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[Waldman's disease. Primary intestinal lymphangiectasis]

L Misery1, A Lachaux, M Chambon

  • 1Clinique Dermatologique, Hôpital Edouard-Herriot, Lyon.

Annales De Dermatologie Et De Venereologie
|January 1, 1996
PubMed
Summary
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Primary intestinal lymphangiectasias, a rare condition, can cause both lymphedema and hypoproteinemic edema. Early diagnosis and dietary management are key for this rare disease.

Area of Science:

  • Gastroenterology
  • Pediatrics
  • Genetics

Context:

  • Primary intestinal lymphangiectasia is a rare condition often linked to lymphedema.
  • This case highlights the diagnostic challenges and clinical presentation in infants.

Purpose:

  • To report a case of Waldmann's disease, a form of primary intestinal lymphangiectasia.
  • To illustrate the typical diagnostic pathway and management of this condition.

Summary:

  • A 4-month-old infant presented with lymphedema, diarrhea, hypoproteinemia, and hypolipidemia.
  • Duodenal biopsies confirmed intestinal lymphangiectasias.
  • Management included a high-protein, low-fat diet, medium-chain triglyceride supplementation, and elastic compression, leading to edema reduction.

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Impact:

  • This case underscores the importance of recognizing the dual edema types (lymphedema and hypoproteinemic edema) in Waldmann's disease.
  • It emphasizes the effectiveness of tailored dietary and conservative management strategies.