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01-12-2003 | Article

Van Buchem disease: lifetime evolution of radioclinical features

Authors: Filip M. Vanhoenacker, Wendy Balemans, Gregorius J. Tan, Frederik G. Dikkers, Arthur M. De Schepper, Danny G. P. Mathysen, Anja Bernaerts, Wim Van Hul

Published in: Skeletal Radiology | Issue 12/2003

Abstract

Objective

The purpose of this study was to evaluate the lifetime evolution of the radioclinical features in a large family with van Buchem disease.

Design and patients

The study population included 13 patients, ranging between 6 and 69 years. The evolution of the clinical features has been assessed by retrospective analysis of the clinical records of the patients. The age-related evolution of the cortical hyperostosis and defective modeling at the tubular bones was evaluated by morphometric analysis of hand films in 9 patients, compared with 9 control individuals. Progression of sclerosis of the craniofacial bones was evaluated by analysis of the skull radiographs of eleven van Buchem patients, taken at different age.

Results and conclusions

Radioclinical features, including sclerosis of the cranial and tubular bones and cranial nerve deficit, become more prominent in older patients. Defective modeling of tubular bones, cortical thickness and medullary width progress with age. Radioclinical abnormalities of van Buchem patients become more prominent in older patients, which suggests that the van Buchem gene is very actively involved in bone metabolism throughout life. Morphometric analysis of the plain films supports the hypothesis that the physiological function of the van Buchem gene is to inhibit bone formation and possibly to regulate bone remodeling.

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Title: Van Buchem disease: lifetime evolution of radioclinical features
Authors: Filip M. Vanhoenacker
Wendy Balemans
Gregorius J. Tan
Frederik G. Dikkers
Arthur M. De Schepper
Danny G. P. Mathysen
Anja Bernaerts
Wim Van Hul
Publication date: 01-12-2003
Publisher: Springer-Verlag
Published in: Skeletal Radiology / Issue 12/2003
Print ISSN: 0364-2348
Electronic ISSN: 1432-2161
DOI: https://doi.org/10.1007/s00256-003-0675-4

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Springer Medicine

Van Buchem disease: lifetime evolution of radioclinical features

Abstract

Objective

Design and patients

Results and conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Design and patients

Results and conclusions

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