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BACKGROUND Bone disease is a serious complication of Gaucher disease. Untreated, it can result in pain, permanent bone damage and disability. Enzyme replacement therapy reverses many of the clinical signs of Gaucher bone disease but early assessment and treatment, and regular monitoring, are essential in optimising outcomes. SCOPE In September 2005, a(More)
Radiological imaging is used in patients with Gaucher disease to estimate the disease burden, to evaluate the presence of specific skeletal complications and to track response to therapy. MRI is currently the best technique for assessing bone marrow involvement in Gaucher disease. Gaucher cell infiltrated bone marrow is characterized by an abnormal low(More)
In Gaucher disease, enzyme replacement therapy usually reduces liver and spleen volumes and improves haematological abnormalities within 1 year. In contrast, skeletal manifestations of Gaucher disease are thought to respond more slowly. For example, decreased bone marrow glycolipid infiltration and increased bone mineral density have been reported to take(More)
Objective. To investigate the frequency and morphology of extraosseous extension in patients with Gaucher disease type I. Design and patients. MRI examinations of the lower extremities were analyzed in 70 patients with Gaucher disease type I. Additionally, the thoracic spine and the midface were investigated on MRI in two patients. Results. Four cases are(More)
. Since 1991, enzyme replacement therapy (ERT) has been available for patients with Gaucher disease in Germany. The aim of this study was to analyse the MR pattern of bone marrow involvement and response to ERT in Gaucher disease type I. . Thirty patients with Gaucher disease type I had MRI examinations prior to initiation of ERT with(More)
PURPOSE To evaluate the effect of ECG-controlled tube current modulation on radiation exposure in retrospectively-ECG-gated multislice CT (MSCT) of the heart. MATERIAL AND METHODS Three different cardiac MSCT protocols with different slice collimation (4 x 1, and 4 x 2.5mm), and a pitch-factor of 1.5 and 1.8 were investigated at a multi-slice CT scanner(More)
Bone stress injuries are rarely being diagnosed in patients with sensory neuropathy, most likely because they may be silent in terms of pain. Load-related pain is considered a key feature of any bone stress injury, a symptom, which may be partially or completely absent in subjects with sensory neuropathy (loss of protective sensation). We evaluated the(More)
Magnetic resonance (MR) images and concomitant plain radiographs of 26 Charcot feet of different stages (stage 0 to III) were reviewed in retrospect for discordant findings. Bone and joint abnormalities were evaluated qualitatively, and quantitatively guided by the AO integral classification of injuries of the foot. MR images revealed traumatic bone and(More)
The worst manifestation of neuro-osteoarthropathy of the pain-insensitive foot is the Charcot foot with its devastating osteoarticular destructions and irreversible deformities. New diagnostic tools such as MRI have revealed that mechanical injury and overuse is the origin of the condition. Traditionally, only feet with bone and joint damage apparent on(More)
PURPOSE Retrospective evaluation of postoperative long-term results after surgery of inflammatory aortic aneurysms (IAAA) with computed tomography (CT). Findings in CT were analysed with particular attention to the development of inflammatory tissue adjacent to the aneurysm site. MATERIAL AND METHODS Of 2101 patients operated on an aortic aneurysm 5.4%(More)