1/24. 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 = 1keywords = osteonecrosis (Clic here for more details about this article) |
2/24. Burkitt's lymphoma presenting with jaw lesions.We report an unusual case of Burkitt's lymphoma (BL) presenting with jaw lesions in a 14-year-old Chinese boy. The patient presented initially with mobile teeth in all 4 jaw quadrants, with corresponding radiographic detection of alveolar bone crest destruction and periapical bone resorption in the absence of clinically detectable jaw tumors. Moreover, radiographs taken only 17 days later showed clearly distinguishable signs of more extensive alveolar bone destruction compared with the initial radiographs.- - - - - - - - - - ranking = 0.064591381513987keywords = jaw (Clic here for more details about this article) |
3/24. Long-term follow-up of two children with a variant of mild autosomal recessive osteopetrosis undergoing bone marrow transplantation.Malignant autosomal recessive (AR) osteopetrosis represents an absolute indication for bone marrow transplantation (BMT). Over the last 15 years, almost 100 BMTs for osteopetrosis have been reported. The median age at transplant of most patients is 4 months. Very few cases of mild AR osteopetrosis have been described. Here, we report the good outcome of two cases of mild AR osteopetrosis with a follow-up of 5 and 6 years, respectively, after an HLA-identical sibling transplant undergone at 5 and 12 years of age, respectively. At the time of BMT, severe visual impairment was present in both children. Bone biopsy demonstrated hypermineralization with virtual obliteration of the medullary spaces, rare microfoci of hematopoiesis and marked deficiency in osteoclastic activity. Successful engraftment was complicated by hypercalcemia, controlled by a combination of bisphosphonate, phosphate infusions, vigorous hydration and calcitonin. Following BMT, radiological and histological findings showed extensive bone resorption with marked augmentation of the osteoclasts in normalized marrow. No improvement was observed in visual acuity, despite complete remodeling of skeletal abnormalities. We conclude that allogeneic BMT is the only chance of curing mild AR osteopetrosis.- - - - - - - - - - ranking = 3.2210616022782keywords = bisphosphonate (Clic here for more details about this article) |
4/24. Clinical effect of etidronate on alveolar pyorrhoea associated with chronic marginal periodontitis: report of four cases.Etidronate 200 mg daily was administered to four female patients with periodontitis and resultant alveolar pyorrhoea for periods of 2 weeks, followed by off-periods of 10 weeks or more, for 2-3 years. The macroscopic appearance of gingival mobility of the teeth, depth of periodontal pockets, and X-ray findings of alveolar bones improved markedly during this time. The effects were first observed after 6-12 months of treatment. These findings indicate that bisphosphonates may be effective in the treatment of periodontitis and resultant alveolar pyorrhoea. The effect may be mediated by the inhibitory action on bone resorption and the anti-inflammatory action of etidronate. Concomitant conventional dental management is also required.- - - - - - - - - - ranking = 3.2210616022782keywords = bisphosphonate (Clic here for more details about this article) |
5/24. parathyroid hormone-related protein induced coupled increases in bone formation and resorption markers for 7 years in a patient with malignant islet cell tumors.parathyroid hormone-related protein (PTHrP) and PTH share the common PTH/PTHrP receptor. Although an elevated level of circulating PTHrP in patients with malignancies causes hypercalcemia as does PTH, chronic and systemic effects of PTHrP on bone metabolism in humans are not well understood because tumor-burden patients showing hypercalcemia usually have a poor prognosis. We investigated bone and calcium metabolism in a patient with malignant islet cell tumors showing hypercalcemia due to the elevated plasma PTHrP level for 7 years. hypercalcemia and hypercalciuria continued throughout the clinical course in spite of frequent infusions of bisphosphonates. bone resorption markers and a bone formation marker were consistently elevated as seen in primary hyperparathyroidism, a disease caused by an autonomous hypersecretion of PTH. Based on biochemical measurements including bone markers and serum 1,25-dihydroxyvitamin D, the clinical features of this case essentially are the same as those of primary hyperparathyroidism except for the elevated level of plasma PTHrP with suppressed intact PTH level. Therefore, it is suggested that chronic and systemic effects of PTHrP on bone as well as calcium metabolism are indistinguishable from those of PTH in human.- - - - - - - - - - ranking = 3.2210616022782keywords = bisphosphonate (Clic here for more details about this article) |
6/24. Hadju-Cheney syndrome: response to therapy with bisphosphonates in two patients.Hadju-Cheney syndrome is characterized by short stature, distinctive facies, and a slowly progressive skeletal dysplasia including acro-osteolysis. Autosomal dominant inheritance is typical, but the genetic defect and molecular pathogenesis of the syndrome are unknown. osteoporosis with atraumatic fracture is a frequent finding, and previous studies have documented biochemical and morphometric evidence of high bone turnover. Here, we report the clinical details and response to therapy with bisphosphonates in two patients (mother and son) with Hadju-Cheney syndrome and postulate that osteoclast-mediated bone resorption is important in the generalized osteoporosis commonly associated with this condition.- - - - - - - - - - ranking = 16.105308011391keywords = bisphosphonate (Clic here for more details about this article) |
7/24. orthognathic surgery and implants.The resorptive pattern of the maxilla and mandible after the dentition has been lost often leads to a discrepancy between the jaws such that a significant Class III malocclusion occurs. This discrepancy between the jaws leads to problems with both fixed and removable prosthetic reconstruction of the jaws with teeth. The jaws are often resorbed to the point where the muscle attachments become dislodging forces for conventional dentures and also to the point where adequate implants cannot be placed. Various procedures have been developed to augment the alveolar ridges to allow for adequate reconstruction of the dentition. orthognathic surgical procedures have been developed to reposition the jaws and have been traditionally used in the dentate patient to correct a skeletal malocclusion. These procedures are usually carried out with orthodontic control of the dentition to produce the best results. These same procedures can be used on the edentulous patient to correct the discrepancies between the jaws in order to reconstruct the dentition with implants. Bone grafting procedures are often required for these procedures so that the alveolus can be augmented at the same time and allow for dental implants to be placed at a later date.- - - - - - - - - - ranking = 0.055364041297703keywords = jaw (Clic here for more details about this article) |
8/24. Treatment of idiopathic hyperphosphatasia with intensive bisphosphonate therapy.In a family with IH, a rare high turnover bone disease, two older siblings were wheelchair-bound with severe skeletal deformity by age 15. Their youngest affected sibling was treated intensively with intravenous bisphosphonates for 3 years. The treatment was well tolerated and prevented the development of deformity and disability. INTRODUCTION: Idiopathic hyperphosphatasia (IH, also known as juvenile Paget's disease) is a rare genetic bone disease characterized by very high bone turnover and progressive bony deformity. Inhibitors of bone resorption have been used to suppress bone turnover in the short term, but there is no published data on long-term efficacy. MATERIALS AND methods: An 11-year-old girl with IH, who had two severely affected older siblings, presented with progressive deformity and deafness and long bone fractures. Conventional pediatric doses of pamidronate had failed to prevent clinical deterioration or suppress bone turnover completely. Intensive bisphosphonate therapy (frequent 5-mg ibandronate infusions) was given to try and arrest progression of the skeletal disease. growth and development, pure tone audiometry, biochemistry, radiology, densitometry (DXA), and bone histology were monitored. RESULTS: A total of 45 mg ibandronate was given over 3 years until skeletal maturity was reached (20, 15, and 10 mg for years 1-3, respectively). Ibandronate treatment was well tolerated, and biochemical markers of bone turnover suppressed to within the age-appropriate normal range There was some progression of her thoracic kyphosis, but she had no further fractures and remained mobile and active at an age when her siblings had become wheelchair-bound. A significant recovery of hearing (p < 0.01) was documented, particularly at low frequencies. Radiographs showed improvement in spinal osteoporosis and cortical bone dimensions and arrest of progressive acetabular protrusion. Areal bone density increased substantially (lumbar spine z-score from -2.2 to 1.8). tetracycline-labeled bone biopsy specimens were taken before and after 18 months of intensive treatment. The second biopsy showed suppression of bone turnover and a doubling of trabecular thickness, with no mineralization defect, and no osteopetrosis. CONCLUSIONS: Intensive bisphosphonate treatment prevented the development of deformity and disability and improved hearing in this child with IH. The dose of bisphosphonate, which is substantially greater than is usually used in pediatric bone disease, had no adverse effects, in particular on bone mineralization.- - - - - - - - - - ranking = 25.768492818226keywords = bisphosphonate (Clic here for more details about this article) |
9/24. osteomyelitis of the mandible during pregnancy.A 21-year-old woman who was 9 weeks pregnant presented with osteomyelitis of the mandible that resolved spontaneously after delivery. To our knowledge a link between osteomyelitis of the jaws and pregnancy has not been reported previously.- - - - - - - - - - ranking = 0.0092273402162839keywords = jaw (Clic here for more details about this article) |
10/24. Recombinant osteoprotegerin for juvenile Paget's disease.Juvenile Paget's disease, a genetic bone disease characterized by accelerated bone turnover, results from inactivating mutations in the gene encoding osteoprotegerin--a key regulator of osteoclastogenesis. The effects of recombinant osteoprotegerin were investigated in two adult siblings with juvenile Paget's disease. bone resorption (assessed by N-telopeptide excretion) was suppressed by once-weekly subcutaneous doses of 0.3 to 0.4 mg per kilogram of body weight. After 15 months of treatment, radial bone mass increased in one patient by 9 percent and in the other by 30 percent, skeletal bisphosphonate retention decreased by 37 percent and 55 percent, respectively, and there was radiographic improvement. Apart from mild hypocalcemia and hypophosphatemia, no apparent adverse events occurred.- - - - - - - - - - ranking = 3.2210616022782keywords = bisphosphonate (Clic here for more details about this article) |
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