Blood concentrations of pancreatitis associated protein in neonates: relevance to neonatal screening for cystic fibrosis
a INSERM U.315
Marseille, France, b Centre de Biogénétique, CDTS Brest, c Service
de Médecine Nucléaire, CHU Brest, d Centres de Dépistage Régionaux of Bretagne,
Rennes, e Centre, Tours, f Nord-Pas de Calais, Lille, g Pays de Loire, Angers, h Provence-Alpes-Côte d'Azur,
Marseille
Correspondence to: Dr J-C Dagorn U.315 INSERM 46 Boulevard de la Gaye 13258 Marseille Cedex 09 France. Email: dagorn{at}marseille.inserm.fr
Accepted 26 October
1998
AIM
To
determine whether pancreatitis associated protein (PAP) is a marker for
cystic fibrosis which could be used in neonatal screening for the disease.
METHODSPAP 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.
RESULTSMedian 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 27 146 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.
CONCLUSIONPAP
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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© 1999 by Archives of Disease in Childhood
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