[Emergency operation of neonates and infants with congenital heart diseases].
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Biomedical subjects
Publications and source records attributed to K Ueda.
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A male Japanese infant was found to have a chromosomal aberration of del(21)(qter leads to 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 manganese 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.
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Nuclear matrix prepared from bovine lymphocytes contained a significant amount of actin. Both nuclear matrix actin and rabbit muscle actin showed the same electrophoretic mobility on SDS-gel. The matrix-associated actin could be separated into three isoproteins which may correspond to alpha-, beta- and gamma-actin. The most acidic spot of these isoproteins co-migrated with rabbit muscle actin (alpha-actin) on two-dimensional electrophoresis. The amino-acid composition of the nuclear matrix actin was closely related to that of rabbit muscle and to that of porcine brain actin. Moreover, the actin filaments, treated with 0.75 M guanidine hydrochloride, changed from the polymerized form of the nuclear matrix actin into a monomeric form (G-actin), which had strong inhibitor activity against pancreatic DNase I. From this inhibition, the actin content of the nuclear matrix was estimated to be about 12% of total matrix protein. When the nuclear matrix was digested with trypsin, the bulk of matrix protein was hydrolyzed, but about 80% of the actin remained associated with sphere structures (trypsin-treated nuclear matrix) precipitable by low speed centrifugation. SDS-gel analysis revealed that actin was one of the major components of the trypsin-treated nuclear matrix, which had a similar size and structure as the untreated nuclear matrix. The fibrogranular structure and residual nucleoli of the original nuclear matrix were well preserved against trypsin digestion; however, the peripheral lamina was removed. These results indicate that the matrix-associated actin is localized predominantly in the matrix interior, where it presumably interacts closely with the fibrogranular structure and/or the residual nucleoli.
A case of melanotic neuroectodermal tumour of infancy (MNTI) with rapid recurrence is described, together with a survey of 16 reported recurrent cases. The recurrent lesion was successfully treated by wide excision of the maxilla. The recurrent tumour had two lesions of different natures, one consisting of a lobular mass indistinguishable from the primary tumour but located remote from the original site and the other showing diffuse infiltration of tumour cells deep in the medulla of the maxilla. A survey of the recurrent cases indicated that, although incomplete removal of the primary tumour, dissemination of tumour cells during surgery and a multicentric origin could be the causes of a recurrence, the presence of a silent lesion which may express an aggressive growth on stimulation may also explain the complex behaviour of the tumour.
A new method (Three-Dimensional (3-D) Analysis of Mandibular Movement) was developed to evaluate the movement of the mandible during surgery and in postoperative years, in three dimensions. The analysis was done with the aid of preoperative cephalograms and preoperative, predicted and postoperative dental models. The method consisted of measuring the amount and direction of the movements of defined points on the dental models and displaying the movement of other bony landmarks in the cephalometric tracings by coordinate transformation. All the calculations for the coordinate transformation were performed with the aid of a micro-computer. The results of an analysis with the method in two cases indicated that it is quite useful in determining the operative procedure as well as in evaluating the postoperative stability of the mandible, because in addition to the vertical and antero-posterior movement, it enables us to see the lateral movement of the mandible.
To elucidate the molecular mechanism by which poly(ADP-ribose) participates in DNA excision repair, we examined the effect of poly(ADP-ribose) on DNA ligase activity in DNA/histone and reconstituted chromatin systems. The ligase activity was markedly inhibited by histones; the inhibition varied depending on histone subfraction and DNA/histone ratio. Poly(ADP-ribose), either exogenous or synthesized in situ by poly(ADP-ribose) synthetase, reversed this inhibition by histone almost completely. This effect was specific for poly(ADP-ribose); polyanions such as mRNA, rRNAs, tRNA, and synthetic poly(A) were less effective or ineffective. The ligase activity with reconstituted chromatin as the substrate was about half of that with free DNA whereas the activities with these two substrates were almost the same in the presence of poly(ADP-ribose) synthesized in situ. The polymers synthesized under these conditions were exclusively bound to the synthetase. Together with our previous finding that the enzyme is the main acceptor of the polymer in DNA-damaged cells, these results suggest that poly(ADP-ribose) in the synthetase-bound form counteracts inhibition by histones and activates DNA ligase to rejoin DNA strands in polynucleosomal structures.
The epidemiology of rubella in most areas of Asia has not been defined. In Fukuoka city in southern Japan, rubella epidemics occurred in 1956-58, in 1966 and in 1976-77. During the first halves of the 1960s and 1970s, neither epidemic nor endemic clinical rubella occurred. Rubella mass vaccination for girls of 14 years of age started in 1978. Rubella seroepidemiologic studies were performed in 1965, 1974, 1978-79 and 1981 in Fukuoka city. Current studies indicated that children of 6-10 years of age during the epidemics of 1956-58, 1966 and 1976-77 were infected with rubella in 72%, 37% and 57% of cases, respectively. Just before the epidemics of 1966 and 1976-77 100% of the children under nine years of age had no antibodies to rubella indicating absence of recent outbreaks. Whereas 95% of the population (young women of 15-18 years of age in 1981) which participated in the rubella mass vaccination programme since 1978 have rubella Hl antibodies, 46% of the nonparticipating population (19-23 years of age in 1981) remains susceptible to rubella. These facts indicate that rubella epidemics occurred in 1956-58, 1966 and 1976-77, that the magnitude of each epidemic was different, and that there were intervals without endemicity in the inter-epidemic periods. However, after the nation wide rubella epidemic in 1975-77, endemic rubella continued, and rubella epidemics again occurred in 1980-82. The unique rubella epidemic pattern in Japan may be changing in the 1980s to that which existed in most areas of the world where rubella was endemic with periodic increased incidence of epidemicity.(ABSTRACT TRUNCATED AT 250 WORDS)
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A case of localized Darier's disease was reported. The patient was a 36-year-old Japanese woman, and hyperkeratotic papules in zosteriforme arrangement were persistently noted on the left forehead since the age of 20 years. Histological appearance was same as in classical Darier's disease. Skin atrophy due to long-term application of corticosteroid ointment was observed. Aromatic retinoid ointment was effective in the treatment of the lesions. The literature on localized Darier's disease is reviewed.
The inter- and intracellular localization of poly(adenosine diphosphate-ribose)(poly(ADP-ribose] synthetase was investigated using an indirect immunofluorescence technique and a specific antibody against the enzyme purified from calf thymus. In various bovine tissues, including liver, heart, pancrease, thyroid, spleen, adrenal, and skeletal muscle, the specific immunofluorescence of poly(ADP-ribose) synthetase was localized exclusively in the nucleus. Immunostaining was inhibited by preabsorption of the antibody with purified calf thymus poly(ADP-ribose) synthetase. Nuclear immunofluorescence appeared to be more prominent in the marginal area than in the central region in most nuclei. This staining pattern is similar to that of naturally occurring poly(ADP-ribose). In bovine peripheral blood the immunofluorescence of poly(ADP-ribose) synthetase was detected in nuclei of lymphocytes, but not in granulocytes, in agreement with the finding that the enzymatic activity of poly(ADP-ribose) synthetase was barely detectable in nuclei isolated from granulocytes.