{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T01:01:28Z","timestamp":1784682088875,"version":"3.55.0"},"reference-count":38,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2025,9,22]],"date-time":"2025-09-22T00:00:00Z","timestamp":1758499200000},"content-version":"vor","delay-in-days":21,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T00:00:00Z","timestamp":1756684800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2025,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Gaucher disease (GD), caused by variants in the\n                    <jats:italic>GBA1<\/jats:italic>\n                    gene, is manifested by the accumulation of glucosylceramide within macrophages in visceral organs and bone marrow and can lead to significant bone disease. The aim of this study was to assess longitudinal bone mineral density (BMD) changes in children with GD and the impact of enzyme replacement therapy (ERT). The study included children and adolescents (5\u201320\u2009years) with GD who had at least two dual\u2010energy x\u2010ray absorptiometry (DXA) scans. These scans, done every 2\u20133\u2009years with a Hologic Discovery densitometer, were part of regular clinic visits. Whole body less head (WBLH), femoral neck, lumbar spine, and total hip BMD\n                    <jats:italic>Z<\/jats:italic>\n                    scores were adjusted for height\u2010for\u2010age\n                    <jats:italic>Z<\/jats:italic>\n                    score (HAZ). Children were grouped by ERT exposure: untreated, treated throughout, or initiated during follow\u2010up. Low BMD at baseline was more common in children who initiated ERT during follow\u2010up. By study end, BMD status was comparable across all groups. WBLH BMD improved in 52\/79, significantly more often than at the femoral neck (18\/79), lumbar spine (18\/79), or hip (20\/79), with no difference between treatment groups. Longitudinal BMD changes were unrelated to sex,\n                    <jats:italic>GBA1<\/jats:italic>\n                    genotype, follow\u2010up duration, calcium, phosphorus, alkaline phosphatase, or lyso\u2010Gb1 levels. In conclusion, with proper clinical selection, some children with GD can be safely monitored without ERT. Adjustment to HAZ and focusing on the WBLH for clinical decision\u2010making in children with GD is important. Continued monitoring into adulthood is essential to clarify long\u2010term skeletal outcomes and confirm the utility of site\u2010specific monitoring in childhood.\n                  <\/jats:p>","DOI":"10.1002\/jimd.70091","type":"journal-article","created":{"date-parts":[[2025,9,23]],"date-time":"2025-09-23T00:49:08Z","timestamp":1758588548000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Effect of Exposure to Enzyme Replacement Therapy on Bone Mineral Density in Children With Gaucher Disease"],"prefix":"10.1002","volume":"48","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9151-0337","authenticated-orcid":false,"given":"Shoshana","family":"Revel\u2010Vilk","sequence":"first","affiliation":[{"name":"Gaucher Unit, The Eisenberg R&amp;D Authority Shaare Zedek Medical Center  Jerusalem Israel"},{"name":"School of Medicine Hebrew University  Jerusalem 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Hebrew University  Jerusalem Israel"},{"name":"Pediatric Endocrinology Unit, The Eisenberg R&amp;D Authority Shaare Zedek Medical Center  Jerusalem Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,9,22]]},"reference":[{"key":"e_1_2_11_2_1","first-page":"1189","volume-title":"Williams Hematology","author":"Revel\u2010Vilk 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