronic Publication: 2020 Jun 03.
Typ publikacji :
Journal Article
MeSH Terms :
Benzo(a)pyrene*/toxicity
Environmental Pollutants*
Idiopathic Interstitial Pneumonias*
Idiopathic Pulmonary Fibrosis*
Animals ; Fibrosis ; Humans ; Lung ; Mice ; Mice, Inbred C57BL ; Receptors, G-Protein-Coupled
Czasopismo naukowe
Tytuł :
Setting a new standard in cystic fibrosis newborn screening illustrates controversial issues as new data emerge.
Autorzy :
Farrell PM; University of Wisconsin, Pediatrics and Population Health Sciences, Madison, WI, USA. .
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Źródło :
European journal of human genetics : EJHG [Eur J Hum Genet] 2020 Oct; Vol. 28 (10), pp. 1305-1306. Date of Electronic Publication: 2020 Jul 13.
Typ publikacji :
Letter; Comment
MeSH Terms :
Cystic Fibrosis*/diagnosis
Cystic Fibrosis*/genetics
Neonatal Screening*
Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Humans ; Infant, Newborn ; Ireland ; Reference Standards
Opinia redakcyjna
Tytuł :
Pancreatic complications in children with cystic fibrosis.
Autorzy :
Sellers ZM; Pediatric Gastroenterology, Hepatology, and Nutrition, Stanford University, Stanford, California, USA.
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Źródło :
Current opinion in pediatrics [Curr Opin Pediatr] 2020 Oct; Vol. 32 (5), pp. 661-667.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
MeSH Terms :
Cystic Fibrosis*/complications
Cystic Fibrosis*/therapy
Diabetes Mellitus*
Exocrine Pancreatic Insufficiency*/etiology
Exocrine Pancreatic Insufficiency*/therapy
Pancreatitis*/etiology
Pancreatitis*/therapy
Child ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Humans ; Mutation ; Pancreas
Czasopismo naukowe
Tytuł :
Membrane Lipids and CFTR: The Yin/Yang of Efficient Ceramide Metabolism.
Autorzy :
Bonfield TL; Case Western Reserve University School of Medicine Cleveland, Ohio.
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Źródło :
American journal of respiratory and critical care medicine [Am J Respir Crit Care Med] 2020 Oct 15; Vol. 202 (8), pp. 1074-1075.
Typ publikacji :
Editorial; Comment
MeSH Terms :
Cystic Fibrosis*/genetics
Infections*
Acid Ceramidase ; Ceramides ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Humans ; Inflammation ; Membrane Lipids
Opinia redakcyjna
Tytuł :
Paracellular bicarbonate flux across human cystic fibrosis airway epithelia tempers changes in airway surface liquid pH.
Autorzy :
Thornell IM; Department of Internal Medicine, Pappajohn Biomedical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
Rehman T; Department of Internal Medicine, Pappajohn Biomedical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
Pezzulo AA; Department of Internal Medicine, Pappajohn Biomedical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
Welsh MJ; Department of Internal Medicine, Pappajohn Biomedical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA, USA.; Department of Molecular Physiology and Biophysics, Pappajohn Biomedical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA, USA.; Howard Hughes Medical Institute, University of Iowa, Iowa City, IA, USA.
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Źródło :
The Journal of physiology [J Physiol] 2020 Oct; Vol. 598 (19), pp. 4307-4320. Date of Electronic Publication: 2020 Jul 24.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms :
Bicarbonates*/metabolism
Cystic Fibrosis*
Cells, Cultured ; Cystic Fibrosis Transmembrane Conductance Regulator ; Epithelium/metabolism ; Humans ; Hydrogen-Ion Concentration ; Respiratory Mucosa/metabolism ; Respiratory System
Czasopismo naukowe
Tytuł :
Influence of pancreatic status, CFTR mutations, Staphylococcus aureus and/or Pseudomonas aeruginosa infection/colonization on lung function in cystic fibrosis during a 2-year follow-up period.
Autorzy :
Pascoal MA; Department of Pediatrics, Faculty of Medical Sciences, University of Campinas, Tessália Vieira de Camargo Street, 126, Cidade Universitária Zeferino Vaz, Barão Geraldo, ZIP CODE: 13083-887, Campinas, São Paulo, Brazil.
Marson FAL; Department of Pediatrics, Faculty of Medical Sciences, University of Campinas, Tessália Vieira de Camargo Street, 126, Cidade Universitária Zeferino Vaz, Barão Geraldo, ZIP CODE: 13083-887, Campinas, São Paulo, Brazil. .; Department of Medical Genetics and Genomic Medicine, Faculty of Medical Sciences, University of Campinas, Tessália Vieira de Camargo Street, 126, Cidade Universitária Zeferino Vaz, Barão Geraldo, ZIP CODE: 13083-887, Campinas, São Paulo, Brazil. .; Department of Clinical Medicine, Faculty of Medical Sciences, University of Campinas, Tessália Vieira de Camargo Street, 126, Cidade Universitária Zeferino Vaz, Barão Geraldo, ZIP CODE: 13083-887, Campinas, São Paulo, Brazil. .; Post-Graduation in Health Sciences, Laboratory of Medical Genetics and Genome Medicine, São Francisco University, Avenida São Francisco de Assis, 218, CEP: 12916-900, Jardim São José, Bragança Paulista, São Paulo, Brazil. .
Paschoal IA; Department of Clinical Medicine, Faculty of Medical Sciences, University of Campinas, Tessália Vieira de Camargo Street, 126, Cidade Universitária Zeferino Vaz, Barão Geraldo, ZIP CODE: 13083-887, Campinas, São Paulo, Brazil.
Levy CE; Department of Clinical Pathology, Faculty of Medical Sciences, University of Campinas, Tessália Vieira de Camargo Street, 126, Cidade Universitária Zeferino Vaz, Barão Geraldo, ZIP CODE: 13083-887, Campinas, São Paulo, Brazil. .
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Źródło :
Wiener klinische Wochenschrift [Wien Klin Wochenschr] 2020 Oct; Vol. 132 (19-20), pp. 572-580. Date of Electronic Publication: 2020 Apr 30.
Typ publikacji :
Journal Article
MeSH Terms :
Cystic Fibrosis*/genetics
Pseudomonas Infections*/genetics
Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Female ; Follow-Up Studies ; Humans ; Lung ; Male ; Mutation/genetics ; Pseudomonas aeruginosa/genetics ; Spirometry ; Staphylococcus aureus/genetics
Czasopismo naukowe

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