1 Scanlin TF. Cystic fibrosis. In: Fishman AP, ed. Pulmonary diseases and disorders. 2nd ed. New York: McGraw-Hill, 1988:1273-94 2 Rommens JM, lanuzzi MC, Kerem B, Drumm ML, Melmer G, Dean M, et al. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science 1989; 245:1059-65 3 Kerem B, Rommens JM, Buchanan JA, Markiewicz D, Cos TK, Chakvarti A, et al. Identification of ...

The median life expectancy for cystic fibrosis is now over 30 years, and it is projected that in newborn infants it will become more than 40 years. The identification of the cystic fibrosis gene and its product, cystic fibrosis transmembrane conductance regulator (CFTR), has widened the spectrum of the disease from the classical case of the infant with cystic fibrosis to the elderly childless m...

Experimental evidence of fluid secretion of rabbit lacrimal gland duct epithelium. A novel, protective role of ursodeoxycholate in bile-induced pancreatic ductal injury. levels and function of the cystic fibrosis transmembrane conductance regulator to promote development of pancreatitis.

INTRODUCTION Cystic fibrosis, an epithelial cell transport disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator gene, is not generally associated with malformations of the central nervous system. We review eight previously published reports detailing an infrequent association between cystic fibrosis and Chiari I malformation. CASE PRESENTATION To the best of...

F. Antigny and co-workers raise interesting points in their comment on the transcriptomic analysis of cystic fibrosis transmembrane conductance regulator (CFTR)-impaired endothelial cells (ECs) revealing a pro-inflammatory phenotype [1]. Indeed, CFTR impairment different models organisms, absence concomitant infection, suggests that ECs are an overlooked mediator exaggerated observed (CF). Whet...