Fibrous dysplasia of occipital bone revealed by acute intracranial hypertension.

Fibrous dysplasia of bone is a stem cell bone disease due to a somatic GNAS mutation which can affect craniofacial bones. Although craniofacial fibrous dysplasia is a benign and progressive disorder, it can cause mass effect on the cranial structures. We describe an 18-year-old man, without past medical history, came at the emergency department with progressively worsening headache, associated with vomiting. Cranial bone CT and then brain MRI revealed fibrous dysplasia of occipital bone with intraosseous cyst, compression of right sigmoid vein. An angiography was performed to stent the right sigmoid vein and symptoms had completely resolved only a few hours after the procedure. Then, a treatment by bisphosphonates was introduced. We believe this is the first description of sigmoid vein compression by a bone cyst, requiring stenting. MRI should be performed urgently in case of unusual severe headache or rapidly evolving neurologic impairment in patients with craniofacial fibrous dysplasia. Treatment of fibrous dysplasia is a controversial subject. In cases with neurologic complications, surgery or endovascular treatment might be performed.

[Pharmaceutical interviews for rheumatoid arthritis patients: Perceptions and expectations of community pharmacists]. / Entretiens pharmaceutiques destinés aux patients atteints de polyarthrite rhumatoïde : perceptions et attentes des pharmaciens d'officine.

INTRODUCTION: Rheumatoid arthritis has a low level of medication adherence. Abroad, the community pharmacist has a positive impact on the patients' adherence in several chronic diseases. In France, community pharmacists' missions are developing with the implementation of pharmaceutical interviews. OBJECTIVE: To evaluate community pharmacists' perceptions on the interest and feasibility of pharmaceutical interviews targeting patients with rheumatoid arthritis. METHOD: Semi-structured interviews were conducted between August and October 2017, with pharmacists in the Auvergne-Rhône-Alpes region. The inductive analysis of the interview verbatim was realized by two independent persons. RESULTS: Fifteen community pharmacists highlighted barriers in recruiting patients for the interviews currently possible at the pharmacy, the complexity of the organization and the financing, a weakness of the hospital-to-community liaison. Nevertheless pharmacists were motivated to expand the service to other pathologies. Regarding rheumatoid arthritis, pharmacists would see them in the form of structured interviews preferentially at the pharmacy, in connection or even "prescribed" by physicians for optimal and multi-professional information sharing. Prior training and funding for these interviews should be considered to motivate pharmacists to this activity. CONCLUSION: This study allowed to discuss with community pharmacists their expectations and needs to widen the service of pharmaceutical interviews in the rheumatoid arthritis. These results will have to be taken into account to build a support interviews model for rheumatoid arthritis patients who can be integrated in their daily pharmaceutical activity.

Post-fracture care: do we need to educate patients rather than doctors? The PREVOST randomized controlled trial.

We conducted a multicenter, randomized controlled trial to evaluate the impact of a population-based patient-centered post-fracture care program with a dedicated case manager, PREVention of OSTeoporosis (PREVOST), on appropriate post-fracture osteoporosis management. We showed that, compared to usual care, BMD investigation post-fracture was significantly improved (+20%) by our intervention program. INTRODUCTION: Our study aims to evaluate the impact of a population-based patient-centered post-fracture care program, PREVOST, on appropriate post-fracture care. METHODS: Multicenter, randomized controlled trial enrolling 436 women aged 50 to 85 years and attending a French hospital, for a low-energy fracture of the wrist or humerus. Randomization was stratified by age, hospital department, and site of fracture. The intervention was performed by a trained case manager who interacted only with the patients, with repeated oral and written information about fragility fractures and osteoporosis management, and prompting them to visit their primary care physicians. Control group received usual care. The primary outcome was the initiation of an appropriate post-fracture care defined by Bone Mineral Density (BMD) and/or anti-osteoporotic treatment prescription at 6 months. RESULTS: At 6 months, 53% of women in intervention group initiated a post-fracture care versus 33% for usual care (adjOR 2.35, 95%CI [1.58-3.50], p < 0.001). Post-fracture care was more frequent after wrist than humerus fracture (adjOR 1.93, 95%CI [1.14-3.30], p = 0.015) and decreased with age (adjOR for 10 years increase 0.76, 95%CI [0.61-0.96], p = 0.02). The intervention resulted in BMD prescription in 50% of patients (adjOR 2.10, 95%CI [1.41-3.11], p < 0.001) and in BMD performance in 41% of patients (adjOR 2.12, 95%CI [1.40-3.20], p < 0.001) versus 33 and 25% for usual care, respectively. Having performed a BMD increased treatment prescription; however, only 46% of women with a low BMD requiring a treatment according to the French guidelines received a prescription. CONCLUSION: A patient-centered care program with a dedicated case manager can significantly improve post-fracture BMD investigation.

Treatment of postmenopausal women with osteoporosis or low bone density with raloxifene. Raloxifene Study Group.

Raloxifene, a selective estrogen receptor modulator (SERM), has been shown to improved bone mineral density (BMD) and serum lipid profiles in healthy postmenopausal women. The objective of this study was to examine the effects of raloxifene on BMD, biochemical markers of bone metabolism and serum lipids in postmenopausal women with low bone density or osteoporosis. This Phase II, multicenter, 24-month, double-masked study assessed the efficacy and safety of raloxifene in 129 postmenopausal women (mean age +/- SD: 60.2 +/- 6.7 years) with osteoporosis or low bone density (baseline mean lumbar spine BMD T-score: -2.8). Women were randomly assigned to one of three treatment groups: placebo, 60 mg/day raloxifene-HCl (RLX 60) or 150 mg/day raloxifene-HCL (RLX 150) and concomitantly received 1000 mg/day calcium and 300 U/day vitamin D3. At 24 months, BMD was significantly increased in the lumbar spine (+3.2%), femoral neck (+2.1%), trochanter (+2.7%) and total hip (+1.6%) in the RLX 60 group compared with the placebo group (p < 0.05). The RLX 150 group had increases in BMD similar to those observed with RLX 60. A greater percentage of raloxifene-treated patients, compared with those receiving placebo, had increased BMD (p < 0.05). Serum bone-specific alkaline phosphatase activity, serum osteocalcin, and urinary type I collagen:creatinine ratio were significantly decreased in the RLX-treated groups, compared with the placebo group (p < 0.01). RLX 60 treatment significantly decreased serum levels of triglycerides, and total- and LDL-cholesterol levels (p < 0.01). The rates of patient discontinuation and adverse events were not significantly different among groups. In this study, raloxifene increased bone density, decreased bone turnover, and improved the serum lipid profile with minimal adverse events, and may be a safe and effective treatment for postmenopausal women with osteoporosis or low bone density.

[Effects of raloxifene on bone loss and fracture risk in the menopausal woman]. / Effets du raloxifène sur la perte osseuse et le risque fracturaire chez la femme ménopausée.

The relevance and efficacy of long-term estrogen therapy is well established, though some undesirable side effects and contraindications persist. Raloxifene, the first selective estrogen receptive modulator (SERM) tested in phase III trials, offers a choice alternative. It increases bone mineral density, lowers serum lipid concentrations and reduces vertebral fractures.

Decreased beta-isomerization of the C-terminal telopeptide of type I collagen alpha 1 chain in Paget's disease of bone.

In Paget's disease of bone, the normal lamellar bone is replaced by a woven structure with an irregular arrangement of collagen fibers. In this study, we investigated whether the degree of beta-isomerization within C-telopeptide of alpha 1 chain of type I collagen was altered in Paget's disease compared with other bone diseases with no alteration of bone structure. In Paget's disease (n = 26), but not in patients with primary hyperparathyroidism (n = 6) or hyperthyroidism (n = 17), the urinary excretion of nonisomerized (alpha) fragments derived from degradation of type I collagen C-telopeptide (CTX) was markedly increased compared with beta-isomerized CTX (+ 13-fold vs. + 3.5-fold over controls) resulting in an urinary alpha CTX/beta CTX ratio 3-fold higher than in controls (2.6 +/- 1.0 vs. 0.8 +/- 0.3, p < 0.001). In five pagetic patients in complete remission, as demonstrated by normal total alkaline phosphatase activity, the alpha CTX/beta CTX ratio was normal. The immunohistochemistry of normal and pagetic human bone sections showed a preferential distribution of alpha CTX within woven structure, while lamellar bone was intensely stained with an anti-beta CTX antibody, suggesting a lower degree of beta-isomerization of type I collagen in the woven pagetic bone. In collagenase digest of human bone specimens, we found a lower proportion of beta-isomerized type I collagen molecules in pagetic bone (40% of beta CTX) than in normal bone taken from trabecular (68%) and cortical compartments (71%). In conclusion, we found that in Paget's disease the alpha CTX/beta CTX ratio in bone and in urine is markedly increased. This altered beta isomerization can be accurately detected in vivo by measuring urinary degradation products arising from bone resorption.

Therapeutic strategy in Paget's disease of bone.

Paget's disease of bone is a localized progressive disease, characterized by increased bone remodeling, bone hypertrophy, and abnormal bone structure, leading to pain and deformity. Disease complications involve the bones, joints, and central nervous system. Short-term treatment objectives are to alleviate or suppress bone pain; the long-term aim is for the prevention of complications caused by the slow disease progression in affected bones. The lifetime risk of complications depends on life expectancy, location, and activity index of Paget's disease. The use of potent and safe new bisphosphonates (tiludronate, pamidronate, alendronate, risedronate, and others) represents a major advance for the management of the condition: these compounds permit normalization of the biochemical indices of remodeling, and clinical doses have lasting effects without disruption to bone mineralization. Bone histomorphometry has shown that bisphosphonates can restore normal bone structure and an optimal therapeutic strategy should be defined. Symptomatic patients (or asymptomatic patients at risk) should be treated, as well as any patient requiring orthopedic surgery. The optimum strategy for preventing complications is to treat as early as possible and to halt disease progression by normalizing the biochemical markers that reflect the increased bone remodeling. There is no consensus on the threshold values for retreatment. Few studies have evaluated the long-term effects of the inhibitors of osteoclastic resorption on the risk of complications. A recent 12 year, long-term follow-up of 41 cases of Paget's disease has shown that antipagetic therapy that did not normalize biochemical markers in 71% of patients did not prevent new complications in 62% of patients. These results suggest that an optimal regimen of treatment and retreatment should target normalization of biochemical markers of bone remodeling; the new bisphosphonates make this quite feasible in most patients.

[Vertebral osteosclerosis, sacro-iliitis and palmoplantar dermatitis. Apropos of a case]. / Condensation vertébrale, sacro-iléite et dermatose palmo-plantaire. A propos d'une observation.

The authors report a case of aseptic vertebral osteitis which they classify in the context of SAPHO in view of the association with unilateral sacro-ileitis and palmoplantar dermatitis. They stress the importance of recognizing this disease which is initially suggestive of either a bone disease (Paget's disease, bone metastasis) or infectious discospondylitis.