Journal of Pediatric Rehabilitation Medicine - Volume 7, issue 2

Article Type: Review Article

DOI: 10.3233/PRM-140279

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 7, no. 2, pp. 107-109, 2014

Authors: Kecskemethy, Heidi H. | Harcke, H. Theodore

Article Type: Research Article

Abstract: Evaluating the bone health of children with disabilities is challenging and requires consideration of many factors in clinical decision-making. Feeding problems and growth deficits, immobility/inability to bear weight, effect of medications, and the nature of his or her disease can all directly affect a child's overall picture of bone health. Familiarity with the tools available to assess bone health is important for practitioners. The most commonly used method to assess bone density, dual energy x-ray …absorptiometry, can be performed effectively when one appreciates the techniques that make scanning patients with disabilities possible. There are specific techniques that are especially useful for measuring bone density in children with disabilities; standard body sites are not always obtainable. Consideration of clinical condition and treatment must be considered when interpreting dual energy x-ray absorptiometry scans. Serial measurements have been shown to be effective in monitoring change in bone content and in providing information on which to base decisions regarding medical treatment. Show more

Keywords: Bone mineral density, disability, DXA, lateral distal femur

DOI: 10.3233/PRM-140280

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 7, no. 2, pp. 111-124, 2014

Authors: Szalay, Elizabeth A.

Article Type: Research Article

Abstract: Bisphosphonates (BPs) are used most commonly in children with osteogenesis imperfecta, resulting in increased trabeculae and cortical thickness, increased bone density as measured by DXA (Dual Energy X-ray Absorptiometry), and improved vertebral morphology. Less well documented in controlled trials are decrease in long bone fractures, improved strength and motor function, and decreased pain [1]. Outside of children with osteogenesis imperfecta, use of bisphosphonates in children is increasing, all of which is off-label. This is …seen in children with other chronic conditions resulting in pediatric osteoporosis and insufficiency fractures. Additional indications include steroid dependency with progressive loss of bone density, avascular necrosis of bone, and chronic regional pain syndrome. This review highlights the potential benefits and risks of the use of bisphosphonates in these unique children at risk for fracture or bone collapse. Show more

Keywords: DXA, bisphosphonates, pediatric osteoporosis

DOI: 10.3233/PRM-140281

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 7, no. 2, pp. 125-132, 2014

Authors: Ness, Kathryn | Apkon, Susan D.

Article Type: Research Article

Abstract: This paper provides a broad review of bone health issues in children with neuromuscular diseases (NMD). As Duchenne muscular dystrophy is the most common neuromuscular disease of childhood, there is a strong emphasis on this. This paper reviews the incidence of fractures, the degree of the reduced bone mineral density, the risk factors associated with this common problem, and the non-pharmacological and pharmacological treatment options. Recommendations for monitoring and treatment are provided.

Keywords: Neuromuscular, fracture, Duchenne, spinal muscular atrophy

DOI: 10.3233/PRM-140282

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 7, no. 2, pp. 133-142, 2014

Authors: Houlihan, Christine Murray

Article Type: Research Article

Abstract: CP is the most prevalent childhood condition associated with low bone mass. Bone density is decreased in children with CP who sustain fragility fractures that impair function and quality of life. Predicting accurately who is at risk for fracture, preventing or reversing low bone mass and maximizing bone accrual during critical stages of growth are essential to minimizing future lifelong risks of fractures. This review article addresses the diagnosis of low bone mass, the anatomy of …bone, risk factors for low bone density and for the prevention and treatment for low bone mass for children with CP. Show more

Keywords: Osteoporosis, bone density, bone health, cerebral palsy, disabilities

DOI: 10.3233/PRM-140283

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 7, no. 2, pp. 143-153, 2014

Authors: Olson, Jennifer Ann

Article Type: Research Article

Abstract: Bisphosphonates are being used more frequently as part of the multi-disciplinary management of moderate to severe Osteogenesis Imperfecta (OI). This report details the development of respiratory failure during the second infusion of pamidronate in a 3.5 year-old male with osteogenesis imperfecta type 1 and no prior history of respiratory disease.

Keywords: Osteogenesis imperfecta, bisphosphonates, pamidronate, respiratory distress, respiratory failure

DOI: 10.3233/PRM-140284

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 7, no. 2, pp. 155-158, 2014

Authors: Stevenson, David A. | Rudser, Kyle | Kunin-Batson, Alicia | Fung, Ellen B. | Viskochil, David | Shapiro, Elsa | Orchard, Paul J. | Whitley, Chester B. | Polgreen, Lynda E.

Article Type: Research Article

Abstract: PURPOSE: Skeletal disease causes significant morbidity in mucopolysaccharidoses (MPS), and bone remodeling processes in MPS have not been well characterized. The objective of this study was to determine if biomarkers of bone turnover are abnormal in children with specific MPS disorders (i.e. MSP-I, MPS-II, and MPS-VI) compared to healthy children. METHODS: A cross-sectional study was performed of serum biomarkers of bone formation (bone-specific alkaline phosphatase [BSAP], osteocalcin) and urine biomarkers of bone resorption (pyridinoline, …deoxypyridinoline) in MPS and healthy controls. Measures of physical function and pain were obtained using the Children's Health Questionnaire (CHQ). RESULTS: The cohort consisted of 39 children with MPS (MPS-I=26; MPS-II=11; MPS-VI=4) and 51 healthy children. Adjusting for sex and Tanner stage group, MPS individuals had statistically significant increases for osteocalcin (p< 0.001), with trends toward higher BSAP (p=0.054) and urinary pyridinoline (p=0.084). These biomarkers were not significantly associated with CHQ bodily pain and physical-function scores. CONCLUSION: Osteocalcin was increased in children with MPS disorders, with trends for increases in BSAP and urinary pyridinoline, suggesting that bone remodeling is altered in children with MPS. Future studies to assess the ability of these biomarkers to quantify and monitor MPS skeletal disease in response to therapy are needed. Show more

Keywords: Bone, lysosomal storage diseases, mucopolysaccharidosis, dysostosis multiplex

DOI: 10.3233/PRM-140285

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 7, no. 2, pp. 159-165, 2014

Authors: Afzal, Sarah Y. | Wender, Anna R. | Jones, Mary D. | Fung, Ellen B. | Pico, Elaine L.

Article Type: Research Article

Abstract: PURPOSE: Low magnitude mechanical stimulation (LMMS) has been used successfully to promote bone formation in certain patient populations. This study evaluated the feasibility and effectiveness of LMMS on improving bone mineral density (BMD) in patients with Rett syndrome. METHODS: A 12-month crossover pilot study design of 6 months of intervention with LMMS and 6 months without was studied in 14 subjects divided in two subgroups. BMD was assessed using Dual Energy X-ray Absorptiometry (DXA). …The levels of 25-hydroxy vitamin D (25OHD), Parathyroid Hormone (PTH), Insulin-Like Growth Factor 1 (IGF-1), and circulating markers of bone resorption (NTx) were analyzed in blood samples. Health questionnaires and diet logs were obtained at 0, 6, and 12 months. RESULTS: Of the 11 subjects who completed the protocol, 9 had an adherence of > 65% and showed an increase in spine BMD Z-scores from the intervention (Z: −2.51) compared to non-intervention period (Z: −2.27) of 0.23 SD (p=0.048). Following intervention, favorable trends were also observed for IGF-1 (p=0.06) and right distal femur BMD Z-scores (p=0.07). CONCLUSIONS: These preliminary results are promising for a larger, placebo-controlled randomized study of subjects with Rett syndrome. Show more

Keywords: Rett syndrome, MECP2, bone mineral density, low magnitude mechanical stimulation (LMMS), Z-scores, DXA, fracture, osteopenia

DOI: 10.3233/PRM-140286

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 7, no. 2, pp. 167-178, 2014

Authors: Palermo, Nadine E. | Holick, Michael F.

Article Type: Research Article

Abstract: Some of the clinically significant causes of vitamin D deficiency in children are secondary to inadequate dietary intake, limited sun exposure, disease states causing malabsorption (celiac disease, gastric and small bowel resection, pancreatic insufficiency from cystic fibrosis) and altered metabolism secondary to medications. In utero and during childhood vitamin D deficiency can have a myriad of health consequences extending beyond musculoskeletal health to acute and chronic disease. In this review, it discusses …the prevalence of vitamin D deficiency, clinical spectrum of disease presentations in children with emphasis in children with disabilities, vitamin D's role in bone and nonskeletal heath as well as recommendations for prevention and treatment of vitamin D deficiency. Show more

Keywords: Vitamin D, 25-hydroxyvitamin D, rickets, children, disability, malabsorption syndromes

DOI: 10.3233/PRM-140287

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 7, no. 2, pp. 179-192, 2014

Article Type: Other

DOI: 10.3233/PRM-140288

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 7, no. 2, pp. 193-194, 2014