Intravenous bisphosphonate therapy in children with spinal muscular atrophy

ConclusionThese findings suggest that in children with SMA, IV BP therapy appears to be safe with minimal acute side effects and effective to reduce fracture rate. Caution is still needed given the occurrence of an atypical femur fracture in SMA population.
Source: Osteoporosis International - Category: Orthopaedics Source Type: research

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CONCLUSIONS: Findings suggest that bone-targeted exercise interventions for young adult women must address perceived time demands and environmental barriers to participation in order to maximise compliance and adherence. SO WHAT?: As physical activity is the most effective lifestyle strategy to improve bone health and young adulthood an important window for its augmentation, increasing convenience, accessibility and understanding of osteoporosis preventative behaviours in this demographic is vital. PMID: 31943497 [PubMed - as supplied by publisher]
Source: Health Promotion Journal of Australia - Category: International Medicine & Public Health Tags: Health Promot J Austr Source Type: research
AbstractPurpose of ReviewTo provide an overview on recent technical development for quantifying marrow composition using magnetic resonance imaging (MRI) and spectroscopy (MRS) techniques, as well as a summary on recent findings of interrelationship between marrow adipose tissue (MAT) and skeletal health in the context of osteoporosis.Recent FindingsThere have been significant technical advances in reliable quantification of marrow composition using MR techniques. Cross-sectional studies have demonstrated a negative correlation between MAT and bone, with trabecular bone associating more strongly with MAT than cortical bone...
Source: Current Osteoporosis Reports - Category: Orthopaedics Source Type: research
ConclusionsHU measurements in CT scans have proven to be a feasible tool to additionally assess bone quality at the lumbar and sacral spine with good sensitivity, when compared with the gold standard DXA.Level of evidenceIII.Graphic abstractThese slides can be retrieved under Electronic Supplementary Material.
Source: European Spine Journal - Category: Orthopaedics Source Type: research
In this study, we considered whether perceived sleep quality is also associated with bone health in older adults. We explored this association in a cohort of 443 older community-dwelling UK adults. Sleep quality was assessed using the Pittsburgh Sleep Quality Index (PSQI); poor sleep quality was defined as  >  5 on this score system. Bone density, shape and microarchitecture were assessed using dual energy X-ray absorptiometry (DXA), peripheral quantitative computed tomography (pQCT) and high-resolution pQCT (HRpQCT). Thirty-seven percent of men and 43% of women had a PSQI score greater than 5, indicat ive...
Source: Calcified Tissue International - Category: Orthopaedics Source Type: research
Authors: Kobayash K, Ando K, Machino M, Morozumi M, Kanbara S, Ito S, Inoue T, Yamaguchi H, Ishiguro N, Imagama S Abstract Study Design: Retrospective study in a single center. Purpose: To examine denosumab persistence in patients of different ages with severe osteoporosis in Japan. Overview of Literature: Denosumab is an antibody drug used for the treatment of osteoporosis. It is mainly used in patients with severe osteoporosis who might have high motivation for treatment, and the need for only semi-annual subcutaneous injection might improve the continuation rate. However, no English-language articles hav...
Source: Asian Spine Journal - Category: Orthopaedics Tags: Asian Spine J Source Type: research
Authors: Pavelka T, Salášek M, Bárta P, Fridrich F, Džupa V Abstract PURPOSE OF THE STUDY Non-operative and operative treatment of acetabular fractures is associated with a risk of development of posttraumatic avascular necrosis of femoral head or with the development of posttraumatic coxarthrosis. The purpose of the study was to identify the occurrence of these two complications in patients in our group and to determine the risk factors for the development of these complications. MATERIAL AND METHODS The retrospective study was conducted in two Level I trauma centres in the period from 2009 t...
Source: Acta Chirurgiae Orthopaedicae et Traumatologiae Cechoslovaca - Category: Orthopaedics Tags: Acta Chir Orthop Traumatol Cech Source Type: research
This study aimed to measure levels of vitamin D in SMA-patients and investigate their association with SMA type, fractures, and supplementation with vitamin D. We conducted a descriptive observational study of the natural history of SMA in Chile between September 2017 and March 2018.
Source: Neuromuscular Disorders - Category: Neurology Authors: Source Type: research
Spinal muscular atrophy (SMA) is a hereditary neuromuscular disease marked by the progressive weakness and muscle loss, with obesity as well as malnutrition, gastrointestinal dysmotility, and osteoporosis. Many patients exhibit metabolic abnormalities, such as insulin resistance, diabetes and fatty acid oxidation disorder. This observational study consisted of a nutritional and medical history survey of children with SMA type II and III collected between 2008-2015. We evaluated 109 patients, 55% of them were males, which 78% SMAII and 22% SMAIII.
Source: Neuromuscular Disorders - Category: Neurology Authors: Source Type: research
Spine-based fixation of magnetically controlled lengthening devices has been successfully performed for children with early-onset scoliosis. However, spinal manipulation may lead to ossifications, stiffness, and autofusion as previously described. To avoid these problems, a surgical technique combining bilateral externally controlled magnetic device implantation with a rib cradle and pelvic hook fixation was introduced by us in 2011. By using a bilateral single-rib or double-rib cradle fixation and a pelvic hook, the magnetic device is percutaneously inserted. The spine corrects indirectly without further manipulation. In ...
Source: Journal of Spinal Disorders and Techniques - Category: Surgery Tags: Surgical Technique Source Type: research
AbstractPurposeHere, we review the skeletal effects of pediatric muscle disorders as well as muscle impairment in pediatric bone disorders.Recent FindingsWhen starting in utero, muscle disorders can lead to congenital multiple contractures. Pediatric-onset muscle weakness such as cerebral palsy, Duchenne muscular dystrophy, spinal muscular atrophy, or spina bifida typically are associated with small diameter of long-bone shafts, low density of metaphyseal bone, and increased fracture incidence in the lower extremities, in particular, the distal femur. Primary bone diseases can affect muscles through generic mechanisms, suc...
Source: Current Osteoporosis Reports - Category: Orthopaedics Source Type: research
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