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Primary lymphoma of bone (PLB) is a rare entity, defined as a lymphoma confined to the bone without evidence of systemic involvement. The disease commonly affects middle-aged to elderly population and it accounts for less than 1% of all malignant lymphomas. We present a case of a 10-year-old child affected by PLB of the forearm and the frontal bone. Characteristic imaging features of PLB and the main differential diagnosis were discussed. Keywords: Bone, Lymphoma, Paediatric

The purpose of the paper was to assess the morphometric parameters to improve the specificity of the ultrasound (US) signs for the early differential diagnosis between two lethal dysplasias, as thanatophoric dysplasia (TD) and osteogenesis imperfecta type 2 (OI-2). The diaphyseal length and the bowed shape of long bones associated with vertebral body dimension assessment were investigated in a group of 14 pregnancy terminations carried out in the time period 2007–2013. The definitive diagnosis was established after pregnancy termination by means of skeletal standardized X-rays, histopathology and gene analysis. TD and OI-2 long bones were significantly shorter than controls. No significant differences were observed between the two dysplasias. The bowing angle was higher in OI-2; a true angulation or eventually axial displacement was present only in the latter. Furthermore, they did not show any evidence of vertebral collapse. The thanatophoric dysplasia presented less bowed long bones, and never true angulation. The spine was steadily characterized by flattened anterior vertebral bodies. Long bone shortening is not a sufficient and accurate sign for early sonographic differential diagnosis between TD and OI-2. Angled diaphysis, axial diaphyseal displacement and a conserved vertebral body height in the prenatal period support the diagnosis of osteogenesis imperfecta type 2, while moderately regular bowed diaphysis associated with platyspondyly that of thanatophoric dysplasia.

ORTHOUNION is a multicentre, open, comparative, three-arm, randomized clinical trial (EudraCT number 2015-000431-32) to compare the efficacy, at one and two years, of autologous human bone marrow-derived expanded mesenchymal stromal cell (hBM-MSC) treatments versus iliac crest autograft (ICA) to enhance bone healing in patients with diaphyseal and/or metaphysodiaphyseal fracture (femur, tibia, and humerus) status of atrophic or oligotrophic nonunion (more than 9 months after the acute fracture, including recalcitrant cases after failed treatments). The primary objective is to determine if the treatment with hBM-MSCs combined with biomaterial is superior to ICA in obtaining bone healing. If confirmed, a secondary objective is set to determine if the dose of 100 × 106 hBM-MSCs is noninferior to that of 200 × 106 hBM-MSCs. The participants (n=108) will be randomly assigned to either the experimental low dose (n=36), the experimental high dose (n=36), or the comparator arm (n=36) using a central randomization service. The trial will be conducted in 20 clinical centres in Spain, France, Germany, and Italy under the same clinical protocol. The confirmation of superiority for the proposed ATMP in nonunions may foster the future of bone regenerative medicine in this indication. On the contrary, absence of superiority may underline its limitations in clinical use.

Polyethylene (PE) wear is a focal issue in joint replacement, so it is essential to understand how it takes place in vivo. PE wear is a multifactorial process with a complex interaction of variables related to the materials used, the mechanical conditions, operative procedures for implantation, activity of the patient and use of prosthesis. We retrieved 65 PE inserts with the respective femoral and tibial components (50 inserts of total knee prostheses and 15 monocompartmental prostheses) from first revision surgeries. The average age of the patients was 68.3 years; the average time to revision was 41.5 months. Macroscopic observation considered the grade, topography and type of wear. Degradation was also studied with scanning electron microscopy. No direct relationship was found between the level of wear and the survival of total or monocompartmental knee prostheses. The duration of 11 (22%) total prosthetic inserts with grade 2 wear was 42.6 months vs. 51.5 months of 17 (34%) total inserts with grade 1 wear. However, study of the relationship between wear grade and wear topography in total inserts suggested that there was a significant connection. In fact, the 22 (44%) prostheses with central and symmetrical wear never showed wear greater than grade 1. Instead, of the remaining 28 prostheses (56%) without central and symmetric wear, only 3 had grade 0 wear. Finally, considering the relationship between wear grade and type of wear, all 18 inserts (27.7%) with grade 2 or 3 wear had 100% delamination. In conclusion, this study suggests that the correct positioning of the prosthetic components, besides its quality, is an important cause of polyethylene wear.

Bone aging was studied in an experimental model (rabbit femur) in three populations aged 0.5, 1.5, and 7.5 years. Cortical bone histology was compared with a data set from a 1.5-month-old population of an earlier published paper. From 0.5-year-old onward, the mean femur length did not increase further. Thereafter, the mean marrow area increased and the cortical area decreased significantly with aging. This was associated with a structural pattern transformation from plexiform to laminar and then Haversian-like type. The distal meta-epiphysis bone trabecular density of the oldest populations also was significantly lower in specific regions of interest (ROI). Percentage sealed primary vascular canals in laminar bone significantly increased with aging without variation of percentage sealed secondary osteons. Remodeling rate reflected by the density of cutting cones did not significantly change among the age populations. These data suggest that laminar bone vascular pattern is more functional in the fast diaphyseal expansion but not much streamlined with the renewal of blood flow during secondary remodeling. Bone aging was characterized by: 1) secondary remodeling subendosteally; 2) increment of sealed primary vascular canals number; 3) increased calcium content of the cortex; 4) cortical and trabecular bone mass loss in specific ROIs. Taken together, the present data may give a morphological and morphometric basis to perform comparative studies on experimental models of osteoporosis in the rabbit. J. Morphol. 276:733–747, 2015. © 2015 Wiley Periodicals, Inc.

Documentation through X-ray morphometry and histology of the steady phenotype expressed by FGFR3 gene mutation and interpolation of mechanical factors on spine and long bones dysmorphism. Long bones and spine of eight thanatophoric dysplasia and three age-matched controls without skeletal dysplasia were studied after pregnancy termination between the 18th and the 22nd week with X-ray morphometry, histology, and molecular analysis. Statistical analysis with comparison between TD cases and controls and intraobserver/interobserver variation were applied to X-ray morphometric data. Generalized shortening of long bones was observed in TD. A variable distribution of axial deformities was correlated with chondrocyte proliferation inhibition, defective seriate cell columns organization, and final formation of the primary metaphyseal trabeculae. The periosteal longitudinal growth was not equally inhibited, so that decoupling with the cartilage growth pattern produced the typical lateral spurs around the metaphyseal growth plates. In spine, platyspondyly was due to a reduced height of the vertebral body anterior ossification center, while its enlargement in the transversal plane was not restricted. The peculiar radiographic and histopathological features of TD bones support the hypothesis of interpolation of mechanical factors with FGFR3 gene mutations. The correlated observations of X-ray morphometry, histopathology, and gene analysis prompted the following diagnostic workup for TD: (1) prenatal sonography suspicion of skeletal dysplasia; (2) post-mortem X-ray morphometry for provisional diagnosis; (3) confirmation by genetic tests (hot-spot exons 7, 10, 15, and 19 analysis with 80–90 % sensibility); (4) in negative cases if histopathology confirms TD diagnosis, research of rare mutations through sequential analysis of FGFR3 gene.

Numerous systems of osteosynthesis of olecranon fractures are avaible. The numero-ulnar articulation badly bears the smallest incongruence and easily faces rigidity. Therefore the aim of therapy is the anatomical and permanent reconstruction of the articular surface of the olecranon, in order to mobilize the elbow as soon as possible. We analyzed 85 olecranon fractures, classified according to Merle d'Aubigne and treated with two different surgical techniques (intramedullart screw and AO tension band wiring technique). The results were compared at an average follow-up of 88 months. Excellent clinical results were obtained in 74,12%, good in 29.00%, sufficient 3,53%, insufficient in 2,35% of patients, with a mean of recovery in 2 months and only 2 cases of pseudoarthrosis (with dynamic procedure). Objective results were similar for two techniques adopted, whereas the subjective results in patients with screw. The screw is more suitable in medio-olecranic and basis-olecranic fractures, while the dynamic procedure gives better results in fractures of the apex of olecranon and comminute fracture. Tolerance of the intramedullary screw has generally proved to be better (65% of cases), while the dynamic assembling has been removed in most cases after fracture consolidation. Periarticular calcifications correlated with the type of fracture (e. g. comminuted, associated) rather than with the means of synthesis employed.

Two cases of pyomyositis in babies, one in the ilio-psoas muscle, the other in the quadriceps, are reported. The presenting sign was a painful hip in both, suggesting osteomyelitis of the proximal femur or arthritis. Pyomyositis is unusual in western countries and in healthy, well-nourished children, but these cases suggest considering this pathology in the differential diagnosis of a painful hip in babies. In the first case an upper respiratory tract infection was followed by haematogenous spread to the ilio-psoas muscle; in the second an injection of vaccine in the quadriceps played a determinant role. The latter case was complicated by secondary osteomyelitis; a metaphyseal radiolucent band just below the growth plate appeared in the early stage together with metaphyseal osteopenia and was interpreted as a consequence of altered blood flow in the metaphysis, not specific osteomyelitis, but indicative of some disturbance of the growth plate-metaphyseal complex.

I report a case of bilateral spontaneous atraumatic anterior subluxation of the sternoclavicular joint in a 19-year-old woman without any known underlying pathology. There was no history of injury. The patient was treated conservatively. One year later the patient was asymptomatic and had returned to her usual activities.

To compare the outcomes of two different surgical treatments for the management of isolated closed Mason Type 2 radial head fractures.Retrospective study. The Student t test and McPearson chi-square test were used to evaluate whether there was a significance difference between the groups.Fifty-nine patients with isolated Mason Type 2 radial head fractures.Twenty-four patients treated with radial head excision (Group I) and 35 treated with open reduction and internal fixation (Group II).Clinical outcomes were assessed using the Broberg and Morrey functional rating scores and the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire. Orthogonal radiographs were performed on both the elbow and the wrist; these were assessed for the presence of arthritis, heterotopic ossification, and the degree of proximal radial migration.The length of postoperative follow-up was 157 ± 61.84 months (Group I) and 125 ± 39.09 months (Group II). The Broberg and Morrey functional rating score was 86.21 ± 6.10 points and 95.09 ± 4.78 points, respectively. The DASH score was 21.82 ± 6.01 points and 2.81 ± 2.73 points, respectively. Radiologically moderate or severe osteoarthritis was present in the elbows of nine patients in Group I and only two patients in Group II.Patients with isolated Mason Type 2 radial head fractures treated by open reduction and internal fixation (Group II) had less residual pain, greater range of motion, and better strength than patients treated by radial head excision (Group I). Additionally, Group II had a lower incidence of severe posttraumatic arthritis, which contributed to improved DASH and Broberg and Morrey functional scores. These results support open reduction and internal fixation as the treatment of choice for these fractures.Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

A patient with a total hip replacement developed optic, acoustic and peripheral neuropathy from metal ions intoxication, due to the wear products released from the prosthesis. Subsequently the kinetics of the metal ions was studied.Massive wear and acute intoxication allowed a study of the metal ions kinetics and of EDTA treatment.Plasma and other organic fluids were saturated by each of the metal ions released from the exposed surface according to the solubility of each ion; a larger fraction of Co ions was bound within red cells, while the plasmatic fraction appeared more movable. In a patient with a prosthesis subjected to wear, the ions released are from the prosthetic and from the debris surface (spread in the body). The latter is a function of the number and size of particles.Revision of the prosthesis from the point of view of the metal ions kinetics corresponded to a reduction of the releasing surface because of debris washed out by irrigation and tissue excision; however, the metal particles spread by lymphatic circulation continued to release ions even though the source of wear had been removed. Early diagnosis of high metal wear can be ascertained with mass spectrometry and after revision high levels of metal ions can only be reduced with repeated chelating treatment. It is preferable not to revise fractured ceramic components with a polyethylene-metal articulation.

In September, 2007, a 58-year-old woman was admitted to our neurology department because of progressive visual and hearing loss which had started 9 months earlier. She had type 2 diabetes and hypertension, both of which were adequately controlled by medication. In 2001, she had a left hip arthroplasty which was revised in October, 2006 because of rupture of the ceramic head. 3 weeks before admission, investigations showed a mild hypothyroidism of unknown origin, which was treated with levothyroxine sodium. The neurological examination at admission showed impairment of cranial nerves II and VIII bilaterally and mild distal sensory-motor dis turbances. Laboratory investigations ruled out haema tological, infectious, neoplastic, metabolic, and immuno logical diseases. Concomitantly our patient underwent various investigations including brain MRI (showing hyperintensity of optic nerves and tracts), electromyography (showing mild lower limb nerve amplitude reduction), and acoustic and visual evoked potentials (positive for bilateral absence of brainstem acoustic responses and irregular cortical visual responses). A working diagnosis of axonal multi-neuropathy caused by a presumed immune-mediated vasculitis was made and the patient was given prednisone (50 mg/day) for 2 months with little improvement. By December, 2007, our patient became completely blind, severely deaf, and wheel-chair bound because of lower limb hyposthenia. Tests for immune-mediated process remained negative and the case was referred to toxicology for further investigation. Unexpectedly, raised concentrations of cobalt and chromium were found in diff erent biological samples (cobalt: 24 h urine collection 1187 μg/L [0·1–1·5], blood 549 μg/L [0·05–2·7], plasma 90 μg/L [0·1–0·6], CSF 11·4 μg [0·05–0·15]; chromium: 24 h urine collection 510 μg/L [0·05–2·2], blood 54 μg/L [0·1–0·5], plasma 210 μg/L [0·1–0·5], CSF 4·4 μg/L [0·01–0·2]). Cobalt-chromium poisoning due to metal wear debris from her hip prosthesis was proposed, although radiology, including CT, showed no sign of prosthesis loosening. In February, 2008, several metal ion chelating treatments were given with edetic acid. Although metal ion concentrations decreased, neurological improve ment was negligible. Therefore, in April, 2008, resection arthroplasty was done. During surgery infi ltration of the peri-prosthesic tissue by metallic debris was evident (fi gure A); analysis of peri-prosthetic fl uids showed high concentrations of cobalt and chromium, and the removed prosthesis showed wear of the head and neck (fi gure B), supporting the hypothesis of endogenous cobalt-chromium poisoning. During the following 8 months the patient showed progressive improvement, although vision only partially improved. Metal ion concentrations decreased but remained higher than reference values at the last follow-up in November, 2008. The role of cobalt or cobalt-chromium on human tissues has not been defi nitively established. Cobalt can produce various toxicological eff ects including local respiratory symptoms due to inhalation of cobalt containing dusts, and systemic eff ects (thyroiditis, cardiomyopathy, erythropoiesis). Neuro logical toxicity, mainly optic atrophy, nerve deafness, and limb paraesthesia, has been occasionally reported in association with exogenous exposure. Neuro logical toxicity as a reult of endogenous exposure (mainly associated with metal prostheses) has been described. Cobalt can induce a hypoxia-like eff ect, possibly targeting mitochondria; of note, our patient’s symptoms were similar to those observed in some mitochondrial cytopathies. Total hip replacement and hip resurfacing arthroplasty are widely used therapeutic procedures; longer-term follow-up would be necessary to evaluate adverse chronic systemic eff ects due to prolonged exposure to high serum cobalt concentrations. In addition to orthopaedic evaluation, careful neurological and toxicological examinations are recommended whenever a patient with a metallic prosthesis complains of visual loss, hearing disturbance, limb weakness, numbness, or paraesthesia, even in the absence of local osteoarticular symptoms.

The chondrodysplasia punctatas (CDP) are a group of genetic diseases presenting with the common hallmark of epiphyseal calcific deposits. A 21.3 week female fetus with a clinical and molecular diagnosis of X-linked dominant CDP (CDPX2 or Conradi-Hunermann syndrome) has been investigated with particular attention to the morphology of the calcific deposits (stipplings). A variable combination of normal endochondral ossification centers, calcific deposits, and a mixture of these two were observed in the lumbar spine. The calcific deposits were characterized by a growing phase, corresponding to the expansion of calcification, a steady phase, and a resorbing/remodeling phase, when calcification was progressively reduced through different biological mechanisms. The pathological mechanism of cartilage matrix calcification seemed to influence the morphology and the distribution pattern of vessels inside the epiphysis. These findings fit well with the radiographic observations of progressive regression of stippling with advancing age of the child and also give a morphological correlation.

We present a child with irregular ossification of tubular bone epiphyses, short bones, and spine. The radiographic evolution of bones undergoing endochondral ossification was followed from the age of 1 year 9 months to 6 years. The unusual features demonstrated in this child made classification difficult: pseudoachondroplasia was excluded because no mutations of the COMP gene were found. Considering the evolution of the radiographic appearances, the most likely diagnosis would seem to be an unusual form of spondyloepiphyseal dysplasia, mimicking some aspects of multiple epiphyseal dysplasia. Endochondral ossification was diffusely altered with a mixture of epiphyseal ossification delay associated with acceleration and early fusion. This case was a unique presentation within the family, suggesting a mutation in the affected child.

The surgical treatment of aseptic nonunion often represents a more challenging situation for the orthopaedic surgeon than treatment of the primary fracture. In fact, it may be necessary not only to "rivitalize" the nonunion area, but also to exchange the bone fixation devices and to place some refill material in the bone gap. Several surgical techniques and different kinds of bone gap refills have been reported in the literature for the treatment of long bone nonunion. We present the results of 193 cases of long bone nonunion that have been treated in a period of 11 years (1992–2003) by a mostly open approach to the nonunion site with or without autologous bone graft interposition. The site (27 humerus, 44 forearm, 48 femur, 74 tibia) and the type of nonunion (179 atrophic, 19 hypertrophic) were considered in the surgical planning as were the mechanic and biological problems. New osteosynthesis was performed in 139 cases: with plate and screws in 82 cases, with intramedullary nails in 31 cases, with external fixators in 15 cases and with other devices in 11 cases (e.g. interfragmentary screws, k-wires). Cancellous or corticocancellous bone graft, always autologous from the iliac crest or from the anterior tibial tuberosity, was used in 183 cases (94.8%). Healing of the nonunion was successful in 179 cases (92.7%) in a mean time of 5.8 months. 14 patients (7.2%), all atrophic nonunion, healed with further surgery in a mean time of 19.2 months. Best results were obtained by the use of the intramedullary nail (31 cases): 99% healed in 5.2 months for the lower limb and 100% healed in 7.4 months for the upper limb. Good results have been achieved by plate (82 cases): 89.5% healed in 4.5 months for the lower limb and 94.1% in 6 months for the upper one. The worst results were observed with external fixation (15 cases). However, this device was used in the most complex situations, when severe soft tissue sufference was present. In this group, the mean healing time was 7.1 months (69.2% of cases) in the lower limb and 8 months (50%) in the upper one. Bone graft alone (54 cases) led to healing in 34 of 35 cases (97%) in the lower limb in 6 months and in 17 of 19 cases (89.4%) in 6.4 months in the upper limb.

The radiographic follow-up of a patient with Proteus syndrome is presented. Review of radiographs obtained at 3 years 10 months, 10 years, and 17 years 8 months indicated that the rate of growth in length of the oversized tubular bones of the hands was similar to that of the normal bones of the same hand. This observation supports the view that the primary lesion occurs in the early embryonic period, when the limb bud mesenchyme cells condense and cartilage differentiates producing oversized cartilage anlages, rather than being a defect of bone cell-mediated apposition and modelling processes of bone. Additional radiographs of the pelvis and spine were obtained at age 4 years 10 months and head CT at 8 years 10 months. This pathogenetic mechanism fits well with the hypothesis of somatic mosaicism, which is at present the most credible explanation for the aetiology of Proteus syndrome. Other skeletal malformations recognized as typical of the syndrome can be interpreted as secondary adaptations to the altered mechanical conditions induced by overgrowth of bones.

A skeletal dysplasia with previously unreported features is presented. Its evolution was characterized by growth abnormalities of bones without involvement of other organs. Advanced bone age, increased stature and irregular epiphyseal ossification with stippling of the main long bones were documented. Physeal overgrowth was massive in the left proximal humerus and femur. Furthermore, the hip joint appeared fused with an abundant mass of pathological calcific tissue extending from the femur to the ilium. Pathological epiphyses were characterized by anarchic cartilaginous proliferation with multiple ossification centres, while lamellar bone apposition and remodelling were normal. The observed bone changes were different from those in any previously reported syndrome, metabolic defect or bone dysplasia. However, they clearly indicated a defect of endochondral ossification with some resemblance to phenotypes observed in dysplasia epiphysealis hemimelica.

1 Iannuzzi MC, Rycicki BA, Teirstein AS. Medical progress: sarcoidosis. N Engl J Med 2007; 357: 2153–65. 2 Newman LS, Rose CS, Bresnitz EA, et al. A case control etiologic study of sarcoidosis: environmental and occupational risk factors. Am J Respir Crit Care Med 2004; 170: 1324–30. 3 Kucera GP, Rybicki BA, Kirkey KL, et al. Occupational risk factors for sarcoidosis in African-American siblings. Chest 2003; 123: 1527–35. 4 Rafnsson V, Ingimarsson O, Hjalmarsson I, Gunnarsdottir H. Association between exposure to crystalline silica and risk of sarcoidosis. Occup Environ Med 1998; 55: 657–60. 5 Rybicki BA, Iannuzzi MC. Epidemiology of sarcoidosis: recent advances and future prospects. Semin Respir Crit Care Med 2007; 28: 22–35. a cobalt-chromium head, or could the placement of a cobalt-chromium head in combination with the previously inserted ceramic socket explain the loss of cobalt-chromium? After the resection arthroplasty, the patient’s vision “partially improved”, while “metal ion concentrations decreased but remained higher than reference values”. These statements do not prove any clear relation to the operation. We believe this Case Report generates more questions than answers and leaves physicians with unnecessary concerns about the safety of total hip replacement. Furthermore, we believe this patient was left with a disabling condition. Did the authors panic and become blind and deaf to the evidence of the superior wear characteristics of modern hip prostheses?

This study investigated the effects of diphosphonates at scalar doses in a high bone turnover structure, namely, the proximal tibial metaphysis of rats. Arrest of bone modeling was represented by cylindrical-shaped metaphyses, increased height of the perichondrial bone bark, and persistence of primary metaphyseal trabeculae; these changes were dose-related. Higher doses of the inhibitors produced extension of the growth plate and arrest of the mineralization process. The dose-related dissociation between the effects on bone resorption and mineralization allows the therapeutic use of this class of drugs.

A case of disappearing bone disease of the proximal femur is reported with histopathological and immunohistochemical studies. There was a densely packed cellular tissue, positive to endothelial antibodies, in areas of massive bone destruction. A more differentiated vascular tissue was present where trabecular cancellous or cortical bone was preserved with only focal zones of accelerated bone remodelling. The self-limited course correlates well with two phases of evolution of the histopathological lesions with neoplastic-like proliferation of endothelial cells corresponding to the rapid and massive bone destruction, and a later differentiation of the cells in mature vascular structures, but still with accelerated bone resorption which is partly compensated by appositional activity.

Inhibition of osteoclastic bone resorption has been induced in growing rats with high doses of salmon calcitonin. This effect was evaluated by measuring the perichondrial ring height of the proximal tibial metaphysis. The aim was to assess whether osteoclastic activity resumed after a period of inhibition with high doses of calcitonin. 20 male Sprague-Dawley rats were treated for 21 days with 100 units/kg/day of salmon calcitonin subcutaneously and killed after 0-60 days, together with non-treated controls at 0 and 60 days. Arrest of metaphyseal modeling and increased height of the perichondrial ring at the end of the period of therapy (P 0.002 versus controls) were observed. Recovery of bone resorption was evident 20 and 40 days after withdrawal of calcitonin.

Quantitative assessment of bone resorption inhibition in vivo is not easily accomplished; methods relying on a count of osteoclasts are questionable, and histomorphometric evaluation of the bone mass presents several technical problems as well. The authors developed a simple method to measure the inhibition of bone resorption by study of the proximal tibial metaphysis of growing rats: the height of the perichondrial bone ring was taken as an index of the balance between osteoblastic and osteoclastic activity because any agent that inhibits osteoclasts (without interference with osteoblasts) produces an increase in the height of this anatomical structure. Since the ring is well demarcated by surrounding tissues, its height can be measured with accuracy and used for quantitative assessment of bone resorption inhibition. This model was tested with salmon calcitonin, and it provides evidence in vivo that this hormone inhibits osteoclastic bone resorption.

Salmon calcitonin (sCT) at doses of 100 and 50 UI given subcutaneously to growing rats produced in vivo evidence of osteoclastic activity inhibition. Histological assessment was carried out by measuring the perichondrial ring of Lacroix height, and a dose-correlated effect was found. These aspects were coupled with an increase in the osteoclast number and suggested that in studies with bone resorption inhibitors, morphological evaluation based on osteoclasts count is not reliable. The changes of the metaphysis suggested also that sCT affects the activity of hypertrophic chondrocytes of the growth plate. Plasma calcium levels did not differ significantly between treated rats and controls; an increased phosphatemia was observed in sCT-treated animals.

A child with myositis ossificans progressiva was treated for 8 years with ethane-1-hydroxy-1,1-diphosphonate (EHDP) 30–40 mg/kg per day. Latterly he complained of severe, progressive bone and joint pain which made standing and walking almost impossible. A radiographic skeletal survey showed diffuse ricket-like lesions. Withdrawal of EHDP therapy produced substantial improvement in his general condition as well as in the radiographic appearance of the bones. Multiple exostoses were observed in this case and, particularly those around the knees, presented a peculiar morphology. This supports the theory that exostoses originate from a defect of metaphyseal modelling.

An 8 year old child was involved in a road accident and sustained a large wound in the left groin; radiographs showed a fracture with loss of the proximal femur. After skeletal traction for 80 days, there was bony regeneration of the proximal femur. At 8 months she was able to walk without support and her left leg was 2 cm only shorter than the right.

Bone changes are a constant feature of mucolipidosis II, with striking differences between newborns and older children. Intracellular, membrane-bound vacuoles were found in the chondrocytes, osteoblasts, osteocytes, and stromal fibroblasts of three affected children. Osteoclasts and marrow cells were unaffected. Ricketslike lesions were present at birth in the two younger cases, whereas signs of high bone turnover and defective calcification were no longer present in the older child. Severe abnormalities of the metaphyseal plate with the loss of normal cartilage architecture and the absence of endochondral ossification were the major changes in this age group.

An in vivo histological and ultrastructural study of the cellular reaction to particulate material currently used in orthopaedic surgery produced evidence that, on a strictly cellular level, the main damage is done by the smallest particles produced by hip prostheses, i.e. metal particles, irrespective of differences in their chemical composition. Particle size and release rate are the critical factors, although other mechanisms of cellular damage may be active once granulation tissue is formed.

Macrophages and foreign-body giant cells from the bone-cement interface of loosened joint replacement were assayed for direct bone resorption in an in vitro experimental model. Human osteoclasts from giant cell tumors and experimental animal osteoclasts were used as controls. Macrophages and giant cells were not capable of resorbing the bone under experimental conditions, but osteoclasts are. It is suggested that macrophages and giant cells in loosened implants may initiate bone resorption, removing the extra-cellular barrier that normally protects mineral crystals from osteoclastic recognition.

Twelve cases of Mucolipidosis II (I-cell disease) with a wide range of severity of skeletal involvement were studied. Pathological findings in two cases provided helpful information in understanding the radiographic features of dysostosis multiplex. Inhibition of the growth plate cartilage calcification and rickets-like lesions were observed in the metaphyses. Enhanced subperiosteal remodelling and paratrabecular fibrosis were also evident in the diaphyses. High levels of parathormone were found in one case. This finding supports the hypothesis that bone lesions may be secondary, at least in part, to damage in such viscera as the kidney and/or the liver and that they are mediated by vitamin D and parathormone.

A femur bone metastasis from breast carcinoma was treated by curetting and filling with acrylic cement and osteosynthesis. The histological study of the resected proximal third of the femur five months after surgery shows a thin layer of connective tissue between bone and cement. There are no neoplastic cells in this connective tissue nor in the spongious bone of the proximal femur. Instead the diaphyseal channel is fully invaded with neoplastic cells. Pathogenesis and validity of surgical treatment by the emptying and filling with acrylic cement of bone metastases are discussed.

A genetic skeletal dysplasia with dwarfism, scoliosis and multiple skeletal defects was observed in two sisters. Only nine cases with similar features have been reported in the literature.

Histological examination of the bones obtained on autopsy of a 5-month-old child with mucolipidosis II (I-cell disease) revealed inhibition of the growth plate calcification with defective vascular invasion and signs of hyperparathyroidism. These findings are the chondro-osseous basis of the early radiological ricket-like appearance of bones in the neonatal period or soon thereafter. Whether the early skeletal abnormalities of mucolipidosis II result from a primary enzymatic defect of cartilage and bone cells or from factors controlling bone metabolism deserves further study.

A case of massive osteolysis of the arm is described where the typical bony lesions were associated with soft tissue lymphangiomatosis.

Results of reimplanted total hip prostheses and resection arthroplasty were compared. Using the Iowa hip rating scale, the reimplanted prostheses population was rated higher for function, gait, and absence of deformity, however, the resection arthroplasty population rated better in freedom from pain. Radiographic findings in the reimplanted prostheses population included radiolucent lines around the cement, loosening, and cortex bone stock loss. These findings cause concern for the future of these implants. Repair and remodeling of the bone lesions were observed in resection arthroplasties. It was concluded that if healing of infection is achieved or if resection is performed for mechanical failure, the results may be considered permanent.

Thirteen failed stem of Total Hip Replacement were studied: 9 were Charnley THR from an homogeneous series, which gives an incidence of 2.4% of stem fractures with a follow-up of 9–16 years; 4 were Mueller THR. Fatigue fracture of the stem occurred by defective support of the proximal part of the femur, following resorption of the calcar. In all cases reactive tissue to foreign body particles, metal and polyethylene, was found where bone resorption occurred. In Mueller THR wear of the cup produced the large amount of polyethylene particles; in Charnley THR metal particles prevailed and corrosion of the stem is suggested to be the initiating factor.

A generalized skeletal dysplasia with features of chronic idiopathic hyperphosphatasia and fibrous dysplasia of the mandible were observed in a 6-year-old child. The abnormal development of the bones resulted from enhanced remodeling and the failure of mature bone to form. The occurrence of the two lesions in the same child and a review of the literature support the hypothesis that pathogenesis of fibrous dysplasia and idiopathic hyperphosphatasia reflect an underlying common defect in the control of bone cell activity.

A case of synchronous multicentric osteosarcoma is reported in an 11-year-old boy. This very rare condition is characterized by simultaneous involvement of multiple skeletal sites, and the prognosis is generally poor.

Evolution of the early bone lesions in two children with mucolipidosis 2 was followed from birth. The progression of the bone changes did not differ from healing of rickets. Low levels of 1,25-(OH)2-D3 were found in one child and he was treated with vitamin D; resolution of the rachitic changes was more rapid than in the untreated child. It is suggested that in mucolipidosis 2 bone reacts to two independent factors, one controlling calcium metabolism, the other depending on the primary lysosomal enzyme defect. Since ricket-like features are not present in the other mucolipidoses or mucopolysaccharidoses, the defect of calcium metabolism seems to be related to the specific enzyme defect of mucolipidosis 2.

Macrophages and giant calls which ingested material particles in loosening of total hip prostheses were tested for their ability to resorb bone in vitro , using osteoclasts as the control. Macrophages and giant cells did not form pits or resorption lacunae on the bone substrates as osteoclasts did. The results support the view that around implants also bone resorption is mediated by osteoclasts. A role of macrophages in the attachment phase of bone resorption is suggested.

A man of 71 was seen with a solitary metastasis in the patella as the first presentation of carcinoma of the lung.

The authors prospectively evaluated 45 patients (60 feet) affected by hallux valgus and treated with a distal metatarsal osteotomy. The surgical procedure consisted of a modified Mitchell osteotomy, in which fixation was achieved with a Kirschner wire that was driven into the proximal osteotomy fragment and buttressed the distal one. Early weightbearing was allowed without a cast. Follow-up averaged 25 months. The mean American Orthopedic Foot and Ankle Society clinical hallux score increased from 44.6/100 preoperatively to 83.2/100. Radiographic evaluation showed that mean metatarsophalangeal and intermetatarsal angles decreased respectively from 31.7 degrees to 16.9 degrees, and from 15.4 degrees to 8.6 degrees. Short-term loss of correction occurred in three cases (4%). Six feet (10%) had unrelieved metatarsalgia that was related to excessive shortening of the first metatarsal and/or inappropriate orientation of the metatarsal head. Stabilization of the Mitchell osteotomy with a Kirschner wire proved safe and effective for the surgical correction of mild to moderate hallux valgus.

Membranous lipodystrophy occurred in a 35-year-old woman and membrane-like material was found in her epiphyses and short bones. Electron microscopy allowed a study of the relationship between the pathologic tissue and normal fat, suggesting that the membrane-like material is derived from degenerated cells. Nucleation of hydroxyapatite crystals was also observed in the membranous material. Because basal ganglia calcifications were demonstrated with the use of computed tomography, brain and bone lesions may be assumed to share the same pathogenesis. Lipid and mucopolysaccharide metabolism was found to be normal.

Introduction: We aimed to assess the incidence and hospitalization rate of hip and "minor" fragility fractures in the Italian population.Methods: We carried out a 3-year survey at 10 major Italian emergency departments to evaluate the hospitalization rate of hip, forearm, humeral, ankle, and vertebral fragility fractures in people 45 years or older between 2004 and 2006, both men and women. These data were compared with those recorded in the national hospitalizations database (SDO) to assess the overall incidence of fragility fractures occurring at hip and other sites, including also those events not resulting in hospital admissions.Results: We observed 29,017 fractures across 3 years, with hospitalization rates of 93.0% for hip fractures, 36.3% for humeral fractures, 31.3% for ankle fractures, 22.6% for forearm/wrist fractures, and 27.6% for clinical vertebral fractures. According to the analyses performed with the Italian hospitalization database in year 2006, we estimated an annual incidence of 87,000 hip, 48,000 humeral, 36,000 ankle, 85,000 wrist, and 155,000 vertebral fragility fractures in people aged 45 years or older (thus resulting in almost 410,000 new fractures per year). Clinical vertebral fractures were recorded in 47,000 events per year.Conclusions: The burden of fragility fractures in the Italian population is very high and calls for effective preventive strategies. © 2010 Tarantino et al.; licensee BioMed Central Ltd.