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

被引：17

作者：

Lee, Beom Hee ^{[1
]}

Kim, Dae-Yeon ^{[2
]}

Kim, Gu-Hwan ^{[3
]}

Cho, Kyung-Ja ^{[4
]}

Yoon, Hye-Kyung ^{[5
]}

Yoo, Han-Wook ^{[1
]}

机构：

[1] Univ Ulsan, Asan Med Ctr, Coll Med, Dept Pediat,Childrens Hosp, Seoul 138736, South Korea

[2] Univ Ulsan, Asan Med Ctr, Coll Med, Dept Surg,Childrens Hosp, Seoul 138736, South Korea

[3] Univ Ulsan, Asan Med Ctr, Coll Med, Dept Pathol, Seoul 138736, South Korea

[4] Univ Ulsan, Asan Med Ctr, Coll Med, Dept Radiol, Seoul 138736, South Korea

[5] Univ Ulsan, Asan Med Ctr, Coll Med, Ctr Med Genet, Seoul 138736, South Korea

来源：

MOLECULAR GENETICS AND METABOLISM | 2012年 / 105卷 / 03期

关键词：

Gaucher disease; Mesenteric lymphadenopathy; Protein-losing enteropathy; CHILDREN; THERAPY; VOLUME; MASS;

D O I：

10.1016/j.ymgme.2011.12.010

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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. (C) 2011 Elsevier Inc. All rights reserved.

引用

页码：522 / 524

页数：3

共 9 条

[1]

Beutler E., 2001, METABOLIC MOL BASES, V3, P3635

[2] Gaucher disease: Progressive mesenteric and mediastinal lymphadenopathy despite enzyme therapy [J].