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1.
Paget’s disease of bone: a review 总被引:1,自引:1,他引:0
Paget's disease of bone (PDB) is a condition of unknown etiology characterized by excessive and abnormal bone remodeling. It may be localized to one or several skeletal segments. The disease seldom appears before the age of 40 years, but its prevalence tends to double each decade from the age of 50 onwards, reaching about 10% after ninth decade. PDB may virtually affect every bone in the skeleton. Affected bones are involved right away with no new involvement during the evolution. The basic symptom of the disease is bone pain, while complications depend on skeletal sites involved and range from secondary osteoarthritis to malignant degeneration. Diagnosis is usually based upon clinical features, imaging, and laboratory analyses. Therapeutic approach is currently based on second-generation bisphosphonates. Their use is recommended when bone alkaline phosphatase is high and/or when the disease localizations are highly suspected for determining complications. 相似文献
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Werner de Castro GR Heiden GI Zimmermann AF Morato EF Neves FS Toscano MA de Magalhães Souza Fialho SC Pereira IA 《Rheumatology international》2012,32(3):627-631
Paget’s disease of bone (PDB) exhibits a marked geographic variation. In Brazil, the prevalence of PDB is unknown and only
a few clinical data are available. The aim is to determine clinical, laboratory, imaging and response to treatment data in
a large PDB case series in the city of Florianopolis, Brazil. We have performed a retrospective study based on charts reviews
of all patients with PDB followed at the University Hospital of the Federal University of Santa Catarina and at five different
private rheumatology outpatient offices in Florianopolis, between 1995 and 2009. One hundred and thirty-four patients with
PDB were identified. Mean age at diagnosis was 63.2 ± 10.5 years, 67.2% were women, and 91.1% were Caucasian. Positive family
history was reported in only 8.2%. Polyostotic disease was found in 75.0% of the cases, bone pain in 77.9%, and bone deformities
in 15.9%. Higher levels of AP were significantly associated with polyostotic disease and skull involvement. Pelvic bones were
the most frequently affected (53.7%). Complications included deafness in 8.2%, bone fractures in 3.0%, hydrocephalus in 2.2%,
and cauda equina syndrome in 0.7% of the cases. Treatment with zoledronic acid achieved the best response with only 2.9% failing
to respond adequately. According to literature data, PDB in South America seems to be characterized by an overall low prevalence,
but with localized clusters with higher prevalence. The authors have described a cluster of PDB in Florianopolis, in Southern
Brazil. Further properly designed studies are necessary to clarify the PDB epidemiology in South America. 相似文献
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Paget's disease is a metabolic bone disease characterized by excessive bone resorption and formation due to activated osteoclasts. Although Paget's disease is a high bone turnover state, the excess bone that is formed lacks the structural stability of normal bone. Complications from Paget's disease include deformity, fracture, and pain. Although still unclear, both prevalence and severity of Paget's disease seem to be declining. Recent progress has focused on the environmental as well as genetic etiologies for this disease. Many studies indicate a role for viral infectious agents, whereas others point to a recently identified candidate gene on chromosome 18q. Therapy with bisphosphonate drugs is the treatment of choice. With newer and more powerful agents from this family now available, the majority of patients affected by Paget's disease can achieve sustained remission and avoid complications. 相似文献
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Patterns of pain in Paget’s disease of bone and their outcomes on treatment with pamidronate 总被引:1,自引:0,他引:1
Two-thirds of patients with Pagets disease seeking medical attention present with pain. We studied patterns of pagetic pain in relation to physical activity, and the effect of coexistent osteoarthritis (OA)/pagetic arthropathy on pain and physical activity before and after treatment with pamidronate. Patients with lower-segment Pagets disease (lumbar spine, pelvis or lower limbs), raised alkaline phosphatase (ALP), and pain as a symptom were included. Two subgroups were identified based on the absence (P group), or presence (POA group) of radiological OA in the lower segment. They received 2–4 fortnightly infusions of pamidronate 30–60 mg and were followed up at 6 weeks, 3 months and 6 months. Outcome measures were visual analog scales for pain at rest, on standing, on walking and at night; standing time, walking distance, 50-yard walking time, and ALP. Twenty-five patients (12 men, 13 women, mean age 70.1±1.9 years), received a mean of 142±9.7 mg of pamidronate. The P group had higher mean ALP (p=0.003) and higher scores for pain (not significant) at baseline than the POA group. Compared to baseline, P group had significant improvements (p<0.01) at 6 months in rest pain, standing pain and walking pain. The POA group had non-significant changes in pain at 6 months. ALP improved significantly at 6 months in both subgroups (p<0.01). The whole group also improved at 6 months in standing time (55.7%, not significant), walking distance (33.9%, not significant), and 50-yard walking time (24.2%, p<0.05). Pagets patients with coexistent joint disease had less severe pain and bone disease at baseline than those without coexistent joint disease, and responded less well to pamidronate, although they did have significant improvement in ALP levels. Radiographic assessment for coexisting joint disease prior to treatment might improve prognostication from the patients point of view, and improve treatment compliance.Abbreviations ALP
alkaline phosphatase
- OA
Osteoarthritis 相似文献
6.
Gkouva L Andrikoula M Kontogeorgakos V Papachristou DJ Tsatsoulis A 《Clinical rheumatology》2011,30(1):139-144
Paget’s disease of bone is a focal skeletal disorder characterized by the formation of structurally abnormal bone, skeletal
deformities, and other complications leading to bone pain and significant disability. It can involve one or more areas in
a single bone (monostotic) or multiple bones (polyostotic). Most of the time the disease is asymptomatic and the diagnosis
is made incidentally by increased levels of bone metabolism markers, especially alkaline phosphatase and is confirmed by specific
findings in radiographs and radionuclide bone scan. In this report, we describe the case of a 65-year-old female with clinical
and radiological findings of active Paget’s disease of bone, but with absence of abnormal biochemical markers. The patient
was given a dose of 5 mg zoledronic acid intravenously with significant clinical improvement within the next 6 months. The
present case not only shows that Paget’s disease of bone can occur in the setting of normal markers of bone metabolism but
also that, in such cases, the response to treatment can be monitored by improvement in the clinical picture or by correct
evaluation of the imaging findings. 相似文献
7.
The incidence of Pagets disease has been estimated to be about 3%, but it is extremely rare in Asia, especially in Korea. In addition, monostotic involvement seems to be far less frequent. In this report, we describe a case of monostotic Pagets disease localized in the right tibia.Abbreviations ALP alkaline phosphatase 相似文献
8.
Yoshihiro Nishida Yoshihisa Yamada Satoshi Tsukushi Hideshi Sugiura Hiroshi Urakawa Naoki Ishiguro 《Clinical rheumatology》2013,32(2):241-245
Although Paget’s disease of bone (PDB) is common in western countries, it is extremely rare in Asian ones including Japan. Recently, oral risedronate (17.5 mg once daily) was approved in Japan as a treatment of PDB besides calcitonin and etidronate. However, there are few data regarding the efficacy of this agent, dose for patients with PDB in Japan, or the durability of its effect. The purpose of this study was to evaluate the midterm outcome of oral risedronate (17.5 mg once daily) for patients with PDB in Japan. Seventeen patients with PDB were treated with risedronate (17.5 mg once daily) for 8 weeks. Efficacy and its durability were accessed based on serum total alkaline phosphatase (ALP) and symptoms. Risedronate effectively suppressed bone turnover evaluated with serum total ALP in all patients. In 8 of 10 patients with bone pain, risedronate reduced the pain. On the other hand, tinnitus and hearing loss did not disappear but somewhat improved. None of the patients suffered severe complications. Seven of 17 patients required readministration of oral bisphosphonate (risedronate, six; alendronate, one) due to elevated total ALP at 27 months (mean ranging from 9 to 39 months) after the initial administration of risedronate. Treatment of oral risedronate (17.5 mg once daily) for 8 weeks is safe and effective for patients with PDB in Japan. However, the durability of its effect is limited in some patients. 相似文献
9.
The diagnosis of Paget’s bone disease (PD) is often reached fortuitously with X-ray or scintigraphy. The peculiar distribution
of the bone tracer in some skeletal locations, such as vertebra, may be highly suggestive of this disorder. Indeed, there
are two distinct vertebral images on the bone scan highly specific of PD: the “clover” and the “heart” images. It has been
previously reported that the specificity of these images for PD diagnosis is very high, nearly 100%, then suggesting that
these findings could be patognomonic of this process. We have recently observed a 55-year-old male patient with back pain
secondary to a vertebral metastasis due to a hepatocellular carcinoma. Initially, the bone scan showed the characteristic
“clover” image at T-12 level and he was misdiagnosed with PD. Afterwards, the MRI suggested the diagnosis of a vertebral metastasis,
and a computed tomography-guided needle biopsy further confirmed the diagnosis. In view of the present data, this image cannot
be considered as patognomonic of PD. 相似文献
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Dae Won Ma Jie-Hyun Kim Tae Joo Jeon Yong Chan Lee Mijin Yun Young Hoon Youn Hyojin Park Sang In Lee 《Digestive and liver disease》2013,45(9):769-775
BackgroundThe roles of positron emission tomography and bone scanning in identifying bone metastasis in gastric cancer are unclear.AimWe compared the usefulness of positron emission tomography–computed tomography and scanning in detecting bone metastasis in gastric cancer.MethodsData from 1485 patients diagnosed with gastric cancer who had undergone positron emission tomography–computed tomography and scanning were reviewed. Of 170 enrolled patients who were suspected of bone metastasis in either positron emission tomography or scanning, 81.2% were confirmed to have bone metastasis.ResultsThe sensitivity, specificity, and accuracy were 93.5%, 25.0%, and 80.6%, respectively, for positron emission tomography and 93.5%, 37.5%, and 82.9%, respectively, for scanning. 87.7% of patients with bone metastasis showed positive findings on two modalities. 15.0% of solitary bone metastases were positive on positron emission tomography only. Positron emission tomography was superior to scanning for the detection of synchronous bone metastasis, but the two modalities were similar for the detection of metachronous bone metastasis. The concordance rate of response assessment after treatment between two modalities was moderate.ConclusionsPositron emission tomography–computed tomography may be more effective for the diagnosis of bone metastasis in the initial staging workup. Conversely, bone scanning and positron emission tomography–computed tomography may be similarly effective for the detection of metachronous bone metastasis. 相似文献
14.
Ioannis Karatzoglou Maria P Yavropoulou Maria Pikilidou George Germanidis Evangelos Akriviadis Alexandra Papazisi Michael Daniilidis Pantelis Zebekakis John G Yovos 《World journal of gastroenterology : WJG》2014,20(28):9534-9540
AIM: To investigate the postprandial response of bone turnover markers in patients with Crohn’s disease (CD).METHODS: Fifty nine patients with CD aged 38 ± 14 years, and 45 healthy individuals matched for age and body mass index were included in the study. All participants underwent an oral glucose tolerance test (OGTT) after an overnight fast and serum levels of the bone resorption marker C-terminal crosslinking telopeptide of type I collagen (CTX-I) and the bone formation marker procollagen type I N propeptide were measured. Activity of the disease was assessed by calculation of the Crohn’s disease activity index (CDAI).RESULTS: Serum CTX-I was significantly higher in patients compared to controls (CTX-I: 453 ± 21 pg/mL vs 365 ± 25 pg/mL, P = 0.008), and values were significantly correlated with the activity of the disease (r = 0.435, P = 0.001). Results from OGTT-induced suppression of CTX-I showed two different trends. Patients with more active disease (assessed as CDAI > 150) had a more excessive suppression of CTX-I compared to controls (55% vs 43% P < 0.001), while patients on remission (assessed as CDAI < 150) demonstrated an attenuated CTX-I suppression (30% vs 43% P < 0.001). In line with this, CTX-I suppression after oral glucose load was significantly correlated with the activity of the disease (r = 0.913, P < 0.001).CONCLUSION: The physiological skeletal response of postprandial suppression of bone resorption is maintained in patients with CD and is strongly dependent to the activity of the disease. 相似文献
15.
Long-Term Outcome of Perianal Paget’s Disease 总被引:11,自引:0,他引:11
McCarter MD Quan SH Busam K Paty PP Wong D Guillem JG 《Diseases of the colon and rectum》2003,46(5):612-616
PURPOSE: Extramammary Pagets disease of the perianal region is a rare finding that often results in delayed diagnosis and treatment. Although the natural history of the disease is not well characterized, it historically has been associated with other cancers. This study summarizes the history and treatment of all patients diagnosed with perianal Pagets disease at a single institution.
METHODS: Charts of all patients with a diagnosis of extramammary Pagets disease of the perianal region confirmed or treated at Memorial Sloan-Kettering Cancer Center between 1950 and 2000 were reviewed. Patients with vulvar Pagets disease or Bowens disease were excluded except when Pagets disease of the perianal region was diagnosed first. Whenever possible, follow-up information was updated. Estimates of overall and disease-free survival were made by the method of Kaplan and Meier.
RESULTS: Twenty-seven patients with a median age of 63 years were diagnosed with perianal Pagets disease. Most patients (74 percent) were treated with wide excision. Local recurrence occurred in 37 percent of all patients treated and in 30 percent of patients (6/20) undergoing a wide excision as part of their treatment. An invasive component was identified in 44 percent of patients (12/27) with perianal Pagets disease. Six patients (22 percent) required a colostomy as part of the treatment for their disease. Adjuvant chemoradiotherapy was used in 22 percent of patients (6/27) who had more aggressive disease. At a median follow-up of 67 months, 56 percent (15/27) had no evidence of disease, and two patients had died of metastatic disease. The overall and disease-free survival at five years was 59 and 64 percent, respectively, which decreased to 33 and 39 percent, respectively, by ten years.
CONCLUSIONS: Perianal Pagets disease is a rare finding even at a large referral center. The disease process is generally a prolonged one marked by frequent recurrences, and the treatment of first choice is wide excision. Patients with invasive malignancies require more extensive surgery. The role of chemoradiotherapy remains undefined in this disease. 相似文献
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Soufleris K Pilpilidis I Tzilves D Moschos J Gatopoulou A Patakiouta F Tarpagos A Katsos I 《Acta gastro-enterologica Belgica》2007,70(2):231-234
Gastric adenocarcinoma is currently the 14th cause of death worldwide. Early gastric cancer, defined as cancer not penetrating deeper than the submucosa, is considered to carry an excellent prognosis with 5-year survival rates reaching more than 90%. Cases of bone metastases due to intramucosal gastric cancer are very rarely described. A case of a 70-year old male presenting with confirmed bone metastases 7 years after a curative resection for a mucosal gastric carcinoma is discussed. The patient was investigated with bone marrow biopsy and bone scan and showed no other signs of disease. The clinicopathologic features included poor differentiation, signet ring cells presence, no lymph node involvement and a negative second laparotomy two years after the initial surgery. Studies concerning the presence of residual disease in the form of bone marrow micrometastases are briefly reviewed emphasizing that intramucosal gastric cancer still carries the p sibility for metastasis, many years after a curative resection, mandating long term alertness from the attending physician. 相似文献
19.
Kersting S Bruewer M Laukoetter MG Rijcken EM Mennigen R Buerger H Senninger N Krieglstein CF 《International journal of colorectal disease》2007,22(4):411-417
Background Surveillance of intestinal cancer in Crohn’s disease (CD) has often been advocated. To date, no clear evidence exists whether
CD patients are at special risk for intestinal cancer. An increased incidence of small bowel adenocarcinoma is suggested.
However, recent figures also suggest an increased risk of CD associated colorectal cancer. We report our experience with 10
cases of CD complicated by intestinal adenocarcinoma.
Materials and methods Our institutional database included 330 patients treated for CD between 1988–2005. Data of patients that developed carcinoma
within Crohn’s lesions of either small or large bowel were analyzed.
Results Ten patients were diagnosed with CD complicated by carcinoma. In nine patients, cancer was present in the colorectum and in
one, in Crohn’s ileitis. Tumors were in conjunction with fistulae in three and developed within strictures in five patients.
Mean age at the time of diagnosis of CD was 43 years. Mean duration of CD until diagnosis of cancer was 14 years. Only five
patients were diagnosed for cancer preoperatively. Staging revealed advanced tumors in almost all patients. Mean survival
after surgery was 29 months (2–149 months).
Conclusions Cancer risk in CD and especially in Crohn’s colitis may still be underestimated. Delayed diagnosis resulted in a poor prognosis.
The value of colonoscopy as surveillance tool is questioned by the fact that in our patients, carcinoma was diagnosed in some
patients preoperatively by routine colonoscopy. Therefore, additional markers should be identified to detect CD patients at
risk. 相似文献
20.
Effects of Linomide on growth and metastasis of implanted human gastric cancer in nude mice 总被引:2,自引:0,他引:2
TAO Hou Quan LIN Yan Zhen YIN Hao Ran GU Qin Long ZHU Zheng Gang YAO Ming 《World journal of gastroenterology : WJG》1997,3(3):153-155
EfectsofLinomideongrowthandmetastasisofimplantedhumangastriccancerinnudemiceTAOHouQuan,LINYanZhen,YINHaoRan,GUQinLong,ZHU... 相似文献