Original Article: Roles of specific cytokines in bone remodeling and hematopoiesis in Gaucher disease

Gaucher disease type 1 and type 3 are characterized by bone disease and hematological symptoms. It is known that monocyte/macrophage lineage is activated in Gaucher disease, and accordingly certain cytokines are elevated in blood. The aim of the present study was to explore the possible relationship...

Full description

Saved in:
Bibliographic Details
Published in:Pediatrics international 2007-12, Vol.49 (6), p.959
Main Authors: YOSHINO, MAKOTO, WATANABE, YORIKO, TOKUNAGA, YASUYUKI, HARADA, EIMIEI, FUJII, CHIEKO, NUMATA, SANAE, HARADA, MASARU, TAJIMA, ASAKO, IDA, HIROYUKI
Format: Article
Language:English
Subjects:
Bone diseases
Cytokines
Enzymes
Hematology
Pediatrics
Proteins
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Gaucher disease type 1 and type 3 are characterized by bone disease and hematological symptoms. It is known that monocyte/macrophage lineage is activated in Gaucher disease, and accordingly certain cytokines are elevated in blood. The aim of the present study was to explore the possible relationships between cytokines and bone remodeling and hematological abnormalities in this disease. The concentrations of seven cytokines and two related proteins were measured in patients with Gaucher disease type 1 and type 3 (n = 8; age range, 2-50 years) who had received enzyme replacement therapy. Concentrations of interleukin-18 and transforming growth factor-[beta]1 were elevated in patients of all clinical types. Elevation of these cytokines in Gaucher disease has not been previously reported. Analysis of correlation among cytokines and bone-turnover markers showed that interleukin-18 concentration was correlated with each of two bone formation markers of bone-specific alkaline phosphatase activity and osteocalcin concentration, whereas macrophage colony-stimulating factor concentration correlated with the bone absorption marker of N-telopeptide to helix in urine. Concentrations of macrophage colony-stimulating factor and tumor necrosis factor-[alpha] were inversely correlated with hemoglobin concentration. Interleukin-18 and monocyte macrophage colony-stimulating factor are cytokines mainly involved in the mechanism of bone disease, while macrophage colony-stimulating factor and tumor necrosis factor-[alpha] may play a role in the development of hematological abnormalities in Gaucher disease. [PUBLICATION ABSTRACT]
ISSN:1328-8067
1442-200X
DOI:10.1111/j.1442-200X.2007.02502.x