1/28. Florid osseous dysplasia of the mandible: report of a case.In 1986, a 60-year-old African American woman visited the Marquette University School of dentistry with a complaint of a vague, dull pain in her lower left quadrant. tooth No. 19 was extracted 10 years earlier because of extensive decay, and tooth No. 18 had received root canal therapy. A panoramic radiograph revealed the presence of ill-defined, multilocular, mixed (radiopaque-radiolucent) lesions present throughout the lower jaw. She was treated with antibiotics and scheduled for follow-up visits. When the symptoms persisted, tooth No. 18 was re-treated with root canal therapy and a representative biopsy was taken from the left mandibular area. The biopsy showed the presence of chronic osteomyelitis. The patient was treated with antibiotics and was scheduled for periodic check-up visits. In February 1995, she returned with the same symptoms in the left mandible. A panoramic radiograph showed persistence of the mixed radiopaque-radiolucent lesions throughout her mandible; however, the mass on the left side was more radiopaque and had assumed a "cotton wool" appearance. tooth No. 18 was extracted and a biopsy was taken from the area. After correlating the clinical behavior, radiographic appearance, and histopathologic features, a diagnosis of florid osseous dysplasia with osteitis was made. This case represents a classic example of the difficulty in diagnosing fibro-osseous lesions using radiographic interpretation alone and the need to correlate the clinical, radiographic, and histopathologic features to reach a diagnosis. Additionally, the present case clearly shows treatment problems of an otherwise self-limiting condition when secondary involvement with osteomyelitis is also present. A brief description of the conditions that were included in the differential diagnosis and their management is presented.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |
2/28. The treatment with alendronate in hemifacial spasm associated with Paget's disease of bone.The association of Paget's disease of bone and hemifacial spasm has rarely been reported. hemifacial spasm is often associated with compression of the facial nerve by a vascular loop at the point where the nerve leaves the brainstem before traversing the cerebellopontine angle. It is believed that narrowing of the cerebellopontine angle cistern caused by Paget's disease increases the chance of vascular compression of the facial nerve. Whilst specific antipagetic therapy such as calcitonin has been used with good response in hemifacial spasm associated with Paget's disease, the usefulness of the newer bisphosphonates is not clear. A 65-year-old woman with hemifacial spasm associated with Paget's disease was treated with alendronate, and the hemifacial spasm became very infrequent 4 months after commencement of the therapy.- - - - - - - - - - ranking = 5423.864348941keywords = bisphosphonate (Clic here for more details about this article) |
3/28. Pseudosarcoma in Paget's disease.The authors describe the case of a male patient who had had Paget's disease since 1973 which led to the diffuse swelling of the proximal femur in 1993. The elevated alkaline phosphatase level and the destructive lytic lesions on the X-ray of the femur raised the possibility of malignancy. The bone scan showed increased accumulation in the proximal part of the left femur. On the MR the low T1 and the elevated T2 signal intensity suggested malignity despite that in some regions signals typical of adipose tissue could also be seen, which is usually interpreted as a sign of benignity. In the histological specimens no signs of malignant tumour cells were identified. The patient received bisphosphonate treatment which decreased his pain and swelling and also the alkaline phosphatase level. The patient died because of a rapidly progressing retroperitoneal fibrosarcoma and lung metastasis in 1996. The autopsy did not prove any malignant transformation of Paget's disease of bone in the proximal femur.- - - - - - - - - - ranking = 5423.864348941keywords = bisphosphonate (Clic here for more details about this article) |
4/28. Atypical familial Paget's disease of bone.We report unusual clinical and radiological features of Paget's disease of bone in three family members. All three patients had satisfactory biochemical and symptomatic response to treatment with an intravenous bisphosphonate (clodronate).- - - - - - - - - - ranking = 5423.864348941keywords = bisphosphonate (Clic here for more details about this article) |
5/28. Bisphosphonate therapy for Paget's disease in a patient with hypoparathyroidism: profound hypocalcemia, rapid response, and prolonged remission.Bisphosphonate treatment for severe Paget's disease leads to hypocalcemia followed by a secondary hyperparathyroid response to restore normocalcemia. A case is presented of a 60-year-old woman with polyostotic Paget's disease and postsurgical hypoparathyroidism. In 1993 her Paget's disease--alkaline phosphatase (ALP), 1260 U/liter (35-135 U/liter), and fasting urinary hydroxyproline excretion, 13.7 micromol/liter GF (0.4-1.9 micromol/liter)--was treated with intravenous pamidronate. Symptomatic hypocalcemia followed the first 60-mg dose, requiring large doses of calcium supplementation and calcitriol. Pamidronate therapy to a total dose of 360 mg was followed by rapid and prolonged remission with indices of bone turnover in the normal range within 2 months and persisting for at least 19 months after treatment. In 1999 relapse of Paget's disease--ALP, 511 U/liter (35-135 U/liter), and fasting urinary deoxypyridinoline/creatinine 53.1 micromol/mol (5-27 micromol/mol)--was treated with alendronate, 10 mg daily. Symptomatic hypocalcemia occurred again, requiring increased calcium and calcitriol therapy. Indices of bone turnover were within the normal range 9 weeks after the start of therapy. These responses were significantly more rapid and sustained than those observed in euparathyroid subjects. This case suggests that the lack of parathyroid response may modify the response to bisphosphonates by: (a) increasing intrinsic uptake of bisphosphonate into the pagetic skeleton, allowing response to a smaller dose; (b) increasing duration and severity of hypocalcemia after bisphosphonate therapy; and (c) removing the hyperparathyroid drive to reactivation of pagetic osteoclasts, leading to a prolonged remission. These observations have implications for optimizing bisphosphonate therapy both in Paget's disease and in osteoporosis.- - - - - - - - - - ranking = 21695.457395764keywords = bisphosphonate (Clic here for more details about this article) |
6/28. osteosarcoma arising in Paget's disease of the mandible.Although osteosarcoma is a well-known complication of Paget's disease of bone, it uncommonly develops in the jaw bones. We present an osteosarcoma arising in Paget's disease of the mandible with unique features of a normal serum alkaline phosphatase level, and histologic features of telangiectatic change in the osteosarcoma and association with cemento-osseous dysplasia. Sixteen reported cases of osteosarcoma arising in Paget's disease of the jaw bones (OPJ) are also reviewed and compared to osteosarcoma arising in Paget's disease occurring in the entire skeleton (OPS) and osteosarcoma arising de novo in the jaw bones (OJ). Females are more commonly involved in OPJ in contrast to a male predominance in OPS and OJ. OPJ also has a distinctively higher percentage involving blacks compared to OPS. The prognosis of OPJ is poor, with 69% of patients dying within two years after diagnosis. Early recognition, early and aggressive treatment are important to improve the prognosis and are hence emphasized.- - - - - - - - - - ranking = 3keywords = jaw (Clic here for more details about this article) |
7/28. osteomyelitis mimicking Paget's disease or a pagetoid phenomenon: a case report.osteomyelitis is an inflammatory infection of the bone marrow. When the maxillofacial region is involved the mandible is the preferred site in adults and the elderly. Maxillary involvement is usually seen in children with only few cases reported in adults. The disease occurs in stages, with some stages highly mimicking other diseases. Clinicians ought to be aware of the full biological course of osteomyelitis of the jaws to avoid mis-diagnosis or underdiagnosis.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |
8/28. Two cases of monostotic Paget's disease: effects of bisphosphonate.We report two cases of monostotic Paget's disease which were effectively treated with bisphosphonate. Case 1 was a 60-year-old female. Medical examination revealed high alkaline phosphatase (ALP) levels making her visit our clinic. Hematological examination showed high levels of ALP isozyme 3 and bone metabolism markers, and bone scintigraphy demonstrated strong accumulation of 99mTc on the skull. With the diagnosis of monostotic Paget's disease of the skull, treatment with bisphosphonate (etidronate) was started. The response to etidronate was good and after 12 weeks of treatment, the ALP levels decreased to about 26% of the levels before treatment, without the appearance of any symptoms or lesion development. One year and three months later, ALP increased again, and etidronate administration was resumed. However, four years after the diagnosis of the disease, etidronate became ineffective and oral administration of alendronate, a stronger bisphosphonate, was started at 5 mg/day. The patient responded favorably to the bisphosphonate and is still under observation. Case 2 was a 71-year-old female. High ALP levels were found during the follow-up of type 2 diabetes, and the case was diagnosed as monostotic Paget's disease of the pelvis based on bone metabolism markers and bone scintigraphy. Etidronate treatment at 200 mg/day resulted in the improvement of bone metabolism markers and bone scintigraphy findings. When she died of colon cancer twelve months later, with no marked progress of the Paget's disease of bone observed clinically.- - - - - - - - - - ranking = 43390.914791528keywords = bisphosphonate (Clic here for more details about this article) |
9/28. Paget disease of bone. diagnosis and indications for treatment.BACKGROUND: Paget disease was first described in 1877 by Sir James Paget. It is a focal disorder of bone remodelling, involving increased bone resorption and formation. The aetiology is uncertain but both environmental and genetic factors are thought to be involved in pathogenesis. OBJECTIVE: This article outlines the clinical presentation, diagnosis and treatment of Paget disease. DISCUSSION: Paget disease is associated with musculoskeletal pain, significant disability and impaired quality of life. Complications include pathological fracture, arthritis in adjacent joints, hearing loss, other neurological complications, heart failure and, rarely, osteosarcoma. Recent clinical trial data has demonstrated histological and radiological improvements in bone of patients treated with bisphosphonates. There is little data evaluating the long term effect of therapy on the risk of complications, however, restoration of normal bony architecture offers the prospect that complications related to deformity and increased bone fragility might be reduced by effective therapy.- - - - - - - - - - ranking = 5423.864348941keywords = bisphosphonate (Clic here for more details about this article) |
10/28. 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 = 43390.914791528keywords = bisphosphonate (Clic here for more details about this article) |
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