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PubMed1983

[Acute myelo-monocytic leukemia with collagenous medullary fibrosis and osteosclerotic lesions].

A case of acute myelo-monocytic leukemia (M4) in a 75 year old man with multiple osteosclerotic lesions is reported. Bone metastases were clinically and radiologically suspected. A surgical bone marrow biopsy at the iliac crest revealed a massive infiltrate of blasts with medullary fibrosis, large areas of osteosclerosis and foci of necrosis. Osteosclerosis is rarely observed during acute leukemias in adults. Differential diagnosis with acute myelofibrosis is discussed.

Aged↗

PubMed1994

[Histopathological study of the effect of preoperative chemotherapy on osteosarcoma].

A total of 70 patients with conventional osteosarcoma underwent preoperative chemotherapy, and here we report the histopathological effects of the chemotherapy on the tumors. The changes in the tumors since pre-chemotherapy observed at resection were classified as: (1) non-degeneration, (2) degeneration, (3) coagulation necrosis, (4) granulation, (5) fibrosis, or (6) osteosclerosis, It was thought that any coagulation necrosis was due to ischemia, while any degeneration in the tumor tissue was mainly caused by the chemotherapy. Coagulation necrosis was found at the center of the tumor, while degeneration, fibrosis, and osteosclerosis tended to be present mainly at the periphery. Coagulation necrosis was less prominent in these patients with a longer period between preoperative chemotherapy and resection. The above results suggest that coagulation necrosis was not a change caused directly by the chemotherapy, and there is a possibility that the tumor cells in these regions survived until just before resection. Accordingly, when evaluating the effects of preoperative chemotherapy, it may be inappropriate to include any area of coagulation necrosis in the calculation of the necrotic ratio.

Adolescent↗

PubMed1993

Pattern of renal osteodystrophy in haemodialysis patients in Saudi Arabia.

In order to know the pattern of renal osteodystrophy in haemodialysis patients in Saudi Arabia we conducted a multicentre study involving 209 patients. The mean age of the patients was 39.4 +/- 14 (18-70) years, 128 were males and 81 females. All patients were on acetate dialysate and their mean duration on dialysis was 3.5 +/- 1.5 years. The major symptom was bone and joint pain (25.8%). The mean serum calcium was 2.1 +/- 0.26 mmol/l, phosphorus 2.0 +/- 0.36 mmol/l, alkaline phosphatase 19.7 +/- 14.6 u/l and parathyroid hormone level was 8.9 +/- 3.9 mg/ml. The mean serum aluminium (AL) level was 25.4 +/- 17.7 micrograms/l, while that of 1,25 vitamin D3 was 8.1 +/- 4.2 ng/l and of fluoride was 92.2 +/- 31.4 micrograms/l. The major radiological finding was osteosclerosis (70%). Dual-photon absorptiometry (DPA) showed low bone mineral density (LBM) in 65% of the patients. Forty-one patients had bone biopsies with AL staining of the biopsies. Of this group, 92% had changes of hyperparathyroidism and 66% of them were pure hyperparathyroidism. Sixty percent of them had variable degrees of AL intoxication. The radiological skeletal survey of those patients could detect abnormalities in only 46% while 70% of them had abnormal bone mineral density (BMD). In conclusion, osteosclerosis is the commonest radiological finding in our dialysis patients while secondary hyperparathyroidism is the main histopathological diagnosis in bone biopsy, even in patients with normal skeletal survey. AL intoxication is a significant problem in our population. DPA is more sensitive in detecting bone abnormalities than X-radiography.

Adolescent↗

PubMed1977

The ultrastructural effect of estrogens on bone cells in thyroparathyroidectomized rats.

To determine the direct effect of estrogen on bone cells, female rats were thyroparathyroidectomized and given either 200 microng of estradiol cypionate or placebo treatment for 4 to 30 days. After 8 days, an osteosclerosis of the tibial metaphysis developed in rats treated with estrogens. Osteoblasts in estrogen-treated rats were interpreted ultrastructurally to be less active in bone formation than controls. Osteocytes in estrogen-treated rats were inactive compared to osteocytes in control rats which were continuing the process of bone formation. Osteoclasts were decreased in numbers in rats treated with estrogen; however, the electron microscopic characteristics of osteoclasts did not differ from controls. These cellular changes were associated with a severe hypocalcemia in both estrogen-treated and control rats. Serum phosphorus and urinary hydroxyproline were lowered significantly by the administration of estrogen. It is concluded that metaphyseal osteosclerosis develops in estrogen-treated rats independent of parathyroid hormone and calcitonin. The osteoclerosis is due to an effect of estrogens to reduce osteoclast-numbers and a lesser inhibitory effect on the bone-forming activity of osteoblasts and osteocytes. (Am J Pathol 87:311-322, 1977).

Animals↗

PubMed1997

[Morphogenesis of occupational fluoride osteopathy].

Fluor osteopathy, as the authors suppose, is a morphologic repetition of phylogenesis early stages in osteogenesis. Thus, osteosclerosis and osteoporosis demonstrated by X-ray should be considered as manifestation of bone fluorosis. Fluor-induced changes of bone tissue could not be adequately termed as "osteoporosis" and "osteosclerosis", so is defined as "fluor osteopathy".

Adult↗

PubMed1997

[SAPHO syndrome and aseptic osteitis].

Palmoplantar pustulosis and severe acne are sometimes associated with aseptic skeletal lesions, but such skeletal conditions can be observed without skin lesions. The acronym SAPHO (Synovitis, Acne, Palmoplantar pustulosis, Hyperostosis, Osteitis) has been suggested for this cluster of manifestations. The most frequent site of the disease is the upper anterior chest wall, characterized by predominantly osteosclerotic lesions, hyperostosis, and arthritis of the adjacent joints. Osteosclerosis of the vertebral bodies, hyperostosis, and erosions of the vertebral plates can be encountered. Unilateral sacroiliitis of frequently observed. Long bone involvement consists of osteosclerosis or osteolysis with periosteal new bone formation. Peripheral arthritis is rarely associated with joint destruction. The pathogenesis of this syndrome remains unknown, but a link with seronegative spondylarthropathies is probable. Awareness of the SAPHO syndrome should facilitate proper diagnosis and treatment.

Acquired Hyperostosis Syndrome↗

PubMed1996

Aluminum potentiates the effect of fluoride on tyrosine phosphorylation and osteoblast replication in vitro and bone mass in vivo.

Osteosclerosis in workers exposed to fluoride (F) and aluminum (Al) (industrial fluorosis) led to the use of F as a treatment to increase bone mass in osteoporosis patients. Because the influence of traces of Al on the effects of F on bone formation is heretofore unknown, we have investigated this issue both in vitro and in vivo. We have found that minute amounts of Al (< or = 10(-5) M) potentiate the effects of F in vitro such that osteoblast proliferation increased by 15 +/- 2.7% at 50 microM (p < 0.001) and by 117.6 +/- 5.1% at 750 microM (p < 0.001), concentrations of F with no mitogenic effect alone. F + Al time-dependently modulated a growth factor signaling pathway(s) associated with enhanced tyrosine phosphorylation (TyrP) of several proteins (p90 [2.9x], p77 [4.9x], p68 [9.6x], and mitogen activated protein kinases [3x]). TyrP was only slightly or not at all changed by F and Al alone, respectively. The effects of F + Al on TyrP and cell proliferation were markedly reduced by 100 microM tyrphostin-51, a tyrosine kinase inhibitor. Protein kinase A (PKA) and protein kinase C (PKC) pathways were not involved in this response. In vivo, F + Al administered for 8 months, at doses that had no effect when the minerals were administered individually, significantly enhanced proximal tibia bone mineral density (BMD) by 6.3 +/- 1% compared with initial values and by 2-fold compared with control ovariectomized rats (p < 0.0001). These effects are consistent with a crucial role of Al in osteosclerosis observed in industrial fluorosis. The results suggest that the combination of F + Al modulates a growth factor-dependent TyrP pathway enhancing mitogen-activated protein kinase and osteoblastic proliferation and bone mass.

3T3 Cells↗

PubMed1989

Metastases to bone in medulloblastoma. A report of five cases.

Medulloblastoma is a relatively common intracranial neoplasm in childhood. Its extraneural spread was, until recently, thought to be a rare occurrence. Metastases are most commonly to bone. Five patients with medulloblastoma metastatic to bone are presented, and findings are compared to those of previous reports. Two of the five cases showed patchy extensive osteosclerosis of the pelvis and/or proximal femora. One case had concurrent lymph node involvement. In patients with past or present medulloblastoma and bone pain, metastases to bone should be excluded. Medulloblastoma metastatic to bone is a rare cause of extensive osteosclerosis.

Adolescent↗

PubMed2002

Type II autosomal dominant osteopetrosis.

Two principal types of osteopetrosis have been distinguished. One is the dominantly inherited, relatively benign condition which is often detected radiologically in asymptomatic adults. A second type is the recessive, lethal, malignant form. Autosomal dominant osteopetrosis (ADO) has two distinct radiological subtypes known as types I and II. We report here a 23-year-old patient with ADO type II. Radiographic investigations of a skeletal survey showed generalised osteosclerosis with thickened cortex. Magnetic resonance imaging (MRI) scan disclosed osteosclerosis in superior and inferior portions of the vertebral bodies which produced a 'sandwich' appearance. The 'bone-within-bone' appearance was seen in the ileum of the patient. The vertebral bone density was found markedly elevated. The carbonic anhydrase II level was found to be normal. We discuss here the genetic etiology of this disorder.

Adult↗

PubMed1987

Primary bone oxalosis: the roles of oxalate deposits and renal osteodystrophy.

Primary oxalosis is a rare congenital disorder. The excessive oxalate biosynthesis induces deposits in many organs, particularly in kidney and bone. The late onset of primary oxalosis is reported in a 50-year-old man. His chronic renal failure was treated by maintenance hemodialysis for 3 years. He then developed a diffuse bone disease with osteosclerosis and roentgenographic features of hyperparathyroidism. A parathyroidectomy was performed, with debatable improvement of bone lesions. Laboratory results and histologic and histomorphometric studies before and after parathyroidectomy suggest a double histopathogenetic mechanism for this bone disease: renal osteodystrophy and massive bone oxalate deposits. Such deposits may induce both a heterogeneous osteosclerosis with dense metaphyseal bands and histologic bone lesions similar to those of hyperparathyroidism. The crystalline deposits induce in the bone tissue a granulomatous macrophagic reaction. These macrophages are unable to phagocytize the crystals and may be involved in active bone resorption. Bone lesions of oxalosis occur in patients with chronic renal failure, and hyperparathyroidism has a worsening role.

Bone and Bones↗

PubMedDate not supplied

Histomorphometric evidence for osteoclast-mediated bone resorption in metastatic breast cancer.

We studied bone biopsies from 65 normocalcaemic women with breast cancer and predominantly osteolytic bone metastases in order to examine the pathophysiology of bone destruction in metastatic bone disease. Quantitative histomorphometric measurements were made at sites of tumour involvement, at sites adjacent to tumour tissue and at sites distant from tumour tissue. There were no significant differences in bone volume or in indices of bone resorption or formation between biopsies taken from sites distant from tumour and the controls. Bone resorption, as judged by eroded surface, increased progressively from bone distant from tumour to tumour-laden bone. The number of osteoclasts was significantly increased in bone immediately adjacent to tumour and within metastases. There was no decrease in the ratio of osteoclast to eroded surface in breast cancer compared to controls suggesting that increased resorption in breast cancer was mainly osteoclast mediated and locally activated by the tumour. Two thirds of the biopsies taken from tumour involved regions showed osteosclerosis with woven bone formation. The volume of the pre-existing lamellar trabecular bone was lower than normal in 75% of these biopsies, suggesting that bone resorption must have been increased before the onset of woven bone formation. Since all patients were receiving hormonal treatment or chemotherapy, it is likely that osteosclerosis at sites of previous resorption mainly resulted from the basic cancer treatment as a sign of response to treatment. Osteoclastic bone resorption was, however, not completely inhibited by the active cancer treatment.

Adult↗

PubMed2000

Complete remission of idiopathic myelofibrosis following donor lymphocyte infusion after failure of allogeneic transplantation: demonstration of a graft-versus-myelofibrosis effect.

A patient with idiopathic myelofibrosis (IM) in the osteosclerotic phase received an allogeneic stem cell transplant. Hemopoietic engraftment was rapid, and full donor chimerism was observed on day +70. However, a few months later, replacement of donor hemopoiesis by the patient's 20q- cell clone was observed, followed by reappearance of the blood IM features, marrow fibrosis and osteosclerosis. At 8 months from transplant donor lymphocytes were infused, which induced chronic GVHD. This resulted in normalization of the blood, with disappearance of the fibrosis and osteosclerosis, effects which persisted 20 months later. This case provides evidence for a graft-versus-disease effect in IM.

Female↗

PubMed1976

[Sarcoidosis of the skeleton. Review of the literature and case report. (author's transl)].

The frequency of sarcoidosis in the skeleton varies between 3 and 36%. Skeletal sarcoidosis is rare in early stages (Löfgren-syndrom), relatively frequent in late stages. The initial phase is characterized by the formation of miliary non-caseating epitheloid-cell granulomas in the bone marrow. The invasion of the bone marrow may either be tolerated by the bone tissue or it initiates a perifocal osteosclerosis or a osteolysis. Correspondingly the X-ray of the skeleton shows normal structure or focal osteosclerosis or osteolysis. Therefore in the first case the sarcoidosis cannot be identified by X-ray. Most frequent locations are the phalanges of the fingers and toes, less common the stem skelton (skull, vertebrae, pelvis) and very rare the long tubular bones. In most cases the skeletal sarcoidosis is well tolerated. Report of a case of osteosclerotic sarcoidosis of the pelvis of a 39-years old woman with generalized sarcoidosis which was diagnozed four years earlier. The X-rays of the phalanges were normal. The biopsy of the iliac crest shows miliary sarcoid granuloma of the bone marrow and accretion of lamellar bone on the surface of the bone trabeculi with a distinct mosaic pattern. Treatment with steroids during the following five years was ineffective.

Adolescent↗

PubMed1996

Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases.

We made a retrospective evaluation of clinical and radiologic features, treatment, and outcome of Erdheim-Chester disease, a rare non-Langerhans cell histiocytosis. We had 7 patients coming from 3 French teaching hospitals and reviewed 52 cases from the literature. These cases were considered to have Erdheim-Chester disease when they had either typical bone radiographs (symmetrical long bones osteosclerosis) and/or histologic criteria disclosing histiocytic infiltration without features for Langerhans cell histiocytosis (no S-100 protein, no intracytoplasmic Birbeck granules). Ages at diagnosis ranged from 7 to 84 years (mean +/- SD = 53 +/- 14 yr) with a male/female ratio of 33/26. Bone pain was the most frequent clinical sign (28/59), mostly located in the lower limbs. Exophthalmos and diabetes insipidus were found in respectively 16/59 and 17/59 patients. General symptoms (fever, weight loss) and "xanthomas" (mainly located on the eyelids) were present in 11/59 patients. Retroperitoneal involvement was found in 17/59 patients. Skeletal X-ray showed typical osteosclerosis of the diaphysis of the long bones in 45/59 patients. Bone radiographs showed osteolytic lesions of the flat bones (skull, ribs) in 8 patients. Histologic diagnosis was performed after a bone biopsy (28 patients), a retroorbital biopsy (9 patients), and/or a biopsy of the retroperitoneal infiltration or the kidney (11 patients). Six of our 7 patients but only 5 of 52 patients from the literature had the complete histologic criteria, disclosing no Birbeck granules or S-100 immunostaining. In other cases, histologic results usually described a xanthogranulomatous infiltration by foamy histiocytes nested in fibrosis. Treatment was corticotherapy (20/59), chemotherapy (8/59), radiotherapy (6/59), surgery (3/59) and immunotherapy (1 patient). Twenty-two patients died after a mean follow-up of 32 +/- 30 mo (range, 3-120 mo). In conclusion, Erdheim-Chester disease may be confused with Langerhans cell histiocytosis as it sometimes shares the same clinical (exophthalmos, diabetes insipidus) or radiologic (osteolytic lesions) findings. However, it also appears to have distinctive features. Patients are older and have a worse prognosis than those with Langerhans cell histiocytosis, and the diagnosis relies on the association of specific radiologic and histologic findings.

Adolescent↗

PubMed1988

Biochemical evidence of disturbed bone metabolism and calcium homeostasis in two types of autosomal dominant osteopetrosis.

Biochemical markers of bone resorption and bone formation were measured in 14 patients with autosomal dominant osteopetrosis, and compared with age- and sex-matched controls. There were eight patients with the radiological type I characterized by diffuse, symmetrical osteosclerosis with pronounced sclerosis of the skull and enlarged thickness of the cranial vault, and six patients with type II characterized by diffuse, symmetrical osteosclerosis, "Rugger-Jersey spine" and "endobones" (bone within a bone) in the pelvis. Serum levels of alkaline phosphatase and osteocalcin in types I and II did not differ from controls indicating normal bone formation. However, a significantly decreased fasting renal excretion of phosphate and hydroxyproline in both types compared with normal controls, suggests a reduced bone resorption. Serum levels of parathyroid hormone (PTH), albumin-corrected calcium, phosphate, and acid phosphatase were normal in type I. In type II serum levels of albumin-corrected calcium and PTH were significantly increased (p less than 0.05 and p less than 0.01). The level of acid phosphatase was markedly increased in this type (p less than 0.01). These findings suggest differences between the two types in calcium homeostasis and bone metabolism, and thus corroborate the evidence that the two radiological types reflect two different disorders of bone resorption.

Adult↗

PubMed1998

Microfocal radiography in the diagnosis of childhood renal osteodystrophy.

BACKGROUND: Renal osteodystrophy is common in children with chronic renal failure (CRF) and X-ray is an intensive method in the diagnosis of the disease. In this study we compared microfocal radiography with conventional method for the diagnosis of renal osteodystrophy. METHODS: The X-rays of left wrists of 21 children with CRF and chronic renal insufficiency were taken by conventional and microfocal methods. RESULTS: Both methods revealed osteopenia in all patients (100%), widening, fraying and cupping of ulnar and radial metaphysis in 10 (47.6%), osteosclerosis in three (14.2%) and pseudofracture in one (4.7%) patient. Microfocal radiography demonstrated osteosclerosis in one patient, pseudofracture in four and subperiosteal resorption in five patients that were not detected by conventional method. CONCLUSION: Two methods were found to be significantly different in demonstrating the changes due to rickets and hyperparathyroidism and it is concluded that microfocal radiography may be preferred in the diagnosis of childhood renal osteodystrophy.

Adolescent↗

PubMed1993

Musculoskeletal manifestations of chronic renal insufficiency.

Abnormalities involving the musculoskeletal system are numerous and frequent in patients with chronic renal insufficiency (CRI). The most common radiologic manifestations of CRI are those referred to collectively as renal osteodystrophy: the findings seen in secondary hyperparathyroidism (bone resorption, periosteal reaction, and brown tumors), osteoporosis, osteosclerosis, osteomalacia, and soft-tissue and vascular calcification. Bone resorption, the most frequent alteration of CRI, occurs in several locations (subperiosteal, subchondral, trabecular, endosteal, and subligamentous), whereas brown tumors and periosteal reaction are much less common. Osteosclerosis primarily affects the axial skeleton and may be the only sign of CRI. Osteoporosis and osteomalacia cause osteopenia and are not infrequent changes of CRI. The prevalence of calcifications increases with the duration of hemodialysis. The other major group of musculoskeletal abnormalities attributable to CRI includes aluminum deposition, amyloid deposition and destructive spondyloarthropathy, tendon rupture, crystal deposition, infection, and avascular necrosis. These changes are less common than those of renal osteodystrophy and are more frequently seen in patients who have undergone long-term hemodialysis or renal transplantation. Recognition of musculoskeletal manifestations is important in the clinical management of patients with CRI.

Bone Diseases↗

PubMed1995

SAPHO syndrome.

Palmoplantar pustulosis and severe acne are sometimes associated with peculiar aseptic skeletal conditions, but such skeletal lesions can be found without skin lesions. The term SAPHO syndrome has been coined for this cluster of manifestations. (The acronym SAPHO refers to synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis.) The most common site of the disease is the upper anterior chest wall, characterized by predominantly osteosclerotic lesions, hyperostosis, and arthritis of the adjacent joints. Osteosclerosis of the vertebral bodies, hyperostosis, and erosions of the vertebral plates can be encountered. Unilateral sacroiliitis is frequently observed. Long bone involvement consists of osteosclerosis or osteolysis with periosteal new bone formation. Peripheral arthritis can be present but is rarely associated with joint destruction. The pathogenesis of this syndrome remains unknown, but a link with seronegative spondyloarthropathies is probable. Radiologists should be aware of this unusual syndrome to avoid misdiagnosis (eg, tumor, infection), unnecessary surgery, and antibiotic therapy.

Acne Vulgaris↗