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PubMed2000

Increased bone formation and osteosclerosis in mice overexpressing the transcription factor Fra-1.

Bone formation by osteoblasts is essential for skeletal growth and remodeling. Fra-1 is a c-Fos-related protein belonging to the AP-1 family of transcription factors. Here we show that transgenic mice overexpressing Fra-1 in various organs develop a progressive increase in bone mass leading to osteosclerosis of the entire skeleton, which is due to a cell-autonomous increase in the number of mature osteoblasts. Moreover, osteoblast differentiation, but not proliferation, was enhanced and osteoclastogenesis was also elevated in vitro. These data indicate that, unlike c-Fos, which causes osteosarcomas, Fra-1 specifically enhances bone formation, which may be exploited to stimulate bone formation in pathological conditions.

Animals↗
PubMed1997

Structure and chromosomal assignment of the mouse fra-1 gene, and its exclusion as a candidate gene for oc (osteosclerosis).

We have determined the genomic structure of the mouse fra-1 gene, which consists of four exons and three introns at positions also found in the other members of the fos gene family. Fra-1 is expressed rather highly in the brain and testes of adult mice, and at low levels in most other tissues. Absence of c-Fos leads to significantly reduced serum stimulation of fra-1 expression in gene targeted mouse fibroblasts, demonstrating that mitogen induction of fra-1 is partially mediated by c-Fos/AP-1. A polymorphic (CA)n microsatellite marker was found in intron 2 of fra-1 and used to map the gene to the centromeric region of mouse chromosome 19. Since fra-1 maps to the same genomic region as oc (osteosclerosis), an autosomal recessive disorder leading to the bone remodelling disease osteopetrosis, we tested it as a candidate gene for oc. The segregation of fra-1 in two different crosses of mice carrying oc and an allelism test between oc and a targeted disruption of fra-1 demonstrate that fra-1 and oc are two distinct genes rather than oc being a mutant allele of fra-1.

Amino Acid Sequence↗
PubMed2002

Idiopathic osteosclerosis of the jaws followed through a period of 20-27 years.

AIM: The aim of the present study was to examine the prevalence of idiopathic osteosclerosis (IO) in a baseline sample and to follow the patients through an extended period of time. METHODOLOGY: The sample consisted of 210 patients treated in a dental school and having a complete series of intraoral radiographs at the time of treatment and at a follow-up 10-17 years later. A further 10 years later, 130 patients had another follow-up. RESULTS: Sixteen of the 210 patients were found to have IO, mostly in the mandibular molar and premolar regions. Fourteen had one lesion, one had two lesions and one had three lesions. The mean age of these patients was 47 years as compared to 44 years for the rest of the patient group. No sex predilection was found. At the first follow-up, one lesion had disappeared, one had reduced in size and one new lesion appeared. At the second follow-up, eight patients with IO could be re-examined. Two lesions, unchanged at the first follow-up, had reduced in size and two new lesions appeared. In the area of the new lesions, residual roots were observed in the earlier radiographs. CONCLUSION: Our findings support the theory that IO lesions should be considered anatomical variants. In some cases, however, a local aetiological agent may cause development of structures with an identical appearance.

Adult↗
PubMed1982

[Acro-osteosclerosis of the fingers, a normal sex- and age-dependent endosteal reaction].

Acro-osteosclerosis (AOS) of the distal phalanges was estimated in a semi-quantitative manner in a sample of patients with polyarthritis and compared with a sample of non-rheumatic patients, corresponding in age and sex incidence. AOS must be regarded as a normal endosteal process which occurs particularly in women; it begins in early adult life and regresses with old age. There was no relationship between AOS and chronic polyarthritis; nevertheless, the bone 'terrain' in chronic polyarthritis is not identical with that in normals.

Adult↗
PubMed1998

Human autosomal recessive osteopetrosis maps to 11q13, a position predicted by comparative mapping of the murine osteosclerosis (oc) mutation.

Autosomal recessive osteopetrosis is a rare congenital disorder characterized by the development of abnormally dense bones, acrocephaly, severe anemia, hepatosplenomegaly and progressive deafness and blindness. The clinical course is rapidly progressive and is lethal at a very young age in the absence of a bone marrow transplant. The failure to remodel developing bone that is the basis of the disease process is most likely due to a dysfunction of the bone resorptive cell, the osteoclast. This phenotype is similar to that of the murine mutation osteosclerosis (oc), which is localized to proximal mouse chromosome 19. Given the similarity between the human and murine phenotypes, we tested whether human osteopetrosis maps to a region of conserved synteny. Microsatellite markers in the region of 11q12-13 were found to be linked to osteopetrosis in two consanguineous Bedouin kindreds. Recombination events were used to define the disease interval to an approximately 14 cM region between D11S1983 and D11S2371. A maximum LOD score of 7. 94 was obtained with D11S449 at straight theta = 0.

Animals↗
PubMedDate not supplied

Osteosclerosis, a recessive skeletal mutation on chromosome 19 in the mouse.

Osteosclerosis (oc) is an osteopetrotic mutation in the mouse inherited as an autosomal recessive on chromosome 19. Affected animals (oc/oc) exhibit the characteristic radiologic and histologic hallmarks of osteopetrosis including a generalized increase in skeletal density and absence of marrow cavities. Most die within three weeks after birth. Osteoclasts are cytologically abnormal by light microscopy in that they do not have cytoplasmic vacuoles. Presumptive evidence of rickets in this mutation includes thick cartilagenous growth plates and excessive osteoid. Extensive extramedullary hemopoiesis occurs in the liver and spleen of mutants. This unique constellation of features suggests that the oc mutation is a valuable model in which to investigate the pathogenesis of osteopetrosis.

Animals↗
PubMed1979

Osteosclerosis (punctate form) in multiple myeloma.

Generalized punctate and nodular osteosclerosis associated with multiple myeloma is reported with review of the literature and differential diagnoses. This patient differs from some others reported earlier in the absence of any recognized osteolytic lesions either during life or at autopsy.

Diagnosis, Differential↗
PubMed1988

Acute monoblastic leukemia with osteosclerosis and extensive myelofibrosis.

A 4-month-old infant was admitted with a monoblastic infiltration of the skin associated with osteosclerosis. Both lesions spontaneously disappeared within a few months, but 2 years later, a monoblastic leukemia occurred that was associated with marked skin erythema and myelofibrosis. Skin and bone marrow specimens showed a monoblastic infiltration with numerous intermingled mast cells of normal appearance. Whether myelofibrosis was a feature of a systemic mastocytosis or of the leukemic process is discussed in this case.

Humans↗
PubMed1994

Clinical and haematological improvement induced by etidronate in a patient with idiopathic myelofibrosis and osteosclerosis.

We report a patient with agnogenic myeloid metaplasia associated with debilitating bone pain due to increased bone turnover and osteosclerosis. Treatment with etidronate at a dose of 6 mg/kg per day on alternate months resulted in a complete recovery of bone symptoms and normalization of metabolic parameters of bone turnover; unexpectedly, a sustained haematological improvement was also observed after several months of therapy, suggesting that bone marrow microenvironment improvement was able to restore a nearly normal haemopoiesis. We suggest that diphosphonate therapy may be of value in patients with AMM and increased bone turnover.

Aged↗
PubMed1980

Distal osteosclerosis.

Hyperostosis of the bones of the forearms and lower legs and mild cranial sclerosis were present in five persons in two generations of a South African kindred of mixed ancestry. This disorder, which is clinically innocuous, is inherited as an autosomal dominant trait. Delineation of this disorder to distinguish it from the other inherited sclerosing bone dysplasias permits accurate prognosis and genetic counselling. In view of the unusual anatomical distribution of the abnormalities, the designation "distal osteosclerosis" is proposed for this entity.

Adult↗
PubMed1983

Chronic myelocytic leukemia with marked myelofibrosis and osteosclerosis.

An autopsy case of a 50 years old woman who manifested chronic myelocytic leukemia (CML) with secondary myelofibrosis (SMF) is reported. The interesting point of this case was a marked diffuse myelofibrosis (MF) with osteosclerosis, so that it was difficult to make a diagnosis of either CML or idiopathic myelofibrosis (IMF), but both clinical and histological findings supported the diagnosis of CML with SMF. Her long term busulfan therapy was suspected as being the cause of this severe MF. The relationship of CML and IMF was also discussed.

Biopsy↗
PubMed1998

A histomorphometric study of haematological disorders with respect to marrow fibrosis and osteosclerosis.

A retrospective investigation of 75 EDTA-decalcified Jamshidi biopsies collected over a 2-year period at Aarhus University Hospital was performed. The biopsies originated from 75 patients suffering from idiopathic myelofibrosis, other chronic myeloproliferative disorders, or other conditions with known associations with bone marrow fibrosis. The relative volumes of trabecular and woven bone, as well as haematopoietic and non-haematopoietic tissue, were estimated histomorphometrically. The degree of fibrosis was estimated semiquantitatively. Finally, the thickness of trabecular osteons was estimated from the number of lamellae by counting. Patients with idiopathic myelofibrosis had statistically significantly more bone tissue than the other groups of patients. The osteosclerosis was primarily due to woven bone. Larger cancellous osteons also suggested a positive balance in bone remodelling. The amount of bone tissue showed furthermore a statistically significant increase through the groups of polycythaemia vera, essential thrombocythaemia, chronic myelogenous leukaemia and idiopathic myelofibrosis. Parallel to the increase in the amount of bone, an increase in the degree of marrow fibrosis was detected. The positive correlation between the amount of bone and the degree of marrow fibrosis was statistically highly significant (p=0.0008).

Adult↗
PubMed1989

Osteosclerosis of the phalanges in Werner syndrome.

Werner syndrome is an autosomal-recessive disease characterized by premature aging, shortness of stature, scleroderma-like skin changes, endocrine abnormalities, and cataracts. Although radiographic findings have been well documented, the presence of distinctive osteosclerotic changes in the phalanges of the hands and feet has not been emphasized in previous publications. The authors' review of radiographs of both hands in nine patients and of both feet in six patients with Werner syndrome documented the frequent occurrence of phalangeal sclerosis related predominantly to endosteal thickening. In the hand, sclerosis was present in every patient, was generally symmetric in distribution, predominated in the distal phalanges, and demonstrated an ulnar predilection. Similar changes in the phalanges of the feet were demonstrated in only two patients. The presence of osteosclerosis in the phalanges of the hand alone or both the hand and foot, when combined with osteoporosis and periarticular calcification, suggests the diagnosis of Werner syndrome.

Adult↗
PubMed1998

Insulin-like growth factor system abnormalities in hepatitis C-associated osteosclerosis. Potential insights into increasing bone mass in adults.

Hepatitis C-associated osteosclerosis (HCAO) is a rare disorder characterized by a marked increase in bone mass during adult life. Despite the rarity of HCAO, understanding the mediator(s) of the skeletal disease is of great interest. The IGFs-I and -II have potent anabolic effects on bone, and alterations in the IGFs and/or IGF-binding proteins (IGFBPs) could be responsible for the increase in bone formation in this disorder. Thus, we assayed sera from seven cases of HCAO for IGF-I, IGF-II, IGF-IIE (an IGF-II precursor), and IGFBPs. The distribution of the serum IGFs and IGFBPs between their ternary ( approximately 150 kD) and binary (approximately 50 kD) complexes was also determined to assess IGF bioavailability. HCAO patients had normal serum levels of IGF-I and -II, but had markedly elevated levels of IGF-IIE. Of the IGFBPs, an increase in IGFBP-2 was unique to these patients and was not found in control hepatitis C or hepatitis B patients. IGF-I and -II in sera from patients with HCAO were carried, as in the case of sera from control subjects, bound to IGFBP-3 in the approximately 150-kD complex, which is retained in the circulation. However, IGF-IIE was predominantly in the approximately 50-kD complex in association with IGFBP-2; this complex can cross the capillary barrier and access target tissues. In vitro, we found that IGF-II enhanced by over threefold IGFBP-2 binding to extracellular matrix produced by human osteoblasts and that in an extracellular matrix-rich environment, the IGF-II/IGFBP-2 complex was as effective as IGF-II alone in stimulating human osteoblast proliferation. Thus, IGFBP-2 may facilitate the targeting of IGFs, and in particular IGF-IIE, to skeletal tissue in HCAO patients, with a subsequent stimulation by IGFs of osteoblast function. Our findings in HCAO suggest a possible means to increase bone mass in patients with osteoporosis.

Alkaline Phosphatase↗
PubMed1978

Hereditary hypophosphataemic rickets with autosomal recessive inheritance and severe osteosclerosis. A report of two cases.

We have observed congenital hypophosphataemic rickets in two sons of a marriage between first cousins, their mother being clinically and biochemically normal. Both patients are now approaching middle age. In addition to severe childhood rickets and lifelong hypophosphataemia, their disease is characterised by gross osteosclerosis with extraskeletal ossification, clinically persistent osteomalacia in one and spinal cord compression in the other. The genetics of this disease can be satisfactorily explained only on the basis of autosomal recessive inheritance, a mode which has only once before been reported in the literature. The severity of certain features, which would be expected in a homozygous state, may help our understanding of the more usual X-linked form.

Adult↗
PubMed1999

Strange osteosclerosis at femoral neck.

A 4 (1/2) yr follow-up of bone mineral density of a young woman revealed a dramatic 84% increase at the femoral neck, whereas the increase at the trochanter was only 11%. This finding suggested a very substantial accumulation of bone mineral in the neck region only. In the immediate repeated DXA scan, however, all the signs of this osteosclerosis disappeared. The primary reason for this strange DXA finding was a discrete contact failure in the control electronics of the scanner that confused the analysis of the scan data. The standard quality control was unable to detect this malfunction.

Absorptiometry, Photon↗
PubMed2003

Bone densitometry of a patient with osteosclerosis.

A 30-yr-old Caucasian man with a history of dorsal and lumbar back pain, which responded partially to antiinflammatory agents, was seen at our Unit. The biochemical bone markers showed an increment in bone alkaline phosphatase and urinary CTX. Serum phosphate tended to be low. Radiographic abnormalities were marked osteosclerosis in the pelvis and vertebral bodies without changes in size. Bone scintigraphy results were normal. The increase in bone mineral density (BMD) was greater in L2-L4 (+ 3.9 SDs) than in total skeleton (+ 1.4 SDs). Analysis of skeletal subareas showed a marked increase in axial skeleton BMD: trunk, +4.0 SDs; spine, +2.5 SDs and pelvis, +4.5 SDs. BMD of the remaining subareas was found to be normal: skull, +0.04 SDs; arms, -0.3 SDs and legs, -0.05 SDs. The patient refused to have a bone biopsy. The radiologic, densitometric, and biochemical findings in the patient presented herein are compatible with axial osteomalacia. Evaluation of total skeleton BMD, and especially skeletal subareas, clearly indicated that the abnormal BMD was restricted to the spine and pelvis whereas the rest of the skeleton was not affected.

Absorptiometry, Photon↗