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Anti-fibrotic effect of intravenous umbilical cord-derived mesenchymal stem cells (UC-MSCs) injection in experimental rats induced liver fibrosis.

Taufik Sungkar, Agung Putra, Dharma Lindarto, Rosita Juwita Sembiring,

Aim To investigate the effect of umbilical cord-derived mesenchymal stem cells (UC-MSCs) administration among liver fibrosis experimental rat model via the regulation of angiotensin II type 1 receptor (AT1R) and platelet-derived growth factor-β (PDGF-β) due to their therapeutic potential to replace liver transplantation for advanced liver fibrosis. Yet the mechanism ... Read more >>

Med Glas (Zenica) (Medicinski glasnik : official publication of the Medical Association of Zenica-Doboj Canton, Bosnia and Herzegovina)
[2021, 18(1):]

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Concordance of non-invasive serology-based scoring indices and transient elastography for liver fibrosis and cirrhosis in chronic hepatitis C.

Emir Trnačević, Nermin Salkić, Alma Trnačević, Anja Divković, Fatima Hukić, Nusret Butković, Amra Serak, Amer Mujkanović,

Aim To assess concordance of eight frequently used serology-based scoring indices for liver fibrosis and cirrhosis with transient elastography (TE) in chronic hepatitis C (CHC) patients in order to determine serum indices with the highest concordance and clinical usability in clinical practice. Methods In this prospective study, 63 CHC patients ... Read more >>

Med Glas (Zenica) (Medicinski glasnik : official publication of the Medical Association of Zenica-Doboj Canton, Bosnia and Herzegovina)
[2021, 18(1):]

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Effect of miR-21 on Renal Fibrosis Induced by Nano-SiO₂ in Diabetic Nephropathy Rats via PTEN/AKT Pathway.

Jifan Guo, Kwangwoo Nam, George Collins,

MicroRNAs are a type of non-coding single-stranded RNA that can mediate target mRNA degradation or inhibit target mRNA translation, thereby regulating target gene expression and have an important role in physiological and pathological processes. At present, miRs have been confirmed to be closely related to kidneys and kidney diseases, and ... Read more >>

J Nanosci Nanotechnol (Journal of nanoscience and nanotechnology)
[2021, 21(2):1079-1084]

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Pulmonary Manifestations of Genetic Disorders in Children.

Beth A Pletcher, Nelson L Turcios,

Congenital bronchopulmonary malformations are relatively common and arise during various periods of morphogenesis. Although some are isolated or sporadic occurrences, others may result from single gene mutations or cytogenetic imbalances. Single gene mutations have been identified, which are etiologically related to primary pulmonary hypoplasia, lung segmentation defects as well as ... Read more >>

Pediatr Clin North Am (Pediatric clinics of North America)
[2021, 68(1):1-24]

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Performance and cutoffs for liver fibrosis staging of a two-dimensional shear wave elastography technique.

Giovanna Ferraioli, Laura Maiocchi, Carolina Dellafiore, Carmine Tinelli, Elisabetta Above, Carlo Filice,

<b>Objectives:</b>To assess performance and cutoffs of the 2-dimensional shear wave elastography technique available on the Aplio i800 ultrasound system (Canon Medical Systems, Japan), using transient elastography as reference standard, and to assess the correlation of shear-wave-speed dispersion with liver fibrosis or steatosis.<br><br><b>Methods:</b>This was a single-center cross-sectional study. The correlations between ... Read more >>

Eur J Gastroenterol Hepatol (European journal of gastroenterology & hepatology)
[2021, 33(1):89-95]

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Current treatment paradigms and emerging therapies for NAFLD/NASH.

Sana Raza, Sangam Rajak, Aditya Upadhyay, Archana Tewari, Rohit Anthony Sinha,

Non-alcoholic fatty liver disease (NAFLD) is one the fastest emerging manifestations of the metabolic syndrome worldwide. Non-alcoholic steatohepatitis (NASH), the progressive form of NAFLD, may culminate into cirrhosis and hepatocellular cancer (HCC) and is presently a leading cause of liver transplant. Although a steady progress is seen in understanding of ... Read more >>

Front Biosci (Landmark Ed) (Frontiers in bioscience (Landmark edition))
[2021, 26:206-237]

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Preparation of Gold Nanoparticles and Its Effect on Autophagy and Oxidative Stress in Chronic Kidney Disease Cell Model.

Ping Zhao, Ting Li, Zhi Li, Lei Cao, Youliang Wang, Yong Wang, Weikai Wang, Togo Shinonaga, Maneesha A Rajora,

Gold nanoparticles (GNPs) are widely used in life sciences and medicine due to their simple preparation, stable physical and chemical properties, controllable optical properties and no significant toxicity. However, in recent years, studies have found that there are still many uncertain factors in the application of gold nanoparticles in the ... Read more >>

J Nanosci Nanotechnol (Journal of nanoscience and nanotechnology)
[2021, 21(2):1266-1271]

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[Evidence-based treatment of cystic fibrosis].

F C Ringshausen, T Hellmuth, A-M Dittrich,

Mucoviscidosis (cystic fibrosis [CF]) is the most common autosomal recessive inherited multisystem disease with fatal outcome. It is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which lead to a dysfunctional chloride channel and a defective CFTR protein. As a consequence, retention of insufficiently hydrated mucus affects multiple ... Read more >>

Internist (Berl) (Der Internist)
[2020, 61(12):1212-1229]

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Engeletin ameliorates pulmonary fibrosis through endoplasmic reticulum stress depending on lnc949-mediated TGF-β1-Smad2/3 and JNK signalling pathways.

Jinjin Zhang, Xiaoqing Chen, Hongbin Chen, Rongrong Li, Pan Xu, Changjun Lv, Bo Liu, Xiaodong Song,

CONTEXT:Pulmonary fibrosis (PF) is a highly heterogeneous and lethal pathological process having no effective drug. Engeletin exerts multiple biological activities including anti-inflammatory and lung repair. Whether engeletin has therapeutic effects on PF remains unclear. OBJECTIVE:Examining effect and mechanism of engeletin on PF in vivo and in vitro. MATERIALS AND METHODS:L929 cells (1 × 106/well) ... Read more >>

Pharm Biol (Pharmaceutical biology)
[2020, 58(1):1105-1114]

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Bibliometric analysis of chloride channel research (2004-2019).

Jingjing Shi, Shuqing Shi, Guozhen Yuan, QiuLei Jia, Shuai Shi, Xueping Zhu, Yan Zhou, Ting Chen, Yuanhui Hu,

Channels (Austin) (Channels (Austin, Tex.))
[2020, 14(1):393-402]

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Epidemiology of Burkholderia Infections in People with Cystic Fibrosis in Canada between 2000 and 2017.

James E A Zlosnik, Deborah A Henry, Trevor J Hird, Rebecca Hickman, Maureen Campbell, Adriana Cabrera, Giulio Laino Chiavegatti, Mark A Chilvers, Manish Sadarangani,

<b>Rationale:</b> Infections by <i>Burkholderia</i> species bacteria in cystic fibrosis (CF) may be transmissible, necessitating infection control measures, and remain a serious cause of morbidity and mortality. The last major study of <i>Burkholderia</i> epidemiology in Canada included cases up until July 2000 and was marked by the dominance of a limited ... Read more >>

Ann Am Thorac Soc (Annals of the American Thoracic Society)
[2020, 17(12):1549-1557]

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Clinical Outcomes Associated with Burkholderia cepacia Complex Infection in Patients with Cystic Fibrosis.

Ranjani Somayaji, Yvonne C W Yau, Elizabeth Tullis, John J LiPuma, Felix Ratjen, Valerie Waters,

<b>Rationale:</b> Little is known in contemporary cystic fibrosis (CF) cohorts about the outcomes after new <i>Burkholderia</i> species infections.<b>Objectives:</b> To evaluate the changing epidemiology and clinical outcomes associated with <i>Burkholderia</i> species infections in persons with CF.<b>Methods:</b> A cohort study of children and adults with CF was conducted from 1997 to 2018 ... Read more >>

Ann Am Thorac Soc (Annals of the American Thoracic Society)
[2020, 17(12):1542-1548]

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Development of elexacaftor - tezacaftor - ivacaftor: highly effective CFTR modulation for the majority of people with cystic fibrosis.

Peter G Middleton, Jennifer L Taylor-Cousar,

INTRODUCTION:Cystic Fibrosis (CF), the most common life-shortening inherited disorder in people of European descent, also occurs in other ethnicities. The identification of the disease, the isolation of the causative gene, termed the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) and the improved survival from comprehensive multidisciplinary treatment is one of the ... Read more >>

Expert Rev Respir Med (Expert review of respiratory medicine)
[2020, :]

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Subtle relationships between Pseudomonas aeruginosa and fungi in patients with cystic fibrosis.

Kaicheng Yan, Hong Yin, Jin Wang, Yun Cai,

Cystic fibrosis (CF) is one of the most common hereditary lung diseases. Pseudomonas aeruginosa (PA), Aspergillus fumigatus (AF) and Candida albicans (CA) are the principal bacterial and fungal pathogens in the airways of CF patients. The interactions of coexisting bacterial-fungal pathogens are of great interest. In the present work, we ... Read more >>

Acta Clin Belg (Acta clinica Belgica)
[2020, :1-11]

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PAD4 Deficiency Improves Bleomycin-induced Neutrophil Extracellular Traps and Fibrosis in Mouse Lung.

Masaki Suzuki, Jun Ikari, Rie Anazawa, Nozomi Tanaka, Yusuke Katsumata, Ayako Shimada, Eiko Suzuki, Koichiro Tatsumi,

Excessive release of neutrophil extracellular traps (NETs) has been implicated in several organ fibrosis, including pulmonary fibrosis. NETs constitute a phenomenon in which decorated nuclear chromatin with cytosolic proteins is released into the extracellular space. PAD4 (peptidylarginine deiminase 4) plays an important role in the formation of NETs. However, the ... Read more >>

Am J Respir Cell Mol Biol (American journal of respiratory cell and molecular biology)
[2020, 63(6):806-818]

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Reduced Intestinal Inflammation With Lumacaftor/Ivacaftor in Adolescents With Cystic Fibrosis.

Candice Tétard, Marie Mittaine, Stéphanie Bui, Fabien Beaufils, Pascale Maumus, Michael Fayon, Pierre-Regis Burgel, Thierry Lamireau, Laurence Delhaes, Emmanuel Mas, Raphaël Enaud,

A chronic intestinal inflammation may occur in patients with cystic fibrosis (CF), while no therapeutic management is proposed. Although Lumacaftor/Ivacaftor is well-known to modulate the defective cystic fibrosis transmembrane conductance regulator (CFTR) protein in lungs, no data are available on the impact of this treatment on CF intestinal disorders. We, ... Read more >>

J Pediatr Gastroenterol Nutr (Journal of pediatric gastroenterology and nutrition)
[2020, 71(6):778-781]

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How to inhibit transforming growth factor beta safely in diabetic kidney disease.

Yuxin Yang, Kexin Shi, Devang M Patel, Fang Liu, Tieqiao Wu, Zhonglin Chai,

PURPOSE OF REVIEW:Diabetic kidney disease (DKD) is a leading cause of mortality and morbidity in diabetes. This review aims to discuss the major features of DKD, to identify the difficult barrier encountered in developing a therapeutic strategy and to provide a potentially superior novel approach to retard DKD. RECENT FINDINGS:Renal ... Read more >>

Curr Opin Nephrol Hypertens (Current opinion in nephrology and hypertension)
[2021, 30(1):115-122]

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Assisting PNA Transport Through Cystic Fibrosis Human Airway Epithelia With Biodegradable Hybrid Lipid-Polymer Nanoparticles

Marika Comegna, Gemma Conte, Andrea Falanga, Maria Marzano, Gustavo Cernera, Antonella Di Lullo, Felice Amato, Nicola Borbone, Stefano D'Errico, Francesca Ungaro, Ivana d'Angelo, Giorgia Oliviero, Giuseppe Castaldo,

Abstract Cystic Fibrosis (CF) is characterized by an airway obstruction caused by a thick mucus due to a malfunctioning Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein. The sticky mucus restricts drugs in reaching target cells limiting the efficiency of treatments. The development of new approaches to enhance drug delivery to ... Read more >>

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Pregnancy in Cystic Fibrosis: Review of the Literature and Expert Recommendations

Raksha Jain, Traci Kazmerski, Lisa Zuckerwise, Natalie West, Kristina Montemayor, Moira Aitken, Edith Cheng, Andrea Roe, Alexandra Wilson, Caitlin Mann, Sigrid Ladores, Jacqui Sjoberg, Jennifer Taylor-Cousar,

Cystic fibrosis (CF) was historically a disease largely afflicting children. Due to therapeutic advancement, there are now more adults with CF than children. In the past decade, medications became available that treat the underlying cause of CF and are dramatically improving lung function as well as quality and quantity of ... Read more >>

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Novel Antioxidant Therapy with the Immediate Precursor to Glutathione, γ-Glutamylcysteine (GGC), Ameliorates LPS-Induced Cellular Stress in In Vitro 3D-Differentiated Airway Model from Primary Cystic Fibrosis Human Bronchial Cells.

Chris K Hewson, Alexander Capraro, Sharon L Wong, Elvis Pandzic, Ling Zhong, Bentotage S M Fernando, Nikhil T Awatade, Gene Hart-Smith, Renee M Whan, Shane R Thomas, Adam Jaffe, Wallace J Bridge, Shafagh A Waters,

Systemic glutathione deficiency, inflammation, and oxidative stress are hallmarks of cystic fibrosis (CF), an inherited disease that causes persistent lung infections and severe damage to the respiratory system and many of the body organs. Improvements to current antioxidant therapeutic strategies are needed. The dietary supplement, γ-glutamylcysteine (GGC), which is the ... Read more >>

Antioxidants (Basel) (Antioxidants (Basel, Switzerland))
[2020, 9(12):]

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Rehmannia Radix Extract Relieves Bleomycin-Induced Pulmonary Fibrosis in Mice via Transforming Growth Factor β1 (TGF-β1).

Xiaoming Hu, Dongzhe Zhu,

BACKGROUND Infants and young children with acute respiratory distress syndrome (ARDS) have acute progressive hypoxic respiratory failure caused by a variety of extrapulmonary pathogenic factors and cardiogenic factors. Diffuse alveolar injury and pulmonary fibrosis both are pathological features of ARDS. This study investigated the effect of Rehmannia Radix extract (RRE) ... Read more >>

Med Sci Monit (Medical science monitor : international medical journal of experimental and clinical research)
[2020, 26:e927240]

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FK506-binding Protein 13 Expression is Upregulated in Interstitial Lung Disease and Correlated with Clinical Severity: A Potentially Protective Role.

Victor Tat, Ehab A Ayaub, Anmar Ayoub, Megan Vierhout, Safaa Naiel, Manreet K Padwal, Soumeya Abed, Olivia Mekhael, Karun Tandon, Spencer D Revill, Tamana Yousof, Pierre-Simon Bellaye, Philipp S Kolb, Anna Dvorkin-Gheva, Asghar Naqvi, Jean-Claude Cutz, Nathan Hambly, Jiro Kato, Martha Vaughan, Joel Moss, Martin Rj Kolb, Kjetil Ask,

Pulmonary fibrosis is a progressive lung disease characterized by myofibroblast accumulation and excessive extracellular matrix deposition. We sought to investigate the role of the 13 kDa FK506-binding protein (FKBP13), an endoplasmic reticulum-resident molecular chaperone, in various forms of pulmonary fibrosis. We first characterized the gene and protein expression of FKBP13 ... Read more >>

Am J Respir Cell Mol Biol (American journal of respiratory cell and molecular biology)
[2020, :]

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Variations in Nutrition Practices in Cystic Fibrosis: A Survey of the DIGEST Program.

Ala K Shaikhkhalil, Alvin J Freeman, Meghana Sathe,

BACKGROUND:Evidence-based management of gastrointestinal (GI) and nutrition manifestations of cystic fibrosis (CF) is limited, and practice variations have not been studied. METHODS:Thus, a survey was developed with the purpose of evaluating current nutrition practices of CF-focused gastroenterologists, specifically utilizing awardees and mentors of the Cystic Fibrosis Foundation (CFF) Developing Innovative ... Read more >>

Nutr Clin Pract (Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition)
[2020, :]

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Elastography and serum markers of fibrosis versus liver biopsy in 1270 Egyptian patients with hepatitis C.

Eman Abdelsameea, Ayman Alsebaey, Wael Abdel-Razek, Nermin Ehsan, Wesam Morad, Mohsen Salama, Imam Waked,

BACKGROUND:Chronic hepatitis C (CHC) is a leading cause of liver fibrosis. OBJECTIVE:To compare utility of liver transient elastography, AST-to-platelet ratio index (APRI), fibrosis-4 index (FIB4), Forns Index and Goteborg University cirrhosis index (GUCI) in predicting fibrosis stage assessed by liver biopsy in Egyptian CHC patients. METHODS:One thousand two-hundred and seventy ... Read more >>

Eur J Gastroenterol Hepatol (European journal of gastroenterology & hepatology)
[2020, 32(12):1553-1558]

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Delayed age at menarche in chronic respiratory diseases.

Rossella Gaudino, Sarah Dal Ben, Paolo Cavarzere, Sonia Volpi, Claudia Piona, Attilio Boner, Franco Antoniazzi, Giorgio Piacentini,

OBJECTIVE:Age at menarche (AAM) is an important indicator of physiological development in women and delayed AAM has been associated with chronic illnesses. We investigated predictive factors at diagnosis that influence AAM in adolescents with chronic respiratory diseases. STUDY DESIGN:AAM was assessed in 1207 northern Italian female aged 11-24; (1062 healthy, ... Read more >>

Eur J Clin Invest (European journal of clinical investigation)
[2020, :e13461]

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