The role of osteogenic cells in the pathophysiology of paget's disease

Robey, Pamela Gehron; Bianco, Paolo

doi:10.1002/jbmr.5650140204

Cited by 14 publications

(7 citation statements)

References 57 publications

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“…11 There is an increased risk of HO with THAs in PDB due to the prolonged operative time and abnormalities in osteogenic differentiation. 23,24 The results shown in this study for both cementless and cemented THAs were significantly higher than the reported rates of HO of 15.7% in nonpagetoid bone in the literature. 25 The presence of HO in cementless and cemented arthroplasties were similar, with no statistically significant difference between the cohorts.…”

Section: Discussioncontrasting

confidence: 66%

Cemented versus Cementless Total Hip Arthroplasty in Paget's Disease of Bone: A Systematic Review

et al. 2017

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The purpose of this study is to systematically review the clinical evidence for the use of cemented and cementless total hip arthroplasty (THA) in Paget's disease of bone (PDB), and to ascertain whether any difference exists in clinical outcomes between the two implant types. MEDLINE, EMBASE, and the Cochrane Library databases were searched in accordance with the preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. The quality of the studies was assessed using the methodological index for nonrandomized studies (MINORS) checklist. The reported clinical outcomes were evaluated using risk ratio (RR) with a p < 0.05 considered as statistically significant. Thirteen clinical studies with 444 primary THAs were included in this review. Cementless THA resulted in lower rates of aseptic loosening and lower rates of revision due to aseptic loosening (p < 0.05), but there was no overall statistically significant increase in the incidence of revision in the cemented THAs (p = 0.21). The postoperative Harris hip score was similar between both the groups. Also, the presence of heterotrophic ossification was similar in both the groups. Functional outcomes and survivorship were equivalent using either cementless or cemented components, with a similar rate of overall revisions. However, cementless components resulted in a decreased incidence of aseptic loosening and revision due to aseptic loosening. The current literature consists of a low level of evidence, level IV, which limits the conclusion of this study.

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Section: Discussioncontrasting

confidence: 66%

Cemented versus Cementless Total Hip Arthroplasty in Paget's Disease of Bone: A Systematic Review

et al. 2017

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“…The involvement of BMSC in skeletal disorders is well known, such as in fibrous dysplasia of bone, Paget's disease, hyperparathyroidism, osteoporosis, and osteoarthritis [13][14][15][16][17]. The evaluation of the biological and functional characteristics of BMSC from patients affected by these diseases has provided additional insights into some of the pathogenetic mechanisms underlying the disorders.…”

Section: Introductionmentioning

confidence: 99%

Hurler Disease Bone Marrow Stromal Cells Exhibit Altered Ability to Support Osteoclast Formation

Gatto

Redaelli

Salvadè

et al. 2012

Stem Cells and Development

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Mucopolysaccharidosis type I (MPS IH; Hurler syndrome) is a rare genetic disorder that is caused by mutations in the α-L-iduronidase (IDUA) gene, resulting in the deficiency of IDUA enzyme activity and intra-cellular accumulation of glycosaminoglycans. A characteristic skeletal phenotype is one of the many clinical manifestations in Hurler disease. Since the mechanism(s) underlying these skeletal defects are not completely understood, and bone and cartilage are mesenchymal lineages, we focused on the characterization of mesenchymal cells isolated from the bone marrow (BM) of 5 Hurler patients. IDUA-mutated BM stromal cells (BMSC) derived from MPS IH patients exhibited decreased IDUA activity, consistent with the disease genotype. The expansion rate, phenotype, telomerase activity, and differentiation capacity toward adipocytes, osteoblasts, chondrocytes, and smooth muscle cells in vitro of the MPS I BMSC lines were similar to those of BMSC from age-matched normal control donors. MPS I BMSC also had a similar in vivo osteogenic capacity as normal BMSC. However, MPS I BMSC displayed an increased capacity to support osteoclastogenesis, which may correlate with the up-regulation of the RANKL/RANK/OPG molecular pathway in MPS I BMSC compared with normal BMSC.

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“…Yet when BMSCs/SSCs carrying natural or targeted genetic changes are transplanted in vivo into the subcutis of immunodeficient mice, they generate ectopic ossicles that recapitulate pathological features of the original lesion; thus, abnormal BMSCs/SSCs can single-handedly re-create a disease (Bianco et al, 2000; Holmbeck et al, 1999; Kuznetsov et al, 2004; Riminucci et al, 2001). Moreover, in several bone disorders, such as fibrous dysplasia of bone, hyperparathyroidism, and early stages of Paget disease, distinct changes in the number, organization, and apparent function of marrow stromal cells are as prominent as the changes in bony structures themselves (Bianco and Robey, 1999; Robey and Bianco, 1999). Consequently, analysis of marrow stroma, both qualitative and quantitative, provides another way to probe pathogenetic mechanisms related to SSC activity in both humans and transgenic animals.…”

Section: Introductionmentioning

confidence: 99%

Enumeration of the colony-forming units–fibroblast from mouse and human bone marrow in normal and pathological conditions

et al. 2009

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Bone marrow stromal cell populations, containing a subset of multipotential skeletal stem cells, are increasingly contemplated for use in tissue engineering and stem cell therapy, whereas their involvement in the pathogenetic mechanisms of skeletal disorders is far less recognized. We compared the concentrations of stromal clonogenic cells, colony forming units–fibroblast (CFU-Fs), in norm and pathology. Initially, culture conditions were optimized by demonstrating that fetal bovine serum heat inactivation could significantly repress colony formation. Using non-heat-inactivated fetal bovine serum, the concentration of CFU-Fs (colony-forming efficiency, CFE) ranged from 3.5 ± 1.0 to 11.5 ± 4.0 per 1 × 105 nucleated cells in five inbred mouse strains. In four transgenic lines with profound bone involvement, CFE was either significantly reduced or increased compared to wild-type littermates. In normal human donors, CFE decreased slightly with age and averaged 52.2 ± 4.1 for children and 32.3 ± 3.0 for adults. CFE was significantly altered in patients with several skeletal, metabolic, and hematological disorders: reduced in congenital generalized lipodystrophy, achondroplasia (SADDAN), pseudoachondroplasia, and Paget disease of bone and elevated in alcaptonuria and sickle cell anemia. Our findings indicate that under appropriate culture conditions, CFE values may provide useful insights into bone/bone marrow pathophysiology.

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The role of osteogenic cells in the pathophysiology of paget's disease

Cited by 14 publications

References 57 publications

Cemented versus Cementless Total Hip Arthroplasty in Paget's Disease of Bone: A Systematic Review

Cemented versus Cementless Total Hip Arthroplasty in Paget's Disease of Bone: A Systematic Review

Hurler Disease Bone Marrow Stromal Cells Exhibit Altered Ability to Support Osteoclast Formation

Enumeration of the colony-forming units–fibroblast from mouse and human bone marrow in normal and pathological conditions

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