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Giant cell tumors of the bones of the hand.

The cases of all patients with a diagnosis of giant cell tumor of bone occurring in the hand and seen at the Mayo Clinic during a 50-year period were reviewed to assess the results of treatment. There were 5 lesions in the phalanges, 7 in the metacarpals, and 1 in the scaphoid. The mean duration of symptoms and interval to recurrence were shorter than those seen in giant cell tumor of bone occurring in sites other than the hand. Radiographically advanced disease was common at presentation. Local recurrence was seen after 11 of 14 intralesional procedures (79%) involving curettage or curettage and bone grafting. Local recurrence was seen after 5 of 14 procedures (36%) involving local excision, wide excision, amputation, or ray resection. Lung metastases developed in 2 patients after or concurrent with local recurrence. Local control was most effectively achieved with wide excision or ray resection.

Adolescent↗

Joint remodelling and the evolution of the human hand.

A funtional morphological study has been made of the joints of the primate hand, particular emphasis being placed upon the carpometacarpal and metacarpophalangeal joints. The presumptive evolutionary history of these joints has been charted by reference to a comparative series of mammals. It has been demonstrated that the human joints have been quite strikingly modified in a number of ways, and that these evolutionary changes may be logically correlated with the refined functional attributes of the human hand. The morphological background thus established has been applied in a preliminary study of the hand bones of various fossil hominids.

Animals↗

Changes in skeletal mineral in patients on prolonged maintenance dialysis.

The measurement of bone loss in patients undergoing maintenance dialysis over a period of two and a half years is reported. The tendency to lose bone is a likely event in renal failure, but depends more on the individual patient than on the type of dialysis used, provided that steps are taken to prevent avoidable calcium loss during dialysis. Vitamin D therapy was an important factor in preventing bone loss in some cases. The tendency to lose bone more readily when both kidneys were absent may have reflected a deficiency of 1-25 dihydrocholecalciferol. On the other hand, bone loss was also observed in transplanted patients. The need to measure bone loss at regular intervals once renal failure has been diagnosed is stressed.

Adolescent↗

[X-ray anatomic features of the skeletons of twin children].

The size of right and left middle phalanges in the II-V fingers and the III finger have been studied in 108 pairs of monozygotic and dizygotic twins at 8--19 years of age and in 60 paris (pedagogical experiment) of separated twins (from the same pair), schoolchildren of the 2d--5th forms trained according different programs of physical culture. A great similarity of osteometric signs has been stated in monozygotic twins. As to local (intradigital) differences, a prevalence of the IIId finger should be mentioned. Maximum of genetic determination is evident for transversal growth--under high mechanical loadings on the bones, for longitudinal--under low mechanical loadings. The data of the pedagogical experiment demonstrate that increased mechanical loading on the hand bones activates osteogenesis. According to the majority of indices the main is the "radial" (II--III fingers) type.

Adolescent↗

Id2 and Id3 define the potency of cell proliferation and differentiation responses to transforming growth factor beta and bone morphogenetic protein.

Transforming growth factors beta (TGF-betas) inhibit growth of epithelial cells and induce differentiation changes, such as epithelial-mesenchymal transition (EMT). On the other hand, bone morphogenetic proteins (BMPs) weakly affect epithelial cell growth and do not induce EMT. Smad4 transmits signals from both TGF-beta and BMP pathways. Stimulation of Smad4-deficient epithelial cells with TGF-beta 1 or BMP-7 in the absence or presence of exogenous Smad4, followed by cDNA microarray analysis, revealed 173 mostly Smad4-dependent, TGF-beta-, or BMP-responsive genes. Among 25 genes coregulated by both factors, inhibitors of differentiation Id2 and Id3 showed long-term repression by TGF-beta and sustained induction by BMP. The opposing regulation of Id genes is critical for proliferative and differentiation responses. Hence, ectopic Id2 or Id3 expression renders epithelial cells refractory to growth inhibition and EMT induced by TGF-beta, phenocopying the BMP response. Knockdown of endogenous Id2 or Id3 sensitizes epithelial cells to BMP, leading to robust growth inhibition and induction of transdifferentiation. Thus, Id genes sense Smad signals and create a permissive or refractory nuclear environment that defines decisions of cell fate and proliferation.

Animals↗

Bone mineral status after renal transplantation. Assessment by noninvasive techniques.

RATIONALE AND OBJECTIVES: Bone mineral status in recipients of renal grafts is related to preexisting renal osteodystrophy and immunosuppressive treatment. The authors assessed bone mineral status after renal transplantation. METHODS: Bone mineral status of 20 male graft recipients was evaluated using the following noninvasive techniques: 1) neutron activation analysis, to measure hand bone phosphorus (HBP); 2) single photon absorptiometry, to measure forearm bone mineral content (BMC); and 3) single energy spectrum quantitative computed tomography, to measure spinal trabecular bone equivalent density (TBED). RESULTS: The mean (+/- SD, P) HBP, BMC, and TBED were found to be respectively, 4.8% (+/- 8.3%, P = .02), 6.6% (+/- 14.6%, P = .07) and 52% (+/- 8.5%, P < .001) lower than that measured in matched normal controls. Renal graft recipients demonstrated mean HBP and BMC decrements that were similar to those observed in matched patients on extrarenal dialysis, although the decrement in TBED was significantly greater in the graft recipients (P < .001). Repeated measurements performed during a 3-year period showed no statistically significant changes. CONCLUSIONS: Renal transplantation was associated with minor degree of osteopenia in the primarily cortical bone tissue. We speculate that the remarkably low TBED values in graft recipients does not reflect a mean 52% decrement of spinal bone minerals, but rather may be attributed in part to the deposition of adipose tissue in the spine as a result of corticosteroid treatment.

Absorptiometry, Photon↗

[Multifocal Langerhans cell granulomatosis. Report of a clinical case].

Langerhans' cell granulomatosis is a rare disease whose incidence on adults is valued about one case out of 3.000.000. The multifocal form is rarer than the single eosinophilic granuloma of the bone. It mainly affects males during childhood and it is usually localized in the long bones, hands, feet, and in the skull. We present the case of a young woman affected by multifocal Langerhans cell granulomatosis with bones and skin involved. Unusual in the presented clinical case is the age in which the disease come out. There is the highest incidence of multifocal lesions during childhood, while the monofocal kind is more typical of the years of adult age. Bone localization in the sternum is also quite unusual. In fact it is impossible to find reports of it in recent literature. As the patient refused to undergo chemotherapy she was treated with radiant therapy as for as bone localization concerned and with mecloretamin for local administration on skin lesion. Eleven months after the diagnosis we have not noticed the appearance of new lesions.

Adolescent↗

Giant cell reparative granuloma of the small bones of the hands and feet: a report of three cases.

Giant cell reparative granuloma (GCRG) involving the small tubular bones of the hands and feet is a rare entity that can have a wide range of morphologic presentations and can be confused with more aggressive tumors. Awareness of this lesion is important to avoid diagnostic errors and potential mismanagement. We report three cases of GCRG that involve the small tubular bones of the hands and feet, with long-term follow-up periods that confirm a benign course. Previous reports included the differential diagnosis of giant cell tumor, brown tumor of hyperparathyroidism, aneurysmal bone cyst, and non-ossifying fibroma. The presence of chondroid material in two of our cases, one of which also shows atypical nuclei and a periosteal reaction, expands the differential diagnosis to include bone- and cartilage-forming neoplasms.

Adolescent↗

Bone-density changes after stroke.

It has been many years since bone loss and fracture risk were first recognized as serious complications of stroke. Hip fracture is associated with a substantial increase in morbidity and mortality for stroke survivors, and therefore, assessing and maintaining skeletal health after stroke should be an important clinical goal. Recent long-term, prospective studies have illustrated a highly nonuniform pattern of bone changes after stroke. In general, there is significant bone loss on the paretic side, which is greatest in those patients with the most severe functional deficits. In some patients, bone loss in the paretic arm during the first year after stroke is the equivalent of >20 yrs of bone loss in healthy individuals of comparable age. Bone density in the nonparetic upper limb can actually increase after stroke, consistent with an increase in habitual use of the nonparetic hand. Bone density in the paretic lower limb can decrease by >10% in <1 yr, with smaller decreases being typical for the nonparetic lower limb. Despite the recent increase in the number of prospective, longitudinal studies, important questions about bone changes after stroke remain unanswered. Longer-term studies quantifying bone loss for periods of >12 mos poststroke are needed to determine how long excess bone loss continues after stroke. Studies with more subjects and with more varied disability levels are needed to better understand the relationships between functional deficits and bone loss. New metrics are needed to quantify the intensity and duration of physical activity in the upper and lower limbs that are consistent with previous research on the role of mechanical stimuli in bone adaptation. Finally, an assessment of skeletal health and the factors that affect bone quantity and quality should be a standard component in the clinical management of all survivors of stroke.

Absorptiometry, Photon↗

In vitro studies of transplantation antigens present on bone cells in the rat.

In vitro studies on isolated bone cells were undertaken to investigate the presence of transplantation (histocompatibility) antigens. Bone cells were cultured with allogeneic lymphocytes and exposed to cytotoxic sera containing antibodies against transplantation antigens, to determine their antigenic profile. Preliminary results suggest that bone cells may not express lymphocyte stimulating antigens in an active form, at least after the isolation procedure performed. On the other hand, bone cells were killed by cytotoxic antibodies in a specific way, providing evidence for the presence of serologically defined (SD) transplantation antigens on the cell surface. Additional studies with absorbed sera suggest "sharing" of histocompatibility antigens between bone cells and lymphocytes. The relevance of the surface antigens of bone cells to clinical fields such as bone allotransplantation, susceptibility to various orthopaedic diseases and skeletal sarcomata is discussed.

Animals↗

Giant cell reaction (giant cell reparative granuloma) of the small bones of the hands and feet.

Giant cell reactions (giant cell reparative granulomas) are uncommon lesions that predominantly involve the small bones of the hands and feet. In 1980, only 13 cases of this entity had been described. Three patients with similar giant cell reactions were identified from the Mayo Clinic files, and an additional 27 patients were collected from the consultation files of one of the authors. Clinical information and follow-up data for these 30 patients suggest that the biologic behavior and, therefore, treatment of giant cell reactions differ from those of true giant cell tumors in the small bones of the hands and feet.

Adolescent↗

Bone morphogenetic protein signaling in articular chondrocyte differentiation.

Articular chondrocytes progressively undergo dedifferentiation into a spindle-shaped mesenchymal cellular phenotype in monolayers. Chondrocyte dedifferentiation is stimulated by retinoic acid. On the other hand, bone morphogenic proteins (BMPs) stimulate differentiation of chondrocytes. We examined the mechanism of effects of BMP in chondrocyte differentiation with use of a recombinant adenovirus vector system. Constitutively active forms of BMP type I receptors (BMPR-IA and BMPR-IB) and those of activin receptor-like kinase (ALK)-1 and ALK-2 maintained differentiation of chondrocytes in the presence of retinoic acid. The BMP receptor-regulated signaling substrates, Smad1/5, weakly induced chondrocyte differentiation; the effects of Smad1/5 were enhanced by BMP-7 treatment. Inhibitory Smad, Smad6, blocked increase of expression of chondrocyte markers by BMP-7 in a dose-dependent manner. SB202190, a p38 mitogen-activated protein kinase inhibitor, inhibited this effect of BMP-7; however, since SB202190 suppressed phosphorylation of Smad1/5, this may be due to blockade of BMP receptor activation. These results together strongly suggest that induction of chondrocyte differentiation by BMP-7 is regulated by Smad pathways.

Activin Receptors, Type I↗

Multifocal plasmacytoma of hand and foot bones.

Simultaneous occurrence of localized plasmacytomas of both hands and feet has not been reported so far. Here we report a 40-year old female patient, who had at presentation pain and deformity. Of hands and feet, with numerous cystic lytic lesions of phalangeal, metacarpal and metatarsal bones, detected by X-rays. The biopsy of the affected bone showed moderately differentiated plasmacytoma of lambda light chain type (lambda-LC). Serum and urine biochemical analysis revealed the existence of lambda LC monoclonal component. The patient was treated by local radiotherapy and subsequent systemic chemotherapy, which consisted of 3 cycles of the M-2 protocol and 7 cycles of melphalan-prednisone. Five years after the diagnosis, the absence of plasmacytoma was confirmed by puncture biopsy of the left hand phalanx. Monoclonal protein in serum and urine was not detected.

Adult↗