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Guillon, Antoine; Chevaleyre, Claire; Barc, Celine; Berri, Mustapha; Adriaensen, Hans; Lecompte, Francois; Villemagne, Thierry; Pezant, Jeremy; Delaunay, Remi; Moenne-Loccoz, Joseph; Berthon, Patricia; Baehr, Andrea; Wolf, Eckhard; Klymiuk, Nikolai; Attucci, Sylvie; Ramphal, Reuben; Sarradin, Pierre; Buzoni-Gatel, Dominique; Si-Tahar, Mustapha; Caballero, Ignacio (2015): Computed Tomography (CT) Scanning Facilitates Early Identification of Neonatal Cystic Fibrosis Piglets.
In: PLOS ONE 10(11), e0143459


Background 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. Methods and Principal Findings 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 postnatal 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. Conclusion 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.