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A Novel Human Epithelial Enteroid Model of Necrotizing Enterocolitis
Published on: April 10, 2019
Necrotizing enterocolitis in a patient with Microvillous Inclusion Disease: A case report
Inna Spector Cohen1,2, H Golden3, O Kobi1,4
1Pediatric Gastroenterology & Nutrition Institute, Ruth Rappaport Children's Hospital, Rambam Health Care Campus, Haifa, Israel.
Background:
Microvillous Inclusion Disease (MVID) is a rare congenital disorder causing severe diarrhea from early infancy, typically requiring long-term parenteral nutrition (PN). It results from MYO5B gene mutations, disrupting brush border protein trafficking. Necrotizing enterocolitis (NEC) is not typically linked to MVID, with only two cases reported so far.
Case Report:
A 5-month-old male with MVID developed worsening diarrhea after switching from breast milk to polymeric formula, leading to dehydration and metabolic acidosis. Amino acid-based formula did not improve his condition. Imaging confirmed NEC, showing pneumatosis intestinalis and gas in the portal system. Conservative treatment stabilized his condition, and stool output improved after switching to donated breast milk.
Conclusion:
This case underscores the potential risk of NEC in MVID patients, emphasizing the need for careful management of enteral feeding. Clinicians should maintain a high index of suspicion for NEC due to these patients' susceptibility to hypoperfusion and mucosal integrity issues.
Insights
Microvillous Inclusion Disease (MVID), a rare genetic disorder, can present with necrotizing enterocolitis (NEC). This case highlights the importance of careful enteral feeding management in MVID patients to prevent NEC complications.
Area of Science:
- Gastroenterology
- Pediatric Medicine
- Genetics
Background:
- Microvillous Inclusion Disease (MVID) is a rare congenital disorder characterized by severe infantile diarrhea, often necessitating long-term parenteral nutrition.
- MVID arises from MYO5B gene mutations, which impair brush border protein trafficking essential for intestinal function.
- Necrotizing enterocolitis (NEC) is infrequently associated with MVID, with limited prior case reports.
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