[Short-term results of the Fontan operation in patients with Down syndrome].

Despite an extensive experience with Fontan operation, there is a paucity of information to guide the indication of the procedure in patients with Down syndrome. Of 79 patients who had undergone a Fontan operation in our hospital between 1995 and 2003, 3 had Down syndrome. All 3 patients had complete atrioventricular septal defect with single ventricular physiology. Two patients survived, and 1 died of chylothorax and respiratory infection. The 2 survivors have done well in the short-term without complications. We consider that in appropriately selected patients with Down syndrome in whom biventricular repair is precluded, the Fontan operation is the choice of the procedures.

Electron microscopic studies of the early cellular events occurring in oral mucosa of rats affected by graft-versus-host disease (GVHD).

Previous histologic studies of tongue lesions in graft-versus-host disease (GVHD) in non-irradiated (Brown Norway x Lewis) F1 rats given parental spleen cells have demonstrated an increase in the number of MHC class II+ cells with dendritic shape in the lamina propria to be the earliest event in the development of the lesion. We studied this histologic finding by electron microscopy to increase understanding of the early cellular events occurring in the lesion. Electron microscopically, the most prominent cell type observed in the lamina propria was the cell with a dendritic shape. These dendritic cells possessed large nuclei that often showed irregular indentations and ample cytoplasm containing numerous filaments and mitochondria. A few lysosomal or phagocytic structures were also seen in the cytoplasm. No Birbeck granules were identified. These findings were very similar to those of indeterminate cells in the epidermis and dermis. Our data strongly support the hypothesis that MHC class II+ cells with dendritic shape present antigen during the induction of local immunological responses in the tongue of GVHD rats.

[The roles of various media in the decision making processes for recycling behavior: a path analysis model].

This study researched the effects of cognitive variables on recycling behavior, as well as effects of various media of influence on the cognition and behavior. According to Hirose (1994), the decision making process for recycling consists of two steps. The first leads to goal intention of an ecological lifestyle. The second is related to behavior intention of recycling in line with the goal intention. Mass media, such as newspaper and TV, are thought to influence beliefs about environmental problems, including three determinants of goal intention: perception of seriousness, responsibility, and effectiveness. Personal media, such as personal contacts with pro-environmental activists, are thought to influence evaluation of behavior, including three determinants of behavior intention: evaluation of feasibility, cost and benefits, and social norms. Local media, such as municipal announcement and circular, are hypothesized to have a mixed effect of the two. Path analysis indicated that goal intention affected recycling behavior through behavior intention. Effects of the three media of influence on the cognitive variables were also consistent with the hypothesis.

Oral mucosal lesions in experimental graft-versus-host disease: morphological and immunohistochemical characterization of infiltrating cells.

To facilitate recognition of the oral mucosal lesion that develops in rats with graft-versus-host disease (GVHD) induced by injecting spleen cells of parental strain rats (Brown Norway) into non-irradiated (Brown Norway x Lewis) F1 hybrid rats, we followed the development of the tongue lesion histologically and immunohistochemically. This assessment revealed an increase in the number of MHC class II+ cells with dendritic shape in the lamina propria to be the earliest stage of the tongue lesions in GVHD rats. The subsequent mononuclear cell infiltration with epithelial cell destruction, characteristic of GVHD, consisted of CD8+ cells and macrophages. Our findings seem to indicate that MHC class II+ cells with dendritic shape may provide antigen presentation in the induction of local immunological responses, including tissue destruction, by CD8+ cells and macrophages in the tongue of GVHD rats.

Gene amplification as a cause of inherited thyroxine-binding globulin excess in two Japanese families.

T4-binding globulin (TBG) is the major thyroid hormone transport protein in man. Inherited abnormalities in the level of serum TBG have been classified as partial deficiency, complete deficiency, and excess. Sequencing analysis of the TBG gene, located on Xq21-22, has uncovered the molecular defects causing partial and complete deficiency. However, the mechanism leading to inherited TBG excess remains unknown. In this study, two Japanese families, F-A and F-T, with inherited TBG excess were analyzed. Serum TBG levels in hemizygous males were 58 and 44 micrograms/mL, 3- and 2-fold the normal value, respectively. The molecule had normal properties in terms of heat stability and isoelectric focussing pattern. The sequence of the coding region and the promoter activity of the TBG gene were also indistinguishable between hemizygotes and normal subjects. The gene dosage of TBG relative to that of beta-globin, which is located on chromosome 11, and Duchenne muscular dystrophy, which is located on Xp, was evaluated by coamplification of these target genes using polymerase chain reaction and subsequent quantitation by HPLC. The TBG/beta-globin ratios of the affected male and female of F-A were 3.13 and 4.13 times, respectively, that in the normal males. The TBG/Duchenne muscular dystrophy ratios were 2.92 and 2.09 times the normal value, respectively. These results are compatible with three copies of TBG gene on the affected X-chromosome. Similarly, a 2-fold increase in gene dosage was demonstrated in the affected hemizygote of F-T. A 3-fold tandem amplification of the TBG gene was shown by in situ hybridization of prometaphase and interphase chromosomes from the affected male with a biotinylated genomic TBG probe, confirming the gene dosage results. Gene amplification of TBG is the cause of inherited TBG excess in these two families.

Cloning, sequence analysis, and expression of ligninolytic phenoloxidase genes of the white-rot basidiomycete Coriolus hirsutus.

Two cDNAs and two genomic DNAs coding for the allelic forms of the ligninolytic phenoloxidase were isolated from the white-rot fungus Coriolus hirsutus. The cloned genes were identified in genetic libraries by hybridization screening using four deoxyoligonucleotide probes which corresponded to the partial amino acid sequence of the purified enzyme. Each cDNA encoded the full-length of the phenoloxidase, a protein consisting of 499 amino acid residues, and its putative signal peptide of 21 amino acid residues. The nucleotide sequences of the two alleles differed by 18 single base changes within the open reading frames resulting in one amino acid substitution. Ten small introns interrupted both genomic DNAs as indicated by direct comparison with the corresponding cDNAs. Putative eukaryotic regulatory sequences, "CAAT" and "TATA," were observed in the 5'-flanking region of both genomic DNAs. Each of the phenoloxidase cDNAs was successfully expressed in an active form in Saccharomyces cerevisiae using the useful yeast expression vector YEp51.

Marked latency change of auditory brainstem response in preterm infants in the early postnatal period.

In order to evaluate the influence of extrauterine life on auditory brainstem response in neonates, we performed serial ABR recordings twice a week in 10 preterm infants. A marked decrease of I-III interpeak latency was observed in 7 infants during the first 2 or 3 weeks after birth. Wave I latency also remarkably reduced during this period in 5. These findings were related not to the conceptional age but to the extrauterine age. In 2 infants III-V interpeak latency increased during the first 1 or 2 weeks after birth, and the rate of latency increase seemed to be inversely proportional to that of decrease of I-III interpeak latency.