Tomofusa Usui

Treatment of seven cases of chronic granulomatous disease with sulfamethoxazole-trimethoprim (SMX-TMP)

Seven male Japanese children with chronic granulomatous disease were given sulfamethoxazole-trimethoprim (SMX-TMP) for recurrent pyogenic infections, most of which had proved difficult to control with other antibiotics. With continuous treatment the children remained free of infections severe enough to necessitate hospitalization, except on one occasion. Serious complications, including hematological disorders, never occurred during therapy and there were no changes in leukocyte function during therapy. These results indicate that SMX-TMP should be considered in the treatment of bacterial infections associated with chronic granulomatous disease.

Trace elements (copper, zinc, manganese, and selenium) in plasma and erythrocytes in relation to dietary intake during infancy.

All determinations of copper, zinc, manganese, and selenium were performed with a flameless atomic absorption spectrophotometer. Seventy-three full-term infants aged 1 to 52 weeks were divided into three age groups. Each age group contained two subgroups, breast-fed and formula-fed. No statistically significant differences between formula-fed and breast-fed subgroups were found in regard to the levels of copper and zinc in plasma and erythrocytes. At 1 to 5 weeks of age, the manganese concentration of erythrocytes was higher in formula-fed than in breast-fed infants (p < 0.001). This might be due to the high dietary intake of this element in the formula-fed subgroup. On the other hand, plasma selenium concentrations were significantly higher in breastfed than in formula-fed infants of all ages (p < 0.01 at 1 to 5 weeks and p < 0.05 at 6 to 52 weeks). This suggests that selenium compounds are biologically more available for infant nutrition in breast milk than in formula.

Plasma Selenium Concentration in Healthy Japanese Children and Adults Determined by Flameless Atomic Absorption Spectrophotometry

This study showed a rapid and direct method for determining selenium concentration in plasma by flameless atomic absorption spectrophotometry, and differences in plasma selenium concentration in healthy children and adults. A direct method is possible, since selenium is heat stable in the presence of nickel. With this method, the recovery of selenium added to plasma was 100.3 +/- 5.7%, and the relative standard deviation in repeated determinations of pooled plasma selenium was 3.0% and 6.8%. The plasma selenium concentration in adults was 99.4 +/- 12.5 ng/ml, lower than reported concentrations from the United States and Canada, and higher than those from New Zealand. These variations may reflect dietary habits, bioavailability of selenium compounds in diet, racial difference, or different analytical methods. The mean concentration of plasma selenium at 1 to 6 months of age (51.0 +/- 13.1 ng/ml) was significantly lower than in adults (p less than 0.001); it increased gradually and steadily to the adult level with age. This age-related difference of plasma selenium level is similar to that reported previously.

Decreased Superoxide Dismutase Activity of Erythrocytes and Leukocytes in Fanconi’s Anemia

Superoxide dismutase (SOD) activity was determined in the erythrocytes and leukocytes of 5 patients with Fanconis anemia (FA) and 1 with atypical Fanconis anemia without any hematological disorder. SOD activity was decreased in the blood cells of the patients with Fanconis anemia, but was normal in the atypical patient. The influence of SOD on the chromosome aberrations and hematological disorder in FA is discussed.

Zinc, copper, manganese, and selenium metabolism in patients with human growth hormone deficiency or acromegaly

Summary This study was designed to evaluate trace metal metabolism in patients with known abnormalities of human growth hormone (hGH). The mean concentration of zinc in plasma and urine decreased in patients with hGH deficiency after hGH injection, whereas, after adenomectomy, in patients with acromegaly, zinc increased in plasma, remained the same in erythrocytes, and decreased in urine. There was a negative correlation between plasma zinc and serum hGH levels and a positive correlation between urinary zinc excretion and serum hGH levels in acromegaly. In hGH deficiency, the copper content remained unchanged in plasma and erythrocytes and rose in urine after treatment; however, in acromegaly, the copper content increased in plasma and remained unchanged in erythrocytes and urine after surgery. The mean concentration of erythrocyte manganese did not change significantly after treatment in patients with hGH deficiency or acromegaly, but the pre hGH treatment level of erythrocyte manganese in hGH deficiency was lower than in the controls. Plasma selenium concentrations were decreased in hGH deficiency and increased in acromegaly patients after therapy. These results suggest that hGH affects the metabolism of zinc, copper, manganese, and selenium. hGH treatment level of erythrocyte manganese in hGH deficiency was lower than in the controls. Plasma selenium concentrations were decreased in hGH deficiency and increased in acromegaly patients after therapy. These results suggest that hGH affects the metabolism of zinc, copper, manganese, and selenium.

Isolated growth hormone deficiency type 1A ina Japanese family

A Japanese family is described in which a 7-year-old child had isolated growth hormone deficiency type 1A, as described by Illig et al. He was shown to be homozygous for a deletion of the structural gene for hGH (hGH-N gene). Initially his growth rate responded well to hGH administration, but rapidly he developed high titers of hGH antibodies, and growth ceased. At that time, a somatomedin-C generation test gave negative results, suggesting that the growth arrest was related to the inability of hGH to generate somatomedin. Both parents were heterozygous for the hGH-N gene deletion and had a low hGH response to arginine and L-dopa tolerance tests, but had normal basal somatomedin-C levels and normal somatomedin-C generation tests. This family is the fourth to be reported with IGHD type 1A caused by deletion of the hGH-N gene. This cause of growth hormone deficiency can be distinguished from other severe autosomal recessive types of hGH deficiency by the demonstration of the deletion of hGH-N gene using restriction endonuclease analysis.

Inhibitory effects of superoxide dismutases and various other proteins on the nitroblue tetrazolium reduction by phagocytizing guinea pig polymorphonuclear leukocytes.

Summary The reduction of nitroblue tetrazolium by guinea pig peritoneal polymorphonuclear leukocytes was studied during the phagocytosis of latex particles. Superoxide dismutases from various sources inhibited the reduction of the dye. However, almost the same degree of inhibition was observed with a number of other proteins including albumins, γ-globulin, histone and trypsin inhibitor, as well as with the heat inactivated dismutases. The results suggest a non-specific inhibitory effect of superoxide dismutase proteins on the reduction of nitroblue tetrazolium rather than a specific enzymic inhibition of the superoxide-mediated reduction of the dye.

A case of 21q-syndrome with half normal SOD-1 activity

SummaryA male Japanese infant was found to have a chromosomal aberration of del(21)(qter→q22.1-2) and decreased superoxide dismutase (SOD) activity in erythrocytes and polymorphonuclear and mononuclear leukocytes. The cuprozinc enzyme (SOD-1) level was 40–50% of normal, while the cyanide-insensitive managanese enzyme (SOD-2) activity was within the normal range. Determination of SOD activity in blood cells is a valuable method of classification of the syndrome.

Superoxide dismutase activity of T lymphocytes and non-T lymphocytes

Superoxide dismutase (SOD, EC 1.15.1. l), which catalyzes the dismutation of superoxide anion, O;-, to less toxic hydrogen peroxide and molecular oxygen, is an ubiquitous enzyme in oxygen-metabolizing cells and serves to protect them from the potentially harmful effects of this reactive free radical [ 11. Clinically, SOD activity has been extensively determined in peripheral blood cells in various conditions [2-71. It was reported that peripheral lymphocytes of human blood have higher SOD activity than do granulocytes [g-lo]. The purpose of this study is to extend this observation and to differentiate between the SOD activities of T and non-T lymphocytes.

Use of logit paper in determination of superoxide dismutase activity in human blood cells.

Superoxide dismutase activity of the human blood cells was determined by the capacity to inhibit nitrite formation from hydroxylammonium chloride. Relationship between percentage of nitrite formation and amount of purified human erythrocyte superoxide dismutase was plotted on conventional graph paper, double log and logit papers. A comparison of the results showed that a linear relationship was obtained only with the logit paper, and thus a more accurate and simple estimation of the activity can be made on a single determination of the specimens.