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Roland Chapurlat - One of the best experts on this subject based on the ideXlab platform.
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medical therapy in adults with fibrous dysplasia of bone
Journal of Bone and Mineral Research, 2006Co-Authors: Roland ChapurlatAbstract:In open studies, bisphosphonate therapy (pamidronate, alendronate) reduced bone pain associated with fibrous dysplasia of bone and was associated to some Radiological improvement. Calcium, vitamin D, and phosphorus supplements may be useful in patients with deficiency. We are awaiting results from controlled trials testing bisphosphonates. Introduction: Fibrous dysplasia of bone (FD), a rare disease caused by osteoblastic lineage differentiation defects, is associated with bone pain, fracture, and bone deformity, but few therapeutic options are available. Materials and Methods: We reviewed published data on the treatment of FD with bisphosphonates (pamidronate, alendronate), calcium, vitamin D, and phosphorus. We also present new results on FD therapy with a more potent bisphosphonate, zoledronic acid, given intravenously at the dose of 4 mg every 6 months. Results: Pamidronate therapy, given intravenously every 6 months at a dose of 180 mg in adults, relieved bone pain, decreased bone resorption, and improved the Radiological Aspect (filling of lytic lesions and/or thickening of cortices) in -50% of patients. BMD in affected sites was also significantly increased after pamidronate treatment. Those results have been obtained only in open studies, without controls, by several research groups. In a series of nine patients on long-term pamidronate treatment, but resisting to this medication and switched to intravenous zoledronic acid, no substantial improvement was observed. There is some biological rationale supporting the use of calcium and vitamin D in patients with deficiency to improve FD lesions by limiting secondary hyperparathyroidism. Phosphorus supplementation may prevent mineralization defects in those patients who have both FD and renal phosphate wasting. However, we are lacking clinical evidence for the efficacy of such supplements. Conclusions: Bisphosphonate treatment reduces increased osteoclastic activity in FD and probably improves bone pain, but their use should be better studied in randomized controlled trials.
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treatment of fibrous dysplasia of bone with intravenous pamidronate long term effectiveness and evaluation of predictors of response to treatment
Bone, 2004Co-Authors: Roland Chapurlat, P Hugueny, P D Delmas, P J MeunierAbstract:Abstract Fibrous dysplasia (FD) of bone is a rare but potentially severe bone disease that often entails fractures, deformities, and bone pain. An activating mutation of the α subunit of Gs proteins leads to differentiation abnormalities of the osteoblastic lineage, which are responsible for development of fibrous tissue in the medulla and increased osteoclastic activity. This increased bone resorption has been the rationale to use bisphosphonates in our center since 1988. So, we have analyzed the largest series, so far, of patients treated with the bisphosphonate pamidronate and sought predictors of response to treatment. We have treated 58 patients (41 adults and 17 under 18 years of age) with FD in an open study, using intravenous (IV) pamidronate 180 mg every 6 months and calcium and vitamin D supplements, in combination with oral phosphate and calcitriol in patients with FD who also had renal phosphate wasting. Patients were followed up with biannual visits, for an average 50 months, with pain assessment, annual radiographs of affected bones, measurement of biochemical markers of bone turnover, and annual bone mineral density measurements in the case of affected hips. We found that pain intensity significantly decreased with treatment in the 44 patients who had bone pain at baseline, biochemical markers of bone turnover were significantly reduced, and about 50% of patients had improvement of bone lesions on radiographs, evidenced by filling of osteolytic lesions and/or cortex thickening. Bone mineral density was substantially increased in the 12 patients who had hip FD. There was no significant clinical or biological predictor of positive radiographic response to pamidronate treatment. Long-term treatment with pamidronate was safe, in particular among the 12 patients who were followed up for more than 8 years. Despite the lack of a control group, our results suggest that intravenous pamidronate improves Radiological Aspect in half of the patients with FD, decreases bone turnover, and may decrease pain intensity.
Philippe Clapuyt - One of the best experts on this subject based on the ideXlab platform.
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Pitfalls in the diagnosis of common benign bone tumours in children
Insights into Imaging, 2014Co-Authors: Dana I. Dumitriu, Renaud Menten, Philippe ClapuytAbstract:Benign bone tumours in children are frequent lesions, often with a typical and very identifiable Radiological presentation. However, their natural evolution and complications may be the source of variations and errors in interpretation. It is therefore important to understand the possible sources of change in the Radiological Aspect and to be familiar with common pseudotumoral lesions. The main aim of this review is to review typical Aspects of the most common benign bone tumours in children, as well as less frequent variants of these tumours. Teaching points • Benign bone tumours in children may have atypical Radiological presentations. • Some normal variants are commonly misinterpreted as tumours. • X-ray is the main imaging tool for focal bone lesions. • Depending on the X-ray, complementary imaging examinations and biopsy may be necessary.
Si Hyun Kang - One of the best experts on this subject based on the ideXlab platform.
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clinical and Radiological evaluation after chemical synovectomy with rifampicin in hemophilic arthropathy korean experience with a 2 week interval protocol
Annals of Rehabilitation Medicine, 2018Co-Authors: Si Hyun KangAbstract:OBJECTIVE: To assess the clinical outcome of chemical synovectomy with rifampicin in hemophilic arthropathy by using the World Federation of Hemophilia (WFH) scoring system and plain radiograph. METHODS: We performed rifampicin synovectomy (RS) on 30 joints of 28 hemophilic patients diagnosed as hemophilic arthropathy stage I-III (based on Fernandez-Palazzi clinical classification). Clinical status (bleeding frequency, pain, joint physical status) and Radiological staging were evaluated as parts of the WFH scoring system before and 1 year after RS. The patients were divided into two groups by the Arnold-Hilgartner scale of the initial X-ray as stage 3 or less for the low-stage group (n=17) and over 3 for the high-stage group (n=13). RESULTS: Total WFH joint physical scores were reduced after injection, and the number of bleeding episodes and pain showed especially significant improvement. For other subscores of the WFH joint physical score, only swelling, range of motion, and crepitus showed statistically significant improvement. According to the severity of the radiologic finding, the WFH joint physical score of both the low-stage and high-stage groups showed significant improvement. In the Radiological Aspect, the low-stage group, without joint space narrowing at the initial plain radiograph, showed no further aggravation after injection. However, in the high-stage group, radiology found aggravation regardless of the procedure. CONCLUSION: It is suggested that chemical synovectomy with rifampicin may prevent hemarthrosis and improve clinical symptoms. Especially in the early stage of arthropathy without joint-space narrowing, it seems to have an additional benefit that delays Radiological aggravation and preserves joint status.
François Bertucci - One of the best experts on this subject based on the ideXlab platform.
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high grade soft tissue sarcoma arising in a desmoid tumor case report and review of the literature
Clinical sarcoma research, 2015Co-Authors: Marjorie Faure, Maria Rosa Ghigna, Laurence Moureauzabotto, Bruno Chetaille, Jérôme Guiramand, Anthony Sarran, François Bertucci, Delphine PerrotAbstract:Desmoid tumors are rare benign monoclonal fibroblastic tumors. Their aggressiveness is local with no potential for metastasis or dedifferentiation. Here we report on a 61-year-old patient who presented a locally advanced breast desmoid tumor diagnosed 20 years after post-operative radiotherapy for breast carcinoma. After 2 years of medical treatment, a high-grade undifferentiated pleomorphic soft tissue sarcoma arose within the desmoid tumor. Despite extensive surgery removing both tumors, the patient showed locoregional relapse by the sarcoma, followed by multimetastatic progression, then death 25 months after the surgery. The arising of a soft tissue sarcoma in a desmoid tumor is an exceptional event since our case is the fourth one reported so far in literature. It reinforces the need for timely and accurate diagnosis when a new mass develops in the region of a preexisting desmoid tumor, and more generally when a desmoid tumor modifies its clinical or Radiological Aspect.
Avneesh Chhabra - One of the best experts on this subject based on the ideXlab platform.
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“Periosteum: An imaging review”
European Journal of Radiology Open, 2020Co-Authors: Carlos Henrique Maia Ferreira Alencar, Cláudio Régis Sampaio Silveira, Matheus Martins Cavalcante, Clarissa Gadelha Maia Vieira, Manoel Joaquim Diógenes Teixeira, Francisco Andrade Neto, Armando De Abreu, Avneesh ChhabraAbstract:Abstract Periosteum is a fibrous sheath, coating the external bone, except in the articular surfaces, tendon insertions and sesamoid bone surface¹. It changes its Aspects and characteristics with aging, becoming progressively less elastic and more firm. It is composed of two different layers: outer fibrous (firm, collagen-filled) and inner proliferative (cambium, containing osteoprogenitor cells)². Four vascular systems are responsible for the blood supply of the periosteum: the intrinsic periosteal system, located between fibrous and proliferative layer; the periosteocortical, the main nutritional arteries of the periosteum; the musculoperiosteal, responsible for the callus formation after fractures; the fascioperiosteal, specifically for each bone.³ It is crucial to bone formation and resorption, reacting to insults in the cortical bone, such as tumors, infections, traumas, medications and arthritic diseases. The aggressiveness of the reaction can be suggested by its Radiological Aspect and appearance4. The periosteum in children is looser compared to adults, resulting in earlier and more exuberant reactions. All these Aspects will be detailed, so the essential information all radiologists need to know will be discussed.