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Bone and Mineral Disease

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https://www.readbyqxmd.com/read/28214296/abnormally-high-and-heterogeneous-bone-matrix-mineralization-after-childhood-solid-organ-transplantation-a-complex-pathology-of-low-bone-turnover-and-local-defects-in-mineralization
#1
Nadja Fratzl-Zelman, Helena Valta, Renata C Pereira, Barbara M Misof, Paul Roschger, Hannu Jalanko, Kathrine Wesseling-Perry, Klaus Klaushofer, Outi Mäkitie
Chronic renal, liver and heart failure in children associates with multiple skeletal complications. Increased fracture incidence often persists after transplantation and could be related to alterations in bone material properties. In the present cohort study we evaluated bone mineralization density distribution (BMDD) by quantitative backscattered electron imaging (qBEI) in 23 pediatric solid organ allograft recipients with suspected osteoporosis. We measured BMDD in the entire cross-sectional area of transiliac bone biopsies obtained from kidney (n = 9), liver (n = 9) and heart (n = 5) transplant recipients (aged 7...
February 18, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28212898/low-serum-iron-is-associated-with-high-serum-intact-fgf23-in-elderly-men-the-swedish-mros-study
#2
Catharina Lewerin, Östen Ljunggren, Herman Nilsson-Ehle, Magnus K Karlsson, Hans Herlitz, Mattias Lorentzon, Claes Ohlsson, Dan Mellström
BACKGROUND: Fibroblast growth factor (FGF23) is a protein that is produced by osteoblasts and osteocytes. Increased serum levels of FGF23 have been associated with increased risks of osteoporotic fractures and cardiovascular disease, particularly in participants with poor renal function. Serum iron (Fe) has been suggested as a regulator of FGF23 homeostasis. OBJECTIVE: To determine whether Fe and iron status are determinants of the levels of intact FGF23 (iFGF23) in elderly men...
February 14, 2017: Bone
https://www.readbyqxmd.com/read/28206698/fkbp10-deletion-in-osteoblasts-leads-to-qualitative-defects-in-bone
#3
Caressa D Lietman, Joohyun Lim, Ingo Grafe, Yuqing Chen, Hao Ding, Xiaohong Bi, Catherine G Ambrose, Nadja Fratzl-Zelman, Paul Roschger, Klaus Klaushofer, Wolfgang Wagermaier, Ingo Schmidt, Peter Fratzl, Jyoti Rai, MaryAnn Weis, David Eyre, Douglas R Keene, Deborah Krakow, Brendan H Lee
Osteogenesis Imperfecta (OI), also known as brittle bone disease, displays a spectrum of clinical severity from mild (OI type I) to severe early lethality (OI type II), with clinical features including low bone mass, fractures and deformities. Mutations in the FK506 Binding Protein 10 (FKBP10), gene encoding the 65KDa protein FKBP65, cause a recessive form of OI and Bruck syndrome, the latter being characterized by joint contractures in addition to low bone mass. We previously showed that Fkbp10 expression is limited to bone, tendon and ligaments in postnatal tissues...
February 16, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28204954/biopsy-vs-peripheral-computed-tomography-to-assess-bone-disease-in-ckd-patients-on-dialysis-differences-and-similarities
#4
I D B Marques, M J C L N Araújo, F G Graciolli, L M Dos Reis, R M Pereira, M R Custódio, V Jorgetti, R M Elias, E David-Neto, R M A Moysés
: Results from bone biopsy and high-resolution peripheral quantitative computed tomography (HR-pQCT) were compared in 31 CKD patients. There was an agreement mainly for cortical compartment that may represent a perspective on the fracture risk assessment. HR-pQCT also provided some clues on the turnover status, which warrants further studies. INTRODUCTION: Chronic kidney disease (CKD) patients are at high risk of bone disease. Although bone biopsy is considered the best method to evaluate bone disease, it is expensive and not always available...
February 16, 2017: Osteoporosis International
https://www.readbyqxmd.com/read/28203361/long-term-iron-polymaltose-infusions-associated-with-hypophosphataemic-osteomalacia-a-report-of-two-cases-and-review-of-the-literature
#5
REVIEW
Ramy H Bishay, Kirtan Ganda, Markus J Seibel
Iron-induced hypophosphataemic osteomalacia remains under-recognized as a potential complication of parenteral iron therapy. We here report two cases of symptomatic hypophosphataemic osteomalacia with multiple insufficiency fractures in the context of chronic gastrointestinal blood loss, necessitating monthly iron polymaltose infusions over prolonged periods of time. Respective blood tests revealed severe hypophosphataemia [0.29 and 0.43; normal range (NR) 0.8-1.5 mmol/l] in the presence of normal serum calcium and 25-hydroxy vitamin D levels...
January 2017: Therapeutic Advances in Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28203357/wrist-bone-mineral-density-utility-in-diagnosing-hip-osteoporosis-in-postmenopausal-women
#6
Bina Eftekhar-Sadat, Mostafa Ghavami, Vahideh Toopchizadeh, Masood Ghahvechi Akbari
BACKGROUND: Osteoporosis is a common bone disease that increases with age. Wrist bone mineral density (BMD) has significant correlation with other skeletal sites and it could be used as a diagnostic method for osteoporosis. The purpose of this study was to evaluate the role of wrist BMD in diagnosing osteoporosis in postmenopausal women. METHODS: In this cross-sectional study, 99 postmenopausal women with mean age of 57 ± 6.9 (range 50-76) years were evaluated...
December 2016: Therapeutic Advances in Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28201994/risedronate-therapy-in-patients-with-mild-to-moderate-chronic-kidney-disease-with-osteoporosis-post-hoc-analysis-of-data-from-the-risedronate-phase-iii-clinical-trials
#7
Takashi Shigematsu, Ryoichi Muraoka, Toshitsugu Sugimoto, Yoshiki Nishizawa
BACKGROUND: The clinical effect of bisphosphonate treatment has not been clearly evaluated by kidney function in Japanese Chronic Kidney Disease (CKD) patients with osteoporosis. This study analyzed the data from three risedronate Japanese phase III trials. The clinical effect of risedronate therapy was evaluated in CKD patients with osteoporosis. METHODS: The Japanese clinical trials involved 852 subjects who received risedronate (2.5 mg once daily or 17.5 mg once weekly) and whose estimated glomerular filtration rate (eGFR) were calculable and at ≥ 30 mL/min...
February 15, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28199411/cortical-porosity-not-superior-to-conventional-densitometry-in-identifying-hemodialysis-patients-with-fragility-fracture
#8
Bernhard Bielesz, Janina M Patsch, Lukas Fischer, Marija Bojic, Wolfgang Winnicki, Michael Weber, Daniel Cejka
Hemodialysis (HD) patients face increased fracture risk, which is further associated with elevated risk of hospitalization and mortality. High-resolution peripheral computed tomography (HR-pQCT) has advanced our understanding of bone disease in chronic kidney disease by characterizing distinct changes in both the cortical and trabecular compartments. Increased cortical porosity (Ct.Po) has been shown to be associated with fracture in patients with osteopenia or in postmenopausal diabetic women. We tested whether the degree of Ct...
2017: PloS One
https://www.readbyqxmd.com/read/28194480/evaluation-of-bone-mineral-density-and-microarchitectural-parameters-by-dxa-and-hr-pqct-in-37-children-and-adults-with-x-linked-hypophosphatemic-rickets
#9
G P Colares Neto, R M R Pereira, J C Alvarenga, L Takayama, M F A Funari, R M Martin
: In X-linked hypophosphatemic (XLH) rickets, dual-energy X-ray absorptiometry (DXA) measurements must be analyzed with caution. High-resolution peripheral quantitative computed tomography (HR-pQCT) analysis suggested that XLH primarily affects the cancellous compartment, with the tibia more affected than the radius. Effective treatment of XLH appears to positively affect bone mineralization, mainly in the bone cortex. INTRODUCTION: The purpose of this study is to evaluate bone mineral density (BMD) and microarchitecture in 37 patients (13 children and 24 adults) with XLH confirmed by PHEX mutations from a tertiary center compared to healthy controls...
February 13, 2017: Osteoporosis International
https://www.readbyqxmd.com/read/28194446/knockin-mouse-with-mutant-g%C3%AE-11-mimics-human-inherited-hypocalcemia-and-is-rescued-by-pharmacologic-inhibitors
#10
Kelly L Roszko, Ruiye Bi, Caroline M Gorvin, Hans Bräuner-Osborne, Xiao-Feng Xiong, Asuka Inoue, Rajesh V Thakker, Kristian Strømgaard, Thomas Gardella, Michael Mannstadt
Heterotrimeric G proteins play critical roles in transducing extracellular signals generated by 7-transmembrane domain receptors. Somatic gain-of-function mutations in G protein α subunits are associated with a variety of diseases. Recently, we identified gain-of-function mutations in Gα11 in patients with autosomal-dominant hypocalcemia type 2 (ADH2), an inherited disorder of hypocalcemia, low parathyroid hormone (PTH), and hyperphosphatemia. We have generated knockin mice harboring the point mutation GNA11 c...
February 9, 2017: JCI Insight
https://www.readbyqxmd.com/read/28193296/comparison-between-clinical-significance-of-height-adjusted-and-weight-adjusted-appendicular-skeletal-muscle-mass
#11
Taishi Furushima, Motohiko Miyachi, Motoyuki Iemitsu, Haruka Murakami, Hiroshi Kawano, Yuko Gando, Ryoko Kawakami, Kiyoshi Sanada
BACKGROUND: This study aimed to compare relationships between height- or weight-adjusted appendicular skeletal muscle mass (ASM/Ht(2) or ASM/Wt) and risk factors for cardiometabolic diseases or osteoporosis in Japanese men and women. METHODS: Subjects were healthy Japanese men (n = 583) and women (n = 1218). The study population included a young group (310 men and 357 women; age, 18-40 years) and a middle-aged and elderly group (273 men and 861 women; age, ≥41 years)...
February 13, 2017: Journal of Physiological Anthropology
https://www.readbyqxmd.com/read/28189120/does-the-opg-rankl-system-contribute-to-the-bone-vascular-axis-in-chronic-kidney-disease-a-systematic-review
#12
REVIEW
Beata Znorko, Ewa Oksztulska-Kolanek, Małgorzata Michałowska, Tomasz Kamiński, Krystyna Pawlak
Vascular calcification (VC) is highly prevalent in patients with chronic kidney disease (CKD) and is strongly associated with cardiovascular mortality and morbidity. Accumulating evidence over the past decade has challenged the hypothesis of close interaction between bone and VC what raises the possibility of a common underlying pathophysiological mechanism. Lately, bone regulatory proteins such as: osteoprotegerin (OPG) and Receptor Activator for Nuclear Factor κB Ligand (RANKL) has attracted attention of researchers as a possible key mediators of bone-vascular calcification imbalance...
February 8, 2017: Advances in Medical Sciences
https://www.readbyqxmd.com/read/28188861/functions-of-rho-family-of-small-gtpases-and-rho-associated-coiled-coil-kinases-in-bone-cells-during-differentiation-and-mineralization
#13
REVIEW
Agnieszka Strzelecka-Kiliszek, Saida Mebarek, Monika Roszkowska, René Buchet, David Magne, Slawomir Pikula
BACKGROUND: Members of Rho-associated coiled-coil kinases (ROCKs) are effectors of Rho family of small GTPases. ROCKs have multiple functions that include regulation of cellular contraction and polarity, adhesion, motility, proliferation, apoptosis, differentiation, maturation and remodeling of the extracellular matrix (ECM). SCOPE OF THE REVIEW: Here, we focus on the action of RhoA and RhoA effectors, ROCK1 and ROCK2, in cells related to tissue mineralization: mesenchymal stem cells, chondrocytes, pre-osteoblasts, osteoblasts, osteocytes, lining cells and osteoclasts...
February 7, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28187731/strontium-ranelate-causes-osteophytes-overgrowth-in-a-model-of-early-phase-osteoarthritis
#14
Jian-Guo Chu, Mu-Wei Dai, Yu Wang, Fa-Ming Tian, Hui-Ping Song, Ya-Ping Xiao, Li-Tao Shao, Ying-Ze Zhang, Liu Zhang
BACKGROUND: Osteoarthritis (OA) involves cartilage changes as well as modifications of subchondral bone and synovial tissues. Strontium ranelate (SR), an anti-osteoporosis compound, which is currently in phase III clinical trial for treatment of OA. Evidences suggest that SR preferably deposited in osteophyte, other than in subchondral bone in early phase of OA. This phenomenon raises concern about its utility for OA treatment as a disease-modifying drug. To evaluate the effect of SR on cartilage, subchondral bone mass and subchondral trabecular bone structure in medial meniscectomized (MNX) guinea pigs...
February 10, 2017: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/28186657/association-of-vitamin-d-metabolites-with-parathyroid-hormone-fibroblast-growth-factor-23-calcium-and-phosphorus-in-dogs-with-various-stages-of-chronic-kidney-disease
#15
V J Parker, L M Harjes, K Dembek, G S Young, D J Chew, R E Toribio
BACKGROUND: Hypovitaminosis D is associated with progression of renal disease, development of renal secondary hyperparathyroidism (RHPT), chronic kidney disease-mineral bone disorder (CKD-MBD), and increased mortality in people with CKD. Despite what is known regarding vitamin D dysregulation in humans with CKD, little is known about vitamin D metabolism in dogs with CKD. OBJECTIVES: The purpose of our study was to further elucidate vitamin D status in dogs with different stages of CKD and to relate it to factors that affect the development of CKD-MBD, including parathyroid hormone (PTH), fibroblast growth factor-23 (FGF-23), calcium, and phosphorus concentrations...
February 10, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28185216/clinical-evaluation-of-bone-strength-and-fracture-risk
#16
REVIEW
Chantal M J de Bakker, Wei-Ju Tseng, Yihan Li, Hongbo Zhao, X Sherry Liu
PURPOSE OF REVIEW: This paper seeks to evaluate and compare recent advances in the clinical assessment of the changes in bone mechanical properties that take place as a result of osteoporosis and other metabolic bone diseases and their treatments. RECENT FINDINGS: In addition to the standard of DXA-based areal bone mineral density (aBMD), a variety of methods, including imaging-based structural measurements, finite element analysis (FEA)-based techniques, and alternate methods including ultrasound, bone biopsy, reference point indentation, and statistical shape and density modeling, have been developed which allow for reliable prediction of bone strength and fracture risk...
February 9, 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28181418/mathematical-modeling-of-the-effects-of-ck2-3-on-mineralization-in-osteoporotic-bone
#17
A Lisberg, R Ellis, K Nicholson, P Moku, A Swarup, P Dhurjati, A Nohe
Osteoporosis is caused by decreased bone mineral density (BMD) and new treatments for this disease are desperately needed. Bone morphogenetic protein 2 (BMP2) is crucial for bone formation. The mimetic peptide CK2.3 acts downstream of BMP2 and increases BMD when injected systemically into the tail vein of mice. However, the most effective dosage needed to induce BMD in humans is unknown. We developed a mathematical model for CK2.3-dependent bone mineralization. We used a physiologically based pharmacokinetic (PBPK) model to derive the CK2...
February 9, 2017: CPT: Pharmacometrics & Systems Pharmacology
https://www.readbyqxmd.com/read/28179094/pediatric-onset-inflammatory-bowel-disease-poses-risk-for-low-bone-mineral-density-at-early-adulthood
#18
Anat Guz-Mark, Firas Rinawi, Oxana Egotubov, Ilan Shimon, Raanan Shamir, Amit Assa
BACKGROUND: Inflammatory bowel disease (IBD) is known to pose a risk for low bone mineral density (BMD) in children and adults. We aimed to evaluate the impact of pediatric-onset IBD on BMD in adulthood. METHODS: Records of pediatric-IBD patients were retrospectively reviewed for documentation of dual-energy X-ray absorptiometry (DXA) scans in adulthood. BMD was expressed as z-score. RESULTS: Sixty one patients were included. Mean (±SD) age at diagnosis was 14...
January 20, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28178857/dietary-2-oxoglutarate-prevents-bone-loss-caused-by-neonatal-treatment-with-maximal-dexamethasone-dose
#19
Piotr Dobrowolski, Ewa Tomaszewska, Siemowit Muszyński, Tomasz Blicharski, Stefan G Pierzynowski
Synthetic glucocorticoids (GCs) are widely used in the variety of dosages for treatment of premature infants with chronic lung disease, respiratory distress syndrome, allergies, asthma, and other inflammatory and autoimmune conditions. Yet, adverse effects such as glucocorticoid-induced osteoporosis and growth retardation are recognized. Conversely, 2-oxoglutarate (2-Ox), a precursor of glutamine, glutamate, and collagen amino acids, exerts protective effects on bone development. Our aim was to elucidate the effect of dietary administered 2-Ox on bone loss caused by neonatal treatment with clinically relevant maximal therapeutic dexamethasone (Dex) dose...
January 1, 2017: Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28177140/serum-phosphate-is-associated-with-fracture-risk-the-rotterdam-study-and-mros
#20
N Campos-Obando, W N H Koek, E R Hooker, B C J van der Eerden, H A Pols, A Hofman, J P T M van Leeuwen, A G Uitterlinden, C M Nielson, M C Zillikens
Extreme phosphate levels (P) have been associated with mineralization defects and increased fracture risk. Whether P within normal range is related to bone health in the general population is not well understood. To investigate the association of P with bone mineral density (BMD) and fracture risk, we assessed two population-based cohorts: the Dutch Rotterdam Study (RS-I, RS-II, RS-III; n = 6791) and the US Osteoporotic Fractures in Men (MrOS; n = 5425) study. The relationship of P with lumbar spine (LS) and femoral neck (FN) BMD was tested in all cohorts via linear models; fracture risk was tested in RS-I, RS-II and MrOS through Cox models, after follow-up of 8...
February 8, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
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