Results 31 to 40 of about 1,274,104 (305)

Upregulation of a CFTR mRNA isoform has therapeutic potential for the treatment of 3′ CFTR PTC variants

Molecular Therapy: Nucleic Acids
Nonsense or premature termination codon (PTC) variants of the CFTR gene are pathogenic and found in ∼10% of North American people with cystic fibrosis.
Normand E. Allaire, Matthew S. Armstrong, Jae Seok Yoon, Mercy C. Walker, Priyanka Bhatt, Jan M. Harrington, Yi Cheng, Eric I. Wong, John E. Mahoney, Hillary C. Valley, Caitlin M. Macadino, Hermann J. Bihler, Andrey Y. Sivachenko, Calvin U. Cotton, John P. Carulli, Martin Mense   +15 more
doaj   +1 more source

Elevated sweat chloride test: is it always cystic fibrosis?

Italian Journal of Pediatrics, 2021
Background The sweat chloride test (ST) is the gold standard for cystic fibrosis (CF) diagnosis in symptomatic patients, within the newborn screening and in the follow-up of CF patients during molecular therapies.
C. Cimbalo, A. Tosco, V. Terlizzi, A. Sepe, A. Castaldo, L. Salvadori, V. Raia   +6 more
doaj   +1 more source

Identification of Novel Fibrosis Modifiers by In Vivo siRNA Silencing. [PDF]

, 2017
Fibrotic diseases contribute to 45% of deaths in the industrialized world, and therefore a better understanding of the pathophysiological mechanisms underlying tissue fibrosis is sorely needed.
Amatucci, Aldo, Anderson, Daniel G, Brenner, David, Burkly, Linda C, Cameron, Thomas O, Cao, Lizhi, Dalkilic-Liddle, Isin, Duffield, Jeremy, Hamann, Stefan, Hossbach, Markus, Kauffman, Kevin J, Kisseleva, Tatiana, Koteliansky, Victor, Mir, Faryal F, Novobrantseva, Tatiana, Reynolds, Taylor, Vollmann, Elisabeth H   +16 more
core   +2 more sources

Pleural Fibrosis

Clinics in Chest Medicine, 2006
Pleural fibrosis can result from a variety of inflammatory processes. The response of the pleural mesothelial cell to injury and the ability to maintain its integrity are crucial in determining whether normal healing or pleural fibrosis occurs.
Division of Pulmonary and Critical Care Medicine, University of Florida SW Archer Road, Room M352, PO Box 100225, Gainesville, FL 32610–0225, USA ( host institution ), Jantz, Michael A. ( author ), Antony, Veena B. ( author )   +2 more
openaire   +3 more sources

REAL‐world clinical effectiveness of ivacaftor therapy in the first 24 months in two infants with cystic fibrosis and different gating mutations—A case report

Clinical Case Reports, 2022
This study summarizes efficacy of ivacaftor treatment in 2 infants in a real‐world setting. A distinct decline of sweat chloride and lung clearance index plus increase in fecal elastase was seen. The results underline the early and sustainable effect and
Teresa Fuchs, Dorothea Appelt, Katharina Niedermayr, Helmut Ellemunter   +3 more
doaj   +1 more source

The evaluation of liver fibrosis regression in chronic hepatitis C patients after the treatment with direct-acting antiviral agents – A review of the literature [PDF]

, 2019
The second-generation of direct-acting antiviral agents are the current treatment for chronic viral hepatitis C infection. To evaluate the regression of liver fibrosis in patients receiving this therapy, liver biopsy remains the most accurate method, but
Alexescu, Teodora G, Cibu, Bianca A, Ciulei, George, Coste, Sorina C, Cozma, Angela, Mureșan, Flaviu, Negrean, Vasile, Orasan, Olga H, Ovidiu, Fabian, Pfingstgraf, Iulia O, Popovici, Ionela E, Tarmure, Simina F, Taut, Adela V. S   +12 more
core   +3 more sources

PTP4A1 promotes TGFβ signaling and fibrosis in systemic sclerosis. [PDF]

, 2017
Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of skin and internal organs. Protein tyrosine phosphatases have received little attention in the study of SSc or fibrosis.
Bai, Yunpeng, Boin, Francesco, Bottini, Nunzio, Ceponis, Arnold, Chernitskiy, Vladimir, Cipriani, Paola, Di Benedetto, Paola, Giacomelli, Roberto, Jimenez, Sergio A, Kaestner, Klaus H, Kiosses, William B, Piera-Velazquez, Sonsoles, Sacchetti, Cristiano, Santelli, Eugenio, Stanford, Stephanie M, Zhang, Zhong-Yin   +15 more
core   +2 more sources

A Prospective Study of Long-term Outcomes in Female Patients with Nonalcoholic Steatohepatitis Using Age- and Body Mass Index-matched Cohorts [PDF]

, 2013
In patients with nonalcoholic steatohepatitis (NASH), the prevalence of cirrhosis is higher among women than men, and hepatocellular carcinoma (HCC) develops mainly in the cirrhotic stage among women.
Hashizume, Hiroaki, Horiguchi, Norio, Kakizaki, Satoru, Mori, Masatomo, Sato, Ken, Yamazaki, Yuichi   +5 more
core   +1 more source

Can early Burkholderia cepacia complex infection in cystic fibrosis be eradicated with antibiotic therapy?

Frontiers in Cellular and Infection Microbiology, 2011
IntroductionOrganisms of the Burkholderia cepacia complex (BCC) are important pathogens in cystic fibrosis (CF). The majority of those who acquire BCC develop chronic infection but it can also result in rapid decline in a significant minority.
Alex eHorsley, Alex eHorsley, Kevin eWebb, Kevin eWebb, Rowland eBright-Thomas, John eGovan, Andrew M Jones, Andrew M Jones   +7 more
doaj   +1 more source

Diagnostic value of two dimensional shear wave elastography combined with texture analysis in early liver fibrosis. [PDF]

, 2019
BACKGROUND: Staging diagnosis of liver fibrosis is a prerequisite for timely diagnosis and therapy in patients with chronic hepatitis B. In recent years, ultrasound elastography has become an important method for clinical noninvasive assessment of liver ...
Hu, Xiang-Dong, Jian, Zhao-Cheng, Li, Wei-Sheng, Liu, Ji-Bin, Liu, Yu-Jiang, Long, Jin-Feng, Qian, Lin-Xue, Shi, Xian-Quan   +7 more
core   +1 more source