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

被引：15
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
收藏
页码：522 / 524
页数：3
相关论文
共 9 条
  • [1] Beutler E., 2001, METABOLIC MOL BASES, V3, P3635
  • [2] Gaucher disease: Progressive mesenteric and mediastinal lymphadenopathy despite enzyme therapy
    Burrow, T. Andrew
    Cohen, Mitchell B.
    Bokulic, Ronald
    Deutsch, Gail
    Choudhary, Arabinda
    Falcone, Richard A., Jr.
    Grabowski, Gregory A.
    [J]. JOURNAL OF PEDIATRICS, 2007, 150 (02) : 202 - 206
  • [3] Management of non-neuronopathic Gaucher disease with special reference to pregnancy, splenectomy, bisphosphonate therapy, use of biomarkers and bone disease monitoring
    Cox, T. M.
    Aerts, J. M. F. G.
    Belmatoug, N.
    Cappellini, M. D.
    vom Dahl, S.
    Goldblatt, J.
    Grabowski, G. A.
    Hollak, C. E. M.
    Hwu, P.
    Maas, M.
    Martins, A. M.
    Mistry, P. K.
    Pastores, G. M.
    Tylki-Szymanska, A.
    Yee, J.
    Weinreb, N.
    [J]. JOURNAL OF INHERITED METABOLIC DISEASE, 2008, 31 (03) : 319 - 336
  • [4] Ultrastructural features of Gaucher disease treated with enzyme replacement therapy presenting as mesenteric mass lesions
    Fowler, D. J.
    Weber, M. A.
    Anderson, G.
    Malone, M.
    Sebire, N. J.
    Vellodi, A.
    [J]. FETAL AND PEDIATRIC PATHOLOGY, 2006, 25 (05) : 241 - 248
  • [5] Clinical and genetic characteristics of Korean patients with Gaucher disease
    Jeong, Seon-Yong
    Park, Sang-Jin
    Kim, Hyon J.
    [J]. BLOOD CELLS MOLECULES AND DISEASES, 2011, 46 (01) : 11 - 14
  • [6] Mesenteric mass in a young girl - an unusual site for Gaucher's disease
    Lim, AKP
    Vellodi, A
    McHugh, K
    [J]. PEDIATRIC RADIOLOGY, 2002, 32 (09) : 674 - 676
  • [7] Liver volume in children measured by computed tomography
    Noda, T
    Todani, T
    Watanabe, Y
    Yamamoto, S
    [J]. PEDIATRIC RADIOLOGY, 1997, 27 (03) : 250 - 252
  • [8] Standard splenic volume in children and young adults measured from CT images
    Watanabe, Y
    Todani, T
    Noda, T
    Yamamoto, S
    [J]. SURGERY TODAY-THE JAPANESE JOURNAL OF SURGERY, 1997, 27 (08): : 726 - 728
  • [9] Giant Lymphadenopathy Infiltrated by Gaucher Cells Mimicking Lymphoma
    Yagci, Beguel
    Salor, Oezge
    Yalcin, Bilgehan
    Guerakan, Figen
    Guecer, Safak
    Bueyuepamukcu, Muenevver
    [J]. PEDIATRIC BLOOD & CANCER, 2009, 52 (07) : 870 - 871
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