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Background/Aim: Several cases of concurrent reduction of expression of polycystin 1 (PKD1) and Tuberous Sclerosis Complex 2 (TSC2) that are contiguous in chromosome 16p13 have been previously reported. This study newly addresses the concurrent reduction of expression of PKD1, TSC2 and NTHL1, which is adjacent to TSC2 and is a tumor suppressor gene. Materials and Methods: We investigated the mRNA expression levels of PKD1, TSC2, PKD2, TSC1 and NTHL1 in blood and renal cell carcinoma (RCC) tissues in a proband with autosomal dominant polycystic kidney disease (ADPKD), tuberous sclerosis complex (TSC) and multiple pathologically diverse RCCs, including clear cell, papillary and chromophobe types. Additionally, we investigated germline variants in blood using whole exome sequencing (WES) in the proband and her four siblings. Results: mRNA expression levels of PKD1, TSC2 and NTHL1 were reduced in the proband’s blood and RCCs, compared with control groups. WES identified one novel variant with amino acid changes in the PKD1 exon in the three subjects with ADPKD, including the proband. Moreover, two variants in the TSC2 intron specific to the proband were also identified. Conclusion: In this study, we report a novel pathogenic variant in the PKD1 exon which likely led to ADPKD, and two variants in the TSC2 intron, which might have led to reduction in the expression of both TSC2 and NTHL1, consequently leading to TSC and multiple pathologically diverse RCCs.

This study investigates the effectiveness of ambient charges for Nonpoint Source (NPS) pollution in an n-firm Cournot oligopoly framework. Previous research showed that ambient charges can decrease NPS pollution in a two-firm Cournot duopoly model. We extend this analysis to demonstrate that ambient charges can abate NPS pollution in an n-firm Cournot oligopoly case. We use standard market demand functions for n-firm Cournot cases in the same industry. The results show that a reduction in NPS pollution occurs not only in a static Cournot duopoly case, but also in a static n-firm Cournot oligopoly cases.

The incidence of male reproductive system disorders, especially hypospadias, has been increasing in developed countries since the latter half of the 20th century. Endocrine-disrupting chemicals from the environment are considered to be involved in hypospadias onset through epigenetic alterations. This pilot study aimed to explore disease-specific methylated CpGs in human patient samples using the methylated-site display-amplified fragment length polymorphism (MSD-AFLP) technique developed by our research group [1]. We compared clinical samples from hypospadias and phimosis patients. Foreskin and blood samples were collected from one- to two-year-old patients with hypospadias (N = 3) and phimosis (N = 3) during surgical treatment. MSD-AFLP analysis showed significantly decreased CpG-methylation levels of genes such as MYH11 and increased CpG-methylation levels of genes such as PLA2G15 in hypospadias patients. Hierarchical clustering analysis showed that genes with significantly altered CpG levels were more markedly altered in DNA from blood than from foreskin. Because of the small number of samples, further investigation is necessary to elucidate the association between variations in CpG levels in foreskin and blood DNA and male genital abnormalities. However, our MSD-AFLP method appears to be a useful tool for exploring disease-specific methylated-CpGs in human epidemiological studies.

Additional file 1: Supplementary Table S1. Hana-chan's best chance for pregnancy and postpartum weight loss.

application/pdf, This study investigates the effectiveness of ambient charges for Non-point Source (NPS) pollution in an n-firm Cournot oligopoly framework. Previous research showed that ambient charges can decrease NPS pollu-tion in a two-firm Cournot duopoly model. We extend this analysis to demonstrate that ambient charges can abate NPS pollution in an n-firm Cournot oligopoly case. We use standard market demand functions for n-firm Cournot cases in the same industry. The results show that a re-duction in NPS pollution occurs not only in a Cournot duopoly case, but also in general Cournot oligopoly cases.

A mild, general and efficient hydrocyanation and hydroarylation of enamines catalyzed by Co(salen) complexes are described. Both reactions include regioselective C H bond formation of enamines, and the corresponding products are obtained in high yield. Hydroarylation critically discriminates the benzyl and benzoyl aromatic rings on nitrogen in cyclization step, and the corresponding isoindolinones including quaternary carbons are exclusively given.

Solitary fibrous tumor (SFT) of the kidney is a rare entity and usually displays a favorable prognosis. We herein report a second case of renal SFT developing local recurrence. A 50-year-old man was referred to our hospital because of a left renal mass. An abdominal CT detected a large renal tumor and radical nephrectomy was performed with a possible diagnosis of renal cell carcinoma. The resected tumor size was measured at 17 × 11 × 8 cm. Grossly, necrosis was observed in central lesion of the tumor but hemorrhage was not observed. Microscopically, the tumor consisted of spindle-shaped cells with scant cytoplasm accompanied by hyalinized collagenous tissue, which displayed hemangiopericytomatous patterns. The cellularity was normal and nuclear pleomorphism was not observed. Ki-67 labeling index was less than 3%. The pathological diagnosis of SFT was made without obvious malignant findings. Three years after the surgery, a follow-up CT scan detected a mass lesion in the tumor bed. Surgical resection was performed and the resected tumor was compatible with local recurrence of the SFT without obvious malignant findings. Renal SFT should be carefully monitored even in the absence of obvious malignant findings.

We report the first case in Japan of paraneoplastic dermatomyositis with pure seminoma, a tumor that extremely rarely accompanies dermatomyositis. The patient presented to the hospital with muscle weakness and erythema and was diagnosed with dermatomyositis from skin biopsy. Routine radiological screening revealed testicular tumor and massive lymph node metastases. We initially performed orchiectomy along with conventional immunotherapy. However, muscle weakness gradually worsened, and he eventually showed dysphagia and forced respiration and became bedridden. Although he seemed close to being too unstable to tolerate further treatment, we started carefully adjusted chemotherapy comprising 4 courses of etoposide plus cisplatin, which proved highly successful. Lymph node metastases completely disappeared and swallowing and respiration fully normalized after completing chemotherapy. We believe that this clinical success was due to our decision to initiate chemotherapy even in such a weak patient.

This paper presents an experimental investigation of reinforced concrete (RC) beams with double layered longitudinal bars, bond-strengthening hooks and 1300MPa-class spirals. The hooks made of mild steel increased the bond strength along longitudinal bars. This experiment is aimed at quantification of not only the effectiveness of the spirals and bond-strengthening hooks on the RC members consisting of double layered longitudinal bars but also the influence of cut-off bars in an inner (second) layer. Twelve specimens were prepared and subjected to cyclic shear loads. All the specimens failed because of bond splitting along the longitudinal bars. The hooks, made of mild steel, increased the bond strength along the longitudinal bars. Applying the bond-strengthening hooks was found to improve the shear capacities of the specimens with the cut-off bars. The authors developed a truss mechanism model for members with longitudinal bars in an inner (second) layer. The model showed that the shear strength depends on the span/depth ratio L/D and inner (second) layer bar length L_d.

Statin therapy reduces enhanced cardiac sympathetic nerve activity (CSNA) in patients with heart disease, and prevents left ventricular (LV) remodeling in chronic heart failure (CHF) patients. We sought to evaluate the effects of statin therapy on CSNA, as evaluated by 123I-metaiodobenzylguanidine (MIBG) scintigraphy, and LV remodeling in CHF patients. This study was sub-analysis of our previous report of the result that the serial 123I-MIBG studies were the most useful prognostic indicator in CHF patients. Patients with CHF (n = 208; left ventricular ejection fraction

Multislice spiral computed tomography (MSCT) is a new non-invasive imaging technique for detecting coronary artery disease. It allows direct visualization of not only the lumen of the coronary arteries, but also plaque within the artery. Identification of soft plaques is of the utmost importance in the therapeutic decision making for patients with acute coronary syndrome (ACS), including acute myocardial infarction and unstable angina pectoris. MSCT detected coronary artery soft plaques in 2 cases of ACS.

Synthesis and characterization of the monoruthenium(III)-substituted Dawson polyoxotungstate K7[α2-P2W17O61RuIII(H2O)]·19H2O 2, expected to be an effective homogeneous oxidation catalyst precursor, is described. The reaction products of cis-[RuIICl2(DMSO)4] as the ruthenium source with mono-lacunary Dawson polyoxotungstate have been examined. By 5 h reaction of cis-[RuIICl2(DMSO)4] with K10[α2-P2W17O61] in ice-cooled, HCl-acidic aqueous solution, a water-soluble 1 ∶ 2-type diamagnetic ruthenium(II) complex, with formula K18[RuII(DMSO)2(P2W17O61)2]·35H2O 1, was unexpectedly obtained as an analytically pure, homogeneous tan-colored solid, in which two DMSO molecules are coordinated to the ruthenium(II) atom. By Br2 oxidation of 1, the 1 ∶ 1-type paramagnetic complex 2 was obtained as a black crystalline solid. Characterization of 1 and 2 has been carried out with complete elemental analysis, thermogravimetric and differential thermal analyses (TG/DTA), FT-IR, magnetic susceptibility measurements, cyclic voltammetry, UV-vis absorption and 31P and 183W NMR spectroscopies, as well as 1H and 13C NMR for 1.

The total amount of nm23 protein, the relative ratios of H1 and H2 isoforms (H2/H1) and the localization of these proteins in human normal tissues were studied by a semiquantitative immunoblot technique followed by densitometry and immunohistochemistry with monoclonal antibody against nm23 protein (Pan-242). All tissues contained both isoforms recognized as the 20.5kD H1 protein and 18.5kD H2 protein by immunoblotting. Nm23 protein was abundant in liver, kidney and adrenal gland tissue, and scarce in heart and muscle. H2 levels were always higher than H1, but the isoform ratios (H2/H1) were variable from tissue to tissue. Immunostaining revealed that nm23 protein was predominantly present in cytoplasm and the pattern of staining was homogeneous in parenchymal cells of the liver, pancreas and colonic mucosa and heterogeneous in gastric mucosa and kidney. These results demonstrated that the levels of nm23 protein and the H2/H1 ratios and distribution of isoforms were different in each tissue, and suggests that, when the alterations of nm23 gene expression in tumor tissues are examined, the levels and ratios in non-tumorous tissues surrounding the tumor nest should be considered., Acta Histochemica et Cytochemica, 31(5), pp.411-418; 1998

La–Eu solid solution nanosheets La2/3−xEuxTa2O7 have been synthesized, and their photoluminescence properties have been investigated. La2/3−xEuxTa2O7 nanosheets were prepared from layered perovskite compounds Li2La2/3−xEuxTa2O7 as the precursors by soft chemical exfoliation reactions. Both the precursors and the exfoliated nanosheets exhibit a decrease in intralayer lattice parameters as the Eu contents increase. However, there is a discontinuity in this trend between the nominal Eu content ranges x≤ 0.3 and x ≥ 0.4. This discontinuity is attributed to the difference in degree of TaO6 octahedra tilting for the La- and Eu-rich phases. La2/3−xEuxTa2O7 nanosheets exhibit red emission, characteristic of the f–f transitions in Eu3+ photoactivators. The photoluminescence emission can be obtained from both host and direct photoactivator excitation. However, photoluminescence emission through host excitation is much more dominant than that through direct photoactivator excitation, and this behavior is consistent with that of all the other rare-earth photoactivated nanosheets reported previously. The absolute photoluminescence quantum efficiency of the La2/3−xEuxTa2O7 nanosheets increases as the experimentally determined Eu contents increase up to x=0.45 and decrease above it. This result is in good agreement with the optimum photoactivator concentration expected from the percolation theory. These solid solution La2/3−xEuxTa2O7 nanosheets are excellent models for validating the theory of optimum photoactivator concentration in the truly two-dimensional photoactivator matrix.

International audience; Extracting structural information from mesh models is crucial for Simulation Driven Design (SDD) in industrial applications. Focusing on thin-plate CAD mesh models (the most commonly used parts in electronic products such as PCs, mobile phones and so on), we present an algorithm based on primitive fitting for segmenting thin-plate CAD mesh models into parts of three different types, two of which are extruding surfaces and the other is a lateral surface. This method can be used for solid model reconstruction in the SDD process. Our approach involves two steps. First, a completely automatic method for accurate primitive fitting on CAD meshes is proposed based on the hierarchical primitive fitting framework. In the second step, a novel procedure is proposed for splitting thin-plate CAD mesh models by detecting parallel extruding surfaces and lateral surfaces. The method presented here has been proved to work smoothly in applications of real product design.

Salinity observations in the Shikoku Basin south of Japan have been made during the period of 1983-1988. Focus is placed on the salinity minimum layer, which exists at depths of 400-600 m over acontinental slope and/or ridge slope and at depths of 700-1000 m over a flat deep basin. In the presentreport, all the salinity data along the observational lines are presented. Some interesting features of the salinity fields are detected. Two dominant salinity minimum areas arefound in the salinity minimum layer. One minimum area exists over the continental slope and the otherminimum exists over the flat basin in the Shikoku Basin, which locates 200-300 km offshore to the formerminimum. It is suggested that the former minimum is originated by the intermediate Oyashio water,which flows sourthward along the Boso Peninsula. The latter minimum over a flat basin is inferred to beoriginated from the second Oyashio intrusion. On the whole, the character with two minimum salinityareas is observed in further south in the Shikoku Basin, which was well observed in the eastern region tothe Izu-Ogasawara Ridge., 1983年から1988年の間に日本南岸の四国海盆北部の塩分分布の観測を特に塩分極小層に注目して行った。塩分極小層は大陸棚斜面上や海嶺の斜面上では400－600m深程度にあり，平坦な大洋底上では700－1000m深程度にある。この報告では観測したすべての観測線上の塩分分布の基礎データを提示する。塩分観測データの解析により，幾つかの特徴的な塩分分布があることが確認された。塩分極小層は明確に大陸斜面上にあるものとそれより200－300km程度沖側の平坦な四国海盆上にあるものの二つの領域に別れて存在する。前者は房総半島沿岸沿いに南下する親潮の中層水に関連し，後者は親潮の第二貫入以東の南下に関連することが示唆された。伊豆海嶺以東では常時観測されるこの二つの塩分極小の存在が伊豆海嶺以西の四国海盆でもみられることが示された。

The Kuroshio south of Japan shows a clear bimodal path characteristic between large meander pathand no large meander path. It has been pointed out by many studies that the volume transport of theKuroshio is an important parameter which controls the selection of the bimodal Kuroshio path. However,a clear relationship between the intersity of volume transport and the selection of the Kuroshio path hasbeen not clarified yet. From these view points, we have made hydrographic observations of the Kuroshiosouth of Japan over the period of 1983-1988. In this report, we present the basic data of geostrophicvolume transport referred to 1500 db and the volume transport observed by suspended current meter fromthe vessel. It is shown that there exists a remarkable variation in volume transport. The geostrophictransport is relatively larger in period of no large meander (70.5±9.8 Sv) than that in large meander (61.8±20.0 Sv. The largest transport is observed during the transition period from no meander path to largemeander path in winter of 1986., 日本南岸の黒潮は大蛇行流路と非大蛇行流路との間で明確な二様性を示す。従来の多くの研究から黒潮流量が流路の重要なパラメータであることが指摘されているが，はっきりした黒潮流量の大小とその時の流路の形との関連が得られていない。このような観点から，1983年から1988年の間に黒潮に関する各層観測を行った。この報告では1500dbを無流面とする地衡流および船から係留した流速計による流速値の鉛直分布を提示した。全データの平均から，黒潮流量は非大蛇行期に70.5±9.8Svで大蛇行期の61.8±20.0Svよりも大きいことがわかった。また，黒潮流量は1986年の冬の非大蛇行流路から大蛇行流路への移行期に最大となることが示された。

A 49-year-old woman was admitted to hospital because of heart failure. She was diagnosed as having mitochondrial cardiomyopathy and diabetes mellitus. Echocardiography revealed a hypertrophic and poorly contracting left ventricle. A diagnosis of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes was established by muscle biopsy. She underwent technetium-99m-sestamibi (99mTc-MIBI) and beta-methyl-p-123I-iodophenyl-pentadecanoic acid (123I-BMIPP) scintigraphic examinations. 99mTc-MIBI single-photon emission computed tomography revealed reduced tracer uptake in the hypertrophic left ventricular inferior wall. In contrast, there was an increase in 123I-BMIPP uptake in the in the region of reduced 99mTc-MIBI uptake (99mTc-MIBI/123I-BMIPP mismatch). There was rapid washout of 99mTc-MIBI from the myocardium (washout rate increased by 30%). Decreased 99mTc-MIBI and increased 123I-BMIPP uptake (99mTc-MIBI/123I-BMIPP mismatch) were the characteristics of cardiac involvement in mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes.

Mid-ventricular hypertrophic obstructive cardiomyopathy is a rare type of cardiomyopathy that can be accompanied by apical aneurysm. We report the case of a patient who presented with ventricular fibrillation, ST-segment elevation on electrocardiography, and cardiac-enzyme elevation, in the presence of normal coronary arteries. Echocardiography and magnetic resonance imaging showed an hourglass appearance of the left ventricle with an aneurysm in the apex. Left-heart catheterization and continuous-wave Doppler echocardiography revealed a pressure gradient between the apical and basal chambers of the left ventricle. Impaired coronary artery circulation might play a role in the development of mid-ventricular obstruction in patients with mid-ventricular hypertrophic obstructive cardiomyopathy.

Multislice spiral computed tomography (MSCT) is a new non-invasive imaging technique for detecting coronary artery disease. It allows direct visualization of not only the lumen of the coronary arteries, but also plaque within the artery. Identification of soft plaques is of the utmost importance in the therapeutic decision making for patients with acute coronary syndrome (ACS), including acute myocardial infarction and unstable angina pectoris. MSCT detected coronary artery soft plaques in 2 cases of ACS.

Summaries of technical papers of Annual Meeting Architectural Institute of Japan. C-2, Structures IV, Reinforced concrete structures prestressed concrete structures masonry wall structures

Summaries of technical papers of Annual Meeting Architectural Institute of Japan. C-2, Structures IV, Reinforced concrete structures prestressed concrete structures masonry wall structures

Summaries of technical papers of Annual Meeting Architectural Institute of Japan. C-2, Structures IV, Reinforced concrete structures prestressed concrete structures masonry wall structures

本研究は風の乱れに起因する空力振動現象であるガスト応答の解析手法に関連している．ガスト応答解析において重要なパラメータの一つが，構造体に作用する変動空気力のspan方向空間相関である．近年の研究によると，接近流の風速鉛直成分のコヒーレンスより変動空気力のコヒーレンスが大きくなるという報告がなされており，この精緻な評価が近年のガスト応答に関する研究の課題となっている．従来の研究によって変動圧力の空間相関を高める要因として，接近流の気流特性(空間相関特性)，断面周りの流れ場(Bluff body flow)，及び構造体後流に形成されるカルマン渦の3つであると言われている．本研究では，矩形断面を対象とした風洞実験を通して，異なる断面辺長比，断面幅及び接近流の気流特性における変動空気力の空間相関に基づいたガスト応答解析をたわみ1自由度ばね支持振動実験結果と比較することで解析手法の適合性について検討を行う．

論文(Article), The basically catalyzed reaction of aromatic aldehyde such as p-anisaldehyde with 1,1'-bis(p-acetylphenyl)ferrocene afforded, in addition to condensation products, ferrocenophane derivatives with two p-phenylene groups between the ferrocene system and the five membered bridge.

To evaluate short-term clinical and hemodynamic effects of tolvaptan therapy and to identify predictors of the therapeutic outcomes, we retrospectively recruited sixty consecutive hospitalized heart failure (HF) patients (70 ± 11 years) with volume overload. The subjects were divided into two groups on the basis of the changes in HF symptom scores and hemodynamic status assessed by right heart catheterization after tolvaptan therapy (median: 7 days). The majority of patients were successfully treated (Group 1). However, 22% of patients (Group 2) were unsuccessfully treated, in whom 1) HF symptom score worsened or 2) there was a stationary HF symptom score ≥6 points, and mean PCWP >18 mmHg and mean RAP >10 mmHg, after tolvaptan therapy. HF symptom scores, hemodynamic parameters and plasma brain natriuretic peptide (BNP) level improved in Group 1, but all of these parameters remained unchanged in Group 2. Lower urine sodium/creatinine ratio (UNa/UCr) and higher BNP level at baseline were independently associated with unsuccessful tolvaptan therapy, and UNa/UCr best predicts unsuccessful tolvaptan therapy with a cut-off value of 46.5 mEq/g-Cr (AUC 0.858, 95% CI: 0.743-0.974, sensitivity 81%, specificity 77%, p 778 pg/ml predicted unsuccessful tolvaptan therapy with high diagnostic accuracy (sensitivity 54%, specificity 100%, positive predictive value 100%, negative predictive value 89% and accuracy 90%). In summary, short-term tolvaptan therapy ameliorated HF symptoms and provided hemodynamic improvement in the majority of patients, and UNa/UCr and BNP level strongly predicted the therapeutic outcomes., 本文 / Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, 36p