Primary intestinal lymphangiectasia
ORPHA:90362· ICD-10 I89.0
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.
- Prevalence
- Unknown
- Age of onset
- All ages