Cystic Fibrosis–Related Liver Diseases: New Paradigm for Treatment Based on PathophysiologyEzrin was first detected in epidermal growth factor-treated skin epithelial cells as a protein-tyrosine kinase substrate prior to its purification and characterization as a structural component of microvilli of ...
mucus caused by CF blocks ducts in your pancreas. This stops proteins that break down your food, called digestive enzymes, from reaching your intestine. As a result, your body has a hard time getting the nutrients it needs. Over time, this can also lead to cystic fibrosis-related diabetes....
Abnormal intestinal motility may also contribute to meconium ileus development. Some patients with cystic fibrosis have prolonged small intestinal transit times. Diseases other than cystic fibrosis in which there is abnormal gut motility (eg, Hirschsprung disease, chronic intestinal pseudo-obstruction) have...
This is a case study depicting the complications of managing cystic fibrosis and cystic fibrosis-related diabetes with the challenges of biological family dysfunction. It illustrates the variability of severity within the diagnosis of cystic fibrosis, as well as the fact that, despite multiple challenge...
Numerous epidemiological studies demonstrate that cystic fibrosis, the most common orphan disease in the world, can not occur in isolation, but can be combined with other serious diseases. The most common such combination in all populations is traditionally cystic fibrosis and celiac disease, which, ...
Cystic fibrosis (CF) was one of the first target diseases for lung gene therapy. Studies of lung gene transfer for CF have provided many insights into the ... V Sueblinvong,BT Suratt,DJ Weiss - 《Clinics in Chest Medicine》 被引量: 102发表: 2007年 The Role of Defensins in Lung Biolog...
Inflammation plays a primary role in the pathogenesis of cystic fibrosis (CF)-related lung disease. Controlling the inflammatory process with antiinflammatory therapy may slow the progression of pulmonary disease and thereby decrease morbidity. Despite potential benefits of antiinflammatory therapy, both th...
In conclusion, this study shows that ECO and ICO have distinct lineage fate and that ECO provide a competent model to study extrahepatic bile duct diseases like cystic fibrosis.Similar content being viewed by others Generation of functional ciliated cholangiocytes from human pluripotent stem cells ...
Simultaneous occurrence of Cystic fibrosis and other inherited diseases or congenital anomalies has been rare. This association has never been described before in the Arab population. In this report we describe the first report on cystic fibrosis in association with other diseases in the same patient...
Jonathan Tait for his contributions to earlier versions of the CFTR-related disorders section of GeneReviews and Dr. Margaret Rosenfeld for her insightful comments on the manuscript. References 1. Rosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis ...