Mucous glycoproteins in cystic fibrosis. pp. 108-21.

Cystic fibrosis, the most common lethal genetic trait of Caucasians, causes much 
of the chronic progressive lung disease in children and young adults. 
It is also responsible for intestinal obstruction at birth (meconium ileus), 
pancreatic insufficiency with maldigestion and failure to grow, and obstructive liver 
disease in this age group. Furthermore, obliteration of the vas deferens 
causes aspermia and infertility in most males with CF. Individuals with 
CF secrete sweat which contains excessive amounts of chloride and sodium. 
Diagnostic criteria include detection of elevated chloride levels in sweat of 
individuals with typical pulmonary, gastrointestinal, or genital manifestations of the disease. 
There are several, well documented alterations in the mucous secretions of 
individuals with cystic fibrosis, any of which alone or in combination, 
may interfere with mucous clearance from the lung or from passageways 
of other organs. It seems clear that additional studies to define 
basic aspects of normal mucous biochemistry, physiology and pharmacology must precede 
or parallel studies of CF mucous secretions if data pertinent to 
CF pathophysiology are to be generated. Particular needs include an understanding 
of the chemical determinants of physical and rheological properties of these 
secretions, and elucidation of mechanisms for control of sulfated glycoprotein biosynthesis. 
