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1.
Dr. P. D. Delmas B. Demiaux L. Malaval M. C. Chapuy P. J. Meunier 《Calcified tissue international》1986,38(1):60-61
Summary Serum bone Gla-protein (sBGP) was measured in 32 patients with untreated Paget's disease of bone. Despite clinical and biological
symptoms of active disease in all patients, sBGP was normal in 13/32 patients (41%). There was a striking discrepancy between
the moderate increase of sBGP above normal values (11.4±4.5 vs 6.0±2.1 ng/ml) and the marked increase of both serum alkaline
phosphatase (sAP) and urinary hydroxyproline (uOHP). sBGP was weakly correlated with sAP (r=0.50, p<0.01), uOHP (r=0.48, p<0.01)
and with the extension of the disease (r=0.48, p<0.01), We conclude that sBGP is not a sensitive marker of bone turnover in
patients with Paget's disease of bone, and should be interpreted with caution in this condition. 相似文献
2.
Heterotopic bone formation in soft tissues occurs commonly in Paget's disease patients following a primary total hip arthroplasty (THA). The nature of this heterotopic bone has not been documented. In this report, we show that the heterotopic bone removed 14 years after primary THA in a case of Paget's disease was sclerotic, contained prominent mosaic cement lines and showed increased remodelling activity on the bone surface. In addition to these typically Pagetic histological features, it was noted ultrastructurally that the osteoclasts contained characteristic intranuclear viral-like inclusions. In contrast, the foreign body macrophages found in the joint pseudocapsule and pseudomembrane, which are a population of mononuclear precursor cells from which osteoclasts can be formed, did not contain viral-like inclusions. These findings are of interest regarding the pathogenesis of heterotopic bone formation following hip arthroplasty and the ontogeny of Pagetic osteoclasts. 相似文献
3.
目的 观察高转换型肾性骨病中骨保护素及其配体 (OPG,RANKL)的表达,并与骨形态计量学指标进行相关分析。 方法 选择10例慢性肾衰尿毒症患者和3例正常人进行髂骨活检术,获得骨组织标本。采用免疫组化方法检测OPG和RANKL蛋白质的表达。采用全自动图像分析系统进行骨组织形态计量学测定。结果 10例慢性肾衰尿毒症患者经骨病理学检查证实均为高转换型骨病,以破骨细胞活化形成骨吸收陷窝伴或不伴骨矿化不全为特点。免疫组化显示尿毒症患者骨组织中以RANKL阳性表达为主。与正常对照相比,RANKL阳性表达细胞数目显著增加,OPG阳性表达细胞数目显著减少。尿毒症患者RANKL的阳性表达细胞数目与骨吸收面积和破骨细胞数目呈显著正相关。结论 高转换型肾性骨病中,PTH的溶骨作用可能是通过OPG/RANKL/RANK系统介导的。 相似文献
4.
Summary We report a 67-year-old man who presented with a 3-month history of progressively increasing pain in the lumbar spine. His past medical history was unremarkable, and physical examination disclosed local tenderness over the lower spine. No neurologic dysfunction was identified. Routine laboratory evaluation including alkaline phosphatase activity was normal. An X-ray film of the lumbar spine showed enlargement and increased density of L-5 vertebra. A wholebody bone scan revealed markedly increased uptake at the L-5 level. To further evaluate the nature of the disorder and the cause of his pain, a computed tomography (CT) scan was obtained. It disclosed multiple lucent areas with some sclerotic changes mainly affecting the vertebral body of L-5. No spinal stenosis was found. Subsequently, a bone biopsy of L-5 was performed that showed typical findings consistent with Paget's disease. The patient was treated with etidronate (200 mg b.i.d. for 6 months) followed by salmon calcitonin (50 IU 3 times/week s.c. for 6 months). The pain declined gradually in severity and the patient became symptom free after 12 months of treatment. A repeat X-ray film, obtained at that time, showed no significant change. However, a bone scan showed almost complete normalization. The present case illustrates that a high index of suspicion is required when only a single vertebra is affected by Paget's disease, especially, when alkaline phosphatase activity is normal. It may present with severe pain without evidence of neurologic dysfunction. CT scan may be a useful adjunct in establishing the diagnosis and elucidating the cause of pain. 相似文献
5.
Takata S Hashimoto J Nakatsuka K Yoshimura N Yoh K Ohno I Yabe H Abe S Fukunaga M Terada M Zamma M Ralston SH Morii H Yoshikawa H 《Journal of bone and mineral metabolism》2006,24(5):359-367
We here propose guidelines for the diagnosis and management of Paget's disease of bone (PDB) in Japan. These guidelines provide
basic information on the epidemiology, pathophysiology, clinical signs and symptoms, diagnosis, indications for treatment,
and available therapy, including orthopedic surgery. PDB is a chronic disorder characterized by focal abnormalities of bone
turnover. The characteristic feature of PDB is excessive osteoclastic bone resorption coupled to increased and disorganized
bone formation. The most common symptom of PDB is pain in involved bones. The most serious complication of PDB is malignant
bone or soft-tissue tumor. PDB is uncommon in Japan; our survey in 2003 found 169 patients with PDB. The prevalence of PDB
in Japan is 0.15/100 000; in patients aged 55 years or more, the proportion reaches 0.41/100 000. A careful medical history
and physical examination are essential for the diagnosis. The diagnosis of PDB is based on finding the typical features on
radiographs. Bone scintigraphy and measurement of serum alkaline phosphatase are sensitive means of screening for PDB. Since
PDB is a rare disease in Japan, bone biopsy is quite often used to exclude bone metastases. The only evidence-based indication
for treatment of PDB is pain in involved bones. In Japan, etidronate and calcitonin are approved by the Ministry of Health,
Labour and Welfare for treating PDB, but currently risedronate is also under development for treating PDB in Japan. Indications
for surgical intervention in PDB include unstable fractures, osteoarthritis, malignant soft-tissue tumor, osteosarcoma, and
bone deformity. 相似文献
6.
Frederick R. Singer 《Calcified tissue international》1991,49(Z2):S7-S8
Clinical interest in salmon calcitonin began in 1972 when this peptide was shown to be effective in the treatment of Paget's
disease. Salmon calcitonin is more potent than porcine calcitonin, with human calcitonin intermediate in potency. Salmon calcitonin
is a highly effective therapeutic agent in the treatment of Paget's disease. During chronic treatment with salmon calcitonin,
alkaline phosphatase activity and urinary hydroxyproline excretion decrease on an average of 50% in patients with Paget's
disease. Patients may experience a variety of clinical benefits during chronic treatment, including relief of bone pain, a
reversal of neurological deficits, stabilization or improvement of hearing loss, and improvement of vascularity of bone. Radiologic
healing of osteolytic lesions is particularly striking with calcitonin treatment. Paget's disease patients prefer treatment
with salmon calcitonin administered by means of a nasal spray. Salmon calcitonin has an excellent safety profile and produces
mild side effects in a small percentage of patients. The most common side effects associated with salmon calcitonin administration
are nausea and facial flushing. It is unusual to observe severe side effects. In about 20% of patients, production of antibodies
may neutralize the effects of the exogenously administered calcitonin; these patients respond to human calcitonin. At this
time salmon calcitonin should still be considered a valuable therapeutic agent in the treatment of Paget's disease, particularly
in patients with osteolytic lesions. 相似文献
7.
Hashimoto J Ohno I Nakatsuka K Yoshimura N Takata S Zamma M Yabe H Abe S Terada M Yoh K Fukunaga M Cooper C Morii H Yoshikawa H;Japanese Committee on Clinical Guidelines of Diagnosis Treatment of Paget's Disease of Bone of the Japan Osteoporosis Society 《Journal of bone and mineral metabolism》2006,24(3):186-190
The present study aimed to evaluate the prevalence and clinical presentation of Paget's disease of bone (PDB) in Japan. As
PDB is a very rare disease in Japan, a nationwide mail survey was conducted targeting doctors in the specialty most frequently
diagnosing and treating PDB patients in Japan. First, the literature for all case reports in Japan published between January
1990 and December 2002 was reviewed to determine who was diagnosing and treating PDB in Japan. This literature review for
all case reports in Japan revealed that 72.1% of cases in Japan were reported from departments of orthopedic surgery. A nationwide
two-phase mail survey was conducted for the departments of orthopedic surgery of 2320 general hospitals accredited by the
Japanese Orthopaedic Association. Phase 1 involved determining how many patients with PDB were followed at each hospital.
If the answer was one or more, phase 2 of the survey gathered information on the clinical presentation of current patients.
The mail survey yielded a final response rate of 75.4% for phase 1 and 87.6% for phase 2. Phase 1 indicated that the prevalence
of PDB in Japan is about 2.8 cases per million capita. Phase 2 revealed a slight female predominance, lower frequency of familial
clustering, higher frequency of femoral fracture in the affected femur, and a higher ratio of symptomatic PDB in Japan compared
with findings in countries displaying a higher prevalence of PDB. The present epidemiological study revealed that the disorder
is extremely rare in Japanese individuals, and that some differences exist with regard to the clinical features of PDB between
Japanese patients and patients from high-prevalence countries. 相似文献
8.
A. Rapado M. Yague M. Diaz Curiel F. Ortiz P. Palomino C. de la Piedra R. Torres B. Peramo E. Lopez Gavilanes 《Calcified tissue international》1991,49(6):436-437
Summary We have found a cellular immune defect (low CD4 T lymphocyte count, high CD8 T lymphocyte count and a low CD4/CD8 ratio) in
39 PDB patients. This finding is not correlated with the bone metabolic activity of the disease. There was an improvement
in the cellular immune defect in fifteen patients after treatment with elcatonin. These changes could be related to the improvement
in the metabolic derangement or else could be the consequence of an effect on altered immunity. 相似文献
9.
This study assessed the efficacy of 200 mg of aminohexane bisphosphonate (neridronate) administered by intravenous infusion in a single dose or in two separate doses on consecutive days in 32 patients (16 males and 16 females, average age 66 years) affected by active Paget’s disease of bone. Fifteen patients had never been treated with any antiresorptive agent and 17 had had unsatisfactory results from a prior clodronate treatment. All of the latter patients had failed to enter a remission stage (i.e., normalization of bone turnover was not reported at any time during treatment) and had had a full relapse within 6 months after clodronate infusion. In the present study bone-specific alkaline phosphatase (bAp), deoxypyridinoline (dPyr), and N- and C-terminal polypeptide of collagen type 1 (Ntx, Ctx) were determined before neridronate administration and at 1, 3, 6, and 12 months thereafter. Basal values of bAp were 51.7 ± 2.3 μg/L, range 31.7–92.5 (normal range 6.2–23.6). No statistical differences in markers of bone turnover were evident in the basal state between new pagetic patients (bAp = 55.1 ± 4.1) and those suffering a relapse after clodronate (bAp = 48.8 ± 2.6). Neridronate induced an average percent change from baseline in excess bAp of 68.0 ± 4.3 and in excess dPyr, Ntx, and Ctx of 68.1 ± 11, 60.6 ± 8.5, and 86.7 ± 7.8, respectively. Markers of bone resorption declined more slowly in patients treated previously with clodronate, although the average change in percent decrement from baseline in excess bAp as well as in excess of bone resorption markers was not different from that registered in untreated pagetic patients. Response to treatment, defined as a percent decrement from baseline in excess bAp of 50% or more at any time during the 12-month follow-up, was observed in 27 patients (84.4%). Remission (a drop in bAp to within normal range) was achieved in 21 patients (65.6%) and was maintained in 12 at 12-month follow-up, with no significant differences between either 1- or 2-day infusions, or between new pagetic patients and those relapsing after clodronate. In 15 of 21 patients requiring analgesics to alleviate bone pain, pain was reduced or completely alleviated in 8. A slight, short-lived acute phase reaction (fever and/or arthromyalgia) occurred in 6 patients. To summarize, 200 mg of intravenous neridronate, in one or two doses, significantly reduced the biochemical indices of disease activity in the majority of patients, showing a normalization of bAp in more than 60%. We conclude that neridronate can be used safely in the treatment of patients with Paget’s disease of bone either as a first bisphosphonate treatment or as retreatment for patients relapsing after clodronate. 相似文献
10.
11.
T. J. Spinks G. F. Joplin I. M. A. Evans J. Pennock F. H. Doyle A. S. O. Ranicar 《Calcified tissue international》1982,34(1):459-464
Summary Total body calcium (an index of skeletal mass), ulnar bone density, and total body potassium (lean body mass) were followed
for up to 5 years in 38 patients with Paget's disease during treatment with synthetic human calcitonin. Calcium was measured
by neutron activation, ulnar density by X-ray photodensitometry, and potassium by counting its natural40K radioactivity.
There was a significant rise in total skeletal mass in a group of 8 patients in the 12 months following the start of therapy,
but overall, total bone mass and ulnar density remained constant during treatment. Small mean losses of body potassium were
observed (∼4%) after several years elapsed on treatment. Over an average period of 12 months after discontinuation of therapy
in 21 patients there was no significant mean change in calcium or potassium. The means of the ratios of total body calcium
divided by predicted normal calcium (over the whole period of measurement) were 1.08 (males) and 0.99 (females) and were not
significantly different. Comparison of the ulnar densities of patients and normal subjects gave similar results. The average
ratio of measured to predicted normal potassium was 1.13 (both males and females).
Thus there was no indication of depletion in skeletal mass below normal either in the untreated disease or resulting from
treatment. 相似文献
12.
Treatment of Paget's disease of bone with intravenous 4-amino-1-hydroxybutylidene-1,1-bisphosphonate
S. Adami G. Salvagno G. Guarrera F. Montesanti S. Garavelli S. Rosini V. Lo Cascio 《Calcified tissue international》1986,39(4):226-229
Summary 4-amino-1-hydroxybutylidene-1,1-bis-phosphonate (AHButBP) was given intravenously (2.5–25 mg/day for 4 days) to 14 patients
with Paget's disease of bone, five of whom had been treated with dichloromethylidene bisphosphonate (Cl2MBP) 32 months earlier. In the nine patients who had not been treated previously with bisphosphonates, the short course of
AHButBP induced a suppression of serum alkaline phosphatase and urinary hydroxyproline values down to 30% of initial values.
The biochemical suppression of the disease was sustained for 2–18 months and the time to relapse did correlate to the logarithm
of the dose (P<0.001). In the five patients previously treated for Paget's disease, an apparent resistence to treatment with AHButBP was
observed. However, in these patients both serum alkaline phosphatase and urinary hydroxyproline fell to or even below the
nadir values which had previously been achieved with Cl2MBP, irrespective of the degree of relapse. Thus the degree of suppression of Paget's disease of bone, achievable after treatment
with bisphosphonates, seems to be constant for each patient, such that normal levels of serum alkaline phosphatase and urinary
hydroxyproline cannot usually be attained in patients with extremely active disease. 相似文献
13.
Summary Forty-five patients with Paget's disease were studied, of whom 25 had fissure fractures affecting the bones of the lower limb. Ten patients treated with salmon calcitonin showed no improvement in their fissure fractures despite reduction in bone turnover. Treatment was effective where bone deformity was improved by traction or intra-medullary nail fixation combined with osteotomy.Surgical treatment should be undertaken in those patients who complain of acute pain at the site of a fissure fracture and who risk completing the fracture. Fissure fractures involving the femoral neck should be fixed even when asymptomatic because of the risk of non-union if they become complete.
Résumé Les auteurs ont étudié 45 malades atteints de maladie de Paget parmi lesquels 25 présentaient des fissures au niveau des os des membres inférieurs. Dix malades traités par la calcitonine ne montrèrent aucune amélioration de ces fissures malgré la diminution du turn-over osseux. Le traitement chirurgical s'est montré efficace quand la déformation de l'os a été corrigée par traction ou par enclouage centromédullaire après ostéotomie.Le traitement chirurgical est indiqué chez les malades qui se plaignent de vives douleurs au niveau de leurs fissures et qui présentent le risque que leurs fractures se complètent, même en l'absence de traumatisme. Les fissures du col du fémur doivent être fixées, même si elles sont asymptomatiques, en raison du risque de pseudarthrose si elles se complètent.相似文献
14.
J. Y. Reginster A. M. Jeugmans-Huynen A. Albert D. Denis R. Deroisy M. P. Lecart M. A. Fontaine J. Collette P. Franchimont 《BONE》1988,9(6):349-354
Several Biophosphonates have been used as therapeutic agents for Paget's bone disease. (Chloro-4 phenyl)thiomethylene-bisphosphonate (CIPsMBP) has recently been shown to have significant antiosteoclastic activity while an affect of CIPsMBP on mineralization was only observed at high doses. We tested this drug for 6 months in 23 pagetic patients distributed in three groups. Gr 1 (n = 5) receiving 200 mg/day showed a decrease of serum alkaline phosphatase (SAP) to 42 ± 4% (p < 0.01) of initial value (100%) while hydroxyprolinuria/creatinuria ratio (OH/Cr) dropped to 69 ± 8% of baseline. In 4 patients receiving 400 mg/day, SAP improved to 48 ± 9% of initial value (p < 0.01) and OH/Cr to 40 ± 3% (p < 0.01). In the last group (n = 14) receiving 200 mg/day for 3 months, and 400 mg/day thereafter up to the 6th month SAP decreased to 53 ± 4% and OH/Cr to 62 ± 6% of initial value (p < 0.01).
Clinical improvement was significant from the first month of treatment. No resistance (mean decrease of SAP lower than 30%) was recorded and no radiological or clinical evidence of mineralization defect appeared. The clinical and biological tolerance was excellent throughout the study. 相似文献
15.
Usefulness of biochemical markers of bone turnover in assessing response to the treatment of Paget's disease. 总被引:3,自引:0,他引:3
L Alvarez N Gua?abens P Peris S Vidal I Ros A Monegal J L Bedini R Deulofeu F Pons J Mu?oz-Gomez A M Ballesta 《BONE》2001,29(5):447-452
The aim of this study was to investigate the usefulness of biochemical markers of bone turnover for monitoring treatment efficacy of Paget's disease of bone, and also to evaluate the utility of biological variation data in choosing the best markers for assessment of biochemical response to therapy. Thirty-eight patients with Paget's disease were included in a prospective study. All received 400 mg/day of oral tiludronate for 3 months. In 31 patients that completed treatment, biochemical markers were measured at baseline and at 1 and 6 months after treatment ended. In serum we determined the levels of total alkaline phosphatase (tAP), bone alkaline phosphatase (bAP), procollagen type I N-terminal propeptide (PINP), and C-terminal telopeptide of type I collagen (sCTx). Urine samples were analyzed for hydroxyproline (Hyp) and for C- and N-terminal telopeptides of type I collagen (CTx and NTx, respectively). Quantitative bone scintigraphy was performed at baseline and at 6 months after discontinuation of therapy. A ratio for monitoring response to treatment was obtained for each marker. This ratio reflected the size of treatment response of the marker in relation to the value of its critical difference. Thus, ratio values of >1 indicated a significant decrease of the marker after therapy. In addition, response to therapy was evaluated according to disease activity. Mean values of all markers of bone turnover decreased significantly after therapy. Serum bAP and PINP and urinary NTx showed the highest percentage reduction (between 58% and 68%). Furthermore, serum bAP and PINP showed the highest ratios for monitoring changes induced by treatment, followed by serum tAP and urinary NTx. sCTx and urinary CTx as well as Hyp showed mean ratios for monitoring changes of <1, indicating a low sensitivity for monitoring treatment. Patients with polyostotic disease showed a continuous decrease in mean values for all markers at 6 months from the end of therapy, whereas, in monostotic patients, there was a trend toward increased levels at this timepoint. In conclusion, serum bAP and PINP were the most sensitive markers for monitoring treatment efficacy in Paget's disease, although serum tAP and urinary NTx were also sensitive markers for monitoring changes. Data on biological variation are useful for assessing actual changes induced by treatment. 相似文献
16.
Paget's disease is a common skeletal disorder in the elderly; however, monostotic Paget's disease of the patella is very rare and only a few cases have been reported. We present the clinical, radiographic, surgical and histological findings in a patient with monostotic Paget's disease of the patella, presenting with knee pain. 相似文献
17.
The urinary excretion of glucosyl-galactosyl-hydroxylysine and of galactosyl-hydroxylysine were studied in Paget's disease of bone and in primary hyperparathyroidism. Both metabolites were increased in these diseases.Although glucosyl-galactosyl-hydroxylysine is not abundant in bone collagen, it is possible that a part of it originates from calcified tissue. 相似文献
18.
Elina Siponen Katja Hukkinen Päivi Heikkilä Heikki Joensuu Marjut Leidenius 《American journal of surgery》2010,200(2):241-256
Background
The aim of this study was to evaluate the outcomes of surgical treatment of Paget's disease of the breast, with special emphasis on magnetic resonance imaging (MRI) and sentinel node biopsy (SNB).Methods
The study included 58 consecutive patients with Paget's disease treated from 1995 to 2006.Results
Twenty-five patients had ductal carcinoma in situ, and 31 had invasive carcinoma. MRI was performed in 14 patients, with positive findings in 7 patients, 5 of whom had negative findings on conventional imaging. The overall mastectomy rate was 76%. Eighteen patients underwent SNB, and 26 patients underwent full or partial axillary clearance. Fourteen patients had no axillary surgery. One patient had local recurrence after breast conservation, and another had axillary recurrence after negative results on SNB. Six patients had distant metastases. Four patients died of breast cancer.Conclusions
Paget's disease is frequently associated with peripheral or multicentric cancer. MRI may be helpful when considering breast conservation or omitting axillary nodal staging. 相似文献19.
Extramammary Paget's disease of the perianal region 总被引:3,自引:0,他引:3
Objective Perianal Paget's disease (PPD) is a rare entity. The standard treatment for either in situ or invasive extra mammary Paget's disease (EMPD) is surgical excision. Local recurrence and morbidity from surgery, especially in the elderly, can, however, be high. The aim of this article is to review our experience with PPD and question the currently preferred treatment approaches in light of its histopathology and therapeutic outcome. Patients and methods A chart review of our patients with PPD from 1996 to 2002 was carried out to determine their outcome after treatment. Data from review of the literature are presented. Results Five patients with in situ disease (four females, median age 68 years) were diagnosed as having PPD. A complete surgical excision was attempted in 4 patients and the fifth was treated by photodynamic therapy. At present, all patients are alive, two are free of disease, one has persistent disease and two have local recurrence. Conclusion Considering the significant rate of recurrence even after wide local excision, the extent of surgery needed and the good prognosis with long‐term survival, we question whether nonsurgical modalities should be considered in place of surgery as primary treatment for noninvasive PPD, with radical surgery being reserved for failures or invasive disease. 相似文献
20.
《Joint, bone, spine : revue du rhumatisme》2019,86(3):373-380
ObjectivesThis study explored the role of outdoor and indoor air pollutants in Paget's disease of bone (PDB).MethodsWe performed a survey in 140 French-Canadian patients with PDB, including 39 carriers of p.Pro392Leu mutation (SQSTM1 gene) and 113 healthy not mutated controls. The survey covered outdoor air pollution near the residence and indoor air pollutants by focusing on heating fuels and exposure to tobacco smoke. In a subgroup of patients, urinary concentrations of 17 heavy metals and 11 polycyclic aromatic hydrocarbons were measured by mass spectrometry. In light of what we learned from the survey and urinary assays, we explored the in vitro effects of certain toxics on osteoclasts in PDB. We conducted in vitro monocytes differentiation from peripheral blood of more than 40 participants, whose osteoclasts were treated with or without the toxic. The morphology of osteoclasts, their bone resorption abilities, gene and protein expression levels, and cellular oxidative stress levels were assayed.ResultsAn inhibitory effect of cigarette smoke condensate and heavy metals was observed on morphology and bone resorption activity of patients’ osteoclasts. SQSTM1 gene expression was upregulated in osteoclasts from patients with PDB versus healthy controls in presence of cadmium, and SQSTM1 protein expression was upregulated in presence of bismuth and tobacco smoke condensates, in particular in osteoclasts from carriers of the SQSTM1 mutation. Furthermore, high levels of oxidative stress in patients’ osteoclasts were observed.ConclusionsOur in vitro experiments suggest an interaction between SQSTM1 gene and exposure to cadmium and tobacco smoke condensates. 相似文献