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slowly progressive mental retardation. Cherry-red spots in the maculae, corneal opacities, and ataxia have been noted in some patients. Vacuolation of lymphocytes, marrow cells, hepatocytes, and Kupffer cells in the liver and metachromatic changes in the sural nerve have been described. In mucolipidosis II ( I-cell disease), the most common of the mucolipidoses, there is usually an early onset of psychomotor retardation, but in some cases this does not appear until the second or third decade. Abnormal facies and periosteal thickening (dysostosis multiplex, like that of GM1 gangliosidosis and Hurler disease) are characteristic. Gingival hyperplasia is prominent, and the liver and spleen are enlarged; but deafness is not found and corneal opacities are slower to develop. Tonic-clonic seizures are frequent in older patients. In most cases, death from heart failure occurs by the third to eighth year. There is a typical vacuolation of lymphocytes, Kupffer cells, and cells of the renal glomeruli. Bone marrow cells are also vacuolated and contain refractile cytoplasmic granules (hence the designation inclusion-cell, or I-cell, disease). A de ciency of several lysosomal enzymes required for the catabolism of mucopolysaccharides, glycolipids, and glycoproteins have been found. In mucolipidosis III (pseudo-Hurler polydystrophy), the biochemical abnormalities are like those of I-cell disease, but there are clinical differences. In the pseudo-Hurler type, symptoms do not appear until 2 years of age or later and are relatively mild. Retardation of growth, ne corneal opacities, and valvular heart disease are the major manifestations. Yet another variant, so-called mucolipidosis IV, has been described (see Tellez-Nagel et al). Here, clouding of the corneas is noticed soon after birth, and profound developmental retardation is evident by 1 year of age. Skeletal deformities, enlargement of liver and spleen, seizures, or other neurologic abnormalities are notably lacking. Ultrastructural examination of conjunctival and skin broblasts has demonstrated lysosomal inclusions of material similar to lipids and mucopolysaccharides that remain to be further characterized. Mannosidosis This is another rare hereditary disorder with poorly differentiated symptomatology. The onset is in the rst 2 years, with Hurler-like facial and skeletal deformities, mental retardation, and slight motor disability. Corticospinal signs, loss of hearing, variable degrees of gingival hyperplasia, and spoke-like opacities of the lens (but no diffuse corneal clouding) may be present. The liver and spleen are enlarged in some cases. Radiographs show beaking of the vertebral bodies and poor trabeculation of long bones. Vacuolated lymphocytes and granulated leukocytes are present and aid in diagnosis. The urinary mucopolysaccharides are normal. Mannosiduria is diagnostic, caused by a defect in mannosidase. Mannose-containing oligosaccharides accumulate in nerve cells, spleen, liver, and leukocytes (see Kistler et al). Fucosidosis This also is a rare autosomal recessive disorder, with neurologic deterioration beginning usually at 12 to 15 months and progressing to spastic quadriplegia, decerebrate rigidity, severe psychomotor regression, and death within 4 to 6 years. Hepatomegaly, splenomegaly, enlarged salivary glands, thickened skin, excessive sweating, normal or typical gargoyle facies, beaking of the vertebral bodies, and vacuolated lymphocytes are the main features. A variant of this disease has been described with slower progression and survival into late childhood and adolescence and even into adult life (Ikeda et al). The latter type is characterized by
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To avoid speci c assumptions related to the GPS satellite con guration, for the discussion of observability, we will assume that we can measure position
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which represents error of -1.927o.An approximate an estimateof the error can be determined usingEq. (21.9):
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Profit/loss by change in CAT common price $ 360 180 0 180 360 540 720 900 18.50 21.50 24.50 27.50 30.50 33.50 36.50 39.50 42.50 45.50
Database Vault Fundamentals
You can safely edit the Registry using the Control Panel in 9x, 2000, and XP,
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The important aspect of the script is not whether an element is a variable or constant, but whether the types of the variables and constants are the same. In this case, both variables and the constant are of the same type, int; therefore, they work together. The following script pair illustrates how C# and ASP.NET use constants and variables in concert.
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Major Moons of Outer Planets
Work the following problems to practice the commands and skills you learned in this chapter. 1. Create the composite solid shown in Fig. 44-9.
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