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At least 433 records · Page 24Linked to original sources
PubMed2000

Erdheim-Chester disease with intramuscular lipogranuloma.

We report on a rare manifestation of Erdheim-Chester disease with intramuscular lipogranuloma. The patient was a 66-year-old man who noted a soft tissue mass in the right quadriceps femoris muscle. Radiographs revealed symmetrical osteosclerosis in the diametaphysis of both femora and tibiae. An open biopsy revealed a proliferation of lipid-laden histiocytes in the femoral bone marrow and the quadriceps femoris muscle. To our knowledge, this is the second case of Erdheim-Chester disease involving muscle.

Aged↗
PubMed2000

Imaging spectrum in sclerotic myelomas: an experience of three cases.

The classic radiographic presentation of multiple myeloma is lytic skeletal lesions. Primary sclerotic manifestations are rare and occur only in 3% of cases. The imaging spectrum in three cases of multiple myeloma with primary osteosclerosis is described. The first patient had spiculated sclerosis of the orbit, which is an uncommon site for myeloma. The second patient with POEMS syndrome had multiple, scattered, skeletal lesions with sclerotic margins. The third patient presented with a chest wall mass and had an expansile thick spiculated sclerosis in the rib. The wide imaging spectrum possible in sclerotic myelomas and their relevant differential diagnosis is emphasized.

Adult↗
PubMed1999

Imaging findings in patients with myelofibrosis.

The purpose of this review is to illustrate the wide range of radiological abnormalities in myelofibrosis. Myelofibrosis, also called myeloid metaplasia, is a myeloproliferative disorder of unknown etiology. The common imaging findings in patients with myelofibrosis are osteosclerosis, hepatosplenomegaly, and lymphadenopathies. In addition, extramedullary hematopoiesis may develop in multiple sites such as chest, abdomen, pelvis, and central nervous system, simulating malignant disease. Selected plain-film, CT, and MR images in patients with myelofibrosis are shown as pictorial essay to allow ready recognition of the most common imaging abnormalities of the disease.

Bone and Bones↗
PubMed2001

Neuropathology of Raine syndrome.

We present three cases of Raine syndrome occurring in siblings of consanguineous parents. Raine syndrome is characterised by generalised osteosclerosis with craniofacial anomalies and intracranial calcifications. So far, only nine cases have been reported, and no evaluation of the distribution and extent of the cerebral mineralisations, as well as their impact on the surrounding tissue, has been undertaken yet. In our cases, calcifications were unevenly distributed throughout the central nervous system, not associated with neuronal loss or dystrophic events and appeared mostly as single calcospherites within the neuropil with occasional confluent deposits at advanced gestational age. There was intense perifocal microgliosis around single immature calcospherites, as well as mild astrogliosis around and within the confluent lesions, in which occasional macrophages could be found. Rarely, mineralisations occurred in blood-vessel walls, mainly affecting basal ganglia. Preferential sites of calcification were parietal and occipital periventricular white matter and corpus callosum, while frontal lobes were mildly affected. The cortex, temporal lobes as well as internal capsule, brain stem, cerebellum, leptomeninges, pituitary gland and choroid plexus were devoid of mineralisations. The subcortical grey matter was moderately involved in the putamen and pallidum, mildly in the caudate nucleus and subependymal germ cell matrix and not at all in the thalamus, Ammon's horn, amygdala and substantia nigra. The distribution of mineral deposits was thus inversely correlated to regional blood circulation and capillary density, with calcifications being concentrated in more sparsely perfused areas but lacking in highly vascularised tissue. This inverse relationship between mineralisation and regional blood flow was reflected in the varying distribution of calcospherites in grey and white matter as well as in the white matter of different lobes.

Brain Diseases↗
PubMed1976

The actions of parathyroid hormone on bone: relation to bone remodeling and turnover, calcium homeostasis, and metabolic bone disease. Part IV of IV parts: The state of the bones in uremic hyperaparathyroidism--the mechanisms of skeletal resistance to PTH in renal failure and pseudohypoparathyroidism and the role of PTH in osteoporosis, osteopetrosis, and osteofluorosis.

In early chronic renal failure, the state of the bones resembles that of type II primary hyperparathyroidism. Cortical bone becomes thinner and more porous, and there is increased extent of surface remodeling. These changes are followed in turn by osteomalacia and osteitis fibrosa, although sometimes these may be alternate rather than successive stages. Bone turnover is less than would be expected for the elevation of PTH level, probably because of 1,25 (OH)2D3 deficiency. The resorption velocity and lamellar bone appositional rates are depressed, but woven bone appositional rate may be increased, possibly because of hyperphosphatemia. Bone mass reflects the summation of three independent processes: loss of lamellar bone due to hyperparathyroidism (depending on the extent of insulation by osteoid); accumulation of partly mineralized osteoid because of osteomalacia; accumulation of woven bone because of osteitis fibrosa. Osteosclerosis may be growth-related metaphyseal, subchondral or diffuse axial, and periosteal neostosis may also occur. Some patients on hemodialysis lose bone because of planing rather than lacunar or dissecting resorption, combined with depression of both lamellar and woven bone formation. Hyperparathyroid bone disease tends to improve slowly after renal transplantation. Persistent hypocalcemia reflects a defect in the calcium homeostatic system and cannot be explained solely by the known stimuli to secondary hyperparathyroidism. The increment in plasma calcium in response to PTH infusion is subnormal, both in early chronic and in acute renal failure, probably because of 1,25(OH)2D3 deficiency. This is also the most likely explanation for the depressed level of blood-bone equilibrium. The activity of all three of the PTH responsive cell systems in bone is depressed in renal failure, probably because all three require 1,25(OH)2D3 in order to function normally. In pseudohypoparathyroidism, as in chronic renal failure, hypocalcemia results from a defect in the regulation of the blood-bone equilibrium. The bone-remodeling system shows all gradations of response, from slight depression of bone turnover to overt osteitis fibrosa, but bone turnover is never as low as in PTH deficiency. These differences may reflect the presence or absence of resistance to PTH of the osteoprogenitor cell as well as of the calcium homeostatic system, or may be due to varying degrees of 1,25(OH)2D3 deficiency, as in chronic renal failure. An increase in plasma calcium in response to PTH can occur either in the untreated state or after treatment with vitamin D because either the error-correcting or remodeling system remains responsive to PTH. Pseudohypoparathyroidism may be subdivided into three types, depending on whether the urinary cyclic-AMP response to PTH remains defective despite treatment with vitamin D, improves with treatment, or is normal before treatment. Only the former is associated with the genetic syndrome of Albright's hereditary osteodystrophy...

Bone Diseases↗
PubMedDate not supplied

Computerized tomography of the Cupid's bow contour of the lumbar spine.

The inferior endplates of the third, fourth, and fifth lumbar-vertebral bodies frequently have paired parasagittal concavities when viewed in the frontal projection. In the lateral projection, these concavities lie posteriorly and are superimposed. This has been called Cupid's bow contour of the spine. On CT, Cupid's bow is visualized in an axial plane as two rounded relatively well-circumscribed areas of hypodensity rimmed by apparant osteosclerosis in the posterior half of the vertebral bodies. This, on occasion, may resemble metastasis or other pathology of the spine. The CT features, however, once appreciated, are characteristic of this normal variant, helping to distinguish it from other contour deformities of the vertebrae having clinical significance. The CT features are described and the incidence is discussed in 100 patients.

Adolescent↗
PubMed1987

Failure of normal osteoclasts to resorb calcified cartilage from osteosclerotic (oc/oc) mice in vitro.

Osteosclerosis is an osteopetrotic mutation in the mouse characterized by reduced bone resorption, numerous small osteoclasts lacking elaborate ruffled borders, and resistance to cure by bone marrow transplants from normal littermates. The failure of osteosclerotic mice to be cured by bone marrow transplants could be due to the production of bone that is not resorbable by normal osteoclasts. We tested this hypothesis using a modification of the metatarsal organ culture system of Burger et al. (1982), in which metatarsals are cultured with various tissues that act as sources of osteoclast precursors. Metatarsals from neonatal mutants were isolated, and live bone rudiments were cultured with small cubes of liver or spleen from normal littermates for 7 days. Controls included normal and mutant metatarsals cultured alone or with spleen or liver from littermates of the same or different genotype. Mutant metatarsals cultured alone or with mutant tissue had small osteoclasts, no marrow spaces, and no evidence of bone resorption. Mutant metatarsals cultured with normal liver or spleen had larger osteoclasts, evidence of resorption of bone but not cartilage, and no marrow spaces because the calcified cartilage cores of metaphyseal trabeculae persisted. Normal metatarsals cultured with normal liver had large osteoclasts, bone resorption, and marrow spaces. By transmission EM, mutant trabeculae contained a layer of amorphous material between the central core of calcified cartilage and the surrounding bone matrix. This material was not present in normal metatarsals.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMedDate not supplied

Osteomesopyknosis: report of a new case with bone histology.

A new case of osteomesopyknosis, a rare autosomal dominant axial osteosclerosis is reported, with 4 affected members of the same family. Biochemical investigations, bone mineral content (BMC) measurement, 99mTc HMDP bone scan and microscopy of iliac crest bone and femoral head have been performed on 1 subject. A marked increase of BMC was found, without abnormality of biochemical data. Microscopy of bone showed an increase of trabecular thickness, and a low rate of bone turnover. No abnormality of mineralization was found on microradiographs.

Bone and Bones↗
PubMed2006

Pathogenesis of myelofibrosis with myeloid metaplasia: Insight from mouse models.

Myelofibrosis with myeloid metaplasia or idiopathic myelofibrosis is a myeloproliferative disease. It is known to be a stem-cell disorder that leads to a secondary and reactive stromal reaction in the bone marrow microenvironment that is responsible for impaired haematopoiesis. Although progress has been made in the elucidation of the pathogenesis of idiopathic myelofibrosis, lack of suitable models has limited our understanding of the pathology. The aim of this chapter is to address recent inferred new insights in mouse models into the pathogenesis of osteomyelofibrosis. These insights outline the role of transforming growth factor-beta1 and osteoprotegerin in the promotion of myelofibrosis and osteosclerosis, respectively, paying special regard to the role of abnormal megakaryocyte proliferation and maturation.

Animals↗
PubMed1997

Lack of vacuolar proton ATPase association with the cytoskeleton in osteoclasts of osteosclerotic (oc/oc) mice.

We examined the pathogenetic mechanism underlying the lack of bone resorption in osteosclerotic oc/oc mice. An immunoelectron microscopic analysis revealed that in the osteoclasts of these mice, no ruffled borders formed, and that vacuolar H+-ATPase (V-ATPase) was present throughout the cytoplasm but not on the apical membranes. The activity of V-ATPase in oc/oc mice was similar to that in normal mice. In normal spleen cell-derived osteoclast-like cells (OCLs), immunoreactivity for V-ATPase was detected in association with Triton X-100-insoluble cellular structure, but not in oc/oc spleen cell-derived OCLs. Moreover, in renal proximal convoluted tubules of oc/oc mice, the basal striation did not form. These results suggest that osteosclerosis in oc/oc mice is possibly due to the dissociation of V-ATPase and cytoskeleton in osteoclasts.

Adenosine Triphosphatases↗
PubMed1986

[Erdheim-Chester disease. The multiviceral form presenting as exophthalmos].

Erdheim-Chester disease is a rare visceral xantho-granulomatosis, the 17th case of which is reported here. The initial symptom, bilateral exophthalmos, was uncommon. The picture was completed by a retroperitoneal xanthogranuloma and by bilateral and symmetrical osteosclerosis of the long bones. The lack of X-bodies at electron microscopy differentiated the disease from Hand-Schüller-Christian disease. The patient's condition improved with chemotherapy (vinblastine and doxorubicin) combined with corticosteroid therapy. After a 5-month remission period, he died of an intercurrent infection. No autopsy was performed.

Diagnosis, Differential↗
PubMed1996

Osteosclerotic myeloma and 'POEMS' syndrome.

Metabolic bone disease is a major cause of morbidity and mortality in patients suffering from multiple myeloma. This usually results from an imbalance between the osteoclast and osteoblast activity of bone resorption and formation due to the secretion of an osteoclast-activating factor by the myeloma cells. This generally takes the form of lytic lesions, hypercalcaemia and osteoporosis but, in a minority of patients, osteosclerosis is a striking feature. In a proportion of patients with gammopathy and the osteosclerotic form of the disease, there appears to be an association with other symptom complexes including Polyneuropathy, organomegaly, endocrine changes, and skin abnormalities. This article summarizes the clinical, radiological and laboratory features of this syndrome, which is known by the acronym 'POEMS'. It is important to recognize this condition, as it carries a better prognosis than the more common lytic form of the disease and because vincristine, and agents frequently used to treat this condition, may severely exacerbate the neuropathy and should be avoided. This syndrome is also important because it may give some insight into the pathophysiology of the plasma-cell dyscrasias.

Humans↗
PubMed1987

Fluoride-induced chronic renal failure.

Renal fluoride toxicity in human beings is difficult to assess in the literature. Although experimental studies and research on methoxyflurane toxicity have shown frank renal damage, observations of renal insufficiency related to chronic fluoride exposure are scarce. We report a case of fluoride intoxication related to potomania of Vichy water, a highly mineralized water containing 8.5 mg/L of fluoride. Features of fluoride osteosclerosis were prominent and end-stage renal failure was present. The young age of the patient, the long duration of high fluoride intake, and the absence of other cause of renal insufficiency suggest a causal relationship between fluoride intoxication and renal failure.

Adult↗
PubMed2000

Gelatinous bone marrow transformation in a case of idiopathic myelofibrosis: a morphological paradox.

A 74-year-old woman with severe, but asymptomatic weight loss was found to have splenomegaly, thrombocytosis, and neutrocytosis. A trephine biopsy showed a hypercellular bone marrow with a proliferation of abnormal megakaryocytes, mild reticulin fibrosis, and osteosclerosis, i.e., features of an early stage of idiopathic myelofibrosis. In contrast to this predominant pattern, the marrow also contained a distinct hypocellular focus of gelatinous transformation (GMT) in which hematopoietic and fat cells were replaced by gelatinous substances that were characterized as hyaluronic acid mucopolysaccharides histochemically. GMT is a rare disorder of unknown pathogenesis, and is an unspecific indicator of severe illness which most often occurs in patients with a background of weight loss. This is the first reported case of gelatinous changes in an otherwise fibrotic bone marrow, a constellation which a priori seemed to be incompatible.

Adipose Tissue↗
PubMed1996

Gigantic dense bone island of the jaw.

The clinical and radiographic features of gigantic dense bone islands of the jaw were reviewed in 21 subjects to better determine the pathogenesis of this condition. Most of the islands were asymptomatic. They showed a striking predilection for occurrence in the premolar to molar region of the mandible. The greatest dimension of gigantic dense bone islands measured on panoramic radiographs ranged from 2.5 to 7.0 cm, and they were most commonly structureless radiopaque areas. None showed bony expansion buccolingually, nor did they displace adjacent teeth or bony anatomic structures. It is concluded that gigantic dense bone island is not a benign bone neoplasm and is perhaps merely a large counterpart of smaller dense bone islands or idiopathic osteosclerosis.

Adolescent↗
PubMed2004

Multiple myeloma presenting with widespread osteosclerotic lesions.

Sclerotic lesions are rare in malignant monoclonal gammopathies, although they are occasionally associated with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes). In most cases, osseous lesions in POEMS syndrome present as an isolated sclerotic deposit or a combination of both lytic and sclerotic lesions. Diffuse osteosclerosis is extremely rare and may lead to the diagnosis of multiple myeloma, classically known to present as lytic lesions in the skeleton, with or without diffuse osteoporosis. We report a 74-year-old woman with widespread and substantial osteosclerotic lesions, associated with IgA-lambda myeloma, and with no other criteria for POEMS syndrome, and who was rapidly diagnosed with compression of the spinal cord. Detailed knowledge of imaging features in myeloma emphasises the need to consider plasma cell neoplasm in the differential diagnosis of any pattern of bone sclerosis. Although exceptional, multiple myeloma must be borne in mind in the presence of diffuse bone sclerosis.

Aged↗
PubMed2000

The gene encoding the mouse homologue of the human osteoclast-specific 116-kDa V-ATPase subunit bears a deletion in osteosclerotic (oc/oc) mutants.

Osteosclerosis (oc) is an autosomal recessive lethal mutation that impairs bone resorption by osteoclasts, and induces a general increase of bone density in affected mice. Genetic mapping of the oc mutation was used as a backbone in a positional cloning approach in the pericentromeric region of mouse chromosome 19. Perfect cosegregation of the osteopetrotic phenotype with polymorphic markers enabled the construction of a sequence-ready bacterial artificial chromosome (BAC) contig of this region. Genomic sequencing of a 200-kb area revealed the presence of the mouse homologue to the human gene encoding the osteoclast-specific 116-kDa subunit of the vacuolar proton pump. This gene was located recently on human 11q13, a genomic region conserved with proximal mouse chromosome 19. Sequencing of the 5' end of the gene in oc/oc mice showed a 1.6-kb deletion, including the translation start site, which impairs genuine transcription of this subunit. The inactivation of this osteoclast-specific vacuolar proton ATPase subunit could be responsible for the lack of this enzyme in the apical membranes of osteoclast cells in oc/oc mice, thereby preventing the resorption function of these cells, which leads to the osteopetrotic phenotype.

Amino Acid Sequence↗
PubMed2000

Overexpression of DeltaFosB transcription factor(s) increases bone formation and inhibits adipogenesis.

Members of the AP-1 family of transcription factors participate in the regulation of bone cell proliferation and differentiation. We report here a potent AP-1-related regulator of osteoblast function: DeltaFosB, a naturally occurring truncated form of FosB that arises from alternative splicing of the fosB transcript and is expressed in osteoblasts. Overexpression of DeltaFosB in transgenic mice leads to increased bone formation throughout the skeleton and a continuous post-developmental increase in bone mass, leading to osteosclerosis. In contrast, DeltaFosB inhibits adipogenesis both in vivo and in vitro, and downregulates the expression of early markers of adipocyte differentiation. Because osteoblasts and adipocytes are thought to share a common precursor, it is concluded that DeltaFosB transcriptionally regulates osteoblastogenesis, possibly at the expense of adipogenesis.

Adipocytes↗