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https://www.readbyqxmd.com/read/29033382/clinical-pathological-correlations-in-three-patients-with-fibrodysplasia-ossificans-progressiva
#1
Kelly L Wentworth, Katherine Bigay, Tea V Chan, Jennifer P Ho, Blanca M Morales, Joseph Connor, Erin Brooks, M Shahriar Salamat, Henry Charles Sanchez, Geoffrey Wool, Robert J Pignolo, Frederick S Kaplan, Edward C Hsiao
OBJECTIVE: Fibrodysplasia ossificans progressiva (FOP) is an ultra-rare genetic disorder in which heterotopic bone forms in the soft tissues. This often occurs in response to injury or inflammation, leading to joint immobilization and significant disability. There are currently no definitive treatment options for this devastating disease. Although the most dramatic phenotype in FOP is the episodic and progressive heterotopic ossification, patients report a number of symptoms that affect other organ systems...
October 12, 2017: Bone
https://www.readbyqxmd.com/read/29033381/the-effect-of-once-yearly-zoledronic-acid-on-hip-structural-and-biomechanical-properties-derived-using-computed-tomography-ct-in-japanese-women-with-osteoporosis
#2
Masako Ito, Teruki Sone, Masataka Shiraki, Satoshi Tanaka, Chika Irie, Yuri Ota, Toshitaka Nakamura
The effects of zoledronic acid on hip structural and biomechanical properties were evaluated in Japanese patients with osteoporosis by computed tomography (CT). The subjects included in this study were a subset of female subjects (zoledronic acid group, 49 subjects; placebo group, 53 subjects) in the phase 3 trial (ZONE study) who were available for multi-detector row CT (MDCT) scanning. Eligible subjects were those diagnosed with primary osteoporosis based on the Diagnostic Criteria for Primary Osteoporosis (2000) by the Japanese Society for Bone and Mineral Research and who had between one and four fractured vertebrae located between the fourth thoracic vertebra and the fourth lumbar vertebra...
October 12, 2017: Bone
https://www.readbyqxmd.com/read/29033380/cd105-is-regulated-by-hsa-mir-1287-and-its-expression-is-inversely-correlated-with-osteopotential-in-shed
#3
Felipe Augusto André Ishiy, Roberto Dalto Fanganiello, Gerson Shigeru Kobayashi, Erika Kague, Patrícia Semedo Kuriki, Maria Rita Passos-Bueno
A more accurate understanding of the molecular mechanisms and signaling pathways underpinning human mesenchymal stem cell (MSC) plasticity and differentiation properties is pivotal for accomplishing solid and diligent translation of MSC-based experimental therapeutics and clinical trials to broad clinical practice. In addition, this knowledge enables selection of MSC subpopulations with increased differentiation potential and/or use of exogenous factors to boost this potential. Here, we report that CD105 (ENG) is a predictive biomarker of osteogenic potential in two types of MSCs: stem cells from human exfoliated deciduous teeth (SHED) and human adipose-derived stem cells (hASC)...
October 12, 2017: Bone
https://www.readbyqxmd.com/read/29032175/the-effect-of-raloxifene-on-bone-marrow-adipose-tissue-and-bone-turnover-in-postmenopausal-women-with-osteoporosis
#4
Kerensa Beekman, Annegreet G Veldhuis-Vlug, Martin den Heijer, Mario Maas, Ania M Oleksik, Michael W Tanck, Susan M Ott, Rob J Van't Hof, Paul Lips, Peter H Bisschop, Nathalie Bravenboer
In patients with postmenopausal osteoporosis low bone volume is associated with high bone marrow adipose tissue (MAT). Moreover, high MAT is associated with increased fracture risk. This suggests an interaction between MAT and bone turnover, however literature remains equivocal. Estrogen treatment decreases MAT, but the effect of raloxifene, a selective estrogen receptor modulator (SERM) registered for treatment of postmenopausal osteoporosis, on MAT is not known. The aim of this study is 1] to determine the effect of raloxifene on MAT and 2] to determine the relationship between MAT and bone turnover in patients with osteoporosis...
October 11, 2017: Bone
https://www.readbyqxmd.com/read/29032174/c-ebp%C3%AE-and-pu-1-exhibit-different-responses-to-rank-signaling-for-osteoclastogenesis
#5
Joel Jules, Wei Chen, Yi-Ping Li
The transcription factors C/EBPα and PU.1 are upregulated by RANKL through activation of its receptor RANK during osteoclastogenesis and are critical for osteoclast differentiation. Herein we investigated the mechanisms underlying how C/EBPα and PU.1 regulate osteoclast differentiation in response to RANK signaling. We showed that C/EBPα or PU.1 overexpression could initiate osteoclastogenesis and upregulate the expressions of the osteoclast genes encoding the nuclear factor of activated T-cells, C1, cathepsin K, and tartrate-resistant acid phosphatase independently of RANKL...
October 11, 2017: Bone
https://www.readbyqxmd.com/read/29032173/the-reduced-osteogenic-potential-of-nf1-deficient-osteoprogenitors-is-egfr-independent
#6
S E Tahaei, G Couasnay, Y Ma, N Paria, J Gu, B F Lemoine, X Wang, J J Rios, F Elefteriou
Neurofibromatosis type 1 (NF1) is a common genetic disorder caused by mutations in the NF1 gene. Recalcitrant bone healing following fracture (i.e. pseudarthrosis) is one of the most problematic skeletal complications associated with NF1. The etiology of this condition is still unclear; thus, pharmacological options for clinical management are limited. Multiple studies have shown the reduced osteogenic potential of Nf1-deficient osteoprogenitors. A recent transcriptome profiling investigation revealed that EREG and EGFR, encoding epiregulin and its receptor Epidermal Growth Factor Receptor 1, respectively, were among the top over-expressed genes in cells of the NF1 pseudarthrosis site...
October 11, 2017: Bone
https://www.readbyqxmd.com/read/29030232/a-novel-bone-targeting-delivery-system-carrying-phytomolecule-icaritin-for-prevention-of-steroid-associated-osteonecrosis-in-rats
#7
Shihui Chen, Lizhen Zheng, Jiayong Zhang, Heng Wu, Nan Wang, Wenxue Tong, Jiankun Xu, Le Huang, Yifeng Zhang, Zhijun Yang, Ge Lin, Xinluan Wang, Ling Qin
One of the effective strategies for prevention of steroid-associated osteonecrosis (SAON) is to inhibit bone resorption and fat formation and promote bone formation at osteonecrotic sensitive skeletal sites. We identified a novel phytomolecule that showed positive effects on osteogenesis, anti-bone resorption and anti-adipogenesis in vitro and also developed a bone-targeting delivery system (BTDS) for in vivo experimental study. The study investigated if our innovative synthesized BTDS carrying this phytomolecule would be able to effectively prevent SAON in a rat model...
October 10, 2017: Bone
https://www.readbyqxmd.com/read/29030231/als-associated-mutation-sod1-g93a-leads-to-abnormal-mitochondrial-dynamics-in-osteocytes
#8
Huan Wang, Jianxun Yi, Xuejun Li, Yajuan Xiao, Kamal Dhakal, Jingsong Zhou
While the death of motor neuron is a pathological hallmark of amyotrophic lateral sclerosis (ALS), defects in other cell types or organs may also actively contribute to ALS disease progression. ALS patients experience progressive skeletal muscle wasting that may not only exacerbate neuronal degeneration, but likely has a significant impact on bone function. In our previous published study, we have discovered severe bone loss in an ALS mouse model with overexpression of ALS-associated mutation SOD1(G93A) (G93A)...
October 10, 2017: Bone
https://www.readbyqxmd.com/read/29017892/muscle-functions-and-bone-strength-are-impaired-in-adolescents-with-type-1-diabetes
#9
Klara Maratova, Ondrej Soucek, Jana Matyskova, Zdenek Hlavka, Lenka Petruzelkova, Barbora Obermannova, Stepanka Pruhova, Stanislava Kolouskova, Zdenek Sumnik
BACKGROUND: Sarcopenia and osteoporosis are among the late complications of type 1 diabetes (T1D) in adults. Whether and to what extent musculoskeletal impairment is present in childhood and adolescence has yet to be determined. The aim of this study was to assess volumetric bone mineral density (BMD) and dynamic muscle function in adolescents with T1D and to assess the clinical and biochemical predictors of their musculoskeletal system. METHODS: Ninety-five children and adolescents (59 boys and 36 girls, mean age 16...
October 7, 2017: Bone
https://www.readbyqxmd.com/read/28993177/dkk1-and-sclerostin-are-early-markers-of-relapse-in-multiple-myeloma
#10
Charlotte Mabille, Adeline Ruyssen Witrand, Yannick Degboe, Isabelle Gennero, Herve Avet Loiseau, Murielle Roussel, Benjamin Hebraud, Delphine Nigon, Michel Attal, Michel Laroche
Recent studies have shown that Dickkopf-related protein (DKK1) and sclerostin decrease when a complete response (CR) is obtained after chemotherapy in myeloma multiple (MM). To study variations in DKK1, sclerostin and P1NP in patients treated for MM, between complete response (CR) and relapse, we carried out a prospective study ancillary to the IFM 2009 protocol (IFM). The aim of IFM was to compare progression-free survival between patients treated with chemotherapy with or without transplantation. We selected 69 patients who reached CR and relapsed...
October 6, 2017: Bone
https://www.readbyqxmd.com/read/28987286/determination-of-sex-differences-of-human-cadaveric-mandibular-condyles-using-statistical-shape-and-trait-modeling
#11
Jessica S Coogan, Do-Gyoon Kim, Todd L Bredbenner, Daniel P Nicolella
The objective of this study was to elucidate sex differences in the anatomy of human temporomandibular joint mandibular condyles using a statistical shape and trait model (SSTM). Mandibles were obtained from 16 human cadavers (79±13years). The condyles were scanned using micro-computed tomography with 27μm resolution. An image processing algorithm was used to segment the bone, determine the border of the entire mandibular condyle and trabecular bone compartments, and create triangulated meshes of the compartments...
October 4, 2017: Bone
https://www.readbyqxmd.com/read/28987285/heterotopic-ossification-and-the-elucidation-of-pathologic-differentiation
#12
David Cholok, Michael T Chung, Kavitha Ranganathan, Serra Ucer, Devaveena Day, Thomas A Davis, Yuji Mishina, Benjamin Levi
Tissue regeneration following acute or persistent inflammation can manifest a spectrum of phenotypes ranging from the adaptive to the pathologic. Heterotopic Ossification (HO), the endochondral formation of bone within soft-tissue structures following severe injury serves as a prominent example of pathologic differentiation; and remains a persistent clinical issue incurring significant patient morbidity and expense to adequately diagnose and treat. The pathogenesis of HO provides an intriguing opportunity to better characterize the cellular and cell-signaling contributors to aberrant differentiation...
October 4, 2017: Bone
https://www.readbyqxmd.com/read/28986143/validation-of-calcaneus-trabecular-microstructure-measurements-by-hr-pqct
#13
Louis M Metcalf, Enrico Dall'Ara, Margaret A Paggiosi, John R Rochester, Nicolas Vilayphiou, Graham J Kemp, Eugene V McCloskey
OBJECTIVE: Assessment of calcaneus microstructure using high-resolution peripheral quantitative computed tomography (HR-pQCT) might be used to improve fracture risk predictions or to assess responses to pharmacological and physical interventions. To develop a standard clinical protocol for the calcaneus, we validated calcaneus trabecular microstructure measured by HR-pQCT against 'gold-standard' micro-CT measurements. METHODS: Ten human cadaveric feet were scanned in situ using HR-pQCT (isotropic 82μm voxel size) at 100, 150 and 200ms integration times, and at 100ms integration time following removal of the calcaneus from the foot (ex vivo)...
October 3, 2017: Bone
https://www.readbyqxmd.com/read/28982589/identification-of-a-novel-loss-of-function-phex-mutation-ala720ser-in-a-sporadic-case-of-adult-onset-hypophosphatemic-osteomalacia
#14
Katarzyna Goljanek-Whysall, Andreas Tridimas, Rachel McCormick, Nicki-Jayne Russell, Melissa Sloman, Alan Sorani, William D Fraser, Fadil M Hannan
Adults presenting with sporadic hypophosphatemia and elevations in circulating fibroblast growth factor-23 (FGF23) concentrations are usually investigated for an acquired disorder of FGF23 excess such as tumor induced osteomalacia (TIO). However, in some cases the underlying tumor is not detected, and such patients may harbor other causes of FGF23 excess. Indeed, coding-region and 3'UTR mutations of phosphate-regulating neutral endopeptidase (PHEX), which encodes a cell-surface protein that regulates circulating FGF23 concentrations, can lead to alterations in phosphate homeostasis, which are not detected until adulthood...
October 3, 2017: Bone
https://www.readbyqxmd.com/read/28982588/low-1-level-mechanical-vibration-improves-bone-microstructure-tissue-mechanical-properties-and-porous-titanium-implant-osseointegration-by-promoting-anabolic-response-in-type-1-diabetic-rabbits
#15
Da Jing, Zedong Yan, Jing Cai, Shichao Tong, Xiaokang Li, Zheng Guo, Erping Luo
Type 1 diabetes mellitus (T1DM) is associated with reduced bone mass, increased fracture risk, and impaired bone defect regeneration potential. These skeletal complications are becoming important clinical challenges due to the rapidly increasing T1DM population, which necessitates developing effective treatment for T1DM-associated osteopenia/osteoporosis and bone trauma. This study aims to investigate the effects of whole-body vibration (WBV), an easy and non-invasive biophysical method, on bone microstructure, tissue-level mechanical properties and porous titanium (pTi) osseointegration in alloxan-diabetic rabbits...
October 2, 2017: Bone
https://www.readbyqxmd.com/read/28978416/effects-of-single-or-combination-therapy-of-teriparatide-and-anti-rankl-monoclonal-antibody-on-bone-defect-regeneration-in-mice
#16
Kazuma Kitaguchi, Masafumi Kashii, Kosuke Ebina, Takashi Kaito, Rintaro Okada, Takahiro Makino, Takaaki Noguchi, Takuya Ishimoto, Takayoshi Nakano, Hideki Yoshikawa
OBJECTIVE: The purpose of this study is to investigate the effects of a single or combination therapy of teriparatide (TPTD) and anti-RANKL Ab (anti-murine receptor activator of nuclear factor κB ligand monoclonal antibody) on the regeneration of both cancellous and cortical bone. METHODS: Nine-week-old mice underwent bone defect surgery on the left femoral metaphysis (cancellous-bone healing model) and right femoral mid-diaphysis (cortical-bone healing model)...
October 1, 2017: Bone
https://www.readbyqxmd.com/read/28974461/bone-replaces-articular-cartilage-in-the-rat-knee-joint-after-prolonged-immobilization
#17
T M Campbell, K Reilly, O Laneuville, H Uhthoff, G Trudel
BACKGROUND: Lost joint range of motion (ROM) is common in chronic osteoarthritis, alters regional weight-bearing across the articular surfaces, and contributes to loss of cartilage and bone alterations. Limited data exist on the regional effects on joints subjected to chronic losses of ROM. OBJECTIVE: To characterize the regional replacement by bone as part of articular cartilage degeneration after prolonged immobilization. METHODS: Eleven rat knees were rigidly-immobilized in flexion for 32weeks with contralateral and sham-operated (n=6) knees as controls...
September 30, 2017: Bone
https://www.readbyqxmd.com/read/28965919/pulsed-electromagnetic-fields-inhibit-human-osteoclast-formation-and-gene-expression-via-osteoblasts
#18
Zhiming He, Nagarajan Selvamurugan, Johanna Warshaw, Nicola C Partridge
Pulsed electromagnetic fields (PEMFs) can be effective in promoting the healing of delayed union or nonunion fractures. We previously reported that PEMF (Spinal-Stim® by Orthofix, Inc., Lewisville, TX) stimulated proliferation, differentiation and mineralization of rat calvarial osteoblastic cells in culture. In the present work we investigated the effects of PEMF (Physio-Stim® by Orthofix, Inc., Lewisville, TX) on human bone marrow macrophages (hBMMs) differentiated to osteoclasts. PEMF had striking inhibitory effects on formation of osteoclasts from hBMMs from both younger and older women...
September 28, 2017: Bone
https://www.readbyqxmd.com/read/28963080/cartilage-derived-retinoic-acid-sensitive-protein-cd-rap-a-stage-specific-biomarker-of-heterotopic-endochondral-ossification-heo-in-fibrodysplasia-ossificans-progressiva-fop
#19
Carter M Lindborg, Tracy A Brennan, Haitao Wang, Frederick S Kaplan, Robert J Pignolo
BACKGROUND: Genesis of a cartilaginous scaffold is an obligate precursor to bone formation in heterotopic endochondral ossification (HEO). We tested the hypothesis that cartilage-derived retinoic acid-sensitive protein (CD-RAP) can serve as a plasma biomarker for the pre-osseous cartilaginous stage of HEO. Palovarotene, a retinoic acid receptor-gamma (RARγ) agonist, has been proposed as a possible treatment for fibrodysplasia ossificans progressiva (FOP) and is a potent inhibitor of HEO in mouse models...
September 28, 2017: Bone
https://www.readbyqxmd.com/read/28963079/comparisons-of-tbs-and-lumbar-spine-bmd-in-the-associations-with-vertebral-fractures-according-to-the-t-scores-a-cross-sectional-observation
#20
Jie-Eun Lee, Kyoung Min Kim, Lee-Kyung Kim, Kyong Young Kim, Tae Jung Oh, Jae Hoon Moon, Sung Hee Choi, Soo Lim, Sang Wan Kim, Chan Soo Shin, Hak Chul Jang
Trabecular bone score (TBS) is a parameter of bone quality that has been shown to be related to vertebral fractures. This study aimed to analyze the difference in discriminatory power of TBS for vertebral fractures according to the bone mineral density (BMD) T-score. Areal BMD at the lumbar spine (LS, L1-L4), femur neck (FN) and total hip were assessed using dual x-ray absorptiometry (Discovery W, Hologic, Bedford, MA) in 929 women aged 50years or older. TBS was analyzed using iNsight software (Med-Imaps, Pessac, France)...
September 28, 2017: Bone
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