Abstract
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.
Impact
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Children with Gaucher disease type 1 (GD1) have significantly reduced total and classical monocyte frequencies, with decreasing percentages for both types of dendritic cells.
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GD1 children had significantly reduced frequencies of myeloid and plasmacytoid dendritic cells as compared to the controls. The GD1 children also had significant changes in monocyte subsets when compared to the controls.
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Our results show that monocytes and dendritic cells’ significant changes could contribute to immunological abnormalities in pediatric patients with GD1.
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A.M.Z., K.S., A.-E.M.A., E.F.G., Y.F.A.-R., K.I.E., and K.H.M. designed the study, followed the patients, analyzed the data, and drafted the manuscript. Z.A.M.Z., A.M.Z., and E.M.N.A. performed all laboratory investigations of the study. A.E., K.S., and T.A. drafted the manuscript. All authors were involved in the critical analysis of the final version of the manuscript. All authors approved the manuscript as submitted and agree to be accountable for all aspects of the work.
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All protocols and investigations of our study followed the regulations of the research ethics committee of Assiut University (No. 120-2015).
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Informed consent was obtained from all guardians of children included in the study.
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Zahran, A.M., Saad, K., Abdallah, Ae.M. et al. Dendritic cells and monocyte subsets in children with Gaucher disease. Pediatr Res 90, 664–669 (2021). https://doi.org/10.1038/s41390-020-01300-w
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DOI: https://doi.org/10.1038/s41390-020-01300-w
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