[Congenital hemolytic jaundice].
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Biomedical subjects
Publications and source records attributed to H Fujii.
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Herein we report a case of hydrometrocolpos with left renal agenesis of a four-month-old girl. The hydrometrocolpos due to a transverse septum of the vagina and left renal agenesis were confirmed by laparotomy. The transverse septum was incised and cured successfully by the abdominoperineal approach. We have collected 21 cases of hydrometrocolpos with or without urinary tract malformations including our case in the Japanese literature and discussed its etiology and treatments.
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Two new glucose-6-phosphate dehydrogenase (G6PD) variants were discovered in Japan. The first, found in a 9-year-old male, was associated with chronic hemolysis and hemolytic crises after upper respiratory infections. The enzyme activity of the variant was 2.9% of normal. The patient's G6PD showed an increased utilization of substrate analogue, deamino-NADP, and thermal instability. The second variant occurred in a 7-year-old male with drug-induced hemolysis. The main enzymatic characteristics were reduced enzyme activity, being 6.4% of normal, faster-than-normal anodal electrophoretic mobility, slightly high Michaelis constant for glucose-6-phosphate, thermal instability, and biphasic pH optima. Enzymatic properties of these variants allowed each to be distinguished from previously reported variants. The first variant was designated Gd(-)Gifu and the other, Gd(-)Fukuoka.
A new glucose 6-phosphate dehydrogenase (G6PD) variant associated with chronic nonspherocytic hemolytic anemia was discovered in Japan. The patient showed hemolytic crises after upper respiratory infections. The enzyme activity was about 3.8% of the normal. The partially purified enzyme revealed slow anodal electrophoretic mobility, high Km NADP, marked thermal-instability, and increased affinity for a substrate analogue (deamino-NADP). A particular characteristic of this enzyme was a biphasic pH curve with a greatly increased activity at low pH values. From these results, this variant was clearly different from hitherto observed G6PD variants, and was designated G6PD Asahikawa.
A case of pseudohypoparathyroidism (PHP) associated with several hormonal abnormalities in addition to parathyroid hormone (PTH) was reported. Several endocrinological examinations before and after treatment with 1 alpha-OH-D3 revealed abnormal thyroid stimulating hormone and prolactin responses to thyrotropin releasing hormone, abnormal plasma renin activity responses to renin stimulation test, and abnormal calcitonin responses to calcium infusion test. These abnormalities were not corrected when serum calcium levels returned to normal after therapy. From these results, it was suggested that in PHP resistance to multiple hormones that worked by stimulating adenylate cyclase might be the cause of these hormonal abnormalities, irrespective of serum calcium levels.
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Venous and lymphatic invasion and Ag-reactive cells were studied in 110 gastric carcinomas. Venous invasion was recognized in 28.2% by H.E. staining only; it was seen in 58.2% by E.V.G. staining. Argyrophil and argentaffin cells were recognizable in 27.3% and 14.5%, respectively. No venous invasion was observed in many carcinomas with Ag-reactive cells. It has been suggested that the incidence of carcinoma with Ag-reactive cells is in inverse proportion to cellular atypism. Our work suggests that venous invasion bears a relation to atypism; this tendency was not recognizable with respect to lymphatic invasion.
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Clinical usefulness of the renoscintigraphy using 99mTc-DTPA in practical urology was investigated for two years. 99mTc-DTPA injection and injectable preparation kit (supplied by Nihon Medi-Physics Co.) were used. The dynamic blood flow of the renal parenchyma could be detected from serial images and curves on the perfusion phase. Information of clear nephrogram and function of the upper urinary tract could be obtained from serial images and renogram. Separate and total renal function could be evaluated. This method of examination is evaluated as very safe, because no side effects were produced and radiation dose is low. This examination may be a useful tool and the first choice in practical urology.
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A case of infected simple renal cyst and three cases of infected polycystic kidney have been treated during the 6 years from July, 1976 to June, 1982. One of the 3 cases of polycystic kidney, was operated. The other 2 cases were treated with antibiotics. The largest cyst in the present studies appeared to be more than 5 cm in diameter on the X-ray films. To define the limits to chemotherapy of the infected cyst, the relationship between the effect of chemotherapy and the size of the cyst was evaluated from clinical prognosis. From these results, it is strongly suggested that surgical treatment rather than chemotherapy is the best treatment for cysts which are more than 6 cm in diameter or in cases of marked protrusion of the kidney and significant deformation of renal calyces.