BACKGROUND: There are minimal data on the frequencies of monocyte subsets and dendritic cells (DCs) in children with Gaucher disease (GD), as nearly all previous studies have involved adult patients. Consequently, we aimed to describe the changes in these cell subpopulations in children with GD type 1 who were on regular enzyme replacement therapy (ERT).
METHODS: This case–control study included 25 children with GD1 and 20 healthy controls. All participants underwent investigations such as complete blood count and flow cytometric assessment of DC and monocyte frequencies and phenotype. RESULTS: We found that GD1 children had significantly reduced percentages of both types of DCs, i.e., plasmacytoid DCs and myeloid DCs, compared to the control group. There was also a significant reduction in absolute monocyte numbers and percentage of classical monocyte. Moreover, the GD1 children had higher frequencies of non-classical and intermediate monocytes than the control group.
CONCLUSIONS: Our results so far indicate that, when compared to the control group, the GD1 children had significantly reduced total and classical monocyte, with significantly decreased frequencies for both types of DCs. These changes can contribute to immunological abnormalities in pediatric patients with GD1.
Research Authors
Asmaa M. Zahran1, Khaled Saad 2, Alam-Eldin M. Abdallah2, Eman F. Gad2, Yasser F. Abdel-Raheem2, Zeinab Albadry M. Zahran3, Eman M. Nagiub Abdelsalam3, Amira Elhoufey4,5, Thamer Alruwaili6, Khaled Hashim Mahmoud2 and Khalid I. Elsayh2
Research Date
Research Department
Research File
Research Journal
Pediatric Research
Research Member
Research Publisher
Springer nature
Research Rank
Clinical Research Article
Research Website
Zahran, A.M., Saad, K., Abdallah, Ae.M. et al. Dendritic cells and monocyte subsets in children with Gaucher disease. Pediatr Res (2021). https://doi.org/10.1038/s41390-020-01300-w
Research Year
9 January 2021
Research Abstract