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.
D000328:Adult; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000097:blood / Q000652:urine*; D005260:Female; D006579:Heterozygote; D006801:Humans; D008297:Male; D011073:Polyamines / Q000652:urine*; D011700:Putrescine / Q000652:urine; D013095:Spermidine / Q000008:administration & dosage / Q000097:blood / Q000652:urine; D013096:Spermine / Q000652:urine
D011073:Polyamines; D013096:Spermine; D013095:Spermidine; D011700:Putrescine