Gaucher disease: progressive mesenteric and mediastinal lymphadenopathy despite enzyme therapy.
case_report · Level V
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- Record sourced from PubMed, PMID 17236903.
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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.
Medical subject headings
- Gaucher Disease
- Glucosylceramidase
- Hepatomegaly
- Lymphatic Diseases
- Splenomegaly