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At least 73 records · Page 4Linked to original sources
PubMed1990

Metastatic calcification within bone. The main cause of osteosclerosis in hypervitaminosis D3. Radiologic-pathologic correlation.

Because the pathogenesis of osteosclerosis in hypervitaminosis D is still not well elucidated, the authors experimentally studied hypervitaminosis D3 in 66 rabbits by injecting different doses of vitamin D3. Contact radiographs of bone specimens showed various signs of osteosclerosis, including dense epiphyses and metaphyses, thickened bony articular surfaces, dense metaphyseal bands, modeling defects at the metaphysis, and dense and thickened cortical bone. The corresponding pathologic sections showed that conspicuous metastatic calcification coated the trabeculae and filled bone marrow cavity and caverns in the original but porotic cortical and periosteal new bone. Rather than being resorbed, the metastatic calcifications were embedded in a thick layer of newly formed bone 6 to 14 weeks after vitamin D3 withdrawal. This study suggests that in hypervitaminosis D3, the osteoblasts and bone marrow undergo degeneration, leading to necrosis and calcification. After vitamin D3 withdrawal, osteoblasts reappear and become overactive, leading to overossification.

Animals↗
PubMed1989

Diffuse osteosclerosis in hairy cell leukemia.

We describe two patients with a new clinical pathologic syndrome of diffuse osteosclerosis in association with hairy cell leukemia. In both patients bone marrow biopsies could not be obtained due to extremely hard bones and inability to insert the biopsy needle; neither patient had a history of bony pain or fracture. The osteosclerotic process in one patient stabilized after successful treatment of her hairy cell leukemia with interferon alpha and deoxycoformycin suggesting that the osteosclerosis observed was related to the underlying malignant disease. Possible etiologic mechanisms are discussed.

Adult↗
PubMed1977

[Acro-osteosclerosis in the course of sarcoidosis (author's transl)].

The authors report two cases of acro-osteosclerosis occurring during sarcoidosis. Condensation lesions involve above all the distal and proximal phalanges of the hands. Comparison of these cases with those in the literature indicates that these particular types of bone changes in association with sarcoidosis are more common than indicated in the studies of Jüngling (occurrence estimated at 54% of subjects suffering from sarcoidosis with bone localisations). This acro-osteosclerosis is however not specific to sarcoidosis. It is seen in patients suffering from rheumatoid arthritis, Hodgkin's disease and other haematological disorders. It has also been seen in normal individuals in X-rays taken at the time of a traumatic accident.

Arthritis, Rheumatoid↗
PubMed1999

Osteosclerosis, hypoplastic nose, and proptosis (Raine syndrome): further delineation.

We describe a newborn girl with a lethal sclerosing bone dysplasia leading to prenatal skeletal alterations and microcephaly, proptosis, hypoplastic nose and midface, small jaw, cleft palate, hypertrophied gums, intracranial calcifications, and generalized osteosclerosis. There is a remarkable similarity between our patient and six previously reported infants subsequently categorized as having a distinct entity: Raine syndrome. Autosomal recessive inheritance is postulated based on parental consanguinity in several of the previous cases and in our patient.

Brain Diseases↗
PubMed2004

Interleukin-11 as an osteoprotegerin-inducing factor in culture medium of blastic cells from a patient with acute megakaryocytic leukemia complicated with osteosclerosis.

We came across a rare case of acute megakaryocytic leukemia, the clinical course of which was relatively chronic and nonaggressive. This case was complicated with generalized severe osteosclerosis (OS). The medium in which blastic cells from the patient were cultured showed a strong activity to enhance the expression of an osteosclerotic cytokine, osteoprotegerin (OPG), as revealed by real-time quantitative RT-PCR and Western blot analysis. The OPG-inducing activity of the culture medium was neutralized by the anti-interleukin-11 (IL-11) antibody. These results indicate that IL-11 produced by the blasts was a causative factor of the OS observed in this patient.

Adult↗
PubMed1978

Mental retardation and osteosclerosis.

We report a girl with profound mental retardation who, at 3 years of age, began to show a progressive osteosclerosis on bone roentgenograms. The bony changes were slightly suggestive of osteopetrosis from which they differed by a number of unusual features.

Adolescent↗
PubMed1995

Autosomal dominant osteosclerosis type Stanescu: the third family.

We describe a family with Stanescu osteosclerosis. The propositus and his mother were short and had cortical sclerosis of the long bones, deficient facial sinus development, cranial bone malformations, and normal intelligence. To the best of our knowledge, only two such families have been described previously. The autosomal dominant pattern of inheritance of this skeletal dysplasia is reinforced, as there are many other reportedly affected relatives, including the maternal grandfather, uncles, and aunts of the propositus. The findings of wormian bones and calcification of the falx, not previously described, may be added to the phenotype.

Adult↗
PubMed1999

A novel putative transporter maps to the osteosclerosis (oc) mutation and is not expressed in the oc mutant mouse.

The phenotype of mice homozygous for the osteosclerosis (oc) mutation includes osteopetrosis, and a variety of studies demonstrate that osteoclasts in these mice are present but nonfunctional. We have identified a novel gene that has homology to a family of 12-transmembrane domain proteins with transport functions and maps to proximal mouse chromosome 19, in a region to which the oc mutation has been previously assigned. The putative transporter is abundant in normal kidney, but its expression is markedly reduced in kidneys from oc/oc mice when tested using Northern and Western analyses. Southern analysis of this gene, which we call Roct (reduced in oc transporter), demonstrates that it is intact and unrearranged in oc/oc mice. In situ studies show that Roct is expressed in developing bone. We propose that the absence of Roct expression results in an osteopetrosis phenotype in mice.

Amino Acid Sequence↗
PubMed1982

Acute megakaryoblastic leukemia (acute "malignant" myelofibrosis): an unusual cause of osteosclerosis.

Acute megakaryoblastic leukemia or acute "malignant" myelosclerosis is an acute and rapidly progressive myeloproliferative syndrome characterized by minimal or absent splenomegaly, pancytopenia, diffuse marrow fibrosis, and circulating blasts of megakaryocytic origin. The disease must be differentiated from other hematologic malignancies especially myelofibrosis with myeloid metaplasia. The radiographic changes of osteosclerosis in our patient have not been previously reported in the literature.

Acid Phosphatase↗
PubMed2005

Hepatitis C-associated osteosclerosis (HCAO): report of a new case with involvement of the OPG/RANKL system.

We report a new case of hepatitis C-associated osteosclerosis (HCAO). The clinical presentation of the patient was an acquired deep severe bone pain with increased serum bone alkaline phosphatase activity (up to 12 times the upper limit of normal), and generalized bone sclerosis, temporally related to the hepatitis C-virus (HCV) infection. We documented in this patient an increase of circulating osteoprotegerin (OPG), and a concentration of circulating receptor activator for nuclear factor-kB ligand (RANKL) below the lower limit of the reference range. The observed abnormalities of the OPG/RANKL system may contribute to the maintenance of the positive balance of bone remodeling that characterizes patients with HCAO.

Aged↗
PubMed2004

Central osteosclerosis with trichothiodystrophy.

Trichothiodystrophy (TTD) is a rare, autosomal recessive, multisystem disorder associated with defects in nucleotide excision repair. We report a 7-year-old boy with TTD due to mutation in the XPD gene. The patient has classic features of this condition, including brittle, sulphur-deficient hair, ichthyosis, growth retardation and developmental delay. In addition, he has radiological evidence of progressive central osteosclerosis. Although similar radiological findings have previously been reported in a small number of patients, this association is not widely recognised. We review the radiological findings in this and other similar cases and discuss the natural history of these bony changes.

Abnormalities, Multiple↗
PubMed1992

The significance of idiopathic osteosclerosis found in panoramic radiographs of sporadic colorectal neoplasia patients and their relatives.

Osteosclerotic lesions in the mandible are reported with increased frequency in patients with colorectal neoplasia. Panoramic radiography has been suggested to detect high-risk patients among relatives of patients with hereditary adenomatous disease and sporadic colorectal cancer. The present study was undertaken to compare the incidence of idiopathic osteosclerotic lesions in patients with sporadic colorectal cancer and their first-degree relatives with the incidence in the general population and to determine whether panoramic radiographs are a reliable method to screen for early detection of risk of colorectal cancer. A total of 283 patients were interviewed and radiographically examined. Idiopathic osteosclerosis appeared in significantly increased incidence only in patients with colorectal cancer or adenomas and a first-degree relative with CRC (chi 2 = 7.214; p less than 0.01, and chi 2 = 4.1827; p less than 0.05 respectively). The incidence was not increased in first-degree relatives of patients with colorectal cancer or adenoma. Therefore panoramic radiographs cannot be the only screening tool for early detection of patients at high risk of colorectal cancer.

Adenoma↗
PubMed1993

Trichothiodystrophy associated with photosensitivity, gonadal failure, and striking osteosclerosis.

Three new unrelated cases of PIBIDS (Photosensitivity, Ichthyosis, Brittle, sulfur-deficient hair [trichothiodystrophy], Impaired intelligence, Decreased fertility, and Short stature) are reported. Decreased survival of skin fibroblast lines after UVB exposure was found. All three male patients had hypogonadism and primary end-organ gonadal failure. Striking osteosclerosis was present in all three patients. To the best of our knowledge the third patient is the first reported case of a black man with PIBIDS.

Adult↗
PubMed2006

Regression of myelofibrosis and osteosclerosis following hematopoietic cell transplantation assessed by magnetic resonance imaging and histologic grading.

Myelofibrosis is a reactive, often inhomogeneous process in the marrow cavity, and sampling errors on biopsies obtained to diagnose and monitor the course of myelofibrosis have been a constant problem in hematopathology. We investigated the potential utility of magnetic resonance imaging (MRI) of the lumbar spine, pelvis, and femora as a diagnostic and monitoring technique for assessment of myelofibrosis. Findings on serial marrow biopsies were correlated with T1-weighted spin-echo and short inversion time inversion recovery (STIR) images in patients with chronic idiopathic myelofibrosis or myelofibrosis developing from polycythemia vera or essential thrombocythemia who underwent hematopoietic cell transplantation (HCT). Thirty-five patients were studied before HCT; 11 were followed for 3 months and 10 patients for >/=1 year after HCT with sequential marrow biopsies and MRI studies. MRI allowed direct visualization of the biopsy sites and correlation of histologic and MRI findings. MRI also provided assessment of the extent and degree of myelofibrosis in a large volume of the skeletal marrow. There was good correlation between biopsy results and MRI findings at specific biopsy sites and between successful HCT and resolution of fibrosis and osteosclerosis as determined by MRI. We conclude that in patients with myelofibrosis, MRI of the skeleton provides a comprehensive assessment of the pattern and extent of fibrosis and allows for correlation with biopsy findings. In patients undergoing HCT, MRI accurately reflects response or progression of marrow disease.

Adult↗
PubMed1977

Familial osteosclerosis with abnormalities of the nervous system and meninges.

A mother and daughter with osteosclerotic dysplasia are described. The daughter had generalized osteosclerosis, flattening of the angles of the mandibles, high-arched palate, mandibular and facial bone hypoplasia, a large sella turcica, and spacious foramen magnum, platybasia, basilar impression, widened spinal cord with enlarged intervertebral foramina, and scalloping of the posterior surfaces of the vertebral bodies. Radiographic contrast studies and operative intervention revealed multiple thoracic and lumbar meningoceles and an "empty" sella, as well as evidence of maldevelopment of the spinal cord, cerebellum, and cerebral cortex. Many of these skeletal features were noted to a lesser degree in the asymptomatic mother.

Abnormalities, Multiple↗
PubMed1999

Autosomal dominant osteosclerosis: report of a kindred.

Autosomal dominant osteosclerosis (ADO), a rare inherited craniotubular bone disorder, is a generalized hyperostosis that manifests itself as increased cortical thickening of the skull, mandible, metacarpals, metatarsals, long bones, vertebral bodies, ribs, and clavicles. Jaw abnormalities, which clinically resemble the widening and deepening of the mandible seen in cherubism, begin in childhood and have been reported to stabilize after puberty. Teeth and alveolar bone are normal. ADO must be distinguished from Van Buchem's disease, which is characterized by elevated serum alkaline phosphatase, neurologic complications, exopthalmos, periosteal excrescences, and an autosomal recessive pattern of inheritance, as well as from other craniotubular bone disorders such as osteopetrosis. We present clinical and radiographic documentation of members of a kindred representing 4 generations affected with ADO. At initial examination of the proband, a differential diagnosis included cherubism, fibrous dysplasia, osteopetrosis, and Paget's disease. Radiographic examination revealed extensive radiopacity of the inferior border and basal bone of the mandible. The proband's clavicles and humerus were also affected. All family members examined were similarly affected and had mandibular and palatal tori. Authors of a previously published report on the dental and dentoalveolar management of patients with craniotubular bone disorders have recommended prophylactic antibiotics to minimize risk of osteomyelitis in all such cases. The members of our kindred received extensive dental treatment before diagnosis, including extractions of severely carious teeth, preprosthetic dentoalveolar surgery, and endodontic therapy; there was no incidence of osteomyelitis or postsurgical complications. Therefore, the use of prophylactic antibiotics may not be warranted in patients with ADO who have otherwise normal medical histories.

Adult↗