Gaucher disease: progressive mesenteric and mediastinal lymphadenopathy despite enzyme therapy
- PMID: 17236903
- DOI: 10.1016/j.jpeds.2006.10.062
Gaucher disease: progressive mesenteric and mediastinal lymphadenopathy despite enzyme therapy
Abstract
A 5-year-old male with Gaucher's disease type 3 developed progressive mesenteric and mediastinal lymphadenopathy over 12 months, despite enzyme replacement therapy, contributing to the development of a protein-losing enteropathy. These complications are unique, indicating poorly accessible, differentially responsive compartments in patients with Gaucher's disease who are receiving enzyme therapy.
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