Literature DB >> 25521082

Mesenteric and Mediastinal Lymphadenopathy in Egyptian Children With Gaucher Disease Types 1 and 3 Treated With Enzyme Replacement Therapy.

Magy Abdelwahab1, Hadeel M SeifEldien.   

Abstract

Gaucher disease (GD) is characterized by 3 clinical subtypes; type 1 GD (non-neuronopathic) and types 2 and 3 GD (acute and chronic neuronopathic forms, respectively). There are few reports of mesenteric and mediastinal lymphadenopathy, and none in type 1 GD or in African people. We report 8 Egyptian GD children (3 type 1 GD and 5 type 3 GD) who developed mesenteric and mediastinal lymphadenopathy despite receiving enzyme replacement therapy. Biopsy showed infiltration with Gaucher cells and no malignant changes. Pediatric physicians should be aware of possible lymphadenopathy associated with both types 1 and 3 GD as it can mimic malignancy.

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Year:  2015        PMID: 25521082     DOI: 10.1097/MPH.0000000000000297

Source DB:  PubMed          Journal:  J Pediatr Hematol Oncol        ISSN: 1077-4114            Impact factor:   1.289


  4 in total

1.  Gaucher Disease with Mesenteric Lymphadenopathy: A Case with 13-year Follow-up.

Authors:  Zi-Xing Ye; Xiang Gao; Qiang Qu; Xin Ye; Xiao-Dong He
Journal:  Chin Med J (Engl)       Date:  2016-10-20       Impact factor: 2.628

2.  Successful therapy for protein-losing enteropathy caused by chronic neuronopathic Gaucher disease.

Authors:  A A Mhanni; M Kozenko; J N Hartley; M Deneau; W El-Matary; C Rockman-Greenberg
Journal:  Mol Genet Metab Rep       Date:  2015-12-29

3.  Very rare condition of multiple Gaucheroma: A case report and review of the literature.

Authors:  Szu-Yin Tseng; Dau-Ming Niu; Tzu-Hung Chu; Yi-Chen Yeh; Man-Hsu Huang; Tsui-Feng Yang; Hsuan-Chieh Liao; Chuan-Chi Chiang; Hui-Chen Ho; Wen-Jue Soong; Chia-Feng Yang
Journal:  Mol Genet Metab Rep       Date:  2019-05-09

4.  Very rare condition of multiple Gaucheroma: A case report and review of the literature.

Authors:  Szu-Yin Tseng; Dau-Ming Niu; Tzu-Hung Chu; Yi-Chen Yeh; Man-Hsu Huang; Tsui-Feng Yang; Hsuan-Chieh Liao; Chuan-Chi Chiang; Hui-Chen Ho; Wen-Jue Soong; Chia-Feng Yang
Journal:  Mol Genet Metab Rep       Date:  2019-07-12
  4 in total

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