title: Computed Tomography (CT) Scanning Facilitates Early Identification of Neonatal Cystic Fibrosis Piglets.
abstract: 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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: INSERM, Centre d'Etude des Pathologies Respiratoires, UMR 1100/EA6305, Tours, France.|CHU Tours, service de réanimation polyvalente, Tours, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Guillon
authors.affiliation: INRA, UMR1282 ISP, Nouzilly, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Chevaleyre
authors.affiliation: INRA, UMR1277 PFIE, Nouzilly, France.
authors.forename: Celine
authors.initials: C
authors.lastname: Barc
authors.affiliation: INRA, UMR1282 ISP, Nouzilly, France.
authors.forename: Mustapha
authors.initials: M
authors.lastname: Berri
authors.affiliation: INRA, CIRE, Nouzilly, France.
authors.forename: Hans
authors.initials: H
authors.lastname: Adriaensen
authors.affiliation: INRA, CIRE, Nouzilly, France.
authors.forename: François
authors.initials: F
authors.lastname: Lecompte
authors.affiliation: CHU Clocheville, Tours, France.
authors.forename: Thierry
authors.initials: T
authors.lastname: Villemagne
authors.affiliation: INRA, UMR1277 PFIE, Nouzilly, France.
authors.forename: Jérémy
authors.initials: J
authors.lastname: Pezant
authors.affiliation: INRA, UMR1277 PFIE, Nouzilly, France.
authors.forename: Rémi
authors.initials: R
authors.lastname: Delaunay
authors.affiliation: CHU Tours, service anesthésie-réanimation, Tours, France.
authors.forename: Joseph
authors.initials: J
authors.lastname: Moënne-Loccoz
authors.affiliation: INRA, UMR1282 ISP, Nouzilly, France.
authors.forename: Patricia
authors.initials: P
authors.lastname: Berthon
authors.affiliation: Gene Center and Center for Innovative Medical Models (CiMM), LMU Munich, Germany.
authors.forename: Andrea
authors.initials: A
authors.lastname: Bähr
authors.affiliation: Gene Center and Center for Innovative Medical Models (CiMM), LMU Munich, Germany.
authors.forename: Eckhard
authors.initials: E
authors.lastname: Wolf
authors.affiliation: Gene Center and Center for Innovative Medical Models (CiMM), LMU Munich, Germany.
authors.forename: Nikolai
authors.initials: N
authors.lastname: Klymiuk
authors.affiliation: INSERM, Centre d'Etude des Pathologies Respiratoires, UMR 1100/EA6305, Tours, France.
authors.forename: Sylvie
authors.initials: S
authors.lastname: Attucci
authors.affiliation: INSERM, Centre d'Etude des Pathologies Respiratoires, UMR 1100/EA6305, Tours, France.
authors.forename: Reuben
authors.initials: R
authors.lastname: Ramphal
authors.affiliation: INRA, UMR1277 PFIE, Nouzilly, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Sarradin
authors.affiliation: INRA, UMR1282 ISP, Nouzilly, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Buzoni-Gatel
authors.affiliation: INSERM, Centre d'Etude des Pathologies Respiratoires, UMR 1100/EA6305, Tours, France.
authors.forename: Mustapha
authors.initials: M
authors.lastname: Si-Tahar
authors.affiliation: INRA, UMR1282 ISP, Nouzilly, France.
authors.forename: Ignacio
authors.initials: I
authors.lastname: Caballero
mesh_terms: D000818:Animals; D000831:Animals, Newborn; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000000981:diagnostic imaging; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D005260:Female; D005838:Genotype; D008297:Male; D013552:Swine; D014057:Tomography, X-Ray Computed / Q000379:methods*
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator