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Hyperimmunoglobulinemia and IgG Subclass Abnormalities in Children With Gaucher Disease

Marzouk, Iman MD*; Deghady, Akram MD; Omar, Omneya M. MD*; Ashour, Rofaida S. MS*

Journal of Pediatric Hematology/Oncology: October 2019 - Volume 41 - Issue 7 - p e416–e420
doi: 10.1097/MPH.0000000000001574
Online Articles: Original Articles

Gaucher disease (GD) is the most common lysosomal storage disorder, the aim of the current study was to investigate hyperimmunoglobulinemia and abnormalities of serum immunoglobulin G (IgG) subclasses in children with GD and the relation of those findings to the GD phenotype and genotype, duration of enzyme replacement therapy (ERT), and infection frequency. The study included 20 Egyptian children with GD receiving ERT and 20 age-matched and sex-matched healthy children as controls. Serum Ig and serum IgG subclass levels were measured in the children with GD. Serum IgG subclass levels were measured in the control subjects. Hyperimmunoglobulinemia was present in 15 of the 20 (75%) children with GD. In addition, it is found significantly lower IgG2 levels and significantly higher IgG3 levels in the GD group than in the control group (P<0.001 and <0.006, respectively). Patients with 12 infections per year had significantly higher IgG3 levels compared with patients with 6 infections per year (P=0.022). In conclusion, hyperimmunoglobulinemia and IgG subclass abnormalities occur in children with GD who are on ERT and may be related to recurrent infections.

Departments of *Pediatrics

Clinical Pathology, Faculty of Medicine, Alexandria University, Alexandria, Egypt

I.M. and A.D.: study concept and design. I.M., R.S.A., and O.M.M.: acquisition of data. I.M., O.M.M., and R.S.A.: analysis and interpretation of data. I.M., A.D., and O.M.M.: drafting of the manuscript. I.M., A.D., and O.M.M.: critical revision of the manuscript for important intellectual content.

The authors declare no conflict of interest.

Reprints: Omneya M. Omar, MD, Department of Pediatrics, Alexandria University, Alexandria 21321, Egypt (e-mail:

Received August 20, 2018

Accepted July 7, 2019

Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.