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A 53-year-old man on hemodialysis suffered from short bowel syndrome after subtotal colectomy and partial resection of the small intestine. Laboratory tests showed multiple electrolyte disorders and enlarged sodium and chloride ion (Cl(−)) gaps despite treatment with large volume of sodium chloride replacement via central venous infusion. Blood gas analysis showed slightly high bicarbonate ion levels and metabolic alkalosis was suspected, which is uncommon in end stage kidney disease. The measurement of electrolytes in feces from ileostomy showed relatively high Cl(−) excretion. The loss of Cl(−) to feces may have caused the metabolic alkalosis; thus, additional Cl(−) replacement may have been necessary.

A 47-year-old man presented with severe hypokalemic paralysis and respiratory failure. A large amount of potassium was administered along with providing intensive care, and his condition improved. Hypokalemia was attributed to increased urinary potassium excretion. A kidney biopsy was performed to make a definitive histological diagnosis. It revealed acute tubulointerstitial nephritis (TIN). After the diagnosis, prednisolone was administered, and the TIN gradually improved. From the clinical course and laboratory findings, the TIN was presumed to be an autoimmune disorder. Further specific autoantibody tests were positive for anti-mitochondrial antibody (AMA), which has been gaining increasing attention in regard to TIN. In addition, all previous cases of TIN associated with AMA have affected females. The detailed pathogenetic mechanisms are as yet unclear and require further investigation.

Background and Aims Transcatheter aortic valve implantation (TAVI) is an effective treatment for aortic stenosis (AS) in high-risk patients, such as those who are suffering ESRD on hemodialysis. We previously showed that long-term survival of hemodialysis patients underwent TAVI was lower than that of non-hemodialysis patients but was comparable with that of hemodialysis patients treated with surgical aortic valve replacement. It is known that hemodialysis is a high risk of the bioprosthetic structural valve deterioration (SVD) after valve replacement surgery. Consistently, higher incidence of re-TAVI operation due to restenosis was observed in hemodialysis patients than in non-hemodialysis patients (Odds ratio [OR]:29.4, p In this study, we aimed to identify predictors of SVD in post-TAVI hemodialysis patients. Method From April 2012 to December 2015, 24 patients on hemodialysis were selected to receive TAVI for the treatment of AS in Osaka University Hospital. Demographic and laboratory data at pre- and post-operation were compared between patients who underwent re-TAVI (valve-in-valve) (SVD group) and those who did not (no-SVD group). Results During the follow-up period (maximal 7 years), 4 out of 24 patients required re-TAVI (valve-in-valve) operation because of SVD. Although serum calcium, phosphate, and PTH levels before operation were comparable between SVD group and no-SVD group, serum magnesium levels were significantly lower in SVD group. Lower serum magnesium quartiles were associated with a higher risk of SVD (OR 5.83, p=0.036). While effective orifice area index (EOAi) of aortic valve at 1 week after TAVI was similar between the 2 groups, EOAi improvement at 1 month was significantly poorer in SVD group than in no-SVD group (1.70 ± 0.15 vs 2.50 ± 0.56, p = 0.049). Conclusion The higher incidence of re-TAVI due to SVD in hemodialysis patients could be related with pre-operative low magnesium levels and with one-month post-operative low EOAi improvement. This was a single center observational study, and the number of patients was very small so that our data would not be necessarily applicable for every patient. Further investigation is necessary for identifying a prognostic predictor of SVD, which will lead to the prevention of unfavorable re-operation in hemodialysis patients.

Selectivity index (SI) of proteinuria, calculated using the clearance ratio of immunoglobulin G to transferrin, predicts the response to glucocorticoids in patients with nephrotic syndrome. However, there is disagreement regarding the suitability of SI. Therefore, alternate indices should be considered. This study investigated whether or not selectivity index protein fraction (SIPF) was inferior to SI for the prediction of the response to glucocorticoids.Forty-nine patients with nephrotic syndrome were evaluated. On the basis of molecular weight and protein fraction, as an inexpensive substitute for SI, the clearance ratio of the albumin to γ fractions measured in serum and urine protein fractions was defined as SIPF. The quality of SIPF was examined. Moreover, the best cutoff value of SIPF was determined; and SIPF distribution, according to histopathological diagnosis by renal biopsy, was examined.SIPF was strongly correlated with SI (r = 0.79, P 0.001). The area under the receiver operating characteristic (ROC) curve of SIPF and SI was not significantly different (P = 0.18). The best cutoff value of SIPF was 0.45. In the group with SIPF 0.45, only two patients with minimal change disease (MCD) achieved complete remission. In the group with SIPF ≤ 0.45, all patients with MCD achieved complete remission, although eight patients with other histopathological diagnoses did not achieve complete remission.Analysis of protein fractions as a substitute for SI may be useful for predicting response to glucocorticoids in patients with nephrotic syndrome.

We herein report a case with aneurysm rupture in the arc of Bühler (AOB) caused by median arcuate ligament syndrome (MALS). The patient experienced a sudden onset of upper abdominal pain. Contrast-enhanced abdominal computed tomography (CT) showed an iso- to hyper-enhancing area mainly ranging from the dorsal aspect of the pancreatic head to the retroperitoneum around the right kidney. Abdominal angiography revealed marked stenosis in the origin of the celiac artery caused by MALS and a 7-mm saccular aneurysm in the AOB. Thus, we diagnosed the pain as having been caused by aneurysm rupture in the AOB due to MALS. The patient’s symptoms and anemia also improved to normal range without surgery. Careful follow-up, considering possible recurrence of aneurysm at other sites in the future, is essential.

Background and study aim: Transjugular liver biopsy (TJLB) can be used in patients who are ineligible for percutaneous liver biopsy (PLB) with acute and chronic hepatic disease. This study aimed to evaluate the usefulness and safety of TJLB in patients who were not indicated for PLB. Methods: Between July 2014 and February 2019, a total of 134 patients underwent liver biopsies at our institution. Among these, PLB was performed in 110 patients and TJLB in 24 patients. A retrospective comparison of clinical results in these patients was then performed. The primary endpoints of this study were the utility and safety of TJLB in patients who were not indicated for PLB. Results: The procedural success rate was 100% in both groups. The clinical response rate and the effective tissue sampling rate were 100% in the TJLB group and 97% in the PLB group (p = 0.55). There was no difference in the number of portal fields examined retrospectively between the two groups. No serious adverse events were observed in either group. Conclusions: It is suggested that TJLB is useful because it can be safely performed in patients with poor general condition who are not indicated for PLB.

Background:Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by the production of autoantibody and systemic tissue damages including glomerulonephritis. Immune responses mediated by autoreactive T-cells, as well as by autoantibody, is involved in the development and progression of end-organ damages1. Biologic agents which manipulate T-cell function such as CTLA4-Ig and anti-CD40L have been revisited and tried to treat human SLE, however, both of them failed to demonstrate efficacy.A mouse specific anti-CD3ε mAb, clone 145-2C11 (2C11) is known to be immunosuppressive by down-modulation of TCR and depletion of T-cells2. Administration of Fc-deleted 145-2C11 F(ab’)2to lupus-prone mice was reported to reduce lymphadenopathy and prolong survival, but had no significant effect on anti-DNA antibody titer3. The mechanisms by which 2C11 ameliorates lupus are still unclear.In this study, we used non-mitogenic Fc-modified silent 145-2c11 (2C11S), which disables interaction between target cells and Fc receptor-bearing cells, abolishes antibody directed cytotoxicity, and has longer half-life than F(ab’)2. 2C11S is expected to exert its effect in safe and stable as compared with functional parent-2C11 (2C11P) antibody.Objectives:The purpose of our study is to clarify the difference between 2C11S and 2C11P and to examine their therapeutic effects against murine lupus-prone (NZB/W F1) mice.Methods:20 μg of 2C11P (absolute antibody), 2C11S (absolute antibody), or isotype control immunoglobulin G1 κ (IC)(BioLegend) were administered intraperitoneally to C57BL6 mice. The difference of their action on T-cells were evaluated in a time series from peripheral blood. Plasma cytokine levels were measured within 24 hours after antibody administration.In NZB/W F1 mice from weeks 10 or 20, 2C11P, 2C11S, and IC were administered (100 μg / week, 4 times, intraperitoneally). Plasma anti-dsDNA antibody titer, spleen and kidney blood cell subpopulation, and histology of renal tissue were evaluated before and/or after treatment.Results:Duration of reduced TCR expression in 2C11S group was approximately twice as long as that in 2C11P group, and the levels of plasma TNF-α was not increased in 2C11S group while significant increase was observed in 2C11P group (IC; mean 48.3 ± SD 16.7 pg/ml, 2C11S; 57.9 ± 6.12, 2C11P; 168 ± 50.6, IC VS 2C11S; p>0.99, IC VS 2C11P; p=0.03, ANOVA).In NZB/W F1 mice, the number of follicular helper T (Tfh) cells in spleen significantly decreased in 2C11S group (IC; median 9.0*104[interquartile range 8.5*104], 2C11S; 1.8*104[1.0*104], 2C11P; 1.0*105[9.4*104], IC VS 2C11S; p=0.03, IC VS 2C11P; p>0.99, Kruskal-Wallis). The number of germinal center B (GCB) cells in spleen also decreased in 2C11S group (IC; 1.2*105[1.7*105], 2C11S; 9.0*103[2.3*104], 2C11P; 8.0*104[2.3*105], IC VS 2C11S; p=0.03, IC VS 2C11P; p>0.99). The number of infiltrating CD4+T-cells in kidney significantly reduced in 2C11S group (IC; 3.4*103[1.0*104], 2C11S; 6.4*102[8.8*102], 2C11P; 1.2*103[4.4*103], IC VS 2C11S; p=0.048, IC VS 2C11P; p=0.23). In addition, the rate of increase in anti-dsDNA IgG titers significantly decreased in 2C11S group (IC; 2.3 [1.3], 2C11S; 0.9 [1.0], 2C11P; 1.3 [1.4], IC VS 2C11S; p=0.03, IC VS 2C11P; p=0.24). Finally, glomerular hypercellularity was markedly alleviated only in 2C11S group (IC; 4.4*10 [8.4], 2C11S; 3.8*10 [1.1], 2C11P; 3.9*10 [8.2], IC VS 2C11S; p=0.02, IC VS 2C11P; p=0.57).Conclusion:2C11S did not induce cytokine release with maintaining longer effect on TCR down-modulation. 2C11S reduced autoantibody production by suppressing GCB differentiation, possibly through down-regulation of Tfh cell number. Consequently, 2C11S ameliorated lupus nephritis. On the other hand, 2C11P did not show therapeutic effect.References:[1]George C Tsokos. et al. Nat. Rev. Rheum (2016) 12: 716-730.[2]Kuhn C. et al. Immunotherapy (2016) 8: 889-906.[3]Henrickson M. et al. Arthritis Rheum (1994) 37: 587-589.Disclosure of Interests: :None declared

The authors fabricated OLED devices with enhanced out-coupling efficiency, made using corrugated substrates which have nanostructures imprinted from a self-assembled block-copolymer pattern. We optimized the substrate for transparent OLED lighting by shortening the pattern pitch to develop a new substrate that is transparent with 0.1 % haze and an out-coupling efficiency 1.79 times that of a device made with a flat glass substrate.

Smectic layer orientation was investigated for the side-chain PM10ME liquid crystal polymer to which steady shear and large amplitude oscillatory shear (LAOS) flows were applied. Two types of orien...

The shear flow orientation in the diblock copolymers composed of polystyrene (PS) and side-chain liquid crystal polymer (LCP) was investigated by the wide and small-angle X-ray diffraction methods. Two diblock copolymers prepared by atom transfer radical polymerization have the weight fractions of the PS segment of 0.13 and 0.21, and form a well-defined microsegregation structure with PS cylinders hexagonally packed in a matrix of LCP which forms smectic A, nematic, and isotropic phases in order of increasing temperature. Two types of shear flow orientation have been observed, depending on the type of the phase in the LCP matrix. The shear flow at nematic temperatures orients well the PS microcylinders as well as the LC mesogens in the velocity direction. This parallel-b orientation is expectable from the general orientation of the microcylinders and the LC mesogens in shear flow. When the LCP matrix transforms to the smectic LC, the mesogens are aligned parallel to the velocity gradient direction entaili...