. 2021 Aug 2;3(10):841–854. doi: 10.1096/fba.2021-00043

TABLE 2.

Pathologic findings in CFTR ^F508del ^/ ^F508del and CFTR ^G542X ^/ ^G542X lambs

	Small intestine	Pancreas	Liver	Gallbladder	Vas deferens	Lung	Kidney
CFTR F508del/F508del (n = 2)	Obstruction, Meconium ileus 2/2 (100%)	Hypoplasia, mild (1) to severe (1) Acini/duct dilation, minimal inflammation 2/2 (100%)	Portal ductular reaction, severe, with mild fibrosis Intrahepatic cholestasis, mild (1) to severe (2) 2/2 (100%)	Hypoplasia 1/2 (50%)	Aplasia or atrophy, bilateral cryptorchidism 2/2 (100%)	No lesions	Hydronephrosis 2/2 (100%)
CFTR G542X/G542X (n = 2)	Obstruction, Meconium ileus 2/2 (100%)	Hypoplasia, mild (1) and severe (1) Acini/duct dilation, minimal inflammation 2/2 (100%)	Portal ductular reaction, mild (1) to severe (1), with mild fibrosis Intrahepatic cholestasis, moderate (1) to severe (1) 2/2 (100%)	Hypoplasia 2/2 (100%)	Aplasia or atrophy, unilateral cryptorchidism 2/2 (100%)	No lesions	Hydronephrosis 2/2 (100%)
CFTR ^−/− (n = 15) ^b	Obstruction, Meconium ileus 15/15 (100%)	Hypoplasia or aplasia 11/15 (73.3%) Fibrosis 6/15 (40%)	Portal ductular reaction and fibrosis, mild to severe 11/14 (78.6%), intrahepatic cholestasis, mild to severe 12/14 (85.7%)	Hypoplasia 12/15 (80%)	Aplasia or atrophy 12/12 (100%)	No lesions	Hydronephrosis 13/15 (86.6%)

Small intestine

Pancreas

Liver

Gallbladder

Vas deferens

Lung

Kidney

CFTR F508del/F508del (n = 2)

Obstruction, Meconium ileus 2/2 (100%)

Hypoplasia, mild (1) to severe (1)

Acini/duct dilation, minimal inflammation 2/2 (100%)

Portal ductular reaction, severe, with mild fibrosis

Intrahepatic cholestasis, mild (1) to severe (2)

2/2 (100%)

Hypoplasia 1/2 (50%)

Aplasia or atrophy, bilateral cryptorchidism 2/2 (100%)

No lesions

Hydronephrosis 2/2 (100%)

CFTR G542X/G542X (n = 2)

Obstruction, Meconium ileus 2/2 (100%)

Hypoplasia, mild (1) and severe (1) Acini/duct dilation, minimal inflammation 2/2 (100%)

Portal ductular reaction, mild (1) to severe (1), with mild fibrosis

Intrahepatic cholestasis, moderate (1) to severe (1) 2/2 (100%)

Hypoplasia 2/2 (100%)

Aplasia or atrophy, unilateral cryptorchidism 2/2 (100%)

No lesions

Hydronephrosis 2/2 (100%)

CFTR ^−/− (n = 15) ^b

Obstruction, Meconium ileus 15/15 (100%)

Hypoplasia or aplasia 11/15 (73.3%) Fibrosis 6/15 (40%)

Portal ductular reaction and fibrosis, mild to severe 11/14 (78.6%), intrahepatic cholestasis, mild to severe 12/14 (85.7%)

Hypoplasia 12/15 (80%)

Aplasia or atrophy 12/12 (100%)

No lesions

Hydronephrosis 13/15 (86.6%)

^{^a}

Reference CFTR^−/− CF sheep data from Fan et al. (2018).