A Purified Serum Glycopeptide from Controls and Cystic Fibrosis Patients. III. The Association of the Ciliary Dyskinetic Activity with the Oligosaccharide Component

Blitzer, Miriam G.; Shapira, Emmanuel

doi:10.1203/00006450-198406000-00012

Cited by 2 publications

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References 6 publications

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“…No Humoral Factors. In the decade of CF research preceding lessons on Clpermeability, there were a number of reports on the sweat duct and other in vitro systems that resulted in the once popular idea that the electrolyte defect as well as ciliary clearance of the lung was due to circulating humoral factors that inhibited cilia (25,39,52,68,172), produced other unique abnormalities (7,21,22,84,95,179,190,191), and inhibited Na + transport (100,101). The demonstration that the defect persisted and was clearly expressed in a completely in vitro system with all systemic fluids replaced by defined media provided a compelling lesson that the defect in transport was inherent in the affected cells and not induced by external "factors," as they were then called (112,121).…”

Section: Reviewsmentioning

confidence: 99%

Cystic Fibrosis: Lessons from the Sweat Gland

2007

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Lessons from the sweat gland on cystic fibrosis (CF) began long before modern medicine became a science. In European folklore, the curse that "a child that taste salty when kissed will soon die" (Alonso y de los Ruyzes de Fonteca J. Diez Previlegios para Mugeres Prenadas. Henares, Spain, 1606) has been taken by many as a direct reference to cystic fibrosis [Busch R. Acta Univ Carol Med (Praha) 36: 13-15, 1990]. The high salt concentration in sweat from patients with CF is now accepted as almost pathognomonic with this fatal genetic disease, but the earliest descriptions of cystic fibrosis as a disease entity did not mention sweat or sweat glands (Andersen DH. Am J Dis Child 56: 344-399, 1938; Andersen DH, Hodges RG. Am J Dis Child 72: 62-80, 1946). Nonetheless, defective sweating soon became an inseparable, and major, component of the constellation of symptoms that diagnose "cystic fibrosis" (Davis PB. Am J Respir Crit Care Med 173: 475-482, 2006). The sweat gland has played a foremost role in diagnosing, defining pathophysiology, debunking misconceptions, and increasing our understanding of the effects of the disease on organs, tissues, cells, and molecules. The sweat gland has taught us much.

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