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Article Dans Une Revue PLoS ONE Année : 2015

Computed tomography (CT) scanning facilitates early identification of neonatal cystic fibrosis piglets

Céline Barc
Mustapha Berri
Patricia Berthon

Résumé

Cystic Fibrosis (CF) is the most prevalent autosomal recessive disease in the Caucasian population. A cystic fibrosis transmembrane conductance regulator knockout (CFTR-/-) pig that displays most of the features of the human CF disease has been recently developed. However, CFTR-/- pigs presents a 100% prevalence of meconium ileus that leads to death in the first hours after birth, requiring a rapid diagnosis and surgical intervention to relieve intestinal obstruction. Identification of CFTR-/- piglets is usually performed by PCR genotyping, a procedure that lasts between 4 to 6 h. Here, we aimed to develop a procedure for rapid identification of CFTR-/- piglets that will allow placing them under intensive care soon after birth and immediately proceeding with the surgical correction. Male and female CFTR+/- pigs were crossed and the progeny was examined by computed tomography (CT) scan to detect the presence of meconium ileus and facilitate a rapid post-natal surgical intervention. Genotype was confirmed by PCR. CT scan presented a 94.4% sensitivity to diagnose CFTR-/- piglets. Diagnosis by CT scan reduced the birth-to-surgery time from a minimum of 10 h down to a minimum of 2.5 h and increased the survival of CFTR-/- piglets to a maximum of 13 days post-surgery as opposed to just 66 h after later surgery. CT scan imaging of meconium ileus is an accurate method for rapid identification of CFTR-/- piglets. Early CT detection of meconium ileus may help to extend the lifespan of CFTR-/- piglets and, thus, improve experimental research on CF, still an incurable disease.
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hal-01594228 , version 1 (26-09-2017)

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Antoine Guillon, Claire Chevaleyre, Céline Barc, Mustapha Berri, Hans Adriaensen, et al.. Computed tomography (CT) scanning facilitates early identification of neonatal cystic fibrosis piglets. PLoS ONE, 2015, 10 (11), pp.1-12. ⟨10.1371/journal.pone.0143459⟩. ⟨hal-01594228⟩
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