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Retarded bone formation in GM1-gangliosidosis: a study of the infantile form and comparison with two canine models (1995)

Alroy, J. ; Knowles, K. ; Schelling, S. H. ; [et al.]

Springer

Virchows Archiv 426 (1995), S. 141-148

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ISSN: 1432-2307

Keywords: Retarded bone formation ; GM1-gangliosidosis ; Canine model

Source: Springer Online Journal Archives 1860-2000

Topics: Medicine

Notes: Abstract The development of skeletal lesions in two canine models of GM1-gangliosidosis, English springer spaniels and Portuguese water dogs, has been studied and compared to osseous abnormalities in a child with the infantile form of the disease. In the canine models, skeletal dysplasia was progressive. Lesions were noted at 2 months of age and characterized by retarded endochondral ossification and osteoporosis. Older puppies had focal cartilage necrosis within lumbar vertebral epiphyses. At the cellular level, lesions were characterized by chondrocytic hypertrohy and lysosomal accumulation of storage compounds. Our studies illustrate that the skeletal lesions in both canine models are similar to those in a child with GM1-gangliosidosis. Furthermore, we proposed that the abnormal storage of partially degraded compounds in affected chondrocytes might explain, at least in part, the retarded bone formation noted in patients with GM1-gangliosidosis.

Type of Medium: Electronic Resource

URL: http://dx.doi.org/10.1007/BF00192635

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Computation of normal impinging jets in cross-flow and comparison with experiment (1991)

Knowles, K. ; Bray, D.

Chichester : Wiley-Blackwell

International Journal for Numerical Methods in Fluids 13 (1991), S. 1225-1233

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ISSN: 0271-2091

Keywords: Jets ; Vortex flows ; k-ε model ; Finite volume ; Engineering ; Engineering General

Source: Wiley InterScience Backfile Collection 1832-2000

Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics

Notes: The PHOENICS code has been used to model the flow field surrounding subsonic and underexpanded jets impinging on a ground plane in the presence of a cross-flow, for cases with both a fixed ground plane and a ‘rolling road’. The standard k-ε turbulence model is used, without correction factors. It is confirmed that this overpredicts the free jet entrainment rate; the wall jet spreading rate is slightly underpredicted but the initial thickness is too high. Agreement with experiment is, nevertheless, much better than for previous calculations, showing the importance of the extent of the grid used. The ground vortex formed in cross-flow is shown to move with varying effective velocity ratio and with rolling road operation in the same manner as experimentally observed. Ground vortex self-similarity is also accurately predicted with the numerical modelling.

Additional Material: 7 Ill.