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Gaucher disease, the most common lysosomal storage disorder, is a heterogeneous multisystem condition. Patients with non-neuronopathic (type 1) Gaucher disease may suffer from hepatomegaly, splenomegaly, thrombocytopenia, bleeding tendencies, anemia, hypermetabolism, skeletal pathology, growth retardation, pulmonary disease, and decreased quality of life.(More)
This retrospective study in 20 untreated type I Gaucher disease patients shows that in Dutch patients clinical manifestations of Gaucher disease type I are progressive in the majority of patients, children as well as adults. This is in contrast with studies among Jewish patients. Our results emphasize the need for a regular follow-up to enable timely(More)
Type 1 Gaucher disease can be effectively treated with enzyme supplementation therapy. Bone disease is a debilitating feature of the disorder and results from infiltration of the bone marrow by Gaucher cells. The effect of treatment on bone marrow infiltration is difficult to measure, necessitating the development of sensitive techniques to allow adequate(More)
OBJECTIVE To investigate the accuracy, procedure time, fluoroscopy time, and dose area product (DAP) of needle placement during percutaneous vertebroplasty (PVP) using cone-beam computed tomography (CBCT) guidance versus fluoroscopy. MATERIALS AND METHODS On 4 spine phantoms with 11 vertebrae (Th7-L5), 4 interventional radiologists (2 experienced with(More)
PURPOSE This investigation correlates the marrow response with the degree of increase in hemoglobin (Hb) and platelet count (Plt); decrease in concentrations of plasma tartrate-resistant acid phosphatase, serum angiotensin-converting enzyme (ACE), serum ferritin, and plasma chitotriosidase; and decrease in liver and spleen size, measured by ultrasonography.(More)
Magnetic resonance imaging (MRI) cell tracking has become an important non-invasive technique to interrogate the fate of cells upon transplantation. At least 6 clinical trials have been published at the end of 2010, all of which have shown that real-time monitoring of the injection procedure, initial engraftment, and short-term biodistribution of cells is(More)
PURPOSE In this paper, a method for the estimation of arterial hemodynamic flow from x-ray video densitometry data is proposed and validated using an in vitro setup. METHODS The method is based on the acquisition of three-dimensional rotational angiography and digital subtraction angiography sequences. A modest contrast injection rate (between 1 and 4(More)
Recently, soluble CD163 (sCD163) has been identified as a macrophage/monocyte-specific plasma protein and increased concentrations have been measured in patients with infection and myeloid leukaemia. In the present study we investigated the levels of sCD163 in patients with Gaucher's disease, an inherited lysosomal storage disorder characterised by hepato-(More)