Structure-activity study of diuretic azanaphthalene derivatives.
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Biomedical subjects
Publications and source records attributed to Y Oka.
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A pregnancy from a family in risk of I-cell disease was monitored. The fetus was diagnosed as having I-cell disease based on the findings that (1) lysosomal enzyme activities except for acid phosphatase and alpha glucosidase were clearly elevated in amniotic fluid and were reduced in cultivated amniotic fluid cells, and (2) cytoplasmic inclusions were seen in cultivated amniotic cells by phase contrast microscopy. The accuracy of prediction was confirmed by cultured skin fibroblast of the aborted fetus.
Pregnancy from a family at risk for fucosidosis was monitored. The fetus was diagnosed as having a carrier state of the disease. alpha-L-fucosidase activity, however, was found to be absent in white blood cells obtained from identical twins after delivery. The diagnostic evaluation of (1) the enzyme activity in amniotic fluid and in cultivated amniotic fluid cells and (2) the presence of fucose rich compound in amniotic fluid are discussed.
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Two brothers, 6 and 13 years old, had histidinemia. On the basis of clinical and biochemical observations, the younger boy was considered to have a classical type of the disease, while the older boy had an atypical form characterized by partial impairment of the skin histidase activity and a moderately prolonged half-life of blood histidine. The mother is a heterozygous carrier, while the father and sister seem to be normal.
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A simplified dynamic method of calibration for dye-dilution curves is described. The method involves appropriate matching of the fluid volume in the mixing chamber, the blood withdrawal rate, and the calibration dose of dye. It was used to compare the observed versus calibrated pump output of a heart-lung machine with individual dye-dilution curves obtained from 15 patients during total cardiopulmonary bypass. The results indicate that the difference (range+14% to-8%) between the two is statistically insignificant. The method is rapid and relatively simple. It is particularly useful for cardiac output determination during unsteady clinical conditions because of the ability to calibrate dye curves at the time they are recorded.
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