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ISSN: 2637-4722

Progressing Aspects in Pediatrics and Neonatology

Editorial(ISSN: 2637-4722)

Meconium Ileus (MI), Presentation of Cystic Fibrosis needs more Research?

Volume 1 - Issue 2

Pramila G Menon*

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    • Department of Genetics, Immunology, Biochemistry and Nutrition, Maharashtra University of Health Sciences, India

    *Corresponding author: Pramila G Menon, Department of Genetics, Immunology, Biochemistry and Nutrition, Maharashtra University of Health Sciences, Aundh Civil hospital bldg., 3rd floor, Aundh, Pune, India

Received: January 22, 2018;   Published: January 30, 2018

DOI: 10.32474/PAPN.2018.01.000106

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Abstract

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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