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Metabolic bone disease

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https://www.readbyqxmd.com/read/28631442/osteoblast-migration-in-vertebrate-bone
#1
Antonia Thiel, Marie K Reumann, Adele Boskey, Johannes Wischmann, Rüdiger von Eisenhart-Rothe, Philipp Mayer-Kuckuk
Bone formation, for example during bone remodelling or fracture repair, requires mature osteoblasts to deposit bone with remarkable spatial precision. As osteoblast precursors derive either from circulation or resident stem cell pools, they and their progeny are required to migrate within the three-dimensional bone space and to navigate to their destination, i.e. to the site of bone formation. An understanding of this process is emerging based on in vitro and in vivo studies of several vertebrate species. Receptors on the osteoblast surface mediate cell adhesion and polarization, which induces osteoblast migration...
June 19, 2017: Biological Reviews of the Cambridge Philosophical Society
https://www.readbyqxmd.com/read/28631412/psychopathology-in-pediatric-bone-marrow-transplantation-survivors-and-their-mothers
#2
Aslı Sürer Adanir, Gülseren Taşkiran, O Alphan Küpesiz, Esin Özatalay
BACKGROUND: Bone Marrow Transplantation(BMT) is used to treat children with various hematologic, oncologic and metabolic diseases. While the treatment is lifesaving, it is also found to be related with anxiety, posttraumatic stress disorder, depression and psychosocial problems both in children and parents. METHODS: The aim of this study was to investigate psychopathology in pediatric BMT survivors and their mothers compared to the healthy controls. All children were interviewed using Kiddie Schedule for Affective Disorders and Schizophrenia for assessing the life-long psychopathology...
June 20, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28630637/evaluation-of-circulating-srage-in-osteoporosis-according-to-bmi-adipokines-and-fracture-risk-a-pilot-observational-study
#3
Emanuela Galliera, Monica Gioia Marazzi, Carmine Gazzaruso, Pietro Gallotti, Adriana Coppola, Tiziana Montalcini, Arturo Pujia, Massimiliano M Corsi Romanelli
BACKGROUND: Osteoporosis is a systemic metabolic disease based on age-dependent imbalance between the rates of bone formation and bone resorption. Recent studies on the pathogenesis of this disease identified that bone remodelling impairment, at the base of osteoporotic bone fragility, could be related to protein glycation, in association to oxidative stress. The glycation reactions lead to the generation of glycation end products (AGEs) which, in turn, accumulates into bone, where they binds to the receptor for AGE (RAGE)...
2017: Immunity & Ageing: I & A
https://www.readbyqxmd.com/read/28630322/tcte1-is-a-conserved-component-of-the-dynein-regulatory-complex-and-is-required-for-motility-and-metabolism-in-mouse-spermatozoa
#4
Julio M Castaneda, Rong Hua, Haruhiko Miyata, Asami Oji, Yueshuai Guo, Yiwei Cheng, Tao Zhou, Xuejiang Guo, Yiqiang Cui, Bin Shen, Zibin Wang, Zhibin Hu, Zuomin Zhou, Jiahao Sha, Renata Prunskaite-Hyyrylainen, Zhifeng Yu, Ramiro Ramirez-Solis, Masahito Ikawa, Martin M Matzuk, Mingxi Liu
Flagella and cilia are critical cellular organelles that provide a means for cells to sense and progress through their environment. The central component of flagella and cilia is the axoneme, which comprises the "9+2" microtubule arrangement, dynein arms, radial spokes, and the nexin-dynein regulatory complex (N-DRC). Failure to properly assemble components of the axoneme leads to defective flagella and in humans leads to a collection of diseases referred to as ciliopathies. Ciliopathies can manifest as severe syndromic diseases that affect lung and kidney function, central nervous system development, bone formation, visceral organ organization, and reproduction...
June 19, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28629885/-prevalence-of-osteoporosis-estimation-of-probability-of-fracture-and-bone-metabolism-study-in-patients-with-newly-diagnosed-prostate-cancer-in-the-health-area-of-lugo
#5
Jonatan Miguel-Carrera, Carlos García-Porrua, Francisco Javier de Toro Santos, Jose Antonio Picallo-Sánchez
OBJECTIVE: To study the prevalence of osteoporosis and fracture probability in patients diagnosed with prostate cancer. DESIGN: Observational descriptive transversal study. SITE: Study performed from Primary Care of Lugo in collaboration with Rheumatology and Urology Services of our referral hospital. PARTICIPANTS: Patients diagnosed with prostate cancer without bone metastatic disease from January to December 2012. MAIN MEASUREMENTS: Epidemiologic, clinical, laboratory and densitometric variables involved in osteoporosis were collected...
June 16, 2017: Atencion Primaria
https://www.readbyqxmd.com/read/28628374/effects-of-a-multispecies-probiotic-supplement-on-bone-health-in-osteopenic-postmenopausal-women-a-randomized-double-blind-controlled-trial
#6
Sadegh Jafarnejad, Kurosh Djafarian, Mohammad Reza Fazeli, Mir Saeed Yekaninejad, Abdolrahman Rostamian, Seyed Ali Keshavarz
OBJECTIVE: The development of alternative approaches to prevent and/or treat osteoporosis, as a chronic progressive bone disease, is being considered currently. Among dietary supplements, probiotics may have favorable effects on bone metabolism. Therefore, the aim of this study was to evaluate the effects of a multispecies probiotic supplementation on bone biomarkers and bone density in osteopenic postmenopausal women. METHODS: This randomized double-blind placebo-controlled clinical trial was performed on 50 patients with osteopenia aged 50-72 years...
June 19, 2017: Journal of the American College of Nutrition
https://www.readbyqxmd.com/read/28626366/current-developments-in-mobilization-of-hematopoietic-stem-and-progenitor-cells-and-their-interaction-with-niches-in-bone-marrow
#7
REVIEW
Rudolf Richter, Wolfgang Forssmann, Reinhard Henschler
The clinical application of hematopoietic stem and progenitor cells (HSPCs) has evolved from a highly experimental stage in the 1980s to a currently clinically established treatment for more than 20,000 patients annually who suffer from hematological malignancies and other severe diseases. Studies in numerous murine models have demonstrated that HSPCs reside in distinct niches within the bone marrow environment. Whereas transplanted HSPCs travel through the bloodstream and home to sites of hematopoiesis, HSPCs can be mobilized from these niches into the blood either physiologically or induced by pharmaceutical drugs...
June 2017: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/28625128/the-wnt-%C3%AE-catenin-signalling-pathway-inhibitor-sclerostin-is-a-biomarker-for-early-atherosclerosis-in-obesity
#8
Djordje S Popovic, Milena Mitrovic, Dragana Tomic-Naglic, Tijana Icin, Ivana Bajkin, Bojan Vukovic, Damir Benc, Zeljko Zivanovic, Branka Kovacev-Zavisic, Edita Stokic
BACKGROUND: Sclerostin is an inhibitor of the wingless-type mouse mammary tumor virus integration site family/β-catenin signalling pathway (WβcSP), which plays an important role in bone metabolism and in vascular biology. It could act protective regarding atherosclerosis development through its effect on WβcSP in vascular cells. Nevertheless, results of studies analyzing association between circulating sclerostin level (CSL) and atherosclerotic diseases (AD) are showing conflicting results...
June 18, 2017: Current Neurovascular Research
https://www.readbyqxmd.com/read/28623449/differential-effects-of-thiopurine-methyltransferase-tpmt-and-multidrug-resistance-associated-protein-gene-4-mrp4-on-mercaptopurine-toxicity
#9
Chengcheng Liu, Laura J Janke, Jun J Yang, William E Evans, John D Schuetz, Mary V Relling
PURPOSE: Mercaptopurine plays a pivotal role in treatment of acute lymphoblastic leukemia (ALL) and autoimmune diseases, and inter-individual variability in mercaptopurine tolerance can influence treatment outcome. Thiopurine methyltransferase (TPMT) and multi-drug resistant Protein 4 (MRP4) have both been associated with mercaptopurine toxicity in clinical studies, but their relative contributions remain unclear. METHODS: We studied the metabolism of and tolerance to mercaptopurine in murine knockout models of Tpmt, Mrp4, and both genes simultaneously...
June 16, 2017: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/28619127/bone-mineral-disturbances-in-patients-with-chronic-kidney-disease-stage-5-not-yet-on-dialysis
#10
Andreja Marn Pernat, Matej Zrimšek
AIMS: This retrospective study evaluates the success of a treatment strategy for secondary hyperparathyroidism in our cohort of patients with chronic kidney disease stage 5 who were not yet on dialysis. MATERIALS AND METHODS: 81 predialysis patients from the outpatient clinic of the Department of Nephrology, University Medical Center Ljubljana were reviewed. We focused on serum markers for bone mineral metabolism including intact parathyroid hormone (PTH), phosphate, corrected calcium, and the usage of phosphate-binding agents and vitamin D analogs...
June 16, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28618652/cross-talk-of-microrna-and-hydrogen-sulfide-a-novel-therapeutic-approach-for-bone-diseases
#11
REVIEW
Yuankun Zhai, Suresh C Tyagi, Neetu Tyagi
Bone homeostasis requires a balance between the bone formation of osteoblasts and bone resorption of osteoclasts to maintain ideal bone mass and bone quality. An imbalance in bone remodeling processes results in bone metabolic disorders such as osteoporosis. Hydrogen sulfide (H2S), a gasotransmitter, has attracted the focus of many researchers due to its multiple physiological functions. It has been implicated in anti-inflammatory, vasodilatory, angiogenic, cytoprotective, anti-oxidative and anti-apoptotic mechanisms...
June 10, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28617979/the-turner-syndrome-life-course-project-karyotype-phenotype-analyses-across-the-lifespan
#12
Antoinette Cameron-Pimblett, Clementina La Rosa, Thomas F J King, Melanie C Davies, Gerard S Conway
INTRODUCTION: Turner Syndrome is associated with a variety of morbidities affecting nearly every body system, some of which increase in prevalence in adult life. The severity of clinical features in TS is roughly in parallel with the magnitude of the deficit of X chromosome material. The aim of this study was to extend the established karyotype phenotype relationships using data from a large adult cohort. MATERIALS & METHODS: Karyotypes were available in 656 (78...
June 15, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28615947/treatment-of-secondary-hyperparathyroidism-the-clinical-utility-of-etelcalcetide
#13
REVIEW
Mario Cozzolino, Andrea Galassi, Ferruccio Conte, Michela Mangano, Luca Di Lullo, Antonio Bellasi
Secondary hyperparathyroidism (SHPT), a very frequent, severe, and worsening complication of chronic kidney disease, is characterized by high serum parathyroid hormone (PTH), parathyroid gland hyperplasia, and disturbances in mineral metabolism. Clinically, SHPT shows renal osteodystrophy, vascular calcification, cardiovascular damage, and fatal outcome. Calcium-sensing receptor (CaSR) is the main physiological regulator of PTH secretion; its activation by calcium rapidly inhibits PTH. Another important player in regulating mineral metabolism is vitamin D receptor (VDR), which is under the influence of vitamin D and influences the intestinal absorption of calcium and phosphate, PTH gene expression, and bone calcium mobilization...
2017: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/28615507/genetic-evaluations-of-chinese-patients-with-odontohypophosphatasia-resulting-from-heterozygosity-for-mutations-in-the-tissue-non-specific-alkaline-phosphatase-gene
#14
Jia Wan, Li Zhang, Tang Liu, Yewei Wang
BACKGROUND: Hypophosphatasia is a rare heritable metabolic disorder characterized by defective bone and tooth mineralization accompanied by a deficiency of tissue-non-specific (liver/bone/kidney) isoenzyme of alkaline phosphatase activity, caused by a number of loss-of-function mutations in the alkaline phosphatase liver type gene. We seek to explore the clinical manifestations and identify the mutations associated with the disease in a Chinese odonto- hypophosphatasia family. RESULTS: The proband and his younger brother affected with premature loss of primary teeth at their 2-year-old...
May 23, 2017: Oncotarget
https://www.readbyqxmd.com/read/28614912/peroxisome-proliferator-activated-receptor-alpha-facilitates-osteogenic-differentiation-in-mc3t3-e1-cells-via-the-sirtuin-1-dependent-signaling-pathway
#15
Kai Gong, Bo Qu, Cairu Wang, Jingsong Zhou, Dongfa Liao, Wei Zheng, Xianming Pan
Type 2 diabetes mellitus (T2DM) is a chronic, metabolic disease characterized by a lack of insulin and high glucose levels. T2DM can cause bone loss and fracture, thus leading to diabetic osteoporosis. Promoting osteogenic differentiation of osteoblasts may effectively treat diabetic osteoporosis. We previously reported that Sirtuin 1 (Sirt1), a NAD+-dependent deacetylase, promotes osteogenic differentiation through downregulation of peroxisome proliferator-activated receptor (PPAR) gamma. We also found that miR-132 regulates osteogenic differentiation by downregulating Sirt1 in a PPARbeta/δ- dependent manner...
June 14, 2017: Molecules and Cells
https://www.readbyqxmd.com/read/28612835/coexisting-variants-in-ostm1-and-maneal-cause-a-complex-neurodegenerative-disorder-with-nbia-like-brain-abnormalities
#16
Diran Herebian, Bader Alhaddad, Annette Seibt, Thomas Schwarzmayr, Katharina Danhauser, Dirk Klee, Stefani Harmsen, Thomas Meitinger, Tim M Strom, Ansgar Schulz, Ertan Mayatepek, Tobias B Haack, Felix Distelmaier
Coexistence of different hereditary diseases is a known phenomenon in populations with a high consanguinity rate. The resulting clinical phenotypes are extremely challenging for physicians involved in the care of these patients. Here we describe a 6-year-old boy with co-occurrence of a homozygous splice defect in OSTM1, causing infantile malignant osteopetrosis, and a loss-of-function variant in MANEAL, which has not been associated with human disease so far. The child suffered from severe infantile-onset neurodegeneration that could not be stopped by bone marrow transplantation...
June 14, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28606849/genetically-modified-mesenchymal-stem-stromal-cells-transfected-with-adiponectin-gene-can-stably-secrete-adiponectin
#17
Md Murad Hossain, Malliga Raman Murali, Tunku Kamarul
AIMS: Mesenchymal stem/stromal cells (MSCs) hold promises for the treatment of diverse diseases and regeneration of injured tissues. Genetic modification of MSCs through gene delivery might enhance their therapeutic potential. Adiponectin has been appeared as a potential biomarker for predicting various diseases. Plasma adiponectin levels are negatively correlated with various metabolic and vascular diseases and supplementation of exogenous adiponectin ameliorates the diseases. This study aims to develop adiponectin secreting genetically modified MSCs as a potent strategic tool to complement endogenous adiponectin for the treatment of adiponectin deficiency diseases...
June 9, 2017: Life Sciences
https://www.readbyqxmd.com/read/28606422/effect-of-aerobic-exercise-on-markers-of-bone-metabolism-of-overweight-and-obese-patients-with-chronic-kidney-disease
#18
Tarcisio Santana Gomes, Danilo Takashi Aoike, Flavia Baria, Fabiana G Graciolli, Rosa M A Moyses, Lilian Cuppari
OBJECTIVE: The aim of the study was to investigate the effect of aerobic exercise on markers of bone metabolism in overweight and obese nondialysis-dependent patients with chronic kidney disease. METHODS: This is a post-hoc study with 39 sedentary patients (55.5 ± 8.3 years, body mass index 31.2 ± 4.4 kg/m(2), estimated glomerular filtration rate 26.9 ± 11.7 mL/minute) who were randomly assigned to the aerobic exercise group (n = 24) or the control group (n = 15)...
June 9, 2017: Journal of Renal Nutrition
https://www.readbyqxmd.com/read/28604599/nutritional-challenges-in-duchenne-muscular-dystrophy
#19
REVIEW
Simona Salera, Francesca Menni, Maurizio Moggio, Sophie Guez, Monica Sciacco, Susanna Esposito
Neuromuscular diseases (NMDs) represent a heterogeneous group of acquired or inherited conditions. Nutritional complications are frequent in NMDs, but they are sometimes underestimated. With the prolongation of survival in patients with NMDs, there are several nutritional aspects that are important to consider, including the deleterious effects of overnutrition on glucose metabolism, mobility, and respiratory and cardiologic functions; the impact of hyponutrition on muscle and ventilatory function; constipation and other gastrointestinal complications; chewing/swallowing difficulties with an increased risk of aspiration that predisposes to infectious diseases and respiratory complications; as well as osteoporosis with an associated increased risk of fractures...
June 10, 2017: Nutrients
https://www.readbyqxmd.com/read/28604383/pyruvate-controls-the-checkpoint-inhibitor-pd-l1-and-suppresses-t-cell-immunity
#20
Ryu Watanabe, Tsuyoshi Shirai, Hong Namkoong, Hui Zhang, Gerald J Berry, Barbara B Wallis, Benedikt Schaefgen, David G Harrison, Jennifer A Tremmel, John C Giacomini, Jörg J Goronzy, Cornelia M Weyand
Patients with coronary artery disease (CAD) are at high risk for reactivation of the varicella zoster virus (VZV) and development of herpes zoster (HZ). Here, we found that macrophages from patients with CAD actively suppress T cell activation and expansion, leading to defective VZV-specific T cell immunity. Monocyte-derived and plaque-infiltrating macrophages from patients with CAD spontaneously expressed high surface density of the immunoinhibitory ligand programmed death ligand-1 (PD-L1), thereby providing negative signals to programmed death-1+ (PD-1+) T cells...
June 12, 2017: Journal of Clinical Investigation
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