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Tytuł :
CFTR Modulators in People with Cystic Fibrosis: Real-World Evidence in France.
Autorzy :
Regard L; French Cystic Fibrosis National Reference Center, Department of Respiratory Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, 75014 Paris, France.; Institut Cochin, Université de Paris Cité, INSERM U1016, 75014 Paris, France.; ERN Lung Cystic Fibrosis Network, Frankfurt, Germany.
Martin C; French Cystic Fibrosis National Reference Center, Department of Respiratory Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, 75014 Paris, France.; Institut Cochin, Université de Paris Cité, INSERM U1016, 75014 Paris, France.; ERN Lung Cystic Fibrosis Network, Frankfurt, Germany.
Burnet E; French Cystic Fibrosis National Reference Center, Department of Respiratory Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, 75014 Paris, France.; Institut Cochin, Université de Paris Cité, INSERM U1016, 75014 Paris, France.; ERN Lung Cystic Fibrosis Network, Frankfurt, Germany.
Da Silva J; French Cystic Fibrosis National Reference Center, Department of Respiratory Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, 75014 Paris, France.; ERN Lung Cystic Fibrosis Network, Frankfurt, Germany.
Burgel PR; French Cystic Fibrosis National Reference Center, Department of Respiratory Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, 75014 Paris, France.; Institut Cochin, Université de Paris Cité, INSERM U1016, 75014 Paris, France.; ERN Lung Cystic Fibrosis Network, Frankfurt, Germany.
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Źródło :
Cells [Cells] 2022 May 28; Vol. 11 (11). Date of Electronic Publication: 2022 May 28.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Cystic Fibrosis*/drug therapy
Cystic Fibrosis*/genetics
Cystic Fibrosis*/metabolism
Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; France ; Homozygote ; Humans ; Quality of Life
Czasopismo naukowe
Tytuł :
CFTR modulation with elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis assessed by the β-adrenergic sweat rate assay.
Autorzy :
Pallenberg ST; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.
Junge S; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.
Ringshausen FC; Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany.
Sauer-Heilborn A; Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany.
Hansen G; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany.
Dittrich AM; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany.
Tümmler B; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany.
Nietert M; Department of Medical Bioinformatics, University Medical Center Göttingen, Göttingen, Germany. Electronic address: .
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Źródło :
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2022 May; Vol. 21 (3), pp. 442-447. Date of Electronic Publication: 2021 Oct 29.
Typ publikacji :
Journal Article
MeSH Terms :
Cystic Fibrosis*/diagnosis
Cystic Fibrosis*/drug therapy
Cystic Fibrosis*/genetics
Adrenergic Agents/analysis ; Adrenergic Agents/therapeutic use ; Aminophenols ; Benzodioxoles ; Chloride Channel Agonists ; Chlorides/analysis ; Cystic Fibrosis Transmembrane Conductance Regulator/analysis ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Drug Combinations ; Humans ; Indoles ; Pyrazoles ; Pyridines ; Pyrrolidines ; Quinolones ; Sweat/chemistry
Czasopismo naukowe
Tytuł :
Worldwide rates of diagnosis and effective treatment for cystic fibrosis.
Autorzy :
Guo J; Faculty of Medicine, Imperial College London, United Kingdom. Electronic address: .
Garratt A; Department of Medical Microbiology, Public Health Wales, United Kingdom.
Hill A; Department of Pharmacology and Therapeutics, University of Liverpool, United Kingdom.
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Źródło :
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2022 May; Vol. 21 (3), pp. 456-462. Date of Electronic Publication: 2022 Feb 04.
Typ publikacji :
Journal Article
MeSH Terms :
Cystic Fibrosis*/diagnosis
Cystic Fibrosis*/drug therapy
Cystic Fibrosis*/epidemiology
Aminophenols ; Benzodioxoles ; Chloride Channel Agonists ; Cystic Fibrosis Transmembrane Conductance Regulator ; Humans ; Registries ; Treatment Outcome
Czasopismo naukowe
Tytuł :
Pregnancy in cystic fibrosis: Review of the literature and expert recommendations.
Autorzy :
Jain R; Department of Medicine, University of Texas Southwestern, 5323 Harry Hines Blvd., Dallas, TX 75390-8558, United States. Electronic address: .
Kazmerski TM; Department of Pediatrics, University of Pittsburgh School of Medicine, 120 Lytton Ave. Suite M060 University Center, Pittsburgh, PA 15213, United States.
Zuckerwise LC; Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Vanderbilt University Medical Center, 1161 21st Avenue South, B-1100 MCN, Nashville, TN 37232, United States.
West NE; Department of Medicine, Johns Hopkins University, 1830 E. Monument St. 5th Floor, Baltimore, MD 21205, United States.
Montemayor K; Department of Medicine, Johns Hopkins University, 1830 E. Monument St. 5th Floor, Baltimore, MD 21205, United States.
Aitken ML; Department of Medicine, University of Washington, 1959 NE Pacific Street - Rm BB 1361, Seattle WA 98195-6522, United States.
Cheng E; Maternal Fetal Medicine, Maternal Fetal Medicine, Medical Genetics, University of Washington, United States.
Roe AH; Department of Obstetrics and Gynecology, University of Pennsylvania Perelman School of Medicine, 3400 Spruce Street, 1000 Courtyard, Philadelphia, PA 19106, United States.
Wilson A; Department of Medicine, Cystic Fibrosis Clinical Research, National Jewish Health, 1400 Jackson Street, K333b, Denver, CO 80206, United States.
Mann C; Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Vanderbilt University Medical Center, 1161 21st Avenue South, B-1100 MCN, Nashville, TN 37232, United States.
Ladores S; School of Nursing, The University of Alabama at Birmingham, NB 470L, 1720 2nd Avenue South, Birmingham, AL 35294-1210, United States.
Sjoberg J; Adult with Cystic Fibrosis, 130 Johnston Dr, Highland Park, IL 60035, United States.
Poranski M; Adult with Cystic Fibrosis, 2700 University Ave. W. Apt. 416, St. Paul, MN 55114, United States.
Taylor-Cousar JL; Departments of Medicine and Pediatrics, National Jewish Health, 1400 Jackson Street; J318, Denver, CO 80206, United States.
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Źródło :
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2022 May; Vol. 21 (3), pp. 387-395. Date of Electronic Publication: 2021 Aug 26.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Cystic Fibrosis*/epidemiology
Cystic Fibrosis*/genetics
Cystic Fibrosis*/therapy
Adult ; Child ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Female ; Humans ; Ion Transport ; Pregnancy
Czasopismo naukowe
Tytuł :
Olfactory dysfunction in people with cystic fibrosis with at least one copy of F508del.
Autorzy :
Beswick DM; Department of Otolaryngology-Head and Neck Surgery, University of California, Los Angeles, CA.
Humphries SM; Department of Radiology, National Jewish Health, Denver, CO.
Balkissoon CD; Clinical Research Services, National Jewish Health, Denver, CO.
Strand M; Division of Biostatistics, National Jewish Health, Denver, CO.
E Miller J; Department of Otolaryngology-Head and Neck Surgery, University of California, Los Angeles, CA.
Khatiwada A; Division of Biostatistics, National Jewish Health, Denver, CO.
Vladar EK; Department of Medicine, Division of Pulmonary Sciences and Critical Care Medicine, Aurora, CO.; Department of Cell and Developmental Biology, University of Colorado School of Medicine, Aurora, CO.
Lynch DA; Department of Radiology, National Jewish Health, Denver, CO.
Taylor-Cousar JL; Department of Medicine, Division of Pulmonary Sciences and Critical Care Medicine, Aurora, CO.; Departments of Medicine and Pediatrics, National Jewish Health, Denver, CO.
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Źródło :
International forum of allergy & rhinology [Int Forum Allergy Rhinol] 2022 Jul; Vol. 12 (7), pp. 963-966. Date of Electronic Publication: 2022 Jan 14.
Typ publikacji :
Journal Article
MeSH Terms :
Cystic Fibrosis*/complications
Cystic Fibrosis*/genetics
Olfaction Disorders*/genetics
Aminophenols ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Humans ; Mutation
Czasopismo naukowe
Tytuł :
Paediatric Ulcerative Colitis Is a Fibrotic Disease and Is Linked with Chronicity of Inflammation.
Autorzy :
Gordon IO; Department of Pathology, Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic Foundation, Cleveland, OH, USA.
Abushamma S; Department of Gastroenterology, Washington University School of Medicine, Barnes Jewish Hospital, St Louis, MO,USA.
Kurowski JA; Department of Paediatric Gastroenterology, Hepatology and Nutrition, Cleveland Clinic Foundation, Cleveland, OH, USA.
Holubar SD; Department of Colorectal Surgery, Digestive Diseases and Surgery Institute, Cleveland Clinic Foundation, Cleveland, OH, USA.
Kou L; Department of Quantitative Health Sciences, Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, OH, USA.
Lyu R; Department of Quantitative Health Sciences, Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, OH, USA.
Rieder F; Department of Gastroenterology, Hepatology and Nutrition, Digestive Diseases and Surgery Institute, and Department of Inflammation and Immunity, Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, OH, USA.
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Źródło :
Journal of Crohn's & colitis [J Crohns Colitis] 2022 Jun 24; Vol. 16 (5), pp. 804-821.
Typ publikacji :
Journal Article
MeSH Terms :
Colitis, Ulcerative*/complications
Colitis, Ulcerative*/pathology
Colitis, Ulcerative*/surgery
Oral Submucous Fibrosis*/complications
Oral Submucous Fibrosis*/pathology
Adolescent ; Adult ; Child ; Child, Preschool ; Cross-Sectional Studies ; Fibrosis ; Humans ; Infant ; Inflammation/pathology ; Intestinal Mucosa/pathology
SCR Disease Name :
Pediatric ulcerative colitis
Czasopismo naukowe
Tytuł :
A highly selective, cell-permeable furin inhibitor BOS-318 rescues key features of cystic fibrosis airway disease.
Autorzy :
Douglas LEJ; School of Pharmacy, Queen's University Belfast, Belfast BT9 7BL, Northern Ireland, UK.
Reihill JA; School of Pharmacy, Queen's University Belfast, Belfast BT9 7BL, Northern Ireland, UK.
Ho MWY; GlaxoSmithKline Research and Development, Collegeville, PA 19426, USA.
Axten JM; GlaxoSmithKline Research and Development, Collegeville, PA 19426, USA.
Campobasso N; GlaxoSmithKline Research and Development, Collegeville, PA 19426, USA.
Schneck JL; GlaxoSmithKline Research and Development, Collegeville, PA 19426, USA.
Rendina AR; GlaxoSmithKline Research and Development, Collegeville, PA 19426, USA.
Wilcoxen KM; Boston Pharmaceuticals, Cambridge, MA 02142, USA.
Martin SL; School of Pharmacy, Queen's University Belfast, Belfast BT9 7BL, Northern Ireland, UK. Electronic address: .
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Źródło :
Cell chemical biology [Cell Chem Biol] 2022 Jun 16; Vol. 29 (6), pp. 947-957.e8. Date of Electronic Publication: 2022 Feb 23.
Typ publikacji :
Journal Article
MeSH Terms :
Cystic Fibrosis*/drug therapy
Cystic Fibrosis*/metabolism
Furin*/antagonists & inhibitors
Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Epithelial Sodium Channels/genetics ; Epithelial Sodium Channels/metabolism ; Humans ; Mucociliary Clearance
Czasopismo naukowe
Tytuł :
The MIR100HG/miR-29a-3p/Tab1 axis modulates TGF-β1-induced fibrotic changes in type II alveolar epithelial cells BLM-caused lung fibrogenesis in mice.
Autorzy :
Guan S; Department of Respiratory and Critical Care Medicine, The First People's Hospital of Changzhou, Changzhou, Jiangsu 213000, China.
Liu H; Department of Respiratory and Critical Care Medicine, the Third Clinical Medicine School of Soochow University, Changzhou, Jiangsu 213000, China.
Zhou J; Department of Respiratory and Critical Care Medicine, The First People's Hospital of Changzhou, Changzhou, Jiangsu 213000, China. Electronic address: .
Zhang Q; Department of Respiratory and Critical Care Medicine, The First People's Hospital of Changzhou, Changzhou, Jiangsu 213000, China.
Bi H; Department of Respiratory and Critical Care Medicine, The First People's Hospital of Changzhou, Changzhou, Jiangsu 213000, China.
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Źródło :
Toxicology letters [Toxicol Lett] 2022 Jun 15; Vol. 363, pp. 45-54. Date of Electronic Publication: 2022 Apr 23.
Typ publikacji :
Journal Article
MeSH Terms :
Idiopathic Pulmonary Fibrosis*/chemically induced
Idiopathic Pulmonary Fibrosis*/genetics
Idiopathic Pulmonary Fibrosis*/metabolism
MicroRNAs*/metabolism
RNA, Long Noncoding*/genetics
RNA, Long Noncoding*/metabolism
Adaptor Proteins, Signal Transducing/metabolism ; Alveolar Epithelial Cells/metabolism ; Animals ; Bleomycin/toxicity ; Epithelial-Mesenchymal Transition ; Fibrosis ; Lung ; Mice ; Transforming Growth Factor beta1/metabolism
Czasopismo naukowe
Tytuł :
Digital quantification of p16-positive foci in fibrotic interstitial lung disease is associated with a phenotype of idiopathic pulmonary fibrosis with reduced survival.
Autorzy :
Keow J; Department of Pathology and Laboratory Medicine, Western University, London, ON, Canada.
Cecchini MJ; Department of Pathology and Laboratory Medicine, Western University, London, ON, Canada.
Jayawardena N; Interstitial Lung Disease Research Laboratory, Lawson Research Institute, Western University, London, ON, Canada.
Zompatori M; MultiMedica Group, I.R.C.C.S. San Giuseppe Hospital, Milan, Italy.
Joseph MG; Department of Pathology and Laboratory Medicine, Western University, London, ON, Canada.
Mura M; Interstitial Lung Disease Research Laboratory, Lawson Research Institute, Western University, London, ON, Canada. .; Division of Respirology, Department of Medicine, Western University, London, ON, Canada. .
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Źródło :
Respiratory research [Respir Res] 2022 Jun 07; Vol. 23 (1), pp. 147. Date of Electronic Publication: 2022 Jun 07.
Typ publikacji :
Journal Article
MeSH Terms :
Idiopathic Pulmonary Fibrosis*/diagnosis
Idiopathic Pulmonary Fibrosis*/drug therapy
Idiopathic Pulmonary Fibrosis*/genetics
Lung Diseases, Interstitial*/diagnosis
Lung Diseases, Interstitial*/drug therapy
Lung Diseases, Interstitial*/genetics
Cyclin-Dependent Kinases/analysis ; Cyclin-Dependent Kinases/genetics ; Cyclin-Dependent Kinases/metabolism ; Fibrosis ; Humans ; Lung/metabolism ; Phenotype
Czasopismo naukowe
Tytuł :
COVID-19 Lockdown Impacts Among Patients with Cystic Fibrosis: An Italian Regional Reference Centre Experience.
Autorzy :
Boni A; Department of Maternal, Infantile and Urological Sciences, Sapienza University of Rome, 155, 00161 Rome, Italy.
Murciano M; Department of Maternal, Infantile and Urological Sciences, Sapienza University of Rome, 155, 00161 Rome, Italy.; Department of Emergency and General Pediatrics, Bambino Gesù Children's Hospital, 4, 00165 Rome, Italy.
De Luca F; Department of Maternal, Infantile and Urological Sciences, Sapienza University of Rome, 155, 00161 Rome, Italy.
Rizzo V; Department of Maternal, Infantile and Urological Sciences, Sapienza University of Rome, 155, 00161 Rome, Italy.
Cappelli C; Lazio Reference Center for Cystic Fibrosis, Policlinico Umberto I University Hospital, Sapienza University, Viale del Policlinico, 155, 00161 Rome, Italy.
D'Alù V; Lazio Reference Center for Cystic Fibrosis, Policlinico Umberto I University Hospital, Sapienza University, Viale del Policlinico, 155, 00161 Rome, Italy.
Troiani P; Lazio Reference Center for Cystic Fibrosis, Policlinico Umberto I University Hospital, Sapienza University, Viale del Policlinico, 155, 00161 Rome, Italy.
Nenna R; Department of Maternal, Infantile and Urological Sciences, Sapienza University of Rome, 155, 00161 Rome, Italy.
Cimino G; Lazio Reference Center for Cystic Fibrosis, Policlinico Umberto I University Hospital, Sapienza University, Viale del Policlinico, 155, 00161 Rome, Italy.
Midulla F; Department of Maternal, Infantile and Urological Sciences, Sapienza University of Rome, 155, 00161 Rome, Italy.
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Źródło :
Frontiers in bioscience (Landmark edition) [Front Biosci (Landmark Ed)] 2022 Jun 02; Vol. 27 (6), pp. 178.
Typ publikacji :
Journal Article
MeSH Terms :
COVID-19*/epidemiology
Cystic Fibrosis*/epidemiology
Cystic Fibrosis*/therapy
Communicable Disease Control ; Cystic Fibrosis Transmembrane Conductance Regulator ; Female ; Humans ; Male ; Retrospective Studies
Czasopismo naukowe
Tytuł :
Left behind: The potential impact of CFTR modulators on racial and ethnic disparities in cystic fibrosis.
Autorzy :
McGarry ME; Division of Pulmonary Medicine, Department of Pediatrics, University of California San Francisco, San Francisco, CA, United States.
Gibb ER; Division of Pulmonary Medicine, Department of Pediatrics, University of California San Francisco, San Francisco, CA, United States.
Oates GR; Division of Pulmonary and Sleep Medicine, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, United States.
Schechter MS; Division of Pulmonary and Sleep Medicine, Department of Pediatrics, Virginia Commonwealth University and Children's Hospital of Richmond at VCU, Richmond, VA, United States.
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Źródło :
Paediatric respiratory reviews [Paediatr Respir Rev] 2022 Jun; Vol. 42, pp. 35-42. Date of Electronic Publication: 2021 Dec 22.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Cystic Fibrosis*/drug therapy
Cystic Fibrosis*/genetics
Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Ethnicity ; Humans ; Minority Groups ; Mutation
Czasopismo naukowe
Tytuł :
Sustained inhibition of ENaC in CF: Potential RNA-based therapies for mutation-agnostic treatment.
Autorzy :
Kota P; Cystic Fibrosis Research and Treatment Center, University of North Carolina at Chapel Hill, NC 27599, USA. Electronic address: .
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Źródło :
Current opinion in pharmacology [Curr Opin Pharmacol] 2022 Jun; Vol. 64, pp. 102209. Date of Electronic Publication: 2022 Apr 25.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Cystic Fibrosis*/drug therapy
Cystic Fibrosis*/genetics
Amiloride/pharmacology ; Amiloride/therapeutic use ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Epithelial Sodium Channel Blockers/pharmacology ; Epithelial Sodium Channel Blockers/therapeutic use ; Epithelial Sodium Channels/genetics ; Epithelial Sodium Channels/metabolism ; Humans ; Mutation ; RNA
Czasopismo naukowe
Tytuł :
Things come in threes: A new complex allele and a novel deletion within the CFTR gene complicate an accurate diagnosis of cystic fibrosis.
Autorzy :
Persico I; Department of Medical Sciences, University of Trieste, Trieste, Italy.
Feresin A; Department of Medical Sciences, University of Trieste, Trieste, Italy.
Faleschini M; Institute for Maternal and Child Health - IRCCS Burlo Garofolo, Trieste, Italy.
Fontana G; Institute for Maternal and Child Health - IRCCS Burlo Garofolo, Trieste, Italy.
Sirchia F; Institute for Maternal and Child Health - IRCCS Burlo Garofolo, Trieste, Italy.
Faletra F; Institute for Maternal and Child Health - IRCCS Burlo Garofolo, Trieste, Italy.
La Bianca M; Institute for Maternal and Child Health - IRCCS Burlo Garofolo, Trieste, Italy.
Suergiu S; Institute for Maternal and Child Health - IRCCS Burlo Garofolo, Trieste, Italy.
Morgutti M; Institute for Maternal and Child Health - IRCCS Burlo Garofolo, Trieste, Italy.
Maschio M; Institute for Maternal and Child Health - IRCCS Burlo Garofolo, Trieste, Italy.
D'Adamo AP; Department of Medical Sciences, University of Trieste, Trieste, Italy.
Raraigh KS; McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Savoia A; Department of Medical Sciences, University of Trieste, Trieste, Italy.; Institute for Maternal and Child Health - IRCCS Burlo Garofolo, Trieste, Italy.
Bottega R; Institute for Maternal and Child Health - IRCCS Burlo Garofolo, Trieste, Italy.
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Źródło :
Molecular genetics & genomic medicine [Mol Genet Genomic Med] 2022 Jun; Vol. 10 (6), pp. e1926. Date of Electronic Publication: 2022 Mar 29.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Cystic Fibrosis*/diagnosis
Cystic Fibrosis*/genetics
Alleles ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Humans ; Mutation ; Phenotype
Czasopismo naukowe
Tytuł :
Protective Effects of Extracellular Matrix-Derived Hydrogels in Idiopathic Pulmonary Fibrosis.
Autorzy :
Evangelista-Leite D; Department of Surgery, School of Veterinary Medicine and Animal Science, University of São Paulo, São Paulo, Brazil.
Carreira ACO; Department of Surgery, School of Veterinary Medicine and Animal Science, University of São Paulo, São Paulo, Brazil.; NUCEL (Cell and Molecular Therapy Center), University of São Paulo, São Paulo, Brazil.
Gilpin SE; Wyss Institute for Biologically Inspired Engineering at Harvard University, Boston, Massachusetts, USA.
Miglino MA; Department of Surgery, School of Veterinary Medicine and Animal Science, University of São Paulo, São Paulo, Brazil.
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Źródło :
Tissue engineering. Part B, Reviews [Tissue Eng Part B Rev] 2022 Jun; Vol. 28 (3), pp. 517-530. Date of Electronic Publication: 2021 Jun 14.
Typ publikacji :
Journal Article; Review; Research Support, Non-U.S. Gov't
MeSH Terms :
Idiopathic Pulmonary Fibrosis*/drug therapy
Idiopathic Pulmonary Fibrosis*/pathology
Extracellular Matrix ; Fibrosis ; Humans ; Hydrogels/pharmacology ; Lung/pathology ; Lung/physiology
Czasopismo naukowe
Tytuł :
Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators.
Autorzy :
Konrad J; Division of Internal Medicine, Department of Gastroenterology and Hepatology, Medical University of Graz, Austria.
Eber E; Division of Paediatric Pulmonology and Allergology, Department of Paediatrics and Adolescent Medicine, Medical University of Graz, Austria.
Stadlbauer V; Division of Internal Medicine, Department of Gastroenterology and Hepatology, Medical University of Graz, Austria. Electronic address: .
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Źródło :
Paediatric respiratory reviews [Paediatr Respir Rev] 2022 Jun; Vol. 42, pp. 9-16. Date of Electronic Publication: 2020 Dec 24.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Cystic Fibrosis*/therapy
Exocrine Pancreatic Insufficiency*/drug therapy
Exocrine Pancreatic Insufficiency*/etiology
Gastrointestinal Diseases*/complications
Gastrointestinal Diseases*/therapy
Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Humans ; Mutation ; Quality of Life
Czasopismo naukowe
Tytuł :
Revisiting a diagnosis of cystic fibrosis - Uncertainties and considerations.
Autorzy :
Balfour-Lynn IM; Depts. Paediatric Cystic Fibrosis, Royal Brompton Hospital, London, UK. Electronic address: .
Puckey M; Depts. Paediatric Clinical Psychology, Royal Brompton Hospital, London, UK.
Simmonds NJ; Adult Cystic Fibrosis Centre, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College London, London, UK.
Davies JC; Depts. Paediatric Cystic Fibrosis, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College London, London, UK.
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Źródło :
Paediatric respiratory reviews [Paediatr Respir Rev] 2022 Jun; Vol. 42, pp. 29-34. Date of Electronic Publication: 2021 Dec 08.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Cystic Fibrosis*/diagnosis
Cystic Fibrosis*/genetics
Adult ; Child ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Genetic Testing ; Humans ; Infant, Newborn ; Neonatal Screening/methods
Czasopismo naukowe
Tytuł :
Gene-specific nonsense-mediated mRNA decay targeting for cystic fibrosis therapy.
Autorzy :
Kim YJ; Cold Spring Harbor Laboratory, Cold Spring Harbor, NY, 11724, USA.; Graduate Program in Genetics, Stony Brook University, Stony Brook, NY, 11794, USA.; Medical Scientist Training Program, Stony Brook University School of Medicine, Stony Brook, NY, 11794, USA.
Nomakuchi T; Cold Spring Harbor Laboratory, Cold Spring Harbor, NY, 11724, USA.; Medical Scientist Training Program, Stony Brook University School of Medicine, Stony Brook, NY, 11794, USA.; Division of Human Genetics, Children's Hospital of Philadelphia, Philadelphia, PA, 19104, USA.
Papaleonidopoulou F; Cold Spring Harbor Laboratory, Cold Spring Harbor, NY, 11724, USA.; Francis Crick Institute, London, 1140062, UK.
Yang L; Cold Spring Harbor Laboratory, Cold Spring Harbor, NY, 11724, USA.; Graduate Program in Genetics, Stony Brook University, Stony Brook, NY, 11794, USA.; Medical Scientist Training Program, Stony Brook University School of Medicine, Stony Brook, NY, 11794, USA.
Zhang Q; Cold Spring Harbor Laboratory, Cold Spring Harbor, NY, 11724, USA.; Graduate Program in Molecular and Cell Biology, Stony Brook University, Stony Brook, NY, 11794, USA.
Krainer AR; Cold Spring Harbor Laboratory, Cold Spring Harbor, NY, 11724, USA. .
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Źródło :
Nature communications [Nat Commun] 2022 May 27; Vol. 13 (1), pp. 2978. Date of Electronic Publication: 2022 May 27.
Typ publikacji :
Journal Article
MeSH Terms :
Cystic Fibrosis*/drug therapy
Cystic Fibrosis*/genetics
Nonsense Mediated mRNA Decay*
Codon, Nonsense/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Humans ; Oligonucleotides, Antisense/genetics ; Oligonucleotides, Antisense/metabolism ; Oligonucleotides, Antisense/pharmacology ; RNA, Messenger/metabolism
Czasopismo naukowe
Tytuł :
Sine oculis homeobox homolog 1 plays a critical role in pulmonary fibrosis.
Autorzy :
Wilson C; Department of Biochemistry and Molecular Biology, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth Houston), Houston, Texas, USA.
Mertens TC; Department of Biochemistry and Molecular Biology, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth Houston), Houston, Texas, USA.
Shivshankar P; Department of Biochemistry and Molecular Biology, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth Houston), Houston, Texas, USA.
Bi W; Department of Biochemistry and Molecular Biology, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth Houston), Houston, Texas, USA.
Collum SD; Department of Biochemistry and Molecular Biology, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth Houston), Houston, Texas, USA.
Wareing N; Department of Biochemistry and Molecular Biology, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth Houston), Houston, Texas, USA.
Ko J; Department of Biochemistry and Molecular Biology, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth Houston), Houston, Texas, USA.
Weng T; Department of Biochemistry and Molecular Biology, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth Houston), Houston, Texas, USA.
Naikawadi RP; Pulmonary, Critical Care, Allergy and Sleep Medicine, UCSF, San Francisco, California, USA.
Wolters PJ; Pulmonary, Critical Care, Allergy and Sleep Medicine, UCSF, San Francisco, California, USA.
Maire P; Université de Paris Cité, Institut Cochin, INSERM, CNRS, Paris, France.
Jyothula SS; Divisions of Critical Care, Pulmonary and Sleep Medicine, Department of Internal Medicine, McGovern Medical School, UTHealth, Houston, Texas, USA.
Thandavarayan RA; Methodist J.C. Walter Jr. Transplant Center, Houston Methodist Hospital, Houston, Texas, USA.
Ren D; Methodist J.C. Walter Jr. Transplant Center, Houston Methodist Hospital, Houston, Texas, USA.
Elrod ND; Department of Biochemistry and Molecular Biology, University of Texas Medical Branch at Galveston, Galveston, Texas, USA.
Wagner EJ; Department of Biochemistry and Biophysics, Center for RNA Biology, Wilmot Cancer Institute, University of Rochester School of Medicine and Dentistry, KMRB G.9629, Rochester, New York, USA.
Huang HJ; Methodist J.C. Walter Jr. Transplant Center, Houston Methodist Hospital, Houston, Texas, USA.
Dickey BF; Department of Pulmonary Medicine, Division of Internal Medicine, The University of Texas M.D. Anderson Cancer Center, Houston, Texas, USA.
Ford HL; Department of Pharmacology, University of Colorado, Anschutz Medical Campus, Aurora, Colorado, USA.
Karmouty-Quintana H; Department of Biochemistry and Molecular Biology, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth Houston), Houston, Texas, USA.; Divisions of Critical Care, Pulmonary and Sleep Medicine, Department of Internal Medicine, McGovern Medical School, UTHealth, Houston, Texas, USA.
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Źródło :
JCI insight [JCI Insight] 2022 May 23; Vol. 7 (10). Date of Electronic Publication: 2022 May 23.
Typ publikacji :
Journal Article
MeSH Terms :
Homeodomain Proteins*/genetics
Homeodomain Proteins*/metabolism
Idiopathic Pulmonary Fibrosis*/chemically induced
Idiopathic Pulmonary Fibrosis*/genetics
Animals ; Fibrosis ; Genes, Homeobox ; Mice ; Transcription Factors/genetics
Czasopismo naukowe
Tytuł :
Role of IL-17 family cytokines in the progression of IPF from inflammation to fibrosis.
Autorzy :
Nie YJ; Department of Basic Medicine, Wuxi School of Medicine, Jiangnan University, Wuxi, 214000, Jiangsu, China.
Wu SH; Department of Radiology, The Second Affiliated Hospital, Medical College of Shantou University, Shantou, 515000, Shandong, China.
Xuan YH; Department of Basic Medicine, Xiamen Medical College, Xiamen, 361000, Fujian, China.
Yan G; Department of Radiology, The Second Affiliated Hospital of Xiamen Medical College, Xiamen, 361000, Fujian, China. .
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Źródło :
Military Medical Research [Mil Med Res] 2022 May 12; Vol. 9 (1), pp. 21. Date of Electronic Publication: 2022 May 12.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Idiopathic Pulmonary Fibrosis*/metabolism
Idiopathic Pulmonary Fibrosis*/pathology
Cytokines ; Fibrosis ; Humans ; Inflammation ; Interleukin-17
Czasopismo naukowe
Tytuł :
Evaluation of Proteasome Inhibitors in the Treatment of Idiopathic Pulmonary Fibrosis.
Autorzy :
Chen IC; Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, Taiwan.; Department of Pediatrics, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 80708, Taiwan.; Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 80708, Taiwan.
Liu YC; Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, Taiwan.
Wu YH; Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, Taiwan.
Lo SH; Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, Taiwan.
Dai ZK; Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, Taiwan.; Department of Pediatrics, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 80708, Taiwan.; Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 80708, Taiwan.
Hsu JH; Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, Taiwan.
Tseng YH; Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, Taiwan.
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Źródło :
Cells [Cells] 2022 May 04; Vol. 11 (9). Date of Electronic Publication: 2022 May 04.
Typ publikacji :
Journal Article; Review; Research Support, Non-U.S. Gov't
MeSH Terms :
Carcinoma, Non-Small-Cell Lung*
Idiopathic Pulmonary Fibrosis*/drug therapy
Idiopathic Pulmonary Fibrosis*/metabolism
Lung Neoplasms*
Fibrosis ; Humans ; Proteasome Inhibitors/pharmacology ; Proteasome Inhibitors/therapeutic use
Czasopismo naukowe

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