Cases reported "Bone Diseases, Metabolic"

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1/61. Mesomelic dysplasia with periosteal thickening, radio-humeral dislocation, osteoporosis and multiple fractures.

    We report a boy with a new form of mesomelic dysplasia characterised by short stature, multifocal periosteal thickening, radio-humeral dislocation, osteoporosis and multiple fractures with minimal trauma. electrophoresis of fibroblast collagens detected defects in type III and type V collagen. CONCLUSION: Bone dysplasias presenting with osteopenia, abnormal trabecular pattern, bone fragility, and periosteal thickening suggest a collagenopathy. A possible collagen defect requires biochemical investigations.
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2/61. Fibrogenesis imperfecta ossium: imaging correlation in three new patients.

    OBJECTIVE: Fibrogenesis imperfecta ossium is an extremely rare disorder that can easily be misdiagnosed. We retrospectively reviewed the clinical and imaging data of three confirmed cases of fibrogenesis imperfecta. DESIGN AND patients: The patients consisted of two men and one woman, ranging in age from 40 to 53 years. radiography was performed in all the patients. One patient had a 3-year follow-up of the thoracolumbar spine with conventional radiography and thoracolumbar magnetic resonance (MR) imaging. Open biopsy was performed in all cases, confirming the diagnosis of fibrogenesis imperfecta ossium. RESULTS: All our cases demonstrated "fishnet" trabecular pattern by conventional radiographs, and a pelvis radiograph of one patient showed an equivocal sclerosis pattern. Multiple fractures were noted in two patients. A pseudoexostosis was present in the ilium in one patient. Thoracolumbar MR imaging demonstrated diffuse low signal intensity within the medullary space on both T1-weighed and T2-weighted images, except for a region of increased signal intensity in the L1 and L2 vertebral bodies on T2-weighed images due to edema from acute collapse. CONCLUSIONS: Although uncommon, fibrogenesis imperfecta ossium should be considered in a previously healthy patient with a combination of progressive bone pain, unexplained fractures, a radiologic pattern of fishnet osteopenia and MR imaging of low signal intensity bone marrow on both T1-weighted and T2-weighted images.
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3/61. hip fracture and bone histomorphometry in a young adult with cystic fibrosis.

    A 25-yr-old male with cystic fibrosis sustained a fragility fracture of the left femoral neck, which required surgical correction. He had several risk factors for the development of low bone density and despite treatment with an oral bisphosphonate, his bone mineral density reduced further. The patient died 2 yrs after sustaining the fracture. Bone specimens obtained at post mortem demonstrated severe cortical and trabecular osteopenia, but the histological features were not typical of osteoporosis or osteomalacia. osteoporosis is thought to be a common complication of cystic fibrosis. The novel histomorphometric appearances reported here suggest that the bone disease of cystic fibrosis may be more complex and possibly unique. Labelled bone biopsies are required to clarify the bone defect leading to low bone density in cystic fibrosis patients so that appropriate therapeutic strategies can be developed.
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ranking = 1.2
keywords = fracture
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4/61. osteomalacia in a patient with severe anorexia nervosa.

    A 27-year-old woman with anorexia nervosa since adolescence was referred to our unit for generalized bone pain most severe at the pelvis and an inability to stand. She reported a pelvic fracture diagnosed one year earlier, which had failed to heal. Laboratory tests showed low serum phosphate, normal total serum calcium corrected for serum albumin, and very low urinary calcium excretion. Serum bone alkaline phosphatase and parathyroid hormone levels were elevated, whereas 25-hydroxy-vitamin d was severely decreased. Multiple vertebral and rib fractures were seen on plain radiographs. Radiographic images consistent with osteomalacia were pseudofractures of the left inferior pubic ramus, a bilateral complete fracture of the superior pubic ramus, and a characteristic pseudofracture (Looser zone) in the lateral margin of the right scapula. Vitamin D-deficient osteomalacia with secondary hyperparathyroidism was strongly suspected at this point, but it was decided not to confirm this diagnosis by bone biopsy with histomorphometry and osteoid labeling because of the emotional instability of the patient. Dual-energy X-ray absorptiometry disclosed severe demineralization. After two months on calcium and vitamin d supplements, the bone pain had abated and the patient was able to stand. Serum calcium had increased; serum phosphate, 25-hydroxy-vitamin d, and parathyroid hormone had returned to normal, and the pseudofractures showed evidence of healing. osteoporosis is a well-known complication of anorexia nervosa. This case shows that osteomalacia can also occur. vitamin d status should be assessed in patients with long-standing severe anorexia nervosa.
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ranking = 1.2
keywords = fracture
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5/61. Lateral insufficiency fractures of the femur caused by osteopenia and varus angulation: a complication of total hip arthroplasty.

    Lateral femoral insufficiency fractures in total hip arthroplasty occur due to osteopenia and varus positioning of the femoral component, the femur itself, or both. The presentation of these fractures can be unclear but usually involves the insidious onset of unexplained thigh or groin pain. The patients are likely to have significant comorbidities as well. Characteristic radiographic findings may be present, depending on when the patient presents. The insufficiency fractures generally occur at the level of the femoral stem tip on the lateral cortex of the femur. If left untreated, pain and loss of function continue. Eventually an insufficiency fracture can progress to a displaced periprosthetic fracture. Nonsurgical treatment is not successful. Recommended treatment involves revision to a long-stem femoral component. The risk of postoperative complications is significant.
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ranking = 1.8
keywords = fracture
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6/61. New technique for treatment of unstable distal femur fractures by locked double-plating: case report and biomechanical evaluation.

    BACKGROUND: A comminuted, intra-articular distal femur fracture was surgically treated by the authors with a locked, double-plating technique because fixation stability could not be initially achieved by using a standard double-plating technique. The purpose of this study was to determine biomechanically whether a locked double-plate construct would enhance fixation stability compared with a nonlocked double-plate construct. methods: Six matched pairs of mildly osteopenic femurs were selected and all had a reproducible intra-articular fracture pattern created. Each pair underwent fixation with either a double-plating construct or a locked, double-plating construct that was randomly assigned. The instrumented femurs were then mechanically tested in several loading modes to determine fixation stability. After initial testing, specimens were cyclically loaded and retested for stability. RESULTS: The locked, double-plating construct provided significantly greater fixation stability than the standard double-plating construct in precycling and postcycling biomechanical testing. CONCLUSION: The technique described is particularly applicable for severely comminuted fractures of the distal femur and fractures in osteopenic bone with poor screw purchase. It offers a simple alternative for enhancing fixation stability, which avoids the potential complications of methylmethacrylate-enhanced screw fixation.
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ranking = 1.6
keywords = fracture
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7/61. association of posterior rib fractures with exaggerated kyphosis and sternal collapse.

    The ribs, sternum, and vertebrae all play an important role in stabilizing the thorax. Failure of one of these components places additional stress on the other supporting structures. We present a case of a 62-year-old man with multiple myeloma and osteopenia who sustained fractures to all three components.
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keywords = fracture
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8/61. rib fractures induced by coughing: an unusual cause of acute chest pain.

    We report three patients with stress fractures of the ribs induced by coughing. Standard radiographs of the chest and ribs did not reveal evidence of rib fractures in any of the patients. Bone scintigraphy, performed 1 to 2 weeks after initial onset of symptoms, showed a focal area of increased uptake along the chest wall in all cases. Thin section angulated helical CT directly visualized the subtle rib fractures. Initial diagnosis of a cough-induced fracture of the rib may be difficult because of the associated underlying disorder, and unnecessary examinations are commonly performed. Identification of a cough-induced fracture of the rib using helical CT may be clinically important to avoid unnecessary concern and additional examinations.
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ranking = 1.8
keywords = fracture
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9/61. Severe osteopenia with recurrent fractures after bone marrow transplant for wiskott-aldrich syndrome: a case report.

    wiskott-aldrich syndrome (WAS) is a rare inherited disorder characterised by thrombocytopenia, eczema, and immunodeficiency. bone marrow transplantation (BMT) is a well-established modality of treatment now routinely used and often curative. We report the case of a boy who developed osteopenia and sustained multiple long-bone fractures over a 5-year period after bone marrow transplant for WAS. The femora and tibiae of both lower limbs were involved with a clinical presentation similar to osteogenesis imperfecta. After commencing calcitriol treatment at the age of 8 years, the patient has not sustained any further fractures. He is now 11 years old. Although short-term changes in bone metabolism after BMT have been documented, the occurrence of repeated fractures associated with osteopenia has not been previously reported.
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ranking = 1.4
keywords = fracture
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10/61. Stress fracture of the hip and pubic rami after fusion to the sacrum in an adult with scoliosis: a case report.

    Correction of adult scoliosis frequently involves long segmental fusions, but controversy still exists whether these fusions should include the sacrum. It has been suggested that forces associated with activities of daily living transfer the stresses to the remaining levels of the spine and to the pelvis. The case described here was a 43-year-old woman with scoliosis and chronic back pain refractory to non-surgical modalities. Radiographically, the patient had a 110 degree lumbar curve. An anterior and posterior fusion with Luque-Galveston instrumentation was performed. Six months postoperatively the patient returned with a 2-week history of right hip pain with no history of trauma. There was radiographic evidence of a displaced femoral neck fracture and pubic rami fractures. The femoral neck fracture was treated with a total hip replacement. Further surgeries were required to correct a lumbar pseudoarthrosis and hardware failure. We believe that this case provides evidence that fusion into the lumbosacral junction may distribute forces through the pelvic bones and hip resulting in stress and potential hardware complications, especially in patients at risk due to osteopenic conditions.
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ranking = 1.4
keywords = fracture
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