Metabolic bone disease and Paget's disease in the elderly. Part I: Metabolic bone disease.
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Aged↗
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Osteomyelitis of the mandible in marble bone disease.
Marble bone disease is a rare skeletal disorder. Osteomyelitic changes in the jaw bones are frequently seen in this disease. Considering the rarity of the disease, a case of osteomyelitis of the mandible in marble bone disease is presented, together with a review of the available literature.
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Disappearance of aluminic bone disease in a long term asymptomatic dialysis population restricting A1(OH)3 intake: emergence of an idiopathic adynamic bone disease not related to aluminum.
In dialysis centers using reverse osmosis-treated water but not restricting A1(OH)3 administration, a high prevalence of histological aluminum bone disease has been reported. To assess whether this is also the case in our center where A1(OH)3 intake has always been restricted and even completely given up after 1980 thanks to high doses of CaCO3, we reviewed 42 bone biopsies performed between 1975 and 1985 in patients dialyzed for a mean duration of 56 months. Seventeen of these patients had been dialyzed before 1978 with softened water moderately contamined by aluminum, 15 had always been dialyzed with reverse osmosis-treated water and 10 had been exclusively treated by hemofiltration. The prevalence of aluminum bone disease in the whole population was 9.5% (4 patients) and consisted only of adynamic bone disease, osteomalacia being totally absent. When the patients dialyzed with aluminum-contaminated water were excluded as well as 1 diabetic patient who had taken A1(OH)3 for 1.5 years the prevalence of aluminum bone disease was null in this population. When the whole population is considered the prevalence of the other types of bone disease was 76% for osteitis fibrosa and 14.5% for a non-aluminic adynamic bone disease (6 cases). These latter cases differed from the osteitis fibrosa group only by a relative hypoparathyroidism not explained by higher plasma concentrations and higher oral cumulative doses of calcium, magnesium and aluminum or by lower plasma concentrations of phosphate and bicarbonate. None had previous parathyroidectomy, one had an unsuccessful transplantation and one was diabetic. Iron overload was excluded by negative Perls staining.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Pathogenesis of myeloma bone disease.
Myeloma bone disease is characterized by osteolytic bone destruction that is not followed by reactive new bone formation. This results in a purely lytic process, which differs from other cancers that metastasize to bone where bone destruction is followed by new bone formation. The bone destructive process in myeloma is mediated by the osteoclast (OCL), the normal bone resorbing cell. Factors that increase OCL formation and activity are produced by both myeloma cells themselves, as well as by marrow stromal cells when myeloma cells bind to marrow stromal cells. The bone destructive process releases factors that further increase the growth and survival of myeloma cells. Thus, there is a symbiotic relationship between the bone destructive process and increased growth of myeloma cells. Several studies have shown that blocking bone destruction can result in decreased tumor burden in animal models of myeloma. This overview will focus on the factors recently identified that appear to play an important role in the bone destructive process in myeloma.
Bone Diseases↗
Rheumatological presentation of developmental bone diseases.
Developmental bone disease may be present, with rheumatological disorders as the major symptoms, even in children. The major lesions encountered are early osteo arthritis, osteo chondromatosis and vertebral involvement with two leading types, pseudo Scheuermann's disease or pseudo ankylosing spondylitis. This paper presents the different features and lists the rheumatological problems in bone dysplasia.
Adolescent↗
Osteocalcin and bone morphometric parameters in adults without bone disease.
Serum bone Gla-protein (s-BGP or osteocalcin) and other serum biochemical parameters were measured in 19 subjects (8 women and 11 men, aged 20-82 years) without any bone disease. Each subject simultaneously underwent an iliac crest biopsy; tetracycline double-labeling was performed in 11 subjects, allowing correlations between s-BGP and bone histomorphometric parameters. s-BGP was significantly correlated with static bone parameters: trabecular bone volume (r = 0.74; P less than 0.001), osteoid surfaces (r = 0.69; P less than 0.001), osteoblastic surfaces (r = 0.68; P less than 0.002); dynamic bone formation parameters: total labeled surfaces (r = 0.72; P less than 0.01); and the bone formation rate (r = 0.69; P less than 0.01). We conclude that s-BGP is a valuable marker for evaluating bone formation in healthy adult subjects.
Adult↗
[Blood diseases caused by bone diseases].
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Bone Diseases↗
The pathogenesis, oral manifestations, and implications for dentistry of metabolic bone disease.
Metabolic bone diseases often result in striking oral manifestations that can lead to a diagnosis of the underlying systemic condition. Numerous studies suggest that subclinical derangements in calcium homeostasis and bone metabolism may also contribute to alveolar ridge resorption and periodontal bone loss in predisposed individuals. The significance of this spectrum of diseases and their overall impact on oral health and dental management are likely to increase as the elderly segment of the population increases in the coming decades.
Alveolar Bone Loss↗
Occupational bone diseases.
Occupational bone diseases are uncommon, with fewer than 400 cases recognized in France since 1990, although many cases have escaped diagnosis. The symptoms lack specificity, raising diagnostic challenges. Greater awareness of these conditions and of their pathophysiological mechanisms would provide much needed improvement in diagnosis and prevention.
Bone Diseases↗
Bone biopsy in various metabolic bone diseases studied by bone histomorphometry in China.
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Adult↗
Bone scanning in metabolic and endocrine bone disease.
99mTc diphosphonate bone scanning has proved to be a sensitive, though nonspecific, way of detecting a wide variety of metabolic bone disorders. Classic scan appearances of generalized increased tracer uptake throughout the skeleton are found in many types of metabolic bone disease, and whole-body retention measurements can be used to detect early forms of disease. Focal disease identified on bone scanning such as pseudofractures and brown tumors are clearly identified on the 99mTc diphosphonate bone scan, with isotope scan changes preceding radiological changes by up to 18 months. The use of whole-body retention measurements will further increase sensitivity of detection in patients with metabolic bone disease.
Bone Diseases↗
Metabolic bone diseases.
Metabolic bone diseases often present in old age and some are more easily treatable than others. Osteoporosis is best managed by prevention, with maximisation of peak bone density and reduction of subsequent bone loss. Although hormone replacement therapy is most useful in prevention, it also has a role in established osteoporosis. Other treating agents include calcium, calcitriol, calcitonin and bisphosphonates. Osteomalacia in the elderly mainly results from vitamin D deficiency and supplementation should be considered in those at risk. The newer bisphosphonates show great promise in the treatment of Paget's disease, while surgery remains the only treatment option in primary hyperparathyroidism.
Aged↗
An investigation of vanishing bone disease.
Vanishing bone disease is a rare condition producing local deformity and instability. Fibrovascular tissue replaces bone completely but the mechanism of bone destruction and resorption is unknown and there is controversy regarding the presence or absence of osteoclasts in the disease. Radiography, clinical chemistry, light microscopy, transmission electron microscopy (TEM) and cytochemistry were used to investigate the condition of a young woman presenting early in the disease process. We detected atypical ultrastructure in osteoblasts and endothelial cells. The rare osteoclasts, numerous mononuclear phagocytes and vascular endothelium found in the condition reacted positively for the enzyme acid phosphatase. Aggressive local excision of diseased tissue and insertion of a free vascularized bone graft at an advanced stage of the disease, accompanied by subsequent radiotherapy for residual disease only were successful in rehabilitating the affected forearm and hand.
Adult↗
Dialysis bone disease.
The bone disease associated with end-stage renal failure (ESRD) and treatment are complex and multifactorial, and has changed in both clinical and imaging features over the past three decades. Whereas previously features of vitamin D deficiency (rickets/osteomalacia) and intense, and prolonged, secondary hyperparathyroidism (bone resorption, osteosclerosis, metastatic calcification) predominated, these features are now rarely evident radiologically. This has occurred through the better understanding of vitamin D metabolism and improvements in therapeutic management. However, metastatic calcification in soft tissues and 'adynamic" bone continue to be problematic. New complications have developed as a consequence of treatment (dialysis and transplantation), including amyloid deposition, noninfective sponyloarthropathy, osteonecrosis, and osteopenia/osteoporosis). Radiographs remain the most widely used imaging technique in examining for skeletal disease in patients with ESRD on maintenance dialysis. Occasionally, more sophisticated imaging (CT, MRI, nuclear medicine scanning) are helpful (parathyroid tumor localization, differentiation between infection and amyloid deposition). Developments in quantitative methods to assess bone density enable the effects of ESRD and treatment to be studied and monitored. Technical developments in computed tomography (rapid, multislice scanning) allow quantitation and monitoring of metastatic cardiac calcification in patients on hemodialysis, which has relevance to prognosis.
Bone Density↗
Radial bone mineral density in hemodialysis patients with adynamic bone disease.
Adynamic bone disease (ABD) has attracted attention as the most frequent type of renal osteodystrophy, but there are few reports about the bone mineral density (BMD) in ABD patients. This study investigated the BMD in hemodialysis patients with ABD and with relatively normal bone turnover. We measured the BMD of the distal one-third of the radius by dual-energy X-ray adsorptiometry. In the ABD group (intact PTH<65 pg/ml, intact osteocalcin<30 ng/ml), there were 19 men and 17 women with a mean age of 56.4 +/- 12.0 years. In the relatively normal bone turnover group (intact PTH: 120-250 pg/ml), there were 24 men and 16 women with a mean age of 57.1 +/- 14.7 years. Although there were no significant differences between the two groups with respect to age, gender, and duration of hemodialysis, a significant increase of the BMD and the calcium x phosphate product was observed in the ABD group (radial BMD: 0.648 +/- 0.137 g/cm2 versus 0.572 +/- 0.132 g/cm2, calcium x phosphate product: 57.53 +/- 14.92 mg2/dl2 versus 49.76 +/- 12.13 mg2/dl2). These findings suggest that an increase in radial BMD may not be a useful marker of the improvement in bone lesions in ABD patients.
Absorptiometry, Photon↗
[Ectopic calcification in patients with aplastic bone disease undergoing dialysis].
Aplastic bone disease (adynamic bone disease) has been attracted attention as the most frequent type of renal osteodystrophy in patients undergoing dialysis, and relatively low serum parathyroid hormone level is considered to be the major cause of aplastic bone disease. In aplastic bone disease, both the number of osteoblast and osteoclast are few and the speed of bone turnover is low, therefore, buffering action of bone for calcium and phosphorus is extremely disturbed. Hypercalcemia due to a prescription of large dose calcium carbonate and hyperphosphatemia derived by high protein diet in this occasion induce high serum calcium and phosphorus product level, and ectopic calcification is likely to occur. When ectopic calcification occur at artery it might develop ischemic change of visceral organ. Recently, the strong association between coronary arterial calcification and high serum calcium and phosphorus products is clarified by a method of electron-beam computed tomography. Based on these findings, high calcium and phosphorus product is now believed as a risk for poor patients' survival. Thus, we should pay attention to calcium and phosphorus product in patients suffering from aplastic bone disease.
English Abstract↗
An in vivo model of human multiple myeloma bone disease.
Osteolytic bone destruction and its complications, such as hypercalcemia, pathologic fractures and nerve compression, are the major source of morbidity in patients with multiple myeloma (MM). The bone destruction in MM is due to increased osteoclast activity, but the mechanisms responsible are not entirely clear. We have utilized a human plasma cell leukemia cell line, ARH-77, that has disseminated growth in mice with severe combined immunodeficiency (SCID) and expresses immunoglobulin G kappa (IgG kappa), as a model for human MM. Fifteen SCID mice were irradiated with 400R and 10 of these were injected with 10(6) ARH-77 cells i.v., 24 h after irradiation. Five mice were used as a control group. Development of bone disease was assessed by blood calcium levels, x-rays and histology. Seven out of seven mice that survived irradiation and received ARH-77 cells developed hind limb paralysis 28-35 days after injection. One hundred percent of these mice developed hypercalcemia (1.35-1.46 mmol/l), a mean of five days after becoming paraplegic. Lytic bone lesions were detected by x-ray in all the hypercalcemic mice examined. No lytic lesions or hypercalcemic developed in the controls. Mice were then sacrificed after developing hypercalcemia. Histologic examination of the ARH-77 mice showed infiltration of myeloma cells in the liver and spleen. Marked infiltration by the tumor was found in vertebrae and long bones, with loss of bony trabeculae and increased osteoclast numbers.(ABSTRACT TRUNCATED AT 250 WORDS)
Animals↗
Assessment of therapeutic response in patients with metastatic bone disease.
Metastatic bone disease is common in cancer patients and causes substantial disease-related morbidity and mortality. However, several effective treatments are available for the management of these patients. Bisphosphonates, which inhibit osteoclast-mediated resorption of bone matrix, are especially important because they decrease the incidence of skeletal-related events in many tumour types and can complement antineoplastic therapies. At present, assessment of treatment for bone metastases is hindered by a lack of effective, rapid methods to measure disease response. We discuss the difficulties of current measures of response assessment and describe the development of new radiological and biochemical markers of bone metastases. Assays that detect type I collagen telopeptides as markers of bone resorption seem to be most promising at present.
Absorptiometry, Photon↗