Table 2.

Gastrointestinal manifestation of CF disease in humans and animal models

	Species
Manifestation	Human	Mouse	Pig	Ferret	Rat
Meconium ileus obstruction	∼15% incidence (41, 86, 127)	Up to 3-fold increased mortality compared with controls in first few days of life (54, 94, 111)	100% incidence (87, 100)	75% incidence (121)	0%* (124)
DIOS obstruction/constipation	DIOS incidence: 6.2 events per 1,000 patient yr (61) Constipation: ≤47% of CF patients (126)	∼0–100% mortality weeks after weaning, dependent on background (54, 94, 111)	Prophylaxis treatment with some “episodes” (113)	Prophylaxis treatment with some “episodes” (119, 121)	∼70% mortality due to intestinal obstruction by 6 wk of age (124)
Diverticula	7- to 14-fold increased risk (50)	NR	Incidence may be proportional to meconium ileus severity (79)	NR	NR
Atresia	CF infants have >200-fold increased risk (99)	NR	Incidence may be proportional to meconium ileus severity (79)	NR	NR
GERD	∼35–80% incidence (106, 132)	NR	NR, but treated as prophylaxis (87, 113)	NR, but treated as prophylaxis (121)	NR
Motility	Delayed transit with prominent intestinal smooth muscle (15, 49, 55, 134)	Increased peak isometric force and increased relaxation in CF intestines with increased smooth muscle (98)	Increased size of tunica muscularis from duodenum to colon (79)	NR	NR
Dysbiosis	Genotype and disease severity influence dysbiosis; SIBO also reported (36, 71, 72, 104)	SIBO/dysbiosis reported, may alter motility and inflammation; defective antimicrobial peptides from crypt may accentuate (25, 32, 33, 75)	NR	Dysbiosis/SIBO influenced by environment, and not genotype (119)	NR
Rectal prolapse	Historically ≤20%, especially in young children (5, 15)	NR	NR	Up to 30% at ∼1 mo of age (119)	NR

DIOS, distal intestinal obstruction syndrome; GERD, gastroesophageal reflux disease; NR, not reported; SIBO, small intestinal bacterial overgrowth.

^*Based on preliminary phenotypic study.