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BACKGROUND: Pediatric patients with Crohn's disease often have low bone mass (osteopenia) for age. No randomized, placebo-controlled trials using zoledronic acid have ever been performed in this population. The objective of this study was to assess the efficacy of zoledronic acid in children with Crohn's disease and osteopenia. METHODS: A double-blind, randomized, placebo-controlled design was used. Thirteen adolescents received either a single intravenous dose of zoledronic acid (0.066 mg/kg, max 4 mg, n= 7) or saline placebo (n= 6). The primary outcome was change in lumbar spine bone mineral density (LSBMD) z-score at 6 months. Secondary outcomes included bone markers and adverse events. RESULTS: At 6 months, the change in LSBMD z-score was significantly higher in the zoledronic acid group compared to placebo (0.7 vs 0.1, P < 0.001). Volumetrically adjusted LSBMD z-score also significantly increased in the treated group. This significant difference persisted until 12 months. With zoledronic acid, urinary C-telopeptide excretion decreased by 50% at 6 months and remained suppressed at 12 months (P= 0.02), but no changes were observed with placebo. Both groups had similar adverse events which included transient fever, arthralgias, and nausea (3/7 treated, 2/6 placebo, P= NS). CONCLUSIONS: In this study, zoledronic acid demonstrated a significant increase in LSBMD at 6 and 12 months following a well-tolerated infusion.

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Caudal dysplasia syndrome (CDS) is associated with hypoplastic lower extremities, caudal vertebrae, sacrum, neural tube, and urogenital organs. Sirenomelia is characterized by a single lower extremity, absent sacrum, urogenital anomalies, and imperforate anus. There is controversy in the medical literature about whether sirenomelia and CDS are part of the spectrum of the same malformation. Patients with CDS and sirenomelia were identified from our pathology files from 1991 to 2006. Maternal history, pathologic examination, and radiographs were collected and tabulated. We found 9 cases with CDS and 6 with sirenomelia. Fully 7 of 9 patients with CDS (77.7%) versus none of sirenomelic babies were infants of diabetic mothers. Congenital heart disease was present in 5 patients with CDS (55.5%) and none of the infants with sirenomelia. Of 9 children with CDS 2 (22.2%) had bilateral renal agenesis versus 66% of sirenomelics. Single umbilical artery was found in 33% of cases with CDS and 100% of children with sirenomelia. External genitalia were ambiguous in 2 of 9 patients (22.2%) with CDS and in all patients with sirenomelia. Imperforate anus was found in 10 cases (66.6%) divided as 4 of 9 babies with CDS (44.4%) and all patients with sirenomelia. Three patients with CDS had concomitant maternal diabetes mellitus and chronic hypertension. These babies also had cleft lip and palate. Congenital heart disease was found in 55.5% of cases with CDS and none of the children with sirenomelia. We conclude that although CDS and sirenomelia share many similar features, they are two different entities.

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OBJECTIVES: To determine the prevalence of and the risk factors for vertebral fractures in a cohort of children with chronic rheumatic diseases considered at risk for osteopenia. STUDY DESIGN: We conducted a cross-sectional study of patients with chronic rheumatic diseases at the Montreal Children's Hospital. RESULTS: Of the 90 study participants (22 boys, 68 girls), 10 boys and 7 girls (19%) were found to have vertebral fractures. These 17 children had a total of 50 fractures, an average of 2.9 per affected child. Fractures in the upper thoracic region (T5-8) accounted for 55%. Only 56% of all fractures were symptomatic. With multivariate regression, we identified male sex (P < .01), body mass index z-score (P < .02), and cumulative glucocorticoid dose (P < .01) as significant predictors of the number of vertebral fractures. CONCLUSIONS: Our study examined the prevalence of vertebral fractures in a high-risk pediatric population. Nineteen percent of our cohort had vertebral fractures. Significant risk factors for the development of vertebral fractures include male sex and cumulative glucocorticoid dose. Better understanding of the extent of the problem in this population will allow us to further refine screening guidelines and treatment in these patients.

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Hyperplastic callus (HPC) formation is a prominent feature of osteogenesis imperfecta (OI) type V; however, little is known about its long-term outcome. In this case report we describe the occurrence, appearance and course of a femoral HPC in a patient with OI type V during 10 years of follow-up. Radiographs of HPC in this child were compared and contrasted with HPC formation in the femur of his father and paternal grandfather, who also were affected with OI type V. This case report makes it clear that HPC can lead to significant morbidity, not only in the acute phase but also long term as a result of residual alteration in bone architecture.

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Anaplastic large cell lymphoma (ALCL) is predominantly a systemic disease with nodal involvement, but extranodal involvement can occur either as the primary presentation or during the disease course. Primary epiphyseal involvement is extremely rare with lymphomas. This case report illustrates an 8-year old boy who first presented with pain over the right upper extremity, which was initially treated as epiphyseal osteomyelitis. A few weeks later, he presented with abdominal pain and an abdominal wall mass, which on biopsy proved to be an anaplastic large-cell lymphoma.

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Dysplasia epiphysealis hemimelica (DEH) is a rare skeletal dysplasia with epiphyseal involvement first described by Mouchet and Belot in 1926. Lower extremity involvement is common and might involve a single or multiple epiphyses in the affected extremity. We report an unusual case of involvement of the tibial tubercle in a girl aged 4 years 8 months, and we present the clinical, radiographic and pathologic findings. We discuss the role of MRI in the diagnosis and treatment plan.

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Langerhans' cell histiocytosis (LCH) is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhans' cell. In bone this may cause pain and adjacent soft-tissue swelling, but some lesions are asymptomatic. LCH can involve any bone, but most lesions occur in the skull (especially the calvarium and temporal bones), the pelvis, spine, mandible, ribs, and tubular bones. Imaging diagnosis of the disease in bone is first based on the plain radiographic appearance, which is usually a central destructive, aggressive-looking lesion. In the skull, the lesions develop in the diploic space, are lytic, and their edges may be beveled, scalloped or confluent (geographic), or show a "button sequestrum." Vertebral body involvement usually causes collapse, resulting in vertebra plana. With significant recent improvements in the quality of gamma cameras, imaging techniques, and in studying children, bone scintigraphy at diagnosis and on follow-up usually reveals the sites of active disease, especially when the involvement is polyostotic. CT and MR imaging are very useful in providing detailed cross-sectional anatomic detail of the involved bone, including the bone marrow and the adjacent soft tissues. CT is better suited for demonstrating bone detail and MR imaging for bone marrow and soft-tissue involvement.

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Infection of bone in children represents a diagnostic and therapeutic challenge to the pediatrician, the surgeon, the radiologist, and the pathologist. The pattern of manifestation varies and is dependent on the site of involvement, the initiating event, the infecting organism, and the acute or chronic nature of the illness. Early diagnosis allows prompt treatment, which can prevent many of the dreaded complications of this disease. Plain radiographs remain the initial imaging modality used in the diagnosis and differential diagnosis of osteomyelitis. However, with the advent of newer imaging techniques such as scintigraphy, ultrasonography, computed tomography, and magnetic resonance imaging, a higher degree of accuracy in diagnosis and definition of the extent of the disease has been achieved. The time interval between the onset of symptoms and diagnosis has also been significantly reduced. Although magnetic resonance imaging has the additional advantage of multiplanar capability, greater anatomic detail, and excellent soft tissue resolution, it is a relatively expensive technique, which somewhat limits its use. Following a brief review of the classification and pathophysiology of osteomyelitis, we have tried to elucidate the utility of various imaging modalities in the diagnosis of osteomyelitis in children, outlining their relative strengths and weaknesses. Certain entities specific to children and a brief review of differential diagnosis are also presented.

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In children with low back pain (LBP), a specific cause is often identified. LBP has a relatively high prevalence during school years. However, only a minority of the children suffering from LBP seek medical attention. Protracted back pain in childhood is a serious condition that should be thoroughly investigated. This article is a systematic review of the intrinsic causes of LBP. Imaging modalities are discussed, with emphasis on magnetic resonance imaging. We have divided the intrinsic causes of LBP into four main groups: mechanical, developmental, infectious/inflammatory, and neoplastic. Disk protrusion is prevalent in young athletes. Spondylolysis and spondylolisthesis are the most common causes of chronic LBP in children. Thoracic or thoracolumbar Scheuermann disease causes kyphosis while a lumbar localization is more painful. Childhood diskitis is associated with fever and leukocytosis. Spinal inflammatory arthritides in children include juvenile rheumatoid arthritis, the juvenile spondyloarthropathies, and SAPHO syndrome, where spine as well as sacroiliac joint changes may be seen. Cysts, tumors, tumor-like lesions, and metastases are infrequent causes of back pain in children. Several of these conditions are described and illustrated in this review of LBP in children and adolescents.

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Imaging is crucial for the diagnosis and differential diagnosis of arthritis. Plain radiography is the primary tool used to assess and monitor the progression of arthritis or its response to therapy. The newer imaging modalities offer a more complete assessment of the joints in health and disease. Magnetic resonance (MR) imaging with gadolinium administration allows direct visualization of the inflamed synovium and pannus. With MR imaging articular cartilage can be seen as well as joint effusion and hemosiderin, if present. Small (and large) joint effusions are readily revealed using ultrasonography, which is a relatively easy and inexpensive technique.

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A benign bone lesion may have a typical appearance on plain radiographs. This is the case with benign cortical defects and osteochondroma. With most other lesions, cross-sectional imaging is needed to complete the study of the tumor. The nidus of osteoid osteoma is well demonstrated on computed tomography, but magnetic resonance imaging also will show the nidus in most cases. Magnetic resonance imaging is considered the modality of choice for evaluation of other benign musculoskeletal lesions because it is highly sensitive to changes in the signal intensity of bone marrow and adjacent soft tissues. It provides useful information for diagnosis of the lesion as in primary or secondary aneurysmal bone cyst, chondroblastoma, osteoblastoma, fibrous dysplasia, and osteofibrous dysplasia, and it helps differentiate these lesions from osteomyelitis, Langerhans' cell histiocytosis, and stress fracture. Bone scanning is most useful for depicting multiple silent lesions as may be seen in multiple osteochondromatosis, nonossifying fibromas, and polyostotic fibrous dysplasia.

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