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Sample records for kallmann syndrome ks

  1. MR tomography in Kallmann's syndrome

    International Nuclear Information System (INIS)

    Dewes, W.; Krahe, T.; Klingmueller, D.; Harder, T.; Bonn Univ.

    1987-01-01

    MR tomography is the only imaging method that can demonstrate atrophy of the olfactory lobe of the brain in olfacto-genital dysplasia (Kallmann's syndrome). The MRT findings in five patients with Kallmann's syndrome are described. The MRT criterion for the presence of Kallmann's syndrome appears to be an interruption or total absence of the olfactory sulcus. (orig.) [de

  2. Kallmann syndrome and paranoid schizophrenia: A rare combination

    NARCIS (Netherlands)

    W.M.A. Verhoeven (Wim); J.I.M. Egger (Jos); J.E.J.M. Hovens (Hans); E.H. Hoefsloot (Lies)

    2013-01-01

    textabstractKallmann syndrome (KS) is a genetically heterogeneous and rare disorder characterised by the combination of hypothalamic hypogonadism and anosmia/hyposmia, a variable degree of intellectual disability and several somatic anomalies. In about one-third of the patients, mutations have been

  3. Kallmann syndrome and paranoid schizophrenia: a rare combination.

    Science.gov (United States)

    Verhoeven, Willem M A; Egger, Jos I M; Hovens, Johannes E; Hoefsloot, Lies

    2013-01-17

    Kallmann syndrome (KS) is a genetically heterogeneous and rare disorder characterised by the combination of hypothalamic hypogonadism and anosmia/hyposmia, a variable degree of intellectual disability and several somatic anomalies. In about one-third of the patients, mutations have been identified in at least seven different genes. Virtually no data are available about possible neuropsychiatric symptoms in KS. Here, a young adult male is described with a previous clinical diagnosis of KS and recent paranoid schizophrenia of which positive, but not negative symptoms, fully remitted upon treatment with antipsychotics. Neither genome-wide array analysis nor mutation analyses disclosed imbalances or mutations in any of presently known KS disease genes. This is the first report on a patient with KS and paranoid schizophrenia in whom extensive genetic analyses were performed. It is concluded that further studies are warranted in order to elucidate a possible increased risk for psychiatric symptoms in patients with KS.

  4. Clinical and inheritance profiles of Kallmann syndrome in Jordan

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    Shegem Nadima S

    2004-10-01

    Full Text Available Abstract Background Proper management of patients with Kallmann syndrome (KS allows them to attain a normal reproductive health. The purpose of this study is to demonstrate the presentation modalities, phenotypes and the modes of inheritance among 32 patients with Kallmann syndrome in Jordan. Recognition of the syndrome allows for prompt proper management and provision of genetic counselling. Subjects Over a period of five years (1999–2004, the clinical and inheritance profiles of 26 male and 6 female patients with Kallmann syndrome from 12 families were evaluated at the National Center for Diabetes, Endocrinology and Genetics in Jordan. Results The patients belonged to twelve Jordanian and Palestinian families and their age at presentation ranged from 4 – 46 years. Nine boys aged 4–14 years presented with cryptorchidism and microphallus, all other males presented with delayed puberty, hypogonadism and/or infertility. The main presentation among six female patients was primary amenorrhea. Intrafamilial variability in clinical phenotype was specifically evident for renal abnormalities and sensorineural hearing impairment. Familial KS was diagnosed in 27 patients belonging to five families with the X-linked mode of inheritance and two families with the autosomal recessive mode of inheritance. Conclusions (1 the majority of cases in this study represented the X-linked form of KS, which might point to a high prevalence of Kal 1 gene in the population. (2 Genetic counselling helps these families to reach a diagnosis at an early age and to decide about their reproductive options. (3 Children presenting with cryptorchidism and microphallus in our population should be investigated for KS.

  5. Hypogonadotrophic short statured with anosmia: kallmann's syndrome

    International Nuclear Information System (INIS)

    Tarique, S.; Abaidullah, S.; Cheema, T.M.; Iqbal, M.H.

    2010-01-01

    Short stature with loss of secondary sex characters can occur due to genetic disorders. One of them is Kallmann's syndrome. The condition has been noted to be present in families. It is associated with anosmia and hypogonadism. We are presenting a case of young boy who was short statured and had anosmia with multiple hormonal deficiencies. (author)

  6. Klinefelter Syndrome (KS): Condition Information

    Science.gov (United States)

    ... Condition Information Share Facebook Twitter Pinterest Email Print Klinefelter Syndrome (KS): Condition Information What is KS? The term " ... such as XXYY. This is called poly-X Klinefelter syndrome, and it causes more severe symptoms. 1 Klinefelter, ...

  7. A case of Kallmann syndrome associated with a non-functional pituitary microadenoma

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    Taieb Ach

    2018-04-01

    Full Text Available Kallmann syndrome (KS is a form of hypogonadotropic hypogonadism in combination with a defect in sense of smell, due to abnormal migration of gonadotropin-releasing hormone-producing neurons. We report a case of a 17-year-old Tunisian male who presented with eunuchoid body proportions, absence of facial, axillary and pubic hair, micropenis and surgically corrected cryptorchidism. Associated findings included anosmia. Karyotype was 46XY and hormonal measurement hypogonadotropic hypogonadism. MRI of the brain showed bilateral agenesis of the olfactory bulbs and 3.5 mm pituitary microadenoma. Hormonal assays showed no evidence of pituitary hypersecretion.

  8. Loss-of-function mutations in SOX10 cause Kallmann syndrome with deafness.

    Science.gov (United States)

    Pingault, Veronique; Bodereau, Virginie; Baral, Viviane; Marcos, Severine; Watanabe, Yuli; Chaoui, Asma; Fouveaut, Corinne; Leroy, Chrystel; Vérier-Mine, Odile; Francannet, Christine; Dupin-Deguine, Delphine; Archambeaud, Françoise; Kurtz, François-Joseph; Young, Jacques; Bertherat, Jérôme; Marlin, Sandrine; Goossens, Michel; Hardelin, Jean-Pierre; Dodé, Catherine; Bondurand, Nadege

    2013-05-02

    Transcription factor SOX10 plays a role in the maintenance of progenitor cell multipotency, lineage specification, and cell differentiation and is a major actor in the development of the neural crest. It has been implicated in Waardenburg syndrome (WS), a rare disorder characterized by the association between pigmentation abnormalities and deafness, but SOX10 mutations cause a variable phenotype that spreads over the initial limits of the syndrome definition. On the basis of recent findings of olfactory-bulb agenesis in WS individuals, we suspected SOX10 was also involved in Kallmann syndrome (KS). KS is defined by the association between anosmia and hypogonadotropic hypogonadism due to incomplete migration of neuroendocrine gonadotropin-releasing hormone (GnRH) cells along the olfactory, vomeronasal, and terminal nerves. Mutations in any of the nine genes identified to date account for only 30% of the KS cases. KS can be either isolated or associated with a variety of other symptoms, including deafness. This study reports SOX10 loss-of-function mutations in approximately one-third of KS individuals with deafness, indicating a substantial involvement in this clinical condition. Study of SOX10-null mutant mice revealed a developmental role of SOX10 in a subpopulation of glial cells called olfactory ensheathing cells. These mice indeed showed an almost complete absence of these cells along the olfactory nerve pathway, as well as defasciculation and misrouting of the nerve fibers, impaired migration of GnRH cells, and disorganization of the olfactory nerve layer of the olfactory bulbs. Copyright © 2013 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  9. SEMA3A, a gene involved in axonal pathfinding, is mutated in patients with Kallmann syndrome.

    Science.gov (United States)

    Hanchate, Naresh Kumar; Giacobini, Paolo; Lhuillier, Pierre; Parkash, Jyoti; Espy, Cécile; Fouveaut, Corinne; Leroy, Chrystel; Baron, Stéphanie; Campagne, Céline; Vanacker, Charlotte; Collier, Francis; Cruaud, Corinne; Meyer, Vincent; García-Piñero, Alfons; Dewailly, Didier; Cortet-Rudelli, Christine; Gersak, Ksenija; Metz, Chantal; Chabrier, Gérard; Pugeat, Michel; Young, Jacques; Hardelin, Jean-Pierre; Prevot, Vincent; Dodé, Catherine

    2012-08-01

    Kallmann syndrome (KS) associates congenital hypogonadism due to gonadotropin-releasing hormone (GnRH) deficiency and anosmia. The genetics of KS involves various modes of transmission, including oligogenic inheritance. Here, we report that Nrp1(sema/sema) mutant mice that lack a functional semaphorin-binding domain in neuropilin-1, an obligatory coreceptor of semaphorin-3A, have a KS-like phenotype. Pathohistological analysis of these mice indeed showed abnormal development of the peripheral olfactory system and defective embryonic migration of the neuroendocrine GnRH cells to the basal forebrain, which results in increased mortality of newborn mice and reduced fertility in adults. We thus screened 386 KS patients for the presence of mutations in SEMA3A (by Sanger sequencing of all 17 coding exons and flanking splice sites) and identified nonsynonymous mutations in 24 patients, specifically, a frameshifting small deletion (D538fsX31) and seven different missense mutations (R66W, N153S, I400V, V435I, T688A, R730Q, R733H). All the mutations were found in heterozygous state. Seven mutations resulted in impaired secretion of semaphorin-3A by transfected COS-7 cells (D538fsX31, R66W, V435I) or reduced signaling activity of the secreted protein in the GN11 cell line derived from embryonic GnRH cells (N153S, I400V, T688A, R733H), which strongly suggests that these mutations have a pathogenic effect. Notably, mutations in other KS genes had already been identified, in heterozygous state, in five of these patients. Our findings indicate that semaphorin-3A signaling insufficiency contributes to the pathogenesis of KS and further substantiate the oligogenic pattern of inheritance in this developmental disorder.

  10. SEMA3A, a gene involved in axonal pathfinding, is mutated in patients with Kallmann syndrome.

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    Naresh Kumar Hanchate

    2012-08-01

    Full Text Available Kallmann syndrome (KS associates congenital hypogonadism due to gonadotropin-releasing hormone (GnRH deficiency and anosmia. The genetics of KS involves various modes of transmission, including oligogenic inheritance. Here, we report that Nrp1(sema/sema mutant mice that lack a functional semaphorin-binding domain in neuropilin-1, an obligatory coreceptor of semaphorin-3A, have a KS-like phenotype. Pathohistological analysis of these mice indeed showed abnormal development of the peripheral olfactory system and defective embryonic migration of the neuroendocrine GnRH cells to the basal forebrain, which results in increased mortality of newborn mice and reduced fertility in adults. We thus screened 386 KS patients for the presence of mutations in SEMA3A (by Sanger sequencing of all 17 coding exons and flanking splice sites and identified nonsynonymous mutations in 24 patients, specifically, a frameshifting small deletion (D538fsX31 and seven different missense mutations (R66W, N153S, I400V, V435I, T688A, R730Q, R733H. All the mutations were found in heterozygous state. Seven mutations resulted in impaired secretion of semaphorin-3A by transfected COS-7 cells (D538fsX31, R66W, V435I or reduced signaling activity of the secreted protein in the GN11 cell line derived from embryonic GnRH cells (N153S, I400V, T688A, R733H, which strongly suggests that these mutations have a pathogenic effect. Notably, mutations in other KS genes had already been identified, in heterozygous state, in five of these patients. Our findings indicate that semaphorin-3A signaling insufficiency contributes to the pathogenesis of KS and further substantiate the oligogenic pattern of inheritance in this developmental disorder.

  11. A MALE CASE OF KALLMANN'S SYNDROME : FERTILITY INDUCED BY GONADOTROPIN (hCG/hMG) THERAPY

    OpenAIRE

    Okamoto, Shingo; Mayumi Mimura, Mayumi; Moch, Tadao; Sakamoto, Takemi; Izumi, Yukiko; Matzui, Yuhji; Hosokawa, Akiko; Kuriyama, Shigeki; Fukui, Hiroshi

    1998-01-01

    A 24-year-old male patient with Kallmann's syndrome who fathered two children after gonadotropin therapy is reported here. He was diagnosed with Kallmann's syndrome because of hypothalamic hypogonadism associated with anosmia. The gonadotropin therapy was initiated which involved treatment with human chorionic gonadotropin (hCG) and human menopausal gonadotropin (hMG). After 3 years of treatment, his secondary sexual characteristics developed to near the adult level and sperm were detected in...

  12. Novel FGFR1 and KISS1R Mutations in Chinese Kallmann Syndrome Males with Cleft Lip/Palate

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    Hao Xu

    2015-01-01

    Full Text Available Kallmann syndrome (KS is characterized by isolated hypogonadotropic hypogonadism (IHH with anosmia and is sometimes associated with cleft lip/palate (CLP. In order to describe the clinical features, genetic etiology, and treatment outcome of KS males with CLP, we performed genetic screening for 15 known causal IHH genes (KAL1, FGFR1, NELF, FGF8, CHD7, WDR11, SEMA3A, KISS1R, KISS1, PROKR2, PROK2, TAC3, TACR3, GNRH1, and GNRHR in four KS with CLP patients and six IHH patients without CLP. Two novel heterozygous missense mutations in FGFR1, (NM_001174066: c.776G>A (p.G259E and (NM_001174066: c.358C>T (p.R120C, were identified in a 23-year-old KS male with cleft lip and an 18-year-old KS patient with cleft lip and palate, dental agenesis, and high arched palate, respectively. These two mutations were not presented in their healthy parents and 200 normal controls. One novel heterozygous missense mutation in KISS1R, (NM_032551: c.587C>A (p.P196H, was identified in an 18-year-old KS male with cleft lip and dental agenesis who developed sperm after being treated with gonadotropin. This mutation was also presented in his healthy father and grandfather. These results have implications for the diagnosis, genetic counseling, and treatment of KS and CLP males with mutations in FGFR1 gene.

  13. The prevalence of CHD7 missense versus truncating mutations is higher in patients with Kallmann syndrome than in typical CHARGE patients

    DEFF Research Database (Denmark)

    Marcos, Séverine; Sarfati, Julie; Leroy, Chrystel

    2014-01-01

    CONTEXT: Mutations in CHD7, a gene previously implicated in CHARGE (coloboma, heart defect, choanal atresia, retardation of growth and/or development, genital hypoplasia, ear anomalies) syndrome, have been reported in patients presenting with Kallmann syndrome (KS) or congenital hypogonadotropic...... hypogonadism (CHH). Most mutations causing CHARGE syndrome result in premature stop codons and occur de novo, but the proportion of truncating vs nontruncating mutations in KS and CHH patients is still unknown. OBJECTIVE: The objective of the study was to determine the nature, prevalence, mode of transmission......, and clinical spectrum of CHD7 mutations in a large series of patients. DESIGN: We studied 209 KS and 94 CHH patients. These patients had not been diagnosed with CHARGE syndrome according to the current criteria. We searched for mutations in 16 KS and CHH genes including CHD7. RESULTS: We found presumably...

  14. Kallmann syndrome and ichthyosis: a case of contiguous gene deletion syndrome

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    Irene Berges-Raso

    2017-09-01

    Full Text Available Kallmann syndrome is a genetically heterogeneous form of hypogonadotropic hypogonadism caused by gonadotropin-releasing hormone deficiency and characterized by anosmia or hyposmia due to hypoplasia of the olfactory bulbs; osteoporosis and metabolic syndrome can develop due to longstanding untreated hypogonadism. Kallmann syndrome affects 1 in 10 000 men and 1 in 50 000 women. Defects in 17 genes, including KAL1, have been implicated. Kallmann syndrome can be associated with X-linked ichthyosis, a skin disorder characterized by early onset dark, dry, irregular scales affecting the limb and trunk, caused by a defect of the steroid sulfatase gene (STS. Both KAL1 and STS are located in the Xp22.3 region; therefore, deletions in this region cause a contiguous gene syndrome. We report the case of a 32-year-old man with ichthyosis referred for evaluation of excessive height (2.07 m and weight (BMI: 29.6 kg/m2, microgenitalia and absence of secondary sex characteristics. We diagnosed Kallmann syndrome with ichthyosis due to a deletion in Xp22.3, a rare phenomenon.

  15. De novo SOX10 Nonsense Mutation in a Patient with Kallmann Syndrome, Deafness, Iris Hypopigmentation, and Hyperthyroidism.

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    Wang, Fang; Zhao, Shaoli; Xie, Yanhong; Yang, Wenjun; Mo, Zhaohui

    2018-03-01

    Kallmann syndrome (KS) is a clinically and genetically heterogeneous disorder characterized by hypogonadotropic hypogonadism and olfactory dysfunction. Recently, mutations in SOX10, a well-known causative gene of Waardenburg syndrome (WS), have been identified in a few KS patients with additional developmental defects including hearing loss. However, the understanding of SOX10 mutation associates with KS and other clinical consequences remains fragmentary. A 30-year-old Chinese male patient presented with no pubertal sex development when he was at the age of twelve years. Additionally, he showed anosmia, sensory deafness, and blue irises. Last year, he developed clinical symptoms of hyperthyroidism with a fast heartbeat, heat intolerance and weight loss. Blood examinations revealed low levels of FSH, LH, and testosterone. Thyroid function showed high levels of FT3, FT4 and extremely low level of TSH. Molecular analysis detected a de novo (c.565G>T/p.E189X) mutation in SOX10, which has previously been reported in a patient with WS4 (WS with Hirschsprung). The mutation was predicted to be probably damaging. These results highlight the significance of SOX10 haploinsufficiency as a genetic cause of KS. Importantly, our result implies that the same SOX10 mutation can underlie both typical KS and WS, while the correlation between SOX10 and hyperthyroidism still needs to be clarified in the future. © 2018 by the Association of Clinical Scientists, Inc.

  16. Editorial: X-chromosome-linked Kallmann's syndrome: Pathology at the molecular level

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    Prager, D.; Braunstein, G.D. (Cedars-Sinai Medical Center, Los Angeles, CA (United States))

    1993-04-01

    Kallmann's syndrome or olfactogenital dysplasia refers to a disorder characterized by hypogonadotropic hypogonadism and anosmia or hyposmia which can occur sporadically or in a familial setting. Originally described in 1856, the first familial cases were reported by Kallmann et al., in 1944. Based on segregation analysis of multiple families, three modes of transmission have been documented: X-linked, autosomal dominant with variable penetrance, and autosomal recessive. Kallmann's syndrome occurs in less than 1 in 10,000 male births, with a 5-fold excess of affected males to females, suggesting that the X-linked form is the most frequent. By genetic linkage analysis the X-linked form of Kallmann's syndrome was localized to Xp22.3. This was confirmed by the description of patients with contiguous gene syndromes due to deletions of various portions of the distal short arm of the X-chromosome. Such patients present with complex phenotypes characterized by a combination of Kallmann's syndrome with X-linked icthyosis due to steroid sulfatase deficiency, chondrodysplasia punctata, short stature, and mental retardation. DNA analysis has identified and mapped the genes responsible for these disorders. 10 refs., 1 fig., 1 tab.

  17. Kallmann syndrome and schizophrenia: Is there a relationship?

    NARCIS (Netherlands)

    Verhoeven, W.M.A.; Egger, J.I.M.; Hovens, J.H.; Leeuw, N. de; Hoefsloot, L.H.

    2013-01-01

    Objective: In the early fourties of the past century, Kallmann and coworkers reported on a small number of patients, most of them with normal intelligence, in whom a combination of eunuchoidism and anosmia was present. About two decades later, hypothalamic dysfunction was demonstrated to be

  18. Klinefelter Syndrome (KS): Other FAQs

    Science.gov (United States)

    ... Breaking tasks into small steps Creating opportunities for social interaction and understanding Reminding them to stay focused Michael, ... 2003). PGD in 47,XXY Klinefelter's syndrome patients. Human Reproduction Update , 9 (4), 319-330. Simpson, J. ...

  19. Profiling and functional data on the developing olfactory/GnRH system reveal cellular and molecular pathways essential for this process and potentially relevant for the Kallmann syndrome

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    Giulia eGaraffo

    2013-12-01

    Full Text Available During embryonic development, immature neurons in the olfactory epithelium (OE extend axons through the nasal mesenchyme, to contact projection neurons in the olfactory bulb. Axon navigation is accompanied by migration of the GnRH+ neurons, which enter the anterior forebrain and home in the septo-hypothalamic area. This process can be interrupted at various points and lead to the onset of the Kallmann syndrome (KS, a disorder characterized by anosmia and central hypogonadotropic hypogonadism. Several genes has been identified in human and mice that cause KS or a KS-like phenotype. In mice a set of transcription factors appears to be required for olfactory connectivity and GnRH neuron migration; thus we explored the transcriptional network underlying this developmental process by profiling the OE and the adjacent mesenchyme at three embryonic ages. We also profiled the OE from embryos null for Dlx5, a homeogene that causes a KS-like phenotype when deleted. We identified 20 interesting genes belonging to the following categories: 1 transmembrane adhesion/receptor, 2 axon-glia interaction, 3 scaffold/adapter for signalling, 4 synaptic proteins. We tested some of them in zebrafish embryos: the depletion of five (of six Dlx5 targets affected axonal extension and targeting, while three (of three affected GnRH neuron position and neurite organization. Thus, we confirmed the importance of cell-cell and cell-matrix interactions and identified new molecules needed for olfactory connection and GnRH neuron migration. Using available and newly generated data, we predicted/prioritized putative KS-disease genes, by building conserved co-expression networks with all known disease genes in human and mouse. The results show the overall validity of approaches based on high-throughput data and predictive bioinformatics to identify genes potentially relevant for the molecular pathogenesis of KS. A number of candidate will be discussed, that should be tested in

  20. Kallmann syndrome and deafness: an uncommon combination: A case report and a literature review

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    Nader Salama

    2016-08-01

    Full Text Available Background: Kallmann syndrome (Kal S is an isolated form of hypogonadotrophic hypogonadism in combination with a defect in smell sensation. Depending on the genetic form of the disease, a number of non-reproductive, non-olfactory abnormalities may also be existent. In the present report, we describe a male with Kal S associated with hearing loss, and the successful treatment of his sexual and reproductive defects. Case: A 23-year-old Caucasian man presented with a lifelong lack of erection and ejaculation. The patient reported also anosmia combined with loss of hearing ability. A diagnostic work-up identified the presence of Kal S associated with sensorineural hearing loss. Administration of gonadotrophins regained the erection and a viable-sperm containing ejaculation. Conclusion: Lack of erection and ejaculation are important components of delayed puberty which could lead to diagnosis of Kal S. The existence of a hearing impairment in the reported patient makes the recommendation to screen the hearing ability in Kal S of utmost importance

  1. Kallmann syndrome: 14 novel mutations in KAL1 and FGFR1 (KAL2).

    Science.gov (United States)

    Albuisson, Juliette; Pêcheux, Chistophe; Carel, Jean-Claude; Lacombe, Didier; Leheup, Bruno; Lapuzina, Pablo; Bouchard, Philippe; Legius, Eric; Matthijs, Gert; Wasniewska, Malgorzata; Delpech, Marc; Young, Jacques; Hardelin, Jean-Pierre; Dodé, Catherine

    2005-01-01

    Kallmann syndrome (KAL) combines hypogonadotropic hypogonadism and anosmia. Hypogonadism is due to Gonadotropin Releasing Hormone (GnRH) deficiency and anosmia is related to hypoplasia of the olfactory bulbs. Occasional symptoms include renal agenesis, bimanual synkinesia, cleft lip palate, dental agenesis. KAL is genetically heterogeneous and two genes have so far been identified, namely KAL1 (Xp22.3) and FGFR1/KAL2 (8p12), which underlie the X chromosome-linked form and an autosomal dominant form of the disease, respectively. We studied a cohort of 98 unrelated Caucasian KAL patients. We identified KAL1 mutations in 14 patients, of which 7 (c.3G>A (p.M1?), g.IVS1+1G>T, c.570_571insA (p.R191fsX14), c.784G>C (p.R262P), c.958G>T (p.E320X), c.1651_1654delinsAGCT (p.P551_E552delinsSX), c.1711T>A (p.W571R)) have not been previously reported. In addition, we found FGFR1 mutations in 7 patients, namely c.303G>A (p.V102I), C.385A>C (p.D129A), c.810G>A (p.V273M), c.1093_1094delAG (p.R365fsX41), c.1561G>A (p.A520T), c.1836_1837insT (p.Y613fsX42), c.2190C>G (p.Y730X), all of which were novel mutations. In this study, unilateral renal agenesis and bimanual synkinesia were exclusively found associated with KAL1mutations, cleft palate and dental agenesia with FGFR1mutations. (c) 2004 Wiley-Liss, Inc.

  2. Delayed puberty, eroticism, and sense of smell: A psychological study of hypogonadotropinism, osmatic and anosmatic (Kallmann's syndrome).

    Science.gov (United States)

    Bobrow, N A; Money, J; Lewis, V G

    1971-12-01

    In 13 male patients with a diagnosis of hypogonadotropic hypogonadism, associated in five cases with verified hyposmia (Kallmann's syndrome), social development and participation were delayed. The patients associated the lack of visible signs of male puberty with their delayed sociosexual maturation. However, the evidence of physical maturation after treatment did not have the ameliorative effect on their social behavior one would have liked to predict. Dating behavior was limited before and after treatment for all the patients, and sexual interest was low even for the three married ones. The experience of falling in love was notably absent in the relationships described. Two characteristic social reaction patterns were social introversion and hostile rejection of agemates. Intellectual functioning and the incidence of personality pathology were not diagnostically noteworthy. None of the patients had any problems with gender identity except for erotic apathy which extended to masturbation. There was an absence of homosexuality. Possibly hypogonadotropic patients have a primary defect of the hypothalamus, manifesting itself as a dysfunction of the behavioral concomitants of puberty as well as a failure of hormonal puberty.

  3. [Social cognition disorders in Klinefelter syndrome: A specific phenotype? (KS)].

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    Babinet, M-N; Rigard, C; Peyroux, É; Dragomir, A-R; Plotton, I; Lejeune, H; Demily, C

    2017-10-01

    The Klinefelter syndrome (KS) is a genetic condition characterized by an X supernumerary sex chromosome in males. The syndrome is frequently associated with cognitive impairment. Indeed, the different areas of the executive sphere can be affected such as inhibition, cognitive flexibility but also attentional and visual-spatial domain. Social cognition disorders, predominantly on emotional recognition processes, have also been documented. In addition, the syndrome may be associated with psychiatric symptoms. Our study aims to characterize of the various components of social cognition in the SK: facial emotional recognition, theory of mind and attributional style. For this two groups (SK group versus control group) of participants (n=16) matched for age and sociocultural level were recruited. Participants with intellectual disabilities, psychiatric or neurological disorders were excluded. Three social cognition tests were available: the TREF, the MASC, the AIHQ. Neurocognitive functions were assessed by the fNart, the subtest "logical memory" of the MEM-III, the subtests of the two VOSP battery, the d2, the TMT and the Stroop test. The SK group had specific social cognition disorders in comparison to the control group. Two emotions in particular were less well recognized: fear and contempt. In addition, the SK group had significantly lower results in theory of mind. Regarding the hostile attribution bias, no significant difference was found. Finally, the results showed correlations between specific attentional disorders and facial emotional recognition. Our study emphasizes social cognition disorders in SK. These disorders could be considered as a phenotypic trait in the syndrome. The interest of better characterizing the cognitive phenotype of genetic disorders that can affect the neurodevelopment is to offer specific cognitive remediation strategies. Copyright © 2016. Published by Elsevier Masson SAS.

  4. The pulmonary histopathology of anti-KS transfer RNA synthetase syndrome.

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    Schneider, Frank; Aggarwal, Rohit; Bi, David; Gibson, Kevin; Oddis, Chester; Yousem, Samuel A

    2015-01-01

    The clinical spectrum of the antisynthetase syndromes (AS) has been poorly defined, although some frequently present with pulmonary manifestations. The anti-KS anti-asparaginyl-transfer RNA synthetase syndrome is one in which pulmonary interstitial lung disease is almost always present and yet the histopathologic spectrum is not well described. To define the morphologic manifestations of pulmonary disease in those patients with anti-KS antiasparaginyl syndrome. We reviewed the connective tissue disorder registry of the University of Pittsburgh and identified those patients with anti-KS autoantibodies who presented with interstitial lung disease and had surgical lung biopsies. The 5 patients with anti-KS antisynthetase syndrome were usually women presenting with dyspnea and without myositis, but with mechanic's hands (60%) and Raynaud phenomenon (40%). They most often presented with a usual interstitial pneumonia pattern of fibrosis (80%), with the final patient displaying organizing pneumonia. Pulmonary interstitial lung disease is a common presentation in patients with the anti-KS-antisynthetase syndrome, who are often women with rather subtle or subclinical connective tissue disease, whereas the literature emphasizes the nonspecific interstitial pneumonia pattern often diagnosed clinically. Usual interstitial pneumonia and organizing pneumonia patterns of interstitial injury need to be added to this clinical differential diagnosis.

  5. Associação singular de síndrome de Kallmann e cisto aracnóide da fossa média: relato de caso Singular association of Kallmann's syndrome and arachnoid cyst of middle fossa: case report

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    Yvens B. Fernandes

    1995-09-01

    disabilities. On physical examination he exhibited mild eunuchoid aspect, 162 cm height (z score = -2,17, pubertal development on stage G II, P II, and 4 cm5 testis. Laboratory investigation revealed pre-pubertal levels of testosterone and normal results of the combined test of anterior pituitary function, except for in GnRH acute and prolonged test. Brain CT showed an arachnoid cyst on left middle fossa with expansion to suprasellar cisterna. He was diagnosed as having hypogonadotrophic hypogonadism secondary to compression by the cyst, and a cyst-peritoneal derivation was performed. After surgery there was no improvement of the pubertal state and bilateral anosmia was discovered, so Kallmann's syndrome was then diagnosed and was confirmed by MRI, even though the hormonal results are not totally matched with the refered syndrome. We did not find in the literature any description of the association between Kallmann's syndrome and arachnoid cyst and we believe that in this case the results of the hormonal measurement may be due to such association that provoked an additional hypophysis dysfunction.

  6. Genetics Home Reference: Kallmann syndrome

    Science.gov (United States)

    ... in other genes, it often has an autosomal dominant pattern of inheritance, which means one copy of an altered gene in each cell is sufficient to cause the disorder. In some cases, an affected person inherits the mutation from an affected mother or father . Other cases result from new mutations in the ...

  7. Unusual presentation of Kallmannn syndrome with contiguous gene deletion in three siblings of a family

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    Sri Venkat Madhu

    2012-01-01

    Full Text Available We report the case of 3 brothers aged 34, 24, and 22 years, unmarried, who presented to our endocrinology clinic with absence of secondary sexual characters. There was no such history in other siblings, but their maternal uncle had similar complaints. On examination, all 3 had pre-pubertal appearance, voice, and genitalia along with anosmia and bimanual synkinesia. Cryptorchidism was noticed in 2 while third person had small hypoplastic testes. It was also noted that all 3 patients had icthyosis mainly involving trunk, back, and limbs. The hormonal assays were consistent with isolated hypogonadotrophic hypogonadism. IQ testing revealed mental retardation in the 2 patients. Ultrasound showed ectopic right kidney in one patient, atrophic right kidney in the second patient while the third patient had normal kidneys. MRI brain of all the patients showed poorly visualized olfactory tract and bulb. Kallmann syndrome (KS was diagnosed based on hormonal evaluation and MRI results. Of the four types of KS: Synkinesia, renal anomaly, and X-linked pedigree pattern in our patients pointed towards X-linked type 1 KS as the possible cause. But, icthyosis and mental retardation are not usual presentation of type 1 KS. They are usually seen as a result of contiguous gene deletion of KAL1, steroid sulfatase (STS, and mental retardation (MRX gene on X chromosome. Hence, the possible gene defect in our cases is inherited defect in contiguous gene deletion. The contiguous gene deletion as the cause of KS in 3 patients of same family is very rare and worth reporting. Also, the significance of phenotype-genotypic association in Kallmann syndrome is discussed

  8. Measurements of the branching fractions for D+→KS0KS0K+, KS0KS0π+ and D0→KS0KS0, KS0KS0KS0

    Directory of Open Access Journals (Sweden)

    M. Ablikim

    2017-02-01

    Full Text Available By analyzing 2.93fb−1 of data taken at the ψ(3770 resonance peak with the BESIII detector, we measure the branching fractions for the hadronic decays D+→KS0KS0K+, D+→KS0KS0π+, D0→KS0KS0 and D0→KS0KS0KS0. They are determined to be B(D+→KS0KS0K+=(2.54±0.05stat.±0.12sys.×10−3, B(D+→KS0KS0π+=(2.70±0.05stat.±0.12sys.×10−3, B(D0→KS0KS0=(1.67±0.11stat.±0.11sys.×10−4 and B(D0→KS0KS0KS0=(7.21±0.33stat.±0.44sys.×10−4, where the second one is measured for the first time and the others are measured with significantly improved precision over the previous measurements.

  9. Reproduction, Smell and Neurodevelopmental disorders: Genetic defects in different hypogonadotropic hypogonadal syndromes.

    Directory of Open Access Journals (Sweden)

    Hernan G VALDES-SOCIN

    2014-07-01

    Full Text Available The neuroendocrine control of reproduction in mammals is governed by a neural hypothalamic network of nearly 1500 gonadotropin-releasing hormone (GnRH secreting neurons that modulate the activity of the reproductive axis across life. Congenital hypogonadotropic hypogonadism (HH is a clinical syndrome that is characterized by partial or complete pubertal failure. HH may result from inadequate hypothalamic GnRH axis activation, or a failure of pituitary gonadotropin secretion/effects. In man, several genes that participate in olfactory and GnRH neuronal migration are thought to interact during the embryonic life. A growing number of mutations in different genes are responsible for congenital HH. Based on the presence or absence of olfaction dysfunction, HH is divided in two syndromes: HH with olfactory alterations (Kallmann syndrome and idiopathic hypogonadotropic hypogonadism (IHH with normal smell (normosmic IHH. Kallmann syndrome (KS is a heterogeneous disorder affecting 1 in 5000 males, with a 3-5 fold of males over females. KS is associated with mutations in KAL1, FGFR1/FGF8, FGF17, IL17RD, PROK2/PROKR2, NELF, CHD7, HS6ST1, FLRT3, SPRY4, DUSP6, SEMA3A, NELF and WDR11 genes that are related to defects in neuronal migration. These reproductive and olfactory deficits include a variable non reproductive phenotype, including sensorineural deafness, coloboma, bimanual synkinesis, craniofacial abnormalities and/or renal agenesis. Interestingly, defects in PROKR2, FGFR1, FGF8, CHD7, DUSP6, and WDR11 genes are also associated with normosmic IHH, whereas mutations in KISS1/KISSR, TAC3/TACR3, GNRH1/GNRHR, LEP/LEPR, HESX1, FSHB and LHB are only present in patients with normosmic IHH. In this paper, we summarize the reproductive, neurodevelopmental and genetic aspects of HH in human pathology.

  10. Geomagnetic Kn, Ks, Km Indices

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — A full description of the indices Kn, Ks, Km is given in a monography,"Indices Kn, Ks et Km, 1964-1967", edited in 1968 by the Centre National de la Recherche...

  11. Neurobehavioral and Psychosocial Issues in Klinefelter Syndrome

    Science.gov (United States)

    Geschwind, Daniel H.; Dykens, Elisabeth

    2004-01-01

    Klinefelter Syndrome (KS) is a relatively common (1/500 to 1/1,000) genetic syndrome caused by an extra X chromosome in males, leading to an XXY karyotype. In most cases, the physical and neurobehavioral characteristics of KS are relatively mild, and KS is not usually associated with moderate or severe mental retardation. However, KS is often…

  12. El síndrome de Kallmann. Correlación fenotipo-genotípica.

    OpenAIRE

    García Piñero, Alfonso José

    2015-01-01

    El Síndrome de Kallmann (SK) es una enfermedad genética del desarrollo, que asocia un hipogonadismo hipogonadotrópico (HH) congénito con pérdida total (anosmia) o parcial (hiposmia) del olfato. La alteración olfativa es secundaria a la aplasia o hipoplasia de las estructuras olfatorias (bulbos, tractos y surcos cerebrales olfatorios). El HH se debe a la deficiencia en la hormona liberadora de gonadotropinas hipotalámica (GnRH) como resultado del fallo en la migración de las neuronas productor...

  13. Eugenics ideals, racial hygiene, and the emigration process of German-American neurogeneticist Franz Josef Kallmann (1897-1965).

    Science.gov (United States)

    Pow, Stephen; Stahnisch, Frank W

    2016-01-01

    Biological psychiatry in the early twentieth century was based on interrelated disciplines, such as neurology and experimental biology. Neuropsychiatrist Franz Josef Kallmann (1897-1965) was a product of this interdisciplinary background who showed an ability to adapt to different scientific contexts, first in the field of neuromorphology in Berlin, and later in New York. Nonetheless, having innovative ideas, as Kallmann did, could be an ambiguous advantage, since they could lead to incommensurable scientific views and marginalization in existing research programs. Kallmann followed his Dr. Med. degree (1919) with training periods at the Charité Medical School in Berlin under psychiatrist Karl Bonhoeffer (1868-1948). Subsequently, he collaborated with Ernst Ruedin (1874-1952), investigating sibling inheritance of schizophrenia and becoming a protagonist of genetic research on psychiatric conditions. In 1936, Kallmann was forced to immigrate to the USA where he published The Genetics of Schizophrenia (1938), based on data he had gathered from the district pathological institutes of Berlin's public health department. Kallmann resumed his role as an international player in biological psychiatry and genetics, becoming president (1952) of the American Society of Human Genetics and Director of the New York State Psychiatric Institute in 1955. While his work was well received by geneticists, the idea of genetic differences barely took hold in American psychiatry, largely because of émigré psychoanalysts who dominated American clinical psychiatry until the 1960s and established a philosophical direction in which genetics played no significant role, being regarded as dangerous in light of Nazi medical atrocities. After all, medical scientists in Nazi Germany had been among the social protagonists of racial hygiene which, under the aegis of Nazi philosophies, replaced medical genetics as the basis for the ideals and application of eugenics.

  14. Üks vapper Jodie / Jaanus Noormets

    Index Scriptorium Estoniae

    Noormets, Jaanus

    2007-01-01

    Mängufilm "Üks vapper naine" ("The Brave One") : režissöör Neil Jordan : peaosas Jodie Foster : Ameerika Ühendriigid - Austraalia 2007. Lisatud nimekiri "Jodie Fosteri menukamad filmid Põhja-Ameerikas"

  15. Update on the slow delayed rectifier potassium current (I(Ks)): role in modulating cardiac function.

    Science.gov (United States)

    Liu, Zhenzhen; Du, Lupei; Li, Minyong

    2012-01-01

    The slow delayed rectifier current (I(Ks)) is the slow component of cardiac delayed rectifier current and is critical for the late phase repolarization of cardiac action potential. This current is also an important target for Sympathetic Nervous System (SNS) to regulate the cardiac electivity to accommodate to heart rate alterations in response to exercise or emotional stress and can be up-regulated by β- adrenergic or other signal molecules. I(Ks) channel is originated by the co-assembly of pore-forming KCNQ1 α-subunit and accessory KCNE1 β-subunit. Mutations in any subunit can bring about severe long QT syndrome (LQT-1, LQT-5) as characterized by deliquium, seizures and sudden death. This review summarizes the normal physiological functions and molecular basis of I(Ks) channels, as well as illustrates up-to-date development on its blockers and activators. Therefore, the current extensive survey should generate fundamental understanding of the role of I(Ks) channel in modulating cardiac function and donate some instructions to the progression of I(Ks) blockers and activators as potential antiarrhythmic agents or pharmacological tools to determine the physiological and pathological function of I(Ks).

  16. "Autograph" in the KS3 Classroom

    Science.gov (United States)

    Catley, Alan

    2006-01-01

    In this article, the author shows some simple examples of ways in which "Autograph" can enhance learning in the KS3 curriculum. He began using version 2 with A-level students to help them visualise concepts in pure mathematics. He has "Autograph" projected to the front board to keep learners focused on mathematical activity…

  17. Analysis of KROTOS KS-2 and KS-4 steam explosion experiments with TEXAS-VI

    Energy Technology Data Exchange (ETDEWEB)

    Chen, Ronghua, E-mail: rhchen@mail.xjtu.edu.cn [State Key Laboratory of Multiphase Flow in Power Engineering, School of Nuclear Science and Technology, Xi’an Jiaotong University, Xi’an 710049 (China); Wang, Jun [Nuclear Engineering and Engineering Physics, College of Engineering, University of Wisconsin Madison, WI 53706 (United States); Su, G.H.; Qiu, Suizheng [State Key Laboratory of Multiphase Flow in Power Engineering, School of Nuclear Science and Technology, Xi’an Jiaotong University, Xi’an 710049 (China); Corradini, M.L., E-mail: Corradini@engr.wisc.edu [Nuclear Engineering and Engineering Physics, College of Engineering, University of Wisconsin Madison, WI 53706 (United States)

    2016-12-01

    Highlights: • The KS-2 and KS-4 steam explosion experiments were analyzed by TEXAS-VI. • The coarse mixing status up to the explosion triggering time was well predicted by TEXAS-VI. • The predicted dynamic explosion pressure was in good agreement with the experimental results. - Abstract: TEXAS-VI is a transient, three-field, one-dimensional mechanistic model for the steam explosion phenomena. A fuel solidification model and associated fragmentation criteria of the solidifying particle for both the mixing phase and explosion phase were developed and incorporated into TEXAS-VI to account for solidification. In the present study, TEXAS-VI was used to analyze the KS-2 and KS-4 steam explosion experiments, which were performed in the KROTOS facility as part of the OECD-SERENA-2 program. In the simulation, the KROTOS experimental facility was modeled as Eulerian control volumes based on the facility geometry. The molten corium jet was divided up into a series of LaGrangian master particles equal to the initial jet diameter. Both the mixing phase and the explosion phase of the experiments were simulated by TEXAS-VI. Comparison to test data indicates that the fuel jet kinematics and the vapor volume during the mixing phase were well predicted by TEXAS-VI. The TEXAS-VI prediction of the dynamic explosion pressure at different axial locations in the test was also in good agreement with the experimental results. The maximum pressure of KS-2 and KS-4 predicted by TEXAS-VI were 16.7 MPa and 41.9 MPa, respectively. The KS-4 maximum steam explosion pressure predicted by TEXAS-VI was higher than that of KS-2, which was consistent with experiment observation. The observed differences of the dynamic explosion pressure between the KS-2 and KS-4 experiments were also successfully simulated by TEXAS-VI. This suggests that TEXAS-VI is able to analyze the effect of prototypic melt compositions on the steam explosion phenomena. Additional benchmarking and evaluations are ongoing.

  18. Drugs that may provoke Kounis syndrome.

    Science.gov (United States)

    Rodrigues, Maria Catarina Luís; Coelho, Daniela; Granja, Cristina

    2013-01-01

    Kounis Syndrome (KS) is the contemporary occurrence of Acute Coronary Syndromes (ACS) with an allergic or hypersensitivity reaction. This syndrome has been reported in association with a variety of drugs, food, insect stings, environmental exposures and medical conditions. Cases of KS seem to be more often encountered in everyday clinical practice than anticipated. It is believed that the lack of awareness of this association may lead to underreporting. We report a case of KS secondary to diclofenac intake.

  19. Normal bone mineral content but unfavourable muscle/fat ratio in Klinefelter syndrome

    DEFF Research Database (Denmark)

    Aksglaede, L.; Mølgaard, Christian; Skakkebaek, N.E.

    2008-01-01

    OBJECTIVE: To evaluate body composition and bone mineral content (BMC) in children and adolescents with Klinefelter syndrome (KS). DESIGN: Retrospective cross-sectional study. SETTING: Tertiary endocrine clinic at the University Hospital, Copenhagen. PATIENTS: Eighteen untreated boys with KS...

  20. Quality of life is reduced in patients with Klinefelter syndrome on androgen replacement therapy

    NARCIS (Netherlands)

    de Ronde, Willem; de Haan, Anne; Drent, Madeleine L

    CONTEXT: Klinefelter syndrome (KS) is a genetic disorder, characterized by an XXY karyotype, hypergonadotrophic hypogonadism and infertility, which affects approximately 1 in 700 men. KS has also been associated with neuropsychological alterations. OBJECTIVE: To investigate whether quality of life

  1. How Do Health Care Providers Diagnose Rett Syndrome?

    Science.gov (United States)

    ... Research Information Find a Study Resources and Publications Klinefelter Syndrome (KS) Condition Information NICHD Research Information Find a ... Rett syndrome occur with another genetic condition called Klinefelter syndrome , in which a boy has two X chromosomes ...

  2. The Psychoeducational Profile of Boys with Klinefelter Syndrome.

    Science.gov (United States)

    Rovet, Joanne; And Others

    1996-01-01

    This article integrates the literature on intelligence and achievement outcomes in boys with Klinefelter syndrome (KS). It reports results of a study following 36 boys with KS and 33 sibling controls. Boys with KS demonstrated verbal cognitive deficits and significant underachievement in reading, spelling, and arithmetic, which increased with age.…

  3. Measurements of time-dependent CP violation in B0→ωKS0, f0(980)KS0, KS0π0 and K+K-KS0 decays

    Science.gov (United States)

    Chao, Y.; Chen, K.-F.; Miyake, H.; Tajima, O.; Trabelsi, K.; Abe, K.; Abe, K.; Adachi, I.; Aihara, H.; Anipko, D.; Bakich, A. M.; Barberio, E.; Bitenc, U.; Bizjak, I.; Blyth, S.; Bondar, A.; Bračko, M.; Browder, T. E.; Chang, M.-C.; Chang, P.; Chen, A.; Chen, W. T.; Cheon, B. G.; Chistov, R.; Choi, Y.; Choi, Y. K.; Cole, S.; Dalseno, J.; Danilov, M.; Dash, M.; Dragic, J.; Drutskoy, A.; Eidelman, S.; Fratina, S.; Gabyshev, N.; Golob, B.; Ha, H.; Haba, J.; Hara, K.; Hara, T.; Hastings, N. C.; Hayashii, H.; Hazumi, M.; Heffernan, D.; Higuchi, T.; Hokuue, T.; Hoshi, Y.; Hou, W.-S.; Hsiung, Y. B.; Iijima, T.; Ikado, K.; Inami, K.; Ishikawa, A.; Ishino, H.; Itoh, R.; Iwasaki, M.; Iwasaki, Y.; Kaji, H.; Kang, J. H.; Kapusta, P.; Kawai, H.; Kawasaki, T.; Kim, H. J.; Kim, H. O.; Kim, Y. J.; Kinoshita, K.; Korpar, S.; Križan, P.; Krokovny, P.; Kulasiri, R.; Kumar, R.; Kuo, C. C.; Kuzmin, A.; Kwon, Y.-J.; Lee, M. J.; Lesiak, T.; Limosani, A.; Lin, S.-W.; Liventsev, D.; Matsumoto, T.; McOnie, S.; Miyabayashi, K.; Miyata, H.; Miyazaki, Y.; Mizuk, R.; Mohapatra, D.; Moloney, G. R.; Nakahama, Y.; Nakano, E.; Nakao, M.; Natkaniec, Z.; Nishida, S.; Nitoh, O.; Ogawa, S.; Okuno, S.; Olsen, S. L.; Onuki, Y.; Ozaki, H.; Pakhlov, P.; Pakhlova, G.; Park, C. W.; Pestotnik, R.; Piilonen, L. E.; Sakai, Y.; Satoyama, N.; Schietinger, T.; Schneider, O.; Schwartz, A. J.; Seidl, R.; Senyo, K.; Sevior, M. E.; Shapkin, M.; Shibuya, H.; Singh, J. B.; Somov, A.; Soni, N.; Stanič, S.; Starič, M.; Stoeck, H.; Sumisawa, K.; Sumiyoshi, T.; Suzuki, S.; Takasaki, F.; Tamai, K.; Tanaka, M.; Taylor, G. N.; Teramoto, Y.; Tian, X. C.; Tikhomirov, I.; Tsukamoto, T.; Uehara, S.; Ueno, K.; Unno, Y.; Uno, S.; Ushiroda, Y.; Usov, Y.; Varner, G.; Varvell, K. E.; Villa, S.; Vinokurova, A.; Wang, C. H.; Watanabe, Y.; Won, E.; Yabsley, B. D.; Yamaguchi, A.; Yamashita, Y.; Yamauchi, M.; Yusa, Y.; Zhilich, V.; Zhulanov, V.; Zupanc, A.

    2007-11-01

    We present measurements of time-dependent CP asymmetries in B0→ωKS0, f0(980)KS0, KS0π0 and K+K-KS0 decays based on a sample of 535×106 BB¯ pairs collected at the Υ(4S) resonance with the Belle detector at the KEKB energy-asymmetric e+e- collider. One neutral B meson is fully reconstructed in one of the specified decay channels, and the flavor of the accompanying B meson is identified from its decay products. CP-violation parameters for each of the decay modes are obtained from the asymmetries in the distributions of the proper-time intervals between the two B decays.

  4. INTEGRAL reports renewed activity from KS 1741-293

    DEFF Research Database (Denmark)

    Chenevez, Jérôme; Kuulkers, E.; Alfonso-Garzón, J.

    2010-01-01

    The low-mass X-ray binary and burster source KS 1741-293 has been detected during recent INTEGRAL Galactic bulge (see ATel #438) observations by the JEM-X instrument. On February 25, 2010, between UTC 13:04 and 14:08, KS 1741-293 was detected at a 3-10 keV flux of 9 +/- 4 mCrab, and an upper limi...

  5. Minimum K-S estimator using PH-transform technique

    Directory of Open Access Journals (Sweden)

    Somchit Boonthiem

    2016-07-01

    Full Text Available In this paper, we propose an improvement of the Minimum Kolmogorov-Smirnov (K-S estimator using proportional hazards transform (PH-transform technique. The data of experiment is 47 fire accidents data of an insurance company in Thailand. This experiment has two operations, the first operation, we minimize K-S statistic value using grid search technique for nine distributions; Rayleigh distribution, gamma distribution, Pareto distribution, log-logistic distribution, logistic distribution, normal distribution, Weibull distribution, lognormal distribution, and exponential distribution and the second operation, we improve K-S statistic using PHtransform. The result appears that PH-transform technique can improve the Minimum K-S estimator. The algorithms give better the Minimum K-S estimator for seven distributions; Rayleigh distribution, logistic distribution, gamma distribution, Pareto distribution, log-logistic distribution, normal distribution, Weibull distribution, log-normal distribution, and exponential distribution while the Minimum K-S estimators of normal distribution and logistic distribution are unchanged

  6. Macrostructural abnormalities in Korsakoff syndrome compared with uncomplicated alcoholism.

    Science.gov (United States)

    Pitel, A-L; Chételat, G; Le Berre, A P; Desgranges, B; Eustache, F; Beaunieux, H

    2012-04-24

    To distinguish, in patients with Korsakoff syndrome (KS), the structural brain abnormalities shared with alcoholic patients without KS (AL), from those specific to KS. MRI data were collected in 11 alcoholic patients with KS, 34 alcoholic patients without KS, and 25 healthy control subjects (CS). Gray and white matter volumes were compared in the 3 groups using a voxel-based approach. A conjunction analysis indicated a large pattern of shared gray and white matter volume deficits in AL and KS. There were graded effects of volume deficits (KS < AL < CS) in the medial portion of the thalami, hypothalamus (mammillary bodies), left insula, and genu of the corpus callosum. Abnormalities in the left thalamic radiation were observed only in KS. Our results indicate considerable similarities in the pattern of gray and white matter damage in AL and KS. This finding confirms the widespread neurotoxic effect of chronic alcohol consumption. Only a few cerebral regions, including the medial thalami, mammillary bodies, and corpus callosum, were more severely damaged in KS than in AL. The continuum of macrostructural damage from AL to KS is therefore restricted to key brain structures. Longitudinal investigations are required to determine whether alcoholic patients with medial thalamic volumes that are comparable to those of patients with KS are at increased risk of developing KS.

  7. Kaposi sarcoma and lymphadenopathy syndrome: limitations of abdominal CT in acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Moon, K.L. Jr.; Federle, M.P.; Abrams, D.I.; Volberding, P.; Lewis, B.J.

    1984-01-01

    Abdominal computed tomography (CT) was performed in 31 patients with Kaposi sarcoma (KS) related to acquired immunodeficiency syndrome (AIDS), three patients with classic KS, and 12 patients with the newly described lymphadenopathy syndrome (LNS). The frequency, distribution, and appearance of lymphadenopathy and splenomegaly were similar in the AIDS-related KS and LNS groups. Rectal and perirectal disease was identified in 86% of homosexual men studied; rectal KS could not be distinguished from proctitis on CT criteria alone. No CT abnormalities were seen in patients with classic KS. The CT demonstration of retroperitoneal, mesenteric, or pelvic adenopathy or of rectal or perirectal disease in patients with AIDS-related KS is not necessarily indicative of widespread involvement with the disease

  8. How Do Health Care Providers Diagnose Klinefelter Syndrome?

    Science.gov (United States)

    ... Email Print How do health care providers diagnose Klinefelter syndrome (KS)? The only way to confirm the presence ... in 166 boys, adolescents and adults with nonmosaic Klinefelter syndrome: A Copenhagen experience. Acta Paediatrica , Jun;100(6), ...

  9. MSD grant läks Narva / MSD Teaduskeskus

    Index Scriptorium Estoniae

    2010-01-01

    MSD (Merck Sharpe & Dohme) regionaalsete grantide komitee kuulutas välja konkursi võitja, selleks osutus Narva MTÜ Rehabilitatsioonikeskus "Sind ei jäeta üksi". 2,4 mln kroonine grant on mõeldud noortele HIV-i ja narkootikumide alaseks ennetustööks Ida-Virumaal

  10. 76 FR 13966 - Television Broadcasting Services; Topeka, KS

    Science.gov (United States)

    2011-03-15

    ... FEDERAL COMMUNICATIONS COMMISSION 47 CFR Part 73 [MB Docket No. 11-33, RM-11623; DA 11-406] Television Broadcasting Services; Topeka, KS AGENCY: Federal Communications Commission. ACTION: Proposed rule... 73 Television, Television broadcasting. Federal Communications Commission. Barbara A. Kreisman, Chief...

  11. Double KS0 photoproduction off the proton at CLAS

    Science.gov (United States)

    Chandavar, S.; Goetz, J. T.; Hicks, K.; Keller, D.; Kunkel, M. C.; Paolone, M.; Weygand, D. P.; Adhikari, K. P.; Adhikari, S.; Akbar, Z.; Ball, J.; Balossino, I.; Barion, L.; Bashkanov, M.; Battaglieri, M.; Bedlinskiy, I.; Biselli, A. S.; Briscoe, W. J.; Brooks, W. K.; Burkert, V. D.; Cao, F.; Carman, D. S.; Celentano, A.; Charles, G.; Chetry, T.; Ciullo, G.; Clark, L.; Cole, P. L.; Contalbrigo, M.; Crede, V.; D'Angelo, A.; Dashyan, N.; De Vita, R.; De Sanctis, E.; Defurne, M.; Deur, A.; Djalali, C.; Dupre, R.; Egiyan, H.; El Alaoui, A.; El Fassi, L.; Eugenio, P.; Fedotov, G.; Filippi, A.; Fradi, A.; Gavalian, G.; Ghandilyan, Y.; Gilfoyle, G. P.; Girod, F. X.; Glazier, D. I.; Gohn, W.; Golovatch, E.; Gothe, R. W.; Griffioen, K. A.; Guidal, M.; Guo, L.; Hafidi, K.; Hakobyan, H.; Hanretty, C.; Harrison, N.; Hattawy, M.; Heddle, D.; Holtrop, M.; Ilieva, Y.; Ireland, D. G.; Isupov, E. L.; Jenkins, D.; Johnston, S.; Joo, K.; Joosten, S.; Kabir, M. L.; Khachatryan, G.; Khachatryan, M.; Khandaker, M.; Kim, W.; Klein, A.; Klein, F. J.; Kubarovsky, V.; Lanza, L.; Lenisa, P.; Livingston, K.; MacGregor, I. J. D.; Markov, N.; McCracken, M. E.; McKinnon, B.; Meyer, C. A.; Mineeva, T.; Mokeev, V.; Movsisyan, A.; Munoz Camacho, C.; Nadel-Turonski, P.; Niccolai, S.; Niculescu, G.; Osipenko, M.; Ostrovidov, A. I.; Paremuzyan, R.; Park, K.; Pasyuk, E.; Phelps, W.; Pogorelko, O.; Price, J. W.; Prok, Y.; Protopopescu, D.; Raue, B. A.; Ripani, M.; Riser, D.; Ritchie, B. G.; Rizzo, A.; Rosner, G.; Sabatié, F.; Salgado, C.; Schumacher, R. A.; Sharabian, Y. G.; Simonyan, A.; Skorodumina, Iu.; Sokhan, D.; Smith, G. D.; Sparveris, N.; Stepanyan, S.; Strakovsky, I. I.; Strauch, S.; Ungaro, M.; Voutier, E.; Wei, X.; Zachariou, N.; Zhang, J.; Zhao, Z. W.; CLAS Collaboration

    2018-02-01

    The f0(1500 ) meson resonance is one of several contenders to have significant mixing with the lightest glueball. This resonance is well established from several previous experiments. Here we present the first photoproduction data for the f0(1500 ) via decay into the KS0KS0 channel using the CLAS detector. The reaction γ p →fJp →KS0KS0p , where J =0 ,2 , was measured with photon energies from 2.7-5.1 GeV. A clear peak is seen at 1500 MeV in the background subtracted invariant mass spectra of the two kaons. This is enhanced if the measured four-momentum transfer to the proton target is restricted to be less than 1.0 GeV2. By comparing data with simulations, it can be concluded that the peak at 1500 MeV is produced primarily at low t , which is consistent with a t -channel production mechanism.

  12. Ericsson Euroopas üks suuremaid teadus- ja arendustööle kulutajaid / Annika Matson

    Index Scriptorium Estoniae

    Matson, Annika, 1976-

    2004-01-01

    USA majandusajakiri BusinessWeek koostas kolm edetabelit firmadest, mis kulutavad enim raha teadus- ja arendustööks. Edetabeli kohaselt investeerib Ericsson Euroopa telekommunikatsioonitehnoloogia firmadest enim teadus- ja arendustööks. Diagramm

  13. Short QTc Interval in Males with Klinefelter Syndrome-Influence of CAG Repeat Length, Body Composition, and Testosterone Replacement Therapy

    DEFF Research Database (Denmark)

    Jørgensen, Inger Norlyk; Skakkebaek, Anne; Andersen, Niels Holmark

    2015-01-01

    BackgroundKlinefelter syndrome (KS) is a sex chromosomal aneuploidy (47,XXY) affecting 1/660 males. Based on findings in Turner syndrome, we hypothesized that electrocardiogram (ECG) abnormalities would be present in males with KS. ObjectiveTo investigate ECGs in males with KS and compare...... syndrome was determined in participants with a QTc ... interval comparable to controls. No mutations in genes related to short QT syndrome were found. ConclusionWe found short QTc interval in males with KS, with further shortening of the QTc interval by T. These results suggest that genes on the X chromosome could be involved in regulation of the QTc interval...

  14. Search for a pentaquark decaying to pKS0

    Science.gov (United States)

    FOCUS Collaboration; Link, J. M.; Yager, P. M.; Anjos, J. C.; Bediaga, I.; Castromonte, C.; Machado, A. A.; Magnin, J.; Massafferri, A.; de Miranda, J. M.; Pepe, I. M.; Polycarpo, E.; Dos Reis, A. C.; Carrillo, S.; Casimiro, E.; Cuautle, E.; Sánchez-Hernández, A.; Uribe, C.; Vázquez, F.; Agostino, L.; Cinquini, L.; Cumalat, J. P.; Frisullo, V.; O'Reilly, B.; Segoni, I.; Stenson, K.; Butler, J. N.; Cheung, H. W. K.; Chiodini, G.; Gaines, I.; Garbincius, P. H.; Garren, L. A.; Gottschalk, E.; Kasper, P. H.; Kreymer, A. E.; Kutschke, R.; Wang, M.; Benussi, L.; Bertani, M.; Bianco, S.; Fabbri, F. L.; Pacetti, S.; Zallo, A.; Reyes, M.; Cawlfield, C.; Kim, D. Y.; Rahimi, A.; Wiss, J.; Gardner, R.; Kryemadhi, A.; Chung, Y. S.; Kang, J. S.; Ko, B. R.; Kwak, J. W.; Lee, K. B.; Cho, K.; Park, H.; Alimonti, G.; Barberis, S.; Boschini, M.; Cerutti, A.; D'Angelo, P.; Dicorato, M.; Dini, P.; Edera, L.; Erba, S.; Inzani, P.; Leveraro, F.; Malvezzi, S.; Menasce, D.; Mezzadri, M.; Moroni, L.; Pedrini, D.; Pontoglio, C.; Prelz, F.; Rovere, M.; Sala, S.; Davenport, T. F.; Arena, V.; Boca, G.; Bonomi, G.; Gianini, G.; Liguori, G.; Lopes Pegna, D.; Merlo, M. M.; Pantea, D.; Ratti, S. P.; Riccardi, C.; Vitulo, P.; Göbel, C.; Olatora, J.; Hernandez, H.; Lopez, A. M.; Mendez, H.; Paris, A.; Quinones, J.; Ramirez, J. E.; Zhang, Y.; Wilson, J. R.; Handler, T.; Mitchell, R.; Engh, D.; Givens, K. M.; Hosack, M.; Johns, W. E.; Luiggi, E.; Nehring, M.; Sheldon, P. D.; Vaandering, E. W.; Webster, M.; Sheaff, M.

    2006-08-01

    We present a search for a pentaquark decaying strongly to pKS0 in γN collisions at a center-of-mass energy up to 25 GeV. Finding no evidence for such a state in the mass range of 1470 MeV/c to 2200 MeV/c, we set limits on the yield and on the cross section times branching ratio relative to Σ1385 and K892.

  15. A note on the accuracy of KS-DFT densities

    Science.gov (United States)

    Ranasinghe, Duminda S.; Perera, Ajith; Bartlett, Rodney J.

    2017-11-01

    The accuracy of the density of wave function methods and Kohn-Sham (KS) density functionals is studied using moments of the density, ⟨rn ⟩ =∫ ρ (r )rnd τ =∫0∞4 π r2ρ (r ) rnd r ,where n =-1 ,-2,0,1,2 ,and 3 provides information about the short- and long-range behavior of the density. Coupled cluster (CC) singles, doubles, and perturbative triples (CCSD(T)) is considered as the reference density. Three test sets are considered: boron through neon neutral atoms, two and four electron cations, and 3d transition metals. The total density and valence only density are distinguished by dropping appropriate core orbitals. Among density functionals tested, CAMQTP00 and ωB97x show the least deviation for boron through neon neutral atoms. They also show accurate eigenvalues for the HOMO indicating that they should have a more correct long-range behavior for the density. For transition metals, some density functional approximations outperform some wave function methods, suggesting that the KS determinant could be a better starting point for some kinds of correlated calculations. By using generalized many-body perturbation theory (MBPT), the convergence of second-, third-, and fourth-order KS-MBPT for the density is addressed as it converges to the infinite-order coupled cluster result. For the transition metal test set, the deviations in the KS density functional theory methods depend on the amount of exact exchange the functional uses. Functionals with exact exchange close to 25% show smaller deviations from the CCSD(T) density.

  16. K.S. Micro-Implant Placement Guide

    OpenAIRE

    Sharma, K; Sangwan, A

    2014-01-01

    A one of the greatest concerns with orthodontic mini-implants is risk of injury to dental roots during placement is, especially when they are inserted between teeth. Many techniques have been used to facilitate safe placement of interradicular miniscrews. Brass Wires or metallic markers are easy to place in the interproximal spaces, but because their relative positions may be inconsistent in different radio -graphic views, they are not always accurate. K.S. micro implant placement guide sugge...

  17. Search for a narrow baryonic resonance decaying to Ks0p or Ks0p¯ in deep inelastic scattering at HERA

    Science.gov (United States)

    H1 Collaboration; Aktas, A.; Andreev, V.; Anthonis, T.; Antunovic, B.; Aplin, S.; Asmone, A.; Astvatsatourov, A.; Babaev, A.; Backovic, S.; Baghdasaryan, A.; Baranov, P.; Barrelet, E.; Bartel, W.; Baudrand, S.; Baumgartner, S.; Becker, J.; Beckingham, M.; Behnke, O.; Behrendt, O.; Belousov, A.; Berger, N.; Bizot, J. C.; Boenig, M.-O.; Boudry, V.; Bracinik, J.; Brandt, G.; Brisson, V.; Bruncko, D.; Büsser, F. W.; Bunyatyan, A.; Buschhorn, G.; Bystritskaya, L.; Campbell, A. J.; Cassol-Brunner, F.; Cerny, K.; Cerny, V.; Chekelian, V.; Contreras, J. G.; Coughlan, J. A.; Cox, B. E.; Cozzika, G.; Cvach, J.; Dainton, J. B.; Dau, W. D.; Daum, K.; de Boer, Y.; Delcourt, B.; Del Degan, M.; de Roeck, A.; de Wolf, E. A.; Diaconu, C.; Dodonov, V.; Dubak, A.; Eckerlin, G.; Efremenko, V.; Egli, S.; Eichler, R.; Eisele, F.; Eliseev, A.; Elsen, E.; Essenov, S.; Falkewicz, A.; Faulkner, P. J. W.; Favart, L.; Fedotov, A.; Felst, R.; Feltesse, J.; Ferencei, J.; Finke, L.; Fleischer, M.; Flucke, G.; Fomenko, A.; Franke, G.; Frisson, T.; Gabathuler, E.; Garutti, E.; Gayler, J.; Gerlich, C.; Ghazaryan, S.; Ginzburgskaya, S.; Glazov, A.; Glushkov, I.; Goerlich, L.; Goettlich, M.; Gogitidze, N.; Gorbounov, S.; Grab, C.; Greenshaw, T.; Gregori, M.; Grell, B. R.; Grindhammer, G.; Gwilliam, C.; Haidt, D.; Hajduk, L.; Hansson, M.; Heinzelmann, G.; Henderson, R. C. W.; Henschel, H.; Herrera, G.; Hildebrandt, M.; Hiller, K. H.; Hoffmann, D.; Horisberger, R.; Hovhannisyan, A.; Hreus, T.; Hussain, S.; Ibbotson, M.; Ismail, M.; Jacquet, M.; Janauschek, L.; Janssen, X.; Jemanov, V.; Jönsson, L.; Johnson, D. P.; Jung, A. W.; Jung, H.; Kapichine, M.; Katzy, J.; Kenyon, I. R.; Kiesling, C.; Klein, M.; Kleinwort, C.; Klimkovich, T.; Kluge, T.; Knies, G.; Knutsson, A.; Korbel, V.; Kostka, P.; Krastev, K.; Kretzschmar, J.; Kropivnitskaya, A.; Krüger, K.; Landon, M. P. J.; Lange, W.; Laštovička-Medin, G.; Laycock, P.; Lebedev, A.; Leibenguth, G.; Lendermann, V.; Levonian, S.; Lindfeld, L.; Lipka, K.; Liptaj, A.; List, B.; List, J.; Lobodzinska, E.; Loktionova, N.; Lopez-Fernandez, R.; Lubimov, V.; Lucaci-Timoce, A.-I.; Lueders, H.; Lüke, D.; Lux, T.; Lytkin, L.; Makankine, A.; Malden, N.; Malinovski, E.; Mangano, S.; Marage, P.; Marshall, R.; Marti, L.; Martisikova, M.; Martyn, H.-U.; Maxfield, S. J.; Mehta, A.; Meier, K.; Meyer, A. B.; Meyer, H.; Meyer, J.; Michels, V.; Mikocki, S.; Milcewicz-Mika, I.; Milstead, D.; Mladenov, D.; Mohamed, A.; Moreau, F.; Morozov, A.; Morris, J. V.; Mozer, M. U.; Müller, K.; Murín, P.; Nankov, K.; Naroska, B.; Naumann, Th.; Newman, P. R.; Niebuhr, C.; Nikiforov, A.; Nowak, G.; Nowak, K.; Nozicka, M.; Oganezov, R.; Olivier, B.; Olsson, J. E.; Osman, S.; Ozerov, D.; Palichik, V.; Panagoulias, I.; Papadopoulou, T.; Pascaud, C.; Patel, G. D.; Peng, H.; Perez, E.; Perez-Astudillo, D.; Perieanu, A.; Petrukhin, A.; Pitzl, D.; Plačakytė, R.; Portheault, B.; Povh, B.; Prideaux, P.; Rahmat, A. J.; Raicevic, N.; Reimer, P.; Rimmer, A.; Risler, C.; Rizvi, E.; Robmann, P.; Roland, B.; Roosen, R.; Rostovtsev, A.; Rurikova, Z.; Rusakov, S.; Salvaire, F.; Sankey, D. P. C.; Sauvan, E.; Schätzel, S.; Schmidt, S.; Schmitt, S.; Schmitz, C.; Schoeffel, L.; Schöning, A.; Schultz-Coulon, H.-C.; Sefkow, F.; Shaw-West, R. N.; Sheviakov, I.; Shtarkov, L. N.; Sloan, T.; Smirnov, P.; Soloviev, Y.; South, D.; Spaskov, V.; Specka, A.; Steder, M.; Stella, B.; Stiewe, J.; Stoilov, A.; Straumann, U.; Sunar, D.; Tchoulakov, V.; Thompson, G.; Thompson, P. D.; Toll, T.; Tomasz, F.; Traynor, D.; Truöl, P.; Tsakov, I.; Tsipolitis, G.; Tsurin, I.; Turnau, J.; Tzamariudaki, E.; Urban, K.; Urban, M.; Usik, A.; Utkin, D.; Valkárová, A.; Vallée, C.; van Mechelen, P.; Vargas Trevino, A.; Vazdik, Y.; Veelken, C.; Vinokurova, S.; Volchinski, V.; Wacker, K.; Weber, G.; Weber, R.; Wegener, D.; Werner, C.; Wessels, M.; Wessling, B.; Wissing, Ch.; Wolf, R.; Wünsch, E.; Xella, S.; Yan, W.; Yeganov, V.; Žáček, J.; Zálešák, J.; Zhang, Z.; Zhelezov, A.; Zhokin, A.; Zhu, Y. C.; Zimmermann, J.; Zimmermann, T.; Zohrabyan, H.; Zomer, F.

    2006-08-01

    A search for a narrow baryonic resonance decaying to Ks0p or Ks0p¯ is carried out in deep inelastic ep scattering with the H1 detector at HERA. Such a resonance could be a strange pentaquark Θ, evidence for which has been reported by several experiments. The Ks0p and Ks0p¯ invariant mass distributions presented here do not show any significant peak in the mass range from threshold up to 1.7 GeV. Mass dependent upper limits on σ(ep→eΘX)×BR(Θ→Kp) are obtained at the 95% confidence level.

  18. Schizotypy: Key feature of Klinefelter's syndrome?

    NARCIS (Netherlands)

    Verhoeven, W.M.A.; Egger, J.I.M.

    2011-01-01

    Klinefelter's syndrome (KS; karyotype 47,XXY) is associated with specific neurocognitive impairments, especially delayed language development and impaired socioemotional evolution. There is an increased risk for psychiatric disturbances, particularly schizophrenia and affective spectrum disorders. A

  19. Advanced `KS-6` dry type lubricant for aluminum sheet forming; Arumi ban seikeiyo koseino kokei junkatsuzai `KS-5`

    Energy Technology Data Exchange (ETDEWEB)

    Matsui, K.; Sugita, T.; Imamura, Y. [Kobe Steel, Ltd., Kobe (Japan)

    1997-09-01

    The advanced `KS-5` dry film type lubricant was developed for press forming of aluminum sheets. KS-5 uses water- soluble resin poly-alkylene-oxide superior in formability, weldability and adhesivity, and contains higher fatty-acid soap as oil solvent to improve a formability. The verification test result of KS-5 is as follows. Both stretchability and drawability were confirmed through a ball head stretching test and a cylinder drawing test as formability test, respectively, and a forming height more than that of mild steel sheets was obtained by using the solid lubricant showing a high stretchability. The drawability of nearly 80% of that of mild steel sheets was also obtained showing a high formability. Since the amount of the solid lubricant has reciprocal effect on the formability and degreasing property, it is important to select the suitable amount of the solid lubricant according to use conditions. Lubricants generally deteriorate a spot weldability, however, this lubricant has no practical problems by coating rust preventive oil. 3 refs., 8 figs., 3 tabs.

  20. Socioeconomic trajectories affect mortality in Klinefelter syndrome

    DEFF Research Database (Denmark)

    Bojesen, Anders; Krag, Kirstine Stochholm; Juul, Svend

    2011-01-01

    Klinefelter syndrome (KS) is associated with male infertility, hypogonadism, and learning disability. Morbidity and mortality are increased and the causes behind remain unknown. Is it the chromosome aberration or is it caused by postulated poorer socioeconomic status?......Klinefelter syndrome (KS) is associated with male infertility, hypogonadism, and learning disability. Morbidity and mortality are increased and the causes behind remain unknown. Is it the chromosome aberration or is it caused by postulated poorer socioeconomic status?...

  1. Array analyses of SmKS waves and the stratification of Earth's outermost core

    Science.gov (United States)

    Kaneshima, Satoshi

    2018-03-01

    We perform array analyses of SmKS waves in order to investigate the Vp structure of the Earth's outermost core. For earthquakes recorded by broadband seismometer networks in the world, we measure differential travel times between S3KS and S2KS, between S4KS and S3KS, and between S5KS and S3KS by array techniques. The differential times are well fit by a Vp model of the Earth's outermost core, KHOMC (Kaneshima and Helffrich, 2013). Differential slownesses of S4KS and S2KS relative to S2KS are also measured for the highest quality data. The measured slownesses, with unique sensitivity to the outer core 200-400 km below the CMB, are matched by KHOMC. These observations consolidate the evidence for the presence at the top of the outer core of a layer that has a distinctively steeper Vp gradient than the bulk of the outer core. We invert new SmKS differential time data set by a tau-p method and attempt to refine the Vp profile of KHOMC. The essential features of KHOMC are preserved after the model refinement. However, the newly estimated layer thickness is nearly 450 km, which is thicker than that of KHOMC. The Vp anomalies relative to PREM for the depths 400-800 km below the CMB are less than 0.03 km/s, consistent with the degree of agreement between different Vp models for the depth range.

  2. Korsakoff's syndrome: A critical review

    NARCIS (Netherlands)

    Arts, N.J.M.; Walvoort, S.J.W.; Kessels, R.P.C.

    2017-01-01

    In this review, we present a survey on Korsakoff's syndrome (KS), a residual syndrome in patients who suffered from a Wernicke encephalopathy (WE) that is predominantly characterized by global amnesia, and in more severe cases also by cognitive and behavioral dysfunction. We describe the history of

  3. Penelitian penentuan konstanta saturasi (Ks pada rbc unit

    Directory of Open Access Journals (Sweden)

    Prayitno Prayitno

    1992-04-01

    Full Text Available Kinetic of Rotating Biological Contractor have been investigated by manu researchers. In general the model emploued either saturation kinetic (following Monod equation or first order kinetic to describe substrate removal. In this experiment, saturation kinetic model was used to find out the saturation constant of the RBC unit. The influent fed into the unit was synthetic waste water with glucose as organic carbon sources. The flow was maintained constant at 20.4 1/d and COD influent resulted the saturation constant (Ks 11.17 mg/I with 20 hours of detention time.

  4. Korsakoff’s syndrome: a critical review

    Science.gov (United States)

    Arts, Nicolaas JM; Walvoort, Serge JW; Kessels, Roy PC

    2017-01-01

    In this review, we present a survey on Korsakoff’s syndrome (KS), a residual syndrome in patients who suffered from a Wernicke encephalopathy (WE) that is predominantly characterized by global amnesia, and in more severe cases also by cognitive and behavioral dysfunction. We describe the history of KS and its definition, its epidemiology, and the lack of consensus criteria for its diagnosis. The cognitive and behavioral symptoms of KS, which include anterograde and retrograde amnesia, executive dysfunction, confabulation, apathy, as well as affective and social-cognitive impairments, are discussed. Moreover, recent insights into the underlying neurocognitive mechanisms of these symptoms are presented. In addition, the evidence so far on the etiology of KS is examined, highlighting the role of thiamine and alcohol and discussing the continuity hypothesis. Furthermore, the neuropathology of KS is reviewed, focusing on abnormalities in the diencephalon, including the mammillary bodies and thalamic nuclei. Pharmacological treatment options and nonpharmacological interventions, such as those based on cognitive rehabilitation, are discussed. Our review shows that thiamine deficiency (TD) is a crucial factor in the etiology of KS. Although alcohol abuse is by far the most important context in which TD occurs, there is no convincing evidence for an essential contribution of ethanol neurotoxicity (EN) to the development of WE or to the progression of WE to KS. Future research on the postmortem histopathological analysis of brain tissues of KS patients is crucial for the advancement of our knowledge of KS, especially for associating its symptoms with lesions in various thalamic nuclei. A necessary requirement for the advancement of studies on KS is the broad acceptance of a comprehensive definition and definite diagnostic criteria. Therefore, in this review, we propose such a definition of KS and draft outlines for prospective diagnostic criteria. PMID:29225466

  5. Isolated and syndromic forms of congenital anosmia

    DEFF Research Database (Denmark)

    Karstensen, H G; Tommerup, N

    2012-01-01

    . Despite a strong degree of heritability, no human disease-causing mutations have been identified. Anosmia is part of the clinical spectrum in various diseases, as seen in Kallmann syndrome, various ciliopathies and congenital insensitivity to pain. This review will focus on ICA through already published......Loss of smell (anosmia) is common in the general population and the frequency increases with age. A much smaller group have no memory of ever being able to smell and are classified as having isolated congenital anosmia (ICA). Families are rare, and tend to present in a dominant inheritance pattern...... families and cases as well as syndromes where anosmia is part of the clinical disease spectrum. Furthermore, olfactory signal transduction pathway genes and animal models may shed light on potential candidate genes and pathways involved in ICA....

  6. Fluid intelligence, traits of personality and personality disorders in a cohort of adult KS patients with the classic 47, XXY karyotype.

    Science.gov (United States)

    Liberato, D; Granato, S; Grimaldi, D; Rossi, F M; Tahani, N; Gianfrilli, D; Anzuini, A; Lenzi, A; Cavaggioni, G; Radicioni, A F

    2017-11-01

    Klinefelter's syndrome (KS) is associated with specific neurobehavioral features and personality traits. The aim of our study was to investigate fluid intelligence, personality traits and personality disorders (PD) and possible correlations with testosterone in a cohort of adult KS patients. We analyzed 58 adult KS patients with the classic 47, XXY karyotype. The Structured Clinical Interview for axis II disorders was used to assess DSM IV personality disorders. Personality traits were assessed using MMPI-2. Fluid intelligence was tested by using Raven's Standard Progressive Matrices (SPM) Test. Testosterone blood concentration was measured by CMIA. PD prevalence was 31%. Four altered MMPI scales (Social Responsibility, Dominance, Ego Strength and Repression) were found in more than 40% of patients. Overcontrolled hostility and MacAndrew Alcoholism Scale-Revised scales were altered in the PD- group only. Biz-Odd Thinking and Post-Traumatic Stress Disorder scale were associated with the presence of personality disorder. The raw SPM score was 44 ± 10.8 without any significant correlation with testosterone. No significant difference in mean age, SPM raw score and MMPI score was observed between eugonadal, hypogonadal and treated patients. Most KS patients had average fluid intelligence. PD prevalence was higher than in the general population. Testosterone was not correlated with fluid intelligence, personality traits or PD, but a reduction in marital distress was observed in treated patients. This could suggest that testosterone therapy can improve physical symptoms and this effect could also improve relationship abilities and wellness awareness.

  7. The role of hypogonadism in Klinefelter Syndrome

    Directory of Open Access Journals (Sweden)

    Christian Høst

    2014-04-01

    Full Text Available Klinefelter syndrome (KS (47, XXY is the most abundant sex-chromosome disorder, and is a common cause of infertility and hypogonadism in men. Most men with KS go through life without knowing the diagnosis, as only 25% are diagnosed and only a few of these before puberty. Apart from hypogonadism and azoospermia, most men with KS suffer from some degree of learning disability and may have various kinds of psychiatric problems. The effects of long-term hypogonadism may be diffi cult to discern from the gene dose effect of the extra X-chromosome. Whatever the cause, alterations in body composition, with more fat and less muscle mass and diminished bone mineral mass, as well as increased risk of metabolic consequences, such as type 2 diabetes and the metabolic syndrome are all common in KS. These fi ndings should be a concern as they are not simply laboratory fi ndings; epidemiological studies in KS populations show an increased risk of both hospitalization and death from various diseases. Testosterone treatment should be offered to KS patients from early puberty, to secure a proper masculine development, nonetheless the evidence is weak or nonexisting, since no randomized controlled trials have ever been published. Here, we will review the current knowledge of hypogonadism in KS and the rationale for testosterone treatment and try to give our best recommendations for surveillance of this rather common, but often ignored, syndrome.

  8. The role of hypogonadism in Klinefelter syndrome.

    Science.gov (United States)

    Høst, Christian; Skakkebæk, Anne; Groth, Kristian A; Bojesen, Anders

    2014-01-01

    Klinefelter syndrome (KS) (47, XXY) is the most abundant sex-chromosome disorder, and is a common cause of infertility and hypogonadism in men. Most men with KS go through life without knowing the diagnosis, as only 25% are diagnosed and only a few of these before puberty. Apart from hypogonadism and azoospermia, most men with KS suffer from some degree of learning disability and may have various kinds of psychiatric problems. The effects of long-term hypogonadism may be diffi cult to discern from the gene dose effect of the extra X-chromosome. Whatever the cause, alterations in body composition, with more fat and less muscle mass and diminished bone mineral mass, as well as increased risk of metabolic consequences, such as type 2 diabetes and the metabolic syndrome are all common in KS. These findings should be a concern as they are not simply laboratory findings; epidemiological studies in KS populations show an increased risk of both hospitalization and death from various diseases. Testosterone treatment should be offered to KS patients from early puberty, to secure a proper masculine development, nonetheless the evidence is weak or nonexisting, since no randomized controlled trials have ever been published. Here, we will review the current knowledge of hypogonadism in KS and the rationale for testosterone treatment and try to give our best recommendations for surveillance of this rather common, but often ignored, syndrome.

  9. Human herpesvirus-8 (HHV-8 sero-detection and HIV association in Kaposi's sarcoma (KS, non-KS tumors and non-neoplastic conditions

    Directory of Open Access Journals (Sweden)

    Pak Fatemeh

    2008-06-01

    Full Text Available Abstract Background The association of the human herpesvirus-8/Kaposi's sarcoma (KS-associated herpesvirus (HHV-8/KSHV serology with various malignancies in Tanzania is not currently well established while previous studies were based on either PCR or immunofluorescence assays [IFA] but not with a sensitive enzyme-linked immunosorbent assay (ELISA. Selected archival diagnostic biopsies (n = 184 and sera from indigenous patients with KS (n = 120, non-KS tumors (n = 24 and non-neoplastic lesions (n = 40 at Muhimbili National Hospital (MNH, Tanzania, were evaluated by diagnostic histopathology, immunohistology [anti-HHV-8 latency-associated nuclear antigen (LANA] and serology for HIV (ELISA and HHV-8 (IFA and ELISA. Results About 66.3% (n = 122 cases including AIDS-associated Kaposi's sarcoma (AKS (n = 93, reactive conditions (n = 28 and only one non-KS tumour were HIV positive. Endemic KS (EKS patients were mostly males (96.3%, 26/27 who were less (69.9%, 65/93 predominant in AIDS-associated (AKS. A high (89% percentage of patients with anti-HHV-8 antibodies was found in the cohort including the HIV positive (92% cases, males (81.2%, KS patients (93%, non-KS tumors (92%, and reactive conditions (75%. All HHV-8 seronegative KS cases were nodular stage whereas both sera and corresponding biopsies from early stage KS were HHV-8+. Assay sensitivity, positive predictive value (PPV and specificity were 98.6%, 93.5% and 16.7% for IFA and 93.5%, 98.6% and 50.0% for ELISA respectively. Conclusion HHV-8 seroprevalence at MNH appears high as expected among AKS cases and males but also in non-KS patients. ELISA showed a combination of high HHV-8 sensitivity as well as higher PPV and specificity than IFA which however, showed higher sensitivity. The apparent stage-dependent, inverted serum HHV-8 immunoreactivity supports a notion of viral immune-segregation during KS development. Routine HHV-8 screening should be considered particularly in patients at risk of

  10. Klinefelter syndrome has increased brain responses to auditory stimuli and motor output, but not to visual stimuli or Stroop adaptation

    DEFF Research Database (Denmark)

    Wallentin, Mikkel; Skakkebæk, Anne; Bojesen, Anders

    2016-01-01

    Klinefelter syndrome (47, XXY) (KS) is a genetic syndrome characterized by the presence of an extra X chromosome and low level of testosterone, resulting in a number of neurocognitive abnormalities, yet little is known about brain function. This study investigated the fMRI-BOLD response from KS...

  11. K.s. Micro-implant placement guide.

    Science.gov (United States)

    Sharma, K; Sangwan, A

    2014-09-01

    A one of the greatest concerns with orthodontic mini-implants is risk of injury to dental roots during placement is, especially when they are inserted between teeth. Many techniques have been used to facilitate safe placement of interradicular miniscrews. Brass Wires or metallic markers are easy to place in the interproximal spaces, but because their relative positions may be inconsistent in different radio -graphic views, they are not always accurate. K.S. micro implant placement guide suggested in this article is simple design and easy in fabrication, required minimal equipment for fabrication and does not disturb the existing appliance system, clearly located in the radiograph and the mini-screw can be easily inserted through the guide reducing the chance of implant misplacement.

  12. Fission product poisoning in KS-150 reactor operation

    International Nuclear Information System (INIS)

    Rana, S.B.

    1978-01-01

    A three-dimensional model of the KS-150 reactor was used to study reactivity changes induced by reactor poisoning with fission products Xe 135 and Sm 149 . A comparison of transients caused by the poisoning showed the following differences: (1) the duration of the transient Xe poisoning (2 days) is shorter by one order of magnitude than the duration of Sm poisoning (20 days); however, the level of Xe poisoning is greater approximately by one order than the level of the Sm poisoning; (2) the level of steady-state Xe poisoning depends on the output level of the reactor; steady-state Sm poisoning does not depend on this level; (3) following reactor shutdown Xe poisoning may increase to the maximum value of up to Δrhosub(Xe)=20% and will then gradually decrease; Sm poisoning may reach maximum values of up to Δrhosub(Sm)=2% and does not decrease. (J.B.)

  13. Measurement of the time-integrated CP asymmetry in $D^0 \\to K_S^0 K_S^0$ decays with LHCb

    CERN Multimedia

    Tuci, Giulia

    2018-01-01

    CP violation in charm has not yet been observed, although measurements of time-integrated CP asymmetries in $D^0 \\to K^+K^-$ and $D^0 \\to \\pi^+\\pi^- decays$ have reached a remarkable precision, O(0.1%). The $D^0 \\to K_S^0 K_S^0$ decay is a promising discovery channel for CP violation in charm. A prediction based on Standard Model gives an upper limit for the CP asymmetry of 1.1% (C.L. 95%). Further enhancements could result from contributions from physics beyond the Standard Model. We present a measurement of the time-integrated CP asymmetry in prompt $D^0 \\to K_S^0 K_S^0$ decays, performed using data collected with LHCb experiment in 2015 and 2016 at a 13 TeV pp center-of-mass energy (Run-2). This result improves the sensitivity obtained by LHCb in Run-1.

  14. Tremors and Klinefelter's Syndrome

    Directory of Open Access Journals (Sweden)

    Marcie L. Rabin

    2015-06-01

    Full Text Available Background: Klinefelter’s syndrome (KS has been associated with tremor, but reports on tremor phenomenology and treatment are limited. Case Reports: Patient 1 is a 17‐year‐old male with a dystonic tremor treated with deep brain stimulation (DBS. Patient 2 is a 57‐year‐old male with a predominant left hand resting tremor and dystonic features. Discussion: Our cases suggest that the tremor in patients with KS may be dystonic in nature. Patient 1 is also the third reported case of successful treatment with DBS. These cases have implications for elucidating the underlying neurobiological mechanism of tremor and identifying treatment options.

  15. Branching fraction and CP asymmetry of the decays B+→KS0π+ and B+→KS0K+

    NARCIS (Netherlands)

    Aaij, R.; Adeva, B.; Adrover, C.; Affolder, A.; Ajaltouni, Z.; Alessio, F.; Alexander, M.; Ali, S.; Alkhazov, G.; Alvarez Cartelle, P.; Alves, A. A.; Amato, S.; Amerio, S.; Amhis, Y.; Anderlini, L.; Anderson, J.; Andreassen, R.; Andrews, J. E.; Appleby, R. B.; Aquines Gutierrez, O.; Archilli, F.; Artamonov, A.; Artuso, M.; Aslanides, E.; Auriemma, G.; Baalouch, M.; Bachmann, S.; Back, J. J.; Baesso, C.; Balagura, V.; Baldini, W.; Barlow, R. J.; Barschel, C.; Barsuk, S.; Barter, W.; Bauer, Th; Bay, A.; Beddow, J.; Bedeschi, F.; Bediaga, I.; Belogurov, S.; Belous, K.; Belyaev, I.; Ben-Haim, E.; Bencivenni, G.; Benson, S.; Benton, J.; Berezhnoy, A.; Bernet, R.; Bettler, M. O.; van Beuzekom, M.; Bien, A.; Bifani, S.; Bird, T.; Bizzeti, A.; Bjørnstad, P. M.; Blake, T.; Blanc, F.; Blouw, J.; Blusk, S.; Bocci, V.; Bondar, A.; Bondar, N.; Bonivento, W.; Borghi, S.; Borgia, A.; Bowcock, T. J.V.; Bowen, E.; Bozzi, C.; Brambach, T.; van den Brand, J.; Bressieux, J.; Brett, D.; Britsch, M.; Britton, T.; Brook, N. H.; Brown, H.; Burducea, I.; Bursche, A.; Busetto, G.; Buytaert, J.; Cadeddu, S.; Callot, O.; Calvi, M.; Calvo Gomez, M.; Camboni, A.; Campana, P.; Campora Perez, D.; Carbone, A.; Carboni, G.; Cardinale, R.; Cardini, A.; Carranza-Mejia, H.; Carvalho Akiba, K.; Casse, G.; Castillo Garcia, L.; Cattaneo, M.; Cauet, Ch; Cenci, R.; Charles, M.; Charpentier, Ph; Chen, P.; Chiapolini, N.; Chrzaszcz, M.; Ciba, K.; Cid Vidal, X.; Ciezarek, G.; Clarke, P. E.L.; Clemencic, M.; Cliff, H. V.; Closier, J.; Coca, C.; Coco, V.; Cogan, J.; Cogneras, E.; Collins, P.; Comerma-Montells, A.; Contu, A.; Cook, A.; Coombes, M.; Coquereau, S.; Corti, G.; Couturier, B.; Cowan, G. A.; Craik, D. C.; Cunliffe, S.; Currie, R.; D'Ambrosio, C.; David, P.; Davis, A.; De Bonis, I.; De Bruyn, K.; De Capua, S.; De Cian, M.; De Miranda, J. M.; De Paula, L.; De Silva, W.; De Simone, P.; Decamp, D.; Deckenhoff, M.; Del Buono, L.; Déléage, N.; Derkach, D.; Deschamps, O.; Dettori, F.; Di Canto, A.; Dogaru, M.; Donleavy, S.; Dordei, F.; Dosil Suárez, A.; Dossett, D.; Dovbnya, A.; Dupertuis, F.; Durante, P.; Dzhelyadin, R.; Dziurda, A.; Dzyuba, A.; Easo, S.; Egede, U.; Egorychev, V.; Eidelman, S.; van Eijk, D.; Eisenhardt, S.; Eitschberger, U.; Ekelhof, R.; Eklund, L.; El Rifai, I.; Elsasser, Ch; Falabella, A.; Färber, C.; Fardell, G.; Farinelli, C.; Farry, S.; Ferguson, D.; Fernandez Albor, V.; Ferreira Rodrigues, F.; Ferro-Luzzi, M.; Filippov, S.; Fiore, M.; Fitzpatrick, C.; Fontana, M.; Fontanelli, F.; Forty, R.; Francisco, O.; Frank, M.; Frei, C.; Frosini, M.; Furcas, S.; Furfaro, E.; Gallas Torreira, A.; Galli, D.; Gandelman, M.; Gandini, P.; Gao, Y.; Garofoli, J.; Garosi, P.; Garra Tico, J.; Garrido, L.; Gaspar, C.; Gauld, R.; Gersabeck, E.; Gersabeck, M.; Gershon, T.; Ghez, Ph; Gibson, V.; Giubega, L.; Gligorov, V. V.; Göbel, C.; Golubkov, D.; Golutvin, A.; Gomes, A.; Gorbounov, P.; Gordon, H.; Gotti, C.; Grabalosa Gándara, M.; Graciani Diaz, R.; Granado Cardoso, L. A.; Graugés, E.; Graziani, G.; Grecu, A.; Greening, E.; Gregson, S.; Grünberg, O.; Gui, B.; Gushchin, E.; Guz, Yu; Gys, T.; Hadjivasiliou, C.; Haefeli, G.; Haen, C.; Haines, S. C.; Hall, S.; Hamilton, B.; Hampson, T.; Hansmann-Menzemer, S.; Harnew, N.; Harnew, S. T.; Harrison, J.; Head, T.; Heijne, V.; Hennessy, K.; Henrard, P.; Hernando Morata, J. A.; van Herwijnen, E.; Hess, M.; Hicheur, A.; Hicks, E.; Hill, D.; Hoballah, M.; Hombach, C.; Hopchev, P.; Hulsbergen, W.; Hunt, P.; Huse, T.; Hussain, N.; Hutchcroft, D.; Hynds, D.; Iakovenko, V.; Idzik, M.; Ilten, P.; Jacobsson, R.; Jaeger, A.; Jans, E.; Jaton, P.; Jawahery, A.; Jing, F.; John, M.; Jones, C. R.; Joram, C.; Jost, B.; Kaballo, M.; Kandybei, S.; Kanso, W.; Karacson, M.; Karbach, T. M.; Kenyon, I. R.; Ketel, T.; Keune, A.; Khanji, B.; Kochebina, O.; Komarov, I.; Koopman, R. F.; Koppenburg, P.; Korolev, M.; Kozlinskiy, A.; Kravchuk, L.; Kreplin, K.; Kreps, M.; Krocker, G.; Krokovny, P.; Kruse, F.; Kucharczyk, M.; Kudryavtsev, V.; Kvaratskheliya, T.; La Thi, V. N.; Lacarrere, D.; Lafferty, G.; Lai, A.; Lambert, D.; Lambert, R. W.; Lanciotti, E.; Lanfranchi, G.; Langenbruch, C.; Latham, T.; Lazzeroni, C.; Le Gac, R.; van Leerdam, J.; Lees, J. P.; Lefèvre, R.; Leflat, A.; Lefrançois, J.; Leo, S.; Leroy, O.; Lesiak, T.; Leverington, B.; Li Gioi, L.; Liles, M.; Lindner, R.; Linn, C.; Lohn, S.; Longstaff, I.; Lopes, J. H.; Lopez-March, N.; Lu, H.; Lucchesi, D.; Luisier, J.; Luo, H.; Machefert, F.; Machikhiliyan, I. V.; Maciuc, F.; Maev, O.; Malde, S.; Manca, G.; Mancinelli, G.; Maratas, J.; Marconi, U.; Marino, P.; Märki, R.; Marks, J.; Martellotti, G.; Martín Sánchez, A.; Martinelli, M.; Martinez Santos, D.; Martins Tostes, D.; Martynov, A.; Massafferri, A.; Matev, R.; Mathe, Z.; Matteuzzi, C.; Maurice, E.; Mazurov, A.; McCarthy, J.; McNab, A.; McNulty, R.; McSkelly, B.; Meadows, B.; Meier, F.; Meissner, M.; Merk, M.; Milanes, D. A.; Minard, M. N.; Molina Rodriguez, J.; Monteil, S.; Moran, D.; Morawski, P.; Mordà, A.; Morello, M. J.; Mountain, R.; Mous, I.; Muheim, F.; Müller, K.; Muresan, R.; Muryn, B.; Muster, B.; Naik, P.; Nakada, T.; Nandakumar, R.; Nasteva, I.; Needham, M.; Neubert, S.; Neufeld, N.; Nguyen, A. D.; Nguyen-Mau, C.; Nicol, M.; Niess, V.; Niet, R.; Nikitin, N.; Nikodem, T.; Nomerotski, A.; Novoselov, A.; Oblakowska-Mucha, A.; Obraztsov, V.; Oggero, S.; Ogilvy, S.; Okhrimenko, O.; Oldeman, R.; Orlandea, M.; Otalora Goicochea, J. M.; Owen, P.; Oyanguren, A.; Pal, B. K.; Palano, A.; Palczewski, T.; Palutan, M.; Panman, J.; Papanestis, A.; Pappagallo, M.; Parkes, C.; Parkinson, C. J.; Passaleva, G.; Patel, G. D.; Patel, M.; Patrick, G. N.; Patrignani, C.; Pavel-Nicorescu, C.; Pazos Alvarez, A.; Pellegrino, A.; Penso, G.; Pepe Altarelli, M.; Perazzini, S.; Perez Trigo, E.; Pérez-Calero Yzquierdo, A.; Perret, P.; Perrin-Terrin, M.; Pescatore, L.; Pesen, E.; Petridis, K.; Petrolini, A.; Phan, A.; Picatoste Olloqui, E.; Pietrzyk, B.; Pilař, T.; Pinci, D.; Playfer, S.; Plo Casasus, M.; Polci, F.; Polok, G.; Poluektov, A.; Polycarpo, E.; Popov, A.; Popov, D.; Popovici, B.; Potterat, C.; Powell, A.; Prisciandaro, J.; Pritchard, A.; Prouve, C.; Pugatch, V.; Puig Navarro, A.; Punzi, G.; Qian, W.; Rademacker, J. H.; Rakotomiaramanana, B.; Rangel, M. S.; Raniuk, I.; Rauschmayr, N.; Raven, G.; Redford, S.; Reid, M. M.; dos Reis, A. C.; Ricciardi, S.; Richards, A.; Rinnert, K.; Rives Molina, V.; Roa Romero, D. A.; Robbe, P.; Roberts, D. A.; Rodrigues, E.; Rodriguez Perez, P.; Roiser, S.; Romanovsky, V.; Romero Vidal, A.; Rouvinet, J.; Ruf, T.; Ruffini, F.; Ruiz, H.; Ruiz Valls, P.; Sabatino, G.; Saborido Silva, J. J.; Sagidova, N.; Sail, P.; Saitta, B.; Salustino Guimaraes, V.; Sanmartin Sedes, B.; Sannino, M.; Santacesaria, R.; Santamarina Rios, C.; Santovetti, E.; Sapunov, M.; Satriano, C.; Satta, A.; Savrie, M.; Savrina, D.; Schaack, P.; Schiller, M.; Schindler, H.; Schlupp, M.; Schmelling, M.; Schmidt, B.; Schneider, O.; Schopper, A.; Schune, M. H.; Schwemmer, R.; Sciascia, B.; Sciubba, A.; Seco, M.; Semennikov, A.; Senderowska, K.; Sepp, I.; Serra, N.; Serrano, J.; Seyfert, P.; Shapkin, M.; Shapoval, I.; Shatalov, P.; Shcheglov, Y.; Shears, T.; Shekhtman, L.; Shevchenko, O.; Shevchenko, V.; Shires, A.; Silva Coutinho, R.; Sirendi, M.; Skidmore, N.; Skwarnicki, T.; Smith, N. A.; Smith, J.; Smith, M.; Sokoloff, M. D.; Soler, F. J.P.; Soomro, F.; Souza, D.; Souza De Paula, B.; Spaan, B.; Sparkes, A.; Spradlin, P.; Stagni, F.; Stahl, S.; Steinkamp, O.; Stevenson, S.; Stoica, S.; Stone, S.; Storaci, B.; Straticiuc, M.; Straumann, U.; Subbiah, V. K.; Sun, L.; Swientek, S.; Syropoulos, V.; Szczekowski, M.; Szczypka, P.; Szumlak, T.; T'Jampens, S.; Teklishyn, M.; Teodorescu, E.; Teubert, F.; Thomas, C.; van Tilburg, J.; Tisserand, V.; Tobin, M.; Tolk, S.; Tonelli, D.; Topp-Joergensen, S.; Torr, N.; Tournefier, E.; Tourneur, S.; Tran, M. T.; Tresch, M.; Tsaregorodtsev, A.; Tsopelas, P.; Tuning, N.; Ubeda Garcia, M.; Ukleja, A.; Urner, D.; Ustyuzhanin, A.; Uwer, U.; Vagnoni, V.; Valenti, G.; Vallier, A.; Vazquez Gomez, R.; Vazquez Regueiro, P.; Vázquez Sierra, C.; Vecchi, S.; Velthuis, J. J.; Veltri, M.; Veneziano, G.; Vesterinen, M.; Viaud, B.; Vieira, D.; Vilasis-Cardona, X.; Vollhardt, A.; Volyanskyy, D.; Voong, D.; Vorobyev, A.; Vorobyev, V.; Voß, C.; Waldi, R.; Wallace, C.; Wallace, R.; Wandernoth, S.; Ward, D. R.; Watson, N. K.; Webber, A. D.; Websdale, D.; Whitehead, M.; Wicht, J.; Wiechczynski, J.; Wiedner, D.; Wilkinson, G.; Williams, M. P.; Williams, M.; Wilson, F. F.; Wimberley, J.; Wishahi, J.; Wislicki, W.; Witek, M.; Wotton, S. A.; Wright, S.; Wyllie, K.; Xie, Y.; Xing, Z.; Yang, Z.; Young, R.; Yuan, X.; Yushchenko, O.; Zangoli, M.; Zavertyaev, M.; Zhang, F.; Zhang, W. C.; Zhelezov, A.; Zhokhov, A.; Zhong, L.; Zvyagin, A.

    2013-01-01

    An analysis of B+→KS0π+ and B+→KS0K+ decays is performed with the LHCb experiment. The pp collision data used correspond to integrated luminosities of 1fb-1 and 2fb-1 collected at centre-of-mass energies of √s=7TeV and √s=8TeV, respectively. The ratio of branching fractions and the direct CP

  16. Schizotypy: key feature of Klinefelter’s syndrome?

    OpenAIRE

    Verhoeven, Willem M A; Egger, Jos I M

    2011-01-01

    Klinefelter’s syndrome (KS; karyotype 47,XXY) is associated with specific neurocognitive impairments, especially delayed language development and impaired socioemotional evolution. There is an increased risk for psychiatric disturbances, particularly schizophrenia and affective spectrum disorders.

  17. A Cancer Gene Selection Algorithm Based on the K-S Test and CFS

    Directory of Open Access Journals (Sweden)

    Qiang Su

    2017-01-01

    Full Text Available Background. To address the challenging problem of selecting distinguished genes from cancer gene expression datasets, this paper presents a gene subset selection algorithm based on the Kolmogorov-Smirnov (K-S test and correlation-based feature selection (CFS principles. The algorithm selects distinguished genes first using the K-S test, and then, it uses CFS to select genes from those selected by the K-S test. Results. We adopted support vector machines (SVM as the classification tool and used the criteria of accuracy to evaluate the performance of the classifiers on the selected gene subsets. This approach compared the proposed gene subset selection algorithm with the K-S test, CFS, minimum-redundancy maximum-relevancy (mRMR, and ReliefF algorithms. The average experimental results of the aforementioned gene selection algorithms for 5 gene expression datasets demonstrate that, based on accuracy, the performance of the new K-S and CFS-based algorithm is better than those of the K-S test, CFS, mRMR, and ReliefF algorithms. Conclusions. The experimental results show that the K-S test-CFS gene selection algorithm is a very effective and promising approach compared to the K-S test, CFS, mRMR, and ReliefF algorithms.

  18. Measurement of the nuclear multiplicity ratio for Ks0 hadronization at CLAS

    Science.gov (United States)

    Daniel, A.; Hicks, K.; Brooks, W. K.; Hakobyan, H.; Adhikari, K. P.; Adikaram, D.; Aghasyan, M.; Amarian, M.; Anghinolfi, M.; Avakian, H.; Baghdasaryan, H.; Battaglieri, M.; Batourine, V.; Bedlinskiy, I.; Bennett, R. P.; Biselli, A. S.; Bookwalter, C.; Briscoe, W. J.; Burkert, V. D.; Carman, D. S.; Casey, L.; Celentano, A.; Chandavar, S.; Cole, P. L.; Contalbrigo, M.; Crede, V.; D'Angelo, A.; Dashyan, N.; De Vita, R.; De Sanctis, E.; Deur, A.; Dey, B.; Dickson, R.; Djalali, C.; Dodge, G. E.; Doughty, D.; Egiyan, H.; El Fassi, L.; Elouadrhiri, L.; Eugenio, P.; Fedotov, G.; Fegan, S.; Gabrielyan, M. Y.; Gevorgyan, N.; Gilfoyle, G. P.; Giovanetti, K. L.; Girod, F. X.; Goetz, J. T.; Gohn, W.; Golovatch, E.; Gothe, R. W.; Griffioen, K. A.; Guidal, M.; Guo, L.; Hanretty, C.; Heddle, D.; Holtrop, M.; Hyde, C. E.; Ilieva, Y.; Ireland, D. G.; Ishkhanov, B. S.; Isupov, E. L.; Jawalkar, S. S.; Jo, H. S.; Joo, K.; Kalantarians, N.; Keller, D.; Khandaker, M.; Khetarpal, P.; Kim, A.; Kim, W.; Klein, A.; Klein, F. J.; Kubarovsky, V.; Kuleshov, S. V.; Kuznetsov, V.; Lu, H. Y.; MacGregor, I. J. D.; Mao, Y.; Markov, N.; Mayer, M.; McAndrew, J.; McKinnon, B.; Meyer, C. A.; Mineeva, T.; Mirazita, M.; Mokeev, V.; Moutarde, H.; Munevar, E.; Nadel-Turonski, P.; Ni, A.; Niccolai, S.; Niculescu, G.; Niculescu, I.; Osipenko, M.; Ostrovidov, A. I.; Paolone, M.; Pappalardo, L.; Paremuzyan, R.; Park, K.; Park, S.; Pasyuk, E.; Anefalos Pereira, S.; Phelps, E.; Pisano, S.; Pogorelko, O.; Pozdniakov, S.; Price, J. W.; Procureur, S.; Protopopescu, D.; Raue, B. A.; Ricco, G.; Rimal, D.; Ripani, M.; Rosner, G.; Rossi, P.; Sabatié, F.; Saini, M. S.; Salgado, C.; Schott, D.; Schumacher, R. A.; Seraydaryan, H.; Sharabian, Y. G.; Smith, G. D.; Sober, D. I.; Sokhan, D.; Stepanyan, S. S.; Stepanyan, S.; Strauch, S.; Taiuti, M.; Tang, W.; Taylor, C. E.; Tkachenko, S.; Ungaro, M.; Vernarsky, B.; Vineyard, M. F.; Voskanyan, H.; Voutier, E.; Watts, D. P.; Weinstein, L. B.; Weygand, D. P.; Wood, M. H.; Zana, L.; Zachariou, N.; Zhao, B.; Zhao, Z. W.

    2011-11-01

    The influence of cold nuclear matter on lepto-production of hadrons in semi-inclusive deep inelastic scattering is measured using the CLAS detector in Hall B at Jefferson Lab and a 5.014 GeV electron beam. We report the Ks0 multiplicity ratios for targets of C, Fe, and Pb relative to deuterium as a function of the fractional virtual photon energy z transferred to the Ks0 and the transverse momentum squared pT2 of the Ks0. We find that the multiplicity ratios for Ks0 are reduced in the nuclear medium at high z and low pT2, with a trend for the Ks0 transverse momentum to be broadened in the nucleus for large pT2.

  19. Study of KS0 pair production in single-tag two-photon collisions

    Science.gov (United States)

    Masuda, M.; Uehara, S.; Watanabe, Y.; Adachi, I.; Ahn, J. K.; Aihara, H.; Al Said, S.; Asner, D. M.; Atmacan, H.; Aulchenko, V.; Aushev, T.; Ayad, R.; Babu, V.; Badhrees, I.; Bansal, V.; Behera, P.; Berger, M.; Bhardwaj, V.; Bhuyan, B.; Biswal, J.; Bondar, A.; Bonvicini, G.; Bozek, A.; Bračko, M.; Červenkov, D.; Chen, A.; Cheon, B. G.; Chilikin, K.; Cho, K.; Choi, Y.; Choudhury, S.; Cinabro, D.; Czank, T.; Dash, N.; Di Carlo, S.; Doležal, Z.; Drásal, Z.; Dutta, D.; Eidelman, S.; Epifanov, D.; Fast, J. E.; Ferber, T.; Fulsom, B. G.; Garg, R.; Gaur, V.; Gabyshev, N.; Garmash, A.; Gelb, M.; Giri, A.; Goldenzweig, P.; Guido, E.; Haba, J.; Hayasaka, K.; Hayashii, H.; Hedges, M. T.; Hou, W.-S.; Iijima, T.; Inami, K.; Inguglia, G.; Ishikawa, A.; Itoh, R.; Iwasaki, M.; Iwasaki, Y.; Jacobs, W. W.; Jaegle, I.; Jin, Y.; Joo, K. K.; Julius, T.; Kang, K. H.; Karyan, G.; Kawasaki, T.; Kichimi, H.; Kiesling, C.; Kim, D. Y.; Kim, H. J.; Kim, J. B.; Kim, K. T.; Kim, S. H.; Kodyš, P.; Kotchetkov, D.; Križan, P.; Kroeger, R.; Krokovny, P.; Kulasiri, R.; Kuzmin, A.; Kwon, Y.-J.; Lee, I. S.; Lee, S. C.; Li, L. K.; Li, Y.; Li Gioi, L.; Libby, J.; Liventsev, D.; Lubej, M.; Luo, T.; Matsuda, T.; Matvienko, D.; Merola, M.; Miyabayashi, K.; Miyata, H.; Mizuk, R.; Mohanty, G. B.; Moon, H. K.; Mori, T.; Mussa, R.; Nakao, M.; Nakazawa, H.; Nanut, T.; Nath, K. J.; Natkaniec, Z.; Nayak, M.; Niiyama, M.; Nisar, N. K.; Nishida, S.; Ogawa, S.; Okuno, S.; Ono, H.; Onuki, Y.; Pakhlov, P.; Pakhlova, G.; Pal, B.; Park, H.; Paul, S.; Pedlar, T. K.; Pestotnik, R.; Piilonen, L. E.; Ritter, M.; Rostomyan, A.; Russo, G.; Sakai, Y.; Salehi, M.; Sandilya, S.; Santelj, L.; Sanuki, T.; Savinov, V.; Schneider, O.; Schnell, G.; Schwanda, C.; Seidl, R.; Seino, Y.; Senyo, K.; Seon, O.; Sevior, M. E.; Shebalin, V.; Shen, C. P.; Shibata, T.-A.; Shimizu, N.; Shiu, J.-G.; Shwartz, B.; Sokolov, A.; Solovieva, E.; Starič, M.; Strube, J. F.; Sumihama, M.; Sumiyoshi, T.; Takizawa, M.; Tamponi, U.; Tanida, K.; Tenchini, F.; Teramoto, Y.; Uchida, M.; Uglov, T.; Unno, Y.; Uno, S.; Urquijo, P.; Van Hulse, C.; Varner, G.; Vinokurova, A.; Vorobyev, V.; Vossen, A.; Wang, B.; Wang, C. H.; Wang, M.-Z.; Wang, P.; Wang, X. L.; Watanabe, M.; Widmann, E.; Won, E.; Ye, H.; Yuan, C. Z.; Yusa, Y.; Zakharov, S.; Zhang, Z. P.; Zhilich, V.; Zhukova, V.; Zhulanov, V.; Zupanc, A.; Belle Collaboration

    2018-03-01

    We report a measurement of the cross section for KS0 pair production in single-tag two-photon collisions, γ*γ →KS0KS0, for Q2 up to 30 GeV2 , where Q2 is the negative of the invariant mass squared of the tagged photon. The measurement covers the kinematic range 1.0 GeV partial decay widths of the χc 0 and χc 2 mesons are measured as a function of Q2 based on 10 candidate events in total.

  20. Observation of the rare decays $K_{s} \\to \\pi^{0} \\mu^{+} \\mu^{-}$

    CERN Document Server

    Batley, J Richard; Lazzeroni, C; Munday, D J; Patel, M; Slater, M W; AWotton, S; Arcidiacono, R; Bocquet, G; Ceccucci, A; Cundy, Donald C; Doble, Niels T; Falaleev, V; Gatignon, L; Gonidec, A; Grafström, P; Kubischta, Werner; Marchetto, F; Mikulec, I; Norton, A; Panzer-Steindel, B; Rubin, P; Wahl, H; Monnier, E; Swallow, E; Winston, R; Goudzovski, E; Gurev, D; Khristov, P Z; Kekelidze, Vladimir D; Kozhuharov, V; Litov, L; Madigozhin, D T; Molokanova, N A; Potrebenikov, Yu K; Stoynev, S; Zinchenko, A I; Sacco, R; Walker, A; Baldini, W; Dalpiaz, Pietro; Duclos, J; Frabetti, P L; Gianoli, A; Martini, M; Petrucci, F; Scarpa, M; Savrié, M; Bizzeti, A; Calvetti, M; Graziani, G; Iacopini, E; Lenti, M; Martelli, F; Ruggiero, G; Veltri, M; Behler, M; Eppard, K; Eppard, M; Hirstius, A; Kleinknecht, K; Koch, U; Masetti, L; Marouelli, P; Moosbrugger, U; Morales-Morales, C; Peters, A; Wanke, R; Winhart, A; Dabrowski, A; Fonseca-Martin, T; Szleper, M; Velasco, M; Anzivino, Giuseppina; Cenci, P; Imbergamo, E; Lamanna, G; Lubrano, P; Michetti, A; Nappi, A; Pepé, M; Petrucci, M C; Piccini, M; Valdata, M; Cerri, C; Collazuol, G; Costantini, F; Fantechi, R; Fiorini, Luca; Giudici, Sergio; Mannelli, I; Pierazzini, G M; Sozzi, M; Cheshkov, C; Chèze, J B; De Beer, M; Debu, P; Gouge, G; Marel, Gérard; Mazzucato, E; Peyaud, B; Vallage, B; Holder, M; Maier, A; Ziolkowski, M; Biino, C; Cartiglia, N; Clemencic, M; Goy-Lopez, S; Menichetti, E; Pastrone, N; Wislicki, W; Dibon, Heinz; Jeitler, Manfred; Markytan, Manfred; Neuhofer, G; Widhalm, L

    2004-01-01

    A search for the decay Ks -> pi0 mu+ mu- has been made by the NA48/1 Colaboration at the CERN SPS accelerator. The data were collected during 2002 with a high intensity Ks beam. Six events were found with a background expectation of 0.22^+0.18_0.11 events. Using a vector matrix element and unit form factor, the measured branching ratio is B(Ks -> pi0 mu+ mu-) = [2.9^+1.5_1.2(stat) +- 0.2 (syst)] x 10^9.

  1. Search for a narrow baryonic state decaying to pKS0 and p‾KS0 in deep inelastic scattering at HERA

    Directory of Open Access Journals (Sweden)

    H. Abramowicz

    2016-08-01

    Full Text Available A search for a narrow baryonic state in the pKS0 and p‾KS0 system has been performed in ep collisions at HERA with the ZEUS detector using an integrated luminosity of 358pb−1 taken in 2003–2007. The search was performed with deep inelastic scattering events at an ep centre-of-mass energy of 318GeV for exchanged photon virtuality, Q2, between 20 and 100GeV2. Contrary to evidence presented for such a state around 1.52 GeV in a previous ZEUS analysis using a sample of 121 pb−1 taken in 1996–2000, no resonance peak was found in the p(p‾KS0 invariant-mass distribution in the range 1.45–1.7 GeV. Upper limits on the production cross section are set.

  2. Procedural Learning and Memory Rehabilitation in Korsakoff’s Syndrome - a Review of the Literature

    NARCIS (Netherlands)

    Oudman, Erik; Nijboer, Tanja C W; Postma, Albert; Wijnia, Jan W.; Van der Stigchel, Stefan

    2015-01-01

    Korsakoff’s syndrome (KS) is a chronic neuropsychiatric disorder caused by alcohol abuse and thiamine deficiency. Patients with KS show restricted autonomy due to their severe declarative amnesia and executive disorders. Recently, it has been suggested that procedural learning and memory are

  3. Klinefelter′s syndrome: Report of a case from Sokoto, Northern Nigeria and review of literature

    Directory of Open Access Journals (Sweden)

    Anas A Sabir

    2013-01-01

    Full Text Available Herein, we report, review and discuss the literature on Klinefelter′s syndrome (KS with our findings during an out-patient medical clinic at Usmanu Danfodiyo University Teaching Hospital Sokoto, Nigeria. The aim of the report is to create awareness and highlight to clinicians, the occurrence of KS in patients with infertility.

  4. Preliminary Analysis of Rapid Condensation Experiment with MARS-KS Code

    Energy Technology Data Exchange (ETDEWEB)

    Bae, Jae Ho; Jun, Hwang Yong; Jeong, Hae Yong [Sejong University, Seoul (Korea, Republic of)

    2016-05-15

    In the present study, the rapid condensation experiment performed in MANOTEA facility is analyzed with the MARS-KS code. It is known that there exists some limitation with a system code to predict this kind of a very active condensation due to direct mixing of cold injection flow and steam. Through the analysis we investigated the applicability of MARS-KS code for the design of various passive safety systems in the future. The configuration of the experimental facility MANOTEA, which has been constructed at the University of Maryland - United States Naval Academy, is described and the modeling approach using the MARS-KS code is also provided. The preliminary result shows that the MARS-KS predicts the general trend of pressure and temperature in the condensing part correctly. However, it is also found that there exist some limitations in the simulation such as an unexpected pressure peak or a sudden temperature change.

  5. [Marie Heiberg. Üks naine kurbade silmadega] / Marko Mägi

    Index Scriptorium Estoniae

    Mägi, Marko

    2010-01-01

    Arvustus: Heiberg, Marie. Üks naine kurbade silmadega : Eesti luuletaja Marie Heibergi õnne ja valu, rõõmu ja mure lugu kirjades / [koostajad Iivi Lepik, Kirsten Simmo]. Tallinn : Eesti Teatri- ja Muusikamuuseum : SE & JS, 2010

  6. Klinefelter syndrome, insulin resistance, metabolic syndrome, and diabetes: review of literature and clinical perspectives.

    Science.gov (United States)

    Salzano, Andrea; D'Assante, Roberta; Heaney, Liam M; Monaco, Federica; Rengo, Giuseppe; Valente, Pietro; Pasquali, Daniela; Bossone, Eduardo; Gianfrilli, Daniele; Lenzi, Andrea; Cittadini, Antonio; Marra, Alberto M; Napoli, Raffaele

    2018-03-23

    Klinefelter syndrome (KS), the most frequent chromosomic abnormality in males, is associated with hypergonadotropic hypogonadism and an increased risk of cardiovascular diseases (CVD). The mechanisms involved in increasing risk of cardiovascular morbidity and mortality are not completely understood. This review summarises the current understandings of the complex relationship between KS, metabolic syndrome and cardiovascular risk in order to plan future studies and improve current strategies to reduce mortality in this high-risk population. We searched PubMed, Web of Science, and Scopus for manuscripts published prior to November 2017 using key words "Klinefelter syndrome" AND "insulin resistance" OR "metabolic syndrome" OR "diabetes mellitus" OR "cardiovascular disease" OR "testosterone". Manuscripts were collated, studied and carried forward for discussion where appropriate. Insulin resistance, metabolic syndrome, and type 2 diabetes are more frequently diagnosed in KS than in the general population; however, the contribution of hypogonadism to metabolic derangement is highly controversial. Whether this dangerous combination of risk factors fully explains the CVD burden of KS patients remains unclear. In addition, testosterone replacement therapy only exerts a marginal action on the CVD system. Since fat accumulation and distribution seem to play a relevant role in triggering metabolic abnormalities, an early diagnosis and a tailored intervention strategy with drugs aimed at targeting excessive visceral fat deposition appear necessary in patients with KS.

  7. Do KS/BE Preschools Help Hawaiian Children Succeed in the Public Schools?

    Science.gov (United States)

    Heath, Robert W.; Plett, Jerald D.

    Presented are results of the tracking of the 1985-86 cohort of preschool alumni of the Kamehameha Schools/Bishop Estate (KS/BE) as they entered and completed kindergarten in the 1986-87 school year. During the 1985-86 program year, 100 preschoolers were enrolled in the KS/BE Pre-kindergarten Education Program at five sites. An additional group of…

  8. Investigation of possible application of the chromazole KS reagent to the analytical chemistry of vanadium

    International Nuclear Information System (INIS)

    Gordeeva, M.N.; Fedorova, L.N.; Basargin, N.N.; Rozovskij, Yu.G.

    1978-01-01

    Complex formation of vanadium (4) with chromazole KS has been investigated by the spectrophotometric method. It has been found that two complex compounds are formed: Me:R=1:1 (pH=4.0) and Me:R=1:2 (pH=6.2). The chemistry of the interaction of vanadium (4) with chromazole KS has been studied. A method of the photometric determination of vanadium (4) in standard steels and optical glasses has been developed

  9. Anti-Müllerian Hormone and Ovarian Morphology in Women With Isolated Hypogonadotropic Hypogonadism/Kallmann Syndrome: Effects of Recombinant Human FSH.

    Science.gov (United States)

    Bry-Gauillard, Hélène; Larrat-Ledoux, Florence; Levaillant, Jean-Marc; Massin, Nathalie; Maione, Luigi; Beau, Isabelle; Binart, Nadine; Chanson, Philippe; Brailly-Tabard, Sylvie; Hall, Janet E; Young, Jacques

    2017-04-01

    Isolated hypogonadotropic hypogonadism (IHH), characterized by gonadotropin deficiency and absent puberty, is very rare in women. IHH prevents pubertal ovarian stimulation, but anti-Müllerian hormone (AMH) and antral follicle count (AFC) have not been studied. (1) To compare, in IHH vs controls, AMH, ovarian volume (OV), and AFC. (2) To compare, in IHH, ovarian responses to recombinant human follicle-stimulating hormone (rhFSH) and rhFSH plus recombinant human luteinizing hormone (rhLH). Sixty-eight IHH women; 51 matched healthy women. Serum LH, FSH, sex steroids, inhibin B (InhB), AMH, and OV and AFC (sonography) were compared. Ovarian response during rhFSH administration was assessed in 12 IHH women with low AMH levels and low AFC and compared with hormonal changes observed in six additional IHH women receiving rhFSH plus rhLH. InhB was lower in IHH than in controls. AMH levels were also significantly lower in the patients, but two-thirds had normal values. Mean OV and total, larger, and smaller AFCs were lower in IHH than in controls. Ovarian stimulation by rhFSH led to a significant increase in serum estradiol and InhB levels and in the number of larger antral follicles. AMH and smaller AFC increased early during rhFSH stimulation but then declined despite continued stimulation. rhFSH plus rhLH stimulation led to a significantly higher increase in estradiol levels but to similar changes in circulating InhB and AMH than with rhFSH alone. IHH women have both low AMH levels and low AFC. However, their decrease can be reversed by follicle-stimulating hormone. Serum AMH and AFC should not serve as prognostic markers of fertility in this population. Copyright © 2017 by the Endocrine Society

  10. A Constructive Extension of the Characterization on Potentially Ks,t-Bigraphic Pairs

    Directory of Open Access Journals (Sweden)

    Guo Ji-Yun

    2017-02-01

    Full Text Available Let Ks,t be the complete bipartite graph with partite sets of size s and t. Let L1 = ([a1, b1], . . . , [am, bm] and L2 = ([c1, d1], . . . , [cn, dn] be two sequences of intervals consisting of nonnegative integers with a1 ≥ a2 ≥ . . . ≥ am and c1 ≥ c2 ≥ . . . ≥ cn. We say that L = (L1; L2 is potentially Ks,t (resp. As,t-bigraphic if there is a simple bipartite graph G with partite sets X = {x1, . . . , xm} and Y = {y1, . . . , yn} such that ai ≤ dG(xi ≤ bi for 1 ≤ i ≤ m, ci ≤ dG(yi ≤ di for 1 ≤ i ≤ n and G contains Ks,t as a subgraph (resp. the induced subgraph of {x1, . . . , xs, y1, . . . , yt} in G is a Ks,t. In this paper, we give a characterization of L that is potentially As,t-bigraphic. As a corollary, we also obtain a characterization of L that is potentially Ks,t-bigraphic if b1 ≥ b2 ≥ . . . ≥ bm and d1 ≥ d2 ≥ . . . ≥ dn. This is a constructive extension of the characterization on potentially Ks,t-bigraphic pairs due to Yin and Huang (Discrete Math. 312 (2012 1241–1243.

  11. Update on the Genetics of Idiopathic Hypogonadotropic Hypogonadism

    OpenAIRE

    Topaloğlu, A. Kemal

    2017-01-01

    Traditionally, idiopathic hypogonadotropic hypogonadism (IHH) is divided into two major categories: Kallmann syndrome (KS) and normosmic IHH (nIHH). To date, inactivating variants in more than 50 genes have been reported to cause IHH. These mutations are estimated to account for up to 50% of all apparently hereditary cases. Identification of further causative gene mutations is expected to be more feasible with the increasing use of whole exome/genome sequencing. Presence of more than one IHH-...

  12. The role of genes, intelligence, personality, and social engagement in cognitive performance in Klinefelter syndrome

    DEFF Research Database (Denmark)

    Skakkebæk, Anne; Moore, Philip J.; Pedersen, Anders Degn

    2017-01-01

    of controls matched for age and years of education. Methods: Sixty-nine patients with KS and 69 controls were assessed in terms of IQ, NEO personality inventory, the Autism Spectrum Quotient (AQ) scale, and measures of cognitive performance reflecting working memory and executive function. Results: Patients......Introduction: The determinants of cognitive deficits among individuals with Klinefelter syndrome (KS) are not well understood. This study was conducted to assess the impact of general intelligence, personality, and social engagement on cognitive performance among patients with KS and a group...... with KS performed more poorly on memory and executive-function tasks. Patients with KS also exhibited greater neuroticism and less extraversion, openness, and conscientiousness than controls. Memory deficits among patients with KS were associated with lower intelligence, while diminished executive...

  13. Anterograde Episodic Memory in Korsakoff Syndrome

    Science.gov (United States)

    Fama, Rosemary; Pitel, Anne-Lise; Sullivan, Edith V.

    2016-01-01

    A profound anterograde memory deficit for information, regardless of the nature of the material, is the hallmark of Korsakoff syndrome, an amnesic condition resulting from severe thiamine (vitamin B1) deficiency. Since the late nineteenth century when the Russian physician, S. S. Korsakoff, initially described this syndrome associated with “polyneuropathy,” the observed global amnesia has been a primary focus of neuroscience and neuropsychology. In this review we highlight the historical studies that examined anterograde episodic memory processes in KS, present a timeline and evidence supporting the myriad theories proffered to account for this memory dysfunction, and summarize what is known about the neuroanatomical correlates and neural systems presumed affected in KS. Rigorous study of KS amnesia and associated memory disorders of other etiologies provide evidence for distinct mnemonic component processes and neural networks imperative for normal declarative and nondeclarative memory abilities and for mnemonic processes spared in KS, from whence emerged the appreciation that memory is not a unitary function. Debate continues regarding the qualitative and quantitative differences between KS and other amnesias and what brain regions and neural pathways are necessary and sufficient to produce KS amnesia. PMID:22644546

  14. Anterograde episodic memory in Korsakoff syndrome.

    Science.gov (United States)

    Fama, Rosemary; Pitel, Anne-Lise; Sullivan, Edith V

    2012-06-01

    A profound anterograde memory deficit for information, regardless of the nature of the material, is the hallmark of Korsakoff syndrome, an amnesic condition resulting from severe thiamine (vitamin B1) deficiency. Since the late nineteenth century when the Russian physician, S. S. Korsakoff, initially described this syndrome associated with "polyneuropathy," the observed global amnesia has been a primary focus of neuroscience and neuropsychology. In this review we highlight the historical studies that examined anterograde episodic memory processes in KS, present a timeline and evidence supporting the myriad theories proffered to account for this memory dysfunction, and summarize what is known about the neuroanatomical correlates and neural systems presumed affected in KS. Rigorous study of KS amnesia and associated memory disorders of other etiologies provide evidence for distinct mnemonic component processes and neural networks imperative for normal declarative and nondeclarative memory abilities and for mnemonic processes spared in KS, from whence emerged the appreciation that memory is not a unitary function. Debate continues regarding the qualitative and quantitative differences between KS and other amnesias and what brain regions and neural pathways are necessary and sufficient to produce KS amnesia.

  15. Korsakoff’s syndrome: a critical review

    Directory of Open Access Journals (Sweden)

    Arts NJM

    2017-11-01

    Full Text Available Nicolaas JM Arts,1,2 Serge JW Walvoort,1 Roy PC Kessels1,3,4 1Centre of Excellence for Korsakoff and Alcohol-Related Cognitive Disorders, Vincent van Gogh Institute for Psychiatry, Venray, 2Neuropsychiatry Center Thalamus, Institution for Integrated Mental Health Care Pro Persona, Wolfheze, 3Department of Neuropsychology and Rehabilitation Psychology, Donders Institute for Brain, Cognition and Behaviour, Radboud University, 4Department of Medical Psychology, Radboud University Medical Center, Nijmegen, the Netherlands Abstract: In this review, we present a survey on Korsakoff’s syndrome (KS, a residual syndrome in patients who suffered from a Wernicke encephalopathy (WE that is predominantly characterized by global amnesia, and in more severe cases also by cognitive and behavioral dysfunction. We describe the history of KS and its definition, its epidemiology, and the lack of consensus criteria for its diagnosis. The cognitive and behavioral symptoms of KS, which include anterograde and retrograde amnesia, executive dysfunction, confabulation, apathy, as well as affective and social-cognitive impairments, are discussed. Moreover, recent insights into the underlying neurocognitive mechanisms of these symptoms are presented. In addition, the evidence so far on the etiology of KS is examined, highlighting the role of thiamine and alcohol and discussing the continuity hypothesis. Furthermore, the neuropathology of KS is reviewed, focusing on abnormalities in the diencephalon, including the mammillary bodies and thalamic nuclei. Pharmacological treatment options and nonpharmacological interventions, such as those based on cognitive rehabilitation, are discussed. Our review shows that thiamine deficiency (TD is a crucial factor in the etiology of KS. Although alcohol abuse is by far the most important context in which TD occurs, there is no convincing evidence for an essential contribution of ethanol neurotoxicity (EN to the development of WE or to

  16. A case of Klinefelter syndrome with hypersexual desire

    Science.gov (United States)

    Perampalam, Sumathy; Barker, Anthony; Nolan, Christopher J

    2017-01-01

    Klinefelter syndrome (KS) is a chromosomal disorder affecting males, with the typical karyotype of 47,XXY due to a supernumerary X chromosome, which causes progressive testicular failure resulting in androgen deficiency and infertility. Despite it being the most common sex chromosomal disorder, its diagnosis is easily missed. In addition to its classical clinical features of tall stature, gynaecomastia, small testes, and symptoms and signs of hypogonadism including infertility, KS is also often associated with neurocognitive, behavioural and psychiatric disorders. We present a 44-year-old man with KS who, despite having erectile dysfunction, paradoxically had increased libido. He used sildenafil to overcome his erectile dysfunction. Hypersexuality was manifested by very frequent masturbation, multiple sexual partners most of whom were casual, and a sexual offence conviction at the age of 17 years. Discussion focuses on the frequent failure of clinicians to diagnose KS, the neurocognitive, behavioural and psychiatric aspects of KS, this unusual presentation of hypersexuality in a man with KS, and the challenges of medical management of hypogonadism in a man with a history of a sexual offence. Learning points: Klinefelter syndrome (KS) is common in men (about 1 in 600 males), but the diagnosis is very often missed. In addition to classic features of hypogonadism, patients with KS can often have associated neurocognitive, behavioural and/or psychiatric disorders. More awareness of the association between KS and difficulties related to verbal skills in boys could improve rates of early diagnosis and prevent longer-term psychosocial disability. Hypersexuality in the context of hypogonadism raises the possibility of sex steroid independent mechanistic pathways for libido. Testosterone replacement therapy in KS with hypersexuality should be undertaken with caution using a multidisciplinary team approach. PMID:28883919

  17. High normal testosterone levels in infants with non-mosaic Klinefelter's syndrome

    DEFF Research Database (Denmark)

    Aksglaede, Lise; Petersen, Jørgen H; Main, Katharina M

    2007-01-01

    Klinefelter's syndrome (KS) is associated with hypergonadotrophic hypogonadism in adulthood. However, limited information exists about the age at which hypogonadism occurs. The hypothalamic-pituitary-gonadal (HPG) axis is transiently activated during the first months of life, offering...

  18. Implantation of cultured thymic fragments in patients with acquired immunodeficiency syndrome

    NARCIS (Netherlands)

    Danner, S. A.; Schuurman, H. J.; Lange, J. M.; Gmelig Meyling, F. H.; Schellekens, P. T.; Huber, J.; Kater, L.

    1986-01-01

    Cultured thymic fragments were implanted in one patient with acquired immunodeficiency syndrome (AIDS)-related complex (ARC) and in eight AIDS patients with opportunistic infections (OIs, four patients), Kaposi's sarcoma (KS, two patients), or both (two patients). Thereafter, objective clinical

  19. High normal testosterone levels in infants with non-mosaic Klinefelter's syndrome

    DEFF Research Database (Denmark)

    Aksglaede, Lise; Petersen, Jørgen H; Main, Katharina M

    2007-01-01

    Klinefelter's syndrome (KS) is associated with hypergonadotrophic hypogonadism in adulthood. However, limited information exists about the age at which hypogonadism occurs. The hypothalamic-pituitary-gonadal (HPG) axis is transiently activated during the first months of life, offering the opportu...

  20. Pharmaceutical significance of Leuconostoc mesenteroides KS-TN11 isolated from Nile Tilapia, Oreochromis niloticus

    Directory of Open Access Journals (Sweden)

    Bilal Ahmad Paray

    2018-05-01

    Full Text Available Aquatic animals are known for their myriad of beneficial bacteria with diverse biologically active compounds. The current study was aimed to isolate and characterize potentially beneficial lactic acid bacteria from Nile Tilapia and evaluate their pharmaceutical applications. The fish samples were dissected and stomach, intestine, and gills were collected and serially diluted for the isolation of lactic acid bacteria (LAB on BCP agar media. Identification of isolate was carried by biochemical and molecular characterization using API kit and 16S rRNA gene sequencing analysis, respectively. Further, KS-TN11 was assessed for α-glucosidase inhibitory potential using the chromogenic method. A lactic acid bacterium KS-TN11 was isolated from the stomach of Nile Tilapia and identified as Leuconostoc mesenteroides. Effect of KS-TN11 on lipid accumulation in adipocytes was done by using Oil Red O staining. The isolate showed strong antibacterial activity against a number of pathogenic bacteria in vitro. In addition, L. mesenteroides KS-TN11 KS-TN11 (50 mg/ml and 100 mg/ml tends to inhibit adipogenesis in 3T3-L1 adipocytes and thus may have possible anti-obesity effects. Moreover, L. mesenteroides KS-TN11 exhibited substantial α–glucosidase inhibitory activities by 41.33% at 50 mg/ml and 64% at 100 mg/ml, respectively. The bacterium showed potent antibacterial activity against a number of pathogenic bacteria; in addition to alpha-glucosidase activity, and inhibition of lipid accumulation in 3T3-L1 cell line. These results reinforce KS-TN11 as a novel bacterium with an impending pharmaceutical application. Keywords: Lactic acid bacteria, Antimicrobial, Fish microflora, Anti-diabetic

  1. Clinical and biological parameters in 166 boys, adolescents and adults with nonmosaic Klinefelter syndrome: a Copenhagen experience

    DEFF Research Database (Denmark)

    Aksglaede, Lise; Skakkebaek, Niels E; Almstrup, Kristian

    2011-01-01

    Klinefelter syndrome (KS) is the most frequent sex chromosome disorder in males, but the phenotype varies greatly and is therefore highly under-diagnosed. We aimed at describing the phenotypic characteristics throughout life from clinical follow-up of our large cohort of patients with KS....

  2. Aberrant ocular architecture and function in patients with Klinefelter syndrome.

    Science.gov (United States)

    Brand, Cristin; Zitzmann, Michael; Eter, Nicole; Kliesch, Sabine; Wistuba, Joachim; Alnawaiseh, Maged; Heiduschka, Peter

    2017-10-13

    Klinefelter Syndrome (KS), the most common chromosomal disorder in men (47,XXY), is associated with numerous comorbidities. Based on a number of isolated case reports, we performed the first systematic and comprehensive evaluation of eye health in KS patients with a focus on ocular structure and vascularization. Twenty-one KS patients and 26 male and 38 female controls underwent a variety of non-invasive examinations investigating ocular morphology (examination of retinal thickness, optic nerve head, and cornea) and function (visual field testing and quantification of ocular vessel density by optical coherence tomography angiography). In comparison to healthy controls, KS patients exhibited a smaller foveal avascular zone and a decreased retinal thickness due to a drastically thinner outer nuclear layer. The cornea of KS patients showed a decreased peripheral thickness and volume. In perimetry evaluation, KS patients required brighter stimuli and gave more irregular values. KS patients show an ocular phenotype including morphological and functional features, which is very likely caused by the supernumerary X chromosome. Thus, KS should not be limited to infertility, endocrine dysfunction, neurocognitive and psychosocial comorbidities. Defining an aberrant ocular morphology and function, awareness for possible eye problems should be raised.

  3. Simulation of loss of feedwater transient of MASLWR test facility by MARS-KS code

    Energy Technology Data Exchange (ETDEWEB)

    Park, Juyeop [Korea Institute of Nuclear Safety, Daejeon (Korea, Republic of)

    2013-05-15

    MASLWR test facility is a mock-up of a passive integral type reactor equipped with helical coil steam generator. Since SMART reactor which is being current developed domestically also adopts helical coil steam generator, KINS has joined this ICSP to evaluate performance of domestic regulatory audit thermal-hydraulic code (MARS-KS code) in various respects including wall-to-fluid heat transfer model modification implemented in the code by independent international experiment database. In the ICSP, two types of transient experiments have been focused and they are loss of feedwater transient with subsequent ADS operation and long term cooling (SP-2) and normal operating conditions at different power levels (SP-3). In the present study, KINS simulation results by the MARS-KS code (KS-002 version) for the SP-2 experiment are presented in detail and conclusions on MARS-KS code performance drawn through this simulation is described. Performance of the MARS-KS code is evaluated through the simulation of the loss of feedwater transient of the MASLWR test facility. Steady state run shows helical coil specific heat transfer models implemented in the code is reasonable. However, through the transient run, it is also found that three-dimensional effect within the HPC and axial conduction effect through the HTP are not well reproduced by the code.

  4. Comparison of SPACE to MARS-KS under SUBO experimental conditions

    International Nuclear Information System (INIS)

    Kim, Min-Gil; Lee, Wonwoong; Lee, Jeong Ik; Bang, Young Seok

    2015-01-01

    To evaluate safety of a Korean Nuclear Power Plant (NPP) MARS-KS code is being used by the Korean regulator. The governing equations of MARS-KS are based on two-phase and two-fluid model. Recently, SPACE (Safety and Performance Analysis CodE for nuclear power plants) was developed by a consortium led by Korea Hydro and Nuclear Power Co., Ltd. (KHNP), which the code is aimed for evaluating the safety of the designed nuclear power plant. The governing equations of SPACE are based on two-phase (liquid and gas phase) three-fluid (continuous liquid, gas and droplet) model. However, MARS-KS and SPACE have different governing equations, as well as model and correlations implemented in two codes. Due to this reason, the authors are studying the difference in the analysis result of SET (Separate Effect Test) of each code.. To compare the SPACE and MARS-KS performances, the authors chose SUBO experiment as the first reference case. Input deck of each code was prepared. The results from the two codes were compared to the experimental data, but due to the lack of information on the uncertainties it is too early to conclude the code performance. However, from the obtained analysis results, some differences between MARS-KS and SPACE are observed. Especially, flow regimes at heated region are considerably different. More detailed analysis of the flow regime and its effect in MARS-KS and SPACE analysis results will be followed in the near future. The heat transfer coefficient and friction factor at the interface and at the wall will be compared with similar method used in this study

  5. LHCb: Measurement of the time-dependent CP asymmetry in $B^{0} \\rightarrow J/\\psi K_S^0$

    CERN Multimedia

    Szilard, D

    2012-01-01

    The LHCb is one of the LHC experiments, specially designed to study CP violation and rare decays of b-quark. In this scenario, the decay $B^{0} \\rightarrow J/\\psi K_S^0$ is a well-known mode to study CP violation. The final state is the same for both CP eigenstates $B^0$ and $\\overline{B^0}$, which enables the interference through the b-quark oscillations.The CKM angle $\\beta$ can be extracted from the above decay. The time dependent decay rate asymmetry can be written as: $$ \\mathcal{A}_{J/\\psi K_S^0} (t) = S_{J/\\psi K_S^0} \\sin(\\Delta m_d t) - C_{J/\\psi K_S^0} \\cos( \\Delta m_d t) $$ where, in the standard model, the parameters $S_{J/\\psi K_S^0}$ and $C_{J/\\psi K_S^0}$ are connected to the CKM angle $\\beta$ through: $$S_{J/\\psi K_S^0} = \\sin 2 \\beta \\, \\rm{ and } \\, C_{J/\\psi K_S^0} = 0 $$ Here we report the time-dependent analysis in the $B^{0} \\rightarrow J/\\psi (\\mu^{+} \\mu^{-}) K_S^0 (\\pi^{+} \\pi^{-})$ decays. Using the $1fb^{-1}$ of integrated luminosity data collected in 2011 at LHCb operat...

  6. Revisiting the continuum hypothesis: toward an in-depth exploration of executive functions in korsakoff syndrome.

    Science.gov (United States)

    Brion, Mélanie; Pitel, Anne-Lise; Beaunieux, Hélène; Maurage, Pierre

    2014-01-01

    Korsakoff syndrome (KS) is a neurological state mostly caused by alcohol-dependence and leading to disproportionate episodic memory deficits. KS patients present more severe anterograde amnesia than Alcohol-Dependent Subjects (ADS), which led to the continuum hypothesis postulating a progressive increase in brain and cognitive damages during the evolution from ADS to KS. This hypothesis has been extensively examined for memory but is still debated for other abilities, notably executive functions (EF). EF have up to now been explored by unspecific tasks in KS, and few studies explored their interactions with memory. Exploring EF in KS by specific tasks based on current EF models could thus renew the exploration of the continuum hypothesis. This paper will propose a research program aiming at: (1) clarifying the extent of executive dysfunctions in KS by tasks focusing on specific EF subcomponents; (2) determining the differential EF deficits in ADS and KS; (3) exploring EF-memory interactions in KS with innovative tasks. At the fundamental level, this exploration will test the continuum hypothesis beyond memory. At the clinical level, it will propose new rehabilitation tools focusing on the EF specifically impaired in KS.

  7. Revisiting the continuum hypothesis: towards an in-depth exploration of executive functions in Korsakoff syndrome.

    Directory of Open Access Journals (Sweden)

    Mélanie eBrion

    2014-07-01

    Full Text Available Korsakoff syndrome (KS is a neurological state mostly caused by alcohol-dependence and leading to disproportionate episodic memory deficits. KS patients present more severe anterograde amnesia than alcohol-dependent subjects (ADS, which led to the continuum hypothesis postulating a progressive increase in brain and cognitive damages during the evolution from ADS to KS. This hypothesis has been extensively examined for memory but is still debated for other abilities, notably executive functions (EF. EF have up to now been explored by unspecific tasks in KS, and few studies explored their interactions with memory. Exploring EF in KS by specific tasks based on current EF models could thus renew the exploration of the continuum hypothesis. This paper will propose a research program aiming at: (1 clarifying the extent of executive dysfunctions in KS by tasks focusing on specific EF subcomponents; (2 determining the differential EF deficits in ADS and KS; (3 exploring EF-memory interactions in KS with innovative tasks. At the fundamental level, this exploration will test the continuum hypothesis beyond memory. At the clinical level, it will propose new rehabilitation tools focusing on the EF specifically impaired in KS.

  8. Structural and Functional Neuroimaging in Klinefelter (47,XXY) Syndrome: A Review of the Literature and Preliminary Results from a Functional Magnetic Resonance Imaging Study of Language

    Science.gov (United States)

    Steinman, Kyle; Ross, Judith; Lai, Song; Reiss, Allan; Hoeft, Fumiko

    2009-01-01

    Klinefelter (47,XXY) syndrome (KS), the most common form of sex-chromosomal aneuploidy, is characterized by physical, endocrinologic, and reproductive abnormalities. Individuals with KS also exhibit a cognitive/behavioral phenotype characterized by language and language-based learning disabilities and executive and attentional dysfunction in the…

  9. Performing below the Targeted Level: An Investigation into KS3 Pupils' Attitudes towards Mathematics

    Science.gov (United States)

    Mirza, Anusha; Hussain, Nasreen

    2018-01-01

    This study sets out to investigate the attitude KS3 pupils have towards mathematics and the factors that influence this attitude. A case study approach was used as the pupils were a unit of the school under study and a survey method was chosen to provide scope to the study. Purposeful sampling was employed for the selection of 200 pupils from…

  10. 75 FR 65404 - Security Savings Bank, FSB; Olathe, KS; Notice of Appointment of Receiver

    Science.gov (United States)

    2010-10-22

    ... DEPARTMENT OF THE TREASURY Office of Thrift Supervision Security Savings Bank, FSB; Olathe, KS... section 5(d)(2) of the Home Owners' Loan Act, the Office of Thrift Supervision (OTS) has duly appointed the Federal Deposit Insurance Corporation as sole Receiver for Security Savings Bank, FSB, Olathe...

  11. NuSTAR discovery of a cyclotron line in KS 1947+300

    DEFF Research Database (Denmark)

    Fürst, Felix; Pottschmidt, Katja; Wilms, Jörn

    2014-01-01

    -resolved spectroscopy reveals that the strength of the feature changes strongly with pulse phase and is most prominent during the broad minimum of the pulse profile. At the same phases the line also becomes visible in the first and third observation at the same energy. This discovery implies that KS 1947+300 has...

  12. Presidendi aiapidu läks maksma üle 72 000 krooni / Allar Viivik

    Index Scriptorium Estoniae

    Viivik, Allar

    2008-01-01

    4. juunil 2008 tähistas presidendi kantselei Kadrioru roosiaias kantselei 15. sünnipäeva. Peol esines Vaiko Eplik & Eliit. Presidendi avalike suhete nõuniku Toomas Sildami sõnul läks pidu maksma 72 082 krooni

  13. Amplitude analysis of the D+ -> K-S(0)pi + (0)(pi) Dalitz plot

    NARCIS (Netherlands)

    Ablikim, M.; Achasov, M. N.; Ai, X. C.; Albayrak, O.; Albrecht, M.; Ambrose, D. J.; An, F. F.; An, Q.; Bai, J. Z.; Ferroli, R. Baldini; Ban, Y.; Bennett, J. V.; Bertani, M.; Bian, J. M.; Boger, E.; Bondarenko, O.; Boyko, I.; Braun, S.; Briere, R. A.; Cai, H.; Cai, X.; Cakir, O.; Calcaterra, A.; Cao, G. F.; Cetin, S. A.; Chang, J. F.; Chelkov, G.; Chen, G.; Chen, H. S.; Chen, J. C.; Chen, M. L.; Chen, S. J.; Chen, X.; Chen, X. R.; Chen, Y. B.; Cheng, H. P.; Chu, X. K.; Chu, Y. P.; Cronin-Hennessy, D.; Dai, H. L.; Dai, J. P.; Dedovich, D.; Deng, Z. Y.; Denig, A.; Denysenko, I.; Kalantar-Nayestanaki, N.; Kavatsyuk, M.; Loehner, H.; Messchendorp, J. G.; Moeini, H.

    2014-01-01

    We perform an analysis of the D+ -> K-S(0)pi + (0)(pi) Dalitz plot using a data set of 2.92 fb(-1) of e(+) e(-) collisions at the (3770) mass accumulated by the BESIII experiment, in which 166694 candidate events are selected with a background of 15.1%. The Dalitz plot is found to be well

  14. Kasetoht annab ainest ka teadustööks / Ave Matsin

    Index Scriptorium Estoniae

    Matsin, Ave, 1973-

    2014-01-01

    TÜ Viljandi Kultuuriakadeemia rahvusliku käsitöö osakonna pärandtehnoloogia magistrant Andres Rattassepp valis oma magistritöö teemaks tohutöö. Üks tema juhendajaid on doktorikraadiga tohutöömeister Vladimir Jarish

  15. Simulation of the KAERI PASCAL Test with MARS-KS and TRACE Codes

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kyung Won; Cheong, Aeju; Shin, Andong; Cho, Min Ki [Korea Institute of Nuclear Safety, Daejeon (Korea, Republic of)

    2016-10-15

    In order to validate the operational performance of the PAFS, KAERI has performed the experimental investigation using the PASCAL (PAFS Condensing heat removal Assessment Loop) facility. In this study, we simulated the KAERI PASCAL SS-540-P1 test with MARS-KS V1.4 and TRACE V5.0 p4 codes to assess the code predictability for the condensation heat transfer inside the passive auxiliary feedwater system. We simulated the KAERI PASCAL SS-540-P1 test with MARS-KS V1.4 and TRACE V5.0 p4 codes to assess the code predictability for the condensation heat transfer inside the passive auxiliary feedwater system. The calculated results of heat flux, inner wall surface temperature of the condensing tube, fluid temperature, and steam mass flow rate are compared with the experimental data. The result shows that the MARS-KS generally under-predict the heat fluxes. The TRACE over-predicts the heat flux at tube inlet region and under-predicts it at tube outlet region. The TRACE prediction shows larger amount of steam condensation by about 3% than the MARS-KS prediction.

  16. The Ks-band Tully–Fisher Relation – A Determination of the Hubble ...

    Indian Academy of Sciences (India)

    to 16 galaxy clusters and 218 ScI galaxies with minimum distances of .... In this work, the value of H0 is re-evaluated using the Ks-band TFR and taking ...... research. This research has made use of the NASA/IPAC Extragalactic Database (NED).

  17. Kuldlõvi läks Veneetsias Saksa paviljonile / Harry Liivrand

    Index Scriptorium Estoniae

    Liivrand, Harry, 1961-

    2001-01-01

    49. Veneetsia biennaali peapreemia Kuldlõvi läks Saksa paviljonile, kus eksponeeritakse Gregor Schneideri environment'i nn. surnud maja teemal. Kuldlõvi elutöö eest anti Ameerika skulptorile Richard Serrale ning tema kaasmaalasele maalikunstnik Cy Twomblyle

  18. Comparison of MARS-KS and SPACE for UPTF TRAM Loop Seal Clearing Experiment

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Min Gil; Lee, Won Woong; Lee, Jeong Ik [KAIST, Daejeon (Korea, Republic of); Bang, Young Seok [KINS, Daejeon (Korea, Republic of)

    2016-05-15

    In this study, the authors assessed SPACE code, which was developed by a consortium led by Korea Hydro and Nuclear Power Co., Ltd. (KHNP), now in licensing process and MARS-KS code for UPTF TRAM loop seal clearing experiment to evaluate the code predictability regarding loop seal clearing for supporting the regulatory review. The sensitivity of PT/CT sagging contact angle has been studied. The results of sagging contact angle could explain in different ways. In the case of wide sagging contact angle, the result is quite conservative in the aspect of containment as the heat is well-transferred to moderator. it causes the moderator to heat up. On the other hand, the narrow sagging contact angle results fuel heatup and give limiting condition for fuel integrity. As a result of estimation, a proper application of sagging contact angle is required to provide limiting condition for subsequent analysis. The results from the two codes were compared to the experimental data, but due to the lack of information on the uncertainties it is too early to conclude the both code's performance. However, from the obtained analysis results, some differences between MARS-KS and SPACE are initially observed. Especially, SPACE has larger oscillation in the calculated mass flow rate value than MARS-KS. This phenomenon was observed in comparison of SPACE and MARS-KS CCFL model as well.

  19. 47,XXY Klinefelter syndrome

    DEFF Research Database (Denmark)

    Aksglaede, Lise; Link, Katarina; Giwercman, Aleksander

    2013-01-01

    47,XXY (Klinefelter syndrome) is the most frequent sex chromosomal disorder and affects approximately one in 660 newborn boys. The syndrome is characterized by varying degrees of cognitive, social, behavioral, and learning difficulties and in adulthood additionally primary testicular failure...... with small testes, hypergonadotropic hypogonadism, tall stature, and eunuchoid body proportions. The phenotype is variable ranging from "near-normal" to a significantly affected individual. In addition, newborns with Klinefelter syndrome generally present with a normal male phenotype and the only consistent...... clinical finding in KS is small testes, that are most often not identified until after puberty. Decreased awareness of this syndrome among health professionals and a general perception that all patients with 47,XXY exhibit the classic textbook phenotype results in a highly under-diagnosed condition with up...

  20. Simulation of power maneuvering experiment of MASLWR test facility by MARS-KS code

    Energy Technology Data Exchange (ETDEWEB)

    Park, Ju Yeop [Korea Institute of Nuclear Safety, Daejeon (Korea, Republic of)

    2013-10-15

    In the present study, KINS simulation result by the MARS-KS code (KS-002 version) for the SP-3 experiment is presented in detail and conclusion on MARS-KS code performance drawn through this simulation is described. Performance of the MARS-KS code is evaluated through the simulation of the power maneuvering experiment of the MASLWR test facility. Steady run shows the helical coil specific heat transfer model of the code is reasonable. However, identified discrepancy of the primary mass flowrate at transient run shows code performance for pressure drop needs to be improved considering sensitivity of the flowrate to the pressure drop at natural circulation. Since 2009, IAEA has conducted a research program entitled as ICSP (International Collaborative Standard Problem) on integral PWR design to evaluate current the state of the art of thermal-hydraulic code in simulating natural circulation flow within integral type reactor. In this ICSP, experimental data obtained from MASLWR (Multi-Application Small Light Water Reactor) test facility located at Oregon state university in the US have been simulated by various thermal-hydraulic codes of each participant of the ICSP and compared among others. MASLWR test facility is a mock-up of a passive integral type reactor equipped with helical coil steam generator. Since SMART reactor which is currently being developed in Korea also adopts a helical coil steam generator, Korea Institute of Nuclear Safety (KINS) has joined this ICSP to assess the applicability of a domestic regulatory audit thermal-hydraulic code (i. e. MARS-KS code) for the SMART reactor including wall-to-fluid heat transfer model modification based on independent international experiment data. In the ICSP, two types of transient experiments have been focused and they are loss of feedwater transient with subsequent ADS operation and long term cooling (SP-2) and normal operating conditions at different power levels (SP-3)

  1. Simulation of power maneuvering experiment of MASLWR test facility by MARS-KS code

    Energy Technology Data Exchange (ETDEWEB)

    Park, Ju Yeop [Korea Institute of Nuclear Safety, Daejeon (Korea, Republic of)

    2013-10-15

    In this ICSP, experimental data obtained from MASLWR (Mulit-Application Small Light Water Reactor) test facility located at Oregon state university in the US have been simulated by various thermal-hydraulic codes of each participant of the ICSP and compared among others. MASLWR test facility is a mock-up of a passive integral type reactor equipped with helical coil steam generator. Since SMART reactor which is currently being developed in Korea also adopts a helical coil steam generator, Korea Institute of Nuclear Safety (KINS) has joined this ICSP to assess the applicability of a domestic regulatory audit thermal-hydraulic code (i. e. MARS-KS code) for the SMART reactor including wall-to-fluid heat transfer model modification based on independent international experiment data. In the ICSP, two types of transient experiments have been focused and they are 1) loss of feedwater transient with subsequent ADS operation and long term cooling (SP-2) and normal operating conditions at different power levels. In the present study, KINS simulation result by the MARS-KS code (KS-002 version) for the SP-3 experiment is presented in detail and conclusion on MARS-KS code performance drawn through this simulation is described. Performance of the MARS-KS code is evaluated through the simulation of the power maneuvering experiment of the MASLWR test facility. Steady run shows the helical coil specific heat transfer model of the code is reasonable. However, identified discrepancy of the primary mass flowrate at transient run shows code performance for pressure drop needs to be improved considering sensitivity of the flowrate to the pressure drop at natural circulation.

  2. Detection of secondary eclipses of WASP-10b and Qatar-1b in the Ks band and the correlation between Ks-band temperature and stellar activity.

    Science.gov (United States)

    Cruz, Patricia; Barrado, David; Lillo-Box, Jorge; Diaz, Marcos; López-Morales, Mercedes; Birkby, Jayne; Fortney, Jonathan J.; Hodgkin, Simon

    2017-10-01

    The Calar Alto Secondary Eclipse study was a program dedicated to observe secondary eclipses in the near-IR of two known close-orbiting exoplanets around K-dwarfs: WASP-10b and Qatar-1b. Such observations reveal hints on the orbital configuration of the system and on the thermal emission of the exoplanet, which allows the study of the brightness temperature of its atmosphere. The observations were performed at the Calar Alto Observatory (Spain). We used the OMEGA2000 instrument (Ks band) at the 3.5m telescope. The data was acquired with the telescope strongly defocused. The differential light curve was corrected from systematic effects using the Principal Component Analysis (PCA) technique. The final light curve was fitted using an occultation model to find the eclipse depth and a possible phase shift by performing a MCMC analysis. The observations have revealed a secondary eclipse of WASP-10b with depth of 0.137%, and a depth of 0.196% for Qatar-1b. The observed phase offset from expected mid-eclipse was of -0.0028 for WASP-10b, and of -0.0079 for Qatar-1b. These measured offsets led to a value for |ecosω| of 0.0044 for the WASP-10b system, leading to a derived eccentricity which was too small to be of any significance. For Qatar-1b, we have derived a |ecosω| of 0.0123, however, this last result needs to be confirmed with more data. The estimated Ks-band brightness temperatures are of 1647 K and 1885 K for WASP-10b and Qatar-1b, respectively. We also found an empirical correlation between the (R'HK) activity index of planet hosts and the Ks-band brightness temperature of exoplanets, considering a small number of systems.

  3. Beyond Thiamine: Treatment for Cognitive Impairment in Korsakoff's Syndrome.

    Science.gov (United States)

    Johnson, Justin M; Fox, Valerie

    2018-03-27

    Wernicke's encephalopathy is a condition whose treatment many consultation-liaison psychiatrists know quite well. Less clear, however, is the treatment of its dementia disorder descendent, the Korsakoff's syndrome (KS). This article seeks to review treatment options and provide recommendations for consultation-liaison psychiatrists treating cognitive impairment in KS. In this nonsystematic review, we reviewed PubMed, CINAHL Plus, and Google Scholar for published reports and studies regarding treatment of KS. The literature revealed case reports and placebo-controlled trials of various medications for treatment of KS, though the samples sizes were small and were mostly case reports. There is more attention devoted toward medications used in other dementia disorders, such as donepezil and memantine. The literature revealed more studies around behavioral interventions recommended for treatment of memory impairment in KS and they focused on cognitive remediation and environmental adaptation, such as the use of PDAs or alarms. There is no single, well-studied intervention proven effective as a primary treatment for cognitive impairment in KS. An approach of using environmental modifications in a well-structured living environment, combined with various cognitive interventions, such as pictorial associations, and perhaps a trial of donepezil or memantine, likely represents the best strategy for treating long-term cognitive impairment in KS. Published by Elsevier Inc.

  4. PÖFFi võit läks Türki, "Sügisballil" läks hästi / Tiit Tuumalu

    Index Scriptorium Estoniae

    Tuumalu, Tiit, 1971-

    2007-01-01

    Lõppes 11. Pimedate Ööde Filmifestival. EurAsia programmi grand prix läks Türgi filmile "Takva", parim režissöör on Aleksandr Sokurov ("Aleksandra"), parima naisnäitleja auhinna sai Maarja Jakobson ("Sügisball"), parima meesnäitleja auhinna sai Zdenek Sverak ("Tühjad pudelid"). Parim Eesti film on "Sügisball", mis üldse sai festivalil kuus auhinda. Lisaks nimekiri "Pimedate Ööde filmifestival jagas auhindu" ja lühiintervjuu "3 küsimust" Veiko Õunpuult

  5. Structural Pituitary Abnormalities Associated With CHARGE Syndrome

    Science.gov (United States)

    Gregory, Louise C.; Gevers, Evelien F.; Baker, Joanne; Kasia, Tessa; Chong, Kling; Josifova, Dragana J.; Caimari, Maria; Bilan, Frederic; McCabe, Mark J.

    2013-01-01

    Introduction: CHARGE syndrome is a multisystem disorder that, in addition to Kallmann syndrome/isolated hypogonadotrophic hypogonadism, has been associated with anterior pituitary hypoplasia (APH). However, structural abnormalities such as an ectopic posterior pituitary (EPP) have not yet been described in such patients. Objective: The aims of the study were: 1) to describe the association between CHARGE syndrome and a structurally abnormal pituitary gland; and 2) to investigate whether CHD7 variants, which are identified in 65% of CHARGE patients, are common in septo-optic dysplasia /hypopituitarism. Methods: We describe 2 patients with features of CHARGE and EPP. CHD7 was sequenced in these and other patients with septo-optic dysplasia/hypopituitarism. Results: EPP, APH, and GH, TSH, and probable LH/FSH deficiency were present in 1 patient, and EPP and APH with GH, TSH, LH/FSH, and ACTH deficiency were present in another patient, both of whom had features of CHARGE syndrome. Both had variations in CHD7 that were novel and undetected in control cohorts or in the international database of CHARGE patients, but were also present in their unaffected mothers. No CHD7 variants were detected in the patients with septo-optic dysplasia/hypopituitarism without additional CHARGE features. Conclusion: We report a novel association between CHARGE syndrome and structural abnormalities of the pituitary gland in 2 patients with variations in CHD7 that are of unknown significance. However, CHD7 mutations are an uncommon cause of septo-optic dysplasia or hypopituitarism. Our data suggest the need for evaluation of pituitary function/anatomy in patients with CHARGE syndrome. PMID:23526466

  6. Preliminary Analysis for K-DEMO Water Cooled Breeding Blanket Using MARS-KS

    International Nuclear Information System (INIS)

    Lee, Jeong-Hun; Kim, Geon-Woo; Park, Goon-Cherl; Cho, Hyoung-Kyu; Im, Kihak

    2014-01-01

    In the present study, thermal-hydraulic analyses for the blanket concept are being conducted using the Multidimensional Analysis of Reactor Safety (MARSKS) code, which has been used for the safety analysis of a pressurized water reactor. The purposes of the analyses are to verify the applicability of the code for the proposed blanket system, to investigate the departure of nucleate boiling (DNB) occurrence during the normal and transient conditions, and to extend the capability of MARS-KS to the entire blanket system which includes a few hundreds of single blanket modules. In this paper, the thermal analysis results of the proposed blanket design using the MARS-KS code are presented for the normal operation and an accident condition of a reduced coolant flow rate. Afterwards, the plan for the whole blanket system analysis using MARSKS is introduced and the result of the first trial for the multiple blanket module analysis is summarized. In the present study, thermal-hydraulic analyses for the blanket concept were conducted using the MARS-KS code for a single blanket module. By comparing the MARS calculation results with the CFD analysis results, it was found that MARS-KS can be applied for the blanket thermal analysis with less number of computational meshes. Moreover, due to its capability on the two-phase flow analysis, it can be used for the transient or accident simulation where a phase change may be resulted in. In the future, the MARS-KS code will be applied for the anticipated transient and design based accident analyses. The investigation of the DNB occurrence during the normal and transient conditions will be of special interest of the analysis using it. After that, a methodology to simulate the entire blanket system was proposed by using the DLL version of MARS-KS. A supervisor program, which controls the multiple DLL files, was developed for the common header modelling. The program explicitly determines the flow rates of each module which can equalize

  7. Assessment of the MARS-KS Code Using Atlas 6-inch cold leg Break Test

    Energy Technology Data Exchange (ETDEWEB)

    Kang, D. G.; Kim, J. S.; Ahn, S. H.; Seul, K. W. [Korea Institute of Nuclear Safety, Daejeon (Korea, Republic of)

    2012-03-15

    An integral effect test on the SBLOCA (Small-Break Loss of Coolant Accident) aiming at 6-inch cold leg bottom break, SB-CL-09, was conducted with the Atlas on November, 13, 2009, by KAERI. In this study, the calculations using MARS-KS Vt1.2 code were conducted for 6-inch cold leg break test of Atlas (SB-CL-09) which is the second domestic standard problem (Dsp-02) to assess MARS-KS code capability to simulate the transient thermal-hydraulic behavior for SBLOCA. The steady state was determined by conducting a null transient calculation and the errors between the calculated and measured values are acceptable for almost primary/secondary system parameters. The predicted pressurizer pressure agrees relatively well with the experimental data and the predicted break flow and mass are in good agreement with experiment. In MARS-KS calculation, the decrease of core collapsed water level is predicted well in blowdown phase, but just before LSC, water level is higher than experiment. However, the sudden decrease and increase of water level is higher than experiment. However, the sudden decrease and increase of water level at the LSC are predicted qualitatively. After LSC, there is another water level dip at Sit injection time which is not in experiment. It is considered that this phenomenon is caused by rapid depressurization of downcomer due to significant condensation rate of vapor in downcomer when Sit water flows in it. For the downcomer water level is predicted well, however, it is significantly over-predicted at SIT injection time, water level is predicted well, however, it is significantly over-predicted at SIT injection time after SIT water flows in downcomer. Predicted cladding temperature generally agrees well with the experiment, while there is peak at SIT injection time in calculation which is not in experiment. The loop seals of 1A, 2B intermediate leg are cleared around 400 seconds in experiment, while only that of 1A is cleared in MARS-KS calculation at the

  8. KS R41B. A high performance steel-aluminium composite material; KS R41B. Ein Stahl-Aluminium-Verbundwerkstoff fuer hohe Belastungen

    Energy Technology Data Exchange (ETDEWEB)

    Deicke, K. [KS Gleitlager GmbH (Germany). Bereich Metall; Matucha, H.; Schubert, W. [KS Gleitlager GmbH, St. Leon-Rot (Germany); Steffens, T. [KS Gleitlager GmbH, Neckarsulm (Germany)

    2002-08-01

    Aluminium-tin alloys have been well-known for a long time and have proved to be suitable bearing materials for crankshaft bearings for many years. The known alloy AlZn4,5SiCuPb is one of the aluminium materials capable of sustaining the highest mechanical loads. In order to achieve optimum sliding properties, it would be necessary to increase the share of the soft lead phase in the alloy. This article by KS Gleitlager GmbH shows a reasonably priced manufacturing technology for high-performance aluminium-zinc-silicon-copper alloys. (orig.) [German] Die Aluminium-Zinn-Lagerlegierungen sind seit langem bekannt und haben sich seit Jahren als Lagerwerkstoff fuer Kurbelwellenlager bewaehrt. Die Legierung AlZn4,5SiCuPb gehoert zu den mechanisch am hoechsten belastbaren Aluminium-Werkstoffen. Um optimale Gleiteigenschaften zu erzielen, muesste in der Legierung der Anteil des weichen Bleis noch weiter erhoeht werden. Dieser Beitrag der KS Gleitlager GmbH zeigt eine kostenguenstige Herstelltechnologie fuer hochbelastbare Aluminium-Zink-Silizium-Kupfer-Legierungen. (orig.)

  9. Thermal-hydraulic analysis of water cooled breeding blanket of K-DEMO using MARS-KS code

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jeong-Hun; Park, Il Woong; Kim, Geon-Woo; Park, Goon-Cherl [Seoul National University, Seoul (Korea, Republic of); Cho, Hyoung-Kyu, E-mail: chohk@snu.ac.kr [Seoul National University, Seoul (Korea, Republic of); Im, Kihak [National Fusion Research Institute, Daejeon (Korea, Republic of)

    2015-10-15

    Highlights: • The thermal design of breeding blanket for the K-DEMO is evaluated using MARS-KS. • To confirm the prediction capability of MARS, the results were compared with the CFD. • The results of MARS-KS calculation and CFD prediction are in good agreement. • A transient simulation was carried out so as to show the applicability of MARS-KS. • A methodology to simulate the entire blanket system is proposed. - Abstract: The thermal design of a breeding blanket for the Korean Fusion DEMOnstration reactor (K-DEMO) is evaluated using the Multidimensional Analysis of Reactor Safety (MARS-KS) code in this study. The MARS-KS code has advantages in simulating transient two-phase flow over computational fluid dynamics (CFD) codes. In order to confirm the prediction capability of the code for the present blanket system, the calculation results were compared with the CFD prediction. The results of MARS-KS calculation and CFD prediction are in good agreement. Afterwards, a transient simulation for a conceptual problem was carried out so as to show the applicability of MARS-KS for a transient or accident condition. Finally, a methodology to simulate the multiple blanket modules is proposed.

  10. Inclusive production of charged hadrons and $K_{s}^{0}$ mesons in photon-photon collisions

    CERN Document Server

    Ackerstaff, K.; Allison, John; Altekamp, N.; Anderson, K.J.; Anderson, S.; Arcelli, S.; Asai, S.; Ashby, S.F.; Axen, D.; Azuelos, G.; Ball, A.H.; Barberio, E.; Barlow, Roger J.; Bartoldus, R.; Batley, J.R.; Baumann, S.; Bechtluft, J.; Behnke, T.; Bell, Kenneth Watson; Bella, G.; Bentvelsen, S.; Bethke, S.; Betts, S.; Biebel, O.; Biguzzi, A.; Bird, S.D.; Blobel, V.; Bloodworth, I.J.; Bobinski, M.; Bock, P.; Bohme, J.; Boutemeur, M.; Braibant, S.; Bright-Thomas, P.; Brown, Robert M.; Burckhart, H.J.; Burgard, C.; Burgin, R.; Capiluppi, P.; Carnegie, R.K.; Carter, A.A.; Carter, J.R.; Chang, C.Y.; Charlton, David G.; Chrisman, D.; Ciocca, C.; Clarke, P.E.L.; Clay, E.; Cohen, I.; Conboy, J.E.; Cooke, O.C.; Couyoumtzelis, C.; Coxe, R.L.; Cuffiani, M.; Dado, S.; Dallavalle, G.Marco; Davis, R.; De Jong, S.; del Pozo, L.A.; De Roeck, A.; Desch, K.; Dienes, B.; Dixit, M.S.; Doucet, M.; Dubbert, J.; Duchovni, E.; Duckeck, G.; Duerdoth, I.P.; Eatough, D.; Estabrooks, P.G.; Etzion, E.; Evans, H.G.; Fabbri, F.; Fanfani, A.; Fanti, M.; Faust, A.A.; Fiedler, F.; Fierro, M.; Fischer, H.M.; Fleck, I.; Folman, R.; Furtjes, A.; Futyan, D.I.; Gagnon, P.; Gary, J.W.; Gascon, J.; Gascon-Shotkin, S.M.; Geich-Gimbel, C.; Geralis, T.; Giacomelli, G.; Giacomelli, P.; Gibson, V.; Gibson, W.R.; Gingrich, D.M.; Glenzinski, D.; Goldberg, J.; Gorn, W.; Grandi, C.; Gross, E.; Grunhaus, J.; Gruwe, M.; Hanson, G.G.; Hansroul, M.; Hapke, M.; Hargrove, C.K.; Hartmann, C.; Hauschild, M.; Hawkes, C.M.; Hawkings, R.; Hemingway, R.J.; Herndon, M.; Herten, G.; Heuer, R.D.; Hildreth, M.D.; Hill, J.C.; Hillier, S.J.; Hobson, P.R.; Hocker, James Andrew; Homer, R.J.; Honma, A.K.; Horvath, D.; Hossain, K.R.; Howard, R.; Huntemeyer, P.; Igo-Kemenes, P.; Imrie, D.C.; Ishii, K.; Jacob, F.R.; Jawahery, A.; Jeremie, H.; Jimack, M.; Joly, A.; Jones, C.R.; Jovanovic, P.; Junk, T.R.; Karlen, D.; Kartvelishvili, V.; Kawagoe, K.; Kawamoto, T.; Kayal, P.I.; Keeler, R.K.; Kellogg, R.G.; Kennedy, B.W.; Klier, A.; Kluth, S.; Kobayashi, T.; Kobel, M.; Koetke, D.S.; Kokott, T.P.; Kolrep, M.; Komamiya, S.; Kowalewski, Robert V.; Kress, T.; Krieger, P.; von Krogh, J.; Kyberd, P.; Lafferty, G.D.; Lanske, D.; Lauber, J.; Lautenschlager, S.R.; Lawson, I.; Layter, J.G.; Lazic, D.; Lee, A.M.; Lefebvre, E.; Lellouch, D.; Letts, J.; Levinson, L.; Liebisch, R.; List, B.; Littlewood, C.; Lloyd, A.W.; Lloyd, S.L.; Loebinger, F.K.; Long, G.D.; Losty, M.J.; Ludwig, J.; Lui, D.; Macchiolo, A.; Macpherson, A.; Mannelli, M.; Marcellini, S.; Markopoulos, C.; Martin, A.J.; Martin, J.P.; Martinez, G.; Mashimo, T.; Mattig, Peter; McDonald, W.John; McKenna, J.; Mckigney, E.A.; McMahon, T.J.; McPherson, R.A.; Meijers, F.; Menke, S.; Merritt, F.S.; Mes, H.; Meyer, J.; Michelini, A.; Mihara, S.; Mikenberg, G.; Miller, D.J.; Mir, R.; Mohr, W.; Montanari, A.; Mori, T.; Nagai, K.; Nakamura, I.; Neal, H.A.; Nellen, B.; Nisius, R.; O'Neale, S.W.; Oakham, F.G.; Odorici, F.; Ogren, H.O.; Oreglia, M.J.; Orito, S.; Palinkas, J.; Pasztor, G.; Pater, J.R.; Patrick, G.N.; Patt, J.; Perez-Ochoa, R.; Petzold, S.; Pfeifenschneider, P.; Pilcher, J.E.; Pinfold, J.; Plane, David E.; Poffenberger, P.; Poli, B.; Polok, J.; Przybycien, M.; Rembser, C.; Rick, H.; Robertson, S.; Robins, S.A.; Rodning, N.; Roney, J.M.; Roscoe, K.; Rossi, A.M.; Rozen, Y.; Runge, K.; Runolfsson, O.; Rust, D.R.; Sachs, K.; Saeki, T.; Sahr, O.; Sang, W.M.; Sarkisian, E.K.G.; Sbarra, C.; Schaile, A.D.; Schaile, O.; Scharf, F.; Scharff-Hansen, P.; Schieck, J.; Schmitt, B.; Schmitt, S.; Schoning, A.; Schorner, T.; Schroder, Matthias; Schumacher, M.; Schwick, C.; Scott, W.G.; Seuster, R.; Shears, T.G.; Shen, B.C.; Shepherd-Themistocleous, C.H.; Sherwood, P.; Siroli, G.P.; Sittler, A.; Skuja, A.; Smith, A.M.; Snow, G.A.; Sobie, R.; Soldner-Rembold, S.; Sproston, M.; Stahl, A.; Stephens, K.; Steuerer, J.; Stoll, K.; Strom, David M.; Strohmer, R.; Tafirout, R.; Talbot, S.D.; Tanaka, S.; Taras, P.; Tarem, S.; Teuscher, R.; Thiergen, M.; Thomson, M.A.; von Torne, E.; Torrence, E.; Towers, S.; Trigger, I.; Trocsanyi, Z.; Tsur, E.; Turcot, A.S.; Turner-Watson, M.F.; Van Kooten, Rick J.; Vannerem, P.; Verzocchi, M.; Vikas, P.; Voss, H.; Wackerle, F.; Wagner, A.; Ward, C.P.; Ward, D.R.; Watkins, P.M.; Watson, A.T.; Watson, N.K.; Wells, P.S.; Wermes, N.; White, J.S.; Wiesler, T.; Wilson, G.W.; Wilson, J.A.; Wyatt, T.R.; Yamashita, S.; Yekutieli, G.; Zacek, V.; Zer-Zion, D.

    1999-01-01

    The production of charged hadrons and K_s mesons in the collisions of quasi-real photons has been measured using the OPAL detector at LEP. The data were taken at e+e- centre-of-mass energies of 161 and 172 GeV. The differential cross-sections as a function of the transverse momentum and the pseudorapidity of the charged hadrons and K_s mesons have been compared to the leading order Monte Carlo simulations of PHOJET and PYTHIA and to perturbative next-to-leading order (NLO) QCD calculations. The distributions have been measured in the range 10-125 GeV of the hadronic invariant mass W. By comparing the transverse momentum distribution of charged hadrons measured in gamma-gamma interactions with gamma-proton and meson-proton data we find evidence for hard photon interactions in addition to the purely hadronic photon interactions.

  11. MARS-KS code validation activity through the atlas domestic standard problem

    International Nuclear Information System (INIS)

    Choi, K. Y.; Kim, Y. S.; Kang, K. H.; Park, H. S.; Cho, S.

    2012-01-01

    The 2 nd Domestic Standard Problem (DSP-02) exercise using the ATLAS integral effect test data was executed to transfer the integral effect test data to domestic nuclear industries and to contribute to improving the safety analysis methodology for PWRs. A small break loss of coolant accident of a 6-inch break at the cold leg was determined as a target scenario by considering its technical importance and by incorporating interests from participants. Ten calculation results using MARS-KS code were collected, major prediction results were described qualitatively and code prediction accuracy was assessed quantitatively using the FFTBM. In addition, special code assessment activities were carried out to find out the area where the model improvement is required in the MARS-KS code. The lessons from this DSP-02 and recommendations to code developers are described in this paper. (authors)

  12. MoKS teistes koostööprojektides / John Grzinich

    Index Scriptorium Estoniae

    Grzinich, John

    2005-01-01

    13. III Genfis toimunud helikunstiüritusest "VernalFLUX / Kevadvool", millest Eestist võtsid osa MoKS'i koordinaatorid John Grzinich ja Evelyn Müürsepp, nende performance'ist "Maaheli. Unustatud unistused". Uuest organisatsioonist Claudmirror, mis on välja andnud seitse CD-ROM-i, nende seas John Grzinichi CD-ROM-i "Võrdsed ja kauged jooned"

  13. Üks uks Eesti ja Jaapani vahel : köitekunst kohtub animatsiooniga / Maarja Undusk

    Index Scriptorium Estoniae

    Undusk, Maarja

    2003-01-01

    Eestis viibiv jaapanlanna Maya Yonesho (sünd. 1965) hakkab tegema stipendiaadifilmi "Üks uks". Raamat-uksed teevad valmis eesti nahakunstnikud Külli Grünbach, Rene Haljasmäe, Pille Kivihall, Helen Maran-Poll, Lennart Mänd, Tiina Piisang, Jane Rannamets ja Maarja Undusk. Filmi muusika autor Sven Grünberg. M. Yonesho juhendas nahakunstnik Kadri Halliku diplomitööd

  14. The role of genes, intelligence, personality, and social engagement in cognitive performance in Klinefelter syndrome.

    Science.gov (United States)

    Skakkebæk, Anne; Moore, Philip J; Pedersen, Anders Degn; Bojesen, Anders; Kristensen, Maria Krarup; Fedder, Jens; Laurberg, Peter; Hertz, Jens Michael; Østergaard, John Rosendahl; Wallentin, Mikkel; Gravholt, Claus Højbjerg

    2017-03-01

    The determinants of cognitive deficits among individuals with Klinefelter syndrome (KS) are not well understood. This study was conducted to assess the impact of general intelligence, personality, and social engagement on cognitive performance among patients with KS and a group of controls matched for age and years of education. Sixty-nine patients with KS and 69 controls were assessed in terms of IQ, NEO personality inventory, the Autism Spectrum Quotient (AQ) scale, and measures of cognitive performance reflecting working memory and executive function. Patients with KS performed more poorly on memory and executive-function tasks. Patients with KS also exhibited greater neuroticism and less extraversion, openness, and conscientiousness than controls. Memory deficits among patients with KS were associated with lower intelligence, while diminished executive functioning was mediated by both lower intelligence and less social engagement. Our results suggest that among patients with KS, memory deficits are principally a function of lower general intelligence, while executive-function deficits are associated with both lower intelligence and poorer social skills. This suggests a potential influence of social engagement on executive cognitive functioning (and/or vice-versa) among individuals with KS, and perhaps those with other genetic disorders. Future longitudinal research would be important to further clarify this and other issues discussed in this research.

  15. Source Memory in Korsakoff Syndrome: Disentangling the Mechanisms of Temporal Confusion.

    Science.gov (United States)

    Brion, Mélanie; de Timary, Philippe; Pitel, Anne-Lise; Maurage, Pierre

    2017-03-01

    Korsakoff syndrome (KS), most frequently resulting from alcohol dependence (ALC), is characterized by severe anterograde amnesia. It has been suggested that these deficits may extend to other memory components, and notably source memory deficits involved in the disorientation and temporal confusion frequently observed in KS. However, the extent of this source memory impairment in KS and its usefulness for the differential diagnosis between ALC and KS remain unexplored. Nineteen patients with KS were compared with 19 alcohol-dependent individuals and 19 controls in a source memory test exploring temporal context confusions ("continuous recognition task"). Episodic memory and psychopathological comorbidities were controlled for. While no source memory deficit was observed in ALC, KS was associated with a significant presence of temporal context confusion, even when the influence of comorbidities was taken into account. This source memory impairment did not appear to be related to performances on episodic memory or executive functions. Patients with KS displayed source memory deficits, as indexed by temporal context confusions. The absence of a relationship with episodic memory performances seems to indicate that source memory impairment is not a mere by-product of amnesia. As ALC was associated with preserved source memory, the presence of temporal context confusion may serve as a complementary tool for the differential diagnosis between ALC and KS. Copyright © 2017 by the Research Society on Alcoholism.

  16. Measurement of the $B_s^0\\to J/\\psi K_S^0$ branching fraction

    CERN Document Server

    Aaij, R; Adeva, B; Adinolfi, M; Adrover, C; Affolder, A; Ajaltouni, Z; Albrecht, J; Alessio, F; Alexander, M; Ali, S; Alkhazov, G; Alvarez Cartelle, P; Alves Jr, A A; Amato, S; Amhis, Y; Anderson, J; Appleby, R B; Aquines Gutierrez, O; Archilli, F; Arrabito, L; Artamonov, A; Artuso, M; Aslanides, E; Auriemma, G; Bachmann, S; Back, J J; Balagura, V; Baldini, W; Barlow, R J; Barschel, C; Barsuk, S; Barter, W; Bates, A; Bauer, C; Bauer, Th; Bay, A; Bediaga, I; Belogurov, S; Belous, K; Belyaev, I; Ben-Haim, E; Benayoun, M; Bencivenni, G; Benson, S; Benton, J; Bernet, R; Bettler, M -O; van Beuzekom, M; Bien, A; Bifani, S; Bird, T; Bizzeti, A; Bjørnstad, P M; Blake, T; Blanc, F; Blanks, C; Blouw, J; Blusk, S; Bobrov, A; Bocci, V; Bondar, A; Bondar, N; Bonivento, W; Borghi, S; Borgia, A; Bowcock, T J V; Bozzi, C; Brambach, T; van den Brand, J; Bressieux, J; Brett, D; Britsch, M; Britton, T; Brook, N H; Brown, H; Büchler-Germann, A; Burducea, I; Bursche, A; Buytaert, J; Cadeddu, S; Callot, O; Calvi, M; Calvo Gomez, M; Camboni, A; Campana, P; Carbone, A; Carboni, G; Cardinale, R; Cardini, A; Carson, L; Carvalho Akiba, K; Casse, G; Cattaneo, M; Cauet, Ch; Charles, M; Charpentier, Ph; Chiapolini, N; Ciba, K; Cid Vidal, X; Ciezarek, G; Clarke, P E L; Clemencic, M; Cliff, H V; Closier, J; Coca, C; Coco, V; Cogan, J; Collins, P; Comerma-Montells, A; Contu, A; Cook, A; Coombes, M; Corti, G; Couturier, B; Cowan, G A; Currie, R; D'Ambrosio, C; David, P; David, P N Y; De Bonis, I; De Bruyn, K; De Capua, S; De Cian, M; De Lorenzi, F; De Miranda, J M; De Paula, L; De Simone, P; Decamp, D; Deckenhoff, M; Degaudenzi, H; Del Buono, L; Deplano, C; Derkach, D; Deschamps, O; Dettori, F; Dickens, J; Dijkstra, H; Diniz Batista, P; Domingo Bonal, F; Donleavy, S; Dordei, F; Dosil Suárez, A; Dossett, D; Dovbnya, A; Dupertuis, F; Dzhelyadin, R; Dziurda, A; Easo, S; Egede, U; Egorychev, V; Eidelman, S; van Eijk, D; Eisele, F; Eisenhardt, S; Ekelhof, R; Eklund, L; Elsasser, Ch; Elsby, D; Esperante Pereira, D; Falabella, A; Färber, C; Fardell, G; Farinelli, C; Farry, S; Fave, V; Fernandez Albor, V; Ferro-Luzzi, M; Filippov, S; Fitzpatrick, C; Fontana, M; Fontanelli, F; Forty, R; Francisco, O; Frank, M; Frei, C; Frosini, M; Furcas, S; Gallas Torreira, A; Galli, D; Gandelman, M; Gandini, P; Gao, Y; Garnier, J-C; Garofoli, J; Garra Tico, J; Garrido, L; Gascon, D; Gaspar, C; Gauld, R; Gauvin, N; Gersabeck, M; Gershon, T; Ghez, Ph; Gibson, V; Gligorov, V V; Göbel, C; Golubkov, D; Golutvin, A; Gomes, A; Gordon, H; Grabalosa Gándara, M; Graciani Diaz, R; Granado Cardoso, L A; Graugés, E; Graziani, G; Grecu, A; Greening, E; Gregson, S; Gui, B; Gushchin, E; Guz, Yu; Gys, T; Hadjivasiliou, C; Haefeli, G; Haen, C; Haines, S C; Hampson, T; Hansmann-Menzemer, S; Harji, R; Harnew, N; Harrison, J; Harrison, P F; Hartmann, T; He, J; Heijne, V; Hennessy, K; Henrard, P; Hernando Morata, J A; van Herwijnen, E; Hicks, E; Holubyev, K; Hopchev, P; Hulsbergen, W; Hunt, P; Huse, T; Huston, R S; Hutchcroft, D; Hynds, D; Iakovenko, V; Ilten, P; Imong, J; Jacobsson, R; Jaeger, A; Jahjah Hussein, M; Jans, E; Jansen, F; Jaton, P; Jean-Marie, B; Jing, F; John, M; Johnson, D; Jones, C R; Jost, B; Kaballo, M; Kandybei, S; Karacson, M; Karbach, T M; Keaveney, J; Kenyon, I R; Kerzel, U; Ketel, T; Keune, A; Khanji, B; Kim, Y M; Knecht, M; Koopman, R F; Koppenburg, P; Korolev, M; Kozlinskiy, A; Kravchuk, L; Kreplin, K; Kreps, M; Krocker, G; Krokovny, P; Kruse, F; Kruzelecki, K; Kucharczyk, M; Kudryavtsev, V; Kvaratskheliya, T; La Thi, V N; Lacarrere, D; Lafferty, G; Lai, A; Lambert, D; Lambert, R W; Lanciotti, E; Lanfranchi, G; Langenbruch, C; Latham, T; Lazzeroni, C; Le Gac, R; van Leerdam, J; Lees, J -P; Lefèvre, R; Leflat, A; Lefrançois, J; Leroy, O; Lesiak, T; Li, L; Li Gioi, L; Lieng, M; Liles, M; Lindner, R; Linn, C; Liu, B; Liu, G; von Loeben, J; Lopes, J H; Lopez Asamar, E; Lopez-March, N; Lu, H; Luisier, J; Mac Raighne, A; Machefert, F; Machikhiliyan, I V; Maciuc, F; Maev, O; Magnin, J; Malde, S; Mamunur, R M D; Manca, G; Mancinelli, G; Mangiafave, N; Marconi, U; Märki, R; Marks, J; Martellotti, G; Martens, A; Martin, L; Martín Sánchez, A; Martinelli, M; Martinez Santos, D; Massafferri, A; Mathe, Z; Matteuzzi, C; Matveev, M; Maurice, E; Maynard, B; Mazurov, A; McGregor, G; McNulty, R; Meissner, M; Merk, M; Merkel, J; Miglioranzi, S; Milanes, D A; Minard, M -N; Molina Rodriguez, J; Monteil, S; Moran, D; Morawski, P; Mountain, R; Mous, I; Muheim, F; Müller, K; Muresan, R; Muryn, B; Muster, B; Mylroie-Smith, J; Naik, P; Nakada, T; Nandakumar, R; Nasteva, I; Needham, M; Neufeld, N; Nguyen, A D; Nguyen-Mau, C; Nicol, M; Niess, V; Nikitin, N; Nikodem, T; Nomerotski, A; Novoselov, A; Oblakowska-Mucha, A; Obraztsov, V; Oggero, S; Ogilvy, S; Okhrimenko, O; Oldeman, R; Orlandea, M; Otalora Goicochea, J M; Owen, P; Pal, B K; Palacios, J; Palano, A; Palutan, M; Panman, J; Papanestis, A; Pappagallo, M; Parkes, C; Parkinson, C J; Passaleva, G; Patel, G D; Patel, M; Paterson, S K; Patrick, G N; Patrignani, C; Pavel-Nicorescu, C; Pazos Alvarez, A; Pellegrino, A; Penso, G; Pepe Altarelli, M; Perazzini, S; Perego, D L; Perez Trigo, E; Pérez-Calero Yzquierdo, A; Perret, P; Perrin-Terrin, M; Pessina, G; Petrolini, A; Phan, A; Picatoste Olloqui, E; Pie Valls, B; Pietrzyk, B; Pilař, T; Pinci, D; Plackett, R; Playfer, S; Plo Casasus, M; Polok, G; Poluektov, A; Polycarpo, E; Popov, D; Popovici, B; Potterat, C; Powell, A; Prisciandaro, J; Pugatch, V; Puig Navarro, A; Qian, W; Rademacker, J H; Rakotomiaramanana, B; Rangel, M S; Raniuk, I; Raven, G; Redford, S; Reid, M M; dos Reis, A C; Ricciardi, S; Richards, A; Rinnert, K; Roa Romero, D A; Robbe, P; Rodrigues, E; Rodrigues, F; Rodriguez Perez, P; Rogers, G J; Roiser, S; Romanovsky, V; Rosello, M; Rouvinet, J; Ruf, T; Ruiz, H; Sabatino, G; Saborido Silva, J J; Sagidova, N; Sail, P; Saitta, B; Salzmann, C; Sannino, M; Santacesaria, R; Santamarina Rios, C; Santinelli, R; Santovetti, E; Sapunov, M; Sarti, A; Satriano, C; Satta, A; Savrie, M; Savrina, D; Schaack, P; Schiller, M; Schleich, S; Schlupp, M; Schmelling, M; Schmidt, B; Schneider, O; Schopper, A; Schune, M -H; Schwemmer, R; Sciascia, B; Sciubba, A; Seco, M; Semennikov, A; Senderowska, K; Sepp, I; Serra, N; Serrano, J; Seyfert, P; Shapkin, M; Shapoval, I; Shatalov, P; Shcheglov, Y; Shears, T; Shekhtman, L; Shevchenko, O; Shevchenko, V; Shires, A; Silva Coutinho, R; Skwarnicki, T; Smith, N A; Smith, E; Sobczak, K; Soler, F J P; Solomin, A; Soomro, F; Souza De Paula, B; Spaan, B; Sparkes, A; Spradlin, P; Stagni, F; Stahl, S; Steinkamp, O; Stoica, S; Stone, S; Storaci, B; Straticiuc, M; Straumann, U; Subbiah, V K; Swientek, S; Szczekowski, M; Szczypka, P; Szumlak, T; T'Jampens, S; Teodorescu, E; Teubert, F; Thomas, C; Thomas, E; van Tilburg, J; Tisserand, V; Tobin, M; Tolk, S; Topp-Joergensen, S; Torr, N; Tournefier, E; Tourneur, S; Tran, M T; Tsaregorodtsev, A; Tuning, N; Ubeda Garcia, M; Ukleja, A; Urquijo, P; Uwer, U; Vagnoni, V; Valenti, G; Vazquez Gomez, R; Vazquez Regueiro, P; Vecchi, S; Velthuis, J J; Veltri, M; Viaud, B; Videau, I; Vieira, D; Vilasis-Cardona, X; Visniakov, J; Vollhardt, A; Volyanskyy, D; Voong, D; Vorobyev, A; Vorobyev, V; Voss, H; Waldi, R; Wandernoth, S; Wang, J; Ward, D R; Watson, N K; Webber, A D; Websdale, D; Whitehead, M; Wiedner, D; Wiggers, L; Wilkinson, G; Williams, M P; Williams, M; Wilson, F F; Wishahi, J; Witek, M; Witzeling, W; Wotton, S A; Wyllie, K; Xie, Y; Xing, F; Xing, Z; Yang, Z; Young, R; Yushchenko, O; Zangoli, M; Zavertyaev, M; Zhang, F; Zhang, L; Zhang, W C; Zhang, Y; Zhelezov, A; Zhong, L; Zvyagin, A

    2012-01-01

    The $B_s^0\\to J/\\psi K_S^0$ branching fraction is measured in a data sample corresponding to 0.41$fb^{-1}$ of integrated luminosity collected with the LHCb detector at the LHC. This channel is sensitive to the penguin contributions affecting the sin2$\\beta$ measurement from $B^0\\to J/\\psi K_S^0$ The time-integrated branching fraction is measured to be $BF(B_s^0\\to J/\\psi K_S^0)=(1.83\\pm0.28)\\times10^{-5}$. This is the most precise measurement to date.

  17. Radiation effects on radiation-hardened KU and KS-4V optical fibres

    International Nuclear Information System (INIS)

    Ivanov, A.A.; Tugarinov, S.N.; Kaschuck, Y.A.; Krasilnikov, A.V.; Bender, S.E.

    1999-01-01

    The aim of this work was to test the un-pretreated and the hardened (H 2 -loaded and pre-irradiated) KS-4V and KU optical fibres in reactor environment by in-situ measurements of both the radiation-induced loss and the luminescence in the visible spectral region. Both the radio-luminescent and the transmission spectra were in-situ detected during irradiation by charge-coupled-device (CCD) linear detector in the visible spectral region of 400 to 700 nm. The radiation induced loss spectra at the fast neutron fluence of 2*10 6 n/cm 2 shows the hardened, H 2 -loading and pre-irradiating effects in the both KU and KS-4V fibres. KU un-pretreated fibre shows a big radiation absorption band of non-bridging oxygen centered at the wavelength of 630 nm. It appears that the KS-4V hardened fibre has a specific point in the loss spectrum in the vicinity of 460 nm. Other measurements were performed, particularly after reactor shutdown and at 3 different neutron fluences with constant neutron flux after restarting

  18. The role of hypogonadism in Klinefelter syndrome

    DEFF Research Database (Denmark)

    Høst, Christian; Skakkebæk, Anne; Groth, Kristian A

    2014-01-01

    and azoospermia, most men with KS suffer from some degree of learning disability and may have various kinds of psychiatric problems. The effects of long-term hypogonadism may be diffi cult to discern from the gene dose effect of the extra X-chromosome. Whatever the cause, alterations in body composition...... populations show an increased risk of both hospitalization and death from various diseases. Testosterone treatment should be offered to KS patients from early puberty, to secure a proper masculine development, nonetheless the evidence is weak or nonexisting, since no randomized controlled trials have ever...... been published. Here, we will review the current knowledge of hypogonadism in KS and the rationale for testosterone treatment and try to give our best recommendations for surveillance of this rather common, but often ignored, syndrome....

  19. The effects of multiple features of alternatively spliced exons on the KA/KS ratio test

    Directory of Open Access Journals (Sweden)

    Chen Feng-Chi

    2006-05-01

    Full Text Available Abstract Background The evolution of alternatively spliced exons (ASEs is of primary interest because these exons are suggested to be a major source of functional diversity of proteins. Many exon features have been suggested to affect the evolution of ASEs. However, previous studies have relied on the KA/KS ratio test without taking into consideration information sufficiency (i.e., exon length > 75 bp, cross-species divergence > 5% of the studied exons, leading to potentially biased interpretations. Furthermore, which exon feature dominates the results of the KA/KS ratio test and whether multiple exon features have additive effects have remained unexplored. Results In this study, we collect two different datasets for analysis – the ASE dataset (which includes lineage-specific ASEs and conserved ASEs and the ACE dataset (which includes only conserved ASEs. We first show that information sufficiency can significantly affect the interpretation of relationship between exons features and the KA/KS ratio test results. After discarding exons with insufficient information, we use a Boolean method to analyze the relationship between test results and four exon features (namely length, protein domain overlapping, inclusion level, and exonic splicing enhancer (ESE frequency for the ASE dataset. We demonstrate that length and protein domain overlapping are dominant factors, and they have similar impacts on test results of ASEs. In addition, despite the weak impacts of inclusion level and ESE motif frequency when considered individually, combination of these two factors still have minor additive effects on test results. However, the ACE dataset shows a slightly different result in that inclusion level has a marginally significant effect on test results. Lineage-specific ASEs may have contributed to the difference. Overall, in both ASEs and ACEs, protein domain overlapping is the most dominant exon feature while ESE frequency is the weakest one in affecting

  20. A phase 1b study of humanized KS-interleukin-2 (huKS-IL2) immunocytokine with cyclophosphamide in patients with EpCAM-positive advanced solid tumors

    International Nuclear Information System (INIS)

    Connor, Joseph P; Henslee-Downey, Jean; Kramer, Daniel; Neugebauer, Roland; Stupp, Roger; Cristea, Mihaela C; Lewis, Nancy L; Lewis, Lionel D; Komarnitsky, Philip B; Mattiacci, Maria R; Felder, Mildred; Stewart, Sarah; Harter, Josephine

    2013-01-01

    Humanized KS-interleukin-2 (huKS-IL2), an immunocytokine with specificity for epithelial cell adhesion molecule (EpCAM), has demonstrated favorable tolerability and immunologic activity as a single agent. Phase 1b study in patients with EpCAM-positive advanced solid tumors to determine the maximum tolerated dose (MTD) and safety profile of huKS-IL2 in combination with low-dose cyclophosphamide. Treatment consisted of cyclophosphamide (300 mg/m 2 on day 1), and escalating doses of huKS-IL2 (0.5–4.0 mg/m 2 IV continuous infusion over 4 hours) on days 2, 3, and 4 of each 21-day cycle. Safety, pharmacokinetic profile, immunogenicity, anti-tumor and biologic activity were evaluated. Twenty-seven patients were treated for up to 6 cycles; 26 were evaluable for response. The MTD of huKS-IL2 in combination with 300 mg/m 2 cyclophosphamide was 3.0 mg/m 2 . At higher doses, myelosuppression was dose-limiting. Transient lymphopenia was the most common grade 3/4 adverse event (AE). Other significant AEs included hypotension, hypophosphatemia, and increase in serum creatinine. All patients recovered from these AEs. The huKS-IL2 exposure was dose-dependent, but not dose-proportional, accumulation was negligible, and elimination half-life and systemic clearance were independent of dose and time. Most patients had a transient immune response to huKS-IL2. Immunologic activity was observed at all doses. Ten patients (38%) had stable disease as best response, lasting for ≥ 4 cycles in 3 patients. The combination of huKS-IL2 with low-dose cyclophosphamide was well tolerated. Although no objective responses were observed, the combination showed evidence of immunologic activity and 3 patients showed stable disease for ≥ 4 cycles.

  1. Neuropsychology and brain morphology in Klinefelter syndrome - the impact of genetics

    DEFF Research Database (Denmark)

    Jensen, Anne Skakkebæk; Bojesen, A; Kristensen, M. K.

    2014-01-01

    Klinefelter syndrome (KS, 47,XXY) is associated with increased psychiatric morbidity and cognitive disabilities, although the neuropsychological phenotype shows great variability. Androgen receptor polymorphism (CAG repeat length), skewed X-chromosome inactivation and parent-of-origin of the extra......-inactivation, CAG repeat length and parent-of-origin have no impact on the neuropsychological phenotype in KS (http://www.clinicaltrials.gov (Clinical trial NCT00999310))....

  2. Criminality in men with Klinefelter's syndrome and XYY syndrome: a cohort study

    OpenAIRE

    Stochholm, Kirstine; Bojesen, Anders; Jensen, Anne Skakkebæk; Juul, Svend; Gravholt, Claus Højbjerg

    2012-01-01

    Objective To investigate the criminal pattern in men between 15 and 70 years of age diagnosed with 47,XXY (Klinefelter's syndrome (KS)) or 47,XYY compared to the general population. Design Register-based cohort study comparing the incidence of convictions among men with KS and with 47,XYY with age- and calendar-matched samples of the general population. Crime was classified into eight types (sexual abuse, homicide, burglary, violence, traffic, drug-related, arson and ‘others’). Setting Denmar...

  3. Reflection of the Geomagnetic Activity Occurring in the Earth's Northern and Southern Hemisphere (KM, KN, KS Indices)

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The NOAA National Centers for Environmental Information (formerly National Geophysical Data Center) receives on a monthly basis, the KM, KN, KS indices from Institue...

  4. Convergence of Multiple MAP3Ks on MKK3 Identifies a Set of Novel Stress MAPK Modules

    KAUST Repository

    Colcombet, Jean; Sö zen, Cé cile; Hirt, Heribert

    2016-01-01

    Since its first description in 1995 and functional characterization 12 years later, plant MKK3-type MAP2Ks have emerged as important integrators in plant signaling. Although they have received less attention than the canonical stress-activated

  5. Observation of the B-s(0) -> J/psi (KsK +/-)-K-0 pi(-/+) decay

    NARCIS (Netherlands)

    Aaij, R.; Collaboration, Lhcb; Adeva, B.; Adinolfi, M.; Affolder, A.; Ajaltouni, Z.; Albrecht, J.; Alessio, F.; Alexander, M.; Ali, S.; Alkhazov, G.; Alvarez Cartelle, P.; Alves, A. A.; Amato, S.; Amerio, S.; Amhis, Y.; An, L.; Anderlini, L.; Anderson, J.; Andreassen, R.; Andreotti, M.; Andrews, J. E.; Appleby, R. B.; Gutierrez, O. Aquines; Archilli, F.; Artamonov, A.; Artuso, M.; Aslanides, E.; Auriemma, G.; Baalouch, M.; Bachmann, S.; Back, J. J.; Badalov, A.; Balagura, V.; Baldini, W.; Barlow, R. J.; Barschel, C.; Barsuk, S.; Barter, W.; Batozskaya, V.; Bay, A.; Beaucourt, L.; Beddow, J.; Bedeschi, F.; Bediaga, I.; Belogurov, S.; Belous, K.; Belyaev, I.; Ben-Haim, E.; Bencivenni, G.; Benson, S.; Benton, J.; Berezhnoy, A.; Bernet, R.; Bettler, M. -O; van Beuzekom, M.; Bien, A.; Bifani, S.; Bird, T.; Bizzetii, A.; Bjornstad, P. M.; Blake, T.; Blanc, F.; Blouw, J.; Blusk, S.; Bocci, V.; Bondar, A.; Bondar, N.; Bonivento, W.; Borghi, S.; Borgia, A.; Borsato, M.; Bowcock, T. J. V.; Bowen, E.; Bozzi, C.; Brambach, T.; van den Brand, J.; Bressieux, J.; Brett, D.; Britsch, M.; Britton, T.; Brodzicka, J.; Brook, N. H.; Brown, H.; Bursche, A.; Busetto, G.; Buytaert, J.; Cadeddu, S.; Calabrese, R.; Calvi, M.; Gomez, M. Calvo; Camboni, A.; Campana, P.; Perez, D. Campora; Carbone, A.; Carboni, G.; Cardinale, R.; Cardini, A.; Carranza-Mejia, H.; Carson, L.; Akiba, K. Carvalho; Casse, G.; Cassina, L.; Garcia, L. Castillo; Cattaneo, M.; Cauet, Ch; Cenci, R.; Charles, M.; Charpentier, Ph.; Chen, S.; Cheung, S. -F.; Chiapolini, N.; Chrzaszcz, M.; Ciba, K.; Vidal, X. Cid; Ciezarek, G.; Clarke, P. E. L.; Clemencic, M.; Cliff, H. V.; Closier, J.; Coco, V.; Cogan, J.; Cogneras, E.; Collins, P.; Comerma-Montells, A.; Contu, A.; Cook, A.; Coombes, M.; Coquereau, S.; Corti, G.; Corvo, M.; Counts, I.; Couturier, B.; Cowan, G. A.; Craik, D. C.; Cruz Torres, M.; Cunliffe, S.; Currie, R.; D'Ambrosio, C.; Dalseno, J.; David, P.; David, P. N. Y.; Davis, A.; De Bruyn, K.; De Capua, S.; De Cian, M.; De Miranda, J. M.; De Paula, L.; De Silva, W.; De Simone, P.; Decamp, D.; Deckenhoff, M.; Del Buono, L.; Deleage, N.; Derkach, D.; Deschamps, O.; Dettori, F.; Di Canto, A.; Dijkstra, H.; Donleavy, S.; Dordei, F.; Dorigo, M.; Dosil Suarez, A.; Dossett, D.; Dovbnya, A.; Dujany, G.; Dupertuis, F.; Durante, P.; Dzhelyadin, R.; Dziurda, A.; Dzyuba, A.; Easo, S.; Egede, U.; Egorychev, V.; Eidelman, S.; Eisenhardt, S.; Eitschberger, U.; Ekelhof, R.; Eklund, L.; El Rifai, I.; Elsasser, Ch.; Ely, S.; Esen, S.; Evans, T.; Falabella, A.; Faerber, C.; Farinelli, C.; Farley, N.; Farry, S.; Ferguson, D.; Fernandez Albor, V.; Rodrigues, F. Ferreira; Ferro-Luzzi, M.; Filippov, S.; Fiore, M.; Fiorini, M.; Firlej, M.; Fitzpatrick, C.; Fiutowski, T.; Fontana, M.; Fontanelli, F.; Forty, R.; Francisco, O.; Frank, M.; Frei, C.; Frosini, M.; Fu, J.; Furfaro, E.; Torreira, A. Gallas; Galli, D.; Gallorini, S.; Gambetta, S.; Gandelman, M.; Gandini, P.; Gao, Y.; Garofoli, J.; Tico, J. Garra; Garrido, L.; Gaspar, C.; Gauld, R.; Gavardi, L.; Gersabeck, E.; Gersabeck, M.; Gershon, T.; Ghez, Ph; Gianelle, A.; Giani', S.; Gibson, V.; Giubega, L.; Gligorov, V. V.; Goebel, C.; Golubkov, D.; Golutvin, A.; Gomes, A.; Gordon, H.; Gotti, C.; Gandara, M. Grabalosa; Diaz, R. Graciani; Cardoso, L. A. Granado; Grauges, E.; Graziani, G.; Grecu, A.; Greening, E.; Gregson, S.; Griffith, P.; Grillo, L.; Gruenberg, O.; Gui, B.; Gushchin, E.; Guz, Yu; Gys, T.; Hadjivasiliou, C.; Haefeli, G.; Haen, C.; Haines, S. C.; Hall, S.; Hamilton, B.; Hampson, T.; Han, X.; Hansmann-Menzemer, S.; Harnew, N.; Harnew, S. T.; Harrison, J.; Hartmann, T.; He, J.; Head, T.; Heijne, V.; Hennessy, K.; Henrard, P.; Henry, L.; Hernando Morata, J. A.; van Herwijnen, E.; Hess, M.; Hicheur, A.; Hill, D.; Hoballah, M.; Hombach, C.; Hulsbergen, W.; Hunt, P.; Hussain, N.; Hutchcroft, D.; Hynds, D.; Idzik, M.; Ilten, P.; Jacobsson, R.; Jaeger, A.; Jalocha, J.; Jans, E.; Jaton, P.; Jawahery, A.; Jezabek, M.; Jing, F.; John, M.; Johnson, D.; Jones, C. R.; Joram, C.; Jost, B.; Jurik, N.; Kaballo, M.; Kandybei, S.; Kanso, W.; Karacson, M.; Karbach, T. M.; Kelsey, M.; Kenyon, I. R.; Kete, T.; Khanji, B.; Khurewathanaku, C.; Klaver, S.; Kochebina, O.; Lpinii, M. Ko; Komarov, I.; Koopman, R. F.; Koppenburg, P.; Korolev, M.; Kozinskiy, A.; Kravchuk, L.; Kreplin, K.; Kreps, M.; Krocker, G.; Krokovny, P.; Kruse, F.; Kucharczyk, M.; Kudryavtsev, V.; Kurek, K.; Kvaratskheliya, T.; La Thi, V. N.; Lacarrere, D.; Lafferty, G.; Lai, A.; Lambert, D.; Lambert, R. W.; Lanciotti, E.; Lanfranchi, G.; Langenbruch, C.; Langhans, B.; Latham, T.; Lazzeroni, C.; Le Gac, R.; van Leerdam, J.; Lees, J. -P.; Lefevre, R.; Leflat, A.; Lefrancois, J.; Leo, S.; Leroy, O.; Lesiak, T.; Leverington, B.; Li, Y.; Liles, M.; Lindner, R.; Linn, C.; Lionetto, F.; Liu, B.; Liu, G.; Lohn, S.; Longtaff, I.; Lopes, J. H.; Lopez-March, N.; Lowdon, P.; Lu, H.; Lucchesi, D.; Luo, H.; Lupato, A.; Luppi, E.; Lupton, O.; Machefert, F.; Machikhiliyan, I. V.; Maciuc, F.; Maev, O.; Malde, S.; Manca, G.; Mancinelli, G.; Manzali, M.; Maratas, J.; Marchand, J. F.; Marconi, U.; Benito, C. Marin; Marino, P.; Maerki, R.; Marks, J.; Martellotti, G.; Martens, A.; Martin Sanchez, A.; Martinelli, M.; Martinez Santos, D.; Martinez Vida, F.; Martins Tostes, D.; Massafferri, A.; Matev, R.; Mathe, Z.; Matteuzzi, C.; Mazurov, A.; McCann, M.; McCarthy, J.; McNab, A.; McNulty, R.; McSkelly, B.; Meadows, B.; Meier, F.; Meissner, M.; Merk, M.; Milanes, D. A.; Minard, M. -N.; Moggi, N.; Molina Rodriguez, J.; Monteil, S.; Moran, D.; Morandin, M.; Morawski, P.; Morda, A.; Morello, M. J.; Moron, J.; Morris, A. -B.; Mountain, R.; Muheim, F.; Mueller, K.; Muresan, R.; Mussini, M.; Muster, B.; Naik, P.; Nakada, T.; Nandakumar, R.; Nasteva, I.; Needham, M.; Neri, N.; Neubert, S.; Neufeld, N.; Neuner, M.; Nguyen, A. D.; Nguyen, T. D.; Nguyen-Mau, C.; Nicol, M.; Niess, V.; Niet, R.; Nikitin, N.; Nikodem, T.; Novoselov, A.; Oblakowska-Mucha, A.; Obraztsov, V.; Oggero, S.; Ogilvy, S.; Okhrimenko, O.; Oldeman, R.; Onderwater, G.; Orlandea, M.; Goicochea, J. M. Otalora; Owen, P.; Oyanguren, A.; Pal, B. K.; Palano, A.; Palombo, F.; Palutan, M.; Panman, J.; Papanestis, A.; Pappagallo, M.; Parkes, C.; Parkinson, C. J.; Passaleva, G.; Patel, G. D.; Patel, M.; Patrignani, C.; Pazos Alvarez, A.; Pearce, A.; Pellegrino, A.; Altarelli, M. Pepe; Perazzini, S.; Trigo, E. Perez; Perret, P.; Perrin-Terrin, M.; Pescatore, L.; Pesen, E.; Petridis, K.; Petrolini, A.; Picatoste Olloqui, E.; Pietrzyk, B.; Pilar, T.; Pinci, D.; Pistone, A.; Playfer, S.; Plo Casasus, M.; Polci, F.; Poluektov, A.; Polycarpo, E.; Popov, A.; Popov, D.; Popovici, B.; Potterat, C.; Powell, A.; Prisciandaro, J.; Pritchard, A.; Prouve, C.; Pugatch, V.; Navarro, A. Puig; Punzi, G.; Qian, W.; Rachwal, B.; Rademacker, J. H.; Rakotomiaramanana, B.; Rama, M.; Rangel, M. S.; Raniuk, I.; Rauschmayr, N.; Raven, G.; Reichert, S.; Reid, M. M.; dos Reis, A. C.; Ricciardi, S.; Richards, A.; Rihl, M.; Rinnert, K.; Rives Molina, V.; Roa Romero, D. A.; Robbe, P.; Rodrigues, A. B.; Rodrigues, E.; Perez, P. Rodriguez; Roiser, S.; Romanovsky, V.; Romero Vidal, A.; Rotondo, M.; Rouvinet, J.; Ruf, T.; Ruffini, F.; Ruiz, H.; Ruiz Valls, P.; Sabatino, G.; Saborido Silva, J. J.; Sagidova, N.; Sail, P.; Saitta, B.; Salustino Guimaraes, V.; Sanchez Mayordomo, C.; Sanmartin Sedes, B.; Santacesaria, R.; Santamarina Rios, C.; Santovetti, E.; Sapunov, M.; Sarti, A.; Satriano, C.; Satta, A.; Savrie, M.; Savrina, D.; Schiller, M.; Schindler, H.; Schlupp, M.; Schmelling, M.; Schmidt, B.; Schneider, O.; Schopper, A.; Schune, M. -H.; Schwemmer, R.; Sciascia, B.; Sciubba, A.; Seco, M.; Semennikov, A.; Senderowska, K.; Sepp, I.; Serra, N.; Serrano, J.; Sestini, L.; Seyfert, P.; Shapkin, M.; Shapoval, I.; Shcheglov, Y.; Shears, T.; Shekhtman, L.; Shevchenko, V.; Shires, A.; Coutinho, R. Silva; Simi, G.; Sirendi, M.; Skidmore, N.; Skwarnicki, T.; Smith, N. A.; Smith, E.; Smith, E.; Smith, J.; Smith, M.; Snoek, H.; Sokoloff, M. D.; Soler, F. J. P.; Soomro, F.; Souza, D.; De Paula, B. Souza; Spaan, B.; Sparkes, A.; Spinella, F.; Spradlin, P.; Stagni, F.; Stahl, S.; Steinkamp, O.; Stenyakin, O.; Stevenson, S.; Stoica, S.; Stone, S.; Storaci, B.; Stracka, S.; Straticiuc, M.; Straumann, U.; Stroili, R.; Subbiah, V. K.; Sun, L.; Sutcliffe, W.; Swientek, K.; Swientek, S.; Syropoulos, V.; Szczekowski, M.; Szczypka, P.; Szilard, D.; Szumlak, T.; T'Jampens, S.; Teklishyn, M.; Tellarini, G.; Teubert, F.; Thomas, C.; Thomas, E.; van Tilburg, J.; Tisserand, V.; Tobin, M.; Tolk, S.; Tomassetti, L.; Tonelli, D.; Topp-Joergensen, S.; Torr, N.; Tournefier, E.; Tourneur, S.; Tran, M. T.; Tresch, M.; Tsaregorodtsev, A.; Tsopelas, P.; Tuning, N.; Garcia, M. Ubeda; Ukleja, A.; Ustyuzhanin, A.; Uwer, U.; Vagnoni, V.; Valenti, G.; Vallier, A.; Gomez, R. Vazquez; Regueiro, P. Vazquez; Sierra, C. Vazquez; Vecchi, S.; Velthuis, J. J.; Veltri, M.; Veneziano, G.; Vesterinen, M.; Viaud, B.; Vieira, D.; Vieites Diaz, M.; Vilasis-Cardona, X.; Vollhardt, A.; Volyanskyy, D.; Voong, D.; Vorobyev, A.; Vorobyev, V.; Voss, C.; Voss, H.; de Vries, J. A.; Waldi, R.; Wallace, C.; Wallace, R.; Walsh, J.; Wandernoth, S.; Wang, J.; Ward, D. R.; Watson, N. K.; Websdale, D.; Whitehead, M.; Wicht, J.; Wiedner, D.; Wilkinson, G.; Williams, M. P.; Williams, M.; Wilson, F. F.; Wimberley, J.; Wishahi, J.; Wislicki, W.; Witek, M.; Wormser, G.; Wotton, S. A.; Wright, S.; Wu, S.; Wyllie, K.; Xie, Y.; Xing, Z.; Xu, Z.; Yang, Z.; Yuan, X.; Yushchenko, O.; Zangoli, M.; Zavertyaev, M.; Zhang, F.; Zhang, L.; Zhang, W. C.; Zhang, Y.; Zhelezov, A.; Zhokhov, A.; Zhong, L.; Zvyagin, A.

    2014-01-01

    Decays of the form B-s(0) -> J/psi K(s)(0)h(+)h((1)-) (h((1)) = K, pi) are searched for in proton-proton collision data corresponding to an integrated luminosity of 1.0 fb(-1) recorded with the LHCb detector. The first observation of the B-s(0) -> J/psi (KsK +/-)-K-0 pi(-/+) decay is reported, with

  6. Klinefelter syndrome comorbidities linked to increased X chromosome gene dosage and altered protein interactome activity

    DEFF Research Database (Denmark)

    Belling, Kirstine González-Izarzugaza; Russo, Francesco; Jensen, Anders Boeck

    2017-01-01

    Klinefelter syndrome (KS) (47,XXY) is the most common male sex chromosome aneuploidy. Diagnosis and clinical supervision remain a challenge due to varying phenotypic presentation and insufficient characterization of the syndrome. Here we combine health data-driven epidemiology and molecular level...

  7. Phenomenological characteristics of autobiographical memory in Korsakoff's syndrome.

    Science.gov (United States)

    El Haj, Mohamad; Nandrino, Jean-Louis

    2017-10-01

    A body of research suggests compromise of autobiographical memory in Korsakoff's syndrome (KS). The present paper extends this literature by investigating the subjective experience of autobiographical recall in the syndrome. Patients with KS and controls were asked to retrieve autobiographical memories. After memory retrieval, participants were asked to rate phenomenological characteristics of their memories (i.e., reliving, back in time, remembering, realness, visual imagery, auditory imagery, language, emotion, rehearsal, importance, spatial recall and temporal recall). Analysis showed lower "Mean Phenomenological Experience" in the Korsakoff patients than in controls. However, the Korsakoff patients attributed relatively high emotional value and importance to their memories. Although our findings suggest compromised phenomenological reliving of autobiographical memory in patients with KS, affective characteristics such as emotion and importance are likely to play a main role in the subjective experience of the past in these patients. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Klinefelter syndrome and fertility: sperm preservation should not be offered to children with Klinefelter syndrome.

    Science.gov (United States)

    Franik, S; Hoeijmakers, Y; D'Hauwers, K; Braat, D D M; Nelen, W L M; Smeets, D; Claahsen-van der Grinten, H L; Ramos, L; Fleischer, K

    2016-09-01

    Should fertility preservation be offered to children with Klinefelter syndrome (KS)? Current evidence shows that fertility preservation should not be offered to adolescents with KS younger than 16 years because of lower retrieval rates for germ cells by testicular sperm extraction (TESE) compared with retrieval rates for adolescents and adults between 16 and 30 years. KS, the most common chromosomal disorder in men leading to non-obstructive azoospermia, is caused by the presence of at least one additional X chromosome. The onset of puberty in adolescents with KS leads to progressive degeneration of the testicular environment. The impact of the subsequent tissue degeneration on fertility potential of patients with KS is unknown, but in previous literature it has been suggested that fertility preservation should be started in adolescents as early as possible. However spermatozoa can be found by TESE in about 50% of adults with KS despite severe testicular degeneration. This review discusses the current evidence for fertility preservation in children and adolescents and possible prognostic markers for fertility treatment in KS. An extensive literature search was conducted, searching Pubmed, Embase, Cinahl and Web of Science from origin until April 2016 for 'Klinefelter syndrome' and 'fertility' and various synonyms. Titles and abstracts have been scanned manually by the authors for eligibility. In total 76 studies were found to be eligible for inclusion in this review. Information from the papers was extracted separately by two authors. Various studies have shown that pre-pubertal children with KS already have a reduced number of germ cells despite a normal hormonal profile during childhood. The presence of spermatozoa in the ejaculate of adolescents with KS is extremely rare. Using TESE, the retrieval rates of spermatozoa for adolescents younger than 16 years old are much lower (0-20%) compared with those for adolescents and young adults between 16 and 30 years old

  9. A case of Klinefelter syndrome with hypersexual desire

    Directory of Open Access Journals (Sweden)

    Kingsley Okolie

    2017-09-01

    Full Text Available Klinefelter syndrome (KS is a chromosomal disorder affecting males, with the typical karyotype of 47,XXY due to a supernumerary X chromosome, which causes progressive testicular failure resulting in androgen deficiency and infertility. Despite it being the most common sex chromosomal disorder, its diagnosis is easily missed. In addition to its classical clinical features of tall stature, gynaecomastia, small testes, and symptoms and signs of hypogonadism including infertility, KS is also often associated with neurocognitive, behavioural and psychiatric disorders. We present a 44-year-old man with KS who, despite having erectile dysfunction, paradoxically had increased libido. He used sildenafil to overcome his erectile dysfunction. Hypersexuality was manifested by very frequent masturbation, multiple sexual partners most of whom were casual, and a sexual offence conviction at the age of 17 years. Discussion focuses on the frequent failure of clinicians to diagnose KS, the neurocognitive, behavioural and psychiatric aspects of KS, this unusual presentation of hypersexuality in a man with KS, and the challenges of medical management of hypogonadism in a man with a history of a sexual offence.

  10. Klinefelter syndrome, cardiovascular system, and thromboembolic disease: review of literature and clinical perspectives.

    Science.gov (United States)

    Salzano, Andrea; Arcopinto, Michele; Marra, Alberto M; Bobbio, Emanuele; Esposito, Daniela; Accardo, Giacomo; Giallauria, Francesco; Bossone, Eduardo; Vigorito, Carlo; Lenzi, Andrea; Pasquali, Daniela; Isidori, Andrea M; Cittadini, Antonio

    2016-07-01

    Klinefelter syndrome (KS) is the most frequently occurring sex chromosomal aberration in males, with an incidence of about 1 in 500-700 newborns. Data acquired from large registry-based studies revealed an increase in mortality rates among KS patients when compared with mortality rates among the general population. Among all causes of death, metabolic, cardiovascular, and hemostatic complication seem to play a pivotal role. KS is associated, as are other chromosomal pathologies and genetic diseases, with cardiac congenital anomalies that contribute to the increase in mortality. The aim of the current study was to systematically review the relationships between KS and the cardiovascular system and hemostatic balance. In summary, patients with KS display an increased cardiovascular risk profile, characterized by increased prevalence of metabolic abnormalities including Diabetes mellitus (DM), dyslipidemia, and alterations in biomarkers of cardiovascular disease. KS does not, however, appear to be associated with arterial hypertension. Moreover, KS patients are characterized by subclinical abnormalities in left ventricular (LV) systolic and diastolic function and endothelial function, which, when associated with chronotropic incompetence may led to reduced cardiopulmonary performance. KS patients appear to be at a higher risk for cardiovascular disease, attributing to an increased risk of thromboembolic events with a high prevalence of recurrent venous ulcers, venous insufficiency, recurrent venous and arterial thromboembolism with higher risk of deep venous thrombosis or pulmonary embolism. It appears that cardiovascular involvement in KS is mainly due to chromosomal abnormalities rather than solely on low serum testosterone levels. On the basis of evidence acquisition and authors' own experience, a flowchart addressing the management of cardiovascular function and prognosis of KS patients has been developed for clinical use. © 2016 European Society of Endocrinology.

  11. Testicular parenchymal abnormalities in Klinefelter syndrome: a question of cancer? Examination of 40 consecutive patients

    Directory of Open Access Journals (Sweden)

    Giacomo Accardo

    2015-02-01

    Full Text Available Klinefelter syndrome (KS is a hypergonadotropic hypogonadism characterized by a 47, XXY karyotype. The risk of testicular cancer in KS is of interest in relation to theories about testicular cancer etiology generally; nevertheless it seems to be low. We evaluated the need for imaging and serum tumor markers for testicular cancer screening in KS. Participants were 40 consecutive KS patients, enrolled from December 2009 to January 2013. Lactate dehydrogenase (LDH, alpha-fetoprotein (AFP, and beta-human chorionic gonadotrophin subunit (β-HCG serum levels assays and testicular ultrasound (US with color Doppler, were carried out at study entry, after 6 months and every year for 3 years. Abdominal magnetic resonance (MR was performed in KS when testicular US showed micro-calcifications, testicular nodules and cysts. Nearly 62% of the KS had regular testicular echotexture, 37.5% showed an irregular echotexture and 17.5% had micro-calcifications and cysts. Eighty seven percent of KS had a regular vascular pattern, 12.5% varicocele, 12.5% nodules 1 cm. MR ruled out the diagnosis of cancer in all KS with testicular micro calcifications, nodules and cysts. No significant variations in LDH, AFP, and β-HCG levels and in US pattern have been detected during follow-up. We compared serum tumor markers and US pattern between KS with and without cryptorchidism and no statistical differences were found. We did not find testicular cancer in KS, and testicular US, tumor markers and MR were, in selected cases, useful tools for correctly discriminating benign from malignant lesions.

  12. Veneetsia Kuldlõvi läks Magdaleena õdedele / Tiit Tuumalu

    Index Scriptorium Estoniae

    Tuumalu, Tiit, 1971-

    2002-01-01

    Veneetsia 59. rahvusvahelise filmifestivali peaauhinna võitis Peter Mullan'i mängufilm "Magdaleena õed" ("The Magdalene Sisters"). Žürii grand prix läks Venemaa-Prantsusmaa koostööfilmile - Andrei Kontšalovski "Lollide maja" (ka "Hullumaja", "Dom durakov"), kus üht peaosa mängib Narva harrastusnäitleja Stas Varkki. Parim naisnäitleja on Julianne Moore ja meesnäitleja Stefano Accorsi. Lisatud auhinnasaajate nimekiri

  13. Biochemical Characterization of a Thiol-Activated, Oxidation Stable Keratinase from Bacillus pumilus KS12

    OpenAIRE

    Rajput, Rinky; Sharma, Richa; Gupta, Rani

    2010-01-01

    An extracellular keratinase from Bacillus pumilus KS12 was purified by DEAE ion exchange chromatography. It was a 45?kDa monomer as determined by SDS PAGE analysis. It was found to be an alkaline, serine protease with pH and temperature optima of 10 and 60?C, respectively. It was thiol activated with two- and eight-fold enhancement in presence of 10 mM DTT and ?-mercaptoethanol, respectively. In addition, its activity was stimulated in the presence of various surfactants, detergents, and oxid...

  14. Evaluation of the Main Steam Line Break Accident for the APR+ Standard Design using MARS-KS

    Energy Technology Data Exchange (ETDEWEB)

    Park, M. H.; Kim, Y. S.; Hwang, Min Jeong; Sim, S. K. [Environment Energy Technology, Daejeon (Korea, Republic of); Bang, Young Seok [Korea Institute of Nuclear Safety, Daejeon (Korea, Republic of)

    2015-05-15

    As a part of licensing evaluation of the APR+ (Advanced Power Reactor +) standard design, Korea Institute of Nuclear Safety(KINS) performed safety evaluation of the APR+ Standard Safety Analysis Report(SSAR). The results of the safety evaluation of the APR+ Main Steam Line Break(MSLB) accident is presented for the most limiting post-trip return-to-power case with the single failure assumption of the Loss Of Offsite Power(LOOP). MARS-KS regulatory safety analysis code has been used to evaluate safety as well as the system behavior during MSLB accident. The MARS-KS analysis results are compared with the results of the MSLB safety analysis presented in the SSAR of the APR+. Independent safety evaluation has been performed using MARS-KS regulatory safety analysis code for the APR+ MSLB accident inside containment for the limiting case of the full power post-trip return-to-power. The results of MARS-KS analysis were compared with the results of the MSLB safety analysis presented in the APR+ SSAR. Due to higher cooldown of the MARS-KS analysis, the MARS-KS analysis results in a higher positive reactivity insertion into the core by the negative moderator and fuel temperature reactivity coefficients than the APR+ SSAR analysis. Both results show no return-to-power during the limiting case of the MSLB inside containment. However, APR+ SSAR moderator temperature reactivity insertion should be evaluated against the MARS-KS moderator density reactivity insertion for is conservatism. This study also clearly shows asymmetric thermal hydraulic behavior during the MSLB accident at intact and affected sides of the downcomer and the core. These asymmetric phenomena should be further investigated for the effects on the system design.

  15. Executive functioning in chronic alcoholism and Korsakoff syndrome.

    Science.gov (United States)

    Maharasingam, Malini; Macniven, Jamie A B; Mason, Oliver J

    2013-01-01

    Korsakoff syndrome (KS) is characterized by dense anterograde and retrograde amnesia. There is often a temporal gradient to the retrograde amnesia, with earlier memories more readily recalled than recent memories. Executive functioning has also been found to be impaired in KS. However, research comparing executive functioning between chronic alcoholics (AL) and patients with KS has been relatively sparse to date. In a group comparison design, executive functioning in 15 KS patients and 16 chronic alcoholic patients was assessed using the Behavioural Assessment of the Dysexecutive Syndrome test (BADS) and other secondary measures. The KS group was found to be significantly more impaired than the AL group on overall performance on the BADS (p Korsakoff patients are significantly more impaired in executive functioning than non-Korsakoff chronic alcoholics. We thank the participants of the study and also acknowledge the support of the University of Nottingham, particularly Nadina Lincoln, and the North East London NHS Foundation Trust. We are also very grateful to the anonymous reviewers of earlier drafts of this manuscript for their invaluable comments.

  16. Gastric and Peritoneal Involvement of Human Herpes Virus 8 Related Kaposi Sarcoma in a Patient with Acquired Immunodeficiency Syndrome

    Directory of Open Access Journals (Sweden)

    Nuno Ribeiro Ferreira

    2015-09-01

    Full Text Available Kaposi's sarcoma (KS is one of the most frequent neoplastic diseases in patients infected with human immunodeficiency virus (HIV. The authors report the case of a 40-year-old male with ascites, peripheral edema and peritoneal carcinomatosis secondary to a gastric KS related to human herpes virus type 8 (HHV-8. The patient had severe immunodeficiency, with a TCD4+ count of 86 cells/µl and newly diagnosed acquired immunodeficiency syndrome. His clinical condition rapidly deteriorated, with multiorgan failure, and he died without the possibility of initiating antiretroviral therapy or chemotherapy. To the authors’ knowledge, carcinomatosis is a rare feature in KS.

  17. Fragmentation, labeling and biodistribution studies of KS1/4, a monoclonal antibody

    International Nuclear Information System (INIS)

    Mohd, S.B.

    1987-01-01

    In this study, an IgG2a (KS1/4), a monoclonal antibody (MoAb) specific against a human lung adenocarcinoma (UCLA P-3) was successfully fragmented enzymatically to yield F(ab') 2 and Fab by using pepsin and papain, respectively. The kinetic of fragmentation of the MoAb was compared to that of human immunoglobulin G (IgG). A similar pattern of fragmentation was observed with both antibodies with a higher percentage yield of the F(ab') 2 and Fab obtained upon the fragmentation of the IgG by the enzymes. The KS1/4 and the two fragments were labeled with three different radionuclides, namely iodine-131, indium-111 and selenium-75. The radioiodination of the MoAb and the fragments was carried out by using a modified chloramine-T method. Radiometal labeling of the MoAb and the fragments with indium-111 was performed by using DTPA as a bifunctional chelating agent, while intrinsic labeling of the MoAb was done by culturing the hybridoma in the presence of 75 Se-methionine. The biodistribution of the radiolabeled MoAb, F(ab') 2 and Fab fragments were performed by injecting the preparations intravenously into nude mice bearing human lung adenocarcinoma

  18. Hydraulic Simulation of In-vessel Downstream Effect Test Using MARS-KS Code

    Energy Technology Data Exchange (ETDEWEB)

    Bang, Young Seok; Lee, Joon Soo; Ryu, Seung Hoon [Korea Institute of Nuclear Safety, Daejeon (Korea, Republic of)

    2015-05-15

    In-vessel downstream effect test (IDET) has been required to evaluate the effect of debris on long term core cooling following a loss of coolant accident (LOCA) in support of resolution of Generic Safety Issue (GSI) 191. Head loss induced by debris (fiber and particle) accumulated on prototypical fuel assembly (FA) should be compared with the available driving head to the core for the various combinations of LOCA and Emergency Core Cooling System (ECCS) injection. The actual simulation was conducted using MARS-KS code. Also the influence of small difference in gap size which was found in the actual experiment is evaluated using the present model. A simple model to determine the form loss factors of FA and gap in clean state and the debris laden state is discussed based on basic fluid mechanics. Those form loss factors were applied to the hydraulic simulation of a selected IDET using MARS-KS code. The result indicated that the present model can be applied to IDET simulation. The pressure drop influenced by small difference in gap size can be evaluated by the present model with practical assumption.

  19. Thermodynamic properties of Kehagias-Sfetsos black hole and KS/CFT correspondence

    Science.gov (United States)

    Pradhan, Parthapratim

    2017-11-01

    We speculate on various thermodynamic features of the inner horizon ({\\mathcal H}-) and outer horizons ({\\mathcal H}+) of Kehagias-Sfetsos (KS) black hole (BH) in the background of the Hořava-Lifshitz gravity. We compute particularly the area product, area sum, area minus and area division of the BH horizons. We find that they all are not showing universal behavior whereas the product is a universal quantity (PRADHAN P., Phys. Lett. B, 747 (2015) 64). Based on these relations, we derive the area bound of all horizons. From the area bound we derive the entropy bound and irreducible mass bound for all the horizons ({\\mathcal H}+/-) . We also observe that the first law of BH thermodynamics and Smarr-Gibbs-Duhem relations do not hold for this BH. The underlying reason behind this failure is due to the scale invariance of the coupling constant. Moreover, we compute the Cosmic-Censorship-Inequality for this BH which gives the lower bound for the total mass of the spacetime and it is supported by the cosmic cencorship conjecture. Finally, we discuss the KS/CFT correspondence via a thermodynamic procedure.

  20. Preliminary Analysis of a Loss of Condenser Vacuum Accident Using the MARS-KS Code

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jieun Kim; Bang, Young Seok; Oh, Deog Yeon; Kim, Kap; Woo, Sweng-Wong [Korea Institute of Nuclear Safety, Daejeon (Korea, Republic of)

    2015-05-15

    In accordance with revision of NUREG-0800 of USNRC, the area of review for loss of condenser vacuum(LOCV) accident has been expanded to analyze both peak pressures of primary and secondary system separately. Currently, the analysis of LOCV accident, which is caused by malfunction of condenser, has been focused to fuel cladding integrity and peak pressure in the primary system. In this paper, accident analysis for LOCV using MARS-KS code were conducted to support the licensing review on transient behavior of secondary system pressure of APR1400 plant. The accident analysis for the loss of condenser vacuum (LOCV) of APR1400 was conducted with the MARS-KS code to support the review on the pressure behavior of primary and secondary system. Total four cases which have different combination of availability of offsite power and the pressurizer spray are considered. The preliminary analysis results shows that the initial conditions or assumptions which concludes the severe consequence are different for each viewpoint, and in some cases, it could be confront with each viewpoint. Therefore, with regard to the each acceptance criteria, figuring out and sensitivity analysis of the initial conditions and assumptions for system pressure would be necessary.

  1. Apc bridges Wnt/{beta}-catenin and BMP signaling during osteoblast differentiation of KS483 cells

    Energy Technology Data Exchange (ETDEWEB)

    Miclea, Razvan L., E-mail: R.L.Miclea@lumc.nl [Department of Pediatrics, Leiden University Medical Centre (LUMC), Leiden (Netherlands); Horst, Geertje van der, E-mail: G.van_der_Horst@lumc.nl [Department of Urology, LUMC, Leiden (Netherlands); Robanus-Maandag, Els C., E-mail: E.C.Robanus@lumc.nl [Department of Human Genetics, LUMC, Leiden (Netherlands); Loewik, Clemens W.G.M., E-mail: C.W.G.M.Lowik@lumc.nl [Department of Endocrinology and Metabolic Diseases, LUMC, Leiden (Netherlands); Oostdijk, Wilma, E-mail: W.Oostdijk@lumc.nl [Department of Pediatrics, Leiden University Medical Centre (LUMC), Leiden (Netherlands); Wit, Jan M., E-mail: J.M.Wit@lumc.nl [Department of Pediatrics, Leiden University Medical Centre (LUMC), Leiden (Netherlands); Karperien, Marcel, E-mail: H.B.J.Karperien@tnw.utwente.nl [MIRA Institute for Biomedical Technology and Technical Medicine, Department of Tissue Regeneration, University of Twente, Zuidhorst Room ZH 144, Drienerlolaan 5, 7522 NB Enschede (Netherlands)

    2011-06-10

    The canonical Wnt signaling pathway influences the differentiation of mesenchymal cell lineages in a quantitative and qualitative fashion depending on the dose of {beta}-catenin signaling. Adenomatous polyposis coli (Apc) is the critical intracellular regulator of {beta}-catenin turnover. To better understand the molecular mechanisms underlying the role of Apc in regulating the differentiation capacity of skeletal progenitor cells, we have knocked down Apc in the murine mesenchymal stem cell-like KS483 cells by stable expression of Apc-specific small interfering RNA. In routine culture, KSFrt-Apc{sub si} cells displayed a mesenchymal-like spindle shape morphology, exhibited markedly decreased proliferation and increased apoptosis. Apc knockdown resulted in upregulation of the Wnt/{beta}-catenin and the BMP/Smad signaling pathways, but osteogenic differentiation was completely inhibited. This effect could be rescued by adding high concentrations of BMP-7 to the differentiation medium. Furthermore, KSFrt-Apc{sub si} cells showed no potential to differentiate into chondrocytes or adipocytes. These results demonstrate that Apc is essential for the proliferation, survival and differentiation of KS483 cells. Apc knockdown blocks the osteogenic differentiation of skeletal progenitor cells, a process that can be overruled by high BMP signaling.

  2. New perspectives in the exploration of Korsakoff’s syndrome: The usefulness of neurophysiological markers.

    Directory of Open Access Journals (Sweden)

    Mélanie eBrion

    2016-02-01

    Full Text Available This perspective aims at underlining the usefulness of event-related potentials (ERP to better understand the brain correlates of Korsakoff’s syndrome (KS, a neuropsychiatric disease characterized by severe memory impairment and most frequently resulting as a neurological complication of alcohol-dependence (AD. While ERP have been broadly used in AD, it has up to now been very little applied in KS or in the comparison of KS and AD. Within the framework of dual-process models, an influential theory postulating that addictive states result from an imbalance between under-activated reflective system and over-activated automatic-affective one, this paper proposes: (1 a brief synthesis of the main results of ERP studies in AD and KS, and (2 new research avenues using ERP to identify the electrophysiological correlates of cognitive and emotional dysfunction in KS. These experimental perspectives aim at exploring the continuity hypothesis, which postulates a gradient of impairments from AD to KS. We conclude on the possibility of developing neuropsychological strategies with electrophysiological follow-up to ensure KS diagnosis and test the efficacy of patient’s neurocognitive rehabilitation.

  3. Measuring KS0K± interactions using Pb–Pb collisions at sNN=2.76 TeV

    Directory of Open Access Journals (Sweden)

    S. Acharya

    2017-11-01

    Full Text Available We present the first ever measurements of femtoscopic correlations between the KS0 and K± particles. The analysis was performed on the data from Pb–Pb collisions at sNN=2.76 TeV measured by the ALICE experiment. The observed femtoscopic correlations are consistent with final-state interactions proceeding via the a0(980 resonance. The extracted kaon source radius and correlation strength parameters for KS0K− are found to be equal within the experimental uncertainties to those for KS0K+. Comparing the results of the present study with those from published identical-kaon femtoscopic studies by ALICE, mass and coupling parameters for the a0 resonance are tested. Our results are also compatible with the interpretation of the a0 having a tetraquark structure instead of that of a diquark.

  4. Implicit Memory in Korsakoff’s Syndrome: A Review of Procedural Learning and Priming Studies

    Science.gov (United States)

    Hayes, Scott M.; Fortier, Catherine B.; Levine, Andrea; Milberg, William P.; McGlinchey, Regina

    2013-01-01

    Korsakoff’s syndrome (KS) is characterized by dense anterograde amnesia resulting from damage to the diencephalon region, typically resulting from chronic alcohol abuse and thiamine deficiency. This review assesses the integrity of the implicit memory system in KS, focusing on studies of procedural learning and priming. KS patients are impaired on several measures of procedural memory, most likely due to impairment in cognitive functions associated with alcohol-related neural damage outside of the diencephalon. The pattern of performance on tasks of implicit priming suggests reliance on a residual, non-flexible memory operating more or less in an automatic fashion. Our review concludes that whether measures of implicit memory reveal intact or impaired performance in individuals with KS depends heavily on specific task parameters and demands, including timing between stimuli, the specific nature of the stimuli used in a task, and the integrity of supportive cognitive functions necessary for performance. PMID:22592661

  5. ks mees nägi unes taevalikku õiglust..." : [luuletused] / Paul-Eerik Rummo

    Index Scriptorium Estoniae

    Rummo, Paul-Eerik, 1942-

    1998-01-01

    Sisu: "Üks mees nägi unes taevalikku õiglust..." ; "Plangusakkidel rõhtne laud..." ; "Sünnipärane tarkus - täpselt niipalju..." ; "Kusagil tuksub süda. Ei kellelegi, ei millelegi..." ; Vaade ; Vihma maitsest ; *** ; Esimene vasikas ; Laul kohmakast kromanjoonlasest ; Mõistmisest ; "Ükskord ennepuiste oli kuningriik, kus kõik oli puust..." ; Mäng ; "Koer oli ketis aiateibas..." ; Hamlet laulud 1-2 ; Kaudu mu vaevakaskede ; "Ma seisan mesilastaruna..." ; "Me hoiame nõnda ühte..." ; "Siin olen kasvanud. Tasasel maal..." ; Ikka Liivist mõteldes 1-2 ; Palmipuud ümber istutades laulda ; "Jah ma nägin lumevalgust..." ; "Oo et sädemeid kiljuks mu hing..." ; Maarjaheina kõrreke

  6. Cannes'i filmifestivali Kuldne Palmioks läks Ken Loachile

    Index Scriptorium Estoniae

    2006-01-01

    Cannes'i filmifestivali võitjad. Kuldse Palmioksa võitis Ken Loachi mängufilm "Tuul, mis sasib odrapõldu" ("The Wind That Shakes the Barley"), Grand Prix' sai prantslase Bruno Dumonti film "Flandria" ("Flandres"), parim režissöör oli Alejandro Gonzalez Inarritu filmi eest "Paabel" ("Babel"), parima naisnäitleja auhinna sai grupp näitlejannasid Pedro Almodovari filmist "Tagasipöördumine" ("Volver") ja meesnäitleja auhinna sai samuti grupp näitlejaid prantsuse filmist "Kuulsuse päevad" ("Indigenes"). Žürii eriauhind läks Andrea Arnoldi filmile "Punane tänav" ("Red Road")

  7. Morphological and glucose metabolism abnormalities in alcoholic Korsakoff's syndrome: group comparisons and individual analyses.

    Directory of Open Access Journals (Sweden)

    Anne-Lise Pitel

    Full Text Available BACKGROUND: Gray matter volume studies have been limited to few brain regions of interest, and white matter and glucose metabolism have received limited research attention in Korsakoff's syndrome (KS. Because of the lack of brain biomarkers, KS was found to be underdiagnosed in postmortem studies. METHODOLOGY/PRINCIPAL FINDINGS: Nine consecutively selected patients with KS and 22 matched controls underwent both structural magnetic resonance imaging and (18F-fluorodeoxyglucose positron emission tomography examinations. Using a whole-brain analysis, the between-group comparisons of gray matter and white matter density and relative glucose uptake between patients with KS and controls showed the involvement of both the frontocerebellar and the Papez circuits, including morphological abnormalities in their nodes and connection tracts and probably resulting hypometabolism. The direct comparison of the regional distribution and degree of gray matter hypodensity and hypometabolism within the KS group indicated very consistent gray matter distribution of both abnormalities, with a single area of significant difference in the middle cingulate cortex showing greater hypometabolism than hypodensity. Finally, the analysis of the variability in the individual patterns of brain abnormalities within our sample of KS patients revealed that the middle cingulate cortex was the only brain region showing significant GM hypodensity and hypometabolism in each of our 9 KS patients. CONCLUSIONS/SIGNIFICANCE: These results indicate widespread brain abnormalities in KS including both gray and white matter damage mainly involving two brain networks, namely, the fronto-cerebellar circuit and the Papez circuit. Furthermore, our findings suggest that the middle cingulate cortex may play a key role in the pathophysiology of KS and could be considered as a potential in vivo brain biomarker.

  8. Neuroanatomy and neuropathology associated with Korsakoff's syndrome.

    Science.gov (United States)

    Kril, Jillian J; Harper, Clive G

    2012-06-01

    Although the neuropathology of Korsakoff's syndrome (KS) was first described well over a century ago and the characteristic brain pathology does not pose a diagnostic challenge to pathologists, there is still controversy over the neuroanatomical substrate of the distinctive memory impairment in these patients. Cohort studies of KS suggest a central role for the mammillary bodies and mediodorsal thalamus, and quantitative studies suggest additional damage to the anterior thalamus is required. Rare cases of KS caused by pathologies other than those of nutritional origin provide support for the role of the anterior thalamus and mammillary bodies. Taken together the evidence to date shows that damage to the thalamus and hypothalamus is required, in particular the anterior thalamic nucleus and the medial mammillary nucleus of the hypothalamus. As these nuclei form part of wider memory circuits, damage to the inter-connecting white matter tracts can also result in a similar deficit as direct damage to the nuclei. Although these nuclei and their connections appear to be the primary site of damage, input from other brain regions within the circuits, such as the frontal cortex and hippocampus, or more distant regions, including the cerebellum and amygdala, may have a modulatory role on memory function. Further studies to confirm the precise site(s) and extend of brain damage necessary for the memory impairment of KS are required.

  9. Criminality in men with Klinefelter's syndrome and XYY syndrome: a cohort study.

    Science.gov (United States)

    Stochholm, Kirstine; Bojesen, Anders; Jensen, Anne Skakkebæk; Juul, Svend; Gravholt, Claus Højbjerg

    2012-01-01

    To investigate the criminal pattern in men between 15 and 70 years of age diagnosed with 47,XXY (Klinefelter's syndrome (KS)) or 47,XYY compared to the general population. Register-based cohort study comparing the incidence of convictions among men with KS and with 47,XYY with age- and calendar-matched samples of the general population. Crime was classified into eight types (sexual abuse, homicide, burglary, violence, traffic, drug-related, arson and 'others'). Denmark 1978-2006. All men diagnosed with KS (N=934) or 47,XYY (N=161) at risk and their age- and calendar-time-matched controls (N=88 979 and 15 356, respectively). The incidence of convictions was increased in men with KS (omitting traffic offenses) compared to controls with a HR of 1.40 (95% CI 1.23 to 1.59, pXYY compared to controls with a HR of 1.42 (95% CI 1.14 to 1.77, pcrime types, except drug-related crimes and traffic. Adjusting for socioeconomic variables (education, fatherhood, retirement and cohabitation) reduced the total HR for both KS and 47,XYY to levels similar to controls, while some specific crime types (sexual abuse, arson, etc) remained increased. The overall risk of conviction (excluding traffic offenses) was moderately increased in men with 47,XYY or KS; however, it was similar to controls when adjusting for socioeconomic parameters. Convictions for sexual abuse, burglary, arson and 'others' were significantly increased. The increased risk of convictions may be partly or fully explained by the poor socioeconomic conditions related to the chromosome aberrations.

  10. Bone Mineral Status in Children and Adolescents with Klinefelter Syndrome

    Directory of Open Access Journals (Sweden)

    Stefano Stagi

    2016-01-01

    Full Text Available Objective. Klinefelter syndrome (KS has long-term consequences on bone health. However, studies regarding bone status and metabolism during childhood and adolescence are very rare. Patients. This cross-sectional study involved 40 (mean age: 13.7±3.8 years KS children and adolescents and 80 age-matched healthy subjects. For both patient and control groups, we evaluated serum levels of ionised and total calcium, phosphate, total testosterone, luteinising hormone, follicle stimulating hormone, parathyroid hormone (PTH, 25-hydroxyvitamin D (25(OHD, 1,25-dihydroxyvitamin D, osteocalcin, bone alkaline phosphatase, and urinary deoxypyridinoline concentrations. We also calculated the z-scores of the phalangeal amplitude-dependent speed of sound (AD-SoS and the bone transmission time (BTT. Results. KS children and adolescents showed significantly reduced AD-SoS (p<0.005 and BTT (p<0.0005 z-scores compared to the controls. However, KS patients presented significantly higher PTH (p<0.0001 and significantly lower 25(OHD (p<0.0001, osteocalcin (p<0.05, and bone alkaline phosphatase levels (p<0.005. Interestingly, these metabolic bone disorders were already present in the prepubertal subjects. Conclusions. KS children and adolescents exhibited impaired bone mineral status and metabolism with higher PTH levels and a significant reduction of 25-OH-D and bone formation markers. Interestingly, this impairment was already evident in prepubertal KS patients. Follow-ups should be scheduled with KS patients to investigate and ameliorate bone mineral status and metabolism until the prepubertal ages.

  11. 78 FR 14097 - Pulse Oximeters-Premarket Notification Submissions [510(k)s]; Guidance for Industry and Food and...

    Science.gov (United States)

    2013-03-04

    ... DEPARTMENT OF HEALTH AND HUMAN SERVICES Food and Drug Administration [Docket No. FDA-2007-D-0205 (Formerly 2007D-0252)] Pulse Oximeters--Premarket Notification Submissions [510(k)s]; Guidance for Industry and Food and Drug Administration Staff; Availability AGENCY: Food and Drug Administration, HHS. ACTION...

  12. Veritasu Inguššias : üks tapmise ja sugulaste kättemaksu lugu / Jaanus Piirsalu

    Index Scriptorium Estoniae

    Piirsalu, Jaanus, 1973-

    2008-01-01

    31. augustil tapeti Nazranis Inguššia üks opositsiooniliidritest Magomed Jevlojev. Mõrvatu isa Jahja Jevlojevi sõnul ei rakendata tapmise organiseerijatele ja täidesaatjatele veritasu juhul, kui tapja saab kohtus karistatud. Vt. samas: Veritasu traditsioon näeb ette ka lepitamisvõimalust

  13. 77 FR 20782 - Foreign-Trade Zone 161-Sedgwick County, KS; Application for Temporary/Interim Manufacturing...

    Science.gov (United States)

    2012-04-06

    ... Turbine Nacelles and Hubs); Hutchinson, KS An application has been submitted to the Executive Secretary of... County), Kansas. Under T/IM procedures, Siemens has requested authority to produce wind turbine nacelles... (4008.11), hydraulic hoses (4009.21, 4009.42), rubber gaskets and o-rings (4016.93), vibration dampeners...

  14. Molluscs and Echinoderms from Palaeolithic deposits in the Rock Shelter of Ksâr'akil, Lebanon

    NARCIS (Netherlands)

    Regteren, van C.O.

    1962-01-01

    INTRODUCTION The palaeolithic deposits of the rock shelter of Ksâr'Akil in the Antelias valley have been excavated by a group of American Jesuits in the years 1937-1938 and 1947-1948. Recently the fossil bones from these deposits were reported upon by my colleague Hooijer (1961). When he received

  15. Menea kui keskaegse slaavi-kreeka teksti üks tüüpidest / Dimitri Mironov

    Index Scriptorium Estoniae

    Mironov, Dimitri

    2000-01-01

    25. apr. 2000 toimus Tallinna Pedagoogikaülikoolis slaavi filoloogia osakonna vene keele õppetooli dotsendi Natalja Netšunajeva doktoritöö "Menea kui keskaegse slaavi-kreeka teksti üks tüüpidest" kaitsmine

  16. Uncertainty Evaluation of a Postulated LBLOCA for APR+ using KINS Realistic Evaluation Methodology and MARS-KS

    Energy Technology Data Exchange (ETDEWEB)

    Hwang, Min Jeong; Marigomena, Ralph; Yoo, Tae Ho; Kim, Y. S.; Sim, S. K. [Environment and Energy Technology, Inc., Daejeon (Korea, Republic of); Bang, Young Seok [KINS, Daejeon (Korea, Republic of)

    2014-05-15

    As a part of the regulatory safety research, Korea Institute of Nuclear Safety(KINS) also developed a best estimate safety analysis regulatory audit code, MARS-KS, to realistically predict and better understand the physical phenomena of the design basis accidents. KINS improved uncertainty propagation methodology using MARS-KS and applied the improved uncertainty evaluation method for the Shinkori Units 3 and 4 LBLOC. This study is to evaluate the uncertainty propagation of a postulated LBLOCA and quantify the safety margin using KINS-REM and MARS-KS code for the APR+ (Advanced Pressurizer Reactor Plus) Standard Safety Analysis Report(SSAR) which is under regulatory review by the KINS for its design approval. KINS-REM LBLOCA realistic evaluation methodology was used for the regulatory assessment of the APR+ LBLOCA using MARS-KS to evaluate the uncertainty propagation of the uncertainty variables as well as to assess the safety margin during the limiting case of the APR+ double ended guillotine cold leg LBLOCA. Uncertainty evaluation for the APR+ LBLOCA shows that the reflood PCT with upper limit of 95% probability at 95% confidence level is 1363.2 K and is higher than the blowdown PCT95/95 of 1275.3 K. The result shows that the current evaluation of APR+ LBLOCA PCT is within the acceptance criteria of 1477 K ECCS.

  17. Tallinna-Tartu liinil jääb käima üks rong / Hindrek Riikoja

    Index Scriptorium Estoniae

    Riikoja, Hindrek

    2001-01-01

    Teede- ja sideminister Toivo Jürgenson ning Edelaraudtee juhatuse esimees Henn Ruubel allkirjastasid reisijateveo lepingu, mille järgi jääb Tallinna-Tartu liinil käima üks rong päevas. Uued bussiliinid

  18. [Kätlin Kätlin. Üks pole ühtegi = One is none] / Katrin Väli

    Index Scriptorium Estoniae

    Väli, Katrin, 1956-

    2008-01-01

    Tutvustus: Kätlin, Kätlin, pseud. Üks pole ühtegi = One is none / Kätlin Kätlin ; [tõlkijad Mika Keränen ja Marina Tervonen ; illustratsioonid: Hanneleele Kaldmaa. Tallinn : Positive Projects], 2008

  19. BoKS 1.0. : Een voorstel voor een body of knowlegde & skills van de communicatieve competentie

    NARCIS (Netherlands)

    Els van der Pool; Monique van Wijk; Cecilia van Dongen

    2010-01-01

    Sinds 2008 bestaat er een landelijke Body of Knowledge & Skills voor vijf domeinen binnen het hoger onderwijs, te weten Business Administration, Commerce, Communications, Economics en Laws (Hbo-raad 2008). De term Body of Knowledge & Skills (BoKS) staat voor het geheel van kennis, vaardigheden en

  20. Kallman syndrome versus idiopathic hypogonadotropic hypogonadism at MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Vogl, T.J.; Stemmler, J.; Bergman, C.; Balzer, J.O.; Felix, R. [Free Univ. of Berlin (Germany); Heye, B.; Schopohl, J.; Danek, A. [Univ. of Munich (Germany)

    1994-04-01

    To identify morphologic differences between Kallman syndrome (KS) and idiopathic hypogonadotropic hypogonadism (IHH) and establish a role for magnetic resonance (MR) imaging in these disorders. Twenty-eight patients were compared with 10 eugonal male volunteers. Eighteen patients had KS (hypogonadotropic hypogonadism with anosmia) and 10 had IHH. All participants underwent hormone analysis, a sniff-bottle smell test, and gadolinium-enhanced MR imaging. Changes in the hypothalamic-hypophyseal region and the rhinencephalon were evaluated. MR imaging revealed intracranial morphologic changes in all patients on plain T1-weighted sections. Seventeen patients with KS demonstrated aplasia of an olfactory bulb; one olfactory sulcus was absent in six, rudimentary in four, and normal in eight. Olfactory bulbs were present in all 10 IHH patients and three showed one slightly hypoplastic bulb. Ten patients with KS and three with IHH showed an enlarged paranasal sinus system. Further MR findings were similar. MR imaging demonstrates abnormalities of the rhinencephalon present in KS patients and occasionally absent in IHH patients. 18 refs., 10 figs., 1 tab.

  1. Investigation of Loop Seal Clearing Phenomena for the ATLAS SBLOCA Long Term Cooling Test using TRACE and MARS-KS

    Energy Technology Data Exchange (ETDEWEB)

    Hwang, Min Jeong; Park, M. H.; Marigomen Ralph; Sim, S. K. [Environment and Energy Technology, Daejeon (Korea, Republic of)

    2016-10-15

    During Design Certificate(DC) review of the APR1400, USNRC raised a long term cooling safety issue on the effect of loop seal clearing during cold leg Small Break Loss Of Coolant Accident(SBLOCA) due to relatively deep cross-over loop compared to the US PWRs. The objective of this study is thus to investigate the loop seal clearing phenomena during cold leg slot break SBLOCA long term cooling and resolve the safety issue on the SBLOCA long term cooling related to the APR1400 DC. TRACE and MARS-KS were used to predict the test results and to perform sensitivity studies for the SBLOCA loop seal clearing phenomena. The calculation shows that the TRACE code well predict the sequence of Test LTC-CL-04R. However, compared to the experiment, the TRACE over predicts the primary pressure due to smaller break flow prediction. MARS-KS well predicts major thermal hydraulic parameters during the transient with reasonable agreement. MARS-KS better predicts ATLAS LTC-CL-04R test data with a good agreement than the TRACE due to better prediction of the break flow. Overall, compared to the experiment, the TRACE and MARS-KS Codes show a discrepancy in predicting the loop seal clearing and reformation time. Both TRACE and MARS-KS correctly predicts core water level and fuel cladding temperatures. From this study, it can be said that even though APR1400 cross-over leg design has slightly deeper loop seals, the effect on the safety of the SBLOCA long term cooling is minimal compared to the SBLOCA cladding failure criteria. Further study on the SBLOCA loop seal clearing phenomena is needed.

  2. Quantitative analysis of the Ca2+‐dependent regulation of delayed rectifier K+ current I Ks in rabbit ventricular myocytes

    Science.gov (United States)

    Bartos, Daniel C.; Morotti, Stefano; Ginsburg, Kenneth S.; Grandi, Eleonora

    2017-01-01

    Key points [Ca2+]i enhanced rabbit ventricular slowly activating delayed rectifier K+ current (I Ks) by negatively shifting the voltage dependence of activation and slowing deactivation, similar to perfusion of isoproterenol.Rabbit ventricular rapidly activating delayed rectifier K+ current (I Kr) amplitude and voltage dependence were unaffected by high [Ca2+]i.When measuring or simulating I Ks during an action potential, I Ks was not different during a physiological Ca2+ transient or when [Ca2+]i was buffered to 500 nm. Abstract The slowly activating delayed rectifier K+ current (I Ks) contributes to repolarization of the cardiac action potential (AP). Intracellular Ca2+ ([Ca2+]i) and β‐adrenergic receptor (β‐AR) stimulation modulate I Ks amplitude and kinetics, but details of these important I Ks regulators and their interaction are limited. We assessed the [Ca2+]i dependence of I Ks in steady‐state conditions and with dynamically changing membrane potential and [Ca2+]i during an AP. I Ks was recorded from freshly isolated rabbit ventricular myocytes using whole‐cell patch clamp. With intracellular pipette solutions that controlled free [Ca2+]i, we found that raising [Ca2+]i from 100 to 600 nm produced similar increases in I Ks as did β‐AR activation, and the effects appeared additive. Both β‐AR activation and high [Ca2+]i increased maximally activated tail I Ks, negatively shifted the voltage dependence of activation, and slowed deactivation kinetics. These data informed changes in our well‐established mathematical model of the rabbit myocyte. In both AP‐clamp experiments and simulations, I Ks recorded during a normal physiological Ca2+ transient was similar to I Ks measured with [Ca2+]i clamped at 500–600 nm. Thus, our study provides novel quantitative data as to how physiological [Ca2+]i regulates I Ks amplitude and kinetics during the normal rabbit AP. Our results suggest that micromolar [Ca2+]i, in the submembrane or junctional cleft

  3. Sediment and plankton lift off recirculations in strong synthetic turbulence (KS)

    Science.gov (United States)

    Redondo, Jose M.; Castilla, Roberto; Sekula, Emil; Furmanek, Petr

    2014-05-01

    The study of particle diffusion and of turbulent sedimentation is of great importance in many geophysical fields, such as in Environmental Science or Oceanography as well as in Bio-environmental and industrial processes. For a long time, the study of diffusion was numerically computed with random free paths, which gives Brownian behavior. (Richardson 1929). These stochastics methods have the objection that do not take into account the flow profile. On the other hand, there are many ways to simulate a fluid flow, but when this is turbulent our aim is to simulate the behaviour of neutral or heavy and inertial particles of biological or geological nature in a turbulent flow, in a simple way with a kinematically simulated model and to validate the results. We use the Kinematic Simulation (KS) model, also known as Synthetic Turbulence, suggested by Kraichnan (1966) and developed further by Castilla et al.(2007), Nicolleau et al.(2012). In this model, velocity field is generated through a Fourier series of random modes. The typical scales and the energy spectrum of the turbulence are inputs of the model. As we do not solve the flow in a discrete grid, but use a random predictive expression, we can simulate the flow at the smallest scales. In an unstratified flow, a KS flow field consists of a random, truncated Fourier representation in space and time, subject to constraints associated with incompressibility, and a prescribed initial energy spectrum. For stratified calculations, two further constraints are imposed, associated with the internal wave field in stratified flows, and the tendency of density variations to suppress vertical motion. With these model modifications, good agreement is found between KS and DNS with regard to the confinement in the vertical direction characteristic of stratified turbulence. Since stratifed flows exhibit this vertical confinement, KS in strictly two dimensions was considered as a first step to understanding dispersion within a

  4. Sequence Alterations of I(Ks Potassium Channel Genes in Kazakhstani Patients with Atrial Fibrillation

    Directory of Open Access Journals (Sweden)

    Ainur Akilzhanova

    2014-12-01

    Full Text Available Introduction. Atrial fibrillation (AF is the most common sustained arrhythmia, and it results in significant morbidity and mortality. However, the pathogenesis of AF remains unclear to date. Recently, more pieces of evidence indicated that AF is a multifactorial disease resulting from the interaction between environmental factors and genetics. Recent studies suggest that genetic mutation of the slow delayed rectifier potassium channel (I(Ks may underlie AF.Objective. To investigate sequence alterations of I(Ks potassium channel genes KCNQ1, KCNE1 and KCNE2 in Kazakhstani patients with atrial fibrillation.Methods. Genomic DNA of 69 cases with atrial fibrillation and 27 relatives were analyzed for mutations in all protein-coding exons and their flanking splice site regions of the genes KCNQ1 (NM_000218.2 and NM_181798.1, KCNE1 (NM_000219.2, and KCNE2 (NM_172201.1 using bidirectional sequencing on the ABI 3730xL DNA Analyzer (Applied Biosystems, Foster City, CA, USA.Results. In total, a disease-causing mutation was identified in 39 of the 69 (56.5% index cases. Of these, altered sequence variants in the KCNQ1 gene accounted for 14.5% of the mutations, whereas a KCNE1 mutation accounted for 43.5% of the mutations and KCNE2 mutation accounted for 1.4% of the mutations. The majority of the distinct mutations were found in a single case (80%, whereas 20% of the mutations were observed more than once. We found two sequence variants in KCNQ1 exon 13 (S546S G1638A and exon 16 (Y662Y, C1986T in ten patients (14.5%. In KCNE1 gene in exon 3 mutation, S59G A280G was observed in 30 of 69 patients (43.5% and KCNE2 exon 2 T10K C29A in 1 patient (1.4%. Genetic cascade screening of 27 relatives to the 69 index cases with an identified mutation revealed 26.9% mutation carriers  who were at risk of cardiac events such as syncope or sudden unexpected death.Conclusion. In this cohort of Kazakhstani index cases with AF, a disease-causing mutation was identified in

  5. An Extra X or Y Chromosome: Contrasting the Cognitive and Motor Phenotypes in Childhood in Boys with 47,XYY Syndrome or 47,XXY Klinefelter Syndrome

    Science.gov (United States)

    Ross, Judith L.; Zeger, Martha P. D.; Kushner, Harvey; Zinn, Andrew R.; Roeltgen, David P.

    2009-01-01

    Objective: The goal of this study was to contrast the cognitive phenotypes in boys with 47,XYY (XYY) karyotype and boys with 47,XXY karyotype [Klinefelter syndrome, (KS)], who share an extra copy of the X-Y pseudoautosomal region but differ in their dosage of strictly sex-linked genes. Methods: Neuropsychological evaluation of general cognitive…

  6. Mediastinal mixed germ cell tumor in an infertile male with Klinefelter syndrome:A case report and literature review

    Directory of Open Access Journals (Sweden)

    Dinesh Pradhan

    2015-01-01

    Full Text Available Klinefelter syndrome (KS is a well-documented abnormality of the sex chromosome, with an incidence of 1 in 600 newborn males. It is characterized by a 47, XXY or a mosaic karyotype, hypergonadotrophic hypogonadism, infertility, reduced body hair, gynecomastia, and tall stature. Different neoplasms such as breast, testicular, and lymphoreticular malignancies may occur in 1% to2% of the cases with KS. Herein we describe a case of mediastinal mixed germ cell tumor (GCT in a 40-year-old male with KS. Interestingly, this case also had mitral valve prolapse, and an incidental papillary microcarcinoma of the thyroid gland. In view of the presence of pulmonary nodules, antemortem differential diagnoses considered were mycobacterial infection, lymphoma, thymic carcinoma, and a primary/metastatic neoplasm of the lung. As GCT was not considered, the serum markers of a GCT were not performed. The diagnosis of this rare mediastinal mixed GCT with KS was made at autopsy.

  7. Extended Station Blackout Analyses of an APR1400 with MARS-KS

    International Nuclear Information System (INIS)

    Kim, WoongBae; Jang, HyungWook; Oh, Seungjong; Lee, Sangyong

    2016-01-01

    The Fukushima Dai-ichi nuclear power plant accident shows that natural disasters such as earthquakes and the subsequent tsunamis can cause station blackout for several days. The electricity required for essential systems during a station blackout is provided from the emergency backup batteries installed at the nuclear power plant. In South Korea, in the event of an extended station blackout, the life of these emergency backup batteries has recently been extended from 8 hours to 24 hours at Shin-Kori 5, 6 and APR1400 for design certification. For a battery life of 24 hours, available safety means system, equipment and procedures are studied and analyzed in their ability to cope with an extended station blackout. A sensitivity study of reactor coolant pump seal leakage is performed to verify how different seal leakages could affect the system. For simulating of extended station blackout scenarios, the best estimate MARS-KS was used. In this paper, an APR1400 RELAP5 input deck was developed for station blackout scenario to analyze operation strategy by manually depressurizing the reactor coolant system through the steam generator's secondary side. Additionally, a sensitivity study was performed on reactor coolant pump seal leakage

  8. K.S. Maniam, Jhumpa Lahiri, Shirley Lim: A Reflection of Culture and Identity

    Directory of Open Access Journals (Sweden)

    Hardev Kaur Jujar Singh

    2012-07-01

    Full Text Available “I do not want my house to be walled in on all sides and my windows to be stuffed. I want the culture of all lands to be blown about my house as freely as possible. But I refuse to be blown off my feet by any”—Mahatma Gandhi. With these sayings by Gandhiji, one will be able to understand why, even in a borderless world where the diffusion of races and culture happens all the time, and many would simply accept without restraint, the cultures and identity of their adopted land, there remain some writers who, despite being part of a new land, are still deeply influenced by their motherland and various aspects of life that are distinctive and peculiar to their motherland. The writers concerned in this paper are K.S Maniam, Shirley Lim and Jhumpa Lahiri. All these writers have nationalities not of their motherland, but somehow, their writings are usually immersed with the thoughts and culture of their motherland. In this study, we will examine the strong influences imbedded in these writers of the culture of their motherland despite being in their new land. We will also portray how some of the characters assimilate in their new land, whereas some still have a sense of belonging towards their motherland.

  9. A Preliminary Analysis for SMART-ITL SBLOCA Tests using the MARS/KS Code

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Yeon Sik; Ko, Yung Joo; Suh, Jae Seung [System Engineering and Technology Co., Ltd., Daejeon (Korea, Republic of)

    2013-10-15

    In this paper, a preliminary analysis was conducted for SMART-ITL SBLOCA tests using the MARS/KS Code. The results of this work are expected to be good guidelines for SBLOCA tests with the SMART-ITL, and used to understand the various thermal-hydraulic phenomena expected to occur in the integral-type reactor, SMART. An integral-effect test (IET) loop for SMART, SMART-ITL (or FESTA), has been designed using a volume scaling methodology. It was installed at KAERI and its commissioning tests were finished in 2012. Its height was preserved and its area and volume were scaled down to 1/49 compared with the prototype plant, SMART. The SMART-ITL consists of a primary system including a reactor pressure vessel with a pressurizer, four steam generators and four main coolant pumps, a secondary system, a safety system, and an auxiliary system. The objectives of IET using the SMART-ITL facility are to investigate the integral performance of the inter-connected components and possible thermal-hydraulic phenomena occurring in the SMART design, and to validate its safety for various design basis events (DBAs)

  10. Development of in-core measurements in the reactor KS-150

    International Nuclear Information System (INIS)

    Rana, S.B.

    1977-01-01

    Mapping of the neutron flux density distribution and of the neutron fluence distribution in the KS-150 reactor core was carried out using an in-core measuring system. The system allows the in-service monitoring of important operating properties of the reactor core and fuel elements and consists of a mapping fuel element assembly with built-in SPN detectors, of transmission paths and a computer facility. The measurement of the neutron flux, neutron fluence and temperature fields in the reactor core was carried out during the power start-up of the reactor using self-powered DPZ-1 detectors. The obtained data are given and the axial distribution of neutron flux is graphically represented for different values of burnup at the same configuration of regulating rods, as is the axial distribution of neutron fluence for different configurations of the regulating rods during operation, and the in-service neutron fluence distribution. The maximal fuel temperature of 500.2 degC was found at a distance of 291.2 cm from the upper boundary of the reactor core, at a neutron flux of 1.46x10 14 n/cm 2 s. In comparison with other methods, this method proved easy and quick, the results reliable, reactivity perturbance negligible and the fuel element cost increase a negligible 4%. Neutron flux mapping using in-core self-powered detectors will be performed on a wider scale. (J.P./J.O.)

  11. Biochemical Characterization of a Thiol-Activated, Oxidation Stable Keratinase from Bacillus pumilus KS12

    Directory of Open Access Journals (Sweden)

    Rinky Rajput

    2010-01-01

    Full Text Available An extracellular keratinase from Bacillus pumilus KS12 was purified by DEAE ion exchange chromatography. It was a 45 kDa monomer as determined by SDS PAGE analysis. It was found to be an alkaline, serine protease with pH and temperature optima of 10 and 60C, respectively. It was thiol activated with two- and eight-fold enhancement in presence of 10 mM DTT and β-mercaptoethanol, respectively. In addition, its activity was stimulated in the presence of various surfactants, detergents, and oxidizing agents where a nearly 2- to 3-fold enhancement was observed in presence of H2O2 and NaHClO3. It hydrolyzed broad range of complex substrates including feather keratin, haemoglobin, fibrin, casein,and α-keratin. Analysis of amidolytic activity revealed that it efficiently cleaved phenylalanine → leucine → alanine- p-nitroanilides. It also cleaved insulin B chain between Val2- Asn3, Leu6-Cys7 and His10-Leu11 residues.

  12. Extended station blackout analyses of an APR1400 with MARS-KS

    Directory of Open Access Journals (Sweden)

    Kim Woongbae

    2016-01-01

    Full Text Available The Fukushima Daiichi nuclear power plant accident shows that natural disasters such as earthquakes and the subsequent tsunamis can cause station blackout for several days. The electric energy required for essential systems during a station blackout is provided from emergency backup batteries installed at the nuclear power plant. In South Korea, in the event of an extended station blackout, the life of these emergency backup batteries has recently been extended from 8 hours to 24 hours at Shin-Kori 5, 6, and APR1400 for design certification. For a battery life of 24 hours, available safety means system, equipment and procedures are studied and analyzed in their ability to cope with an extended station blackout. A sensitivity study of reactor coolant pump seal leakage is performed to verify how different seal leakages could affect the system. For simulating extended station blackout scenarios, the best estimate MARS-KS computer code was used. In this paper, an APR1400 RELAP5 input deck was developed for station blackout scenario to analyze operation strategy by manually depressurizing the reactor coolant system through the steam generator's secondary side. Additionally, a sensitivity study on reactor coolant pump seal leakage was carried out.

  13. Extended Station Blackout Analyses of an APR1400 with MARS-KS

    Energy Technology Data Exchange (ETDEWEB)

    Kim, WoongBae; Jang, HyungWook; Oh, Seungjong; Lee, Sangyong [KEPCO International Nuclear Graduate School, Ulsan (Korea, Republic of)

    2016-10-15

    The Fukushima Dai-ichi nuclear power plant accident shows that natural disasters such as earthquakes and the subsequent tsunamis can cause station blackout for several days. The electricity required for essential systems during a station blackout is provided from the emergency backup batteries installed at the nuclear power plant. In South Korea, in the event of an extended station blackout, the life of these emergency backup batteries has recently been extended from 8 hours to 24 hours at Shin-Kori 5, 6 and APR1400 for design certification. For a battery life of 24 hours, available safety means system, equipment and procedures are studied and analyzed in their ability to cope with an extended station blackout. A sensitivity study of reactor coolant pump seal leakage is performed to verify how different seal leakages could affect the system. For simulating of extended station blackout scenarios, the best estimate MARS-KS was used. In this paper, an APR1400 RELAP5 input deck was developed for station blackout scenario to analyze operation strategy by manually depressurizing the reactor coolant system through the steam generator's secondary side. Additionally, a sensitivity study was performed on reactor coolant pump seal leakage.

  14. A Preliminary Analysis for SMART-ITL SBLOCA Tests using the MARS/KS Code

    International Nuclear Information System (INIS)

    Cho, Yeon Sik; Ko, Yung Joo; Suh, Jae Seung

    2013-01-01

    In this paper, a preliminary analysis was conducted for SMART-ITL SBLOCA tests using the MARS/KS Code. The results of this work are expected to be good guidelines for SBLOCA tests with the SMART-ITL, and used to understand the various thermal-hydraulic phenomena expected to occur in the integral-type reactor, SMART. An integral-effect test (IET) loop for SMART, SMART-ITL (or FESTA), has been designed using a volume scaling methodology. It was installed at KAERI and its commissioning tests were finished in 2012. Its height was preserved and its area and volume were scaled down to 1/49 compared with the prototype plant, SMART. The SMART-ITL consists of a primary system including a reactor pressure vessel with a pressurizer, four steam generators and four main coolant pumps, a secondary system, a safety system, and an auxiliary system. The objectives of IET using the SMART-ITL facility are to investigate the integral performance of the inter-connected components and possible thermal-hydraulic phenomena occurring in the SMART design, and to validate its safety for various design basis events (DBAs)

  15. Serotonin syndrome

    Science.gov (United States)

    Hyperserotonemia; Serotonergic syndrome; Serotonin toxicity; SSRI - serotonin syndrome; MAO - serotonin syndrome ... brain area. For example, you can develop this syndrome if you take migraine medicines called triptans together ...

  16. Translational rodent models of Korsakoff syndrome reveal the critical neuroanatomical substrates of memory dysfunction and recovery.

    Science.gov (United States)

    Savage, Lisa M; Hall, Joseph M; Resende, Leticia S

    2012-06-01

    Investigation of the amnesic disorder Korsakoff Syndrome (KS) has been vital in elucidating the critical brain regions involved in learning and memory. Although the thalamus and mammillary bodies are the primary sites of neuropathology in KS, functional deactivation of the hippocampus and certain cortical regions also contributes to the chronic cognitive dysfunction reported in KS. The rodent pyrithiamine-induced thiamine deficiency (PTD) model has been used to study the extent of hippocampal and cortical neuroadaptations in KS. In the PTD model, the hippocampus, frontal and retrosplenial cortical regions display loss of cholinergic innervation, decreases in behaviorally stimulated acetylcholine release and reductions in neurotrophins. While PTD treatment results in significant impairment in measures of spatial learning and memory, other cognitive processes are left intact and may be recruited to improve cognitive outcome. In addition, behavioral recovery can be stimulated in the PTD model by increasing acetylcholine levels in the medial septum, hippocampus and frontal cortex, but not in the retrosplenial cortex. These data indicate that although the hippocampus and frontal cortex are involved in the pathogenesis of KS, these regions retain neuroplasticity and may be critical targets for improving cognitive outcome in KS.

  17. Search for the CP-Violating Decays Υ(4S)→B0B¯0→J/ψKS0+J/ψ(ηc)KS0

    Science.gov (United States)

    Tajima, O.; Hazumi, M.; Adachi, I.; Aihara, H.; Aulchenko, V.; Aushev, T.; Bakich, A. M.; Barberio, E.; Bay, A.; Bedny, I.; Bhardwaj, V.; Bitenc, U.; Bozek, A.; Bračko, M.; Browder, T. E.; Chang, M.-C.; Chang, P.; Chen, A.; Chen, K.-F.; Chen, W. T.; Cheon, B. G.; Chiang, C.-C.; Chistov, R.; Cho, I.-S.; Choi, Y.; Choi, Y. K.; Dalseno, J.; Danilov, M.; Dash, M.; Drutskoy, A.; Eidelman, S.; Epifanov, D.; Go, A.; Gokhroo, G.; Golob, B.; Haba, J.; Hayasaka, K.; Hayashii, H.; Heffernan, D.; Hokuue, T.; Hoshi, Y.; Hou, W.-S.; Hsiung, Y. B.; Hyun, H. J.; Iijima, T.; Ikado, K.; Inami, K.; Ishikawa, A.; Ishino, H.; Itoh, R.; Iwasaki, M.; Iwasaki, Y.; Joshi, N. J.; Kah, D. H.; Kaji, H.; Kang, J. H.; Kataoka, S. U.; Kawai, H.; Kawasaki, T.; Kichimi, H.; Kim, H. J.; Kim, H. O.; Kim, S. K.; Kim, Y. J.; Kinoshita, K.; Korpar, S.; Križan, P.; Krokovny, P.; Kumar, R.; Kuo, C. C.; Kwon, Y.-J.; Lange, J. S.; Lee, J. S.; Lee, M. J.; Lee, S. E.; Lesiak, T.; Li, J.; Lin, S.-W.; Liventsev, D.; Mandl, F.; Marlow, D.; McOnie, S.; Medvedeva, T.; Mitaroff, W.; Miyabayashi, K.; Miyake, H.; Miyata, H.; Mizuk, R.; Mohapatra, D.; Nagasaka, Y.; Nakano, E.; Nakao, M.; Nishida, S.; Nitoh, O.; Noguchi, S.; Nozaki, T.; Ogawa, S.; Ohshima, T.; Okuno, S.; Ozaki, H.; Pakhlov, P.; Pakhlova, G.; Park, C. W.; Park, H.; Pestotnik, R.; Piilonen, L. E.; Sahoo, H.; Sakai, Y.; Schneider, O.; Sekiya, A.; Senyo, K.; Sevior, M. E.; Shapkin, M.; Shen, C. P.; Shibuya, H.; Shiu, J.-G.; Shwartz, B.; Singh, J. B.; Sokolov, A.; Somov, A.; Stanič, S.; Starič, M.; Sumisawa, K.; Sumiyoshi, T.; Takasaki, F.; Tanaka, M.; Taylor, G. N.; Teramoto, Y.; Trabelsi, K.; Uehara, S.; Ueno, K.; Uglov, T.; Unno, Y.; Uno, S.; Urquijo, P.; Usov, Y.; Varner, G.; Varvell, K. E.; Vervink, K.; Villa, S.; Vinokurova, A.; Wang, C. C.; Wang, C. H.; Wang, M.-Z.; Wang, P.; Watanabe, Y.; Wedd, R.; Won, E.; Yabsley, B. D.; Yamaguchi, A.; Yamashita, Y.; Yamauchi, M.; Yuan, C. Z.; Yusa, Y.; Zhang, C. C.; Zhang, Z. P.; Zhilich, V.; Zhulanov, V.; Zupanc, A.

    2007-11-01

    We report the first search for CP-violating decays of the Υ(4S) using a data sample that contains 535×106 Υ(4S) mesons with the Belle detector at the KEKB asymmetric-energy e+e- collider. A partial reconstruction technique is employed to enhance the signal sensitivity. No significant signals were observed. We obtain an upper limit of 4×10-7 at the 90% confidence level for the branching fractions of the CP violating modes, Υ(4S)→B0B¯0→J/ψKS0+J/ψ(ηc)KS0. Extrapolating the result, we find that an observation with 5σ significance is expected with a 30ab-1 data sample, which is within the reach of a future super B factory.

  18. Distinctive pattern of expression of spermatogenic molecular markers in testes of azoospermic men with non-mosaic Klinefelter syndrome.

    Science.gov (United States)

    Kleiman, Sandra E; Yogev, Leah; Lehavi, Ofer; Yavetz, Haim; Hauser, Ron

    2016-06-01

    Mature sperm cells can be found in testicular specimens extracted from azoospermic men with non-mosaic Klinefelter syndrome (KS). The present study evaluates the expression of various known molecular markers of spermatogenesis in a population of men with KS and assesses the ability of those markers to predict spermatogenesis. Two groups of men with non-obstructive azoospermia who underwent testicular sperm-retrieval procedures were included in the study: 31 had non-mosaic KS (KS group) and 91 had normal karyotype (NK group). Each group was subdivided into mixed atrophy (containing some mature sperm cells) or Sertoli cell only syndrome according to testicular histology and cytology observations. Semi-quantitative histological morphometric analysis (interstitial hyperplasia and hyalinization, tubules with cells and abnormal thickness of the basement membrane) and expression of spermatogenetic markers (DAZ, RBM, BOLL, and CDY1) were evaluated and compared among those subgroups. Clear differences in the histological morphometry and spermatogenetic marker expression were noted between the KS and NK groups. There was a significant difference in the expression of spermatogenetic markers between the subgroups of the NK group (as expected), while no difference could be discerned between the two subgroups in the KS group. We conclude that molecular spermatogenetic markers have a pattern of expression in men with KS that is distinctively different from that of men with NK, and that it precludes and limits their use for predicting spermatogenesis in the former. It is suggested that this difference might be due to the specific highly abnormal histological morphometric parameters in KS specimens.

  19. Dysregulation of X-Linked Gene Expression in Klinefelter’s Syndrome and Association With Verbal Cognition

    Science.gov (United States)

    Vawter, Marquis P.; Harvey, Philip D.; DeLisi, Lynn E.

    2007-01-01

    Klinefelter’s Syndrome (KS) is a chromosomal karyotype with one or more extra X chromosomes. KS individuals often show language impairment and the phenotype might be due to overexpression of genes on the extra X chromosome(s). We profiled mRNA derived from lymphoblastoid cell lines from males with documented KS and control males using the Affymetrix U133P microarray platform. There were 129 differentially expressed genes (DEGs) in KS group compared with controls after Benjamini–Hochberg false discovery adjustment. The DEGs included 14 X chromosome genes which were significantly over-represented. The Y chromosome had zero DEGs. In exploratory analysis of gene expression–cognition relationships, 12 DEGs showed significant correlation of expression with measures of verbal cognition in KS. Overexpression of one pseudoautosomal gene, GTPBP6 (GTP binding protein 6, putative) was inversely correlated with verbal IQ (r = −0.86, P < 0.001) and four other measures of verbal ability. Overexpression of XIST was found in KS compared to XY controls suggesting that silencing of many genes on the X chromosome might occur in KS similar to XX females. The microarray findings for eight DEGs were validated by quantitative PCR. The 14 X chromosome DEGs were not differentially expressed in prior studies comparing female and male brains suggesting a dysregulation profile unique to KS. Examination of X-linked DEGs, such as GTPBP6, TAF9L, and CXORF21, that show verbal cognition–gene expression correlations may establish a causal link between these genes, neurodevelopment, and language function. A screen of candidate genes may serve as biomarkers of KS for early diagnosis. PMID:17347996

  20. Gorlin Syndrome with Bilateral Polydactyly: A Rare Case Report

    Science.gov (United States)

    Acharya, Sonu; Panda, Swagatika; Sahoo, Sujit Ranjan; Ray, Prayas

    2013-01-01

    ABSTRACT Gorlin's syndrome is a rare disorder transmitted as an autosomal dominant trait. It is characterized by multiple disorders involving multiple systems. We present a case of 11-year-old male child presenting with multiple odontogenic keratocyst to the dental clinic. Retrograde diagnosis of Gorlin-Goltz syndrome was made after clinical and radiological investigation. How to cite this article: Acharya S, Panda S, Dhull KS, Sahoo SR, Ray P. Gorlin Syndrome with Bilateral Polydactyly: A Rare Case Report. Int J Clin Pediatr Dent 2013;6(3):208-212. PMID:25206225

  1. Observation of B_{s}^{0}→D[over ¯]^{0}K_{S}^{0} and Evidence for B_{s}^{0}→D[over ¯]^{*}^{0}K_{S}^{0} Decays.

    Science.gov (United States)

    Aaij, R; Abellán Beteta, C; Adeva, B; Adinolfi, M; Affolder, A; Ajaltouni, Z; Akar, S; Albrecht, J; Alessio, F; Alexander, M; Ali, S; Alkhazov, G; Alvarez Cartelle, P; Alves, A A; Amato, S; Amerio, S; Amhis, Y; An, L; Anderlini, L; Andreassi, G; Andreotti, M; Andrews, J E; Appleby, R B; Aquines Gutierrez, O; Archilli, F; d'Argent, P; Artamonov, A; Artuso, M; Aslanides, E; Auriemma, G; Baalouch, M; Bachmann, S; Back, J J; Badalov, A; Baesso, C; Baldini, W; Barlow, R J; Barschel, C; Barsuk, S; Barter, W; Batozskaya, V; Battista, V; Bay, A; Beaucourt, L; Beddow, J; Bedeschi, F; Bediaga, I; Bel, L J; Bellee, V; Belloli, N; Belyaev, I; Ben-Haim, E; Bencivenni, G; Benson, S; Benton, J; Berezhnoy, A; Bernet, R; Bertolin, A; Bettler, M-O; van Beuzekom, M; Bifani, S; Billoir, P; Bird, T; Birnkraut, A; Bizzeti, A; Blake, T; Blanc, F; Blouw, J; Blusk, S; Bocci, V; Bondar, A; Bondar, N; Bonivento, W; Borghi, S; Borisyak, M; Borsato, M; Bowcock, T J V; Bowen, E; Bozzi, C; Braun, S; Britsch, M; Britton, T; Brodzicka, J; Brook, N H; Buchanan, E; Burr, C; Bursche, A; Buytaert, J; Cadeddu, S; Calabrese, R; Calvi, M; Calvo Gomez, M; Campana, P; Campora Perez, D; Capriotti, L; Carbone, A; Carboni, G; Cardinale, R; Cardini, A; Carniti, P; Carson, L; Carvalho Akiba, K; Casse, G; Cassina, L; Castillo Garcia, L; Cattaneo, M; Cauet, Ch; Cavallero, G; Cenci, R; Charles, M; Charpentier, Ph; Chatzikonstantinidis, G; Chefdeville, M; Chen, S; Cheung, S-F; Chiapolini, N; Chrzaszcz, M; Cid Vidal, X; Ciezarek, G; Clarke, P E L; Clemencic, M; Cliff, H V; Closier, J; Coco, V; Cogan, J; Cogneras, E; Cogoni, V; Cojocariu, L; Collazuol, G; Collins, P; Comerma-Montells, A; Contu, A; Cook, A; Coombes, M; Coquereau, S; Corti, G; Corvo, M; Couturier, B; Cowan, G A; Craik, D C; Crocombe, A; Cruz Torres, M; Cunliffe, S; Currie, R; D'Ambrosio, C; Dall'Occo, E; Dalseno, J; David, P N Y; Davis, A; De Aguiar Francisco, O; De Bruyn, K; De Capua, S; De Cian, M; De Miranda, J M; De Paula, L; De Simone, P; Dean, C-T; Decamp, D; Deckenhoff, M; Del Buono, L; Déléage, N; Demmer, M; Derkach, D; Deschamps, O; Dettori, F; Dey, B; Di Canto, A; Di Ruscio, F; Dijkstra, H; Donleavy, S; Dordei, F; Dorigo, M; Dosil Suárez, A; Dovbnya, A; Dreimanis, K; Dufour, L; Dujany, G; Dungs, K; Durante, P; Dzhelyadin, R; Dziurda, A; Dzyuba, A; Easo, S; Egede, U; Egorychev, V; Eidelman, S; Eisenhardt, S; Eitschberger, U; Ekelhof, R; Eklund, L; El Rifai, I; Elsasser, Ch; Ely, S; Esen, S; Evans, H M; Evans, T; Falabella, A; Färber, C; Farley, N; Farry, S; Fay, R; Ferguson, D; Fernandez Albor, V; Ferrari, F; Ferreira Rodrigues, F; Ferro-Luzzi, M; Filippov, S; Fiore, M; Fiorini, M; Firlej, M; Fitzpatrick, C; Fiutowski, T; Fleuret, F; Fohl, K; Fol, P; Fontana, M; Fontanelli, F; Forshaw, D C; Forty, R; Frank, M; Frei, C; Frosini, M; Fu, J; Furfaro, E; Gallas Torreira, A; Galli, D; Gallorini, S; Gambetta, S; Gandelman, M; Gandini, P; Gao, Y; García Pardiñas, J; Garra Tico, J; Garrido, L; Gascon, D; Gaspar, C; Gauld, R; Gavardi, L; Gazzoni, G; Gerick, D; Gersabeck, E; Gersabeck, M; Gershon, T; Ghez, Ph; Gianì, S; Gibson, V; Girard, O G; Giubega, L; Gligorov, V V; Göbel, C; Golubkov, D; Golutvin, A; Gomes, A; Gotti, C; Grabalosa Gándara, M; Graciani Diaz, R; Granado Cardoso, L A; Graugés, E; Graverini, E; Graziani, G; Grecu, A; Greening, E; Griffith, P; Grillo, L; Grünberg, O; Gui, B; Gushchin, E; Guz, Yu; Gys, T; Hadavizadeh, T; Hadjivasiliou, C; Haefeli, G; Haen, C; Haines, S C; Hall, S; Hamilton, B; Han, X; Hansmann-Menzemer, S; Harnew, N; Harnew, S T; Harrison, J; He, J; Head, T; Heijne, V; Heister, A; Hennessy, K; Henrard, P; Henry, L; Hernando Morata, J A; van Herwijnen, E; Heß, M; Hicheur, A; Hill, D; Hoballah, M; Hombach, C; Hulsbergen, W; Humair, T; Hushchyn, M; Hussain, N; Hutchcroft, D; Hynds, D; Idzik, M; Ilten, P; Jacobsson, R; Jaeger, A; Jalocha, J; Jans, E; Jawahery, A; John, M; Johnson, D; Jones, C R; Joram, C; Jost, B; Jurik, N; Kandybei, S; Kanso, W; Karacson, M; Karbach, T M; Karodia, S; Kecke, M; Kelsey, M; Kenyon, I R; Kenzie, M; Ketel, T; Khairullin, E; Khanji, B; Khurewathanakul, C; Kirn, T; Klaver, S; Klimaszewski, K; Kochebina, O; Kolpin, M; Komarov, I; Koopman, R F; Koppenburg, P; Kozeiha, M; Kravchuk, L; Kreplin, K; Kreps, M; Krokovny, P; Kruse, F; Krzemien, W; Kucewicz, W; Kucharczyk, M; Kudryavtsev, V; Kuonen, A K; Kurek, K; Kvaratskheliya, T; Lacarrere, D; Lafferty, G; Lai, A; Lambert, D; Lanfranchi, G; Langenbruch, C; Langhans, B; Latham, T; Lazzeroni, C; Le Gac, R; van Leerdam, J; Lees, J-P; Lefèvre, R; Leflat, A; Lefrançois, J; Lemos Cid, E; Leroy, O; Lesiak, T; Leverington, B; Li, Y; Likhomanenko, T; Liles, M; Lindner, R; Linn, C; Lionetto, F; Liu, B; Liu, X; Loh, D; Longstaff, I; Lopes, J H; Lucchesi, D; Lucio Martinez, M; Luo, H; Lupato, A; Luppi, E; Lupton, O; Lusardi, N; Lusiani, A; Machefert, F; Maciuc, F; Maev, O; Maguire, K; Malde, S; Malinin, A; Manca, G; Mancinelli, G; Manning, P; Mapelli, A; Maratas, J; Marchand, J F; Marconi, U; Marin Benito, C; Marino, P; Marks, J; Martellotti, G; Martin, M; Martinelli, M; Martinez Santos, D; Martinez Vidal, F; Martins Tostes, D; Massacrier, L M; Massafferri, A; Matev, R; Mathad, A; Mathe, Z; Matteuzzi, C; Mauri, A; Maurin, B; Mazurov, A; McCann, M; McCarthy, J; McNab, A; McNulty, R; Meadows, B; Meier, F; Meissner, M; Melnychuk, D; Merk, M; Michielin, E; Milanes, D A; Minard, M-N; Mitzel, D S; Molina Rodriguez, J; Monroy, I A; Monteil, S; Morandin, M; Morawski, P; Mordà, A; Morello, M J; Moron, J; Morris, A B; Mountain, R; Muheim, F; Müller, D; Müller, J; Müller, K; Müller, V; Mussini, M; Muster, B; Naik, P; Nakada, T; Nandakumar, R; Nandi, A; Nasteva, I; Needham, M; Neri, N; Neubert, S; Neufeld, N; Neuner, M; Nguyen, A D; Nguyen, T D; Nguyen-Mau, C; Niess, V; Niet, R; Nikitin, N; Nikodem, T; Novoselov, A; O'Hanlon, D P; Oblakowska-Mucha, A; Obraztsov, V; Ogilvy, S; Okhrimenko, O; Oldeman, R; Onderwater, C J G; Osorio Rodrigues, B; Otalora Goicochea, J M; Otto, A; Owen, P; Oyanguren, A; Palano, A; Palombo, F; Palutan, M; Panman, J; Papanestis, A; Pappagallo, M; Pappalardo, L L; Pappenheimer, C; Parker, W; Parkes, C; Passaleva, G; Patel, G D; Patel, M; Patrignani, C; Pearce, A; Pellegrino, A; Penso, G; Pepe Altarelli, M; Perazzini, S; Perret, P; Pescatore, L; Petridis, K; Petrolini, A; Petruzzo, M; Picatoste Olloqui, E; Pietrzyk, B; Pikies, M; Pinci, D; Pistone, A; Piucci, A; Playfer, S; Plo Casasus, M; Poikela, T; Polci, F; Poluektov, A; Polyakov, I; Polycarpo, E; Popov, A; Popov, D; Popovici, B; Potterat, C; Price, E; Price, J D; Prisciandaro, J; Pritchard, A; Prouve, C; Pugatch, V; Puig Navarro, A; Punzi, G; Qian, W; Quagliani, R; Rachwal, B; Rademacker, J H; Rama, M; Ramos Pernas, M; Rangel, M S; Raniuk, I; Rauschmayr, N; Raven, G; Redi, F; Reichert, S; Dos Reis, A C; Renaudin, V; Ricciardi, S; Richards, S; Rihl, M; Rinnert, K; Rives Molina, V; Robbe, P; Rodrigues, A B; Rodrigues, E; Rodriguez Lopez, J A; Rodriguez Perez, P; Roiser, S; Romanovsky, V; Romero Vidal, A; Ronayne, J W; Rotondo, M; Ruf, T; Ruiz Valls, P; Saborido Silva, J J; Sagidova, N; Saitta, B; Salustino Guimaraes, V; Sanchez Mayordomo, C; Sanmartin Sedes, B; Santacesaria, R; Santamarina Rios, C; Santimaria, M; Santovetti, E; Sarti, A; Satriano, C; Satta, A; Saunders, D M; Savrina, D; Schael, S; Schiller, M; Schindler, H; Schlupp, M; Schmelling, M; Schmelzer, T; Schmidt, B; Schneider, O; Schopper, A; Schubiger, M; Schune, M-H; Schwemmer, R; Sciascia, B; Sciubba, A; Semennikov, A; Serra, N; Serrano, J; Sestini, L; Seyfert, P; Shapkin, M; Shapoval, I; Shcheglov, Y; Shears, T; Shekhtman, L; Shevchenko, V; Shires, A; Siddi, B G; Silva Coutinho, R; Silva de Oliveira, L; Simi, G; Sirendi, M; Skidmore, N; Skwarnicki, T; Smith, E; Smith, E; Smith, I T; Smith, J; Smith, M; Snoek, H; Sokoloff, M D; Soler, F J P; Soomro, F; Souza, D; Souza De Paula, B; Spaan, B; Spradlin, P; Sridharan, S; Stagni, F; Stahl, M; Stahl, S; Stefkova, S; Steinkamp, O; Stenyakin, O; Stevenson, S; Stoica, S; Stone, S; Storaci, B; Stracka, S; Straticiuc, M; Straumann, U; Sun, L; Sutcliffe, W; Swientek, K; Swientek, S; Syropoulos, V; Szczekowski, M; Szumlak, T; T'Jampens, S; Tayduganov, A; Tekampe, T; Tellarini, G; Teubert, F; Thomas, C; Thomas, E; van Tilburg, J; Tisserand, V; Tobin, M; Todd, J; Tolk, S; Tomassetti, L; Tonelli, D; Topp-Joergensen, S; Torr, N; Tournefier, E; Tourneur, S; Trabelsi, K; Traill, M; Tran, M T; Tresch, M; Trisovic, A; Tsaregorodtsev, A; Tsopelas, P; Tuning, N; Ukleja, A; Ustyuzhanin, A; Uwer, U; Vacca, C; Vagnoni, V; Valenti, G; Vallier, A; Vazquez Gomez, R; Vazquez Regueiro, P; Vázquez Sierra, C; Vecchi, S; van Veghel, M; Velthuis, J J; Veltri, M; Veneziano, G; Vesterinen, M; Viaud, B; Vieira, D; Vieites Diaz, M; Vilasis-Cardona, X; Volkov, V; Vollhardt, A; Voong, D; Vorobyev, A; Vorobyev, V; Voß, C; de Vries, J A; Waldi, R; Wallace, C; Wallace, R; Walsh, J; Wang, J; Ward, D R; Watson, N K; Websdale, D; Weiden, A; Whitehead, M; Wicht, J; Wilkinson, G; Wilkinson, M; Williams, M; Williams, M P; Williams, M; Williams, T; Wilson, F F; Wimberley, J; Wishahi, J; Wislicki, W; Witek, M; Wormser, G; Wotton, S A; Wraight, K; Wright, S; Wyllie, K; Xie, Y; Xu, Z; Yang, Z; Yu, J; Yuan, X; Yushchenko, O; Zangoli, M; Zavertyaev, M; Zhang, L; Zhang, Y; Zhelezov, A; Zhokhov, A; Zhong, L; Zhukov, V; Zucchelli, S

    2016-04-22

    The first observation of the B_{s}^{0}→D[over ¯]^{0}K_{S}^{0} decay mode and evidence for the B_{s}^{0}→D[over ¯]^{*0}K_{S}^{0} decay mode are reported. The data sample corresponds to an integrated luminosity of 3.0  fb^{-1} collected in pp collisions by LHCb at center-of-mass energies of 7 and 8 TeV. The branching fractions are measured to be B(B_{s}^{0}→D[over ¯]^{0}K[over ¯]^{0})=[4.3±0.5(stat)±0.3(syst)±0.3(frag)±0.6(norm)]×10^{-4},B(B_{s}^{0}→D[over ¯]^{*0}K[over ¯]^{0})=[2.8±1.0(stat)±0.3(syst)±0.2(frag)±0.4(norm)]×10^{-4},where the uncertainties are due to contributions coming from statistical precision, systematic effects, and the precision of two external inputs, the ratio f_{s}/f_{d} and the branching fraction of B^{0}→D[over ¯]^{0}K_{S}^{0}, which is used as a calibration channel.

  2. When does germ cell loss and fibrosis occur in patients with Klinefelter syndrome?

    Science.gov (United States)

    Van Saen, D; Vloeberghs, V; Gies, I; Mateizel, I; Sermon, K; De Schepper, Jean; Tournaye, H; Goossens, E

    2018-06-01

    When does germ cell loss and fibrosis occur in patients with Klinefelter syndrome (KS)? In KS, germ cell loss is not observed in testicular tissue from fetuses in the second semester of pregnancy but present at a prepubertal age when the testicular architecture is still normal, while fibrosis is highly present at an adolescent age. Most KS patients are azoospermic at adult age because of a massive germ cell loss. However, the timing when this germ cell loss starts is not known. It is assumed that germ cell loss increases at puberty. Therefore, testicular sperm extraction (TESE) at an adolescent age has been suggested to increase the chances of sperm retrieval at onset of spermatogenesis. However, recent data indicate that testicular biopsies from peripubertal KS patients contain only a few germ cells. In this study, we give an update on fertility preservation in adolescent KS patients and evaluate whether fertility preservation would be beneficial at prepubertal age. The possibility of retrieving testicular spermatozoa by TESE was evaluated in adolescent and adult KS men. The presence of spermatogonia and the degree of fibrosis were also analysed in testicular biopsies from KS patients at different ages. The patients were divided into four age groups: foetal (n = 5), prepubertal (aged 4-7 years; n = 4), peripubertal (aged 12-16 years; n = 20) and adult (aged 18-41 years; n = 27) KS patients. In peripubertal and adult KS patients, retrieval of spermatozoa was attempted by semen analysis after masturbation, vibrostimulation, electroejaculation or by TESE. MAGE-A4 immunohistochemistry was performed to evaluate the presence of germ cells in testicular biopsies from foetal, prepubertal, peripubertal and adult KS patients. Tissue morphology was evaluated by haematoxylin-periodic acid Schiff (H/PAS) staining. Testicular spermatozoa were collected by TESE in 48.1% of the adult KS patients, while spermatozoa were recovered after TESE in only one peripubertal patient (5

  3. Genotype and phenotype in Klinefelter syndrome - impact of androgen receptor polymorphism and skewed X inactivation

    DEFF Research Database (Denmark)

    Bojesen, A; Hertz, J M; Gravholt, C H

    2011-01-01

    The phenotypic variation of Klinefelter syndrome (KS) is wide and may by caused by various genetic and epigenetic effects. Skewed inactivation of the supra-numerical X chromosome and polymorphism in the androgen receptor (AR) have been suggested as plausible causes. We wanted to describe X...

  4. Reconstructing cognition in Korsakoff’s syndrome : Diagnosis, residual capacities, and rehabilitation

    NARCIS (Netherlands)

    Oudman, F.A.

    2016-01-01

    Korsakoff's syndrome (KS) is a preventable neuropsychiatric disorder caused by alcoholism induced vitamine B1 deficiency. In the acute phase of this vitamine B1 deficiency, called Wernicke’s encephalopathy (WE), the neuropsychiatric disorder is fully reversibly by fast intravenous or intramuscular

  5. Observation of the CP-conserving $K_{S} \\rightarrow \\pi^{+}\\pi^{- }\\pi^{0}$ decay amplitude

    CERN Document Server

    Adler, R; Angelopoulos, Angelos; Aspostolakis, A; Aslanides, Elie; Backenstoss, Gerhard; Bee, C P; Behnke, O; Benelli, A; Bennet, J; Bertin, V; Bienlein, J K; Blanc, F; Bloch, P; Bula, C; Carlson, P J; Carroll, M; Carvalho, J; Cawley, E; Charalambous, S; Chardin, G; Chertok, M B; Cody, A; Danielsson, M; Dejardin, M; Derré, J; Dodgson, M; Duclos, J; Ealet, A; Eckart, B; Eleftheriadis, C; Evangelou, I; Faravel, L; Fassnacht, P; Faure, J L; Felder, C; Ferreira-Marques, R; Fetscher, W; Fidecaro, Maria; Filipcic, A; Francis, D; Fry, J; Fuglesang, C; Gabathuler, Erwin; Gamet, R; Garreta, D; Geralis, T; Gerber, H J; Go, A; Gumplinger, P; Guyot, C; Harrison, P F; Haselden, A; Hayman, P J; Henry-Coüannier, F; Heyes, W G; Hollander, R W; Hubert, E; Jansson, K; Johner, H U; Jon-And, K; Kettle, P R; Kochowski, Claude; Kokkas, P; Kreuger, R; Lawry, T; Le Gac, R; Leimgruber, F; Liolios, A; Machado, E; Maley, P; Mandic, I; Manthos, N; Marel, Gérard; Mikuz, M; Miller, J; Montanet, François; Nakada, Tatsuya; Onofre, A; Pagels, B; Papadopoulos, I M; Pavlopoulos, P; Pelucchi, F; Pinto da Cunha, J; Policarpo, Armando; Polivka, G; Postma, H; Rickenbach, R; Roberts, B L; Rozaki, E; Ruf, T; Sacks, L; Sakelliou, L; Sanders, P; Santoni, C; Sarigiannis, K; Schäfer, M; Schaller, L A; Schietinger, T; Schopper, A; Schune, P; Soares, A; Tauscher, Ludwig; Thibault, C; Touchard, F; Touramanis, C; Triantis, F A; Tröster, D A; Van Beveren, E; van Eijk, C W E; Varner, G S; Vlachos, S; Weber, P; Wigger, O; Witzig, C; Wolter, M; Yéche, C; Zavrtanik, D; Zimmerman, D

    1996-01-01

    The interference between CP-conserving $\\ks$ and $\\kl \\rightarrow \\threepi$ decay amplitudes was observed by studying the decay rate asymmetries between initial $\\ko$ and $\\kob$ separately for the phase space regions $E_{\\mbox{\\rm \\scriptsize CM}}(\\pi^+)> E_{\\mbox{\\rm \\scriptsize CM}}(\\pi^-)$ and $E_{\\mbox{\\rm \\scriptsize CM}}(\\pi^+)< E_{\\mbox{\\rm \\scriptsize CM}}(\\pi^-)$. For the parameter $\\lambda$ we found $\\mbox{\\rm Re}(\\lambda )=0.036\\pm0.010(\\mbox{\\rm stat.}) ^{+0.002}_{-0.003} (\\mbox{\\rm syst.)}$ and $\\mbox{Im}(\\lambda)$ consistent with zero, leading for the CP-conserving $\\ks \\rightarrow \\threepi$ decay, to a branching ratio $\\mbox{\\rm B} = \\left[4.1 ^{+2.5}_{-1.9 } (\\mbox{\\rm stat.}) ^{+0.5} _{-0.6} (\\mbox{\\rm syst.)}\\right] \\times 10^{-7}$.

  6. EPR study of gamma and neutron irradiation effects on KU1, KS-4V and Infrasil 301 silica glasses

    International Nuclear Information System (INIS)

    Lagomacini, Juan C.; Bravo, David; Leon, Monica; Martin, Piedad; Ibarra, Angel; Martin, Agustin; Lopez, Fernando J.

    2011-01-01

    Electron paramagnetic resonance (EPR) studies have been carried out on KU1 and KS-4V high purity quartz glasses and commercial silica Infrasil 301, irradiated with gamma rays up to a dose of 11.6 MGy and neutron fluences of 10 21 and 10 22 n/m 2 . Gamma irradiations produce a much higher concentration of defect centres (mainly E', POR and NBOHC) for KU1 and I301 than for KS-4V silica. In contrast, neutron irradiation at the highest fluence produces similar concentrations in all silica types. These results agree to a good extent with those obtained in previous optical absorption measurements. Moreover, oxygen-related centres (POR and NBOHC) have been well characterized by means of electron paramagnetic resonance.

  7. EPR study of gamma and neutron irradiation effects on KU1, KS-4V and Infrasil 301 silica glasses

    Energy Technology Data Exchange (ETDEWEB)

    Lagomacini, Juan C., E-mail: jc.lagomacini@uam.es [Dept. Fisica de Materiales, Universidad Autonoma de Madrid, E-28049 Madrid (Spain); Bravo, David [Dept. Fisica de Materiales, Universidad Autonoma de Madrid, E-28049 Madrid (Spain); Leon, Monica; Martin, Piedad; Ibarra, Angel [Materiales para Fusion, CIEMAT, Avda. Complutense 22, E-28040 Madrid (Spain); Martin, Agustin [Dept. Fisica e Instalaciones, ETS Arquitectura UPM, E-28040 Madrid (Spain); Lopez, Fernando J. [Dept. Fisica de Materiales, Universidad Autonoma de Madrid, E-28049 Madrid (Spain)

    2011-10-01

    Electron paramagnetic resonance (EPR) studies have been carried out on KU1 and KS-4V high purity quartz glasses and commercial silica Infrasil 301, irradiated with gamma rays up to a dose of 11.6 MGy and neutron fluences of 10{sup 21} and 10{sup 22} n/m{sup 2}. Gamma irradiations produce a much higher concentration of defect centres (mainly E', POR and NBOHC) for KU1 and I301 than for KS-4V silica. In contrast, neutron irradiation at the highest fluence produces similar concentrations in all silica types. These results agree to a good extent with those obtained in previous optical absorption measurements. Moreover, oxygen-related centres (POR and NBOHC) have been well characterized by means of electron paramagnetic resonance.

  8. Kino repertuāra pārlūks Android ierīcēm

    OpenAIRE

    Zvirbulis, Jānis

    2013-01-01

    Kvalifikācijas darbā “Kino repertuāra pārlūks Android ierīcēm” tiek aprakstīta Android lietojumprogrammas “Kino repertuāra pārlūks” izstrāde un funkcionalitāte. Lietotne paredzēta kinoteātra repertuāra aplūkošanai izmantojot planšetdatorus un mobilos tālruņus, kas darbojas ar Android operētājsistēmu. Tā ir domāta kā parocīgāka alternatīva filmu apraksta un seansa laiku uzzināšanai caur kinoteātra mājaslapu, skrejlapām vai afišām. Atslēgvārdi: Android, filmas, pārlūks....

  9. Dalitz plot studies of D0 → KS0π+π− decays in a factorization approach

    Directory of Open Access Journals (Sweden)

    Leśniak Leonard

    2014-01-01

    Full Text Available A quasi two-body QCD factorization is used to study the D0 → KS0π+π− decays. The presently available high-statistics Dalitz plot data of this process measured by the Belle and BABAR Collaborations are analyzed together with the τ− → KS0π−ντ decay data. The total experimental branching fraction is also included in the fits which show a very good overall agreement with the experimental Dalitz plot density distributions. The branching fractions of the dominant channels compare well with those of the isobar Belle or BABAR models. We show that the branching fractions corresponding to the annihilation amplitudes are significant.

  10. Convergence of Multiple MAP3Ks on MKK3 Identifies a Set of Novel Stress MAPK Modules

    KAUST Repository

    Colcombet, Jean

    2016-12-22

    Since its first description in 1995 and functional characterization 12 years later, plant MKK3-type MAP2Ks have emerged as important integrators in plant signaling. Although they have received less attention than the canonical stress-activated mitogen-activated protein kinases (MAPKs), several recent publications shed light on their important roles in plant adaptation to environmental conditions. Nevertheless, the MKK3-related literature is complicated. This review summarizes the current knowledge and discrepancies on MKK3 MAPK modules in plants and highlights the singular role of MKK3 in green plants. In the light of the latest data, we hypothesize a general model that all clade-III MAP3Ks converge on MKK3 and C-group MAPKs, thereby defining a set of novel MAPK modules which are activated by stresses and internal signals through the transcriptional regulation of MAP3K genes.

  11. Improved limit on the branching fraction of the rare decay K-S(0) -> mu(+)mu(-)

    NARCIS (Netherlands)

    Aaij, R.; Adeva, B.; Ajaltouni, Z.; Akar, S.; Alessio, F.; Alexander, M.; Ali, S.; Alkhazov, G.; Cartelle, P. Alvarez; Alves, A. A., Jr.; Amato, S.; Amerio, S.; Amhis, Y.; An, L.; Anderlini, L.; Andreassi, G.; Andreotti, M.; Andrews, J. E.; Appleby, R. B.; Archilli, F.; d'Argent, P.; Romeu, J. Arnau; Artamonov, A.; Artuso, M.; Aslanides, E.; Auriemma, G.; Baalouch, M.; Babuschkin, I.; Bachmann, S.; Back, J. J.; Badalov, A.; Baesso, C.; Baker, S.; Balagura, V.; Baldini, W.; Baranov, A.; Barlow, R. J.; Barschel, C.; Barsuk, S.; Barter, W.; Baryshnikov, F.; Baszczyk, M.; Batozskaya, V.; Battista, V.; Bay, A.; Beaucourt, L.; Beddow, J.; Bedeschi, F.; Bediaga, I.; Beiter, A.; Bel, L. J.; Bellee, V.; Belloli, N.; Belous, K.; Belyaev, I.; Ben-Haim, E.; Bencivenni, G.; Benson, S.; Beranek, S.; Berezhnoy, A.; Bernet, R.; Bertolin, A.; Betancourt, C.; Betti, F.; Bettler, M. -O.; van Beuzekom, M.; Bezshyiko, Ia.; Bifani, S.; Billoir, P.; Birnkraut, A.; Bitadze, A.; Bizzeti, A.; Blake, T.; Blanc, F.; Blouw, J.; Blusk, S.; Bocci, V.; Boettcher, T.; Bondar, A.; Bondar, N.; Bonivento, W.; Bordyuzhin, I.; Borgheresi, A.; Borghi, S.; Borisyak, M.; Borsato, M.; Bossu, F.; Boubdir, M.; Bowcock, T. J. V.; Bowen, E.; Bozzi, C.; Britton, T.; Brodzicka, J.; Buchanan, E.; Burr, C.; Bursche, A.; Buytaert, J.; Cadeddu, S.; Calabrese, R.; Calvi, M.; Calvo Gomez, M.; Camboni, A.; Campana, P.; Perez, D. H. Campora; Capriotti, L.; Carbone, A.; Carboni, G.; Cardinale, R.; Cardini, A.; Carniti, P.; Carvalho Akiba, K.; Casse, G.; Cassina, L.; Garcia, L. Castillo; Cattaneo, M.; Cavallero, G.; Cenci, R.; Chamont, D.; Charles, M.; Charpentier, Ph.; Chatzikonstantinidis, G.; Chefdeville, M.; Cheung, S. F.; Chobanova, V.; Chrzaszcz, M.; Chubykin, A.; Cid Vidal, X.; Ciezarek, G.; Clarke, P. E. L.; Clemencic, M.; Cliff, H. V.; Closier, J.; Coco, V.; Cogan, J.; Cogneras, E.; Cogoni, V.; Cojocariu, L.; Collins, P.; Comerma-Montells, A.; Contu, A.; Cook, A.; Coombs, G.; Coquereau, S.; Corti, G.; Corvo, M.; Sobral, C. M. Costa; Couturier, B.; Cowan, G. A.; Craik, D. C.; Crocombe, A.; Cruz Torres, M.; Currie, R.; D'Ambrosio, C.; Da Cunha Marinho, F.; Dall'Occo, E.; Dalseno, J.; Davis, A.; De Bruyn, K.; De Capua, S.; De Cian, M.; De Miranda, J. M.; De Paula, L.; De Serio, M.; De Simone, P.; Dean, C. T.; Decamp, D.; Deckenhoff, M.; Del Buono, L.; Dembinski, H. -P.; Demmer, M.; Dendek, A.; Derkach, D.; Deschamps, O.; Dettori, F.; Dey, B.; Di Canto, A.; Di Nezza, P.; Dordei, F.; Dorigo, M.; Dosil Suarez, A.; Dovbnya, A.; Dreimanis, K.; Dujany, G.; Dungs, K.; Durante, P.; Dzhelyadin, R.; Dziewiecki, M.; Dziurda, A.; Dzyuba, A.; Deleage, N.; Easo, S.; Ebert, M.; Egede, U.; Egorychev, V.; Eidelman, S.; Eisenhardt, S.; Eitschberger, U.; Ekelhof, R.; Eklund, L.; Ely, S.; Esen, S.; Evans, H. M.; Evans, T.; Falabella, A.; Farley, N.; Farry, S.; Fay, R.; Fazzini, D.; Ferguson, D.; Fernandez, G.; Fernandez Prieto, A.; Ferrari, F.; Ferreira Rodrigues, F.; Ferro-Luzzi, M.; Filippov, S.; Fini, R. A.; Fiore, M.; Fiorini, M.; Firlej, M.; Fitzpatrick, C.; Fiutowski, T.; Fleuret, F.; Fohl, K.; Fontana, M.; Fontanelli, F.; Forshaw, D. C.; Forty, R.; Lima, V. Franco; Frank, M.; Frei, C.; Fu, J.; Funk, W.; Furfaro, E.; Farber, C.; Gallas Torreira, A.; Galli, D.; Gallorini, S.; Gambetta, S.; Gandelman, M.; Gandini, P.; Gao, Y.; Garcia Martin, L. M.; Garcia Pardinas, J.; Tico, J. Garra; Garrido, L.; Garsed, P. J.; Gascon, D.; Gaspar, C.; Gavardi, L.; Gazzoni, G.; Gerick, D.; Gersabeck, E.; Gersabeck, M.; Gershon, T.; Ghez, Ph.; Giani, S.; Gibson, V.; Girard, O. G.; Giubega, L.; Gizdov, K.; Gligorov, V. V.; Golubkov, D.; Golutvin, A.; Gomes, A.; Gorelov, I. V.; Gotti, C.; Govorkova, E.; Graciani Diaz, R.; Cardoso, L. A. Granado; Grauges, E.; Graverini, E.; Graziani, G.; Grecu, A.; Greim, R.; Grillo, L.; Cazon, B. R. Gruberg; Gruenberg, O.; Gushchin, E.; Guz, Yu.; Gys, T.; Gobel, C.; Hadavizadeh, T.; Hadjivasiliou, C.; Haefeli, G.; Haen, C.; Haines, S. C.; Hamilton, B.; Han, X.; Hansmann-Menzemer, S.; Harnew, N.; Harnew, S. T.; Harrison, J.; Hatch, M.; Head, T.; Heister, A.; Hennessy, K.; Henrard, P.; van Herwijnen, E.; Hess, M.; Hicheur, A.; Hill, D.; Hombach, C.; Hopchev, P. H.; Huard, Z. -C.; Hulsbergen, W.; Humair, T.; Hushchyn, M.; Hutchcroft, D.; Idzik, M.; Ilten, P.; Jacobsson, R.; Jalocha, J.; Jans, E.; Jawahery, A.; John, M.; Jones, C. R.; Joram, C.; Jost, B.; Jurik, N.; Kandybei, S.; Karacson, M.; Kariuki, J. M.; Karodia, S.; Kecke, M.; Kelsey, M.; Kenzie, M.; Ketel, T.; Khairullin, E.; Khanji, B.; Khurewathanakul, C.; Kirn, T.; Klaver, S.; Klimaszewski, K.; Klimkovich, T.; Koliiev, S.; Kolpin, M.; Komarov, I.; Kopecna, R.; Koppenburg, P.; Kosmyntseva, A.; Kotriakhova, S.; Kozeiha, M.; Kravchuk, L.; Kreps, M.; Krokovny, P.; Kruse, F.; Krzemien, W.; Kucewicz, W.; Kucharczyk, M.; Kudryavtsev, V.; Kuonen, A. K.; Kurek, K.; Kvaratskheliya, T.; Lacarrere, D.; Lafferty, G.; Lai, A.; Lanfranchi, G.; Langenbruch, C.; Latham, T.; Lazzeroni, C.; Le Gac, R.; van Leerdam, J.; Leflat, A.; Lefrancois, J.; Lefevre, R.; Lemaitre, F.; Lemos Cid, E.; Leroy, O.; Lesiak, T.; Leverington, B.; Likhomanenko, T.; Lindner, R.; Lionetto, F.; Loh, D.; Longstaff, I.; Lopes, J. H.; Lucchesi, D.; Lucio Martinez, M.; Luo, H.; Lupato, A.; Luppi, E.; Lupton, O.; Lusiani, A.; Lyu, X.; Machefert, F.; Maciuc, F.; Maev, O.; Maguire, K.; Malde, S.; Malinin, A.; Maltsev, T.; Manca, G.; Mancinelli, G.; Manning, P.; Maratas, J.; Marchand, J. F.; Marconi, U.; Marin Benito, C.; Marinangeli, M.; Marino, P.; Marks, J.; Martellotti, G.; Martin, M.; Martinelli, M.; Martinez Santos, D.; Martinez Vidal, F.; Martins Tostes, D.; Massacrier, L. M.; Massafferri, A.; Matev, R.; Mathad, A.; Mathe, Z.; Matteuzzi, C.; Mauri, A.; Maurice, E.; Maurin, B.; Mazurov, A.; McCann, M.; McNab, A.; McNulty, R.; Meadows, B.; Meier, F.; Melnychuk, D.; Merk, M.; Merli, A.; Michielin, E.; Milanes, D. A.; Minard, M. -N.; Mitzel, D. S.; Mogini, A.; Molina Rodriguez, J.; Monroy, I. A.; Monteil, S.; Morandin, M.; Morello, M. J.; Morgunova, O.; Moron, J.; Morris, A. B.; Mountain, R.; Muheim, F.; Mussini, M.; Mueller, D.; Mueller, J.; Mueller, K.; Mueller, V.; Naik, P.; Nakada, T.; Nandakumar, R.; Nandi, A.; Nasteva, I.; Needham, M.; Neri, N.; Neubert, S.; Neufeld, N.; Neuner, M.; Nguyen-Mau, C.; Nieswand, S.; Niet, R.; Nikitin, N.; Nikodem, T.; Nogay, A.; Novoselov, A.; O'Hanlon, D. P.; Oblakowska-Mucha, A.; Obraztsov, V.; Ogilvy, S.; Oldeman, R.; Onderwater, C. J. G.; Ossowska, A.; Otalora Goicochea, J. M.; Owen, P.; Oyanguren, A.; Pais, P. R.; Palano, A.; Palutan, M.; Papanestis, A.; Pappagallo, M.; Pappalardo, L. L.; Pappenheimer, C.; Parker, W.; Parkes, C.; Passaleva, G.; Pastore, A.; Patel, M.; Patrignani, C.; Pearce, A.; Pellegrino, A.; Penso, G.; Altarelli, M. Pepe; Perazzini, S.; Perret, P.; Pescatore, L.; Petridis, K.; Petrolini, A.; Petrov, A.; Petruzzo, M.; Picatoste Olloqui, E.; Pietrzyk, B.; Pikies, M.; Pinci, D.; Pistone, A.; Piucci, A.; Placinta, V.; Playfer, S.; Plo Casasus, M.; Poikela, T.; Polci, F.; Lener, M. Poli; Poluektov, A.; Polyakov, I.; Polycarpo, E.; Pomery, G. J.; Ponce, S.; Popov, A.; Popov, D.; Popovici, B.; Poslavskii, S.; Potterat, C.; Price, E.; Prisciandaro, J.; Prouve, C.; Pugatch, V.; Navarro, A. Puig; Punzi, G.; Qian, W.; Quagliani, R.; Rachwal, B.; Rademacker, J. H.; Rama, M.; Ramos Pernas, M.; Rangel, M. S.; Raniuk, I.; Ratnikov, F.; Raven, G.; Redi, F.; Reichert, S.; dos Reis, A. C.; Remon Alepuz, C.; Renaudin, V.; Ricciardi, S.; Richards, S.; Rihl, M.; Rinnert, K.; Rives Molina, V.; Robbe, P.; Rodrigues, A. B.; Rodrigues, E.; Rodriguez Lopez, J. A.; Perez, P. Rodriguez; Rogozhnikov, A.; Roiser, S.; Rollings, A.; Romanovskiy, V.; Romero Vidal, A.; Ronayne, J. W.; Rotondo, M.; Rudolph, M. S.; Ruf, T.; Ruiz Valls, P.; Saborido Silva, J. J.; Sadykhov, E.; Sagidova, N.; Saitta, B.; Salustino Guimaraes, V.; Sanchez Gonzalo, D.; Sanchez Mayordomo, C.; Sanmartin Sedes, B.; Santacesaria, R.; Santamarina Rios, C.; Santimaria, M.; Santovetti, E.; Satriano, C.; Satta, A.; Saunders, D. M.; Savrina, D.; Schael, S.; Schellenberg, M.; Schiller, M.; Schindler, H.; Schlupp, M.; Schmelling, M.; Schmelzer, T.; Schmidt, B.; Schneider, O.; Schopper, A.; Schreiner, H. F.; Schubert, K.; Schubiger, M.; Schune, M. -H.; Schwemmer, R.; Sciascia, B.; Sciubba, A.; Semennikov, A.; Sergi, A.; Serra, N.; Serrano, J.; Sestini, L.; Seyfert, P.; Shapkin, M.; Shapoval, I.; Shcheglov, Y.; Shears, T.; Shekhtman, L.; Shevchenko, V.; Siddi, B. G.; Coutinho, R. Silva; Silva de Oliveira, L.; Simi, G.; Simone, S.; Sirendi, M.; Skidmore, N.; Skwarnicki, T.; Smith, J.; Smith, M.; Soares Lavra, L.; Sokoloff, M. D.; Soler, F. J. P.; Souza De Paula, B.; Spaan, B.; Spradlin, P.; Sridharan, S.; Stagni, F.; Stahl, M.; Stahl, S.; Stefko, P.; Stefkova, S.; Steinkamp, O.; Stemmle, S.; Stenyakin, O.; Stevens, H.; Stoica, S.; Stone, S.; Storaci, B.; Stracka, S.; Stramaglia, M. E.; Straticiuc, M.; Straumann, U.; Sun, L.; Sutcliffe, W.; Swientek, K.; Syropoulos, V.; Szczekowski, M.; Szumlak, T.; T'Jampens, S.; Tayduganov, A.; Tekampe, T.; Tellarini, G.; Teubert, F.; van Tilburg, J.; Tilley, M. J.; Tisserand, V.; Tobin, M.; Tolk, S.; Tomassetti, L.; Tonelli, D.; Topp-Joergensen, S.; Toriello, F.; Tourinho Jadallah Aoude, R.; Tournefier, E.; Tourneur, S.; Trabelsi, K.; Traill, M.; Tran, M. T.; Tresch, M.; Trisovic, A.; Tsaregorodtsev, A.; Tsopelas, P.; Tully, A.; Tuning, N.; Ukleja, A.; Ustyuzhanin, A.; Uwer, U.; Vacca, C.; Vagnoni, V.; Valassi, A.; Valat, S.; Valenti, G.; Gomez, R. Vazquez; Vazquez Regueiro, P.; Vecchi, S.; van Veghel, M.; Velthuis, J. J.; Veltri, M.; Veneziano, G.; Venkateswaran, A.; Verlage, T. A.; Vernet, M.; Vesterinen, M.; Barbosa, J. V. Viana; Viaud, B.; Vieites Diaz, M.; Viemann, H.; Vilasis-Cardona, X.; Vitti, M.; Volkov, V.; Vollhardt, A.; Voneki, B.; Vorobyev, A.; Vorobyev, V.; Voss, C.; Vazquez Sierra, C.; Waldi, R.; Wallace, C.; Wallace, R.; Walsh, J.; Ward, D. R.; Wark, H. M.; Watson, N. K.; Websdale, D.; Weiden, A.; Whitehead, M.; Wicht, J.; Wilkinson, G.; Wilkinson, M.; Williams, M.; Williams, M. P.; Williams, M.; Wilson, F. F.; Wimberley, J.; Winn, M. A.; Wishahi, J.; Wislicki, W.; Witek, M.; Wormser, G.; Wotton, S. A.; Wraight, K.; Wyllie, K.; Xie, Y.; Yang, Z.; Yao, Y.; Yin, H.; Yuan, X.; Yushchenko, O.; Zarebski, K. A.; Zavertyaev, M.; Zhelezov, A.; Zhukov, V.; Zucchelli, S.

    2017-01-01

    A search for the decay K 0 S →μ + μ − KS0→μ+μ− is performed, based on a data sample of proton-proton collisions corresponding to an integrated luminosity of 3 fb −1 3 fb −1 , collected by the LHCb experiment at centre-of-mass energies of 7 and 8TeV TeV . The observed yield is consistent with the

  12. Process-based evaluation of the ÖKS15 Austrian climate scenarios: First results

    Science.gov (United States)

    Mendlik, Thomas; Truhetz, Heimo; Jury, Martin; Maraun, Douglas

    2017-04-01

    The climate scenarios for Austria from the ÖKS15 project consists of 13 downscaled and bias-corrected RCMs from the EURO-CORDEX project. This dataset is meant for the broad public and is now available at the central national archive for climate data (CCCA Data Center). Because of this huge public outreach it is absolutely necessary to objectively discuss the limitations of this dataset and to publish these limitations, which should also be understood by a non-scientific audience. Even though systematical climatological biases have been accounted for by the Scaled-Distribution-Mapping (SDM) bias-correction method, it is not guaranteed that the model biases have been removed for the right reasons. If climate scenarios do not get the patterns of synoptic variability right, biases will still prevail in certain weather patterns. Ultimately this will have consequences for the projected climate change signals. In this study we derive typical weather types in the Alpine Region based on patterns from mean sea level pressure from ERA-INTERIM data and check the occurrence of these synoptic phenomena in EURO-CORDEX data and their corresponding driving GCMs. Based on these weather patterns we analyze the remaining biases of the downscaled and bias-corrected scenarios. We argue that such a process-based evaluation is not only necessary from a scientific point of view, but can also help the broader public to understand the limitations of downscaled climate scenarios, as model errors can be interpreted in terms of everyday observable weather.

  13. First observation of the decay $B_{s}^{0} \\to K_{S}^{0} K^{*}(892)^{0}$

    CERN Document Server

    Aaij, Roel; Adinolfi, Marco; Affolder, Anthony; Ajaltouni, Ziad; Akar, Simon; Albrecht, Johannes; Alessio, Federico; Alexander, Michael; Ali, Suvayu; Alkhazov, Georgy; Alvarez Cartelle, Paula; Alves Jr, Antonio Augusto; Amato, Sandra; Amerio, Silvia; Amhis, Yasmine; An, Liupan; Anderlini, Lucio; Anderson, Jonathan; Andreotti, Mirco; Andrews, Jason; Appleby, Robert; Aquines Gutierrez, Osvaldo; Archilli, Flavio; d'Argent, Philippe; Artamonov, Alexander; Artuso, Marina; Aslanides, Elie; Auriemma, Giulio; Baalouch, Marouen; Bachmann, Sebastian; Back, John; Badalov, Alexey; Baesso, Clarissa; Baldini, Wander; Barlow, Roger; Barschel, Colin; Barsuk, Sergey; Barter, William; Batozskaya, Varvara; Battista, Vincenzo; Bay, Aurelio; Beaucourt, Leo; Beddow, John; Bedeschi, Franco; Bediaga, Ignacio; Bel, Lennaert; Belyaev, Ivan; Ben-Haim, Eli; Bencivenni, Giovanni; Benson, Sean; Benton, Jack; Berezhnoy, Alexander; Bernet, Roland; Bertolin, Alessandro; Bettler, Marc-Olivier; van Beuzekom, Martinus; Bien, Alexander; Bifani, Simone; Bird, Thomas; Birnkraut, Alex; Bizzeti, Andrea; Blake, Thomas; Blanc, Frédéric; Blouw, Johan; Blusk, Steven; Bocci, Valerio; Bondar, Alexander; Bondar, Nikolay; Bonivento, Walter; Borghi, Silvia; Borsato, Martino; Bowcock, Themistocles; Bowen, Espen Eie; Bozzi, Concezio; Braun, Svende; Brett, David; Britsch, Markward; Britton, Thomas; Brodzicka, Jolanta; Brook, Nicholas; Bursche, Albert; Buytaert, Jan; Cadeddu, Sandro; Calabrese, Roberto; Calvi, Marta; Calvo Gomez, Miriam; Campana, Pierluigi; Campora Perez, Daniel; Capriotti, Lorenzo; Carbone, Angelo; Carboni, Giovanni; Cardinale, Roberta; Cardini, Alessandro; Carniti, Paolo; Carson, Laurence; Carvalho Akiba, Kazuyoshi; Casanova Mohr, Raimon; Casse, Gianluigi; Cassina, Lorenzo; Castillo Garcia, Lucia; Cattaneo, Marco; Cauet, Christophe; Cavallero, Giovanni; Cenci, Riccardo; Charles, Matthew; Charpentier, Philippe; Chefdeville, Maximilien; Chen, Shanzhen; Cheung, Shu-Faye; Chiapolini, Nicola; Chrzaszcz, Marcin; Cid Vidal, Xabier; Ciezarek, Gregory; Clarke, Peter; Clemencic, Marco; Cliff, Harry; Closier, Joel; Coco, Victor; Cogan, Julien; Cogneras, Eric; Cogoni, Violetta; Cojocariu, Lucian; Collazuol, Gianmaria; Collins, Paula; Comerma-Montells, Albert; Contu, Andrea; Cook, Andrew; Coombes, Matthew; Coquereau, Samuel; Corti, Gloria; Corvo, Marco; Couturier, Benjamin; Cowan, Greig; Craik, Daniel Charles; Crocombe, Andrew; Cruz Torres, Melissa Maria; Cunliffe, Samuel; Currie, Robert; D'Ambrosio, Carmelo; Dalseno, Jeremy; David, Pieter; Davis, Adam; De Bruyn, Kristof; De Capua, Stefano; De Cian, Michel; De Miranda, Jussara; De Paula, Leandro; De Silva, Weeraddana; De Simone, Patrizia; Dean, Cameron Thomas; Decamp, Daniel; Deckenhoff, Mirko; Del Buono, Luigi; Déléage, Nicolas; Derkach, Denis; Deschamps, Olivier; Dettori, Francesco; Dey, Biplab; Di Canto, Angelo; Di Ruscio, Francesco; Dijkstra, Hans; Donleavy, Stephanie; Dordei, Francesca; Dorigo, Mirco; Dosil Suárez, Alvaro; Dossett, David; Dovbnya, Anatoliy; Dreimanis, Karlis; Dufour, Laurent; Dujany, Giulio; Dupertuis, Frederic; Durante, Paolo; Dzhelyadin, Rustem; Dziurda, Agnieszka; Dzyuba, Alexey; Easo, Sajan; Egede, Ulrik; Egorychev, Victor; Eidelman, Semen; Eisenhardt, Stephan; Eitschberger, Ulrich; Ekelhof, Robert; Eklund, Lars; El Rifai, Ibrahim; Elsasser, Christian; Ely, Scott; Esen, Sevda; Evans, Hannah Mary; Evans, Timothy; Falabella, Antonio; Färber, Christian; Farinelli, Chiara; Farley, Nathanael; Farry, Stephen; Fay, Robert; Ferguson, Dianne; Fernandez Albor, Victor; Ferrari, Fabio; Ferreira Rodrigues, Fernando; Ferro-Luzzi, Massimiliano; Filippov, Sergey; Fiore, Marco; Fiorini, Massimiliano; Firlej, Miroslaw; Fitzpatrick, Conor; Fiutowski, Tomasz; Fohl, Klaus; Fol, Philip; Fontana, Marianna; Fontanelli, Flavio; Forty, Roger; Francisco, Oscar; Frank, Markus; Frei, Christoph; Frosini, Maddalena; Fu, Jinlin; Furfaro, Emiliano; Gallas Torreira, Abraham; Galli, Domenico; Gallorini, Stefano; Gambetta, Silvia; Gandelman, Miriam; Gandini, Paolo; Gao, Yuanning; García Pardiñas, Julián; Garofoli, Justin; Garra Tico, Jordi; Garrido, Lluis; Gascon, David; Gaspar, Clara; Gauld, Rhorry; Gavardi, Laura; Gazzoni, Giulio; Geraci, Angelo; Gerick, David; Gersabeck, Evelina; Gersabeck, Marco; Gershon, Timothy; Ghez, Philippe; Gianelle, Alessio; Gianì, Sebastiana; Gibson, Valerie; Girard, Olivier Göran; Giubega, Lavinia-Helena; Gligorov, V.V.; Göbel, Carla; Golubkov, Dmitry; Golutvin, Andrey; Gomes, Alvaro; Gotti, Claudio; Grabalosa Gándara, Marc; Graciani Diaz, Ricardo; Granado Cardoso, Luis Alberto; Graugés, Eugeni; Graverini, Elena; Graziani, Giacomo; Grecu, Alexandru; Greening, Edward; Gregson, Sam; Griffith, Peter; Grillo, Lucia; Grünberg, Oliver; Gui, Bin; Gushchin, Evgeny; Guz, Yury; Gys, Thierry; Hadjivasiliou, Christos; Haefeli, Guido; Haen, Christophe; Haines, Susan; Hall, Samuel; Hamilton, Brian; Hampson, Thomas; Han, Xiaoxue; Hansmann-Menzemer, Stephanie; Harnew, Neville; Harnew, Samuel; Harrison, Jonathan; He, Jibo; Head, Timothy; Heijne, Veerle; Hennessy, Karol; Henrard, Pierre; Henry, Louis; Hernando Morata, Jose Angel; van Herwijnen, Eric; Heß, Miriam; Hicheur, Adlène; Hill, Donal; Hoballah, Mostafa; Hombach, Christoph; Hulsbergen, Wouter; Humair, Thibaud; Hussain, Nazim; Hutchcroft, David; Hynds, Daniel; Idzik, Marek; Ilten, Philip; Jacobsson, Richard; Jaeger, Andreas; Jalocha, Pawel; Jans, Eddy; Jawahery, Abolhassan; Jing, Fanfan; John, Malcolm; Johnson, Daniel; Jones, Christopher; Joram, Christian; Jost, Beat; Jurik, Nathan; Kandybei, Sergii; Kanso, Walaa; Karacson, Matthias; Karbach, Moritz; Karodia, Sarah; Kelsey, Matthew; Kenyon, Ian; Kenzie, Matthew; Ketel, Tjeerd; Khanji, Basem; Khurewathanakul, Chitsanu; Klaver, Suzanne; Klimaszewski, Konrad; Kochebina, Olga; Kolpin, Michael; Komarov, Ilya; Koopman, Rose; Koppenburg, Patrick; Kravchuk, Leonid; Kreplin, Katharina; Kreps, Michal; Krocker, Georg; Krokovny, Pavel; Kruse, Florian; Kucewicz, Wojciech; Kucharczyk, Marcin; Kudryavtsev, Vasily; Kuonen, Axel Kevin; Kurek, Krzysztof; Kvaratskheliya, Tengiz; La Thi, Viet Nga; Lacarrere, Daniel; Lafferty, George; Lai, Adriano; Lambert, Dean; Lambert, Robert W; Lanfranchi, Gaia; Langenbruch, Christoph; Langhans, Benedikt; Latham, Thomas; Lazzeroni, Cristina; Le Gac, Renaud; van Leerdam, Jeroen; Lees, Jean-Pierre; Lefèvre, Regis; Leflat, Alexander; Lefrançois, Jacques; Leroy, Olivier; Lesiak, Tadeusz; Leverington, Blake; Li, Yiming; Likhomanenko, Tatiana; Liles, Myfanwy; Lindner, Rolf; Linn, Christian; Lionetto, Federica; Liu, Bo; Liu, Xuesong; Lohn, Stefan; Longstaff, Iain; Lopes, Jose; Lucchesi, Donatella; Lucio Martinez, Miriam; Luo, Haofei; Lupato, Anna; Luppi, Eleonora; Lupton, Oliver; Machefert, Frederic; Maciuc, Florin; Maev, Oleg; Maguire, Kevin; Malde, Sneha; Malinin, Alexander; Manca, Giulia; Mancinelli, Giampiero; Manning, Peter Michael; Mapelli, Alessandro; Maratas, Jan; Marchand, Jean François; Marconi, Umberto; Marin Benito, Carla; Marino, Pietro; Märki, Raphael; Marks, Jörg; Martellotti, Giuseppe; Martinelli, Maurizio; Martinez Santos, Diego; Martinez Vidal, Fernando; Martins Tostes, Danielle; Massafferri, André; Matev, Rosen; Mathad, Abhijit; Mathe, Zoltan; Matteuzzi, Clara; Matthieu, Kecke; Mauri, Andrea; Maurin, Brice; Mazurov, Alexander; McCann, Michael; McCarthy, James; McNab, Andrew; McNulty, Ronan; Meadows, Brian; Meier, Frank; Meissner, Marco; Merk, Marcel; Milanes, Diego Alejandro; Minard, Marie-Noelle; Mitzel, Dominik Stefan; Molina Rodriguez, Josue; Monteil, Stephane; Morandin, Mauro; Morawski, Piotr; Mordà, Alessandro; Morello, Michael Joseph; Moron, Jakub; Morris, Adam Benjamin; Mountain, Raymond; Muheim, Franz; Müller, Janine; Müller, Katharina; Müller, Vanessa; Mussini, Manuel; Muster, Bastien; Naik, Paras; Nakada, Tatsuya; Nandakumar, Raja; Nasteva, Irina; Needham, Matthew; Neri, Nicola; Neubert, Sebastian; Neufeld, Niko; Neuner, Max; Nguyen, Anh Duc; Nguyen, Thi-Dung; Nguyen-Mau, Chung; Niess, Valentin; Niet, Ramon; Nikitin, Nikolay; Nikodem, Thomas; Ninci, Daniele; Novoselov, Alexey; O'Hanlon, Daniel Patrick; Oblakowska-Mucha, Agnieszka; Obraztsov, Vladimir; Ogilvy, Stephen; Okhrimenko, Oleksandr; Oldeman, Rudolf; Onderwater, Gerco; Osorio Rodrigues, Bruno; Otalora Goicochea, Juan Martin; Otto, Adam; Owen, Patrick; Oyanguren, Maria Aranzazu; Palano, Antimo; Palombo, Fernando; Palutan, Matteo; Panman, Jacob; Papanestis, Antonios; Pappagallo, Marco; Pappalardo, Luciano; Parkes, Christopher; Passaleva, Giovanni; Patel, Girish; Patel, Mitesh; Patrignani, Claudia; Pearce, Alex; Pellegrino, Antonio; Penso, Gianni; Pepe Altarelli, Monica; Perazzini, Stefano; Perret, Pascal; Pescatore, Luca; Petridis, Konstantinos; Petrolini, Alessandro; Picatoste Olloqui, Eduardo; Pietrzyk, Boleslaw; Pilař, Tomas; Pinci, Davide; Pistone, Alessandro; Piucci, Alessio; Playfer, Stephen; Plo Casasus, Maximo; Poikela, Tuomas; Polci, Francesco; Poluektov, Anton; Polyakov, Ivan; Polycarpo, Erica; Popov, Alexander; Popov, Dmitry; Popovici, Bogdan; Potterat, Cédric; Price, Eugenia; Price, Joseph David; Prisciandaro, Jessica; Pritchard, Adrian; Prouve, Claire; Pugatch, Valery; Puig Navarro, Albert; Punzi, Giovanni; Qian, Wenbin; Quagliani, Renato; Rachwal, Bartolomiej; Rademacker, Jonas; Rakotomiaramanana, Barinjaka; Rama, Matteo; Rangel, Murilo; Raniuk, Iurii; Rauschmayr, Nathalie; Raven, Gerhard; Redi, Federico; Reichert, Stefanie; Reid, Matthew; dos Reis, Alberto; Ricciardi, Stefania; Richards, Sophie; Rihl, Mariana; Rinnert, Kurt; Rives Molina, Vincente; Robbe, Patrick; Rodrigues, Ana Barbara; Rodrigues, Eduardo; Rodriguez Lopez, Jairo Alexis; Rodriguez Perez, Pablo; Roiser, Stefan; Romanovsky, Vladimir; Romero Vidal, Antonio; Rotondo, Marcello; Rouvinet, Julien; Ruf, Thomas; Ruiz, Hugo; Ruiz Valls, Pablo; Saborido Silva, Juan Jose; Sagidova, Naylya; Sail, Paul; Saitta, Biagio; Salustino Guimaraes, Valdir; Sanchez Mayordomo, Carlos; Sanmartin Sedes, Brais; Santacesaria, Roberta; Santamarina Rios, Cibran; Santimaria, Marco; Santovetti, Emanuele; Sarti, Alessio; Satriano, Celestina; Satta, Alessia; Saunders, Daniel Martin; Savrina, Darya; Schiller, Manuel; Schindler, Heinrich; Schlupp, Maximilian; Schmelling, Michael; Schmelzer, Timon; Schmidt, Burkhard; Schneider, Olivier; Schopper, Andreas; Schubiger, Maxime; Schune, Marie Helene; Schwemmer, Rainer; Sciascia, Barbara; Sciubba, Adalberto; Semennikov, Alexander; Sepp, Indrek; Serra, Nicola; Serrano, Justine; Sestini, Lorenzo; Seyfert, Paul; Shapkin, Mikhail; Shapoval, Illya; Shcheglov, Yury; Shears, Tara; Shekhtman, Lev; Shevchenko, Vladimir; Shires, Alexander; Silva Coutinho, Rafael; Simi, Gabriele; Sirendi, Marek; Skidmore, Nicola; Skillicorn, Ian; Skwarnicki, Tomasz; Smith, Edmund; Smith, Eluned; Smith, Iwan Thomas; Smith, Jackson; Smith, Mark; Snoek, Hella; Sokoloff, Michael; Soler, Paul; Soomro, Fatima; Souza, Daniel; Souza De Paula, Bruno; Spaan, Bernhard; Spradlin, Patrick; Sridharan, Srikanth; Stagni, Federico; Stahl, Marian; Stahl, Sascha; Steinkamp, Olaf; Stenyakin, Oleg; Sterpka, Christopher Francis; Stevenson, Scott; Stoica, Sabin; Stone, Sheldon; Storaci, Barbara; Stracka, Simone; Straticiuc, Mihai; Straumann, Ulrich; Sun, Liang; Sutcliffe, William; Swientek, Krzysztof; Swientek, Stefan; Syropoulos, Vasileios; Szczekowski, Marek; Szczypka, Paul; Szumlak, Tomasz; T'Jampens, Stephane; Tekampe, Tobias; Teklishyn, Maksym; Tellarini, Giulia; Teubert, Frederic; Thomas, Christopher; Thomas, Eric; van Tilburg, Jeroen; Tisserand, Vincent; Tobin, Mark; Todd, Jacob; Tolk, Siim; Tomassetti, Luca; Tonelli, Diego; Topp-Joergensen, Stig; Torr, Nicholas; Tournefier, Edwige; Tourneur, Stephane; Trabelsi, Karim; Tran, Minh Tâm; Tresch, Marco; Trisovic, Ana; Tsaregorodtsev, Andrei; Tsopelas, Panagiotis; Tuning, Niels; Ukleja, Artur; Ustyuzhanin, Andrey; Uwer, Ulrich; Vacca, Claudia; Vagnoni, Vincenzo; Valenti, Giovanni; Vallier, Alexis; Vazquez Gomez, Ricardo; Vazquez Regueiro, Pablo; Vázquez Sierra, Carlos; Vecchi, Stefania; Velthuis, Jaap; Veltri, Michele; Veneziano, Giovanni; Vesterinen, Mika; Viaud, Benoit; Vieira, Daniel; Vieites Diaz, Maria; Vilasis-Cardona, Xavier; Vollhardt, Achim; Volyanskyy, Dmytro; Voong, David; Vorobyev, Alexey; Vorobyev, Vitaly; Voß, Christian; de Vries, Jacco; Waldi, Roland; Wallace, Charlotte; Wallace, Ronan; Walsh, John; Wandernoth, Sebastian; Wang, Jianchun; Ward, David; Watson, Nigel; Websdale, David; Weiden, Andreas; Whitehead, Mark; Wiedner, Dirk; Wilkinson, Guy; Wilkinson, Michael; Williams, Mark Richard James; Williams, Matthew; Williams, Mike; Williams, Timothy; Wilson, Fergus; Wimberley, Jack; Wishahi, Julian; Wislicki, Wojciech; Witek, Mariusz; Wormser, Guy; Wotton, Stephen; Wright, Simon; Wyllie, Kenneth; Xie, Yuehong; Xu, Zhirui; Yang, Zhenwei; Yu, Jiesheng; Yuan, Xuhao; Yushchenko, Oleg; Zangoli, Maria; Zavertyaev, Mikhail; Zhang, Liming; Zhang, Yanxi; Zhelezov, Alexey; Zhokhov, Anatoly; Zhong, Liang

    2016-01-04

    A search for $B_{(s)}^{0} \\to K_{S}^{0} K^{*}(892)^{0}$ decays is performed using $pp$ collision data, corresponding to an integrated luminosity of $1.0~\\text{fb}^{-1}$, collected with the LHCb detector at a centre-of-mass energy of $7~\\text{TeV}$. The $B_{s}^{0} \\to K_{S}^{0} K^{*}(892)^{0}$ decay is observed for the first time, with a significance of 7.1 standard deviations. The branching fraction is measured to be \\begin{equation*} \\mathcal{B}(B_{s}^{0} \\to \\bar{K}^{0} K^{*}(892)^{0}) + \\mathcal{B}(B_{s}^{0} \\to K^{0} \\bar{K}^{*}(892)^{0}) = (16.4 \\pm 3.4 \\pm 2.3) \\times 10^{-6},\\\\ \\end{equation*} where the first uncertainty is statistical and the second is systematic. No evidence is found for the decay $B^{0} \\to K_{S}^{0} K^{*}(892)^{0}$ and an upper limit is set on the branching fraction, $\\mathcal{B}(B^{0} \\to \\bar{K}^{0} K^{*}(892)^{0}) + \\mathcal{B}(B^{0} \\to K^{0} \\bar{K}^{*}(892)^{0}) < 0.96 \\ \\times 10^{-6}, $ at $90\\,\\% $ confidence level. All results are consistent with Standard Model pr...

  14. Beals Syndrome

    Science.gov (United States)

    ... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of the ... bone) and aortic enlargement problems as people with Marfan syndrome, and treatments for these problems are the same. ...

  15. Characteristics of a nationwide cohort of patients presenting with isolated hypogonadotropic hypogonadism (IHH).

    Science.gov (United States)

    Bonomi, Marco; Vezzoli, Valeria; Krausz, Csilla; Guizzardi, Fabiana; Vezzani, Silvia; Simoni, Manuela; Bassi, Ivan; Duminuco, Paolo; Di Iorgi, Natascia; Giavoli, Claudia; Pizzocaro, Alessandro; Russo, Gianni; Moro, Mirella; Fatti, Letizia; Ferlin, Alberto; Mazzanti, Laura; Zatelli, Maria Chiara; Cannavò, Salvo; Isidori, Andrea M; Pincelli, Angela Ida; Prodam, Flavia; Mancini, Antonio; Limone, Paolo; Tanda, Maria Laura; Gaudino, Rossella; Salerno, Mariacarolina; Francesca, Pregnolato; Maghnie, Mohamad; Maggi, Mario; Persani, Luca

    2018-01-01

    Isolated hypogonadotropic hypogonadism (IHH) is a rare disorder with pubertal delay, normal (normoosmic-IHH, nIHH) or defective sense of smell (Kallmann syndrome, KS). Other reproductive and non-reproductive anomalies might be present although information on their frequency are scanty, particularly according to the age of presentation. Observational cohort study carried out between January 2008 and June 2016 within a national network of academic or general hospitals. We performed a detailed phenotyping of 503 IHH patients with: (1) manifestations of hypogonadism with low sex steroid hormone and low/normal gonadotropins; (2) absence of expansive hypothalamic/pituitary lesions or multiple pituitary hormone defects. Cohort was divided on IHH onset (PPO, pre-pubertal onset or AO, adult onset) and olfactory function: PPO-nIHH ( n  = 275), KS ( n  = 184), AO-nIHH ( n  = 36) and AO-doIHH (AO-IHH with defective olfaction, n  = 8). 90% of patients were classified as PPO and 10% as AO. Typical midline and olfactory defects, bimanual synkinesis and familiarity for pubertal delay were also found among the AO-IHH. Mean age at diagnosis was significantly earlier and more frequently associated with congenital hypogonadism stigmata in patients with Kallmann's syndrome (KS). Synkinesis, renal and male genital tract anomalies were enriched in KS. Overweight/obesity are significantly associated with AO-IHH rather than PPO-IHH. Patients with KS are more prone to develop a severe and complex phenotype than nIHH. The presence of typical extra-gonadal defects and familiarity for PPO-IHH among the AO-IHH patients indicates a common predisposition with variable clinical expression. Overall, these findings improve the understanding of IHH and may have a positive impact on the management of patients and their families. © 2018 European Society of Endocrinology.

  16. LHCb: Search for CP violation in $B_0 \\to J/\\psi K_s^0$ decays with first LHCb data

    CERN Multimedia

    Santana Rangel, Murilo

    2011-01-01

    We report a measurement of the well-established CP violation in $B_0 \\to J/\\psi K_s^0$ decays. We perform a time-dependent analysis of the decays reconstructed in 35 pb $^{-1}$ of LHCb data that was taken in 2010. We measure the CP asymmetry parameter $S_{ J/\\psi K_s^0}$ , which is connected to the CKM angle $\\beta$ through $S_{ J/\\psi K_s^0}$ = sin2$\\beta$, neglecting CP violation in $B^0$-$ \\overline{B^0}$ mixing and decay. We find $S_{ J/\\psi K_s^0} $ = 0.53$^{+0.28}_{-0.29}$(stat) $\\pm$ 0.05(syst).

  17. Clinical and Neurobehavioral Features of Three Novel Kabuki Syndrome Patients with Mosaic KMT2D Mutations and a Review of Literature.

    Science.gov (United States)

    Lepri, Francesca Romana; Cocciadiferro, Dario; Augello, Bartolomeo; Alfieri, Paolo; Pes, Valentina; Vancini, Alessandra; Caciolo, Cristina; Squeo, Gabriella Maria; Malerba, Natascia; Adipietro, Iolanda; Novelli, Antonio; Sotgiu, Stefano; Gherardi, Renzo; Digilio, Maria Cristina; Dallapiccola, Bruno; Merla, Giuseppe

    2017-12-28

    Kabuki syndrome (KS) is a rare disorder characterized by multiple congenital anomalies and variable intellectual disability caused by mutations in KMT2D/MLL2 and KDM6A/UTX , two interacting chromatin modifier responsible respectively for 56-75% and 5-8% of the cases. To date, three KS patients with mosaic KMT2D deletions in blood lymphocytes have been described. We report on three additional subjects displaying KMT2D gene mosaics including one in which a single nucleotide change results in a new frameshift mutation (p.L1199HfsX7), and two with already-known nonsense mutations (p.R4484X and p.R5021X). Consistent with previously published cases, mosaic KMT2D mutations may result in mild KS facial dysmorphisms and clinical and neurobehavioral features, suggesting that these characteristics could represent the handles for genetic testing of individuals with slight KS-like traits.

  18. Clinical and Neurobehavioral Features of Three Novel Kabuki Syndrome Patients with Mosaic KMT2D Mutations and a Review of Literature

    Directory of Open Access Journals (Sweden)

    Francesca Romana Lepri

    2017-12-01

    Full Text Available Kabuki syndrome (KS is a rare disorder characterized by multiple congenital anomalies and variable intellectual disability caused by mutations in KMT2D/MLL2 and KDM6A/UTX, two interacting chromatin modifier responsible respectively for 56–75% and 5–8% of the cases. To date, three KS patients with mosaic KMT2D deletions in blood lymphocytes have been described. We report on three additional subjects displaying KMT2D gene mosaics including one in which a single nucleotide change results in a new frameshift mutation (p.L1199HfsX7, and two with already-known nonsense mutations (p.R4484X and p.R5021X. Consistent with previously published cases, mosaic KMT2D mutations may result in mild KS facial dysmorphisms and clinical and neurobehavioral features, suggesting that these characteristics could represent the handles for genetic testing of individuals with slight KS-like traits.

  19. Contraction of high eccentricity satellite orbits using uniformly regular KS canonical elements with oblate diurnally varying atmosphere.

    Science.gov (United States)

    Raj, Xavier James

    2016-07-01

    Accurate orbit prediction of an artificial satellite under the influence of air drag is one of the most difficult and untraceable problem in orbital dynamics. The orbital decay of these satellites is mainly controlled by the atmospheric drag effects. The effects of the atmosphere are difficult to determine, since the atmospheric density undergoes large fluctuations. The classical Newtonian equations of motion, which is non linear is not suitable for long-term integration. Many transformations have emerged in the literature to stabilize the equations of motion either to reduce the accumulation of local numerical errors or allowing the use of large integration step sizes, or both in the transformed space. One such transformation is known as KS transformation by Kustaanheimo and Stiefel, who regularized the nonlinear Kepler equations of motion and reduced it into linear differential equations of a harmonic oscillator of constant frequency. The method of KS total energy element equations has been found to be a very powerful method for obtaining numerical as well as analytical solution with respect to any type of perturbing forces, as the equations are less sensitive to round off and truncation errors. The uniformly regular KS canonical equations are a particular canonical form of the KS differential equations, where all the ten KS Canonical elements αi and βi are constant for unperturbed motion. These equations permit the uniform formulation of the basic laws of elliptic, parabolic and hyperbolic motion. Using these equations, developed analytical solution for short term orbit predictions with respect to Earth's zonal harmonic terms J2, J3, J4. Further, these equations were utilized to include the canonical forces and analytical theories with air drag were developed for low eccentricity orbits (e 0.2) orbits by assuming the atmosphere to be oblate only. In this paper a new non-singular analytical theory is developed for the motion of high eccentricity satellite

  20. Cushing syndrome

    Science.gov (United States)

    Hypercortisolism; Cortisol excess; Glucocorticoid excess - Cushing syndrome ... The most common cause of Cushing syndrome is taking too much ... Cushing syndrome . Prednisone, dexamethasone, and prednisolone ...

  1. LEOPARD syndrome

    Science.gov (United States)

    Multiple lentigines syndrome; Noonan syndrome with multiple lentigines ... Genetics Home Reference -- ghr.nlm.nih.gov/condition/noonan-syndrome-with-multiple-lentigines National Organization for Rare Disorders -- ...

  2. Neutron and gamma-ray spectra measurement on the model of the KS-150 reactor radial shielding

    International Nuclear Information System (INIS)

    Holman, M.; Hogel, J.; Marik, J.; Kovarik, K.; Franc, L.; Vespalec, R.

    1977-01-01

    A shortened model of the peripheral region of the KS-150 reactor core consisting of two rows of fuel elements and a reflector was constructed from the peripheral fuel elements of the KS-150 reactor core in an experiment on the TR-0 reactor. The mockup of the thermal shield (10 cm of steel), the pressure vessel (15 cm of steel) and the inner wall of the water biological shielding (2 cm of steel) of the KS-150 reactor were erected outside the TR-0 vessel. Fast neutron and gamma spectra were measured with a stilbene crystal scintillation spectrometer. The resonance neutron spectra were measured with 197 Au, 63 Cu and 23 Na resonance activation detectors. Fast neutron spectra inside the reactor were measured with a 10 mm diameter by 10 mm thick stilbene crystal spectrometer, outside the reactor with a 10 mm diameter by 10 mm thick and a 20 mm diameter by 20 mm thick stilbene crystal spectrometer. Neutron spectra in the energy regions of 1 eV to 3 keV and 0.6 MeV to 0.8 MeV were obtained on the core periphery, on the reflector half-thickness and in front of and behind the reactor thermal shield. Gamma spectra were obtained in front of and behind the thermal shield. It was found that the attenuation of neutron fluxes by the reflector and the thermal shield increased with increasing energy while gamma radiation attenuation decreased with increasing energy. It was not possible to obtain the neutron spectrum in the 10 to 600 keV energy range because suitable detection instrumentation was not available. (J.P.)

  3. Amplitude Analysis of the Decay $D^+ \\to K_s \\pi^- \\pi^+ \\pi^+$ in the {FOCUS} Experiment

    Energy Technology Data Exchange (ETDEWEB)

    Otalora Goicochea, Juan Martin [Rio de Janeiro, Pont. U. Catol.

    2007-07-01

    This thesis is devoted to the study of the decay D+ → KS π−π+π+ with data collected from the FOCUS experiment, at Fermilab. The fi state composed of 4 pseudo-scalars can be produced through a number of resonant sub-structures. The purpose of this analysis is to fi the contributing intermediate states by measuring their relative strenghts and phases. For that, the Amplitude Analysis formalism is used, with the so-called Isobar Model. The decay dynamics is described through a function which has the features of the contributing channels (functional forms of the resonances, angular distribution, etc) and which domain is a phase space determined by 5 invariants (due to the kinematical constraints of a 4-body spinless decay). The data sample is thus fi to this function. Our results show a dominant contribution of the axial-vector meson a1(1260) (52%), followed by the K1(1400) axial-vector (34%). Moreover, the model presents a contribution from the σ meson (about 8% as a1(σπ)KS and σKS π) and a significant contribution from the scalar κ−. The κ state has been reported in its neutral mode in other charm decays but not is its charged mode. We fi no significant contribution from the direct 4-body decay (non-resonant). This work adds to the effort in the understanding of the strong-interaction dynamics at low energies, which in recent years have been receiving an important contribution from charm meson physics.

  4. Immunoexpression of androgen receptor and nine markers of maturation in the testes of adolescent boys with Klinefelter syndrome

    DEFF Research Database (Denmark)

    Wikström, Anne M; Hoei-Hansen, Christina E; Dunkel, Leo

    2007-01-01

    CONTEXT: The pathogenesis and mechanisms behind the degeneration of the seminiferous tubules in testes of subjects with Klinefelter syndrome (KS) are yet unknown. OBJECTIVE: The objective of this prospective clinical study was to characterize the testicular degeneration process during puberty in ...

  5. Bone geometry, volumetric density, microarchitecture, and estimated bone strength assessed by HR-pQCT in Klinefelter syndrome

    DEFF Research Database (Denmark)

    Shanbhogue, Vikram V; Hansen, Stinus; Jørgensen, Niklas Rye

    2014-01-01

    Although the expected skeletal manifestations of testosterone deficiency in Klinefelter's syndrome (KS) are osteopenia and osteoporosis, the structural basis for this is unclear. The aim of this study was to assess bone geometry, volumetric bone mineral density (vBMD), microarchitecture...

  6. B → nK* and B → φKS Decays in the Two Higgs Doublet Model III

    International Nuclear Information System (INIS)

    Shuai-Wei, Wang; Tai-Ping, Song; Lin-Xia, Lü

    2008-01-01

    Using the QCD factorization approach, we investigate the large branching ratios of B →φ K s decays and tjhe S φKs anomaly of B →K s decay in the two Higgs doublet model III. With the contributions of flavour-changing neutral current mediated by the neutral Higgs bosons H 0 , h 0 and A 0 at the tree level, we provide a coherent resolution to these anomalies within the constrained parameter spaces, which are 120 bs λ ss | <136. This will be really interesting in searching for the signs of new physics. (the physics of elementary particles and fields)

  7. Metastatic triple-negative breast cancer is dependent on SphKs/S1P signaling for growth and survival.

    Science.gov (United States)

    Maiti, Aparna; Takabe, Kazuaki; Hait, Nitai C

    2017-04-01

    About 40,000 American women die from metastatic breast cancer each year despite advancements in treatment. Approximately, 15% of breast cancers are triple-negative for estrogen receptor, progesterone receptor, and HER2. Triple-negative cancer is characterized by more aggressive, harder to treat with conventional approaches and having a greater possibility of recurrence. Sphingosine-1-phosphate (S1P) is a bioactive sphingolipid signaling mediator has emerged as a key regulatory molecule in breast cancer progression. Therefore, we investigated whether cytosolic sphingosine kinase type 1 (SphK1) and nuclear sphingosine kinase type 2 (SphK2), the enzymes that make S1P are critical for growth and PI3K/AKT, ERK-MAP kinase mediated survival signaling of lung metastatic variant LM2-4 breast cancer cells, generated from the parental triple-negative MDA-MB-231 human breast cancer cell line. Similar with previous report, SphKs/S1P signaling is critical for the growth and survival of estrogen receptor positive MCF-7 human breast cancer cells, was used as our study control. MDA-MB-231 did not show a significant effect of SphKs/S1P signaling on AKT, ERK, and p38 pathways. In contrast, LM2-4 cells that gained lung metastatic phenotype from primary MDA-MB-231 cells show a significant effect of SphKs/S1P signaling requirement on cell growth, survival, and cell motility. PF-543, a selective potent inhibitor of SphK1, attenuated epidermal growth factor (EGF)-mediated cell growth and survival signaling through inhibition of AKT, ERK, and p38 MAP kinase pathways mainly in LM2-4 cells but not in parental MDA-MB-231 human breast cancer cells. Moreover, K-145, a selective inhibitor of SphK2, markedly attenuated EGF-mediated cell growth and survival of LM2-4 cells. We believe this study highlights the importance of SphKs/S1P signaling in metastatic triple-negative breast cancers and targeted therapies. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Cancer incidence in men with Klinefelter syndrome.

    Science.gov (United States)

    Hasle, H.; Mellemgaard, A.; Nielsen, J.; Hansen, J.

    1995-01-01

    Many case reports have suggested an association between Klinefelter syndrome (KS) and cancer, but studies of the cancer incidence in larger groups of men with KS are lacking. A cohort of 696 men with KS was established from the Danish Cytogenetic Register. Information on the cancer incidence in the cohort was obtained from the Danish Cancer Registry and compared with the expected number calculated from the age, period and site specific cancer rates for Danish men. A total of 39 neoplasms were diagnosed (relative risk = 1.1). Four mediastinal tumours were observed (relative risk = 67); all four were malignant germ cell tumours. No cases of breast cancer or testis cancer were observed. One case of prostate cancer occurred within a previously irradiated field. No excess of leukaemia or lymphoma was found. An increased risk of cancer occurred in the age group 15-30 years (relative risk = 2.7). All six tumours in this group were germ cell tumours or sarcomas. The overall cancer incidence is not increased and no routine cancer screening seems to be justified. A considerably elevated risk of mediastinal germ cell tumours occurs in the period from early adolescence until the age of 30. PMID:7841064

  9. Racial/Ethnic Differences in the Incidence of Kawasaki Syndrome among Children in Hawai‘i

    Science.gov (United States)

    Christensen, Krista Y; Belay, Ermias D; Steiner, Claudia A; Effler, Paul V; Miyamura, Jill; Forbes, Susan; Schonberger, Lawrence B; Melish, Marian

    2010-01-01

    Objective To describe the occurrence of Kawasaki syndrome (KS) among different racial/ethnic groups in Hawai‘i. Methods Retrospective analysis of children <18 years of age, with a focus on children <5 years of age, living in Hawai‘i who were hospitalized with KS using the 1996–2006 Hawai‘i State Inpatient Data. Results Children <5 years of age accounted for 84% of the 528 patients <18 years of age with KS. The average annual incidence among this age group was 50.4 per 100,000 children <5 years of age, ranging from 45.5 to 56.5. Asian and Pacific Islander children accounted for 92% of the children <5 years of age with KS during the study period; the average annual incidence was 62.9 per 100,000. Within this group, Japanese children had the highest incidence (210.5), followed by Native Hawaiian children (86.9), other Asian children (84.9), and Chinese children (83.2). The incidence for white children (13.7) was lower than for these racial/ethnic groups. The median age of KS admission for children <5 years of age was 21 months overall, 24 months for Japanese children, 14.5 months for Native Hawaiian children and 26.5 months for white children. Conclusions The high average annual KS incidence for children <5 years of age in Hawai‘i compared to the rest of the United States population reflects an increased KS incidence among Asian and Pacific Islander children, especially Japanese children. The incidence for white children was slightly higher than or similar to that generally reported nationwide. PMID:20845285

  10. Model-independent measurement of the CKM angle $\\gamma$ using $B^0 \\to D K^{\\ast 0}$ decays with $D \\to K_{S}^{0} \\pi^{+} \\pi^{-}$ and $K_{S}^{0} K^{+} K^{-}$

    CERN Document Server

    Aaij, Roel; Adeva, Bernardo; Adinolfi, Marco; Ajaltouni, Ziad; Akar, Simon; Albrecht, Johannes; Alessio, Federico; Alexander, Michael; Ali, Suvayu; Alkhazov, Georgy; Alvarez Cartelle, Paula; Alves Jr, Antonio Augusto; Amato, Sandra; Amerio, Silvia; Amhis, Yasmine; An, Liupan; Anderlini, Lucio; Andreassi, Guido; Andreotti, Mirco; Andrews, Jason; Appleby, Robert; Aquines Gutierrez, Osvaldo; Archilli, Flavio; d'Argent, Philippe; Artamonov, Alexander; Artuso, Marina; Aslanides, Elie; Auriemma, Giulio; Baalouch, Marouen; Bachmann, Sebastian; Back, John; Badalov, Alexey; Baesso, Clarissa; Baker, Sophie; Baldini, Wander; Barlow, Roger; Barschel, Colin; Barsuk, Sergey; Barter, William; Batozskaya, Varvara; Battista, Vincenzo; Bay, Aurelio; Beaucourt, Leo; Beddow, John; Bedeschi, Franco; Bediaga, Ignacio; Bel, Lennaert; Bellee, Violaine; Belloli, Nicoletta; Belyaev, Ivan; Ben-Haim, Eli; Bencivenni, Giovanni; Benson, Sean; Benton, Jack; Berezhnoy, Alexander; Bernet, Roland; Bertolin, Alessandro; Betti, Federico; Bettler, Marc-Olivier; van Beuzekom, Martinus; Bifani, Simone; Billoir, Pierre; Bird, Thomas; Birnkraut, Alex; Bizzeti, Andrea; Blake, Thomas; Blanc, Frédéric; Blouw, Johan; Blusk, Steven; Bocci, Valerio; Bondar, Alexander; Bondar, Nikolay; Bonivento, Walter; Borgheresi, Alessio; Borghi, Silvia; Borisyak, Maxim; Borsato, Martino; Boubdir, Meriem; Bowcock, Themistocles; Bowen, Espen Eie; Bozzi, Concezio; Braun, Svende; Britsch, Markward; Britton, Thomas; Brodzicka, Jolanta; Buchanan, Emma; Burr, Christopher; Bursche, Albert; Buytaert, Jan; Cadeddu, Sandro; Calabrese, Roberto; Calvi, Marta; Calvo Gomez, Miriam; Campana, Pierluigi; Campora Perez, Daniel; Capriotti, Lorenzo; Carbone, Angelo; Carboni, Giovanni; Cardinale, Roberta; Cardini, Alessandro; Carniti, Paolo; Carson, Laurence; Carvalho Akiba, Kazuyoshi; Casse, Gianluigi; Cassina, Lorenzo; Castillo Garcia, Lucia; Cattaneo, Marco; Cauet, Christophe; Cavallero, Giovanni; Cenci, Riccardo; Charles, Matthew; Charpentier, Philippe; Chatzikonstantinidis, Georgios; Chefdeville, Maximilien; Chen, Shanzhen; Cheung, Shu-Faye; Chobanova, Veronika; Chrzaszcz, Marcin; Cid Vidal, Xabier; Ciezarek, Gregory; Clarke, Peter; Clemencic, Marco; Cliff, Harry; Closier, Joel; Coco, Victor; Cogan, Julien; Cogneras, Eric; Cogoni, Violetta; Cojocariu, Lucian; Collazuol, Gianmaria; Collins, Paula; Comerma-Montells, Albert; Contu, Andrea; Cook, Andrew; Coquereau, Samuel; Corti, Gloria; Corvo, Marco; Couturier, Benjamin; Cowan, Greig; Craik, Daniel Charles; Crocombe, Andrew; Cruz Torres, Melissa Maria; Cunliffe, Samuel; Currie, Robert; D'Ambrosio, Carmelo; Dall'Occo, Elena; Dalseno, Jeremy; David, Pieter; Davis, Adam; De Aguiar Francisco, Oscar; De Bruyn, Kristof; De Capua, Stefano; De Cian, Michel; De Miranda, Jussara; De Paula, Leandro; De Simone, Patrizia; Dean, Cameron Thomas; Decamp, Daniel; Deckenhoff, Mirko; Del Buono, Luigi; Déléage, Nicolas; Demmer, Moritz; Derkach, Denis; Deschamps, Olivier; Dettori, Francesco; Dey, Biplab; Di Canto, Angelo; Dijkstra, Hans; Dordei, Francesca; Dorigo, Mirco; Dosil Suárez, Alvaro; Dovbnya, Anatoliy; Dreimanis, Karlis; Dufour, Laurent; Dujany, Giulio; Dungs, Kevin; Durante, Paolo; Dzhelyadin, Rustem; Dziurda, Agnieszka; Dzyuba, Alexey; Easo, Sajan; Egede, Ulrik; Egorychev, Victor; Eidelman, Semen; Eisenhardt, Stephan; Eitschberger, Ulrich; Ekelhof, Robert; Eklund, Lars; El Rifai, Ibrahim; Elsasser, Christian; Ely, Scott; Esen, Sevda; Evans, Hannah Mary; Evans, Timothy; Falabella, Antonio; Färber, Christian; Farley, Nathanael; Farry, Stephen; Fay, Robert; Fazzini, Davide; Ferguson, Dianne; Fernandez Albor, Victor; Ferrari, Fabio; Ferreira Rodrigues, Fernando; Ferro-Luzzi, Massimiliano; Filippov, Sergey; Fiore, Marco; Fiorini, Massimiliano; Firlej, Miroslaw; Fitzpatrick, Conor; Fiutowski, Tomasz; Fleuret, Frederic; Fohl, Klaus; Fontana, Marianna; Fontanelli, Flavio; Forshaw, Dean Charles; Forty, Roger; Frank, Markus; Frei, Christoph; Frosini, Maddalena; Fu, Jinlin; Furfaro, Emiliano; Gallas Torreira, Abraham; Galli, Domenico; Gallorini, Stefano; Gambetta, Silvia; Gandelman, Miriam; Gandini, Paolo; Gao, Yuanning; García Pardiñas, Julián; Garra Tico, Jordi; Garrido, Lluis; Garsed, Philip John; Gascon, David; Gaspar, Clara; Gavardi, Laura; Gazzoni, Giulio; Gerick, David; Gersabeck, Evelina; Gersabeck, Marco; Gershon, Timothy; Ghez, Philippe; Gianì, Sebastiana; Gibson, Valerie; Girard, Olivier Göran; Giubega, Lavinia-Helena; Gligorov, V.V.; Göbel, Carla; Golubkov, Dmitry; Golutvin, Andrey; Gomes, Alvaro; Gotti, Claudio; Grabalosa Gándara, Marc; Graciani Diaz, Ricardo; Granado Cardoso, Luis Alberto; Graugés, Eugeni; Graverini, Elena; Graziani, Giacomo; Grecu, Alexandru; Griffith, Peter; Grillo, Lucia; Grünberg, Oliver; Gushchin, Evgeny; Guz, Yury; Gys, Thierry; Hadavizadeh, Thomas; Hadjivasiliou, Christos; Haefeli, Guido; Haen, Christophe; Haines, Susan; Hall, Samuel; Hamilton, Brian; Han, Xiaoxue; Hansmann-Menzemer, Stephanie; Harnew, Neville; Harnew, Samuel; Harrison, Jonathan; He, Jibo; Head, Timothy; Heister, Arno; Hennessy, Karol; Henrard, Pierre; Henry, Louis; Hernando Morata, Jose Angel; van Herwijnen, Eric; Heß, Miriam; Hicheur, Adlène; Hill, Donal; Hoballah, Mostafa; Hombach, Christoph; Hongming, Li; Hulsbergen, Wouter; Humair, Thibaud; Hushchyn, Mikhail; Hussain, Nazim; Hutchcroft, David; Idzik, Marek; Ilten, Philip; Jacobsson, Richard; Jaeger, Andreas; Jalocha, Pawel; Jans, Eddy; Jawahery, Abolhassan; John, Malcolm; Johnson, Daniel; Jones, Christopher; Joram, Christian; Jost, Beat; Jurik, Nathan; Kandybei, Sergii; Kanso, Walaa; Karacson, Matthias; Karbach, Moritz; Karodia, Sarah; Kecke, Matthieu; Kelsey, Matthew; Kenyon, Ian; Kenzie, Matthew; Ketel, Tjeerd; Khairullin, Egor; Khanji, Basem; Khurewathanakul, Chitsanu; Kirn, Thomas; Klaver, Suzanne; Klimaszewski, Konrad; Kolpin, Michael; Komarov, Ilya; Koopman, Rose; Koppenburg, Patrick; Kozeiha, Mohamad; Kravchuk, Leonid; Kreplin, Katharina; Kreps, Michal; Krokovny, Pavel; Kruse, Florian; Krzemien, Wojciech; Kucewicz, Wojciech; Kucharczyk, Marcin; Kudryavtsev, Vasily; Kuonen, Axel Kevin; Kurek, Krzysztof; Kvaratskheliya, Tengiz; Lacarrere, Daniel; Lafferty, George; Lai, Adriano; Lambert, Dean; Lanfranchi, Gaia; Langenbruch, Christoph; Langhans, Benedikt; Latham, Thomas; Lazzeroni, Cristina; Le Gac, Renaud; van Leerdam, Jeroen; Lees, Jean-Pierre; Lefèvre, Regis; Leflat, Alexander; Lefrançois, Jacques; Lemos Cid, Edgar; Leroy, Olivier; Lesiak, Tadeusz; Leverington, Blake; Li, Yiming; Likhomanenko, Tatiana; Lindner, Rolf; Linn, Christian; Lionetto, Federica; Liu, Bo; Liu, Xuesong; Loh, David; Longstaff, Iain; Lopes, Jose; Lucchesi, Donatella; Lucio Martinez, Miriam; Luo, Haofei; Lupato, Anna; Luppi, Eleonora; Lupton, Oliver; Lusardi, Nicola; Lusiani, Alberto; Lyu, Xiao-Rui; Machefert, Frederic; Maciuc, Florin; Maev, Oleg; Maguire, Kevin; Malde, Sneha; Malinin, Alexander; Manca, Giulia; Mancinelli, Giampiero; Manning, Peter Michael; Mapelli, Alessandro; Maratas, Jan; Marchand, Jean François; Marconi, Umberto; Marin Benito, Carla; Marino, Pietro; Marks, Jörg; Martellotti, Giuseppe; Martin, Morgan; Martinelli, Maurizio; Martinez Santos, Diego; Martinez Vidal, Fernando; Martins Tostes, Danielle; Massacrier, Laure Marie; Massafferri, André; Matev, Rosen; Mathad, Abhijit; Mathe, Zoltan; Matteuzzi, Clara; Mauri, Andrea; Maurin, Brice; Mazurov, Alexander; McCann, Michael; McCarthy, James; McNab, Andrew; McNulty, Ronan; Meadows, Brian; Meier, Frank; Meissner, Marco; Melnychuk, Dmytro; Merk, Marcel; Merli, Andrea; Michielin, Emanuele; Milanes, Diego Alejandro; Minard, Marie-Noelle; Mitzel, Dominik Stefan; Molina Rodriguez, Josue; Monroy, Ignacio Alberto; Monteil, Stephane; Morandin, Mauro; Morawski, Piotr; Mordà, Alessandro; Morello, Michael Joseph; Moron, Jakub; Morris, Adam Benjamin; Mountain, Raymond; Muheim, Franz; Müller, Dominik; Müller, Janine; Müller, Katharina; Müller, Vanessa; Mussini, Manuel; Muster, Bastien; Naik, Paras; Nakada, Tatsuya; Nandakumar, Raja; Nandi, Anita; Nasteva, Irina; Needham, Matthew; Neri, Nicola; Neubert, Sebastian; Neufeld, Niko; Neuner, Max; Nguyen, Anh Duc; Nguyen-Mau, Chung; Niess, Valentin; Nieswand, Simon; Niet, Ramon; Nikitin, Nikolay; Nikodem, Thomas; Novoselov, Alexey; O'Hanlon, Daniel Patrick; Oblakowska-Mucha, Agnieszka; Obraztsov, Vladimir; Ogilvy, Stephen; Okhrimenko, Oleksandr; Oldeman, Rudolf; Onderwater, Gerco; Osorio Rodrigues, Bruno; Otalora Goicochea, Juan Martin; Otto, Adam; Owen, Patrick; Oyanguren, Maria Aranzazu; Palano, Antimo; Palombo, Fernando; Palutan, Matteo; Panman, Jacob; Papanestis, Antonios; Pappagallo, Marco; Pappalardo, Luciano; Pappenheimer, Cheryl; Parker, William; Parkes, Christopher; Passaleva, Giovanni; Patel, Girish; Patel, Mitesh; Patrignani, Claudia; Pearce, Alex; Pellegrino, Antonio; Penso, Gianni; Pepe Altarelli, Monica; Perazzini, Stefano; Perret, Pascal; Pescatore, Luca; Petridis, Konstantinos; Petrolini, Alessandro; Petruzzo, Marco; Picatoste Olloqui, Eduardo; Pietrzyk, Boleslaw; Pikies, Malgorzata; Pinci, Davide; Pistone, Alessandro; Piucci, Alessio; Playfer, Stephen; Plo Casasus, Maximo; Poikela, Tuomas; Polci, Francesco; Poluektov, Anton; Polyakov, Ivan; Polycarpo, Erica; Popov, Alexander; Popov, Dmitry; Popovici, Bogdan; Potterat, Cédric; Price, Eugenia; Price, Joseph David; Prisciandaro, Jessica; Pritchard, Adrian; Prouve, Claire; Pugatch, Valery; Puig Navarro, Albert; Punzi, Giovanni; Qian, Wenbin; Quagliani, Renato; Rachwal, Bartolomiej; Rademacker, Jonas; Rama, Matteo; Ramos Pernas, Miguel; Rangel, Murilo; Raniuk, Iurii; Raven, Gerhard; Redi, Federico; Reichert, Stefanie; dos Reis, Alberto; Renaudin, Victor; Ricciardi, Stefania; Richards, Sophie; Rihl, Mariana; Rinnert, Kurt; Rives Molina, Vincente; Robbe, Patrick; Rodrigues, Ana Barbara; Rodrigues, Eduardo; Rodriguez Lopez, Jairo Alexis; Rodriguez Perez, Pablo; Rogozhnikov, Alexey; Roiser, Stefan; Romanovsky, Vladimir; Romero Vidal, Antonio; Ronayne, John William; Rotondo, Marcello; Ruf, Thomas; Ruiz Valls, Pablo; Saborido Silva, Juan Jose; Sagidova, Naylya; Saitta, Biagio; Salustino Guimaraes, Valdir; Sanchez Mayordomo, Carlos; Sanmartin Sedes, Brais; Santacesaria, Roberta; Santamarina Rios, Cibran; Santimaria, Marco; Santovetti, Emanuele; Sarti, Alessio; Satriano, Celestina; Satta, Alessia; Saunders, Daniel Martin; Savrina, Darya; Schael, Stefan; Schiller, Manuel; Schindler, Heinrich; Schlupp, Maximilian; Schmelling, Michael; Schmelzer, Timon; Schmidt, Burkhard; Schneider, Olivier; Schopper, Andreas; Schubiger, Maxime; Schune, Marie Helene; Schwemmer, Rainer; Sciascia, Barbara; Sciubba, Adalberto; Semennikov, Alexander; Sergi, Antonino; Serra, Nicola; Serrano, Justine; Sestini, Lorenzo; Seyfert, Paul; Shapkin, Mikhail; Shapoval, Illya; Shcheglov, Yury; Shears, Tara; Shekhtman, Lev; Shevchenko, Vladimir; Shires, Alexander; Siddi, Benedetto Gianluca; Silva Coutinho, Rafael; Silva de Oliveira, Luiz Gustavo; Simi, Gabriele; Sirendi, Marek; Skidmore, Nicola; Skwarnicki, Tomasz; Smith, Eluned; Smith, Iwan Thomas; Smith, Jackson; Smith, Mark; Snoek, Hella; Sokoloff, Michael; Soler, Paul; Soomro, Fatima; Souza, Daniel; Souza De Paula, Bruno; Spaan, Bernhard; Spradlin, Patrick; Sridharan, Srikanth; Stagni, Federico; Stahl, Marian; Stahl, Sascha; Stefkova, Slavomira; Steinkamp, Olaf; Stenyakin, Oleg; Stevenson, Scott; Stoica, Sabin; Stone, Sheldon; Storaci, Barbara; Stracka, Simone; Straticiuc, Mihai; Straumann, Ulrich; Sun, Liang; Sutcliffe, William; Swientek, Krzysztof; Swientek, Stefan; Syropoulos, Vasileios; Szczekowski, Marek; Szumlak, Tomasz; T'Jampens, Stephane; Tayduganov, Andrey; Tekampe, Tobias; Tellarini, Giulia; Teubert, Frederic; Thomas, Christopher; Thomas, Eric; van Tilburg, Jeroen; Tisserand, Vincent; Tobin, Mark; Tolk, Siim; Tomassetti, Luca; Tonelli, Diego; Topp-Joergensen, Stig; Tournefier, Edwige; Tourneur, Stephane; Trabelsi, Karim; Traill, Murdo; Tran, Minh Tâm; Tresch, Marco; Trisovic, Ana; Tsaregorodtsev, Andrei; Tsopelas, Panagiotis; Tuning, Niels; Ukleja, Artur; Ustyuzhanin, Andrey; Uwer, Ulrich; Vacca, Claudia; Vagnoni, Vincenzo; Valat, Sebastien; Valenti, Giovanni; Vallier, Alexis; Vazquez Gomez, Ricardo; Vazquez Regueiro, Pablo; Vázquez Sierra, Carlos; Vecchi, Stefania; van Veghel, Maarten; Velthuis, Jaap; Veltri, Michele; Veneziano, Giovanni; Vesterinen, Mika; Viaud, Benoit; Vieira, Daniel; Vieites Diaz, Maria; Vilasis-Cardona, Xavier; Volkov, Vladimir; Vollhardt, Achim; Voong, David; Vorobyev, Alexey; Vorobyev, Vitaly; Voß, Christian; de Vries, Jacco; Waldi, Roland; Wallace, Charlotte; Wallace, Ronan; Walsh, John; Wang, Jianchun; Ward, David; Watson, Nigel; Websdale, David; Weiden, Andreas; Whitehead, Mark; Wicht, Jean; Wilkinson, Guy; Wilkinson, Michael; Williams, Mark Richard James; Williams, Matthew; Williams, Mike; Williams, Timothy; Wilson, Fergus; Wimberley, Jack; Wishahi, Julian; Wislicki, Wojciech; Witek, Mariusz; Wormser, Guy; Wotton, Stephen; Wraight, Kenneth; Wright, Simon; Wyllie, Kenneth; Xie, Yuehong; Xu, Zhirui; Yang, Zhenwei; Yin, Hang; Yu, Jiesheng; Yuan, Xuhao; Yushchenko, Oleg; Zangoli, Maria; Zavertyaev, Mikhail; Zhang, Liming; Zhang, Yanxi; Zhelezov, Alexey; Zheng, Yangheng; Zhokhov, Anatoly; Zhong, Liang; Zhukov, Valery; Zucchelli, Stefano

    2016-06-21

    A binned Dalitz plot analysis of the decays $B^0 \\to D K^{\\ast 0}$, with $D \\to K_{S}^{0} \\pi^{+} \\pi^{-}$ and $D \\to K_{S}^{0} K^{+} K^{-}$, is performed to measure the observables $x_\\pm$ and $y_\\pm$, which are related to the CKM angle $\\gamma$ and the hadronic parameters of the decays. The $D$ decay strong phase variation over the Dalitz plot is taken from measurements performed at the CLEO-c experiment, making the analysis independent of the $D$ decay model. With a sample of proton-proton collision data, corresponding to an integrated luminosity of $3.0\\,\\rm{fb}^{-1}$, collected by the LHCb experiment, the values of the $CP$ violation parameters are found to be $x_+ = 0.05 \\pm 0.35 \\pm 0.02$, $x_-=-0.31\\pm 0.20 \\pm 0.04$, $y_+=-0.81\\pm 0.28\\pm 0.06$ and $y_-=0.31\\pm 0.21 \\pm 0.05$, where the first uncertainties are statistical and the second systematic. These observables correspond to values $\\gamma$ = $(71 \\pm 20)^\\circ$, $r_{B^0} = 0.56\\pm 0.17$ and $\\delta_{B^0} = (204\\,^{+21}_{-20})^\\circ$. The parame...

  11. Fanconi syndrome

    Science.gov (United States)

    De Toni-Fanconi syndrome ... Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown. Common causes of Fanconi syndrome in ...

  12. Duane Syndrome

    Science.gov (United States)

    ... Frequently Asked Questions Español Condiciones Chinese Conditions Duane Syndrome En Español Read in Chinese What is Duane Syndrome? Duane syndrome, also called Duane retraction syndrome (DRS), ...

  13. Obesity and Hyperglycemia in Korean Men with Klinefelter Syndrome: The Korean Endocrine Society Registry

    Directory of Open Access Journals (Sweden)

    Seung Jin Han

    2016-12-01

    Full Text Available BackgroundThe aim of this study was to investigate the prevalence of obesity in Korean men with Klinefelter syndrome (KS and the associated risk factors for obesity and hyperglycemia.MethodsData were collected retrospectively from medical records from 11 university hospitals in Korea between 1994 and 2014. Subjects aged ≥18 years with newly diagnosed KS were enrolled. The following parameters were recorded at baseline before treatment: chief complaint, height, weight, fasting glucose level, lipid panel, blood pressure, testosterone, luteinizing hormone, follicle-stimulating hormone, karyotyping patterns, and history of hypertension, diabetes, and dyslipidemia.ResultsData were analyzed from 376 of 544 initially enrolled patients. The rate of the 47 XXY chromosomal pattern was 94.1%. The prevalence of obesity (body mass index ≥25 kg/m2 in Korean men with KS was 42.6%. The testosterone level was an independent risk factor for obesity and hyperglycemia.ConclusionObesity is common in Korean men with KS. Hypogonadism in patients with KS was associated with obesity and hyperglycemia.

  14. Crossmodal processing of emotions in alcohol-dependence and Korsakoff syndrome.

    Science.gov (United States)

    Brion, Mélanie; D'Hondt, Fabien; Lannoy, Séverine; Pitel, Anne-Lise; Davidoff, Donald A; Maurage, Pierre

    2017-09-01

    Decoding emotional information from faces and voices is crucial for efficient interpersonal communication. Emotional decoding deficits have been found in alcohol-dependence (ALC), particularly in crossmodal situations (with simultaneous stimulations from different modalities), but are still underexplored in Korsakoff syndrome (KS). The aim of this study is to determine whether the continuity hypothesis, postulating a gradual worsening of cognitive and brain impairments from ALC to KS, is valid for emotional crossmodal processing. Sixteen KS, 17 ALC and 19 matched healthy controls (CP) had to detect the emotion (anger or happiness) displayed by auditory, visual or crossmodal auditory-visual stimuli. Crossmodal stimuli were either emotionally congruent (leading to a facilitation effect, i.e. enhanced performance for crossmodal condition compared to unimodal ones) or incongruent (leading to an interference effect, i.e. decreased performance for crossmodal condition due to discordant information across modalities). Reaction times and accuracy were recorded. Crossmodal integration for congruent information was dampened only in ALC, while both ALC and KS demonstrated, compared to CP, decreased performance for decoding emotional facial expressions in the incongruent condition. The crossmodal integration appears impaired in ALC but preserved in KS. Both alcohol-related disorders present an increased interference effect. These results show the interest of more ecological designs, using crossmodal stimuli, to explore emotional decoding in alcohol-related disorders. They also suggest that the continuum hypothesis cannot be generalised to emotional decoding abilities.

  15. Translation and validation of the new version of the Knee Society Score - The 2011 KS Score - into Brazilian Portuguese.

    Science.gov (United States)

    Silva, Adriana Lucia Pastore E; Croci, Alberto Tesconi; Gobbi, Riccardo Gomes; Hinckel, Betina Bremer; Pecora, José Ricardo; Demange, Marco Kawamura

    2017-01-01

    Translation, cultural adaptation, and validation of the new version of the Knee Society Score - The 2011 KS Score - into Brazilian Portuguese and verification of its measurement properties, reproducibility, and validity. In 2012, the new version of the Knee Society Score was developed and validated. This scale comprises four separate subscales: (a) objective knee score (seven items: 100 points); (b) patient satisfaction score (five items: 40 points); (c) patient expectations score (three items: 15 points); and (d) functional activity score (19 items: 100 points). A total of 90 patients aged 55-85 years were evaluated in a clinical cross-sectional study. The pre-operative translated version was applied to patients with TKA referral, and the post-operative translated version was applied to patients who underwent TKA. Each patient answered the same questionnaire twice and was evaluated by two experts in orthopedic knee surgery. Evaluations were performed pre-operatively and three, six, or 12 months post-operatively. The reliability of the questionnaire was evaluated using the intraclass correlation coefficient (ICC) between the two applications. Internal consistency was evaluated using Cronbach's alpha. The ICC found no difference between the means of the pre-operative, three-month, and six-month post-operative evaluations between sub-scale items. The Brazilian Portuguese version of The 2011 KS Score is a valid and reliable instrument for objective and subjective evaluation of the functionality of Brazilian patients who undergo TKA and revision TKA.

  16. Isolation and identification of Aeromonas caviae strain KS-1 as TBTC- and lead-resistant estuarine bacteria.

    Science.gov (United States)

    Shamim, Kashif; Naik, Milind Mohan; Pandey, Anju; Dubey, Santosh Kumar

    2013-06-01

    Tributyltin chloride (TBTC)- and lead-resistant estuarine bacterium from Mandovi estuary, Goa, India was isolated and identified as Aeromonas caviae strain KS-1 based on biochemical characteristics and FAME analysis. It tolerates TBTC and lead up to 1.0 and 1.4 mM, respectively, in the minimal salt medium (MSM) supplemented with 0.4 % glucose. Scanning electron microscopy clearly revealed a unique morphological pattern in the form of long inter-connected chains of bacterial cells on exposure to 1 mM TBTC, whereas cells remained unaltered in presence of 1.4 mM Pb(NO₃)₂ but significant biosorption of lead (8 %) on the cell surface of this isolate was clearly revealed by scanning electron microscopy coupled with energy dispersive X-ray spectroscopy. SDS-PAGE analysis of whole-cell proteins of this lead-resistant isolate interestingly demonstrated three lead-induced proteins with molecular mass of 15.7, 16.9 and 32.4 kDa, respectively, when bacterial cells were grown under the stress of 1.4 mM Pb (NO₃)₂. This clearly demonstrated their possible involvement exclusively in lead resistance. A. caviae strain KS-1 also showed tolerance to several other heavy metals, viz. zinc, cadmium, copper and mercury. Therefore, we can employ this TBTC and lead-resistant bacterial isolate for lead bioremediation and also for biomonitoring TBTC from lead and TBTC contaminated environment.

  17. Extracting $\\gamma$ from $B_{s(d)} \\to J/\\psi K_{S}$ and $B_{d(s)} \\to D^{+}_{d(s)} D^{-}_{d(s)}$

    CERN Document Server

    Fleischer, Robert

    1999-01-01

    A completely general parametrization of the time-dependent decay rates of the modes $B_s\\to J/\\psi K_S$ and $B_d\\to J/\\psi K_S$ is given, which are related to each other through the $U$-spin flavour symmetry of strong interactions. Owing to the interference of current--current and penguin processes, the $B_s\\to J/\\psi K_S$ observables probe the angle $\\gamma$ of the unitarity triangle. Using the $U$-spin symmetry, the overall normalization of the $B_s\\to J/\\psi K_S$ rate can be fixed with the help of the CP-averaged $B_d\\to J/\\psi K_{\\rm S}$ rate, providing a new strategy to determine $\\gamma$. This extraction of $\\gamma$ is not affected by any final-state-interaction effects, and its theoretical accuracy is only limited by $U$-spin-breaking corrections. As a by-product, this strategy allows us to take into account also the penguin effects in the determination of $\\beta$ from $B_d\\to J/\\psi K_S$, which are presumably very small, and to predict the direct CP asymmetry arising in this mode. An analogous strateg...

  18. Analysis study of the condensation heat transfer coefficient in the presence of noncondensable on PCCS vertical condenser tube using MARS-KS

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Dong jae; Jang, Yeong jun; Lee, Yeon-Gun [Jeju National University, Jeju (Korea, Republic of); Kim, Sin [Chung-Ang University, Seoul (Korea, Republic of)

    2015-10-15

    The Passive Containment Cooling System (PCCS) to be introduced in advanced LWRs removes released energy to an external heat sink by a naturally driven flow. Containment through the condensation heat transfer phenomenon in the event of the loss of coolant accident (LOCA) or main steam line break (MSLB). As the released steam pressurizes the containment, the PCCS will activate to transport the decay heat In this study, a numerical analysis of the condensation heat transfer coefficients on the PCCS condenser tube is conducted using the MARS-KS code. The condensation heat transfer coefficients are obtained from JNU condensation tests performed on a 1000 long and 40 mm O.D. tube. The analysis condition covers 2 and 4 bar for the air mass fraction ranging from 0.1 to 0.8. The JNU single vertical condensation experimental results, Uchida's and Dehbi's correlation compared with the MARS-KS code's results at 2 and 4 bar. Experimental results and MARS-KS predicted heat transfer coefficient is different from the thermal resistances and Wall subcooling. An average relative error is 18.8% and 15% at 2 and 4 bar, respectively. Uchida's correlation is considered the noncondensable gas mass fraction only. Therefore, that is lower than MARS-KS results at 4 bar. Dehbi's correlation affected by ratio of the height-to-diameter, so its results are higher condensation heat transfer coefficient than MARS-KS predicted results.

  19. Hamartomatous polyposis syndromes

    DEFF Research Database (Denmark)

    Jelsig, Anne Marie; Qvist, Niels; Brusgaard, Klaus

    2014-01-01

    Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes such as ......Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes...

  20. Evaluation of in-core neutron flux and temperature field measurements during the second period of power commissioning of the KS-150 reactor

    International Nuclear Information System (INIS)

    Rana, S.B.; Pecho, J.

    1975-01-01

    The in-core flux mapping system in the KS-150 reactor using mapping fuel elements with self-powered detectors is described. Experimental data evaluation using the Fourier analysis and determination of important operation parameters from the detectors and temperature field distribution using thermocouples for measuring coolant outlet temperatures and fuel temperatures are given. The DPZ-1 detectors used, mapping fuel elements and the method of signal registration are described. The results of operation of mapping fuel elements during the 2nd period of the KS-150 reactor commissioning are given. (author)

  1. Üks kaame naine väikses kambris ... : [luuletused] / Gyula Illyés ; tlk. Sander Liivak, Ene Asu-Õunas

    Index Scriptorium Estoniae

    Illyés, Gyula

    2004-01-01

    Sisu: Üks kaame naine väikses kambris ... ; Sa ei saa pageda ; Purukslöödud vägi ; Mäe otsas puhkan ... ; Kodumaa kõrgel ; Magav naine ; Doleo, ergo surm ; Bart̤k ; Üks lause vägivallast ; Jalutuskäik varjuga ; Tuuline öö ; Liginev külm ; Viltune puri ; Näod ; Aovalgus ; Korraloomine ; Värdjate sünd ; Charoni vene ; Tõusul ; Leegihingaja ; Lähenev vaikus ; Teekond videvikul ; Pärg. Andmeid autori kohta lk. 87-88

  2. Status of the measurement of KS → πeν branching ratio and lepton charge asymmetry with the KLOE detector

    Directory of Open Access Journals (Sweden)

    Kamińska Daria

    2014-01-01

    Full Text Available We present the current status of the analysis of about 1.7 billion KS KL pair events collected at DAΦNE with the KLOE detector to determine the branching ratio of KS → πeν decay and the lepton charge asymmetry. This sample is ∼ 4 times larger in statistics than the one used in the previous most precise result, from KLOE as well, allowing us to improve the accuracy on the measurement and related tests of CPT symmetry and ∆S = ∆Q rule.

  3. Beam-Energy Dependence of Directed Flow of Λ , Λ ¯, K±, Ks0, and ϕ in Au +Au Collisions

    Science.gov (United States)

    Adamczyk, L.; Adams, J. R.; Adkins, J. K.; Agakishiev, G.; Aggarwal, M. M.; Ahammed, Z.; Ajitanand, N. N.; Alekseev, I.; Anderson, D. M.; Aoyama, R.; Aparin, A.; Arkhipkin, D.; Aschenauer, E. C.; Ashraf, M. U.; Attri, A.; Averichev, G. S.; Bai, X.; Bairathi, V.; Barish, K.; Behera, A.; Bellwied, R.; Bhasin, A.; Bhati, A. K.; Bhattarai, P.; Bielcik, J.; Bielcikova, J.; Bland, L. C.; Bordyuzhin, I. G.; Bouchet, J.; Brandenburg, J. D.; Brandin, A. V.; Brown, D.; Bunzarov, I.; Butterworth, J.; Caines, H.; Calderón de la Barca Sánchez, M.; Campbell, J. M.; Cebra, D.; Chakaberia, I.; Chaloupka, P.; Chang, Z.; Chankova-Bunzarova, N.; Chatterjee, A.; Chattopadhyay, S.; Chen, X.; Chen, J. H.; Chen, X.; Cheng, J.; Cherney, M.; Christie, W.; Contin, G.; Crawford, H. J.; Das, S.; De Silva, L. C.; Dedovich, T. G.; Deng, J.; Derevschikov, A. A.; Didenko, L.; Dilks, C.; Dong, X.; Drachenberg, J. L.; Draper, J. E.; Dunkelberger, L. E.; Dunlop, J. C.; Efimov, L. G.; Elsey, N.; Engelage, J.; Eppley, G.; Esha, R.; Esumi, S.; Evdokimov, O.; Ewigleben, J.; Eyser, O.; Fatemi, R.; Fazio, S.; Federic, P.; Federicova, P.; Fedorisin, J.; Feng, Z.; Filip, P.; Finch, E.; Fisyak, Y.; Flores, C. E.; Fujita, J.; Fulek, L.; Gagliardi, C. A.; Garand, D.; Geurts, F.; Gibson, A.; Girard, M.; Grosnick, D.; Gunarathne, D. S.; Guo, Y.; Gupta, A.; Gupta, S.; Guryn, W.; Hamad, A. I.; Hamed, A.; Harlenderova, A.; Harris, J. W.; He, L.; Heppelmann, S.; Heppelmann, S.; Hirsch, A.; Horvat, S.; Huang, X.; Huang, B.; Huang, T.; Huang, H. Z.; Humanic, T. J.; Huo, P.; Igo, G.; Jacobs, W. W.; Jentsch, A.; Jia, J.; Jiang, K.; Jowzaee, S.; Judd, E. G.; Kabana, S.; Kalinkin, D.; Kang, K.; Kapukchyan, D.; Kauder, K.; Ke, H. W.; Keane, D.; Kechechyan, A.; Khan, Z.; Kikoła, D. P.; Kim, C.; Kisel, I.; Kisiel, A.; Kochenda, L.; Kocmanek, M.; Kollegger, T.; Kosarzewski, L. K.; Kraishan, A. F.; Krauth, L.; Kravtsov, P.; Krueger, K.; Kulathunga, N.; Kumar, L.; Kvapil, J.; Kwasizur, J. H.; Lacey, R.; Landgraf, J. M.; Landry, K. D.; Lauret, J.; Lebedev, A.; Lednicky, R.; Lee, J. H.; Li, C.; Li, X.; Li, Y.; Li, W.; Lidrych, J.; Lin, T.; Lisa, M. A.; Liu, P.; Liu, H.; Liu, Y.; Liu, F.; Ljubicic, T.; Llope, W. J.; Lomnitz, M.; Longacre, R. S.; Luo, S.; Luo, X.; Ma, Y. G.; Ma, L.; Ma, R.; Ma, G. L.; Magdy, N.; Majka, R.; Mallick, D.; Margetis, S.; Markert, C.; Matis, H. S.; Meehan, K.; Mei, J. C.; Miller, Z. W.; Minaev, N. G.; Mioduszewski, S.; Mishra, D.; Mizuno, S.; Mohanty, B.; Mondal, M. M.; Morozov, D. A.; Mustafa, M. K.; Nasim, Md.; Nayak, T. K.; Nelson, J. M.; Nie, M.; Nigmatkulov, G.; Niida, T.; Nogach, L. V.; Nonaka, T.; Nurushev, S. B.; Odyniec, G.; Ogawa, A.; Oh, K.; Okorokov, V. A.; Olvitt, D.; Page, B. S.; Pak, R.; Pandit, Y.; Panebratsev, Y.; Pawlik, B.; Pei, H.; Perkins, C.; Pile, P.; Pluta, J.; Poniatowska, K.; Porter, J.; Posik, M.; Pruthi, N. K.; Przybycien, M.; Putschke, J.; Qiu, H.; Quintero, A.; Ramachandran, S.; Ray, R. L.; Reed, R.; Rehbein, M. J.; Ritter, H. G.; Roberts, J. B.; Rogachevskiy, O. V.; Romero, J. L.; Roth, J. D.; Ruan, L.; Rusnak, J.; Rusnakova, O.; Sahoo, N. R.; Sahu, P. K.; Salur, S.; Sandweiss, J.; Saur, M.; Schambach, J.; Schmah, A. M.; Schmidke, W. B.; Schmitz, N.; Schweid, B. R.; Seger, J.; Sergeeva, M.; Seto, R.; Seyboth, P.; Shah, N.; Shahaliev, E.; Shanmuganathan, P. V.; Shao, M.; Sharma, A.; Sharma, M. K.; Shen, W. Q.; Shi, S. S.; Shi, Z.; Shou, Q. Y.; Sichtermann, E. P.; Sikora, R.; Simko, M.; Singha, S.; Skoby, M. J.; Smirnov, N.; Smirnov, D.; Solyst, W.; Song, L.; Sorensen, P.; Spinka, H. M.; Srivastava, B.; Stanislaus, T. D. S.; Strikhanov, M.; Stringfellow, B.; Suaide, A. A. P.; Sugiura, T.; Sumbera, M.; Summa, B.; Sun, Y.; Sun, X. M.; Sun, X.; Surrow, B.; Svirida, D. N.; Tang, Z.; Tang, A. H.; Taranenko, A.; Tarnowsky, T.; Tawfik, A.; Thäder, J.; Thomas, J. H.; Timmins, A. R.; Tlusty, D.; Todoroki, T.; Tokarev, M.; Trentalange, S.; Tribble, R. E.; Tribedy, P.; Tripathy, S. K.; Trzeciak, B. A.; Tsai, O. D.; Ullrich, T.; Underwood, D. G.; Upsal, I.; Van Buren, G.; van Nieuwenhuizen, G.; Vasiliev, A. N.; Videbæk, F.; Vokal, S.; Voloshin, S. A.; Vossen, A.; Wang, G.; Wang, Y.; Wang, F.; Wang, Y.; Webb, J. C.; Webb, G.; Wen, L.; Westfall, G. D.; Wieman, H.; Wissink, S. W.; Witt, R.; Wu, Y.; Xiao, Z. G.; Xie, G.; Xie, W.; Xu, J.; Xu, Z.; Xu, Q. H.; Xu, Y. F.; Xu, N.; Yang, S.; Yang, Y.; Yang, C.; Yang, Q.; Ye, Z.; Ye, Z.; Yi, L.; Yip, K.; Yoo, I.-K.; Yu, N.; Zbroszczyk, H.; Zha, W.; Zhang, Z.; Zhang, J. B.; Zhang, J.; Zhang, S.; Zhang, Y.; Zhang, X. P.; Zhang, J.; Zhang, S.; Zhao, J.; Zhong, C.; Zhou, C.; Zhou, L.; Zhu, X.; Zhu, Z.; Zyzak, M.; STAR Collaboration

    2018-02-01

    Rapidity-odd directed-flow measurements at midrapidity are presented for Λ , Λ ¯, K±, Ks0, and ϕ at √{sN N }=7.7 , 11.5, 14.5, 19.6, 27, 39, 62.4, and 200 GeV in Au +Au collisions recorded by the Solenoidal Tracker detector at the Relativistic Heavy Ion Collider. These measurements greatly expand the scope of data available to constrain models with differing prescriptions for the equation of state of quantum chromodynamics. Results show good sensitivity for testing a picture where flow is assumed to be imposed before hadron formation and the observed particles are assumed to form via coalescence of constituent quarks. The pattern of departure from a coalescence-inspired sum rule can be a valuable new tool for probing the collision dynamics.

  4. Model-independent measurement of mixing parameters in $D^0 \\to K_S^0 \\pi^+ \\pi^-$ decays

    CERN Document Server

    Aaij, Roel; Adeva, Bernardo; Adinolfi, Marco; Affolder, Anthony; Ajaltouni, Ziad; Akar, Simon; Albrecht, Johannes; Alessio, Federico; Alexander, Michael; Ali, Suvayu; Alkhazov, Georgy; Alvarez Cartelle, Paula; Alves Jr, Antonio Augusto; Amato, Sandra; Amerio, Silvia; Amhis, Yasmine; An, Liupan; Anderlini, Lucio; Anderson, Jonathan; Andreassi, Guido; Andreotti, Mirco; Andrews, Jason; Appleby, Robert; Aquines Gutierrez, Osvaldo; Archilli, Flavio; d'Argent, Philippe; Artamonov, Alexander; Artuso, Marina; Aslanides, Elie; Auriemma, Giulio; Baalouch, Marouen; Bachmann, Sebastian; Back, John; Badalov, Alexey; Baesso, Clarissa; Baldini, Wander; Barlow, Roger; Barschel, Colin; Barsuk, Sergey; Barter, William; Batozskaya, Varvara; Battista, Vincenzo; Bay, Aurelio; Beaucourt, Leo; Beddow, John; Bedeschi, Franco; Bediaga, Ignacio; Bel, Lennaert; Bellee, Violaine; Belloli, Nicoletta; Belyaev, Ivan; Ben-Haim, Eli; Bencivenni, Giovanni; Benson, Sean; Benton, Jack; Berezhnoy, Alexander; Bernet, Roland; Bertolin, Alessandro; Bettler, Marc-Olivier; van Beuzekom, Martinus; Bien, Alexander; Bifani, Simone; Billoir, Pierre; Bird, Thomas; Birnkraut, Alex; Bizzeti, Andrea; Blake, Thomas; Blanc, Frédéric; Blouw, Johan; Blusk, Steven; Bocci, Valerio; Bondar, Alexander; Bondar, Nikolay; Bonivento, Walter; Borghi, Silvia; Borsato, Martino; Bowcock, Themistocles; Bowen, Espen Eie; Bozzi, Concezio; Braun, Svende; Britsch, Markward; Britton, Thomas; Brodzicka, Jolanta; Brook, Nicholas; Buchanan, Emma; Burr, Christopher; Bursche, Albert; Buytaert, Jan; Cadeddu, Sandro; Calabrese, Roberto; Calvi, Marta; Calvo Gomez, Miriam; Campana, Pierluigi; Campora Perez, Daniel; Capriotti, Lorenzo; Carbone, Angelo; Carboni, Giovanni; Cardinale, Roberta; Cardini, Alessandro; Carniti, Paolo; Carson, Laurence; Carvalho Akiba, Kazuyoshi; Casse, Gianluigi; Cassina, Lorenzo; Castillo Garcia, Lucia; Cattaneo, Marco; Cauet, Christophe; Cavallero, Giovanni; Cenci, Riccardo; Charles, Matthew; Charpentier, Philippe; Chefdeville, Maximilien; Chen, Shanzhen; Cheung, Shu-Faye; Chiapolini, Nicola; Chrzaszcz, Marcin; Cid Vidal, Xabier; Ciezarek, Gregory; Clarke, Peter; Clemencic, Marco; Cliff, Harry; Closier, Joel; Coco, Victor; Cogan, Julien; Cogneras, Eric; Cogoni, Violetta; Cojocariu, Lucian; Collazuol, Gianmaria; Collins, Paula; Comerma-Montells, Albert; Contu, Andrea; Cook, Andrew; Coombes, Matthew; Coquereau, Samuel; Corti, Gloria; Corvo, Marco; Couturier, Benjamin; Cowan, Greig; Craik, Daniel Charles; Crocombe, Andrew; Cruz Torres, Melissa Maria; Cunliffe, Samuel; Currie, Robert; D'Ambrosio, Carmelo; Dall'Occo, Elena; Dalseno, Jeremy; David, Pieter; Davis, Adam; De Aguiar Francisco, Oscar; De Bruyn, Kristof; De Capua, Stefano; De Cian, Michel; De Miranda, Jussara; De Paula, Leandro; De Simone, Patrizia; Dean, Cameron Thomas; Decamp, Daniel; Deckenhoff, Mirko; Del Buono, Luigi; Déléage, Nicolas; Demmer, Moritz; Derkach, Denis; Deschamps, Olivier; Dettori, Francesco; Dey, Biplab; Di Canto, Angelo; Di Ruscio, Francesco; Dijkstra, Hans; Donleavy, Stephanie; Dordei, Francesca; Dorigo, Mirco; Dosil Suárez, Alvaro; Dossett, David; Dovbnya, Anatoliy; Dreimanis, Karlis; Dufour, Laurent; Dujany, Giulio; Dupertuis, Frederic; Durante, Paolo; Dzhelyadin, Rustem; Dziurda, Agnieszka; Dzyuba, Alexey; Easo, Sajan; Egede, Ulrik; Egorychev, Victor; Eidelman, Semen; Eisenhardt, Stephan; Eitschberger, Ulrich; Ekelhof, Robert; Eklund, Lars; El Rifai, Ibrahim; Elsasser, Christian; Ely, Scott; Esen, Sevda; Evans, Hannah Mary; Evans, Timothy; Falabella, Antonio; Färber, Christian; Farley, Nathanael; Farry, Stephen; Fay, Robert; Ferguson, Dianne; Fernandez Albor, Victor; Ferrari, Fabio; Ferreira Rodrigues, Fernando; Ferro-Luzzi, Massimiliano; Filippov, Sergey; Fiore, Marco; Fiorini, Massimiliano; Firlej, Miroslaw; Fitzpatrick, Conor; Fiutowski, Tomasz; Fohl, Klaus; Fol, Philip; Fontana, Marianna; Fontanelli, Flavio; Forshaw, Dean Charles; Forty, Roger; Frank, Markus; Frei, Christoph; Frosini, Maddalena; Fu, Jinlin; Furfaro, Emiliano; Gallas Torreira, Abraham; Galli, Domenico; Gallorini, Stefano; Gambetta, Silvia; Gandelman, Miriam; Gandini, Paolo; Gao, Yuanning; García Pardiñas, Julián; Garra Tico, Jordi; Garrido, Lluis; Gascon, David; Gaspar, Clara; Gauld, Rhorry; Gavardi, Laura; Gazzoni, Giulio; Gerick, David; Gersabeck, Evelina; Gersabeck, Marco; Gershon, Timothy; Ghez, Philippe; Gianì, Sebastiana; Gibson, Valerie; Girard, Olivier Göran; Giubega, Lavinia-Helena; Gligorov, V.V.; Göbel, Carla; Golubkov, Dmitry; Golutvin, Andrey; Gomes, Alvaro; Gotti, Claudio; Grabalosa Gándara, Marc; Graciani Diaz, Ricardo; Granado Cardoso, Luis Alberto; Graugés, Eugeni; Graverini, Elena; Graziani, Giacomo; Grecu, Alexandru; Greening, Edward; Gregson, Sam; Griffith, Peter; Grillo, Lucia; Grünberg, Oliver; Gui, Bin; Gushchin, Evgeny; Guz, Yury; Gys, Thierry; Hadavizadeh, Thomas; Hadjivasiliou, Christos; Haefeli, Guido; Haen, Christophe; Haines, Susan; Hall, Samuel; Hamilton, Brian; Han, Xiaoxue; Hansmann-Menzemer, Stephanie; Harnew, Neville; Harnew, Samuel; Harrison, Jonathan; He, Jibo; Head, Timothy; Heijne, Veerle; Hennessy, Karol; Henrard, Pierre; Henry, Louis; van Herwijnen, Eric; Heß, Miriam; Hicheur, Adlène; Hill, Donal; Hoballah, Mostafa; Hombach, Christoph; Hulsbergen, Wouter; Humair, Thibaud; Hussain, Nazim; Hutchcroft, David; Hynds, Daniel; Idzik, Marek; Ilten, Philip; Jacobsson, Richard; Jaeger, Andreas; Jalocha, Pawel; Jans, Eddy; Jawahery, Abolhassan; Jing, Fanfan; John, Malcolm; Johnson, Daniel; Jones, Christopher; Joram, Christian; Jost, Beat; Jurik, Nathan; Kandybei, Sergii; Kanso, Walaa; Karacson, Matthias; Karbach, Moritz; Karodia, Sarah; Kecke, Matthieu; Kelsey, Matthew; Kenyon, Ian; Kenzie, Matthew; Ketel, Tjeerd; Khairullin, Egor; Khanji, Basem; Khurewathanakul, Chitsanu; Klaver, Suzanne; Klimaszewski, Konrad; Kochebina, Olga; Kolpin, Michael; Komarov, Ilya; Koopman, Rose; Koppenburg, Patrick; Kozeiha, Mohamad; Kravchuk, Leonid; Kreplin, Katharina; Kreps, Michal; Krocker, Georg; Krokovny, Pavel; Kruse, Florian; Krzemien, Wojciech; Kucewicz, Wojciech; Kucharczyk, Marcin; Kudryavtsev, Vasily; Kuonen, Axel Kevin; Kurek, Krzysztof; Kvaratskheliya, Tengiz; Lacarrere, Daniel; Lafferty, George; Lai, Adriano; Lambert, Dean; Lanfranchi, Gaia; Langenbruch, Christoph; Langhans, Benedikt; Latham, Thomas; Lazzeroni, Cristina; Le Gac, Renaud; van Leerdam, Jeroen; Lees, Jean-Pierre; Lefèvre, Regis; Leflat, Alexander; Lefrançois, Jacques; Lemos Cid, Edgar; Leroy, Olivier; Lesiak, Tadeusz; Leverington, Blake; Li, Yiming; Likhomanenko, Tatiana; Liles, Myfanwy; Lindner, Rolf; Linn, Christian; Lionetto, Federica; Liu, Bo; Liu, Xuesong; Loh, David; Longstaff, Iain; Lopes, Jose; Lucchesi, Donatella; Lucio Martinez, Miriam; Luo, Haofei; Lupato, Anna; Luppi, Eleonora; Lupton, Oliver; Lusiani, Alberto; Machefert, Frederic; Maciuc, Florin; Maev, Oleg; Maguire, Kevin; Malde, Sneha; Malinin, Alexander; Manca, Giulia; Mancinelli, Giampiero; Manning, Peter Michael; Mapelli, Alessandro; Maratas, Jan; Marchand, Jean François; Marconi, Umberto; Marin Benito, Carla; Marino, Pietro; Marks, Jörg; Martellotti, Giuseppe; Martin, Morgan; Martinelli, Maurizio; Martinez Santos, Diego; Martinez Vidal, Fernando; Martins Tostes, Danielle; Massafferri, André; Matev, Rosen; Mathad, Abhijit; Mathe, Zoltan; Matteuzzi, Clara; Mauri, Andrea; Maurin, Brice; Mazurov, Alexander; McCann, Michael; McCarthy, James; McNab, Andrew; McNulty, Ronan; Meadows, Brian; Meier, Frank; Meissner, Marco; Melnychuk, Dmytro; Merk, Marcel; Michielin, Emanuele; Milanes, Diego Alejandro; Minard, Marie-Noelle; Mitzel, Dominik Stefan; Molina Rodriguez, Josue; Monroy, Ignacio Alberto; Monteil, Stephane; Morandin, Mauro; Morawski, Piotr; Mordà, Alessandro; Morello, Michael Joseph; Moron, Jakub; Morris, Adam Benjamin; Mountain, Raymond; Muheim, Franz; Müller, Dominik; Müller, Janine; Müller, Katharina; Müller, Vanessa; Mussini, Manuel; Muster, Bastien; Naik, Paras; Nakada, Tatsuya; Nandakumar, Raja; Nandi, Anita; Nasteva, Irina; Needham, Matthew; Neri, Nicola; Neubert, Sebastian; Neufeld, Niko; Neuner, Max; Nguyen, Anh Duc; Nguyen, Thi-Dung; Nguyen-Mau, Chung; Niess, Valentin; Niet, Ramon; Nikitin, Nikolay; Nikodem, Thomas; Novoselov, Alexey; O'Hanlon, Daniel Patrick; Oblakowska-Mucha, Agnieszka; Obraztsov, Vladimir; Ogilvy, Stephen; Okhrimenko, Oleksandr; Oldeman, Rudolf; Onderwater, Gerco; Osorio Rodrigues, Bruno; Otalora Goicochea, Juan Martin; Otto, Adam; Owen, Patrick; Oyanguren, Maria Aranzazu; Palano, Antimo; Palombo, Fernando; Palutan, Matteo; Panman, Jacob; Papanestis, Antonios; Pappagallo, Marco; Pappalardo, Luciano; Pappenheimer, Cheryl; Parker, William; Parkes, Christopher; Passaleva, Giovanni; Patel, Girish; Patel, Mitesh; Patrignani, Claudia; Pearce, Alex; Pellegrino, Antonio; Penso, Gianni; Pepe Altarelli, Monica; Perazzini, Stefano; Perret, Pascal; Pescatore, Luca; Petridis, Konstantinos; Petrolini, Alessandro; Petruzzo, Marco; Picatoste Olloqui, Eduardo; Pietrzyk, Boleslaw; Pilař, Tomas; Pinci, Davide; Pistone, Alessandro; Piucci, Alessio; Playfer, Stephen; Plo Casasus, Maximo; Poikela, Tuomas; Polci, Francesco; Poluektov, Anton; Polyakov, Ivan; Polycarpo, Erica; Popov, Alexander; Popov, Dmitry; Popovici, Bogdan; Potterat, Cédric; Price, Eugenia; Price, Joseph David; Prisciandaro, Jessica; Pritchard, Adrian; Prouve, Claire; Pugatch, Valery; Puig Navarro, Albert; Punzi, Giovanni; Qian, Wenbin; Quagliani, Renato; Rachwal, Bartolomiej; Rademacker, Jonas; Rama, Matteo; Rangel, Murilo; Raniuk, Iurii; Rauschmayr, Nathalie; Raven, Gerhard; Redi, Federico; Reichert, Stefanie; Reid, Matthew; dos Reis, Alberto; Ricciardi, Stefania; Richards, Sophie; Rihl, Mariana; Rinnert, Kurt; Rives Molina, Vincente; Robbe, Patrick; Rodrigues, Ana Barbara; Rodrigues, Eduardo; Rodriguez Lopez, Jairo Alexis; Rodriguez Perez, Pablo; Roiser, Stefan; Romanovsky, Vladimir; Romero Vidal, Antonio; Ronayne, John William; Rotondo, Marcello; Rouvinet, Julien; Ruf, Thomas; Ruiz Valls, Pablo; Saborido Silva, Juan Jose; Sagidova, Naylya; Sail, Paul; Saitta, Biagio; Salustino Guimaraes, Valdir; Sanchez Mayordomo, Carlos; Sanmartin Sedes, Brais; Santacesaria, Roberta; Santamarina Rios, Cibran; Santimaria, Marco; Santovetti, Emanuele; Sarti, Alessio; Satriano, Celestina; Satta, Alessia; Saunders, Daniel Martin; Savrina, Darya; Schiller, Manuel; Schindler, Heinrich; Schlupp, Maximilian; Schmelling, Michael; Schmelzer, Timon; Schmidt, Burkhard; Schneider, Olivier; Schopper, Andreas; Schubiger, Maxime; Schune, Marie Helene; Schwemmer, Rainer; Sciascia, Barbara; Sciubba, Adalberto; Semennikov, Alexander; Serra, Nicola; Serrano, Justine; Sestini, Lorenzo; Seyfert, Paul; Shapkin, Mikhail; Shapoval, Illya; Shcheglov, Yury; Shears, Tara; Shekhtman, Lev; Shevchenko, Vladimir; Shires, Alexander; Siddi, Benedetto Gianluca; Silva Coutinho, Rafael; Silva de Oliveira, Luiz Gustavo; Simi, Gabriele; Sirendi, Marek; Skidmore, Nicola; Skwarnicki, Tomasz; Smith, Edmund; Smith, Eluned; Smith, Iwan Thomas; Smith, Jackson; Smith, Mark; Snoek, Hella; Sokoloff, Michael; Soler, Paul; Soomro, Fatima; Souza, Daniel; Souza De Paula, Bruno; Spaan, Bernhard; Spradlin, Patrick; Sridharan, Srikanth; Stagni, Federico; Stahl, Marian; Stahl, Sascha; Stefkova, Slavorima; Steinkamp, Olaf; Stenyakin, Oleg; Stevenson, Scott; Stoica, Sabin; Stone, Sheldon; Storaci, Barbara; Stracka, Simone; Straticiuc, Mihai; Straumann, Ulrich; Sun, Liang; Sutcliffe, William; Swientek, Krzysztof; Swientek, Stefan; Syropoulos, Vasileios; Szczekowski, Marek; Szumlak, Tomasz; T'Jampens, Stephane; Tayduganov, Andrey; Tekampe, Tobias; Teklishyn, Maksym; Tellarini, Giulia; Teubert, Frederic; Thomas, Christopher; Thomas, Eric; van Tilburg, Jeroen; Tisserand, Vincent; Tobin, Mark; Todd, Jacob; Tolk, Siim; Tomassetti, Luca; Tonelli, Diego; Topp-Joergensen, Stig; Torr, Nicholas; Tournefier, Edwige; Tourneur, Stephane; Trabelsi, Karim; Tran, Minh Tâm; Tresch, Marco; Trisovic, Ana; Tsaregorodtsev, Andrei; Tsopelas, Panagiotis; Tuning, Niels; Ukleja, Artur; Ustyuzhanin, Andrey; Uwer, Ulrich; Vacca, Claudia; Vagnoni, Vincenzo; Valenti, Giovanni; Vallier, Alexis; Vazquez Gomez, Ricardo; Vazquez Regueiro, Pablo; Vázquez Sierra, Carlos; Vecchi, Stefania; Velthuis, Jaap; Veltri, Michele; Veneziano, Giovanni; Vesterinen, Mika; Viaud, Benoit; Vieira, Daniel; Vieites Diaz, Maria; Vilasis-Cardona, Xavier; Volkov, Vladimir; Vollhardt, Achim; Volyanskyy, Dmytro; Voong, David; Vorobyev, Alexey; Vorobyev, Vitaly; Voß, Christian; de Vries, Jacco; Waldi, Roland; Wallace, Charlotte; Wallace, Ronan; Walsh, John; Wandernoth, Sebastian; Wang, Jianchun; Ward, David; Watson, Nigel; Websdale, David; Weiden, Andreas; Whitehead, Mark; Wilkinson, Guy; Wilkinson, Michael; Williams, Mark Richard James; Williams, Matthew; Williams, Mike; Williams, Timothy; Wilson, Fergus; Wimberley, Jack; Wishahi, Julian; Wislicki, Wojciech; Witek, Mariusz; Wormser, Guy; Wotton, Stephen; Wyllie, Kenneth; Xie, Yuehong; Xu, Zhirui; Yang, Zhenwei; Yu, Jiesheng; Yuan, Xuhao; Yushchenko, Oleg; Zangoli, Maria; Zavertyaev, Mikhail; Zhang, Liming; Zhang, Yanxi; Zhelezov, Alexey; Zhokhov, Anatoly; Zhong, Liang; Zucchelli, Stefano

    2016-04-06

    The first model-independent measurement of the charm mixing parameters in the decay $D^0 \\to K_S \\pi^+ \\pi^-$ is reported, using a sample of $pp$ collision data recorded by the LHCb experiment, corresponding to an integrated luminosity of 1.0 fb$^{-1}$ at a centre-of-mass energy of 7 TeV. The measured values are \\begin{eqnarray*} x &=& ( -0.86 \\pm 0.53 \\pm 0.17 ) \\times 10^{-2}, \\\\ y &=& ( +0.03 \\pm 0.46 \\pm 0.13 ) \\times 10^{-2}, \\end{eqnarray*} where the first uncertainties are statistical and include small contributions due to the external input for the strong phase measured by the CLEO collaboration, and the second uncertainties are systematic.

  5. Circulating matrix gamma-carboxyglutamate protein (MGP) species are refractory to vitamin K treatment in a new case of Keutel syndrome

    NARCIS (Netherlands)

    Cranenburg, E. C. M.; van Spaendonck-Zwarts, K. Y.; Bonafe, L.; Crettol, L. Mittaz; Rodiger, L. A.; Dikkers, F. G.; van Essen, A. J.; Superti-Furga, A.; Alexandrakis, E.; Vermeer, C.; Schurgers, L. J.; Laverman, G. D.

    Background and objectives: Matrix gamma-carboxyglutamate protein (MGP), a vitamin K-dependent protein, is recognized as a potent local inhibitor of vascular calcification. Studying patients with Keutel syndrome (KS), a rare autosomal recessive disorder resulting from MGP mutations, provides an

  6. A case of exceptional reading accuracy in a child with Down syndrome – underlying skills and the relation to reading comprehension

    NARCIS (Netherlands)

    Groen, M.A.; Laws, G.; Nation, K.; Bishop, D.V.M.

    2006-01-01

    We report on a case of a girl with Down syndrome (DS), K.S., whose reading accuracy is exceptional. This ability is associated with robust phonological skills and relative strengths in visual and verbal short-term memory, articulation, and speech fluency. Although her reading comprehension is age

  7. Do Amnesic Patients with Korsakoff's Syndrome Use Feedback when Making Decisions under Risky Conditions? An Experimental Investigation with the Game of Dice Task with and without Feedback

    Science.gov (United States)

    Brand, Matthias; Pawlikowski, Mirko; Labudda, Kirsten; Laier, Christian; von Rothkirch, Nadine; Markowitsch, Hans J.

    2009-01-01

    We investigated the role of feedback processing in decision making under risk conditions in 50 patients with amnesia in the course of alcoholic Korsakoff's syndrome (KS). Half of the patients were administered the Game of Dice Task (GDT) and the remaining 25 patients were examined with a modified version of the GDT in which no feedback was…

  8. Circulating matrix gamma-carboxyglutamate protein (MGP) species are refractory to vitamin K treatment in a new case of Keutel syndrome

    NARCIS (Netherlands)

    Cranenburg, E. C. M.; van Spaendonck-Zwarts, K. Y.; Bonafe, L.; Mittaz Crettol, L.; Rödiger, L. A.; Dikkers, F. G.; van Essen, A. J.; Superti-Furga, A.; Alexandrakis, E.; Vermeer, C.; Schurgers, L. J.; Laverman, G. D.

    2011-01-01

    Background and objectives: Matrix gamma-carboxyglutamate protein (MGP), a vitamin K-dependent protein, is recognized as a potent local inhibitor of vascular calcification. Studying patients with Keutel syndrome (KS), a rare autosomal recessive disorder resulting from MGP mutations, provides an

  9. Rational Arithmetic Mathematica Functions to Evaluate the Two-Sided One Sample K-S Cumulative Sampling Distribution

    Directory of Open Access Journals (Sweden)

    J. Randall Brown

    2007-06-01

    Full Text Available One of the most widely used goodness-of-fit tests is the two-sided one sample Kolmogorov-Smirnov (K-S test which has been implemented by many computer statistical software packages. To calculate a two-sided p value (evaluate the cumulative sampling distribution, these packages use various methods including recursion formulae, limiting distributions, and approximations of unknown accuracy developed over thirty years ago. Based on an extensive literature search for the two-sided one sample K-S test, this paper identifies an exact formula for sample sizes up to 31, six recursion formulae, and one matrix formula that can be used to calculate a p value. To ensure accurate calculation by avoiding catastrophic cancelation and eliminating rounding error, each of these formulae is implemented in rational arithmetic. For the six recursion formulae and the matrix formula, computational experience for sample sizes up to 500 shows that computational times are increasing functions of both the sample size and the number of digits in the numerator and denominator integers of the rational number test statistic. The computational times of the seven formulae vary immensely but the Durbin recursion formula is almost always the fastest. Linear search is used to calculate the inverse of the cumulative sampling distribution (find the confidence interval half-width and tables of calculated half-widths are presented for sample sizes up to 500. Using calculated half-widths as input, computational times for the fastest formula, the Durbin recursion formula, are given for sample sizes up to two thousand.

  10. S6Ks isoforms contribute to viability, migration, docetaxel resistance and tumor formation of prostate cancer cells

    International Nuclear Information System (INIS)

    Amaral, Camila L.; Freitas, Lidia B.; Tamura, Rodrigo E.; Tavares, Mariana R.; Pavan, Isadora C. B.; Bajgelman, Marcio C.; Simabuco, Fernando M.

    2016-01-01

    The S6 Kinase (S6K) proteins are some of the main downstream effectors of the mammalian Target Of Rapamycin (mTOR) and act as key regulators of protein synthesis and cell growth. S6K is overexpressed in a variety of human tumors and is correlated to poor prognosis in prostate cancer. Due to the current urgency to identify factors involved in prostate cancer progression, we aimed to reveal the cellular functions of three S6K isoforms–p70-S6K1, p85-S6K1 and p54-S6K2–in prostate cancer, as well as their potential as therapeutic targets. In this study we performed S6K knockdown and overexpression and investigated its role in prostate cancer cell proliferation, colony formation, viability, migration and resistance to docetaxel treatment. In addition, we measured tumor growth in Nude mice injected with PC3 cells overexpressing S6K isoforms and tested the efficacy of a new available S6K1 inhibitor in vitro. S6Ks overexpression enhanced PC3-luc cell line viability, migration, resistance to docetaxel and tumor formation in Nude mice. Only S6K2 knockdown rendered prostate cancer cells more sensitive to docetaxel. S6K1 inhibitor PF-4708671 was particularly effective for reducing migration and proliferation of PC3 cell line. These findings demonstrate that S6Ks play an important role in prostate cancer progression, enhancing cell viability, migration and chemotherapy resistance, and place both S6K1 and S6K2 as a potential targets in advanced prostate cancer. We also provide evidence that S6K1 inhibitor PF-4708671 may be considered as a potential drug for prostate cancer treatment. The online version of this article (doi:10.1186/s12885-016-2629-y) contains supplementary material, which is available to authorized users

  11. Insights into pathogenic events of HIV-associated Kaposi sarcoma and immune reconstitution syndrome related Kaposi sarcoma

    Directory of Open Access Journals (Sweden)

    Lemmer Johan

    2008-01-01

    Full Text Available Abstract A decrease in the incidence of human immune deficiency virus-associated Kaposi sarcoma (HIV-KS and regression of some established HIV-KS lesions is evident after the introduction of highly active anti-retroviral treatment (HAART, and is attributed to generalized immune restoration, to the reconstitution of human herpesvirus (HHV-8 specific cellular immune responses, and to the decrease in HIV Tat protein and HHV-8 loads following HAART. However, a small subset of HIV-seropositive subjects with a low CD4+ T cell count at the time of introduction of HAART, may develop HIV-KS as immune reconstitution inflammatory syndrome (IRIS within 8 weeks thereafter.

  12. Marfan Syndrome

    Science.gov (United States)

    Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, blood vessels, ... A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, and ...

  13. Aarskog syndrome

    Science.gov (United States)

    Aarskog disease; Aarskog-Scott syndrome; AAS; Faciodigitogenital syndrome; Gaciogenital dysplasia ... Aarskog syndrome is a genetic disorder that is linked to the X chromosome. It affects mainly males, but females ...

  14. Williams syndrome

    Science.gov (United States)

    Williams-Beuren syndrome ... Williams syndrome is caused by not having a copy of several genes. It may be passed down in families. ... history of the condition. However, people with Williams syndrome have a 50% chance of passing the disorder ...

  15. Cushing's Syndrome

    OpenAIRE

    宗, 友厚; 伊藤, 勇; 諏訪, 哲也; 武田, 純; MUNE, Tomoatsu

    2003-01-01

    Sixteen cases of verified Cushing's syndrome, and twelve cases of probable Cushing's syndrome were reviewed and data on them were compared with various reports on Cushing's syndrome in the literature.

  16. Tourette syndrome

    Science.gov (United States)

    Gilles de la Tourette syndrome; Tic disorders - Tourette syndrome ... Tourette syndrome is named for Georges Gilles de la Tourette, who first described this disorder in 1885. The disorder is likely passed down through families. ...

  17. Long-term follow-up of a child with Klinefelter syndrome and achondroplasia from infancy to 16 years.

    Science.gov (United States)

    Arditi, Jessica D; Thomaidis, Loretta; Frysira, Helen; Doulgeraki, Artemis; Chrousos, George P; Kanaka-Gantenbein, Christina

    2017-07-26

    Achondroplasia (ACH), an autosomal dominant skeletal dysplasia, occurs in approximately 1:20,000 births. On the other hand, 47,XXY aneuploidy (Klinefelter syndrome [KS]) is the most common sex chromosome disorder, with a prevalence of approximately 1:600 males. To the best of our knowledge, only five cases of patients presenting both ACH and KS have been reported to date in the international literature. However, none of these cases has been longitudinally followed during the entire childhood. We report a male patient with ACH and KS, diagnosed in early infancy because of his typical phenotype of ACH. The diagnosis was confirmed by molecular analysis revealing a de novo heterozygous 1138 G-to-A mutation of the FGFR3 gene. During his first assessment, a karyotype was performed, which also revealed coexistence of KS. He was followed by our pediatric endocrinology team until the age of 16 years, then he was gradually transferred to adult endocrine care. This is the first reported case with both conditions that was diagnosed in infancy and was longitudinally followed by a pediatric endocrinology team regularly, from infancy to late adolescence. With a typical phenotype of ACH, it is striking and noteworthy that he did not develop the classical endocrine complications of a child with KS, neither did he necessitate testosterone supplementation during his pubertal development, due to his normal virilization and testosterone levels.

  18. Interaction Between Emotion and Memory: Importance of Mammillary Bodies Damage in a Mouse Model of the Alcoholic Korsakoff Syndrome

    Directory of Open Access Journals (Sweden)

    Daniel Béracochéa

    2005-01-01

    Full Text Available Chronic alcohol consumption (CAC can lead to the Korsakoff syndrome (KS, a memory deficiency attributed to diencephalie damage and/or to medial temporal or cortical related dysfunction. The etiology of KS remains unclear. Most animal models of KS involve thiaminedeficient diets associated with pyrithiamine treatment. Here we present a mouse model of CAC-induced KS. We demonstrate that CAC-generated retrieval memory deficits in working/ episodic memory tasks, together with a reduction of fear reactivity, result from damage to the mammillary bodies (MB. Experimental lesions of MB in non-alcoholic mice produced the same memory and emotional impairments. Drugs having anxiogenic-like properties counteract such impairments produced by CAC or by MB lesions. We suggest (a that MB are the essential components of a brain network underlying emotional processes, which would be critically important in the retrieval processes involved in working/ episodic memory tasks, and (b that failure to maintain emotional arousal due to MB damage can be a main factor of CAC-induced memory deficits. Overall, our animal model fits well with general neuropsychological and anatomic impairments observed in KS.

  19. Interaction between emotion and memory: importance of mammillary bodies damage in a mouse model of the alcoholic Korsakoff syndrome.

    Science.gov (United States)

    Béracochéa, Daniel

    2005-01-01

    Chronic alcohol consumption (CAC) can lead to the Korsakoff syndrome (KS), a memory deficiency attributed to diencephalic damage and/or to medial temporal or cortical related dysfunction. The etiology of KS remains unclear. Most animal models of KS involve thiamine-deficient diets associated with pyrithiamine treatment. Here we present a mouse model of CAC-induced KS. We demonstrate that CAC-generated retrieval memory deficits in working/ episodic memory tasks, together with a reduction of fear reactivity, result from damage to the mammillary bodies (MB). Experimental lesions of MB in non-alcoholic mice produced the same memory and emotional impairments. Drugs having anxiogenic-like properties counteract such impairments produced by CAC or by MB lesions. We suggest (a) that MB are the essential components of a brain network underlying emotional processes, which would be critically important in the retrieval processes involved in working/ episodic memory tasks, and (b) that failure to maintain emotional arousal due to MB damage can be a main factor of CAC-induced memory deficits. Overall, our animal model fits well with general neuropsychological and anatomic impairments observed in KS.

  20. 78 FR 100 - Guidance for Industry and Food and Drug Administration Staff; Refuse To Accept Policy for 510(k)s...

    Science.gov (United States)

    2013-01-02

    ... DEPARTMENT OF HEALTH AND HUMAN SERVICES Food and Drug Administration [Docket No. FDA-2012-D-0523] Guidance for Industry and Food and Drug Administration Staff; Refuse To Accept Policy for 510(k)s; Availability AGENCY: Food and Drug Administration, HHS. ACTION: Notice. SUMMARY: The Food and Drug...

  1. Draft Genome Sequence of the Hydrocarbon-Degrading Bacterium Alcanivorax dieselolei KS-293 Isolated from Surface Seawater in the Eastern Mediterranean Sea

    KAUST Repository

    Barbato, Marta; Mapelli, Francesca; Chouaia, Bessem; Crotti, Elena; Daffonchio, Daniele; Borin, Sara

    2015-01-01

    We report here the draft genome sequence of Alcanivorax dieselolei KS-293, a hydrocarbonoclastic bacterium isolated from the Mediterranean Sea, by supplying diesel oil as the sole carbon source. This strain contains multiple putative genes associated with hydrocarbon degradation pathways and that are highly similar to those described in A. dieselolei type strain B5.

  2. Benefits of Continuing Professional Development (CPD) Programmes in Music for KS2 (Primary) Teachers through the Example of the London Symphony Orchestra (LSO) on Track Programme

    Science.gov (United States)

    Varvarigou, Maria; Creech, Andrea; Hallam, Susan

    2012-01-01

    Between September 2008 and August 2010 24 KS2 classroom teachers were involved in a two-year programme of continuing professional development (CPD), delivered by the LSO in partnership with Local Authority Music Services. The teachers indicated that they embarked on the CPD programme looking forward to opportunities to share good practice, gain…

  3. Measurement of CP violation and constraints on the CKM angle γ in B±→DK± with D→KS0π+π− decays

    Directory of Open Access Journals (Sweden)

    R. Aaij

    2014-11-01

    where the first uncertainty is statistical, the second systematic and the third arises from the uncertainty of the D→KS0π+π− amplitude model. The value of γ is determined to be (84−42+49°, including all sources of uncertainty. Neutral D meson mixing is found to have negligible effect.

  4. Draft Genome Sequence of the Hydrocarbon-Degrading Bacterium Alcanivorax dieselolei KS-293 Isolated from Surface Seawater in the Eastern Mediterranean Sea

    KAUST Repository

    Barbato, Marta

    2015-12-10

    We report here the draft genome sequence of Alcanivorax dieselolei KS-293, a hydrocarbonoclastic bacterium isolated from the Mediterranean Sea, by supplying diesel oil as the sole carbon source. This strain contains multiple putative genes associated with hydrocarbon degradation pathways and that are highly similar to those described in A. dieselolei type strain B5.

  5. Effect of caffeine inhibitor in corrosion rate and microstructure of KS01 carbon steel and AISI 1045 at media sea water

    International Nuclear Information System (INIS)

    Sulistioso Giat S; Setyo Purwanto; Deswita; Ari Handayani; Berta Vidyananda

    2013-01-01

    Many synthetic materials are good inhibitors for the prevention of corrosion . Many inhibitors are toxic, because of the influence of these toxic properties, recently use organic materials as corrosion inhibitors, that are not hazardous and environmentally friendly. In this study caffeine compounds used as corrosion inhibitors. This compound could be used as corrosion inhibitor because of the existence of their chemical groups that containing free electron pair, that is nitrogen. Corrosion rate testing conducted in sea water medium taken from the Northern region of Indramayu with variations of the concentration of caffeine 0, 50, 100, 150, and 200 ppm to determine the optimum concentration of caffeine in corrosion rate of carbon steel AISI 1045 and KS01 that a widely used on the cooling system in the industry. Corrosion rate of KS-01 steel before used in inhibitor media is 25,07 mpy that less than corrosion rate of carbon steel AISI 1045, is 45,82 mpy . The results of this study indicate that caffeine is able to inhibit the corrosion rate of both of samples with optimum efficiency KS01 for 64.38%, and AISI 1045 of 66.63%. The optimum concentration of caffeine to inhibited AISI 1045 is 150 ppm and for KS01 is 100 ppm,. Beside that analysis of microstructure for both samples have done, for media before and after inhibitor addition. (author)

  6. Viis aastat Saviliiva kinnisvarakingitusest. Sundüürnikele läks vaid veidi enam kui veerand elamistest / Kristina Traks

    Index Scriptorium Estoniae

    Traks, Kristina, 1976-

    2011-01-01

    Viis aastat tagasi võitis 214 peret võimaluse osta eluase Tallinnas Kakumäel Saviliiva elamurajoonis. Toona läks 214 majast või korterist sundüürnikele vaid 64. Praeguseks pole enamik loosivõitjaid oma elukohta vahetanud

  7. Hypersexuality, Paraphilic Behaviors, and Gender Dysphoria in Individuals with Klinefelter's Syndrome.

    Science.gov (United States)

    Fisher, Alessandra D; Castellini, Giovanni; Casale, Helen; Fanni, Egidia; Bandini, Elisa; Campone, Beatrice; Ferruccio, Naika; Maseroli, Elisa; Boddi, Valentina; Dèttore, Davide; Pizzocaro, Alessandro; Balercia, Giancarlo; Oppo, Alessandro; Ricca, Valdo; Maggi, Mario

    2015-12-01

    An increased risk of autistic traits in Klinefelter syndrome (KS) has been reported. In addition, some studies have shown an increased incidence of gender dysphoria (GD) and paraphilia in autism spectrum disorder. The aim of this study was to evaluate the presence of (i) paraphilic fantasies and behaviors; and (ii) GD symptomatology in KS. A sample of 46 KS individuals and 43 healthy male controls (HC) were evaluated. Subjects were studied by means of several psychometric tests, such as Autism Spectrum Quotient (AQ) and Reading the Mind in the Eyes Revised (RME) to measure autistic traits, Gender Identity/GD questionnaire (GIDYQ-AA), and Sexual Addiction Screening Test (SAST). In addition, body uneasiness psychopathological symptoms were assessed using Symptom Checklist 90 Revised (SCL-90-R). The presence and frequency of any paraphilic fantasy and behavior was assessed by means of a clinical interview based on Diagnostic and Statistical Manual of Mental Disorders, 5th Edition criteria. Finally, all individuals included were assessed by Wechsler Adult Intelligence Scale-Revised to evaluate intelligence quotient (IQ). Data from a subsample of a previous published series of male to female GD individuals, with the battery of psychological measures useful to provide a psychopathological explanation of GD in KS population available, was also considered. When compared with HC, KS reported significantly lower total, verbal and performance IQ scores and higher SCL-90 obsession-compulsive symptoms (all P gender dysphoric symptoms than HC (P = 0.004), which were mediated by the presence of autistic traits (Sobel's test; P < 0.05). KS is associated with hypersexuality, paraphilic behaviors, and GD, which were mediated by obsessive-compulsive and autistic traits. © 2015 International Society for Sexual Medicine.

  8. Hepatorenal syndrome

    Science.gov (United States)

    ... 2016:chap 153. Nevah MI, Fallon MB. Hepatic encephalopathy, hepatorenal syndrome, hepatopulmonary syndrome, and other systemic complications of liver disease. In: Feldman M, Friedman LS, Brandt LJ, ...

  9. Successful Multi-Leg Completion of KS-13 ML-1 & Increased Power Generation of Puna Geothermal Venture (PGV), Hawai'i

    Science.gov (United States)

    Drakos, P. S.; Spielman, P.; Peters, B.

    2017-12-01

    Located in the Puna district on the Big Island in Hawaii, Puna Geothermal Venture (PGV) is the only geothermal power plant in the state. PGV is comprised of two air-cooled power plants with a total generating capacity of 38 MW. Commercial operation commenced in 1993 and the project was acquired by Ormat in June 2004. Over the years, generation has increased by upgrading the plant through resource development and with the addition of a bottoming OEC (Ormat Energy Converter) in 2011. The geothermal reservoir at PGV is hosted within a step-over along the axis of the Kilauea Lower East Rift Zone (LERZ). Subsurface permeability at PGV is controlled by sub-vertical and rift-parallel fractures/faults and dike swarms which are the result of active tectonic dilation across the rift and shallow volcanic activity related to Kilauea. At PGV, the location and attitude of these fractures are well constrained at depth by drilling to be orientated at N63°E and dipping at 5° NW. These fractures are aligned en-echelon and form a major left-step along the rift axis which results in a localized zone of enhanced dilation. In 2016, a program was initiated to increase injection capacity and enthalpy in the PGV wellfield. Existing injection well KS-13 was selected as a candidate for re-drill based on a comprehensive resource model and reservoir modeling predictions. KS-13 ML1 was designed as a multi-leg completion from the existing KS-13 well, whereby the final completion is a forked well composed of the original wellbore and the newly completed second wellbore. The target area for the new multi-leg (ML) were large aperture, steeply dipping fractures associated with the 1955 eruptive fissure. Well KS-13 ML1 was drilled using PGV's Rig and a retrievable whipstock to mill a casing exit window. With the original wellbore temporarily plugged, a multi-rate water loss test was performed and an injectivity of 6 gpm/psi was measured. Following the removal of the whipstock ramp and packer from

  10. Low fertility awareness in United States reproductive-aged women and medical trainees: creation and validation of the Fertility & Infertility Treatment Knowledge Score (FIT-KS).

    Science.gov (United States)

    Kudesia, Rashmi; Chernyak, Elizabeth; McAvey, Beth

    2017-10-01

    To create, validate, and use a fertility awareness survey based on current U.S. Cross-sectional study. Not applicable. Phase 1 included U.S. women ages 18-45; phase 2 included female medical students and obstetrics and gynecology trainees at two urban academic programs. Survey including demographics, the Fertility & Infertility Treatment Knowledge Score (FIT-KS) instrument, and General Nutrition Knowledge Questionnaire. Knowledge of natural fertility and infertility treatments. The FIT-KS was validated through detailed item and validity analyses. In phase 1, 127 women participated; their median age was 31 years, and 43.7% had children. Their mean FIT-KS score was 16.2 ± 3.5 (55.9% correct). In phase 2, 118 medical trainees participated; their median age was 25 years, and 12.4% had children. Their mean FIT-KS score was 18.8 ± 2.1 (64.9% correct), with year of training correlating to a higher score (r=0.40). Participant awareness regarding lifestyle factors varied, but it was particularly low regarding the effects of lubricants. The majority underestimated the spontaneous miscarriage rate and overestimated the fecundability of 40-year-old women. There was general overestimation of success rates for assisted reproductive technologies, particularly among medical trainees. The FIT-KS is validated to current U.S. data for use in both general and medical populations as a quick assessment of fertility knowledge. The knowledge gaps demonstrated in this study correlate with national trends in delayed childbearing and time to initiate treatment. For medical trainees, these results raise concerns about the quality of fertility counseling they may be able to offer patients. Greater educational outreach must be undertaken to enhance fertility awareness. Copyright © 2017 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.

  11. Hypogonadotropic Hypogonadism Revisited

    Directory of Open Access Journals (Sweden)

    Renato Fraietta

    2013-01-01

    Full Text Available Impaired testicular function, i.e., hypogonadism, can result from a primary testicular disorder (hypergonadotropic or occur secondary to hypothalamic-pituitary dysfunction (hypogonadotropic. Hypogonadotropic hypogonadism can be congenital or acquired. Congenital hypogonadotropic hypogonadism is divided into anosmic hypogonadotropic hypogonadism (Kallmann syndrome and congenital normosmic isolated hypogonadotropic hypogonadism (idiopathic hypogonadotropic hypogonadism. The incidence of congenital hypogonadotropic hypogonadism is approximately 1-10:100,000 live births, and approximately 2/3 and 1/3 of cases are caused by Kallmann syndrome (KS and idiopathic hypogonadotropic hypogonadism, respectively. Acquired hypogonadotropic hypogonadism can be caused by drugs, infiltrative or infectious pituitary lesions, hyperprolactinemia, encephalic trauma, pituitary/brain radiation, exhausting exercise, abusive alcohol or illicit drug intake, and systemic diseases such as hemochromatosis, sarcoidosis and histiocytosis X. The clinical characteristics of hypogonadotropic hypogonadism are androgen deficiency and a lack/delay/stop of pubertal sexual maturation. Low blood testosterone levels and low pituitary hormone levels confirm the hypogonadotropic hypogonadism diagnosis. A prolonged stimulated intravenous GnRH test can be useful. In Kallmann syndrome, cerebral MRI can show an anomalous morphology or even absence of the olfactory bulb. Therapy for hypogonadotropic hypogonadism depends on the patient's desire for future fertility. Hormone replacement with testosterone is the classic treatment for hypogonadism. Androgen replacement is indicated for men who already have children or have no desire to induce pregnancy, and testosterone therapy is used to reverse the symptoms and signs of hypogonadism. Conversely, GnRH or gonadotropin therapies are the best options for men wishing to have children. Hypogonadotropic hypogonadism is one of the rare conditions in

  12. Hypogonadotropic Hypogonadism Revisited

    Science.gov (United States)

    Fraietta, Renato; Zylberstejn, Daniel Suslik; Esteves, Sandro C

    2013-01-01

    Impaired testicular function, i.e., hypogonadism, can result from a primary testicular disorder (hypergonadotropic) or occur secondary to hypothalamic-pituitary dysfunction (hypogonadotropic). Hypogonadotropic hypogonadism can be congenital or acquired. Congenital hypogonadotropic hypogonadism is divided into anosmic hypogonadotropic hypogonadism (Kallmann syndrome) and congenital normosmic isolated hypogonadotropic hypogonadism (idiopathic hypogonadotropic hypogonadism). The incidence of congenital hypogonadotropic hypogonadism is approximately 1-10:100,000 live births, and approximately 2/3 and 1/3 of cases are caused by Kallmann syndrome (KS) and idiopathic hypogonadotropic hypogonadism, respectively. Acquired hypogonadotropic hypogonadism can be caused by drugs, infiltrative or infectious pituitary lesions, hyperprolactinemia, encephalic trauma, pituitary/brain radiation, exhausting exercise, abusive alcohol or illicit drug intake, and systemic diseases such as hemochromatosis, sarcoidosis and histiocytosis X. The clinical characteristics of hypogonadotropic hypogonadism are androgen deficiency and a lack/delay/stop of pubertal sexual maturation. Low blood testosterone levels and low pituitary hormone levels confirm the hypogonadotropic hypogonadism diagnosis. A prolonged stimulated intravenous GnRH test can be useful. In Kallmann syndrome, cerebral MRI can show an anomalous morphology or even absence of the olfactory bulb. Therapy for hypogonadotropic hypogonadism depends on the patient's desire for future fertility. Hormone replacement with testosterone is the classic treatment for hypogonadism. Androgen replacement is indicated for men who already have children or have no desire to induce pregnancy, and testosterone therapy is used to reverse the symptoms and signs of hypogonadism. Conversely, GnRH or gonadotropin therapies are the best options for men wishing to have children. Hypogonadotropic hypogonadism is one of the rare conditions in which specific

  13. The relationship between fat depot-specific preadipocyte differentiation and metabolic syndrome in obese women

    Directory of Open Access Journals (Sweden)

    N V Mazurina

    2013-03-01

    Full Text Available Реферат по материалам статьи The relationship between fat depot-specific preadipocyte differentiation and metabolic syndrome in obese women. Park HТ, Lee ES, Cheon EP, Lee DR, Yang K-S, Kim YT, Hur JY, Kim SH, Lee KW, Kim T. Clinical Endocrinology 2012; 76, 59-66.

  14. Efficacy of imidacloprid + moxidectin and selamectin topical solutions against the KS1 Ctenocephalides felis flea strain infesting cats

    Directory of Open Access Journals (Sweden)

    Dryden Michael W

    2011-09-01

    Full Text Available Abstract Background Two studies were conducted to evaluate and compare the efficacy of imidacloprid + moxidectin and selamectin topical solutions against the KS1 flea strain infesting cats. In both studies the treatment groups were comprised of non-treated controls, 6% w/v selamectin (Revolution®; Pfizer Animal Health topical solution and 10% w/v imidacloprid + 1% w/v moxidectin (Advantage Multi® for Cats, Bayer Animal Health topical solution. All cats were infested with 100 fleas on Days -2, 7, 14, 21, and 28. The difference in the studies was that in study #1 efficacy evaluations were conducted at 24 and 48 hours post-treatment or post-infestation, and in study #2 evaluations were conducted at 12 and 24 hours. Results In study #1 imidacloprid + moxidectin and the selamectin formulation provided 99.8% and 99.0% efficacy at 24 hours post-treatment. On day 28, the 24 hour efficacy of the selamectin formulation dropped to 87.1%, whereas the imidacloprid + moxidectin formulation provided 98.9% efficacy. At the 48 hour assessments following the 28 day infestations, efficacy of the imidacloprid + moxidectin and selamectin formulations was 96.8% and 98.3% respectively. In study # 2 the efficacy of the imidacloprid + moxidectin and selamectin formulations 12 hours after treatment was 100% and 69.4%, respectively. On day 28, efficacy of the imidacloprid + moxidectin and selamectin formulations 12 hours after infestation was 90.2% and 57.3%, respectively. In study #2 both formulations provided high levels of efficacy at the 24 hour post-infestation assessments, with selamectin and imidacloprid + moxidectin providing 95.3% and 97.5% efficacy, following infestations on day 28. Conclusions At the 24 and 48 hour residual efficacy assessments, the imidacloprid + moxidectin and selamectin formulations were similarly highly efficacious. However, the imidacloprid + moxidectin formulation provided a significantly higher rate of flea kill against the KS1 flea

  15. Folk food and medicinal botanical knowledge among the last remaining Yörüks of the Balkans

    Directory of Open Access Journals (Sweden)

    Anely Nedelcheva

    2017-03-01

    Full Text Available The present study examines the current lifestyle of the last remaining Balkan Yörüks, a small and isolated group found within the Republic of Macedonia, and the modern representatives of an important portion of the Balkan nomads. The aim of this study was to document knowledge concerning local wild food plants and wild and cultivated medicinal plants, and to compare the Yörük ethnobotany with that of similar, more or less isolated ethnic groups occurring in the Balkan region (Macedonia, Bulgaria, Albania, and Turkey in order to assess how cultural adaptation processes may have affected Yörük plant folklore. We conducted this study by means of detailed, semi-structured interviews with 48 key informants. Sixty-seven plant taxa were recorded from 55 genera, based on the compilation of more than 150 reports relating to medicinal, food, forage, ornamental, and dye plants, as well as some elements relating to animals and minerals. Our field study data show several major ethnic boundary markers that contribute to the homogeneity of the community and also distinguish it sufficiently from the surrounding society: (i well-isolated locality; (ii local dialect and endogamy; (iii casual clothing worn by women; (iv ceremonial jewelry: a necklace of cloves (Syzygium aromaticum; (v Sempervivum marmoreum as an only ornamental plant which also has a medicinal use; and (vi Mentha spicata as the dominant culinary herb, which has a medicinal use too. Comparison of the collected ethnobotanical data with that of similar, more or less isolated ethnic groups in the Balkan region shows that overlapping taxa include mainly plants whose fresh fruit are used; both nuts as well as edible greens. These plants are simultaneously used for medicinal purposes too, as home remedies, but in very different ways to other ethnic groups. Yörüks represent a remarkable cultural group in the Balkans. This community has nomadic traditions, but nowadays the people have a settled

  16. Pitavastatin suppresses diethylnitrosamine-induced liver preneoplasms in male C57BL/KsJ-db/db obese mice

    International Nuclear Information System (INIS)

    Shimizu, Masahito; Tanaka, Takuji; Moriwaki, Hisataka; Yasuda, Yoichi; Sakai, Hiroyasu; Kubota, Masaya; Terakura, Daishi; Baba, Atsushi; Ohno, Tomohiko; Kochi, Takahiro; Tsurumi, Hisashi

    2011-01-01

    Obesity and related metabolic abnormalities, including inflammation and lipid accumulation in the liver, play a role in liver carcinogenesis. Adipocytokine imbalances, such as decreased serum adiponectin levels, are also involved in obesity-related liver tumorigenesis. In the present study, we examined the effects of pitavastatin - a drug used for the treatment of hyperlipidemia - on the development of diethylnitrosamine (DEN)-induced liver preneoplastic lesions in C57BL/KsJ-db/db (db/db) obese mice. Male db/db mice were administered tap water containing 40 ppm DEN for 2 weeks and were subsequently fed a diet containing 1 ppm or 10 ppm pitavastatin for 14 weeks. At sacrifice, feeding with 10 ppm pitavastatin significantly inhibited the development of hepatic premalignant lesions, foci of cellular alteration, as compared to that in the untreated group by inducing apoptosis, but inhibiting cell proliferation. Pitavastatin improved liver steatosis and activated the AMPK-α protein in the liver. It also decreased free fatty acid and aminotransferases levels, while increasing adiponectin levels in the serum. The serum levels of tumor necrosis factor (TNF)-α and the expression of TNF-α and interleukin-6 mRNAs in the liver were decreased by pitavastatin treatment, suggesting attenuation of the chronic inflammation induced by excess fat deposition. Pitavastatin is effective in inhibiting the early phase of obesity-related liver tumorigenesis and, therefore, may be useful in the chemoprevention of liver cancer in obese individuals

  17. KaKs_Calculator 2.0: A Toolkit Incorporating Gamma-Series Methods and Sliding Window Strategies

    KAUST Repository

    Wang, Dapeng

    2010-05-05

    We present an integrated stand-alone software package named KaKs_Calculator 2.0 as an updated version. It incorporates 17 methods for the calculation of nonsynonymous and synonymous substitution rates; among them, we added our modified versions of several widely used methods as the gamma series including γ-NG, γ-LWL, γ-MLWL, γ-LPB, γ-MLPB, γ-YN and γ-MYN, which have been demonstrated to perform better under certain conditions than their original forms and are not implemented in the previous version. The package is readily used for the identification of positively selected sites based on a sliding window across the sequences of interests in 5\\' to 3\\' direction of protein-coding sequences, and have improved the overall performance on sequence analysis for evolution studies. A toolbox, including C++ and Java source code and executable files on both Windows and Linux platforms together with a user instruction, is downloadable from the website for academic purpose at https://sourceforge.net/projects/kakscalculator2/.

  18. Spanish version of the Kidney Disease Knowledge Survey (KiKS) in Peru: cross-cultural adaptation and validation.

    Science.gov (United States)

    Mota-Anaya, Evelin; Yumpo-Cárdenas, Daniel; Alva-Bravo, Edmundo; Wright-Nunes, Julie; Mayta-Tristán, Percy

    2016-08-08

    Chronic kidney disease (CKD) affects 50 million people globally. Several studies show the importance of implementing interventions that enhance patients’ knowledge about their disease. In 2011 the Kidney Disease Knowledge Survey (KiKS) was developed: a questionnaire that assesses the specific knowledge about chronic kidney disease in pre-dialysis patients. To translate to Spanish, culturally adapt and validate the Kidney Disease Knowledge Survey questionnaire in a population of patients with pre-dialysis chronic kidney disease. We carried out a Spanish translation and cross-cultural adaptation of the Kidney Disease Knowledge Survey questionnaire. Subsequently, we determined its validity and reliability. We determined the validity through construct validity; and reliability by evaluating its internal consistency and its intra-observer reliability (test-retest). We found a good internal consistency (Kuder-Richardson = 0.85). The intra-observer reliability was measured by the intra-class correlation coefficient that yielded a value of 0.78 (95% CI: 0.5-1.0). This value indicated a good reproducibility; also, the mean difference of -1.1 test-retest SD 6.0 (p = 0.369) confirms this finding. The translated Spanish version of the Kidney Disease Knowledge Survey is acceptable and equivalent to the original version; it also has a good reliability, validity and reproducibility. Therefore, it can be used in a population of patients with pre-dialysis chronic kidney disease.

  19. KaKs_Calculator 2.0: A Toolkit Incorporating Gamma-Series Methods and Sliding Window Strategies

    KAUST Repository

    Wang, Dapeng; Zhang, Yubin; Zhang, Zhang; Zhu, Jiang; Yu, Jun

    2010-01-01

    We present an integrated stand-alone software package named KaKs_Calculator 2.0 as an updated version. It incorporates 17 methods for the calculation of nonsynonymous and synonymous substitution rates; among them, we added our modified versions of several widely used methods as the gamma series including γ-NG, γ-LWL, γ-MLWL, γ-LPB, γ-MLPB, γ-YN and γ-MYN, which have been demonstrated to perform better under certain conditions than their original forms and are not implemented in the previous version. The package is readily used for the identification of positively selected sites based on a sliding window across the sequences of interests in 5' to 3' direction of protein-coding sequences, and have improved the overall performance on sequence analysis for evolution studies. A toolbox, including C++ and Java source code and executable files on both Windows and Linux platforms together with a user instruction, is downloadable from the website for academic purpose at https://sourceforge.net/projects/kakscalculator2/.

  20. Patients with Korsakoff syndrome in nursing homes: characteristics, comorbidity, and use of psychotropic drugs.

    Science.gov (United States)

    Gerridzen, Ineke J; Goossensen, M Anne

    2014-01-01

    Very limited literature exists on the care and course of patients with Korsakoff syndrome (KS) living in long-term care facilities (LTCFs). Even less literature can be found on the pharmacological treatment of behavioral symptoms of KS. The purpose of the present study was to describe baseline characteristics, comorbidity, and the use of psychotropic drugs in institutionalized patients with KS. In this cross-sectional descriptive study, 556 patients were included living in ten specialized care units in Dutch nursing homes. Data were collected by means of a retrospective chart review. The majority of patients were men (75%) and single (78%) with a mean age on admission of 56.7 years (SD 8.9, range 29.8-85.3). Mean length of stay was 6.0 years (SD 5.4, range 0.2-33.3). Sixty-eight percent of patients suffered from at least one somatic disease and 66% from at least one extra psychiatric disorder. One or more psychotropic drugs were prescribed to 71% of patients with a great variation in prescription patterns between the different nursing homes. Patients with KS depending on long-term care usually have comorbidity in more than one domain (somatic and psychiatric). The indications for prescribing psychotropic drugs are in many cases unclear and it seems probable that they are often given to manage challenging behavior. Longitudinal studies on the evidence for this prescription behavior and possible alternatives are recommended.

  1. The effect of errorless learning on quality of life in patients with Korsakoff’s syndrome

    Science.gov (United States)

    Rensen, Yvonne CM; Egger, Jos IM; Westhoff, Josette; Walvoort, Serge JW; Kessels, Roy PC

    2017-01-01

    Background Errorless learning (EL) is a promising rehabilitation principle for (re)learning instrumental activities in patients with amnesia, including patients with Korsakoff’s syndrome (KS). Successfully (re)learning tasks might improve the sense of competence and independence, and subsequently the quality of life. Quality of life in patients with KS has received limited attention, and no studies have been conducted to experimentally examine the effect of EL on quality of life in patients in this patient group. Materials and methods The QUALIDEM, an observation scale for quality of life, was completed by professional nurses before and after EL training in a group of fifty-one patients with KS. This scale was also completed for a group of thirty-one control patients receiving care as usual but no EL training. Results Quality of life was significantly increased on eight of the nine subscales in the Korsakoff group who participated in an EL training. There was a trend toward a significant increase in “positive affect” (ie, the ninth subscale). In contrast, no changes over time were found on any of the subscales in the control group that did not participate in any EL training. Conclusion Despite severe memory impairments, patients with KS still have the potential to (partially) (re)learn tasks using EL. This potential should be exploited, as the successes of (re)-learning might improve the quality of life of Korsakoff patients in nursing homes. PMID:29225465

  2. Cognitive performance of detoxified alcoholic Korsakoff syndrome patients remains stable over two years.

    Science.gov (United States)

    Fujiwara, Esther; Brand, Matthias; Borsutzky, Sabine; Steingass, Hans-P; Markowitsch, Hans J

    2008-07-01

    Excessive alcohol consumption is assumed to promote cognitive decline, eventually increasing the risk of dementia. However, little is known about the time course of cognitive functions in patients with chronic alcoholic Korsakoff syndrome (KS). Therefore, we assessed neuropsychological performance in 20 detoxified chronic KS inpatients at time 1 (T1) with a follow-up after two years (T2). The neuropsychological tests assessed verbal and visual short- and long-term memory, working memory, basic executive functions, language, general knowledge, and visual-spatial abilities. Surveys with caregivers and medical records provided information about current and previous disease-related parameters, drinking history, additional pathologies, as well as psychosocial and cognitive therapy within the two-year period. At both sessions, the majority of the KS patients' results were inferior to those of normal subjects. Comparing T1 and T2 revealed no significant decline in any of the investigated functions. Instead, general knowledge, visual long-term memory, and verbal fluency improved slightly after two years, though they still remained within pathological range. Comparing most improved and most deteriorated patients, better outcome occurred more frequently in men than women and was associated with higher premorbid education and fewer detoxifications in the past. In this sample of detoxified KS patients there was no indication of accelerated cognitive decline or onset of dementia-like symptoms over two years.

  3. Comparative studies on the interactions of the natural isolate KS5 and one reference strain (DSM 10134) with uranium(VI)

    Energy Technology Data Exchange (ETDEWEB)

    Gerber, Ulrike; Krawczyk-Baersch, Evelyn [Helmholtz-Zentrum Dresden-Rossendorf e.V., Dresden (Germany). Div. Biogeochemistry; Arnold, Thuro [Helmholtz-Zentrum Dresden-Rossendorf e.V., Dresden (Germany). Inst. of Resource Ecology

    2016-07-01

    The yeast KS5 (Rhodosporidium toruloides) was isolated by culture dependent method directly from the flooding water in Koenigstein (Germany). To compare the U tolerance and immobilization ability of the isolate a reference culture DSM 10134 (R. toruloides) was applied. Both cultures displayed the ability to tolerate high amounts of U, in contrast the reference KS5 showed a six-fold higher U tolerance in comparison to the reference strain. U immobilization studies displayed that both organisms are able to remove high amounts. The flooding water in Koenigstein has to be cleaned up for many years by an intensive waste water treatment plant. Possibly, with the help of natural occuring microorganisms the flooding water could be cleaned up using in situ bioremediation.

  4. Deep Sequencing-Identified Kanamycin-Resistant Paenibacillus sp. Strain KS1 Isolated from Epiphyte Tillandsia usneoides (Spanish Moss) in Central Florida, USA.

    Science.gov (United States)

    Lata, Pushpa; Govindarajan, Subramaniam S; Qi, Feng; Li, Jian-Liang; Sahoo, Malaya K

    2017-02-02

    Paenibacillus sp. strain KS1 was isolated from an epiphyte, Tillandsia usneoides (Spanish moss), in central Florida, USA. Here, we report a draft genome sequence of this strain, which consists of a total of 398 contigs spanning 6,508,195 bp, with a G+C content of 46.5% and comprising 5,401 predicted coding sequences. Copyright © 2017 Lata et al.

  5. The KS-KT-100 plant for two-stage vitrification of radioactive waste: results of tests with simulators

    International Nuclear Information System (INIS)

    Davydov, V.I.; Dobrygin, P.G.; Dolgov, V.V.; Sergeev, G.A.

    1976-01-01

    The Soviet Union has developed a two-stage process for phosphate vitrification of liquid radioactive waste involving the use, at the initial stage, of calcination in the pseudo-liquefied layer, followed by melting of the calcinate in a ceramic crucible (second stage). On the basis of the laboratory studies and bench tests using experimental equipment, the authors have developed and tried out an enlarged plant - the KS-KT-100. The plant includes units for preparing the solution, evaporation, calcination, melting and gas purification. The initial solution containing 240 g/litre of aluminium nitrate, 125 g/litre of sodium nitrate, 120 to 130 g/litre of orthophosphoric acid, and 90 to 150 g/litre of industrial molasses simulated fluxed nitrate waste. The tests have shown that the various units operate satisfactorily. The authors have determined the technological parameters for evaporation, calcination of the solution and melting of the calcinate. The presence of molasses in the solution (150 g/litre) makes it possible to decompose and distil 40% of the nitrate ion during evaporation. The calcination temperature is 350 to 400 0 C, and the fluidization rate 1.5 m/s. The capacity of the plant for the initial solution is 100 litres/h, for the evaporated solution 65 litres/h, and for the glass 20 kg/h. The efficiency of the gas purification system ranges between 10 7 and 10 9 . The test results show the feasibility of the two-stage method of vitrification in actual practice. (author)

  6. Nucleation behavior of melted Bi films at cooling rates from 101 to 104 K/s studied by combining scanning AC and DC nano-calorimetry techniques

    International Nuclear Information System (INIS)

    Xiao, Kechao; Vlassak, Joost J.

    2015-01-01

    Highlights: • We proposed a general data reduction scheme that combines scanning AC and DC calorimetry results for the study of reaction kinetics. • Calorimetry measurements at cooling rates ranging from 30 K/s to 20,000 K/s were achieved. • Upon initial melting, the Bi thin-film sample breaks up into thousands of isolated islands, and highly repeatable nucleation behavior is observed. • The nucleation rate of melted Bi is calculated, which can be well described by classical nucleation theory over a wide range of cooling rates. - Abstract: We study the nucleation behavior of undercooled liquid Bi at cooling rates ranging from 10 1 to 10 4 K/s using a combination of scanning DC and AC nano-calorimetry techniques. Upon initial melting, the Bi thin-film sample breaks up into silicon nitride-coated isolated islands. The number of islands in a typical sample is sufficiently large that highly repeatable nucleation behavior is observed, despite the stochastic nature of the nucleation process. We establish a data reduction technique to evaluate the nucleation rate from DC and AC calorimetry results. The results show that the driving force for the nucleation of melted Bi is well described by classical nucleation theory over a wide range of cooling rates. The proposed technique provides a unique and efficient way to examine nucleation kinetics with cooling rates over several orders of magnitude. The technique is quite general and can be used to evaluate reaction kinetics in other materials

  7. Imbalance between cognitive systems in alcohol-dependence and Korsakoff syndrome: An exploration using the Alcohol Flanker Task.

    Science.gov (United States)

    Brion, Mélanie; Dormal, Valérie; Lannoy, Séverine; Mertens, Serge; de Timary, Philippe; Maurage, Pierre

    2018-03-06

    Alcohol-dependent individuals (ALC) simultaneously present decreased inhibitory control and increased attention towards alcohol-related cues. The dual-process models have proposed that these symptoms reflect an imbalance between prefrontal/reflective and limbic/automatic systems, respectively leading to cognitive dysfunctions in executive processes and to alcohol-related bias. However, most previous research has focused on a separate exploration of these systems among ALC, and the direct measure of their interactions remains to be conducted. Moreover, no study has explored the evolution of this imbalance across the successive stages of alcohol-related disorders, and particularly in Korsakoff syndrome (KS), the most frequent neurological complication of alcohol-dependence. Ten KS, 14 ALC, and 14 matched control participants performed a modified Flanker task, the "Alcohol Flanker Task," based on congruent, incongruent, and neutral conditions with alcohol-related stimuli. This task required inhibitory processing on alcohol-related stimuli and evaluated, through a behavioral approach, the interaction between reflective and automatic systems, as well as its evolution between ALC and KS. ALC and KS both presented high reactivity towards alcohol-related stimuli, confirming the presence of alcohol-related bias. KS showed increased omission rates (related to distractor interference) while ALC showed higher false-alarm rates (related to prepotent response inhibition). These results suggest that different inhibitory subcomponents might be altered at the successive stages of the pathology, and experimentally confirms the crucial role of the interaction between reflective and automatic processes in alcohol-use disorders. The present results reinforce the proposal that alcohol-related cues significantly impact inhibitory control in alcohol-related disorders. However, ALC and KS present different patterns of deficits depending on task complexity (i.e., executive load), thus

  8. FLOODPLAIN, DONIPHAN COUNTY, KS

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Floodplain Mapping/Redelineation study deliverables depict and quantify the flood risks for the study area. The primary risk classifications used are the...

  9. KS-150 reactor control

    International Nuclear Information System (INIS)

    Wagner, K.

    1974-01-01

    A thorough description is presented of the control and protection system of the Bohunice A-1 reactor. The system including auxiliary facilities was developed, manufactured and installed at the reactor by the SKODA Works, Plzen. The system parameters are listed and a brief account is also given of the development efforts and of the physical and power start-up of the A-1 nuclear power plant. (L.O.)

  10. Cushing's Syndrome

    Science.gov (United States)

    Cushing's syndrome is a hormonal disorder. The cause is long-term exposure to too much cortisol, a hormone that ... your body to make too much cortisol. Cushing's syndrome is rare. Some symptoms are Upper body obesity ...

  11. Usher Syndrome

    Science.gov (United States)

    Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder that causes ... and vision. There are three types of Usher syndrome: People with type I are deaf from birth ...

  12. Metabolic Syndrome

    Science.gov (United States)

    Metabolic syndrome is a group of conditions that put you at risk for heart disease and diabetes. These conditions ... agree on the definition or cause of metabolic syndrome. The cause might be insulin resistance. Insulin is ...

  13. Reye Syndrome

    Science.gov (United States)

    Reye syndrome is a rare illness that can affect the blood, liver, and brain of someone who has recently ... a viral illness, seek medical attention immediately. Reye syndrome can lead to a coma and brain death, ...

  14. Rett Syndrome

    Science.gov (United States)

    Rett syndrome is a rare genetic disease that causes developmental and nervous system problems, mostly in girls. It's related to autism spectrum disorder. Babies with Rett syndrome seem to grow and develop normally at first. ...

  15. Caplan syndrome

    Science.gov (United States)

    ... enable JavaScript. Rheumatoid pneumoconiosis (RP; also known as Caplan syndrome) is swelling (inflammation) and scarring of the ... avoid exposure to inorganic dust. Alternative Names RP; Caplan syndrome; Pneumoconiosis - rheumatoid; Silicosis - rheumatoid pneumoconiosis; Coal worker's ...

  16. Turner Syndrome

    Science.gov (United States)

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

  17. Gardner's syndrome

    International Nuclear Information System (INIS)

    Sobrado Junior, C.W.; Bresser, A.; Cerri, G.G.; Habr-Gama, A.; Pinotti, H.W.; Magalhaes, A.

    1988-01-01

    A case of familiar poliposis of colon related to a right mandibular osteoma is reported (this association is usually called Gardner's syndrome). Radiologic pictures ae shown and some commentaries about this syndrome concerning the treatment are made. (author) [pt

  18. Sotos Syndrome

    Science.gov (United States)

    ... Clinical Trials Organizations Publications Definition Sotos syndrome (cerebral gigantism) is a rare genetic disorder caused by mutation ... have also been reported. × Definition Sotos syndrome (cerebral gigantism) is a rare genetic disorder caused by mutation ...

  19. Felty syndrome

    Science.gov (United States)

    Seropositive rheumatoid arthritis (RA); Felty's syndrome ... The cause of Felty syndrome is unknown. It is more common in people who have had rheumatoid arthritis (RA) for a long time. People with ...

  20. Bartter syndrome

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000308.htm Bartter syndrome To use the sharing features on this page, please enable JavaScript. Bartter syndrome is a group of rare conditions that affect ...

  1. Pendred Syndrome

    Science.gov (United States)

    ... other possible long-term consequences of the syndrome. Children with Pendred syndrome should start early treatment to gain communication skills, such as learning sign language or cued speech or learning to ...

  2. Dravet Syndrome

    Science.gov (United States)

    ... and supports a broad program of basic and clinical research on all types of epilepsy, including Dravet syndrome. Study of the genetic defects responsible for Dravet syndrome and related ... Publications Definition Dravet ...

  3. Study of counter current flow limitation model of MARS-KS and SPACE codes under Dukler's air/water flooding test conditions

    International Nuclear Information System (INIS)

    Lee, Won Woong; Kim, Min Gil; Lee, Jeong Ik; Bang, Young Seok

    2015-01-01

    In particular, CCFL(the counter current flow limitation) occurs in components such as hot leg, downcomer annulus and steam generator inlet plenum during LOCA which is possible to have flows in two opposite directions. Therefore, CCFL is one of the thermal-hydraulic models which has significant effect on the reactor safety analysis code performance. In this study, the CCFL model will be evaluated with MARS-KS based on two-phase two-field governing equations and SPACE code based on two-phase three-field governing equations. This study will be conducted by comparing MARS-KS code which is being used for evaluating the safety of a Korean Nuclear Power Plant and SPACE code which is currently under assessment for evaluating the safety of the designed nuclear power plant. In this study, comparison of the results of liquid upflow and liquid downflow rate for different gas flow rate from two code to the famous Dukler's CCFL experimental data are presented. This study will be helpful to understand the difference between system analysis codes with different governing equations, models and correlations, and further improving the accuracy of system analysis codes. In the nuclear reactor system, CCFL is an important phenomenon for evaluating the safety of nuclear reactors. This is because CCFL phenomenon can limit injection of ECCS water when CCFL occurs in components such as hot leg, downcomer annulus or steam generator inlet plenum during LOCA which is possible to flow in two opposite directions. Therefore, CCFL is one of the thermal-hydraulic models which has significant effect on the reactor safety analysis code performance. In this study, the CCFL model was evaluated with MARS-KS and SPACE codes for studying the difference between system analysis codes with different governing equations, models and correlations. This study was conducted by comparing MARS-KS and SPACE code results of liquid upflow and liquid downflow rate for different gas flow rate to the famous Dukler

  4. An exploration of the prevalence and psychosocial aspects of Klinefelter Syndrome in the context of population-based genetic screening.

    OpenAIRE

    Herlihy, Amy Simone

    2017-01-01

    Klinefelter Syndrome (KS) is a genetic condition (47,XXY) affecting males and resulting in a spectrum of clinical features, ranging from azoospermia, small testes, breast development, testosterone deficiency, and decreased body and facial hair to varying levels of cognitive, social, behavioural and learning difficulties. The phenotype can be highly variable between individuals, and genetic factors such as functionality of the androgen receptor have been postulated to play a role. The pre...

  5. Down Syndrome

    Science.gov (United States)

    ... Down syndrome increases as a woman gets older. Down syndrome cannot be cured. Early treatment programs can help improve skills. They may include ... occupational, and/or educational therapy. With support and treatment, many ... Down syndrome live happy, productive lives. NIH: National Institute of ...

  6. Rowell syndrome

    Directory of Open Access Journals (Sweden)

    Ramesh Y Bhat

    2014-01-01

    Full Text Available Rowell syndrome is a rare disease consisting of erythema multiforme-like lesions associated with lupus erythematosus. The syndrome occurs mostly in middle-aged women. The authors describe the syndrome in a 15-year-old boy who responded well to systemic steroids and hydroxychloroquine.

  7. Aicardi Syndrome

    Science.gov (United States)

    ... from Aicardi-Goutieres syndrome, which is an inherited encephalopathy that affects newborn infants.) × Definition Aicardi syndrome is a rare genetic ... from Aicardi-Goutieres syndrome, which is an inherited encephalopathy that affects newborn infants.) View Full Definition Treatment There is no ...

  8. Klinefelter syndrome has increased brain responses to auditory stimuli and motor output, but not to visual stimuli or Stroop adaptation.

    Science.gov (United States)

    Wallentin, Mikkel; Skakkebæk, Anne; Bojesen, Anders; Fedder, Jens; Laurberg, Peter; Østergaard, John R; Hertz, Jens Michael; Pedersen, Anders Degn; Gravholt, Claus Højbjerg

    2016-01-01

    Klinefelter syndrome (47, XXY) (KS) is a genetic syndrome characterized by the presence of an extra X chromosome and low level of testosterone, resulting in a number of neurocognitive abnormalities, yet little is known about brain function. This study investigated the fMRI-BOLD response from KS relative to a group of Controls to basic motor, perceptual, executive and adaptation tasks. Participants (N: KS = 49; Controls = 49) responded to whether the words "GREEN" or "RED" were displayed in green or red (incongruent versus congruent colors). One of the colors was presented three times as often as the other, making it possible to study both congruency and adaptation effects independently. Auditory stimuli saying "GREEN" or "RED" had the same distribution, making it possible to study effects of perceptual modality as well as Frequency effects across modalities. We found that KS had an increased response to motor output in primary motor cortex and an increased response to auditory stimuli in auditory cortices, but no difference in primary visual cortices. KS displayed a diminished response to written visual stimuli in secondary visual regions near the Visual Word Form Area, consistent with the widespread dyslexia in the group. No neural differences were found in inhibitory control (Stroop) or in adaptation to differences in stimulus frequencies. Across groups we found a strong positive correlation between age and BOLD response in the brain's motor network with no difference between groups. No effects of testosterone level or brain volume were found. In sum, the present findings suggest that auditory and motor systems in KS are selectively affected, perhaps as a compensatory strategy, and that this is not a systemic effect as it is not seen in the visual system.

  9. Klinefelter syndrome has increased brain responses to auditory stimuli and motor output, but not to visual stimuli or Stroop adaptation

    Directory of Open Access Journals (Sweden)

    Mikkel Wallentin

    2016-01-01

    Full Text Available Klinefelter syndrome (47, XXY (KS is a genetic syndrome characterized by the presence of an extra X chromosome and low level of testosterone, resulting in a number of neurocognitive abnormalities, yet little is known about brain function. This study investigated the fMRI-BOLD response from KS relative to a group of Controls to basic motor, perceptual, executive and adaptation tasks. Participants (N: KS = 49; Controls = 49 responded to whether the words “GREEN” or “RED” were displayed in green or red (incongruent versus congruent colors. One of the colors was presented three times as often as the other, making it possible to study both congruency and adaptation effects independently. Auditory stimuli saying “GREEN” or “RED” had the same distribution, making it possible to study effects of perceptual modality as well as Frequency effects across modalities. We found that KS had an increased response to motor output in primary motor cortex and an increased response to auditory stimuli in auditory cortices, but no difference in primary visual cortices. KS displayed a diminished response to written visual stimuli in secondary visual regions near the Visual Word Form Area, consistent with the widespread dyslexia in the group. No neural differences were found in inhibitory control (Stroop or in adaptation to differences in stimulus frequencies. Across groups we found a strong positive correlation between age and BOLD response in the brain's motor network with no difference between groups. No effects of testosterone level or brain volume were found. In sum, the present findings suggest that auditory and motor systems in KS are selectively affected, perhaps as a compensatory strategy, and that this is not a systemic effect as it is not seen in the visual system.

  10. Hypothalamic dysfunction

    Science.gov (United States)

    ... apoplexy , subarachnoid hemorrhage Genetic disorders, such as Prader-Willi syndrome , familial diabetes insipidus , Kallmann syndrome Infections and ... commercial use must be authorized in writing by ADAM Health Solutions. About MedlinePlus Site Map FAQs Customer ...

  11. Dravets syndrom

    DEFF Research Database (Denmark)

    Hansen, Lars Kjaersgård; Rasmussen, Niels Henrik; Ousager, Lilian Bomme

    2010-01-01

    Dravet syndrome is an epileptic syndrome of infancy and early childhood. Most cases of Dravet syndrome seem to be due to a genetic defect causing the sodium channel to malfunction. We describe the main features of the syndrome. This epilepsy is medically intractable, but we call attention...... to the fact that some medications are of benefit and some could exacerbate the condition. Early recognition of the syndrome including by genetic testing could possibly improve outcome and reduce the need for other specialized investigations. Udgivelsesdato: 2010-Feb-22...

  12. Insights into Nitrate-Reducing Fe(II) Oxidation Mechanisms through Analysis of Cell-Mineral Associations, Cell Encrustation, and Mineralogy in the Chemolithoautotrophic Enrichment Culture KS.

    Science.gov (United States)

    Nordhoff, M; Tominski, C; Halama, M; Byrne, J M; Obst, M; Kleindienst, S; Behrens, S; Kappler, A

    2017-07-01

    Most described nitrate-reducing Fe(II)-oxidizing bacteria (NRFeOB) are mixotrophic and depend on organic cosubstrates for growth. Encrustation of cells in Fe(III) minerals has been observed for mixotrophic NRFeOB but not for autotrophic phototrophic and microaerophilic Fe(II) oxidizers. So far, little is known about cell-mineral associations in the few existing autotrophic NRFeOB. Here, we investigate whether the designated autotrophic Fe(II)-oxidizing strain (closely related to Gallionella and Sideroxydans ) or the heterotrophic nitrate reducers that are present in the autotrophic nitrate-reducing Fe(II)-oxidizing enrichment culture KS form mineral crusts during Fe(II) oxidation under autotrophic and mixotrophic conditions. In the mixed culture, we found no significant encrustation of any of the cells both during autotrophic oxidation of 8 to 10 mM Fe(II) coupled to nitrate reduction and during cultivation under mixotrophic conditions with 8 to 10 mM Fe(II), 5 mM acetate, and 4 mM nitrate, where higher numbers of heterotrophic nitrate reducers were present. Two pure cultures of heterotrophic nitrate reducers ( Nocardioides and Rhodanobacter ) isolated from culture KS were analyzed under mixotrophic growth conditions. We found green rust formation, no cell encrustation, and only a few mineral particles on some cell surfaces with 5 mM Fe(II) and some encrustation with 10 mM Fe(II). Our findings suggest that enzymatic, autotrophic Fe(II) oxidation coupled to nitrate reduction forms poorly crystalline Fe(III) oxyhydroxides and proceeds without cellular encrustation while indirect Fe(II) oxidation via heterotrophic nitrate-reduction-derived nitrite can lead to green rust as an intermediate mineral and significant cell encrustation. The extent of encrustation caused by indirect Fe(II) oxidation by reactive nitrogen species depends on Fe(II) concentrations and is probably negligible under environmental conditions in most habitats. IMPORTANCE Most described nitrate

  13. Insights into Nitrate-Reducing Fe(II) Oxidation Mechanisms through Analysis of Cell-Mineral Associations, Cell Encrustation, and Mineralogy in the Chemolithoautotrophic Enrichment Culture KS

    Science.gov (United States)

    Nordhoff, M.; Tominski, C.; Halama, M.; Byrne, J. M.; Obst, M.; Behrens, S.

    2017-01-01

    ABSTRACT Most described nitrate-reducing Fe(II)-oxidizing bacteria (NRFeOB) are mixotrophic and depend on organic cosubstrates for growth. Encrustation of cells in Fe(III) minerals has been observed for mixotrophic NRFeOB but not for autotrophic phototrophic and microaerophilic Fe(II) oxidizers. So far, little is known about cell-mineral associations in the few existing autotrophic NRFeOB. Here, we investigate whether the designated autotrophic Fe(II)-oxidizing strain (closely related to Gallionella and Sideroxydans) or the heterotrophic nitrate reducers that are present in the autotrophic nitrate-reducing Fe(II)-oxidizing enrichment culture KS form mineral crusts during Fe(II) oxidation under autotrophic and mixotrophic conditions. In the mixed culture, we found no significant encrustation of any of the cells both during autotrophic oxidation of 8 to 10 mM Fe(II) coupled to nitrate reduction and during cultivation under mixotrophic conditions with 8 to 10 mM Fe(II), 5 mM acetate, and 4 mM nitrate, where higher numbers of heterotrophic nitrate reducers were present. Two pure cultures of heterotrophic nitrate reducers (Nocardioides and Rhodanobacter) isolated from culture KS were analyzed under mixotrophic growth conditions. We found green rust formation, no cell encrustation, and only a few mineral particles on some cell surfaces with 5 mM Fe(II) and some encrustation with 10 mM Fe(II). Our findings suggest that enzymatic, autotrophic Fe(II) oxidation coupled to nitrate reduction forms poorly crystalline Fe(III) oxyhydroxides and proceeds without cellular encrustation while indirect Fe(II) oxidation via heterotrophic nitrate-reduction-derived nitrite can lead to green rust as an intermediate mineral and significant cell encrustation. The extent of encrustation caused by indirect Fe(II) oxidation by reactive nitrogen species depends on Fe(II) concentrations and is probably negligible under environmental conditions in most habitats. IMPORTANCE Most described nitrate

  14. Multiple Module Simulation of Water Cooled Breeding Blankets in K-DEMO Using Thermal-Hydraulic Analysis Code MARS-KS

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Geon-Woo; Lee, Jeong-Hun; Park, Goon-Cherl; Cho, Hyoung-Kyu [Seoul National University, Seoul (Korea, Republic of); Im, Kihak [National Fusion Research Institute, Daejeon (Korea, Republic of)

    2015-10-15

    A preliminary concept for the Korean fusion demonstration reactor (K-DEMO) has been studied by the National Fusion Research Institute (NFRI) based on the National Fusion Roadmap of Korea. The feasibility studies have been performed in order to establish the conceptual design guidelines of the breeding blanket. As a part of the NFRI research, Seoul National University (SNU) is conducting thermal design, evaluation and validation of the water-cooled breeding blanket for the K-DEMO reactor. The purpose of this study is to extend the capability of MARS-KS to the overall blanket system analysis which includes 736 blanket modules in total. The strategy for the multi-module blanket system analysis using MARS-KS is introduced and the analysis result of the 46 blanket modules of single sector was summarized. A thermal-hydraulic analysis code for a nuclear reactor safety, MARS-KS, was applied for thermal analysis of the conceptual design of the K-DEMO breeding blanket. Then, a methodology to simulate multiple blanket modules was proposed, which uses a supervisor program to handle each blanket module individually at first and then distribute the flow rate considering the pressure drop that occurs in each module. For a feasibility test of the proposed methodology, 46 blankets in a sector, which are connected with each other through the common headers for the sector inlet and outlet, were simulated. The calculation results of flow rates, pressure drops, and temperatures showed the validity of the calculation. Because of parallelization using the MPI system, the computational time could be reduced significantly. In future, this methodology will be extended to an efficient simulation of multiple sectors, and further validation for transient simulation will be carried out for more practical applications.

  15. Time-dependent amplitude analysis of semileptonically-tagged $D^0 \\to K_S^0 \\pi^+ \\pi^-$ decays at LHCb

    CERN Document Server

    INSPIRE-00392459

    2013-01-01

    The hadronic decay $D^0 \\to K_S^0 \\pi^+ \\pi^-$ provides access to the measurement of the mixing parameters of the neutral D-meson system and allows to test for CP violation. A measurement of the mixing parameters $x_D$ and $y_D$ as well as of the parameters $|q/p|$ and $\\phi=arg(q,p)$, which govern indirect CP violation, will be performed based on a time-dependent amplitude-model analysis of the full LHCb dataset of 2011 and 2012 corresponding to an integrated luminosity of 3/fb.

  16. MARS-KS Code Assessment for Condensation Heat Transfer in Horizontal Tube with the Presence of Non-Condensable Gas using Purdue Experiment

    International Nuclear Information System (INIS)

    Jeon, Seong Su; Lee, Byung Chul; Park, Ju Yeop; Seul, Kwang Won

    2011-01-01

    In South Korea, advanced power reactor plus (APR+), as a Korean specific reactor, is currently under development for the export strategy. In order to raise competitiveness of the APR+ in the world market, it is necessary to develop the original technology for the improved technology, economics, and safety features. For this purpose, a passive auxiliary feedwater system (PAFS) was adopted as an improved safety design concept of APR+: and then there have been many efforts to develop the PAFS. According to PAFS design concept, PAFS can completely replace the auxiliary feedwater system. When the design basis accident, in which feedwater is unavailable, occurs, the PAFS can remove the residual heat in the core and then prevent the core damage. In the PAFS with the horizontal type heat exchanger, two-phase natural circulation, condensation heat transfer in tube, boiling heat transfer in pool, natural convection in pool, etc. are considered as very important thermalhydraulic phenomena (see Fig. 1). Compared with the vertical heat exchanger from these phenomena, the major difference of the horizontal heat exchanger is the condensation heat transfer phenomena in the tube side. There have been many efforts to understand the condensation heat transfer with in the presence of NC gas in tube but most researches focused on the condensation heat transfer in vertical tube. Therefore the details of the condensation heat transfer in the presence of NC gas in horizontal condenser tubes are not well understood. In order to develop the safety evaluation system for APR+ PAFS, it is required to evaluate the capability and applicability of the MARS-KS code for modeling the condensation heat transfer in the horizontal tube with NC gas because many heat transfer correlations in MARS-KS are known to have much uncertainty. In particular, there is no reliable model for the condensation phenomena in horizontal tube with NC gas. In order to assess the MARS-KS code results and identify the

  17. Urofacial syndrome

    Directory of Open Access Journals (Sweden)

    Kamal F Akl

    2012-01-01

    Full Text Available The urofacial syndrome is characterized by functional obstructive uropathy asso-ciated with an inverted smile. The importance of the subject is that it sheds light, not only on the muscles of facial expression, but also on the inheritance of voiding disorders and lower urinary tract malformations. We report a 10-year-old-male patient who had the urofacial syndrome. Early diagnosis of the urofacial syndrome is important to avoid upper urinary tract damage and renal failure.

  18. Refeeding syndrome

    OpenAIRE

    Tripathy, Swagata; Mishra, Padmini; Dash, S. C.

    2008-01-01

    Refeeding syndrome is a potentially fatal medical condition that may affect malnourished patients in response to an inappropriately rapid overfeeding. This commonly occurs following the institution of nutritional support, especially parenteral or enteral nutrition. The most characteristic pathophysiology of refeeding syndrome relates to the rapid consumption of phosphate after glucose intake and subsequent hypophosphatemia. Refeeding syndrome can manifest as either metabolic changes (hypokala...

  19. Revesz syndrome

    Directory of Open Access Journals (Sweden)

    Dayane Cristine Issaho

    2015-04-01

    Full Text Available Revesz syndrome is a rare variant of dyskeratosis congenita and is characterized by bilateral exudative retinopathy, alterations in the anterior ocular segment, intrauterine growth retardation, fine sparse hair, reticulate skin pigmentation, bone marrow failure, cerebral calcification, cerebellar hypoplasia and psychomotor retardation. Few patients with this syndrome have been reported, and significant clinical variations exist among patients. This report describes the first Brazilian case of Revesz syndrome and its ocular and clinical features.

  20. Reye's Syndrome

    Science.gov (United States)

    ... that contain aspirin. Some hospitals and medical facilities conduct newborn screenings for fatty acid oxidation disorders to determine which children are at greater risk of developing Reye's syndrome. ...

  1. Marfan Syndrome (For Teens)

    Science.gov (United States)

    ... genetic disorder called Marfan syndrome. What Is Marfan Syndrome? Marfan syndrome is named after Antoine Marfan, the French ... immediately. What's Life Like for Teens With Marfan Syndrome? Marfan syndrome affects people differently, so life is not ...

  2. Learning about Marfan Syndrome

    Science.gov (United States)

    ... Additional Resources for Marfan Syndrome What is Marfan syndrome? Marfan syndrome is one of the most common inherited ... FAQ Top of page Additional Resources For Marfan Syndrome Marfan syndrome [nlm.nih.gov] From Medline Plus Marfan ...

  3. Russell-Silver syndrome

    Science.gov (United States)

    Silver-Russell syndrome; Silver syndrome; RSS; Russell-Silver syndrome ... One in 10 children with this syndrome has a problem involving chromosome 7. In other people with the syndrome, it may affect chromosome 11. Most of the time, it ...

  4. What Is Usher Syndrome?

    Science.gov (United States)

    ... Action You are here Home › Retinal Diseases Listen Usher Syndrome What is Usher syndrome? How is Usher syndrome ... available? Are there any related diseases? What is Usher Syndrome? Usher syndrome is an inherited condition characterized by ...

  5. Prevalence and severity of behavioural symptoms in patients with Korsakoff syndrome and other alcohol-related cognitive disorders: a systematic review.

    Science.gov (United States)

    Gerridzen, Ineke J; Moerman-van den Brink, Wiltine G; Depla, Marja F; Verschuur, Els M L; Veenhuizen, Ruth B; van der Wouden, Johannes C; Hertogh, Cees M P M; Joling, Karlijn J

    2017-03-01

    Experiences from clinical practice suggest that behavioural symptoms in patients with Korsakoff syndrome (KS) are a frequent problem. Knowledge about behavioural symptoms is important in understanding and managing these symptoms. The aim of this study is to review the prevalence and severity of behavioural symptoms in KS. Relevant articles were identified by searching Medline (PubMed), PsycINFO, Embase and CINAHL up to 4 June 2014. Two reviewers independently selected the studies, extracted their baseline data and assessed methodological quality using a standardized checklist. Fifteen studies fulfilled the inclusion criteria. A diversity of diagnoses was used indicating that KS and other alcohol-related cognitive disorders and terms were used interchangeably. None of the studies were primarily designed to estimate the prevalence or severity of behavioural symptoms in patients with KS. Most studies had serious methodological limitations. The reported prevalence estimates of behavioural symptoms in the included studies varied strongly. Most prevalent were depressive symptoms and disorders (2-50%, median 27%) and agitation and aggression (10-54%, median 27%). None of the reported, mean severity estimates met pathological thresholds. The highest severity estimates were found for apathy. Good quality studies on behavioural symptoms in patients with KS are lacking. Observational research designed to provide reliable estimates of the prevalence and severity of behavioural symptoms in patients with KS is needed. This could improve understanding and managing these symptoms and help care staff to better support the needs of this specific patient group. Copyright © 2016 John Wiley & Sons, Ltd. Copyright © 2016 John Wiley & Sons, Ltd.

  6. Seckel syndrome: an overdiagnosed syndrome.

    OpenAIRE

    Thompson, E; Pembrey, M

    1985-01-01

    Five children in whom a diagnosis of Seckel syndrome had previously been made were re-examined in the genetic unit. One child had classical Seckel syndrome, a sib pair had the features of the syndrome with less severe short stature, and in two children the diagnosis was not confirmed. Seckel syndrome is only one of a group of low birth weight microcephalic dwarfism and careful attention should be paid to fulfillment of the major criteria defined by Seckel before the diagnosis is made. There r...

  7. A model-independent approach to mixing in prompt $D^{0} \\rightarrow K_S^0 \\pi^+ \\pi^-$ decays at LHCb

    CERN Document Server

    Torr, Nicholas; Charles, Matthew

    This thesis presents a measurement of the charm mixing parameters $x_D$ and $y_D$ in prompt $D^{0} \\rightarrow K_S^0 \\pi^+ \\pi^-$ decays using 1 fb$^{-1}$ of data collected by the LHCb experiment during 2011. \\par Mixing in charm is predicted to be small within the Standard Model, but there are significant uncertainties associated with calculating the long range contributions to the decay. Recent measurements made by LHCb and others have confirmed that mixing in charm exists at a rate of less than 1 %. With LHCb due to collect more data and Belle II being commissioned, the reduction of systematic uncertainties will become increasingly important. The $D^{0} \\rightarrow K_S^0 \\pi^+ \\pi^-$ decay provides sensitivity to both the magnitude and relative sign between the mixing parameters. It is also one of the few channels that can measure $x_D$ directly. It is therefore crucial to study this mode in detail as more data becomes available. The work presented in this thesis utilises a model-independent description of...

  8. Burnout Syndrome

    OpenAIRE

    Panova, Gordana; Panov, Nenad; Stojanov, H; Sumanov, Gorgi; Panova, Blagica; Stojanovski, Angel; Nikolovska, Lence; Jovevska, Svetlana; Trajanovski, D; Asanova, D

    2013-01-01

    Introduction: Increasing work responsibilities, allocation of duties, loss of energy and motivation in everyday activities, emotional exhaustion, lack of time for themselves, insuffi cient time for rest and recreation, dissatisfaction in private life. All these symptoms can be cause of Burnout Syndrome. Aim: To see the importance of this syndrome, the consequences of job dissatisfaction, the environment, family and expression in drastic chan...

  9. Tourette Syndrome

    Science.gov (United States)

    If you have Tourette syndrome, you make unusual movements or sounds, called tics. You have little or no control over them. Common tics are throat- ... spin, or, rarely, blurt out swear words. Tourette syndrome is a disorder of the nervous system. It ...

  10. Fahr's Syndrome

    Science.gov (United States)

    ... or 50s, although it can occur at any time in childhood or adolescence. × Definition Fahr's Syndrome is a rare, genetically dominant, inherited ... or 50s, although it can occur at any time in childhood or adolescence. View Full Definition Treatment There is no cure for Fahr's Syndrome, ...

  11. Lemierre's syndrome

    DEFF Research Database (Denmark)

    Johannesen, Katrine; Bødtger, Uffe; Heltberg, Ole

    2014-01-01

    Lemierre's syndrome is an often un-diagnosed disease seen in previously healthy young subjects, presenting with symptoms of pharyngitis, fever and elevated markers of inflammation. The syndrome is characterised by infectious thrombosis of the jugular vein due to infection with Fusobacteria, causing...

  12. Ambras syndrome

    Directory of Open Access Journals (Sweden)

    Sudhir Malwade

    2015-01-01

    Full Text Available Ambras syndrome, a form of congenital hypertrichosis lanuginosa, is extremely rare in neonates. It is characterized by typical pattern of hair distribution, dysmorphic facial features and a familial pattern of inheritance. We report a case of Ambras syndrome in a preterm neonate with history of consanguinity and positive family history.

  13. Antiphospholipid syndrome

    DEFF Research Database (Denmark)

    Cervera, Ricard; Piette, Jean-Charles; Font, Josep

    2002-01-01

    To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression.......To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression....

  14. Noonan syndrome

    OpenAIRE

    Roberts, Amy E; Allanson, Judith E; Tartaglia, Marco; Gelb, Bruce D

    2013-01-01

    Noonan syndrome is a genetic multisystem disorder characterised by distinctive facial features, developmental delay, learning difficulties, short stature, congenital heart disease, renal anomalies, lymphatic malformations, and bleeding difficulties. Mutations that cause Noonan syndrome alter genes encoding proteins with roles in the RAS–MAPK pathway, leading to pathway dysregulation. Management guidelines have been developed. Several clinically relevant genotype–phenotype correlations aid ris...

  15. TAFRO Syndrome.

    Science.gov (United States)

    Igawa, Takuro; Sato, Yasuharu

    2018-02-01

    TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD. TAFRO syndrome follows a more aggressive course, compared with iMCD-NOS, and there is no standard treatment. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Goldenhar syndrome

    Directory of Open Access Journals (Sweden)

    Neeraj Sharma

    2013-01-01

    Full Text Available Goldenhar syndrome is a syndrome of complex structures developing from first and second branchial arches during blastogenesis. The etiology of this rare disease is not fully understood, as it has shown itself variable genetically and of unclear causes. The disorder is characterized by a wide spectrum of symptoms and physical features that may vary greatly in range and severity from case to case. Here we present a unique case of Goldenhar syndrome with absence of left condyle, hypoplasia of the zygomatic bone, no pneumatization of the mastoid process, underdeveloped mandible, bifid tongue and the skin tags in the preauricular area.

  17. Cowden syndrome

    Directory of Open Access Journals (Sweden)

    Ravi Prakash S

    2010-01-01

    Full Text Available Cowden syndrome or multiple hamartoma syndrome is an autosomal dominant condition with variable expressions that result mainly from mutation in the PTEN gene on arm 10q. It is characterized by multiple hamartomatous neoplasms of the skin, oral mucosa, gastrointestinal tract, bones, CNS, eyes, and genitourinary tract. Mucocutaneous features include trichilemmomas, oral mucosal papillomatosis, acral keratosis, and palmoplantar keratosis. Here we present a case of Cowden syndrome in a 14-year-old female patient with the chief complaint of multiple oral papillomatous lesions.

  18. Costello syndrome

    Directory of Open Access Journals (Sweden)

    Madhukara J

    2007-01-01

    Full Text Available Costello syndrome is a rare, distinctive, multiple congenital anomaly syndrome, characterized by soft, loose skin with deep palmar and plantar creases, loose joints, distinctive coarse facial features and skeletal and cardiac abnormalities. The affected patients have a predisposition to develop malignancy, developmental delays and mental retardation. Recently, a 7-year-old male child born to normal nonconsanguineous parents presented to us with abnormal facial features, arrhythmia, mitral valve dysfunction and growth retardation. His cutaneous examination revealed lax and pigmented skin over hands and feet with deep creases, acanthosis nigricans and short curly hairs. Its differentiation from other syndromes with similar clinical features is discussed in this article.

  19. Simulation of an SBLOCA Test of Shutdown Cooling System Line Break with the SMARTITL Facility using the MARS-KS Code

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Yeon-Sik; Suh, Jae-Seung [System Engineering and Technology, Daejeon (Korea, Republic of); Bae, Hwang; Ryu, Sung-Uk; Yi, Sung-Jae; Park, Hyun-Sik [Korea Atomic Energy Research Institute, Daejeon (Korea, Republic of)

    2014-10-15

    An LBLOCA (Large-Break Loss of Coolant Accident) was inherently eliminated in the design stage. The SMART design has a thermal power of 330MW. Its core exit temperature and pressurizer pressure are 323 .deg. C and 15MPa during normal operating conditions, respectively. An integral-effect test loop for SMART (SMARTITL), called FESTA (Facility for Experimental Simulation of Transients and Accidents), was designed to simulate the integral thermal-hydraulic behavior of SMART. The objectives of SMART-ITL are to investigate and understand the integral performance of reactor systems and components, and the thermal-hydraulic phenomena occurring in the system during normal, abnormal, and emergency conditions, and to verify the system safety during various design basis events of SMART. SMART-ITL with four steam generators and PRHRS, has an advantage for a multi-loop effect compared with VISTA-ITL with a single loop. The integral-effect test data will also be used to validate the related thermal-hydraulic models of the safety analysis code such as TASS/SMR-S which is used for a performance and accident analysis of the SMART design. In addition, a scoping analysis on the scaling difference between the standard design of SMART and the basic design of SMART-ITL was performed for an SBLOCA (Small-Break Loss of Coolant Accident) scenario using a best-estimate safety analysis code, MARS-KS. This paper introduces a comparison of an SBLOCA test of a shutdown cooling system line break using SMART-ITL with its post-test calculation using the MARS-KS code. An SBLOCA test and its post-test calculation were successfully performed using the SMART-ITL facility and MARS-KS code. The SBLOCA break is a guillotine break, and its location is on the SCS line (nozzle part of the RCP suction). The steady-state conditions were achieved to satisfy the initial test conditions presented in the test requirement and its boundary conditions were properly simulated.

  20. Deafness and Mondini dysplasia in Kabuki (Niikawa-Kuroki) syndrome. Report of a case and review of the literature.

    Science.gov (United States)

    Toutain, A; Plée, Y; Ployet, M J; Benoit, S; Perrot, A; Sembely, C; Barthez, M A; Moraine, C

    1997-01-01

    Report of a case and review of the literature: We report the case of a seven-year-old female kabuki patient suffering from severe bilateral deafness related to Mondini dysplasia and ossicular anomalies. A review of the literature in English confirms that hearing loss is a major component of Kabuki Syndrome (KS) with a frequency at around 32%. However the possible mechanisms have not been fully described and hearing loss is often attributed to otitis media, but one reported case had severe ossicular malformations, two had sensorineural deafness and three others had mixed deafness. Our observation is the first reported case of Mondini dysplasia in KS. Awareness by physicians of this problem has a major practical consequence as diagnosis of Mondini dysplasia implies searching for and surgical prevention and treatment of perilymphatic fistula in order to prevent meningitis.

  1. Expanding the phenotypic profile of Kleefstra syndrome: A female with low-average intelligence and childhood apraxia of speech.

    Science.gov (United States)

    Samango-Sprouse, Carole; Lawson, Patrick; Sprouse, Courtney; Stapleton, Emily; Sadeghin, Teresa; Gropman, Andrea

    2016-05-01

    Kleefstra syndrome (KS) is a rare neurogenetic disorder most commonly caused by deletion in the 9q34.3 chromosomal region and is associated with intellectual disabilities, severe speech delay, and motor planning deficits. To our knowledge, this is the first patient (PQ, a 6-year-old female) with a 9q34.3 deletion who has near normal intelligence, and developmental dyspraxia with childhood apraxia of speech (CAS). At 6, the Wechsler Preschool and Primary Intelligence testing (WPPSI-III) revealed a Verbal IQ of 81 and Performance IQ of 79. The Beery Buktenica Test of Visual Motor Integration, 5th Edition (VMI) indicated severe visual motor deficits: VMI = 51; Visual Perception = 48; Motor Coordination explanation for the previously reported speech delay and expressive language disorder. Further research is warranted on the impact of CAS on intelligence and behavioral outcome in KS. Therapeutic and prognostic implications are discussed. © 2016 Wiley Periodicals, Inc.

  2. Neuroimaging of the Wernicke–Korsakoff Syndrome

    Science.gov (United States)

    Sullivan, Edith V.; Pfefferbaum, Adolf

    2009-01-01

    Aim: Presented is the neuroradiological signature of acute Wernicke's encephalopathy (WE), derived from different types of magnetic resonance imaging (MRI) sequences. WE results from thiamine depletion, and its most typical antecedent is chronic alcohol dependence. Brain regions observed with in vivo MRI affected in acute WE include the mammillary bodies, periaqueductal and periventricular gray matter, collicular bodies and thalamus. These affected areas are usually edematous and are best visualized and quantified with MRI sequences that highlight such tissue. Following the acute WE phase and resolution of edema and inflammation of affected brain tissue, WE, if not adequately treated with thiamine repletion, can herald Korsakoff's syndrome (KS), with its symptomatic hallmark of global amnesia, that is, the inability to commit newly encountered (episodic) information to memory for later recall or recognition. Methods: Neuropathology of KS detectable with MRI has a different neuroradiological signature from the acute stage and can be observed as tissue shrinkage or atrophy of selective brain structures, including the mammillary bodies and thalamus and ventricular expansion, probably indicative of atrophy of surrounding gray matter nuclei. Quantification of these and additional gray matter structures known to underlie global amnesia reveal substantial bilateral volume deficits in the hippocampus, in addition to the mammillary bodies and thalamus, and modest deficits in the medial septum/diagonal band of Broca. The infratentorium is also affected, exhibiting volume deficits in cerebellar hemispheres, anterior superior vermis and pons, contributing to ataxia of gait and stance. Results: Consideration of WKS structural brain changes in the context of the neuropathology of non-WKS alcoholism revealed a graded pattern of volume deficits, from mild in non-WKS alcoholics to moderate or severe in WKS, in the mammillary bodies, hippocampus, thalamus, cerebellum and pons. The

  3. Neuroimaging of the Wernicke-Korsakoff syndrome.

    Science.gov (United States)

    Sullivan, Edith V; Pfefferbaum, Adolf

    2009-01-01

    Presented is the neuroradiological signature of acute Wernicke's encephalopathy (WE), derived from different types of magnetic resonance imaging (MRI) sequences. WE results from thiamine depletion, and its most typical antecedent is chronic alcohol dependence. Brain regions observed with in vivo MRI affected in acute WE include the mammillary bodies, periaqueductal and periventricular gray matter, collicular bodies and thalamus. These affected areas are usually edematous and are best visualized and quantified with MRI sequences that highlight such tissue. Following the acute WE phase and resolution of edema and inflammation of affected brain tissue, WE, if not adequately treated with thiamine repletion, can herald Korsakoff's syndrome (KS), with its symptomatic hallmark of global amnesia, that is, the inability to commit newly encountered (episodic) information to memory for later recall or recognition. Neuropathology of KS detectable with MRI has a different neuroradiological signature from the acute stage and can be observed as tissue shrinkage or atrophy of selective brain structures, including the mammillary bodies and thalamus and ventricular expansion, probably indicative of atrophy of surrounding gray matter nuclei. Quantification of these and additional gray matter structures known to underlie global amnesia reveal substantial bilateral volume deficits in the hippocampus, in addition to the mammillary bodies and thalamus, and modest deficits in the medial septum/diagonal band of Broca. The infratentorium is also affected, exhibiting volume deficits in cerebellar hemispheres, anterior superior vermis and pons, contributing to ataxia of gait and stance. Consideration of WKS structural brain changes in the context of the neuropathology of non-WKS alcoholism revealed a graded pattern of volume deficits, from mild in non-WKS alcoholics to moderate or severe in WKS, in the mammillary bodies, hippocampus, thalamus, cerebellum and pons. The development and

  4. Categorization abilities for emotional and nonemotional stimuli in patients with alcohol-related Korsakoff syndrome.

    Science.gov (United States)

    Labudda, Kirsten; von Rothkirch, Nadine; Pawlikowski, Mirko; Laier, Christian; Brand, Matthias

    2010-06-01

    To investigate whether patients with alcohol-related Korsakoff syndrome (KR) have emotion-specific or general deficits in multicategoric classification performance. Earlier studies have shown reduced performance in classifying stimuli according to their emotional valence in patients with KS. However, it is unclear whether such classification deficits are of emotion-specific nature or whether they can also occur when nonemotional classifications are demanded. In this study, we examined 35 patients with alcoholic KS and 35 healthy participants with the Emotional Picture Task (EPT) to assess valence classification performance, the Semantic Classification Task (SCT) to assess nonemotional categorizations, and an extensive neuropsychologic test battery. KS patients exhibited lower classification performance in both tasks compared with the healthy participants. EPT and SCT performance were related to each other. EPT and SCT performance correlated with general knowledge and EPT performance in addition with executive functions. Our results indicate a common underlying mechanism of the patients' reductions in emotional and nonemotional classification performance. These deficits are most probably based on problems in retrieving object and category knowledge and, partially, on executive functioning.

  5. Endocrinological issues and hormonal manipulation in children and men with Klinefelter syndrome.

    Science.gov (United States)

    Wosnitzer, Matthew S; Paduch, Darius A

    2013-02-15

    47, XXY or Klinefelter syndrome (KS), the most common chromosomal aberration in males, is characterized by either absolute or relative hypogonadism with frequent decline in serum testosterone (T) following the onset of puberty. Decreased T levels are the result of testicular dysfunction with decrease in size of Leydig cells, and loss of germs and Sertoli cells leading to tubular hyalinization. Increase in estradiol results from over-expression of aromatase CYP19. Deficient androgen production and observed varied response of end-organs to T leads to delayed progression of puberty with decreased facial/body hair, poor muscle development, osteoporosis, and gynecomastia. It is possible that hypogonadism and excessive estradiol production contribute to emotional and social immaturity, and specific learning disabilities in KS. Based on the authors' experience and literature review, early fertility preservation and hormonal supplementation may normalize pubertal development, prevent metabolic sequelae of hypogonadism, and have a positive effect on academic and social development. No randomized clinical trials are available studying the effects of T supplementation on reproductive or cognitive issues in KS. Aggressive T supplementation (topical gel) and selective use of aromatase inhibitors may be considered at the onset of puberty with careful follow-up and titration to reach age-specific high-normal physiologic serum values. The decision to institute hormonal therapy should be part of a multidisciplinary approach including physical, speech, behavioral, and occupational therapy. © 2013 Wiley Periodicals, Inc. Copyright © 2013 Wiley Periodicals, Inc.

  6. Visualization of coronary arteries in patients after childhood Kawasaki syndrome: value of multidetector CT and MR imaging in comparison to conventional coronary catheterization

    International Nuclear Information System (INIS)

    Arnold, Raoul; Ley, Sebastian; Ley-Zaporozhan, Julia; Kauczor, Hans-Ulrich; Eichhorn, Joachim; Ulmer, Herbert; Schenk, Jens-Peter

    2007-01-01

    After childhood Kawasaki syndrome (KS) the coronary arteries undergo a lifelong dynamic pathological change, and follow-up coronary artery imaging is essential. At present, conventional coronary catheterization (CCC) and angiography is still regarded as the gold standard. Less-invasive methods such as multidetector CT angiography (MDCT-A) and MRI have been used sporadically. To compare the diagnostic quality of MDCT-A and MRI with that of CCC for coronary imaging in a group of patients with coronary artery pathology after childhood KS. A total of 16 patients (aged 5-27 years) underwent CCC and 16-row MDCT-A and 14 patients MRI (1.5 T). There was 100% agreement between MDCT-A and CCC in the detection of coronary aneurysms and stenoses. MDCT-A was superior for the visualization of calcified lesions. MRI and CCC showed 93% agreement for the detection of aneurysms. Visualization of coronary artery stenoses was difficult using MRI - one stenosis was missed. MDCT-A has excellent correlation with CCC regarding all changes affecting the coronary arteries in the follow-up of childhood KS. In comparison to MDCT-A and CCC, MRI is less precise in the detection of stenotic lesions. Due to its high image quality and ease of performance MDCT-A should be the primary diagnostic modality in patients following childhood KS. (orig.)

  7. Long-Term Mortality of Patients with an Alcohol-Related Wernicke-Korsakoff Syndrome.

    Science.gov (United States)

    Sanvisens, Arantza; Zuluaga, Paola; Fuster, Daniel; Rivas, Inmaculada; Tor, Jordi; Marcos, Miguel; Chamorro, Antonio J; Muga, Roberto

    2017-07-01

    To characterize a series of contemporary patients with alcohol-related Wernicke's encephalopathy (WE) or Korsakoff's syndrome (KS) and to update the current prognosis of disease. Retrospective and prospective study of patients diagnosed with an alcohol-related WE or KS between 2002 and 2011 in a tertiary hospital. Socio-demographic, alcohol use characteristics, signs and symptoms, co-morbidity and blood parameters were obtained at admission. Patients were followed up until 2013 and causes of death were ascertained through the review of charts. Sixty-one patients were included (51 with WE and 10 with KS). Among patients with WE, 78% were men and age at diagnosis was 57 years (interquartile range (IQR): 49-66). Twenty-three percent fulfilled the classic WE triad. Regarding Caine's criteria for WE, 70.6% presented with at least two out of four signs or symptoms. Median follow-up of patients with WE syndrome was 5.3 years (IQR: 2.6-8.8), the cumulated mortality was 45% and death rate of 7.4 × 100 person-years (95% confidence interval (CI): 4.8-10.9). Overall, 50% of patients would be expected to die within 8 years of WE episode and main causes of death included serious bacterial infections (44.5%) and cancer (33.3%). Survival of patients with an alcohol-related Wernicke-Korsakoff syndrome is poor; pursuing treatment of alcohol use disorder and early diagnosis of thiamine deficiency is a priority for improving clinical outcomes. © The Author 2017. Medical Council on Alcohol and Oxford University Press. All rights reserved.

  8. Reye Syndrome

    Science.gov (United States)

    ... Legacy Society Make Gifts of Stock Donate Your Car Personal Fundraising Partnership & Support Share Your Story Spread the Word Give While You Shop Contact Us Donate Now What Is Reye’s Syndrome? ...

  9. Alagille Syndrome

    Science.gov (United States)

    ... Legacy Society Make Gifts of Stock Donate Your Car Personal Fundraising Partnership & Support Share Your Story Spread the Word Give While You Shop Contact Us Donate Now Alagille Syndrome Back Alagille ...

  10. Turner Syndrome

    Science.gov (United States)

    ... Failure to begin sexual changes expected during puberty Sexual development that "stalls" during teenage years Early end to menstrual cycles not due to pregnancy For most women with Turner syndrome, inability to ...

  11. [Refeeding syndrome].

    Science.gov (United States)

    Ševela, Stanislav; Novák, František; Kazda, Antonín; Brodská, Helena

    Despite being known more than 60 years, refeeding syndrome (RS) still bears many uncertainties. For example, its definition is not clear and definite, and the attitude to it varies from the complete neglect to over-prevention.The term "refeeding syndrome" refers to electrolyte and metabolic changes occurring in malnourished patients after the readministration of nutrition. These changes concern especially to phosphates and ions. Potassium, magnesium, naturism and fluids balance are involved. The changes lead to cell energetic metabolism and electric potential disturbances, with related clinical symptoms.Fully developed refeeding syndrome is quite rare; nevertheless it can be fatal for the patient. However, even its development can lead to many complications increasing the patient's morbidity and the length of stay in the hospital. Yet the refeeding syndrome is more or less predictable and if kept in mind also preventable.The aim of this article is to get the reader to know more about this metabolic phenomenon and possible attitudes towards it.

  12. Cockayne syndrome

    DEFF Research Database (Denmark)

    Karikkineth, Ajoy C; Scheibye-Knudsen, Morten; Fivenson, Elayne

    2017-01-01

    Cockayne syndrome (CS) is a disorder characterized by a variety of clinical features including cachectic dwarfism, severe neurological manifestations including microcephaly and cognitive deficits, pigmentary retinopathy, cataracts, sensorineural deafness, and ambulatory and feeding difficulties...

  13. Alagille Syndrome

    Science.gov (United States)

    ... Liver Function Tests Clinical Trials Liver Transplant FAQs Medical Terminology Diseases of the Liver Alagille Syndrome Alcohol-Related ... the Liver The Progression of Liver Disease FAQs Medical Terminology HOW YOU CAN HELP Sponsorship Ways to Give ...

  14. Reye Syndrome

    Science.gov (United States)

    ... Liver Function Tests Clinical Trials Liver Transplant FAQs Medical Terminology Diseases of the Liver Alagille Syndrome Alcohol-Related ... the Liver The Progression of Liver Disease FAQs Medical Terminology HOW YOU CAN HELP Sponsorship Ways to Give ...

  15. Turner Syndrome

    Science.gov (United States)

    ... crowding, and osteoporosis (brittle bones). Because of their physical conditions, health concerns, and infertility, some girls and women with TS may have low self- esteem, anxiety, or depression. How is Turner syndrome diagnosed? Physical features may ...

  16. Cushing's Syndrome

    Science.gov (United States)

    ... person cured of Cushing’s syndrome might have some memory loss and slight mental decline. But the change is ... Categories: Family Health, Infants and Toddlers, Kids and Teens, Men, Seniors, WomenTags: acth, adenomas, hormone, sickness September ...

  17. Levator Syndrome

    Science.gov (United States)

    ... Abscess Anorectal Fistula Foreign Objects in the Rectum Hemorrhoids Levator Syndrome Pilonidal Disease Proctitis Rectal Prolapse (See ... out other painful rectal conditions (such as thrombosed hemorrhoids , fissures , or abscesses ). The physical examination is often ...

  18. Alport Syndrome

    Science.gov (United States)

    ... signs and symptoms may differ, based on age, gender and inherited type of Alport syndrome. For example, ... prevention and treatment of kidney disease. The Better Business Bureau Wise Giving Alliance Charity Seal provides the ...

  19. Gilbert's Syndrome

    Science.gov (United States)

    ... not know you have the condition until it's discovered by accident, such as when a blood test ... chemotherapy drug Some protease inhibitors used to treat HIV If you have Gilbert's syndrome, talk to your ...

  20. Potter syndrome

    Science.gov (United States)

    Potter phenotype ... In Potter syndrome, the primary problem is kidney failure. The kidneys fail to develop properly as the baby is ... kidneys normally produce the amniotic fluid (as urine). Potter phenotype refers to a typical facial appearance that ...

  1. Moebius Syndrome

    Science.gov (United States)

    ... delays; high or cleft palate; hearing problems and speech difficulties. Children with Moebius syndrome are unable to move their eyes back and forth. Decreased numbers of muscle fibers have been reported. Deformities of the tongue, jaw, and limbs, such ...

  2. Fraser syndrome

    DEFF Research Database (Denmark)

    Barisic, Ingeborg; Odak, Ljubica; Loane, Maria

    2013-01-01

    Fraser syndrome is a rare autosomal recessive disorder characterized by cryptophthalmos, cutaneous syndactyly, laryngeal, and urogenital malformations. We present a population-based epidemiological study using data provided by the European Surveillance of Congenital Anomalies (EUROCAT) network of...

  3. Angelman Syndrome

    Science.gov (United States)

    ... therapy for seizures is usually necessary. Physical and occupational therapies, communication therapy, and behavioral therapies are important in allowing individuals with Angelman syndrome to reach their maximum developmental potential. × Treatment There ...

  4. Joubert Syndrome

    Science.gov (United States)

    ... CEP290 . View Full Definition Treatment Treatment for Joubert syndrome is symptomatic and supportive. Infant stimulation and physical, occupational, and speech therapy may benefit some children. Infants with abnormal breathing ...

  5. Zellweger Syndrome

    Science.gov (United States)

    ... swallow. Some babies will be born with glaucoma, retinal degeneration, and impaired hearing. Jaundice and gastrointestinal bleeding also may occur. Treatment There is no cure for Zellweger syndrome, nor ...

  6. Nephrotic Syndrome

    Science.gov (United States)

    ... your blood — typically with an artificial kidney machine (dialyzer). Chronic kidney disease. Nephrotic syndrome may cause your ... opportunities Reprint Permissions A single copy of these materials may be reprinted for noncommercial personal use only. " ...

  7. Ohtahara Syndrome

    Science.gov (United States)

    ... are more often affected than girls. View Full Definition Treatment Antiepileptic drugs are used to control seizures, but are unfortunately ... Other therapies are symptomatic and supportive. × ... Definition Ohtahara syndrome is a neurological disorder characterized by ...

  8. Usher Syndrome

    Science.gov (United States)

    ... to abnormal development of the vestibular hair cells, sensory cells that detect gravity and head movement. RP ... 3 Ben-Rebeh, I., et al. (2016). Genetic analysis of Tunisian families with Usher syndrome type 1: ...

  9. Clinical characteristics of 138 Chinese female patients with idiopathic hypogonadotropic hypogonadism

    Directory of Open Access Journals (Sweden)

    Rui-yi Tang

    2017-11-01

    Full Text Available Objective: To evaluate the clinical features of Chinese women with idiopathic hypogonadotropic hypogonadism (IHH. Methods: We retrospectively reviewed the clinical characteristics, laboratory and imaging findings, therapeutic management and fertility outcomes of 138 women with IHH. All patients had been treated and followed up at an academic medical centre during 1990–2016. Results: Among the 138 patients, 82 patients (59.4% were diagnosed with normosmic IHH and 56 patients (40.6% were diagnosed with Kallmann syndrome (KS. The patients with IHH experienced occasional menses (4.3%, spontaneous thelarche (45.7% or spontaneous pubarche (50.7%. Women with thelarche had a higher percentage of pubarche (P < 0.001 and higher gonadotropin concentrations (P < 0.01. Olfactory bulb/sulci abnormalities were found during the magnetic resonance imaging (MRI of all patients with KS. Most patients with IHH had osteopenia and low bone age. Among the 16 women who received gonadotropin-releasing hormone treatment, ovulation induction or assisted reproductive technology, the clinical pregnancy rate was 81.3% and the live birth rate was 68.8%. Conclusions: The present study revealed that the phenotypic spectrum of women with IHH is broader than typical primary amenorrhoea with no secondary sexual development, including occasional menses, spontaneous thelarche or pubarche. MRI of the olfactory system can facilitate the diagnosis of KS. Pregnancy can be achieved after receiving appropriate treatment.

  10. Eagle's Syndrome

    OpenAIRE

    Pinheiro,Thaís Gonçalves; Soares,Vítor Yamashiro Rocha; Ferreira,Denise Bastos Lage; Raymundo,Igor Teixeira; Nascimento,Luiz Augusto; Oliveira,Carlos Augusto Costa Pires de

    2013-01-01

    Summary Introduction:?Eagle's syndrome is characterized by cervicopharyngeal signs and symptoms associated with elongation of the styloid apophysis. This elongation may occur through ossification of the stylohyoid ligament, or through growth of the apophysis due to osteogenesis triggered by a factor such as trauma. Elongation of the styloid apophysis may give rise to intense facial pain, headache, dysphagia, otalgia, buzzing sensations, and trismus. Precise diagnosis of the syndrome is diffic...

  11. Barth Syndrome

    DEFF Research Database (Denmark)

    Saric, Ana; Andreau, Karine; Armand, Anne-Sophie

    2016-01-01

    Mutations in the gene encoding the enzyme tafazzin, TAZ, cause Barth syndrome (BTHS). Individuals with this X-linked multisystem disorder present cardiomyopathy (CM) (often dilated), skeletal muscle weakness, neutropenia, growth retardation, and 3-methylglutaconic aciduria. Biopsies of the heart......, liver and skeletal muscle of patients have revealed mitochondrial malformations and dysfunctions. It is the purpose of this review to summarize recent results of studies on various animal or cell models of Barth syndrome, which have characterized biochemically the strong cellular defects associated...

  12. Pendred's syndrome

    International Nuclear Information System (INIS)

    Hashmi, M.I.; Cheema, I.A.; Qasim, G.

    2003-01-01

    This report describes Pendred's syndrome in three siblings of a consanguineous marriage, belonging to Rahimyar Khan. The children presented with deafmutism and goiters. The investigations included scintigram, perchlorate discharge test and audiometery. The perchlorate discharge was positive in index case. Bilateral sensorineural hearing defect was detected on Pure Tone Average (PTA) audiometry. Meticulous clinical and laboratory evaluation is mandatory for the detection of rare disorders like Pendred's syndrome. (author)

  13. Progressive retinal degeneration in a girl with Knobloch syndrome who presented with signs of ocular albinism.

    Science.gov (United States)

    Gradstein, Libe; Hansen, Ronald M; Cox, Gerald F; Altschwager, Pablo; Fulton, Anne B

    2017-04-01

    We report for the first time electroretinographic (ERG) evidence of progressive retinal abnormalities in a girl who presented in infancy with ocular features of albinism and gradually developed choroidal sclerosis and patchy retinal atrophy leading to a diagnosis of Knobloch syndrome (KS, OMIM 267750, COL18A1). At age 2 months, nystagmus and esotropia prompted ophthalmic evaluation. The appearance of choroidal sclerosis and atrophic retinal patches led to further evaluation at age 8 years. Genetics consultation was obtained in infancy and again at age 8 years as retinal findings evolved. Full field ERG responses in both scotopic and photopic conditions were recorded at both ages and compared to those in healthy control subjects. At age 2 months ERG response parameters were within normal limits for age and tyrosinase (TYR) gene sequencing revealed one novel mutation, p.S466F, and the temperature-sensitive polymorphism, p.R402Q, suggesting the diagnosis of oculocutaneous albinism type 1 (OCA1). At age 8 years, there was significant attenuation of both scotopic and photopic ERG responses. Genetic re-analysis led to the identification of a homozygous mutation, c.3213dupC, in the COL18A1 gene, thus confirming the diagnosis of Knobloch syndrome. Our patient with Knobloch syndrome developed abnormal ERG responses similar to those found in col18a1 knockout mice. Thus, we have documented progressive attenuation of the scotopic and photopic responses in KS.

  14. Wernicke-Korsakoff-syndrome: under-recognized and under-treated.

    Science.gov (United States)

    Isenberg-Grzeda, Elie; Kutner, Haley E; Nicolson, Stephen E

    2012-01-01

    Wernicke-Korsakoff syndrome (WKS) is a well described syndrome of neurological and cognitive problems that comprises both Wernicke's encephalopathy (WE) and Korsakoff syndrome (KS). WE is an acute neuropsychiatric disorder caused by thiamine deficiency. KS is a chronic consequence of thiamine deficiency with prominent impairment in memory formation. The authors review the literature on the pathophysiology, presentation, and treatment of WKS, focusing on the acute identification and treatment of WE. Most cases of WE are missed by clinicians, likely because patients do not present with the classic signs associated with the condition. Attaining high serum levels of thiamine during treatment may be important to restore cognitive function as quickly as possible, though the exact dosing and route needed for effective treatment is unknown. Data indicates that the administration of intravenous (IV) thiamine has little risk. In order to prevent this potentially devastating disease, physicians should have a high index of suspicion for WKS and dose thiamine accordingly. Copyright © 2012 The Academy of Psychosomatic Medicine. Published by Elsevier Inc. All rights reserved.

  15. INTEGRAL Galactic Bulge monitoring: transient activity from KS 1741-293, MXB 1730-335, and IGR J17498-2921

    Science.gov (United States)

    Chenevez, J.; Brandt, S.; Kuulkers, E.; Alfonso-Garzón, J.; Beckmann, V.; Bird, T.; Courvoisier, Th.; Del Santo, M.; Domingo, A.; Ebisawa, K.; Jonker, P.; Kretschmar, P.; Markwardt, C.; Oosterbroek, T.; Paizis, A.; Pottschmidt, K.; Sánchez-Fernández, C.; Wijnands, R.

    2011-09-01

    As part of its regular monitoring of the Galactic Bulge (see ATel #438) INTEGRAL observed this region of the sky on September 13, 2011, between UTC 9:14:50 and 12:56:26. Both the JEM-X and the IBIS/ISGRI instruments detect the transient neutron star low-mass X-ray binary KS 1741-293 at the following flux levels: JEM-X: 6 ±3 mCrab (3-10 keV) and 14 ±6 mCrab (10-25 keV) ISGRI: 11 ±2 mCrab (18-40 keV) and 13 ±2 mCrab (40-100 keV) We note that the activity of this source already started two weeks ago as has been reported by Linares et al.

  16. Measurement of the Branching Ratios of D+ and D+s Hadronic Decays to Four-Body Final States Containing a KS

    Science.gov (United States)

    Link, J. M.; Reyes, M.; Yager, P. M.; Anjos, J. C.; Bediaga, I.; Göbel, C.; Magnin, J.; Massafferi, A.; de Miranda, J. M.; Pepe, I. M.; Dos Reis, A. C.; Simão, F. R.; Carrillo, S.; Casimiro, E.; Sánchez-Hernández, A.; Uribe, C.; Vázquez, F.; Cinquini, L.; Cumalat, J. P.; O'Reilly, B.; Ramirez, J. E.; Vaandering, E. W.; Butler, J. N.; Cheung, H. W.; Gaines, I.; Garbincius, P. H.; Garren, L. A.; Gottschalk, E.; Kasper, P. H.; Kreymer, A. E.; Kutschke, R.; Bianco, S.; Fabbri, F. L.; Sarwar, S.; Zallo, A.; Cawlfield, C.; Kim, D. Y.; Rahimi, A.; Wiss, J.; Gardner, R.; Chung, Y. S.; Kang, J. S.; Ko, B. R.; Kwak, J. W.; Lee, K. B.; Park, H.; Alimonti, G.; Boschini, M.; Caccianiga, B.; D'Angelo, P.; Dicorato, M.; Dini, P.; Giammarchi, M.; Inzani, P.; Leveraro, F.; Malvezzi, S.; Menasce, D.; Mezzadri, M.; Milazzo, L.; Moroni, L.; Pedrini, D.; Pontoglio, C.; Prelz, F.; Rovere, M.; Sala, A.; Sala, S.; Davenport, T. F.; Agostino, L.; Arena, V.; Boca, G.; Bonomi, G.; Gianini, G.; Liguori, G.; Merlo, M.; Pantea, D.; Ratti, S. P.; Riccardi, C.; Segoni, I.; Viola, L.; Vitulo, P.; Hernandez, H.; Lopez, A. M.; Mendez, H.; Mendez, L.; Mirles, A.; Montiel, E.; Olaya, D.; Paris, A.; Quinones, J.; Rivera, C.; Xiong, W.; Zhang, Y.; Wilson, J. R.; Cho, K.; Handler, T.; Engh, D.; Hosack, M.; Johns, W. E.; Nehring, M.; Sheldon, P. D.; Stenson, K.; Webster, M.; Sheaff, M.

    2001-10-01

    We have studied hadronic four-body decays of D+ and D+s mesons with a KS in the final state using data recorded during the 1996-1997 fixed-target run of the Fermilab high energy photoproduction experiment FOCUS. We report a new branching ratio measurement of Γ(D+-->KSK- π+π+)/Γ(D+-->KSπ+π+π-) = 0.0768+/-0.0041+/-0.0032. We make the first observation of three new decay modes with branching ratios Γ(D+-->KSK+π+π- )/Γ(D+-->KSπ+π+π-) = 0.0562+/-0.0039+/-0.0040, Γ(D+-->KSK+K- π+)/Γ(D+-->KSπ+π+π-) = 0.0077+/-0.0015+/-0.0009, and Γ(D+s-->KSK+π+π- )/Γ(D+s-->KSK- π+π+) = 0.586+/-0.052+/-0.043, where in each case the first error is statistical and the second error is systematic.

  17. Beam-Energy Dependence of Directed Flow of Λ, Λ[over ¯], K^{±}, K_{s}^{0}, and ϕ in Au+Au Collisions.

    Science.gov (United States)

    Adamczyk, L; Adams, J R; Adkins, J K; Agakishiev, G; Aggarwal, M M; Ahammed, Z; Ajitanand, N N; Alekseev, I; Anderson, D M; Aoyama, R; Aparin, A; Arkhipkin, D; Aschenauer, E C; Ashraf, M U; Attri, A; Averichev, G S; Bai, X; Bairathi, V; Barish, K; Behera, A; Bellwied, R; Bhasin, A; Bhati, A K; Bhattarai, P; Bielcik, J; Bielcikova, J; Bland, L C; Bordyuzhin, I G; Bouchet, J; Brandenburg, J D; Brandin, A V; Brown, D; Bunzarov, I; Butterworth, J; Caines, H; Calderón de la Barca Sánchez, M; Campbell, J M; Cebra, D; Chakaberia, I; Chaloupka, P; Chang, Z; Chankova-Bunzarova, N; Chatterjee, A; Chattopadhyay, S; Chen, X; Chen, J H; Chen, X; Cheng, J; Cherney, M; Christie, W; Contin, G; Crawford, H J; Das, S; De Silva, L C; Dedovich, T G; Deng, J; Derevschikov, A A; Didenko, L; Dilks, C; Dong, X; Drachenberg, J L; Draper, J E; Dunkelberger, L E; Dunlop, J C; Efimov, L G; Elsey, N; Engelage, J; Eppley, G; Esha, R; Esumi, S; Evdokimov, O; Ewigleben, J; Eyser, O; Fatemi, R; Fazio, S; Federic, P; Federicova, P; Fedorisin, J; Feng, Z; Filip, P; Finch, E; Fisyak, Y; Flores, C E; Fujita, J; Fulek, L; Gagliardi, C A; Garand, D; Geurts, F; Gibson, A; Girard, M; Grosnick, D; Gunarathne, D S; Guo, Y; Gupta, A; Gupta, S; Guryn, W; Hamad, A I; Hamed, A; Harlenderova, A; Harris, J W; He, L; Heppelmann, S; Heppelmann, S; Hirsch, A; Horvat, S; Huang, X; Huang, B; Huang, T; Huang, H Z; Humanic, T J; Huo, P; Igo, G; Jacobs, W W; Jentsch, A; Jia, J; Jiang, K; Jowzaee, S; Judd, E G; Kabana, S; Kalinkin, D; Kang, K; Kapukchyan, D; Kauder, K; Ke, H W; Keane, D; Kechechyan, A; Khan, Z; Kikoła, D P; Kim, C; Kisel, I; Kisiel, A; Kochenda, L; Kocmanek, M; Kollegger, T; Kosarzewski, L K; Kraishan, A F; Krauth, L; Kravtsov, P; Krueger, K; Kulathunga, N; Kumar, L; Kvapil, J; Kwasizur, J H; Lacey, R; Landgraf, J M; Landry, K D; Lauret, J; Lebedev, A; Lednicky, R; Lee, J H; Li, C; Li, X; Li, Y; Li, W; Lidrych, J; Lin, T; Lisa, M A; Liu, P; Liu, H; Liu, Y; Liu, F; Ljubicic, T; Llope, W J; Lomnitz, M; Longacre, R S; Luo, S; Luo, X; Ma, Y G; Ma, L; Ma, R; Ma, G L; Magdy, N; Majka, R; Mallick, D; Margetis, S; Markert, C; Matis, H S; Meehan, K; Mei, J C; Miller, Z W; Minaev, N G; Mioduszewski, S; Mishra, D; Mizuno, S; Mohanty, B; Mondal, M M; Morozov, D A; Mustafa, M K; Nasim, Md; Nayak, T K; Nelson, J M; Nie, M; Nigmatkulov, G; Niida, T; Nogach, L V; Nonaka, T; Nurushev, S B; Odyniec, G; Ogawa, A; Oh, K; Okorokov, V A; Olvitt, D; Page, B S; Pak, R; Pandit, Y; Panebratsev, Y; Pawlik, B; Pei, H; Perkins, C; Pile, P; Pluta, J; Poniatowska, K; Porter, J; Posik, M; Pruthi, N K; Przybycien, M; Putschke, J; Qiu, H; Quintero, A; Ramachandran, S; Ray, R L; Reed, R; Rehbein, M J; Ritter, H G; Roberts, J B; Rogachevskiy, O V; Romero, J L; Roth, J D; Ruan, L; Rusnak, J; Rusnakova, O; Sahoo, N R; Sahu, P K; Salur, S; Sandweiss, J; Saur, M; Schambach, J; Schmah, A M; Schmidke, W B; Schmitz, N; Schweid, B R; Seger, J; Sergeeva, M; Seto, R; Seyboth, P; Shah, N; Shahaliev, E; Shanmuganathan, P V; Shao, M; Sharma, A; Sharma, M K; Shen, W Q; Shi, S S; Shi, Z; Shou, Q Y; Sichtermann, E P; Sikora, R; Simko, M; Singha, S; Skoby, M J; Smirnov, N; Smirnov, D; Solyst, W; Song, L; Sorensen, P; Spinka, H M; Srivastava, B; Stanislaus, T D S; Strikhanov, M; Stringfellow, B; Suaide, A A P; Sugiura, T; Sumbera, M; Summa, B; Sun, Y; Sun, X M; Sun, X; Surrow, B; Svirida, D N; Tang, Z; Tang, A H; Taranenko, A; Tarnowsky, T; Tawfik, A; Thäder, J; Thomas, J H; Timmins, A R; Tlusty, D; Todoroki, T; Tokarev, M; Trentalange, S; Tribble, R E; Tribedy, P; Tripathy, S K; Trzeciak, B A; Tsai, O D; Ullrich, T; Underwood, D G; Upsal, I; Van Buren, G; van Nieuwenhuizen, G; Vasiliev, A N; Videbæk, F; Vokal, S; Voloshin, S A; Vossen, A; Wang, G; Wang, Y; Wang, F; Wang, Y; Webb, J C; Webb, G; Wen, L; Westfall, G D; Wieman, H; Wissink, S W; Witt, R; Wu, Y; Xiao, Z G; Xie, G; Xie, W; Xu, J; Xu, Z; Xu, Q H; Xu, Y F; Xu, N; Yang, S; Yang, Y; Yang, C; Yang, Q; Ye, Z; Ye, Z; Yi, L; Yip, K; Yoo, I-K; Yu, N; Zbroszczyk, H; Zha, W; Zhang, Z; Zhang, J B; Zhang, J; Zhang, S; Zhang, Y; Zhang, X P; Zhang, J; Zhang, S; Zhao, J; Zhong, C; Zhou, C; Zhou, L; Zhu, X; Zhu, Z; Zyzak, M

    2018-02-09

    Rapidity-odd directed-flow measurements at midrapidity are presented for Λ, Λ[over ¯], K^{±}, K_{s}^{0}, and ϕ at sqrt[s_{NN}]=7.7, 11.5, 14.5, 19.6, 27, 39, 62.4, and 200 GeV in Au+Au collisions recorded by the Solenoidal Tracker detector at the Relativistic Heavy Ion Collider. These measurements greatly expand the scope of data available to constrain models with differing prescriptions for the equation of state of quantum chromodynamics. Results show good sensitivity for testing a picture where flow is assumed to be imposed before hadron formation and the observed particles are assumed to form via coalescence of constituent quarks. The pattern of departure from a coalescence-inspired sum rule can be a valuable new tool for probing the collision dynamics.

  18. Komunikační strategie ve vazbě na CSR ve firmě THIMM Obaly, k.s.

    OpenAIRE

    Deutschová, Veronika

    2014-01-01

    Diplomová práce zachycuje koncepci CSR v podnikové strategii společnosti THIMM Obaly, k.s. včetně uvedení teoretických východisek. Analyzuje a hodnotí stávající úroveň komunikace o aktivitách CSR. Výstup práce představuje návrh komunikační strategie, který umožní efektivní naplňování programu CSR pro zvolenou společnost. This diploma thesis gives a comprehensive survey of the CSR concept in corporate strategy in the limited partnership company THIMM Obaly. Theoretical bases are included. T...

  19. Measurement of the time-integrated C P asymmetry in D"0→K_S"0K_S"0 decays

    International Nuclear Information System (INIS)

    Aaij, R.; Adeva, B.; Adinolfi, M.; Affolder, A.

    2015-01-01

    The time-integrated C P asymmetry in the decay D"0→K_S"0K_S"0 is measured using 3 fb"−"1 of proton-proton collision data collected by the LHCb experiment at centre-of-mass energies of 7 and 8 TeV. The flavour of the D"0 meson is determined by use of the decay D"∗"+→D"0π"+ and its charge conjugate mode. The result is A_C _P=−0.029±0.052±0.022, where the first uncertainty is statistical and the second systematic. The result is consistent with Standard Model expectations and improves the uncertainty with respect to the only previous measurement of this quantity by more than a factor of three.

  20. Ks0 and Lambda production in pp interactions at √s = 0.9 and 7 TeV measured with the ATLAS detector at the LHC

    International Nuclear Information System (INIS)

    Aad, G.; Abbott, B.; Abdallah, J.; Abdelalim, A.A.; Abdesselam, A.; Abdinov, O.; Abi, B.; Abolins, M.; Abramowicz, H.; Abreu, H.; Acerbi, E.; Acharya, B.S.; Adams, D.L.; Addy, T.N.; Adelman, J.; Aderholz, M.; Adomeit, S.; Adragna, P.; Adye, T.; Aefsky, S.; Aguilar-Saavedra, J.A.

    2012-01-01

    The production of K S 0 and Λ hadrons is studied in pp collision data at √s = 0.9 and 7 TeV collected with the ATLAS detector at the LHC using a minimum-bias trigger. The observed distributions of transverse momentum, rapidity, and multiplicity are corrected to hadron level in a model-independent way within well-defined phase-space regions. The distribution of the production ratio of Λ - to Λ baryons is also measured. The results are compared with various Monte Carlo simulation models. Although most of these models agree with data to within 15% in the KS0 distributions, substantial disagreements are found in the Λ distributions of transverse momentum.

  1. [Poland's syndrome].

    Science.gov (United States)

    Slezak, R; Sasiadek, M

    2000-08-01

    Poland's syndrome consists of the variable clinical features, but always includes unilateral aplasia of the chest wall muscles and ipsilateral anomalies of upper extremity. The incidence of Poland's syndrome, reported by different authors ranges from 1:10,000 to 1:100,000 and is observed more frequently in males than in females with the right side of the body affected more often than the left. The etiology of this syndrome is still discussed. However most of described cases were sporadic, rare familial incidence of Poland's syndrome were also presented. Therefore different etiologic factors of the Poland's syndrome are taken into account: genetic, vascular compromise during early stages of embriogenesis but also teratogenic effect of environmental xenobiotics (e.g. cigarette smoking by pregnant women). The authors present also the case of 20-years old man with inherited bilateral syndactyly with the right side aplasia of major pectoralis muscle and face asymmetry. The familial history was negative in respect to the features, associated with Poland's syndrome.

  2. Preliminary Analysis of a Steam Line Break Accident with the MARS-KS code for the SMART Design with Passive Safety Systems

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Doohyuk; Ko, Yungjoo; Suh, Jaeseung [Hannam Univ., Daejeon (Korea, Republic of); Bae, Hwang; Ryu, Sunguk; Yi, Sungjae; Park, Hyunsik [Korea Atomic Energy Research Institute, Daejeon (Korea, Republic of)

    2014-05-15

    SMART has been developed by KAERI, and SMART-Standard Design Approval (SDA) was recently granted in 2012. A SMART design with Passive Safety System (PSS) features (called SMART-PSS) is being developed and added to the standard design of SMART by KAERI to improve its safety system. Active safety systems such as safety injection pumps will be replaced by a passive safety system, which is actuated only by the gravity force caused by the height difference. All tanks for the passive safety systems are higher than the injection nozzle, which is located around the reactor coolant pumps (RCPs). In this study, a preliminary analysis of the main steam line break accident (MSLB) was performed using the MARS-KS code to understand the general behavior of the SMART-PSS design and to prepare its validation test with the SMART-ITL (FESTA) facility. An anticipated accident for the main steam line break (MSLB) was performed using the MARS-KS code to understand the thermal-hydraulic behaviors of the SMART-PSS design. The preliminary analysis provides good insight into the passive safety system design features of the SMART-PSS and the thermal-hydraulic characteristics of the SMART design. The analysis results of the MSLB showed that the core water collapsed level inside the core support barrel was maintained high over the active core top level during the transient period. Therefore, the SMART-PSS design has satisfied the requirements to maintain the plant at a safe shutdown condition during 72 hours without AC power or operator action after an anticipated accident.

  3. What is Metabolic Syndrome?

    Science.gov (United States)

    ... Intramural Research Home / Metabolic Syndrome Metabolic Syndrome Also known as What Is Metabolic syndrome ... metabolic risk factors to be diagnosed with metabolic syndrome. Metabolic Risk Factors A Large Waistline Having a large ...

  4. Loeys-Dietz Syndrome

    Science.gov (United States)

    ... to the signs and symptoms of Loeys-Dietz syndrome. Marfan syndrome is different from Loeys-Dietz syndrome in that the gene mutation which causes Marfan syndrome is in fibrillin-1 (FBN-1), a protein ...

  5. Milk-alkali syndrome

    Science.gov (United States)

    Calcium-alkali syndrome; Cope syndrome; Burnett syndrome; Hypercalcemia; Calcium metabolism disorder ... Milk-alkali syndrome is almost always caused by taking too many calcium supplements, usually in the form of calcium carbonate. Calcium ...

  6. Exogenous Cushing syndrome

    Science.gov (United States)

    Cushing syndrome - corticosteroid induced; Corticosteroid-induced Cushing syndrome; Iatrogenic Cushing syndrome ... Cushing syndrome is a disorder that occurs when your body has a higher than normal level of the hormone ...

  7. Turner Syndrome: Other FAQs

    Science.gov (United States)

    ... Other FAQs Share Facebook Twitter Pinterest Email Print Turner Syndrome: Other FAQs Basic information for topics, such as " ... been diagnosed with Turner syndrome. Now what? Is Turner syndrome inherited? Turner syndrome is usually not inherited, but ...

  8. Pfeiffer syndrome

    Directory of Open Access Journals (Sweden)

    Fryns Jean-Pierre

    2006-06-01

    Full Text Available Abstract Pfeiffer syndrome is a rare autosomal dominantly inherited disorder that associates craniosynostosis, broad and deviated thumbs and big toes, and partial syndactyly on hands and feet. Hydrocephaly may be found occasionally, along with severe ocular proptosis, ankylosed elbows, abnormal viscera, and slow development. Based on the severity of the phenotype, Pfeiffer syndrome is divided into three clinical subtypes. Type 1 "classic" Pfeiffer syndrome involves individuals with mild manifestations including brachycephaly, midface hypoplasia and finger and toe abnormalities; it is associated with normal intelligence and generally good outcome. Type 2 consists of cloverleaf skull, extreme proptosis, finger and toe abnormalities, elbow ankylosis or synostosis, developmental delay and neurological complications. Type 3 is similar to type 2 but without a cloverleaf skull. Clinical overlap between the three types may occur. Pfeiffer syndrome affects about 1 in 100,000 individuals. The disorder can be caused by mutations in the fibroblast growth factor receptor genes FGFR-1 or FGFR-2. Pfeiffer syndrome can be diagnosed prenatally by sonography showing craniosynostosis, hypertelorism with proptosis, and broad thumb, or molecularly if it concerns a recurrence and the causative mutation was found. Molecular genetic testing is important to confirm the diagnosis. Management includes multiple-staged surgery of craniosynostosis. Midfacial surgery is performed to reduce the exophthalmos and the midfacial hypoplasia.

  9. Nevoid basal cell carcinoma syndrome

    Science.gov (United States)

    NBCC syndrome; Gorlin-Goltz syndrome; Basal cell nevus syndrome; BCNS; Basal cell cancer - nevoid basal cell carcinoma syndrome ... Nevoid basal cell carcinoma nevus syndrome is a rare genetic ... syndrome is known as PTCH ("patched"). The gene is passed down ...

  10. Getting the Most from Working with Higher Education: A Review of Methods Used within a Participatory Design Activity Involving KS3 Special School Pupils and Undergraduate and Post-Graduate Industrial Design Students

    Science.gov (United States)

    Torrens, George Edward; Newton, Helen

    2013-01-01

    This paper provides education-based researchers and practitioners with the preferred research and design methods used by Higher Education Institute (HEI) students and Key Stage 3 (KS3) pupils applied within a participatory approach to a design activity. The outcomes were that both pupils and students found informal (unstructured) interview to be…

  11. Nutcracker syndrome

    International Nuclear Information System (INIS)

    Jolley, Ingrid

    2014-01-01

    Purpose: The purpose of this case study is to highlight the symptoms of the Nutcracker Syndrome (NCS), the methods of clinical investigations and the importance of differential diagnosis. Introduction: The NCS refers to left renal vein entrapment caused by abnormal branching patterns of the superior mesenteric artery from the aorta. 1,2 Clinical case presentation: A 27 years old female presented to the emergency department with complaints of abdominal discomfort, bloating, loose bowel motions and irregular micro-haematuria. The radiologist's report indicated the findings from computed tomography examination to be consistent with anterior NCS. Discussion: In most of the NCS cases the clinical symptoms are non-specific. 3 The syndrome is caused by a vascular disorder, but its clinical manifestation can relate to a wide range of abdominal, urological, endovascular or gynaecological pathologies. 4 Conclusion: Nutcracker Syndrome is a relatively rare disease and underdiagnosed may lead to left renal vein thrombosis

  12. Compartment syndromes

    Science.gov (United States)

    Mubarak, S. J.; Pedowitz, R. A.; Hargens, A. R.

    1989-01-01

    The compartment syndrome is defined as a condition in which high pressure within a closed fascial space (muscle compartment) reduces capillary blood perfusion below the level necessary for tissue viability'. This condition occurs in acute and chronic (exertional) forms, and may be secondary to a variety of causes. The end-result of an extended period of elevated intramuscular pressure may be the development of irreversible tissue injury and Volkmann's contracture. The goal of treatment of the compartment syndrome is the reduction of intracompartmental pressure thus facilitating reperfusion of ischaemic tissue and this goal may be achieved by decompressive fasciotomy. Controversy exists regarding the critical pressure-time thresholds for surgical decompression and the optimal diagnostic methods of measuring intracompartmental pressures. This paper will update and review some current knowledge regarding the pathophysiology, aetiology, diagnosis, and treatment of the acute compartment syndrome.

  13. Usher Syndrome

    Directory of Open Access Journals (Sweden)

    Ana Fakin

    2012-06-01

    Full Text Available Usher syndrome is an autosomal recessive disease with prevalence of 3–6/100.000 and is the most common syndrome that affects vision and hearing. Three subtypes are distinguished on the basis of different degree of hearing loss. All patients develop retinitis pigmentosa with night vision difficulties and constriction of visual field, and ultimately a decline in visual acuity and color vision. Future holds promise for gene therapy. We present a patient with typical clinical picture of Usher syndrome, who started noticing night vision problems at age 13. At age 25 he was operated on for posterior cortical cataracts. At age 34 he has only 5–10° of visual field remaining with 1.0 visual acuity in both eyes. Fundus autofluorescence imaging revealed a typical hyperautofluorescent ring on the border between normal and affected retina.

  14. Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Sevil Ikinci

    2010-10-01

    Full Text Available Metabolic Syndrome is a combination of risk factors including common etiopathogenesis. These risk factors play different roles in occurence of atherosclerotic diseases, type 2 diabetes, and cancers. Although a compromise can not be achieved on differential diagnosis for MS, the existence of any three criterias enable to diagnose MS. These are abdominal obesity, dislipidemia (hypertrigliceridemia, hypercholesterolemia, and reduced high density lipoprotein hypertension, and elevated fasting blood glucose. According to the results of Metabolic Syndrome Research (METSAR, the overall prevalence of MS in Turkey is 34%; in females 40%, and in males it is 28%. As a result of “Western” diet, and increased frequency of obesity, MS is observed in children and in adolescents both in the world and in Turkey. Resulting in chronic diseases, it is thought that the syndrome can be prevented by healthy lifestyle behaviours. [TAF Prev Med Bull 2010; 9(5.000: 535-540

  15. Eagle's Syndrome

    Science.gov (United States)

    Pinheiro, Thaís Gonçalves; Soares, Vítor Yamashiro Rocha; Ferreira, Denise Bastos Lage; Raymundo, Igor Teixeira; Nascimento, Luiz Augusto; Oliveira, Carlos Augusto Costa Pires de

    2013-01-01

    Summary Introduction: Eagle's syndrome is characterized by cervicopharyngeal signs and symptoms associated with elongation of the styloid apophysis. This elongation may occur through ossification of the stylohyoid ligament, or through growth of the apophysis due to osteogenesis triggered by a factor such as trauma. Elongation of the styloid apophysis may give rise to intense facial pain, headache, dysphagia, otalgia, buzzing sensations, and trismus. Precise diagnosis of the syndrome is difficult, and it is generally confounded by other manifestations of cervicopharyngeal pain. Objective: To describe a case of Eagle's syndrome. Case Report: A 53-year-old man reported lateral pain in his neck that had been present for 30 years. Computed tomography (CT) of the neck showed elongation and ossification of the styloid processes of the temporal bone, which was compatible with Eagle's syndrome. Surgery was performed for bilateral resection of the stylohyoid ligament by using a transoral and endoscopic access route. The patient continued to present pain laterally in the neck, predominantly on his left side. CT was performed again, which showed elongation of the styloid processes. The patient then underwent lateral cervicotomy with resection of the stylohyoid process, which partially resolved his painful condition. Final Comments: Patients with Eagle's syndrome generally have a history of chronic pain. Appropriate knowledge of this disease is necessary for adequate treatment to be provided. The importance of diagnosing this uncommon and often unsuspected disease should be emphasized, given that correct clinical-surgical treatment is frequently delayed. The diagnosis of Eagle's syndrome is clinical and radiographic, and the definitive treatment in cases of difficult-to-control pain is surgical. PMID:25992033

  16. Eagle's Syndrome

    Directory of Open Access Journals (Sweden)

    Pinheiro, Thaís Gonçalves

    2014-01-01

    Full Text Available Introduction: Eagle's syndrome is characterized by cervicopharyngeal signs and symptoms associated with elongation of the styloid apophysis. This elongation may occur through ossification of the stylohyoid ligament, or through growth of the apophysis due to osteogenesis triggered by a factor such as trauma. Elongation of the styloid apophysis may give rise to intense facial pain, headache, dysphagia, otalgia, buzzing sensations, and trismus. Precise diagnosis of the syndrome is difficult, and it is generally confounded by other manifestations of cervicopharyngeal pain. Objective: To describe a case of Eagle's syndrome. Case Report: A 53-year-old man reported lateral pain in his neck that had been present for 30 years. Computed tomography (CT of the neck showed elongation and ossification of the styloid processes of the temporal bone, which was compatible with Eagle's syndrome. Surgery was performed for bilateral resection of the stylohyoid ligament by using a transoral and endoscopic access route. The patient continued to present pain laterally in the neck, predominantly on his left side. CT was performed again, which showed elongation of the styloid processes. The patient then underwent lateral cervicotomy with resection of the stylohyoid process, which partially resolved his painful condition. Final Comments: Patients with Eagle's syndrome generally have a history of chronic pain. Appropriate knowledge of this disease is necessary for adequate treatment to be provided. The importance of diagnosing this uncommon and often unsuspected disease should be emphasized, given that correct clinical-surgical treatment is frequently delayed. The diagnosis of Eagle's syndrome is clinical and radiographic, and the definitive treatment in cases of difficult-to-control pain is surgical.

  17. Peering through the veil: near-infrared photometry and extinction for the Galactic nuclear star cluster. Accurate near infrared H, Ks, and L' photometry and the near-infrared extinction-law toward the central parsec of the Galaxy

    Science.gov (United States)

    Schödel, R.; Najarro, F.; Muzic, K.; Eckart, A.

    2010-02-01

    Context. The nuclear star cluster of the Galaxy is an important template for understanding its extragalactic counterparts, which can currently not be resolved into individual stars. Important drawbacks of observations of the Galactic center are, however, the presence of strong and spatially highly variable interstellar extinction and extreme crowding of the sources, which makes the use of adaptive optics techniques necessary. Both points pose serious obstacles to precise photometry that is needed for analyzing the stellar population. Aims: The aims of this work are to provide accurate photometry in multiple near-infrared broadband filters, to determine the power-law index of the extinction-law toward the central parsec of the Galaxy, to provide measurements of the absolute extinction toward the Galactic center, and finally to measure the spatial variability of extinction on arcsecond scales. Methods: We use observations of the central parsec of the Milky Way that were obtained with the near-infrared camera and adaptive optics system NAOS/CONICA at the ESO VLT unit telescope 4. The photometric method takes into account anisoplanatic effects and limits the corresponding systematic uncertainties to ≲2%. Absolute values for the extinction in the H, Ks, and L'-bands as well as of the power-law indices of the H to Ks and Ks to L' extinction-laws are measured based on the well-known properties of red clump stars. Extinction maps are derived based on H-Ks and Ks-L' colors. Results: We present Ks-band photometry for ~7700 stars, and additionally photometry for stars detected in the H and/or L'-bands. From a number of recently published values we compute a mean distance of the Galactic center of R0=8.03±0.15 kpc, which has an uncertainty of just 2%. Based on this R0 and on the RC method, we derive absolute mean extinction values toward the central parsec of the Galaxy of AH=4.48±0.13 mag, AKs=2.54±0.12 mag, and AL'=1.27±0.18 mag. We estimate values of the power

  18. Rapunzel syndrome

    International Nuclear Information System (INIS)

    Al-Wadan, Ali H.; Al-Saai, Azan S.; Abdoulgafour, Mohamed; Al-Absi, Mohamed

    2006-01-01

    An 18-year-old single female patient, presented with non specific gastrointestinal symptoms of anorexia, abdominal pain, and change in bowel habit. Clinically she was anemic, cachectic, and depressed. Abdominal examination revealed mobile epigastric mass. The scalp alopecia and endoscopy coupled by computed tomography scan, confirmed the diagnoses of trichobezoar, but it was not diagnosed as Rapunzel syndrome except after laparotomy, gastrotomy, and enterotomy. There are less than 16 cases of Rapunzel syndrome described worldwide, and this is the first case to be described in the middle east. (author)

  19. Waardenburg syndrome

    Directory of Open Access Journals (Sweden)

    Tagra Sunita

    2006-01-01

    Full Text Available Waardenburg syndrome is a rare inherited and genetically heterogenous disorder of neural crest cell development. Four distinct subtypes showing marked interfamilial and intrafamilial variability have been described. We report a girl showing constellation of congenital hearing impairment with 110 dB and 105 dB loss in right and left ear respectively, hypoplastic blue iridis, white forelock, dystopia canthorum and broad nasal root. Other affected relatives of the family, with variable features of the syndrome, have been depicted in the pedigree.

  20. Olmsted syndrome

    Directory of Open Access Journals (Sweden)

    Kumar Pramod

    2008-01-01

    Full Text Available Olmsted syndrome is a rare disorder characterized by the combination of periorificial, keratotic plaques and bilateral palmoplantar keratoderma. New associated features are being reported. Olmsted syndrome is particularly rare in a female patient, and we report such a case in a six year-old Indian girl, who presented with keratoderma of her soles since birth and on her palms since the age of two years along with perioral and perinasal hyperkeratosis. She had sparse, light brown, thin hair. Although the psychomotor development of the child was normal until 18 months of age, the keratoderma plaques had restricted the child′s mobility after that stage.

  1. Eagle syndrome

    International Nuclear Information System (INIS)

    Raina, Deepika; Gothi, Rajesh; Rajan, Sriram

    2009-01-01

    Eagle syndrome occurs due to elongation of the styloid process or calcification of the stylohyoid ligament, which then may produce a pain sensation due the pressure exerted on various structures in the head and neck. When suspected, imaging helps in identifying the abnormally elongated styloid process or the calcified ligament. In recent years, three-dimensional CT (3DCT) has proved to be valuable in these cases. We report the case of a 62-year-old man with this syndrome in whom imaging with 3DCT conclusively established the diagnosis

  2. Turner Syndrome

    Directory of Open Access Journals (Sweden)

    Ramachandran Sudarshan

    2012-08-01

    Full Text Available Turner syndrome is a genetic disorder that affects mostly females. Affected females have characteristic features such as short stature, premature ovarian failure, and several other features. Oral manifestations of this condition are not much discussed in the literature. But reported literature includes teeth, palate, periodontal and salivary changes. So the aim of this review is to illustrate the general manifestations, and especially the oral manifestations of Turner syndrome and evaluate their possible management. [Archives Medical Review Journal 2012; 21(4.000: 246-252

  3. Fenton's syndrome

    International Nuclear Information System (INIS)

    Rimondi, E.; Albasini, V.

    1989-01-01

    The authors report two recent cases of Fenton's syndrome, a very rare carpal fracture-dislocation. After some anatomophysiopathological considerations and a review of the literature, a wider nosographic frame is proposed in which the entity of the dislocation of the head of capitate bone is not essential. According to both the literature and personal findings, the authors remark that this syndrome is always found in the presence of two morphological variants of the distal radioulnar joint. Finally, the authors stress the importance of a corect diagnosis of this lesion to avoid unnecessary attempts of reduction

  4. Reiter's Syndrome.

    Science.gov (United States)

    Savant, S S; Fernandez, J C; Dhurandhar, M W; Fernandez, R J

    1979-01-01

    A case of Reiter's syndrome occurring in a young mate aged 20 years having extensive skin lesions of keratoderina blenoffhagica is presented along with a review of literature. Although urethritis was absent, other clinical and histopathological features of the cutaneous lesions led us to the diagnosis. The-possible relationship of postural psoriasis to Reiter's syndrome is discussed. Failure of the patient to respond satisfactorily to steroids, antibiotics etc, prompted the use of rnethotrexate in the case. The result was dramatic, as the patient completely recovered within ten days of starting treatment.

  5. Larsen syndrome

    Directory of Open Access Journals (Sweden)

    Mohammed Mahbubul Islam

    2016-08-01

    Full Text Available Larsen syndrome is a rare inherited disorder characterized by congenital dislocation of multiple joints along with other anomalies of heart, face, hands and bones. Larsen syndrome was first described in 1950 by Larsen, Schottstaedt and Bost. In the present report, we describe a 10 year old girl who presented with mid facial hypoplasia with depressed nasal bridge, high arched palate, bilateral talipes equinovarus and high arched feet. On examination, she had short stature (HAZ -3.5 SD with hyperextension of knee joint, fixed flexion of elbow joint. Awareness of this condition and associated complications may help in management and follow up of these patients. 

  6. Joubert syndrome

    International Nuclear Information System (INIS)

    Villanua, J.A.; Lopez, J.M.; Recondo, J.A.; Garcia, J.M.; Gaztanaga, R.

    1998-01-01

    Joubert syndrome is a rare malformation of the posterior fossa, mainly affecting the cerebellar vermis, which generally appears as a dysplastic lesion. Other structures of the cervico medullary junction may be involved, with accompanying brainstem hypoplasia according to neuroimaging studies. The diagnosis is usually reached during, childhood, based on a constellation of changes in the child's neurological development that are supported by the results of imaging studied. Respiratory problems are the most common signs in newborns,leading to the suspicion of the presence of this syndrome. (Author) 11 refs

  7. Lemierre's syndrome.

    LENUS (Irish Health Repository)

    O'Dwyer, D N

    2012-02-01

    Lemierre\\'s syndrome is a rare disease that results in an oropharyngeal infection, which precipitates an internal jugular vein thrombosis and metastatic infection. Fusobacterium necrophorum is an anaerobic Gram-negative bacillus and has been identified as the causative agent. We describe the case of a young girl whose presentation and diagnosis were confounded by a history of valvular heart disease. Infection of heart valves can produce many of the signs and symptoms associated with Lemierre\\'s syndrome. We describe the diagnosis, investigation and optimal management of this rare disorder.

  8. Meigs' Syndrome

    International Nuclear Information System (INIS)

    Baloch, S.; Khaskheli, M.; Farooq, S.

    2006-01-01

    Meigs' syndrome is a rare clinical condition commonly considered to be associated with malignant ovarian tumour. A case of unmarried female is presented who came with a slowly increasing abdominal mass. Clinical and ultrasonic investigations revealed a mobile, solid right adenexal tumour in the lower abdomen, along with ascites and pleural effusion of the right lung. The level of CA 125 was also raised. Diagnosis of Meigs' syndrome was confirmed after surgical intervention. The tumour was successfully removed and pleural effusion disappeared 15 days after the intervention. Cytomorphologic study of both the tumour and ascitic fluid was negative for malignancy. (author)

  9. [Elsberg syndrome].

    Science.gov (United States)

    Nielsen, Kristine Esbjerg; Knudsen, Troels Bygum

    2013-12-16

    A syndrome involving acute urinary retention in combination with sacral radiculitis and cerebrospinal fluid pleocytosis was first described by the American neurosurgeon Charles Elsberg in 1931. In many instances the aetiology is herpes simplex virus type 2 (HSV-2) reactivation from sensory neurons. In this case report we present a 34-year-old pregnant woman with previous undiagnosed sensory lumbosacral symptoms. She was hospitalized with HSV-2 meningitis and lumbosacral radiculitis but no genital rash. A week after the onset of symptoms she developed acute urinary retention, thus indicating Elsberg syndrome.

  10. Neuropsychiatric Symptoms in People With Korsakoff Syndrome and Other Alcohol-Related Cognitive Disorders Living in Specialized Long-Term Care Facilities: Prevalence, Severity, and Associated Caregiver Distress.

    Science.gov (United States)

    Gerridzen, Ineke J; Hertogh, Cees M P M; Depla, Marja F; Veenhuizen, Ruth B; Verschuur, Els M L; Joling, Karlijn J

    2018-03-01

    Caring for people with Korsakoff syndrome (KS) residing in specialized long-term care facilities (LTCFs) can be distressing because of challenging neuropsychiatric symptoms (NPS). However, good-quality studies on NPS in this under-researched population are lacking. This study examined the prevalence and severity of NPS in people with KS living in specialized LTCFs and the associated caregiver distress. Cross-sectional, observational study. Data were obtained using structured interviews with care staff, elderly care physicians, and residents. Nine specialized LTCFs in the Netherlands. KS residents admitted for at least 3 months. The prevalence and severity of NPS were measured with the Neuropsychiatric Inventory-Questionnaire (NPI-Q). The associated caregiver distress was assessed with the NPI Distress Scale (NPI-D) according to the nurse or nurse assistant. Almost all of the 281 residents (96.4%) showed at least 1 NPS and 45.8% showed 5 or more symptoms. Irritability/lability (68.3%), agitation/aggression (58.7%), and disinhibition (52.7%) were most prevalent. Although the mean level of severity for all NPS was relatively low, half of the residents (49.1%) had at least 1 severe NPS. Care staff experienced low levels of distress associated with NPS. NPS are highly prevalent in KS residents. Unexpectedly, these did not have any severe impact on residents and care staff. Acquiring more insight into the persistence and course of NPS, and its associations, among KS residents is important to better understand and reduce these symptoms and, ultimately, improve the quality of care for these residents. Copyright © 2017 AMDA – The Society for Post-Acute and Long-Term Care Medicine. Published by Elsevier Inc. All rights reserved.

  11. Marfan syndrome masked by Down syndrome?

    NARCIS (Netherlands)

    Vis, J.C.; Engelen, K. van; Timmermans, J.; Hamel, B.C.J.; Mulder, B.J.

    2009-01-01

    Down syndrome is the most common chromosomal abnormality. A simultaneous occurrence with Marfan syndrome is extremely rare. We present a case of a 28-year-old female with Down syndrome and a mutation in the fibrillin-1 gene. The patient showed strikingly few manifestations of Marfan syndrome.

  12. Lemierre's syndrome

    DEFF Research Database (Denmark)

    Johannesen, Katrine M; Bodtger, Uffe

    2016-01-01

    This is a systematic review of cases with Lemierre's syndrome (LS) in the past 5 years. LS is characterized by sepsis often evolving after a sore throat or tonsillitis and then complicated by various septic emboli and thrombosis of the internal jugular vein. Symptoms include sepsis, pain, and/or ...... LS in this day and age appears to be low, however the syndrome is difficult to recognize, and still requires the full attention of the clinician.......This is a systematic review of cases with Lemierre's syndrome (LS) in the past 5 years. LS is characterized by sepsis often evolving after a sore throat or tonsillitis and then complicated by various septic emboli and thrombosis of the internal jugular vein. Symptoms include sepsis, pain, and....../or swelling in the throat or neck, as well as respiratory symptoms. Laboratory findings show elevated infectious parameters and radiological findings show thrombosis of the internal jugular vein and emboli in the lungs or other organs. The syndrome is often associated with an infection with Fusobacterium...

  13. Sjogren syndrome

    NARCIS (Netherlands)

    Brito-Zeron, Pilar; Baldini, Chiara; Bootsma, Hendrika; Bowman, Simon J.; Jonsson, Roland; Mariette, Xavier; Sivils, Kathy; Theander, Elke; Tzioufas, Athanasios; Ramos-Casals, Manuel

    2016-01-01

    Sjogren syndrome (SjS) is a systemic autoimmune disease that primarily affects the exocrine glands (mainly the salivary and lacrimal glands) and results in the severe dryness of mucosal surfaces, principally in the mouth and eyes. This disease predominantly affects middle-aged women, but can also be

  14. Rett Syndrome

    Science.gov (United States)

    ... loss of interest in normal play Delayed speech development or loss of previously acquired speech abilities Problem behavior or marked mood swings Any clear loss of previously gained milestones in gross motor or fine motor skills Causes Rett syndrome is a rare genetic disorder. ...

  15. Nodding Syndrome

    Centers for Disease Control (CDC) Podcasts

    2013-12-19

    Dr. Scott Dowell, a CDC director, discusses the rare illness, nodding syndrome, in children in Africa.  Created: 12/19/2013 by National Center for Emerging and Zoonotic Infectious Diseases (NCEZID).   Date Released: 1/27/2014.

  16. Piriformis Syndrome

    Science.gov (United States)

    ... can usually resume their normal activities. In some cases, exercise regimens may need to be modified in order to reduce the likelihood of recurrence or worsening. Clinical Trials Throughout the U.S. ... Definition Piriformis syndrome is a rare neuromuscular disorder that ...

  17. Hellp syndrome

    International Nuclear Information System (INIS)

    Chaudhry, A.A.

    2002-01-01

    A 24 years old female presented with hypertension, haemolysis, elevated liver enzymes and thrombocytopenia in an unconscious state after undergoing an emergency caesarian section. A diagnosis of HELLP syndrome was made on the above findings. Patient made an uneventful recovery with conservative management. A brief review of the literature is included along with the case report. (author)

  18. Kartagener's Syndrome.

    Science.gov (United States)

    Dhar, D K; Ganguly, K C; Alam, S; Hossain, A; Sarker, U K; Das, B K; Haque, M J

    2009-01-01

    Kartagener's Syndrome or Immotile Cilia Syndrome, a variant of Primary Ciliary Dyskinesia (PCD), is a rare autosomal recessive genetic disorder caused by defect in the tiny hair like structure, the cilia lining the respiratory tract (upper and lower), sinuses, eustachian tubes, middle ear and fallopian tubes. Here electron microscopy shows abnormal arrangement of ciliary tubules and patients with Kartagener's syndrome has an absence of dynein arms at the base of the cilia. The inability of cilia to move results in inadequate clearance of bacteria from the air passages, resulting in an increased risk of infection and causing bronchiectasis. Another result of ciliary immobility is infertility. A 60 years old lady was diagnosed as a case of Kartagener's syndrome. She had history of chronic cough for 20 years, irregular fever for 20 years and occasional shortness of breath for 5 years. Relevant investigations revealed dextrocardia, situs inversus, bilateral maxillary sinusitis with non pneumatised frontal sinus and bronchiectasis. She was treated with low concentration oxygen inhalation, antibiotic, bronchodilator, chest physiotherapy including postural drainage, vitamins and other supportive treatment.

  19. Carraro syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Wendler, H.; Schwarz, R.

    1980-07-01

    The report concerns a girl aged 9 1/2 years who was deaf and dumb and had marked shortening of the calves with deformities of the feet and bilateral, congenital hypoplasia of the tibiae. This syndrome was first described by Carraro in 1931, but there have been no further reports since then.

  20. Rett Syndrome.

    Science.gov (United States)

    Culbert, Linda A.

    This pamphlet reviews the historical process involved in initially recognizing Rett Syndrome as a specific disorder in girls. Its etiology is unknown, but studies have considered factors as hyperammonemia, a two-step mutation, a fragile X chromosome, metabolic disorder, environmental causation, dopamine deficiency, and an inactive X chromosome.…

  1. Alagille Syndrome

    Science.gov (United States)

    ... 3] Kamath BM, Loomes KM, Piccoli DA. Medical management of Alagille syndrome. Journal of Pediatric Gastroenterology and Nutrition. 2010;50(6): ... 30 a.m. to 5 p.m. eastern time, M-F Follow Us NIH… Turning Discovery Into ... Disease Urologic Diseases Endocrine Diseases Diet & Nutrition ...

  2. Kounis syndrome

    African Journals Online (AJOL)

    neoplastic agents), exposure to radiological contrast media, poison ivy, bee stings, shellfish and coronary stents. In addition to coronary arterial involvement, Kounis syndrome com prises other arterial systems with similar physiologies, such as mesenteric and cerebral circulation resulting in ischaemia/infarction of the vital ...

  3. Proteus syndrome

    Directory of Open Access Journals (Sweden)

    Debi Basanti

    2005-01-01

    Full Text Available Proteus syndrome is a variable and complex disorder characterized by multifocal overgrowths affecting any tissue or structure of the body. We present a girl aged 3 years and 8 months with an epidermal nevus, port-wine stain, macrodactyly with gigantism of the feet, lymphohemagiomas and multiple lipomas.

  4. Crest syndrome

    International Nuclear Information System (INIS)

    Koch, B.; Roedl, W.

    1988-01-01

    If a patient has peri- and intra-articular calcinosis, as well as acro-osteolysis and esophageal hypomotility, and rheumatic symptoms, Crest syndrome should be considered as a manifestation of progressive systemic sclerosis. In connection with relevant symptoms on the skin and visceral involvement, radiological studies offer the possibility of classifying progressive systemic sclerosis more accurately. (orig.) [de

  5. Gitelman syndrome.

    NARCIS (Netherlands)

    Knoers, N.V.A.M.; Levtchenko, E.N.

    2008-01-01

    Gitelman syndrome (GS), also referred to as familial hypokalemia-hypomagnesemia, is characterized by hypokalemic metabolic alkalosis in combination with significant hypomagnesemia and low urinary calcium excretion. The prevalence is estimated at approximately 1:40,000 and accordingly, the prevalence

  6. Marfan Syndrome

    Science.gov (United States)

    ... can treat many of its symptoms. Thanks to new research and treatments, people with Marfan syndrome who are diagnosed early ... This helps doctors stay on top of any new problems. Doctors might also ... or kids with amblyopia or strabismus will probably need to wear glasses. ...

  7. Kartagener's Syndrome

    African Journals Online (AJOL)

    GB

    presenting with recurrent upper and lower respiratory tract infections, sinusitis or bronchiectasis. Inability to diagnose this condition may subject the patient to unnecessary and repeated hospital admissions, investigations and treatment failure. KEY WORDS: Kartagener's syndrome, primary cilliary dyskinesia, situs inversus, ...

  8. Clinical Presentation of Klinefelter's Syndrome: Differences According to Age

    Directory of Open Access Journals (Sweden)

    Néstor Pacenza

    2012-01-01

    Full Text Available The aim of the study was to establish the characteristics of presentation of 94 patients with Kinelfelter's syndrome (KS referred to the endocrinologist at different ages. The diagnosis of KS was more frequent in the age group between 11 and 20 years (46.8%. Most of the patients (83.7% showed the classic 47,XXY karyotype and 7.1% showed a 47,XXY/46,XY mosaicism. Half of the patients younger than 18 years presented mild neurodevelopmental disorders. The most frequent clinical findings were cryptorchidism in prepubertal patients, and small testes, cryptorchidism, and gynecomastia in pubertal patients. FSH, LH, AMH, and inhibin B levels were normal in prepubertal patients and became abnormal from midpuberty. Most adults were referred for small testes, infertility, and gynecomastia; 43.6% had sexual dysfunction. Testosterone levels were low in 45%. Mean stature was above the 50th percentile, and 62.5% had BMI ≥25.0 kg/m2. In conclusion, the diagnosis of Klinefelter syndrome seems to be made earlier nowadays probably because pediatricians are more aware that boys and adolescents with neuro-developmental disorders and cryptorchidism are at increased risk. The increasing use of prenatal diagnosis has also decreased the mean age at diagnosis and allowed to get insight into the evolution of previously undiagnosed cases, which probably represent the mildest forms. In adults average height and weight are slightly higher than those in the normal population. Bone mineral density is mildly affected, more at the spine than at the femoral neck level, in less than half of cases.

  9. CASKS (Computer Analysis of Storage casKS): A microcomputer based analysis system for storage cask design review. User's manual to Version 1b (including program reference)

    International Nuclear Information System (INIS)

    Chen, T.F.; Gerhard, M.A.; Trummer, D.J.; Johnson, G.L.; Mok, G.C.

    1995-02-01

    CASKS (Computer Analysis of Storage casKS) is a microcomputer-based system of computer programs and databases developed at the Lawrence Livermore National Laboratory (LLNL) for evaluating safety analysis reports on spent-fuel storage casks. The bulk of the complete program and this user's manual are based upon the SCANS (Shipping Cask ANalysis System) program previously developed at LLNL. A number of enhancements and improvements were added to the original SCANS program to meet requirements unique to storage casks. CASKS is an easy-to-use system that calculates global response of storage casks to impact loads, pressure loads and thermal conditions. This provides reviewers with a tool for an independent check on analyses submitted by licensees. CASKS is based on microcomputers compatible with the IBM-PC family of computers. The system is composed of a series of menus, input programs, cask analysis programs, and output display programs. All data is entered through fill-in-the-blank input screens that contain descriptive data requests

  10. Measurement of the branching ratios of D(+) and D(+)(s) hadronic decays to four-body final states containing a K(S).

    Science.gov (United States)

    Link, J M; Reyes, M; Yager, P M; Anjos, J C; Bediaga, I; Göbel, C; Magnin, J; Massafferi, A; de Miranda, J M; Pepe, I M; dos Reis, A C; Simão, F R; Carrillo, S; Casimiro, E; Sánchez-Hernández, A; Uribe, C; Vázquez, F; Cinquini, L; Cumalat, J P; O'Reilly, B; Ramirez, J E; Vaandering, E W; Butler, J N; Cheung, H W; Gaines, I; Garbincius, P H; Garren, L A; Gottschalk, E; Kasper, P H; Kreymer, A E; Kutschke, R; Bianco, S; Fabbri, F L; Sarwar, S; Zallo, A; Cawlfield, C; Kim, D Y; Rahimi, A; Wiss, J; Gardner, R; Chung, Y S; Kang, J S; Ko, B R; Kwak, J W; Lee, K B; Park, H; Alimonti, G; Boschini, M; Caccianiga, B; D'Angelo, P; DiCorato, M; Dini, P; Giammarchi, M; Inzani, P; Leveraro, F; Malvezzi, S; Menasce, D; Mezzadri, M; Milazzo, L; Moroni, L; Pedrini, D; Pontoglio, C; Prelz, F; Rovere, M; Sala, A; Sala, S; Davenport, T F; Agostino, L; Arena, V; Boca, G; Bonomi, G; Gianini, G; Liguori, G; Merlo, M; Pantea, D; Ratti, S P; Riccardi, C; Segoni, I; Viola, L; Vitulo, P; Hernandez, H; Lopez, A M; Mendez, H; Mendez, L; Mirles, A; Montiel, E; Olaya, D; Paris, A; Quinones, J; Rivera, C; Xiong, W; Zhang, Y; Wilson, J R; Cho, K; Handler, T; Engh, D; Hosack, M; Johns, W E; Nehring, M; Sheldon, P D; Stenson, K; Webster, M; Sheaff, M

    2001-10-15

    We have studied hadronic four-body decays of D(+) and D(+)(s) mesons with a K(S) in the final state using data recorded during the 1996-1997 fixed-target run of the Fermilab high energy photoproduction experiment FOCUS. We report a new branching ratio measurement of gamma(D(+)-->K(S)K-pi(+)pi(+))/gamma(D(+)-->K(S)pi(+)pi(+)pi(-)) = 0.0768+/-0.0041+/-0.0032. We make the first observation of three new decay modes with branching ratios gamma(D(+)-->K(S)K+pi(+)pi(-))/gamma(D(+)-->K(S)pi(+)pi(+)pi(-)) = 0.0562+/-0.0039+/-0.0040, gamma(D(+)-->K(S)K+K-pi(+))/gamma(D(+)-->K(S)pi(+)pi(+)pi(-)) = 0.0077+/-0.0015+/-0.0009, and gamma(D(+)(s)-->K(S)K+pi(+)pi(-))/gamma(D(+)(s)-->K(S)K-pi(+)pi(+)) = 0.586+/-0.052+/-0.043, where in each case the first error is statistical and the second error is systematic.

  11. MLL2 mutation detection in 86 patients with Kabuki syndrome: a genotype-phenotype study.

    Science.gov (United States)

    Makrythanasis, P; van Bon, B W; Steehouwer, M; Rodríguez-Santiago, B; Simpson, M; Dias, P; Anderlid, B M; Arts, P; Bhat, M; Augello, B; Biamino, E; Bongers, E M H F; Del Campo, M; Cordeiro, I; Cueto-González, A M; Cuscó, I; Deshpande, C; Frysira, E; Izatt, L; Flores, R; Galán, E; Gener, B; Gilissen, C; Granneman, S M; Hoyer, J; Yntema, H G; Kets, C M; Koolen, D A; Marcelis, C l; Medeira, A; Micale, L; Mohammed, S; de Munnik, S A; Nordgren, A; Psoni, S; Reardon, W; Revencu, N; Roscioli, T; Ruiterkamp-Versteeg, M; Santos, H G; Schoumans, J; Schuurs-Hoeijmakers, J H M; Silengo, M C; Toledo, L; Vendrell, T; van der Burgt, I; van Lier, B; Zweier, C; Reymond, A; Trembath, R C; Perez-Jurado, L; Dupont, J; de Vries, B B A; Brunner, H G; Veltman, J A; Merla, G; Antonarakis, S E; Hoischen, A

    2013-12-01

    Recently, pathogenic variants in the MLL2 gene were identified as the most common cause of Kabuki (Niikawa-Kuroki) syndrome (MIM#147920). To further elucidate the genotype-phenotype correlation, we studied a large cohort of 86 clinically defined patients with Kabuki syndrome (KS) for mutations in MLL2. All patients were assessed using a standardized phenotype list and all were scored using a newly developed clinical score list for KS (MLL2-Kabuki score 0-10). Sequencing of the full coding region and intron-exon boundaries of MLL2 identified a total of 45 likely pathogenic mutations (52%): 31 nonsense, 10 missense and four splice-site mutations, 34 of which were novel. In five additional patients, novel, i.e. non-dbSNP132 variants of clinically unknown relevance, were identified. Patients with likely pathogenic nonsense or missense MLL2 mutations were usually more severely affected (median 'MLL2-Kabuki score' of 6) as compared to the patients without MLL2 mutations (median 'MLL2-Kabuki score' of 5), a significant difference (p < 0.0014). Several typical facial features such as large dysplastic ears, arched eyebrows with sparse lateral third, blue sclerae, a flat nasal tip with a broad nasal root, and a thin upper and a full lower lip were observed more often in mutation positive patients. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  12. Hepatorenal Syndrome

    Directory of Open Access Journals (Sweden)

    Ebru Yilmaz

    2014-06-01

    Full Text Available Hepatorenal syndrome (HRS is functional renal failure that occurs with advanced liver failure. HRS is considered the most severe complication of cirrhosis. Type 1 HRS develops due to severe reduction of effective circulating volume results in hemodynamic dysfunction. Type 1 HRS is characterized by acute renal failure and rapid deterioration in the function of other organs. It can ocur spontaneously or in the setting of a precipitating event. Type 2 hepatorenal syndrome (HRS, which is characterized by slowly progressive renal failure and refractory ascites. Liver transplantation is the only definitive treatment for both type. The most suitable and ldquo;bridge treatments and rdquo; or treatment for patients ineligible for a liver transplant include terlipressin plus albumin. [J Contemp Med 2014; 4(2.000: 106-113

  13. Dravet syndrome

    Directory of Open Access Journals (Sweden)

    Incorpora Gemma

    2009-09-01

    Full Text Available Abstract "Dravet syndrome" (DS previously named severe myoclonic epilepsy of infancy (SMEI, or epilepsy with polymorphic seizures, is a rare disorder characterized by an early, severe, generalized, epileptic encephalopathy. DS is characterized by febrile and afebrile seizures beginning in the 1st year of life followed by different types of seizures (either focal or generalized, which are typically resistant to antiepileptic drugs. A developmental delay from the 2nd to 3rd year of life becomes evident, together with motor disturbances and personality disorders. Beside the classic syndrome, there are milder cases which have been called severe myoclonic epilepsy borderline (SMEB. DS is caused by a mutation in the neuronal sodium channel gene, SCN1A , that is also mutated in generalized epilepsy with FS+ (GEFS+.

  14. Apert syndrome

    Directory of Open Access Journals (Sweden)

    Premalatha

    2010-01-01

    Full Text Available Apert syndrome (acrocephalosyndactyly is a rare developmental malformation characterized by craniosynostosis, mid-face hypoplasia, symmetrical syndactyly of hands and feet. The prodromal characteristics for the typical cranio-facial appearance are early craniosynostosis of the coronal suture, cranial base and agenesis of the sagittal suture. The purpose of this paper is to report a case of Apert syndrome with emphasis on craniofacial and oral features in an eighteen-month-old male child. The patient presented with several craniofacial deformities, including brachycephaly, midface hypoplasia, flat face, hypertelorism, ocular proptosis, downslanting palpebral fissures. Syndactylies with osseous fusion of the hands and feet were also observed. Intraoral findings included delayed eruption of teeth, high arched palate with pseudo cleft in the posterior one third.

  15. Paraneoplastic syndromes

    Energy Technology Data Exchange (ETDEWEB)

    Weller, R.E.

    1994-03-01

    Paraneoplastic syndromes (PNS) comprise a diverse group of disorders that are associated with cancer but unrelated to the size, location, metastases, or physiologic activities of the mature tissue of origin. They are remote effects of tumors that may appear as signs, symptoms, or syndromes which can mimic other disease conditions encountered in veterinary medicine. Recognition of PNS is valuable for several reasons: the observed abnormalities may represent tumor cell markers and facilitate early diagnosis of the tumor; they may allow assessment of premalignant states; they may aid in the search metastases; they may help quantify and monitor response to therapy; and, they may provide insight into the study of malignant transformation and oncogene expression. This review will concentrate on the pathophysiology, diagnosis, and treatment of some of the common PNS encountered in veterinary medicine.

  16. Paraneoplastiske syndromer

    OpenAIRE

    Røsbekk, Stein Helge

    2007-01-01

    During the last 50 years it has become clear that malignant tumours can induce symptoms unrelated to the mechanical effects of the primary tumour itself or its metastasis. Today, the name Paraneoplastic syndrome is given to those symptom complexes that may affect the blood cells, electrolytes, coagulation system, muscle, skin, nerve and the endocrine system. Endocrine symptoms were first recognised, and different hormones were isolated from the tumour tissue. However, tumour derived hormones ...

  17. Caroli's syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Numan, F; Cokyueksel, O; Camuscu, S; Demir, K; Dueren, M

    1986-07-01

    In 1958 Caroli described the syndrome of congenital, either segmental or involving the entire bile duct system, saccular extensions of the intrahepatic bile ducts. He differentiated between two types of this disease pattern. The first form concerns pure cystic dilatations of the intrahepatic bile ducts, whereas the second one is combined with hepatic fibrosis and portal hypertension. Both types are characterised by cystic dilatations in the kidneys and in the extrahepatic bile ducts, pancreas and spleen.

  18. Griscelli syndrome

    Directory of Open Access Journals (Sweden)

    Kumar T

    2006-01-01

    Full Text Available Partial albinism with immunodeficiency is a rare and fatal immunologic disorder characterized by pigmentary dilution and variable cellular immunodeficiency. It was initially described in 1978. Primary abnormalities included silvery grayish sheen to the hair, large pigment agglomerations in hair shafts and an abundance of mature melanosomes in melanocytes, with reduced pigmentation of adjacent keratinocytes. We describe a child with Griscelli syndrome who presented with hepatitis, pancytopenia and silvery hair. The diagnosis was confirmed by microscopic skin and hair examination.

  19. Waardenburg syndrome

    OpenAIRE

    Mehta, Manish; Kavadu, Paresh; Chougule, Sachin

    2004-01-01

    We report a case of Waardenburg syndrome in a female child aged 2yrs. Petrus Johannes Waardenburg(1) , a Dutch Ophthalmologist in 1951 described individuals with retinal pigmentary differences who had varying degrees of hearing loss and dystopia canthorum (i.e., latral displacement of inner canthi of eyes). The disease runs in families with a dominant inheritance pattern with varying degree of clinical presentation. Patient usually present with heterochromic iris, pigmentary abnormalities of ...

  20. [PHACES syndrome].

    Science.gov (United States)

    Morcillo Azcárate, J; Bernabeu-Wittel, J; Fernández-Pineda, I; Conejo-Mir, M D; Tuduri Limousin, I; Aspiazu Salinas, D A; de Agustín Asensio, J C

    2010-04-01

    PHACES syndrome associates a segmental facial hemangioma with cerebral malformations, aortic branches/cranial arteries anomalies, cardiac defects, eye anomalies or ventral wall defects. The aim of this study is to analyze our experience with this syndrome. Retrospective study of the cases seen at our unit in the last year. We treat 4 cases; 3 girls and 1 child. Besides the segmental hemangioma they presented: 3 vascular cerebral malformations; 2 structural cardiopathies; 2 cerebral malformations, 1 microftalmia. We did not find ventral wall defects. A case received treatment with two cycles of metilprednisolone i.v. and oral prednisone, with favourable course; two cases received initial treatment with oral prednisone continued of oral propanolol in rising pattern up to 2 mg/kg/day, Obtaining both the detention of the tumour growth and regression of the lesion, with very good tolerance. A 7-year-old patient has been treated with colouring pulse laser for her residual lesions. When we see a segmental facial hemangioma we must perform a wide diagnostic study in order to discard a PHACES syndrome. Multidisciplinar approach to the patient by a wide expert's group gets an earlier diagnose and improves the outcome. Propranolol is a promising therapeutic alternative.

  1. Anserine syndrome.

    Science.gov (United States)

    Helfenstein, Milton; Kuromoto, Jorge

    2010-01-01

    Knee pain is a common complaint in clinical practice, and pes anserinus tendino-bursitis syndrome (PATB) has been frequently diagnosed based only on clinical features that may cause equivocal interpretations. Patients complain of characteristic spontaneous medial knee pain with tenderness in the inferomedial aspect of the joint. Studies with different imaging modalities have been undertaken during the last years to identify whether these patients suffer from bursitis, tendinitis, or both. Nevertheless, little is known regarding the structural defect responsible for this disturbance. Due to these problems and some controversies, we suggest the term "anserine syndrome" for this condition. Diabetes Mellitus is a known predisposing factor for this syndrome. Overweight and osteoarthritis seem to represent additional risk factors; however, their role in the pathophysiology of the disease is not yet understood. Treatment includes non-steroidal anti-inflammatory drugs, physiotherapy, and injections of corticosteroid, with highly variable responses, from 10 days to 36 months to achieve recovery. The lack of knowledge about its epidemiological, etiological, and pathophysiological aspects requires future studies for this common and intriguing disorder.

  2. Neonatal respiratory distress syndrome

    Science.gov (United States)

    Hyaline membrane disease (HMD); Infant respiratory distress syndrome; Respiratory distress syndrome in infants; RDS - infants ... improves slowly after that. Some infants with severe respiratory distress syndrome will die. This most often occurs ...

  3. Toxic shock syndrome

    Science.gov (United States)

    Staphylococcal toxic shock syndrome; Toxic shock-like syndrome; TSLS ... Toxic shock syndrome is caused by a toxin produced by some types of staphylococcus bacteria. A similar problem, called toxic shock- ...

  4. Prune belly syndrome

    Science.gov (United States)

    Eagle-Barrett syndrome; Triad syndrome ... The exact causes of prune belly syndrome are unknown. The condition affects mostly boys. While in the womb, the developing baby's abdomen swells with fluid. Often, the cause is ...

  5. What Causes Cushing's Syndrome?

    Science.gov (United States)

    ... Share Facebook Twitter Pinterest Email Print What causes Cushing syndrome? Cushing syndrome can develop for two reasons: Medication ... uhs ), thyroid, or thymus How Tumors Can Cause Cushing Syndrome Normally, the pituitary gland in the brain controls ...

  6. Molecular Analysis of Collagen XVIII Reveals Novel Mutations, Presence of a Third Isoform, and Possible Genetic Heterogeneity in Knobloch Syndrome

    Science.gov (United States)

    Suzuki, O. T.; Sertié, A. L.; Der Kaloustian, V. M.; Kok, F.; Carpenter, M.; Murray, J.; Czeizel, A. E.; Kliemann, S. E.; Rosemberg, S.; Monteiro, M.; Olsen, B. R.; Passos-Bueno, M. R.

    2002-01-01

    Knobloch syndrome (KS) is a rare disease characterized by severe ocular alterations, including vitreoretinal degeneration associated with retinal detachment and occipital scalp defect. The responsible gene, COL18A1, has been mapped to 21q22.3, and, on the basis of the analysis of one family, we have demonstrated that a mutation affecting only one of the three COL18A1 isoforms causes this phenotype. We report here the results of the screening of both the entire coding region and the exon-intron boundaries of the COL18A1 gene (which includes 43 exons), in eight unrelated patients with KS. Besides 20 polymorphic changes, we identified 6 different pathogenic changes in both alleles of five unrelated patients with KS (three compound heterozygotes and two homozygotes). All are truncating mutations leading to deficiency of one or all collagen XVIII isoforms and endostatin. We have verified that, in exon 41, the deletion c3514-3515delCT, found in three unrelated alleles, is embedded in different haplotypes, suggesting that this mutation has occurred more than once. In addition, our results provide evidence of nonallelic genetic heterogeneity in KS. We also show that the longest human isoform (NC11-728) is expressed in several tissues (including the human eye) and that lack of either the short variant or all of the collagen XVIII isoforms causes similar phenotypes but that those patients who lack all forms present more-severe ocular alterations. Despite the small sample size, we found low endostatin plasma levels in those patients with mutations leading to deficiency of all isoforms; in addition, it seems that absence of all collagen XVIII isoforms causes predisposition to epilepsy. PMID:12415512

  7. Genetics Home Reference: antiphospholipid syndrome

    Science.gov (United States)

    ... Share: Email Facebook Twitter Home Health Conditions Antiphospholipid syndrome Antiphospholipid syndrome Printable PDF Open All Close All Enable ... area? Other Names for This Condition anti-phospholipid syndrome antiphospholipid antibody syndrome Hughes syndrome Related Information How are ...

  8. Genetics Home Reference: Costello syndrome

    Science.gov (United States)

    ... other genetic conditions, cardiofaciocutaneous syndrome (CFC syndrome) and Noonan syndrome . In affected infants, it can be difficult to ... These individuals may actually have CFC syndrome or Noonan syndrome , which are caused by mutations in related genes. ...

  9. Tuba üks null üks / Abdul Turay

    Index Scriptorium Estoniae

    Turay, Abdul, 1967-

    2010-01-01

    Autor leiab, et iga Eesti külaline võib täheldada, et eestlased tunnevad suurt hirmu rahvusliku väljasuremise ees. Eesti noorte taibukusest ja andekusest, autor ei usu, et Eesti noortel on alkoholiga probleeme, pidades seda teemat meedia ja poliitikute poolt ületähtsustatuks

  10. Acute nephritic syndrome

    Science.gov (United States)

    Glomerulonephritis - acute; Acute glomerulonephritis; Nephritis syndrome - acute ... Acute nephritic syndrome is often caused by an immune response triggered by an infection or other disease. Common causes in children ...

  11. ks, tuks. Sjoo elo om - tuks..." : [luuletused : võru keeles] / Aljona Jeltsova ; komi keelest tõlkinud Nikolai Kuznetsov, võru keelde Kauksi Ülle

    Index Scriptorium Estoniae

    Jeltsova, Aljona

    2005-01-01

    Sisu: "Üks, tuks. Sjoo elo om - tuks..." ; "Ammuq lännüq pilveq - luigaq..." ; "Ma üles märki laulu jovva-õs..." ; Sukka mi ; "Täämbä üüse om taivah üts täht..." ; "Ma tulõ viirde uma süäme jäti..."

  12. The effect of errorless learning on quality of life in patients with Korsakoff’s syndrome

    Directory of Open Access Journals (Sweden)

    Rensen YCM

    2017-11-01

    Full Text Available Yvonne CM Rensen,1 Jos IM Egger,2,3 Josette Westhoff,1 Serge JW Walvoort,1 Roy PC Kessels1,3,4 1Center of Excellence for Korsakoff and Alcohol-Related Cognitive Disorders, Vincent van Gogh Institute for Psychiatry, Venray, the Netherlands; 2Center of Excellence for Neuropsychiatry, Vincent van Gogh Institute for Psychiatry, Venray, the Netherlands; 3Donders Institute for Brain, Cognition, and Behavior, Radboud University, Nijmegen, 4Department of Medical Psychology, Radboud University Medical Center, Nijmegen, the Netherlands Background: Errorless learning (EL is a promising rehabilitation principle for (relearning instrumental activities in patients with amnesia, including patients with Korsakoff’s syndrome (KS. Successfully (relearning tasks might improve the sense of competence and independence, and subsequently the quality of life. Quality of life in patients with KS has received limited attention, and no studies have been conducted to experimentally examine the effect of EL on quality of life in patients in this patient group. Materials and methods: The QUALIDEM, an observation scale for quality of life, was completed by professional nurses before and after EL training in a group of fifty-one patients with KS. This scale was also completed for a group of thirty-one control patients receiving care as usual but no EL training. Results: Quality of life was significantly increased on eight of the nine subscales in the Korsakoff group who participated in an EL training. There was a trend toward a significant increase in “positive affect” (ie, the ninth subscale. In contrast, no changes over time were found on any of the subscales in the control group that did not participate in any EL training. Conclusion: Despite severe memory impairments, patients with KS still have the potential to (partially (relearn tasks using EL. This potential should be exploited, as the successes of (re-learning might improve the quality of life of Korsakoff

  13. Morvan Syndrome

    Science.gov (United States)

    Maskery, Mark; Chhetri, Suresh K.; Dayanandan, Rejith; Gall, Claire

    2016-01-01

    A 74-year-old gentleman was admitted to the regional neurosciences center with encephalopathy, myokymia, and dysautonomia. Chest imaging had previously identified an incidental mass in the anterior mediastinum, consistent with a primary thymic tumor. Antivoltage-gated potassium channel (anti-VGKC) antibodies were positive (titer 1273 pmol/L) and he was hypokalemic. Electromyogram and nerve conduction studies were in keeping with peripheral nerve hyperexcitability syndrome, and an electroencephalogram was consistent with encephalopathy. A diagnosis of Morvan syndrome was made, for which he was initially treated with high-dose steroids, followed by a 5-day course of intravenous immunoglobulin (IVIG) therapy. He also underwent thymectomy, followed by a postexcision flare of his symptoms requiring intensive care management. Further steroids, plasmapheresis, and IVIG achieved stabilization of his clinical condition, enabling transfer for inpatient neurorehabilitation. He was commenced on azathioprine and a prolonged oral steroid taper. A subsequent presumed incipient relapse responded well to further IVIG treatment. This case report documents a thymoma-associated presentation of anti-VGKC-positive Morvan syndrome supplemented by patient and carer narrative and video, both of which provide valuable further insights into this rare disorder. There are a limited number of publications surrounding this rare condition available in the English literature. This, combined with the heterogenous presentation, association with underlying malignancy, response to treatment, and prognosis, provides a diagnostic challenge. However, the association with anti-VGKC antibody-associated complexes and 2 recent case series have provided some scope for both accurate diagnosis and management. PMID:26740856

  14. Jacobsen syndrome

    Directory of Open Access Journals (Sweden)

    Grossfeld Paul

    2009-03-01

    Full Text Available Abstract Jacobsen syndrome is a MCA/MR contiguous gene syndrome caused by partial deletion of the long arm of chromosome 11. To date, over 200 cases have been reported. The prevalence has been estimated at 1/100,000 births, with a female/male ratio 2:1. The most common clinical features include pre- and postnatal physical growth retardation, psychomotor retardation, and characteristic facial dysmorphism (skull deformities, hypertelorism, ptosis, coloboma, downslanting palpebral fissures, epicanthal folds, broad nasal bridge, short nose, v-shaped mouth, small ears, low set posteriorly rotated ears. Abnormal platelet function, thrombocytopenia or pancytopenia are usually present at birth. Patients commonly have malformations of the heart, kidney, gastrointestinal tract, genitalia, central nervous system and skeleton. Ocular, hearing, immunological and hormonal problems may be also present. The deletion size ranges from ~7 to 20 Mb, with the proximal breakpoint within or telomeric to subband 11q23.3 and the deletion extending usually to the telomere. The deletion is de novo in 85% of reported cases, and in 15% of cases it results from an unbalanced segregation of a familial balanced translocation or from other chromosome rearrangements. In a minority of cases the breakpoint is at the FRA11B fragile site. Diagnosis is based on clinical findings (intellectual deficit, facial dysmorphic features and thrombocytopenia and confirmed by cytogenetics analysis. Differential diagnoses include Turner and Noonan syndromes, and acquired thrombocytopenia due to sepsis. Prenatal diagnosis of 11q deletion is possible by amniocentesis or chorionic villus sampling and cytogenetic analysis. Management is multi-disciplinary and requires evaluation by general pediatrician, pediatric cardiologist, neurologist, ophthalmologist. Auditory tests, blood tests, endocrine and immunological assessment and follow-up should be offered to all patients. Cardiac malformations can be

  15. Robinow syndrome

    Directory of Open Access Journals (Sweden)

    Suresh S

    2008-01-01

    Full Text Available Robinow syndrome is a rare autosomal recessive mesomelic dwarfism with just more than 100 cases reported in the literature so far. The lower extremity is spared with skeletal deformity usually confined to the forearm, hand, and the dorsal spine. Diagnosis is made easily in the early childhood by the typical "fetal facies" appearance, which disappears to a certain extent as the patient grows. The author reports two cases of this entity with vertebral segmentation defects, rib fusion, and typical severe brachymelia and facial features.

  16. Trichorhinophalangeal syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Tuzovic, S.; Fiebach, B.J.O.; Magnus, L.; Sauerbrei, H.U.

    1982-11-01

    This article reports on 14 cases of a trichorhinophalangeal syndrome in five successive generations. Besides the well-known characteristics of the TRPS the following symptoms observed in this family are new: Teething was considerably delayed, intelligence was reduced, and there were skin manifestations resembling eczema. Besides, struma colli and colitis ulcerosa were also observed. Subsequent observations have to clarify whether these symptoms are a facultative part of the TRPS pattern. The constant appearance of carriers of these characteristics during five generation points to dominant heredity.

  17. Olmsted Syndrome

    Directory of Open Access Journals (Sweden)

    Sirka C

    1999-01-01

    Full Text Available A 20-year-old Sikh man had palmoplantar keratoderma, flexion deformity of digits, universal alopecia, keratotic plaques at the angles of mouth, gluteal cleft, knees and dorsal aspects of the metacarpophalangeal joints of the hand; features of Olmsted syndrome. He had normal nails, teeth, oral mucosa and normal joint movements. Treatment with acitretin, 25mg/day for three and a half months, followed by 25mg once daily alternating with 50mg once daily for 3 months resulted in significant improvement.

  18. OCULO-CEREBRO-RENAL SYNDROME (LOWE'S SYNDROME)

    Institute of Scientific and Technical Information of China (English)

    1991-01-01

    Oculo-cerebro-renal syndrome (Lowe's syndrome) is characterized by mental and motor retardation, cataract, glaucoma and renal abnormalities. It is an X-linked recessive metabolic disease. Two brothers suffering from Lowe's syndrome are reported. Their mother with lenticular opacities and peculiar facial appearance is in concordance with the obligate carrier. The ocular changes and heridity are discussed.

  19. Cardiorenal syndrome

    Directory of Open Access Journals (Sweden)

    Sabry Omar

    2013-01-01

    Full Text Available Cardiovascular disease is the leading cause of death in patients with chronic kidney disease.  Heart failure may lead to acute kidney injury and vice versa. Chronic kidney disease may affect the clinical outcomes in patients with cardiovascular disorders. Renal impairment with any degree of albuminuria has been increasingly recognized as an independent risk factor for cardiovascular events and heart failure hospitalizations, while chronic heart failure may cause chronic kidney disease. The bidirectional nature of these disorders contributes to the complexity and the composite definitions of cardiorenal syndromes. However, the most important clinical trials in heart failure tend to exclude patients with significant renal dysfunction. The mechanisms whereby renal insufficiency worsens the outcome in heart failure are not known, and several pathways could contribute to the ‘‘vicious heart/kidney circle.’’ Traditionally, renal impairment has been attributed to the renal hypoperfusion due to reduced cardiac output and decreased systemic pressure. The hypovolemia leads to sympathetic activity, increased renin-angiotensin aldosterone pathway, and arginine-vasopressin release. These mechanisms cause fluid and sodium retention, peripheral vasoconstriction, and volume overload. Therapy to improve renal dysfunction, reduce neurohormonal activation and ameliorate renal blood flow could lead to a reduction in mortality and hospitalization in patients with cardiorenal syndrome.

  20. Lowe syndrome

    Directory of Open Access Journals (Sweden)

    Loi Mario

    2006-05-01

    Full Text Available Abstract Lowe syndrome (the oculocerebrorenal syndrome of Lowe, OCRL is a multisystem disorder characterised by anomalies affecting the eye, the nervous system and the kidney. It is a uncommon, panethnic, X-linked disease, with estimated prevalence in the general population of approximately 1 in 500,000. Bilateral cataract and severe hypotonia are present at birth. In the subsequent weeks or months, a proximal renal tubulopathy (Fanconi-type becomes evident and the ocular picture may be complicated by glaucoma and cheloids. Psychomotor retardation is evident in childhood, while behavioural problems prevail and renal complications arise in adolescence. The mutation of the gene OCRL1 localized at Xq26.1, coding for the enzyme phosphatidylinositol (4,5 bisphosphate 5 phosphatase, PtdIns (4,5P2, in the trans-Golgi network is responsible for the disease. Both enzymatic and molecular testing are available for confirmation of the diagnosis and for prenatal detection of the disease. The treatment includes: cataract extraction, glaucoma control, physical and speech therapy, use of drugs to address behavioural problems, and correction of the tubular acidosis and the bone disease with the use of bicarbonate, phosphate, potassium and water. Life span rarely exceeds 40 years.

  1. Cotard Syndrome.

    Science.gov (United States)

    Dieguez, Sebastian

    2018-01-01

    Cotard's syndrome is often described as the delusional belief that one is dead or non-existent. However, Jules Cotard's initial description (1880) of the "delusion of negations" was much richer and also involved delusions and claims of immortality and enormity, feelings of damnation, and illusions of bodily dissolution and transformation. Alternatively conceived as an extreme case of depression, hypochondria, or psychosis, the condition is considered rare and remains poorly understood. Cotard himself provided a taxonomy and several explanations for the condition, focusing on its distinction from classical persecutory delusions and suggesting that it could be a kind of reversed grandiosity. He proposed a psychosensory basis in the dissolution of mental imagery, which he then extended to a more general psychomotor impairment of volition. Other early authors highlighted a disorder of the bodily self, and more recent theories postulated an impairment of right hemispheric functions, leading to perceptual and somatosensory feelings of unreality, which coupled with reasoning impairments and an internalized attributional style led in turn to beliefs of non-existence. However, despite its striking presentation and its relevance to our understanding of self-awareness, Cotard's syndrome remains an elusive condition, rarely reported and poorly researched. © 2018 S. Karger AG, Basel.

  2. KBG syndrome

    Directory of Open Access Journals (Sweden)

    Brancati Francesco

    2006-12-01

    Full Text Available Abstract KBG syndrome is a rare condition characterised by a typical facial dysmorphism, macrodontia of the upper central incisors, skeletal (mainly costovertebral anomalies and developmental delay. To date, KBG syndrome has been reported in 45 patients. Clinical features observed in more than half of patients that may support the diagnosis are short stature, electroencephalogram (EEG anomalies (with or without seizures and abnormal hair implantation. Cutaneous syndactyly, webbed short neck, cryptorchidism, hearing loss, palatal defects, strabismus and congenital heart defects are less common findings. Autosomal dominant transmission has been observed in some families, and it is predominantly the mother, often showing a milder clinical picture, that transmits the disease. The diagnosis is currently based solely on clinical findings as the aetiology is unknown. The final diagnosis is generally achieved after the eruption of upper permanent central incisors at 7–8 years of age when the management of possible congenital anomalies should have been already planned. A full developmental assessment should be done at diagnosis and, if delays are noted, an infant stimulation program should be initiated. Subsequent management and follow-up should include an EEG, complete orthodontic evaluation, skeletal investigation with particular regard to spine curvatures and limb asymmetry, hearing testing and ophthalmologic assessment.

  3. Elsberg syndrome

    Science.gov (United States)

    Savoldi, Filippo; Kaufmann, Timothy J.; Flanagan, Eoin P.; Toledano, Michel

    2017-01-01

    Objective: Elsberg syndrome (ES) is an established but often unrecognized cause of acute lumbosacral radiculitis with myelitis related to recent herpes virus infection. We defined ES, determined its frequency in patients with cauda equina syndrome (CES) with myelitis, and evaluated its clinical, radiologic, and microbiologic features and outcomes. Methods: We searched the Mayo Clinic medical records for ES and subsequently for combinations of index terms to identify patients with suspected CES and myelitis. Results: Our search yielded 30 patients, 2 diagnosed with ES and an additional 28 with clinical or radiologic evidence of CES retrospectively suspected of having ES. We classified patients in 5 groups according to diagnostic certainty. MRI and EMG confirmed that 2 had only myelitis, 5 only radiculitis, and 16 both. Two had preceding sacral herpes infection and 1 oral herpes simplex. Spinal cord lesions were commonly multiple, discontinuous, not expansile, and centrally or ventrally positioned. Lesions generally spared the distal conus. Nerve root enhancement was occasionally prominent and was smooth rather than nodular. Lymphocytic CSF pleocytosis was common. Thirteen patients (43%) had viral isolation studies, which were commonly delayed; the delay may have accounted for the low rate of viral detection. Acyclovir was administered to 6 patients. Most patients recovered with sequelae; 1 patient experienced encephalomyelitis and died. Conclusion: ES is a definable condition likely responsible for 10% of patients with combined CES and myelitis. Radiologic findings are not entirely specific but may help in differentiating ES from some competing diagnostic considerations. We propose criteria to facilitate diagnosis. PMID:28534040

  4. Sotos syndrome

    Directory of Open Access Journals (Sweden)

    Cormier-Daire Valérie

    2007-09-01

    Full Text Available Abstract Sotos syndrome is an overgrowth condition characterized by cardinal features including excessive growth during childhood, macrocephaly, distinctive facial gestalt and various degrees of learning difficulty, and associated with variable minor features. The exact prevalence remains unknown but hundreds of cases have been reported. The diagnosis is usually suspected after birth because of excessive height and occipitofrontal circumference (OFC, advanced bone age, neonatal complications including hypotonia and feeding difficulties, and facial gestalt. Other inconstant clinical abnormalities include scoliosis, cardiac and genitourinary anomalies, seizures and brisk deep tendon reflexes. Variable delays in cognitive and motor development are also observed. The syndrome may also be associated with an increased risk of tumors. Mutations and deletions of the NSD1 gene (located at chromosome 5q35 and coding for a histone methyltransferase implicated in transcriptional regulation are responsible for more than 75% of cases. FISH analysis, MLPA or multiplex quantitative PCR allow the detection of total/partial NSD1 deletions, and direct sequencing allows detection of NSD1 mutations. The large majority of NSD1 abnormalities occur de novo and there are very few familial cases. Although most cases are sporadic, several reports of autosomal dominant inheritance have been described. Germline mosaicism has never been reported and the recurrence risk for normal parents is very low (

  5. Variations in PROKR2, But Not PROK2, Are Associated With Hypopituitarism and Septo-optic Dysplasia

    Science.gov (United States)

    McCabe, Mark J.; Gaston-Massuet, Carles; Gregory, Louise C.; Alatzoglou, Kyriaki S.; Tziaferi, Vaitsa; Sbai, Oualid; Rondard, Philippe; Masumoto, Koh-hei; Nagano, Mamoru; Shigeyoshi, Yasufumi; Pfeifer, Marija; Hulse, Tony; Buchanan, Charles R.; Pitteloud, Nelly; Martinez-Barbera, Juan-Pedro

    2013-01-01

    Context: Loss-of-function mutations in PROK2 and PROKR2 have been implicated in Kallmann syndrome (KS), characterized by hypogonadotropic hypogonadism and anosmia. Recent data suggest overlapping phenotypes/genotypes between KS and congenital hypopituitarism (CH), including septo-optic dysplasia (SOD). Objective: We screened a cohort of patients with complex forms of CH (n = 422) for mutations in PROK2 and PROKR2. Results: We detected 5 PROKR2 variants in 11 patients with SOD/CH: novel p.G371R and previously reported p.A51T, p.R85L, p.L173R, and p.R268C—the latter 3 being known functionally deleterious variants. Surprisingly, 1 patient with SOD was heterozygous for the p.L173R variant, whereas his phenotypically unaffected mother was homozygous for the variant. We sought to clarify the role of PROKR2 in hypothalamopituitary development through analysis of Prokr2−/− mice. Interestingly, these revealed predominantly normal hypothalamopituitary development and terminal cell differentiation, with the exception of reduced LH; this was inconsistent with patient phenotypes and more analogous to the healthy mother, although she did not have KS, unlike the Prokr2−/− mice. Conclusions: The role of PROKR2 in the etiology of CH, SOD, and KS is uncertain, as demonstrated by no clear phenotype-genotype correlation; loss-of-function variants in heterozygosity or homozygosity can be associated with these disorders. However, we report a phenotypically normal parent, homozygous for p.L173R. Our data suggest that the variants identified herein are unlikely to be implicated in isolation in these disorders; other genetic or environmental modifiers may also impact on the etiology. Given the phenotypic variability, genetic counseling may presently be inappropriate. PMID:23386640

  6. Marfan Syndrome (For Parents)

    Science.gov (United States)

    ... en español Síndrome de Marfan What Is Marfan Syndrome? Marfan syndrome is a genetic disorder of the body's ... bones , blood vessels, and organs. What Causes Marfan Syndrome? Marfan syndrome happens because of an abnormality in one ...

  7. Burnout Syndrome of Teachers

    OpenAIRE

    Semrádová, Michaela

    2013-01-01

    The bachelor's thesis covers burnout syndrome of teachers. Defines burnout syndrome, describes its causes and symptoms. Describes teaching as helping profession and focousing on stressful situations at school. In the last chapter described different prevention strategies burnout syndrome. Key words: burnout syndrome, teaching, teacher, helping professions, beginning teacher, stress

  8. Turner Syndrome (For Teens)

    Science.gov (United States)

    ... Staying Safe Videos for Educators Search English Español Turner Syndrome KidsHealth / For Teens / Turner Syndrome What's in this ... en español El síndrome de Turner What Is Turner Syndrome? Turner syndrome (TS) is a genetic condition found ...

  9. Understanding Bartter syndrome and Gitelman syndrome.

    Science.gov (United States)

    Fremont, Oliver T; Chan, James C M

    2012-02-01

    We aim to review the clinical features of two renal tubular disorders characterized by sodium and potassium wasting: Bartter syndrome and Gitelman syndrome. Selected key references concerning these syndromes were analyzed, together with a PubMed search of the literature from 2000 to 2011. The clinical features common to both conditions and those which are distinct to each syndrome were presented. The new findings on the genetics of the five types of Bartter syndrome and the discrete mutations in Gitelman syndrome were reviewed, together with the diagnostic workup and treatment for each condition. Patients with Bartter syndrome types 1, 2 and 4 present at a younger age than classic Bartter syndrome type 3. They present with symptoms, often quite severe in the neonatal period. Patients with classic Bartter syndrome type 3 present later in life and may be sporadically asymptomatic or mildly symptomatic. The severe, steady-state hypokalemia in Bartter syndrome and Gitelman syndrome may abruptly become life-threatening under certain aggravating conditions. Clinicians need to be cognizant of such renal tubular disorders, and promptly treat at-risk patients.

  10. Variants in congenital hypogonadotrophic hypogonadism genes identified in an Indonesian cohort of 46,XY under-virilised boys.

    Science.gov (United States)

    Ayers, Katie L; Bouty, Aurore; Robevska, Gorjana; van den Bergen, Jocelyn A; Juniarto, Achmad Zulfa; Listyasari, Nurin Aisyiyah; Sinclair, Andrew H; Faradz, Sultana M H

    2017-02-16

    Congenital hypogonadotrophic hypogonadism (CHH) and Kallmann syndrome (KS) are caused by disruption to the hypothalamic-pituitary-gonadal (H-P-G) axis. In particular, reduced production, secretion or action of gonadotrophin-releasing hormone (GnRH) is often responsible. Various genes, many of which play a role in the development and function of the GnRH neurons, have been implicated in these disorders. Clinically, CHH and KS are heterogeneous; however, in 46,XY patients, they can be characterised by under-virilisation phenotypes such as cryptorchidism and micropenis or delayed puberty. In rare cases, hypospadias may also be present. Here, we describe genetic mutational analysis of CHH genes in Indonesian 46,XY disorder of sex development patients with under-virilisation. We present 11 male patients with varying degrees of under-virilisation who have rare variants in known CHH genes. Interestingly, many of these patients had hypospadias. We postulate that variants in CHH genes, in particular PROKR2, PROK2, WDR11 and FGFR1 with CHD7, may contribute to under-virilisation phenotypes including hypospadias in Indonesia.

  11. Superior Mesenteric Artery Syndrome or Wilkie Syndrome

    International Nuclear Information System (INIS)

    Castano Llano, Rodrigo; Chams Anturi, Abraham; Arango Vargas, Paula

    2009-01-01

    We described three cases of superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, chronic duodenal ileus, or cast syndrome. This syndrome occurs when the third portion of the duodenum is compressed between the SMA and the aorta. The major risk factors for development of SMA syndrome are rapid weight loss and surgical correction of spinal deformities. The clinical presentation of SMA syndrome is variable and nonspecific, including nausea, vomiting, abdominal pain, and weight loss. The diagnosis is based on endoscopic, radiographic and tomographic findings of duodenal compression by the SMA. The treatment of SMA syndrome is aimed at the precipitating factor, which usually is related to weight loss. Therefore, conservative therapy with nutritional supplementation is the initial approach, and surgery is reserved for those who do not respond to nutritional therapy.

  12. [Syndrome X vs metabolic syndrome].

    Science.gov (United States)

    Morales Villegas, Enrique

    2006-01-01

    Himsworth in 1939 postulated that Diabetes Mellitus type 2 (DM2) was not only an insulin deficiency state but also a cellular insulin insensitivity disease. Thirty years later, DeFronzo and Reaven demonstrated that insulin resistance (IR) preceded and predisposed for DM2 and atherosclerotic-cardiovascular-disease (ACVD). Reaven was the first to point out the relationship between IR and with hyperglycemia, dyslipidosis, and hypertension as mediators for ACVD, creating the concept of Syndrome X (SX) in 1988. WHO and, thereafter, other medical societies and medical groups, mainly ATP-III, in 2002, based on the difficulty of diagnosing IR in a simple, reliable, and inexpensive way, proposed and published the Metabolic Syndrome (MS) concept, as a group of five variables, i.e., obesity, hyperglycemia, hypertriglyceridemia, low HDL, and hypertension, as an easy clinical approximation to suspect and treat an increased cardiometabolic risk. Nowadays, there are deep and extensive controversies on this issue; however, these controversies do not really exist since all discordant points of view are rather quantitative and not qualitative in nature. This article is aimed at differentiating and harmonizing the complementary concepts of SX and MS, at analyzing why MS is a good "clinical window" to look for IR and its underlying manifestations, and finally to accept that the MS concept complements, but does not substitute or antagonize, traditional scales used to asses cardiovascular risk, such as the Framingham scale.

  13. Metabolic Syndrome: Polycystic Ovary Syndrome.

    Science.gov (United States)

    Mortada, Rami; Williams, Tracy

    2015-08-01

    Polycystic ovary syndrome (PCOS) is a heterogeneous condition characterized by androgen excess, ovulatory dysfunction, and polycystic ovaries. It is the most common endocrinopathy among women of reproductive age, affecting between 6.5% and 8% of women, and is the most common cause of infertility. Insulin resistance is almost always present in women with PCOS, regardless of weight, and they often develop diabetes and metabolic syndrome. The Rotterdam criteria are widely used for diagnosis. These criteria require that patients have at least two of the following conditions: hyperandrogenism, ovulatory dysfunction, and polycystic ovaries. The diagnosis of PCOS also requires exclusion of other potential etiologies of hyperandrogenism and ovulatory dysfunction. The approach to PCOS management differs according to the presenting symptoms and treatment goals, particularly the patient's desire for pregnancy. Weight loss through dietary modifications and exercise is recommended for patients with PCOS who are overweight. Oral contraceptives are the first-line treatment for regulating menstrual cycles and reducing manifestations of hyperandrogenism, such as acne and hirsutism. Clomiphene is the first-line drug for management of anovulatory infertility. Metformin is recommended for metabolic abnormalities such as prediabetes, and a statin should be prescribed for cardioprotection if the patient meets standard criteria for statin therapy. Written permission from the American Academy of Family Physicians is required for reproduction of this material in whole or in part in any form or medium.

  14. Syndromes with supernumerary teeth.

    Science.gov (United States)

    Lubinsky, Mark; Kantaputra, Piranit Nik

    2016-10-01

    While most supernumerary teeth are idiopathic, they can be associated with a number of Mendelian syndromes. However, this can also be a coincidental finding, since supernumerary teeth occur in 6% or more of the normal population. To better define this relationship, we analyzed the evidence for specific associations. We excluded conditions with a single affected patient reported, supernumerary teeth adjacent to clefts or other forms of alveolar disruption (as secondary rather than primary findings), and natal teeth, which can involve premature eruption of a normal tooth. Since, the cause of supernumerary teeth shows considerable heterogeneity, certain findings are less likely to be coincidental, such as five or more supernumerary teeth in a single patient, or locations outside of the premaxilla. We found only eight genetic syndromes with strong evidence for an association: cleidocranial dysplasia; familial adenomatous polyposis; trichorhinophalangeal syndrome, type I; Rubinstein-Taybi syndrome; Nance-Horan syndrome; Opitz BBB/G syndrome; oculofaciocardiodental syndrome; and autosomal dominant Robinow syndrome. There is also suggestive evidence of an association with two uncommon disorders, Kreiborg-Pakistani syndrome (craniosynostosis and dental anomalies), and insulin-resistant diabetes mellitus with acanthosisnigricans. An association of a Mendelian disorder with a low frequency manifestation of supernumerary teeth is difficult to exclude without large numbers, but several commonly cited syndromes lacked evidence for clear association, including Hallermann-Streiff syndrome, Fabry disease, Ehlers-Danlos syndrome, Apert and Crouzon syndromes, Zimmermann-Laband syndrome, and Ellis-van Creveld syndrome. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  15. Gorlin's syndrome.

    Science.gov (United States)

    Ramsden, R T; Barrett, A

    1975-06-01

    The uncommon familial syndrome of multiple odontogenic keratocysts, basal cell naevi and skeletal anomalies is reviewed, and seven cases are described, including one patient who developed squamous cell carcinoma in a previous odontogenic keratocyst of the maxilla. We wish to thank Consultants from the Royal National Throat, Nose and Ear Hospital, The Middlesex Hospital and the Eastman Dental Hospital, who allowed us access to their patients; Mr. D. Garfield Davies, Dr. M. F. Spittle, Mr. D. Winstock, Mr. H. P. Cook, Professor H. C. Killey and Mr. L. W. Kay. We are grateful to Professor L. Michaels and Mr. D. J. Connolly for preparation of the illustrations and to Mrs. A. Matthews for the typescript.

  16. HEPATORENAL SYNDROME

    Directory of Open Access Journals (Sweden)

    Matjaž Hafner

    2001-12-01

    Full Text Available Background. Hepatorenal syndrome (HRS is acommon complication of advanced hepatic disease characterizedby marked abnormalities in arterial circulation and byrenal failure. An extreme arteriolar vasodilatation located inthe splanchnic circulation results in a reduction of total systemicvascular resistence and arterial hypotension. Vasoconstrictionoccurs in the renal circulation as in all other extrasplanchnicvascular territories. In the kidney, marked renalvasoconstriction results in a low glomerular filtration rate.Conclusions. The diagnosis of HRS is currently based on exclusionof other causes of renal failure. Prognosis of patientswith HRS is very poor. Liver transplantation is the best therapeuticoption, but it is seldom applicable due to the short survivalexpectancy of most patients with HRS, particularly thosewith the rapidly progressive type of HRS. New therapies developedduring the last few years, such as the use of systemicvasoconstrictors or transjugular intrahepatic portosystemicshunts (TIPS appear promising. Such treatments are of interestnot only as a bridge to liver transplantation but also as atherapy for patients who are not candidates for transplantation.

  17. Noonan syndrome

    Directory of Open Access Journals (Sweden)

    van der Burgt Ineke

    2007-01-01

    Full Text Available Abstract Noonan Syndrome (NS is characterised by short stature, typical facial dysmorphology and congenital heart defects. The incidence of NS is estimated to be between 1:1000 and 1:2500 live births. The main facial features of NS are hypertelorism with down-slanting palpebral fissures, ptosis and low-set posteriorly rotated ears with a thickened helix. The cardiovascular defects most commonly associated with this condition are pulmonary stenosis and hypertrophic cardiomyopathy. Other associated features are webbed neck, chest deformity, mild intellectual deficit, cryptorchidism, poor feeding in infancy, bleeding tendency and lymphatic dysplasias. The syndrome is transmitted as an autosomal dominant trait. In approximately 50% of cases, the disease is caused by missense mutations in the PTPN11 gene on chromosome 12, resulting in a gain of function of the non-receptor protein tyrosine phosphatase SHP-2 protein. Recently, mutations in the KRAS gene have been identified in a small proportion of patients with NS. A DNA test for mutation analysis can be carried out on blood, chorionic villi and amniotic fluid samples. NS should be considered in all foetuses with polyhydramnion, pleural effusions, oedema and increased nuchal fluid with a normal karyotype. With special care and counselling, the majority of children with NS will grow up and function normally in the adult world. Management should address feeding problems in early childhood, evaluation of cardiac function and assessment of growth and motor development. Physiotherapy and/or speech therapy should be offered if indicated. A complete eye examination and hearing evaluation should be performed during the first few years of schooling. Preoperative coagulation studies are indicated. Signs and symptoms lessen with age and most adults with NS do not require special medical care.

  18. Twin Pregnancy Obtention of Patient with Nonmosaic Klinefelter’s Syndrome and His Wife with Chromosome 9 Inversion by ICSI Treatment

    OpenAIRE

    Yueyue Hu; Haiying Peng; Changjun Zhang

    2013-01-01

    A 24-year-old man was diagnosed with klinefelter’s syndrome (KS) and his wife was found to have an inversion on chromosome 9-46, XX, inv (9) (p11q21)- because of infertility. Intracytoplasmic sperm injection (ICSI) was performed for fertilization after fluorescence in-situ hybridization (FISH) was used to analyze the aneuploidy rate of the X and Y chromosomes of the ejaculated sperms of the patient, and 99 sperms were haploid among 100 sperms that were to be analyzed. A twin pregnancy was ach...

  19. Goldenhar Syndrome in Association with Duane Syndrome

    Directory of Open Access Journals (Sweden)

    U D Shrestha

    2012-03-01

    Full Text Available Goldenhar syndrome (GHS is also known as Oculo-Auriculo-Vertebral (OAV syndrome or Branchial arch syndrome. Duane retraction syndrome (DRS is a congenital disorder of ocular motility characterized by limited abduction, adduction or both. It is unilateral in 80% of cases. The important and interesting part of this eight months old child is presence of GHS with DRS. She has bilateral invol-vement, which is seen in only 5-8% of GHS, as compared to high incidence of unilateral involve-ment. This child also had refractive error of + 6.00/ - 1.5 * 180. At four year of age her vision with glass was 6/9. Children with GHS and DRS should have early eye examination done to treat the problem of refractive error. Keywords: Duane retraction syndrome; goldenhar syndrome, refractive error.

  20. The Montreal Cognitive Assessment (MoCA) is superior to the Mini Mental State Examination (MMSE) in detection of Korsakoff's syndrome.

    Science.gov (United States)

    Oudman, Erik; Postma, Albert; Van der Stigchel, Stefan; Appelhof, Britt; Wijnia, Jan W; Nijboer, Tanja C W

    2014-01-01

    The Montreal Cognitive Assessment (MoCA) and Mini Mental State Examination (MMSE) are brief screening instruments for cognitive disorders. Although these instruments have frequently been used in the detection of dementia, there is currently little knowledge on the validity to detect Korsakoff's syndrome (KS) with both screening instruments. KS is a chronic neuropsychiatric disorder associated with profound declarative amnesia after thiamine deficiency. A representative sample of 30 patients with KS and 30 age-, education-, gender- and premorbid-IQ-matched controls was administered the MoCA and MMSE. The area under the receiver operating characteristic curve (AUC) was calculated in addition to the sensitivity, specificity, positive predictive value, and negative predictive value for various cut-off points on the MoCA and MMSE. Compared with the MMSE, the MoCA demonstrated consistently superior psychometric properties and discriminant validity--AUC: MoCA (1.00 SE .003) and MMSE (0.92 SE .033). When applying a cut-off value as suggested in the manuals of both instruments, the MMSE (< 24) misdiagnosed 46.7% of the patients, while the MoCA (< 26) diagnosed all patients correctly. As a screening instrument with the most optimal cut-offs, the MoCA (optimal cutoff point 22/23, 98.3% correctly diagnosed) was superior to the MMSE (optimal cutoff point 26/27, 83.3% correctly diagnosed). We conclude that both tests have adequate psychometric properties as a screening instrument for the detection of KS, but the MoCA is superior to the MMSE for this specific patient population.

  1. Gitelman syndrome

    Directory of Open Access Journals (Sweden)

    Levtchenko Elena N

    2008-07-01

    Full Text Available Abstract Gitelman syndrome (GS, also referred to as familial hypokalemia-hypomagnesemia, is characterized by hypokalemic metabolic alkalosis in combination with significant hypomagnesemia and low urinary calcium excretion. The prevalence is estimated at approximately 1:40,000 and accordingly, the prevalence of heterozygotes is approximately 1% in Caucasian populations, making it one of the most frequent inherited renal tubular disorders. In the majority of cases, symptoms do not appear before the age of six years and the disease is usually diagnosed during adolescence or adulthood. Transient periods of muscle weakness and tetany, sometimes accompanied by abdominal pain, vomiting and fever are often seen in GS patients. Paresthesias, especially in the face, frequently occur. Remarkably, some patients are completely asymptomatic except for the appearance at adult age of chondrocalcinosis that causes swelling, local heat, and tenderness over the affected joints. Blood pressure is lower than that in the general population. Sudden cardiac arrest has been reported occasionally. In general, growth is normal but can be delayed in those GS patients with severe hypokalemia and hypomagnesemia. GS is transmitted as an autosomal recessive trait. Mutations in the solute carrier family12, member 3 gene, SLC12A3, which encodes the thiazide-sensitive NaCl cotransporter (NCC, are found in the majority of GS patients. At present, more than 140 different NCC mutations throughout the whole protein have been identified. In a small minority of GS patients, mutations in the CLCNKB gene, encoding the chloride channel ClC-Kb have been identified. Diagnosis is based on the clinical symptoms and biochemical abnormalities (hypokalemia, metabolic alkalosis, hypomagnesemia and hypocalciuria. Bartter syndrome (especially type III is the most important genetic disorder to consider in the differential diagnosis of GS. Genetic counseling is important. Antenatal diagnosis for GS

  2. Burning Mouth Syndrome and "Burning Mouth Syndrome".

    Science.gov (United States)

    Rifkind, Jacob Bernard

    2016-03-01

    Burning mouth syndrome is distressing to both the patient and practitioner unable to determine the cause of the patient's symptoms. Burning mouth syndrome is a diagnosis of exclusion, which is used only after nutritional deficiencies, mucosal disease, fungal infections, hormonal disturbances and contact stomatitis have been ruled out. This article will explore the many causes and treatment of patients who present with a chief complaint of "my mouth burns," including symptomatic treatment for those with burning mouth syndrome.

  3. Immunoexpression of androgen receptors and aromatase in testes of patient with Klinefelter's syndrome.

    Directory of Open Access Journals (Sweden)

    Stanisław Fracki

    2005-02-01

    Full Text Available Klinefelter's syndrome (47, XXY is the most common chromosome aneuploidy in men and is usually characterized by underdeveloped testes and sterility. The aim of the present study was to detect cellular distribution of androgen receptors (AR and aromatase in testes of patient with KS. The tissue sections were processed for morphological and immunohistochemical staining. Additionally, levels of FSH, LH, PRL, estradiol, and testosterone were measured in the plasma. Morphological analysis revealed a complete absence of spermatogenesis. No germ cells were present in seminiferous tubules. In some tubules, nests of apparently degenerating Sertoli cells were found. In the interstitium, Leydig cell hyperplasia was observed. Using immunohistochemistry, nuclear AR staining was detected in Sertoli cells and peritubular cells, whereas in Leydig cells the staining was exclusively cytoplasmic. The immunostaining of aromatase was detected in the cytoplasm of Sertoli cells and Leydig cells. Increased levels of gonadotropins and decreased level of testosterone concomitantly with the cytoplasmic localization of AR in Leydig cells might contribute to the impaired testicular function in patient with KS.

  4. Hepatorenal Syndrome

    Directory of Open Access Journals (Sweden)

    Pınar Zeyneloğlu

    2012-04-01

    Full Text Available Renal failure is a common major complication in patients with advanced cirrhosis and generally indicates a poor prognosis when combined with liver failure. Hepatorenal syndrome (HRS is characterised by a combination of disturbances in circulatory and kidney function. Arterial pressure is decreased in the systemic circulation due to reduced total systemic vascular resistance. Kidney dysfunction is caused by reduction in renal blood flow. The diagnosis of HRS is based on exclusion of other disorders that cause acute kidney injury in cirrhosis as there are no specific tests. There are two types of HRS with different characteristics and prognostics. Liver transplantation is the treatment of choice for all patients without contraindication. The best approach to the pharmacologic management is the administration vasoconstrictor drugs based on the pathogenesis. Many vasoconstrictors including vasopressin analogues (terlipressin, ornipressin and vasopressin, somatostatin analogues (octreotide and alpha-adrenergic analogues (midodrine and norepinephrine have been studied. In most of the studies intravenous albumin therapy was coadministered with vasoconstrictor drugs and suggested that albumin should be considered as the component of pharmacologic intervention in patients with HRS. Renal replacement therapy in the form of hemodialysis or continuous venovenous hemofiltration has been used in the management of HRS patients awaiting transplantation or in those with acute potentially reversible conditions. The artificial hepatic support systems require further investigation. (Journal of the Turkish Society Intensive Care 2012; 10: 37-44

  5. Pseudohypopituitary syndromes.

    Science.gov (United States)

    Heinze, E; Holl, R W

    1992-07-01

    In a child with short stature, the finding of normal or elevated GH levels in the presence of low concentrations of IGF-I raises the following possibilities. (1) A modification of the GH molecule, which is still detected by RIA, but inactive biologically. Therefore, an RRA or bioassay for hGH should result in considerably lower GH measurements compared with RIA determinations in the same sample. As both bioassays as well as RRAs are not widely available and are hampered by several difficulties, few children with this presumptive diagnosis have been described. So far, it has not been possible to define a specific molecular defect in one of these patients. (2) Abnormalities of the GH receptor or postreceptor mechanisms lead to a GH insensitivity syndrome. Laron-type dwarfism is usually due to a deletion in the gene for hepatic GH receptors: the serum binding protein for GH is absent. In three additional populations, the Pygmies of Zaire, the little women of Loja in Ecuador and the Mountain Ok people in Papua New Guinea, alterations of GH receptor function have been described. Finally, some reports describe patients with normal or elevated serum levels of both growth hormone and IGF-I in whom resistance to IGF has been implied in the pathogenesis of small stature.

  6. Hepatorenal syndrome.

    Science.gov (United States)

    Papper, S

    1980-01-01

    Renal failure without apparent cause (the hepatorenal syndrome) may develop in the course of cirrhosis of the liver. While the development of renal failure bears a poor prognosis, spontaneous recovery can occur. The data suggest that for the most part patients die in rather than of renal failure. The latter seems to be only part of a broader more fundamental disturbance. The pathogenesis of HRS is unknown, but the evidence supports an impairment of effective renal perfusion. The two major hypotheses concerning the nature of the impaired perfusion are that it is a physiologic response to alterations in the extrarenal circulation, and that there is an unidentified humoral agent(s) produced by or inadequately inactivated by or bypassing the diseased liver and causing circulatory changes in the kidney as well as in other organs. It is possible that both mechanisms are operative. Treatment is unsatisfactory and emphasis is presently best placed upon searching for more treatable causes of renal functional impairment in individual patients.

  7. Üks kuningas En-metena raidkiri (En-metena E1.9.5.3.). Kas esimene teadaolev sõprusleping muistses Lähis-Idas? / Vladimir Sazonov

    Index Scriptorium Estoniae

    Sazonov, Vladimir, 1979-

    2015-01-01

    Üks raidkiri varadünastilise perioodi III perioodist on eriline, kuna nimetab "sõprust" või olemuslikult võimalikku "sõpruspakti" kahe Sumeri valitseja vahel. Tegemist on Lagaši kuninga El-metena raidkirjaga. Esmakordselt on tekst avaldatud eesti keeles. Juttu on sõprusest Lagaši kuninga E-metena ja Uruki vürsti Lugal-kiğine-dudu vahel. En-metena isikust. Sündmuste rekontruktsioonist pärast En-metena surma Lagašis 2340. a. paiku e. Kr. Uruki ja Uri valitsejast Lugal-kiğine-dudu'st

  8. Cross-cultural adaptation of the US consumer form of the short Primary Care Assessment Tool (PCAT): the Korean consumer form of the short PCAT (KC PCAT) and the Korean standard form of the short PCAT (KS PCAT).

    Science.gov (United States)

    Jeon, Ki-Yeob

    2011-01-01

    It is well known that countries with well-structured primary care have better health outcomes, better health equity and reduced healthcare costs. This study aimed to culturally modify and validate the US consumer form of the short Primary Care Assessment Tool (PCAT) in primary care in the Republic of Korea (hereafter referred to as Korea). The Korean consumer form of the short PCAT (KC PCAT) was cross-culturally modified from the original version using a standardised transcultural adaptation method. A pre-test version of the KC PCAT was formulated by replacement of four items and modification of a further four items from the 37 items of the original consumer form of the short PCAT at face value evaluation meetings. Pilot testing was done with a convenience sample of 15 responders at two different sites. Test-retest showed high reliability. To validate the KC PCAT, 606 clients participated in a survey carried out in Korea between February and May 2006. Internal consistency reliability, test-retest reliability and factor analysis were conducted in order to test validity. Psychometric testing was carried out on 37 items of the KC PCAT to make the KS PCAT which has 30 items and has seven principal domains: first contact utilisation, first contact accessibility, ongoing accountable care (ongoing care and coordinated rapport care), integrated care (patient-centred care with integration between primary and specialty care or between different specialties), comprehensive care, community-oriented care and culturally-oriented care. Component factors of the verified KS PCAT explained 58.28% of the total variance in the total item scores of primary care. The verified KS PCAT has been characterised by the seven classic domains of primary care with minor modifications. This may provide clues concerning differences in expectations for primary care in the Korean population as compared with that of the US. The KS PCAT is a reliable and valid tool for the evaluation of the quality of

  9. Terlipressin for hepatorenal syndrome

    DEFF Research Database (Denmark)

    Gluud, Lise Lotte; Christensen, Kurt; Christensen, Erik

    2012-01-01

    Clinical trials suggest that terlipressin improves renal function in hepatorenal syndrome, but the evidence concerning mortality is equivocal.......Clinical trials suggest that terlipressin improves renal function in hepatorenal syndrome, but the evidence concerning mortality is equivocal....

  10. Chinese restaurant syndrome

    Science.gov (United States)

    Chinese restaurant syndrome is a set of symptoms that some people have after eating Chinese food. A food additive ... Chinese restaurant syndrome is most often diagnosed based on the symptoms. The health care provider may ask the following ...

  11. Obesity hypoventilation syndrome (OHS)

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000085.htm Obesity hypoventilation syndrome (OHS) To use the sharing features on this page, please enable JavaScript. Obesity hypoventilation syndrome (OHS) is a condition in some ...

  12. Hermansky-Pudlak syndrome

    Directory of Open Access Journals (Sweden)

    Preena A

    2017-04-01

    Full Text Available Hermansky-Pudlak syndrome is a rare autosomal recessive multisystem disease, with oculocutneous albinism, pulmonary fibrosis and bleeding diathesis. Here we report a case of Hermansky-Pudlak syndrome who presented with dyspnea, oculocutaneous albinism and nystagmus.

  13. Marfan syndrome (image)

    Science.gov (United States)

    Marfan syndrome is a disorder of connective tissue which causes skeletal defects typically recognized in a tall, lanky person. A person with Marfan syndrome may exhibit long limbs and spider-like fingers, ...

  14. Acute respiratory distress syndrome

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/000103.htm Acute respiratory distress syndrome To use the sharing features on this page, please enable JavaScript. Acute respiratory distress syndrome (ARDS) is a life-threatening lung ...

  15. Oculoauriculovertebral dysplasia (Goldenhar's syndrome).

    Science.gov (United States)

    Nkrumah, F K

    1971-03-01

    A case of Goldenhar's Syndrome or Oculoauriculovertebral dysplasia in a Ghanaian infant is described. Significant were the additional findings of congenital esophageal atresia and arthrogryposis which have so far not been reported in association with the syndrome.

  16. Guillain-Barre Syndrome

    Science.gov (United States)

    Guillain-Barre syndrome is a rare disorder that causes your immune system to attack your peripheral nervous system (PNS). The PNS ... your brain. No one knows what causes the syndrome. Sometimes it is triggered by an infection, surgery, ...

  17. Carpal Tunnel Syndrome

    Science.gov (United States)

    ... a passing cramp? It could be carpal tunnel syndrome. The carpal tunnel is a narrow passageway of ... three times more likely to have carpal tunnel syndrome than men. Early diagnosis and treatment are important ...

  18. Polycystic Ovary Syndrome

    Science.gov (United States)

    Polycystic ovary syndrome (PCOS) happens when a woman's ovaries or adrenal glands produce more male hormones than normal. PCOS causes cysts ( ... PCOS are at higher risk of diabetes, metabolic syndrome, heart disease, and high blood pressure. PCOS is ...

  19. Hyperimmunoglobulin E syndrome

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/001311.htm Hyperimmunoglobulin E syndrome To use the sharing features on this page, please enable JavaScript. Hyperimmunoglobulin E syndrome is a rare, inherited disease. It causes ...

  20. Holmes-Adie Syndrome

    Science.gov (United States)

    ... other diseases of the nervous system, such as Sjogren’s syndrome or migraine. It is most often seen in ... other diseases of the nervous system, such as Sjogren’s syndrome or migraine. It is most often seen in ...