[Experience with valved conduits for the repair of complex congenital cardiac anomalies in children].
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Biomedical subjects
Publications and source records attributed to S Sano.
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The external auditory canal opens medial to lateral in embryonal development. It is obvious that a developmental arrest anywhere during this stage would produce an absence or discontinuity of the canal, that is, congenital atretic ear. Whether or not the obliterative tissue has a bony structure, cholesteatoma can be formed behind it. Microtia is usually associated with an atretic meatus, and it may involve the possibility of epithelial cyst or cholesteatoma. The diagnosis can usually be made by the finding of tender swelling in the deformed auricle or its surroundings. On occasions, it may become necessary to distinguish these conditions from secondary infection of an epidermal cyst on the auricular surface. Several recurrent painful swellings on the deformed auricle in spite of conservative treatment indicate the need for routine otologic radiographs.
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A 51-year-old man had been working in a dye factory for 25 years and had noticed itching and pigmentation on the extremities for the past 5 years. Patch testing showed positive reactions to Sudan I and Vacanceine Red, among the azo dyes which he had been handling. After he changed his work, he became free from erythematous lesions with itching. The pigmentation almost disappeared 10 months later.
6 young people (19-31 years old) exhibited unusual light or dark brown, ill-circumscribed or reticular pigmentation distributed over protrudent bones. 5 of them have been using nylon towels and scrub brushes for over 3-10 years. Biopsy specimens showed that the amount of melanin granules in the basal layer was increased and melanophages were scattered in the upper dermis. Inflammatory infiltrates were minimal.
Oral administration of a commercial PCB mixture to chickens caused a hepatic-type porphyria characterized by hepatic accumulation and urinary excretion of uroporphyrin. To clarify the mechanism of the porphyrinogenic activity of these PCBs, we studied the structural requirement of synthetic PCB for porphyrinogenic activities by using the cultured chick embryo liver cells and examined the relationship between induction of delta-aminolevulinic acid (ALA) synthetase and inhibition of uroporphyrinogen decarboxylase. We established that the porphyrinogenic effect of PCBs exhibits a sharply defined structure-activity relationship in that only 3,4,3',4'-tetrachlorobiphenyl and 3,4,5,3',4',5'-hexachlorobiphenyl produced a marked accumulation of uroporphyrin. We also demonstrated that in ALA-supplemented cultures, these same compounds lead to accumulation of a large amount of uroporphyrin III, whereas with other PCBs, which were weak inducers of porphyrin synthesis, the accumulated porphyrin was mostly protoporphyrin. was mostly protoporphyrin. Kinetic studies of the sequential decarboxylation of uroporphyrinogen with purified uroporphyrinogen decarboxylase were performed. The 3,4,3',4'-tetrachlorobiphenyl and 3,4,5,3',4',5'-hexachlorobiphenyl strongly inhibit uroporphyrinogen decarboxylase directly at two steps, i.e. first in the formation of hexacarboxylic porphyrinogen III from heptacarboxylic porphyrinogen III and second in the formation of heptacarboxylic porphyrinogen III from uroporphyrinogen III. The results confirmed that porphyrinogenic PCBs primarily inhibit uroporphyrinogen decarboxylase, leading to a depletion of heme as a result of which synthesis of ALA synthetase increased.
Lysyl oxidase was purified to about 3,000- to 5,000-fold from epiphyseal cartilages of chick embryos. Purification was accomplished by sequential column chromatographies of collagen-sepharose, DEAE-Cellulose and Sephacryl S-200. The molecular weight of the most purified enzyme from 4 different isomers as determined by gel-filtration technique using Sephacryl S-200 column was 32,000 and the purified enzyme contained one copper atom per mole. When the enzyme (copper content: 1.12 g-atom per mole) was incubated with 1 X 10(-4)M cadmium, followed by gel-filtration, 0.12 g-atom of cadmium was incorporated into the enzyme per mole and consequently 0.23 g-atom of prosthetic copper was released from the enzyme (loss of 21%) with a substantial decrease of the enzyme activity by 22.8%, but 50% loss of copper caused by treatment with 1 X 10(-3)M cadmium did not further decrease the activity. When the enzyme was incubated with 1 X 10(-4)M zinc, 0.38 g-atom of zinc bound to the enzyme and 65% copper was lost, resulting in 34% loss of the activity. Loss of 84% copper (experiment with 1 X 10(-3)M zinc), however, led to 47% inhibition. These findings indicate the existence of metal-metal interaction in the enzyme that can significantly influence the activity, though inhibition of the enzyme was not strictly proportional to either the amount of copper released or the amount of cadmium or zinc bound to the enzyme.
Measurement of autoradiographic grains produced by the decay of incorporated radioisotopes is often used for a quantitative assay of the rate of DNA replication and DNA repair in cells or tissues. However, visual grain counting by microscopic observation is time-consuming and tedious process. Recently, Kraemer et al. reported that automated measurement of grains in cultured human cells may be facilitated by using appropriate grain counting instruments. Under their experimental conditions using Kodak NTB-3 emulsion, instrument-determined grain number per nucleus was proportional to visual counts up to 30 grains, and then leveled off at much larger visual counts. The saturation phenomenon was due to counting-loss by the instrument caused by overlapping of neighboring grains. To prevent the counting-loss, we have used in the present study Japanese Sakura NR-M2 emulsion which is less sensitive to radiation exposure than Kodak NTB-3, thereby yielding smaller size of grains per radioactive decay. Samples were prepared from cultured skin fibroblasts derived from normal individuals and xeroderma pigmentosum (XP) patients defective in DNA repair. These cells were irradiated with 254 nm UV incubated for 3 h with culture medium containing 3H-thymidine, and autoradiograms were made by dipping in Sakura NR-M2 emulsion. The number of grains as well as grain surface area per nucleus was measured by using ARTEK CYTO TALLY MODEL 900 counting instrument, and compared with visual counts. The results showed that, under our optimum condition, the instrument-determined number of grains was directly proportional to visual counts, at least up to 150 grains per nucleus, with a correlation coefficient of 0.971.(ABSTRACT TRUNCATED AT 250 WORDS)
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In the present study we investigated some of the cellular mechanisms for the generation of macrophage-activating factor(s) (MAF) in immune responses to tumor antigens. C3H/HeN mice were immunized to syngeneic MH134 hepatoma or MCH-1-A1 fibrosarcoma by intradermal inoculation of viable tumor cells, followed by the surgical resection of the tumor. Spleen and lymph node cells from these tumor-immune mice were stimulated in vitro with the corresponding tumor cells, and supernatant from such a culture was tested for an ability to activate macrophages to exert their cytostatic and cytolytic activities as detected on tumor cells unrelated to immunizing tumors. Peritoneal adherent cells as a macrophage source, which were preincubated with supernatant from co-culture of tumor-unimmunized normal spleen and lymph node cells plus tumor cells, failed to exhibit any significant antitumor effect on unrelated X5563 tumor cells, whereas the addition of supernatant from cultures containing immune lymphocytes to adherent cells resulted in appreciably potent cytostatic and cytolytic effects on X5563 tumor cells, indicating the generation of MAF in culture supernatant. The activation of tumor-immune spleen and lymph node cells for MAF generation was tumor-specific, because anti-MH134- and anti-MCH-1-A1-immune lymphocytes produced MAF by the stimulation with the respective but not with the other alternative tumor cells. Such MAF production was abolished by treatment of tumor-immune spleen and lymph node cells with anti-Thy-1.2 or anti-Lyt-1.1 but not with anti-Lyt-2.1 antibody plus complement before culturing. These results indicate that the tumor-specific Lyt-1+2- T cell subset has a crucial role in generating MAF by which an adherent cell population as a source of macrophages acquires the potential for inducing a cytolytic as well as a cytostatic effect on tumor cells.
Clinical and histological findings in 37 cases of cutaneous lymphomas other than mycosis fungoides and Sezary syndrome were investigated; there were 31 in adults and 6 in children. Cutaneous lesions were the first manifestations of the diseases in all cases, and they appeared mostly as tumors or nodules. Cytomorphologically, about a half of the cases showed proliferations of large cleaved, non-cleaved cells or immunoblasts (Group I). Eight cases showed a polymorphous appearance containing convoluted cells of various size (Group II). Five cases in children demonstrated monomorphous proliferation of uniform-sized lymphoblasts (Group III). The cytologic findings in 6 cases did not fit into any lymphoid groups (Group IV). The clinical findings observed in each group were reviewed and compared. Follow-up study revealed that the prognosis of Group I was the poorest among the four groups.
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A 5-year-old boy had a nodular erythema, elongated and thickened fingers, and emaciation. His condition was a rare congenital disease inherited as an autosomal recessive trait. Eleven cases have been previously reported in the Japanese literature. The onset is early in childhood, and nodular erythema is an essential and initial finding. Growth retardation and emaciation progress slowly with age. The characteristic clinical features include large eyes, nose, lips, and ears, disproportionately long and thick fingers, and the loss of adipose tissue from the upper half of the body. Cardiomegaly and hypertrophy of the periosteum of the phalanges have been described in some cases.
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Interaction between new N atom-containing tumor inhibitors (1-9) and nucleic acids has been investigated utilizing the spectroscopic methods efficiently. On the basis of their data, it is well suggested that FNT-4 (4) and benzo[c]phenanthridine alkaloids (6-8) bind to nucleic acids by a fashion involving intercalation of aromatic molecule(s).