Recent advances in cystic fibrosis research.

Cystic fibrosis at one time was considered to be a lethal condition 
with death occurring at birth or within the first year of 
life. With the advent of antibiotic therapy, pancreatic enzyme replacement, extensive 
physical therapy and generally improved medical treatment, many patients now survive 
to adulthood. Clinical manifestations in CF vary from patient to patient 
and may include pulmonary disease, malnutrition, pancreatic insufficiency and defective ion 
transport. The most reliable laboratory method for diagnosis of CF is 
testing for elevated sweat electrolyte levels. Estimates of the incidence of 
CF vary from 1 in 2000 to 1 in 3500 among 
white populations of mid-European heritage. The disease is rare in black 
populations and almost non-existent in Orientals. Since 1967 there has been 
an intensive search for a circulating factor or factors which may 
be directly or indirectly responsible for the malfunctions manifest in this 
disease. Sweat factors, serum factors, metachromasia, bioassay variations, and properties of 
the ciliary inhibitor are discussed. Several groups working in CF research 
have centered their attention on the serum complement system. Agglutination and 
mucus factors, transport studies, enzyme studies, and prenatal diagnosis are also 
examined. 