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This paper focuses on gaps as the relations of evaluations against the professions with same occupation with those by two professions with different occupations and those against them between nurses, care and welfare professions as terminal care. For example, interprofessional communications（environment where it is easy to communication and information sharing with each expertise）, support based on wills of users and family care givers, one to put user’s need first and necessity of medical knowledge are analyzed. The results are as follows. The gaps that professions with same occupation approach more enough than those with another ones with different occupation as interprofessional communications and putting need of user exist first exist between nurses and care professions. On one hand, the same kind of gaps as guessing user’s will are between nurses and care professions, care and welfare professions. In addition, the same kind of gaps as eliciting desires from family exist between care and welfare professions. Further more, the ratio of opinion that they are not sure to what extent professions with different occupation support users and family care givers exist among nurses and care professions. As necessity of upgrading medical knowledge, more professions with different occupations agree strongly against care and welfare professions than nurses.

Background Many RRMS patients who had been treated for over 20 years with GA 20 mg/ml daily (GA20) switched to 40 mg/ml three times-a-week (GA40) to reduce injection-related adverse events. Although GA40 is as effective as GA20 in reducing annualized relapse rate and MRI activity, it remains unknown how switching to GA40 from GA20 affects the development of pathogenic and regulatory immune cells. Objective To investigate the difference in immunological parameters in response to GA20 and GA40 treatments. Methods We analyzed five pro-inflammatory cytokines (IL-1β, IL-23, IL-12, IL-18, TNF-α), and three anti-inflammatory/regulatory cytokines (IL-10, IL-13, and IL-27) in serum. In addition, we analyzed six cytokines (IFN-γ, IL-17A, GM-CSF, IL-10, IL-6, and IL-27) in cultured PBMC supernatants. The development of Th1, Th17, Foxp3 Tregs, M1-like, and M2-like macrophages were examined by flow cytometry. Samples were analyzed before and 12 months post switching to GA40 or GA20. Results Pro- and anti-inflammatory cytokines were comparable between the GA40 and GA20 groups. Development of Th1, Th17, M1-like macrophages, M2-like macrophages, and Foxp3 Tregs was also comparable between the two groups. Conclusions The immunological parameters measured in RRMS patients treated with GA40 three times weekly are largely comparable to those given daily GA20 treatment.

Dimethyl fumarate (DMF) is an oral agent for relapsing-remitting multiple sclerosis (RRMS). In this study, we investigated the therapeutic mechanism of DMF using experimental autoimmune encephalomyelitis (EAE). DMF treatment decreased the proliferation of T cells and the production of IL-17A and GM-CSF. DMF treatment also decreased the development and/or infiltration of macrophages in the central nervous system (CNS), and reduced the ratio of iNOS+ pro-inflammatory macrophage versus Ym1+ immunomodulatory macrophages. Furthermore, DMF treatment suppressed the deposition of complement C3 (C3) and development of reactive C3+ astrocytes. The decrease in iNOS+ macrophages, C3+astrocytes, and C3 deposition in the CNS resulted in the reduction in demyelination and axonal loss. This study suggests that the beneficial effects of DMF involve the suppression of iNOS+ pro-inflammatory macrophages, C3+ astrocytes, and deposition of C3 in the CNS.

Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system (CNS). Recent studies suggest that migration of Th1 and Th17 cells specific for enteric bacteria from the gut to the CNS may lead to the initiation and/or exacerbation of autoimmune diseases including MS. Human leukocyte antigen (HLA)-DR15 is an MHC class II (MHCII) haplotype highly associated with the development of MS that contains the two HLA-DRB* genes, DRB1*1501 (DR2b) and DRB5*0101 (DR2a). To identify enteric bacteria which harbor antigenic epitopes that activate myelin-specific T cells and drive CNS inflammation, we screened for enteric bacteria which express cross-reactive epitopes (&lsquo, mimotopes&rsquo, ) of an immunodominant myelin basic protein 89&ndash, 98 (MBP89-98) epitope. Based on known MHCII HLA-DR2a amino acid binding motifs and cultivation with splenic T cells isolated from MBP-T cell receptor (TCR)/DR2a transgenic (Tg) mice, we discovered that a certain variant of surface layer protein A (SLPA), which is expressed by a subtype of Clostridioides difficile, contains an amino acid sequence that activates MBP89-98-reactive T cells. Furthermore, activation of MBP-specific T cells by SLPA upon active immunization induced experimental autoimmune encephalomyelitis (EAE) in MBP-TCR/DR2a Tg mice. This study suggests that a unique strain of C. difficile possesses an encephalitogenic mimotope of MBP that activates autoreactive, myelin-specific T cells.

To elucidate the molecular mechanism of juvenility and annual flowering of fruit trees, FLOWERING LOCUS C (FLC), an integrator of flowering signals, was investigated in apple as a model. We performed sequence and expression analyses and transgenic experiments related to juvenility with annual flowering to characterize the apple FLC homologs MdFLC. The phylogenetic tree analysis, which included other MADS-box genes, showed that both MdFLC1 and MdFLC3 belong to the same FLC group. MdFLC1c from one of the MdFLC1 splice variants and MdFLC3 contain the four conserved motives of an MIKC-type MADS protein. The mRNA of variants MdFLC1a and MdFLC1b contain intron sequences, and their deduced amino acid sequences lack K- and C-domains. The expression levels of MdFLC1a, MdFLC1b, and MdFLC1c decreased during the flowering induction period in a seasonal expression pattern in the adult trees, whereas the expression level of MdFLC3 did not decrease during that period. This suggests that MdFLC1 is involved in flowering induction in the annual growth cycle of adult trees. In apple seedlings, because phase change can be observed in individuals, seedlings can be used for analysis of expression during phase transition. The expression levels of MdFLC1b, MdFLC1c, and MdFLC3 were high during the juvenile phase and low during the transitional and adult phases. Because the expression pattern of MdFLC3 suggests that it plays a specific role in juvenility, MdFLC3 was subjected to functional analysis by transformation of Arabidopsis. The results revealed the function of MdFLC3 as a floral repressor. In addition, MdFT had CArG box-like sequences, putative targets for the suppression of flowering by MdFLC binding, in the introns and promoter regions. These results indicate that apple homologs of FLC, which might play a role upstream of the flowering signals, could be involved in juvenility as well as in annual flowering. Apples with sufficient genome-related information are useful as a model for studying phenomena unique to woody plants such as juvenility and annual flowering.

Aim To describe the characteristics of objectively measured physical activity (PA) during the 2nd and 3rd trimesters of pregnancy using a accelerometer. Methods This was a longitudinal observational study wherein PA in pregnant women in the 2nd and 3rd trimesters was measured for seven consecutive days using a accelerometer (Silmee W10, TDK, Japan). Results A total of 34 primiparous women were examined. There was no statistically significant difference in the amount of PA during the 2nd and 3rd trimesters of pregnancy. Sub-group analysis demonstrated that PA in the full-time housewife group increased significantly from the 2nd to the 3rd trimester, while total PA and moderate and vigorous PA of the stopped-working group, decreased significantly over that time. There was no difference in the PA of the currently working group between trimesters. In the full-time housewife group, the amount of PA for each hour significantly increased at 12:00, 18:00, and 22:00 hr. In the currently working group, the amount of PA for each hour significantly increased at 9:00, 10:00, and 16:00 hr. In the stopped-working group, the amount of PA for each hour significantly decreased at 7:00, 8:00, and 18:00 hr. Conclusion Objective measurements using an accelerometer did not identify any significant changes in PA during the different trimesters of pregnancy. Sub-group analysis revealed clear patterns in PA change correlating with different lifestyles during pregnancy. Future research may enable the development of personalized health guidance by identifying the relationship between PA and pregnancy outcomes.

Branchio-oto-renal (BOR) syndrome is a rare autosomal dominant disorder characterized by branchiogenic anomalies, hearing loss, and renal anomalies. The aim of this study was to reveal the clinical phenotypes and their causative genes in Japanese BOR patients. Patients clinically diagnosed with BOR syndrome were analyzed by direct sequencing, multiplex ligation-dependent probe amplification (MLPA), array-based comparative genomic hybridization (aCGH), and next-generation sequencing (NGS). We identified the causative genes in 38/51 patients from 26/36 families; EYA1 aberrations were identified in 22 families, SALL1 mutations were identified in two families, and SIX1 mutations and a 22q partial tetrasomy were identified in one family each. All patients identified with causative genes suffered from hearing loss. Second branchial arch anomalies, including a cervical fistula or cyst, preauricular pits, and renal anomalies, were frequently identified (>60%) in patients with EYA1 aberrations. Renal hypodysplasia or unknown-cause renal insufficiency was identified in more than half of patients with EYA1 aberrations. Even within the same family, renal phenotypes often varied substantially. In addition to direct sequencing, MLPA and NGS were useful for the genetic analysis of BOR patients.

Glomerular parietal epithelial cell (PEC) activation, as revealed by de novo expression of CD44 and cell migration toward the injured filtration barrier, is a hallmark of podocyte injury-driven focal segmental glomerulosclerosis (FSGS). However, the signaling pathway that mediates activation of PECs in response to podocyte injury is unknown. The present study focused on CD44 signaling, particularly the roles of two CD44-related chemokines, migration inhibitory factor (MIF) and stromal cell-derived factor 1 (SDF1), and their common receptor, chemokine (C-X-C motif) receptor 4 (CXCR4), in the NEP25/LMB2 mouse podocyte-toxin model of FSGS. In the early phase of the disease, CD44-positive PECs were locally evident on the opposite side of the intact glomerular tuft and subsequently increased in the vicinity of synechiae with podocyte loss. Expression of MIF and SDF1 was first increased in injured podocytes and subsequently transferred to activated PECs expressing CD44 and CXCR4. In an immortalized mouse PEC (mPEC) line, recombinant MIF and SDF1 (rMIF and rSDF1, respectively) individually increased CD44 and CXCR4 mRNA and protein levels. rMIF and rSDF1 stimulated endogenous MIF and SDF1 production. rMIF- and rSDF1-induced mPEC migration was suppressed by CD44 siRNA. However, MIF and SDF1 inhibitors failed to show any impact on proteinuria, podocyte number, and CD44 expression in NEP25/LMB2 mice. Our data suggest that injured podocytes upregulate MIF and SDF1 that stimulate CD44 expression and CD44-mediated migration, which is enhanced by endogenous MIF and SDF1 in PECs. This biphasic expression pattern of the chemokine-CD44 axis in podocytes and PECs may be a novel mechanism of “podocyte-PEC cross-talk” signaling underlying podocyte injury-driven FSGS.

Purpose This study was performed to determine the effects of expiratory muscle strength training (EMST) on the oral and respiratory functions of community-dwelling older people. Methods Older people using a visiting-rehabilitation center were divided into an intervention group of 31 subjects and a control group of 15 subjects. Those in the intervention group were assigned home training for 8 weeks, which included 5 sets of 5 breaths per day with a 75% load of the maximum expiratory pressure using an EMST device. The outcome indices included (1) oral functions, evaluated by the cumulative time spent swallowing three times and the maximum phonation time (MPT) and (2) respiratory functions, evaluated by the maximum expiratory pressure and maximum inspiratory pressure (MEP/MIP). An independent t-test and paired t-test were used to analyze the data. Results The cumulative time spent swallowing three times was lower in the intervention group than in the control group. This difference remained significant even after adjusting for sex, age, and baseline values. The MPT was 2.1 seconds higher than baseline in the intervention group but 0.4 seconds lower than baseline in the control group. An average increase of 5.7 cmH2O in the PEmax was observed in the intervention group compared with an average decrease of 4.6 cmH2O in the control group, indicating a significant difference. Conclusion These results suggest that EMST improves the oral and respiratory functions of community elderly subjects. This may be explained by the fact that the pathway for swallowing is partially shared with that for phonation, which contributes to a shortened swallowing time by repeated suprahyoid muscle contractions.

C3 glomerulopathy (C3G) is a recently identified disease entity caused by dysregulation of the alternative complement pathway, and dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are its components. Because laboratory detection of complement dysregulation is still uncommon in practice, "dominant C3 deposition by two orders greater than that of immunoglobulins in the glomeruli by immunofluorescence", as stated in the consensus report, defines C3G. However, this morphological definition possibly includes the cases with glomerular diseases of different mechanisms such as post-infectious glomerulonephritis. In addition, the differential diagnosis between DDD and C3GN is often difficult because the distinction between these two diseases is based solely on electron microscopic features. Recent molecular and genetic advances provide information to characterize C3G. Some C3G cases are found with genetic abnormalities in complement regulatory factors, but majority of cases seem to be associated with acquired factors that dysregulate the alternative complement pathway. Because clinical courses and prognoses among glomerular diseases with dominant C3 deposition differ, further understanding the background mechanism, particularly complement dysregulation in C3G, is needed. This may resolve current dilemmas in practice and shed light on novel targeted therapies to remedy the dysregulated alternative complement pathway in C3G.

伊庭孝(1887-1937)の多彩な活動のうち、本稿では新劇から浅草オペラへの移行期に焦点を当て、とくに浅草オペラにおける活動の実際を概観・考察した。その際、ダンサーおよび作品の立案者として重要な役割を果たした高木徳子との協働に言及、演目としてはしばしば浅草オペラの嚆矢とされる歌舞劇『女軍出征』(1917)を取り上げ、その特徴や上演状況を明らかにするとともに、浅草オペラのレパートリーの一つとなっていった理由をテーマ、音楽の使用、演者、伊庭が命名した「歌舞劇」というジャンル面などから考察した。最後に伊庭の浅草オペラ観について触れ、通俗や流行といった表層的な面を忌避することなく「芸術的良心」をいかに保持するかを模索していたのではないかとの推測に至った。

研究報告, Original Articles, 近年500 ｇ以下の低出生体重児など重い障害をもつ子どもたちの生存の可能性が高まり，彼らの社会参加，充実した生活への支援の必要性が議論される．しかし，彼らがどのように生活を経験し，将来を希望しているか理解することは，言語的，非言語的表出の困難やその経験の不足のために困難である．今後の重度障害児の支援の方向を探るために，自立生活を営む重度障害者F 氏のライフチャート，ライフストーリーを作成しその生活を社会的文脈で探索した．インタビューおよび手記や手紙のナラティブ（語り），さらに記録，記事をデータとし，Frank（1996）を参考に，F 氏が記述した文章，写真，出版物，両親の記録などから，F 氏のライフヒストリーを理解する．F 氏が出会った出来事をライフチャートに整理する．それを通して，F 氏の自立生活がどのように可能になったかを，生活の転換点（ターニングポイント）を探りながら理解した．F 氏の自立生活は①時代背景や社会環境，②コミュニケーション手段の発展と教育環境，③関わる人々の理解や扱いと大きく影響しあいながら，可能になったことが明らかになった．, In recent years, we have a new issue regarding a healthy life for children with severe disabilities. The number of low-birth-weight newborns (i.e. < 500g) and their survival rate is growing. Supports are required for such babies to grow and to have a socially satisfying life; however, because of their verbal and/or nonverbal communication problems and the babies ongoing lack of life experience, it is hard for health professionals to understand how such babies experience life, and what hopes they or their families may have for their future. This is a qualitative study, the purpose of which was to investigate how a child with severe movement and communication problems lives life and finds direction to make a future. To build understand, I followed Mr. F, a 38 year old man with severe motor and communication disabilities, living an ‘independent life in a social context and create his “life chart”. I also investigated his life story to understand him as an occupational being. Using Frank (1996), I investigated F’s life history. Data was collected through interviewing, essays written by F, his photos, letters and public documentation. His life events were used to develop his life chart. I searched for his turning points in the process of his creating an independent life. Through my studies, I concluded that F’s independent life was constructed in mutual relation with,① Time background and the social environment of his life,② Development of communication technologies and the educational environment he had,③ Interactions with people, from his family to people in the ‘independent living’ movement abroad.

We have systematically measured the transport properties in the layered rhodium oxide K$_{x}$RhO$_{2}$ single crystals ($0.5\lesssim x \lesssim 0.67$), which is isostructural to the thermoelectric oxide Na$_{x}$CoO$_{2}$. We find that below $x = 0.64$ the Seebeck coefficient is anomalously enhanced at low temperatures with increasing $x$, while it is proportional to the temperature like a conventional metal above $x=0.65$, suggesting an existence of a critical content $x^{*} \simeq 0.65$. For the origin of this anomalous behavior, we discuss a filling-induced Lifshitz transition, which is characterized by a sudden topological change in the cylindrical hole Fermi surfaces at the critical content $x^*$.

Aim The Japan Diabetes Society (JDS)/Japan Geriatrics Society (JGS) Joint Committee reported 'Glycemic Targets for Elderly Patients with Diabetes' in 2016. Based on this recommendation, we aimed to clarify 1) the achievement status of glycemic targets in the elderly and 2) the presence of hypoglycemia in real life among elderly individuals with an HbA1c below the lower limit. Subjects and methods [Analysis I] In 326 elderly with diabetes ≥65 years of age visiting the outpatient department specializing in diabetes, the proportions of patients with HbA1c values below the lower limit and the use of drugs potentially associated with severe hypoglycemia (e.g. insulin formulations, sulfonylureas, glinides) were investigated. [Analysis II] Of the patients with HbA1c values below the lower limit, seven were tested for hypoglycemia in real life using a continuous glucose monitoring system (CGM). Results [Analysis I] Among the 326 subjects, 235 (72.1%) were using drugs potentially associated with severe hypoglycemia, and 63 (19.3%) had an HbA1c value below the lower limit. [Analysis II] In the seven patients examined using CGM, hypoglycemia was detected in five, all of whom were unaware. Conclusions A considerable number of elderly patients were taking drugs associated with hypoglycemic risks and had an HbA1c value below the lower limit, some of whom actually had hypoglycemia as detected by CGM. Using tools such as CGM, preventive measures against hypoglycemia should be taken.

Our previous study detected aflatoxins in red chili peppers from Chile, Bolivia, and Peru, each of which have a high incidence of gallbladder cancer (GBC). Since the aflatoxin B1 concentration was not so high in these peppers, it is important to clarify the presence of other mycotoxins. Here we attempted to determine any associations between the concentrations of aflatoxins and ochratoxin A (OTA) in red chili peppers, and the corresponding GBC incidences. We collected red chili peppers from three areas in Peru: Trujillo (a high GBC incidence area), Cusco (an intermediate GBC incidence area), and Lima (a low GBC incidence rate), and from Chile and Bolivia. Aflatoxins and OTA were extracted with organic solvents. The concentrations of aflatoxins B1, B2, G1, and G2, and OTA were measured by high-performance liquid chromatography. The values obtained were compared with the incidence of GBC in each area or country. All of the red chili peppers from the three areas showed contamination with aflatoxins below the Commission of the European Communities (EC) recommended limits (5 μg/kg), but the OTA contamination of two samples was above the EC recommended limit (15 μg/kg). The mean concentrations of OTA in the peppers from Chile (mean 355 μg/kg, range

新劇ブームに沸く1913(大正2)年3月、帝国劇場でゲーテの『ファウスト』第一部が近代劇協会により上演された。完成間もない森?外の翻訳を台本とし、伊庭孝が舞台監督兼俳優を務め、上山草人ら新劇俳優と、帝劇歌劇部の歌手や管弦楽団が結集しての待望の公演であった。伊庭は演出に意を尽くし、原作25場を、一部省略しながら5幕14場にまとめ、グノーのオペラから数曲の管弦楽曲が編曲・流用され、また原作中の「歌」の部分には清水金太郎が付曲した。ゲーテの原作に音楽的指示が施されているとはいえ、帝劇の本公演は伊庭流にアレンジされた一種の音楽劇の様相を呈し、このあと浅草オペラなどで活躍する伊庭に音楽劇制作への志向を促す舞台になったと考えられる。

Autosomal dominant distal renal tubular acidosis (dRTA) is a rare disorder caused by a mutation in the AE1 gene encoding the chloride–bicarbonate (Cl−/HCO3 −) anion exchanger 1 (AE1). Most patients with this disorder present with clinical symptoms in adulthood and their phenotype is milder than that of those with autosomal recessive dRTA. In this report, we describe a Japanese family with autosomal dominant dRTA in which the mother and her daughter presented with severe symptoms caused by hypokalemia at 2 years of age. The heterozygous AE1 mutation G609R, which is a known causative mutation of dRTA, was identified in both patients. To our knowledge, this is the first report of a Japanese family with autosomal dominant type dRTA caused by an AE1 mutation. We, therefore, propose that alterations of AE1 should be considered causative of autosomal dominant dRTA even if typical symptoms appear during early childhood and the clinical features are severe.

Purpose To characterize the peripheral cones in the images obtained by spectral-domain optical coherence tomography (OCT), swept source OCT, and adaptive optics fundus camera in a patient with peripheral cone dystrophy. Methods A 28-year-old Japanese man underwent detailed ophthalmic evaluations including high-resolution imaging of the fundus of both eyes. Results The decimal best-corrected visual acuity was 1.2 in both eyes. The results of slit-lamp biomicroscopy and ophthalmoscopy were essentially normal. Fluorescein and indocyanine green angiographies did not show any hyper- or hypofluorescent areas of the retina. Goldmann perimetry showed full peripheral visual fields but relative central scotomas within the central 20°. The results of the Humphrey Visual Field Analyzer showed a limited preservation of the central sensitivity. Color vision tests showed no errors in both eyes. Spectral-domain OCT showed attenuation of both the ellipsoid and interdigitation zones throughout the macular region except the center of the fovea. The scotopic full-field ERGs were normal, but the photopic ERGs were markedly reduced. Regular cone mosaics were not observed especially more than 450 μm radius from the fovea in the adaptive optics retinal images. The parafoveal cone densities were severely decreased in both eyes. Conclusions Our findings indicate that the peripheral cone dystrophy diagnosed by full-field ERGs and perimetry is due to a reduction in the density of parafoveal and peripheral cones.

Quality of life, comfort, and wellbeing during pregnancy are essential for every country in the world. Pregnancy is considered a preparation period for becoming a mother. Maternal role development, including confidence and satisfaction as a mother, is important in the transition to motherhood. Negative psychosocial affect, such as increased anxiety and distress, during pregnancy adversely influences the childbirth experience and childcare, which contributes to postpartum depression. However, the impact of positive feelings on the maternal role development remains unclear. Therefore, the study purpose was to clarify the relationship between comfort in late pregnancy and maternal role attainment and childcare during early postpartum. We designed a descriptive, longitudinal, correlational study by using the Prenatal Comfort Scale, the Postpartum Maternal Role Confidence Scale, and the Postpartum Maternal Satisfaction Scale. Among 339 participants who had received care at a university hospital located in Sendai city in Japan, 215 subjects completed the longitudinal study by answering a questionnaire for the respective Scale late in their pregnancy or during early postpartum. The subjects consisted of 114 primipara (32.0 ± 5.4 years) and 101 multipara (33.4 ± 4.9 years). In primipara, comfort with motherhood was significantly correlated with maternal confidence regarding knowledge and childcare skills and maternal satisfaction. In multipara, comfort in late pregnancy was related to maternal confidence and satisfaction. Positive affect was related to maternal confidence and maternal satisfaction in early postpartum. Therefore, a prenatal nursing intervention helps women become more comfortable with impending motherhood, thereby promoting maternal role attainment after delivery.

Spin-state transition, also known as spin crossover, plays a key role in diverse systems, including minerals and biological materials. In theory, the boundary range between the low- and high-spin states is expected to enrich the transition and give rise to unusual physical states. However, no compound that realizes a nearly degenerate critical range as the ground state without requiring special external conditions has yet been experimentally identified. This study reports that, by comprehensive measurements of macroscopic physical properties, X-ray diffractometry, and neutron spectroscopy, the Sc substitution in LaCoO$_3$ destabilizes its nonmagnetic low-spin state and generates an anomalous paramagnetic state accompanied by the enhancement of transport gap and magneto-lattice-expansion as well as the contraction of Co--O distance with the increase of electron site-transfer. These phenomena are not well described by the mixture of conventional low- and high-spin states, but by their quantum superposition occurring on the verge of a spin-state transition. The present study enables us to significantly accelerate the design of new advanced materials without requiring special equipment based on the concept of quantum spin-state criticality., Keywords: excitonic insulator, bi-exciton

C3 glomerulopathy (C3G) is a recently identified disease entity caused by dysregulation of the alternative complement pathway, and dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are its components. Because laboratory detection of complement dysregulation is still uncommon in practice, “dominant C3 deposition by two orders greater than that of immunoglobulins in the glomeruli by immunofluorescence”, as stated in the consensus report, defines C3G. However, this morphological definition possibly includes the cases with glomerular diseases of different mechanisms such as post-infectious glomerulonephritis. In addition, the differential diagnosis between DDD and C3GN is often difficult because the distinction between these two diseases is based solely on electron microscopic features. Recent molecular and genetic advances provide information to characterize C3G. Some C3G cases are found with genetic abnormalities in complement regulatory factors, but majority of cases seem to be associated with acquired factors that dysregulate the alternative complement pathway. Because clinical courses and prognoses among glomerular diseases with dominant C3 deposition differ, further understanding the background mechanism, particularly complement dysregulation in C3G, is needed. This may resolve current dilemmas in practice and shed light on novel targeted therapies to remedy the dysregulated alternative complement pathway in C3G.

Postoperative hyperglycemia is associated with infectious complications after various types of surgery. Our objective was to determine whether postoperative blood glucose levels up to 1 week after highly invasive esophageal cancer surgery are associated with the incidence of postoperative infections (POIs). We conducted a retrospective chart review of 109 consecutive thoracic esophageal squamous cell cancer patients who underwent invasive esophagectomy with thoracotomy and laparotomy. The incidence of postoperative POIs and risk factors for POIs, including postoperative blood glucose levels, were evaluated. Of the 109 patients, 37 (34.0 %) developed POIs. Clinically, 73.0 % of the POIs became evident on or after postoperative day 4 (median, 5.25 days; interquartile range, 3.00–9.25 days). On and after postoperative day 3, chronological changes in blood glucose levels were significantly different between two groups of patients with or without POIs, as indicated by repeated measures ANOVA (P = 0.006). Multivariate logistic regression analysis results showed that an increased blood glucose concentration on postoperative day 3 was a significant risk factor for POIs. Our findings suggested that postoperative hyperglycemia on postoperative day 3 was a predictive factor of POIs after highly invasive esophageal cancer surgery.

A 9-year-old girl developed influenza A H1N1 pdm09-associated myocarditis and pericarditis 2 days after starting zanamivir therapy. The virus was detected in the respiratory tract but not in the serum or pericardial effusion. The virus sampled from the respiratory tract had normal susceptibility to neuraminidase inhibitors. Although no differences in interferon-γ, interleukin (IL)-1β, and tumor necrosis factor-α were observed between the plasma and pericardial effusion, some inflammatory cytokines or chemokines (IL-6 and IL-8) and vascular endothelial growth factor were remarkably elevated in the pericardial effusion compared with the plasma. This suggested that the influenza virus, after infecting the respiratory tract, affected the myocardium, causing myocarditis to gradually develop, which might have been followed by an autoreactive pericarditis causing increased pericardial effusion. Therefore, influenza-associated myocarditis should be considered when influenza patients have respiratory and cardiac involvement, even during treatment with a neuraminidase inhibitor.

Darbepoetin alfa (DA) is beneficial for pediatric patients for its less injection frequency and greater maximum dose compared to recombinant human erythropoietin. Here, we evaluated pharmacokinetics of DA in Japanese pediatric patients with chronic kidney disease (CKD).CKD patients (2-18 years old, n = 8 each) received a single dose of body weight adjusted DA either intravenously or subcutaneously.When administered intravenously, the area under the concentration-time curve from time zero to infinity (AUC0-∞), clearance (CL) and terminal half-life (t 1/2) of DA were 263.7 ng · h/mL, 1.77 mL/h/kg and 26.25 h, respectively (mean). In patients under 12 years old, AUC0-∞, CL and t 1/2 were 219.1 ng · h/mL, 2.19 mL/h/kg, 23.62 h, respectively. These values were mostly similar to those of Japanese adult CKD patients, though AUC0-∞ tended to be lower and CL tended to be higher in the subjects under 12 years old. When administered subcutaneously, time to reach maximum concentration (t max) and maximum concentration (C max) were 24.47 h and 1.704 ng/mL, and AUC0-∞, apparent clearance (CL/F) and t 1/2 were 141.1 ng · h/mL, 3.23 mL/h/kg and 46.73 h, respectively. In patients under 12 years old, t max and C max were 7.50 h and 2.053 ng/mL, and AUC0-∞, CL/F and t 1/2 were 136.7 ng · h/mL, 3.29 mL/h/kg and 37.75 h, respectively, which was higher in C max, faster in t max and shorter t 1/2 compared to adult CKD patients, while AUC was not obviously different.The pharmacokinetics of DA in pediatric CKD patients is not obviously different from those in adult.

This study analyzed the factors responsible for determining the flavor detection time for chewed semi-solid foods. Thirteen healthy young adults (eight males and five females) were asked to chew gummy candies with five different fruit flavors (i.e., apple, grape, orange, pear, and strawberry) in a random order. The detection time to flavor was measured using an electromyography-based system, which was recently developed by the authors. Briefly, each participant was recorded with surface masseter electromyograms on both sides to determine the start of chewing. Each participant was asked to press a button as soon as possible with his/her preferred hand after detecting the flavor. The time elapsed between the start of chewing and the button press was measured. Two taste components, sugars and organic acids, of the gummy candies were chemically analyzed, and two major sugars and organic acid were individually detected. The odor intensity was also analyzed for the gummy candies. The average detection time significantly differed among the five gummy candies and among the participants. Simple regression analyses revealed that the intensity of the odors was significantly associated with the average time intervals, but the amounts of the two major sugars and the organic acids were not. The analysis yielded the following equation for the regression estimation: (y = the time interval, x = the intensity of odors). The results suggest that the intensity of odors of the gummy candies, not the taste components, is responsible for determining the flavor detection time.

Few studies have evaluated the effects of activity patterns of the jaw closing muscles assessed by specific parameters on jaw opening in subsequent cycles during the chewing of food. The objective of this study was to quantitatively analyze the effect of the masseter (jaw closer) activity patterns on suprahyoid (jaw opener) activity during subsequent cycles. The assessments were performed while participants naturally chewed six test foods that differed in size dimensions and textural properties. Surface electromyograms of the masseter (on the habitual working side) and suprahyoid muscles were recorded in ten healthy young adults, each of whom randomly received one of the six test foods. The activity patterns were assessed using three parameters specifically developed for their quantification. Changes in suprahyoid activity during each of the subsequent chewing cycles were examined by three amplitudinal (minimum, maximum, and net values of the integrated suprahyoid electromyogram) parameters and one durational (active duration) parameter. The main finding was that two of the three activity pattern parameters had a statistically significant effect only on the three amplitudinal parameters in three of the six test foods. These results suggest that masseter activity patterns partially affect suprahyoid activity during subsequent chewing cycles and that the effect is food dependent. A possible neural mechanism responsible for this effect is presented.

Kabuki syndrome is a congenital anomaly syndrome characterized by developmental delay, intellectual disability, specific facial features including long palpebral fissures and ectropion of the lateral third of the lower eyelids, prominent digit pads, and skeletal and visceral abnormalities. Mutations in MLL2 and KDM6A cause Kabuki syndrome. We screened 81 individuals with Kabuki syndrome for mutations in these genes by conventional methods (n = 58) and/or targeted resequencing (n = 45) or whole exome sequencing (n = 5). We identified a mutation in MLL2 or KDM6A in 50 (61.7%) and 5 (6.2%) cases, respectively. Thirty-five MLL2 mutations and two KDM6A mutations were novel. Non-protein truncating-type MLL2 mutations were mainly located around functional domains, while truncating-type mutations were scattered through the entire coding region. The facial features of patients in the MLL2 truncating-type mutation group were typical based on those of the 10 originally reported patients with Kabuki syndrome; those of the other groups were less typical. High arched eyebrows, short fifth finger, and hypotonia in infancy were more frequent in the MLL2 mutation group than in the KDM6A mutation group. Short stature and postnatal growth retardation were observed in all individuals with KDM6A mutations, but in only half of the group with MLL2 mutations.