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Sample records for bone diseases developmental

  1. Rheumatological presentation of developmental bone diseases

    Energy Technology Data Exchange (ETDEWEB)

    Kalifa, Gabriel; Cohen, Pierre alain; Hamidou, Amine

    2000-02-01

    Developmental bone disease may be present, with rheumatological disorders as the major symptoms, even in children. The major lesions encountered are early osteo arthritis, osteo chondromatosis and vertebral involvement with two leading types, pseudo Scheuermann's disease or pseudo ankylosing spondylitis. This paper presents the different features and lists the rheumatological problems in bone dysplasia.

  2. Bone Diseases

    Science.gov (United States)

    ... avoid smoking and drinking too much alcohol. Bone diseases can make bones easy to break. Different kinds ... break Osteogenesis imperfecta makes your bones brittle Paget's disease of bone makes them weak Bones can also ...

  3. [Bone diseases].

    Science.gov (United States)

    Uebelhart, Brigitte; Rizzoli, René

    2016-01-13

    Calcium intake shows a small impact on bone mineral density and fracture risk. Denosumab is a more potent inhibitor of bone resorption than zoledronate. Abaloparatide, PTHrP analog, increases bone mineral density and decreases fracture incidence. Teriparatide could be delivered via a transdermic device. Romosozumab and odanacatib improve calculated bone strength. Sequential or combined treatments with denosumab and teriparatide could be of interest, but not denosumab followed by teriparatide. Fibrous dysplasia, Paget disease and hypophosphatasia are updated, as well as atypical femoral fracture and osteonecrosis of the jaw. PMID:26946704

  4. Developmental orthopaedic diseases in foals

    International Nuclear Information System (INIS)

    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

  5. Bone Marrow Diseases

    Science.gov (United States)

    ... that help with blood clotting. With bone marrow disease, there are problems with the stem cells or ... marrow makes too many white blood cells Other diseases, such as lymphoma, can spread into the bone ...

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

    Energy Technology Data Exchange (ETDEWEB)

    Mikosch, P. [Dept. of Nuclear Medicine and Endocrinology, State Hospital Klagenfurt (Austria); Dept. of Internal Medicine II, State Hospital Klagenfurt (Austria); Zitter, F. [Dept. of Internal Medicine II, State Hospital Klagenfurt (Austria); Gallowitsch, H.J.; Lind, P. [Dept. of Nuclear Medicine and Endocrinology, State Hospital Klagenfurt (Austria); Wuertz, F. [Dept. of Pathology, State Hospital Klagenfurt (Austria); Mehta, A.B.; Hughes, D.A. [Lysosomal Storage Disorder Unit, Dept. of Academic Haematology, Royal Free and Univ. Coll. Medical School, London (United Kingdom)

    2008-07-01

    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.

  7. Hypercalciuric Bone Disease

    Science.gov (United States)

    Favus, Murray J.

    2008-09-01

    Hypercalciuria plays an important causal role in many patients with calcium oxalate (CaOx) stones. The source of the hypercalciuria includes increased intestinal Ca absorption and decreased renal tubule Ca reabsorption. In CaOx stone formers with idiopathic hypercalciuria (IH), Ca metabolic balance studies have revealed negative Ca balance and persistent hypercalciuria in the fasting state and during low dietary Ca intake. Bone resorption may also contribute to the high urine Ca excretion and increase the risk of bone loss. Indeed, low bone mass by DEXA scanning has been discovered in many IH patients. Thiazide diuretic agents reduce urine Ca excretion and may increase bone mineral density (BMD), thereby reducing fracture risk. Dietary Ca restriction that has been used unsuccessfully in the treatment of CaOx nephrolithiasis in the past may enhance negative Ca balance and accelerate bone loss. DEXA scans may demonstrate low BMD at the spine, hip, or forearm, with no predictable pattern. The unique pattern of bone histologic changes in IH differs from other causes of low DEXA bone density including postmenopausal osteoporosis, male hypogonadal osteoporosis, and glucocorticoid-induced osteoporosis. Hypercalciuria appears to play an important pathologic role in the development of low bone mass, and therefore correction of urine Ca losses should be a primary target for treatment of the bone disease accompanying IH.

  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. PMID:24811356

  9. Investigations of Diabetic Bone Disease

    DEFF Research Database (Denmark)

    Linde, Jakob Starup

    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...... 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 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...

  10. Metastatic Bone Disease

    Science.gov (United States)

    ... concern for patients with MBD is the general loss in quality of life. How much of an effect MBD has on ... to be most effective in maintaining quality of life. A technetium bone scan ... blood count, because loss of red blood cells (anemia) is a frequent ...

  11. Bone Mineralization in Celiac Disease

    Directory of Open Access Journals (Sweden)

    Tiziana Larussa

    2012-01-01

    Full Text Available Evidence indicates a well-established relationship between low bone mineral density (BMD and celiac disease (CD, but data on the pathogenesis of bone derangement in this setting are still inconclusive. In patients with symptomatic CD, low BMD appears to be directly related to the intestinal malabsorption. Adherence to a strict gluten-free diet (GFD will reverse the histological changes in the intestine and also the biochemical evidence of calcium malabsorption, resulting in rapid increase of BMD. Nevertheless, GFD improves BMD but does not normalize it in all patients, even after the recovery of intestinal mucosa. Other mechanisms of bone injury than calcium and vitamin D malabsorption are thought to be involved, such as proinflammatory cytokines, parathyroid function abnormalities, and misbalanced bone remodeling factors, most of all represented by the receptor activator of nuclear factor B/receptor activator of nuclear factor B-ligand/osteoprotegerin system. By means of dual-energy X-ray absorptiometry (DXA, it is now rapid and easy to obtain semiquantitative values of BMD. However, the question is still open about who and when submit to DXA evaluation in CD, in order to estimate risk of fractures. Furthermore, additional information on the role of nutritional supplements and alternative therapies is needed.

  12. 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.

  13. Myeloma bone disease: Pathophysiology and management

    OpenAIRE

    Silbermann, Rebecca; Roodman, G. David

    2013-01-01

    Multiple myeloma bone disease is marked by severe dysfunction of both bone formation and resorption and serves as a model for understanding the regulation of osteoblasts (OBL) and osteoclasts (OCL) in cancer. Myeloma bone lesions are purely osteolytic and are associated with severe and debilitating bone pain, pathologic fractures, hypercalcemia, and spinal cord compression, as well as increased mortality. Interactions within the bone marrow microenvironment in myeloma are responsible for the ...

  14. Cellular Mechanisms of Multiple Myeloma Bone Disease

    OpenAIRE

    Angela Oranger; Claudia Carbone; Maddalena Izzo; Maria Grano

    2013-01-01

    Multiple myeloma (MM) is a hematologic malignancy of differentiated plasma cells that accumulates and proliferates in the bone marrow. MM patients often develop bone disease that results in severe bone pain, osteolytic lesions, and pathologic fractures. These skeletal complications have not only a negative impact on quality of life but also a possible effect in overall survival. MM osteolytic bone lesions arise from the altered bone remodeling due to both increased osteoclast activation and d...

  15. Pain and Paget's Disease of Bone

    Science.gov (United States)

    ... Home Paget’s Disease of Bone Paget’s Disease Management Pain and Paget’s Disease of Bone Publication available in: ... focus(); */ } //--> Print-Friendly Page May 2015 Types of Pain Paget’s disease can cause several different kinds of ...

  16. Developmental Programming of Hypertension and Kidney Disease

    Directory of Open Access Journals (Sweden)

    Euming Chong

    2012-01-01

    Full Text Available A growing body of evidence supports the concept that changes in the intrauterine milieu during “sensitive” periods of embryonic development or in infant diet after birth affect the developing individual, resulting in general health alterations later in life. This phenomenon is referred to as “developmental programming” or “developmental origins of health and disease.” The risk of developing late-onset diseases such as hypertension, chronic kidney disease (CKD, obesity or type 2 diabetes is increased in infants born prematurely at <37 weeks of gestation or in low birth weight (LBW infants weighing <2,500 g at birth. Both genetic and environmental events contribute to the programming of subsequent risks of CKD and hypertension in premature or LBW individuals. A number of observations suggest that susceptibility to subsequent CKD and hypertension in premature or LBW infants is mediated, at least in part, by reduced nephron endowment. The major factors influencing in utero environment that are associated with a low final nephron number include uteroplacental insufficiency, maternal low-protein diet, hyperglycemia, vitamin A deficiency, exposure to or interruption of endogenous glucocorticoids, and ethanol exposure. This paper discusses the effect of premature birth, LBW, intrauterine milieu, and infant feeding on the development of hypertension and renal disease in later life as well as examines the role of the kidney in developmental programming of hypertension and CKD.

  17. Photodynamic therapy of diseased bone

    Science.gov (United States)

    Bisland, Stuart K.; Yee, Albert; Siewerdsen, Jeffery; Wilson, Brian C.; Burch, Shane

    2005-08-01

    Objective: Photodynamic therapy (PDT) defines the oxygen-dependent reaction that occurs upon light-mediated activation of a photosensitizing compound, culminating in the generation of cytotoxic, reactive oxygen species, predominantly, singlet oxygen. We are investigating PDT treatment of diseased bone. Methods: Using a rat model of human breast cancer (MT-1)-derived bone metastasis we confirmed the efficacy of benzoporphyrin-derivative monoacid (BPD-MA)-PDT for treating metastatic lesions within vertebrae or long bones. Results: Light administration (150 J) 15 mins after BPDMA (2.5 mg/Kg, i.v.) into the lumbar (L3) vertebra of rats resulted in complete ablation of the tumour and surrounding bone marrow 48 hrs post-PDT without paralysis. Porcine vertebrae provided a model comparable to that of human for light propagation (at 150 J/cm) and PDT response (BPD-MA; 6 mg/m2, i.v.) in non-tumour vertebrae. Precise fibre placement was afforded by 3-D cone beam computed tomography. Average penetration depth of light was 0.16 +/- 0.04 cm, however, the necrotic/non-necrotic interface extended 0.6 cm out from the treatment fiber with an average incident fluence rate of 4.3 mW/cm2. Non-necrotic tissue damage was evident 2 cm out from the treatment fiber. Current studies involving BPD-MA-PDT treatment of primary osteosarcomas in the forelimbs of dogs are very promising. Magnetic resonance imaging 24 hr post treatment reveal well circumscribed margins of treatment that encompass the entire 3-4 cm lesion. Finally, we are also interested in using 5-aminolevulinic acid (ALA) mediated PDT to treat osteomyelitis. Response to therapy was monitored as changes in bioluminescence signal of staphylococcus aureus (SA)-derived biofilms grown onto 0.5 cm lengths of wire and subjected to ALA-PDT either in vitro or in vivo upon implant into the intramedullary space of rat tibia. Transcutaneous delivery of PDT (75 J/cm2) effectively eradicated SAbiofilms within bone. Conclusions: Results support

  18. Developmental origins of inflammatory and immune diseases.

    Science.gov (United States)

    Chen, Ting; Liu, Han-Xiao; Yan, Hui-Yi; Wu, Dong-Mei; Ping, Jie

    2016-08-01

    Epidemiological and experimental animal studies show that suboptimal environments in fetal and neonatal life exert a profound influence on physiological function and risk of diseases in adult life. The concepts of the 'developmental programming' and Developmental Origins of Health and Diseases (DOHaD) have become well accepted and have been applied across almost all fields of medicine. Adverse intrauterine environments may have programming effects on the crucial functions of the immune system during critical periods of fetal development, which can permanently alter the immune function of offspring. Immune dysfunction may in turn lead offspring to be susceptible to inflammatory and immune diseases in adulthood. These facts suggest that inflammatory and immune disorders might have developmental origins. In recent years, inflammatory and immune disorders have become a growing health problem worldwide. However, there is no systematic report in the literature on the developmental origins of inflammatory and immune diseases and the potential mechanisms involved. Here, we review the impacts of adverse intrauterine environments on the immune function in offspring. This review shows the results from human and different animal species and highlights the underlying mechanisms, including damaged development of cells in the thymus, helper T cell 1/helper T cell 2 balance disturbance, abnormal epigenetic modification, effects of maternal glucocorticoid overexposure on fetal lymphocytes and effects of the fetal hypothalamic-pituitary-adrenal axis on the immune system. Although the phenomena have already been clearly implicated in epidemiologic and experimental studies, new studies investigating the mechanisms of these effects may provide new avenues for exploiting these pathways for disease prevention. PMID:27226490

  19. Developmental origins of inflammatory and immune diseases.

    Science.gov (United States)

    Chen, Ting; Liu, Han-Xiao; Yan, Hui-Yi; Wu, Dong-Mei; Ping, Jie

    2016-08-01

    Epidemiological and experimental animal studies show that suboptimal environments in fetal and neonatal life exert a profound influence on physiological function and risk of diseases in adult life. The concepts of the 'developmental programming' and Developmental Origins of Health and Diseases (DOHaD) have become well accepted and have been applied across almost all fields of medicine. Adverse intrauterine environments may have programming effects on the crucial functions of the immune system during critical periods of fetal development, which can permanently alter the immune function of offspring. Immune dysfunction may in turn lead offspring to be susceptible to inflammatory and immune diseases in adulthood. These facts suggest that inflammatory and immune disorders might have developmental origins. In recent years, inflammatory and immune disorders have become a growing health problem worldwide. However, there is no systematic report in the literature on the developmental origins of inflammatory and immune diseases and the potential mechanisms involved. Here, we review the impacts of adverse intrauterine environments on the immune function in offspring. This review shows the results from human and different animal species and highlights the underlying mechanisms, including damaged development of cells in the thymus, helper T cell 1/helper T cell 2 balance disturbance, abnormal epigenetic modification, effects of maternal glucocorticoid overexposure on fetal lymphocytes and effects of the fetal hypothalamic-pituitary-adrenal axis on the immune system. Although the phenomena have already been clearly implicated in epidemiologic and experimental studies, new studies investigating the mechanisms of these effects may provide new avenues for exploiting these pathways for disease prevention.

  20. Developmental Programming, a Pathway to Disease.

    Science.gov (United States)

    Padmanabhan, Vasantha; Cardoso, Rodolfo C; Puttabyatappa, Muraly

    2016-04-01

    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

  1. 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 reproductive and developmental environment on lifelong health, a concept known as the Developmental Origins of Health and Disease (DOHaD). Environmental exposures including paren...

  2. Cat-Scratch Disease With Bone Involvemnet

    OpenAIRE

    Maia, R; Brito, MJ; Sousa, R.; Gouveia, C.

    2011-01-01

    Background: Bartonella henselae infection typically presents as a self-limiting regional lymphadenopathy. Bone involvement is a very rare form of the disease. Aims: To describe bone infection associated to cat-scratch disease (CSD) in a portuguese pediatric hospital. Methods: Clinical records of children admitted at the hospital with the diagnosis of CSD associated bone infection, during 2010, were reviewed. Diagnosis was confirmed by serology using indirect fluorescence assay ...

  3. Bone Diseases - Multiple Languages: MedlinePlus

    Science.gov (United States)

    ... Supplements Videos & Tools You Are Here: Home → Multiple Languages → All Health Topics → Bone Diseases URL of this page: https://medlineplus.gov/languages/bonediseases.html Other topics A-Z A B ...

  4. Bone changes in alcoholic liver disease

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    Alcoholism has been associated with growth impairment,osteomalacia, delayed fracture healing, and asepticnecrosis (primarily necrosis of the femoral head), butthe main alterations observed in the bones of alcoholicpatients are osteoporosis and an increased risk offractures. Decreased bone mass is a hallmark of osteoporosis,and it may be due either to decreased bone synthesis and/or to increased bone breakdown. Ethanolmay affect both mechanisms. It is generally acceptedthat ethanol decreases bone synthesis, and most authorshave reported decreased osteocalcin levels (a "marker" ofbone synthesis), but some controversy exists regardingthe effect of alcohol on bone breakdown, and, indeed,disparate results have been reported for telopeptideand other biochemical markers of bone resorption.In addition to the direct effect of ethanol, systemicalterations such as malnutrition, malabsorption, liverdisease, increased levels of proinflammatory cytokines,alcoholic myopathy and neuropathy, low testosteronelevels, and an increased risk of trauma, play contributoryroles. The treatment of alcoholic bone disease should beaimed towards increasing bone formation and decreasingbone degradation. In this sense, vitamin D and calciumsupplementation, together with biphosphonates areessential, but alcohol abstinence and nutritional improvementare equally important. In this review we study thepathogenesis of bone changes in alcoholic liver diseaseand discuss potential therapies.

  5. Oral Health and Bone Disease

    Science.gov (United States)

    ... for periodontitis and tooth loss. Role of the Dentist and Dental X Rays Research supported by the ... normal bone density. Because many people see their dentist more regularly than their doctor, dentists are in ...

  6. Role of bone scan in rheumatic disease

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Yun Young [Hanyang University College of Medicine, Seoul (Korea, Republic of)

    2003-06-01

    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.

  7. 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.

  8. Bone scan in inflammatory osseous disease

    International Nuclear Information System (INIS)

    The /sup 99m/Tc-phosphate bone scan has become a sensitive, reliable, and safe method for evaluating the patient with suspected inflammatory disease of bone. The scan may become positive as early as the first 24 hr after the symptoms and 10-14 days before roentgenographic changes occur. It can be used to differentiate successfully a variety of diseases from osteomyelitis, and in conjunction with 67Ga-citrate scan has become a mainstay in the work-up of the patient with infectious disease. Applications of the bone scan to infectious diseases in pediatric practice are especially helpful, since these diseases are common problems in this age group. Increased experience with the /sup 99m/Tc-phosphate bone scan has already defined several areas of ''limitations'' in evaluating inflammatory disease. ''Cold'' defects, negative scans in early stages of osteomyelitis, and ''extended uptake'' may all pose problems in interpretation, but careful correlation of the bone scan results with clinical history and physical findings, blood cultures, and roentgenography will significantly reduce these problems

  9. [The new bone formation and bone metabolism in Forestier disease].

    Science.gov (United States)

    Kotrych, Daniel; Bohatyrewicz, Andrzej; Woźniak, Wojciech; Zietek, Paweł; Kołodziej, Łukasz; Karaczun, Maciej; Grzegorczyk, Wojciech; Antoniak, Krzysztof

    2008-01-01

    The study was performed on 36 male patients between 65 and 83 years who were either hospitalised or treated in the out-patients clinic due to Forestier's disease. The aim of the study was to evaluate the advance of ectopic bone formation process in cervical spine and bony metabolic changes in treated patients. The study showed reverse corelation between the degree of advance of cervical hyperostosis and the prevalence of osteoporosis and metabolic disorders in the tested group. The authors have emphasized the need of precise evaluation and differentiation of Forestier's disease and degenerative spine disease. PMID:18847002

  10. The Pathogenesis of the Bone Disease of Multiple Myeloma

    OpenAIRE

    Edwards, Claire M; Zhuang, Junling; Mundy, Gregory R.

    2008-01-01

    Multiple myeloma is a fatal hematologic malignancy associated with clonal expansion of malignant plasma cells within the bone marrow and the development of a destructive osteolytic bone disease. The principal cellular mechanisms involved in the development of myeloma bone disease are an increase in osteoclastic bone resorption, and a reduction in bone formation. Myeloma cells are found in close association with sites of active bone resorption, and the interactions between myeloma cells, and o...

  11. Developmental Disabilities and Alzheimer's Disease...What You Should Know.

    Science.gov (United States)

    Arc, Arlington, TX.

    This booklet provides an overview of Alzheimer's disease along with a description of the disease, how to find out if someone has it, and how it affects adults with developmental disabilities. It also provides information on what to do and suggests where to seek help. Specific sections discuss: (1) the etiology of the disease; (2) symptoms of…

  12. What Is Paget's Disease of Bone?

    Science.gov (United States)

    ... with Paget’s disease do not need a special diet. But, to maintain strong bones, you should get 1,200 mg of calcium and at least 400 IU of vitamin D every day. After age 70, you should take 600 IU of vitamin D each day. If you have had kidney stones, talk with your doctor about how much calcium ...

  13. Diabetes mellitus related bone metabolism and periodontal disease

    Institute of Scientific and Technical Information of China (English)

    Ying-Ying Wu; E Xiao; Dana T Graves

    2015-01-01

    Diabetes mellitus and periodontal disease are chronic diseases affecting a large number of populations worldwide. Changed bone metabolism is one of the important long-term complications associated with diabetes mellitus. Alveolar bone loss is one of the main outcomes of periodontitis, and diabetes is among the primary risk factors for periodontal disease. In this review, we summarise the adverse effects of diabetes on the periodontium in periodontitis subjects, focusing on alveolar bone loss. Bone remodelling begins with osteoclasts resorbing bone, followed by new bone formation by osteoblasts in the resorption lacunae. Therefore, we discuss the potential mechanism of diabetes-enhanced bone loss in relation to osteoblasts and osteoclasts.

  14. Impact of bone marrow on respiratory disease.

    Science.gov (United States)

    Rankin, Sara M

    2008-06-01

    The bone marrow is not only a site of haematopoiesis but also serves as an important reservoir for mature granulocytes and stem cells, including haematopoietic stem cells, mesenchymal stem cells and fibrocytes. In respiratory diseases, such as asthma and idiopathic pulmonary fibrosis these cells are mobilised from the bone marrow in response to blood-borne mediators and subsequently recruited to the lungs. Although the granulocytes contribute to the inflammatory reaction, stem cells may promote tissue repair or remodelling. Understanding the factors and molecular mechanisms that regulate the mobilisation of granulocytes and stem cells from the bone marrow may lead to the identification of novel therapeutic targets for the treatment of a wide range of respiratory disorders. PMID:18372214

  15. Osteoporosis and adynamic bone in chronic kidney disease

    OpenAIRE

    Cannata, J.B. (Jorge); Rodríguez, Minerva; Gómez, Carlos

    2013-01-01

    Among the chronic kidney disease–mineral bone disease (CKD-MBD) disorders, osteoporosis and adynamic bone are highly prevalent, and they have been consistently associated with low bone mass, bone fractures, vascular calcifications and greater mortality in general and CKD populations. Despite the fact that osteoporosis and adynamic bone have similar clinical outcomes, they have different pathogeneses and clinical management. In osteoporosis, there is a lack of balance between bone format...

  16. Bone histology in chronic kidney disease-related mineral and bone disorder.

    Science.gov (United States)

    Kazama, Junichiro James

    2011-06-01

    A quantitative histological analysis of biopsied bone samples is currently regarded as the gold standard for a diagnosing procedure for bone diseases associated with chronic kidney disease-related mineral and bone disorder. Conventionally, "bone cell activities" and "bone mineralization" are applied as two independent assessment axes, and the histology results are classified into five categories according to these axes. Recently, a new bone histology classification system called the Turnover-Mineralization-Volume system, which applied "cancellous bone volume" as another major assessing axis, was advocated; however, both classification systems have many unsolved problems. Clinicians must realize the limitations in evaluating bone metabolism by bone histology. We will need to establish a new classification method for renal bone diseases independent of histological findings.

  17. Pathophysiology of chronic kidney disease-mineral and bone disorder.

    Science.gov (United States)

    Mac Way, Fabrice; Lessard, Myriam; Lafage-Proust, Marie-Hélène

    2012-12-01

    Chronic kidney disease (CKD) alters the metabolism of several minerals, thereby inducing bone lesions and vessel-wall calcifications that can cause functional impairments and excess mortality. The histological bone abnormalities seen in CKD, known as renal osteodystrophy, consist of alterations in the bone turnover rate, which may be increased (osteitis fibrosa [OF]) or severely decreased (adynamic bone disease [AD]); abnormal mineralization (osteomalacia [OM]), and bone loss. Secondary hyperparathyroidism is related to early phosphate accumulation (responsible for FGF23 overproduction by bone tissue), decreased calcitriol production by the kidneys, and hypocalcemia. Secondary hyperparathyroidism is associated with OF. Other factors that affect bone include acidosis, chronic inflammation, nutritional deficiencies, and iatrogenic complications.

  18. 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.

  19. 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 c

  20. [Bone and Calcium Metabolisms Associated with Dental and Oral-Maxillofacial Diseases. Bone remodeling and alveolar bone homeostasis].

    Science.gov (United States)

    Nakashima, Tomoki

    2015-08-01

    Bone, which support motile organ and periodontal tissue, is renewing throughout our life. This restructuring process is called "bone remodeling" , and osteoclasts and osteoblasts play a crucial role in this process. 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. Alveolar bone remodeling is directly influenced by occlusal force from the teeth. Thus, the elucidation of the regulatory mechanisms involved in alveolar bone remodeling is critical for a deeper understanding of the maintenance of healthy tooth and dental disease.

  1. 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.

  2. Gender differences in developmental programming of cardiovascular diseases.

    Science.gov (United States)

    Dasinger, John Henry; Alexander, Barbara T

    2016-03-01

    Hypertension is a risk factor for cardiovascular disease, the leading cause of death worldwide. Although multiple factors contribute to the pathogenesis of hypertension, studies by Dr David Barker reporting an inverse relationship between birth weight and blood pressure led to the hypothesis that slow growth during fetal life increased blood pressure and the risk for cardiovascular disease in later life. It is now recognized that growth during infancy and childhood, in addition to exposure to adverse influences during fetal life, contributes to the developmental programming of increased cardiovascular risk. Numerous epidemiological studies support the link between influences during early life and later cardiovascular health; experimental models provide proof of principle and indicate that numerous mechanisms contribute to the developmental origins of chronic disease. Sex has an impact on the severity of cardiovascular risk in experimental models of developmental insult. Yet, few studies examine the influence of sex on blood pressure and cardiovascular health in low-birth weight men and women. Fewer still assess the impact of ageing on sex differences in programmed cardiovascular risk. Thus, the aim of the present review is to highlight current data about sex differences in the developmental programming of blood pressure and cardiovascular disease. PMID:26814204

  3. Gender differences in developmental programming of cardiovascular diseases.

    Science.gov (United States)

    Dasinger, John Henry; Alexander, Barbara T

    2016-03-01

    Hypertension is a risk factor for cardiovascular disease, the leading cause of death worldwide. Although multiple factors contribute to the pathogenesis of hypertension, studies by Dr David Barker reporting an inverse relationship between birth weight and blood pressure led to the hypothesis that slow growth during fetal life increased blood pressure and the risk for cardiovascular disease in later life. It is now recognized that growth during infancy and childhood, in addition to exposure to adverse influences during fetal life, contributes to the developmental programming of increased cardiovascular risk. Numerous epidemiological studies support the link between influences during early life and later cardiovascular health; experimental models provide proof of principle and indicate that numerous mechanisms contribute to the developmental origins of chronic disease. Sex has an impact on the severity of cardiovascular risk in experimental models of developmental insult. Yet, few studies examine the influence of sex on blood pressure and cardiovascular health in low-birth weight men and women. Fewer still assess the impact of ageing on sex differences in programmed cardiovascular risk. Thus, the aim of the present review is to highlight current data about sex differences in the developmental programming of blood pressure and cardiovascular disease.

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

    OpenAIRE

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

    2012-01-01

    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,...

  5. 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.

  6. 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.

  7. Polyphosphate bone scanning on non-malignant bone disease in children

    Energy Technology Data Exchange (ETDEWEB)

    Paul, D.J.; Gilday, D.L.

    1975-12-01

    The advent of /sup 99m/technetium phosphate bone scanning radiopharmaceuticals has opened new methods of investigation of pediatric bone diseases. In axial skeleton pain, suspected osteomyelitis, evaluation of vascular integrity and suspected but undetected fractures, the bone scan has proved to be a highly complementary study to the radiologic examination.

  8. 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. PMID:26051481

  9. Osteoporosis and adynamic bone in chronic kidney disease.

    Science.gov (United States)

    Cannata-Andía, Jorge B; Rodriguez García, Minerva; Gómez Alonso, Carlos

    2013-01-01

    Among the chronic kidney disease-mineral bone disease (CKD-MBD) disorders, osteoporosis and adynamic bone are highly prevalent, and they have been consistently associated with low bone mass, bone fractures, vascular calcifications and greater mortality in general and CKD populations. Despite the fact that osteoporosis and adynamic bone have similar clinical outcomes, they have different pathogeneses and clinical management. In osteoporosis, there is a lack of balance between bone formation and bone resorption, and less new bone is formed to replace bone losses. Osteoporosis is defined by the World Health Organization as "a disease characterized by low bone mineral density and micro architectural deterioration leading to low bone strength and increased risk of fractures." In the general population, there is a good correlation between dual-energy X-ray absorptiometry measurements and bone fractures, but this is not the case with CKD patients. Despite the fact that we have a great number of active antiosteoporotic drugs, the experience in CKD patients is limited. Adynamic bone is suspected based on biochemical parameters, mainly parathyroid hormone (PTH) and bone alkaline phosphatase, but it needs to be proven using a bone biopsy, where a low or zero bone formation rate and a reduction or absence of osteoblasts and osteoclasts should be found. The clinical management of adynamic bone has important limitations and currently does not allow taking many active measures. Treatment is mainly based on the prevention of risk factors known to induce PTH oversuppression, such as aluminium and calcium load and very high doses of vitamin D receptor activators. Due to the limitations in the treatment of both conditions, prevention plays a key role in the management of these disorders. PMID:23023723

  10. Lessons from rare diseases of cartilage and bone.

    Science.gov (United States)

    Gallagher, James A; Ranganath, Lakshminarayan R; Boyde, Alan

    2015-06-01

    Studying severe phenotypes of rare syndromes can elucidate disease mechanisms of more common disorders and identify potential therapeutic targets. Lessons from rare bone diseases contributed to the development of the most successful class of bone active agents, the bisphosphonates. More recent research on rare bone diseases has helped elucidate key pathways and identify new targets in bone resorption and bone formation including cathepsin K and sclerostin, for which drugs are now in clinical trials. By contrast, there has been much less focus on rare cartilage diseases and osteoarthritis (OA) remains a common disease with no effective therapy. Investigation of rare cartilage syndromes is identifying new potential targets in OA including GDF5 and lubricin. Research on the arthropathy of the ultra-rare disease alkaptonuria has identified several new features of the OA phenotype, including high density mineralized protrusions (HDMPs) which constitute a newly identified mechanism of joint destruction.

  11. Bone

    International Nuclear Information System (INIS)

    Bone scanning provides information on the extent of primary bone tumors, on possible metastatic disease, on the presence of osteomyelitis prior to observation of roentgenographic changes so that earlier therapy is possible, on the presence of collagen diseases, on the presence of fractures not disclosed by x-ray films, and on the evaluation of aseptic necrosis. However, the total effect and contribution of bone scanning to the diagnosis, treatment, and ultimate prognosis of pediatric skeletal diseases is, as yet, unknown. (auth)

  12. Asymptomatic Paget's disease of bone presenting with complete atrioventricular block

    Institute of Scientific and Technical Information of China (English)

    A.Rauoof Malik; Nazir A.Lone; Hilal A.Rather; Vicar M Jan; Javid A.Malik; Khursheed A.Khan; S.Jalal

    2008-01-01

    @@ Paget's disease of bone is a deforming bone disease (osteitis deformans) characterized by increased bone remodeling,bone hypertrophy,and abnormal bone structure,leading to bone expansion,deformities,easy fractures,and occasionally,neoplastic transformation.It is the second most common bone disorder after osteoporosis.1 The disease is relatively rare in Asia but is common in Europe and North America,affecting approximately 2% of the population over 50 years,although lately,a decline in the prevalence has been reported.2 Paget's disease commonly affects people in or past their middle age and is slightly more common in men than in women.1 The exact cause of Paget's disease is not known.Environmental agents,particularly paramyxoviral infections (measles and canine distemper viruses) have been postulated as potential etiological factors.3 Recently,a strong genetic component has been described,with candidate loci suggested at 18q,5q35-QTER,and particularly,the squestosome 1/p62.2,3 The pathological process in Paget's disease consists of one or more areas of aggressive and relentless osteoclastic activity,coupled with deposition of structurally abnormal excessive bone and matrix tissues.1,4 Most of the cases involve only one (monostotic) or few bones,particularly skull,vertebrae,pelvis,femur,and tibia.

  13. Alcohol-Induced Developmental Origins of Adult-Onset Diseases.

    Science.gov (United States)

    Lunde, Emilie R; Washburn, Shannon E; Golding, Michael C; Bake, Shameena; Miranda, Rajesh C; Ramadoss, Jayanth

    2016-07-01

    Fetal alcohol exposure may impair growth, development, and function of multiple organ systems and is encompassed by the term fetal alcohol spectrum disorders (FASD). Research has so far focused on the mechanisms, prevention, and diagnosis of FASD, while the risk for adult-onset chronic diseases in individuals exposed to alcohol in utero is not well explored. David Barker's hypothesis on Developmental Origins of Health and Disease (DOHaD) suggests that insults to the milieu of the developing fetus program it for adult development of chronic diseases. In the 25 years since the introduction of this hypothesis, epidemiological and animal model studies have made significant advancements in identifying in utero developmental origins of chronic adult-onset diseases affecting cardiovascular, endocrine, musculoskeletal, and psychobehavioral systems. Teratogen exposure is an established programming agent for adult diseases, and recent studies suggest that prenatal alcohol exposure correlates with adult onset of neurobehavioral deficits, cardiovascular disease, endocrine dysfunction, and nutrient homeostasis instability, warranting additional investigation of alcohol-induced DOHaD, as well as patient follow-up well into adulthood for affected individuals. In utero epigenetic alterations during critical periods of methylation are a key potential mechanism for programming and susceptibility of adult-onset chronic diseases, with imprinted genes affecting metabolism being critical targets. Additional studies in epidemiology, phenotypic characterization in response to timing, dose, and duration of exposure, as well as elucidation of mechanisms underlying FASD-DOHaD inter relation, are thus needed to clinically define chronic disease associated with prenatal alcohol exposure. These studies are critical to establish interventional strategies that decrease incidence of these adult-onset diseases and promote healthier aging among individuals affected with FASD.

  14. 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

  15. Zoledronic acid in the management of metastatic bone disease

    Directory of Open Access Journals (Sweden)

    Thomas J Polascik

    2008-03-01

    Full Text Available Thomas J Polascik, Vladimir MouravievDuke Prostate Center and Division of Urologic Surgery, Duke University Medical Center, Durham, NC, USAAbstract: Many patients with advanced cancer experience decreased bone strength due to metastatic foci, underlying osteoporosis and/or cancer treatment induced bone loss. The clinical consequences of metastatic disease involving the skeleton are widespread. This review focuses on the efficacy, pharmacology, and safety when using intravenous biphosphonate such a zoledronic acid for cancer bone metastases. Zoledronic acid is the gold standard for the medical management of metastatic bone disease. The indications for treatment include prevention of skeletal relevant events (SRE, osteoporotic complications, and palliation of bone pain, among others. Zoledronic acid is the only bisphosphonate effective in decreasing SREs associated with bone metastases from advanced renal cell carcinoma and prostate cancer. Regarding prostate cancer, zoledronic acid effectively prevents both bone loss in patients with locally advanced disease receiving androgen deprivation therapy and SREs in men with hormone-refractory or hormonesensitive metastatic disease. Zoledronic acid has an acceptable safety profile and tolerability, and has been effective at significantly decreasing the incidence, delaying the onset, and reducing the overall risk of experiencing an SRE compared to placebo. It is the only bisphosphonate currently approved for the prevention and treatment of skeletal complications in patients with bone metastases due to all solid tumors.Keywords: zoledronic acid, metastatic bone disease, osteoporosis, skeletal relevant events, advanced prostate cancer

  16. [Developmental origins of cardiovascular disease and early intervention windows].

    Science.gov (United States)

    Mi, Jie

    2016-01-01

    Cardiovascular diseases are the major threat to human health and underlie almost half of all deaths in China. Even more serious, obesity and cardiometabolic risk factors have emerged to be prevalent in children and adolescents of some affluent regions. As scientific knowledge emerges on the role of nutritional factors and exposures to environmental risk factors in the developmental origins of health and disease, evidence suggests that it is imperative to create and implement early effective prevention strategies, including optimisation of nutrition at first 1 000 days in life course and reduction of risk factors of obesity exposures during whole childhood, to suppress the rising trend of cardiovascular disease, otherwise, the future costs of diagnosis and treatment are likely to be unaffordable.

  17. Bone marrow invasion in multiple myeloma and metastatic disease.

    Science.gov (United States)

    Vilanova, J C; Luna, A

    2016-04-01

    Magnetic resonance imaging (MRI) of the spine is the imaging study of choice for the management of bone marrow disease. MRI sequences enable us to integrate structural and functional information for detecting, staging, and monitoring the response the treatment of multiple myeloma and bone metastases in the spine. Whole-body MRI has been incorporated into different guidelines as the technique of choice for managing multiple myeloma and metastatic bone disease. Normal physiological changes in the yellow and red bone marrow represent a challenge in analyses to differentiate clinically significant findings from those that are not clinically significant. This article describes the findings for normal bone marrow, variants, and invasive processes in multiple myeloma and bone metastases.

  18. Developmental diseases and the hypothetical Master Development Program.

    Science.gov (United States)

    Parris, George E

    2010-03-01

    Small deletions and duplications frequently occur in the pericentromeric region of chromosomes and many of these are associated with developmental abnormalities. These developmental syndromes are conventionally attributed to abnormal expression of protein-coding genes in the affected region. A hypothesis has recently been published concerning a Master Development Program based on noncoding transcripts from these regions (Parris GE. A hypothetical Master Development Program for multi-cellular organisms: Ontogeny and phylogeny. Biosci Hypotheses 2009;2:3-12.). This paper summarizes and expands the recently published hypothesis to include it application to developmental diseases. The author proposes that development of multi-cellular organisms is guided by a Master Development Program (MDP) located primarily in the pericentromeric heterochromatin. The MDP is believed to consist of a series of Generation-Specific Control Keys (GSCK) transcribed in sequence by Ikaros family transcription factors unless the GSCKs are suppressed by Sall1-family or Dnmt3b-family proteins. The MDP is proposed to increment with each cell cycle to the next GSCK resulting in development of the clone. A clone may be programmed to split into two clones as necessary through a two-cycle mitosis processes. The transcripts of the GSCKs presumably yield noncoding nuclear messenger RNAs (nmRNAs, 8-30 nt units) that act directly (e.g., as primers for RNA polymerase II) and indirectly to regulate HOX and other high-level transcription factor and developmental genes. As envisioned, the MDP would evolve by terminal addition of new GSCKs. The new GSCKs are produced by evolutionary consolidation of retro-transcripts into pyknons that collect and evolve at the end of the pericentromeric heterochromatin and are eventually incorporated into the MDP. The retro-transcripts are though to be produced during episodic retrovirus epidemics and account for punctuated equilibrium in species evolution. PMID:19833446

  19. Cancer treatment-related bone disease

    OpenAIRE

    Brown, Sue A.; Guise, Theresa A.

    2009-01-01

    Bone health may be impaired in many patients being treated for cancer. Primary tumors that reside in or form metastases to bone can result in compromised skeletal integrity. It has also been increasingly recognized that patients undergoing therapies for treatment of cancer are at higher risk of bone loss. These include androgen-deprivation therapy for prostate cancer and aromatase inhibitor therapy for breast cancer among others. Hypogonadism induced by many of these cancer treatments results...

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

    International Nuclear Information System (INIS)

    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. 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.

  2. 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

  3. 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

  4. 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.

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

    Science.gov (United States)

    Blashki, Daniel; Murphy, Matthew B; Ferrari, Mauro; Simmons, Paul J; Tasciotti, Ennio

    2016-01-01

    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. PMID:27579159

  6. 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.

  7. 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...

  8. Disease-specific clinical problems associated with the subchondral bone

    NARCIS (Netherlands)

    D. Pape; G. Filardo; E. Kon; C.N. van Dijk; H. Madry

    2010-01-01

    The subchondral bone is involved in a variety of diseases affecting both the articular cartilage and bone. Osteochondral defects in distinct locations and of variable sizes are the final results of different etiologies. These include traumatic osteochondral defects, osteochondritis dissecans, osteon

  9. Bone Marrow Diseases - Multiple Languages: MedlinePlus

    Science.gov (United States)

    ... Supplements Videos & Tools You Are Here: Home → Multiple Languages → All Health Topics → Bone Marrow Diseases URL of this page: https://medlineplus.gov/languages/bonemarrowdiseases.html Other topics A-Z A B ...

  10. 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.

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

    International Nuclear Information System (INIS)

    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

  12. MANAGEMENT OF DISEASES OF LONG BONES WITH KUNTSCHER NAILS

    OpenAIRE

    Ravikant; Vibha; Singh,, G.; Srivastava

    2015-01-01

    BACKGROUND: AIMS: SETTINGS AND DESIGN : The aim of this study was, to devise economical, easy, simple, quick method of fixation of diseased long bones, so that pathological fracture could be prevented and to provide rigid fixation, in those cases which have already developed pathological fractu re, and to achieve arthrodesis. Ten, cases of long bone diseases were managed with the help of K nails, in the Department of Orthopaedics, in CIMS, between, December 2002 ...

  13. Anemia and bone disease of chronic kidney disease: pathogenesis, diagnosis, and management.

    Science.gov (United States)

    Shemin, Douglas

    2014-12-02

    Anemia and metabolic bone disease accompany chronic kidney disease (CKD), and worsen as CKD progresses. It is likely that both processes contribute to the increased morbidity and mortality seen in CKD. This paper briefly reviews the pathogenesis and diagnosis of anemia and bone disease in CKD, and summarizes recent consensus guidelines for treatment.

  14. 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.)

  15. Bone disease in calcium stone forming patients.

    Science.gov (United States)

    Heilberg, I P; Martini, L A; Szejnfeld, V L; Carvalho, A B; Draibe, S A; Ajzen, H; Ramos, O L; Schor, N

    1994-09-01

    The association between idiopathic hypercalciuria and osteopenia (OP) has been recently recognized. It is not established whether or not calcium intake plays a critical role in the loss of bone mass. Fifty-five calcium stone forming patients with either absorptive hypercalciuria (AH) or fasting hypercalciuria (FH), 29 males and 26 premenopausal females, were submitted to dual photon absorptiometry at lumbar spine. Calcium intake was assessed by a 72 hr dietary record. OP was detected in 20% (11/55) of patients, being more common among men, 9/26 (35%) than in women, 2/29 (7%), p < 0.05. Male FH patients presented lower mean bone mineral density (BMD) than sex, weight and age-matched control (1.058 +/- 0.18 vs 1.209 +/- 0.13 g/cm2, X +/- SD, p < 0.05). OP was more frequent in FH patients, 7/20 (35%) than in AH patients 4/35 (11%), albeit the difference was not statistically significant. There was no correlation between calcium intake and BMD measurement. Six osteopenic male FH patients were further submitted to histomorphometric evaluation with tetracycline double labeling. Bone volume was lower than the controls (13.2 +/- 3.0 vs 27.2 +/- 3.7%, p < 0.05). Osteoid surfaces were reduced, although not significantly (10.1 +/- 8.2% vs 15.9 +/- 6.7%). Eroded surfaces were markedly increased (23.9 +/- 13.4 vs 4.2 +/- 1.4%, p < 0.05). The bone formation rate was very low with a complete lack of tetracycline double labeling in 4 patients. These data suggest low bone volume, tendency to low bone formation, increased bone resorption and a severe mineralization defect, consistent with normal or low bone turnover osteoporosis. PMID:7994936

  16. The Potential Application of Pulsed Ultrasound on Bone Defect Repair via Developmental Engineering: An In Vitro Study.

    Science.gov (United States)

    Wang, Jue; Tang, Na; Xiao, Qiang; Zhao, Lixing; Li, Yu; Li, Juan; Wang, Jun; Zhao, Zhihe; Tan, Lijun

    2016-05-01

    Repairing bone defect by recapitulation of endochondral bone formation, known as developmental engineering, has been a promising approach in bone tissue engineering. The critical issue in this area is how to effectively construct the hypertrophic cartilaginous template in vitro and enhance in vivo endochondral ossification process after implantation. Pulsed ultrasound stimulation has been widely used in the clinic for accelerating bone healing in fractures and nonunions. The aim of this study was to investigate whether ultrasound (US) could accelerate in vitro chondrogenesis and the hypertrophic process in certain microenvironments. Rat bone marrow mesenchymal stem cells were chondrogenic or hypertrophic differentiated in a three-dimensional pellet culture system with different media, and treated with different intensities of US. US exposure promoted chondrogenic differentiation of stem cells and inhibited their transition into the hypertrophic stage in a chondrogenic-friendly microenvironment. US significantly advanced hypertrophic differentiation of bone marrow stem cell pellets in hypertrophic medium after chondrogenesis. Our data indicated that pulsed US promoted in vitro chondrogenic and hypertrophic differentiation of stem cell pellets in specific culture conditions. The present study proves the potential application of US in the in vitro stage of "developmental engineering" for bone development and repair. PMID:26526417

  17. Celiac Disease Presenting with Bone Pain: Two Case Reports

    OpenAIRE

    Nural Albayrak Aydın; Kamil Yazıcıoğlu

    2011-01-01

    Celiac disease or gluten sensitive enteropathy is an autoimmune disease characterized by inflammation of the small-bowel mucosa. As can be asymptomatic, involvement of the hematologic, gastrointestinal system, musculosceletal system, nervous system or endocrine system may occur as well. The presence of osteoporosis in celiac disease, may be the only sign of patients who have not been diagnosed yet. The direct effect of celiac disease on bones happens secondary to decreased absorbsion of calci...

  18. Bone disease in multiple myeloma and precursor disease: novel diagnostic approaches and implications on clinical management

    OpenAIRE

    Kristinsson, Sigurdur Y.; Minter, Alex R; Korde, Neha; TAN, ESTHER; Landgren, Ola

    2011-01-01

    The manifestations of bone involvement in patients with multiple myeloma (MM) can have devastating clinical effects and increase mortality. Recent studies demonstrate that patients with the precursor conditions smoldering MM (SMM) and monoclonal gammopathy of undetermined significance (MGUS) show evidence of bone disease and increased risk of fractures. The understanding of the pathogenesis of bone disease in MM has expanded in recent years. The traditional skeletal survey will probably be re...

  19. 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.

  20. Lipids and Bone Mineral Density in Patients with Vascular Disease

    Directory of Open Access Journals (Sweden)

    José-Luis Pérez-Castrillón

    2008-01-01

    Full Text Available Objective: To evaluate the relationship between cholesterol and triglycerides and bone mineral density in patients with vascular disease (hypertension and acute coronary syndrome.Methods: The study included 217 patients (83 men and 134 women, aged between 36 and 76 (mean age 59 ± 10, with hypertension and acute coronary syndrome. Information obtained included anthropometric measurements, total cholesterol, HDL cholesterol, LDL cholesterol and triglycerides; bone mineral density (BMD was recorded at the lumbar spine.Results: BMD was significantly lower in patients in the higher tertiles of cholesterol (p = 0.041. The effect was maintained after adjustment for age and Body Mass Index (BMI. However, there was no association between the range of triglycerides, LDL cholesterol, HDL cholesterol and bone mass.Conclusions: A relationship was found between total cholesterol and bone mineral density in patients with vascular disease.

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

    International Nuclear Information System (INIS)

    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)

  2. [Insights into cystic fibrosis-related bone disease].

    Science.gov (United States)

    Braun, C; Bacchetta, J; Reix, P

    2016-08-01

    With the increasing life expectancy of patients with cystic fibrosis (CF), prevalence of late complications such as CF-related bone disease (CFBD) has increased. It was initially described in 24% of the adult population with CF and has also been reported in the pediatric population. CFBD is multifactorial and progresses in different steps. Both decreased bone formation and increased bone resorption (in different amounts) are observed. CFBD is likely primitive (directly related to the CFTR defect itself), but is also worsened by acquired secondary factors such as lung infections, chronic inflammation, denutrition, vitamin deficiency, and decreased physical activity. CFBD may be clinically apparent (i.e., mainly vertebral and costal fractures), or clinically asymptomatic (therefore corresponding to abnormalities in bone density and architecture). CFBD management mainly aims to prevent the occurrence of fractures. Prevention and regular monitoring of bone disease as early as 8 years of age is of the utmost importance, as is the control of possible secondary deleterious CFBD factors. New radiological tools, such as high-resolution peripheral quantitative computed tomography, allow an accurate evaluation of cortical and trabecular bone micro-architecture in addition to compartmental density; as such, they will likely improve the assessment of the bone fracture threat in CF patients in the near future. PMID:27345551

  3. LRP4 in neuromuscular junction and bone development and diseases.

    Science.gov (United States)

    Shen, Chengyong; Xiong, Wen-Cheng; Mei, Lin

    2015-11-01

    Low-density lipoprotein receptor-related protein 4 (LRP4) is a member of the low-density lipoprotein receptor (LDLR) family. Recent studies have revealed multiple functions and complex signaling mechanisms of LRP4 in different organs and tissues. LPR4 mutation or malfunction has been implicated in neurological disorders including congenital myasthenic syndrome, myasthenia gravis, and diseases of bone or kidney. This article is part of a Special Issue entitled "Muscle Bone Interactions". PMID:26071838

  4. Chronic Kidney Disease Impairs Bone Defect Healing in Rats.

    Science.gov (United States)

    Liu, Weiqing; Kang, Ning; Seriwatanachai, Dutmanee; Dong, Yuliang; Zhou, Liyan; Lin, Yunfeng; Ye, Ling; Liang, Xing; Yuan, Quan

    2016-03-09

    Chronic kidney disease (CKD) has been regarded as a risk for bone health. The aim of this study was to evaluate the effect of CKD on bone defect repair in rats. Uremia was induced by subtotal renal ablation, and serum levels of BUN and PTH were significantly elevated four weeks after the second renal surgery. Calvarial defects of 5-mm diameter were created and implanted with or without deproteinized bovine bone mineral (DBBM). Micro-CT and histological analyses consistently revealed a decreased newly regenerated bone volume for CKD rats after 4 and 8 weeks. In addition, 1.4-mm-diameter cortical bone defects were established in the distal end of femora and filled with gelatin sponge. CKD rats exhibited significantly lower values of regenerated bone and bone mineral density (BMD) within the cortical gap after 2 and 4 weeks. Moreover, histomorphometric analysis showed an increase in both osteoblast number (N.Ob/B.Pm) and osteoclast number (N.Oc/B.Pm) in CKD groups due to hyperparathyroidism. Notably, collagen maturation was delayed in CKD rats as verified by Masson's Trichrome staining. These data indicate that declined renal function negatively affects bone regeneration in both calvarial and femoral defects.

  5. BONE IN OSTEOPETROSIS

    Directory of Open Access Journals (Sweden)

    Ramkumar

    2014-04-01

    Full Text Available Osteopetrosis, a generalized developmental bone disease due to genetic disturbances, characterized by failure of bone re sorption and continuous bone formation making the bone hard, dense and brittle. Bones of intramembranous ossification and enchondrial ossification are affected genetically and symmetrically. During the process of disease the excess bone formation obliterates the cranial foramina and presses the optic, auditory and facial nerves resulting in defective vision, impaired hearing and facial paralysis. The bone formation in osteopetrosis affects bone marrow function leading to severe anemia and deficient of blood cells. The bone devoid of blood supply due to compression of blood vessels by excess formation of bone are prone to osteomyelitic changes with suppuration and pathological fracture if exposed to infection. Though the condition is chronic progressive, it produces changes leading to fatal condition, it should be studied thoroughly by everyone and hence this article presents a classical case of osteopetrosis with detailed description and discussion for the benefit of readers

  6. 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.

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

    OpenAIRE

    Lohit Kumar; Bhubaneswar

    2016-01-01

    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,...

  8. Emerging strategies and therapies for treatment of Paget’s disease of bone

    OpenAIRE

    Brown, Jacques P.

    2011-01-01

    Laëtitia Michou, Jacques P BrownLaval University, Department of Medicine, CHUQ (CHUL) Research Centre and Division of Rheumatology, Quebec City, QC, CanadaAbstract: Paget’s disease of bone (PDB) is a progressive monostotic or polyostotic metabolic bone disease characterized by focal abnormal bone remodeling, with increased bone resorption and excessive, disorganized, new bone formation. PDB rarely occurs before middle age, and it is the second most frequent metabolic bone d...

  9. 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.

  10. Developmentally arrested structures preceding cerebellar tumors in von Hippel–Lindau disease

    OpenAIRE

    Shively, Sharon B; Falke, Eric A; Li, Jie; Tran, Maxine G B; Thompson, Eli R; Maxwell, Patrick H; Roessler, Erich; Oldfield, Edward H; Lonser, Russell R.; Vortmeyer, Alexander O

    2011-01-01

    There is increasing evidence that suggests that knockout of tumor-suppressor gene function causes developmental arrest and protraction of cellular differentiation. In the peripheral nervous system of patients with the tumor-suppressor gene disorder, von Hippel–Lindau disease, we have demonstrated developmentally arrested structural elements composed of hemangioblast progenitor cells. Some developmentally arrested structural elements progress to a frank tumor, hemangioblastoma. However, in von...

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

    International Nuclear Information System (INIS)

    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

  12. 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

  13. Cardiovascular implications of endodontic bone disease

    OpenAIRE

    Dessì, Cristina

    2012-01-01

    Cardiovascular diseases (CVD) have a complex etiology determined by risk factors, which are in turn associated to a strong genetic component and to environmental factors. In the biological background for the development of CVD, low-grade chronic inflammation plays a role as a pathogenetic determinant of atherosclerosis. Dental infections have been associated with CVD. Periodontal disease is a chronic infection of the supporting tissues of the tooth that can lead to teeth loss. In recent years...

  14. Paget’s Disease of Bone among Various Ethnic Groups

    OpenAIRE

    Mira Merashli; Ali Jawad

    2015-01-01

    Paget’s disease of bone (PDB) is a relatively benign disease common among many European populations, including those in the UK, Italy and Spain. However, it appears to be rare among Scandinavians and non-European immigrants living in Europe. The prevalence among Asian populations may be underestimated because a large number of reported cases were discovered incidentally. There is a need for surveys addressing the prevalence rate and consequences of PDB to be carried out in various parts of th...

  15. In vivo bone aluminum measurements in patients with renal disease

    Energy Technology Data Exchange (ETDEWEB)

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

    1986-01-01

    Contamination of the dialysis solution with trace amounts of aluminum and long-term use of aluminum-based phosphate binders have led to increased body burden of aluminum in patients with end-stage renal disease. A significant clinical problem associated with aluminum-overload is the early diagnosis of aluminum-induced dialysis dementia and osteomalacic osteodystrophy. There are few, if any, blood or urine indices that provide an early monitor of this bone disease, especially in the asymptomatic patient. Although a bone biopsy is usually the basis for the final clinical diagnosis, this procedure is not recommended for routine monitoring of patients. The present technique demonstrates the direct in vivo measurement of bone aluminum levels in patients with renal failure. The interference normally present from activation of bone phosphorus is eliminated by using a thermal/epithermal neutron beam. For the clinical management of the patients, the Al/Ca ratio for the hand may be more useful than an absolute measurement of the total body or skeletal aluminum burden. The relationship between the increased serum Al levels following disferrioxamine infusion and the direct in vivo measurement of bone aluminum using the Al/Ca ratio are currently under investigation. The neutron activation procedure presented in this pilot study is a promising new technique with an immediate clinical application. 5 refs., 3 figs., 1 tab.

  16. Neridronic acid for the treatment of bone metabolic diseases.

    Science.gov (United States)

    Gatti, Davide; Viapiana, Ombretta; Idolazzi, Luca; Fracassi, Elena; Adami, Silvano

    2009-10-01

    Neridronic acid (6-amino-1-idroxyesilidene-1,1-bisphosphonate) is a nitrogen-containing bisphosphonate licensed in Italy for the treatment of osteogenesis imperfecta and Paget's disease of bone. The pharmacodynamic profile is similar to that of other nitrogen-containing bisphosphonates and is characterized by its high affinity for bone tissue particularly at sites undergoing a process of remodeling. In growing children affected by osteogenesis imperfect, neridronic acid rapidly increases bone mineral density as measured by dual X-ray absortiometry and this is associated with a significant decrease in fracture cumulative number. Similar results have been obtained also in newborns ( 75% of bone turnover markers) in 95% of the patients. Neridronic acid treatment has been reported to be effective also in other skeletal diseases such as osteoporosis, algodystrophy, hypercalcemia of malignancy and bone metastasis. Neridronic acid has been developed only for parenteral use, and it is the only one used as intramuscular injection. This avoids all the limitations of oral bisphosphonates and may be offered for a home treatment with simple nursing assistance. PMID:19761412

  17. Bone histomorphometry after treatment with teriparatide (PTH 1-34) in a patient with adynamic bone disease subsequent to parathyroidectomy

    OpenAIRE

    Lehmann, Gabriele; Ott, Undine; Maiwald, Jörg; Wolf, Gunter

    2008-01-01

    A 33-year-old male patient suffered from adynamic bone disease because of parathyroidectomy due to tertiary hyperparathyroidism. Histomorphometric analysis of bone biopsies taken before and 8 months after treatment with teriparatide (human parathyroid hormone 1-34 of recombinant DNA origin) for 18 months is demonstrated. A considerable increase in mineralized bone volume and also stimulated bone remodelling were detected after treatment with teriparatide. Although teriparatide is currently on...

  18. Turner's syndrome presenting as metabolic bone disease

    OpenAIRE

    Sadishkumar Kamalanathan; Karthik Balachandran; Ramesh Ananthakrishnan; Abdoul Hamide

    2012-01-01

    Turner′s syndrome is a genetic disorder with a complete or partial absence of one X chromosome with characteristic phenotypic features. The prevalence of renal anomalies in turner syndrome is 30-40%. However, the renal function is usually normal. We report a case of Turner′s syndrome presenting with chronic kidney disease and renal osteodystrophy.

  19. Turner's syndrome presenting as metabolic bone disease.

    Science.gov (United States)

    Kamalanathan, Sadishkumar; Balachandran, Karthik; Ananthakrishnan, Ramesh; Hamide, Abdoul

    2012-07-01

    Turner's syndrome is a genetic disorder with a complete or partial absence of one X chromosome with characteristic phenotypic features. The prevalence of renal anomalies in turner syndrome is 30-40%. However, the renal function is usually normal. We report a case of Turner's syndrome presenting with chronic kidney disease and renal osteodystrophy. PMID:22837932

  20. Bone morphogenetic protein 15 in the pro-mature complex form enhances bovine oocyte developmental competence.

    Directory of Open Access Journals (Sweden)

    Jaqueline Sudiman

    Full Text Available Developmental competence of in vitro matured (IVM oocytes needs to be improved and this can potentially be achieved by adding recombinant bone morphogenetic protein 15 (BMP15 or growth differentiation factor (GDF9 to IVM. The aim of this study was to determine the effect of a purified pro-mature complex form of recombinant human BMP15 versus the commercially available bioactive forms of BMP15 and GDF9 (both isolated mature regions during IVM on bovine embryo development and metabolic activity. Bovine cumulus oocyte complexes (COCs were matured in vitro in control medium or treated with 100 ng/ml pro-mature BMP15, mature BMP15 or mature GDF9 +/- FSH. Metabolic measures of glucose uptake and lactate production from COCs and autofluorescence of NAD(PH, FAD and GSH were measured in oocytes after IVM. Following in vitro fertilisation and embryo culture, day 8 blastocysts were stained for cell numbers. COCs matured in medium +/- FSH containing pro-mature BMP15 displayed significantly improved blastocyst development (57.7±3.9%, 43.5±4.2% compared to controls (43.3±2.4%, 28.9±3.7% and to mature GDF9+FSH (36.1±3.0%. The mature form of BMP15 produced intermediate levels of blastocyst development; not significantly different to control or pro-mature BMP15 levels. Pro-mature BMP15 increased intra-oocyte NAD(PH, and reduced glutathione (GSH levels were increased by both forms of BMP15 in the absence of FSH. Exogenous BMP15 in its pro-mature form during IVM provides a functional source of oocyte-secreted factors to improve bovine blastocyst development. This form of BMP15 may prove useful for improving cattle and human artificial reproductive technologies.

  1. Kimura's disease involving a long bone

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Yang-Guk; Kang, Yong-Koo; Bahk, Won-Jong; Cho, Hyun-Min [The Catholic University of Korea, Department of Orthopedic Surgery, Seoul (Korea); Jee, Won-Hee [The Catholic University of Korea, Department of Radiology, Seoul St. Mary' s Hospital, Seoul (Korea); Jung, Chan-Kwon; Park, Gyeong-Sin; Lee, An-hi [The Catholic University of Korea, Department of Hospital Pathology, Seoul (Korea); Park, Jong-Won [The Catholic University of Korea, Department of Internal Medicine, College of Medicine, Seoul (Korea)

    2010-05-15

    Kimura's disease is a rare, benign lymphoproliferative disorder of unknown etiology. It occurs most often in Asian men, usually in the second or third decade of life. Most lesions occur in the head and neck followed by the axilla, groin, popliteal region, and arm. The lesions are commonly found in soft tissues. To the best of our knowledge, there has been only one case report of bone involvement in Kimura's disease presented on plain radiography. We report a case of Kimura's disease that involved the proximal meta-diaphysis of the humerus and adjacent soft tissue shown on radiography and MR imaging. (orig.)

  2. 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.

  3. Bone disease in newly diagnosed lupus nephritis patients.

    Directory of Open Access Journals (Sweden)

    Aline Lázara Resende

    Full Text Available Bone loss in Lupus Nephritis (LN patients is common and multifactorial. The aim of this study was to evaluate the bone status of newly diagnosed LN patients and their correlation with inflammatory factors involved in LN physiopathology.We studied 15 pre-menopausal patients with ≤2 months of diagnosed SLE and LN. Patients with prior kidney or bone disease were excluded. In addition to biochemical evaluation (including 25-hydroxyvitamin D3 [25(OHD] and Monocyte Chemotactic Protein (MCP1 dosage, we performed bone biopsies followed by osteoblast culture, histomorphometric and immunohistochemistry analysis.LN patients presented a mean age of 29.5±10 years, a proteinuria of 4.7±2.9 g/day and an estimated glomerular filtration rate (GFR of 37(31-87 ml/min/1,73 m2. They were on glucocorticoid therapy for 34±12 days. All patients presented vitamin D insufficiency (9.9±4.4 ng/ml, range 4-20. Urinary MCP1 correlated negatively with 25(OHD (r = -0.53, p = 0.003 and positively with serum deoxypyridinoline (r = 0.53, p = 0.004. Osteoblasts isolated from LN bone biopsies presented a significantly higher expression of MCP-1 when compared to controls (32.0.±9.1 vs. 22.9±5.3 mean fluorescence intensities, p = 0.01. LN patients presented a significantly reduced osteoid volume, osteoid thickness, osteoid surface, mineralization surface and bone formation rate, associated with an increased eroded surface and osteoclast surface. Patient's bone specimens demonstrated a reduced immunostaining for osteoprotegerin (0.61±0.82 vs. 1.08±0.50%, p = 0.003, and an increased expression of Receptor Activator of NF-κB ligand (RANKL (1.76±0.92 vs. 0.41±0.28%, p<0.001 when compared to controls.Newly diagnosed LN patients presented a significant disturbance in bone metabolism, characterized by an impaired bone formation and mineralization, associated with an increase in resorption parameters. Glucocorticoid use, vitamin D insufficiency and

  4. Bone marrow-derived stem cells and respiratory disease.

    Science.gov (United States)

    Jones, Carla P; Rankin, Sara M

    2011-07-01

    Adult bone marrow contains a number of discrete populations of progenitor cells, including endothelial, mesenchymal, and epithelial progenitor cells and fibrocytes. In the context of a range of diseases, endothelial progenitor cells have been reported to promote angiogenesis, mesenchymal stem cells are potent immunosuppressors but can also contribute directly to tissue regeneration, and fibrocytes have been shown to induce tissue fibrosis. This article provides an overview of the basic biology of these different subsets of progenitor cells, reporting their distinct phenotypes and functional activities. The differences in their secretomes are highlighted, and the relative role of cellular differentiation vs paracrine effects of progenitor cells is considered. The article reviews the literature examining the contribution of progenitor cells to the pathogenesis of respiratory disease, and discusses recent studies using bone marrow progenitor cells as stem cell therapies in the context of pulmonary hypertension, COPD, and asthma. PMID:21729891

  5. 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.

  6. MANAGEMENT OF DISEASES OF LONG BONES WITH KUNTSCHER NAILS

    Directory of Open Access Journals (Sweden)

    Ravikant

    2015-07-01

    Full Text Available BACKGROUND: AIMS: SETTINGS AND DESIGN : The aim of this study was, to devise economical, easy, simple, quick method of fixation of diseased long bones, so that pathological fracture could be prevented and to provide rigid fixation, in those cases which have already developed pathological fractu re, and to achieve arthrodesis. Ten, cases of long bone diseases were managed with the help of K nails, in the Department of Orthopaedics, in CIMS, between, December 2002 to January 2005, and cases were followed for about ten years for recurrence, relapse and complications or deterioration of the underlying disease process. METHODS AND MATERIAL : K nailing was done by standard procedure of nailing, applying A O Principles of internal fixation with due emphasis on exact length and thickness of the nail, so as to span the whole length of the bone and should occupy the entire medullary cavity at isthmus, and should achieve three point fixation of the nail in the bone, in order to provide complete rotational stability of the bone and fracture. RESULT : All the cases healed within three to six months of operation. The results were similar to internal fixation done with interlocking nailing or plating, in all the parameters including, knee and hip range of movement, both active and passive, thigh and leg girth, muscle wasting, extensor lag and time of union, but the operative time was half that of interlocking. CONCLUSION : By this study we came to the conclusion that, K nailing is still indispensable implant, and should not be discarded completely. It shou ld be an important inclusion in the inventory of implants in Orthopaedic surgeon basket. Especially in management diseases of long bones, a surgeon has to take care of so many surgical steps that the surgeon is left with very little, anaesthesia and surgic al time to put in complicated lengthy processed implant. In such a situation a K nail serves the purpose. Therefore it should be the most preferred

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

    International Nuclear Information System (INIS)

    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)

  8. Consensus on Surgical Management of Myeloma Bone Disease.

    Science.gov (United States)

    2016-08-01

    Myeloma bone disease (MBD), the skeletal lesions caused by multiple myeloma, is also known as skeletal related events and includes bone pain, osteoporosis, pathological fractures, osteolytic bone lesions, spinal instability, spinal cord and nerve root compression and extramedullary plasmacytoma. It is now generally accepted that patients with these complications usually require surgical management and that such treatment is safe and effective. The aims of surgical interventions are to alleviate pain, improve quality of life, treat potential or existing pathological fractures, decompress the spinal cord and nerve roots, and reestablish bone continuity. Thus far, there have not been uniform standards for surgical treatment of MBD. The Surgeon's Committee of the Chinese Myeloma Working Group has therefore achieved a consensus with the aim of providing guidance for clinicians and benefitting patients with MBD. This consensus focuses on the treatment of MBD, including its clinical definition and characteristics, diagnosis and surgical management. This expert consensus document was compiled after discussion and revision by experts from several relevant institutions in China. However, it is only an interim guide that cannot be enforced legally. It will be updated with development of new techniques of treatment. PMID:27627707

  9. 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.

  10. An Unusual Developmental Profile of Salla Disease in a Patient with the SallaFIN Mutation

    OpenAIRE

    Paavola, Liisa E.; Remes, Anne M.; Sonninen, Pirkko H.; Vesa V. Kiviniemi; Korhonen, Tapio T.; Kari Majamaa

    2012-01-01

    Salla disease (SD) is a disorder caused by defective storage of free sialic acid and results from mutations in the SLC17A5 gene. Early developmental delay of motor functions, and later cognitive skills, is typical. We describe a developmental profile of an unusual homozygous patient, who harboured the SallaFIN (p.R39C) mutation gene. The study involved neurological examination, neuropsychological investigation, and brain imaging. The neurocognitive findings were atypical in comparison with ot...

  11. 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…

  12. How rare bone diseases have informed our knowledge of complex diseases.

    Science.gov (United States)

    Johnson, Mark L

    2016-01-01

    Rare bone diseases, generally defined as monogenic traits with either autosomal recessive or dominant patterns of inheritance, have provided a rich database of genes and associated pathways over the past 2-3 decades. The molecular genetic dissection of these bone diseases has yielded some major surprises in terms of the causal genes and/or involved pathways. The discovery of genes/pathways involved in diseases such as osteopetrosis, osteosclerosis, osteogenesis imperfecta and many other rare bone diseases have all accelerated our understanding of complex traits. Importantly these discoveries have provided either direct validation for a specific gene embedded in a group of genes within an interval identified through a complex trait genome-wide association study (GWAS) or based upon the pathway associated with a monogenic trait gene, provided a means to prioritize a large number of genes for functional validation studies. In some instances GWAS studies have yielded candidate genes that fall within linkage intervals associated with monogenic traits and resulted in the identification of causal mutations in those rare diseases. Driving all of this discovery is a complement of technologies such as genome sequencing, bioinformatics and advanced statistical analysis methods that have accelerated genetic dissection and greatly reduced the cost. Thus, rare bone disorders in partnership with GWAS have brought us to the brink of a new era of personalized genomic medicine in which the prevention and management of complex diseases will be driven by the molecular understanding of each individuals contributing genetic risks for disease. PMID:27688878

  13. Osteoclast formation from peripheral blood of patients with bone-lytic diseases

    NARCIS (Netherlands)

    T.J. de Vries; V. Everts

    2009-01-01

    Recent literature indicates that osteoclast formation in vitro from peripheral blood of patients with diseases associated with bone loss such as rheumatoid arthritis, osteoporosis, periodontitis and bone metastatic cancer may occur spontaneously being independent of addition of osteoclast formation

  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)

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

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Seung Hun; Choi, Yun Young; Cho, Suk Shin [College of Medicine, Hanyang Univ., Seoul (Korea, Republic of)

    2001-12-01

    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.

  16. 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. PMID:20577135

  17. The effect of maternal nutrition on the developmental origins of respiratory disease

    OpenAIRE

    Davis, Shelley A.

    2011-01-01

    Environmental challenges during early life have been shown to result in greater risk of chronic diseases such as diabetes and coronary disease in later life. Factors such as unbalanced nutrition before birth result in metabolic and structural adaptations that lead to persistent modifications to offspring phenotype. There is evidence that respiratory disease is influenced by developmental environment. Reduced fetal growth is associated with impaired lung development, increasing risk of develop...

  18. Circulating microRNAs as novel biomarkers for bone diseases - Complex signatures for multifactorial diseases?

    Science.gov (United States)

    Hackl, Matthias; Heilmeier, Ursula; Weilner, Sylvia; Grillari, Johannes

    2016-09-01

    Biomarkers are essential tools in clinical research and practice. Useful biomarkers must combine good measurability, validated association with biological processes or outcomes, and should support clinical decision making if used in clinical practice. Several types of validated biomarkers have been reported in the context of bone diseases. However, because these biomarkers face certain limitations there is an interest in the identification of novel biomarkers for bone diseases, specifically in those that are tightly linked to the disease pathology leading to increased fracture-risk. MicroRNAs (miRNAs) are the most abundant RNA species to be found in cell-free blood. Encapsulated within microvesicles or bound to proteins, circulating miRNAs are remarkably stable analytes that can be measured using gold-standard technologies such as quantitative polymerase-chain-reaction (qPCR). Nevertheless, the analysis of circulating miRNAs faces several pre-analytical as well as analytical challenges. From a biological view, there is accumulating evidence that miRNAs play essential roles in the regulation of various biological processes including bone homeostasis. Moreover, specific changes in miRNA transcription levels or miRNA secretory levels have been linked to the development and progression of certain bone diseases. Only recently, results from circulating miRNAs analysis in patients with osteopenia, osteoporosis and fragility fractures have been reported. By comparing these findings to studies on circulating miRNAs in cellular senescence and aging or muscle physiology and sarcopenia, several overlaps were observed. This suggests that signatures observed during osteoporosis might not be specific to the pathophysiology in bone, but rather integrate information from several tissue types. Despite these promising first data, more work remains to be done until circulating miRNAs can serve as established and robust diagnostic tools for bone diseases in clinical research, clinical

  19. Graves-Basedow disease after allogeneic bone marrow transplantation

    International Nuclear Information System (INIS)

    One severe aplastic anaemia case who presented autoimmune thyroid disease after allogeneic bone marrow transplantation (alloBMT) is described. A 19 year old Polish girldeveloped Graves' hyperthyroidisms 19 months after allogeneic BMT for severe aplastic anaemia (SAA) donated from her brother. Her serum was positive for thyroid stimulating antibody (TSAb) and anti-thyroid peroxidase autoantibodies (aTPO) while her brother remained euthyroid, seronegative for TSAb, and showed no clinical signs of thyroid pathology. The genetic studies of lymphocytes FISH (fluorescence in situ hybridization) and analysis of STR (short tandem repeated) fragments suggested, that lymphocytes responsible for hyperthyroidisms were of donor origin. (author)

  20. MRI for response assessment in metastatic bone disease

    Energy Technology Data Exchange (ETDEWEB)

    Lecouvet, F.E.; Larbi, A.; Pasoglou, V.; Omoumi, P.; Michoux, N.; Malghem, J.; Berg, B.C. vande [UCL, Universite Catholique de Louvain, Department of Radiology, Cliniques Universitaires Saint-Luc, IREC, Institut de Recherche Clinique, Centre du Cancer, Brussels (Belgium); Tombal, B. [UCL, Universite Catholique de Louvain, Service d' Urologie, Cliniques Universitaires Saint-Luc, IREC, Institut de Recherche Clinique, Centre du Cancer, Brussels (Belgium); Lhommel, R. [UCL, Universite Catholique de Louvain, Department of Nuclear Medicine, Cliniques Universitaires Saint-Luc, IREC, Institut de Recherche Clinique, Centre du Cancer, Brussels (Belgium)

    2013-07-15

    Beyond lesion detection and characterisation, and disease staging, the quantification of the tumour load and assessment of response to treatment are daily expectations in oncology. Bone lesions have been considered ''non-measurable'' for years as opposed to lesions involving soft tissues and ''solid'' organs like the lungs or liver, for which response evaluation criteria are used in every day practice. This is due to the lack of sensitivity, specificity and measurement capabilities of imaging techniques available for bone assessment, i.e. skeletal scintigraphy (SS), radiographs and computed tomography (CT). This paper reviews the possibilities and limitations of these techniques and highlights the possibilities of positron emission tomography (PET), but mainly concentrates on magnetic resonance imaging (MRI). Practical morphological and quantitative approaches are proposed to evaluate the treatment response of bone marrow lesions using ''anatomical'' MRI. Recent developments of MRI, i.e. dynamic contrast-enhanced (DCE) imaging and diffusion-weighted imaging (DWI), are also covered. (orig.)

  1. Role of Bone Biopsy in Stages 3 to 4 Chronic Kidney Disease

    OpenAIRE

    Gal-Moscovici, Anca; Sprague, Stuart M.

    2008-01-01

    Secondary hyperparathyroidism develops relatively early in chronic kidney disease as a consequence of impaired phosphate, calcium, and vitamin D homeostasis. The disease state in chronic kidney disease, which includes the histologic features of bone disease, defined as renal osteodystrophy, and the hormonal and biochemical disturbances, have recently been redefined as a disease syndrome and is referred to as “chronic kidney disease–mineral and bone disorder.” As chronic kidney disease progres...

  2. The Application of Bone Marrow Transplantation to the Treatment of Genetic Diseases

    Science.gov (United States)

    Parkman, Robertson

    1986-06-01

    Genetic diseases can be treated by transplantation of either normal allogeneic bone marrow or, potentially, autologous bone marrow into which the normal gene has been inserted in vitro (gene therapy). Histocompatible allogeneic bone marrow transplantation is used for the treatment of genetic diseases whose clinical expression is restricted to lymphoid or hematopoietic cells. The therapeutic role of bone marrow transplantation in the treatment of generalized genetic diseases, especially those affecting the central nervous system, is under investigation. The response of a generalized genetic disease to allogeneic bone marrow transplantation may be predicted by experiments in vitro. Gene therapy can be used only when the gene responsible for the disease has been characterized. Success of gene therapy for a specific genetic disease may be predicted by its clinical response to allogeneic bone marrow transplantation.

  3. Bone: a new endocrine organ at the heart of chronic kidney disease and mineral and bone disorders.

    Science.gov (United States)

    Vervloet, Marc G; Massy, Ziad A; Brandenburg, Vincent M; Mazzaferro, Sandro; Cozzolino, Mario; Ureña-Torres, Pablo; Bover, Jordi; Goldsmith, David

    2014-05-01

    Recent reports of several bone-derived substances, some of which have hormonal properties, have shed new light on the bone-cardiovascular axis. Deranged concentrations of humoral factors are not only epidemiologically connected to cardiovascular morbidity and mortality, but can also be causally implicated, especially in chronic kidney disease. FGF23 rises exponentially with advancing chronic kidney disease, seems to reach maladaptive concentrations, and then induces left ventricular hypertrophy, and is possibly implicated in the process of vessel calcification. Sclerostin and DKK1, both secreted mainly by osteocytes, are important Wnt inhibitors and as such can interfere with systems for biological signalling that operate in the vessel wall. Osteocalcin, produced by osteoblasts or released from mineralised bone, interferes with insulin concentrations and sensitivity, and its metabolism is disturbed in kidney disease. These bone-derived humoral factors might place the bone at the centre of cardiovascular disease associated with chronic kidney disease. Most importantly, factors that dictate the regulation of these substances in bone and subsequent secretion into the circulation have not been researched, and could provide entirely new avenues for therapeutic intervention.

  4. 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

  5. A post-developmental genetic screen for zebrafish models of inherited liver disease.

    Science.gov (United States)

    Kim, Seok-Hyung; Wu, Shu-Yu; Baek, Jeong-In; Choi, Soo Young; Su, Yanhui; Flynn, Charles R; Gamse, Joshua T; Ess, Kevin C; Hardiman, Gary; Lipschutz, Joshua H; Abumrad, Naji N; Rockey, Don C

    2015-01-01

    Nonalcoholic fatty liver disease (NAFLD) is one of the most common causes of chronic liver disease such as simple steatosis, nonalcoholic steatohepatitis (NASH), cirrhosis and fibrosis. However, the molecular pathogenesis and genetic variations causing NAFLD are poorly understood. The high prevalence and incidence of NAFLD suggests that genetic variations on a large number of genes might be involved in NAFLD. To identify genetic variants causing inherited liver disease, we used zebrafish as a model system for a large-scale mutant screen, and adopted a whole genome sequencing approach for rapid identification of mutated genes found in our screen. Here, we report on a forward genetic screen of ENU mutagenized zebrafish. From 250 F2 lines of ENU mutagenized zebrafish during post-developmental stages (5 to 8 days post fertilization), we identified 19 unique mutant zebrafish lines displaying visual evidence of hepatomegaly and/or steatosis with no developmental defects. Histological analysis of mutants revealed several specific phenotypes, including common steatosis, micro/macrovesicular steatosis, hepatomegaly, ballooning, and acute hepatocellular necrosis. This work has identified multiple post-developmental mutants and establishes zebrafish as a novel animal model for post-developmental inherited liver disease.

  6. A post-developmental genetic screen for zebrafish models of inherited liver disease.

    Directory of Open Access Journals (Sweden)

    Seok-Hyung Kim

    Full Text Available Nonalcoholic fatty liver disease (NAFLD is one of the most common causes of chronic liver disease such as simple steatosis, nonalcoholic steatohepatitis (NASH, cirrhosis and fibrosis. However, the molecular pathogenesis and genetic variations causing NAFLD are poorly understood. The high prevalence and incidence of NAFLD suggests that genetic variations on a large number of genes might be involved in NAFLD. To identify genetic variants causing inherited liver disease, we used zebrafish as a model system for a large-scale mutant screen, and adopted a whole genome sequencing approach for rapid identification of mutated genes found in our screen. Here, we report on a forward genetic screen of ENU mutagenized zebrafish. From 250 F2 lines of ENU mutagenized zebrafish during post-developmental stages (5 to 8 days post fertilization, we identified 19 unique mutant zebrafish lines displaying visual evidence of hepatomegaly and/or steatosis with no developmental defects. Histological analysis of mutants revealed several specific phenotypes, including common steatosis, micro/macrovesicular steatosis, hepatomegaly, ballooning, and acute hepatocellular necrosis. This work has identified multiple post-developmental mutants and establishes zebrafish as a novel animal model for post-developmental inherited liver disease.

  7. Paget's disease diagnosed on bone scintigraphy: Case report and literature review

    International Nuclear Information System (INIS)

    Paget's disease of bone is a chronic bone remodeling disorder. Although most patients are asymptomatic, a variety of symptoms and complications may develop directly from bone involvement or secondarily due to compression by the expanded bone. It is usually diagnosed from radiological and biochemical abnormalities or in advanced cases it becomes clinically evident due to the expanded bone. We report a case of Paget's disease which was detected incidentally during evaluation of nephrolithiasis and polyarthritis but had normal radiographs and normal biochemical markers

  8. Quantitative magnetic resonance imaging in autologous bone marrow transplantation for Hodgkin's disease.

    OpenAIRE

    Smith, S. R.; Williams, C E; Edwards, R H; Davies, J M

    1989-01-01

    Fifteen consecutive patients with refractory or relapsed Hodgkin's disease (HD) referred for autologous bone marrow transplantation (ABMT) underwent quantitative magnetic resonance (MR) studies of the lumbar vertebral bone marrow. Markedly elevated lumbar vertebral marrow T1 values suggestive of bone marrow involvement with HD were seen in four patients, two of whom had no evidence of HD on bilateral iliac crest bone marrow biopsy. Serial studies showed normalisation of T1 values in the post-...

  9. Physiology and molecular characterization of metabolism related mouse models for bone disease

    OpenAIRE

    Chi, Shen

    2015-01-01

    Bone disorders are commonly associated with various metabolic diseases. Two ENU- induced mutant mouse lines were analyzed to explore the relationship between bone and metabolic phenotypes. SATB2 was proven to regulate bone development. In addition, a previously unknown role of the gene in energy metabolism was uncovered. Only a minor influence on bone homeostasis and energy metabolism could be attributed to the T720A mutation of DLL1.

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

    Institute of Scientific and Technical Information of China (English)

    Pal; Miheller; Katalin; Lrinczy; Peter; Laszlo; Lakatos

    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 ...

  11. 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

  12. Treatment of Niemann-Pick disease type B by allogeneic bone marrow transplantation.

    OpenAIRE

    Vellodi, A.; Hobbs, J. R.; O'Donnell, N M; Coulter, B S; Hugh-Jones, K.

    1987-01-01

    Allogenic bone marrow transplantation was carried out on a 3 year old girl with Niemann-Pick disease type B. Successful engraftment was achieved, and nine months after the procedure there was definite clearing of the sphingomyelin from the liver and pronounced clearing from the bone marrow. Any patient with Niemann-Pick disease type B complicated by early or severe hepatic impairment should be considered for bone marrow transplantation.

  13. SERUM YKL-40 IS ASSOCIATED WITH BONE DISEASE IN MULTIPLE MYELOMA

    DEFF Research Database (Denmark)

    Mylin, Anne Kjærsgaard; Abildgaard, Niels; Johansen, Julia S.;

    2007-01-01

     Introduction. The secreted glycoprotein YKL-40 (CHI3L1, HC gp-39) is a potential player in the tumor-host interactions affecting several aspects of multiple myeloma (MM) including bone destruction. Previous studies support a role for YKL-40 in remodelling of the extracellular matrix...... to progression of myeloma-related bone disease. A potential role for YKL-40 in the bone disease of MM must be considered....

  14. The role of biochemical of bone turnover markers in osteoporosis and metabolic bone disease: a consensus paper of the Belgian Bone Club.

    Science.gov (United States)

    Cavalier, E; Bergmann, P; Bruyère, O; Delanaye, P; Durnez, A; Devogelaer, J-P; Ferrari, S L; Gielen, E; Goemaere, S; Kaufman, J-M; Toukap, A Nzeusseu; Reginster, J-Y; Rousseau, A-F; Rozenberg, S; Scheen, A J; Body, J-J

    2016-07-01

    The exact role of biochemical markers of bone turnover in the management of metabolic bone diseases remains a topic of controversy. In this consensus paper, the Belgian Bone Club aimed to provide a state of the art on the use of these biomarkers in different clinical or physiological situations like in postmenopausal women, osteoporosis in men, in elderly patients, in patients suffering from bone metastasis, in patients with chronic renal failure, in pregnant or lactating women, in intensive care patients, and in diabetics. We also gave our considerations on the analytical issues linked to the use of these biomarkers, on potential new emerging biomarkers, and on the use of bone turnover biomarkers in the follow-up of patients treated with new drugs for osteoporosis. PMID:27026330

  15. MicroRNAs in the control of metastatic bone disease

    OpenAIRE

    Browne, Gillian; Taipaleenmäki, Hanna; Stein, Gary S.; Stein, Janet L.; Lian, Jane B.

    2014-01-01

    Bone metastasis is a common and devastating complication of late stage breast and prostate cancer. Complex interactions between tumor cells, bone cells and a milieu of components in their microenvironment contribute to the osteolytic, osteoblastic or mixed lesions present in patients with metastasis to bone. In the last decade, miRNAs have emerged as key players in cancer progression yet the importance of miRNAs in regulating cancer metastasis to bone is now being appreciated. Here, we emphas...

  16. Hirschsprung disease: a developmental disorder of the enteric nervous system.

    Science.gov (United States)

    McKeown, Sonja J; Stamp, Lincon; Hao, Marlene M; Young, Heather M

    2013-01-01

    Hirschsprung disease (HSCR), which is also called congenital megacolon or intestinal aganglionosis, is characterized by an absence of enteric (intrinsic) neurons from variable lengths of the most distal bowel. Because enteric neurons are essential for propulsive intestinal motility, infants with HSCR suffer from severe constipation and have a distended abdomen. Currently the only treatment is surgical removal of the affected bowel. HSCR has an incidence of around 1:5,000 live births, with a 4:1 male:female gender bias. Most enteric neurons arise from neural crest cells that emigrate from the caudal hindbrain and then migrate caudally along the entire gut. The absence of enteric neurons from variable lengths of the bowel in HSCR results from a failure of neural crest-derived cells to colonize the affected gut regions. HSCR is therefore regarded as a neurocristopathy. HSCR is a multigenic disorder and has become a paradigm for understanding complex factorial disorders. The major HSCR susceptibility gene is RET. The penetrance of several mutations in HSCR susceptibility genes is sex-dependent. HSCR can occur as an isolated disorder or as part of syndromes; for example, Type IV Waardenburg syndrome is characterized by deafness and pigmentation defects as well as intestinal aganglionosis. Studies using animal models have shown that HSCR genes regulate multiple processes including survival, proliferation, differentiation, and migration. Research into HSCR and the development of enteric neurons is an excellent example of the cross fertilization of ideas that can occur between human molecular geneticists and researchers using animal models. WIREs Dev Biol 2013, 2:113-129. doi: 10.1002/wdev.57 For further resources related to this article, please visit the WIREs website. PMID:23799632

  17. Molecular Characterization of Three Canine Models of Human Rare Bone Diseases: Caffey, van den Ende-Gupta, and Raine Syndromes.

    Science.gov (United States)

    Hytönen, Marjo K; Arumilli, Meharji; Lappalainen, Anu K; Owczarek-Lipska, Marta; Jagannathan, Vidhya; Hundi, Sruthi; Salmela, Elina; Venta, Patrick; Sarkiala, Eva; Jokinen, Tarja; Gorgas, Daniela; Kere, Juha; Nieminen, Pekka; Drögemüller, Cord; Lohi, Hannes

    2016-05-01

    One to two percent of all children are born with a developmental disorder requiring pediatric hospital admissions. For many such syndromes, the molecular pathogenesis remains poorly characterized. Parallel developmental disorders in other species could provide complementary models for human rare diseases by uncovering new candidate genes, improving the understanding of the molecular mechanisms and opening possibilities for therapeutic trials. We performed various experiments, e.g. combined genome-wide association and next generation sequencing, to investigate the clinico-pathological features and genetic causes of three developmental syndromes in dogs, including craniomandibular osteopathy (CMO), a previously undescribed skeletal syndrome, and dental hypomineralization, for which we identified pathogenic variants in the canine SLC37A2 (truncating splicing enhancer variant), SCARF2 (truncating 2-bp deletion) and FAM20C (missense variant) genes, respectively. CMO is a clinical equivalent to an infantile cortical hyperostosis (Caffey disease), for which SLC37A2 is a new candidate gene. SLC37A2 is a poorly characterized member of a glucose-phosphate transporter family without previous disease associations. It is expressed in many tissues, including cells of the macrophage lineage, e.g. osteoclasts, and suggests a disease mechanism, in which an impaired glucose homeostasis in osteoclasts compromises their function in the developing bone, leading to hyperostosis. Mutations in SCARF2 and FAM20C have been associated with the human van den Ende-Gupta and Raine syndromes that include numerous features similar to the affected dogs. Given the growing interest in the molecular characterization and treatment of human rare diseases, our study presents three novel physiologically relevant models for further research and therapy approaches, while providing the molecular identity for the canine conditions. PMID:27187611

  18. Molecular Characterization of Three Canine Models of Human Rare Bone Diseases: Caffey, van den Ende-Gupta, and Raine Syndromes.

    Directory of Open Access Journals (Sweden)

    Marjo K Hytönen

    2016-05-01

    Full Text Available One to two percent of all children are born with a developmental disorder requiring pediatric hospital admissions. For many such syndromes, the molecular pathogenesis remains poorly characterized. Parallel developmental disorders in other species could provide complementary models for human rare diseases by uncovering new candidate genes, improving the understanding of the molecular mechanisms and opening possibilities for therapeutic trials. We performed various experiments, e.g. combined genome-wide association and next generation sequencing, to investigate the clinico-pathological features and genetic causes of three developmental syndromes in dogs, including craniomandibular osteopathy (CMO, a previously undescribed skeletal syndrome, and dental hypomineralization, for which we identified pathogenic variants in the canine SLC37A2 (truncating splicing enhancer variant, SCARF2 (truncating 2-bp deletion and FAM20C (missense variant genes, respectively. CMO is a clinical equivalent to an infantile cortical hyperostosis (Caffey disease, for which SLC37A2 is a new candidate gene. SLC37A2 is a poorly characterized member of a glucose-phosphate transporter family without previous disease associations. It is expressed in many tissues, including cells of the macrophage lineage, e.g. osteoclasts, and suggests a disease mechanism, in which an impaired glucose homeostasis in osteoclasts compromises their function in the developing bone, leading to hyperostosis. Mutations in SCARF2 and FAM20C have been associated with the human van den Ende-Gupta and Raine syndromes that include numerous features similar to the affected dogs. Given the growing interest in the molecular characterization and treatment of human rare diseases, our study presents three novel physiologically relevant models for further research and therapy approaches, while providing the molecular identity for the canine conditions.

  19. 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.

  20. Lumbar gibbus in storage diseases and bone dysplasias

    International Nuclear Information System (INIS)

    Objective. The objective of this study was to review the problem of lumbar gibbus in children with storage diseases and bone dysplasias utilizing plain films and MR imaging. Materials and methods. Clinical histories and radiographic images in five patients with storage diseases [four mucopolysaccharidosis (MPS) and one mucolipidosis[ and two with achondroplasia were reviewed. The International Skeletal Dysplasia Registry (Los Angeles, Calif.), surveyed for all patients with lumbar gibbus and skeletal dysplasias, provided 12 additional cases. Results. All patients had localized gibbus of the upper lumbar spine, characterized by anterior wedging and posterior displacement of the vertebrae at the apex of the curve, producing a beaked appearance. The curve, exaggerated in the sitting or standing position, was most severe in the two patients with MPS-IV (one of whom died). Both developed severe neurologic signs and symptoms requiring surgical intervention. In four patients, MR images demonstrated the apex of the curve to be at or below the conus. Two patients demonstrated anterior herniation of the intervertebral discs at the apex of the curve, though the signal intensity of the intervertebral discs was normal. Conclusion. Lumbar gibbus has important neurologic and orthopedic implications, and is most severe in patients with MPS. The etiology of the gibbus with vertebral beaking is multifactorial and includes poor truncal muscle tone, weight-bearing forces, growth disturbance and anterior disc herniation. The curve is generally at or below the conus. Neurologic complications are unusual, although orthopedic problems can arise. Due to their longer survival, patients with achondroplasia or Morquio's disease are more vulnerable to eventual gibbus-related musculoskeletal complications. (orig.). With 6 figs., 2 tabs

  1. Lumbar gibbus in storage diseases and bone dysplasias

    Energy Technology Data Exchange (ETDEWEB)

    Levin, T.L. [Department of Radiology, Division of Pediatric Radiology, Columbia-Presbyterian Medical Center, Babies and Children`s Hospital of New York, NY (United States); Berdon, W.E. [Department of Radiology, Division of Pediatric Radiology, Columbia-Presbyterian Medical Center, Babies and Children`s Hospital of New York, NY (United States); Lachman, R.S. [International Skeletal Dysplasia Registry, Los Angeles, CA (United States); Anyane-Yeboa, K. [Department of Pediatrics, Columbia-Presbyterian Medical Center, Babies and Children`s Hospital of New York, NY (United States); Ruzal-Shapiro, C. [Department of Radiology, Division of Pediatric Radiology, Columbia-Presbyterian Medical Center, Babies and Children`s Hospital of New York, NY (United States); Roye, D.P. Jr. [Department of Orthopedic Surgery, Columbia-Presbyterian Medical Center, Babies and Children`s Hospital of New York, NY (United States)

    1997-04-01

    Objective. The objective of this study was to review the problem of lumbar gibbus in children with storage diseases and bone dysplasias utilizing plain films and MR imaging. Materials and methods. Clinical histories and radiographic images in five patients with storage diseases [four mucopolysaccharidosis (MPS) and one mucolipidosis] and two with achondroplasia were reviewed. The International Skeletal Dysplasia Registry (Los Angeles, Calif.), surveyed for all patients with lumbar gibbus and skeletal dysplasias, provided 12 additional cases. Results. All patients had localized gibbus of the upper lumbar spine, characterized by anterior wedging and posterior displacement of the vertebrae at the apex of the curve, producing a beaked appearance. The curve, exaggerated in the sitting or standing position, was most severe in the two patients with MPS-IV (one of whom died). Both developed severe neurologic signs and symptoms requiring surgical intervention. In four patients, MR images demonstrated the apex of the curve to be at or below the conus. Two patients demonstrated anterior herniation of the intervertebral discs at the apex of the curve, though the signal intensity of the intervertebral discs was normal. Conclusion. Lumbar gibbus has important neurologic and orthopedic implications, and is most severe in patients with MPS. The etiology of the gibbus with vertebral beaking is multifactorial and includes poor truncal muscle tone, weight-bearing forces, growth disturbance and anterior disc herniation. The curve is generally at or below the conus. Neurologic complications are unusual, although orthopedic problems can arise. Due to their longer survival, patients with achondroplasia or Morquio`s disease are more vulnerable to eventual gibbus-related musculoskeletal complications. (orig.). With 6 figs., 2 tabs.

  2. Bone Grafts

    Science.gov (United States)

    ... repair and rebuild diseased bones in your hips, knees, spine, and sometimes other bones and joints. Grafts can also repair bone loss caused by some types of fractures or cancers. Once your body accepts the bone ...

  3. Autologous bone marrow cell therapy for peripheral arterial disease

    Directory of Open Access Journals (Sweden)

    Botti C

    2012-09-01

    Full Text Available C Botti, C Maione, A Coppola, V Sica, G CobellisDepartment of General Pathology, Second University of Naples, Naples, ItalyAbstract: Inadequate blood supply to tissues caused by obstruction of arterioles and/or capillaries results in ischemic injuries – these injuries can range from mild (eg, leg ischemia to severe conditions (eg, myocardial infarction, stroke. Surgical and/or endovascular procedures provide cutting-edge treatment for patients with vascular disorders; however, a high percentage of patients are currently not treatable, owing to high operative risk or unfavorable vascular involvement. Therapeutic angiogenesis has recently emerged as a promising new therapy, promoting the formation of new blood vessels by the introduction of bone marrow–derived stem and progenitor cells. These cells participate in the development of new blood vessels, the enlargement of existing blood vessels, and sprouting new capillaries from existing blood vessels, providing evidence of the therapeutic utility of these cells in ischemic tissues. In this review, the authors describe peripheral arterial disease, an ischemic condition affecting the lower extremities, summarizing different aspects of vascular regeneration and discussing which and how stem cells restore the blood flow. The authors also present an overview of encouraging results from early-phase clinical trials using stem cells to treat peripheral arterial disease. The authors believe that additional research initiatives should be undertaken to better identify the nature of stem cells and that an intensive cooperation between laboratory and clinical investigators is needed to optimize the design of cell therapy trials and to maximize their scientific rigor. Only this will allow the results of these investigations to develop best clinical practices. Additionally, although a number of stem cell therapies exist, many treatments are performed outside international and national regulations and many

  4. Bone mineral disorder in chronic kidney disease: Klotho and FGF23; cardiovascular implications.

    Science.gov (United States)

    Salanova Villanueva, Laura; Sánchez González, Carmen; Sánchez Tomero, José Antonio; Aguilera, Abelardo; Ortega Junco, Esther

    2016-01-01

    Cardiovascular factors are one of the main causes of morbidity and mortality in patients with chronic kidney disease. Bone mineral metabolism disorders and inflammation are pathological conditions that involve increased cardiovascular risk in chronic kidney disease. The cardiovascular risk involvement of bone mineral metabolism classical biochemical parameters such as phosphorus, calcium, vitamin D and PTH is well known. The newest markers, FGF23 and klotho, could also be implicated in cardiovascular disease.

  5. Rheumatic manifestations of primary and metastatic bone tumors and paraneoplastic bone disease.

    Science.gov (United States)

    Waimann, Christian A; Lu, Huifang; Suarez Almazor, Maria E

    2011-11-01

    Bone tumors can show a wide range of nonspecific rheumatic manifestations. The presence of unexplained or atypical chronic bone pain, an enlarging bone mass, neurovascular compression syndromes, or pathologic fractures should alert us to the possibility of a bone tumor causing these symptoms. These patients must undergo a complete physical examination; adequate imaging; and, if needed, a biopsy to confirm their diagnosis and offer them an opportune treatment. In addition, bone tumors and other malignancies can present remote clinical manifestations and unusual laboratory findings (eg, HOA, hypophosphatemia, hyperphosphaturia, and hypercalcemia) that may be the first and early manifestation of an occult cancer. These findings should motivate a cancer screening according to age, sex, and personal history. Cancer therapies also have a big impact on bone health, increasing the risk of osteoporosis, osteomalacia, and/or osteonecrosis. Rheumatologists should be aware of possible long-term adverse events of cancer treatment to avoid future complications.

  6. Developmental origins of adult diseases and neurotoxicity: Epidemiological and experimental studies

    DEFF Research Database (Denmark)

    Fox, Donald A; Grandjean, Philippe; de Groot, Didima;

    2012-01-01

    and the development of metabolic-related diseases and neurotoxicity later in life. The four speakers at this symposium presented their research results on different neurotoxic chemicals relating to the developmental origins of health and adult disease (DOHaD). Philippe Grandjean presented epidemiological data...... on children exposed to inorganic mercury and methylmercury, and discussed the behavioral outcome measures as they relate to age and stage of brain development. Donald A. Fox presented data that low-dose human equivalent gestational lead exposure produces late-onset obesity only in male mice that is associated...

  7. Burden of metastatic bone disease from genitourinary malignancies.

    Science.gov (United States)

    Mulders, Peter F; Abrahamsson, Per-Anders; Bukowski, Ronald M

    2010-11-01

    Bone metastases are common among patients with stage IV genitourinary cancers. Most patients with bone metastases develop at least one debilitating and potentially life-limiting skeletal-related event. These events are associated with increased medical expenses and decreased quality of life. Current guidelines recommend screening for bone metastases in men with high-risk prostate cancer, but guidance for screening and treatment of bone metastases from genitourinary cancers varies by country and setting. Several bisphosphonates have been evaluated in the advanced genitourinary cancer setting. Zoledronic acid has demonstrated efficacy in significantly reducing the risk of skeletal-related events in patients with bone metastases from a broad range of solid tumors including prostate, renal and bladder cancers, and is recommended for preserving bone health.

  8. 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.

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

    OpenAIRE

    Juan Marcos Mucci; Paula Rozenfeld

    2015-01-01

    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,...

  10. Bone marrow scintigraphy and computed tomography in myloproliferative disease

    Energy Technology Data Exchange (ETDEWEB)

    Goldsmith, S.J.; Gilbert, H.S.; Hermann, G.

    1985-05-01

    Peripheral bone marrow (BM) expansion in myeloproliferative disease (MPD) is demonstrated by scintigraphy (scint) with Technetium 99m sulfur colloid (TSC) or Indium III chloride (In). Computed tomography (CT) of the normal adult medullary cavity yields negative attenuation coefficients (AC) which become positive when BM fat is replaced. BM scint and CT of the medullary cavity are obtained in 23 studies in 21 pts: 6 polycythemia vera (PCV), 6 post PCV myeloid metaplasis (MyM), 4 agnogenic MyM, 3 myelodysplasia with refractory anemia, 1 acute myelocytic leukemia and 1 chronic myelocytic with acute leukemic transformation. AC were measured for BM cavity of lower extremities at each third of the femur and tibia. Values ranged from -89 to +289 Hounsfield units. The results are presented in this paper. There was agreement between SCINT and CT in 83% pts and segments. 80% of MB segments with + AC had scint identified BM. BM biopsy of the iliac crest demonstrated fibrosis or blast proliferation in pts with +AC rather than hypercellularity or osteosclerosis. The highest AC values (>200) were seen in pts with blast proliferation and fibrosis. Decreased BM scint visualization and +CT AC correlated with BM fibrosis and may reflect replacement of BM elements or decreased RES function. BM scint and CT are useful to monitor MPD and select BM sites for biopsy.

  11. Bone

    Science.gov (United States)

    Helmberger, Thomas K.; Hoffmann, Ralf-Thorsten

    The typical clinical signs in bone tumours are pain, destruction and destabilization, immobilization, neurologic deficits, and finally functional impairment. Primary malignant bone tumours are a rare entity, accounting for about 0.2% of all malignancies. Also benign primary bone tumours are in total rare and mostly asymptomatic. The most common symptomatic benign bone tumour is osteoid osteoma with an incidence of 1:2000.

  12. Pelvic bone and hip joint hydatid disease revealing a retroperitoneal location

    Directory of Open Access Journals (Sweden)

    Abdelhalim El Ibrahimi

    2009-07-01

    Full Text Available Echinococcosis is a parasitic disease produced by the larval stage of Echinococcus granulosus. Hydatid disease of bone is rarely seen in humans and it has been reported in only 1-2% of cases of echinococcosis. We present a patient who developed hydatid disease of the left pelvic and femoral bones with cartilage destruction of the ipsilateral hip joint revealing a retroperitoneal location of hydatid cyst. Hydatid bone must be present in the differential diagnosis of chronic monoarthritis; the risk is to perform a total hip replacement in a septic environment.

  13. Combined bone scintigraphy and indium-111 leukocyte scans in neuropathic foot disease

    Energy Technology Data Exchange (ETDEWEB)

    Schauwecker, D.S.; Park, H.M.; Burt, R.W.; Mock, B.H.; Wellman, H.N.

    1988-10-01

    It is difficult to diagnose osteomyelitis in the presence of neurotrophic osteoarthropathy. We performed combined (99mTc)MDP bone scans and indium-111 (111In) leukocyte studies on 35 patients who had radiographic evidence of neuropathic foot disease and clinically suspected osteomyelitis. The (111In)leukocyte study determined if there was an infection and the bone scan provided the anatomic landmarks so that the infection could be localized to the bone or the adjacent soft tissue. Seventeen patients had osteomyelitis and all showed increased (111In)leukocyte activity localized to the bone, giving a sensitivity of 100%. Among the 18 patients without osteomyelitis, eight had no accumulation of (111In)leukocytes, seven had the (111In)leukocyte activity correctly localized to the soft tissue, two had (111In)leukocyte activity mistakenly attributed to the bone, and one had (111In)leukocyte accumulation in a proven neuroma which was mistakenly attributed to bone. These three false-positive results for osteomyelitis reduced the specificity to 83%. Considering only the 27 patients with a positive (111In)leukocyte study, the combined bone scan and (111In)leukocyte study correctly localized the infection to the soft tissues or bone in 89%. Uninfected neurotrophic osteoarthropathy does not accumulate (111In)leukocytes. We found the combined bone scan and (111In) leukocyte study useful for the detection and localization of infection to soft tissue or bone in patients with neuropathic foot disease.

  14. Clinical application of high resolution computed tomography of temporal bone diseases

    Energy Technology Data Exchange (ETDEWEB)

    Kanzaki, J.; Saito, S.; Shiga, H. (Keio Univ., Tokyo (Japan). School of Medicine)

    1981-11-01

    Using CT/T 8800 with high spatial resolution, target imaging of the various temporal bone diseases was performed. Coronal and axial scans were made. Target imaging is an effective means of studying patients with cholesteatomas, semicircular canal fistula, diseases of the internal auditory canal (acoustic neuroma, or stenosis) and inner ear anomaly (hypoplasia, or distension of the vestibulum). The results of the comparison of CT and polytomography of the temporal bone were as follows: 1. In most cases, the pathological process was demonstrated with CT scans better than or same as with polytomograms. 2. The radiation dose of CT scan is low. 3. The axial images of the temporal bone were easily obtained with CT. 4. CT is more effective than polytomography in the diagnosis and evaluation of the temporal bone involved by tumor and cholesteatoma, especially attic cholesteatoma. 5. In cases with temporal bone fracture, acoustic neuroma or other intracranial diseases, contrast-enhanced CT can be done.

  15. Petroleum ether extract of Cissus quadrangularis (LINN) stimulates the growth of fetal bone during intra uterine developmental period: a morphometric analysis

    OpenAIRE

    Bhagath Kumar Potu; Rao, Muddanna S.; N. Gopalan Kutty; Kumar MR Bhat; Mallikarjuna Rao Chamallamudi; Soubhagya Ranjan Nayak

    2008-01-01

    OBJECTIVE: The aim of the present study was to analyze the effect Cissus quadrangularis plant petroleum ether extract on the development of long bones during the intra-uterine developmental stage in rats. METHODS: Pregnant rats (n=12) were randomly assigned into either a control group (n=6) or a Cissus quadrangularis treatment (n=6) group. Pregnant rats in the Cissus quadrangularis group were treated with Cissus quadrangularis petroleum ether extract at a dose of 500 mg/kg body weight from ge...

  16. Risk Factors for Low Bone Mineral Density in Institutionalized Individuals with Developmental Disabilities

    Directory of Open Access Journals (Sweden)

    Michael A. Vice

    2015-07-01

    Conclusion: Follow-up studies should focus on how supplementation and medication changes may or may not alter BMD. Persons with IDD are experiencing longer life expectancies, and therefore, studies ascertaining information on diseases associated with this aging population are warranted.

  17. 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...... and capillarization, with the development of continuous sheets of basement membrane material beneath endothelial cells....

  18. Scedosporium apiospermum in chronic granulomatous disease treated with an HLA matched bone marrow transplant

    OpenAIRE

    Gompels, M M; Bethune, C A; Jackson, G; Spickett, G P

    2002-01-01

    A patient with chronic granulomatous disease who was being treated with steroids was diagnosed with a soft tissue Scedosporium apiospermum infection. Despite extensive treatment with antifungals progression to involve solid tissue (bone) occurred. Treatment required an HLA matched bone marrow transplant, which led to complete clearance of the fungal infection, although the patient subsequently died.

  19. 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.

  20. An Unusual Developmental Profile of Salla Disease in a Patient with the SallaFIN Mutation

    Directory of Open Access Journals (Sweden)

    Liisa E. Paavola

    2012-01-01

    Full Text Available Salla disease (SD is a disorder caused by defective storage of free sialic acid and results from mutations in the SLC17A5 gene. Early developmental delay of motor functions, and later cognitive skills, is typical. We describe a developmental profile of an unusual homozygous patient, who harboured the SallaFIN (p.R39C mutation gene. The study involved neurological examination, neuropsychological investigation, and brain imaging. The neurocognitive findings were atypical in comparison with other patients with the SallaFIN mutation. Interestingly, there was no deterioration in the patient's neurological condition during adulthood. Her neurocognitive skills were remarkably higher than those of other patients with a conventional phenotype of SD. Our results suggest that the phenotype of SD is broad. Unidentified genetic or environmental variation might explain the unique SD type of this case.

  1. Burden of metastatic bone disease from genitourinary malignancies.

    NARCIS (Netherlands)

    Mulders, P.F.A.; Abrahamsson, P.A.; Bukowski, R.M.

    2010-01-01

    Bone metastases are common among patients with stage IV genitourinary cancers. Most patients with bone metastases develop at least one debilitating and potentially life-limiting skeletal-related event. These events are associated with increased medical expenses and decreased quality of life. Current

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

    Directory of Open Access Journals (Sweden)

    Rodney R. Dietert

    2014-01-01

    Full Text Available 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.

  3. Emerging strategies and therapies for treatment of Paget’s disease of bone

    Directory of Open Access Journals (Sweden)

    Brown JP

    2011-04-01

    Full Text Available Laëtitia Michou, Jacques P BrownLaval University, Department of Medicine, CHUQ (CHUL Research Centre and Division of Rheumatology, Quebec City, QC, CanadaAbstract: Paget’s disease of bone (PDB is a progressive monostotic or polyostotic metabolic bone disease characterized by focal abnormal bone remodeling, with increased bone resorption and excessive, disorganized, new bone formation. PDB rarely occurs before middle age, and it is the second most frequent metabolic bone disorder after osteoporosis, affecting up to 3% of adults over 55 years of age. One of the most striking and intriguing clinical features is the focal nature of the disorder, in that once the disease is established within a bone, there is only local spread within that bone and no systemic dissemination. Despite many years of intense research, the etiology of PDB has still to be conclusively determined. Based on a detailed review of genetic and viral factors incriminated in PDB, we propose a unifying hypothesis from which we can suggest emerging strategies and therapies. PDB results in weakened bone strength and abnormal bone architecture, leading to pain, deformity or, depending on the bone involved, fracture in the affected bone. The diagnostic assessment includes serum total alkaline phosphatase, total body bone scintigraphy, skull and enlarged view pelvis x-rays, and if needed, additional x-rays. The ideal therapeutic option would eliminate bone pain, normalize serum total alkaline phosphatase with prolonged remission, heal radiographic osteolytic lesions, restore normal lamellar bone, and prevent recurrence and complications. With the development of increasingly potent bisphosphonates, culminating in the introduction of a single intravenous infusion of zoledronic acid 5 mg, these goals of treatment are close to being achieved, together with long-term remission in almost all patients. Based on the recent pathophysiological findings, emerging strategies and therapies are

  4. Development, validation and characterization of a novel mouse model of Adynamic Bone Disease (ABD).

    Science.gov (United States)

    Ng, Adeline H; Willett, Thomas L; Alman, Benjamin A; Grynpas, Marc D

    2014-11-01

    The etiology of Adynamic Bone Disease (ABD) is poorly understood but the hallmark of ABD is a lack of bone turnover. ABD occurs in renal osteodystrophy (ROD) and is suspected to occur in elderly patients on long-term anti-resorptive therapy. A major clinical concern of ABD is diminished bone quality and an increased fracture risk. To our knowledge, experimental animal models for ABD other than ROD-ABD have not been developed or studied. The objectives of this study were to develop a mouse model of ABD without the complications of renal ablation, and to characterize changes in bone quality in ABD relative to controls. To re-create the adynamic bone condition, 4-month old female Col2.3Δtk mice were treated with ganciclovir to specifically ablate osteoblasts, and pamidronate was used to inhibit osteoclastic resorption. Four groups of animals were used to characterize bone quality in ABD: Normal bone controls, No Formation controls, No Resorption controls, and an Adynamic group. After a 6-week treatment period, the animals were sacrificed and the bones were harvested for analyses. Bone quality assessments were conducted using established techniques including bone histology, quantitative backscattered electron imaging (qBEI), dual energy X-ray absorptiometry (DXA), microcomputed tomography (microCT), and biomechanical testing. Histomorphometry confirmed osteoblast-related hallmarks of ABD in our mouse model. Bone formation was near complete suppression in the No Formation and Adynamic specimens. Inhibition of bone resorption in the Adynamic group was confirmed by tartrate-resistant acid phosphatase (TRAP) stain. Normal bone mineral density and architecture were maintained in the Adynamic group, whereas the No Formation group showed a reduction in bone mineral content and trabecular thickness relative to the Adynamic group. As expected, the No Formation group had a more hypomineralized profile and the Adynamic group had a higher mean mineralization profile that is

  5. Petroleum ether extract of Cissus quadrangularis (LINN stimulates the growth of fetal bone during intra uterine developmental period: a morphometric analysis

    Directory of Open Access Journals (Sweden)

    Bhagath Kumar Potu

    2008-01-01

    Full Text Available OBJECTIVE: The aim of the present study was to analyze the effect Cissus quadrangularis plant petroleum ether extract on the development of long bones during the intra-uterine developmental stage in rats. METHODS: Pregnant rats (n=12 were randomly assigned into either a control group (n=6 or a Cissus quadrangularis treatment (n=6 group. Pregnant rats in the Cissus quadrangularis group were treated with Cissus quadrangularis petroleum ether extract at a dose of 500 mg/kg body weight from gestation day 9 until delivery. The animals in the control group received an equal volume of saline. Newborn pups were collected from both groups for alizarin red S - alcian blue staining to differentiate ossified and unossified cartilage. The ossified cartilage (bone was morphometrically analyzed using Scion image software. RESULTS: Morphometric analysis revealed that the percentage of the total length of ossified cartilage (bone in pups born to treated dams was significantly higher (P<0.001- -0.0001 than that of the control group. CONCLUSION: The results of the present study suggest that maternal administration of Cissus quadrangularis petroleum ether extract during pregnancy can stimulate the development of fetal bone growth during the intra-uterine developmental period.

  6. Identification of microRNAs regulating the developmental pathways of bone marrow derived mast cells.

    Directory of Open Access Journals (Sweden)

    Yang Xiang

    Full Text Available BACKGROUND: MicroRNAs (miRNAs play important roles in leukocyte differentiation, although those utilised for specific programs and key functions remain incompletely characterised. As a global approach to gain insights into the potential regulatory role of miRNA in mast cell differentiation we characterised expression in BM cultures from the initiation of differentiation. In cultures enriched in differentiating mast cells we characterised miRNA expression and identified miRNA targeting the mRNA of putative factors involved in differentiation pathways and cellular identity. Detailed pathway analysis identified a unique miRNA network that is intimately linked to the mast cell differentiation program. METHODOLOGY/PRINCIPAL FINDINGS: We identified 86 unique miRNAs with expression patterns that were up- or down- regulated at 5-fold or more during bone marrow derived mast cells (BMMC development. By employing TargetScan and MeSH databases, we identified 524 transcripts involved in 30 canonical pathways as potentially regulated by these specific 86 miRNAs. Furthermore, by applying miRanda and IPA analyses, we predict that 7 specific miRNAs of this group are directly associated with the expression of c-Kit and FcεRIα and likewise, that 18 miRNAs promote expression of Mitf, GATA1 and c/EBPα three core transcription factors that direct mast cell differentiation. Furthermore, we have identified 11 miRNAs that may regulate the expression of STATs-3, -5a/b, GATA2 and GATA3 during differentiation, along with 13 miRNAs that target transcripts encoding Ndst2, mMCP4 and mMCP6 and thus may regulate biosynthesis of mast cell secretory mediators. CONCLUSIONS/SIGNIFICANCE: This investigation characterises changes in miRNA expression in whole BM cultures during the differentiation of mast cells and predicts functional links between miRNAs and their target mRNAs for the regulation of development. This information provides an important resource for further

  7. 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.

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

    Science.gov (United States)

    ... calculated to be one in 2.8 billion [Russo 1995]. Therefore, the established exclusionary criteria combined with ... bone allograft. J Periodontol 1992;12:979–983. Russo R, Scarborough N. Inactivation of viruses in demineralized ...

  9. Bone and mineral metabolism in adult celiac disease

    International Nuclear Information System (INIS)

    Bone mineral density (125I 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

  10. Bone Disease in Myeloma: The Claws of CRAB.

    Science.gov (United States)

    Fonseca, Rafael; Jain, Tania

    2016-03-15

    A dynamic approach to use bisphosphonates according to biomarkers of bone metabolism is presented in the Z-MARK study by Raje and colleagues. This is a major step forward toward a rational approach to bisphosphonate usage.

  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. Paget’s Disease of Bone Presented as Normal Pressure Hydrocephalus: A Case Report and Review of Literature

    Directory of Open Access Journals (Sweden)

    Abbas Tafakhori

    2012-07-01

    Full Text Available Background: Paget’s disease is a focal bone disorder manifested as bone overgrowth and disrupted bone integrity as a result of accelerated bone remodelling rate. Rarely, Paget’s disease of the base of the skull results in hydrocephalic dementia, and the triad of normal pressure hydrocephalus syndrome is a much more scarce entity.Case Report: Herein, we report an elderly woman who presented in Imam Khomeini Hospital, Tehran, Iran, with normal pressure hydrocephalus syndrome due to Paget’s bone disease. Furthermore, we have reviewed relevant previous studiesConclusion: Paget’s disease can be presented as normal pressure hydrocephalus syndrome

  13. Contemporary Approaches for Identifying Rare Bone Disease Causing Genes

    OpenAIRE

    Farber, Charles R; Clemens, Thomas L.

    2013-01-01

    Recent improvements in the speed and accuracy of DNA sequencing, together with increasingly sophisticated mathematical approaches for annotating gene networks, have revolutionized the field of human genetics and made these once time consuming approaches assessable to most investigators. In the field of bone research, a particularly active area of gene discovery has occurred in patients with rare bone disorders such as osteogenesis imperfecta (OI) that are caused by mutations in single genes. ...

  14. Altered interaction and distribution of glycosaminoglycans and growth factors in mucopolysaccharidosis type I bone disease.

    Science.gov (United States)

    Kingma, Sandra D K; Wagemans, Tom; IJlst, Lodewijk; Bronckers, Antonius L J J; van Kuppevelt, Toin H; Everts, Vincent; Wijburg, Frits A; van Vlies, Naomi

    2016-07-01

    The mucopolysaccharidoses (MPSs) comprise a group of lysosomal storage disorders characterized by deficient degradation and subsequent accumulation of glycosaminoglycans (GAGs). Progressive bone and joint disease are a major cause of morbidity, and current therapeutic strategies have limited effect on these symptoms. By elucidating pathophysiological mechanisms underlying bone disease, new therapeutic targets may be identified. Longitudinal growth is regulated by interaction between GAGs and growth factors. Because GAGs accumulate in the MPSs, we hypothesized that altered interaction between growth factors and GAGs contribute to the pathogenesis of MPS bone disease. In this study, binding between GAGs from MPS I chondrocytes and fibroblast growth factor 2 (FGF2) was not significantly different from binding of FGF2 to GAGs from control chondrocytes. FGF2 signaling, however, was increased in MPS I chondrocytes after incubation with FGF2, as compared to control chondrocytes. Using bone cultures, we demonstrated decreased growth of WT mouse bones after incubation with FGF2, but no effect on MPS I bone growth. However, MPS I bones showed decreased growth in the presence of GAGs from MPS I chondrocytes. Finally, we demonstrate altered GAG distribution in MPS I chondrocytes, and altered GAG, FGF2 and Indian hedgehog distribution in growth plates from MPS I mice. In summary, our results suggest that altered interaction and distribution of growth factors and accumulated GAGs may contribute to the pathogenesis of MPS bone disease. In the future, targeting growth factor regulation or the interaction between in growth factors and GAGs might be a promising therapeutic strategy for MPS bone disease. PMID:27105565

  15. Imaging Paget's disease of bone-from head to toe

    Energy Technology Data Exchange (ETDEWEB)

    Cortis, K.; Micallef, K. [Medical Imaging Department, Mater Dei Hospital, Msida (Malta); Mizzi, A., E-mail: adrian.mizzi@gov.mt [Medical Imaging Department, Mater Dei Hospital, Msida (Malta)

    2011-07-15

    Paget's disease of the bone is a common, non-inflammatory, metabolic, skeletal disorder of unknown aetiology characterized by an increase in osteoclast-mediated bone resorption and compensatory excessive osteoblast activation. Prevalence increases with age, and a pronounced geographical variation is well documented. The disease is often an incidental finding on a radiological examination requested for an unrelated indication. The osteolytic, mixed osteolytic/osteoblastic, and osteosclerotic phases may occur in the same patient and same bone in a synchronous or metachronous fashion. Radiological features in each phase mirror the histopathological appearances, and are distinctive enough to establish a diagnosis with confidence. Using multi-technique imaging, this review illustrates the most common and the not so common radiological patterns of involvement in Paget's disease of bone observed at our centre during the past 20 years.

  16. 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).

  17. Preliminary results of automated removal of degenerative joint disease in bone scan lesion segmentation

    Science.gov (United States)

    Chu, Gregory H.; Lo, Pechin; Kim, Hyun J.; Auerbach, Martin; Goldin, Jonathan; Henkel, Keith; Banola, Ashley; Morris, Darren; Coy, Heidi; Brown, Matthew S.

    2013-03-01

    Whole-body bone scintigraphy (or bone scan) is a highly sensitive method for visualizing bone metastases and is the accepted standard imaging modality for detection of metastases and assessment of treatment outcomes. The development of a quantitative biomarker using computer-aided detection on bone scans for treatment response assessment may have a significant impact on the evaluation of novel oncologic drugs directed at bone metastases. One of the challenges to lesion segmentation on bone scans is the non-specificity of the radiotracer, manifesting as high activity related to non-malignant processes like degenerative joint disease, sinuses, kidneys, thyroid and bladder. In this paper, we developed an automated bone scan lesion segmentation method that implements intensity normalization, a two-threshold model, and automated detection and removal of areas consistent with non-malignant processes from the segmentation. The two-threshold model serves to account for outlier bone scans with elevated and diffuse intensity distributions. Parameters to remove degenerative joint disease were trained using a multi-start Nelder-Mead simplex optimization scheme. The segmentation reference standard was constructed manually by a panel of physicians. We compared the performance of the proposed method against a previously published method. The results of a two-fold cross validation show that the overlap ratio improved in 67.0% of scans, with an average improvement of 5.1% points.

  18. 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.

  19. 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

  20. Contemporary Approaches for Identifying Rare Bone Disease Causing Genes

    Institute of Scientific and Technical Information of China (English)

    Charles R.Farber; Thomas L.Clemens

    2013-01-01

    Recent improvements in the speed and accuracy of DNA sequencing, together with increasingly sophisti-cated mathematical approaches for annotating gene networks, have revolutionized the field of human genetics and made these once time consuming approaches assessable to most investigators. In the field of bone research, a particularly active area of gene discovery has occurred in patients with rare bone disorders such as osteogenesis imperfecta (OI) that are caused by mutations in single genes. In this perspective, we highlight some of these technological advances and describe how they have been used to identify the genetic determinants underlying two previously unexplained cases of OI. The widespread availability of advanced methods for DNA sequencing and bioinformatics analysis can be expected to greatly facilitate identification of novel gene networks that normally function to control bone formation and maintenance.

  1. TGF-βand BMP signaling in osteoblast, skeletal development, and bone formation, homeostasis and disease

    Institute of Scientific and Technical Information of China (English)

    Mengrui Wu; Guiqian Chen; and Yi-Ping Li

    2016-01-01

    Transforming growth factor-beta (TGF-β) and bone morphogenic protein (BMP) signaling has fundamental roles in both embryonic skeletal development and postnatal bone homeostasis. TGF-βs and BMPs, acting on a tetrameric receptor complex, transduce signals to both the canonical Smad-dependent signaling pathway (that is, TGF-β/BMP ligands, receptors, and Smads) and the non-canonical-Smad-independent signaling pathway (that is, p38 mitogen-activated protein kinase/p38 MAPK) to regulate mesenchymal stem cell differentiation during skeletal development, bone formation and bone homeostasis. Both the Smad and p38 MAPK signaling pathways converge at transcription factors, for example, Runx2 to promote osteoblast differentiation and chondrocyte differentiation from mesenchymal precursor cells. TGF-βand BMP signaling is controlled by multiple factors, including the ubiquitin–proteasome system, epigenetic factors, and microRNA. Dysregulated TGF-βand BMP signaling result in a number of bone disorders in humans. Knockout or mutation of TGF-βand BMP signaling-related genes in mice leads to bone abnormalities of varying severity, which enable a better understanding of TGF-β/BMP signaling in bone and the signaling networks underlying osteoblast differentiation and bone formation. There is also crosstalk between TGF-β/BMP signaling and several critical cytokines’ signaling pathways (for example, Wnt, Hedgehog, Notch, PTHrP, and FGF) to coordinate osteogenesis, skeletal development, and bone homeostasis. This review summarizes the recent advances in our understanding of TGF-β/BMP signaling in osteoblast differentiation, chondrocyte differentiation, skeletal development, cartilage formation, bone formation, bone homeostasis, and related human bone diseases caused by the disruption of TGF-β/BMP signaling.

  2. Sudeck-type dystrophy in Paget's disease of bone. An anatomico-radiological approach.

    Science.gov (United States)

    Lagier, R

    1985-03-01

    Anatomico-radiological study of a humerus with Paget's disease and unhealed fracture makes it possible to demonstrate the uneven development of the disease according to local conditions. The possible role of a bone dystrophy similar to that in Sudeck's disease which might - along with a slow virus infection - be involved in the development of Paget's disease is discussed. The discussion is based on the present case, on two previous studies as well as on data found in the literature. PMID:2580661

  3. A Small Molecule, Odanacatib, Inhibits Inflammation and Bone Loss Caused by Endodontic Disease

    OpenAIRE

    Hao, Liang; Chen, Wei; McConnell, Matthew; Zhu, Zheng; Li, Sheng; Reddy, Michael; Eleazer, Paul D; Wang, Min; Li, Yi-Ping

    2015-01-01

    Periapical disease, an inflammatory disease mainly caused by dental caries, is one of the most prevalent infectious diseases of humans, affecting both children and adults. The infection travels through the root, leading to inflammation, bone destruction, and severe pain for the patient. Therefore, the development of a new class of anti-periapical disease therapies is necessary and critical for treatment and prevention. A small molecule, odanacatib (ODN), which is a cathepsin K (Ctsk) inhibito...

  4. Pain and bone disease: a patient’s view

    Directory of Open Access Journals (Sweden)

    L. Brunetta

    2011-12-01

    Full Text Available Pain in thalassemia proves to be an emergent issue even if it is not possible to correlate it definitely to bone disease, but we strongly believe that a multidisciplinary approach, may be as decisive in this case as it was in the struggle against thalassemia. In fact, we strongly believe that the involvement of various specialists such as endocrinologists, orthopedist, anesthesiologist, in a close team coordinated by a specialist in thalassemia is absolutely necessary for achieving our aims. First of all, we need to implement clinical trials to identify the mechanisms of disease, to find the optimal management of the problem in order to provide new therapeutic methods for preventing the thalassemia-induced osteoporosis and to reduce the presence of very disabling pain for patients. Patients’ expectations for the future are to continuously improve the quality of life. To do that it is needed to identify pathways to prevent all the complications of thalassemia that cause widespread pain, above all osteoporosis. Although we have seen that osteoporosis is not the sole cause of pain for thalassemia patients, it is true that this seems to have a great incidence in thalassemia patients and it gives a significant contribute to an increased pain. 地中海贫血疼痛亟待解决,即使它可能与和骨病毫不相关,但我们坚信可以找到一种战胜地中海贫血症的多学科结合疗法。 事实上,如果要完成我们的目标,绝对有必要邀请一名地中海贫血专家,在内分泌学家、矫形外科医师和麻醉学家组成的队伍的配合下紧密展开工作。 首先,我们需要开展临床试验,确认发病机制,找出疾病最佳的控制方法,以找到预防地中海贫血诱发骨质疏松症的新疗法和减少疼痛的频率。 病患者对未来的期望是能够不断地提高自己的生活质量。要做到,病患者需要找到预防地中海贫血所有并发症引起的疼痛的方

  5. 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

  6. A Case Report of Hydatid Disease in Long Bone

    Directory of Open Access Journals (Sweden)

    H Fanian

    2005-03-01

    Full Text Available Hydatid cyst, caused by echinococcus granulosa, can produce tissue cyst everywhere in body. Skeletal cystic lesion is rare especially in long bones like tibia and because of its unusual presentation, its diagnosis may easily be missed, unless be kept in mind.

  7. Chronic osteomyelitis: bone and gallium scan patterns associated with active disease

    International Nuclear Information System (INIS)

    Bone and gallium scans are used to assess osteomyelitis patients with prior bone disease. To refine the criteria for interpreting these scans, the data from 136 consecutive patients with clinically suspected osteomyelitis were reviewed. Active osteomyelitis was diagnosed with surgery or biopsy and culture in 49 patients, excluded with the same criteria in 16, and excluded by clinical follow-up for at least 6 months in 71. Five different scintigraphic patterns were found. The true-positive and false-positive ratios, the likelihood ratios, and posterior probabilities for active osteomyelitis in each pattern were calculated. Only one pattern (gallium uptake exceeding bone-seeking radiopharmaceutical uptake) was indicative of active disease. Other patterns slightly raised or decreased the probability of disease. The extent of these changes varies directly with the prior probability of disease, determined from patient-specific factors (e.g., clinical data, laboratory data, findings on plain films) known best by the referring clinician

  8. 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.

  9. Diagnosis and Treatment of Bone Disease in Multiple Myeloma: Spotlight on Spinal Involvement

    Directory of Open Access Journals (Sweden)

    Patrizia Tosi

    2013-01-01

    Full Text Available Bone disease is observed in almost 80% of newly diagnosed symptomatic multiple myeloma patients, and spine is the bone site that is more frequently affected by myeloma-induced osteoporosis, osteolyses, or compression fractures. In almost 20% of the cases, spinal cord compression may occur; diagnosis and treatment must be carried out rapidly in order to avoid a permanent sensitive or motor defect. Although whole body skeletal X-ray is considered mandatory for multiple myeloma staging, magnetic resonance imaging is presently considered the most appropriate diagnostic technique for the evaluation of vertebral alterations, as it allows to detect not only the exact morphology of the lesions, but also the pattern of bone marrow infiltration by the disease. Multiple treatment modalities can be used to manage multiple myeloma-related vertebral lesions. Surgery or radiotherapy is mainly employed in case of spinal cord compression, impending fractures, or intractable pain. Percutaneous vertebroplasty or balloon kyphoplasty can reduce local pain in a significant fraction of treated patients, without interfering with subsequent therapeutic programs. Systemic antimyeloma therapy with conventional chemotherapy or, more appropriately, with combinations of conventional chemotherapy and compounds acting on both neoplastic plasma cells and bone marrow microenvironment must be soon initiated in order to reduce bone resorption and, possibly, promote bone formation. Bisphosphonates should also be used in combination with antimyeloma therapy as they reduce bone resorption and prolong patients survival. A multidisciplinary approach is thus needed in order to properly manage spinal involvement in multiple myeloma.

  10. [Developmental origins of adult health and disease: an important concept for social inequalities in health].

    Science.gov (United States)

    Charles, M-A

    2013-08-01

    According to the theory of the developmental origins of adult health and disease, development in utero and in the first years of life are critical phases during which susceptibility to many chronic diseases is set. Diseases eventually occur only if the environment and lifestyle in later life is favorable. Exposure to chemicals (environmental or drug), to infectious agents, unbalanced nutrition, or psychosocial stress prenatally or in the first months/years of life are all factors which have been shown to impact long-term health of individuals. The consequences, however, are not limited to health. A demonstrative example was provided by the study of the influenza epidemic of 1918-1919 in the United States. Nationwide, it was estimated that the loss of income over a lifetime for individuals exposed during fetal life to this epidemic amounted to 14 billion dollars. This example demonstrates that an exposure during fetal life, which is not socially differentiated, may affect the social situation of individuals in adulthood. In many situations, it is much more difficult to separate the specific effect of a given exposure from the overall effect of the social environment. Indeed, it has been shown that socioeconomic status in childhood is associated with increased risk of mortality in adulthood, even after accounting for the socioeconomic status and risky behaviors in adulthood. Among the explanations, the theory of developmental origins of health credits of biological plausibility the model of critical periods early in which the individual is particularly vulnerable to certain exposures. Thus, ensuring the best conditions for the biological, physical, emotional and cognitive development of children in early life will enable them to reach their potential in terms of health and socioeconomic return to society. Investment in this period also brings the hope of reducing the perpetuation of social inequalities and health from generation to generation.

  11. Diagnosis and Treatment of Bone Disease in Multiple Myeloma: Spotlight on Spinal Involvement

    OpenAIRE

    Patrizia Tosi

    2013-01-01

    Bone disease is observed in almost 80% of newly diagnosed symptomatic multiple myeloma patients, and spine is the bone site that is more frequently affected by myeloma-induced osteoporosis, osteolyses, or compression fractures. In almost 20% of the cases, spinal cord compression may occur; diagnosis and treatment must be carried out rapidly in order to avoid a permanent sensitive or motor defect. Although whole body skeletal X-ray is considered mandatory for multiple myeloma staging, magnetic...

  12. 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.

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

    OpenAIRE

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

    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, ...

  14. Multiple Bone and Joint Diseases in a Nigerian Sickle Cell Anaemia: a Case Report

    OpenAIRE

    Olaniyi, John A.; Alagbe, Adekunle E.; Olutoogun, Toluwalase A.; Busari, Oluwasogo E.

    2012-01-01

    This case highlights the fact that bone involvement is the commonest clinical manifestation of Sickle Cell Disease (SCD) both in the acute settings such as painful vaso-occlusive crisis (VOC) and as a source of chronic, progressive debility such as avascular necrosis (AVN), chronic osteomyelitis and fixed flexion deformity of joints. Protracted multiple bone involvement i.e. bilateral femoral and left humeral chronic osteomyelitis, Left elbow, Left knee and right humeral septic arthritis toge...

  15. Health promotion and disease prevention strategies in older adults with intellectual and developmental disabilities.

    Science.gov (United States)

    Carmeli, Eli; Imam, Bita

    2014-01-01

    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.

  16. 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)

  17. The impact of methods for estimating bone health and the global burden of bone disease.

    Science.gov (United States)

    Cole, Zoë A; Dennison, Elaine M; Cooper, Cyrus

    2009-01-01

    Osteoporosis constitutes a major public health problem through its association with age related fractures. Fracture rates are generally higher in caucasian women than in other populations. Important determinants include estrogen deficiency in women, low body mass index, cigarette smoking, alcohol consumption, poor dietary calcium intake, physical inactivity, certain drugs and illnesses. Thus, modification of physical activity and dietary calcium/vitamin D nutrition should complement high risk approaches. In addition, the recently developed WHO algorithm for evaluation of 10-year absolute risk of fracture provides a means whereby various therapies can be targeted cost-effectively to those at risk. Risk factors, together with bone mineral density (BMD) and biochemical indices of bone turnover, can be utilised to derive absolute risks of fracture and cost-utility thresholds at which treatment is justified. These data will provide the basis for translation into coherent public health strategies aiming to prevent osteoporosis both in individuals and in the general population.

  18. 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. PMID:27630398

  19. 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.

  20. Possible deletion of a developmentally regulated heavy-chain variable region gene in autoimmune diseases

    Energy Technology Data Exchange (ETDEWEB)

    Yang, Pei-Ming; Olee, Tsaiwei; Kozin, F.; Carson, D.A.; Chen, P.P. (Research Institute of Scripps Clinic, La Jolla, CA (USA)); Olsen, N.J. (Vanderbilt Univ., Nashville, TN (USA)); Siminovitch, K.A. (Univ. of Toronto (Canada))

    1990-10-01

    Several autoantibody-associated variable region (V) genes are preferentially expressed during early ontogenic development, suggesting strongly that they are of developmental and physiological importance. As such, it is possible that polymorphisms in one or more of these genes may alter susceptibility to autoimmune disease. The authors have searched extensively for a probe related to a developmentally regulated V gene that has the power to differentiate among highly homologous V genes in human populations. Using such a probe (i.e., Humhv3005/P1) related to both anti-DNA and anti-IgG autoantibodies, they studied restriction fragment length polymorphisms in patients with rheumatoid arthritis and systemic lupus erythematosus and found an apparent heavy-chain V (V{sub H}) gene deletion that was nearly restricted to the autoimmune patients. These data suggest that deletions of physiologically important V{sub H} genes may increase the risk of autoimmunity through indirect effects on the development and homeostasis of the B-cell repertoire.

  1. The Seven Stages of Man: The Role of Developmental Stage on Medication Adherence in Respiratory Diseases.

    Science.gov (United States)

    Costello, Richard W; Foster, Juliet M; Grigg, Jonathan; Eakin, Michelle N; Canonica, Walter; Yunus, Fasail; Ryan, Dermot

    2016-01-01

    The circumstances and drivers of the decision to initiate, implement, or persist with a medication differ for individuals at each developmental stage. For school-age children with asthma, the social environment of their family's cultural beliefs and the influence of peer networks and school policies are strong determinants of medication adherence. The stage of adolescence can be a particularly challenging time because there is a reduction in parental supervision of asthma management as the young person strives to become more autonomous. To illustrate the importance of such factors, adherence interventions in children and young adults with asthma have used peer-based supports and social supports, particularly social media platforms. In older patients, it is internal rather than external factors and age-related decline that pose challenges to medication adherence. Seniors face the challenges of polypharmacy, reduced social support, increased isolation, and loss of cognitive function. Strategies to promote adherence must be tailored to the developmental stage and respective behavioral determinants of the target group. This review considers the different attitudes toward medication and the different adherence behaviors in young and elderly patients with chronic respiratory conditions, specifically asthma and chronic obstructive pulmonary disease. Opportunities to intervene to optimize adherence are suggested. PMID:27587315

  2. Bone diseases in rabbits with hyperparathyroidism:computed tomography, magnetic resonance imaging and histopathology

    Institute of Scientific and Technical Information of China (English)

    BAI Rong-jie; CONG De-gang; SHEN Bao-zhong; HAN Ming-jun; WU Zhen-hua

    2006-01-01

    Background Hyperparathyroidism (HPT) occurs at an early age and has a high disability rate. Unfortunately,confirmed diagnosis in most patients is done at a very late stage, when the patients have shown typical symptomsand signs, and when treatment does not produce any desirable effect. It has become urgent to find a method thatwould detect early bone diseases in HPT to obtain time for the ideal treatment. This study evaluated the accuracyof high field magnetic resonance imaging (MRI) combined with spiral computed tomography (SCT) scan indetecting early bone diseases in HPT, through imaging techniques and histopathological examinations on ananimal model of HPT.Methods Eighty adult rabbits were randomly divided into two groups with forty in each. The control group wasfed normal diet (Ca:P = 1:0.7); the experimental group was fed high phosphate diet (Ca:P = 1:7) for 3, 4, 5, or6-month intervals to establish the animal model of HPT. The staging and imaging findings of the early bonediseases in HPT were determined by high field MRI and SCT scan at the 3rd, 4th, 5th and 6th month. Each rabbitwas sacrificed after high field MRI and SCT scan, and the parathyroid and bones were removed for pathologicalexamination to evaluate the accuracy of imaging diagnosis.Results Parathyroid histopathological studies revealed hyperplasia, osteoporosis and early cortical boneresorption. The bone diseases in HPT displayed different levels of low signal intensity on T1WI and low tointermediate signal intensity on T2WI in bone of stage 0, Ⅰ, Ⅱ or Ⅲ, but showed correspondingly absent, probable,osteoporotic and subperiosteal cortical resorption on SCT scan.Conclusion High field MRI combined with SCT scan not only detects early bone diseases in HPT, but alsoindicates staging, and might be a reliable method of studying early bone diseases in HPT.

  3. Bone marrow transplantation for treatment of radiation disease. Problems involved

    International Nuclear Information System (INIS)

    Transplantation of bone marrow cells still is one of the major means available for treatment of radiation injuries. The decisive indication is the diagnostic of irreversible damage to the hemopoietic stem cells, which becomes manifest about 5 or 6 days after exposure, by severe granulocytopenia and simultaneous, progressive thrombopenia. The radiation dose provoking such severe injury is estimated to be at least 9-10 Gy of homogeneous whole-body irradiation. Preparatory measures for transplantation include proof of tissue compatibility of donor and patient, sufficient immunosuppression prior to and/or after irradiation and bone marrow transplantation. The donor's marrow should be free of T-cells. In spite of preparatory treatment, complications such as immunological reactions or disturbance of organ functions are to be very probable. These are treated according to therapy protocols. (orig./MG)

  4. Deregulation of Bone Forming Cells in Bone Diseases and Anabolic Effects of Strontium-Containing Agents and Biomaterials

    OpenAIRE

    Shuang Tan; Binbin Zhang; Xiaomei Zhu; Ping Ao; Huajie Guo; Weihong Yi; Guang-Qian Zhou

    2014-01-01

    Age-related bone loss and osteoporosis are associated with bone remodeling changes that are featured with decreased trabecular and periosteal bone formation relative to bone resorption. Current anticatabolic therapies focusing on the inhibition of bone resorption may not be sufficient in the prevention or reversal of age-related bone deterioration and there is a big need in promoting osteoblastogenesis and bone formation. Enhanced understanding of the network formed by key signaling pathways ...

  5. The CT flare response of metastatic bone disease in prostate cancer

    Energy Technology Data Exchange (ETDEWEB)

    Messiou, Christina; deSouza, Nandita M. (Cancer Research UK Clinical Magnetic Resonance Research Group, Inst. of Cancer Research and Royal Marsden NHS Foundation Trust, Surrey (United Kingdom)), email: Christina.Messiou@icr.ac.uk; Cook, Gary (Dept. of Nuclear Medicine, Royal Marsden NHS Foundation Trust, Surrey (United Kingdom)); Reid, Alison H.M.; Attard, Gerhardt; Dearnaley, David; deBono, Johann S. (Inst. of Cancer Research and Royal Marsden NHS Foundation Trust, Surrey (United Kingdom))

    2011-06-15

    Background New or worsening bone lesions in patients responding to treatment, known as the flare phenomenon is well described on 99mTc-MDP bone scintigraphy, but to our knowledge has not previously been described on CT. The appearance of new or worsening bone sclerosis on CT in patients with prostate cancer may therefore be erroneously classified as disease progression. Purpose To assess the incidence of osteoblastic healing flare response at 3-month CT assessment in patients with castrate-resistant prostate cancer and to identify associated features that enable differentiation from progressive metastatic bone disease at 3 months. Material and Methods CT scans of 67 patients with castrate-resistant prostate cancer undergoing treatment were reviewed by a radiologist blinded to clinical outcome. Changes in number, size, and density of metastatic bone lesions were documented and Response Evaluation Criteria in Solid Tumours (RECIST) in soft tissue lesions, alkaline phosphatase, prostate specific antigen, and 99mTc-MDP bone scans were used for correlation. Results Of the 39 patients who had 3- and 6-month follow-up, eight patients (21%) demonstrated an increase in number, size, or density of sclerotic lesions on the 3-month CT scan despite improvement in PSA and soft tissue lesions. Three out of eight patients (8%) maintained partial response/remained stable at follow-up and were defined as showing a flare response: in this group bone metastases evident on CT showed a qualitative and quantitative increase in density and no lesions faded at 3 months. In contrast, in all patients who progressed at 3 months by PSA/RECIST criteria (n = 8) bone lesions showed a mixed pattern with some lesions increasing and others decreasing in density. Conclusion The incidence of flare response of metastatic bone disease evident at 3-month post-treatment CT in patients with prostate cancer undergoing systemic treatment is 8%. In patients with falling PSA and stable/responding soft tissue

  6. 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.

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

    Science.gov (United States)

    Kagiya, Tadayoshi

    2016-01-01

    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. PMID:27529224

  8. 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)

  9. 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...

  10. 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.

  11. Alveolar bone loss associated to periodontal disease in lead intoxicated rats under environmental hypoxia.

    Science.gov (United States)

    Terrizzi, Antonela R; Fernandez-Solari, Javier; Lee, Ching M; Bozzini, Clarisa; Mandalunis, Patricia M; Elverdin, Juan C; Conti, María Ines; Martínez, María Pilar

    2013-10-01

    Previously reported studies from this laboratory revealed that rats chronically intoxicated with lead (Pb) under hypoxic conditions (HX) impaired growth parameters and induced damages on femoral and mandibular bones predisposing to fractures. We also described periodontal inflammatory processes under such experimental conditions. Periodontitis is characterised by inflammation of supporting tissues of the teeth that result in alveolar bone loss. The existence of populations living at high altitudes and exposed to lead contamination aimed us to establish the macroscopic, biochemical and histological parameters consistent with a periodontal disease in the same rat model with or without experimental periodontitis (EP). Sixty female rats were divided into: Control; Pb (1000ppm of lead acetate in drinking water); HX (506mbar) and PbHX (both treatments simultaneously). EP was induced by placing ligatures around the molars of half of the rats during the 14 days previous to the autopsy. Hemi-mandibles were extracted to evaluate bone loss by histomorphometrical techniques. TNFα plasmatic concentration was greater (palveolar bone loss, while Pb showed spontaneous bone loss also. In conclusion, these results show that lead intoxication under hypoxic environment enhanced not only alveolar bone loss but also systemic and oral tissues inflammatory parameters, which could aggravate the physiopathological alterations produced by periodontal disease.

  12. SPECT/CT in the assessment of bone scintigraphy in staging of patients with oncologic diseases

    International Nuclear Information System (INIS)

    We aimed to assess the role of single photon emission computed tomography-computed tomography (SPECT/ CT) for characterizing nonconclusive lesions, observed by planar WB bone scintigraphy and to evaluate the impact of SPECT/CT on oncologic patient management. Data from 269 patients with oncologic diseases who had undergone WB BS for staging and 29 of them with nonconclusive lesions, passed SPECT/CT of a selected volume, were retrospectively analyzed. Planar scintigraphy and SPECT/CT images were independently evaluated in separate sessions to minimize recall bias. Among the 269 WB BS, in 37 patients multiple bone metastases were confirmed. In 161 patients degenerative lesions in the skeleton were defined, 42 patients were with normal bone scan and in 29 patients the results were nonconclusive for bone metastases. The fused scan after SPECT/CT imaging confirmed bone metastases in 11 (38%) patients with 27 new metastatic lesions and benign degenerative lesions in 18 patients. SPECT/CT is better than planar WB BS for characterizing equivocal lesions that are observed by bone scintigraphy, thus SPECT/CT can have a significant impact on patient management in staging the oncologic disease. (authors)

  13. 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

  14. Surviving radiation disease with and without bone marrow transplantation

    International Nuclear Information System (INIS)

    It appears that the damage done by bone marrow transplantation far outweights its advantages. Victims of high radiation doses died from their injuries before they could benefit from the transplant functions. Following lower doses, the transplant was seen to take, but led to detrimental effects in the majority of patients. The few who survived the procedure did so because the graft had been rejected. It would be much wiser, if the time and energy needed for a transplantation were dedicated to an adequate cell substitution therapy carried out until such time that the patient's own stem cells are repaired. (orig./MG)

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

    Science.gov (United States)

    Marycz, Krzysztof; Mierzejewska, Katarzyna; Śmieszek, Agnieszka; Suszynska, Ewa; Malicka, Iwona; Kucia, Magda; Ratajczak, Mariusz Z

    2016-01-01

    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.

  16. Usefulness of bone marrow magnetic resonance imaging and indium-111-chloride bone marrow scintigraphy in patients with various hematological diseases

    International Nuclear Information System (INIS)

    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.)

  17. 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.).

  18. SPECTRUM OF DISEASES DIAGNOSED BY BONE MARROW EXAMINATION IN CENTRAL INDIA

    Directory of Open Access Journals (Sweden)

    Rajendra Kumar

    2014-01-01

    Full Text Available BACKGROUND : Hematological diseases are quite frequent in population and mostly present with anemia. Although diagnosed mostly by clinical examination and simple investigations , bone marrow examination is confirmatory for the diagnosis. Bone marrow aspiration is simple and safe invasive procedure. Spectrum of hematological diseases in the developing countries is different from those observed in developed countries. OBJECTIVE : There is a lack of information about the exact relative prevalence of hematological disorders especially in Central India that’s why this study was undertaken to correlate clinico - hematological and bone marrow findings. MATERIAL AND METHOD S : It is a retrospective study of 345 cases who underwent bone marrow examination in Department Of Pathology , Gandhi Medical College , Bhopal. RESULTS : In our study age range of cases was 2 - 80 years with male: female ratio of 1.28:1. Most common diseases observe d were megaloblastic anemia (41.81% , dimorphic anemia (16.72% , hypoplastic anemia (9.09% , ITP (8.36% and infective pathology (4.72%. In Hematological malignancies (20.28% , most common were AML (6.95% of overall & 34.28% of malignant diseases % follo wed by ALL (4.92 % overall and 24.28% % of malignancies. 3 cases of Gaucher’s disease were also reported.96.81% patients were anemic while 44.92% were pancytopenic. Most common presenting symptoms were fever (44.92% and bleeding manifestations (30.14% w hich include ecchymosis , epistaxis , pete chiae , me lena , hematuria and hematemesis. Splenomegaly , hepatomegaly and lymphadenopathy were found in 35.65% , 21.15% and 14.78% of cases respectively. CONCLUSION : In our study final diagnosis could be made in majori ty of cases (92.76% by bone marrow examination , although having varied clinical presentations such as fever , bleeding manifestations , ascites , hepatomegaly , splenomegalyetc and usually associated with anemia / pancytopenia/ thrombocytopenia

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

    International Nuclear Information System (INIS)

    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.)

  20. Comparative diagnostic value of bone scintigraphy and roentgenography in children with Legg-Calve-Perthes disease

    Energy Technology Data Exchange (ETDEWEB)

    Cavailloles, F.; Brauner, M.; Dandine, M.; Lonchampt, M.F.; Esteban, C.; Bensahel, H.; Patois, E.; Alperovitch, A.; Bock, B.

    1985-01-01

    To compare the value of hip roentgenograms and bone scintigraphy in Legg-Calve-Perthes (LCP) disease, a double blind study was done in 153 children, of whom 51 had LCP. Each procedure was read separately by two investigators who had no knowledge of clinical data. Variations across investigators were less significant for roentgenograms than for bone scans. Agreement on diagnosis between the two investigators was 95% for roentgenograms, against only 89% for bone scans. As regards the detailed analysis of each separate finding upon bone scintigraphy and assessment of this procedure's prognostic value, major discrepancies occurred between the two investigators. Diagnostic accuracy was very similar for the two procedures. Both bone scintigraphy and roentgenography showed a 0.86 sensitivity. Specificities were 0.93 and 0.99 respectively. For the subgroup of patients with early-stage LCP disease (33 cases), scintigraphy identified a slightly greater number of cases than roentgenography (0.88 and 0.76 respectively). Some disagreements between our results and those previously published in the literature may stem from biases such as the criteria for entering patients into the study or for establishing the final diagnosis. Roentgenography is obviously the first procedure to perform in a child with a painful hip or a limp. However, the results may be inconclusive in early-stage disease. In such cases, scintigraphy is needed and often avoids unnecessary traction or diagnostic delay.

  1. 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

  2. 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.

  3. A Framework to Address Challenges in Communicating the Developmental Origins of Health and Disease.

    Science.gov (United States)

    Winett, Liana; Wallack, Lawrence; Richardson, Dawn; Boone-Heinonen, Janne; Messer, Lynne

    2016-09-01

    Findings from the field of Developmental Origins of Health and Disease (DOHaD) suggest that some of the most pressing public health problems facing communities today may begin much earlier than previously understood. In particular, this body of work provides evidence that social, physical, chemical, environmental, and behavioral influences in early life play a significant role in establishing vulnerabilities for chronic disease later in life. Further, because this work points to the importance of adverse environmental exposures that cluster in population groups, it suggests that existing opportunities to intervene at a population level may need to refocus their efforts "upstream" to sufficiently combat the fundamental causes of disease. To translate these findings into improved public health, however, the distance between scientific discovery and population application will need to be bridged by conversations across a breadth of disciplines and social roles. And importantly, those involved will likely begin without a shared vocabulary or conceptual starting point. The purpose of this paper is to support and inform the translation of DOHaD findings from the bench to population-level health promotion and disease prevention, by: (1) discussing the unique communication challenges inherent to translation of DOHaD for broad audiences, (2) introducing the First-hit/Second-hit Framework with an epidemiologic planning matrix as a model for conceptualizing and structuring communication around DOHaD, and (3) discussing the ways in which patterns of communicating DOHaD findings can expand the range of solutions considered and encourage discussion of population-level solutions in relation to one another, rather than in isolation. PMID:27449924

  4. Early clinical characteristics according to developmental stage in children with definite moyamoya disease.

    Science.gov (United States)

    Kim, Young Ok; Joo, Sung-Pil; Seo, Bo-Ra; Rho, Young Il; Yoon, Woong; Woo, Young Jong

    2013-06-01

    The objective is to clarify the early clinical characteristics in childhood moyamoya disease (MD). Epidemiologic characteristics, symptoms and diagnostic rates were assessed in 64 children (0-18 years) with definite MD according to developmental stage: infancy (5; 0-1 years); toddlerhood/preschool age (22; 2-5 years); school age (29; 6-10 years); and adolescence (8; 11-18 years). The median ages at onset was 6.25 years and the female to male ratio was 1.9 (~2.5 in toddlerhood/preschool age and in adolescence, P=0.71). Previous headache was observed in 23% (14/64): frequently in school age (38%, P=0.02) and within 6 months before main symptoms (6/11). As an initial symptom, weakness was observed in 78% (50/64) mainly as transient ischemic attack (TIA, 61%) in limbs (90%) and unilaterally (82%). TIA was less frequent in infancy (40%, P=0.04). Seizure was observed in 27% (17/64): frequently in infancy (100%, Pchildren ~5 years (P<0.01). Severe headache associated with MD was observed in 14% (9/64). Provoking events were positive in 42% (27/64): in school age, frequently during eating (28%); and in toddlerhood/preschool age, during crying (27%). The diagnostic rates at 3 and 12 months from symptom-onset were 39% (80% during infancy vs. 28% in school age, P=0.14) and 67%, respectively. Symptomatic progression at diagnosis was observed in 38% (24/64). Initial clinical characteristics in childhood definite MD differed according to developmental stage and from at diagnosis.

  5. Cytopenia and Bone Marrow Dysplasia in a Case of Wilson's Disease.

    Science.gov (United States)

    Rau, Aarathi R; Usha, M; Mallya, Pooja; Rau, A T K

    2014-09-01

    We describe a sixteen year old with Wilson's disease on copper chelation and subsequent high dose oral zinc who developed severe anemia and neutropenia. Bone marrow aspirate done to evaluate the cause of bicytopenia revealed trilineage dysplasia. Correlating the clinical context with bone marrow and biochemical parameters, copper deficiency was suspected and he was given a trial of therapy, following which the hematological parameters improved. This case highlights hypocupremia as a reversible cause of bone marrow dysplasia in patients with Wilson's disease on chelation, where serum copper levels are not useful in the diagnosis. We also believe that monitoring of the blood counts in patients on copper chelation may provide a clue to impending copper deficiency.

  6. Cardiovascular Risk and Mineral Bone Disorder in Patients with Chronic Kidney Disease

    Directory of Open Access Journals (Sweden)

    Hagen Staude

    2013-03-01

    Full Text Available The term chronic kidney disease-mineral bone disorder has been coined recently to highlight that the disturbed mineral and bone metabolism is a major contributor to vascular calcification and finally cardiovascular disease. This syndrome is characterized by clinical, biochemical and/or histological findings, i.e. i biochemical alterations in the homeostasis of calcium, phosphate and their key player parathyroid hormone (PTH, Fibroblast growth factor-23 (FGF-23, klotho and vitamin-D, ii the occurrence of vascular and/or soft tissue calcification, and iii an abnormal bone structure and/or turnover. Apart from the combined and synergistic action of "traditional" and uremia-related risk factors, promoters and inhibitors of calcification have to be considered as well. This review will focus on the disturbed mineral metabolism as the triggering force behind distortion of vascular integrity and cardiovascular malfunction in CKD patients.

  7. Cardiovascular risk and mineral bone disorder in patients with chronic kidney disease.

    Science.gov (United States)

    Staude, Hagen; Jeske, Susann; Schmitz, Karin; Warncke, Gert; Fischer, Dagmar-Christiane

    2013-01-01

    The term chronic kidney disease-mineral bone disorder has been coined recently to highlight that the disturbed mineral and bone metabolism is a major contributor to vascular calcification and finally cardiovascular disease. This syndrome is characterized by clinical, biochemical and/or histological findings, i.e. i) biochemical alterations in the homeostasis of calcium, phosphate and their key player parathyroid hormone (PTH), Fibroblast growth factor-23 (FGF-23), klotho and vitamin-D, ii) the occurrence of vascular and/or soft tissue calcification, and iii) an abnormal bone structure and/or turnover. Apart from the combined and synergistic action of "traditional" and uremia-related risk factors, promoters and inhibitors of calcification have to be considered as well. This review will focus on the disturbed mineral metabolism as the triggering force behind distortion of vascular integrity and cardiovascular malfunction in CKD patients.

  8. Known VDR polymorphisms are not associated with bone mineral density measures in pediatric Cushing disease.

    Science.gov (United States)

    Lodish, Maya B; Mastroyannis, Spyridon A; Sinaii, Ninet; Boikos, Sosipatros A; Stratakis, Constantine A

    2012-01-01

    Decreased bone mineral density (BMD) has been documented in adults with Cushing disease (CD), and allelic variants of the vitamin D receptor (VDR) gene have been associated with osteopenia. Genetic factors play an important role in bone accrual and its response to various diseases; among them, the most studied are the allelic variants of the VDR gene. There is debate as to whether described variants in the VDR gene have an effect on BMD. In the current study, we sought to analyze whether BMD differences in patients with CD were associated with the Taq1 and Apal VDR allelotypes. The data showed lack of association between BMD and these widely studied VDR polymorphisms, suggesting that the effect of endogenous hypercortisolism on bone in the context of CD does not depend on VDR genotypes.

  9. A small molecule, odanacatib, inhibits inflammation and bone loss caused by endodontic disease.

    Science.gov (United States)

    Hao, Liang; Chen, Wei; McConnell, Matthew; Zhu, Zheng; Li, Sheng; Reddy, Michael; Eleazer, Paul D; Wang, Min; Li, Yi-Ping

    2015-04-01

    Periapical disease, an inflammatory disease mainly caused by dental caries, is one of the most prevalent infectious diseases of humans, affecting both children and adults. The infection travels through the root, leading to inflammation, bone destruction, and severe pain for the patient. Therefore, the development of a new class of anti-periapical disease therapies is necessary and critical for treatment and prevention. A small molecule, odanacatib (ODN), which is a cathepsin K (Ctsk) inhibitor, was investigated to determine its ability to treat this disease in a mouse model of periapical disease. While Ctsk was originally found in osteoclasts as an osteoclast-specific lysosomal protease, we were surprised to find that ODN can suppress the bacterium-induced immune response as well as bone destruction in the lesion area. X rays and microcomputed tomography (micro-CT) showed that ODN treatment had significant bone protection effects at different time points. Immunohistochemical and immunofluorescent staining show that ODN treatment dramatically decreased F4/80+ macrophages and CD3+ T cells in the lesion areas 42 days after infection. Consistent with these findings, quantitative real-time PCR (qRT-PCR) and enzyme-linked immunosorbent assay (ELISA) analysis showed low levels of proinflammatory mRNAs (for tumor necrosis factor alpha, interleukin 6, and interleukin 23α) and corresponding cytokine expression in the ODN-treated disease group. The levels of mRNA for Toll-like receptors 4, 5, and 9 also largely decreased in the ODN-treated disease group. Our results demonstrated that ODN can inhibit endodontic disease development, bone erosion, and immune response. These results indicate that application of this small molecule offers a new opportunity to design effective therapies that could prevent periapical inflammation and revolutionize current treatment options. PMID:25583522

  10. Suspected fetal skeletal malformations or bone diseases: how to explore

    Energy Technology Data Exchange (ETDEWEB)

    Cassart, Marie [Erasme Hospital, Medical Imaging, Brussels (Belgium)

    2010-06-15

    Skeletal dysplasias are a heterogeneous and complex group of conditions that affect bone growth and development and result in various anomalies in shape and size of the skeleton. Although US has proved reliable for the prenatal detection of skeletal abnormalities, the precise diagnosis of a dysplasia is often difficult to make before birth (especially in the absence of a familial history) due to their various phenotypic presentations, the variability in the time at which they manifest and often, the lack of precise molecular diagnosis. In addition to the accuracy of the antenatal diagnosis, it is very important to establish a prognosis. This is a clinically relevant issue as skeletal dysplasias may be associated with severe disability and may even be lethal. We will therefore describe the respective role of two-dimensional (2-D) US, three-dimensional (3-D) US and CT in the antenatal assessment of skeletal malformations. (orig.)

  11. Paget's Disease of Bone and Osteoarthritis: Different Yet Related

    Science.gov (United States)

    ... pain relief techniques, and alternative therapies such as acupuncture and nutritional supplements. In certain cases, surgery on ... of Dental and Craniofacial Research National Institute of Diabetes and Digestive and Kidney Diseases NIH Office of ...

  12. Information for Patients about Paget's Disease of Bone

    Science.gov (United States)

    ... complications of Paget’s disease: Fractures. Surgery may allow fractures to heal in better position. Severe degenerative arthritis. Hip or knee replacement may be considered if disability is severe ...

  13. Chronic kidney disease and bone fracture: a growing concern

    OpenAIRE

    Nickolas, Thomas L.; Leonard, Mary B.; Shane, Elizabeth

    2008-01-01

    Susceptibility to fracture is increased across the spectrum of chronic kidney disease (CKD). Moreover, fracture in patients with end-stage kidney disease (ESKD) results in significant excess mortality. The incidence and prevalence of CKD and ESKD are predicted to increase markedly over the coming decades in conjunction with the aging of the population. Given the high prevalence of both osteoporosis and CKD in older adults, it is of the utmost public health relevance to be able to assess fract...

  14. NIEMANN PICK DISEASE DIAGNOSED ON BONE MARROW ASPIRATION

    OpenAIRE

    Amita; Ankit; Mahendra

    2015-01-01

    Niemann - pick disease is a rare lysosomal storage disorder affecting 1 in 2,50,000 children. We are describing here, a case of Niemann - pick disease in a 19 months old female baby, who presented with distension of abdomen for 9 months and she was not able to sit or stand u n til this age. On examina tion of abdomen there was hepatosplenomegaly. Ultrasound abdomen also revealed hepatosplenomegaly along with multiple mesenteric and periportal lymph no...

  15. 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 A.); K. Estrada Gil (Karol); U. Styrkarsdottir (Unnur); E. Evangelou (Evangelos); Y.-H. Hsu; E.L. Duncan (Emma); E.E. Ntzani (Evangelia); L. Oei; 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; 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; 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; 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 (Huibert A. P.); 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 (Cock); 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 (J. 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. W

  16. Bone marrow diseases in children significance of MRI in comparison to other imaging modalities

    Energy Technology Data Exchange (ETDEWEB)

    Reither, M.; Kaiser, W.; Zeitler, E.

    1987-01-01

    The authors present the study of MRI in comparison to other imaging modalities in bone marrow diseases in children. The technic turned out to be interesting, its main advantages are: high sensitivity, good contrast, visualization of vascular structures, but its disadvantages are inferior detail resolution of periosteal changes and low specificity.

  17. 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...

  18. Photon-deficient bone scan lesion as a precursor of active Paget's disease

    Energy Technology Data Exchange (ETDEWEB)

    Jaspers, M.M.J.R.; Pauwels, E.K.J.; Blom, J. (Department of Diagnostic Radiology, Division of Nuclear Medicine, University Hospital Leiden (Netherlands)); Harinck, H.I.J. (Clinical Investigation Unit, Department of Endocrinology, University Hospital Leiden, Netherands)

    1984-05-15

    A case is presented in which a Pagetoid lesion was demonstrated as a photon-deficient area (cold spot) on the bone scan. This area changed into a hot spot 3 years after its discovery. Clinical and radiological observations provide evidence that a scintigraphically photon-deficient area may represent a precursor of active Paget's disease.

  19. D-dimer assay in Egyptian patients with Gaucher disease: correlation with bone and lung involvement.

    Science.gov (United States)

    Sherif, Eman M; Tantawy, Azza A G; Adly, Amira A M; Kader, Hossam A; Ismail, Eman A R

    2011-04-01

    Gaucher disease is the most frequent lysosomal storage disorder. Bone and lung involvement are two major causes of morbidity in this disease. D-dimer is a reliable indicator of active microvascular thrombosis, even in patients without overt hypercoagulation. This study aimed to assess D-dimer levels in Gaucher disease, correlating this marker to clinical characteristics and radiological parameters to investigate its role as a potential predictor for the occurrence and severity of skeletal and pulmonary manifestations. The study population consisted of 56 Egyptian patients with Gaucher disease, 36 had type 1 Gaucher disease (64.3%) and 20 had type 3 Gaucher disease (35.7%). Thirty healthy individuals were enrolled as a control group. D-dimer levels were significantly higher in all patients with Gaucher disease compared with controls (P < 0.001). Patients with type 3 showed significantly higher D-dimer concentrations compared with type 1 (P < 0.001). Pulmonary involvement was present in a significant proportion among type 3 Gaucher patients (P < 0.05), whereas bone changes were present in a higher percentage in type 1 compared with type 3 Gaucher patients. D-dimers were significantly higher in patients with abnormal MRI findings of the long bones and in those with ground glass appearance on high-resolution computerized tomography of the chest compared with patients with normal radiology (P < 0.001). Splenectomized patients displayed significantly higher D-dimer levels compared with nonsplenectomized patients (P < 0.001). Our results suggest that D-dimer is significantly elevated in Gaucher disease, particularly type 3, and may be considered as a potential marker of risk prediction of bone and lung involvement that could be used to monitor treatment response.

  20. 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)

    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

  1. Contemporary management of metastatic bone disease: tips and tools of the trade for general practitioners.

    Science.gov (United States)

    Quinn, Robert H; Randall, R Lor; Benevenia, Joseph; Berven, Sigurd H; Raskin, Kevin A

    2014-01-01

    Metastatic bone disease has a significant effect on a patient's mortality and health-related quality of life. An aging US population and improved survival rates of patients with cancer have led to an increase in the incidence of symptomatic bony metastatic lesions that may require orthopaedic care. Skeletal-related events in neoplastic disease include pain, pathologic fracture, hypercalcemia, and neural compression, including spinal cord compression. The clinical evaluation and diagnostic study of a patient with a skeletal lesion of unknown etiology should be approached carefully. In patients with widespread metastatic disease, the treatment of a skeletal-related event may be limited to stabilization of the pathologic fracture or local disease control. The treatment of metastatic bone disease is guided by the nature of the skeletal-related event, the responsiveness of the lesion to adjuvant care, and the overall condition and survival expectations of the patient. Impending pathologic fractures are often more easily treated, with less morbidity and easier recovery for patients, than completed fractures. Quality of life is the most important outcome measure in these patients. When surgery is indicated, the approach, choice of fixation, and use of adjuvant should allow for immediate and unrestricted weight bearing. Because metastatic lesions to the skeleton have a limited capacity for spontaneous healing, surgical fixation should be durable for the life expectancy of the patient. In the epiphyseal region of long bones, replacement arthroplasty is generally preferred over internal fixation. Metaphyseal and diaphyseal regions can generally be addressed with intramedullary nailing or plate fixation with adjuvant. The specific treatment of acetabular lesions is dictated by the anatomy and the degree of bone loss. Spinal stability and neural compromise are important considerations in choosing a strategy for managing spine tumors. Effective surgical approaches to metastatic

  2. Turner′s syndrome presenting as metabolic bone disease

    Directory of Open Access Journals (Sweden)

    Sadishkumar Kamalanathan

    2012-01-01

    Full Text Available Turner′s syndrome is a genetic disorder with a complete or partial absence of one X chromosome with characteristic phenotypic features. The prevalence of renal anomalies in turner syndrome is 30-40%. However, the renal function is usually normal. We report a case of Turner′s syndrome presenting with chronic kidney disease and renal osteodystrophy.

  3. 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

  4. 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.

  5. Developmental origins of health and disease--global public health implications.

    Science.gov (United States)

    Hanson, M A; Gluckman, P D

    2015-01-01

    The rapidly rising prevalence of non-communicable diseases (NCDs) represents a major challenge to public health and clinical medicine globally. NCDs are increasing rapidly in high-income countries, but even more rapidly in some low-middle-income countries with insufficient resources to meet the challenge. Whilst not identified in the Millennium Development Goals, there is much attention paid to NCDs in the discussions at many levels on the Sustainable Development Goals, as they underpin economic, social and environmental development in the post-2015 era. In this article, we discuss how a life-course approach to health, commencing of necessity in early development, can provide new opportunities for addressing this challenge. The approach can leverage human health capital throughout life and across generations. New insights into mechanisms, especially those processes by which the developmental environment affects epigenetic processes in the developing offspring, offer the prospect of identifying biomarkers of future risks. New interventions to promote health literacy, lifestyle and physical fitness in adolescents, young adults and their children hold great promise. In this respect, health-care professionals concerned with preconceptional, pregnancy and newborn care will have a vital role to play. PMID:25225058

  6. Conventional radiography and bone scintigraphy in the prognostic evaluation of Legg-Calve-Perthes disease

    Energy Technology Data Exchange (ETDEWEB)

    Kaniklides, C. [Dept. of Diagnostic Radiology, Univ. Hospital, Uppsala (Sweden); Sahlstedt, B. [Dept. of Diagnostic Radiology, Univ. Hospital, Uppsala (Sweden); Loennerholm, T. [Dept. of Diagnostic Radiology, Univ. Hospital, Uppsala (Sweden); Moberg, A. [Dept of Orthopedics, Univ. Hospital, Uppsala (Sweden)

    1996-07-01

    Purpose: The role of conventional radiography and bone scintigraphy in predicting the outcome of Legg-Calve-Perthes disease was investigated. Material and Methods: The 75 children reviewed (86 hips) were followed up to the primary healing of the disease. The findings at conventional radiography (obtained at presentation, at the time of maximum capital head involvement, and at the end of the healing process of the disease) were compared to early bone scintigraphy features. Results and Conclusion: Bone scintigraphy provided more accurate information concerning the extent of the necrotic process than initial radiographs. Moreover if could determine revascularization and consequently the stage of the disease. The method was, however, unable to predict the outcome of the disease in some of the cases, particularly if it was performed late after the onset of symptoms. Conventional radiography provided important information about other parameters such as `head-at`risk` signs which facilitated treatment selection. Of thse signs not only lateral subluxation but also metaphyseal changes strongly predispose to severe deformity of the hip joint. (orig.).

  7. Laser technologies in treatment of degenerative-dystrophic bone diseases in children

    Science.gov (United States)

    Abushkin, Ivan A.; Privalov, Valery A.; Lappa, Alexander V.; Noskov, Nikolay V.; Neizvestnykh, Elena A.; Kotlyarov, Alexander N.; Shekunova, Yulia G.

    2014-03-01

    Two low invasive laser technologies for treatment of degenerative-dystrophic bone diseases in children are presented. The first is the transcutaneous laser osteoperforation developed by us and initially applied for treatment of different inflammatory and traumatic diseases (osteomyelitides, osteal and osteoarticular panaritiums, delayed unions, false joints, and others). Now the technology was applied to treatment of aseptic osteonecrosis of different localizations in 134 children aged from 1 to 16 years, including 56 cases with necrosis of femoral head (Legg-Calve-Perthes disease), 42 with necrosis of 2nd metatarsal bone head (Kohler II disease), and 36 with necrosis of tibial tuberosity (Osgood-Schlatter disease). The second technology is the laser intracystic thermotherapy for treatment of bone cysts. The method was applied to 108 children aged from 3 to 16 years with aneurismal and solitary cysts of different localizations. In both technologies a 970 nm diode laser was used. The suggested technologies increase the efficiency of treatment, reduce its duration, can be performed on outpatient basis, which resulted in great economical effect.

  8. Developmental characteristics of various types of hand bones of Poland's syndrome%Poland综合征手部不同类型骨骼的发育特征

    Institute of Scientific and Technical Information of China (English)

    吴冯胜; 田文; 赵俊会; 马炜; 郭阳; 殷耀斌

    2016-01-01

    Objective To explore development status in different types of the hand bone and its developmental characteristics with Poland syndrome.Methods There were 32 cases with Poland's syndrome who accepted bilateral hand X-ray examination in Department of Hand Surgery,Beijing Jishuitan Hospital from February 2013 to August 2014.There were 24 male and 8 female patients aged from 1.0 to 15.0 years with median age of 2.4 years.Right hand deformity was 23 cases and left hand deformity was 9 cases.According to Tanner-Whitehouse skeletal age scoring system,20 bones (radius and ulna,7 carpal bones,11 metacarpal and phalangeal bones) selected from the affected and contralateral limb respectively,were evaluated.Besides,hand deformity of the cases was classified into 5 types based on relevant literature.Each bone was given an individual age using the references of Greulich-Pyle chart.The average of all individual ages was taken as gross bone age,the average of individual ages of radius and ulna was taken as bone age of long bones,the average of individual ages of carpal bone was taken as bone age of carpal bones,and the average of individual age of metacarpal and phalangeal bones was taken as bone age of short bones.The delay of bone age was evaluated by correlation test,while the curve of cubic equation was used for analyzing the variance of skeletal development with age.Results The delay of long bone age of patients with Poland's syndrome in this study were 0-1.9 years ((0.5 ±0.5) years),0-2.2 years ((0.7 ±0.5)years) for carpal bone,0.5-2.0 years((0.6 ±0.4) years)for short bone and 0.1-1.7 years((0.6 ±0.4)years) for gross bone.Twelve cases in type Ⅱ hand deformity,15 cases in type Ⅲ and 5 cases in type Ⅳ.The delay of bone ages,including long bone age,carpal bone age,short bone age and gross bone age,was not related with gender and side(all P > 0.05),but related with degree of deformity(F =3.663-12.971,P =0.000-0.038).Conclusion Compared with normal upper limb,the bone

  9. Detection of Unknown sites of multiple enchondroma (Ollier′s Disease mimicking like metastasis using bone scintigraphy

    Directory of Open Access Journals (Sweden)

    Koramadai Karuppusamy Kamaleshwaran

    2015-01-01

    Full Text Available Ollier′s disease characterized by multiple skeletal enchondroma is a rare noninherited disease of unknown etiology. Majority of the skeletal enchondroma are present in the metaphyses and diaphysis of tubular limb bones. Ollier′s disease has a predilection for unilateral distribution. Malignant changes in Ollier′s disease may occur in adult patients. Radionuclide bone scanning is one method used to assess lesions depicted on radiographs or magnetic resonance images that are presumed to be enchondromas. Furthermore, a bone scan may give a clue to the multifocality of the disease. We report a case of right first phalangeal enchondroma in a 23-year-old male, who underwent bone scintigraphy detected multifocal asymmetric right side involvement of radius, humerus, femur, and tibia which confirm a diagnosis of Ollier′s disease.

  10. Legg-Calve-Perthes disease. Comparison of conventional radiography, MR imaging, bone scintigraphy and arthrography

    Energy Technology Data Exchange (ETDEWEB)

    Kaniklides, C. [Dept. of Diagnostic Radiology, Univ. Hospital, Uppsala (Sweden); Loennerholm, T. [Dept. of Diagnostic Radiology, Univ. Hospital, Uppsala (Sweden); Moberg, A. [Dept. of Orthopaedics, Univ. Hospital, Uppsala (Sweden); Sahlstedt, B. [Dept. of Diagnostic Radiology, Univ. Hospital, Uppsala (Sweden)

    1995-07-01

    In a prospective study of 22 patients (24 hips) with Legg-Calve-Perthes disease (LCPD) the findings at conventional radiography, arthrography, bone scintigraphy and MR imaging, obtained at the time of diagnosis, were compared. MR was superior to conventional radiography and bone scintigraphy in the detection of the extent of involvement in the femoral head. Arthrography was as good as or better than MR imaging in determining the shape of the articular surfaces and the occurrence of lateral subluxation. Conventional radiography was less sensitive in identifying the degree of lateral subluxation and the extent of the necrosis in the femoral head. MR imaging provided anatomical and pathophysiological information about the extent and location of head involvement as well as the degree of lateral subluxation. Revascularisation was more clearly demonstrated with MR than with bone scintigraphy, irrespective of symptom duration. (orig.).

  11. Prevention and Control of Secondary Disease following Allogenic Bone-Marrow Transplantation

    International Nuclear Information System (INIS)

    A review is presented of the various methods found to be effective in preventing or ameliorating acute secondary disease in rodents and primates. Selective physical elimination of lymphoid cells by centrifugation over a discontinuous albumin gradient, post-transplantation administration of cyclophosphamide, amethopterin or ALS and the selection of histocompatible bone-marrow donors are considered to be the most promising methods in primares. It is not known to what extent delayed secondary disease will occur in monkeys and man in cases where acute secondary disease has been successfully avoided. (author)

  12. Increased serum IgE concentrations during infection and graft versus host disease after bone marrow transplantation.

    OpenAIRE

    Walker, S. A.; Rogers, T. R.; Perry, D; Hobbs, J R; Riches, P G

    1984-01-01

    Serum IgE concentrations estimated in 25 bone marrow transplant recipients during episodes of infection or graft versus host disease, or both, were raised not only in some patients with acute graft versus host disease but also in many patients with infection. Raised values were not seen in chronic graft versus host disease. The routine estimation of serum IgE in bone marrow transplant recipients had minimal value because of the lack of specificity of the IgE response.

  13. Bone Mineral Densitometry Findings of Children with Newly Diagnosed Celiac Disease

    Directory of Open Access Journals (Sweden)

    Tansel Ansal Balcı

    2011-08-01

    Full Text Available Objective: The effect of Celiac Disease (CD on children’s bone is the decrease in bone mineral density (BMD. Osteoporosis is a consequence of this decrease and usually manifests in adult ages. Studies in CD patients generally show that bone density of these patients can be different at the same ages for the same duration of disease. The aim of this study is to investigate the relationship between age and bone mineral density of CD patients at first diagnosis. Material and Methods: Ninety one patients (M/F: 36/55; age range: 3-16; mean age: 9.6±3.5 with diagnosis of CD were included in the study. BMD survey from L1-L4 lumbar spine and total hip of the patients was evaluated at presentation. We evaluated the patients in 3 groups according to their ages: Group 1: pre-school (3-7 years old, Group 2: elementary school (8-11 years old and Group 3: adolescent (12-16 years old. Results were compared using Student’s t test and correlation analysis. Results: The mean disease duration of the patients was 16.4±16.3 months. Mean height and weight of the patients were 124.8±17.9 cm and 27±9.3 kg, respectively and height and weight of 37 patients were in ≤ 3. percentile according to age. The BMD values of both lumbar spine and total hip and Z-scores of lumbar region were in mild correlation with age (r>0.5. There was significant difference between mean ages of patients with low bone mass for chronological age and normal bone densitometry values (p<0.05. There were 27, 36 and 28 patients in Group 1, Group 2 and Group 3, respectively. The difference between mean BMD values of these groups were statistically significant (p<0.05. The mean values of lumbar Z- scores of patients were -1.08±1.27, -1.42±1, -1.86±1.14, respectively for these three groups. Conclusion: Bone mineral densities of CD patients in childhood were lower in elder children at the time of diagnosis. This confirms the opinion that the diagnosis at earlier age results better treatment

  14. Low bone mineral density in men with chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    Bolton Charlotte E

    2011-08-01

    Full Text Available Abstract Background Osteoporosis is common in patients with COPD but the likely multi-factorial causes contributing to this condition (e.g. sex, age, smoking, therapy mask the potential contribution from elements related to COPD. In order to study osteoporosis and bone mineral density (BMD related to COPD, we studied a well-defined group of patients and controls. Methods BMD, forced expiratory volume in one second (FEV1, circulating bone biomarkers and biochemistry were determined in 30 clinically stable male ex-smokers with confirmed COPD and 15 age matched "ex-smoker" male controls. None of the patients were on inhaled corticosteroids or received more than one short course of steroids. Results Mean (SD FEV1% predicted of patients was 64(6%, the majority having Global Initiative for Chronic Obstructive Lung Disease (GOLD II airflow obstruction. There were 5/30 patients and 1/15 controls who were osteoporotic, while a further 17 patients and 5 controls were osteopenic. The BMD at the hip was lower in patients than controls, but not at the lumbar spine. Mean values of procollagen type 1 amino-terminal propeptide and osteocalcin, both markers of bone formation, and Type 1 collagen β C-telopeptide, a marker of bone resorption, were similar between patients and controls. However, all bone biomarkers were inversely related to hip BMD in patients (r = -0.51, r = -0.67, r = -0.57, p Conclusions Men with COPD had a greater prevalence of osteoporosis and osteopenia than age matched male controls, with a marked difference in BMD at the hip. Bone biomarkers suggest increased bone turnover.

  15. Effects of dietary phosphate on adynamic bone disease in rats with chronic kidney disease--role of sclerostin?

    Science.gov (United States)

    Ferreira, Juliana C; Ferrari, Guaraciaba O; Neves, Katia R; Cavallari, Raquel T; Dominguez, Wagner V; Dos Reis, Luciene M; Graciolli, Fabiana G; Oliveira, Elizabeth C; Liu, Shiguang; Sabbagh, Yves; Jorgetti, Vanda; Schiavi, Susan; Moysés, Rosa M A

    2013-01-01

    High phosphate intake is known to aggravate renal osteodystrophy along various pathogenetic pathways. Recent studies have raised the possibility that dysregulation of the osteocyte Wnt/β-catenin signaling pathway is also involved in chronic kidney disease (CKD)-related bone disease. We investigated the role of dietary phosphate and its possible interaction with this pathway in an experimental model of adynamic bone disease (ABD) in association with CKD and hypoparathyroidism. Partial nephrectomy (Nx) and total parathyroidectomy (PTx) were performed in male Wistar rats. Control rats with normal kidney and parathyroid function underwent sham operations. Rats were divided into three groups and underwent pair-feeding for 8 weeks with diets containing either 0.6% or 1.2% phosphate: sham 0.6%, Nx+PTx 0.6%, and Nx+PTx 1.2%. In the two Nx+PTx groups, serum creatinine increased and blood ionized calcium decreased compared with sham control group. They also presented hyperphosphatemia and reduced serum parathyroid hormone (PTH) and fibroblast growth factor 23 (FGF23) levels. Fractional urinary excretion of phosphate increased in Nx+PTx 1.2% rats despite lower PTH and FGF23 levels than in sham group. These biochemical changes were accompanied by a decrease in bone formation rates. The Nx+PTx 1.2% group had lower bone volume (BV/TV), higher osteoblast and osteocyte apoptosis, and higher SOST and Dickkopf-1 gene expression than the Nx+PTx 0.6% group. Nx+PTx 0.6% rat had very low serum sclerostin levels, and Nx+PTx 1.2% had intermediate sclerostin levels compared with sham group. Finally, there was a negative correlation between BV/TV and serum sclerostin. These results suggest that high dietary phosphate intake decreases bone volume in an experimental model of CKD-ABD, possibly via changes in SOST expression through a PTH-independent mechanism. These findings could have relevance for the clinical setting of CKD-ABD in patients who low turnover bone disease might be attenuated

  16. Effects of dietary phosphate on adynamic bone disease in rats with chronic kidney disease--role of sclerostin?

    Directory of Open Access Journals (Sweden)

    Juliana C Ferreira

    Full Text Available High phosphate intake is known to aggravate renal osteodystrophy along various pathogenetic pathways. Recent studies have raised the possibility that dysregulation of the osteocyte Wnt/β-catenin signaling pathway is also involved in chronic kidney disease (CKD-related bone disease. We investigated the role of dietary phosphate and its possible interaction with this pathway in an experimental model of adynamic bone disease (ABD in association with CKD and hypoparathyroidism. Partial nephrectomy (Nx and total parathyroidectomy (PTx were performed in male Wistar rats. Control rats with normal kidney and parathyroid function underwent sham operations. Rats were divided into three groups and underwent pair-feeding for 8 weeks with diets containing either 0.6% or 1.2% phosphate: sham 0.6%, Nx+PTx 0.6%, and Nx+PTx 1.2%. In the two Nx+PTx groups, serum creatinine increased and blood ionized calcium decreased compared with sham control group. They also presented hyperphosphatemia and reduced serum parathyroid hormone (PTH and fibroblast growth factor 23 (FGF23 levels. Fractional urinary excretion of phosphate increased in Nx+PTx 1.2% rats despite lower PTH and FGF23 levels than in sham group. These biochemical changes were accompanied by a decrease in bone formation rates. The Nx+PTx 1.2% group had lower bone volume (BV/TV, higher osteoblast and osteocyte apoptosis, and higher SOST and Dickkopf-1 gene expression than the Nx+PTx 0.6% group. Nx+PTx 0.6% rat had very low serum sclerostin levels, and Nx+PTx 1.2% had intermediate sclerostin levels compared with sham group. Finally, there was a negative correlation between BV/TV and serum sclerostin. These results suggest that high dietary phosphate intake decreases bone volume in an experimental model of CKD-ABD, possibly via changes in SOST expression through a PTH-independent mechanism. These findings could have relevance for the clinical setting of CKD-ABD in patients who low turnover bone disease might

  17. 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.

  18. On the way to building an integrated computational environment for the study of developmental patterns and genetic diseases

    OpenAIRE

    Turinsky, Andrei L.; Sensen, Christoph W.

    2006-01-01

    Genetic diseases and developmental patterns should be studied on several levels: from macroscale (organs and tissues) to nanoscale (cells, genes, proteins). Due to the overwhelming complexity of the life science data, it is common that disparate data pieces are meticulously stored but never fully analyzed or correlated. We have begun to develop a novel methodology based on virtual reality techniques for the study of these phenomena. Our key approach to knowledge integration is a top-down mapp...

  19. 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.

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

    Science.gov (United States)

    Mesentier-Louro, Louise A.; Zaverucha-do-Valle, Camila; Rosado-de-Castro, Paulo H.; Silva-Junior, Almir J.; Pimentel-Coelho, Pedro M.; Mendez-Otero, Rosalia; Santiago, Marcelo F.

    2016-01-01

    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. PMID:26649049

  1. 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 chro...

  2. 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.)

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

    International Nuclear Information System (INIS)

    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.)

  4. ACTINOMYCES NAESLUNDII ISOLATED FROM BONE MARROW BIOPSIES OBTAINED FROM PATIENTS WITH MALIGNANT LYMPHORETICULAR DISEASES

    Directory of Open Access Journals (Sweden)

    I.Mobedi

    1981-08-01

    Full Text Available Different types of Actinomyces spp isolated from the oral cavity of human in healthy as well as in pathological state. Internally it is only found in association with pathological organs. Recently Actinomyces naeslundii has been isolated from blood culture of a leukemic patient. Present studies indicate the association of this microorganism with cells obtained from bone marrow by punction in more than 40 percent of patients of patients with malignant lymphoreticular disease.

  5. ACTINOMYCES NAESLUNDII ISOLATED FROM BONE MARROW BIOPSIES OBTAINED FROM PATIENTS WITH MALIGNANT LYMPHORETICULAR DISEASES

    OpenAIRE

    Mobedi, I; J.Vandyoussefi; H.Rassa; HA.Fardin

    1981-01-01

    Different types of Actinomyces spp isolated from the oral cavity of human in healthy as well as in pathological state. Internally it is only found in association with pathological organs. Recently Actinomyces naeslundii has been isolated from blood culture of a leukemic patient. Present studies indicate the association of this microorganism with cells obtained from bone marrow by punction in more than 40 percent of patients of patients with malignant lymphoreticular disease.

  6. Case report 496: Intraosseous gas in Proteus mirabilis osteomyelitis complicating bone infarcts in sickle cell disease

    Energy Technology Data Exchange (ETDEWEB)

    Marx, A.C.; Hartshorne, M.F.; Stull, M.A.; Truwit, C.L.

    1988-10-01

    Plain film radiographs showed dramatic changes of this rare complication of sickle cell disease in an adult patient, eventually resulting in bilateral amputations of the lower extremities. Cross table radiographs of the knees showed the air-fluid levels without soft tissue swelling or periosteal reaction. The balance of the studies performed confirmed and refined the initial impression that infarction and infection had occurred within the medullary spaces of four long bones. The offending organism was Proteus mirabilis.

  7. Pulmonary, Gonadal, and Central Nervous System Status after Bone Marrow Transplantation for Sickle Cell Disease

    OpenAIRE

    Walters, Mark C.; Hardy, Karen; Edwards, Sandie; Adamkiewicz, Thomas; Barkovich, James; Bernaudin, Francoise; Buchanan, George R.; Bunin, Nancy; Dickerhoff, Roswitha; Giller, Roger; Haut, Paul R.; Horan, John; Hsu, Lewis L.; Kamani, Naynesh; Levine, John E.

    2009-01-01

    We conducted a prospective, multicenter investigation of human-leukocyte antigen (HLA) identical sibling bone marrow transplantation (BMT) in children with severe sickle cell disease (SCD) between 1991 and 2000. To determine if children were protected from complications of SCD after successful BMT, we extended our initial study of BMT for SCD to conduct assessments of the central nervous system (CNS) and of pulmonary function 2 or more years after transplantation. In addition, the impact on g...

  8. Bone Disease in Medullary Sponge Kidney and Effect of Potassium Citrate Treatment

    OpenAIRE

    Fabris, Antonia; Bernich, Patrizia; Abaterusso, Cataldo; Marchionna, Nicola; Canciani, Chiara; Nouvenne, Antonio; Zamboni, Mauro; Lupo, Antonio; Gambaro, Giovanni

    2009-01-01

    Background and objectives: In medullary sponge kidney (MSK)—a common malformative renal condition in patients with calcium nephrolithiasis—hypercalciuria, incomplete distal renal tubular acidosis, and hypocitraturia are common. Clinical conditions with concomitant hypercalciuria and/or incomplete distal renal tubular acidosis are almost invariably associated with bone disease, making osteopathy highly likely in MSK, too. Patients with MSK have never been investigated for osteopathy; neither h...

  9. Vanishing bone disease of the orbital roof: now you see it, now you don't

    International Nuclear Information System (INIS)

    We describe a rare case of vascularised orbital roof and calvarial erosions with an associated venous malformation. In the absence of infection, malignancy, trauma and eosinophillic granuloma, the closest previously described entity is vanishing bone disease. Computed tomography (CT), MRI, catheter angiography and pathology were all important in the diagnostic workup to enable surgical planning for biopsy and reconstruction. Ongoing CT and MRI follow-up imaging will determine future treatment planning.

  10. Stemness Evaluation of Mesenchymal Stem Cells from Placentas According to Developmental Stage: Comparison to Those from Adult Bone Marrow

    OpenAIRE

    Sung, Hwa Jung; Hong, Soon Cheol; Yoo, Ji Hyun; Oh, Jee Hyun; Shin, Hye Jin; Choi, In Young; Ahn, Ki Hoon; Kim, Sun Haeng; Park, Yong; Kim, Byung Soo

    2010-01-01

    This study was done to evaluate the stemness of human mesenchymal stem cells (hMSCs) derived from placenta according to the development stage and to compare the results to those from adult bone marrow (BM). Based on the source of hMSCs, three groups were defined: group I included term placentas, group II included first-trimester placentas, and group III included adult BM samples. The stemness was evaluated by the proliferation capacity, immunophenotypic expression, mesoderm differentiation, e...

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

    International Nuclear Information System (INIS)

    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.)

  12. Systemic mast cell disease (SMCD) and bone pain. A case treated with radiotherapy

    International Nuclear Information System (INIS)

    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.)

  13. 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.

  14. Multiple myeloma mesenchymal stromal cells: Contribution to myeloma bone disease and therapeutics

    Institute of Scientific and Technical Information of China (English)

    Antonio; Garcia-Gomez; Fermin; Sanchez-Guijo; M; Consuelo; del; Caizo; Jesus; F; San; Miguel; Mercedes; Garayoa

    2014-01-01

    Multiple myeloma is a hematological malignancy inwhich clonal plasma cells proliferate and accumulate within the bone marrow. The presence of osteolytic le-sions due to increased osteoclast(OC) activity and sup-pressed osteoblast(OB) function is characteristic of the disease. The bone marrow mesenchymal stromal cells(MSCs) play a critical role in multiple myeloma patho-physiology, greatly promoting the growth, survival, drug resistance and migration of myeloma cells. Here, we specifically discuss on the relative contribution of MSCs to the pathophysiology of osteolytic lesions in light of the current knowledge of the biology of my-eloma bone disease(MBD), together with the reported genomic, functional and gene expression differences between MSCs derived from myeloma patients(pMSCs) and their healthy counterparts(dMSCs). Being MSCs the progenitors of OBs, pMSCs primarily contribute to the pathogenesis of MBD because of their reduced osteogenic potential consequence of multiple OB inhibi-tory factors and direct interactions with myeloma cells in the bone marrow. Importantly, pMSCs also readily contribute to MBD by promoting OC formation and ac-tivity at various levels(i.e., increasing RANKL to OPG expression, augmenting secretion of activin A, uncou-pling ephrinB2-EphB4 signaling, and through augment-ed production of Wnt5a), thus further contributing to OB/OC uncoupling in osteolytic lesions. In this review, we also look over main signaling pathways involved in the osteogenic differentiation of MSCs and/or OB activity, highlighting amenable therapeutic targets; in parallel, the reported activity of bone-anabolic agents(at preclinical or clinical stage) targeting those signaling pathways is commented.

  15. Multiple myeloma mesenchymal stromal cells: Contribution to myeloma bone disease and therapeutics.

    Science.gov (United States)

    Garcia-Gomez, Antonio; Sanchez-Guijo, Fermin; Del Cañizo, M Consuelo; San Miguel, Jesus F; Garayoa, Mercedes

    2014-07-26

    Multiple myeloma is a hematological malignancy in which clonal plasma cells proliferate and accumulate within the bone marrow. The presence of osteolytic lesions due to increased osteoclast (OC) activity and suppressed osteoblast (OB) function is characteristic of the disease. The bone marrow mesenchymal stromal cells (MSCs) play a critical role in multiple myeloma pathophysiology, greatly promoting the growth, survival, drug resistance and migration of myeloma cells. Here, we specifically discuss on the relative contribution of MSCs to the pathophysiology of osteolytic lesions in light of the current knowledge of the biology of myeloma bone disease (MBD), together with the reported genomic, functional and gene expression differences between MSCs derived from myeloma patients (pMSCs) and their healthy counterparts (dMSCs). Being MSCs the progenitors of OBs, pMSCs primarily contribute to the pathogenesis of MBD because of their reduced osteogenic potential consequence of multiple OB inhibitory factors and direct interactions with myeloma cells in the bone marrow. Importantly, pMSCs also readily contribute to MBD by promoting OC formation and activity at various levels (i.e., increasing RANKL to OPG expression, augmenting secretion of activin A, uncoupling ephrinB2-EphB4 signaling, and through augmented production of Wnt5a), thus further contributing to OB/OC uncoupling in osteolytic lesions. In this review, we also look over main signaling pathways involved in the osteogenic differentiation of MSCs and/or OB activity, highlighting amenable therapeutic targets; in parallel, the reported activity of bone-anabolic agents (at preclinical or clinical stage) targeting those signaling pathways is commented.

  16. Kidney bone disease and mortality in CKD: revisiting the role of vitamin D, calcimimetics, alkaline phosphatase, and minerals.

    Science.gov (United States)

    Kalantar-Zadeh, Kamyar; Shah, Anuja; Duong, Uyen; Hechter, Rulin C; Dukkipati, Ramanath; Kovesdy, Csaba P

    2010-08-01

    Recent evidence suggests that the traditional syndromes known as renal osteodystrophy, secondary hyperparathyroidism, and vitamin D deficiency are related to mortality in persons with moderate to advanced chronic kidney disease (CKD). The so-called 'kidney bone disease', also known as 'mineral and bone disorders', is defined to include bone disorders, mineral disarrays, and vascular calcification. We have identified 14 common and clinically relevant conditions of contemporary nature that are related to the kidney bone disease, including calcitriol (active vitamin D) deficiency, 25(OH)-vitamin D deficiency, biochemical hyperparathyroidism, relatively low parathyroid hormone (PTH) level, increased serum alkaline phosphatase (hyperphosphatasemia), elevated fibroblast growth factor (FGF)-23, high turnover bone disease, adynamic bone disease, uremic osteoporosis, vascular calcification, hyper- and hypophosphatemia, and hyper- and hypocalcemia. We present a critical review of these 14 conditions with emphasis on patient survival and other pertinent clinical outcomes. We also review unresolved controversies surrounding the management of these conditions by administration of nutritional vitamin D (ergocalciferol and cholecalciferol), vitamin D receptor activators (calcitriol, alphacalcidiol, doxercalciferol), D-mimetics (paricalcitol, maxacalcitol), calcimimetics (cinacalcet), recombinant PTH (teriparatide), and receptor activator of nuclear factor-kappaB ligand modulators (denosumab); compare mortality predictability of PTH and alkaline phosphatase; and examine potential risks of bone disorders and mineral disarrays in CKD patients. PMID:20671739

  17. Dynamic contrast-enhanced MRI in Paget's disease of bone-correlation of regional microcirculation and bone turnover

    Energy Technology Data Exchange (ETDEWEB)

    Libicher, M. [University of Cologne, Department of Radiology, Cologne (Germany); Klinikum der Universitaet zu Koeln, Radiologische Klinik, Koeln (Germany); Kasperk, C. [University of Heidelberg, Department of Medicine, Division of Osteology, Heidelberg (Germany); Daniels, M.; Hosch, W. [University of Heidelberg, Department of Radiology, Heidelberg (Germany); Kauczor, H.U.; Delorme, S. [German Cancer Research Center, Department of Radiology, Heidelberg (Germany)

    2008-05-15

    The purpose of this study was to evaluate regional microcirculation in Paget's disease of bone (PD) with dynamic contrast-enhanced MR imaging (DCE-MRI). Additionally, we correlated regional bone perfusion with alkaline phosphatase as serum marker of bone turnover. We examined 71 patients with PD (27 men, 44 women, 67{+-}10 years) localized at the axial and appendicular skeleton. Contrast uptake was analyzed using a two-compartment model with the output variables amplitude A and exchange rate constant k{sub ep}. Color-coded parametric images were generated to visualize microcirculation. Serum levels of alkaline phosphatase (AP) were compared with DCE-MRI parameters. Amplitude A and exchange rate constant k{sub ep} were significantly increased in PD compared to unaffected bone (A{sub PD} 0.81{+-}0.24 vs. A{sub control} 0.34{+-}0.1 and k{sub ep} {sub PD} 4.0 {+-}2.86 vs. k{sub ep} {sub control} 1.73 {+-}0.88, p <0.001). There was a significant correlation (r{sub s}=0.5-0.7) of DCE-MRI parameters and AP at the axial (pelvis, spine) and appendicular skeleton (femur, tibia). The long bones showed increased circulation of the advancing peripheral zones and no vascularization of the central part, which had been replaced by fatty tissue. Regional microcirculation in PD is inhomogeneous with focal areas of excessive hypervascularity, especially in the advancing peripheral zone. There is a significant correlation of bone circulation and bone turnover in PD. DCE-MRI might therefore be a diagnostic tool for monitoring therapeutic effects of bisphoshonates in Paget's disease of bone. (orig.)

  18. Is chronic kidney disease-mineral bone disorder (CKD-MBD) really a syndrome?

    Science.gov (United States)

    Cozzolino, Mario; Ureña-Torres, Pablo; Vervloet, Marc G; Brandenburg, Vincent; Bover, Jordi; Goldsmith, David; Larsson, Tobias E; Massy, Ziad A; Mazzaferro, Sandro

    2014-10-01

    The concept of chronic kidney disease-mineral bone disorder (CKD-MBD) does not appear to fulfil the requirements for a syndrome at first glance, but its definition has brought some clear-cut benefits for clinicians and patients, including wider and more complex diagnostic and therapeutic approaches to the management of this challenging set of issues. Admittedly, not all components of CKD-MBD are present in all patients at all times, but these are highly interrelated, involving mineral and bone laboratory abnormalities, clinical and histological bone disease and finally, cardiovascular disease. The presence of typical biological bone ossification processes in an ectopic anatomical location in CKD has helped to define the existence of an unprecedented bone-vascular relationship, extending its interest even to other medical specialities. For now, we believe that CKD-MBD does not reach full criteria to be defined as a syndrome. However, this novel concept has clearly influenced current clinical guidelines. The National Kidney Foundation Kidney Disease Outcomes Quality Initiative (NKF/KDOQI™) guidelines in 2003 for instance recommended that calcium-based phosphate binders should be avoided to treat hyperphosphataemia in the presence of cardiovascular calcifications. In 2009, the KDIGO and other guidelines reinforced and extended this recommendation by stating that it is reasonable to choose oral phosphate binder therapy by taking into consideration other components of CKD-MBD. Similarly, it is also considered reasonable to use information on vascular/valvular calcification to guide the management of CKD-MBD. Our current assumption as a working group 'CKD-MBD' is that CKD-MBD has the potential to be defined a true syndrome, such as a constellation of concurrent signs and symptoms that suggest a common underlying mechanism for these components as opposed to the term disease. The term 'syndrome' also implies that in any patient at risk due to the presence of one or a few

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

    Energy Technology Data Exchange (ETDEWEB)

    Juhng, Seon Kwan [Wonkwang Univ. School of Medicine, Iksan (Korea, Republic of); Koplyay, Peter; Jeffrey Carr, J.; Lenchik, Leon [Wake Forest Univ. School of Medicine, Winston-salem (United States)

    2001-04-01

    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{sup 2} in the femoral neck, while for controls the corresponding figures were 0.989g/cm{sup 2} and 0.765g/cm{sup 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.

  20. 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

    2016-01-01

    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. PMID:27417633

  1. [Limb torsion and developmental regression for one month after hand, foot and mouth disease in an infant].

    Science.gov (United States)

    Feng, Li-Fang; Chen, Xiao-Hong; Li, Dong-Xiao; Ding, Yuan; Jin, Ying; Song, Jin-Qing; Yang, Yan-Ling

    2016-05-01

    A one-year-old girl visited the hospital due to limb torsion and developmental regression for one month after hand, foot and mouth disease. At the age of 11 months, she visited a local hospital due to fever for 5 days and skin rash with frequent convulsions for 2 days and was diagnosed with severe hand, foot and mouth disease, viral encephalitis, and status epilepticus. Brain MRI revealed symmetric abnormal signals in the bilateral basal ganglia, bilateral thalamus, cerebral peduncle, bilateral cortex, and hippocampus. She was given immunoglobulin, antiviral drugs, and anticonvulsant drugs for 2 weeks, and the effect was poor. Blood and urine screening for inherited metabolic diseases were performed to clarify the etiology. The analysis of urine organic acids showed significant increases in glutaric acid and 3-hydroxyglutaric acid, which suggested glutaric aciduria type 1, but her blood glutarylcarnitine was normal, and free carnitine significantly decreased. After the treatment with low-lysine diets, L-carnitine, and baclofen for 1 month, the patient showed a significant improvement in symptoms. Hand, foot and mouth disease is a common viral infectious disease in children, and children with underlying diseases such as inherited metabolic diseases and immunodeficiency may experience serious complications. For children with hand, foot and mouth disease and unexplained encephalopathy, inherited metabolic diseases should be considered. PMID:27165592

  2. Molecular Characterization of Three Canine Models of Human Rare Bone Diseases: Caffey, van den Ende-Gupta, and Raine Syndromes

    OpenAIRE

    Hytönen, Marjo K.; Meharji Arumilli; Lappalainen, Anu K; Marta Owczarek-Lipska; Vidhya Jagannathan; Sruthi Hundi; Elina Salmela; Patrick Venta; Eva Sarkiala; Tarja Jokinen; Daniela Gorgas; Juha Kere; Pekka Nieminen; Cord Drögemüller; Hannes Lohi

    2016-01-01

    One to two percent of all children are born with a developmental disorder requiring pediatric hospital admissions. For many such syndromes, the molecular pathogenesis remains poorly characterized. Parallel developmental disorders in other species could provide complementary models for human rare diseases by uncovering new candidate genes, improving the understanding of the molecular mechanisms and opening possibilities for therapeutic trials. We performed various experiments, e.g. combined ge...

  3. Molecular Characterization of Three Canine Models of Human Rare Bone Diseases: Caffey, van den Ende-Gupta, and Raine Syndromes.

    OpenAIRE

    Hytönen, Marjo K.; Arumilli, Meharji; Lappalainen, Anu K; Owczarek, Marta; Jagannathan, Vidhya; Hundi, Sruthi; Salmela, Elina; Venta, Patrick; Sarkiala, Eva; Jokinen, Tarja; Gorgas, Daniela; Kere, Juha; Nieminen, Pekka; Drögemüller, Cord; Lohi, Hannes

    2016-01-01

    One to two percent of all children are born with a developmental disorder requiring pediatric hospital admissions. For many such syndromes, the molecular pathogenesis remains poorly characterized. Parallel developmental disorders in other species could provide complementary models for human rare diseases by uncovering new candidate genes, improving the understanding of the molecular mechanisms and opening possibilities for therapeutic trials. We performed various experiments, e.g. combined ge...

  4. 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. PMID:26173293

  5. Seeking genes responsible for developmental origins of health and disease from the fetal mouse liver following maternal food restriction.

    Science.gov (United States)

    Ogawa, Tetsuo; Shibato, Junko; Rakwal, Randeep; Saito, Tomomi; Tamura, Gaku; Kuwagata, Makiko; Shioda, Seiji

    2014-11-01

    Low birthweight resulting from a non-optimal fetal environment is correlated epidemiologically to a higher risk of adult diseases, and which has also been demonstrated using animal models for maternal undernutrition. In this study, we subjected pregnant mice to 50% food restriction (FR), and profiled gene expression and promoter DNA methylation genome-wide using the fetal livers. The fact that effect of food restriction is opposite between before and after birth encouraged us to hunt for genes that are expressed oppositely to adult calorie restriction (CR) using the maternal livers. Among oppositely regulated genes, we identified trib1 (tribbles homolog 1). Using genetically modified mice, trib1 has been shown to have a demonstrable contribution to a risk of hypertriglyceridaemia and insulin resistance. Our data showed that the trib1 expression and its promoter DNA methylation could be affected physiologically (by maternal nutrition), and therefore might be a strong candidate gene for developmental origins of adult diseases. Furthermore, lepr (leptin receptor) gene was downregulated by maternal FR, indicating its potential role in induction of obesity and diabetes. Gene expression as well as promoter DNA methylation profiling revealed that glucocorticoid receptor target genes were regulated by maternal FR. This supports previous studies that suggest an important role of fetal glucocorticoid exposure in the mechanism of developmental origins of diseases. Our transcriptomics profiling data also suggested that maternal FR impaired development of the immune system. An inventory of candidate genes responsible for developmental origins of health and disease is presented and discussed in this study. PMID:24754856

  6. Bone mineral density and disorders of mineral metabolism in chronic liver disease

    Institute of Scientific and Technical Information of China (English)

    Joe George; Hosahithlu K Ganesh; Shrikrishna Acharya; Tushar R Bandgar; Vyankatesh Shivane; Anjana Karvat; Shobna J Bhatia; Samir Shah; Padmavathy S Menon; Nalini Shah

    2009-01-01

    AIM: To estimate the prevalence and identify the risk factors for metabolic bone disease in patients with cirrhosis. METHODS: The study was performed on 72 Indian patients with cirrhosis (63 male, 9 female; aged < 50 years). Etiology of cirrhosis was alcoholism ( n = 37), hepatitis B ( n = 25) and hepatitis C ( n = 10). Twenty-three patients belonged to Child class A, while 39 were in class B and 10 in class C. Secondary causes for metabolic bone disease and osteoporosis were ruled out. Sunlight exposure, physical activity and dietary constituents were calculated. Complete metabolic profiles were derived, and bone mineral density (BMD) was measured using dual energy X ray absorptiometry. Low BMD was defined as a Z score below -2. RESULTS: Low BMD was found in 68% of patients. Lumbar spine was the most frequently and severely affected site. Risk factors for low BMD included low physical activity, decreased sunlight exposure, and low lean body mass. Calcium intake was adequate, with unfavorable calcium: protein ratio and calcium: phosphorus ratio. Vitamin D deficiency was highly prevalent (92%). There was a high incidence of hypogonadism (41%). Serum estradiol level was elevated significantly in patients with normal BMD. Insulin-like growth factor (IGF) 1 and IGF binding protein 3 levels were below the age-related normal range in both groups. IGF-1 was significantly lower in patients with low BMD. Serum osteocalcin level was low (68%) and urinary deoxypyridinoline to creatinine ratio was high (79%), which demonstrated low bone formation with high resorption. CONCLUSION: Patients with cirrhosis have low BMD. Contributory factors are reduced physical activity, low lean body mass, vitamin D deficiency and hypogonadism and low IGF-1 level.

  7. Extent of Surgery Does Not Influence 30-Day Mortality in Surgery for Metastatic Bone Disease

    Science.gov (United States)

    Sørensen, Michala Skovlund; Hindsø, Klaus; Hovgaard, Thea Bechmann; Petersen, Michael Mørk

    2016-01-01

    Abstract Estimating patient survival has hitherto been the main focus when treating metastatic bone disease (MBD) in the appendicular skeleton. This has been done in an attempt to allocate the patient to a surgical procedure that outlives them. No questions have been addressed as to whether the extent of the surgery and thus the surgical trauma reduces survival in this patient group. We wanted to evaluate if perioperative parameters such as blood loss, extent of bone resection, and duration of surgery were risk factors for 30-day mortality in patients having surgery due to MBD in the appendicular skeleton. We retrospectively identified 270 consecutive patients who underwent joint replacement surgery or intercalary spacing for skeletal metastases in the appendicular skeleton from January 1, 2003 to December 31, 2013. We collected intraoperative (duration of surgery, extent of bone resection, and blood loss), demographic (age, gender, American Society of Anesthesiologist score [ASA score], and Karnofsky score), and disease-specific (primary cancer) variables. An association with 30-day mortality was addressed using univariate and multivariable analyses and calculation of odds ratio (OR). All patients were included in the analysis. ASA score 3 + 4 (OR 4.16 [95% confidence interval, CI, 1.80–10.85], P = 0.002) and Karnofsky performance status below 70 (OR 7.34 [95% CI 3.16–19.20], P < 0.001) were associated with increased 30-day mortality in univariate analysis. This did not change in multivariable analysis. No parameters describing the extent of the surgical trauma were found to be associated with 30-day mortality. The 30-day mortality in patients undergoing surgery for MBD is highly dependent on the general health status of the patients as measured by the ASA score and the Karnofsky performance status. The extent of surgery, measured as duration of surgery, blood loss, and degree of bone resection were not associated with 30-day mortality. PMID:27082592

  8. Soy isoflavones avert chronic inflammation-induced bone loss and vascular disease

    Directory of Open Access Journals (Sweden)

    Lightfoot Stan A

    2007-09-01

    Full Text Available Abstract Background Evidence from epidemiological, clinical and animal studies suggests a link may exist between low bone density and cardiovascular disease, with inflammatory mediators implicated in the pathophysiology of both conditions. This project examined whether supplementation with soy isoflavones (IF, shown to have anti-inflammatory properties, could prevent tissue expression of TNF-α and the development of skeletal pathology in an animal model of chronic inflammation. Methods Eight-week old, intact, female C57BL/6J mice were used. In Phase 1, a lipopolysaccharide (LPS-dose response study (0, 0.133, 1.33 and 13.3 μg/d was conducted to determine the LPS dose to use in Phase 2. The results indicated the 1.33 μg LPS/d dose produced the greatest decrease in lymphocytes and increase in neutrophils. Subsequently, in Phase 2, mice were randomly assigned to one of six groups (n = 12–13 per group: 0 or 1.33 μg LPS/d (placebo or LPS in combination with 0, 126 or 504 mg aglycone equivalents of soy IF/kg diet (Control, Low or High dose IF. Mice were fed IF beginning 2 wks prior to the 30-d LPS study period. Results At the end of the study, no differences were detected in final body weights or uterine weights. In terms of trabecular bone microarchitecture, μCT analyses of the distal femur metaphysis indicated that LPS significantly decreased trabecular bone volume (BV/TV and number (TbN, and increased separation (TbSp. Trabecular bone strength (i.e. total force and stiffness were also compromised in response to LPS. The High IF dose provided protection against these detrimental effects on microarchitecture, but not biomechanical properties. No alterations in trabecular thickness (TbTh, or cortical bone parameters were observed in response to the LPS or IF. Immunohistomchemical staining showed that tumor necrosis factor (TNF-α was up-regulated by LPS in the endothelium of small myocardial arteries and arterioles as well as the tibial

  9. Overexpression of RANKL in osteoblasts: a possible mechanism of susceptibility to bone disease in cystic fibrosis.

    Science.gov (United States)

    Delion, Martial; Braux, Julien; Jourdain, Marie-Laure; Guillaume, Christine; Bour, Camille; Gangloff, Sophie; Pimpec-Barthes, Françoise Le; Sermet-Gaudelus, Isabelle; Jacquot, Jacky; Velard, Frédéric

    2016-09-01

    Bone fragility and loss are a significant cause of morbidity in patients with cystic fibrosis (CF), and the lack of effective therapeutic options means that treatment is more often palliative rather than curative. A deeper understanding of the pathogenesis of CF-related bone disease (CFBD) is necessary to develop new therapies. Defective CF transmembrane conductance regulator (CFTR) protein and chronic inflammation in bone are important components of the CFBD development. The receptor activator of nuclear factor kappa-B ligand (RANKL) and osteoprotegerin (OPG) drive the regulation of bone turnover. To investigate their roles in CFBD, we evaluated the involvement of defective CFTR in their production level in CF primary human osteoblasts with and without inflammatory stimulation, in the presence or not of pharmacological correctors of the CFTR. No major difference in cell ultrastructure was noted between cultured CF and non-CF osteoblasts, but a delayed bone matrix mineralization was observed in CF osteoblasts. Strikingly, resting CF osteoblasts exhibited strong production of RANKL protein, which was highly localized at the cell membrane and was enhanced in TNF (TNF-α) or IL-17-stimulated conditions. Under TNF stimulation, a defective response in OPG production was observed in CF osteoblasts in contrast to the elevated OPG production of non-CF osteoblasts, leading to an elevated RANKL-to-OPG protein ratio in CF osteoblasts. Pharmacological inhibition of CFTR chloride channel conductance in non-CF osteoblasts replicated both the decreased OPG production and the enhanced RANKL-to-OPG ratio. Interestingly, using CFTR correctors such as C18, we significantly reduced the production of RANKL by CF osteoblasts, in both resting and TNF-stimulated conditions. In conclusion, the overexpression of RANKL and high membranous RANKL localization in osteoblasts are related to defective CFTR, and may worsen bone resorption, leading to bone loss in patients with CF. Targeting

  10. 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

  11. The Biomechanical Testing for the Assessment of Bone Quality in an Experimental Model of Chronic Kidney Disease.

    Science.gov (United States)

    Oksztulska-Kolanek, Ewa; Znorko, Beata; Michałowska, Małgorzata; Pawlak, Krystyna

    2016-01-01

    Mineral metabolism disturbances are common in chronic kidney disease (CKD) and have been classified as a new clinical entity, also known as CKD-mineral and bone disorders (CKD-MBD). A decrease in the bone strength, whose clinical manifestation is a tendency for fracture, has been recognized as an important component of CKD-MBD. Because of ethical issues, measurements of the bone strength in the human body are usually limited to noninvasive techniques, such as radiography, dual-energy X-ray absorptiometry and the assays of bone turnover biomarkers. However, it has been postulated recently that the evidence concerning bone strength based solely on the determination of the bone quantity may be insufficient and that bone quality should also be examined. In this regard, an animal model of CKD can represent an experimental tool to test the effectiveness of new therapeutic strategies. Despite the many available methods that are used to diagnose metabolic bone disorders and predict fracture risk especially in small rodents with CKD, it turns out that the most appropriate are biomechanical tests, which can provide information about the structural and material properties of bone. The present review summarizes and discusses the principles for carrying out selected biomechanical tests (3-point bending test and compression test) and their application in clinical practice. PMID:26680019

  12. Bone marrow transplantation in the prevention of intellectual disability due to inherited metabolic disease: ethical issues.

    Science.gov (United States)

    Louhiala, P

    2009-07-01

    Many inherited metabolic diseases may lead to varying degrees of brain damage and thus also to intellectual disability. Bone marrow transplantation (BMT) has been used for over two decades as a form of secondary prevention to stop or reverse the progress of the disease process in some of these conditions. At the population level the impact of BMT on the prevalence of intellectual disability is minute, but at the individual level its impact on the prognosis of the disease and the well-being of the patient can be substantial. The dark side of BMT use is the burden of side effects, complications and transplantation-related mortality in less successful cases. The ethical issues involved in this therapy are discussed in this review. PMID:19567689

  13. 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.

  14. The effect of naturally occurring chronic kidney disease on the micro-structural and mechanical properties of bone.

    Directory of Open Access Journals (Sweden)

    Anna Shipov

    Full Text Available Chronic kidney disease (CKD is a growing public health concern worldwide, and is associated with marked increase of bone fragility. Previous studies assessing the effect of CKD on bone quality were based on biopsies from human patients or on laboratory animal models. Such studies provide information of limited relevance due to the small size of the samples (biopsies or the non-physiologic CKD syndrome studied (rodent models with artificially induced CKD. Furthermore, the type, architecture, structure and biology of the bone of rodents are remarkably different from human bones; therefore similar clinicopathologic circumstances may affect their bones differently. We describe the effects of naturally occurring CKD with features resembling human CKD on the skeleton of cats, whose bone biology, structure and composition are remarkably similar to those of humans. We show that CKD causes significant increase of resorption cavity density compared with healthy controls, as well as significantly lower cortical mineral density, cortical cross-sectional area and cortical cross-sectional thickness. Young's modulus, yield stress, and ultimate stress of the cortical bone material were all significantly decreased in the skeleton of CKD cats. Cancellous bone was also affected, having significantly lower trabecular thickness and bone volume over total volume in CKD cats compared with controls. This study shows that naturally occurring CKD has deleterious effects on bone quality and strength. Since many similarities exist between human and feline CKD patients, including the clinicopathologic features of the syndrome and bone microarchitecture and biology, these results contribute to better understanding of bone abnormalities associated with CKD.

  15. Inhibition of autoimmune Chagas-like heart disease by bone marrow transplantation.

    Directory of Open Access Journals (Sweden)

    Maria C Guimaro

    2014-12-01

    Full Text Available Infection with the protozoan Trypanosoma cruzi manifests in mammals as Chagas heart disease. The treatment available for chagasic cardiomyopathy is unsatisfactory.To study the disease pathology and its inhibition, we employed a syngeneic chicken model refractory to T. cruzi in which chickens hatched from T. cruzi inoculated eggs retained parasite kDNA (1.4 kb minicircles. Southern blotting with EcoRI genomic DNA digests revealed main 18 and 20 kb bands by hybridization with a radiolabeled minicircle sequence. Breeding these chickens generated kDNA-mutated F1, F2, and F3 progeny. A targeted-primer TAIL-PCR (tpTAIL-PCR technique was employed to detect the kDNA integrations. Histocompatible reporter heart grafts were used to detect ongoing inflammatory cardiomyopathy in kDNA-mutated chickens. Fluorochromes were used to label bone marrow CD3+, CD28+, and CD45+ precursors of the thymus-dependent CD8α+ and CD8β+ effector cells that expressed TCRγδ, vβ1 and vβ2 receptors, which infiltrated the adult hearts and the reporter heart grafts.Genome modifications in kDNA-mutated chickens can be associated with disruption of immune tolerance to compatible heart grafts and with rejection of the adult host's heart and reporter graft, as well as tissue destruction by effector lymphocytes. Autoimmune heart rejection was largely observed in chickens with kDNA mutations in retrotransposons and in coding genes with roles in cell structure, metabolism, growth, and differentiation. Moreover, killing the sick kDNA-mutated bone marrow cells with cytostatic and anti-folate drugs and transplanting healthy marrow cells inhibited heart rejection. We report here for the first time that healthy bone marrow cells inhibited heart pathology in kDNA+ chickens and thus prevented the genetically driven clinical manifestations of the disease.

  16. 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.

  17. Early life environment and developmental immunotoxicity in inflammatory dysfunction and disease

    OpenAIRE

    Leifer, Cynthia A.; Dietert, Rodney R.

    2011-01-01

    Components of the innate immune system such as macrophages and dendritic cells are instrumental in determining the fate of immune responses and are, also, among the most sensitive targets of early life environmental alterations including developmental immunotoxicity (DIT). DIT can impede innate immune cell maturation, disrupt tissue microenvironment, alter immune responses to infectious challenges, and disrupt regulatory responses. Dysregulation of inflammation, such as that observed with DIT...

  18. Sex differences in the developmental origins of hypertension and cardiorenal disease

    OpenAIRE

    Gilbert, Jeffrey S; Nijland, Mark J.

    2008-01-01

    The “developmental origins of health and disease” (DOHAD) hypothesis derives from clinical observations, indicating long-term health consequences for persons of low birth weight. There is growing evidence, primarily from animal studies, that supports the idea that processes put in motion during development that contribute to DOHAD do not necessarily reflect as significantly compromised growth and altered birth weight. Throughout the body of work investigating the DOHAD hypothesis, several the...

  19. Higher bone turnover is related to spinal radiographic damage and low bone mineral density in ankylosing spondylitis patients with active disease: a cross-sectional analysis.

    Directory of Open Access Journals (Sweden)

    Suzanne Arends

    Full Text Available INTRODUCTION: Ankylosing spondylitis (AS is characterized by excessive bone formation and bone loss. Our aim was to investigate the association of bone turnover markers (BTM with spinal radiographic damage and bone mineral density (BMD in AS patients with active disease. METHODS: 201 consecutive AS outpatients of the Groningen Leeuwarden AS (GLAS cohort were included. Serum markers of bone resorption (C-telopeptides of type-I collagen, sCTX and bone formation (procollagen type-I N-terminal peptide, PINP; bone-specific alkaline phosphatase, BALP were measured. Z-scores were used to correct for the normal influence that age and gender have on bone turnover. Radiographs were scored by two independent readers according to modified Stoke AS Spinal Score (mSASSS. The presence of complete bridging (ankylosis of at least two vertebrae was considered as measure of more advanced radiographic damage. Low BMD was defined as lumbar spine and/or hip BMD Z-score ≤ -1. RESULTS: Of the 151 patients with complete data, 52 (34% had ≥ 1 complete bridge, 49 (33% had ≥ 1 syndesmophyte (non-bridging, and 50 (33% had no syndesmophytes. 66 (44% had low BMD. Patients with bridging had significantly higher sCTX and PINP Z-scores than patients without bridging (0.43 vs. -0.55 and 0.55 vs. 0.04, respectively. Patients with low BMD had significantly higher sCTX Z-score than patients with normal BMD (-0.08 vs. -0.61. After correcting for gender, symptom duration, and CRP, sCTX Z-score remained significantly related to the presence of low BMD alone (OR: 1.60, bridging alone (OR: 1.82, and bridging in combination with low BMD (OR: 2.26. CONCLUSIONS: This cross-sectional study in AS patients with active and relatively long-standing disease demonstrated that higher serum levels of sCTX, and to a lesser extent PINP, are associated with the presence of complete bridging. sCTX was also associated with low BMD. Longitudinal studies are needed to confirm that serum levels of s

  20. Skeletal muscle fiber type: using insights from muscle developmental biology to dissect targets for susceptibility and resistance to muscle disease.

    Science.gov (United States)

    Talbot, Jared; Maves, Lisa

    2016-07-01

    Skeletal muscle fibers are classified into fiber types, in particular, slow twitch versus fast twitch. Muscle fiber types are generally defined by the particular myosin heavy chain isoforms that they express, but many other components contribute to a fiber's physiological characteristics. Skeletal muscle fiber type can have a profound impact on muscle diseases, including certain muscular dystrophies and sarcopenia, the aging-induced loss of muscle mass and strength. These findings suggest that some muscle diseases may be treated by shifting fiber type characteristics either from slow to fast, or fast to slow phenotypes, depending on the disease. Recent studies have begun to address which components of muscle fiber types mediate their susceptibility or resistance to muscle disease. However, for many diseases it remains largely unclear why certain fiber types are affected. A substantial body of work has revealed molecular pathways that regulate muscle fiber type plasticity and early developmental muscle fiber identity. For instance, recent studies have revealed many factors that regulate muscle fiber type through modulating the activity of the muscle regulatory transcription factor MYOD1. Future studies of muscle fiber type development in animal models will continue to enhance our understanding of factors and pathways that may provide therapeutic targets to treat muscle diseases. WIREs Dev Biol 2016, 5:518-534. doi: 10.1002/wdev.230 For further resources related to this article, please visit the WIREs website. PMID:27199166

  1. 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.

  2. 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.

  3. Patterns of bone diseases in transfusion-dependent homozygous thalassaemia major: predominance of osteoporosis and desferrioxamine-induced bone dysplasia

    International Nuclear Information System (INIS)

    Objective: To study the radiographic skeletal changes in transfusion-dependent homozygous β-thalassaemia. Materials and methods: This was a retrospective review of radiographs of 41 homozygous β-thalassaemic patients over 3 years. These included 55 left hand radiographs for bone age, 37 chest radiographs, 7 scanograms of lower limbs, 8 knee radiographs and 3 skull radiographs. The radiographs were evaluated for the skeletal changes owing to medullary expansion, as well as for the skeletal dysplasia related to desferrioxamine therapy. The combined cortical width of the mid shaft of the second metacarpal was measured on left hand radiographs to assess osteoporosis. Results: Sixteen patients had radiographic evidence of desferrioxamine-induced bone dysplasia. These included metaphyseal sclerosis in long bone (n=16), irregular sclerosis at the costochondral junction (n=3) and platyspondyly (n= 1). Two patients had radiographic evidence of medullary expansion with widening of medulla and marked thinning of cortex in the tubular bones. Osteoporosis, as indicated by thinning of metacarpal cortex, was noted in 17 patients (8 with and 9 without desferrioxamine-induced bone dysplasia). Conclusions: With provision of the modern regime of regular transfusion and desferrioxamine chelation, desferrioxamine-induced bone dysplasia was a much more frequently detected radiographic abnormality in β-thalassaemia major than radiographic features owing to medullary expansion. Osteoporosis, as indicated by thinned metacarpal cortices, remained a frequent feature irrespective of the status of the skeletal dysplasia. (orig.)

  4. Patterns of bone diseases in transfusion-dependent homozygous thalassaemia major: predominance of osteoporosis and desferrioxamine-induced bone dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Chan, Yu-Leung; Pang, Lai-Man [Department of Diagnostic Radiology and Organ Imaging, Prince of Wales Hospital, Shatin (Hong Kong); Chik, Ki-Wai; Li, Chi-Kong [Department of Paediatrics, Prince of Wales Hospital, Chinese University of Hong Kong (Hong Kong); Cheng, Jack C.Y. [Department Orthopaedics and Traumatology, Prince of Wales Hospital, Chinese University of Hong Kong (Hong Kong)

    2002-07-01

    Objective: To study the radiographic skeletal changes in transfusion-dependent homozygous {beta}-thalassaemia. Materials and methods: This was a retrospective review of radiographs of 41 homozygous {beta}-thalassaemic patients over 3 years. These included 55 left hand radiographs for bone age, 37 chest radiographs, 7 scanograms of lower limbs, 8 knee radiographs and 3 skull radiographs. The radiographs were evaluated for the skeletal changes owing to medullary expansion, as well as for the skeletal dysplasia related to desferrioxamine therapy. The combined cortical width of the mid shaft of the second metacarpal was measured on left hand radiographs to assess osteoporosis. Results: Sixteen patients had radiographic evidence of desferrioxamine-induced bone dysplasia. These included metaphyseal sclerosis in long bone (n=16), irregular sclerosis at the costochondral junction (n=3) and platyspondyly (n= 1). Two patients had radiographic evidence of medullary expansion with widening of medulla and marked thinning of cortex in the tubular bones. Osteoporosis, as indicated by thinning of metacarpal cortex, was noted in 17 patients (8 with and 9 without desferrioxamine-induced bone dysplasia). Conclusions: With provision of the modern regime of regular transfusion and desferrioxamine chelation, desferrioxamine-induced bone dysplasia was a much more frequently detected radiographic abnormality in {beta}-thalassaemia major than radiographic features owing to medullary expansion. Osteoporosis, as indicated by thinned metacarpal cortices, remained a frequent feature irrespective of the status of the skeletal dysplasia. (orig.)

  5. Expression and significance of IL-18 in the bone marrow of patients with hema tological diseases

    Institute of Scientific and Technical Information of China (English)

    张斌; 饶青; 郑国光; 曹震宇; 马小彤; 李戈; 林永敏; 耿以琪; 吴克复

    2003-01-01

    Objective To investigate the levels of IL-18 in the bone marrow of both normal subjects a nd patients with hematological diseases and to determine the possible significan ce of IL-18 in pathogenesis of some hematological malignancies. Methods The IL-18 mRNA levels in the bone marrow of 140 patients with hematological dis eases and 15 normal donors were determined by the semi-quantitative reverse tra nscriptase polymerase chain reaction (RT-PCR). Immunohistochemical method was used to detect IL-18 protein in 12 patients with acute myeloid leukemia (AML). The possible regulation of IL-18 for proliferation of some leukemia cells was investigated using antisense techniques.Results IL-18 mRNA levels were obviously higher in the patients with leukemia or other malignant hematological diseases (OMHD) than in normal donors. However, no sign ificant difference was found in the level of transcription between patients with iron deficiency anemia (IDA) and normal controls. Immunohistochemical method c onfirmed the presence of IL-18 protein in 10 out of 12 AML cases with positive transcription. By 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl tetrazolium b romide (MTT) assay, IL-18 antisense oligodeoxynucleotides (ASON) clearly inhibi ted the growth of J6-1 and HL-60 cells (42% and 12% inhibited, respectively) i n a dose-dependant manner.Conclusions IL-18 was detected at elevated levels in the bone marrow of patients with some hematological malignancies, and might be involved in the proliferation of certai n leukemic cells in vivo through an autocrine mechanism.

  6. Interleukin-1 gene polymorphism disease activity and bone mineral metabolism in rheumatoid arthritis

    Institute of Scientific and Technical Information of China (English)

    2002-01-01

    Objective To determine whether interleukin-1α and 1β gene polymorphism is associated with rheumatoid arthritis disease activity and bone mineral metabolism, and whether there is any relationship between IL-1β and rheumatoid arthritis (RA) motif gene. Methods IL-1 gene polymorphisms were analyzed in 65 RA patients who met American College of Radiology (ACR) criteria and 60 controls. From genomic DNA, 2 polymorphisms in each gene for IL1α-889 and IL-1β+3953 were typed by PCR-RFLP and HLA-DRB1 allele typing was also undertaken by PCR-SSOP. Some clinical and laboratory parameters were collected. The allelic frequencies and carriage rates were compared between RA patients and controls and between patients with active and quiescent disease. Comparison was also made between IL-1 polymorphism and parameters of bone mineral metabolism and between patients with the HLA-DRB1 RA motif plus IL-1β2 and patients without the two alleles. Fisher test and the analysis of variance was used to analyze the data.Results There was no significant difference in the frequency and carriage rate of IL-1α polymorphisms between RA patients and the controls. The β2/2 genotype of IL-1β was more common in female RA patients compared with controls (P=0.001). A lower carriage rate of IL-1β2 occurred in male RA patients (P=0.001). A higher carriage rate of IL-1α2 is associated with a higher ESR (P=0.008), HAQ score (P=0.03), and vit-D3 (P<0.001), but conversely a lower SJC (p=0.002), a lower RF (P=0.002) and a lower BMD at the lumbar spine (P=0.001). A higher frequency of IL-1α1 is associated with a lower CRP value (P=0.009). An increased IL-1β2 carriage is associated with active rheumatoid disease as indicated by a higher CRP (P<0.001), ESR (P<0.001) and pain score (P=0.001) and a higher BMD at the lumbar spine (P=0.007), lower vit-D3 and. Udpd/Crea level The presence of the HLA DRB1 RA motif and IL-1β allele 2 at same time did not contribute to disease activity

  7. Caloric test results in Paget ́s disease of bone

    Directory of Open Access Journals (Sweden)

    De la Fuente-Cañibano R

    2013-04-01

    Full Text Available Introduction: In Paget's disease, the most common neurological complication relates to the temporal bone involvement intrapetrous level. Vestibular symptoms are common in Paget's disease, but you can pass easily overlooked for its mild intensity. The literature is not clear regarding the result of caloric testing.Methods: The study comprised 50 patient s, 30 patients with PD without skull involvement, 13 patients with skull involvement and without temporal involvement and 7 patients with PD with skull and temporal involvement in scintigraphy. Results: 22% of patient s in the sample had history crises dizzying and yet 46% had deficits in vestibular caloric test result.Conclusions: We found no statistically significant differences between the groups in the analysis of EP vestibular deficit.

  8. 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

  9. Hepatocyte growth factor pathway upregulation in the bone marrow microenvironment in multiple myeloma is associated with lytic bone disease

    DEFF Research Database (Denmark)

    Kristensen, Ida B; Christensen, Jacob H; Lyng, Maria Bibi;

    2013-01-01

    using bone marrow biopsies (BMBs) of patients with MM and monoclonal gammopathy of undetermined significance (MGUS), and healthy volunteers (HV). BMBs (N = 110) obtained at diagnosis were snap-frozen and used to evaluate gene expression by quantitative reverse transcription polymerase chain reaction....... LBD was evaluated using standard radiographs. Enzyme-linked immunosorbent assay (ELISA) was performed on matched bone marrow plasma and immunohistochemistry on matched formalin-fixed paraffin-embedded biopsies. Gene expression of HGF, SDC1, and MET in BMBs were significantly altered in MM versus HV...... and MGUS, and HGF and MET correlated with the extent of LBD. A significant correlation between gene and protein expression levels was observed for SDC1 (Syndecan-1) and HGF. The HGF bone marrow plasma level was significantly lower in MM patients with no/limited versus advanced LBD. Our novel approach using...

  10. Novel Lesions of Bones and Joints Associated with Chikungunya Virus Infection in Two Mouse Models of Disease: New Insights into Disease Pathogenesis.

    Directory of Open Access Journals (Sweden)

    Brad A Goupil

    Full Text Available Chikungunya virus is an arbovirus spread predominantly by Aedes aegypti and Ae. albopictus mosquitoes, and causes debilitating arthralgia and arthritis. While these are common manifestations during acute infection and it has been suggested they can recur in patients chronically, gaps in knowledge regarding the pathogenesis still exist. Two established mouse models were utilized (adult IRF 3/7 -/- -/- and wild-type C57BL/6J mice to evaluate disease manifestations in bones and joints at various timepoints. Novel lesions in C57BL/6J mice consisted of periostitis (91% and foci of cartilage of necrosis (50% of mice at 21 DPI. Additionally, at 21 DPI, 50% and 75% of mice exhibited periosteal bone proliferation affecting the metatarsal bones, apparent via histology and μCT, respectively. μCT analysis did not reveal any alterations in trabecular bone volume measurements in C57BL/6J mice. Novel lesions demonstrated in IRF 3/7 -/- -/- mice at 5 DPI included focal regions of cartilage necrosis (20%, periosteal necrosis (66%, and multifocal ischemic bone marrow necrosis (100%. Contralateral feet in 100% of mice of both strains had similar, though milder lesions. Additionally, comparison of control IRF 3/7 -/- -/- and wild-type C57BL/6J mice demonstrated differences in cortical bone. These experiments demonstrate novel manifestations of disease similar to those occurring in humans, adding insight into disease pathogenesis, and representing new potential targets for therapeutic interventions. Additionally, results demonstrate the utility of μCT in studies of bone and joint pathology and illustrate differences in bone dynamics between mouse strains.

  11. Deficiency of trace elements and bone diseases%微量元素缺乏与骨病

    Institute of Scientific and Technical Information of China (English)

    刘媛; 王永福; 刘忠厚

    2014-01-01

    Bone diseases are one kind of the motor system diseases involving of bone, joint, tendons and fascia.They include osteoporosis, rickets, osteosclerosis, femoral head necrosis, fractures, etc.Several factors affect the development of the diseases, such as genetic, environmental, endocrinal and nutritional factors.In addition to calcium, phosphorus and vitamins which are associated with bone diseases, a number of trace elements are also involved in the growth, development and metabolism of the bone.Furthermore, various trace elements play the different roles in bone metabolism and diseases.Therefore, deficiency of trace elements, which participate in bone metabolism and regeneration, may cause the disorder of bone growth, resorption, and mineral deposition, leading to different types of bone diseases.This paper reviews the distribution of trace elements in the bone, the regulation of bone metabolism, bone diseases due to the deficiency of trace elements, and supplementation of trace elements for prevention and treatment of bone diseases, thus providing theoretical basis for clinical diagnosis and therapy of bone diseases for trace element deficiency.%骨病是一类发生在骨骼、关节、肌腱及筋膜等运动系统的疾病,包括骨质疏松、骨软骨病、骨质增生硬化、股骨头坏死及骨折等。骨病的发生发展受众多因素的影响,包括遗传、环境、内分泌和营养等。除了钙、磷等常量元素及维生素与骨病的发生发展相关,体内一些微量元素也参与骨的生长发育和新陈代谢,且各种微量元素在骨代谢调节和骨病发生发展中发挥的作用不同。因而,体内缺乏参与骨代谢和骨再生的微量元素,使骨生长、骨吸收及骨矿物质沉积等过程紊乱,引起不同类型的骨病。本文就与骨代谢相关的微量元素在骨组织中的分布及在骨代谢调节中的作用,微量元素缺乏所致的骨病及补充微量元素对骨病的防治

  12. Fibroblast growth factor 23 contributes to diminished bone mineral density in childhood inflammatory bowel disease

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    El-Hodhod Mostafa

    2012-05-01

    Full Text Available Abstract Background Diminished bone mineral density (BMD is of significant concern in pediatric inflammatory bowel disease (IBD. Exact etiology is debatable. The recognition of fibroblast growth factor 23 (FGF23, a phosphaturic hormone related to tumor necrosis factor alpha (TNF-α makes it plausible to hypothesize its possible relation to this pathology. Methods In this follow up case control study, BMD as well as serum levels of FGF23, calcium, phosphorus, alkaline phosphatase, creatinine, parathyroid hormone, 25 hydroxy vitamin D3 and 1, 25 dihydroxy vitamin D3 were measured in 47 children with IBD during flare and reassessed in the next remission. Results Low BMD was frequent during IBD flare (87.2% with significant improvement after remission (44.7%. During disease flare, only 21.3% of patients had vitamin D deficiency, which was severe in 12.8%. During remission, all patients had normal vitamin D except for two patients with Crohn’s disease (CD who remained vitamin D deficient. Mean value of serum FGF23 was significantly higher among patients with IBD during flare compared to controls. It showed significant improvement during remission but not to the control values. 1, 25 dihydroxy vitamin D3, FGF23, serum calcium and urinary phosphorus were significant determinants of BMD in IBD patients. Conclusions We can conclude that diminished BMD in childhood IBD is a common multifactorial problem. Elevated FGF23 would be a novel addition to the list of factors affecting bone mineral density in this context. Further molecular studies are warranted to display the exact interplay of these factors.

  13. [Functional endoscopic ethmoid bone revision in inflammatory paranasal sinus diseases in childhood].

    Science.gov (United States)

    Küttner, K; Siering, U; Looke, G; Eichhorn, M

    1992-05-01

    A total of 57 children aged between 5 and 15 years underwent endoscopic ethmoid surgery for recurrent acute or chronic rhinogenic sinusitis, chronic sinusitis associated with bronchial asthma or chronic bronchitis, early rhinogenous orbital complications and paranasal sinus disease associated with mucoviscidosis. The indications for surgical intervention are based on symptoms, the findings on nasal endoscopy and a CT scan of the paranasal sinuses in the coronal plane. The preoperative examination shows that in childhood there are endoscopically detectable anatomical variations of the ethmoid bone (pneumatised middle nasal concha, reversed curvature of the middle turbinate and contact between the uncinate process and the turbinate) that create the conditions for the development of inflammatory paranasal sinus diseases. Careful elimination of these obstructions can accomplish complete cure of the inflammation, and early orbital complications can be treated effectively by combination of surgery with infusion of antibiotics. All surgery was performed under endotracheal anaesthesia using the endoscopic technique described by Messerklinger. The follow-up period was between 6 and 18 months. With the exception of children suffering from mucoviscidosis, cure of the paranasal sinus disease and rhinomanometrically demonstrable improvement of the nasal breathing was achieved in all cases. On the basis of our results a conservative endoscopic approach is recommended as an effective surgical method for the treatment of paranasal sinus disease in childhood. PMID:1612930

  14. Mutations in mitochondrial enzyme GPT2 cause metabolic dysfunction and neurological disease with developmental and progressive features.

    Science.gov (United States)

    Ouyang, Qing; Nakayama, Tojo; Baytas, Ozan; Davidson, Shawn M; Yang, Chendong; Schmidt, Michael; Lizarraga, Sofia B; Mishra, Sasmita; Ei-Quessny, Malak; Niaz, Saima; Gul Butt, Mirrat; Imran Murtaza, Syed; Javed, Afzal; Chaudhry, Haroon Rashid; Vaughan, Dylan J; Hill, R Sean; Partlow, Jennifer N; Yoo, Seung-Yun; Lam, Anh-Thu N; Nasir, Ramzi; Al-Saffar, Muna; Barkovich, A James; Schwede, Matthew; Nagpal, Shailender; Rajab, Anna; DeBerardinis, Ralph J; Housman, David E; Mochida, Ganeshwaran H; Morrow, Eric M

    2016-09-20

    Mutations that cause neurological phenotypes are highly informative with regard to mechanisms governing human brain function and disease. We report autosomal recessive mutations in the enzyme glutamate pyruvate transaminase 2 (GPT2) in large kindreds initially ascertained for intellectual and developmental disability (IDD). GPT2 [also known as alanine transaminase 2 (ALT2)] is one of two related transaminases that catalyze the reversible addition of an amino group from glutamate to pyruvate, yielding alanine and α-ketoglutarate. In addition to IDD, all affected individuals show postnatal microcephaly and ∼80% of those followed over time show progressive motor symptoms, a spastic paraplegia. Homozygous nonsense p.Arg404* and missense p.Pro272Leu mutations are shown biochemically to be loss of function. The GPT2 gene demonstrates increasing expression in brain in the early postnatal period, and GPT2 protein localizes to mitochondria. Akin to the human phenotype, Gpt2-null mice exhibit reduced brain growth. Through metabolomics and direct isotope tracing experiments, we find a number of metabolic abnormalities associated with loss of Gpt2. These include defects in amino acid metabolism such as low alanine levels and elevated essential amino acids. Also, we find defects in anaplerosis, the metabolic process involved in replenishing TCA cycle intermediates. Finally, mutant brains demonstrate misregulated metabolites in pathways implicated in neuroprotective mechanisms previously associated with neurodegenerative disorders. Overall, our data reveal an important role for the GPT2 enzyme in mitochondrial metabolism with relevance to developmental as well as potentially to neurodegenerative mechanisms. PMID:27601654

  15. Autologous bone marrow stromal cells are promising candidates for cell therapy approaches to treat bone degeneration in sickle cell disease

    Directory of Open Access Journals (Sweden)

    Angélique Lebouvier

    2015-11-01

    SCD-ON patients have a higher frequency of BMSCs that retain their bone regeneration potential. Our findings suggest that BMSCs isolated from SCD-ON patients can be used clinically in cell therapy approaches. This work provides important preclinical data that is necessary for the clinical application of expanded BMSCs in advanced therapies and medical products.

  16. 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. PMID:27668003

  17. The Effect of Glucocorticoids on Bone Mass in Patients with Asthma and Chronic obstructive Pulmonary Disease

    Directory of Open Access Journals (Sweden)

    Evrim Karadağ Saygı

    2005-06-01

    Full Text Available Inhaled corticosteroids and bronchodilators have become a key element in the maintenance treatment of bronchial asthma and chronic obstructive pulmanory disease (COPD. It is well known that long-term systemic steroid use causes osteoporosis, whereas inhaled corticosteroids and bronchodilators have been discussed to be cause of such side-affect. The aim of this study was to detect the effect of long term inhaled/oral steroids and bronchodilators on bone mineral density (BMD with asthma and COPD. Fifty-three patients with bronchial asthma (n=44 and COPD (n=9 were enrolled in this study. BMD were measured and risk factors for osteoporosis were detected. BMD measurements of lumbar area of the spine (L2-4, neck of femur and femoral ward’s triangle zone were performed by the dual energy x-ray absorptiometer (LUNAR. 53 patients evaluated in three groups according to treatment type; 26 patients were using inhaled corticosteroids and bronchodilators (group 1, 18 patients were using only bronchodilators (group 2 and 9 patients were using (group 3 oral corticosteroids and bronchodilators. There were significant differences between group 3 and other two groups in terms of BMD, T and Z scores of the lumbar and femoral neck (p0.05. As a result, we suggest that systemic corticosteroids negatively affect bone mineral density more than inhaled corticosteroids in patients with COPD.

  18. 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.

  19. 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.

  20. 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.

  1. CT Features of Bone Flare Phenomena of Metastatic Bone Disease in Lung Cancer%肺癌骨转移骨闪烁现象的CT表现

    Institute of Scientific and Technical Information of China (English)

    邱艳芳; 文智; 许晓燕; 王永丽; 于莹莹; 赵艳萍

    2015-01-01

    PurposeThe bone flare phenomena has been well described on bone scintigraphy for efficacy monitoring up to now, but our knowledge has been rarely described on MSCT, the phenomena may be erroneously classiifed as disease progression. This article intends to evaluate the existence and CT features of bone flare phenomena of metastatic bone disease in lung cancer patients treated with ibandronate, to raise awareness of this phenomenon.Materials and Methods The clinical and image data of 45 patients with bone metastases of lung cancer were retrospectively analyzed prior to treatment and 3, 6 months after treatment, the change of CT value and CT features 3 months after treatment between bone flare phenomena group, progressive disease group 1 and progressive disease group 2 were compared.Results The incidence of bone flare phenomena was 6.7% (3/45). 3 months after treatment, CT value of the bone flare phenomena group and progressive disease group 1 changed when compared with before treatment, the differences were statistically signiifcant (t=-5.787 and-2.788,P0.05) of CT value in the progressive disease group 2 after 3 months' treatment. After 3 months' treatment, the bone flare phenomena group mostly appeared as osteogenic sclerosis of osteolytic lesions, while the cases of progressive disease group mostly appeared as new periosteal reaction of the lesion, or osteogenic/mixed lesion combined with osteolytic damages, the difference between the two groups was statistically signiifcant (χ2=10.139, 8.041 and 4.154,P0.05).Conclusion In patients treated with ibandronate, when there is therapeutic effect evaluation standard of bone metastases (disease progression) and clinical comprehensive curative effect evaluation standard (effective) discordance at 3 months after treatment, it can be interpreted as bone flare phenomena, and the change of CT features contributes to the differential diagnosis of bone flare phenomena with progressive disease.%目的:骨闪烁现象在核

  2. A closer look at the developmental interplay between parenting and perceived health in adolescents with congenital heart disease.

    Science.gov (United States)

    Rassart, Jessica; Luyckx, Koen; Goossens, Eva; Apers, Silke; Moons, Philip

    2014-12-01

    The present study examined associations between parenting and perceived health in adolescents with congenital heart disease (CHD) using a longitudinal trajectory approach. Adolescents with CHD were selected from the database of pediatric and congenital cardiology of the University Hospitals Leuven. A total of 429 adolescents (M age = 16 at T1) participated in the present study, comprising four measurement waves spanning approximately 3 years. Latent class growth analysis was used to identify trajectory classes of parenting and perceived health. Whereas adolescents from democratic households reported the most favorable health outcomes, adolescents from authoritarian, overprotective, and psychologically controlling families (all characterized by relatively high levels of psychological control) showed an increased risk for poor perceived health over time. Hence, the present study found substantial developmental associations between parenting and perceived health in adolescents with CHD. Future research should investigate whether working on the parent-adolescent relationship can foster patients' health. PMID:24819301

  3. Clinical benefits of biochemical markers of bone turnover in Egyptian children with chronic liver diseases

    Institute of Scientific and Technical Information of China (English)

    Karam A Mahdy; Hanaa H Ahmed; Fathia Mannaa; Azza Abdel-Shaheed

    2007-01-01

    AIM: To investigate the association between serum insulin-like growth factor 1 (IGF-1), osteocalcin, and parathyroid hormone (PTH) levels with the etiology and clinical condition of patients with chronic liver disease.METHODS: Eighty children with hepatocellular damage were divided into 3 groups according to the etiology of disease infection: bilharziasis (9 patients), hepatitis B virus (HBV, 12 patients) and hepatitis C virus (HCV, 29 patients). The Child score index was found as A in 24 patients, B in 22 patients, C in 4 patients. Thirty healthy children served as control group. HBsAg, HBcAbIgM,HBcAbIgG, and anti-HCV were detected using ELISA technique. HCV-RNA was measured by reverse transcription polymerase chain reaction (RT-PCR). Antibilharzial antibodies were detected by indirect haemagglutination test. Liver function tests were performed using autoanalyser. Serum IGF-1, osteocalcin and PTH levels were measured by ELISA technique. Abdominal ultrasonography was also conducted.RESULTS: Serum IGF-1 level was significantly lower in all patient groups with liver diseases, while serum osteocalcin and PTH levels were significantly elevated in patients with HBV and HCV infections compared with the control group. Serum osteocalcin and PTH concentrations were measured with the severity of liver disease from Child A to C. Child A patients unexpectedly showed significantly reduced IGF-1 levels in comparison to patients staged as Child B or C. Serum osteocalcin level was negatively correlated with albumin (14.7 ± 0.54 vs 3.6± 0.10, P < 0.05), while that for PTH was positively correlated with total protein (70.1 ± 2.17 vs 6.7 + 0.10,P < 0.05) in patients with HCV infection.CONCLUSION: Low serum IGF-1 level seems to play a critical role in the bone loss in patients with chronic liver disease. Elevated biochemical markers of bone remodeling suggest high-turnover in patients with viral infection and reflect severity of the clinical stage.

  4. Osteoblast CFTR inactivation reduces differentiation and osteoprotegerin expression in a mouse model of cystic fibrosis-related bone disease.

    Directory of Open Access Journals (Sweden)

    Michael S Stalvey

    Full Text Available Low bone mass and increased fracture risk are recognized complications of cystic fibrosis (CF. CF-related bone disease (CFBD is characterized by uncoupled bone turnover--impaired osteoblastic bone formation and enhanced osteoclastic bone resorption. Intestinal malabsorption, vitamin D deficiency and inflammatory cytokines contribute to CFBD. However, epidemiological investigations and animal models also support a direct causal link between inactivation of skeletal cystic fibrosis transmembrane regulator (CFTR, the gene that when mutated causes CF, and CFBD. The objective of this study was to examine the direct actions of CFTR on bone. Expression analyses revealed that CFTR mRNA and protein were expressed in murine osteoblasts, but not in osteoclasts. Functional studies were then performed to investigate the direct actions of CFTR on osteoblasts using a CFTR knockout (Cftr-/- mouse model. In the murine calvarial organ culture assay, Cftr-/- calvariae displayed significantly less bone formation and osteoblast numbers than calvariae harvested from wildtype (Cftr+/+ littermates. CFTR inactivation also reduced alkaline phosphatase expression in cultured murine calvarial osteoblasts. Although CFTR was not expressed in murine osteoclasts, significantly more osteoclasts formed in Cftr-/- compared to Cftr+/+ bone marrow cultures. Indirect regulation of osteoclastogenesis by the osteoblast through RANK/RANKL/OPG signaling was next examined. Although no difference in receptor activator of NF-κB ligand (Rankl mRNA was detected, significantly less osteoprotegerin (Opg was expressed in Cftr-/- compared to Cftr+/+ osteoblasts. Together, the Rankl:Opg ratio was significantly higher in Cftr-/- murine calvarial osteoblasts contributing to a higher osteoclastogenesis potential. The combined findings of reduced osteoblast differentiation and lower Opg expression suggested a possible defect in canonical Wnt signaling. In fact, Wnt3a and PTH-stimulated canonical Wnt

  5. Subtle changes in bone mineralization density distribution in most severely affected patients with chronic obstructive pulmonary disease.

    Science.gov (United States)

    Misof, B M; Roschger, P; Jorgetti, V; Klaushofer, K; Borba, V Z C; Boguszewski, C L; Cohen, A; Shane, E; Zhou, H; Dempster, D W; Moreira, C A

    2015-10-01

    Chronic obstructive pulmonary disease (COPD) is associated with low aBMD as measured by DXA and altered microstructure as assessed by bone histomorphometry and microcomputed tomography. Knowledge of bone matrix mineralization is lacking in COPD. Using quantitative backscatter electron imaging (qBEI), we assessed cancellous (Cn.) and cortical (Ct.) bone mineralization density distribution (BMDD) in 19 postmenopausal women (62.1 ± 7.3 years of age) with COPD. Eight had sustained fragility fractures, and 13 had received treatment with inhaled glucocorticoids. The BMDD outcomes from the patients were compared with healthy reference data and were correlated with previous clinical and histomorphometric findings. In general, the BMDD outcomes for the patients were not significantly different from the reference data. Neither the subgroups of with or without fragility fractures or of who did or did not receive inhaled glucocorticoid treatment, showed differences in BMDD. However, subgroup comparison according to severity revealed 10% decreased cancellous mineralization heterogeneity (Cn.CaWidth) for the most severely affected compared with less affected patients (p=0.042) and compared with healthy premenopausal controls (p=0.021). BMDD parameters were highly correlated with histomorphometric cancellous bone volume (BV/TV) and formation indices: mean degree of mineralization (Cn.CaMean) versus BV/TV (r=0.58, p=0.009), and Cn.CaMean and Ct.CaMean versus bone formation rate (BFR/BS) (r=-0.71, p50th percentile) BV/TV. The normality in most of the BMDD parameters and bone formation rates as well as the significant correlations between them suggests unaffected mineralization processes in COPD. Our findings also indicate no significant negative effect of treatment with inhaled glucocorticoids on the bone mineralization pattern. However, the observed concomitant occurrence of relatively lower bone volumes with lower bone matrix mineralization will both contribute to the reduced a

  6. Bone marrow derived stem cells for the treatment of end-stage liver disease.

    Science.gov (United States)

    Margini, Cristina; Vukotic, Ranka; Brodosi, Lucia; Bernardi, Mauro; Andreone, Pietro

    2014-07-21

    End-stage disease due to liver cirrhosis is an important cause of death worldwide. Cirrhosis results from progressive, extensive fibrosis and impaired hepatocyte regeneration. The only curative treatment is liver transplantation, but due to the several limitations of this procedure, the interest in alternative therapeutic strategies is increasing. In particular, the potential of bone marrow stem cell (BMSC) therapy in cirrhosis has been explored in different trials. In this article, we evaluate the results of 18 prospective clinical trials, and we provide a descriptive overview of recent advances in the research on hepatic regenerative medicine. The main message from the currently available data in the literature is that BMSC therapy is extremely promising in the context of liver cirrhosis. However, its application should be further explored in randomized, controlled trials with large cohorts and long follow-ups. PMID:25083082

  7. Role of RANKL-RANK/Osteoprotegerin Pathway in Cardiovascular and Bone Disease Associated with HIV Infection

    Science.gov (United States)

    Kelesidis, Theodoros; Currier, Judith S.; Yang, Otto O.; Brown, Todd T

    2016-01-01

    Patients with HIV-1 infection often develop multiple complications and comorbidities, including osteoporosis and atherosclerosis. The receptor activator of nuclear factor kappa-B/receptor activator of nuclear factor kappa-B ligand/osteoprotegerin axis has been identified as a possible common link between osteoporosis and vascular diseases. Since the discovery of this axis, much has been learned about its role in controlling skeletal biology and less about its role in the context of vascular biology. However, the exact role of the receptor activator of nuclear factor kappa-B ligand/osteoprotegerin axis in HIV infection is not completely understood. In this review we examine the mechanisms by which inflammation and immune dysregulation in HIV-1 infection may impact bone turnover and atherogenesis through perturbations in the receptor activator of nuclear factor kappa-B/receptor activator of nuclear factor kappa-B ligand/osteoprotegerin axis. PMID:25102334

  8. Targeting the molecular and cellular interactions of the bone marrow niche in immunologic disease.

    Science.gov (United States)

    Brozowski, Jaime M; Billard, Matthew J; Tarrant, Teresa K

    2014-02-01

    Recent investigations have expanded our knowledge of the regulatory bone marrow (BM) niche, which is critical in maintaining and directing hematopoietic stem cell (HSC) self-renewal and differentiation. Osteoblasts, mesenchymal stem cells (MSCs), and CXCL12-abundant reticular (CAR) cells are niche components in close association with HSCs and have been more clearly defined in immune cell function and homeostasis. Importantly, cellular inhabitants of the BM niche signal through G protein-coupled surface receptors (GPCRs) for various appropriate immune functions. In this article, recent literature on BM niche inhabitants (HSCs, osteoblasts, MSCs, CAR cells) and their GPCR mechanistic interactions are reviewed for better understanding of the BM cells involved in immune development, immunologic disease, and current immune reconstitution therapies. PMID:24408534

  9. Potential of bone marrow mesenchymal stem cells in management of Alzheimer's disease in female rats.

    Science.gov (United States)

    Salem, Ahmed M; Ahmed, Hanaa H; Atta, Hazem M; Ghazy, Mohamed A; Aglan, Hadeer A

    2014-12-01

    Alzheimer's disease (AD) has been called the disease of the century with significant clinical and socioeconomic impacts. Pharmacological treatment has limited efficacy and only provides symptomatic relief without long-term cure. Accordingly, there is an urgent need to develop novel and effective medications for AD. Stem cell-based therapy is a promising approach to handling neurodegenerative diseases. Therefore, the current study aimed to explore the possible therapeutic role of single intravenous injection of bone marrow derived mesenchymal stem cells (BM-MSCs) after 4 months in management of AD in the experimental model. The work also extended to compare the therapeutic potential of BM-MSCs with 2 conventional therapies of AD; rivastigmine and cerebrolysin administered daily. BM-MSCs were able to home at the injured brains and produced significant increases in the number of positive cells for choline acetyltransferase (ChAT) and survivin expression, as well as selective AD indicator-1 (seladin-1) and nestin gene expression. Histopathological examination indicated that BM-MSCs could remove beta-amyloid plaques from hippocampus. Significant improvement in these biomarkers was similar to or better sometimes than the reference drugs, clearly showing the potential therapeutic role of BM-MSCs against AD through their anti-apoptotic, neurogenic and immunomodulatory properties. PMID:25044885

  10. Potential of bone marrow mesenchymal stem cells in management of Alzheimer's disease in female rats.

    Science.gov (United States)

    Salem, Ahmed M; Ahmed, Hanaa H; Atta, Hazem M; Ghazy, Mohamed A; Aglan, Hadeer A

    2014-12-01

    Alzheimer's disease (AD) has been called the disease of the century with significant clinical and socioeconomic impacts. Pharmacological treatment has limited efficacy and only provides symptomatic relief without long-term cure. Accordingly, there is an urgent need to develop novel and effective medications for AD. Stem cell-based therapy is a promising approach to handling neurodegenerative diseases. Therefore, the current study aimed to explore the possible therapeutic role of single intravenous injection of bone marrow derived mesenchymal stem cells (BM-MSCs) after 4 months in management of AD in the experimental model. The work also extended to compare the therapeutic potential of BM-MSCs with 2 conventional therapies of AD; rivastigmine and cerebrolysin administered daily. BM-MSCs were able to home at the injured brains and produced significant increases in the number of positive cells for choline acetyltransferase (ChAT) and survivin expression, as well as selective AD indicator-1 (seladin-1) and nestin gene expression. Histopathological examination indicated that BM-MSCs could remove beta-amyloid plaques from hippocampus. Significant improvement in these biomarkers was similar to or better sometimes than the reference drugs, clearly showing the potential therapeutic role of BM-MSCs against AD through their anti-apoptotic, neurogenic and immunomodulatory properties.

  11. Activator protein 1 (Fos/Jun) functions in inflammatory bone and skin disease.

    Science.gov (United States)

    Zenz, Rainer; Eferl, Robert; Scheinecker, Clemens; Redlich, Kurt; Smolen, Josef; Schonthaler, Helia B; Kenner, Lukas; Tschachler, Erwin; Wagner, Erwin F

    2008-01-01

    Activator protein 1 (AP-1) (Fos/Jun) is a transcriptional regulator composed of members of the Fos and Jun families of DNA binding proteins. The functions of AP-1 were initially studied in mouse development as well as in the whole organism through conventional transgenic approaches, but also by gene targeting using knockout strategies. The importance of AP-1 proteins in disease pathways including the inflammatory response became fully apparent through conditional mutagenesis in mice, in particular when employing gene inactivation in a tissue-specific and inducible fashion. Besides the well-documented roles of Fos and Jun proteins in oncogenesis, where these genes can function both as tumor promoters or tumor suppressors, AP-1 proteins are being recognized as regulators of bone and immune cells, a research area termed osteoimmunology. In the present article, we review recent data regarding the functions of AP-1 as a regulator of cytokine expression and an important modulator in inflammatory diseases such as rheumatoid arthritis, psoriasis and psoriatic arthritis. These new data provide a better molecular understanding of disease pathways and should pave the road for the discovery of new targets for therapeutic applications.

  12. Bone scintigraphy as cornerstone in the diagnosis of Erdheim-Chester disease.

    Science.gov (United States)

    García-Gómez, F J; Cambil-Molina, T; Ríos-Martín, J J; de la Riva-Pérez, P A; Calvo-Morón, C; Castro-Montaño, J

    2016-01-01

    The Erdheim-Chester disease (ECD) is an extremely rare form of non-Langerhans cell histiocytosis. The main difficulty for its diagnosis lies in the wide variety of non-specific symptoms and signs that can occur in the disease process, leading, therefore, to there being no clear-cut algorithm as a guide for an optimal biopsy to confirm the diagnosis. An 81-year-old male with history of diabetes insipidus was admitted due to non-specific respiratory signs. Imaging techniques revealed osteoblastic lesions in the lumbar spine. Whole-body bone-scintigraphy (BS) was performed, in which lesions involving the axial and appendicular skeleton, with different rates of osteoblastic activity, were observed. This highlighted a symmetrical severely intense uptake in the knees, leading to an accurate biopsy specimen that enabled making the definitive diagnosis. BS is a widely available, safe, and inexpensive technique that shows a characteristic pattern of uptake for ECD, thus its use is highly recommended for screening and guiding biopsy if clinical suspicion exists. Furthermore, when the scintigraphy pattern is incidentally observed, biopsy of increased uptake areas (tibia preferably) is mandatory in order to rule out the disease. PMID:26750553

  13. Graft versus host disease in the bone marrow, liver and thymus humanized mouse model.

    Directory of Open Access Journals (Sweden)

    Matthew B Greenblatt

    Full Text Available Mice bearing a "humanized" immune system are valuable tools to experimentally manipulate human cells in vivo and facilitate disease models not normally possible in laboratory animals. Here we describe a form of GVHD that develops in NOD/SCID mice reconstituted with human fetal bone marrow, liver and thymus (NS BLT mice. The skin, lungs, gastrointestinal tract and parotid glands are affected with progressive inflammation and sclerosis. Although all mice showed involvement of at least one organ site, the incidence of overt clinical disease was approximately 35% by 22 weeks after reconstitution. The use of hosts lacking the IL2 common gamma chain (NOD/SCID/γc(-/- delayed the onset of disease, but ultimately did not affect incidence. Genetic analysis revealed that particular donor HLA class I alleles influenced the risk for the development of GVHD. At a cellular level, GVHD is associated with the infiltration of human CD4+ T cells into the skin and a shift towards Th1 cytokine production. GVHD also induced a mixed M1/M2 polarization phenotype in a dermal murine CD11b+, MHC class II+ macrophage population. The presence of xenogenic GVHD in BLT mice both presents a major obstacle in the use of humanized mice and an opportunity to conduct preclinical studies on GVHD in a humanized model.

  14. Autotransplantation of bone marrow-derived stem cells as a therapy for neurodegenerative diseases.

    Science.gov (United States)

    Kan, I; Melamed, E; Offen, D

    2007-01-01

    Neurodegenerative diseases are characterized by a progressive degeneration of selective neural populations. This selective hallmark pathology and the lack of effective treatment modalities make these diseases appropriate candidates for cell therapy. Bone marrow-derived mesenchymal stem cells (MSCs) are self-renewing precursors that reside in the bone marrow and may further be exploited for autologous transplantation. Autologous transplantation of MSCs entirely circumvents the problem of immune rejection, does not cause the formation of teratomas, and raises very few ethical or political concerns. More than a few studies showed that transplantation of MSCs resulted in clinical improvement. However, the exact mechanisms responsible for the beneficial outcome have yet to be defined. Possible rationalizations include cell replacement, trophic factors delivery, and immunomodulation. Cell replacement theory is based on the idea that replacement of degenerated neural cells with alternative functioning cells induces long-lasting clinical improvement. It is reasoned that the transplanted cells survive, integrate into the endogenous neural network, and lead to functional improvement. Trophic factor delivery presents a more practical short-term approach. According to this approach, MSC effectiveness may be credited to the production of neurotrophic factors that support neuronal cell survival, induce endogenous cell proliferation, and promote nerve fiber regeneration at sites of injury. The third potential mechanism of action is supported by the recent reports claiming that neuroinflammatory mechanisms play an important role in the pathogenesis of neurodegenerative disorders. Thus, inhibiting chronic inflammatory stress might explain the beneficial effects induced by MSC transplantation. Here, we assemble evidence that supports each theory and review the latest studies that have placed MSC transplantation into the spotlight of biomedical research.

  15. UNREMITTING EARLY STAGE HODGKIN’S DISEASE: REPORT OF 7 CASES AND BONE MARROW TISSUE IMMUNOHISTOCHEMICAL MARKER STUDY

    Directory of Open Access Journals (Sweden)

    D. Tamiolakis

    2006-07-01

    Full Text Available Bone marrow is infrequently implicated in early stages of Hodgkin’s disease. We studied the immunohistochemical bone marrow tissue of 7 out of 20 cases with early stage Hodgkin’s disease of the mixed cellularity variant, diagnosed by lymph node biopsy at initial presentation, not responding to radiotherapy alone, in order to examine possible marrow attack. A statistically significant prevalence of CD45, CD45RO, and CD4 positive infiltrates, to the advantage of unremitting hosts, was found. The predominance of CD4-positive cells in the bone marrow space might be suggestive of involvement in the process and could explain the abnormal cytokine production leading to reduced T-cell immunity and inefficient antitumor response despite the existence of a vast majority of reactive infiltrating immune cells.

  16. Coupling of porcine bone blood flow and metabolism in high-turnover bone disease measured by [{sup 15}O]H{sub 2}O and [{sup 18}F]fluoride ion positron emission tomography

    Energy Technology Data Exchange (ETDEWEB)

    Piert, Morand [Nuclear Medicine, Klinikum Rechts der Isar, Technical University of Munich (Germany); Machulla, Hans-Juergen; Stahlschmidt, Anke; Becker, Georg A. [Radiopharmacy Section, PET Center, University of Tuebingen (Germany); Jahn, Michael; Zittel, Tilman T. [Department of Abdominal and Transplantation Surgery, University of Tuebingen (Germany)

    2002-07-01

    Previously, we identified a parathyroid hormone-related high-turnover bone disease after gastrectomy in mini pigs. Dynamic [{sup 18}F]fluoride ion positron emission tomography (PET) revealed that bone metabolism was significantly increased, but that bone blood flow derived from permeability-surface area product (PS product)-corrected K{sub 1} values was not. Since bone blood flow and metabolism are coupled in normal bone tissues, we hypothesised that the capillary permeability and/or surface area might be altered in high-turnover bone disease. The ''true'' bone blood flow (f{sub H2O}) was measured in vertebral bodies by dynamic [{sup 15}O]H{sub 2}O PET, followed by a 120-min dynamic [{sup 18}F]fluoride ion PET study, 6 months after total gastrectomy (n=5) and compared with results in sham-operated animals (n=5). Estimates for bone blood flow based on PS-corrected K{sub 1} values (f) and the net uptake of fluoride in bone tissue (K{sub i}), representing the bone metabolic activity, were calculated using standard compartmental modelling and non-linear fitting. Gastrectomy was followed by a significant elevation of K{sub i} and k{sub 3} (P<0.05), which was mainly caused by an increase of the fraction of bound tracer in tissue (P<0.01). In contrast, f{sub H2O}, f, the single-pass extraction fraction of [{sup 18}F]fluoride (E) and the volume of distribution (DV) of [{sup 18}F]fluoride were not significantly different between groups. In both groups, a coupling of the mean f{sub H2O} and K{sub i} values was found, but the intercept with the y-axis was higher in high-turnover bone disease. It is concluded that in high-turnover bone disease following gastrectomy, the PS product for [{sup 18}F]fluoride remains unchanged. Therefore, even in high-turnover bone diseases, [{sup 18}F]fluoride ion PET can provide reliable blood flow estimates (f), as long as a proper PS product correction is applied. The increased bone metabolism in high-turnover bone disease

  17. Coupling of porcine bone blood flow and metabolism in high-turnover bone disease measured by [15O]H2O and [18F]fluoride ion positron emission tomography

    International Nuclear Information System (INIS)

    Previously, we identified a parathyroid hormone-related high-turnover bone disease after gastrectomy in mini pigs. Dynamic [18F]fluoride ion positron emission tomography (PET) revealed that bone metabolism was significantly increased, but that bone blood flow derived from permeability-surface area product (PS product)-corrected K1 values was not. Since bone blood flow and metabolism are coupled in normal bone tissues, we hypothesised that the capillary permeability and/or surface area might be altered in high-turnover bone disease. The ''true'' bone blood flow (fH2O) was measured in vertebral bodies by dynamic [15O]H2O PET, followed by a 120-min dynamic [18F]fluoride ion PET study, 6 months after total gastrectomy (n=5) and compared with results in sham-operated animals (n=5). Estimates for bone blood flow based on PS-corrected K1 values (f) and the net uptake of fluoride in bone tissue (Ki), representing the bone metabolic activity, were calculated using standard compartmental modelling and non-linear fitting. Gastrectomy was followed by a significant elevation of Ki and k3 (PH2O, f, the single-pass extraction fraction of [18F]fluoride (E) and the volume of distribution (DV) of [18F]fluoride were not significantly different between groups. In both groups, a coupling of the mean fH2O and Ki values was found, but the intercept with the y-axis was higher in high-turnover bone disease. It is concluded that in high-turnover bone disease following gastrectomy, the PS product for [18F]fluoride remains unchanged. Therefore, even in high-turnover bone diseases, [18F]fluoride ion PET can provide reliable blood flow estimates (f), as long as a proper PS product correction is applied. The increased bone metabolism in high-turnover bone disease after gastrectomy is mainly related to an up-regulation of the amount of ionic exchange of [18F]fluoride with the bone matrix, while tracer delivery remains unchanged. (orig.)

  18. Genetic and Developmental Perspective of Language Abnormality in Autism and Schizophrenia: One Disease Occurring at Different Ages in Humans?

    Science.gov (United States)

    Wang, Haoran George; Jeffries, Joseph Joel; Wang, Tianren Frank

    2016-04-01

    Language and communication through it are two of the defining features of normally developed human beings. However, both these functions are often impaired in autism and schizophrenia. In the former disorder, the problem usually emerges in early childhood (~2 years old) and typically includes a lack of communication. In the latter condition, the language problems usually occur in adolescence and adulthood and presents as disorganized speech. What are the fundamental mechanisms underlying these two disorders? Is there a shared genetic basis? Are the traditional beliefs about them true? Are there any common strategies for their prevention and management? To answer these questions, we searched PubMed by using autism, schizophrenia, gene, and language abnormality as keywords, and we reconsidered the basic concepts about these two diseases or syndromes. We found many functional genes, for example, FOXP2, COMT, GABRB3, and DISC1, are actually implicated in both of them. After observing the symptoms, genetic correlates, and temporal progression of these two disorders as well as their relationships more carefully, we now infer that the occurrence of these two diseases is likely developmentally regulated via interaction between the genome and the environment. Furthermore, we propose a unified view of autism and schizophrenia: a single age-dependently occurred disease that is newly named as Systemic Integral Disorder: if occurring in children before age 2, it is called autism; if in adolescence or a later age, it is called schizophrenia. PMID:25686622

  19. Regulation of Sclerostin Expression in Multiple Myeloma by Dkk-1: A Potential Therapeutic Strategy for Myeloma Bone Disease.

    Science.gov (United States)

    Eda, Homare; Santo, Loredana; Wein, Marc N; Hu, Dorothy Z; Cirstea, Diana D; Nemani, Neeharika; Tai, Yu-Tzu; Raines, Sarah E; Kuhstoss, Stuart Allen; Munshi, Nikhil C; Kronenberg, Henry M; Raje, Noopur S

    2016-06-01

    Sclerostin is a potent inhibitor of osteoblastogenesis. Interestingly, newly diagnosed multiple myeloma (MM) patients have high levels of circulating sclerostin that correlate with disease stage and fractures. However, the source and impact of sclerostin in MM remains to be defined. Our goal was to determine the role of sclerostin in the biology of MM and its bone microenvironment as well as investigate the effect of targeting sclerostin with a neutralizing antibody (scl-Ab) in MM bone disease. Here we confirm increased sclerostin levels in MM compared with precursor disease states like monoclonal gammopathy of undetermined significance (MGUS) and smoldering MM. Furthermore, we found that a humanized MM xenograft mouse model bearing human MM cells (NOD-SCID.CB17 male mice injected intravenously with 2.5 million of MM1.S-Luc-GFP cells) demonstrated significantly higher concentrations of mouse-derived sclerostin, suggesting a microenvironmental source of sclerostin. Associated with the increased sclerostin levels, activated β-catenin expression levels were lower than normal in MM mouse bone marrow. Importantly, a high-affinity grade scl-Ab reversed osteolytic bone disease in this animal model. Because scl-Ab did not demonstrate significant in vitro anti-MM activity, we combined it with the proteasome inhibitor carfilzomib. Our data demonstrated that this combination therapy significantly inhibited tumor burden and improved bone disease in our in vivo MM mouse model. In agreement with our in vivo data, sclerostin expression was noted in marrow stromal cells and osteoblasts of MM patient bone marrow samples. Moreover, MM cells stimulated sclerostin expression in immature osteoblasts while inhibiting osteoblast differentiation in vitro. This was in part regulated by Dkk-1 secreted by MM cells and is a potential mechanism contributing to the osteoblast dysfunction noted in MM. Our data confirm the role of sclerostin as a potential therapeutic target in MM bone disease

  20. 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)

    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 D3, 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 Ca2+ 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.

  1. 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.

  2. HIV and Bone Disease: A Perspective of the Role of microRNAs in Bone Biology upon HIV Infection

    OpenAIRE

    Del Carpio-Cano, Fabiola E.; DeLa Cadena, Raul A.; Sawaya, Bassel E.

    2013-01-01

    Increased life expectancy and the need for long-term antiretroviral therapy have brought new challenges to the clinical management of HIV-infected individuals. The prevalence of osteoporosis and fractures is increased in HIV-infected patients; thus optimal strategies for risk management and treatment in this group of patients need to be defined. Prevention of bone loss is an important component of HIV care as the HIV population grows older. Understanding the mechanisms by which HIV infection...

  3. Treatment for Progressive Hearing Loss Due to Paget's Disease of Bone - A Case Report and Literature Review.

    Science.gov (United States)

    Aoki, Mitsuhiro; Tanahashi, Shigeaki; Mizuta, Keisuke; Kato, Hiroki

    2015-12-01

    Paget's disease is a common bone remodeling disorder that typically begins with excessive bone resorption in the elderly. Bilateral progressive hearing loss is the most frequently encountered complication of Paget's disease. The types of hearing loss identified by audiometry are conductive, sensorineural, or both. However, the precise mechanism of hearing loss remains unclear, and the treatment has been controversial. We present a 73-year-old man who suffered from bilateral progressive hearing loss due to Paget's disease. Potent bisphosphonates, oral risedronate in daily adjusted dosages for 6 months, did not decrease or suppress the worsening of the hearing loss. The Nucleus CI24 Contour electrode array was successfully inserted on the left side without surgical and postoperative complications. The Japanese open set monosyllable word recognition test in a sound field at 65 dB had a result of 74%. This cochlear implantation can be an indication for cases of profound hearing loss due to Paget's disease. PMID:26915163

  4. INFLUENCE OF VITAMIN D RECEPTOR GENE POLYMORPHISM ON THE EFFECTIVENESS OF α-D3 FOR RENAL BONE DISEASE

    Institute of Scientific and Technical Information of China (English)

    赵宝春; 傅淑霞; 戴秀华

    2002-01-01

    Objective To discuss the effectiveness of Vitamin D receptor (VDR)gene polymorphism on renal bone disease with α-D3.Methods VDR genotype was tested in 150 patients suffering from chronic renal failure using RFLPS method meanwhile the patients’ thyroxin in wholephase blood was assessed using radioactive and immune methods, and the bone specific mass (BUA) of right heel was detected by quantitative ultrasound before and eight weeks after the medication began.Results The incidence of the disease for BB type was 80%, for Bb was 60.87%, and for bb was 50. 5%. The difference of cure rates(P<0.05) was significant, being 100% for BB, 75.03% for Bb and 81.64% for bb.Conclusion The VDR genotype has important impact upon the occurrence of renal bone disease for chronic renal filure patients. Patients with BB genotype and b-equal grade have a low incidence rate for renal bone disease and those with α-D3 have good effects.

  5. 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

  6. Bone mineral metabolism in patients with neurofibromatosis type 1 (von Recklingausen disease).

    Science.gov (United States)

    Petramala, Luigi; Giustini, Sandra; Zinnamosca, Laura; Marinelli, Cristiano; Colangelo, Luciano; Cilenti, Giuseppina; Formicuccia, Maria Chiara; D'Erasmo, Emilio; Calvieri, Stefano; Letizia, Claudio

    2012-05-01

    The neurofibromatosis type 1 (NF1) is characterized by specific cutaneous features (neurofibromas, "café-au-lait" spots of the skin) and alterations of several tissue (nervous, vascular) and bone deformities, such as scoliosis, congenital pseudoarthrosis and bone dysplasia of tibia. Moreover, several studies have shown systemic involvement of bone tissue in NF1 patients, leading to reduced bone mass. The aim of our study was to evaluate some bone mineral metabolism parameters before and after calcium and vitamin D supplementation in NF1 patients. We evaluated in 70 NF1 consecutive patients the mineral metabolism and bone mineral density compared with 40 normal subjects. We showed bone alterations in 35% of patients and the increase of bone formation markers, such as bone isoenzyme of alkaline phosphatase (41.2 ± 15.5 vs. 25.6 ± 8.7 UI; P 60%). Moreover, we revealed a significant reduction of bone mass density at spine (L1-L4) (0.935 ± 0.13 vs. 1.110 ± 0.17 g/cm(2); P mineral bone involvement, with vitamin D deficiency; calcium and vitamin D supplementation is necessary to restore these bone mineral metabolic alterations. PMID:22120694

  7. Bone metabolism in patients with systemic lupus erythematosus. Effect of disease activity and glucocorticoid treatment

    DEFF Research Database (Denmark)

    Hansen, M; Halberg, P; Kollerup, G;

    1998-01-01

    The bone metabolism in patients with systemic lupus erythematosus (SLE) has previously been examined, but the results are conflicting. In the present study the bone mineral density (BMD) of the axial and the appendicular skeleton was examined by means of dual energy x-ray absorptiometry. The bone...

  8. PPTOX III: Environmental Stressors in the Developmental Origins of Disease - Evidence and Mechanisms

    DEFF Research Database (Denmark)

    Schug, Thaddeus T; Barouki, Robert; Gluckman, Peter D;

    2013-01-01

    Fetal and early postnatal development constitutes the most vulnerable time period of human life in regard to adverse effects of environmental hazards. Subtle effects during development can lead to functional deficits and increased disease risk later in life. The hypothesis stating that environmen......Fetal and early postnatal development constitutes the most vulnerable time period of human life in regard to adverse effects of environmental hazards. Subtle effects during development can lead to functional deficits and increased disease risk later in life. The hypothesis stating...

  9. Psychological and behavioral disease during developmental age: the importance of the alliance with parents

    Directory of Open Access Journals (Sweden)

    Michela Gatta

    2009-10-01

    Full Text Available Michela Gatta1, Elisabetta Ramaglioni3, Jessica Lai3, Lorenza Svanellini3,  Irene Toldo1, Lara Del Col3, Cinzia Salviato3,  Andrea Spoto2,  Battistella Pier Antonio31Paediatrics Department, 2Department of General Psychology, University of Padua, Padua, Italy; 3Neuropsychiatric Unit for Children and Adolescents, Azienda ULSS 16, Padua, ItalyAbstract: The aim of the study is to analyze the clinician’s alliance with parents during the diagnostic process in relation to therapeutic compliance and clinical evolution of individuals aged 0–11 years. The sample was formed by 84 individuals aged 0 to 11 years (18 < 6 years, 66 aged 6 to 11 years; 62 males and 22 females who came to the Neuropsychiatric Unit for Children and Adolescents for a consultation regarding psychorelational and behavioral problems. Neuropsychiatric consultation took place in five diagnostic interviews with child and parents, separately. The last session was devoted to communication of psychiatric diagnosis (according to ICD 10 and therapeutic suggestions, if any. The clinician’s relationship with parents and patients’ participation were evaluated in terms of collaboration and quality of interaction, on the basis of pre-established criteria. Data about patients’ therapeutic compliance and clinical outcome were collected during a follow-up visit eight months after the last session. Results suggest that the better the alliance between parents and clinician, the higher the therapeutic compliance and the likelihood of a positive outcome for patients. Our data suggest that good communication with parents benefits child patients, both in terms of response to the parents’ need to report their children’s worrying behavior and as a response to the discomfort expressed by children when they come in for consultation.Keywords: psychopathology, developmental age, psychotherapy, alliance relationship, parental function

  10. Developmental Dyspraxia

    Science.gov (United States)

    ... Enhancing Diversity Find People About NINDS NINDS Developmental Dyspraxia Information Page Synonym(s): Dyspraxia Table of Contents (click ... being done? Clinical Trials Organizations What is Developmental Dyspraxia? Developmental dyspraxia is a disorder characterized by an ...

  11. Normal Parathyroid Function with Decreased Bone Mineral Density in Treated Celiac Disease

    Directory of Open Access Journals (Sweden)

    Bernard Lemieux

    2001-01-01

    Full Text Available Decreased bone mineral density (BMD has been reported in patients with celiac disease in association with secondary hyperparathyroidism. The present study investigated whether basal parathyroid hormone (PTH remained elevated and whether abnormalities of parathyroid function were still present in celiac disease patients treated with a gluten-free diet. Basal seric measurements of calcium and phosphate homeostasis and BMD were obtained in 17 biopsy-proven patients under treatment for a mean period of 5.7±3.7 years (range 1.1 to 15.9. In addition, parathyroid function was studied with calcium chloride and sodium citrate infusions in seven patients. Basal measurements of patients were compared with those of 26 normal individuals, while parathyroid function results were compared with those of seven sex- and age-matched controls. Basal results were similar in patients and controls except for intact PTH (I-PTH (3.77±0.88 pmol/L versus 2.28±0.63 pmol/L, P<0.001, which was higher in the former group but still within normal limits. Mean 25-hydroxy vitamin D and 1,25-dihydroxy vitamin D values were normal in patients. Parathyroid function results were also found to be similar in both groups. Compared with a reference population of the same age (Z score, patients had significantly lower BMDs of the hip (-0.60±0.96 SDs, P<0.05 and lumbar spine (-0.76±1.15 SDs, P<0.05. T scores were also decreased for the hip (-1.3±0.9 SDs, P<0.0001 and lumbar spine (-1.4±1.35 SDs, P<0.0001, with two to three patients being osteoporotic (T score less than -2.5 SDs and seven to eight osteopenic (T score less than -1 SDs but greater than or equal to -2.5 SDs in at least one site. Height and weight were the only important determinants of BMD values by multivariate or logistical regression analysis in these patients. The results show higher basal I-PTH values with normal parathyroid function in treated celiac disease. Height and weight values are, but I-PTH values are not

  12. Epidemiological study of Paget's disease of bone in a zone of the Province of Salamanca (spain)

    International Nuclear Information System (INIS)

    Bone Paget's disease is heterogeneously distributed and several foci of high prevalence have been reported in Spain. The aim of the present work was to determine the prevalence of the disease in a zone situated in the northwestern sector of the Province of Salamanca (Spain) using a cross sectional epidemiological study. Sample choice was based on a stratified sampling according to the residence, age and sex of the inhabitants of the zone. A sampling error of 5% and a confidence level of 95% were considered; these afforded a sample of 378 units. Final choice of the subjects was based on a random pathway method. Data collection was accomplished with a personal interview using a 31-item questionnaire and analytical screening (AP and GGT). The field work was carried out over a one-year period. The data were input onto a calculation sheet for analysis and epidemiological interpretation. Finally, clinico-radiological confirmation of the cases deemed positive in the screening was accomplished. The prevalence of PBD in the zone studied is 5.7% (95% CI: 4.5-6.9). The highest percentage of patients lies within the age group between 70-79 years; most of these patients were women. The mean residence time in the zone was 66 years. According to the findings, this geographic zone has a high prevalence of PBD

  13. Quality Indices Of Jaw Bone Tissue In Screening Diagnostics Of Parodontal Diseases

    Directory of Open Access Journals (Sweden)

    V.P. Konev

    2009-12-01

    Full Text Available The objective of the work is to develop and motivate screening methods of the estimation of paradontal tissues by digital X-ray study. The used methods are X-ray digital scene with estimation of bone tissue density in program Trophy 2000, clinically anatomical parallels of bone tissue density and their qualitative indices. Parallels between qualitative indices of bones of mandibular alveolar process and bone tissue density of these areas have been revealed. It is reasonable to carry out the estimation of bone tissue density indices for revealing early stages of parodontal pathology

  14. Bone scan in rheumatology

    International Nuclear Information System (INIS)

    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

  15. 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......-resolution peripheral quantitative tomography (HR-pQCT) was used to assess bone structure at the non-dominant distal radius and tibia. Estimated bone strength was calculated using finite element analysis. Biochemical markers of bone turnover were measured in all participants. RESULTS: After adjusting for BMI, MVD......+ patients displayed lower cortical volumetric BMD (P=0.02) and cortical thickness (P=0.02) and higher cortical porosity at the radius (P=0.02) and a trend towards higher cortical porosity at the tibia (P=0.07) compared to controls. HR-pQCT parameters did not differ between MVD- and control subjects...

  16. Paradoxical acute hypercalcemic effect of salmon calcitonin in patients having Paget's disease of bone after treatment with dichloromethylene diphosphonate.

    Science.gov (United States)

    Delmas, P D; Chapuy, M C; Meunier, P J

    1984-05-01

    The hypocalcemia following administration of calcitonin may be an index to disease activity in Paget's disease of bone. Therefore, we assessed the effect of a single injection of 100 MRC units of salmon calcitonin (SCT) on plasma calcium in 28 patients with active Paget's disease before and after 6 months of treatment with dichloromethylene diphosphonate (Cl2MDP) at a dose of 400 mg/day (3 patients), 800 mg/day (8 patients), 1.600 mg/day (9 patients) or 2.600 mg/day (8 patients). The mean SCT-induced hypocalcemia was reduced by Cl2MDP and there was a significant positive correlation between the decrease of serum calcium induced by SCT and bone resorption evaluated by the number of osteoclasts on bone biopsy taken in pagetic iliac crest. After Cl2MCP treatment, 5 patients manifested a paradoxical hypercalcemic response to SCT injection ranging from +0.3 mg/dl to +0.5 mg/dl, which was sustained over the 9 hours following injection. As these patients had a dramatic inhibition of bone resorption induced by Cl2 MDP, it is suggested that the hypercalcemic response to SCT might reflect persistence or exaggeration of the early hypercalcemic effect of CT which reportedly precedes the hypocalcemic response to SCT. PMID:6234216

  17. Intracerebroventricular transplanted bone marrow stem cells survive and migrate into the brain of rats with Parkinson’s disease

    Institute of Scientific and Technical Information of China (English)

    Ping Gu; Zhongxia Zhang; Dongsheng Cui; Yanyong Wang; Lin Ma; Yuan Geng; Mingwei Wang

    2012-01-01

    In this study, 6-hydroxydopamine was stereotaxically injected into the right substantia nigra compact and ventral tegmental area of rats to establish Parkinson’s disease models. The rats then received a transplantation of bone marrow stromal cells that were previously isolated, cultured and labeled with 5-bromo-2’-deoxyuridine in vitro. Transplantation of the bone marrow stromal cells significantly decreased apomorphine-induced rotation time and the escape latency in the Morris water maze test as compared with rats with untreated Parkinson’s disease. Immunohistochemical staining showed that, 5-bromo-2’-deoxyuridine-immunoreactive cells were present in the lateral ventricular wall and the choroid plexus 1 day after transplantation. These immunoreactive cells migrated to the surrounding areas of the lateral cerebral ventricle along the corpus callosum. The results indicated that bone marrow stromal cells could migrate to tissues surround the cerebral ventricle via the cerebrospinal fluid circulation and fuse with cells in the brain, thus altering the phenotype of cells or forming neuron-like cells or astrocytes capable of expressing neuron-specific proteins. Taken together, the present findings indicate that bone marrow stromal cells transplanted intracerebroventricularly could survive, migrate and significantly improve the rotational behavior and cognitive function of rats with experimentally induced Parkinson’s disease.

  18. Bone marrow abnormalities and early bone lesions in multiple myeloma and its precursor disease: a prospective study using functional and morphologic imaging.

    Science.gov (United States)

    Bhutani, Manisha; Turkbey, Baris; Tan, Esther; Korde, Neha; Kwok, Mary; Manasanch, Elisabet E; Tageja, Nishant; Mailankody, Sham; Roschewski, Mark; Mulquin, Marcia; Carpenter, Ashley; Lamping, Elizabeth; Minter, Alex R; Weiss, Brendan M; Mena, Esther; Lindenberg, Liza; Calvo, Katherine R; Maric, Irina; Usmani, Saad Z; Choyke, Peter L; Kurdziel, Karen; Landgren, Ola

    2016-05-01

    The incidence and importance of bone marrow involvement and/or early bone lesions in multiple myeloma (MM) precursor diseases is largely unknown. This study prospectively compared the sensitivity of several imaging modalities in monoclonal gammopathy of undetermined significance (MGUS), smoldering multiple myeloma (SMM) and MM. Thirty patients (10 each with MGUS, SMM and MM) were evaluated with skeletal survey, [(18)F]FDG-PET/CT, [(18)F]NaF-PET/CT and morphologic dynamic contrast enhanced (DCE)-MRI. An additional 16 SMM patients had skeletal surveys and FDG-PET/CT. Among MGUS patients, DCE-MRI found only one focal marrow abnormality; other evaluations were negative. Among 26 SMM patients, five (19%) were re-classified as MM based on lytic bone lesions on CT and six had unifocal or diffuse marrow abnormality. Among MM, marrow abnormalities were observed on FDG-PET/CT in 8/10 patients and on DCE-MRI in nine evaluable patients. Abnormal NaF uptake was observed only in MM patients with lytic lesions on CT, providing no additional clinical information. PMID:26690712

  19. A New Data Analysis System to Quantify Associations between Biochemical Parameters of Chronic Kidney Disease-Mineral Bone Disease.

    Directory of Open Access Journals (Sweden)

    Mariano Rodriguez

    Full Text Available In hemodialysis patients, deviations from KDIGO recommended values of individual parameters, phosphate, calcium or parathyroid hormone (PTH, are associated with increased mortality. However, it is widely accepted that these parameters are not regulated independently of each other and that therapy aimed to correct one parameter often modifies the others. The aim of the present study is to quantify the degree of association between parameters of chronic kidney disease and mineral bone disease (CKD-MBD.Data was extracted from a cohort of 1758 adult HD patients between January 2000 and June 2013 obtaining a total of 46.141 records (10 year follow-up. We used an advanced data analysis system called Random Forest (RF which is based on self-learning procedure with similar axioms to those utilized for the development of artificial intelligence. This new approach is particularly useful when the variables analyzed are closely dependent to each other.The analysis revealed a strong association between PTH and phosphate that was superior to that of PTH and Calcium. The classical linear regression analysis between PTH and phosphate shows a correlation coefficient is 0.27, p<0.001, the possibility to predict PTH changes from phosphate modification is marginal. Alternatively, RF assumes that changes in phosphate will cause modifications in other associated variables (calcium and others that may also affect PTH values. Using RF the correlation coefficient between changes in serum PTH and phosphate is 0.77, p<0.001; thus, the power of prediction is markedly increased. The effect of therapy on biochemical variables was also analyzed using this RF.Our results suggest that the analysis of the complex interactions between mineral metabolism parameters in CKD-MBD may demand a more advanced data analysis system such as RF.

  20. Crohn's Disease and Primary Alveolar Bone Loss A literature Review and Case Report

    Institute of Scientific and Technical Information of China (English)

    HUANG Xing-xing; ZHANG Wen-feng; CHEN Xin-min; ZHAO Yi-fa

    2006-01-01

    Crohn's disease is a granulomatous inflammatory bowel disease of unknown cause. Intraoral tissues can be affected occasionally. Oral lesions most frequently appear in lips, gingival tissue, and buccal mucosa. We reported here a case of a 24-year-old man with a 6-month history of mouth ulceration and 2-month history of occasional obdominal pain. Histopathological diagnosis was Crohn's disease. Clinical examinations revealed established intestinal lesions, mouth ulcerations, thickening of the antral mucosa and an unusual pattern presentation rarely previously reported in the literature: generalized alveolar bone loss without obvious periodontitis and history of steroids use.%克罗恩病是一种病因尚不明确的慢性肠道炎性肉芽肿性疾病.偶有口腔组织受累,损害主要见于唇、颊黏膜及牙龈.本文报道一例有口腔病变的克罗恩病,24岁,男性,口腔溃疡不愈半年,伴有2月腹痛史.口腔溃疡活检病理诊断为克罗恩病.临床检查发现除了消化道病损、口腔溃疡、上颌窦黏膜增厚外,出现了罕见的广泛牙槽骨吸收,而患者无牙周病及激素用药史,这一特点在以前的文献中未见报道.

  1. 99mTc-sestamibi and 99mTc-tetrofosmin scintigraphy in myeloma bone disease

    International Nuclear Information System (INIS)

    Full text: Evaluation of the role of scintigraphy with 99mTc-Sestamibi (99mTc-MIBI) and 99mTc-Tetrofosmin (99mTc-TF) in the detection of bone marrow involvement in patients (pts) with multiple myeloma (MM) and in follow-up. 62 pts with MM and 38 pts with monoclonal gammopathy of undetermined significance (MGUS) were enrolled in this study. Forty-seven out sixty-two MM pts had active disease (AD), 10/62 were in complete remission (CR) and 5/62 in partial remission (PR) after chemotherapy. Whole-body scans were obtained 10 min after the i.v. injection of 740 MBq of 99mTc-MIBI in anterior and posterior view. The scans were scored semiquantitatively according to extension and intensity of tracer uptake. All 38 MGUS pts had a negative 99mTc-MIBI scan. As to the MM pts, 49/62 pts (44 with AD, 4 with PR and 1 with CR) had a positive 99mTc-MIBI scan, while the 99mTc-MIBI scan was negative in 13/62 pts (9 with CR, 1 with PR and 3 with AD). The overall sensitivity of the scintigraphic procedure was 92 % while specificity was 97 %. A total of 42 follow-up scans with 99mTc-MIBI were performed in 30 MM pts after high-dose chemotherapy, with an average follow-up duration of 14.6 ± 8.9. The follow-up 99mTc-MIBI scintigraphy was positive in 14/15 of the AD pts, while 6/8 negative scans were observed in the CR pts; the seven PR pts exhibited in five cases a negative scan and in two a mild scintigrafic positivity. The overall sensitivity and specificity of the follow-up scan were, respectively, 86 % and 75 %. In six patients, one at the diagnosis and five showing a positive 99mTc-MIBI scan during the follow-up, 740 MBq of 99mTc-TF was administered within two days with the same acquisition protocol. The scans provided substantially identical information. 99mTc-TF provided a mildly higher contrast between lesion and background activities. The results obtained in this study provide additional evidence indicating that 99mTc-MIBI scintigraphy closely reflects myeloma disease activity in

  2. Health and developmental origins of disease%健康和疾病的发育起源

    Institute of Scientific and Technical Information of China (English)

    李君; 李婷

    2013-01-01

      20世纪90年代,基于临床流行病学的一些发现,英国学者David Barker教授最早提出健康和疾病的发育起源这一概念。目前发现,几乎所有的成人慢性疾病,例如代谢性疾病、心血管疾病、肿瘤等都与发育早期的环境有关。这个假说得到了大量临床流行病学和动物实验等基础研究的证实。它的意义在于,在临床上,有这样一个“窗口期”,通过合理的规范孕妇的营养和行为,以及合理的出生后早期营养,能够避免胎儿和婴儿暴露于不良的环境中,从而降低他们成年后发生慢性疾病的风险。因此健康和疾病的发育起源这一概念有现实的临床意义。%ABSTRACT Based on some findings of clinical epidemiology, the British professor David Barker proposed the concept of the developmental origins of health and disease (DOHaD) in the 1990s. Almost all the chronic diseases, such as metabolic diseases, cardiovascular diseases, cancers, are proved to be related with the early stage of development. DOHaD has been verified by the clinical epidemiological researches and animal experiments. It is important to know that we have a“window“in the early stage, in which, if we care the pregnant women or new babies carefully to avoid them from harmful environment, we can reduced the morbidity of adult chronic disease of these babies when they grow up. Therefore, DOHaD has a clinical significance.

  3. Predictors of bone disease in Egyptian prepubertal children with β-thalassaemia major

    OpenAIRE

    Tantawy, Azza A.G.; El-Bostany, Eman A.; Matter, Randa M; El-Ghoroury, Eman A.; Ragab, Shadia

    2010-01-01

    Introduction Thalassaemic osteopathy is a multifactorial disorder and limited information exists about bone accrual and bone mineral density (BMD) in prepubertal thalassaemic children. The study aimed to investigate some potential genetic and biochemical bone markers as possible early predictors of BMD variations in children with β-thalassaemia major (TM) before puberty. Material and methods Thirt-one prepubertal children with β-TM, and 43 matched controls were subjected to BMD assessment by ...

  4. Femoral head vascularisation in Legg-Calve-Perthes disease: comparison of dynamic gadolinium-enhanced subtraction MRI with bone scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Lamer, Sylvie; Dorgeret, Sophie; Brillet, Pierre-Yves; Hassan, Max; Sebag, Guy H. [Department of Paediatric Radiology, Hopital Robert Debre, 48 boulevard Serurier, 75935 Paris Cedex (France); Lariboisiere-Saint-Louis University, Paris (France); Khairouni, Abdeslam; Mazda, Keyvan; Bacheville, Eric; Pennecot, Georges F. [Department of Paediatric Orthopaedics, Hopital Robert Debre, Paris (France); Bloch, Juliette [Department of Biostatistics, Hopital Robert Debre, Paris (France)

    2002-08-01

    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.)

  5. Bone marrow transplantation in the rat. I. Histologic correlations and quantitation of cellular infiltrates in acute graft-versus-host disease.

    OpenAIRE

    Renkonen, R; Häyry, P.

    1984-01-01

    In an attempt to define which cytologic manifestations of inflammation are characteristic of acute graft-versus-host disease (aGVHD), the authors have analyzed hematologic reconstitution in the bone marrow, spleen, and blood of bone marrow transplant recipients and correlated these events to concomitant cytologic changes in the parenchymal target organs. After bone marrow transplantation from Lewis to BN strain, the strongest inflammatory changes were observed in the liver, a "model" parenchy...

  6. Radiographic evaluation of the effect of obesity on alveolar bone in rats with ligature-induced periodontal disease

    Directory of Open Access Journals (Sweden)

    do Nascimento CM

    2013-10-01

    Full Text Available Cassiane Merigo do Nascimento,1 Tiago Cassol,2 Fernanda Soares da Silva,3 Maria Lucia Bonfleur,4 Carlos Augusto Nassar,5 Patricia Oehlmeyer Nassar5 1Biologica Science and Health Center, State University of West Paraná (UNIOESTE, Cascavel, Paraná, Brazil; 2State University of West Paraná (UNIOESTE, Cascavel, Paraná, Brazil; Department of 3Pharmacy, 4Fisiology, 5Periodontology, Dental School, State University of West Paraná (UNIOESTE, Cascavel, Paraná, Brazil Abstract: There is evidence that the lack of metabolic control of obese patients may accelerate periodontitis. The aim of this study was to evaluate radiographically the effect of cafeteria-diet-induced obesity on alveolar bone loss in rats subjected to periodontal disease. Twenty male Wistar rats were randomly divided into four groups: 1 control group, 2 control and ligature group; 3 cafeteria group; and 4 cafeteria and ligature group. The animals were evaluated for obesity and euthanized, and the mandible of each rat was removed to perform a radiographic evaluation of alveolar bone loss and its effect on diet-induced obesity. The results showed greater alveolar bone loss in the mice in Group 4 (P<0.01. Thus, we concluded that obese mice, on average, showed greater radiographic evidence of alveolar bone loss than mice undergoing induction of obesity. Keywords: periodontal disease, radiography, obesity

  7. Temporary brittle bone disease: relationship between clinical findings and judicial outcome

    Directory of Open Access Journals (Sweden)

    Colin R. Paterson

    2011-10-01

    Full Text Available There is a wide differential diagnosis for the child with unexplained fractures including non-accidental injury, osteogenesis imperfecta and vitamin D deficiency rickets. Over the last 20 years we and others have described a self-limiting syndrome characterised by fractures in the first year of life. This has been given the provisional name temporary brittle bone disease. This work had proved controversial mostly because the fractures, including rib fractures and metaphyseal fractures, were those previously regarded as typical or even diagnostic of non-accidental injury. Some have asserted that the condition does not exist. Over the years 1985 to 2000 we investigated 87 such cases with fractures with a view to determining the future care of the children. In 85 of these the judiciary was involved. We examined the clinical and radiological findings in the 33 cases in which there was a judicial finding of abuse, the 24 cases in which the parents were exonerated and the 28 cases in which no formal judicial finding was made. The three groups of patients were similar in terms of demographics, age at fracturing and details of the fractures. The clinical similarities between the three groups of patients contrasts with the very different results of the judicial process.

  8. Dietary phosphorus excess: a risk factor in chronic bone, kidney, and cardiovascular disease?

    Science.gov (United States)

    Uribarri, Jaime; Calvo, Mona S

    2013-01-01

    There is growing evidence in the nephrology literature supporting the deleterious health effect of excess dietary phosphorus intake. This issue has largely escaped the attention of nutrition experts until this symposium, which raised the question of whether the same health concerns should be extended to the general population. The potential hazard of a high phosphorus intake in the healthy population is illustrated by findings from acute and epidemiologic studies. Acute studies in healthy young adults demonstrate that phosphorus intakes in excess of nutrient needs may significantly disrupt the hormonal regulation of phosphorus contributing to disordered mineral metabolism, vascular calcification, bone loss, and impaired kidney function. One of the hormonal factors acutely affected by dietary phosphorus loading is fibroblast growth factor-23, which may be a key factor responsible for many of the cardiovascular disease (CVD) complications of high phosphorus intake. Increasingly, large epidemiological studies suggest that mild elevations of serum phosphorus within the normal range are associated with CVD risk in healthy populations. Few population studies link high dietary phosphorus intake to mild changes in serum phosphorus due to study design issues specific to phosphorus and inaccurate nutrient composition databases. The increasing phosphorus intake due to the use of phosphorus-containing ingredients in processed food and the growing consumption of processed convenience and fast foods is an important factor that needs to be emphasized. PMID:24038251

  9. 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.

  10. On the way to building an integrated computational environment for the study of developmental patterns and genetic diseases.

    Science.gov (United States)

    Turinsky, Andrei L; Sensen, Christoph W

    2006-01-01

    Genetic diseases and developmental patterns should be studied on several levels: from macroscale (organs and tissues) to nanoscale (cells, genes, proteins). Due to the overwhelming complexity of the life science data, it is common that disparate data pieces are meticulously stored but never fully analyzed or correlated. We have begun to develop a novel methodology based on virtual reality techniques for the study of these phenomena. Our key approach to knowledge integration is a top-down mapping of data onto visual contexts. For each organism that we want to study, a structural model is created and used as a visual "wireframe" onto which other data types are superimposed in a top-down assembly. Data analysis tools, visual controls, and queries are enabled so that users can interactively explore data. Our visualization technology gives users an opportunity to map disparate data onto a common model, and search visually for hitherto unknown patterns and correlations contained within the data. It is our goal to eventually transform genomics research from measuring various data pieces analytically into a fully interactive exploration of combined data in a 4D immersive visual environment that best matches a researcher's intuition.

  11. Evidence that asthma is a developmental origin disease influenced by maternal diet and bacterial metabolites.

    Science.gov (United States)

    Thorburn, Alison N; McKenzie, Craig I; Shen, Sj; Stanley, Dragana; Macia, Laurence; Mason, Linda J; Roberts, Laura K; Wong, Connie H Y; Shim, Raymond; Robert, Remy; Chevalier, Nina; Tan, Jian K; Mariño, Eliana; Moore, Rob J; Wong, Lee; McConville, Malcolm J; Tull, Dedreia L; Wood, Lisa G; Murphy, Vanessa E; Mattes, Joerg; Gibson, Peter G; Mackay, Charles R

    2015-01-01

    Asthma is prevalent in Western countries, and recent explanations have evoked the actions of the gut microbiota. Here we show that feeding mice a high-fibre diet yields a distinctive gut microbiota, which increases the levels of the short-chain fatty acid, acetate. High-fibre or acetate-feeding led to marked suppression of allergic airways disease (AAD, a model for human asthma), by enhancing T-regulatory cell numbers and function. Acetate increases acetylation at the Foxp3 promoter, likely through HDAC9 inhibition. Epigenetic effects of fibre/acetate in adult mice led us to examine the influence of maternal intake of fibre/acetate. High-fibre/acetate feeding of pregnant mice imparts on their adult offspring an inability to develop robust AAD. High fibre/acetate suppresses expression of certain genes in the mouse fetal lung linked to both human asthma and mouse AAD. Thus, diet acting on the gut microbiota profoundly influences airway responses, and may represent an approach to prevent asthma, including during pregnancy. PMID:26102221

  12. Effects of recombinant human erythropoietin on the haemopoietic bone marrow monitored by magnetic resonance spectroscopy in patients with end-stage renal disease

    DEFF Research Database (Denmark)

    Jensen, K E; Stenver, D; Jensen, M;

    1990-01-01

    Volume selective magnetic resonance (MR) proton spectroscopy was used to investigate changes in the haemopoietic bone marrow in patients with end-stage renal disease undergoing treatment with recombinant human erythropoietin (rHuEPO). Significant changes could be detected in the spectra 14 days a......, as well as investigating bone marrow response from different modes of rHuEPO administration....

  13. The effects of untreated and treated HIV infection on bone disease.

    LENUS (Irish Health Repository)

    Cotter, Aoife G

    2013-11-20

    Low bone mineral density (BMD) is common in those with HIV, associated with higher bone turnover and a higher prevalence of fractures. This review explores low BMD in HIV, focusing on underlying mechanisms and relationships between low BMD and HIV infection, immune dysfunction, and antiretroviral therapy (ART).

  14. Osseous and Nonosseous Bone Scan Findings in Liver Transplant Candidates with end-stage Chronic Liver Disease

    OpenAIRE

    Seval Erhamamcı; Ayşe Aktaş; Tatiana Bahçeci; Kevser Kavak

    2013-01-01

    Objective: End-stage chronic liver disease (CLD) adversely affects the function of multiple organ systems including the skeletal system. The aim of this study was to assess osseous and nonosseous bone scintigraphy (BS) findings in liver transplant (LT) candidates with end-stage CLD. Methods: We retrospectively evaluated BS findings in 50 consecutive patients with end-stage CLD who were undergoing preoperative assessment for LT from January 2006 to December 2011. All the patients were analyzed...

  15. Bone disease in patients with haemophilia A and B--where are we now?

    Science.gov (United States)

    Anagnostis, P; Karras, S N; Goulis, D G

    2015-01-01

    It is evident that haemophilia A and B are associated with decreased bone mass in both adults and children. Decreased physical activity and vitamin D deficiency are some of the major factors leading to bone loss. Hepatitis C virus (HCV) infection may also contribute to low bone mineral density (BMD). However, definite conclusions regarding the exact prevalence and pathogenesis of osteoporosis cannot be conducted yet, due to the small sample size and significant heterogeneity among studies. Discordant findings with regard to the skeletal site of low BMD have also been reported. Furthermore, data on fracture risk are sparse. The use of the Fracture Risk Assessment Tool (FRAX) for assessing fracture risk, regular BMD assessment at the age of 25 and thereafter, careful evaluation of risk factors associated with bone loss and optimal calcium and vitamin D intake are recommended. Long-term prophylactic factor replacement therapy, resistance exercise and bisphosphonates, in severe cases of increased fracture risk, can prevent bone loss. PMID:25251867

  16. Computed tomography of the inner ear: size of anatomical structures in the normal temporal bone and in the temporal bone of patients with Meniere's disease

    Energy Technology Data Exchange (ETDEWEB)

    Krombach, Gabriele A.; Boom, Martin van den; Schmitz-Rode, Thomas; Guenther, Rolf W.; Wildberger, Joachim E. [University of Technology (RWTH) Aachen, Department of Diagnostic Radiology, University Hospital, Aachen (Germany); Martino, Ercole Di; Westhofen, Martin [University of Technology (RWTH) Aachen, Department of Otorhinolaryngology, Aachen (Germany); Prescher, Andreas [University of Technology (RWTH) Aachen, Institute of Anatomy, Aachen (Germany)

    2005-08-01

    The purpose of the study was to obtain reference values for the sizes of anatomical structures of the inner ear on computed tomography (CT) images and to compare these values with those obtained from patients with Meniere's disease. CT images of the temporal bone of 67 patients without inner ear pathology and 53 patients with Meniere's disease have been evaluated. CT was performed in the sequential mode (1-mm slice thickness, 120 kV, 125 mA). Anatomical structures, such as the length and the width of the cochlea and of the vestibule, the height of the basal turn, the length and the width of the cochlear, the vestibular and the singular aqueduct and the internal auditory meatus and the diameter of the semicircular canals, were measured, using a dedicated postprocessing workstation. Reference values from the control group could be obtained. In the patients with Meniere's disease, the length and the width of the vestibular aqueduct were smaller, compared with the values from the control group. The values obtained from the control group can serve as reference values for adult patients. The different sizes of anatomical structures of the control group and of patients suffering from Meniere's disease suggest that functional impairment might be related to subtle morphological changes. (orig.)

  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)

    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. The management of metastatic bone disease with the combination of bisphosphonates and radiotherapy: from theory to clinical practice.

    Science.gov (United States)

    Vassiliou, V; Kardamakis, D

    2009-03-01

    Bone metastases are common in the event of malignancy and are inevitably associated with serious complications that may deteriorate the quality of life (QOL) of patients and threaten life. Both radiotherapy (RT) and bisphosphonates (BPs) have an established role in the management of metastatic bone disease. Many clinical trials have demonstrated their effectiveness when used as sole treatment modalities, but only a few have evaluated their therapeutic value when applied concomitantly. We herein discuss the pathophysiology of bone metastases and the potential interactions between RT and BPs. Moreover, the results of both animal models and clinical studies are presented in detail. Apart from aspects of normal tissue tolerance, other interactions include spatial cooperation and additive or super-additive effects. The latter brings about a synergistic activity that results in an enhanced reossification, improved bone stability and microarchitecture, and increased mechanical strength, as documented through animal model studies. The results of published clinical studies investigating the effectiveness of concomitant application of RT and BPs are promising, reporting a significant clinical and radiologic response. More specifically, a significant reduction of pain scores and a worth noticing improvement in QOL and performance status (PS) were noted, accompanied by a considerable increase in bone density. Pain relief was accompanied by a marked reduction in analgesic opioid need. The enhanced reossification may be responsible for the improved therapeutic response, since it was shown that the correlation between pain and bone density is negative and strong. Although promising and encouraging, the results of such studies should be corroborated by larger, randomized trials.

  20. Genetic background impacts developmental potential of enteric neural crest-derived progenitors in the Sox10Dom model of Hirschsprung disease.

    Science.gov (United States)

    Walters, Lauren C; Cantrell, V Ashley; Weller, Kevin P; Mosher, Jack T; Southard-Smith, E Michelle

    2010-11-15

    Abnormalities in the development of enteric neural crest-derived progenitors (ENPs) that generate the enteric nervous system (ENS) can lead to aganglionosis in a variable portion of the distal gastrointestinal tract. Cumulative evidence suggests that variation of aganglionosis is due to gene interactions that modulate the ability of ENPs to populate the intestine; however, the developmental processes underlying this effect are unknown. We hypothesized that differences in enteric ganglion deficits could be attributable to the effects of genetic background on early developmental processes, including migration, proliferation, or lineage divergence. Developmental processes were investigated in congenic Sox10(Dom) mice, an established Hirschsprung disease (HSCR) model, on distinct inbred backgrounds, C57BL/6J (B6) and C3HeB/FeJ (C3Fe). Immuno-staining on whole-mount fetal gut tissue and dissociated cell suspensions was used to assess migration and proliferation. Flow cytometry utilizing the cell surface markers p75 and HNK-1 was used to isolate live ENPs for analysis of developmental potential. Frequency of ENPs was reduced in Sox10(Dom) embryos relative to wild-type embryos, but was unaffected by genetic background. Both migration and developmental potential of ENPs in Sox10(Dom) embryos were altered by inbred strain background with the most highly significant differences seen for developmental potential between strains and genotypes. In vivo imaging of fetal ENPs and postnatal ganglia demonstrates that altered lineage divergence impacts ganglia in the proximal intestine. Our analysis demonstrates that genetic background alters early ENS development and suggests that abnormalities in lineage diversification can shift the proportions of ENP populations and thus may contribute to ENS deficiencies in vivo.

  1. The role of 18F–NaF PET/CT in metastatic bone disease

    Directory of Open Access Journals (Sweden)

    Mine Araz

    2015-09-01

    Conclusion: Although further prospective clinical studies in specific cancer populations are indicated to set the place of 18F–NaF PET/CT in diagnostic scheme, the results of this pilot study from our country support the superiority of 18F–NaF PET/CT in investigation of bone metastasis over 99mTc-MDP bone scan and 18F-FDG PET/CT in various malignancies. 18F–NaF PET/CT is coming forward as a single step bone seeking study, considering all the advantages, but especially potential of detecting occult metastases and reliably directing patient management.

  2. Myeloma bone and extra-medullary disease: Role of PET/CT and other whole-body imaging techniques.

    Science.gov (United States)

    Rubini, Giuseppe; Niccoli-Asabella, Artor; Ferrari, Cristina; Racanelli, Vito; Maggialetti, Nicola; Dammacco, Francesco

    2016-05-01

    Multiple myeloma (MM) is the second most common hematological malignancy. Although it can affect different organs, the bone compartment stands out both in terms of prevalence and clinical impact. Despite the striking advances in MM therapy, bone disease can remarkably affect the patient's quality of life. The occurrence and extension of bone marrow and extra-medullary involvement should be carefully assessed to confirm the diagnosis, to locate and whenever possible prevent dreadful complications such as pathological fractures and spinal cord compression, and to establish suitable therapeutic measures. Many imaging techniques have been proposed for the detection of MM skeletal involvement. With the development of more sophisticated imaging tools, it is time to use the right technique at the right time. Based on the review of the literature and our own experience, this article discusses advantages and disadvantages of the different imaging methods in the work-up of MM patients, with particular emphasis on the role that PET/CT can play. It is emphasized that whole body low-dose computed tomography should be the preferred imaging technique at baseline. However, bone marrow infiltration and extra-medullary manifestations are better detected by whole body magnetic resonance imaging. Positron emission tomography/computed tomography, on the other hand, combines the benefits of the two mentioned imaging procedures and is particularly useful not only for the detection of osteolytic lesions unrevealed by conventional X-ray, but also in the assessment of prognosis and therapeutic response. PMID:26997302

  3. High serum YKL-40 concentration is associated with severe bone disease in newly diagnosed multiple myeloma patients

    DEFF Research Database (Denmark)

    Mylin, Anne Kærsgaard; Abildgaard, N.; Johansen, J.S.;

    2008-01-01

    higher total X-ray score (P = 0.003) and higher levels of the markers of bone resorption serum C-terminal telopeptide of collagen type I (P = 0.003), urine pyridinoline (P = 0.04) and urine deoxypyridinoline (P = 0.002), while the levels of urine N-terminal telopeptide of collagen type I (NTX-1) and the...... markers of bone formation equalled those seen in patients with a normal serum YKL-40. Serum YKL-40 level (beta = 7.6; P = 0.002) and urine NTX-1 (log(2)) (beta = 3.5; P = 0.006) were independent predictors of total X-ray score at diagnosis. Patients with elevated serum YKL-40 at diagnosis had shorter time......Objectives: In multiple myeloma (MM) YKL-40 is present in the bone marrow microenvironment and is suggested to play a role in remodelling of the extracellular matrix. Here, the association between serum YKL-40 and severity of bone disease in MM is investigated. Methods: Serum YKL-40 was measured in...

  4. Natural history of malignant bone disease in breast cancer and the use of cumulative mean functions to measure skeletal morbidity

    Directory of Open Access Journals (Sweden)

    Smith Matthew R

    2009-08-01

    Full Text Available Abstract Background Bone metastases are a common cause of skeletal morbidity in patients with advanced cancer. The pattern of skeletal morbidity is complex, and the number of skeletal complications is influenced by the duration of survival. Because many patients with cancer die before trial completion, there is a need for survival-adjusted methods to accurately assess the effects of treatment on skeletal morbidity. Methods Recently, a survival-adjusted cumulative mean function model has been generated that can provide an intuitive graphic representation of skeletal morbidity throughout a study. This model was applied to the placebo-control arm of a pamidronate study in patients with malignant bone disease from breast cancer. Results Analysis by bone lesion location showed that spinal metastases were associated with the highest cumulative mean incidence of skeletal-related events (SREs, followed by chest and pelvic metastases. Metastases located in the extremities were associated with an intermediate incidence of SREs, and those in the skull were associated with the lowest incidence of SREs. Conclusion Application of this model to data from the placebo arm of this trial revealed important insight into the natural history of skeletal morbidity in patients with bone metastases. Based on these observations, treatment for the prevention of SREs is warranted regardless of lesion location except for metastases on the skull.

  5. Pre-B cell colony enhancing factor/NAMPT/visfatin and its role in inflammation-related bone disease

    Energy Technology Data Exchange (ETDEWEB)

    Moschen, Alexander R.; Geiger, Sabine; Gerner, Romana [Christian Doppler Research Laboratory for Gut Inflammation and Department of Internal Medicine II (Gastroenterology and Hepatology), Innsbruck Medical University, Anichstrasse 35, 6020 Innsbruck (Austria); Tilg, Herbert, E-mail: herbert.tilg@i-med.ac.at [Christian Doppler Research Laboratory for Gut Inflammation and Department of Internal Medicine II (Gastroenterology and Hepatology), Innsbruck Medical University, Anichstrasse 35, 6020 Innsbruck (Austria)

    2010-08-07

    Chronic inflammation affects bone metabolism and is commonly associated with the presence of osteoporosis. Bone loss is directed by various immune mediators such as the pro-inflammatory cytokines tumour necrosis factor-alpha, interleukin 1-beta or interferon-gamma. Pre-B cell colony enhancing factor (PBEF)/nicotinamide phosphoribosyl transferase (NAMPT)/visfatin is a pleiotropic mediator acting as growth factor, cytokine and enzyme involved in energy and nicotinamide adenine dinucleotide (NAD) metabolism. PBEF/NAMPT/visfatin has been recently demonstrated to exert several pro-inflammatory functions. We studied serum levels of PBEF/NAMPT/visfatin in patients with inflammatory bowel diseases (IBD) and their relation with bone mineral density (BMD). Furthermore, we were interested whether PBEF/NAMPT/visfatin affects osteoclastogenesis and involved mediators. PBEF/NAMPT/visfatin serum levels were increased in patients with IBD, correlated positively with disease activity and negatively with BMD, especially in the lumbar spine. Osteoclast precursor cells were generated from peripheral blood mononuclear cells after stimulation with various growth factors such as macrophage colony-stimulating factor (M-CSF) and soluble ligand of receptor activator of nuclear factor kappa B (RANK). In these in vitro studies, PBEF/NAMPT/visfatin suppressed osteoclastogenesis and inhibited the differentiation of osteoclast precursors into tartrate-resistant acid phosphatase positive multinucleated cells. These effects were paralleled by the suppression of the osteoclast typical markers RANK, nuclear factor of activated T-cells c1 (NFATc1) and cathepsin-K. This is the first report demonstrating a potential role for this important cytokine/enzyme in inflammation-related bone disease.

  6. 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

  7. Multiple bone and joint disease in a sickle cell anaemia patient: a case report

    OpenAIRE

    John Ayodele Olaniyi; Alagbe, Adekunle E.; Olutoogun O Toluwalase; Olorunsogo E Busari

    2012-01-01

    Objective: To further highlight the fact that bone involvement is the commonest clinical manifestation of SCD both in the acute settins such painful VOC and as a source of chronic , progressive debility such as AVN, chronic osteomyelitis and fixed flexion deformity of joints. Protracted multiple bone involvement i.e. Bilateral femoral and left humeral chronic osteomyelitis, Left elbow, Left knee and right humeral septic arthritis together with aseptic necrosis of both femoral and right hum...

  8. Disease-modifying antirheumatic drugs and bone mass in rheumatoid arthritis.

    Science.gov (United States)

    Di Munno, O; Delle Sedie, A; Rossini, M; Adami, S

    2005-01-01

    This article reviews the effects of DMARDs (including biologic agents) on bone metabolism in rheumatoid arthritis (RA). At present there is no evidence that methotrexate, at least at dosages ranging from 5 to 20 mg/week, negatively affects bone mass as measured by DXA (BMD) as documented in both cross-sectional and longitudinal studies. Most studies of cyclosporine (CyA) use reporting a reduction in erosions and joint damage with no adverse effects on bone, did not measure BMD; CyA treatment is associated with a dose-dependent increase of bone turnover as well as a decrease in both animal and human studies; however, its use in RA setting at a dose < or =5 mg/Kg/ day has so far not been associated with clinical relevant adverse effects on bone metabolism. Anti-TNF-alpha agents, infliximab reduced markers of bone turnover in two longitudinal studies. Data on BMD are not available in RA; nevertheless, an increase in BMD has been documented in spondyloarthropathies with infliximab and etanercept. No clinical data concerning BMD are available on leflunomide as well as on the newer biologic agents (adalimumab, rituximab, anakinra).

  9. 团头鲂肌间骨发育的形态学观察%DEVELOPMENTAL AND MORPHOLOGICAL OBSERVATION OF INTERMUSCULAR BONES IN MEGALOBRAMA AMBLYCEPHALA

    Institute of Scientific and Technical Information of China (English)

    万世明; 易少奎; 仲嘉; 王卫民; 蒋恩明; 陈柏湘; 高泽霞

    2014-01-01

    To investigate the emergent periods and morphogenesis of Megalobrama amblycephala intermuscular bones, the modified bone clearing and X-ray transmission methods were conducted to study the number, morphology, distribu-tion and length of intermuscular bone of M. amblycephala with different sizes. The results showed that initial inter-muscular bones were ossified at 20 days post hatching (dph) with the body length of 1.33 cm, which first appeared in the tail and then turned toward the head. When the fries were 40 dph with the body length of 2.36 cm, all the intermuscular bones were basically appeared. The average number of intermuscular bones of M. amblycephala was 119 with the range from 108 to 129. The number of intermuscular bones in both body sides was not absolutely equal, although the number of each side was quite close. There were more intermuscular bones in the front dorsal part than rear part, and the number of intermuscular bones was almost equal in rear dorsal and rear abdominal parts. There were 6 kinds of intermuscular bones, including “1” “卜” “y”, one-end-multifork, two-end-multifork, and “(” types. Various types of intermuscular bones were evolved from the“1”shape, and more complex shapes were formed in the front part of body. The length of intermuscular bones in front dorsal part was significantly longer than that in tail part (P<0.05), while the intermuscular bones in the front abdominal part were shorter than others. The length of intermuscular bones of M. amblycephala was positively related with the body weight and body length. The emergence and differentiation of intermuscular bones was more related to the body size than to the age in M. amblycephala. The result from this study will contribute to under-stand the molecular mechanisms of intermuscular bone occurrence and development, the research on the inhibition of the intermuscular bone ossification as well as the breeding of M. amblycephala without intermuscular bone.%研

  10. Relationship of Dickkopf1 (DKK1 with cardiovascular disease and bone metabolism in Caucasian type 2 diabetes mellitus.

    Directory of Open Access Journals (Sweden)

    Antonia Garcia-Martín

    Full Text Available OBJECTIVES: Dickkopf-1 (DKK1 is a potent inhibitor of Wnt signalling, which exerts anabolic effects on bone and also takes part in the regulation of vascular cells. Our aims were to evaluate serum DKK1 in type 2 diabetes (T2DM patients and to analyze its relationships with cardiovascular disease (CVD. We also evaluated the relationship between DKK1 and bone metabolism. DESIGN: We conducted a cross-sectional study in which we measured serum DKK1 (ELISA, Biomedica in 126 subjects: 72 patients with T2DM and 54 non-diabetic subjects. We analysed its relationship with clinical CVD, preclinical CVD expressed as carotid intima media thickness (IMT, and bone metabolism. RESULTS: T2DM patients with CVD (P = 0,026 and abnormal carotid IMT (P = 0,038 had higher DKK1 concentrations. DKK1 was related to the presence of CVD in T2DM, independently of the presence of risk factors for atherosclerosis. Therefore, for each increase of 28 pg/ml of serum DKK1 there was a 6,2% increase in the risk of CVD in T2DM patients. The ROC curve analysis to evaluate the usefulness of DKK1 as a marker for high risk of CVD showed an area under the curve of 0,667 (95% CI: 0,538-0,795; P = 0,016. In addition, there was a positive correlation between serum DKK1 and spine bone mineral density in the total sample (r =  0,183; P = 0,048. CONCLUSION: In summary, circulating DKK1 levels are higher in T2DM with CVD and are associated with an abnormal carotid IMT in this cross-sectional study. DKK1 may be involved in vascular disease of T2DM patients.

  11. Meat and bone meal and mineral feed additives may increase the risk of oral prion disease transmission

    Science.gov (United States)

    Johnson, Christopher J.; McKenzie, Debbie; Pedersen, Joel A.; Aiken, Judd M.

    2011-01-01

    Ingestion of prion-contaminated materials is postulated to be a primary route of prion disease transmission. Binding of prions to soil (micro)particles dramatically enhances peroral disease transmission relative to unbound prions, and it was hypothesized that micrometer-sized particles present in other consumed materials may affect prion disease transmission via the oral route of exposure. Small, insoluble particles are present in many substances, including soil, human foods, pharmaceuticals, and animal feeds. It is known that meat and bone meal (MBM), a feed additive believed responsible for the spread of bovine spongiform encephalopathy (BSE), contains particles smaller than 20 μm and that the pathogenic prion protein binds to MBM. The potentiation of disease transmission via the oral route by exposure to MBM or three micrometer-sized mineral feed additives was determined. Data showed that when the disease agent was bound to any of the tested materials, the penetrance of disease was increased compared to unbound prions. Our data suggest that in feed or other prion-contaminated substances consumed by animals or, potentially, humans, the addition of MBM or the presence of microparticles could heighten risks of prion disease acquisition.

  12. MRI of the wrist and finger joints in inflammatory joint diseases at 1-year interval: MRI features to predict bone erosions

    Energy Technology Data Exchange (ETDEWEB)

    Savnik, Anette [Department of Rheumatology, Parker Institute, Frederiksberg Hospital, Copenhagen (Denmark); Department of Radiology, University Hospital at Herlev, Copenhagen (Denmark); Department of Radiology, Frederiksberg Hospital, Copenhagen (Denmark); Malmskov, Hanne; Graff, Lykke B.; Danneskiold-Samsoee, Bente; Bliddal, Henning [Department of Rheumatology, Parker Institute, Frederiksberg Hospital, Copenhagen (Denmark); Thomsen, Henrik S. [Department of Radiology, University Hospital at Herlev, Copenhagen (Denmark); Nielsen, Henrik [Department of Rheumatology, University Hospital at Herlev, Copenhagen (Denmark); Boesen, Jens [Department of Radiology, Frederiksberg Hospital, Copenhagen (Denmark)

    2002-05-01

    The aim of this study was to assess the ability of MRI determined synovial volumes and bone marrow oedema to predict progressions in bone erosions after 1 year in patients with different types of inflammatory joint diseases. Eighty-four patients underwent MRI, laboratory and clinical examination at baseline and 1 year later. Magnetic resonance imaging of the wrist and finger joints was performed in 22 patients with rheumatoid arthritis less than 3 years (group 1) who fulfilled the American College of Rheumatology (ACR) criteria for rheumatoid arthritis, 18 patients with reactive arthritis or psoriatic arthritis (group 2), 22 patients with more than 3 years duration of rheumatoid arthritis, who fulfilled the ACR criteria for rheumatoid arthritis (group 3), and 20 patients with arthralgia (group 4). The volume of the synovial membrane was outlined manually before and after gadodiamide injection on the T1-weighted sequences in the finger joints. Bones with marrow oedema were summed up in the wrist and fingers on short-tau inversion recovery sequences. These MRI features was compared with the number of bone erosions 1 year later. The MR images were scored independently under masked conditions. The synovial volumes in the finger joints assessed on pre-contrast images was highly predictive of bone erosions 1 year later in patients with rheumatoid arthritis (groups 1 and 3). The strongest individual predictor of bone erosions at 1-year follow-up was bone marrow oedema, if present at the wrist at baseline. Bone erosions on baseline MRI were in few cases reversible at follow-up MRI. The total synovial volume in the finger joints, and the presence of bone oedema in the wrist bones, seems to be predictive for the number of bone erosions 1 year later and may be used in screening. The importance of very early bone changes on MRI and the importance of the reversibility of these findings remain to be clarified. (orig.)

  13. Effect of age and disease on bone mass in Japanese patients with schizophrenia

    Directory of Open Access Journals (Sweden)

    Sugawara Norio

    2012-02-01

    Full Text Available Abstract Background There have been a limited number of studies comparing bone mass between patients with schizophrenia and the general population. The aim of this study was to compare the bone mass of schizophrenia patients with that of healthy subjects in Japan. Methods We recruited patients (n = 362, aged 48.8 ± 15.4 (mean ± SD years who were diagnosed with schizophrenia or schizoaffective disorder based on the Diagnostic and Statistical Manual of Mental Disorders, fourth edition (DSM-IV. Bone mass was measured using quantitative ultrasound densitometry of the calcaneus. The osteosono-assessment index (OSI was calculated as a function of the speed of sound and the transmission index. For comparative analysis, OSI data from 832 adults who participated in the Iwaki Health Promotion Project 2009 was used as representative of the general community. Results Mean OSI values among male schizophrenic patients were lower than those in the general population in the case of individuals aged 40 and older. In females, mean OSI values among schizophrenic patients were lower than those in the general community in those aged 60 and older. In an analysis using the general linear model, a significant interaction was observed between subject groups and age in males. Conclusions Older schizophrenic patients exhibit lower bone mass than that observed in the general population. Our data also demonstrate gender and group differences among schizophrenic patients and controls with regard to changes in bone mass associated with aging. These results indicate that intervention programs designed to delay or prevent decreased bone mass in schizophrenic patients might be tailored according to gender.

  14. Haematological toxicity of radiotherapy following high-dose chemotherapy and autologous bone marrow transplantation in patients with recurrent Hodgkin's disease

    International Nuclear Information System (INIS)

    17 patients with recurrent Hodgkin's disease received 21 courses of radiotherapy (RT) 1-23 months after high-dose chemotherapy and autologous bone marrow transplantation. WHO grade III-IV haematological toxicity, of median duration 38 days (range 4-236), was observed following 10 courses of radiotherapy in 9 patients. This haematological morbidity could be predicted with an 80.0% sensitivity when the pre-RT white cell count was 9/1 or the platelet count 9/1. It occurred to 9/11 patients with initial stage III-IV disease, including all 6 given extended radiotherapy fields, but in no patients with initial stage II disease (χ2 = 9.35, P < 0.005). Age, histology, the presence of B symptoms, performance status, previous radiotherapy or chemotherapy, the interval between autologous bone marrow transplantations and radiotherapy, the high-dose regimen used, and the radiotherapy dose or field size, did not appear to affect haematological toxicity. The median survival was 18 months from the date of starting radiotherapy. (author)

  15. Inflammatory diseases of the petrous portion of the temporal bone; Entzuendliche Erkrankungen des Felsenbeins

    Energy Technology Data Exchange (ETDEWEB)

    Koerner, H. [Universitaetsklinikum des Saarlandes, Klinik fuer Diagnostische und Interventionelle Neuroradiologie, Homburg/Saar (Germany)

    2014-04-15

    Inflammatory lesions of the petrous portion of the temporal bone are very common and can be followed by cerebral complications. Thin layer computed tomography (CT) is useful for detecting bony changes of the temporal bone and contrast-enhanced magnetic resonance imaging (CE MRI) is a sensitive method for detecting cerebral complications. (orig.) [German] Entzuendliche Erkrankungen des Felsenbeins sind sehr haeufig und koennen mit zerebralen Komplikationen einhergehen. Mit der Duennschicht-CT lassen sich sehr gut ossaere Veraenderungen im Felsenbein nachweisen. Die kontrastmittelgestuetzte MRT ist sensitiv fuer zerebrale Komplikationen. (orig.)

  16. Smoking and Bone Health

    Science.gov (United States)

    ... It has been called a childhood disease with old age consequences because building healthy bones in youth helps ... stronger. Weight-bearing exercise that forces you to work against gravity is the best exercise for bone. ...

  17. Assessment of Bone Mineral Density in Male Patients with Chronic Obstructive Pulmonary Disease by DXA and Quantitative Computed Tomography

    OpenAIRE

    George Fountoulis; Theodora Kerenidi; Constantinos Kokkinis; Panagiotis Georgoulias; Paschal Thriskos; Konstantinos Gourgoulianis; Ioannis Fezoulidis; Katerina Vassiou; Marianna Vlychou

    2016-01-01

    The purpose of this study is to identify the prevalence of osteoporosis in male patients with chronic obstructive pulmonary disease (COPD) by dual-energy X-ray absorptiometry (DXA) and quantitative computed tomography (QCT) and to compare the diagnostic abilities of the above methods. Thirty-seven male patients with established COPD were examined with DXA and standard QCT in lumbar spine, including L1, L2, and L3 vertebrae. T-scores and bone mineral density values were calculated by DXA and Q...

  18. Feasibility and safety of autologous bone marrow mononuclear cell transplantation in patients with advanced chronic liver disease

    Institute of Scientific and Technical Information of China (English)

    Andre Castro Lyra; Bernd Genser; Ricardo Ribeiro dos Santos; Luiz Guilherme Costa Lyra; Milena Botelho Pereira Soares; Luiz Flavio Maia da Silva; Marcos Fraga Fortes; André Goyanna Pinheiro Silva; Augusto César de Andrade Mota; Sheilla A Oliveira; Eduardo Lorens Braga; Wilson Andrade de Carvalho

    2007-01-01

    AIM:To evaluate the safety and feasibility of bone marrow cell(BMC)transplantation in patients with chronic liver disease on the waiting list for liver transplantation.METHODS:Ten patients(eight males)with chronic liver disease were enrolled to receive infusion of autologous bone marrow-derived cells.Seven patients were classified as Child-Pugh B and three as Child-Pugh C.Baseline assessment included complete clinical and laboratory evaluation and abdominal MRI.Approximately 50 mL of bone marrow aspirate was prepared by centrifugation in a ficoll-hypaque gradient.At least of 100 millions of mononuclear-enriched BMCs were infused into the hepatic artery using the routine technique for arterial chemoembolization for liver tumors.Patients were followed up for adverse events up to 4 mo.RESULTS:The median age of the patients was 52 years(range 24-70 years).All patients were discharged 48 h after BMC infusion.Two patients complained of mild pain at the bone marrow needle puncture site.No other complications or specific side effects related to the procedure were observed.Bilirubin levels were lower at 1(2.19 ± 0.9)and 4 mo(2.10 ± 1.0)after cell transplantation that baseline levels(2.78 ± 1.2).Albumin levels 4 mo after BMC infusion(3.73 ± 0.5)were higher than baseline levels(3.47 ± 0.5).International normalized ratio(INR)decreased from 1.48(SD = 0.23)to 1.43(SD = 0.23)one month after cell transplantation.CONCLUSION:BMC infusion into hepatic artery of patients with advanced chronic liver disease is safe and feasible.In addition,a decrease in mean serum bilirubin and INR levels and an increase in albumin levels are observed.Our data warrant further studies in order to evaluate the effect of BMC transplantation in patients with advanced chronic liver disease.

  19. Defective Neutrophil Recruitment in Leukocyte Adhesion Deficiency Type I Disease Causes Local IL-17-Driven Inflammatory Bone Loss.

    OpenAIRE

    Moutsopoulos, N. M.; Konkel, J.; Sarmadi, M.; Eskan, M. A.; Wild, T.; N Dutzan; L Abusleme; Zenobia, C; Hosur, K. B.; Abe, T.; Uzel, G.; Chen, W.; Chavakis, T.; Holland, S.M.; Hajishengallis, G

    2014-01-01

    Leukocyte adhesion deficiency Type I (LAD-I), a disease syndrome associated with frequent microbial infections, is caused by mutations on the CD18 subunit of β2 integrins. LAD-I is invariably associated with severe periodontal bone loss, historically attributed to lack of neutrophil surveillance of the periodontal infection. Here, we challenge this dogma by showing that the cytokine IL-17 plays a major role in the oral pathology of LAD-I. Defective neutrophil recruitment in LAD-I patients, or...

  20. Bone scintigraphy in the diagnosis, prognosis, and follow-up of Legg-Calve-Perthes' disease

    Energy Technology Data Exchange (ETDEWEB)

    Bok, B.; Cavailloles, F.; Lonchampt, M.F. (Hopital Beaujon, 92 - Clichy (France)); Bensahel, H. (Hopital Bretonneau, 75 - Paris (France))

    1983-12-01

    Bone scintigraphy using high resolution pinhole collimation is of high diagnostic value in the early diagnosis of Legg-Calve-Perthes' disease in children with hip pain or limp. False positive as well as false-negative are very unusual, and scintigraphy is of help in the early management of these patients. There is also evidence of interesting data in the early prognosis and in the follow-up of these patients, but the exact role of these studies has not been clearly established yet.

  1. Mathematical modeling of bone marrow--peripheral blood dynamics in the disease state based on current emerging paradigms, part I.

    Science.gov (United States)

    Afenya, Evans K; Ouifki, Rachid; Camara, Baba I; Mundle, Suneel D

    2016-04-01

    Stemming from current emerging paradigms related to the cancer stem cell hypothesis, an existing mathematical model is expanded and used to study cell interaction dynamics in the bone marrow and peripheral blood. The proposed mathematical model is described by a system of nonlinear differential equations with delay, to quantify the dynamics in abnormal hematopoiesis. The steady states of the model are analytically and numerically obtained. Some conditions for the local asymptotic stability of such states are investigated. Model analyses suggest that malignancy may be irreversible once it evolves from a nonmalignant state into a malignant one and no intervention takes place. This leads to the proposition that a great deal of emphasis be placed on cancer prevention. Nevertheless, should malignancy arise, treatment programs for its containment or curtailment may have to include a maximum and extensive level of effort to protect normal cells from eventual destruction. Further model analyses and simulations predict that in the untreated disease state, there is an evolution towards a situation in which malignant cells dominate the entire bone marrow - peripheral blood system. Arguments are then advanced regarding requirements for quantitatively understanding cancer stem cell behavior. Among the suggested requirements are, mathematical frameworks for describing the dynamics of cancer initiation and progression, the response to treatment, the evolution of resistance, and malignancy prevention dynamics within the bone marrow - peripheral blood architecture. PMID:26877072

  2. Metabolic bone disease in lion cubs at the London Zoo in 1889: the original animal model of rickets.

    Science.gov (United States)

    Chesney, Russell W; Hedberg, Gail

    2010-08-24

    In 1889 Dr. John Bland-Sutton, a prominent London surgeon, was consulted about fatal rickets in over 20 successive litters of lion cubs born at the London Zoo. He evaluated the diet and found the cause of rickets to be nutritional in origin. He recommended that goat meat with crushed bones and cod-liver oil be added to the lean horsemeat diet of the cubs and their mothers. Rickets were reversed, the cubs survived, and subsequent litters thrived. Thirty years later, in classic controlled studies conducted in puppies and young rats, the definitive role of calcium, phosphate and vitamin D in prevention and therapy of rickets was elucidated. Further studies led to identifying the structural features of vitamin D.Although the Bland-Sutton diet provided calcium and phosphate from bones and vitamins A and D from cod-liver oil, some other benefits of this diet were not recognized. Taurine-conjugated bile salts, necessary for intestinal absorption of fat-soluble vitamins, were provided in the oil cold-pressed from cod liver. Unlike canine and rodent species, felines are unable to synthesize taurine, yet conjugate bile acids exclusively with taurine; hence, it must be provided in the diet. The now famous Bland-Sutton "experiment of nature," fatal rickets in lion cubs, was cured by addition of minerals and vitamin D. Taurine-conjugated bile salts undoubtedly permitted absorption of vitamins A and D, thus preventing the occurrence of metabolic bone disease and rickets.

  3. Dual photon absorptiometry in patients with Paget disease (bone mineral contents of lumbar spine and femoral neck)

    International Nuclear Information System (INIS)

    The bone mineral contents (BMC) of the lumbar spine (84 cases) and of one femoral neck (53 cases) were measured by dual photon absorptiometry (DPA) in patients suffering from Paget's disease of bone. The BMC of the patients and of 53 controls were compared, taking into account the occurrence of a scintigraphic hyperfixation, in each patient. A hyperfixation of the technetium labelled diphosphonate in the lumbar spine, more frequent in males, was associated with an elevated mean BMC value. The difference versus controls was significant in men but not in women. A hyperfixation of the femoral neck was associated with an elevated mean BMC value in both sexes. BMC values greater than the mean BMC of controls + 2 SD were observed in 83 per cent of these male patients and 50 per cent of the females. In contrast, BMC values lower than in controls were observed in non-pagetic areas. DPA allows the quantification of these abnormalities which cannot be evidenced by bone scintigraphy even if alkaline phosphatases levels are assayed

  4. Renal bone disease and extraskeletal calcification during dialysis and after transplantation

    International Nuclear Information System (INIS)

    The author reports 10 studies concerning the diagnosis of renal osteodystrophy and extraskeletal calcification in patients on maintenance hemodialysis as well as some aspects of persistent hyperparathyroidism after renal transplantation. The majority of the studies focus on the value of bone scintigraphy with Tc-99m HEDP in the diagnosis of these disorders. (Auth.)

  5. Reliability analysis of instrument design of noninvasive bone marrow disease detector

    Science.gov (United States)

    Su, Yu; Li, Ting; Sun, Yunlong

    2016-02-01

    Bone marrow is an important hematopoietic organ, and bone marrow lesions (BMLs) may cause a variety of complications with high death rate and short survival time. Early detection and follow up care are particularly important. But the current diagnosis methods rely on bone marrow biopsy/puncture, with significant limitations such as invasion, complex operation, high risk, and discontinuous. It is highly in need of a non-invasive, safe, easily operated, and continuous monitoring technology. So we proposed to design a device aimed for detecting bone marrow lesions, which was based on near infrared spectrum technology. Then we fully tested its reliabilities, including the sensitivity, specificity, signal-to-noise ratio (SNR), stability, and etc. Here, we reported this sequence of reliability test experiments, the experimental results, and the following data analysis. This instrument was shown to be very sensitive, with distinguishable concentration less than 0.002 and with good linearity, stability and high SNR. Finally, these reliability-test data supported the promising clinical diagnosis and surgery guidance of our novel instrument in detection of BMLs.

  6. Bone Marrow and Kidney Transplant for Patients With Chronic Kidney Disease and Blood Disorders

    Science.gov (United States)

    2016-10-03

    Chronic Kidney Disease; Acute Myeloid Leukemia (AML); Acute Lymphoblastic Leukemia (ALL); Chronic Myelogenous Leukemia (CML); Chronic Lymphocytic Leukemia (CLL); Non-Hodgkin's Lymphoma (NHL); Hodgkin Disease; Multiple Myeloma; Myelodysplastic Syndrome (MDS); Aplastic Anemia; AL Amyloidosis; Diamond Blackfan Anemia; Myelofibrosis; Myeloproliferative Disease; Sickle Cell Anemia; Autoimmune Diseases; Thalassemia

  7. NOD/SCID-GAMMA mice are an ideal strain to assess the efficacy of therapeutic agents used in the treatment of myeloma bone disease.

    Science.gov (United States)

    Lawson, Michelle A; Paton-Hough, Julia M; Evans, Holly R; Walker, Rebecca E; Harris, William; Ratnabalan, Dharshi; Snowden, John A; Chantry, Andrew D

    2015-01-01

    Animal models of multiple myeloma vary in terms of consistency of onset, degree of tumour burden and degree of myeloma bone disease. Here we describe five pre-clinical models of myeloma in NOD/SCID-GAMMA mice to specifically study the effects of therapeutic agents on myeloma bone disease. Groups of 7-8 week old female irradiated NOD/SCID-GAMMA mice were injected intravenously via the tail vein with either 1x106 JJN3, U266, XG-1 or OPM-2 human myeloma cell lines or patient-derived myeloma cells. At the first signs of morbidity in each tumour group all animals were sacrificed. Tumour load was measured by histological analysis, and bone disease was assessed by micro-CT and standard histomorphometric methods. Mice injected with JJN3, U266 or OPM-2 cells showed high tumour bone marrow infiltration of the long bones with low variability, resulting in osteolytic lesions. In contrast, mice injected with XG-1 or patient-derived myeloma cells showed lower tumour bone marrow infiltration and less bone disease with high variability. Injection of JJN3 cells into NOD/SCID-GAMMA mice resulted in an aggressive, short-term model of myeloma with mice exhibiting signs of morbidity 3 weeks later. Treating these mice with zoledronic acid at the time of tumour cell injection or once tumour was established prevented JJN3-induced bone disease but did not reduce tumour burden, whereas, carfilzomib treatment given once tumour was established significantly reduced tumour burden. Injection of U266, XG-1, OPM-2 and patient-derived myeloma cells resulted in less aggressive longer-term models of myeloma with mice exhibiting signs of morbidity 8 weeks later. Treating U266-induced disease with zoledronic acid prevented the formation of osteolytic lesions and trabecular bone loss as well as reducing tumour burden whereas, carfilzomib treatment only reduced tumour burden. In summary, JJN3, U266 or OPM-2 cells injected into NOD/SCID-GAMMA mice provide robust models to study anti-myeloma therapies

  8. NOD/SCID-GAMMA mice are an ideal strain to assess the efficacy of therapeutic agents used in the treatment of myeloma bone disease.

    Directory of Open Access Journals (Sweden)

    Michelle A Lawson

    Full Text Available Animal models of multiple myeloma vary in terms of consistency of onset, degree of tumour burden and degree of myeloma bone disease. Here we describe five pre-clinical models of myeloma in NOD/SCID-GAMMA mice to specifically study the effects of therapeutic agents on myeloma bone disease. Groups of 7-8 week old female irradiated NOD/SCID-GAMMA mice were injected intravenously via the tail vein with either 1x106 JJN3, U266, XG-1 or OPM-2 human myeloma cell lines or patient-derived myeloma cells. At the first signs of morbidity in each tumour group all animals were sacrificed. Tumour load was measured by histological analysis, and bone disease was assessed by micro-CT and standard histomorphometric methods. Mice injected with JJN3, U266 or OPM-2 cells showed high tumour bone marrow infiltration of the long bones with low variability, resulting in osteolytic lesions. In contrast, mice injected with XG-1 or patient-derived myeloma cells showed lower tumour bone marrow infiltration and less bone disease with high variability. Injection of JJN3 cells into NOD/SCID-GAMMA mice resulted in an aggressive, short-term model of myeloma with mice exhibiting signs of morbidity 3 weeks later. Treating these mice with zoledronic acid at the time of tumour cell injection or once tumour was established prevented JJN3-induced bone disease but did not reduce tumour burden, whereas, carfilzomib treatment given once tumour was established significantly reduced tumour burden. Injection of U266, XG-1, OPM-2 and patient-derived myeloma cells resulted in less aggressive longer-term models of myeloma with mice exhibiting signs of morbidity 8 weeks later. Treating U266-induced disease with zoledronic acid prevented the formation of osteolytic lesions and trabecular bone loss as well as reducing tumour burden whereas, carfilzomib treatment only reduced tumour burden. In summary, JJN3, U266 or OPM-2 cells injected into NOD/SCID-GAMMA mice provide robust models to study anti

  9. Non-traumatic evaluation of bone mineral by single and dual photon absorptiometry implications for medical research and diagnosis of skeletal disease

    International Nuclear Information System (INIS)

    Specific requirements for the non-traumatic measurements of bone mineral mass can be defined as follows: First, for longitudinal measurements high precision or reproducibility is required. There should be few obstacles to repeated measurements such as radiation dose, cost, patient involvement, and there should be little room for systematic error such as positioning. Second, for the detection of osteopenia or bone loss, accuracy is very important and a good normal population study for comparison. Third, the method should be of sufficient sensitivity to detect clinically significant bone loss. This is about 1% per year in normal women and may be up to 10% in the postmenopausal period in women with osteoporosis. Thus, changes of bone mineral of 3% to 5% should be detected. Fourth, the method should allow separate measurements of regional, predominantly cortical, and trabecular bone sites. Radioscopy, radiographic morphometry, radiographic photo-densitometry are generally not sensitive enough for present day demands but have been used successfully in the past for the definition of marked abnormalities and disease. Radionuclide uptake or neutron activation analysis give results in the entire skeleton and do not allow separation of cortical and trabecular bone sites. CT scanning and Compton scattering methods are presently being developed. Their potential of being developed to clinically-practical tests is not clear today. At this time, single and dual photon absorptiometry are the best practical methods to study regional bone mineral mass of predominantly cortical and trabecular bone sites with good sensitivity, accuracy and precision

  10. Amelioration of murine sickle cell disease by nonablative conditioning and γ-globin gene-corrected bone marrow cells.

    Science.gov (United States)

    Pestina, Tamara I; Hargrove, Phillip W; Zhao, Huifen; Mead, Paul E; Smeltzer, Matthew P; Weiss, Mitchell J; Wilber, Andrew; Persons, Derek A

    2015-01-01

    Patients with severe sickle cell disease (SCD) are candidates for gene therapy using autologous hematopoietic stem cells (HSCs), but concomitant multi-organ disease may contraindicate pretransplant conditioning with full myeloablation. We tested whether nonmyeloablative conditioning, a regimen used successfully for allogeneic bone marrow transplantation of adult SCD patients, allows engraftment of γ-globin gene-corrected cells to a therapeutic level in the Berkeley mouse model of SCD. Animals transplanted according to this regimen averaged 35% engraftment of transduced hematopoietic stem cells with an average vector copy liver, spleen, and kidneys. Thus, modest levels of chimerism with donor cells expressing high levels of HbF from an insulated γ-globin lentiviral vector can improve the pathology of SCD in mice, thereby illustrating a potentially safe and effective strategy for gene therapy in humans. PMID:26665131

  11. Low Infection Rate after Tumor Hip Arthroplasty for Metastatic Bone Disease in a Cohort Treated with Extended Antibiotic Prophylaxis

    Directory of Open Access Journals (Sweden)

    Werner H. Hettwer

    2015-01-01

    Full Text Available Background. Compared to conventional hip arthroplasty, endoprosthetic reconstruction after tumor resection is associated with a substantially increased risk of periprosthetic joint infection (PJI, with reported rates of around 10% in a recent systematic review. The optimal duration of antibiotic prophylaxis for this patient population remains unknown. Material and Methods. To establish the infection rate associated with prolonged antibiotic prophylaxis in our department, we performed a retrospective review of all adult patients who underwent endoprosthetic reconstruction of the proximal femur after tumor resection for metastatic bone disease during a 4-year period from 2010 to 2013 (n=105 patients. Results. Intravenous antibiotic prophylaxis was administrated for an extended duration of a mean of 7.4 days. The overall infection rate was 3.6% (4/111 implants, infection free survival was 96% at 2 years, and the risk of amputation associated with infection was 25% (1/4 patients. Discussion. Preemptive eradication of bacterial contamination may be of value in certain clinical situations, where the risk level and consequences of implant-associated infection are unacceptable. Our findings suggest that extended postoperative antibiotic prophylaxis may reduce the risk of PJI in patients undergoing tumor resection and endoprosthetic replacement for metastatic bone disease associated impending or de facto pathologic fractures of the proximal femur.

  12. Low Infection Rate after Tumor Hip Arthroplasty for Metastatic Bone Disease in a Cohort Treated with Extended Antibiotic Prophylaxis

    Science.gov (United States)

    Hettwer, Werner H.; Horstmann, Peter Frederik; Hovgaard, Thea Bechmann; Grum-Scwensen, Tomas Andreas; Petersen, Michael M.

    2015-01-01

    Background. Compared to conventional hip arthroplasty, endoprosthetic reconstruction after tumor resection is associated with a substantially increased risk of periprosthetic joint infection (PJI), with reported rates of around 10% in a recent systematic review. The optimal duration of antibiotic prophylaxis for this patient population remains unknown. Material and Methods. To establish the infection rate associated with prolonged antibiotic prophylaxis in our department, we performed a retrospective review of all adult patients who underwent endoprosthetic reconstruction of the proximal femur after tumor resection for metastatic bone disease during a 4-year period from 2010 to 2013 (n = 105 patients). Results. Intravenous antibiotic prophylaxis was administrated for an extended duration of a mean of 7.4 days. The overall infection rate was 3.6% (4/111 implants), infection free survival was 96% at 2 years, and the risk of amputation associated with infection was 25% (1/4 patients). Discussion. Preemptive eradication of bacterial contamination may be of value in certain clinical situations, where the risk level and consequences of implant-associated infection are unacceptable. Our findings suggest that extended postoperative antibiotic prophylaxis may reduce the risk of PJI in patients undergoing tumor resection and endoprosthetic replacement for metastatic bone disease associated impending or de facto pathologic fractures of the proximal femur. PMID:25705521

  13. Low infection rate after tumor hip arthroplasty for metastatic bone disease in a cohort treated with extended antibiotic prophylaxis.

    Science.gov (United States)

    Hettwer, Werner H; Horstmann, Peter Frederik; Hovgaard, Thea Bechmann; Grum-Scwensen, Tomas Andreas; Petersen, Michael M

    2015-01-01

    Background. Compared to conventional hip arthroplasty, endoprosthetic reconstruction after tumor resection is associated with a substantially increased risk of periprosthetic joint infection (PJI), with reported rates of around 10% in a recent systematic review. The optimal duration of antibiotic prophylaxis for this patient population remains unknown. Material and Methods. To establish the infection rate associated with prolonged antibiotic prophylaxis in our department, we performed a retrospective review of all adult patients who underwent endoprosthetic reconstruction of the proximal femur after tumor resection for metastatic bone disease during a 4-year period from 2010 to 2013 (n = 105 patients). Results. Intravenous antibiotic prophylaxis was administrated for an extended duration of a mean of 7.4 days. The overall infection rate was 3.6% (4/111 implants), infection free survival was 96% at 2 years, and the risk of amputation associated with infection was 25% (1/4 patients). Discussion. Preemptive eradication of bacterial contamination may be of value in certain clinical situations, where the risk level and consequences of implant-associated infection are unacceptable. Our findings suggest that extended postoperative antibiotic prophylaxis may reduce the risk of PJI in patients undergoing tumor resection and endoprosthetic replacement for metastatic bone disease associated impending or de facto pathologic fractures of the proximal femur.

  14. Bone marrow oedema on MR imaging indicates ARCO stage 3 disease in patients with AVN of the femoral head

    Energy Technology Data Exchange (ETDEWEB)

    Meier, Reinhard; Schaeffeler, Christoph; Waldt, Simone; Rummeny, Ernst J.; Woertler, Klaus [Klinikum rechts der Isar, Technische Universitaet Muenchen, Department of Diagnostic and Interventional Radiology, Munich (Germany); Kraus, Tobias M. [Klinikum rechts der Isar, Technische Universitaet Muenchen, Department of Orthopaedics, Munich (Germany); Berufsgenossenschaftliche Unfallklinik Tuebingen, Department of Trauma and Orthopaedics, Tuebingen (Germany); Torka, Sebastian [Klinikum rechts der Isar, Technische Universitaet Muenchen, Department of Orthopaedics, Munich (Germany); Berufsgenossenschaftliche Unfallklinik Murnau, Department of Trauma and Orthopaedics, Murnau (Germany); Schlitter, Anna Melissa; Specht, Katja [Klinikum rechts der Isar, Technische Universitaet Muenchen, Institute of Pathology, Munich (Germany); Haller, Bernhard [Klinikum rechts der Isar, Technische Universitaet Muenchen, Institute of Medical Statistics and Epidemiology, Munich (Germany); Rechl, Hans [Klinikum rechts der Isar, Technische Universitaet Muenchen, Department of Orthopaedics, Munich (Germany)

    2014-09-15

    To test the hypothesis that bone marrow oedema (BME) observed on MRI in patients with avascular necrosis (AVN) of the femoral head represents an indicator of subchondral fracture. Thirty-seven symptomatic hips of 27 consecutive patients (53 % women, mean age 49.2) with AVN of the femoral head and associated BME on magnetic resonance (MR) imaging were included. MR findings were correlated with computed tomography (CT) of the hip and confirmed by histopathological examination of the resected femoral head. Imaging studies were analysed by two radiologists with use of the ARCO classification. On MR imaging a fracture line could be identified in 19/37 (51 %) cases, which were classified as ARCO stage 3 (n = 15) and stage 4 (n = 4). The remaining 18/37 (49 %) cases were classified as ARCO stage 2. However, in all 37/37 (100 %) cases a subchondral fracture was identified on CT, indicating ARCO stage 3/4 disease. The extent of subchondral fractures and the femoral head collapse was graded higher on CT as compared to MRI (P < 0.05). Histopathological analysis confirmed bone necrosis and subchondral fractures. In patients with AVN, BME of the femoral head represents a secondary sign of subchondral fracture and thus indicates ARCO stage 3 disease. circle BME on MRI in AVN of femoral head indicates a subchondral fracture. (orig.)

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

    Directory of Open Access Journals (Sweden)

    Agata Witkowska

    2014-10-01

    Full Text Available 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

  16. Dose escalation of consolidation radiation therapy (involved field) following autologous bone marrow transplant for recurrent Hodgkin's disease and lymphoma

    International Nuclear Information System (INIS)

    Purpose: Patients with recurrent or refractory non-Hodgkin's lymphoma (NHL) and Hodgkin's disease (HD) are frequently treated with intensive chemotherapy and autologous stem-cell rescue. Subsequent relapse is usually in sites of previous disease. We questioned whether radiotherapy (RT) to such sites after autologous bone marrow transplant (ABMT) might diminish such failures while not interrupting pre-ABMT chemotherapy or increasing peri-transplant morbidity. Methods: Since 11/88, 225 patients with recurrent or refractory NHL or or HD have undergone ABMT. Since 9/90, involved field (IF) RT was administered between 4-12 weeks post-ABMT to 70 of these patients who entered pre-transplant salvage chemotherapy with clinical or radiographic evidence of disease. The dose of IFRT was dependent on the disease response to induction chemotherapy and the BMT conditioning regimen. Patients demonstrating a complete response (CR) to reinduction chemo received 20 Gy IFRT. Patients with residual disease at the time of BMT but demonstrating a CR to the BMT conditioning regimen received 30 Gy. Patients with identifiable disease post BMT who showed diminution of disease after 30 Gy were boosted to 36 - 40 Gy. Patients were not irradiated if they had received TBI, previous RT to sites of concern, refused RT, relapsed too quickly to receive RT, or were in complete remission by ABMT. Patients were also analyzed according to their disease burden at ABMT defined as 2 cm disease. Field placement and design to include tumor volume was tailored to response but initially included the preBMT tumor volume with cone-down as dose was escalated in order to exclude dose limiting normal tissue. Results: The results are promising, and similar to our previously reported 3 years survival. For all patients, the 3-year actuarial event-free survival (EFS) rate (Kaplan-Meier log rank test) for 150 NHL and 75 HD patients is 45% and 50%, respectively. The 2 year EFS for NHL patients treated with or without

  17. The impact of bone marrow micrometastases on metastatic disease-free survival in patients with colorectal carcinoma.

    LENUS (Irish Health Repository)

    O'Connor, O J

    2012-02-03

    AIMS: The biological relevance of bone marrow micrometastases (BMM) in colorectal cancer remains unknown. Here, we investigate their nature by examining the impact of the presence of BMM on metastatic disease-free survival in a cohort of patients with this disease. METHODS: Sixty-three consecutive patients undergoing surgery for colorectal cancer of any stage were studied after approval of the study protocol by the local ethics committee and with full individual informed consent. All had bilateral iliac crest bone marrow aspirates prior to operation. Aspirates were then examined for the presence of aberrant cytokeratin-18-positive cells by a blinded observer using both flow cytometric and APAAP immunohistochemical techniques. RESULTS: Mean follow-up after surgery was 4.6 years (range 1.9-6.9) for those without hepatic metastases at diagnosis. Seven of 34 patients with Dukes\\' stage A or B developed metastatic disease after a mean interval of 4.7 years (range 3.8-6.8). However, only 2 of these patients demonstrated BMM at the time of surgery. Nine of 15 patients with Dukes\\' C carcinoma at the time of surgery subsequently developed metastases after a mean interval of 4.4 years (range 1.9-6.9). Again, only two of these patients had BMM detectable initially. In only three of the 14 patients known to have metastases at the time of operation (i.e. Dukes\\'\\'D\\' disease) were BMM found. CONCLUSION: The presence of BMM as detected by this methodology was not predictive of tumour recurrence or metastasis. This study does not support the consideration of adjuvant therapy based on the presence of BMM at a single pre-operative time point in patients with colorectal cancer.

  18. Pediatric and adult MRI atlas of bone marrow. Normal appearances, variants and diffuse disease states

    International Nuclear Information System (INIS)

    This comprehensive atlas is unique in being devoted to the MRI appearances of bone marrow in the axial and appendicular skeleton of adults and children. Normal MRI findings, including common variants and degenerative changes, are first documented. MRI appearances in the entire spectrum of neoplastic and non-neoplastic infiltrative marrow disorders are then presented, with accompanying explanatory text. Among the conditions considered are multiple myeloma, the acute and chronic leukemias, diffuse metastases, diffuse lymphomas, the anemias, polycythemia vera, myelofibrosis, storage disorders, and infections. Characteristic changes to bone marrow following various forms of treatment are also displayed and discussed. The selected images reflect the use of a variety of sequences and techniques, such as fat suppression, and contrast-enhanced imaging.

  19. Pediatric and adult MRI atlas of bone marrow. Normal appearances, variants and diffuse disease states

    Energy Technology Data Exchange (ETDEWEB)

    Ilaslan, Hakan; Sundaram, Murali [Cleveland Clinic Lerner College of Medicine, OH (United States); Cleveland Clinic Department of Radiology, OH (United States)

    2016-08-01

    This comprehensive atlas is unique in being devoted to the MRI appearances of bone marrow in the axial and appendicular skeleton of adults and children. Normal MRI findings, including common variants and degenerative changes, are first documented. MRI appearances in the entire spectrum of neoplastic and non-neoplastic infiltrative marrow disorders are then presented, with accompanying explanatory text. Among the conditions considered are multiple myeloma, the acute and chronic leukemias, diffuse metastases, diffuse lymphomas, the anemias, polycythemia vera, myelofibrosis, storage disorders, and infections. Characteristic changes to bone marrow following various forms of treatment are also displayed and discussed. The selected images reflect the use of a variety of sequences and techniques, such as fat suppression, and contrast-enhanced imaging.

  20. Current Opinion in Developmental Origins of Health and Disease%健康和疾病的发育起源研究现状

    Institute of Scientific and Technical Information of China (English)

    曾婵娟; 杨慧霞

    2011-01-01

    Environmental factors during early life in development have been shown to influence the susceptibility to develop diseases in later life. In particular, there is a wealth of evidence from both epidemiological and animal studies for greater effect on an offspring's risk of developing adult chronic diseases (including hypertension, diabetes, coronary artery disease, obesity, et al) as a result of unbalanced maternal nutrition, ranging from poor to rich environments.These findings indicate new bridges of causality, inferring the possibility of early establishment of persistent metabolic and physiological adjustments that determine morbid outcomes throughout life, grouped as the developmental origins of health and disease (DOHaD). The mechanisms by which nutritional insults during a period of developmental plasticity result in adult metabolic phenotype are now beginning to receive considerable scientific interest, in particular the epigenetic and transcriptional regulation of key metabolic genes in response to nutritional stimuli that mediate persistent changes and an adult metabolic phenotype, a continued and greater understanding of these mechanisms will eventually allow specific interventions, with a favorable impact on the global incidence of cardiovascular disease and type 2 diabetes in the future.%大量流行病学和动物实验研究显示,发育早期不利因素,特别是宫内营养失衡(包括营养不良及营养过剩).导致机体生理和代谢发生永久改变,引发子代肥胖、2型糖尿病、高血压、冠心病等成年慢性疾病的发生发展.这一过程发生在胎儿发育的窗11期,即健康和疾病的发育起源(developmental origins ofhealth and disease.DOHaD).目前.其具体机制引发了国内外学者的广泛关注,特别是代谢调节关键基因在转录水平的改变及表观遗传学在引发成年代谢表型中的作用.深入了解并进一步探讨其机制以寻求有效的干预方式对控制心血管疾病及2

  1. Adseverin plays a role in osteoclast differentiation and periodontal disease-mediated bone loss.

    Science.gov (United States)

    Jiang, Hongwei; Wang, Yongqiang; Viniegra, Ana; Sima, Corneliu; McCulloch, Christopher A; Glogauer, Michael

    2015-06-01

    Osteoclast differentiation and function are highly dependent on the assembly and turnover of actin filaments, but little is known about the roles of actin binding proteins in these processes. Adseverin (Ads), a member of the gelsolin superfamily of actin capping and severing proteins, regulates actin filament turnover and can regulate the turnover of cortical actin filaments of chromaffin cells during exocytosis. Using a conditional Ads knockout mouse model, we confirmed our previous finding in cultured cells that Ads plays a role in osteoclastogenesis (OCG) and actin cytoskeletal organization in osteoclasts. Here we show that Ads is required for osteoclast formation and that when alveolar bone resorption is experimentally induced in mice, genetic deletion of Ads prevents osteoclast-mediated bone loss. Further, when Ads-null osteoclasts are cultured, they exhibit defective OCG, disorganized podosome-based actin filament superstructures, and decreased bone resorption. Reintroduction of Ads into Ads-null osteoclast precursor cells restored these osteoclast defects. Collectively, these data demonstrate a unique and osteoclast-specific role for Ads in OCG and osteoclast function.

  2. Adseverin plays a role in osteoclast differentiation and periodontal disease-mediated bone loss.

    Science.gov (United States)

    Jiang, Hongwei; Wang, Yongqiang; Viniegra, Ana; Sima, Corneliu; McCulloch, Christopher A; Glogauer, Michael

    2015-06-01

    Osteoclast differentiation and function are highly dependent on the assembly and turnover of actin filaments, but little is known about the roles of actin binding proteins in these processes. Adseverin (Ads), a member of the gelsolin superfamily of actin capping and severing proteins, regulates actin filament turnover and can regulate the turnover of cortical actin filaments of chromaffin cells during exocytosis. Using a conditional Ads knockout mouse model, we confirmed our previous finding in cultured cells that Ads plays a role in osteoclastogenesis (OCG) and actin cytoskeletal organization in osteoclasts. Here we show that Ads is required for osteoclast formation and that when alveolar bone resorption is experimentally induced in mice, genetic deletion of Ads prevents osteoclast-mediated bone loss. Further, when Ads-null osteoclasts are cultured, they exhibit defective OCG, disorganized podosome-based actin filament superstructures, and decreased bone resorption. Reintroduction of Ads into Ads-null osteoclast precursor cells restored these osteoclast defects. Collectively, these data demonstrate a unique and osteoclast-specific role for Ads in OCG and osteoclast function. PMID:25681458

  3. Metabolic bone disease in the preterm infant: Current state and future directions

    Science.gov (United States)

    Rehman, Moghis Ur; Narchi, Hassib

    2015-01-01

    Neonatal osteopenia is an important area of interest for neonatologists due to continuing increased survival of preterm infants. It can occur in high-risk infants such as preterm infants, infants on long-term diuretics or corticosteroids, and those with neuromuscular disorders. Complications such as rickets, pathological fractures, impaired respiratory function and poor growth in childhood can develop and may be the first clinical evidence of the condition. It is important for neonatologists managing such high-risk patients to regularly monitor biochemical markers for evidence of abnormal bone turnover and inadequate mineral intake in order to detect the early phases of impaired bone mineralization. Dual-energy X-ray absorptiometry has become an increasingly used research tool for assessing bone mineral density in children and neonates, but more studies are still needed before it can be used as a useful clinical tool. Prevention and early detection of osteopenia are key to the successful management of this condition and oral phosphate supplements should be started as soon as is feasible. PMID:26413483

  4. Diagnosis and treatment of 6 cases with Paget′s disease of bone%畸形性骨炎六例的临床诊治分析

    Institute of Scientific and Technical Information of China (English)

    郑燕; 杜洪泉; 章振林

    2015-01-01

    6例畸形性骨炎患者(男5,女1),平均年龄为(57.7±11.8)岁,平均病程为(7.5±6.5)年。临床症状均表现为受累骨骼疼痛,病变主要累及骨盆和股骨。 X 线摄片均显示畸形性骨炎特征性的改变。骨扫描检查显示受累骨骼的核素异常浓聚。 6例患者的血清碱性磷酸酶(ALP)均有不同程度升高,中位数为235 U/ L。5例患者予唑来膦酸钠静脉滴注治疗,治疗后3个月疼痛明显缓解,活动功能改善,血清 ALP水平下降,疗效明显。%Six cases of Paget′s disease of bone, including 5 males and 1 female, aged (57. 7 ±11. 8) years old, were recruited. Mean duration of disease was (7. 5±6. 5) years. Clinical manifestations were bone pain and bone deformity. The lesions mainly reside in the pelvis and femur. X-ray film showed typical lesion of Paget′s disease of bone, such as impaired bone trabecular structure with coarseness and disorder, cortical thickening, medullary cavity narrowing and skeletal deformation. Bone scan revealed abnormal radionuclide concentration in the involved bone. Serum alkaline phosphatase ( ALP) in 6 patients was increased ( median 235 U/ L). 5 patients received zoledronic acid sodium intravenous infusion therapy. Bone pain was relieved obviously in 5 patients after treatment for 2-3 months. Physical activity was greatly improved, and serum ALP levels significantly decreased.

  5. Bone tumor

    Science.gov (United States)

    Tumor - bone; Bone cancer; Primary bone tumor; Secondary bone tumor ... The cause of bone tumors is unknown. They often occur in areas of the bone that grow rapidly. Possible causes include: Genetic defects ...

  6. Aortic calcification and femoral bone density are independently associated with left ventricular mass in patients with chronic kidney disease.

    Directory of Open Access Journals (Sweden)

    Colin D Chue

    Full Text Available BACKGROUND: Vascular calcification and reduced bone density are prevalent in chronic kidney disease and linked to increased cardiovascular risk. The mechanism is unknown. We assessed the relationship between vascular calcification, femoral bone density and left ventricular mass in patients with stage 3 non-diabetic chronic kidney disease in a cross-sectional observational study. METHODOLOGY AND PRINCIPAL FINDINGS: A total of 120 patients were recruited (54% male, mean age 55 ± 14 years, mean glomerular filtration rate 50 ± 13 ml/min/1.73 m(2. Abdominal aortic calcification was assessed using lateral lumbar spine radiography and was present in 48%. Mean femoral Z-score measured using dual energy x-ray absorptiometry was 0.60 ± 1.06. Cardiovascular magnetic resonance imaging was used to determine left ventricular mass. One patient had left ventricular hypertrophy. Subjects with aortic calcification had higher left ventricular mass compared to those without (56 ± 16 vs. 48 ± 12 g/m(2, P = 0.002, as did patients with femoral Z-scores below zero (56 ± 15 vs. 49 ± 13 g/m(2, P = 0.01. In univariate analysis presence of aortic calcification correlated with left ventricular mass (r = 0.32, P = 0.001; mean femoral Z-score inversely correlated with left ventricular mass (r = -0.28, P = 0.004. In a multivariate regression model that included presence of aortic calcification, mean femoral Z-score, gender and 24-hour systolic blood pressure, 46% of the variability in left ventricular mass was explained (P<0.001. CONCLUSIONS: In patients with stage 3 non-diabetic chronic kidney disease, lower mean femoral Z-score and presence of aortic calcification are independently associated with increased left ventricular mass. Further research exploring the pathophysiology that underlies these relationships is warranted.

  7. 多学科协同治疗骨关节疾病%Multidisciplinary treatment of bone and joint diseases

    Institute of Scientific and Technical Information of China (English)

    张奉春

    2012-01-01

    The bone and joint diseases are very common, have chronic, recurrent characteristics and often lead to discomfort and disability in China. These diseases affect human health seriously. The etiology and pathogenesis are unknown. They may be related with genetic, environmental factors and autoimmune reaction. Rehabilitation exercise, health education, medical treatment and operation are the main methods in the treatment of the diseases. The expected benefits of the treatment are pain relief, improvement or prevention of further deterioration, and prevention of deformity. The types of treatments are different for various diseases. Short-term or long-term treatment with non-steroidal anti-inflammatory drugs (NSAIDs) can relive the pain of bone and joint. Disease-modifying antirheumatic drugs (DMARDs) are selected in the first-line treatment as soon as possible in rheumatoid arthritis, and seronegative spondylo-arthropathies can be treated with SASP and symptoms of some patients are relieved. Advances in the understanding of the pathogenesis of the disease have fostered the development of new therapeutics (biological drugs such as TNF-a inhibitors and rituximab), with improved outcomes. However, operation for advanced stage or medicine ineffective patients is also very important. Rheumatology is very young in China, which is still facing the lack of the rheumatology physician after more than 20 years' development. The cooperation of surgeons and rheumatology physicians is very important, but it has not been recognized and emphasized so far. We should pay more attention to the cooperation in the clinical work in the future.%@@ 一、概述 世界卫生组织将2000年至2010年定为"骨与关节的十年",这一项以"增进患有肌肉与骨骼疾病患者的健康,进一步改善他们的生活质量"作为目标的活动意义重大.骨关节病的范围广泛,除了骨关节炎、类风湿关节炎等各种关节疾病外,还包括脊柱疾病、骨质疏松症以

  8. Lower bone mineral density in Patients with Parkinson’s disease: a cross-sectional study from Chinese Mainland

    Directory of Open Access Journals (Sweden)

    Huimin eGao

    2015-10-01

    Full Text Available Objectives Although several lines of evidence have suggested that patients with Parkinson’s disease (PD have a higher risk of osteoporosis and fracture, the association between bone mineral density and severity of PD patients is unknown. Methods We performed a cross-sectional study of 54 patients with PD and 59 healthy age-matched controls. Multiple clinical scales were used to evaluate the severity of PD, and serum levels of calcium, phosphorus, and homocysteine were measured to determine bone mineral density’s association with PD severity. Results BMD in PD patients was significantly lower than in healthy controls. The BMD scores of the spine, femoral neck, and hip were lower in females than in males in the healthy group. In the PD group, BMD in the hip was significantly lower in females compared to males. There was a negative correlation between daily L-dopa dosage and BMD in the spine and hip in the PD group, while BMD in the spine, neck, and hip was significantly correlated with severity of PD. Besides, we found that among the lumbar spine, femoral neck and hip, bone loss in the lumbar spine was the most severe in PD patients based on the T-scores. Conclusions Our findings support the hypothesis that patients with PD have a higher risk of osteoporosis, and that low BMD in the spine, femoral neck, and hip may indirectly reflect the severity of PD. Our findings have prompted us to pay more attention to osteoporosis in the lumbar spine in Chinese PD patients.

  9. [Cytokines in bone diseases. Genetic defects of PTH/PTHrP receptor in chondrodysplasia].

    Science.gov (United States)

    Ogata, Naoshi

    2010-10-01

    Parathyroid hormone-related protein (PTHrP) signaling plays important roles in regulating the differentiation of chondrocytes in endochondral bone development. PTHrP signaling functions as an inhibitory effect on chondrocyte hypertrophy which is a terminal stage of differentiation at a growth plate. Mutations of the PTH÷PTHrP receptor have been identified in Jansen metaphyseal chondrodysplasia, Blomstrand's lethal chondrodysplasia, and enchondromatosis. Furthermore, genetic manipulations of the PTHrP and its receptor genes in mice have demonstrated the critical roles of these proteins in regulating both the switch between proliferation and differentiation of chondrocytes.

  10. Intra-bone marrow-bone marrow transplantation slows disease progression and prolongs survival in G93A mutant SOD1 transgenic mice, an animal model mouse for amyotrophic lateral sclerosis.

    Science.gov (United States)

    Ohnishi, Shizuo; Ito, Hidefumi; Suzuki, Yasuhiro; Adachi, Yasushi; Wate, Reika; Zhang, Jianhua; Nakano, Satoshi; Kusaka, Hirofumi; Ikehara, Susumu

    2009-11-01

    It has been reported that bone marrow transplantation (BMT) has clinical effects on not only hematopoietic diseases and autoimmune diseases but also solid malignant tumors and metabolic diseases. We have found that intra-bone marrow-bone marrow transplantation (IBM-BMT) is superior to conventional intravenous BMT, since IBM-BMT enables rapid recovery of donor hematopoiesis and reduces the extent of graft-versus-host disease (GVHD). In this experiment, we examined the effects of IBM-BMT on symptomatic G93A mutant SOD1 transgenic mice (mSOD1 Tg mice), a model mouse line for amyotrophic lateral sclerosis (ALS). Symptomatic mSOD1 Tg mice (12 weeks old) were irradiated with 6Gyx2 at a 4-hour interval, one day before IBM-BMT. The mice were transplanted with bone marrow cells (BMCs) from 12-wk-old eGFP-transgenic C57BL/6 mice (eGFP Tg mice) or BMCs from 12-wk-old mSOD1 Tg mice. The ALS model mice transplanted with BMCs from eGFP Tg mice showed longer survival and slower disease progression than those transplanted with BMCs from mSOD1 Tg mice or untreated mSOD1 Tg mice. There was a significantly high number of eGFP(+) cells in the anterior horn of the spinal cord of the mSOD1 Tg mice transplanted with BMCs of eGFP Tg mice, some of which expressed Iba-1, a marker of microglia, although they did not differentiate into neural cells. These results suggest that the replacement with normal hematopoietic cells improved the neural cell environment, thereby slowing the progression of the disease.

  11. Bone Marrow Stromal Cells Attenuate Lung Injury in a Murine Model of Neonatal Chronic Lung Disease

    OpenAIRE

    Aslam, Muhammad; Baveja, Rajiv; Liang, Olin D.; Fernandez-Gonzalez, Angeles; Lee, Changjin; Mitsialis, S. Alex; Kourembanas, Stella

    2009-01-01

    Rationale: Neonatal chronic lung disease, known as bronchopulmonary dysplasia (BPD), remains a serious complication of prematurity despite advances in the treatment of extremely low birth weight infants.

  12. Long-term observations of autoimmune-prone mice treated for autoimmune disease by allogeneic bone marrow transplantation

    International Nuclear Information System (INIS)

    Long-term effects of allogeneic bone marrow transplantation (ABMT) across major histocompatibility complex barriers were studied in (NZB x NZW)F1 (B/W), BXSB, and MRL/Mr-lpr-lpr (MRL/lpr) mice with established autoimmune disease at the time of ABMT. In the BXSB or B/W mice, ABMT cured all aspects of autoimmune disease. Glomerular damage, revealed by histological study was dramatically improved. Serological abnormalities and immunologic functions also were normalized. Correction of autoimmune disease and advanced renal disease in BXSB and B/W mice regularly lasted greater than 5-6 mo and even 1 yr after ABMT. In the MRL/lpr mice, however, autoimmune and renal disease at first improved but then recurred after ABMT, apparently because of intolerance of mice for high doses of irradiation and a high degree of resistance of recipient stem cells to irradiation. In this model, H-2 typing revealed that by the time of relapse, immunocompetent cells of the chimeric mice had been replaced by host (MRL/lpr; H-2k) cells. B220+ Ly-1+ cells, present in increased numbers in untreated MRL/lpr mice, initially returned to normal levels after ABMT but then reappeared in the MRL/lpr mice that had received marrow from donors having few such lymphocytes. Thus, our results show that MRL/lpr mice possess abnormal radioresistant stem cells and provide impressive evidence that the origin of autoimmune diseases in this strain, as in the several other strains studied, resids in abnormalities present in stem cells

  13. Assessment of Bone Mineral Density in Male Patients with Chronic Obstructive Pulmonary Disease by DXA and Quantitative Computed Tomography.

    Science.gov (United States)

    Fountoulis, George; Kerenidi, Theodora; Kokkinis, Constantinos; Georgoulias, Panagiotis; Thriskos, Paschal; Gourgoulianis, Konstantinos; Fezoulidis, Ioannis; Vassiou, Katerina; Vlychou, Marianna

    2016-01-01

    The purpose of this study is to identify the prevalence of osteoporosis in male patients with chronic obstructive pulmonary disease (COPD) by dual-energy X-ray absorptiometry (DXA) and quantitative computed tomography (QCT) and to compare the diagnostic abilities of the above methods. Thirty-seven male patients with established COPD were examined with DXA and standard QCT in lumbar spine, including L1, L2, and L3 vertebrae. T-scores and bone mineral density values were calculated by DXA and QCT method, respectively. Comparative assessment of the findings was performed and statistical analysis was applied. QCT measurements found more COPD patients with impaired bone mineral density compared to DXA, namely, 13 (35.1%) versus 12 (32.4%) patients with osteopenia and 16 (43.2%) versus 9 (16.2%) patients with osteoporosis (p = 0.04). More vertebrae were found with osteoporosis by QCT compared to DXA (p = 0.03). The prevalence of osteoporosis among male patients with COPD is increased and DXA may underestimate this risk. QCT measurements have an improved discriminating ability to identify low BMD compared to DXA measurements because QCT is able to overcome diagnostic pitfalls including aortic calcifications and degenerative spinal osteophytes. PMID:27087809

  14. Assessment of Bone Mineral Density in Male Patients with Chronic Obstructive Pulmonary Disease by DXA and Quantitative Computed Tomography

    Directory of Open Access Journals (Sweden)

    George Fountoulis

    2016-01-01

    Full Text Available The purpose of this study is to identify the prevalence of osteoporosis in male patients with chronic obstructive pulmonary disease (COPD by dual-energy X-ray absorptiometry (DXA and quantitative computed tomography (QCT and to compare the diagnostic abilities of the above methods. Thirty-seven male patients with established COPD were examined with DXA and standard QCT in lumbar spine, including L1, L2, and L3 vertebrae. T-scores and bone mineral density values were calculated by DXA and QCT method, respectively. Comparative assessment of the findings was performed and statistical analysis was applied. QCT measurements found more COPD patients with impaired bone mineral density compared to DXA, namely, 13 (35.1% versus 12 (32.4% patients with osteopenia and 16 (43.2% versus 9 (16.2% patients with osteoporosis (p=0.04. More vertebrae were found with osteoporosis by QCT compared to DXA (p=0.03. The prevalence of osteoporosis among male patients with COPD is increased and DXA may underestimate this risk. QCT measurements have an improved discriminating ability to identify low BMD compared to DXA measurements because QCT is able to overcome diagnostic pitfalls including aortic calcifications and degenerative spinal osteophytes.

  15. Bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin SC disease

    Directory of Open Access Journals (Sweden)

    Fernando Peixoto Ferraz de Campos

    2014-06-01

    Full Text Available Sickle Cell Disease encompasses a group of disorders related with the hemoglobin S and other hemoglobin genotypes. The clinical manifestation and the severity of symptoms are dependent on the specific genotype. In this setting, homozygous genotype (HbSS presents an early onset of symptoms and a low expectancy of lifetime. However, the SC genotype (HbSC, which apparently shows a less severe clinical course, may exhibit the same complications of HbSS. These complications are usually manifested late in the course of life, when compared with the HbSS patients. It is noteworthy that HbSC may present a normal hematocrit, and therefore stays unknown until the first complication, that may be disastrous. The authors report a case of an African-Descendant woman, aging 65 years, with no previous diagnosis of anemia who sought medical attention because of a thoracic back pain followed by fever and altered mental status. The clinical picture deteriorated very fast with multiple organ failure and death. The autopsy findings concluded by generalized vaso-occlusive crisis, bone marrow necrosis and bone marrow and fat embolism, mainly to the lungs and kidney. The authors call attention for the knowledge of this severe life threatening complication, mainly in a country with a high Afro-Descendant population.

  16. Transplanted Bone Marrow Mesenchymal Stem Cells Improve Memory in Rat Models of Alzheimer's Disease

    Directory of Open Access Journals (Sweden)

    Parvin Babaei

    2012-01-01

    Full Text Available The present study aims to evaluate the effect of bone marrow mesenchymal stem cells (MSCs grafts on cognition deficit in chemically and age-induced Alzheimer's models of rats. In the first experiments aged animals (30 months were tested in Morris water maze (MWM and divided into two groups: impaired memory and unimpaired memory. Impaired groups were divided into two groups and cannulated bilaterally at the CA1 of the hippocampus for delivery of mesenchymal stem cells (500×103/ and PBS (phosphate buffer saline. In the second experiment, Ibotenic acid (Ibo was injected bilaterally into the nucleus basalis magnocellularis (NBM of young rats (3 months and animals were tested in MWM. Then, animals with memory impairment received the following treatments: MSCs (500×103/ and PBS. Two months after the treatments, cognitive recovery was assessed by MWM in relearning paradigm in both experiments. Results showed that MSCs treatment significantly increased learning ability and memory in both age- and Ibo-induced memory impairment. Adult bone marrow mesenchymal stem cells show promise in treating cognitive decline associated with aging and NBM lesions.

  17. [A study on observation of bone metabolism in middle-aged and senile female Graves' disease].

    Science.gov (United States)

    Zhu, L Q; Liu, Y H; Zhou, Y B

    1996-08-01

    Sixty-nine cases of middle aged and senile female Graves' desease (GD) patients suffered from abnormal bone metabolism have been studied. They were divided randomly into group A and B, treated separately with antithyroid drugs (Tapazol and inderal, etc.) in group A, and added with Chinese herbal medicine for tonifying Kidney and promoting blood circulation in group B. Before treatment, patients of both groups showed obvious higher blood calcium (Ca) 24-hour urinary Ca, phosphorus (P) and serum clcitonin (CT) levels than that in normal subjects. These patients' serum Ca, moreover, had a parallel relationship with serum T3 levels (r = 0.6142, P < 0.01) and the serum Ca also a paralleled with serum CT levels (r = 0.5714, P < 0.05). After six months of treatment, the serum Ca, 24-hour urinary Ca, P and blood CT values were all reduced in various degree. The decrease of these bone metabolic parameters were more significant in group B than that in group A. PMID:9387746

  18. The transition state analog inhibitor of Purine Nucleoside Phosphorylase (PNP) Immucillin-H arrests bone loss in rat periodontal disease models.

    Science.gov (United States)

    Deves, Candida; de Assunção, Thiago Milech; Ducati, Rodrigo Gay; Campos, Maria Martha; Basso, Luiz Augusto; Santos, Diogenes Santiago; Batista, Eraldo L

    2013-01-01

    Purine nucleoside phosphorylase (PNP) is a purine-metabolizing enzyme that catalyzes the reversible phosphorolysis of 6-oxypurine (deoxy)nucleosides to their respective bases and (deoxy)ribose-1-phosphate. It is a key enzyme in the purine salvage pathway of mammalian cells. The present investigation sought to determine whether the PNP transition state analog inhibitor (Immucillin-H) arrests bone loss in two models of induced periodontal disease in rats. Periodontal disease was induced in rats using ligature or LPS injection followed by administration of Immucillin-H for direct analysis of bone loss, histology and TRAP staining. In vitro osteoclast differentiation and activation of T CD4+ cells in the presence of Immucillin-H were carried out for assessment of RANKL expression, PNP and Cathepsin K activity. Immucillin-H inhibited bone loss induced by ligatures and LPS, leading to a reduced number of infiltrating osteoclasts and inflammatory cells. In vitro assays revealed that Immucillin-H could not directly abrogate differentiation of osteoclast precursor cells, but affected lymphocyte-mediated osteoclastogenesis. On the other hand, incubation of pre-activated T CD4+ with Immucillin-H decreased RANKL secretion with no compromise of cell viability. The PNP transition state analog Immucillin-H arrests bone loss mediated by T CD4+ cells with no direct effect on osteoclasts. PNP inhibitor may have an impact in the treatment of diseases characterized by the presence of pathogens and imbalances of bone metabolism.

  19. 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)

    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

  20. Sequential Cadaveric Lung and Bone Marrow Transplant for Immune Deficiency Diseases

    Science.gov (United States)

    2015-12-01

    Severe Combined Immunodeficiency (SCID); Immunodeficiency With Predominant T-cell Defect, Unspecified; Severe Chronic Neutropenia; Chronic Granulomatous Disease (CGD); Hyper IgE Syndromes; Hyper IgM Deficiencies; Wiskott-Aldrich Syndrome; Mendelian Susceptibility to Mycobacterial Disease; Common Variable Immune Deficiency (CVID)

  1. Intramyocardial Bone Marrow Cell Injection : Clinical and Functional Effects in Ischemic Heart Disease

    NARCIS (Netherlands)

    Ramshorst, Jan van

    2014-01-01

    Coronary artery disease is a major cause of mortality and morbidity in the western world. Despite successive revascularization procedures, a large number of patients ends up with end-stage coronary artery disease, not amenable for conventional revascularization. These patients often have stress-indu

  2. A novel and feasible way to cultivate and purify endothelial progenitor cells from bone marrow of children with congenital heart diseases

    Institute of Scientific and Technical Information of China (English)

    WU Yong-tao; LI Jing-xing; LIU Shuo; XIN Yi; WANG Zi-jian; GAO Jin; JI Bing-yang; FAN Xiang-ming; ZHOU Qi-wen

    2012-01-01

    Background Endothelial progenitor cells (EPCs) are used in vascular tissue engineering and clinic therapy.Some investigators get EPCs from the peripheral blood for clinic treatment,but the number of EPCs is seldom enough.We have developed the cultivation and purification of EPCs from the bone marrow of children with congenital heart disease,to provide enough seed cells for a small calibre vascular tissue engineering study.Methods The 0.5-ml of bone marrow was separated from the sternum bone,and 5-ml of peripheral blood was collected from children with congenital heart diseases who had undergone open thoracic surgery.CD34+ and CD34+/VEGFR+cells in the bone marrow and peripheral blood were quantified by flow cytometry.CD34+NEGFR+ cells were defined as EPCs.Mononuclear cells in the bone marrow were isolated by Ficoll(R) density gradient centrifugation and cultured by the EndoCult Liquid Medium KitTM.Colony forming endothelial cells was detected.Immunohistochemistry staining for Dil-ac-LDL and FITC-UEA-1 confirmed the endothelial lineage of these cells.Results CD34+ and CD34+NEGFR+ cells in peripheral blood were (0.07±0.05)% and (0.05±0.02)%,respectively.The number of CD34+ and CD34+NEGFR+ cells in bone marrow were significantly higher than in blood,(4.41±1.47)% and (0.98±0.65)%,respectively (P <0.0001).Many colony forming units formed in the culture.These cells also expressed high levels of Dil-ac-LDL and FITC-UEA-1.Conclusion This is a novel and feasible approach that can cultivate and purify EPCs from the bone marrow of children with congenital heart disease,and provide seed cells for small calibre vascular tissue engineering.

  3. Elevated interferon-gamma in CNS inflammatory disease: a potential complication for bone marrow reconstitution in MS

    DEFF Research Database (Denmark)

    Hassan-Zahraee, M; Tran, E H; Bourbonnière, L;

    2000-01-01

    Bone marrow transplantation (BMT) is increasingly used to treat Multiple Sclerosis (MS) a CNS inflammatory disease with elevated CNS and systemic IFNgamma levels. We wished to determine the effect of IFNgamma on BM graft survival in a transgenic mouse model for chronic MS. BM transplantation...... into transgenic mice which express elevated levels of IFNgamma in the CNS was unsuccessful. By contrast, there was 100% survival of even fully allogeneic, T-depleted transplants to transgenics that over express TNFalpha in the CNS, using the same MBP promoter. IFNgamma was detectable in spleen of irradiated mice...... but levels were higher in IFNgamma transgenics. BM transplantation into IFNgamma-deficient recipients also had a high failure rate. Transplants of BM from mice lacking expression of IFNgamma-receptor failed, whereas IFNgamma-deficient grafts survived, suggesting that IFNgamma response status of the graft can...

  4. 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 ...

  5. Bone regeneration in dentistry

    OpenAIRE

    Tonelli, Paolo; Duvina, Marco; Barbato, Luigi; Biondi, Eleonora; Nuti, Niccolò; Brancato, Leila; Rose, Giovanna Delle

    2011-01-01

    The edentulism of the jaws and the periodontal disease represent conditions that frequently leads to disruption of the alveolar bone. The loss of the tooth and of its bone of support lead to the creation of crestal defects or situation of maxillary atrophy. The restoration of a functional condition involves the use of endosseous implants who require adequate bone volume, to deal with the masticatory load. In such situations the bone need to be regenerated, taking advantage of the biological p...

  6. Diagnosis of bone marrow lesions in the MR tomogram in children suffering from diseases of the haematopoietic system with special reference to post-therapy changes

    International Nuclear Information System (INIS)

    119 MR-examinations of both tibiae, knees and the lower part of both femura were performed in 41 children suffering from bone marrow disease (27 ALL, 4 AML, 3 NHL, 1 agranulocytosis, 6 anaemia). T1- and T2-spinecho sequences and a T2-gradientecho sequence were used. Bone marrow changes in leukaemia were diffuse before therapy and patchy after therapy. Due to their different signal in T2-weighted images, differentitation of the post-therapeutic patchy findings into infiltrations, fibrosis, necrosis and siderosis seems to be possible. In future, MRI will be the method of choice for screening and controlling bone marrow disease if the examination time is shortened by using only a T1-spinecho sequence and a T2-gradient-echo sequence. (orig.)

  7. Osseous and Nonosseous Bone Scan Findings in Liver Transplant Candidates with end-stage Chronic Liver Disease

    Directory of Open Access Journals (Sweden)

    Seval Erhamamcı

    2013-08-01

    Full Text Available Objective: End-stage chronic liver disease (CLD adversely affects the function of multiple organ systems including the skeletal system. The aim of this study was to assess osseous and nonosseous bone scintigraphy (BS findings in liver transplant (LT candidates with end-stage CLD. Methods: We retrospectively evaluated BS findings in 50 consecutive patients with end-stage CLD who were undergoing preoperative assessment for LT from January 2006 to December 2011. All the patients were analyzed with respect to clinical and laboratory parameters, and BS findings. Scintigrams were visually assessed for the presence of osseous and nonosseous abnormalities. Osseous abnormalities were classified as those indicating bone metabolism changes or metastatic bone disease. Typical scintigraphic findings denoting to changes in bone metabolism were the presence of decreased osseous uptake, increased periarticular uptake, asymmetrical or unusual uptake patterns. Nonosseous findings were classified according to the degree of soft-tissue uptake as mild and severe. Results: The group consisted of 46 adult and 4 adolescent patients. All adolescent patients had normal skeletal accumulation with growth plate uptake and one had mildly increased renal cortical activity. A total of 46 adult patients had one or more of the following osseous findings: generalized decrease in osseous uptake (n=4, 8.7%; bilateral decrease in lower extremity uptake (n=26, 56.5%; symmetrically increased periarticular uptake (n=26, 56.5%; bilateral cortical/periosteal increased uptake in the lower extremity indicating hepatic hypertrophic osteoarthropathy (HOA (n=8, 17.4%; bilateral increased sacroiliac activity (n=16, 34.8%; sacral activity (n=10, 21.7%, coccygeal activity (n=2, 4.3%, focally increased uptake suggestive of metastases (n=5, 10.9%. Three rib metastases appeared to be linear. Nonosseous findings observed in adult patients were mild diffuse liver uptake (n=4, 8.7% and bilateral

  8. Mesenchymal bone marrow cell therapy in a mouse model of chagas disease. Where do the cells go?

    Directory of Open Access Journals (Sweden)

    Jasmin

    Full Text Available BACKGROUND: Chagas disease, resulting from infection with the parasite Trypanosoma cruzi (T. cruzi, is a major cause of cardiomyopathy in Latin America. Drug therapy for acute and chronic disease is limited. Stem cell therapy with bone marrow mesenchymal cells (MSCs has emerged as a novel therapeutic option for cell death-related heart diseases, but efficacy of MSC has not been tested in Chagas disease. METHODS AND RESULTS: We now report the use of cell-tracking strategies with nanoparticle labeled MSC to investigate migration of transplanted MSC in a murine model of Chagas disease, and correlate MSC biodistribution with glucose metabolism and morphology of heart in chagasic mice by small animal positron emission tomography (microPET. Mice were infected intraperitoneally with trypomastigotes of the Brazil strain of T. cruzi and treated by tail vein injection with MSC one month after infection. MSCs were labeled with near infrared fluorescent nanoparticles and tracked by an in vivo imaging system (IVIS. Our IVIS results two days after transplant revealed that a small, but significant, number of cells migrated to chagasic hearts when compared with control animals, whereas the vast majority of labeled MSC migrated to liver, lungs and spleen. Additionally, the microPET technique demonstrated that therapy with MSC reduced right ventricular dilation, a phenotype of the chagasic mouse model. CONCLUSIONS: We conclude that the beneficial effects of MSC therapy in chagasic mice arise from an indirect action of the cells in the heart rather than a direct action due to incorporation of large numbers of transplanted MSC into working myocardium.

  9. Trichostatin A-Mediated Epigenetic Transformation of Adult Bone Marrow-Derived Mesenchymal Stem Cells Biases the In Vitro Developmental Capability, Quality, and Pluripotency Extent of Porcine Cloned Embryos

    Directory of Open Access Journals (Sweden)

    Marcin Samiec

    2015-01-01

    Full Text Available The current research was conducted to explore the in vitro developmental outcome and cytological/molecular quality of porcine nuclear-transferred (NT embryos reconstituted with adult bone marrow-derived mesenchymal stem cells (ABM-MSCs that were epigenetically transformed by treatment with nonspecific inhibitor of histone deacetylases, known as trichostatin A (TSA. The cytological quality of cloned blastocysts was assessed by estimation of the total cells number (TCN and apoptotic index. Their molecular quality was evaluated by real-time PCR-mediated quantification of gene transcripts for pluripotency- and multipotent stemness-related markers (Oct4, Nanog, and Nestin. The morula and blastocyst formation rates of NT embryos derived from ABM-MSCs undergoing TSA treatment were significantly higher than in the TSA-unexposed group. Moreover, the NT blastocysts generated using TSA-treated ABM-MSCs exhibited significantly higher TCN and increased pluripotency extent measured with relative abundance of Oct4 and Nanog mRNAs as compared to the TSA-untreated group. Altogether, the improvements in morula/blastocyst yields and quality of cloned pig embryos seem to arise from enhanced abilities for promotion of correct epigenetic reprogramming of TSA-exposed ABM-MSC nuclei in a cytoplasm of reconstructed oocytes. To our knowledge, we are the first to report the successful production of mammalian high-quality NT blastocysts using TSA-dependent epigenomic modulation of ABM-MSCs.

  10. Peripheral blood minimal residual disease may replace bone marrow minimal residual disease as an immunophenotypic biomarker for impending relapse in acute myeloid leukemia.

    Science.gov (United States)

    Zeijlemaker, W; Kelder, A; Oussoren-Brockhoff, Y J M; Scholten, W J; Snel, A N; Veldhuizen, D; Cloos, J; Ossenkoppele, G J; Schuurhuis, G J

    2016-03-01

    As relapses are common in acute myeloid leukemia (AML), early relapse prediction is of high importance. Although conventional minimal residual disease (MRD) measurement is carried out in bone marrow (BM), peripheral blood (PB) would be an advantageous alternative source. This study aims to investigate the specificity of leukemia-associated immunophenotypes used for MRD detection in blood samples. Consistency of PB MRD as compared with BM MRD was determined in flow cytometric data of 205 paired BM and PB samples of 114 AML patients. A significant correlation was found between PB and BM MRD (r=0.67, Pconsolidation therapy. As PB MRD appeared to be an independent predictor for response duration, the highly specific PB MRD assay may have a prominent role in future MRD assessment in AML. PMID:26373238

  11. Strategies to Target the Bone Morphogenetic Protein Signalling Pathway in Lung Disease

    OpenAIRE

    Purcell, James

    2011-01-01

    Lung disease and lung injury are responsible for 20% of deaths of the Irish population every year, and the country has the 2nd highest death rate in Europe for respiratory diseases. Conditions related to the respiratory system are the second largest long term illness by young adults. Lung cancer is the largest cause of cancer related death in Europe as a whole. New and refined mechanisms of drug delivery for the prevention, cure or delayed progression of disease, represents a pathway for t...

  12. Minimal residual disease in bone marrow and peripheral blood of patients with metastatic breast cancer.

    Science.gov (United States)

    Bischoff, Joachim; Rosenberg, Robert; Dahm, Michael; Janni, Wolfgang; Gutschow, Klaus

    2003-01-01

    The presence of occult micrometastases in bone marrow (BM) of patients with early breast cancer increases the risk of relapse. Detection of circulation tumor cells in peripheral blood (PB) may also influence the patient's prognosis. Few data are available on the correlation between tumor cell dissemination in BM and PB in solid epithelial tumors. Twenty-milliliter blood samples were collected from PB of 42 patients with advanced breast cancer and centrifuged using the density gradient OncoQuick (OncoQuick Greiner BioOne, Frickenhausen, Germany). The BM aspirates available from 11 of the 42 patients were centrifuged using density centrifugation Ficoll. Tumor cell detection was performed by microscopy after cytospin preparation and immunocytochemical staining with the monoclonal antibody A45-B/B3. Cytokeratin-positive cells were detected in 23 patients (55%) in the PB and in three patients (27%) in the BM. A cohort with bone lesions as the only metastatic side showed a correlation as follows: 7 of the 11 patients (64%) had negative findings in BM and PB, whereas cytokeratin-positive cells in PB were present in 3 of these 11 patients (27%). The presence of visceral metastases was associated with the detection of cytokeratin-positive cells in the PB in 20 of the 31 patients (65%) in this subgroup. The density gradient OncoQuick in combination with immunocytochemical staining allows the detection of cytokeratin-positive cells in PB of patients with advanced breast cancer. The immunocytochemical detection of cytokeratin-positive cells in PB seems to be associated with the site of metastatic manifestation.

  13. Fluorine-18 fluorodeoxyglucose positron emission tomography in infectious bone diseases: results of histologically confirmed cases

    International Nuclear Information System (INIS)

    The aim of this study was to evaluate the clinical use of fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET) in acute and chronic osteomyelitis and inflammatory spondylitis. The study population comprised 21 patients suspected of having acute or chronic osteomyelitis or inflammatory spondylitis. Fifteen of these patients subsequently underwent surgery. FDG-PET results were correlated with histopathological findings. The remaining six patients, who underwent conservative therapy, were excluded from any further evaluation due to the lack of histopathological data. The histopathological findings revealed osteomyelitis or inflammatory spondylitis in all 15 patients: seven patients had acute osteomyelitis and eight patients had chronic osteomyelitis or inflammatory spondylitis. FDG-PET yielded 15 true-positive results. The tracer uptake correlated with the histopathological findings in each case. Bone scintigraphy performed in 11 patients yielded ten true-positive results and one false-negative result. Follow-up carried out on two patients revealed normal or clearly reduced tracer uptake, which correlated with a normalisation of clinical data. In early postoperative follow-up it was impossible to differentiate between postsurgical reactive changes and further infection using FDG-PET. It is concluded that acute and chronic osteomyelitis of the peripheral as well as the central skeleton can be detected using FDG-PET. Osteomyelitis can be differentiated from soft tissue infection surrounding the bone. Unlike computed tomography and magnetic resonance imaging, FDG-PET is not affected by metal implants used for fixing fractures. FDG-PET demonstrated promising initial results with respect to treatment monitoring. Nevertheless, in the early postoperative phase FDG-PET seems to be of limited value owing to unspecific tracer uptake. (orig.)

  14. Fluorine-18 fluorodeoxyglucose positron emission tomography in infectious bone diseases: results of histologically confirmed cases

    Energy Technology Data Exchange (ETDEWEB)

    Kaelicke, T. [Dept. of Surgery, University of Bochum (Germany); Schmitz, A.; Schmitt, O. [Dept. of Orthopaedics, University of Bonn (Germany); Risse, J.H.; Biersack, H.J. [Dept. of Nuclear Medicine, University of Bonn (Germany); Arens, S.; Hansis, M. [Dept. of Trauma Surgery, University of Bonn (Germany); Keller, E. [Department of Radiology, University of Bonn (Germany); Gruenwald, F. [Department of Nuclear Medicine, University of Frankfurt/Main (Germany)

    2000-05-01

    The aim of this study was to evaluate the clinical use of fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET) in acute and chronic osteomyelitis and inflammatory spondylitis. The study population comprised 21 patients suspected of having acute or chronic osteomyelitis or inflammatory spondylitis. Fifteen of these patients subsequently underwent surgery. FDG-PET results were correlated with histopathological findings. The remaining six patients, who underwent conservative therapy, were excluded from any further evaluation due to the lack of histopathological data. The histopathological findings revealed osteomyelitis or inflammatory spondylitis in all 15 patients: seven patients had acute osteomyelitis and eight patients had chronic osteomyelitis or inflammatory spondylitis. FDG-PET yielded 15 true-positive results. The tracer uptake correlated with the histopathological findings in each case. Bone scintigraphy performed in 11 patients yielded ten true-positive results and one false-negative result. Follow-up carried out on two patients revealed normal or clearly reduced tracer uptake, which correlated with a normalisation of clinical data. In early postoperative follow-up it was impossible to differentiate between postsurgical reactive changes and further infection using FDG-PET. It is concluded that acute and chronic osteomyelitis of the peripheral as well as the central skeleton can be detected using FDG-PET. Osteomyelitis can be differentiated from soft tissue infection surrounding the bone. Unlike computed tomography and magnetic resonance imaging, FDG-PET is not affected by metal implants used for fixing fractures. FDG-PET demonstrated promising initial results with respect to treatment monitoring. Nevertheless, in the early postoperative phase FDG-PET seems to be of limited value owing to unspecific tracer uptake. (orig.)

  15. Impact of Maternal Diet on the Epigenome during In Utero Life and the Developmental Programming of Diseases in Childhood and Adulthood

    Directory of Open Access Journals (Sweden)

    Ho-Sun Lee

    2015-11-01

    Full Text Available Exposure to environmental factors in early life can influence developmental processes and long-term health in humans. Early life nutrition and maternal diet are well-known examples of conditions shown to influence the risk of developing metabolic diseases, including type 2 diabetes mellitus and cardiovascular diseases, in adulthood. It is increasingly accepted that environmental compounds, including nutrients, can produce changes in the genome activity that, in spite of not altering the DNA sequence, can produce important, stable and, in some instances, transgenerational alterations in the phenotype. Epigenetics refers to changes in gene function that cannot be explained by changes in the DNA sequence, with DNA methylation patterns/histone modifications that can make important contributions to epigenetic memory. The epigenome can be considered as an interface between the genome and the environment that is central to the generation of phenotypes and their stability throughout the life course. To better understand the role of maternal health and nutrition in the initiation and progression of diseases in childhood and adulthood, it is necessary to identify the physiological and/or pathological roles of specific nutrients on the epigenome and how dietary interventions in utero and early life could modulate disease risk through epigenomic alteration.

  16. Serum levels of parathyroid hormone and markers of bone metabolism in patients with rheumatoid arthritis. Relationship to disease activity and glucocorticoid treatment

    DEFF Research Database (Denmark)

    Jensen, Tonny Joran; Hansen, M; Madsen, J C;

    2001-01-01

    OBJECTIVE: To evaluate the influence of inflammatory activity and glucocorticoid (GC) treatment on serum parathyroid hormone (s-PTH) and bone metabolism in patients with rheumatoid arthritis (RA). Furthermore, in patients with active RA, to examine the PTH secretion and Ca2+ set point before...... and after treatment with GC. METHODS: A range of biochemical markers of bone metabolism and calcium homeostasis were measured in 95 patients with definite RA stratified into groups according to disease activity and GC treatment. In a subgroup of 12 patients with active disease, initiating slow...... groups. The levels of urine pyridinoline (Pyr) and s-albumin-corrected calcium (s-AlbCorrCa2+) were elevated in patients with active disease and patients treated with GC. S-PTH and s-phosphate were within normal ranges. S-TAP, s-ICTP, Pyr and s-AlbCorrCa2+ correlated positively with indices of disease...

  17. Arresting rampant dental caries with silver diamine fluoride in a young teenager suffering from chronic oral graft versus host disease post-bone marrow transplantation: a case report

    OpenAIRE

    Chu, Chun-Hung; Lee, Angeline Hui-Cheng; Zheng, Liwu; Mei, May Lei; Chan, Godfrey Chi-fung

    2014-01-01

    BACKGROUND: Rampant caries is an advanced and severe dental disease that affects multiple teeth. This case describes the management of rampant caries in a young teenager suffering from chronic oral graft versus host disease after allogeneic bone marrow transplantation. CASE PRESENTATION: A 14-year-old Chinese boy suffering from beta-thalassemia major was referred to the dental clinic for the management of rampant dental caries. An oral examination revealed pale conjunctiva, bruising of lips, ...

  18. Bone Biopsy

    Science.gov (United States)

    ... Physician Resources Professions Site Index A-Z Bone Biopsy Bone biopsy uses a needle and imaging guidance ... limitations of Bone Biopsy? What is a Bone Biopsy? A bone biopsy is an image-guided procedure ...

  19. Exploring the role of FDG-PET in the assessment of bone marrow involvement in lymphoma patients as interpreted by qualitative and semiquantitative disease metabolic activity parameter

    Directory of Open Access Journals (Sweden)

    P G Kand

    2010-01-01

    Full Text Available Bone marrow biopsy (BMB is currently the standard method to evaluate marrow involvement in malignant lymphomas. However, there exist a number of pitfalls in this technique that can have important implications for initial staging, prognostification, and treatment of the disease. The present study was undertaken to investigate the utility of FDG-PET imaging in the detection of bone marrow involvement in untreated lymphoma patients. Forty untreated patients (36 males and 12 females with either Hodgkin′s disease (HD (n = 17 or non-Hodgkin′s lymphoma (NHL (n = 31 underwent whole body FDG-PET study for disease evaluation. Bone marrow uptake of FDG was graded as absence or presence of disease activity at marrow sites by qualitative assessment. Semiquantitative analysis involved deriving disease metabolic index (DMI using the following formula: DMI = SUV max of suitable circular ROI over PSIS or trochanteric region/ SUVmax of similar ROI over adjoining background. Findings of BMB and FDG-PET were compared for final analysis. Eleven out of 17 HD patients (12 males and 5 females demonstrated concordance between FDG PET findings and BMB reports. Remaining 6 cases showed discordance of FDG-PET demonstrating presence of marrow involvement at marrow sites and uninvolved marrow on BMB. Twenty six of the 31 NHL cases (24 males and 7 females demonstrated concordance between FDG PET findings and BMB reports. Remaining 5 cases showed discordance of FDG-PET demonstrating presence of marrow involvement at marrow sites and uninvolved marrow on BMB. All the BMB positive patients (2 of HD and 5 of NHL demonstrated disease activity in bone marrow on FDG-PET study. All patients with absence of disease activity at marrow sites on FDG-PET scan (9 of HD and 21 of NHL had histology proven uninvolved marrow. The quantitative assessment by DMI showed a mean of >2.5 in HD and NHL patients at the PSIS region and the trochanteric region bilaterally in cases of bone marrow

  20. Staphylococcal enterotoxin A regulates bone marrow granulocyte trafficking during pulmonary inflammatory disease in mice

    Energy Technology Data Exchange (ETDEWEB)

    Takeshita, W.M.; Gushiken, V.O.; Ferreira-Duarte, A.P.; Pinheiro-Torres, A.S.; Roncalho-Buck, I.A. [Department of Biology and Physiology, Faculty of Medicine of Jundiai (FMJ), Jundiai, SP (Brazil); Squebola-Cola, D.M.; Mello, G.C.; Anhê, G.F.; Antunes, E. [Department of Pharmacology, Faculty of Medical Sciences, University of Campinas (UNICAMP), Campinas, SP (Brazil); DeSouza, I.A., E-mail: ivanidesouza@uol.com.br [Department of Biology and Physiology, Faculty of Medicine of Jundiai (FMJ), Jundiai, SP (Brazil)

    2015-09-15

    Pulmonary neutrophil infiltration produced by Staphylococcal enterotoxin A (SEA) airway exposure is accompanied by marked granulocyte accumulation in bone marrow (BM). Therefore, the aim of this study was to investigate the mechanisms of BM cell accumulation, and trafficking to circulating blood and lung tissue after SEA airway exposure. Male BALB/C mice were intranasally exposed to SEA (1 μg), and at 4, 12 and 24 h thereafter, BM, circulating blood, bronchoalveolar lavage (BAL) fluid and lung tissue were collected. Adhesion of BM granulocytes and flow cytometry for MAC-1, LFA1-α and VLA-4 and cytokine and/or chemokine levels were assayed after SEA-airway exposure. Prior exposure to SEA promoted a marked PMN influx to BAL and lung tissue, which was accompanied by increased counts of immature and/or mature neutrophils and eosinophils in BM, along with blood neutrophilia. Airway exposure to SEA enhanced BM neutrophil MAC-1 expression, and adhesion to VCAM-1 and/or ICAM-1-coated plates. Elevated levels of GM-CSF, G-CSF, INF-γ, TNF-α, KC/CXCL-1 and SDF-1α were detected in BM after SEA exposure. SEA exposure increased production of eosinopoietic cytokines (eotaxin and IL-5) and BM eosinophil VLA-4 expression, but it failed to affect eosinophil adhesion to VCAM-1 and ICAM-1. In conclusion, BM neutrophil accumulation after SEA exposure takes place by integrated action of cytokines and/or chemokines, enhancing the adhesive responses of BM neutrophils and its trafficking to lung tissues, leading to acute lung injury. BM eosinophil accumulation in SEA-induced acute lung injury may occur via increased eosinopoietic cytokines and VLA-4 expression. - Highlights: • Airway exposure to SEA causes acute lung inflammation. • SEA induces accumulation of bone marrow (BM) in immature and mature neutrophils. • SEA increases BM granulocyte or BM PMN adhesion to ICAM-1 and VCAM-1, and MAC-1 expression. • SEA induces BM elevations of CXCL-1, INF-γ, TNF-α, GM-CSF, G-CSF and

  1. Staphylococcal enterotoxin A regulates bone marrow granulocyte trafficking during pulmonary inflammatory disease in mice

    International Nuclear Information System (INIS)

    Pulmonary neutrophil infiltration produced by Staphylococcal enterotoxin A (SEA) airway exposure is accompanied by marked granulocyte accumulation in bone marrow (BM). Therefore, the aim of this study was to investigate the mechanisms of BM cell accumulation, and trafficking to circulating blood and lung tissue after SEA airway exposure. Male BALB/C mice were intranasally exposed to SEA (1 μg), and at 4, 12 and 24 h thereafter, BM, circulating blood, bronchoalveolar lavage (BAL) fluid and lung tissue were collected. Adhesion of BM granulocytes and flow cytometry for MAC-1, LFA1-α and VLA-4 and cytokine and/or chemokine levels were assayed after SEA-airway exposure. Prior exposure to SEA promoted a marked PMN influx to BAL and lung tissue, which was accompanied by increased counts of immature and/or mature neutrophils and eosinophils in BM, along with blood neutrophilia. Airway exposure to SEA enhanced BM neutrophil MAC-1 expression, and adhesion to VCAM-1 and/or ICAM-1-coated plates. Elevated levels of GM-CSF, G-CSF, INF-γ, TNF-α, KC/CXCL-1 and SDF-1α were detected in BM after SEA exposure. SEA exposure increased production of eosinopoietic cytokines (eotaxin and IL-5) and BM eosinophil VLA-4 expression, but it failed to affect eosinophil adhesion to VCAM-1 and ICAM-1. In conclusion, BM neutrophil accumulation after SEA exposure takes place by integrated action of cytokines and/or chemokines, enhancing the adhesive responses of BM neutrophils and its trafficking to lung tissues, leading to acute lung injury. BM eosinophil accumulation in SEA-induced acute lung injury may occur via increased eosinopoietic cytokines and VLA-4 expression. - Highlights: • Airway exposure to SEA causes acute lung inflammation. • SEA induces accumulation of bone marrow (BM) in immature and mature neutrophils. • SEA increases BM granulocyte or BM PMN adhesion to ICAM-1 and VCAM-1, and MAC-1 expression. • SEA induces BM elevations of CXCL-1, INF-γ, TNF-α, GM-CSF, G-CSF and

  2. Effect of phylloquinone (vitamin K1) supplementation for 12 months on the indices of vitamin K status and bone health in adult patients with Crohn's disease.

    Science.gov (United States)

    O'Connor, Eibhlís M; Grealy, Geraldine; McCarthy, Jane; Desmond, Alan; Craig, Orla; Shanahan, Fergus; Cashman, Kevin D

    2014-10-14

    Although epidemiological findings support a role for vitamin K status in the improvement of bone indices in adult patients with Crohn's disease (CD), this needs to be confirmed in double-blind, randomised controlled trials (RCT) with phylloquinone (vitamin K1). By conducting two RCT, the present study aimed to first establish whether supplementation with 1000 μg of phylloquinone daily near-maximally suppresses the percentage of undercarboxylated osteocalcin in serum (%ucOC; marker of vitamin K status) in adult patients with CD currently in remission as it does in healthy adults and second determine the effect of supplementation with phylloquinone at this dose for 12 months on the indices of bone turnover and bone mass. The initial dose-ranging RCT was conducted in adult patients with CD (n 10 per group) using 0 (placebo), 1000 or 2000 μg of phylloquinone daily for 2 weeks. In the main RCT, the effect of placebo v. 1000 μg vitamin K/d (both co-administered with Ca (500 mg/d) and vitamin D3 (10 μg/d)) for 12 months (n 43 per group) on the biochemical indices of bone turnover (determined by enzyme immunoassay) and bone mass (determined by dual-energy X-ray absorptiometry) were investigated. At baseline, the mean %ucOC was 47 %, and this was suppressed upon supplementation with 1000 μg of phylloquinone daily ( - 81 %; P0·1) on bone turnover markers or on the bone mass of the lumbar spine or femur, but modestly increased (Pvitamin D3) had no effect on the indices of bone health in adult CD patients with likely vitamin K insufficiency.

  3. Ceacam1 separates graft-versus-host-disease from graft-versus-tumor activity after experimental allogeneic bone marrow transplantation.

    Directory of Open Access Journals (Sweden)

    Sydney X Lu

    Full Text Available Allogeneic bone marrow transplantation (allo-BMT is a potentially curative therapy for a variety of hematologic diseases, but benefits, including graft-versus-tumor (GVT activity are limited by graft-versus-host-disease (GVHD. Carcinoembryonic antigen related cell adhesion molecule 1 (Ceacam1 is a transmembrane glycoprotein found on epithelium, T cells, and many tumors. It regulates a variety of physiologic and pathological processes such as tumor biology, leukocyte activation, and energy homeostasis. Previous studies suggest that Ceacam1 negatively regulates inflammation in inflammatory bowel disease models.We studied Ceacam1 as a regulator of GVHD and GVT after allogeneic bone marrow transplantation (allo-BMT in mouse models. In vivo, Ceacam1(-/- T cells caused increased GVHD mortality and GVHD of the colon, and greater numbers of donor T cells were positive for activation markers (CD25(hi, CD62L(lo. Additionally, Ceacam1(-/- CD8 T cells had greater expression of the gut-trafficking integrin α(4β(7, though both CD4 and CD8 T cells were found increased numbers in the gut post-transplant. Ceacam1(-/- recipients also experienced increased GVHD mortality and GVHD of the colon, and alloreactive T cells displayed increased activation. Additionally, Ceacam1(-/- mice had increased mortality and decreased numbers of regenerating small intestinal crypts upon radiation exposure. Conversely, Ceacam1-overexpressing T cells caused attenuated target-organ and systemic GVHD, which correlated with decreased donor T cell numbers in target tissues, and mortality. Finally, graft-versus-tumor survival in a Ceacam1(+ lymphoma model was improved in animals receiving Ceacam1(-/- vs. control T cells.We conclude that Ceacam1 regulates T cell activation, GVHD target organ damage, and numbers of donor T cells in lymphoid organs and GVHD target tissues. In recipients of allo-BMT, Ceacam1 may also regulate tissue radiosensitivity. Because of its expression on both the

  4. Early Results of Clinical Application of Autologous Whole Bone Marrow Stem Cell Transplantation for Critical Limb Ischemia with Buerger's Disease.

    Science.gov (United States)

    Heo, Seon-Hee; Park, Yoong-Seok; Kang, Eun-Suk; Park, Kwang-Bo; Do, Young-Soo; Kang, Kyung-Sun; Kim, Dong-Ik

    2016-01-01

    Our goal was to evaluate early results of the clinical application of autologous whole bone marrow stem cell transplantation (AWBMSCT) for critical limb ischemia (CLI) in patients with Buerger's disease. We retrospectively analyzed the data of 58 limbs of 37 patients (mean age, 43.0 years; range, 28-63 years; male, 91.9%) with Buerger's disease with CLI who were treated with AWBMSCT from March 2013 to December 2014. We analyzed Rutherford category, pain score, pain-free walking time (PFWT), total walking time (TWT), ankle brachial pressure index (ABPI), and toe brachial pressure index (TBPI), and investigated wound healing and occurrence of unplanned amputations. The mean follow-up duration was 11.9 ± 7.2 months (range, 0.9-23.9 months) and 100%, 72.4%, and 74.1% of patients were available to follow-up 1, 3 and 6 months after AWBMST, respectively. At 6 months, patients demonstrated significant improvements in Rutherford category (P ABPI was increased compared to baseline, but the difference was not significant. A total of 76.5% ischemic wounds achieved complete or improved healing. AWBMSCT is a safe and effective alternative or adjunctive treatment modality to achieve clinical improvement in patients with CLI. PMID:26791280

  5. Relationship between body mass index, bone mineral density, and oral hygiene with periodontal disease in a Mexican elderly group.

    Directory of Open Access Journals (Sweden)

    José Francisco Murrieta

    2016-05-01

    Full Text Available The aim of this study was to evaluate the relationship of body mass index (BMI, bone mineral density (BMD, and oral hygiene with periodontal disease (PD in a group of elderly adults in Mexico City. Material and Methods: A cross-sectional study with a convenience sample of 151 elderly adults was conducted. Before applying the epidemiological survey, each subject was asked to sign an informed consent. Standardization for measuring Ramfjord’s Periodontal Disease Index (PDI, BMI, and Green and Vermilion’s OHI-S was carried out. Descriptive statistics and linear regression models were performed. Results: The 93.4% of the group had PD, 33.8% showed severe gingivitis and 20.5% mild gingivitis. A 28.5% five percent of the group had osteopenia and 18.5% had osteoporosis, being more common in people over 69 years. The 38.4% percent of the group was underweight and 53.0% had poor oral hygiene. Oral hygiene accounted for 63.1% of the PD variance (p=0.0001, figure that did not increase considerably by adding BMD and BMI variables to the regression model. Conclusion: The frequency of PD in this group of elderly adults was high and significantly associated with BMD, BMI, and mainly oral hygiene.

  6. Structural and mechanical differences between collagen homo- and heterotrimers: relevance for the molecular origin of brittle bone disease.

    Science.gov (United States)

    Chang, Shu-Wei; Shefelbine, Sandra J; Buehler, Markus J

    2012-02-01

    Collagen constitutes one-third of the human proteome, providing mechanical stability, elasticity, and strength to organisms. Normal type I collagen is a heterotrimer triple-helical molecule consisting of two α-1 chains and one α-2 chain. The homotrimeric isoform of type I collagen, which consists of three α-1 chains, is only found in fetal tissues, fibrosis, and cancer in humans. A mouse model of the genetic brittle bone disease, osteogenesis imperfect, oim, is characterized by a replacement of the α-2 chain by an α-1 chain, resulting also in a homotrimer collagen molecule. Experimental studies of oim mice tendon and bone have shown reduced mechanical strength compared to normal mice. The relationship between the molecular content and the decrease in strength is, however, still unknown. Here, fully atomistic simulations of a section of mouse type I heterotrimer and homotrimer collagen molecules are developed to explore the effect of the substitution of the α-2 chain. We calculate the persistence length and carry out a detailed analysis of the structure to determine differences in structural and mechanical behavior between hetero- and homotrimers. The results show that homotrimer persistence length is half of that of the heterotrimer (96 Å vs. 215 Å), indicating it is more flexible and confirmed by direct mechanical testing. Our structural analyses reveal that in contrast to the heterotrimer, the homotrimer easily forms kinks and freely rotates with angles much larger than heterotrimer. These local kinks may explain the larger lateral distance between collagen molecules seen in the fibrils of oim mice tendon and could have implications for reducing the intermolecular cross-linking, which is known to reduce the mechanical strength. PMID:22325288

  7. Autologous bone marrow mononuclear cell transplantation in patients with decompensated alcoholic liver disease: a randomized controlled trial.

    Directory of Open Access Journals (Sweden)

    Laurent Spahr

    Full Text Available OBJECTIVE: Impaired liver regeneration is associated with a poor outcome in patients with decompensated alcoholic liver disease (ALD. We assessed whether autologous bone marrow mononuclear cell transplantation (BMMCT improved liver function in decompensated ALD. DESIGN: 58 patients (mean age 54 yrs; mean MELD score 19, all with cirrhosis, 81% with alcoholic steatohepatitis at baseline liver biopsy were randomized early after hospital admission to standard medical therapy (SMT alone (n = 30, including steroids in patients with a Maddrey's score ≥32, or combined with G-CSF injections and autologous BMMCT into the hepatic artery (n = 28. Bone marrow cells were harvested, isolated and reinfused the same day. The primary endpoint was a ≥3 points decrease in the MELD score at 3 months, corresponding to a clinically relevant improvement in liver function. Liver biopsy was repeated at week 4 to assess changes in Ki67+/CK7+ hepatic progenitor cells (HPC compartment. RESULTS: Both study groups were comparable at baseline. After 3 months, 2 and 4 patients died in the BMMCT and SMT groups, respectively. Adverse events were equally distributed between groups. Moderate alcohol relapse occurred in 31% of patients. The MELD score improved in parallel in both groups during follow-up with 18 patients (64% from the BMMCT group and 18 patients (53% from the SMT group reaching the primary endpoint (p = 0.43 (OR 1.6, CI 0.49-5.4 in an intention to treat analysis. Comparing liver biopsy at 4 weeks to baseline, steatosis improved (p<0.001, and proliferating HPC tended to decrease in both groups (-35 and -33%, respectively. CONCLUSION: Autologous BMMCT, compared to SMT is a safe procedure but did not result in an expanded HPC compartment or improved liver function. These data suggest either insufficient regenerative stimulation after BMMCT or resistance to liver regenerative drive in patients with decompensated alcoholic cirrhosis. TRIAL REGISTRATION

  8. 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.

  9. Transfer of the inflammatory disease of HLA-B27 transgenic rats by bone marrow engraftment

    OpenAIRE

    1993-01-01

    We have previously produced lines of rats transgenic for HLA-B27 and human beta 2-microglobulin (h beta 2m) that develop a progressive inflammatory disease sharing many clinical and histologic features with the B27-associated human spondyloarthropathies, including gut and male genital inflammation, arthritis, and psoriasiform skin lesions. Other transgenic lines that express lower levels of B27 and h beta 2m remain healthy. To investigate the cellular basis for the multisystem inflammatory di...

  10. Shockwave treatment for musculoskeletal diseases and bone consolidation: qualitative analysis of the literature

    OpenAIRE

    Paulo Kertzman; Mario Lenza; André Pedrinelli; Benno Ejnisman

    2015-01-01

    Shockwave treatment is an option within orthopedics. The exact mechanism through which shockwaves function for treating musculoskeletal diseases is unknown. The aim of this study was to make a qualitative analysis on the effectiveness of shockwave treatment among patients with musculoskeletal pathological conditions and pseudarthrosis. Searches were conducted in the Cochrane Library, Medline and Lilacs databases. Thirty-nine studies that reported using shockwave treatment for musculoskeletal ...

  11. Liver Transplantation After Bone Marrow Transplantation for End Stage Liver Disease with Severe Hepatopulmonary Syndrome in Dyskeratosis Congenita: A Literature First.

    Science.gov (United States)

    Mahansaria, Shyam Sunder; Kumar, Senthil; Bharathy, Kishore G S; Kumar, Sachin; Pamecha, Viniyendra

    2015-12-01

    Dyskeratosis congenita is a multisystem genetic disorder. Although hepatic involvement is reported in about 7% of patients with dyskeratosis congenita, it is not well characterized and often attributed to hemochromatosis from frequent blood transfusions. A few case reports describe cirrhosis and hepatic cell necrosis in affected individuals in autosomal dominant pedigrees. Bone marrow failure and malignancies are the principal causes of death in dyskeratosis congenita. We describe the first case of living donor liver transplantation, in dyskeratosis congenita for decompensated cirrhosis with portal hypertension. The patient also had associated severe hepatopulmonary syndrome, interstitial lung disease, bilateral hip replacement for avascular necrosis of the head of femur, and a past history of bone marrow transplantation for bone marrow failure. PMID:26900277

  12. Developmental Work

    DEFF Research Database (Denmark)

    Møller, Niels; Hvid, Helge; Kristensen, Tage Søndergaard;

    2003-01-01

    Human Deveoplment and Working Life - Work for Welfare explores whether the development of human resources at company level can improve individuals' quality of life, companies' possibilities of development, and welfare and democracy in society. Chapter two discuss the concept "developmental work...

  13. Impact of cytomegalovirus and grafts versus host disease on the dynamics of CD57+CD28-CD8+ T cells after bone marrow transplant

    Directory of Open Access Journals (Sweden)

    Ana Verena Almeida Mendes

    2008-01-01

    Full Text Available OBJECTIVES: The present study aimed to evaluate the dynamics of CD28 and CD57 expression in CD8+ T lymphocytes during cytomegalovirus viremia in bone marrow transplant recipients. METHODS: In a prospective study, blood samples were obtained once weekly once from 33 healthy volunteers and weekly from 33 patients. To evaluate the expression of CD57 and CD28 on CD8+ T lymphocytes, flow cytometry analysis was performed on blood samples for four months after bone marrow transplant, together with cytomegalovirus antigenemia assays. RESULTS: Compared to cytomegalovirus-seronegative healthy subjects, seropositive healthy subjects demonstrated a higher percentage of CD57+ and a lower percentage of CD28+ cells (p<0.05. A linear regression model demonstrated a continuous decrease in CD28+ expression and a continuous increase in CD57+ expression after bone marrow transplant. The occurrence of cytomegalovirus antigenemia was associated with a steep drop in the percentage of CD28+ cells (5.94%, p<0.01 and an increase in CD57+ lymphocytes (5.60%, p<0.01. This cytomegalovirus-dependent effect was for the most part concentrated in the allogeneic bone marrow transplant patients. The development of acute graft versus host disease, which occurred at an earlier time than antigenemia (day 26 vs. day 56 post- bone marrow transplant, also had an impact on the CD57+ subset, triggering an increase of 4.9% in CD57+ lymphocytes (p<0.05. CONCLUSION: We found continuous relative changes in the CD28+ and CD57+ subsets during the first 120 days post- bone marrow transplant, as part of immune system reconstitution and maturation. A clear correlation was observed between the expansion of the CD57+CD28-CD8+ T lymphocyte subpopulation and the occurrence of graft versus host disease and cytomegalovirus viremia.

  14. Radiation treatment of bone hydatid disease%放射线治疗骨包虫病的研究

    Institute of Scientific and Technical Information of China (English)

    谢增如; 陈亮; 陈永明; 张耀; 温浩

    2013-01-01

    Objective Explore the efficacy that radiation (6WV-X-ray) treat bone hydatid disease.Methods Establish seventy gerbils bone hydatid disease animal models which were randomly divided into four groups,one group was to be control group,the animal models in other three groups were given 40 Gy/5 times,50 Gy/5 times,60 Gy/5 times,6MV-X-ray positioning radiotherapy,3 months later(the end of radiotherapy),to observe the gerbils,general situation,to compare the mortality of scolex,protein and calcium concentration in capsule fluid,to observe the pathological changes of the horny layers and germinal layers,the varying of the bone cells in the irradiation areas and the ultrastructure of echinococcus granulosus cysts.Results Over the past 3 months,gerbils in each group has death cases,but the survival ones,general behaviors were normal.Among the groups,the difference of the mortality of scolex(such as Control group:22.6,40 Gy/5 times group:96.6,50 Gy/5 times group:131.5,60 Gy/5 times group:155.5) was statistically significant (P < 0.01).Protein concentration (calcium ion concentration) in cyst fluid decrease (increase) with irradiation doses increase,The protein (calcium) concentrations are such as-the control group:1.0241 (2.8875),40 Gy/5 time group:0.7245 (3.0541),50 Gy/5 times group:0.6171 (3.5446) Gy to 60 Gy/5 times group:0.5058 (3.8241),which comgpared among each group there were significant differences(P < 0.01).Histopathological results showed that echinococcus sac in the treatment groups showed different degrees of degeneration or necrosis,which compared with control group there were significant changing.Compared between 40 Gy and 50 Gy group,40 Gy and 60 Gy group,the pathological changes of echinoccus sac were significant difference (P < 0.01),but between 50 Gy and 60 Gy group the pathological changes is not obvious (P > 0.05).The bone cells of the irradiated groups have been varying degrees of degeneration or necrosis.By electron microscopy,with the

  15. Shockwave treatment for musculoskeletal diseases and bone consolidation: qualitative analysis of the literature.

    Science.gov (United States)

    Kertzman, Paulo; Lenza, Mario; Pedrinelli, André; Ejnisman, Benno

    2015-01-01

    Shockwave treatment is an option within orthopedics. The exact mechanism through which shockwaves function for treating musculoskeletal diseases is unknown. The aim of this study was to make a qualitative analysis on the effectiveness of shockwave treatment among patients with musculoskeletal pathological conditions and pseudarthrosis. Searches were conducted in the Cochrane Library, Medline and Lilacs databases. Thirty-nine studies that reported using shockwave treatment for musculoskeletal diseases were found. Their results varied greatly, as did the types of protocol used. The studies that evaluated the effectiveness of shockwave treatment for lateral epicondylitis, shoulder tendinopathy, knee osteoarthrosis, femoral head osteonecrosis and trochanteric bursitis reported inconsistent results for most of their patients. Those that evaluated patients with calcifying tendinopathy, plantar fasciitis, Achilles tendinopathy, patellar tendinopathy and pseudarthrosis showed benefits. Shockwave treatment is a safe and non-invasive method for chronic cases in which conventional techniques have been unsatisfactory and should be used in association with other treatment methods for tendinopathy. Further quality studies are needed. PMID:26229889

  16. Shockwave treatment for musculoskeletal diseases and bone consolidation: qualitative analysis of the literature

    Directory of Open Access Journals (Sweden)

    Paulo Kertzman

    2015-02-01

    Full Text Available Shockwave treatment is an option within orthopedics. The exact mechanism through which shockwaves function for treating musculoskeletal diseases is unknown. The aim of this study was to make a qualitative analysis on the effectiveness of shockwave treatment among patients with musculoskeletal pathological conditions and pseudarthrosis. Searches were conducted in the Cochrane Library, Medline and Lilacs databases. Thirty-nine studies that reported using shockwave treatment for musculoskeletal diseases were found. Their results varied greatly, as did the types of protocol used. The studies that evaluated the effectiveness of shockwave treatment for lateral epicondylitis, shoulder tendinopathy, knee osteoarthrosis, femoral head osteonecrosis and trochanteric bursitis reported inconsistent results for most of their patients. Those that evaluated patients with calcifying tendinopathy, plantar fasciitis, Achilles tendinopathy, patellar tendinopathy and pseudarthrosis showed benefits. Shockwave treatment is a safe and non-invasive method for chronic cases in which conventional techniques have been unsatisfactory and should be used in association with other treatment methods for tendinopathy. Further quality studies are needed.

  17. Kinetics of lymphocyte reconstitution after allogeneic bone marrow transplantation: markers of graft-versus-host disease.

    Science.gov (United States)

    Zinöcker, Severin; Sviland, Lisbet; Dressel, Ralf; Rolstad, Bent

    2011-07-01

    GVHD causes extensive morbidity and mortality in patients who receive alloHCT. Predictive and reliable markers for GVHD are currently lacking but required to improve the safety and accessibility of alloHCT. We present an experimental rat model of myeloablative total body irradiation and fully mismatched major and minor histoincompatible, T cell-depleted BMT, followed by delayed infusion of donor lymphocytes. This treatment, in contrast to marrow transplantation alone, resulted in severe aGVHD and 100% lethality within 2-6 weeks. We investigated the reconstitution kinetics and phenotypes of donor leukocyte subpopulations as well as the histopathology of selected organs that may correlate with GVHD, with the goal to find potential disease-related markers. We observed histological changes mainly confined to the skin, with degenerative changes in the basal layer. LNs and spleen showed deranged architecture with markedly increased accumulation of lymphocytes, whereas the gut, liver, and lungs appeared normal. Of the lymphocyte markers tested, donor-derived CD62L(+) T cells were markedly decreased in animals suffering from GVHD. Furthermore, we observed peripheral depletion of CD4(+)CD25(hi)FoxP3(+) T(reg), which was in contrast to controls. The relative frequency of these lymphocyte subpopulations in blood may therefore serve as accessible cellular markers of aGVHD. We propose that the animal model presented is instructive for the identification of clinically relevant markers of GVHD, which could improve disease diagnosis and management in alloHCT.

  18. Evaluation of bone marrow infiltration in non-neuropathic Gaucher disease patients with use of whole-body MRI. A retrospective data analysis

    International Nuclear Information System (INIS)

    To evaluate whole-body magnetic resonance imaging (WB-MRI) for the assessment of bone marrow infiltration in patients with confirmed Gaucher disease type 1 under long-term enzyme replacement therapy (ERT). This retrospective data analysis included 38 patients in two subgroups. Group A: 10 females, 9 males, 15-29 years, mean age 22 years and Group B: 11 females, 8 males, 29-77 years, mean age 49 years, all treated with alglucerase or imiglucerase for at least 12.5 years. Whole-body MRI was carried out in all patients using a standard MRI protocol. Two radiologists assessed all MR images retrospectively with the use of three different MRI score systems: The bone marrow burden (BMB) score, the Duesseldorf-Gaucher score (DGS) and the vertebra disc ratio (VDR). As a clinical component, severity score index type 1 (GD-DS3) was determined. In both groups the MR scores showed low to moderate pathologic levels but no statistically significant difference was found between both groups. The median scores in group A/group B were 7.00/9.00 for the BMB score (p=0.07), 4.00/3.00 for the DGS score (p=0.062) and 1.54/1.62 for the VDR score (p=0.267). The GD-DS3 score was statistically significantly different between both groups (1.6/3.9, p=0.000) and osseous Gaucher disease complications were only found in group B. Bone marrow involvement and typical clinical manifestations are reduced to a minimum, when ERT starts immediately after the confirmed diagnosis of Gaucher disease type 1. The applied MR scores are useful markers to control bone marrow infiltration under enzyme replacement therapy in older patients. Pathologic MR scores in young patients may reflect postponed fat conversion of the juvenile bone marrow. This issue has to be examined in further studies.

  19. Evaluation of bone marrow infiltration in non-neuropathic Gaucher disease patients with use of whole-body MRI. A retrospective data analysis

    Energy Technology Data Exchange (ETDEWEB)

    Laudemann, K.; Moos, L.; Lollert, A.; Wagner, D.; Staatz, G. [University Medical Center of the Johannes Gutenberg University, Mainz (Germany). Section of Pediatric Radiology; Mengel, K.E.; Reinke, J.; Brixius-Huth, M. [University Medical Center of the Johannes Gutenberg University, Mainz (Germany). Clinic for Metabolic Diseases; Dueber, C. [University Medical Center of the Johannes Gutenberg University, Mainz (Germany). Dept. of Diagnostic and Interventional Radiology

    2015-12-15

    To evaluate whole-body magnetic resonance imaging (WB-MRI) for the assessment of bone marrow infiltration in patients with confirmed Gaucher disease type 1 under long-term enzyme replacement therapy (ERT). This retrospective data analysis included 38 patients in two subgroups. Group A: 10 females, 9 males, 15-29 years, mean age 22 years and Group B: 11 females, 8 males, 29-77 years, mean age 49 years, all treated with alglucerase or imiglucerase for at least 12.5 years. Whole-body MRI was carried out in all patients using a standard MRI protocol. Two radiologists assessed all MR images retrospectively with the use of three different MRI score systems: The bone marrow burden (BMB) score, the Duesseldorf-Gaucher score (DGS) and the vertebra disc ratio (VDR). As a clinical component, severity score index type 1 (GD-DS3) was determined. In both groups the MR scores showed low to moderate pathologic levels but no statistically significant difference was found between both groups. The median scores in group A/group B were 7.00/9.00 for the BMB score (p=0.07), 4.00/3.00 for the DGS score (p=0.062) and 1.54/1.62 for the VDR score (p=0.267). The GD-DS3 score was statistically significantly different between both groups (1.6/3.9, p=0.000) and osseous Gaucher disease complications were only found in group B. Bone marrow involvement and typical clinical manifestations are reduced to a minimum, when ERT starts immediately after the confirmed diagnosis of Gaucher disease type 1. The applied MR scores are useful markers to control bone marrow infiltration under enzyme replacement therapy in older patients. Pathologic MR scores in young patients may reflect postponed fat conversion of the juvenile bone marrow. This issue has to be examined in further studies.

  20. Skeletal blood flow in Paget's disease of bone and its response to calcitonin therapy.

    Science.gov (United States)

    Wootton, R; Reeve, J; Spellacy, E; Tellez-Yudilevich, M

    1978-01-01

    1. Blood flow to the skeleton was measured by the 18F clearance method of Wooton, Reeve & Veall (1976) in 24 patients with untreated Paget's disease. In every patient but one, resting skeletal blood flow was increased. There was a significant positive correlation between skeletal blood flow and serum alkaline phosphatase and between skeletal blood flow and urinary total hydroxyproline excretion. 2. Fourteen patients were re-studied after they had received short-term (7 days or less) or long-term (7 weeks or more) calcitonin. Skeletal blood flow, alkaline phosphatase and urinary hydroxy-proline excretion fell towards normal in every case. There was some evidence from the short-term studies that calcitonin produced a more rapid fall in skeletal blood flow than in alkaline phosphatase. 3. Glomerular filtration rate appeared to increase transiently in response to calcitonin.

  1. RNA Sequence Analysis of Human Huntington Disease Brain Reveals an Extensive Increase in Inflammatory and Developmental Gene Expression

    OpenAIRE

    Labadorf, Adam; Hoss, Andrew G.; Lagomarsino, Valentina; Latourelle, Jeanne C.; Hadzi, Tiffany C.; Bregu, Joli; MacDonald, Marcy E.; Gusella, James F.; Chen, Jiang-Fan; Akbarian, Schahram; Weng, Zhiping; Myers, Richard H

    2015-01-01

    Huntington’s Disease (HD) is a devastating neurodegenerative disorder that is caused by an expanded CAG trinucleotide repeat in the Huntingtin (HTT) gene. Transcriptional dysregulation in the human HD brain has been documented but is incompletely understood. Here we present a genome-wide analysis of mRNA expression in human prefrontal cortex from 20 HD and 49 neuropathologically normal controls using next generation high-throughput sequencing. Surprisingly, 19% (5,480) of the 28,087 confident...

  2. Hydatid disease of scapula and upper third of humerus treated by en bloc excision and fibular bone grafting

    Directory of Open Access Journals (Sweden)

    Chari P

    2007-01-01

    Full Text Available 35-year-old male patient presented with gradually increasing painful swelling of the right shoulder, which was incised and drained and wound persisted as a discharging sinus on the anterolateral aspect of the deltoid region with seropurulent discharge. A clinical diagnosis of tuberculosis of the shoulder was made. Plain skiagram of the right shoulder revealed multicystic lesion involving the entire scapula and upper third of the humerus with loss of joint space and pathological fracture at the junction of upper one-third and lower two-thirds of the humerus. A clinico-radiological diagnosis of hydatid disease was made. In view of the extensive involvement of the scapula with stiff shoulder and an active sinus, a two-stage surgical procedure was performed. Stage 1 consisted of en bloc excision of the scapula, upper half of the humerus and lateral end of the clavicle. Stage II surgery, consisting of fibular bone grafting. Tablet albendazole (400 mg, thrice daily was given as systemic scolicidal agent. This case is reported in view of it′s rarity and to highlight the management.

  3. Bone marrow involvement in Gaucher disease at MRI: what long-term evolution can we expect under enzyme replacement therapy?

    Energy Technology Data Exchange (ETDEWEB)

    Fedida, Benjamin; Touraine, Sebastien; Laredo, Jean-Denis [Hopital Lariboisiere, AP-HP, Department of Musculoskeletal Imaging, Paris (France); Stirnemann, Jerome [Universite Paris-Diderot Hopital Bichat, AP-HP, Department of Biostatistics and Medical Data Processing, INSERM UMR 738, Paris (France); Geneva University Hospital, Division of General Internal Medicine, Faculty of Medicine, Geneva (Switzerland); Belmatoug, Nadia [Hopital Beaujon, AP-HP, Referral Center for Lysosomal Diseases (RCLD), Clichy (France); Hopital Beaujon, AP-HP, Department of Internal Medicine, Clichy (France); Petrover, David [Hopital Lariboisiere, AP-HP, Department of Musculoskeletal Imaging, Paris (France); Hopital Beaujon, AP-HP, Referral Center for Lysosomal Diseases (RCLD), Clichy (France)

    2015-10-15

    To study the long-term evolution of the bone marrow burden (BMB) score at MRI in patients with Gaucher disease (GD) under enzyme replacement therapy (ERT). Forty patients treated for GD were retrospectively studied in a referral centre. BMB scores were assessed on spine and femur MR examinations performed between January 2003 and June 2014. The long-term evolution of the BMB scores was analyzed using a linear mixed model. A total of 121 MRI examinations were performed during the study period with a mean follow-up of 7.1 years ± 5.6, an average rate of 3.1 MR examinations ± 1.7 per patient and an interval of 2.3 years ± 1.1 between examinations. Patients had received ERT during 12 years on average ± 6.7. The trend of BMB scores with time decreased significantly by 15 % (P = 0.008) during the total study period and 39 % (P = 0.01) during the first 5 years of treatment. No changes in BMB scores were observed after five years of treatment. In Gaucher patients, the trend of MRI BMB scores with time decreased significantly under ERT the first 5 years of treatment before a long-term stabilization. (orig.)

  4. Intravenous transplantation of bone marrow-derived mononuclear cells prevents memory impairment in transgenic mouse models of Alzheimer's disease.

    Science.gov (United States)

    Kanamaru, Takuya; Kamimura, Naomi; Yokota, Takashi; Nishimaki, Kiyomi; Iuchi, Katsuya; Lee, Hyunjin; Takami, Shinya; Akashiba, Hiroki; Shitaka, Yoshitsugu; Ueda, Masayuki; Katsura, Ken-Ichiro; Kimura, Kazumi; Ohta, Shigeo

    2015-04-24

    Stem cell transplantation therapy is currently in clinical trials for the treatment of ischemic stroke, and several beneficial aspects have been reported. Similarly, in Alzheimer's disease (AD), stem cell therapy is expected to provide an efficient therapeutic approach. Indeed, the intracerebral transplantation of stem cells reduced amyloid-β (Aβ) deposition and rescued memory deficits in AD model mice. Here, we show that intravenous transplantation of bone marrow-derived mononuclear cells (BMMCs) improves cognitive function in two different AD mouse models, DAL and APP mice, and prevents neurodegeneration. GFP-positive BMMCs were isolated from tibiae and femurs of 4-week-old mice and then transplanted intravenously into DAL and APP mice. Transplantation of BMMCs suppressed neuronal loss and restored memory impairment of DAL mice to almost the same level as in wild-type mice. Transplantation of BMMCs to APP mice reduced Aβ deposition in the brain. APP mice treated with BMMCs performed significantly better on behavioral tests than vehicle-injected mice. Moreover, the effects were observed even with transplantation after the onset of cognitive impairment in DAL mice. Together, our results indicate that intravenous transplantation of BMMCs has preventive effects against the cognitive decline in AD model mice and suggest a potential therapeutic effect of BMMC transplantation therapy.

  5. Mineral and Bone Disorder and Its Association with Cardiovascular Parameters in Chinese Patients with Chronic Kidney Disease

    Science.gov (United States)

    Zhou, Chu; Wang, Fang; Wang, Jin-Wei; Zhang, Lu-Xia; Zhao, Ming-Hui

    2016-01-01

    Background: Mineral and bone disorder (MBD), especially hyperphosphatemia, is an independently risk factor for adverse prognosis in patients with chronic kidney disease (CKD). However, CKD-MBD among Chinese population was poorly studied. This study aimed to investigate the status of MBD and its association with cardiovascular parameters in Chinese patients with predialysis CKD. Methods: Chinese Cohort Study of Chronic Kidney Disease (C-STRIDE) is a prospective multicenter cohort study involving predialysis CKD patients in China. Markers of MBD, including serum phosphorus, calcium, and intact parathyroid hormone, were measured in baseline samples at the patients’ entry. The association between serum phosphorus and abdominal aortic calcification (AAC), left ventricular hypertrophy (LVH) were examined by logistic regression models. Results: Altogether 3194 predialysis patients with mean estimated glomerular filtration of 51.8 ± 33.1 ml·min−1.1.73 m−2 were included. The proportion of patients with hyperphosphatemia were 2.6%, 2.9%, 6.8%, and 27.1% in CKD Stages 3a, 3b, 4, and 5, respectively. Moreover, 71.6% of the patients with hyperphosphatemia did not receive any phosphate-binder (PB). Lateral abdominal X-rays were obtained in 2280 patients, 9.8% of the patients were diagnosed as having AAC. Altogether 2219 patients had data of echocardiography, and 13.2% of them were diagnosed with LVH. Multivariate logistic regression analysis showed that serum phosphorus was independently associated with the presence of AAC and LVH. Conclusions: In Chinese patients with CKD, the percentage of hyperphosphatemia is comparable to that of other countries while the usage of PBs is suboptimal. The prevalence of vascular calcification in Chinese patients is relatively lower compared with the Caucasian population. PMID:27647184

  6. Chinese expert consensus statement on clinical diagnosis and treatment of malignant tumor bone metastasis and bone related diseases%恶性肿瘤骨转移及骨相关疾病临床诊疗中国专家共识

    Institute of Scientific and Technical Information of China (English)

    Shiying Yu; Zefei Jiang; Li Zhang; Xiaohui Niu; Lvhua Wang; Ning Wu; Jianhui Ma

    2010-01-01

    @@ Clinical diagnosis and treatment of malignant tumor bone metastases Overview Bone is the most common place of metastases in ad vanced malignant diseases. Constant improvement in the treatment of malignant tumors has resulted in prolonged survival time, and so as an increased incidence of osseous metastases and skeletal complications.

  7. 早产儿代谢性骨病防治进展%Metabolic bone disease in the preterm newborn:an update on prevention and treatment

    Institute of Scientific and Technical Information of China (English)

    王丹虹(综述); 陈平洋(审校)

    2014-01-01

    Metabolic bone disease is one of the common complications in preterm neonates,which has important influence on the quality of life,even increases the risk of adulthood osteoporosis. Early diagnosis and therapy are important for the improvement of outcome of preterm neonates. This article reviews the progress of prevention and treatment of metabolic bone disease in preterm neonates.%早产儿代谢性骨病是早产儿常见的并发症,对其生存质量会造成重要影响,甚至增加成年期骨质疏松的风险。早期干预能有效地减少代谢性骨病的发生,改善早产儿预后。该文对早产儿代谢性骨病的防治进展作一综述。

  8. The effect of intrathecal delivery of bone marrow stromal cells on hippocampal neurons in rat model of Alzheimer’s disease

    Directory of Open Access Journals (Sweden)

    Mina Eftekharzadeh

    2015-05-01

    Full Text Available Objective(s: Intracerebral injection of bone marrow stromal cells (BMSCs is being investigated as a therapeutic tool to prevent Alzheimer's disease (AD. Our aim was to investigate the effects of BMSCs by intrathecal injection in AD rat model. Materials and Methods: BMSCs were obtained from the bone marrow of Wistar rat and transplanted into AD rat model via intrathecal injection. The rat model had received an injection of β amyloid into the hippocampus for histological and immunohistochemical studies. Results: Histological examination of the brains in transplanted rats compared to controls demonstrated the migration of BrdU-labeled BMSCs from the site of delivery, confirmed the differentiation of BMSCs transplanted cells into the cholinergic neurons, and increased number of healthy and decreased number of dark neurons. Conclusion: Our results showed that BMSCs intratechal administration could be a promising method for treatment ofAlzheimer’s disease in rat model.

  9. Tapentadol prolonged release for patients with multiple myeloma suffering from moderate-to-severe cancer pain due to bone disease

    Directory of Open Access Journals (Sweden)

    Coluzzi F

    2015-05-01

    Full Text Available Flaminia Coluzzi,1,2 Robert B Raffa,3 Joseph Pergolizzi,4 Alessandra Rocco,1 Pamela Locarini,1 Natalia Cenfra,5 Giuseppe Cimino,5 Consalvo Mattia1,2 1Department of Medical and Surgical Sciences and Biotechnologies, Faculty of Pharmacy and Medicine, Unit of Anaesthesiology, Intensive Care Medicine and Pain Therapy, Polo Pontino, Sapienza University of Rome, Latina, Italy; 2SIAARTI Study Group on Acute and Chronic Pain, Rome, Italy; 3Department of Pharmaceutical Sciences, Temple University School of Pharmacy, Philadelphia, PA, USA; 4Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA; 5Department of Cellular Biotechnology and Hematology, Sapienza University of Rome, Rome, Italy Context: Myeloma bone disease (MBD is a devastating complication of multiple myeloma that leads to severe pain. Objectives: The aim of this study was to evaluate the efficacy and tolerability of tapentadol prolonged release (PR in the management of patients with MBD suffering from moderate-to-severe cancer pain. Methods: A 12-week prospective study was carried out in 25 opioid-naïve MBD patients. Patients initially received twice-daily doses of tapentadol PR 50 mg. Doses were then managed to maintain adequate relief or dose-limiting toxicity. The following parameters were recorded at weekly intervals for 4 weeks, and then at weeks 8 and 12: pain, opioid-related adverse effects, use of other analgesics, DN4 (Douleur Neuropathique 4 score. Quality of life (SF-36 [36-item short-form health survey] was measured at baseline and at final evaluation. Results: Of 25 patients, 22 completed the study. Pain intensity significantly decreased from baseline to all the week intervals (P<0.01. Quality of life significantly improved with respect to all SF-36 subscale parameters (P<0.01, and so did both the physical and mental status (P<0.01. Tapentadol PR significantly reduced DN4 mean value (P<0.01 and the number of patients with neuropathic component

  10. Reduced graft-versus-host disease-inducing capacity of T cells after activation, culturing, and magnetic cell sorting selection in an allogeneic bone marrow transplantation model in rats

    NARCIS (Netherlands)

    Weijtens, M; van Spronsen, A; Hagenbeek, A; Braakman, E; Martens, A

    2002-01-01

    Graft-versus-host disease (GvHD), a major complication of allogeneic bone marrow transplantation, has been ascribed to mature T cells in the graft. Because T cells play an important role in engraftment of the bone marrow and decrease the probability of relapse of leukemia, a treatment strategy was d

  11. [Structural and functional characteristics of bone tissue and blood cytokines in health and disease of the joints].

    Science.gov (United States)

    Kariakina, E V; Norkin, I A; Gladkova, E V; Persova, E A; Matveeva, O V; Puchin'ian, D M

    2014-02-01

    Change of structural and functional state of bone in patients with primary osteoarthrosis of the hip joint compared to healthy individuals is characterized by decreased bone formation with a relative predominance of resorption. Osteopenic syndrome develops in the background of evident imbalance of a blood cytokine profile with increasing the level of proinflammatory and the variability of the level of anti-inflammatory cytokines.

  12. A case of Paget's disease of bone with mycosis fungoides%Paget's骨病伴皮肤蕈样霉菌病一例报告

    Institute of Scientific and Technical Information of China (English)

    卓勤俭; 王瑞明; 苏小芳; 孟彩云; 郑恂; 段京明; 况成国

    2008-01-01

    @@ Paget's骨病(Paget's disease of bone,PDB),又称畸形性骨炎,系原因不明骨吸收增加的缓慢、局限性骨骼病变.可致骨再建异常,新形成不正常骨质增加,局部骨骼膨大、疏松、血管增多,易畸形和骨折.

  13. A Novel Role for CCL3 (MIP-1α) in Myeloma-induced Bone Disease via Osteocalcin Downregulation and Inhibition of Osteoblast Function

    OpenAIRE

    Vallet, Sonia; Pozzi, Samantha; Patel, Kishan; Vaghela, Nileshwari; Fulciniti, Mariateresa; Veiby, Petter; Hideshima, Teru; Santo, Loredana; Cirstea, Diana; Scadden, David T.; Anderson, Kenneth C.; Raje, Noopur

    2011-01-01

    Upregulation of cytokines and chemokines is a frequent finding in multiple myeloma (MM). CCL3 (also known as MIP-1α) is a pro-inflammatory chemokine whose levels in the MM microenvironment correlate with osteolytic lesions and tumor burden. CCL3 and its receptors, CCR1 and CCR5, contribute to the development of bone disease in MM by supporting tumor growth and regulating osteoclast (OC) differentiation. Here, we identify inhibition of osteoblast (OB) function as an additional pathogenic mecha...

  14. Bone biomarkers help grading severity of coronary calcifications in non dialysis chronic kidney disease patients.

    Directory of Open Access Journals (Sweden)

    Marion Morena

    Full Text Available BACKGROUND: Osteoprotegerin (OPG and fibroblast growth factor-23 (FGF23 are recognized as strong risk factors of vascular calcifications in non dialysis chronic kidney disease (ND-CKD patients. The aim of this study was to investigate the relationships between FGF23, OPG, and coronary artery calcifications (CAC in this population and to attempt identification of the most powerful biomarker of CAC: FGF23? OPG? METHODOLOGY/PRINCIPAL FINDINGS: 195 ND-CKD patients (112 males/83 females, 70.8 [27.4-94.6] years were enrolled in this cross-sectional study. All underwent chest multidetector computed tomography for CAC scoring. Vascular risk markers including FGF23 and OPG were measured. Logistic regression analyses were used to study the potential relationships between CAC and these markers. The fully adjusted-univariate analysis clearly showed high OPG (≥10.71 pmol/L as the only variable significantly associated with moderate CAC ([100-400[ (OR = 2.73 [1.03;7.26]; p = 0.04. Such association failed to persist for CAC scoring higher than 400. Indeed, severe CAC was only associated with high phosphate fractional excretion (FEPO(4 (≥38.71% (OR = 5.47 [1.76;17.0]; p = 0.003 and high FGF23 (≥173.30 RU/mL (OR = 5.40 [1.91;15.3]; p = 0.002. In addition, the risk to present severe CAC when FGF23 level was high was not significantly different when OPG was normal or high. Conversely, the risk to present moderate CAC when OPG level was high was not significantly different when FGF23 was normal or high. CONCLUSIONS: Our results strongly suggest that OPG is associated to moderate CAC while FGF23 rather represents a biomarker of severe CAC in ND-CKD patients.

  15. Bone within a bone

    Energy Technology Data Exchange (ETDEWEB)

    Williams, H.J.; Davies, A.M. E-mail: wendy.turner@roh.nhs.uk; Chapman, S

    2004-02-01

    The 'bone within a bone' appearance is a well-recognized radiological term with a variety of causes. It is important to recognize this appearance and also to be aware of the differential diagnosis. A number of common conditions infrequently cause this appearance. Other causes are rare and some remain primarily of historical interest, as they are no longer encountered in clinical practice. In this review we illustrate some of the conditions that can give the bone within a bone appearance and discuss the physiological and pathological aetiology of each where known.

  16. Genetic epidemiology of Paget's disease of bone in italy: sequestosome1/p62 gene mutational test and haplotype analysis at 5q35 in a large representative series of sporadic and familial Italian cases of Paget's disease of bone.

    Science.gov (United States)

    Falchetti, Alberto; Di Stefano, Marco; Marini, Francesca; Ortolani, Sergio; Ulivieri, Massimo Fabio; Bergui, Simona; Masi, Laura; Cepollaro, Chiara; Benucci, Maurizio; Di Munno, Ombretta; Rossini, Maurizio; Adami, Silvano; Del Puente, Antonio; Isaia, Giancarlo; Torricelli, Francesca; Brandi, Maria Luisa

    2009-01-01

    Families affected by Paget's disease of bone frequently harbor mutations in the SQSTM1/p62 gene. In this multicentric study we collected 345 sporadic and 12 familial PDB cases throughout Italy, identifying 12 different mutations, 5 of which are newly reported and 3, D335E, A381V, and Y383X, external to the UBA domain. Subjects with truncating mutations, E396X, showed a significantly younger age at clinical diagnosis, while the Y383X subjects had a higher average number of affected skeletal sites. All the mutants exhibited the CGTG-H2 haplotype. In two pairs and one triad of unrelated Italian PDB families from different Italian regions, we detected a common SQSTM1/p62 mutation for each P392L, M404V, and G425R group. Since the CGTG-H2 haplotype frequency was also high in normal subjects, and genetic influence due to migratory fluxes of different ethnic groups exists in the Italian population, to refine the search for a more geographically specific founder effect, we extended the haplotype analysis in these families using polymorphic microsatellite repeat markers, within and flanking the SQSTM1/p62 locus, from chromosome 5q35, other than the exon 6 and 3'UTR polymorphisms. All mutant carriers from two of the three M404V families and from the G425R families exhibited common extended chromosome 5q35 haplotypes, IT01 and IT02, respectively, which may be reflecting influences of past migrations. This may be helpful in estimating the true rate of de novo mutations. We confirm the data on the existence of both a mutational hotspot at the UBA domain of SQSTM1/p62 and a founder effect in the PDB population.

  17. D-D dimer levels in patients with sickle cell disease during bone pain crises and in the steady state

    Directory of Open Access Journals (Sweden)

    Fakunle EE

    2012-05-01

    Full Text Available Eyitayo Emmanuel Fakunle,1 Kapoona Iwara Ibiang Eteng,2 Wuraola Adebola Shokunbi21Department of Pathology, King Edward VII Memorial Hospital, Bermuda; 2Department of Hematology, University College Hospital, Ibadan, NigeriaObjective: To determine the presence of ongoing thrombosis by measuring the D-D dimer levels in bone pain crises (BPCs and in the steady state of patients with sickle cell disease, comparing these levels with those in individuals with normal hemoglobin (HbAA in southwest Nigeria.Study design, patients, and methods: The study design involved 38 patients with homozygous sickle cell anemia (HbSS and 78 adults with the HbAA phenotype, seen at the Hematology Day Care and Accident and Emergency units of the University College Hospital, Ibadan, Nigeria. The TintElize kit was used to quantitatively determine human D-D dimer levels in the plasma with enzyme immunoassay.Results: The mean D-D dimer level measured of the 78 individuals with HbAA was 73.59 ng/mL. The mean D-D dimer level of the patients with HbSS during BPCs was 4002.40 ng/mL, while the mean level in the same patients in the steady state measured 6 weeks after their BPCs, with no other painful crisis episode before the sample was collected, was 1320.00 ng/mL.Conclusion: This study demonstrated a significant increase in the D-D dimer levels of patients with HbSS in the steady state, when compared with those of individuals with HbAA of the same age and sex distribution. There was also an approximate threefold increase in the D-D dimer levels in the same patients with HbSS during BPCs. This confirms the activation of coagulation and fibrinolytic systems in patients with HbSS in the steady state, which is further escalated during BPCs. A multicenter study on the use of anticoagulants in BPCs in patients with sickle cell disease is required.Keywords: anticoagulant, dimer, sickle cell disease, BPC, Nigeria, chronic hemolytic anemia

  18. Bone Regeneration in Odontostomatology

    OpenAIRE

    Tonelli, P; Duvina, M.; Brancato, L.; Delle Rose, G.; Biondi, E.; Civitelli, V.

    2010-01-01

    Maxillary edentulism, together with periodontal disease, is the condition that most frequently induces disruption of alveolar bone tissue. Indeed, the stimulus of the periodontal ligament is lost and the local bone tissue becomes subject to resorption processes that, in the six months following the loss of the tooth, result in alveolar defects or more extensive maxillary atrophy. In both cases, loss of vestibular cortical bone is followed by reduction in the vertical dimension of the alveolar...

  19. Metabolic bone disease and central retinal degeneration in a kitten due to nutritional inadequacy of an all-meat raw diet

    Directory of Open Access Journals (Sweden)

    Catherine Lenox

    2015-05-01

    Full Text Available A 5-month-old castrated male Sphynx kitten presented with left hindlimb lameness shortly after adoption. Prior to adoption, the breeder had fed the kitten an exclusively raw chicken diet. Radiographs revealed generalized osteopenia and a left tibia–fibula fracture. Ophthalmic examination revealed corneal vascularization and opacity in the right eye, and lesions suggestive of feline central retinal degeneration in the left eye. The patient’s diagnoses included metabolic bone disease and feline central retinal degeneration, which can result from taurine deficiency. The kitten’s nutritional diseases were managed with a complete and balanced canned diet designed for kitten growth and with taurine supplementation.

  20. 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;

    2015-01-01

    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...... only differed from patients without in terms of plasma phosphate levels (1·95 ± 0·15 (n = 37) versus 1·27 ± 0·08 (n = 26), P = 0·0012). All patients with myocardial uptake (n = 27) had a coronary arteriography-verified history of coronary artery disease (CAD), whereas CAD was only present in six...