A rare intestinal disease characterized by potentially life-threatening inflammatory bowel necrosis predominantly affecting preterm neonates. Patients may present with feeding intolerance lethargy temperature instability abdominal distention blood-stained stools diarrhea bilious vomiting apnea and signs of sepsis. Radiographic features include pneumatosis intestinalis portal venous gas presence of fixed dilated intestinal loops bowel wall edema and (in case of bowel perforation) pneumoperitoneum.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: //www.orphadata.org. Data version September 2023.
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