Orphanet: Primary intestinal lymphangiectasia

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Primary intestinal lymphangiectasia

Disease definition

A rare intestinal disease characterized by dilated intestinal lacteals which cause lymph leakage into the small bowel lumen. Clinical manifestations include edema related to hypoalbuminemia (protein-losing gastro-enteropathy), asthenia, moderate diarrhea, lymphedema, serous effusion and failure to thrive in children.


Classification level: Disorder
  • Synonym(s):
    • Waldmann disease
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: All ages
  • ICD-10: I89.0
  • OMIM: 152800
  • UMLS: C0267372  C2931241
  • MeSH: C536567
  • GARD: 7873
  • MedDRA: -

Detailed information


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