PEDIATRICS Vol. 48 No. 5 November 1971, pp. 695-710
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RELATING MUCUS, CALCIUM, AND SWEAT IN A NEW CONCEPT OF CYSTIC FIBROSIS

Lewis E. Gibson M.D.1, Wallace J. Matthews Jr. M.D.1, Patrick T. Minihan M.D.1, and John A. Patti M.D.1

1 Department of Pediatrics, Yale University School of Medicine, and the Yale-New Haven Hospital, New Haven, Connecticut

Six experiments are described. A mucus-containing secretion from cystic fibrosis (CF) patients is shown to be hyperpermeable in vitro. Hyperpermeability is demonstrated in vivo after either mucus removal or the addition of CF serum. Calcitim is shown to elevate sweat chloride, and to cause hvperpermeability both in vitro and in vivo.

The implications of these experiments are discussed. The investigations of other workers are reviewed, and a unified theory concerning CF is presented. In this theory it is suggested that mucus has a biological function of inhibiting the passive flow of water and small ions, that CF is a disease in which mucus is hyperpermeable and fails to perform this function adequately, and that this failure is caused by an excess secretion of calcium from exocrine glands.

Submitted on October 22, 1970
Accepted on July 9, 1971




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