Gorham-Stout disease (GSD) is a rare disorder characterized by progressive osteolysis and angiomatous proliferation. Since the mechanisms leading to bone loss in GSD are not completely understood, we performed histological, serum, cellular and molecular analyses of 7 patients. Increased vessels, osteoclast number and osteocyte lacunar area were revealed in patients’ bone biopsies. Biochemical analysis of sera showed high levels of ICTP, Sclerostin, VEGF-A and IL-6. In vitro experiments revealed increased osteoclast differentiation and activity, and impaired mineralization ability of osteoblasts. To evaluate the involvement of systemic factors in GSD, control cells were treated with patients’ sera and displayed an increase of osteoclastogenesis, bone resorption activity and a reduction of osteoblast function. Interestingly, GSD sera stimulated the vessel formation by endothelial cells EA.hy926. These results suggest that bone cell autonomous alterations with the cooperation of systemic factors are involved in massive bone loss and angiomatous proliferation observed in GSD patients.

Dissecting the mechanisms of bone loss in Gorham-Stout disease / Rossi, M.; Buonuomo, P. S.; Battafarano, G.; Conforti, A.; Mariani, E.; Algeri, M.; Pelle, S.; D'Agostini, M.; Macchiaiolo, M.; De Vito, R.; Gonfiantini, M. V.; Jenkner, A.; Rana, I.; Bartuli, A.; Del Fattore, A.. - In: BONE. - ISSN 8756-3282. - 130:(2020), pp. 1-16. [10.1016/j.bone.2019.115068]

Dissecting the mechanisms of bone loss in Gorham-Stout disease

Battafarano G.;Pelle S.
Membro del Collaboration Group
;
2020

Abstract

Gorham-Stout disease (GSD) is a rare disorder characterized by progressive osteolysis and angiomatous proliferation. Since the mechanisms leading to bone loss in GSD are not completely understood, we performed histological, serum, cellular and molecular analyses of 7 patients. Increased vessels, osteoclast number and osteocyte lacunar area were revealed in patients’ bone biopsies. Biochemical analysis of sera showed high levels of ICTP, Sclerostin, VEGF-A and IL-6. In vitro experiments revealed increased osteoclast differentiation and activity, and impaired mineralization ability of osteoblasts. To evaluate the involvement of systemic factors in GSD, control cells were treated with patients’ sera and displayed an increase of osteoclastogenesis, bone resorption activity and a reduction of osteoblast function. Interestingly, GSD sera stimulated the vessel formation by endothelial cells EA.hy926. These results suggest that bone cell autonomous alterations with the cooperation of systemic factors are involved in massive bone loss and angiomatous proliferation observed in GSD patients.
2020
bone histomorphometry; gorham-stout disease; osteoblast; osteoclast; osteolysis
01 Pubblicazione su rivista::01a Articolo in rivista
Dissecting the mechanisms of bone loss in Gorham-Stout disease / Rossi, M.; Buonuomo, P. S.; Battafarano, G.; Conforti, A.; Mariani, E.; Algeri, M.; Pelle, S.; D'Agostini, M.; Macchiaiolo, M.; De Vito, R.; Gonfiantini, M. V.; Jenkner, A.; Rana, I.; Bartuli, A.; Del Fattore, A.. - In: BONE. - ISSN 8756-3282. - 130:(2020), pp. 1-16. [10.1016/j.bone.2019.115068]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1337345
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