[A case of mastocytoma with leukemic manifestation of its immature form in the terminal stage (author's transl)].
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Biomedical subjects
Publications and source records attributed to K Fujimura.
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A kindred with hereditary spherocytosis was found to have erythrocyte glutathione reductase deficiency. The glutathione reductase was evaluated enzymatically and immunologically. The enzyme activity could not be restored either by flavin adenine dinucleotide (FAD) in vitro, or by oral administration of FAD. Kinetic and electrophoretic studies, heat stability tests, and pH curves revealed no evidence of a varient enzyme. The results of electroimmunodiffusion and neutralization tests using an antiserum indicated that the deficiency is caused by a diminished production of enzyme protein. Hereditary spherocytosis and glutathione reductase deficiency traits segregated independently. The amount of reduced glutathione in the erythrocyte was normal, and the deficiency state without hereditary spherocytosis was asymptomatic. All deficient members in this kindred were heterozygous and the residual enzyme activity in the heterozygote might be sufficient to maintain the reduced glutathione level to prevent oxidative stress.
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This report presents the results of operation for congenital heart disease using two different methods of hypothermia: (1) Immersion hypothermia alone. Of the 782 patients who underwent open-heart operations using this method the results were good in patients whose intracardiac surgical repair took less than one hour (average mortality rate, 5.6%). (2) Rapid extracorporeal cooling. Of the 269 patients with congenital heart diseases such as ventricular septal defect, tetralogy of Fallot, or atrioventricular canal with low cardiac reserve who underwent operation with mild to moderate hypothermia utilizing rapid extracorporeal cooling, the mortality was 11.2%. In the 151 patients with more serious defects, including the extreme form of tetralogy of Fallot, single ventricle, and truncus arteriosus, who underwent open-heart operations with deep hypothermia utilizing extracorporeal cooling, the mortality rate was 15.2%.
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The visually evoked response (VER) to dim lights with intensities within the scotopic or lower mesopic range increased in amplitude during the progressive dark adaptation. The VER amplitude vs. time curve resembled the psychophysical dark adaptation curve. The spectral sensitivity curve of the dark-adapted VER matched the C.I.E. scotopic sensitivity curve. The dark-adapted VER was abnormal in patients with retinitis pigmentosa and with congenital stationary night blindness and recordable in a patient with cone dysfunction. There was a close correlation between the VER threshold and subjective threshold of visual perception. The dark-adapted VER may be useful as an objective index of dark adaptation.
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