Cases reported "Bone Diseases"

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1/10. bacteremia and skin/bone infections in two patients with X-linked agammaglobulinemia caused by an unusual organism related to Flexispira/helicobacter species.

    Two patients with Bruton's X-linked agammaglobulinemia are described with bacteremia and skin/bone infection due to an organism which by 16S rRNA gene sequence analysis was most closely related to "Flexispira" rappini (and thus designated a Flexispira-like organism, FLO) and more distantly related to the helicobacter species. The organism required microaerobic conditions and, supplemental H(2) gas for growth and was reliably stained with acridine orange. In common with helicobacter cinaedi infections, the focus of the FLO infection was in one case in the blood vessels or lymphatics of an extremity and in the other case in the skin and adjacent bone of an extremity. In both cases, prolonged IV antibiotic therapy was necessary to clear the infection. The susceptibility of XLA patients to FLO infection appears to be related to the fact that XLA is associated with severe B cell (humoral) immunodeficiency and thus these patients have difficulty with intravascular or intralymphatic infection. These findings elucidate the nature of FLO infections in humans and point the way to their detection and treatment.
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2/10. technetium-99m MDP bone scintigraphic findings of hypercalcemia in accelerated phase of chronic myelogenous leukemia.

    hypercalcemia in accelerated phase of chronic myelogenous leukemia (CML) is very rare. Its pathogenesis is considered humoral hypercalcemia of malignancies mediated by parathyroid hormone-related protein (PTHrP). In severe hypercalcemia, calcifications in kidneys, skin, vessels, heart, and stomach may occur. Our two cases were admitted because of severe hypercalcemia in accelerated phase of CML. On Tc-99m methylene diphosphonate (MDP) bone scintigraphies, a marked tracer accumulation was seen in the lung, heart, stomach and kidney. We report increased tracer accumulation of multiple organs on Tc-99m MDP bone scintigraphy in two rare hypercalcemic patients with CML.
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3/10. Intraosseous cholesterol granuloma of radius showing a lamellar structure on MR images: a case report.

    We report a case of an intraosseous cholesterol granuloma in the radius showing a lamellar structure on MRI. T1-weighted imaging revealed a central area of mildly increased signal intensity with a surrounding low-intensity layer. On T2-weighted images, the central lesion demonstrated low-intensity signals, and the surrounding area exhibited a lamellar structure with three layers of high, low, and high intensity from the inside. On histological examination, the central area was found to consist of cholesterine crystals embedded in erythrocytes, and in the surrounding lamellar structure collagenous tissue may have provided the low signals, with vessel- and giant cell-enriched structures present at both the inner and outer margins, presumably evoking the high-intensity signals. The present lesion may have been formed by chronic hemorrhage into a preexisting simple bone cyst.
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4/10. Free osteocutaneous lateral arm flap: anatomy and clinical applications.

    For many surgeons, the potential to reconstruct skin, fascia, tendon, or bone in a single-stage procedure has made the lateral arm flap the technique of choice for reconstruction of complex defects. The aim of this study was to examine more closely how the humeral bone is supplied by the posterior collateral radial artery. To this end, we dissected 30 cadaver arms to determine the vascular relationship of the lateral arm flap to the humerus. The number of directly supplying vessels, and height to the lateral epicondyle of the humerus, were examined. The reconstructive potential of the osteocutaneous flap in different indications is analyzed in a series of five clinical cases. In all dissected extremities, we found one or two branches of the posterior collateral artery directly and constantly supplying the bone between 2-7 cm proximal to the lateral epicondyle. In five cases, combined defects, including bone, were successfully reconstructed with lateral arm flaps, including vascularized bone.
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5/10. Gray-scale and Doppler characteristics of fibrous cortical defects in a child.

    We report the case of a 9.5-year-old boy who underwent sonographic and radiographic examinations because of knee pain. Two fibrous cortical defects were discovered, in his right femur and left tibia; the femoral lesion was seen more easily on sonography than on radiography. On gray-scale sonography, the lesions were characterized as a scalloped indentation of the cortical surface containing hypoechoic soft tissue. On color Doppler sonography, discrete vessels were found at the periphery and within the substance of the lesions, and on spectral Doppler analysis, low-resistance arterial flow was detected in those vessels. Follow-up examinations performed 10 and 19 months after our initial examination showed signs that the lesions were healing. The diagnosis of fibrous cortical defect was suggested by the gray-scale and Doppler sonographic characteristics and confirmed on radiography. Although radiography is required to document these lesions if found incidentally on sonography, follow-up examinations using only sonography may be feasible.
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6/10. Diffuse pulmonary uptake of 99mTc bone-imaging agents: case report and survey.

    Over the past 5 years, we have encountered 6 cases of diffuse pulmonary uptake of 99m-Tc bone-scanning agents (incidence, 0.04%). To assess the significance of this phenomenon, we reviewed all of the cases reported since 1974 (Including our series, a total of 32 cases). Three groups can be discerned, the first consisting of 24 patients without radiological calcifications in the lungs and with hypercalcemia of different origins (mostly hyperparathyroidism). Of the eight autopsies performed in this group, seven revealed extensive calcifications in alveolar walls and lung vessels; the other autopsy showed no calcification at all and only bronchopneumonic lesions. The second group consists of 6 patients in chronic dialysis. The last group consists of 2 patients having diffuse pulmonary alveolar microlithiasis with extensive radiologic calcifications. The mechanism of lung uptake of 99m-Tc bone-imaging agents is probably the same as that of bone uptake (chemisorption on hydroxyapatite crystals), although other uptake mechanisms have also been discussed. Bone scintigraphy can be useful in the detection of early pulmonary calcifications, which have been associated with impaired pulmonary function and, due to their size, are generally not detected by X-ray.
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7/10. Skeletal complications of gaucher disease.

    gaucher disease is a collection of related disorders of sphingolipid catabolism caused by the deficiency of a specific beta-glucosidase. The inefficiency of this enzyme, glucocerebrosidase, to degrade its natural substrate leads to the accumulation of the complex lipid glucocerebroside in tissue macrophages. The pathogenesis of the disease is, as yet, poorly understood. The manifestations of the disease are protean with hepatosplenomegaly and bone deterioration frequently the predominating signs. The disease most frequently causes disability because of its effects on the skeleton. This review seeks to summarize the current clinical understanding of these complications. Experience with 327 patients reveals that the bone disease in this disorder is extremely variable. The severity of the problems range from asymptomatic persons with neither radiographic, scintigraphic, nor histologic evidence of bone involvement to those whose skeleton is completely devastated by a process of osteopenia, osteonecrosis, and osteosclerosis. These severely affected individuals show the most bizarre deformities in their bones and are subject to pathologic fracture. Most patients fortunately, are less profoundly affected, but many are plagued by bone pain of an arthritic nature or by an acute prostrating bone crisis probably best described as a bone infarction. The accepted etiology that these crises are a result of vascular compromise produced by occlusion of vessels by Gaucher cells is not supported by scintigraphic or histologic studies. Moreover, the vascular hypothesis does not explain the variety of lesions of the skeleton seen in this multifocal bone disease. Preliminary metabolic and endocrinologic studies suggest that this is not a systemic disorder of metabolism which affects bone uniformly. On the contrary, the lesions are multiple and localized, and sometimes much of the skeleton is preserved. These observations suggest that bone is affected because of collections of Gaucher cells scattered throughout its substance and may be the result of a toxic process around these foci. Alternatively, the storage of glucocerebroside in tissue macrophages may disturb the generation of competent osteoclasts and thus result in a failure to maintain a healthy skeleton. Further research is needed to delineate the pathogenesis of this disorder before any effective therapy can be developed.
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8/10. Vascularized periosteal bone graft from the supracondylar region of the femur.

    Free, vascularized thin corticoperiosteal grafts and small periosteal bone grafts harvested from the supracondylar region of the femur are described. These grafts are nourished from the articular branch of the descending genicular artery and vein. Thin corticoperiosteal grafts consist of periosteum with a thin layer of outer cortical bone and include the cambium layer, which has a better osteogenic capacity. This graft is elastic and readily conforms to the recipient bed configuration. Thin corticoperiosteal grafts were used to treat 11 patients with fracture non-union of an upper extremity that had no massive bone defects. Early, rapid union occurred in all patients except three: one in which the anastomosed vessel became obstructed and two in which the internal fixation of the fracture was unsecured. The small bone grafts consist of periosteum, full thickness cortex, and the underlying cancellous bone. This graft can be successfully harvested without disturbing the vascularity, unlike the currently used vascularized bone grafts. This graft was used to treat three patients with avascular necrosis of the body of the talus and could prevent the necrotic talus body from progressive collapse in patients in early stages of the disease. One patient with an infectious bone defect of the first metatarsal bone was successfully treated by vascularized bone graft with an accompanying skin flap.
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9/10. magnetic resonance imaging of petrous carotid aneurysms.

    Aneurysms arising from the petrous segment of the internal carotid artery are rare and the majority of reports are based on evaluations with x-ray catheter angiography (XRA) and computed tomography (CT). Due to the paucity of magnetic resonance imaging (MRI) information, the author describes 2 patients with petrous carotid aneurysms evaluated with MRI and magnetic resonance angiography (MRA). Compared to XRA and CT, MRI and MRA are less invasive and provide more information regarding the aneurysm, including the size, shape, and site; the absence or presence and extent of hemorrhage, thrombus, and bony erosion; patency of the vessel; neck; and the extent of perianeurysmal compromise of vessels, cranial nerves, and parenchyma.
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10/10. Cranial and intracranial aspergillosis of sino-nasal origin. Report of nine cases.

    This paper is an attempt at defining the most efficacious surgical and antifungal therapy for invasive cranial and intracranial aspergillosis, and is based on experience with nine non-immunocompromised patients treated and followed-up by the authors between 1983 and 1994; as well as on the summary of previously reported cases and advances in therapy of this condition. Depending on the degree of aspergillar involvement of the cranial base and intracranial structures, a classification, with implications for treatment and prognosis, is also proposed. Two patients had extracranial skull base erosion; whereas relentlessly progressive granulomas, mimicking malignancy, invaded the skull base and intracranial contents in seven cases. Of these seven patients with cranial and intracranial invasion, two died of acute intracranial haemorrhage due to fungal invasion of cerebral blood vessels. In two patients, complete surgical eradication of the disease proved impossible due to cavernous sinus involvement, while residual aspergillomas are still present in orbit and paranasal sinuses (PNS) in a further two patients in spite of multiple surgical procedures and prolonged antifungal chemotherapy (AFC). What appears to be a cure has been effected in one patient only. Multiple therapeutic strategies were used. biopsy plus systemic AFC was ineffective, surgical drainage and debridement plus systemic AFC resulted in long-term survivals but no cure. Radical surgery in conjunction with systemic and local (intracavitary) AFC should be considered to improve an otherwise poor prognosis.
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