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Idiopathic juvenile osteoporosi

Manuela G M Rocha-Braz, Bruno Ferraz-de-Souza
The pathogenesis of osteoporosis, a common disease with great morbidity and mortality, comprises environmental and genetic factors. As with other complex disorders, the genetic basis of osteoporosis has been difficult to identify. Nevertheless, several approaches have been undertaken in the past decades in order to identify candidate genes for bone fragility, including the study of rare monogenic syndromes with striking bone phenotypes (e.g. osteogenesis imperfecta and osteopetroses), the analysis of individuals or families with extreme osteoporotic phenotypes (e...
August 2016: Archives of Endocrinology and Metabolism
Alison M Boyce, Michael T Collins, Laura L Tosi, Rachel I Gafni
Atypical subtrochanteric and diaphyseal femoral fractures (AFFs) have emerged as a potential complication of bisphosphonate treatment in adults. Despite increasing off-label use of bisphosphonates in children and adolescents for a variety of skeletal disorders, there have been no reports of AFFs in children or adolescents outside of the osteogenesis imperfecta population. We present the case of a 16-year-old girl who developed a subtrochanteric femoral stress fracture following pamidronate treatment for idiopathic juvenile osteoporosis...
July 6, 2016: Hormone Research in Pædiatrics
Ignacio Garcia, Vincent Chiodo, Yan Ma, Adele Boskey
PURPOSE: Idiopathic juvenile osteoporosis (IJO) is a rare condition in children, characterized by bone pain and long bone and vertebral fractures. Previously, IJO bone was solely characterized by histomorphometry and quantitative computed tomography. The goal of this study is to describe IJO bone composition. MATERIALS AND METHODS: Fourier transform infrared imaging (FTIRI), a vibrational spectroscopic technique providing spatially resolved images of chemical composition, was used to determine whether iliac crest biopsies from children with IJO differed in composition from and age- and sex-matched controls, and, as a secondary analysis, whether IJO bone showed the same disease dependent change in composition as do iliac crest bone biopsies from women with post-menopausal osteoporosis (PMO)...
2016: Connective Tissue Research
Halenur Altan, Gül Tosun, Yaşar Şen
Idiopathic Juvenile Osteoporosis (IJO) is a very rare disease, self restrictive and shows marked, spontaneous improvement during adolescence. The major clinical features were pain with difficulty walking, growth retardation, oral and dental abnormalities with radiographically porous bone structure. A 13-year-old male referred to paediatric dentistry clinic for toothache. The observations made with extra-intraoral clinic examination that one revealed short and skinny stature, diffuse caries in deciduous teeth, abraded lower incisor, deep bite and dysmorphic appearance in permanent incisor...
August 2015: Journal of Clinical and Diagnostic Research: JCDR
I Castrejon, L Carmona, N Agrinier, M Andres, K Briot, M Caron, R Christensen, A Consolaro, R Curbelo, Montserrat Ferrer, Violaine Foltz, C Gonzalez, F Guillemin, P M Machado, Birgit Prodinger, A Ravelli, M Scholte-Voshaar, T Uhlig, L H D van Tuyl, A Zink, L Gossec
OBJECTIVES: Patient reported outcomes (PROs) are relevant in rheumatology. Variable accessibility and validity of commonly used PROs are obstacles to homogeneity in evidence synthesis. The objective of this project was to provide a comprehensive library of "validated PROs". METHODS: A launch meeting with rheumatologists, PROs methodological experts, and patients, was held to define the library's aims and scope, and basic requirements. To feed the library we performed systematic reviews on selected diseases and domains...
November 2015: Clinical and Experimental Rheumatology
Roberto Franceschi, Monica Vincenzi, Marta Camilot, Franco Antoniazzi, Anthony J Freemont, Judith E Adams, Christine Laine, Outi Makitie, M Zulf Mughal
We report clinical findings, bone mineral density (BMD) and bone biopsy data in ten children with features of classic idiopathic juvenile osteoporosis (IJO). We also screened the patients for mutations in LRP5 and LRP6. We found low BMD in the lumbar spine, the hip and distal radius. In the spine and distal radius, the reduction in BMD was more marked in the trabecular compartment. Biopsy confirmed that the trabecular compartment is more severely involved with reduction in bone formation and increase in bone resorption...
June 2015: Calcified Tissue International
Ahmet Imerci, Umut Canbek, Sema Haghari, Levent Sürer, Muge Kocak
INTRODUCTION: Idiopathic Juvenile Osteoporosis is an uncommon condition that has few case reports in the literature. Reported series indicate that it is a condition classically accompanying vertebral and metaphyseal fractures during the immediate pre-puberty years but that seems to develop naturally during puberty. Current clinical treatment is complicated because of lack of understanding on the origins of Idiopathic Juvenile Osteoporosis. PRESENTATION OF CASE: The 13-year-old female patient with no former complaints had pain in her left hip while walking 2 years ago...
2015: International Journal of Surgery Case Reports
Olympia Papakonstantinou, Maria Sakalidou, Erato Atsali, Vasiliki Bizimi, Maria Mendrinou, Efthymia Alexopoulou
Bisphosphonates are employed with increasing frequency in various pediatric disorders, mainly associated with osteoporosis. After cessation of bisphosphonate treatment in children, skeletal radiologic changes have been documented including dense metaphyseal lines of the long bones and "bone in bone" appearance of the vertebrae. However, the evolution of these radiographic changes has not been fully explored. We describe the MR imaging appearance of the spine that, to our knowledge, has not been previously addressed in a child with idiopathic juvenile osteoporosis who had received bisphosphonates and emphasize the evolution of the radiographic findings of the spine and pelvis over a four-year period...
2015: Case Reports in Radiology
Dalal El Badri, Samira Rostom, Ilham Bouaddi, Asmae Hassani, Bouchra Chkirate, Bouchra Amine, Najia Hajjaj-Hassouni
INTRODUCTION: The link between bone mass and body composition is widely recognized, but only few works were selectively performed on subjects with juvenile idiopathic arthritis. The aim of our study was to investigate the effect of body composition on bone mineral density (BMD) in Moroccan patients with juvenile idiopathic arthritis. METHODS: Thirty three children with juvenile idiopathic arthritis (JIA) were included in a cross-sectional study. The diagnosis of JIA was made according to the criteria of the International League of Association of Rheumatology (ILAR)...
2014: Pan African Medical Journal
Julie Autmizguine, Michael Cohen-Wolkowiez, Norman Ilowite
No abstract text is available yet for this article.
January 2015: Journal of Clinical Pharmacology
N Maruotti, A Corrado, F P Cantatore
Numerous rheumatic diseases, including rheumatoid arthritis, juvenile idiopathic arthritis, psoriatic arthritis, ankylosing spondylitis, systemic lupus erythematosus, systemic sclerosis, dermatomyositis/polymyositis and vasculitis are characterized by osteoporosis and fragility fractures. Inflammatory cytokines, glucocorticoid treatment, immobilization and reduced physical activity due to painful joints and muscle weakness are considered the main risk factors that cause low body mass density values in these diseases...
2014: Reumatismo
Emily von Scheven, Kathleen Jo Corbin, Stefano Stagi, Stagi Stefano, Rolando Cimaz
Children with chronic illnesses such as Juvenile Idiopathic Arthritis and Crohn's disease, particularly when taking glucocorticoids, are at significant risk for bone fragility. Furthermore, when childhood illness interferes with achieving normal peak bone mass, life-long fracture risk is increased. Osteopenia and osteoporosis, which is increasingly recognized in pediatric chronic disease, likely results from numerous disease- and treatment-related factors, including glucocorticoid exposure. Diagnosing osteoporosis in childhood is complicated by the limitations of current noninvasive techniques such as DXA, which despite its limitations remains the gold standard...
September 2014: Current Osteoporosis Reports
Cheriya K Sreejan, Nair Gopakumar, Gogineni Subhas Babu
Chronic idiopathic hyperphosphatasia(CIH) or juvenile Paget disease is believed to be a distinct disease characterized by an increase in the serum alkaline phosphatase, cortical thickening and bowing of the long bones, especially the femora. It is a rare autosomal recessive bone disorder, with excessive bone resorption and bone formation. Skeletal malformations in the legs may cause problems in walking and may eventually result in short stature. The radiographic appearances include widening of the diaphyses, vertebral osteoporosis, acetabular protrusion, and thickening of the skull vault...
December 1, 2012: Journal of Clinical and Experimental Dentistry
Qiong Wu, Anne M Pesenacker, Alka Stansfield, Douglas King, Dawn Barge, Helen E Foster, Mario Abinun, Lucy R Wedderburn
Children with systemic Juvenile Idiopathic Arthritis (sJIA), the most severe subtype of JIA, are at risk from destructive polyarthritis and growth failure, and corticosteroids as part of conventional treatment can result in osteoporosis and growth delay. In children where there is failure or toxicity from drug therapies, disease has been successfully controlled by T-cell-depleted autologous stem cell transplantation (ASCT). At present, the immunological basis underlying remission after ASCT is unknown. Immune reconstitution of T cells, B cells, natural killer cells, natural killer T cells and monocytes, in parallel with T-cell receptor (TCR) diversity by analysis of the β variable region (TCRVb) complementarity determining region-3 (CDR3) using spectratyping and sequencing, were studied in five children with sJIA before and after ASCT...
June 2014: Immunology
Kwok-Kit Ngan, John Bowe, Nicolas Goodger
UNLABELLED: Bisphosphonate use has been described in children diagnosed with osteogenesis imperfecta (OI), fibrous dysplasia, neuromuscular disorders, bone dysplasia, idiopathic juvenile osteoporosis, rheumatologic disorder and even Crohn's disease. In OI patients, bisphosphonates have become an important symptomatic therapy for moderate and severe forms of the disease, because their inhibitory effect on osteoclasts increases bone mineralization and density, thereby reducing the risk of bone fractures...
November 2013: Dental Update
Giampiero I Baroncelli, Francesco Vierucci, Silvano Bertelloni, Paola Erba, Elisa Zampollo, Maria Rita Giuca
Although spontaneous remission occurs in patients with idiopathic juvenile osteoporosis (IJO), permanent bone deformities may occur. The effects of long-term pamidronate treatment on clinical findings, bone mineral status, and fracture rate were evaluated. Nine patients (age 9.8 ± 1.1 years, 7 males) with IJO were randomized to intravenous pamidronate (0.8 ± 0.1 mg/kg per day for 3 days; cycles per year 2.0 ± 0.1; duration 7.3 ± 1.1 years; n = 5) or no treatment (n = 4). Fracture rate, phalangeal quantitative ultrasound, and lumbar bone mineral density (BMD) by dual energy X-ray absorptiometry at entry and during follow-up (range 6...
September 2013: Journal of Bone and Mineral Metabolism
Justine Bacchetta, Katherine Wesseling-Perry, Vicente Gilsanz, Barbara Gales, Renata C Pereira, Isidro B Salusky
BACKGROUND: Idiopathic juvenile osteoporosis (IJO) is a rare condition of poorly understood etiology and pathophysiology that affects otherwise healthy children. This condition is characterized clinically by bone pain and vertebral fractures; spontaneous recovery is observed after puberty in the majority of cases. Although decreased trabecular bone turnover has been noted previously, cortical and trabecular bone characteristics as determined by quantitative computed tomography (QCT) and their relationship to bone histomorphometry are unknown...
2013: Pediatric Rheumatology Online Journal
Shumpei Yokota, Toshio Tanaka, Tadamitsu Kishimoto
Systemic juvenile idiopathic arthritis (SJIA), a subtype of juvenile idiopathic arthritis, is characterized by systemic features, such as spiking fever, salmon-colored macular rash, serositis, lymphadenopathy, hepatosplenomegaly, and joint inflammation. It is also often complicated with growth retardation, osteoporosis, and sometimes macrophage activation syndrome (MAS) develops, a potentially fatal disease. Pathogenesis of SJIA and MAS is not yet fully understood, but activation of the innate immune system, which causes phagocytosis by dendritic cells, monocytes, and macrophages to produce proinflammatory cytokines such as interleukin-6 (IL-6), IL-1β and IL-18, is thought to be a primary abnormality associated with SJIA...
December 2012: Therapeutic Advances in Musculoskeletal Disease
Yutaka Inaba, Remi Ozawa, Chie Aoki, Tomoyuki Imagawa, Masaaki Mori, Ryoki Hara, Takako Miyamae, Tomoyuki Saito, Shumpei Yokota
OBJECTIVES: To assess the efficacy of tocilizumab for preventing damage to the joints of systemic juvenile idiopathic arthritis (sJIA) patients, we examined serial radiographs of the hands and large weight-bearing joints of these patients before and after treatment with this agent. METHODS: Nine patients with sJIA receiving 8 mg/kg of tocilizumab intravenously every 2 weeks were studied. The mean follow-up period was 82 months. The number of active joints and laboratory markers of inflammation were assessed before and after tocilizumab treatment, together with radiologic evaluation of the hips, knees, ankles, shoulders, and elbows...
July 2013: Modern Rheumatology
Remi Ozawa, Yutaka Inaba, Masaaki Mori, Ryoki Hara, Masako Kikuchi, Rumiko Higuchi, Takako Miyamae, Tomoyuki Imagawa, Takeo Fujiwara, Tomoyuki Saito, Shumpei Yokota
We performed this study to investigate the differences in radiological and laboratory findings between systemic juvenile idiopathic arthritis (s-JIA) and polyarthritis (p-JIA). Twenty-two patients with s-JIA and 18 with p-JIA were enrolled. Their laboratory findings and radiographs were examined retrospectively. Plain radiographs were obtained before the induction of biological agents. All radiographs were examined for the presence of soft tissue swelling, juxta-articular osteopenia, joint space narrowing, subchondral bone cyst, erosion, epiphyseal irregularity, and growth abnormalities...
August 2012: Modern Rheumatology
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