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PubMed1976

Increased bone mineral content in young adults with familial hypophosphatemic vitamin D refractory rickets.

Seven adults with familial hypophosphatemia have been investigated by histologic and radiographic examination of bone, and estimates of bone mineral status by in vivo neutron activation analysis (IVNAA). Histological examination showed severe osteomalacia and osteosclerosis in all cases. Radiography showed skeletal deformities and other sequelae of severe rickets of childhood in five of the seven cases, with, in addition, thickened well-mineralized bones; the other two showed normal radiographs. IVNAA measurements showed that the first five had greater than normal bone calcium and that the other two had normal values. Thus, in all cases there is a greater than normal total bone tissue (osteoid and mineralized bone together). The quantitative body calcium measurements show clearly that osteosclerosis occurs in familial hypophosphatemia, confirming the opinions based on histological and radiological data. Although there has been occasional reference to this sclerosis in the literature, up to the present it has received little attention.

Adolescent↗
PubMed1994

[Multiple complications of monoclonal IgM].

A 64 year old woman with monoclonal IgM Kappa cryoglobulinemia had developed neurologic, vascular and bone involvements. If each features were previously reported, their association appears to be rare. The polyneuropathy was consistent with an IgM anti MAG neuropathy rather than a cryoglobulinemic neuropathy. The vascular occlusion is well known in dysglobulinemia. The diffuse osteosclerosis without medullary fibrosis or myeloproliferative disorder was as like as a Schnitzler's syndrome (chronic urticaria, osteosclerosis, monoclonal IgM Kappa gammopathy.

Cryoglobulinemia↗
PubMed2002

Regulating DeltaFosB expression in adult Tet-Off-DeltaFosB transgenic mice alters bone formation and bone mass.

The DeltaFosB isoforms are naturally occurring AP-1 family members that increase bone volume via a cell-autonomous effect on osteoblastic bone formation. Mice overexpressing DeltaFosB demonstrate a very high level of bone formation, resulting in a progressive osteosclerosis. Despite the linkage of bone formation and resorption in physiological systems, no alteration in bone resorption was detected in mice overexpressing DeltaFosB. To determine whether altering DeltaFosB expression can regulate bone formation independently of bone resorption in adult mice, we used the Tet-Off-inducible transgene system to induce or block transgenic DeltaFosB overexpression and thereby regulate bone formation in vivo. Overexpression of DeltaFosB after skeletal maturity increased trabecular bone volume by increasing bone formation, again without altering bone resorption, indicating that developmental DeltaFosB overexpression is not required for the osteosclerotic phenotype. Similarly, switching off DeltaFosB overexpression after osteosclerosis had developed led to a marked decrease in bone formation and loss of bone mass such that trabecular bone volume approached normal levels. Despite this dramatic reduction, no alteration in bone resorption was detected. These results clearly demonstrate that DeltaFosB regulates bone formation and bone mass in adult mice with no effect on bone resorption.

Animals↗
PubMed2003

FLAG chemotherapy followed by allogeneic stem cell transplant using nonmyeloablative conditioning induces regression of myelofibrosis with myeloid metaplasia.

A 38-year-old woman with agnogenic myeloid metaplasia complicated by the poor prognostic factors of severe osteosclerosis, prominent hepatosplenomegaly, and profound anemia was treated with FLAG chemotherapy to decrease her organomegaly before undergoing a nonmyeloablative allogeneic stem cell transplant from a matched-sibling donor. The patient's pre- and post transplant course were complicated by an autoimmune disorder and her post transplant course was complicated by severe hepatic and gastrointestinal GVHD. A technetium-99m sulfur colloid scan 4 months post transplant and bone marrow studies 8 months post transplant demonstrated intramedullary hematopoiesis, complete resolution of marrow fibrosis, and partial resolution of osteosclerosis.

Adult↗
PubMedDate not supplied

[Radiological and histomorphological correlation of bone tumors and tumor-like diseases (author's transl)].

In the diagnosis of bone tumors and tumor-like lesions the radiographic examination plays a dominant role in the phase before performing a biopsy, with the support of this examination important facts are available for the further diagnostic procedure, for planning the biopsy and also for getting the preliminary guiding principles how the therapy has to be performed. In order to interpret a radiograph of a diseased bone optimally it is necessary to know the histomorphological background of an X-ray feature. This more particularly as the reaction of bone to a pathological process is limited to only 2 radiographic images: osteolysis or/and osteosclerosis. On the basis of the histological pattern and the dynamic micromorphological process the picture of osteolysis and osteosclerosis is analysed. The periosteal reaction is also considered in this manner. After the representation of these general principles a detailed tabulated synopsis is given about the correlation between the histological and roentgenological findings of all benign and malignant bone tumors and tumor-like conditions.

Biopsy↗
PubMed2005

Reactive sclerosis: hyperintense appearance on T2-weighted magnetic resonance imaging.

Osteosclerosis is defined as increased density of bone on X-ray imaging studies. It is known that osteosclerosis appears hypointense on both T1- and T2-weighted magnetic resonance imaging sequences. In this review, we present our experience in various sclerotic skeletal pathologies that appear hyperintense on T2-weighted magnetic resonance imaging. We emphasize the possible pathophysiological mechanisms that may cause this appearance, such as bone marrow edema and/or composition of newly formed bone.

Adolescent↗
PubMed1997

A case of hyperoxaluria. Radiological aspects.

PURPOSE: Oxalosis is an unusual pathological condition with calcium oxalate deposits in soft tissue and bone, recognized as osteosclerosis on radiography. Osteosclerotic bone changes in patients treated with hemodialysis are in most cases due to secondary hyperparathyroidism, but several other diagnoses have to be considered. MATERIAL, METHODS AND RESULTS: We describe the case of a young woman with advanced renal failure treated with hemodialysis since her youth. She had skeletal pain and radiological examination showed: osteosclerosis with sclerotic vertebral bodies; irregular sclerosis and unsharp periostal outline in the tubular bones of the extremities; and acrolysis and calcifications of vascular and soft tissue in the hands. Histological examination showed changes typical of oxalosis. A liver biopsy excluded primary oxalosis type I, and she probably had a secondary oxalosis due to renal failure. This condition (as opposed to primary oxalosis) can be treated with renal transplantation. CONCLUSION: Oxalosis is a rare condition but it should be considered in patients with radiological skeletal changes and chronic renal failure and should not be misinterpreted as renal osteodystrophy. The classification of oxalosis as primary or secondary is important for further treatment.

Adult↗
PubMed1990

Morphometric analysis of osteosclerotic bone resulting from hexachlorobenzene exposure.

Hexachlorobenzene (HCB) exposure has been shown to induce hyperparathyroidism and osteosclerosis in rats. Experiments were undertaken to investigate the effects of HCB-induced hyperparathyroidism and osteosclerosis on femur morphometry as well as femur breaking strength. Fischer 344 rats were dosed 5 d/wk for 15 wk with 0, 0.1, 1, 10, or 25 mg HCB/kg body weight. Hyperparathyroidism was produced in the two higher dose groups as reported previously (Andrews et al., 1989). Femur weight was significantly increased in the rats receiving 0.1, 1, and 25 mg HCB/kg body weight, whereas density was increased significantly at 1, 10, and 25 mg HCB/kg dose levels. Bone strength was also significantly increased at the three higher dose levels. Cross-sectional area of the midpoint of the femur was significantly increased at the 1 mg/kg HCB dose level. Cortical area and the proportion of the total area of the bone that the cortex occupied were significantly increased at the three higher dose levels. Medullary cavity area was significantly increased at the 0.1 mg/kg dose level but significantly decreased at the 2 higher dose levels of HCB. The right femur was significantly predominant to the left femur in weight, volume, and density through all dosing regimens. HCB exposure significantly altered bone morphometry and strength characteristics in the Fischer 344 rat.

Alkaline Phosphatase↗
PubMedDate not supplied

Solitary hot spots in the ribs on bone scan: value of thin-section reformatted computed tomography to exclude radiography-negative fractures.

PURPOSE: The purpose of this study was to classify solitary, scintigraphy-positive and radiography-negative rib lesions and to clarify the features of rib fractures by using thin-section reformatted helical computed tomography (CT). METHODS: Forty-seven patients whose whole-body bone scans showed a solitary hot spot in a rib as their first abnormal scintigraphic finding and whose plain radiograph showed negative or indeterminate results were examined by thin-section reformatted CT. Final diagnosis was based on follow-up over a 20-month period. RESULTS: The final diagnosis included 17 cases of fractures where CT findings were fracture line, focal sclerosis, and callus formation. Fourteen ribs demonstrated intramedullary, focal osteosclerosis, and 8 ribs did not demonstrate any abnormalities. Four metastatic lesions appeared as intramedullary mixture of osteolysis and osteosclerosis, or bone destruction. Four intramedullary lesions with cystic appearance remained unchanged. CONCLUSIONS: Thin-section reformatted helical CT delineated minute, radiographically occult fractures of the rib. Excluding fractures, helical CT can avoid further unnecessary examinations.

Adult↗
PubMed1993

Trichothiodystrophy and associated anomalies: a variant of SIBIDS or new symptom complex?

Trichothiodystrophy is characterized by sparse, short, sulfur-deficient hair. Numerous symptom complexes have been described in which the hair abnormality represents a constant feature. We report a boy with trichothiodystrophy, ichthyotic skin changes, onychodystrophy, chronic neutropenia, osteosclerosis, hypothyroidism, nystagmus, growth and mental retardation, and microcephaly, who developed a progressive encephalopathy with ataxia and optic atrophy at 2.5 years of age. In addition to a deficient cystine level identified on a hair sample, a disturbance in the composition of other amino acids was present. Although features were reminiscent of osteosclerosis, ichthyosis, brittle hair due to trichothiodystrophy, impaired intelligence, decreased fertility, and short stature (SIBIDS) and could represent a variant of this disorder, findings in our patient may reflect a new trichothiodystrophy symptom complex that carries a poor prognosis for survival beyond childhood.

Growth Disorders↗
PubMed1977

Renal secondary hyperparathyroidism in aged Sprague-Dawley rats.

Fourteen aged Sprague-Dawley rats had typical renal secondary hyperparathyroidism. The renal disease was severe glomerulosclerosis with tubular dilation. The parathyroid glands were enlarged and had hyperplasia of chief cells. Bone lesions were generalized osteodystrophia fibrosa and osteosclerosis. All rats had osteodystrophia fibrosa and it was severe in 10 rats. Only four rats had osteosclerosis and it was moderate. Metastatic calcification was seen in various soft tissues in 13 rats.

Age Factors↗
PubMed2005

Variegation of the phenotype induced by the Gata1low mutation in mice of different genetic backgrounds.

All mice harboring the X-linked Gata1low mutation in a predominantly CD1 background are born anemic and thrombocytopenic. They recover from anemia at 1 month of age but remain thrombocytopenic all their life and develop myelofibrosis, a syndrome similar to human idiopathic myelofibrosis, at 12 months. The effects of the genetic background on the myelofibrosis developed by Gata1low mice was assessed by introducing the mutation, by standard genetic approaches, in the C57BL/6 and DBA/2 backgrounds and by analyzing the phenotype of the different mutants at 12 to 13 (by histology) and 16 to 20 (by cytofluorimetry) months of age. Although all the Gata1low mice developed fibrosis at 12 to 13 months, variegations were observed in the severity of the phenotype expressed by mutants of different backgrounds. In C57BL/6 mice, the mutation was no longer inherited in a Mendelian fashion, and fibrosis was associated with massive osteosclerosis. Instead, DBA/2 mutants, although severely anemic, expressed limited fibrosis and osteosclerosis and did not present tear-drop poikilocytes in blood or extramedullary hemopoiesis in liver up to 20 months of age. We propose that the variegation in myelofibrosis expressed by Gata1low mutants of different strains might represent a model to study the variability of the clinical picture of the human disease.

Aging↗
PubMed2004

Decreased bone turnover and deterioration of bone structure in two cases of pycnodysostosis.

Pycnodysostosis is an uncommon human genetic disorder characterized by osteosclerosis of the skeleton, short stature, and bone fragility. The disease results from mutations in the cathepsin K gene, a lysosomal cysteine protease highly expressed in osteoclasts and crucial for the degradation of organic matrix from mineralized bone. Recently, interest has focused on a pharmaceutical inhibition of cathepsin K to prevent bone loss. However, little is known about the cellular activity or material quality of bone in pycnodysostosis. In the present study, transiliac bone biopsies from two affected individuals, aged 5 and 21 yr, were investigated using light microscopy, quantitative backscattered electron imaging, and small angle x-ray scattering. Results were compared with published age-matched reference data. The mutations in the cathepsin K gene of both patients were identified, including one novel defect. Both individuals had severe osteosclerosis, and their biopsies displayed multinucleated osteoclasts apposed to areas of demineralized matrix as well as bone-lining cells adjacent to this undigested collagen left over by osteoclasts. The homogeneity of the mineralized matrix was markedly disturbed due to large inclusions of mineralized cartilage residues. Histomorphometric evaluation showed a quantitative decrease in static parameters of bone formation. In contrast and despite deficient cathepsin K activity, osteoclastic parameters were close to normal range. At the nanostructural level, there was a marked increase in the mean thickness of the mineral particles, reflecting decreased bone remodeling. Examination of the trabecular structure revealed that the lamellae were highly disordered, which was also apparent from a poor alignment of mineral crystals oriented along the longitudinal axis of collagen fibrils. Taken together, these results strongly suggest that functional cathepsin K is important for balanced bone turnover, and enzyme deficiency results in a profound deterioration of bone quality with respect to trabecular architecture and lamellar arrangement, which is presumably the reason for bone fragility in pycnodysostosis.

Adult↗
PubMed2002

Skeletal aspects of Gaucher disease: a review.

In Gaucher disease, a genetic deficiency in the activity of the lysosomal enzyme beta-glucocerebrosidase (acid beta-glucosidase) causes monocytes and macrophages to store excessive amounts of glucocerebroside in lysosomes. The resulting distended cells are called Gaucher cells, and the pathology associated with this condition stems from the accumulation of Gaucher cells in organ systems. The skeletal manifestations are probably the most disabling aspect of the disease. Patients commonly experience bone pain, some suffer bone crises, and up to 20% have impaired mobility. Radiological findings include Erlenmeyer flask deformity, osteopenia, osteosclerosis, osteonecrosis, fractures and bone marrow infiltration. Findings from the Gaucher Registry show that nearly all patients with Gaucher disease have radiological evidence of skeletal involvement, and the majority have a history of serious skeletal complications. Skeletal involvement follows three basic processes: focal disease (irreversible lesions such as osteonecrosis and osteosclerosis), local disease (reversible abnormalities adjacent to heavily involved marrow such as cortical thinning and long bone deformity) and generalized osteopenia. Infarctions are involved in some of the skeletal manifestations, but the mechanisms causing high rates of bone turnover and failure of remodelling are not known. The availability of a beta-glucocerebrosidase-deficient mouse model of Gaucher disease with long-term survival should help elucidate the skeletal pathology in Gaucher disease and may ultimately lead to improved management of skeletal complications.

Animals↗
PubMedDate not supplied

A report of fluorosis in the United States secondary to drinking well water.

A 54-year-old female resident of Wellston, Okla, was found to have osteosclerosis on a routine chest roentgenogram. Subsequent investigation disclosed the cause of her osteosclerosis to be fluorosis secondary to the ingestion of well water containing 429 mumol/L of fluoride (recommended levels, 11 to 58 mumol/L). Water samples were also obtained from the 12 wells on properties adjacent to the index case. In three other wells, all at similar depths as the well of the index case, the fluoride concentration of the water was greater than 212 mumol/L. Urine samples from members of the four households who obtain their drinking water from these wells contained elevated urinary fluoride levels. Thus, fluorosis may develop in certain areas of the United States as a result of the natural occurrence of fluoride in the groundwater. Consequently, in known endemic areas, it would appear reasonable to measure the fluoride concentration of the well water at the time of drilling.

Female↗
PubMed1991

Determination of erythrocyte pyruvate kinase deficiency in Basenjis with chronic hemolytic anemia.

Erythrocyte pyruvate kinase (PK) deficiency was first described in Basenjis 20 years ago. Although the approach to diagnosis had not been well established, a screening program to detect heterozygotes was thought to have eliminated PK deficiency from the Basenjis of the United States. Four not closely related Basenjis with severe chronic hemolytic anemia, from various parts of the United States, were studied. Their PCV ranged from 16 to 25% and their reticulocyte count was always above 15%. A progressive osteosclerosis was seen in all of the Basenjis and hepatic failure developed in 2 of them. The erythrocyte intermediary metabolite patterns indicated a glycolytic defect at the PK step. Erythrocyte PK activities were markedly increased in the anemic Basenjis, compared with those of a control group, but the enzyme in these Basenjis had abnormal kinetic properties and was thermolabile. An antibody against R-type PK, the regular erythrocyte PK form, did not neutralize the PK activity of affected Basenjis, and results of electrophoretic studies suggested the expression of M2-type PK, a leukocyte and fetal erythroid PK-type. Clinically healthy heterozygous Basenjis had half-normal R-type PK activity and did not express the M2-type in their erythrocytes. We conclude that severe chronic hemolytic anemia, caused by erythrocyte PK deficiency, and associated osteosclerosis still develop in Basenjis. A definitive diagnosis cannot be reached by simply measuring erythrocyte PK activity; rather, diagnosis requires measurement of glycolytic substrate accumulation and enzyme stability and immunologic or electrophoretic studies of erythrocyte PK.

Anemia, Hemolytic↗
PubMed1985

Dialysis osteodystrophy. A study involving 94 patients.

Dynamic skeletal histomorphometry was performed in 94 unselected patients receiving maintenance dialysis for chronic renal failure. An attempt was made to correlate the results with the clinical, biochemical and radiological findings. Skeletal histology was abnormal in each case. Hyperparathyroidism was present as the only abnormality in 18 patients and osteomalacia in 26; 50 patients showed both abnormalities. Osteomalacia, in contrast to hyperparathyroidism, increased in prevalence and severity with the duration of dialysis and with bone aluminum content. The majority of patients had histological osteosclerosis. It was impossible to predict either the nature or the severity of the histological lesions on the basis of symptoms and physical signs or on the basis of most biochemical parameters (including serum concentrations of three vitamin D metabolites). Serum alkaline phosphatase values and serum immunoreactive parathyroid hormone (iPTH) concentrations were positively correlated with the severity of histological hyperparathyroidism. Subperiosteal erosions of the phalanges were associated with severe histological hyperparathyroidism in each case but this radiological sign was absent in 66% of patients with histological hyperparathyroidism. Radiological osteosclerosis was associated with severe histological osteomalacia in each case, but this radiological sign was absent in 87% of patients with histological osteomalacia. No other radiological sign proved a reliable guide to the underlying skeletal histology. In the majority of dialysis patients, a skeletal biopsy is required for an accurate diagnosis of the nature and severity of azotemic osteodystrophy.

Adolescent↗
PubMed1985

[Risk of bone disease as a result of fluoride intake in chronic renal insufficiency].

Four cases of osteosclerosis were observed in patients with renal failure. All subjects presented with moderate reduction in renal function which had been stabilized for several years. Osteosclerosis appeared progressively but was clinically symptomatic in only one patient. Fluoride intoxication was ascertained on the basis of X-ray examination and bone biopsy. In addition, the source of fluoride intoxication was easily recognized as the drinking water (2 to 3 1/day), "Vichy Saint Yorre" commercial mineral water (fluoride concentration 9 mg/l) in 3 cases, and tap water in the fourth case. These observations emphasize the risk of high chronic fluoride intake in patients with renal failure, even with mild reduction of glomerular filtration rate.

Adult↗