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PLoS One. 2015 Nov 23;10(11):e0143459. doi: 10.1371/journal.pone.0143459. eCollection 2015.

Computed Tomography (CT) Scanning Facilitates Early Identification of Neonatal Cystic Fibrosis Piglets.

Author information

1
INSERM, Centre d'Etude des Pathologies Respiratoires, UMR 1100/EA6305, Tours, France.
2
CHU Tours, service de réanimation polyvalente, Tours, France.
3
INRA, UMR1282 ISP, Nouzilly, France.
4
INRA, UMR1277 PFIE, Nouzilly, France.
5
INRA, CIRE, Nouzilly, France.
6
CHU Clocheville, Tours, France.
7
CHU Tours, service anesthésie-réanimation, Tours, France.
8
Gene Center and Center for Innovative Medical Models (CiMM), LMU Munich, Germany.

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.

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 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.

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.

PMID:
26600426
PMCID:
PMC4658176
DOI:
10.1371/journal.pone.0143459
[Indexed for MEDLINE]
Free PMC Article

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