Wednesday 26 September 2018

Meconium Ileus (MI), Presentation of Cystic Fibrosis needs more Research? (PAPN) - Lupine Publisehrs

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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