[A clinical study of the siblings of Sanfilippo B syndrome (MPS III B)].
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Biomedical subjects
Publications and source records attributed to H Koide.
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Acrinol in dry solid state after gamma irradiation with 80 kGy (8 Mrad) undergoes 8% decomposition, with G(-M)=30, and in wet solid state (10% moisture content) undergoes 15% decomposition, with G(-M)=56, as shown by absorbed spectrometric measurement. From these results, it is estimated that the irradiation dose of 25 kGy (2.5 Mrad) prefered by many countries as sterilization dose will achieve the radiolysis rate of 2.8% or 4.7% for dry solid state or wet solid state acrinol, respectively. The stickiness power of plaster tape made of raw rubber and polyterpene resin don't decrease with irradiation dose of less than 40 kGy (4 Mrad). Then, the radio-sterilization on the dose of 25 kGy (2.5 Mrad) may be applied to solid pharmaceuticals such as commercial rubber adhesive plaster with acrinol pad. Besides, acrinol in 0.1% aqueous solution after irradiation of 10 kGy (1 Mrad) at room temperature undergoes 20% decomposition, with G(-M)=0.5. The radiolysis rate in this state is estimated 45% with the dose of 25 kGy (2.5 Mrad). Then, the radio-sterilization with this dose must not be applied to liquid pharmaceuticals such as 0.1% acrinol aqueous solution. While, it was reported by Hosobuchi and Sato that the antimicrobial effect of irradiated acrinol to Staphlococcus aureus increased with irradiation dose. Then, it is expectable that some materials with antimicrobial activity are prodused by gamma irradiation.
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Glomerular basement membrane (GBM) were isolated from the kidneys of rats suffering from daunomycin nephrosis or nephrotoxic serum nephritis. The GBM from daunomycin nephrotic rats contained significantly less hydroxyproline, hydroxylysine and glycine than that of control rats. There was an increase in glucosamine content in the membrane. No significant change was found in the neutral sugar content. In nephrotoxic serum nephritis, the relative amounts of hydroxyproline, glycine and half-cystine were decreased, whereas the relative amounts of aspartic acid, alanine, lysine and hydroxylysine were increased. The ratio of hydroxyproline to proline and the ratio of hydroxylysine to lysine were decreased. An increase in sialic acid content and a decrease in fucose and hexosamine content and glucosyl-galactosyl-hydroxylysine content were noted in nephrotoxic nephritic GBM. These chemical structural alterations could accounted for the functional disorders of diseased GBM.
Radiolysis of bilirubin and cholic acids (cholic acid, desoxycholic acid and lithocholic acid) in hydrous pellet have been investigated with following parameters, which were hydrous content, radiation dose and the dose rate, to discuss the application of gamma-irradiation for sterilization of crude drug pill involving bezoar bovis. At 774 C/kg (3.0 MR) irradiation for 5% and 10% hydrous contents pellets, the radiolysis percent of those components were less than 5%. However, the higher hydrous pellet, the radiolysis percents of those are more increase. At the same irradiated condition for 20% hydrous contents pellets, the radiolysis percents of those were 15-22%. The hydrous percent of commercial crude drug pill involving bezoar bovis are about 9%, so that the radiolysis of those components will be less than 5% on the sterilization. The radiolysis percent of bilirubin are constant to variation of radiation dose rate between 51.6-722.5 C/kg.hr (0.2-2.8 MR/hr). But, the values of cholic acids don't definite such as that of bilirubin, because of larger analytical error.
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Development of daily rhythms in the chick heart rate was investigated during the post-hatching period. The measurements were made in free-moving chicks at one-hour intervals under different light conditions. Clear daily rhythms were seen 3 days after hatching under LD (12:12 hr.) whereas no daily rhythm was observed under either constant illumination or constant dark. The present study demonstrated that while heart rate increased with age during the post-hatching period, the light-time heart rate increased more rapidly than the dark-time heart rate. It was also found that the daily rhythms in heart rate were not formed after food removal even under LD. These results indicate that the light and feeding play an important role in the development of the daily rhythms in chick heart rate.
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