ArticleJBMR plus2025
Long-term clinical and bone mineral density changes of adult patients with sclerostin deficiency due to van Buchem disease: a follow-up study.
Article in JBMR plus, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Authors and funding
6 authors.
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Abstract
Van Buchem disease (VBD) is an inherited rare sclerosing bone disorder, due to defective synthesis of sclerostin, a negative regulator of bone formation. Our earlier cross-sectional studies of patients with VBD and the closely related sclerosteosis suggested that the accrual of bone mass does not continue after puberty but longitudinal studies of patients with sclerostin deficiency are not available. The aim, therefore, of the present study was the long-term assessment of adult patients with VBD. Fifteen previously evaluated patients with genetically confirmed VBD were invited to participate in the study and 11 (4 women) consented. Mean follow-up time was 8.9 ± 1.1 yr and median age at follow-up was 47 yr (range 20-60). Seven patients developed permanent facial paresis, 9 had progressing hearing loss, and 2 developed had increased intracranial pressure requiring cranial surgery. Dental problems were common, and 3 patients developed osteoarthritis during follow-up. None experienced a cardiovascular event. BMD did not change at the LS or the left FN; Z-scores were 10.2 ± 1.3 SD vs 9.4 ± 2.3 SD,
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