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Archives of Disease in Childhood. Fetal and Neonatal Edition logoLink to Archives of Disease in Childhood. Fetal and Neonatal Edition
. 1999 Mar;80(2):F118–F122. doi: 10.1136/fn.80.2.f118

Blood concentrations of pancreatitis associated protein in neonates: relevance to neonatal screening for cystic fibrosis

J Sarles, S Barthellemy, C Ferec, J Iovanna, M Roussey, J Farriaux, A Toutain, J Berthelot, N Maurin, J Codet, P Berthezene, J Dagorn
PMCID: PMC1720905  PMID: 10325788

Abstract

AIM—To determine whether pancreatitis associated protein (PAP) is a marker for cystic fibrosis which could be used in neonatal screening for the disease.
METHODS—PAP was assayed on screening cards from 202 807 neonates. Babies with PAP ⩾ 15 ng/ml, or ⩾ 11.5 ng/ml and immunoreactive trypsinogen (IRT) ⩾ 700 ng/ml were recalled for clinical examination, sweat testing, and cystic fibrosis transmembrane regulator (CFTR) gene analysis.
RESULTS—Median PAP value was 2.8 ng/ml. Forty four cases of cystic fibrosis were recorded. Recalled neonates (n=398) included only 11 carriers. A receiver operating characteristic curve analysis showed that PAP above 8.0 ng/ml would select 0.76% of babies, including all those with cystic fibrosis, except for one with meconium ileus and two with mild CFTR mutations. Screening 27146 babies with both PAP and IRT showed that only 0.12% had PAP> 8.0 ng/ml and IRT > 700 ng/ml, including all cases of cystic fibrosis.
CONCLUSION—PAP is increased in most neonates with cystic fibrosis and could be used for CF screening. Its combination with IRT looks promising.



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Selected References

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