Related Experiment Video
Updated: Apr 18, 2026
Generation and Identification of GM-CSF Derived Alveolar-like Macrophages and Dendritic Cells From Mouse Bone Marrow
Published on: June 25, 2016
Macrophage-derived soluble CD163 level in young patients with Gaucher disease: relation to phenotypes, disease
Amira Abdel Moneam Adly1, Eman Abdel Rahman Ismail2, Taghreed Mahmoud Ibraheem1
1Pediatrics Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Objectives:
Bone and lung involvement are two major causes of morbidity in Gaucher disease (GD). The soluble form of CD163 (sCD163) is a valuable diagnostic biomarker for monitoring diseases with increased macrophage activation. We determined sCD163 levels in 30 children and adolescence with GD compared with 30 healthy controls and assessed the relation to phenotypes, disease severity and complications.
Methods:
Thirty GD patients (10 had type 1 and 20 had type 3) were studied stressing on skeletal, pulmonary or neurological manifestations, enzyme replacement therapy (ERT), hematological profile, plasma chitotriosidase activity, D-dimer and sCD163. Liver and spleen volumes and bone mineral density (BMD) were assessed.
Results:
sCD163 levels were markedly elevated in patients compared with controls. D-dimer, chitotriosidase activity and sCD163 levels were significantly increased in type 3 GD patients compared with type 1. sCD163 was significantly elevated in GD patients with dysphagia, developmental delay, pulmonary hypertension risk or abnormal BMD (osteopenia/osteoporosis) than those without. GD patients receiving ERT every 2weeks had lower levels than those under ERT for more than 2weeks. sCD163 was positively correlated with age, disease duration, severity score index, D-dimer and chitotriosidase activity. The cutoff value of sCD163 at 9400ng/mL could differentiate GD patients with and without pulmonary hypertension risk with a sensitivity of 90% and specificity of 95%.
Conclusions:
sCD163 is a biomarker for the clinical assessment of macrophage proliferation and activity that would help in risk prediction of bone and lung involvement and monitoring treatment response.
Insights
Soluble CD163 (sCD163) is elevated in Gaucher disease (GD) patients, indicating macrophage activation. Higher sCD163 levels correlate with disease severity and complications, aiding in risk prediction for bone and lung involvement.
Area of Science:
- Biochemistry
- Immunology
- Pediatrics
Background:
- Gaucher disease (GD) is a lysosomal storage disorder with significant bone and lung morbidity.
- Soluble CD163 (sCD163) reflects macrophage activation and is a potential biomarker in inflammatory and metabolic diseases.
Purpose of the Study:
- To determine sCD163 levels in pediatric GD patients.
- To assess the correlation of sCD163 with GD phenotypes, disease severity, and complications.
- To evaluate sCD163 as a biomarker for predicting bone and lung involvement.
Main Methods:
- Studied 30 children and adolescents with GD (10 type 1, 20 type 3) and 30 healthy controls.
- Assessed skeletal, pulmonary, and neurological manifestations, enzyme replacement therapy (ERT), hematological profile, chitotriosidase activity, D-dimer, and sCD163 levels.
- Measured liver and spleen volumes and bone mineral density (BMD).
Main Results:
- sCD163 levels were significantly elevated in GD patients compared to controls.
- Type 3 GD patients showed higher D-dimer, chitotriosidase, and sCD163 levels than type 1.
- Elevated sCD163 correlated with specific GD complications including dysphagia, developmental delay, pulmonary hypertension risk, and abnormal BMD.
- A cutoff of 9400 ng/mL for sCD163 effectively predicted pulmonary hypertension risk.
Conclusions:
- sCD163 serves as a valuable biomarker for assessing macrophage activity in GD.
- sCD163 aids in the clinical assessment and risk prediction of bone and lung involvement in GD.
- sCD163 monitoring can help track treatment response in Gaucher disease patients.
More Related Videos
06:35An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
Published on: February 8, 2019
10:05Development and Identification of a Novel Subpopulation of Human Neutrophil-derived Giant Phagocytes In Vitro
Published on: January 25, 2017