• Clinical and Experimental Pediatrics
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• v.48 no.10
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• pp.1132-1138
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• 2005

Purpose : The mucopolysaccharidoses (MPSs) are a heterogeneous group of lysosomal storage disorders. They are caused by a deficiency of the enzymes involved in the degradation of glycosaminoglycans. Early recognition is important because recombinant enzyme replacement therapy is now available for MPS. We studied the clinical characteristics of 80 MPS children with the object of determining the epidemiological, clinical and radiological features in Korean MPS children. Methods : Diagnosis of MPS was confirmed by skin fibroblast enzyme analysis in 80 patients between February 1995 and December 2004. Charts were retrospectively reviewed for clinical and radiological findings, as well as for intelligence and speech evaluations. Results : Hunter syndrome (MPS type II) was the most prevalent type, appearing in 51/80 cases (64 %), followed by Sanfilippo syndrome (MPS III-18%), Hurler syndrome (MPS I-15%), and Morquio syndrome (MPS IV-4%). The average age at diagnosis was 5.5 years (range 1 to 20), and the male-to-female ratio was 4.7 : 1. Typical radiographic changes were observed in 45/54 cases (83%). Mitral regurgitation was the most common cardiac defect. Moderate to profound mental retardation and hearing loss were present in 14/35 cases (56%) and 33/38 cases (82%), respectively. Four MPS II patients had bone marrow transplantation, with mixed outcomes. Five MPS I patients are currently on enzyme replacement therapy. Conclusion : Our study showed a high proportion of MPS II cases (64%), which may represent population variability. By studying the clinical features of these patients, we hope to alert pediatricians of the warning signs of MPS.

• Fashion & Textile Research Journal
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• v.24 no.6
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• pp.667-685
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• 2022

This paper aims to investigate 4D printing materials for soft robots. 4D printing is a targeted evolution of the 3D printed structure in shape, property, and functionality. It is capable of self-assembly, multi-functionality, and self-repair. In addition, it is time-dependent, printer-independent, and predictable. The shape-shifting behaviors considered in 4D printing include folding, bending, twisting, linear or nonlinear expansion/contraction, surface curling, and generating surface topographical features. The shapes can shift from 1D to 1D, 1D to 2D, 2D to 2D, 1D to 3D, 2D to 3D, and 3D to 3D. In the 4D printing auxetic structure, the kinetiX is a cellular-based material design composed of rigid plates and elastic hinges. In pneumatic auxetics based on the kirigami structure, an inverse optimization method for designing and fabricating morphs three-dimensional shapes out of patterns laid out flat. When 4D printing material is molded into a deformable 3D structure, it can be applied to the exoskeleton material of soft robots such as upper and lower limbs, fingers, hands, toes, and feet. Research on 4D printing materials for soft robots is essential in developing smart clothing for healthcare in the textile and fashion industry.