241 research outputs found

    Node Query Preservation for Deterministic Linear Top-Down Tree Transducers

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    This paper discusses the decidability of node query preservation problems for XML document transformations. We assume a transformation given by a deterministic linear top-down data tree transducer (abbreviated as DLT^V) and an n-ary query based on runs of a tree automaton. We say that a DLT^V Tr strongly preserves a query Q if there is a query Q' such that for every document t, the answer set of Q' for Tr(t) is equal to the answer set of Q for t. Also we say that Tr weakly preserves Q if there is a query Q' such that for every t_d in the range of Tr, the answer set of Q' for t_d is equal to the union of the answer set of Q for t such that t_d = Tr(t). We show that the weak preservation problem is coNP-complete and the strong preservation problem is in 2-EXPTIME.Comment: In Proceedings TTATT 2013, arXiv:1311.505

    Differential diagnosis of nonepileptic twilight state with convulsive manifestations after febrile seizures

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    Background Nonepileptic twilight state with convulsive manifestations (NETC) is a nonepileptic state following a febrile seizure (FS), which may be misdiagnosed as a prolonged seizure and result in overtreatment. We aimed to describe clinical manifestations of NETC and to determine characteristics that are helpful to distinguish NETC from other pathological conditions. Methods We conducted a retrospective chart review from January 2010 to December 2016 and selected the patients who presented with symptoms resembling status epilepticus with fever and a confirmed diagnosis using an electroencephalogram (EEG). We compared the NETC clinical features and venous blood gas analysis results with those of other conditions that mimic NETC. We also compared the characteristics of NETC with past reports. Results Our NETC patients presented with short durations of the preceding generalized convulsions followed by tonic posturing, closed eyes, no cyanosis, responsiveness to painful stimulation, and no accumulation of CO2 in the venous blood gas. Most of these characteristics were consistent with past reports. Prolonged FS or acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) showed several of these features, but all the characteristics were not consistent with our study. Conclusions Prolonged FS and AESD need to be differentiated from NETC, and close clinical observation makes it possible to partially distinguish NETC from the other conditions. EEG is recommended for patients with symptoms that are inconsistent with these features

    An accurate algorithm of numerical integration for computing seismic responses of inelastic structures

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    The paper proposes an algorithm that makes numerical integrations with conditions for stability to be usable for computing inelastic seismic responses of structures. The numerical integrations with conditions cannot be applied to solving directly the equation of the motion. These numerical integrations, however, give exacter results in linear seismic responses than that by the numerical integrations without any conditions. The method of modal analysis can clear the condition by applying the eigenvalue analysis to the equation of the motion expressed by the tangent stiffness in the inelasticity. The paper shows considerable difference between the result by the numerical integration with conditions and that without any condition, and the former result will be exacter than the latter from several proofs in the discussion

    Laboratory changes during adrenocorticotropic hormone therapy associated with renal calcified lesions

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    Background Renal calcified lesions are known as one of the complications during adrenocorticotropic hormone (ACTH) therapy for intractable epilepsy. However, laboratory changes during the therapy or laboratory features of high‐risk cases with renal calcified lesions are yet to be clarified. Methods In this study, 43 patients with West syndrome aged ≤2 years were included. We retrospectively reviewed age and body mass index at the beginning of ACTH therapy, as well as the amount of fluid intake, daily urinary volume, and laboratory data during therapy. In addition, we studied the urinary sediment of the cases with renal calcified lesions diagnosed by computed tomography. Results After initiating ACTH treatment, urinary calcium (Ca)/creatinine ratio and urinary pH increased within 2 weeks. Urinary crystals and renal tubular epithelial cells (RTECs) in urinary sediment were frequently found in most cases. Urinary Ca levels, proteinuria or frequency of urinary crystals, and number of RTECs in the urinary sediment were significantly higher in patients with epithelial casts (ECs) or hematuria than in patients without these findings. Among the seven patients who underwent abdominal CT, ECs or hematuria were found only in those with renal calcified lesions. These findings suggested that patients with ECs or hematuria were more likely to have calcified lesions. Conclusions The risk of renal calcified lesions increased after 2 weeks of ACTH treatment. Abnormal findings in urinary sediments might be an early sign of renal calcification during ACTH therapy

    Novel and recurrent COMP gene variants in five Japanese patients with pseudoachondroplasia: skeletal changes from the neonatal to infantile periods

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    Pseudoachondroplasia (PSACH) is an autosomal dominant skeletal dysplasia caused by pathogenic variants of cartilage oligomeric matrix protein (COMP). Clinical symptoms of PSACH are characterized by growth disturbances after the first year of life. These disturbances lead to severe short stature with short limbs, brachydactyly, scoliosis, joint laxity, joint pain since childhood, and a normal face. Epimetaphyseal dysplasia, shortened long bones, and short metacarpals and phalanges are common findings on radiological examination. Additionally, anterior tonguing of the vertebral bodies in the lateral view is an important finding in childhood because it is specific to PSACH and normalizes with age. Here, we report five Japanese patients with PSACH, with one recurrent (p.Cys351Tyr) and four novel heterozygous pathogenic COMP variants (p.Asp437Tyr, p.Asp446Gly, p.Asp507Tyr, and p.Asp518Val). These five pathogenic variants were located in the calcium-binding type 3 (T3) repeats. In four of the novel variants, the affected amino acid was aspartic acid, which is abundant in each of the eight T3 repeats. We describe the radiological findings of these five patients. We also retrospectively analyzed the sequential changes in the vertebral body and epimetaphysis of the long bones from the neonatal to infantile periods in a patient with PSACH and congenital heart disease

    Thioredoxin interacting protein protects mice from fasting induced liver steatosis by activating ER stress and its downstream signaling pathways

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    Under normal conditions, fasting results in decreased protein disulfide isomerase (PDI) activity and accumulation of unfolded proteins, leading to the subsequent activation of the unfolded protein response (UPR)/autophagy signaling pathway to eliminate damaged mitochondria. Fasting also induces upregulation of thioredoxin-interacting protein (TXNIP) expression and mice deficient of this protein (TXNIP-KO mice) was shown to develop severe hypoglycemia, hyperlipidemia and liver steatosis (LS). In the present study, we aimed to determine the role of TXNIP in fasting-induced LS by using male TXNIP-KO mice that developed LS without severe hypoglycemia. In TXNIP-KO mice, fasting induced severe microvesicular LS. Examinations by transmission electron microscopy revealed mitochondria with smaller size and deformities and the presence of few autophagosomes. The expression of beta-oxidation-associated genes remained at the same level and the level of LC3-II was low. PDI activity level stayed at the original level and the levels of p-IRE1 and X-box binding protein 1 spliced form (sXBP1) were lower. Interestingly, treatment of TXNIP-KO mice with bacitracin, a PDI inhibitor, restored the level of LC3-II after fasting. These results suggest that TXNIP regulates PDI activity and subsequent activation of the UPR/autophagy pathway and plays a protective role in fasting-induced LS

    [6]Cyclo-2,7-naphthyl­ene: a redetermination

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    Single crystals of a macrocyclic hydro­carbon, [6]cyclo-2,7-naphthyl­ene ([6]CNAP, C60H36) were prepared from anthracene melt with a prolonged time for the recrystallization. The crystal of improved quality led to the correction of the space-group assignment to Cmca from in the original determination [Nakanishi et al. (2011 ▶) Angew. Chem. Int. Ed. 50, 5323–5326] and the refinement of anisotropic displacement parameters of all C atoms. The refined mol­ecular structure with C 2h point symmetry indicated that the strain on the naphthyl rings of [6]CNAP is smallest among the congeners. Despite the large macrocyclic structure, mol­ecules are packed in a ubiquitous herringbone motif. A short C—C distance of 3.119 (4) Å was found in the stacking direction, and a short C—H distance of 2.80 Å was found in the inter­columnar contact

    Mothers\u27 Perceptions of the Sexual Development and Behavior of their Children and Persons with Autism in General

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    In order to ascertain and evaluate mothers\u27 perceptions of the sexual development and behavior of their children with autism, we conducted a questionnaire survey on participants in meetings held in Nagasaki. From among 172 persons who consented to participate in the survey and responded to the self-administered questionnaire, we selected 71 mothers of sons with autism (aged 6 years or over) as the study subjects. The questionnaire included items on children\u27s sexual behaviors, the appearance of secondary sexual characters/sexual development, the mothers\u27 perceptions of the children\u27s sexual development and behavior, and assessments of their own child and persons with autism in general with respect to masturbation, romantic relationships, and marriage. According to the severity of the children\u27s autism, subjects were classified into severe and non-severe groups and the above-mentioned items were compared between the two groups. No significant difference was observed between the two groups in terms of the age at first appearance of secondary sex characters; however, the age at which interest in the opposite sex was first shown was significantly earlier in the non-severe group (p = 0.031). Among 58 children aged 120 months or over, 29 (50.0%) were masturbating and the proportion of such children was significantly larger in the severe group (p = 0.014). Regarding problem sexual behavior, no significant difference was observed in the frequency of worried mothers between the two groups (p = 0.80), while "talking about sex in public" was more frequent in the non-severe group (p = 0.018). Approximately 80% of the mothers in both groups had positive views about masturbation both for their own child and for persons with autism in general; however, the proportion of mothers showing positive views about romantic relationships or marriage was significantly smaller in the severe group (p<0.0001). The present study demonstrated that mothers\u27 perceptions of sexual development and behavior in their children with autism vary according to the disease severity. This finding indicates the necessity for healthcare professionals to actively concern themselves with the mothers
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