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At least 811 records · Page 45Linked to original sources

Long-term survival and reversal of iron overload after marrow transplantation in dogs with congenital hemolytic anemia.

Severe hemolytic in Basenji dogs secondary to pyruvate kinase deficiency was corrected by marrow transplantation from hematologically normal littermates. These dogs have now been followed for more than 5.5 yr. Essentially normal hematopoiesis has persisted, and the dogs remain in good health without cirrhosis or osteosclerosis. Furthermore, hepatic iron overload present before transplantation has gradually decreased. These results in dogs suggest that marrow transplantation could prevent the morbidity and mortality of severe hemolytic anemia and associated iron overload in man.

Anemia, Hemolytic, Congenital↗

[Morphological data on gerontostomatology].

On the base of the study of serial histological sections, freezed-sections and special bone-praeparates senile alterations of the structure of the palatine mucosa and the mandibular bones are discussed, with special regard on the gerontostomatological aspects. After the loss of the teeth the structure of the connective tissue of the gingiva diappears, in the processus alveolaris develops fibrosis. The glandular tissue of individual extension on the edges shows regression. Thickening of the epithelium in some area can be seen. The lamina propria in the anterior third is very thick in the pharyngeal part becomes thinner. Alveolar processes of the maxilla show signs of involution and atrophy of disuse, with a number of defects and assymetries on the palate. On the macrorelief of the mandibula characteristic changes can be observed: in the region of the corpus eburneationg and osteosclerosis of the spongiosa can be revealed.

Aging↗

[Skin and bone findings in mastocytosis].

The syndrome of mastocytosis extends from cutaneous urticaria pigmentosa through systemic mastocytosis to the rarely occurring mast cell leukaemia. Our investigations with a large patient collective have shown that systemic forms of the disease occur more often than is generally supposed. In particular the incidence of bone marrow manifestations deserve more attention. The inflammatory-granulomatous findings in the bone marrow suggest an immunoactive component in the pathogenesis of this disease. The bone lesions that occur in about 50% of patients are probably the result of the common endosteal site of the mastocytosis granuloma. These lesions can be generalized (osteoporosis-osteosclerosis) or localized (osteolytic-osteosclerotic foci). In clinical practice bone biopsy and skeletal radiology complement one another; in addition to skin biopsy bone biopsy supplies the initial diagnosis of mastocytosis and documents systemic manifestation; the X-ray picture informs the clinician about the type and extent of the bone pathology.

Adolescent↗

beta-Estradiol reduces natural killer cells in mice.

beta-estradiol was administered to mice continuously by diffusion from a silastic tube that was implanted subcutaneously at 4 weeks of age. Four to 6 weeks of estrogen administration caused a substantial reduction in natural killer cell activity in the spleens from mice of either sex. Androgen (5alpha-dihydrotestosterone) did not. Castration of male or female mice did not affect natural killing and did not alter the effect of beta-estradiol. Estradiol did not affect natural killing in vitro and the loss of natural killing was not due to a soluble or a cellular suppressor of natural killing. The effects of estradiol were not dependent on the thymus, since estradiol reduced natural killing in mice that had been neonatally thymectomized. After removal of the estrogen implant, natural killing recovered over a period of 8 weeks. The loss of natural killing may reflect a loss of bone marrow secondary to estrogen-induced osteosclerosis.

Animals↗

[Laboratory diagnosis of idiopathic myelofibrosis. Review of the literature and presentation of 15 cases].

We report on 15 patients affected by myelofibrosis. The main clinical aspects were hepatosplenomegaly, anemia and leukocytosis. Immature cells and anisopoikilocytosis of the erythrocytes were frequently found in the peripheral blood. At bone biopsy, various aspects of panhyperplasia, increase in the reticular fibers, myeloid fibrosis and osteosclerosis were observed. Leukocyte alkaline phosphatase was frequently elevated. The clinical and laboratory course of the disease, causes of death and the possible etiopathogenetic aspects of this disease are considered and discussed.

Adult↗

Chordoma of the cervical spine.

Eight cases of chordoma limited to the cervical spine are presented. The radiological features are analyzed. Although there is no single diagnostic feature, the combination of osteosclerosis and lysis, multiple vertebral involvement, and the presence of a pre- or paracervical mass is strongly suggestive of a chordoma. Although none of our patients can be considered cured, we recommend an anterior cervical approach with radical removal of the tumor and interbody fusion followed by immobilization in a halo vest and postoperative radiation therapy. The biological behavior of the tumor is extremely variable, and multiple operations for symptomatic recurrences may be helpful.

Adult↗

Skeletal fluorosis from eating soil.

A woman with chronic pyelonephritis developed progressive muscular weakness and bone pain. For twenty years she had habitually ingested fluoride-rich soil. Osteosclerosis was found on x-ray examination, and fluorosis was confirmed by bone biopsy. Renal failure augmented skeletal retention of excessive fluoride intake which, in turn, appears to have intensified symptomatic renal osteodystrophy. Skeletal fluorosis from this unexpected source has not been previously described.

Adult↗

Niflumic acid-induced skeletal fluorosis: iatrogenic disease or therapeutic perspective for osteoporosis?

A case of skeletal fluorosis induced by prolonged treatment with niflumic acid, a fast-acting non-steroid antiinflammatory agent, is reported in a 35-year-old woman suffering from rheumatoid arthritis and treated, in addition, with corticosteroids. The case report discussed is, to our knowledge, the third of its kind regarding bone fluorosis resulting from use of this nicotinic derivative. This clinically asymptomatic case of skeletal fluorosis was discovered, as in the 2 previously reported cases, by the examination of bone X-ray (performed as part of the routine work-up for rheumatoid arthritis) which showed evident osteosclerosis. Quantitative histologic study of iliac crest biopsy revealed marked increase in trabecular bone volume and osteoid volume, suggestive of fluorosis. Abnormally high urine and bone fluoride confirmed the diagnosis. After ruling out a hydrotelluric source of fluorine, the patient's fluorosis was linked to chronic use of niflumic acid, following the publication in 1978 of the 2 previously reported cases affected by this drug. The fluorine contained in niflumic acid induced a marked densification of trabecular bone in all 3 cases. Long-term clinical and pharmacokinetic studies will be required to determine whether or not niflumic acid can be employed in the prevention or treatment of both of apparently idiopathic osteoporosis or corticosteroid-induced osteoporosis.

Adult↗

[Roentgenographic features in pigmented villonodular synovitis (concerning a carpal location) (author's transl)].

Pigmented villonodular synovitis is a benign proliferation of the synovial membrane. Its pathogenesis is not well known. It results in an articular swelling without great pain. Its roentgenographic aspect is a combination of an opacity of soft tissue and epiphyseal damages, i.e. cortical erosions and lacunae surrounded by osteosclerosis. Hyperplasty of the synovial membrane is explicited by arthrography and arteriography. The authors report one case of pigmented villonodular synovitis in the carpus: that is an uncommon location of that disease which most often involves the knee.

Adolescent↗

Idiopathic avascular necrosis of the scaphoid. A case report.

Avascular necrosis of the carpal scaphoid is a common disorder after a trauma, i.e. a fracture or mild and repetitive injury. Sometime it can be associated with a systemic disease or chronic steroid intake. Rarely avascular necrosis is found in the absence of a known etiology and so termed "idiopathic". We report a case of idiopathic avascular necrosis of the scaphoid. A painful wrist of six months' duration was observed in a 62-year-old housewife. No history of trauma or steroid administration could be elicited. X-ray showed an osteolytic area with irregular edges surrounded by a ring of osteosclerosis at the proximal pole of the left scaphoid. This datum was confirmed by the magnetic resonance imaging.

Carpal Bones↗

[Albers-Schönberg disease].

The authors while describing their patients suffering from osteopetrosis disease, discuss its morphological aspects and possible patho-mechanism. The disease with osteosclerosis can be inherited recessively or dominantly. The recessively inherited type is less frequent and leads to early death due to secondary developing myelofibrosis. The dominantly inherited form is more benevolent, the patients are free of symptoms in half of cases. The patients described by the authors belong to the dominantly inherited type of the Albers-Schönberg disease. One of their patients suffers from rheumatoid arthritis and myelodisplastic syndrome apart from osteopetrosis. Having considered the publications authors have found data based on which the common source and connection of these three diseases can be rendered possible. Analyzing these data they draw attention to the possible pathogenic role of cytokines, first of all of the macrophag colony stimulating factor, moreover to the rheumatic manifestation of the paraneoplastic syndrome.

Arthritis, Rheumatoid↗

Bone densitometry and histomorphometry in patients with hairy cell leukemia.

Tumor necrosis factor alpha, the cytokine that participates in the autocrine growth control of hairy cell leukemia has strong bone resorptive properties. This prompted us to look for bone involvement in HCL. Bone mineral density (BMD) was not decreased in 14 HCL patients who did not have radiographic evidence of bone destruction. Osteopenia was found in only two HCL patients with skeletal complications of the disease and bone pain preceded diagnosis in both cases. Lymphomatous infiltration of the vertebral bodies T 12/L1 was confirmed histologically in the female patient while bilateral necrosis of the femoral head preceded the diagnosis of HCL in the male patient. Histomorphometry of bone biopsy samples was performed in another 12, previously untreated male HCL patients. Trabecular bone volume was found to be reduced in 11, greatly reduced in 5 of them, while osteosclerosis was found in only one patient. The increase of trabecular bone pattern factor, a new parameter for simple quantification of trabecular interconnection indicated a poorly connected trabecular lattice in eight of these 12 patients.

Adult↗

Radiographic and scintigraphic features of modeling and remodeling in the heterotopic skeleton of patients who have fibrodysplasia ossificans progressiva.

To characterize the radiographic and scintigraphic features of modeling and remodeling in the heterotopic skeleton of patients who have fibrodysplasia ossificans progressiva, radiographs from 47 patients and radionuclide bone scans from 12 of those patients, all of whom had a confirmed diagnosis of the disease, were reviewed. A wide range of normal bone modeling and remodeling features was seen in the heterotopic skeleton of all but the youngest two (age, 1 year) of the 47 patients. Characteristic features of normal bone modeling identified on radiographs of the heterotopic skeleton included: (a) the development of tubular and flat bones with mature cortical and trabecular organization; (b) the presence of well defined cortical-endosteal borders enclosing medullary canals; and (c) the presence of metaphyseal funnelization in isolated ossicles or at sites of synostoses. Characteristic features of normal bone remodeling identified on radiographs of the heterotopic skeleton included: (a) the response of heterotopic bone to weight bearing stress with osteosclerosis of use and osteopenia of disuse, and (b) the resistance of heterotopic bone to fatigue failure with the absence of pathologic fractures and stress fractures. Radionuclide bone scans in 12 patients showed that remodeling of mature heterotopic bone occurred at a rate consistent with that of mature normotopic bone. This study documents the radiographic and scintigraphic features of a heterotopic skeletal system in 47 patients who have fibrodysplasia ossificans progressiva. These data provide additional support for the hypothesis that the genetic defect leading to the formation of a heterotopic skeleton involves normal skeletal morphogenesis at heterotopic sites.

Adolescent↗

Long term results of subtotal parathyroidectomy in patients with end-stage renal disease.

This is a retrospective, clinical study evaluating the long-term outcome of subtotal parathyroidectomy (PTX) in 60 patients with chronic renal failure and severe secondary hyperparathyroidism. Patients were 41 +/- 2 years old (mean +/- SE) at the time of PTX, and followed for 69 +/- 6 months since the procedure. At the time of PTX, three patients had chronic renal failure, 53 had been on chronic hemodialysis, and four had received successful kidney transplants. In more than 80 per cent of patients, symptoms of hyperparathyroidism (bone pain and muscle weakness) resolved within weeks, and biochemical signs (hypercalcemia, and high plasma alkaline phosphatase and parathyroid hormone concentrations) returned to normal ranges within a year. Subperiosteal resorption, bone fractures, and soft tissue calcification frequently improved. Osteosclerosis (rugger-jersey spine), cystic bone changes, osteopenia, and vascular calcifications were, however, often unchanged or progressive. Five patients (8%) who had either persistent or recurrent hyperparathyroidism required additional surgical procedures, and two had subsequent improvement. Twelve patients who had aluminum associated bone disease diagnosed later continued to progress with a high incidence of bone fractures and severe osteopenia. Cystic bone changes, especially of the carpal bones, in association with carpal tunnel syndrome, probably representing amyloid bone disease, also did not respond to PTX. In conclusion, PTX is an effective surgical procedure to reverse complications of hyperparathyroidism in patients with end-stage renal disease, provided that other causes of osteodystrophy, such as aluminum or amyloid-associated bone diseases, are adequately excluded. We feel that subtotal PTX, leaving a small remnant in place, is the procedure of choice.

Adolescent↗

[Association of monomelic melorheostosis and synovial chondromatosis of the knee].

Melorheostosis is a rare affection, of obscure etiopathogenesis, corresponding to osteosclerosis with running hyperostosis. We report the observation of melorheostosis of a lower limb occurring to an 18-year old patient who consulted for tumefaction of the lower limb with an aching and stiffened knee. The radiography allowed the diagnosis by showing an aspect characterized by a running like of a candle accompanied by osteochondromatosis of the knee and the calcification of soft tissues. The synovectomy of the knee brought about indolence and the lesions seem to stabilise after a 3 years follow-up. The synovial osteochondromatosis seems to be an integral part of the disease. The characteristics of melorheostosis are studied according to the data of the literature.

Adolescent↗

Evaluation of spinal bone changes in patients with chronic renal failure by CT and MR imaging with pathologic correlation.

To investigate bone changes in patients with chronic renal failure (CRF), bone mineral density (BMD) and T1 relaxation times were measured with CT and MR imaging and the results were correlated to histology. Excised lumbar vertebrae from 25 autopsy cases of CRF (18 males and 7 females), including 12 cases in which the patients had been receiving hemodialysis were examined. BMD and T1 relaxation time values were associated with specific histologic findings for cellularity, trabeculae, and peritrabecular fibrosis. Three vertebrae with low BMD showed increased hematopoietic marrow content, a finding not observed in primary osteoporosis. The vertebrae with osteosclerosis showed prolonged T1 relaxation time, which was due to increased amount of hematopoietic marrow, and the presence of thickened or many small irregular trabeculae or peritrabecular fibrosis. These findings may be useful in the evaluation of bone changes in patients with CRF.

Adult↗

[The necessity for the combined use of the ultrasonic study of the abdominal cavity organs and of the histomorphometry of the bone marrow in chronic myeloleukemia].

Forty-one patients with chronic myeloid leukemia (CML) were divided into 3 groups: 13 patients in a chronic phase prior to cytostatic treatment group 1; 17 patients in a chronic phase treated with myelosan group 2; 11 patients in a blast transformation phase group 3. All of them underwent abdominal sonography in line with marrow histomorphometry. Ultrasound investigation is thought indispensable in examination of CML patients as it registers negligible enlargement of the liver and spleen and provided their echo structure. It makes possible to trace stages of the tumor process development. CML involves in the pathological process the kidneys as shown by changes in the echo pattern, concrements are often formed in the pelvicalyceal system. Group 1 patients demonstrated reduced area of the bone tissue by morphometrical evaluation of the bone marrow. This means prevalence of osteolysis in CML onset. In group 3 patients this value is higher indicating osteosclerosis predominance. Comparison of the ultrasonic and histomorphometric data suggest that enlargement of the spleen and liver with diffuse vegetations of the connective tissue and their fibrotic lesion of the parenchyma correlated with the severity dissemination of hemopoietic tissue collagen fibrosis. It is evident that CML is characterized by fibrosis involving simultaneously bone marrow, splenic and hepatic parenchyma.

Abdomen↗

[The significance of preoperative chemotherapy for thermopreservation of limbs].

Preoperative chemotherapeutic effects on 42 osteosarcomas treated with CDDP intra-arterial infusion protocol were compared with the effects of treatment of same by local perfusion only, following administration of adriamycin (ADR) or high doses methotrexate and leucovorin (HD-MTX). Thirteen cases were further given local hyperthermic perfusion (LHP) treatment. The preoperative local treatment resulted in a poor response in cases in which the serum ALP failed to fall down the normal range, those with angiographically demonstrated tumor stains remaining, and those with reactive zones remaining as indicated by T2 MR imaging. The ratios of necrotic areas were significantly higher in the LHP group, while those of viable areas were significantly lower in the LHP group compared to other groups. Total tumor cell killing was possible in six of 13 cases in the LHP group. Post-necrotic fibrosis and post-necrotic osteosclerosis were significantly higher in the LPH group. The results suggested that, in selected cases treated with LHP, conservative surgery may well be possible so as to preserve all functional activity.

Adolescent↗