Osteoporosis: Bianchi ML

Gordon CM, Baim S, Bianchi ML, Bishop NJ, Hans DB, Kalkwarf H, Langman C, Leonard MB, Plotkin H, Rauch F, Zemel BS, Anonymous00043. · Children's Hospital Boston, Boston, MA 02115, USA. · South Med J. · Pubmed #18580718 No free full text.

Abstract: The International Society for Clinical Densitometry periodically holds Position Development Conferences (PDCs) for the purpose of establishing standards and guidelines for the assessment of skeletal health, including nomenclature, indications, acquisition, analysis, quality control, interpretation, and reporting of bone density tests. Topics are selected for consideration according to criteria that include clinical relevancy, uncertainty in the application of medical evidence to clinical practice, and the likelihood of the expert panel to reach a consensus agreement. The first Pediatric PDC was June 20 to 21, 2007 in Montreal, Quebec, Canada. Topics included fracture prediction and definition of osteoporosis in children; dual-energy x-ray absorptiometry (DXA) assessment in children with chronic disease that may affect the skeleton; DXA interpretation and reporting in children and adolescents; and the use of peripheral quantitative computed tomography in children and adolescents. This report describes the methodology and presents the results of this recent PDC.

Bishop N, Braillon P, Burnham J, Cimaz R, Davies J, Fewtrell M, Hogler W, Kennedy K, Mäkitie O, Mughal Z, Shaw N, Vogiatzi M, Ward K, Bianchi ML. · Sheffield University, Sheffield Childrens NHS Foundation Trust, Western Bank, Sheffield, UK. <> · J Clin Densitom. · Pubmed #18442751 No free full text.

Abstract: The Task Force focusing on the use of dual energy X-ray absorptiometry (DXA) in children and adolescents with diseases that may affect the skeleton reviewed over 300 articles to establish the basis for the Official Positions. A significant number of studies used DXA-based outcome measures to assess the effects of specific interventions and charted the natural history of incremental changes in bone size and mass in specific disease states in children. However, the utility of DXA in clinical practice has not been evaluated systematically, in large part due to the lack of a workable definition for childhood osteoporosis. Thus, in combination with the Official Positions addressing the diagnosis of osteoporosis in children, and the reporting of DXA results in children, this document presents clear guidelines from which clinicians and researchers alike can work. This report delineates a set of disorders in which it is appropriate to use DXA as part of the comprehensive assessment of skeletal health in children and adolescents, and provides guidance concerning the initiation of assessment and the frequency of monitoring. Importantly, this document also highlights significant gaps in our knowledge, emphasizing areas for future research.

Bianchi ML. · Bone Metabolism Unit, Istituto Auxologico Italiano IRCCS Via L. Ariosto 13 20145, Milano, Italy. · Bone. · Pubmed #17706477 No free full text.

Abstract: In recent years, the issue of low bone density in children and adolescents has attracted much attention. The classical definition of osteoporosis should be valid at any age, yet its practical applicability to children and adolescents remains a matter of debate and there is no consensus on a diagnosis based solely on the BMD value. The clinical relevance of uncomplicated low bone density in the young and its long-term consequences remain difficult to evaluate and there is only preliminary evidence that the BMD value is a predictor of fracture risk in growing subjects. Moreover, the interpretation of densitometric data in the young is difficult because the "normal" BMD values to be used for comparison are continuously changing with age, and in addition, depend on several variables, such as gender, body size, pubertal stage, skeletal maturation and ethnicity. Although Z-score values below -2 are generally considered a serious warning, most bone specialists make a diagnosis of osteoporosis in children and adolescents only in the presence of low BMD and at least one fragility fracture. The scope of this review is limited to presenting a picture of the available knowledge. The literature on fractures will be presented in detail, since fractures are one of the key elements in the debate. There are countless papers on fractures in childhood and adolescence, but very few of them attempt to identify fragility fractures, and still fewer develop the concept of osteoporosis in the young in relation to fractures. The different forms of primary and secondary osteoporosis, the more technical aspects of bone densitometry in pediatrics, and the delicate issue of treatment will be discussed only briefly.

Bianchi ML. · Bone Metabolism Unit, Istituto Auxologico Italiano, IRCCS, Via L. Ariosto, 13, Milano, Italy. · Best Pract Res Clin Rheumatol. · Pubmed #16301193 No free full text.

Abstract: Osteoporosis is increasingly being seen in young people. The primitive forms are relatively rare, but the secondary forms--particularly in long-term corticosteroid therapy--are a relevant problem given the much longer survival in chronic diseases such as cystic fibrosis, chronic renal insufficiency, leukaemia, and Duchenne muscular dystrophy. Controlled, prospective studies to evaluate the results of prevention and therapy in children are still lacking. The basis of therapy is the correct daily intake of calcium and the use of vitamin D (or active metabolites). This helps the growing skeleton to restore its equilibrium in many cases. Restraining the long-term use of corticosteroids to the minimum effective dose and shorter duration is essential. In severe cases, particularly in the presence of fractures, bisphosphonates can be remarkably effective. In some cases, such as idiopathic juvenile osteoporosis, the rule is spontaneous resolution, and the advisability of an aggressive drug therapy is discussed.

Bianchi ML, Bardella MT. · Bone Metabolic Unit, Istituto Auxologico Italiano-IRCCS Via Ludovico Ariosto, 13, 20145, Milan, Italy. · Calcif Tissue Int. · Pubmed #12232681 No free full text.

Abstract: Celiac disease is an intestinal disease due to an abnormal immuno-mediated response to gluten and other peptides from different cereals in genetically susceptible subjects. Several systemic alterations, including bone alterations, may be present in affected subjects. Once considered rare, it is now known to be quite frequent in both Europe and North America, as the recent availability of specific serological markers has drastically changed our perspective on its prevalence. The diagnosis of celiac disease may be very difficult because the clinical picture is highly variable and the characteristic intestinal signs and symptoms may be completely absent. Among the extra-intestinal alterations, bone mass decrease and bone metabolism derangement are frequently present and can be the only signs of an otherwise silent celiac disease. Clinical and epidemiological data are now plentiful but no conclusive data on the pathogenesis of bone involvement in celiac disease are available yet. Bone alterations were once thought to derive from calcium and vitamin D deficiency secondary to simple intestinal malabsorption, but now a more complex interaction between cytokines and local/systemic factors influencing bone formation and reabsorption is envisaged, Also, there is now substantial evidence supporting a lifelong gluten-free diet as the first-choice therapy for celiac disease, and as far as we know, this is the only effective measure to restore bone metabolism to an apparent normality. In the young, an early-started gluten-free diet can even lead to a satisfactory recovery of bone mass. In adults, however, there is no spontaneous recovery, and there are no conclusive data on the efficacy of standard therapies for osteoporosis in reducing the fracture risk. For these reasons, we feel that a review of the clinical findings on bone problems in celiac disease may be useful for both gastroenterologists and osteoporosis specialists.

Bianchi ML. · Bone Metabolic Unit, Istituto Auxologico Italiano, IRCCS, Milano, Italy. ml.bianchi@auxo logico.it · Calcif Tissue Int. · Pubmed #11960203 No free full text.

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Bianchi ML, Cimaz R, Bardare M, Zulian F, Lepore L, Boncompagni A, Galbiati E, Corona F, Luisetto G, Giuntini D, Picco P, Brandi ML, Falcini F. · Università di Milano, Milan, Italy. · Arthritis Rheum. · Pubmed #11014345 links to  free full text

Abstract: OBJECTIVE: Osteopenia/osteoporosis is being increasingly reported as a complication of many chronic diseases, even in children. In this preliminary study, we evaluated the effect of an oral bisphosphonate (alendronate) on bone mass in children with diffuse connective tissue diseases. METHODS: Thirty-eight children with low bone mass were treated with alendronate for 1 year; 38 children who had the same primary disorders as the study patients but in a less severe form served as untreated control patients. We were also able to evaluate changes in bone mass (before and after alendronate) in 16 of the treated patients whose bone mineral density (BMD) had been routinely measured before the present study was initiated. RESULTS: BMD increased by a mean +/- SD of 14.9 +/- 9.8% (P < 0.002 versus baseline) in the treated patients (reaching the normal range in 13 patients), while the BMD was 2.6 +/- 5% (not significant versus baseline) in the control group (15 had a decrease). Most interestingly, there was a large increase in BMD (15.3 +/-9.9%) after alendronate therapy in the 16 children who had their BMD followed up in the year before the study, during which time they had shown little increase in BMD (1.03 +/- 6.3%), and often a decrease. Considering their condition, increases in the height of all patients was satisfactory. No new fractures were observed after alendronate therapy was initiated. CONCLUSION: Bisphosphonates can be considered essential components of the treatment of secondary osteoporosis, not only in adults, but also in pediatric patients. Alendronate has a positive effect on secondary osteopenia/osteoporosis in children with connective tissue diseases.

Lewiecki EM, Gordon CM, Baim S, Binkley N, Bilezikian JP, Kendler DL, Hans DB, Silverman S, Bishop NJ, Leonard MB, Bianchi ML, Kalkwarf HJ, Langman CB, Plotkin H, Rauch F, Zemel BS. · New Mexico Clinical Research & Osteoporosis Center, 300 Oak Street NE, Albuquerque, NM 87106, USA. · Osteoporos Int. · Pubmed #18633664 No free full text.

Abstract: The International Society for Clinical Densitometry (ISCD) conducts Position Development Conferences (PDCs) for the purpose of establishing standards and guidelines in the field of bone densitometry. Topics for consideration are selected according to clinical relevance, a perceived need for standardization, and the likelihood of achieving agreement. Questions regarding nomenclature, indications, acquisition, analysis, quality control, interpretation, and reporting of bone density tests for each topic area are assigned to task forces for a comprehensive review of the scientific literature. The findings of the review and recommendations are then presented to an international panel of experts at the PDC. The expert panel votes on potential Official Positions for appropriateness, necessity, quality of the evidence, strength of the recommendation, and applicability (worldwide or variable according to local requirements). Recommendations that are approved by the ISCD Board of Directors become Official Positions. The first Pediatric PDC was 20-21 June 2007 in Montreal, QC, Canada. The most recent Adult PDC was held 20-22 July 2007, in Lansdowne, VA, USA. This Special Report summarizes the methodology of the ISCD PDCs and presents selected Official Positions of general interest.

Baim S, Leonard MB, Bianchi ML, Hans DB, Kalkwarf HJ, Langman CB, Rauch F. · Medical College of Wisconsin, Milwaukee, WI, USA. <> · J Clin Densitom. · Pubmed #18442749 No free full text.

Abstract: The International Society for Clinical Densitometry (ISCD) convenes a Position Development Conference (PDC) every 2 yr to make recommendations for standards in the field of bone densitometry. The recommendations are based on clinically relevant issues in bone densitometry such as quality control, acquisition, analysis, interpretation, and reporting. In 2007, ISCD convened its first Pediatric Position Development Conference to address issues specific to the assessment of skeletal health in children and adolescents. Topics for consideration are developed by the ISCD Board of Directors and its Scientific Advisory Committee. Clinically relevant questions related to each topic area are assigned to task forces for a comprehensive review of the medical literature and subsequent presentation of the reports to an international panel of experts. For this PDC, the Expert Panel included representatives of the American Society for Bone and Mineral Research and International Bone and Mineral Society. The recommendations of the PDC Expert Panel are then reviewed by the ISCD Board of Directors. Recommendations that are approved become Official Positions of the ISCD. The Pediatric PDC was held June 20-21, 2007, in Montreal, Quebec, Canada. Topics considered were restricted to children and adolescents, and included DXA prediction of fracture and definition of osteoporosis; DXA assessment in diseases that may affect the skeleton; DXA interpretation and reporting; and peripheral quantitative computed tomography measurement. This report describes the methodology and results of the 2007 Pediatric PDC, and a summary of all ISCD Official Positions, including the ones recently adopted by this 2007 Pediatric PDC and the 2007 Lansdowne, Virginia, USA Adult PDC.

Bianchi ML, Bardella MT. · Bone Metabolism Unit, Istituto Auxologico Italiano IRCCS, Milan, Italy. · Osteoporos Int. · Pubmed #18418638 No free full text.

Abstract: Chronic inflammation and malabsorption in celiac disease (CD) can cause bone metabolism alterations and bone mineral loss in children and adults. Bone status before and after gluten-free diet, epidemiology of fractures, and possible treatment options for CD-related osteoporosis are presented. Controversial aspects of this complication of CD are discussed. The relationship between bone derangements and celiac disease (CD) was recognized almost 50 years ago, but many questions are still open. We are now aware that osteoporosis is a relatively frequent atypical presentation of CD, especially in adults, and that undiagnosed CD can be the cause of osteoporosis and related fractures. Chronic inflammatory intestinal diseases, including CD, can affect bone and mineral metabolism because of alterations in both systemic and local regulatory factors. The pathogenetic processes are still controversial, but two main mechanisms seem to be involved: intestinal malabsorption and the presence of chronic inflammation. This review analyzes the published data on bone involvement in children, adolescents, and adults either before or after a gluten-free diet. Special attention is paid to the epidemiology of fractures in celiac patients, considering that fractures are a major complication of osteoporosis and an important problem in the management of a chronic disease like CD. The usefulness of screening osteoporotic patients systematically for CD is still an open question, but some rules can be given. Finally, the current treatment options for children and adults are discussed. Recommendations for future clinical research are proposed.

Bianchi ML, Orsini MR, Saraifoger S, Ortolani S, Radaelli G, Betti S. · Bone Metabolic Unit, Istituto Auxologico Italiano, IRCSS, Milan, Italy. · Health Qual Life Outcomes. · Pubmed #16321148 links to  free full text

Abstract: BACKGROUND: To evaluate the impact of osteoporosis on the patients' quality of life, particularly in the absence of fractures. METHODS: 100 post-menopausal women (age 50-85)--62 with uncomplicated primary osteoporosis and 38 with primary osteoporosis complicated by vertebral fractures; all already treated--were studied using two validated questionnaires: Qualeffo-41 for quality of life in osteoporosis, and Zung for depression. Data were compared to those of 35 controls of comparable age, affected by a different chronic disease (hypothyroidism). RESULTS: Family history of osteoporosis and T-score of spine were similar in the two subgroups of osteoporotic women. Body mass index, age at menopause and education level were similar in the two subgroups of osteoporotic women and in the control group. The patients affected by osteoporosis perceived it as a disease affecting their personal life with undesirable consequences: chronic pain (66% of women with fractures and 40% of women without fractures), impaired physical ability, reduced social activity, poor well-being (21% of women without fractures) and depressed mood (42% of women irrespective of fractures). Overall, 41% of the women showed a reduced quality of life. On the contrary, in the control group only 11% reported a reduced quality of life. CONCLUSION: The quality of life of osteoporotic patients should be investigated even before fractures, in order to develop appropriate counselling, support and care interventions to help patients develop efficient strategies for accepting the disease and coping with it.

Bardella MT, Bianchi ML, Teti A. · No affiliation provided · Gut. · Pubmed #16162965 links to  free full text

This publication has no abstract.

Bianchi ML, Mazzanti A, Galbiati E, Saraifoger S, Dubini A, Cornelio F, Morandi L. · Bone Metabolic Unit, Istituto Auxologico Italiano IRCCS, via L. Ariosto 13, 20145 Milano, Italy. · Osteoporos Int. · Pubmed #12897980 No free full text.

Abstract: Very few studies on bone mineral density and bone metabolism in Duchenne muscular dystrophy (DMD) have been reported. DMD is a severe, progressive muscular disease resulting in death at a young age. No specific therapies are available, but corticosteroids induce improvement and slower progression of the disease. However, long-term steroid therapy is a serious risk factor for osteoporosis. This study was aimed at evaluating bone mineral density and calciotropic hormones in a group of children affected by DMD, with or without steroid therapy. Bone mineral density was measured by DXA scan on lumbar spine and total body. Evaluation of calcium, phosphorus, bone turnover markers and calciotropic hormones was performed. Thirty-two children affected by DMD were studied: twenty-two on long-term prednisone therapy, ten not taking corticosteroids. Bone mineral density was lower than normal for age in all patients, and even lower in the group of steroid-treated children. Trunk and lower limb bone mineral densities were more reduced than upper limb mineral density, especially in the steroid-treated subjects. A marked reduction in spine bone mineral density, hypocalciuria, low 25-hydroxyvitamin D levels, and increased bone turnover markers were observed, and even these especially in the steroid-treated group. In conclusion, decreased bone mineral density and derangement of calcium metabolism were present in DMD patients, and were worsening during corticosteroid therapy. It is thus recommended that bone and mineral metabolism be carefully evaluated in patients with DMD, so that appropriate measures could be taken, especially now that chronic corticosteroid therapy is frequently given.

Lewiecki EM, Gordon CM, Baim S, Leonard MB, Bishop NJ, Bianchi ML, Kalkwarf HJ, Langman CB, Plotkin H, Rauch F, Zemel BS, Binkley N, Bilezikian JP, Kendler DL, Hans DB, Silverman S. · New Mexico Clinical Research &Osteoporosis Center, 300 Oak Street NE, Albuquerque, NM 87106, USA. · Bone. · Pubmed #18793764 No free full text.

Abstract: The International Society for Clinical Densitometry (ISCD) periodically convenes Position Development Conferences (PDCs) in order to establish standards and guidelines for the assessment of skeletal health. The most recent Adult PDC was held July 20-22, 2007, in Lansdowne, Virginia, USA; the first Pediatric PDC was June 20-21, 2007 in Montreal, Quebec, Canada. PDC topics were selected according to clinical relevancy, perceived need for standardization, and likelihood of achieving agreement. Each topic area was assigned to a task force for a comprehensive review of the scientific literature. The findings of the review and recommendations were presented to adult and pediatric international panels of experts. The panels voted on the appropriateness, necessity, quality of the evidence, strength, and applicability (worldwide or variable according to local requirements) of each recommendation. Those recommendations that were approved by the ISCD Board of Directors become Official Positions. This is a review of the methodology of the PDCs and selected ISCD Official Positions.