1/64. Study of the cell biology and biochemistry of cherubism.AIMS: To establish whether the multinucleate cells in lesions of patients with cherubism are also osteoclasts and if this is the case whether they were responsive to calcitonin; to carry out cytogenetic studies on two members of the same family affected by cherubism in an attempt to identify any major chromosomal defects; and to perform an in-depth modern biochemical study of four children in the same family. SUBJECTS AND methods: Four related children with cherubism were studied. Tissue taken from one of the children at elective decompression of an optic nerve was submitted to in vitro bone resorption studies. Cytogenetic studies were done on two of the children and biochemical studies on all four. RESULTS: The multinucleate cells in the cherubic lesions were shown to be osteoclasts since they synthesised tartrate resistant acid phosphatase, expressed the vitronectin receptor, and resorbed bone. bone resorption by the cultured multinucleate cells was significantly inhibited by calcitonin. High resolution cytogenetic studies failed to detect any chromosomal abnormalities in two children with cherubism. The biochemistry profile of all four children with cherubism showed that serum calcium, parathyroid hormone, parathyroid related hormone, calcitonin, and alkaline phosphatase were within normal levels. urine analysis of pyridinium and deoxypyridinium cross links, hydroxyproline, and calcium in relation to urine creatinine were measured to assess bone resorption in these children, and the values were at the upper end of the normal range in all four. CONCLUSIONS: Further studies are required to determine whether calcitonin treatment will control this grossly deforming disease until the time when the physiological changes that occur at puberty rectify the pathology. It is not recommended that biochemical markers of bone resorption are used in isolation to monitor the activity of cherubism in individuals because the results are based on a small number of children and because of reports of marked interindividual variation in the levels of these markers, particularly in children.- - - - - - - - - - ranking = 1keywords = compression (Clic here for more details about this article) |
2/64. Reactions and complications after the implantation of Endobon including morphological examination of explants.In the study described here, the integration of hydroxyapatite (HA) ceramic implants (Endobon) was investigated. These implants have an interconnecting system of pores and are free from foreign protein. The material is not toxic, genotoxic, nor zytotoxic, and it is biocompatible. The progress of integration was investigated by means of clinical and radiological check-ups. From 10 patients, it was possible to obtain samples for histological analysis during a second operation (e.g., metal explantation). Microscopic examination showed bony integration with newly formed bone in direct contact with the HA ceramic; it also showed osteoblasts and osteoid seams. No second operation took place earlier than 4 months after the first operation, yet even after this relatively short period, bony integration was already evident. Clinical observation (based on x-rays, reports of pain, signs of inflammation) showed that in most cases healing was taking place without complications. More general operational complications such as thrombosis or nerve injury were observed in 4 patients. If the implant is not sufficiently protected from mechanical stress, bony integation will not take place, and the implant may fracture. HA ceramic, with a porosity between 30% and 80%, is not comparable to cortical bone but only to spongy bone. This factor must be taken into account when deciding whether a HA ceramic implant is indicated.- - - - - - - - - - ranking = 108.39045820008keywords = fracture (Clic here for more details about this article) |
3/64. Progressive bone resorption after pathological fracture of the femoral neck in Hunter's syndrome.We report a case of Hunter's syndrome associated with a transverse fracture of the left femoral neck after minor trauma, followed by progressive resorption of the femoral head at 12 years of age and a stress fracture of the right femoral neck at 16 years of age. MRI performed at 15 years of age revealed intra-articular low intensity on T1-weighted and T2-weighted images of both hip joints. The MR finding may represent fibrous synovial thickening, which caused pressure erosion of the femoral neck, resultant pathological and/or stress fractures, and subsequent osteonecrosis with rapid absorption of the femoral head.- - - - - - - - - - ranking = 758.73320740057keywords = fracture (Clic here for more details about this article) |
4/64. Severe osteoporosis in familial hajdu-cheney syndrome: progression of acro-osteolysis and osteoporosis during long-term follow-up.hajdu-cheney syndrome is an autosomal dominant inherited osteodysplastic bone disease with the hallmarks of acro-osteolysis, skull deformations, and generalized osteoporosis. Very few patients have been followed long-term with respect to the prognosis of acro-osteolysis and osteoporosis. Here we describe a 39-year-old woman and her 19-year-old daughter who are both affected with the hajdu-cheney syndrome. Skeletal lesions were followed in the mother between the ages of 22 and 39 years. The acro-osteolytic lesions progressed markedly and caused shortening of several fingers; some end phalanges had completely disappeared. Severe spinal osteoporosis with serial vertebral fractures was found at the age of 22 years. New vertebral fractures developed until the age of 33 years, but did not progress afterward. High turnover osteoporosis was found in the bone histology of iliac crest biopsies performed at the ages of 22 and 34 years. Bone mineral content (BMC) was strikingly decreased at the age of 34 years (T score -5.1 SD) and did not significantly change during further follow-up. In the daughter, BMC failed to increase between the ages of 12 and 19 years and was also markedly decreased (T score -4.4 SD). This suggests that osteoporosis in hajdu-cheney syndrome is related to a low peak bone mass and a high bone turnover, leading to insufficient bone formation compared with the increased bone resorption.- - - - - - - - - - ranking = 216.78091640016keywords = fracture (Clic here for more details about this article) |
5/64. Recombinant bone morphogenetic protein-7 as an intracorporal bone growth stimulator in unstable thoracolumbar burst fractures in humans: preliminary results.The study presented here is a pilot study in five patients with unstable thoracolumbar spine fractures treated with transpedicular OP-1 transplantation, short segment instrumentation and posterolateral fusion. Recombinant bone morphogenetic protein-7 in combination with a collagen carrier, also referred to as OP-1, has demonstrated ability to induce healing in long-bone segmental defects in dogs, rabbits and monkeys and to induce successful posterolateral spinal fusion in dogs without need for autogenous bone graft. Furthermore OP-1 has been demonstrated to be effective as a bone graft substitute when performing the PLIF maneuver in a sheep model. Five patients with single-level unstable burst fracture and no neurological impairment were treated with intracorporal OP-1 transplantation, posterior fixation (USS) and posterolateral fusion. One patient with osteomalacia and an L2 burst fracture had an additional intracorporal transplantation performed proximal to the instrumented segment, i.e. OP-1 into T 12 and autogenous bone into T 11. Follow-up time was 12-18 months. On serial radiographs, Cobb and kyphotic angles, as well as anterior, middle and posterior column heights, were measured. Serial CT scans were performed to determine the bone mineral density at fracture level.In one case, radiographic and CT evaluation after 3 and 6 months showed severe resorption at the site of transplantation, but after 12 months, new bone had started to fill in at the area of resorption. In all cases there was loss of correction with regard to anterior and middle column height and sagittal balance at the latest follow-up. These preliminary results regarding OP-1 as a bone graft substitute and stimulator of new bone formation have been disappointing, as the OP-1 device in this study was not capable of inducing an early sufficient structural bone support. There are indications to suggest that OP-1 application to a fracture site in humans might result in detrimental enhanced bone resorption as a primary event.- - - - - - - - - - ranking = 975.51412380073keywords = fracture (Clic here for more details about this article) |
6/64. Painful trigeminal neuropathy caused by severe mandibular resorption and nerve compression in a patient with systemic sclerosis: case report and literature review.Systemic sclerosis is a multi-system disorder characterized by abundant fibrosis of the skin, blood vessels, and visceral organs. Although resorption of the mandible has been commonly observed and reported, we found no report of resorption leading to a painful neuropathy of the inferior alveolar nerve. We report a case of a patient with systemic sclerosis, severe resorption of the angles, and inferior alveolar border of the mandible, resulting in a compression neuropathy of the inferior alveolar nerve. Diagnostic tests, medical treatment, and surgical treatment are discussed, and the relevant literature is reviewed.- - - - - - - - - - ranking = 5keywords = compression (Clic here for more details about this article) |
7/64. Primary neonatal hyperparathyroidism. Report of a case and review of the literature.The seventh case in the world literature of primary hyperparathyroidism in a neonate is reported. This is the fifth case in which an autopsy was performed. The clinical and anatomic findings in all seven cases are reviewed and compared. Neonates with primary hyperparathyroidism show diffuse hyperplasia of the parathyroid glands. The bones show disturbed osteogenesis, bone resorption, and widespread fibrosis of the marrow cavities. Bony cysts are not appreciated. Pathologic fractures are common. Marked hypercalciuria or hyperphosphaturia is usually not observed, perhaps because the immature renal tubules fail to respond to the influence of excess parathormone. Aminoaciduria and anemia are commonly observed. The prognosis is grave, and the etiology of this syndrome remains unexplained.- - - - - - - - - - ranking = 108.39045820008keywords = fracture (Clic here for more details about this article) |
8/64. Massive osteolysis of the humerus with spontaneous recovery.An elderly woman presented with a pathological fracture of the right humerus. Progressive dissolution of the shaft of this bone took place over six months. No cause could be established and the patient refused biopsy. With only simple splintage for treatment the humeral shaft gradually reformed and re-ossified over a period of two years. The patient has been under review for four and a half years and no further pathology has come to light. The cause of the osteolysis remains obscure.- - - - - - - - - - ranking = 108.39045820008keywords = fracture (Clic here for more details about this article) |
9/64. Primary hyperparathyroidism: what every orthopedic surgeon should know.The diagnosis of hyperparathyroidism should rarely by missed by the orthopedic surgeon. When a patient presents with a pathologic fracture, routine serum calcium should be obtained. If there is evidence of elevated serum calcium or any of the pathognomonic findings of primary hyperparathyroidism on plain radiographs, total and ionized calcium and an intact parathyroid hormone levels should be obtained to make the diagnosis (Figure 5). When patients require surgical treatment for an orthopedic condition and also need surgery for hyperparathyroidism, the procedures can be safely performed simultaneously. Simultaneous parathyroidectomy corrects the underlying endocrinopathy, thereby improving the outcome of the orthopedic procedure. In addition, these procedures can easily be performed simultaneously under one anesthetic and thereby minimize cost and length of hospitalization.- - - - - - - - - - ranking = 108.39045820008keywords = fracture (Clic here for more details about this article) |
10/64. Complications with intermaxillary fixation screws in the management of fractured mandibles.A dedicated bicortical bone screw for temporary intraoperative intermaxillary fixation (IMF) during open reduction and fixation of mandibular fractures offers many benefits to surgeons and patients. We have used this system for 2 years and confirm the benefits over traditional methods of intermaxillary fixation. However, complications can arise. One hundred and twenty-two patients with mandibular fractures had IMF screws of which five (4%) developed complications intraoperatively and postoperatively. Complications included fracture of the screws on insertion, iatrogenic damage to teeth causing loss and bony sequestra around the area of screw placement.- - - - - - - - - - ranking = 758.73320740057keywords = fracture (Clic here for more details about this article) |
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