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https://www.readbyqxmd.com/read/28938491/inhibition-of-fgfr-signaling-partially-rescues-hypophosphatemic-rickets-in-hmwfgf2-tg-male-mice
#1
Liping Xiao, Erxia Du, Collin Homer-Bouthiette, Marja M Hurley
Transgenic mice harboring high molecular weight FGF2 isoforms (HMWTg) in osteoblast lineage cells phenocopy human X-linked hypophosphatemic rickets (XLH) and Hyp murine model of XLH demonstrating increased FGF23/FGF receptor signaling and hypophosphatemic rickets. Since HMWFGF2 was upregulated in bones of Hyp mice and abnormal FGF receptor (FGFR) signaling is important in XLH, HMWTg mice were used to examine the effect of the FGFR inhibitor NVP-BGJ398, now in clinical trials for cancer therapy, on hypophosphatemic rickets...
August 14, 2017: Endocrinology
https://www.readbyqxmd.com/read/28938444/lack-of-lrp5-signaling-in-osteoblasts-sensitizes-male-mice-to-diet-induced-disturbances-in-glucose-metabolism
#2
Soohyun P Kim, Julie L Frey, Zhu Li, Brian C Goh, Ryan C Riddle
Wnt signaling through the Lrp5 co-receptor regulates osteoblast maturation, matrix mineralization, and intermediary metabolism. In the mature osteoblast, signals downstream of Lrp5 are required for normal long-chain fatty acid β-oxidation and mice rendered deficient for this co-receptor in osteoblasts and osteocytes accumulate body fat with elevated serum lipid levels, but retain normal insulin sensitivity. In this study, we challenged Lrp5 mutant mice with a high fat diet to determine if they are more susceptible to diet-induced disturbances in glucose homeostasis...
September 11, 2017: Endocrinology
https://www.readbyqxmd.com/read/28938420/bone-structural-characteristics-and-response-to-bisphosphonate-treatment-in-children-with-hajdu-cheney-syndrome
#3
Sophia Sakka, Rachel I Gafni, Justin H Davies, Bart Clarke, Peter Tebben, Mark Samuels, Vrinda Saraff, Klaus Klaushofer, Nadja Fratzl-Zelman, Paul Roschger, Frank Rauch, Wolfgang Högler
Context: Hajdu-Cheney syndrome (HJCYS) is a rare, multisystem, bone disease caused by heterozygous mutations in the NOTCH2 gene. Histomorphometric and bone ultrastructural analyses in children have not been reported and sparse evidence exists on response to bisphosphonate (BP) therapy. Objective: To investigate clinical and bone histomorphometric characteristics, bone matrix mineralization and the response of bone geometry and density to BP therapy. Patients: Five children with HJCYS (3 males) aged between 6...
September 8, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28938396/transgenic-expression-of-the-vitamin-d-receptor-restricted-to-the-ileum-cecum-and-colon-of-vitamin-d-receptor-knockout-mice-rescues-vitamin-d-receptor-dependent-rickets
#4
Puneet Dhawan, Vaishali Veldurthy, Ghassan Yehia, Connie Hsaio, Angela Porta, Ki-In Kim, Nishant Patel, Liesbet Lieben, Lieve Verlinden, Geert Carmeliet, Sylvia Christakos
Although the intestine plays the major role in 1,25-dihydroxyvitamin D3 (1,25(OH)2D3) action on calcium homeostasis, the mechanisms involved remain incompletely understood. The established model of 1,25(OH)2D3 regulated intestinal calcium absorption postulates a critical role for the duodenum. However it is the distal intestine where 70 -80% of the ingested calcium is absorbed. In order to test directly the role of 1,25(OH)2D3 and the vitamin D receptor (VDR) in the distal intestine, 3 independent knockout (KO) /transgenic (TG) lines expressing VDR exclusively in the ileum, cecum and colon were generated by breeding VDR KO mice with TG mice expressing human (h)VDR under the control of the 9...
September 11, 2017: Endocrinology
https://www.readbyqxmd.com/read/28938290/body-composition-changes-in-severely-burned-children-during-icu-hospitalization
#5
Janos Cambiaso-Daniel, Ioannis Malagaris, Eric Rivas, Gabriel Hundeshagen, Charles D Voigt, Elizabeth Blears, Ron P Mlcak, David N Herndon, Celeste C Finnerty, Oscar E Suman
OBJECTIVES: Prolonged hospitalization due to burn injury results in physical inactivity and muscle weakness. However, how these changes are distributed among body parts is unknown. The aim of this study was to evaluate the degree of body composition changes in different anatomical regions during ICU hospitalization. DESIGN: Retrospective chart review. SETTING: Children's burn hospital. PATIENTS: Twenty-four severely burned children admitted to our institution between 2000 and 2015...
September 21, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28938197/a-collagen-membrane-containing-osteogenic-protein-1-facilitates-bone-regeneration-in-a-rat-mandibular-bone-defect
#6
Manami Ozaki, Tadahiro Takayama, Takanobu Yamamoto, Yasumasa Ozawa, Mayu Nagao, Natsuko Tanabe, Akira Nakajima, Naoto Suzuki, Masao Maeno, Seiichi Yamano, Shuichi Sato
OBJECTIVES: Osteogenic protein-1 (OP-1) has shown osteoinductive activities and is useful for clinical treatments, including bone regeneration. Regenerative procedures using a bioabsorbable collagen membrane (BCM) are well established in periodontal and implant dentistry. We evaluated the subsequent effects of the BCM in combination with OP-1 on bone regeneration in a rat mandibular circular critical-sized bone defect in vivo. DESIGN: We used 8 rats that received surgery in both sides of the mandible, and created the total 16 defects which were divided into 4 groups: Group 1; no treatment, as a control, Group 2; BCM alone, Group 3; BCM containing low dose 0...
September 8, 2017: Archives of Oral Biology
https://www.readbyqxmd.com/read/28938035/guided-bone-regeneration-with-collagen-membranes-and-particulate-graft-materials-a-systematic-review-and-meta-analysis
#7
Bastian Wessing, Stefan Lettner, Werner Zechner
PURPOSE: The aim of this meta-analysis was to evaluate different methods for guided bone regeneration using collagen membranes and particulate grafting materials in implant dentistry. MATERIALS AND METHODS: An electronic database search and hand search were performed for all relevant articles dealing with guided bone regeneration in implant dentistry published between 1980 and 2014. Only randomized clinical trials and prospective controlled studies were included...
September 22, 2017: International Journal of Oral & Maxillofacial Implants
https://www.readbyqxmd.com/read/28937541/body-composition-predicts-growth-in-infants-and-toddlers-with-chronic-liver-disease
#8
Erika F Hurtado-López, Edgar M Vásquez-Garibay, Xóchitl Trujillo, Alfredo Larrosa-Haro
This cross-sectional study was conducted on 15 infants and toddlers with chronic liver disease (CLD) to validate arm anthropometry as an accurate measure of body composition (BC) compared to dual-energy X-ray absorptiometry (DXA) and to predict growth from BC.The z-score means of the anthropometric indicators were < -2SD, except for body fat index and subscapular skinfold which were between -2 and +2 SD. Fat mass was predicted by arm adiposity indicators and fat-free mass by arm muscle area. Bone mineral content explained 87% of variation in length...
September 20, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28937054/the-importance-of-bone-biopsy-in-chronic-kidney-disease-mineral-bone-disorders
#9
REVIEW
Periklis Dousdampanis, Kostantina Trigka
Renal osteodystrophy (ROD) is not a uniform bone disease; it is a heterogeneous group of metabolic bone diseases due to chronic kidney disease (CKD). The traditional term of ROD does not accurately include the wide spectrum of "CKD-mineral and bone disorder" (CKD-MBD) and has been restricted to define the several specific histologic disturbances of bone disease associated with CKD. Circulating parathyroid hormone (PTH) and total alkaline phosphatase levels do not always reflect bone turnover in CKD-MBD, whereas bone biopsy provides precise information regarding bone pathology...
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28936606/denosumab-significantly-improves-bone-mineral-density-with-or-without-bisphosphonate-pre-treatment-in-osteoporosis-with-rheumatoid-arthritis-denosumab-improves-bone-mineral-density-in-osteoporosis-with-rheumatoid-arthritis
#10
Yukio Nakamura, Takako Suzuki, Hiroyuki Kato
Bone mineral density (BMD) sometimes cannot be improved by long-term bisphosphonate (BP) therapy in osteoporosis (OP) patients with rheumatoid arthritis (RA). This study showed that BMD significantly increased after denosumab treatment in patients with long-term BP pre-treatment as much as in treatment-naïve patients. Thus, denosumab can be a strong OP treatment option for long-term BP pre-treated RA patients. INTRODUCTION: The aim of this 24-month retrospective study was to evaluate differences in outcomes of denosumab with or without bisphosphonate (BP) pre-treatment in osteoporosis (OP) patients with rheumatoid arthritis (RA)...
September 21, 2017: Archives of Osteoporosis
https://www.readbyqxmd.com/read/28936199/bone-status-in-a-patient-with-insulin-like-growth-factor-1-receptor-deletion-syndrome-bone-quality-and-structure-evaluation-using-dual-energy-x-ray-absorptiometry-peripheral-quantitative-computed-tomography-and-quantitative-ultrasonography
#11
Paola Pelosi, Elisabetta Lapi, Loredana Cavalli, Alberto Verrotti, Marilena Pantaleo, Maurizio de Martino, Stefano Stagi
Haploinsufficiency of the insulin-like growth factor (IGF)-1 receptor (IGF1R) gene is a rare, probably under-diagnosed, cause of short stature. However, the effects of IGF1R haploinsufficiency on glucose metabolism, bone status, and metabolism have rarely been investigated. We report the case of a patient referred to our center at the age of 18 months for short stature, failure to thrive, and Silver-Russell-like phenotype. Genetic analysis did not show hypomethylation of the 11p15.5 region or uniparental disomy of chromosome 7...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28935593/factors-affecting-peri-implant-fracture-following-locking-plate-for-osteoporotic-distal-femur-fractures
#12
Ki-Chul Park, Seung-Jae Lim, Young Sik Song, Kyu-Tae Hwang
INTRODUCTION: The purpose of this study is to evaluate the outcomes and to analyze the risk factors for the occurrence of peri-implant fracture after treatment of osteoporotic distal femoral fracturesusing a locking plate. HYPOTHESIS: Risk factors affecting peri-implant fracture exist after locking plate fixation in osteoporotic distal femur fracture. MATERIALS AND METHODS: Eighty nine(88 patients) with osteoporotic distal femoral fractures were evaluated between January2006 and January2014...
September 18, 2017: Orthopaedics & Traumatology, Surgery & Research: OTSR
https://www.readbyqxmd.com/read/28935141/antioxidative-peptide-from-milk-exhibits-antiosteopenic-effects-through-inhibition-of-oxidative-damage-and-bone-resorbing-cytokines-in-ovariectomized-rats
#13
Sanusi Bello Mada, Srinu Reddi, Naveen Kumar, Rohit Kumar, Suman Kapila, Rajeev Kapila, Ritu Trivedi, Anirudha Karvande, Naseer Ahmad
OBJECTIVES: Oxidative stress has been implicated as a crucial pathogenic factor in the development of postmenopausal osteoporosis. Milk-derived antioxidative peptides are gaining much attention toward the development of prodrugs for alleviating several human diseases, including osteoporosis. The aim of the present study was to determine whether antioxidant peptides are good candidates for alleviating postmenopausal osteoporosis. METHODS: In the present study, an ovariectomized (OVX) osteoporotic rat model was used to investigate the protective effects of buffalo milk casein-derived novel peptide VLPVPQK (PEP) against OVX-induced bone loss and the related mechanisms...
November 2017: Nutrition
https://www.readbyqxmd.com/read/28934396/evaluation-of-shared-genetic-aetiology-between-osteoarthritis-and-bone-mineral-density-identifies-smad3-as-a-novel-osteoarthritis-risk-locus
#14
Sophie Hackinger, Katerina Trajanoska, Unnur Styrkarsdottir, Eleni Zengini, Julia Steinberg, Graham R S Ritchie, Konstantinos Hatzikotoulas, Arthur Gilly, Evangelos Evangelou, John P Kemp, David Evans, Thorvaldur Ingvarsson, Helgi Jonsson, Unnur Thorsteinsdottir, Kari Stefansson, Andrew W McCaskie, Roger A Brooks, Jeremy M Wilkinson, Fernando Rivadeneira, Eleftheria Zeggini
Osteoarthritis (OA) is a common complex disease with high public health burden and no curative therapy. High bone mineral density (BMD) is associated with an increased risk of developing OA, suggesting a shared underlying biology. Here, we performed the first systematic overlap analysis of OA and BMD on a genome wide scale. We used summary statistics from the GEFOS consortium for lumbar spine (n = 31,800) and femoral neck (n = 32,961) BMD, and from the arcOGEN consortium for three OA phenotypes (hip, ncases=3,498; knee, ncases=3,266; hip and/or knee, ncases=7,410; ncontrols=11,009)...
October 1, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28933358/substrate-deprivation-therapy-to-reduce-glycosaminoglycan-synthesis-improves-aspects-of-neurological-and-skeletal-pathology-in-mps-i-mice
#15
Ainslie L K Derrick-Roberts, Matilda R Jackson, Carmen E Pyragius, Sharon Byers
Mucopolysaccharidosis type I (MPS I) is the most common form of the MPS group of genetic diseases. MPS I results from a deficiency in the lysosomal enzyme α-l-iduronidase, leading to accumulation of undegraded heparan and dermatan sulphate glycosaminoglycan (GAG) chains in patient cells. MPS children suffer from multiple organ failure and die in their teens to early twenties. In particular, MPS I children also suffer from profound mental retardation and skeletal disease that restricts growth and movement. Neither brain nor skeletal disease is adequately treated by current therapy approaches...
February 23, 2017: Diseases (Basel)
https://www.readbyqxmd.com/read/28933309/cigarette-smoking-is-associated-with-decreased-bone-gla-protein-bgp-levels-in-hemodialysis-patients
#16
Maria Fusaro, Maurizio Gallieni, Andrea Aghi, Giorgio Iervasi, Maria Antonietta Rizzo, Andrea Stucchi, Marianna Noale, Giovanni Tripepi, Thomas Nickolas, Nicola Veronese, Fabrizio Fabris, Sandro Giannini, Lorenzo Calò, Antonio Piccoli, Maria Cristina Mereu, Laura Cosmai, Alberto Ferraro, Fiorenza Magonara, Michela Spinello, Stefania Sella, Mario Plebani
BACKGROUND: Bone gamma-carboxyglutamic acid (Gla)-protein (BGP or osteocalcin) is a vitamin K-dependent protein involved in the regulation of bone mineralization. Smoking is a risk factor for osteoporosis. METHODS: We carried out a secondary analysis of the vitamin K Italian (VIKI) study to investigate the association between cigarette smoking and BGP levels in patients with end stage renal disease. Data were collected in 370 haemodialysis patients, 37% (136) smokers (or ex-smokers) and 63% (234) non-smokers...
September 19, 2017: Current Vascular Pharmacology
https://www.readbyqxmd.com/read/28931999/the-impact-of-closed-versus-open-kinetic-chain-exercises-on-osteoporotic-femur-neck-and-risk-of-fall-in-postmenopausal-women
#17
Ali Abd El-Monsif Thabet, Mansour Abdullah Alshehri, Omar Farouk Helal, Bassem Refaat
[Purpose] This study aimed to investigate how closed and open kinetic chain exercises differed in their impact on bone mineral density (BMD) and fall risk in postmenopausal women with osteoporosis. [Subjects and Methods] The research sample consisted of 40 postmenopausal women with osteoporosis with ages between 51 and 58 years old. They were divided at random into two groups of 20 each, respectively receiving closed and open kinetic chain exercises. These exercises were administered three times per week over a period of four sequential months...
September 2017: Journal of Physical Therapy Science
https://www.readbyqxmd.com/read/28931828/a-novel-h2s-releasing-amino-bisphosphonate-which-combines-bone-anti-catabolic-and-anabolic-functions
#18
Simona Rapposelli, Laura Gambari, Maria Digiacomo, Valentina Citi, Gina Lisignoli, Cristina Manferdini, Vincenzo Calderone, Francesco Grassi
Bisphosphonates (BPs) are the first-line treatment of bone loss resulting from various pathological conditions. Due to their high affinity to bone they have been used to develop conjugates with pro-anabolic or anti-catabolic drugs. We recently demontrated that hydrogen sulfide (H2S), promotes osteogenesis and inhibits osteoclast differentiation. Here we developed an innovative molecule, named DM-22, obtained from the combination of alendronate (AL) and the H2S-releasing moiety aryl-isothiocyanate. DM-22 and AL were assayed in vitro in the concentration range 1-33 μM for effects on viability and function of human osteoclasts (h-OCs) and mesenchymal stromal cells (h-MSCs) undergoing osteogenic differentiation...
September 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28930627/fitness-body-composition-and-vascular-health-in-adolescent-and-young-adult-survivors-of-paediatric-brain-cancer-and-cranial-radiotherapy
#19
Treya M Long, Shoshana R Rath, Tessa D Maroni, Karen E Wallman, Helen C Atkinson, Nicholas G Gottardo, Catherine H Cole, Catherine S Choong, Louise H Naylor
Background Survivors of paediatric brain cancer and/or cranial radiotherapy (CRT) are at an increased risk of developing serious comorbidities. Established risk factors for chronic disease include central obesity, endothelial abnormalities and diminished fitness. Objectives Here we characterised anthropometry, body composition, bone mineral density (BMD), heart rate (HR), blood pressure (BP), endothelial function, muscular strength and endurance and aerobic fitness in adolescent and young adult (AYA) survivors...
September 20, 2017: International Journal of Adolescent Medicine and Health
https://www.readbyqxmd.com/read/28930090/biomimetic-matrix-fabricated-by-lmp-1-gene-transduced-mc3t3-e1-cells-for-bone-regeneration
#20
Junxuan Ma, Wei Guo, Manman Gao, Baoding Huang, Qihua Qi, Zemin Ling, Yan Chen, Hao Hu, Hailian Zhou, Fei Yu, Ke Chen, Zhiyu Zhou, Deming Xiao, Xuenong Zou
Bone healing is regulated by multiple microenvironmental signals provided by the extracellular matrix (ECM). The study aimed to mimic native osteoinductive microenvironment by developing an ECM using gene-transduced cells. LIM mineralization protein-1 (LMP-1) gene was transferred to the murine pre-osteoblast cells (MC3T3-E1) using lentiviral vectors. Western blotting essay indicated that the MC3T3-E1 cells expressed an increased level of bone morphologic protein-2, -4 and-7 (BMP-2, -4 and -7) after LMP-1 gene transduction...
September 20, 2017: Biofabrication
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