Meconium Ileus (MI), Presentation of Cystic Fibrosis needs more Research? by Pramila G Menon in Progressing Aspects in Pediatrics and Neonatology - Lupine Publishers
Gastrointestinal (GI) tract gets affected earlier in the development
stage of cystic fibrosis due to CFTR mutations [1]. CFTR gene is present
all over the intestinal epithelial cells [2]. 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 [3]. 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 [4].
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