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[Primary intestinal lymphangiectasis or Waldmann's disease (author's transl)]
La Semaine Des Hopitaux : Organe Fonde Par L'Association D'Enseignement Medical Des Hopitaux De Paris
|December 8, 1979
Summary
Primary intestinal lymphangiectasy, also known as Waldmann's disease, was diagnosed in a young girl presenting with leg edema. This rare condition involves protein loss through the intestines, leading to low protein and lymph levels.
Area of Science:
- Gastroenterology
- Pediatrics
- Internal Medicine
Background:
- Primary intestinal lymphangiectasia (Waldmann's disease) is a rare disorder characterized by protein-losing enteropathy.
- It often presents with symptoms like edema, hypoalbuminemia, and lymphocytopenia.
Observation:
- A young girl presented with recently developed isolated edema of her lower limbs.
- Clinical examination was unremarkable, but laboratory tests revealed hypoproteinemia (33 g/l) and lymphocytopenia (183 L/mm3).
- Hepatic and renal functions were normal.
Findings:
- Albumin turnover studies confirmed exudative enteropathy, with fecal radioactivity at 3.6% (normal <1%).
- Small intestinal biopsy revealed lymphangiectasias of the intestinal mucosa, confirming the diagnosis of primary intestinal lymphangiectasia.
Implications:
- This case highlights the importance of considering rare causes of edema and hypoalbuminemia in pediatric patients.
- Early diagnosis and management of primary intestinal lymphangiectasia are crucial to prevent complications associated with protein loss.