Bone Fragility in Hereditary Connective Tissue Disorders: A Systematic Review and Meta-Analysis |
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| Institution: | 1. Division of Endocrinology and Metabolism, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand;2. Division of Nuclear Medicine, Department of Radiology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand;3. Department of Medicine, Mount Auburn Hospital, Harvard Medical School, Cambridge, MA, USA;4. Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand;5. Harvard T.H. Chan School of Public Health, Boston, MA, USA;2. Department of Biostatistics (MK), School of Medicine, Yokohama City University, Yokohama, Japan;3. Department of Anesthesiology (MK), Keiyu Hospital, Yokohama, Japan;4. Department of Health Data Science (TM), Graduate School of Data Science, Yokohama City University, Yokohama, Japan;5. Department of Anesthesiology (TM), School of Medicine, Yokohama City University, Yokohama, Japan;11. Department of Anesthesiology (MO), Saitama Cancer Center, Saitama, Japan |
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| Abstract: | ObjectiveTo investigate bone fragility in patients with hereditary connective tissue disorders (HCTD), including Ehlers-Danlos syndrome (EDS), Marfan’s syndrome (MFS) and Loeys-Dietz syndrome (LDS).MethodsFrom inception to June 2022, potentially eligible studies were identified in the Medline and EMBASE databases using search strategy that included terms for “HCTD”, “Fracture” and “Osteoporosis”. Eligible studies must consist of a group of patients with HCTD and report prevalence/incidence of fracture/osteoporosis in their participants, with or without comparison with healthy individuals. Point estimates with standard errors were obtained from each study and combined using the generic inverse variance method.ResultsAmong the 4206 articles identified, 19 studies were included. The pooled prevalence of fracture in EDS, MFS, and LDS were 44% (95% confidence interval CI], 25% to 65%, I2 88%), 17% (95% CI, 11% to 26%, I2 68%), 69% (95% CI, 47% to 85%, I2 83%), respectively. The pooled prevalence of osteoporosis in EDS was 17% (95% CI, 8% to 34%, I2 96%). EDS was associated with fracture pooled odds ratio {OR} 4.90 (95% CI, 1.49 – 16.08, I2 86%)], but not osteoporosis pooled OR 1.34 (95% CI, 0.28 – 6.36, I2 87%). One study reported a 5% (95% CI, 3% to 8%) prevalence of osteoporosis in MFS, which was associated with fracture incidence rate ratio 1.35 (95% CI, 1.18 – 1.55)] and osteoporosis subhazard ratio 3.97 (95% CI, 2.53 – 6.25)].ConclusionEDS was associated with fracture, which could be independent of osteoporosis status. MFS had a milder degree of increased risk of fracture and osteoporosis. Despite no data from cohort studies, there was a significantly higher rate of fracture in LDS. |
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| Keywords: | hereditary connective tissue disorder Ehlers-Danlos syndrome Marfan’s syndrome Loeys-Dietz syndrome fracture osteoporosis CI"} {"#name":"keyword" "$":{"id":"kwrd0010a"} "$$":[{"#name":"text" "_":"confidence interval EDS"} {"#name":"keyword" "$":{"id":"kwrd0010c"} "$$":[{"#name":"text" "_":"Ehlers-Danlos syndrome FBN-1"} {"#name":"keyword" "$":{"id":"kwrd0010e"} "$$":[{"#name":"text" "_":"fibrillin-1 HCTD"} {"#name":"keyword" "$":{"id":"kwrd0010h"} "$$":[{"#name":"text" "_":"hereditary connective tissue disorders hEDS"} {"#name":"keyword" "$":{"id":"kwrd0010j"} "$$":[{"#name":"text" "_":"Ehlers-Danlos syndrome Hypermobility type LDS"} {"#name":"keyword" "$":{"id":"kwrd0010l"} "$$":[{"#name":"text" "_":"Loeys-Dietz syndrome MFS"} {"#name":"keyword" "$":{"id":"kwrd0010n"} "$$":[{"#name":"text" "_":"Marfan’s syndrome OR"} {"#name":"keyword" "$":{"id":"kwrd0010p"} "$$":[{"#name":"text" "_":"odds ratio TGF-β"} {"#name":"keyword" "$":{"id":"kwrd0010r"} "$$":[{"#name":"text" "_":"transforming growth factor-β |
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