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Neonatal diabetes mellitus (NDM), defined as diabetes with an onset during the first 6 months of life, is a rare form of monogenic diabetes. The initial publications on this condition began appearing in the second half of the 1990s and quite surprisingly, the search for new NDM genes is still ongoing with great vigor. Between 2018 and early 2024, six brand new NDM‐genes have been discovered (CNOT1, FICD, ONECUT1, PDIA6, YIPF5, ZNF808) and three genes known to cause different diseases were identified as NDM‐genes (EIF2B1, NARS2, KCNMA1). In addition, NDM cases carrying mutations in three other genes known to give rise to diabetes during childhood have been also identified (AGPAT2, BSCL2, PIK3R1). As a consequence, the list of NDM genes now exceeds 40. This genetic heterogeneity translates into many different mechanism(s) of disease that are being investigated with state‐of‐the‐art methodologies, such as induced pluripotent stem cells (iPSC) and human embryonic stem cells (hESC) manipulated with the CRISPR technique of genome editing. This diversity in genetic causes and the pathophysiology of diabetes dictate the need for a variety of therapeutic approaches. The aim of this paper is to provide an overview on recent achievements in all aspects of this area of research. [ABSTRACT FROM AUTHOR]

The development of pneumococcal conjugate vaccines has substantially decreased the incidence of invasive pneumococcal diseases. However, the incidence of bacteremia due to nonvaccine serotypes has increased. Therefore, it is important to recognize the symptoms and complications of pneumococcal infection to expedite diagnosis. Herein, we report the case of a 22-month-old patient diagnosed with pneumococcal bacteremia who presented with cholestatic jaundice and fever without any other signs of systemic sepsis. The patient's condition improved soon after antibacterial therapy. Other causes of jaundice were possibly ruled out based on the screening test for viral hepatitis, and there were no abnormal findings on abdominal ultrasonography. Therefore, the present case suggests that cholestatic jaundice could be a manifestation of pneumococcal bacteremia. [ABSTRACT FROM AUTHOR]

The article describes the case of a 24-year old male patient with hepatic nuclear factor 4 alpha-maturity onset diabetes of the young (HNF4A-MODY) who was treated with a sodium-glucose cotransporter 2 inhibitor (SGLT2i). The patient showed absence of autoantibodies, insulin deficiency and a family history of young-onset diabetes without obesity. His treatment plan included empagliflozin and gliclazide to achieve the targeted glycemic control.

Purpose: To compare image quality of abdominal arteries between full-iodine-dose conventional CT and half-iodine-dose virtual monochromatic imaging (VMI).Materials and Methods: We retrospectively evaluated images of 21 patients (10 men, 11 women; mean age, 73.9 years) who underwent both full-iodine (600 mg/kg) conventional CT and half-iodine (300 mg/kg) VMI. For each patient, we measured and compared CT attenuation and the contrast-to-noise ratio (CNR) of the aorta, celiac artery, and superior mesenteric artery (SMA). We also compared CT dose index (CTDI). Two board-certified diagnostic radiologists evaluated visualisation of the main trunks and branches of the celiac artery and SMA in maximum-intensity-projection images. We evaluated spatial resolution of the two scans using an acrylic phantom.Results: The two scans demonstrated no significant difference in CT attenuation of the aorta, celiac artery, and SMA, but CNRs of the aorta and celiac artery were significantly higher in VMI (p = 0.011 and 0.030, respectively). CTDI was significantly higher in VMI (p = 0.024). There was no significant difference in visualisation of the main trunk of the celiac artery and SMA, but visualisation of the gastroduodenal artery, pancreatic arcade, branch of the SMA, marginal arteries, and vasa recta was significantly better in the conventional scan (p < 0.001). The calculated modular transfer function (MTF) suggested decreased spatial resolution of the half-iodine VMI.Conclusion: Large-vessel depiction and CNRs were comparable between full-iodine conventional CT and half-iodine VMI images, but VMI did not permit clear visualisation of small arteries and required a larger radiation dose.Key Points: ・Reducing the dose of iodine contrast medium is essential for chronic kidney disease patients to prevent contrast-induced nephropathy. ・In virtual monochromatic images at low keV, contrast of relatively large vessels is maintained even with reduced iodine load, but visibility of small vessels is impaired with decreased spatial resolution. ・We should be aware about the advantages and disadvantages associated with virtual monochromatic imaging with reduced iodine dose. [ABSTRACT FROM AUTHOR]

Glucocorticoids have strong effects on diverse human activities through the glucocorticoid receptor (GR). Sirtuin 1 (SIRT1) is a NAD + -dependent histone deacetylase and promotes longevity by influencing intermediary metabolism and other regulatory activities including mitochondrial function. In this study, we examined the effects of SIRT1 on GR-mediated transcriptional activity. We found that SIRT1 enhanced GR-induced transcriptional activity on endogenous and exogenous glucocorticoid-responsive genes, whereas knockdown of SIRT1 attenuated it. This effect of SIRT1 was independent to its deacetylase activity, as the SIRT1 mutant defective in this activity (H363Y) enhanced GR transcriptional activity, and the compounds inhibiting or activating the SIRT1 deacetylase activity did not influence it. RNA-seq analysis revealed that SIRT1 knockdown influenced ∼30% of the glucocorticoid-responsive transcriptome for most of which it acted as an enhancer for positive/negative effects of this hormone. SIRT1 physically interacted with GR, and was attracted to GR-bound glucocorticoid response elements in a glucocorticoid-dependent fashion. SIRT1 cooperatively activated GR transcriptional activity with the PPARγ coactivator-1α also in its deacetylase activity-independent fashion. Thus, SIRT1 is a novel transcriptional enhancer of GR-induced transcriptional activity possibly by functioning as a scaffold for the transcriptional complex formed on GR. [ABSTRACT FROM AUTHOR]

Background Neonatal diabetes mellitus (NDM) is a monogenic insulin-dependent diabetes that develops within 6 months of age. The progression of hyperglycemia until diagnosis is unknown. Glycemic control indicators at diagnosis are useful to estimate the extent and duration of hyperglycemia. We recently established that age-adjusted glycated albumin (GA) is a useful indicator of glycemic control, regardless of age. Objective To compare the levels of various glycemic control indicators at diagnosis between NDM and other types of insulin-dependent diabetes mellitus. Patients and Methods We included 8 patients with NDM, 8 with fulminant type 1 diabetes (FT1D), and 24 with acute-onset autoimmune type 1 diabetes (T1AD). Plasma glucose, glycated hemoglobin (HbA1c), GA, and age-adjusted GA (calculated as previously reported) were measured and compared. Results There were no significant differences in the plasma glucose levels of the group of patients with NDM and those with FT1D or T1AD. HbA1c and GA levels in the NDM group were not significantly different from those in the FT1D group, and both indicators were lower than those in the T1AD group. Age-adjusted GA levels in the NDM group did not differ significantly from those in the T1AD group, but were higher than those in the FT1D group. Conclusions These findings suggest that the time-course of plasma glucose elevation in NDM until diagnosis is similar to that in T1AD. In addition, the high age-adjusted GA value at diagnosis of NDM indicates that this test is useful for assessing chronic hyperglycemia in NDM. [ABSTRACT FROM AUTHOR]

Background While the association between scoliosis and cardiac and respiratory function impairments has been well characterized in clinical practice and research, the potential effect of scoliosis on urinary tract structure and renal function has received little attention. Therefore, the purpose of this study was to evaluate the preoperative clinical characteristics of urinary tract structure and renal function in pediatric patients with idiopathic scoliosis, using a combination of blood tests, urinalysis, and imaging. Methods Preoperative measures of urinary tract structure and renal function were obtained for 16 patients, 13-17 years old, scheduled for corrective surgery for idiopathic scoliosis. Preoperative assessment included blood test and urinalysis, combined with structural imaging on ultrasound ( US), magnetic resonance imaging ( MRI), magnetic resonance urography ( MRU), and radioisotope tracing ( RI), using technetium-99 m mercaptoacetyltriglycine (99mTc- MAG3). Differences in blood and urine tests between patients with and without urinary tract obstruction ( UTO) were evaluated for significance using Mann-Whitney U test. Results For all 16 patients, blood tests and MRU were within normal limits. Dilatation of the renal pelvis was identified on US in eight patients (50.0%). UTO was identified on RI in six patients (37.5%). UTO was associated with elevated β2-microglobulin concentration. Urinary β2-microglobulin concentration >0.7 μg/mg Cr differentiated patients with UTO from those without UTO, with a sensitivity of 100% and specificity of 70%. Conclusions β2-Microglobulin concentration may be a useful marker to screen for asymptomatic UTO in patients with idiopathic scoliosis. [ABSTRACT FROM AUTHOR]

Background: Glycated albumin is a useful glycaemic control indicator for neonatal diabetes mellitus. However, glycated albumin concentrations in infants are lower than those in adults and increase in an age-dependent manner. Based on our investigation of non-diabetic subjects, we proposed the possibility that the reference range for adults may be used regardless of age, provided that age-adjusted glycated albumin is employed. In the present study, we evaluate the usefulness of age-adjusted glycated albumin in neonatal diabetes mellitus patients.Methods: Six neonatal diabetes mellitus patients (four patients with permanent neonatal diabetes mellitus and two patients with transient neonatal diabetes mellitus) were included. Measured glycated albumin or age-adjusted glycated albumin was compared to calculated glycated albumin, which was determined using calculation formulae we had reported based on past blood glucose over the 50 days before measurement of glycated albumin.Results: Measured glycated albumin was significantly lower than calculated glycated albumin (20.5 ± 4.9% versus 28.2 ± 6.1%; p < 0.0001), whereas age-adjusted glycated albumin was equivalent to calculated glycated albumin, showing no significant difference (27.5 ± 6.8% versus 28.2 ± 6.1%). Measured glycated albumin concentrations in patients with transient neonatal diabetes mellitus in remission were lower than the reference range for adults, whereas age-adjusted glycated albumin concentrations were within the reference range for adults.Conclusion: We demonstrated that age-adjusted glycated albumin concentrations were consistent with calculated glycated albumin. Age-adjusted glycated albumin is therefore a useful glycaemic control indicator for neonatal diabetes mellitus patients. [ABSTRACT FROM AUTHOR]

An Fe-Ga alloy single crystal of about 10 mm in diameter and about 60 mm in length was grown by the Czochralski (CZ) method. The as-grown ingot was approximately oriented in the \(\langle 100\rangle \) direction parallel to the pull-up direction. The gallium concentration of the as-grown ingot was close to that of the starting polycrystalline alloy. The magnetostriction of about −212 ppm was observed in the \(\langle 100\rangle \) direction by applying a magnetic field perpendicular to the measured direction. In addition, the superior performance of vibration power generation due to the large inverse magnetostrictive effect was demonstrated by a prototype device using the as-grown Fe-Ga alloy single crystal. It is concluded that Fe-Ga alloy single crystals grown by the CZ method are promising for application to vibration power generators. [ABSTRACT FROM AUTHOR]

The article focuses on level of glycated albumin (GA) in non-diabetic subjects of different age groups. Increase in GA levels in an age-dependent manner in infancy is discussed. It involves the study of GA levels of 376 non-diabetic subjects and establishes a relationship between GA and logarithmically transformed age. A formula is devised to use the adult reference range as a potential for treatment monitoring of diabetic patients regardless of age.

The most common cause of neonatal diabetes, KCNJ11 gene mutation, can manifest as a neurological disorder. The most severe form consists of a constellation of developmental delay, epilepsy, and neonatal diabetes ( DEND). Intermediate DEND ( iDEND) refers to a milder presentation without epilepsy. We present a child with iDEND, for whom insulin injections were replaced with glibenclamide therapy at 17 months of age because of poor glycemic control and delayed motor development. Three months after initiation of glibenclamide, HbA1c decreased from 10.2% to 5.6%. Continuous glucose monitoring indicated that blood glucose fluctuations were suppressed while on glibenclamide. Furthermore, after initiating glibenclamide therapy, the developmental quotient ( DQ) for motor ability markedly improved from 60 to 91, whereas the DQ for language and adoptive ability remained as they had been before the sulfonylurea treatment. Sulfonylurea treatment improved glycemic control and motor development in the present patient. [ABSTRACT FROM AUTHOR]

Protein ingestion can significantly affect glucagon secretion along with blood urea nitrogen alteration in type 1 diabetes We have read with great interest the article by Kawamori I et al i .1 showing that dysregulated glucagon secretion, with respect to plasma glucose (PG) in patients with type 1 diabetes, persists for a year. Similarly, dysregulated glucagon secretion with respect to glucose responds to protein in patients with type 1 diabetes, although it is unknown whether the response is physiologically appropriate. [Extracted from the article]

Background: Both HbA1c and glycated albumin (GA) values are influenced by weighted mean of preceding blood glucose levels depending on each half-life. Based on this principle, we developed formulas for calculation of HbA1c and GA using data from self-monitored blood glucose (SMBG). We also calculated HbA1c and GA in diabetic patients using the developed formulas. Methods: This study included 9 patients with childhood-onset type 1 diabetes mellitus (6 males and 3 females; aged 11.4±4.2y). From the weekly mean blood glucose (MBG) values obtained by the SMBG data during the previous 20weeks, we calculated HbA1c and GA using the developed formulas and compared the calculated values with the measured values (n=42). Results: The measured and the calculated values of HbA1c were 8.5±0.9% and 8.3±1.2%, respectively. The measured and the calculated values of GA were 24.9±3.7% and 26.4±4.0%, respectively. There were strong positive correlations between both values of HbA1c and GA (P<0.0001). Conclusions: The calculated HbA1c and GA values using the developed formulas from the SMBG data were generally in agreement with the measured values. Using the calculation formulas, the values of HbA1c and GA could be estimated from serially measured SMBG data. [ABSTRACT FROM AUTHOR]

Background As the presence of fetal hemoglobin ( HbF) affects the accuracy of hemoglobin A1c ( HbA1c) analysis methods, HbA1c measurement may not be a good indicator for patients with neonatal diabetes mellitus, whereas glycated albumin ( GA) may be a good indicator. Objective To investigate whether total glycated hemoglobin ( GHb) or HbF-adjusted HbA1c (adj- HbA1c) can act as a glycemic control marker in infants. Subjects and Methods Plasma glucose ( PG), GA, HbF, GHb measured using the affinity method, and HbA1c measured using the latex-immunoturbidimetry ( LA) or the high-performance liquid chromatography ( HPLC) methods were determined in 26 full-term newborn infants aged 4-234 d. Adj- HbA1c was calculated as HbA1c/(total Hb − HbF). Results GHb, adj- HbA1c measured using the LA and the HPLC methods were 4.8 ± 0.5%, 4.5 ± 0.5%, and 4.7 ± 0.6%, respectively. GA was most positively correlated with PG (r = 0.696, p < 0.0001). GHb was positively correlated with both PG (r = 0.479, p = 0.013) and GA (r = 0.727, p < 0.0001). Adj- HbA1c measured using the LA method was positively correlated with GA (r = 0.465, p = 0.017), but not PG (r = 0.304, p = 0.132). Adj- HbA1c measured using the HPLC method was correlated with neither PG (r = −0.077, p = 0.710) nor GA (r = 0.360, p = 0.071). Conclusions GHb measured using the affinity method may be a useful glycemic control marker in infants. Although adj- HbA1c measured using the LA method was correlated with GA, it may not be a practical measure because it was not correlated with PG and determining HbF levels using HPLC method can be troublesome. Adj- HbA1c measured using the HPLC method should not be used as a glycemic marker in infants. [ABSTRACT FROM AUTHOR]

Background Glycated albumin ( GA) reflects glycemic control in patients with neonatal diabetes mellitus ( NDM). However, GA in NDM patients is apparently low in relation to glycemia. Objective To establish the reference intervals for GA in healthy infants. Subjects and Methods Fifty-eight healthy, full-term newborn infants were used to define the GA reference values and to investigate its relationship to plasma glucose ( PG) and serum albumin. The infants were categorized into three groups according to age: group A, 5 (4-6) median (range) d: n = 18; group B, 33 (30-38) d: n = 19; and group C, 181 (50-352) d: n = 21. We also studied 212 non-diabetic adults [group D, 53 (28-78) yr old] and the 5 NDM patients previously reported for GA comparisons. Results In the infants, GA was strongly positively correlated with logarithmic transformation of age [log (age)] (p = 0.831, p < 0.0001). The GA in groups A, B, C, and D were 7.3 ± 1.0%, 8.6 ± 1.1%, 10.9 ± 0.8%, and 14.0 ± 1.1%, respectively. The GA was more strongly positively correlated with serum albumin (r = 0.768, p < 0.0001) than with PG (r = 0.596, p < 0.0001). When GA levels were compared with the age-dependent reference values, GA in the transient NDM patient was normalized although GA in the four permanent NDM patients decreased but remained high after insulin therapy. Conclusions This study showed that the reference range for GA in infants is lower than that of adults and increases with age, with which we confirmed that GA in the NDM patients reflected the clinical course. Consequently, GA in NDM patients should be compared with the age-based reference values to assess the accurate glycemic status. [ABSTRACT FROM AUTHOR]

In this study, we performed three breath tests – l-[1-13C ]phenylalanine breath test (PBT), l-[1-13C ] methionine breath test, and [13C]methacetin breath test (MethaBT) – in patients with chronic liver disease to determine the optimal timing of expired air collection for diagnosing chronic liver disease and evaluating the grade of fibrosis. The subjects were 61 adults with normal livers, 98 chronic hepatitis patients, and 91 liver cirrhosis patients. We investigated the relationships of breath test results with routine biochemical tests and the Child–Pugh score, as well as the diagnostic capacities of the breath tests for liver dysfunction/cirrhosis and grade of liver fibrosis. For the diagnosis of liver cirrhosis and correlations with liver fibrosis, the accuracy of the PBT at 30 min (PBT30) was similar to that of the MethaBT at 15 min (Metha15). For liver function assessment by two-point measurement with 13C breath tests, we recommend the PBT30 and the Metha15. [ABSTRACT FROM PUBLISHER]

Abstract: Objective: To evaluate the MR findings of ruptured endometrial cyst, focusing on the differentiation from tubo-ovarian abscess (TOA). Patients and methods: We reviewed the records of 21 patients who underwent preoperative MR examinations for TOAs (n =15) or ruptured endometrial cysts (n =6). We evaluated the presence of hyper-intense ascites and hyper-intense peritoneum in T1-weighted sequences, strong enhancement of the peritoneum, hyper-intense content and hyper-intense rim of the ovarian lesion in T1-weighted sequences, and strong wall enhancement of the ovarian lesion. χ 2 test was used to assess the relationship between TOA cases versus cases with ruptured endometrial cysts, and the three MR peritoneal findings. We evaluated the relationship between TOA versus non-infected endometrial cysts, and the ovarian MR peritoneal findings, too. Results: Hyper-intense ascites was found in all of the patients with ruptured endometrial cyst and none with TOA (p <0.0001). Hyper-intense peritoneum was observed in only TOAs cases (4 of 8). Strong peritoneal enhancement was seen in 3 of the 3 patients with ruptured endometrial cyst and 7 of the 13 patients with TOA (p =0.1366). Hyper-intense content of the ovarian lesion was seen more often in the non-infected endometrial cysts than in the TOAs (p =0.001607), while hyper-intense rim was more frequent in TOAs (p =0.000402). Strong wall enhancement was observed only in TOAs (11 of 15) (p =0.001355). Conclusions: MR images are useful to differentiate ruptured endometrial cyst from TOA. [Copyright &y& Elsevier]

Abstract: Objectives: To compare the performance of model-based iterative reconstruction (MBIR) with that of standard filtered back projection (FBP) for measuring vascular wall attenuation. Study design: After subjecting 9 vascular models (actual attenuation value of wall, 89HU) with wall thickness of 0.5, 1.0, or 1.5mm that we filled with contrast material of 275, 396, or 542HU to scanning using 64-detector computed tomography (CT), we reconstructed images using MBIR and FBP (Bone, Detail kernels) and measured wall attenuation at the center of the wall for each model. We performed attenuation measurements for each model and additional supportive measurements by a differentiation curve. We analyzed statistics using analyzes of variance with repeated measures. Results: Using the Bone kernel, standard deviation of the measurement exceeded 30HU in most conditions. In measurements at the wall center, the attenuation values obtained using MBIR were comparable to or significantly closer to the actual wall attenuation than those acquired using Detail kernel. Using differentiation curves, we could measure attenuation for models with walls of 1.0- or 1.5-mm thickness using MBIR but only those of 1.5-mm thickness using Detail kernel. We detected no significant differences among the attenuation values of the vascular walls of either thickness (MBIR, P =0.1606) or among the 3 densities of intravascular contrast material (MBIR, P =0.8185; Detail kernel, P =0.0802). Conclusions: Compared with FBP, MBIR reduces both reconstruction blur and image noise simultaneously, facilitates recognition of vascular wall boundaries, and can improve accuracy in measuring wall attenuation. [Copyright &y& Elsevier]

Background: Although glycated albumin (GA) is a useful glycemic control marker in neonatal diabetes mellitus (NDM), there has been no report comparing GA levels between NDM patients and non-diabetic infants. Moreover, GA in NDM patients may be apparently low in relation to glycemia due to the assumed elevation of albumin metabolism in neonates. Methods: We compared GA levels between 6 patients with NDM and 18 non-diabetic infants or 14 patients with type 1 diabetes mellitus (T1DM). Mean blood glucose (MBG) was calculated on the basis of self-monitoring of blood glucose for 1 month before GA measurement. Results: GA in NDM patients was significantly higher than that in non-diabetic infants (22.0 ± 5.8 vs. 10.2 ± 1.4%; p < 0.0001), and GA levels significantly correlated with MBG in both NDM and T1DM patients. However, GA in NDM patients was significantly lower than in T1DM patients (25.8 ± 5.3%; p = 0.0046), whereas MBG in NDM patients was significantly higher than in T1DM patients (233 ± 79 vs. 183 ± 41 mg/dl; p = 0.0006). Conclusion: GA levels in NDM patients were apparently low in relation to glycemia. Therefore, reference values for infants should be used for assessing the GA level in NDM. Copyright © 2012 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Abstract: Objectives: To evaluate the precision and accuracy in CT attenuation measurement of vascular wall using region-of-interest (ROI) supported by differentiation curves. Study design: We used vascular models (actual attenuation value of the wall: 87HU) with wall thicknesses of 1.5, 1.0, or 0.5mm, filled with contrast material of 250, 348, or 436HU. The nine vascular models were scanned with a 64-detector CT. The wall attenuation values were measured using three sizes (diameter: 0.5, 1.0, and 1.5mm) of ROIs without differentiation curves. Sixteen measurements were repeated for each vascular model by each of two operators. Measurements supported by differentiation curves were also performed. We used analyses of variance with repeated measures for the measured attenuations for each size of the ROI. Results: Without differentiation curves, there were significant differences in the attenuation values of the wall among the three densities of contrast material, and the attenuation values tended to be overestimated more as the contrast material density increased. Operator dependencies were also found in measurements for 0.5- and 1.5-mm thickness models. With differentiation curves, measurements were not possible for 0.5- and 1.0-mm thickness models. Using differentiation curves for 1.5-mm thickness models with a ROI of 1.0- or 1.5-mm diameter, the wall attenuations were not affected by the contrast material densities and were operator independent, measuring between 75 and 103HU. Conclusions: The use of differentiation curves can improve the precision and accuracy in wall attenuation measurement using a ROI technique, while measurements for walls of ≤1.0mm thickness are difficult. [Copyright &y& Elsevier]

Abstract: Objectives: To evaluate the skin dose during cerebral CT perfusion on a phantom, and estimate the weighted CT dose index (CTDIw) to maximum skin dose conversion factors for four types of CT scanners. Study design: We evaluated the relationship between surface dose during cerebral CT perfusion and distance from the scan center in the x–y plane using a 64-multidetector row CT scanner. Skin doses were also assessed with 4 different 64-multidetector CT scanners. Results: The surface doses decreased with the distance from the scan center in the x–y plane. The surface doses at the points 6cm and 10cm from the scan center in the x–y plane were different from the dose at the point 8cm by about 15%. CTDIw and skin doses differed among the CT scanners (CTDIw, 143–590mGy; averaged temporal skin dose, 126–590mGy). For all the four types of CT scanner, the doses increased in the following order: occipital point

To assess the entrance skin dose (ESD) during radiofrequency catheter ablation procedures for tachyarrhythmia including atrial fibrillation (Af). This study focused on 99 consecutive patients who underwent procedures for tachyarrhythmia (Af; n = 34, non-Af; n = 65) in three institutions. The non-Af group included atrial flutter, atrial tachycardia, paroxysmal supraventricular tachycardia, ventricular tachycardia, ventricular premature contraction, atrial premature contraction, atrioventricular nodal reentry tachycardia, and Wolff-Parkinson-White syndrome. In two of the three institutions, the procedures were performed for both Af and non-Af. The ESDs were measured using 100 radiosensitive indicators attached to the back of each patient's jacket at 5-cm intervals. For statistical analyses, multiple regression analysis (the dependent variable, Max-ESD; and the independent variables, dose area product [DAP], total fluoroscopic time [TFT], body mass index, etc.), Pearson's correlation test, and the Mann-Whitney test were employed. The overall averages for the TFTs, the DAPs, and the Max-ESDs were 49.9 ± 28.2 minutes, 71.2 ± 73.7 Gy cm, and 0.57 ± 0.51 Gy, respectively. DAP was positively related to the Max-ESD and was significant in stepwise multiple regression analysis (P < 0.0001). There was a significant association between TFT and Max-ESD in five of the six kinds of angiographic unit, and between DAP and Max-ESD in all three systems with available DAP measures. In one institution, TFT, DAP, and Max-ESD differed significantly between the Af and non-Af groups (P = 0.0002, P < 0.0001, and P < 0.0001). During the cardiac catheter ablation, ESDs of only a few patients exceeded the thresholds of radiation skin injuries, and the DAP proved useful to estimate each patient's Max-ESD. (PACE 2011; 34:563-570) [ABSTRACT FROM AUTHOR]

S-1 is an oral anticancer drug containing tegafur (FT), a pro-drug of fluorouracil, combined with two modulators, 5-chloro-2,4-dihydroxypyridine and potassium oxonate (Oxo), at a molar ratio of 1:0.4:1. CYP2A6 genetic polymorphism and dihydropyrimidine dehydrogenase (DPD) inhibition are important for the antitumor effect of S-1. Exploiting the usefulness of the 2-13C-uracil breath test (UrBT) as an indicator of DPD activity, we examined whether the results of CYP2A6 genetic polymorphism analysis and UrBT could be used to predict the antitumor effect of S-1. Thirty-four patients with advanced or recurrent cancer (15, 16 and 3 with gastric, colorectal and pancreatic cancer, respectively) were orally administered 40 mg/m2 S-1 twice daily in the morning and evening. Eighteen patients with a complete response (CR)/partial response (PR) (2 with CR, 16 with PR) and 16 with progressive disease (PD) were compared with respect to CYP2A6 genetic polymorphisms (1- vs. 2-allele mutation), UrBT results, and plasma FT and 5-fluorouracil levels at 3 h after S-1 ingestion in the morning. On multivariate analysis between the CR/PR and PD groups, only the UrBT results was an independent factor of CR/PR to S-1 (95% CI 1.02–1.10). These results suggest that the anticancer effect of S-1 can be predicted by performing UrBT 3 h after the initial oral S-1 administration. [ABSTRACT FROM AUTHOR]

A simple and quick online solid-phase extraction (SPE) coupled to liquid chromatography (LC)/tandem mass spectrometry (MS/MS) for the determination of the five antibiotics (florfenicol, FF; lincomycin, LCM; oxytetracyclin, OTC; tylosin, TS; valnemulin, VLM) in swine wastewater has been developed. After filtration, aliquots (100 μl) of wastewater samples were directly injected to a column-switching LC system. Some matrix interference was removed by washing up SPE column with 0.2% formic acid solution and acetonitrile. Antibiotics eluted from SPE column were separated on analytical column by converting switching valve and were detected by MS/MS. Calibration curves using the method of standard addition had very good correlation coefficients ( r > 0.99) in the range of 0.1 to 2 ng/ml. The intra-day precision of the method was less than 12% and the inter-day precision was between 6 to 17%. The detection limits were 0.01–0.1 ng/ml. When this method was applied to wastewater samples in swine facilities, four compounds (LCM, OTC, TS, and VLM) were detected. [ABSTRACT FROM AUTHOR]

Abstract: Secondary ion mass spectrometry (SIMS) was used for characterizing the depth distribution of small amounts of alloying elements in three different copper-based dilute alloys, i.e., copper–chromium, copper–iron, and copper–nickel–silicon alloys. The samples were annealed at high temperatures under a low oxygen partial pressure. The SIMS depth profiles revealed that oxygen penetrated the copper-based alloys, and chromium and silicon were enriched on the surface side so as to form oxides during annealing. On the other hand, chromium and silicon were depleted beneath the enriched surface layer. However, the depth profile of iron was similar to that of copper. These phenomena were likely to be correlated with the reactivity of the alloying elements with oxygen. The formation kinetics of the depleted zones of chromium and silicon has been discussed on the basis of the selective oxidation of these alloying elements. [Copyright &y& Elsevier]

Abstract: The intrinsic zero-energy loss profiles of transition metal 2p and 3p XPS spectra for Cr, Mn, and Fe oxides are obtained by spectral deconvolution and compared with Shirley-type background corrected profiles. The metal core level spectra are deconvoluted by O 1s spectra as the response function of each oxide. As the O 1s spectra include intrinsic and extrinsic energy loss parts, the background corrected core level spectra are zero-energy loss spectra. The good agreement of the deconvoluted spectra with the reported spectra obtained by the many body effect theory indicates that the background subtraction method is accurate. A comparison of the deconvoluted with the background corrected spectra of the Shirely-type subtraction reveals that almost all the spectra coincide with each other except for Fe 3p with α-Fe2O3. The good coincidence of the Shirley-type corrected spectra with the deconvoluted and calculated spectra indicates that Shirley-type background correction can be used for daily quantitative surface analysis. [Copyright &y& Elsevier]

Novel luminescent material has been prepared by the reaction of Eu3+ and molybdate species in the matrix of faujasite (FAU) type zeolite X and successive calcination. Eu3+ exchanged FAU was reacted with MoO3 in the solid-state at 723 K, giving a precursor. By calcining it at 1073 K, different crystalline phases were derived depending on MoO3-loading levels. Scheelite type crystal of NaEu(MoO4)2 was formed at high MoO3-loading levels, whereas europium sodalite was formed at low loading levels. For the former sample, X-ray diffraction analysis and transmission electron microscopy revealed that the nanosized NaEu(MoO4)2 was dispersed homogeneously within amorphous aluminosilicate matrix originated from FAU. The amorphous particles containing NaEu(MoO4)2 maintained the original morphology, which the starting FAU particles possessed. The emission intensity of nanosized NaEu(MoO4)2 in the matrix was one order higher than that of europium sodalite. The emission lifetime of nanosized NaEu(MoO4)2 (0.39 ms) in the matrix was longer than that of bulk NaEu(MoO4)2 (0.35 ms) fabricated by conventional solid-state processes. [ABSTRACT FROM AUTHOR]

We report on a new species of enantiornithine bird from the Late Cretaceous of the Mongolian Gobi Desert, which adds to the meager record of birds from the latest part of the Mesozoic in this region. The exceptional, three-dimensional bones of the new fossil increase the known anatomical diversity of enantiornithine birds, providing information about previously unreported morphologies such as a pneumatised furcula. Several synapomorphies nest the new bird within an enantiornithine clade of proficient fliers, but the anatomy and proportions of its flight apparatus imply significant aerodynamic restrictions. The combination of phylogeny and our functional interpretation suggests that this new fossil bird is a representative of a flightless lineage. This discovery thus provides the first evidence of a trend towards more limited flying capabilities among Enantiornithes, a group of Cretaceous birds otherwise believed to be represented by competent fliers. [ABSTRACT FROM AUTHOR]

Abstract: Ex-situ X-ray diffraction measurements of a small amount of samples extracted from wet corrosion products freshly formed on a pure iron and iron–2mass% silicon surfaces have been conducted using synchrotron radiation for clarifying the formation process of corrosion products. The results showed that γ-FeOOH was formed on the outer side of wet corrosion products formed on the surface of the pure iron by sodium chloride solution, while γ-FeOOH, α-FeOOH, Fe3O4, and green rusts were formed on the inner side. On the other hand, in comparison to the case of the pure iron, a significant formation of β-FeOOH was observed in the iron–silicon alloy. Influences of silicon alloying on corrosion products formed by aqueous solution containing sulfate ions were also observed. Furthermore, in-situ diffraction measurements by a conventional X-ray source were conducted for analyzing corrosion products formed on the pure iron and iron–silicon alloy surfaces by cyclic exposure to wet and dry atmospheres. The results obtained by the in-situ diffraction and ex-situ diffraction measurements on the corrosion products were consistent. [Copyright &y& Elsevier]

Abstract: Fine particles of corrosion products consisting of one kind of ferric oxyhydroxide, either γ-FeOOH (lepidocrocite) or α-FeOOH (goethite), were directly prepared by dipping pure iron into aqueous solutions containing sodium chloride, sodium sulfate, or their mixed salts with sodium bicarbonate. Quantitative X-ray structural analysis using an in-house X-ray diffraction apparatus has been used for characterizing the atomic-scale structure of these ferric oxyhydroxide particles. The morphology of the γ-FeOOH and α-FeOOH particles was observed by transmission electron microscopy (TEM), and their bonding structures were analyzed using Fourier transform infrared spectroscopy (FT-IR). The realistic atomic-scale structures in the γ-FeOOH and α-FeOOH particles were estimated by fitting the interference functions with the help of the reverse Monte Carlo (RMC) simulation technique. The results showed that the linkages of fundamental FeO6 octahedral units in the particles were deviated from the ideal crystal structure. The structural deviation is believed to be due to the incorporation of foreign anions during the formation of these particles in the aqueous solutions. The resultant atomic-scale structures in the γ-FeOOH and α-FeOOH particles were correlated with their morphology and bonding structure. [Copyright &y& Elsevier]

Abstract: Quantitative X-ray structural analysis coupled with reverse Monte-Carlo (RMC) simulation and infrared spectroscopy have been used for characterizing the atomic scale structure in α-FeOOH particles with and without anions consisting mainly of carbonate. The results showed that these α-FeOOH particles fundamentally have the α-FeOOH structure, but the linkages of FeO6 octahedral structural units in the α-FeOOH particles with the anions are significantly distorted, and the distortion appears to be related with the anions in the α-FeOOH particles. As α-FeOOH are formed through olation and oxolation of iron complexes described by FeO6 structural units with hydrogen in aqueous solution, the atomic scale structure in the α-FeOOH particles may be influenced by anions. [Copyright &y& Elsevier]

Abstract: The realistic atomic-scale structure of synthesized α-FeOOH (goethite) particles, which is a typical ferric oxyhydroxide of corrosion product formed on the steel surface, has been estimated by fitting both the ordinary interference function and the environmental interference function obtained from the anomalous X-ray scattering (AXS) with model calculation using the reverse Monte Carlo (RMC) simulation technique. The results indicated that the FeO6 octahedral unit structure is unchanged in α-FeOOH particles with and without chromium, but distortion of the linkage of FeO6 octahedral unit structure is quite likely induced by chromium addition, where chromium is found to substitute for iron site in α-FeOOH particles. The middle-range ordering (≈nm scale) characterized by the FeO6 octahedral unit structure in α-FeOOH particles was also visualized. [Copyright &y& Elsevier]

Air samples collected at 23 sites in Kanagawa Prefecture, Japan in 1998 and 1999 were analyzed for PCBs by a high-resolution gas chromatograph/mass spectrometer. Concentrations of PCBs ranged from 10 to 3290 pg/m3 N (geometric average, 120 pg/m3N) and were mostly less than 140 pg/m3 N. The toxicity equivalency quantity (TEQ) values of co-planar PCBs ranges from not detected (ND) to 0.35 pg-TEQ/m3 N (mean value, 0.00063 pg-TEQ/m3 N). Cl3-PCBs comprised the highest level in many air samples. PCB congeners in the samples collected on three consecutive days did not vary significantly, suggesting that variation of PCBs concentrations is minimal among the samples collected at same sites. On the other hand, the pattern similarities of PCBs in the samples collected at various areas were not consistent. Contamination from commercial PCBs (Kaneclor) was observed in some samples with high PCBs concentrations. The compositions of PCB congeners in the samples collected at the sites near municipal waste incinerators were similar to those of PCB congeners in the samples collected at common environments in Japan, whereas they were completely different from those in the exhaust gases collected directly from municipal waste incinerators. Consequently, contribution of exhaust gases from incinerators to PCB concentrations and/or compositions of all air samples collected in the present study was not observed. [ABSTRACT FROM AUTHOR]

Technopolis, a plan for developing science parks in Japan, came into force in the early 1980's. Promoted by MITI, local governments and the general public held high hopes that this policy would promote the advancement of high-tech industries and provide increased employment possibilities for young graduates. The policy was developed against the backdrop of the post oil-crisis depression of the 1970's and changes in the Japanese economic structure. There were 26 regions designated by Technopolis. However, the Technopolis Act was terminated in 1998, and the program discontinued. The long depression following the bursting of the bubble economy, globalisation of the Japanese economy, and stagnation of private company investment were among the main factors contributing to the policy's failure. Subsequently, the government has developed a new policy that concentrates on promoting venture business and links between industries and universities. [ABSTRACT FROM AUTHOR]

Mold shrinkage of the colored plastics based on partially crystalline polymers is a well-known phenomenon and is assumed to be caused by pigment-induced crystallization of polymers. A systematic investigation has been carried out for a variety of organic pigments in polypropylene (PP) and polyethylene (HDPE) systems in order to clarify the correlation between the nucleation effect due to pigments and the mold shrinkage. Special attention was paid to the onset temperature of crystallization for both PP and HDPE systems as monitored by DSC (differential scanning colorimeter). In parallel, the crystallization rate was also studied in the isothermal process by depolarization method for PP while by dilatometry for HDPE systems. As a result, the mold shrinkage is found to become more significant as the onset temperature is higher and the crystallization rate is faster. This tendency is well correlated with the formation of small spherulites (diameter: less than 1 μm). These results lead us to conclude that the organic pigments in colored plastics serve as a nucleation agent for partially crystalline polymers to form small spherulites, causing the mold shrinkage to occur. [Copyright &y& Elsevier]

Compared to healthy subjects, patients with severe liver cirrhosis (LC) are reported to show lower values in the L-[1-[sup 13]C] phenylalanine breath test (PBT). We performed this test several times during the clinical course in two patients with severe liver cirrhosis (LC). Patient 1 was a 67-year-old woman with non-B, non-C LC and hepatocellular carcinoma (HCC) in the lateral hepatic segment. Because the patient wanted to receive nonsurgical treatment for HCC, intraarterial administration of zinostatin stimalamer was performed. The patient was hospitalized four times before her death from liver failure on December 20, 2000. During her clinical course, PBT was performed four times. Values for both the rate of hepatic phenylalanine oxidation (%[sup 13]C dose h[sup -1]) and %[sup 13]C cumulative excretion gradually decreased during her clinical course. Patient 2 was a 57-year-old man with hepatitis C virus (HCV)-positive LC. He was hospitalized seven times between December 1998 and his death on May 24, 2001. During his clinical course, PBT was performed four times. Values for both %[sup 13]C dose h[sup -1] and %[sup 13]C cumulative excretion decreased during his clinical course. We confirmed that PBT was useful for following the course of LC. [ABSTRACT FROM AUTHOR]

Non-equilibrium solute grain boundary segregation, a model for which was proposed by Xu, is discussed with particular focus on the influences of atomic diffusion and microstructure on grain boundary segregation. [Copyright &y& Elsevier]

Black tea adsorption on calcium carbonate has been studied for a new application to chalk powder as a method for surface modification. Black tea was used as a dyeing material for making brown powder materials. Chalk and pure calcium carbonate powders were reacted with the black tea aqueous solution from 30 min to 3 weeks at room temperature and the brown powders were obtained as the reaction products. The brown powders were characterised by X-ray diffraction, infrared spectroscopy, scanning electron microscopy with energy-dispersive spectroscopy, analysis of CHN and other elements and high performance liquid chromatography. Thermal study was also performed. The results indicate that the tea organic compounds are adsorbed on the surface of calcite crystals as a thin layer. They resembled tea scum-like materials transformed from at least two tea pigments such as theaflavins and thearubigins in the tea solution. In the adsorption process, the Ca(II) ions leached from calcite crystals mediated to form them. Possible tea adsorption mechanisms are also discussed in explaining the obtained results. [Copyright &y& Elsevier]

Background. Using a rat model of hepatectomy, we investigated whether the severity of hepatopathy could be quantitatively measured from changes in expiratory 13CO2 levels after intravenous administration of l-[1-13C]alanine.Materials and Methods. Under nembutal anesthesia, 20 mg/kg l-[1-13C]alanine was administered to rats via the femoral vein, and expiratory 13CO2 levels were measured for 15 min. Then, 30, 70, or 90% hepatectomy was performed. In the control group, simple laparotomy was performed. A breath test was conducted 20 min after laparotomy. We examined the correlation of total 13CO2 output (S) or single point 13CO2 level (SP) every 1 min for 15 min with liver weight/body weight (LW/BW) (%).Results. In the control group, the breath test graph showed a specific peak level about 3 min after administration, but in all groups undergoing hepatectomy, it did not show any peak level during measurement. The correlation coefficient between S12–15 after l-[1-13C]alanine administration and LW/BW was 0.902 (P < 0.0001). The correlation coefficient between SP7 after l-[1-13C]alanine administration and LW/BW was highest, 0.908 (P < 0.0001). The severity of hepatopathy could also be evaluated, with significant differences in S12–14 compared to control when the volume of resected liver was 30% or greater, but there was no significant difference between the groups undergoing 70 and 90% hepatectomy. However, the severity of hepatopathy could be evaluated, with significant differences in S15 and SP7 in all comparisons between groups.Conclusion. In the breath test with intravenously administered l-[1-13C]alanine, the severity of hepatopathy could be quantitatively evaluated in a short period by measuring S15 and SP7. [Copyright &y& Elsevier]

Background: Using a rat model of hepatectomy, we investigated whether the severity of hepatopathy could be quantitatively measured from changes in expiratory [sup 13] CO[sub 2] levels after intravenous administration of [1-[sup 13] C]galactose. Materials and Methods: Under nembutal anesthesia, 100 mg/kg [1-[sup 13] C]galactose was administered to rats via the femoral vein, and expiratory [sup 13] CO[sub 2] levels were measured for 60 min. Then, 30, 70 or 90% hepatectomy was performed. In the control group, simple laparotomy was performed. Breath test was conducted 20 min after laparotomy. We examined the correlation of total [sup 13] CO[sub 2] output (S) or single point [sup 13] CO[sub 2] level (SP) every 5 min until 30 min, and at 45 and 60 min with liver weight/body weight (LW/BW) (%). Results: In the control group, the breath test graph reached a plateau level, but in all groups undergoing hepatectomy a plateau level was not reached during measurement. The correlation coefficient between S[sup 30] after [1-[sup 13] C]galactose administration and LW/BW was 0.889 (p < 0.0001). The correlation coefficient between SP[sup 25] after [1-[sup 13] C]galactose administration and LW/BW was highest, 0.923 (p < 0.0001). Conclusion: In the breath test with intravenously administered [1-[sup 13] C]galactose, hepatopathy could be evaluated by measuring S[sup 30] and hepatopathy could be more accurately quantitatively evaluated by measuring SP[sup 25] over a short period.Copyright © 2000 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

• Fe-Ga alloy single crystals with 〈0 0 1〉 orientation grown by the Czochralski method acted as a magnetic core in the vibration power generation. • Electromagnetic induction occurred by inverse magnetostriction of the single crystal and the relative motion between magnets and a coil with the single crystal. • Alternating current power generated by the vibration was converted to direct current power for charging a battery. In order to apply magnetostrictive materials in vibration energy harvesting devices, large-sized ingots of Fe–Ga alloy single crystals exhibiting large magnetostriction were successfully grown using the Czochralski method. The performance of power generation using the 〈1 0 0〉-oriented crystal has been evaluated using simply designed devices. Specifically, influences of magnetic fields produced by inverse magnetostriction of the crystal and permanent magnets on power generation have been studied. The results indicated that power generation using the Fe-Ga alloy single crystal result from the inverse magnetostriction and the magnetic fluxes generated by electromagnetic inductions, which are caused by the cyclic relative motion between a coil and permanent magnets using the simple device frame structures. It was shown that the alloy single crystal acts as a magnetic core, and that the alternating current produced via vibration energy harvesting can be converted to direct current for charging batteries. [ABSTRACT FROM AUTHOR]

We examined the interaction between intertroop transfer and male dominance ranks in a wild population of Japanese macaques ( Macaca fuscata yakui) in Yakushima using data collected over 15 years. Intertroop transfer tended to maintain a linear, stable, and age-graded dominance rank order among nonnatal males irrespective of variation in troop size or composition. All males that joined a troop at the top of the rank order were prime adults. Among males joining at lower ranks, entry at the most subordinate position in the hierarchy was common. Males joining at lower ranks tended to join troops in which all other resident males were the same age or older. Adult males tended to join troops with few or no males. Young males tended to join troops with many resident males, and in which a relatively large proportion of males was other young ones. Intertroop transfer was responsible for most rank changes of resident males. The most common cause of males rising in rank was the emigration or death of a higher-ranking male. Males fell in rank most frequently as a result of a new male joining the troop at the top of the hierarchy. Rank reversals among resident males were rare. The cumulative effects of male transfers produce sociodemographic variation within a troop over time and sociodemographic diversity among troops in a local population. A key feature of intertroop diversity is that larger troops have a significantly greater proportion of young males than smaller troops. This diversity also creates the potential for intertroop variation in the severity of male competition and provides a range of options for transferring males. [ABSTRACT FROM AUTHOR]

Endoscopic ultrasonography was used for assessment of the extent of tumour invasion of the upper gastrointestinal (GI) tract, including analysis of submucosal tumour and detection of lymph-node metastasis. The normal oesophageal and gastric wall was depicted as five layers by endoscopic ultrasonography (EUS). The outer layer invaded by cancer was defined as the depth of tumour invasion. In 173 cases of oesophageal cancer, the depth of cancer invasion was diagnosed correctly in 88%. In 146 cases of gastric cancer, it was diagnosed correctly in 79%. In submucosal tumours of the GI tract, the site of tumour in the wall was diagnosed correctly in 99% and the histological type of tumour was predicted. EUS can also be used to detect small lymph nodes. According to the criteria, used in this study, EUS had a sensitivity of 84%, a specificity of 88% and an overall accuracy of 88% for detection of lymph-node metastases. [ABSTRACT FROM AUTHOR]

To realize a ring-type optical LAN which is affected little by node failure and cable failure and yet is flexible in wiring, a star-ring LAN with optical accessors within an optical cabinet is proposed. Although the present system has art advantage of realizing a high reliability with a one-fold ring configuration, the system is susceptible to signal interference in optical accessors. The effect of this signal interference on the transmission quality has been evaluated by means of the equation for the equivalent transmission loss by Tokura et al. However, this equation was derived in the case where the discriminating level is always set at the optimum value of the main signal and is not applicable to a general receiving system which is set at the optimum value of the composite signal. In this paper, a configuration of the star-ring LAN is proposed first and its advantages are described. Next, an approximate equation for calculation of the equivalent transmission loss is derived for an optical receiver system which 15 set such that the discrimination level is an optimum value of the composite signal. Further, by means of experiments, the relationship is derived between the degree of interference and the equivalent transmission loss, and the experimental values are compared with the computed results. It is demonstrated that the computed and experimental results agree well. Finally, by the use of the results, the optimum value of the transmission loss of the optical accessors in a star-ring LAN is determined. [ABSTRACT FROM AUTHOR]

A wild Japanese macaque troop decreased in size because the birth rate dropped and infant mortality increased. In the 1989 mating season, the last male left the troop, and the remaining two females joined a neighboring troop. This, the troop ceased to exist as an independent troop. a lower limit to troop size may exist, below which a troop cannot effectively defend its range, forcing the females to join a larger troop. [ABSTRACT FROM AUTHOR]

Biallelic neuroblastoma amplified sequence (NBAS) gene mutations have recently been identified to cause a reduction in its protein expression and a broad phenotypic spectrum, from isolated short stature, optic nerve atrophy, and Pelger–Huët anomaly (SOPH) syndrome or infantile liver failure syndrome 2 to a combined, multi-systemic disease including skeletal dysplasia and immunological and neurological abnormalities. Herein, we report a 34-year-old patient with a range of phenotypes for NBAS deficiency due to compound heterozygous variants; one is a SOPH-specific variant, p.Arg1914His, and the other is a novel splice site variant, c.6433-2A>G. The patient experienced recurrent acute liver failure until early childhood. Hypogammaglobulinemia, a decrease in natural killer cells, and optic nerve atrophy were evident from infancy to childhood. In adulthood, the patient exhibited novel phenotypic features such as hepatic cirrhosis complicated by portal hypertension and autoimmune hemolytic anemia. The patient also suffered from childhood-onset insulin-requiring diabetes with progressive beta cell dysfunction. The patient had severe short stature and exhibited dysmorphic features compatible with SOPH, intellectual disability, and epilepsy. NBAS protein expression in the patient's fibroblasts was severely low. RNA expression analysis for the c.6433-2A>G variant showed that this variant activated two cryptic splice sites in intron 49 and exon 50, for which the predicted consequences at the protein level were an in-frame deletion/insertion, p.(Ile2199_Asn2202delins16), and a premature termination codon, p.(Ile2199Tyrfs*17), respectively. These findings indicate that NBAS deficiency is a multi-systemic progressive disease. The results of this study extend the spectrum of clinical and genetic findings related to NBAS deficiency. [ABSTRACT FROM AUTHOR]

The purpose of this study was to evaluate the image quality in virtual monochromatic imaging (VMI) at 40 kilo-electron volts (keV) with three-dimensional iterative image reconstruction (3D-IIR). A phantom study and clinical study (31 patients) were performed with dual-energy CT (DECT). VMI at 40 keV was obtained and the images were reconstructed using filtered back projection (FBP), 50% adaptive statistical iterative reconstruction (ASiR), and 3D-IIR. We conducted subjective and objective evaluations of the image quality with each reconstruction technique. The image contrast-to-noise ratio and image noise in both the clinical and phantom studies were significantly better with 3D-IIR than with 50% ASiR, and with 50% ASiR than with FBP (all, p < 0.05). The standard deviation and noise power spectra of the reconstructed images decreased in the order of 3D-IIR to 50% ASiR to FBP, while the modulation transfer function was maintained across the three reconstruction techniques. In most subjective evaluations in the clinical study, the image quality was significantly better with 3D-IIR than with 50% ASiR, and with 50% ASiR than with FBP (all, p < 0.001). Regarding the diagnostic acceptability, all images using 3D-IIR were evaluated as being fully or probably acceptable. The quality of VMI at 40 keV is improved by 3D-IIR, which allows the image noise to be reduced and structural details to be maintained. The improvement of the image quality of VMI at 40 keV by 3D-IIR may increase the subjective acceptance in the clinical setting. [ABSTRACT FROM AUTHOR]

We have been involved in the research and development on H2 gas sensors utilizing a high proton affinity of the pyridyl rings integrated in organic pigments such as diketopyrrolopyrroles, peryleneimides, and copperphthalocyanes. Our sensors exhibit a remarkable increase in electrical conductivity by three orders of magnitude even under 0.01% H2. However, an anomalous phenomenon has been observed in the title compounds (pyridylimidazoperylene and pyridylimidazonaphthalene) that shows an additional increase in conductivity (i.e., reverse of the sensor current) as soon as H2 shuts off. The unusual effect has been investigated in the present investigation on the basis of the crystal structure, molecular orbital calculations, and UV-visible/IR spectra with special attention to the sign of the charge carriers before and after exposure to H2. As a result, the pyridylimidazole ring is found to be responsible for the unusual effect that involves two protonation sites. In addition, the charge carriers have been determined to be solely electrons in the build up of the sensor current; whereas both electrons and protons are involved in the decay process. The contribution of the proton conduction in the decay process prevails over the electron current, ending up with the reverse in sensor current. [ABSTRACT FROM AUTHOR]

Aortic and valvular calcification are well-known risk factors for cardio-cerebrovascular events in patients undergoing hemodialysis. We investigated the clinical impact of an angulated aorto-septal angle as a result of aortic elongation due to aortic calcification on cardio-cerebrovascular outcomes in patients undergoing hemodialysis. We investigated 306 patients (mean age 65.4 years, 68% male) who underwent pre-scheduled routine echocardiography between April and September 2018. The angle between the anterior wall of the aorta and the ventricular septal surface (ASA) was quantified. We determined aortic and mitral valve calcification scores based on calcified cardiac changes; the aortic and mitral valve scores ranged between 0–9 and 0–6, respectively. The primary endpoint was a composite including cardio-cerebrovascular events and cardio-cerebrovascular death. The mean duration of dialysis among the patients in this analysis was 9.6 years. The primary endpoint was observed in 54 patients during the observational period (median 1095 days). Multivariable Cox proportional hazards analyses identified left ventricular ejection fraction (per 10% increase: hazard ratio [HR] 0.67; 95% confidential interval [CI] 0.53–0.84, P = 0.001), left ventricular mass index (per 10 g/m2 increase: HR 1.14; 95% CI 1.05–1.24, P = 0.001), ASA (per 10 degree increase: HR 0.69; 95% CI 0.54–0.88; P = 0.003), and aortic valve calcification score (HR 1.15; 95% CI 1.04–1.26, P = 0.005) as independent determinants of the primary endpoint. Kaplan-Meier analysis showed a higher incidence of the primary endpoint in patients with ASA <119.4 degrees than those with ASA ≥119.4 degrees (Log-rank P < 0.001). An angulated aorto-septal angle is an independent risk factor for cardio-cerebrovascular events and cardio-cerebrovascular death in patients undergoing hemodialysis. [ABSTRACT FROM AUTHOR]

Metropolitan Tokyo is located directly above a subduction zone that has generated two megathrust earthquakes in the past 300 years. However, the timing of older megathrusts on this margin is poorly understood. In this study, we aim to constrain the timings of past megathrust earthquakes, using coastal stratigraphy, paleoecology, radiocarbon dating and archaeological records from coastal lowlands. An investigation of 13 boreholes in the southern coastal area of metropolitan Tokyo found evidence for 4 m of uplift in a 6000-year period. However, we found that net vertical displacement in the last 1000 years is approximately zero. Results suggest that preservation of usually ephemeral lagoon sediments occurred on three occasions in the past 1000 years, and radiocarbon dating results show that the timings of these preservation episodes are close to that of major historical earthquakes. We thus attribute the intermittent preservation of the ephemeral lagoon deposits to coseismic uplift caused by the megathrust earthquakes. The candidates of the megathrust earthquakes are events that took place in 1703 CE, the 13th century, and 878 CE. Since these events produced no net vertical displacement due to inter-seismic subsidence, we propose that earthquakes responsible for long-term uplift of this region took place prior to the 9th century. This research also demonstrates the value of preserved intertidal sediments as paleoseismological archives where net tectonic displacement is neutral. [ABSTRACT FROM AUTHOR]