Gastrointestinal (GI) tract gets affected earlier in the
development stage of cystic fibrosis due to CFTR mutations .
CFTR gene is present all over the intestinal epithelial cells .
It controls secretion of chloride, bicarbonate and fluids. CFTR
mutations result in abnormal electrolyte composition leading to
abnormality in fluid secretions which alters the epithelial surface.
This creates a dry luminal environment and bicarbonate deficiency
in the proximal small intestine . This can lead to terminal ileum
obstruction, which when left untreated can result in rupture and
sepsis known as meconium ileus (MI) in cystic fibrosis neonates.
This can also present itself as distal intestinal obstructive syndrome
(DIOS). The material which is mucofeculent, adheres to mucosal
surface, giving appearance as bubbly-granular mass in the right
lower quadrant on radiographs of the abdomen. It is found that less
than 50% of patients that develop DIOS had MI as infants .