Orphanet: Partial pancreatic agenesis

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Partial pancreatic agenesis

Disease definition

Partial agenesis of the pancreas is characterized by the congenital absence of a critical mass of pancreatic tissue.


Classification level: Disorder
  • Synonym(s):
    • Congenital pancreatic agenesis
    • Partial agenesis of the pancreas
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q45.0
  • OMIM: 167755  260370  615935
  • UMLS: C1850096
  • MeSH: -
  • GARD: 4203
  • MedDRA: -
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