Gaucher disease: Progressive mesenteric and mediastinal lymphadenopathy despite enzyme therapy
Gaucher disease: Progressive mesenteric and mediastinal lymphadenopathy despite enzyme therapy
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DOI：
10.1016/j.jpeds.2006.10.062
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发表时间：
2007-02-01
影响因子：
5.1
通讯作者：
Grabowski, Gregory A.
中科院分区：
文献类型：
--
作者：
Burrow, T. Andrew;Cohen, Mitchell B.;Grabowski, Gregory A.
1例患有高谢病3型的5岁男童，在12个月后发展为进行性肠系膜和纵隔淋巴结病，尽管进行了酶替代治疗，但导致了蛋白缺失性肠病的发生。这些并发症是独一无二的，表明在接受酶治疗的高谢病患者中，难以接触到不同反应的间隔。
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.