Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0005940 (bone disease)
 7,459 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A female pet wooly monkey with metabolic bone disease initially presented with a proliferating bony mass in the left humerus which had many features of osteosarcoma. At necropsy, parathyroid hyperplasia, osteoclastic resorption, proliferative osteoid deposition in the calvarium and cortex of long bones, and fibrous proliferation of the marrow indicated the presence of generalized osteodystrophia fibrosa. The dietary history of deficient vitamin D3 and protein and minimal exposure to sunlight supported this diagnosis, as did depressed levels of serum calcium and elevated levels of serum parathyroid hormone, alkaline phosphatase, and acid phosphatase.
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PMID:Metabolic bone disease resembling osteosarcoma in a wooly monkey (Lagothrix lagotricha). 21 39

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.
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PMID:Disappearing bone disease. A clinical and histological study. 83 76

Bone metabolic homeostasis is regulated by a number of hormones and local modulators, and the study of these factors has been of major help in our understanding of bone disease. However, these parameters do not, in a strict sense reflect the metabolic and biochemical changes in the diseased bone tissue. Thus, there is a great interest in the study of biochemical specific "markers" of bone metabolic processes, namely of bone formation and bone resorption. Alkaline phosphatase, osteocalcin, osteonectin, and procollagen type I propeptides are the currently known markers of bone formation, whereas urinary hydroxyproline and hydroxylysine glycosides, plasma tartrate resistant acid phosphatase, and urinary hydroxy-pyridinium crosslinks of collagen are considered markers of bone resorption. In this paper, we review the background work on each of these markers, and subsequently give an overview of the currently available data on their usefulness in metabolic bone diseases, namely in Paget's disease of bone, primary hyperparathyroidism, osteoporosis, and renal osteodystrophy.
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PMID:Biochemical markers of bone metabolism. 192 60

Twenty five hormone manipulated patients with prostate cancer and metastatic bone disease, treated at least 6/12 previously by hormone manipulation, were given intravenous infusions of Disodium Pamidronate (APD) over a 6 month period. Patients received 30 mg weekly for 4 weeks then twice monthly for 5 months. No other treatment was administered during study. Eleven of 17 patients with pain at the start of the study were pain free at the end. Fasting morning calcium excretion and serum osteocalcin fell significantly with Pamidronate (P less than 0.0001) and urine hydroxyproline was lowered in 13/20 evaluable patients at 6 months. Alkaline phosphatase fell in a proportion of patients and five of 17 patients with previously progressive bone scans stabilised (4) or regressed (1) on treatment. Rising acid phosphatase levels were also lowered in five patients. It is concluded that Pamidronate may be effective in palliating bone pain in some patients and has a stabilising influence on abnormally high bone turnover in metastatic prostate cancer. Further controlled studies of the compound are now warranted.
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PMID:Osteoclast inhibition by pamidronate in metastatic prostate cancer: a preliminary study. 200 84

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.
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PMID:An investigation of vanishing bone disease. 239 Mar 78

Tartrate resistant acid phosphatase (TRAP) is a reliable histochemical marker of osteoclasts when used on tissue sections of undecalcified bone. This paper presents an original morphometric analysis which can be done after histochemical identification of osteoclasts. These bone resorbing cells were demonstrated on undecalcified bone biopsies from control subjects and patients presenting a malignant disease of the lymphocyte B lineage. Computerized analysis of the osteoclastic population revealed that: (1) all TRAP positive cells along bone trabeculae belong to a osteoclastic population; (2) that B cell malignancies had an increased bone resorption. At the scanning electron microscopic level small resorption bays (about 10 microns in diameter) were observed either associated or separated from eroded surfaces presenting abnormal appearance; TRAP staining of histological sections of undecalcified bone, coupled with morphometric studies, may help in the understanding of bone disease pathobiology.
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PMID:Osteoclast cytomorphometry and scanning electron microscopy of bone eroded surfaces during leukemic disorders. 240 8

One hundred and thirty-nine patients with advanced prostate cancer were entered into a randomised trial to test the efficacy and tolerance of goserelin 3.6 mg depot (Zoladex) versus stilboestrol 3 mg/day. As well as the usual clinical and radiological assessments of extent of disease, we used an immunoradiometric assay of prostate specific antigen (PSA) (Hybritech Europe) and normal laboratory enzymatic assays of acid phosphatase (AP) and alkaline phosphatase (ALKP) for biochemical assessment. The upper limit of normal for PSA was taken as 10 micrograms/l. The range of PSA was wide and differed significantly from that of AP and to a lesser extent ALKP in metastatic cases. PSA outperformed both AP and ALKP in both the local and advanced groups in terms of sensitivity. There was no correlation, however, between histological grade and level of PSA, AP or ALKP (the latter in cases with bone disease). In patients with metastatic disease diagnosed by bone scan, nine patients had one abnormal site/one "hot spot", and all of these had a PSA greater than twice the normal upper limit. Early death due to prostate cancer was noted in four patients with levels of PSA greater than 2500 micrograms/l. PSA is more sensitive than either enzymatic AP or ALKP in both locally advanced and metastatic prostate cancer and is useful in identifying those advanced cases who have single lesions on bone scan. In this series PSA gave an overall sensitivity of 89%, compared with 63% for AP and 64% for ALKP in patients with metastatic disease.
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PMID:The role of prostate specific antigen in the baseline assessment of patients undergoing hormone therapy for advanced prostate cancer. 244 97

This report is the first cytochemical investigation of vanishing bone disease "Gorham's Disease" (Gorham and Stout 1955). The ultrastructural localization of non-specific alkaline phosphatase and of specific and non-specific acid phosphatase activity was studied in slices of tissue removed from a patient with this rare disorder. Sodium beta-glycerophosphate and phosphorylcholine chloride were used as substrates. Alkaline phosphatase was present around the plasma membranes of osteoblasts and associated with extracellular matrix vesicles in new woven bone. This is consistent with the proposed role for this enzyme (Robison 1923) and for matrix vesicles (Bonucci 1967) in the mineralization of bone (Bernard and Marvaso 1981). Concentrations of specific secretory acid phosphatase reaction product in the cytoplasm of degenerating osteoblasts may contribute to the imbalance between bone formation and resorption. Osteoclasts, while few in number, showed non-specific and specific acid phosphatase activity. The Golgi apparatus and heterophagic lysosomes of mononuclear phagocytes were rich in non-specific acid phosphatase. This was also present in the Golgi lamellae and lysosomes of endothelial cells. Acid phosphatase cytochemistry suggests that mononuclear phagocytes, multinuclear osteoclasts and the vascular endothelium are involved in bone resorption in this disease.
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PMID:Cytochemical localization of alkaline and acid phosphatase in human vanishing bone disease. 369 22

Cytochemical studies were performed on peripheral blood from 30 patients with type 1 Gaucher disease. In 29 of the patients, peripheral blood monocytes stained positively for tartrate-resistant acid phosphatase, whereas monocytes from 18 normal individuals and 14 patients with monocytosis did not. In the Gaucher patients, the percentage of monocyte positivity for tartrate-resistant acid phosphatase ranged from 2 to 97. There was no correlation between the percent monocyte staining and the degree of disease severity, as measured by hepatosplenomegaly, pancytopenia, or extent of bone disease, for the group as a whole. In Gaucher patients who had not undergone splenectomy, however, there was a significant correlation between percent monocyte staining and the degree of hepatosplenomegaly, anemia, and thrombocytopenia. The presence of tartrate-resistant acid phosphatase may be secondary to the lysosomal accumulation of glucosyl ceramide within these monocytes, although this remains to be confirmed. If so, these circulating cells may represent precursors of the Gaucher cells in tissues. Tartrate-resistant acid phosphatase staining of peripheral blood monocytes may be useful as a diagnostic marker for Gaucher type 1 disease and for further studies on the pathogenesis of the disease.
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PMID:Tartrate-resistant acid phosphatase staining of monocytes in Gaucher disease. 401 24

Radiostrontium ((85)Sr) skeletal scintiscanning was done on 640 cases and 520 were included in a review. Forty-eight percent of 359 patients with biopsy-proved malignant disease had secondary skeletal involvement; in 17 percent the involvement was identified by scintiscanning alone. False-negative scintiscans were recorded in 0.9 percent. Unusual (85)Sr localization was found in a bone infarct, in proteus bursitis and in a pulmonary aspergillosis infiltrate. Serum alkaline phosphatase levels were found to be of little value in the evaluation for osseous metastasis, and normal acid phosphatase values in patients with prostatic carcinoma did not exclude the possibility of spread to the skeleton. Both the scintiscan and roentgenograms are essential in the evaluation of patients for metastatic bone disease.
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PMID:Review of a 5-year experience with the radiostrontium bone scintiscan. 503


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