Gaucher Disease: Progressive Mesenteric and Mediastinal Lymphadenopathy Despite Enzyme Therapy - 12/08/11
Doi : 10.1016/j.jpeds.2006.10.062
T. Andrew Burrow, MD, Mitchell B. Cohen, MD, Ronald Bokulic, MD, Gail Deutsch, MD, Arabinda Choudhary, MD, Richard A. Falcone, MD, Gregory A. Grabowski, MD ⁎ 
Divisions of Human Genetics, Gastroenterology, Hepatology and Nutrition, and Pulmonology, Departments of Pathology, Radiology, and Pediatric Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio; and the Departments of Pediatrics, Pathology, Radiology, and Surgery, University of Cincinnati College of Medicine, Cincinnati, Ohio.
Reprint requests: Dr Gregory A. Grabowski, Director, Division of Human Genetics, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Avenue, MLC 4006, Cincinnati, OH 45229.Résumé |
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.
Le texte complet de cet article est disponible en PDF.Abbreviations : CT, GCase, MRI
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Vol 150 - N° 2
P. 202-206 - février 2007 Retour au numéro
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