Progressive mesenteric lymphadenopathy with protein-losing enteropathy; a devastating complication in Gaucher disease

Mol Genet Metab. 2012 Mar;105(3):522-4. doi: 10.1016/j.ymgme.2011.12.010. Epub 2011 Dec 16.

Abstract

Mesenteric lymphadenopathy has been rarely reported in pediatric patients with Gaucher disease, developing despite the enzyme replacement therapy. The clinical implication of this condition is undetermined, with no consensus on treatment strategies. However, this condition can reflect the progression of Gaucher disease. Moreover, it can be accompanied by the serious complication, protein-losing enteropathy. Our experience underlines the importance of careful monitoring and early intervention for mesenteric lymphadenopathy, especially in pediatric patients with neuronopathic Gaucher disease.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child
  • Disease Progression
  • Enzyme Replacement Therapy
  • Gaucher Disease / complications*
  • Gaucher Disease / pathology
  • Humans
  • Lymphatic Diseases / etiology*
  • Lymphatic Diseases / pathology
  • Male
  • Mesenteric Lymphadenitis / etiology*
  • Mesenteric Lymphadenitis / pathology
  • Protein-Losing Enteropathies / etiology*
  • Protein-Losing Enteropathies / pathology
  • Republic of Korea