Rate of disease progression during long-term follow-up of patients with late-onset Pompe disease
ABSTRACT To determine the rate of disease progression in patients with late-onset Pompe disease, we collected longitudinal data on pulmonary function and skeletal muscle strength in 16 patients whose symptoms had started in childhood or adulthood. The mean duration of follow-up was 16 years (range 4-29 years). During the follow-up period, eight patients (50%) became wheelchair bound and three (19%) became ventilator dependent. At a group level, pulmonary function deteriorated by 1.6% per year, and proximal muscle weakness progressed gradually. At the individual level, however, the rate and extent of progression varied highly between patients. In two thirds of patients, pulmonary function and muscle strength declined simultaneously and to the same extent. The remaining one third of patients showed a variable, sometimes rapidly progressive course, leading to early respirator or wheelchair dependency. These individual differences, especially in pulmonary dysfunction, indicate the need for regular monitoring every 6-12 months depending on the rate of disease progression.
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ABSTRACT: Describes bloodflow velocimetry using wavelet transforms and wideband signals. Acoustic signals with high fractional bandwidths and/or large time-bandwidth products are processed with wideband/wavelet techniques, thereby removing many of the narrowband assumptions typically invoked. The received signals are assumed to be reflected from particles moving in the blood stream. Instead of measuring the Doppler shift associated with the reflection, the time-scaling of the signal is obtained. This time-scaling more accurately reflects the effects of motion on the signals than does a Doppler shift since a Doppler shift is an approximation to time-scaling. The continuous wavelet transform processing is then used to obtain the axial velocity of scatterers
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ABSTRACT: Microfabricated platinum redox sensors can be used for indication in redox diffusional microtitration of microliter size body fluid samples. The sensor can be deposited onto different substrate materials. Adsorption of reagent and/or sample on alumina ceramic substrate versus Pyrex glass substrate is compared. It is demonstrated that in a classical iodimetric redox titration system, significant adsorption occurs on alumina ceramic substrate, causing irreproducible results. Reproducibility with a 1.7% coefficient of variation (CV) is achieved using a sensor deposited on Pyrex substrate for the analysis of 20 μL sample dropletsEngineering in Medicine and Biology Society, 1994. Engineering Advances: New Opportunities for Biomedical Engineers. Proceedings of the 16th Annual International Conference of the IEEE; 02/1994
Conference Paper: Optoelectronic computer-aided design[Show abstract] [Hide abstract]
ABSTRACT: The success of the modern silicon electronics industry can be attributed, in part, to the availability of advanced modeling and simulation computer-aided design (CAD) tools. This modeling and simulation infrastructure is crucial for product design; without these tools, multiple design and fabrication iterations are required in order to optimize design and system parameters, a process which severely impacts cycle time and end-product cost. Because the field of optoelectronics has not yet reached the same level of product proliferation as the electronics industry, a corresponding CAD infrastructure does not yet exist for the modeling and simulation of optoelectronic devices, circuits, and systems. We address this issueBroadband Optical Networks and Technologies: An Emerging Reality/Optical MEMS/Smart Pixels/Organic Optics and Optoelectronics. 1998 IEEE/LEOS Summer Topical Meetings; 08/1998