Noninvasive visualization and measurement of posterior descending coronary artery flow velocity by transthoracic Doppler echocardiography in normal children.
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Adolescent↗
Biomedical subjects
Goro Takada
Publications and source records attributed to Goro Takada.
The efficacy of using pulmonary vein wedge pressure for the estimation of pulmonary artery pressure in children with congenital heart disease.
The objectives of this study were to assess the accuracy of pulmonary vein wedge pressure (PVWP) in estimating pulmonary artery pressure (PAP) in various types of congenital heart disease, including single-ventricle physiology. The systolic, diastolic, and mean values of both PAPs and PVWPs were measured in 30 patients (a total of 46 points). Pulmonary artery pressure ranged from 13 to 74 (34 +/- 15) mm Hg in systole, 5 to 25 (13 +/- 6) mm Hg in diastole, and 6 to 48 (18 +/- 10) mm Hg in mean. As a whole, good correlations between PAPs and PVWPs were observed (systole, r = 0.70; diastole, r = 0.85; mean, r = 0.82; P < 0.0001). However, with an increase in PAP, the discrepancy between PAPs and PVWPs increased. When the mean PVWP was more than 18 mm Hg, the mean PVWP in 14 of 24 (58%) underestimated the mean PAP by up to 22 mm Hg (mean difference, -1.7 +/- 5.8 mm Hg). On the other hand, all of the patients with mean PVWPs less than 18 mm Hg (n = 22) had mean PAPs less than 18 mm Hg (r = 0.86; PAP = 1.11 x PVWP - 1.41; P < 0.0001), and the mean difference was -0.2 +/- 1.8 mm Hg. The mean PVWP can accurately estimate the mean PAP in children with congenital heart disease who have a mean PVWP less than 18 mm Hg.
Cardiac Catheterization↗
A novel bioconversion of L-fructose to L-glucose by Klebsiella pneumoniae.
L-Fructose, which was produced from L-psicose using immobilized D-tagatose 3-epimerase, was utilized as a starting material in the preparation of an uncommon aldose-hexose, L-glucose, by cell reaction. A mutant strain, Klebsiella pneumoniae strain 40bXX, produced D-arabinose isomerase constitutively. Toluene-treated cells of the mutant strain, which were used as the source of crude D-arabinose isomerase, were employed in the conversion of L-fructose to L-glucose. Empirically, 0.35 g of L-glucose was obtained from 1.0 g of L-fructose, viz an overall yield of 35%. The product obtained was purified and identified to be L-glucose by high performance liquid chromatography (HPLC) analysis, and was ultimately confirmed by 13C nuclear magnetic resonance (13C NMR) spectra.
Journal Article↗
Choroid plexus carcinoma presented with spinal dysfunction caused by a drop metastasis: a case report.
A 2-year-old girl demonstrating gait disturbance and dysuria was evaluated and showed two large remote tumors at the left lateral ventricle and lower spinal canal. Pathological analysis demonstrated both of the tumors to be choroid plexus carcinoma (CPC) with high MIB-1 labeling index. The enhanced mitotic propensity would have contributed to an early stage of drop metastasis from the primary site to the sacral sac and following accelerated formation of a longitudinal tumor, which had grown in the subarachnoid space conforming to the spinal canal and finally caused the presenting symptoms of spinal dysfunction. This report shows that CPC can develop exophytically in the subarachnoid space as well as in the ventricle simultaneously before appearance of clinical symptoms and confirms the importance of extensive neuroimaging in its evaluation.
Brain Neoplasms↗
Normal values for left anterior descending coronary artery flow velocity assessed by transthoracic doppler echocardiography in healthy children.
Normal values for left anterior descending coronary artery (LAD) flow velocity were assessed from a large number of normal children. In 303 healthy children, LAD peak flow velocity was measured by Doppler echocardiography. LAD peak flow velocities were calculated considering the angle between the Doppler beam and the coronary flow direction. The flow signals of LAD were recorded in 95% (288/303). The mean angle between the Doppler beam and Doppler flow signals of LAD was 42 +/- 8 degrees. The ratio of AT to total diastolic spectral duration was 0.19 +/- 0.088 and constant with age. LAD peak flow velocity correlated significantly with age (r = -0.57, p < 0.0001) and heart rate (r = 0.63, p < 0.0001). Multiple linear regression analysis showed that LAD peak flow velocity was associated with age and heart rate (LAD peak flow velocity = 20-0.34 (age) + 0.16 (heart rate), r2 = 0.41, p < 0 .0001). Normal data obtained in the present study provide a basis of the understanding and investigation in children with congenital heart disease or acquired heart disease such as atherosclerosis, left ventricular hypertrophy, or Kawasaki's disease.
Adolescent↗
Direct production of D-arabinose from D-xylose by a coupling reaction using D-xylose isomerase, D-tagatose 3-epimerase and D-arabinose isomerase.
Klebsiella pneumoniae 40bXX, a mutant strain that constitutively produces D-arabinose isomerase (D-AI), was isolated through a series of repeated subcultures from the parent strain on a mineral salt medium supplemented with L-Xylose as the sole carbon source. D-AI could be efficiently immobilized on chitopearl beads. The optimum temperature for the activity of the immobilized enzyme was 40 degrees C and the enzyme was stable up to 50 degrees C. The D-Al was active at pH 10.0 and was stable in the range of pH 6.0-11.0. The enzyme required manganese ions for maximum activity. Three immobilized enzymes, D-xylose isomerase (D-XI), D-tagatose 3-epimerase (D-TE and D-AI were used for the preparation of D-arabinose from D-xylose in a coupling reaction. After completion of the reaction, degradation of D-xylulose was carried out by Saccharomyces cerevisiae. The reaction mixture containing D-Xylose, D-ribulose and the product was then separated by ion exchange column chromatography. After crystallization, the product was checked by HPLC, IR spectroscopy, NMR spectroscopy and optical rotation measurements. Finally, 2.0 g of D-arabinose could be obtained from 5 g of the substrate.
Journal Article↗
Overexpression of Aspergillus aculeatus cellobiohydrolase I in Aspergillus oryzae.
To express the cbhI gene, encoding Aspergillus aculeatus cellobiohydrolase I (CBHI), in Aspergillus oryzae, a plasmid was constructed. The strain that displayed the strongest CBHI activity among the transformants produced about 941 mg/l in liquid culture. It was confirmed by a PCR method that the plasmid was integrated at the niaD locus.
Journal Article↗
Signaling pathway for radiation-induced apoptosis in the lymphoblasts from neuronopathic (type A) and non-neuronopathic (type B) forms of Niemann-Pick disease.
Deficient activity of human lysosomal hydrolase, acid sphingomyelinase (ASM), results in the neuronopathic (type A) and non-neuronopathic (type B) forms of Niemann-Pick disease (NPD). A deficiency of ASM is known to deprive lymphoblasts of their response to apoptotic induction by X-ray irradiation. To elucidate the genetic heterogeneity of apoptotic induction in NPD cells, we investigated radiation-induced apoptosis of lymphoblasts in patients with type A (genotype: IVS3-2A-G/IVS3-2A-G) and type B (genotype: S436R/S436R) NPD. Epstein-Barr virus (EBV)-transformed lymphoblasts established from a patient with type A NPD, a patient with type B NPD and a normal control were irradiated with 20 Gy and incubated for 24 h. The cells were harvested and the morphological features of apoptosis were observed with DNA-specific fluorochrome bis-benzimide. Exposure of lymphoblasts to 20 Gy of radiation resulted in 25-30% apoptosis of total cells in normal lymphoblasts, 8-9% apoptosis in type A NPD and 20-27% apoptosis in type B NPD. The radiation-induced apoptotic response in the lymphoblasts of type A NPD was significantly different from that of the normal lymphoblasts (P<0.0005). On the other hand, the radiation-induced apoptotic response in type B NPD was not markedly different from that in normal lymphoblasts (P=0.624). In the patient with type B NPD, the signaling pathway for radiation-induced apoptosis was preserved in lymphoblasts, which suggests that the extent of cell signaling system disturbance due to ASM deficiency may be related to the phenotypes in types A and B NPD.
Amino Acid Substitution↗
Juvenile Alexander disease with a novel mutation in glial fibrillary acidic protein gene.
Early-onset (infantile) Alexander disease is associated with mutations in the glial fibrillary acidic protein (GFAP) gene and two hot spots correlate to the severe phenotype. No molecular mechanisms have been elucidated in late-onset (juvenile) Alexander disease. The authors report a novel GFAP mutation in a patient with juvenile Alexander disease. The authors discuss similar molecular mechanisms in another intermediate filament disease and propose a possible molecular pathogenesis in juvenile Alexander disease.
Adolescent↗
Transthoracic Doppler echocardiographic measurement of flow velocity and flow velocity reserve in the great cardiac vein and in the left anterior descending coronary artery in children with left ventricular volume overload secondary to ventricular septal defect.
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Adenosine Triphosphate↗
Hollow fibers for delivery of harmonic pulses of Q-switched Nd:YAG lasers.
Flexible hollow fibers for delivery of the second, third, and fourth harmonic pulses of Q-switched Nd:YAG lasers are introduced. For the doubled (532-nm) wavelength, we fabricated a hollow fiber with an internal metal and polymer film by using a silver-mirror plating and a liquid-phase-coating method. For tripled (355-nm) and quadrupled (266-nm) Nd:YAG in the ultraviolet region, we fabricated aluminum hollow fibers with or without an internal polymer layer by using the metal-organic chemical-vapor deposition method. Both types of fiber show high stability for the transmission of high-peak power laser pulses with low transmission losses.
Journal Article↗
Five novel SLC7A7 variants and y+L gene-expression pattern in cultured lymphoblasts from Japanese patients with lysinuric protein intolerance.
Two distinct human light subunits of the heteromeric amino acid transporter, y+LAT-1 coded by SLC7A7 and y+LAT-2 coded by SLC7A6, are both known to induce transport system y+L activity. SLC7A7 has already been identified as the gene responsible for lysinuric protein intolerance (LPI). We successfully identified five novel SLC7A7 variants (S238F, S489P, 1630delC, 1673delG, and IVS3-IVS5del9.7kb) in Japanese patients with LPI by PCR amplification and direct DNA sequencing. In addition, we performed a semi-quantitative expression analysis of SLC7A7 and SLC7A6 in human tissue. In normal tissue, the gene-expression ratio of SLC7A6 to SLC7A7 was high in the brain, muscle, and cultured skin fibroblasts; low in the kidneys and small intestine; and at an intermediate level in peripheral blood leukocytes, the lungs, and cultured lymphoblasts. The gene-expression ratio of SLC7A6 to SLC7A7 in cultured lymphoblasts was significantly different between normal subjects and LPI patients with R410X and/or S238F, where the relative amount of SLC7A7 mRNA was significantly lower and the relative amount of SLC7A6 mRNA was statistically higher in affected lymphoblasts than in normal cells. Expression of SLC7A7 and SLC7A6 may thus be interrelated in cultured lymphoblasts.
Adolescent↗
Cloning and transcription analysis of the Aspergillus aculeatus No. F-50 endoglucanase 2 (cmc2) gene.
The cmc2 gene, coding for an endoglucanase 2 (CMC2) of Aspergillus aculeatus, was cloned using both genomic and cDNA libraries, and sequenced. The gene consists of 1230 bp encoding a protein of 410 amino acid residues with a molecular mass of 43,697 Da. The CMC2, composed of an N-terminal catalytic domain belonging to the family 5 of glycosyl hydrolases and a C-terminal cellulose-binding domain (CBD) belonging to the family I of CBDs, showed identity with other fungal endoglucanases, particularly with that of A. niger, A. nidulans, A. kawachii and A. aculeatus. The transcription of the cmc2 gene in A. aculeatus cells that were grown on different carbon sources was measured. Analysis by the ribonuclease protection assay revealed that expression of the cmc2 gene is induced by cellulose and some disaccharides and repressed by glucose.
Journal Article↗
Characterization of the 105-kDa molecular chaperone. Identification, biochemical properties, and localization.
We have characterized the biochemical properties of the testis and brain-specific 105-kDa protein which is cross-reacted with an anti-bovine HSP90 antibody. The protein was induced in germ cells by heat stress, resulting in a protein which is one of the heat shock proteins [Kumagai, J., Fukuda, J., Kodama, H., Murata, M., Kawamura, K., Itoh, H. & Tanaka, T. (2000) Eur. J. Biochem.267, 3073-3078]. In the present study, we characterized the biochemical properties of the protein. The 105-kDa protein inhibited the aggregation of citrate synthase as a molecular chaperone in vitro. ATP/MgCl2 has a slight influence of the suppression of the citrate synthase aggregation by the 105-kDa protein. The protein possessed chaperone activity. The protein was able to bind to ATP-Sepharose like the other molecular chaperone HSP70. A partial amino-acid sequence (24 amino-acid residues) of the protein was determined and coincided with those of the mouse testis- and brain-specific APG-1 and osmotic stress protein 94 (OSP94). The 105-kDa protein was detected only in the medulla of the rat kidney sections similar to OSP94 upon immunoblotting. The purified 105-kDa protein was cross-reacted with an antibody against APG-1. These results suggested that APG-1 and OSP94 are both identical to the 105-kDa protein. There were highly homologous regions between the 105-kDa protein/APG-1/OSP94 and HSP90. The region of HSP90 was also an immunoreactive site. An anti-bovine HSP90 antibody may cross-react with the 105-kDa protein similar to HSP90 in the rat testis and brain. We have investigated the localization and developmental induction of the protein in the rat brain. In the immunohistochemical analysis, the protein was mainly detected in the cytoplasm of the nerve and glial cells of the rat brain. Although the 105-kDa protein was localized in all rat brain segments, the expression pattern was fast in the cerebral cortex and hippocampus and slow in the cerebellum.
Adenosine Triphosphate↗
Case of congenital infection with Candida glabrata in one infant in a set of twins.
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Candidiasis↗
Lidocaine-dependent early infantile status epilepticus with highly suppressed EEG.
We report an early infantile patient characterized by intractable hyponatremia, progressive megalencephaly, and epileptic seizures with an EEG pattern that alternated between interictal low-voltage background and ictal burst activity. Repeatedly all the abnormal findings improved in a lidocaine-dependent manner. Given the pharmacologic mechanisms of lidocaine as a sodium channel blocker, we speculate that our patient had a sodium channel dysfunction.
Electroencephalography↗
Left anterior descending coronary artery flow and its relation to age in children.
Recent advances in Doppler and color echocardiographic techniques enable coronary flow dynamics to be estimated even in children. To assess quantitatively left anterior descending coronary artery (LAD) volumetric flow and to determine its relation to age and left ventricular (LV) mass, healthy children participated in a study that used high-frequency transthoracic echocardiography. We also studied whether Doppler echocardiography can reliably measure LAD flow in a clinical setting. In 57 healthy children, 2-dimensional echocardiography was used to measure the diameter and cross-sectional area of the LAD and LV mass. LAD peak flow velocity, flow velocity integral, and flow volume were measured by Doppler echocardiography. We then calculated the ratio of LAD cross-sectional area to LV mass and the ratio of LAD flow volume to LV mass. In 12 patients with Kawasaki disease, LAD flow velocity and flow velocity integral were measured by Doppler echocardiography at the time of Doppler guide wire examination. There were significant correlations between echocardiographic and Doppler guide wire methods for flow velocity and flow velocity integral (r = 0.77 and 0.83, P <.01, respectively). The LAD flow velocity decreased significantly with age (r = -0.43, P <.01). The LAD flow volume per minute increased significantly with age (r = 0.55, P <.01). However, LAD flow volume/LV mass ratio in younger infants was high and decreased significantly with age (r = -0.66, P <.01). This study shows that LAD flow patterns can be reliably assessed by transthoracic Doppler echocardiography in the majority of pediatric subjects. In the current study, the LAD flow velocity and the ratio of LAD flow volume to LV mass in infants was high and decreased with age, suggesting high myocardial perfusion. High LAD peak velocity in infants may be related with high resting coronary flow. Age-related changes in the LAD flow characteristics must be taken into consideration in the study of the coronary circulation in children.
Adolescent↗
Blood flow in the left anterior descending coronary artery in children with ventricular septal defect.
High-frequency echocardiography offers a noninvasive approach for imaging left anterior descending coronary artery (LAD) blood flow from a transthoracic window. The purpose of this study was to assess the effects of left ventricular (LV) volume overload on LAD flow in pediatric patients with ventricular septal defect (VSD). The study subjects consisted of 38 children with VSD and 15 healthy children. LV mass, LAD diameter, and LAD flow were measured by using transthoracic echocardiography, then LAD diameter and LV mass were indexed for body surface area. Pulmonary to systemic flow ratios (Qp/Qs) were obtained by cardiac catheterization. The Qp/Qs ratios ranged from 1.2 to 3.1 (mean 2.1 +/- 0.5). The mean LAD flow velocities, flow velocity integrals, and flow volumes were significantly higher in the patients than in the control subjects. LAD flow velocity and flow volume showed significant positive correlations with Qp/Qs, LV mass, and LV end-diastolic volume. Stepwise regression analysis revealed that Qp/Qs was the most important determinant of both LAD flow velocity (r(2) = 0.45, P < .0001) and LAD flow volume (r(2) = 0.44, P < .0001). The ratios of LAD flow volume to LV mass did not differ between the 2 groups. In 8 patients who underwent surgical treatment, LAD flow velocity, flow velocity integral, and flow volume decreased significantly after surgery. The current results suggest that patients with VSD have a higher resting coronary blood flow, and that LAD flow pattern is dependent on LV volume overload and changes after surgery.
Blood Flow Velocity↗