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At least 469 records · Page 26Linked to original sources
PubMed1980

Extensive bone marrow infarction followed by myelofibrosis in patient with Ph' positive chronic granulocytic leukaemia.

A 28-year-old man with Philadelphia chromosome positive chronic granulocytic leukaemia developed extensive bone marrow and bone infarction which was associated with anaemia and thrombocytopenia. He survived 20 months from the first symptoms of bone marrow infarction; during this time he developed myelofibrosis and osteosclerosis followed by blastic transformation. Extensive bone marrow infarction is a possible pathogenetic mechanism when chronic granulocytic leukaemia is followed by myelofibrosis.

Adult↗
PubMed1980

The spectrum of radiographic bone changes in children with fluorosis.

Painful, crippling deformities in Tanzanian children from an area of endemic fluorosis are reported. Excessive fluoride ingestion in pregnant women may possibly poison and alter enzyme and hormonal systems in the fetus causing disturbances to osteoid formation and mineralization. Knock-knees, bowlegs, and saber shins develop when walking begins. Combinations of osteomalacia, osteoporosis, and osteosclerosis result in a spectrum of bone changes from an early age. Male hormones, and dietary and genetic deficiencies may aggravate individual response of actively growing bones. Some radiographic changes suggestive of rickets, hyperparathyroidism and thalassemia were observed.

Adolescent↗
PubMed1985

Disodium cromoglycate in the treatment of systemic mastocytosis involving only bone.

A 50-year-old woman with a 5-year history of low back pain and osteosclerosis was diagnosed as having systemic mastocytosis. She had no cutaneous or gastrointestinal manifestations of this disease. She was successfully treated with oral disodium cromoglycate. This is the first reported case of systemic mastocytosis involving only bone which responded to treatment with oral disodium cromoglycate.

Administration, Oral↗
PubMed2000

Mice lacking beta3 integrins are osteosclerotic because of dysfunctional osteoclasts.

Osteoclasts express the alphavbeta3 integrin, an adhesion receptor that has been implicated in bone resorption and that is therefore a potential therapeutic target. To assess the role of this heterodimer in skeletal development in vivo, we engineered mice in which the gene for the beta3 integrin subunit was deleted. Bone marrow macrophages derived from these mutants differentiate in vitro into numerous osteoclasts, thus establishing that alphavbeta3 is not necessary for osteoclast recruitment. Furthermore, the closely related integrin, alphavbeta5, does not substitute for alphavbeta3 during cytokine stimulation or authentic osteoclastogenesis. beta3 knockout mice, but not their heterozygous littermates, develop histologically and radiographically evident osteosclerosis with age. Despite their increased bone mass, beta3-null mice contain 3.5-fold more osteoclasts than do heterozygotes. These mutant osteoclasts are, however, dysfunctional, as evidenced by their reduced ability to resorb whale dentin in vitro and the significant hypocalcemia seen in the knockout mice. The resorptive defect in beta3-deficient osteoclasts may reflect absence of matrix-derived intracellular signals, since their cytoskeleton is distinctly abnormal and they fail to spread in vitro, to form actin rings ex vivo, or to form normal ruffled membranes in vivo. Thus, although it is not required for osteoclastogenesis, the integrin alphavbeta3 is essential for normal osteoclast function.

Animals↗
PubMed1993

Vocal granuloma, including sclerosis of the arytenoid cartilage: radiographic findings.

Vocal granulomas are uncommon benign tumors that may not respond readily to medical treatment or surgical removal. Gastroesophageal reflux is said to be a causative factor. Osteosclerosis of the arytenoid cartilage was found on computed tomographic scan in a series of 21 consecutive patients. The relationship of vocal granuloma to so-called contact ulcer (better called contact pachydermia) is discussed. Imaging of the granuloma and the arytenoid has little, if any, place in management, but should be recognized in the differential diagnosis of a vocal cord mass if the mass lies immediately adjacent to a sclerotic arytenoid.

Adult↗
PubMed1984

Radiography of healing dialysis osteodystrophy.

Characteristic skeletal changes of dialysis bone disease associated with multiple fractures were found in ten patients on prolonged regular (high aluminium) haemodialysis. A subsequent prospective investigation with serial radiography demonstrated healing approximately twelve months after a changed treatment regime. The treatment used was deionized or reverse osmosis water dialysis and renal transplantation. The typical osteomalacic and osteosclerotic changes and particularly metaphyseal sclerosis were found to be associated with more rapid clinical healing, whereas secondary hyperparathyroidism indicated delayed healing. It is suggested that plasma aluminium imbalance may lead to disturbance of bone formation with fractures, while healing is associated with normal serum aluminium levels and is manifested radiologically by osteosclerosis, particularly in the metaphyses.

Adult↗
PubMed2003

Autosomal dominant hyperostosis/osteosclerosis with high serum alkaline phosphatase activity.

We studied eight affected and four unaffected individuals from a Colombian family with autosomal dominant diffuse high bone density. Affected individuals have normal, proportional height and high serum alkaline phosphatase activity. Radiographically, affected members exhibit generalized, symmetrically diffuse endosteal hyperostosis of the long bones and skull with narrow medullary cavities and loss of the diploë, respectively. There is no periosteal reaction or decreased hematopoiesis. Furthermore, osteosclerosis affects vertebral bodies, ribs, pelvis, mandible, clavicles, and scapulae. Bone mineral density is 2.4-7.3 SD above the mean for age and gender in affected individuals. Affected vs. unaffected individuals' Z-scores were (mean +/- SD) 5.03 +/- 1.77 vs. 0.08 +/- 0.97, respectively, P = 0.0004). Three affected subjects older than 40 yr old lost bone mass in 6 yr. No dysmorphism, abnormal facial features, bone fractures, or cranial nerve involvement was found. The pattern of inheritance, the absence of asymmetries and malformations, the increased serum alkaline phosphatase, the peak bone mass that appears to decrease physiologically with age, and the involvement of cortical and trabecular bone suggest a new variant of hyperostosis/osteosclerosis that affects the entire skeleton.

Adult↗
PubMed2000

Novel mutations of the cathepsin K gene in patients with pycnodysostosis and their characterization.

Pycnodysostosis is a rare autosomal recessive skeletal dysplasia characterized by short stature, osteosclerosis, acroosteolysis, bone fragility, and skull deformities. Recently, mutations in the gene encoding cathepsin K (CK), a lysosomal cysteine protease localized exclusively in osteoclasts, were found to be responsible for this disease. We analyzed genomic DNA from four unrelated Japanese patients with this disorder and identified three different mutations of their CK genes: a previously reported missense mutation (A277 V), a novel single base deletion mutation (531 del T) causing a frame shift from codon 142 that results in a premature termination codon, and a novel missense mutation (L9P) in the signal peptide region. To investigate whether the L9P mutation disrupts signal peptide function and decreases protein synthesis, mutant and wild-type CK complementary DNAs driven by the cytomegalovirus promoter were transfected into COS-7 cells, and their gene products were detected by immunohistochemistry and Western blotting. Expression of the mutant protein was markedly reduced, suggesting decreased mature CK production in this patient, which may have been due to dysfunction of the signal peptide. These results provide evidence that a structural change in the signal peptide of the CK protein was involved in the pathogenesis of pycnodysostosis.

Adult↗
PubMed1998

Health effects of indoor fluoride pollution from coal burning in China.

The combustion of high fluoride-content coal as an energy resource for heating, cooking, and food drying is a major exhaust emission source of suspended particulate matter and fluoride. High concentrations of these pollutants have been observed in indoor air of coal-burning families in some rural areas in China. Because airborne fluoride has serious toxicological properties, fluoride pollution in indoor air and the prevalence of fluorosis have been analyzed in a fluorosis area and a healthy nonfluorosis area in China and in a rural area in Japan. For human health, fluoride in indoor air has not only been directly inhaled by residents but also has been absorbed in stored food such as corn, chilies, and potatoes. In the fluorosis area in China, concentrations of urinary fluoride in the residents have been much higher than in the nonfluorosis area in China and in the rural area in Japan. In the fluorosis area, almost all elementary and junior high school students 10-15 years of age had dental fluorosis. Osteosclerosis in the skeletal fluorosis patients was very serious. Urinary deoxypyridinoline in rural residents in China was much higher than in rural residents in Japan. Data suggest that bone resorption was extremely stimulated in the residents in China and that fluoride may stimulate both bone resorption and bone formation. Because indoor fluoride from combustion of coal is easily absorbed in stored food and because food consumption is a main source of fluoride exposure, it is necessary to reduce airborne fluoride and food contamination to prevent serious fluorosis in China.

Adolescent↗
PubMed2000

Expansile skeletal hyperphosphatasia: a new familial metabolic bone disease.

We describe a new familial metabolic bone disease characterized by expanding hyperostotic long bones, early onset deafness, premature tooth loss, and episodic hypercalcemia. The condition affects a mother and daughter studied at the age of 36 years and 11 years, respectively. Both individuals lost all hearing in early childhood and suffered premature shedding of teeth. Skeletal pains began just before puberty. Swelling and aching of most middle phalanges in the hands is an especially troublesome manifestation. The mother also had episodes of symptomatic hypercalcemia first documented in late childhood and subsequently during intercurrent illness and postpartum lactation. Radiographs show hyperostosis and/or osteosclerosis predominantly in the skull and appendicular skeleton. Long bones also are expanded considerably, especially the middle phalanges in the fingers. The mother's skeletal abnormalities are more severe. Biochemical parameters of bone turnover, including serum alkaline phosphatase (ALP) activity, are elevated substantially. In the proposita, dynamic histomorphometry of nondecalcified sections of iliac crest revealed rapid skeletal remodeling. In the mother, who had been treated with bisphosphonates, electron microscopy (EM) showed disorganized collagen bundles as well as necrotic and apoptotic bone cells but no osteocytic osteolysis. Measles virus gene transcripts were not detected in peripheral blood monocytes. Karyotyping was normal, 46,XX. Hyperphosphatasia with bone disease previously has been reported as either a sporadic or autosomal recessive condition. Expansile skeletal hyperphosphatasia (ESH) is probably inherited as an autosomal dominant trait with a high degree of penetrance.

Adult↗
PubMed1997

A study on the biological significance of midregion and intact parathyroid hormone in hemodialysis patients.

In this study, we investigated the relationship between the concentrations of intact parathyroid hormone (i-PTH) and midregion PTH (m-PTH) measured by an immunoradiometric assay and a radioimmunoassay, respectively, versus various demographic and biochemical parameters, bone mineral density (BMD) of the lumbar spine (LS) and radius, and the radiographic findings of osteosclerosis and aortic calcification in hemodialysis (HD) patients. m-PTH correlated positively and more significantly with serum calcium (Ca), serum phosphorus (P), Ca-P solubility products (Ca x P) and LS-BMD than i-PTH did (P = 0.024 vs. 0.531, 0.001 vs. 0.061, 0.0001 vs. 0.125, and 0.017 vs. 0.284, respectively). A positive correlation between the percent changes in serum P over the 1-month measurement period and those in m-PTH rather than in i-PTH was also observed (P = 0.021 vs. 0.869). These data indicate than m-PTH is distinct from i-PTH in its positive correlation with serum Ca, serum P, Ca [symbol: see text] P and LS-BMD in HD patients. Since m-PTH is known to consist mostly of the midregion and carboxyl-terminal fragments of PTH in HD patients, the present study suggests that these PTH fragments may be biologically significant in the patients in vivo.

Adult↗
PubMed1975

Renal osteodystrophy in patients on hemodialysis as reflected in the bony pelvis.

Roentgen studies in a group of 131 patients on maintenance hemodialysis reveal renal osteodystrophy in 25 patients (19 per cent). In 24 patients of the latter group (98 per cent), the various manifestations of renal osteodystrophy appear in the bony pelvis. These include: osteomalacia; osteitis fibrosa; osteosclerosis; dialysis osteopenia; and extraosseous calcifications. Some of the changes, i.e., bending of bone, Looser's lines, metaphyseal fractures and dialysis osteopenia, are due, at least in part, to the weight bearing nature of the bony pelvis. These findings are ararely, if ever, seen in the digits or in other non-weight bearing bones. It is therefore proposed to evaluate the bony pelvis, together with the magnification digital roentgenogram, when a less costly and less time consuming skeletal survey is needed in hemodialized patients.

Adolescent↗
PubMed1988

Spinal chordoma: radiologic features in 14 cases.

The radiologic appearance of chordoma of the cervical (three patients), thoracic (four patients), and lumbar spine (seven patients) was studied. Eleven patients were over 50 years old and presented with long-standing back pain. All were examined with conventional radiographs; three cases also had CT examinations. In thirteen patients, the tumor originated in the vertebral body and, in one patient, in the posterior element of a vertebra. In nine (64%) of the 14 cases, osteosclerosis was a prominent feature. In the remaining five cases (36%), the bone lesion was purely osteolytic. Involvement of the intervertebral disk was found in three patients; in two of these the tumor extended to an adjacent vertebra. In nine patients, a soft-tissue mass was a distinctive additional feature. A sclerotic and/or osteolytic lesion in a vertebral body with a large, paraspinal soft-tissue mass in an older patient with long-standing back pain should raise the possibility of a chordoma.

Adolescent↗
PubMed1989

A clinicoradiological study of maxillary osteomyelitis.

Eleven cases of maxillary osteomyelitis were studied clinically and radiologically. Clinically, the molar area was most affected. The average age of the patients was 47 years and there was no sex difference. Radiologically, the spotty type of osteolytic change was the most frequent, and osteosclerosis was rare. Sequestrum was not a clear feature radiologically. The features of maxillary and mandibular osteomyelitis were compared and the findings discussed.

Adult↗
PubMed1994

Evolution of fibro-osteosclerotic bone marrow lesions in primary (idiopathic) osteomyelofibrosis--a histomorphometric study on sequential trephine biopsies.

Evolution of fibro-osteosclerotic bone marrow lesions in the course of primary (idiopathic) osteomyelofibrosis (OMF) was studied in 36 patients (17 males, 19 females; median age 57 years) by morphometric evaluation of sequential trephine biopsies. The mean interval between first and terminal examination was 33 months (range 6 to 121 months). Two biopsies were performed in 31 and three and more in five patients. Morphometry consisted of a determination of argyrophilic (reticulin and collagen) fiber density, measured per area of hematopoiesis or marrow cellularity, and the calculation of the extent of trabecular bone tissue. In 13 of our 36 patients increase in reticulin and collagen deposits was only borderline to minimal during the observation period. On the other hand, in 23 of the 36 patients a slight to gross accumulation of reticulin and collagen fibers, partially associated with osteosclerotic changes was recognizable. No regression of fibrosis was encountered in our cohort of patients which included three cases with preceding low-dose busulfan therapy. Analysis of the different lengths of intervals between the first and the last biopsy and degree of fibrosis as well as osteosclerosis, suggested that alterations developed progressively, however, at an unpredictable and considerably varying rate. Thus our findings were not in keeping with several studies on smaller series of patients, which generally contested a progression of fibro-osteosclerotic lesions in OMF and additionally reported reversal of the pathology following chemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Biopsy↗
PubMed1999

Thrombin, but not bradykinin, stimulates proliferation in isolated human osteoblasts, via a mechanism not dependent on endogenous prostaglandin formation.

Osteolysis or osteosclerosis often occurs in bone tissue adjacent to chronic inflammatory processes. Numerous cytokines and inflammatory mediators have been implicated as osteoclast-activating agents, explaining inflammation-induced bone resorption. In many cases, the cause of the sclerosis seen in these lesions is less thoroughly investigated. We have studied the effects of thrombin and bradykinin, 2 inflammatory mediators, on the rate of proliferation in isolated human osteoblasts (hOBs). Thrombin, at and above 1 U/mL, stimulated the rate of thymidine incorporation into hOBs. The absolute cell number also increased, as measured by an assay based on the detection of cell metabolism. A synthetic peptide ligand for the thrombin receptor enhanced the rate of [3H]thymidine incorporation in hOBs, indicating that thrombin-induced proliferation is mediated via the tetheric thrombin receptor. The thrombin-induced proliferation was not affected by indomethacin, excluding prostanoids as mediators of this effect. Bradykinin did not affect either the rate of thymidine incorporation, or number of cells in long-term cultures of hOBs. In conclusion, the inflammatory mediator, thrombin, stimulates proliferation in isolated human osteoblasts probably via the recently described G-protein-coupled tetheric thrombin receptor. Thrombin may therefore be involved as a mediator of inflammation-induced sclerosis and bone formation.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1978

Subacute fluorosis: a consequence of abuse of an organofluoride anesthetic.

A young woman presented with a novel multisystem disease: painful periostitis, osteosclerosis, hypertension, and renal dysfunction. The similarity of some of this clinical picture to fluoride intoxication led to the discovery of massively elevated fluoride levels in serum, urine, and bone. Although initially an enigma, the source of fluoride was later found to be the illicit use of an anesthetic agent, methoxyflurane. This agents is one of a class of organofluorides that, by virtue of biotransformation, is a known cause of inorganic fluoride exposure. Though the drug is potentially nephrotoxic as generally used, exposure to it is transient and has not previously led to discernible bone disease.

Acute Disease↗
PubMed1998

[A new location of osteochondritis: the os trapezium. Apropos of a case].

The authors report a case of osteochondritis dissecans of the trapezium in a 10 year old boy. The main symptom was pain. X-Rays showed osteosclerosis and diminished volume of the trapezium. Magnetic resonance imaging confirmed the diagnosis. At six months, the patient was almost asymptomatic and at one year, X-Rays and the MRI were judged normal. This localisation has never been reported before. The treatment was conservative. Remission was complete.

Carpal Bones↗