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Objective High-dose melphalan and autologous stem cell transplantation (ASCT) therapy for AL amyloidosis are now associated with reduced mortality based on the application of strict criteria. However, there is no long-term evidence concerning the performance of induction therapy with newer agents, such as bortezomib or daratumumab. Concerns regarding long-term relapse despite treatment with ASCT exist, and missing the opportunity to perform ASCT might occur if induction proves to not be efficacious and cardiac amyloidosis progression deprives the patients of a chance to receive ASCT. We herein report good amyloid control by vincristine, doxorubicin, and dexamethasone (VAD) induction therapy and argue the importance of induction therapy before ASCT. Methods We compared patients who underwent VAD induction and ASCT (VAD+ASCT) with patients who underwent frontline ASCT in our hospital. Patients A total of 26 patients with histologically proven AL amyloidosis were included (18 in the VAD+ASCT group and 8 in the frontline ASCT). Results In the VAD+ASCT group, the 10-year overall survival and renal response rates were 82% and 43%, respectively. The renal response rate at two years in the VAD+ASCT group was significantly better than that in the frontline ASCT group. Although there was no significant difference in the survival rates between the two groups, the time to next treatment or death was significantly better in the VAD+ASCT group than in the the frontline ASCT group. Acute kidney injury was the most frequent reason for failure to receive two courses of VAD, and early mortality was mainly due to gastrointestinal complications. Conclusion Considering that only those who underwent 2 courses of VAD experienced a 10-year renal response, induction therapy was deemed to be directly related to the long-term control of AL amyloidosis.

Interstitial and intracavitary ultrasound applicators had been developed and studied for minimally invasive treatments (MIT). However, the acoustic outputs are limited by the small-size PZT. We therefore studied the acoustic waveguide (AW) applicator which enables the use of a large-size PZT, and we aimed to advance AW applicators towards thermal ablation applications.Double parabolic reflectors wave-guided ultrasonic transducer (DPLUS) was introduced which has two parabolic reflectors for enhancing the acoustic output. Theoretical modeling was conducted for optimizing the DPLUS thin waveguide.Modeling results showed that optimal [Formula: see text] (thin waveguide radius/wavelength) can be found and the optimal [Formula: see text] depends on the excitable vibration amplitude in the thin waveguide. A local optimal [Formula: see text]=0.2392 was considered the best choice, which results in the optimal frequency of 2.2 MHz at the radius [Formula: see text] of 0.6 mm. To verify this optimal frequency, experiments under two working frequencies of 1.0282 MHz and 2.2579 MHz were conducted. Temperature rise curves in the chicken breast tissue showed good agreements between experiments and modeling results, which proved the effectiveness of the modeling. In addition, experiments showed an ablated area with a diameter of 1.03±0.12 mm under continuous excitation of 2.2579 MHz and 5 s.The developed DPLUS advanced the AW applicators towards thermal ablation applications.This study provides the evidence for recognizing AW applicators as a technique for thermal ablation.

Austenitic stainless steels including type SUS304 of the Japanese Industrial Standard (JIS), which is similar to ASME Type 304 SS, are candidate materials for the various facilities of high-pressure gaseous hydrogen, such as a hydrogen station containers and piping. To prevent the hydrogen penetration into SUS304, we developed the passivation films mainly composed of chromium oxide and investigated the hydrogen barrier properties using the gas permeability test and the slow strain rate tensile (SSRT) test. The passivation films with a maximum thickness of 300 nm was formed on the surface of hot-rolled SUS304 (Ni equivalent 22.4) by a series of wet processing steps such as electro-polishing, chemical oxidation, cathode precipitation of chromium and passivation immersion. Cross-sectional views of TEM observation suggested that the film was amorphous like structure including many independent voids with a size of 10 to 20 nm. The hydrogen gas permeability test was performed in a hydrogen gas pressure of 400 kPa at 573 K, 673 K, and 773 K. The estimated hydrogen transmittance of SUS304 substrate with passivation coating was 2.8 × 10−13 mol / (m · s · Pa) at 773 K, while that without passivation coating was 2.2 × 10−11 mol / (m · s · Pa) at 773 K. SSRT test was performed in 110 MPa hydrogen and nitrogen gas atmosphere at room temperature, and strain rate was 4.17 × 10−5 s−1. The fracture surface of the specimen without passivation coating showed brittle like and relative reduction rate (RRA) was 0.61. On the other hand, the fracture surface of the specimen with passivation coating showed typical ductile like dimple structure and RRA was 0.88. As the passivation films did not peel, adhesion between passivation film and SUS304 surface seems to be well. From these considerations, we anticipate the developed passivation film can inhibit hydrogen embrittlement of SUS304.

Morphology of nova ejecta is essential for fully understanding the physical processes involved in nova eruptions. We studied the 3D morphology of the expanding ejecta of the extremely slow nova V1280 Sco with a unique light curve. Synthetic line profile spectra were compared to the observed [O III] 4959, 5007 and [N II] 5755 emission line profiles in order to find the best-fit morphology, inclination angle, and maximum expansion velocity of the ejected shell. We derive the best fitting expansion velocity, inclination, and squeeze as $V_{\rm exp} = 2100^{+100}_{-100}$ \,km\,s$^{-1}$, $i = 80^{+1}_{-3}$ deg, and $squ = 1.0^{+0.0}_{-0.1}$ using [O III] line profiles, and $V_{\rm exp} = 1600^{+100}_{-100}$ \,km\,s$^{-1}$, $i = 81^{+2}_{-4}$ deg, and $squ = 1.0^{+0.0}_{-0.1}$ using [N II] 5755 line profile. A high inclination angle is consistent with the observational results showing multiple absorption lines originating from clumpy gases which are produced in dense and slow equatorially focused outflows. Based on additional observational features such as optical flares near the maximum light and dust formation on V1280 Sco, a model of internal shock interaction between slow ejecta and fast wind proposed for the $\gamma$-ray emission detected in other novae seems to be applicable to this extremely slow and peculiar nova. Increasing the sample size of novae whose morphology is studied will be helpful in addressing long-standing mysteries in novae such as the dominant energy source to power the optical light at the maximum, optical flares near the maximum, clumpiness of the ejecta, and dust formation., Comment: 21 pages, 8 figures, accepted for publication in ApJ

We report a case of superinfection of liver cysts caused by Candida albicans and Staphylococcus lugdunensis in a patient with autosomal dominant polycystic kidney disease. A 69-year-old man with chief complaints of headache and blurred vision was admitted to the former institution for the evaluation of suspected temporal arteritis. He was prescribed oral prednisolone (55 mg/day) as a preemptive treatment; however, he became febrile and presented with bilateral flank pain during prednisolone tapering. Blood culture revealed fungemia as a result of Candida famata infection; thus, micafungin treatment was started. Thereafter, recrudescence of the C-reactive protein level ensued. Then a cyst infection as visualized on magnetic resonance imaging led to cyst aspiration sclerotherapy. Cyst aspirate culture revealed the presence of C. albicans in one of the cysts and S. lugdunensis in two of the cysts. The patient was clinically stabilized with an additional 3 weeks of antibiotic and antifungal drug administration.

High intensity focused ultrasound therapeutics are widely used to noninvasively treat various types of primary tumors and metastasis. However, ultrasound penetration depth is shallowed with increasing frequency which limits the therapeutic accuracy for deep tissues. Although acoustic waveguides are commonly inserted into tissue for localized therapy, powerful ultrasound delivery is difficult. Here, we invent double-parabolic-reflectors acoustic waveguides, where high-power ultrasound emission and large mechanical vibration enhance the therapeutic efficiency. High-energy-density ultrasound with around 20 times amplification by two parabolic reflectors propagates through the thin waveguide between 1 to 2 MHz, and wideband large mechanical vibration at the waveguide tip from 1 kHz to 2.5 MHz accelerates the therapeutics. This fundamental work serves as a milestone for future biomedical applications, from therapeutics to diagnostics. Since the high-power ability at high frequencies, our waveguide will also open up new research fields in medical, bio, physics and so on.

Orofaciodigital syndrome 1 (OFD-1) is a rare, X-linked, dominantly inherited disorder caused by an OFD1 mutation that can cause polycystic kidneys. A 37-year-old woman on hemodialysis therapy was admitted to our hospital for trans-catheter arterial embolization therapy for enlarged polycystic kidneys. Lobulated tongue and brachydactyly were noticed, prompting an OFD1 sequencing analysis. Sequencing revealed a causal four-base-pair deletion in exon 13, both in the patient and in her mother, whose renal function had been retained. The peripheral leukocyte X chromosome inactivation pattern was skewed in the patient but not in her mother, suggesting some role in their phenotypic difference.

Scanning Kelvin probe force microscopy (SKPFM) in nitrogen atmosphere, which can detect the variation of surface contact potential difference (CPD) caused by hydrogen ingress, was used to investigate hydrogen distribution in thermally hydrogen-precharged austenitic stainless steel with strain-induced martensite. Furthermore, the evolutions of hydrogen concentrations in lattice and trapping site are calculated by McNabb–Foster model. Results show that work function of the material is decreased by hydrogen. Hydrogen concentration evolution simulated by the model is consistent with that measured by SKPFM. Particularly, hydrogen is proved to be trapped at the phase boundary between austenite and martensite by SKPFM.

Pb(Zr1−x Hf x )O3 (x = 0, 0.1, 0.2, 0.5, 1) films were fabricated on Pt/Ti/SiO2/Si substrates using a chemical solution deposition process in this study. The effect of the Zr/Hf ratio on energy-storage performance was evaluated based on the measurement of P–E hysteresis loops. It is shown that the maximum polarization and the recoverable energy density decrease with the increase of the Hf concentration. The energy efficiency of the Hf-contained films is close to each other but higher than the PbZrO3 film. As a result, the Pb(Zr0.9Hf0.1)O3 film achieved in this work exhibited the highest recoverable energy density of 11.3 J cm−3 and a larger energy efficiency of 55% at 800 kV cm−1, exceeding those of either PbZrO3 or PbHfO3 single-component film. This enhancement was related with the size and homogeneity of the crystal grains.

Objective: To treat tissues that are difficult to access, ultrasound based minimally invasive treatment (MIT) is promising. However, high-power ultrasound delivery through waveguides had been difficult which can increase treatment duration. It is our effort to design the waveguide that can transmit powerful ultrasound. Methods: The waveguide with two parabolic reflectors was proposed by us to produce high-energy-density plane wave. Use of flexible and long thin waveguide was demonstrated here. Results: Double Parabolic refLectors wave-guided high-power Ultrasonic tranSducer (DPLUS) including a φ 1 mm ×1 m Nitinol thin waveguide was fabricated. It was shown that high-power ultrasound between 1 to 2 MHz can be propagated through the thin waveguide. Low-loss waveguide material was confirmed to be important to enhance output. As ultrasound is transmitted into working medium, energy mainly flows from the side surface. Temperature of target soft tissue was demonstrated to drastically increase by 10 degree in 30 seconds. Conclusion: The developed DPLUS makes high-power ultrasound transmission in long and flexible thin waveguide possible. Significance: The concept of DPLUS for delivering high-power ultrasound is powerful in the field of Ultrasonics.

A 17-year-old boy was referred to our institution for a re-evaluation of congenital nephrogenic diabetes insipidus. A water restriction test revealed no urine concentration or volume reduction and a subsequent pitressin test revealed a lack of an anti-diuretic response. Nephrogenic diabetes insipidus was confirmed, and the patient was treated using trichlormethiazide 4 mg, indomethacin 175 mg, and desmopressin 20 μg. His blood pressure and weight were not controlled owing to polydipsia and polyuria secondary to acquired excessive water drinking behavior. Repeated admissions for weight control were necessary and despite consultation with a psychiatrist for his obsessive water drinking behavior, he had end-stage renal failure after 30 years of treatment. Genetic testing revealed AVPR2 mutation (c. T866C: p. L289P) that had previously been reported as a pathogenic mutation. His excessive drinking behavior persisted, leading to hyponatremia even after initiation of hemodialysis. There was also difficulty in achieving body weight control, which was managed by repeated admissions with restriction of water intake, being the mainstay of management.

Among the various fuel cell electrocatalysts studied for the cathodic oxygen reduction reaction and anodic fuel oxidation reaction, platinum-based materials have attracted much attention over the past decade because of their high activity for both reactions. However, Pt-based catalysts suffer from several problems, such as their high cost, low abundance, and low long-term stability. Bimetallic Pt-based nanostructures with a core–shell structure have been considered as candidate cathode catalysts for polymer electrolyte fuel cells. In this study, a Pt/Pd/C catalyst was successfully synthesized by the hydrogen-sacrificial protection method to improve the performance of a membrane electrode assembly. Furthermore, on the basis of electrochemical spectroscopic findings, it was shown that control of the surface structure is an effective strategy to improve the cell performance of the Pt/Pd/C catalyst.

This research presents double-parabolic-reflector wave-guided ultrasonic transducers in order to realize wideband (0–2.5 MHz), multiharmonic mode excitations (over 20 modes), and to obtain large mechanical/acoustic outputs. The double-parabolic-reflector mechanism serves as a horn structure at low frequencies and acoustic-focusing structure at high frequencies to enhance the energy density of the incident ultrasound. Upon combining simulation and experimental methods, we examined and verified the basic performance and working mechanisms of the double-parabolic-reflector waveguides: multimode excitation belongs to the harmonic modes from the thin waveguide. At the megahertz range near the thickness mode of the piezoelectric element (PZT), energy density of the incident ultrasound is enhanced by double-parabolic reflections, and the amplification ranges 10 to $40\times $ between 1 and 2.5 MHz. At burst excitations, the amplification performance is independent of the length of the thin waveguide. Compared with conventional Langevin transducers and high-intensity focused ultrasound (HIFU) transducers, our transducers possess a wide working frequency with large mechanical/acoustic outputs and large vibration velocity amplification. By introducing these new features, our proposed method is a promising candidate for examining basic physics parameters, such as frequency dependence, in the fields of medicine, biology, industry, etc.

A combination of scanning Kelvin probe force microscopy (SKPFM) and electron backscatter diffraction (EBSD) has been applied to investigate the hydrogen release from thermally hydrogen-precharged type 304 austenitic stainless steel (γ-SS). The evolution of contact potential difference (CPD) well exhibits the evolution of hydrogen concentration in the near surface area. It is found for the first time that the hydrogen diffusivity in γ-SS depends on the crystallographic orientation. The hydrogen diffusion out from (001) and (101) grains is faster than from (111) grains while no obvious difference is found between (001) and (101) grains.

SSRT and fatigue life tests of SUS301 austenitic stainless steel were performed to examine the effect of hydrogen on the mechanical properties. Ni content of SUS301, 6.00–8.00 mass%, is lower than that of SUS304 in JIS standard for austenitic stainless steels. In the case of SSRT tests, specimens with and without hydrogen charging were tested in laboratory air at room temperature (R.T.), −45 °C, and −80 °C. The 0.2% offset yield strength (Ys) of the hydrogen charged specimens was less than 300 MPa in the tested temperature range. The tensile strength (Ts) and total elongation (El) of hydrogen charged specimens decreased remarkably. With decreasing testing temperature, fracture surface facet of the hydrogen charged specimens became dominant. Therefore, the effect of hydrogen on the tensile properties of SUS301 is supposed to be large. Specimens with and without hydrogen charging were fatigued in laboratory air at R.T., and specimens without hydrogen charging were fatigued in 100 MPa hydrogen gas atmosphere at R.T. Number of cycles (Nf) at finite fatigue life region of the hydrogen charged specimens and of the specimens tested in hydrogen gas were two orders shorter than that of the specimens tested in air. However, the finite fatigue life region of the hydrogen charged specimens and the specimens tested in hydrogen gas showed a different profile. Additionally, ferrite equivalents of all fatigue tested specimens and fatigued fracture surface morphology suggested the fatigue fracture mechanism between the hydrogen charged specimens tested in air and the non-charged specimens tested in 100 MPa hydrogen gas seems to be different. Therefore, further investigations are required to clear this difference.

Three types of strength tests, slow strain rate tensile (SSRT), fatigue life, and fatigue crack growth (FCG) tests, were performed using six types of aluminum alloys, 5083-O, 6061-T6, 6066-T6, 7N01-T5, 7N01-T6, and 7075-T6, in air and 115 MPa hydrogen gas at room temperature. All the strength properties of every material were not deteriorated in 115 MPa hydrogen gas. In all the materials, FCG rates were lower in 115 MPa hydrogen gas than in air. This was considered to be due to a lack of water- or oxygen-adsorbed film at crack tip in hydrogen gas. In 5083-O, 6061-T6 and 6066-T6, relative reduction in area (RRA) were remarkably higher in 115 MPa hydrogen gas than in air. These differences were attributed to a hydrostatic pressure produced in 115 MPa hydrogen gas. In contrast, in 7N01-T5, 7N01-T6 and 7075-T6, the values of RRA in 115 MPa hydrogen gas were nearly the same as those in air. Observation of fractured specimens inferred that the degree of such a hydrogen-induced improvement was determined by the fracture mode (e.g. cup-and-cone or shear fracture), which is dominated by the microstructure morphology.

The effect of hydrogen on the deformation and fracture behavior in pure Cu, pure Ni and Cu–Ni alloy was studied via tensile tests of H-charged, smooth and circumferentially-notched specimens at room temperature (RT) and 77 K. Hydrogen-diffusion properties were determined by the desorption method. To obtain a uniform hydrogen concentration in the H-charged specimens, specimens were exposed to 100-MPa hydrogen gas at 543 K for 200 h, based on the determined hydrogen diffusivity. In tensile tests of smooth pure Ni and Cu–Ni alloy specimens at RT, common hydrogen effects were detected, namely, an increase in yield and flow stresses — a hardening effect; and a ductility loss that was accompanied by a change in fracture surface from ductile to brittle feature — an embrittling effect. With regard to the embrittling effect, the pure Ni and Cu–Ni alloy showed different fracture-surface morphologies at RT; the pure Ni showed an intergranular (IG) surface and the Cu–Ni alloy surface was flat. However, a number of IG cracks were detected beneath the fracture surfaces on the smooth Cu-Ni alloy. The tensile tests of the H-charged smooth specimens at 77 K yielded an IG surface for the pure Ni and a ductile fracture surface with dimples in the Cu–Ni alloy. In contrast, tensile tests of the H-charged, notched specimens at RT demonstrated clear IG fractures for the pure Ni and Cu–Ni alloy. These facts indicate that IG cracking was the first step in the embrittling process for the pure Ni and Cu–Ni alloy, and IG cracking was accompanied by a large plastic deformation that formed the flat surface (unclear IG surface) for the smooth Cu–Ni alloy. Considering that the HE of both pure Ni and Cu–Ni alloy was related to IG cracking, possible mechanisms were discussed and tensile tests performed at 77 K suggested two possibilities: (I) interaction between hydrogen-moving dislocation is more important in the HE process of the Cu-Ni alloy compared to the pure Ni; (II) hydrogen transportation towards grain boundaries are required to cause the IG fracture in the Cu-Ni alloy.

Hydrogen barrier coating is a promising technology for preventing hydrogen embrittlement in metals. In this study, characterizations of hydrogen barrier films coated on surfaces of austenitic stainless steel, SUS304 of the Japanese Industrial Standard (JIS), by wet coating processes applied electro-polishing and chemical oxidation method are carried out using cross-sectional transmission electron microscopy (TEM) analyses, and then slow strain rate tensile (SSRT) tests are performed in 1.1MPa hydrogen and nitrogen gases at room temperature. The hydrogen barrier films show 200–300nm total thickness of compositionally modulated Chromium oxide dense layer. The SSRT results reveal that both the elongation and reduction of area are decreased in hydrogen gas compared with those in nitrogen gas for the non barrier-coated specimens but no significant differences appear for the barrier-coated specimens. The fracture surface of the non barrier-coated specimen shows quasi-cleavagy cracking in hydrogen gas while that of the barrier-coated specimen shows only ductile dimple fracture in hydrogen gas, indicating that the coated films effectively prevent the hydrogen embrittlement of SUS304 stainless steel in hydrogen.

Aim Polycystic liver disease (PLD) occasionally leads to abdominal pain, distention, and discomfort due to massively enlarged cysts. Patients with small number of large liver cysts, which correspond to Gigot's classification type 1 PLD, are said to be good candidates for treatment by aspiration sclerotherapy. However, there is a 21% reported recurrence rate of an enlarged cyst. A rule to predict outcome of aspiration sclerotherapy is explored in this study. Methods Autosomal dominant polycystic kidney disease or isolated polycystic liver disease patients receiving minocycline hydrochloride cyst aspiration sclerotherapy for their symptomatic PLD were retrospectively analyzed by medical records. Cysts’ volume changes between before and one year after treatment were calculated using CT images. Mean CT values of the largest planes of cysts were also calculated. Specific gravity and other laboratory parameters of aspirated cysts fluid were also retrospectively investigated. Results In total, 12 patients were selected and 21 cysts receiving aspiration sclerotherapy were analyzed. Mean CT values more than 13.34 HU were predictive for one-year non-sustainability of a cyst volume of less than 30% compared with the volume prior to the therapy. Specific gravity had good positive correlation with mean CT value and other laboratory parameters indicating exudative property were also high in recurred cysts. Conclusions Cysts’ CT values may become an aid in appropriate selection of therapy modality in symptomatic polycystic disease patients by telling cysts that are resistant to aspiration sclerotherapy.

In this study a technique is described that enables the ceramic coating on stainless steel with good adhesion via a simple application, drying, and calcination process using an aqueous paste based on clay mineral dispersion. On the basis of this technique, the cross-sectional microstructure of the interfacial area between the clay-derived ceramic coating and stainless steel was analyzed in detail, and the mechanism behind the coating of the ceramic layer with good adhesion was investigated. From the results of elemental mapping using transmission electron microscopy, scanning transmission electron microscopy-energy dispersive X-ray spectroscopy, and X-ray photoelectron spectroscopy, it was discovered that iron from the stainless steel diffuses into the ceramic coat, and the specific passivation film of the stainless steel integrates with the clay mineral-derived ceramic coating during the calcination, revealing that such chemical reactions are effective in promoting strong adhesion between the coat and stainless steel surface. In addition, a unique advantage of using this coating method was discovered in that even if there are small natural scratches on the raw stainless steel, the clay mineral paste can be used to cover these, resulting in a good, flat surface.

In Japan, with regards to the widespread commercialization of 70 MPa-class hydrogen refueling stations and fuel cell vehicles, two national projects have been promoted on both the infrastructure and the automobile sides. These projects have been promoted to establish the criteria for determining hydrogen compatibility of materials and to expand the usable materials for high-pressure hydrogen environment. For these projects, establishing test methods to evaluate the hydrogen compatibility of materials is one of the most important tasks. This paper describes the status of common standardization of testing methods. Two projects share a common database for the testing results, which is currently put to practical use.

A high pressure material testing system (max. pressure: 140 MPa, temperature range: −80 ∼ 90 °C) was developed to investigate the testing method of material compatibility for high pressure gaseous hydrogen. In this study, SSRT and fatigue life test of JIS SUS304 and SUS316 austenitic stainless steel were performed in high pressure gaseous hydrogen at room temperature, −45, and −80 °C. These testing results were compared with those in laboratory air atmosphere at the same test temperature range. The SSRT tests were performed at a strain rate of 5 × 10−5 s−1 in 105 MPa hydrogen gas, and nominal stress-strain curves were obtained. The 0.2% offset yield strength (Ys) did not show remarkable difference between in hydrogen gas and in laboratory air atmosphere for SUS304 and SUS316. Total elongation after fracture (El) in hydrogen gas at −45 and −80 °C were approximately 15 % for SUS304 and 20% for SUS316. In the case of fatigue life tests, a smooth surface round bar test specimen with a diameter of 7 mm was used at a frequency of 1, 0.1, and 0.01 Hz under stress rate of R = −1 (tension-compression) in 100 MPa hydrogen gas. It can be seen that the fatigue life test results of SUS304 and SUS316 showed same tendency. The fatigue limit at room temperature in 100 MPa hydrogen gas was comparable with that in laboratory air. The room temperature fatigue life in high pressure hydrogen gas appeared to be the more severe condition compared to the fatigue life at low temperature. The normalized stress amplitude (σa / Ts) at the fatigue limit was 0.37 to 0.39 for SUS304 and SUS316 austenitic stainless steels, respectively.

A 48-year-old woman was admitted to our hospital to undergo evaluation for fatigue, severe weight loss, and nephrotic range proteinuria. Light microscopy of a renal biopsy specimen revealed class III (A) lupus nephritis, while immunofluorescence and electron microscopy only showed sparse immune deposits with findings that were not typical of lupus nephritis. Computed tomography revealed a mass in the anterior mediastinum, which was resected. The examination of the surgical specimen revealed type A noninvasive thymoma. In combination with thymomectomy, postoperative steroid therapy achieved the prompt remission of lupus nephritis. In this patient, thymoma-related autoimmunity may have contributed to the exacerbation of lupus nephritis.

Patients with autosomal dominant polycystic kidney disease and polycystic liver disease (PLD) often have elevated serum levels of alkaline phosphatase (ALP) and gamma-glutamyl transpeptidase (GGT). Ursodeoxycholic acid (UDCA) is used to treat biliary tract diseases, but its effect on PLD remains unclear. UDCA was administered for 1 year at a dose of 300 mg daily to seven PLD patients with elevated ALP or GGT levels who were selected for this treatment by experienced clinicians. Laboratory data and liver volumes were compared among three time points: 1 year before UDCA treatment, at the start of UDCA therapy, and 1 year after the start of therapy. Median GGT did not show a significant change between 1 year before UDCA (180 IU/L) and the start of UDCA therapy (209 IU/L), but it decreased significantly to 98 IU/L after 1 year of UDCA therapy (P = 0.015 vs. the start of therapy). ALP showed a significant increase from 1 year before UDCA (456 IU/L) to the start of UDCA therapy (561 IU/L), and then decreased significantly after 1 year of UDCA therapy (364 IU/L). Median liver volume did not show any significant changes among these three time points of assessment. UDCA may be effective for reducing biliary enzyme levels and inhibiting the growth of liver cysts in patients with PLD.

Detecting hydrogen distribution at micro- and nano-scale is important for understanding the mechanisms of hydrogen embrittlement in metals. In this study, scanning Kelvin probe force microscopy (SKPFM), which can detect the variation of surface contact potential difference (CPD) caused by hydrogen, is applied to investigate the hydrogen distribution and evolution in thermally hydrogen-charged (HC) super duplex stainless steel. The SKPFM observations reveal that the CPD distribution becomes nonuniform in both the ferrite and austenite phases after hydrogen charging, implying that hydrogen distributes heterogeneously in the two phases. The average CPDs of both the ferrite and austenite phases are significantly decreased and the difference of CPD between two phases reaches a maximum shortly after thermal hydrogen-charging. The average CPDs of both the ferrite and austenite phases recover and the difference of CPD between two phases is decreased upon release of the hydrogen. These results are discussed in terms of the hydrogen outgasing behavior and the difference of hydrogen diffusivity in the two phases.

We report a Japanese woman with systemic rheumatoid vasculitis (SRV) complicated by necrotizing crescentic glomerulonephritis (NCGN). Rheumatoid arthritis first occurred at the age of 19 years, followed by interstitial pneumonia, hepatitis, rheumatoid nodules, mononeuritis multiplex, and hypocomplementemia in chronological order. At the age of 51 years, rapidly progressive renal failure occurred with nephrotic proteinuria, and NCGN with subepithelial deposits was revealed by renal biopsy. Severe destructive changes of multiple joints and scleritis were detected, but anti-neutrophil cytoplasmic antibody was negative on enzyme-linked immunosorbent assays and indirect immunofluorescence. SRV was diagnosed due to involvement of multiple extra-articular organs. An anti-interleukin (IL)-6 receptor antibody (tocilizumab) was started at dosage of 280 mg (8 mg/kg) monthly. After 18 months, her serum creatinine decreased from 1.7 to 1.3 mg/dL, and urinary protein excretion declined from 5.2 to 1.2 g daily. Tocilizumab may be a therapeutic option for SRV associated with NCGN.

In order to clarify the contribution of dislocation‒hydrogen interaction on the hydrogen embrittlement (HE) of pure Ni and of Cu‒55 wt% Ni binary alloy, slow strain rate tensile (SSRT) tests were conducted at room temperature (RT) and at 77 K on hydrogen-precharged specimens. Regarding the SSRT test at RT, hydrogen increased the flow stress and induced intergranular fracture in both pure Ni and Cu–Ni alloy. Furthermore, based on scanning transmission electron microscopy investigations, it was suggested that the evolution of dislocation structures had been enhanced by hydrogen, but only in the case of pure Ni. At 77 K, the ductility of pure Ni was degraded by hydrogen, whereas that of Cu–Ni alloy was not. The difference in temperature dependence of the dislocation‒hydrogen interaction between pure Ni and Cu–Ni alloy was discussed, based on the previously proposed HE mechanisms.

Ultrasound imaging transducers possess serious trade-off design in performances between the penetration depth and the imaging resolution, which loses detection accuracy of diseases in large and deep-depth organs. To ease this problem, this study combines a piezoelectric transducer with the waveguide technique to realize invasive imaging. However, conventional configurations with waveguides suffer from low input energy or low energy transmission efficiency, thus leading to the proposal of the double-parabolic-reflector focusing and guiding mechanism to achieve high power transmission. In this letter, three different physical configurations of waveguides for ultrasound propagation are compared in terms of acoustic pressure, power, and propagation modes. The proposed mechanism with double parabolic reflectors enhances the acoustic pressure at the tip of the cylindrical waveguide to over 17 times compared to the conventional mechanism and increases the total power flow to around 213 times. Due to the superior performances in powerful ultrasound transmission, the proposal is expected to widen the applications in ultrasound imaging, ultrasound therapeutics, and ultrasound microscopy.

Lead-free piezoelectric (Bi0.5Na0.5)TiO3 (BNT) thin films were prepared by chemical-solution deposition. Perovskite single-phase BNT and Mn-doped BNT thin films were successfully fabricated at 600–700 °C on Pt/TiOx/SiO2/Si substrates. The BNT thin films fabricated from a stoichiometric BNT precursor solution showed considerable surface roughness and poor ferroelectric properties. The surface roughness of the films was improved by incorporating a controlled excess of volatile elements in the composition. In addition, the leakage current density of the BNT films, especially in the high-applied-field region, was reduced by doping with a small amount of Mn. Moreover, Mn doping markedly improved the ferroelectric properties of these films. Thus, 0.5 and 1.0 mol% Mn-doped BNT thin films exhibited well-shaped ferroelectric polarization (P)–electric field (E) hysteresis loops at room temperature. Furthermore, the synthesized BNT films showed a typical field-induced strain loop, and the effective d33 values were estimated at 41–60 and 39 pm/V for the BNT thin films with and without Mn doping, respectively.

Lead-free ferroelectric (Bi0.5Na0.5)TiO3(BNT) thin films were prepared by chemical solution deposition. BNT and Mn-doped BNT precursor thin films crystallized in the perovskite single phase at 700 °C on Pt/TiOx/SiO2/Si substrates. The leakage current density of the perovskite BNT films, especially in the high applied field region, was reduced by doping with a small amount of Mn. Also, Mn doping markedly improved the ferroelectric properties of the films. 0.5 and 1.0 mol% Mn-doped BNT thin films exhibited well-shaped ferroelectric polarization (P) electric field (E) hysteresis loops at room temperature. Furthermore, the 1 mol% Mn-doped BNT films showed a typical field-induced strain loop, and the effectived33values were estimated to be about 60 pm/V.