Altered polyamine metabolism in cystic fibrosis.

Children with cystic fibrosis excreted elevated urinary levels of all three polyamines--putrescine, 
spermidine, and spermine. Heterozygote parents excreted intermediate concentrations of the polyamines, 
but not levels significantly different from levels in normal controls. Patients 
with cystic fibrosis who were administered a tracer amount of [14C]spermidine 
excreted 11--13% of the radiolabel within 72 hr whereas normal controls 
excreted 60--76% of the radiolabel within 72 hr. Spermine excretion was 
positively correlated with increased pathology as assessed by the National Institutes 
of Health (NIH) clinical score, whereas urinary putrescine and spermidine levels 
were negatively correlated with increased pathology. 