Dendritic cells and monocyte subsets in children with Gaucher disease.

Autor: Zahran AM; Department of Clinical Pathology, South Egypt Cancer Institute, Assiut, Egypt., Saad K; Department of Pediatrics, Faculty of Medicine, Assiut University, Assiut, Egypt. khaled.ali@med.au.edu.eg., Abdallah AM; Department of Pediatrics, Faculty of Medicine, Assiut University, Assiut, Egypt., Gad EF; Department of Pediatrics, Faculty of Medicine, Assiut University, Assiut, Egypt., Abdel-Raheem YF; Department of Pediatrics, Faculty of Medicine, Assiut University, Assiut, Egypt., Zahran ZAM; Department of Clinical Pathology, Faculty of Medicine, Assiut University, Assiut, Egypt., Nagiub Abdelsalam EM; Department of Clinical Pathology, Faculty of Medicine, Assiut University, Assiut, Egypt., Elhoufey A; Department of Community Health Nursing, Faculty of Nursing, Assiut University, Assiut, Egypt.; Department of Community Health Nursing, Alddrab University College, Jazan University, Jazan, Saudi Arabia., Alruwaili T; Faculty of Medicine, Jouf University, Jouf, Sakaka, Saudi Arabia., Mahmoud KH; Department of Pediatrics, Faculty of Medicine, Assiut University, Assiut, Egypt., Elsayh KI; Department of Pediatrics, Faculty of Medicine, Assiut University, Assiut, Egypt.
Jazyk: angličtina
Zdroj: Pediatric research [Pediatr Res] 2021 Sep; Vol. 90 (3), pp. 664-669. Date of Electronic Publication: 2021 Jan 19.
DOI: 10.1038/s41390-020-01300-w
Abstrakt: 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: Children with Gaucher disease type 1 (GD1) have significantly reduced total and classical monocyte frequencies, with decreasing percentages for both types of dendritic cells. 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. Our results show that monocytes and dendritic cells' significant changes could contribute to immunological abnormalities in pediatric patients with GD1.
(© 2021. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.)
Databáze: MEDLINE
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