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Pediatric osteoporosis

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https://www.readbyqxmd.com/read/29108345/mri-based-evaluation-of-multiorgan-iron-overload-is-a-predictor-of-adverse-outcomes-in-pediatric-patients-undergoing-allogeneic-hematopoietic-stem-cell-transplantation
#1
Natalia Maximova, Massimo Gregori, Giulia Boz, Roberto Simeone, Davide Zanon, Giulia Schillani, Floriana Zennaro
The medical records of 44 pediatric patients who underwent allogeneic transplantation from 2011 to 2015 were retrospectively reviewed. Magnetic resonance imaging was used to measure iron concentrations in the liver, spleen, pancreas and bone. These patients were divided into two groups, 18 with non-elevated (< 100 μmol/g; Group 1) liver iron concentration before transplantation and 26 with elevated (> 100 μmol/g; Group 2) concentration . We compared transplant-related outcomes in the two groups. Iron overload was a negative prognostic risk factor for sinusoidal obstruction syndrome (OR = 17), osteoporosis (OR = 6...
October 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/29058268/early-diagnosed-cerebrotendinous-xanthomatosis-patients-clinical-neuroradiological-characteristics-and-therapy-results-of-a-single-center-from-turkey
#2
Tanyel Zubarioglu, Ertugrul Kiykim, Gozde Yesil, Duhan Demircioglu, Mehmet Serif Cansever, Cengiz Yalcinkaya, Cigdem Aktuglu-Zeybek
Cerebrotendinous xanthomatosis (CTX) is a lipid storage disorder caused by defective sterol 27-hydroxylase activity. In spite of subtle clinical signs beginning from childhood, CTX is generally diagnosed lately. The aim of this study is to evaluate clinical, neuroradiological findings and therapy responses of pediatric CTX patients and raise awareness to early features of disease. Patients who were molecularly diagnosed as CTX before 18 years of age were included in study. Clinical, epidemiological, radiological and genotypic features of patients and chenodeoxycholic acid (CDCA) therapy responses were reviewed retrospectively...
October 22, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/28977339/glucocorticoid-induced-bone-disease-mechanisms-and-importance-in-pediatric-practice
#3
Emanuel Sávio Cavalcanti Sarinho, Verônica Maria Pinho Pessoa Melo
OBJECTIVE: To describe mechanisms by which glucocorticoids cause osteoporosis, with fracture risk, combining this learning with a possible professional behavior change. DATA SOURCES: A systematic search on SciELO, PubMed, Scopus, and Medline databases was carried out for consensus, review articles, including systematic reviews and meta-analysis, which were published in English, between 2000 and 2016. Keywords used on the search were the following: glucocorticoids, fractures, osteoporosis, bone health, vitamin D, children, and adolescents...
April 2017: Revista Paulista de Pediatria: Orgão Oficial da Sociedade de Pediatria de São Paulo
https://www.readbyqxmd.com/read/28965204/dental-manifestations-of-pediatric-bone-disorders
#4
REVIEW
Juan F Yepes
PURPOSE OF REVIEW: Several bone disorders affecting the skeleton often are manifest in the maxillofacial region. This review presents the most common bone disorders in children and their dental-oral manifestations: fibrous dysplasia, Paget's disease, osteogenesis imperfecta, renal osteodystrophy, hypophosphatasia, and osteoporosis. The specific intraoral characteristics will reviewed in detail. RECENT FINDINGS: Recent studies confirmed the close relationship between the mandible and the maxilla with the most prevalent systemic bone disorders in children...
September 30, 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28894358/therapy-with-pamidronate-in-children-with-osteogenesis-imperfecta
#5
Otilia Marginean, Raluca Corina Tamasanu, Niculina Mang, Ioana Mozos, Giorgiana Flavia Brad
Osteogenesis imperfecta (OI) is a genetic disease characterized by excessive bone fragility with fractures consecutive to minor trauma. Considering lack of standardization of therapy with pamidronate in children, it was our aim to present our experience over a period of 10 years regarding evolution and treatment in patients diagnosed with osteoporosis and OI. Nine patients diagnosed with OI were admitted to the First Pediatric Clinic, Timisoara. They were investigated (clinical, biomarkers of bone metabolism and imaging studies), and a quality-of-life questionnaire was used to evaluate the impact of OI...
2017: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/28830650/basics-of-bone-metabolism-and-osteoporosis-in-common-pediatric-neuromuscular-disabilities
#6
REVIEW
Evren Yaşar, Emre Adigüzel, Mutluay Arslan, Dennis J Matthews
Bone modeling is a process that starts with fetal life and continues during adolescence. Complex factors such as hormones, nutritional and environmental factors affect this process. In addition to these factors, physical conditioning and medications that have toxic effects on bony tissue should be carefully considered in patient follow-up. Osteoporosis is a significant problem in pediatric population because of ongoing growth and development of skeletal system. Two types of osteoporosis are primary and secondary types and children with neuromuscular disabilities constitute a major group with secondary osteoporosis...
August 10, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28708597/mri-based-evaluation-of-multiorgan-iron-overload-is-a-predictor-of-adverse-outcomes-in-pediatric-patients-undergoing-allogeneic-hematopoietic-stem-cell-transplantation
#7
Natalia Maximova, Massimo Gregori, Giulia Boz, Roberto Simeone, Davide Zanon, Giulia Schillani, Floriana Zennaro
The medical records of 44 pediatric patients who underwent allogeneic transplantation from 2011 to 2015 were retrospectively reviewed. Magnetic resonance imaging was used to measure iron concentrations in the liver, spleen, pancreas and bone. These patients were divided into two groups, 18 with non-elevated (< 100 μmol/g; Group 1) liver iron concentration before transplantation and 26 with elevated (> 100 μmol/g; Group 2) concentration . We compared transplant-related outcomes in the two groups. Iron overload was a negative prognostic risk factor for sinusoidal obstruction syndrome (OR = 17), osteoporosis (OR = 6...
July 5, 2017: Oncotarget
https://www.readbyqxmd.com/read/28644353/extraintestinal-manifestations-of-celiac-disease-effectiveness-of-the-gluten-free-diet
#8
Hilary Jericho, Naire Sansotta, Stefano Guandalini
OBJECTIVE: The aim of the study was to evaluate the effectiveness of the gluten-free diet (GFD) on extraintestinal symptoms in pediatric and adult celiac populations at the University of Chicago. METHODS: We conducted a retrospective chart review of the University of Chicago Celiac Center clinic charts from January 2002 to October 2014. Demographics, serologic testing, intestinal biopsies, and extraintestinal symptoms at presentation, 12, 24, and >24 months were recorded...
July 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28643220/denosumab-an-emerging-therapy-in-pediatric-bone-disorders
#9
REVIEW
Alison M Boyce
PURPOSE OF REVIEW: Denosumab is an inhibitor of receptor activator of nuclear factor kappa-B ligand (RANKL), and has emerged as an important novel therapy for skeletal disorders. This article examines the use of denosumab in children. RECENT FINDINGS: Considerable safety and efficacy data exists for denosumab treatment of adults with osteoporosis, bone metastases, and giant cell tumors. Pediatric data is limited; however, evidence suggests denosumab may be beneficial in decreasing bone turnover, increasing bone density, and preventing growth of certain skeletal neoplasms in children...
August 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28620868/osteoporosis-in-children-with-chronic-illnesses-diagnosis-monitoring-and-treatment
#10
REVIEW
Monica Grover, Laura K Bachrach
PURPOSE OF REVIEW: Osteoporosis is an under-recognized complication of chronic illness in childhood. This review will summarize recent literature addressing the risk factors, evaluation, and treatment for early bone fragility. RECENT FINDINGS: Criteria for the diagnosis of pediatric osteoporosis include the presence of low trauma vertebral fractures alone or the combination of low bone mineral density and several long bone fractures. Monitoring for bone health may include screening for vertebral fractures that are common but often asymptomatic...
August 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28582319/osteoporosis-in-childhood
#11
Francesco Vierucci, Giuseppe Saggese, Rolando Cimaz
PURPOSE OF REVIEW: The aim of this review is to highlight recent findings in prevention, diagnosis, and treatment of pediatric osteoporosis. RECENT FINDINGS: Several genes are involved in bone mass acquisition, and various monogenic bone disorders characterized by reduced bone mineral density and increased bone fragility have been recently described. Moreover, many chronic diseases and/or their treatment have been associated with impaired bone mass acquisition. Pediatric osteoporosis should be adequately suspected and properly diagnosed in children at risk of fractures...
September 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28463945/epidemiology-of-fractures-in-children-younger-than-12-months
#12
Diana Rodà, Victoria Trenchs, Ana Isabel Curcoy, Anibal Dolores Martínez, Jordi Pou, Carles Luaces
OBJECTIVES: The aim of this study is to determine the prevalence and characteristics of fractures in young infants attended at the pediatric emergency department (PED). METHODS: This is a retrospective study for 2 years (2011-2012) of children younger than 12 months attended with a fracture at the PED. Age, sex, site and type of fracture, mechanism of injury, time interval before seeking medical attention, and management were analyzed. RESULTS: One hundred one patients were included...
May 1, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28290112/protecting-bone-health-in-pediatric-rheumatic-diseases-pharmacological-considerations
#13
REVIEW
Yujuan Zhang, Diana Milojevic
Bone health in children with rheumatic conditions may be compromised due to several factors related to the inflammatory disease state, delayed puberty, altered life style, including decreased physical activities, sun avoidance, suboptimal calcium and vitamin D intake, and medical treatments, mainly glucocorticoids and possibly some disease-modifying anti-rheumatic drugs. Low bone density or even fragility fractures could be asymptomatic; therefore, children with diseases of high inflammatory load, such as systemic onset juvenile idiopathic arthritis, juvenile dermatomyositis, systemic lupus erythematosus, and those requiring chronic glucocorticoids may benefit from routine screening of bone health...
June 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28289431/management-issues-of-congenital-adrenal-hyperplasia-during-the-transition-from-pediatric-to-adult-care
#14
REVIEW
Jin-Ho Choi, Han-Wook Yoo
Steroid 21-hydroxylase deficiency is the most prevalent form of congenital adrenal hyperplasia (CAH), accounting for approximately 95% of cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, tumor, osteoporosis, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and health-care providers...
February 2017: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28277416/incidence-and-risk-factors-of-hardware-related-complications-after-proximal-femoral-osteotomy-in-children-and-adolescents
#15
Myung Ki Chung, Soon-Sun Kwon, Byung Chae Cho, Gye Wang Lee, Jaeyoung Kim, Seung Jun Moon, Jae Woo Lee, Chin Youb Chung, Ki Hyuk Sung, Kyoung Min Lee, Moon Seok Park
Proximal femoral osteotomy has been used in cerebral palsy, Perthes disease, hip dysplasia, idiopathic femoral anteversion, and various hip diseases in children and adolescents. Conventionally, a blade plate (BP) has been used. However, the pediatric locking compression plate (LCP) has recently been applied widely. We compared the hardware-related complications of the BP and the LCP as well as the factors influencing these complications in patients who have undergone a proximal femoral osteotomy in children and adolescents...
March 8, 2017: Journal of Pediatric Orthopedics. Part B
https://www.readbyqxmd.com/read/28275812/urinary-stone-disease-in-pediatric-and-adult-metabolic-bone-clinic-patients
#16
Andrew L Schwaderer, Abimbola Oduguwa, Kirsten Kusumi
Kidney stones are increasing in the pediatric and adult populations; similarly osteoporosis is increasingly recognized in children. While kidney stone formers are known to suffer from low bone density, metabolic bone patients have not been considered a high risk population for kidney stones. Retrospective chart review of Nationwide Children's Hospital Metabolic Bone Clinic patients from October 2009-2013. Patients were identified by ICD 9 codes for osteoporosis, osteopenia, low bone density and kidney stones...
March 8, 2017: Urolithiasis
https://www.readbyqxmd.com/read/28271302/pediatric-osteoporosis-diagnosis-and-treatment-considerations
#17
REVIEW
Edoardo Marrani, Teresa Giani, Gabriele Simonini, Rolando Cimaz
Osteoporosis is now increasingly recognized in children due to the increased prevalence of disorders associated with bone loss. Fragility fractures represent the cardinal clinical features of pediatric osteoporosis and children presenting with fragility fractures deserve an accurate assessment to rule out a secondary cause. Indeed, in the pediatric population, a low bone mass is often a consequence of a chronic disease or its treatment; genetic bone disorders represent the cause of only a small fraction of cases...
April 2017: Drugs
https://www.readbyqxmd.com/read/28258940/low-bone-mineral-density-and-fractures-are-highly-prevalent-in-pediatric-patients-with-spinal-muscular-atrophy-regardless-of-disease-severity
#18
Halley M Wasserman, Lindsey N Hornung, Peggy J Stenger, Meilan M Rutter, Brenda L Wong, Irina Rybalsky, Jane C Khoury, Heidi J Kalkwarf
Patients with Spinal Muscular Atrophy (SMA) are at risk for poor bone health. The prevalence of fractures, low areal bone mineral density (aBMD; Z-score ≤-2.0) of the lateral distal femur and of osteoporosis by SMA subtype is not known. We aimed to describe the natural history of bone health in patients with SMA prior to bisphosphonate treatment. We reviewed data from 85 eligible patients with SMA ages 12 months to 18 years, seen at a single institution between January 2005 and July 2016. Fracture history was reported at annual clinic visits...
February 1, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28235964/how-does-a-physical-activity-programme-in-elementary-school-affect-fracture-risk-a-prospective-controlled-intervention-study-in-malmo-sweden
#19
Marcus E Cöster, Jesper Fritz, Jan-Åke Nilsson, Caroline Karlsson, Björn E Rosengren, Magnus Dencker, Magnus K Karlsson
OBJECTIVES: Recent evidence from the 7-year follow-up of the Pediatric Osteoporosis Prevention (POP) study indicates an inverse correlation between years of participation in a physical activity (PA) intervention and fracture risk in children. However, we could not see a statistically significant reduction in fracture risk, which urged for an extension of the intervention. SETTING: The study was conducted in 4 neighbouring elementary schools, where 1 school functioned as intervention school...
February 23, 2017: BMJ Open
https://www.readbyqxmd.com/read/28214296/abnormally-high-and-heterogeneous-bone-matrix-mineralization-after-childhood-solid-organ-transplantation-a-complex-pathology-of-low-bone-turnover-and-local-defects-in-mineralization
#20
Nadja Fratzl-Zelman, Helena Valta, Renata C Pereira, Barbara M Misof, Paul Roschger, Hannu Jalanko, Katherine Wesseling-Perry, Klaus Klaushofer, Outi Mäkitie
Chronic renal, liver, and heart failure in children associates with multiple skeletal complications. Increased fracture incidence often persists after transplantation and could be related to alterations in bone material properties. In the present cohort study we evaluated bone mineralization density distribution (BMDD) by quantitative backscattered electron imaging (qBEI) in 23 pediatric solid organ allograft recipients with suspected osteoporosis. We measured BMDD in the entire cross-sectional area of transiliac bone biopsies obtained from kidney (n = 9), liver (n = 9), and heart (n = 5) transplant recipients (aged 7...
May 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
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