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Umbilical cord ulceration-intestinal atresia syndrome

Disease definition

A rare syndromic intestinal malformation characterized by ulcer formation in the umbilical cord associated with congenital upper-intestinal atresia, typically presenting with intra-uterine hemorrhaging from the ulcer site and subsequent fetal bradycardia.

ORPHA:3405

Classification level: Disorder
  • Synonym(s): -
  • Prevalence: <1 / 1 000 000
  • Inheritance: Unknown 
  • Age of onset: Antenatal
  • ICD-10: Q43.8
  • ICD-11: LD2F.1Y
  • OMIM: -
  • UMLS: C4304396
  • MeSH: C536938
  • GARD: 5403
  • MedDRA: -
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