Cystic fibrosis since 1938.
@article{Davis2006CysticFS, title={Cystic fibrosis since 1938.}, author={Pamela B. Davis}, journal={American journal of respiratory and critical care medicine}, year={2006}, volume={173 5}, pages={ 475-82 } }
Cystic fibrosis (CF) was distinguished from celiac disease in 1938. Then, it was a pathologic diagnosis, life expectancy was approximately 6 months, and the autosomal recessive disease was believed to arise from abnormal mucus plugging exocrine ducts. Death often occurred from lung infection. Discovery of the sweat electrolyte defect in 1953 and standardization of the sweat test in 1959 allowed identification of milder cases, and CF was no longer considered only a disorder of mucus. In 1955…
590 Citations
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- MedicineAmerican journal of respiratory and critical care medicine
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It is now appreciated that CF airway disease involves intricate interrelationships among airway surface liquid, mucus clearance, infection, inflammation, repair, and fibrosis.
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The optimal perioperative management of patients with CF requires an understanding of the relevant pathophysiology and the unique challenges presented by these patients, including special considerations such as liver and lung transplantation and pregnancy.
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It is now appreciated that CF airway disease involves intricate interrelationships among airway surface liquid, mucus clearance, infection, inflammation, repair, and fibrosis.
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The results shows that newborn screening for CF with an IRT/DNA approach is possible in Sweden and that a majority of parents in Sweden support the inclusion of CF in the newborn screening programme.
Studies as a basis for a possible introduction of newborn screening for cystic fibrosis in Sweden
- Medicine
- 2011
The results shows that newborn screening for CF with an IRT/DNA approach is possible in Sweden and that a majority of parents in Sweden support the inclusion of CF in the newborn screening programme.
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It appears that respiratory infections and pancreatic status are the dominant factors in pulmonary prognosis in patients with CF who received similar treatment after being assigned to an early diagnosis (screened) or to a standard diagnosis (control) group.