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Sample records for bone diseases developmental

  1. Developmental orthopaedic diseases in foals

    International Nuclear Information System (INIS)

    Şİrİn, Özlem; Alkan, Zeki

    2010-01-01

    Developmental Orthopaedic Diseases (DOD) is seen frequently in horses which completed their maturity. Osteochondrosis, physitis, angular limb deformities, flexural deformities, juvenil arthritis, cervical vertebral anomalies, cuboidal bone abnormalities are problems investigated under Developmental Orthopaedic Diseases title. This diseases can develop single or some together in fast growing, heavy animals (especially Arabian and English Thoroughbreds). Multifactorial causes of this diseases etiopathogenesis can be listed as genetic predisposition, trauma, nutrition, vitamins/minerals and endocrine disorders. But the exact causes of these diseases are not known. In this review detailed information are given about the diseases mentioned above

  2. Bone Marrow Adipocyte Developmental Origin and Biology.

    Science.gov (United States)

    Bukowska, Joanna; Frazier, Trivia; Smith, Stanley; Brown, Theodore; Bender, Robert; McCarthy, Michelle; Wu, Xiying; Bunnell, Bruce A; Gimble, Jeffrey M

    2018-06-01

    This review explores how the relationships between bone marrow adipose tissue (BMAT) adipogenesis with advancing age, obesity, and/or bone diseases (osteopenia or osteoporosis) contribute to mechanisms underlying musculoskeletal pathophysiology. Recent studies have re-defined adipose tissue as a dynamic, vital organ with functions extending beyond its historic identity restricted solely to that of an energy reservoir or sink. "State of the art" methodologies provide novel insights into the developmental origin, physiology, and function of different adipose tissue depots. These include genetic tracking of adipose progenitors, viral vectors application, and sophisticated non-invasive imaging modalities. While constricted within the rigid bone cavity, BMAT vigorously contributes to local and systemic metabolic processes including hematopoiesis, osteogenesis, and energy metabolism and undergoes dynamic changes as a function of age, diet, bone topography, or sex. These insights will impact future research and therapies relating to osteoporosis.

  3. Bone- and bone marrow scintigraphy in Gaucher disease type 1

    International Nuclear Information System (INIS)

    Mikosch, P.; Zitter, F.; Gallowitsch, H.J.; Lind, P.; Wuertz, F.; Mehta, A.B.; Hughes, D.A.

    2008-01-01

    Scintigraphy is a method for imaging metabolism and should be viewed as complimentary to morphological imaging. Bone and bone marrow scintigraphy can particularly contribute to the detection of focal disease in Gaucher disease. In bone crises it can discriminate within three days after pain onset between local infection and aseptic necrosis. A further advantage of bone- and bone marrow scintigraphy is the visualization of the whole skeleton within one setting. Whole body imaging for focal lesions might thus be an objective in GD, in particular in patients complaining of several painful sites. Direct imaging of bone marrow deposits in GD by MIBI scintigraphy might be of special interest in children in whom bone marrow undergoes a developmental conversion from red to yellow marrow in the ap-pendicular skeleton. MRI interpretation in young GD patients is thus difficult in order to estimate the exact amount and extent of bone marrow infiltration by Gaucher cells. 99mTc-MIBI scintigraphy with its direct visualization of lipid storage could thus add interesting additional information not shown with other methods including MRI. Although MRI is the most accepted imaging modality in assessing the skeletal status in GD, a selective use of scintigraphy for imaging bone and bone marrow may add information in the evaluation of patients with Gaucher disease

  4. Bone disease in diabetes

    DEFF Research Database (Denmark)

    Shanbhogue, Vikram V.; Hansen, Stinus; Frost, Morten

    2017-01-01

    Type 1 and type 2 diabetes are generally accepted to be associated with increased bone fracture risk. However, the pathophysiological mechanisms of diabetic bone disease are poorly understood, and whether the associated increased skeletal fragility is a comorbidity or a complication of diabetes...... remains under debate. Although there is some indication of a direct deleterious effect of microangiopathy on bone, the evidence is open to question, and whether diabetic osteopathy can be classified as a chronic, microvascular complication of diabetes remains uncertain. Here, we review the current...... knowledge of potential contributory factors to diabetic bone disease, particularly the association between diabetic microangiopathy and bone mineral density, bone structure, and bone turnover. Additionally, we discuss and propose a pathophysiological model of the effects of diabetic microvascular disease...

  5. Bone Marrow Diseases

    Science.gov (United States)

    Bone marrow is the spongy tissue inside some of your bones, such as your hip and thigh bones. It contains stem cells. The stem cells can ... the platelets that help with blood clotting. With bone marrow disease, there are problems with the stem ...

  6. Two developmentally temporal quantitative trait loci underlie convergent evolution of increased branchial bone length in sticklebacks

    Science.gov (United States)

    Erickson, Priscilla A.; Glazer, Andrew M.; Cleves, Phillip A.; Smith, Alyson S.; Miller, Craig T.

    2014-01-01

    In convergent evolution, similar phenotypes evolve repeatedly in independent populations, often reflecting adaptation to similar environments. Understanding whether convergent evolution proceeds via similar or different genetic and developmental mechanisms offers insight towards the repeatability and predictability of evolution. Oceanic populations of threespine stickleback fish, Gasterosteus aculeatus, have repeatedly colonized countless freshwater lakes and streams, where new diets lead to morphological adaptations related to feeding. Here, we show that heritable increases in branchial bone length have convergently evolved in two independently derived freshwater stickleback populations. In both populations, an increased bone growth rate in juveniles underlies the convergent adult phenotype, and one population also has a longer cartilage template. Using F2 crosses from these two freshwater populations, we show that two quantitative trait loci (QTL) control branchial bone length at distinct points in development. In both populations, a QTL on chromosome 21 controls bone length throughout juvenile development, and a QTL on chromosome 4 controls bone length only in adults. In addition to these similar developmental profiles, these QTL show similar chromosomal locations in both populations. Our results suggest that sticklebacks have convergently evolved longer branchial bones using similar genetic and developmental programmes in two independently derived populations. PMID:24966315

  7. Clinical research of bone scan characteristics for metabolic bone diseases

    International Nuclear Information System (INIS)

    Zhu Ruisen; Luo Qiong; Lu Haikui; Chen Libo; Luo Quanyong

    2009-01-01

    Characteristic images of 99m Tc-MDP bone scintigraphy in patients with metabolic bone diseases (MBD) were analyzed and compared, in an attempt to improve the capability of differential diagnosis in this aspect. A total of 142 cases, clinically confirmed as (MBD), were categorized into six groups: hyperparathyroidism (117), renal osteodystrophy (4), Paget's disease (16), hypophosphatemic osteomalacia (2), Albers-Schonberg disease (2), and Brittle bone disease (1). They were diagnosed clinically or pathologically, and scanned with 99m Tc-MDP bone scintegraphy, from which the 142 MBD cases were classified into 4 types. The cases of Type I had increased amount of 99m Tc-MDP uptake in whole body bones, including hyperparathyroidism, Albers-Schonberg disease, brittle bone disease and renal osteodystrophy. The cases of Type II had high uptake of 99m Tc-MDP in local region of bones, including paget's disease, hypophosphatemic osteomalacia and hyperparathyroidism. A Type I case with pathological fracture or secondary osteopathy was classified as Type III. Type IV cases were in early stage of hyperparathyroidism, with normal bone scan image. Analysis of the characteristics of 99m Tc-MDP bone scintigraphic findings (locations, morphology and intensities) in patients with MBD may be helpful in the differential diagnosis of MBD, in association with the patient's history and X-ray data altogether. (authors)

  8. Developmental and Evolutionary Significance of the Zygomatic Bone.

    Science.gov (United States)

    Heuzé, Yann; Kawasaki, Kazuhiko; Schwarz, Tobias; Schoenebeck, Jeffrey J; Richtsmeier, Joan T

    2016-12-01

    The zygomatic bone is derived evolutionarily from the orbital series. In most modern mammals the zygomatic bone forms a large part of the face and usually serves as a bridge that connects the facial skeleton to the neurocranium. Our aim is to provide information on the contribution of the zygomatic bone to variation in midfacial protrusion using three samples; humans, domesticated dogs, and monkeys. In each case, variation in midface protrusion is a heritable trait produced by one of three classes of transmission: localized dysmorphology associated with single gene dysfunction, selective breeding, or long-term evolution from a common ancestor. We hypothesize that the shape of the zygomatic bone reflects its role in stabilizing the connection between facial skeleton and neurocranium and consequently, changes in facial protrusion are more strongly reflected by the maxilla and premaxilla. Our geometric morphometric analyses support our hypothesis suggesting that the shape of the zygomatic bone has less to do with facial protrusion. By morphometrically dissecting the zygomatic bone we have determined a degree of modularity among parts of the midfacial skeleton suggesting that these components have the ability to vary independently and thus can evolve differentially. From these purely morphometric data, we propose that the neural crest cells that are fated to contribute to the zygomatic bone experience developmental cues that distinguish them from the maxilla and premaxilla. The spatiotemporal and molecular identity of the cues that impart zygoma progenitors with their identity remains an open question that will require alternative data sets. Anat Rec, 299:1616-1630, 2016. © 2016 The Authors The Anatomical Record Published by Wiley Periodicals, Inc. © 2016 The Authors The Anatomical Record Published by Wiley Periodicals, Inc.

  9. Evaluation of bone diseases using dynamic bone scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Kumano, Machiko; Tamura, Kenji; Hamada, Tatsumi; Ishida, Osamu [Kinki Univ., Higashi-Osaka, Osaka (Japan); Kajita, Akiyoshi

    1983-12-01

    Dynamic bone scintigraphy with sup(99m)Tc-EHDP was performed on 96 patients with various bone diseases. The dynamic scintigrams obtained were then used to aid in the differential diagnosis of malignant (49 cases) and benign (8 cases) diseases. Short-term local deposition of the tracer in all cases of malignant bone diseases was observed in vascular (10-40 sec. after injection), and blood pool (1-3 min. after injection) phases. In the cases of malignant bone tumors where osteosclerotic lesions were present, tracer accumulation appeared in the blood pool phase. If osteolytic lesions were present, accumulation appeared in the vascular phase, and when the lesion was larger than 2 cm, accumulation was frequently found in the arterial phase. Scintigraphic differentiation of early primary and metastatic bone tumors from other lesions was facilitated by performing the dynamic scintigraphy with sup(99m)Tc-EHDP. Dynamic bone scintigraphy also allowed early diagnosis of avascular necrosis (14 cases) prior to the appearance of minimally abnormal X-ray findings, especially in cases of corticosteroid-induced necrosis.

  10. Bone Disease in Axial Spondyloarthritis.

    Science.gov (United States)

    Van Mechelen, Margot; Gulino, Giulia Rossana; de Vlam, Kurt; Lories, Rik

    2018-05-01

    Axial spondyloarthritis is a chronic inflammatory skeletal disorder with an important burden of disease, affecting the spine and sacroiliac joints and typically presenting in young adults. Ankylosing spondylitis, diagnosed by the presence of structural changes to the skeleton, is the prototype of this disease group. Bone disease in axial spondyloarthritis is a complex phenomenon with the coexistence of bone loss and new bone formation, both contributing to the morbidity of the disease, in addition to pain caused by inflammation. The skeletal structural changes respectively lead to increased fracture risk and to permanent disability caused by ankylosis of the sacroiliac joints and the spine. The mechanism of this new bone formation leading to ankylosis is insufficiently known. The process appears to originate from entheses, specialized structures that provide a transition zone in which tendon and ligaments insert into the underlying bone. Growth factor signaling pathways such as bone morphogenetic proteins, Wnts, and Hedgehogs have been identified as molecular drivers of new bone formation, but the relationship between inflammation and activation of these pathways remains debated. Long-standing control of inflammation appears necessary to avoid ankylosis. Recent evidence and concepts suggest an important role for biomechanical factors in both the onset and progression of the disease. With regard to new bone formation, these processes can be understood as ectopic repair responses secondary to inflammation-induced bone loss and instability. In this review, we discuss the clinical implications of the skeletal changes as well as the underlying molecular mechanisms, the relation between inflammation and new bone formation, and the potential role of biomechanical stress.

  11. Bone scan and SPECT/CT findings in marble bone disease

    International Nuclear Information System (INIS)

    Kapoor, Jiten; Joshi, Prathamesh; Lele, Vikram

    2012-01-01

    Marble bone disease or osteopetrosis, is a rare inborn disorder characterized by the failure of osteoclasts to resorb bone. Overall incidence of the disease is estimated to be 1 case in 100,000-500,000 population. Whereas the radiographic features of the disease are well known, information on bone scan imaging is sparse in the literature. We present technitium 99m methylene diphosphonate ( 99m Tc MDP) bone scan features of osteopetrosis, along with single photon emission computed tomography-computed tomography(SPECT/CT) correlation in a young male.

  12. Bone scan and SPECT/CT findings in marble bone disease

    Energy Technology Data Exchange (ETDEWEB)

    Kapoor, Jiten; Joshi, Prathamesh; Lele, Vikram [Jaslok Hospital and Research Centre, Woril (India)

    2012-03-15

    Marble bone disease or osteopetrosis, is a rare inborn disorder characterized by the failure of osteoclasts to resorb bone. Overall incidence of the disease is estimated to be 1 case in 100,000-500,000 population. Whereas the radiographic features of the disease are well known, information on bone scan imaging is sparse in the literature. We present technitium 99m methylene diphosphonate ({sup 99m}Tc MDP) bone scan features of osteopetrosis, along with single photon emission computed tomography-computed tomography(SPECT/CT) correlation in a young male.

  13. Denosumab for bone diseases: translating bone biology into targeted therapy.

    Science.gov (United States)

    Tsourdi, Elena; Rachner, Tilman D; Rauner, Martina; Hamann, Christine; Hofbauer, Lorenz C

    2011-12-01

    Signalling of receptor activator of nuclear factor-κB (RANK) ligand (RANKL) through RANK is a critical pathway to regulate the differentiation and activity of osteoclasts and, hence, a master regulator of bone resorption. Increased RANKL activity has been demonstrated in diseases characterised by excessive bone loss such as osteoporosis, rheumatoid arthritis and osteolytic bone metastases. The development and approval of denosumab, a fully MAB against RANKL, has heralded a new era in the treatment of bone diseases by providing a potent, targeted and reversible inhibitor of bone resorption. This article summarises the molecular and cellular biology of the RANKL/RANK system and critically reviews preclinical and clinical studies that have established denosumab as a promising novel therapy for metabolic and malignant bone diseases. We will discuss the potential indications for denosumab along with a critical review of safety and analyse its potential within the concert of established therapies.

  14. Management of adynamic bone disease in chronic kidney disease: A brief review

    Directory of Open Access Journals (Sweden)

    Swathi K. Sista

    2016-09-01

    Full Text Available The Kidney Disease: Improving Global Outcomes (KDIGO work group released recommendations in 2006 to define the bone-related pathology associated with chronic kidney disease as renal osteodystrophy. In 2009, KDIGO released revised clinical practice guidelines which redefined systemic disorders of bone and mineral metabolism due to chronic kidney disease as chronic kidney disease-mineral and bone disorders. Conditions under this overarching term include osteitis fibrosa cystica, osteomalacia, and adynamic bone disease. We aim to provide a brief review of the histopathology, pathophysiology, epidemiology, and diagnostic features of adynamic bone disease, focusing on current trends in the management of this complex bone disorder.

  15. Metabolic, endocrine, and related bone diseases

    International Nuclear Information System (INIS)

    Rogers, L.F.

    1987-01-01

    Bone is living tissue, and old bone is constantly removed and replaced with new bone. Normally this exchange is in balance, and the mineral content remains relatively constant. This balance may be disturbed as a result of certain metabolic and endocrinologic disorders. The term dystrophy, referring to a disturbance of nutrition, is applied to metabolic and endocrine bone diseases and should be distinguished from the term dysplasia, referring to a disturbance of bone growth. The two terms are easily confused but are not interchangeable. Metabolic bone disease is caused by endocrine imbalance, vitamin deficiency or excess, and other disturbances in bone metabolism leading to osteoporosis and osteomalacia

  16. Paget's disease of bone resembling bone metastasis from gastric cancer.

    Science.gov (United States)

    Shimoyama, Yasuyuki; Kusano, Motoyasu; Shimoda, Yoko; Ishihara, Shingo; Toyomasu, Yoshitaka; Ohno, Tetsuro; Mochiki, Erito; Sano, Takaaki; Hirato, Junko; Mori, Masatomo

    2011-08-01

    A 74-year-old man had an endoscopic type 0'-IIc tumor in the upper gastric body on the greater curvature and biopsy showed the tumor to be a well-differentiated adenocarcinoma (Group 5). He was referred to us for endoscopic submucosal dissection (ESD). Endoscopy revealed fold convergency, fold swelling, and fusion of the fold, indicating tumor invasion into the submucosa, which was outside the indications for ESD. In addition, there was an increase of serum bone-type alkaline phosphatase (ALP-III and ALP-IV) and urinary cross-linked N-terminal telopeptide of type I collagen (a bone metabolism marker), while (18)F-fluorodeoxyglucose positron emission tomography showed increased uptake in the left pelvis and Th10, suggesting bone metastases. We first diagnosed gastric cancer with bone metastases; however, the symptoms suggested pathological bone fracture and no bone pain. Therefore, a computed tomography-guided aspiration bone biopsy was performed to exclude the possibility of Paget's disease of bone. Biopsy specimens revealed no tumor and a mosaic pattern. No increased uptake of (18)F-FAMT (L-[3-(18)F] α-methyltyrosine) supported a diagnosis of no bone metastases from gastric cancer. We finally diagnosed gastric cancer accompanied by Paget's disease of bone and performed a laparoscopy-assisted proximal gastrectomy. The pathological diagnosis was U less 0-IIb, and U post 0-IIc ypT1a (M) N0H0P0M0 yp stage IA. In gastric cancer patients with suspected bone metastasis, we also need to consider Paget's disease of bone.

  17. Caisson disease of bone.

    Science.gov (United States)

    Gregg, P J; Walder, D N

    1986-09-01

    Caisson disease of bone, which may affect compressed air workers and divers, is characterized by regions of bone and marrow necrosis that may lead to secondary osteoarthrosis of the hip and shoulder joints. A review of the pathologic, radiologic, and clinical aspects demonstrated uncertainties in the exact etiology. Early diagnosis is often not possible because of the delayed appearance of radiologic abnormalities. Research into these two aspects of this condition was carried out by the Medical Research Council Decompression Sickness Research Team in Newcastle upon Tyne over a ten-year period (1972 to 1982). Because no suitable animal model exists for the study of this condition, bone and marrow necrosis was produced by embolism of bone blood vessels with glass microspheres. With this model, it was shown that the presence of bone and marrow necrosis could be detected by bone scintigraphy using 99mTc-MDP and by measuring changes in serum ferritin concentration at a much earlier stage than was possible by radiography. However, only the former method has proved useful in clinical practice. Investigations into the etiology of caisson disease of bone have shown evidence for an increase in marrow fat cell size resulting from hyperoxia. This phenomenon may play a role in the production and localization of gas bubble emboli, which are thought to be the cause of the bone and marrow necrosis.

  18. 3D Bioprinting of Developmentally Inspired Templates for Whole Bone Organ Engineering.

    Science.gov (United States)

    Daly, Andrew C; Cunniffe, Gráinne M; Sathy, Binulal N; Jeon, Oju; Alsberg, Eben; Kelly, Daniel J

    2016-09-01

    The ability to print defined patterns of cells and extracellular-matrix components in three dimensions has enabled the engineering of simple biological tissues; however, bioprinting functional solid organs is beyond the capabilities of current biofabrication technologies. An alternative approach would be to bioprint the developmental precursor to an adult organ, using this engineered rudiment as a template for subsequent organogenesis in vivo. This study demonstrates that developmentally inspired hypertrophic cartilage templates can be engineered in vitro using stem cells within a supporting gamma-irradiated alginate bioink incorporating Arg-Gly-Asp adhesion peptides. Furthermore, these soft tissue templates can be reinforced with a network of printed polycaprolactone fibers, resulting in a ≈350 fold increase in construct compressive modulus providing the necessary stiffness to implant such immature cartilaginous rudiments into load bearing locations. As a proof-of-principal, multiple-tool biofabrication is used to engineer a mechanically reinforced cartilaginous template mimicking the geometry of a vertebral body, which in vivo supported the development of a vascularized bone organ containing trabecular-like endochondral bone with a supporting marrow structure. Such developmental engineering approaches could be applied to the biofabrication of other solid organs by bioprinting precursors that have the capacity to mature into their adult counterparts over time in vivo. © 2016 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  19. Bone disease in primary hyperparathyroidism

    Science.gov (United States)

    Bandeira, Francisco; Cusano, Natalie E.; Silva, Barbara C.; Cassibba, Sara; Almeida, Clarissa Beatriz; Machado, Vanessa Caroline Costa; Bilezikian, John P.

    2015-01-01

    Bone disease in severe primary hyperparathyroidism (PHPT) is described classically as osteitis fibrosa cystica (OFC). Bone pain, skeletal deformities and pathological fractures are features of OFC. Bone mineral density is usually extremely low in OFC, but it is reversible after surgical cure. The signs and symptoms of severe bone disease include bone pain, pathologic fractures, proximal muscle weakness with hyperreflexia. Bone involvement is typically characterized as salt-and-pepper appearance in the skull, bone erosions and bone resorption of the phalanges, brown tumors and cysts. In the radiography, diffuse demineralization is observed, along with pathological fractures, particularly in the long bones of the extremities. In severe, symptomatic PHPT, marked elevation of the serum calcium and PTH concentrations are seen and renal involvement is manifested by nephrolithiasis and nephrocalcinosis. A new technology, recently approved for clinical use in the United States and Europe, is likely to become more widely available because it is an adaptation of the lumbar spine DXA image. Trabecular bone score (TBS) is a gray-level textural analysis that provides an indirect index of trabecular microarchitecture. Newer technologies, such as high-resolution peripheral quantitative computed tomography (HR-pQCT), have provided further understanding of the microstructural skeletal features in PHPT. PMID:25166047

  20. Semi-quantitative interpretation of the bone scan in metabolic bone disease

    Energy Technology Data Exchange (ETDEWEB)

    Fogelman, I; Turner, J G; Hay, I D; Boyle, I T [Royal Infirmary, Glasgow (UK). Dept. of Nuclear Medicine; Citrin, D L [Wisconsin Univ., Madison (USA). Dept. of Human Oncology; Bessent, G R

    1979-01-01

    Certain easily recognisable features are commonly seen in the bone scans of patients with metabolic bone disorders. Seven such features have been numerically graded by three independent observers in the scans of 100 patients with metabolic bone disease and of 50 control subjects. The total score for each patient is defined as the metabolic index. The mean metabolic index for each group of patients with metabolic bone disease is significantly greater than that for the control group (P < 0.001). (orig.).

  1. Developmental Programming of Adult Disease: Reprogramming by Melatonin?

    OpenAIRE

    Tain, You-Lin; Huang, Li-Tung; Hsu, Chien-Ning

    2017-01-01

    Adult-onset chronic non-communicable diseases (NCDs) can originate from early life through so-called the “developmental origins of health and disease” (DOHaD) or “developmental programming”. The DOHaD concept offers the “reprogramming” strategy to shift the treatment from adulthood to early life, before clinical disease is apparent. Melatonin, an endogenous indoleamine produced by the pineal gland, has pleiotropic bioactivities those are beneficial in a variety of human diseases. Emerging evi...

  2. Mesenchymal stem cells from cortical bone demonstrate increased clonal incidence, potency, and developmental capacity compared to their bone marrow–derived counterparts

    Directory of Open Access Journals (Sweden)

    Daniel Blashki

    2016-08-01

    Full Text Available In this study, we show that matrix dense cortical bone is the more potent compartment of bone than bone marrow as a stromal source for mesenchymal stem cells as isolated from adult rats. Lineage-depleted cortical bone-mesenchymal stem cells demonstrated >150-fold enrichment of colony forming unit–fibroblasts per cell incidence. compared to lineage-depleted bone marrow-mesenchymal stem cells, corresponding to a 70-fold increase in absolute recovered colony forming unit–fibroblasts. The composite phenotype Lin−/CD45−/CD31−/VLA-1+/Thy-1+ enriched for clonogenic mesenchymal stem cells solely from cortical bone–derived cells from which 70% of clones spontaneously differentiated into all lineages of bone, cartilage, and adipose. Both populations generated vascularized bone tissue within subcutaneous implanted collagen scaffolds; however, cortical bone–derived cells formed significantly more osteoid than bone marrow counterparts, quantified by histology. The data demonstrate that our isolation protocol identifies and validates mesenchymal stem cells with superior clonal, proliferative, and developmental potential from cortical bone compared to the bone marrow niche although marrow persists as the typical source for mesenchymal stem cells both in the literature and current pre-clinical therapies.

  3. Developmental Programming of Adult Disease: Reprogramming by Melatonin?

    Science.gov (United States)

    Tain, You-Lin; Huang, Li-Tung; Hsu, Chien-Ning

    2017-02-16

    Adult-onset chronic non-communicable diseases (NCDs) can originate from early life through so-called the "developmental origins of health and disease" (DOHaD) or "developmental programming". The DOHaD concept offers the "reprogramming" strategy to shift the treatment from adulthood to early life, before clinical disease is apparent. Melatonin, an endogenous indoleamine produced by the pineal gland, has pleiotropic bioactivities those are beneficial in a variety of human diseases. Emerging evidence support that melatonin is closely inter-related to other proposed mechanisms contributing to the developmental programming of a variety of chronic NCDs. Recent animal studies have begun to unravel the multifunctional roles of melatonin in many experimental models of developmental programming. Even though some progress has been made in research on melatonin as a reprogramming strategy to prevent DOHaD-related NCDs, future human studies should aim at filling the translational gap between animal models and clinical trials. Here, we review several key themes on the reprogramming effects of melatonin in DOHaD research. We have particularly focused on the following areas: mechanisms of developmental programming; the interrelationship between melatonin and mechanisms underlying developmental programming; pathophysiological roles of melatonin in pregnancy and fetal development; and insight provided by animal models to support melatonin as a reprogramming therapy. Rates of NCDs are increasing faster than anticipated all over the world. Hence, there is an urgent need to understand reprogramming mechanisms of melatonin and to translate experimental research into clinical practice for halting a growing list of DOHaD-related NCDs.

  4. Nanotechnology in the targeted drug delivery for bone diseases and bone regeneration

    Directory of Open Access Journals (Sweden)

    Gu W

    2013-06-01

    Full Text Available Wenyi Gu,1,2 Chengtie Wu,3 Jiezhong Chen,1 Yin Xiao1 1Institute of Health and Biomedical Innovation, Queensland University of Technology, Brisbane, QLD, Australia; 2Australian Institute for Bioengineering and Nanotechnology, The University of Queensland, Brisbane, QLD, Australia; 3State Key Laboratory of High Performance Ceramics and Superfine Microstructure, Shanghai Institute of Ceramics, Chinese Academy of Sciences, Shanghai, People's Republic of China Abstract: Nanotechnology is a vigorous research area and one of its important applications is in biomedical sciences. Among biomedical applications, targeted drug delivery is one of the most extensively studied subjects. Nanostructured particles and scaffolds have been widely studied for increasing treatment efficacy and specificity of present treatment approaches. Similarly, this technique has been used for treating bone diseases including bone regeneration. In this review, we have summarized and highlighted the recent advancement of nanostructured particles and scaffolds for the treatment of cancer bone metastasis, osteosarcoma, bone infections and inflammatory diseases, osteoarthritis, as well as for bone regeneration. Nanoparticles used to deliver deoxyribonucleic acid and ribonucleic acid molecules to specific bone sites for gene therapies are also included. The investigation of the implications of nanoparticles in bone diseases have just begun, and has already shown some promising potential. Further studies have to be conducted, aimed specifically at assessing targeted delivery and bioactive scaffolds to further improve their efficacy before they can be used clinically. Keywords: nanoparticles, nanostructured scaffold, cancer bone metastasis, bone diseases, target drug delivery, bone regeneration

  5. Nanotechnology in the targeted drug delivery for bone diseases and bone regeneration

    Science.gov (United States)

    Gu, Wenyi; Wu, Chengtie; Chen, Jiezhong; Xiao, Yin

    2013-01-01

    Nanotechnology is a vigorous research area and one of its important applications is in biomedical sciences. Among biomedical applications, targeted drug delivery is one of the most extensively studied subjects. Nanostructured particles and scaffolds have been widely studied for increasing treatment efficacy and specificity of present treatment approaches. Similarly, this technique has been used for treating bone diseases including bone regeneration. In this review, we have summarized and highlighted the recent advancement of nanostructured particles and scaffolds for the treatment of cancer bone metastasis, osteosarcoma, bone infections and inflammatory diseases, osteoarthritis, as well as for bone regeneration. Nanoparticles used to deliver deoxyribonucleic acid and ribonucleic acid molecules to specific bone sites for gene therapies are also included. The investigation of the implications of nanoparticles in bone diseases have just begun, and has already shown some promising potential. Further studies have to be conducted, aimed specifically at assessing targeted delivery and bioactive scaffolds to further improve their efficacy before they can be used clinically. PMID:23836972

  6. Nanotechnology in the targeted drug delivery for bone diseases and bone regeneration.

    Science.gov (United States)

    Gu, Wenyi; Wu, Chengtie; Chen, Jiezhong; Xiao, Yin

    2013-01-01

    Nanotechnology is a vigorous research area and one of its important applications is in biomedical sciences. Among biomedical applications, targeted drug delivery is one of the most extensively studied subjects. Nanostructured particles and scaffolds have been widely studied for increasing treatment efficacy and specificity of present treatment approaches. Similarly, this technique has been used for treating bone diseases including bone regeneration. In this review, we have summarized and highlighted the recent advancement of nanostructured particles and scaffolds for the treatment of cancer bone metastasis, osteosarcoma, bone infections and inflammatory diseases, osteoarthritis, as well as for bone regeneration. Nanoparticles used to deliver deoxyribonucleic acid and ribonucleic acid molecules to specific bone sites for gene therapies are also included. The investigation of the implications of nanoparticles in bone diseases have just begun, and has already shown some promising potential. Further studies have to be conducted, aimed specifically at assessing targeted delivery and bioactive scaffolds to further improve their efficacy before they can be used clinically.

  7. Biochemical markers of bone turnover in diagnosis of myeloma bone disease.

    Science.gov (United States)

    Dizdar, Omer; Barista, Ibrahim; Kalyoncu, Umut; Karadag, Omer; Hascelik, Gulsen; Cila, Aysenur; Pinar, Asli; Celik, Ismail; Kars, Ayse; Tekuzman, Gulten

    2007-03-01

    This study was designed to explore the value of markers of bone turnover, macrophage inflammatory protein-1alpha (MIP-1alpha), and osteopontin (OPN) in the diagnosis of myeloma bone disease. Twenty-five patients with newly diagnosed and untreated multiple myeloma (MM), and 22 age-, sex-, and bone mineral density-matched control subjects were enrolled. Levels of MIP-1alpha, OPN, carboxy-terminal telopeptide of Type-1 collagen (C-telopeptide or Ctx), deoxypyridinoline (DPD), Type-1 collagen propeptide (T1Pro), and bone-specific alkaline phosphatase (BALP) were assessed in both groups. Twenty-two of the patients had bone involvement documented by skeletal surveys and lumbar spinal magnetic resonance imaging. Levels of serum Ctx, OPN, MIP-1alpha, and urine DPD were significantly higher in MM patients with bone disease than in controls (P<0.01). Serum Ctx levels were elevated in 90.9% of patients with MM and 40.9% of controls (P<0.001). Urine DPD levels were elevated in 90.4% of the patients and 31.8% of the controls (P<0.001). The serum OPN and MIP-1alpha levels of the patients were significantly correlated with beta2-microglobulin and lactate dehydrogenase levels (P<0.05). Our study indicates that Ctx and DPD are sensitive markers of bone disease in MM, and higher than normal values suggest presence of bone disease rather than benign osteoporosis in MM. The utility of OPN and MIP-1alpha needs to be further investigated. Copyright (c) 2006 Wiley-Liss, Inc.

  8. [Metabolic bone disease osteomalacia].

    Science.gov (United States)

    Reuss-Borst, M A

    2014-05-01

    Osteomalacia is a rare disorder of bone metabolism leading to reduced bone mineralization. Underlying vitamin D deficiency and a disturbed phosphate metabolism (so-called hypophosphatemic osteomalacia) can cause the disease. Leading symptoms are dull localized or generalized bone pain, muscle weakness and cramps as well as increased incidence of falls. Rheumatic diseases, such as polymyalgia rheumatica, rheumatoid arthritis, myositis and fibromyalgia must be considered in the differential diagnosis. Alkaline phosphatase (AP) is typically elevated in osteomalacia while serum phosphate and/or 25-OH vitamin D3 levels are reduced. The diagnosis of osteomalacia can be confirmed by an iliac crest bone biopsy. Histological correlate is reduced or deficient mineralization of the newly synthesized extracellular matrix. Treatment strategies comprise supplementation of vitamin D and calcium and for patients with intestinal malabsorption syndromes vitamin D and calcium are also given parenterally. In renal phosphate wasting syndromes substitution of phosphate is the treatment of choice, except for tumor-induced osteomalacia when removal of the tumor leads to a cure in most cases.

  9. Developmental Programming, a Pathway to Disease

    Science.gov (United States)

    Cardoso, Rodolfo C.; Puttabyatappa, Muraly

    2016-01-01

    Accumulating evidence suggests that insults occurring during the perinatal period alter the developmental trajectory of the fetus/offspring leading to long-term detrimental outcomes that often culminate in adult pathologies. These perinatal insults include maternal/fetal disease states, nutritional deficits/excess, stress, lifestyle choices, exposure to environmental chemicals, and medical interventions. In addition to reviewing the various insults that contribute to developmental programming and the benefits of animal models in addressing underlying mechanisms, this review focuses on the commonalities in disease outcomes stemming from various insults, the convergence of mechanistic pathways via which various insults can lead to common outcomes, and identifies the knowledge gaps in the field and future directions. PMID:26859334

  10. Role of bone scan in rheumatic disease

    International Nuclear Information System (INIS)

    Choi, Yun Young

    2003-01-01

    Rheumatic diseases can be categorized by pathology into several specific types of musculoskeletal problems, including synovitis (e.g. rheumatoid arthritis), enthesopathy (e.g. ankylosing spondylitis) and cartilage degeneration (e.g. osteoarthritis). Skeletal radiographs have contributed to the diagnosis of these articular diseases, and some disease entities need typical radiographic changes as a factor of the diagnostic criteria. However, they sometimes show normal radiographic findings in the early stage of disease, when there is demineralization of less than 30-50%. Bone scans have also been used in arthritis, but not widely because the findings are nonspecific and it is thought that bone scans do not add significant information to routine radiography. Bone scans do however play a different role than simple radiography, and it is a complementary imaging method in the course of management of arthritis. The image quality of bone scans can be improved by obtaining regional views and images under al pin-hole collimator, and through a variety of scintigraphic techniques including the three phase bone scan and bone SPECT. Therefore, bone scans could improve the diagnostic value, and answer multiple clinical questions, based on the pathophysiology of various forms of arthritis

  11. Developmental Bisphenol A Exposure Modulates Immune-Related Diseases

    Science.gov (United States)

    Xu, Joella; Huang, Guannan; Guo, Tai L.

    2016-01-01

    Bisphenol A (BPA), used in polycarbonate plastics and epoxy resins, has a widespread exposure to humans. BPA is of concern for developmental exposure resulting in immunomodulation and disease development due to its ability to cross the placental barrier and presence in breast milk. BPA can use various mechanisms to modulate the immune system and affect diseases, including agonistic and antagonistic effects on many receptors (e.g., estrogen receptors), epigenetic modifications, acting on cell signaling pathways and, likely, the gut microbiome. Immune cell populations and function from the innate and adaptive immune system are altered by developmental BPA exposure, including decreased T regulatory (Treg) cells and upregulated pro- and anti-inflammatory cytokines and chemokines. Developmental BPA exposure can also contribute to the development of type 2 diabetes mellitus, allergy, asthma and mammary cancer disease by altering immune function. Multiple sclerosis and type 1 diabetes mellitus may also be exacerbated by BPA, although more research is needed. Additionally, BPA analogs, such as bisphenol S (BPS), have been increasing in use, and currently, little is known about their immune effects. Therefore, more studies should be conducted to determine if developmental exposure BPA and its analogs modulate immune responses and lead to immune-related diseases. PMID:29051427

  12. Developmental Bisphenol A Exposure Modulates Immune-Related Diseases

    Directory of Open Access Journals (Sweden)

    Joella Xu

    2016-09-01

    Full Text Available Bisphenol A (BPA, used in polycarbonate plastics and epoxy resins, has a widespread exposure to humans. BPA is of concern for developmental exposure resulting in immunomodulation and disease development due to its ability to cross the placental barrier and presence in breast milk. BPA can use various mechanisms to modulate the immune system and affect diseases, including agonistic and antagonistic effects on many receptors (e.g., estrogen receptors, epigenetic modifications, acting on cell signaling pathways and, likely, the gut microbiome. Immune cell populations and function from the innate and adaptive immune system are altered by developmental BPA exposure, including decreased T regulatory (Treg cells and upregulated pro- and anti-inflammatory cytokines and chemokines. Developmental BPA exposure can also contribute to the development of type 2 diabetes mellitus, allergy, asthma and mammary cancer disease by altering immune function. Multiple sclerosis and type 1 diabetes mellitus may also be exacerbated by BPA, although more research is needed. Additionally, BPA analogs, such as bisphenol S (BPS, have been increasing in use, and currently, little is known about their immune effects. Therefore, more studies should be conducted to determine if developmental exposure BPA and its analogs modulate immune responses and lead to immune-related diseases.

  13. Outcomes of bone density measurements in coeliac disease.

    Science.gov (United States)

    Bolland, Mark J; Grey, Andrew; Rowbotham, David S

    2016-01-29

    Some guidelines recommend that patients with newly diagnosed coeliac disease undergo bone density scanning. We assessed the bone density results in a cohort of patients with coeliac disease. We searched bone density reports over two 5-year periods in all patients from Auckland District Health Board (2008-12) and in patients under 65 years from Counties Manukau District Health Board (2009-13) for the term 'coeliac.' Reports for 137 adults listed coeliac disease as an indication for bone densitometry. The average age was 47 years, body mass index (BMI) 25 kg/m(2), and 77% were female. The median time between coeliac disease diagnosis and bone densitometry was 261 days. The average bone density Z-score was slightly lower than expected (Z-score -0.3 to 0.4) at the lumbar spine, total hip and femoral neck, but 88-93% of Z-scores at each site lay within the normal range. Low bone density was strongly related to BMI: the proportions with Z-score 30 kg/m(2) were 28%, 15%, 6% and 0% respectively. Average bone density was normal, suggesting that bone density measurement is not indicated routinely in coeliac disease, but could be considered on a case-by-case basis for individuals with strong risk factors for fracture.

  14. Maternal perinatal diet induces developmental programming of bone architecture.

    Science.gov (United States)

    Devlin, M J; Grasemann, C; Cloutier, A M; Louis, L; Alm, C; Palmert, M R; Bouxsein, M L

    2013-04-01

    Maternal high-fat (HF) diet can alter offspring metabolism via perinatal developmental programming. This study tests the hypothesis that maternal HF diet also induces perinatal programming of offspring bone mass and strength. We compared skeletal acquisition in pups from C57Bl/6J mice fed HF or normal diet from preconception through lactation. Three-week-old male and female pups from HF (HF-N) and normal mothers (N-N) were weaned onto normal diet. Outcomes at 14 and 26 weeks of age included body mass, body composition, whole-body bone mineral content (WBBMC) via peripheral dual-energy X-ray absorptiometry, femoral cortical and trabecular architecture via microcomputed tomography, and glucose tolerance. Female HF-N had normal body mass and glucose tolerance, with lower body fat (%) but higher serum leptin at 14 weeks vs. N-N (Pbone volume fraction was 20% higher at 14 weeks in female HF-N vs. N-N (Pbone area was 6% higher at 14 weeks vs. N-N (Pbone, supporting the hypothesis that maternal diet alters postnatal skeletal homeostasis.

  15. Posttranslational heterogeneity of bone alkaline phosphatase in metabolic bone disease.

    Science.gov (United States)

    Langlois, M R; Delanghe, J R; Kaufman, J M; De Buyzere, M L; Van Hoecke, M J; Leroux-Roels, G G

    1994-09-01

    Bone alkaline phosphatase is a marker of osteoblast activity. In order to study the posttranscriptional modification (glycosylation) of bone alkaline phosphatase in bone disease, we investigated the relationship between mass and catalytic activity of bone alkaline phosphatase in patients with osteoporosis and hyperthyroidism. Serum bone alkaline phosphatase activity was measured after lectin precipitation using the Iso-ALP test kit. Mass concentration of bone alkaline phosphatase was determined with an immunoradiometric assay (Tandem-R Ostase). In general, serum bone alkaline phosphatase mass and activity concentration correlated well. The activity : mass ratio of bone alkaline phosphatase was low in hyperthyroidism. Activation energy of the reaction catalysed by bone alkaline phosphatase was high in osteoporosis and in hyperthyroidism. Experiments with neuraminidase digestion further demonstrated that the thermodynamic heterogeneity of bone alkaline phosphatase can be explained by a different glycosylation of the enzyme.

  16. Paget's disease of the bone in Chinese woman

    International Nuclear Information System (INIS)

    H'ng, M.W.C.; Ho, Y.Y.

    2005-01-01

    Paget's disease, otherwise known as osteitis deformans, is an unusual condition in the Oriental population. We report a case of Paget's disease in a Chinese woman, incidentally diagnosed on a bone scan. This was confirmed by clinical history, biochemistry and imaging findings using other modalities. Although bone scans are commonly performed to diagnose traumatic occult fractures and bone metastases, they can also be used to diagnose metabolic bone diseases, such as osteoporotic fractures, and to evaluate Paget's disease. It can also diagnose acute fractures secondary to renal osteodystrophy and osteomalacia Copyright (2005) Blackwell Publishing Asia Pty Ltd

  17. An insight in to Paget′s disease of bone

    Directory of Open Access Journals (Sweden)

    Robin Sabharwal

    2014-01-01

    Full Text Available Paget′s disease of bone (PDB is a common disorder which may affect one or many bones. Although many patients are asymptomatic, a variety of symptoms and complications may occur. PDB is a focal disorder of bone turnover characterized by excessive bone resorption coupled with bone formation. PDB begins with a period of increased osteoclastic activity and bone resorption, followed by increased osteoblast production of woven bone that is poorly mineralized. In the final phase of the disease process, dense cortical and trabecular bone deposition predominates, but the bone is sclerotic and poorly organized and lacks the structural integrity and strength of normal bone. This article briefly reviews the etiopathogenesis, clinical radiographic and histological features of Paget′s disease.

  18. Developmental Programming of Adult Disease: Reprogramming by Melatonin?

    Directory of Open Access Journals (Sweden)

    You-Lin Tain

    2017-02-01

    Full Text Available Adult-onset chronic non-communicable diseases (NCDs can originate from early life through so-called the “developmental origins of health and disease” (DOHaD or “developmental programming”. The DOHaD concept offers the “reprogramming” strategy to shift the treatment from adulthood to early life, before clinical disease is apparent. Melatonin, an endogenous indoleamine produced by the pineal gland, has pleiotropic bioactivities those are beneficial in a variety of human diseases. Emerging evidence support that melatonin is closely inter-related to other proposed mechanisms contributing to the developmental programming of a variety of chronic NCDs. Recent animal studies have begun to unravel the multifunctional roles of melatonin in many experimental models of developmental programming. Even though some progress has been made in research on melatonin as a reprogramming strategy to prevent DOHaD-related NCDs, future human studies should aim at filling the translational gap between animal models and clinical trials. Here, we review several key themes on the reprogramming effects of melatonin in DOHaD research. We have particularly focused on the following areas: mechanisms of developmental programming; the interrelationship between melatonin and mechanisms underlying developmental programming; pathophysiological roles of melatonin in pregnancy and fetal development; and insight provided by animal models to support melatonin as a reprogramming therapy. Rates of NCDs are increasing faster than anticipated all over the world. Hence, there is an urgent need to understand reprogramming mechanisms of melatonin and to translate experimental research into clinical practice for halting a growing list of DOHaD-related NCDs.

  19. Developmental origins of health and disease

    National Research Council Canada - National Science Library

    Gluckman, Peter D; Hanson, Mark A

    2006-01-01

    ... development and the onset of many chronic diseases such as coronary heart disease, diabetes and osteoporosis also raises important public health issues. Another fascinating theme in the book concerns evolutionary developmental biology and how the 'evo-devo' debate can cast light on these concepts. Clinicians and basic scientists alike will find this an ...

  20. Intractable Diseases Treated with Intra-Bone Marrow-Bone Marrow Transplantation

    Directory of Open Access Journals (Sweden)

    Ming eLi

    2014-09-01

    Full Text Available Bone marrow transplantation (BMT is used to treat hematological disorders, autoimmune diseases and lymphoid cancers. Intra bone marrow-BMT (IBM-BMT has been proven to be a powerful strategy for allogeneic BMT due to the rapid hematopoietic recovery and the complete restoration of T cell functions. IBM-BMT not only replaces hematopoietic stem cells but also mesenchymal stem cells (MSMCs. MSMCs are multi-potent stem cells that can be isolated from bone marrow, umbilical cord blood, and adipose tissue. MSMCs play an important role in the support of hematopoiesis, and modify and influence the innate and adaptive immune systems. MSMCs also differentiate into mesodermal, endodermal and ectodermal lineage cells to repair tissues. This review aims to summarize the functions of bone marrow-derived- MSMCs, and the treatment of intractable diseases such as rheumatoid arthritis and malignant tumors with IBM-BMT.

  1. A murine model of human myeloma bone disease

    NARCIS (Netherlands)

    Garrett, I.R.; Dallas, S.; Radl, J.; Mundy, G.R.

    1997-01-01

    Myeloma causes a devastating and unique form of osteolytic bone disease. Although osteoclast activation is responsible for bone destruction, the precise mechanisms by which myeloma cells increase osteoclast activity have not been defined. An animal model of human myeloma bone disease mould help in

  2. Bone scintigraphy in a case of Ollier's disease

    Energy Technology Data Exchange (ETDEWEB)

    Otsuka, Nobuaki; Ito, Yasuhiko; Morita, Rikushi [Kawasaki Medical School, Kurashiki, Okayama (Japan)

    1983-11-01

    Bone scintigraphy with sup(99m)Tc-MDP was performed on a case with a Ollier's disease. Compared with Bone X-ray examinations, increased activity is noted in the ribs, hands (esp. left), left humerus, left tibia and left foot. Tumor scintigraphy with /sup 67/Ga-citrate shows slightly increased accumulation in comparison with sup(99m)Tc-MDP findings. However, apparent change was not noted compared with the previous scans. So, malignant change was negative. Malignant bone tumors usually show high activity, but some benign tumors also show high uptake. So, a differential diagnosis of bone disease using sup(99m)Tc-phosphorous compounds is occasionally difficult. In case of Ollier's disease, a follow-up bone scintigraphy is useful for evaluation of tumor growth, because malignant changes were accompanied by intensive uptake of sup(99m)Tc-MDP. Also, /sup 67/Ga-study is necessary for the differentiation of bone disease.

  3. Histologic diagnosis of metabolic bone diseases: bone histomorphometry

    Directory of Open Access Journals (Sweden)

    L. Dalle Carbonare

    2011-09-01

    Full Text Available Histomorphometry or quantitative histology is the analysis on histologic sections of bone resorption parameters, formation and structure. It is the only technique that allows a dynamic evaluation of the activity of bone modelling after labelling with tetracycline. Moreover, the new measurement procedures through the use of the computer allow an assessment of bone microarchitecture too. Histomorphometric bone biopsy is a reliable and well-tolerated procedure. Complications are reported only in 1% of the subjects (hematoma, pain, transient neuralgia. Histomorphometry is used to exclude or confirm the diagnosis of osteomalacia. It is employed in the evaluation of bone damage associated with particular treatments (for example, anticonvulsants or in case of rare bone diseases (osteogenesis imperfecta, systemic mastocytosis. It is also an essential approach when clinical, biochemical and other diagnostic data are not consistent. Finally, it is a useful method to understand the pathophysiologic mechanisms of drugs. The bone sample is taken at the level of iliac crest under local anesthesia. It is then put into methyl-metacrilate resin where the sections are prepared for the microscopic analysis of the various histomorphometric parameters.

  4. Protecting Bone Health in Pediatric Rheumatic Diseases: Pharmacological Considerations.

    Science.gov (United States)

    Zhang, Yujuan; Milojevic, Diana

    2017-06-01

    Bone health in children with rheumatic conditions may be compromised due to several factors related to the inflammatory disease state, delayed puberty, altered life style, including decreased physical activities, sun avoidance, suboptimal calcium and vitamin D intake, and medical treatments, mainly glucocorticoids and possibly some disease-modifying anti-rheumatic drugs. Low bone density or even fragility fractures could be asymptomatic; therefore, children with diseases of high inflammatory load, such as systemic onset juvenile idiopathic arthritis, juvenile dermatomyositis, systemic lupus erythematosus, and those requiring chronic glucocorticoids may benefit from routine screening of bone health. Most commonly used assessment tools are laboratory testing including serum 25-OH-vitamin D measurement and bone mineral density measurement by a variety of methods, dual-energy X-ray absorptiometry as the most widely used. Early disease control, use of steroid-sparing medications such as disease-modifying anti-rheumatic drugs and biologics, supplemental vitamin D and calcium, and promotion of weight-bearing physical activities can help optimize bone health. Additional treatment options for osteoporosis such as bisphosphonates are still controversial in children with chronic rheumatic diseases, especially those with decreased bone density without fragility fractures. This article reviews common risk factors leading to compromised bone health in children with chronic rheumatic diseases and discusses the general approach to prevention and treatment of bone fragility.

  5. [Impact of thyroid diseases on bone].

    Science.gov (United States)

    Tsourdi, E; Lademann, F; Siggelkow, H

    2018-05-09

    Thyroid hormones are key regulators of skeletal development in childhood and bone homeostasis in adulthood, and thyroid diseases have been associated with increased osteoporotic fractures. Hypothyroidism in children leads to an impaired skeletal maturation and mineralization, but an adequate and timely substitution with thyroid hormones stimulates bone growth. Conversely, hyperthyroidism at a young age accelerates skeletal development, but may also cause short stature because of a premature fusion of the growth plates. Hypothyroidism in adults causes an increase in the duration of the remodeling cycle and, thus, leads to low bone turnover and enhanced mineralization, but an association with a higher fracture risk is less well established. In adults, a surplus of thyroid hormones enhances bone turnover, mostly due to an increased bone resorption driven by osteoclasts. Thus, hyperthyroidism is a well-recognized cause of high-bone turnover secondary osteoporosis, resulting in an increased susceptibility to fragility fractures. Subclinical hyperthyroidism, especially resulting from endogenous disease, also has an adverse effect on bone mineral density and is associated with fractures. In most patients with overt or subclinical hyperthyroidism restoration of the euthyroid status reverses bone loss. In postmenopausal women who receive thyroid-stimulating hormone suppression therapy because of thyroid cancer, antiresorptive treatments may be indicated. Overall, extensive data support the importance of a euthyroid status for bone mineral accrual and growth in childhood as well as maintenance of bone health in adulthood.

  6. Epigenetics and the Developmental Origins of Health and Disease#

    Science.gov (United States)

    Epigenetic programming is likely to be an important mechanism underlying the lasting influence of the developmental environment on lifelong health, a concept known as the Developmental Origins of Health and Disease (DOHaD). DNA methylation, posttranslational histone protei n modi...

  7. Radioisotopic studies of bone diseases

    International Nuclear Information System (INIS)

    Ell, P.J.

    1976-01-01

    Consideration is given to the study of bone diseases. The most used radionuclides in the skeletal investigation are analysed and a table of radiopharmaceuticals of localization in the skeleton is showed. Emphasis is given to the use of Strontium 85 and 87m, fluorine 18 and technetium 99m. The phosphate compounds labelled with Technetium 99m are studied in detail and the structures of these organic and inorganic compounds are given. A table with values of the blood clearance of those compounds is presented. The skeletal distribution of the phosphate compounds-sup(99m)Tc, as well as the abnormal scintigraphy of skeleton by means of them, are analysed. Referring to bone diseases, the benign and malignant ones are studied: a table is given of bone diseases with positive imaging to the skeleton scintigraphy in the former case and the main applications of this scintigraphy in the latter one. Emphasis is given, in all the cases, to the clinical applications of the method, with recommendations in each one. Scintigraphic imagings are presented referring to each item studied [pt

  8. The spectrum of bone disease in Jordanian hemodialysis patients

    International Nuclear Information System (INIS)

    Younes, Nidal A.; Al-Mansour, M.; Sroujieh, Ahmad S.; Wahbeh, A.; Ailabouni, W.; Hamzah, Y.; Mahafzah, W.

    2006-01-01

    To evaluate the spectrum of mineral abnormalities and bone disease (BD) in hemodialysis patients at Jordan University Hospital (JUH), Amman, Jordan. A cross-sectional study was conducted among 63 patients (38 males and 25 females), mean age 44.19 years (range 17-76 years), with chronic kidney disease (CKD) on regular hemodialysis at JUH between November 2004 and April 2005. All patients have undergone complete blood count, chemistry profile, alkaline phosphatase, serum albumin, intact parathyroid hormone (iPTH) and plain x-rays. Bone disorders were identified in 45 patients on x-rays (70%). Osteopenia was found in 43 patients (68.3%), subperiosteal resorption in 24 patients (38.3%) and metastatic calcification in 22 patients (35%). Hypocalcemia was found in 28.6% and hypercalcemia in 7.9%. All patients were taking calcium carbonate, and 55.5% of patients were on vitamin D supplements. The calcium levels in 63.5% and the phosphorus levels in 50.8% of patients were within the recommended guidelines of the National Kidney Foundation Kidney Disease Outcomes Quality Initiative (K/DOQI). Serum i-PTH level was above 300 pg/ml high turnover bone disease in 24.6% of patients, 21.3% had iPTH of 150-300 pg/ml target, and 44.3% had i-PTH levels below 100 pg/mL suggesting a dynamic bone disease. Patients with severe bone disease had a statistically significant higher iPTH levels (p<0.005). Bone disease and mineral abnormalities are common in hemodialysis patients at JUH. Earlier detection of bone disease and better overall management strategy may reduce the frequency and severity of bone disease in CKD patients in Jordan. (author)

  9. The use of bone turnover markers in chronic kidney disease-mineral and bone disorders.

    Science.gov (United States)

    Chiang, Cherie

    2017-03-01

    Bone turnover markers assist in fracture risk prediction, management and monitoring of osteoporosis in patients without chronic kidney disease (CKD). The use in CKD-mineral bone disorder (MBD) has been limited as many of these markers and breakdown products are renally excreted, including the most commonly used and well standardized procollagen type I N propeptide and C-terminal cross-linking telopeptide of type I collagen. Of the markers unaffected by renal function, bone specific alkaline phosphatase is associated with mortality and fracture rate in CKD subjects and is now available on several automated analysers. When used in combination with PTH, bone specific alkaline phosphatase as a bone formation marker correlated well with bone biopsy histomorphometry in predicting adynamic bone disease. Tartrate-resistant acid phosphatase 5b is a resorption marker that is under development for automation. Both high and low bone turnover in CKD-MBD patients are associated with increased fracture and mortality risk. Bone biopsy as the gold standard to differentiate between adynamic bone disease and osteitis fibrosa is limited by availability and cost. Appropriate use of bone turnover markers is vital in the decision to commence anti-resorptive agents, and to monitor efficacy in order to avoid over suppression of bone turnover, which may lead to stress fractures. Further efforts are required to develop markers unaffected by renal function with standardized cut-off values and fracture as well as vascular calcification end-points. © 2017 Asian Pacific Society of Nephrology.

  10. HIV and bone disease.

    Science.gov (United States)

    Stone, Benjamin; Dockrell, David; Bowman, Christine; McCloskey, Eugene

    2010-11-01

    Advances in management have resulted in a dramatic decline in mortality for individuals infected with human immunodeficiency virus (HIV). This decrease in mortality, initially the result of improved prophylaxis and treatment of opportunistic infections but later mediated by the use of highly-active antiretroviral therapy (HAART) has led to the need to consider long-term complications of the disease itself, or its treatment. Bone disease is increasingly recognised as a concern. The prevalence of reduced BMD and possibly also fracture incidence are increased in HIV-positive individuals compared with HIV-negative controls. There are many potential explanations for this - an increased prevalence of established osteoporosis risk factors in the HIV-positive population, a likely direct effect of HIV infection itself and a possible contributory role of ARV therapy. At present, the assessment of bone disease and fracture risk remains patchy, with little or no guidance on identifying those at increased risk of reduced BMD or fragility fracture. Preventative and therapeutic strategies with bone specific treatments need to be developed. Limited data suggest bisphosphonates may be beneficial in conjunction with vitamin D and calcium supplementation in the treatment of reduced BMD in HIV-infected patients but larger studies of longer duration are needed. The safety and cost-effectiveness of these and other treatments needs to be evaluated. Copyright © 2010. Published by Elsevier Inc.

  11. A case of osteomyelitis of mandibular bone in Kimura's disease

    Energy Technology Data Exchange (ETDEWEB)

    Kinugawa, Hiroshi; Shimada, Fumihiko; Sai, Yoshikazu; Amakata, Yoshikuni; Yoshitake, Kazusada [Shiga Univ. of Medical Science, Otsu (Japan)

    1991-07-01

    We experienced a case of osteomyelitis of mandibular bone in Kimura's disease. The patient received radiation therapy to head and neck area against the same disease. Bone tissue which received radiation therapy had developed malnutrition as side effect of radiation, and osteomyelitis was induced by infection from the teeth. Even in the benign soft tissue disease like Kimura's disease, especially after radiation therapy, pathologic fracture of bone may happen due to bone damage caused by radiation. (author).

  12. Developmental origin of immune diseases-Environmental influences

    DEFF Research Database (Denmark)

    Strom, M.; Halldorsson, T. I.; Hansen, S.

    2015-01-01

    (PCBs), organochlorine pesticides, andmorerecently perfluoroalkyl substances (PFAS). Developmental exposures to PCBs have, for example, been associated with both otitis media and lower respiratory infections. Evidence regarding asthma and allergic disease is less well established, partly due to lack......Experimental studies have shown that developmental exposures to environmental chemicals may have long lasting adverse consequences for the development of the immune system. In humans such findings have mostly been explored for persistent organic pollutants (POPs) including polychlorinated biphenyls...... years of age we have examined the long term consequences of in utero exposure to POPs on offspring use of asthma medication and biomarkers of allergic airway disease. Using registry based information on offspring use of asthma medication until 20 years of age, prenatal exposures to PCB-118...

  13. Progress in the clinical imaging research of bone diseases on ankle and foot sesamoid bones and accessory ossicles

    Science.gov (United States)

    Li, Xiaozhong; Shi, Lenian; Liu, Taiyun; Wang, Lin

    2012-01-01

    Summary Sesamoid bones and accessory ossicles are research focuses of foot and ankle surgery. Pains of the foot and ankle are related to sesamoid bones and accessory ossicles. The specific anatomical and functional relationship of sesamoid bones and accessory ossicles can cause such bone diseases as the dislocation of sesamoid bones and accessory bones, infection, inflammation and necrosis of sesamoid bones, cartilage softening, tenosynovitis of sesamoid bones and the sesamoid bone syndrome. However, these bone diseases are often misdiagnosed or mistreated. In patients with trauma history, relevant diseases of sesamoid bones and accessory ossicles as above mentioned are highly probable to be misdiagnosed as avulsion fractures. In such cases, radiographic findings may provide a basis for clinical diagnosis. PMID:25343083

  14. Celiac Disease in Patients with Cystic Fibrosis-Related Bone Disease

    Directory of Open Access Journals (Sweden)

    Melissa S. Putman

    2017-01-01

    Full Text Available Both cystic fibrosis (CF and celiac disease can cause low bone mineral density (BMD and fractures. Celiac disease may occur at a higher frequency in patients with CF than the general population, and symptoms of these conditions may overlap. We report on two patients presenting with CF-related bone disease in the past year who were subsequently found to have concurrent celiac disease. Because adherence to a gluten-free diet may improve BMD in patients with celiac disease, this could have important implications for treatment. Clinicians should consider screening for celiac disease in patients with CF who have low BMD, worsening BMD in the absence of other risk factors, and/or difficult to treat vitamin D deficiency.

  15. Paget's disease of the bone after treatment with Denosumab

    DEFF Research Database (Denmark)

    Schwarz, Peter; Rasmussen, Anne Qvist; Kvist, Torben M

    2012-01-01

    Bone affection in Paget's disease is characterized by increased bone turnover localised at one or more sites of the skeleton. Bisphosphonates are the drugs of choice when treating the increased bone turnover in Paget's disease. However, in cases of decreased kidney function only less effective tr...... treatments that are available as bisphosphonates are contraindicated in these patients. We present a case of a male patient aged 86 years with GFR of 11 mL/min and Paget's disease successfully treated by Denosumab. The bone turnover and pain decreased upon treatment....

  16. Investigations of Diabetic Bone Disease

    DEFF Research Database (Denmark)

    Linde, Jakob Starup

    measures in patients with diabetes. This PhD thesis reports the results of two systematic reviews and a meta-analysis, a state-of-the-art intervention study, a clinical cross-sectional study and a registry-based study all examining the relationship between diabetes, glucose, and bone. Patients with type 2......Diabetes mellitus is associated with an increased risk of fracture with and current fracture predictors underestimate fracture risk in both type 1 and type 2 diabetes. Thus, further understanding of the underlying causes of diabetic bone disease may lead to better fracture predictors and preventive...... diabetes had lower bone turnover markers compared to patients with type 1 diabetes and bone mineral density and tissue stiffness were increased in patients with type 2 diabetes. The bone turnover markers were inversely associated with blood glucose in patients with diabetes and both an oral glucose...

  17. Dense bone - too much bone: Radiological considerations and differential diagnosis. Pt. 2

    Energy Technology Data Exchange (ETDEWEB)

    Jacobson, H.G.

    1985-02-01

    In conclusion, the attempt has been made to demonstrate that three major forms of new bone formation exist: reactive, neoplastic, and the newborn or relative skeletal sclerosis in congenital (developmental) disorders. A classification of skeletal disorders has been presented and four major groups have been selected from the nine categories in this classification. These are: congenital-developmental, metabolic and endocrine, benign neoplasms and malignant neoplasms. In all four categories a large group of entities which may present with new bone (sclerosis) are listed and are discussed in some, but limited, detail. A number of these entities in each of the four categories are illustrated. Some difficulty is encountered in considering the mechanisms for the production of bony sclerosis in the group of congenital-developmental disorders. In such entities as osteopetrosis, the overproduction of cartilage cords and subsequent excessive mineralization is known to be responsible for the dense bone. However, in various skeletal dysplasias (e.g. pyknodysostosis, van Bucherm disease), the exact mechanism for the development of the diffuse sclerotic process is not clearly understood. In the metabolic and endocrine category, the situation as to mechanism is less unclear in considering the reason for the development of bony sclerosis. Yet even in evaluating disorders such as renal osteodystrophy, the reactive bony sclerosis in the presence of secondary hyperparathyroidism and osteomalacia is a source of speculation with no definite proof, as yet.

  18. Bone disease in haemoglobin disorders

    Directory of Open Access Journals (Sweden)

    Ersi Voskaridou

    2013-03-01

    Full Text Available Bone disease represents a prominent cause of morbidity in patients with thalassaemia and other haemoglobin disorders. The delay in sexual maturation, the presence of diabetes and hypothyroidism, the parathyroid gland dysfunction, the haemolytic anaemia, the progressive marrow expansion, the iron toxicity on osteoblasts, the iron chelators, and the deficiency of growth hormone or insulin growth factors have been identified as major causes of osteoporosis in thalassaemia. Adequate hormonal replacement, effective iron chelation, improvement of hemoglobin levels, calcium and vitamin D administration, physical activity, and smoking cessation are the main to-date measures for the management of the disease. During the last decade, novel pathogenetic data suggest that the reduced osteoblastic activity, which is believed to be the basic mechanism of bone loss in thalassemia, is accompanied by a comparable or even greater increase in bone resorption. Therefore, potent inhibitors of osteoclast activation, such as the aminobisphosphonates, arise as key drugs for the management of osteoporosis in thalassaemia patients and other haemoglobin disorders.

  19. Measurement of lumbar spine bone mineral content using dual photon absorptiometry. Usefulness in metabolic bone diseases

    International Nuclear Information System (INIS)

    Delmas, P.D.; Duboeuf, F.; Braillon, P.; Meunier, P.J.

    1988-01-01

    Measurement of bone density using an accurate, non-invasive method is a crucial step in the clinical investigation of metabolic bone diseases, especially osteoporosis. Among the recently available techniques, measurement of lumbar spine bone mineral content (BMC) using dual photon absorptiometry appears as the primary method because it is simple, inexpensive, and involves low levels of radiation exposure. In this study, we measured the BMC in 168 normal adults and 95 patients. Results confirmed the good reproducibility and sensitivity of this technique for quantifying bone loss in males and females with osteoporosis. Significant bone loss was found in most females with primary hyperparathyroidism. Dual photon absorptiometry can also be used for quantifying increases in bone mass in Paget disease of bone and diffuse osteosclerosis. Osteomalacia is responsible for a dramatic fall in BMC reflecting lack of mineralization of a significant portion of the bone matrix, a characteristic feature in this disease. Furthermore, in addition to being useful for diagnostic purposes and for evaluation of the vertebral fracture risk, lumbar spine absorptiometry can be used for monitoring the effectiveness of bone-specific treatments [fr

  20. Measurement of lumbar spine bone mineral content using dual photon absorptiometry. Usefulness in metabolic bone diseases

    Energy Technology Data Exchange (ETDEWEB)

    Delmas, P.D.; Duboeuf, F.; Braillon, P.; Meunier, P.J.

    1988-06-02

    Measurement of bone density using an accurate, non-invasive method is a crucial step in the clinical investigation of metabolic bone diseases, especially osteoporosis. Among the recently available techniques, measurement of lumbar spine bone mineral content (BMC) using dual photon absorptiometry appears as the primary method because it is simple, inexpensive, and involves low levels of radiation exposure. In this study, we measured the BMC in 168 normal adults and 95 patients. Results confirmed the good reproducibility and sensitivity of this technique for quantifying bone loss in males and females with osteoporosis. Significant bone loss was found in most females with primary hyperparathyroidism. Dual photon absorptiometry can also be used for quantifying increases in bone mass in Paget disease of bone and diffuse osteosclerosis. Osteomalacia is responsible for a dramatic fall in BMC reflecting lack of mineralization of a significant portion of the bone matrix, a characteristic feature in this disease. Furthermore, in addition to being useful for diagnostic purposes and for evaluation of the vertebral fracture risk, lumbar spine absorptiometry can be used for monitoring the effectiveness of bone-specific treatments.

  1. Unusual radiological features in Paget's disease of bone

    International Nuclear Information System (INIS)

    Moore, T.E.; Kathol, M.H.; El-Khoury, G.Y.; Walker, C.W.; Gendall, P.W.; Whitten, C.G.

    1994-01-01

    The radiological diagnosis of Paget's disease of bone is usually straightforward because most cases conform to well-established classic descriptions. Diagnosis becomes more difficult, however, when radiological appearances are not typical or other disease processes mask or alter the behavior of Paget's disease. Examples are presented to illustrate four categories of unusual radiological presentation of Paget's disease; (1) unusual disease progression, (2) massive post-immobilization lysis, (3) metastatic spread to pagetic bone, and (4) vertebral end-plate destruction that mimics infection. (orig.)

  2. Bone turnover markers in patients with type 1 Gaucher disease

    Directory of Open Access Journals (Sweden)

    Gaetano Giuffrida

    2012-11-01

    Full Text Available Bone complications occur frequently in Gaucher disease (GD and reduce the quality of life of these patients. Skeletal involvement is an important indication for treatment to ameliorate symptoms and reduce the risk of irreversible and debilitating disease. Bone biomarkers have been used to assess disease status and the response to therapy in a number of bone disorders. Here, we examine the literature for evidence of abnormalities in bone turnover markers in patients with type 1 GD to assess whether they might be useful for the assessment of bone involvement in GD. We have found that bone biomarkers in GD show highly variable results which do not currently support their routine use for clinical assessment of bone status, as an indication for therapy initiation, or for monitoring the response to therapy. A greater understanding of bone markers and their relation to the bone manifestations of GD is required.

  3. Developmental anomaly of the hyoid bone: an unusual cause of dysphagia

    International Nuclear Information System (INIS)

    Bayadjian, A.; Marsot-Dupuch, K.; Schmitt, E.; Chouard, Ch.; Tubiana, J.M.

    2001-01-01

    We report the case of a hyoid syndrome caused by a developmental anomaly of the second branchial cleft, presenting at adult age by dysphagia without any abnormality detected at the barium swallow and at naso-pharyngeal endoscopy, first examinations to perform in case of dysphagia. The MRI findings of this anomaly showed a hyperintense well-limited vallecular mass syndrome. The diagnosis of hyoid bone anomaly was established at spiral CT with 3-D reconstructions showing an incurvated and elongated lesser cornua causing persistent impingement on the lateral wall of the oropharynx. CT scan performed during Valsalva maneuver showed the persistence of the compression during pharyngeal insufflation. (author)

  4. Hydrated disease of bone

    International Nuclear Information System (INIS)

    Mishwani, A.H.; Ahmed, M.; Anwar, S.D.

    2003-01-01

    A case of primary hydatid disease of the right femur is reported that presented with pathological fracture and was diagnosed at the time of exploration for biopsy. The patient was treated by removal of all cysts, irrigation with colloidal solution, bone grafting and immobilization of the fracture followed by four cycles of oral Albendazole. Eosinophilia and serological tests reverted to normal but the patient died due to acute myocardial infarction six months later. This uncommon condition should be considered in the differential diagnosis of pathological fractures, bone pain or osteolytic lesions, especially in patients of rural and farmer background.(author)

  5. Skeletal scintigraphy and quantitative tracer studies in metabolic bone disease

    Science.gov (United States)

    Fogelman, Ignac

    Bone scan imaging with the current bone seeking radiopharmaceuticals, the technetium-99m labelled diphosphonates, has dramatically improved our ability to evaluate skeletal pathology. In this thesis, chapter 1 presents a review of the history of bone scanning, summarises present concepts as to the mechanism of uptake of bone seeking agents and briefly illustrates the role of bone scanning in clinical practice. In chapter 2 the applications of bone scan imaging and quantitative tracer techniques derived from the bone scan in the detection of metabolic bone disease are discussed. Since skeletal uptake of Tc-99m diphosphonate depends upon skeletal metabolism one might expect that the bone scan would be of considerable value in the assessment of metabolic bone disease. However in these disorders the whole skeleton is often diffusely involved by the metabolic process and simple visual inspection of the scan image may not reveal the uniformly increased uptake of tracer. Certain patterns of bone scan abnormality have, however, been reported in patients with primary hyperparathyroidism and renal osteo-dystrophy; the present studies extend these observations and introduce the concept of "metabolic features" which are often recognisable in conditions with generalised increased bone turnover. As an aid to systematic recognition of these features on a given bone scan image a semi-quantitative scoring system, the metabolic index, was introduced. The metabolic index allowed differentiation between various groups of patients with metabolic disorders and a control population. In addition, in a bone scan study of patients with acromegaly, it was found that the metabolic index correlated well with disease activity as measured by serum growth hormone levels. The metabolic index was, however, found to be a relatively insensitive means of identifying disease in individual patients. Patients with increased bone turnover will have an absolute increase in skeletal uptake of tracer. As a

  6. The dental implications of bisphosphonates and bone disease.

    Science.gov (United States)

    Cheng, A; Mavrokokki, A; Carter, G; Stein, B; Fazzalari, N L; Wilson, D F; Goss, A N

    2005-12-01

    In 2002/2003 a number of patients presented to the South Australian Oral and Maxillofacial Surgery Unit with unusual non-healing extraction wounds of the jaws. All were middle-aged to elderly, medically compromised and on bisphosphonates for bone pathology. Review of the literature showed similar cases being reported in the North American oral and maxillofacial surgery literature. This paper reviews the role of bisphosphonates in the management of bone disease. There were 2.3 million prescriptions for bisphosphonates in Australia in 2003. This group of drugs is very useful in controlling bone pain and preventing pathologic fractures. However, in a small number of patients on bisphosphonates, intractable, painful, non-healing exposed bone occurs following dental extractions or denture irritation. Affected patients are usually, but not always, over 55 years, medically compromised and on the potent nitrogen containing bisphosphonates pamidronate (Aredia/Pamisol), alendronate (Fosamax) and zolendronate (Zometa) for non-osteoporotic bone disease. Currently, there is no simple, effective treatment and the painful exposed bone may persist for years. The main complications are marked weight loss from difficulty in eating and severe jaw and neck infections. Possible preventive and therapeutic strategies are presented although at this time there is no evidence of their effectiveness. Dentists must ask about bisphosphonate usage for bone disease when recording medical histories and take appropriate actions to avoid the development of this debilitating condition in their patients.

  7. Re-evaluation of bone pain in patients with type 1 Gaucher disease suggests that bone crises occur in small bones as well as long bones.

    Science.gov (United States)

    Baris, Hagit N; Weisz Hubshman, Monika; Bar-Sever, Zvi; Kornreich, Liora; Shkalim Zemer, Vered; Cohen, Ian J

    2016-09-01

    Bone crises in type 1 Gaucher disease are reported in long bones and occasionally in weight bearing bones and other bones, but rarely in small bones of the hands and feet. We retrospectively examined the incidence of bone pain in patients followed at the Rabin Medical Center, Israel, before and following the initiation of enzyme replacement therapy (ERT) and evaluated them for bone crises. Of 100 type I Gaucher disease patients, 30 (30%) experienced one or more bone crises. Small bone crises represented 31.5% of all bone crises and were always preceded by crises in other bones. While the incidence of long bone crises reduced after the initiation of ERT, small bone crises increased. Almost 60% of patients with bone crises were of the N370S/84GG genotype suggesting a greater susceptibility of N370S/84GG patients to severe bone complications. These patients also underwent the greatest number of splenectomies (70.6% of splenectomised patients). Splenectomised patients showed a trend towards increased long and small bone crises after surgery. Active investigation of acute pain in the hands and feet in patients in our cohort has revealed a high incidence of small bone crises. Physicians should consider imaging studies to investigate unexplained pain in these areas. Copyright © 2015 Elsevier Inc. All rights reserved.

  8. Temporary brittle bone disease: fractures in medical care.

    Science.gov (United States)

    Paterson, Colin R

    2009-12-01

    Temporary brittle bone disease is the name given to a syndrome first reported in 1990, in which fractures occur in infants in the first year of life. The fractures include rib fractures and metaphyseal fractures which are mostly asymptomatic. The radiological features of this disorder mimic those often ascribed to typical non-accidental injury. The subject has been controversial, some authors suggesting that the disorder does not exist. This study reports five infants with typical features of temporary brittle bone disease in whom all or most of the fractures took place while in hospital. A non-accidental cause can be eliminated with some confidence, and these cases provide evidence in support of the existence of temporary brittle bone disease.

  9. A simple method of screening for metabolic bone disease

    International Nuclear Information System (INIS)

    Broughton, R.B.K.; Evans, W.D.

    1982-01-01

    The purpose of this investigation was to find a simple method -to be used as an adjunct to the conventional bone scintigram- that could differentiate between decreased bone metabolism or mass, i.e., osteoporosis -normal bone- and the group of conditions of increased bone metabolism or mass namely, osteomalacia, renal osteodystrophy, hyperparathyroidism and Paget's disease. The Fogelman's method using the bone to soft tissue ratios from region of interest analysis at 4 hours post injection, was adopted. An initial experience in measuring a value for the count rate density in lumbar vertebrae at 1 hr post injection during conventional bone scintigraphy appears to give a clear indication of the overall rate of bone metabolism. The advantage over whole body retention methods is that the scan performed at the end of the metabolic study will reveal localized bone disease that may otherwise not be anticipated

  10. Diversity of activity participation determines bone mineral content in the lower limbs of pre-pubertal children with developmental coordination disorder.

    Science.gov (United States)

    Fong, S S M; Vackova, D; Choi, A W M; Cheng, Y T Y; Yam, T T T; Guo, X

    2018-04-01

    This study examined the relationships between activity participation and bone mineralization in children with developmental coordination disorder. Limited participation in physical, recreational, social, and skill-based and self-improvement activities contributed to lower bone mineral content. For improved bone health, these children should participate in a variety of activities, not only physical activities. Limited activity participation in children with developmental coordination disorder (DCD) may have a negative impact on bone mineral accrual. The objectives of this study were to compare bone mineralization and activity participation patterns of pre-pubertal children with DCD and those with typical development, and to determine the association between activity participation patterns and bone mineralization in children with DCD. Fifty-two children with DCD (mean age = 7.51 years) and 61 children with typical development (mean age = 7.22 years) participated in the study. Appendicular and total body (less head) bone mineral content (BMC) and bone mineral density (BMD) were evaluated by a whole-body dual-energy X-ray absorptiometry scan. Activity participation patterns were assessed using the Children's Assessment of Participation and Enjoyment (CAPE) questionnaire. Children with DCD had lower appendicular and total body BMCs and BMDs than children with typical development overall (p accounting for the effects of age, sex, height, lean mass, and fat mass, the total activity diversity score remained independently associated with leg BMC in children with DCD, explaining 5.1% of the variance (p = 0.030). However, the physical activity diversity score was no longer associated with leg BMC (p = 0.090). Diversity of activity participation and bone mineralization were lower in pre-pubertal children with DCD. Decreased total activity participation diversity was a contributing factor to lower BMC in the legs of children with DCD.

  11. Celiac disease: A missed cause of metabolic bone disease

    Directory of Open Access Journals (Sweden)

    Ashu Rastogi

    2012-01-01

    Full Text Available Introduction: Celiac disease (CD is a highly prevalent autoimmune disease. The symptoms of CD are varied and atypical, with many patients having no gastrointestinal symptoms. Metabolic bone disease (MBD is a less recognized manifestation of CD associated with spectrum of musculoskeletal signs and symptoms, viz. bone pains, proximal muscle weakness, osteopenia, osteoporosis, and fracture. We here report five patients who presented with severe MBD as the only manifestation of CD. Materials and Methods: Records of 825 patients of CD diagnosed during 2002-2010 were retrospectively analyzed for clinical features, risk factors, signs, biochemical, and radiological parameters. Results: We were able to identify five patients (0.6% of CD who had monosymptomatic presentation with musculoskeletal symptoms and signs in the form of bone pains, proximal myopathy, and fragility fractures without any gastrointestinal manifestation. All the five patients had severe MBD in the form of osteopenia, osteoporosis, and fragility fractures. Four of the five patients had additional risk factors such as antiepileptic drugs, chronic alcohol consumption, malnutrition, and associated vitamin D deficiency which might have contributed to the severity of MBD. Conclusion: Severe metabolic disease as the only presentation of CD is rare. Patients show significant improvement in clinical, biochemical, and radiological parameters with gluten-free diet, calcium, and vitamin D supplementation. CD should be looked for routinely in patients presenting with unexplained MBD.

  12. vanishing bone disease in a tertiary teaching hospital in uganda

    African Journals Online (AJOL)

    prior to the above presentation and review of systems were unremarkable. General examination revealed a ... syndrome or disease, massive osteolysis, disappearing bone disease, vanishing bone disease, idiopathic ... patient symptoms and anatomic location. Medical treatment involves, radiation therapy, anti-osteoclastic.

  13. Optimal management of bone mineral disorders in chronic kidney disease and end stage renal disease.

    Science.gov (United States)

    Lundquist, Andrew L; Nigwekar, Sagar U

    2016-03-01

    The review summarizes recent studies on chronic kidney disease-mineral bone disorders, with a focus on new developments in disease management. The term chronic kidney disease-mineral bone disorder has come to describe an increasingly complex network of alterations in minerals and skeletal disorders that contribute to the significant cardiovascular morbidity and mortality seen in patients with chronic kidney disease and end stage renal disease. Clinical studies continue to suggest associations with clinical outcomes, yet current clinical trials have failed to support causality. Variability in practice exists as current guidelines for management of mineral bone disorders are often based on weak evidence. Recent studies implicate novel pathways for therapeutic intervention in clinical trials. Mineral bone disorders in chronic kidney disease arise from alterations in a number of molecules in an increasingly complex physiological network interconnecting bone and the cardiovascular system. Despite extensive associations with improved outcomes in a number of molecules, clinical trials have yet to prove causality and there is an absence of new therapies available to improve patient outcomes. Additional clinical trials that can incorporate the complexity of mineral bone disorders, and with the ability to intervene on more than one pathway, are needed to advance patient care.

  14. DIAGNOSTIC ASPECTS OF PAGET’S DISEASE OF BONE IN CLINICAL PRACTICE

    Directory of Open Access Journals (Sweden)

    I. B. Bashkova

    2017-01-01

    Full Text Available Paget’s disease of bone (PDB is a chronic localized skeletal disease that belongs to a group of metabolic osteopathies and is characterized by impaired bone remodeling to form foci of increased bone resorption followed by replacement with an excessive amount of defective, less durable bone that is prone to deformities and pathologic fractures. The course of PDB shows three stages: rarefaction, compaction, and coarse-trabecular remodeling – each of which is characterized by certain clinical, biochemical, and radiological manifestations. The majority of the clinical manifestations of the disease are associated with skeletal injury. The disease is characterized by the appearance of bone and joint pain in case of secondary osteoarthritis, bone deformities, pathological fractures, hearing loss due to damage to the skull bones, etc. In many patients, the disease is asymptomatic and detected incidentally after finding a high serum alkaline phosphatase activity or during bone X-ray for any pathological processes, but it can be diagnosed fairly late in the development of complications, as shown in the clinical examples. A combination of clinical, biochemical, morphological data and radiological findings allows for a diagnosis. The use of bisphosphonates is the method of choice for the treatment of PDB. 

  15. Engineering 3D Models of Tumors and Bone to Understand Tumor-Induced Bone Disease and Improve Treatments

    Science.gov (United States)

    Kwakwa, Kristin A.; Vanderburgh, Joseph P.; Guelcher, Scott A.

    2018-01-01

    Purpose of Review Bone is a structurally unique microenvironment that presents many challenges for the development of 3D models for studying bone physiology and diseases, including cancer. As researchers continue to investigate the interactions within the bone microenvironment, the development of 3D models of bone has become critical. Recent Findings 3D models have been developed that replicate some properties of bone, but have not fully reproduced the complex structural and cellular composition of the bone microenvironment. This review will discuss 3D models including polyurethane, silk, and collagen scaffolds that have been developed to study tumor-induced bone disease. In addition, we discuss 3D printing techniques used to better replicate the structure of bone. Summary 3D models that better replicate the bone microenvironment will help researchers better understand the dynamic interactions between tumors and the bone microenvironment, ultimately leading to better models for testing therapeutics and predicting patient outcomes. PMID:28646444

  16. The impact of thyroid diseases on bone metabolism and fracture risk.

    Science.gov (United States)

    Amashukeli, M; Giorgadze, E; Tsagareli, M; Nozadze, N; Jeiranashvili, N

    2010-01-01

    Osteoporosis is a systemic skeletal disease characterized by low bone mass and microarchitectural deterioration of bone tissue, leading to enhanced bone fragility and a consequent increase in fracture risk. One of the leading causes of secondary osteoporosis are thyroid diseases; this fact carries special importance for Georgia because of thyroid disease prevalence in Georgian population. In the present article we discuss the mechanisms, by which thyroid hormones and thyroid stimulating hormone (TSH) act on bone. We also present the data of meta-analysis of large studies, which demonstrate the complex relationship between the thyroid diseases and bone mineral density as well as the fracture risk; namely by overt and subclinical thyrotoxicosis, hypothyroidism and the treatment with the suppressive doses of levothyroxine. Beside that, we review the related data and the possible reasons, why different treatment regimens of Grave's disease: conservative, operative and radioiodine are related to different fracture risks. Finally, we discuss briefly the practical aspects of the treatment of secondary osteoporosis, related with thyroid diseases.

  17. New developmental evidence clarifies the evolution of wrist bones in the dinosaur-bird transition.

    Science.gov (United States)

    Botelho, João Francisco; Ossa-Fuentes, Luis; Soto-Acuña, Sergio; Smith-Paredes, Daniel; Nuñez-León, Daniel; Salinas-Saavedra, Miguel; Ruiz-Flores, Macarena; Vargas, Alexander O

    2014-09-01

    From early dinosaurs with as many as nine wrist bones, modern birds evolved to develop only four ossifications. Their identity is uncertain, with different labels used in palaeontology and developmental biology. We examined embryos of several species and studied chicken embryos in detail through a new technique allowing whole-mount immunofluorescence of the embryonic cartilaginous skeleton. Beyond previous controversy, we establish that the proximal-anterior ossification develops from a composite radiale+intermedium cartilage, consistent with fusion of radiale and intermedium observed in some theropod dinosaurs. Despite previous claims that the development of the distal-anterior ossification does not support the dinosaur-bird link, we found its embryonic precursor shows two distinct regions of both collagen type II and collagen type IX expression, resembling the composite semilunate bone of bird-like dinosaurs (distal carpal 1+distal carpal 2). The distal-posterior ossification develops from a cartilage referred to as "element x," but its position corresponds to distal carpal 3. The proximal-posterior ossification is perhaps most controversial: It is labelled as the ulnare in palaeontology, but we confirm the embryonic ulnare is lost during development. Re-examination of the fossil evidence reveals the ulnare was actually absent in bird-like dinosaurs. We confirm the proximal-posterior bone is a pisiform in terms of embryonic position and its development as a sesamoid associated to a tendon. However, the pisiform is absent in bird-like dinosaurs, which are known from several articulated specimens. The combined data provide compelling evidence of a remarkable evolutionary reversal: A large, ossified pisiform re-evolved in the lineage leading to birds, after a period in which it was either absent, nonossified, or very small, consistently escaping fossil preservation. The bird wrist provides a modern example of how developmental and paleontological data illuminate

  18. Low Bone Mineral Density Risk Factors and Testing Patterns in Institutionalized Adults with Intellectual and Developmental Disabilities

    Science.gov (United States)

    Hess, Mailee; Campagna, Elizabeth J.; Jensen, Kristin M.

    2018-01-01

    Background: Adults with intellectual or developmental disability (ID/DD) have multiple risks for low bone mineral density (BMD) without formal guidelines to guide testing. We sought to identify risk factors and patterns of BMD testing among institutionalized adults with ID/DD. Methods: We evaluated risk factors for low BMD (Z-/T-score < -1) and…

  19. Radiological diagnostic in cat stratch disease and bone lesions - a case report

    International Nuclear Information System (INIS)

    Abreu, Marcelo Rodrigues de

    1999-01-01

    Cat scratch disease is not a common disease in immunocompetent patients. Its association with bone lesions is rare. A patient with bone complain and radiologic alterations of bone lesion must be investigated for this disease. A simple story can make the differential diagnosis with more complex disease like Ewing sarcoma or eosinophilic granuloma. (author)

  20. Early Developmental Conditioning of Later Health and Disease: Physiology or Pathophysiology?

    Science.gov (United States)

    Hanson, M. A.; Gluckman, P. D.

    2014-01-01

    Extensive experimental animal studies and epidemiological observations have shown that environmental influences during early development affect the risk of later pathophysiological processes associated with chronic, especially noncommunicable, disease (NCD). This field is recognized as the developmental origins of health and disease (DOHaD). We discuss the extent to which DOHaD represents the result of the physiological processes of developmental plasticity, which may have potential adverse consequences in terms of NCD risk later, or whether it is the manifestation of pathophysiological processes acting in early life but only becoming apparent as disease later. We argue that the evidence suggests the former, through the operation of conditioning processes induced across the normal range of developmental environments, and we summarize current knowledge of the physiological processes involved. The adaptive pathway to later risk accords with current concepts in evolutionary developmental biology, especially those concerning parental effects. Outside the normal range, effects on development can result in nonadaptive processes, and we review their underlying mechanisms and consequences. New concepts concerning the underlying epigenetic and other mechanisms involved in both disruptive and nondisruptive pathways to disease are reviewed, including the evidence for transgenerational passage of risk from both maternal and paternal lines. These concepts have wider implications for understanding the causes and possible prevention of NCDs such as type 2 diabetes and cardiovascular disease, for broader social policy and for the increasing attention paid in public health to the lifecourse approach to NCD prevention. PMID:25287859

  1. Heterogeneous pattern of bone disease in adult type 1 Gaucher disease: clinical and pathological correlates.

    Science.gov (United States)

    van Dussen, L; Lips, P; van Essen, H W; Hollak, C E M; Bravenboer, N

    2014-09-01

    Gaucher disease (GD) is a lysosomal storage disorder characterized by accumulation of glucosylceramide in macrophages, so-called Gaucher cells, as a result of a deficiency of the lysosomal enzyme glucocerebrosidase. Bone complications are an important cause of morbidity of GD and are thought to result from imbalance in bone remodeling. Bone manifestations among GD patients demonstrate a large variation including increased osteoclastic bone resorption, low bone formation and osteonecrosis. The purpose of the current case series is to describe the histological features observed in undecalcified bone samples, obtained from three GD patients, and evaluate the relationship with clinical features in these patients. Bone fragments were obtained from three adult type 1 GD patients with variable degrees of bone disease during orthopedic surgery. Specimens were embedded without prior decalcification in methylmethacrylate and prepared for histology according to standardized laboratory procedures. Histology revealed a heterogeneous pattern of bone involvement. High cellularity of bone marrow, abundant presence of Gaucher cells (GCs) and high turnover were observed in a patient with a history of multiple bone complications, while minimal bone turnover and few GCs were detected in the mildest affected patient in this series. An intermediate picture with relatively low bone turnover and a substantial amount of Gaucher cells was demonstrated in the third, moderately affected patient. No gross abnormalities in three biochemical markers of bone turnover (osteocalcin, N-terminal propeptide of type 1 procollagen and type 1 collagen C-terminal telopeptide) were noted. Plastic embedding and subsequent Goldner and TRAP staining offered a unique possibility to study bone histological findings in GD. Our data show that bone manifestations in GD may vary both clinically as well as histologically and bone disease in GD will likely require a personalized approach. Copyright © 2014 Elsevier

  2. A probable new type of osteopenic bone disease

    Energy Technology Data Exchange (ETDEWEB)

    Widhe, Torulf L. [Department of Orthopaedics, Huddinge University Hospital (Sweden)

    2002-06-01

    A probable new type of osteopenic bone disease in two sisters and one female cousin is described. In infancy, the radiological findings were osteopenia, coxa vara, periosteal cloaking, bowing of the long bones, and flaring of the metaphyses. During growth, spinal pathology developed with compression of the vertebral bodies and scoliosis in one girl and kyphosis in another. All three children had genu valgum and two developed severe S-shaped bowing of the tibiae. Growth was stunted. Inheritance of this disorder is probably recessive. Type I and III collagen biosynthesis was normal. This condition is probably a hitherto undescribed form of osteogenesis imperfecta type III or a new bone disease. (orig.)

  3. A probable new type of osteopenic bone disease

    International Nuclear Information System (INIS)

    Widhe, Torulf L.

    2002-01-01

    A probable new type of osteopenic bone disease in two sisters and one female cousin is described. In infancy, the radiological findings were osteopenia, coxa vara, periosteal cloaking, bowing of the long bones, and flaring of the metaphyses. During growth, spinal pathology developed with compression of the vertebral bodies and scoliosis in one girl and kyphosis in another. All three children had genu valgum and two developed severe S-shaped bowing of the tibiae. Growth was stunted. Inheritance of this disorder is probably recessive. Type I and III collagen biosynthesis was normal. This condition is probably a hitherto undescribed form of osteogenesis imperfecta type III or a new bone disease. (orig.)

  4. Standard bone-age of infant and children in Korea

    International Nuclear Information System (INIS)

    Yeon, K. M.

    1996-01-01

    To evaluate the developmental status of children and adolescents, bone-age chart based on the radiograph of hand and wrist has been used in many countries. The bone-age reflects not only the functional status of various hormones but also the influence of chronic disease, and it has been used more widely than other indices such as height-weight-age table. As the standard bone-age chart has not been established in Korea, the foreign bone-age chart has been used radiographs in the clinics. To make Korean standard bone-age chart, we took the radiographs of the left hand in about 5400 children covering the whole country, and 3407 radiographs of 1830 boys and 1577 girls ranging from two months to 16 years of age were selected and analyzed for bone maturity scores by TW2-20 method. The range of age were divided into 27 groups, and the radiographs of 50th percentile score were chosen as the standard bone-ages for the median age of each group. The youngest and oldest chronological age which had the same TW2-20 score of the standard bone-age were decided as the range of variation from the median age. We hope that Korean standard bone-age chart be used as the radiological criteria in the evaluation of the developmental status in Korean children and adolescents

  5. Radionuclide imaging of bone marrow in hematologic systemic disease

    Energy Technology Data Exchange (ETDEWEB)

    Kessel, F.; Hahn, K.; Gamm, H.

    1987-02-01

    Radionuclide imaging studies of the bone marrow were carried out in 164 patients suffering from hematologic systemic disease. One third of 90 patients with Hodgkin lymphoma (HL) or Non Hodgkin lymphoma (NHL) displayed a pathological distribution pattern representing bone marrow expansion. In HL there were 17% accumulation defects caused by metastases in contrast to only 7% in NHL. Among 30 patients with chronic myelocytic leukemia bone marrow expansion was found in 60%, bone marrow displacement and aplasia 10%. Focal bone marrow defects were found in 3 patients. All patients with primary polycythemia rubra vera displayed a pathologic bone marrow distribution pattern as well as splenomegaly. All patients with acute myelocytic leukemia (AML) and one patient with an acute lymphatic leukemia (ALL) had a pathological distribution pattern with bone marrow expansion and displacement. Focal bone marrow defects were not seen. Multiple myeloma with bone marrow expansion was found in 6 of 12 patients and focal accumulation defects were found in 40%, the latter lesions being not visible or equivocal on skeletal imaging studies. Pathological changes in liver and spleen were found in a high percentage of the total collective. The results document the important clinical value of bone marrow scintigraphy among the hematologic diseases studied.

  6. Developmental Toxicity Studies with Pregabalin in Rats: Significance of Alterations in Skull Bone Morphology.

    Science.gov (United States)

    Morse, Dennis C; Henck, Judith W; Bailey, Steven A

    2016-04-01

    Pregabalin was administered to pregnant Wistar rats during organogenesis to evaluate potential developmental toxicity. In an embryo-fetal development study, compared with controls, fetuses from pregabalin-treated rats exhibited increased incidence of jugal fused to maxilla (pregabalin 1250 and 2500 mg/kg) and fusion of the nasal sutures (pregabalin 2500 mg/kg). The alterations in skull development occurred in the presence of maternal toxicity (reduced body weight gain) and developmental toxicity (reduced fetal body weight and increased skeletal variations), and were initially classified as malformations. Subsequent investigative studies in pregnant rats treated with pregabalin during organogenesis confirmed the advanced jugal fused to maxilla, and fusion of the nasal sutures at cesarean section (gestation day/postmating day [PMD] 21) in pregabalin-treated groups. In a study designed to evaluate progression of skull development, advanced jugal fused to maxilla and fusion of the nasal sutures was observed on PMD 20-25 and PMD 21-23, respectively (birth occurs approximately on PMD 22). On postnatal day (PND) 21, complete jugal fused to maxilla was observed in the majority of control and 2500 mg/kg offspring. No treatment-related differences in the incidence of skull bone fusions occurred on PND 21, indicating no permanent adverse outcome. Based on the results of the investigative studies, and a review of historical data and scientific literature, the advanced skull bone fusions were reclassified as anatomic variations. Pregabalin was not teratogenic in rats under the conditions of these studies. © 2016 Wiley Periodicals, Inc.

  7. Wnt and the Wnt signaling pathway in bone development and disease

    Science.gov (United States)

    Wang, Yiping; Li, Yi-Ping; Paulson, Christie; Shao, Jian-Zhong; Zhang, Xiaoling; Wu, Mengrui; Chen, Wei

    2014-01-01

    Wnt signaling affects both bone modeling, which occurs during development, and bone remodeling, which is a lifelong process involving tissue renewal. Wnt signals are especially known to affect the differentiation of osteoblasts. In this review, we summarize recent advances in understanding the mechanisms of Wnt signaling, which is divided into two major branches: the canonical pathway and the noncanonical pathway. The canonical pathway is also called the Wnt/β-catenin pathway. There are two major noncanonical pathways: the Wnt-planar cell polarity pathway (Wnt-PCP pathway) and the Wnt-calcium pathway (Wnt-Ca2+ pathway). This review also discusses how Wnt ligands, receptors, intracellular effectors, transcription factors, and antagonists affect both the bone modeling and bone remodeling processes. We also review the role of Wnt ligands, receptors, intracellular effectors, transcription factors, and antagonists in bone as demonstrated in mouse models. Disrupted Wnt signaling is linked to several bone diseases, including osteoporosis, van Buchem disease, and sclerosteosis. Studying the mechanism of Wnt signaling and its interactions with other signaling pathways in bone will provide potential therapeutic targets to treat these bone diseases. PMID:24389191

  8. Prevalence of metabolic bone disease in children with celiac disease is independent of symptoms at diagnosis.

    Science.gov (United States)

    Turner, Justine; Pellerin, Genevieve; Mager, Diana

    2009-11-01

    : Given dietary gluten exposure, growing children with celiac disease may experience malabsorption of nutrients, negatively affecting bone health. The purpose of this study was to determine the prevalence of low bone mass in children with celiac disease, according to the presence of symptoms at diagnosis. : A retrospective chart review of the Stollery Children's Hospital Celiac Clinic charts (April 1989-September 2007) was conducted. Bone mineral density (BMD) of the spine was measured using dual energy x-ray absorptiometry. Demographics, symptoms at presentation, and anthropometric and biochemical data relevant to bone health were recorded. : Seventy-four children (9.6 +/- 3.7 years; range 3.3-16.0 years) were included. Lumbar BMD z scores more than or equal to -1 were observed in 58 cases (65%), z scores below -1 but above -2 were observed in 14 cases (19%) and z scores less than or equal to -2 were observed in 12 cases (16%). There was no significant difference in mean lumbar BMD z scores between symptomatic and asymptomatic children (P = 0.34). When adjusted for bone age and bone surface area, BMD lumbar z score was inversely correlated with age at diagnosis (P celiac disease at diagnosis regardless of the presence of symptoms. Delayed diagnosis of children with celiac disease may increase the risk of adult osteoporosis. Appropriate screening of children at risk of celiac disease for the purpose of early diagnosis, as well as routine evaluation of bone mineral density in such children, are important to prevent long-term complications associated with poor bone health.

  9. Bioprinting and Organ-on-Chip Applications Towards Personalized Medicine for Bone Diseases.

    Science.gov (United States)

    Arrigoni, Chiara; Gilardi, Mara; Bersini, Simone; Candrian, Christian; Moretti, Matteo

    2017-06-01

    The skeleton supports and confers structure to the whole body but several pathological and traumatic conditions affect the bone tissue. Most of those pathological conditions are specific and different among different patients, such as bone defects due to traumatic injuries or bone remodeling alterations due to congenital diseases. In this context, the development of personalized therapies would be highly desirable. In recent years the advent of innovative techniques like bioprinting and microfluidic organ-on-chip raised hopes of achieving key tools helping the application of personalized therapies for bone diseases. In this review we will illustrate the latest progresses in the bioprinting of personalized bone grafts and generation of patient-specific bone-on-chip devices, describing current approaches and limitations and possible future improvements for more effective personalized bone grafts and disease models.

  10. Genetic Expression in Cystic Fibrosis Related Bone Disease. An Observational, Transversal, Cross-Sectional Study.

    Science.gov (United States)

    Ciuca, Ioana M; Pop, Liviu L; Rogobete, Alexandru F; Onet, Dan I; Guta-Almajan, Bogdan; Popa, Zoran; Horhat, Florin G

    2016-09-01

    Cystic fibrosis (CF) is the most frequent monogenic genetic disease with autosomal recessive transmission and characterized by important clinical polymorphism and significant lethal prospective. CF related bone disease occurs frequently in adults with CF. Childhood is the period of bone formation, and therefore, children are more susceptible to low bone density. Several factors like pancreatic insufficiency, hormone imbalance, and physical inactivity contribute to CF bone disease development. Revealing this would be important for prophylactic treatment against bone disease occurrence. The study was observational, transversal, with a cross-sectional design. The study included 68 children with cystic fibrosis, genotyped and monitored in the National CF Centre. At the annual assessment, besides clinical examination, biochemical evaluation for pancreatic insufficiency, and diabetes, they were evaluated for bone mineral density using dual energy X-ray absorptiometry (DXA). Twenty-six patients, aged over 10 years were diagnosed with CF bone disease, without significant gender gap. Bone disease was frequent in patients aged over 10 years with exocrine pancreatic insufficiency, carriers of severe mutations, and CF liver disease. CF carriers of a severe genotype which associates pancreatic insufficiency and CF liver disease, are more likely predisposed to low bone mineral density. Further studies should discover other significant influences in order to prevent the development of CF bone disease and an improved quality of life in cystic fibrosis children.

  11. The relationship between developmental lumbar spinal stenosis and its BMD value : comparison by single energy quantitative CT

    International Nuclear Information System (INIS)

    Kim, Hak Jin; Kim, Kun Il; Song, Keun Sung

    1996-01-01

    The purpose of this study is to evaluate the relationship between developmental lumbar spinal stenosis and its BMD value by using the single energy quantitative CT(SEQCT). Eighty normal volunteers(20-60years) were selected as a control group and 28 patients with developmental lumbar spinal stenosis were selected as a disease group. The two groups were divided into a younger (20-39 years) and an older subgroup (40-60 years), and were further divided into male and female subgroups. All the cases showed no evidence of metabolic disease, fracture, herniated nucleus pulposus, degererative spondylosis, infectious disease, tumors or had no history of absolute immobilization of more than two weeks. All underwent lumbar spine CT and SEQCT. we measured bone mineral density(BMD) at the cancellous bone of L1, 2, 3 and obtained the mean and its one standard deviation, and compared the data between each sub-group of the control and the disease group using ANOVA. There was a significant low BMD value in the younger male patient subgroup compared with the control subgroup(p<0.005). Developmental lumbar spinal stenosis in a young male may be a factor of decreasing BMD of the body of the spine

  12. The relationship between developmental lumbar spinal stenosis and its BMD value : comparison by single energy quantitative CT

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hak Jin; Kim, Kun Il; Song, Keun Sung [Pusan National Univ. Hospital, Pusan (Korea, Republic of)

    1996-03-01

    The purpose of this study is to evaluate the relationship between developmental lumbar spinal stenosis and its BMD value by using the single energy quantitative CT(SEQCT). Eighty normal volunteers(20-60years) were selected as a control group and 28 patients with developmental lumbar spinal stenosis were selected as a disease group. The two groups were divided into a younger (20-39 years) and an older subgroup (40-60 years), and were further divided into male and female subgroups. All the cases showed no evidence of metabolic disease, fracture, herniated nucleus pulposus, degererative spondylosis, infectious disease, tumors or had no history of absolute immobilization of more than two weeks. All underwent lumbar spine CT and SEQCT. we measured bone mineral density(BMD) at the cancellous bone of L1, 2, 3 and obtained the mean and its one standard deviation, and compared the data between each sub-group of the control and the disease group using ANOVA. There was a significant low BMD value in the younger male patient subgroup compared with the control subgroup(p<0.005). Developmental lumbar spinal stenosis in a young male may be a factor of decreasing BMD of the body of the spine.

  13. Bone Diseases

    Science.gov (United States)

    Your bones help you move, give you shape and support your body. They are living tissues that rebuild constantly ... childhood and your teens, your body adds new bone faster than it removes old bone. After about ...

  14. Metabolic Bone Disease in the Bariatric Surgery Patient

    Directory of Open Access Journals (Sweden)

    Susan E. Williams

    2011-01-01

    Full Text Available Bariatric surgery has proven to be a life-saving measure for some, but for others it has precipitated a plethora of metabolic complications ranging from mild to life-threatening, sometimes to the point of requiring surgical revision. Obesity was previously thought to be bone protective, but this is indeed not the case. Morbidly obese individuals are at risk for metabolic bone disease (MBD due to chronic vitamin D deficiency, inadequate calcium intake, sedentary lifestyle, chronic dieting, underlying chronic diseases, and the use of certain medications used to treat those diseases. After bariatric surgery, the risk for bone-related problems is even greater, owing to severely restricted intake, malabsorption, poor compliance with prescribed supplements, and dramatic weight loss. Patients presenting for bariatric surgery should be evaluated for MBD and receive appropriate presurgical interventions. Furthermore, every patient who has undergone bariatric surgery should receive meticulous lifetime monitoring, as the risk for developing MBD remains ever present.

  15. Current perspectives on bisphosphonate treatment in Paget’s disease of bone

    Directory of Open Access Journals (Sweden)

    Wat WZM

    2014-11-01

    Full Text Available Winnie Zee Man Wat Department of Medicine, Pamela Youde Nethersole Eastern Hospital, Chai Wan, Hong Kong Abstract: Paget’s disease of bone is a chronic metabolic bone disease with focal increase in bone turnover. The exact etiology of the disease is uncertain, although genetic and environmental factors are believed to be important. Bisphosphonate is the main class of medication being used to control disease activity via its antiresorptive effect. This review discusses the controversies concerning the use of bisphosphonates in the treatment of Paget’s disease of bone, the efficacy of different bisphosphonates in controlling disease activity, and the possible rare side effects of bisphosphonates. Symptoms are the main indication for treatment in Paget’s disease of bone. As treatment benefits in asymptomatic individuals remain controversial and nonevidence based, the decision to treat these patients should be individualized to their risk and benefit profiles. There are several trials conducted to evaluate and compare the efficacy of different regimes of bisphosphonates for treating Paget’s disease of bone. Most trials used biochemical markers rather than clinical symptoms or outcomes as parameters for comparison. Zoledronate is an attractive option as it can achieve high rates of biochemical remission and sustain long duration of suppression by a single dose. Atypical femoral fracture and osteonecrosis of the jaw are two rare and severe side effects reported, possibly related to the use of bisphosphonates in patients with osteoporosis and malignancy-induced hypercalcemia. As the regimes of bisphosphonates used for treating Paget’s disease of bone are different from those two diseases, the risks of developing these two possible side effects are expected to be very low, although this remains unknown. Vitamin D and calcium supplement should be given to patients at risk of vitamin D insufficiency when given zoledronate, as symptomatic

  16. Expression of Wnt-Inhibitors and SDF-1 in Whole Bone Marrow Biopsies in Association to the Osteolytic Bone Disease of Multiple Myeloma

    DEFF Research Database (Denmark)

    Kristensen, Ida Bruun; Christensen, Jacob Haaber; Lyng, Maria Bibi

    Expression of Wnt-Inhibitors and SDF-1 in Whole Bone Marrow Biopsies in Association to the Osteolytic Bone Disease of Multiple Myeloma......Expression of Wnt-Inhibitors and SDF-1 in Whole Bone Marrow Biopsies in Association to the Osteolytic Bone Disease of Multiple Myeloma...

  17. New developmental evidence clarifies the evolution of wrist bones in the dinosaur-bird transition.

    Directory of Open Access Journals (Sweden)

    João Francisco Botelho

    2014-09-01

    Full Text Available From early dinosaurs with as many as nine wrist bones, modern birds evolved to develop only four ossifications. Their identity is uncertain, with different labels used in palaeontology and developmental biology. We examined embryos of several species and studied chicken embryos in detail through a new technique allowing whole-mount immunofluorescence of the embryonic cartilaginous skeleton. Beyond previous controversy, we establish that the proximal-anterior ossification develops from a composite radiale+intermedium cartilage, consistent with fusion of radiale and intermedium observed in some theropod dinosaurs. Despite previous claims that the development of the distal-anterior ossification does not support the dinosaur-bird link, we found its embryonic precursor shows two distinct regions of both collagen type II and collagen type IX expression, resembling the composite semilunate bone of bird-like dinosaurs (distal carpal 1+distal carpal 2. The distal-posterior ossification develops from a cartilage referred to as "element x," but its position corresponds to distal carpal 3. The proximal-posterior ossification is perhaps most controversial: It is labelled as the ulnare in palaeontology, but we confirm the embryonic ulnare is lost during development. Re-examination of the fossil evidence reveals the ulnare was actually absent in bird-like dinosaurs. We confirm the proximal-posterior bone is a pisiform in terms of embryonic position and its development as a sesamoid associated to a tendon. However, the pisiform is absent in bird-like dinosaurs, which are known from several articulated specimens. The combined data provide compelling evidence of a remarkable evolutionary reversal: A large, ossified pisiform re-evolved in the lineage leading to birds, after a period in which it was either absent, nonossified, or very small, consistently escaping fossil preservation. The bird wrist provides a modern example of how developmental and paleontological

  18. New Developmental Evidence Clarifies the Evolution of Wrist Bones in the Dinosaur–Bird Transition

    Science.gov (United States)

    Botelho, João Francisco; Ossa-Fuentes, Luis; Soto-Acuña, Sergio; Smith-Paredes, Daniel; Nuñez-León, Daniel; Salinas-Saavedra, Miguel; Ruiz-Flores, Macarena; Vargas, Alexander O.

    2014-01-01

    From early dinosaurs with as many as nine wrist bones, modern birds evolved to develop only four ossifications. Their identity is uncertain, with different labels used in palaeontology and developmental biology. We examined embryos of several species and studied chicken embryos in detail through a new technique allowing whole-mount immunofluorescence of the embryonic cartilaginous skeleton. Beyond previous controversy, we establish that the proximal–anterior ossification develops from a composite radiale+intermedium cartilage, consistent with fusion of radiale and intermedium observed in some theropod dinosaurs. Despite previous claims that the development of the distal–anterior ossification does not support the dinosaur–bird link, we found its embryonic precursor shows two distinct regions of both collagen type II and collagen type IX expression, resembling the composite semilunate bone of bird-like dinosaurs (distal carpal 1+distal carpal 2). The distal–posterior ossification develops from a cartilage referred to as “element x,” but its position corresponds to distal carpal 3. The proximal–posterior ossification is perhaps most controversial: It is labelled as the ulnare in palaeontology, but we confirm the embryonic ulnare is lost during development. Re-examination of the fossil evidence reveals the ulnare was actually absent in bird-like dinosaurs. We confirm the proximal–posterior bone is a pisiform in terms of embryonic position and its development as a sesamoid associated to a tendon. However, the pisiform is absent in bird-like dinosaurs, which are known from several articulated specimens. The combined data provide compelling evidence of a remarkable evolutionary reversal: A large, ossified pisiform re-evolved in the lineage leading to birds, after a period in which it was either absent, nonossified, or very small, consistently escaping fossil preservation. The bird wrist provides a modern example of how developmental and paleontological

  19. The Role of Hedgehog Signaling in Tumor Induced Bone Disease

    Directory of Open Access Journals (Sweden)

    Shellese A. Cannonier

    2015-08-01

    Full Text Available Despite significant progress in cancer treatments, tumor induced bone disease continues to cause significant morbidities. While tumors show distinct mutations and clinical characteristics, they behave similarly once they establish in bone. Tumors can metastasize to bone from distant sites (breast, prostate, lung, directly invade into bone (head and neck or originate from the bone (melanoma, chondrosarcoma where they cause pain, fractures, hypercalcemia, and ultimately, poor prognoses and outcomes. Tumors in bone secrete factors (interleukins and parathyroid hormone-related protein that induce RANKL expression from osteoblasts, causing an increase in osteoclast mediated bone resorption. While the mechanisms involved varies slightly between tumor types, many tumors display an increase in Hedgehog signaling components that lead to increased tumor growth, therapy failure, and metastasis. The work of multiple laboratories has detailed Hh signaling in several tumor types and revealed that tumor establishment in bone can be controlled by both canonical and non-canonical Hh signaling in a cell type specific manner. This review will explore the role of Hh signaling in the modulation of tumor induced bone disease, and will shed insight into possible therapeutic interventions for blocking Hh signaling in these tumors.

  20. The Role of Hedgehog Signaling in Tumor Induced Bone Disease

    Energy Technology Data Exchange (ETDEWEB)

    Cannonier, Shellese A.; Sterling, Julie A., E-mail: Julie.sterling@vanderbilt.edu [Department of Veterans Affairs, Tennessee Valley Healthcare System, Nashville, TN 37235 (United States); Vanderbilt Center for Bone Biology, Department of Medicine, Division of Clinical Pharmacology Vanderbilt University, Nashville, TN 372335 (United States); Department of Cancer Biology, Vanderbilt University, Nashville, TN 37235 (United States)

    2015-08-26

    Despite significant progress in cancer treatments, tumor induced bone disease continues to cause significant morbidities. While tumors show distinct mutations and clinical characteristics, they behave similarly once they establish in bone. Tumors can metastasize to bone from distant sites (breast, prostate, lung), directly invade into bone (head and neck) or originate from the bone (melanoma, chondrosarcoma) where they cause pain, fractures, hypercalcemia, and ultimately, poor prognoses and outcomes. Tumors in bone secrete factors (interleukins and parathyroid hormone-related protein) that induce RANKL expression from osteoblasts, causing an increase in osteoclast mediated bone resorption. While the mechanisms involved varies slightly between tumor types, many tumors display an increase in Hedgehog signaling components that lead to increased tumor growth, therapy failure, and metastasis. The work of multiple laboratories has detailed Hh signaling in several tumor types and revealed that tumor establishment in bone can be controlled by both canonical and non-canonical Hh signaling in a cell type specific manner. This review will explore the role of Hh signaling in the modulation of tumor induced bone disease, and will shed insight into possible therapeutic interventions for blocking Hh signaling in these tumors.

  1. The Role of Hedgehog Signaling in Tumor Induced Bone Disease

    International Nuclear Information System (INIS)

    Cannonier, Shellese A.; Sterling, Julie A.

    2015-01-01

    Despite significant progress in cancer treatments, tumor induced bone disease continues to cause significant morbidities. While tumors show distinct mutations and clinical characteristics, they behave similarly once they establish in bone. Tumors can metastasize to bone from distant sites (breast, prostate, lung), directly invade into bone (head and neck) or originate from the bone (melanoma, chondrosarcoma) where they cause pain, fractures, hypercalcemia, and ultimately, poor prognoses and outcomes. Tumors in bone secrete factors (interleukins and parathyroid hormone-related protein) that induce RANKL expression from osteoblasts, causing an increase in osteoclast mediated bone resorption. While the mechanisms involved varies slightly between tumor types, many tumors display an increase in Hedgehog signaling components that lead to increased tumor growth, therapy failure, and metastasis. The work of multiple laboratories has detailed Hh signaling in several tumor types and revealed that tumor establishment in bone can be controlled by both canonical and non-canonical Hh signaling in a cell type specific manner. This review will explore the role of Hh signaling in the modulation of tumor induced bone disease, and will shed insight into possible therapeutic interventions for blocking Hh signaling in these tumors

  2. Current options for the treatment of Paget’s disease of the bone

    Directory of Open Access Journals (Sweden)

    Daniela Merlotti

    2009-07-01

    Full Text Available Daniela Merlotti, Luigi Gennari, Giuseppe Martini, Ranuccio NutiDepartment of Internal Medicine, Endocrine-Metabolic Sciences and Biochemistry, University of Siena, Siena, ItalyAbstract: Paget’s disease of bone (PDB is a chronic bone remodeling disorder characterized by increased osteoclast-mediated bone resorption, with subsequent compensatory increases in new bone formation, resulting in a disorganized mosaic of woven and lamellar bone at affected skeletal sites. This disease is most often asymptomatic but can be associated with bone pain or deformity, fractures, secondary arthritis, neurological complications, deafness, contributing to substantial morbidity and reduced quality of life. Neoplastic degeneration of pagetic bone is a relatively rare event, occurring with an incidence of less than 1%, but has a grave prognosis. Specific therapy for PDB is aimed at decreasing the abnormal bone turnover and bisphosphonates are currently considered the treatment of choice. These treatments are associated with a reduction in plasma alkaline phosphatase (ALP activity and an improvement in radiological and scintigraphic appearance and with a reduction in bone pain and bone deformity, Recently, the availability of newer, more potent nitrogen-containing bisphosphonates has improved treatment outcomes, allowing a more effective and convenient management of this debilitating disorder.Keywords: Paget’s disease of bone, bisphosphonates, aminobisphosphonates, bone remodeling

  3. Occult Metabolic Bone Disease in Chronic Pancreatitis

    African Journals Online (AJOL)

    2017-10-26

    Oct 26, 2017 ... KEYWORDS: Chronic pancreatitis, metabolic bone disease, osteomalacia, osteopenia ... with malabsorption, and endocrine dysfunction results in diabetes .... of insufficiency and deficiency were not assessed separately due ...

  4. Computer assisted analysis of sup(99m)Tc pyrophosphate bone uptake in Paget's disease

    International Nuclear Information System (INIS)

    Maayan, M.L.; Eisenberg, J.; Volpert, E.; Shai, F.; Mroczek, R.

    1982-01-01

    The present clinical study describes a method of evaluation of Paget's disease bone by computer assisted analysis of activity curves obtained over normal and pathological portions of the skeleton in the same patient. The data obtained lead to a differential diagnosis between Paget's and metastatic disease of the bone, as well as an evaluation of subsequent therapy. The results indicate a higher bone activity, (expressed by bone flow and bone uptake, of sup(99m)Tc pyrophosphate) in Paget's than in metastatic disease of the bone, as well as a normalization of these parameters after prolonged therapy of Paget's patients with salmon calcitonin

  5. Bones of contention: bone mineral density recovery in celiac disease--a systematic review.

    Science.gov (United States)

    Grace-Farfaglia, Patricia

    2015-05-07

    Metabolic bone disease is a frequent co-morbidity in newly diagnosed adults with celiac disease (CD), an autoimmune disorder triggered by the ingestion of dietary gluten. This systematic review of studies looked at the efficacy of the gluten-free diet, physical activity, nutrient supplementation, and bisphosphonates for low bone density treatment. Case control and cohort designs were identified from PubMed and other academic databases (from 1996 to 2015) that observed newly diagnosed adults with CD for at least one year after diet treatment using the dual-energy x-ray absorptiometry (DXA) scan. Only 20 out of 207 studies met the inclusion criteria. Methodological quality was assessed using the Strengthening of the Reporting of Observational Studies in Epidemiology (STROBE) statement checklist. Gluten-free diet adherence resulted in partial recovery of bone density by one year in all studies, and full recovery by the fifth year. No treatment differences were observed between the gluten-free diet alone and diet plus bisphosphonates in one study. For malnourished patients, supplementation with vitamin D and calcium resulted in significant improvement. Evidence for the impact of physical activity on bone density was limited. Therapeutic strategies aimed at modifying lifestyle factors throughout the lifespan should be studied.

  6. Magnetic resonance in hematological diseases. Imaging of bone marrow

    DEFF Research Database (Denmark)

    Jensen, K.E.

    1995-01-01

    Magnetic resonance imaging (MRI) is a highly sensitive alternative to plain radiography, CT, and radionuclide studies for the imaging of normal and abnormal bone marrow. The cellularity and the corresponding fat/water ratio within the bone marrow show clear changes in haematological diseases. Thi...

  7. Bone Allografts: What Is the Risk of Disease Transmission with Bone Allografts?

    Science.gov (United States)

    ... HIV virus in freeze-dried bone allografts. Pract Periodontics Aesthet Dent 1995;7:13–22. Mellonig JT, ... source: Division of Oral Health , National Center for Chronic Disease Prevention and Health Promotion Follow CDC Email ...

  8. Developmental plasticity and epigenetic mechanisms underpinning metabolic and cardiovascular diseases.

    Science.gov (United States)

    Low, Felicia M; Gluckman, Peter D; Hanson, Mark A

    2011-06-01

    The importance of developmental factors in influencing the risk of later-life disease has a strong evidence base derived from multiple epidemiological, clinical and experimental studies in animals and humans. During early life, an organism is able to adjust its phenotypic development in response to environmental cues. Such developmentally plastic responses evolved as a fitness-maximizing strategy to cope with variable environments. There are now increasing data that these responses are, at least partially, underpinned by epigenetic mechanisms. A mismatch between the early and later-life environments may lead to inappropriate early life-course epigenomic changes that manifest in later life as increased vulnerability to disease. There is also growing evidence for the transgenerational transmission of epigenetic marks. This article reviews the evidence that susceptibility to metabolic and cardiovascular disease in humans is linked to changes in epigenetic marks induced by early-life environmental cues, and discusses the clinical, public health and therapeutic implications that arise.

  9. What is the optimal bone-preserving strategy for patients with Addison's disease?

    Science.gov (United States)

    Lee, Paul; Greenfield, Jerry R

    2015-08-01

    Addison's disease is associated with low bone mineral density and increased risk of hip fractures. Causes are multifactorial, contributed by underlying adrenocortical hormonal deficiency, associated autoimmune endocrinopathies, electrolyte disturbances and, in some patients, supraphysiologic glucocorticoid replacement. Recent realization of physiologic cortisol production rate has revised downwards glucocorticoid replacement dosages. Meanwhile, new research has emerged suggesting complex interplay between sodium and calcium homoeostasis under the influence of mineralocorticoid and parathyroid hormone that may impact bone health. As the prevalence of Addison's disease is rising, and osteoporosis and fractures are associated with significant morbidity and increased mortality, attention to bone preservation in Addison's disease is of clinical relevance and importance. We suggest an approach to bone health in Addison's disease integrating physiologic adrenocortical hormonal replacement with electrolyte and mineral homoeostasis optimization. © 2015 John Wiley & Sons Ltd.

  10. Painful os styloideum: bone scintigraphy in carpe bossu disease

    International Nuclear Information System (INIS)

    Apple, J.S.; Martinez, S.; Nunley, J.A.

    1984-01-01

    The os styloideum (ninth carpal bone) is an anatomic variant that may occur as an accessory ossicle located dorsally between the capitate and trapezoid, and the bases of the second and third metacarpals. The association of dorsal wrist pain or fatigability with an os styloideum is known as carpe bossu disease. The authors describe a woman with dorsal wrist pain in whom the diagnosis of painful os styloideum (carpe bossu disease) was made using plain radiography, bone scintigraphy and tomography

  11. Orthopantomographic study of the alveolar bone level on periodontal disease

    International Nuclear Information System (INIS)

    Lee, Ki Sik; You, Dong Soo

    1972-01-01

    The author had measured the alveolar bone level of periodontal disease on 50 cases of orthopantomogram to detect the degree of alveolar bone resorption of both sexes of Korean. The results were obtained as follows; 1. Alveolar bone resorption of mesial and distal portion was similar in same patient. 2. The order of alveolar bone resorption was mandibular anterior region, posterior region, canine and premolar region of both jaws. 3. The degree of alveolar bone destruction was severe in shorter root length than longer one. 4. The degree of alveolar bone resorption was severe in fourth decades.

  12. Orthopantomographic study of the alveolar bone level on periodontal disease

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ki Sik; You, Dong Soo [College of Dentistry, Seoul National University, Seoul (Korea, Republic of)

    1972-11-15

    The author had measured the alveolar bone level of periodontal disease on 50 cases of orthopantomogram to detect the degree of alveolar bone resorption of both sexes of Korean. The results were obtained as follows; 1. Alveolar bone resorption of mesial and distal portion was similar in same patient. 2. The order of alveolar bone resorption was mandibular anterior region, posterior region, canine and premolar region of both jaws. 3. The degree of alveolar bone destruction was severe in shorter root length than longer one. 4. The degree of alveolar bone resorption was severe in fourth decades.

  13. Pathogenesis of Bone Alterations in Gaucher Disease: The Role of Immune System

    Directory of Open Access Journals (Sweden)

    Juan Marcos Mucci

    2015-01-01

    Full Text Available Gaucher, the most prevalent lysosomal disorder, is an autosomal recessive inherited disorder due to a deficiency of glucocerebrosidase. Glucocerebrosidase deficiency leads to the accumulation of glucosylceramide primarily in cells of mononuclear-macrophage lineage. Clinical alterations are visceral, hematological, and skeletal. Bone disorder in Gaucher disease produces defects on bone metabolism and structure and patients suffer from bone pain and crisis. Skeletal problems include osteopenia, osteoporosis, osteolytic lesions, and osteonecrosis. On the other hand a chronic stimulation of the immune system is a well-accepted hallmark in this disease. In this review we summarize the latest findings in the mechanisms leading to the bone pathology in Gaucher disease in relationship with the proinflammatory state.

  14. Differential diagnosis of metastatic bone disease and benign bone disease on spine SPECT in patients with low back pain

    International Nuclear Information System (INIS)

    Lee, Seung Hun; Choi, Yun Young; Cho, Suk Shin

    2001-01-01

    One or more abnormal vertebrae detected on bone scintigraphy is a common finding in clinical practice, and it could pose a diagnostic dilemma especially in cancer patients, as either metastasis or benign disease may cause scintigraphic abnormality. The purpose of this study was to determine whether additional spine SPECT has a role in differentiating malignant from benign lesions in patients with back pain. We reviewed spine SPECT studies obtained over a three-year period in 108 patients. Among them, forty-five patients with abnormal SPECT and clinically followed records were evaluated (20 cancer patients were included). Uptake patterns were classified as follows: 1. Body: diffusely increased uptake, linear increased uptake of end plate, segmental increased uptake, and cold defect, 2 Posterior element; posterior to body (pedicle), posterior to intervertebral disc space (facet joint), and spinous process. Lesions were correlated with radiological findings and with final diagnosis. Sixty-nine bone lesions were detected on SPECT images, including 18 metastases, 28 degenerative diseases and 21 compression fractures. Cold defect (6) and segmental increased uptake (5) were dominant findings in metastasis: linear increased uptake (12), and facet joint uptake (15) were in degenerative change; and diffuse increased uptake (9), and linear increased uptake (9) were in compression fracture. Cold defect and segmental increased uptake of body were characteristic findings of metastasis, but care should be taken because compression fracture also shows segmental increased uptake in some cases. Degenerative disease was easily diagnosed because of the typical finding of linear increased uptake of end plate and facet joint. Therefore, additional bone SPECT after planar bone scan would be helpful for differentiating metastasis from benign condition in cancer patients

  15. Mechanism of cancer-induced bone destruction: An association of connective tissue growth factor (CTGF/CCN2 in the bone metastasis

    Directory of Open Access Journals (Sweden)

    Tsuyoshi Shimo

    2011-02-01

    Full Text Available Connective tissue growth factor (CTGF/CCN2 is a member of the CCN family, a novel class of extracellular signal modulators. CCN2 is composed of four conserved modules connected in tandem, each of which is rich in cysteines and highly interactive with other molecules. CCN2 has various biological functions, being active in developmental processes including angiogenesis, chondrogenesis, and osteogenesis. Recently CCN2 has gained more clinical interest due to its role in cancer-induced bone destruction. In this article, the role of CCN2 in bone-destroying events as an organizer of the microenvironmental cell society is comprehensively described, and a brief summary of the recent findings on regulatory factors involved in tumor-induced bone disease is given.

  16. Natural history of malignant bone disease in hepatocellular carcinoma: final results of a multicenter bone metastasis survey.

    Directory of Open Access Journals (Sweden)

    Daniele Santini

    Full Text Available BACKGROUND: Bone is an uncommon site of metastasis in patients with advanced hepatocellular carcinoma (HCC. Therefore, there are few studies concerning the natural history of bone metastasis in patients with HCC. PATIENTS AND METHODS: Data on clinicopathology, survival, skeletal-related events (SREs, and bone-directed therapies for 211 deceased HCC patients with evidence of bone metastasis were statistically analyzed. RESULTS: The median age was 70 years; 172 patients were male (81.5%. The median overall survival was 19 months. The median time to the onset of bone metastasis was 13 months (22.2% at HCC diagnosis; 64.9% patients had multiple bone metastases. Spine was the most common site of bone metastasis (59.7%. Most of these lesions were osteolytic (82.4%; 88.5% of them were treated with zoledronic acid. At multivariate analysis, only the Child Score was significantly correlated with a shorter time to diagnosis of bone metastases (p = 0.001, HR = 1.819. The median survival from bone metastasis was 7 months. At multivariate analysis, HCC etiology (p = 0.005, ECOG performance status (p = 0.002 and treatment with bisphosphonate (p = 0.024 were associated with shorter survival after bone disease occurrence. The site of bone metastasis but not the number of bone lesions was associated with the survival from first skeletal related event (SRE (p = 0.021 and OS (p = 0.001. CONCLUSIONS: This study provides a significant improvement in the understanding the natural history of skeletal disease in HCC patients. An early and appropriate management of these patients is dramatically needed in order to avoid subsequent worsening of their quality of life.

  17. Alcoholic liver disease and changes in bone mineral density

    Directory of Open Access Journals (Sweden)

    Germán López-Larramona

    2013-12-01

    Full Text Available Osteoporosis and osteopenia are alterations in bone mineral density (BMD that frequently occur in the context of chronic liver disease (CLD. These alterations have been studied predominantly in chronic cholestatic disease and cirrhosis of the liver. Alcohol consumption is an independent risk factor for the onset of osteoporosis, whose estimated prevalence in patients with alcoholic liver disease (ALD ranges between 5 % and 40 %. The loss of BMD in ALD is the result of an imbalance between bone formation and resorption. Its pathogenesis is multifactorial and includes the toxic effects of alcohol on bone and endocrine and nutritional disorders secondary to alcoholism and a deficiency of osteocalcin, vitamin D and insulin growth factor-1. The diagnosis of BMD alterations in ALD is based on its measurement using bone densitometry. Treatment includes smoking and alcohol cessation and general measures such as changes in nutrition and exercise. Calcium and vitamin D supplements are recommended in all patients with ALD and osteoporosis. Bisphosphonates are the most commonly prescribed drugs for the specific treatment of this condition. Alternatives include raloxifene, hormone replacement therapy and calcitonin. This review will address the most important aspects involved in the clinical management of abnormal BMD in the context of ALD, including its prevalence, pathogenesis and diagnosis. We will also review the treatment of osteoporosis in CLD in general, focusing on specific aspects related to bone loss in ALD.

  18. Cytokines in Gaucher disease: Role in the pathogenesis of bone ...

    African Journals Online (AJOL)

    Azza A.G. Tantawy

    2015-03-03

    Mar 3, 2015 ... The impact of therapy on bone manifestations of Gaucher disease . ... types: classical or alternative, depending on the predominant cytokine in the .... avascular necrosis, bone infarcts and localised cortical thin- ning may be ...

  19. Bone loss in rheumatoid arthritis. Influence of disease activity, duration of the disease, functional capacity, and corticosteroid treatment

    DEFF Research Database (Denmark)

    Hansen, M; Florescu, A; Stoltenberg, M

    1996-01-01

    Axial and appendicular bone mass were studied in 95 patients with rheumatoid arthritis. The aims were to quantify bone mineral density (BMD) and to evaluate the importance of disease activity, duration of disease, functional capacity, and corticosteroid treatment for bone loss in patients...... after the start of the disease. The overall functional capacity in terms of physical activity increased BMD in the axial skeleton. The local functional capacity in terms of grip strength was positively related to BMD in the appendicular skeleton. Patients with severe functional impairment had the lowest...

  20. Bones and Crohn's: Estradiol deficiency in men with Crohn's disease is not associated with reduced bone mineral density

    Directory of Open Access Journals (Sweden)

    Boehm BO

    2008-10-01

    Full Text Available Abstract Background Reduced bone mineral density (BMD and osteoporosis are frequent in Crohn's disease (CD, but the underlying mechanisms are still not fully understood. Deficiency of sex steroids, especially estradiol (E2, is an established risk factor in postmenopausal osteoporosis. Aim To assess if hormonal deficiencies in male CD patients are frequent we investigated both, sex steroids, bone density and bone metabolism markers. Methods 111 male CD patients underwent osteodensitometry (DXA of the spine (L1–L4. Disease related data were recorded. Disease activity was estimated using Crohn's disease activity index (CDAI. Testosterone (T, dihydrotestosterone (DHT, estradiol (E2, sex hormone binding globulin (SHBG, Osteocalcin and carboxyterminal cross-linked telopeptids (ICTP were measured in 111 patients and 99 age-matched controls. Results Patients had lower T, E2 and SHBG serum levels (p 10 g had lower BMD. 32 (28.8% patients showed osteoporosis, 55 (49.5% osteopenia and 24 (21.6% had normal BMD. Patients with normal or decreased BMD showed no significant difference in their hormonal status. No correlation between markers of bone turnover and sex steroids could be found. ICTP was increased in CD patients (p Conclusion We found an altered hormonal status – i.e. E2 and, to a lesser extent T deficiency – in male CD patients but failed to show an association to bone density or markers of bone turnover. The role of E2 in the negative skeletal balance in males with CD, analogous to E2 deficiency in postmenopausal females, deserves further attention.

  1. Fracture, aging and disease in bone

    Energy Technology Data Exchange (ETDEWEB)

    Ager, J.W.; Balooch, G.; Ritchie, R.O.

    2006-02-01

    fracture resistance, whereas regulating the level of the cytokine TGF-beta can offer significant improvements in the stiffness, strength and toughness of bone, and as such may be considered as a therapeutic target to treat increased bone fragility induced by aging, drugs, and disease.

  2. Improvement of adynamic bone disease after renal transplantation.

    Science.gov (United States)

    Abdallah, K A; Jorgetti, V; Pereira, R C; Reis, L M dos; Pereira, L M; Corrêa, P H S; Borelli, A; Ianhez, L E; Moysés, R M A; David-Neto, E

    2006-01-01

    Low bone remodeling and relatively low serum parathyroid hormone (PTH) levels characterize adynamic bone disease (ABD). The impact of renal transplantation (RT) on the course of ABD is unknown. We studied prospectively 13 patients with biopsy-proven ABD after RT. Bone histomorphometry and bone mineral density (BMD) measurements were performed in the 1st and 12th months after RT. Serum PTH, 25-hydroxyvitamin D, 1,25-dihydroxyvitamin D, and osteocalcin were measured regularly throughout the study. Serum PTH levels were slightly elevated at transplantation, normalized at the end of the third month and remained stable thereafter. Bone biopsies performed in the first month after RT revealed low bone turnover in all patients, with positive bone aluminum staining in 5. In the 12th month, second biopsies were performed on 12 patients. Bone histomorphometric dynamic parameters improved in 9 and were completely normalized in 6, whereas no bone mineralization was detected in 3 of these 12 patients. At 12 months post-RT, no bone aluminum was detected in any patient. We also found a decrease in lumbar BMD and an increase in femoral BMD. Patients suffering from ABD, even those with a reduction in PTH levels, may present partial or complete recovery of bone turnover after successful renal transplantation. However, it is not possible to positively identify the mechanisms responsible for the improvement. Identifying these mechanisms should lead to a better understanding of the physiopathology of ABD and to the development of more effective treatments.

  3. Bone metabolism and RANKL/RANK/OPG trail in periodontal disease

    Directory of Open Access Journals (Sweden)

    Czupkallo Lukasz

    2016-12-01

    Full Text Available Periodontal disease is an inflammatory disease of multifactorial etiology. In order for it to appear there must come to an imbalance between the effects of pathogens and host defense mechanisms. As a result of its course the destruction of structures supporting the teeth appears (periodontium, cement, bone, and consequently leads to teeth loosening and loss. In recent years, the participation of RANKL/RANK/OPG in bone remodeling process was highligted.

  4. Childhood developmental vulnerabilities associated with early life exposure to infectious and noninfectious diseases and maternal mental illness.

    Science.gov (United States)

    Green, Melissa J; Kariuki, Maina; Dean, Kimberlie; Laurens, Kristin R; Tzoumakis, Stacy; Harris, Felicity; Carr, Vaughan J

    2017-12-26

    Fetal exposure to infectious and noninfectious diseases may influence early childhood developmental functioning, on the path to later mental illness. Here, we investigated the effects of in utero exposure to maternal infection and noninfectious diseases during pregnancy on offspring developmental vulnerabilities at age 5 years, in the context of estimated effects for early childhood exposures to infectious and noninfectious diseases and maternal mental illness. We used population data for 66,045 children from an intergenerational record linkage study (the New South Wales Child Development Study), for whom a cross-sectional assessment of five developmental competencies (physical, social, emotional, cognitive, and communication) was obtained at school entry, using the Australian Early Development Census (AEDC). Child and maternal exposures to infectious or noninfectious diseases were determined from the NSW Ministry of Health Admitted Patients Data Collection (APDC) and maternal mental illness exposure was derived from both APDC and Mental Health Ambulatory Data collections. Multinomial logistic regression analyses were used to examine unadjusted and adjusted associations between these physical and mental health exposures and child developmental vulnerabilities at age 5 years. Among the physical disease exposures, maternal infectious diseases during pregnancy and early childhood infection conferred the largest associations with developmental vulnerabilities at age 5 years; maternal noninfectious illness during pregnancy also retained small but significant associations with developmental vulnerabilities even when adjusted for other physical and mental illness exposures and covariates known to be associated with early childhood development (e.g., child's sex, socioeconomic disadvantage, young maternal age, prenatal smoking). Among all exposures examined, maternal mental illness first diagnosed prior to childbirth conferred the greatest odds of developmental

  5. Founders lecture 2007. Metabolic bone disease: what has changed in 30 years?

    Energy Technology Data Exchange (ETDEWEB)

    Sundaram, Murali [Cleveland Clinic, Diagnostic Radiology, MSK, Cleveland, OH (United States)

    2009-09-15

    To provide an update on imaging of metabolic bone disease based on new developments, findings, and changing practices over the past 30 years. Literature review of osteoporosis, osteomalacia, renal osteodystrophy, Paget's disease, bisphosphonates, with an emphasis on imaging. Cited references and pertinent findings. Significant developments have occurred in the imaging of metabolic bone disease over the past 30 years. (orig.)

  6. RECENT ADVANCES IN PATHO-BIOLOGY OF MYELOMA BONE DISEASE: CLINICOPATHOLOGY AND LITERATURE OF REVIEW

    Directory of Open Access Journals (Sweden)

    Lohit Kumar

    2016-03-01

    Full Text Available Bone disease is a hallmark of multiple myeloma, presenting as lytic lesions associated with bone pain, pathological fractures requiring surgery and/or radiation to bone, spinal cord compression and hypercalcaemia. Increased osteoclastic activity unaccompanied by a compensatory increase in osteoblast function, leading to enhanced bone resorption results in bone disease. The interaction of plasma cells with the bone marrow microenvironment has been shown to play a vital role. Also, interactions of myeloma cells with osteoclasts enhance myeloma growth and survival, and thereby create a vicious cycle leading to extensive bone destruction and myeloma cell expansion.

  7. Questions from the clinician to the radiologist regarding the diagnosis of metabolic bone diseases

    Energy Technology Data Exchange (ETDEWEB)

    Schulz, W.; Schmidt, M.

    1986-12-01

    Macromorphological X-ray findings in metabolic bone diseases can be established only in advanced stages. Micromorphological X-ray diagnostic procedures will support the diagnosis even in early stages. Mineralometric examinations are adjuvant methods for early diagnosis and survey of therapy in metabolic bone diseases. The synopsis of parameters of calcium phosphate metabolism, bone histology (histomorphometry) and radiological morphology enables the type and stage of osteopathy to be diagnosed. The supplementary diagnostic methods are helpful in distinguishing bone diseases with increased turnover, inpaired bone modelling and absorption, disturbed mineralization and ectopic calcification. Within the metabolic osteopathies, osteoporosis is gaining more and more importance as a socioeconomic problem; therefore, early diagnosis and treatment are of significant relevance. Hyper-, hypoparathyroidism and osteoidosis are diseases at can be cured if diagnosed early.

  8. Questions from the clinician to the radiologist regarding the diagnosis of metabolic bone diseases

    International Nuclear Information System (INIS)

    Schulz, W.; Schmidt, M.; Klinikum Bamberg

    1986-01-01

    Macromorphological X-ray findings in metabolic bone diseases can be established only in advanced stages. Micromorphological X-ray diagnostic procedures will support the diagnosis even in early stages. Mineralometric examinations are adjuvant methods for early diagnosis and survey of therapy in metabolic bone diseases. The synopsis of parameters of calcium phosphate metabolism, bone histology (histomorphometry) and radiological morphology enables the type and stage of osteopathy to be diagnosed. The supplementary diagnostic methods are helpful in distinguishing bone diseases with increased turnover, inpaired bone modelling and absorption, disturbed mineralization and ectopic calcification. Within the metabolic osteopathies, osteoporosis is gaining more and more importance as a socioeconomic problem; therefore, early diagnosis and treatment are of significant relevance. Hyper-, hypoparathyroidism and osteoidosis are diseases at can be cured if diagnosed early. (orig.) [de

  9. Nuclear Medicine in Diagnosis and Therapy of Bone and Joint Diseases

    International Nuclear Information System (INIS)

    Riccabona, G.

    1999-01-01

    Concerning bone and joint diseases therapy of rheumatic synovitis (radiosynoviorthesis) was introduced in 1952 before clinically relevant diagnostic procedures were developed. Radionuclides of Sr and later on 99mTc phosphonates then started the wide use of bone scintigraphy since > 30 years. The diagnostic methods have an excellent sensitivity for detection of local abnormalities of bone metabolism, the specificity of such studies, however, is low. Modifications of the technique (3-phase-bone-scintigraphy, pinhole collimators, ROI-technique), increasing knowledge of pathological scan patterns and introduction of other radionuclide studies (67Ga, 201Tl, inflammation scans with 99mTc-leukocytes or 99mTc-HIG) as well as 18FDG-PET have increased the specificity significantly in recent years and improvements of imaging systems (SPECT) also increased the accuracy of diagnostic methods in diseases of bone and joints. Therapy of such diseases has made considerable progress: inflamed, swollen joints can effectively be treated with 90Y-, 186Re, 169Er-colloids or with 165Dy-particles by radiosynoviorthesis. Severe pain due to disseminated bone metastases of cancer or polyarthritis can be controlled by radionuclide therapy with 89Sr, 153Sm-EDTMP, 186Re- or 188Re-HEDP and possibly 117mSn-DTPA with an acceptable risk of myelodepression. Possibilities, technical details and limitations of radionuclide applications for diagnostic and therapeutic purposes must be considered if optimal benefit for individual patients should be achieved. Overall Nuclear Medicine can become an essential element in management of bone and joint diseases. The relationship of Nuclear Medicine to bone and joint pathology is peculiar: In 1952 treatment of rheumatic synovitis by radiosynoviorthesis with 198Au Colloid was started by Fellinger and Schmid before diagnostic approaches to bone pathology existed. Bone scintigraphy was introduced only in 1961 using 85Sr but obviously the unfavourable radiation

  10. Bone scintigraphy in systemic and metabolic diseases

    International Nuclear Information System (INIS)

    Engels, H.J.; Schmidt, H.A.E.

    1984-01-01

    Bone scintigraphy is a very sensitive method to identify pathological processes affecting the bone. Its specificity is, however, considerably lower than its sensitivity, particularly in systemic diseases. We therefore investigated the possibilities of differential diagnosis based on typical sites or patterns of distribution. The Paget syndrome with characteristic manifestation in the pelvic region, including crutch-shaped accumulation in the proximal femur, may be diagnosed by scintigraphy alone. If these typical sites are absent, however, differential diagnosis is difficult. Differential diagnosis for multiple myeloma, fibrous dysplasia, enchondromatosis, hyperparathyroidism, osteopathies, osteomalacia, inflammatory rheumatic diseases is also required and should be based on further examinations, taking into consideration the history, clinical signs and course. In this connexion scintigraphy is relevant both for early assessment and documentation of the spread of pathological processes and for the follow-up. (orig.) [de

  11. GORHAM-STOUT SYNDROME: PHANTOM BONE DISEASE.

    Science.gov (United States)

    El-Kouba, Gabriel; de Araújo Santos, Romilton; Pilluski, Paulo César; Severo, Antonio; Lech, Osvandré

    2010-01-01

    Gorham-Stout syndrome is a disease that presents idiopathic osteolysis of a bone or closely contiguous area. The etiology is unknown. It is a rare condition that is difficult to diagnose, and its treatment is controversial. It affects individuals irrespective of age or sex. In this study, we conducted a bibliographic review of the disease, specifically focusing on the differential diagnosis, and we demonstrated the follow-up on a patient with this syndrome from the time of its diagnosis, through treatment, to its current state of evolution.

  12. A comparison of bone scanning and radiology in the evaluation of patients with metabolic bone disease

    International Nuclear Information System (INIS)

    Fogelman, I.; Carr, D.

    1980-01-01

    Bone scan and radiographs were evaluated in 80 patients with metabolic bone disease (27 with osteoporosis, 14 with primary hyperparathyroidism, 24 with renal osteodystrophy and 15 with osteomalacia). The bone scan did not suggest a metabolic bone disorder in any of 27 patients with histologically proven osteoporosis. In 22 (81%) patients radiographs were reported as showing osteoporosis. In 19 (70%) vertebral fractures were seen on X-ray while these were noted in 11 (41%) patients on the bone scan. Vertebral fractures were usually visualised on the bone scan when these had occurred less than one year previously. In primary hyperparathyroidism the bone scan was suggestive of a metabolic bone disorder in 7 of 14 (50%) patients, while radiographs were reported as showing evidence of hyperparathyrodism in three (21%) cases. The bone scan suggested the presence of a metabolic bone disorder in all 24 patients with renal osteodystrophy and 15 patients with osteomalacia while the correct diagnosis was obtained in 14 (58%) and nine (60%) of these patients on X-ray. It is concluded that the bone scan is the more sensitive investigation in patients with osteomalacia, primary hyperparathyroidism and renal osteodystrophy. For osteoporosis radiology is the investigation of choice but the bone scan may be of value in assessing the duration of vertebral collapse. (author)

  13. Effects of carbocalcitonin treatment on PAGET's disease assessed by quantitative bone scintigraphy

    International Nuclear Information System (INIS)

    Vattimo, A.; Di Maggio, G.; Burroni, L.

    1988-01-01

    Sixteen patients with PAGET's disease of bone underwent quantitative bone scintigraphy with 99m Tc-MDP before and after a long-term treatment with carbocalcitonin, a synthetic analogue of eel calcitonin. The radionuclide study consisted in the blood pool and bone uptake measurement and in the bone avidity calculation (bone uptake - blood pool ratio). In our patients, a significant decrease in bone uptake and bone avidity was found, whereas the blood pool remained statistically unchanged. This behaviour could be accounted for the shift of the compact bone to a lamellar pattern. (orig.) [de

  14. Bone marrow transplantation for correction of enzyme deficiency disease

    International Nuclear Information System (INIS)

    Hong, C.; Sutherland, D.E.R.; Matas, A.J.; Najarian, J.S.

    1979-01-01

    Mutant acatalasemic mice provide a prototype of congenital enzyme deficiency disease. Normal blood catalase levels were achieved permanently in congenitally acatalasemic mice by transplantation of bone marrow cells from congeneic normal catalasemic mice using relatively small numbers of cells following whole body irradiation. The increase in blood catalase activity was physiologically effective as demonstrated by the protection of the previously acatalasemic mice against the otherwise lethal effects of hydrogen peroxide injections. Bone marrow transplantation has the potential to provide a continuous source of some enzymes and may be applicable as treatment for certain congenital enzyme deficiency diseases

  15. Bone hydatid disease refractory to nitazoxanide treatment

    NARCIS (Netherlands)

    Schipper, Hans G.; Simsek, Suat; van Agtmael, Michiel A.; van Lienden, Krijn P.

    2009-01-01

    We report a patient with bone hydatid disease that was refractory to both long-term daily treatment with albendazole, combined with cimetidine or administered as monotherapy ( approximately 15 years) and a relatively short course of nitazoxanide combined with albendazole (3 months). Despite

  16. GORHAM-STOUT SYNDROME: PHANTOM BONE DISEASE

    Science.gov (United States)

    El-Kouba, Gabriel; de Araújo Santos, Romilton; Pilluski, Paulo César; Severo, Antonio; Lech, Osvandré

    2015-01-01

    Gorham-Stout syndrome is a disease that presents idiopathic osteolysis of a bone or closely contiguous area. The etiology is unknown. It is a rare condition that is difficult to diagnose, and its treatment is controversial. It affects individuals irrespective of age or sex. In this study, we conducted a bibliographic review of the disease, specifically focusing on the differential diagnosis, and we demonstrated the follow-up on a patient with this syndrome from the time of its diagnosis, through treatment, to its current state of evolution. PMID:27026974

  17. Infrared imaging microscopy of bone: illustrations from a mouse model of Fabry disease.

    Science.gov (United States)

    Boskey, Adele L; Goldberg, Michel; Kulkarni, Ashok; Gomez, Santiago

    2006-07-01

    Bone is a complex tissue whose composition and properties vary with age, sex, diet, tissue type, health and disease. In this review, we demonstrate how infrared spectroscopy and infrared spectroscopic imaging can be applied to the study of these variations. A specific example of mice with Fabry disease (a lipid storage disease) is presented in which it is demonstrated that the bones of these young animals, while showing typical spatial variation in mineral content, mineral crystal size, and collagen maturity, do not differ from the bones of age- and sex-matched wild type animals.

  18. New aspects of radionuclide therapy of bone and joint diseases

    International Nuclear Information System (INIS)

    Fischer, M.

    2001-01-01

    Whereas in developing countries P-32 is widely used for radionuclide therapy of painful bone metastases, in Europe three radionuclides or radiopharmaceutical agents are available for pain palliation: Sr-89, Sm-153-EDTMP, and Re-186-HEDP. Radionuclide therapy for pain palliation is indicated for bone pain due to metastatic malignancy that has involved multiple skeletal sites and has evoked an osteoblastic response on bone scintigraphy. Response rates of about 70-80% in patients with breast or prostate cancer is reported in the literature, less in metastatic lesions of other primary malignancies. Sm-153-EDTMP may also be used for curative treatment of primary bone tumours or their metastases. Radiosynovectomy as therapeutic procedure or rheumatoid arthritis, other inflammatory joint diseases, persistent synovial perfusion, and other joint diseases is widely used. Using Y-90 for the knee joint, Re-186 for middle sized joints, and Er-169 for small joints an improvement of symptoms may be observed in about 70-80%. (author)

  19. Zoledronic acid in metastatic bone disease: an audit based discussion

    International Nuclear Information System (INIS)

    Akbar, R.A.; Gosh, S.

    2010-01-01

    Background: Metastatic bone disease is a common problem in patients with advanced cancer causing significant morbidity and poor quality of life. Effective and less toxic treatments, like bisphophonates, can reduce morbidity in such cases. Objectives: The objectives of this study were to determine whether Zoledronic acid was administered in accordance with current recommendations for its prescribing and to produce protocols for improved patient outcomes. Methods: The study was a retrospective audit of 39 consecutive patients with metastatic bone disease secondary to solid tumours who were treated with Zoledronic acid. The records were analysed to establish the administered dose of Zoledronic acid relative to creatinine clearance. The standards for Zoledronic acid therapy were defined from best practice guidelines. Results: The commonest diagnosis in patients receiving Zoledronic acid was carcinoma prostate 19/39 (49%) followed by carcinoma breast 11/39 (28%), gastrointestinal malignancies 4/39 (10%) and renal cell carcinoma 3/39 (8%). Indications for therapy were metastatic bone disease alone 31 (79%), hypercalcaemia alone 0/39 (0%), metastatic bone disease with hypercalcaemia 5/39 (13%), and prevention of chemotherapy induced bone loss 1/39 (3%). The dose of Zoledronic acid was appropriate to the creatinine clearance in 25/39 (6 4%), inappropriate in 5/39 (13%) and unclear from the notes in 9/39 (23%). Conclusions: Majority of patients received Zoledronic acid for the appropriate indications. The dose of Zoledronic acid was appropriate to serum creatinine clearance in a majority of patients. Poor documentation of data pertaining to Zoledronic acid treatment is observed which can potentially lead to major errors in prescribing. We recommend using a standard form to document each episode of therapy with Zoledronic acid. (author)

  20. Hemorrhage associated with 'bone crisis' in Gaucher's disease identified by magnetic resonance imaging

    International Nuclear Information System (INIS)

    Horev, G.; Kornreich, L.; Hadar, H.; Katz, K.

    1991-01-01

    Children suffering from Gaucher's disease were examined by magnetic resonance imaging (MRI) during a characteristic episode of 'bone crisis'. An unexpectedly high intramedullary as well as subperiosteal signal was observed on both the T1- and T2-weighted sequences in 5 patients, suggesting a subacute hemorrhage or hematoma. It is conceivable that such a painful hemorrhage is an important component of the 'bone crisis' phenomenon. Furthermore, in these cases this is a specific sign which may enable differentiation of bone crises from other types of bone pain associated with Gaucher's disease. (orig.)

  1. Bone Marrow and Peripheral Blood Leptin Levels in Lymphoproliferative Diseases - Relation to the Bone Marrow Fat and Infiltration

    Czech Academy of Sciences Publication Activity Database

    Gaja, A.; Churý, Z.; Pecen, Ladislav; Fraňková, H.; Jandáková, H.; Hejlová, N.

    2000-01-01

    Roč. 47, č. 5 (2000), s. 307-312 ISSN 0028-2685 Institutional research plan: AV0Z1030915 Keywords : leptin * bone marrow fat * bone marrow infiltration * lymphoproliferative disease Subject RIV: BA - General Mathematics Impact factor: 0.579, year: 2000

  2. Alveolar bone loss and mineralization in the pig with experimental periodontal disease

    Directory of Open Access Journals (Sweden)

    Mandee Yang

    2018-03-01

    Full Text Available Objective: To address how experimental periodontal disease affects alveolar bone mass and mineral apposition in a young pig model. Materials and methods: Seven three-month-old pigs were periodically inoculated with 4 types of periodontal bacteria, along with a ligature around the last maxillary deciduous molar for 8 weeks to induce periodontal disease (PG. Eight same-aged pigs served as the control (CG. Segmentations of 3D cone-beam CT images were performed to quantify volumes of the total alveolar bone, alveolar ridge, and all roots of the target molar. Calcein and alizarin were administered for labeling mineral apposition before euthanasia. The harvested molar blocks were sectioned and examined under epifluorescence. The inter-label distance between the two vital markers at regional bone surfaces were measured and mineral apposition rate (MAR was calculated. Results: A significant reduction of total alveolar bone volume was seen in PG with the major loss at the alveolar ridge. MAR was significantly higher at the root furcation region than those at both buccal and palatal ridges in CG. Compared with CG, PG animals showed more interrupted labeled bands with significantly lower MAR at the furcation region. MARs were positively associated with both the volumes of total alveolar bone and ridge in CG, but only with the total alveolar bone in PG. Conclusions: In young growing pigs, mineral apposition is region specific. The experimental periodontal disease not only leads to alveolar bone loss, but also perturbs mineral apposition for new bone formation, thus impairing the homeostasis of alveolar bone remodeling. Keyword: Dentistry

  3. Postnatal Changes in Humerus Cortical Bone Thickness Reflect the Development of Metabolic Bone Disease in Preterm Infants

    Directory of Open Access Journals (Sweden)

    Shuko Tokuriki

    2016-01-01

    Full Text Available Objective. To use cortical bone thickness (CBT of the humerus to identify risk factors for the development of metabolic bone disease in preterm infants. Methods. Twenty-seven infants born at <32 weeks of gestational age, with a birth weight of <1,500 g, were enrolled. Humeral CBT was measured from chest radiographs at birth and at 27-28, 31-32, and 36–44 weeks of postmenstrual age (PMA. The risk factors for the development of osteomalacia were statistically analyzed. Results. The humeral CBT at 36–44 weeks of PMA was positively correlated with gestational age and birth weight and negatively correlated with the duration of mechanical ventilation. CBT increased with PMA, except in six very early preterm infants in whom it decreased. Based on logistic regression analysis, gestational age and duration of mechanical ventilation were identified as risk factors for cortical bone thinning. Conclusions. Humeral CBT may serve as a radiologic marker of metabolic bone disease at 36–44 weeks of PMA in preterm infants. Cortical bones of extremely preterm infants are fragile, even when age is corrected for term, and require extreme care to lower the risk of fractures.

  4. Bone marrow accumulation in gallium scintigraphy in patients with adult still's disease

    International Nuclear Information System (INIS)

    Kanegae, Futoshi; Tada, Yoshifumi; Ohta, Akihide; Ushiyama, Osamu; Suzuki; Noriaki; Koarada, Syuichi; Haruta, Yoshio; Yoshikai, Tomonori; Nagasawa, Kohei

    2002-01-01

    We investigated the features and the usefulness of gallium scintigraphy in the diagnosis and the assessment of Adult Still's disease (ASD) by retrospective case review. Gallium scintigraphy have been done for 11 cases of ASD (3 males and 8 females) and 4 females were positive. Among these, 67 Ga-citrate was accumulated to the bone marrow in all 4 cases and to the major joints in 2 cases. Positive cases were rather serious and administered more immunosuppressants than negative cases. In order to characterize gallium scintigraphy findings of ASD, i.e. bone marrow accumulation, we analyzed 130 cases of collagen vascular disease. Although 101 cases (77.7%) were positive, only 7 cases (5.4%) showed the accumulation of 67 Ga-citrate to the bone marrow. These include 3 cases with ASD, and 1 case with systemic lupus erythematosus, polyarteritis nodosa, Wegener's granulomatosis and Sjogren's syndrome. We also accumulated 18 patients who exhibited bone marrow accumulation of 69 Ga-citrate, and found that 7 patients had collagen vascular and their related diseases. In conclusion, bone marrow accumulation in gallium scintigraphy is a specific feature of collagen vascular diseases, especially ASD, and it is suggested that cases with positive gallium scintigraphy in ASD can be serious and resistant to treatment. (author)

  5. Osteomalacia in a patient with Paget's bone disease treated with long-term etidronate.

    Science.gov (United States)

    Hoppé, E; Masson, C; Laffitte, A; Chappard, D; Audran, M

    2012-08-01

    A 93 year-old woman with Paget's disease of bone had been treated with etidronate without interruption during 20 years. The daily dose was usual (5mg/kg/day) but this prescription had never been stopped by her physicians. Two fractures had already occurred in pagetic (right tibia) and non pagetic bones (right fibula) within the last 2 years, and she presented rib fractures, another right tibia fracture and right femur fracture during hospitalization time. X-rays films showed major osteolysis of left ulna and right tibia. Blood samples and technetium bone scan brought no evidence for sarcoma or lytic evolution of the disease. A transiliac bone biopsy on non pagetic bone site confirmed the diagnosis of osteomalacia (increased osteoid parameters), with secondary hyperparathyroidism (hook resorption). In Paget's disease of bone, continuous treatment by etidronate may induce generalized osteomalacia, and increase the risk of fracture in both pagetic and non-pagetic bones. Whereas physicians and pharmaceutical industry try to improve the observance of those drugs, this striking observation also points out that a prescription always needs to be updated. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  6. Bone grafting: An overview

    Directory of Open Access Journals (Sweden)

    D. O. Joshi

    2010-08-01

    Full Text Available Bone grafting is the process by which bone is transferred from a source (donor to site (recipient. Due to trauma from accidents by speedy vehicles, falling down from height or gunshot injury particularly in human being, acquired or developmental diseases like rickets, congenital defects like abnormal bone development, wearing out because of age and overuse; lead to bone loss and to replace the loss we need the bone grafting. Osteogenesis, osteoinduction, osteoconduction, mechanical supports are the four basic mechanisms of bone graft. Bone graft can be harvested from the iliac crest, proximal tibia, proximal humerus, proximal femur, ribs and sternum. An ideal bone graft material is biologically inert, source of osteogenic, act as a mechanical support, readily available, easily adaptable in terms of size, shape, length and replaced by the host bone. Except blood, bone is grafted with greater frequency. Bone graft indicated for variety of orthopedic abnormalities, comminuted fractures, delayed unions, non-unions, arthrodesis and osteomyelitis. Bone graft can be harvested from the iliac crest, proximal tibia, proximal humerus, proximal femur, ribs and sternum. By adopting different procedure of graft preservation its antigenicity can be minimized. The concept of bone banking for obtaining bone grafts and implants is very useful for clinical application. Absolute stability require for successful incorporation. Ideal bone graft must possess osteogenic, osteoinductive and osteocon-ductive properties. Cancellous bone graft is superior to cortical bone graft. Usually autologous cancellous bone graft are used as fresh grafts where as allografts are employed as an alloimplant. None of the available type of bone grafts possesses all these properties therefore, a single type of graft cannot be recomm-ended for all types of orthopedic abnormalities. Bone grafts and implants can be selected as per clinical problems, the equipments available and preference of

  7. Maintaining bone health in prostate cancer throughout the disease continuum.

    Science.gov (United States)

    Saad, Fred; Eastham, James

    2010-06-01

    Prostate cancer (PC) is the most prevalent malignancy in men, with 604,506 new cases diagnosed yearly worldwide. Maintaining bone health is important during all stages of PC, including patients who experience bone loss from androgen-deprivation therapy and patients who develop bone metastases. Patients with bone metastases often experience severe bone pain and are at increased risk for potentially debilitating skeletal-related events. Bisphosphonates are a well-established treatment option for patients with bone metastases from solid tumors and bone lesions from multiple myeloma. Zoledronic acid (ZOL) is the only bisphosphonate (BP) that has been extensively studied in patients with castration-recurrent PC and is indicated for treating patients with bone metastases from PC in conjunction with standard antineoplastic therapy. This review will examine the breadth of evidence supporting a role for ZOL and emerging therapies in managing patients with PC throughout the disease continuum. 2010 Elsevier Inc. All rights reserved.

  8. Patient and implant survival following joint replacement because of metastatic bone disease

    DEFF Research Database (Denmark)

    Sørensen, Michala S; Gregersen, Kristine G; Grum-Schwensen, Tomas

    2013-01-01

    Patients suffering from a pathological fracture or painful bony lesion because of metastatic bone disease often benefit from a total joint replacement. However, these are large operations in patients who are often weak. We examined the patient survival and complication rates after total joint...... replacement as the treatment for bone metastasis or hematological diseases of the extremities....

  9. Genetics Home Reference: Paget disease of bone

    Science.gov (United States)

    ... is most common in people of western European heritage. Early-onset Paget disease of bone is much rarer. This form of the disorder has been reported in only a few families. Related Information What information about a genetic condition can statistics provide? Why are some genetic ...

  10. X-ray diagnoses of metabolic bone diseases in infants

    International Nuclear Information System (INIS)

    Oestreich, A.E.; Missouri Univ., Columbia

    1979-01-01

    In X-ray pictures of patients with metabolic bone diseases, there are some important differences between adults and children due to the fact that childrens' skeletons are still graving. Metabolically induced changes to be observed by the radiologist in osteoporosis, rickets, and other metabolic diseases are described. In many cases, specific treatment of these diseases is necessary and also possible. (orig./MG) [de

  11. Animal models for bone tissue engineering and modelling disease

    Science.gov (United States)

    Griffin, Michelle

    2018-01-01

    ABSTRACT Tissue engineering and its clinical application, regenerative medicine, are instructing multiple approaches to aid in replacing bone loss after defects caused by trauma or cancer. In such cases, bone formation can be guided by engineered biodegradable and nonbiodegradable scaffolds with clearly defined architectural and mechanical properties informed by evidence-based research. With the ever-increasing expansion of bone tissue engineering and the pioneering research conducted to date, preclinical models are becoming a necessity to allow the engineered products to be translated to the clinic. In addition to creating smart bone scaffolds to mitigate bone loss, the field of tissue engineering and regenerative medicine is exploring methods to treat primary and secondary bone malignancies by creating models that mimic the clinical disease manifestation. This Review gives an overview of the preclinical testing in animal models used to evaluate bone regeneration concepts. Immunosuppressed rodent models have shown to be successful in mimicking bone malignancy via the implantation of human-derived cancer cells, whereas large animal models, including pigs, sheep and goats, are being used to provide an insight into bone formation and the effectiveness of scaffolds in induced tibial or femoral defects, providing clinically relevant similarity to human cases. Despite the recent progress, the successful translation of bone regeneration concepts from the bench to the bedside is rooted in the efforts of different research groups to standardise and validate the preclinical models for bone tissue engineering approaches. PMID:29685995

  12. An interesting case of peripheral vascular disease, vascular reperfusion, and subsequent development of pain due to Paget's disease of bone.

    Science.gov (United States)

    Kwun, Sunna; Tucci, Joseph R

    2013-01-01

    To present a case of Paget's disease of bone that was unmasked after vascular reperfusion. In this case study, we review the presentation, evaluation, diagnosis, and management of a patient with Paget's disease and peripheral vascular disease. A 79-year-old-woman with a history of coronary artery heart disease and recent finding of a T5 compression fracture was hospitalized for evaluation of right lower extremity claudication. Angiography demonstrated a focal complete occlusion of the distal right femoral and popliteal arteries. A self-expanding stent was placed in the distal femoral and popliteal arteries. Approximately 48 hours after the procedure, the patient developed severe, right lower leg pain. On endocrine evaluation, the patient was found to have clinical signs suggesting Paget's disease of bone, which was subsequently confirmed by imaging. This patient's development of severe pain following reperfusion of distal femoral and popliteal arteries is in keeping with the known and aforementioned hypervascularity of pagetic bone. The finding of increased warmth over an area of skeletal deformation should always raise the possibility of Paget's disease of bone.

  13. Low serum and bone vitamin K status in patients with longstanding Crohn's disease: another pathogenetic factor of osteoporosis in Crohn's disease?

    Science.gov (United States)

    Schoon, E; Muller, M; Vermeer, C; Schurgers, L; Brummer, R; Stockbrugger, R

    2001-01-01

    BACKGROUND—A high prevalence of osteoporosis is reported in Crohn's disease. The pathogenesis is not completely understood but is probably multifactorial. Longstanding Crohn's disease is associated with a deficiency of fat soluble vitamins, among them vitamin K. Vitamin K is a cofactor in the carboxylation of osteocalcin, a protein essential for calcium binding to bone. A high level of circulating uncarboxylated osteocalcin is a sensitive marker of vitamin K deficiency.
AIMS—To determine serum and bone vitamin K status in patients with Crohn's disease and to elucidate its relationship with bone mineral density.
METHODS—Bone mineral density was measured in 32 patients with longstanding Crohn's disease and small bowel involvement, currently in remission, and receiving less than 5 mg of prednisolone daily. Serum levels of vitamins D and K, triglycerides, and total immunoreactive osteocalcin, as well as uncarboxylated osteocalcin ("free" osteocalcin) were determined. The hydroxyapatite binding capacity of osteocalcin was calculated. Data were compared with an age and sex matched control population.
RESULTS—Serum vitamin K levels of CD patients were significantly decreased compared with normal controls (p<0.01). "Free" osteocalcin was higher and hydroxyapatite binding capacity of circulating osteocalcin was lower than in matched controls (p<0.05 and p<0.001, respectively), indicating a low bone vitamin K status in Crohn's disease. In patients, an inverse correlation was found between "free" osteocalcin and lumbar spine bone mineral density (r=−0.375, p<0.05) and between "free" osteocalcin and the z score of the lumbar spine (r=−0.381, p<0.05). Multiple linear regression analysis showed that "free" osteocalcin was an independent risk factor for low bone mineral density of the lumbar spine whereas serum vitamin D was not.
CONCLUSIONS—The finding that a poor vitamin K status is associated with low bone mineral density in longstanding Crohn

  14. Scintigraphy versus radiography in the early diagnosis of experimental bone necrosis with special reference to caisson disease of bone

    International Nuclear Information System (INIS)

    Gregg, P.J.; Walder, D.N.

    1980-01-01

    The early diagnosis of caisson disease of bone is hindered by the long delay which must elapse before an abnormality becomes apparent on a radiograph. The possible use of bone scintigraphy for this purpose was investigated. Necrosis of the bone and marrow was produced in rabbits by glass microspheres to simulate persistent gas-bubble emboli and then serial radiographs and scintigrams using sup(99m)Tc-diphosphonate were obtained. Regions of necrosis could be detected as 'hot spots' on the scintigrams as early as three weeks after the causative insult, which was many weeks before any abnormality could be detected on the radiographs. Histological examination of excised femora suggested that the scintigraphic abnormality might depend on the new bone formation established during a reactive or repair process. It is suggested that scintigraphy may have clinical value in caisson disease. (author)

  15. Scintigraphy versus radiography in the early diagnosis of experimental bone necrosis, with special reference to caisson disease of bone.

    Science.gov (United States)

    Gregg, P J; Walder, D N

    1980-05-01

    The early diagnosis of caisson disease of bone is hindered by the long delay which must elapse before an abnormality becomes apparent on a radiograph. The possible use of bone scintigraphy for this purpose was investigated. Necrosis of the bone and marrow was produced in rabbits by glass microspheres to simulate persistent gas-bubble emboli and then serial radiographs and scintigrams using 99mTc-diphosphonate were obtained. Regions of necrosis could be detected as "hot-spots" on the scintigrams as early as three weeks after the causative insult, which was many weeks before any abnormality could be detected on the radiographs. Histological examination of excised femora suggested that the scintigraphic abnormality might depend on the new bone formation during a reactive or repair process. It is suggested that scintigraphy may have clinical value in caisson disease.

  16. Bone histomorphometric changes after liver transplantation for chronic cholestatic liver disease

    NARCIS (Netherlands)

    Guichelaar, MMJ; Malinchoc, M; Sibonga, JD; Clarke, BL; Hay, JE

    2003-01-01

    Introduction: Patients with advanced liver disease, especially chronic cholestasis, often have osteopenia, which worsens early after orthotopic liver transplantation (OLT) before starting to recover. The changes in bone metabolism leading to this rapid loss of bone after OLT, and to its recovery,

  17. Bone mineral density and vitamin D status in Parkinson's disease patients

    NARCIS (Netherlands)

    Bos, F. van den; Speelman, A.D.; Nimwegen-Arrachart, M.L. van; Schouw, Y.T. van der; Backx, F.J.; Bloem, B.R.; Munneke, M.; Verhaar, H.J.

    2013-01-01

    Bone loss is more common in Parkinson's disease (PD) than in the general population. Several factors may be involved in the development of bone loss, including malnutrition, immobilization, low body mass index, decreased muscle strength, vitamin D deficiency and medication use. This study

  18. Bone scintigraphy in Erdheim-chester disease: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Siqueira, V.L.; Soares, L.M.M.; Ribeiro, V.P.B.; Coura Filho, G.B.; Sapienza, M.T.; Ono, C.R.; Watanabe, T.; Costa, P.L.A.; Hironaka, F.; Buchpiguel, C.A. [Universidade de Sao Paulo (FM/USP), SP (Brazil). Fac. de Medicina. Hospital das Clinicas

    2008-07-01

    Full text: Introduction: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis, of unknown etiology, characterized by infiltration of foamy histiocytes. Clinically, patients usually present with bone pain, and various extraskeletal manifestations. ECD differs from Langerhans cell histiocytosis (LCH) by radiologic and immunohistochemistry features. Case report: A 57-year-old woman presented with a history of intense pain on her left hand, besides eyelid xanthelasmas and xanthoms on frontal area ten years ago. Four years late she presented with pain on hips, legs and feet. Xanthoms spread to perioral area, mento and neck. Radiographs of the hands showed osteolysis of carpal bones bilaterally, osteolysis of fifth left metacarpal bone, osteosclerosis of all metacarpal bones bilaterally, except the fifth, and osteosclerosis of the second and third proximal falanges bilaterally. The legs showed bilateral diaphyseal and metaphyseal osteosclerosis. Bone scintigraphy demonstrated increased uptake on face bone (maxilla), and symmetric intense uptake on elbows, distal radii and ulnae, hands, distal area of femurs, tibias particularly on proximal and distal area, and feet. A tibia biopsy and a biopsy of neck lesion were made. The analysis of histology and immunohistochemistry were consistent with ECD. She has been treated with a-interferon for 1,5 year, and she reports delay in xanthoms progression and bone pain remission. Discussion: ECD is an adult multisystemic xanthogranulomatous infiltrative disease of unknown etiology. It may be confused with LCH, however ECD have distinctive immunohistochemistry and radiologic findings. LCH shows typically lytic bone lesions on axial skeleton, whereas symmetrical long-bone osteosclerosis is the radiologic sign for ECD. LCH stain positive for CD1a and S-100 protein, and the electron microscopy of cytoplasm discloses Biberck granules. ECD stain positive for CD68, negative for CD1a and S-100 protein, shows absent of

  19. Bone and motor organ diseases

    International Nuclear Information System (INIS)

    Fujiwara, Saeko

    1992-01-01

    Osteosarcoma arising from X-ray radiation therapy has first been reported in the 1920s. The 1950-1965 ABCC-RERF Life Span Study using the sample population of approximately 76,000 persons have revealed no evidence of correlation between osteosarcoma and A-bomb radiation. There is a relative paucity of data supporting the statistical correlation between A-bomb radiation and osseous cancer. This paper deals with the correlation between A-bomb exposure and bone and motor organ diseases. In prenatally exposed children using the Nagasaki's sample (n=74) and the Hiroshima's sample (n=219), there was no difference in skeletal abnormalities between the exposed and control groups in both cities. In the ABCC-study using 264 A-bomb survivors, the incidence of osteoporosis was found to be high in women aged 50 years or older at the time of A-bombing who were exposed at 2,000 m or less from the hypocenter. The RERF Adult Health Study using approximately 14,000 persons have revealed no evidence of correlation between the incidence of lumbar vertebral bone fractures and radiation doses. There was no correlation between the prevalence of rheumatoid arthritis and distance from the hypocenter in A-bomb survivors. Continuing studies are expected to confirm the delayed effects of A-bomb radiation on the bone and motor organs with aging in A-bomb survivors. (N.K.)

  20. The influence of genetic variability and proinflammatory status on the development of bone disease in patients with Gaucher disease.

    Directory of Open Access Journals (Sweden)

    Javier Gervas-Arruga

    Full Text Available Gaucher disease, the most common lysosomal storage disorder, is caused by β-glucocerebrosidase deficiency. Bone complications are the major cause of morbidity in patients with type 1 Gaucher disease (GD1. Genetic components strongly influence bone remodelling. In addition, chronic inflammation produced by Gaucher cells induces the production of several cytokines, which leads to direct changes in the bone remodelling process and can also affect the process indirectly through other immune cells. In this study, we analysed the association between bone mineral density (BMD, bone marrow burden score, and relevant genetic polymorphisms related to bone metabolism, as well as profiles of proinflammatory cytokines in a GD1 cohort. This study included 83 patients distributed according to bone status. BMD was measured with DXA and broadband ultrasound attenuation; bone marrow involvement was evaluated using MRI. We also analysed 26 SNPs located in 14 genes related to bone metabolism. To assess proinflammatory status, we analysed IL-4, IL-6, IL-7, IL-10, IL-13, MIP-1α, MIP-1β, and TNFα in plasma samples from 71 control participants and GD1 patients. SNP genotype proportions and BMD differed significantly between ESRI c.453-397T>C and VDR c.1024+283G>A variants. We also observed significant associations between GD1 genotypes and bone affectation. When patients were stratified by spleen status, we observed significant correlations between non-/splenectomized groups and Spanish MRI (S-MRI score. Across genotype proportions of non-/splenectomized patients and S-MRI, we observed significant differences in ESRI c.453-397T>C, VDR c.-83-25988G>A, and TNFRSF11B c.9C>G polymorphisms. We observed different significant proinflammatory profiles between control participants, treatment-naïve patients, and patients on enzyme replacement therapy (ERT; between non-/splenectomized patients (between untreated and ERT-treated patients and among those with differing GBA

  1. 99mTc-MDP bone imaging Legg-Calve'-Perthes disease

    Energy Technology Data Exchange (ETDEWEB)

    Nam, Kyung Jin; Lee, Sun Wha; Kim, Eun Kyung; Kim, Soon Yong [School of Medicine, Kyung Hee University Hospital, Seoul (Korea, Republic of)

    1983-03-15

    The pathology of Legg-Calve'-Perthes disease be can described as a vascular disturbance leading to necrosis and subsequent revascularization of the femoral epiphyseal ossification center. The regional distribution of bone-seeking radiopharmaceuticals in the skeleton can depend on a number of factors, but bone blood flow is a major physiological determinant of regional skeletal uptake of Tc-99m polyphosphate and bone imaging may thus be used for the detection of areas with both decreased and increased vascular supply. The authors analyzed the scintigraphic findings of 14 cases of Legg-Calve'-Perthes disease at Kyung Hee University Hospital from Mar. '81 to Oct. '82. The results were as follows: 1. Scintigraphy revealed area of absent radioactivity in 14 cases; lateral 2/3 portion of the femoral epiphyseal ossification center was involved in 9 cases among 14 cases, and also increased radioactivity in epiphyseal ossification center, epiphyseal plate, or proximal metaphysis of femur were associated in 7 cases. 2. Three cases had radiographically normal hip, whereas scintigraphy reviewed distinctly absent radioactivity in femoral epiphyseal ossification center. 3. Magnification of hip imaging using a pinhole collimator provided good definition of presence and extent of pathologic defect or uptake in epiphyseal ossification center, epiphyseal plate, and metaphysis. Bone imaging provided earlier and more accurate information concerning the extent of the necrotic and revascularization process of Legg-Calve'-Perthes disease than the radiographys alone.

  2. Critical assessment of bone scan quantitation (bone to soft tissue ratios) in the diagnosis of metabolic bone disease

    Energy Technology Data Exchange (ETDEWEB)

    Fogelman, I.; Gordon, D.; Bessent, R.G.

    1981-03-01

    Accurate quantitation from the bone scan image of skeletal uptake of radiopharmaceutical would be of value in the assessment of patients with metabolic bone disease. Repeat measurements of bone to soft tissue (B/ST) ratios on the one set of images were made for 103 subjects, a) by the same observer using lumbar vertebra 2 for the area of bone; b) by the same observer using lumbar vertebra 2 then lumbar vertebra 4; c) by two observers both using lumbar vertebra 2. The median difference between repeat measurements by the same observer was well under 1% but the 5-95 percentile range was -13 to +14%. Between the two observers there was a median difference of 10.6% with a 5-95 percentile range of -11 to +44%. We also measured B/ST ratios in 150 control subjects and 139 patients with various metabolic bone disorders. While statistically significant differences for B/ST ratios were found between the osteomalacia, renal osteodystrophy, Paget's groups, and the control population (P < 0.001 in all cases), there was appreciable overlap between individual patient results and the control range. It is concluded, therefore, that measurement of B/ST ratios for the individual is of limited value in clinical practice.

  3. Adaptive developmental delay in Chagas disease vectors: an evolutionary ecology approach.

    Directory of Open Access Journals (Sweden)

    Frédéric Menu

    2010-05-01

    Full Text Available The developmental time of vector insects is important in population dynamics, evolutionary biology, epidemiology and in their responses to global climatic change. In the triatomines (Triatominae, Reduviidae, vectors of Chagas disease, evolutionary ecology concepts, which may allow for a better understanding of their biology, have not been applied. Despite delay in the molting in some individuals observed in triatomines, no effort was made to explain this variability.We applied four methods: (1 an e-mail survey sent to 30 researchers with experience in triatomines, (2 a statistical description of the developmental time of eleven triatomine species, (3 a relationship between development time pattern and climatic inter-annual variability, (4 a mathematical optimization model of evolution of developmental delay (diapause.85.6% of responses informed on prolonged developmental times in 5(th instar nymphs, with 20 species identified with remarkable developmental delays. The developmental time analysis showed some degree of bi-modal pattern of the development time of the 5(th instars in nine out of eleven species but no trend between development time pattern and climatic inter-annual variability was observed. Our optimization model predicts that the developmental delays could be due to an adaptive risk-spreading diapause strategy, only if survival throughout the diapause period and the probability of random occurrence of "bad" environmental conditions are sufficiently high.Developmental delay may not be a simple non-adaptive phenotypic plasticity in development time, and could be a form of adaptive diapause associated to a physiological mechanism related to the postponement of the initiation of reproduction, as an adaptation to environmental stochasticity through a spreading of risk (bet-hedging strategy. We identify a series of parameters that can be measured in the field and laboratory to test this hypothesis. The importance of these findings is

  4. Angiomatosis of bone and soft tissue: A spectrum of disease from diffuse lymphangiomatosis to vanishing bone disease in young patients

    International Nuclear Information System (INIS)

    Aviv, R.I.; McHugh, K.; Hunt, J.

    2001-01-01

    The application of cross-sectional imaging in the investigation of patients with angiomatosis reveals that lymphangiomatosis and vanishing bone disease should not be considered as separate entities, but rather as a spectrum of disease. We present a pictorial review of eight patients demonstrating the manifestations of soft tissue and bony involvement. We highlight a subgroup of patients with chyloid pleural effusions who have a poor prognosis. Aviv, R. I. et al. (2001)

  5. Kidney transplantation restored uncoupled bone turnover in end-stage renal disease.

    Science.gov (United States)

    Kawarazaki, Hiroo; Shibagaki, Yugo; Kido, Ryo; Nakajima, Ichiro; Fuchinoue, Shohei; Ando, Katsuyuki; Fujita, Toshiro; Fukagawa, Masafumi; Teraoka, Satoshi; Fukumoto, Seiji

    2012-07-01

    While kidney transplantation (KTx) reverses many disorders associated with end-stage renal disease (ESRD), patients who have received KTx often have chronic kidney disease and bone and mineral disorder (CKD-MBD). However, it is unknown how bone metabolism changes by KTx. Living donor-KTx recipients (n = 34) at Tokyo Women's Medical University were prospectively recruited and the levels of bone-specific alkaline phosphatase (BAP) and serum cross-linked N-telopeptides of Type 1 collagen (NTX) were measured before, 6 and 12 months after transplantation. Before KTx, serum BAP was within the reference range in more than half of patients while NTX was high in most patients. Serum NTX was higher in patients with longer dialysis durations compared to that with shorter durations before KTx. However, there was no difference in serum BAP between these patients. After KTx, BAP increased while NTX decreased along with the decline of PTH. In addition, the numbers of patients who showed high BAP and NTX were comparable after KTx. These results suggest that bone formation is suppressed and uncoupled with bone resorption in patients with ESRD and this uncoupling is restored by KTx. Further studies are necessary to clarify the mechanism of bone uncoupling in patients with ESRD.

  6. Evolution of bone disease after kidney transplantation: A prospective histomorphometric analysis of trabecular and cortical bone.

    Science.gov (United States)

    Carvalho, Catarina; Magalhães, Juliana; Pereira, Luciano; Simões-Silva, Liliana; Castro-Ferreira, Inês; Frazão, João Miguel

    2016-01-01

    Post-transplant bone disease results from multiple factors, including previous bone and mineral metabolism disturbances and effects from transplant-related medications. Bone biopsy remains the gold-standard diagnostic tool. We aimed to prospectively evaluate trabecular and cortical bone by histomorphometry after kidney transplantation. Seven patients, willing to perform follow-up bone biopsy, were included in the study. Dual-X-ray absorptiometry and trans-iliac bone biopsy were performed within the first 2 months after renal transplantation and repeated after 2-5 years of follow-up. Follow-up biopsy revealed a significant decrease in osteoblast surface/bone surface (0.91 ± 0.81 to 0.47 ± 0.12%, P = 0.036), osteoblasts number/bone surface (0.45 (0.23, 0.94) to 0.00/mm(2) , P = 0.018) and erosion surface/bone surface (3.75 ± 2.02 to 2.22 ± 1.38%, P = 0.044). A decrease in trabecular number (3.55 (1.81, 2.89) to 1.55/mm (1.24, 2.06), P = 0.018) and increase in trabecular separation (351.65 ± 135.04 to 541.79 ± 151.91 μm, P = 0.024) in follow-up biopsy suggest loss in bone quantity. We found no significant differences in cortical analysis, except a reduction in external cortical osteonal eroded surface (5.76 (2.94, 13.97) to 3.29% (0.00, 6.67), P = 0.043). Correlations between bone histomorphometric and dual-X-ray absorptiometry parameters gave inconsistent results. The results show a reduction in bone activity, suggesting increased risk of adynamic bone and loss of bone volume. Cortical bone seems less affected by post-transplant biological changes in the first years after kidney transplantation. © 2015 Asian Pacific Society of Nephrology.

  7. Effects of anticonvulsants and inactivity on bone disease in epileptics

    Science.gov (United States)

    Murchison, Lilian E.; Bewsher, P. D.; Chesters, Marion; Gilbert, J.; Catto, G.; Law, Elizabeth; McKay, E.; Ross, H. S.

    1975-01-01

    No significant biochemical or radiological features of vitamin D deficiency were found in groups of juvenile and adult epileptics and control groups of non-epileptic patients in hospitals for the mentally retarded. There was evidence of hepatic enzyme induction in patients on anticonvulsants, in that urinary D-glucaric acid concentration and excretion were raised. No effect was found of prolonged anticonvulsant therapy on bone densitometry, but in children immobility was closely associated with decreased bone density. The evidence suggests that disuse osteoporosis is the major bone disease in these mentally retarded children. PMID:1161672

  8. Effect of antitumour necrosis factor-alpha therapy on bone turnover in patients with active Crohn's disease: a prospective study.

    Science.gov (United States)

    Ryan, B M; Russel, M G V M; Schurgers, L; Wichers, M; Sijbrandij, J; Stockbrugger, R W; Schoon, E

    2004-10-15

    Patients with Crohn's disease are at increased risk of osteoporosis. Disease activity and circulating proinflammatory cytokines are thought to play a role in this process. Infliximab, a chimaeric antitumour necrosis factor-alpha antibody is effective in the treatment of Crohn's disease. The aim of this study was to investigate the impact of treatment with infliximab on bone turnover in Crohn's disease patients. This was a prospective trial. Twenty-four patients with active Crohn's disease were treated with infliximab (5 mg/kg). Bone markers were assayed pre- and post-treatment. Bone formation was measured using serum bone-specific alkaline phosphatase and total osteocalcin and bone resorption using serum N-telopeptide cross-linked type 1 collagen. Infliximab therapy caused a significant increase in both markers of bone formation in patients with active Crohn's disease. No significant change in the bone resorption marker serum N-telopeptide cross-linked type 1 was found. Infliximab therapy had a significant beneficial effect on bone metabolism in patients with active Crohn's disease. These findings further support the theory that active ongoing inflammation and high levels of circulating cytokines play a pivotal role in the pathogenesis of bone loss in patients with Crohn's disease.

  9. [Bone metabolism, biochemical markers of bone resorption and formation processes and interleukine 6 cytokin level during coeliac disease].

    Science.gov (United States)

    Fekih, Monia; Sahli, Hela; Ben Mustapha, Nadia; Mestiri, Imen; Fekih, Moncef; Boubaker, Jalel; Kaabachi, Naziha; Sellami, Mohamed; Kallel, Lamia; Filali, Azza

    2013-01-01

    Celiac disease (CD) is characterized by a malabsorption syndrom. The bone anomalies are one of the principal complications of this disease. The osteoporosis frequency is high: 3.4% among patients having with CD versus 0.2% in the general population. To study the bone mineral density during the CD, to compare it to a control group and to determine the anomalies of biochemical markers of bone turn over and level of interleukin 6 cytokin (IL6) in these patients. All patients with CD have a measurement of bone mineral density by dual-energy x-ray absorptiometry (DXA), a biological exam with dosing calcemia, vitamin D, parathormone (PTH), the osteoblastic bone formation markers (serum osteocalcin, ALP phosphates alkaline), bone osteoclastic activity (C Télopeptide: CTX) and of the IL6. 42 patients were included, with a median age of 33.6 years. 52. 8% of the patients had a low level of D vitamine associated to a high level of PTH. An osteoporosis was noted in 21.5% of patients. No case of osteoporosis was detected in the control group. The mean level of the CTX, ostéocalcine and the IL6 was higher among patients having an osteoporosis or ostéopenia compared to patients with normal bone (p = 0,017). The factors associated with an bone loss (osteopenia or osteoporosis) were: an age > 30 years, a weight 90 UI/ml, an hypo albuminemia < 40 g/l and a level of CTX higher than 1.2. Our study confirms the impact of the CD on the bone mineral statute. The relative risk to have an osteopenia or an osteoporosis was 5 in our series. The measurement of the osseous mineral density would be indicated among patients having a CD.

  10. How B cells influence bone biology in health and disease.

    Science.gov (United States)

    Horowitz, Mark C; Fretz, Jackie A; Lorenzo, Joseph A

    2010-09-01

    It is now well established that important regulatory interactions occur between the cells in the hematopoietic, immune and skeletal systems (osteoimmunology). B lymphocytes (B cells) are responsible for the generation and production of antibodies or immunoglobulins in the body. Together with T cells these lymphocytes comprise the adaptive immune system, which allows an individual to develop specific responses to an infection and retain memory of that infection, allowing for a faster and more robust response if that same infection occurs again. In addition to this immune function, B cells have a close and multifaceted relationship with bone cells. B cells differentiate from hematopoietic stem cells (HSCs) in supportive niches found on endosteal bone surfaces. Cells in the osteoblast lineage support HSC and B cell differentiation in these niches. B cell differentiation is regulated, at least in part, by a series of transcription factors that function in a temporal manner. While these transcription factors are required for B cell differentiation, their loss causes profound changes in the bone phenotype. This is due, in part, to the close relationship between macrophage/osteoclast and B cell differentiation. Cross talk between B cells and bone cells is reciprocal with defects in the RANKL-RANK, OPG signaling axis resulting in altered bone phenotypes. While the role of B cells during normal bone remodeling appears minimal, activated B cells play an important role in many inflammatory diseases with associated bony changes. This review examines the relationship between B cells and bone cells and how that relationship affects the skeleton and hematopoiesis during health and disease. Copyright 2010 Elsevier Inc. All rights reserved.

  11. Magnetic resonance imaging of bone marrow disease in children

    International Nuclear Information System (INIS)

    Cohen, M.D.; Klatte, E.C.; Baehner, R.

    1984-01-01

    Seven children underwent magnetic resonance imaging (MRI) of the bone marrow: results showed that it is technically feasible to obtain good MR images of marrow in children. MR has detected abnormality in the bone marrow of a child who had metastatic neuroblastoma. The extent of abnormality in the femur correlated well with findings of a bone marrow isotope scan. In one child who had idiopathic aplastic anemia, diseased marrow could not be distinguished from normal marrow on MR images. MRI identified abnormality of the marrow in osteogenic sarcoma, and demonstrated change in response to chemotherapy. It displayed marrow spread of tumors as well as CT. MRI showed marrow abnormality in four children who had leukemia

  12. Oral health- related quality of life in patients with rare inherited diseases affecting bone and teeth

    DEFF Research Database (Denmark)

    Gjørup, Hans; Haubek, Dorte

    Background X-linked hypophosphatemia (XLH) is a rare hereditary disease characterized by insufficient bone mineralization. Osteogenesis imperfecta (OI) is another rare inherited disease characterized by fragile bones because of defective collagen synthesis. Both diseases may have impact on teeth...

  13. Endurance Exercise Mobilizes Developmentally Early Stem Cells into Peripheral Blood and Increases Their Number in Bone Marrow: Implications for Tissue Regeneration

    Directory of Open Access Journals (Sweden)

    Krzysztof Marycz

    2016-01-01

    Full Text Available Endurance exercise has been reported to increase the number of circulating hematopoietic stem/progenitor cells (HSPCs in peripheral blood (PB as well as in bone marrow (BM. We therefore became interested in whether endurance exercise has the same effect on very small embryonic-like stem cells (VSELs, which have been described as a population of developmentally early stem cells residing in BM. Mice were run daily for 1 hour on a treadmill for periods of 5 days or 5 weeks. Human volunteers had trained in long-distance running for one year, six times per week. FACS-based analyses and RT-PCR of murine and human VSELs and HSPCs from collected bone marrow and peripheral blood were performed. We observed that endurance exercise increased the number of VSELs circulating in PB and residing in BM. In parallel, we observed an increase in the number of HSPCs. These observations were subsequently confirmed in young athletes, who showed an increase in circulating VSELs and HSPCs after intensive running exercise. We provide for the first time evidence that endurance exercise may have beneficial effects on the expansion of developmentally early stem cells. We hypothesize that these circulating stem cells are involved in repairing minor exercise-related tissue and organ injuries.

  14. Vanishing bone disease (Gorham′s disease - A rare occurrence of unknown etiology

    Directory of Open Access Journals (Sweden)

    Sumit Ray

    2012-01-01

    Full Text Available A 20-year-old male patient presented with painful swelling around left elbow joint. Radiographic examination revealed osteolytic lesion with pathological fracture of lower end of humerus and upper radius. Upper end of ulna was completely absent along with bony erosion. Histopathology of the bony tissue revealed hemangioma-like lesion composed of vascular channels lined by benign endothelium replacing bone. The diagnosis of Gorham′s massive osteolysis was made. Gorham′s disease is a benign self-limiting condition affecting any age, may involve any part of the skeleton and is characterized by replacement of bone by hemangiomatous tissue resulting in formation of lesions exhibiting massive osteolysis, which may be to the extent of disappearance of the affected bone in radiograph. This nonhereditary case was not associated with nephropathy, which is often a coexistent condition. The case is being reported for its rarity.

  15. Quality of life and relation to disease in patients with bone sarcoma.

    Directory of Open Access Journals (Sweden)

    Shchelkova O.Yu.

    2015-03-01

    Full Text Available The study aimed to investigate the basic aspects of quality of life and relation to disease in patients with malignant or premalignant bone tumors. Study participants (N=82 were aged 18 to 67 years (average age 34 ± 2 years. They were separated into three groups depending on diagnosis: patients with osteosarcoma, patients with giant cell tumor and patients with chondrosarcoma. The SF-36 Health Status Survey and the Quality of Life Questionnaire - Core 30 with Bone Metastasis (BM22 Module were used to assess patient quality of life. The type of relation to disease method (TOBOL was used to determine the relation to disease of the patients. According to the results of the quality of life study, patients with giant cell tumor exhibited the highest degree of limiting physical activity and reduced social functioning, the greatest financial difficulties and more pain sites than either patients with osteosarcoma or patients with chondrosarcoma. The study of relation to disease revealed that all studied groups of patients were susceptible to ergopathic and sensitive types of relation to disease. Moreover, patients with giant cell tumor experienced increased levels of tension and irritability with respect to relation to disease and treatment, while patients with chondrosarcoma were more susceptible to anxiety and hypochondria with respect to relation to disease. Patients with different types of bone tumors have different experiences with respect to their physical and mental health, their social functioning and their general health. The results of the study may be useful in developing individualized psychological aid programs for patients with malignant and premalignant bone tumors.

  16. Effects of carbocalcitonin treatment on PAGET's disease assessed by quantitative bone scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Vattimo, A.; Di Maggio, G.; Burroni, L.

    1988-06-01

    Sixteen patients with PAGET's disease of bone underwent quantitative bone scintigraphy with /sup 99m/Tc-MDP before and after a long-term treatment with carbocalcitonin, a synthetic analogue of eel calcitonin. The radionuclide study consisted in the blood pool and bone uptake measurement and in the bone avidity calculation (bone uptake - blood pool ratio). In our patients, a significant decrease in bone uptake and bone avidity was found, whereas the blood pool remained statistically unchanged. This behaviour could be accounted for the shift of the compact bone to a lamellar pattern.

  17. Different findings in bone scintigraphy between ulnar impaction syndrome and Kienbock's disease

    International Nuclear Information System (INIS)

    Chung, J.K.; Lee, H.Y.; Lee, M.C.; Baik, G.H.

    2002-01-01

    Objectives: There is an ethnic variance on the length of the ulnar and radius. In Caucasian the length of the radius is relatively longer than the ulnar, but in Oriental the length of the ulnar is longer. This difference results in the different ethnic prevalence of ulnar impaction syndrome and Kienbock's disease, avascular necrosis of the lunate. It is important to differentiate ulnar impaction syndrome from Kienbock's disease, because they show similar symptoms but need different treatment and different method of operation. Methods: Seventeen patients with the wrist pain were enrolled in this study (mean age: 45+/-13yrs M:F=12:5). Nine patients were diagnosed as Kienbock's disease and Eight patients were diagnosed as ulnar impaction. Three to four hours after injection of 20mCi of Tc-99m MDP, bone scintigraphy images of the both hands were obtained and analyzed visually. Results: In Kienbock's disease, 8 patients showed increased uptake in the lunate with or without other carpal bones. One patient showed photon defect in the lunate. Otherwise in all patients with ulnar impaction syndrome, increased uptakes were found in the lunate, with or without increase uptake in the triquetrum or the distal ulnar, which are related to triangular fibrocartilage complex. Pin hole scintigraphy and pin hole SPECT demonstrated more precise structure of the wrist. Conclusion: We could differentiate ulnar impaction syndrome from Kienbock's disease in bone scan. We should carefully evaluate bone scintigraphic findings in patients with wrist pain

  18. Clinical relevance of changes in bone metabolism in inflammatory bowel disease

    Science.gov (United States)

    Miheller, Pal; Lőrinczy, Katalin; Lakatos, Peter Laszlo

    2010-01-01

    Low bone mineral density is an established, frequent, but often neglected complication in patients with inflammatory bowel disease (IBD). Data regarding the diagnosis, therapy and follow-up of low bone mass in IBD has been partially extrapolated from postmenopausal osteoporosis; however, the pathophysiology of bone loss is altered in young patients with IBD. Fracture, a disabling complication, is the most important clinical outcome of low bone mass. Estimation of fracture risk in IBD is difficult. Numerous risk factors have to be considered, and these factors should be weighed properly to help in the identification of the appropriate patients for screening. In this editorial, the authors aim to highlight the most important clinical aspects of the epidemiology, prevention, diagnosis and treatment of IBD-related bone loss. PMID:21105186

  19. Acute bone crises in sickle cell disease: the T1 fat-saturated sequence in differentiation of acute bone infarcts from acute osteomyelitis

    International Nuclear Information System (INIS)

    Jain, R.; Sawhney, S.; Rizvi, S.G.

    2008-01-01

    Aim: To prove the hypothesis that acute bone infarcts in sickle cell disease are caused by sequestration of red blood cells (RBCs) in bone marrow, and to evaluate the unenhanced T1 fat-saturated (fs) sequence in the differentiation of acute bone infarction from acute osteomyelitis in patients with sickle-cell disease. Materials and methods: Two studies were undertaken: an experimental study using in-vitro packed red blood cells and normal volunteers, and a retrospective clinical study of 86 magnetic resonance imaging (MRI) studies. For the experimental study containers of packed RBCs were placed between the knees of four healthy volunteers with a saline bag under the containers as an additional control, and were scanned with the pre-contrast T1-fs sequence. Signal intensity (SI) ratios were obtained for packed RBCs:skeletal muscle and packed RBCs:saline. For the clinical study, the SIs of normal bone marrow, packed RBCs, bone and/or soft-tissue lesions, and normal skeletal muscle of 74 patients (86 MRI studies) were measured using unenhanced, T1 fat-saturated MRI. The ratios of the above SIs to normal skeletal muscle were calculated and subjected to statistical analysis. Results: Fifty-one of 86 MRI studies were included in the final analysis. The ratios of SIs for normal bone marrow, packed red cells, bone infarction, acute osteomyelitis, and soft-tissue lesions associated with bone infarct, compared with normal skeletal muscle were (mean ± SD) 0.9 ± 0.2, 2.1 ± 0.7, 1.7 ± 0.5, 1.0 ± 0.3, and 2.2 ± 0.7, respectively. The difference in the ratio of SIs of bone infarcts and osteomyelitis was significant (p = 0.003). The final diagnoses were bone infarction (n = 50), acute osteomyelitis (n = 1), and co-existent bone infarction and osteomyelitis (n = 2). Seven patients who had suspected osteomyelitis underwent image-guided aspiration. Conclusion: Acute bone infarcts in sickle cell disease are caused by sequestration of red blood cells in the bone marrow. The

  20. Molecular and Chemical Genetic Approaches to Developmental Origins of Aging and Disease in Zebrafish

    Science.gov (United States)

    Sasaki, Tomoyuki; Kishi, Shuji

    2013-01-01

    The incidence of diseases increases rapidly with age, accompanied by progressive deteriorations of physiological functions in organisms. Aging-associated diseases are sporadic but mostly inevitable complications arising from senescence. Senescence is often considered the antithesis of early development, but yet there may be factors and mechanisms in common between these two phenomena over the dynamic process of aging. The association between early development and late-onset disease with advancing age is thought to come from a consequence of developmental plasticity, the phenomenon by which one genotype can give rise to a range of physiologically and/or morphologically adaptive states in response to different environmental or genetic perturbations. On the one hand, we hypothesized that the future aging process can be predictive based on adaptivity during the early developmental period. Modulating the thresholds of adaptive plasticity by chemical genetic approaches, we have been investigating whether any relationship exists between the regulatory mechanisms that function in early development and in senescence using the zebrafish (Danio rerio), a small freshwater fish and a useful model animal for genetic studies. We have successfully conducted experiments to isolate zebrafish mutants expressing apparently altered senescence phenotypes during embryogenesis (“embryonic senescence”), subsequently showing shortened lifespan in adulthoods. We anticipate that previously uncharacterized developmental genes may mediate the aging process and play a pivotal role in senescence. On the other hand, unexpected senescence-related genes might also be involved in the early developmental process and regulation. The ease of manipulation using the zebrafish system allows us to conduct an exhaustive exploration of novel genes and small molecular compounds that can be linked to the senescence phenotype, and thereby facilitates searching for the evolutionary and developmental origins

  1. Developmental Immunotoxicity, Perinatal Programming, and Noncommunicable Diseases: Focus on Human Studies

    Science.gov (United States)

    Dietert, Rodney R.

    2014-01-01

    Developmental immunotoxicity (DIT) is a term given to encompass the environmentally induced disruption of normal immune development resulting in adverse outcomes. A myriad of chemical, physical, and psychological factors can all contribute to DIT. As a core component of the developmental origins of adult disease, DIT is interlinked with three important concepts surrounding health risks across a lifetime: (1) the Barker Hypothesis, which connects prenatal development to later-life diseases, (2) the hygiene hypothesis, which connects newborns and infants to risk of later-life diseases and, (3) fetal programming and epigenetic alterations, which may exert effects both in later life and across future generations. This review of DIT considers: (1) the history and context of DIT research, (2) the fundamental features of DIT, (3) the emerging role of DIT in risk of noncommunicable diseases (NCDs) and (4) the range of risk factors that have been investigated through human research. The emphasis on the human DIT-related literature is significant since most prior reviews of DIT have largely focused on animal research and considerations of specific categories of risk factors (e.g., heavy metals). Risk factors considered in this review include air pollution, aluminum, antibiotics, arsenic, bisphenol A, ethanol, lead (Pb), maternal smoking and environmental tobacco smoke, paracetamol (acetaminophen), pesticides, polychlorinated biphenyls, and polyfluorinated compounds. PMID:26556429

  2. Bone marrow contributes to epithelial cancers in mice and humans as developmental mimicry.

    Science.gov (United States)

    Cogle, Christopher R; Theise, Neil D; Fu, Dongtao; Ucar, Deniz; Lee, Sean; Guthrie, Steven M; Lonergan, Jean; Rybka, Witold; Krause, Diane S; Scott, Edward W

    2007-08-01

    Bone marrow cells have the capacity to contribute to distant organs. We show that marrow also contributes to epithelial neoplasias of the small bowel, colon, and lung, but not the skin. In particular, epithelial neoplasias found in patients after hematopoietic cell transplantations demonstrate that human marrow incorporates into neoplasias by adopting the phenotype of the surrounding neoplastic environment. To more rigorously evaluate marrow contribution to epithelial cancer, we employed mouse models of intestinal and lung neoplasias, which revealed specifically that the hematopoietic stem cell and its progeny incorporate within cancer. Furthermore, this marrow involvement in epithelial cancer does not appear to occur by induction of stable fusion. Whereas previous claims have been made that marrow can serve as a direct source of epithelial neoplasia, our results indicate a more cautionary note, that marrow contributes to cancer as a means of developmental mimicry. Disclosure of Potential Conflicts of Interest is found at the end of this article.

  3. Biochemical markers of bone turnover in the clinical development of drugs for osteoporosis and metastatic bone disease: potential uses and pitfalls.

    Science.gov (United States)

    Cremers, Serge; Garnero, Patrick

    2006-01-01

    Biochemical markers of bone turnover are used increasingly during the clinical development of drugs for the treatment of metabolic bone diseases such as Paget's disease, osteoporosis and cancer that has metastasised to the bone. However, assessing the optimal value of these markers is often complicated, and such an assessment is an obvious prerequisite for rational use of the markers and, consequently, potential improvement of clinical drug development. Biochemical markers of bone turnover are substances in the blood or urine that are produced or released during bone remodelling. They provide semiquantitative information on bone remodelling, and are often the most adequate tool to describe the pharmacodynamics of the drug. Their use has increased considerably because of dose-effect relationships that have been seen with certain drugs, but also because they have proven relationships with clinical outcomes in several metabolic bone diseases. However, there is a lack of information on the kinetics of these markers, and the immunoassays that are frequently used in their monitoring often measure a mixture of fragments rather than a single molecular entity. For drug development it should also be realised that different markers, but also different assays for the same marker, may provide different results, considerably limiting the ability to compare results. In postmenopausal osteoporosis, relationships have been shown between several biochemical markers of bone turnover, and either fracture risk and/or the antifracture efficacy of drugs. Such relationships can be used for the development of drugs with similar mechanisms of action, but also for the development of these drugs for closely related indications, such as corticosteroid-induced osteoporosis. In both of these instances, data on effects on biochemical markers of bone turnover are usually employed in combination with information about effects on bone mineral density. However, the relationships of these parameters

  4. What Is Breast in the Bone?

    Science.gov (United States)

    Shemanko, Carrie S; Cong, Yingying; Forsyth, Amanda

    2016-10-22

    The normal developmental program that prolactin generates in the mammary gland is usurped in the cancerous process and can be used out of its normal cellular context at a site of secondary metastasis. Prolactin is a pleiotropic peptide hormone and cytokine that is secreted from the pituitary gland, as well as from normal and cancerous breast cells. Experimental and epidemiologic data suggest that prolactin is associated with mammary gland development, and also the increased risk of breast tumors and metastatic disease in postmenopausal women. Breast cancer spreads to the bone in approximately 70% of cases with advanced breast cancer. Despite treatment, new bone metastases will still occur in 30%-50% of patients. Only 20% of patients with bone metastases survive five years after the diagnosis of bone metastasis. The breast cancer cells in the bone microenvironment release soluble factors that engage osteoclasts and/or osteoblasts and result in bone breakdown. The breakdown of the bone matrix, in turn, enhances the proliferation of the cancer cells, creating a vicious cycle. Recently, it was shown that prolactin accelerated the breast cancer cell-mediated osteoclast differentiation and bone breakdown by the regulation of breast cancer-secreted proteins. Interestingly, prolactin has the potential to affect multiple proteins that are involved in both breast development and likely bone metastasis, as well. Prolactin has normal bone homeostatic roles and, combined with the natural "recycling" of proteins in different tissues that can be used for breast development and function, or in bone function, increases the impact of prolactin signaling in breast cancer bone metastases. Thus, this review will focus on the role of prolactin in breast development, bone homeostasis and in breast cancer to bone metastases, covering the molecular aspects of the vicious cycle.

  5. S-MRI score: A simple method for assessing bone marrow involvement in Gaucher disease

    International Nuclear Information System (INIS)

    Roca, M.; Mota, J.; Alfonso, P.; Pocovi, M.; Giraldo, P.

    2007-01-01

    Semi quantitative MRI is a very useful procedure for evaluating the bone marrow burden (BMB) in Gaucher disease (GD). Score systems have been applied to obtain a parameter for evaluating the severity of bone disease. Our purpose was to test a simple, reproducible and accurate score to evaluate bone marrow involvement in GD patients. MRI was performed in spine, pelvis and femora at diagnosis in 54 adult GD1 patients, 61.1% of whom were female. Three MRI patterns and punctuation in each location were defined: normal, 0; non-homogeneous infiltration subtypes reticular, 1; mottled, 2; diffuse, 3; and homogeneous infiltration, 4. This score was called Spanish-MRI (S-MRI). Two independent observers applied the S-MRI and bone marrow burden score and compared the differences using the non-parametric Mann-Whitney test. Correlation rank test was calculated. In 46 patients (85.2%), bone involvement was observed. Thirty-nine (72.3%) had their spine affected, 35 (64.8%) pelvis and 33 (61.2%) femora. Fourteen patients had bone infarcts, 14 avascular necrosis, 2 vertebral fractures and 2 bone crises. Correlation analysis between S-MRI and BMB was (r 2 = .675; p = .0001). No evidence of correlation was observed between CT activity and S-MRI nor between CT activity and BMB. We have found a relationship between genotype and bone infiltration according to S-MRI site and complications. S-MRI is a simple method that provides useful information to evaluate bone infiltration and detect silent complications. Our results correlated with the BMB score but offer higher sensitivity, specificity and accuracy for classifying the extent of bone disease

  6. S-MRI score: A simple method for assessing bone marrow involvement in Gaucher disease

    Energy Technology Data Exchange (ETDEWEB)

    Roca, M. [Radiology (Magnetic Resonance) Instituto Aragones de Ciencias de la Salud (I-CS), Zaragoza (Spain); Mota, J. [Diagnostic Imaging Department, Medimagen, Barcelona (Spain); Alfonso, P. [Radiology (Magnetic Resonance) Instituto Aragones de Ciencias de la Salud (I-CS), Zaragoza (Spain); Pocovi, M. [Biochemistry and Cellular and Molecular Biology Department, Zaragoza University (Spain); Giraldo, P. [Haematology Department, Miguel Servet University Hospital, 50009 Zaragoza (Spain)]. E-mail: pgiraldo@salud.aragon.es

    2007-04-15

    Semi quantitative MRI is a very useful procedure for evaluating the bone marrow burden (BMB) in Gaucher disease (GD). Score systems have been applied to obtain a parameter for evaluating the severity of bone disease. Our purpose was to test a simple, reproducible and accurate score to evaluate bone marrow involvement in GD patients. MRI was performed in spine, pelvis and femora at diagnosis in 54 adult GD1 patients, 61.1% of whom were female. Three MRI patterns and punctuation in each location were defined: normal, 0; non-homogeneous infiltration subtypes reticular, 1; mottled, 2; diffuse, 3; and homogeneous infiltration, 4. This score was called Spanish-MRI (S-MRI). Two independent observers applied the S-MRI and bone marrow burden score and compared the differences using the non-parametric Mann-Whitney test. Correlation rank test was calculated. In 46 patients (85.2%), bone involvement was observed. Thirty-nine (72.3%) had their spine affected, 35 (64.8%) pelvis and 33 (61.2%) femora. Fourteen patients had bone infarcts, 14 avascular necrosis, 2 vertebral fractures and 2 bone crises. Correlation analysis between S-MRI and BMB was (r {sup 2} = .675; p = .0001). No evidence of correlation was observed between CT activity and S-MRI nor between CT activity and BMB. We have found a relationship between genotype and bone infiltration according to S-MRI site and complications. S-MRI is a simple method that provides useful information to evaluate bone infiltration and detect silent complications. Our results correlated with the BMB score but offer higher sensitivity, specificity and accuracy for classifying the extent of bone disease.

  7. Calcium Regulation and Bone Mineral Metabolism in Elderly Patients with Chronic Kidney Disease

    Directory of Open Access Journals (Sweden)

    Vickram Tejwani

    2013-05-01

    Full Text Available The elderly chronic kidney disease (CKD population is growing. Both aging and CKD can disrupt calcium (Ca2+ homeostasis and cause alterations of multiple Ca2+-regulatory mechanisms, including parathyroid hormone, vitamin D, fibroblast growth factor-23/Klotho, calcium-sensing receptor and Ca2+-phosphate product. These alterations can be deleterious to bone mineral metabolism and soft tissue health, leading to metabolic bone disease and vascular calcification and aging, termed CKD-mineral and bone disorder (MBD. CKD-MBD is associated with morbid clinical outcomes, including fracture, cardiovascular events and all-cause mortality. In this paper, we comprehensively review Ca2+ regulation and bone mineral metabolism, with a special emphasis on elderly CKD patients. We also present the current treatment-guidelines and management options for CKD-MBD.

  8. Recommendations for evaluation and management of bone disease in HIV.

    Science.gov (United States)

    Brown, Todd T; Hoy, Jennifer; Borderi, Marco; Guaraldi, Giovanni; Renjifo, Boris; Vescini, Fabio; Yin, Michael T; Powderly, William G

    2015-04-15

    Thirty-four human immunodeficiency virus (HIV) specialists from 16 countries contributed to this project, whose primary aim was to provide guidance on the screening, diagnosis, and monitoring of bone disease in HIV-infected patients. Four clinically important questions in bone disease management were identified, and recommendations, based on literature review and expert opinion, were agreed upon. Risk of fragility fracture should be assessed primarily using the Fracture Risk Assessment Tool (FRAX), without dual-energy X-ray absorptiometry (DXA), in all HIV-infected men aged 40-49 years and HIV-infected premenopausal women aged ≥40 years. DXA should be performed in men aged ≥50 years, postmenopausal women, patients with a history of fragility fracture, patients receiving chronic glucocorticoid treatment, and patients at high risk of falls. In resource-limited settings, FRAX without bone mineral density can be substituted for DXA. Guidelines for antiretroviral therapy should be followed; adjustment should avoid tenofovir disoproxil fumarate or boosted protease inhibitors in at-risk patients. Dietary and lifestyle management strategies for high-risk patients should be employed and antiosteoporosis treatment initiated. © The Author 2015. Published by Oxford University Press on behalf of the Infectious Diseases Society of America. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.

  9. [Bone ultrasonography in kidney disease: applications and limitations].

    Science.gov (United States)

    Aucella, Filippo; Gesuete, Antonio; Cicchella, Antonio; Granata, Antonio; Fiorini, Fulvio; Guglielmi, Giuseppe

    2012-01-01

    Quantitative ultrasound (QUS) of the bone is a technique that is generating great interest among bone structure researchers because of its intrinsic features. Its safety and low cost make it an ideal technique for repeated measurements over time such as in chronic disease or when it is necessary to monitor the effects of prescribed therapies. The method was developed for the study of osteoporosis and the sites of measurement are all peripheral, including the distal diaphyses and metaphyses of the phalanges, calcaneus, radius and tibia. QUS parameters, however, cannot be used directly for the diagnosis of osteoporosis according to the WHO criteria, although many authors have shown that ultrasound parameters, particularly those of calcaneal QUS, can predict the risk of osteoporotic fractures independently of MBD. Very promising results with the use of QUS have been obtained in corticosteroid-induced osteoporosis, rheumatoid arthritis, Cushing's syndrome, cystic fibrosis, osteomalacia, thalassemia and osteopenia related to parenteral nutrition. QUS can also monitor the effectiveness of therapy in various pathological conditions. In nephrology the combined use of phalangeal QUS and biochemical markers of bone turnover allows adequate follow-up of patients on dialysis and renal transplant recipients with alterations or disorders of the bone.

  10. Effects of developmental exposure to perfluorooctanoic acid (PFOA) on long bone morphology and bone cell differentiation

    Energy Technology Data Exchange (ETDEWEB)

    Koskela, A., E-mail: antti.koskela@oulu.fi [Institute of Cancer Research and Translational Medicine, MRC Oulu and Department of Anatomy and Cell Biology, Faculty of Medicine, University of Oulu, Oulu (Finland); Finnilä, M.A. [Research Unit of Medical Imaging, Physics and Technology, University of Oulu, Oulu (Finland); Korkalainen, M. [National Institute for Health and Welfare, Department of Health Protection, Kuopio (Finland); Spulber, S. [Department of Neuroscience, Karolinska Institutet, Stockholm (Sweden); Koponen, J. [National Institute for Health and Welfare, Department of Health Protection, Kuopio (Finland); Håkansson, H. [Institute of Environmental Medicine, Karolinska Institutet, Stockholm (Sweden); Tuukkanen, J. [Institute of Cancer Research and Translational Medicine, MRC Oulu and Department of Anatomy and Cell Biology, Faculty of Medicine, University of Oulu, Oulu (Finland); Viluksela, M. [National Institute for Health and Welfare, Department of Health Protection, Kuopio (Finland); Department of Environmental and Biological Sciences, University of Eastern Finland, Kuopio (Finland)

    2016-06-15

    Perfluorooctanoic acid (PFOA) is a ubiquitous and persistent environmental chemical, which has been used extensively due to its stability and surface tension-lowering properties. Toxicological effects include induction of neonatal mortality and reproductive toxicity. In this study, pregnant C57BL/6 mice were exposed orally to 0.3 mg PFOA/kg/day throughout pregnancy, and female offspring were studied at the age of 13 or 17 months. Morphometrical and biomechanical properties of femurs and tibias were analyzed with micro-computed tomography and 3-point bending, and bone PFOA concentrations were determined by mass spectrometry. The effects of PFOA on bone cell differentiation were studied in osteoclasts from C57BL/6 mice and in the MC3T3 pre-osteoblast cell line. PFOA exposed mice showed increased femoral periosteal area as well as decreased mineral density of tibias. Biomechanical properties of these bones were not affected. Bone PFOA concentrations were clearly elevated even at the age of 17 months. In osteoblasts, low concentrations of PFOA increased osteocalcin (OCN) expression and calcium secretion, but at PFOA concentrations of 100 μM and above osteocalcin (OCN) expression and calcium secretion were decreased. The number of osteoclasts was increased at all PFOA concentrations tested and resorption activity dose-dependently increased from 0.1–1.0 μM, but decreased at higher concentrations. The results show that PFOA accumulates in bone and is present in bones until the old age. PFOA has the potential to influence bone turnover over a long period of time. Therefore bone is a target tissue for PFOA, and altered bone geometry and mineral density seem to persist throughout the life of the animal. - Highlights: • Bone is a target tissue for PFOA both in vivo and in vitro. • Maternal exposure during pregnancy results in PFOA accumulation in bone of the offspring. • PFOA is present in bones until the old age. • PFOA causes mild alterations in bone morphometry

  11. Effects of developmental exposure to perfluorooctanoic acid (PFOA) on long bone morphology and bone cell differentiation

    International Nuclear Information System (INIS)

    Koskela, A.; Finnilä, M.A.; Korkalainen, M.; Spulber, S.; Koponen, J.; Håkansson, H.; Tuukkanen, J.; Viluksela, M.

    2016-01-01

    Perfluorooctanoic acid (PFOA) is a ubiquitous and persistent environmental chemical, which has been used extensively due to its stability and surface tension-lowering properties. Toxicological effects include induction of neonatal mortality and reproductive toxicity. In this study, pregnant C57BL/6 mice were exposed orally to 0.3 mg PFOA/kg/day throughout pregnancy, and female offspring were studied at the age of 13 or 17 months. Morphometrical and biomechanical properties of femurs and tibias were analyzed with micro-computed tomography and 3-point bending, and bone PFOA concentrations were determined by mass spectrometry. The effects of PFOA on bone cell differentiation were studied in osteoclasts from C57BL/6 mice and in the MC3T3 pre-osteoblast cell line. PFOA exposed mice showed increased femoral periosteal area as well as decreased mineral density of tibias. Biomechanical properties of these bones were not affected. Bone PFOA concentrations were clearly elevated even at the age of 17 months. In osteoblasts, low concentrations of PFOA increased osteocalcin (OCN) expression and calcium secretion, but at PFOA concentrations of 100 μM and above osteocalcin (OCN) expression and calcium secretion were decreased. The number of osteoclasts was increased at all PFOA concentrations tested and resorption activity dose-dependently increased from 0.1–1.0 μM, but decreased at higher concentrations. The results show that PFOA accumulates in bone and is present in bones until the old age. PFOA has the potential to influence bone turnover over a long period of time. Therefore bone is a target tissue for PFOA, and altered bone geometry and mineral density seem to persist throughout the life of the animal. - Highlights: • Bone is a target tissue for PFOA both in vivo and in vitro. • Maternal exposure during pregnancy results in PFOA accumulation in bone of the offspring. • PFOA is present in bones until the old age. • PFOA causes mild alterations in bone morphometry

  12. Low bone mass density is associated with hemolysis in brazilian patients with sickle cell disease

    Directory of Open Access Journals (Sweden)

    Gabriel Baldanzi

    2011-01-01

    Full Text Available OBJECTIVES: To determine whether kidney disease and hemolysis are associated with bone mass density in a population of adult Brazilian patients with sickle cell disease. INTRODUCTION: Bone involvement is a frequent clinical manifestation of sickle cell disease, and it has multiple causes; however, there are few consistent clinical associations between bone involvement and sickle cell disease. METHODS: Patients over 20 years of age with sickle cell disease who were regularly followed at the Hematology and Hemotherapy Center of Campinas, Brazil, were sorted into three groups, including those with normal bone mass density, those with osteopenia, and those with osteoporosis, according to the World Health Organization criteria. The clinical data of the patients were compared using statistical analyses. RESULTS: In total, 65 patients were included in this study: 12 (18.5% with normal bone mass density, 37 (57% with osteopenia and 16 (24.5% with osteoporosis. Overall, 53 patients (81.5% had bone mass densities below normal standards. Osteopenia and osteoporosis patients had increased lactate dehydrogenase levels and reticulocyte counts compared to patients with normal bone mass density (p<0.05. Osteoporosis patients also had decreased hemoglobin levels (p<0.05. Hemolysis was significantly increased in patients with osteoporosis compared with patients with osteopenia, as indicated by increased lactate dehydrogenase levels and reticulocyte counts as well as decreased hemoglobin levels. Osteoporosis patients were older, with lower glomerular filtration rates than patients with osteopenia. There was no significant difference between the groups with regard to gender, body mass index, serum creatinine levels, estimated creatinine clearance, or microalbuminuria. CONCLUSION: A high prevalence of reduced bone mass density that was associated with hemolysis was found in this population, as indicated by the high lactate dehydrogenase levels, increased

  13. Long-term follow-up of children thought to have temporary brittle bone disease

    Directory of Open Access Journals (Sweden)

    Paterson CR

    2011-06-01

    Full Text Available Colin R Paterson1, Elizabeth A Monk21Department of Medicine (retired, 2School of Accounting and Finance, University of Dundee, Dundee, ScotlandBackground: In addition to nonaccidental injury, a variety of bone disorders may underlie the finding of unexplained fractures in young children. One controversial postulated cause is temporary brittle bone disease, first described in 1990.Methods: Eighty-five patients with fractures showing clinical and radiological features of temporary brittle bone disease were the subject of judicial hearings to determine whether it was appropriate for them to return home. Sixty-three patients did, and follow-up information was available for 61 of these. The mean follow-up period was 6.9 years (range 1–17, median 6.Results: We found that none of the children had sustained any further injuries that were thought to represent nonaccidental injury; no child was re-removed from home. Three children had fractures. In each case there was general agreement that the fractures were accidental. Had the original fractures in these children been the result of nonaccidental injury, it would have been severe and repeated; the average number of fractures was 9.1.Conclusion: The fact that no subsequent suspicious injuries took place after return home is consistent with the view that the fractures were unlikely to have been caused by nonaccidental injury, and that temporary brittle bone disease is a distinctive and identifiable disorder.Keywords: fractures, osteogenesis imperfecta, temporary brittle bone disease, nonaccidental injury

  14. Is there a role for scintigraphic imaging of bone manifestations in Gaucher disease? A review of the literature

    International Nuclear Information System (INIS)

    Mikosch, P.; State Hospital Klagenfurt; Kohlfuerst, S.; Gallowitsch, H.J.; Kresnik, E.; Lind, P.; Mehta, A.B.; Hughes, D.A.

    2008-01-01

    Gaucher disease is the most prevalent inherited, lysosomal storage disease and is caused by deficient activity of the enzyme β-glucocerebrosidase. Bone and bone marrow alterations are frequent in the most prevalent non-neuronopathic form of Gaucher disease. Imaging of bone manifestations in Gaucher disease is performed by a variety of imaging methods, conventional X-ray and MRI as the most frequently and most important ones. However, different modalities of scintigraphic imaging have also been used. This article gives an overview on scintigraphic imaging with respect to bone manifestations in Gaucher disease discussing the advantages and limitations of scintigraphic imaging in comparison to other imaging methods. (orig.)

  15. Homage to the 'H' in developmental origins of health and disease.

    Science.gov (United States)

    Rosenfeld, C S

    2017-02-01

    Abundant evidence exists linking maternal and paternal environments from pericopconception through the postnatal period to later risk to offspring diseases. This concept was first articulated by the late Sir David Barker and as such coined the Barker Hypothesis. The term was then mutated to Fetal Origins of Adult Disease and finally broadened to developmental origins of adult health and disease (DOHaD) in recognition that the perinatal environment can shape both health and disease in resulting offspring. Developmental exposure to various factors, including stress, obesity, caloric-rich diets and environmental chemicals can lead to detrimental offspring health outcomes. However, less attention has been paid to date on measures that parents can take to promote the long-term health of their offspring. In essence, have we neglected to consider the 'H' in DOHaD? It is the 'H' component that should be of primary concern to expecting mothers and fathers and those seeking to have children. While it may not be possible to eliminate exposure to all pernicious factors, prevention/remediation strategies may tip the scale to health rather than disease. By understanding disruptive DOHaD mechanisms, it may also illuminate behavioral modifications that parents can adapt before fertilization and throughout the neonatal period to promote the lifelong health of their male and female offspring. Three possibilities will be explored in the current review: parental exercise, probiotic supplementation and breastfeeding in the case of mothers. The 'H' paradigm should be the focus going forward as a healthy start can indeed last a lifetime.

  16. The spectrum of bone disease in 200 chronic hemodialysis patients: a correlation between clinical, biochemical and histological findings

    Directory of Open Access Journals (Sweden)

    Maria Eugênia Leite Duarte

    Full Text Available INTRODUCTION: Renal osteodystrophy includes the complete range of mineral metabolism disorders that affect the skeleton in patients with chronic renal failure. PATIENTS AND METHODS: 200 patients with end-stage renal disease and on dialysis were investigated regarding the clinical, biochemical and histological findings of bone disease. RESULTS: The spectrum of renal osteodystrophy consisted mainly of high turnover bone lesions (74.5%, including osteitis fibrosa in 57.5%. Patients with mild bone disease were on dialysis for shorter periods of time and were mostly asymptomatic. Patients with aluminum-related bone disease (16.5% had the greatest aluminum exposure, either orally or parenterally, and together with patients with high turnover mixed disease, were the most symptomatic. Although on a non-regular basis, the vast majority of the patients (82.5% had been receiving vitamin D. The incidence of adynamic bone disease was high (n=8 among parathyroidectomized patients (n=12. Significantly higher serum levels of alkaline phosphatase were observed in osteitis fibrosa. CONCLUSIONS: The use of calcitriol and phosphate-binding agents on a non-regular basis seems to be the reason for the apparent reduced response to the treatment of secondary hyperparathyroidism. Alkaline phosphatase has been shown to be a fair marker for bone turnover in patients with osteitis fibrosa. The severity of the clinical manifestations of bone disease correlates with the histological features of bone lesion and to the time spent on dialysis.

  17. Hydatid disease of bone: a mimic of other skeletal pathologies

    International Nuclear Information System (INIS)

    Morris, B.S.; Garg, A.; Chavhan, G.B.; Madiwale, C.V.

    2002-01-01

    Skeletal hydatidosis results from the deposition of the larval form of the Echinococcus, a genus of tapeworm. The incidence of bone disease is extremely low as most larvae are trapped by the liver and lung upon release of the embryo into the portal blood stream. The interpretation of imaging studies can prove very confusing because bone changes evolve with time, and the non-specificity of these findings often leads to a mistaken diagnosis. We present the case of a 35-year-old woman with long-standing pain in the left hip joint in which the findings on CT were thought of as being either tuberculous or neoplastic in nature. The result of a CT-guided biopsy and another done following surgery concurred on an unexpected diagnosis of a hydatid cyst. This case illustrates that in the absence of a high index of suspicion for echinococcal infection, the semblance of imaging findings of hydatid disease in bone to those of other skeletal pathologies can lead to misinterpretation. Copyright (2002) Blackwell Science Pty Ltd

  18. Bone marrow stroma in idiopathic myelofibrosis and other haematological diseases. An immunohistochemical study

    DEFF Research Database (Denmark)

    Lisse, I; Hasselbalch, H; Junker, P

    1991-01-01

    Bone marrow stroma was investigated immunohistochemically in 31 patients with haematological diseases, mainly idiopathic myelofibrosis (n = 8) and related chronic myeloproliferative disorders (n = 14). The bone marrow from patients with idiopathic myelofibrosis and some CML patients showed marked...

  19. Bone quality changes associated with aging and disease: a review.

    Science.gov (United States)

    Boskey, Adele L; Imbert, Laurianne

    2017-12-01

    Bone quality encompasses all the characteristics of bone that, in addition to density, contribute to its resistance to fracture. In this review, we consider changes in architecture, porosity, and composition, including collagen structure, mineral composition, and crystal size. These factors all are known to vary with tissue and animal ages, and health status. Bone morphology and presence of microcracks, which also contribute to bone quality, will not be discussed in this review. Correlations with mechanical performance for collagen cross-linking, crystallinity, and carbonate content are contrasted with mineral content. Age-dependent changes in humans and rodents are discussed in relation to rodent models of disease. Examples are osteoporosis, osteomalacia, osteogenesis imperfecta (OI), and osteopetrosis in both humans and animal models. Each of these conditions, along with aging, is associated with increased fracture risk for distinct reasons. © 2017 New York Academy of Sciences.

  20. Developmental Origins of Chronic Renal Disease: An Integrative Hypothesis

    Directory of Open Access Journals (Sweden)

    F. Boubred

    2013-01-01

    Full Text Available Cardiovascular diseases are one of the leading causes of mortality. Hypertension (HT is one of the principal risk factors associated with death. Chronic kidney disease (CKD, which is probably underestimated, increases the risk and the severity of adverse cardiovascular events. It is now recognized that low birth weight is a risk factor for these diseases, and this relationship is amplified by a rapid catch-up growth or overfeeding during infancy or childhood. The pathophysiological and molecular mechanisms involved in the “early programming” of CKD are multiple and partially understood. It has been proposed that the developmental programming of arterial hypertension and chronic kidney disease is related to a reduced nephron endowment. However, this mechanism is still discussed. This review discusses the complex relationship between birth weight and nephron endowment and how early growth and nutrition influence long term HT and CKD. We hypothesize that fetal environment reduces moderately the nephron number which appears insufficient by itself to induce long term diseases. Reduced nephron number constitutes a “factor of vulnerability” when additional factors, in particular a rapid postnatal growth or overfeeding, promote the early onset of diseases through a complex combination of various pathophysiological pathways.

  1. The Evolving World of Chronic Kidney Disease Mineral Bone Disorder

    Directory of Open Access Journals (Sweden)

    Antonio Bellasi

    2013-07-01

    Full Text Available Chronic kidney disease – mineral and bone disorder (CKD-MBD is associated with a significant morbidity and mortality. In vitro and animal models suggest that phosphorous, calcium, parathyroid hormone, and vitamin D abnormalities, mediate the cardiovascular and bone diseases that characterise CKD-MBD and increase the risk of death. Currently, mineral abnormalities are corrected through phosphorous restriction, phosphate binders, calcimimetics and vitamin D administration. Nonetheless, data in humans that support the use of these compounds are still scarce, mainly based on observational studies. Thus, a considerable number of doubts and questions still challenge clinicians dealing with CKD patients and mineral metabolism imbalances. We herein critically review clinical evidence that support the use of different drugs in CKD-MBD.

  2. Chronic Kidney Disease-Mineral Bone Disorder in the Elderly Peritoneal Dialysis Patient

    DEFF Research Database (Denmark)

    Heaf, James Goya

    2015-01-01

    PURPOSE: The purpose of this paper was to review the literature concerning the treatment of chronic kidney disease-mineral bone disorder (CKD-MBD) in the elderly peritoneal dialysis (PD) patient. ♦ RESULTS: Chronic kidney disease-mineral bone disorder is a major problem in the elderly PD patient......, with its associated increased fracture risk, vascular calcification, and accelerated mortality fracture risk. Peritoneal dialysis, however, bears a lower risk than hemodialysis (HD). The approach to CKD-MBD prophylaxis and treatment in the elderly PD patient is similar to other CKD patients, with some...

  3. Bone marrow accumulation in gallium scintigraphy in patients with adult still's disease

    Energy Technology Data Exchange (ETDEWEB)

    Kanegae, Futoshi; Tada, Yoshifumi; Ohta, Akihide; Ushiyama, Osamu; Suzuki; Noriaki; Koarada, Syuichi; Haruta, Yoshio; Yoshikai, Tomonori; Nagasawa, Kohei [Saga Medical School (Japan)

    2002-12-01

    We investigated the features and the usefulness of gallium scintigraphy in the diagnosis and the assessment of Adult Still's disease (ASD) by retrospective case review. Gallium scintigraphy have been done for 11 cases of ASD (3 males and 8 females) and 4 females were positive. Among these, 67 Ga-citrate was accumulated to the bone marrow in all 4 cases and to the major joints in 2 cases. Positive cases were rather serious and administered more immunosuppressants than negative cases. In order to characterize gallium scintigraphy findings of ASD, i.e. bone marrow accumulation, we analyzed 130 cases of collagen vascular disease. Although 101 cases (77.7%) were positive, only 7 cases (5.4%) showed the accumulation of {sup 67}Ga-citrate to the bone marrow. These include 3 cases with ASD, and 1 case with systemic lupus erythematosus, polyarteritis nodosa, Wegener's granulomatosis and Sjogren's syndrome. We also accumulated 18 patients who exhibited bone marrow accumulation of {sup 69}Ga-citrate, and found that 7 patients had collagen vascular and their related diseases. In conclusion, bone marrow accumulation in gallium scintigraphy is a specific feature of collagen vascular diseases, especially ASD, and it is suggested that cases with positive gallium scintigraphy in ASD can be serious and resistant to treatment. (author)

  4. Early detection of Freiberg's disease by radionuclide bone imaging

    International Nuclear Information System (INIS)

    Peng Jingjing

    1993-01-01

    56 hallux valgus deformities of 28 patients were studied with radionuclide bone imaging (RNBI). Among them, 24 feet(42.85%) revealed increased uptake of radioactivity in second or third metatarsal. The ratio of radioactivity in lesion and contralateral normal site (D/N) was increased, the difference between the patient and normal groups was significant (P<0.01). The histologic study showed that there have been degenerative changes and bone cell necrosis in increased uptake area. It was concluded that RNBI was more sensitive than X ray and can be used for the early diagnosis of Freiberg's Disease

  5. The use of bone scintigraphy to detect active Hansen's disease in mutilated patients

    International Nuclear Information System (INIS)

    Braga, F.J.H.N.; Foss, N.T.; Ferriolli, E.; Pagnano, C.; Miranda, J.R.A.; De Moraes, R.

    1999-01-01

    Mutilation of extremities was very frequent in patients affected by leprosy in the past; although it is now much less common, it is still seen, mainly in patients with long-term disease. In general, mutilation of the nose and ears is caused by the bacillus and mutilation of the hands and feet a consequence of chronic trauma. Leprosy must be chronically treated and any decision to interrupt therapy is based on laboratory tests and biopsy. Scintigraphy is a non-invasive procedure which could be of great value in to determining disease activity. We studied eight patients (five males and three females, aged 64-73 years) who presented with mutilation of the nose (2), ear (1), feet (3) or foot and hand (2). Conventional three-phase bone scintigraphy (750 MBq) and X-ray examinations of the affected areas were performed in all patients. Bone scintigraphy was abnormal in four patients (the presence of bacilli was confirmed by biopsy in two of them), and normal in the other four. In all patients except for the one with ear mutilation, radiography only showed the absence of bone. We conclude that bone scintigraphy is very useful to determine disease activity in cases of mutilation caused by leprosy. It seems to be superior to conventional radiography and may enable bone biopsies to be avoided. (orig.)

  6. Regulation of placental calcium transport and offspring bone health

    Directory of Open Access Journals (Sweden)

    Laura eGoodfellow

    2011-02-01

    Full Text Available Osteoporosis causes considerable morbidity and mortality in later life, and the risk of the disease is strongly determined by peak bone mass, which is achieved in early adulthood. Poor intrauterine and early childhood growth are associated with reduced peak bone mass, and increased risk of osteoporotic fracture in older age. In this review we describe the regulatory aspects of intrauterine bone development, and then summarise the evidence relating early growth to later fracture risk. Physiological systems include vitamin D, PTH; leptin; GH/ IGF-1; finally the potential role of epigenetic processes in the underlying mechanisms will be explored. Thus factors such as maternal lifestyle, diet, body build, physical activity and vitamin D status in pregnancy all appear to influence offspring bone mineral accrual. These data demonstrate a likely interaction between environmental factors and gene expression, a phenomenon ubiquitous in the natural world (developmental plasticity, as the potential key process. Intervention studies are now required to test the hypotheses generated by these epidemiological and physiological findings, to inform potential novel public health interventions aimed at improving childhood bone health and reducing the burden of osteoporotic fracture in future generations.

  7. The consequences of chronic kidney disease on bone metabolism and growth in children.

    Science.gov (United States)

    Bacchetta, Justine; Harambat, Jérôme; Cochat, Pierre; Salusky, Isidro B; Wesseling-Perry, Katherine

    2012-08-01

    Growth retardation, decreased final height and renal osteodystrophy (ROD) are common complications of childhood chronic kidney disease (CKD), resulting from a combination of abnormalities in the growth hormone (GH) axis, vitamin D deficiency, hyperparathyroidism, hypogonadism, inadequate nutrition, cachexia and drug toxicity. The impact of CKD-associated bone and mineral disorders (CKD-MBD) may be immediate (serum phosphate/calcium disequilibrium) or delayed (poor growth, ROD, fractures, vascular calcifications, increased morbidity and mortality). In 2012, the clinical management of CKD-MBD in children needs to focus on three main objectives: (i) to provide an optimal growth in order to maximize the final height with an early management with recombinant GH therapy when required, (ii) to equilibrate calcium/phosphate metabolism so as to obtain acceptable bone quality and cardiovascular status and (iii) to correct all metabolic and clinical abnormalities that can worsen bone disease, growth and cardiovascular disease, i.e. metabolic acidosis, anaemia, malnutrition and 25(OH)vitamin D deficiency. The aim of this review is to provide an overview of the mineral, bone and vascular abnormalities associated with CKD in children in terms of pathophysiology, diagnosis and clinical management.

  8. The Kidney-Vascular-Bone Axis in the Chronic Kidney Disease-Mineral Bone Disorder.

    Science.gov (United States)

    Seifert, Michael E; Hruska, Keith A

    2016-03-01

    The last 25 years have been characterized by dramatic improvements in short-term patient and allograft survival after kidney transplantation. Long-term patient and allograft survival remains limited by cardiovascular disease and chronic allograft injury, among other factors. Cardiovascular disease remains a significant contributor to mortality in native chronic kidney disease as well as cardiovascular mortality in chronic kidney disease more than doubles that of the general population. The chronic kidney disease (CKD)-mineral bone disorder (MBD) is a syndrome recently coined to embody the biochemical, skeletal, and cardiovascular pathophysiology that results from disrupting the complex systems biology between the kidney, skeleton, and cardiovascular system in native and transplant kidney disease. The CKD-MBD is a unique kidney disease-specific syndrome containing novel cardiovascular risk factors, with an impact reaching far beyond traditional notions of renal osteodystrophy and hyperparathyroidism. This overview reviews current knowledge of the pathophysiology of the CKD-MBD, including emerging concepts surrounding the importance of circulating pathogenic factors released from the injured kidney that directly cause cardiovascular disease in native and transplant chronic kidney disease, with potential application to mechanisms of chronic allograft injury and vasculopathy.

  9. [Cathepsin K as a biomarker of bone involvement in type 1 Gaucher disease].

    Science.gov (United States)

    Bobillo Lobato, Joaquín; Durán Parejo, Pilar; Núñez Vázquez, Ramiro J; Jiménez Jiménez, Luis M

    2015-10-05

    Gaucher disease is an inherited disorder caused by deficit of acid β-glucocerebrosidase, responsible for the degradation of glucosylceramide to ceramide and glucose. Although the disorder is primarily hematologic, bone is the second most commonly affected structure. Cathepsin K (CATK) is an enzyme involved in bone remodelling process. It has been proposed that determination of its serum concentrations may provide additional information to other biomarkers. The study included 20 control subjects and 20 Gaucher type 1 patients from Andalusia and Extremadura regions. We analyzed the biomarkers of bone remodelling: the bone alkaline phosphatase (B-ALP), the N-terminal propeptide of type 1 procollagen (P1NP), the β carboxyterminal telopeptide of type 1 collagen (CTx) and the CATK through electrochemiluminescence and immunoassay techniques. There is an increase in levels of CATK, CATK/P1NP and CATK/B-ALP ratios in type 1 Gaucher patients compared to the control group. Considering the existence of skeletal manifestations in the patient group, the CATK and CATK/P1NP ratio showed higher levels in patients with bone damage compared to those without it. Although imaging studies are the gold standard for monitoring bone disease in type 1 Gaucher patients, the utility of CATK should be considered as a possible indicator of bone damage in these patients. Furthermore, this parameter can be used in the monitoring of the treatment of bone pathology. Copyright © 2015 Elsevier España, S.L.U. All rights reserved.

  10. Pervasive developmental disorder behavior in adolescents with intellectual disability and co-occurring somatic chronic diseases

    NARCIS (Netherlands)

    Oeseburg, B.; Groothoff, J. W.; Dijkstra, G. J.; Reijneveld, S. A.; Jansen, D. E. M. C.

    2010-01-01

    Evidence on the association between somatic chronic diseases in ID-adolescents and the full range of pervasive developmental disorder behavior (PDD behavior) is scarce. The aim of the present study is to assess the association between somatic chronic diseases in ID-adolescents and mild PDD behavior.

  11. Improvement of Lumbar Bone Mass after Infliximab Therapy in Crohn’s Disease Patients

    Directory of Open Access Journals (Sweden)

    Marina Mauro

    2007-01-01

    Full Text Available BACKGROUND: Patients with Crohn’s disease (CD have a high risk of developing osteoporosis, but the mechanisms underlying bone mass loss are unclear. Elevated proinflammatory cytokines, such as tumour necrosis factor-alpha (TNFα, have been implicated in the pathogenesis of bone resorption.

  12. Lumbar spine degenerative disease : effect on bone mineral density measurements in the lumbar spine and femoral neck

    International Nuclear Information System (INIS)

    Juhng, Seon Kwan; Koplyay, Peter; Jeffrey Carr, J.; Lenchik, Leon

    2001-01-01

    To determine the effect of degenerative disease of the lumbar spine on bone mineral density in the lumbar spine and femoral neck. We reviewed radiographs and dual energy x-ray absorptiometry scans of the lumbar spine and hip in 305 Caucasian women with suspected osteoporosis. One hundred and eight-six patient remained after excluding women less than 40 years of age (n=18) and those with hip osteoarthritis, scoliosis, lumbar spine fractures, lumbar spinal instrumentation, hip arthroplasty, metabolic bone disease other than osteoporosis, or medications known to influence bone metabolism (n=101). On the basis of lumbar spine radiographs, those with absent/mild degenerative disease were assigned to the control group and those with moderate/severe degenerative disease to the degenerative group. Spine radiographs were evaluated for degenerative disease by two radiologists working independently; discrepant evaluations were resolved by consensus. Lumbar spine and femoral neck bone mineral density was compared between the two groups. Forty-five (24%) of 186 women were assigned to the degenerative group and 141 (76%) to the control group. IN the degenerative group, mean bone mineral density measured 1.075g/cm? in the spine and 0.788g/cm 2 in the femoral neck, while for controls the corresponding figures were 0.989g/cm 2 and 0.765g/cm 2 . Adjusted for age, weight and height by means of analysis of variance, degenerative disease of the lumbar spine was a significant predictor of increased bone mineral density in the spine (p=0.0001) and femoral neck (p=0.0287). Our results indicate a positive relationship between degenerative disease of the lumbar spine and bone mineral density in the lumbar spine and femoral neck, and suggest that degenerative disease in that region, which leads to an intrinsic increase in bone mineral density in the femoral neck, may be a good negative predictor of osteoporotic hip fractures

  13. Diffuse bone marrow infiltration in neoplastic hematological disease. Comparison between MR imaging and histopathological findings

    International Nuclear Information System (INIS)

    Kozawa, Eito; Sato, Youichi; Heshiki, Atsuko; Kayano, Shuuichi

    2005-01-01

    The purpose of this study was to compare the signal intensity ratio (SIR) between out-of-phase and in-phase imaging with pathologic data of patients with bone marrow invasion by tumor-like hematological disease. Twenty-three patients with hematological disease (malignant lymphoma [10], multiple myeloma [7], leukemia [2], myelodysplastic syndrome [MDS; 3], and myelofibrosis [1]) were studied. Fast low angle shot (FLASH) sequencing was performed to obtain out-of-phase and in-phase images with breath-holding at 110/2.3 and 4.7. Out-of-phase and in-phase imaging were measured over a region of interest (ROI) at spinal vertebra L3, and SIR (out of phase/in phase) was calculated. Results were confirmed by bone marrow aspiration or biopsy. Patients with hematological disease were divided into those with and without diffuse bone marrow infiltration. The statistical significance between these ratios in the two groups was assessed by unpaired t-test (p<0.01). The SIRs were 0.94±0.12 (mean±SD) for the group with diffuse bone marrow infiltration and 0.54±0.17 (mean±SD) for the group without (p<0.01). In-phase and out-of-phase imaging can be helpful in predicting the diffuse infiltration of bone marrow by hematological disease. (author)

  14. Bone mass and vitamin D levels in Parkinson's disease: is there any difference between genders?

    Science.gov (United States)

    Ozturk, Erhan Arif; Gundogdu, Ibrahim; Tonuk, Burak; Kocer, Bilge Gonenli; Tombak, Yasemin; Comoglu, Selcuk; Cakci, Aytul

    2016-08-01

    [Purpose] The aim of this study was to determine the bone mineral density, vitamin D level, and frequencies of osteopenia and osteoporosis in patients with Parkinson's disease and to compare male and female patients with the controls separately. [Subjects and Methods] One hundred fifteen Parkinson's disease patients (47 males, 68 females; age range: 55-85 years) and 117 age- and gender-matched controls (47 males, 70 females) were enrolled in the study. Bone mineral density measured by dual-energy X-ray absorptiometry and serum D vitamin levels of each participant were recorded. [Results] The mean lumbar spine, femur neck, and total femur bone mineral density levels, T-scores, and vitamin D levels were found to be significantly lower in Parkinson's disease patients in both genders. Furthermore, osteoporosis rates were found be significantly higher only in female Parkinson's disease patients compared with female controls. [Conclusion] Data from the present study revealed that while osteoporosis was significantly higher only in female Parkinson's disease patients, all Parkinson's disease patients had lower bone mineral density scores and vitamin D levels compared with the controls regardless of gender, suggesting that clinicians should pay attention to the osteoporosis risk in Parkinson's disease and that adequate preventive measures should be taken in order to limit the future risk due to osteoporotic fractures.

  15. Bilateral orbital bone infarction in sickle-cell disease.

    Science.gov (United States)

    Ghafouri, Roya H; Lee, Irene; Freitag, Suzanne K; Pira, Tony N

    2011-01-01

    This is a case of a 2-year-old boy with sickle cell disease who presented with bilateral eyelid swelling, limited extraocular motility, and lateral subperiosteal fluid collection associated with bilateral lateral orbital wall infarctions on MRI. The patient was managed medically with intravenous fluids, analgesics, broad-spectrum antibiotics, systemic steroids, and clinically improved. Patients with sickle cell disease are susceptible to infarction of the orbital bones during vaso-occlusive crises. Orbital wall infarction can lead to acute proptosis and restricted extraocular motility. Orbital wall infarction should be considered in sickle cell patients with orbital diseases so that appropriate treatment can be instituted promptly to prevent the serious sequelae of orbital compression syndrome.

  16. Measurement of bone mineral content by dual photon absorptiometry in patients with metabolic bone diseases

    International Nuclear Information System (INIS)

    Ohtani, Masami; Hino, Megumu; Ikekubo, Katsuji

    1991-01-01

    Dual photon absorptiometry was used to measure bone mineral content in 225 patients with metabolic bone diseases (84 males and 102 females) and 186 healthy subjects (25 males and 200 females). Mineral content of the lumbar vertebrae tended to rapidly decrease after the age of 40 in healthy female subjects. For males, gradual decrease in mineral content was associated with aging. Bone mineral content showed a correlation with the severity of osteoporosis as shown on X-ray films. Mineral content tended to be decreased in the lumbar vertebrae in patients with vertebral compression fracture, and in the femur in patients with vertebral or femoral fracture. For hyperthyroidism, mineral content of the lumbar vertebrae was decreased in some females, but was within normal limit in males. Hyperparathyroidism and hypoparathyroidism tended to be associated with decrease and increase in mineral content, respectively. Two each patients with osteomalacia or Cushing syndrome had a decreased mineral content. In these patients, it was increased after the treatment. (N.K.)

  17. Measurement of bone mineral content by dual photon absorptiometry in patients with metabolic bone diseases

    Energy Technology Data Exchange (ETDEWEB)

    Ohtani, Masami; Hino, Megumu; Ikekubo, Katsuji (Kobe City General Hospital (Japan)) (and others)

    1991-12-01

    Dual photon absorptiometry was used to measure bone mineral content in 225 patients with metabolic bone diseases (84 males and 102 females) and 186 healthy subjects (25 males and 200 females). Mineral content of the lumbar vertebrae tended to rapidly decrease after the age of 40 in healthy female subjects. For males, gradual decrease in mineral content was associated with aging. Bone mineral content showed a correlation with the severity of osteoporosis as shown on X-ray films. Mineral content tended to be decreased in the lumbar vertebrae in patients with vertebral compression fracture, and in the femur in patients with vertebral or femoral fracture. For hyperthyroidism, mineral content of the lumbar vertebrae was decreased in some females, but was within normal limit in males. Hyperparathyroidism and hypoparathyroidism tended to be associated with decrease and increase in mineral content, respectively. Two each patients with osteomalacia or Cushing syndrome had a decreased mineral content. In these patients, it was increased after the treatment. (N.K.).

  18. Gaucher disease: MR evaluation of bone marrow features during treatment with enzyme replacement

    International Nuclear Information System (INIS)

    Poll, L.W.; Koch, J.A.; Boerner, D.; Cohnen, M.; Jung, G.; Scherer, A.; Moedder, U.; Niederau, C.

    2001-01-01

    Purpose: Enzyme replacement therapy (ERT) arrests and reverses the hematological and visceral symptoms of adult Gaucher disease, the most frequent lysosomal storage disorder. There are only a few studies available evaluating bone disease during ERT. The aim of this study was to investigate the features of bone marrow (bm) by magnetic resonance imaging (MRI) in these patients during ERT. Materials and Methods: MRI was performed prospectively in thirty adult type I Gaucher patients before and during ERT with a mean follow-up of 3 years. Spin-echo sequences (T 1 /T 2 ) of the lower extremities were obtained and the reconversion (response) or lack of reconversion (non-response) to fatty marrow during treatment was analyzed. The morphological features of bm involvement, a homogeneous or non-homogeneous distribution of bm changes and focal bone lesions surrounded by a rim of reduced signal intensity (SI), were analyzed. Results: Infiltration of bm by Gaucher cells is characterized by a reduction of Sl on both T 1 - and T 2 -weighted sequences. Bone marrow responses were seen in 19 patients (63%) during treatment. Focal bone lesions, surrounded by a rim of reduced Sl, did not respond to ERT and correlated with a non-homogenous distribution of bone involvement and splenectomy. (orig.) [de

  19. Parental tobacco smoke exposure: Epigenetics and the developmental origins of health and disease

    Science.gov (United States)

    Epigenetic programming is an important mechanism underlying the Developmental Origins of Health and Disease (DOHaD). Much of the research in this area has focused on maternal nutrition. Parental smoking has emerged as a prime example of how exposure to environmental toxicants dur...

  20. Surgery or radiotherapy for the treatment of bone hydatid disease: a retrospective case series

    Directory of Open Access Journals (Sweden)

    Zengru Xie

    2015-04-01

    Conclusion: This retrospective case series describes, for the first time, the clinical outcomes in a series of patients treated with radiotherapy for bone hydatid disease. Although no direct comparison between the treatment groups could be made due to methodological limitations of the study design, this study indicates that well-designed prospective randomized controlled clinical trials assessing radiotherapy may be warranted in patients with inoperable hydatid disease of the bones.

  1. [Synthetic human calcitonin in Paget's disease of bone and osteoporosis (author's transl)].

    Science.gov (United States)

    Nuti, R; Vattimo, A

    1981-01-30

    Synthetic human calcitonin was used in the treatment of 26 patients over a period of 1-14 months. 17 patients had Paget's disease of the bone, 6 postmenopausal osteoporosis and 3 Sudeck's syndrome. Subjective improvement (reduction of pain, improvement of mobility) was found in 15 patients with Paget's disease, in 4 females with postmenopausal osteoporosis and in all 3 patients with Sudeck's syndrome. Radiographic improvement of bone changes developed only very slowly. These results were confirmed by diminution of the exchangeable calcium pool indicating reduction of rates of osseous degradation. Calcitonin tolerance was acceptable. Transitory nausea and occasional vomiting occurred in 3 patients.

  2. Autologous Bone Marrow Stem Cell Infusion (AMBI therapy for Chronic Liver Diseases

    Directory of Open Access Journals (Sweden)

    Rajkumar JS

    2007-01-01

    Full Text Available Liver Cirrhosis is the end stage of chronic liver disease which may happen due to alcoholism, viral infections due to Hepatitis B, Hepatitis C viruses and is difficult to treat. Liver transplantation is the only available definitive treatment which is marred by lack of donors, post operative complications such as rejection and high cost. Autologous bone marrow stem cells have shown a lot of promise in earlier reported animal studies and clinical trials. We have in this study administered in 22 patients with chronic liver disease, autologous bone marrow stem cell whose results are presented herewith.

  3. Computed tomography of the petrous bone in otosclerosis and Meniere's disease

    International Nuclear Information System (INIS)

    Groot, J.A.M. de.

    1987-01-01

    The aim of this investigation was to examine if, in the case of otosclerosis and Meniere's disease, one could obtain more information with high resolution computer tomography than with conventional polytomography. In the first part of this thesis a brief survey of some principles of computer tomography is given and a computer tomographic atlas of the normal petrous bone in the otoradiologic planes used in this study. In the introduction of the second part some aspects of otosclerose are discussed (definition, clinical aspects). Subsequently a treatment is given of a part of the histopathology referring to the otospongiotic and otosclerotic changes in the labyrinthine capsule. A survey is given of preceding studies on the petrous bone in the case of otosclerosis; in these studies mostly classical planigraphic techniques were employed. In the third part some general aspects of Meniere's disease are discussed: definition, incidence, clinical aspects and pathophysiology. It is assumed that the symptoms of Meniere's disease are caused by an overpressure in the endolymphatic areas of the labyrinthum ('hydrops'), however till now a proof for this is still not given. The ductus endolympathicus, which passes through a channel in the petrous bone (the vestibular aqueduct), plays a role in the drainage of endolymph. Stagnation of this drainage could cause hydrops. (Auth.)

  4. Prevalence of low bone mineral density in children and adolescents with celiac disease under treatment

    Directory of Open Access Journals (Sweden)

    Maria Eugênia Farias Almeida Motta

    Full Text Available CONTEXT AND OBJECTIVE: Low bone mineral density may be a finding among children and adolescents with celiac disease, including those undergoing treatment with a gluten-free diet, but the data are contradictory. The aim of this study was to determine the frequency of bone mineral density abnormalities in patients on a gluten-free diet, considering age at diagnosis and duration of dietary treatment. DESIGN AND SETTING: Cross-sectional prevalence study at the Pediatric Gastroenterology Outpatient Clinic of Instituto Materno Infantil Professor Fernando Figueira. METHODS: Thirty-one patients over five years of age with celiac disease and on a gluten-free diet were enrolled. Bone mineral density (in g/cm² was measured in the lumbar spine and whole body using bone densitometry and categorized using the criteria of the International Society for Clinical Densitometry, i.e. low bone mineral density for chronological age < -2.0 Z-scores. Age at diagnosis and duration of dietary treatment were confirmed according to the date of starting the gluten-free diet. RESULTS: Low bone density for chronological age was present in 3/31 patients in the lumbar spine and 1/31 in the whole body (also with lumbar spine abnormality. At diagnosis, three patients with low bone mineral density for the chronological age were more than 7.6 years old. These patients had been on a gluten-free diet for six and seven months and 3.4 years. CONCLUSION: Pediatric patients with celiac disease on long-term treatment are at risk of low bone mineral density. Early diagnosis and long periods of gluten-free diet are directly implicated in bone density normalization.

  5. MicroRNAs: Potential Biomarkers and Therapeutic Targets for Alveolar Bone Loss in Periodontal Disease

    Directory of Open Access Journals (Sweden)

    Tadayoshi Kagiya

    2016-08-01

    Full Text Available Periodontal disease is an inflammatory disease caused by bacterial infection of tooth-supporting structures, which results in the destruction of alveolar bone. Osteoclasts play a central role in bone destruction. Osteoclasts are tartrate-resistant acid phosphatase (TRAP-positive multinucleated giant cells derived from hematopoietic stem cells. Recently, we and other researchers revealed that microRNAs are involved in osteoclast differentiation. MicroRNAs are novel, single-stranded, non-coding, small (20–22 nucleotides RNAs that act in a sequence-specific manner to regulate gene expression at the post-transcriptional level through cleavage or translational repression of their target mRNAs. They regulate various biological activities such as cellular differentiation, apoptosis, cancer development, and inflammatory responses. In this review, the roles of microRNAs in osteoclast differentiation and function during alveolar bone destruction in periodontal disease are described.

  6. Oral Health and Bone Disease

    Science.gov (United States)

    ... that the loss of alveolar bone mineral density leaves bone more susceptible to periodontal bacteria, increasing the ... bone density will have a favorable impact on dental health. Bisphosphonates, a group of medications available for the treatment of osteoporosis, have been linked to the development ...

  7. Bone tumors

    International Nuclear Information System (INIS)

    Unni, K.K.

    1988-01-01

    This book contains the proceedings on bone tumors. Topics covered include: Bone tumor imaging: Contribution of CT and MRI, staging of bone tumors, perind cell tumors of bone, and metastatic bone disease

  8. Development of a molecular test of Paget's disease of bone.

    Science.gov (United States)

    Guay-Bélanger, Sabrina; Simonyan, David; Bureau, Alexandre; Gagnon, Edith; Albert, Caroline; Morissette, Jean; Siris, Ethel S; Orcel, Philippe; Brown, Jacques P; Michou, Laëtitia

    2016-03-01

    Depending on populations, 15 to 40% of patients have a familial form of Paget's disease of bone (PDB), which is transmitted in an autosomal-dominant mode of inheritance with incomplete penetrance. To date, only SQSTM1 gene mutations have been linked to the disease. Several single nucleotide polymorphisms (SNPs) have been associated with PDB in patient non-carriers of SQSTM1 mutations, but they have minor size effects. The current clinical practice guidelines still recommend to measure total serum alkaline phosphatase (sALP) for PDB screening. However, genetic or bone biomarkers alone may lack sensitivity to detect PDB. Thus, the objective of this study was to develop a molecular test of PDB, combining genetic and bone biomarkers, in order to detect PDB, which is frequently asymptomatic. We genotyped 35 SNPs previously associated with PDB in 305 patients, and 292 healthy controls. In addition, serum levels of 14 bone biomarkers were assayed in 51 patients and 151 healthy controls. Bivariate and multivariate logistic regression models with adjustment for age and sex were fitted to search for a combination of SNPs and/or bone biomarkers that could best detect PDB in patient non-carriers of SQSTM1 mutations. First, a combination of five genetic markers gave rise to the highest area under the ROC curve (AUC) with 95% confidence interval [95% CI] of 0.731 [0.688; 0.773], which allowed us to detect 81.5% of patients with PDB. Second, a combination of two bone biomarkers had an AUC of 0.822 [0.726; 0.918], and was present in 81.5% of patients with PDB. Then, the combination of the five genetic markers and the two bone biomarkers increased the AUC up to 0.892 [0.833; 0.951], and detected 88.5% of patients with PDB. These results suggested that an algorithm integrating first a screen for SQSTM1 gene mutations, followed by either a genetic markers combination or a combined genetic and biochemical markers test in patients non-carrier of any SQSTM1 mutation, may detect the PDB

  9. Occult Metabolic Bone Disease in Chronic Pancreatitis | Hari Kumar ...

    African Journals Online (AJOL)

    Background: Chronic pancreatitis (CP) leads to malabsorption and metabolic bone disease (MBD). Alcoholic CP (ACP) and tropical CP (TCP) are the two common types of CP. Objective: We investigated the presence of occult MBD in patients with CP and compared the same between ACP and TCP. Materials and Methods: ...

  10. A New Murine Model of Chronic Kidney Disease-Mineral and Bone Disorder

    Directory of Open Access Journals (Sweden)

    Bianca Frauscher

    2017-01-01

    Full Text Available Chronic kidney disease (CKD is associated with mineral and bone disorder (MBD, which is the main cause of the extensively increased cardiovascular mortality in the CKD population. We now aimed to establish a new murine experimental CKD-MBD model. Dilute brown non-Agouti (DBA/2 mice were fed with high-phosphate diet for 4 (HPD4 or 7 (HPD7 days, then with standard chow diet (SCD and subsequently followed until day 84. They were compared to DBA/2 mice maintained on SCD during the whole study period. Both 4 and 7 days HPD-fed mice developed phosphate nephropathy with tubular atrophy, interstitial fibrosis, decreased glomerular filtration rate, and increased serum urea levels. The abdominal aorta of HPD-treated mice showed signs of media calcification. Histomorphometric analysis of HPD-treated mice showed decreased bone volume/tissue volume, low mineral apposition rate, and low bone formation rate as compared to SCD-fed mice, despite increased parathyroid hormone levels. Overall, the observed phenotype was more pronounced in the HPD7 group. In summary, we established a new, noninvasive, and therefore easy to perform reproducible CKD-MBD model, which showed media calcification, secondary hyperparathyroidism, and low-turnover bone disease.

  11. Two Cases of Sternal 'Cold' Lesions on Bone Imaging in the Metastatic Skeletal Disease

    Energy Technology Data Exchange (ETDEWEB)

    Park, Hyung Gun; Seo, Bong Kwan; Lee, Hoon Yong; Lee, Myung Chul; Choi, Sung Jae; Kim, Noe Kyeong; Koh, Chang Soon [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1983-09-15

    Traditionally, a positive bone scan shows single or multiple areas of increased uptake in them metastatic skeletal disease. The occurrence of 'cold' lytic-like or photon-deficient lesions in bone imaging is probably uncommon. Photon-deficient focus or cold lesion of the sternum was demonstrated on {sup 99m}Tc-MDP bone imaging in 2 individuals with acute myeloid leukemia and primary hepatoma, respectively.

  12. Responsibility in the age of Developmental Origins of Health and Disease (DOHaD) and epigenetics.

    Science.gov (United States)

    Ismaili M'hamdi, H; de Beaufort, I; Jack, B; Steegers, E A P

    2018-02-01

    Insights from the Developmental Origins of Health and Disease paradigm and epigenetics are elucidating the biological pathways through which social and environmental signals affect human health. These insights prompt a serious debate about how the structure of society affects health and what the responsibility of society is to counteract health inequalities. Unfortunately, oversimplified interpretations of insights from Developmental Origins of Health and Disease and epigenetics may be (mis)used to focus on the importance of individual responsibility for health rather than the social responsibility for health. In order to advance the debate on responsibility for health, we present an ethical framework to determine the social responsibility to counteract health inequalities. This is particularly important in a time where individual responsibility often justifies a passive response from policymakers.

  13. Pervasive Developmental Disorder Behavior in Adolescents with Intellectual Disability and Co-Occurring Somatic Chronic Diseases

    Science.gov (United States)

    Oeseburg, B.; Groothoff, J. W.; Dijkstra, G. J.; Reijneveld, S. A.; Jansen, D. E. M. C.

    2010-01-01

    Evidence on the association between somatic chronic diseases in ID-adolescents and the full range of pervasive developmental disorder behavior (PDD behavior) is scarce. The aim of the present study is to assess the association between somatic chronic diseases in ID-adolescents and mild PDD behavior. We obtained data on 1044 ID-adolescents, aged…

  14. Radiological features of Paget disease of bone associated with VCP myopathy

    Energy Technology Data Exchange (ETDEWEB)

    Farpour, Farzin [University of California, Department of Radiology, VA Long Beach Health Care, Irvine, CA (United States); Queens Hospital Center, Mount Sinai School of Medicine, New York, NY (United States); Tehranzadeh, Jamshid [University of California, Department of Radiology, VA Long Beach Health Care, Irvine, CA (United States); Donkervoort, Sandra; Vanjara, Pari [University of California, Division of Genetics and Metabolism, Department of Pediatrics, Irvine, CA (United States); Smith, Charles [University of Kentucky, Department of Neurology and Sanders-Brown Center on Aging, Lexington, KY (United States); Martin, Barbara [University of Kentucky, Lexington, KY (United States); Osann, Kathryn [University of California, Department of Medicine, Division of Hematology/Oncology, Irvine, CA (United States); Kimonis, Virginia E. [University of California, Division of Genetics and Metabolism, Department of Pediatrics, Irvine, CA (United States); UC Irvine Medical Center, Division of Genetics and Metabolism, Orange, CA (United States)

    2012-03-15

    Mutations in the Valosin-containing protein (VCP) gene cause a unique disorder characterized by classic Paget disease of bone (PDB), inclusion body myopathy, and frontotemporal dementia (IBMPFD). Our objective was to analyze the radiographic features of PDB associated with VCP mutations since there is a dearth of literature on the PDB component of VCP disease. Radiographic bone surveys were examined in 23 individuals with VCP mutation and compared with their unaffected relatives. Laboratory testing relevant for VCP disease was performed in all individuals. Of the 17 affected individuals with clinical manifestations of VCP disease, 16 of whom had myopathy, radiographic analysis revealed classic PDB in 11 individuals (65%). The mean age of diagnosis for myopathy was 43.8 years and for PDB was 38.1 years of age. Radiological evidence of PDB was seen in one individual (16%) amongst six clinically asymptomatic VCP mutation carriers. Alkaline phosphatase was a useful marker for diagnosing PDB in VCP disease. Radiographic findings of classic PDB are seen in 52% of individuals carrying VCP mutations at a significantly younger age than conventional PDB. Screening for PDB is warranted in at-risk individuals because of the benefit of early treatment with the new powerful bisphosphonates that hold the potential for prevention of disease. (orig.)

  15. Gender peculiarities of bone mineral density in patients with Parkinson’s disease

    Directory of Open Access Journals (Sweden)

    V.V. Povoroznyuk

    2018-02-01

    Full Text Available Background. Parkinson’s disease is a chronic progressive disease of the brain manifested by a combination of hypokinesia with rigidity, resting tremor and postural instability, as well as a wide spectrum of non-motor manifestations accompanied by an increased risk of osteoporotic fractures. The purpose of the study was to study the sexual characteristics of bone mineral density in patients with Parkinson’s disease. Materials and methods. Eighty nine patients with Parkinson’s disease and 89 patients of the corresponding age and sex without neurologic pathology were examined. Women and men of both groups did not differ in age, height and body weight. The bone mineral density (BMD was determined by dual energy X-ray absorptiometry. Results. Osteoporosis in patients with Parkinson’s disease was registered significantly more often in women than in men (48 and 24.3 %, respectively and significantly more often than in the control group. Significantly lower BMD was registered in patients with Parkinson’s disease compared with those of the control group: in men — at the level of the femoral neck, the distal forearm and the total body, in women — at the level of the lumbar spine, femoral neck and total hip, distal forearm and total body. The negative correlation of the BMD in different regions of the skeleton and age was recorded in women, in men this relationship was registered at the level of the distal forearm. The positive correlation was found between the BMD and body mass index in men and women. Conclusions. The results of our study suggest that Parkinson’s disease leads to the development of osteoporosis, but the condition of bone tissue in patients with Parkinson’s disease has gender features, in particular, the BMD in women is more dependent on age, and in men — on body mass index.

  16. Vitamin D and Bone Disease

    Directory of Open Access Journals (Sweden)

    S. Christodoulou

    2013-01-01

    Full Text Available Vitamin D is important for normal development and maintenance of the skeleton. Hypovitaminosis D adversely affects calcium metabolism, osteoblastic activity, matrix ossification, bone remodeling and bone density. It is well known that Vit. D deficiency in the developing skeleton is related to rickets, while in adults is related to osteomalacia. The causes of rickets include conditions that lead to hypocalcemia and/or hypophosphatemia, either isolated or secondary to vitamin D deficiency. In osteomalacia, Vit. D deficiency leads to impairment of the mineralisation phase of bone remodeling and thus an increasing amount of the skeleton being replaced by unmineralized osteoid. The relationship between Vit. D and bone mineral density and osteoporosis are still controversial while new evidence suggests that Vit. D may play a role in other bone conditions such as osteoarthritis and stress fractures. In order to maintain a “good bone health” guidelines concerning the recommended dietary intakes should be followed and screening for Vit. D deficiency in individuals at risk for deficiency is required, followed by the appropriate action.

  17. Tibial and fibular developmental fields defects

    International Nuclear Information System (INIS)

    Khoury, N.J.; Haddad, M.C.; Hourani, M.H.

    1999-01-01

    Malformations of the lower limbs are rare and heterogeneous anomalies. To explain the diversity and complexity of these abnormalities, authors introduced the concept of tibial and fibular developmental fields. Defects in these fields are responsible for different malformations, which have been described, to our knowledge, in only one report in the radiology literature. We present a case of a newborn with femoral bifurcation, absent fibulae and talar bones, ankle and foot malformations, and associated atrial septal defect. Our case is an example of defects in both fibular and tibial developmental fields. (orig.)

  18. Bone mineral density and nutritional status in children with chronic inflammatory bowel disease

    NARCIS (Netherlands)

    A.M. Boot (Annemieke); J. Bouquet (Jan); E.P. Krenning (Eric); S.M.P.F. de Muinck Keizer-Schrama (Sabine)

    1998-01-01

    textabstractBACKGROUND: Osteoporosis has been reported in adult patients with inflammatory bowel disease. AIMS: To evaluate bone mineral density (BMD), nutritional status, and determinants of BMD in children with inflammatory bowel disease. PATIENTS: Fifty five patients

  19. Roentgenological semiotics of bone and bone joints pathology

    International Nuclear Information System (INIS)

    Zedgenidze, G.A.; Kishkovskij, A.N.; Elashov, Yu.G.

    1984-01-01

    Physiologic and pathologic processes in bones followed by alternations of bone structure and reflected on roentgenograms are considered and described. Most frequent reasons for roentgenodiagnosis errors in diseases of bone and bone joint apparatus are presented

  20. Dynamic contrast-enhanced MR imaging of the water fraction of normal bone marrow and diffuse bone marrow disease

    Energy Technology Data Exchange (ETDEWEB)

    Katsuya, Tomoo; Inoue, Tomio; Ishizaka, Hiroshi; Aoki, Jun; Endo, Keigo [Gunma Univ., Maebashi (Japan). School of Medicine

    2000-10-01

    To clarify the contrast-enhancement pattern of the normal hematopoietic element by isolating the signal of the water fraction in vertebral bone marrow and to investigate whether this approach can be used to characterize bone marrow pathology in several diffuse bone marrow diseases. Two groups were examined: 30 normal healthy volunteers and 19 patients with primary diffuse bone marrow disease (aplastic anemia [n=8], myelodysplastic syndrome (MDS) [n=5], chronic myelogenic leukemia (CML) [n=4], polycythemia vera [n=2]). Isolation of the signal of hematopoietic tissue was done by the chemical-shift misregistration effect. Twenty consecutive T1-weighted midsagittal lumber vertebral images were obtained immediately after the intravenous administration of Gd-DTPA of 0.1 mmol/kg body weight, and the pattern of the time-intensity curve, the peak contrast-enhancement (CE) ratio, and the washout rate (%/min) of bone marrow in normal volunteers were compared with those in patients suffering from primary diffuse bone marrow disease. The pattern of the time-intensity curve of patients with aplastic anemia showed a low peak value followed by a slow washout. However, the pattern of time-intensity curves in patients with MDS, CML, and polycythemia vera was similar to that of normal volunteers. The peak CE ratio of the water fraction in normal marrow ranged from 0.45 to 1.26 (mean {+-}S.D.: 0.87{+-}0.18). Patients with aplastic anemia showed an abnormally lower peak CE ratio of the water fraction (mean {+-}S.D.: 0.34{+-}0.19, p<0.0001). On the other hand, the peak CE ratio of the water fraction in patients with MDS was significantly higher than that of normal volunteers (mean {+-}S.D. 1.35{+-}0.39, p<0.05). In contrast, the peak CE ratio of patients with CML or polycythemia vera did not differ significantly from that of normal volunteers. The mean washout rate of patients with aplastic anemia was significantly lower than that of normal volunteers (mean {+-}S.D.: 3.50{+-}2.51 %/min

  1. Dynamic contrast-enhanced MR imaging of the water fraction of normal bone marrow and diffuse bone marrow disease

    International Nuclear Information System (INIS)

    Katsuya, Tomoo; Inoue, Tomio; Ishizaka, Hiroshi; Aoki, Jun; Endo, Keigo

    2000-01-01

    To clarify the contrast-enhancement pattern of the normal hematopoietic element by isolating the signal of the water fraction in vertebral bone marrow and to investigate whether this approach can be used to characterize bone marrow pathology in several diffuse bone marrow diseases. Two groups were examined: 30 normal healthy volunteers and 19 patients with primary diffuse bone marrow disease (aplastic anemia [n=8], myelodysplastic syndrome (MDS) [n=5], chronic myelogenic leukemia (CML) [n=4], polycythemia vera [n=2]). Isolation of the signal of hematopoietic tissue was done by the chemical-shift misregistration effect. Twenty consecutive T1-weighted midsagittal lumber vertebral images were obtained immediately after the intravenous administration of Gd-DTPA of 0.1 mmol/kg body weight, and the pattern of the time-intensity curve, the peak contrast-enhancement (CE) ratio, and the washout rate (%/min) of bone marrow in normal volunteers were compared with those in patients suffering from primary diffuse bone marrow disease. The pattern of the time-intensity curve of patients with aplastic anemia showed a low peak value followed by a slow washout. However, the pattern of time-intensity curves in patients with MDS, CML, and polycythemia vera was similar to that of normal volunteers. The peak CE ratio of the water fraction in normal marrow ranged from 0.45 to 1.26 (mean ±S.D.: 0.87±0.18). Patients with aplastic anemia showed an abnormally lower peak CE ratio of the water fraction (mean ±S.D.: 0.34±0.19, p<0.0001). On the other hand, the peak CE ratio of the water fraction in patients with MDS was significantly higher than that of normal volunteers (mean ±S.D. 1.35±0.39, p<0.05). In contrast, the peak CE ratio of patients with CML or polycythemia vera did not differ significantly from that of normal volunteers. The mean washout rate of patients with aplastic anemia was significantly lower than that of normal volunteers (mean ±S.D.: 3.50±2.51 %/min vs. 7.13±1

  2. Bone mass and vitamin D levels in Parkinson’s disease: is there any difference between genders?

    Science.gov (United States)

    Ozturk, Erhan Arif; Gundogdu, Ibrahim; Tonuk, Burak; Kocer, Bilge Gonenli; Tombak, Yasemin; Comoglu, Selcuk; Cakci, Aytul

    2016-01-01

    [Purpose] The aim of this study was to determine the bone mineral density, vitamin D level, and frequencies of osteopenia and osteoporosis in patients with Parkinson’s disease and to compare male and female patients with the controls separately. [Subjects and Methods] One hundred fifteen Parkinson’s disease patients (47 males, 68 females; age range: 55–85 years) and 117 age- and gender-matched controls (47 males, 70 females) were enrolled in the study. Bone mineral density measured by dual-energy X-ray absorptiometry and serum D vitamin levels of each participant were recorded. [Results] The mean lumbar spine, femur neck, and total femur bone mineral density levels, T-scores, and vitamin D levels were found to be significantly lower in Parkinson’s disease patients in both genders. Furthermore, osteoporosis rates were found be significantly higher only in female Parkinson’s disease patients compared with female controls. [Conclusion] Data from the present study revealed that while osteoporosis was significantly higher only in female Parkinson’s disease patients, all Parkinson’s disease patients had lower bone mineral density scores and vitamin D levels compared with the controls regardless of gender, suggesting that clinicians should pay attention to the osteoporosis risk in Parkinson’s disease and that adequate preventive measures should be taken in order to limit the future risk due to osteoporotic fractures. PMID:27630398

  3. Computed tomography analysis of guinea pig bone: architecture, bone thickness and dimensions throughout development.

    Science.gov (United States)

    Witkowska, Agata; Alibhai, Aziza; Hughes, Chloe; Price, Jennifer; Klisch, Karl; Sturrock, Craig J; Rutland, Catrin S

    2014-01-01

    The domestic guinea pig, Cavia aperea f. porcellus, belongs to the Caviidae family of rodents. It is an important species as a pet, a source of food and in medical research. Adult weight is achieved at 8-12 months and life expectancy is ∼5-6 years. Our aim was to map bone local thickness, structure and dimensions across developmental stages in the normal animal. Guinea pigs (n = 23) that had died of natural causes were collected and the bones manually extracted and cleaned. Institutional ethical permission was given under the UK Home Office guidelines and the Veterinary Surgeons Act. X-ray Micro Computed Tomography (microCT) was undertaken on the left and right scapula, humerus and femur from each animal to ascertain bone local thickness. Images were also used to undertake manual and automated bone measurements, volumes and surface areas, identify and describe nutrient, supratrochlear and supracondylar foramina. Statistical analysis between groups was carried out using ANOVA with post-hoc testing. Our data mapped a number of dimensions, and mean and maximum bone thickness of the scapula, humerus and femur in guinea pigs aged 0-1 month, 1-3 months, 3-6 months, 6 months-1 year and 1-4 years. Bone dimensions, growth rates and local bone thicknesses differed between ages and between the scapula, humerus and femur. The microCT and imaging software technology showed very distinct differences between the relative local bone thickness across the structure of the bones. Only one bone showed a singular nutrient foramen, every other bone had between 2 and 5, and every nutrient canal ran in an oblique direction. In contrast to other species, a supratrochlear foramen was observed in every humerus whereas the supracondylar foramen was always absent. Our data showed the bone local thickness, bone structure and measurements of guinea pig bones from birth to 4 years old. Importantly it showed that bone development continued after 1 year, the point at which most guinea pigs have

  4. IgG4-Related Disease of Bilateral Temporal Bones.

    Science.gov (United States)

    Li, Lilun; Ward, Bryan; Cocks, Margaret; Kheradmand, Amir; Francis, Howard W

    2017-03-01

    IgG4-related disease (IgG4-RD) is an idiopathic inflammatory condition that causes pseudotumor formation in single or multiple organs, including those of the head and neck. Temporal bone involvement is rare, with only 3 cases of unilateral temporal bone IgG4-RD described in the literature. We report the first known case of IgG4-RD of bilateral temporal bones and describe its clinical presentation, diagnosis, and treatment. The patient was a 52-year-old man with latent tuberculosis (TB) who presented with a 10-year history of bilateral profound hearing loss and vestibular dysfunction. Computed tomography and magnetic resonance imaging demonstrated bilateral labyrinthine destruction with invasion of the posterior fossa. Immunoglobulin level testing showed elevated total serum IgG levels with normal IgG4 levels. Bilateral mastoidectomies were performed, with biopsy samples demonstrating IgG4 staining with IgG4-positive plasma cells up to 40/HPF (high power field) on the right and 20/HPF on the left, consistent with bilateral IgG4-RD. IgG4-RD of bilateral temporal bones presents with chronic and progressive bilateral hearing loss and vestibular dysfunction. Clinical presentation and radiologic findings are nonspecific, and definitive diagnosis must be made with histopathology and immunostaining. Corticosteroids are therapeutic, but surgical resection may be necessary for temporal bone IgG4-RD to improve long-term remission.

  5. Bone mineral density in adult coeliac disease: An updated review

    Directory of Open Access Journals (Sweden)

    Alfredo J. Lucendo

    2013-03-01

    Full Text Available Introduction and objectives: coeliac disease (CD affects around 1-2 % of the world population. Most patients are now diagnosed when adults, suffering the consequences of an impaired bone mineralization. This review aims to provide an updated discussion on the relationship between low bone mineral density (BMD, osteopenia and osteoporosis, and CD. Methods: a PubMed search restricted to the last 15 years was conducted. Sources cited in the results were also reviewed to identify potential sources of information. Results: low BMD affects up to 75 % of celiac patients, and can be found at any age, independently of positive serological markers and presence of digestive symptoms. The prevalence of CD among osteoporotic patients is also significantly increased. Two theories try to explain this origin of low BMD: Micronutrients malabsorption (including calcium and vitamin D determined by villous atrophy has been related to secondary hyperparathyroidism and incapacity to achieve the potential bone mass peak; chronic inflammation was also related with RANKL secretion, osteoclasts activation and increased bone resorption. As a consequence, celiac patients have a risk for bone fractures that exceed 40 % that of matched non-affected population. Treatment of low BMD in CD comprises gluten-free diet, calcium and vitamin D supplementation, and biphosphonates, although its effects on CD have not been specifically assessed. Conclusions: up to 75 % of celiac patients and 40 % of that diagnosed in adulthood present a low BMD and a variable increase in the risk of bone fractures. Epidemiological changes in CD make bone density scans more relevant for adult coeliacs.

  6. Echinococcal disease of the bone: An unusual cause of a pathological fracture

    Directory of Open Access Journals (Sweden)

    Matthew Goodier

    2010-12-01

    Full Text Available Echinococcosis is caused by the larva of the tapeworm, Echinococcus granulosus or Echinococcus multiloccularis and is endemic in many rural areas of Southern Africa. Echinococcosis of the bone is an unusual manifestation of echinococcal disease and a rare cause of a lytic lesion of bone. This report describes a 30-yr old female who presented with an Echinococcal cyst of the right radius complicated by a pathological fracture.

  7. A fatal case of immune hyperhemolysis with bone marrow necrosis in a patient with sickle cell disease

    Directory of Open Access Journals (Sweden)

    Matthew S. Karafin

    2017-03-01

    Full Text Available In patients with sickle cell disease, hyperhemolysis is a rare but life-threatening complication of transfusion. In this case report, we describe a 61 year-old woman with hemoglobin sickle cell (SC disease and history of alloimmunization who developed hyperhemolysis associated with a transfusion. She was found to have a warm and a clinically-significant cold autoantibody. Severe anemia (Hb 2.7 g/dL with reticulocytopenia and thrombocytopenia prompted a bone marrow biopsy, which demonstrated extensive bone marrow necrosis. Despite treatment, the bone marrow failure did not improve and the patient died on hospital day 38. This case illustrates the potential risks of transfusion in a patient with sickle cell disease, especially one with previous hemolytic reactions. While uncommon, hyperhemolysis can cause death, in this case by extensive bone marrow necrosis. In patients with sickle cell disease, judicious use of red cell transfusions with phenotypically-matched units can diminish, but never completely abrogate, the risks associated with transfusion.

  8. Uremic bone diseases - Clinical laboratorial, scintigraphic and radiological study

    International Nuclear Information System (INIS)

    Silva, W.C.F. da.

    1982-01-01

    This paper evaluated the uremic bone disease in 10 patients on peritoneal dialysis, 10 on hemodialysis and 10 submited to renal transplantation. According to biochemical evaluation we observed hypocalcemia in some patients on dialysis and hipercalcemia in a renal transplanted and in another on peritoneal dialysis. However, there was no significative difference in the serum calcium concentration between the groups and the control group. Hiperphosphatemia occured in 8 patients on peritoneal dialysis and in 9 on hemodialysis and slight hiperphosphatemia occured in 2 renal transplanted patient. The product calcium X phosphorus was elevated in 2 patients on peritoneal dialysis and in 2 on hemodialysis. The magnesium serum concentration were hight in all patients on dialysis and the alkaline phosphatase serum levels were hight in 3 patients dialysis peritoneal and 4 on hemodialysis. A skeleton curvey showed abnormalities in 3 patients on peritoneal dialysis, 5 on hemodialysis and of 5 renal transplanted patients. However there was no significant difference between these results. The bone scanning was abnormal in 6 patients on peritoneal dialysis, 9 patients on hemodialysis and in 8 renal transplanted. The positive results of bone scanning compared with X ray were statistically significative. Bone scanning was the most sensitive method used to detect early abnormalities. (author)

  9. Iron storage in liver, bone marrow and splenic Gaucheroma reflects residual disease in type 1 Gaucher disease patients on treatment.

    Science.gov (United States)

    Regenboog, Martine; Bohte, Anneloes E; Akkerman, Erik M; Stoker, Jaap; Hollak, Carla E M

    2017-11-01

    Gaucher disease (GD) is a lysosomal storage disorder characterized by the storage of glycosphingolipids in macrophages. Despite effective therapy, residual disease is present in varying degrees and may be associated with late complications, such as persistent bone or liver disease and increased cancer risk. Gaucher macrophages are capable of storing iron and locations of residual disease may thus be detectable with iron imaging. Forty type 1 GD (GD1) patients and 40 matched healthy controls were examined using a whole-body magnetic resonance imaging protocol consisting of standard sequences, allowing analysis of iron content per organ, expressed as R2* (Hz). Median R2* values were significantly elevated in GD1 patients as compared to healthy controls in liver [41 Hz (range 29-165) vs. 38 Hz (range 28-53), P Gaucher lesions known as Gaucheroma were found to have increased R2* values. R2* values of liver, spleen and vertebral bone marrow strongly correlated with serum ferritin levels. GD1 patients with persistent hyperferritinaemia demonstrate increased iron levels in liver and bone marrow, which may carry a risk for liver fibrosis and cancer. © 2017 John Wiley & Sons Ltd.

  10. Positive Celiac Disease Serology and Reduced Bone Mineral Density in Adult Women

    Directory of Open Access Journals (Sweden)

    Donald R Duerksen

    2010-01-01

    Full Text Available BACKGROUND: Low bone density and osteoporosis have been demonstrated in celiac disease populations in Europe, South America and the United States. Serological testing with tissue transglutaminase (TTG and immunoglobulin A endomysial (EMA antibodies is highly specific for celiac disease, while antigliadin antibody (AGA testing is less specific.

  11. Imaging The Complications Of Paget's Disease Of Bones In A ...

    African Journals Online (AJOL)

    The skull and chest radiographs, CT scan and echocardiography with their findings are highlighted so as to increase awareness and index of suspicion in physicians to this relatively rare bone disease. Classical features were illustrated on plain skull radiograph and CT Scan. Echocardiography revealed left ventricular ...

  12. Functional bracing for delayed union of a femur fracture associated with Paget's disease of the bone in an Asian patient: a case report

    Directory of Open Access Journals (Sweden)

    Fukuta Masashi

    2010-05-01

    Full Text Available Abstract Paget's disease of the bone is a common metabolic bone disease in most European countries, Australia, New Zealand, and North America. Conversely, this disease is rare in Scandinavia, Asia, and Africa. In Japan, it is extremely rare, with a prevalence of 0.15/100000. Paget's disease is a localized disorder of bone remodeling. Excessive bone resorption and abnormal bone formation result in biomechanically weakened bone and predispose patients to fracture. Delayed union and non-union of fractures have been reported in patients with Paget's disease. Therefore, open reduction and internal fixation of fractures has been recommended to prevent such complications. Here we report an unusual case of a 63-year-old Asian woman with delayed union of a femur fracture secondary to Paget's disease, which was treated successfully by functional bracing.

  13. MR analysis of sternal bone marrow using STIR in hematologic diseases

    Energy Technology Data Exchange (ETDEWEB)

    Kozawa, Eito [Saitama Medical School, Moroyama (Japan)

    1998-12-01

    The magnetic resonance (MR) signal intensity pattern of sternal bone marrow was examined in 21 normal volunteers and 10 patients with aplastic anemia (n=4), multiple myeloma (2), AML (2), gammaglobulinemia (1) and MDS (1) using a sagittal STIR sequence. Double Echo STIR images (TR/TI/TE/NEX=2000/180/20, 100/1) were obtained with a CP body array coil. Craniocaudal phase-encoding with a handmade positioning device effectively avoided overlapping artifacts due to cardiac pulsation. In the normal volunteers, age showed a significant inverse correlation with the calculated SIR (signal intensity ratio of bone marrow relative to subcutaneous fat) using STIR with short TE. The SIR in the sternal body was significantly higher than that in the manubrium (p<0.05). Knowledge of the sternal bone marrow distribution according to age is useful for evaluating hematologic diseases. The proposed method provided high spatial resolution and an excellent bone marrow signal, and may be useful for determining site for aspiration. (author)

  14. Assessment of Bone Health in Patients With Type 1 Gaucher Disease Using Impact Microindentation.

    Science.gov (United States)

    Herrera, Sabina; Pérez-López, Jordi; Moltó-Abad, Marc; Güerri-Fernández, Roberto; Cabezudo, Elena; Novelli, Silvana; Esteve, Jordi; Hernández, Albert; Roig, Inmaculada; Solanich, Xavier; Prieto-Alhambra, Daniel; Nogués, Xavier; Díez-Pérez, Adolfo

    2017-07-01

    Gaucher disease (GD), one of the most common lysosomal disorders (a global population incidence of 1:50,000), is characterized by beta-glucocerebrosidase deficiency. Some studies have demonstrated bone infiltration in up to 80% of patients, even if asymptomatic. Bone disorder remains the main cause of morbidity in these patients, along with osteoporosis, avascular necrosis, and bone infarcts. Enzyme replacement therapy (ERT) has been shown to improve these symptoms. This cross-sectional study included patients with type 1 Gaucher disease (GD1) selected from the Catalan Study Group on GD. Clinical data were collected and a general laboratory workup was performed. Bone mineral density (BMD) was measured at the lumbar spine and hip using dual-energy X-ray absorptiometry (DXA). Patients with bone infarcts or any other focal lesion in the area of indentation visible on imaging were excluded. Bone Material Strength index (BMSi) was measured by bone impact microindentation using an Osteoprobe instrument. Analysis of covariance (ANCOVA) models were fitted to adjust for age, sex, weight, and height. Sixteen patients with GD1 and 29 age- and sex-matched controls were included. GD1 was associated with significantly lower BMSi (adjusted beta -9.30; 95% CI, -15.18 to -3.42; p = 0.004) and reduced lumbar BMD (adjusted beta -0.14; 95% CI, -0.22 to -0.06; p = 0.002) and total hip BMD (adjusted beta -0.09; 95% CI, -0.15 to -0.03; p = 0.006), compared to GD1-free controls. Chitotriosidase levels were negatively correlated with BMSi (linear R 2  = 51.6%, p = 0.004). Bone tissue mechanical characteristics were deteriorated in patients with GD1. BMSi was correlated with chitotriosidase, the marker of GD activity. Bone disorder requires special consideration in this group of patients, and microindentation could be an appropriate tool for assessing and managing their bone health. © 2017 American Society for Bone and Mineral Research. © 2017 American Society for Bone

  15. [Bone remodeling and modeling/mini-modeling.

    Science.gov (United States)

    Hasegawa, Tomoka; Amizuka, Norio

    Modeling, adapting structures to loading by changing bone size and shapes, often takes place in bone of the fetal and developmental stages, while bone remodeling-replacement of old bone into new bone-is predominant in the adult stage. Modeling can be divided into macro-modeling(macroscopic modeling)and mini-modeling(microscopic modeling). In the cellular process of mini-modeling, unlike bone remodeling, bone lining cells, i.e., resting flattened osteoblasts covering bone surfaces will become active form of osteoblasts, and then, deposit new bone onto the old bone without mediating osteoclastic bone resorption. Among the drugs for osteoporotic treatment, eldecalcitol(a vitamin D3 analog)and teriparatide(human PTH[1-34])could show mini-modeling based bone formation. Histologically, mature, active form of osteoblasts are localized on the new bone induced by mini-modeling, however, only a few cell layer of preosteoblasts are formed over the newly-formed bone, and accordingly, few osteoclasts are present in the region of mini-modeling. In this review, histological characteristics of bone remodeling and modeling including mini-modeling will be introduced.

  16. Bone pain

    DEFF Research Database (Denmark)

    Frost, Charlotte Ørsted; Hansen, Rikke Rie; Heegaard, Anne-Marie

    2016-01-01

    Skeletal conditions are common causes of chronic pain and there is an unmet medical need for improved treatment options. Bone pain is currently managed with disease modifying agents and/or analgesics depending on the condition. Disease modifying agents affect the underlying pathophysiology...... of the disease and reduce as a secondary effect bone pain. Antiresorptive and anabolic agents, such as bisphosphonates and intermittent parathyroid hormone (1-34), respectively, have proven effective as pain relieving agents. Cathepsin K inhibitors and anti-sclerostin antibodies hold, due to their disease...... modifying effects, promise of a pain relieving effect. NSAIDs and opioids are widely employed in the treatment of bone pain. However, recent preclinical findings demonstrating a unique neuronal innervation of bone tissue and sprouting of sensory nerve fibers open for new treatment possibilities....

  17. [Bone and joint decade--"mile step" in diagnostics and treatment of movement system diseases?].

    Science.gov (United States)

    Brongel, Leszek; Lorkowski, Jacek; Hładki, Waldemar; Trybus, Marek

    2006-01-01

    Musculoskeletal disorders affect hundreds of millions of people across the world and are the most common causes of severe long-term pain and physical disability. The impact from such disorders on the individual and on society let to propose by WHO for the Decade of the Bone and Joint from 2000 to 2010. The goal of the Decade is to improve the health-related quality of life for people with musculoskeletal disorders throughout the world and this could be achieved by raising awareness of the growing burden of bone and joint diseases on society, promoting prevention and treatment and advancing understanding of musculoskeletal disorders through research. The main fields of interest during the Decade are joint diseases, spinal disorders and low back pain, osteoporosis and severe trauma of the extremities. In our Department we study problems concerning on traumatology of old patients, multitrauma injury, biomechanics in spinal disorders, in degenerative joint disease and foot diseases. Apart from contemporary imaging methods like US or CT we use pedobarographic diagnostics and fotogrammetric examination. In this study we present strategic goals and the summary of our ongoing projects in our Department related to the goals of the Bone and Joint Decade.

  18. Reduced bone mass and preserved marrow adipose tissue in patients with inflammatory bowel diseases in long-term remission.

    Science.gov (United States)

    Bastos, C M; Araújo, I M; Nogueira-Barbosa, M H; Salmon, C E G; de Paula, F J A; Troncon, L E A

    2017-07-01

    Bone marrow adipose tissue has not been studied in patients with inactive inflammatory bowel disease. We found that these patients have preserved marrow adiposity even with low bone mass. Factors involved in bone loss in active disease may have long-lasting effects but do not seem to affect bone marrow adiposity. Reduced bone mass is known to occur at varying prevalence in patients with inflammatory bowel diseases (IBD) because of inflammation, malnutrition, and steroid therapy. Osteoporosis may develop in these patients as the result of an imbalanced relationship between osteoblasts and adipocytes in bone marrow. This study aimed to evaluate for the first time bone mass and bone marrow adipose tissue (BMAT) in a particular subgroup of IBD patients characterized by long-term, steroid-free remission. Patients with Crohn's disease (CD; N = 21) and ulcerative colitis (UC; N = 15) and controls (C; N = 65) underwent dual X-ray energy absorptiometry and nuclear magnetic resonance spectroscopy of the L3 lumbar vertebra for BMAT assessment. Both the CD and UC subgroups showed significantly higher proportions of patients than controls with Z-score ≤-2.0 at L1-L4 (C 1.54%; CD 19.05%; UC 20%; p = 0.02), but not at other sites. The proportions of CD patients with a T-score ˂-1.0 at the femoral neck (C 18.46%; CD 47.62%; p = 0.02) and total hip (C 16.92%; CD 42.86%; p = 0.03) were significantly higher than among controls. There were no statistically significant differences between IBD patients and controls regarding BMAT at L3 (C 28.62 ± 8.15%; CD 29.81 ± 6.90%; UC 27.35 ± 9.80%; p = 0.67). IBD patients in long-term, steroid-free remission may have a low bone mass in spite of preserved BMAT. These findings confirm the heterogeneity of bone disorders in IBD and may indicate that factors involved in bone loss in active disease may have long-lasting effects on these patients.

  19. Evolutionary medicine and bone loss in chronic inflammatory diseases--A theory of inflammation-related osteopenia.

    Science.gov (United States)

    Straub, Rainer H; Cutolo, Maurizio; Pacifici, Roberto

    2015-10-01

    Bone loss is typical in chronic inflammatory diseases such as rheumatoid arthritis, psoriasis, ankylosing spondylitis, systemic lupus erythematosus, multiple sclerosis, inflammatory bowel diseases, pemphigus vulgaris, and others. It is also typical in transplantation-related inflammation and during the process of aging. While we recognized that bone loss is tightly linked to immune system activation or inflamm-aging in the form of acute, chronic active, or chronic smoldering inflammation, bone loss is typically discussed to be an "accident of inflammation." Extensive literature search in PubMed central. Using elements of evolutionary medicine, energy regulation, and neuroendocrine regulation of homeostasis and immune function, we work out that bone waste is an adaptive, evolutionarily positively selected program that is absolutely necessary during acute inflammation. However, when acute inflammation enters a chronic state due to the inability to terminate inflammation (e.g., in autoimmunity or in continuous immunity against microbes), the acute program of bone loss is a misguided adaptive program. The article highlights the complexity of interwoven pathways of osteopenia. Copyright © 2015 The Authors. Published by Elsevier Inc. All rights reserved.

  20. Rosai-Dorfman Disease with Epidural and Spinal Bone Marrow Involvement: Magnetic Resonance Imaging and Diffusion-Weighted Imaging Features

    International Nuclear Information System (INIS)

    Oner, A.Y.; Akpek, S.; Tali, T.

    2007-01-01

    Sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai-Dorfman disease, is a rare histiocytic disorder that typically presents with chronic, self-limiting cervical lymphadenopathy. Although this disease mainly affects histiocytes, there are a few reports of bone marrow infiltration. Diffusion-weighted imaging (DWI) is a promising technology in differentiating between various bone marrow pathologies. We here present conventional magnetic resonance imaging and DWI features of a patient with SHML and bone marrow involvement

  1. Rosai-Dorfman Disease with Epidural and Spinal Bone Marrow Involvement: Magnetic Resonance Imaging and Diffusion-Weighted Imaging Features

    Energy Technology Data Exchange (ETDEWEB)

    Oner, A.Y.; Akpek, S.; Tali, T. [Dept. of Radiology, Gazi Univ. School of Medicine. Besevler-Ankara (Turkey)

    2007-04-15

    Sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai-Dorfman disease, is a rare histiocytic disorder that typically presents with chronic, self-limiting cervical lymphadenopathy. Although this disease mainly affects histiocytes, there are a few reports of bone marrow infiltration. Diffusion-weighted imaging (DWI) is a promising technology in differentiating between various bone marrow pathologies. We here present conventional magnetic resonance imaging and DWI features of a patient with SHML and bone marrow involvement.

  2. The use of bone scintigraphy to detect active Hansen's disease in mutilated patients

    Energy Technology Data Exchange (ETDEWEB)

    Braga, F.J.H.N. [Seccao de Medicina Nuclear do Hospital das Clinicas da Faculdade de Medicina de Ribeirao Preto, USP (Brazil); Departamento de Fisica e Biofisica, Instituto de Biociencias, UNESP, Botucato (Brazil); Foss, N.T.; Ferriolli, E. [Departamento de Clinica Medica da Faculdade de Medicina de Ribeirao Preto, USP (Brazil); Pagnano, C. [Secretaria da Saude, Ribeirao Preto (Brazil); Miranda, J.R.A.; De Moraes, R. [Departamento de Fisica e Biofisica, Instituto de Biociencias, UNESP, Botucato (Brazil)

    1999-11-01

    Mutilation of extremities was very frequent in patients affected by leprosy in the past; although it is now much less common, it is still seen, mainly in patients with long-term disease. In general, mutilation of the nose and ears is caused by the bacillus and mutilation of the hands and feet a consequence of chronic trauma. Leprosy must be chronically treated and any decision to interrupt therapy is based on laboratory tests and biopsy. Scintigraphy is a non-invasive procedure which could be of great value in to determining disease activity. We studied eight patients (five males and three females, aged 64-73 years) who presented with mutilation of the nose (2), ear (1), feet (3) or foot and hand (2). Conventional three-phase bone scintigraphy (750 MBq) and X-ray examinations of the affected areas were performed in all patients. Bone scintigraphy was abnormal in four patients (the presence of bacilli was confirmed by biopsy in two of them), and normal in the other four. In all patients except for the one with ear mutilation, radiography only showed the absence of bone. We conclude that bone scintigraphy is very useful to determine disease activity in cases of mutilation caused by leprosy. It seems to be superior to conventional radiography and may enable bone biopsies to be avoided. (orig.)

  3. Bone aluminum measurements in patients with end-stage renal disease

    International Nuclear Information System (INIS)

    Ellis, K.J.; Kelleher, S.P.

    1986-01-01

    Long-term use of aluminum-based phosphate binders and trace aluminum contamination of dialysate solution have led to increased body burden of this metal in patients with end-stage renal disease. Aluminum accumulates in bone and has been associated with the development of a renal osteodystrophy, called aluminum-induced osteomalacia. At present, bone biopsy is the method of diagnosis of this condition. When examined by quantitative histomorphometry, the aluminum accumulation was reported to correlate with the severity of the osteomalacia. This project was therefore undertaken to investigate the possibility of developing a non-invasive technique using neutron activation analysis for the direct in vivo assessment of bone aluminum levels. A bilateral exposure of the patient's hand is performed at the patient port of the Brookhaven Medical Research Reactor. The induced activity is then counted for 5 min using four 4'' x 4'' x 16'' NaI(T1) detectors arranged in a quasi-4! geometry. In addition to Al, Ca is also detected and serves as each individual's internal standard for the volume of bone mass irradiated. The Al/Ca ratio provides an index of the amount of elevated aluminum per unit bone mass. When this ratio is multiplied by the total body calcium value, an estimate of total skeletal aluminum is obtained. These measurements will be presented for a pilot study of ten asymptomatic renal patients

  4. Role of periodontal pathogenic bacteria in RANKL-mediated bone destruction in periodontal disease

    OpenAIRE

    Mikihito Kajiya; Gabriela Giro; Martin A. Taubman; Xiaozhe Han; Marcia P.A. Mayer; Toshihisa Kawai

    2010-01-01

    Accumulated lines of evidence suggest that hyperimmune responses to periodontal bacteria result in the destruction of periodontal connective tissue and alveolar bone. The etiological roles of periodontal bacteria in the onset and progression of periodontal disease (PD) are well documented. However, the mechanism underlying the engagement of periodontal bacteria in RANKL-mediated alveolar bone resorption remains unclear. Therefore, this review article addresses three critical subjects. First, ...

  5. Reduced Bone Mineral Density in Children With Screening-detected Celiac Disease.

    Science.gov (United States)

    Björck, Sara; Brundin, Charlotte; Karlsson, Magnus; Agardh, Daniel

    2017-11-01

    The aim of the study was to assess whether bone mass and metabolism are impaired in genetically at-risk children with screening-detected celiac disease. Included were 71 children with screening-detected celiac disease diagnosed at 10.0 ± 0.7 (mean ± standard deviation) years and 142 matched controls and 30 children with screening-detected celiac disease diagnosed at 3.3 ± 0.4 years of age presently on a gluten-free diet for 6.9 ± 1.1 years and 60 matched controls. All participants were assessed for bone mineral density (BMD) of total body and spine by dual x-ray absorptiometry, serum 25(OH) vitamin D3, parathyroid hormone (PTH), interleukin (IL)-1β, IL-2, IL-4, IL-5, IL-6, IL-8, IL-10, IL-12p70, IL-13, IL-15, interferon gamma, and tumor necrosis factor alpha. At diagnosis, screening-detected celiac disease children as compared to controls had a mean -0.03 g/cm reduced BMD of both total body and spine (P = 0.009 and P = 0.005, respectively), a mean -11.4 nmol/L lower level of 25(OH) vitamin D3 (P celiac disease as compared to controls (P celiac disease have reduced BMD, lower levels of vitamin D3, higher levels of PTH, and signs of systemic inflammation compared with controls. These differences were not found in celiac disease children on a gluten-free diet, indicating that children with screening-detected celiac disease benefit from an early diagnosis and treatment.

  6. Negative correlation between bone mineral density and TSH receptor antibodies in long-term euthyroid postmenopausal women with treated Graves’ disease

    DEFF Research Database (Denmark)

    Ercolano, Monica A; Drnovsek, Monica L; Croome, Maria C

    2013-01-01

    Thyrotoxicosis is a cause of secondary osteoporosis. High concentrations of triiodotironine (T3) in Graves' disease stimulate bone turnover, but it is unclear if euthyroidism will always normalize bone metabolism. Thyrotropin (TSH) is known to affect directly the bone metabolism through the TSH...... receptor and TSH receptor antibodies (TRAb) may have an important role in bone turn-over.The aim of our study was to determine, in pre and postmenopausal euthyroidism patients with previous overt hyperthyroidism due to Graves' disease the bone mineral density (BMD) as well as factors that could affect BMD...

  7. Unbiased Stereologic Estimation of the Spatial Distribution of Paget’s Disease in the Human Temporal Bone

    DEFF Research Database (Denmark)

    Bloch, Sune Land; Sørensen, Mads Sølvsten

    2014-01-01

    remodeling around the inner ear space and to compare it with that of otosclerosis in a contemporary context of temporal bone dynamics. MATERIALS AND METHODS: From the temporal bone collection of Massachusetts Eye and Ear Infirmary, 15 of 29 temporal bones with Paget's disease were selected to obtain...... an independent sample. All volume distributions were obtained along the normal axis of capsular bone remodeling activity by the use of vector-based stereology. RESULTS: Pagetic bone remodeling was distributed centrifugally around the inner ear space at the individual and the general level. This pattern...

  8. Effect of chronic hepatitis C virus infection on bone disease in postmenopausal women.

    LENUS (Irish Health Repository)

    Nanda, Kavinderjit S

    2012-02-01

    BACKGROUND & AIMS: Limited data are available on the contribution of chronic HCV infection to the development of bone disease in postmenopausal women. We studied whether women who acquired HCV infection through administration of HCV genotype 1b-contaminated anti-D immunoglobulin from a single source had decreased bone mineral density (BMD) or altered levels of bone turnover markers (BTMs), compared with women who spontaneously resolved infection or age-matched healthy controls. METHODS: From a cohort of postmenopausal Irish women, we compared BMD, determined by dual-energy x-ray absorptiometry, and a panel of BTMs in 20 women chronically infected with HCV (PCR(+)), 21 women who had spontaneously resolved infection (PCR(-)), and 23 age-matched healthy controls. RESULTS: Levels of BTMs and BMD were similar in PCR(+) and PCR(-) women and healthy age-matched controls. However, there was an increased frequency of fractures in PCR(+) (n = 6) compared with PCR(-) women (n = 0, P = .007). PCR(+) women with fractures were postmenopausal for a longer time (median, 15.5, range, 5-20 years vs 4.5, range, 1-20 years in PCR(+) women without fractures; P = .033), had lower BMD at the hip (0.79, range, 0.77-0.9 g\\/cm(2) vs 0.96, range, 0.81-1.10 g\\/cm(2); P = .007), and had a lower body mass index (23.7, range 21.2-28.5 kg\\/m(2) vs 25.6, range 22.1-36.6 kg\\/m(2); P = .035). There was no difference in liver disease severity or BTMs in PCR(+) women with or without fractures. CONCLUSIONS: Chronic HCV infection did not lead to discernable metabolic bone disease in postmenopausal women, but it might be a risk factor for bone fractures, so preventive measures should be introduced. To view this article\\'s video abstract, go to the AGA\\'s YouTube Channel.

  9. Impact of primary metastatic bone disease in germ cell tumors

    DEFF Research Database (Denmark)

    Oing, C; Oechsle, K; Necchi, A

    2017-01-01

    (multivariate Cox regression; HR, 0.32; P=0.011) with respective 2-year PFS and OS rates of 68% and 75% compared with 24% and 36% for non-seminoma patients. Conclusions: Outcome of GCT patients with primary metastatic bone disease is particularly poor in non-seminoma patients, even worse than the expected...

  10. Bone scan in rheumatology

    International Nuclear Information System (INIS)

    Morales G, R.; Cano P, R.; Mendoza P, R.

    1993-01-01

    In this chapter a revision is made concerning different uses of bone scan in rheumatic diseases. These include reflex sympathetic dystrophy, osteomyelitis, spondyloarthropaties, metabolic bone diseases, avascular bone necrosis and bone injuries due to sports. There is as well some comments concerning pediatric pathology and orthopedics. (authors). 19 refs., 9 figs

  11. Detection of degenerative disease of the temporomandibular joint by bone scintigraphy: concise communication

    International Nuclear Information System (INIS)

    Goldstein, H.A.; Bloom, C.Y.

    1980-01-01

    Nine patients with facial pain were evaluated with limited bone scans. The scintigrams correlated with microscopy in all patients, although radiographs correlated with microscopy in only five patients. The degenerative disease process in the temporomandibular joint was more extensive in the patients with radiographic and scintigraphic abnormalities than in those with scintigraphic abnormalities alone. The limited bone scan appears useful in detecting early degenerative changes in the temporomandibular joint

  12. Bone Marrow-Derived Cells as a Therapeutic Approach to Optic Nerve Diseases

    Directory of Open Access Journals (Sweden)

    Louise A. Mesentier-Louro

    2016-01-01

    Full Text Available Following optic nerve injury associated with acute or progressive diseases, retinal ganglion cells (RGCs of adult mammals degenerate and undergo apoptosis. These diseases have limited therapeutic options, due to the low inherent capacity of RGCs to regenerate and due to the inhibitory milieu of the central nervous system. Among the numerous treatment approaches investigated to stimulate neuronal survival and axonal extension, cell transplantation emerges as a promising option. This review focuses on cell therapies with bone marrow mononuclear cells and bone marrow-derived mesenchymal stem cells, which have shown positive therapeutic effects in animal models of optic neuropathies. Different aspects of available preclinical studies are analyzed, including cell distribution, potential doses, routes of administration, and mechanisms of action. Finally, published and ongoing clinical trials are summarized.

  13. Developmental psychoneuroendocrine and psychoneuroimmune pathways from childhood adversity to disease.

    Science.gov (United States)

    Kuhlman, Kate Ryan; Chiang, Jessica J; Horn, Sarah; Bower, Julienne E

    2017-09-01

    Childhood adversity has been repeatedly and robustly linked to physical and mental illness across the lifespan. Yet, the biological pathways through which this occurs remain unclear. Functioning of the inflammatory arm of the immune system and the hypothalamic-pituitary-adrenal (HPA)-axis are both hypothesized pathways through which childhood adversity leads to disease. This review provides a novel developmental framework for examining the role of adversity type and timing in inflammatory and HPA-axis functioning. In particular, we identify elements of childhood adversity that are salient to the developing organism: physical threat, disrupted caregiving, and unpredictable environmental conditions. We propose that existing, well-characterized animal models may be useful in differentiating the effects of these adversity elements and review both the animal and human literature that supports these ideas. To support these hypotheses, we also provide a detailed description of the development and structure of both the HPA-axis and the inflammatory arm of the immune system, as well as recent methodological advances in their measurement. Recommendations for future basic, developmental, translational, and clinical research are discussed. Copyright © 2017 Elsevier Ltd. All rights reserved.

  14. Machine learning based analytics of micro-MRI trabecular bone microarchitecture and texture in type 1 Gaucher disease.

    Science.gov (United States)

    Sharma, Gulshan B; Robertson, Douglas D; Laney, Dawn A; Gambello, Michael J; Terk, Michael

    2016-06-14

    Type 1 Gaucher disease (GD) is an autosomal recessive lysosomal storage disease, affecting bone metabolism, structure and strength. Current bone assessment methods are not ideal. Semi-quantitative MRI scoring is unreliable, not standardized, and only evaluates bone marrow. DXA BMD is also used but is a limited predictor of bone fragility/fracture risk. Our purpose was to measure trabecular bone microarchitecture, as a biomarker of bone disease severity, in type 1 GD individuals with different GD genotypes and to apply machine learning based analytics to discriminate between GD patients and healthy individuals. Micro-MR imaging of the distal radius was performed on 20 type 1 GD patients and 10 healthy controls (HC). Fifteen stereological and textural measures (STM) were calculated from the MR images. General linear models demonstrated significant differences between GD and HC, and GD genotypes. Stereological measures, main contributors to the first two principal components (PCs), explained ~50% of data variation and were significantly different between males and females. Subsequent PCs textural measures were significantly different between GD patients and HC individuals. Textural measures also significantly differed between GD genotypes, and distinguished between GD patients with normal and pathologic DXA scores. PCA and SVM predictive analyses discriminated between GD and HC with maximum accuracy of 73% and area under ROC curve of 0.79. Trabecular STM differences can be quantified between GD patients and HC, and GD sub-types using micro-MRI and machine learning based analytics. Work is underway to expand this approach to evaluate GD disease burden and treatment efficacy. Copyright © 2016 Elsevier Ltd. All rights reserved.

  15. Determinación de la edad ósea a través del desarrollo dental en pacientes de Ortodoncia Assessment of bone age by dental developmental seen in Orthodontia patients

    Directory of Open Access Journals (Sweden)

    Gladia Toledo Mayarí

    2009-09-01

    Full Text Available La tendencia actual en Ortodoncia consiste en reducir el número de radiaciones con fines diagnósticos a las estrictamente necesarias, por lo que se han desarrollado índices de maduración ósea a través del desarrollo dental, dentro de los que se encuentra el método de Demirjian y colaboradores, sustituyéndose la radiografía de la mano que constituye una radiografía adicional para los pacientes, además de que la misma no se realiza en los servicios de Estomatología. Con el objetivo de determinar la edad ósea a través del desarrollo dental, se realizó un estudio descriptivo y transversal, para lo cual fueron realizadas una radiografía de la mano izquierda y una radiografía panóramica o periapicales de la hemiarcada mandibular izquierda, a 150 pacientes de Ortodoncia y se determinó la edad ósea, a través de los métodos Tanner-Whitehouse 2 (TW2 y Demirjian y colaboradores. Se encontraron correlaciones altas muy significativas entre las edades óseas calculadas en ambos sexos (hembras r= 0,828 y varones r= 0,957. Concluyéndose que la edad ósea calculada a través del desarrollo dental, en nuestra muestra, puede ser aplicada para predecir la edad ósea a través del método TW2, siendo posible sustituir la radiografía de la mano izquierda.Present trend in Orthodontics is to reduce the number of strictly needed diagnostic radiations that is why bone maturation indexes have been created by dental developmental including the Demirjian et al method, substituting the hand radiography which is an additional strategy for patients due to it is not performed in Stomatology Services. To determine the bone age by dental developmental, a cross-sectional and transversal study was carried out and also a left hand radiography and a panoramic and a periapical one of left mandibular hemicardia in 150 orthodontic patients determining the bone age by Tanner-Whitehouse 2 method (TW2 and Demirjian et al. There were very significant high

  16. Compromised cortical bone compartment in type 2 diabetes mellitus patients with microvascular disease

    DEFF Research Database (Denmark)

    Shanbhogue, Vikram Vinod; Hansen, Stinus; Nielsen, Morten Frost Munk

    2016-01-01

    OBJECTIVE AND DESIGN: Patients with type 2 diabetes mellitus (T2D) have an increased fracture risk despite a normal or elevated bone mineral density (BMD). The aim of this cross-sectional in vivo study was to assess parameters of peripheral bone microarchitecture, estimated bone strength and bone...... remodeling in T2D patients with and without diabetic microvascular disease (MVD+ and MVD- respectively) and to compare them with healthy controls. METHODS: Fifty-one T2D patients (MVD+ group: n=25) were recruited from Funen Diabetic Database and matched for age, sex and height with 51 healthy subjects. High...... deficits are not a characteristic of all T2D patients but of a subgroup characterized by the presence of microvascular complications. Whether this influences fracture rates in these patients needs further investigation....

  17. Carryover effect of postpartum inflammatory diseases on developmental biology and fertility in lactating dairy cows.

    Science.gov (United States)

    Ribeiro, E S; Gomes, G; Greco, L F; Cerri, R L A; Vieira-Neto, A; Monteiro, P L J; Lima, F S; Bisinotto, R S; Thatcher, W W; Santos, J E P

    2016-03-01

    The objective of this series of studies was to investigate the effects of inflammatory diseases occurring before breeding on the developmental biology and reproductive responses in dairy cows. Data from 5 studies were used to investigate different questions associating health status before breeding and reproductive responses. Health information for all studies was composed of the incidence of retained fetal membranes, metritis, mastitis, lameness, and respiratory and digestive problems from parturition until the day of breeding. Retained placenta and metritis were grouped as uterine disease (UTD). Mastitis, lameness, digestive and respiratory problems were grouped as nonuterine diseases (NUTD). Study 1 evaluated the effect of disease before artificial insemination (AI), anovulation before synchronization of the estrous cycle, and low body condition score at AI on pregnancy per AI, as well as their potential interactions or additive effects. Study 2 investigated the effect of site of inflammation (UTD vs. NUTD) and time of occurrence relative to preantral or antral stages of ovulatory follicle development, and the effect of UTD and NUTD on fertility responses of cows bred by AI or by embryo transfer. Study 3 evaluated the effect of disease on fertilization and embryonic development to the morula stage. Study 4 evaluated the effect of disease on preimplantation conceptus development as well as secretion of IFN-τ and transcriptome. Study 5 investigated the effect of diseases before AI on the transcript expression of interferon-stimulated genes in peripheral blood leukocytes during peri-implantation stages of conceptus development after first AI postpartum. Altogether, these studies demonstrated that inflammatory disease before breeding reduced fertilization of oocytes and development to morula, and impaired early conceptus development to elongation stages and secretion of IFN-τ in the uterine lumen. Diseases caused inflammation-like changes in transcriptome of

  18. Computational segmentation of collagen fibers in bone matrix indicates bone quality in ovariectomized rat spine.

    Science.gov (United States)

    Daghma, Diaa Eldin S; Malhan, Deeksha; Simon, Paul; Stötzel, Sabine; Kern, Stefanie; Hassan, Fathi; Lips, Katrin Susanne; Heiss, Christian; El Khassawna, Thaqif

    2018-05-01

    Bone loss varies according to disease and age and these variations affect bone cells and extracellular matrix. Osteoporosis rat models are widely investigated to assess mechanical and structural properties of bone; however, bone matrix proteins and their discrepant regulation of diseased and aged bone are often overlooked. The current study considered the spine matrix properties of ovariectomized rats (OVX) against control rats (Sham) at 16 months of age. Diseased bone showed less compact structure with inhomogeneous distribution of type 1 collagen (Col1) and changes in osteocyte morphology. Intriguingly, demineralization patches were noticed in the vicinity of blood vessels in the OVX spine. The organic matrix structure was investigated using computational segmentation of collagen fibril properties. In contrast to the aged bone, diseased bone showed longer fibrils and smaller orientation angles. The study shows the potential of quantifying transmission electron microscopy images to predict the mechanical properties of bone tissue.

  19. New treatment of periodontal diseases by using NF-kappaB decoy oligodeoxynucleotides via prevention of bone resorption and promotion of wound healing.

    Science.gov (United States)

    Shimizu, Hideo; Nakagami, Hironori; Morita, Shosuke; Tsukamoto, Ikuyo; Osako, Mariana Kiomy; Nakagami, Futoshi; Shimosato, Takashi; Minobe, Noriko; Morishita, Ryuichi

    2009-09-01

    Nuclear factor-kappa B (NF-kappaB) is involved in osteoclast differentiation and activation. Thus, the blockade of the NF-kappaB pathway might be a novel therapeutic strategy for treating bone metabolic diseases. Periodontitis is subgingival inflammation caused by bacterial infection; this disease also is thought to be a chronic focal point responsible for systemic diseases. In this study, NF-kappaB decoy oligodeoxynucleotides (ODNs) were topically applied for experimental periodontitis in a debris-accumulation model and wound healing in a bone-defect model of beagle dogs to investigate the effect of decoy ODN on bone metabolism. Application of NF-kappaB decoy ODN significantly reduced interleukin-6 activity in crevicular fluid and improved alveolar bone loss in the analysis of dental radiographs and DEXA. Direct measurement of exposed root that lost alveolar bone support revealed that NF-kappaB decoy treatment dramatically protected bone from loss. In a bone-defect model, NF-kappaB decoy ODN promoted the healing process as compared with control scrambled decoy in micro-CT analysis. Overall, inhibition of NF-kappaB by decoy strategy prevented the progression of bone loss in periodontitis and promoted the wound healing in bone defects through the inhibition of osteoclastic bone resorption. Targeting of NF-kappaB might be a potential therapy in various bone metabolic diseases.

  20. Gene expression analysis in human osteoblasts exposed to dexamethasone identifies altered developmental pathways as putative drivers of osteoporosis

    Directory of Open Access Journals (Sweden)

    Sadlier Denise M

    2007-02-01

    Full Text Available Abstract Background Osteoporosis, a disease of decreased bone mineral density represents a significant and growing burden in the western world. Aging population structure and therapeutic use of glucocorticoids have contributed in no small way to the increase in the incidence of this disease. Despite substantial investigative efforts over the last number of years the exact molecular mechanism underpinning the initiation and progression of osteoporosis remain to be elucidated. This has meant that no significant advances in therapeutic strategies have emerged, with joint replacement surgery being the mainstay of treatment. Methods In this study we have used an integrated genomics profiling and computational biology based strategy to identify the key osteoblast genes and gene clusters whose expression is altered in response to dexamethasone exposure. Primary human osteoblasts were exposed to dexamethasone in vitro and microarray based transcriptome profiling completed. Results These studies identified approximately 500 osteoblast genes whose expression was altered. Functional characterization of the transcriptome identified developmental networks as being reactivated with 106 development associated genes found to be differentially regulated. Pathway reconstruction revealed coordinate alteration of members of the WNT signaling pathway, including frizzled-2, frizzled-7, DKK1 and WNT5B, whose differential expression in this setting was confirmed by real time PCR. Conclusion The WNT pathway is a key regulator of skeletogenesis as well as differentiation of bone cells. Reactivation of this pathway may lead to altered osteoblast activity resulting in decreased bone mineral density, the pathological hallmark of osteoporosis. The data herein lend weight to the hypothesis that alterations in developmental pathways drive the initiation and progression of osteoporosis.

  1. Developmental programming of cardiovascular disease by prenatal hypoxia.

    Science.gov (United States)

    Giussani, D A; Davidge, S T

    2013-10-01

    It is now recognized that the quality of the fetal environment during early development is important in programming cardiovascular health and disease in later life. Fetal hypoxia is one of the most common consequences of complicated pregnancies worldwide. However, in contrast to the extensive research effort on pregnancy affected by maternal nutrition or maternal stress, the contribution of pregnancy affected by fetal chronic hypoxia to developmental programming is only recently becoming delineated and established. This review discusses the increasing body of evidence supporting the programming of cardiac susceptibility to ischaemia and reperfusion (I/R) injury, of endothelial dysfunction in peripheral resistance circulations, and of indices of the metabolic syndrome in adult offspring of hypoxic pregnancy. An additional focus of the review is the identification of plausible mechanisms and the implementation of maternal and early life interventions to protect against adverse programming.

  2. Distinct characteristics of mandibular bone collagen relative to long bone collagen: relevance to clinical dentistry.

    Science.gov (United States)

    Matsuura, Takashi; Tokutomi, Kentaro; Sasaki, Michiko; Katafuchi, Michitsuna; Mizumachi, Emiri; Sato, Hironobu

    2014-01-01

    Bone undergoes constant remodeling throughout life. The cellular and biochemical mechanisms of bone remodeling vary in a region-specific manner. There are a number of notable differences between the mandible and long bones, including developmental origin, osteogenic potential of mesenchymal stem cells, and the rate of bone turnover. Collagen, the most abundant matrix protein in bone, is responsible for determining the relative strength of particular bones. Posttranslational modifications of collagen, such as intermolecular crosslinking and lysine hydroxylation, are the most essential determinants of bone strength, although the amount of collagen is also important. In comparison to long bones, the mandible has greater collagen content, a lower amount of mature crosslinks, and a lower extent of lysine hydroxylation. The great abundance of immature crosslinks in mandibular collagen suggests that there is a lower rate of cross-link maturation. This means that mandibular collagen is relatively immature and thus more readily undergoes degradation and turnover. The greater rate of remodeling in mandibular collagen likely renders more flexibility to the bone and leaves it more suited to constant exercise. As reviewed here, it is important in clinical dentistry to understand the distinctive features of the bones of the jaw.

  3. Distinct Characteristics of Mandibular Bone Collagen Relative to Long Bone Collagen: Relevance to Clinical Dentistry

    Directory of Open Access Journals (Sweden)

    Takashi Matsuura

    2014-01-01

    Full Text Available Bone undergoes constant remodeling throughout life. The cellular and biochemical mechanisms of bone remodeling vary in a region-specific manner. There are a number of notable differences between the mandible and long bones, including developmental origin, osteogenic potential of mesenchymal stem cells, and the rate of bone turnover. Collagen, the most abundant matrix protein in bone, is responsible for determining the relative strength of particular bones. Posttranslational modifications of collagen, such as intermolecular crosslinking and lysine hydroxylation, are the most essential determinants of bone strength, although the amount of collagen is also important. In comparison to long bones, the mandible has greater collagen content, a lower amount of mature crosslinks, and a lower extent of lysine hydroxylation. The great abundance of immature crosslinks in mandibular collagen suggests that there is a lower rate of cross-link maturation. This means that mandibular collagen is relatively immature and thus more readily undergoes degradation and turnover. The greater rate of remodeling in mandibular collagen likely renders more flexibility to the bone and leaves it more suited to constant exercise. As reviewed here, it is important in clinical dentistry to understand the distinctive features of the bones of the jaw.

  4. Influences of transplantation on metabolic bone diseases in dialysis patients. Measurement of bone density with multiple X-ray photodensitometry

    International Nuclear Information System (INIS)

    Hida, Miho

    1994-01-01

    Renal osteodystrophy is a grave complication in dialysis patients. In this study, we evaluated the effect of renal transplantation (Txp) on metabolic bone diseases in renal transplant recipients (RTR), by multiple scanning X-ray photodensitometry (MD/MS). In only about 10% of RTR, bone metabolism recovered following improvement of renal function 1-2 years after Txp. Most cases showed decreased ΣGS and μ' scores on the MD/MS 1-2 years after Txp. Then ΣGS and μ' gradually increased over a long period. In seven of ten RTR with long-term graft survival (10 years<), ΣGS and μ' scores were within normal limits and densitometry bone patterns were normal. In four of five cases that received ciclosporin and had undergone Txp more than five years before, densitometry bone patterns were normal. Treatment with high doses of steroids due to acute rejection caused a sharp decline of ΣGS and μ' scores. In FK506-medicated RTR, ΣGS and μ' scores 1-2 years after Txp were decreased. In a 21-year-old female patient who had undergone Txp as the age of 13-year-old, there was little bone growth and ΣGS and μ' scores were significantly decreased. (author)

  5. The negative correlation between thyrotropin receptor-stimulating antibodies and bone mineral density in postmenopausal patients with Graves' disease.

    Science.gov (United States)

    Amashukeli, Medea; Korinteli, Maka; Zerekidze, Tamar; Jikurauli, Nino; Shanava, Shorena; Tsagareli, Marina; Giorgadze, Elen

    2013-06-01

    Graves' disease is an autoimmune disorder with various clinical manifestations. Thyrotropin receptor antibodies (TRAbs), the circulating autoantibodies specific to Graves' disease, are the cause for hyperthyroidism, the most prevalent abnormality. Hyperthyroidism leads to increased bone turnover and a negative bone balance. The aims of the present study were to determine the relationship between TRAbs and bone mineral density (BMD), to assess the extent of BMD change in patients with Graves' disease, and to determine the impact of conservative and surgical therapy on BMD. Fifty female postmenopausal patients with Graves' disease were chosen for this study. Twenty women had a recent diagnosis of Graves' disease, 30 women presented with a compensated disease state after either conservative or surgical treatment, and 30 healthy postmenopausal women served as controls. Thyroid parameters were measured, and BMD values were obtained by dual energy x-ray absorptiometry scan.Femoral neck and lumbar spine BMD and T-scores were significantly lower in newly diagnosed patients compared with the control group, but a difference was not observed between the treated and control groups. Statistical analysis revealed a strong and significant negative correlation between femoral neck and lumbar spine BMD and TRAb values.Both surgical and conservative therapies are effective for restoring BMD in postmenopausal patients with Graves' disease, and the increased level of TRAb can be a useful marker of bone density impairment.

  6. Assessment of bone mineral status in children with Marfan syndrome

    Science.gov (United States)

    Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder with skeletal involvement. It is caused by mutations in fibrillin1 (FBN1) gene resulting in activation of TGF-ßeta, which developmentally regulates bone mass and matrix properties. There is no consensus regarding bone minerali...

  7. Developmental programming: the role of growth hormone.

    Science.gov (United States)

    Oberbauer, Anita M

    2015-01-01

    Developmental programming of the fetus has consequences for physiologic responses in the offspring as an adult and, more recently, is implicated in the expression of altered phenotypes of future generations. Some phenotypes, such as fertility, bone strength, and adiposity are highly relevant to food animal production and in utero factors that impinge on those traits are vital to understand. A key systemic regulatory hormone is growth hormone (GH), which has a developmental role in virtually all tissues and organs. This review catalogs the impact of GH on tissue programming and how perturbations early in development influence GH function.

  8. Modeling Human Bone Marrow Failure Syndromes Using Pluripotent Stem Cells and Genome Engineering.

    Science.gov (United States)

    Jung, Moonjung; Dunbar, Cynthia E; Winkler, Thomas

    2015-12-01

    The combination of epigenetic reprogramming with advanced genome editing technologies opened a new avenue to study disease mechanisms, particularly of disorders with depleted target tissue. Bone marrow failure syndromes (BMFS) typically present with a marked reduction of peripheral blood cells due to a destroyed or dysfunctional bone marrow compartment. Somatic and germline mutations have been etiologically linked to many cases of BMFS. However, without the ability to study primary patient material, the exact pathogenesis for many entities remained fragmentary. Capturing the pathological genotype in induced pluripotent stem cells (iPSCs) allows studying potential developmental defects leading to a particular phenotype. The lack of hematopoietic stem and progenitor cells in these patients can also be overcome by differentiating patient-derived iPSCs into hematopoietic lineages. With fast growing genome editing techniques, such as CRISPR/Cas9, correction of disease-causing mutations in iPSCs or introduction of mutations in cells from healthy individuals enable comparative studies that may identify other genetic or epigenetic events contributing to a specific disease phenotype. In this review, we present recent progresses in disease modeling of inherited and acquired BMFS using reprogramming and genome editing techniques. We also discuss the challenges and potential shortcomings of iPSC-based models for hematological diseases.

  9. Differentiation of malignant and degenerative benign bone disease using 99mTc-citrate scintigraphy

    International Nuclear Information System (INIS)

    Guo Rui; Jin Jianhua; Li Sijin; Li Xianfeng; Zhang Xiaojuan; Ren Yuan

    2008-01-01

    Objective: To differentiate malignant and degenerative benign bone disease using 99m Tc- citrate scintigraphy. Methods: Thirty-nine patients (92 lesions) with confirmed malignant bone disease or degenerative benign bone disease were studied, for which the results of 99m Te-methylene diphosphonate( 99m Tc- MDP) scintigraphy were positive. 99m Tc-citrate scintigraphy was performed within a time interval of 2-7 days after 99m Tc-MDP scintigraphy. Visual analysis and semiquantitative analysis were applied. Each lesion was scored as malignant or benign, which was independently verified, using conventional techniques (histopathology, X-ray, CT, MRI and clinical follow up). Results: In visual analysis of 99m Tc-citrate imaging, most malignant lesions (35/48, 72.92%) clearly showed high radioactivity accumulation, while most benign lesions (39/44, 88.64%) had not obviously visible uptake of 99m Tc-citrate. In semiquantitative analysis of 99m Tc- citrate image, malignant lesions demonstrated a higher lesion-to-background radioisotope uptake ratio (RUR) than that of benign degenerative lesions (1.47 ± 0.42 vs. 1.09 ± 0.38, t=2.887, P 99m Tc-MDP in the two groups is of the same (1.96 ± 0.25 vs. 1.87 ± 0.21, t=1.178, P>0.20). Conclusion: 99m Tc- citrate scintigraphy is a promising method to differentiate malignant from benign degenerative lesions seen as areas of increased activity on 99m Tc-MDP bone scintigraphy. (authors)

  10. Duodenal histopathology and laboratory deficiencies related to bone metabolism in coeliac disease.

    Science.gov (United States)

    Posthumus, Lotte; Al-Toma, Abdul

    2017-08-01

    Coeliac disease (CD) is a chronic immune-mediated small intestine enteropathy precipitated by gluten in genetically predisposed individuals. Adult presentation is often atypical and malabsorption of vitamins and minerals is common, with a consequent disturbance of bone metabolism. We aim to evaluate laboratory deficiencies related to bone metabolism and the relationship between severity of histological damage and degree of bone mass loss at diagnosis of CD. A retrospective cross-sectional study of 176 adult coeliac patients was carried out. All patients fulfilled the histopathological criteria for CD. Biochemical data were analysed (calcium/phosphate/alkaline-phosphatase/vitamin D/parathormone). Duodenal histology was classified according to the Marsh classification. Bone mass density (BMD) at the lumbar and femoral regions measured by dual X-ray absorptiometry. A P-value of less than 0.05 was considered significant. No correlation was found between the presence of gastrointestinal symptoms and the Marsh histopathological stage (P>0.05). Vitamin D deficiency was most common (44.5%), whereas only 5.7% had hypocalcaemia. Calcium was lower (Pcoeliac patients older than 30 years, evaluation of bone biomarkers and dual X-ray absorptiometry examination should be considered.

  11. Understanding the biology of bone sarcoma from early initiating events through late events in metastasis and disease progression.

    Directory of Open Access Journals (Sweden)

    Limin eZhu

    2013-09-01

    Full Text Available The two most common primary bone malignancies, osteosarcoma and Ewing sarcoma, are both aggressive, highly metastatic cancers that most often strike teens, though both can be found in younger children and adults. Despite distinct origins and pathogenesis, both diseases share several mechanisms of progression and metastasis, including neovascularization, invasion, anoikis resistance, chemoresistance and evasion of the immune response. Some of these processes are well-studies in more common carcinoma models, and the observation from adult diseases may be readily applied to pediatric bone sarcomas. Neovascularization, which includes angiogenesis and vasculogenesis, is a clear example of a process that is likely to be similar between carcinomas and sarcomas, since the responding cells are the same in each case. Chemoresistance mechanisms also may be similar between other cancers and the bone sarcomas. Since osteosarcoma and Ewing sarcoma are mesenchymal in origin, the process of epithelial-to-mesenchymal transformation is largely absent in bone sarcomas, necessitating different approaches to study progression and metastasis in these diseases. One process that is less well-studied in bone sarcomas is dormancy, which allows micrometastatic disease to remain viable but not growing in distant sites – typically the lungs – for months or years before renewing growth to become overt metastatic disease. By understanding the basic biology of these processes, novel therapeutic strategies may be developed that could improve survival in children with osteosarcoma or Ewing sarcoma.

  12. Bone marrow in pediatric patients with Hodgkin's disease.

    Science.gov (United States)

    Khan, Fauzia Shafi; Hasan, Rabiya Fayyaz

    2012-01-01

    Hodgkin's disease is a malignant process of lymphoreticular system that constitutes 6% of childhood cancers Accurate staging of lymphoma is the basis for rational therapeutic planning and assessment of the presence or absence of marrow involvement is a basic part of the staging evaluation. The objective of this study was to determine the incidence of marrow infiltration in paediatric patients with Hodgkin's disease and to ascertain its morphological spectrum in the marrow. The study included 85 paediatric patients with diagnosed Hodgkin's disease seen at The Children's Hospital/Institute of Child Health, Lahore, from January 2010 to December 2011, referred to haematology department for bone marrow biopsies. Ages ranged between two years to fourteen years with an average age of seven years, the male female ratio being 13:1. Mixed cellularity was the commonest histological type present in 66 (78%) cases. The presenting feature common in all cases was superficial lymphadenopathy followed by hepatomegaly in 17 (20%) cases and splenomegaly in 16 (19%). All the marrow aspirates were negative for infiltration. Trephine biopsies revealed marrow infiltration in 9 (10.5%). Five (56%) cases had bilateral while 4 (44%) had unilateral involvement. Pattern of infiltration was diffuse in 8 (89%) and focal in one (11%) trephines. Increased marrow fibrosis was present in eight (89%) cases. Diagnostic Reed Sternberg cells were identified in only one case and the mononuclear variants were present in six cases and atypical cells were present in two cases in these immunohistochemistry for CD15 and CD30 was performed which was positive. Granulomas in one and lymphoid aggregates were present in two trephine biopsies otherwise negative for Hodgkin's infiltration. Bone marrow infiltration was present in 10.5% cases, immunohistochemistry was used to confirm infiltration in two cases, the pattern of infiltration being diffuse in majority (89%).

  13. Upper airway obstruction and pulmonary abnormalities due to lymphoproliferative disease following bone marrow transplantation in children

    Energy Technology Data Exchange (ETDEWEB)

    Fletcher, B.D. [Department of Diagnostic Imaging, St. Jude Children`s Research Hospital, 332 N. Lauderdale St., Memphis, TN 38105 (United States)]|[Departments of Radiology and Pediatrics, University of Tennessee, Memphis, Tennessee (United States); Heslop, H.E. [Department of Hematology/Oncology, St. Jude Children`s Research Hospital, Department of Pediatrics, University of Tennessee, Memphis, Tennessee (United States); Kaste, S.C. [Department of Diagnostic Imaging, St. Jude Children`s Research Hospital, Department of Radiology, University of Tennessee, Memphis, Tennessee (United States); Bodner, S. [Department of Pathology, St. Jude Children`s Research Hospital, Department of Pathology, University of Tennessee, Memphis, Tennessee (United States)

    1998-07-01

    We report three patients who developed severe supraglottic airway obstruction due to Epstein-Barr virus lymphoproliferative disease following allogeneic bone marrow transplantation. In addition to enlarged pharyngeal lymphoid tissue seen in all three patients, two had supraglottic airway narrowing and two developed pulmonary lymphoproliferative disease. They were treated with unmanipulated T cells or EBV-specific cytotoxic T lymphocytes. Life-threatening upper airway obstruction is a radiologically detectable complication of allogeneic bone marrow transplantation in children. (orig.) With 3 figs., 1 tab., 12 refs.

  14. The clinical research of bone scan in patients with fibrous dysplasia of bone

    International Nuclear Information System (INIS)

    Yuan Zhibin; Yu Jianfang; Luo Quanyong; Lu Hankui; Zhu Jifang; Zhu Ruisen

    2002-01-01

    Objective: To study the characteristics of fibrous dysplasia of bone in bone imaging and evaluate the diagnostic value of radionuclide bone scan in fibrous dysplasia of bone. Methods: All 42 cases of fibrous dysplasia of bone patients had radionuclide bone scan performed and compared with other imaging modalities. A retrospective study method was used to analyze the imaging results. Results: Although fibrous dysplasia of bone showed uptake of 99m Tc-MDP in the images, its appearance characteristic was different from those metastatic bone tumors and other bone diseases. Combining with X rays and other imaging modalities can improve the diagnostic accuracy of this disease. Conclusion: Radionuclide bone scan has got certain value in the diagnosis of fibrous dysplasia of bone. Combining with other imaging modality can make up its disadvantage of low specificity

  15. Beyond the functional matrix hypothesis: a network null model of human skull growth for the formation of bone articulations.

    Science.gov (United States)

    Esteve-Altava, Borja; Rasskin-Gutman, Diego

    2014-09-01

    Craniofacial sutures and synchondroses form the boundaries among bones in the human skull, providing functional, developmental and evolutionary information. Bone articulations in the skull arise due to interactions between genetic regulatory mechanisms and epigenetic factors such as functional matrices (soft tissues and cranial cavities), which mediate bone growth. These matrices are largely acknowledged for their influence on shaping the bones of the skull; however, it is not fully understood to what extent functional matrices mediate the formation of bone articulations. Aiming to identify whether or not functional matrices are key developmental factors guiding the formation of bone articulations, we have built a network null model of the skull that simulates unconstrained bone growth. This null model predicts bone articulations that arise due to a process of bone growth that is uniform in rate, direction and timing. By comparing predicted articulations with the actual bone articulations of the human skull, we have identified which boundaries specifically need the presence of functional matrices for their formation. We show that functional matrices are necessary to connect facial bones, whereas an unconstrained bone growth is sufficient to connect non-facial bones. This finding challenges the role of the brain in the formation of boundaries between bones in the braincase without neglecting its effect on skull shape. Ultimately, our null model suggests where to look for modified developmental mechanisms promoting changes in bone growth patterns that could affect the development and evolution of the head skeleton. © 2014 Anatomical Society.

  16. Deoxypyridinoline level in gingival crevicular fluid as alveolar bone loss biomarker in periodontal disease

    Directory of Open Access Journals (Sweden)

    Agustin Wulan Suci Dharmayanti

    2012-06-01

    Full Text Available Background: Periodontal diseases have high prevalence in Indonesia. They are caused by bacteria plaque that induced host response to release pro inflammatory mediator. Pro inflammatory mediators and bacteria product cause degradation of collagen fibers in periodontal tissue. Deoxypyridinoline is one of pyridinoline cross-link of collagen type I that can be used as biomarker in bone metabolic diseases, however, their contribution to detect alveolar bone loss in periodontal diseases remains unclear. Purpose: This study was to evaluate deoxypyridinoline level in gingival crevicular fluid as alveolar bone loss biomarker on periodontal disease. Methods: This study used 24 subjects with periodontal diseases and 6 healthy subjects. Dividing of periodontal disease was based on index periodontal. Gingival crevicular fluid was taken at mesial site of maxillary posterior tooth by paper point and deoxypyridinoline be measured by ELISA technique. Results: We found increasing of deoxypyridinoline level following of the severity of periodontal diseases. There was also significant difference between healthy subjects and periodontal diseases subjects (p<0.05. Conclusion: Deoxypyridinoline level in gingiva crevicular fluid can be used as alveolar bone loss biomarker in periodontal disease subjects.Latar belakang: Prevalensi penyakit periodontal di Indonesia cukup tinggi. Ini disebabkan oleh bakteri plak yang merangsang respon tubuh untuk mengeluarkan mediator keradangan. Mediator keradangan dan produk bakteri menyebabkan degradasi serat kolagen jaringan periodontal. Deoksipiridinolin merupakan salah satu ikatan piridinium dari kolagen tipe I yang dapat digunakan sebagai biomarker penyakit metabolisme tubuh. Akan tetapi, penggunaan deoksipiridinolin untuk mendeteksi kehilangan tulang alveolar pada penyakit periodontal masih belum jelas. Tujuan: Tujuan penelitian ini untuk mengetahui bahwa kadar deoksipiridinolin pada cairan krevikular gingival dapat digunakan

  17. Sacro-iliac joint disease in drug abusers: The role of bone scintigraphy

    International Nuclear Information System (INIS)

    Lopez-Majano, V.; Miskew, D.B.W.; Cook County Hospital, Chicago, IL

    1980-01-01

    Bone scintigrams demonstrated increased uptake in the sacroiliac joint in twenty drug addicts with low back pain and signs of localized sepsis. The localization of the disease was decisive for the orthopedist in the aspiration of the affected joint. (orig.)

  18. Effects of single-agent bortezomib as post-transplant consolidation therapy on multiple myeloma-related bone disease

    DEFF Research Database (Denmark)

    Sezer, Orhan; Beksac, Meral; Hajek, Roman

    2017-01-01

    This phase II study explored the effects of bortezomib consolidation versus observation on myeloma-related bone disease in patients who had a partial response or better after frontline high-dose therapy and autologous stem cell transplantation. Patients were randomized to receive four 35-day cycles...... from baseline to end of treatment in bone mineral density (BMD). End-of-treatment rates (bortezomib versus observation) of complete response/stringent complete response were 22% vs. 11% (P = 0·19), very good partial response or better of 80% vs. 68% (P = 0·17), and progressive disease of 8% vs. 23% (P...... with observation, bortezomib appeared to have little impact on bone metabolism/health, but was associated with trends for improved myeloma response and survival....

  19. Bone marrow cell migration to the heart in a chimeric mouse model of acute chagasic disease.

    Science.gov (United States)

    Irion, Camila Iansen; Paredes, Bruno Diaz; Brasil, Guilherme Visconde; Cunha, Sandro Torrentes da; Paula, Luis Felipe; Carvalho, Alysson Roncally; Carvalho, Antonio Carlos Campos de; Carvalho, Adriana Bastos; Goldenberg, Regina Coeli Dos Santos

    2017-08-01

    Chagas disease is a public health problem caused by infection with the protozoan Trypanosoma cruzi. There is currently no effective therapy for Chagas disease. Although there is some evidence for the beneficial effect of bone marrow-derived cells in chagasic disease, the mechanisms underlying their effects in the heart are unknown. Reports have suggested that bone marrow cells are recruited to the chagasic heart; however, studies using chimeric mouse models of chagasic cardiomyopathy are rare. The aim of this study was to investigate the migration of bone marrow cells to the heart after T. cruzi infection in a model of chagasic disease in chimeric mice. To obtain chimerical mice, wild-type (WT) C57BL6 mice were exposed to full body irradiation (7 Gy), causing bone marrow ablation. Then, bone marrow cells from green fluorescent protein (GFP)-transgenic mice were infused into the mice. Graft effectiveness was confirmed by flow cytometry. Experimental mice were divided into four groups: (i) infected chimeric (iChim) mice; (ii) infected WT (iWT) mice, both of which received 3 × 104 trypomastigotes of the Brazil strain; (iii) non-infected chimeric (Chim) mice; and (iv) non-infected WT mice. At one-month post-infection, iChim and iWT mice showed first degree atrioventricular block with decreased heart rate and treadmill exercise parameters compared to those in the non-infected groups. iChim mice showed an increase in parasitaemia, myocarditis, and the presence of amastigote nests in the heart tissue compared to iWT mice. Flow cytometry analysis did not detect haematopoietic progenitor cells in the hearts of infected mice. Furthermore, GFP+ cardiomyocytes were not detected in the tissues of chimeric mice.

  20. Multifocal bone and bone marrow lesions in children - MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Raissaki, Maria; Demetriou, Stelios; Spanakis, Konstantinos; Skiadas, Christos; Karantanas, Apostolos H. [University of Crete, Faculty of Medicine, Department of Radiology, University Hospital of Heraklion, Heraklion, Crete (Greece); Katzilakis, Nikolaos; Stiakaki, Eftichia [University of Crete, Faculty of Medicine, Department of Pediatric Hematology-Oncology, University Hospital of Heraklion, Heraklion, Crete (Greece); Velivassakis, Emmanouil G. [University Hospital of Heraklion, Orthopedic Clinic, Heraklion, Crete (Greece)

    2017-03-15

    Polyostotic bone and bone marrow lesions in children may be due to various disorders. Radiographically, lytic lesions may become apparent after loss of more than 50% of the bone mineral content. Scintigraphy requires osteoblastic activity and is not specific. MRI may significantly contribute to the correct diagnosis and management. Accurate interpretation of MRI examinations requires understanding of the normal conversion pattern of bone marrow in childhood and of the appearances of red marrow rests and hyperplasia. Differential diagnosis is wide: Malignancies include metastases, multifocal primary sarcomas and hematological diseases. Benign entities include benign tumors and tumor-like lesions, histiocytosis, infectious and inflammatory diseases, multiple stress fractures/reactions and bone infarcts/ischemia. (orig.)

  1. Chemical and biomechanical characterization of hyperhomocysteinemic bone disease in an animal model

    Directory of Open Access Journals (Sweden)

    Howell David S

    2003-02-01

    Full Text Available Abstract Background Classical homocystinuria is an autosomal recessive disorder caused by cystathionine β-synthase (CBS deficiency and characterized by distinctive alterations of bone growth and skeletal development. Skeletal changes include a reduction in bone density, making it a potentially attractive model for the study of idiopathic osteoporosis. Methods To investigate this aspect of hyperhomocysteinemia, we supplemented developing chicks (n = 8 with 0.6% dl-homocysteine (hCySH for the first 8 weeks of life in comparison to controls (n = 10, and studied biochemical, biomechanical and morphologic effects of this nutritional intervention. Results hCySH-fed animals grew faster and had longer tibiae at the end of the study. Plasma levels of hCySH, methionine, cystathionine, and inorganic sulfate were higher, but calcium, phosphate, and other indices of osteoblast metabolism were not different. Radiographs of the lower limbs showed generalized osteopenia and accelerated epiphyseal ossification with distinct metaphyseal and suprametaphyseal lucencies similar to those found in human homocystinurics. Although biomechanical testing of the tibiae, including maximal load to failure and bone stiffness, indicated stronger bone, strength was proportional to the increased length and cortical thickness in the hCySH-supplemented group. Bone ash weights and IR-spectroscopy of cortical bone showed no difference in mineral content, but there were higher Ca2+/PO43- and lower Ca2+/CO32- molar ratios than in controls. Mineral crystallization was unchanged. Conclusion In this chick model, hyperhomocysteinemia causes greater radial and longitudinal bone growth, despite normal indices of bone formation. Although there is also evidence for an abnormal matrix and altered bone composition, our finding of normal biomechanical bone strength, once corrected for altered morphometry, suggests that any increase in the risk of long bone fracture in human hyperhomocysteinemic

  2. Evaluation of Medial Acetabular Wall Bone Stock in Patients with Developmental Dysplasia of the Hip Using a Helical Computed Tomography Multiplanar Reconstruction Technique

    Energy Technology Data Exchange (ETDEWEB)

    Rui Yu Liu; Kun Zheng Wang; Chun Sheng Wang; Xiao Qian Dang; Zhi Qin Tong (Second Hospital Affiliated to the Medical College of Xi' an Jiaotong Univ., Xi' an Shaanxi (China))

    2009-08-15

    Background: The technique of medialization has been used to reconstruct acetabula at the level of true acetabula in total hip arthroplasty (THA) in patients with developmental dysplasia of the hip (DDH). Appreciation of the bone stock in the medial acetabular wall is significant for making an optimal acetabular reconstruction plan and avoiding complications. Purpose: To evaluate the bone stock of the medial acetabular wall and its relation to the degree of subluxation in patients with DDH using computed tomography (CT). Material and Methods: Helical CT scans of 27 hips were obtained from 21 patients with osteoarthritis secondary to DDH who were scheduled for total hip arthroplasty. Eleven hips belonged to Crowe class I, while 16 hips belonged to Crowe class II/III. The raw CT data were reprocessed in various planes by scrolling multiplanar reformation (MPR). Acetabular opening, depth, and medial bone stock, as indicated by the minimum thickness of the medial acetabular wall, were measured in the transverse reformed MPR plane. Results: The minimum thicknesses of the medial acetabular wall in Crowe-I and Crowe-II/III hips were 3.8+-2.1 mm and 7.1+-3.1 mm, respectively, with statistically significant differences between the groups (P<0.05). Furthermore, the bone stock in the medial acetabular wall correlated with the degree of subluxation (R=0.69) and the acetabular depth (R= ;- ;0.71). Conclusion: There was significantly more bone stock in the medial acetabular wall in patients with higher-degree subluxation than there was in the less-severe class. This difference should be taken into consideration when reconstructing acetabula in THA in patients with DDH using the technique of medialization

  3. Evaluation of Medial Acetabular Wall Bone Stock in Patients with Developmental Dysplasia of the Hip Using a Helical Computed Tomography Multiplanar Reconstruction Technique

    International Nuclear Information System (INIS)

    Rui Yu Liu; Kun Zheng Wang; Chun Sheng Wang; Xiao Qian Dang; Zhi Qin Tong

    2009-01-01

    Background: The technique of medialization has been used to reconstruct acetabula at the level of true acetabula in total hip arthroplasty (THA) in patients with developmental dysplasia of the hip (DDH). Appreciation of the bone stock in the medial acetabular wall is significant for making an optimal acetabular reconstruction plan and avoiding complications. Purpose: To evaluate the bone stock of the medial acetabular wall and its relation to the degree of subluxation in patients with DDH using computed tomography (CT). Material and Methods: Helical CT scans of 27 hips were obtained from 21 patients with osteoarthritis secondary to DDH who were scheduled for total hip arthroplasty. Eleven hips belonged to Crowe class I, while 16 hips belonged to Crowe class II/III. The raw CT data were reprocessed in various planes by scrolling multiplanar reformation (MPR). Acetabular opening, depth, and medial bone stock, as indicated by the minimum thickness of the medial acetabular wall, were measured in the transverse reformed MPR plane. Results: The minimum thicknesses of the medial acetabular wall in Crowe-I and Crowe-II/III hips were 3.8±2.1 mm and 7.1±3.1 mm, respectively, with statistically significant differences between the groups (P<0.05). Furthermore, the bone stock in the medial acetabular wall correlated with the degree of subluxation (R=0.69) and the acetabular depth (R= ;- ;0.71). Conclusion: There was significantly more bone stock in the medial acetabular wall in patients with higher-degree subluxation than there was in the less-severe class. This difference should be taken into consideration when reconstructing acetabula in THA in patients with DDH using the technique of medialization

  4. Investigation of the collagen-mineral-relation in bone with special respect to bone diseases with collagen defects by small-angle X-ray scattering

    International Nuclear Information System (INIS)

    Schreiber, S. A.

    1996-06-01

    Small-angle X-ray scattering (SAXS) was used to study the structure of the collagen/mineral composite of bone in the nanometer range. The most important results were: - In horse radius, the angular distribution of mineral crystals as measured by SAXS agreed well with previous measurements of collagen orientation using circularly polarized light microscopy. This shows that the crystals are parallel to the collagen fibrils. - The effect of sodium fluoride, which stimulates bone formation, and bisphosphonates, which reduce bone resorption, were analyzed. A slight increase in the average thickness of the mineral crystals as well as changes in the structure of the mineral/collagen composite were found in the case of fluoride treated animals. No differences were found between alendronate treated animals and controls. The changes with NaF correlate with bone weakening found in an earlier study with the same animals. - In cortical bone from 9 patients with Osteogenesis Imperfecta (brittle bone disease) the mean thickness of the mineral crystals was found approximately constant around 2.4 nm, while in control bones it constantly increased with age up to about 3.5 nm. In addition, the parallel alignment of the mineral crystals was less in OI-bone than in normal controls. Hence, despite the great variability of this genetic collagen defect, smaller and less well aligned mineral crystals seem to characterize the collagen/mineral composite in OI-bone. (author)

  5. Combined scintigraphic and radiographic diagnosis of bone and joint diseases. Including gamma correction interpretation. 4. rev. and enl. ed.

    Energy Technology Data Exchange (ETDEWEB)

    Bahk, Yong-Whee [Sung Ae General Hospital, Seoul (Korea, Republic of). Dept. of Nuclear Medicine and Radiology

    2013-07-01

    In this fourth edition of Combined Scintigraphic and Radiographic Diagnosis of Bone and Joint Diseases, the text has been thoroughly amended, updated, and partially rearranged to reflect the latest advances. In addition to discussing the role of pinhole imaging in the range of disorders previously covered, the new edition pays detailed attention to the novel diagnostic use of gamma correction pinhole bone scan in a broad spectrum of skeletal disorders, including physical, traumatic, and sports injuries, infectious and non-infectious bone diseases, benign and malignant bone tumors, and soft tissue diseases. A large number of state of the art pinhole scans and corroborative CT, MRI, and/or ultrasound images are presented side by side. The book has been enlarged to encompass various new topics, including occult fractures; cervical sprain and whiplash trauma; bone marrow edema; microfractures of trabeculae; evident, gaping, and stress fractures; and differential diagnosis. This new edition will be essential reading for practitioners and researchers in not only nuclear medicine but also radiology, orthopedic surgery, and pathology.

  6. Combined scintigraphic and radiographic diagnosis of bone and joint diseases. Including gamma correction interpretation. 4. rev. and enl. ed.

    International Nuclear Information System (INIS)

    Bahk, Yong-Whee

    2013-01-01

    In this fourth edition of Combined Scintigraphic and Radiographic Diagnosis of Bone and Joint Diseases, the text has been thoroughly amended, updated, and partially rearranged to reflect the latest advances. In addition to discussing the role of pinhole imaging in the range of disorders previously covered, the new edition pays detailed attention to the novel diagnostic use of gamma correction pinhole bone scan in a broad spectrum of skeletal disorders, including physical, traumatic, and sports injuries, infectious and non-infectious bone diseases, benign and malignant bone tumors, and soft tissue diseases. A large number of state of the art pinhole scans and corroborative CT, MRI, and/or ultrasound images are presented side by side. The book has been enlarged to encompass various new topics, including occult fractures; cervical sprain and whiplash trauma; bone marrow edema; microfractures of trabeculae; evident, gaping, and stress fractures; and differential diagnosis. This new edition will be essential reading for practitioners and researchers in not only nuclear medicine but also radiology, orthopedic surgery, and pathology.

  7. Bone and marrow imaging in sickle cell disease: diagnosis of infarction

    International Nuclear Information System (INIS)

    Lutzker, L.G.; Alavi, A.

    1976-01-01

    Sickling of erythrocytes in patients with S-hemoglobin causes marrow and bone infarction. The former can be demonstrated as a lack of /sup 99m/Tc-sulfur colloid uptake on marrow imaging examination. These defects may resolve or persist long after the acute episode. If the bone is involved in the acute episode, imaging within the first few days of onset of symptoms can show lack of /sup 99m/Tc-labeled phosphate uptake, usually in a smaller area than that shown by marrow scanning. Follow-up bone imaging shows increased activity, particularly along the circumference of the bone where periosteal reaction can be demonstrated radiographically. Magnification by use of the pinhole collimator provides better definition of the uptake defect and the distribution of the increased reactive uptake. Timing of examination is important. If marrow imaging is performed in an asymptomatic period, the repeat examination during a painful crisis permits differentiation of old and acute marrow infarction. If /sup 99m/Tc-phosphate imaging is performed after about 2 days of symptoms, acute infarction can be differentiated from osteomyelitis, which it may mimic clinically. To assist in differentiating bone infection in a site of marrow infarction demonstrated by marrow imaging, serial bone imaging with magnification may be useful. The uptake defect, followed in several days to 2 weeks, by circumferential increased activity, is a different pattern than the homogeneously intense activity of osteomyelitis, but the peripheral distribution may not be apparent on routine imaging. It is hoped that the utilization of these techniques can decrease the emotional and economic costs of prolonged hospitalization for suspected infection and can also expand our knowledge of the complex pathophysiologic changes of sickle cell bone disease

  8. Biochemical parameters of bone metabolism in bone metastases of solid tumors (Review)

    NARCIS (Netherlands)

    Meijer, Wilhelmus; van der Veer, E; Willemse, P H

    1998-01-01

    The role of biochemical markers of bone metabolism in the diagnosis and monitoring of bone metastases in solid tumors is reviewed. Emphasis is on the recently developed markers, which may provide a more accurate quantitation of bone metabolism. In metastatic bone disease, bone formation and

  9. Unusual case of metabolic bone disease in a common marmoset (Callithrix jacchus)

    International Nuclear Information System (INIS)

    Hatt, J.M.; Sainsbury, A.W.

    1998-01-01

    Metabolic bone disease was diagnosed in an 11-month-old female common marmoset (Callithrix jacchus). It was depressed, reluctant to move, and was cachectic and small for its age. Laboratory findings included anaemia, azotaemia and an inverse calcium to phosphorus ratio. The radiological findings showed simultaneous signs of osteomalacia and soft-tissue calcification. There was decreased bone density with lytic areas in the pelvis and femur, and severe bilateral nephrocalcinosis. Postmortem examination revealed marked focal dystrophic calcification of the epi- and myocardium. Calcium and vitamin D3 deficiency (nutritional secondary hyperparathyroidism) was the most likely cause of the osteomalacia

  10. Meeting report of the 2016 bone marrow adiposity meeting.

    Science.gov (United States)

    van der Eerden, Bram; van Wijnen, André

    2017-10-02

    There is considerable interest in the physiology and pathology, as well as the cellular and molecular biology, of bone marrow adipose tissue (BMAT). Because bone marrow adiposity is linked not only to systemic energy metabolism, but also to both bone marrow and musculoskeletal disorders, this biologic compartment has become of major interest to investigators from diverse disciplines. Bone marrow adiposity represents a virtual multi-tissue endocrine organ, which encompasses cells from multiple developmental lineages (e.g., mesenchymal, myeloid, lymphoid) and occupies all the non-osseous and non-cartilaginous space within long bones. A number of research groups are now focusing on bone marrow adiposity to understand a range of clinical afflictions associated with bone marrow disorders and to consider mechanisms-based strategies for future therapies.

  11. Study on the relationship between serum IL-6, Sgp80, Sgp130 levels and bone metabolism in patients with Graves' disease

    International Nuclear Information System (INIS)

    Liu Chunyu; Mu Junqing; Zou Jianwen; Liu Songtao

    2007-01-01

    Objective: To investigate the relationship between serum IL-6, Sgp80, Sgpl30 levels and bone metabolism in patients with Graves' disease. Methods: Serum levels of IL-6 (with RIA), Sgp80, Sgpl30 (with ELISA) as well as BMD of proximal femur region were determined in 78 patients with Graves' disease (26 not treated yet, 26 partially remitted and 26 in remission) and 30 controls. Results: The serum IL-6 and Sgpl30 levels in all the patients with Graves' disease were significantly higher than those in controls (P 3 , FT 4 , phosphorous, BGP and urinary hydroxy proline levels. BMD of the proximal femur region in patients partially remitted were much lower than that in controls (P<0.05). Conclusion: Serum Sgp80 level might be taken as an important parameter in studying of Graves' disease. Bone loss, both cortical and trabecular, occurred in patients with Graves' disease. Increase of bone formation as well as bone resorption were primary, not secondary to each other. (authors)

  12. Differentiation of malignant and degenerative benign bone disease using Tc-99m Citrate and Tc-99m MDP scintigraphy

    International Nuclear Information System (INIS)

    Jin, J.; Guo, R.; Li, S.-J.; Ren, Y.; Zhang, C.; Zhang, X.

    2007-01-01

    Full text: For the evaluation of bone metastases in patients (pts) with cancer, 99mTcMDP bone scintigraphy is an important tool, but some limitations exist. One of these is the differential diagnosis of malignant and degenerative benign bone disease. The aim of this study was to differentiate them using 99mTcCitrate and 99mTcMDP scintigraphy. Methods: 39 pts (92 lesions) with known malignant or degenerative benign bone disease were studied. 23 pts had malignant bone disease (48 lesions, group 1), the other 16 pts had degenerative benign bone disease (44 lesions, group2), for which the results of 99mTcMDP scintigraphy were positive. In both groups, 99mTcCitrate scintigraphy was performed within a time interval of 2-7 days after 99mTcMDP scintigraphy (555∼740MBq. static, 3hr, planar or SPECT i m a g e s w h e n r e q u i r e d ) . The 99mTccitrate/99mTcMDP lesion-to-background radioisotope uptake ratio (RUR) was calculated for each lesion. Conventional techniques (histopathology, X-ray, CT, MRI and clinical follow up) were considered to be proof of the presence of bone metastases and degenerative benign bone disease. Results: Uptake of 99mTcMDP in the two groups is the same (1.96±0.25 vs. 1.87±0.21; t=1.178, P>0.20), while in 99mTcCitrate image, malignant lesions demonstrated a higher uptake of lesion activity than that of benign degenerative lesions (1.47±0.42 vs. 1.09±0.38; t=2.887, P<0.01). The mean 99mTccitrate/99mTcMDP RUR in the malignant group was significantly higher than the mean in the benign group (0.78±0.21 vs. 0.54±0.19; t=3.646, P<0.001). Conclusions: The preliminary results of the study confirm the usefulness and feasibility of 99mTcCitrate scintigraphy for differentiating malignant from benign degenerative lesions seen as areas of increased activity on 99mTcMDP bone scintigraphy. (author)

  13. The Impact of Conventional and Biological Disease Modifying Antirheumatic Drugs on Bone Biology. Rheumatoid Arthritis as a Case Study.

    Science.gov (United States)

    Barreira, Sofia Carvalho; Fonseca, João Eurico

    2016-08-01

    The bone and the immune system have a very tight interaction. Systemic immune-mediated inflammatory diseases, such as rheumatoid arthritis (RA), induce bone loss, leading to a twofold increase in osteoporosis and an increase of fragility fracture risk of 1.35-2.13 times. This review focuses on the effects of conventional and biological disease modifying antirheumatic drugs (DMARDs) on bone biology, in the context of systemic inflammation, with a focus on RA. Published evidence supports a decrease in osteoclastic activity induced by DMARDs, which leads to positive effects on bone mineral density (BMD). It is unknown if this effect could be translated into fracture risk reduction. The combination with antiosteoclastic drugs can have an additional benefit.

  14. Co-existent Paget’s Disease of the Bone, Prostate Carcinoma Skeletal Metastases and Fracture on Skeletal Scintigraphy-Lessons to be Learned

    Directory of Open Access Journals (Sweden)

    Luke I Sonoda

    2013-08-01

    Full Text Available Bone scintigraphy, despite being non-specific, is a very sensitive and simple investigation for patients with active Paget’s disease of the bone. Skeletal metastases and Paget’s disease may co-exist in the elderly patients as both conditions are commonly seen in this age group. Clinical and radiological correlation may help to improve the diagnostic specificity of a bone scintigram. We report a patient in whom concurrent Paget’s disease and a rib fracture became evident only on repeat scintigraphy following successful treatment of prostate carcinoma skeletal metastases.

  15. Bone and bone-marrow blood flow in chronic granulocytic leukemia and primary myelofibrosis

    International Nuclear Information System (INIS)

    Lahtinen, R.; Lahtinen, T.; Romppanen, T.

    1982-01-01

    Blood flow in hematopoietic bone marrow and in nonhematopoietic bone has been measured with a Xe-133 washout method in 20 patients with chronic granulocytic leukemia (CGL) and in seven with primary myelofibrosis. Age-matched healthy persons served as controls. Bone-marrow blood flow in CGL was dependent upon the phase of the disease. In the metamorphosis phase, bone-marrow blood flow was high compared with that in the well-controlled phase. Apart from the initial phase, the mean values for bone blood flow in CGL were increased compared with the values of the healthy controls. In myelofibrosis the bone blood flow was also increased. Bone-marrow blood flow in these diseases was dependent upon the cellularity of bone marrow as measured morphometrically

  16. Bone markers in craniofacial bone deformations and dysplasias

    Directory of Open Access Journals (Sweden)

    Monika Seifert

    2015-10-01

    Full Text Available Various forms of bony deformations and dysplasias are often present in the facial skeleton. Bone defects can be either localized or general. Quite often they are not only present in the skull but also can be found in other parts of the skeleton. In many cases the presence and levels of specific bone markers should be measured in order to fully describe their activity and presence in the skeleton. Fibrous dysplasia (FD is the most common one in the facial skeleton; however, other bone deformations regarding bone growth and activity can also be present. Every clinician should be aware of all common, rare and uncommon bony diseases and conditions such as cherubism, Paget’s disease, osteogenesis imperfecta and others related to genetic conditions. We present standard (calcium, parathyroid hormone, calcitonin, alkaline phosphatase, vitamin D and specialized bone markers (pyridinium, deoxypyridinium, hydroxyproline, RANKL/RANK/OPG pathway, growth hormone, insulin-like growth hormone-1 that can be used to evaluate, measure or describe the processes occurring in craniofacial bones.

  17. Magnetic resonance imaging of bone marrow changes in Gaucher disease during enzyme replacement therapy: first German long-term results

    International Nuclear Information System (INIS)

    Poll, L.W.; Koch, J.A.; Scherer, A.; Boerner, D.; Moedder, U.; Dahl, S. vom; Niederau, C.; Haeussinger, D.; Willers, R.

    2001-01-01

    Objective:. Since 1991, enzyme replacement therapy (ERT) has been available for patients with Gaucher disease in Germany. The aim of this study was to analyse the MR pattern of bone marrow involvement and response to ERT in Gaucher disease type I. Patients and design:. Thirty patients with Gaucher disease type I had MRI examinations prior to initiation of ERT with alglucerase/imiglucerase and during follow-up. Median MR follow-up and duration of ERT were 36 months. Coronal T1- and T2-weighted spin-echo images of the lower extremities were obtained to evaluate changes in the appearance of yellow marrow. MR images were categorized as having either a homogeneous (type A) or non-homogeneous patchy (type B) appearance of bone involvement and response to ERT was assessed by two radiologists. Results:. Overall, 19 of 30 patients (63%) showed an increased signal intensity on T1- and T2-weighted images after 36 months of ERT, consistent with partial reconversion of fatty marrow during treatment. Focal bone lesions surrounded by a low signal intensity (SI) rim did not respond to ERT, suggesting bone infarcts. Of the 11 patients with bone infarcts (low SI rim lesion), 82% had the non-homogeneous type B pattern (P=0.0021). In 86% of patients with splenectomy, bone infarcts were seen (P<0.05). Conclusions:. MRI using T1- and T2-weighted spin-echo sequences is a valuable, non-invasive method for monitoring bone marrow response in patients receiving ERT. A non- homogeneous patchy signal intensity of bone marrow involvement correlates with the presence of bone infarcts (P=0.0021). (orig.)

  18. Magnetic resonance imaging of bone marrow changes in Gaucher disease during enzyme replacement therapy: first German long-term results

    Energy Technology Data Exchange (ETDEWEB)

    Poll, L.W.; Koch, J.A.; Scherer, A.; Boerner, D.; Moedder, U. [Duesseldorf Univ. (Germany). Inst. fuer Diagnostische Radiologie; Dahl, S. vom; Niederau, C.; Haeussinger, D. [Duesseldorf Univ. (Germany). Medizinische Fakultaet; Willers, R. [Duesseldorf Univ. (Germany). Rechenzentrum

    2001-09-01

    Objective:. Since 1991, enzyme replacement therapy (ERT) has been available for patients with Gaucher disease in Germany. The aim of this study was to analyse the MR pattern of bone marrow involvement and response to ERT in Gaucher disease type I. Patients and design:. Thirty patients with Gaucher disease type I had MRI examinations prior to initiation of ERT with alglucerase/imiglucerase and during follow-up. Median MR follow-up and duration of ERT were 36 months. Coronal T1- and T2-weighted spin-echo images of the lower extremities were obtained to evaluate changes in the appearance of yellow marrow. MR images were categorized as having either a homogeneous (type A) or non-homogeneous patchy (type B) appearance of bone involvement and response to ERT was assessed by two radiologists. Results:. Overall, 19 of 30 patients (63%) showed an increased signal intensity on T1- and T2-weighted images after 36 months of ERT, consistent with partial reconversion of fatty marrow during treatment. Focal bone lesions surrounded by a low signal intensity (SI) rim did not respond to ERT, suggesting bone infarcts. Of the 11 patients with bone infarcts (low SI rim lesion), 82% had the non-homogeneous type B pattern (P=0.0021). In 86% of patients with splenectomy, bone infarcts were seen (P<0.05). Conclusions:. MRI using T1- and T2-weighted spin-echo sequences is a valuable, non-invasive method for monitoring bone marrow response in patients receiving ERT. A non- homogeneous patchy signal intensity of bone marrow involvement correlates with the presence of bone infarcts (P=0.0021). (orig.)

  19. Prospective evaluation of the super scan of metabolic bone disease (abstract)

    International Nuclear Information System (INIS)

    Khan, A.U.; Ahmad, S.; Khan, A.A.; Khan, S.M.; Rauf, M.

    1999-01-01

    A total of 27 cases (23 females and 4 males) having super scan of metabolic bone disease were prospectively evaluated over a period of 2 years (Jan. 1997 to Dec. 1998) at the Nuclear Medicine Department (NMD), institute of Radiotherapy and Nuclear Medicine (IRNUM), Peshawar to identify various causes for the super scan picture on Tc-99m MDP bone scintigraphy in our clinical environment. After the three observer confirmation of the bone scan, the serum calcium and Parathyroid Hormone (PTH) estimation was done. The patients having serum PTH greater than 250 Pg/L under went two phase Parathyroid MIBI Scintigraphy 2PP MIBI scan) for the detection of parathyroid adenoma. The patients having positive scans for parathyroid Adenoma were subjected to surgery and histopathological confirmation was obtained. Selected cases under went a trial of deport preparation of vitamin D3 calcium supplementation. The final diagnosis of 16 patients was osteomalacia (59%), six patients had histopathologically confirmed parathyroid adenoma (22.2%), one case each was that of toxic thyroid adenoma (3.7%) and chronic renal failure (3.7%). In three cases the final diagnosis was not reached (11.21%). Osteomalacia and parathyroid adenoma are the two most common causes for the super scan picture on bone scintigraphy. (author)

  20. Detection of active alveolar bone destruction in human periodontal disease by analysis of radiopharmaceutical uptake after a single injection of 99m-Tc-methylene diphosphonate

    International Nuclear Information System (INIS)

    Jeffcoat, M.K.; Williams, R.C.; Holman, B.L.; English, R.; Goldhaber, P.

    1986-01-01

    Previous studies have shown that, following a single injection of 99m-Tc-MDP, measurement of bone-seeking radiopharmaceutical uptake can detect ''active'' alveolar bone loss due to periodontal disease in beagle dogs, as determined by radiographs taken at the time of, and several months after, the nuclear medicine procedure. The efficacy of this diagnostic test, however, had not been assessed in human periodontal disease. The ability of a single boneseeking radiopharmaceutical uptake examination to detect ''active'' alveolar bone loss due to periodontal disease in human patients was assessed by comparing a single uptake measurement to the rate of bone loss determined from serial radiographs taken over a 6-month period. Uptake was expressed as a ratio of the cpm from the alveolar bone divided by the cpm from the non-tooth supporting bone of the nuchal crest. High uptake ratios were associated with ''active'' loss and low uptake ratios were associated with little if any change in alveolar bone height (p<0.001). The nuclear medicine examination was an accurate detector of periodontal disease activity in nearly 80% of the individual teeth studied. These data indicate that high bone-seeking radiopharmaceutical uptake ratios may be pathognomonic of active bone loss in human periodontal disease. (author)

  1. DIAGNOSTICS OF BONE METABOLISM DISORDERS IN ONCOLOGICAL DISEASES

    Directory of Open Access Journals (Sweden)

    O. I. Apolikhin

    2015-01-01

    Full Text Available Osteoporosis is one of the most significant bone complications of cancer. About 1.5 million cancer patients worldwide have bone metastases. Patients with myeloma, breast cancer, prostate, thyroid, bladder and lung have very high risk of development of bone lesions and related complications. Currently, osteodensitometry is the gold standard for the diagnosis of osteoporosis. In recent years we frequently use the innovative imaging techniques for bone metastases, such as CT, MRI, PET/CT. Unfortunately, the diagnostic value of these methods is that it is not always possible to identify abnormalities of bone metabolism in cancer, especially in the early stages. This review shows the world experience of usage of biochemical markers of bone resorption (calcium, hydroxyproline, NTX, CTX, PYD, DPD, TRAP-5b, bone sialoprotein - BSP and markers of bone synthesis (osteocalcin, CSF, ACF, Karlovy vary IFF, their advantages and disadvantages. The level of these markers is increased in most patients with osteoporosis and bone metastases, it is suggesting a potential role in early diagnosis of bone metastases.

  2. Development of a preclinical orthotopic xenograft model of ewing sarcoma and other human malignant bone disease using advanced in vivo imaging.

    Directory of Open Access Journals (Sweden)

    Britta Vormoor

    Full Text Available Ewing sarcoma and osteosarcoma represent the two most common primary bone tumours in childhood and adolescence, with bone metastases being the most adverse prognostic factor. In prostate cancer, osseous metastasis poses a major clinical challenge. We developed a preclinical orthotopic model of Ewing sarcoma, reflecting the biology of the tumour-bone interactions in human disease and allowing in vivo monitoring of disease progression, and compared this with models of osteosarcoma and prostate carcinoma. Human tumour cell lines were transplanted into non-obese diabetic/severe combined immunodeficient (NSG and Rag2(-/-/γc(-/- mice by intrafemoral injection. For Ewing sarcoma, minimal cell numbers (1000-5000 injected in small volumes were able to induce orthotopic tumour growth. Tumour progression was studied using positron emission tomography, computed tomography, magnetic resonance imaging and bioluminescent imaging. Tumours and their interactions with bones were examined by histology. Each tumour induced bone destruction and outgrowth of extramedullary tumour masses, together with characteristic changes in bone that were well visualised by computed tomography, which correlated with post-mortem histology. Ewing sarcoma and, to a lesser extent, osteosarcoma cells induced prominent reactive new bone formation. Osteosarcoma cells produced osteoid and mineralised "malignant" bone within the tumour mass itself. Injection of prostate carcinoma cells led to osteoclast-driven osteolytic lesions. Bioluminescent imaging of Ewing sarcoma xenografts allowed easy and rapid monitoring of tumour growth and detection of tumour dissemination to lungs, liver and bone. Magnetic resonance imaging proved useful for monitoring soft tissue tumour growth and volume. Positron emission tomography proved to be of limited use in this model. Overall, we have developed an orthotopic in vivo model for Ewing sarcoma and other primary and secondary human bone malignancies, which

  3. [Effects of the combined calcitonin and sodium etidronate therapy in Paget's disease of bone].

    Science.gov (United States)

    Nuti, R; Turchetti, V; Righi, G; Vattimo, A

    1982-03-03

    The therapy of Paget's bone disease is essentially based on the use of calcitonin and diphosphonates: both drugs, if used in large doses for long periods, have shown themselves able to provoke particular side-effects. It was, therefore, decided to study the therapeutic efficacy of combined low-dosage treatment using synthetic salmon calcitonin and sodium-etidronate on a group of patients with Paget's osteodystrophy. A clear evident diminution in plasma alkaline phosphatase, hydroxyprolinuria and whole body retention (WBR) of MDP-Tc99m was observed, demonstrating a reduction of metabolic turnover in the bone. No changes in the bone mass (BMC), evaluated by bone mineral detector, were observed at the end of treatment. With this treatment the plateau effect was shown to be appreciably less than normally occurs when either calcitonin or sodium etidronate are used alone.

  4. MRI assessment of bone marrow involvement in Hodgkin disease and non-Hodgkin lymphoma

    International Nuclear Information System (INIS)

    Tesoro Tess, J.D.; Balzarini, L.; Ceglia, E.; Petrillo, R.; Musumeci, R.

    1990-01-01

    In order to evaluate the possible role of MRI in detecting lymphomatous marrow involvement, a MRI examination was performed in newly diagnosed patients with Hodgkin's disease (HD) and nonHodgkin lymphoma (NHL). From this the authors concluded that MRI should not be used as a replacement for bone marrow biopsies in HD and NHL, but rather as a complementary tool utilizing the panoramic view offered by MRI which permit to disclose focal areas of bone involvement different from the sacrum, thus not valuable with routine biopsies. (author). 4 refs.; 1 tab

  5. Chronological study for solitary bone metastasis in the sternum from breast cancer with bone scintigraphy

    International Nuclear Information System (INIS)

    Miyoshi, Hidenao; Otsuka, Nobuaki; Sone, Teruki; Nagai, Kiyohisa; Tamada, Tsutomu; Mimura, Hiroaki; Yanagimoto, Shinichi; Tomomitsu, Tatsushi; Fukunaga, Masao

    1999-01-01

    Since breast cancer is frequently associated with bone metastasis, bone scintigraphies have been performed to determine pre-operative staging and to survey postoperative bone metastasis. The sternum, in particular, is a site at which is difficult to differentiate between benign bone disease and bone metastasis, because of varied uptake and wide individual variations. In this study, chronological bone images were scintigraphied in six cases with solitary sternal metastasis and three cases with benign bone disease including two fracture cases and one arthritis case. On bone scintigrams in which solitary sternal metastasis appeared, increased uptake was found in five cases, and photon deficiency was observed in one case. During follow-up scintigraphies, abnormal accumulations, such as hot spots and cold lesions, increased in the bone metastasis while abnormal uptake disappeared or was unchanged in the benign bone disease cases. On CT, four cases showed osteolytic change, and one exhibited osteosclerotic change. These findings indicate that sternal metastasis usually shows osteolytic change, even if a hot lesion is recognized on bone scintigraphy. In solitary sternal metastasis, for which early diagnosis is difficult, both an integrated diagnosis using other imaging techniques and chronological bone scintigraphy are important. (author)

  6. Biology of Bone: The Vasculature of the Skeletal System.

    Science.gov (United States)

    Watson, Emma C; Adams, Ralf H

    2017-09-11

    Blood vessels are essential for the distribution of oxygen, nutrients, and immune cells, as well as the removal of waste products. In addition to this conventional role as a versatile conduit system, the endothelial cells forming the innermost layer of the vessel wall also possess important signaling capabilities and can control growth, patterning, homeostasis, and regeneration of the surrounding organ. In the skeletal system, blood vessels regulate developmental and regenerative bone formation as well as hematopoiesis by providing vascular niches for hematopoietic stem cells. Here we provide an overview of blood vessel architecture, growth and properties in the healthy, aging, and diseased skeletal system. Copyright © 2017 Cold Spring Harbor Laboratory Press; all rights reserved.

  7. Global and disease-associated genetic variation in the human Fanconi anemia gene family

    OpenAIRE

    Rogers, Kai J.; Fu, Wenqing; Akey, Joshua M.; Monnat, Raymond J.

    2014-01-01

    Fanconi anemia (FA) is a human recessive genetic disease resulting from inactivating mutations in any of 16 FANC (Fanconi) genes. Individuals with FA are at high risk of developmental abnormalities, early bone marrow failure and leukemia. These are followed in the second and subsequent decades by a very high risk of carcinomas of the head and neck and anogenital region, and a small continuing risk of leukemia. In order to characterize base pair-level disease-associated (DA) and population gen...

  8. Vitamin D and adverse pregnancy outcomes: beyond bone health and growth.

    Science.gov (United States)

    Brannon, Patsy M

    2012-05-01

    Concerns exist about adequacy of vitamin D in pregnant women relative to both maternal and fetal adverse health outcomes. Further contributing to these concerns is the prevalence of inadequate and deficient vitamin D status in pregnant women, which ranges from 5 to 84% globally. Although maternal vitamin D metabolism changes during pregnancy, the mechanisms underlying these changes and the role of vitamin D during development are not well understood. Observational evidence links low maternal vitamin D status with an increased risk of non-bone health outcome in the mother (pre-eclampsia, gestational diabetes, obstructed labour and infectious disease), the fetus (gestational duration) and the older offspring (developmental programming of type 1 diabetes, inflammatory and atopic disorders and schizophrenia); but the totality of the evidence is contradictory (except for maternal infectious disease and offspring inflammatory and atopic disorders), lacking causality and, thus, inconclusive. In addition, recent evidence links not only low but also high maternal vitamin D status with increased risk of small-for-gestational age and schizophrenia in the offspring. Rigorous and well-designed randomised clinical trials need to determine whether vitamin D has a causal role in non-bone health outcomes in pregnancy.

  9. A newly developed snack effective for enhancing bone volume

    Directory of Open Access Journals (Sweden)

    Hayashi Hidetaka

    2009-07-01

    Full Text Available Abstract Background The incidence of primary osteoporosis is higher in Japan than in USA and European countries. Recently, the importance of preventive medicine has been gradually recognized in the field of orthopaedic surgery with a concept that peak bone mass should be increased in childhood as much as possible for the prevention of osteoporosis. Under such background, we have developed a new bean snack with an aim to improve bone volume loss. In this study, we examined the effects of a newly developed snack on bone volume and density in osteoporosis model mice. Methods Orchiectomy (ORX and ovariectomy (OVX were performed for C57BL/6J mice of twelve-week-old (Jackson Laboratory, Bar Harbar, ME, USA were used in this experiment. We prepared and given three types of powder diet e.g.: normal calcium diet (NCD, Ca: 0.9%, Clea Japan Co., Tokyo, Japan, low calcium diet (LCD, Ca: 0.63%, Clea Japan Co., and special diet (SCD, Ca: 0.9%. Eighteen weeks after surgery, all the animals were sacrified and prepared for histomorphometric analysis to quantify bone density and bone mineral content. Results As a result of histomorphometric examination, SCD was revealed to enhance bone volume irrespective of age and sex. The bone density was increased significantly in osteoporosis model mice fed the newly developmental snack as compared with the control mice. The bone mineral content was also enhanced significantly. These phenomena were revealed in both sexes. Conclusion It is shown that the newly developed bean snack is highly effective for the improvement of bone volume loss irrespective of sex. We demonstrated that newly developmental snack supplements may be a useful preventive measure for Japanese whose bone mineral density values are less than the ideal condition.

  10. Bone scintiscanning updated.

    Science.gov (United States)

    Lentle, B C; Russell, A S; Percy, J S; Scott, J R; Jackson, F I

    1976-03-01

    Use of modern materials and methods has given bone scintiscanning a larger role in clinical medicine, The safety and ready availability of newer agents have led to its greater use in investigating both benign and malignant disease of bone and joint. Present evidence suggests that abnormal accumulation of 99mTc-polyphosphate and its analogues results from ionic deposition at crystal surfaces in immature bone, this process being facilitated by an increase in bone vascularity. There is, also, a component of matrix localization. These factors are in keeping with the concept that abnormal scintiscan sites represent areas of increased osteoblastic activity, although this may be an oversimplification. Increasing evidence shows that the bone scintiscan is more sensitive than conventional radiography in detecting focal disease of bone, and its ability to reflect the immediate status of bone further complements radiographic findings. The main limitation of this method relates to nonspecificity of the results obtained.

  11. Bone marrow edema of the knee joint

    International Nuclear Information System (INIS)

    Breitenseher, M.J.; Mayerhoefer, M.E.; Hofmann, S.

    2006-01-01

    Bone marrow edema of the knee joint is a frequent clinical picture in MR diagnostics. It can be accompanied by symptoms and pain in the joint. Diseases that are associated with bone marrow edema can be classified into different groups. Group 1 includes vascular ischemic bone marrow edema with osteonecrosis (synonyms: SONK or Ahlbaeck's disease), osteochondrosis dissecans, and bone marrow edema syndrome. Group 2 comprises traumatic or mechanical bone marrow edema. Group 3 encompasses reactive bone marrow edemas such as those occurring in gonarthrosis, postoperative bone marrow edemas, and reactive edemas in tumors or tumorlike diseases. Evidence for bone marrow edema is effectively provided by MRI, but purely morphological MR information is often unspecific so that anamnestic and clinical details are necessary in most cases for definitive disease classification. (orig.) [de

  12. β-Glucans (Saccharomyces cereviseae) Reduce Glucose Levels and Attenuate Alveolar Bone Loss in Diabetic Rats with Periodontal Disease

    Science.gov (United States)

    2015-01-01

    The objective of this study was to assess the effects of oral ingestion of β-glucans isolated from Saccharomyces cereviseae on the metabolic profile, expression of gingival inflammatory markers and amount of alveolar bone loss in diabetic rats with periodontal disease. Diabetes mellitus was induced in 48 Wistar rats by intraperitoneal injection of streptozotocin (80 mg/kg). After confirming the diabetes diagnosis, the animals were treated with β-glucans (by gavage) for 28 days. On the 14th day of this period, periodontal disease was induced using a ligature protocol. β-glucans reduced the amount of alveolar bone loss in animals with periodontal disease in both the diabetic and non-diabetic groups (p periodontal disease (p periodontal disease (p periodontal effects in diabetic rats with periodontal disease. PMID:26291983

  13. Detection of active alveolar bone destruction in human periodontal disease by analysis of radiopharmaceutical uptake after a single injection of 99m-Tc-methylene diphosphonate

    Energy Technology Data Exchange (ETDEWEB)

    Jeffcoat, M.K.; Williams, R.C.; Holman, B.L.; English, R.; Goldhaber, P.

    1986-01-01

    Previous studies have shown that, following a single injection of 99m-Tc-MDP, measurement of bone-seeking radiopharmaceutical uptake can detect ''active'' alveolar bone loss due to periodontal disease in beagle dogs, as determined by radiographs taken at the time of, and several months after, the nuclear medicine procedure. The efficacy of this diagnostic test, however, had not been assessed in human periodontal disease. The ability of a single boneseeking radiopharmaceutical uptake examination to detect ''active'' alveolar bone loss due to periodontal disease in human patients was assessed by comparing a single uptake measurement to the rate of bone loss determined from serial radiographs taken over a 6-month period. Uptake was expressed as a ratio of the cpm from the alveolar bone divided by the cpm from the non-tooth supporting bone of the nuchal crest. High uptake ratios were associated with ''active'' loss and low uptake ratios were associated with little if any change in alveolar bone height (p<0.001). The nuclear medicine examination was an accurate detector of periodontal disease activity in nearly 80% of the individual teeth studied. These data indicate that high bone-seeking radiopharmaceutical uptake ratios may be pathognomonic of active bone loss in human periodontal disease.

  14. Osteopenia (metabolic bone disease) of prematurity

    Science.gov (United States)

    Osteopenia is defined as postnatal bone mineralization that is inadequate to fully mineralize bones. Osteopenia occurs commonly in very low birth weight (VLBW) infants. Prior to the use of high-mineral containing diets for premature infants, which is the current practice, significant radiographic ch...

  15. Developmental plasticity: Friend or foe?

    Science.gov (United States)

    Michels, Karin B

    2017-01-01

    Developmental plasticity - the concept that adaptation to changing and unfavorable environmental conditions are possible but may come at the price of compromised health potentials - has evolutionary grounding as it facilitates survival but dissents with fundamental evolutionary principles in that it may advance the lesser fit. It is an important cornerstone of the Developmental Origins of Health and Disease (DOHaD). Unlike evolutionary adaptation developmental plasticity may be short-lived and restricted to one or few generations and inheritance is uncertain. Potential mechanisms include epigenetic modifications adopted in utero which may not transmit to the next generation; future insights may allow adjustments of the outcomes of developmental plasticity.

  16. MRI bone marrow findings in 63 patients with type I Gaucher's disease

    International Nuclear Information System (INIS)

    Poll, L.W.; Willers, R.; Haeussinger, D.; Moedder, U.; Dahl, S. vom

    2010-01-01

    Purpose: To determine whether MR bone marrow findings in Gaucher patients may help to identify patients at high risk of developing severe Gaucher bone complications exemplified by avascular necrosis (AVN) of the femoral head. Materials and Methods: MR images were obtained in 63 Type I Gaucher patients through a standard protocol using coronal T1 and T2-weighted sequences of the lower extremities. The location and extent of infiltrated marrow was established using a semi-quantitative MRI scoring method (Duesseldorf Gaucher score, DGS) and the morphological pattern of bone marrow involvement determined (whether homogeneous type A or non-homogeneous type B). The active marrow process with bone edema and AVN of the femoral head were also analyzed. Results: Bone marrow involvement was observed in femoral sites more than in tibial sites. A high DGS was significantly correlated with type B morphology and femoral AVN (both p < 0.0001). Splenectomized patients showed a significantly higher Duesseldorf Gaucher score and type B morphology than non-splenectomized patients (both p < 0.05). AVN was seen in 46 % of patients with type B morphology versus 3 % in type A morphology (p < 0.0001). DGS and morphology of bone marrow involvement were not significantly correlated with active marrow processes. Conclusion: Type B marrow morphology and extensive marrow packing were significantly associated with AVN of the femoral head (both p < 0.0001). These patterns are considered predictive and may be employed in a disease management context to alert physicians to the need for urgent therapeutic measures. (orig.)

  17. Elevated Levels of Peripheral Kynurenine Decrease Bone Strength in Rats with Chronic Kidney Disease

    Directory of Open Access Journals (Sweden)

    Bartlomiej Kalaska

    2017-10-01

    Full Text Available The diagnosis and treatment of bone disorders in patients with chronic kidney disease (CKD represent a clinical challenge. CKD leads to mineral and bone complications starting early in the course of renal failure. Recently, we have observed the positive relationship between intensified central kynurenine turnover and bone strength in rats with subtotal 5/6 nephrectomy (5/6 Nx-induced CKD. The aim of the present study was to determine the association between peripheral kynurenine pathway metabolites and bone strength in rats with 5/6 Nx-induced CKD. The animals were sacrificed 1 and 3 months after 5/6 Nx or sham operation. Nephrectomized rats presented higher concentrations of serum creatinine, urea nitrogen, and parathyroid hormone both 1 and 3 months after nephrectomy. These animals revealed higher concentrations of kynurenine and 3-hydroxykynurenine in the serum and higher gene expression of aryl hydrocarbon receptor (AhR as a physiological receptor for kynurenine and AhR-dependent cytochrome in the bone tissue. Furthermore, nephrectomy significantly increased the number of osteoclasts in the bone without affecting their resorptive activity measured in serum. These changes were particularly evident in rats 1 month after 5/6 Nx. The main bone biomechanical parameters of the tibia were unchanged between nephrectomized and sham-operated rats but were significantly increased in older compared to younger animals. A similar trend was observed for geometrical parameters measured with calipers, bone mineral density based on Archimedes' method and image of bone microarchitecture obtained from micro-computed tomography analyses of tibial cortical bone. In nephrectomized animals, peripheral kynurenine levels correlated negatively with the main parameters of bone biomechanics, bone geometry, and bone mineral density values. In conclusion, our data suggest that CKD-induced elevated levels of peripheral kynurenine cause pathological changes in bone

  18. [Bone Cell Biology Assessed by Microscopic Approach. Bone histomorphometry of remodeling, modeling and minimodeling].

    Science.gov (United States)

    Yamamoto, Noriaki; Shimakura, Taketoshi; Takahashi, Hideaki

    2015-10-01

    Bone histomorphometry is defined as a quantitative evaluation of bone remodeling. In bone remodeling, bone resorption and bone formation are coupled with scalloped cement lines. Another mechanism of bone formation is minimodeling which bone formation and resorption are independent. The finding of minimodeling appeared in special condition with metabolic bone disease or anabolic agents. We need further study for minimodeling feature and mechanism.

  19. The importance of vitamins D and K for the bone health and immune function in inflammatory bowel disease.

    Science.gov (United States)

    Iijima, Hideki; Shinzaki, Shinichiro; Takehara, Tetsuo

    2012-11-01

    This review summarizes the recent literature about the roles of vitamins D and K in bone metabolism and immunity-mediated inflammatory processes in inflammatory bowel diseases (IBDs). The levels of vitamins D and K are lower than normal in patients with IBD, especially in Crohn's disease. Although vitamins D and K are important for the maintenance of bone mineral density in non-IBD patients, an association between vitamins D or K and bone metabolism is not apparent in IBD patients. Recent studies showed that vitamins D and K are suggested to have immune-suppressive effects, both in animal models of colitis and human trials. In particular, vitamin D suppresses dendritic and T-cell functions by inhibiting the production of proinflammatory cytokines. Insufficiency of vitamin D is associated with the activated phenotype of IBD. Vitamins D and K potentially contribute to the maintenance of bone health in IBD, but this effect may be diminished by other factors such as steroid use, reduced exposure to sunlight, and inflammatory cytokines. Vitamin D and possibly vitamin K are suggested to be involved in the suppression of immune-mediated inflammation and modulation of disease activity.

  20. Aplastic Anemia and MDS International Foundation (AAMDSIF): Bone marrow failure disease scientific symposium 2016.

    Science.gov (United States)

    Zeidan, Amer M; Battiwalla, Minoo; Berlyne, Deborah; Winkler, Thomas

    2017-02-01

    Patients with acquired and inherited bone marrow failure syndromes (BMFS) have ineffective hematopoiesis due to impairments of the hematopoietic stem cell compartment. Common manifestations of BMFS include varying degrees of peripheral blood cytopenias and, sometimes, progression to acute myelogenous leukemia. Research efforts have been made all over the world to improve understanding of the pathogenesis of these diseases and their clinical implications. The Aplastic Anemia and MDS International Foundation (AAMDSIF) is an independent nonprofit organization whose mission is to help patients and family members cope with BMFS. Here, we summarize recent scientific discoveries in several BMFS that were presented at the fifth International Bone Marrow Failure Disease Scientific Symposium 2016 that AAMDSIF sponsored on March 17-18, 2016, in Rockville, Maryland. Copyright © 2016 Elsevier Ltd. All rights reserved.

  1. Sickle Cell Disease with Cyanotic Congenital Heart Disease: Long-Term Outcomes in 5 Children.

    Science.gov (United States)

    Iannucci, Glen J; Adisa, Olufolake A; Oster, Matthew E; McConnell, Michael; Mahle, William T

    2016-12-01

    Sickle cell disease is a risk factor for cerebrovascular accidents in the pediatric population. This risk is compounded by hypoxemia. Cyanotic congenital heart disease can expose patients to prolonged hypoxemia. To our knowledge, the long-term outcome of patients who have combined sickle cell and cyanotic congenital heart disease has not been reported. We retrospectively reviewed patient records at our institution and identified 5 patients (3 girls and 2 boys) who had both conditions. Their outcomes were uniformly poor: 4 died (age range, 12 mo-17 yr); 3 had documented cerebrovascular accidents; and 3 developed ventricular dysfunction. The surviving patient had developmental delays. On the basis of this series, we suggest mitigating hypoxemia, and thus the risk of stroke, in patients who have sickle cell disease and cyanotic congenital heart disease. Potential therapies include chronic blood transfusions, hydroxyurea, earlier surgical correction to reduce the duration of hypoxemia, and heart or bone marrow transplantation.

  2. Systemic mast cell disease (SMCD) and bone pain. A case treated with radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Hesselmann, S.; Micke, O.; Schaefer, U.; Willich, N. [University Hospital Muenster (Germany). Dept. of Radiotherapy and Radiooncology

    2002-05-01

    Background: Systemic mast cell disease (SMCD) is a rare disease characterized by a multitopic proliferation of cytologically and/or functionally abnormal tissue mast cells. SMCD preferentially involves the skin, spleen, liver, lymph nodes and the bone marrow. The cause of SMCD is unknown. Bony pain, caused by mast cell infiltration of the marrow cavity, is present in up to 28% of cases and is frequently chronic and difficult to palliate with medical therapy. Case Report: We report one case of refractory bone pain in a 54-year-old female Caucasian patient with advanced SMCD and associated bony involvement, which was treated with radiotherapy for pain palliation. Between 1995 and 1998, the patient was irradiated at four different locations: 1) right shoulder and proximal right humerus, 2) both hands, 3) both knees, 4) left humerus with a total dose of 40 Gy in 2.0 or 2.5 Gy daily fractions. Results: Different results of pain palliation were achieved. In one location the pain was reduced for 55 months until her death due to disease progression, whereas in two other locations a pain control was maintained for 3 and 6 months after radiotherapy. In one location, no pain reduction was achieved. Severe side effects were not observed. Conclusion: Palliative radiotherapy has a role in the control of severe intractable bone pain in patients with advanced SMCD, though in some cases the effect may be short or incomplete. The observed palliation of pain can even differ in the same patient. (orig.)

  3. Systemic mast cell disease (SMCD) and bone pain. A case treated with radiotherapy

    International Nuclear Information System (INIS)

    Hesselmann, S.; Micke, O.; Schaefer, U.; Willich, N.

    2002-01-01

    Background: Systemic mast cell disease (SMCD) is a rare disease characterized by a multitopic proliferation of cytologically and/or functionally abnormal tissue mast cells. SMCD preferentially involves the skin, spleen, liver, lymph nodes and the bone marrow. The cause of SMCD is unknown. Bony pain, caused by mast cell infiltration of the marrow cavity, is present in up to 28% of cases and is frequently chronic and difficult to palliate with medical therapy. Case Report: We report one case of refractory bone pain in a 54-year-old female Caucasian patient with advanced SMCD and associated bony involvement, which was treated with radiotherapy for pain palliation. Between 1995 and 1998, the patient was irradiated at four different locations: 1) right shoulder and proximal right humerus, 2) both hands, 3) both knees, 4) left humerus with a total dose of 40 Gy in 2.0 or 2.5 Gy daily fractions. Results: Different results of pain palliation were achieved. In one location the pain was reduced for 55 months until her death due to disease progression, whereas in two other locations a pain control was maintained for 3 and 6 months after radiotherapy. In one location, no pain reduction was achieved. Severe side effects were not observed. Conclusion: Palliative radiotherapy has a role in the control of severe intractable bone pain in patients with advanced SMCD, though in some cases the effect may be short or incomplete. The observed palliation of pain can even differ in the same patient. (orig.)

  4. Optimising diffusion weighted MRI for imaging metastatic and myeloma bone disease and assessing reproducibility

    International Nuclear Information System (INIS)

    Messiou, C.; Collins, D.J.; Morgan, V.A.; DeSouza, N.M.

    2011-01-01

    To establish normal bone marrow values of apparent diffusion coefficient (ADC) over an age range, compare them with metastatic and myelomatous involvement, to establish reproducibility and to optimise b values. The ADCs of bone marrow in 7 volunteers (mean age 29.7 years), 34 volunteers (mean age 63.3 years) and 43 patients with metastatic and myelomatous involvement (mean age 65.5 years) were measured. In 9 volunteers diffusion weighted MRI was repeated within 7 days. b values were derived to optimise contrast between normal and pathological marrow. The mean ADC of bone marrow in younger volunteers was significantly higher than that of older volunteers. The coefficient of reproducibility was 14.8%. The ADC mean of metastatic and myeloma bone disease was 1054+/-456 x 10 -6 mm 2 s -1 . An ADC threshold of 655 x 10 -6 mm 2 s -1 separated normal and abnormal marrow with a sensitivity and specificity of 90% and 93% respectively. Contrast between normal and abnormal marrow was optimal at b = 1389 smm -2 . The reproducibility of ADC measurements in bone is equivalent to published data for soft tissue with a high sensitivity and specificity for separating abnormal from age matched normal bone marrow. A b value of around 1,400 smm -2 is optimal for imaging bone marrow. (orig.)

  5. Optimising diffusion weighted MRI for imaging metastatic and myeloma bone disease and assessing reproducibility

    Energy Technology Data Exchange (ETDEWEB)

    Messiou, C. [Institute of Cancer Research and Royal Marsden, NHS Foundation Trust, Cancer Research UK Clinical Magnetic Resonance Research Group, Surrey (United Kingdom); Institute of Cancer Research and Royal Marsden, NHS Foundation Trust, MRI Department, Surrey (United Kingdom); Collins, D.J.; Morgan, V.A.; DeSouza, N.M. [Institute of Cancer Research and Royal Marsden, NHS Foundation Trust, Cancer Research UK Clinical Magnetic Resonance Research Group, Surrey (United Kingdom)

    2011-08-15

    To establish normal bone marrow values of apparent diffusion coefficient (ADC) over an age range, compare them with metastatic and myelomatous involvement, to establish reproducibility and to optimise b values. The ADCs of bone marrow in 7 volunteers (mean age 29.7 years), 34 volunteers (mean age 63.3 years) and 43 patients with metastatic and myelomatous involvement (mean age 65.5 years) were measured. In 9 volunteers diffusion weighted MRI was repeated within 7 days. b values were derived to optimise contrast between normal and pathological marrow. The mean ADC of bone marrow in younger volunteers was significantly higher than that of older volunteers. The coefficient of reproducibility was 14.8%. The ADC mean of metastatic and myeloma bone disease was 1054+/-456 x 10{sup -6} mm{sup 2}s{sup -1}. An ADC threshold of 655 x 10{sup -6} mm{sup 2}s{sup -1} separated normal and abnormal marrow with a sensitivity and specificity of 90% and 93% respectively. Contrast between normal and abnormal marrow was optimal at b = 1389 smm{sup -2}. The reproducibility of ADC measurements in bone is equivalent to published data for soft tissue with a high sensitivity and specificity for separating abnormal from age matched normal bone marrow. A b value of around 1,400 smm{sup -2} is optimal for imaging bone marrow. (orig.)

  6. The Presence of Thyroid-Stimulation Blocking Antibody Prevents High Bone Turnover in Untreated Premenopausal Patients with Graves' Disease.

    Science.gov (United States)

    Cho, Sun Wook; Bae, Jae Hyun; Noh, Gyeong Woon; Kim, Ye An; Moon, Min Kyong; Park, Kyoung Un; Song, Junghan; Yi, Ka Hee; Park, Do Joon; Chung, June-Key; Cho, Bo Youn; Park, Young Joo

    2015-01-01

    Osteoporosis-related fractures are one of the complications of Graves' disease. This study hypothesized that the different actions of thyroid-stimulating hormone receptor (TSHR) antibodies, both stimulating and blocking activities in Graves' disease patients might oppositely impact bone turnover. Newly diagnosed premenopausal Graves' disease patients were enrolled (n = 93) and divided into two groups: patients with TSHR antibodies with thyroid-stimulating activity (stimulating activity group, n = 83) and patients with TSHR antibodies with thyroid-stimulating activity combined with blocking activity (blocking activity group, n = 10). From the stimulating activity group, patients who had matched values for free T4 and TSH binding inhibitor immunoglobulin (TBII) to the blocking activity group were further classified as stimulating activity-matched control (n = 11). Bone turnover markers BS-ALP, Osteocalcin, and C-telopeptide were significantly lower in the blocking activity group than in the stimulating activity or stimulating activity-matched control groups. The TBII level showed positive correlations with BS-ALP and osteocalcin levels in the stimulating activity group, while it had a negative correlation with the osteocalcin level in the blocking activity group. In conclusion, the activation of TSHR antibody-activated TSH signaling contributes to high bone turnover, independent of the actions of thyroid hormone, and thyroid-stimulation blocking antibody has protective effects against bone metabolism in Graves' disease.

  7. The Presence of Thyroid-Stimulation Blocking Antibody Prevents High Bone Turnover in Untreated Premenopausal Patients with Graves' Disease.

    Directory of Open Access Journals (Sweden)

    Sun Wook Cho

    Full Text Available Osteoporosis-related fractures are one of the complications of Graves' disease. This study hypothesized that the different actions of thyroid-stimulating hormone receptor (TSHR antibodies, both stimulating and blocking activities in Graves' disease patients might oppositely impact bone turnover. Newly diagnosed premenopausal Graves' disease patients were enrolled (n = 93 and divided into two groups: patients with TSHR antibodies with thyroid-stimulating activity (stimulating activity group, n = 83 and patients with TSHR antibodies with thyroid-stimulating activity combined with blocking activity (blocking activity group, n = 10. From the stimulating activity group, patients who had matched values for free T4 and TSH binding inhibitor immunoglobulin (TBII to the blocking activity group were further classified as stimulating activity-matched control (n = 11. Bone turnover markers BS-ALP, Osteocalcin, and C-telopeptide were significantly lower in the blocking activity group than in the stimulating activity or stimulating activity-matched control groups. The TBII level showed positive correlations with BS-ALP and osteocalcin levels in the stimulating activity group, while it had a negative correlation with the osteocalcin level in the blocking activity group. In conclusion, the activation of TSHR antibody-activated TSH signaling contributes to high bone turnover, independent of the actions of thyroid hormone, and thyroid-stimulation blocking antibody has protective effects against bone metabolism in Graves' disease.

  8. Radioimmune imaging of bone marrow in patients with suspected bone metastases from primary breast cancer

    International Nuclear Information System (INIS)

    Duncker, C.M.; Carrio, I.; Berna, L.; Estorch, M.; Alonso, C.; Ojeda, B.; Blanco, R.; Germa, J.R.; Ortega, V.

    1990-01-01

    Radioimmune imaging of bone marrow was performed by technetium-99m- (99mTc) labeled antigranulocyte monoclonal antibody BW 250/183 (AGMoAb) scans in 32 patients with suspected bone metastases from primary breast cancer. AGMoAb scans showed bone marrow defects in 25/32 (78%) patients; bone invasion was subsequently confirmed in 23 (72%) patients. Conventional bone scans performed within the same week detected bone metastases in 17/32 (53%) patients (p less than 0.001). AGMoAb scans detected more sites indicating metastatic disease than bone scans in 12 of these 17 patients (71%). All patients with bone metastases in the axial skeleton had bone marrow defects at least at the sites of bone metastases. Of 15 patients with normal, or indicative of, benign disease bone scans, 8 patients (53%) presented with bone marrow defects in the AGMoAb scans. Bone invasion was confirmed in six of them. AGMoAb bone marrow scans provide a method for the early detection of bone metastatic invasion in patients with breast cancer and suspected bone metastases

  9. Bone metabolism and arterial stiffness after renal transplantation.

    Science.gov (United States)

    Cseprekál, Orsolya; Kis, Eva; Dégi, Arianna A; Kerti, Andrea; Szabó, Attila J; Reusz, György S

    2014-01-01

    To assess the relationship between bone and vascular disease and its changes over time after renal transplantation. Metabolic bone disease (MBD) is common in chronic kidney disease (CKD) and is associated with cardiovascular (CV) disease. Following transplantation (Tx), improvement in CV disease has been reported; however, data regarding changes in bone disease remain controversial. Bone turnover and arterial stiffness (pulse wave velocity (PWV)) were assessed in 47 Tx patients (38 (3-191) months after Tx). Bone alkaline phosphatase (BALP), osteocalcin (OC) and beta-crosslaps were significantly higher in Tx patients, and decreased significantly after one year. There was a negative correlation between BALP, OC and steroid administered (r = -0.35; r = -0.36 respectively). PWV increased in the Tx group (1.15 SD). In patients with a follow up of bone turnover and arterial stiffness are present following kidney transplantation. While bone turnover decreases with time, arterial stiffness correlates initially with bone turnover, after which the influence of cholesterol becomes significant. Non-invasive estimation of bone metabolism and arterial stiffness may help to assess CKD-MBD following renal transplantation.

  10. Improved MDCT monitoring of pelvic myeloma bone disease through the use of a novel longitudinal bone subtraction post-processing algorithm

    Energy Technology Data Exchange (ETDEWEB)

    Horger, Marius; Thaiss, Wolfgang M.; Nikolaou, Konstantin; Kloth, Christopher [Eberhard-Karls-University Tuebingen, Department of Diagnostic and Interventional Radiology, Tuebingen (Germany); Ditt, Hendrik [Sector Imaging and Interventional Radiology, Siemens AG Healthcare, Forchheim (Germany); Weisel, Katja [Eberhard-Karls-University Tuebingen, Department of Internal Medicine II, Tuebingen (Germany); Fritz, Jan [Johns Hopkins University School of Medcine, Russell H. Morgan Department of Radiology and Radiological Science, Baltimore, MD (United States); Liao, Shu [Siemens Medical Solutions, Malvern, PA (United States)

    2017-07-15

    To evaluate the diagnostic performance of a novel CT post-processing software that generates subtraction maps of baseline and follow-up CT examinations in the course of myeloma bone lesions. This study included 61 consecutive myeloma patients who underwent repeated whole-body reduced-dose MDCT at our institution between November 2013 and June 2015. CT subtraction maps classified a progressive disease (PD) vs. stable disease (SD)/remission. Bone subtraction maps (BSMs) only and in combination with 1-mm (BSM+) source images were compared with 5-mm axial/MPR scans. Haematological response categories at follow-up were: complete remission (n = 9), very good partial remission (n = 2), partial remission (n = 17) and SDh (n = 19) vs. PDh (n = 14). Five-millimetre CT scan yielded PD (n = 14) and SD/remission (n = 47) whereas bone subtraction + 1-mm axial scans (BSM+) reading resulted in PD (n = 18) and SD/remission (n = 43). Sensitivity/ specificity/accuracy for 5-mm/1-mm/BSM(alone)/BSM + in ''lesion-by-lesion'' reading was 89.4 %/98.9 %/98.3 %/ 99.5 %; 69.1 %/96.9 %/72 %/92.1 % and 83.8 %/98.4 %/92.1 %/98.3 %, respectively. The use of BSM+ resulted in a change of response classification in 9.8 % patients (n = 6) from SD to PD. BSM reading is more accurate for monitoring myeloma compared to axial scans whereas BSM+ yields similar results with 1-mm reading (gold standard) but by significantly reduced reading time. (orig.)

  11. Improved MDCT monitoring of pelvic myeloma bone disease through the use of a novel longitudinal bone subtraction post-processing algorithm

    International Nuclear Information System (INIS)

    Horger, Marius; Thaiss, Wolfgang M.; Nikolaou, Konstantin; Kloth, Christopher; Ditt, Hendrik; Weisel, Katja; Fritz, Jan; Liao, Shu

    2017-01-01

    To evaluate the diagnostic performance of a novel CT post-processing software that generates subtraction maps of baseline and follow-up CT examinations in the course of myeloma bone lesions. This study included 61 consecutive myeloma patients who underwent repeated whole-body reduced-dose MDCT at our institution between November 2013 and June 2015. CT subtraction maps classified a progressive disease (PD) vs. stable disease (SD)/remission. Bone subtraction maps (BSMs) only and in combination with 1-mm (BSM+) source images were compared with 5-mm axial/MPR scans. Haematological response categories at follow-up were: complete remission (n = 9), very good partial remission (n = 2), partial remission (n = 17) and SDh (n = 19) vs. PDh (n = 14). Five-millimetre CT scan yielded PD (n = 14) and SD/remission (n = 47) whereas bone subtraction + 1-mm axial scans (BSM+) reading resulted in PD (n = 18) and SD/remission (n = 43). Sensitivity/ specificity/accuracy for 5-mm/1-mm/BSM(alone)/BSM + in ''lesion-by-lesion'' reading was 89.4 %/98.9 %/98.3 %/ 99.5 %; 69.1 %/96.9 %/72 %/92.1 % and 83.8 %/98.4 %/92.1 %/98.3 %, respectively. The use of BSM+ resulted in a change of response classification in 9.8 % patients (n = 6) from SD to PD. BSM reading is more accurate for monitoring myeloma compared to axial scans whereas BSM+ yields similar results with 1-mm reading (gold standard) but by significantly reduced reading time. (orig.)

  12. Prevalence of Paget's disease of bone in Italy.

    Science.gov (United States)

    Gennari, Luigi; Di Stefano, Marco; Merlotti, Daniela; Giordano, Nicola; Martini, Giuseppe; Tamone, Cristina; Zatteri, Roberto; De Lucchi, Roberto; Baldi, Carlo; Vattimo, Angelo; Capoccia, Silvia; Burroni, Luca; Geraci, Simone; De Paola, Vincenzo; Calabrò, Anna; Avanzati, Annalisa; Isaia, Giancarlo; Nuti, Ranuccio

    2005-10-01

    We examined the prevalence of PDB in Italy from radiological, scintigraphic, and biochemical surveys in two Italian towns. Prevalence rates varied from 0.7% to 2.4%, were higher in males than in females, and slightly differed between the two towns. Unlike previous studies in populations of British descent, no secular trend for a decreasing prevalence emerged. Clinical, radiological, and necropsy data from different countries suggested pronounced geographical variations in the prevalence of Paget's disease of bone (PDB). Despite the impact of the disease on the population, there are limited data on the prevalence of PDB in Italy. The objective of this study was to estimate the prevalence of PDB in the district of Siena (Central Italy) and Turin (Northern Italy) from radiological, biochemical, and scintigraphic surveys. We examined a sample of 1778 consecutive pelvic radiographs performed between 1999 and 2000 at the Hospital Radiology Unit in Siena and 6609 pelvic radiographs performed in 1986-1987, 1992-1993, and 1999-2002 from the Radiology Department of Molinette Hospital in Turin. In Siena, 7906 consecutive (99m)TC-MDP bone scans performed over a 4-year period (January 2000 to May 2004) were also screened for the presence of PDB, and the prevalence of elevated alkaline phosphatase (ALP) levels (>300 UI/liter) was estimated from 7449 computerized medical records over a 3-year period (January 2000 to February 2003). The finding of PDB on the pelvic radiograph and bone scan was based on standardized radiological criteria. At the end of the radiological surveys, 16/1778 pelvic PDB cases (8 males and 8 females) were observed in Siena and 41/6609 (27 males and 14 females) in Turin. The crude prevalence of the disease was 0.89% in Siena and 0.62% in Turin. Given that pelvic involvement is commonly described in 60-90% of PDB patients, the estimated overall prevalence of PDB ranged from 1.0% to 1.5% in Siena and from 0.7% to 1.0% in Turin. No decrease in the prevalence

  13. Dioxin-induced up-regulation of the active form of vitamin D is the main cause for its inhibitory action on osteoblast activities, leading to developmental bone toxicity

    International Nuclear Information System (INIS)

    Nishimura, Noriko; Nishimura, Hisao; Ito, Tomohiro; Miyata, Chie; Izumi, Keiko; Fujimaki, Hidekazu; Matsumura, Fumio

    2009-01-01

    Dioxin (2,3,7,8-tetrachlorodibenzo-p-dioxin, TCDD) is known to cause bone toxicity, particularly during animal development, although its action mechanism to cause this toxicity has yet to be elucidated. Mouse pups were exposed to TCDD via dam's milk that were administered orally with 15 μg TCDD/kg b.w. on postnatal day 1. Here we report that TCDD causes up-regulation of vitamin D 1α-hydroxylase in kidney, resulting in a 2-fold increase in the active form of vitamin D, 1,25-dihydroxyvitamin D 3 , in serum. This action of TCDD is not caused by changes in parathyroid hormone, a decrease in vitamin D degrading enzyme, vitamin D 24-hydroxylase, or alterations in serum Ca 2+ concentration. Vitamin D is known to affect bone mineralization. Our data clearly show that TCDD-exposed mice exhibit a marked decrease in osteocalcin and collagen type 1 as well as alkaline phosphatase gene expression in tibia by postnatal day 21, which is accompanied with a mineralization defect in the tibia, lowered activity of osteoblastic bone formation, and an increase in fibroblastic growth factor-23, a sign of increased vitamin D effect. Despite these significant effects of TCDD on osteoblast activities, none of the markers of osteoclast activities was found to be affected. Histomorphometry confirmed that osteoblastic activity, but not bone resorption activity, was altered by TCDD. A prominent lesion commonly observed in these TCDD-treated mice was impaired bone mineralization that is characterized by an increased volume and thickness of osteoids lining both the endosteum of the cortical bone and trabeculae. Together, these data suggest that the impaired mineralization resulting from reduction of the osteoblastic activity, which is caused by TCDD-induced up-regulation of vitamin D, is responsible for its bone developmental toxicity.

  14. Instrumental activation and X-ray fluorescent analysis of human bone in health and disease

    International Nuclear Information System (INIS)

    Zaichick, V.Y.

    1994-01-01

    A complex of methods for the in-vitro and in-vivo bone analysis was developed. Among the in-vitro methods are: INAA with reactor and 14 MeV neutrons, IGAA with 25 MeV linear accelerator; XRF with 55 Fe, 109 Cd, 241 Am radionuclide sources. Twenty-five elements could be analyzed by it: N, F, Na, Mg, P, Cl, K, Ca, Sc, Cr, Mn, Fe, Co, Zn, Se, Br, Rb, Sr, Ag, Sb, Cs, Ba, Tb, Hg, and Pb. Among the in-vivo methods are: INAA of band, foot and spine Ca and limb bone tumour Ca, Na and Cl with 238 Pu-Be neutron sources; IGAA of N and P in limb bone tumours; XRF of tooth Ca, Zn, Sr and Pb with 109 Cs radionuclide sources. The methods developed were used both in clinical and experimental medicine for studying the healthy human and animal bone with different diseases and environmental influence. (author) 28 refs.; 7 tabs

  15. Comparison of two methods for alveolar bone loss measurement in an experimental periodontal disease model in rats

    Directory of Open Access Journals (Sweden)

    Diego Nique Liberman

    2011-02-01

    Full Text Available There are many studies that evaluate possible risk factors for periodontal diseases in animals. Most of them have focused only on the biological aspects of disease occurrence; therefore, it has been difficult to compare studies of the different methodological approaches. The aim of the present study was to compare different methods - linear and area - of the evaluation of morphometrical alveolar bone loss. Sixty hemimaxillae, defleshed and stained with 1% methylene blue to delineate the cementoenamel junction and alveolar bone crest, were obtained from a previous study that induced periodontal disease by means of ligatures in two groups of fifteen Wistar rats during 9 weeks. Ligatures were placed around the right upper second molars, and the contra-lateral teeth remained as intra-group controls. Digital photographs were taken from the specimens and submitted to a single, calibrated, blind examiner who performed the morphometrical evaluation of alveolar bone loss using both linear and area methods. Mean values of linear and area measurements were obtained from each side - buccal and palatal - of the specimens. The degree of association between the two methods was determined by Pearson's Correlation Coefficient. An almost perfect association (0.98 was determined between the linear and area evaluations. A mathematical formula was subsequently created to estimate the total area of alveolar bone loss, from linear mean measurements. Both methods were suitable for detecting bone level alterations. The results of the present study allow for the transformation of data and better compilation of results from different studies.

  16. Prospective assessment of bone turnover and clinical bone diseases after allogeneic hematopoietic stem-cell transplantation.

    Science.gov (United States)

    Petropoulou, Anna D; Porcher, Raphael; Herr, Andrée-Laure; Devergie, Agnès; Brentano, Thomas Funck; Ribaud, Patricia; Pinto, Fernando O; Rocha, Vanderson; Peffault de Latour, Régis; Orcel, Philippe; Socié, Gérard; Robin, Marie

    2010-06-15

    Bone complications after hematopoietic stem-cell transplantation (HSCT) are relatively frequent. Evaluation of biomarkers of bone turnover and dual energy x-ray absorptiometry (DEXA) are not known in this context. We prospectively evaluated bone mineral density, biomarkers of bone turnover, and the cumulative incidence of bone complications after allogeneic HSCT. One hundred forty-six patients were included. Bone mineral density was measured by DEXA 2-month and 1-year post-HSCT. The markers of bone turnover were serum C-telopeptide (C-TP), 5 tartrate-resistant acid phosphatase (bone resorption), and osteocalcin (bone formation) determined pre-HSCT and 2 months and 1 year thereafter. Potential association between osteoporosis at 2 months, osteoporotic fracture or avascular necrosis and, individual patient's characteristics and biologic markers were tested. C-TP was high before and 2 months after transplant. At 2 months, DEXA detected osteoporosis in more than half the patients tested. Male sex, median age less than or equal to 15 years, and abnormal C-TP before HSCT were risk factors significantly associated with osteoporosis. Three-year cumulative incidences of fractures and avascular necrosis were 8% and 11%, respectively. Children were at higher risk of fracture, whereas corticosteroid treatment duration was a significant risk factor for developing a clinical bone complication post-HSCT. Bone complications and osteoporosis are frequent after HSCT. Bone biologic markers and DEXA showed that subclinical bone abnormalities appeared early post-HSCT. The risk factors, age, gender, and C-TP easily available at the time of transplantation were identified. Biphosphonates should probably be given to patients with those risk factors.

  17. Use of dual energy X-ray absorptiometry, the trabecular bone score and quantitative computed tomography in the evaluation of chronic kidney disease-mineral and bone disorders.

    Science.gov (United States)

    Pocock, Nicholas

    2017-03-01

    In subjects with chronic kidney disease (CKD) who suffer a minimal trauma fracture, the problem is to differentiate between osteoporosis and the various forms of renal bone disease associated with CKD-mineral and bone disorder. This problem is exacerbated by the fact that renal osteodystrophy may coexist with osteoporosis. The World Health Organization's bone mineral density (BMD) criteria for osteopenia ( -2.5 < T-score < -1.0) and osteoporosis (a T-score ≤ -2.5) may be used in patients with CKD stages 1-3. In CKD stages 4-5, BMD by dual-energy X-ray absorptiometry (DXA) is less predictive and may underestimate fracture risk. The development of absolute fracture risk (AFR) algorithms, such as FRAX® and the Garvan absolute fracture risk calculator, to predict risk of fracture over a given time (usually 10 years) aims to incorporate non-BMD risk factors into the clinical assessment. FRAX® has been shown to be useful to assess fracture risk in CKD but may underestimate fracture risk in advanced CKD. The trabecular bone score is a measure of grey scale homogeneity obtained from spine DXA, which correlates to trabecular microarchitecture and is an independent risk factor for fracture. Recent data demonstrate the potential utility of the trabecular bone score adjustment of AFR through the FRAX® algorithm in subjects with CKD. Parameters of bone microarchitecture using peripheral quantitative computed tomography (pQCT) or high-resolution pQCT are also able to discriminate fracture status in subjects with CKD. However, there are at present no convincing data that the addition of pQCT or high-resolution pQCT parameters to DXA BMD improves fracture discrimination. More advanced estimates of bone strength derived from measurements of micro-architecture, by QCT-derived finite element analysis may be incorporated into AFR algorithms in the future. © 2017 Asian Pacific Society of Nephrology.

  18. Wormian bones in osteogenesis imperfecta and other disorders

    International Nuclear Information System (INIS)

    Cremin, B.; Goodman, H.; Spranger, J.; Beighton, P.

    1982-01-01

    When are Wormian bones significant is not an easy question to answer, but its relevance is important in relation to bone dysplasias such as osteogenesis imperfecta. Recognition will differ with age of patient, radiographic objectivity, and personal subjectivity. In order to attempt an answer, the skull radiographs of 81 cases of osteogenesis imperfecta of varying ages were examined for the presence of Wormian bones. These were compared against the incidence of Wormian bones in 500 skull radiographs of normal children. Significant Wormian bones as against normal developmental variants were considered to be those more than 10 in number, measuring greater than 6 mm by 4 mm, and arranged in a general mosaic pattern. They were found in all the cases of osteogenesis imperfecta but not in the normal skulls. The occurrence of significant Wormian bones in other bone dysplasias from our material and that of the literature was recorded. Other incidental findings in the skulls of the cases of osteogenesis imperfecta were also appraised. (orig.)

  19. Developmental anomaly of the hyoid bone: an unusual cause of dysphagia; Anomalie de developpement de l'os hyoide: une cause inhabituelle de dysphagie

    Energy Technology Data Exchange (ETDEWEB)

    Bayadjian, A.; Marsot-Dupuch, K.; Schmitt, E.; Chouard, Ch.; Tubiana, J.M. [Hopital Saint Antoine, 75 - Paris (France)

    2001-04-01

    We report the case of a hyoid syndrome caused by a developmental anomaly of the second branchial cleft, presenting at adult age by dysphagia without any abnormality detected at the barium swallow and at naso-pharyngeal endoscopy, first examinations to perform in case of dysphagia. The MRI findings of this anomaly showed a hyperintense well-limited vallecular mass syndrome. The diagnosis of hyoid bone anomaly was established at spiral CT with 3-D reconstructions showing an incurvated and elongated lesser cornua causing persistent impingement on the lateral wall of the oropharynx. CT scan performed during Valsalva maneuver showed the persistence of the compression during pharyngeal insufflation. (author)

  20. Meeting report - TGF-β superfamily: signaling in development and disease.

    Science.gov (United States)

    Zhang, Ying E; Newfeld, Stuart J

    2013-11-01

    The latest advances on the transforming growth factor β (TGF-β) and bone morphogenetic protein (BMP) signaling pathways were reported at the July 2013 FASEB Summer Research Conference 'The TGF-β Superfamily: Development and Disease'. The meeting was held in Steamboat Springs, Colorado, USA at 6700 feet above sea level in the Rocky Mountains. This was the seventh biannual meeting in the series. In attendance were investigators from a broad range of disciplines with a common interest in the mechanics of TGF-β and BMP signaling pathways, their normal developmental and homeostatic functions, and the diseases associated with pathway misregulation.

  1. Can bone loss be reversed by antithyroid drug therapy in premenopausal women with Graves' disease?

    Directory of Open Access Journals (Sweden)

    Belsing Tina Z

    2010-09-01

    Full Text Available Abstract Context Hyperthyroidism can lead to reduced bone mineral density (BMD and increased fracture risk particularly in postmenopausal women, but the mechanism behind is still unclear. Objective Prospective examination of the influence of thyroid hormones and/or thyroid autoantibodies on BMD in premenopause. Design We have examined 32 premenopausal women with untreated active Graves' disease from time of diagnosis, during 18 months of antithyroid drug therapy (ATD and additionally 18 months after discontinuing ATD. Variables of thyroid metabolism, calcium homeostasis and body composition were measured every 3 months. BMD of lumbar spine and femoral neck were measured at baseline, 18 ± 3 and 36 ± 3 months. Data were compared to base line, a sex- and age matched control group and a group of patients with Hashimoto's thyroiditis treated with non-suppressive doses of levothyroxine. Results The study showed significantly (p Conclusion The results indicated a clinically relevant impact of thyroid function on bone modulation also in premenopausal women with Graves' disease, and further indicated the possibility for a direct action of TRAb on bones.

  2. [Polymethylmethacrylate augmentation of bone cement-injectable cannulated pedicle screws for the treatment of degenerative lumbar diseases with osteoporosis].

    Science.gov (United States)

    Sun, H L; Li, C D; Yang, Z C; Yi, X D; Liu, H; Lu, H L; Li, H; Wang, Y

    2016-12-18

    To describe the application of polymethylmethacrylate augmentation of bone cement-injectable cannulated pedicle screws for the treatment of degenerative lumbar diseases with osteoporosis. Observation group included 14 cases of degenerative lumbar diseases with osteoporosis received polymethylmethacrylate augmentation of bone cement-injectable cannulated pedicle screws from November 2014 to July 2015, control group included 12 cases of degenerative lumbar diseases with osteoporosis received polymethylmethacrylate augmentation with traditional pedicle screws.The operation time, blood loss, number of pedicle screws and number of augmented pedicle screws in the two groups were compared. The bone cement leakage and pulmonary bone cement embolism in the two groups were also compared. The fusion rate and pedicle screws loosening by lumbar X ray and dynamic X ray were evaluated. The clinical results were assessed by visual analog scale (VAS) of pain on lumbar and lower limbers, lumbar Japanese Orthopaedic Association scores (JOA), Prolo functional scores and Oswestry disability (ODI) scores. Differences of operation time and blood loss in the two groups were not statistically significant. The average number of pedicle screws was 9.9±4.7 and the average number of augmented pedicle screws was 5.9±2.6 in observation group while the average number of pedicle screws was 7.1±2.8 and the average number of augmented pedicle screws was 3.0±1.9 in control group. The ratio of augmented pedicle screws was higher in observation group than in control group (0.69±0.30 vs.0.47±0.30,Pdegenerative lumbar diseases with osteoporosis was effective, with simple working processes and lower risk of bone cement leakage. The short-term clinical result was good.

  3. Use of lymphokine-activated killer cells to prevent bone marrow graft rejection and lethal graft-vs-host disease

    International Nuclear Information System (INIS)

    Azuma, E.; Yamamoto, H.; Kaplan, J.

    1989-01-01

    Prompted by our recent finding that lymphokine-activated killer (LAK) cells mediate both veto and natural suppression, we tested the ability of adoptively transferred LAK cells to block two in vivo alloreactions which complicate bone marrow transplantation: resistance to transplanted allogeneic bone marrow cells, and lethal graft-vs-host disease. Adoptive transfer of either donor type B6D2 or recipient-type B6 lymphokine-activated bone marrow cells, cells found to have strong LAK activity, abrogated or inhibited the resistance of irradiated B6 mice to both B6D2 marrow and third party-unrelated C3H marrow as measured by CFU in spleen on day 7. The ability of lymphokine-activated bone marrow cells to abrogate allogeneic resistance was eliminated by C lysis depletion of cells expressing asialo-GM1, NK1.1, and, to a variable degree, Thy-1, but not by depletion of cells expressing Lyt-2, indicating that the responsible cells had a LAK cell phenotype. Similar findings were obtained by using splenic LAK cells generated by 3 to 7 days of culture with rIL-2. Demonstration that allogeneic resistance could be blocked by a cloned LAK cell line provided direct evidence that LAK cells inhibit allogeneic resistance. In addition to inhibiting allogeneic resistance, adoptively transferred recipient-type LAK cells prevented lethal graft-vs-host disease, and permitted long term engraftment of allogeneic marrow. Irradiation prevented LAK cell inhibition of both allogeneic resistance and lethal graft-vs-host disease. These findings suggest that adoptive immunotherapy with LAK cells may prove useful in preventing graft rejection and graft-versus-host disease in human bone marrow transplant recipients

  4. Vitamin D, Phosphate and Fibroblast Growth Factor 23: A role in the pathogenesis and management of Chronic Kidney Disease and Chronic Kidney Disease Mineral and Bone Disorder

    OpenAIRE

    Damasiewicz, Matthew John

    2017-01-01

    Chronic kidney disease (CKD) is defined by the presence of proteinuria or decreased kidney function, with a prevalence of 10-15% in the adult population. CKD can progress to end-stage kidney disease (ESKD) and is associated with progressive abnormalities of bone and mineral metabolism, defined as CKD mineral and bone disorder (CKD-MBD). The use of vitamin D in CKD, the optimal level for initiating treatment and the use of current and novel biomarkers in the management of ...

  5. Developmental and radiobiologic characteristics of canine multinucleated, osteoclast-like cells generated in vitro from canine bone marrow

    International Nuclear Information System (INIS)

    Seed, T.M.; Kaspar, L.V.; Domann, F.; Niiro, G.K.; LeBuis, D.A.

    1988-01-01

    We report here our initial observations on the growth and morphology, and developmental radiosensitivity of giant, multinucleated, osteoclast-like cells (MN-OS) generated through in vitro cultivation of hematopoietic progenitor-enriched canine bone marrow samples. Maximum cell densities of 5.5 x 10(3) to 6.5 x 10(3) MN-OS per cm2 of growth area were achieved following 10 to 14 days of culture at 37 degrees C. Acute gamma irradiation of the initial marrow inocula resulted in significant, dose-dependent perturbations of MN-OS formation, growth, and development. Attempts to estimate radiosensitivity of MN-OS progenitors from canine marrow yielded a range of Do values from a low of 212 cGy measured at six days of culture to higher values of 405 to 542 cGy following 10 to 22 days of culture. At the intermediate times of culture (10 to 14 days), the radiation-induced responses were clearly biphasic, reflecting either (a) the presence of multiple subpopulations of MN-OS progenitors with varying degrees of radiosensitivity or (b) the inherent biphasic nature of MN-OS development involving early progenitor cell proliferation followed by maturation and subsequent fusion. Morphologically, MN-OS generated from irradiated marrow inocula appeared only marginally altered, with alterations expressed largely in a biphasic, dose-dependent fashion in terms of smaller cell size, reduced number of nuclei, increased expression of both surface microprojections, and a unique set of crystalloid cytoplasmic inclusions. Functionally, MN-OS appeared to be impaired by irradiation of marrow progenitors, as evidenced by failure to initiate resorptive attachments to devitalized bone spicules in vitro

  6. Bone scintigraphy and metabolic disorders

    International Nuclear Information System (INIS)

    Mari', C.; Catafau, A.; Carrio', I.

    1999-01-01

    The paper discusses the main clinical value of bone scan in metabolic bone disease: its detection of focal conditions or focal complications of such generalized disease, its most common use of being the detection of fractures in osteoporosis, pseudofractures in osteomalacia and the evaluation of Paget's disease

  7. Developmental basis for filamin-A-associated myxomatous mitral valve disease

    Science.gov (United States)

    Sauls, Kimberly; de Vlaming, Annemarieke; Harris, Brett S.; Williams, Katherine; Wessels, Andy; Levine, Robert A.; Slaugenhaupt, Susan A.; Goodwin, Richard L.; Pavone, Luigi Michele; Merot, Jean; Schott, Jean-Jacques; Le Tourneau, Thierry; Dix, Thomas; Jesinkey, Sean; Feng, Yuanyi; Walsh, Christopher; Zhou, Bin; Baldwin, Scott; Markwald, Roger R.; Norris, Russell A.

    2012-01-01

    Aims We hypothesized that the structure and function of the mature valves is largely dependent upon how these tissues are built during development, and defects in how the valves are built can lead to the pathological progression of a disease phenotype. Thus, we sought to uncover potential developmental origins and mechanistic underpinnings causal to myxomatous mitral valve disease. We focus on how filamin-A, a cytoskeletal binding protein with strong links to human myxomatous valve disease, can function as a regulatory interface to control proper mitral valve development. Methods and results Filamin-A-deficient mice exhibit abnormally enlarged mitral valves during foetal life, which progresses to a myxomatous phenotype by 2 months of age. Through expression studies, in silico modelling, 3D morphometry, biochemical studies, and 3D matrix assays, we demonstrate that the inception of the valve disease occurs during foetal life and can be attributed, in part, to a deficiency of interstitial cells to efficiently organize the extracellular matrix (ECM). This ECM organization during foetal valve gestation is due, in part, to molecular interactions between filamin-A, serotonin, and the cross-linking enzyme, transglutaminase-2 (TG2). Pharmacological and genetic perturbations that inhibit serotonin-TG2-filamin-A interactions lead to impaired ECM remodelling and engender progression to a myxomatous valve phenotype. Conclusions These findings illustrate a molecular mechanism by which valve interstitial cells, through a serotonin, TG, and filamin-A pathway, regulate matrix organization during foetal valve development. Additionally, these data indicate that disrupting key regulatory interactions during valve development can set the stage for the generation of postnatal myxomatous valve disease. PMID:22843703

  8. Hypercalcaemia due to immobilization of a patient with Paget's disease of bone.

    OpenAIRE

    Nathan, A. W.; Ludlam, H. A.; Wilson, D. W.; Dandona, P.

    1982-01-01

    A man with Paget's disease of bone was admitted with a fractured neck of femur. On admission he was normocalcaemic, but with immobilization he rapidly became hypercalcaemic, despite a normal diet and no medication. The hypercalcaemia responded to calcitonin. Although hypercalcaemia is often quoted as complicating the immobilization of such patients, well documented uncomplicated cases have rarely been reported.

  9. Not All Bones are Created Equal - Using Zebrafish and Other Teleost Species in Osteogenesis Research

    NARCIS (Netherlands)

    Apschner, A.; Schulte-Merker, S.; Witten, P.E.

    2011-01-01

    Developmental osteogenesis and pathologies of mineralized tissues are areas of intense investigations in the mammalian field, but different from other areas of organ formation and developmental biology, zebrafish have been somewhat slow in joining the area of bone research. In recent years, however,

  10. Modelling Gaucher disease progression: long-term enzyme replacement therapy reduces the incidence of splenectomy and bone complications

    NARCIS (Netherlands)

    van Dussen, Laura; Biegstraaten, Marieke; Dijkgraaf, Marcel Gw; Hollak, Carla Em

    2014-01-01

    Long-term complications and associated conditions of type 1 Gaucher Disease (GD) can include splenectomy, bone complications, pulmonary hypertension, Parkinson disease and malignancies. Enzyme replacement therapy (ERT) reverses cytopenia and reduces organomegaly. To study the effects of ERT on

  11. A fresh look at metabolic bone diseases in reptiles and amphibians.

    Science.gov (United States)

    Klaphake, Eric

    2010-09-01

    Metabolic bone diseases (MBDs) are a common presenting complaint in reptiles and amphibians to veterinarians; however, understanding of the causes and diagnostic and treatment options is often extrapolated from human or other mammalian medicine models. Although the roles of UV-B, calcium, phosphorus, and cholecalciferol are better understood in some MBDs, there remain many X factors that are not. Likewise, quantitative diagnosis of MBDs has been difficult not only in terms of staging a disease but also regarding whether or not a condition is present. Treatment options also present challenges in corrective husbandry and diet modifications, medication/modality selection, and dosing/regimen parameters. Copyright 2010 Elsevier Inc. All rights reserved.

  12. Bone marrow transplantation in patients with storage diseases: a developing country experience

    Directory of Open Access Journals (Sweden)

    Lange Marcos C.

    2006-01-01

    Full Text Available Bone marrow transplantation (BMT is a therapeutic option for patients with genetic storage diseases. Between 1979 and 2002, eight patients, four females and four males (1 to 13 years old were submitted to this procedure in our center. Six patients had mucopolysaccharidosis (MPS I in 3; MPS III in one and MPS VI in 2, one had adrenoleukodystrophy (ALD and one had Gaucher disease. Five patients had related and three unrelated BMT donor. Three patients developed graft versus host disease (two MPS I and one MPS VI and died between 37 and 151 days after transplantation. Five patients survived 4 to 16 years after transplantation. Three patients improved (one MPS I; one MPS VI and the Gaucher disease patient, one patient had no disease progression (ALD and in one patient this procedure did not change the natural course of the disease (MPS III.

  13. Deconvolution analysis of sup(99m)Tc-methylene diphosphonate kinetics in metabolic bone disease

    Energy Technology Data Exchange (ETDEWEB)

    Knop, J.; Kroeger, E.; Stritzke, P.; Schneider, C.; Kruse, H.P.

    1981-02-01

    The kinetics of sup(99m)Tc-methylene diphosphonate (MDP) and /sup 47/Ca were studied in three patients with osteoporosis, three patients with hyperparathyroidism, and two patients with osteomalacia. The activities of sup(99m)Tc-MDP were recorded in the lumbar spine, paravertebral soft tissues, and in venous blood samples for 1 h after injection. The results were submitted to deconvolution analysis to determine regional bone accumulation rates. /sup 47/Ca kinetics were analysed by a linear two-compartment model quantitating short-term mineral exchange, exchangeable bone calcium, and calcium accretion. The sup(99m)Tc-MDP accumulation rates were small in osteoporosis, greater in hyperparathyroidism, and greatest in osteomalacia. No correlations were obtained between sup(99m)Tc-MDP bone accumulation rates and the results of /sup 47/Ca kinetics. However, there was a significant relationship between the level of serum alkaline phosphatase and bone accumulation rates (R = 0.71, P < 0.025). As a result deconvolution analysis of regional sup(99m)Tc-MDP kinetics in dynamic bone scans might be useful to quantitate osseous tracer accumulation in metabolic bone disease. The lack of correlation between the results of sup(99m)Tc-MDP kinetics and /sup 47/Ca kinetics might suggest a preferential binding of sup(99m)Tc-MDP to the organic matrix of the bone, as has been suggested by other authors on the basis of experimental and clinical investigations.

  14. Study of /sup 201/Tl uptake by bone and bone marrow on /sup 201/Tl scintigraphy. With special reference to bone marrow abnormalities

    Energy Technology Data Exchange (ETDEWEB)

    Fujii, Tadashige; Tanaka, Masao; Hirose, Yoshiki; Hirayama, Jiro; Handa, Kenjiro; Nakanishi, Fumiko; Yano, Kesato; Ueda, Hitoshi

    1989-04-01

    Thallium-201 (Tl-201) uptake in the bone and bone marrow was examined in a total of 93 patients with various diseases. Sternal uptake of Tl-201 was observed when patients had bone marrow abnormality especially associated with hematopoietic disease. It was associated with proliferation of immature cells and of various types of bone marrow cells, especially erythroblastic and plasma cells. Whole-body Tl-201 scanning showed a high uptake (82%) in the sternum, chest, lumbar vertebrae, and pelvis. Thallium-201 was definitively taken up by the sternum in polycythemia (5/41), hemolytic anemia (2/2), iron deficiency anemia (2/2), and multiple myeloma (2/5). For leukemia, Tl-201 uptake was slight or negative. Thallium-201 scanning proved useful in visualizing bone marrow abnormality, although careful interpretation of bone and bone marrow uptake is required. (Namekawa, K).

  15. Analysis on Developmental Factors of the Liver Diseases in Ultrasound Diagnosis of Healthcare

    International Nuclear Information System (INIS)

    Lee, Mi Yeon; Jung, Hong Ryang; Lim, Chang Hwan

    2009-01-01

    The study found out developmental factors of the liver diseases in 29, 531 cases of the healthy adults who were diagnosed by using ultrasound at domestic healthcare centers in 6 cities. The results are as follows. Based on the result of the study, the liver diseases diagnosed by using ultrasound was revealed to show 43.1% of prevalence, and the occurrence was significantly higher in male (23.3%) than in female (19.8%). The prevalence of hepatic diseases related to the BMI was revealed to show highest prevalence of the fatty liver in obese group (BMI 25) by recording 44.3%. Smoking contributed to the high prevalence of all liver diseases. Although the fatty liver was the most frequently occurred form of liver diseases by recording the prevalence of 49.1% (22.2% in male, 26.9% in female), the significant difference was found only in female (p 0.05). The prevalence of hepatic diseases related to the hypertension was revealed to show highest prevalence of the fatty liver in hypertension group by recording 67.7%. The prevalence of hepatic diseases related to the diabetes was revealed to show highest prevalence of the fatty liver in diabetes group by recording 66.2%. The high prevalence of all hepatic diseases was related to diabetes mellitus with statistical significance (p 0.05).

  16. Bone scintigraphy and metabolic disorders

    Energy Technology Data Exchange (ETDEWEB)

    Mari' , C.; Catafau, A.; Carrio' , I. [Hospital de Sant Pau, Barcelone (Spain). Serv. of Nuclear Medicine

    1999-09-01

    The paper discusses the main clinical value of bone scan in metabolic bone disease: its detection of focal conditions or focal complications of such generalized disease, its most common use of being the detection of fractures in osteoporosis, pseudo fractures in osteomalacia and the evaluation of Paget's disease.

  17. [Effect of tumour necrosis factor α blockade on bone metabolism in chronic inflammatory joint diseases].

    Science.gov (United States)

    Aguilar Del Rey, Francisco Javier; García Portales, Rosa; Haro Liger, Manuel; Rodríguez Andreu, José; Casals Sánchez, José Luis; Pérez González, Rita

    2016-07-15

    To evaluate the effect of anti-TNF treatments on bone mineral density (BMD), bone remodelling markers (BRM) and receptor activator of nuclear factor κB ligand (RANKL) and osteoprotegerin (OPG) in patients with chronic inflammatory joint diseases. A longitudinal prospective study was performed under clinical practice conditions on 31 patients diagnosed of rheumatoid arthritis, psoriatic arthropathy and ankylosing spondylitis who had received treatment with anti-TNF alpha drugs for one year. BMD, OPG and RANKL soluble form (sRANKL) were studied at the onset and end of the study. During the study (0, 3, 6, 9 and 12 month), disease activity (SDAI, BASDAI and CRP), functional capacity (HAQ, BASFI), BRM and vitamin D were studied. BMD was not modified after one year of treatment. The patients who took corticosteroids had a mean bone mass loss of 3% in the lumbar spine (±1.6, P=.02). In regards to the BRM, did not experience significant changes over the course of the study. Disease activity, both SDAI (P=.002) and BASDAI (P=.002), decreased. OPG was maintained without changes during the year of treatment while both the sRANKL (0.28±0.22, P=.013) and sRANKL/OPG ratio significantly decreased (0.04±0.03, P=.031). The patients being treated with anti-TNF did not present with a significant loss of DMO during the study (one year), at the same time experiencing an improvement in disease activity. This protection has been clearer in the responding patients. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  18. Neuroimmune mechanisms of stress: sex differences, developmental plasticity, and implications for pharmacotherapy of stress-related disease.

    Science.gov (United States)

    Deak, Terrence; Quinn, Matt; Cidlowski, John A; Victoria, Nicole C; Murphy, Anne Z; Sheridan, John F

    2015-01-01

    The last decade has witnessed profound growth in studies examining the role of fundamental neuroimmune processes as key mechanisms that might form a natural bridge between normal physiology and pathological outcomes. Rooted in core concepts from psychoneuroimmunology, this review utilizes a succinct, exemplar-driven approach of several model systems that contribute significantly to our knowledge of the mechanisms by which neuroimmune processes interact with stress physiology. Specifically, we review recent evidence showing that (i) stress challenges produce time-dependent and stressor-specific patterns of cytokine/chemokine expression in the CNS; (ii) inflammation-related genes exhibit unique expression profiles in males and females depending upon individual, cooperative or antagonistic interactions between steroid hormone receptors (estrogen and glucocorticoid receptors); (iii) adverse social experiences incurred through repeated social defeat engage a dynamic process of immune cell migration from the bone marrow to brain and prime neuroimmune function and (iv) early developmental exposure to an inflammatory stimulus (carageenin injection into the hindpaw) has a lasting influence on stress reactivity across the lifespan. As such, the present review provides a theoretical framework for understanding the role that neuroimmune mechanisms might play in stress plasticity and pathological outcomes, while at the same time pointing toward features of the individual (sex, developmental experience, stress history) that might ultimately be used for the development of personalized strategies for therapeutic intervention in stress-related pathologies.

  19. Animal Models for Evaluation of Bone Implants and Devices: Comparative Bone Structure and Common Model Uses.

    Science.gov (United States)

    Wancket, L M

    2015-09-01

    Bone implants and devices are a rapidly growing field within biomedical research, and implants have the potential to significantly improve human and animal health. Animal models play a key role in initial product development and are important components of nonclinical data included in applications for regulatory approval. Pathologists are increasingly being asked to evaluate these models at the initial developmental and nonclinical biocompatibility testing stages, and it is important to understand the relative merits and deficiencies of various species when evaluating a new material or device. This article summarizes characteristics of the most commonly used species in studies of bone implant materials, including detailed information about the relevance of a particular model to human bone physiology and pathology. Species reviewed include mice, rats, rabbits, guinea pigs, dogs, sheep, goats, and nonhuman primates. Ultimately, a comprehensive understanding of the benefits and limitations of different model species will aid in rigorously evaluating a novel bone implant material or device. © The Author(s) 2015.

  20. Chronic obstructive pulmonary disease and low bone mass: A case-control study

    Directory of Open Access Journals (Sweden)

    Rakesh K Gupta

    2014-01-01

    Full Text Available Background and Objective: Low bone mass (osteopenia and osteoporosis is one of the effects associated with chronic obstructive pulmonary disease (COPD. There is very little data from Saudi Arabia on COPD and low bone mass. This retrospective study was done to assess the prevalence of osteoporosis and osteopenia in COPD patients attending King Fahd Hospital of the University (KFHU, Alkhobar. Patients and Methods: After obtaining the ethical approval from the research committee, all patients seen between at the King Fahd Hospital of the University between January 2010 and December 2012 were included. The inclusion criteria included a follow up of a minimum 2 years, and the Medical Records should have the details of forced expiratory volume in one second (FEV 1 , blood bone profile and bone biomarkers and dual-energy X-ray absorptiometry (DEXA scan. Patients were labeled as osteopenia if the T score was -<1 to <-2.5 and osteoporosis of <-2.5 as per the WHO definition of osteopenia and osteoporosis. Results: Seventy-three patients were being followed in the clinics and 49 patients satisfied the inclusion criteria. The average age was 60.6 ± 10.47 years; males were 43 and females 6. Three (6.1% were normal and the remaining 46 (93.9% were with low bone mass. Thirty-two (65.3% were osteoporotic and 14 (28.57% were osteopenic. The average duration of COPD was 4.5 ± 6.2 years. Majority (n = 36, 73.4% of patients were in the Global Initiative for COPD (GOLD class II and III. FEV 1 was significantly lower in the patients with low bone mass 1.66 ± 0.60 versus 3.61 ± 0.58 (P < 0.001. Conclusions: Our study shows that over 90% of Saudi Arabian patients with COPD suffer from osteopenia and osteoporosis and unfortunately they remain under-diagnosed and undertreated.

  1. Interesting bone scans - unusual findings

    International Nuclear Information System (INIS)

    Dobson, M.; Wadhwa, S.S.; Mansberg, R.; Fernandes, V.B.

    1997-01-01

    A 59-year-old female with carcinoma of the colon and known liver metastatic disease was referred for bone scan to evaluate for bone metastases. Although no bone metastases were found, there was abnormal uptake noted in the liver corresponding to a metastatic calcified lesion. The only other findings were of degenerative disease in the cervical spine, right shoulder and small joints of the hands. A 69-year-old male with carcinoma of the prostate and right side low back pain was referred for bone scan. No focal abnormalities to suggest metastatic disease were identified; findings within the cervical spine, lumber spine and knees were presumed secondary to degenerative disease. Intermittent pain persisted and the patient was referred for a repeat bone scan six months later. Previous scan findings of degenerative disease and no metastatic disease were confirmed; however, closer inspection revealed an enlarged right kidney with significant retention of tracer in the pelvicalyceal system suggesting possible obstruction. A Retrograde pyelogram was performed, and no obvious obstruction demonstrated. As bone scan findings were very suggestive of obstruction, a DTPA scan with lasix was performed showing a dilated right collecting system with no functional obstruction. Given the degree of dilation, it is possible that the patient experiences intermittent PUJ obstruction causing his symptoms. A 33-year-old male with insulin dependent diabetes mellitus and viral arthritis was referred for a bone scan. A three phase revealed increased uptake in the region of the knee and leR proximal tibia. Delayed whole body images revealed multiple focal areas of osteoblastic activity in the leR tibia. Abnormal uptake was also seen in the upper third of the leR femur. The remainder of the skeletal survey was normal. X-ray correlation of the leR tibia and femoral findings was undertaken. Combinating unilateral changes on bone scan and X-ray although very suggestive of sclerotic polyostotic

  2. Bone marrow blood vessel ossification and "microvascular dead space" in rat and human long bone.

    Science.gov (United States)

    Prisby, Rhonda D

    2014-07-01

    Severe calcification of the bone microvascular network was observed in rats, whereby the bone marrow blood vessels appeared ossified. This study sought to characterize the magnitude of ossification in relation to patent blood vessels and adipocyte content in femoral diaphyses. Additionally, this study confirmed the presence of ossified vessels in patients with arteriosclerotic vascular disease and peripheral vascular disease and cellulitis. Young (4-6 month; n=8) and old (22-24 month; n=8) male Fischer-344 rats were perfused with barium sulfate to visualize patent bone marrow blood vessels. Femoral shafts were processed for bone histomorphometry to quantify ossified (Goldner's Trichrome) and calcified (Alizarin Red) vessels. Adipocyte content was also determined. Additional femora (n=5/age group) were scanned via μCT to quantify microvascular ossification. Bone marrow blood vessels from the rats and the human patients were also isolated and examined via microscopy. Ossified vessels (rats and humans) had osteocyte lacunae on the vessel surfaces and "normal" vessels were transitioning into bone. The volume of ossified vessels was 4800% higher (pnecrosis. Progression of bone microvascular ossification may provide the common link associated with age-related changes in bone and bone marrow. The clinical implications may be evident in the difficulties treating bone disease in the elderly. Copyright © 2014 Elsevier Inc. All rights reserved.

  3. Bone Marrow Blood Vessel Ossification and “Microvascular Dead Space” in Rat and Human Long Bone

    Science.gov (United States)

    Prisby, Rhonda D.

    2014-01-01

    Severe calcification of the bone microvascular network was observed in rats, whereby the bone marrow blood vessels appeared ossified. This study sought to characterize the magnitude of ossification in relation to patent blood vessels and adipocyte content in femoral diaphyses. Additionally, this study confirmed the presence of ossified vessels in patients with arteriosclerotic vascular disease and peripheral vascular disease and cellulitis. Young (4–6 mon; n=8) and old (22–24 mon; n=8) male Fischer-344 rats were perfused with barium sulfate to visualize patent bone marrow blood vessels. Femoral shafts were processed for bone histomorphometry to quantify ossified (Goldner’s Trichrome) and calcified (Alizarin Red) vessels. Adipocyte content was also determined. Additional femora (n=5/age group) were scanned via µCT to quantify microvascular ossification. Bone marrow blood vessels from rats and the human patients were also isolated and examined via microscopy. Ossified vessels (rats and humans) had osteocyte lacunae on the vessel surfaces and “normal” vessels were transitioning into bone. The volume of ossified vessels was 4800% higher (p necrosis. The progression of bone microvascular ossification may provide the common link associated with age-related changes in bone and bone marrow. The clinical implications may be evident in the difficulties treating bone disease in the elderly. PMID:24680721

  4. Omics analysis of human bone to identify genes and molecular networks regulating skeletal remodeling in health and disease.

    Science.gov (United States)

    Reppe, Sjur; Datta, Harish K; Gautvik, Kaare M

    2017-08-01

    The skeleton is a metabolically active organ throughout life where specific bone cell activity and paracrine/endocrine factors regulate its morphogenesis and remodeling. In recent years, an increasing number of reports have used multi-omics technologies to characterize subsets of bone biological molecular networks. The skeleton is affected by primary and secondary disease, lifestyle and many drugs. Therefore, to obtain relevant and reliable data from well characterized patient and control cohorts are vital. Here we provide a brief overview of omics studies performed on human bone, of which our own studies performed on trans-iliacal bone biopsies from postmenopausal women with osteoporosis (OP) and healthy controls are among the first and largest. Most other studies have been performed on smaller groups of patients, undergoing hip replacement for osteoarthritis (OA) or fracture, and without healthy controls. The major findings emerging from the combined studies are: 1. Unstressed and stressed bone show profoundly different gene expression reflecting differences in bone turnover and remodeling and 2. Omics analyses comparing healthy/OP and control/OA cohorts reveal characteristic changes in transcriptomics, epigenomics (DNA methylation), proteomics and metabolomics. These studies, together with genome-wide association studies, in vitro observations and transgenic animal models have identified a number of genes and gene products that act via Wnt and other signaling systems and are highly associated to bone density and fracture. Future challenge is to understand the functional interactions between bone-related molecular networks and their significance in OP and OA pathogenesis, and also how the genomic architecture is affected in health and disease. Copyright © 2017 Elsevier Inc. All rights reserved.

  5. Bone metastases from breast cancer at the time or radical mastectomy as detected by bone scan. Eight-year follow-up.

    Science.gov (United States)

    Sklaroff, R B; Sklaroff, D M

    1976-07-01

    Sixty-four women with Stage II breast cancer who had Sr85 bone scans at the time of radical mastectomy were followed for 8 years in a prospective study. Those women with positive scans had a slight, but statistically significant, increased incidence of metastic disease, particularly for metastases to bone.However, 40% of those women with positive bone scans and negative roentgenograms survived 8 years without evidence of any metastatic disease. Therefore, it has not been shown at this time that bone scans should be obtained in order to exclude bone metastasis before regional therapy for breast cancer is instituted. Also, a significant percentage of women with negative bone scans developed both bone and soft tissue metastases. As many as 30% of asymptomatic women with a history of breast cancer and positive bone scans and negative bone roentgenograms may still harbor disease in bone after 8 years.

  6. Immunology of Gut-Bone Signaling.

    Science.gov (United States)

    Collins, Fraser L; Schepper, Jonathan D; Rios-Arce, Naiomy Deliz; Steury, Michael D; Kang, Ho Jun; Mallin, Heather; Schoenherr, Daniel; Camfield, Glen; Chishti, Saima; McCabe, Laura R; Parameswaran, Narayanan

    2017-01-01

    In recent years a link between the gastrointestinal tract and bone health has started to gain significant attention. Dysbiosis of the intestinal microbiota has been linked to the pathology of a number of diseases which are associated with bone loss. In addition modulation of the intestinal microbiota with probiotic bacteria has revealed to have both beneficial local and systemic effects. In the present chapter, we discuss the intestinal and bone immune systems, explore how intestinal disease affects the immune system, and examine how these pathologic changes could adversely impact bone health.

  7. Oxidative Stress, Bone Marrow Failure, and Genome Instability in Hematopoietic Stem Cells

    Directory of Open Access Journals (Sweden)

    Christine Richardson

    2015-01-01

    Full Text Available Reactive oxygen species (ROS can be generated by defective endogenous reduction of oxygen by cellular enzymes or in the mitochondrial respiratory pathway, as well as by exogenous exposure to UV or environmental damaging agents. Regulation of intracellular ROS levels is critical since increases above normal concentrations lead to oxidative stress and DNA damage. A growing body of evidence indicates that the inability to regulate high levels of ROS leading to alteration of cellular homeostasis or defective repair of ROS-induced damage lies at the root of diseases characterized by both neurodegeneration and bone marrow failure as well as cancer. That these diseases may be reflective of the dynamic ability of cells to respond to ROS through developmental stages and aging lies in the similarities between phenotypes at the cellular level. This review summarizes work linking the ability to regulate intracellular ROS to the hematopoietic stem cell phenotype, aging, and disease.

  8. The value of bone scintigram in elderly subjects

    International Nuclear Information System (INIS)

    Tanno, Munehiko; Yamada, Hideo; Hayashida, Koichi; Endoh, Kazuo; Nagashima, Junichi; Yamagata, Atsushi; Nishino, Hideo; Chiba, Kazuo

    1986-01-01

    To evaluate increased bone uptake of radionuclides at lumbar vertebra in elderly subjects, comparative study of bone scintigram with CT findings and autopsy finding of bone were performed. Incidence of abnormal increased bone uptake were 59 % in neoplastic disease group, while 50 % in benign disease group, respectively. Bone uptake of radionuclides to thoracic and lumbar vertebrae revealed marked high incidence even in both group. 13 out of 25 cases which revealed bone uptake at lumbar vertebrae in malignant disease group showed metastatic bone lesions and the rest of these cases did not show no apparent macroscopic metastatic bone lesions. In comparative study with CT findings of 18 cases most cases revealed osteosclerotic and osteoperotic changes of bone and fracture on CT imaging at the sites where bone uptake of radionuclides were observed. Thus, these evidence support that ageing process of bone must be considered as important factors to evaluate bone scintigram imaging in elderly subjects. (author)

  9. Endurance exercise and growth hormone improve bone formation in young and growth-retarded chronic kidney disease rats.

    Science.gov (United States)

    Troib, Ariel; Guterman, Mayan; Rabkin, Ralph; Landau, Daniel; Segev, Yael

    2016-08-01

    Childhood chronic kidney disease (CKD) is associated with both short stature and abnormal bone mineralization. Normal longitudinal growth depends on proper maturation of epiphyseal growth plate (EGP) chondrocytes, leading to the formation of trabecular bone in the primary ossification centre. We have recently shown that linear growth impairment in CKD is associated with impaired EGP growth hormone (GH) receptor signalling and that exercise improved insulin-like growth factor I (IGF-I) signalling in CKD-related muscle atrophy. In this study, 20-day-old rats underwent 5/6 nephrectomy (CKD) or sham surgery (C) and were exercised with treadmill, with or without GH supplementation. CKD-related growth retardation was associated with a widened EGP hypertrophic zone. This was not fully corrected by exercise (except for tibial length). Exercise in CKD improved the expression of EGP key factors of endochondral ossification such as IGF-I, vascular endothelial growth factor (VEGF), receptor activator of nuclear factor kappa-B ligand (RANKL) and osteocalcin. Combining GH treatment with treadmill exercise for 2 weeks improved the decreased trabecular bone volume in CKD, as well as the expression of growth plate runt-related transcription factor 2, RANKL, metalloproteinase 13 and VEGF, while GH treatment alone could not do that. Treadmill exercise improves tibial bone linear growth, as well as growth plate local IGF-I. When combined with GH treatment, running exercise shows beneficial effects on trabecular bone formation, suggesting the potential benefit of this combination for CKD-related short stature and bone disease. © The Author 2015. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

  10. Lack of biochemical hypogonadism in elderly Arab males with low bone mineral density disease.

    Science.gov (United States)

    Al Attia, Haider M; Jaysundaram, Krishnasamy; Saraj, Fouad

    2010-01-01

    The aim of the study is to study the relationship between androgen levels and bone mineral density (BMD) in elderly Arab males. Forty-five elderly Arab males underwent Dual X-ray absorptiometry for measurement of BMD. The outcomes were defined as per WHO description. Assays for testosterone (T), gonadotropins (LH and FSH) and estradiol (E2), in the serum were carried out. The ratio of T/LH was used as a surrogate for the cFT assay. We excluded patients receiving hormonal ablation for prostatic neoplasm and patients with chronic liver or renal disease and patients receiving corticosteroids. Twelve were osteoporotic (26.5%); 22 osteopenic (49%); and 11(24.5%) had normal outcome. Osteoporotic patients were significantly older (78.17 +/- 7.59 years) than the osteopenic (70.14 +/- 5.92, P Arab males had reduced bone density that appears to be independent of androgen levels. Osteoporotics were significantly older than those with osteopenia or normal bone density. Aging seemed to have overridden the effect of normal sex hormones on bone density in these patients. Before considering these results as a possible exception to the widely established role of the hypoandrogenemia in male osteoporosis, other potential factors impacting on bone density need to be considered.

  11. Bilateral Knee Pain Associated with Bone Infarction in a Patient with Behcet's Disease

    Directory of Open Access Journals (Sweden)

    Pelin Oktayoglu

    2012-01-01

    Full Text Available We describe a 31-years-old female patient with severe pain in both knees who had been diagnosed as Behcet’s disease (BD for 12 years. She had had a history of complications due to BD including superior vena cava thrombosis, pulmonary thromboembolism, uveitis, and erythema nodosum and has reported the administration of corticosteroid therapy irregularly. After radiologic evaluation, she has been diagnosed with bone infarction of both left and right knee with the existance of lupus anticoagulants (LA positivity. Severe joint pain without the evidence of arthritis must alert the clinician to the possibility of bone necrosis of the extremity, although those may rarely occur bilateral in BD.

  12. Application of bone scintigraphy

    International Nuclear Information System (INIS)

    Rondain, J.E.S.

    1996-01-01

    Bone scanning has varied applications, particularly in the file of oncology. It is used in the diagnosis and follow-up of patients with cancers that metastatize to the bones (breast, prostate CA), also in primary bone cancers, infections of the bones and joints. In early stages of primary breast CA (stage I and II), the incidence of unsuspected bone metastasis is only 1-5%. On the other hand, bone scans serve as a baseline study if bone mets occur at some later stage. In patients with stage II and III breast CA, the conversion from normal to abnormal bone scans is 15% and 17%, respectively, clearly in favor of a baseline bone scan. For prostate CA, bone scanning should be used in conjunction with PSA level determination. In advanced disease, a bone scan will define the extent of the metastases, show problematic lesions in weight-bearing bones, and even allow us to evaluate response to therapy in follow-up bone scans. In patients with lung CA, a positive bone scan will make surgery of the primary lesion inappropriate. For other cancers, a bone scan maybe used if there are other signs, whether clinical or chemical, indicating bone involvement. In patients with GIT, liver, skin, brain or bladder CA, routine bone scanning may be considered superfluous. For patients with suspected infection, a 3-phase bone scan is more desirable. In patients with septic arthritis, the bones of each side of the joint take up the radiopharmaceutical while in patients with cellulitis without bony involvement, only the first two phases (dynamic and bloodpool images) will be abnormal. Bone scanning is also used in avascular lesions such as Legg-calve-Perthes disease where one will see reduced uptake of Tc99m MDP. The advent of SPECT imaging has greatly increased the sensitivity in diagnosing AVN. (author)

  13. Is retention of zoledronic acid onto bone different in multiple myeloma and breast cancer patients with bone metastasis?

    DEFF Research Database (Denmark)

    Søe, Kent; Plesner, Torben; Jakobsen, Erik H

    2013-01-01

    Zoledronic acid (Zol) is used to treat bone disease in both multiple myeloma (MM) and breast cancer patients with bone metastasis (BC). However, bones of MM and BC patients show a difference in retention of the bisphosphonate used for bone scintigraphy. Therefore, we hypothesized that disease...... of Zol correlated with bone-specific alkaline phosphatase (bALP) levels in BC (p = 0.001), and with CTX/bALP in Zol naive MM patients (p = 0.012). Especially in BC patients, WBrt correlated with age (p = 0.014) independently of kidney function. In MM patients WBrt was found to primarily correlate...... with the extent of bone disease (p = 0.028). Multivariate linear regression analyses of the entire cohort pointed out that WBrt of Zol was best predicted by age (p ...

  14. An intelligent medical system for diagnosis of bone diseases

    International Nuclear Information System (INIS)

    Hatzilygeroudis, I.; Vassilakos, P.J.; Tsakalidis, A.

    1994-01-01

    In this paper, aspects of the design of an intelligent medical system for diagnosis of bone diseases that can be detected by scintigraphic images are presented. The system comprises three major parts: a user interface (UI), a database management system (DBMS), and an expert system (ES). The DBMS is used for manipulation of various patient data. A number of patient cases are selected as prototype and stored in separate database. Diagnosis is performed via the ES, called XBONE, based on patient data. Knowledge is represented via an integrated formalism that combines production rules and a neural network. This results in better representation, and facilitates knowledge acquisition and maintenance. (authors)

  15. Transient developmental Purkinje cell axonal torpedoes in healthy and ataxic mouse cerebellum

    Directory of Open Access Journals (Sweden)

    Lovisa Ljungberg

    2016-11-01

    Full Text Available Information is carried out of the cerebellar cortical microcircuit via action potentials propagated along Purkinje cell axons. In several human neurodegenerative diseases, focal axonal swellings on Purkinje cells – known as torpedoes – have been associated with Purkinje cell loss. Interestingly, torpedoes are also reported to appear transiently during development in rat cerebellum. The function of Purkinje cell axonal torpedoes in health as well as in disease is poorly understood. We investigated the properties of developmental torpedoes in the postnatal mouse cerebellum of wildtype and transgenic mice. We found that Purkinje cell axonal torpedoes transiently appeared on axons of Purkinje neurons, with the largest number of torpedoes observed at postnatal day 11 (P11. This was after peak developmental apoptosis had occurred, when Purkinje cell counts in a lobule were static, suggesting that most developmental torpedoes appear on axons of neurons that persist into adulthood. We found that developmental torpedoes were not associated with a presynaptic GABAergic marker, indicating that they are not synapses. They were seldom found at axonal collateral branch points, and lacked microglia enrichment, suggesting that they are unlikely to be involved in axonal refinement. Interestingly, we found several differences between developmental torpedoes and disease-related torpedoes: developmental torpedoes occured largely on myelinated axons, and were not associated with changes in basket cell innervation on their parent soma. Disease-related torpedoes are typically reported to contain neurofilament; while the majority of developmental torpedoes did as well, a fraction of smaller developmental torpedoes did not. These differences indicate that developmental torpedoes may not be functionally identical to disease-related torpedoes. To study this further, we used a mouse model of spinocerebellar ataxia type 6 (SCA6, and found elevated disease

  16. Bone position emission tomography with or without CT Is more accurate than bone scan for detection of bone metastasis

    International Nuclear Information System (INIS)

    Lee, Soo Jin; Lee, Wom Woo; Kim, Sang Eun

    2013-01-01

    Na1 8F bone positron emission tomography (bone PET) is a new imaging modality which is useful for the evaluation of bone diseases. Here, we compared the diagnostic accuracies between bone PET and bone scan for the detection of bone metastasis (BM). Sixteen cancer patients (M:F = 10:6, mean age = 60 ± 12 years) who underwent both bone PET and bone scan were analyzed. Bone PET was conducted 30 minutes after the injection of 370 MBq Na1 8F , and a bone scan was performed 3 hours after the injection of 1295 MBq 9 9mT c-hydroxymethylene diphosphonate. In the patient-based analysis (8 patients with BM and 8 without BM), the sensitivities of bone PET (100% 8/8) and bone scan (87.5% = 7/8) were not significantly different (p > 0.05), whereas the specificity of bone PET (87.5% = 7/8) was significantly greater than that of the bone scan (25% = 2/8) (p 8F bone PET is more accurate than bone scan for BM evaluation.

  17. Short faces, big tongues: developmental origin of the human chin.

    Directory of Open Access Journals (Sweden)

    Michael Coquerelle

    Full Text Available During the course of human evolution, the retraction of the face underneath the braincase, and closer to the cervical column, has reduced the horizontal dimension of the vocal tract. By contrast, the relative size of the tongue has not been reduced, implying a rearrangement of the space at the back of the vocal tract to allow breathing and swallowing. This may have left a morphological signature such as a chin (mental prominence that can potentially be interpreted in Homo. Long considered an autopomorphic trait of Homo sapiens, various extinct hominins show different forms of mental prominence. These features may be the evolutionary by-product of equivalent developmental constraints correlated with an enlarged tongue. In order to investigate developmental mechanisms related to this hypothesis, we compare modern 34 human infants against 8 chimpanzee fetuses, whom development of the mandibular symphysis passes through similar stages. The study sets out to test that the shared ontogenetic shape changes of the symphysis observed in both species are driven by the same factor--space restriction at the back of the vocal tract and the associated arrangement of the tongue and hyoid bone. We apply geometric morphometric methods to extensive three-dimensional anatomical landmarks and semilandmarks configuration, capturing the geometry of the cervico-craniofacial complex including the hyoid bone, tongue muscle and the mandible. We demonstrate that in both species, the forward displacement of the mental region derives from the arrangement of the tongue and hyoid bone, in order to cope with the relative horizontal narrowing of the oral cavity. Because humans and chimpanzees share this pattern of developmental integration, the different forms of mental prominence seen in some extinct hominids likely originate from equivalent ontogenetic constraints. Variations in this process could account for similar morphologies.

  18. Bone-composition imaging using coherent-scatter computed tomography: Assessing bone health beyond bone mineral density

    International Nuclear Information System (INIS)

    Batchelar, Deidre L.; Davidson, Melanie T.M.; Dabrowski, Waldemar; Cunningham, Ian A.

    2006-01-01

    Quantitative analysis of bone composition is necessary for the accurate diagnosis and monitoring of metabolic bone diseases. Accurate assessment of the bone mineralization state is the first requirement for a comprehensive analysis. In diagnostic imaging, x-ray coherent scatter depends upon the molecular structure of tissues. Coherent-scatter computed tomography (CSCT) exploits this feature to identify tissue types in composite biological specimens. We have used CSCT to map the distributions of tissues relevant to bone disease (fat, soft tissue, collagen, and mineral) within bone-tissue phantoms and an excised cadaveric bone sample. Using a purpose-built scanner, we have measured hydroxyapatite (bone mineral) concentrations based on coherent-scatter patterns from a series of samples with varying hydroxyapatite content. The measured scatter intensity is proportional to mineral density in true g/cm 3 . Repeated measurements of the hydroxyapatite concentration in each sample were within, at most, 2% of each other, revealing an excellent precision in determining hydroxyapatite concentration. All measurements were also found to be accurate to within 3% of the known values. Phantoms simulating normal, over-, and under-mineralized bone were created by mixing known masses of pure collagen and hydroxyapatite. An analysis of the composite scatter patterns gave the density of each material. For each composite, the densities were within 2% of the known values. Collagen and hydroxyapatite concentrations were also examined in a bone-mimicking phantom, incorporating other bone constituents (fat, soft tissue). Tomographic maps of the coherent-scatter properties of each specimen were reconstructed, from which material-specific images were generated. Each tissue was clearly distinguished and the collagen-mineral ratio determined from this phantom was also within 2% of the known value. Existing bone analysis techniques cannot determine the collagen-mineral ratio in intact specimens

  19. Treatment for avascular necrosis of bone in people with sickle cell disease.

    Science.gov (United States)

    Martí-Carvajal, Arturo J; Solà, Ivan; Agreda-Pérez, Luis H

    2016-08-09

    Avascular necrosis of bone is a frequent and severe complication of sickle cell disease and its treatment is not standardised. This is an update of a previously published Cochrane Review. To determine the impact of any surgical procedure compared with other surgical interventions or non-surgical procedures, on avascular necrosis of bone in people with sickle cell disease in terms of efficacy and safety. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Additional trials were sought from the reference lists of papers identified by the search strategy.Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 27 May 2016. Randomized clinical trials comparing specific therapies for avascular necrosis of bone in people with sickle cell disease. Each author independently extracted data and assessed trial quality. Since only one trial was identified, meta-analysis was not possible. One trial (46 participants) was eligible for inclusion. After randomization eight participants were withdrawn, mainly because they declined to participate in the trial. Data were analysed for 38 participants at the end of the trial. After a mean follow up of three years, hip core decompression and physical therapy did not show clinical improvement when compared with physical therapy alone using the score from the original trial (an improvement of 18.1 points for those treated with intervention therapy versus an improvement of 15.7 points with control therapy). There was no significant statistical difference between groups regarding major complications (hip pain, risk ratio 0.95 (95% confidence interval 0.56 to 1.60; vaso-occlusive crises, risk ratio 1.14 (95% confidence interval 0.72 to 1.80; very low quality of evidence); and acute chest syndrome, risk

  20. Increased incidence of murine graft-versus-host disease after allogeneic bone marrow transplantation by previous infusion of syngeneic bone marrow cells

    International Nuclear Information System (INIS)

    Waer, M.; Ang, K.K.; van der Schueren, E.; Vandeputte, M.

    1984-01-01

    Different groups of BALB/c mice received supralethal total-body irradiation (TBI; 8.5 Gy, day 0). When 30 x 10(6) allogeneic (C57B1) bone marrow (BM) cells were infused with or without 10 x 10(6) syngeneic (BALB/c) bM cells on day 1, many animals (60%) died from graft-versus-host disease (GVHD). Typing of peripheral blood leukocytes for donor antigens showed that, respectively, 22/22 and 17/21 of the mice in both groups became chimeric. When syngeneic bone marrow was given on day 1 and allogeneic bone marrow on day 2 after TBI, a similar number of animals (21/23) became chimeric, but GVHD occurred more frequently in this group (25/26 mice, P less than 0.01). When the syngeneic bone marrow cells were replaced by spleen cells, or when the transplantation of allogeneic bone marrow was delayed till days 3 or 6 after TBI, almost all mice rejected the allogeneic BM graft and became long-term survivors. BALB/c mice receiving 30 x 10(6) C57B1 BM cells after 17 daily fractions of 0.2 Gy of total lymphoid irradiation (TLI), showed a high incidence of chimerism (15/17) and in none of the latter animals was GVHD observed. Despite the high incidence of GVHD in the mice receiving allogeneic BM after TBI and syngeneic BM transplantation, as compared with mice prepared with TLI which do not develop GVHD, suppressor cells were as easily induced after TBI and syngeneic BM transplantation as after TLI

  1. Clinical Usefulness of 18F-fluoride Bone PET

    International Nuclear Information System (INIS)

    Kang, Ji Yeon; Lee, Won Woo; Lee, Byung Chul; Kim, Sang Eun; So, Young

    2010-01-01

    18 F-fluoride bone positron emission tomography (PET) has been reported as a useful bone imaging modality. However, no clinical bone PET study had been performed previously in Korea. The authors investigated the usefulness of 18 F-fluoride bone PET in Korean patients with malignant or benign bone disease. Eighteen consecutive patients (eight women, ten men; mean age, 55±12 years) who had undergone 18 F-fluoride bone PET for the evaluation of bone metastasis (n=13) or benign bone lesions (n=5) were included. The interpretation of bone lesions on 18 F-fluoride bone PET was determined by consensus of two nuclear medicine physicians, and final results were confirmed using combination of all imaging studies and/or clinical follow-up. The analysis was performed on the basis of lesion group. Thirteen patients with malignant disease had 15 lesion groups, among which seven were confirmed as metastatic bone lesions and eight were confirmed as non-metastatic lesions. 18 F-fluoride bone PET correctly identified six of seven metastatic lesions (sensitivity, 86%), and seven of eight non-metastatic lesions (specificity, 88%). On the other hand, five patients with benign conditions had five bone lesion groups; four were confirmed as benign bone diseases and the other one was confirmed as not a bone lesion. 18 F-fluoride bone PET showed correct results in all the five lesion groups. 18 F-fluoride bone PET showed promising potential for bone imaging in Korean patients with malignant diseases as well as with various benign bone conditions. Therefore, further studies are required on the diagnostic performance and cost-effectiveness of 18 F-fluoride bone PET.

  2. Procedural learning in Parkinson's disease, specific language impairment, dyslexia, schizophrenia, developmental coordination disorder, and autism spectrum disorders: A second-order meta-analysis.

    Science.gov (United States)

    Clark, Gillian M; Lum, Jarrad A G

    2017-10-01

    The serial reaction time task (SRTT) has been used to study procedural learning in clinical populations. In this report, second-order meta-analysis was used to investigate whether disorder type moderates performance on the SRTT. Using this approach to quantitatively summarise past research, it was tested whether autism spectrum disorder, developmental coordination disorder, dyslexia, Parkinson's disease, schizophrenia, and specific language impairment differentially affect procedural learning on the SRTT. The main analysis revealed disorder type moderated SRTT performance (p=0.010). This report demonstrates comparable levels of procedural learning impairment in developmental coordination disorder, dyslexia, Parkinson's disease, schizophrenia, and specific language impairment. However, in autism, procedural learning is spared. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Profile of chronic kidney disease related-mineral bone disorders in newly diagnosed advanced predialysis diabetic kidney disease patients: A hospital based cross-sectional study.

    Science.gov (United States)

    Ray, S; Beatrice, A M; Ghosh, A; Pramanik, S; Bhattacharjee, R; Ghosh, S; Raychaudhury, A; Mukhopadhyay, S; Chowdhury, S

    2017-12-01

    Chronic kidney disease related-mineral bone disorder (CKD-MBD) has been poorly studied in pre-dialysis Indian CKD population. There are limited data on the pattern of these disturbances in diabetic CKD patients. Therefore, a study was conducted to find out the profile of mineral bone disorders in T2DM patients with pre-dialysis CKD. In this cross-sectional design, diabetic patients with newly-diagnosed stage 4 and 5 CKD were evaluated. Serum levels of calcium, phosphorus, intact parathyroid hormone (iPTH), 25 hydroxy vitamin D and total alkaline phosphatase (ALP) were measured in all patients. Bone mineral density (BMD) was measured using dual-energy X-ray absorptiometry (DXA). A total of 72 eligible patients participated (44 males, 28 females; age 54.2±11.7). Patients with CKD Stage 5 had a lower level of corrected serum calcium and significantly higher level of inorganic phosphorus, total ALP and iPTH as compared to stage 4 patients. Overall, 38.5% were hypocalcemic, 31.43% were hyperphosphatemic. 24.2% of CKD subjects were vitamin D deficient (110pg/ml) was detected in nearly 43% of patients. In stage 5, only 32% patients was found to have hyperparathyroidism (iPTH>300pg/ml). There was a good correlation between iPTH and total ALP (r=0.5, p=0.0001) in this cohort. 25 (OH) vitamin D was inversely correlated with ALP (r=-0.39, P=0.001) and showed negative correlation with urine ACR (r=-0.37, P=0.002). As a group, the osteoporotic CKD subjects exhibited higher iPTH (220.1±153.8 vs. 119±108pg/ml, p<0.05) as compared to those who were osteopenic or had normal bone density. There was significant correlation between BMD and iPTH (adjusted r=-0.436; P=0.001). In the multivariate regression model, we found intact PTH to predict BMD even after adjustment of all the confounders. The current study showed that adynamic bone disease is prevalent even in pre-dialysis CKD population. High bone turnover disease may not be the most prevalent type in diabetic CKD. However, it

  4. Health promotion and disease prevention strategies in older adults with Intellectual and Developmental Disabilities

    Directory of Open Access Journals (Sweden)

    Eli eCarmeli

    2014-04-01

    Full Text Available The rapid growth in the number of individuals living with Intellectual and Developmental Disabilities (IDD along with their increased longevity present challenges to those concerned about health and well-being of this unique population. While much is known about health promotion and disease prevention in the general geriatric population, far less is known about those in older adults with IDD. Effective and efficient health promotion and disease prevention strategies need to be developed and implemented for improving the health and quality of life of older adults living with IDD. This is considered to be challenging given the continued shrinkage in the overall health care and welfare system services due to the cut in the governmental budget in some of the western countries. The ideal health promotion and disease prevention strategies for older adults with IDD should be tailored to the individuals’ health risks, address primary and secondary disease prevention and prevent avoidable impairments that cause premature institutionalization. Domains of intervention should include cognitive, mental and physical health, accommodations, workplace considerations, assistive technology, recreational activities and nutrition.

  5. Measurement of 24-hr whole-body retention of Tc-99mMDP with a thyroid uptake probe: quantitative assessment of metabolic and metastatic bone diseases

    International Nuclear Information System (INIS)

    Seto, H.; Futatsuya, R.; Kamei, T.; Furumoto, N.; Ishizaki, Y.; Hada, M.; Kakishita, M.

    1983-01-01

    A new method for measurement of 24-hr whole body retention (WBR) of Tc-99mMDP, using a thyroid uptake probe was established and its clinical significance was evaluated in 102 patients with various bone disorders, including metabolic and metastatic bone diseases, aged above 20 years old. Reproducibility of 24-hr WBR in 10 patients was very good (r=0.996). The 24-hr WBR of Tc-99mMDP in the normal subjects was 30.4 +- 4.6%. The WBR values of chronic renal failure, metastatic bone disease and hyperthyroidism groups were 98.4 +- 3.0, 44.0 +- 8.0, 40,6 +- 6.3% respectively, which were significantly higher (p < 0.001). However the WBR of steroid-induced osteoporotic group was significantly lower (17.3 +- 5.4%) as compared with the normal group (p < 0.001). Based on these results the method is simple, reproducible and accurate to measure 24-hr WBR of Tc-99mMDP. Quantification of WBR is of great clinical value to diagnose metabolic bone disease and to follow-up metabolic and metastatic bone disease after treatment

  6. Doença óssea em Mieloma Múltiplo Bone disease in Multiple Myeloma

    Directory of Open Access Journals (Sweden)

    Vania T. M. Hungria

    2007-03-01

    Full Text Available As principais manifestações clínicas do mieloma múltiplo estão relacionadas à destruição óssea. Esta doença óssea pode levar a fraturas patológicas, compressão da medula espinhal, hipercalcemia e dor, sendo uma das principais causas de morbidade e mortalidade. Estas complicações resultam do desequilíbrio da reabsorção e formação óssea, decorrente do aumento da atividade osteoclástica. Este aumento é mediado pela liberação de fatores ativadores dos osteoclastos, que são produzidos no microambiente da medula óssea por células tumorais e não tumorais. Os bisfosfonatos são inibidores específicos da atividade osteoclástica e são eficazes no tratamento da hipercalcemia associada às neoplasias malignas e podem reduzir o aparecimento de complicações esqueléticas. Estudos recentes identificaram novas moléculas como o receptor de ativação nuclear kappa B (RANK, seu ligante (RANKL, osteoprotegerina (OPG, e a proteína inflamatória dos macrófagos-1alfa, que estão envolvidas na ativação e diferenciação dos osteoclastos, enquanto que a proteína dikkopf-1 inibe a formação óssea osteoblástica. Estas novas moléculas parecem não só interferir na biologia da destruição óssea do mieloma, mas também com a sobrevida e crescimento tumoral, sendo novos alvos para o desenvolvimento de drogas antimieloma. Estudos recentes com anticorpo monoclonal anti-RANKL são promissores. O tratamento da doença óssea do mieloma múltiplo inclui principalmente o uso de bisfosfonatos, radioterapia e procedimentos cirúrgicos.The major clinical manifestation of multiple myeloma is related to osteolytic bone destruction. Bone disease can lead to pathologic fractures, spinal cord compression, hypercalcemia, and pain, and is a major cause of morbidity and mortality. These complications result from asynchronous bone turnover wherein increased osteoclastic bone resorption is not accompanied by a comparable increase in bone formation

  7. Early Diagnosis of Avascular Necrosis of Bone Following Renal Transplantation By Bone Scan

    International Nuclear Information System (INIS)

    Shin, Hyun Ho; Kim, Han Su; Ihn, Chun Gyoo; Kim, Myung Jae

    1982-01-01

    Avascular necrosis of bone has become a well-recognized complication of renal transplantation. While preexisting metabolic bone disease, especially hyperparathyroidism, and metabolic disturbances induced by steroids have been implicated as etiological factors, the pathogenesis is controversial. The diagnosis of avascular necrosis of bone had been based on a history of joint pain and radiographic demonstration of bone necrosis. Recently the bone scan using 99m Tc-methylene diphosphonate is helpful in determining the early stage of bone necrosis. We report two cases of avascular necrosis of femur head, of which diagnosis was made by the bone scan using 99m Tc-methylene diphosphonate.

  8. Context Matters: Distinct Disease Outcomes as a Result of Crebbp Hemizygosity in Different Mouse Bone Marrow Compartments.

    Directory of Open Access Journals (Sweden)

    Ting Zhou

    Full Text Available Perturbations in CREB binding protein (CREBBP are associated with hematopoietic malignancies, including myelodysplastic syndrome (MDS. Mice hemizygous for Crebbp develop myelodysplasia with proliferative features, reminiscent of human MDS/myeloproliferative neoplasm-unclassifiable (MDS/MPN-U, and a proportion goes on to develop acute myeloid leukemia (AML. We have also shown that the Crebbp+/- non-hematopoietic bone marrow microenvironment induces excessive myeloproliferation of wild-type cells. We now report that transplantation of unfractionated Crebbp+/- bone marrow into wild-type recipients resulted in either early-onset AML or late-onset MDS and MDS/MPN-U. In contrast, purified Lin-Sca-1+c-Kit++ cells primarily gave rise to MDS with occasional transformation to AML. Furthermore, Crebbp+/- common myeloid progenitors and granulocyte/macrophage progenitors could trigger skewed myelopoiesis, myelodysplasia and late-onset AML. Surprisingly, the phenotypically abnormal cells were all of wild-type origin. MDS, MPN and AML can thus all be transferred from Crebbp+/- BM to wild-type hosts but fractionated bone marrow does not recapitulate the full disease spectrum of whole bone marrow, indicating that not only mutational status but also cellular context contribute to disease outcome. This has important consequences for structuring and interpreting future investigations into the underlying mechanisms of myeloid malignancies as well as for their treatment.

  9. Long-term consequences of developmental vascular defects on retinal vessel homeostasis and function in a mouse model of Norrie disease.

    Science.gov (United States)

    Beck, Susanne C; Feng, Yuxi; Sothilingam, Vithiyanjali; Garcia Garrido, Marina; Tanimoto, Naoyuki; Acar, Niyazi; Shan, Shenliang; Seebauer, Britta; Berger, Wolfgang; Hammes, Hans-Peter; Seeliger, Mathias W

    2017-01-01

    Loss of Norrin signalling due to mutations in the Norrie disease pseudoglioma gene causes severe vascular defects in the retina, leading to visual impairment and ultimately blindness. While the emphasis of experimental work so far was on the developmental period, we focus here on disease mechanisms that induce progression into severe adult disease. The goal of this study was the comprehensive analysis of the long-term effects of the absence of Norrin on vascular homeostasis and retinal function. In a mouse model of Norrie disease retinal vascular morphology and integrity were studied by means of in vivo angiography; the vascular constituents were assessed in detailed histological analyses using quantitative retinal morphometry. Finally, electroretinographic analyses were performed to assess the retinal function in adult Norrin deficient animals. We could show that the primary developmental defects not only persisted but developed into further vascular abnormalities and microangiopathies. In particular, the overall vessel homeostasis, the vascular integrity, and also the cellular constituents of the vascular wall were affected in the adult Norrin deficient retina. Moreover, functional analyses indicated to persistent hypoxia in the neural retina which was suggested as one of the major driving forces of disease progression. In summary, our data provide evidence that the key to adult Norrie disease are ongoing vascular modifications, driven by the persistent hypoxic conditions, which are ineffective to compensate for the primary Norrin-dependent defects.

  10. Controlled release pharmaceutical composition useful for the treatment of diseases and conditions affecting metabolism and/or structural integrity of cartilage and/or bone in male comprises strontium salt

    DEFF Research Database (Denmark)

    2004-01-01

    , hyperparathyroidism, periarticular erosions in rheumatoid arthritis, osteodystrophy, myositis ossificans, Bechterew's disease, osteolytic lesions produced by bone metastasis, bone pain due to bone metastasis, bone loss due to sex steroid hormone deficiency, bone abnormalities due to steroid hormone treatment, bone...

  11. Paget's disease of jaw bones as primary manifestation: A case report of a proper diagnosis made by general dentist.

    Science.gov (United States)

    Campolongo, Martin G; Cabras, Marco; Bava, Luca; Arduino, Paolo G; Carbone, Mario

    2018-06-01

    To present a case of early diagnosis mandibular Paget's disease of bone (PDB), recognised by a general dentist. PDB is responsible of rapid bone resorption and disorganised bone formation. The patient was a 72-year-old female patient complaining of dental malposition and blatant prognathism. Clinicians should consider PDB in differential diagnosis for an elderly patient undergoing unexplained alteration in face profile and occlusion. © 2018 John Wiley & Sons A/S and The Gerodontology Association. Published by John Wiley & Sons Ltd.

  12. Samarium-153 Oksabifor in the treatment of metastatic bone disease

    International Nuclear Information System (INIS)

    Solodyannikova, O.; Voit, N.; Sukach, G.; Sagan, D.

    2015-01-01

    Full text of publication follows. Aim. Bone metastases (BM) are one of the most common complications of solid cancers. Frequency of metastatic bone breast cancer (BC) at different stages of the disease ranges from 47 to 85%, for prostate cancer (PC) - from 33 to 85%, for lung cancer - from 30 to 60%, kidneys - from 33 to 40%, thyroid cancer - from 28 to 60%. Initial stages of BM are often clinically asymptomatic, but later manifested with fractures and pain which greatly reduces patients' quality of life. In world practice for palliative treatment of BM are widely used isotopes 32 P, 89 Sr, 186 Re, 188 Re, 153 Sm, and 177 Lu. Materials and methods. The results of examination and treatment with 153 Sm of 25 BM patients were analyzed. Among them 5 men and 20 women, mean age 61.2 ± 7.9 (min - 51.1, max 73.0). In 4 patients PC was diagnosed, 2 - lung cancer, 17 - BC, 1 - kidney cancer, 1 - cervical cancer. All patients after preliminary survey (bone scan, blood count, blood biochemical analysis) have got administration of 153 Sm Oksabifor in doses of 1 mCi (37 MBq) per 1 kg of weight. To determine the dynamics of BM re-scan with 99m Tc Tehnefor carried out in 1.5 and 3 months after starting treatment. Results. Reduction of pain intensity appeared at 6 + 4.6 days (min 2, max-18). There was a decline consumption of analgetics. According to the assessment of bone pain scales and efficiency, we can say that the therapy was effective and 90% of patients have got pain relief for 3 months and only in 2 patients pain-free period lasted 70 days. Quality of life was assessed by Karnofsky scale and improved statistically significantly. Most patients return to normal life. Only one patient's quality of life did not change (remained at 50%) and one - has changed slightly (from 50% to 60% on the Karnofsky scale). However, this is due to progression of primary disease and not related to pain symptoms. According to our data, 10 patients had stabilization process, in 15

  13. Bone island and leprosy

    International Nuclear Information System (INIS)

    Carpintero, P.; Garcia-Frasquet, A.; Tarradas, E.; Logrono, C.; Carrascal, A.; Carreto, A.

    1998-01-01

    Objective. To determine the incidence of bone islands in leprosy patients. Design. X-rays of feet and hands of patients with Hansen's disease (leprosy) were reviewed retrospectively. A second group of related age- and sex-matched patients who did not have Hansen's disease was used for control purposes. Controls had undergone hand or foot X-rays during diagnosis of other pathologies. The patients with Hansen's disease were compared with the control group, and were also analyzed as subgroups with different types of leprosy. The results were subjected to statistical analysis. Patients. Ninety patients with Hansen's disease were randomly selected for this study. Patients who had had ulcers on hands or feet were excluded from the study. Results and conclusions. Bone islands were demonstrated in 20 patients with Hansen's disease; no bone islands were observed in the controls. This was statistically significant (P<0.01). Bone islands were only seen in patients with lepromatous leprosy and borderline types but were not demonstrated in patients with tuberculoid leprosy. There was also a statistically significant relationship for a disease duration of 15 years or more. The cause of this raised incidence of enostosis in leprosy patients is not clear, but there may be a genetic predisposition in patients with leprosy, or it may be a side effect of leprosy, especially the lepromatous form. (orig.)

  14. Cardiovascular diseases and diabetes as economic and developmental challenges in Africa.

    Science.gov (United States)

    Kengne, Andre Pascal; June-Rose McHiza, Zandile; Amoah, Albert George Baidoe; Mbanya, Jean-Claude

    2013-01-01

    Current estimates and projections suggest that the burden of cardiovascular diseases (CVDs), diabetes and related risk factors in African countries is important, somewhat unique and rapidly growing. Various segments of the population are affected; however, the group mostly affected is young adults residing in urban areas, and increasingly those in the low socioeconomic strata. The African milieu/environment is compounded by weak health systems, which are unable to cope with the looming double burden of communicable and chronic non-communicable diseases. This review discusses the economic and developmental challenges posed by CVDs and diabetes in countries in Africa. Using several lines of evidence, we demonstrate that the cost of care for major CVDs and diabetes is beyond the coping capacities of individuals, households, families and governments in most African countries. We have reviewed modeling studies by the International Diabetes Federation (IDF) and other major international agencies on the current and projected impact that CVDs and diabetes have on the economy and development of countries in the region. Locally, appropriate strategies to limit the impact of the conditions on the economies and development of countries in Africa are suggested and discussed. These include monitoring diseases and risk factors, and primordial, primary and secondary preventions implemented following a life-course perspective. Structural, logistic, human capacity and organizational challenges to be surmounted during the implementations of these strategies will be reviewed. © 2013.

  15. Gut microbiome and bone.

    Science.gov (United States)

    Ibáñez, Lidia; Rouleau, Matthieu; Wakkach, Abdelilah; Blin-Wakkach, Claudine

    2018-04-11

    The gut microbiome is now viewed as a tissue that interacts bidirectionally with the gastrointestinal, immune, endocrine and nervous systems, affecting the cellular responses in numerous organs. Evidence is accumulating of gut microbiome involvement in a growing number of pathophysiological processes, many of which are linked to inflammatory responses. More specifically, data acquired over the last decade point to effects of the gut microbiome on bone mass regulation and on the development of bone diseases (such as osteoporosis) and of inflammatory joint diseases characterized by bone loss. Mice lacking a gut microbiome have bone mass alteration that can be reversed by gut recolonization. Changes in the gut microbiome composition have been reported in mice with estrogen-deficiency osteoporosis and have also been found in a few studies in humans. Probiotic therapy decreases bone loss in estrogen-deficient animals. The effect of the gut microbiome on bone tissue involves complex mechanisms including modulation of CD4 + T cell activation, control of osteoclastogenic cytokine production and modifications in hormone levels. This complexity may contribute to explain the discrepancies observed betwwen some studies whose results vary depending on the age, gender, genetic background and treatment duration. Further elucidation of the mechanisms involved is needed. However, the available data hold promise that gut microbiome manipulation may prove of interest in the management of bone diseases. Copyright © 2018 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  16. Developmental origins of adult health and disease: the role of periconceptional and foetal nutrition.

    Science.gov (United States)

    McMillen, I Caroline; MacLaughlin, Severence M; Muhlhausler, Beverly S; Gentili, Sheridan; Duffield, Jaime L; Morrison, Janna L

    2008-02-01

    The 'developmental origins of adult health and disease' hypothesis stated that environmental factors, particularly maternal undernutrition, act in early life to programme the risks for adverse health outcomes, such as cardiovascular disease, obesity and the metabolic syndrome in adult life. Early physiological tradeoffs, including activation of the foetal hypothalamo-pituitary-adrenal (HPA) axis, confer an early fitness advantage such as foetal survival, while incurring delayed health costs. We review the evidence that such tradeoffs are anticipated from conception and that the periconceptional nutritional environment can programme the developmental trajectory of the stress axis and the systems that maintain and regulate arterial blood pressure. There is also evidence that restriction of placental growth and function, results in an increased dependence of the maintenance of arterial blood pressure on the sequential recruitment of the sympathetic nervous system and HPA axis. While the 'early origins of adult disease' hypothesis has focussed on the impact of maternal undernutrition, an increase in maternal nutritional intake and in maternal body mass intake has become more prevalent in developed countries. Exposure to overnutrition in foetal life results in a series of central and peripheral neuroendocrine responses that in turn programme development of the fat cell and of the central appetite regulatory system. While the physiological responses to foetal undernutrition result in the physiological trade off between foetal survival and poor health outcomes that emerge after reproductive senescence, exposure to early overnutrition results in poor health outcomes that emerge in childhood and adolescence. Thus, the effects of early overnutrition can directly impact on reproductive fitness and on the health of the next generation. In this context, the physiological responses to relative overnutrition in early life may directly contribute to an intergenerational cycle of

  17. Markers of bone metabolism are affected by renal function and growth hormone therapy in children with chronic kidney disease.

    Science.gov (United States)

    Doyon, Anke; Fischer, Dagmar-Christiane; Bayazit, Aysun Karabay; Canpolat, Nur; Duzova, Ali; Sözeri, Betül; Bacchetta, Justine; Balat, Ayse; Büscher, Anja; Candan, Cengiz; Cakar, Nilgun; Donmez, Osman; Dusek, Jiri; Heckel, Martina; Klaus, Günter; Mir, Sevgi; Özcelik, Gül; Sever, Lale; Shroff, Rukshana; Vidal, Enrico; Wühl, Elke; Gondan, Matthias; Melk, Anette; Querfeld, Uwe; Haffner, Dieter; Schaefer, Franz

    2015-01-01

    The extent and relevance of altered bone metabolism for statural growth in children with chronic kidney disease is controversial. We analyzed the impact of renal dysfunction and recombinant growth hormone therapy on a panel of serum markers of bone metabolism in a large pediatric chronic kidney disease cohort. Bone alkaline phosphatase (BAP), tartrate-resistant acid phosphatase 5b (TRAP5b), sclerostin and C-terminal FGF-23 (cFGF23) normalized for age and sex were analyzed in 556 children aged 6-18 years with an estimated glomerular filtration rate (eGFR) of 10-60 ml/min/1.73 m2. 41 children receiving recombinant growth hormone therapy were compared to an untreated matched control group. Standardized levels of BAP, TRAP5b and cFGF-23 were increased whereas sclerostin was reduced. BAP was correlated positively and cFGF-23 inversely with eGFR. Intact serum parathormone was an independent positive predictor of BAP and TRAP5b and negatively associated with sclerostin. BAP and TRAP5B were negatively affected by increased C-reactive protein levels. In children receiving recombinant growth hormone, BAP was higher and TRAP5b lower than in untreated controls. Sclerostin levels were in the normal range and higher than in untreated controls. Serum sclerostin and cFGF-23 independently predicted height standard deviation score, and BAP and TRAP5b the prospective change in height standard deviation score. Markers of bone metabolism indicate a high-bone turnover state in children with chronic kidney disease. Growth hormone induces an osteoanabolic pattern and normalizes osteocyte activity. The osteocyte markers cFGF23 and sclerostin are associated with standardized height, and the markers of bone turnover predict height velocity.

  18. The negative bone effects of the disease and of chronic corticosteroid treatment in premenopausal women affected by rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    A. Fassio

    2016-09-01

    Full Text Available Osteoporosis is a well-known extra-articular complication in rheumatoid arthritis (RA. The chronic corticosteroid treatment, the functional impairment associated with RA and the disease itself appear to be the most relevant determinants. Most of the previous studies involved postmenopausal women, in whom the estrogenic deficiency might amplify the negative effect towards bone of both RA and corticosteroid therapy. We decided to evaluate bone health in a cohort of premenopausal RA patients. The study population includes 47 premenopausal women attending our outpatient clinic for RA and twice as many healthy age-matched control women selected from the hospital personnel. The bone density at the spine and femoral neck were significantly lower in patients with RA as compared with controls. When spine bone mineral density (BMD values were adjusted for the cumulative glucocorticoid (GC dose alone and for the cumulative GC dose plus body mass index (BMI the mean differences between two groups decreased but they remained statistically significant. We found no difference when the spine BMD was adjusted for cumulative GC dose, BMI and health assessment questionnaire. The difference in femoral neck BMD remained statistically significant also after all the same adjustments. In conclusion, our study shows that a BMD deficiency is frequent also in premenopausal women affected by RA, especially at femoral site and that the main determinants of this bone loss are not only the disease-related weight loss, corticosteroid therapy and functional impairment, but also the systemic effects of the disease itself.

  19. Partial resolution of bone lesions. A child with severe combined immunodeficiency disease and adenosine deaminase deficiency after enzyme-replacement therapy

    International Nuclear Information System (INIS)

    Yulish, B.S.; Stern, R.C.; Polmar, S.H.

    1980-01-01

    A child with severe combined immunodeficiency disease and adenosine deaminase deficiency, with characteristic bone dysplasia, was treated with transfusions of frozen irradiated RBCs as a means of enzyme replacement. This therapy resulted in restoration of immunologic competence and partial resolution of the bone lesions. Although the natural history of these lesions without therapy is not known, enzyme-replacement therapy may have played a role in the resolution of this patient's bone lesions

  20. Genetic sharing with cardiovascular disease risk factors and diabetes reveals novel bone mineral density loci

    NARCIS (Netherlands)

    S. Reppe (Sjur); Y. Wang (Yunpeng); W.K. Thompson (Wesley K.); L.K. McEvoy (Linda K.); N.J. Schork (Nicholas); V. Zuber (Verena); M. Leblanc (Marissa); F. Bettella (Francesco); I.G. Mills (Ian G.); R.S. Desikan (Rahul S.); S. Djurovic (Srdjan); K.M. Gautvik (Kaare); A.M. Dale (Anders); O.A. Andreassen (Ole); K. Estrada Gil (Karol); U. Styrkarsdottir (Unnur); E. Evangelou (Evangelos); Y.-H. Hsu (Yi-Hsiang); E.L. Duncan (Emma); E.E. Ntzani (Evangelia); L. Oei (Ling); O.M.E. Albagha (Omar M.); N. Amin (Najaf); J.P. Kemp (John); D.L. Koller (Daniel); G. Li (Guo); C.-T. Liu (Ching-Ti); R.L. Minster (Ryan); A. Moayyeri (Alireza); L. Vandenput (Liesbeth); D. Willner (Dana); S.-M. Xiao (Su-Mei); L.M. Yerges-Armstrong (Laura); H.-F. Zheng (Hou-Feng); N. Alonso (Nerea); J. Eriksson (Joel); C.M. Kammerer (Candace); S. Kaptoge (Stephen); P.J. Leo (Paul); G. Thorleifsson (Gudmar); S.G. Wilson (Scott); J.F. Wilson (James F); V. Aalto (Ville); M. Alen (Markku); A.K. Aragaki (Aaron); T. Aspelund (Thor); J.R. Center (Jacqueline); Z. Dailiana (Zoe); C. Duggan; M. Garcia (Melissa); N. Garcia-Giralt (Natàlia); S. Giroux (Sylvie); G. Hallmans (Göran); L.J. Hocking (Lynne); L.B. Husted (Lise Bjerre); K. Jameson (Karen); R. Khusainova (Rita); G.S. Kim (Ghi Su); C. Kooperberg (Charles); T. Koromila (Theodora); M. Kruk (Marcin); M. Laaksonen (Marika); A.Z. Lacroix (Andrea Z.); S.H. Lee (Seung Hun); P.C. Leung (Ping C.); J.R. Lewis (Joshua); L. Masi (Laura); S. Mencej-Bedrac (Simona); T.V. Nguyen (Tuan); X. Nogues (Xavier); M.S. Patel (Millan); J. Prezelj (Janez); L.M. Rose (Lynda); S. Scollen (Serena); K. Siggeirsdottir (Kristin); G.D. Smith; O. Svensson (Olle); S. Trompet (Stella); O. Trummer (Olivia); N.M. van Schoor (Natasja); J. Woo (Jean); K. Zhu (Kun); S. Balcells (Susana); M.L. Brandi; B.M. Buckley (Brendan M.); S. Cheng (Sulin); C. Christiansen; C. Cooper (Charles); G.V. Dedoussis (George); I. Ford (Ian); M. Frost (Morten); D. Goltzman (David); J. González-Macías (Jesús); M. Kähönen (Mika); M. Karlsson (Magnus); E.K. Khusnutdinova (Elza); J.-M. Koh (Jung-Min); P. Kollia (Panagoula); B.L. Langdahl (Bente); W.D. Leslie (William D.); P. Lips (Paul); O. Ljunggren (Östen); R. Lorenc (Roman); J. Marc (Janja); D. Mellström (Dan); B. Obermayer-Pietsch (Barbara); D. Olmos (David); U. Pettersson-Kymmer (Ulrika); D.M. Reid (David); J.A. Riancho (José); P.M. Ridker (Paul); M.F. Rousseau (Francois); P.E. Slagboom (Eline); N.L.S. Tang (Nelson L.S.); R. Urreizti (Roser); W. Van Hul (Wim); J. Viikari (Jorma); M.T. Zarrabeitia (María); Y.S. Aulchenko (Yurii); M.C. Castaño Betancourt (Martha); E. Grundberg (Elin); L. Herrera (Lizbeth); T. Ingvarsson (Torvaldur); H. Johannsdottir (Hrefna); T. Kwan (Tony); R. Li (Rui); R.N. Luben (Robert); M.C. Medina-Gomez (Carolina); S.T. Palsson (Stefan Th); J.I. Rotter (Jerome I.); G. Sigurdsson (Gunnar); J.B.J. van Meurs (Joyce); D.J. Verlaan (Dominique); F.M. Williams (Frances); A.R. Wood (Andrew); Y. Zhou (Yanhua); T. Pastinen (Tomi); S. Raychaudhuri (Soumya); J.A. Cauley (Jane); D.I. Chasman (Daniel); G.R. Clark (Graeme); S.R. Cummings (Steven R.); P. Danoy (Patrick); E.M. Dennison (Elaine); R. Eastell (Richard); J.A. Eisman (John); V. Gudnason (Vilmundur); A. Hofman (Albert); R.D. Jackson (Rebecca); G. Jones (Graeme); J.W. Jukema (Jan Wouter); K.T. Khaw; T. Lehtimäki (Terho); Y. Liu (YongMei); M. Lorentzon (Mattias); E. McCloskey (Eugene); B.D. Mitchell (Braxton); K. Nandakumar (Kannabiran); G.C. Nicholson (Geoffrey); B.A. Oostra (Ben); M. Peacock (Munro); H.A.P. Pols (Huib); R.L. Prince (Richard); O. Raitakari (Olli); I.R. Reid (Ian); J. Robbins (John); P.N. Sambrook (Philip); P.C. Sham (Pak Chung); A.R. Shuldiner (Alan); F.A. Tylavsky (Frances); C.M. van Duijn (Cornelia); N.J. Wareham (Nicholas J.); L.A. Cupples (Adrienne); M.J. Econs (Michael); D.M. Evans (David); T.B. Harris (Tamara B.); A.W.C. Kung (Annie Wai Chee); B.M. Psaty (Bruce); J. Reeve (Jonathan); T.D. Spector (Timothy); E.A. Streeten (Elizabeth); M.C. Zillikens (Carola); U. Thorsteinsdottir (Unnur); C. Ohlsson (Claes); D. Karasik (David); J.B. Richards (Brent); M.A. Brown (Matthew); J-A. Zwart (John-Anker); A.G. Uitterlinden (André); S.H. Ralston (Stuart); J.P.A. Ioannidis (John P.A.); D.P. Kiel (Douglas P.); F. Rivadeneira Ramirez (Fernando)

    2015-01-01

    textabstractBone Mineral Density (BMD) is a highly heritable trait, but genome-wide association studies have identified few genetic risk factors. Epidemiological studies suggest associations between BMD and several traits and diseases, but the nature of the suggestive comorbidity is still unknown.

  1. [Consensus statement: recommendations for the management of metabolic bone disease in human immunodeficiency virus patients].

    Science.gov (United States)

    Martínez, Esteban; Jódar Gimeno, Esteban; Reyes García, Rebeca; Carpintero, Pedro; Casado, José Luis; Del Pino Montes, Javier; Domingo Pedrol, Pere; Estrada, Vicente; Maalouf, Jorge; Negredo, Eugenia; Ocampo, Antonio; Muñoz-Torres, Manuel

    2014-04-01

    To provide practical recommendations for the evaluation and treatment of metabolic bone disease in human immunodeficiency virus (HIV) patients. Members of scientific societies related to bone metabolism and HIV: Grupo de Estudio de Sida (GeSIDA), Sociedad Española de Endocrinología y Nutrición (SEEN), Sociedad Española de Investigación Ósea y del Metabolismo Mineral (SEIOMM), and Sociedad Española de Fractura Osteoporótica (SEFRAOS). A systematic search was carried out in PubMed, and papers in English and Spanish with a publication date before 28 May 2013 were included. Recommendations were formulated according to GRADE system (Grading of Recommendations, Assessment, Development, and Evaluation) setting both their strength and the quality of supporting evidence. Working groups were established for each major part, and the final resulting document was later discussed in a face-to-face meeting. All the authors reviewed the final written document and agreed with its content. The document provides evidence-based practical recommendations on the detection and treatment of bone disease in HIV-infected patients. Copyright © 2013 Elsevier España, S.L. y Sociedad Española de Enfermedades Infecciosas y Microbiología Clínica. All rights reserved.

  2. Bone scintigraphy and radiology in untreated Paget's disease

    International Nuclear Information System (INIS)

    Vellenga, C.J.L.R.; Pauwels, E.K.J.; Bijvost, O.L.M.

    1985-01-01

    This study is based on 107 patients with untreated Paget's disease of bone. Scintigraphy was performed 3 hours after 20 mCi Tc-99m-Sn-EHDP, using a Toshiba 204 or 404 gamma camera. In each patient multiple spot films of the total skeleton were made and evaluated according to a six-point scale. Moreover, in 42 patients the ratio of uptake in a lesion over comparable normal bone was measured using an MDS Trinary computer. Radiographs were made only of affected parts and evaluated according to a three-point scale. In 59 (16%) of the 373 lesions found on the scintigam no radiographic abnormalities were found. These roentgen-negative lesions generally show rather low scintigraphic uptake and are mostly asymptomatic. There exists a clear correlation between scintigraphic uptake and radiological deformity: the scintigraphic scores 5 and 6 occur in only 24% of the ro-grade 1 lesions, but in 65% of the grade 3 lesions; after computer evaluation the mean ratio in grade 1 and 3 lesions is 3.0 and 6.5 resp. (p<0.01). There also appears to be a correlation between scint. uptake and symptomatology: typical Pagetic pain is present in 10% of scint. score 2 and 3 lesions, but in 40% of score 5 and 6 lesions; the mean computer measured scint. ratio is 4.0 for asymptomatic and 7.5 for painful lesions (p<0.001)

  3. The effect of bone marrow aspirate, bone graft and collagen composites on fixation of bone implants

    DEFF Research Database (Denmark)

    Babiker, Hassan; Ding, Ming; Overgaard, Søren

    2007-01-01

     Introduction: Replacement of extensive local bone loss especially in revision joint arthroplasties is a significant clinical challenge. Autogenous and allogenic cancellous bone grafts have been the gold standard in reconstructive orthopaedic surgery, but it is well known that there is morbidity...... associated with harvesting of autogenous bone graft and limitations in the quantity of bone available. Disadvantages of allograft include the risk of bacterial or viral contamination and non union as well as the potential risk of disease transmission. Alternative options are attractive and continue...... to be sought. Hydroxyapatite and collagen composites have the potential in mimicking and replacing skeletal bones. Aim: This study attempted to determine the effect of hydroxyapatite/collagen composites in the fixation of bone implants. The composites used in this study is produced by Institute of Science...

  4. MRI bone marrow findings in 63 patients with type I Gaucher's disease

    Energy Technology Data Exchange (ETDEWEB)

    Poll, L.W. [Berufsgenossenschaftliche Unfallklinik Duisburg GmbH (Germany). Abt. Radiologie; Willers, R. [Duesseldorf Univ. (Germany). Zentrum fuer Information, Kommunikation und Medientechnologie; Haeussinger, D. [Universitaetsklinikum Duesseldorf (Germany). Klinik fuer Gastroenterologie, Hepatologie und Infektiologie; Moedder, U. [Universitaetsklinikum Duesseldorf (Germany). Inst. fuer Radiologie; Dahl, S. vom [St.-Franziskus-Hospital Koeln, Akademisches Lehrkrankenhaus der Koeln Univ. (Germany). Klinik fuer Innere Medizin.

    2010-11-15

    Purpose: To determine whether MR bone marrow findings in Gaucher patients may help to identify patients at high risk of developing severe Gaucher bone complications exemplified by avascular necrosis (AVN) of the femoral head. Materials and Methods: MR images were obtained in 63 Type I Gaucher patients through a standard protocol using coronal T1 and T2-weighted sequences of the lower extremities. The location and extent of infiltrated marrow was established using a semi-quantitative MRI scoring method (Duesseldorf Gaucher score, DGS) and the morphological pattern of bone marrow involvement determined (whether homogeneous type A or non-homogeneous type B). The active marrow process with bone edema and AVN of the femoral head were also analyzed. Results: Bone marrow involvement was observed in femoral sites more than in tibial sites. A high DGS was significantly correlated with type B morphology and femoral AVN (both p < 0.0001). Splenectomized patients showed a significantly higher Duesseldorf Gaucher score and type B morphology than non-splenectomized patients (both p < 0.05). AVN was seen in 46 % of patients with type B morphology versus 3 % in type A morphology (p < 0.0001). DGS and morphology of bone marrow involvement were not significantly correlated with active marrow processes. Conclusion: Type B marrow morphology and extensive marrow packing were significantly associated with AVN of the femoral head (both p < 0.0001). These patterns are considered predictive and may be employed in a disease management context to alert physicians to the need for urgent therapeutic measures. (orig.)

  5. Transgenerational developmental programming.

    Science.gov (United States)

    Aiken, Catherine E; Ozanne, Susan E

    2014-01-01

    The concept of developmental programming suggests that the early life environment influences offspring characteristics in later life, including the propensity to develop diseases such as the metabolic syndrome. There is now growing evidence that the effects of developmental programming may also manifest in further generations without further suboptimal exposure. This review considers the evidence, primarily from rodent models, for effects persisting to subsequent generations, and evaluates the mechanisms by which developmental programming may be transmitted to further generations. In particular, we focus on the potential role of the intrauterine environment in contributing to a developmentally programmed phenotype in subsequent generations. The literature was systematically searched at http://pubmed.org and http://scholar.google.com to identify published findings regarding transgenerational (F2 and beyond) developmental programming effects in human populations and animal models. Transmission of programming effects is often viewed as a form of epigenetic inheritance, either via the maternal or paternal line. Evidence exists for both germline and somatic inheritance of epigenetic modifications which may be responsible for phenotypic changes in further generations. However, there is increasing evidence for the role of both extra-genomic components of the zygote and the interaction of the developing conceptus with the intrauterine environment in propagating programming effects. The contribution of a suboptimal reproductive tract environment or maternal adaptations to pregnancy may be critical to inheritance of programming effects via the maternal line. As the effects of age exacerbate the programmed metabolic phenotype, advancing maternal age may increase the likelihood of developmental programming effects being transmitted to further generations. We suggest that developmental programming effects could be propagated through the maternal line de novo in generations

  6. Markers of bone metabolism are affected by renal function and growth hormone therapy in children with chronic kidney disease

    DEFF Research Database (Denmark)

    Doyon, Anke; Fischer, Dagmar Christiane; Bayazit, Aysun Karabay

    2015-01-01

    Objectives: The extent and relevance of altered bone metabolism for statural growth in children with chronic kidney disease is controversial. We analyzed the impact of renal dysfunction and recombinant growth hormone therapy on a panel of serum markers of bone metabolism in a large pediatric...... turnover state in children with chronic kidney disease. Growth hormone induces an osteoanabolic pattern and normalizes osteocyte activity. The osteocyte markers cFGF23 and sclerostin are associated with standardized height, and the markers of bone turnover predict height velocity......./min/ 1.73m2. 41 children receiving recombinant growth hormone therapy were compared to an untreated matched control group. Results: Standardized levels of BAP, TRAP5b and cFGF-23 were increased whereas sclerostin was reduced. BAP was correlated positively and cFGF-23 inversely with eGFR. Intact serum...

  7. An intelligent medical system for diagnosis of bone diseases

    Energy Technology Data Exchange (ETDEWEB)

    Hatzilygeroudis, I [University of Patras, School of Engineering, Department of Computer Engineering and Informatics, 26500 Patras, Greece (Greece); Vassilakos, P J [Regional University Hospital of Patras, Department of Nuclear Medicine, Patras Greece (Greece); Tsakalidis, A [Computer Technology Institute, P.O. Box 1122, 26110 Patras, Greece (Greece)

    1994-12-31

    In this paper, aspects of the design of an intelligent medical system for diagnosis of bone diseases that can be detected by scintigraphic images are presented. The system comprises three major parts: a user interface (UI), a database management system (DBMS), and an expert system (ES). The DBMS is used for manipulation of various patient data. A number of patient cases are selected as prototype and stored in separate database. Diagnosis is performed via the ES, called XBONE, based on patient data. Knowledge is represented via an integrated formalism that combines production rules and a neural network. This results in better representation, and facilitates knowledge acquisition and maintenance. (authors). 10 refs., 2 figs.

  8. Bone tissue engineering and regeneration: from discovery to the clinic--an overview.

    Science.gov (United States)

    O'Keefe, Regis J; Mao, Jeremy

    2011-12-01

    A National Institutes of Health sponsored workshop "Bone Tissue Engineering and Regeneration: From Discovery to the Clinic" gathered thought leaders from medicine, science, and industry to determine the state of art in the field and to define the barriers to translating new technologies to novel therapies to treat bone defects. Tissue engineering holds enormous promise to improve human health through prevention of disease and the restoration of healthy tissue functions. Bone tissue engineering, similar to that for other tissues and organs, requires integration of multiple disciplines such as cell biology, stem cells, developmental and molecular biology, biomechanics, biomaterials science, and immunology and transplantation science. Although each of the research areas has undergone enormous advances in last decade, the translation to clinical care and the development of tissue engineering composites to replace human tissues has been limited. Bone, similar to other tissue and organs, has complex structure and functions and requires exquisite interactions between cells, matrices, biomechanical forces, and gene and protein regulatory factors for sustained function. The process of engineering bone, thus, requires a comprehensive approach with broad expertise. Although in vitro and preclinical animal studies have been pursued with a large and diverse collection of scaffolds, cells, and biomolecules, the field of bone tissue engineering remains fragmented up to the point that a clear translational roadmap has yet to emerge. Translation is particularly important for unmet clinical needs such as large segmental defects and medically compromised conditions such as tumor removal and infection sites. Collectively, manuscripts in this volume provide luminary examples toward identification of barriers and strategies for translation of fundamental discoveries into clinical therapeutics. © Mary Ann Liebert, Inc.

  9. Bone Tissue Engineering and Regeneration: From Discovery to the Clinic—An Overview

    Science.gov (United States)

    2011-01-01

    A National Institutes of Health sponsored workshop “Bone Tissue Engineering and Regeneration: From Discovery to the Clinic” gathered thought leaders from medicine, science, and industry to determine the state of art in the field and to define the barriers to translating new technologies to novel therapies to treat bone defects. Tissue engineering holds enormous promise to improve human health through prevention of disease and the restoration of healthy tissue functions. Bone tissue engineering, similar to that for other tissues and organs, requires integration of multiple disciplines such as cell biology, stem cells, developmental and molecular biology, biomechanics, biomaterials science, and immunology and transplantation science. Although each of the research areas has undergone enormous advances in last decade, the translation to clinical care and the development of tissue engineering composites to replace human tissues has been limited. Bone, similar to other tissue and organs, has complex structure and functions and requires exquisite interactions between cells, matrices, biomechanical forces, and gene and protein regulatory factors for sustained function. The process of engineering bone, thus, requires a comprehensive approach with broad expertise. Although in vitro and preclinical animal studies have been pursued with a large and diverse collection of scaffolds, cells, and biomolecules, the field of bone tissue engineering remains fragmented up to the point that a clear translational roadmap has yet to emerge. Translation is particularly important for unmet clinical needs such as large segmental defects and medically compromised conditions such as tumor removal and infection sites. Collectively, manuscripts in this volume provide luminary examples toward identification of barriers and strategies for translation of fundamental discoveries into clinical therapeutics. PMID:21902614

  10. Height and blood chemistry in adults with a history of developmental arsenic poisoning from contaminated milk powder

    DEFF Research Database (Denmark)

    Yorifuji, Takashi; Matsuoka, Kenichi; Grandjean, Philippe

    2017-01-01

    BACKGROUND: Arsenic poisoning interferes with bone metabolism in laboratory animal studies, and human studies suggest lowered bone mass density at elevated exposures. As the long-term consequences of developmental arsenic toxicity are poorly known, we carried out a clinical pilot study of survivors...... the unexposed participants (191 (44) U/L) (p=0.01). No other statistically significant difference was observed, and liver enzymes were within normal ranges. CONCLUSIONS: Adults who had suffered arsenic poisoning during infancy showed decreased height and elevated ALP that suggests abnormalities in bone...... metabolism possibly induced by arsenic incorporated in the bone matrix....

  11. Efficacy and safety of bisphosphonates in management of low bone density in inflammatory bowel disease

    Science.gov (United States)

    Yao, Liwei; Wang, Haiqing; Dong, Wenwei; Liu, Zhenxin; Mao, Haijiao

    2017-01-01

    Abstract This study aims to determine whether bisphosphonates are safe, as well as effective against bone mineral loss in inflammatory bowel disease (IBD). A computerized search of electronic databases from 1966 to 2016 was performed. Randomized controlled trials (RCTs) were included in this review to evaluate the role of bisphosphonates in the management of osteoporosis in IBD patients. A revised 7-point Jadad scale was used to evaluate the quality of each study. Overall, 13 RCTs and 923 patients met the inclusion criteria of this meta-analysis. The result showed that bisphosphonates decreased bone mass density (BMD) loss at the lumbar spine (P = 0.0002), reduced the risk of new fractures (P = 0.01), and retained the similar adverse events (P = 0.86). Bisphosphonates may provide protection and safety against bone mineral loss in IBD patients. PMID:28099343

  12. Increased technetium-99 m hydroxy diphosphonate soft tissue uptake on bone scintigraphy in chronic kidney disease patients with secondary hyperparathyroidism

    DEFF Research Database (Denmark)

    Enevoldsen, Lotte Hahn; Heaf, James Goya; Højgaard, Liselotte

    2017-01-01

    In bone scan patients with dialysis-treated chronic kidney disease (CKD) and hyperparathyroidism, soft tissue accumulation of technetium-99 m hydroxy/methylene diphosphonate (Tc-99 m-HDP/MDP) has been reported primarily in case reports and usually explained by hypercalcaemia and/or hyperphosphata......In bone scan patients with dialysis-treated chronic kidney disease (CKD) and hyperparathyroidism, soft tissue accumulation of technetium-99 m hydroxy/methylene diphosphonate (Tc-99 m-HDP/MDP) has been reported primarily in case reports and usually explained by hypercalcaemia and...... patients diagnosed with secondary hyperparathyroidism admitted for Tc-99 m-HDP bone scan. Baseline characteristics and mean concentrations of biochemical markers (including P-calcium and P-phosphate) taken 0-3 months prior to the bone scans were collected. Soft tissue uptake was detected on bone scans....../or hyperphosphataemia. As human vascular smooth muscle cells produce hydroxyapatite during cell culture with increased phosphate levels and as Tc-99 m-HDP/MDP primarily binds to hydroxyapatite, we hypothesized that soft tissue accumulation would be found in patients with hyperphosphataemia. We identified 63 CKD...

  13. Biology of Bone Tissue: Structure, Function, and Factors That Influence Bone Cells.

    Science.gov (United States)

    Florencio-Silva, Rinaldo; Sasso, Gisela Rodrigues da Silva; Sasso-Cerri, Estela; Simões, Manuel Jesus; Cerri, Paulo Sérgio

    2015-01-01

    Bone tissue is continuously remodeled through the concerted actions of bone cells, which include bone resorption by osteoclasts and bone formation by osteoblasts, whereas osteocytes act as mechanosensors and orchestrators of the bone remodeling process. This process is under the control of local (e.g., growth factors and cytokines) and systemic (e.g., calcitonin and estrogens) factors that all together contribute for bone homeostasis. An imbalance between bone resorption and formation can result in bone diseases including osteoporosis. Recently, it has been recognized that, during bone remodeling, there are an intricate communication among bone cells. For instance, the coupling from bone resorption to bone formation is achieved by interaction between osteoclasts and osteoblasts. Moreover, osteocytes produce factors that influence osteoblast and osteoclast activities, whereas osteocyte apoptosis is followed by osteoclastic bone resorption. The increasing knowledge about the structure and functions of bone cells contributed to a better understanding of bone biology. It has been suggested that there is a complex communication between bone cells and other organs, indicating the dynamic nature of bone tissue. In this review, we discuss the current data about the structure and functions of bone cells and the factors that influence bone remodeling.

  14. Mastoiditis and facial paralysis as initial manifestations of temporal bone systemic diseases - the significance of the histopathological examination.

    Science.gov (United States)

    Maniu, Alma Aurelia; Harabagiu, Oana; Damian, Laura Otilia; Ştefănescu, Eugen HoraŢiu; FănuŢă, Bogdan Marius; Cătană, Andreea; Mogoantă, Carmen Aurelia

    2016-01-01

    Several systemic diseases, including granulomatous and infectious processes, tumors, bone disorders, collagen-vascular and other autoimmune diseases may involve the middle ear and temporal bone. These diseases are difficult to diagnose when symptoms mimic acute otomastoiditis. The present report describes our experience with three such cases initially misdiagnosed. Their predominating symptoms were otological with mastoiditis, hearing loss, and subsequently facial nerve palsy. The cases were considered an emergency and the patients underwent tympanomastoidectomy, under the suspicion of otitis media with cholesteatoma, in order to remove a possible abscess and to decompress the facial nerve. The common features were the presence of severe granulation tissue filling the mastoid cavity and middle ear during surgery, without cholesteatoma. The definitive diagnoses was made by means of biopsy of the granulation tissue from the middle ear, revealing granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) in one case, middle ear tuberculosis and diffuse large B-cell lymphoma respectively. After specific associated therapy facial nerve functions improved, and atypical inflammatory states of the ear resolved. As a group, systemic diseases of the middle ear and temporal bone are uncommon, but aggressive lesions. After analyzing these cases and reviewing the literature, we would like to stress upon the importance of microscopic examination of the affected tissue, required for an accurate diagnosis and effective treatment.

  15. Femoral head vascularisation in Legg-Calve-Perthes disease: comparison of dynamic gadolinium-enhanced subtraction MRI with bone scintigraphy

    International Nuclear Information System (INIS)

    Lamer, Sylvie; Dorgeret, Sophie; Brillet, Pierre-Yves; Hassan, Max; Sebag, Guy H.; Khairouni, Abdeslam; Mazda, Keyvan; Bacheville, Eric; Pennecot, Georges F.; Bloch, Juliette

    2002-01-01

    Heading AbstractBackground. It has been reported that MRI using a dynamic gadolinium-enhanced subtraction technique can allow the early identification of ischaemia and the pattern of revascularisation in Legg-Calve-Perthes (LCP) disease with increased spatial and contrast resolution. Therefore, dynamic gadolinium-enhanced subtraction (DGS) MRI may be a possible non-ionising substitute for bone scintigraphy.Objective. The purpose of this prospective study was to compare DGS MRI and bone scintigraphy in the assessment of femoral head perfusion in LCP disease.Materials and methods. Twenty-six DGS MR images and bone scintigraphies of 25 hips in 23 children were obtained at different stages of LCP disease; three stage I, 12 stage II, six stage III and five stage IV (Waldenstroem classification). The extent of necrosis, epiphyseal revascularisation pathways (lateral pillar, medial pillar, and/or transphyseal perfusion) and metaphyseal changes were analysed.Results. Total agreement between both techniques was noted in the depiction of epiphyseal necrosis (kappa=1), and metaphyseal abnormalities (kappa=0.9). DGS MRI demonstrated better revascularisation in the lateral (kappa=0.62) and medial pillars (kappa=0.52). The presence of basal transphyseal reperfusion was more conspicuous with MRI.Conclusions. DGS MRI allows early detection of epiphyseal ischaemia and accurate analysis of the different revascularisation patterns. These changes are directly related to the prognosis of LCP disease and can aid therapeutic decision making. (orig.)

  16. Early Diagnosis of Avascular Necrosis of Bone Following Renal Transplantation By Bone Scan

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Hyun Ho; Kim, Han Su; Ihn, Chun Gyoo; Kim, Myung Jae [Kyung Hee University College of Medicine, Seoul (Korea, Republic of)

    1982-09-15

    Avascular necrosis of bone has become a well-recognized complication of renal transplantation. While preexisting metabolic bone disease, especially hyperparathyroidism, and metabolic disturbances induced by steroids have been implicated as etiological factors, the pathogenesis is controversial. The diagnosis of avascular necrosis of bone had been based on a history of joint pain and radiographic demonstration of bone necrosis. Recently the bone scan using {sup 99m}Tc-methylene diphosphonate is helpful in determining the early stage of bone necrosis. We report two cases of avascular necrosis of femur head, of which diagnosis was made by the bone scan using {sup 99m}Tc-methylene diphosphonate.

  17. New genetic associations in thiopurine-related bone marrow toxicity among inflammatory bowel disease patients.

    Science.gov (United States)

    Zabala, William; Cruz, Raquel; Barreiro-de Acosta, Manuel; Chaparro, María; Panes, Julián; Echarri, Ana; Esteve, Maria; Carpio, Daniel; Andreu, Montserrat; García-Planella, Esther; Domenech, Eugeni; Carracedo, Angel; Gisbert, Javier P; Barros, Francisco

    2013-04-01

    The toxicity related to thiopurine drug therapy for inflammatory bowel disease (IBD) varies widely among patients. Almost 15-30% of patients with IBD develop side effects during treatment, often bone marrow suppression. Several factors have been implicated in determining this toxicity, mainly individual genetic variation related to formation of active thiopurine metabolites. The aim was to identify genes involved in thiopurine-related myelosuppression. A two-stage investigation of 19,217 coding SNPs (cSNPs) was performed in a Spanish (Inflammatory Bowel Disease Group of Galicia [EIGA]) cohort of 173 IBD patients, 15 with bone marrow suppression. The top 20 cSNPs identified in the first stage with p ENEIDA) cohort (87 patients, 29 with bone marrow suppression). Several cSNPs showed a significant p-value in the allelic joint analysis (p-Cochran-Mantel-Haenszel test ≤2.55 × 10(-3)) despite no cSNP passing correction for multiple testing in the first cohort. Of note is rs3729961 in the gene IL6ST, a transducer signal chain shared by many cytokines including IL6 (p-value combined = 2.36 × 10(-4), odds ratio [95% CI]: 3.41 [1.71-6.78]). In addition, we detected association with rs3749598 in the FSTL5 gene that appears to interact with metalloproteases at the extracellular matrix level (p-value combined = 4.89 × 10(-4)), odds ratio (95% CI): 3.67 (1.68-8.01). We have identified IL6ST and FSLT5 as new bone marrow suppression susceptibility candidate genes after thiopurine treatment in IBD patients. This is the first report of variants associated with thiopurine-related myelosuppression that was identified by a genome-wide association study. Its validation awaits functional analyses and replication in additional studies. Original submitted 14 September 2012; Revision submitted 13 February 2013.

  18. Premature hair greying may predict reduced bone mineral density in Graves' disease.

    LENUS (Irish Health Repository)

    Leary, A C

    2012-02-03

    BACKGROUND: Premature hair greying has been associated with low bone mineral density (BMD), and it may be more frequent in Graves\\' disease. AIMS: To determine whether premature greying is associated with reduced BMD in women with Graves\\' disease and in control women, and to examine whether premature greying is more common in Graves\\' disease. METHODS: Premature greying (> 50% grey by 40 years) and BMD were determined in 44 women with a history of Graves\\' disease and 133 female controls referred for routine BMD measurement. Exclusion criteria included diseases or drugs known to affect BMD. RESULTS: Mean Z and T scores at the lumbar spine were significantly lower (P < 0.04) in subjects with premature greying than in those not prematurely grey among women with Graves\\' disease, but not among control women. Multiple regression confirmed this difference between Graves\\' and control women (P = 0.041). There were no differences at other measurement sites. Of Graves\\' patients, 36% were prematurely grey compared with 25% of control women (P = 0.14). CONCLUSION: Premature greying may be a weak marker for reduced BMD in women with a history of Graves\\' disease, but it is not a marker in normal women.

  19. Quantitative assessment of metabolic bone disease in rat models by dual tracer method

    Energy Technology Data Exchange (ETDEWEB)

    Ihara, Fumishige; Seto, Hikaru

    1989-05-01

    The usefulness of radionuclide techniques for early differential diagnosis of metabolic bone disease has been controversial. We tried to develop a new method to distinguish alterations in bone metabolism prior to radiologic changes, measuring 24-hr whole-body retention (WBR) and femoral uptake of two radiopharmaceuticals (/sup 47/Ca-chloride, /sup 99m/Tc-MDT). Control normal (C), osteoporosis (P), osteomalacia (M) and steroid-induced osteoporosis (S) were produced in 60 eight-week old Wistar male rats by means of dietary manipulation and steroid administration. Fine detail radiographs of the femurs and bone specimens were obtained over six weeks at two week intervals. Good correlation between WBR and femoral uptake of /sup 47/Ca was noted (r=0.86, p<0.01). WBR ratios of /sup 47/Ca were significantly higher in the M and S groups and were lower in the P group when compared to the C group throughout the study. WBR ratios of /sup 99m/Tc-MDP were significantly higher in the M group and were lower in the S group from the 2nd week. Fine detail radiographs analyzed by microdensitometry revealed significant osteopenia in the S, M and P groups from the 4th week. The dual tracer method was found to distinguish alterations in bone metabolism in the groups examined prior to detectable radiologic changes. (author).

  20. Chondrosarcoma of bone complicating Ollier's disease: Report of a favourable response to radiotherapy

    International Nuclear Information System (INIS)

    Le, A.; Ball, D.; Pitman, A.; Fox, R.; King, K.

    2003-01-01

    Because chondrosarcoma of bone is traditionally thought to be a radioresistant malignancy, it is usually managed surgically. We report a case of multifocal chondrosarcoma arising in Ollier's disease for which the patient declined surgery. He was given a course of radical radiotherapy that resulted in symptom palliation and a radiologically confirmed remission that not only palliated his symptoms but also enabled him to achieve his goal of pursuing his profession before he died, 16 months later of multifocal sarcomatous transformation and metastatic disease. It is concluded that in patients with inoperable chondrosarcoma, radiotherapy can provide palliative benefit. Copyright (2003) Blackwell Science Pty Ltd

  1. Constitutional bone impairment in Noonan syndrome.

    Science.gov (United States)

    Baldassarre, Giuseppina; Mussa, Alessandro; Carli, Diana; Molinatto, Cristina; Ferrero, Giovanni Battista

    2017-03-01

    Noonan syndrome (NS) is an autosomal dominant trait characterized by genotypic and phenotypic variability. It belongs to the Ras/MAPK pathway disorders collectively named Rasopathies or neurocardiofaciocutaneous syndromes. Phenotype is characterized by short stature, congenital heart defects, facial dysmorphisms, skeletal and ectodermal anomalies, cryptorchidism, mild to moderate developmental delay/learning disability, and tumor predisposition. Short stature and skeletal dysmorphisms are almost constant and several studies hypothesized a role for the RAS pathway in regulating bone metabolism. In this study, we investigated the bone quality assessed by phalangeal quantitative ultrasound (QUS) and the metabolic bone profiling in a group of patients with NS, to determine whether low bone mineralization is primary or secondary to NS characteristics. Thirty-five patients were enrolled, including 20 males (55.6%) and 15 females (44.5%) aged 1.0-17.8 years (mean 6.4 ± 4.5, median 4.9 years). Each patients was submitted to clinical examination, estimation of the bone age, laboratory assays, and QUS assessment. Twenty-five percent of the cohort shows reduced QUS values for their age based on bone transmission time. Bone measurement were adjusted for multiple factors frequently observed in NS patients, such as growth retardation, delayed bone age, retarded puberty, and reduced body mass index, potentially affecting bone quality or its appraisal. In spite of the correction attempts, QUS measurement indicates that bone impairment persists in nearly 15% of the cohort studied. Our results indicate that bone impairment in NS is likely primary and not secondary to any of the phenotypic traits of NS, nor consistent with metabolic disturbances. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

  2. Alert for bone alterations and low serum concentrations of vitamin D in patients with intestinal inflammatory disease

    Directory of Open Access Journals (Sweden)

    Lorete Maria da Silva Kotze

    Full Text Available Summary Background: Inflammatory bowel diseases (IBD, including Crohn's disease (CD and ulcerative colitis (UC, are characterized by chronic inflammation of the intestine that can reduce the absorption of nutrients such as vitamin D and calcium. Objective: To investigate bone alterations and serum levels of vitamin D in patients with IBD. Method: This was a cross-sectional study based on a review of medical records of patients from a private office in Curitiba, PR, Brazil. Serum levels of vitamin D and bone densitometry were measured at diagnosis of IBD. A total of 105 patients were included; 38 (58.4% with CD; 27 (41.6% with UC and 40 with irritable bowel syndrome (IBS as comparison group. Results: When compared to patients with UC, CD patients showed a higher prevalence of bone alterations, being 15.8% with osteoporosis and 36.8% with osteopenia. In UC, bone alterations occurred in 29.6% of cases, 3.7% with osteoporosis and 25.9% with osteopenia. As for vitamin D levels, among CD patients, 10.5% had vitamin deficiency, 65.8% insufficiency and 23.7% were sufficient. In UC, 7.4% of cases had deficiency, 74.1% insufficiency and 18.5% had sufficient serum levels of vitamin D. In the group with IBS, deficiency was observed in 17.5% of cases, insufficiency in 55% and sufficiency in 27.5% of them. There was no significant difference between groups. Conclusion: IBD patients have a high prevalence of bone changes, especially those with CD. Serum levels of vitamin D are below the recommended in all the evaluated groups.

  3. Magnetic resonance imaging in diffuse malignant bone marrow diseases

    Energy Technology Data Exchange (ETDEWEB)

    Nyman, R.; Rehn, S.; Glimelius, B.; Hagberg, H.; Hemmingsson, A.; Jung, B.; Simonsson, B.; Sundstroem, C.

    Twenty-four patients with malignant bone marrow involvement or polycythemia vera, 8 patients with reactive bone marrow and 7 healthy individuals were examined with spin-echo magnetic resonance imaging at 0.35 T and 0.5 T. Signs of an increased longitudinal relaxation time, T1, were found when normal bone marrow was replaced by malignant cells, polycythemia vera or reactive marrow. A shortened T1 was indicated in 4 patients in bone marrow regions treated by radiation therapy; the marrow was most likely hypocellular in these cases. The estimated T1 relaxation times were highly correlated to the cellularity of the bone marrow as assessed by histology. Among patients with close to 100% cellularity neither T1 nor T2 discriminated between the various malignancies or between malignant and reactive, non-malignant bone marrow. Characterization of tissues in terms of normalized image intensities was also attempted, the motive being to avoid approximations and uncertainties in the assessment of T1 and T2. The normalization was carried out with respect to the image of highest intensity, i.e. the proton density weighted image. The results were in agreement with those for T1 and T2. It was concluded that MRI is valuable for assessing bone marrow cellularity, but not for differentiating between various bone marrow disorders having a similar degree of cellularity.

  4. Influence of anti-cyclic citrullinated peptide on disease activity, structural severity, and bone loss in Moroccan women with rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    Imad Ghozlani

    2018-04-01

    Full Text Available Aim of the work: The aim of this study was to assess the influence of anti-cyclic citrullinated peptide (anti-CCP on disease activity, radiological severity, functional disability and bone loss in Moroccan women with rheumatoid arthritis (RA. Patients and methods: One hundred and thirty-six women with RA were recruited. Age, weight, height, disease duration and steroids cumulative dose were identified. Anti-CCP and Rheumatoid factor (RF were determined. Disease activity score (DAS28 was assessed and functional repercussion measured by the Health Assessment Questionnaire-disability index (HAQ-DI. Radiological status was assessed by the Sharp/van der Heijde (SvH erosion and narrowing score. Bone mineral density was determined by a Lunar Prodigy Vision Dual-energy X-ray absorptiometry and vertebral fracture assessment was classified using a combination of Genant semi-quantitative approach and morphometry. Results: Patients mean age was 49.6 ± 7.4 years and disease duration 7.7 ± 5 years. 109 (80.1% patients were anti-CCP positive. There was no significant difference in DAS28 between patients with and without anti-CCP. Nevertheless, weight, erythrocyte sedimentation rate (ESR, rheumatoid factor titer and positivity, SvH narrowing and erosion score and osteoporosis were significantly higher in patients with positive anti-CCP. Stepwise regression analysis showed that the presence of anti-CCP was independently associated with osteoporosis and SvH erosion score. Conclusions: Anti-CCP antibodies are strongly predictive for the development of osteoporosis and erosions in Moroccan RA patients. They not only have a valuable role in the disease prognosis prediction but also may be a relevant determinant of bone loss in RA. The presence of these antibodies warrants special attention. Keywords: Rheumatoid arthritis, Anti-cyclic citrullinated peptide, Disease activity, Joint damage, Bone loss

  5. Recent Changes in Chronic Kidney Disease-Mineral and Bone Disorders and Associated Fractures After Kidney Transplantation.

    Science.gov (United States)

    Perrin, Peggy; Kiener, Clotilde; Javier, Rose-Marie; Braun, Laura; Cognard, Noelle; Gautier-Vargas, Gabriela; Heibel, Francoise; Muller, Clotilde; Olagne, Jerome; Moulin, Bruno; Ohlmann, Sophie

    2017-08-01

    The management of chronic kidney disease-mineral and bone disorders has recently changed. We investigated the modifications of chronic kidney disease-mineral and bone disorder with a special focus on the incidence of fractures in the first year after kidney transplantation (KT). We retrospectively compared 2 groups of patients who consecutively underwent transplantation at our center 5 years from each other. Group 1 consisted of patients (n = 152) transplanted between 2004 and 2006, whereas patients in group 2 (n = 137) underwent KT between 2009 and 2011. During the end-stage renal disease phase at the time of transplant, cinacalcet, and native vitamin D were used significantly more frequently in group 2. Median intact parathyroid hormone levels were lower and severe hyperparathyroidism decreased significantly. Vitamin D deficiency dropped from 64% to 20%. After transplantation, persistent hyperparathyroidism (parathyroid hormone > 130 ng/L) and bone turnover markers were significantly reduced in group 2. Native vitamin D supplementation increased over time, whereas the use of active vitamin D was unchanged. The 25-hydroxyvitamin D and 1,25-dihydroxyvitamin D levels were significantly higher. The fracture incidence at 1 year decreased significantly (3.1% vs 9.1%; P = 0.047). No steroid sparing was observed in group 2. Bisphosphonates after KT were more frequently used in group 2. Recent changes in clinical practice are associated with reductions in pretransplant and posttransplant hyperparathyroidism, vitamin D deficiency, and fracture risk after KT.

  6. Bone island and leprosy

    Energy Technology Data Exchange (ETDEWEB)

    Carpintero, P.; Garcia-Frasquet, A. [Department of Orthopaedic Surgery, Cordoba University, Medical School, Reina Sofia University Hospital, Cordoba (Spain); Tarradas, E. [Department of Imaging, Cordoba University, Medical School, Cordoba (Spain); Logrono, C. [Department of Dermatology, Reina Sofia University Hospital, Cordoba (Spain); Carrascal, A. [Department of Radiology, Infanta Elena Hospital, Huelva (Spain); Carreto, A. [Department of Radiology, Reina Sofia University Hospital, Cordoba (Spain)

    1998-06-01

    Objective. To determine the incidence of bone islands in leprosy patients. Design. X-rays of feet and hands of patients with Hansen`s disease (leprosy) were reviewed retrospectively. A second group of related age- and sex-matched patients who did not have Hansen`s disease was used for control purposes. Controls had undergone hand or foot X-rays during diagnosis of other pathologies. The patients with Hansen`s disease were compared with the control group, and were also analyzed as subgroups with different types of leprosy. The results were subjected to statistical analysis. Patients. Ninety patients with Hansen`s disease were randomly selected for this study. Patients who had had ulcers on hands or feet were excluded from the study. Results and conclusions. Bone islands were demonstrated in 20 patients with Hansen`s disease; no bone islands were observed in the controls. This was statistically significant (P<0.01). Bone islands were only seen in patients with lepromatous leprosy and borderline types but were not demonstrated in patients with tuberculoid leprosy. There was also a statistically significant relationship for a disease duration of 15 years or more. The cause of this raised incidence of enostosis in leprosy patients is not clear, but there may be a genetic predisposition in patients with leprosy, or it may be a side effect of leprosy, especially the lepromatous form. (orig.) With 4 figs., 2 tabs., 9 refs.

  7. Antibacterial glass and glass-biodegradable matrix composites for bone tissue engineering

    OpenAIRE

    Fernandes, João Pedro Silva

    2017-01-01

    Multiple joint and bone diseases affect millions of people worldwide. In fact the Bone and Joint Decade’s association predicted that the percentage of people over 50 years of age affected by bone diseases will double by 2020. Bone diseases commonly require the need for surgical intervention, often involving partial or total bone substitution. Therefore biodegradable biomaterials designed as bone tissue engineered (BTE) devices to be implanted into the human body, function as a ...

  8. Composition and dosage of a multipartite enhancer cluster control developmental expression of Ihh (Indian hedgehog).

    Science.gov (United States)

    Will, Anja J; Cova, Giulia; Osterwalder, Marco; Chan, Wing-Lee; Wittler, Lars; Brieske, Norbert; Heinrich, Verena; de Villartay, Jean-Pierre; Vingron, Martin; Klopocki, Eva; Visel, Axel; Lupiáñez, Darío G; Mundlos, Stefan

    2017-10-01

    Copy number variations (CNVs) often include noncoding sequences and putative enhancers, but how these rearrangements induce disease is poorly understood. Here we investigate CNVs involving the regulatory landscape of IHH (encoding Indian hedgehog), which cause multiple, highly localized phenotypes including craniosynostosis and synpolydactyly. We show through transgenic reporter and genome-editing studies in mice that Ihh is regulated by a constellation of at least nine enhancers with individual tissue specificities in the digit anlagen, growth plates, skull sutures and fingertips. Consecutive deletions, resulting in growth defects of the skull and long bones, showed that these enhancers function in an additive manner. Duplications, in contrast, caused not only dose-dependent upregulation but also misexpression of Ihh, leading to abnormal phalanges, fusion of sutures and syndactyly. Thus, precise spatiotemporal control of developmental gene expression is achieved by complex multipartite enhancer ensembles. Alterations in the composition of such clusters can result in gene misexpression and disease.

  9. From "Kidneys Govern Bones" to Chronic Kidney Disease, Diabetes Mellitus, and Metabolic Bone Disorder: A Crosstalk between Traditional Chinese Medicine and Modern Science.

    Science.gov (United States)

    Wang, Xiao-Qin; Zou, Xin-Rong; Zhang, Yuan Clare

    2016-01-01

    Although traditional Chinese medicine (TCM) and Western medicine have evolved on distinct philosophical foundations and reasoning methods, an increasing body of scientific data has begun to reveal commonalities. Emerging scientific evidence has confirmed the validity and identified the molecular mechanisms of many ancient TCM theories. One example is the concept of "Kidneys Govern Bones." Here we discuss the molecular mechanisms supporting this theory and its potential significance in treating complications of chronic kidney disease (CKD) and diabetes mellitus. Two signaling pathways essential for calcium-phosphate metabolism can mediate the effect of kidneys in bone homeostasis, one requiring renal production of bioactive vitamin D and the other involving an endocrine axis based on kidney-expressed Klotho and bone-secreted fibroblast growth factor 23. Disruption of either pathway can lead to calcium-phosphate imbalance and vascular calcification, accelerating metabolic bone disorder. Chinese herbal medicine is an adjunct therapy widely used for treating CKD and diabetes. Our results demonstrate the therapeutic effects and underlying mechanisms of a Chinese herbal formulation, Shen-An extracts, in diabetic nephropathy and renal osteodystrophy. We believe that the smart combination of Eastern and Western concepts holds great promise for inspiring new ideas and therapies for preventing and treating complications of CKD and diabetes.

  10. [Bone homeostasis and Mechano biology.

    Science.gov (United States)

    Nakashima, Tomoki

    The weight-bearing exercises help to build bones and to maintain them strength. Bone is constantly renewed by the balanced action of osteoblastic bone formation and osteoclastic bone resorption both of which mainly occur at the bone surface. This restructuring process called "bone remodeling" is important not only for normal bone mass and strength, but also for mineral homeostasis. Bone remodeling is stringently regulated by communication between bone component cells such as osteoclasts, osteoblasts and osteocytes. An imbalance of this process is often linked to various bone diseases. During bone remodeling, resorption by osteoclasts precedes bone formation by osteoblasts. Based on the osteocyte location within the bone matrix and the cellular morphology, it is proposed that osteocytes potentially contribute to the regulation of bone remodeling in response to mechanical and endocrine stimuli.

  11. Archaeological skeletons support a northwest European origin for Paget's disease of bone.

    Science.gov (United States)

    Mays, Simon

    2010-08-01

    The strong genetic component in the etiology of Paget's disease of bone (PDB), together with marked geographic variation in its prevalence, with high frequencies in British populations, has led some to suggest that the disease originated in Britain and spread around the world in recent times by the migration and admixture of British populations. This study aims to investigate this hypothesis by studying the world geographic distribution of PDB cases identified in ancient skeletons excavated from archaeological sites. The methodology is a review of PDB cases described in the literature. There were 109 cases that met modern diagnostic criteria. All came from Western Europe, 94% from England. These data support the hypothesis that PDB originated in this geographic region.

  12. Bone scintigraphy in renal osteopathy

    International Nuclear Information System (INIS)

    Hermann, H.J.; Gahl, G.; Freie Univ. Berlin

    1976-01-01

    25 patients with chronic renal disease are investigated. In 16 cases with conservative treatment the bone scintigram showed pathological uptake according to the creatinine level, mainly in the joints of iliosacrum, hip, knee and ankles. In three patients increased uptake in the skull was found. The bone uptake found by scintigraphy was highly pronounced in the patients treated by dialysis. The most frequently involved regions were the joints of iliocacrum and hip, facial cranium, skull, pelvis and metatarsus. The count-rate ratio of cranium to chest was significantly increased in 6 patients. The investigations 6 months later showed in 4 cases a further increase compared with the first values. Count-rates of the skull were found to be comparable to the highly increased uptake in Paget's disease. Bone scintigraphy is a suitable method to estimate semiquantitatively the bone turnover in renal disease. (orig.) [de

  13. Developmental Origins of Health and Disease (DOHaD): Implications for health and nutritional issues among rural children in China.

    Science.gov (United States)

    Feng, Aihua; Wang, Lijie; Chen, Xiang; Liu, Xiaoyan; Li, Ling; Wang, Baozhen; Luo, Huiwen; Mo, Xiuting; Tobe, Ruoyan Gai

    2015-04-01

    In China, with fast economic growth, health and nutrition status among the rural population has shown significant improvement in the past decades. On the other hand, burden of non-communicable diseases and prevalence of related risk factors such as overweight and obesity has also increased. Among rural children, the double burden of malnutrition and emerging overweight and obesity has been neglected so far. According to the theory of Developmental Origin of Health and Diseases (DOHaD), malnutrition, including both undernutrition (stunting and wasting) and over-nutrition (overweight and obesity) during childhood is closely related to worsened health outcomes during adulthood. Such a neglected problem is attributable to a complicated synergy of social and environmental factors such as parental migration, financial situation of the household, child-rearing knowledge and practices of the primary caregivers, and has implications for public health. Based on literature review of lessons from the field, intervention to address malnutrition among rural children should be a comprehensive package, with consideration of their developmental environment and geographical and socioeconomic diversity. The scientific evidence on DOHaD indicates the probability and necessity of prevention of adult disease by promotion of maternal and child health and reducing malnutrition by provision of high-quality complementary foods, promotion of a well-balanced dietary pattern, and promotion of health literacy in the public would bring a potential benefit to reduce potential risk of diseases.

  14. [Bone scintigraphy with 99mTc-MDP in a patient with acute lymphoblastic leukemia initially diagnosed of Still's disease].

    Science.gov (United States)

    Benítez Velazco, A; González García, F M; Albalá González, M D; Pacheco Capote, C; Latre Romero, J M

    2005-01-01

    We present a 43-year-old male, who was admitted with the diagnosis of Adult-onset Still's disease, after several months of arthralgias, febricula and loss of weight. Chest x-ray, abdominal ultrasonography, chest, abdomen and pelvic CT scan and bone scintigraphy were performed. Scintigraphic findings oriented to the performance of a bone marrow biopsy with diagnosis of acute lymphoblastic leukemia.

  15. Long-term consequences of developmental vascular defects on retinal vessel homeostasis and function in a mouse model of Norrie disease.

    Directory of Open Access Journals (Sweden)

    Susanne C Beck

    Full Text Available Loss of Norrin signalling due to mutations in the Norrie disease pseudoglioma gene causes severe vascular defects in the retina, leading to visual impairment and ultimately blindness. While the emphasis of experimental work so far was on the developmental period, we focus here on disease mechanisms that induce progression into severe adult disease. The goal of this study was the comprehensive analysis of the long-term effects of the absence of Norrin on vascular homeostasis and retinal function. In a mouse model of Norrie disease retinal vascular morphology and integrity were studied by means of in vivo angiography; the vascular constituents were assessed in detailed histological analyses using quantitative retinal morphometry. Finally, electroretinographic analyses were performed to assess the retinal function in adult Norrin deficient animals. We could show that the primary developmental defects not only persisted but developed into further vascular abnormalities and microangiopathies. In particular, the overall vessel homeostasis, the vascular integrity, and also the cellular constituents of the vascular wall were affected in the adult Norrin deficient retina. Moreover, functional analyses indicated to persistent hypoxia in the neural retina which was suggested as one of the major driving forces of disease progression. In summary, our data provide evidence that the key to adult Norrie disease are ongoing vascular modifications, driven by the persistent hypoxic conditions, which are ineffective to compensate for the primary Norrin-dependent defects.

  16. Intersystem Implications of the Developmental Origins of Health and Disease: Advancing Health Promotion in the 21st Century.

    Science.gov (United States)

    Barnes, Michael D; Heaton, Thomas L; Goates, Michael C; Packer, Justin M

    2016-07-15

    The developmental origins of health and disease (DOHaD) theory and life course theory (LCT) are emerging fields of research that have significant implications for the public health and health promotion professions. Using a DOHaD/LCT perspective, social determinants of health (SDH) take on new critical meaning by which health promotion professionals can implement DOHaD/LCT guided interventions, including recommended policies. Through these interventions, public health could further address the sources of worldwide chronic disease epidemics and reduce such disease rates substantially if related policy, programs, and interdisciplinary and multi-sector collaboration are emphasized. Additional characteristics of the most effective interventions involve context-specific adaptation and societal structures that impact upstream, early life environments on a broad scale, influencing multiple locations and/or diseases.

  17. Analysis on Developmental Factors of the Liver Diseases in Ultrasound Diagnosis of Healthcare

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Mi Yeon [Dept. of Radiology of Healthcare Center Kyobo Life Insurance Science, Seoul (Korea, Republic of); Jung, Hong Ryang; Lim, Chang Hwan [Dept. of Radiological Science, Hanseo University, Seosan (Korea, Republic of)

    2009-03-15

    The study found out developmental factors of the liver diseases in 29, 531 cases of the healthy adults who were diagnosed by using ultrasound at domestic healthcare centers in 6 cities. The results are as follows. Based on the result of the study, the liver diseases diagnosed by using ultrasound was revealed to show 43.1% of prevalence, and the occurrence was significantly higher in male (23.3%) than in female (19.8%). The prevalence of hepatic diseases related to the BMI was revealed to show highest prevalence of the fatty liver in obese group (BMI 25) by recording 44.3%. Smoking contributed to the high prevalence of all liver diseases. Although the fatty liver was the most frequently occurred form of liver diseases by recording the prevalence of 49.1% (22.2% in male, 26.9% in female), the significant difference was found only in female (p < 0.05), but male group did not show significant difference (p > 0.05). The prevalence of hepatic diseases related to the hypertension was revealed to show highest prevalence of the fatty liver in hypertension group by recording 67.7%. The prevalence of hepatic diseases related to the diabetes was revealed to show highest prevalence of the fatty liver in diabetes group by recording 66.2%. The high prevalence of all hepatic diseases was related to diabetes mellitus with statistical significance (p < 0.001). The multiple regression analysis for the related factors which affect the prevalence of the liver diseases showed the higher prevalence by age. Sex, obesity and diabetes mellitus were positively related to the prevalence (p < 0.05) while hypertension and smoking showed no significant relationship to the prevalence of the disease (p > 0.05).

  18. A Roadmap to the Brittle Bones of Cystic Fibrosis

    Directory of Open Access Journals (Sweden)

    Ashwini P. Gore

    2011-01-01

    Full Text Available Cystic fibrosis (CF is an autosomal recessive disorder which despite advances in medical care continues to be a life-limiting and often fatal disease. With increase in life expectancy of the CF population, bone disease has emerged as a common complication. Unlike the osteoporosis seen in postmenopausal population, bone disease in CF begins at a young age and is associated with significant morbidity due to fractures, kyphosis, increased pain, and decreased lung function. The maintenance of bone health is essential for the CF population during their lives to prevent pain and fractures but also as they approach lung transplantation since severe bone disease can lead to exclusion from lung transplantation. Early recognition, prevention, and treatment are key to maintaining optimal bone health in CF patients and often require a multidisciplinary approach. This article will review the pathophysiology, current clinical practice guidelines, and potential future therapies for treating CF-related bone disease.

  19. The diagnostic value of the alveolar lamina dura in generalised bone disease

    International Nuclear Information System (INIS)

    Kuhlencordt, J.; Kruse, H.P.; Franke, J.; Hamburg Univ.

    1981-01-01

    Changes in the alveolar lamina dura in 134 patients have been analysed. They included 32 cases with urolithiasis in whom generalised bone disease had been excluded, 37 cases of primary hyperparathyroidism, 31 cases of secondary hyperparathyroidism and 34 cases with primary osteoporosis. The state of the lamina dura was related to biochemical, radiological and histological findings in the various groups. The value of the lamina dura in the diagnosis of generalised skeletal abnormalities has been defined. (orig.) [de

  20. Biology of Bone Tissue: Structure, Function, and Factors That Influence Bone Cells

    Directory of Open Access Journals (Sweden)

    Rinaldo Florencio-Silva

    2015-01-01

    Full Text Available Bone tissue is continuously remodeled through the concerted actions of bone cells, which include bone resorption by osteoclasts and bone formation by osteoblasts, whereas osteocytes act as mechanosensors and orchestrators of the bone remodeling process. This process is under the control of local (e.g., growth factors and cytokines and systemic (e.g., calcitonin and estrogens factors that all together contribute for bone homeostasis. An imbalance between bone resorption and formation can result in bone diseases including osteoporosis. Recently, it has been recognized that, during bone remodeling, there are an intricate communication among bone cells. For instance, the coupling from bone resorption to bone formation is achieved by interaction between osteoclasts and osteoblasts. Moreover, osteocytes produce factors that influence osteoblast and osteoclast activities, whereas osteocyte apoptosis is followed by osteoclastic bone resorption. The increasing knowledge about the structure and functions of bone cells contributed to a better understanding of bone biology. It has been suggested that there is a complex communication between bone cells and other organs, indicating the dynamic nature of bone tissue. In this review, we discuss the current data about the structure and functions of bone cells and the factors that influence bone remodeling.

  1. Building strong bones: molecular regulation of the osteoblast lineage.

    Science.gov (United States)

    Long, Fanxin

    2011-12-22

    The past 15 years have witnessed tremendous progress in the molecular understanding of osteoblasts, the main bone-forming cells in the vertebrate skeleton. In particular, all of the major developmental signals (including WNT and Notch signalling), along with an increasing number of transcription factors (such as RUNX2 and osterix), have been shown to regulate the differentiation and/or function of osteoblasts. As evidence indicates that osteoblasts may also regulate the behaviour of other cell types, a clear understanding of the molecular identity and regulation of osteoblasts is important beyond the field of bone biology.

  2. Methods and theory in bone modeling drift: comparing spatial analyses of primary bone distributions in the human humerus.

    Science.gov (United States)

    Maggiano, Corey M; Maggiano, Isabel S; Tiesler, Vera G; Chi-Keb, Julio R; Stout, Sam D

    2016-01-01

    This study compares two novel methods quantifying bone shaft tissue distributions, and relates observations on human humeral growth patterns for applications in anthropological and anatomical research. Microstructural variation in compact bone occurs due to developmental and mechanically adaptive circumstances that are 'recorded' by forming bone and are important for interpretations of growth, health, physical activity, adaptation, and identity in the past and present. Those interpretations hinge on a detailed understanding of the modeling process by which bones achieve their diametric shape, diaphyseal curvature, and general position relative to other elements. Bone modeling is a complex aspect of growth, potentially causing the shaft to drift transversely through formation and resorption on opposing cortices. Unfortunately, the specifics of modeling drift are largely unknown for most skeletal elements. Moreover, bone modeling has seen little quantitative methodological development compared with secondary bone processes, such as intracortical remodeling. The techniques proposed here, starburst point-count and 45° cross-polarization hand-drawn histomorphometry, permit the statistical and populational analysis of human primary tissue distributions and provide similar results despite being suitable for different applications. This analysis of a pooled archaeological and modern skeletal sample confirms the importance of extreme asymmetry in bone modeling as a major determinant of microstructural variation in diaphyses. Specifically, humeral drift is posteromedial in the human humerus, accompanied by a significant rotational trend. In general, results encourage the usage of endocortical primary bone distributions as an indicator and summary of bone modeling drift, enabling quantitative analysis by direction and proportion in other elements and populations. © 2015 Anatomical Society.

  3. Subchondral bone density distribution in the human femoral head

    Energy Technology Data Exchange (ETDEWEB)

    Wright, David A.; Meguid, Michael; Lubovsky, Omri; Whyne, Cari M. [Sunnybrook Research Institute, Orthopaedic Biomechanics Laboratory, Toronto, Ontario (Canada)

    2012-06-15

    This study aims to quantitatively characterize the distribution of subchondral bone density across the human femoral head using a computed tomography derived measurement of bone density and a common reference coordinate system. Femoral head surfaces were created bilaterally for 30 patients (14 males, 16 females, mean age 67.2 years) through semi-automatic segmentation of reconstructed CT data and used to map bone density, by shrinking them into the subchondral bone and averaging the greyscale values (linearly related to bone density) within 5 mm of the articular surface. Density maps were then oriented with the center of the head at the origin, the femoral mechanical axis (FMA) aligned with the vertical, and the posterior condylar axis (PCA) aligned with the horizontal. Twelve regions were created by dividing the density maps into three concentric rings at increments of 30 from the horizontal, then splitting into four quadrants along the anterior-posterior and medial-lateral axes. Mean values for each region were compared using repeated measures ANOVA and a Bonferroni post hoc test, and side-to-side correlations were analyzed using a Pearson's correlation. The regions representing the medial side of the femoral head's superior portion were found to have significantly higher densities compared to other regions (p < 0.05). Significant side-to-side correlations were found for all regions (r {sup 2} = 0.81 to r {sup 2} = 0.16), with strong correlations for the highest density regions. Side-to-side differences in measured bone density were seen for two regions in the anterio-lateral portion of the femoral head (p < 0.05). The high correlation found between the left and right sides indicates that this tool may be useful for understanding 'normal' density patterns in hips affected by unilateral pathologies such as avascular necrosis, fracture, developmental dysplasia of the hip, Perthes disease, and slipped capital femoral head epiphysis. (orig.)

  4. Bone Marrow Transplantation: MedlinePlus Health Topic

    Science.gov (United States)

    ... marrow transplant - discharge (Medical Encyclopedia) Also in Spanish Topic Image MedlinePlus Email Updates Get Bone Marrow Transplantation ... transplant - slideshow Graft-versus-host disease Related Health Topics Bone Marrow Diseases Stem Cells National Institutes of ...

  5. Application of 2 mm thin-slice scanning with bone algorithm on conventional CT in diagnosis of the pulmonary diseases

    International Nuclear Information System (INIS)

    Zhang Xianheng; Li Xiuhua; Wang Fenghua

    2004-01-01

    Objective: To evaluate the value of 2 mm thin-slice conventional CT scan with bone algorithm in diagnosis and differential diagnosis in the pulmonary diseases. Methods: In total 135 cases of the pulmonary diseases were routinely scanned by conventional scan, 10 mm per slice, with standard algorithm, then the 2 mm thin-slice scan with bone algorithm was performed at the interested region of the lungs. Result: According to the comparative study of the CT signs between 10 mm slice scan with standard algorithm and 2 mm thin-slice scan with bone algorithm, the latter was better on displaying the pulmonary axial interstium, intralobular septum, subpleura lines, honeycombing, 2-5 mm nodulars and anomalies of bronchial wall. Conclusion: According to the study of 135 cases, 2 mm thin-slice scan with bone algorithm is superior to 10 mm slice scan with standard algorithm in demonstrating the pulmonary lesions. It has a similar value with high-resolution spiral CT in the diagnosis of the pulmonary solitary or diffuse nodules, pulmonary diffuse interstitial lesions and the lesions of the airway. It is practical and advisable in the community hospital

  6. Role of periodontal pathogenic bacteria in RANKL-mediated bone destruction in periodontal disease.

    Science.gov (United States)

    Kajiya, Mikihito; Giro, Gabriela; Taubman, Martin A; Han, Xiaozhe; Mayer, Marcia P A; Kawai, Toshihisa

    2010-11-08

    Accumulated lines of evidence suggest that hyperimmune responses to periodontal bacteria result in the destruction of periodontal connective tissue and alveolar bone. The etiological roles of periodontal bacteria in the onset and progression of periodontal disease (PD) are well documented. However, the mechanism underlying the engagement of periodontal bacteria in RANKL-mediated alveolar bone resorption remains unclear. Therefore, this review article addresses three critical subjects. First, we discuss earlier studies of immune intervention, ultimately leading to the identification of bacteria-reactive lymphocytes as the cellular source of osteoclast-induction factor lymphokine (now called RANKL) in the context of periodontal bone resorption. Next, we consider (1) the effects of periodontal bacteria on RANKL production from a variety of adaptive immune effector cells, as well as fibroblasts, in inflamed periodontal tissue and (2) the bifunctional roles (upregulation vs. downregulation) of LPS produced from periodontal bacteria in a RANKL-induced osteoclast-signal pathway. Future studies in these two areas could lead to new therapeutic approaches for the management of PD by down-modulating RANKL production and/or RANKL-mediated osteoclastogenesis in the context of host immune responses against periodontal pathogenic bacteria.

  7. Genomic deletion of a long-range bone enhancer misregulatessclerostin in Van Buchem disease

    Energy Technology Data Exchange (ETDEWEB)

    Loots, Gabriela G.; Kneissel, Michaela; Keller, Hansjoerg; Baptist, Myma; Chang, Jessie; Collette, Nicole M.; Ovcharenko, Dmitriy; Plajzer-Frick, Ingrid; Rubin, Edward M.

    2005-04-15

    Mutations in distant regulatory elements can negatively impact human development and health, yet due to the difficulty of detecting these critical sequences we predominantly focus on coding sequences for diagnostic purposes. We have undertaken a comparative sequence-based approach to characterize a large noncoding region deleted in patients affected by Van Buchem disease (VB), a severe sclerosing bone dysplasia. Using BAC recombination and transgenesis we characterized the expression of human sclerostin (sost) from normal (hSOSTwt) or Van Buchem(hSOSTvb D) alleles. Only the hSOSTwt allele faithfully expressed high levels of human sost in the adult bone and impacted bone metabolism, consistent with the model that the VB noncoding deletion removes a sost specific regulatory element. By exploiting cross-species sequence comparisons with in vitro and in vivo enhancer assays we were able to identify a candidate enhancer element that drives human sost expression in osteoblast-like cell lines in vitro and in the skeletal anlage of the E14.5 mouse embryo, and discovered a novel function for sclerostin during limb development. Our approach represents a framework for characterizing distant regulatory elements associated with abnormal human phenotypes.

  8. Cellular and Molecular Mechanisms of Bone Remodeling*

    OpenAIRE

    Raggatt, Liza J.; Partridge, Nicola C.

    2010-01-01

    Physiological bone remodeling is a highly coordinated process responsible for bone resorption and formation and is necessary to repair damaged bone and to maintain mineral homeostasis. In addition to the traditional bone cells (osteoclasts, osteoblasts, and osteocytes) that are necessary for bone remodeling, several immune cells have also been implicated in bone disease. This minireview discusses physiological bone remodeling, outlining the traditional bone biology dogma in light of emerging ...

  9. Thyroid disorders and bone mineral metabolism

    Directory of Open Access Journals (Sweden)

    Dinesh Kumar Dhanwal

    2011-01-01

    Full Text Available Thyroid diseases have widespread systemic manifestations including their effect on bone metabolism. On one hand, the effects of thyrotoxicosis including subclinical disease have received wide attention from researchers over the last century as it an important cause of secondary osteoporosis. On the other hand, hypothyroidism has received lesser attention as its effect on bone mineral metabolism is minimal. Therefore, this review will primarily focus on thyrotoxicosis and its impact on bone mineral metabolism.

  10. Bone and mineral metabolism in adult celiac disease

    International Nuclear Information System (INIS)

    Caraceni, M.P.; Molteni, N.; Bardella, M.T.; Ortolani, S.; Nogara, A.; Bianchi, P.A.

    1988-01-01

    Bone mineral density ( 125 I photon absorptiometry) was lower in 20 untreated adult celiac patients than in sex- and age-matched controls (p less than 0.001), and plasma alkaline phosphatase, parathyroid hormone, urinary hydroxyproline/creatinine levels were higher than normal (p less than 0.05, less than 0.001, less than 0.05, respectively). Gluten-free diet was started, and the patients were divided randomly into two treatment groups, one which received oral 25-hydroxyvitamin D 50 micrograms/day and one which did not. After 12 months' treatment, bone turnover markers showed a decrease, which did not reach statistical significance, and bone mineral density did not show significant modifications compared with base line in either group. It was found that a gluten-free diet followed for 1 yr can prevent further bone loss, but no significant differences were detected between the two groups

  11. Bone and mineral metabolism in adult celiac disease

    Energy Technology Data Exchange (ETDEWEB)

    Caraceni, M.P.; Molteni, N.; Bardella, M.T.; Ortolani, S.; Nogara, A.; Bianchi, P.A.

    1988-03-01

    Bone mineral density (/sup 125/I photon absorptiometry) was lower in 20 untreated adult celiac patients than in sex- and age-matched controls (p less than 0.001), and plasma alkaline phosphatase, parathyroid hormone, urinary hydroxyproline/creatinine levels were higher than normal (p less than 0.05, less than 0.001, less than 0.05, respectively). Gluten-free diet was started, and the patients were divided randomly into two treatment groups, one which received oral 25-hydroxyvitamin D 50 micrograms/day and one which did not. After 12 months' treatment, bone turnover markers showed a decrease, which did not reach statistical significance, and bone mineral density did not show significant modifications compared with base line in either group. It was found that a gluten-free diet followed for 1 yr can prevent further bone loss, but no significant differences were detected between the two groups.

  12. Use of Gamma Correction Pinhole Bone Scans in Trauma

    International Nuclear Information System (INIS)

    Bahk, Youg Whee; Chung, Youg An; Park, Jung Mee

    2012-01-01

    99 mTc hydroxydiphosphonate (HDP) bone scanning is a classic metabolic nuclear imaging method and the most frequently performed examination. Clinically, it has long been cherished as an indispensable diagnostic screening tool and for monitoring of patients with bone, joint, and soft tissue diseases. The HDP bone scan, the pinhole scan in particular, is known for its ability to detect increased, decreased, or defective tracer uptake along with magnified anatomy. Unfortunately, however, the findings of such uptake changes are not specific in many traumatic bone disorders, especially when lesions are minute and complex. This study discusses the recently introduced gamma correction pinhole bone scan (GCPBS), emphasizing its usefulness in the diagnosis of traumatic bone diseases including occult fractures; and fish vertebra. Indeed, GCPBS can remarkably enhance the diagnostic feasibility of HDP pinhole bone scans by refining the topography, pathologic anatomy, and altered chemical profile of the traumatic diseases in question. The fine and precise depiction of anatomic and metabolic changes in these diseases has been shown to be unique to GCPBS, and they are not appreciated on conventional radiographs, multiple detector CT, or ultrasonographs. It is true that MR imaging can portray proton change, but understandably, it is a manifestation that is common to any bone disease

  13. Bone and bone marrow scintigraphy in a patient with sickle cell-thalassemia and recurrent pain attacks

    International Nuclear Information System (INIS)

    Mikosch, P.; Gallowitsch, H.-J.; Lind, P.; Jauk, B.; Kaulfersch, W.

    2003-01-01

    The case of an eight years old African boy who suffers from sickle cell-thalassemia is presented. In the course of the disease frequent pain attacks occurred within the abdomen and extremities, recently also within the trunk. Local pain, at some occasions in combination with local swelling and always positive laboratory parameters for inflammation, hindered a solely clinical differentiation between bone infarcts and osteomyelitis. Bone scintigraphy, eventually in combination with bone marrow scintigraphy, can assist the clinician in the differentiation of aseptic bone infarcts versus secondary osteomyelitis. Based on the presented case scintigraphic results for bone infarcts, osteomyelitis and special scintigraphic pattern seen in sickle cell disease are presented. Furthermore, problems regarding the interpretation of the scintigraphies in relation to the delayed time after the beginning of pain attacks are discussed. (author)

  14. Imaging investigation of metabolic and endocrine bone disease of vertebral density

    International Nuclear Information System (INIS)

    Cai Yuezeng; Tian Xiali; Li Jingxue

    2006-01-01

    Objective: To probe vertebral density of metabolic and endocrine bone disease imaging features, characterize the regional distribution of bone trabecular in sandwich spine. Methods: Thirty-six patients who had the bone density abnormality appearance in radiograms were collected in this study. Twelve patients with sandwich spine were performed lumbar CT scan. Thirty-two healthy volunteers as control group were performed lumbar CT scan too. CT values of two groups were measured from different portions of vertebral body, and then were analysed. Twenty two patients were performed dual-energy x-ray absorptiometry (DXA). One patient was performed bone histomorphometry. Results: Abnormal density included decreased and increased density. Decreased density was found in different portions of all patients, which divided into general and regional type. Increased density was obviously in vertebrae, including diffusely increased density and sandwich spine. The mean CT values of superior, middle and inferior portions of sandwich vertebral body were (259.94±18.08), (182.96±34.85), (270.34±19.40) HU. The mean CT values of both superior and inferior portions of sandwich vertebral body were higher than that of control group. The mean CT values of superior and inferior portions of sandwich spine were higher than that of middle portion. The difference of mean CT values between superior and inferior portions had no statistical significance. The difference of CT values among the regions of superior and inferior portions had no statistical significance (F=0.457, 0.462, P>0.05). The difference of CT values among the regions of middle portion had statistical significance(F=4.539, P<0.05). The DXA measurement of sandwich spine showed high, normal and low BMD. Conclusion: The sandwich spine is useful to measure superior and inferior portions of sandwich vertebral body if QCT would be performed. Sandwich spine sign can be used as an imaging index of state evaluation. Increased density in

  15. Diagnosis of temporal bone diseases using three-dimensional images with multislice CT

    Energy Technology Data Exchange (ETDEWEB)

    Toyama, Yoshihiro; Togami, Taro; Murota, Makiko; Fukunaga, Kotaro; Hino, Ichiro; Sato, Katashi; Ohkawa, Motoomi [Kagawa Medical Univ., Miki (Japan)

    2001-08-01

    We evaluated the usefulness of three-dimensional images with multislice CT in the temporal bone diseases. Fifty-nine cases (26 with medial otitis, 8 choresteatoma, 10 congenital malformation, 3 high jugular bulb, 2 otosclerosis, and 10 others) were included in this study. In the ossicular and inner ear lesions, oblique multiplanar images of the long axis of each ossicle was useful the detection of abnormality. Structural deformity of ossicles and bony labyrinth were clearly delineated by surface rendering images. (author)

  16. Utility of unenhanced fat-suppressed T1-weighted MRI in children with sickle cell disease -- can it differentiate bone infarcts from acute osteomyelitis?

    Science.gov (United States)

    Delgado, Jorge; Bedoya, Maria A; Green, Abby M; Jaramillo, Diego; Ho-Fung, Victor

    2015-12-01

    Children with sickle cell disease (SCD) are at risk of bone infarcts and acute osteomyelitis. The clinical differentiation between a bone infarct and acute osteomyelitis is a diagnostic challenge. Unenhanced T1-W fat-saturated MR images have been proposed as a potential tool to differentiate bone infarcts from osteomyelitis. To evaluate the reliability of unenhanced T1-W fat-saturated MRI for differentiation between bone infarcts and acute osteomyelitis in children with SCD. We retrospectively reviewed the records of 31 children (20 boys, 11 girls; mean age 10.6 years, range 1.1-17.9 years) with SCD and acute bone pain who underwent MR imaging including unenhanced T1-W fat-saturated images from 2005 to 2010. Complete clinical charts were reviewed by a pediatric hematologist with training in infectious diseases to determine a clinical standard to define the presence or absence of osteomyelitis. A pediatric radiologist reviewed all MR imaging and was blinded to clinical information. Based on the signal intensity in T1-W fat-saturated images, the children were further classified as positive for osteomyelitis (low bone marrow signal intensity) or positive for bone infarct (high bone marrow signal intensity). Based on the clinical standard, 5 children were classified as positive for osteomyelitis and 26 children as positive for bone infarct (negative for osteomyelitis). The bone marrow signal intensity on T1-W fat-saturated imaging was not significant for the differentiation between bone infarct and osteomyelitis (P = 0.56). None of the additional evaluated imaging parameters on unenhanced MRI proved reliable in differentiating these diagnoses. The bone marrow signal intensity on unenhanced T1-W fat-saturated MR images is not a reliable criterion to differentiate bone infarcts from osteomyelitis in children.

  17. Utility of unenhanced fat-suppressed T1-weighted MRI in children with sickle cell disease - can it differentiate bone infarcts from acute osteomyelitis?

    International Nuclear Information System (INIS)

    Delgado, Jorge; Bedoya, Maria A.; Green, Abby M.; Jaramillo, Diego; Ho-Fung, Victor

    2015-01-01

    Children with sickle cell disease (SCD) are at risk of bone infarcts and acute osteomyelitis. The clinical differentiation between a bone infarct and acute osteomyelitis is a diagnostic challenge. Unenhanced T1-W fat-saturated MR images have been proposed as a potential tool to differentiate bone infarcts from osteomyelitis. To evaluate the reliability of unenhanced T1-W fat-saturated MRI for differentiation between bone infarcts and acute osteomyelitis in children with SCD. We retrospectively reviewed the records of 31 children (20 boys, 11 girls; mean age 10.6 years, range 1.1-17.9 years) with SCD and acute bone pain who underwent MR imaging including unenhanced T1-W fat-saturated images from 2005 to 2010. Complete clinical charts were reviewed by a pediatric hematologist with training in infectious diseases to determine a clinical standard to define the presence or absence of osteomyelitis. A pediatric radiologist reviewed all MR imaging and was blinded to clinical information. Based on the signal intensity in T1-W fat-saturated images, the children were further classified as positive for osteomyelitis (low bone marrow signal intensity) or positive for bone infarct (high bone marrow signal intensity). Based on the clinical standard, 5 children were classified as positive for osteomyelitis and 26 children as positive for bone infarct (negative for osteomyelitis). The bone marrow signal intensity on T1-W fat-saturated imaging was not significant for the differentiation between bone infarct and osteomyelitis (P = 0.56). None of the additional evaluated imaging parameters on unenhanced MRI proved reliable in differentiating these diagnoses. The bone marrow signal intensity on unenhanced T1-W fat-saturated MR images is not a reliable criterion to differentiate bone infarcts from osteomyelitis in children. (orig.)

  18. Utility of unenhanced fat-suppressed T1-weighted MRI in children with sickle cell disease - can it differentiate bone infarcts from acute osteomyelitis?

    Energy Technology Data Exchange (ETDEWEB)

    Delgado, Jorge; Bedoya, Maria A. [The Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); Green, Abby M. [The Children' s Hospital of Philadelphia, Division of Oncology, Philadelphia, PA (United States); Jaramillo, Diego; Ho-Fung, Victor [The Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA (United States)

    2015-12-15

    Children with sickle cell disease (SCD) are at risk of bone infarcts and acute osteomyelitis. The clinical differentiation between a bone infarct and acute osteomyelitis is a diagnostic challenge. Unenhanced T1-W fat-saturated MR images have been proposed as a potential tool to differentiate bone infarcts from osteomyelitis. To evaluate the reliability of unenhanced T1-W fat-saturated MRI for differentiation between bone infarcts and acute osteomyelitis in children with SCD. We retrospectively reviewed the records of 31 children (20 boys, 11 girls; mean age 10.6 years, range 1.1-17.9 years) with SCD and acute bone pain who underwent MR imaging including unenhanced T1-W fat-saturated images from 2005 to 2010. Complete clinical charts were reviewed by a pediatric hematologist with training in infectious diseases to determine a clinical standard to define the presence or absence of osteomyelitis. A pediatric radiologist reviewed all MR imaging and was blinded to clinical information. Based on the signal intensity in T1-W fat-saturated images, the children were further classified as positive for osteomyelitis (low bone marrow signal intensity) or positive for bone infarct (high bone marrow signal intensity). Based on the clinical standard, 5 children were classified as positive for osteomyelitis and 26 children as positive for bone infarct (negative for osteomyelitis). The bone marrow signal intensity on T1-W fat-saturated imaging was not significant for the differentiation between bone infarct and osteomyelitis (P = 0.56). None of the additional evaluated imaging parameters on unenhanced MRI proved reliable in differentiating these diagnoses. The bone marrow signal intensity on unenhanced T1-W fat-saturated MR images is not a reliable criterion to differentiate bone infarcts from osteomyelitis in children. (orig.)

  19. Meeting report on the 3rd International Congress on Developmental Origins of Health and Disease (DOHaD).

    Science.gov (United States)

    Gillman, Matthew W; Barker, David; Bier, Dennis; Cagampang, Felino; Challis, John; Fall, Caroline; Godfrey, Keith; Gluckman, Peter; Hanson, Mark; Kuh, Diana; Nathanielsz, Peter; Nestel, Penelope; Thornburg, Kent L

    2007-05-01

    Developmental origins of health and disease (DOHaD) focuses on the earliest stages of human development, and provides a novel paradigm to complement other strategies for lifelong prevention of common chronic health conditions. The 3 International Congress on DOHaD, held in 2005, retained the most popular features from the first two biannual Congresses, while adding a number of innovations, including increased emphasis on implications of DOHaD for the developing world; programs for trainees and young investigators; and new perspectives, including developmental plasticity, influences of social hierarchies, effects of prematurity, and populations in transition. Emerging areas of science included, first, the controversial role of infant weight gain in predicting adult obesity, diabetes, and cardiovascular disease. Second, in the era of epidemic obesity, paying attention to the over-nourished fetus is as important as investigating the growth retarded one. Third, environmental toxins appear to have abroad range of long-lasting effects on the developing human. Fourth, epigenetic mechanisms could unite several strands of human and animal observations, and explain how genetically identical individuals raised in similar postnatal environments can nonetheless develop widely differing phenotypes. Improving the environment to which an individual is exposed during development may be as important as any other public health effort to enhance population health world wide.

  20. The Bone Marrow-Derived Stromal Cells

    DEFF Research Database (Denmark)

    Tencerova, Michaela; Kassem, Moustapha

    2016-01-01

    Bone marrow (BM) microenvironment represents an important compartment of bone that regulates bone homeostasis and the balance between bone formation and bone resorption depending on the physiological needs of the organism. Abnormalities of BM microenvironmental dynamics can lead to metabolic bone...... diseases. BM stromal cells (also known as skeletal or mesenchymal stem cells) [bone marrow stromal stem cell (BMSC)] are multipotent stem cells located within BM stroma and give rise to osteoblasts and adipocytes. However, cellular and molecular mechanisms of BMSC lineage commitment to adipocytic lineage...

  1. Treatment of bone loss in osteopenic patients with Crohn's disease: a double-blind, randomised trial of oral risedronate 35 mg once weekly or placebo, concomitant with calcium and vitamin D supplementation

    NARCIS (Netherlands)

    van Bodegraven, A.A.; Bravenboer, N.; Witte, B.I.; Dijkstra, G.; van der Woude, C.J.; Stokkers, P.C.M.; Russel, M.G.; Oldenburg, B.; Pierik, M.; Roos, J.C.; van Hogezand, R.A.; Dik, V.K.; Oostlander, A.E.; Netelenbos, J.C.; van de Langerijt, L.; Hommes, D.W.; Lips, P.T.A.M.

    2014-01-01

    Objective: Osteoporosis and fractures are frequently encountered in patients with Crohn's disease. In order to prevent fractures, treatment with bone protecting drugs appears warranted early in the course of bone disease when bone loss is not yet prominent. We therefore aimed to demonstrate a

  2. Treatment of bone loss in osteopenic patients with Crohn's disease : a double-blind, randomised trial of oral risedronate 35 mg once weekly or placebo, concomitant with calcium and vitamin D supplementation

    NARCIS (Netherlands)

    van Bodegraven, Ad A.; Bravenboer, Nathalie; Witte, Birgit I.; Dijkstra, Gerard; van der Woude, C. Janneke; Stokkers, Pieter C. M.; Russel, Maurice G.; Oldenburg, Bas; Pierik, Marieke; Roos, Jan C.; van Hogezand, Ruud A.; Dik, Vincent K.; Oostlander, Angela E.; Netelenbos, J. Coen; van de langerijt, Lex; Hommes, Daniel W.; Lips, Paul

    Objective Osteoporosis and fractures are frequently encountered in patients with Crohn's disease. In order to prevent fractures, treatment with bone protecting drugs appears warranted early in the course of bone disease when bone loss is not yet prominent. We therefore aimed to demonstrate a

  3. Bone marrow morphology and disease progression in congenital thrombocytopenia: a detailed clinicopathologic and genetic study of eight cases.

    Science.gov (United States)

    Tsang, Hamilton C; Bussel, James B; Mathew, Susan; Liu, Yen-Chun; Imahiyerobo, Allison A; Orazi, Attilio; Geyer, Julia T

    2017-04-01

    Patients with congenital thrombocytopenia have an increased risk of developing myeloid neoplasms. In these cases, the morphologic distinction between disease at baseline and at progression is challenging. This report analyzes clinicopathologic features of congenital thrombocytopenia with long-term follow-up at one referral center. Records from the last 20 years were searched for cases of congenital thrombocytopenia with bone marrow biopsies and peripheral blood smears. The clinical, morphologic, immunophenotypic, and molecular features were analyzed. Six adult and two pediatric patients were identified (six male, two female). Age range at first biopsy was 1-47 (median, 31) years. Underlying diseases included thrombocytopenia-absent radius syndrome, congenital thrombocytopenia with radial-ulnar synostosis, MYH9-related disorder, shortened telomere syndrome, congenital thrombocytopenia with ANKRD26 mutation, and familial platelet disorder with predisposition to acute myeloid leukemia. Four patients had myelodysplastic/myeloproliferative neoplasm-like marrow changes such as hypercellularity, increased myeloid to erythroid ratio, numerous micromegakaryocytes (highlighted by CD42b), and marrow fibrosis. Two patients had marrow hypoplasia and two had unremarkable marrow morphology. Three patients-all in the myelodysplastic/myeloproliferative neoplasm-like group-developed disease progression characterized by erythroid and myeloid dysplasia, elevated bone marrow blasts, and new cytogenetic abnormalities. Unlike non-familial myeloid neoplasms, congenital thrombocytopenia patients in the myelodysplastic/myeloproliferative neoplasm-like group had a long and indolent clinical course (average age at disease progression, 47 years). In summary, three distinct morphologic types of congenital thrombocytopenia were identified: a hyperplastic myelodysplastic/myeloproliferative neoplasm-like group, a hypoplastic bone marrow failure-like group, and a group with relatively normal marrow

  4. Cancer Metastases to Bone: Concepts, Mechanisms, and Interactions with Bone Osteoblasts

    Directory of Open Access Journals (Sweden)

    Alison B. Shupp

    2018-06-01

    Full Text Available The skeleton is a unique structure capable of providing support for the body. Bone resorption and deposition are controlled in a tightly regulated balance between osteoblasts and osteoclasts with no net bone gain or loss. However, under conditions of disease, the balance between bone resorption and deposition is upset. Osteoblasts play an important role in bone homeostasis by depositing new bone osteoid into resorption pits. It is becoming increasingly evident that osteoblasts additionally play key roles in cancer cell dissemination to bone and subsequent metastasis. Our laboratory has evidence that when osteoblasts come into contact with disseminated breast cancer cells, the osteoblasts produce factors that initially reduce breast cancer cell proliferation, yet promote cancer cell survival in bone. Other laboratories have demonstrated that osteoblasts both directly and indirectly contribute to dormant cancer cell reactivation in bone. Moreover, we have demonstrated that osteoblasts undergo an inflammatory stress response in late stages of breast cancer, and produce inflammatory cytokines that are maintenance and survival factors for breast cancer cells and osteoclasts. Advances in understanding interactions between osteoblasts, osteoclasts, and bone metastatic cancer cells will aid in controlling and ultimately preventing cancer cell metastasis to bone.

  5. Impact of additional SPECT in bone scanning in tumor patients with suspected metastatic bone disease

    International Nuclear Information System (INIS)

    Apostolova, I.; Goelcuek, E.; Buchert, R.; Brenner, W.; Bohuslavizki, K.H.

    2009-01-01

    The aim of this study was to investigate the additional value of single-photon emission computed tomography (SPECT) for patient staging compared to planar bone scanning in an unselected cohort of cancer patients. The study included 271 consecutive tumor patients in whom planar imaging and two-bed position SPECT of the spine and the pelvis had been performed. Retrospective image interpretation was performed independently for planar and SPECT scans. Findings were categorized as 'benign', 'equivocal', or malignant' on a lesion base, and as 'no metastatic disease', 'equivocal', or metastatic disease' on a patient base. Four hundred and forty seven lesions were detected by SPECT. Missing of lesions in planar images was rare (4.3% of all SPECT lesions). Planar findings differed from SPECT findings in 149 lesions (33.3%). Most of these 'inconsistent' lesions were rated as equivocal in the planar images but benign (14.5% of all lesions) or malignant (11.0%) by SPECT. On a patient base, 81.6% of patients with planar equivocal staging were classified as either benign (55.3%) or malignant (26.3%) by SPECT. Patients definitively staged as 'no metastatic disease' or 'metastatic disease' in planar images were staged differently by SPECT in only 3.7% of cases (up-staging in 2.6% and down-staging in 1.1%). Single-photon emission computed tomography changed a definite staging as based on planar images in less than 4% of the patients. In patients with planar equivocal staging, however, SPECT allowed a definite diagnosis in more than 80% of these cases, and, thus, should be performed routinely in patients with equivocal findings. (author)

  6. Epidemiology of fractures in people with severe and profound developmental disabilities

    Science.gov (United States)

    Glick, N.R.; Fischer, M.H.; Heisey, D.M.; Leverson, G.E.; Mann, D.C.

    2005-01-01

    Fractures are more prevalent among people with severe and profound developmental disabilities than in the general population. In order to characterize the tendency of these people to fracture, and to identify features that may guide the development of preventive strategies, we analyzed fracture epidemiology in people with severe and profound developmental disabilities who lived in a stable environment. Data from a 23-year longitudinal cohort registry of 1434 people with severe and profound developmental disabilities were analyzed to determine the effects of age, gender, mobility, bone fractured, month of fracture, and fracture history upon fracture rates. Eighty-five percent of all fractures involved the extremities. The overall fracture rate increased as mobility increased. In contrast, femoral shaft fracture risk was substantially higher in the least mobile [relative risk (RR), 10.36; 95% confidence interval (CI), 3.29-32.66] compared with the most mobile group. Although the overall fracture rate was not associated with age, the femoral shaft fractures decreased but hand/foot fractures increased with age. Overall fracture risk declined in August and September (RR, 0.70; 95% CI, 0.55-0.89), being especially prominent for tibial/fibular fractures (RR, 0.31; 95% CI, 0.13-0.70). Gender was not a factor in fracture risk. Two primary fracture mechanisms are apparent: one, largely associated with lack of weight-bearing in people with the least mobility, is exemplified by femoral fractures during non-traumatic events as simple as diapering or transfers; the other, probably due to movement- or fall-related trauma, is exemplified by hand/foot fractures in people who ambulate. The fracture experience of people with severe and profound developmental disabilities is unique and, because it differs qualitatively from postmenopausal osteoporosis, may require population-specific methods for assessing risk, for improving bone integrity, and for reduction of falls and accidents

  7. Antiepileptic drugs and bone metabolism

    Directory of Open Access Journals (Sweden)

    Labban Barbara

    2006-09-01

    Full Text Available Abstract Anti-epileptic medications encompass a wide range of drugs including anticonvulsants, benzodiazepines, enzyme inducers or inhibitors, with a variety effects, including induction of cytochrome P450 and other enzyme, which may lead to catabolism of vitamin D and hypocalcemia and other effects that may significantly effect the risk for low bone mass and fractures. With the current estimates of 50 million people worldwide with epilepsy together with the rapid increase in utilization of these medications for other indications, bone disease associated with the use of anti-epileptic medications is emerging as a serious health threat for millions of people. Nevertheless, it usually goes unrecognized and untreated. In this review we discuss the pathophysiologic mechanisms of bone disease associated with anti-epileptic use, including effect of anti-epileptic agents on bone turnover and fracture risk, highlighting various strategies for prevention of bone loss and associated fractures a rapidly increasing vulnerable population.

  8. Radiography and bone scintigraphy in bone marrow transplant multiple myeloma patients

    International Nuclear Information System (INIS)

    Aagren, B.; Aspelin, P.

    1997-01-01

    Purpose: To compare conventional radiography and bone scintigraphy in relation to clinical outcome in bone marrow transplant multiple myeloma patients. Material and Methods: A total of 70 radiographies and 70 bone scintigraphies were compared in 35 patients. Results: The skull, the extremities, the iliac and public bones were better assessed with radiography. For new vertebral lesions and for lesions in the ribs and sternum, bone scintigraphy proved superior. For the sacrum, the methods were equal. When bone scintigraphy was used as a complement to radiography, 4% more pathological sites were found. No patient had both a normal radiography and a pathological bone scintigraphy, but 5 patients had both a normal bone scintigraphy and a pathological radiography. The results of the radiological examinations did not always correlate with the clinician's grading of the patient's disease. The radiological examinations had no prognostic value for the 7 patients examined on several occasions. Conclusion: The ability of conventional radiography and bone scintigraphy to disclose myeloma lesions varies, depending on location and size of the lesions. Radiography should remain the primary examination modality also for bone marrow transplant multiple myeloma patients. Bone scintigraphy can severe as a complement for investigating unexplained pain, e.g. caused by lesions in vertebrae or ribs. (orig.)

  9. Usefulness of bone marrow magnetic resonance imaging and indium-111-chloride bone marrow scintigraphy in patients with various hematological diseases

    Energy Technology Data Exchange (ETDEWEB)

    Kobayashi, Yutaka; Umekawa, Tsunekazu; Chikayama, Satoshi [Osaka General Hospital of West Japan Railway Compapy (Japan)] [and others

    1995-03-01

    This study investigated the ability of magnetic resonance (MR) imaging and indium-111 chloride (In-111) scintigraphy to assess bone marrow in various hematological lesions. The subjects were 7 with aplastic anemia (AA), 4 with myelodysplastic syndrome (MDS), 3 with polycythemia (PC), 3 with essential thrombocythemia (ET), 2 with multiple myeloma (MM), 2 with monoclonal gammopathy of undetermined significance (MGUS), 3 with idiopathic thrombocytopenic purpura (ITP), one with acute lymphocytic leukemia (ALL), and one with secondary anemia due to chronic inflammation (SA). Bone marrow cellularity was assessed on MR images and both uptake and tissue distribution were assessed on In-111 scintigraphy. Hypo-cellularity was seen in all AA patients, but not seen in any other patient in each group. On the other hand, hyper-cellularity was seen in 3 MDS, one PC, all 3 ET, one ALL, and one SA patients. In the group of MM, the vertebral body was seen as heterogenous signal intensity on MR images. Bone marrow was seen as iso-intensity in one MDS, 2 PC, all 2 MGUS, and all 3 ITP patients. In-111 scintigraphy showed decrease or disappearance of tracer uptake and decreased tissue distribution in all 7 AA, one MDS, one PC, and one ALL patients. Increased tracer uptake and enlarged tissue distribution were seen in one MDS, one PC, and one SA patients. One MDS, one ET, all 2 MM, all 2 MGUS, all 3 ITP patients had tracer uptake and tissue distribution that were equal to those in the normal tissues. Since MR imaging and In-111 scintigraphy provided qualitatively different information, the combination of both modalities would contribute to the understanding of bone marrow condition in hematopoietic diseases. (N.K.).

  10. Avascular necrosis of the lunate bone (Kienböck’s disease) secondary to scapholunate ligament tear as a consequence of trauma – a case study

    International Nuclear Information System (INIS)

    Kulhawik, Dorota; Szałaj, Tomasz; Grabowska, Monika

    2014-01-01

    Avascular necrosis of the lunate bone (Kienböck’s disease), is a condition in which lunate bone, loses its blood supply, leading to necrosis of the bone. There is probably no single cause of Kienbock’s disease. Its origin may involve multiple factors, such as the blood supply (arteries), blood drainage (veins), and skeletal variations. Trauma, either isolated or repeated, may possibly be a factor in some cases. This case presented with multifactorial etiology. In the presented case, a patient with negative ulnar variant had injured her right wrist and presented at an orthopedic clinic due to nonspecific pain 6 months later. An arthro-MRI examination revealed necrosis of the lunate bone, scapholunate ligament tear and coexisting TFCC (triangular fibrocartilage complex) tear. Early diagnosis and treatment can prevent progression of necrotic lesions and bone collapse. MRI examination seems to be the key diagnostic method in the early stage of the Kienböck’s disease with negative x-ray and CT images. Arthro-MRI examination also allows us to identify the underlying ligamentous injury. In cases of traumatic etiology, an additional CT test enables stating the final diagnosis

  11. In vitro model of vascularized bone: synergizing vascular development and osteogenesis.

    Directory of Open Access Journals (Sweden)

    Cristina Correia

    Full Text Available Tissue engineering provides unique opportunities for regenerating diseased or damaged tissues using cells obtained from tissue biopsies. Tissue engineered grafts can also be used as high fidelity models to probe cellular and molecular interactions underlying developmental processes. In this study, we co-cultured human umbilical vein endothelial cells (HUVECs and human mesenchymal stem cells (MSCs under various environmental conditions to elicit synergistic interactions leading to the colocalized development of capillary-like and bone-like tissues. Cells were encapsulated at the 1:1 ratio in fibrin gel to screen compositions of endothelial growth medium (EGM and osteogenic medium (OM. It was determined that, to form both tissues, co-cultures should first be supplied with EGM followed by a 1:1 cocktail of the two media types containing bone morphogenetic protein-2. Subsequent studies of HUVECs and MSCs cultured in decellularized, trabecular bone scaffolds for 6 weeks assessed the effects on tissue construct of both temporal variations in growth-factor availability and addition of fresh cells. The resulting grafts were implanted subcutaneously into nude mice to determine the phenotype stability and functionality of engineered vessels. Two important findings resulted from these studies: (i vascular development needs to be induced prior to osteogenesis, and (ii the addition of additional hMSCs at the osteogenic induction stage improves both tissue outcomes, as shown by increased bone volume fraction, osteoid deposition, close proximity of bone proteins to vascular networks, and anastomosis of vascular networks with the host vasculature. Interestingly, these observations compare well with what has been described for native development. We propose that our cultivation system can mimic various aspects of endothelial cell-osteogenic precursor interactions in vivo, and could find utility as a model for studies of heterotypic cellular interactions that

  12. Expression of Five Neuroblastoma Genes in Bone Marrow or Blood of Patients with Relapsed/Refractory Neuroblastoma Provides a New Biomarker for Disease and Prognosis.

    Science.gov (United States)

    Marachelian, Araz; Villablanca, Judith G; Liu, Cathy W; Liu, Betty; Goodarzian, Fariba; Lai, Hollie A; Shimada, Hiroyuki; Tran, Hung C; Parra, Jaime A; Gallego, Richard; Bedrossian, Nora; Young, Sabrina; Czarnecki, Scarlett; Kennedy, Rebekah; Weiss, Brian D; Goldsmith, Kelly; Granger, Meaghan; Matthay, Katherine K; Groshen, Susan; Asgharzadeh, Shahab; Sposto, Richard; Seeger, Robert C

    2017-09-15

    Purpose: We determined whether quantifying neuroblastoma-associated mRNAs (NB-mRNAs) in bone marrow and blood improves assessment of disease and prediction of disease progression in patients with relapsed/refractory neuroblastoma. Experimental Design: mRNA for CHGA, DCX, DDC, PHOX2B, and TH was quantified in bone marrow and blood from 101 patients concurrently with clinical disease evaluations. Correlation between NB-mRNA (delta cycle threshold, Δ C t , for the geometric mean of genes from the TaqMan Low Density Array NB5 assay) and morphologically defined tumor cell percentage in bone marrow, 123 I-meta-iodobenzylguanidine (MIBG) Curie score, and CT/MRI-defined tumor longest diameter was determined. Time-dependent covariate Cox regression was used to analyze the relationship between Δ C t and progression-free survival (PFS). Results: NB-mRNA was detectable in 83% of bone marrow (185/223) and 63% (89/142) of blood specimens, and their Δ C t values were correlated (Spearman r = 0.67, P neuroblastoma. Clin Cancer Res; 23(18); 5374-83. ©2017 AACR . ©2017 American Association for Cancer Research.

  13. Findings of skin and bones in mastocytosis

    International Nuclear Information System (INIS)

    Rohner, H.G.; Bartl, R.; Koischwitz, D.; Rodermund, O.E.

    1982-01-01

    The syndrome of mastocytosis can include isolated urticaria pigmentosa, systemic mastocytosis, or the extremely rare form of mast cell leucemia. Our investigations of many patients have shown more frequently than earlier suspected, that the mastocytosis is a systemic disease. The frequency of attacked bone marrow is noteworthy. Because of the inflammatorygranulomatous manifestation in bone marrow, considerations of the pathogenesis of an immune and reactive event are taken into account. The mast cell granulomas are mostly found in the endosteal region, which is the reason for frequenctly occurring bone lesions (half on all patients show bone lesions). The bone changes can develop generalized (osteoporosis-osteosclerosis) or localized (osteolytic-osteosclerotic foci). In clinical work bone biopsies and skeletal radiology are supplementing each other: bone biopsy and skin biopsy give the first diagnosis of mastocytosis and reveal the systemic disease; X-ray pictures give information of shape and dimension of the induced osteopathy. (orig.)

  14. Findings of skin and bones in mastocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Rohner, H.G.; Bartl, R.; Koischwitz, D.; Rodermund, O.E.

    1982-12-01

    The syndrome of mastocytosis can include isolated urticaria pigmentosa, systemic mastocytosis, or the extremely rare form of mast cell leucemia. Our investigations of many patients have shown more frequently than earlier suspected, that the mastocytosis is a systemic disease. The frequency of attacked bone marrow is noteworthy. Because of the inflammatory granulomatous manifestation in bone marrow, considerations of the pathogenesis of an immune and reactive event are taken into account. The mast cell granulomas are mostly found in the endosteal region, which is the reason for frequently occurring bone lesions (half of all patients show bone lesions). The bone changes can develop generalized (osteoporosis-osteosclerosis) or localized (osteolytic-osteosclerotic foci). In clinical work bone biopsies and skeletal radiology are supplementing each other: bone biopsy and skin biopsy give the first diagnosis of mastocytosis and reveal the systemic disease; X-ray pictures give information of shape and dimension of the induced osteopathy.

  15. Ex vivo exposure of bone marrow from chronic kidney disease donor rats to pravastatin limits renal damage in recipient rats with chronic kidney disease

    NARCIS (Netherlands)

    Koppen, A. van; Papazova, D.A.; Oosterhuis, N.R.; Gremmels, H.; Giles, R.H.; Fledderus, J.O.; Joles, J.A.; Verhaar, M.C.

    2015-01-01

    Introduction: Healthy bone marrow cell (BMC) infusion improves renal function and limits renal injury in a model of chronic kidney disease (CKD) in rats. However, BMCs derived from rats with CKD fail to retain beneficial effects, demonstrating limited therapeutic efficacy. Statins have been reported

  16. Ex vivo exposure of bone marrow from chronic kidney disease donor rats to pravastatin limits renal damage in recipient rats with chronic kidney disease

    NARCIS (Netherlands)

    van Koppen, Arianne; Papazova, Diana A.; Oosterhuis, Nynke R.; Gremmels, Hendrik; Giles, Rachel H.; Fledderus, Joost O.; Joles, Jaap A.; Verhaar, Marianne C.

    2015-01-01

    INTRODUCTION: Healthy bone marrow cell (BMC) infusion improves renal function and limits renal injury in a model of chronic kidney disease (CKD) in rats. However, BMCs derived from rats with CKD fail to retain beneficial effects, demonstrating limited therapeutic efficacy. Statins have been reported

  17. Magnetic resonance imaging of the bone marrow

    International Nuclear Information System (INIS)

    Baur-Melnyk, Andrea

    2013-01-01

    The first book devoted to MRI of the bone marrow. Describes the MRI appearances of normal bone marrows and the full range of bone marrow disorders. Discusses the role of advanced MRI techniques and contrast enhancement. On account of its unrivalled imaging capabilities and sensitivity, magnetic resonance imaging (MRI) is considered the modality of choice for the investigation of physiologic and pathologic processes affecting the bone marrow. This book describes the MRI appearances of both the normal bone marrow, including variants, and the full range of bone marrow disorders. Detailed discussion is devoted to malignancies, including multiple myeloma, lymphoma, chronic myeloproliferative disorders, leukemia, and bone metastases. Among the other conditions covered are benign and malignant compression fractures, osteonecrosis, hemolytic anemia, Gaucher's disease, bone marrow edema syndrome, trauma, and infective and non-infective inflammatory disease. Further chapters address the role of MRI in assessing treatment response, the use of contrast media, and advanced MRI techniques. Magnetic Resonance Imaging of the Bone Marrow represents an ideal reference for both novice and experienced practitioners.

  18. Magnetic resonance imaging of the bone marrow

    Energy Technology Data Exchange (ETDEWEB)

    Baur-Melnyk, Andrea (ed.) [Klinikum der Univ. Muenchen (Germany). Inst. fuer Klinische Radiologie

    2013-08-01

    The first book devoted to MRI of the bone marrow. Describes the MRI appearances of normal bone marrows and the full range of bone marrow disorders. Discusses the role of advanced MRI techniques and contrast enhancement. On account of its unrivalled imaging capabilities and sensitivity, magnetic resonance imaging (MRI) is considered the modality of choice for the investigation of physiologic and pathologic processes affecting the bone marrow. This book describes the MRI appearances of both the normal bone marrow, including variants, and the full range of bone marrow disorders. Detailed discussion is devoted to malignancies, including multiple myeloma, lymphoma, chronic myeloproliferative disorders, leukemia, and bone metastases. Among the other conditions covered are benign and malignant compression fractures, osteonecrosis, hemolytic anemia, Gaucher's disease, bone marrow edema syndrome, trauma, and infective and non-infective inflammatory disease. Further chapters address the role of MRI in assessing treatment response, the use of contrast media, and advanced MRI techniques. Magnetic Resonance Imaging of the Bone Marrow represents an ideal reference for both novice and experienced practitioners.

  19. Aspects of osteo-articular complications in sickle-cell disease on planar bone scintigraphy (infection excluded). Apropos of three cases

    International Nuclear Information System (INIS)

    Oufroukhi, Y.; Biyi, A.; Zekri, A.; Doudouh, A.

    2008-01-01

    Skeletal complications of sickle-cell anemia are multiple and can appear on the acute (osseous infarction, acute osteomyelitis) or chronic mode (osteonecrosis, chronic osteomyelitis). The radio-labelled diphosphonate bone scintigraphy remains an important tool in the early diagnosis and in the follow-up of these complications and must form part of the initial assessment of the disease. Through clinical observations, the authors undertake to sum up the bone scintigraphy aspects of these complications. (author)

  20. Comparing three novel endpoints for developmental osteotoxicity in the embryonic stem cell test

    International Nuclear Information System (INIS)

    Nieden, Nicole I. zur; Davis, Lesley A.; Rancourt, Derrick E.

    2010-01-01

    Birth defects belong to the most serious side effects of pharmaceutical compounds or environmental chemicals. In vivo, teratogens most often affect the normal development of bones, causing growth retardation, limb defects or craniofacial malformations. The embryonic stem cell test (EST) is one of the most promising models that allow the in vitro prediction of embryotoxicity, with one of its endpoints being bone tissue development. The present study was designed to describe three novel inexpensive endpoints to assess developmental osteotoxicity using the model compounds penicillin G (non-teratogenic), 5-fluorouracil (strong teratogen) and all-trans retinoic acid (bone teratogen). These three endpoints were: quantification of matrix incorporated calcium by (1) morphometric analysis and (2) measurement of calcium levels as well as (3) activity of alkaline phosphatase, an enzyme involved in matrix calcification. To evaluate our data, we have compared the concentration curves and resulting ID 50 s of the new endpoints with mRNA expression for osteocalcin. Osteocalcin is an exclusive marker found only in mineralized tissues, is regulated upon compound treatment and reliably predicts the potential of a chemical entity acting as a bone teratogen. By comparing the new endpoints to quantitative expression of osteocalcin, which we previously identified as suitable to detect developmental osteotoxicity, we were ultimately able to illustrate IMAGE analysis and Ca 2+ deposition assays as two reliable novel endpoints for the EST. This is of particular importance for routine industrial assessment of novel compounds as these two new endpoints may substitute previously used molecular read-out methods, which are often costly and time-consuming.