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[Action of calcitonin on osteoclasts in Paget's bone disease during long term treatment (author's transl)].

Comparison of biopsies from patients with Paget's bone disease before and during long-term treatment with calcitonin reveals that the ration of bone-adherent osteoclasts to free osteoclasts is not modified by the hormone. Nor does treatment alter the average number of nuclei in osteoclasts. Under electron microscopy, all the cytological anomalies of osteoclasts in Paget's bone disease and, in particular, the characteristic nuclear inclusions, persist through treatment. Thus, although such osteoclasts do react to calcitonin as demonstrated by several authors, the specific morphological anomalies remain unaffected by the treatment. It is likely that the osteoclast in Paget's bone disease is an abnormal cell with peculiarities which may be related to the yet unknown etiology of the disease.

Bone and Bones

[Additional comments on the differential diagnosis of rare bone diseases (author's transl)].

We report about two seldom types of diseases of bones. Both came to our hospital with the diagnosis Osteomyelitis. The first case, the Mafucci-Syndrome, is presented--a systemic Chondrodysplasia with Angiomatosis. This disease is not hereditary. There are found multiple Enchondromas mostly in the longer bones. In 20% the cases of the Mafucci-Syndrome get malignant. The second case shows the Conradi-Hünermann-Syndrome a Chondrodysplasia calcificans congenita, which seems to be hereditary. The characteristic sign is the minor growth of the bones, caused by early deposition of lime in the regions of ossification. Malignity is not described.

Angiomatosis

Comparative study of alkaline phosphatase isoenzymes, bone histology, and skeletal radiography in dialysis bone disease.

Liver, intestinal, and bone alkaline phosphatase isoenzymes were measured using heat stability and L-phenylalanine inhibition techniques in 78 patients on intermittent haemodialysis. Fifty-five patients had abnormalities in one or more of the isoenzymes. Changes in bone and intestinal alkaline phosphatase activities seemed to be related and raised liver isoenzyme activity was associated with the development of liver disease. Abnormal histological and radiological findings were better correlated with bone alkaline phosphatase levels than with total alkaline phosphatase, and serial estimations of bone isoenzyme activity were useful in assessing the response of renal osteodystrophy to treatment with a vitamin D analogue. Serum alkaline phosphatase isoenzyme measurement provides another useful and non-invasive index for monitoring metabolic bone disease in patients with chronic renal failure.

Adult

Treatment of bone disease with dihydrotachysterol in patients undergoing long-term hemodialysis.

Nine of 24 patients undergoing long-term hemodialysis were found to have evidence of moderate to severe bone disease. Two had bone pain and muscle weakness and two had pseudofractures. Eight of the nine were treated with dihydrotachysterol (DHT), 0.25 to 0.375 mg/d initially, but four required doses between 0.5 and 1.0 mg/d. Ther serum alkaline phosphatase value decreased in all patients and returned to normal in six. The bone pain and muscle weakness resolved and the pseudofractures healed. Bone biopsies in six patients before and after initiation of treatment with DHT showed that the osteoid area decreased significantly from 29.6 +/- 22.8% (mean +/- standard deviation) to 11.5 +/- 7.5% (P less than 0.025) and the resorptive surface decreased in all patients. DHT, in doses of up to 1.0 mg/d, is effective in treating both the osteitis fibrosa and the osteomalacic components of bone disease in patients undergoing hemodialysis.

Bone and Bones

Factors influencing the response to 1alpha-hydroxyvitamin D3 in patients with renal bone disease.

Twenty-three patients with bone disease and chronic renal failure were treated for periods of 4--28 months with 1alpha-hydroxyvitamin D3 (1alpha-OHD3). Improvements in bone histology were consistently seen in patients with features both of osteitis fibrosa and osteomalacia but were not invariably observed in patients with osteitis fibrosa or osteomalacia alone (37 and 50% improved respectively). Several factors influencing the outcome of treatment were assessed on the basis of histological responses in bone. A low level of plasma calcium before treatment, rather than the dose of 1alpha-OHD3 tolerated, was the major detectable factor which favourably affected the histological outcome. Other factors examined, including initial plasma concentrations of phosphate, immunoreactive parathyroid hormone and alkaline phosphatase, and treatment with haemodialysis or dietary supplements of calcium did not apparently influence the response.

Alkaline Phosphatase

Treatment of bone disease in patients on chronic hemodialysis with dihydrotachysterol.

Nine out of 24 patients on chronic hemodialysis were found to have biochemical, radiologic and bone biopsy evidence of moderate to severe bone disease. Two patients had bone pain and muscle weakness, 2 had pseudofractures, and one patient had a pathologic fracture of the neck of the femur. Eight patients were treated with dihydrotachysterol (D.H.T.), 0.25 to 0.37 mg/day initially. Four patients required doses between 0.5 and 1.0 mg daily. The alkaline phosphatase decreased in all patients, returning to normal in 6 patients. The symptoms of bone pain and muscle weakness resolved, and the pseudofractures healed. Repeat bone biopsies were performed in 6 patients 12 mos or more after treatment with D.H.T. The osteoid area fell from 29.6 +/- 22.8 to 11.5 +/- 7.5% (p less than 0.025). Resorptive surface decreased in all patients. D.H.T., in doses of up to 1.0 mg/day, is effective in the treatment of both the osteitis fibrosa and the osteomalacic component of bone disease in patients on hemodialysis.

Calcium

Semi-quantitative interpretation of the bone scan in metabolic bone disease: definition and validation of the metabolic index.

Certain easily recognisable features are commonly seen in the bone scans of patients with metabolic bone disorders. Seven such features have been numerically graded by three independent observers in the scans of 100 patients with metabolic bone disease and of 50 control subjects. The total score for each patient is defined as the metabolic index. The mean metabolic index for each group of patients with metabolic bone disease is significantly greater than that for the control group (P less than 0.001).

Bone Diseases

Relationship of the activity of the bone isoenzyme of serum alkaline phosphatase to urinary hydroxyproline excretion in metabolic and neoplastic bone diseases.

A significant correlation between the activity of the bone isoenzyme or serum alkaline phosphatase and the urinary hydroxyproline excretion in osteomalacia, osteoporosis, primary hyperparathyroidism with osteodystrophy, Paget's disease, secondary bone tumours, and in a control group was found (P less than 0.001). This close correlation was not observed between these variables in patients with active acromegaly. Diagnosis determined from these indices of formation and turnover of bone matrix agreed with that established by histological and histochemical examination of bone, by X-ray investigation of the skeleton, and by the radionuclear 85Sr test. The relationship between the activity of bone isoenzyme and urinary hydroxyproline excretion differed in metabolic bone diseases with a high bone turnover, in patients with osteoporosis and in patients with early osteoclastic bone metastases.

Acromegaly

Radiological/pathological correlations in uremic bone disease.

Skeletal radiographs and non-decalcified bone specimens from 17 chronically uremic patients with radiographic evidence of bone disease were studied quantitatively. The results of each morphological technique were compared in an attempt to define the roentgenographic manifestations of renal osteodystrophy histologically. The radiographs correlated best with the trabecular bone manifestations of osteitis fibrosa but showed poor correlation with histological evidence of osteomalacia. Radiographic signs of osteosclerosis could not be correlated with any radiographic or histiolgical feature of bone resorption.

Adult

Nutrition and bone disease in the dog and cat.

The most commonly encountered nutritional bone disease is nutritional secondary hyperparathyroidism. This is primarily of importance in the dog but is occasionally seen in kittens, particularly of the Siamese breed, and is often associated with the feeding of owner compiled, meat-rich diets. Classic rickets is now a rare clinical entity. Hypertrophic osteodystrophy is regularly seen in the larger breeds of dog and the aetiology remains obscure. Hypervitaminosis A associated with liver-rich diets is often encountered in the cat. Hypovitaminosis A has been described but its true clinical significance is unknown.

Animals

Bone disease.

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Animals

Disappearing bone disease. A clinical and histological study.

The case histories of three patients, a man and two boys, with disappearing bone disease are reported. The clinical, roentgenographic, and histopathological features are described in detail. Histologically, in the early stages of the disease, the vanishing bone is replaced by numerous wide engorged capillaries. Eventually the bone is replaced by dense fibrous tissue. All three patients were treated by radiotherapy. Histochemical studies performed in one case revealed strong acid phosphatase and leucine aminopeptidase activities in perivascular mononuclear cells (possibly pericytes), suggesting that these cells took part in the bone resorption.

Acid Phosphatase

Clinical and laboratory considerations in metabolic bone disease.

An overview of the common types of metabolic bone disease is described. When the disease is present in pure form, diagnosis is not difficult. When mixed disease is present, as may be the case, the pathophysiology involved must be clearly understood for accurate diagnosis and treatment.

Chronic Kidney Disease-Mineral and Bone Disorder

Total urinary and free serum hydroxyproline in metastatic bone disease.

The present study examines the possibility of correlation between free serum and urinary total hydroxyproline and whether this correlation can be applied to clinical conditions. The correlation between the two indices was 0.80 (P less than 0.001) in 18 patients, mostly suffering from malignant disease. On comparing the same measurements in 37 patients, all with known metastatic bone disease, we found 29/37 normal results for free serum hydroxyproline, whereas only 2/37 values of urinary total hydroxyproline were normal. The authors therefore conclude that urinary total hydroxyproline, measured as the ratio hydroxyproline/creatinine in a fresh specimen of early-morning urine, is the best index of collagen breakdown in metastatic bone disease and preferable to measurement of free serum hydroxyproline.

Adult

The relationship between disturbed metabolism of vitamin D and bone disease in chronic renal failure.

Following the discovery that the kidney is involved in the metabolism of vitamin D, a causal relationship has been sought between defective production of 1,25-dihydroxy vitamin D3 and bone disease in chronic renal failure. This paper reviews some of the clinical evidence for and against such a relationship, and considers the possible role of other vitamin D metabolites in the pathophysiology of renal bone disease.

Bone Diseases