SAS doctors career progression survey 2013.

PubMed

Oroz, Carlos; Sands, Lorna R; Lee, John

2016-03-01

We conducted a national survey of Staff, Associate Specialists and Specialty (SAS) doctors working in sexual health clinics in the UK in 2013 in order to explore their career progression. The aim of the survey was to assess SAS doctors' experience in passing through the thresholds and to gather information about the adherence by SAS doctors and employers to the terms and conditions of service laid out by the new 2008 contract. Out of 185 responders, whom the authors estimate comprise 34% of the total workforce, 159 were on the new contract. Of those, most SAS doctors were women (84%), the majority (67%) worked less than nine programmed activities per week; only a few had intentions to join the consultant grade (15%), and a considerable minority (26%) were older than 54 years of age and likely to retire in the next ten years. The survey showed that most participating SAS doctors had undergone appraisal in the previous 15 months (90%), most had a job planning discussion (83%) with their employer and most had some allocated time for supporting professional activities (86%). However, a significant minority had no appraisal (10%), no job planning discussion (17%) and had no allocated supporting professional activities (14%), which allows time for career development in the specialty. Most SAS doctors, who had the opportunity, had progressed through the thresholds automatically (88%); some experienced difficulties in passing (8%) and only a few did not pass (4%). SAS doctors must ensure that they work together with their employer in order to improve adherence to the terms and conditions of service of the contract, which allow for career progression and benefit both the individual doctors and ultimately service provision. © The Author(s) 2015.

The Spectrum Analysis Solution (SAS) System: Theoretical Analysis, Hardware Design and Implementation.

PubMed

Narayanan, Ram M; Pooler, Richard K; Martone, Anthony F; Gallagher, Kyle A; Sherbondy, Kelly D

2018-02-22

This paper describes a multichannel super-heterodyne signal analyzer, called the Spectrum Analysis Solution (SAS), which performs multi-purpose spectrum sensing to support spectrally adaptive and cognitive radar applications. The SAS operates from ultrahigh frequency (UHF) to the S-band and features a wideband channel with eight narrowband channels. The wideband channel acts as a monitoring channel that can be used to tune the instantaneous band of the narrowband channels to areas of interest in the spectrum. The data collected from the SAS has been utilized to develop spectrum sensing algorithms for the budding field of spectrum sharing (SS) radar. Bandwidth (BW), average total power, percent occupancy (PO), signal-to-interference-plus-noise ratio (SINR), and power spectral entropy (PSE) have been examined as metrics for the characterization of the spectrum. These metrics are utilized to determine a contiguous optimal sub-band (OSB) for a SS radar transmission in a given spectrum for different modalities. Three OSB algorithms are presented and evaluated: the spectrum sensing multi objective (SS-MO), the spectrum sensing with brute force PSE (SS-BFE), and the spectrum sensing multi-objective with brute force PSE (SS-MO-BFE).

The Spectrum Analysis Solution (SAS) System: Theoretical Analysis, Hardware Design and Implementation

PubMed Central

Pooler, Richard K.; Martone, Anthony F.; Gallagher, Kyle A.; Sherbondy, Kelly D.

2018-01-01

This paper describes a multichannel super-heterodyne signal analyzer, called the Spectrum Analysis Solution (SAS), which performs multi-purpose spectrum sensing to support spectrally adaptive and cognitive radar applications. The SAS operates from ultrahigh frequency (UHF) to the S-band and features a wideband channel with eight narrowband channels. The wideband channel acts as a monitoring channel that can be used to tune the instantaneous band of the narrowband channels to areas of interest in the spectrum. The data collected from the SAS has been utilized to develop spectrum sensing algorithms for the budding field of spectrum sharing (SS) radar. Bandwidth (BW), average total power, percent occupancy (PO), signal-to-interference-plus-noise ratio (SINR), and power spectral entropy (PSE) have been examined as metrics for the characterization of the spectrum. These metrics are utilized to determine a contiguous optimal sub-band (OSB) for a SS radar transmission in a given spectrum for different modalities. Three OSB algorithms are presented and evaluated: the spectrum sensing multi objective (SS-MO), the spectrum sensing with brute force PSE (SS-BFE), and the spectrum sensing multi-objective with brute force PSE (SS-MO-BFE). PMID:29470448

Adolescent adaptive behavior profiles in Williams-Beuren syndrome, Down syndrome, and autism spectrum disorder.

PubMed

Del Cole, Carolina Grego; Caetano, Sheila Cavalcante; Ribeiro, Wagner; Kümmer, Arthur Melo E E; Jackowski, Andrea Parolin

2017-01-01

Adaptive behavior can be impaired in different neurodevelopmental disorders and may be influenced by confounding factors, such as intelligence quotient (IQ) and socioeconomic classification. Our main objective was to verify whether adaptive behavior profiles differ in three conditions-Williams Beuren syndrome (WBS), Down syndrome (DS), and autism spectrum disorder (ASD), as compared with healthy controls (HC) and with each other. Although the literature points towards each disorder having a characteristic profile, no study has compared profiles to establish the specificity of each one. A secondary objective was to explore potential interactions between the conditions and socioeconomic status, and whether this had any effect on adaptive behavior profiles. One hundred and five adolescents were included in the study. All adolescents underwent the following evaluations: the Vineland Adaptive Behavior Scale (VABS), the Wechsler Intelligence Scale for Children (WISC), and the Brazilian Economic Classification Criteria. Our results demonstrated that the WBS group performed better than the DS group in the communication domain, β = -15.08, t(3.45), p = .005, and better than the ASD group in the socialization domain, β = 8.92, t(-2.08), p = .013. The DS group also performed better than the ASD group in socialization, β = 16.98, t(-2.32), p = .024. IQ was an important confounding factor, and socioeconomic status had an important effect on the adaptive behavior of all groups. There is a heterogeneity regarding adaptive behavior profiles in WBS, DS, and ASD. These data are important to better design specific strategies related to the health and social care of each particular group.

Behavior and adaptive functioning in adolescents with Down syndrome: specifying targets for intervention.

PubMed

Jacola, Lisa M; Hickey, Francis; Howe, Steven R; Esbensen, Anna; Shear, Paula K

2014-01-01

Research suggests that adolescents with Down syndrome experience increased behavior problems as compared to age matched peers; however, few studies have examined how these problems relate to adaptive functioning. The primary aim of this study was to characterize behavior in a sample of adolescents with Down syndrome using two widely-used caregiver reports: the Behavioral Assessment System for Children, 2 nd Edition (BASC-2) and Child Behavioral Checklist (CBCL). The clinical utility of the BASC-2 as a measure of behavior and adaptive functioning in adolescents with Down syndrome was also examined. Fifty-two adolescents with Down syndrome between the ages of 12 and 18 (24 males) completed the Peabody Picture Vocabulary Test, 4 th Edition (PPVT-IV) as an estimate of cognitive ability. Caregivers completed the BASC-2 and the CBCL for each participant. A significant proportion of the sample was reported to demonstrate behavior problems, particularly related to attention and social participation. The profile of adaptive function was variable, with caregivers most frequently rating impairment in skills related to activities of daily living and functional communication. Caregiver ratings did not differ by gender and were not related to age or estimated cognitive ability. Caregiver ratings of attention problems on the BASC-2 accounted for a significant proportion of variance in Activities of Daily Living ( Adj R 2 = 0.30) , Leadership ( Adj R 2 = 0.30) Functional Communication ( Adj R 2 = 0.28, Adaptability ( Adj R 2 = 0.29), and Social Skills ( Adj R 2 = 0.17). Higher frequencies of symptoms related to social withdrawal added incremental predictive validity for Functional Communication, Leadership, and Social Skills. Convergent validity between the CBCL and BASC-2 was poor when compared with expectations based on the normative sample. Our results confirm and extend previous findings by describing relationships between specific behavior problems and targeted areas of

Behavior and adaptive functioning in adolescents with Down syndrome: specifying targets for intervention

PubMed Central

Jacola, Lisa M.; Hickey, Francis; Howe, Steven R.; Esbensen, Anna; Shear, Paula K.

2016-01-01

Background Research suggests that adolescents with Down syndrome experience increased behavior problems as compared to age matched peers; however, few studies have examined how these problems relate to adaptive functioning. The primary aim of this study was to characterize behavior in a sample of adolescents with Down syndrome using two widely-used caregiver reports: the Behavioral Assessment System for Children, 2nd Edition (BASC-2) and Child Behavioral Checklist (CBCL). The clinical utility of the BASC-2 as a measure of behavior and adaptive functioning in adolescents with Down syndrome was also examined. Methods Fifty-two adolescents with Down syndrome between the ages of 12 and 18 (24 males) completed the Peabody Picture Vocabulary Test, 4th Edition (PPVT-IV) as an estimate of cognitive ability. Caregivers completed the BASC-2 and the CBCL for each participant. Results A significant proportion of the sample was reported to demonstrate behavior problems, particularly related to attention and social participation. The profile of adaptive function was variable, with caregivers most frequently rating impairment in skills related to activities of daily living and functional communication. Caregiver ratings did not differ by gender and were not related to age or estimated cognitive ability. Caregiver ratings of attention problems on the BASC-2 accounted for a significant proportion of variance in Activities of Daily Living (Adj R2 = 0.30), Leadership (Adj R2 = 0.30) Functional Communication (Adj R2 = 0.28, Adaptability (Adj R2 = 0.29), and Social Skills (Adj R2 = 0.17). Higher frequencies of symptoms related to social withdrawal added incremental predictive validity for Functional Communication, Leadership, and Social Skills. Convergent validity between the CBCL and BASC-2 was poor when compared with expectations based on the normative sample. Conclusion Our results confirm and extend previous findings by describing relationships between specific behavior problems

Effects of Germline Mutations in the Ras/MAPK Signaling Pathway on Adaptive Behavior: Cardiofaciocutaneous Syndrome and Noonan Syndrome

PubMed Central

Pierpont, Elizabeth I.; Pierpont, Mary Ella; Mendelsohn, Nancy J.; Roberts, Amy E.; Tworog-Dube, Erica; Rauen, Katherine A.; Seidenberg, Mark S.

2011-01-01

Cardiofaciocutaneous syndrome (CFC) and Noonan syndrome (NS) are two phenotypically overlapping genetic disorders whose underlying molecular etiologies affect a common signaling pathway. Mutations in the BRAF, MEK1 and MEK2 genes cause most cases of CFC and mutations in PTPN11, SOS1, KRAS and RAF1 typically cause NS. Although both syndromes are associated with developmental delays of varying severity, the extent to which the behavioral profiles differ may shed light on the different roles these respective genes play in development of skills necessary for everyday functioning. In this study, profiles of adaptive behavior of individuals with CFC and NS who had confirmed pathogenic mutations in Ras/MAPK pathway genes were investigated. Patterns of strengths and weaknesses, age-related differences, and risk factors for difficulties in adaptive skills were assessed. Although genes acting more downstream in the Ras/MAPK pathway were associated with more difficulties in adaptive functioning than genes more upstream in the pathway, several inconsistencies highlight the wide spectrum of possible developmental courses in CFC and NS. Along with clinical and genetic factors, variables such as chronological age, gestational age at birth and parental education levels accounted for significant variance in adaptive skills. Results indicate that there is wide heterogeneity in adaptive ability in CFC and NS, but that these abilities are correlated to some extent with the specific disease-causing genes. PMID:20186801

Intestinal adaptation in short bowel syndrome: A case report.

PubMed

Palla, Viktoria-Varvara; Karaolanis, Georgios; Pentazos, Panagiotis; Ladopoulos, Alexios; Papageorgiou, Evaggelos

2015-06-01

Short bowel syndrome is a clinical entity that includes loss of energy, fluid, electrolytes or micronutrient balance because of inadequate functional intestinal length. This case report demonstrates the case of a woman who compensated for short bowel syndrome through intestinal adaptation, which is a complex process worthy of further investigation for the avoidance of dependence on total parenteral nutrition and of intestinal transplantation in such patients. Copyright © 2015 Arab Journal of Gastroenterology. Published by Elsevier B.V. All rights reserved.

Adaptive Functioning in Williams Syndrome: A Systematic Review

ERIC Educational Resources Information Center

Brawn, Gabrielle; Porter, Melanie

2018-01-01

Literature on the level of adaptive functioning and relative strengths and weaknesses in functioning of individuals with Williams syndrome (WS) was reviewed. The electronic databases PsycINFO, PubMed, Expanded Academic, Web of Science, Scopus and ProQuest were searched electronically for relevant articles and dissertations using the search terms…

Anomalous brain functional connectivity contributing to poor adaptive behavior in Down syndrome.

PubMed

Pujol, Jesus; del Hoyo, Laura; Blanco-Hinojo, Laura; de Sola, Susana; Macià, Dídac; Martínez-Vilavella, Gerard; Amor, Marta; Deus, Joan; Rodríguez, Joan; Farré, Magí; Dierssen, Mara; de la Torre, Rafael

2015-03-01

Research in Down syndrome has substantially progressed in the understanding of the effect of gene overexpression at the molecular level, but there is a paucity of information on the ultimate consequences on overall brain functional organization. We have assessed the brain functional status in Down syndrome using functional connectivity MRI. Resting-state whole-brain connectivity degree maps were generated in 20 Down syndrome individuals and 20 control subjects to identify sites showing anomalous synchrony with other areas. A subsequent region-of-interest mapping served to detail the anomalies and to assess their potential contribution to poor adaptive behavior. Down syndrome individuals showed higher regional connectivity in a ventral brain system involving the amygdala/anterior temporal region and the ventral aspect of both the anterior cingulate and frontal cortices. By contrast, lower functional connectivity was identified in dorsal executive networks involving dorsal prefrontal and anterior cingulate cortices and posterior insula. Both functional connectivity increases and decreases contributed to account for patient scoring on adaptive behavior related to communication skills. The data overall suggest a distinctive functional organization with system-specific anomalies associated with reduced adaptive efficiency. Opposite effects were identified on distinct frontal and anterior temporal structures and relative sparing of posterior brain areas, which is generally consistent with Down syndrome cognitive profile. Relevantly, measurable connectivity changes, as a marker of the brain functional anomaly, could have a role in the development of therapeutic strategies addressed to improve the quality of life in Down syndrome individuals. Copyright © 2014 Elsevier Ltd. All rights reserved.

Adaptive Skills, Behavior Problems, and Parenting Stress in Mothers of Boys with Fragile X Syndrome

ERIC Educational Resources Information Center

Sarimski, Klaus

2010-01-01

The relationship of temperament, atypical behaviors, and adaptive behavior of young boys with Fragile X syndrome on mothers' parenting stress was analyzed. Twenty-six boys with Fragile X syndrome (30-88 months of age) participated. The overall development of the participants was significantly delayed with a specific profile of adaptive behaviors…

Primary Molecular Disorders and Secondary Biological Adaptations in Bartter Syndrome

PubMed Central

Deschênes, Georges; Fila, Marc

2011-01-01

Bartter syndrome is a hereditary disorder that has been characterized by the association of hypokalemia, alkalosis, and the hypertrophy of the juxtaglomerular complex with secondary hyperaldosteronism and normal blood pressure. By contrast, the genetic causes of Bartter syndrome primarily affect molecular structures directly involved in the sodium reabsorption at the level of the Henle loop. The ensuing urinary sodium wasting and chronic sodium depletion are responsible for the contraction of the extracellular volume, the activation of the renin-aldosterone axis, the secretion of prostaglandins, and the biological adaptations of downstream tubular segments, meaning the distal convoluted tubule and the collecting duct. These secondary biological adaptations lead to hypokalemia and alkalosis, illustrating a close integration of the solutes regulation in the tubular structures. PMID:21941653

The Mexican Sistema de Alerta Sismica (SAS) Experience

NASA Astrophysics Data System (ADS)

Espinosa-Aranda, J.; Rodriguez, F.

2003-12-01

The Mexico City's SAS is an Early Warning System developed in accordance with recommendations of foreign and national seismology experts, after the disaster generated in 1985 by the M8.1 Michoacan earthquake. The SAS aim is to help mitigate future seismic disasters in Mexico City (MC), mainly if the big forecast "Guerrero Earthquake" hits. In 1991 the SAS started its service in an experimental basis for evaluating its performance both with a group of basic education students in public schools, and with the emergency stop function for the Mexico City Metropolitan Subway System (METRO of MC); after this The Federal District Government (FDG) opened the SAS as a public service in August, 1993, shortly after the SAS warned, with more than 60 seconds of anticipation, the incoming effect of May 13, 1993, M5.8, and M6 double earthquake originated more than 300 Km from MC between Guerrero and Oaxaca, on the coast. That year, an initial technical flaw generated one false alert signal; the cause was readily corrected. The SAS has 12 seismic sensor stations covering a stripe close to 400 X 100 Km on the Guerrero's coast, between Papanoa and Punta Maldonado, that send information through a dedicated radio relay system to the SAS Central Control in MC., using the valuable support of some TELMEX field installations. Until August, 2003, the SAS sensor system has detected more than 1550 earthquakes in the 2.55 magnitude events, which are the ones felt in MC. The SAS has generated 57 warning signals with an average of 60 sec in advance to earthquake effects: 46 of restricted use for M<6, and 11 of public use for M>6 events. Public warnings have the automatic broadcast support of many commercial radio AM/FM and TV stations in Mexico City and Toluca valleys. The FDG controls the SAS signal service that includes the more frequent events (from M>5 up), asking the user to develop procedures and practice useful actions to mitigate seismic

The autistic phenotype in Down syndrome: differences in adaptive behaviour versus Down syndrome alone and autistic disorder alone.

PubMed

Dressler, Anastasia; Perelli, Valentina; Bozza, Margherita; Bargagna, Stefania

2011-01-01

The autistic phenotype in Down syndrome (DS) is marked by a characteristic pattern of stereotypies, anxiety and social withdrawal. Our aim was to study adaptive behaviour in DS with and without autistic comorbidity using the Vineland Adaptive Behaviour Scales (VABS), the Childhood Autism Rating Scales (CARS) and the DSM IV-TR criteria. We assessed 24 individuals and established three groups: Down syndrome (DS), DS and autistic disorder (DS-AD), and autistic disorder (AD). The DS and DS-AD groups showed statistically significantly similar strengths on the VABS (in receptive and domestic skills). The DS and DS-AD subjects also showed similar strengths on the CARS (in imitation and relating), differing significantly from the AD group. The profile of adaptive functioning and symptoms in DS-AD seemed to be more similar to that found in DS than to the profile emerging in AD. We suggest that the comorbidity of austistic symptoms in DS hampered the acquisition of adaptive skills more than did the presence of DS alone.

The autistic phenotype in Down syndrome: differences in adaptive behaviour versus Down syndrome alone and autistic disorder alone

PubMed Central

Dressler, Anastasia; Perelli, Valentina; Bozza, Margherita; Bargagna, Stefania

Summary The autistic phenotype in Down syndrome (DS) is marked by a characteristic pattern of stereotypies, anxiety and social withdrawal. Our aim was to study adaptive behaviour in DS with and without autistic comorbidity using the Vineland Adaptive Behaviour Scales (VABS), the Childhood Autism Rating Scales (CARS) and the DSM IV-TR criteria. We assessed 24 individuals and established three groups: Down syndrome (DS), DS and autistic disorder (DS-AD), and autistic disorder (AD). The DS and DS-AD groups showed statistically significantly similar strengths on the VABS (in receptive and domestic skills). The DS and DS-AD subjects also showed similar strengths on the CARS (in imitation and relating), differing significantly from the AD group. The profile of adaptive functioning and symptoms in DS-AD seemed to be more similar to that found in DS than to the profile emerging in AD. We suggest that the comorbidity of austistic symptoms in DS hampered the acquisition of adaptive skills more than did the presence of DS alone. PMID:22152436

Adaptation in families of children with Down syndrome in East Asian countries: an integrative review.

PubMed

Choi, Hyunkyung; Van Riper, Marcia

2017-08-01

The purpose of this integrative literature review was to understand the experiences of East Asian families of children with Down syndrome and identify factors affecting their adaptation in the Resiliency Model of Family Stress, Adjustment and Adaptation. Socio-cultural factors influence how well families adapt following the birth of a child with Down syndrome. Existing literature in this area has focused primarily on families from Western cultures. This is problematic because nurses care for families from all over the world. Therefore, the focus of this review is on families of children with Down syndrome living in East Asia, where Confucianism is dominant. Integrative literature review. Online databases (i.e. PubMed, CINAHL and PsycINFO) and a public search engine (i.e. Google Scholar) were used along with manual searches of reference lists and major journals. Studies were limited to original publications written in English and published between 1990-2014. Two authors independently performed integrative review processes proposed by Whittemore and Knafl and a quality assessment using the Mixed Methods Appraisal Tool. Like families in Western cultures, some East Asian families of children with Down syndrome adapted well and even thrived while others struggled. Various socio-cultural factors, including some associated with Confucianism, played a role in how individuals, dyads and families adapted. An understanding of socio-cultural influences can help nurses implement culturally sensitive family-centred interventions with families of children with Down syndrome. It may also facilitate policy changes concerning resources for these families. © 2016 John Wiley & Sons Ltd.

The SAS Education Value-Added Assessment System (SAS[R] EVAAS[R]) in the Houston Independent School District (HISD): Intended and Unintended Consequences

ERIC Educational Resources Information Center

Amrein-Beardsley, Audrey; Collins, Clarin

2012-01-01

The SAS Educational Value-Added Assessment System (SAS[R] EVAAS[R]) is the most widely used value-added system in the country. It is also self-proclaimed as "the most robust and reliable" system available, with its greatest benefit to help educators improve their teaching practices. This study critically examined the effects of SAS[R] EVAAS[R] as…

A SAS IML Macro for Loglinear Smoothing

ERIC Educational Resources Information Center

Moses, Tim; von Davier, Alina

2011-01-01

Polynomial loglinear models for one-, two-, and higher-way contingency tables have important applications to measurement and assessment. They are essentially regarded as a smoothing technique, which is commonly referred to as loglinear smoothing. A SAS IML (SAS Institute, 2002a) macro was created to implement loglinear smoothing according to…

A Review of PROC IRT in SAS

ERIC Educational Resources Information Center

Choi, Jinnie

2017-01-01

This article reviews PROC IRT, which was added to Statistical Analysis Software in 2014. We provide an introductory overview of a free version of SAS, describe what PROC IRT offers for item response theory (IRT) analysis and how one can use PROC IRT, and discuss how other SAS macros and procedures may compensate the IRT functionalities of PROC IRT.

Hemiparesis is a clinical correlate of general adaptive dysfunction in children and adolescents with Sturge-Weber syndrome.

PubMed

Reesman, Jennifer; Gray, Robert; Suskauer, Stacy J; Ferenc, Lisa M; Kossoff, Eric H; Lin, Doris D M; Turin, Elizabeth; Comi, Anne M; Brice, Patrick J; Zabel, T Andrew

2009-06-01

This study sought to identify neurologic correlates of adaptive functioning in individuals with Sturge-Weber syndrome. A total of 18 children, adolescents, and young adults with Sturge-Weber syndrome with brain involvement were recruited from our Sturge-Weber center. All underwent neurologic examination (including review of clinical brain magnetic resonance imaging) and neuropsychological assessment. Neuropsychological assessment included measures of intellectual ability and standardized parent report of adaptive functioning. Overall, Full Scale IQ and ratings of global adaptive functioning were both lower than the population-based norms (P < .05). Negative correlations were identified between adaptive functioning ratings, clinician ratings of cortical abnormality, and ratings of neurologic status. Hemiparesis (minimal versus prominent) was the only individual component of the rating scales that differentiated between individuals with nonimpaired and impaired adaptive functioning scores. Information obtained during neurological examination of children and adolescents with Sturge-Weber syndrome particularly hemiparetic status is useful for identifying children who may need additional intervention.

The School Anxiety Scale-Teacher Report (SAS-TR): translation and psychometric properties of the Iranian version.

PubMed

Hajiamini, Zahra; Mohamadi, Ashraf; Ebadi, Abbas; Fathi- Ashtiani, Ali; Tavousi, Mahmoud; Montazeri, Ali

2012-07-18

The School Anxiety Scale-Teacher Report (SAS-TR) was designed to assess anxiety in children at school. The SAS-TR is a proxy rated measure and could assess social anxiety, generalized anxiety and also gives a total anxiety score. This study aimed to translate and validate the SAS-TR in Iran. The translation and cultural adaptation of the original questionnaire were carried out in accordance with the published guidelines. A sample of students participated in the study. Reliability was estimated using internal consistency and test-retest analysis. Validity was assessed using content validity. The factor structure of the questionnaire was extracted by performing both exploratory and confirmatory factor analyses. In all 200 elementary students aged 6 to 10 years were studied. Considering the recommended cut-off values, overall the prevalence of high anxiety condition in elementary students was found to be 21 %. Cronbach's alpha coefficient for the Iranian SAS-TR was 0.92 and intraclass correlation coefficient (ICC) was found to be 0.81. The principal component analysis indicated a two-factor structure for the questionnaire (generalized and social anxiety) that jointly accounted for 55.3 % of variances observed. The confirmatory factory analysis also indicated a good fit to the data for the two-latent structure of the questionnaire. In general the findings suggest that the Iranian version of SAS-TR has satisfactory reliability, and validity for measuring anxiety in 6 to 10 years old children in Iran. It is simple and easy to use and now can be applied in future studies.

Leisure Activities in Prader-Wlli Syndrome: Implications for Health, Cognition and Adaptive Functioning

ERIC Educational Resources Information Center

Dykens, Elisabeth M.

2014-01-01

Although hyperphagia and compulsivity in Prader-Willi syndrome (PWS) are well described, recreation and adaptive skills are relatively unexplored. Parents of 123 participants with PWS (4--48 years) completed measures of their child's adaptive, recreation, and problem behaviors. Offspring received cognitive testing. Watching TV was the most…

INTERFACING SAS TO ORACLE IN THE UNIX ENVIRONMENT

EPA Science Inventory

SAS is an EPA standard data and statistical analysis software package while ORACLE is EPA's standard data base management system software package. RACLE has the advantage over SAS in data retrieval and storage capabilities but has limited data and statistical analysis capability....

Adaptation to transient postural perturbations

NASA Technical Reports Server (NTRS)

Andres, Robert O.

1992-01-01

This research was first proposed in May, 1986, to focus on some of the problems encountered in the analysis of postural responses gathered from crewmembers. The ultimate driving force behind this line of research was the desire to treat, predict, or explain 'Space Adaptation Syndrome' (SAS) and hence circumvent any adverse effects of space motion sickness on crewmember performance. The aim of this project was to develop an easily implemented analysis of the transient responses to platform translation that can be elicited with a protocol designed to force sensorimotor reorganization, utilizing statistically reliable criterion measures. This report will present: (1) a summary of the activity that took place in each of the three funded years of the project; (2) discussion of experimental results and their implications for future research; and (3) a list of presentations and publications resulting from this project.

Validity of Simpson-Angus Scale (SAS) in a naturalistic schizophrenia population.

PubMed

Janno, Sven; Holi, Matti M; Tuisku, Katinka; Wahlbeck, Kristian

2005-03-17

Simpson-Angus Scale (SAS) is an established instrument for neuroleptic-induced parkinsonism (NIP), but its statistical properties have been studied insufficiently. Some shortcomings concerning its content have been suggested as well. According to a recent report, the widely used SAS mean score cut-off value 0.3 of for NIP detection may be too low. Our aim was to evaluate SAS against DSM-IV diagnostic criteria for NIP and objective motor assessment (actometry). Ninety-nine chronic institutionalised schizophrenia patients were evaluated during the same interview by standardised actometric recording and SAS. The diagnosis of NIP was based on DSM-IV criteria. Internal consistency measured by Cronbach's alpha, convergence to actometry and the capacity for NIP case detection were assessed. Cronbach's alpha for the scale was 0.79. SAS discriminated between DSM-IV NIP and non-NIP patients. The actometric findings did not correlate with SAS. ROC-analysis yielded a good case detection power for SAS mean score. The optimal threshold value of SAS mean score was between 0.65 and 0.95, i.e. clearly higher than previously suggested threshold value. We conclude that SAS seems a reliable and valid instrument. The previously commonly used cut-off mean score of 0.3 has been too low resulting in low specificity, and we suggest a new cut-off value of 0.65, whereby specificity could be doubled without loosing sensitivity.

Reduced orexin immunoreactivity in Perry syndrome and multiple system atrophy.

PubMed

Mishima, Takayasu; Kasanuki, Koji; Koga, Shunsuke; Castanedes-Casey, Monica; Wszolek, Zbigniew K; Tsuboi, Yoshio; Dickson, Dennis W

2017-09-01

Orexin is a neuropeptide that plays a key role in maintaining a state of arousal, and possibly associates with sleep apnea syndrome (SAS). Reduced orexin immunoreactivity has been reported in various neurologic conditions such as narcolepsy, Alzheimer's disease, Lewy body disease and multiple system atrophy (MSA); however, there has been no report investigating orexin in Perry syndrome, a rare hereditary neurodegenerative disease characterized by four clinical cardinal signs (parkinsonism, depression/apathy, weight loss, and central hypoventilation). Perry syndrome patients frequently have sleep disturbances, including SAS and insomnia. We evaluated orexin immunoreactivity in Perry syndrome. Using imaging analysis, we quantitatively assessed orexin immunoreactivity in the nucleus basalis of Meynert in three Perry syndrome cases, as well as five cases of frontotemporal lobar degeneration with motor neuron disease, five cases of MSA and five age-matched controls. For these cases, antemortem clinical information on sleep disturbances has been reviewed. In Perry syndrome and MSA, there was reduction of orexin immunoreactivity compared with controls (Perry syndrome: p = 0.020, MSA: p < 0.001). In contrast, FTLD-MND did not have significant reduction of orexin immunoreactivity. Two out of three cases of Perry syndrome had SAS confirmed by polysomnography. This is the first report assessing orexin immunoreactivity in Perry syndrome, and it showed significant reduction, similar to select neurodegenerative diseases, such as MSA. Further analysis with more cases will be needed to elucidate the specific mechanism of orexin loss in these disorders. Copyright © 2017 Elsevier Ltd. All rights reserved.

Validity of Simpson-Angus Scale (SAS) in a naturalistic schizophrenia population

PubMed Central

Janno, Sven; Holi, Matti M; Tuisku, Katinka; Wahlbeck, Kristian

2005-01-01

Background Simpson-Angus Scale (SAS) is an established instrument for neuroleptic-induced parkinsonism (NIP), but its statistical properties have been studied insufficiently. Some shortcomings concerning its content have been suggested as well. According to a recent report, the widely used SAS mean score cut-off value 0.3 of for NIP detection may be too low. Our aim was to evaluate SAS against DSM-IV diagnostic criteria for NIP and objective motor assessment (actometry). Methods Ninety-nine chronic institutionalised schizophrenia patients were evaluated during the same interview by standardised actometric recording and SAS. The diagnosis of NIP was based on DSM-IV criteria. Internal consistency measured by Cronbach's α, convergence to actometry and the capacity for NIP case detection were assessed. Results Cronbach's α for the scale was 0.79. SAS discriminated between DSM-IV NIP and non-NIP patients. The actometric findings did not correlate with SAS. ROC-analysis yielded a good case detection power for SAS mean score. The optimal threshold value of SAS mean score was between 0.65 and 0.95, i.e. clearly higher than previously suggested threshold value. Conclusion We conclude that SAS seems a reliable and valid instrument. The previously commonly used cut-off mean score of 0.3 has been too low resulting in low specificity, and we suggest a new cut-off value of 0.65, whereby specificity could be doubled without loosing sensitivity. PMID:15774006

Development and validation of a smartphone addiction scale (SAS).

PubMed

Kwon, Min; Lee, Joon-Yeop; Won, Wang-Youn; Park, Jae-Woo; Min, Jung-Ah; Hahn, Changtae; Gu, Xinyu; Choi, Ji-Hye; Kim, Dai-Jin

2013-01-01

The aim of this study was to develop a self-diagnostic scale that could distinguish smartphone addicts based on the Korean self-diagnostic program for Internet addiction (K-scale) and the smartphone's own features. In addition, the reliability and validity of the smartphone addiction scale (SAS) was demonstrated. A total of 197 participants were selected from Nov. 2011 to Jan. 2012 to accomplish a set of questionnaires, including SAS, K-scale, modified Kimberly Young Internet addiction test (Y-scale), visual analogue scale (VAS), and substance dependence and abuse diagnosis of DSM-IV. There were 64 males and 133 females, with ages ranging from 18 to 53 years (M = 26.06; SD = 5.96). Factor analysis, internal-consistency test, t-test, ANOVA, and correlation analysis were conducted to verify the reliability and validity of SAS. Based on the factor analysis results, the subscale "disturbance of reality testing" was removed, and six factors were left. The internal consistency and concurrent validity of SAS were verified (Cronbach's alpha = 0.967). SAS and its subscales were significantly correlated with K-scale and Y-scale. The VAS of each factor also showed a significant correlation with each subscale. In addition, differences were found in the job (p<0.05), education (p<0.05), and self-reported smartphone addiction scores (p<0.001) in SAS. This study developed the first scale of the smartphone addiction aspect of the diagnostic manual. This scale was proven to be relatively reliable and valid.

PROC IRT: A SAS Procedure for Item Response Theory

PubMed Central

Matlock Cole, Ki; Paek, Insu

2017-01-01

This article reviews the procedure for item response theory (PROC IRT) procedure in SAS/STAT 14.1 to conduct item response theory (IRT) analyses of dichotomous and polytomous datasets that are unidimensional or multidimensional. The review provides an overview of available features, including models, estimation procedures, interfacing, input, and output files. A small-scale simulation study evaluates the IRT model parameter recovery of the PROC IRT procedure. The use of the IRT procedure in Statistical Analysis Software (SAS) may be useful for researchers who frequently utilize SAS for analyses, research, and teaching.

The Longitudinal Effects of Parenting on Adaptive Behavior in Children with Fragile X Syndrome

ERIC Educational Resources Information Center

Warren, Steven F.; Brady, Nancy; Fleming, Kandace K.; Hahn, Laura J.

2017-01-01

Several studies have reported declines in adaptive behavior amongst children with fragile X syndrome (FXS) starting in middle childhood. We examined the effects of maternal responsivity on adaptive behavior in 55 children with FXS visited 5-6 times in their homes from early through middle childhood. Our analyses indicated that sustained maternal…

First report of sasX-positive methicillin-resistant Staphylococcus aureus in Japan.

PubMed

Nakaminami, Hidemasa; Ito, Teruyo; Han, Xiao; Ito, Ayumu; Matsuo, Miki; Uehara, Yuki; Baba, Tadashi; Hiramatsu, Keiichi; Noguchi, Norihisa

2017-09-01

SasX is a known virulence factor of Staphylococcus aureus involved in colonisation and immune evasion of the bacterium. The sasX gene, which is located on the ϕSPβ prophage, is frequently found in the sequence type (ST) 239 S. aureus lineage, which is the predominant healthcare-associated clone in Asian countries. In Japan, ST239 clones have rarely been identified, and sasX-positive strains have not been reported to date. Here, we report the first identification of 18 sasX-positive methicillin-resistant S. aureus (MRSA) strains in Japanese hospitals between 2009 and 2011. All sasX-positive isolates belonged to an ST239-staphylococcal cassette chromosome mec type III (ST239-III) lineage. However, we were unable to identify additional sasX-positive MRSA strains from 2012 to 2016, indicating that the small epidemic of sasX-positive isolates observed in this study was temporary. The sequence surrounding sasX in the strain TOHH628 lacked 51 genes that encode phage packaging and structural proteins, and no bacteriophage was induced by mitomycin C. Additionally, in the TOHH628 strain, the region (64.6 kb) containing sasX showed high identity to the ϕSPβ-like element (71.3 kb) of the Taiwanese MRSA strain Z172. The data strongly suggest that the present sasX-positive isolates found in Japanese hospitals were transmitted incidentally from other countries. © FEMS 2017. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Development and Validation of a Smartphone Addiction Scale (SAS)

PubMed Central

Kwon, Min; Lee, Joon-Yeop; Won, Wang-Youn; Park, Jae-Woo; Min, Jung-Ah; Hahn, Changtae; Gu, Xinyu; Choi, Ji-Hye; Kim, Dai-Jin

2013-01-01

Objective The aim of this study was to develop a self-diagnostic scale that could distinguish smartphone addicts based on the Korean self-diagnostic program for Internet addiction (K-scale) and the smartphone's own features. In addition, the reliability and validity of the smartphone addiction scale (SAS) was demonstrated. Methods A total of 197 participants were selected from Nov. 2011 to Jan. 2012 to accomplish a set of questionnaires, including SAS, K-scale, modified Kimberly Young Internet addiction test (Y-scale), visual analogue scale (VAS), and substance dependence and abuse diagnosis of DSM-IV. There were 64 males and 133 females, with ages ranging from 18 to 53 years (M = 26.06; SD = 5.96). Factor analysis, internal-consistency test, t-test, ANOVA, and correlation analysis were conducted to verify the reliability and validity of SAS. Results Based on the factor analysis results, the subscale “disturbance of reality testing” was removed, and six factors were left. The internal consistency and concurrent validity of SAS were verified (Cronbach's alpha = 0.967). SAS and its subscales were significantly correlated with K-scale and Y-scale. The VAS of each factor also showed a significant correlation with each subscale. In addition, differences were found in the job (p<0.05), education (p<0.05), and self-reported smartphone addiction scores (p<0.001) in SAS. Conclusions This study developed the first scale of the smartphone addiction aspect of the diagnostic manual. This scale was proven to be relatively reliable and valid. PMID:23468893

[Hemodynamics, the autonomic nervous system and water metabolism as criteria for developing the general adaptation syndrome in pregnant women].

PubMed

Gur'ianov, V A; Shepetovskaia, N L; Pivovarova, G M; Tolmachev, G N; Volodin, A V

2007-01-01

By taking into account the fact that the autonomic nervous and cardiovascular systems (ANS and CVS) are the major links of development of the general adaptation syndrome in pregnancy, which are affected by all the processes involved in the development of the syndrome, the author analyzed the state of these systems in healthy non-pregnant and pregnant women (HNPW and HPW) and in pregnant women with gestosis. HNPW were found to have already a prerequisite for impairing pregnancy adaptive processes as ANS and CVS dysfunction. In HPW, these impairments were more pronounced. In the pregnant women, impaired adaptive processes manifested themselves as excess sympathicotonia in 72% and parasympathicotonia in 23% of cases despite the treatment performed, which was accompanied by hypokinetic hemodynamics in 53 and 50%, respectively. In hyper- and eukinetic hemodynamics, there were no physiologically required decreases in total peripheral vascular resistance while in hypokinetic hemodynamics, there was its pathological increase. Such disorders enhance the significance of abdominal compartment syndrome, aortocaval compression, ischemia-reperfusion, hydrodynamic and membranogenic (capillary leakage) factors of impaired water metabolism, which contributes to adaptation derangement. Based on the findings, the authors have created a developmental modulation algorithm for the general adaptation syndrome by completed pregnancy and surgical delivery.

Sir- and silencer-independent disruption of silencing in Saccharomyces by Sas10p.

PubMed

Kamakaka, R T; Rine, J

1998-06-01

A promoter fusion library of Saccharomyces cerevisiae genes was used to exploit phenotypes associated with altered protein dosage. We identified a novel gene, SAS10, by the ability of Sas10p, when overproduced, to disrupt silencing. The predicted Sas10p was 70,200 kD and strikingly rich in charged amino acids. Sas10p was exclusively nuclear in all stages of the cell cycle. Overproduction of Sas10p caused derepression of mating type genes at both HML and HMR, as well as of URA3, TRP1, and ADE2 when inserted near a telomere or at HMR or the rDNA locus. Repressed genes not associated with silenced chromatin were unaffected. Sas10p was essential for viability, and the termination point following Sas10p depletion was as large budded cells. Remarkably, Sas10p overproduction disrupted silencing even under conditions that bypassed the requirement for Sir proteins, ORC, and Rap1p in silencing. These data implied that Sas10p function was intimately connected with the structure of silenced chromatin.

Structures of SAS-6 suggest its organization in centrioles.

PubMed

van Breugel, Mark; Hirono, Masafumi; Andreeva, Antonina; Yanagisawa, Haru-aki; Yamaguchi, Shoko; Nakazawa, Yuki; Morgner, Nina; Petrovich, Miriana; Ebong, Ima-Obong; Robinson, Carol V; Johnson, Christopher M; Veprintsev, Dmitry; Zuber, Benoît

2011-03-04

Centrioles are cylindrical, ninefold symmetrical structures with peripheral triplet microtubules strictly required to template cilia and flagella. The highly conserved protein SAS-6 constitutes the center of the cartwheel assembly that scaffolds centrioles early in their biogenesis. We determined the x-ray structure of the amino-terminal domain of SAS-6 from zebrafish, and we show that recombinant SAS-6 self-associates in vitro into assemblies that resemble cartwheel centers. Point mutations are consistent with the notion that centriole formation in vivo depends on the interactions that define the self-assemblies observed here. Thus, these interactions are probably essential to the structural organization of cartwheel centers.

Treatment of complex sleep apnea syndrome: a retrospective comparative review.

PubMed

Pusalavidyasagar, Snigdha S; Olson, Eric J; Gay, Peter C; Morgenthaler, Timothy I

2006-09-01

Some patients with obstructive sleep apnea syndrome (OSAS) develop problematic central apneas or Cheyne-Stokes pattern with acute application of continuous positive airway pressure (CPAP), herein called complex sleep apnea syndrome (CompSAS). This response makes it difficult to be certain that CPAP will be a successful treatment strategy. We sought to compare treatments between patients with CompSAS vs. OSAS and hypothesized that CompSAS patients would find CPAP less effective and have more problems with adherence than patients with OSAS. We performed a retrospective review of patients studied in our sleep disorders center over 1 month. There were 133 patients with OSAS (mean age=57.6+/-12.2 years; males=63.9%) and 34 with CompSAS (mean age=54.4+/-16 years; males=82.35%). CPAP was prescribed in 93.7 and 87.9% of OSAS and CompSAS patients, respectively (P=0.284), with no significant difference in required CPAP pressures (P=0.112). There was no difference in prescription frequency of alternative therapies. Mean time to the first follow-up was shorter in CompSAS patients (46.2+/-47.3 vs. 53.8+/-36.8 days; P=0.022). CPAP compliance in OSAS and CompSAS patients (5.1+/-1.6 vs. 6.1+/-1.5h, P=0.156) and improvement in Epworth Sleepiness Scale (ESS) (-4.6+/-4.8 vs. -5.9+/-6.9, P=0.483) was similar. However, interface problems were more common in CompSAS patients, especially air hunger/dyspnea (0.8 vs. 8.8%) and inadvertent mask removal (2.6 vs. 17.7%) (all P<0.050). CompSAS patients have more CPAP interface problems and require more follow-up than OSAS patients but with intervention may have similar treatment results compared to patients with OSAS.

Recrystallization of fluconazole using the supercritical antisolvent (SAS) process.

PubMed

Park, Hee Jun; Kim, Min-Soo; Lee, Sibeum; Kim, Jeong-Soo; Woo, Jong-Soo; Park, Jeong-Sook; Hwang, Sung-Joo

2007-01-10

The supercritical antisolvent (SAS) process was used to modify solid state characteristics of fluconazole. Fluconazole was recrystallized at various temperatures (60-80 degrees C) and pressures (8-16MPa) using dichloromethane (DCM) as a solvent. Acetone and ethanol were also employed as solvents. The fluconazole polymorphs prepared by the SAS process were characterized by differential scanning calorimetry (DSC), thermogravimetry analysis (TGA), powder X-ray diffraction (PXRD), Fourier transform infrared spectroscopy (FT-IR) and scanning electron microscopy (SEM). Furthermore, the equilibrium solubility of the samples in aqueous solution was determined. Fluconazole anhydrate form I was obtained at low temperature (40 degrees C) and anhydrate form II was obtained at high temperature (80 degrees C). The variation of pressure during the SAS process may influence the preferred orientation. Anhydrate forms I and II were also obtained using various solvents. Therefore, it was shown that solid state characteristics of fluconazole, including the polymorphic form and preferred orientation, can be controlled by changing operating conditions of the SAS process such as temperature, pressure, and solvent.

SATB2-associated syndrome presenting with Rett-like phenotypes.

PubMed

Lee, J S; Yoo, Y; Lim, B C; Kim, K J; Choi, M; Chae, J-H

2016-06-01

The SATB2-associated syndrome (SAS) was proposed recently, after the SATB2 gene was initially discovered to be associated with isolated cleft palate. This syndrome is characterized by intellectual disability with delayed speech development, facial dysmorphism, cleft or high-arched palate, and dentition problems. Here, we describe two novel SATB2 sequence variants in two unrelated patients presenting with Rett-like phenotypes. We performed trio-based whole-exome sequencing in a 17-month-old girl presenting with severe retardation and Rett-like phenotypes, which revealed a de novo missense variant in SATB2 (p.Glu396Gln). Moreover, targeted sequencing of the SATB2 gene was performed in a 2-year-old girl with severe psychomotor retardation, facial hypotonia, and cleft palate who also exhibited some features of Rett syndrome. A nonsense variant in SATB2 was identified in this patient (p.Arg459*). This study expanded the clinical and genetic spectrum of SAS. SATB2 variants should be considered in cases with psychomotor retardation alone or in any cases with Rett-like phenotypes, regardless of the typical features of SAS such as cleft palate. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Family functioning mediates adaptation in caregivers of individuals with Rett syndrome.

PubMed

Lamb, Amanda E; Biesecker, Barbara B; Umstead, Kendall L; Muratori, Michelle; Biesecker, Leslie G; Erby, Lori H

2016-11-01

The objective of this study was to investigate factors related to family functioning and adaptation in caregivers of individuals with Rett syndrome (RS). A cross-sectional quantitative survey explored the relationships between demographics, parental self-efficacy, coping methods, family functioning and adaptation. A forward-backward, step-wise model selection procedure was used to evaluate variables associated with both family functioning and adaptation. Analyses also explored family functioning as a mediator of the relationship between other variables and adaptation. Bivariate analyses (N=400) revealed that greater parental self-efficacy, a greater proportion of problem-focused coping, and a lesser proportion of emotion-focused coping were associated with more effective family functioning. In addition, these key variables were significantly associated with greater adaptation, as was family functioning, while controlling for confounders. Finally, regression analyses suggest family functioning as a mediator of the relationships between three variables (parental self-efficacy, problem-focused coping, and emotion-focused coping) with adaptation. This study demonstrates the potentially predictive roles of expectations and coping methods and the mediator role of family functioning in adaptation among caregivers of individuals with RS, a chronic developmental disorder. A potential target for intervention is strengthening of caregiver competence in the parenting role to enhance caregiver adaptation. Published by Elsevier Ireland Ltd.

SAS FORMATS: USES AND ABUSES

EPA Science Inventory

SAS formats are a very powerful tool. They allow you to display the data in a more readable manner without modifying the data. They can also be used to group data into categories for use in various procedures like PROC FREQ, PROC TTEST, and PROC MEANS (as a class variable). ...

SAS FORMATS: USES AND ABUSES

EPA Science Inventory

SAS formats are a very powerful tool. They allow you to display the data in a more readable manner without modifying it. Formats can also be used to group data into categories for use in various procedures like PROC FREQ, PROC TTEST, and PROC MEANS (as a class variable). As ...

SAS FORMATS: USES AND ABUSES

EPA Science Inventory

SAS formats are a very powerful tool. They allow you to display the data in a more readable manner without modifying it. Formats can also be used to group data into categories for use in various procedures like PROC FREQ, PROC TTEST, and PROC MEANS (as a class variable). As w...

[Gastroesophageal reflux and obstructive sleep apnea syndrome].

PubMed

Esteller, E; Modolell, I; Segarra, F; Matiño, E; Enrique, A; Ademà, J M; Estivill, E

2005-11-01

Patients with mild or moderate Sleep Apnea Syndrome (SAS) need wider therapeutic scope options according to their disease severity. To consider including proton pump inhibitors (PPI) to the therapeutical alternatives of these patients. A prospective study was designed, among patients with SAS. Nocturnal polysomnography and double channel pHmetry were performed simultaneously. From the 18 patients included in this preliminary phase, in three (16.7%) nocturnal proximal ph monitoring was positive. These 3 patients were treated with PPI during at least 3 months with a very satisfactory outcome in two of them. Treatment with PPI may be a useful therapeutical alternative in patients with mild to moderate SAS.

Continuous positive airway pressure intolerance associated with elevated nasal resistance is possible mechanism of complex sleep apnea syndrome.

PubMed

Nakazaki, Chie; Noda, Akiko; Yasuda, Yoshinari; Nakata, Seiichi; Koike, Yasuo; Yasuma, Fumihiko; Murohara, Toyoaki; Nakashima, Tsutomu

2012-09-01

Complex sleep apnea syndrome (CompSAS) is diagnosed after an elimination of obstructive events with continuous positive airway pressure (CPAP), when a central apnea index ≥5/h or Cheyne-Stokes respiration pattern emerges in patients with obstructive sleep apnea syndrome (OSAS). However, the pathophysiology of CompSAS remains controversial. Of the 281 patients with suspected OSAS, all of whom underwent polysomnography conducted at Nagoya University Hospital, we enrolled 52 patients with apnea-hypopnea index ≥15/h (age 51.4 ± 13.3 years). The polysomnographic findings, left ventricular ejection fraction (LVEF), and nasal resistance were compared between the CompSAS patients and OSAS patients. Forty-three patients were diagnosed with OSAS and nine patients with central sleep apnea syndrome by natural sleep PSG. Furthermore, 43 OSAS patients were classified into the OSAS patients (OSAS group, n = 38) and the CompSAS patients (CompSAS group, n = 5) by the night on CPAP PSG. The nasal resistance was significantly higher in CompSAS group than in OSAS group (0.30 ± 0.10 vs. 0.19 ± 0.07 Pa/cm(3)/s, P = 0.004). The arousal index, percentage of stage 1 sleep, and oxygen desaturation index were significantly decreased, and the percentage of stage REM sleep was significantly increased in the OSAS group with the initial CPAP treatment, but not in the CompSAS group. In addition, the patients with CompSAS showed normal LVEF. CPAP intolerance secondary to an elevated nasal resistance might relate to frequent arousals, which could presumably contribute to an increase in central sleep apnea. Further evaluation in a large study is needed to clarify the mechanism of CompSAS.

Social Anxiety Scale for Adolescents (SAS-A) Short Form.

PubMed

Nelemans, Stefanie A; Meeus, Wim H J; Branje, Susan J T; Van Leeuwen, Karla; Colpin, Hilde; Verschueren, Karine; Goossens, Luc

2017-01-01

In this study, we examined the longitudinal measurement invariance of a 12-item short version of the Social Anxiety Scale for Adolescents (SAS-A) in two 4-year longitudinal community samples ( N sample 1 = 815, M age T 1 = 13.38 years; N sample 2 = 551, M age T 1 = 14.82 years). Using confirmatory factor analyses, we found strict longitudinal measurement invariance for the three-factor structure of the SAS-A across adolescence, across samples, and across gender. Some developmental changes in social anxiety were found from early to mid-adolescence, as well as gender differences across adolescence. These findings suggest that the short version of the SAS-A is a developmentally appropriate instrument that can be used effectively to examine adolescent social anxiety development.

[Tracheobronchial prosthesis in Mounier-Kuhn syndrome: New perspectives].

PubMed

Sauvage, M; Tiffet, O; Vergnon, J-M

2015-05-01

Mounier-Kuhn syndrome or tracheobronchomegaly is a rare congenital condition, the management of which is complex. We report the case of a patient who was treated with interventional endoscopy. We describe the case of a 74-year-old man with a diagnosis of tracheobronchomegaly who was admitted in 2003 with a background of deteriorating respiratory status and the occurrence of postural syncope. He initially received a tracheobronchial silicone Y prosthesis, extended with metal prostheses at the tracheal and bronchial level. This arrangement remained stable until 2011. He then began to develop episodes of asphyxia related to posterior dislocation of the tracheobronchial prosthesis, after breakage of the metallic mesh tracheal prosthesis. A new tracheobronchial prosthesis Y was then placed, custom-made from a 3D model of the airways. This was clinically and functionally effective. This case describes the management of a patient with Mounier-Kuhn syndrome by interventional bronchoscopy, with the adaptation of prosthetic materials, on an individual basis, to the anatomy of the patient's airway. Copyright © 2015 SPLF. Published by Elsevier Masson SAS. All rights reserved.

Extension of the sasCIF format and its applications for data processing and deposition

DOE PAGES

Kachala, Michael; Westbrook, John; Svergun, Dmitri

2016-02-01

Recent advances in small-angle scattering (SAS) experimental facilities and data analysis methods have prompted a dramatic increase in the number of users and of projects conducted, causing an upsurge in the number of objects studied, experimental data available and structural models generated. To organize the data and models and make them accessible to the community, the Task Forces on SAS and hybrid methods for the International Union of Crystallography and the Worldwide Protein Data Bank envisage developing a federated approach to SAS data and model archiving. Within the framework of this approach, the existing databases may exchange information and providemore » independent but synchronized entries to users. At present, ways of exchanging information between the various SAS databases are not established, leading to possible duplication and incompatibility of entries, and limiting the opportunities for data-driven research for SAS users. In this work, a solution is developed to resolve these issues and provide a universal exchange format for the community, based on the use of the widely adopted crystallographic information framework (CIF). The previous version of the sasCIF format, implemented as an extension of the core CIF dictionary, has been available since 2000 to facilitate SAS data exchange between laboratories. The sasCIF format has now been extended to describe comprehensively the necessary experimental information, results and models, including relevant metadata for SAS data analysis and for deposition into a database. Processing tools for these files (sasCIFtools) have been developed, and these are available both as standalone open-source programs and integrated into the SAS Biological Data Bank, allowing the export and import of data entries as sasCIF files. Software modules to save the relevant information directly from beamline data-processing pipelines in sasCIF format are also developed. Lastly, this update of sasCIF and the relevant tools are

Leisure activities in Prader-Wili syndrome: implications for health, cognition and adaptive functioning.

PubMed

Dykens, Elisabeth M

2014-02-01

Although hyperphagia and compulsivity in Prader-Willi syndrome (PWS) are well described, recreation and adaptive skills are relatively unexplored. Parents of 123 participants with PWS (4-48 years) completed measures of their child's adaptive, recreation, and problem behaviors. Offspring received cognitive testing. Watching TV was the most frequent recreational activity, and was associated with compulsivity and skin picking. BMIs were negatively correlated with physical play, and highest in those who watched TV and played computer games. Computer games and physical activities were associated with higher IQ and adaptive scores. People with PWS and other disabilities need to watch less TV and be more engaged in physical activities, games, and leisure pursuits that are fun, and may bring cognitive or adaptive advantages.

Pharmacological interventions to improve cognition and adaptive functioning in Down syndrome: Strides to date.

PubMed

Hart, Sarah J; Visootsak, Jeannie; Tamburri, Paul; Phuong, Patrick; Baumer, Nicole; Hernandez, Maria-Clemencia; Skotko, Brian G; Ochoa-Lubinoff, Cesar; Liogier D'Ardhuy, Xavier; Kishnani, Priya S; Spiridigliozzi, Gail A

2017-11-01

Although an increasing number of clinical trials have been developed for cognition in Down syndrome, there has been limited success to date in identifying effective interventions. This review describes the progression from pre-clinical studies with mouse models to human clinical trials research using pharmacological interventions to improve cognition and adaptive functioning in Down syndrome. We also provide considerations for investigators when conducting human clinical trials and describe strategies for the pharmaceutical industry to advance the field in drug discovery for Down syndrome. Future research focusing on earlier pharmaceutical interventions, development of appropriate outcome measures, and greater collaboration between industry, academia, advocacy, and regulatory groups will be important for addressing limitations from prior studies and developing potential effective interventions for cognition in Down syndrome. © 2017 Wiley Periodicals, Inc.

Evolving experience with prevention and treatment of splenic artery syndrome after orthotopic liver transplantation.

PubMed

Mogl, Martina T; Nüssler, Natascha C; Presser, Sabine J; Podrabsky, Petr; Denecke, Timm; Grieser, Christian; Neuhaus, Peter; Guckelberger, Olaf

2010-08-01

Impaired hepatic arterial perfusion after orthotopic liver transplantation (OLT) may lead to ischemic biliary tract lesions and graft-loss. Hampered hepatic arterial blood flow is observed in patients with hypersplenism, often described as arterial steal syndrome (ASS). However, arterial and portal perfusions are directly linked via the hepatic arterial buffer response (HABR). Recently, the term 'splenic artery syndrome' (SAS) was coined to describe the effect of portal hyperperfusion leading to diminished hepatic arterial blood flow. We retrospectively analyzed 650 transplantations in 585 patients. According to preoperative imaging, 78 patients underwent prophylactic intraoperative ligation of the splenic artery. In case of postoperative SAS, coil-embolization of the splenic artery was performed. After exclusion of 14 2nd and 3rd retransplantations and 83 procedures with arterial interposition grafts, SAS was diagnosed in 28 of 553 transplantations (5.1%). Twenty-six patients were treated with coil-embolization, leading to improved liver function, but requiring postinterventional splenectomy in two patients. Additionally, two patients with SAS underwent splenectomy or retransplantation without preceding embolization. Prophylactic ligation could not prevent SAS entirely (n = 2), but resulted in a significantly lower rate of complications than postoperative coil-embolization. We recommend prophylactic ligation of the splenic artery for patients at risk of developing SAS. Post-transplant coil-embolization of the splenic artery corrected hemodynamic changes of SAS, but was associated with a significant morbidity.

[Effect of glutamine and growth hormone on adaptation in short bowel syndrome].

PubMed

Wu, Guo-hao; Wu, Zhao-han; Wu, Zhao-guang

2005-09-01

To assess the effects of parenteral glutamine and growth hormone supplementation on gut adaptation for patients with short bowel syndrome. Twenty-six patients [male 15, female 11, aged (39 +/- 23) years] with short bowel syndrome received parenteral nutrition (PN) 3-52 months after surgical resection. The median length of remnant small intestine was 42.5(0-100)cm. All patients received growth hormone (0.10+/- 0.06) mg.kg(-1).d(-1) plus glutamine (0.30 +/- 0.17) mg.kg(-1).d(-1) for two or three weeks. Among the 26 patients, PN was not required soon after treatment in 34.6% (n=9) of the patients, the frequency and volume of PN decreased from (6.0 +/- 1.0) d to (4.2 +/- 1.0) d, from (13.6 +/- 5.2) L per week to (8.2 +/- 3.3) L per week respectively in 30.8% (n=8) of the patients,while 34.6% (n=9) still required PN after treatment. The combined administration of glutamine and growth hormone can promote remnant intestinal adaptation in short bowel patients.

Behavior and Adaptive Functioning in Adolescents with Down Syndrome: Specifying Targets for Intervention

ERIC Educational Resources Information Center

Jacola, Lisa M.; Hickey, Francis; Howe, Steven R.; Esbensen, Anna; Shear, Paula K.

2014-01-01

Adolescents with Down syndrome can demonstrate increased behavior problems as compared with typical peers. Few studies have explored whether behavior impacts adaptive functioning. Caregiver report from the Behavioral Assessment System for Children, 2nd Edition (BASC-2; Reynolds & Kamphaus, 2004) and the Child Behavioral Checklist (CBCL;…

Factors associated with adaptation to Klinefelter syndrome: The experience of adolescents and adults

PubMed Central

Turriff, Amy; Levy, Howard P.; Biesecker, Barbara

2016-01-01

Objective The purpose of this study was to understand the impact of living with Klinefelter syndrome (XXY) as an adolescent or an adult and to examine the factors that contribute to adaptation. Methods Individuals (n = 310) aged 14–75 years with self-reported XXY were recruited from online support networks to complete a self-administered survey. Perceived consequences, perceived severity, perceived stigma, and coping were measured and evaluated as correlates of adaptation. Results The use of problem-focused coping strategies was positively correlated with adaptation (p < 0.01) and age was negatively correlated with adaptation (p < 0.05). Conclusion The majority of participants reported significant negative consequences of XXY, including infertility, psychological co-morbidities and differences in appearance. How participants coped with their negative appraisals was the greatest predictor of adaptation. Practice implications Interventions designed to help individuals reframe negative appraisals, to increase perceived manageability of the challenges of living with XXY, and to facilitate effective coping may improve adaptation among individuals with XXY. PMID:25239793

Dynamic event tree analysis with the SAS4A/SASSYS-1 safety analysis code

DOE PAGES

Jankovsky, Zachary K.; Denman, Matthew R.; Aldemir, Tunc

2018-02-02

The consequences of a transient in an advanced sodium-cooled fast reactor are difficult to capture with the traditional approach to probabilistic risk assessment (PRA). Numerous safety-relevant systems are passive and may have operational states that cannot be represented by binary success or failure. In addition, the specific order and timing of events may be crucial which necessitates the use of dynamic PRA tools such as ADAPT. The modifications to the SAS4A/SASSYS-1 sodium-cooled fast reactor safety analysis code for linking it to ADAPT to perform a dynamic PRA are described. A test case is used to demonstrate the linking process andmore » to illustrate the type of insights that may be gained with this process. Finally, newly-developed dynamic importance measures are used to assess the significance of reactor parameters/constituents on calculated consequences of initiating events.« less

Dynamic event tree analysis with the SAS4A/SASSYS-1 safety analysis code

DOE Office of Scientific and Technical Information (OSTI.GOV)

Jankovsky, Zachary K.; Denman, Matthew R.; Aldemir, Tunc

The consequences of a transient in an advanced sodium-cooled fast reactor are difficult to capture with the traditional approach to probabilistic risk assessment (PRA). Numerous safety-relevant systems are passive and may have operational states that cannot be represented by binary success or failure. In addition, the specific order and timing of events may be crucial which necessitates the use of dynamic PRA tools such as ADAPT. The modifications to the SAS4A/SASSYS-1 sodium-cooled fast reactor safety analysis code for linking it to ADAPT to perform a dynamic PRA are described. A test case is used to demonstrate the linking process andmore » to illustrate the type of insights that may be gained with this process. Finally, newly-developed dynamic importance measures are used to assess the significance of reactor parameters/constituents on calculated consequences of initiating events.« less

Longitudinal Trajectories of Intellectual and Adaptive Functioning in Adolescents and Adults with Williams Syndrome

ERIC Educational Resources Information Center

Fisher, M. H.; Lense, M. D.; Dykens, E. M.

2016-01-01

Background: Williams syndrome (WS) is associated with a distinct cognitive-behavioural phenotype including mild to moderate intellectual disability, visual-spatial deficits, hypersociability, inattention and anxiety. Researchers typically characterise samples of individuals with WS by their intellectual functioning and adaptive behaviour. Because…

[Adaptive optics for ophthalmology].

PubMed

Saleh, M

2016-04-01

Adaptive optics is a technology enhancing the visual performance of an optical system by correcting its optical aberrations. Adaptive optics have already enabled several breakthroughs in the field of visual sciences, such as improvement of visual acuity in normal and diseased eyes beyond physiologic limits, and the correction of presbyopia. Adaptive optics technology also provides high-resolution, in vivo imaging of the retina that may eventually help to detect the onset of retinal conditions at an early stage and provide better assessment of treatment efficacy. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

SAS-6 engineering reveals interdependence between cartwheel and microtubules in determining centriole architecture.

PubMed

Hilbert, Manuel; Noga, Akira; Frey, Daniel; Hamel, Virginie; Guichard, Paul; Kraatz, Sebastian H W; Pfreundschuh, Moritz; Hosner, Sarah; Flückiger, Isabelle; Jaussi, Rolf; Wieser, Mara M; Thieltges, Katherine M; Deupi, Xavier; Müller, Daniel J; Kammerer, Richard A; Gönczy, Pierre; Hirono, Masafumi; Steinmetz, Michel O

2016-04-01

Centrioles are critical for the formation of centrosomes, cilia and flagella in eukaryotes. They are thought to assemble around a nine-fold symmetric cartwheel structure established by SAS-6 proteins. Here, we have engineered Chlamydomonas reinhardtii SAS-6-based oligomers with symmetries ranging from five- to ten-fold. Expression of a SAS-6 mutant that forms six-fold symmetric cartwheel structures in vitro resulted in cartwheels and centrioles with eight- or nine-fold symmetries in vivo. In combination with Bld10 mutants that weaken cartwheel-microtubule interactions, this SAS-6 mutant produced six- to eight-fold symmetric cartwheels. Concurrently, the microtubule wall maintained eight- and nine-fold symmetries. Expressing SAS-6 with analogous mutations in human cells resulted in nine-fold symmetric centrioles that exhibited impaired length and organization. Together, our data suggest that the self-assembly properties of SAS-6 instruct cartwheel symmetry, and lead us to propose a model in which the cartwheel and the microtubule wall assemble in an interdependent manner to establish the native architecture of centrioles.

Sas-4 proteins are required during basal body duplication in Paramecium

PubMed Central

Gogendeau, Delphine; Hurbain, Ilse; Raposo, Graca; Cohen, Jean; Koll, France; Basto, Renata

2011-01-01

Centrioles and basal bodies are structurally related organelles composed of nine microtubule (MT) triplets. Studies performed in Caenorhabditis elegans embryos have shown that centriole duplication takes place in sequential way, in which different proteins are recruited in a specific order to assemble a procentriole. ZYG-1 initiates centriole duplication by triggering the recruitment of a complex of SAS-5 and SAS-6, which then recruits the final player, SAS-4, to allow the incorporation of MT singlets. It is thought that a similar mechanism (that also involves additional proteins) is present in other animal cells, but it remains to be investigated whether the same players and their ascribed functions are conserved during basal body duplication in cells that exclusively contain basal bodies. To investigate this question, we have used the multiciliated protist Paramecium tetraurelia. Here we show that in the absence of PtSas4, two types of defects in basal body duplication can be identified. In the majority of cases, the germinative disk and cartwheel, the first structures assembled during duplication, are not detected. In addition, if daughter basal bodies were formed, they invariably had defects in MT recruitment. Our results suggest that PtSas4 has a broader function than its animal orthologues. PMID:21289083

SAS-6 assembly templated by the lumen of cartwheel-less centrioles precedes centriole duplication.

PubMed

Fong, Chii Shyang; Kim, Minhee; Yang, T Tony; Liao, Jung-Chi; Tsou, Meng-Fu Bryan

2014-07-28

Centrioles are 9-fold symmetric structures duplicating once per cell cycle. Duplication involves self-oligomerization of the centriolar protein SAS-6, but how the 9-fold symmetry is invariantly established remains unclear. Here, we found that SAS-6 assembly can be shaped by preexisting (or mother) centrioles. During S phase, SAS-6 molecules are first recruited to the proximal lumen of the mother centriole, adopting a cartwheel-like organization through interactions with the luminal wall, rather than via their self-oligomerization activity. The removal or release of luminal SAS-6 requires Plk4 and the cartwheel protein STIL. Abolishing either the recruitment or the removal of luminal SAS-6 hinders SAS-6 (or centriole) assembly at the outside wall of mother centrioles. After duplication, the lumen of engaged mother centrioles becomes inaccessible to SAS-6, correlating with a block for reduplication. These results lead to a proposed model that centrioles may duplicate via a template-based process to preserve their geometry and copy number. Copyright © 2014 Elsevier Inc. All rights reserved.

SAS-6 assembly templated by the lumen of cartwheel-less centrioles precedes centriole duplication

PubMed Central

Fong, Chii Shyang; Kim, Minhee; Yang, T. Tony; Liao, Jung-Chi; Tsou, Meng-Fu Bryan

2014-01-01

SUMMARY Centrioles are 9-fold symmetric structures duplicating once per cell cycle. Duplication involves self-oligomerization of the centriolar protein SAS-6, but how the 9-fold symmetry is invariantly established remains unclear. Here, we found that SAS-6 assembly can be shaped by preexisting (or mother) centrioles. During S phase, SAS-6 molecules are first recruited to the proximal lumen of the mother centriole, adopting a cartwheel-like organization through interactions with the luminal wall, rather than via their self-oligomerization activity. The removal or release of luminal SAS-6 requires Plk4 and the cartwheel protein STIL. Abolishing either the recruitment or the removal of luminal SAS-6 hinders SAS-6 (or centriole) assembly at the outside wall of mother centrioles. After duplication, the lumen of engaged mother centrioles becomes inaccessible to SAS-6, correlating with a block for re-duplication. These results lead to a proposed model that centrioles may duplicate via a template-based process to preserve their geometry and copy number. PMID:25017693

Adaptive behavior in Chinese children with Williams syndrome

PubMed Central

2014-01-01

Background Williams syndrome (WS) is a neurodevelopmental disease characterized by compelling psychological phenotypes. The symptoms span multiple cognitive domains and include a distinctive pattern of social behavior. The goal of this study was to explore adaptive behavior in WS patients in China. Methods We conducted a structured interview including the Infants-Junior Middle School Students Social-life Abilities Scale in three participant groups: children with WS (n = 26), normally-developing children matched for mental age (MA, n = 30), and normally-developing children matched for chronological age (CA, n = 40). We compared the mean scores for each domain between the three groups. Results Children with WS had more siblings than children in the two control groups. The educational level of the caregivers of WS children was lower than that of the control children. We found no differences in locomotion, work skill, socialization, or self-management between the WS and MA groups. WS children obtained higher scores of self-dependence (df = 54, Z = −2.379, p = 0.017) and had better communication skills (df = 54, Z = −2.222, p = 0.026) compared with MA children. The CA children achieved higher scores than the WS children for all dimensions of adaptive behavior. Conclusions WS children have better adaptive behavior skills regarding communication and self-dependence than normal children matched for mental age. Targeted intervention techniques should be designed to promote social development in this population. PMID:24708693

Risk, resources and state-dependent adaptive behavioural syndromes

PubMed Central

Luttbeg, Barney; Sih, Andrew

2010-01-01

Many animals exhibit behavioural syndromes—consistent individual differences in behaviour across two or more contexts or situations. Here, we present adaptive, state-dependent mathematical models for analysing issues about behavioural syndromes. We find that asset protection (where individuals with more ‘assets’ tend be more cautious) and starvation avoidance, two state-dependent mechanisms, can explain short-term behavioural consistency, but not long-term stable behavioural types (BTs). These negative-feedback mechanisms tend to produce convergence in state and behaviour over time. In contrast, a positive-feedback mechanism, state-dependent safety (where individuals with higher energy reserves, size, condition or vigour are better at coping with predators), can explain stable differences in personality over the long term. The relative importance of negative- and positive-feedback mechanisms in governing behavioural consistency depends on environmental conditions (predation risk and resource availability). Behavioural syndromes emerge more readily in conditions of intermediate ecological favourability (e.g. medium risk and medium resources, or high risk and resources, or low risk and resources). Under these conditions, individuals with higher initial state maintain a tendency to be bolder than individuals that start with low initial state; i.e. later BT is determined by state during an early ‘developmental window’. In contrast, when conditions are highly favourable (low risk, high resources) or highly unfavourable (high risk, low resources), individuals converge to be all relatively bold or all relatively cautious, respectively. In those circumstances, initial differences in BT are not maintained over the long term, and there is no early developmental window where initial state governs later BT. The exact range of ecological conditions favouring behavioural syndromes depends also on the strength of state-dependent safety. PMID:21078650

The small tellurium-based compound SAS suppresses inflammation in human retinal pigment epithelium

PubMed Central

Livnat, Tami; Halpert, Gilad; Jawad, Shayma; Nisgav, Yael; Azar-Avivi, Shirley; Liu, Baoying; Nussenblatt, Robert B.; Weinberger, Dov; Sredni, Benjamin

2016-01-01

Purpose Pathological angiogenesis and chronic inflammation greatly contribute to the development of choroidal neovascularization (CNV) in chorioretinal diseases involving abnormal contact between retinal pigment epithelial (RPE) and endothelial cells (ECs), associated with Bruch’s membrane rupture. We explored the ability of the small organotellurium compound octa-O-bis-(R,R)-tartarate ditellurane (SAS) to mitigate inflammatory processes in human RPE cells. Methods Cell adhesion assays and analyses of gene and protein expression were used to examine the effect of SAS on ARPE-19 cells or primary human RPE cells that were grown alone or in an RPE-EC co-culture. Results Adhesion assays showed that SAS inhibited αv integrins expressed on RPE cells. Co-cultures of RPE cells with ECs significantly reduced the gene expression of PEDF, as compared to RPE cells cultured alone. Both SAS and the anti-αvβ3 antibody LM609 significantly enhanced the production of PEDF at both mRNA and protein levels in RPE cells. RPE cells co-cultured with EC exhibited increased gene expression of CXCL5, COX1, MMP2, IGF1, and IL8, all of which are involved in both angiogenesis and inflammation. The enhanced expression of these genes was greatly suppressed by SAS, but interestingly, remained unaffected by LM609. Zymography assay showed that SAS reduced the level of MMP-2 activity in RPE cells. We also found that SAS significantly suppressed IL-1β-induced IL-6 expression and secretion from RPE cells by reducing the protein levels of phospho-IkappaBalpha (pIκBα). Conclusions Our results suggest that SAS is a promising anti-inflammatory agent in RPE cells, and may be an effective therapeutic approach for controlling chorioretinal diseases. PMID:27293373

The small tellurium-based compound SAS suppresses inflammation in human retinal pigment epithelium.

PubMed

Dardik, Rima; Livnat, Tami; Halpert, Gilad; Jawad, Shayma; Nisgav, Yael; Azar-Avivi, Shirley; Liu, Baoying; Nussenblatt, Robert B; Weinberger, Dov; Sredni, Benjamin

2016-01-01

Pathological angiogenesis and chronic inflammation greatly contribute to the development of choroidal neovascularization (CNV) in chorioretinal diseases involving abnormal contact between retinal pigment epithelial (RPE) and endothelial cells (ECs), associated with Bruch's membrane rupture. We explored the ability of the small organotellurium compound octa-O-bis-(R,R)-tartarate ditellurane (SAS) to mitigate inflammatory processes in human RPE cells. Cell adhesion assays and analyses of gene and protein expression were used to examine the effect of SAS on ARPE-19 cells or primary human RPE cells that were grown alone or in an RPE-EC co-culture. Adhesion assays showed that SAS inhibited αv integrins expressed on RPE cells. Co-cultures of RPE cells with ECs significantly reduced the gene expression of PEDF, as compared to RPE cells cultured alone. Both SAS and the anti-αvβ3 antibody LM609 significantly enhanced the production of PEDF at both mRNA and protein levels in RPE cells. RPE cells co-cultured with EC exhibited increased gene expression of CXCL5, COX1, MMP2, IGF1, and IL8, all of which are involved in both angiogenesis and inflammation. The enhanced expression of these genes was greatly suppressed by SAS, but interestingly, remained unaffected by LM609. Zymography assay showed that SAS reduced the level of MMP-2 activity in RPE cells. We also found that SAS significantly suppressed IL-1β-induced IL-6 expression and secretion from RPE cells by reducing the protein levels of phospho-IkappaBalpha (pIκBα). Our results suggest that SAS is a promising anti-inflammatory agent in RPE cells, and may be an effective therapeutic approach for controlling chorioretinal diseases.

Anterior horn syndrome: A rare manifestation of primary Sjögren's syndrome.

PubMed

Zahlane, Safaa; Louhab, Nissrine; El Mellakh, Meriem; Kissani, Najib

2016-07-01

The authors report an exceptional case of an anterior horn syndrome associated with Sjögren's syndrome in a 58-year-old patient with a flaccid tetraparesis revealed by asymmetric atrophy and diffuse fasciculations associated with xerostomia and xerophthalmia. The electroneuromyography objectified a diffuse anterior horn syndrome. The brain MRI and spinal cord were normal. Laboratory tests revealed positive anti-SSA and anti-SSB antibody. The salivary glands biopsy objectified lymphocytic sialadenitis grade 3 of Chisholm. The Schirmer's test was abnormally low. Diagnosis of anterior horn syndrome as part of Sjögren's syndrome was retained. The methylprednisolone bolus allowed partial clinical improvement after 12 months of evolution. Therefore, in patients with isolated anterior horn involvement, a correct diagnosis of the underlying SS is often delayed or overlooked entirely; in these instances, standard clinicoserological assessment is recommendable. Copyright © 2016 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

[A SAS marco program for batch processing of univariate Cox regression analysis for great database].

PubMed

Yang, Rendong; Xiong, Jie; Peng, Yangqin; Peng, Xiaoning; Zeng, Xiaomin

2015-02-01

To realize batch processing of univariate Cox regression analysis for great database by SAS marco program. We wrote a SAS macro program, which can filter, integrate, and export P values to Excel by SAS9.2. The program was used for screening survival correlated RNA molecules of ovarian cancer. A SAS marco program could finish the batch processing of univariate Cox regression analysis, the selection and export of the results. The SAS macro program has potential applications in reducing the workload of statistical analysis and providing a basis for batch processing of univariate Cox regression analysis.

The General Adaptation Syndrome: A Foundation for the Concept of Periodization.

PubMed

Cunanan, Aaron J; DeWeese, Brad H; Wagle, John P; Carroll, Kevin M; Sausaman, Robert; Hornsby, W Guy; Haff, G Gregory; Triplett, N Travis; Pierce, Kyle C; Stone, Michael H

2018-04-01

Recent reviews have attempted to refute the efficacy of applying Selye's general adaptation syndrome (GAS) as a conceptual framework for the training process. Furthermore, the criticisms involved are regularly used as the basis for arguments against the periodization of training. However, these perspectives fail to consider the entirety of Selye's work, the evolution of his model, and the broad applications he proposed. While it is reasonable to critically evaluate any paradigm, critics of the GAS have yet to dismantle the link between stress and adaptation. Disturbance to the state of an organism is the driving force for biological adaptation, which is the central thesis of the GAS model and the primary basis for its application to the athlete's training process. Despite its imprecisions, the GAS has proven to be an instructive framework for understanding the mechanistic process of providing a training stimulus to induce specific adaptations that result in functional enhancements. Pioneers of modern periodization have used the GAS as a framework for the management of stress and fatigue to direct adaptation during sports training. Updates to the periodization concept have retained its founding constructs while explicitly calling for scientifically based, evidence-driven practice suited to the individual. Thus, the purpose of this review is to provide greater clarity on how the GAS serves as an appropriate mechanistic model to conceptualize the periodization of training.

The Prospects of SAS Interferometry for Detection and Classification (SAS Interferometrie voor Detectie en Classificatie)

DTIC Science & Technology

2008-10-01

DV2008A176 Opdrachtnummer Datum October 2008 Auteur (s) dr. R. van Vossen B.A.J. Quesson dr.ir. J.C. Sabel Rubricering rapport Ongerubriceerd TH9...TNO report | TNO-DV 2008 A176 4/44 Summary This report presents an overview of the theory and implementation of interferometric SAS processing at TNO... theory in software has been tested on two types of data, simulated and measured. Chapter 3 presents results obtained with simulated data; Chapter 4

[Registration study on analysis of adaptation syndromes and medication characteristics of tanreqing injection].

PubMed

Xie, Peng-Yang; Xie, Yan-Ming; Wang, Lian-Xin; Chang, Yan-Peng; You, Li; Zhang, Xiao-Li

2014-09-01

Tanreqing injection is suitable for early pneumonia, acute bronchitis, acute exacerbations of chronic, and upper respiratory tract infection which are classified with phlegm-heat obstructing lung syndrome of traditional Chinese medicine. To understand the clinical adaptation syndromes and medication characteristics of the post-market Tanreqing injection, the research team of the paper monitored the patients who are used with Tanreqing injection from September 2012 to October 2013 in four leader hospitals based on the method--prospective, multi-center, large sample, registration-type hospital centralized monitoring,and analyzes the general information, diagnostic information and medication characteristics of patients, in order to produce evidence for clinical practice and medication decisions and to establish the foundation of rational drug use.

SAS- Semantic Annotation Service for Geoscience resources on the web

NASA Astrophysics Data System (ADS)

Elag, M.; Kumar, P.; Marini, L.; Li, R.; Jiang, P.

2015-12-01

There is a growing need for increased integration across the data and model resources that are disseminated on the web to advance their reuse across different earth science applications. Meaningful reuse of resources requires semantic metadata to realize the semantic web vision for allowing pragmatic linkage and integration among resources. Semantic metadata associates standard metadata with resources to turn them into semantically-enabled resources on the web. However, the lack of a common standardized metadata framework as well as the uncoordinated use of metadata fields across different geo-information systems, has led to a situation in which standards and related Standard Names abound. To address this need, we have designed SAS to provide a bridge between the core ontologies required to annotate resources and information systems in order to enable queries and analysis over annotation from a single environment (web). SAS is one of the services that are provided by the Geosematnic framework, which is a decentralized semantic framework to support the integration between models and data and allow semantically heterogeneous to interact with minimum human intervention. Here we present the design of SAS and demonstrate its application for annotating data and models. First we describe how predicates and their attributes are extracted from standards and ingested in the knowledge-base of the Geosemantic framework. Then we illustrate the application of SAS in annotating data managed by SEAD and annotating simulation models that have web interface. SAS is a step in a broader approach to raise the quality of geoscience data and models that are published on the web and allow users to better search, access, and use of the existing resources based on standard vocabularies that are encoded and published using semantic technologies.

Coupling the System Analysis Module with SAS4A/SASSYS-1

DOE Office of Scientific and Technical Information (OSTI.GOV)

Fanning, T. H.; Hu, R.

2016-09-30

SAS4A/SASSYS-1 is a simulation tool used to perform deterministic analysis of anticipated events as well as design basis and beyond design basis accidents for advanced reactors, with an emphasis on sodium fast reactors. SAS4A/SASSYS-1 has been under development and in active use for nearly forty-five years, and is currently maintained by the U.S. Department of Energy under the Office of Advanced Reactor Technology. Although SAS4A/SASSYS-1 contains a very capable primary and intermediate system modeling component, PRIMAR-4, it also has some shortcomings: outdated data management and code structure makes extension of the PRIMAR-4 module somewhat difficult. The user input format formore » PRIMAR-4 also limits the number of volumes and segments that can be used to describe a given system. The System Analysis Module (SAM) is a fairly new code development effort being carried out under the U.S. DOE Nuclear Energy Advanced Modeling and Simulation (NEAMS) program. SAM is being developed with advanced physical models, numerical methods, and software engineering practices; however, it is currently somewhat limited in the system components and phenomena that can be represented. For example, component models for electromagnetic pumps and multi-layer stratified volumes have not yet been developed. Nor is there support for a balance of plant model. Similarly, system-level phenomena such as control-rod driveline expansion and vessel elongation are not represented. This report documents fiscal year 2016 work that was carried out to couple the transient safety analysis capabilities of SAS4A/SASSYS-1 with the system modeling capabilities of SAM under the joint support of the ART and NEAMS programs. The coupling effort was successful and is demonstrated by evaluating an unprotected loss of flow transient for the Advanced Burner Test Reactor (ABTR) design. There are differences between the stand-alone SAS4A/SASSYS-1 simulations and the coupled SAS/SAM simulations, but these are

New Shortwave Array Spectroradiometer-Hemispheric (SAS-He): Hyperspectral Design and Initial Applications

DOE Office of Scientific and Technical Information (OSTI.GOV)

Kassianov, Evgueni I.; Flynn, Connor J M.; Barnard, James C.

2016-10-31

Aerosol optical depth (AOD) derived from hyperspectral measurements can serve as an invaluable input for simultaneous retrievals of particle size distributions and major trace gases. The required hyperspectral measurements are provided by a new ground-based radiometer, the so-called Shortwave Array Spectroradiometer-Hemispheric (SAS-He), recently developed with support from the Department of Energy (DOE) Office Atmospheric Radiation Measurement (ARM) Program. The SAS-He has wide spectral coverage (350-1700nm) and high spectral resolution: about 2.4 nm and 6 nm within 350-1000 nm and 970-1700 nm spectral ranges, respectively. To illustrate an initial performance of the SAS-He, we take advantage of integrated dataset collected duringmore » the ARM-supported Two-Column Aerosol Project (TCAP) over the US coastal region (Cape Cod, Massachusetts). This dataset includes AODs derived using data from Aerosol Robotic Network (AERONET) sunphotometer and Multi-Filter Rotating Shadowband Radiometer (MFRSR). We demonstrate that, on average, the SAS-He AODs closely match the MFRSR and AERONET AODs in the ultraviolet and visible spectral ranges for this area with highly variable AOD. Also, we discuss corrections of SAS-He total optical depth for gas absorption in the near-infrared spectral range and their operational implementation.« less

Want independent validation and assurance? Ask for a SAS-70.

PubMed

Boutin, Christopher C

2008-08-01

The AICPA's Statement on Auditing Standards No.70, Service Organizations addresses CPA audits of service providers conducted to verify that a provider has adequate controls over its operations. Hospitals should request a SAS-70, the report produced by such an audit, from all of their third-party service providers. SAS-70s can be issued for a specific date or for a six-month period, and they typically consist of three sections: a CPA opinion, a description of controls, and information about the design of the controls.

[Management of myelodysplastic syndromes].

PubMed

Duchmann, Matthieu; Fenaux, Pierre; Cluzeau, Thomas

2015-11-01

Myelodysplastic syndromes are heterogeneous diseases whose molecular characteristics have only been identified in recent years. Better identification of prognostic factors, larger access to allogeneic stem cell transplantation and the advent of new drugs notably hypomethylating agents (azacitidine, decitabine) and lenalidomide have improved patient outcome. Copyright © 2015 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.

[Good's syndrome and congenital toxoplasmosis due to maternal reactivation during pregnancy].

PubMed

Tahiri, J; Fouyssac, F; Morel, O; Maatouk, A

2017-05-01

Good syndrome is a rare condition in which thymoma is associated with hypogammaglobulinemia. It is characterized by an increased susceptibility to infections. We report a woman with Good's syndrome diagnosed after severe congenital toxoplasmosis in her daughter, even though she was immunized against this infection during pregnancy. This presentation is very unusual by its early diagnosis and to our knowledge is the first report of parasitic infection in this syndrome. Copyright © 2016. Published by Elsevier SAS.

Preparation and pharmaceutical characterization of amorphous cefdinir using spray-drying and SAS-process.

PubMed

Park, Junsung; Park, Hee Jun; Cho, Wonkyung; Cha, Kwang-Ho; Kang, Young-Shin; Hwang, Sung-Joo

2010-08-30

The aim of this study was to investigate the effects of micronization and amorphorization of cefdinir on solubility and dissolution rate. The amorphous samples were prepared by spray-drying (SD) and supercritical anti-solvent (SAS) process, respectively and their amorphous natures were confirmed by DSC, PXRD and FT-IR. Thermal gravimetric analysis was performed by TGA. SEM was used to investigate the morphology of particles and the processed particle had a spherical shape, while the unprocessed crystalline particle had a needle-like shape. The mean particle size and specific surface area were measured by dynamic light scattering (DLS) and BET, respectively. The DLS result showed that the SAS-processed particle was the smallest, followed by SD and the unprocessed cefdinir. The BET result was the same as DLS result in that the SAS-processed particle had the largest surface area. Therefore, the processed cefdinir, especially the SAS-processed particle, appeared to have enhanced apparent solubility, improved intrinsic dissolution rate and better drug release when compared with SD-processed and unprocessed crystalline cefdinir due not only to its amorphous nature, but also its reduced particle size. Conclusions were that the solubility and dissolution rate of crystalline cefdinir could be improved by physically modifying the particles using SD and SAS-process. Furthermore, SAS-process was a powerful methodology for improving the solubility and dissolution rate of cefdinir. Copyright 2010 Elsevier B.V. All rights reserved.

Breast Cancer Risk Assessment SAS Macro (Gail Model)

Cancer.gov

A SAS macro (commonly referred to as the Gail Model) that projects absolute risk of invasive breast cancer according to NCI’s Breast Cancer Risk Assessment Tool (BCRAT) algorithm for specified race/ethnic groups and age intervals.

The Relationship between the Triglyceride to High-Density Lipoprotein Cholesterol Ratio and Metabolic Syndrome.

PubMed

Shin, Hyun-Gyu; Kim, Young-Kwang; Kim, Yong-Hwan; Jung, Yo-Han; Kang, Hee-Cheol

2017-11-01

Metabolic syndrome is associated with cardiovascular diseases and is characterized by insulin resistance. Recent studies suggest that the triglyceride/high-density lipoprotein cholesterol (TG/HDLC) ratio predicts insulin resistance better than individual lipid levels, including TG, total cholesterol, low-density lipoprotein cholesterol (LDLC), or HDLC. We aimed to elucidate the relationship between the TG/HDLC ratio and metabolic syndrome in the general Korean population. We evaluated the data of adults ≥20 years old who were enrolled in the Korean National Health and Nutrition Examination Survey in 2013 and 2014. Subjects with angina pectoris, myocardial infarction, stroke, or cancer were excluded. Metabolic syndrome was defined by the harmonized definition. We examined the odds ratios (ORs) of metabolic syndrome according to TG/HDLC ratio quartiles using logistic regression analysis (SAS ver. 9.4; SAS Institute Inc., Cary, NC, USA). Weighted complex sample analysis was also conducted. We found a significant association between the TG/HDLC ratio and metabolic syndrome. The cutoff value of the TG/HDLC ratio for the fourth quartile was ≥3.52. After adjustment, the OR for metabolic syndrome in the fourth quartile compared with that of the first quartile was 29.65 in men and 20.60 in women (P<0.001). The TG/HDLC ratio is significantly associated with metabolic syndrome.

A Non-Traditional Model of the Metabolic Syndrome: The Adaptive Significance of Insulin Resistance in Fasting-Adapted Seals

PubMed Central

Houser, Dorian S.; Champagne, Cory D.; Crocker, Daniel E.

2013-01-01

Insulin resistance in modern society is perceived as a pathological consequence of excess energy consumption and reduced physical activity. Its presence in relation to the development of cardiovascular risk factors has been termed the metabolic syndrome, which produces increased mortality and morbidity and which is rapidly increasing in human populations. Ironically, insulin resistance likely evolved to assist animals during food shortages by increasing the availability of endogenous lipid for catabolism while protecting protein from use in gluconeogenesis and eventual oxidation. Some species that incorporate fasting as a predictable component of their life history demonstrate physiological traits similar to the metabolic syndrome during prolonged fasts. One such species is the northern elephant seal (Mirounga angustirostris), which fasts from food and water for periods of up to 4 months. During this time, ∼90% of the seals metabolic demands are met through fat oxidation and circulating non-esterified fatty acids are high (0.7–3.2 mM). All life history stages of elephant seal studied to date demonstrate insulin resistance and fasting hyperglycemia as well as variations in hormones and adipocytokines that reflect the metabolic syndrome to some degree. Elephant seals demonstrate some intriguing adaptations with the potential for medical advancement; for example, ketosis is negligible despite significant and prolonged fatty acid oxidation and investigation of this feature might provide insight into the treatment of diabetic ketoacidosis. The parallels to the metabolic syndrome are likely reflected to varying degrees in other marine mammals, most of which evolved on diets high in lipid and protein content but essentially devoid of carbohydrate. Utilization of these natural models of insulin resistance may further our understanding of the pathophysiology of the metabolic syndrome in humans and better assist the development of preventative measures and therapies

A non-traditional model of the metabolic syndrome: the adaptive significance of insulin resistance in fasting-adapted seals.

PubMed

Houser, Dorian S; Champagne, Cory D; Crocker, Daniel E

2013-11-01

Insulin resistance in modern society is perceived as a pathological consequence of excess energy consumption and reduced physical activity. Its presence in relation to the development of cardiovascular risk factors has been termed the metabolic syndrome, which produces increased mortality and morbidity and which is rapidly increasing in human populations. Ironically, insulin resistance likely evolved to assist animals during food shortages by increasing the availability of endogenous lipid for catabolism while protecting protein from use in gluconeogenesis and eventual oxidation. Some species that incorporate fasting as a predictable component of their life history demonstrate physiological traits similar to the metabolic syndrome during prolonged fasts. One such species is the northern elephant seal (Mirounga angustirostris), which fasts from food and water for periods of up to 4 months. During this time, ∼90% of the seals metabolic demands are met through fat oxidation and circulating non-esterified fatty acids are high (0.7-3.2 mM). All life history stages of elephant seal studied to date demonstrate insulin resistance and fasting hyperglycemia as well as variations in hormones and adipocytokines that reflect the metabolic syndrome to some degree. Elephant seals demonstrate some intriguing adaptations with the potential for medical advancement; for example, ketosis is negligible despite significant and prolonged fatty acid oxidation and investigation of this feature might provide insight into the treatment of diabetic ketoacidosis. The parallels to the metabolic syndrome are likely reflected to varying degrees in other marine mammals, most of which evolved on diets high in lipid and protein content but essentially devoid of carbohydrate. Utilization of these natural models of insulin resistance may further our understanding of the pathophysiology of the metabolic syndrome in humans and better assist the development of preventative measures and therapies.

Microgravity

NASA Image and Video Library

1992-09-12

Space Shuttle Endeavour (STS-47) onboard photo of Japanese Payload Specialist Dr. Mamoru Mohri participating in Comparative Measurement of Visual Stability in Earth Cosmic Space experiment to learn more about Space Adaptation Syndrome (SAS).

Onboard photo: Japanese Payload Specialist Dr. Mamoru Mohri at work in Spacelab-J module

NASA Technical Reports Server (NTRS)

1992-01-01

Space Shuttle Endeavour (STS-47) onboard photo of Japanese Payload Specialist Dr. Mamoru Mohri participating in Comparative Measurement of Visual Stability in Earth Cosmic Space experiment to learn more about Space Adaptation Syndrome (SAS).

Clinical heterogeneity of patients with complex sleep apnea syndrome.

PubMed

Kuźniar, Tomasz J; Kasibowska-Kuźniar, Kamilla; Ray, Daniel W; Freedom, Thomas

2013-12-01

The definition of complex sleep apnea (CompSAS) encompasses patients with obstructive sleep apnea (OSA) who develop central apnea activity upon restitution of airway patency. Presence of arterial hypertension (HTN), coronary artery disease (CAD) and heart failure (HF) have been proposed as risk factors for CompSAS among OSA patients. Using our database of patients with CompSAS, we examined the prevalence of these risk factors and defined other clinical characteristics of patients with CompSAS. Through retrospective search of the database, we examined the medical and clinical characteristics of consecutive patients diagnosed with CompSAS between 11/1/2006 and 6/30/2011 at NorthShore University HealthSystem. One hundred and fifty patients with CompSAS were identified. Among patients included in the study, 97 (64.7 %) had at least one risk factor for CompSAS, while 53 (35.3 %) did not have any of them. Prevalence of low left ventricular ejection fraction and hypocapnia were low. Therapeutic interventions consisted of several positive airway pressure therapies, mainly adaptive servo ventilation. A hundred and ten patients (73.3 %) complied with recommended therapy and improved clinically. Although most patients with CompSAS have cardiac comorbidities, about one third of patients do not have any risk factors of CompSAS prior to sleep testing. Further research on factors involved in development of CompSAS will allow for better tailoring of therapy to pathophysiology involved in an individual case.

[Down syndrome maternal serum screening: results' comments recommended by accredited biologists].

PubMed

Muller, F; Dreux, S; Czerkiewicz, I; Bernard, M; Guibourdenche, J; Lacroix, I; Moineau, M-P; Read, M-H; Sault, C; Thibaud, D; Veyrat, B; Bidat, L

2014-11-01

Down syndrome maternal serum screening is largely used in France. The aim of this article is to specify and to explain the different comments applied on the reports in order to optimize the management of the patient. These comments represent the consensus of the study group of the biologist accredited for Down syndrome maternal serum screening. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Maternal perceptions of sibling adaptation in Korean families of children with Down syndrome.

PubMed

Choi, H; Van Riper, M

2014-10-01

It is estimated that more than 500 infants with Down syndrome (DS) are born each year in Korea. DS affects not only these individuals, but family members as well. Some siblings deal successfully with the challenges of living with a child with DS and adapt well while others struggle or fail to adapt. The aims of this descriptive study were to explore how Korean mothers of children with DS perceive the adaptation of their typically developing (TD) children aged 4 to 19 and how family variables contribute to sibling adaptation. This descriptive, cross-sectional study was conducted with 105 Korean mothers. Most mothers indicated that their TD children were not experiencing psychological or behavioural problems; however, many described problems in the sibling relationship. It was found that family factors (i.e. condition management effort, condition management ability, child's daily life, parental mutuality, family hardiness and social support) were strong predictors of sibling psychological, behavioural and relational adaptation. Demographic characteristics of the child with DS, the mother and the family appeared to significantly influence sibling adaptation. These findings highlight the importance of familial contexts in understanding sibling adaptation. Knowledge of family factors associated with better adaptation in Korean siblings of child with DS will facilitate the development of culturally appropriate interventions for these children and their families. In addition, an awareness of demographic characteristics associated with sibling adaptation will help health care professionals identify siblings who are at increased risk of experiencing difficulties in adapting. © 2014 MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.

Aging-Resistant Functionalized LDH⁻SAS/Nitrile-Butadiene Rubber Composites: Preparation and Study of Aging Kinetics/Anti-Aging Mechanism.

PubMed

Li, Tianxiang; Shi, Zhengren; He, Xianru; Jiang, Ping; Lu, Xiaobin; Zhang, Rui; Wang, Xin

2018-05-18

With the aim of improving the anti-aging properties of nitrile-butadiene rubber (NBR), a functional organic filler, namely LDH⁻SAS, prepared by intercalating 4-amino-benzenesulfonic acid monosodium salt (SAS) into layered double hydroxides (LDHs) through anion exchange, was added to nitrile-butadiene rubber (NBR), giving the NBR/LDH⁻SAS composites. Successful preparation of LDH⁻SAS was confirmed by XRD, TGA and FTIR. LDH⁻SAS was well dispersed in the NBR matrix, owing to its strong interaction with the nitrile group of NBR. The obtained NBR/LDH⁻SAS composites exhibited excellent thermo-oxidative aging resistance as shown by TGA-DSC. Further investigation by ATR-FTIR indicated that SAS can capture the radical groups, even during the aging process, which largely accounts for the improved aging resistance.

Using SAS PROC MCMC for Item Response Theory Models

PubMed Central

Samonte, Kelli

2014-01-01

Interest in using Bayesian methods for estimating item response theory models has grown at a remarkable rate in recent years. This attentiveness to Bayesian estimation has also inspired a growth in available software such as WinBUGS, R packages, BMIRT, MPLUS, and SAS PROC MCMC. This article intends to provide an accessible overview of Bayesian methods in the context of item response theory to serve as a useful guide for practitioners in estimating and interpreting item response theory (IRT) models. Included is a description of the estimation procedure used by SAS PROC MCMC. Syntax is provided for estimation of both dichotomous and polytomous IRT models, as well as a discussion on how to extend the syntax to accommodate more complex IRT models. PMID:29795834

Aging-Resistant Functionalized LDH–SAS/Nitrile-Butadiene Rubber Composites: Preparation and Study of Aging Kinetics/Anti-Aging Mechanism

PubMed Central

Li, Tianxiang; Shi, Zhengren; He, Xianru; Jiang, Ping; Lu, Xiaobin; Zhang, Rui

2018-01-01

With the aim of improving the anti-aging properties of nitrile-butadiene rubber (NBR), a functional organic filler, namely LDH–SAS, prepared by intercalating 4-amino-benzenesulfonic acid monosodium salt (SAS) into layered double hydroxides (LDHs) through anion exchange, was added to nitrile-butadiene rubber (NBR), giving the NBR/LDH–SAS composites. Successful preparation of LDH–SAS was confirmed by XRD, TGA and FTIR. LDH–SAS was well dispersed in the NBR matrix, owing to its strong interaction with the nitrile group of NBR. The obtained NBR/LDH–SAS composites exhibited excellent thermo-oxidative aging resistance as shown by TGA-DSC. Further investigation by ATR-FTIR indicated that SAS can capture the radical groups, even during the aging process, which largely accounts for the improved aging resistance. PMID:29783656

Turner syndrome and meningioma: support for a possible increased risk of neoplasia in Turner syndrome.

PubMed

Pier, Danielle B; Nunes, Fabio P; Plotkin, Scott R; Stemmer-Rachamimov, Anat O; Kim, James C; Shih, Helen A; Brastianos, Priscilla; Lin, Angela E

2014-01-01

Neoplasia is uncommon in Turner syndrome, although there is some evidence that brain tumors are more common in Turner syndrome patients than in the general population. We describe a woman with Turner syndrome (45,X) with a meningioma, in whom a second neoplasia, basal cell carcinomas of the scalp and nose, developed five years later in the absence of therapeutic radiation. Together with 7 cases of Turner syndrome with meningioma from a population-based survey in the United Kingdom, and 3 other isolated cases in the literature, we review this small number of patients for evidence of risk factors related to Turner syndrome, such as associated structural anomalies or prior treatment. We performed histological and fluorescent in situ hybridization (FISH) of 22q (NF2 locus) analyses of the meningeal tumor to search for possible molecular determinants. We are not able to prove causation between these two entities, but suggest that neoplasia may be a rare associated medical problem in Turner syndrome. Additional case reports and extension of population-based studies are needed. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Chiari malformation and central sleep apnea syndrome: efficacy of treatment with adaptive servo-ventilation*

PubMed Central

do Vale, Jorge Marques; Silva, Eloísa; Pereira, Isabel Gil; Marques, Catarina; Sanchez-Serrano, Amparo; Torres, António Simões

2014-01-01

The Chiari malformation type I (CM-I) has been associated with sleep-disordered breathing, especially central sleep apnea syndrome. We report the case of a 44-year-old female with CM-I who was referred to our sleep laboratory for suspected sleep apnea. The patient had undergone decompressive surgery 3 years prior. An arterial blood gas analysis showed hypercapnia. Polysomnography showed a respiratory disturbance index of 108 events/h, and all were central apnea events. Treatment with adaptive servo-ventilation was initiated, and central apnea was resolved. This report demonstrates the efficacy of servo-ventilation in the treatment of central sleep apnea syndrome associated with alveolar hypoventilation in a CM-I patient with a history of decompressive surgery. PMID:25410846

5p13 microduplication syndrome: a new case and better clinical definition of the syndrome.

PubMed

Novara, Francesca; Alfei, Enrico; D'Arrigo, Stefano; Pantaleoni, Chiara; Beri, Silvana; Achille, Valentina; Sciacca, Francesca L; Giorda, Roberto; Zuffardi, Orsetta; Ciccone, Roberto

2013-01-01

Chromosome 5p13 duplication syndrome (OMIM #613174), a contiguous gene syndrome involving duplication of several genes on chromosome 5p13 including NIPBL (OMIM 608667), has been described in rare patients with developmental delay and learning disability, behavioral problems and peculiar facial dysmorphisms. 5p13 duplications described so far present with variable sizes, from 0.25 to 13.6 Mb, and contain a variable number of genes. Here we report another patient with 5p13 duplication syndrome including NIPBL gene only. Proband's phenotype overlapped that reported in patients with 5p13 microduplication syndrome and especially that of subjects with smaller duplications. Moreover, we better define genotype-phenotype relationship associated with this duplication and confirmed that NIPBL was likely the major dosage sensitive gene for the 5p13 microduplication phenotype. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

[Neonatal Pearson syndrome. two case studies].

PubMed

Collin-Ducasse, H; Maillotte, A-M; Monpoux, F; Boutté, P; Ferrero-Vacher, C; Paquis, V

2010-01-01

Among the etiologies of anemia in the newborn, those related to mitochondrial cytopathies are rare. Pearson syndrome is mostly diagnosed during infancy and characterized by refractory sideroblastic anemia with vacuolization of marrow progenitor cells and exocrine pancreatic dysfunction. We describe two diagnosed cases of Pearson syndrome in the early neonatal period caused by severe macrocytic aregenerative anemia. Bone marrow aspiration revealed sideroblastic anemia and vacuolization of erythroblastic precursors. The diagnosis was confirmed by genetic analysis revealing a deletion in the mitochondrial DNA. These two newborns received monthly transfusions. Five other newborns suffering from Pearson syndrome with various clinical symptoms were found in literature. Pearson syndrome, rarely diagnosed in newborns, should be suspected in the presence of macrocytic aregenerative anemia and requires a bone marrow aspirate followed by a genetic analysis from a blood sample. Copyright 2009 Elsevier Masson SAS. All rights reserved.

Laugier-Hunziker syndrome: A case report.

PubMed

Wei, Z; Li, G-Y; Ruan, H-H; Zhang, L; Wang, W-M; Wang, X

2018-04-01

Laugier-Hunziker syndrome (LHS) is a rare, benign, acquired pigmentary condition mainly affecting lips, oral mucosa and acral area, frequently associated with longitudinal melanonychia. Herein, we reported a 45-year-old female case with LHS. The clinical, dermoscopic, histopathologic features of LHS were reviewed and the important differential diagnosis was discussed. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Multifractal spectra of laser Doppler flowmetry signals in healthy and sleep apnea syndrome subjects

NASA Astrophysics Data System (ADS)

Buard, Benjamin; Trzepizur, Wojciech; Mahe, Guillaume; Chapeau-Blondeau, François; Rousseau, David; Gagnadoux, Frédéric; Abraham, Pierre; Humeau, Anne

2009-07-01

Laser Doppler flowmetry (LDF) signals give a peripheral view of the cardiovascular system. To better understand the possible modifications brought by sleep apnea syndrome (SAS) in LDF signals, we herein propose to analyze the complexity of such signals in obstructive SAS subjects, and to compare the results with those obtained in healthy subjects. SAS is a pathology that leads to a drop in the parasympathetic tone associated with an increase in the sympathetic tone in awakens SAS patients. Nine men with obstructive SAS and nine healthy men participated awaken in our study and LDF signals were recorded in the forearm. In our work, complexity of LDF signals is analyzed through the computation and analysis of their multifractal spectra. The multifractal spectra are estimated by first estimating the discrete partition function of the signals, then by determining their Renyi exponents with a linear regression, and finally by computing their Legendre transform. The results show that, at rest, obstructive SAS has no or little impact on the multifractal spectra of LDF signals recorded in the forearm. This study shows that the physiological modifications brought by obstructive SAS do not modify the complexity of LDF signals when recorded in the forearm.

Sexual Assertiveness Scale (SAS) for women: development and validation.

PubMed

Morokoff, P J; Quina, K; Harlow, L L; Whitmire, L; Grimley, D M; Gibson, P R; Burkholder, G J

1997-10-01

Four studies were conducted to develop and validate the Sexual Assertiveness Scale (SAS), a measure of sexual assertiveness in women that consists of factors measuring initiation, refusal, and pregnancy-sexually transmitted disease prevention assertiveness. A total of 1,613 women from both university and community populations were studied. Confirmatory factor analyses demonstrated that the 3 factors remained stable across samples of university and community women. A structural model was tested in 2 samples, indicating that sexual experience, anticipated negative partner response, and self-efficacy are consistent predictors of sexual assertiveness. Sexual assertiveness was found to be somewhat related to relationship satisfaction, power, and length. The community sample was retested after 6 months and 1 year to establish test-retest reliability. The SAS provides a reliable instrument for assessing and understanding women's sexual assertiveness.

[Corneal lesions in Kindler syndrome: a case report].

PubMed

Chéour, M; Mazlout, H; Ben Jalel, W; Brour, J; Baroudi, B; Kraiem, A

2012-01-01

Kindler syndrome is a rare autosomal recessive genodermatosis belonging to the class of bullous poikiloderma. Corneal lesions are rare. We report a case of ocular lesions in this syndrome. We report the case of a 57-year-old patient followed since childhood in dermatology with the diagnosis of Kindler syndrome. He presented to the ophthalmology department with decreased vision. Ophthalmologic examination showed symblepharon, ectropion in both eyes, and corneal deformation. The role played by the abnormal protein in epithelial integrity suggests that ocular and more particularly corneal involvement is not rare in Kindler syndrome. In fact, it is less known by ophthalmologists and dermatologists are not aware of the ophthalmologic manifestations. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

SIMCA T 1.0: A SAS Computer Program for Simulating Computer Adaptive Testing

ERIC Educational Resources Information Center

Raiche, Gilles; Blais, Jean-Guy

2006-01-01

Monte Carlo methodologies are frequently applied to study the sampling distribution of the estimated proficiency level in adaptive testing. These methods eliminate real situational constraints. However, these Monte Carlo methodologies are not currently supported by the available software programs, and when these programs are available, their…

Ambiguities and completeness of SAS data analysis: investigations of apoferritin by SAXS/SANS EID and SEC-SAXS methods

NASA Astrophysics Data System (ADS)

Zabelskii, D. V.; Vlasov, A. V.; Ryzhykau, Yu L.; Murugova, T. N.; Brennich, M.; Soloviov, D. V.; Ivankov, O. I.; Borshchevskiy, V. I.; Mishin, A. V.; Rogachev, A. V.; Round, A.; Dencher, N. A.; Büldt, G.; Gordeliy, V. I.; Kuklin, A. I.

2018-03-01

The method of small angle scattering (SAS) is widely used in the field of biophysical research of proteins in aqueous solutions. Obtaining low-resolution structure of proteins is still a highly valuable method despite the advances in high-resolution methods such as X-ray diffraction, cryo-EM etc. SAS offers the unique possibility to obtain structural information under conditions close to those of functional assays, i.e. in solution, without different additives, in the mg/mL concentration range. SAS method has a long history, but there are still many uncertainties related to data treatment. We compared 1D SAS profiles of apoferritin obtained by X-ray diffraction (XRD) and SAS methods. It is shown that SAS curves for X-ray diffraction crystallographic structure of apoferritin differ more significantly than it might be expected due to the resolution of the SAS instrument. Extrapolation to infinite dilution (EID) method does not sufficiently exclude dimerization and oligomerization effects and therefore could not guarantee total absence of dimers account in the final SAS curve. In this study, we show that EID SAXS, EID SANS and SEC-SAXS methods give complementary results and when they are used all together, it allows obtaining the most accurate results and high confidence from SAS data analysis of proteins.

Social Anxiety Scale for Adolescents (SAS-A): Measuring Social Anxiety among Finnish Adolescents

ERIC Educational Resources Information Center

Ranta, Klaus; Junttila, Niina; Laakkonen, Eero; Uhmavaara, Anni; La Greca, Annette M.; Niemi, Paivi M.

2012-01-01

The aim of this study was to investigate symptoms of social anxiety and the psychometric properties of the "Social Anxiety Scale for Adolescents" (SAS-A) among Finnish adolescents, 13-16 years of age. Study 1 (n = 867) examined the distribution of SAS-A scores according to gender and age, and the internal consistency and factor structure…

Usual Dietary Intakes: SAS Macros for the NCI Method

Cancer.gov

SAS macros are currently available to facilitate modeling of a single dietary component, whether consumed daily or episodically; ratios of two dietary components that are consumed nearly every day; multiple dietary components, whether consumed daily or episodically.

The standardized functional support sectional for the Small Astronomy Satellite (SAS)

NASA Technical Reports Server (NTRS)

Townsend, M. R.

1974-01-01

The standardized functional support section for the improved Small Astronomy Satellite (SAS) spacecraft, which can be used virtually without change for a wide variety of experimental packages and missions, is described. This functional support section makes the spacecraft remarkably flexible for a small satellite. Able to point its thrust axis to any direction in space, it can also spin or slow its outer body rotation to zero for star- or earth-locked pointing of side-viewing experiments. It features a reprogrammable telemetry system, a delayed command system, and an improved control system. Experiments can be built independently and attached to the SAS spacecraft just prior to final acceptance testing and launch. The spacecraft subsystems are described in detail. Included are a summary of the spacecraft characteristics, special design considerations, project reliability requirements, and environmental test conditions. It is intended that this new functional support section afford virtual off-the-shelf availability of the SAS spacecraft to independently built experiments, thus providing quick response time and minimum cost in meeting a wide variety of experimenter needs.

SPSS and SAS programming for the testing of mediation models.

PubMed

Dudley, William N; Benuzillo, Jose G; Carrico, Mineh S

2004-01-01

Mediation modeling can explain the nature of the relation among three or more variables. In addition, it can be used to show how a variable mediates the relation between levels of intervention and outcome. The Sobel test, developed in 1990, provides a statistical method for determining the influence of a mediator on an intervention or outcome. Although interactive Web-based and stand-alone methods exist for computing the Sobel test, SPSS and SAS programs that automatically run the required regression analyses and computations increase the accessibility of mediation modeling to nursing researchers. To illustrate the utility of the Sobel test and to make this programming available to the Nursing Research audience in both SAS and SPSS. The history, logic, and technical aspects of mediation testing are introduced. The syntax files sobel.sps and sobel.sas, created to automate the computation of the regression analysis and test statistic, are available from the corresponding author. The reported programming allows the user to complete mediation testing with the user's own data in a single-step fashion. A technical manual included with the programming provides instruction on program use and interpretation of the output. Mediation modeling is a useful tool for describing the relation between three or more variables. Programming and manuals for using this model are made available.

Guillain-Barré syndrome: 100 years on.

PubMed

Créange, A

2016-12-01

The Guillain-Barré syndrome is associated with acute polyradiculoneuritis for almost one century. Its spectrum has considerably been enlarged since its first description. It now includes pure motor or sensory syndromes, focal forms, demyelinating and axonal neurophysiological features that characterise excitability dysfunctions, and immunological differentiations. We can hope that this improved classification will facilitate development of treatment innovations for a condition that is still a life-threatening condition with a severe functional prognosis in a significant proportion of cases. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Adapting Phonological Awareness Interventions for Children with Down Syndrome Based on the Behavioral Phenotype: A Promising Approach?

ERIC Educational Resources Information Center

Lemons, Christopher J.; King, Seth A.; Davidson, Kimberly A.; Puranik, Cynthia S.; Fulmer, Deborah; Mrachko, Alicia A.; Partanen, Jane; Al Otaiba, Stephanie; Fidler, Deborah J.

2015-01-01

Many children with Down syndrome demonstrate deficits in phonological awareness, a prerequisite to learning to read in an alphabetic language. The purpose of this study was to determine whether adapting a commercially available phonological awareness program to better align with characteristics associated with the behavioral phenotype of Down…

Longitudinal trajectories of intellectual and adaptive functioning in adolescents and adults with Williams syndrome.

PubMed

Fisher, M H; Lense, M D; Dykens, E M

2016-10-01

Williams syndrome (WS) is associated with a distinct cognitive-behavioural phenotype including mild to moderate intellectual disability, visual-spatial deficits, hypersociability, inattention and anxiety. Researchers typically characterise samples of individuals with WS by their intellectual functioning and adaptive behaviour. Because of the low prevalence of the syndrome, researchers often include participants with WS across a broad age range throughout childhood and adulthood and assume participants demonstrate consistent cognitive development across ages. Indeed, IQ scores are generally stable for children and adolescents with WS, although there are significant individual differences. It is less clear whether this pattern of stable intellectual ability persists into adulthood. Furthermore, while adaptive behaviour is an important indicator of an individual's ability to apply their conceptual skills to everyday functioning, conflicting findings on the trajectories of adaptive behaviour in adolescents and adults with WS have been reported. The current study examined longitudinal profiles of cognitive and adaptive functioning in adolescents and adults with WS. To examine cognitive functioning, participants included 52 individuals with WS (51.9% men) who were assessed with the Kaufman Brief Intelligence Test, 2nd edition (KBIT-2) between two and seven times. At their first assessment, participants had a mean age of 25.4 years (SD = 8.4), ranging in age from 14.2 to 48.9 years. To assess adaptive behaviour, participants included a subset of 28 individuals with WS whose parents completed the Vineland Adaptive Behavior Scale, 2nd edition (VABS-II) between two and five times. At their initial administration, participants ranged from 17.1-40.2 years of age, with a mean age of 26.5 years (SD = 7.3). A series of multilevel models were used to examine changes in KBIT-2 Composite IQ, Verbal IQ and Nonverbal IQ standard scores over time, as well as the

Clinical SAS programming in India: A study of industry needs versus wants.

PubMed

Ananthakrishnan, Nithiyanandhan

2014-07-01

The clinical SAS (www.sas.com) programming industry, in India, has seen a rapid growth in the last decade and the trend seems set to continue, for the next couple of years, due to cost advantage and the availability of skilled labor. On one side the industry needs are focused on less execution time, high margins, segmented tasks and the delivery of high quality output with minimal oversight. On the other side, due to the increased demand for skilled resources, the wants of the programmers have taken a different shift toward diversifying exposure, unsustainable wage inflation due to multiple opportunities and generally high expectations around career progression. If the industry needs are not going to match with programmers want, or vice versa, then there is the possibility that the current year on year growth may start to slow or even go into decline. This paper is intended to identify the gap between wants and need and puts forwards some suggestions, for both sides, in ways to change the equation to benefit all. Questionnaire on similar themes created to survey managers and programmers working in clinical SAS programming industry and was surveyed online to collect their perspectives. Their views are compared for each theme and presented as results. Two surveys were created in www.surveymonkey.com. https://www.surveymonkey.com/s/SAS_India_managment_needvswant_survey. Programmer: https://www.surveymonkey.com/s/SAS_India_programmer_needvswant_survey. Bar chart and pie chart used on data collect to show segmentation of data. In conclusion, it seeks to highlight the future industry direction and the skillset that existing programmers need to have, in order to sustain the momentum and remain competitive, to contribute to the future pipeline and the development of the profession in India.

The SAS4A/SASSYS-1 Safety Analysis Code System, Version 5

DOE Office of Scientific and Technical Information (OSTI.GOV)

Fanning, T. H.; Brunett, A. J.; Sumner, T.

The SAS4A/SASSYS-1 computer code is developed by Argonne National Laboratory for thermal, hydraulic, and neutronic analysis of power and flow transients in liquidmetal- cooled nuclear reactors (LMRs). SAS4A was developed to analyze severe core disruption accidents with coolant boiling and fuel melting and relocation, initiated by a very low probability coincidence of an accident precursor and failure of one or more safety systems. SASSYS-1, originally developed to address loss-of-decay-heat-removal accidents, has evolved into a tool for margin assessment in design basis accident (DBA) analysis and for consequence assessment in beyond-design-basis accident (BDBA) analysis. SAS4A contains detailed, mechanistic models of transientmore » thermal, hydraulic, neutronic, and mechanical phenomena to describe the response of the reactor core, its coolant, fuel elements, and structural members to accident conditions. The core channel models in SAS4A provide the capability to analyze the initial phase of core disruptive accidents, through coolant heat-up and boiling, fuel element failure, and fuel melting and relocation. Originally developed to analyze oxide fuel clad with stainless steel, the models in SAS4A have been extended and specialized to metallic fuel with advanced alloy cladding. SASSYS-1 provides the capability to perform a detailed thermal/hydraulic simulation of the primary and secondary sodium coolant circuits and the balance-ofplant steam/water circuit. These sodium and steam circuit models include component models for heat exchangers, pumps, valves, turbines, and condensers, and thermal/hydraulic models of pipes and plena. SASSYS-1 also contains a plant protection and control system modeling capability, which provides digital representations of reactor, pump, and valve controllers and their response to input signal changes.« less

Development of National Program of Cancer Registries SAS Tool for Population-Based Cancer Relative Survival Analysis.

PubMed

Dong, Xing; Zhang, Kevin; Ren, Yuan; Wilson, Reda; O'Neil, Mary Elizabeth

2016-01-01

Studying population-based cancer survival by leveraging the high-quality cancer incidence data collected by the Centers for Disease Control and Prevention's National Program of Cancer Registries (NPCR) can offer valuable insight into the cancer burden and impact in the United States. We describe the development and validation of a SASmacro tool that calculates population-based cancer site-specific relative survival estimates comparable to those obtained through SEER*Stat. The NPCR relative survival analysis SAS tool (NPCR SAS tool) was developed based on the relative survival method and SAS macros developed by Paul Dickman. NPCR cancer incidence data from 25 states submitted in November 2012 were used, specifically cases diagnosed from 2003 to 2010 with follow-up through 2010. Decennial and annual complete life tables published by the National Center for Health Statistics (NCHS) for 2000 through 2009 were used. To assess comparability between the 2 tools, 5-year relative survival rates were calculated for 25 cancer sites by sex, race, and age group using the NPCR SAS tool and the National Cancer Institute's SEER*Stat 8.1.5 software. A module to create data files for SEER*Stat was also developed for the NPCR SAS tool. Comparison of the results produced by both SAS and SEER*Stat showed comparable and reliable relative survival estimates for NPCR data. For a majority of the sites, the net differences between the NPCR SAS tool and SEER*Stat-produced relative survival estimates ranged from -0.1% to 0.1%. The estimated standard errors were highly comparable between the 2 tools as well. The NPCR SAS tool will allow researchers to accurately estimate cancer 5-year relative survival estimates that are comparable to those produced by SEER*Stat for NPCR data. Comparison of output from the NPCR SAS tool and SEER*Stat provided additional quality control capabilities for evaluating data prior to producing NPCR relative survival estimates.

A model of adaptation of overseas nurses: exploring the experiences of Japanese nurses working in Australia.

PubMed

Kishi, Yuka; Inoue, Kumiyo; Crookes, Patrick; Shorten, Allison

2014-04-01

The purpose of the study was to investigate the experiences of Japanese nurses and their adaptation to their work environment in Australia. Using a qualitative research method and semistructured interviews, the study aimed to discover, describe, and analyze the experiences of 14 Japanese nurses participating in the study. A qualitative study. Fourteen Japanese registered nurses working in Australian hospitals participated in the study. Individual semistructured interviews were conducted from April to June in 2008. Thematic analysis was used to identify themes within the data. Analysis of qualitative open-ended questions revealed the participants' adaptation process. It consists of three themes or phases: seeking (S), acclimatizing (A), and settling (S), subsequently named the S.A.S. model. The conceptual model of the adaptation processes of 14 Japanese nurses working in Australia includes the seeking, acclimatizing, and settling phases. Although these phases are not mutually exclusive and the process is not necessarily uniformly linear, all participants in this study passed through this S.A.S. model in order to adapt to their new environment. The S.A.S. model of adaptation helps to describe the experiences of Japanese overseas qualified nurses working in Australian hospitals. Future research is needed to examine whether this model can be applied to nurses from other countries and in other settings outside Australia.

[The characteristics of the development of an adaptation syndrome in severe gestosis].

PubMed

Ivanchenko, S A

2000-01-01

Basic metabolic pathways were studied of formation of the adaptive syndrome in the organism of patients with grave gestoses: glycolysis, gluconeogenesis, and pentosephosphate pathway of production of nicotinamide coenzymes. It has been found out that a stressful character of reconstruction of metabolic homeostasis tends to change the processes of glycolysis and gluconeogenesis that had come to be formed by evolution. This warrants further study, its purpose being a specific correction of intracellular metabolism and prevention of complications. Ozonohemo- and antioxidant therapy in a complex of intensive treatment measures for patients with severe gestoses make for stimulation of pentosephosphate pathway and glycolysis.

[Adaptation strategies faced with chronic pain].

PubMed

Bioy, Antoine

2017-05-01

Chronic pain constitutes a challenge for patients. It makes them uneasy with regard to their personality, their corporality and their life balance, and leaves long-lasting effects on their experience as a patient. The development of adaptation strategies and resources to deal with chronic pain is therefore essential. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

CTTITEM: SAS macro and SPSS syntax for classical item analysis.

PubMed

Lei, Pui-Wa; Wu, Qiong

2007-08-01

This article describes the functions of a SAS macro and an SPSS syntax that produce common statistics for conventional item analysis including Cronbach's alpha, item difficulty index (p-value or item mean), and item discrimination indices (D-index, point biserial and biserial correlations for dichotomous items and item-total correlation for polytomous items). These programs represent an improvement over the existing SAS and SPSS item analysis routines in terms of completeness and user-friendliness. To promote routine evaluations of item qualities in instrument development of any scale, the programs are available at no charge for interested users. The program codes along with a brief user's manual that contains instructions and examples are downloadable from suen.ed.psu.edu/-pwlei/plei.htm.

Centriolar SAS-7 acts upstream of SPD-2 to regulate centriole assembly and pericentriolar material formation

PubMed Central

Sugioka, Kenji; Hamill, Danielle R; Lowry, Joshua B; McNeely, Marie E; Enrick, Molly; Richter, Alyssa C; Kiebler, Lauren E; Priess, James R; Bowerman, Bruce

2017-01-01

The centriole/basal body is a eukaryotic organelle that plays essential roles in cell division and signaling. Among five known core centriole proteins, SPD-2/Cep192 is the first recruited to the site of daughter centriole formation and regulates the centriolar localization of the other components in C. elegans and in humans. However, the molecular basis for SPD-2 centriolar localization remains unknown. Here, we describe a new centriole component, the coiled-coil protein SAS-7, as a regulator of centriole duplication, assembly and elongation. Intriguingly, our genetic data suggest that SAS-7 is required for daughter centrioles to become competent for duplication, and for mother centrioles to maintain this competence. We also show that SAS-7 binds SPD-2 and regulates SPD-2 centriolar recruitment, while SAS-7 centriolar localization is SPD-2-independent. Furthermore, pericentriolar material (PCM) formation is abnormal in sas-7 mutants, and the PCM-dependent induction of cell polarity that defines the anterior-posterior body axis frequently fails. We conclude that SAS-7 functions at the earliest step in centriole duplication yet identified and plays important roles in the orchestration of centriole and PCM assembly. DOI: http://dx.doi.org/10.7554/eLife.20353.001 PMID:28092264

The yeast SAS (something about silencing) protein complex contains a MYST-type putative acetyltransferase and functions with chromatin assembly factor ASF1

PubMed Central

Osada, Shigehiro; Sutton, Ann; Muster, Nemone; Brown, Christine E.; Yates, John R.; Sternglanz, Rolf; Workman, Jerry L.

2001-01-01

It is well established that acetylation of histone and nonhistone proteins is intimately linked to transcriptional activation. However, loss of acetyltransferase activity has also been shown to cause silencing defects, implicating acetylation in gene silencing. The something about silencing (Sas) 2 protein of Saccharomyces cerevisiae, a member of the MYST (MOZ, Ybf2/Sas3, Sas2, and TIP60) acetyltransferase family, promotes silencing at HML and telomeres. Here we identify a ∼450-kD SAS complex containing Sas2p, Sas4p, and the tf2f-related Sas5 protein. Mutations in the conserved acetyl-CoA binding motif of Sas2p are shown to disrupt the ability of Sas2p to mediate the silencing at HML and telomeres, providing evidence for an important role for the acetyltransferase activity of the SAS complex in silencing. Furthermore, the SAS complex is found to interact with chromatin assembly factor Asf1p, and asf1 mutants show silencing defects similar to mutants in the SAS complex. Thus, ASF1-dependent chromatin assembly may mediate the role of the SAS complex in silencing. PMID:11731479

The Development of Student’s Activity Sheets (SAS) Based on Multiple Intelligences and Problem-Solving Skills Using Simple Science Tools

NASA Astrophysics Data System (ADS)

Wardani, D. S.; Kirana, T.; Ibrahim, M.

2018-01-01

The aim of this research is to produce SAS based on MI and problem-solving skills using simple science tools that are suitable to be used by elementary school students. The feasibility of SAS is evaluated based on its validity, practicality, and effectiveness. The completion Lesson Plan (LP) implementation and student’s activities are the indicators of SAS practicality. The effectiveness of SAS is measured by indicators of increased learning outcomes and problem-solving skills. The development of SAS follows the 4-D (define, design, develop, and disseminate) phase. However, this study was done until the third stage (develop). The written SAS was then validated through expert evaluation done by two experts of science, before its is tested to the target students. The try-out of SAS used one group with pre-test and post-test design. The result of this research shows that SAS is valid with “good” category. In addition, SAS is considered practical as seen from the increase of student activity at each meeting and LP implementation. Moreover, it was considered effective due to the significant difference between pre-test and post-test result of the learning outcomes and problem-solving skill test. Therefore, SAS is feasible to be used in learning.

[Multiple bladder diverticula caused by occipital horn syndrome].

PubMed

Legros, L; Revencu, N; Nassogne, M-C; Wese, F-X; Feyaerts, A

2015-11-01

We report on the case of a child who presented with recurrent, multiple, and voluminous bladder diverticula. Bladder diverticula are defined as a herniation of the mucosa through the bladder muscle or the detrusor. Causes are numerous and diverticula can be classified into primary congenital diverticula (para-ureteral - or Hutch diverticula - and posterolateral diverticula); secondary diverticula (resulting from chronic mechanical obstruction or from neurological disease; and diverticula secondary to connective tissue or muscle fragility. The latter is seen in disease entities such as prune belly syndrome, Ehlers-Danlos syndrome, cutis laxa syndrome, OHS (occipital horn syndrome), Menkes disease, and Williams-Beuren syndrome. In this patient, the cause of these diverticula was OHS, a genetic, recessive X-chromosome-linked syndrome, responsible for abnormal tissue caused by a disorder in copper metabolism. This case reminds us of the importance of pushing the diagnostic workup when presented with multiple and/or large bladder diverticula, and in particular to search for rare malformation syndromes after exclusion of an obstacle. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Physicochemical properties and oral bioavailability of ursolic acid nanoparticles using supercritical anti-solvent (SAS) process.

PubMed

Yang, Lei; Sun, Zhen; Zu, Yuangang; Zhao, Chunjian; Sun, Xiaowei; Zhang, Zhonghua; Zhang, Lin

2012-05-01

The objective of the study was to prepare ursolic acid (UA) nanoparticles using the supercritical anti-solvent (SAS) process and evaluate its physicochemical properties and oral bioavailability. The effects of four process variables, pressure, temperature, drug concentration and drug solution flow rate, on drug particle formation during SAS process, were investigated. Particles with mean particle size ranging from 139.2±19.7 to 1039.8±65.2nm were obtained by varying the process parameters. The UA was characterised by scanning electron microscopy, X-ray diffraction, Fourier-transform infrared spectroscopy, thermal gravimetric analysis, specific surface area, dissolution test and bioavailability test. It was concluded that physicochemical properties and bioavailability of crystalline UA could be improved by physical modification, such as particle size reduction and generation of amorphous state using SAS process. Further, SAS process was a powerful methodology for improving the physicochemical properties and bioavailability of UA. Copyright © 2011 Elsevier Ltd. All rights reserved.

Validation of an adapted arabic version of fibromyalgia syndrome impact questionnaire.

PubMed

El-Naby, Mai Abd; Hefny, Mohamed Ahmed; Fahim, Ayman Ekram; Awadalla, Magdy Ahmed

2013-10-01

Fibromyalgia (FM) is the most common chronic pain syndrome encountered in medical practice, affecting females more than males, and the estimated prevalence of FM in Egypt is 1.3 %. The aim was to translate and adapt the Fibromyalgia Impact Questionnaire (FIQ) into Arabic and assess reliability and validity. The Arabic version of Fibromyalgia Impact Questionnaire (FIQ-A) was adapted following the forward/backward translation approach. Fifty-one female patients with FM were studied to assess psychometric properties of the FIQ-A. Reliability was analyzed by the correlation coefficient between test and retest. Internal consistency was checked by the Cronbach's alpha coefficient. Construct validity was assessed comparing FIQ-A with Health Assessment Questionnaire (HAQ), Health Assessment Questionnaire of Fibromyalgia (FHAQ), The Medical Outcome Survey Short-Form-36 (SF-36), and the Total Visual Analog Scale (TVAS) for FM symptom, and feasibility was assessed by the time taken in completing the FIQ-A and the proportion of patients completed the questionnaire. Patients studied were 33.2 ± 9.8 years old. Translation was concordant. Adaptation affected 4 sub-items of physical function. Test-retest correlation coefficient was 0.89 for total FIQ-A and Cronbach's alpha was 0.76. Excellent to good statistically significant correlations (p < 0.05) were found between the FIQ-A items and HAQ, FHAQ, and SF-36. The FIQ-A is a reliable, valid for measuring health status and physical function in Arabic-speaking FM patients.

Adapting the Mullen Scales of Early Learning for a Standardized Measure of Development in Children with Rett Syndrome

ERIC Educational Resources Information Center

Clarkson, Tessa; LeBlanc, Jocelyn; DeGregorio, Geneva; Vogel-Farley, Vanessa; Barnes, Katherine; Kaufmann, Walter E.; Nelson, Charles A.

2017-01-01

Rett Syndrome (RTT) is characterized by severe impairment in fine motor (FM) and expressive language (EL) function, making accurate evaluations of development difficult with standardized assessments. In this study, the administration and scoring of the Mullen Scales of Early Learning (MSEL) were adapted to eliminate the confounding effects of FM…

The Adaptive Behaviour Dementia Questionnaire (ABDQ): Screening Questionnaire for Dementia in Alzheimer's Disease in Adults with Down Syndrome

ERIC Educational Resources Information Center

Prasher, V.; Farooq, A.; Holder, R.

2004-01-01

The diagnosis of dementia in Alzheimer's disease remains at times problematic in adults with intellectual disability. The analysis of 5-year consecutive data developed a researched-based clinical screening tool for dementia in Alzheimer's disease in adults with Down syndrome. The Adaptive Behaviour Dementia Questionnaire (ABDQ) is a 15-item…

[Schnitzler's syndrome].

PubMed

Henry, B; Néel, A; Barbarot, S; Masseau, A; Hamidou, M

2013-04-01

Schnitzler syndrome (SS) is a rare clinical entity, which belongs to the spectrum of monoclonal gammapathy-associated systemic disorders. Its pathophysiology remains elusive, even if it is tempting to consider it as a late onset and probably acquired auto-inflammatory syndrome. SS mainly occurs in the fifth and sixth decade, and present with an urticariform rash with periodic fever and/or osteoarticular pain. Systemic inflammation and monoclonal gammapathy (overwhelmingly IgM kappa) are constant features. SS is a chronic disease, which can severely impair quality of life of the affected individuals. Many drugs have been used and proved disappointing. In the last few years, accumulating reports provided evidence for the dramatic efficacy of anakinra, which has revolutionized the management of most severe cases. The main long-term threat to these patients is to develop a lymphoproliferative disorder (mainly Waldenström's macroglobulinemia). The mechanisms underlying the different facets of the disease remain to be elucidated. Copyright © 2013. Published by Elsevier SAS.

PS3-21: Extracting Utilization Data from Clarity into VDW Using Oracle and SAS

PubMed Central

Chimmula, Srivardhan

2013-01-01

Background/Aims The purpose of the presentation is to demonstrate how we use SAS and Oracle to load VDW_Utilization, VDW_DX, and VDW_PX tables from Clarity at the Kaiser Permanente Northern California (KPNC) Division of Research (DOR) site. Methods DOR uses the best of Oracle PL/ SQL and SAS capabilities in building Extract Transform and Load (ETL) processes. These processes extract patient encounter, diagnosis, and procedure data from Teradata-based Clarity. The data is then transformed to fit HMORN’s VDW definitions of the table. This data is then loaded into the Oracle-based VDW table on DOR’s research database and then finally a copy of the table is also created as a SAS dataset. Results DOR builds robust and efficient ETL processes that refresh VDW Utilization table on a monthly basis processing millions of records/observations. The ETL processes have the capability to identify daily changes in Clarity and update the VDW tables on a daily basis. Conclusions KPNC DOR combines the best of both Oracle and SAS worlds to build ETL processes that load the data into VDW Utilization tables efficiently.

[Laron syndrome: Presentation, treatment and prognosis].

PubMed

Latrech, Hanane; Polak, Michel

2016-01-01

Laron syndrome is a rare cause of short stature due to an abnormality of growth hormone receptor (GHR). It is characterized by poor phenotype-genotype correlation and geographic predilection essentially in the Mediterranean rim, the Middle East and Indian subcontinent. This syndrome corresponds to an endogenous and exogenous complete insensitivity of GH and manifests by early hypoglycemia, an extremely severe short stature and dysmorphic features contrasting with high levels of circulating GH. To date, treatment with recombinant IGF1 is the only treatment option that has improved the terrible prognosis in these patients but does not actually realize the conditions for genuine replacement therapy. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Munchausen syndrome by proxy and pediatric nephrology.

PubMed

Bertulli, Cristina; Cochat, Pierre

2017-11-01

Munchausen syndrome by proxy is a persistent fabrication of illness done by a person to another. Renal and urologic forms of this syndrome are not as uncommon as can be thought; a review of all the cases of Munchausen-by-proxy syndrome reveals that 25% of the children had renal or urologic issues. This syndrome can result in a serious diagnostic dilemma for the physicians; knowing this entity can allow early recognition of falsification and limit the physical and psychological damages caused in the victim. In this study, we reviewed the pediatric nephrology cases of Munchausen syndrome by proxy, grouping them through the principal signs of presentation. Copyright © 2017 Société francophone de néphrologie, dialyse et transplantation. Published by Elsevier Masson SAS. All rights reserved.

Conserved TCP domain of Sas-4/CPAP is essential for pericentriolar material tethering during centrosome biogenesis

PubMed Central

Zheng, Xiangdong; Gooi, Li Ming; Wason, Arpit; Gabriel, Elke; Mehrjardi, Narges Zare; Yang, Qian; Zhang, Xingrun; Debec, Alain; Basiri, Marcus L.; Avidor-Reiss, Tomer; Pozniakovsky, Andrei; Poser, Ina; Šarić, Tomo; Hyman, Anthony A.; Li, Haitao; Gopalakrishnan, Jay

2014-01-01

Pericentriolar material (PCM) recruitment to centrioles forms a key step in centrosome biogenesis. Deregulation of this process leads to centrosome aberrations causing disorders, one of which is autosomal recessive primary microcephaly (MCPH), a neurodevelopmental disorder where brain size is reduced. During PCM recruitment, the conserved centrosomal protein Sas-4/CPAP/MCPH6, known to play a role in centriole formation, acts as a scaffold for cytoplasmic PCM complexes to bind and then tethers them to centrioles to form functional centrosomes. To understand Sas-4’s tethering role, we determined the crystal structure of its T complex protein 10 (TCP) domain displaying a solvent-exposed single-layer of β-sheets fold. This unique feature of the TCP domain suggests that it could provide an “extended surface-like” platform to tether the Sas-4–PCM scaffold to a centriole. Functional studies in Drosophila, human cells, and human induced pluripotent stem cell-derived neural progenitor cells were used to test this hypothesis, where point mutations within the 9–10th β-strands (β9–10 mutants including a MCPH-associated mutation) perturbed PCM tethering while allowing Sas-4/CPAP to scaffold cytoplasmic PCM complexes. Specifically, the Sas-4 β9–10 mutants displayed perturbed interactions with Ana2, a centrosome duplication factor, and Bld-10, a centriole microtubule-binding protein, suggesting a role for the β9–10 surface in mediating protein–protein interactions for efficient Sas-4–PCM scaffold centriole tethering. Hence, we provide possible insights into how centrosomal protein defects result in human MCPH and how Sas-4 proteins act as a vehicle to tether PCM complexes to centrioles independent of its well-known role in centriole duplication. PMID:24385583

Ocular dermoid in Pai Syndrome: A review.

PubMed

Tormey, Peter; Bilic Cace, Iva; Boyle, Michael A

2017-04-01

Pai Syndrome is a rare congenital malformation syndrome of unknown cause with hypertelorism, midline cleft lip, nasal and facial polyps, ocular anomalies and the presence of distinctive lipomas adjacent to the corpus callosum. Herein, we present an infant girl with Pai Syndrome diagnosed in the first week of life with typical facial findings and associated pericallosal lipoma identified on cranial ultrasound and brain MRI. These typical features identified included median cleft of the upper lip (in her case as a forme fruste) with a cleft alveolus and a mid-anterior alveolar process congenital polyp. In addition to these findings there was mild hypertelorism and an ocular abnormality on the right eye. An ophthalmology assessment on day 5 identified the ocular lesion as a limbal dermoid. Several ocular anomalies have been reported in association with Pai Syndrome, however, dermoids have not been frequently described in this Syndrome and not before in a limbal location. Increasing identification of previously unreported ocular abnormalities in Pai Syndrome may improve diagnosis and may prove useful in future work attempting to elucidate the aetiology of this rare syndrome. Copyright © 2017. Published by Elsevier Masson SAS.

Adaptation of a Biomarker-Based Sepsis Mortality Risk Stratification Tool for Pediatric Acute Respiratory Distress Syndrome.

PubMed

Yehya, Nadir; Wong, Hector R

2018-01-01

-based risk stratification tool designed for pediatric sepsis was adapted for use in pediatric acute respiratory distress syndrome. The newly derived Pediatric Acute Respiratory Distress Syndrome Biomarker Risk Model demonstrates good test characteristics internally and requires external validation in a larger cohort. Tools such as Pediatric Acute Respiratory Distress Syndrome Biomarker Risk Model have the potential to provide improved risk stratification and prognostic enrichment for future trials in pediatric acute respiratory distress syndrome.

Mechanisms of Host Receptor Adaptation by Severe Acute Respiratory Syndrome Coronavirus

DOE Office of Scientific and Technical Information (OSTI.GOV)

Wu, Kailang; Peng, Guiqing; Wilken, Matthew

The severe acute respiratory syndrome coronavirus (SARS-CoV) from palm civets has twice evolved the capacity to infect humans by gaining binding affinity for human receptor angiotensin-converting enzyme 2 (ACE2). Numerous mutations have been identified in the receptor-binding domain (RBD) of different SARS-CoV strains isolated from humans or civets. Why these mutations were naturally selected or how SARS-CoV evolved to adapt to different host receptors has been poorly understood, presenting evolutionary and epidemic conundrums. In this study, we investigated the impact of these mutations on receptor recognition, an important determinant of SARS-CoV infection and pathogenesis. Using a combination of biochemical, functional,more » and crystallographic approaches, we elucidated the molecular and structural mechanisms of each of these naturally selected RBD mutations. These mutations either strengthen favorable interactions or reduce unfavorable interactions with two virus-binding hot spots on ACE2, and by doing so, they enhance viral interactions with either human (hACE2) or civet (cACE2) ACE2. Therefore, these mutations were viral adaptations to either hACE2 or cACE2. To corroborate the above analysis, we designed and characterized two optimized RBDs. The human-optimized RBD contains all of the hACE2-adapted residues (Phe-442, Phe-472, Asn-479, Asp-480, and Thr-487) and possesses exceptionally high affinity for hACE2 but relative low affinity for cACE2. The civet-optimized RBD contains all of the cACE2-adapted residues (Tyr-442, Pro-472, Arg-479, Gly-480, and Thr-487) and possesses exceptionally high affinity for cACE2 and also substantial affinity for hACE2. These results not only illustrate the detailed mechanisms of host receptor adaptation by SARS-CoV but also provide a molecular and structural basis for tracking future SARS-CoV evolution in animals.« less

Mechanisms of Host Receptor Adaptation by Severe Acute Respiratory Syndrome Coronavirus*

PubMed Central

Wu, Kailang; Peng, Guiqing; Wilken, Matthew; Geraghty, Robert J.; Li, Fang

2012-01-01

The severe acute respiratory syndrome coronavirus (SARS-CoV) from palm civets has twice evolved the capacity to infect humans by gaining binding affinity for human receptor angiotensin-converting enzyme 2 (ACE2). Numerous mutations have been identified in the receptor-binding domain (RBD) of different SARS-CoV strains isolated from humans or civets. Why these mutations were naturally selected or how SARS-CoV evolved to adapt to different host receptors has been poorly understood, presenting evolutionary and epidemic conundrums. In this study, we investigated the impact of these mutations on receptor recognition, an important determinant of SARS-CoV infection and pathogenesis. Using a combination of biochemical, functional, and crystallographic approaches, we elucidated the molecular and structural mechanisms of each of these naturally selected RBD mutations. These mutations either strengthen favorable interactions or reduce unfavorable interactions with two virus-binding hot spots on ACE2, and by doing so, they enhance viral interactions with either human (hACE2) or civet (cACE2) ACE2. Therefore, these mutations were viral adaptations to either hACE2 or cACE2. To corroborate the above analysis, we designed and characterized two optimized RBDs. The human-optimized RBD contains all of the hACE2-adapted residues (Phe-442, Phe-472, Asn-479, Asp-480, and Thr-487) and possesses exceptionally high affinity for hACE2 but relative low affinity for cACE2. The civet-optimized RBD contains all of the cACE2-adapted residues (Tyr-442, Pro-472, Arg-479, Gly-480, and Thr-487) and possesses exceptionally high affinity for cACE2 and also substantial affinity for hACE2. These results not only illustrate the detailed mechanisms of host receptor adaptation by SARS-CoV but also provide a molecular and structural basis for tracking future SARS-CoV evolution in animals. PMID:22291007

Social Approach and Autistic Behavior in Children with Fragile X Syndrome

ERIC Educational Resources Information Center

Roberts, Jane E.; Weisenfeld, Leigh Anne H.; Hatton, Deborah D.; Heath, Morgan; Kaufmann, Walter E.

2007-01-01

Social avoidance is a core phenotypic characteristic of fragile X syndrome (FXS) that has critical cognitive and social consequences. However, no study has examined modulation of multiple social avoidant behaviors in children with FXS. In the current study, we introduce the "Social Approach Scale" (SAS), an observation scale that includes physical…

A SAS Interface for Bayesian Analysis with WinBUGS

ERIC Educational Resources Information Center

Zhang, Zhiyong; McArdle, John J.; Wang, Lijuan; Hamagami, Fumiaki

2008-01-01

Bayesian methods are becoming very popular despite some practical difficulties in implementation. To assist in the practical application of Bayesian methods, we show how to implement Bayesian analysis with WinBUGS as part of a standard set of SAS routines. This implementation procedure is first illustrated by fitting a multiple regression model…

Usual Dietary Intakes: SAS Macros for Fitting Multivariate Measurement Error Models & Estimating Multivariate Usual Intake Distributions

Cancer.gov

The following SAS macros can be used to create a multivariate usual intake distribution for multiple dietary components that are consumed nearly every day or episodically. A SAS macro for performing balanced repeated replication (BRR) variance estimation is also included.

[From rehabilitation to re-adaptation, preparing the return home].

PubMed

Froger, Jérôme; Jourdan, Claire; Dabek, Marie-Christine; Petitqueux, Sophie; Gardes, Raphaël; Dupeyron, Arnaud

2017-03-01

After a serious head trauma, the return home constitutes a key moment in the patient's reintegration. It is prepared by a multi-disciplinary team throughout the rehabilitation and re-adaptation process, taking into account the patient's prognosis for recovery. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

[Glaucoma and sleep apnea syndrome].

PubMed

Blumen-Ohana, E; Blumen, M; Aptel, F; Nordmann, J P

2011-06-01

Glaucomatous optic neuropathy is multifactorial, with currently one known and modifiable risk factor, with good results on the prognosis and intraocular pressure. Nevertheless, some patients may experience progression of their neuropathy even though their intraocular pressure seems appropriately controlled. Vascular risk factors are hypothesized and researched. Obstructive sleep apnea syndrome (OSAS) could be considered one of these risk factors. Screening for this cardiovascular risk factor in glaucomatous patients presenting evocative signs, should be proposed. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Using SAS PROC MCMC for Item Response Theory Models

ERIC Educational Resources Information Center

Ames, Allison J.; Samonte, Kelli

2015-01-01

Interest in using Bayesian methods for estimating item response theory models has grown at a remarkable rate in recent years. This attentiveness to Bayesian estimation has also inspired a growth in available software such as WinBUGS, R packages, BMIRT, MPLUS, and SAS PROC MCMC. This article intends to provide an accessible overview of Bayesian…

Recent high energy gamma-ray results from SAS-2

NASA Technical Reports Server (NTRS)

Thompson, D. J.; Fichtel, C. E.; Hartman, R. C.; Kniffen, D. A.; Bignami, G. F.; Ogelman, H. B.; Ozel, M. E.; Tumer, T.; Lamb, R. C.

1977-01-01

Recent developments in gamma-ray astronomy due to the results from SAS-2 have focused on two areas. First, the emission from the plane of the Galaxy is the dominant feature in the gamma-ray sky. The galactic latitude and longitude distributions are consistent with the concept that the high-energy radiation originates from cosmic rays interacting with interstellar matter, and the measurements support a galactic origin for cosmic rays. Second, searches of the SAS-2 data for emission from localized sources have shown three strong discrete gamma-ray sources: the Crab nebula and PSR 0531 + 21, the Vela supernova remnant and PSR 0833-45, and a source near galactic coordinates 193 deg longitude, +3 deg latitude, which does not appear to be associated with other known celestial objects. Evidence has also been found for pulsed gamma-ray emission from two other radio pulsars, PSR 1818-04 and PSR 1747-46. A localized source near longitudes 76-80 deg may be associated with the X-ray source Cyg X-3.

POWERLIB: SAS/IML Software for Computing Power in Multivariate Linear Models

PubMed Central

Johnson, Jacqueline L.; Muller, Keith E.; Slaughter, James C.; Gurka, Matthew J.; Gribbin, Matthew J.; Simpson, Sean L.

2014-01-01

The POWERLIB SAS/IML software provides convenient power calculations for a wide range of multivariate linear models with Gaussian errors. The software includes the Box, Geisser-Greenhouse, Huynh-Feldt, and uncorrected tests in the “univariate” approach to repeated measures (UNIREP), the Hotelling Lawley Trace, Pillai-Bartlett Trace, and Wilks Lambda tests in “multivariate” approach (MULTIREP), as well as a limited but useful range of mixed models. The familiar univariate linear model with Gaussian errors is an important special case. For estimated covariance, the software provides confidence limits for the resulting estimated power. All power and confidence limits values can be output to a SAS dataset, which can be used to easily produce plots and tables for manuscripts. PMID:25400516

SAS macro programs for geographically weighted generalized linear modeling with spatial point data: applications to health research.

PubMed

Chen, Vivian Yi-Ju; Yang, Tse-Chuan

2012-08-01

An increasing interest in exploring spatial non-stationarity has generated several specialized analytic software programs; however, few of these programs can be integrated natively into a well-developed statistical environment such as SAS. We not only developed a set of SAS macro programs to fill this gap, but also expanded the geographically weighted generalized linear modeling (GWGLM) by integrating the strengths of SAS into the GWGLM framework. Three features distinguish our work. First, the macro programs of this study provide more kernel weighting functions than the existing programs. Second, with our codes the users are able to better specify the bandwidth selection process compared to the capabilities of existing programs. Third, the development of the macro programs is fully embedded in the SAS environment, providing great potential for future exploration of complicated spatially varying coefficient models in other disciplines. We provided three empirical examples to illustrate the use of the SAS macro programs and demonstrated the advantages explained above. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Fluid control mechanisms in weightlessness

NASA Technical Reports Server (NTRS)

Leach, Carolyn S.

1987-01-01

Experiments performed on Space Shuttle flights have emphasized study of the earliest effects of the cephalad fluid shift resulting from microgravity. Analysis of one subject's urine collected during flight showed that a sharp increase in antidiuretic hormone occurred within 2 h of launch, followed by an increase in cortisol excretion. Although this subject had symptoms of the space adaptation syndrome (SAS), inflight data from Spacelab missions suggested that these transient changes were not caused by SAS. Unpaired t-tests and Mann-Whitney tests showed that before and after flight, plasma thyroxine and urine osmolality were significantly higher in Shuttle crewmembers who exhibited more severe symptoms of SAS than in asymptomatic crewmembers.

Impact of sacubitril–valsartan combination in patients with chronic heart failure and sleep apnoea syndrome: the ENTRESTO‐SAS study design

PubMed Central

Jaffuel, Dany; Molinari, Nicolas; Berdague, Philippe; Pathak, Atul; Galinier, Michel; Dupuis, Marion; Ricci, Jean‐Etienne; Mallet, Jean‐Pierre; Bourdin, Arnaud

2018-01-01

Abstract Aims Sleep‐disordered breathing (SDB) is a highly prevalent co‐morbidity in patients with chronic heart failure (CHF) and can play a detrimental role in the pathophysiology course of CHF. However, the best way to manage SDB in CHF remains a matter of debate. Sacubitril–valsartan has been included in the 2016 European Society of Cardiology guidelines as an alternative to angiotensin‐converting enzyme inhibitors to further reduce the risk of progression of CHF, CHF hospitalization, and death in ambulatory patients. Sacubitril and valsartan are good candidates for correcting SDB of CHF patients because their known mechanisms of action are likely to counteract the pathophysiology of SDB in CHF. Methods and results The ENTRESTO‐SAS trial is a 3‐month, multicentric, prospective, open‐label real‐life cohort study. Patients eligible for sacubitril–valsartan treatment (i.e. adults with left ventricular ejection fraction ≤35%, who remain symptomatic despite optimal treatment with an angiotensin‐converting enzyme inhibitor, a beta‐blocker, and a mineralocorticoid receptor antagonist) will be evaluated before and after 3 months of treatment (nocturnal ventilatory polygraphy, echocardiography, laboratory testing, and quality‐of‐life and SDB questionnaires). The primary outcome is the change in the Apnoea–Hypopnoea Index, before and after 3 months of treatment. One hundred twenty patients are required to detect a significant 20% improvement of the Apnoea–Hypopnoea Index with a power of 90% at an alpha risk of 5%. Conclusions In the context of the SERVE‐HF study, physicians are waiting for new trials and alternative therapies. We sought to assess in the ENTRESTO‐SAS trial whether sacubitril–valsartan could improve the outcome of SDB in CHF patients. PMID:29469206

[Treatment and management after acute coronary syndrome without ST-elevation].

PubMed

Drogoul, Laurent; Scarlatti, Didier; Ferrari, Emile

2010-03-01

Coronary syndromes without ST elevation, previously known as unstable angina, are now more frequent than ST elevation myocardial infarction. Evidence-based studies should guide their management after hospital discharge. This management seeks to fulfill precise objectives and has been demonstrated to be effective in terms of survival. Copyright (c) 2009 Elsevier Masson SAS. All rights reserved.

[Kindler syndrome: clinical and ultra-structural particularities, a propos of three cases].

PubMed

El Fekih, Nadia; Mahfoudh, Anis; Zekri, Samy; Kharfi, Monia; Fazaa, Bécima; Jaafoura, Mohamed Habib; Kamoun, Mohamed Ridha

2011-08-01

Kindler's syndrome is a rare type of genetic skin condition belonging to the class of bullous poikilodermia. We report three new sibling cases of this rare syndrome. The condition was seen in three sisters aged 12, 16 and 20 years, born of a first-degree consanguineous marriage with no family history of Kindler's syndrome. The three patients presented spontaneously regressive bullous eruptions, poikilodermia of gradual onset, major cutaneous atrophy on the back of the hands and the feet, photosensitivity and gingival hypertrophy. Electron microscopy examination of poikilodermic skin showed normal anchoring filaments and intraepidermal cleavage. Diagnosis of Kindler's syndrome is based upon clinical evidence. Kidler's syndrome is a well defined clinical entity. Ultra-structural studies show intraepidermal, junctional, and dermal cleavage. This syndrome must be differentiated from congenital epidermolysis bullosa, Weary's syndrome, and other bullous hereditary poikilodermas. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

[A parotitis as primary infection of Lemierre's syndrome].

PubMed

Valleix, B; Floccard, B; Hautin, E; Faure, F; Allaouchiche, B

2011-09-01

Lemierre's syndrome is a classical presentation of human necrobacillosis. It is characterized by a primary infection in the face including a septic thrombophlebitis of the internal jugular vein and disseminated metastatic abcesses. Fusobacterium necrophorum is the main pathogen found in that syndrome. The diagnosis is based on clinical features, then on the microbiology with positive anaerobic blood cultures as key role and finally on the computed tomography. Most of the time a well-chosen antibiotic treatment against anaerobic pathogens and Gram negative bacilli is efficient but surgery can be useful. We report a case of a 73 years old man, which seems to be unique because it is the first case reported of a Lemierre's syndrome characterized by a parotitis infected by F. necrophorum. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

PSHREG: A SAS macro for proportional and nonproportional subdistribution hazards regression

PubMed Central

Kohl, Maria; Plischke, Max; Leffondré, Karen; Heinze, Georg

2015-01-01

We present a new SAS macro %pshreg that can be used to fit a proportional subdistribution hazards model for survival data subject to competing risks. Our macro first modifies the input data set appropriately and then applies SAS's standard Cox regression procedure, PROC PHREG, using weights and counting-process style of specifying survival times to the modified data set. The modified data set can also be used to estimate cumulative incidence curves for the event of interest. The application of PROC PHREG has several advantages, e.g., it directly enables the user to apply the Firth correction, which has been proposed as a solution to the problem of undefined (infinite) maximum likelihood estimates in Cox regression, frequently encountered in small sample analyses. Deviation from proportional subdistribution hazards can be detected by both inspecting Schoenfeld-type residuals and testing correlation of these residuals with time, or by including interactions of covariates with functions of time. We illustrate application of these extended methods for competing risk regression using our macro, which is freely available at: http://cemsiis.meduniwien.ac.at/en/kb/science-research/software/statistical-software/pshreg, by means of analysis of a real chronic kidney disease study. We discuss differences in features and capabilities of %pshreg and the recent (January 2014) SAS PROC PHREG implementation of proportional subdistribution hazards modelling. PMID:25572709

Brief Report: Repetitive Behaviour Profiles in Williams syndrome: Cross Syndrome Comparisons with Prader-Willi and Down syndromes.

PubMed

Royston, R; Oliver, C; Moss, J; Adams, D; Berg, K; Burbidge, C; Howlin, P; Nelson, L; Stinton, C; Waite, J

2018-01-01

This study describes the profile of repetitive behaviour in individuals with Williams syndrome, utilising cross-syndrome comparisons with people with Prader-Willi and Down syndromes. The Repetitive Behaviour Questionnaire was administered to caregivers of adults with Williams (n = 96), Prader-Willi (n = 103) and Down (n = 78) syndromes. There were few group differences, although participants with Williams syndrome were more likely to show body stereotypies. Individuals with Williams syndrome also showed more hoarding and less tidying behaviours than those with Down syndrome. IQ and adaptive ability were negatively associated with repetitive questioning in people with Williams syndrome. The profile of repetitive behaviour amongst individuals with Williams syndrome was similar to the comparison syndromes. The cognitive mechanisms underlying these behaviours in genetic syndromes warrant further investigation.

Association between adaptive behaviour and age in adults with Down syndrome without dementia: examining the range and severity of adaptive behaviour problems.

PubMed

Makary, A T; Testa, R; Tonge, B J; Einfeld, S L; Mohr, C; Gray, K M

2015-08-01

Studies on adaptive behaviour and ageing in adults with Down syndrome (DS) (without dementia) have typically analysed age-related change in terms of the total item scores on questionnaires. This research extends the literature by investigating whether the age-related changes in adaptive abilities could be differentially attributed to changes in the number or severity (intensity) of behavioural questionnaire items endorsed. The Adaptive Behaviour Assessment System-II Adult (ABAS-II Adult) was completed by parents and caregivers of 53 adults with DS aged between 16 and 56 years. Twenty adults with DS and their parents/caregivers were a part of a longitudinal study, which provided two time points of data. In addition 33 adults with DS and their parents/caregivers from a cross-sectional study were included. Random effects regression analyses were used to examine the patterns in item scores associated with ageing. Increasing age was found to be significantly associated with lower adaptive behaviour abilities for all the adaptive behaviour composite scores, expect for the practical composite. These associations were entirely related to fewer ABAS-II Adult items being selected as present for the older participants, as opposed to the scores being attributable to lower item severity. This study provides evidence for a differential pattern of age-related change for various adaptive behaviour skills in terms of range, but not severity. Possible reasons for this pattern will be discussed. Overall, these findings suggest that adults with DS may benefit from additional support in terms of their social and conceptual abilities as they age. © 2014 MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.

Sustainable biodegradation of phenol by immobilized Bacillus sp. SAS19 with porous carbonaceous gels as carriers.

PubMed

Ke, Qian; Zhang, Yunge; Wu, Xilin; Su, Xiaomei; Wang, Yuyang; Lin, Hongjun; Mei, Rongwu; Zhang, Yu; Hashmi, Muhammad Zaffar; Chen, Chongjun; Chen, Jianrong

2018-09-15

In this study, high-efficient phenol-degrading bacterium Bacillus sp. SAS19 which was isolated from activated sludge by resuscitation-promoting factor (Rpf) addition, were immobilized on porous carbonaceous gels (CGs) for phenol degradation. The phenol-degrading capabilities of free and immobilized Bacillus sp. SAS19 were evaluated under various initial phenol concentrations. The obtained results showed that phenol could be removed effectively by both free and immobilized Bacillus sp. SAS19. Furthermore, for degradation of phenol at high concentrations, long-term utilization and recycling were more readily achieved for immobilized bacteria as compared to free bacteria. Immobilized bacteria exhibited significant increase in phenol-degrading capabilities in the third cycle of recycling and reuse, which demonstrated 87.2% and 100% of phenol (1600 mg/L) degradation efficiency at 12 and 24 h, respectively. The present study revealed that immobilized Bacillus sp. SAS19 can be potentially used for enhanced treatment of synthetic phenol-laden wastewater. Copyright © 2018 Elsevier Ltd. All rights reserved.

[Irritable bowel syndrome, levator ani syndrome, proctalgia fugax and chronic pelvic and perineal pain].

PubMed

Watier, Alain; Rigaud, Jérôme; Labat, Jean-Jacques

2010-11-01

To define functional gastrointestinal pain, irritable bowel syndrome (IBS), levator ani syndrome, proctalgia fugax, the pathophysiology of these syndromes and the treatments that can be proposed. Review of articles published on the theme based on a Medline (PubMed) search and consensus conferences selected according to their scientific relevance. IBS is very common. Patients report abdominal pain and/or discomfort, bloating, and abnormal bowel habit (diarrhoea, constipation or both), in the absence of any structural or biochemical abnormalities. IBS has a complex, multifactorial pathophysiology, involving biological and psychosocial interactions resulting in dysregulation of the brain-gut axis associated with disorders of intestinal motility, hyperalgesia, immune disorders and disorders of the intestinal bacterial microflora and autonomic and hormonal dysfunction. Many treatments have been proposed, ranging from diet to pharmacology and psychotherapy. Patients with various types of chronic pelvic and perineal pain, especially those seen in urology departments, very often report associated IBS. This syndrome is also part of a global and integrated concept of pelviperineal dysfunction, avoiding a rigorous distinction between the posterior segment and the midline and anterior segments of the perineum. Copyright © 2010 Elsevier Masson SAS. All rights reserved.

Cognitive and adaptive advantages of growth hormone treatment in children with Prader-Willi syndrome.

PubMed

Dykens, Elisabeth M; Roof, Elizabeth; Hunt-Hawkins, Hailee

2017-01-01

People with Prader-Willi syndrome (PWS) typically have mild to moderate intellectual deficits, compulsivity, hyperphagia, obesity, and growth hormone deficiencies. Growth hormone treatment (GHT) in PWS has well-established salutatory effects on linear growth and body composition, yet cognitive benefits of GHT, seen in other patient groups, have not been well studied in PWS. Study 1 included 96 children and youth with PWS aged 4-21 years who naturalistically varied in their exposures to GHT. Controlling for socioeconomic status, analyses compared cognitive and adaptive behavior test scores across age-matched treatment naïve versus growth hormone treated children. Study II assessed if age of treatment initiation or treatment duration was associated with subsequent cognition or adaptive behavior in 127, 4- to 21-year olds with PWS. Study III longitudinally examined cognitive and adaptive behavior in 168 participants who were either consistently on versus off GHT for up to 4-5 years. Compared to the treatment naïve group, children receiving GHT had significantly higher Verbal and Composite IQs, and adaptive communication and daily living skills. Children who began treatment before 12 months of age had higher Nonverbal and Composite IQs than children who began treatment between 1 and 5 years of age. Longitudinally, the groups differed in their intercepts, but not slopes, with each group showing stable IQ and adaptive behavior scores over time. Cognitive and adaptive advantages should be considered an ancillary benefit and additional justification for GHT in people with PWS. Future efforts need to target apparent socioeconomic inequities in accessing GHT in the PWS population. © 2016 Association for Child and Adolescent Mental Health.

Cognitive and adaptive advantages of growth hormone treatment in children with Prader-Willi syndrome

PubMed Central

Dykens, Elisabeth M; Roof, Elizabeth; Hunt-Hawkins, Hailee

2016-01-01

Background People with Prader-Willi syndrome (PWS) typically have mild to moderate intellectual deficits, compulsivity, hyperphagia, obesity, and growth hormone deficiencies. Growth hormone treatment (GHT) in PWS has well-established salutatory effects on linear growth and body composition, yet cognitive benefits of GHT, seen in other patient groups, have not been well studied in PWS. Methods Study 1 included 96 children and youth with PWS aged 4 to 21 years who naturalistically varied in their exposures to GHT. Controlling for socio-economic status, analyses compared cognitive and adaptive behavior test scores across age-matched treatment naïve versus growth hormone treated children. Study II assessed if age of treatment initiation or treatment duration was associated with subsequent cognition or adaptive behavior in 127, 4- to-21 year olds with PWS. Study III longitudinally examined cognitive and adaptive behavior in 168 participants who were either consistently on versus off GHT for up to 4–5 years. Results Compared to the treatment naïve group, children receiving growth hormone treatment had significantly higher Verbal and Composite IQs, and adaptive communication and daily living skills. Children who began treatment before 12 months of age had higher Nonverbal and Composite IQs than children who began treatment between 1 to 5 years of age. Longitudinally, the groups differed in their intercepts, but not slopes, with each group showing stable IQ and adaptive behavior scores over time. Conclusions Cognitive and adaptive advantages should be considered an ancillary benefit and additional justification for GHT in people with PWS. Future efforts need to target apparent socio-economic inequities in accessing GHT in the PWS population. PMID:27481444

[Parotid involvement in Churg-Strauss syndrome].

PubMed

Bonnet, R; Bertin, H; Delemazure, A S; Clairand, R; Mercier, J; Corre, P

2014-06-01

Churg-Strauss syndrome is a rare systemic vascularitis. This disease causes eosinophilic tissue infiltration. The most frequent manifestations are cortico-dependent asthma, mono- or polyneuropathy, paranasal sinus polyposis, and digestive and renal dysfunction. Salivary glands are very rarely involved. We describe a case of CSS in a patient presenting with bilateral parotid swelling. The morphological study of salivary glands revealed an unusual thickening of the salivary duct walls. Salivary gland involvement in Churg and Strauss syndrome can be difficult to demonstrate histologically; it does not usually present in the clinical foreground of the disease, and can be a source of misdiagnosis. The biopsy should be performed in the symptomatic gland, away from any previous corticoid treatment. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Factors associated with severity of sleep apnoea syndrome in patients with chronic kidney disease.

PubMed

Tanaka, Akihito; Inaguma, Daijo; Ito, Eri; Kamegai, Naoki; Kato, Akiko; Mizutani, Minami; Shimogushi, Hiroya; Shinjo, Hibiki; Otsuka, Yasuhiro; Takeda, Asami

2017-08-01

Background In patients with chronic kidney disease (CKD), prevalence of sleep apnoea syndrome (SAS) is reported to be markedly high. However, the factors associated with severity of SAS in such patients rarely have been reported. Methods This was a cross-sectional study of 100 stable non-dialysis patients with CKD who attended a CKD educational programme from April 2014 to August 2015. Diagnosis of SAS and its severity were assessed using a type-3 portable monitor. Results Eighty-six men and 14 women with a mean age of 71.6 ± 9.7 years were included. Mean apnoea-hypopnoea index (AHI) was 26.0 ± 13.8. Severe SAS was seen in 39 patients. Significant differences in brain natriuretic peptide (BNP) level (213.6 ± 329.6 pg/mL vs 107.8 ± 141.3 pg/mL, P < 0.05) and cardiothoracic ratio (CTR, 52.4% ± 6.3% vs 49.6% ± 5.7%, P < 0.05) were seen between patients with and without severe SAS. After adjusting for various parameters, BNP level, CTR, and diameter of the inferior vena cava at the end of inhalation were found to correlate with AHI. Conclusions In patients with CKD, prevalence of severe SAS is extremely high. In these patients, fluid retention, rather than systolic or diastolic function, correlates with severity of SAS.

The General Adaptation Syndrome: Potential misapplications to resistance exercise.

PubMed

Buckner, Samuel L; Mouser, J Grant; Dankel, Scott J; Jessee, Matthew B; Mattocks, Kevin T; Loenneke, Jeremy P

2017-11-01

Within the resistance training literature, one of the most commonly cited tenets with respect to exercise programming is the "General Adaptation Syndrome" (GAS). The GAS is cited as a central theory behind the periodization of resistance exercise. However, after examining the original stress research by Hans Selye, the applications of GAS to resistance exercise may not be appropriate. To examine the original work of Hans Selye, as well as the original papers through which the GAS was established as a central theory for periodized resistance exercise. We conducted a review of Selye's work on the GAS, as well as the foundational papers through which this concept was applied to resistance exercise. The work of Hans Selye focused on the universal physiological stress responses noted upon exposure to toxic levels of a variety of pharmacological agents and stimuli. The extrapolations that have been made to resistance exercise appear loosely based on this concept and may not be an appropriate basis for application of the GAS to resistance exercise. Copyright © 2017 Sports Medicine Australia. Published by Elsevier Ltd. All rights reserved.

SAS6-like protein in Plasmodium indicates that conoid-associated apical complex proteins persist in invasive stages within the mosquito vector.

PubMed

Wall, Richard J; Roques, Magali; Katris, Nicholas J; Koreny, Ludek; Stanway, Rebecca R; Brady, Declan; Waller, Ross F; Tewari, Rita

2016-06-24

The SAS6-like (SAS6L) protein, a truncated paralogue of the ubiquitous basal body/centriole protein SAS6, has been characterised recently as a flagellum protein in trypanosomatids, but associated with the conoid in apicomplexan Toxoplasma. The conoid has been suggested to derive from flagella parts, but is thought to have been lost from some apicomplexans including the malaria-causing genus Plasmodium. Presence of SAS6L in Plasmodium, therefore, suggested a possible role in flagella assembly in male gametes, the only flagellated stage. Here, we have studied the expression and role of SAS6L throughout the Plasmodium life cycle using the rodent malaria model P. berghei. Contrary to a hypothesised role in flagella, SAS6L was absent during gamete flagellum formation. Instead, SAS6L was restricted to the apical complex in ookinetes and sporozoites, the extracellular invasive stages that develop within the mosquito vector. In these stages SAS6L forms an apical ring, as we show is also the case in Toxoplasma tachyzoites. The SAS6L ring was not apparent in blood-stage invasive merozoites, indicating that the apical complex is differentiated between the different invasive forms. Overall this study indicates that a conoid-associated apical complex protein and ring structure is persistent in Plasmodium in a stage-specific manner.

Group Training in Interpersonal Problem-Solving Skills for Workplace Adaptation of Adolescents and Adults with Asperger Syndrome: A Preliminary Study

ERIC Educational Resources Information Center

Bonete, Saray; Calero, María Dolores; Fernández-Parra, Antonio

2015-01-01

Adults with Asperger syndrome show persistent difficulties in social situations which psychosocial treatments may address. Despite the multiple studies focusing on social skills interventions, only some have focused specifically on problem-solving skills and have not targeted workplace adaptation training in the adult population. This study…

ENVIRONMENTAL TECHNOLOGY VERIFICATION REPORT-A AND A ENVIRONMENTAL SEALS, INC., SEAL ASSIST SYSTEM (SAS) PHASE II REPORT

EPA Science Inventory

The Environmental Technology Verification report discusses the technology and performance of Seal Assist System (SAS) for natural gas reciprocating compressor rod packing manufactured by A&A Environmental Seals, Inc. The SAS uses a secondary containment gland to collect natural g...

Impact of sacubitril-valsartan combination in patients with chronic heart failure and sleep apnoea syndrome: the ENTRESTO-SAS study design.

PubMed

Jaffuel, Dany; Molinari, Nicolas; Berdague, Philippe; Pathak, Atul; Galinier, Michel; Dupuis, Marion; Ricci, Jean-Etienne; Mallet, Jean-Pierre; Bourdin, Arnaud; Roubille, François

2018-06-01

Sleep-disordered breathing (SDB) is a highly prevalent co-morbidity in patients with chronic heart failure (CHF) and can play a detrimental role in the pathophysiology course of CHF. However, the best way to manage SDB in CHF remains a matter of debate. Sacubitril-valsartan has been included in the 2016 European Society of Cardiology guidelines as an alternative to angiotensin-converting enzyme inhibitors to further reduce the risk of progression of CHF, CHF hospitalization, and death in ambulatory patients. Sacubitril and valsartan are good candidates for correcting SDB of CHF patients because their known mechanisms of action are likely to counteract the pathophysiology of SDB in CHF. The ENTRESTO-SAS trial is a 3-month, multicentric, prospective, open-label real-life cohort study. Patients eligible for sacubitril-valsartan treatment (i.e. adults with left ventricular ejection fraction ≤35%, who remain symptomatic despite optimal treatment with an angiotensin-converting enzyme inhibitor, a beta-blocker, and a mineralocorticoid receptor antagonist) will be evaluated before and after 3 months of treatment (nocturnal ventilatory polygraphy, echocardiography, laboratory testing, and quality-of-life and SDB questionnaires). The primary outcome is the change in the Apnoea-Hypopnoea Index, before and after 3 months of treatment. One hundred twenty patients are required to detect a significant 20% improvement of the Apnoea-Hypopnoea Index with a power of 90% at an alpha risk of 5%. In the context of the SERVE-HF study, physicians are waiting for new trials and alternative therapies. We sought to assess in the ENTRESTO-SAS trial whether sacubitril-valsartan could improve the outcome of SDB in CHF patients. © 2018 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of the European Society of Cardiology.

Data set and SAS analysis of the effects of in utero Dipentyl ...

EPA Pesticide Factsheets

there is no abstract, this is a dataset A mathematical statistician in EPA, NCEA has requested the attached data from us for analysis. The file includes individual fetal male rat testosterone production and identifying information about the dose of the chemical and the litter of origin. It also includes the SAS data file, SAS commands and output. These data were used in our recent publication Gray LE Jr, Furr J, Tatum-Gibbs KR, Lambright C, Sampson H, Hannas BR, Wilson VS, Hotchkiss A, Foster PM. Establishing the "Biological Relevance" of Dipentyl Phthalate Reductions in Fetal Rat Testosterone Production and Plasma and Testis Testosterone Levels. Toxicol Sci. 2016 Jan;149(1):178-91. doi: 10.1093/toxsci/kfv224.

Staphylococcal toxic shock syndrome: superantigen-mediated enhancement of endotoxin shock and adaptive immune suppression.

PubMed

Kulhankova, Katarina; King, Jessica; Salgado-Pabón, Wilmara

2014-08-01

Infectious diseases caused by Staphylococcus aureus present a significant clinical and public health problem. S. aureus causes some of the most severe hospital-associated and community-acquired illnesses. Specifically, it is the leading cause of infective endocarditis and osteomyelitis, and the second leading cause of sepsis in the USA. While pathogenesis of S. aureus infections is at the center of current research, many questions remain about the mechanisms underlying staphylococcal toxic shock syndrome (TSS) and associated adaptive immune suppression. Both conditions are mediated by staphylococcal superantigens (SAgs)-secreted staphylococcal toxins that are major S. aureus virulence factors. Toxic shock syndrome toxin-1 (TSST-1) is the SAg responsible for almost all menstrual TSS cases in the USA. TSST-1, staphylococcal enterotoxin B and C are also responsible for most cases of non-menstrual TSS. While SAgs mediate all of the hallmark features of TSS, such as fever, rash, hypotension, and multi-organ dysfunction, they are also capable of enhancing the toxic effects of endogenous endotoxin. This interaction appears to be critical in mediating the severity of TSS and related mortality. In addition, interaction between SAgs and the host immune system has been recognized to result in a unique form of adaptive immune suppression, contributing to poor outcomes of S. aureus infections. Utilizing rabbit models of S. aureus infective endocarditis, pneumonia and sepsis, and molecular genetics techniques, we aim to elucidate the mechanisms of SAg and endotoxin synergism in the pathogenesis of TSS, and examine the cellular and molecular mechanisms underlying SAg-mediated immune dysfunction.

[Sturge-Weber syndrome].

PubMed

Maruani, Annabel

2010-04-01

Facial port-wine stains are capillary malformations, which can reveal, very rarely, Sturge-Weber syndrome (SWS). SWS is a severe neurocutaneous syndrome, which involves a facial port-wine stain reaching the first branch of trigeminal nerve (V1), ophthalmologic abnormalities (especially congenital glaucoma) and neurologic signs (seizure, mental retardation, hemiparesis). Neuroimaging (CT-scan/angio-magnetic resonance imaging [MRI]) provides the diagnosis of SWS, when it shows ipsilateral leptomeningeal angioma; the best age to perform the exam is not established. Extension to superior eyelid, to other territories of trigeminal nerve (V2, V3) or to the contralateral hemiface is statistically associated to SWS. When a new-born has a facial port-wine stain reaching V1, ophthalmologic examination must be performed in the first months of life, as well as neuroimaging (at the age of 6-12 months, earlier in case of neurologic signs); a treatment of the port-wine stain by pulsed dye laser must also be considered. (c) 2010. Published by Elsevier Masson SAS.

De novo centriole formation in human cells is error-prone and does not require SAS-6 self-assembly.

PubMed

Wang, Won-Jing; Acehan, Devrim; Kao, Chien-Han; Jane, Wann-Neng; Uryu, Kunihiro; Tsou, Meng-Fu Bryan

2015-11-26

Vertebrate centrioles normally propagate through duplication, but in the absence of preexisting centrioles, de novo synthesis can occur. Consistently, centriole formation is thought to strictly rely on self-assembly, involving self-oligomerization of the centriolar protein SAS-6. Here, through reconstitution of de novo synthesis in human cells, we surprisingly found that normal looking centrioles capable of duplication and ciliation can arise in the absence of SAS-6 self-oligomerization. Moreover, whereas canonically duplicated centrioles always form correctly, de novo centrioles are prone to structural errors, even in the presence of SAS-6 self-oligomerization. These results indicate that centriole biogenesis does not strictly depend on SAS-6 self-assembly, and may require preexisting centrioles to ensure structural accuracy, fundamentally deviating from the current paradigm.

[Asperger syndrome in contemporary fictions].

PubMed

Pourre, F; Aubert, E; Andanson, J; Raynaud, J-P

2012-12-01

During recent years, fictions featuring a character with Asperger syndrome have been increasingly produced in literature, cinema and TV. Thus, the public has gradually discovered the existence of this specific category of autism spectrum, which is far removed from old popular representations of autistic disorders, often associated with mental retardation. To describe the reactions generated by these characters in order to identify their major functions and also to try to explain their recent increase in fictions. First, we explored international publications concerning this topic. A group of experienced clinicians systematically examined works of fiction produced between 2000 and 2010 that included a character with Asperger syndrome. More than 30 productions have been identified and analyzed using a method adapted from focus group. Over 30 productions have been recorded and analyzed. The reactions generated by these characters are described. They range from fascination to empathy; if these heroes sometimes induce laughter (because of comedy situations), they also lead us to question our vision of the world and ask ourselves about notions such as difference, normality and tolerance. We illustrate this phenomenon with examples from literature, cinema or television. Four hypotheses are proposed trying to explain the recent multiplication of these fictional characters with Asperger syndrome. The first puts forward authors' informative and educational motivations, these authors being aware of this issue. The second is supported by the "hero" concept, which has evolved gradually into the figures of the scientific world and the so-called "Geek" community. The third hypothesis, a metaphorical one, considers these heroes as symbols of a future society: a hyper systematized society, devoid of empathy, as if to warn of a risk of evolution of humanity toward a generalized mental blindness. The fourth and last hypothesis explores the personal resonance, supported by

The homo-oligomerisation of both Sas-6 and Ana2 is required for efficient centriole assembly in flies

PubMed Central

Cottee, Matthew A; Muschalik, Nadine; Johnson, Steven; Leveson, Joanna; Raff, Jordan W; Lea, Susan M

2015-01-01

Sas-6 and Ana2/STIL proteins are required for centriole duplication and the homo-oligomerisation properties of Sas-6 help establish the ninefold symmetry of the central cartwheel that initiates centriole assembly. Ana2/STIL proteins are poorly conserved, but they all contain a predicted Central Coiled-Coil Domain (CCCD). Here we show that the Drosophila Ana2 CCCD forms a tetramer, and we solve its structure to 0.8 Å, revealing that it adopts an unusual parallel-coil topology. We also solve the structure of the Drosophila Sas-6 N-terminal domain to 2.9 Å revealing that it forms higher-order oligomers through canonical interactions. Point mutations that perturb Sas-6 or Ana2 homo-oligomerisation in vitro strongly perturb centriole assembly in vivo. Thus, efficient centriole duplication in flies requires the homo-oligomerisation of both Sas-6 and Ana2, and the Ana2 CCCD tetramer structure provides important information on how these proteins might cooperate to form a cartwheel structure. DOI: http://dx.doi.org/10.7554/eLife.07236.001 PMID:26002084

[Postural tachycardia syndrome (PoTS): An up-to-date].

PubMed

Astudillo, L; Laure, A; Fabry, V; Pugnet, G; Maury, P; Labrunée, M; Sailler, L; Pavy-Le Traon, A

2018-06-13

Postural tachycardia syndrome (PoTS) is a multifactorial syndrome defined by an increase in heart rate ≥30bpm, within 10minutes of standing (or during a head up tilt test to at least 60°), in absence of orthostatic hypotension. It is associated with symptoms of cerebral hypoperfusion that are worse when upright and improve in supine position. Patients have an intense fatigue with a high incidence on quality of life. This syndrome can be explained by many pathophysiological mechanisms. It can be associated with Ehlers-Danlos disease and some autoimmune disorders. The treatment is based on nonpharmacological measures and treatment with propranolol, fludrocortisone or midodrine. Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

[Demons-Meigs syndrome: information on a new case and review of the literature].

PubMed

Boufettal, H; Zaghba, N; Morad, S; Bakhatar, A; Yassine, N; Bahlaoui, A; Noun, M; Hermas, S; Samouh, N

2011-04-01

Demons-Meigs syndrome is a benign tumor of the ovary. It is very rare and its physiopathology remains obscure. We report a case of Demons-Meigs syndrome in a woman aged 51 years. It was discovered following a right pleural effusion syndrome with ascites and an abdominopelvic mass. Rate of serum CA 125 was 412IU/mL. Surgical exploration revealed ascites of one litre with no suspicious peritoneal lesion and an ovarian fibrothecoma of 70 mm. There were no post-operative complications and three months later, the level of CA 125 was negative with a total drainage of effusions. Copyright © 2011. Published by Elsevier Masson SAS.

PREFACE Proceedings of the XIV International Conference on Small-Angle Scattering, SAS-2009

NASA Astrophysics Data System (ADS)

King, Stephen; Terrill, Nicholas

2010-10-01

The XIV International Conference on Small-Angle Scattering, SAS-2009, was held in Oxford UK, 13-18 September 2009, and was jointly organised under the auspices of the International Union of Crystallography Commission on SAS by a team from the Diamond Light Source and the ISIS Pulsed Neutron Source - their first such joint venture - with help from the UK Science and Technology Facilities Council. It was the first time that this long running and successful series of conferences on the application, science and technology of small-angle scattering techniques had been staged in the UK. The UK has a proud heritage in small-angle scattering: as home to one of the world's first SANS instruments (at AERE Harwell), as the site of the world's first 2nd generation X-ray Synchrotron (the SRS at Daresbury with its suite of SAXS beamlines), and latterly as the location of the world's most successful pulsed source SANS instrument. Indeed, 2009 also marked the 25th Anniversary of neutron operations at ISIS and the opening of a Second Target Station. Whilst the SRS ceased operations in 2008, its mantle has been inherited by the Diamond synchrotron. Many delegates took the opportunity to visit both Diamond and ISIS during a conference excursion. Despite the prevailing global economic downturn, we were delighted that 434 delegates from 32 different countries were able to attend SAS-2009; two-thirds were drawn from the UK, Germany, Japan, the USA and France, but there were also sizeable contingents from Australia, Korea, Taiwan and South America. In many ways this geographical spread reflects the present and emerging distribution, respectively, of 3rd generation X-ray synchrotrons and high-flux neutron sources, although the scope of the conference was not solely limited to these probes. Financial support from the IUCr enabled us to grant bursaries to attend SAS-2009 to 12 delegates from emerging countries (Algeria, Argentina, Brazil, India, Nepal, Romania, Russia and the Ukraine). The

Guillain-Barré syndrome: What have we learnt during one century? A personal historical perspective.

PubMed

Uncini, A

2016-10-01

We are approaching the centenary of the first description of Guillain-Barré syndrome. The past 30 years had witnessed an amazing progress in the understanding of the immunological and pathological mechanisms of this disorder. We now recognize that Guillain-Barré syndrome is remarkably heterogeneous and under this umbrella term are several variants and subtypes with distinct clinical, electrophysiological and immunopathological features. This review is a historical journey, through a personal perspective, following the milestones that led to the current substantial knowledge of Guillain-Barré syndrome. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

STS-41 crewmembers conduct DSO 0474 Retinal Photography on OV-103's middeck

NASA Image and Video Library

1990-10-10

STS041-02-015 (6-10 Oct. 1990) --- A 35mm scene showing astronauts Robert D. Cabana (right), STS-41 pilot, and Bruce E. Melnick, mission specialist, participating in a detailed supplemental objective for STS-41 involving retinal photography. The hypothesis of this experiment is that retinal photographs taken on orbit will show evidence of increased intracranial pressure (ICP) and the evidence of increased ICP and the development of Space Adaptation Syndrome (SAS) will be correlated. SAS has been a subject of on-orbit analysis since the early days of space shuttle.

Repositioning the substrate activity screening (SAS) approach as a fragment-based method for identification of weak binders.

PubMed

Gladysz, Rafaela; Cleenewerck, Matthias; Joossens, Jurgen; Lambeir, Anne-Marie; Augustyns, Koen; Van der Veken, Pieter

2014-10-13

Fragment-based drug discovery (FBDD) has evolved into an established approach for "hit" identification. Typically, most applications of FBDD depend on specialised cost- and time-intensive biophysical techniques. The substrate activity screening (SAS) approach has been proposed as a relatively cheap and straightforward alternative for identification of fragments for enzyme inhibitors. We have investigated SAS for the discovery of inhibitors of oncology target urokinase (uPA). Although our results support the key hypotheses of SAS, we also encountered a number of unreported limitations. In response, we propose an efficient modified methodology: "MSAS" (modified substrate activity screening). MSAS circumvents the limitations of SAS and broadens its scope by providing additional fragments and more coherent SAR data. As well as presenting and validating MSAS, this study expands existing SAR knowledge for the S1 pocket of uPA and reports new reversible and irreversible uPA inhibitor scaffolds. © 2014 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

Increased low-grade inflammation and plasminogen-activator inhibitor-1 level in non-dippers with sleep apnea syndrome

PubMed Central

Ishikawa, Joji; Hoshide, Satoshi; Eguchi, Kazuo; Ishikawa, Shizukiyo; Pickering, Thomas G; Shimada, Kazuyuki; Kario, Kazuomi

2010-01-01

Objective Patients with sleep apnea syndrome (SAS) have an increased risk of cardiovascular events and frequently show a non-dipper pattern (blunted nocturnal decline <10%) of systolic blood pressure (BP). We investigated neurohumoral activation and risk factors in relation to nocturnal BP dipping pattern and SAS. Methods We conducted sleep polysomnography and ambulatory BP monitoring, and measured high-sensitivity C-reactive protein (hsCRP), tissue-type plasminogen activator inhibitor-1 (PAI-1), and neurohumoral factors in 121 outpatients with suspected SAS, who were classified in 4 groups depending on the presence or absence of dipping/non-dipping and SAS. Results Non-dippers with SAS had higher hsCRP (overall P<0.001), PAI-1 (overall P=0.004), and aldosterone levels (overall P=0.010) than any of the other 3 groups. After adjustment for significant covariates such as age, sex, body mass index, waist circumference, smoking, alcohol drinking, aspirin use, presence of DM, and insulin, non-dippers with SAS still had a higher hsCRP level than non-dippers without SAS (geometric mean: 1.47 vs. 0.37 mg/L, P=0.001). In multiple linear regression analysis controlling for confounding factors that related with SAS, hsCRP was significantly correlated with 3% oxygen desaturation index (P=0.047). PAI-1 was also highest in the non-dippers with SAS, but this was not independent of obesity. PAI-1 was correlated with insulin (r=0.32, P=0.002) and hsCRP levels (r=0.26, P=0.005). Conclusion Non-dipper status was associated with an increased hsCRP in patients who also had SAS, but not in those who did not, and hsCRP was closely affected by the desaturation level. PAI-1 is also increased in non-dippers with SAS, and is related to insulin and hsCRP. PMID:18475156

[Lemierre's syndrome: Diagnosis, exploration, treatment].

PubMed

Mesrar, H; Mesrar, J; Maillier, B; Kraoua, S; Chapoutot, L; Delclaux, B

2018-05-01

Lemierre's syndrome is a rare and severe sepsis that can rapidly lead to a life-threatening condition in the absence of early management. This syndrome described at the beginning of the 20th century combines oropharyngeal infection complicated with septic thrombosis of the internal jugular vein and septic emboli predominantly pulmonary. Fusobacterium necrophorum, anaerobic germ, Gram negative bacillus is the main germ in this "necrobacillosis". The diagnosis is should be confirmed precociously with cervicothoracic CT-scan, reference exam, and bacteriological examinations (especially in atypical forms). Its management consists of an emergency antibiotic treatment, combining a third-generation cephalosporin or a betalactam with metronidazole, anticoagulant therapy to be reserved for high-risk situations related to thrombosis. Surgical treatment may be required. Copyright © 2017 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Modification of the SAS4A Safety Analysis Code for Integration with the ADAPT Discrete Dynamic Event Tree Framework.

DOE Office of Scientific and Technical Information (OSTI.GOV)

Jankovsky, Zachary Kyle; Denman, Matthew R.

It is difficult to assess the consequences of a transient in a sodium-cooled fast reactor (SFR) using traditional probabilistic risk assessment (PRA) methods, as numerous safety-related sys- tems have passive characteristics. Often there is significant dependence on the value of con- tinuous stochastic parameters rather than binary success/failure determinations. One form of dynamic PRA uses a system simulator to represent the progression of a transient, tracking events through time in a discrete dynamic event tree (DDET). In order to function in a DDET environment, a simulator must have characteristics that make it amenable to changing physical parameters midway through themore » analysis. The SAS4A SFR system analysis code did not have these characteristics as received. This report describes the code modifications made to allow dynamic operation as well as the linking to a Sandia DDET driver code. A test case is briefly described to demonstrate the utility of the changes.« less

[Guillain-Barré syndrome with preserved reflexes].

PubMed

Zouiri, G; Abilkassem, R; Zerhouni, A; Dini, N; Agadr, A

2016-05-01

Guillain-Barré is a rare, autoimmune disease of the peripheral nervous system. It can affect all ages beginning in the intrauterine or neonatal period. Clinical forms are diverse and include acute motor axonal neuropathy (AMAN). We report on a pediatric case of AMAN. A 2.5-year-old child presented with acute flaccid paralysis and preserved reflexes. Etiologic investigations argued in favor of Guillain-Barré syndrome in its AMAN form. Treatment based on IV immunoglobulins resulted in a total decline of paralysis and motor recovery. The AMAN form of Guillain-Barré syndrome should be considered as a potential diagnosis in all cases of acute flaccid paralysis with preserved reflexes. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Aerosol Optical Depth Value-Added Product for the SAS-He Instrument

DOE Office of Scientific and Technical Information (OSTI.GOV)

Ermold, B; Flynn, CJ; Barnard, J

2013-11-27

The Shortwave Array Spectroradiometer – Hemispheric (SAS-He) is a ground-based, shadowband instrument that measures the direct and diffuse solar irradiance. In this regard, the instrument is similar to the Multi-Filter Rotating Shadowband Radiometer (MFRSR) – an instrument that has been in the ARM suite of instruments for more than 15 years. However, the two instruments differ significantly in wavelength resolution and range. In particular, the MFRSR only observes the spectrum in six discrete wavelength channels of about 10 nm width from 415 to 940 nm. The SAS-He, in contrast, incorporates two fiber-coupled grating spectrometers: a Si CCD spectrometer with overmore » 2000 pixels covering the range from 325-1040 nm with ~ 2.5 nm resolution ,and an InGaAs array spectrometer with 256 pixels covering the wavelength range from 960-1700 nm with ~ 6 nm resolution.« less

De novo centriole formation in human cells is error-prone and does not require SAS-6 self-assembly

PubMed Central

Wang, Won-Jing; Acehan, Devrim; Kao, Chien-Han; Jane, Wann-Neng; Uryu, Kunihiro; Tsou, Meng-Fu Bryan

2015-01-01

Vertebrate centrioles normally propagate through duplication, but in the absence of preexisting centrioles, de novo synthesis can occur. Consistently, centriole formation is thought to strictly rely on self-assembly, involving self-oligomerization of the centriolar protein SAS-6. Here, through reconstitution of de novo synthesis in human cells, we surprisingly found that normal looking centrioles capable of duplication and ciliation can arise in the absence of SAS-6 self-oligomerization. Moreover, whereas canonically duplicated centrioles always form correctly, de novo centrioles are prone to structural errors, even in the presence of SAS-6 self-oligomerization. These results indicate that centriole biogenesis does not strictly depend on SAS-6 self-assembly, and may require preexisting centrioles to ensure structural accuracy, fundamentally deviating from the current paradigm. DOI: http://dx.doi.org/10.7554/eLife.10586.001 PMID:26609813

[Multiple sclerosis associated with antiphospholipid syndrome: diagnostic and therapeutic difficulties].

PubMed

Ahbeddou, N; Ait Ben Haddou, E; Hammi, S; Slimani, C; Regragui, W; Benomar, A; Yahyaoui, M

2012-01-01

Strokes are the main neurological manifestation of antiphospholipid syndrome. Other clinical presentations are possible and may mimic classic symptoms of multiple sclerosis (MS). A 46-year-old woman, with a history of two miscarriages, presented four subacute neurological episodes (optic neuritis, right facial paralysis, paraparesis of the thigh, and right brachial monoparesis). Using McDonald criteria, the diagnosis of multiple sclerosis was retained. Because of the occurrence of thrombocytopenia during a final relapse, we reconsidered the diagnosis of MS. Search for antiphospholipid antibodies was positive. All clinical manifestations and complementary tests were compatible with the diagnosis of antiphospholipid syndrome associated with multiple sclerosis. Given the great similarity of clinical, radiological and biological findings in the two diseases, non-thrombotic neurological manifestations of antiphospholipid syndrome can be difficult to distinguish from MS associated with antiphospholipid syndrome. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Adaptive behavior of institutionalized individuals with Down syndrome.

PubMed

Silverstein, A B; Ageno, D; Alleman, A C; Derecho, K T; Gray, S B; White, J F

1985-03-01

Institutional residents with Down syndrome (N = 413) were matched with an equal number of residents in other diagnostic categories with respect to sex, age, length of hospitalization, and IQ. The two groups were compared on 62 items of the Client Development Evaluation Report, and significant differences were found on 19 of these items. The subjects with Down syndrome tended to show greater social competence (except in the clarity of their speech) and less maladaptive behavior. To account for these differences, we offer the speculative hypothesis that the favorable expectations of service providers for their clients with Down syndrome may come to serve as self-fulfilling prophecies.

Comparison of Stanford B aortic dissection patients who received TEVAR combined with or without sleep apnea syndrome.

PubMed

Li, Xin; Cai, Wenwu; Zhang, Ping; Fang, Kun; Zhu, Jieting; Shu, Chang

2018-05-18

Patients with Stanford B aortic dissection usually complicated with sleep apnea syndrome. This condition always threatens the patients' respiration situation. In this study, we collected and analysis data of patients' peri-operative managements of Thoracic Endovascular Aortic Repair (TEVAR) for Stanford B Aortic Dissection (AD) complicated with Sleep Apnea Syndrome (SAS). Comparison has been made between these SAS patients and those who without SAS. Between June 2013 and June 2014, the clinical data and outcomes of the Stanford B AD patients in the department of vascular surgery in the Second Xiangya Hospital were retrospectively reviewed and studied. According to the result of intentionally polysomonography by using portable Polysomnography monitor (Nox T3, Nox Medical Co. Iceland) in TEVAR candidates, patients has been divided to SAS positive and SAS negative group. Comparison has been made in various variables between these two groups. 134 patients, with Stanford B AD and treated by TEVAR in our center, were enrolled in this study. Patients' mean age was 52.46±10.84 years old. Gender ratio is 114:20, including male 85.07% (114/134) and female 14.93% (20/134). 71.64% (96/134) patients had been performed TEVAR under general anesthesia (GA) and 38 patients were under local anesthesia (LA).The mean body mass index (BMI) was 23.5±4.2, and the longest follow-up time was 46 months. The patients were divided into SAS-positive group (n=23) and SAS-negative group (n=111). Compared with the SAS-negative group, patients in the SAS-positive group were younger (54.36±0.97 vs 43.3±1.84 p<0.0001) but in higher BMI (25.48±0.71 vs 22.24±0.23, p<0.0001 ), with longer hospitalization time (25.52±0.59 vs 15.68±0.27; p<0.0001) but without significant differences of ICU stay time (54.87±12.57 vs 40.27±8.10; p=0.3369). Furthermore, the complication rate of both pulmonary infection (65.22% vs 13.51%; p<0.0001), respiratory failure (26.09% vs 1.80%; p=0.003), heart failure ( 26

Age-Related Changes of Adaptive and Neuropsychological Features in Persons with Down Syndrome

PubMed Central

Ghezzo, Alessandro; Salvioli, Stefano; Solimando, Maria Caterina; Palmieri, Alice; Chiostergi, Chiara; Scurti, Maria; Lomartire, Laura; Bedetti, Federica; Cocchi, Guido; Follo, Daniela; Pipitone, Emanuela; Rovatti, Paolo; Zamberletti, Jessica; Gomiero, Tiziano; Castellani, Gastone; Franceschi, Claudio

2014-01-01

Down Syndrome (DS) is characterised by premature aging and an accelerated decline of cognitive functions in the vast majority of cases. As the life expectancy of DS persons is rapidly increasing, this decline is becoming a dramatic health problem. The aim of this study was to thoroughly evaluate a group of 67 non-demented persons with DS of different ages (11 to 66 years), from a neuropsychological, neuropsychiatric and psychomotor point of view in order to evaluate in a cross-sectional study the age-related adaptive and neuropsychological features, and to possibly identify early signs predictive of cognitive decline. The main finding of this study is that both neuropsychological functions and adaptive skills are lower in adult DS persons over 40 years old, compared to younger ones. In particular, language and short memory skills, frontal lobe functions, visuo-spatial abilities and adaptive behaviour appear to be the more affected domains. A growing deficit in verbal comprehension, along with social isolation, loss of interest and greater fatigue in daily tasks, are the main features found in older, non demented DS persons evaluated in our study. It is proposed that these signs can be alarm bells for incipient dementia, and that neuro-cognitive rehabilitation and psycho-pharmacological interventions must start as soon as the fourth decade (or even earlier) in DS persons, i.e. at an age where interventions can have the greatest efficacy. PMID:25419980

CVD silicon carbide monofilament reinforced SrO-Al2O3-2SiO2 (SAS) glass-ceramic composites

NASA Technical Reports Server (NTRS)

Bansal, Narottam P.

1995-01-01

Unidirectional CVD SiC fiber-reinforced SrO.Al2O3.2SiO2 (SAS) glass-ceramic matrix composites have been fabricated by hot pressing at various combinations of temperature, pressure and time. Both carbon-rich surface coated SCS-6 and uncoated SCS-0 fibers were used as reinforcements. Almost fully dense composites have been obtained. Monoclinic celsian, SrAl2Si2O8, was the only crystalline phase observed in the matrix from x-ray diffraction. During three point flexure testing of composites, a test span to thickness ratio of approximately 25 or greater was necessary to avoid sample delamination. Strong and tough SCS-6/SAS composites having a first matrix crack stress of approximately 300 MPa and an ultimate bend strength of approximately 825 MPa were fabricated. No chemical reaction between the SCS-6 fibers and the SAS matrix was observed after high temperature processing. The uncoated SCS-0 fiber-reinforced SAS composites showed only limited improvement in strength over SAS monolithic. The SCS-0/SAS composite having a fiber volume fraction of 0.24 and hot pressed at 1400 deg C exhibited a first matrix cracking stress of approximately 231 +/- 20 MPa and ultimate strength of 265 +/- 17 MPa. From fiber push-out tests, the fiber/matrix interfacial debonding strength (tau(sub debond)) and frictional sliding stress (tau(sub friction)) in the SCS-6/SAS system were evaluated to be approximately 6.7 +/- 2.3 MPa and 4.3 +/- 0.6 MPa, respectively, indicating a weak interface. However, for the SCS-0/SAS composite, much higher values of approximately 17.5 +/- 2.7 MPa for tau(sub debond) and 11.3 +/- 1.6 MPa for tau(sub friction) respectively, were observed; some of the fibers were so strongly bonded to the matrix that they could not be pushed out. Examination of fracture surfaces revealed limited short pull-out length of SCS-0 fibers. The applicability of various micromechanical models for predicting the values of first matrix cracking stress and ultimate strength of these

Group training in interpersonal problem-solving skills for workplace adaptation of adolescents and adults with Asperger syndrome: a preliminary study.

PubMed

Bonete, Saray; Calero, María Dolores; Fernández-Parra, Antonio

2015-05-01

Adults with Asperger syndrome show persistent difficulties in social situations which psychosocial treatments may address. Despite the multiple studies focusing on social skills interventions, only some have focused specifically on problem-solving skills and have not targeted workplace adaptation training in the adult population. This study describes preliminary data from a group format manual-based intervention, the Interpersonal Problem-Solving for Workplace Adaptation Programme, aimed at improving the cognitive and metacognitive process of social problem-solving skills focusing on typical social situations in the workplace based on mediation as the main strategy. A total of 50 adults with Asperger syndrome received the programme and were compared with a control group of typical development. The feasibility and effectiveness of the treatment were explored. Participants were assessed at pre-treatment and post-treatment on a task of social problem-solving skills and two secondary measures of socialisation and work profile using self- and caregiver-report. Using a variety of methods, the results showed that scores were significantly higher at post-treatment in the social problem-solving task and socialisation skills based on reports by parents. Differences in comparison to the control group had decreased after treatment. The treatment was acceptable to families and subject adherence was high. The Interpersonal Problem-Solving for Workplace Adaptation Programme appears to be a feasible training programme. © The Author(s) 2014.

Physiologic adaptation to space - Space adaptation syndrome

NASA Technical Reports Server (NTRS)

Vanderploeg, J. M.

1985-01-01

The adaptive changes of the neurovestibular system to microgravity, which result in space motion sickness (SMS), are studied. A list of symptoms, which range from vomiting to drowsiness, is provided. The two patterns of symptom development, rapid and gradual, and the duration of the symptoms are described. The concept of sensory conflict and rearrangements to explain SMS is being investigated.

Acute effects of the glucagon-like peptide 2 analogue, teduglutide, on intestinal adaptation in short bowel syndrome.

PubMed

Thymann, Thomas; Stoll, Barbara; Mecklenburg, Lars; Burrin, Douglas G; Vegge, Andreas; Qvist, Niels; Eriksen, Thomas; Jeppesen, Palle B; Sangild, Per T

2014-06-01

Neonatal short bowel syndrome following massive gut resection is associated with malabsorption of nutrients. The intestinotrophic factor glucagon-like peptide 2 (GLP-2) improves gut function in adult patients with short bowel syndrome, but its effect in pediatric patients remains unknown. Our objective was to test the efficacy of the long-acting synthetic human GLP-2 analogue, teduglutide (ALX-0600), in a neonatal piglet jejunostomy model. Two-day-old pigs were subjected to resection of 50% of the small intestine (distal part), and the remnant intestine was exteriorized on the abdominal wall as a jejunostomy. All pigs were given total parenteral nutrition for 7 days and a single daily injection of the following doses of teduglutide: 0.01 (n = 6), 0.02 (n = 6), 0.1 (n = 5), or 0.2 mg · kg · day (n = 6), and compared with placebo (n = 9). Body weight increment was similar for all 4 teduglutide groups but higher than placebo (P < 0.05). There was a dose-dependent increase in weight per length of the remnant intestine (P < 0.01) and fractional protein synthesis rate in the intestine was increased in the 0.2 mg · kg · day group versus placebo (P < 0.001); however, functional and structural endpoints including activity of digestive enzymes, absorption of enteral nutrients, and immunohistochemistry (Ki67, villin, FABP2, ChgA, and GLP-2R) were not affected by the treatment. Teduglutide induces trophicity on the remnant intestine but has limited acute effects on functional endpoints. Significant effects of teduglutide on gut function may require a longer adaptation period and/or a more frequent administration of the peptide. In perspective, GLP-2 or its analogues may be relevant to improve intestinal adaptation in pediatric patients with short bowel syndrome.

Melorheostosis of the humerus: a rare differential diagnosis of carpal tunnel syndrome.

PubMed

De Vos, J; Mulliez, A; De Loore, G

2010-04-01

Melorheostosis is an uncommon and rare linear hyperostosis, which can be complicated by soft tissue changes. We present a case of this disorder in the humerus, clinically referred because of carpal tunnel syndrome. Although treatment is usually conservative, in this case, a neurolysis and resection of the sclerotic bone were done with good clinical result. Copyright 2010 Elsevier Masson SAS. All rights reserved.

Severity of psychosis syndrome and change of metabolic abnormality in chronic schizophrenia patients: severe negative syndrome may be related to a distinct lipid pathophysiology.

PubMed

Chen, S-F; Hu, T-M; Lan, T-H; Chiu, H-J; Sheen, L-Y; Loh, E-W

2014-03-01

Metabolic abnormality is common among schizophrenia patients. Some metabolic traits were found associated with subgroups of schizophrenia patients. We examined a possible relationship between metabolic abnormality and psychosis profile in schizophrenia patients. Three hundred and seventy-two chronic schizophrenia patients treated with antipsychotics for more than 2 years were assessed with the Positive and Negative Syndrome Scale. A set of metabolic traits was measured at scheduled checkpoints between October 2004 and September 2006. Multiple regressions adjusted for sex showed negative correlations between body mass index (BMI) and total score and all subscales; triglycerides (TG) was negatively correlated with total score and negative syndrome, while HDLC was positively correlated with negative syndrome. When sex interaction was concerned, total score was negatively correlated with BMI but not with others; negative syndrome was negatively correlated with BMI and positively with HDLC. No metabolic traits were correlated with positive syndrome or general psychopathology. Loss of body weight is a serious health problem in schizophrenia patients with severe psychosis syndrome, especially the negative syndrome. Schizophrenia patients with severe negative syndrome may have a distinct lipid pathophysiology in comparison with those who were less severe in the domain. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Stress, Locus of Control, and Family Cohesion and Adaptability in Parents of Children with Down, Williams, Fragile X, and Prader-Willi Syndromes

ERIC Educational Resources Information Center

Lanfranchi, Silvia; Vianello, Renzo

2012-01-01

The present study analyzes differences in parental stress in families of children with Down, Williams, Fragile X, and Prader-Willi syndromes, exploring factors that influence parental stress, such as child's characteristics, parental locus of control, and family cohesion and adaptability. Differences between mothers and fathers are also…

How applicable is the general adaptation syndrome to the unicellular Tetrahymena?

PubMed

Csaba, György; Pállinger, Eva

2009-01-01

Hormone receptors, hormones and signal transduction pathways characteristic of higher vertebrates can be observed also in the unicellular Tetrahymena. Previous work showed that stress conditions (starvation, high temperature, high salt concentration, formaldehyde or alcohol treatment) elevated the intracellular level of four hormones (ACTH, endorphin, serotonin and T(3)). Here, the effect of other stressors (CuSO4 poisoning, tryptophan hydroxylase inhibitor parachlorophenylalanine (PCPA) treatment) on the same and other hormones (epinephrine, insulin, histamine) was studied, using immunocytochemistry and flow cytometric analysis. It was found, that each effect increased the intracellular hormone contents, but some hormones (histamine, T(3)) were less reactive. Insulin--which is a life-saving factor for Tetrahymena--itself provoked elevation of hormone amounts in association with a stressor, further increased the level of hormones. It was concluded that the ancestor of Selye's General Adaptation Syndrome (GAS) can be found already at unicellular level, and this possibly has a life saving function. Copyright 2008 John Wiley & Sons, Ltd.

Space sickness on earth

NASA Astrophysics Data System (ADS)

Nooij, S. A. E.; Bos, J. E.; Groen, E. L.; Bles, W.; Ockels, W. J.

2007-09-01

During the first days in space, i.e., after a transition from 1G to 0G, more than 50% of the astro- (and cosmonauts) suffer from the Space Adaptation Syndrome (SAS).The symptoms of SAS, like nausea and dizziness, are especially provoked by head movements. Astronauts have mentioned close similarities between the symptoms of SAS and the symptoms they experienced after a 1 hour centrifuge run on Earth, i.e., after a transition from 3G to 1G (denoted by Sickness Induced by Centrifugation, SIC). During several space missions, we related susceptibility to SAS and to SIC in 11 astronauts and found 4 of them being susceptible to both SIC and SAS, and 7 being not susceptible to SIC nor to SAS. This correspondence in susceptibility suggests that SIC and SAS share the same underlying mechanism. To further study this mechanism, several vestibular parameters have been investigated (e.g. postural stability, vestibularly driven eye movements, subjective vertical). We found some striking changes in individual cases that are possibly due to the centrifuge run. However, the variability between subjects generally is very large, making physiological links to SIC and SAS still hard to find.

[Pain, from symptom to syndrome].

PubMed

Piano, Virginie

2017-05-01

Acute pain is a symptom enabling us to implement a response when faced with an attack. Chronic pain is complex and multifactorial. The care of the patient by a multidisciplinary team comprises the diagnosis of the pain and the putting in place of a treatment for each of its components. This includes physical reconditioning, adaptation strategies and work on the psychological elements relating to the representation of the pain. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

[Psychological commitment: adaptive functions, paradoxes, modalities and components].

PubMed

Brault-Labbé, Anne

2017-12-01

The process of psychological commitment, its adaptive functions and some of the difficulties that go along with it are specific. A description of the multimodal model of commitment provides an illustration of how motivational, affective, cognitive and behavioural mechanisms can be combined. These lead to different ways of entering into the commitment, with differing consequences on how the individual functions. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

[Staphylococcal toxic shock syndrome after breast surgery].

PubMed

Pelissier, A; Dumesnil, J; Levy, R; Charron, C; Rouzier, R

2014-09-01

The surgical site infection occurs within 30 days after surgery. It is the most common complication of surgery, with a rate of 1 to 5% without antibiotic prophylaxis and less than 1% with antibiotic prophylaxis. The toxic shock syndrome (TSS) is a dramatic complication. We report the case 39-year-old woman who presented a life-threatening TSS acquired after breast surgery. We describe the signs and symptoms of this condition as well as treatment principles. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Modest changes in cerebral glucose metabolism in patients with sleep apnea syndrome after continuous positive airway pressure treatment.

PubMed

Ju, Gawon; Yoon, In-Young; Lee, Sang Don; Kim, Yu Kyeong; Yoon, Eunjin; Kim, Jeong-Whun

2012-01-01

Decreased cerebral glucose metabolism has been reported in patients with sleep apnea syndrome (SAS), but it has yet to be decided whether cerebral glucose metabolism in SAS can be altered by continuous positive airway pressure (CPAP) treatment. The aim of this study was to evaluate cerebral glucose metabolism changes in patients with SAS after CPAP treatment. Thirteen middle-aged male patients with severe SAS [mean age 49.3 ± 7.2 years, mean apnea-hypopnea index (AHI) 60.4 ± 21.2] and 13 male controls (mean age 46.0 ± 9.4 years, mean AHI 4.1 ± 3.7) participated in the study. All 26 study subjects underwent fluorodeoxyglucose-positron emission tomography (FDG-PET), but SAS patients underwent FDG-PET twice, namely before and 3 months after acceptable CPAP usage. Significant hypometabolism was observed in the bilateral prefrontal areas, left cuneus and left cingulate cortex of SAS patients before CPAP, and after CPAP, significant increases in cortical glucose metabolism were observed in the bilateral precentral gyri and left anterior cingulate cortex. However, these improvements in hypometabolism in both areas were insufficient to reach control levels, and hypometabolism in other regions persisted after CPAP treatment. Reduced cerebral glucose metabolism in the precentral gyrus and the cingulate cortex in patients with SAS was modestly improved by acceptable CPAP treatment. The findings of this study suggest that acceptable CPAP usage cannot completely reverse reduced cerebral glucose metabolism in SAS patients. Further studies are required to evaluate the long-term effects of CPAP treatment with total compliance. Copyright © 2012 S. Karger AG, Basel.

Solubilization of the poorly water soluble drug, telmisartan, using supercritical anti-solvent (SAS) process.

PubMed

Park, Junsung; Cho, Wonkyung; Cha, Kwang-Ho; Ahn, Junhyun; Han, Kang; Hwang, Sung-Joo

2013-01-30

Telmisartan is a biopharmaceutical classification system (BCS) class II drug that has extremely low water solubility but is freely soluble in highly alkalized solutions. Few organic solvents can dissolve telmisartan. This solubility problem is the main obstacle achieving the desired bioavailability. Because of its unique characteristics, the supercritical anti-solvent (SAS) process was used to BCS class II drug in a variety of ways including micronization, amorphization and solid dispersion. Solid dispersions were prepared using hydroxypropylmethylcellulose/polyvinylpyrrolidone (HPMC/PVP) at 1:0.5, 1:1, and 1:2 weight ratios of drug to polymer, and pure telmisartan was also treated using the SAS process. Processed samples were characterized for morphology, particle size, crystallinity, solubility, dissolution rate and polymorphic stability. After the SAS process, all samples were converted to the amorphous form and were confirmed to be hundreds nm in size. Solubility and dissolution rate were increased compared to the raw material. Solubility tended to increase with increases in the amount of polymer used. However, unlike the solubility results, the dissolution rate decreased with increases in polymer concentration due to gel layer formation of the polymer. Processed pure telmisartan showed the best drug release even though it had lower solubility compared to other solid dispersions; however, because there were no stabilizers in processed pure telmisartan, it recrystallized after 1 month under severe conditions, while the other solid dispersion samples remained amorphous form. We conclude that after controlling the formulation of solid dispersion, the SAS process could be a promising approach for improving the solubility and dissolution rate of telmisartan. Copyright © 2012 Elsevier B.V. All rights reserved.

Expanding the cardiac spectrum of Noonan syndrome with RIT1 variant: Left main coronary artery atresia causing sudden death.

PubMed

Ramond, Francis; Duband, Sébastien; Croisille, Pierre; Cavé, Hélène; Teyssier, Georges; Adouard, Véronique; Touraine, Renaud

2017-06-01

Noonan syndrome is a well-known genetic condition associating congenital heart defects, short stature, and distinctive facial features. Pulmonary valve stenosis and hypertrophic cardiomyopathy are the most frequent cardiac abnormalities, the latter being associated with a higher mortality. Here we report for the first time, a case of congenital left main coronary artery atresia in a Noonan syndrome associated with RIT1 variant, leading to unrescued sudden death. This case-report supports the already-suspected severity of the RIT1-related Noonan syndrome compared to average Noonan syndrome, and should encourage clinicians to be very cautious with these patients. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Hypothesis: SLC12A3 Polymorphism modifies thiazide hypersensitivity of antenatal Bartter syndrome to thiazide resistance.

PubMed

Mammen, Cherry; Rupps, Rosemarie; Trnka, Peter; Boerkoel, Cornelius F

2012-02-01

We report a 5-year-old boy with thiazide-resistant Bartter syndrome. This is highly unusual since thiazide hypersensitivity is a common diagnostic finding in Bartter syndrome patients. Subsequent molecular testing identified compound heterozygosity for two novel mutations in KCNJ1, (c.556A > G and c.683G > A) which is associated with Bartter syndrome, and a paternally inherited polymorphism in SLC12A3 (c.791G > C). Mutations in SLC12A3 cause the thiazide-resistant tubulopathy Gitelman syndrome. Based on published studies of this polymorphism in SLC12A3 and the features of the proband's father, we postulate that this polymorphism modifies the phenotype of Bartter syndrome in the proband to thiazide resistance. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Sleep apnea syndrome and patent foramen ovale: a dangerous association in ischemic stroke?

PubMed

Tur, Silvia; de la Peña, Mónica; Vives, Bárbara; Martínez, Ana Belén; Gorospe, Arantza; Legarda, Inés; Torres, Maria José; Jiménez, Carmen; Soriano, Joan B

2016-09-01

The coexistence of patent foramen ovale (PFO) and sleep apnea syndrome (SAS) might be related to the pathogenesis of cryptogenic stroke (CS). We aimed to determine the prevalence of SAS in patients with cryptogenic stroke and PFO. This is a prospective case-control study in which we included ischemic stroke patients consecutively admitted to our hospital's Acute Stroke Unit. Contrast transcranial Doppler (c-TCD) and sleep polygraphy within the first 72 h after stroke onset were performed to detect PFO and SAS. Demographic and clinical characteristics, time of stroke onset, score in the National Institute of Health Stroke Scale (NIHSS), and stroke subtype were registered. A total of 97 patients were studied. Overall, 76% were men, with a mean ± SD age of 61 ± 13 years, and an NIHSS of 5 ± 5. Subtype of stroke was cryptogenic (CS) in 28 (29%) and non-CS in 69 (71%) of patients. PFO was more frequent among patients with CS (64% vs 29%, p = 0.002) and without SAS (60% vs 32%, p = 0.013). SAS was diagnosed in 74% of the whole group, with a higher prevalence in patients with known stroke etiology (83% vs 53%, p = 0.003). Finally, the prevalence of SAS and PFO coexistence was similar in patients with or without cryptogenic stroke (25% vs 22%, p = 1), and when comparing the group of patients with cryptogenic wake-up stroke to the other stroke patients (43% vs 21%, p = 0.35). According to our results, there is no evidence of an association of PFO and SAS in the pathogenesis of cryptogenic stroke. Copyright © 2016 Elsevier B.V. All rights reserved.

Characterizing Flexible and Instrinsically Unstructured Biological Macromolecules by SAS using the Porod-Debye Law

PubMed Central

Rambo, Robert P.; Tainer, John A.

2011-01-01

Unstructured proteins, RNA or DNA components provide functionally important flexibility that is key to many macromolecular assemblies throughout cell biology. As objective, quantitative experimental measures of flexibility and disorder in solution are limited, small angle scattering (SAS), and in particular small angle X-ray scattering (SAXS), provides a critical technology to assess macromolecular flexibility as well as shape and assembly. Here, we consider the Porod-Debye law as a powerful tool for detecting biopolymer flexibility in SAS experiments. We show that the Porod-Debye region fundamentally describes the nature of the scattering intensity decay, which captures information needed for distinguishing between folded and flexible particles. Particularly for comparative SAS experiments, application of the law, as described here, can distinguish between discrete conformational changes and localized flexibility relevant to molecular recognition and interaction networks. This approach aids insightful analyses of fully and partly flexible macromolecules that is more robust and conclusive than traditional Kratky analyses. Furthermore, we demonstrate for prototypic SAXS data that the ability to calculate particle density by the Porod-Debye criteria, as shown here, provides an objective quality assurance parameter that may prove of general use for SAXS modeling and validation. PMID:21509745

[Cryopyrin-associated periodic syndromes].

PubMed

Quartier, P; Rodrigues, F; Georgin-Lavialle, S

2018-04-01

Cryopyrin-associated periodic syndromes (CAPS) are linked to one single gene mutations, however they are associated with 3 syndromes, which are, from the mildest to the most severe phenotype familial cold urticaria, Muckle-Wells syndrome and chronic, infantile, neurologic, cutaneous, articular (CINCA) syndrome also called neonatal-onset multisystem inflammatory disease (NOMID). Autosomic dominant inheritance is present in most cases but in CINCA/NOMID syndrome where neomutations are more common. Mutations in the gene encoding cryopyrin, NLRP3, are associated with deregulation of caspase-1 activity, excessive interleukin-1 production and an autoinflammatory syndrome, which in familial cold urticaria and Muckle-Wells syndrome may be triggered or worsened by exposure to coldness. More and more mutations are described and even somatic mutations that can explain some clinical signs beginning in adulthood. Patients disclose a pseudo-urticarial rash, arthralgia, headaches, sometimes fever, biological inflammation but also, in severe forms of the disease, neurologic inflammation with central deafness, ophthalmologic inflammation, chronic meningitis. Some CINCA/NOMID patients also develop growth cartilage pseudo-tumoral hypertrophy. Natural disease history is usually benign in familial cold urticarial but severe in the other forms, particularly regarding neuro-sensorial involvement. In addition, secondary AA amyloidosis may develop in all forms in the absence of control of chronic inflammation. Anti-interleukin-1 treatment with anakinra, rilonacept or canakinumab induces in most cases complete remission, however sequelae may be present, particularly if central deafness or cartilage bone hypertrophy have already developed. This treatment is also important to prevent secondary amyloidosis or stabilize and even sometimes allow improvement of amyloidosis lesions. Copyright © 2017 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights

[Cardiac tamponade and myocarditis in Churg-Strauss syndrome].

PubMed

Baili, L; Aydi, Z; Soussi, G; Ben Dhaou B, B; Zidi, A; Berraies, A; Boussema, F; Kammoun, S; Hamzaoui, A; Kraiem, S; Ben Miled M'rad, K; Rokbani, L

2014-09-01

The successive occurrence of pericardial tamponade and myocarditis during a Churg-Strauss syndrome is exceptionally described. We report a patient in whom pericardial tamponade and myocarditis were the presenting manifestation of a Churg-Strauss syndrome. A 58-year-old woman was admitted because of alteration of the clinical status with eosinophilia. One month ago, she was hospitalized for a pericardial tamponade treated by pericardial drainage. Acute myocarditis was diagnosed on chest pain during the second hospitalization. The etiologic inquiry ended in the diagnosis of Churg-Strauss complicated with a double cardiac involvement. A good response of clinical and biological anomalies was obtained after corticosteroid and immunosuppressive treatment. Isolated or multiple involvements of cardiac tunics should lead to make diagnosis of systemic vasculitis. A complete initial assessment and a close observation of the patients followed for Churg-Strauss syndrome is imperative to detect a cardiac achievement and set up an early treatment. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Comparison of ArcGIS and SAS Geostatistical Analyst to Estimate Population-Weighted Monthly Temperature for US Counties.

PubMed

Xiaopeng, Q I; Liang, Wei; Barker, Laurie; Lekiachvili, Akaki; Xingyou, Zhang

Temperature changes are known to have significant impacts on human health. Accurate estimates of population-weighted average monthly air temperature for US counties are needed to evaluate temperature's association with health behaviours and disease, which are sampled or reported at the county level and measured on a monthly-or 30-day-basis. Most reported temperature estimates were calculated using ArcGIS, relatively few used SAS. We compared the performance of geostatistical models to estimate population-weighted average temperature in each month for counties in 48 states using ArcGIS v9.3 and SAS v 9.2 on a CITGO platform. Monthly average temperature for Jan-Dec 2007 and elevation from 5435 weather stations were used to estimate the temperature at county population centroids. County estimates were produced with elevation as a covariate. Performance of models was assessed by comparing adjusted R 2 , mean squared error, root mean squared error, and processing time. Prediction accuracy for split validation was above 90% for 11 months in ArcGIS and all 12 months in SAS. Cokriging in SAS achieved higher prediction accuracy and lower estimation bias as compared to cokriging in ArcGIS. County-level estimates produced by both packages were positively correlated (adjusted R 2 range=0.95 to 0.99); accuracy and precision improved with elevation as a covariate. Both methods from ArcGIS and SAS are reliable for U.S. county-level temperature estimates; However, ArcGIS's merits in spatial data pre-processing and processing time may be important considerations for software selection, especially for multi-year or multi-state projects.

More adaptive versus less maladaptive coping: What is more predictive of symptom severity? Development of a new scale to investigate coping profiles across different psychopathological syndromes.

PubMed

Moritz, Steffen; Jahns, Anna Katharina; Schröder, Johanna; Berger, Thomas; Lincoln, Tania M; Klein, Jan Philipp; Göritz, Anja S

2016-02-01

Lack of adaptive and enhanced maladaptive coping with stress and negative emotions are implicated in many psychopathological disorders. We describe the development of a new scale to investigate the relative contribution of different coping styles to psychopathology in a large population sample. We hypothesized that the magnitude of the supposed positive correlation between maladaptive coping and psychopathology would be stronger than the supposed negative correlation between adaptive coping and psychopathology. We also examined whether distinct coping style patterns emerge for different psychopathological syndromes. A total of 2200 individuals from the general population participated in an online survey. The Patient Health Questionnaire-9 (PHQ-9), the Obsessive-Compulsive Inventory revised (OCI-R) and the Paranoia Checklist were administered along with a novel instrument called Maladaptive and Adaptive Coping Styles (MAX) questionnaire. Participants were reassessed six months later. MAX consists of three dimensions representing adaptive coping, maladaptive coping and avoidance. Across all psychopathological syndromes, similar response patterns emerged. Maladaptive coping was more strongly related to psychopathology than adaptive coping both cross-sectionally and longitudinally. The overall number of coping styles adopted by an individual predicted greater psychopathology. Mediation analysis suggests that a mild positive relationship between adaptive and certain maladaptive styles (emotional suppression) partially accounts for the attenuated relationship between adaptive coping and depressive symptoms. Results should be replicated in a clinical population. Results suggest that maladaptive and adaptive coping styles are not reciprocal. Reducing maladaptive coping seems to be more important for outcome than enhancing adaptive coping. The study supports transdiagnostic approaches advocating that maladaptive coping is a common factor across different psychopathologies

SPSS and SAS programs for comparing Pearson correlations and OLS regression coefficients.

PubMed

Weaver, Bruce; Wuensch, Karl L

2013-09-01

Several procedures that use summary data to test hypotheses about Pearson correlations and ordinary least squares regression coefficients have been described in various books and articles. To our knowledge, however, no single resource describes all of the most common tests. Furthermore, many of these tests have not yet been implemented in popular statistical software packages such as SPSS and SAS. In this article, we describe all of the most common tests and provide SPSS and SAS programs to perform them. When they are applicable, our code also computes 100 × (1 - α)% confidence intervals corresponding to the tests. For testing hypotheses about independent regression coefficients, we demonstrate one method that uses summary data and another that uses raw data (i.e., Potthoff analysis). When the raw data are available, the latter method is preferred, because use of summary data entails some loss of precision due to rounding.

Early Intestinal Rehabilitation Therapy Ameliorates Intestinal Adaptation in Children with Short Bowel Syndrome: The Long-Term Outcome.

PubMed

Guo, Mingxiao; Lu, Chunlei; Li, Yousheng

2016-12-01

In the management of short bowel syndrome (SBS), the benefits of treatment with growth hormone (GH), glutamine, and enteral nutrition (EN) on intestinal adaptation among children patients is still controversial. The aim of present study is to determine whether GH, glutamine, and EN have positive effect on intestinal adaptation in children with SBS. Sixteen children with SBS (small bowel remnant length, 56.75 ± 8.09 cm; mean ± SE) were treated with GH (0.05 mg/kg/d), glutamine (0.45 mg/kg/d), plus EN-enriched fiber diet for four weeks. After four weeks of treatment, patients were discharged home; GH was discontinued, but the EN with glutamine was continued. Repeated treatment was performed if there were lose weight, dysplasia, or severe diarrhea. All patients completed the treatment. Body weight, intestinal absorptive capacity, and plasma levels of proteins were significantly improved after complete treatment, without any major adverse effects. On follow-up, no death was reported. Treatment with GH, glutamine, and EN in early stage significantly improved intestinal adaptation in pediatric patients with SBS. Furthermore, the positive effect of the treatment does not seem to be sustained once GH discontinued until the residual intestinal adaptation reaches its maximum.

Oxybuprocaine induces a false-positive response in immunochromatographic SAS Adeno Test.

PubMed

Hoshino, Takeshi; Takanashi, Taiji; Okada, Morio; Uchida, Sunao

2002-04-01

To investigate whether a solution of oxybuprocaine hydrochloride, 0.4%, results in a false-positive response in an immunochromatographic SAS Adeno Test. Experimental study. Physiologic saline and 2% lidocaine. Each chemical (100 microl) was diluted in a transport medium. Five drops (200 microl) of the resultant solution were dispensed into the round sample well of a test device. Fifteen samples were tested in each group. Ten minutes after the start of the test, a colored line in the "specimen" portion of the test membrane was visually read as positive or negative by a masked technician. No positive reaction was observed in the control groups (physiologic saline and lidocaine). A false-positive reaction was observed in six samples (33.3%) in the oxybuprocaine group. The positive rate was significantly higher in the oxybuprocaine group compared with those in control groups (P = 0.0062, Fisher's extract probability test). Oxybuprocaine may induce a false-positive reaction in an immunochromatographic SAS Adeno Test. We recommend the use of lidocaine, instead of oxybuprocaine, for local anesthesia in taking eye swabs from patients with suspected adenovirus infection.

Houston, We Have a Problem: Teachers Find No Value in the SAS Education Value-Added Assessment System (EVAAS®)

ERIC Educational Resources Information Center

Collins, Clarin

2014-01-01

This study examined the SAS Education Value-Added Assessment System (EVAAS®) in practice, as perceived and experienced by teachers in the Southwest School District (SSD). To evaluate teacher effectiveness, SSD is using SAS EVAAS® for high-stakes consequences more than any other district or state in the country. A mixed-method design including a…

Considerations for Serving Adolescents with Usher's Syndrome.

ERIC Educational Resources Information Center

Fillman, Robyn D.; And Others

1989-01-01

Usher's syndrome is described, with emphasis on the visual symptomatology characteristic of retinitis pigmentosa. Also discussed are the services needed by individuals with Usher's Syndrome, the syndrome's psychosocial aspects, ways to prevent self-defeating behavior, orientation and mobility, and classroom adaptations. (JDD)

The importance of chromosome studies in Roberts syndrome/SC phocomelia and other cohesinopathies.

PubMed

Gerkes, Erica H; van der Kevie-Kersemaekers, Anne-Marie F; Yakin, Mariam; Smeets, Dominique F C M; van Ravenswaaij-Arts, Conny M A

2010-01-01

Roberts syndrome/SC phocomelia is a rare, autosomal recessive syndrome characterised by pre- and postnatal growth retardation, microcephaly, craniofacial anomalies, mental retardation, and tetraphocomelia in varying degrees of severity. The clinical diagnosis can be challenging in phenotypically mild cases. In the extremely mild case presented here, specific mitotic abnormalities were detected and proved to be very helpful, since Roberts syndrome/SC phocomelia could be diagnosed after finding premature centromere separation and somatic aneuploidy at routine karyotyping. We discuss these and other mitotic cytogenetic abnormalities that can be of significant diagnostic importance, but which will be missed if only array studies are performed. We also discuss the difference between premature centromere separation and premature (sister) chromatid separation. Copyright (c) 2009 Elsevier Masson SAS. All rights reserved.

[L-carnitine treatment and fish odor syndrome: an unwaited adverse effect].

PubMed

Rocher, F; Caruba, C; Broly, F; Lebrun, C

2011-01-01

Levocarnitine treatment is usually well tolerated, with essentially dose-dependent diarrhea as the main induced adverse effect. We report a case of fish odor syndrome during levocarnitine treatment which resolved after levocarnitine discontinuation. This adverse effect seems to be correlated with excedent carnitine intake and might be expressed when the elimination pathway becomes saturated or in a situation of deficiency enzymatic metabolism. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Adult zebrafish intestine resection: a novel model of short bowel syndrome, adaptation, and intestinal stem cell regeneration.

PubMed

Schall, K A; Holoyda, K A; Grant, C N; Levin, D E; Torres, E R; Maxwell, A; Pollack, H A; Moats, R A; Frey, M R; Darehzereshki, A; Al Alam, D; Lien, C; Grikscheit, T C

2015-08-01

Loss of significant intestinal length from congenital anomaly or disease may lead to short bowel syndrome (SBS); intestinal failure may be partially offset by a gain in epithelial surface area, termed adaptation. Current in vivo models of SBS are costly and technically challenging. Operative times and survival rates have slowed extension to transgenic models. We created a new reproducible in vivo model of SBS in zebrafish, a tractable vertebrate model, to facilitate investigation of the mechanisms of intestinal adaptation. Proximal intestinal diversion at segment 1 (S1, equivalent to jejunum) was performed in adult male zebrafish. SBS fish emptied distal intestinal contents via stoma as in the human disease. After 2 wk, S1 was dilated compared with controls and villus ridges had increased complexity, contributing to greater villus epithelial perimeter. The number of intervillus pockets, the intestinal stem cell zone of the zebrafish increased and contained a higher number of bromodeoxyuridine (BrdU)-labeled cells after 2 wk of SBS. Egf receptor and a subset of its ligands, also drivers of adaptation, were upregulated in SBS fish. Igf has been reported as a driver of intestinal adaptation in other animal models, and SBS fish exposed to a pharmacological inhibitor of the Igf receptor failed to demonstrate signs of intestinal adaptation, such as increased inner epithelial perimeter and BrdU incorporation. We describe a technically feasible model of human SBS in the zebrafish, a faster and less expensive tool to investigate intestinal stem cell plasticity as well as the mechanisms that drive intestinal adaptation. Copyright © 2015 the American Physiological Society.

Adult zebrafish intestine resection: a novel model of short bowel syndrome, adaptation, and intestinal stem cell regeneration

PubMed Central

Schall, K. A.; Holoyda, K. A.; Grant, C. N.; Levin, D. E.; Torres, E. R.; Maxwell, A.; Pollack, H. A.; Moats, R. A.; Frey, M. R.; Darehzereshki, A.; Al Alam, D.; Lien, C.

2015-01-01

Loss of significant intestinal length from congenital anomaly or disease may lead to short bowel syndrome (SBS); intestinal failure may be partially offset by a gain in epithelial surface area, termed adaptation. Current in vivo models of SBS are costly and technically challenging. Operative times and survival rates have slowed extension to transgenic models. We created a new reproducible in vivo model of SBS in zebrafish, a tractable vertebrate model, to facilitate investigation of the mechanisms of intestinal adaptation. Proximal intestinal diversion at segment 1 (S1, equivalent to jejunum) was performed in adult male zebrafish. SBS fish emptied distal intestinal contents via stoma as in the human disease. After 2 wk, S1 was dilated compared with controls and villus ridges had increased complexity, contributing to greater villus epithelial perimeter. The number of intervillus pockets, the intestinal stem cell zone of the zebrafish increased and contained a higher number of bromodeoxyuridine (BrdU)-labeled cells after 2 wk of SBS. Egf receptor and a subset of its ligands, also drivers of adaptation, were upregulated in SBS fish. Igf has been reported as a driver of intestinal adaptation in other animal models, and SBS fish exposed to a pharmacological inhibitor of the Igf receptor failed to demonstrate signs of intestinal adaptation, such as increased inner epithelial perimeter and BrdU incorporation. We describe a technically feasible model of human SBS in the zebrafish, a faster and less expensive tool to investigate intestinal stem cell plasticity as well as the mechanisms that drive intestinal adaptation. PMID:26089336

Defective adaptive thermogenesis contributes to metabolic syndrome and liver steatosis in obese mice.

PubMed

Poekes, Laurence; Legry, Vanessa; Schakman, Olivier; Detrembleur, Christine; Bol, Anne; Horsmans, Yves; Farrell, Geoffrey C; Leclercq, Isabelle A

2017-02-01

Fatty liver diseases are complications of the metabolic syndrome associated with obesity, insulin resistance and low grade inflammation. Our aim was to uncover mechanisms contributing to hepatic complications in this setting. We used foz/foz mice prone to obesity, insulin resistance and progressive fibrosing non-alcoholic steatohepatitis (NASH). Foz/foz mice are hyperphagic but wild-type (WT)-matched calorie intake failed to protect against obesity, adipose inflammation and glucose intolerance. Obese foz/foz mice had similar physical activity level but reduced energy expenditure. Thermogenic adaptation to high-fat diet (HFD) or to cold exposure was severely impaired in foz/foz mice compared with HFD-fed WT littermates due to lower sympathetic tone in their brown adipose tissue (BAT). Intermittent cold exposure (ICE) restored BAT function and thereby improved glucose tolerance, decreased fat mass and liver steatosis. We conclude that failure of BAT adaptation drives the metabolic complications of obesity in foz/foz mice, including development of liver steatosis. Induction of endogenous BAT function had a significant therapeutic impact on obesity, glucose tolerance and liver complications and is a potential new avenue for therapy of non-alcoholic fatty liver disease (NAFLD). © 2017 The Author(s). published by Portland Press Limited on behalf of the Biochemical Society.

Kindler syndrome complicated by invasive squamous cell carcinoma of the palate.

PubMed

Souldi, H; Bajja, M Y; Mahtar, M

2018-02-01

Kindler syndrome is a very rare, autosomal recessive genodermatosis characterized by skin fragility and photosensitivity in infancy with progressive poikiloderma. We report the case of a young woman with a history of Kindler syndrome predominantly characterized by extensive involvement of the oropharyngeal mucosa. The patient presented with an ulcerative lesion of the palate. Computed tomography and biopsy concluded on unresectable invasive squamous cell carcinoma of the hard palate. Neoadjuvant chemotherapy was proposed, but the patient died after the first course of chemotherapy in a context of severe gastrointestinal mucositis and generalized sepsis. Mucosal manifestations of Kindler syndrome have been described in the literature, but very few cases of malignant transformation to squamous cell carcinoma have been reported, although it is a very well known, long-term complication of this disease. To our knowledge, this is the second reported case of Kindler syndrome complicated by invasive squamous cell carcinoma of the hard palate. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

System definition phase and acquisition phase project plan for Small Astronomy Satellite SAS-D

NASA Technical Reports Server (NTRS)

1971-01-01

The objective of the SAS-D project is to conduct spectral distribution studies of celestial ultraviolet sources using an Explorer-class spacecraft launched by a Delta vehicle into a geosynchronous orbit in the last half of 1975. The telescope system is intended for use by guest astronomers for a major portion of the total observing time. The concept of the overall system, designed to resemble functionally the operation of a ground-based observatory, should maximize the usefulness of the instrument to the astronomical community by limiting the amount of special instruction needed to use the spaceborne telescope. The SAS-D mission will obtain information on what stars, nebulae, and galaxies are and how they develop.

[Prevalence of chronic hyperventilation syndrome in children and teenagers].

PubMed

Gridina, I; Bidat, E; Chevallier, B; Stheneur, C

2013-03-01

The aim of this study was to investigate the prevalence of hyperventilation syndrome in the general population of children and teenagers from the Île-de-France region (France). Three hundred children and teenagers (170 girls and 130 boys, aged 1 to17 years) were included in the study. To evaluate the probability of hyperventilation syndrome, we asked the children and teenagers to complete the Hyperventilation Syndrome Ambroise-Paré Enfant (SHAPE) questionnaire. The frequency of occurrence of the signs was evaluated by the child himself or herself with or without parental help. Children and teenagers with a score of 25 or over were considered to have hyperventilation syndrome. Sixty-three out of 300 questionnaires with a score of 25 or over revealed the presence of hyperventilation syndrome: 21% of the population evaluated. Among those surveyed, 42 were girls and 21 boys: 24.7 and 16.2%, respectively. The 280 questionnaires filled out among the non-asthmatics showed that 52 were positive (18.6%), while the positivity rate in the asthma group amounted to 55%. Although the diagnostic criteria for hyperventilation syndrome remains contested, this study shows that the disorder is real and frequent. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Comparison of ArcGIS and SAS Geostatistical Analyst to Estimate Population-Weighted Monthly Temperature for US Counties

PubMed Central

Xiaopeng, QI; Liang, WEI; BARKER, Laurie; LEKIACHVILI, Akaki; Xingyou, ZHANG

2015-01-01

Temperature changes are known to have significant impacts on human health. Accurate estimates of population-weighted average monthly air temperature for US counties are needed to evaluate temperature’s association with health behaviours and disease, which are sampled or reported at the county level and measured on a monthly—or 30-day—basis. Most reported temperature estimates were calculated using ArcGIS, relatively few used SAS. We compared the performance of geostatistical models to estimate population-weighted average temperature in each month for counties in 48 states using ArcGIS v9.3 and SAS v 9.2 on a CITGO platform. Monthly average temperature for Jan-Dec 2007 and elevation from 5435 weather stations were used to estimate the temperature at county population centroids. County estimates were produced with elevation as a covariate. Performance of models was assessed by comparing adjusted R2, mean squared error, root mean squared error, and processing time. Prediction accuracy for split validation was above 90% for 11 months in ArcGIS and all 12 months in SAS. Cokriging in SAS achieved higher prediction accuracy and lower estimation bias as compared to cokriging in ArcGIS. County-level estimates produced by both packages were positively correlated (adjusted R2 range=0.95 to 0.99); accuracy and precision improved with elevation as a covariate. Both methods from ArcGIS and SAS are reliable for U.S. county-level temperature estimates; However, ArcGIS’s merits in spatial data pre-processing and processing time may be important considerations for software selection, especially for multi-year or multi-state projects. PMID:26167169

[Which treatments for the hyperventilation syndrome in adults?

PubMed

Rapin, A; Deslee, G; Percebois-Macadre, L; Jonvel, A-C; Demangeon, S; Boyer, F C

2017-02-01

The hyperventilation syndrome is a complex entity whose management is poorly codified. We report a synthesis about the management of adult patients diagnosed with hyperventilation syndrome. A systematic literature review has identified fifteen articles dealing, among them three studies about drug treatment and the others about non-pharmacological approaches. Among the last ones, a re-educational approach based on abdominal ventilation and regulation of the ventilatory rate seems and an educative approach seems to be the most effective. Methodological biases did not permit a conclusion on the efficacy of these treatments. Practically, teaching abdominal ventilation and respiratory rate regulation, associated with a personalized therapeutic education, seems to be a pertinent management approach. Other clinical studies should explore this issue. Copyright © 2016 SPLF. Published by Elsevier Masson SAS. All rights reserved.

Placental transfer of antidepressant medications: implications for postnatal adaptation syndrome.

PubMed

Ewing, Grace; Tatarchuk, Yekaterina; Appleby, Dina; Schwartz, Nadav; Kim, Deborah

2015-04-01

Seven to thirteen percent of women are either prescribed or taking (depending on the study) an antidepressant during pregnancy. Because antidepressants freely cross into the intrauterine environment, we aim to summarize the current findings on placental transfer of antidepressants. Although generally low risk, antidepressants have been associated with postnatal adaptation syndrome (PNAS). Specifically, we explore whether the antidepressants most closely associated with PNAS (paroxetine, fluoxetine, venlafaxine) cross the placenta to a greater extent than other antidepressants. We review research on antidepressants in the context of placental anatomy, placental transport mechanisms, placental metabolism, pharmacokinetics, as well as non-placental maternal and fetal factors. This provides insight into the complexity involved in understanding how placental transfer of antidepressants may relate to adverse perinatal outcomes. Ultimately, from this data there is no pattern in which PNAS is related to placental transfer of antidepressant medications. In general, there is large interindividual variability for each type of antidepressant. To make the most clinically informed decisions about the use of antidepressants in pregnancy, studies that link maternal, placental and fetal genetic polymorphisms, placental transfer rates and infant outcomes are needed.

SAS-2 observations of celestial diffuse gamma radiation above 30 MeV

NASA Technical Reports Server (NTRS)

Thompson, D. J.; Fichtel, C. E.; Kniffen, D. A.; Hartman, R. C.

1974-01-01

The small astronomy satellite, SAS-2, used a 32-deck magnetic core digitized spark chamber to study gamma rays with energies above 30 MeV. Data for four regions of the sky away from the galactic plane were analyzed. These regions show a finite, diffuse flux of gamma rays with a steep energy spectrum, and the flux is uniform over all the regions. Represented by a power law, the differential energy spectrum shows an index of 2.5 + or - 0.4. The steep SAS-2 spectrum and the lower energy data are reasonably consistent with a neutral pion gamma-ray spectrum which was red-shifted (such as that proposed by some cosmological theories). It is concluded that the diffuse celestial gamma ray spectrum observed presents the possibility of cosmological studies and possible evidence for a residual cosmic ray density, and supports the galactic superclusters of matter and antimatter remaining from baryon-symmetric big bang.

Assessing the severity of sleep apnea syndrome based on ballistocardiogram

PubMed Central

Zhou, Xingshe; Zhao, Weichao; Liu, Fan; Ni, Hongbo; Yu, Zhiwen

2017-01-01

Background Sleep Apnea Syndrome (SAS) is a common sleep-related breathing disorder, which affects about 4-7% males and 2-4% females all around the world. Different approaches have been adopted to diagnose SAS and measure its severity, including the gold standard Polysomnography (PSG) in sleep study field as well as several alternative techniques such as single-channel ECG, pulse oximeter and so on. However, many shortcomings still limit their generalization in home environment. In this study, we aim to propose an efficient approach to automatically assess the severity of sleep apnea syndrome based on the ballistocardiogram (BCG) signal, which is non-intrusive and suitable for in home environment. Methods We develop an unobtrusive sleep monitoring system to capture the BCG signals, based on which we put forward a three-stage sleep apnea syndrome severity assessment framework, i.e., data preprocessing, sleep-related breathing events (SBEs) detection, and sleep apnea syndrome severity evaluation. First, in the data preprocessing stage, to overcome the limits of BCG signals (e.g., low precision and reliability), we utilize wavelet decomposition to obtain the outline information of heartbeats, and apply a RR correction algorithm to handle missing or spurious RR intervals. Afterwards, in the event detection stage, we propose an automatic sleep-related breathing event detection algorithm named Physio_ICSS based on the iterative cumulative sums of squares (i.e., the ICSS algorithm), which is originally used to detect structural breakpoints in a time series. In particular, to efficiently detect sleep-related breathing events in the obtained time series of RR intervals, the proposed algorithm not only explores the practical factors of sleep-related breathing events (e.g., the limit of lasting duration and possible occurrence sleep stages) but also overcomes the event segmentation issue (e.g., equal-length segmentation method might divide one sleep-related breathing event

[Exceptional association of bilateral popliteal aneurysm with an abdominal aortic aneurysm in Marfan syndrome].

PubMed

Tijani, Y; Mameli, A; Chtata, H; Taberkant, M; Lekehal, B; Sefiani, Y; Elmesnaoui, A; Ammar, F; Bensaid, Y; Feito, B; Bellenot, F; Fallouh, A; Cheysson, E

2014-07-01

Marfan syndrome is an autosomal dominant disorder with rheumatoid, ophthalmological, neurological, cutaneous and cardiovascular manifestations. Aneurysmal lesions affecting both the abdominal aorta and the peripheral arteries are not often described in the literature. We report a case associating a bilateral popliteal aneurysm and an aneurysm of the infra-renal abdominal aorta. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

RAAS inhibition and cardiorenal syndrome.

PubMed

Onuigbo, Macaulay Amechi C

2014-01-01

The consensus conference on cardio-renal syndromes (2008) defined 'cardio-renal syndromes' as 'disorders of the heart and kidneys whereby acute or chronic dysfunction in one organ may induce acute or chronic dysfunction of the other' and identified five subtypes of the syndromes. Various pathophysiologic mechanisms underlie cardiorenal syndrome including hemodynamic derangements, reduced cardiac output leading to impaired renal perfusion, reduced stroke volume, raised atrial filling pressures, elevated atrial pressures, sodium and water retention, venous congestion, right ventricular dysfunction and venous hypertension causing increased renal venous pressure, intra-abdominal hypertension, various neurohormonal adaptations including activation of the renin-angiotensin-aldosterone system, adaptive activation of the sympathetic nervous system, cytokine release and oxidative stress. Although there are standardized clinical guidelines for the management of heart failure, and chronic kidney disease, respectively, there are no similar consensus clinical guidelines for the management of the cardiorenal syndromes. RAAS inhibition is advocated in treating systolic heart failure. There is evidence that RAAS inhibition is also useful in cardiorenal syndrome. However, RAAS inhibition, while potentially useful in the management of cardiorenal syndrome, is not the 'magic bullet', is sometimes limited by adverse renal events, is not applicable to all patients, and must be applied by physicians with due diligence and caution. Nevertheless, a more comprehensive multidisciplinary multipronged approach to managing patients with cardiorenal syndrome is even more pragmatic and commonsense given the multiple mechanisms and pathogenetic pathways implicated in the causation and perpetuation of cardiorenal syndrome.

Development and Validation of the Self-Acceptance Scale for Persons with Early Blindness: The SAS-EB

PubMed Central

Morgado, Fabiane Frota da Rocha; Campana, Angela Nogueira Neves Betanho; Tavares, Maria da Consolação Gomes Cunha Fernandes

2014-01-01

Investigations of self-acceptance are critical to understanding the development and maintenance of psychological health. However, valid and reliable instruments for measuring self-acceptance in persons with early blindness have yet to be developed. The current research describes three studies designed to develop and validate the Self-acceptance Scale for Persons with Early Blindness (SAS-EB). In Study 1, we developed the initial item pool. Thirty-three items were generated, based on data from specialized literature and from 2 focus groups. Items were organized in a three-factor structure, theoretically predicted for SAS-EB - (1) body acceptance, (2) self-protection from social stigmas, and (3) feeling and believing in one's capacities. In Study 2, information obtained from a panel of 9 experts and 22 persons with early blindness representing the target population was used to refine the initial item pool, generating a new pool of 27 items. In Study 3, 318 persons with early blindness (141 women and 177 men), between 18 and 60 years of age (M = 37.74 years, SD = 12.37) answered the new pool of 27 items. After the elimination of 9 items using confirmatory factor analysis, we confirmed the theoretical three-factor structure of the SAS-EB. Study 3 also provided support for the scale's internal consistency and construct validity. Finally, the psychometric properties of the SAS-EB, its utility, and its limitations are discussed along with considerations for future research. PMID:25268633

Effects of enteral arginine supplementation on the structural intestinal adaptation in a rat model of short bowel syndrome.

PubMed

Sukhotnik, Igor; Lerner, Aaron; Sabo, Edmund; Krausz, Michael M; Siplovich, Leonardo; Coran, Arnold G; Mogilner, Jorge; Shiloni, Eitan

2003-07-01

The nitric oxide precursor L-arginine (ARG) has been shown to influence intestinal morphology and intestinal absorptive function. The purpose of the present study was to determine the effect of enteral ARG supplementation on structural intestinal adaptation, cell proliferation, and apoptosis in a rat model of short bowel syndrome (SBS). Thirty male Sprague-Dawley rats were divided into three experimental groups: Sham rats underwent bowel transection, SBS rats underwent 75% small bowel resection, and SBS-ARG rats underwent bowel resection and were treated with ARG given in the drinking water (2%). Parameters of intestinal adaptation, enterocyte proliferation and enterocyte apoptosis were determined on day 14 following operation. We have demonstrated that SBS-ARG animals had a lower jejunal and ileal mucosal weight, jejunal mucosal DNA and protein, ileal mucosal protein, jejunal villus height, jejunal and ileal crypt depth, and enterocyte proliferation index and a greater enterocyte apoptosis compared to SBS untreated animals. We conclude that in a rat model of SBS enteral L-arginine inhibits structural intestinal adaptation. Possible mechanism for this effect may be decreased cell proliferation and increased cell apoptosis.

[Head and neck adaptive radiotherapy].

PubMed

Graff, P; Huger, S; Kirby, N; Pouliot, J

2013-10-01

Onboard volumetric imaging systems can provide accurate data of the patient's anatomy during a course of head and neck radiotherapy making it possible to assess the actual delivered dose and to evaluate the dosimetric impact of complex daily positioning variations and gradual anatomic changes such as geometric variations of tumors and normal tissues or shrinkage of external contours. Adaptive radiotherapy is defined as the correction of a patient's treatment planning to adapt for individual variations observed during treatment. Strategies are developed to selectively identify patients that require replanning because of an intolerable dosimetric drift. Automated tools are designed to limit time consumption. Deformable image registration algorithms are the cornerstones of these strategies, but a better understanding of their limits of validity is required before adaptive radiotherapy can be safely introduced to daily practice. Moreover, strict evaluation of the clinical benefits is yet to be proven. Copyright © 2013 Société française de radiothérapie oncologique (SFRO). Published by Elsevier SAS. All rights reserved.

An essential role of the basal body protein SAS-6 in Plasmodium male gamete development and malaria transmission

PubMed Central

Marques, Sara R; Ramakrishnan, Chandra; Carzaniga, Raffaella; Blagborough, Andrew M; Delves, Michael J; Talman, Arthur M; Sinden, Robert E

2015-01-01

Gametocytes are the sole Plasmodium parasite stages that infect mosquitoes; therefore development of functional gametes is required for malaria transmission. Flagellum assembly of the Plasmodium male gamete differs from that of most other eukaryotes in that it is intracytoplasmic but retains a key conserved feature: axonemes assemble from basal bodies. The centriole/basal body protein SAS-6 normally regulates assembly and duplication of these organelles and its depletion causes severe flagellar/ciliary abnormalities in a diverse array of eukaryotes. Since basal body and flagellum assembly are intimately coupled to male gamete development in Plasmodium, we hypothesized that SAS-6 disruption may cause gametogenesis defects and perturb transmission. We show that Plasmodium berghei sas6 knockouts display severely abnormal male gametogenesis presenting reduced basal body numbers, axonemal assembly defects and abnormal nuclear allocation. The defects in gametogenesis reduce fertilization and render Pbsas6 knockouts less infectious to mosquitoes. Additionally, we show that lack of Pbsas6 blocks transmission from mosquito to vertebrate host, revealing an additional yet undefined role in ookinete to sporulating oocysts transition. These findings underscore the vulnerability of the basal body/SAS-6 to malaria transmission blocking interventions. PMID:25154861

SAS-4 is recruited to a dynamic structure in newly forming centrioles that is stabilized by the gamma-tubulin-mediated addition of centriolar microtubules.

PubMed

Dammermann, Alexander; Maddox, Paul S; Desai, Arshad; Oegema, Karen

2008-02-25

Centrioles are surrounded by pericentriolar material (PCM), which is proposed to promote new centriole assembly by concentrating gamma-tubulin. Here, we quantitatively monitor new centriole assembly in living Caenorhabditis elegans embryos, focusing on the conserved components SAS-4 and SAS-6. We show that SAS-4 and SAS-6 are coordinately recruited to the site of new centriole assembly and reach their maximum levels during S phase. Centriolar SAS-6 is subsequently reduced by a mechanism intrinsic to the early assembly pathway that does not require progression into mitosis. Centriolar SAS-4 remains in dynamic equilibrium with the cytoplasmic pool until late prophase, when it is stably incorporated in a step that requires gamma-tubulin and microtubule assembly. These results indicate that gamma-tubulin in the PCM stabilizes the nascent daughter centriole by promoting microtubule addition to its outer wall. Such a mechanism may help restrict new centriole assembly to the vicinity of preexisting parent centrioles that recruit PCM.

C-C1-04: How to Win Friends and Influence People with the SAS Output Delivery System

PubMed Central

Tolbert, William

2010-01-01

Background and Aims: Long-time SAS users remember the days when SAS output was embarrassingly ugly. Version 7 saw the introduction of the Output Delivery System (ODS). ODS has matured into a very capable subsystem that gives users powerful reporting options. This presentation will highlight useful features and outline a macro-based system for handling multiple ODS destinations simultaneously. Nowadays there is no excuse for ugly SAS output! When building reports, SAS users should think about the needs of those using the reports. Some people just want to review frequency tables, and are happy to do so on a monitor. Others want to be able to print data for review in a meeting. And, there are always those that want to work with the data in a spreadsheet. Consider the ideal formats for each of the users outlined above. For the casual data browser, HTML output is ideal. For printing, PDF is preferred. And for the additional analysis, Excel is a popular option. With ODS, we can meet all of these needs. Methods: Because ODS permits opening multiple output destinations simultaneously, a single procedure can be used to generate data in HTML, PDF, and Excel at once. The presentation will demonstrate the following: o- basic ODS syntax for HTML, PDF, and Excel output o- custom HTML table of contents o- using the ExcelXP tagset for multi-tab spreadsheets o- a custom macro for managing multiple ODS destinations simultaneously o- simple PROC Template code for easy customization o- techniques for consistent output from multiple platforms. Results: The techniques outlined here have been well-received in a variety of business reporting environments. Conclusions: The SAS ODS provides a wide array of reporting options. Don’t limit yourself to just one type of output.

SPSS and SAS programs for generalizability theory analyses.

PubMed

Mushquash, Christopher; O'Connor, Brian P

2006-08-01

The identification and reduction of measurement errors is a major challenge in psychological testing. Most investigators rely solely on classical test theory for assessing reliability, whereas most experts have long recommended using generalizability theory instead. One reason for the common neglect of generalizability theory is the absence of analytic facilities for this purpose in popular statistical software packages. This article provides a brief introduction to generalizability theory, describes easy to use SPSS, SAS, and MATLAB programs for conducting the recommended analyses, and provides an illustrative example, using data (N = 329) for the Rosenberg Self-Esteem Scale. Program output includes variance components, relative and absolute errors and generalizability coefficients, coefficients for D studies, and graphs of D study results.

Non-linear Growth Models in Mplus and SAS

PubMed Central

Grimm, Kevin J.; Ram, Nilam

2013-01-01

Non-linear growth curves or growth curves that follow a specified non-linear function in time enable researchers to model complex developmental patterns with parameters that are easily interpretable. In this paper we describe how a variety of sigmoid curves can be fit using the Mplus structural modeling program and the non-linear mixed-effects modeling procedure NLMIXED in SAS. Using longitudinal achievement data collected as part of a study examining the effects of preschool instruction on academic gain we illustrate the procedures for fitting growth models of logistic, Gompertz, and Richards functions. Brief notes regarding the practical benefits, limitations, and choices faced in the fitting and estimation of such models are included. PMID:23882134

The presence of alpha interferon at the time of infection alters the innate and adaptive immune responses to porcine reproductive and respiratory syndrome virus

USDA-ARS?s Scientific Manuscript database

Porcine reproductive and respiratory syndrome (PRRS) is one of the most devastating and costly diseases to the swine industry world-wide. Overall, the adaptive immune response to PRRS virus (PRRSV) is weak and results in delayed elimination of virus from the host and inferior vaccine protection. PRR...

Arthroscopic treatment of painful Sinding-Larsen-Johansson syndrome in a professional handball player.

PubMed

Kajetanek, C; Thaunat, M; Guimaraes, T; Carnesecchi, O; Daggett, M; Sonnery-Cottet, B

2016-09-01

Sinding-Larsen-Johansson (SLJ) syndrome is a type of osteochondrosis of the distal pole of the patella most often caused by repeated microtrauma. Here, we describe the case of a professional athlete with painful SLJ syndrome treated arthroscopically. A 29-year-old male professional handball player presented with anterior knee pain that persisted after 4 months of an eccentric rehabilitation protocol and platelet-rich plasma injections. Despite this conservative treatment, the patient could not participate in his sport. The SLJ lesion was excised arthroscopically, which led to complete disappearance of symptoms and return to competitive sports after 5 months. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

A Comparison of Three Programming Models for Adaptive Applications

NASA Technical Reports Server (NTRS)

Shan, Hong-Zhang; Singh, Jaswinder Pal; Oliker, Leonid; Biswa, Rupak; Kwak, Dochan (Technical Monitor)

2000-01-01

We study the performance and programming effort for two major classes of adaptive applications under three leading parallel programming models. We find that all three models can achieve scalable performance on the state-of-the-art multiprocessor machines. The basic parallel algorithms needed for different programming models to deliver their best performance are similar, but the implementations differ greatly, far beyond the fact of using explicit messages versus implicit loads/stores. Compared with MPI and SHMEM, CC-SAS (cache-coherent shared address space) provides substantial ease of programming at the conceptual and program orchestration level, which often leads to the performance gain. However it may also suffer from the poor spatial locality of physically distributed shared data on large number of processors. Our CC-SAS implementation of the PARMETIS partitioner itself runs faster than in the other two programming models, and generates more balanced result for our application.

Williams syndrome and mature B-Leukemia: A random association?

PubMed

Decimi, Valentina; Fazio, Grazia; Dell'Acqua, Fabiola; Maitz, Silvia; Galbiati, Marta; Rizzari, Carmelo; Biondi, Andrea; Cazzaniga, Giovanni; Selicorni, Angelo

2016-12-01

Williams syndrome (WBS) is a rare neurodevelopmental disorder with specific phenotypic characteristics and cardiac abnormalities, but is not considered as a cancer predisposing condition. However, in rare cases, malignancies have been described in patients with WBS, with hematologic cancer (mainly Burkitt Lymphoma and Acute Lymphoblastic Leukemia) as the most represented. We report here the case of a boy with WS and B-NHL. This is the unique case within the large cohort of patients (n = 117) followed in our institution for long time (mean clinical follow-up, 13 years). We herewith propose that the BCL7B gene, located in the chromosomal region commonly deleted in Williams syndrome, could potentially have a role in this particular association. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Osteomyelitis: A rare complication of pancreatitis and PPP-syndrome.

PubMed

Langenhan, Ronny; Reimers, Niklas; Probst, Axel

2016-03-01

Pancreatic diseases can be accompanied by periarthritis with bone necrosis and panniculitis (PPP-syndrome). It is postulated that this is caused by systemic activity of pancreatic enzymes leading to microcirculatory disturbances and fat necrosis. The morbidity and mortality of the PPP-syndrome is high. Successful treatment of pancreatitis can lead to resolution of accompanying panniculitis and periarthritis without adverse sequelae, but weeks or months after pancreatitis, asymptomatic necrosis of the bone may become symptomatic by fracturing spontaneously. In this report, we also describe osteomyelitis as a severe septic complication of bone necrosis caused by pancreatitis, in one case as acute tissue necrosis and in another case months after pancreatitis spread haematogenously. Copyright © 2015 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

Interferon alpha inhibits viral replication of a live-attenuated porcine reproductive and respiratory syndrome virus vaccine preventing development of an adaptive immune response in swine

USDA-ARS?s Scientific Manuscript database

Type I interferons, such as interferon alpha (IFNa), contribute to innate antiviral immunity by promoting production of antiviral mediators and are also involved in promoting an adaptive immune response. Porcine reproductive and respiratory syndrome virus (PRRSV) is one of the most devastating and c...

Design and implementation of reliability evaluation of SAS hard disk based on RAID card

NASA Astrophysics Data System (ADS)

Ren, Shaohua; Han, Sen

2015-10-01

Because of the huge advantage of RAID technology in storage, it has been widely used. However, the question associated with this technology is that the hard disk based on the RAID card can not be queried by Operating System. Therefore how to read the self-information and log data of hard disk has been a problem, while this data is necessary for reliability test of hard disk. In traditional way, this information can be read just suitable for SATA hard disk, but not for SAS hard disk. In this paper, we provide a method by using LSI RAID card's Application Program Interface, communicating with RAID card and analyzing the feedback data to solve the problem. Then we will get the necessary information to assess the SAS hard disk.

No evidence for adaptation of current egg drop syndrome 1976 viruses to chickens.

PubMed

Tsukamoto, K; Kuwabara, M; Kaneko, M; Mase, M; Imai, K

2004-01-01

In order to determine whether the current field strains of egg drop syndrome (EDS) 1976 viruses adapt to chickens, we compared the growth efficiency of three Japanese field strains (PA-1/79, AWI/98, Gifu/01) in chicken and duck embryo liver cells. The growth efficiency in chicken or duck embryo liver cells was almost similar in these strains. The fiber protein may carry the type-specific antigen and the hemagglutination activity, and hexon protein may contain the subgroup-specific antigenic determinants. Therefore, the fiber head and hexon loop 1 DNA domain sequences of the six Japanese field strains UPA-1/79, ME/80, 44/81, Kyoto/91, AWI/98, Gifu/01) were compared, but these DNA domains were identical among the six field strains. Our data suggested that the EDS virus was maintained without discernible changes for the last two decades in the field.

Effect of subcutaneous insulin on intestinal adaptation in a rat model of short bowel syndrome.

PubMed

Sukhotnik, Igor; Mogilner, Jorge; Shamir, Raanan; Shehadeh, Naim; Bejar, Jacob; Hirsh, Mark; Coran, Arnold G

2005-03-01

Insulin has been shown to influence intestinal structure and absorptive function. The purpose of the present study was to evaluate the effects of parenteral insulin on structural intestinal adaptation, cell proliferation, and apoptosis in a rat model of short bowel syndrome (SBS). Male Sprague-Dawley rats were divided into three experimental groups: sham rats underwent bowel transection and reanastomosis, SBS rats underwent a 75% small bowel resection, and SBS-INS rats underwent a 75% small bowel resection and were treated with insulin given subcutaneously at a dose of 1 U/kg, twice daily, from day 3 through day 14. Parameters of intestinal adaptation, enterocyte proliferation, and enterocyte apoptosis were determined on day 15 following operation. SBS rats demonstrated a significant increase in jejunal and ileal bowel and mucosal weight, villus height and crypt depth, and cell proliferation index compared with the sham group. SBS-INS animals demonstrated higher jejunal and ileal bowel and mucosal weights, jejunal and ileal mucosal DNA and protein, and jejunal and ileal crypt depth compared with SBS animals. SBS-INS rats also had a greater cell proliferation index in both jejunum and ileum and a trend toward a decrease in enterocyte apoptotic index in jejunum and ileum compared with the SBS untreated group. In conclusion, parenteral insulin stimulates structural intestinal adaptation in a rat model of SBS. Increased cell proliferation is the main mechanism responsible for increased cell mass.

Effects of microalgae Chlorella species crude extracts on intestinal adaptation in experimental short bowel syndrome.

PubMed

Kerem, Mustafa; Salman, Bulent; Pasaoglu, Hatice; Bedirli, Abdulkadir; Alper, Murat; Katircioglu, Hikmet; Atici, Tahir; Percin, E Ferda; Ofluoglu, Ebru

2008-07-28

To evaluate the effects of chlorella crude extract (CCE) on intestinal adaptation in rats subjected to short bowel syndrome (SBS). Wistar rats weighing 230-260 g were used in the study. After anesthesia a 75% small bowel resection was performed. Rats were randomized and divided into groups. Control group (n = 10): where 5% dextrose was given through a gastrostomy tube, Enteral nutrition (EN) group (n = 10): Isocaloric and isonitrogen EN (Alitraq, Abbott, USA), study group (n = 10): CCE was administrated through a gastrostomy tube. Rats were sacrificed on the fifteenth postoperative day and blood and tissue samples were taken. Histopathologic evaluation, intestinal mucosal protein and DNA levels, intestinal proliferation and apoptosis were determined in intestinal tissues, and total protein, albumin and citrulline levels in blood were studied. In rats receiving CCE, villus lengthening, crypt depth, mucosal DNA and protein levels, intestinal proliferation, and serum citrulline, protein and albumin levels were found to be significantly higher than those in control group. Apoptosis in CCE treated rats was significantly reduced when compared to EN group rats. CCE has beneficial effects on intestinal adaptation in experimental SBS.

[Posterior reversible encephalopathy syndrome induced by a cough and cold drug containing pseudoephedrine].

PubMed

Ebbo, M; Benarous, L; Thomas, G; Jourde, N; Genot, S; Bernit, E; Veit, V; Harlé, J-R; Schleinitz, N

2010-06-01

Posterior reversible encephalopathy syndrome is a clinico-radiological entity characterized by neurologic symptoms in association with usually reversible bilateral posterior hemispheric oedema on neuroimaging. Many pathological conditions and treatments have been associated with this syndrome. We report a 19-year-old woman, followed-up for hypocomplementemic urticarial vasculitis, who presented with a posterior reversible encephalopathy syndrome induced by the intake of an over-the-counter cold remedy containing pseudoephedrine. Clinical manifestations and radiological abnormalities resolved after anti-hypertensive therapy and withdrawal of sympathomimetic drug. The diagnosis of posterior reversible encephalopathy syndrome should be considered in patients with compatible clinical and radiological presentation because of its potential reversibility with an appropriate management. Intake of drugs, including over-the-counter cough and cold drugs, should be looked for in the history as well as autoimmune disorders. Copyright 2010 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

A new SAS program for behavioral analysis of Electrical Penetration Graph (EPG) data

USDA-ARS?s Scientific Manuscript database

A new program is introduced that uses SAS software to duplicate output of descriptive statistics from the Sarria Excel workbook for EPG waveform analysis. Not only are publishable means and standard errors or deviations output, the user also is guided through four relatively simple sub-programs for ...

Adaptive-filtering of trisomy 21: risk of Down syndrome depends on family size and age of previous child

NASA Astrophysics Data System (ADS)

Neuhäuser, Markus; Krackow, Sven

2007-02-01

The neonatal incidence rate of Down syndrome (DS) is well-known to accelerate strongly with maternal age. This non-linearity renders mere accumulation of defects at recombination during prolonged first meiotic prophase implausible as an explanation for DS rate increase with maternal age, but might be anticipated from chromosomal drive (CD) for trisomy 21. Alternatively, as there is selection against genetically disadvantaged embryos, the screening system that eliminates embryos with trisomy 21 might decay with maternal age. In this paper, we provide the first evidence for relaxed filtering stringency (RFS) to represent an adaptive maternal response that could explain accelerating DS rates with maternal age. Using historical data, we show that the proportion of aberrant live births decrease with increased family size in older mothers, that inter-birth intervals are longer before affected neonates than before normal ones, and that primiparae exhibit elevated levels of DS incidence at higher age. These findings are predicted by adaptive RFS but cannot be explained by the currently available alternative non-adaptive hypotheses, including CD. The identification of the relaxation control mechanism and therapeutic restoration of a stringent screen may have considerable medical implications.

A SAS macro for testing differences among three or more independent groups using Kruskal-Wallis and Nemenyi tests.

PubMed

Liu, Yuewei; Chen, Weihong

2012-02-01

As a nonparametric method, the Kruskal-Wallis test is widely used to compare three or more independent groups when an ordinal or interval level of data is available, especially when the assumptions of analysis of variance (ANOVA) are not met. If the Kruskal-Wallis statistic is statistically significant, Nemenyi test is an alternative method for further pairwise multiple comparisons to locate the source of significance. Unfortunately, most popular statistical packages do not integrate the Nemenyi test, which is not easy to be calculated by hand. We described the theory and applications of the Kruskal-Wallis and Nemenyi tests, and presented a flexible SAS macro to implement the two tests. The SAS macro was demonstrated by two examples from our cohort study in occupational epidemiology. It provides a useful tool for SAS users to test the differences among three or more independent groups using a nonparametric method.

Mexican Seismic Alert System's SAS-I algorithm review considering strong earthquakes felt in Mexico City since 1985

NASA Astrophysics Data System (ADS)

Cuellar Martinez, A.; Espinosa Aranda, J.; Suarez, G.; Ibarrola Alvarez, G.; Ramos Perez, S.; Camarillo Barranco, L.

2013-05-01

The Seismic Alert System of Mexico (SASMEX) uses three algorithms for alert activation that involve the distance between the seismic sensing field station (FS) and the city to be alerted; and the forecast for earthquake early warning activation in the cities integrated to the system, for example in Mexico City, the earthquakes occurred with the highest accelerations, were originated in the Pacific Ocean coast, whose distance this seismic region and the city, favors the use of algorithm called Algorithm SAS-I. This algorithm, without significant changes since its beginning in 1991, employs the data that generate one or more FS during P wave detection until S wave detection plus a period equal to the time employed to detect these phases; that is the double S-P time, called 2*(S-P). In this interval, the algorithm performs an integration process of quadratic samples from FS which uses a triaxial accelerometer to get two parameters: amplitude and growth rate measured until 2*(S-P) time. The parameters in SAS-I are used in a Magnitude classifier model, which was made from Guerrero Coast earthquakes time series, with reference to Mb magnitude mainly. This algorithm activates a Public or Preventive Alert if the model predicts whether Strong or Moderate earthquake. The SAS-I algorithm has been operating for over 23 years in the subduction zone of the Pacific Coast of Mexico, initially in Guerrero and followed by Oaxaca; and since March 2012 in the seismic region of Pacific covering the coasts among Jalisco, Colima, Michoacan, Guerrero and Oaxaca, where this algorithm has issued 16 Public Alert and 62 Preventive Alerts to the Mexico City where its soil conditions increase damages by earthquake such as the occurred in September 1985. This work shows the review of the SAS-I algorithm and possible alerts that it could generate from major earthquakes recordings detected by FS or seismometers near the earthquakes, coming from Pacific Ocean Coast whose have been felt in Mexico

Catalog of SAS-2 gamma-ray observations (Fichtel, et al. 1990)

NASA Technical Reports Server (NTRS)

Warren, Wayne H., Jr.

1990-01-01

The machine-readable version of the catalog, as it is currently being distributed from the Astronomical Data Center, is described. The SAS-2 gamma ray catalog contains fluxes measured with the high energy gamma ray telescope flown aboard the second NASA Small Astronomy Satellite. The objects measured include various types of galaxies, quasi-stellar, and BL Lacertae objects, and pulsars. The catalog contains separate files for galaxies, pulsars, other objects, notes, and references.

SAS molecular tests Escherichia coli O157 detection kit. Performance tested method 031203.

PubMed

Bapanpally, Chandra; Montier, Laura; Khan, Shah; Kasra, Akif; Brunelle, Sharon L

2014-01-01

The SAS Molecular tests Escherichia coli O157 Detection method, a loop-mediated isothermal amplification method, performed as well as or better than the U.S. Department of Agriculture, Food Safety Inspection Service Microbiology Laboratory Guidebook and the U.S. Food and Drug Administration Bacteriological Analytical Manual reference methods for ground beef, beef trim, bagged mixed lettuce, and fresh spinach. Ground beef (30% fat, 25 g test portion) was validated for 7-8 h enrichment, leafy greens were validated in a 6-7 h enrichment, and ground beef (30% fat, 375 g composite test portion) and beef trim (375 g composite test portion) were validated in a 16-20 h enrichment. The method performance for meat and leafy green matrixes was also shown to be acceptable under conditions of co-enrichment with Salmonella. Thus, after a short co-enrichment step, ground beef, beef trim, lettuce, and spinach can be tested for both Salmonella and E. coli O157. The SAS Molecular tests Salmonella Detection Kit was validated using the same test portions as for the SAS Molecular tests E. coli O157 Detection Kit and those results are presented in a separate report. Inclusivity and exclusivity testing revealed no false negatives and no false positives among the 50 E. coli 0157 strains, including H7 and non-motile strains, and 30 non-E. coli O157 strains examined. Finally, the method was shown to be robust when variations to DNA extract hold time and DNA volume were varied. The method comparison and robustness data suggest a full 7 h enrichment time should be used for 25 g ground beef test portions.

Asperger syndrome related suicidal behavior: two case studies.

PubMed

Kocourkova, Jana; Dudova, Iva; Koutek, Jiri

2013-01-01

Asperger syndrome hinders adaptation to developmental challenges during childhood and adolescence, particularly with regard to interpersonal relationships. Individuals with Asperger syndrome display lack of empathy and limited ability to understand social and emotional exchanges with other people. Individuals with Asperger syndrome are significantly exposed to the risk of suicidal behavior, especially during adolescence. The authors describe cases of suicidal behavior in two adolescent boys with Asperger syndrome.

Cerebro-fronto-facial syndrome type 3 with polymicrogyria: a clinical presentation of Baraitser-Winter syndrome.

PubMed

Eker, Hatice Koçak; Derinkuyu, Betül Emine; Ünal, Sevim; Masliah-Planchon, Julien; Drunat, Séverine; Verloes, Alain

2014-01-01

Baraitser-Winter syndrome (BRWS) is a rare condition affecting the development of the brain and the face. The most common characteristics are unusual facial appearance including hypertelorism and ptosis, ocular colobomas, hearing loss, impaired neuronal migration and intellectual disability. BRWS is caused by mutations in the ACTB and ACTG1 genes. Cerebro-fronto-facial syndrome (CFFS) is a clinically heterogeneous condition with distinct facial dysmorphism, and brain abnormalities. Three subtypes are identified. We report a female infant with striking facial features and brain anomalies (included polymicrogyria) that fit into the spectrum of the CFFS type 3 (CFFS3). She also had minor anomalies on her hands and feet, heart and kidney malformations, and recurrent infections. DNA investigations revealed c.586C>T mutation (p.Arg196Cys) in ACTB. This mutation places this patient in the spectrum of BRWS. The same mutation has been detected in a polymicrogyric patient reported previously in literature. We expand the malformation spectrum of BRWS/CFFS3, and present preliminary findings for phenotype-genotype correlation in this spectrum. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Study on the social adaptation of Chinese children with down syndrome.

PubMed

Wang, Yan-Xia; Mao, Shan-Shan; Xie, Chun-Hong; Qin, Yu-Feng; Zhu, Zhi-Wei; Zhan, Jian-Ying; Shao, Jie; Li, Rong; Zhao, Zheng-Yan

2007-06-30

To evaluate social adjustment and related factors among Chinese children with Down syndrome (DS). A structured interview and Peabody Picture Vocabulary Test (PPVT) were conducted with a group of 36 DS children with a mean age of 106.28 months, a group of 30 normally-developing children matched for mental age (MA) and a group of 40 normally-developing children matched for chronological age (CA). Mean scores of social adjustment were compared between the three groups, and partial correlations and stepwise multiple regression models were used to further explore related factors. There was no difference between the DS group and the MA group in terms of communication skills. However, the DS group scored much better than the MA group in self-dependence, locomotion, work skills, socialization and self-management. Children in the CA group achieved significantly higher scores in all aspects of social adjustment than the DS children. Partial correlations indicate a relationship between social adjustment and the PPVT raw score and also between social adjustment and age (significant r ranging between 0.24 and 0.92). A stepwise linear regression analysis showed that family structure was the main predictor of social adjustment. Newborn history was also a predictor of work skills, communication, socialization and self-management. Parental education was found to account for 8% of self-dependence. Maternal education explained 6% of the variation in locomotion. Although limited by the small sample size, these results indicate that Chinese DS children have better social adjustment skills when compared to their mental-age-matched normally-developing peers, but that the Chinese DS children showed aspects of adaptive development that differed from Western DS children. Analyses of factors related to social adjustment suggest that effective early intervention may improve social adaptability.

Chiari I malformation as part of the Floating-Harbor syndrome?

PubMed

Kurzbuch, Arthur R; Magdum, Shailendra

2016-12-01

We report the first case of a patient diagnosed with Floating-Harbor syndrome (FHS) and Chiari I malformation. The 3-year-old girl was of proportional short stature, had delay of language development, conductive hearing loss and a high threshold of pain. Diagnosis of Chiari I malformation may be difficult in FHS patients who present with communication problems. Clinicians following patients with FHS should be aware of a possible relation between FHS and Chiari I malformation. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Intestinal mucosal atrophy and adaptation

PubMed Central

Shaw, Darcy; Gohil, Kartik; Basson, Marc D

2012-01-01

Mucosal adaptation is an essential process in gut homeostasis. The intestinal mucosa adapts to a range of pathological conditions including starvation, short-gut syndrome, obesity, and bariatric surgery. Broadly, these adaptive functions can be grouped into proliferation and differentiation. These are influenced by diverse interactions with hormonal, immune, dietary, nervous, and mechanical stimuli. It seems likely that clinical outcomes can be improved by manipulating the physiology of adaptation. This review will summarize current understanding of the basic science surrounding adaptation, delineate the wide range of potential targets for therapeutic intervention, and discuss how these might be incorporated into an overall treatment plan. Deeper insight into the physiologic basis of adaptation will identify further targets for intervention to improve clinical outcomes. PMID:23197881

Modelling the detachment dependence on strike point location in the small angle slot divertor (SAS) with SOLPS

NASA Astrophysics Data System (ADS)

Casali, Livia; Covele, Brent; Guo, Houyang

2017-10-01

The new Small Angle Slot (SAS) divertor in DIII-D is characterized by a shallow-angle target enclosed by a slot structure about the strike point (SP). SOLPS modelling results of SAS have demonstrated divertor closure's utility in widening the range of acceptable densities for adequate heat handling. An extensive database of runs has been built to study the detachment dependence on SP location in SAS. Density scans show that lower Te at lower upstream density occur when the SP is at the critical location in the slot. The cooling front spreads across the entire target at higher densities, in agreement with experimental Langmuir probe measurements. A localized increase of the atomic and molecular density takes place near the SP, which reduces the target incident power density and facilitates detachment at lower upstream density. Systematic scans of variables such as power, transport, and viscosity have been carried out to assess the detachment sensitivity. Therein, a positive role of the viscosity is found. This work supported by DOE Contract Number DE-FC02-04ER54698.

Association of brain-derived neurotrophic factor (BDNF) haploinsufficiency with lower adaptive behaviour and reduced cognitive functioning in WAGR/11p13 deletion syndrome.

PubMed

Han, Joan C; Thurm, Audrey; Golden Williams, Christine; Joseph, Lisa A; Zein, Wadih M; Brooks, Brian P; Butman, John A; Brady, Sheila M; Fuhr, Shannon R; Hicks, Melanie D; Huey, Amanda E; Hanish, Alyson E; Danley, Kristen M; Raygada, Margarita J; Rennert, Owen M; Martinowich, Keri; Sharp, Stephen J; Tsao, Jack W; Swedo, Susan E

2013-01-01

In animal studies, brain-derived neurotrophic factor (BDNF) is an important regulator of central nervous system development and synaptic plasticity. WAGR (Wilms tumour, Aniridia, Genitourinary anomalies, and mental Retardation) syndrome is caused by 11p13 deletions of variable size near the BDNF locus and can serve as a model for studying human BDNF haploinsufficiency (+/-). We hypothesized that BDNF+/- would be associated with more severe cognitive impairment in subjects with WAGR syndrome. Twenty-eight subjects with WAGR syndrome (6-28 years), 12 subjects with isolated aniridia due to PAX6 mutations/microdeletions (7-54 years), and 20 healthy controls (4-32 years) received neurocognitive assessments. Deletion boundaries for the subjects in the WAGR group were determined by high-resolution oligonucleotide array comparative genomic hybridization. Within the WAGR group, BDNF+/- subjects (n = 15), compared with BDNF intact (+/+) subjects (n = 13), had lower adaptive behaviour (p = .02), reduced cognitive functioning (p = .04), higher levels of reported historical (p = .02) and current (p = .02) social impairment, and higher percentage meeting cut-off score for autism (p = .047) on Autism Diagnostic Interview-Revised. These differences remained nominally significant after adjusting for visual acuity. Using diagnostic measures and clinical judgement, 3 subjects (2 BDNF+/- and 1 BDNF+/+) in the WAGR group (10.7%) were classified with autism spectrum disorder. A comparison group of visually impaired subjects with isolated aniridia had cognitive functioning comparable to that of healthy controls. In summary, among subjects with WAGR syndrome, BDNF+/- subjects had a mean Vineland Adaptive Behaviour Compose score that was 14-points lower and a mean intelligence quotient (IQ) that was 20-points lower than BDNF+/+ subjects. Our findings support the hypothesis that BDNF plays an important role in human neurocognitive development. Published by Elsevier Ltd.

Transition management as an intervention for survivor syndrome.

PubMed

Rogers, K A

2000-01-01

In today's health care environment of merged organizations, downsizing and restructuring, employees can be experiencing a debilitating syndrome called "layoff survivor syndrome." This syndrome can have a crippling effect on workers and organizations as employees struggle to adapt to the changed working environment. This article represents my self-reflection as a nursing unit manager who personally experienced survivor sickness and witnessed its impact on the unit staff that I was leading at the time. The work of Noer (1993) is explored to clarify the syndrome and describe how the nursing staff and I manifested the syndrome. The writings of Bridges (1991), Brockner (1992) and Noer (1993) provide timely and relevant insights into managing the impact of layoffs and downsizing on those left behind to carry on. Noer (1993) sees the adaptation to the change as the ability to make the psychological shift from the old business paradigm that perpetuated codependency to the new business paradigm of fostering empowered employees. Bridges (1991) takes us a step further in making this psychological shift to adapt to the new work environment by providing a three phase process he calls transitions. The works of these three authors hold an important message for organizations and employees working in environments that abound with constant change.

[Steady car engine noise does not affect the cognitive abilities of sleep apnea syndrome patients].

PubMed

Mayoralas, L R; Barbé, F; Muñoz, A; Agustí, A G N

2003-09-01

Traffic accidents are more frequent for sleep apnea syndrome (SAS) patients than in the population at large. The mechanisms that underlie this observation are poorly defined. Our working hypothesis was that in SAS patients the steady noise of a car engine might alter cognitive capacities that may be involved in driving, thus increasing the risk of traffic accidents. To test this hypothesis we designed a prospective randomized controlled trial. Eighteen SAS patients (apnea-hypopnea index [SEM] 62 [6] h1) and 18 healthy controls were studied. All the participants were evaluated in random order both in basal conditions and after exposure to the steady noise of a car engine recorded on a compact disc. Their level of vigilance was evaluated (Steer-Clear) as well as their reaction time (PVT 192). Attention, coordination, and memory were measured using the following tests: Wechsler Memory Scale (digit span), the Wechsler Intelligence Scale (digit symbol), and Lezack's Trail Making tests A and B. The SAS patients were slightly younger than the control group (mean 50 [7] vs 57 [11] years, respectively; P=.05). The patients showed a lower level of vigilance than the controls both in basal and engine noise conditions (P<.05). No differences between groups were found for the other variables studied. Exposure to steady car engine noise had no effect on the tests of either group. In conclusion, the results of our study do not support the hypothesis that steady car engine noise significantly alters the cognitive ability of SAS patients.

Small for Size and Flow (SFSF) syndrome: An alternative description for posthepatectomy liver failure.

PubMed

Golriz, Mohammad; Majlesara, Ali; El Sakka, Saroa; Ashrafi, Maryam; Arwin, Jalal; Fard, Nassim; Raisi, Hanna; Edalatpour, Arman; Mehrabi, Arianeb

2016-06-01

Small for Size Syndrome (SFSS) syndrome is a recognizable clinical syndrome occurring in the presence of a reduced mass of liver, which is insufficient to maintain normal liver function. A definition has yet to be fully clarified, but it is a common clinical syndrome following partial liver transplantation and extended hepatectomy, which is characterized by postoperative liver dysfunction with prolonged cholestasis and coagulopathy, portal hypertension, and ascites. So far, this syndrome has been discussed with focus on the remnant size of the liver after partial liver transplantation or extended hepatectomy. However, the current viewpoints believe that the excessive flow of portal vein for the volume of the liver parenchyma leads to over-pressure, sinusoidal endothelial damages and haemorrhage. The new hypothesis declares that in both extended hepatectomy and partial liver transplantation, progression of Small for Size Syndrome is not determined only by the "size" of the liver graft or remnant, but by the hemodynamic parameters of the hepatic circulation, especially portal vein flow. Therefore, we suggest the term "Small for Size and Flow (SFSF)" for this syndrome. We believe that it is important for liver surgeons to know the pathogenesis and manifestation of this syndrome to react early enough preventing non-reversible tissue damages. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

tran-SAS v1.0: a numerical model to compute catchment-scale hydrologic transport using StorAge Selection functions

NASA Astrophysics Data System (ADS)

Benettin, Paolo; Bertuzzo, Enrico

2018-04-01

This paper presents the tran-SAS package, which includes a set of codes to model solute transport and water residence times through a hydrological system. The model is based on a catchment-scale approach that aims at reproducing the integrated response of the system at one of its outlets. The codes are implemented in MATLAB and are meant to be easy to edit, so that users with minimal programming knowledge can adapt them to the desired application. The problem of large-scale solute transport has both theoretical and practical implications. On the one side, the ability to represent the ensemble of water flow trajectories through a heterogeneous system helps unraveling streamflow generation processes and allows us to make inferences on plant-water interactions. On the other side, transport models are a practical tool that can be used to estimate the persistence of solutes in the environment. The core of the package is based on the implementation of an age master equation (ME), which is solved using general StorAge Selection (SAS) functions. The age ME is first converted into a set of ordinary differential equations, each addressing the transport of an individual precipitation input through the catchment, and then it is discretized using an explicit numerical scheme. Results show that the implementation is efficient and allows the model to run in short times. The numerical accuracy is critically evaluated and it is shown to be satisfactory in most cases of hydrologic interest. Additionally, a higher-order implementation is provided within the package to evaluate and, if necessary, to improve the numerical accuracy of the results. The codes can be used to model streamflow age and solute concentration, but a number of additional outputs can be obtained by editing the codes to further advance the ability to understand and model catchment transport processes.

A Next Generation Sequencing custom gene panel as first line diagnostic tool for atypical cases of syndromic obesity: Application in a case of Alström syndrome.

PubMed

Maltese, Paolo E; Iarossi, Giancarlo; Ziccardi, Lucia; Colombo, Leonardo; Buzzonetti, Luca; Crinò, Antonino; Tezzele, Silvia; Bertelli, Matteo

2018-02-01

Obesity phenotype can be manifested as an isolated trait or accompanied by multisystem disorders as part of a syndromic picture. In both situations, same molecular pathways may be involved to different degrees. This evidence is stronger in syndromic obesity, in which phenotypes of different syndromes may overlap. In these cases, genetic testing can unequivocally provide a final diagnosis. Here we describe a patient who met the diagnostic criteria for Alström syndrome only during adolescence. Genetic testing was requested at 25 years of age for a final confirmation of the diagnosis. The genetic diagnosis of Alström syndrome was obtained through a Next Generation Sequencing genetic test approach using a custom-designed gene panel of 47 genes associated with syndromic and non-syndromic obesity. Genetic analysis revealed a novel homozygous frameshift variant p.(Arg1550Lysfs*10) on exon 8 of the ALMS1 gene. This case shows the need for a revision of the diagnostic criteria guidelines, as a consequence of the recent advent of massive parallel sequencing technology. Indications for genetic testing reported in these currently accepted diagnostic criteria for Alström syndrome, were drafted when sequencing was expensive and time consuming. Nowadays, Next Generation Sequencing testing could be considered as first line diagnostic tool not only for Alström syndrome but, more generally, for all those atypical or not clearly distinguishable cases of syndromic obesity, thus avoiding delayed diagnosis and treatments. Early diagnosis permits a better follow-up and pre-symptomatic interventions. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

SAS program for quantitative stratigraphic correlation by principal components

USGS Publications Warehouse

Hohn, M.E.

1985-01-01

A SAS program is presented which constructs a composite section of stratigraphic events through principal components analysis. The variables in the analysis are stratigraphic sections and the observational units are range limits of taxa. The program standardizes data in each section, extracts eigenvectors, estimates missing range limits, and computes the composite section from scores of events on the first principal component. Provided is an option of several types of diagnostic plots; these help one to determine conservative range limits or unrealistic estimates of missing values. Inspection of the graphs and eigenvalues allow one to evaluate goodness of fit between the composite and measured data. The program is extended easily to the creation of a rank-order composite. ?? 1985.

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

PubMed Central

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. PMID:26958463

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

PubMed

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.

[A new case of Rowell's syndrome].

PubMed

Schissler, C; Banea, S; Tortel, M-C; Mahé, A

2017-04-01

This article introduces a new case of Rowell's syndrome, a controversial entity defined by the association of lupus erythematosus and erythema multiforme. A 43-year-old woman was diagnosed with lupus erythematosus induced by esomeprazole. Because her eruption did not improve after withdrawal of the drug, hydroxychloroquine was administered. Two weeks later, the patient described new annular lesions on her chest and arms, both erosive and crusted, and some had a target-like appearance. The oral mucosa was also affected. Histology revealed sub-epidermal blistering with keratinocytic necrosis, strongly suggesting erythema multiforme. Screening for other causes of erythema multiforme proved negative. A positive outcome was achieved with corticosteroids and hydroxychloroquine. One year later, the patient was in complete remission for both lupus erythematosus and erythema multiforme. The association of lupus erythematosus and erythema multiforme first described in 1963 is known as Rowell's syndrome. While diagnostic criteria have been established in the literature, the reality of this entity is still contested. The annular lesions of subacute lupus erythematosus may be confused with the lesions of erythema multiforme. As suggested in the above section, other authors consider Rowell's syndrome to be a singular entity. Indeed, our patient developed lesions distinct from those initially suggesting subacute lupus erythematosus, in particular: the target-like aspect of the elementary lesions, mucosal involvement, a distinct histological aspect, and dissociated outcomes. Ultimately, the definition of Rowell's syndrome remains highly debated. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

[Mastitis revealing Churg-Strauss syndrome].

PubMed

Dannepond, C; Le Fourn, E; de Muret, A; Ouldamer, L; Carmier, D; Machet, L

2014-01-01

Churg-Strauss syndrome often involves the skin, and this may sometimes reveal the disease. A 25-year-old woman was referred to a gynaecologist for inflammation of the right breast with breast discharge. Cytological analysis of the liquid showed numerous inflammatory cells, particularly polymorphonuclear eosinophils and neutrophils. Ultrasound examination of the breast was consistent with galactophoritis. CRP was normal, and hypereosinophilia was seen. The patient was subsequently referred to a dermatology unit. Skin examination revealed inflammation of the entire breast, which was painful, warm and erythematous; the border was oedematous with blisters. Necrotic lesions were also present on the thumbs and knees. Skin biopsy of the breast showed a dermal infiltrate with abundant infiltrate of polymorphonuclear eosinophils, including patchy necrosis and intraepidermal vesicles. Histological examination of a biopsy sample from a thumb revealed eosinophilic granuloma and leukocytoclastic vasculitis. The patient was also presenting asthma, pulmonary infiltrates and mononeuropathy at L3, consistent with Churg-Strauss syndrome. Breast involvement in Churg-Strauss syndrome is very rare (only one other case has been reported). This is the first case in which the breast condition revealed the disease. Cutaneous involvement of the breast is, however, also compatible with Wells' cellulitis. The lesions quickly disappeared with 1mg/kg/d oral prednisolone. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Development and psychometric properties of the Suicidality: Treatment Occurring in Paediatrics (STOP) Suicidality Assessment Scale (STOP-SAS) in children and adolescents.

PubMed

Flamarique, I; Santosh, P; Zuddas, A; Arango, C; Purper-Ouakil, D; Hoekstra, P J; Coghill, D; Schulze, U; Dittmann, R W; Buitelaar, J K; Lievesley, K; Frongia, R; Llorente, C; Méndez, I; Sala, R; Fiori, F; Castro-Fornieles, J

2016-12-13

To create a self-reported, internet-based questionnaire for the assessment of suicide risk in children and adolescents. As part of the EU project 'Suicidality: Treatment Occurring in Paediatrics' (STOP project), we developed web-based Patient Reported Outcome Measures (PROMs) for children and adolescents and for proxy reports by parents and clinicians in order to assess suicidality. Based on a literature review, expert panels and focus groups of patients, we developed the items of the STOP Suicidality Assessment Scale (STOP-SAS) in Spanish and English, translated it into four more languages, and optimized it for web-based presentation using the HealthTracker TM platform. Of the total 19 questions developed for the STOP-SAS, four questions that assess low-level suicidality were identified as screening questions (three of them for use with children, and all four for use with adolescents, parents and clinicians). A total of 395 adolescents, 110 children, 637 parents and 716 clinicians completed the questionnaire using the HealthTracker TM , allowing us to evaluate the internal consistency and convergent validity of the STOP-SAS with the clinician-rated Columbia Suicide Severity Rating Scale (C-SSRS). Validity was also assessed with the receiver operating characteristic (ROC) area of the STOP-SAS with the C-SSRS. The STOP-SAS comprises 19 items in its adolescent, parent, and clinician versions, and 14 items in its children's version. Good internal consistency was found for adolescents (Cronbach's alpha: 0.965), children (Cronbach's alpha: 0.922), parents (Cronbach's alpha: 0.951) and clinicians (Cronbach's alpha: 0.955) versions. A strong correlation was found between the STOP-SAS and the C-SSRS for adolescents (r:0.670), parents (r:0.548), clinicians (r:0.863) and children (r:0.654). The ROC area was good for clinicians' (0.917), adolescents' (0.834) and parents' (0.756) versions but only fair (0.683) for children's version. The STOP-SAS is a comprehensive, web

[Cushing syndrome: Physiopathology, etiology and principles of therapy].

PubMed

Chabre, Olivier

2014-04-01

The most frequent cause of Cushing's syndrome is iatrogenic, as Cushing's syndrome is the unavoidable consequence of long-term glucocorticoid treatment using more than 7.5 mg prednisone per day. The most frequent cause of endogenous Cushing's syndrome is Cushing's disease (CD), which is an ACTH dependent hypercortisolism linked to a pituitary corticotroph adenoma. This adenoma is often very small, its diagnosis may require bilateral inferior petrosal sinus sampling and the first line treatment of CD is transsphenoidal surgery by an expert neurosurgeon. The second line treatments include drugs that can act either on the pituitary adenoma or on adrenal steroidogenesis, pituitary radiotherapy or bilateral adrenalectomy. Ectopic ACTH dependent Cushing's syndrome is linked either to poorly differentiated endocrine tumors with a very poor prognosis, such as small cell lung cancer, or to well differentiated endocrine tumors, such as bronchial carcinoid tumors, which have a good prognosis when treated by surgery, but may be very difficult to localize. Adrenal Cushing's syndromes, which are independent of pituitary ACTH secretion, include adrenal cortex carcinoma, which requires abdominal surgery with extended adrenalectomy by an expert surgeon, adrenal adenoma which is treated by laparoscopic unilateral adrenalectomy and bilateral macronodular hyperplasia, whose surgical treatment may require unilateral or bilateral adrenalectomy. Treatment of Cushing's syndrome generally leads to spectacular clinical results, which must not hide the fact that the reversibility of some signs is actually incomplete. This underlines the need for a timely multidisciplinary management of the patients by an expert team. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

A fine structure genomic map of the region of 12q13 containing SAS and CDK4

DOE Office of Scientific and Technical Information (OSTI.GOV)

Linder, C.Y.; Elkahloun, A.G.; Su, Y.A.

1994-09-01

We have recently adapted a method, originally described by Rackwitz, to the rapid restriction mapping of multiple cosmid DNA samples. Linearization of the cosmids at the lambda cohesive site using lambda terminase is followed by partial digestion with selected restriction enzymes and hybridization to oligonucleotides specific for the right or left hand termini. Partial digestions are performed in a microtiter plate thus allowing up to 12 cosmid clones to be digested with one restriction enzyme. We have applied this rapid restriction mapping method to cosmids derived from a region of chromosome 12q13 that has recently been shown to be amplifiedmore » in a variety of cancers including malignant fibrous histiocytoma, fibrosarcoma, liposarcoma, osteosarcoma and brain tumors. A small segment of this amplification unit containing three genes, SAS (a membrane protein), CDK4 (a cyclin dependent kinase) and OS-9 (a recently described cDNA) has been analyzed with the system described above. This fine structure genomic map will be useful for completing the expression map of this region as well as characterizing its pattern of amplification in tumor specimens.« less

[A rare cause of oral pain: The pterygoid hamulus syndrome].

PubMed

Bandini, M; Corre, P; Huet, P; Khonsari, R H

2015-12-01

Pterygoid hamulus syndrome (PHS) is a rare cause of orofacial and oropharyngeal pain. PHS can be associated with a hamulus hypertrophy or with a bursitis of the palatosalpingeus but it has not always an anatomic cause. A 36-year-old woman was seen for a constant posterior palatal pain spreading towards oropharynx, increasing during swallowing and lasting for more than 6 months. Physical examination showed an erythema of the soft palate, medially to the hamulus. Hamulus palpation was painful and revealed hamulus hypertrophia on both sides. A bilateral PHS was evocated. This observation is typical of a PHS. We propose a review of the literature of this little-known syndrome. Treatment is initially conservative (corticosteroids) but surgery can be proposed in case of morphological anomalies of the hamulus. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

ENVIRONMENTAL TECHNOLOGY VERIFICATION REPORT, PERFORMANCE TEST RESULTS FOR THE A AND A ENVIRONMENTAL SEALS' SEAL ASSIST SYSTEM (SAS), PHASE I--TECHNOLOGY VERIFICATION REPORT

EPA Science Inventory

The report presents results of tests determining the efficacy of A&A Environmental Seals, Inc's Seal Assist System (SAS) in preventing natural gas compressor station's compressor rod packing leaks from escaping into the atmosphere. The SAS consists of an Emission Containment Glan...

[Traumatic asphyxia or Perthe's syndrome. About two paediatric cases].

PubMed

El koraichi, A; Benafitou, R; Tadili, J; Rafii, M; El Kharaz, H; Al Haddoury, M; El Kettani, S

2012-03-01

The traumatic asphyxia or Perthes' syndrome is a condition characterized by a classic triad involving head and neck cyanosis, subconjonctival bleeding, and petechiae. The pathophysiology of traumatic asphyxia is different in children compared to adults, because of the greater elasticity of the thorax in children. The purpose of this paper is to describe through two cases of traumatic asphyxia in young children the characteristics of this disease in children. Copyright © 2011 Société française d’anesthésie et de réanimation (Sfar). Published by Elsevier SAS. All rights reserved.

Comparison of the Results of Studies of Air Pollution Fungi Using the SAS Super 100, MAS 100, and Air IDEAL.

PubMed

Łukaszuk, Cecylia; Krajewska-Kułak, Elżbieta; Guzowski, Andrzej; Kułak, Wojciech; Kraszyńska, Bogumiła

2017-07-20

Although several air sampling devices for identifying and enumerating airborne microorganisms are commercially available, each poses some limitations. The aim of this study was to evaluate air pollution fungi using three such samplers: SAS Super 100, Microbiological Air Sampler (MAS) 100, and Air IDEAL. Mycological air was taken from the cellars of a 17th-century church in Siemiatycze, Poland, and the nearby outdoor environment. With samplers placed 1.5 m above the floor, microbial flora in air samples collected inside and outside the cellar were detected. The number of colony-forming units (CFU) of fungi obtained with the three samplers from the cellars and outdoor environment differed; the most CFU were obtained with the Air IDEAL and the least with the SAS Super 100. Significant differences emerged in CFUs collected from air samples with the MAS 100 and SAS Super 100, on the one hand, and the SAS Super 100 and Air IDEAL, on the other. Otherwise, results among the samplers were different. More Cladosporium species were collected with the MAS 100 sampler, whereas more Fusarium and Aspergillus species were collected with the Air IDEAL sampler. Significant differences among CFU/m³ values among the tested sites depended on the sampler used.

Classroom Performance and Adaptive Skills in Children with Epilepsy.

ERIC Educational Resources Information Center

Huberty, Thomas J.; And Others

1992-01-01

Studied relationships of age at onset, seizure syndrome, seizure type, and seizure frequency to classroom performance and adaptive skills of 131 children with epilepsy. Epilepsy syndrome and frequency of seizures significantly related to some analyses. Results suggest that seizure disorder associated with diffuse or multifocal brain insult can…

Variants at multiple loci implicated in both innate and adaptive immune responses are associated with Sjögren’s syndrome

PubMed Central

Lessard, Christopher J.; Li, He; Adrianto, Indra; Ice, John A.; Rasmussen, Astrid; Grundahl, Kiely M.; Kelly, Jennifer A.; Dozmorov, Mikhail G.; Miceli-Richard, Corinne; Bowman, Simon; Lester, Sue; Eriksson, Per; Eloranta, Maija-Leena; Brun, Johan G.; Gøransson, Lasse G.; Harboe, Erna; Guthridge, Joel M.; Kaufman, Kenneth M.; Kvarnström, Marika; Jazebi, Helmi; Graham, Deborah S. Cunninghame; Grandits, Martha E.; Nazmul-Hossain, Abu N. M.; Patel, Ketan; Adler, Adam J.; Maier-Moore, Jacen S.; Farris, A. Darise; Brennan, Michael T.; Lessard, James A.; Chodosh, James; Gopalakrishnan, Rajaram; Hefner, Kimberly S.; Houston, Glen D.; Huang, Andrew J.W.; Hughes, Pamela J.; Lewis, David M.; Radfar, Lida; Rohrer, Michael D.; Stone, Donald U.; Wren, Jonathan D.; Vyse, Timothy J.; Gaffney, Patrick M.; James, Judith A.; Omdal, Roald; Wahren-Herlenius, Marie; Illei, Gabor G.; Witte, Torsten; Jonsson, Roland; Rischmueller, Maureen; Rönnblom, Lars; Nordmark, Gunnel; Ng, Wan-Fai; Mariette, Xavier; Anaya, Juan-Manuel; Rhodus, Nelson L.; Segal, Barbara M.; Scofield, R. Hal; Montgomery, Courtney G.; Harley, John B.; Sivils, Kathy L. Moser

2013-01-01

Sjögren’s syndrome is a common autoimmune disease (~0.7% of European Americans) typically presenting as keratoconjunctivitis sicca and xerostomia. In addition to strong association within the HLA region at 6p21 (Pmeta=7.65×10−114), we establish associations with IRF5-TNPO3 (Pmeta=2.73×10−19), STAT4 (Pmeta=6.80×10−15), IL12A (Pmeta =1.17×10−10), FAM167A-BLK (Pmeta=4.97×10−10), DDX6-CXCR5 (Pmeta=1.10×10−8), and TNIP1 (Pmeta=3.30×10−8). Suggestive associations with Pmeta<5×10−5 were observed with 29 regions including TNFAIP3, PTTG1, PRDM1, DGKQ, FCGR2A, IRAK1BP1, ITSN2, and PHIP amongst others. These results highlight the importance of genes involved in both innate and adaptive immunity in Sjögren’s syndrome. PMID:24097067

[Cryopyrine-associated periodic syndrome: CAPS seen from adulthood].

PubMed

Koné-Paut, I

2015-04-01

Cryopyrin-associated periodic syndrome is a rare hereditary periodic fever syndrome for which, the genetic mechanism, mutation in the NLRP3 gene, has allowed to gather 3 clinical phenotypes (familial cold urticaria [FCAS], Muckle-Wells syndrome [MWS], and chronic infantile neurological cutaneous and articular syndrome [CINCA]) initially described independently, and to discover the NLRP3 inflammasome, a key receptor of the innate immunity, which regulates the interleukine-1β secretion into the mononuclear cells. The clinical manifestation of CAPS : urticaria-like skin rash, eyes redness, myalgia and sensory deafness are not specific, if considered separately, and that often leads to a wandering diagnosis through a complex medical journey including various specialists. The diagnostic delay is deleterious to patients compromising their quality of life and exposing them to neurosensory complications and renal failure by secondary amyloidosis. The paediatric onset of disease, the family history, the trigger of symptoms by the cold, and the recognition of the skin rash as neutrophilic are important clues before diagnostic confirmation by genetic testing. Interleukine-1 blockade is the only effective treatment of CAPS symptoms which often may stabilize (rarely regression) the sensory involvement and in some cases may allow the regression of secondary amyloidosis. Copyright © 2014 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Sinusoidal obstruction syndrome.

PubMed

Valla, Dominique-Charles; Cazals-Hatem, Dominique

2016-09-01

Sinusoidal obstruction syndrome (SOS) is characterized by damage to small hepatic vessels affecting particularly sinusoidal endothelium. Damaged sinusoids can be associated with a partial or complete occlusion of small hepatic veins, hence the previous denomination of hepatic veno-occlusive disease (VOD). Exposure to certain exogenous toxins appears to be specific to this condition and is frequently included in its definition. Typical histopathological features of SOS in a liver biopsy specimen are presented in the text. The purpose of this article is to provide an overview on the different entities corresponding to this general definition. Such entities include: (i) liver disease related to pyrrolizidine alcaloids; (ii) liver injury related to conditioning for hematopoietic stem cell transplantation; (iii) vascular liver disease occurring in patients treated with chemotherapy for liver metastasis of colorectal cancer; and (iv) other liver diseases related to toxic agents. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

FY2017 Updates to the SAS4A/SASSYS-1 Safety Analysis Code

DOE Office of Scientific and Technical Information (OSTI.GOV)

Fanning, T. H.

The SAS4A/SASSYS-1 safety analysis software is used to perform deterministic analysis of anticipated events as well as design-basis and beyond-design-basis accidents for advanced fast reactors. It plays a central role in the analysis of U.S. DOE conceptual designs, proposed test and demonstration reactors, and in domestic and international collaborations. This report summarizes the code development activities that have taken place during FY2017. Extensions to the void and cladding reactivity feedback models have been implemented, and Control System capabilities have been improved through a new virtual data acquisition system for plant state variables and an additional Block Signal for a variablemore » lag compensator to represent reactivity feedback for novel shutdown devices. Current code development and maintenance needs are also summarized in three key areas: software quality assurance, modeling improvements, and maintenance of related tools. With ongoing support, SAS4A/SASSYS-1 can continue to fulfill its growing role in fast reactor safety analysis and help solidify DOE’s leadership role in fast reactor safety both domestically and in international collaborations.« less

A SAS(®) macro implementation of a multiple comparison post hoc test for a Kruskal-Wallis analysis.

PubMed

Elliott, Alan C; Hynan, Linda S

2011-04-01

The Kruskal-Wallis (KW) nonparametric analysis of variance is often used instead of a standard one-way ANOVA when data are from a suspected non-normal population. The KW omnibus procedure tests for some differences between groups, but provides no specific post hoc pair wise comparisons. This paper provides a SAS(®) macro implementation of a multiple comparison test based on significant Kruskal-Wallis results from the SAS NPAR1WAY procedure. The implementation is designed for up to 20 groups at a user-specified alpha significance level. A Monte-Carlo simulation compared this nonparametric procedure to commonly used parametric multiple comparison tests. Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.

Attentional Set-Shifting in Fragile X Syndrome

ERIC Educational Resources Information Center

Van der Molen, M. J. W.; Van der Molen, M. W.; Ridderinkhof, K. R.; Hamel, B. C. J.; Curfs, L. M. G.; Ramakers, G. J. A.

2012-01-01

The ability to flexibly adapt to the changing demands of the environment is often reported as a core deficit in fragile X syndrome (FXS). However, the cognitive processes that determine this attentional set-shifting deficit remain elusive. The present study investigated attentional set-shifting ability in fragile X syndrome males with the…

"Serpentine-like syndrome"-A very rare multiple malformation syndrome characterised by brachioesophagus and vertebral anomalies.

PubMed

Beleza-Meireles, Ana; Steenhaut, Patricia; Hocq, Catheline; Clapuyt, Philippe; Bernard, Pierre; Debauche, Christian; Sznajer, Yves

2017-02-01

"Serpentine-like syndrome" is a severe and rare association of multiple congenital malformations, characterised by brachioesophagus, secondary intrathoracic stomach, and vertebral anomalies. Other associated anomalies have been described, such as malposition and herniation of abdominal organs. We report the natural history of a baby girl born at 29 weeks of gestation with intra uterine growth restriction, short neck, large rachischisis from cervical to thoracic spine, a very short oesophagus, thoracic stomach associated with a midline diaphragmatic hernia, malrotated gut and median cleft lip. Most of these anomalies were detected antenatally. Molecular karyotype was normal. She died at age 12 days. To our knowledge, the present patient represents the 8th report of a case of "Serpentine-like syndrome". Brachioesophagus and congenital vertebral anomalies, in particular rachischisis, are the cardinal features of this condition. All reported cases have been sporadic and the cause is still unknown. We believe that the specificity of the presentation as well as the similarities between available descriptions of patients suggests a common, yet to identify, molecular cause, possibly involving a developmental "toolkit"/homeobox gene or related pathways. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Drug recrystallization using supercritical anti-solvent (SAS) process with impinging jets: Effect of process parameters

NASA Astrophysics Data System (ADS)

Careno, Stéphanie; Boutin, Olivier; Badens, Elisabeth

2012-03-01

The aim of this study is to improve mixing in supercritical anti-solvent process (SAS) with impinging jets in order to form finer particles of sulfathiazole, a poorly water-soluble drug. The influence of several process parameters upon the powder characteristics is studied. Parameters are jets' velocity (0.25 m s-1 to 25.92 m s-1), molar ratio solvent/CO2 (2.5% to 20%), temperature (313 K to 343 K), pressure (10 MPa to 20 MPa) and sulfathiazole concentration in the organic solution (0.5% to 1.8%). Two solvents are used: acetone and methanol. Smaller particles with a more homogeneous morphology are obtained from acetone solutions. For the smallest jets' velocity, corresponding to a non-atomized jet, the stable polymorphic form is obtained, pure or in mixture. At this velocity, pressure is the most influential parameter controlling the polymorphic nature of the powder formed. The pure stable polymorph is formed at 20 MPa. Concerning the particle size, the most influential parameters are temperature and sulfathiazole concentration. The use of impinging jets with different process parameters allows the crystallization of four polymorphs among the five known, and particle sizes are varied. This work demonstrates the studied device ability of the polymorph and the size control. A comparison with the classical SAS process shows that particle size, size distribution and morphology of particles crystallized with impinging jets are different from the ones obtained with classical SAS introduction device in similar operating conditions. Mean particle sizes are significantly smaller and size distributions are narrower with impinging jets device.

Autoimmune liver disease in Noonan Syndrome.

PubMed

Loddo, Italia; Romano, Claudio; Cutrupi, Maria Concetta; Sciveres, Marco; Riva, Silvia; Salpietro, Annamaria; Ferraù, Valeria; Gallizzi, Romina; Briuglia, Silvana

2015-03-01

Noonan Syndrome (NS) is characterized 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 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. Autoimmune Hepatitis (AIH) is a cryptogenic, chronic and progressive necroinflammatory liver disease. Common features of AIH are hypergammaglobulinemia (IgG), presence of circulating autoantibodies, histological picture of interface hepatitis and response to immunosuppressant drugs. Conventional treatment with Prednisone and Azathioprine is effective in most patients. We describe the case of a 6 years-old girl with Noonan Syndrome and Autoimmune Hepatitis type 1. Molecular analysis of PTPN11 gene showed heterozygous mutation c.923A>G (Asn308Ser) in exon 8. Though association between NS and autoimmune disorders is known, this is the second case of association between Noonan Syndrome and Autoimmune Hepatitis type 1 described in literature. In the management of NS, an accurate clinical evaluation would be recommended. When there is a clinical suspicion of autoimmune phenomena, appropriate laboratory tests should be performed with the aim of clarifying whether the immune system is involved in NS. We think that autoimmunity represents a characteristic of NS, even if the etiopathogenesis is still unknown. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

[Fragile X syndrome and white matter abnormalities: Case study of two brothers].

PubMed

Wallach, E; Bieth, E; Sevely, A; Cances, C

2017-03-01

Fragile X syndrome is the most usual cause of hereditary intellectual deficiency. Typical symptoms combine intellectual deficiency, social anxiety, intense emotional vigilance, and a characteristic facial dysmorphy. This is subsequent to a complete mutation of the FMR1 gene, considering a semidominant transmission linked to the unstable X. The expansion of the CGG triplet greater than 200 units combined with a high methylation pattern lead to a transcriptional silence of the FMR1 gene, and the protein product, the FMRP, is not synthesized. This protein is involved in synaptic plasticity. Brain MRI can show an increased volume of the caudate nucleus and hippocampus, combined with hypoplasia of the cerebellar vermis. Fragile X Associated Tremor Ataxia Syndrome (FXTAS) syndrome is a neurodegenerative disorder occurring in carriers of the premutation in FMR1. Brain MRI shows an increased T2 signal in the middle cerebellar peduncles. This syndrome is linked to a premutation in the FMR1 gene. We report here the case of two brothers presenting a typical fragile X symptomatology. Brain MRI showed hyperintensities of the middle cerebellar peduncles. Such MRI findings support the assumption of a genetic mosaicism. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Preparation and Physicochemical Properties of 10-Hydroxycamptothecin (HCPT) Nanoparticles by Supercritical Antisolvent (SAS) Process

PubMed Central

Zhao, Xiuhua; Zu, Yuangang; Jiang, Ru; Wang, Ying; Li, Yong; Li, Qingyong; Zhao, Dongmei; Zu, Baishi; Zhang, Baoyou; Sun, Zhiqiang; Zhang, Xiaonan

2011-01-01

The goal of the present work was to study the feasibility of 10-hydroxycamptothecin (HCPT) nanoparticle preparation using supercritical antisolvent (SAS) precipitation. The influences of various experimental factors on the mean particle size (MPS) of HCPT nanoparticles were investigated. The optimum micronization conditions are determined as follows: HCPT solution concentration 0.5 mg/mL, the flow rate ratio of CO2 and HCPT solution 19.55, precipitation temperature 35 °C and precipitation pressure 20 MPa. Under the optimum conditions, HCPT nanoparticles with a MPS of 180 ± 20.3 nm were obtained. Moreover, the HCPT nanoparticles obtained were characterized by Scanning electron microscopy, Dynamic light scattering, Fourier-transform infrared spectroscopy, High performance liquid chromatography-mass spectrometry, X-ray diffraction and Differential scanning calorimetry analyses. The physicochemical characterization results showed that the SAS process had not induced degradation of HCPT. Finally, the dissolution rates of HCPT nanoparticles were investigated and the results proved that there is a significant increase in dissolution rate compared to unprocessed HCPT. PMID:21731466

[Ehler-Danlos syndrome type VIII].

PubMed

Ciarloni, L; Perrigouard, C; Lipsker, D; Cribier, B

2010-03-01

Ehlers-Danlos syndrome (EDS) comprises a heterogeneous group of diseases involving genetic collagen fibre impairment. We describe a case of a patient presenting the rare type VIII, in which dermatitis ocre was associated with parodontal disease, and which was diagnosed late. A 29-year-old man consulted for a pretibial ulcer present for seven years, resulting from a post-traumatic haematoma that had failed to heal. In view of the longiliner morphology, it had previously been diagnosed as Marfan syndrome. Subsequently, edentation was observed as well as "alveolar bone fragility". Examination revealed a marfanoid morphotype, a pretibial ulcer set within long-standing bilateral dermatitis ocre and papyraceous scars, but no joint hyperlaxity or cutaneous hyperelasticity. The diagnosis was consequently corrected to EDS type VIII. Type VIII is a rare form of EDS, and the molecular mechanism is poorly understood. The involvement of parodontal connective tissue suggests impairment of collagen I and III proteins. It is important to identify this type of the disease since it involves parodontal disease for which early treatment is required in order to try to prevent edentation. The present case demonstrates the importance of diagnosis, which may be based upon appearance of bilateral dermatitis ocre from the age of 15 years associated with skin fragility. This sign is not part of the classical picture of Marfan syndrome, with which EDS type VIII is often confounded. Copyright 2009 Elsevier Masson SAS. All rights reserved.

Emotional Responsivity in Young Children with Williams Syndrome

ERIC Educational Resources Information Center

Fidler, Debbie J.; Hepburn, Susan L.; Most, David E.; Philofsky, Amy; Rogers, Sally J.

2007-01-01

The hypothesis that young children with Williams syndrome show higher rates of emotional responsivity relative to other children with developmental disabilities was explored. Performance of 23 young children with Williams syndrome and 30 MA-matched children with developmental disabilities of nonspecific etiologies was compared on an adaptation of…

[Adult-onset opsoclonus-myoclonus-ataxia syndrome revealing rubella meningoencephalitis].

PubMed

Nasri, A; Mansour, M; Messelmani, M; Riahi, A; Derbali, H; Bedoui, I; Zaouali, J; Mrissa, R

2016-12-01

Opsoclonus-myoclonus-ataxia (OMS) is a rare clinical syndrome, of paraneoplastic infectious, post-infectious, post-vaccinal or idiopathic origin. We report a 24-year-old young man who presented with gait disorder preceded by a febrile rash and retroauricular lymph nodes. Three days before admission, he had headache, vertigo, nausea and vomiting followed by gait unsteadiness and movement disorders of limbs and eyes. On examination, he had OMS syndrome. Brain MRI, total body scan, MIBG scintigraphy, tumor markers and onconeural antibodies were normal. Cerebro-spinal fluid (CSF) analysis showed lymphocytic meningitis. Positive serum and CSF immunoglobulin M antibody against rubella virus was demonstrated. He received acyclovir with full recovery within two weeks. We discuss the peculiarities of this association with a literature review. This observation enlarges the spectrum of neurological manifestations of rubella as well as that of OMS etiologies. Copyright © 2016 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Biodiversity, evolution and adaptation of cultivated crops.

PubMed

Vigouroux, Yves; Barnaud, Adeline; Scarcelli, Nora; Thuillet, Anne-Céline

2011-05-01

The human diet depends on very few crops. Current diversity in these crops is the result of a long interaction between farmers and cultivated plants, and their environment. Man largely shaped crop biodiversity from the domestication period 12,000 B.P. to the development of improved varieties during the last century. We illustrate this process through a detailed analysis of the domestication and early diffusion of maize. In smallholder agricultural systems, farmers still have a major impact on crop diversity today. We review several examples of the major impact of man on current diversity. Finally, biodiversity is considered to be an asset for adaptation to current environmental changes. We describe the evolution of pearl millet in West Africa, where average rainfall has decreased over the last forty years. Diversity in cultivated varieties has certainly helped this crop to adapt to climate variation. Copyright © 2011 Académie des sciences. Published by Elsevier SAS. All rights reserved.

Fragile X Syndrome

ERIC Educational Resources Information Center

Schwarte, Andrea R.

2008-01-01

This article provides an overview of current research on Fragile X Syndrome, and how that knowledge can be used to guide successful intervention. The genetic etiology of Fragile X is reviewed and the physical, cognitive, adaptive, behavioral, and emotional phenotypes of children with the disorder are described, highlighting the differences in…

Children with Williams syndrome: Developmental trajectories for intellectual abilities, vocabulary abilities, and adaptive behavior.

PubMed

Mervis, Carolyn B; Pitts, C Holley

2015-06-01

To examine longitudinal trajectories of intellectual abilities, single-word vocabulary abilities, and adaptive behavior for 76 children with Williams syndrome (WS) aged 4-15 years, we compared their standard scores (SSs) at two time points approximately 3 years apart on the same standardized measures. At the group level, mean SS declined significantly for 8 of the 12 measures and showed a slight (nonsignificant) increase or decrease for 4 measures. However, for most measures significant changes in SS were found for only a small proportion of the children, with some children evidencing significant declines and a smaller proportion evidencing significant increases. Significant SS changes were most common for adaptive behavior. For all measures, the mean magnitude of SS change was smaller for older children (>7.5 years at Time 1) than for younger children (<7.5 years at Time 1). Furthermore, correlations between Time 1 and Time 2 SSs were larger for the older cohort than for the younger cohort, indicating that SS stability was greater for older children than for younger children. Although mean SSs declined for most measures, indicating that children with WS as a group were not making the expected amount of progress relative to their general population peers who earned the same SS at Time 1, there was little evidence either of regression (loss of skills) or stagnation (failure to increase raw scores). The relations of these results to those of previous smaller-sample longitudinal studies of children with WS and the implications of the findings are considered. © 2015 Wiley Periodicals, Inc.

RNA editing and regulation of Drosophila 4f-rnp expression by sas-10 antisense readthrough mRNA transcripts

PubMed Central

PETERS, NICK T.; ROHRBACH, JUSTIN A.; ZALEWSKI, BRIAN A.; BYRKETT, COLLEEN M.; VAUGHN, JACK C.

2003-01-01

We have previously described an example of extensively A-to-G edited cDNA derived from adult heads of the fruitfly Drosophila melanogaster. In that study, the source of the predicted antisense RNA pairing strand for template recognition by dADAR editase was not identified, and the biological significance of the observed hyperediting was not known. Here, we address each of these questions. 4f-rnp and sas-10 are closely adjacent X-linked genes located on opposite DNA strands that produce convergent transcripts. We show that developmentally regulated antisense sas-10 readthrough mRNA arises by activation of an upstream promoter P2 during the late embryo stage of fly development. The sas-10 readthrough transcripts pair with 4f-rnp mRNA to form double-stranded molecules, as indicated by A-to-G editing observed in both RNA strands. It would be predicted that perfect RNA duplexes would be targeted for modification/degradation by enzyme pathways that recognize double-stranded RNAs, leading to decline in 4f-rnp mRNA levels, and this is what we observe. The observation using quantitative RT-PCR that sas-10 readthrough and 4f-rnp transcript levels are inversely related suggests a role for the antisense RNA in posttranscriptional regulation of 4f-rnp gene expression during development. Potential molecular mechanisms that could lead to this result are discussed, one of which is targeted transcript degradation via the RNAi pathway. Insofar as the dADAR editase and RNAi pathways are known to be constitutive in this system, it is likely that control of antisense RNA transcription is the rate-limiting factor. The results provide insight into roles of naturally occurring antisense RNAs in regulation of eukaryotic gene expression. PMID:12756328

[Prevalence and characteristics of acute coronary syndromes in a sub-Saharan Africa population].

PubMed

N'Guetta, R; Yao, H; Ekou, A; N'Cho-Mottoh, M P; Angoran, I; Tano, M; Konin, C; Coulibaly, I; Anzouan-Kacou, J B; Seka, R; Adoh, A M

2016-04-01

To assess prevalence, characteristics and management of acute coronary syndromes in sub-Saharan Africa population. Prospective survey from January, 2010 to December, 2013, carried out among patients aged 18 years old, admitted to intensive care unit of Abidjan Heart Institute for acute coronary syndrome (ACS). Four hundred and twenty-five (425) patients were enrolled in this study. Prevalence of ACS was 13.5%. Mean age was 55.4±11 years. Clinical presentation was predominantly ST-segment elevation myocardial infarction (STEMI) in 71.5% of subjects, non-ST-segment elevation acute coronary syndrome (NSTE-ACS) accounted for 28.5%. Two hundred and eighty patients (65.9%) were transferred by unsafe transportation. Among the 89 patients admitted within 12hours of the onset of symptoms, primary percutaneous coronary intervention was performed in 20 patients (22.5%), or 6.6% of STEMI as a whole. Twenty-five patients (8.2%) received fibrinolytic therapy with alteplase. In-hospital death rate was 10%. The prevalence of acute coronary syndromes is increasing in sub-Saharan Africa. Excessive delays of admission and limited technical facilities are the major difficulties of their management in our regions. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

User’s Guide for the SAS (Stand-Off Attack Simulation) Computer Model.

DTIC Science & Technology

1982-01-15

A99QAXFD000-01 Albuquerque, New Mexico 87110 I1. CONTROLLING OFFICE NAME AND ADDRESS 12. REPORT DATE Director 15 January 1982 Defense Nuclear Aqency 13...computer model. SAS is an effective survivability and security system design tool which allows an analyst to compare the relative effectiveness of selected...mounted against other systems during uploading for dispersal or for non -emergency relocation. GLCM and LANCE must be mobilized and formed into convoys

IMAGING WITH MULTIMODAL ADAPTIVE-OPTICS OPTICAL COHERENCE TOMOGRAPHY IN MULTIPLE EVANESCENT WHITE DOT SYNDROME: THE STRUCTURE AND FUNCTIONAL RELATIONSHIP.

PubMed

Labriola, Leanne T; Legarreta, Andrew D; Legarreta, John E; Nadler, Zach; Gallagher, Denise; Hammer, Daniel X; Ferguson, R Daniel; Iftimia, Nicusor; Wollstein, Gadi; Schuman, Joel S

2016-01-01

To elucidate the location of pathological changes in multiple evanescent white dot syndrome (MEWDS) with the use of multimodal adaptive optics (AO) imaging. A 5-year observational case study of a 24-year-old female with recurrent MEWDS. Full examination included history, Snellen chart visual acuity, pupil assessment, intraocular pressures, slit lamp evaluation, dilated fundoscopic exam, imaging with Fourier-domain optical coherence tomography (FD-OCT), blue-light fundus autofluorescence (FAF), fundus photography, fluorescein angiography, and adaptive-optics optical coherence tomography. Three distinct acute episodes of MEWDS occurred during the period of follow-up. Fourier-domain optical coherence tomography and adaptive-optics imaging showed disturbance in the photoreceptor outer segments (PR OS) in the posterior pole with each flare. The degree of disturbance at the photoreceptor level corresponded to size and extent of the visual field changes. All findings were transient with delineation of the photoreceptor recovery from the outer edges of the lesion inward. Hyperautofluorescence was seen during acute flares. Increase in choroidal thickness did occur with each active flare but resolved. Although changes in the choroid and RPE can be observed in MEWDS, Fourier-domain optical coherence tomography, and multimodal adaptive optics imaging localized the visually significant changes seen in this disease at the level of the photoreceptors. These transient retinal changes specifically occur at the level of the inner segment ellipsoid and OS/RPE line. En face optical coherence tomography imaging provides a detailed, yet noninvasive method for following the convalescence of MEWDS and provides insight into the structural and functional relationship of this transient inflammatory retinal disease.

Relationship between hepatocellular carcinoma, metabolic syndrome and non-alcoholic fatty liver disease: which clinical arguments?

PubMed

Rosmorduc, Olivier

2013-05-01

Obesity and the metabolic syndrome are growing epidemics associated with an increased risk for many types of cancer. In the liver, inflammatory and angiogenic changes due to insulin resistance and fatty liver disease are associated with an increased incidence of liver cancer. Regardless of underlying liver disease, cirrhosis remains the most important risk factor for hepatocellular carcinoma (HCC) although are cases of HCC arising without cirrhosis raise the possibility of a direct carcinogenesis secondary to Non-alcoholic Fatty Liver Disease (NAFLD). Moreover, metabolic syndrome and its different features may also increase the risk of HCC in the setting of chronic liver diseases of other causes such as viral hepatitis or alcohol abuse. Taking into account all these data, it is necessary to better determine the risk of developing HCC in patients with metabolic syndrome to improve the screening guidelines and develop prophylactic treatments in this setting. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Relationship dimensions of the ‘Down syndrome advantage’

PubMed Central

Mitchell, D. B.; Hauser-Cram, P.; Crossman, M. K.

2014-01-01

Background Some researchers have proposed an ‘advantage’ for parents of children with Down syndrome over parents of children with other intellectual disabilities, especially in relation to experiencing less parenting stress. Others have maintained that these differences are an artefact of demographic and related differences. This study extends the investigation of possible differences in dimensions of parenting stress and also examines whether differences exist in maternal and child contingent responsiveness during mother–child interaction in these two groups. Method Mothers of children with Down syndrome (n = 43) and undifferentiated developmental disabilities (n = 54) completed measures of children's adaptive functioning and behaviour problems, parenting stress and maternal social support. Observers rated the contingent interactions between mothers and children using the Nursing Child Assessment Teaching Scale. Results Once mother's age, education and social support as well as child adaptive functioning and behaviour problems were considered, neither parent nor child related parenting stress demonstrated an advantage for parents of children with Down syndrome. However, a ‘Down syndrome advantage’ was apparent for both maternal and child contingent responsiveness after accounting for maternal demographic and contextual variables and child attributes. Conclusions Children with Down syndrome and their mothers have more positive interactions than children with other developmental disabilities, both in terms of the responsiveness of mothers and of child responses contingent on maternal behaviour. These findings suggest that both children with Down syndrome themselves and their mothers are contributing to a Down syndrome advantage. PMID:25070618

Coping Strategies and Adaptation of Mothers of Children with Handicapping Conditions.

ERIC Educational Resources Information Center

Hooshyar, Nahid T.

Mothers' coping mechanisms and adaptations to having a handicapped child were analyzed through extensive structured interviews with mothers of eight preschool-aged Down syndrome children and a language impaired child. Three illustrative case studies are presented, and general conclusions are drawn. Mothers of Down syndrome children go through the…

Lack of repercussions of sleep apnea syndrome on recovery and attention disorders at the subacute stage after stroke: a study of 45 patients.

PubMed

Lefèvre-Dognin, C; Stana, L; Jousse, M; Lucas, C; Sportouch, P; Bradai, N; Guettard, E; Vicaut, E; Yelnik, A P

2014-12-01

Sleep apnea syndrome (SAS) frequently occurs after a stroke. Its association with a poor prognosis is open to discussion. To study, in a physical and rehabilitation medicine (PRM) unit, the possible repercussions of SAS on neurological and functional recovery as well as attentional abilities following a stroke. Forty-five patients, all of whom had recently had a stroke without previously documented SAS, were screened using the ApneaLink(®) system. An apnea-hypopnea index (AHI) score ≥10 was considered as indicative of SAS. The NIHSS, Fugl-Meyer (FM) and Functional Independence Measure (FIM) Scales were applied on admission and at two months as means of assessing neurological and functional recovery, which was expressed by the difference between the first and the second scores (delta FM, delta NIHSS, delta FIM). The Battery Attention William Lennox (BAWL) Test was given once in order to evaluate attention disorders. SAS severity was categorized according to the AHI. We compared the groups formed (mild, moderate and severe) using the same method. Twenty-eight patients (62.2%) presented AHI ≥ 10. Stroke characteristics were comparable in the SAS+ and the SAS- groups, with average post-stroke time lapse of 26 days, initial average FIM score of 71.2 points ± 26.3 and initial average NIHSS score of 8.9 ± 4.9. The demographic characteristics of the two groups were likewise comparable with the exception of age, as the SAS+ group was pronouncedly older (65.4 vs. 53.5 years). As for delta FIM, which evaluated functional recovery, it averaged 31.8 ± 20.6. Cases of SAS were found to be mild (37.1%), moderate (28.6%) or severe (34.3%). No significant difference was observed on admission or at 2 months as regards the clinical scales or the BAWL test between the two groups or according to severity, except for the NIHSS score at 2 months in the severe sub-group. This study did not demonstrate the supposed repercussions of SAS on the recovery or attentional

[Effects of glucagon-like peptide 2 on the adaptation of residual small bowel in a rat model of short bowel syndrome].

PubMed

Wu, Guo-Hao; Chen, Ji; Li, Hang; Wu, Zhao-Han

2006-09-01

To investigate the effects of glucagon-like peptide 2 (GLP-2) on the morphology and functional adaptation of the residual small bowel in rat model of short bowel syndrome. Twenty rats with 75% of the midjejunoileum removed were randomly divided into two groups, and received intra-peritoneal injection of GLP-2(250 micro*gd*kg-1*d-1) or subcutaneous injection saline(0.5 ml, twice one day) after operation. On postoperative day 6, the morphological changes of the residual jejunum and ileum, the expression of proliferating cell nuclear antigen(PCNA), and the mRNA expressions of Na-D-glucose cotransporters (SGLT1) and peptide cotransporters (PEPT1) were determined. The intestinal glucose absorption data per unit length as well as per unit weight of ileum were measured by in vivo circulatory perfusion experiment. The morphological parameters of the residual gut such as the thickness of mucosa, height of villus, depth of crypt, and PCNA positive index were significantly higher, while the apoptosis rate per unit of mucosal square was significantly lower in GLP-2 treatment group than those in the control group. The expressions of mRNA SGTLl and PEPT1 in the residual ileum were significantly higher than those in the control group. There was no significant difference in glucose absorption rate per gram of mucosal wet weight between the two groups (P > 0.05). GLP-2 could improve morphological and functional adaptation of the residual small bowel by stimulating enterocyte proliferation and decreasing enterocyte apoptosis in short bowel syndrome.

Using SAS PROC CALIS to fit Level-1 error covariance structures of latent growth models.

PubMed

Ding, Cherng G; Jane, Ten-Der

2012-09-01

In the present article, we demonstrates the use of SAS PROC CALIS to fit various types of Level-1 error covariance structures of latent growth models (LGM). Advantages of the SEM approach, on which PROC CALIS is based, include the capabilities of modeling the change over time for latent constructs, measured by multiple indicators; embedding LGM into a larger latent variable model; incorporating measurement models for latent predictors; and better assessing model fit and the flexibility in specifying error covariance structures. The strength of PROC CALIS is always accompanied with technical coding work, which needs to be specifically addressed. We provide a tutorial on the SAS syntax for modeling the growth of a manifest variable and the growth of a latent construct, focusing the documentation on the specification of Level-1 error covariance structures. Illustrations are conducted with the data generated from two given latent growth models. The coding provided is helpful when the growth model has been well determined and the Level-1 error covariance structure is to be identified.

Genetics Home Reference: Smith-Magenis syndrome

MedlinePlus

... segment most often includes approximately 3.7 million DNA building blocks (base pairs), also written as 3. ... AM, Lupski JR, Potocki L. Cognitive and adaptive behavior profiles in Smith-Magenis syndrome. J Dev Behav ...

From Uhuru at CfA to SAS-3 at MIT: Looking for X-Ray Binaries in all the Right Places

NASA Astrophysics Data System (ADS)

Cominsky, Lynn R.

2013-01-01

My career in X-ray astronomy started almost accidentally, when in 1975 I was hired fresh out of college as a “data aide” for the Uhuru satellite, in Riccardo Giacconi’s group at the Harvard-Smithsonian Center for Astrophysics. Working first for Mel Ulmer, and later for Bill Forman and Christine Jones, I learned the fundamentals of data analysis, and helped produce the Fourth Uhuru catalog of X-ray sources (Forman et al. 1978), as well as studying transient X-ray sources (Cominsky et al. 1978). Christine was the first woman scientist I had ever met, and with her encouragement, I applied to graduate school to continue on in X-ray astronomy. Lured down the street to MIT by the chance to work on SAS-3, I eagerly learned how to operate the satellite from a control room in the Center for Space Research. The SAS-3 group was led by Prof. George Clark, and it was my good luck that he was around during the holiday break in January 1978 when everyone else in the group was at an AAS meeting in Hawaii. A source I recognized from my Uhuru work, 4U0115+63, had reappeared, and I knew that it was likely to be a pulsar. With the help of George and Project Scientist Bill Mayer, I managed to send the commands to stop SAS-3 and point at the source. The 3.6 second pulsations were so strong that they could be seen in the raw data! This discovery made the New York Times, as 4U0115+63 was the first transient x-ray source shown to be in a binary system (Cominsky et al. 1978, Rappaport et al. 1978). Another personal SAS-3 highlight included working on Prof. Walter Lewin’s “World-wide Burst Watch” - coordinated multi-wavelength observations of x-ray burst sources which led to the discoveries of slightly delayed but coincident optical bursts (Grindlay et al. 1978, McClintock et al. 1979). Although live operations of SAS-3 ended when it re-entered on April 9, 1979, many years of additional data analysis remained. And later, as a continuation of work I began in my Ph.D. Thesis on X

[Fortuitous discovery of intracerebral foreign body leads to the diagnosis of Munchausen syndrome by proxy].

PubMed

Bouaziz Abed, A; Mustapha, R; Chiha, M

2011-11-01

An intracerebral foreign body is a rare situation in childhood. It often occurs accidentally with transorbital or more rarely transnasal penetration. We report the case of a child who had been followed since the age of 2.5 months for chronic vomiting without failure to thrive. The fortuitous discovery at 22 months of age of a 4.5-cm-long sewing needle in his brain probably introduced via the fontanel led us to the diagnosis of Munchausen syndrome by proxy. Copyright © 2011. Published by Elsevier SAS.

The Southeast Atmosphere Studies (SAS): coordinated investigation and discovery to answer critical questions about fundamental atmospheric processes

EPA Science Inventory

The Southeast Atmosphere Studies (SAS), encompassing the Southern Oxidant and Aerosol Study (SOAS), the Southeast Nexus of Air Quality and Climate (SENEX) study, the Nitrogen, Oxidants, Mercury and Aerosols: Distributions, Sources and Sinks (NOMADSS) study deployed in the field f...

Simple and flexible SAS and SPSS programs for analyzing lag-sequential categorical data.

PubMed

O'Connor, B P

1999-11-01

This paper describes simple and flexible programs for analyzing lag-sequential categorical data, using SAS and SPSS. The programs read a stream of codes and produce a variety of lag-sequential statistics, including transitional frequencies, expected transitional frequencies, transitional probabilities, adjusted residuals, z values, Yule's Q values, likelihood ratio tests of stationarity across time and homogeneity across groups or segments, transformed kappas for unidirectional dependence, bidirectional dependence, parallel and nonparallel dominance, and significance levels based on both parametric and randomization tests.

Singapore Cancer Network (SCAN) Guidelines for Referral for Genetic Evaluation of Common Hereditary Cancer Syndromes.

PubMed

2015-10-01

The SCAN cancer genetics workgroup aimed to develop Singapore Cancer Network (SCAN) clinical practice guidelines for referral for genetic evaluation of common hereditary cancer syndromes. The workgroup utilised a modified ADAPTE process to calibrate high quality international evidence-based clinical practice guidelines to our local setting. To formulate referral guidelines for the 3 most commonly encountered hereditary cancer syndromes to guide healthcare providers in Singapore who care for cancer patients and/or their family members, 7, 5, and 3 sets of international guidelines respectively for hereditary breast and ovarian cancer (HBOC) syndrome, Lynch syndrome (LS), and familial adenomatous polyposis (FAP) were evaluated. For each syndrome, the most applicable one was selected, with modifications made such that they would be appropriate to the local context. These adapted guidelines form the SCAN Guidelines 2015 for referral for genetic evaluation of common hereditary cancer syndromes.

STS-47 Payload Specialist Mohri conducts visual stability experiment in SLJ

NASA Image and Video Library

1992-09-20

STS047-204-006 (12 - 20 Sept 1992) --- Dr. Mamoru Mohri, payload specialist representing Japan's National Space Development Agency (NASDA), participates in an experiment designed to learn more about Space Adaptation Syndrome (SAS). The experiment is titled, "Comparative Measurement of Visual Stability in Earth and Cosmic Space." During the experiment, Dr. Mohri tracked a flickering light target while eye movements and neck muscle tension were measured. This 45-degree angle position was one of four studied during the eight-day Spacelab-J mission.

The Nature of Fatigue in Chronic Fatigue Syndrome.

PubMed

Olson, Karin; Zimka, Oksana; Stein, Eleanor

2015-10-01

In this article, we report the findings of our study on the nature of fatigue in patients diagnosed with chronic fatigue syndrome. Using ethnoscience as a design, we conducted a series of unstructured interviews and card sorts to learn more about how people with chronic fatigue syndrome describe fatigue. Participants (N = 14) described three distinct domains: tiredness, fatigue, and exhaustion. Most participants experienced tiredness prior to diagnosis, fatigue during daily life, and exhaustion after overexertion. We also discuss participants' ability to adapt to a variety of stressors and prevent shifts to exhaustion, and relate our findings to stress theory and other current research. Primary strategies that promoted adaptation to stressors included pacing and extended rest periods. These findings can aid health care professionals in detecting impending shifts between tiredness, fatigue, and exhaustion and in improving adaptive strategies, thereby improving quality of life. © The Author(s) 2015.

[Mutism and acute behavioral disorders revealing MELAS syndrome].

PubMed

Coomans, H; Barroso, B; Bertandeau, E; Bonnan, M; Dakar, A; Demasles, S; Garraud, S; Krim, E; Martin-Négrier, M-L

2011-11-01

MELAS syndrome (mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes) is a rare genetic mitochondrial disease which can cause cerebral (cerebrovascular accident, migraine, mental deterioration..), sensorial (bilateral symmetrical deafness) and peripheral (muscular involvement, neuropathy) disorders potentially associated with diabetes, renal or cardiac disorders, or growth retardation. Eighty percent of the patients have the 3243 A>G mutation in the leucine RNA transfer gene. Clinical manifestations leading to discovery of the mutation can be extremely varied, affecting patients of different age groups. We report the case of a 49-year-old man who presented acute fits of confusion followed by mutism and praxic disorders. History taking revealed recently diagnosed type 2 diabetes, axonal neuropathy, and bilateral symmetrical deafness requiring hearing aids. The initial MRI showed FLAIR sequences with bi-parietal abnormalities, no signs of recent stroke on the DW/B10000 sequences, and basal ganglia calcifications. Blood tests and morphological findings ruled out a vascular origin. Search for lactic acidosis remained constantly negative in blood samples despite positive cerebrospinal fluid samples (N×3). The 3243 A>G mitochondrial DNA mutation was identified. The neuropsychological evaluation revealed a serious dysexecutive syndrome with a major impact on the patient's self sufficiency. Neurocognitive disorders are not common in MELAS syndrome. Brain MRI results and the presence of extra-neurological signs can be helpful for diagnosis. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Growth hormone deficiency and pituitary malformation in a recurrent Cat-Eye syndrome: a family report.

PubMed

Jedraszak, Guillaume; Braun, Karine; Receveur, Aline; Decamp, Matthieu; Andrieux, Joris; Rabbind Singh, Amrathlal; Copin, Henri; Bremond-Gignac, Dominique; Mathieu, Michèle; Rochette, Jacques; Morin, Gilles

2015-10-01

Growth hormone deficiency affects roughly between one in 3000 and one in 4000 children with most instances of growth hormone deficiency being idiopathic. Growth hormone deficiency can also be associated with genetic diseases or chromosome abnormalities. Association of growth hormone deficiency together with hypothalamic-pituitary axis malformation and Cat-Eye syndrome is a very rare condition. We report a family with two brothers presenting with growth delay due to a growth hormone deficiency associated with a polymalformation syndrome. They both displayed pre-auricular pits and tags, imperforate anus and Duane retraction syndrome. Both parents and a third unaffected son displayed normal growth pattern. Cerebral MRI showed a hypothalamic-pituitary axis malformation in the two affected brothers. Cytogenetic studies revealed a type I small supernumerary marker chromosome derived from chromosome 22 resulting in a tetrasomy 22pter-22q11.21 characteristic of the Cat-Eye syndrome. The small supernumerary marker chromosome was present in the two affected sons and the mother in a mosaic state. Patients with short stature due to growth hormone deficiency should be evaluated for chromosomal abnormality. Family study should not be underestimated. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

A Practitioner-Driven Research Agenda for Syndromic Surveillance.

PubMed

Hopkins, Richard S; Tong, Catherine C; Burkom, Howard S; Akkina, Judy E; Berezowski, John; Shigematsu, Mika; Finley, Patrick D; Painter, Ian; Gamache, Roland; Vilas, Victor J Del Rio; Streichert, Laura C

Syndromic surveillance has expanded since 2001 in both scope and geographic reach and has benefited from research studies adapted from numerous disciplines. The practice of syndromic surveillance continues to evolve rapidly. The International Society for Disease Surveillance solicited input from its global surveillance network on key research questions, with the goal of improving syndromic surveillance practice. A workgroup of syndromic surveillance subject matter experts was convened from February to June 2016 to review and categorize the proposed topics. The workgroup identified 12 topic areas in 4 syndromic surveillance categories: informatics, analytics, systems research, and communications. This article details the context of each topic and its implications for public health. This research agenda can help catalyze the research that public health practitioners identified as most important.

Hybrid measurement chains for the SAS-C spacecraft. [advantages over analog signal processing circuits

NASA Technical Reports Server (NTRS)

Goeke, R. F.

1975-01-01

Spacecraft electronic systems usually demand tight packaging. It was this consideration which initially forced us to consider hybrid circuits for the analog signal processing circuits in the Small Astronomy Satellite-C (SAS-C) scientific payload. We gradually discovered that increased reliability, low power consumption, and reduced program costs all followed. This paper will attempt to share our laboratory's first experience with hybrid circuits and indicate those areas which we found to be important.

Role of adaptive and innate immune cells in chronic fatigue syndrome/myalgic encephalomyelitis.

PubMed

Brenu, Ekua Weba; Huth, Teilah K; Hardcastle, Sharni L; Fuller, Kirsty; Kaur, Manprit; Johnston, Samantha; Ramos, Sandra B; Staines, Don R; Marshall-Gradisnik, Sonya M

2014-04-01

Perturbations in immune processes are a hallmark of a number of autoimmune and inflammatory disorders. Chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME) is an inflammatory disorder with possible autoimmune correlates, characterized by reduced NK cell activity, elevations in regulatory T cells (Tregs) and dysregulation in cytokine levels. The purpose of this article is to examine innate and adaptive immune cell phenotypes and functional characteristics that have not been previously examined in CFS/ME patients. Thirty patients with CFS/ME and 25 non-fatigued controls were recruited for this study. Whole blood samples were collected from all participants for the assessment of cell phenotypes, functional properties, receptors, adhesion molecules, antigens and intracellular proteins using flow cytometric protocols. The cells investigated included NK cells, dendritic cells, neutrophils, B cells, T cells, γδT cells and Tregs. Significant changes were observed in B-cell subsets, Tregs, CD4(+)CD73(+)CD39(+) T cells, cytotoxic activity, granzyme B, neutrophil antigens, TNF-α and IFN-γ in the CFS/ME patients in comparison with the non-fatigued controls. Alterations in B cells, Tregs, NK cells and neutrophils suggest significant impairments in immune regulation in CFS/ME and these may have similarities to a number of autoimmune disorders.

Vestibular Restoration and Adaptation in Vestibular Neuritis and Ramsay Hunt Syndrome With Vertigo.

PubMed

Martin-Sanz, Eduardo; Rueda, Almudena; Esteban-Sanchez, Jonathan; Yanes, Joaquin; Rey-Martinez, Jorge; Sanz-Fernandez, Ricardo

2017-08-01

To evaluate vestibular restoration and the evolution of the compensatory saccades in acute severe inflammatory vestibular nerve paralysis, including vestibular neuritis and Ramsay Hunt syndrome with vertigo. Prospective. Tertiary referral center. Vestibular neuritis (n = 18) and Ramsay Hunt syndrome patients with vertigo (n = 13) were enrolled. After treatment with oral corticosteroids, patients were followed up for 6 months. Functional recovery of the facial nerve was scored according to the House-Brackman grading system. Caloric and video head impulse tests were performed in every patient at the time of enrolment. Subsequently, successive video head impulse test (vHIT) exploration was performed at the 1, 3, and 6-month follow-up. Eighteen patients with vestibular neuritis and 13 with Ramsay Hunt syndrome and associated vertigo were included. Vestibular function was significantly worse in patients with Ramsay Hunt syndrome than in those with vestibular neuritis. Similar compensatory saccades velocity and latency values were observed in both groups, in both the caloric and initial vHIT tests. Successive vHIT results showed a significantly higher vestibulo-ocular reflex gain recovery in vestibular neuritis patients than in Ramsay Hunt syndrome patients. A significantly faster reduction in the latency, velocity, and organization of the compensatory saccades was observed in neuritis than in Ramsay Hunt syndrome patients. In addition to the recovery of the vestibulo-ocular reflex, the reduction of latency, velocity and the organization of compensatory saccades play a role in vestibular compensation.

An update on Cushing syndrome in pediatrics.

PubMed

Stratakis, Constantine A

2018-04-09

Cushing syndrome (CS) in childhood results mostly from the exogenous administration of glucocorticoids; endogenous CS is a rare disease. The latter is the main reason pediatric patients with CS escape diagnosis for too long. Other barriers to optimal care of a pediatric patient with CS include improper following of the proper sequence of testing for diagnosing CS, which stems from lack of understanding of pathophysiology of the hypothalamic-pituitary-adrenal axis; lack of access to proper (i.e., experienced, state-of-the-art) surgical treatment; and unavailability of well-tolerated and effective medications to control hypercortisolemia. This report reviews the state-of-the-art in diagnosing CS and provides an update on the most recent discoveries in its genetics and treatment. Copyright © 2018. Published by Elsevier Masson SAS.

[Loeys-Dietz syndrome (TGFβR2 mutation) in a 4-year-old child with thoracic aortic aneurysm].

PubMed

De Potter, M-J; Edouard, T; Amadieu, R; Plaisancié, J; Julia, S; Hadeed, K; Hascoët, S; Acar, P; Dulac, Y

2016-05-01

Loeys-Dietz syndrome is a rare form of connective tissue disorder, whose clinical features can resemble those of Marfan syndrome, but with a more unpolished appearance. Recently brought out, this pathology remains little known; however, its consequences may be dramatic. We report on the case of a 4-year-old girl followed for a congenital hip dislocation, in which a systematic exam found increased cutaneous elasticity and a bifid uvula, suggesting a connective tissue disorder. Symptoms were unpolished, as the child's height was normal, without any positive cardiac, rheumatological, or ophthalmological family history. Cardiovascular tests found a thoracic aortic aneurysm at the Valsalva sinus (26mm, Z-score=+4.24). A genetic investigation found a TGFβR2 gene mutation, leading to the diagnosis of Loeys-Dietz syndrome type 2. Skeletal damage associated with bifid uvula and/or hypertelorism and an aneurysm of the ascending aorta should guide the genetic investigation to the search for TGF-β vasculopathy such as Loeys-Dietz syndrome. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

People with Usher Syndrome, Type II: Issues and Adaptations.

ERIC Educational Resources Information Center

Miner, I. D.

1997-01-01

Describes the experiences of individuals with Usher Syndrome, discusses the lack of appropriate services and the failure of professionals to provide sufficient information on the condition, and stresses the importance of access to information and the acquisition of new skills before the visual impairment becomes severe. (Author/CR)

Parenting of children with Down syndrome compared to fragile X syndrome.

PubMed

Sterling, Audra; Warren, Steven F

2018-01-01

Children with Down syndrome (DS) and fragile X syndrome (FXS) struggle with language development. Parenting variables, such as responsiveness to children's communication attempts (Maternal Responsivity), and techniques used to support and teach appropriate behavior (Behavior Management) are known to have a significant impact on early child development. We examined these two aspects of parenting style via coded, videotaped parent-child interactions in two groups of participants matched on child age (2-5 years) and child expressive language level: mothers of children with DS and mothers of children with FXS. The mothers differed in their use of gestures and redirecting the child's attention. Overall, mothers in both groups of children appeared to adapt appropriately to their children's developmental needs.

Severe early onset retinitis pigmentosa in a Moroccan patient with Heimler syndrome due to novel homozygous mutation of PEX1 gene.

PubMed

Ratbi, Ilham; Jaouad, Imane Cherkaoui; Elorch, Hamza; Al-Sheqaih, Nada; Elalloussi, Mustapha; Lyahyai, Jaber; Berraho, Amina; Newman, William G; Sefiani, Abdelaziz

2016-10-01

Heimler syndrome (HS) is a rare recessive disorder characterized by sensorineural hearing loss (SNHL), amelogenesis imperfecta, nail abnormalities, and occasional or late-onset retinal pigmentation. It is the mildest form known to date of peroxisome biogenesis disorder caused by hypomorphic mutations of PEX1 and PEX6 genes. We report on a second Moroccan family with Heimler syndrome with early onset, severe visual impairment and important phenotypic overlap with Usher syndrome. The patient carried a novel homozygous missense variant c.3140T > C (p.Leu1047Pro) of PEX1 gene. As standard biochemical screening of blood for evidence of a peroxisomal disorder did not provide a diagnosis in the individuals with HS, patients with SNHL and retinal pigmentation should have mutation analysis of PEX1 and PEX6 genes. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Extensive Intestinal Resection Triggers Behavioral Adaptation, Intestinal Remodeling and Microbiota Transition in Short Bowel Syndrome

PubMed Central

Mayeur, Camille; Gillard, Laura; Le Beyec, Johanne; Bado, André; Joly, Francisca; Thomas, Muriel

2016-01-01

Extensive resection of small bowel often leads to short bowel syndrome (SBS). SBS patients develop clinical mal-absorption and dehydration relative to the reduction of absorptive area, acceleration of gastrointestinal transit time and modifications of the gastrointestinal intra-luminal environment. As a consequence of severe mal-absorption, patients require parenteral nutrition (PN). In adults, the overall adaptation following intestinal resection includes spontaneous and complex compensatory processes such as hyperphagia, mucosal remodeling of the remaining part of the intestine and major modifications of the microbiota. SBS patients, with colon in continuity, harbor a specific fecal microbiota that we called “lactobiota” because it is enriched in the Lactobacillus/Leuconostoc group and depleted in anaerobic micro-organisms (especially Clostridium and Bacteroides). In some patients, the lactobiota-driven fermentative activities lead to an accumulation of fecal d/l-lactates and an increased risk of d-encephalopathy. Better knowledge of clinical parameters and lactobiota characteristics has made it possible to stratify patients and define group at risk for d-encephalopathy crises. PMID:27681910

Radiation hazards to synchronous satellites: The IUE (SAS-D) mission

NASA Technical Reports Server (NTRS)

Stassinopoulos, E. G.

1973-01-01

The ambient trapped particle fluxes incident on the IUE (SAS-D) satellite were studied. Several synchronous elliptical and circular flight paths were evaluated and the effect of inclination, eccentricity, and parking longitude on vehicle encountered intensities was investigated. Temporal variations in the electron environment were considered and partially accounted for. Magnetic field calculations were performed with a current field model extrapolated to a later epoch with linear time terms. Orbital flux integrations were performed with the latest proton and electron environment models using new improved computational methods. The results are presented in graphical and tabular form; they are analyzed, explained, and discussed. Estimates of energetic solar proton fluxes are given for a one year mission at selected integral energies ranging from 10 to 100 MeV, calculated for a year of maximum solar activity during the next solar cycle.

[Usher syndrome].

PubMed

Preda, Mirela; Damian, Carmen; Irimia, Anca; Sollosy, Mihaela; Ciuca, Cristi Adelina; Totolin, Mariana

2008-01-01

We present the case report of two brothers, PF-21 years old and PN-19 years old, to whom the fundus examination, perimetry and dark adaptation established the diagnosis of Retinitis Pigmentosa. The otorhinolaryngology exam and the audiogram revealed, in both cases, bilateral sensorineural deafness. The simultaneous presence of these two conditions completes the clinical findings of Usher syndrome. The common ectodermic origin of the retina and the inner ear could explain this pathological association.

Atomistic modelling of scattering data in the Collaborative Computational Project for Small Angle Scattering (CCP-SAS)

DOE Office of Scientific and Technical Information (OSTI.GOV)

Perkins, Stephen J.; Wright, David W.; Zhang, Hailiang

2016-10-14

The capabilities of current computer simulations provide a unique opportunity to model small-angle scattering (SAS) data at the atomistic level, and to include other structural constraints ranging from molecular and atomistic energetics to crystallography, electron microscopy and NMR. This extends the capabilities of solution scattering and provides deeper insights into the physics and chemistry of the systems studied. Realizing this potential, however, requires integrating the experimental data with a new generation of modelling software. To achieve this, the CCP-SAS collaboration (http://www.ccpsas.org/) is developing open-source, high-throughput and user-friendly software for the atomistic and coarse-grained molecular modelling of scattering data. Robust state-of-the-artmore » molecular simulation engines and molecular dynamics and Monte Carlo force fields provide constraints to the solution structure inferred from the small-angle scattering data, which incorporates the known physical chemistry of the system. The implementation of this software suite involves a tiered approach in whichGenAppprovides the deployment infrastructure for running applications on both standard and high-performance computing hardware, andSASSIEprovides a workflow framework into which modules can be plugged to prepare structures, carry out simulations, calculate theoretical scattering data and compare results with experimental data.GenAppproduces the accessible web-based front end termedSASSIE-web, andGenAppandSASSIEalso make community SAS codes available. Applications are illustrated by case studies: (i) inter-domain flexibility in two- to six-domain proteins as exemplified by HIV-1 Gag, MASP and ubiquitin; (ii) the hinge conformation in human IgG2 and IgA1 antibodies; (iii) the complex formed between a hexameric protein Hfq and mRNA; and (iv) synthetic `bottlebrush' polymers.« less

Atomistic modelling of scattering data in the Collaborative Computational Project for Small Angle Scattering (CCP-SAS).

PubMed

Perkins, Stephen J; Wright, David W; Zhang, Hailiang; Brookes, Emre H; Chen, Jianhan; Irving, Thomas C; Krueger, Susan; Barlow, David J; Edler, Karen J; Scott, David J; Terrill, Nicholas J; King, Stephen M; Butler, Paul D; Curtis, Joseph E

2016-12-01

The capabilities of current computer simulations provide a unique opportunity to model small-angle scattering (SAS) data at the atomistic level, and to include other structural constraints ranging from molecular and atomistic energetics to crystallography, electron microscopy and NMR. This extends the capabilities of solution scattering and provides deeper insights into the physics and chemistry of the systems studied. Realizing this potential, however, requires integrating the experimental data with a new generation of modelling software. To achieve this, the CCP-SAS collaboration (http://www.ccpsas.org/) is developing open-source, high-throughput and user-friendly software for the atomistic and coarse-grained molecular modelling of scattering data. Robust state-of-the-art molecular simulation engines and molecular dynamics and Monte Carlo force fields provide constraints to the solution structure inferred from the small-angle scattering data, which incorporates the known physical chemistry of the system. The implementation of this software suite involves a tiered approach in which GenApp provides the deployment infrastructure for running applications on both standard and high-performance computing hardware, and SASSIE provides a workflow framework into which modules can be plugged to prepare structures, carry out simulations, calculate theoretical scattering data and compare results with experimental data. GenApp produces the accessible web-based front end termed SASSIE-web , and GenApp and SASSIE also make community SAS codes available. Applications are illustrated by case studies: (i) inter-domain flexibility in two- to six-domain proteins as exemplified by HIV-1 Gag, MASP and ubiquitin; (ii) the hinge conformation in human IgG2 and IgA1 antibodies; (iii) the complex formed between a hexameric protein Hfq and mRNA; and (iv) synthetic 'bottlebrush' polymers.

A Tool that Uses the SAS (registered trademark) PRX Functions to Fix Delimited Text Files

DTIC Science & Technology

2015-07-07

service names are registered trademarks or trademarks of SAS Institute Inc. in the USA and other countries...indicates USA registration. Other brand and product names are trademarks of their respective companies. 20 Distribution A: Approved for public release; distribution is unlimited. Case Number: 88ABW-2015-1635, 31 Mar 2015 ...including suggestions for reducing this burden to Department of Defense, Washington Headquarters Services , Directorate for Information

[Effect of vitamin sufficiency on adaptation syndrome in growing rats].

PubMed

Sidorova, Iu S; Beketova, N A; Vrzhesinskaia, O A; Kodentsova, V M; Kosheleva, O V; Zorin, S N; Selifanov, A V; Mazo, V K

2014-01-01

The influence of vitamin supply of growing male -Wistar rats (n=21) with an initial body weight 53,5±0,9 g on their resistance to a single distress induced by the electric shock has been investigated. Control rats within 21 days received a complete semisynthetic diet,providingadequate amounts of vitamins. Combined vitamin deficiency in experimental rats was caused by 5-fold decrease of vitamin mixture amount in the feed and the total vitamin E exclusion from the mixture. On the 21st day, one day before the end of the experiment, both groups of rats were subjected to stress impact (electrocutaneous irritation on paws, 0,4 mA for 8 sec) and then animals were placed in metabolic cages to collect urine. By the end of the experiment, the animals with the combined vitamin deficiency lag behind in growth. Vitamin B2, A, B1 and E liver content decreased in experimental rats by 1,6, 2,3, 4,4 and 15 fold accordingly. Retinol plasma concentration was significantly reduced by 18%, α-tocopherol level - by 5 fold, urinary excretionof riboflavin and 4-pyridoxic acid (vitamin B6 metabolite) was significantly reduced by 6,5 and 2,46 times accordingly. MDA blood plasma concentration and the urinary ratio of oxidized and not oxidized form of 8-hydroxy-2'-deoxy-guanosine did not differ in both groups of rats. Urinary excretion of stress biomarker corticosterone in rats with combined vitamin deficit was 2,5-fold higher than in control rats. Thus, reducing of vitamins supply resulted in an increase of urine corticosterone in stressed rats, that characterized the intensity of general adaptation syndrome. This fact shows the importance of optimal sufficiency with vitamins in nonspecific (general) resistance to stress.

[Effect of enteral supplement of arginine on intestinal adaptation and its mechanism in experimental rats with short bowel syndrome].

PubMed

Jiang, Xiao-hua; Li, Ning; Zhu, Wei-ming; Li, Jie-shou

2009-09-01

To evaluate the effect of enteral supplement of arginine on intestinal adaptation in rats with short bowel syndrome (SBS) and to study its mechanism. SD rats were randomly assigned to three groups: sham rats (Con), SBS rats (SB) and SBS rats supplemented with enteral arginine (SB-Arg). All the animals received isonitrogenic and isocaloric enteral nutrition, except that SB-Arg rats received enteral nutrition supplemented with arginine (300 mg kg(-1) d(-1)). Fat absorbability, plasma free fatty acids, parameters of intestinal adaptation, enterocytes proliferation and apoptosis were determined. After massive small bowel resection, rats had significant bowel adaptation. Compared with SB rats, SB-Arg rats demonstrated a significant increase in fat absorbability [(84.9+/-3.2)% vs [(81.3+/-3.9)%], plasma level of free fatty acids [(650.0+/-86.5) vs (289.5+/-76.9) mg/L], ileal mucosal weight [(18.0+/-3.5) vs (13.5+/-3.0) mg cm(-1) 100 g(-1)], ileal DNA content [(29.6+/-3.3) vs (26.0+/-2.6) microg cm(-1) 100 g(-1)], jejunal mucosal protein content [(65.5+/-7.3) vs (59.8+/-6.2) microg cm(-1) 100 g(-1)], ileal mucosal protein content[(39.2+/-2.3) vs(35.4+/-2.3) microg cm(-1) 100 g(-1)], jejunal mucosal proliferation index [31+/-4 vs 22+/-3] and ileal mucosal proliferation index [32+/-2 vs 25+/-3] (all P<0.05). Moreover, jejunal and ileal villus length, crypt depth and mucosal thickness in SBS-Arg rats were higher than those in SB rats (P<0.05). In rat SBS model, enteral supplement of arginine appears to stimulate intestinal structural and functional adaptation. The mechanism may be that arginine can stimulate enterocyte proliferation and inhibit enterocyte apoptosis.

Space adaptation syndrome experiments (8-IML-1)

NASA Technical Reports Server (NTRS)

Watt, D.

1992-01-01

A set of seven experiments will study adaptation of the human nervous system to weightlessness. Particular emphasis will be placed on the vestibular and proprioceptive systems. The experiments are as follows: the sled/H-reflex; rotation/vestibulo-ocular reflex; the visual stimulator experiment; proprioception (relaxed) experiment; proprioception (active) experiment; proprioception (illusion) experiment; and tactile acuity.

[Hair-thread tourniquet syndrome].

PubMed

Claudet, I; Pasian, N; Maréchal, C; Salanne, S; Debuisson, C; Grouteau, E

2010-05-01

Describe the epidemiology of tourniquet syndromes and a cohort of such children admitted to the pediatric emergency department (PED), analyze the family's social situation to detect neglect behaviors, and analyze subsequent hospital admissions. From 1st January 2003 to 31st May 2009 in the PED, all patients admitted for tourniquet syndrome were included in the study. The data collected were day and time of admission, age, sex, length of stay, medical coverage, type and location of the constrictive agent, therapeutic management, progression, and complications. PED social workers in relation with child protective services (CPS) recovered information on the family's social situation. During the study period, 57 children were registered. The mean number of admissions per year was 8 (range: 2-15). The mean age was 5.5+/-4 months. The toe was the most frequent location (95%). The penis was injured in 2 cases and labia majora in 1 case. The constrictive agent was often a hair (95%). One case of abuse was detected. The analysis of family social situations showed that 53% had no or incomplete medical coverage, 67% were already followed by CPS with extreme poverty or lived in dilapidated housing. Insufficient hygiene or neglect was found in 67% of the families with incomplete or no social coverage. The analysis of 2003-2007 period for later admissions identified that 15 accidents in the home occurred in 12 children. Among these families, 58% were already known by CPS for neglect behavior. Although most tourniquet syndromes seem accidental, this entity is often associated with a lack of hygiene. Several and distant locations (e.g., toes and genitals), multiple and/or separated knots, constrictive agents inconsistent with a safe environment for the child, and penile location in an infant require meticulous investigation because of a higher incidence of child neglect. Copyright 2010 Elsevier Masson SAS. All rights reserved.

Usual Dietary Intakes: SAS Macros for Estimating Ratios of Two Dietary Components that are Consumed Nearly Every Day

Cancer.gov

The following SAS macros can be used to create a bivariate distribution of usual intake of two dietary components that are consumed nearly every day and to calculate percentiles of the population distribution of the ratio of usual intakes.

The identification of Lynch syndrome in Congolese colorectal cancer patients.

PubMed

Poaty, Henriette; Aba Gandzion, Chandra; Soubeyran, Isabelle; Gassaye, Déby; Peko, Jean Félix; Nkoua Bon, Jean Bernard; Gombé Mbalawa, Charles

2017-10-01

We aimed to investigate the prevalence of Lynch syndrome as one of hereditary causes of colorectal cancer (CRC) among young Congolese individuals affected by the CRC, and to define methods for diagnosis in Congo Brazzaville. We conducted a transversal cohort study of 34 patients having a CRC with a family history for a period of eight years. They were selected among 89 CRCs of any type from the Bethesda guidelines criteria combined with pedigrees. Mismatch repair (MMR) genes alterations were researched by immunohistochemistry (IHC). We identified with the Bethesda criteria a total of 38.2% (34/89) patients having familial CRC with a confidence interval (CI) of 95%=[0.34-0.41]. Only 14.7% (5/34) 95% CI=[0.34-2.32] patients showed MMR immunodeficiency involving firstly MLH1 protein then MSH2 protein. These data account for 5.6% (5/89) 95% CI=[0.15-0.33] of patients affected by Lynch syndrome with an earlier median age of 35 years (range 20 to 47 years). The prevalence of Lynch syndrome found in Brazzaville is comparable to that is found in northern countries. The combined Bethesda guidelines, pedigree and IHC is an accessible and good alternative method for the positive diagnosis of Lynch syndrome in current practice in Congo. Copyright © 2017 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.

Space adaptation syndrome: multiple etiological factors and individual differences

NASA Technical Reports Server (NTRS)

Lackner, J. R.; DiZio, P.

1991-01-01

Space motion sickness is a significant operational concern in the American and Soviet space programs. Nearly 70% of all astronauts and cosmonauts are affected to some degree during their first several days of flight. It is now beginning to appear that space motion sickness like terrestrial motion sickness is the consequence of multiple etiological factors. As we come to understand basic mechanisms of spatial orientation and sensory-motor adaptation we can begin to predict etiological factors in different motion environments. Individuals vary greatly in the extent to which they are susceptible to these different factors. However, individuals seem to be relatively self-consistent in terms of their rates of adaptation to provocative stimulation and their retention of adaptation. Attempts to relate susceptibility to motion sickness during the microgravity phases of parabolic flight maneuvers to vestibular function under 1G and 0G test conditions are described.

Reliability study of the Behavioral Assessment of the Dysexecutive Syndrome adapted for a Brazilian sample of older-adult controls and probable early Alzheimer's disease patients.

PubMed

Canali, Fabíola; Brucki, Sonia M D; Bertolucci, Paulo H F; Bueno, Orlando F A

2011-12-01

Ecological tests are useful in assessing executive function deficits and may be of value in appraising response to treatment in Alzheimer's disease patients. Our aims were to examine executive function using the Behavioral Assessment of the Dysexecutive Syndrome for a Brazilian sample of older-adult controls and probable early Alzheimer's disease patients, and verify the applicability of this test battery. Forty-one older-adult controls were matched with mild Alzheimer's disease patients by age, education, and gender. There significant inter-group differences in overall profile and almost all subtests except temporal judgment, time spent on planning the first and second Zoo Map visit, number of errors when copying drawings, naming pictures and Six Modified Elements arithmetic, and dysexecutive questionnaire self-rating. The Behavioral Assessment of the Dysexecutive Syndrome item that best discriminated controls from patients was the Modified Six Elements - adapted (general index), with a sensitivity of 80% and specificity of 90%, (AUC = 0.91, p < 0.001). Behavioral Assessment of the Dysexecutive Syndrome was effective in detecting executive function deficits in mild Alzheimer's disease patients, particularly the task switching, time monitoring, and rule-shift subtests.

Treatment-related toxicities in children with acute lymphoblastic leukaemia predisposition syndromes.

PubMed

Schmiegelow, Kjeld

2016-12-01

Although most children with acute lymphoblastic leukaemia (ALL) do not harbor germline mutations that strongly predispose them to development of this malignancy, large syndrome registries and detailed mapping of exomes or whole genomes of familial leukaemia kindreds have revealed that 3-5% of all childhood ALL cases are due to such germline mutations, but the figure may be higher. Most of these syndromes are primarily characterized by their non-malignant phenotype, whereas ALL may be the dominating or even only striking manifestation of the syndrome in some families. Identification of such ALL patients is important in order to adjust therapy and offer genetic counseling and cancer surveillance to mutation carriers in the family. In the coming years large genomic screening projects are expected to reveal further hitherto unrecognised familial ALL syndromes. The treatment of ALL cases harboring cancer predisposing mutations can be challenging for both the physician and the patient due to their preexisting symptoms, their reduced tolerance to radio- and/or chemotherapy with enhanced risk of life-threatening organ toxicities, and the paucity of data from ALL patients with the same or similar syndromes being treated by contemporary protocols. Recent studies clearly indicate that many of these patients stand a good chance of cure, and that they should be offered chemotherapy with the intention to cure. Some of these syndromes are characterized by reduced tolerance to radiotherapy and/or specific anticancer agents, while others are not. This review summarises our current knowledge on the risk of acute toxicities for these ALL patients and provides guidance for treatment adjustments. Copyright © 2016. Published by Elsevier Masson SAS.

A newly recognized syndrome of severe growth deficiency, microcephaly, intellectual disability, and characteristic facial features.

PubMed

Vinkler, Chana; Leshinsky-Silver, Esther; Michelson, Marina; Haas, Dorothea; Lerman-Sagie, Tally; Lev, Dorit

2014-01-01

Genetic syndromes with proportionate severe short stature are rare. We describe two sisters born to nonconsanguineous parents with severe linear growth retardation, poor weight gain, microcephaly, characteristic facial features, cutaneous syndactyly of the toes, high myopia, and severe intellectual disability. During infancy and early childhood, the girls had transient hepatosplenomegaly and low blood cholesterol levels that normalized later. A thorough evaluation including metabolic studies, radiological, and genetic investigations were all normal. Cholesterol metabolism and transport were studied and no definitive abnormality was found. No clinical deterioration was observed and no metabolic crises were reported. After due consideration of other known hereditary causes of post-natal severe linear growth retardation, microcephaly, and intellectual disability, we propose that this condition represents a newly recognized autosomal recessive multiple congenital anomaly-intellectual disability syndrome. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

SAS Code for Calculating Intraclass Correlation Coefficients and Effect Size Benchmarks for Site-Randomized Education Experiments

ERIC Educational Resources Information Center

Brandon, Paul R.; Harrison, George M.; Lawton, Brian E.

2013-01-01

When evaluators plan site-randomized experiments, they must conduct the appropriate statistical power analyses. These analyses are most likely to be valid when they are based on data from the jurisdictions in which the studies are to be conducted. In this method note, we provide software code, in the form of a SAS macro, for producing statistical…

Microduplications at 22q11.21 are associated with non-syndromic classic bladder exstrophy.

PubMed

Draaken, Markus; Reutter, Heiko; Schramm, Charlotte; Bartels, Enrika; Boemers, Thomas M; Ebert, Anne-Karoline; Rösch, Wolfgang; Schröder, Annette; Stein, Raimund; Moebus, Susanne; Stienen, Dietlinde; Hoffmann, Per; Nöthen, Markus M; Ludwig, Michael

2010-01-01

The exstrophy-epispadias complex (EEC) comprises a spectrum of urogenital anomalies in which part or all of the distal urinary tract fails to close. The present study aimed to identify microaberrations characterized by loss or gain of genomic material that contribute to the EEC at a genome-wide level. Molecular karyotyping, utilizing 549,839 single nucleotide polymorphisms (SNPs) with an average spacing of 5.7 kilobases, was performed to screen an initial cohort of 16 patients with non-syndromic EEC. A de novo microduplication involving chromosomal region 22q11.21 was identified in one patient with classic exstrophy of the bladder (CBE). Subsequent multiplex ligation-dependent probe amplification (MLPA) analysis was performed with an MLPA 22q11 kit in a further 50 non-syndromic EEC cases. We identified one CBE patient with an overlapping 22q11.21 duplication in whom the duplication had been transmitted from the unaffected mother. Chromosomal region 22q11 is well known for its susceptibility to genomic rearrangements, and these are associated with various syndromes including the velo-cardio-facial/DiGeorge syndrome (VCFS/DGS), the der(22) syndrome, and the cat-eye syndrome. Duplications in this region result in a wide and variable spectrum of clinical presentations that include features of the VCFS/DGS, while some carriers present with a completely normal phenotype. Our findings extend the phenotypic spectrum of the 22q11.2 duplication syndrome, and indicate that this aberration predisposes to CBE with incomplete penetrance. Copyright 2009 Elsevier Masson SAS. All rights reserved.

Behavioral Phenotype of Fragile X Syndrome in Adolescence and Adulthood

PubMed Central

Smith, Leann E.; Barker, Erin T.; Seltzer, Marsha Mailick; Abbeduto, Leonard; Greenberg, Jan S.

2012-01-01

The present study explored the behavioral profile of individuals with fragile X syndrome during adolescence and adulthood. Individuals with both fragile X syndrome and autism (n = 30) were compared with (a) individuals diagnosed with fragile X syndrome (but not autism; n = 106) and (b) individuals diagnosed with autism (but not fragile X syndrome; n = 135) on measures of autism symptoms, adaptive functioning, behavior problems, and psychological symptoms. Results indicated that individuals dually diagnosed with fragile X syndrome and autism displayed greater communication and social reciprocity impairments than individuals with fragile X syndrome only. Individuals in the dually diagnosed group also exhibited higher levels of repetitive and challenging behaviors than either comparison group, suggesting a unique profile of vulnerability for those diagnosed with both fragile X syndrome and autism. PMID:22264109

Understanding How Astronauts Adapt to Space and to Earth: Anatomical Studies of Central Vestibular Adaptation

NASA Technical Reports Server (NTRS)

Holstein, Gay; Vasques, Marilyn; Aquilina, Rudy (Technical Monitor)

2002-01-01

Significant changes take place in the nervous systems of astronauts during and following exposure to microgravity. These changes, particularly in the part of the brain that controls balance, the vestibular system, can cause sensations of rotation, dizziness, and vertigo, as well as space adaptation syndrome. Adaptation to the microgravity environment usually occurs within one week, and a subsequent re-adaptation period of several days is often required upon return to Earth. In order to realize long-term spaceflight, effective countermeasures for these symptoms must be developed. The structural changes that take place in one of the vestibular regions of the brain (the cerebellar cortex) during the process of adaptation to Earth's gravity remain unclear and are the subject of an experiment being conducted on STS-107 by Dr. Gay Holstein of the Mount Sinai School of Medicine in New York. Using the rat as a model, Dr. Holstein and her team will seek to identify the cellular changes underlying the vestibular changes experienced by astronauts.

Model-based adaptive phase I trial design of post-transplant decitabine maintenance in myelodysplastic syndrome.

PubMed

Han, Seunghoon; Kim, Yoo-Jin; Lee, Jongtae; Jeon, Sangil; Hong, Taegon; Park, Gab-Jin; Yoon, Jae-Ho; Yahng, Seung-Ah; Shin, Seung-Hwan; Lee, Sung-Eun; Eom, Ki-Seong; Kim, Hee-Je; Min, Chang-Ki; Lee, Seok; Yim, Dong-Seok

2015-10-23

This report focuses on the adaptive phase I trial design aimed to find the clinically applicable dose for decitabine maintenance treatment after allogeneic hematopoietic stem cell transplantation in patients with higher-risk myelodysplastic syndrome and secondary acute myeloid leukemia. The first cohort (three patients) was given the same initial daily dose of decitabine (5 mg/m(2)/day, five consecutive days with 4-week intervals). In all cohorts, the doses for Cycles 2 to 4 were individualized using pharmacokinetic-pharmacodynamic modeling and simulations. The goal of dose individualization was to determine the maximum dose for each patient at which the occurrence of grade 4 (CTC-AE) toxicities for both platelet and neutrophil counts could be avoided. The initial doses for the following cohorts were also estimated with the data from the previous cohorts in the same manner. In all but one patient (14 out of 15), neutrophil count was the dose-limiting factor throughout the cycles. In cycles where doses were individualized, the median neutrophil nadir observed was 1100/mm(3) (grade 2) and grade 4 toxicity occurred in 5.1 % of all cycles (while it occurred in 36.8 % where doses were not individualized). The initial doses estimated for cohorts 2 to 5 were 4, 5, 5.5, and 5 mg/m(2)/day, respectively. The median maintenance dose was 7 mg/m(2)/day. We determined the acceptable starting dose and individualized the maintenance dose for each patient, while minimizing the toxicity using the adaptive approach. Currently, 5 mg/m(2)/day is considered to be the most appropriate starting dose for the regimen studied. Clinicaltrials.gov NCT01277484.

Diagnosis of motor fascicle compression in carpal tunnel syndrome.

PubMed

Modi, C S; Ho, K; Hegde, V; Boer, R; Turner, S M

2010-06-01

Median nerve motor fascicle compression in patients with carpal tunnel syndrome is usually characterised by reduced finger grip and pinch strength, loss of thumb abduction and opposition strength and thenar atrophy. The functional outcome in patients with advanced changes may be poor due to irreversible intraneural changes. The aim of this study was to investigate patient-reported symptoms, which may enable a clinical diagnosis of median nerve motor fascicle compression to be made irrespective of the presence of advanced signs. One hundred and twelve patients (166 hands) with a clinical diagnosis of carpal tunnel syndrome were referred to the neurophysiology department and completed symptom severity questionnaires with subsequent neurophysiological testing. An increasing frequency of pain experienced by patients was significantly associated with an increased severity of median nerve motor fascicle compression with prolonged motor latencies measured in patients that described pain as a predominant symptom. An increasing frequency of paraesthesia and numbness and weakness associated with dropping objects was significantly associated with both motor and sensory involvement but not able to distinguish between them. This study suggests that patients presenting with a clinical diagnosis of carpal tunnel syndrome with pain as a frequently experienced and predominant symptom require consideration for urgent investigation and surgical treatment to prevent chronic motor fascicle compression with permanent functional deficits. Copyright 2010 Elsevier Masson SAS. All rights reserved.

Success of tardive electroconvulsive therapy sessions after loxapine-induced malignant syndrome in the context of very poor metabolisation.

PubMed

Descoeur, Juliette; Philibert, Laurent; Chalard, Kevin; Attal, Jérôme; Petit, Pierre; Klouche, Kada; Olivier, Mathieu

2017-12-01

We report the success of tardive electroconvulsive therapy in a case of loxapine malignant syndrome with catatonia. Loxapine and its metabolites were measured in biological samples by liquid chromatography coupled to tandem mass spectrometry. Genes were studied by sequencing and quantitative polymerase chain reaction (PCR). Plasmatic drug concentrations showed a supratherapeutic concentration of loxapine with a very low 8-hydroxyloxapine/loxapine ratio (range from 0.32 to 0.66, normal value>2 for 100mg) and a very long elimination half-life of loxapine (half-life>140h, normal value from 1 to 4hours). We tried to explain this kinetics by exploring the main pharmacogenes implicated in the metabolism of loxapine. No genetic abnormality for CYP1A2 was observed. The study of associated treatments showed the potential contribution of valproate. Pharmacokinetics and pharmacogenetics investigations revealed a blockade of the CYP1A2 metabolic pathway without genetic abnormalities, probably due to valproate co-medication. Toxicological monitoring of loxapine and its metabolites helped to explain the persistence of symptoms and to adapt the therapeutic management. Copyright © 2017 Société française de pharmacologie et de thérapeutique. Published by Elsevier Masson SAS. All rights reserved.

Increasing circular synthetic aperture sonar resolution via adapted wave atoms deconvolution.

PubMed

Pailhas, Yan; Petillot, Yvan; Mulgrew, Bernard

2017-04-01

Circular Synthetic Aperture Sonar (CSAS) processing computes coherently Synthetic Aperture Sonar (SAS) data acquired along a circular trajectory. This approach has a number of advantages, in particular it maximises the aperture length of a SAS system, producing very high resolution sonar images. CSAS image reconstruction using back-projection algorithms, however, introduces a dissymmetry in the impulse response, as the imaged point moves away from the centre of the acquisition circle. This paper proposes a sampling scheme for the CSAS image reconstruction which allows every point, within the full field of view of the system, to be considered as the centre of a virtual CSAS acquisition scheme. As a direct consequence of using the proposed resampling scheme, the point spread function (PSF) is uniform for the full CSAS image. Closed form solutions for the CSAS PSF are derived analytically, both in the image and the Fourier domain. The thorough knowledge of the PSF leads naturally to the proposed adapted atom waves basis for CSAS image decomposition. The atom wave deconvolution is successfully applied to simulated data, increasing the image resolution by reducing the PSF energy leakage.

Impact of environmental adaptation on tear film assessments.

PubMed

Fagehi, R

2018-03-01

The purpose of this study was to investigate the effect of ocular environmental adaptation on clinical tear film assessment. Thirty subjects (male, mean age 23±2.5) participated in this study. A number of clinical tear film tests were applied, including: fluorescein tear break-up time (FTBUT), Schirmer test and tear prism height test (TPH). The tear physiology of each subject was evaluated twice, once immediately when they arrived from the external environment, and then after 30minutes adaptation in the exam room environment. The mean values were: Schirmer test A (22.1±2.99), Schirmer test B (24.2±2.63), FTBUT A (8.00±1.94), FTBUT B (9.13±2.04), TPH A (0.179±0.026) and TPH B* (0.187±0.023). Statistical testing using Wilcoxon-signed rank test showed a significant difference between the Schirmer test results measured at the different times (P=0.008). Also, the FTBUT and tear prism height test results showed significant differences between the two evaluation times, (P=0.001, 0.011, respectively) (A: tear assessed when the subject comes from the outside environment, B: tear film assessed after 30min adaptation in the clinical environment). This study showed a significant difference between the tear film test results evaluated when the subjects were assessed immediately from the outside environment and after an adaptation time in the clinic environment. Practitioners must consider the effect of differences between external and clinical environment adaptation on clinical tear film physiology. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

Ambulatory Feedback System

NASA Technical Reports Server (NTRS)

Finger, Herbert; Weeks, Bill

1985-01-01

This presentation discusses instrumentation that will be used for a specific event, which we hope will carry on to future events within the Space Shuttle program. The experiment is the Autogenic Feedback Training Experiment (AFTE) scheduled for Spacelab 3, currently scheduled to be launched in November, 1984. The objectives of the AFTE are to determine the effectiveness of autogenic feedback in preventing or reducing space adaptation syndrome (SAS), to monitor and record in-flight data from the crew, to determine if prediction criteria for SAS can be established, and, finally, to develop an ambulatory instrument package to mount the crew throughout the mission. The purpose of the Ambulatory Feedback System (AFS) is to record the responses of the subject during a provocative event in space and provide a real-time feedback display to reinforce the training.

The Music Therapy Session Assessment Scale (MT-SAS): Validation of a new tool for music therapy process evaluation.

PubMed

Raglio, Alfredo; Gnesi, Marco; Monti, Maria Cristina; Oasi, Osmano; Gianotti, Marta; Attardo, Lapo; Gontero, Giulia; Morotti, Lara; Boffelli, Sara; Imbriani, Chiara; Montomoli, Cristina; Imbriani, Marcello

2017-11-01

Music therapy (MT) interventions are aimed at creating and developing a relationship between patient and therapist. However, there is a lack of validated observational instruments to consistently evaluate the MT process. The purpose of this study was the validation of Music Therapy Session Assessment Scale (MT-SAS), designed to assess the relationship between therapist and patient during active MT sessions. Videotapes of a single 30-min session per patient were considered. A pilot study on the videotapes of 10 patients was carried out to help refine the items, define the scoring system and improve inter-rater reliability among the five raters. Then, a validation study on 100 patients with different clinical conditions was carried out. The Italian MT-SAS was used throughout the process, although we also provide an English translation. The final scale consisted of 7 binary items accounting for eye contact, countenance, and nonverbal and sound-music communication. In the pilot study, raters were found to share an acceptable level of agreement in their assessments. Explorative factorial analysis disclosed a single homogeneous factor including 6 items (thus supporting an ordinal total score), with only the item about eye contact being unrelated to the others. Moreover, the existence of 2 different archetypal profiles of attuned and disattuned behaviours was highlighted through multiple correspondence analysis. As suggested by the consistent results of 2 different analyses, MT-SAS is a reliable tool that globally evaluates sonorous-musical and nonverbal behaviours related to emotional attunement and empathetic relationship between patient and therapist during active MT sessions. Copyright © 2017 John Wiley & Sons, Ltd.

Effects of 2D and 3D Error Fields on the SAS Divertor Magnetic Topology

NASA Astrophysics Data System (ADS)

Trevisan, G. L.; Lao, L. L.; Strait, E. J.; Guo, H. Y.; Wu, W.; Evans, T. E.

2016-10-01

The successful design of plasma-facing components in fusion experiments is of paramount importance in both the operation of future reactors and in the modification of operating machines. Indeed, the Small Angle Slot (SAS) divertor concept, proposed for application on the DIII-D experiment, combines a small incident angle at the plasma strike point with a progressively opening slot, so as to better control heat flux and erosion in high-performance tokamak plasmas. Uncertainty quantification of the error fields expected around the striking point provides additional useful information in both the design and the modeling phases of the new divertor, in part due to the particular geometric requirement of the striking flux surfaces. The presented work involves both 2D and 3D magnetic error field analysis on the SAS strike point carried out using the EFIT code for 2D equilibrium reconstruction, V3POST for vacuum 3D computations and the OMFIT integrated modeling framework for data analysis. An uncertainty in the magnetic probes' signals is found to propagate non-linearly as an uncertainty in the striking point and angle, which can be quantified through statistical analysis to yield robust estimates. Work supported by contracts DE-FG02-95ER54309 and DE-FC02-04ER54698.

Enhancing the solubility and bioavailability of poorly water-soluble drugs using supercritical antisolvent (SAS) process.

PubMed

Abuzar, Sharif Md; Hyun, Sang-Min; Kim, Jun-Hee; Park, Hee Jun; Kim, Min-Soo; Park, Jeong-Sook; Hwang, Sung-Joo

2018-03-01

Poor water solubility and poor bioavailability are problems with many pharmaceuticals. Increasing surface area by micronization is an effective strategy to overcome these problems, but conventional techniques often utilize solvents and harsh processing, which restricts their use. Newer, green technologies, such as supercritical fluid (SCF)-assisted particle formation, can produce solvent-free products under relatively mild conditions, offering many advantages over conventional methods. The antisolvent properties of the SCFs used for microparticle and nanoparticle formation have generated great interest in recent years, because the kinetics of the precipitation process and morphologies of the particles can be accurately controlled. The characteristics of the supercritical antisolvent (SAS) technique make it an ideal tool for enhancing the solubility and bioavailability of poorly water-soluble drugs. This review article focuses on SCFs and their properties, as well as the fundamentals of overcoming poorly water-soluble drug properties by micronization, crystal morphology control, and formation of composite solid dispersion nanoparticles with polymers and/or surfactants. This article also presents an overview of the main aspects of the SAS-assisted particle precipitation process, its mechanism, and parameters, as well as our own experiences, recent advances, and trends in development. Copyright © 2017 Elsevier B.V. All rights reserved.

QT Adaptation and Intrinsic QT Variability in Congenital Long QT Syndrome.

PubMed

Seethala, Srikanth; Singh, Prabhpreet; Shusterman, Vladimir; Ribe, Margareth; Haugaa, Kristina H; Němec, Jan

2015-12-16

Increased variability of QT interval (QTV) has been linked to arrhythmias in animal experiments and multiple clinical situations. Congenital long QT syndrome (LQTS), a pure repolarization disease, may provide important information on the relationship between delayed repolarization and QTV. Twenty-four-hour Holter monitor tracings from 78 genotyped congenital LQTS patients (52 females; 51 LQT1, 23 LQT2, 2 LQT5, 2 JLN, 27 symptomatic; age, 35.2±12.3 years) were evaluated with computer-assisted annotation of RR and QT intervals. Several models of RR-QT relationship were tested in all patients. A model assuming exponential decrease of past RR interval contributions to QT duration with 60-second time constant provided the best data fit. This model was used to calculate QTc and residual "intrinsic" QTV, which cannot be explained by heart rate change. The intrinsic QTV was higher in patients with long QTc (r=0.68; P<10(-4)), and in LQT2 than in LQT1/5 patients (5.65±1.28 vs 4.46±0.82; P<0.0002). Both QTc and intrinsic QTV were similar in symptomatic and asymptomatic patients (467±52 vs 459±53 ms and 5.10±1.19 vs 4.74±1.09, respectively). In LQTS patients, QT interval adaptation to heart rate changes occurs with time constant ≈60 seconds, similar to results reported in control subjects. Intrinsic QTV correlates with the degree of repolarization delay and might reflect action potential instability observed in animal models of LQTS. © 2015 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

A Comparison of Behavioral and Emotional Characteristics in Children with Autism, Prader-Willi Syndrome, and Williams Syndrome

ERIC Educational Resources Information Center

Dimitropoulos, Anastasia; Ho, Alan Y.; Klaiman, Cheryl; Koenig, Kathy; Schultz, Robert T.

2009-01-01

In order to investigate unique and shared characteristics and to determine factors predictive of group classification, quantitative comparisons of behavioral and emotional problems were assessed using the Developmental Behavior Checklist (DBC-P) and the Vineland Adaptive Behavior Scales in autistic disorder, Williams syndrome (WS), and…

Overview of Social Cognitive Dysfunctions in Rare Developmental Syndromes With Psychiatric Phenotype

PubMed Central

Morel, Aurore; Peyroux, Elodie; Leleu, Arnaud; Favre, Emilie; Franck, Nicolas; Demily, Caroline

2018-01-01

Rare neurodevelopmental syndromes often present social cognitive deficits that may underlie difficulties in social interactions and increase the risk of psychosis or autism spectrum disorders. However, little is known regarding the specificities of social cognitive impairment across syndromes while it remains a major challenge for the care. Our review provides an overview of social cognitive dysfunctions in rare diseases associated with psychiatric symptoms (with a prevalence estimated between 1 in 1,200 and 1 in 25,000 live births: 22q11.2 deletion syndrome, Angelman syndrome, Fragile X syndrome, Klinefelter syndrome, Prader–Willi syndrome, Rett syndrome, Smith–Magenis syndrome, Turner syndrome, and Williams syndrome) and shed some light on the specific mechanisms that may underlie these skills in each clinical presentation. We first detail the different processes included in the generic expression “social cognition” before summarizing the genotype, psychiatric phenotype, and non-social cognitive profile in each syndrome. Then, we offer a systematic review of the social cognitive abilities and the disturbed mechanisms they are likely associated with. We followed the PRISMA process, including the definition of the relevant search terms, the selection of studies based on clear inclusion, and exclusion criteria and the quality appraisal of papers. We finally provide insights that may have considerable influence on the development of adapted therapeutic interventions such as social cognitive training (SCT) therapies specifically designed to target the psychiatric phenotype. The results of this review suggest that social cognition impairments share some similarities across syndromes. We propose that social cognitive impairments are strongly involved in behavioral symptoms regardless of the overall cognitive level measured by intelligence quotient. Better understanding the mechanisms underlying impaired social cognition may lead to adapt therapeutic

[Demons and Meigs syndromes and pseudosyndromes today].

PubMed

Brun, G-H

2010-05-01

There is a total confusion between Demons or Meigs syndromes and pseudosyndromes.The need for greater precision in publications titles has become an emergency. We have analysed 297 observations gathered in litterature from 1904 to 2004. The increase in the number of Meigs pseudosyndromes based on diverse associated condition means that we must include under the name Demons syndrome, as he did himself, all tumours of the female genital tract, providing they are benign, wherever the effusion takes place, reserve the name Demons Meigs because it has been used historically and because it was Meigs wish, solely for fibrothecomas of the ovary and for tumours of the granulosa bring together under the name Demons pseudosyndrome all other non tumoral benign entities. Malignant tumours, with or without malignant cells in the effusions are not pseudosyndromes but authentic neoplastic lesions. An over-lax definition of pseudosyndromes runs the risk of the name simply becoming a catch-all term. On the other hand we can say that 100years later diagnosis is not earlier, a bilateral tumor is not a rarity, mechanisms of effusions genesis remain mysterious and the treatment has been enriched with coeliosurgery. Copyright 2009 Elsevier Masson SAS. All rights reserved.

Unusual association of turner syndrome and hypopituitarism in a Tunisian family.

PubMed

Bougacha-Elleuch, N; Elleuch, M; Charfi, N; Mnif, F; Belghith, N; Abdelhedi, F; Kammoun, H; Hachicha, M; Mnif, M; Abid, M

2016-01-01

Familial occurrence of either Turner syndrome or hypopituitarism is very rare. Particularly, their association is an uncommon finding. In this context, we describe for the first time 4 sisters with Turner syndrome, hypopituitarism was reported in three among them. Our cohort consists of four Tunisian adult sisters belonging to a consanguineous family. Biochemical analysis, resonance magnetic imaging and cytogenetic analyses were performed. Turner syndrome was diagnosed at the ages of 14, 17, 31 and 43 years in cases 1, 2, 3 and 4 respectively. They suffered from short stature, dysmorphic syndrome and/or delayed puberty. Interestingly, 3 among them showed also hypopituitarism, hypogonadotrophic hypogonadism and central hypothyroidism. Somatotropic insufficiency was proven in one case. Pituitary MRI has shown an empty sella turcica with hypoplastic pituitary gland in three cases. Their karyotypes were compatible with 45X in one case, 45X/46XX in the second and 45X/46XX/47XXY with x label in two cases. Hence, the presence of these familial cases of TS must evoke new etiopathogenetic arguments. Coincidence of hypopituitarism in this family, might suggest common genetic background for the two diseases. This particular family would be a precious tool for an extensive molecular analysis. More attention should be given to other family's members mainly in the presence of delayed puberty and sterility in other members. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Cdk1 Phosphorylates Drosophila Sas-4 to Recruit Polo to Daughter Centrioles and Convert Them to Centrosomes.

PubMed

Novak, Zsofia A; Wainman, Alan; Gartenmann, Lisa; Raff, Jordan W

2016-06-20

Centrosomes and cilia are organized by a centriole pair comprising an older mother and a younger daughter. Centriole numbers are tightly regulated, and daughter centrioles (which assemble in S phase) cannot themselves duplicate or organize centrosomes until they have passed through mitosis. It is unclear how this mitotic "centriole conversion" is regulated, but it requires Plk1/Polo kinase. Here we show that in flies, Cdk1 phosphorylates the conserved centriole protein Sas-4 during mitosis. This creates a Polo-docking site that helps recruit Polo to daughter centrioles and is required for the subsequent recruitment of Asterless (Asl), a protein essential for centriole duplication and mitotic centrosome assembly. Point mutations in Sas-4 that prevent Cdk1 phosphorylation or Polo docking do not block centriole disengagement during mitosis, but block efficient centriole conversion and lead to embryonic lethality. These observations can explain why daughter centrioles have to pass through mitosis before they can duplicate and organize a centrosome. Copyright © 2016 The Author(s). Published by Elsevier Inc. All rights reserved.

[A tamponade complicating an acute eosinophilic pericarditis due to a myeloproliferative/myelodysplastic syndrome].

PubMed

de Moreuil, C; Lieber, A; Marjanovic, Z; Bobbio, A; Alavi, Z; Blacher, J; Marie, J-P

2016-02-01

Cardiac involvement in eosinophilia is potentially fatal and requires early diagnosis and prompt treatment. We report here the case of a 71-year-old female patient with eosinophilia>10,000/mm(3) for 2 months due to a myeloproliferative/myelodysplastic syndrome, with a rapidly progressive exertional dyspnea explained by an important circumferential eosinophilic pericarditis. Due to a rapid evolution to a tamponade, an emergent surgical drainage was performed. Subsequent medical treatment combined high-dose corticosteroids (1mg/kg/day) with hydroxyurea and imatinib. The outcome was favourable with regression of the effusion, of the volume overload symptoms and decrease in eosinophilia. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

International Symposium on Epidemic Hemorrhagic Fever (Hemorrhagic Fever with Renal Syndrome) Held in Wuhan, Hubei, China on 31 October - 2 November 1988

DTIC Science & Technology

1989-10-01

15 Song Gan Prophylaxis of Hemorrhagic Fever With Renal Syndrome, Development of Inactivated Cell Culture Vaccine Against HFRS 11:45 END EPIDEMIOLOGYt...Intraveinous Ribavirin or Placebo 11:15 Yang Zhi-cheng Effect of Ribavirin on the White Blood Cell System and the Platelet of the Patients With EHF...from R. nortegicus ias madc by Hlung T. et al (5. A). It %sas a ers brilliant piecc of %;ork. because by observing thin-sections of infected cells in

[Bilateral chronic dislocation of the temporomandibular joints and Meige syndrome].

PubMed

Arzul, L; Henoux, M; Marion, F; Corre, P

2015-04-01

Chronic dislocation of the temporo-mandibular joint (TMJ) is rare. It occurs when an acute dislocation is left untreated, in certain situations, including severe illness, neurologic or psychiatric diseases or prolonged oral intubation. A 79 years old woman, with Meige syndrome, suffered from bilateral dislocation of the TMJ for over 1 year. Surgical repositioning of the mandibular condyles and temporal bone eminectomy were performed. At the 18 postoperative months control, no recurrence has been noted. Treatment of chronic TMJ dislocations often requires a surgical procedure. Manual reduction, even under general anaesthesia, often fails because of severe muscular spasm and periarticular fibrotic changes. The management of this disorder is still controversial. We review available surgical procedures. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

A new mutation in the COL4A3 gene responsible for autosomal dominant Alport syndrome, which only generates hearing loss in some carriers.

PubMed

Rosado, Consolación; Bueno, Elena; Fraile, Pilar; García-Cosmes, Pedro; González-Sarmiento, Rogelio

2015-01-01

Bilateral sensorineural hearing loss is a characteristic feature of Alport syndrome, which is always linked to renal manifestations so they have a parallel evolution and prognosis, and deafness helps to identify the renal disease. We report a family that suffers an autosomal dominant Alport syndrome caused by a previously undescribed mutation in the COL4A3 gene, in which several members have hearing impairment as the only clinical manifestation, suggesting that in this family deafness can occur independent of renal disease. This mutation is also present in a patient with anterior lenticonus, an observation only found in families with recessive and sex-linked Alport disease. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

SAS-2 gamma-ray observations of PSR 1747-46. [radio pulsar

NASA Technical Reports Server (NTRS)

Thompson, D. J.; Fichtel, C. E.; Kniffen, D. A.; Ogelman, H. B.; Lamb, R. C.

1976-01-01

Evidence is reported for the observation of gamma-ray emission from the radio pulsar PSR 1747-46 by the gamma-ray telescope aboard SAS 2. The evidence is based on the presence of both an approximately 3-sigma enhancement of gamma rays at the pulsar's location and an approximately 4-sigma peak in the phase plot of 79 gamma-ray events whose phase was calculated from the pulsar's known period. The gamma-ray pulsation is found to appear at a phase lag of about 0.16 from that predicted by the radio observations. The pulsed gamma-ray fluxes above 35 MeV and 100 MeV are estimated, and it is shown that the gamma-ray pulse width is similar to the radio pulse width. It is concluded that PSR 1747-46 is a most likely candidate for pulsed gamma-ray emission.

Cognitive, Behavioral, and Adaptive Functioning in Fragile X and Non-Fragile X Retarded Men.

ERIC Educational Resources Information Center

Dykens, Elisabeth; And Others

1988-01-01

Evaluation of the cognitive, behavioral, and adaptive functioning of 12 retarded men with fragile X syndrome indicated that fragile X men were largely indistinguishable from comparison groups. They were, however, significantly more likely to have achieved levels of adaptive functioning commensurate with their intellectual abilities. (Author/DB)

Transfer of perceptual adaptation to space sickness: What enhances an individual's ability to adapt?

NASA Technical Reports Server (NTRS)

1993-01-01

The objectives of this project were to explore systematically the determiners of transfer of perceptual adaptation as these principles might apply to the space adaptation syndrome. The perceptual experience of an astronaut exposed to the altered gravitational forces involved in spaceflight shares much with that of the subject exposed in laboratory experiments to optically induced visual rearrangement with tilt and dynamic motion illusions such as vection; and experiences and symptoms reported by the trainee who is exposed to the compellingly realistic visual imagery of flight simulators and virtual reality systems. In both of these cases the observer is confronted with a variety of inter- and intrasensory conflicts that initially disrupt perception, as well as behavior, and also produce symptoms of motion sickness.

[Dent's syndrome. Nephrology follow-up of four patients of the same family].

PubMed

Coulibaly, G; Babinet, F; Champion, G

2012-04-01

Dent's syndrome is a rare inherited tubulopathy. Factors influencing renal function in this disease are not well known. The aim of our study is to investigate the evolution of the Dent's syndrome in renal plan. The study was retrospective and conducted in 2006, concerning four brothers. The genetic defect was a mutation S244L missense in exon 6 of gene CLCN5. Various parameters were studied. Patients were 8.5 to 21-years-old at the beginning of the follow-up. Two of them had chronic renal insufficiency (CRI) which evolved, at least 7 years of moderate to terminal stage. Tubular signs were made of hypokalemia, hypercalciuria, hypophosphatemia and proteinuria mostly β2 microglobulin. Improvement of these abnormalities was obtained with symptomatic treatment which has not always been well tolerated. A case of beginner nephrocalcinosis was observed. There was size and weight delay at the beginning of patient monitoring. Dent's syndrome may be complicated by CRI. It seems to appear in the second decade of life and hypercalciuria would contribute to it. Our CRI patients had significant bone disease. The hypercalciuria and proteinuria are factors over which we try to act. Clinical trials are needed to evaluate the efficacy of treatment on the reduction of CRI or its progression by reducing these factors in patients with Dent's syndrome. Copyright © 2011 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.

Development of a Bayesian response-adaptive trial design for the Dexamethasone for Excessive Menstruation study.

PubMed

Holm Hansen, Christian; Warner, Pamela; Parker, Richard A; Walker, Brian R; Critchley, Hilary Od; Weir, Christopher J

2017-12-01

It is often unclear what specific adaptive trial design features lead to an efficient design which is also feasible to implement. This article describes the preparatory simulation study for a Bayesian response-adaptive dose-finding trial design. Dexamethasone for Excessive Menstruation aims to assess the efficacy of Dexamethasone in reducing excessive menstrual bleeding and to determine the best dose for further study. To maximise learning about the dose response, patients receive placebo or an active dose with randomisation probabilities adapting based on evidence from patients already recruited. The dose-response relationship is estimated using a flexible Bayesian Normal Dynamic Linear Model. Several competing design options were considered including: number of doses, proportion assigned to placebo, adaptation criterion, and number and timing of adaptations. We performed a fractional factorial study using SAS software to simulate virtual trial data for candidate adaptive designs under a variety of scenarios and to invoke WinBUGS for Bayesian model estimation. We analysed the simulated trial results using Normal linear models to estimate the effects of each design feature on empirical type I error and statistical power. Our readily-implemented approach using widely available statistical software identified a final design which performed robustly across a range of potential trial scenarios.

Linking behavioural syndromes and cognition: a behavioural ecology perspective.

PubMed

Sih, Andrew; Del Giudice, Marco

2012-10-05

With the exception of a few model species, individual differences in cognition remain relatively unstudied in non-human animals. One intriguing possibility is that variation in cognition is functionally related to variation in personality. Here, we review some examples and present hypotheses on relationships between personality (or behavioural syndromes) and individual differences in cognitive style. Our hypotheses are based largely on a connection between fast-slow behavioural types (BTs; e.g. boldness, aggressiveness, exploration tendency) and cognitive speed-accuracy trade-offs. We also discuss connections between BTs, cognition and ecologically important aspects of decision-making, including sampling, impulsivity, risk sensitivity and choosiness. Finally, we introduce the notion of cognition syndromes, and apply ideas from theories on adaptive behavioural syndromes to generate predictions on cognition syndromes.

Linking behavioural syndromes and cognition: a behavioural ecology perspective

PubMed Central

Sih, Andrew; Del Giudice, Marco

2012-01-01

With the exception of a few model species, individual differences in cognition remain relatively unstudied in non-human animals. One intriguing possibility is that variation in cognition is functionally related to variation in personality. Here, we review some examples and present hypotheses on relationships between personality (or behavioural syndromes) and individual differences in cognitive style. Our hypotheses are based largely on a connection between fast–slow behavioural types (BTs; e.g. boldness, aggressiveness, exploration tendency) and cognitive speed–accuracy trade-offs. We also discuss connections between BTs, cognition and ecologically important aspects of decision-making, including sampling, impulsivity, risk sensitivity and choosiness. Finally, we introduce the notion of cognition syndromes, and apply ideas from theories on adaptive behavioural syndromes to generate predictions on cognition syndromes. PMID:22927575

Selye's general adaptation syndrome: stress-induced gastro-duodenal ulceration and inflammatory bowel disease.

PubMed

Fink, George

2017-03-01

Hans Selye in a note to Nature in 1936 initiated the field of stress research by showing that rats exposed to nocuous stimuli responded by way of a 'general adaptation syndrome' (GAS). One of the main features of the GAS was the 'formation of acute erosions in the digestive tract, particularly in the stomach, small intestine and appendix'. This provided experimental evidence for the view based on clinical data that gastro-duodenal (peptic) ulcers could be caused by stress. This hypothesis was challenged by Marshall and Warren's Nobel Prize (2005)-winning discovery of a causal association between Helicobacter pylori and peptic ulcers. However, clinical and experimental studies suggest that stress can cause peptic ulceration in the absence of H. pylori Predictably, the etiological pendulum of gastric and duodenal ulceration has swung from 'all stress' to 'all bacteria' followed by a sober realization that both factors play a role, separately as well as together. This raises the question as to whether stress and H. pylori interact, and if so, how? Stress has also been implicated in inflammatory bowel disease (IBD) and related disorders; however, there is no proof yet that stress is the primary etiological trigger for IBD. Central dopamine mechanisms seem to be involved in the stress induction of peptic ulceration, whereas activation of the sympathetic nervous system and central and peripheral corticotrophin-releasing factor appears to mediate stress-induced IBD. © 2017 Society for Endocrinology.

Preparation, characterization and in vivo evaluation of amorphous atorvastatin calcium nanoparticles using supercritical antisolvent (SAS) process.

PubMed

Kim, Min-Soo; Jin, Shun-Ji; Kim, Jeong-Soo; Park, Hee Jun; Song, Ha-Seung; Neubert, Reinhard H H; Hwang, Sung-Joo

2008-06-01

In this work, amorphous atorvastatin calcium nanoparticles were successfully prepared using the supercritical antisolvent (SAS) process. The effect of process variables on particle size and distribution of atorvastatin calcium during particle formation was investigated. Solid state characterization, solubility, intrinsic dissolution, powder dissolution studies and pharmacokinetic study in rats were performed. Spherical particles with mean particle size ranging between 152 and 863 nm were obtained by varying process parameters such as precipitation vessel pressure and temperature, drug solution concentration and feed rate ratio of CO2/drug solution. XRD, TGA, FT-IR, FT-Raman, NMR and HPLC analysis indicated that atorvastatin calcium existed as anhydrous amorphous form and no degradation occurred after SAS process. When compared with crystalline form (unprocessed drug), amorphous atorvastatin calcium nanoparticles were of better performance in solubility and intrinsic dissolution rate, resulting in higher solubility and faster dissolution rate. In addition, intrinsic dissolution rate showed a good correlation with the solubility. The dissolution rates of amorphous atorvastatin calcium nanoparticles were highly increased in comparison with unprocessed drug by the enhancement of intrinsic dissolution rate and the reduction of particle size resulting in an increased specific surface area. The absorption of atorvastatin calcium after oral administration of amorphous atorvastatin calcium nanoparticles to rats was markedly increased.

Phenotypic divergence along geographic gradients reveals potential for rapid adaptation of the White-nose Syndrome pathogen, Pseudogymnoascus destructans, in North America.

PubMed

Forsythe, Adrian; Giglio, Victoria; Asa, Jonathan; Xu, Jianping

2018-06-18

White-nose Syndrome (WNS) is an ongoing epizootic affecting multiple species of North American bats, caused by epidermal infections of the psychrophilic filamentous fungus, Pseudogymnoascus destructans Since its introduction from Europe, WNS has spread rapidly across eastern North America and resulted in high mortality rates in bats. At present, the mechanisms behind its spread and the extent of its adaptation to different geographic and ecological niches remain unknown. The objective of this study was to examine the geographic patterns of phenotypic variation and the potential evidence for adaptation among strains representing broad geographic locations in eastern North America. The morphological features of these strains were evaluated on artificial medium, and the viability of asexual arthroconidia of representative strains were investigated after storage at high (23°C), moderate (14°C), and low (4°C) temperatures at different lengths of times. Our analyses identified evidence for a geographic pattern of colony morphology changes among the clonal descendants of the fungus, with trait values correlated with increased distance from the epicenter of WNS. Our genomic comparisons of three representative isolates revealed novel genetic polymorphisms and suggested potential candidate mutations that might be related to some of the phenotypic changes. These results show that even though this pathogen arrived in North America only recently and reproduces asexually, there has been substantial evolution and phenotypic diversification during its rapid clonal expansion. Importance The causal agent of White-nose Syndrome in bats is Pseudogymnoascus destructans , a filamentous fungus recently introduced from its native range in Europe. Infections caused by P. destructans have progressed across the eastern parts of Canada and the United States over the last ten years. It is not clear how the disease is spread as the pathogen is unable to grow above 23°C and ambient

Using the sun analog sensor (SAS) data to investigate solar array yoke motion on the GOES-8 and -9 spacecraft

NASA Astrophysics Data System (ADS)

Phenneger, Milton; Knack, Jennifer L.

1996-10-01

The GOES-8 and -9 Sun analog sensor (SAS) flight data is analyzed to evaluate the attitude motion environment of payloads mounted on the solar array. The work was performed in part to extend analysis in progress to support the solar x-ray imager to be flown on the GOES-M. The SAS is a two axis sensor mounted on the x-ray sensor pointing (XRP) module to measure the east/west error angle between the SUn and the solar array normal and to provide a north south error angle for automatic solar pointing of the x-ray sensor by the XRP. The goal was to search for evidence of solar array vibrational modes in the 2 Hz and 0.5 Hz range and to test the predicted amplitudes. The results show that the solar array rotates at the rate of the mean Sun with unexpected oscillation periods of 5.6 minutes, 90 minutes, and 1440 minutes originating from the two 16.1 gear drive train stages between the solar array drive stepper motor and the solar array yoke. The higher frequency oscillations are detected as random noise at the 1/16 Hz telemetry sampling rate of the SAS. This supports the preflight predictions for the high frequency modes but provide s no detailed measurement of the frequency as expected for this data period. In addition to this the data indicates that the solar array is responding unexpectedly to GOES imager instrument blackbody calibration events.

Adaptive decoding of convolutional codes

NASA Astrophysics Data System (ADS)

Hueske, K.; Geldmacher, J.; Götze, J.

2007-06-01

Convolutional codes, which are frequently used as error correction codes in digital transmission systems, are generally decoded using the Viterbi Decoder. On the one hand the Viterbi Decoder is an optimum maximum likelihood decoder, i.e. the most probable transmitted code sequence is obtained. On the other hand the mathematical complexity of the algorithm only depends on the used code, not on the number of transmission errors. To reduce the complexity of the decoding process for good transmission conditions, an alternative syndrome based decoder is presented. The reduction of complexity is realized by two different approaches, the syndrome zero sequence deactivation and the path metric equalization. The two approaches enable an easy adaptation of the decoding complexity for different transmission conditions, which results in a trade-off between decoding complexity and error correction performance.

Scale Adaptive Simulation Model for the Darrieus Wind Turbine

NASA Astrophysics Data System (ADS)

Rogowski, K.; Hansen, M. O. L.; Maroński, R.; Lichota, P.

2016-09-01

Accurate prediction of aerodynamic loads for the Darrieus wind turbine using more or less complex aerodynamic models is still a challenge. One of the problems is the small amount of experimental data available to validate the numerical codes. The major objective of the present study is to examine the scale adaptive simulation (SAS) approach for performance analysis of a one-bladed Darrieus wind turbine working at a tip speed ratio of 5 and at a blade Reynolds number of 40 000. The three-dimensional incompressible unsteady Navier-Stokes equations are used. Numerical results of aerodynamic loads and wake velocity profiles behind the rotor are compared with experimental data taken from literature. The level of agreement between CFD and experimental results is reasonable.

Mother-child play: children with Down syndrome and typical development.

PubMed

Venuti, P; de Falco, S; Esposito, G; Bornstein, Marc H

2009-07-01

Child solitary and collaborative mother-child play with 21 children with Down syndrome and 33 mental-age-matched typically developing children were compared. In solitary play, children with Down syndrome showed less exploratory but similar symbolic play compared to typically developing children. From solitary to collaborative play, children with Down syndrome increased their exploratory play, attaining the same level as typically developing children. Pretense significantly increased from solitary to collaborative play only in typically developing children. Differences between mothers' play in the two groups mirrored those between their children. Both groups showed similar attunement and synchrony. Mothers contribute to the play development of children with Down syndrome through their own adaptation to their children's limitations and potentialities.

Interferon alpha inhibits replication of a live-attenuated porcine reproductive and respiratory syndrome virus vaccine preventing development of an adaptive immune response in swine.

PubMed

Brockmeier, Susan L; Loving, Crystal L; Eberle, Kirsten C; Hau, Samantha J; Buckley, Alexandra; Van Geelen, Albert; Montiel, Nestor A; Nicholson, Tracy; Lager, Kelly M

2017-12-01

Type I interferons, such as interferon alpha (IFN-α), contribute to innate antiviral immunity by promoting production of antiviral mediators and are also involved in promoting an adaptive immune response. Porcine reproductive and respiratory syndrome virus (PRRSV) is one of the most devastating and costly viruses to the swine industry world-wide and has been shown to induce a meager IFN-α response. Previously we administered porcine IFN-α using a replication-defective adenovirus vector (Ad5-IFN-α) at the time of challenge with virulent PRRSV and demonstrated an increase in the number of virus-specific IFNγ secreting cells, indicating that the presence of IFN-α at the time of infection can alter the adaptive immune responses to PRRSV. In the current experiment, we explored the use of IFN-α as an adjuvant administered with live-attenuated PRRSV vaccine as a method to enhance immune response to the vaccine. Unlike the previous studies with fully virulent virus, one injection of the Ad5-IFN-α abolished replication of the vaccine virus and as a result there was no detectible adaptive immune response. Although IFN-α did not have the desired adjuvant effect, the results further highlight the use of IFN-α as a treatment for PRRSV infection. Published by Elsevier B.V.

[Quality of oocytes and embryos from women with polycystic ovaries syndrome: State of the art].

PubMed

Fournier, A; Torre, A; Delaroche, L; Gala, A; Mullet, T; Ferrières, A; Hamamah, S

The frequency of polycystic ovary syndrome (PCOS) and the consequent fertility disorders cause many difficulties in the management of the assisted reproductive technics. Some studies are focused on different additional treatments, stimulation protocols or techniques that could optimize the in vitro fertilization cycles. The quality of the oocytes and embryos of these patients is also an outstanding issue. They remain difficult to actually evaluate during management, and none of the few published studies on this subject demonstrated any inferiority, compared to control patients. However, many differences have been highlighted, studying intra- and extra-ovarian factors. The advent of new genetic techniques could allow a better understanding of the pathophysiological mechanisms of the syndrome, as well as refining the evaluation of oocytes and embryos, in order to better predict the results of in vitro fertilization attempts. Pregnancy and birth rates, however, appear to be comparable to those of the general population. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Variability of the Aging Process in Dementia-Free Adults with Down Syndrome

ERIC Educational Resources Information Center

Tsao, Raphaele; Kindelberger, Cecile; Freminville, Benedicte; Touraine, Renaud; Bussey, Gerald

2015-01-01

The aim of this cross-sectional study was to analyze the typical aging process in adults with Down syndrome, focusing on its variability. The sample comprised 120 adults with Down syndrome who were free of dementia. Ages ranged from 20 to 69 years. Each participant was assessed on cognitive functioning and social adaptation, and was checked for…

Method selection and adaptation for distributed monitoring of infectious diseases for syndromic surveillance.

PubMed

Xing, Jian; Burkom, Howard; Tokars, Jerome

2011-12-01

Automated surveillance systems require statistical methods to recognize increases in visit counts that might indicate an outbreak. In prior work we presented methods to enhance the sensitivity of C2, a commonly used time series method. In this study, we compared the enhanced C2 method with five regression models. We used emergency department chief complaint data from US CDC BioSense surveillance system, aggregated by city (total of 206 hospitals, 16 cities) during 5/2008-4/2009. Data for six syndromes (asthma, gastrointestinal, nausea and vomiting, rash, respiratory, and influenza-like illness) was used and was stratified by mean count (1-19, 20-49, ≥50 per day) into 14 syndrome-count categories. We compared the sensitivity for detecting single-day artificially-added increases in syndrome counts. Four modifications of the C2 time series method, and five regression models (two linear and three Poisson), were tested. A constant alert rate of 1% was used for all methods. Among the regression models tested, we found that a Poisson model controlling for the logarithm of total visits (i.e., visits both meeting and not meeting a syndrome definition), day of week, and 14-day time period was best. Among 14 syndrome-count categories, time series and regression methods produced approximately the same sensitivity (<5% difference) in 6; in six categories, the regression method had higher sensitivity (range 6-14% improvement), and in two categories the time series method had higher sensitivity. When automated data are aggregated to the city level, a Poisson regression model that controls for total visits produces the best overall sensitivity for detecting artificially added visit counts. This improvement was achieved without increasing the alert rate, which was held constant at 1% for all methods. These findings will improve our ability to detect outbreaks in automated surveillance system data. Published by Elsevier Inc.

Re-examining the role of Drosophila Sas-4 in centrosome assembly using two-colour-3D-SIM FRAP

PubMed Central

Conduit, Paul T; Wainman, Alan; Novak, Zsofia A; Weil, Timothy T; Raff, Jordan W

2015-01-01

Centrosomes have many important functions and comprise a ‘mother’ and ‘daughter’ centriole surrounded by pericentriolar material (PCM). The mother centriole recruits and organises the PCM and templates the formation of the daughter centriole. It has been reported that several important Drosophila PCM-organising proteins are recruited to centrioles from the cytosol as part of large cytoplasmic ‘S-CAP’ complexes that contain the centriole protein Sas-4. In a previous paper (Conduit et al., 2014b) we showed that one of these proteins, Cnn, and another key PCM-organising protein, Spd-2, are recruited around the mother centriole before spreading outwards to form a scaffold that supports mitotic PCM assembly; the recruitment of Cnn and Spd-2 is dependent on another S-CAP protein, Asl. We show here, however, that Cnn, Spd-2 and Asl are not recruited to the mother centriole as part of a complex with Sas-4. Thus, PCM recruitment in fly embryos does not appear to require cytosolic S-CAP complexes. DOI: http://dx.doi.org/10.7554/eLife.08483.001 PMID:26530814

Seizures and pain uncertainty associated with parenting stress and Rett syndrome.

PubMed

Byiers, Breanne J; Tervo, Raymond C; Feyma, Timothy J; Symons, Frank J

2014-04-01

Data were collected parenting stress, adaptive behavior, pain, and health issues from the caregivers of 35 girls and women with Rett syndrome (mean age = 20.3). A majority (60%) of parents reported stress in the clinical range on at least 1 subscale of the Parenting Stress Index-Short Form. Seizures and uncertainty about their daughter's gastrointestinal pain experience were significantly associated with higher levels of parenting stress. No other child factors (adaptive behavior, age, residential status) were significantly related to parenting stress. Factors related to chronic health concerns (seizures, ambiguous pain presentation) may be important when considering family stress issues in relation to general outcomes for girls with Rett syndrome and related developmental disorders.

[Calculating Pearson residual in logistic regressions: a comparison between SPSS and SAS].

PubMed

Xu, Hao; Zhang, Tao; Li, Xiao-song; Liu, Yuan-yuan

2015-01-01

To compare the results of Pearson residual calculations in logistic regression models using SPSS and SAS. We reviewed Pearson residual calculation methods, and used two sets of data to test logistic models constructed by SPSS and STATA. One model contained a small number of covariates compared to the number of observed. The other contained a similar number of covariates as the number of observed. The two software packages produced similar Pearson residual estimates when the models contained a similar number of covariates as the number of observed, but the results differed when the number of observed was much greater than the number of covariates. The two software packages produce different results of Pearson residuals, especially when the models contain a small number of covariates. Further studies are warranted.

Diagnosis and treatment of movement system impairment syndromes.

PubMed

Sahrmann, Shirley; Azevedo, Daniel C; Dillen, Linda Van

Diagnoses and treatments based on movement system impairment syndromes were developed to guide physical therapy treatment. This masterclass aims to describe the concepts on that are the basis of the syndromes and treatment and to provide the current research on movement system impairment syndromes. The conceptual basis of the movement system impairment syndromes is that sustained alignment in a non-ideal position and repeated movements in a specific direction are thought to be associated with several musculoskeletal conditions. Classification into movement system impairment syndromes and treatment has been described for all body regions. The classification involves interpreting data from standardized tests of alignments and movements. Treatment is based on correcting the impaired alignment and movement patterns as well as correcting the tissue adaptations associated with the impaired alignment and movement patterns. The reliability and validity of movement system impairment syndromes have been partially tested. Although several case reports involving treatment using the movement system impairment syndromes concept have been published, efficacy of treatment based on movement system impairment syndromes has not been tested in randomized controlled trials, except in people with chronic low back pain. Copyright © 2017 Associação Brasileira de Pesquisa e Pós-Graduação em Fisioterapia. Publicado por Elsevier Editora Ltda. All rights reserved.

Embolization of ovarian vein for pelvic congestion syndrome with ethylene vinyl alcohol copolymer (Onyx®).

PubMed

Marcelin, C; Izaaryene, J; Castelli, M; Barral, P A; Jacquier, A; Vidal, V; Bartoli, J M

2017-12-01

To evaluate the safety and efficacy of pelvic embolization using ethylene vinyl alcohol copolymer (Onyx ® ) for pelvic congestion syndrome. Between March 2012 to September 2016, 17 women (mean age, 44.7± 12.2 (SD) years; range: 34-71years) presenting with pelvic congestion syndrome were evaluated for transvenous embolization with Onyx ® . Pelvic congestion syndrome was initially diagnosed by clinical examination and the results of transvaginal Doppler ultrasound and further confirmed by pelvic venography. Primary and secondary clinical efficacy was defined respectively by the resolution of the symptoms after embolization and at the end of the follow-up, irrespective to the number of embolization procedures. Technical efficacy of embolization was 100% with no significant complications during and after embolization. After a mean follow-up time of 24.2 months (range: 6-69months) a primary and secondary clinical efficacy of 76.4% (13/17 women) and 94.1% (16/17 women) respectively were observed. Four women (23.5%) underwent a second embolization procedure with one woman requiring a third embolization procedure. These additional embolization procedures were associated with direct puncture of vulvar varices for sclerotherapy in two women. Five women (29%) had recurrent symptoms 21 months post-treatment (7-42months). Pelvic embolization using ethylene vinyl alcohol copolymer (Onyx ® ) has a favorable clinical success for pelvic congestion syndrome. Copyright © 2017. Published by Elsevier Masson SAS.

Development, content validity, and cross-cultural adaptation of a patient-reported outcome measure for real-time symptom assessment in irritable bowel syndrome.

PubMed

Vork, L; Keszthelyi, D; Mujagic, Z; Kruimel, J W; Leue, C; Pontén, I; Törnblom, H; Simrén, M; Albu-Soda, A; Aziz, Q; Corsetti, M; Holvoet, L; Tack, J; Rao, S S; van Os, J; Quetglas, E G; Drossman, D A; Masclee, A A M

2018-03-01

End-of-day questionnaires, which are considered the gold standard for assessing abdominal pain and other gastrointestinal (GI) symptoms in irritable bowel syndrome (IBS), are influenced by recall and ecological bias. The experience sampling method (ESM) is characterized by random and repeated assessments in the natural state and environment of a subject, and herewith overcomes these limitations. This report describes the development of a patient-reported outcome measure (PROM) based on the ESM principle, taking into account content validity and cross-cultural adaptation. Focus group interviews with IBS patients and expert meetings with international experts in the fields of neurogastroenterology & motility and pain were performed in order to select the items for the PROM. Forward-and-back translation and cognitive interviews were performed to adapt the instrument for the use in different countries and to assure on patients' understanding with the final items. Focus group interviews revealed 42 items, categorized into five domains: physical status, defecation, mood and psychological factors, context and environment, and nutrition and drug use. Experts reduced the number of items to 32 and cognitive interviewing after translation resulted in a few slight adjustments regarding linguistic issues, but not regarding content of the items. An ESM-based PROM, suitable for momentary assessment of IBS symptom patterns was developed, taking into account content validity and cross-cultural adaptation. This PROM will be implemented in a specifically designed smartphone application and further validation in a multicenter setting will follow. © 2017 John Wiley & Sons Ltd.

Shwachman-Diamond syndrome with autoimmune-like liver disease and enteropathy mimicking celiac disease.

PubMed

Veropalumbo, Claudio; Campanozzi, Angelo; De Gregorio, Fabiola; Correra, Antonio; Raia, Valeria; Vajro, Pietro

2015-02-01

Liver abnormalities that normalize during infancy as well an enteropathy are reported in Shwachman-Diamond syndrome (SDS). The pathogenesis of both conditions is unknown. We report two SDS cases with autoimmune-like (antismooth muscle and/or antinuclear antibody positivity) liver disease and antigliadin antibody positive inflammatory enteropathy. Hypertransaminasemia did not resolve after immunosuppressive therapy and/or a gluten-free diet. These transient autoimmune phenomena and gut-liver axis perturbations may have played a role in transient SDS hepatopathy and enteropathy. Our report may stimulate other studies to define the relationship between the SDS genetic defect and intestinal permeability as the pathogenic mechanism underlying SDS related liver and intestinal inflammation. Copyright © 2014. Published by Elsevier Masson SAS.

Psychosocial Aspects of the Sudden Infant Death Syndrome ("Cot Death").

ERIC Educational Resources Information Center

Bluglass, Kerry

1981-01-01

Reviews literature on reactions of parents and siblings to Sudden Infant Death Syndrome (SIDS). The prospects for prolonged, adverse reactions are considered, and professional concerns regarding abnormal adaptation are noted. (Author/DB)

[Acute respiratory distress syndrome in childhood: Changing definition and news from the Pediatric Consensus Conference].

PubMed

Dauger, S; Le Bourgeois, F; Guichoux, J; Brissaud, O

2017-05-01

Acute respiratory distress syndrome (ARDS) is a rapidly progressive hypoxemic respiratory insufficiency induced by alveolar filling mainly caused by alveolocapillary wall disruption, following direct or indirect pulmonary injury. Much less frequent in children than in adults, pediatric intensivists had long applied adult guidelines to their daily practice. In 2015, experts from the Pediatric Acute Lung Injury Consensus Conference (PALICC) published the first international guidelines specifically dedicated to pediatric ARDS. After a short summary of the history of the ARDS definition since its first report in 1967, we describe the main diagnostic and therapeutic guidelines for PALICC. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Effect of dietary fiber and growth hormone on colonic adaptation in short bowel syndrome treated by enteral nutrition.

PubMed

Xu, Jianmin; Zhong, Yunshi; Jin, Dayong; Zhang, Hongwei; Wu, Zhaohan

2008-08-01

Colon adaptation can partially compensate for the reduced capacity of nutrient absorption in patients with short bowel syndrome (SBS). The aim of this study was to assess the effect of combined treatment with enteral nutrition (EN), dietary fiber, and recombinant human growth hormone (rhGH) on promoting colonic adaptation. A group of 40 male Sprague-Dawley rats undergoing up to 80% to 85% small intestine resection were randomly assigned to four groups of 10 rats each: enteral nutrition (EN, the control); enteral nutrition/dietary fiber (EF); enteral nutrition/rhGH (EG); and enteral nutrition/dietary fiber/rhGH (EFG). All groups received isonitrogenous, isocaloric enteral feeding for 21 days. Body weight, daily nitrogen balance, colonic morphology, DNA, insulin-like growth factor-1/IGF-1 receptor (IGF-1)/IGF-1R) expression were determined. Morphologic adaptation of the colon (including increased mucosal thickness and plica height, enlarged surface area, increased hyditloid cells) was observed on postoperative day 21. GH is superior to fiber in several aspects: increasing colon diameters (0.46 +/- 0.03 vs. 0.38 +/- 0.02 cm, P < 0.05), villous height (356 +/- 23 vs. 307 +/- 21 microm, P < 0.05) and total surface area (15,222 +/- 1344 vs. 13,178 +/- 1727 microm(2), P < 0.05). Increased DNA content-1.66 +/- 0.13 (EG) and 1.71 +/- 0.13 (EGF) vs. 1.28 +/- 0.11(EF), P < 0.05-in the colon was also found in the EG and EGF groups. GH administration led to a significant increase in plasma IGF-1 (439.6 +/- 88.3 ng/ml in the EG group, 455.4 +/- 107.4 ng/ml in the EGF group) and growth hormone (9.29 +/- 6.49 ng/ml in the EG group, 9.68 +/- 3.26 ng/ml in the EGF group) as compared to the EN group (IGF-1, 328.7 +/- 68.1 ng/ml; GH, 5.81 +/- 2.41 ng/ml) and the EF group (IGF-1, 356.4 +/- 52.1 ng/ml; GH, 6.51 +/- 4.66 ng/ml). Analysis of IGF-1 and IGF-1 receptor mRNA also demonstrated a significantly higher IGF-1 mRNA in the EG and EFG groups than in the EN and EF groups. Colon

Children with 7q11.23 Duplication Syndrome: Psychological Characteristics

PubMed Central

Mervis, Carolyn B.; Klein-Tasman, Bonita P.; Huffman, Myra J.; Velleman, Shelley L.; Pitts, C. Holley; Henderson, Danielle R.; Woodruff-Borden, Janet; Morris, Colleen A.; Osborne, Lucy R.

2015-01-01

To begin to delineate the psychological characteristics associated with classic 7q11.23 duplication syndrome (duplication of the classic Williams syndrome region; hereafter classic Dup7), we tested 63 children with classic Dup7 aged 4–17 years. Sixteen toddlers aged 18–45 months with classic Dup7 and 12 adults identified by cascade testing also were assessed. For the child group, median General Conceptual Ability (similar to IQ) on the Differential Ability Scales-II was 85.0 (low average), with a range from severe disability to high average ability. Median reading and mathematics achievement standard scores were at the low average to average level, with a range from severe impairment to high average or superior ability. Adaptive behavior was considerably more limited; median Scales of Independent Behavior—Revised Broad Independence standard score was 62.0 (mild impairment), with a range from severe adaptive impairment to average adaptive ability. Anxiety disorders were common, with 50.0% of children diagnosed with Social Phobia, 29.0% with Selective Mutism, 12.9% with Separation Anxiety Disorder, and 53.2% with Specific Phobia. In addition, 35.5% were diagnosed with Attention Deficit/Hyperactivity Disorder and 24.2% with Oppositional Defiant Disorder or Disruptive Behavior Disorder-Not Otherwise Specified. 33.3% of the children screened positive for a possible Autism Spectrum Disorder and 82.3% were diagnosed with Speech Sound Disorder. We compare these findings to previously reported results for children with Williams syndrome and argue that genotype/phenotype studies involving the Williams syndrome region offer important opportunities to understand the contribution of genes in this region to common disorders affecting the general population. PMID:25900101

Children with 7q11.23 duplication syndrome: psychological characteristics.

PubMed

Mervis, Carolyn B; Klein-Tasman, Bonita P; Huffman, Myra J; Velleman, Shelley L; Pitts, C Holley; Henderson, Danielle R; Woodruff-Borden, Janet; Morris, Colleen A; Osborne, Lucy R

2015-07-01

To begin to delineate the psychological characteristics associated with classic 7q11.23 duplication syndrome (duplication of the classic Williams syndrome region; hereafter classic Dup7), we tested 63 children with classic Dup7 aged 4-17 years. Sixteen toddlers aged 18-45 months with classic Dup7 and 12 adults identified by cascade testing also were assessed. For the child group, median General Conceptual Ability (similar to IQ) on the Differential Ability Scales-II was 85.0 (low average), with a range from severe disability to high average ability. Median reading and mathematics achievement standard scores were at the low average to average level, with a range from severe impairment to high average or superior ability. Adaptive behavior was considerably more limited; median Scales of Independent Behavior-Revised Broad Independence standard score was 62.0 (mild impairment), with a range from severe adaptive impairment to average adaptive ability. Anxiety disorders were common, with 50.0% of children diagnosed with Social Phobia, 29.0% with Selective Mutism, 12.9% with Separation Anxiety Disorder, and 53.2% with Specific Phobia. In addition, 35.5% were diagnosed with Attention Deficit/Hyperactivity Disorder and 24.2% with Oppositional Defiant Disorder or Disruptive Behavior Disorder-Not Otherwise Specified. 33.3% of the children screened positive for a possible Autism Spectrum Disorder and 82.3% were diagnosed with Speech Sound Disorder. We compare these findings to previously reported results for children with Williams syndrome and argue that genotype/phenotype studies involving the Williams syndrome region offer important opportunities to understand the contribution of genes in this region to common disorders affecting the general population. © 2015 Wiley Periodicals, Inc.

Adaptive Behaviour in Angelman Syndrome: Its Profile and Relationship to Age

ERIC Educational Resources Information Center

Gasca, C. Brun; Obiols, J. E.; Bonillo, A.; Artigas, J.; Lorente, I.; Gabau, E.; Guitart, M.; Turk, J.

2010-01-01

Background: Angelman syndrome (AS) is a neurodevelopmental disorder usually caused by an anomaly in the maternally inherited chromosome 15. The main features are severe intellectual disability, speech impairment, ataxia, epilepsy, sleep disorder and a behavioural phenotype that reportedly includes happy disposition, attraction to/fascination with…

Using SAS functions and high-resolution isotope data to unravel travel time distributions in headwater catchments

NASA Astrophysics Data System (ADS)

Benettin, Paolo; Soulsby, Chris; Birkel, Christian; Tetzlaff, Doerthe; Botter, Gianluca; Rinaldo, Andrea

2017-03-01

We use high-resolution tracer data from an experimental site to test theoretical approaches that integrate catchment-scale flow and transport processes in a unified framework centered on selective age sampling by streamflow and evapotranspiration fluxes. Transport processes operating at the catchment scale are reflected in the evolving residence time distribution of the catchment water storage and in the age selection operated by out-fluxes. Such processes are described here through StorAge Selection (SAS) functions parameterized as power laws of the normalized rank storage. Such functions are computed through appropriate solution of the master equation defining formally the evolution of residence and travel times. By representing the way in which catchment storage generates outflows composed by water of different ages, the main mechanism regulating the tracer composition of runoff is clearly identified and detailed comparison with empirical data sets are possible. Properly calibrated numerical tools provide simulations that convincingly reproduce complex measured signals of daily deuterium content in stream waters during wet and dry periods. Results for the catchment under consideration are consistent with other recent studies indicating a tendency for natural catchments to preferentially release younger available water. The study shows that power law SAS functions prove a powerful tool to explain catchment-scale transport processes that also has potential in less intensively monitored sites.

Head Injury and the Post-Trauma Vision Syndrome.

ERIC Educational Resources Information Center

Padula, William V.; Shapiro, Jannie B.

1993-01-01

This article considers the visual rehabilitation of patients recovering from traumatic brain injuries. Characteristics, symptoms, and associated neuromotor difficulties of posttrauma vision syndrome are listed, as are common posture and gait adaptations. A neuro-optometric rehabilitative and orientation/mobility evaluation is recommended, as is…

Interleukin-25: a cytokine linking eosinophils and adaptive immunity in Churg-Strauss syndrome.

PubMed

Terrier, Benjamin; Bièche, Ivan; Maisonobe, Thierry; Laurendeau, Ingrid; Rosenzwajg, Michèlle; Kahn, Jean-Emmanuel; Diemert, Marie-Claude; Musset, Lucile; Vidaud, Michel; Sène, Damien; Costedoat-Chalumeau, Nathalie; Le Thi-Huong, Du; Amoura, Zahir; Klatzmann, David; Cacoub, Patrice; Saadoun, David

2010-11-25

Churg-Strauss syndrome (CSS) is characterized by systemic vasculitis and blood and tissue eosinophilia. Blood eosinophilia correlates with disease activity, and activated T cells from CSS patients are predominantly T helper 2 (Th2). Interleukin (IL)-25 has been shown to link innate and adaptive immunity by enhancing Th2 cytokine production. We sought to determine the involvement of IL-25 and its receptor IL-17RB in the pathogenesis of CSS. We found increased levels of IL-25 in the serum of active CSS patients (952 ± 697 vs 75 ± 49 pg/mL in inactive patients and 47 ± 6 pg/mL in healthy donors). IL-25 was correlated with disease activity and eosinophil level. Eosinophils were the main source of IL-25, whereas activated CD4(+) memory T cells were the IL-17RB-expressing cells in CSS. IL-25 enhanced the production of IL-4, IL-5, and IL-13 by activated peripheral blood mononuclear cells. IL-25 and IL-17RB were observed within the vasculitic lesions of patients with CSS, and IL-17RB colocalized with T cells. Increased expression of IL-17RB, tumor necrosis factor receptor-associated factor 6, and JunB in vasculitic lesions of CSS underscored the IL-25-mediated activation, whereas up-regulation of GATA3 and IL-10 supported Th2 differentiation. Our findings suggest that eosinophils, through the production of IL-25, exert a critical role in promoting Th2 responses in target tissues of CSS.

Microbiota Is Involved in Post-resection Adaptation in Humans with Short Bowel Syndrome.

PubMed

Gillard, Laura; Mayeur, Camille; Robert, Véronique; Pingenot, Isabelle; Le Beyec, Johanne; Bado, André; Lepage, Patricia; Thomas, Muriel; Joly, Francisca

2017-01-01

Short bowel syndrome (SBS) is characterized by severe intestinal malabsorption following restrictive surgery. The objective of this study was to determine the functional contribution of SBS-microbiota after resection. It is well-known that SBS-microbiota displayed specific features with a prevalence of Lactobacillus, a low amount of some anaerobic microbes ( Clostridium leptum ) and an accumulation of fecal lactate in some patients. Patients with jejuno-colonic anastomosis were stratified according to the presence of lactate in their feces and, we observe that the lactate-producing bacteria were predominant in the sub-group of patients accumulating fecal lactate. One case of D-encephalopathy crisis occurred when the D-lactate isoform accumulated in the feces and plasma bicarbonate levels decreased. The fecal sample at the time of the encephalopathy was transferred to germ free rats (SBS-H rats). The SBS-H microbiota conserved some characteristics of the SBS donnor, predominated by lactate-producing bacteria (mainly Lactobacillus ), a low level of lactate-consuming bacteria and undetectable C. leptum . However, lactate did not accumulate in feces of recipient rats and the D-encephalopathy was not reproduced in SBS-H rats. This suggests that the intact small bowel of the recipient rats protected them from lactate accumulation and that D-lactate encephalopathy can occur only in the absence of small intestine. After fecal transfer, we also show that gnotobiotic rats exhibited high levels of circulating GLP-1 and ghrelin, two hormones that are known to be induced in SBS patients. Therefore, the microbiota of SBS is a reservoir of biological signals involved in post-resection adaptation.

Microbiota Is Involved in Post-resection Adaptation in Humans with Short Bowel Syndrome

PubMed Central

Gillard, Laura; Mayeur, Camille; Robert, Véronique; Pingenot, Isabelle; Le Beyec, Johanne; Bado, André; Lepage, Patricia; Thomas, Muriel; Joly, Francisca

2017-01-01

Short bowel syndrome (SBS) is characterized by severe intestinal malabsorption following restrictive surgery. The objective of this study was to determine the functional contribution of SBS-microbiota after resection. It is well-known that SBS-microbiota displayed specific features with a prevalence of Lactobacillus, a low amount of some anaerobic microbes (Clostridium leptum) and an accumulation of fecal lactate in some patients. Patients with jejuno-colonic anastomosis were stratified according to the presence of lactate in their feces and, we observe that the lactate-producing bacteria were predominant in the sub-group of patients accumulating fecal lactate. One case of D-encephalopathy crisis occurred when the D-lactate isoform accumulated in the feces and plasma bicarbonate levels decreased. The fecal sample at the time of the encephalopathy was transferred to germ free rats (SBS-H rats). The SBS-H microbiota conserved some characteristics of the SBS donnor, predominated by lactate-producing bacteria (mainly Lactobacillus), a low level of lactate-consuming bacteria and undetectable C. leptum. However, lactate did not accumulate in feces of recipient rats and the D-encephalopathy was not reproduced in SBS-H rats. This suggests that the intact small bowel of the recipient rats protected them from lactate accumulation and that D-lactate encephalopathy can occur only in the absence of small intestine. After fecal transfer, we also show that gnotobiotic rats exhibited high levels of circulating GLP-1 and ghrelin, two hormones that are known to be induced in SBS patients. Therefore, the microbiota of SBS is a reservoir of biological signals involved in post-resection adaptation. PMID:28469580

Changes in fibroblast mechanostat set point and mechanosensitivity: an adaptive response to mechanical stress in floppy eyelid syndrome.

PubMed

Ezra, Daniel G; Ellis, James S; Beaconsfield, Michèle; Collin, Richard; Bailly, Maryse

2010-08-01

Floppy eyelid syndrome (FES) is an acquired hyperelasticity disorder affecting the upper eyelid. The tarsal plate becomes hyperelastic with a loss of intrinsic rigidity. As a result, the eyelid is subjected to cyclic mechanical stress. This condition was used as a model to investigate changes in dynamic fibroblast contractility in the context of chronic cyclic mechanical stress. Contractile efficiency was investigated in a free-floating, three-dimensional collagen matrix model. Intrinsic cellular force measurements and responses to changes in gel tension were explored using a tensioning culture force monitor (t-CFM). Gene expression differences between cell lines exhibiting differences in contractile phenotype were explored with a genome level microarray platform and RT-PCR. FES tarsal plate fibroblasts (TFs) showed an increased contractile efficiency compared with the control, and t-CFM measurements confirmed a higher intrinsic cellular force at plateau levels. Cyclic stretch/relaxation experiments determined that TFs in FES maintained a functional tensional homeostasis response but with an altered sensitivity, operating around a higher mechanostat set point. Gene expression array and RT-PCR analysis identified V-CAM1 and PPP1R3C as being upregulated in FES TFs. These changes may represent an adaptive response that allows tensional homeostasis to be maintained at the high levels of tissue stress experienced in FES. Gene expression studies point to a role for V-CAM1 and PPP1R3C in mediating changes in the dynamic range of mechanosensitivity of TFs. This work identifies FES as a useful model for the study of adaptive physiological responses to mechanical stress.

Summary of Payload Integration Plan (PIP) for Starlab-1 flight experiment, enclosure 3

NASA Technical Reports Server (NTRS)

Cowings, Patricia S.; Toscano, W.; Kamiya, J.; Miller, N.; Sharp, J.

1988-01-01

The objectives of the Autogenic Feedback Training (AFT) are to: determine if preflight AFT is an effective treatment for space adaptation syndrome (SAS); determine if preflight improvements in motion sickness tolerance can be used to predict crewmembers' success in controlling symptoms in flight; and identify differences and similarities between the physiological data from preflight motion sickness tests and data collected during symptom episodes in space. The goal is to test the AFT on 8 trained and 8 control subjects. At present 2 trained and 2 contol subjects were tested. The testing will continue until the experimental goal of testing 16 individual is reached.

Numerical exploration of non-axisymmetric divertor closure in the small angle slot (SAS) divertor at DIII-D

NASA Astrophysics Data System (ADS)

Frerichs, H.; Schmitz, O.; Covele, B.; Feng, Y.; Guo, H. Y.; Hill, D.

2018-05-01

Numerical simulations of toroidal asymmetries in a tightly baffled small angle slot (SAS) divertor on the DIII-D tokamak show that toroidal asymmetries in divertor closure result in (non-axisymmetric) local onset of detachment within a density window of 10-15% on top of the nominal threshold separatrix density. The SAS divertor is explored at DIII-D for improving access to cold, dissipative/detached divertor conditions. The narrow width of the slot divertor coupled with a small magnetic field line-to-target angle facilitates the buildup of neutral density, thereby increasing radiative and neutrals-related (atoms and molecules) losses in the divertor. Small changes in the strike point location can be expected to have a large impact on divertor conditions. The combination of misaligned slot structure and non-axisymmetric perturbations to the magnetic field configuration causes the strike point to move along the divertor target plate, possibly leaving the divertor slot at some locations. The latter extreme case essentially introduces an opening in the divertor slot from where recycling neutrals can easily escape, and thereby degrade the performance of the slot divertor. Such a strike point dislocation is approximated by a finite gap in the divertor baffle for which 3D edge plasma and neutral gas simulations are performed with the EMC3-EIRENE code.

Projected number of children with isolated spina bifida or down syndrome in England and Wales by 2020.

PubMed

Best, Kate E; Glinianaia, Svetlana V; Lingam, Raghu; Morris, Joan K; Rankin, Judith

2018-05-19

Children with major congenital anomalies often require lifelong access to health and social care services. Estimating future numbers of affected individuals can aid health and social care planning. This study aimed to estimate the number of children aged 0-15 years living with spina bifida or Down syndrome in England and Wales by 2020. Cases of spina bifida and Down syndrome born during 1998-2013 were identified from the Northern Congenital Abnormality Survey and the National Down Syndrome Cytogenetic Register, respectively. The number of infants born with spina bifida during 1998-2019 were estimated by applying the average prevalence rate in the North of England to actual and projected births in England and Wales. Poisson regression was performed to estimate the number of infants born with Down syndrome in England and Wales during 1998-2013 and 2004-2019. The numbers of children aged 0-15 living with spina bifida or Down syndrome in 2014 and in 2020 were then estimated by multiplying year- and age-specific survival estimates by the number of affected births. An estimated 956 children with isolated spina bifida, 623 children with spina bifida and hydrocephalus and 11,592 children with Down syndrome aged 0-15 years will be living in England and Wales by 2020, increases of 7.2%, 12.0% and 12.7% since 2014, respectively. Due to improvements in survival, an increase in population size and changes in maternal age distribution at delivery, we anticipate further increases in the number of children living with spina bifida or Down syndrome by 2020. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

Copeptin in acute coronary syndromes and heart failure management: State of the art and future directions.

PubMed

Schurtz, Guillaume; Lamblin, Nicolas; Bauters, Christophe; Goldstein, Patrick; Lemesle, Gilles

2015-01-01

Over the past two decades, the use of multiple biomarkers has changed cardiovascular disease management. Recently, several trials have assessed the diagnostic and prognostic performances of copeptin, especially in patients with heart failure or acute coronary syndromes. Primary results are interesting, with copeptin looking promising for: the management of patients who present at emergency departments early after chest pain onset and the risk stratification of patients with heart failure. The purpose of this article is to review the data on the place of copeptin in the management of patients with chest pain or heart failure. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

[Absent or hypoplastic thymus: A marker for 22q11.2 microdeletion syndrome in case of polyhydramnios].

PubMed

Lamouroux, A; Mousty, E; Prodhomme, O; Bigi, N; Le Gac, M-P; Letouzey, V; De Tayrac, R; Mares, P

2016-04-01

In prenatal diagnosis of 22q11.2 microdeletion syndrome, without cardiac malformation or multiple associated congenital anomalies, we study the presence of polyhydramnios and its association with thymic dysgenesis. This was a multicenter retrospective observational study. It was performed in two multidisciplinary centers for prenatal diagnosis in the south of France between January 1, 2010 and June 30, 2013. Inclusion criteria were prenatal diagnosis of 22q11.2 deletion syndrome. We excluded from the study any fetus with cardiac malformation or multiple associated congenital anomalies. During the inclusion period, eleven antenatal diagnoses of 22q11.2 microdeletion syndrome have been made. Six cases were excluded: 5 fetuses with cardiac malformation and one with multiple associated congenital anomalies. Therefore, five cases of isolated polyhydramnios were included. All 5 fetuses had a thymic dysgenesis: 3 had a thymic agenesis and 1 thymic hypoplasia diagnosed by sonography and 1 had a thymic agenesis diagnosed by retrospective reading of fetal MRI. When faced with a polyhydramnios, the presence of a thymic dysgenesis should be search for by ultrasound screening and would alert to the possibility of a 22q11.2 microdeletion syndrome. The confirmation of this is diagnosis by amniocentesis would enable improved antenatal support for parents and would enable early implementation of the multidisciplinary neonatal care that is required to avoid serious complications of this syndrome. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

The Educational Experiences of Students with Asperger Syndrome

ERIC Educational Resources Information Center

Tippett, Jennifer

2004-01-01

The Ministry of Education's policy, "Special Education 2000" (SE 2000) was intended to promote a model that ensured all students received an education that involved successful experiences (Ministry of Education, 1996). Students with Asperger Syndrome (AS) often have difficulty adapting to and achieving these successful experiences in…

Personality Traits and Behavioral Syndromes in Differently Urbanized Populations of House Sparrows (Passer domesticus)

PubMed Central

Bókony, Veronika; Kulcsár, Anna; Tóth, Zoltán; Liker, András

2012-01-01

Urbanization creates novel environments for wild animals where selection pressures may differ drastically from those in natural habitats. Adaptation to urban life involves changes in various traits, including behavior. Behavioral traits often vary consistently among individuals, and these so-called personality traits can be correlated with each other, forming behavioral syndromes. Despite their adaptive significance and potential to act as constraints, little is known about the role of animal personality and behavioral syndromes in animals' adaptation to urban habitats. In this study we tested whether differently urbanized habitats select for different personalities and behavioral syndromes by altering the population mean, inter-individual variability, and correlations of personality traits. We captured house sparrows (Passer domesticus) from four different populations along the gradient of urbanization and assessed their behavior in standardized test situations. We found individual consistency in neophobia, risk taking, and activity, constituting three personality axes. On the one hand, urbanization did not consistently affect the mean and variance of these traits, although there were significant differences between some of the populations in food neophobia and risk taking (both in means and variances). On the other hand, both urban and rural birds exhibited a behavioral syndrome including object neophobia, risk taking and activity, whereas food neophobia was part of the syndrome only in rural birds. These results indicate that there are population differences in certain aspects of personality in house sparrows, some of which may be related to habitat urbanization. Our findings suggest that urbanization and/or other population-level habitat differences may not only influence the expression of personality traits but also alter their inter-individual variability and the relationships among them, changing the structure of behavioral syndromes. PMID:22574204

What Do Parents Want?: An Analysis of Education-Related Comments Made by Parents of Children with Different Genetic Syndromes.

ERIC Educational Resources Information Center

Fidler, Deborah J.; Lawson, John E.; Hodapp, Robert M.

2003-01-01

An analysis of educational desires found parents of children with Down syndrome (n=39) wanted changes in speech therapy and reading services, parents of children with Prader-Willi syndrome (n=25) wanted increases in adaptive physical education services, and parents of children with Williams syndrome (n=26) wanted increases in music services and…

Integrative Biological Chemistry Program Includes The Use Of Informatics Tools, GIS And SAS Software Applications

PubMed Central

D’Souza, Malcolm J.; Kashmar, Richard J.; Hurst, Kent; Fiedler, Frank; Gross, Catherine E.; Deol, Jasbir K.; Wilson, Alora

2015-01-01

Wesley College is a private, primarily undergraduate minority-serving institution located in the historic district of Dover, Delaware (DE). The College recently revised its baccalaureate biological chemistry program requirements to include a one-semester Physical Chemistry for the Life Sciences course and project-based experiential learning courses using instrumentation, data-collection, data-storage, statistical-modeling analysis, visualization, and computational techniques. In this revised curriculum, students begin with a traditional set of biology, chemistry, physics, and mathematics major core-requirements, a geographic information systems (GIS) course, a choice of an instrumental analysis course or a statistical analysis systems (SAS) programming course, and then, students can add major-electives that further add depth and value to their future post-graduate specialty areas. Open-sourced georeferenced census, health and health disparity data were coupled with GIS and SAS tools, in a public health surveillance system project, based on US county zip-codes, to develop use-cases for chronic adult obesity where income, poverty status, health insurance coverage, education, and age were categorical variables. Across the 48 contiguous states, obesity rates are found to be directly proportional to high poverty and inversely proportional to median income and educational achievement. For the State of Delaware, age and educational attainment were found to be limiting obesity risk-factors in its adult population. Furthermore, the 2004–2010 obesity trends showed that for two of the less densely populated Delaware counties; Sussex and Kent, the rates of adult obesity were found to be progressing at much higher proportions when compared to the national average. PMID:26191337

Integrative Biological Chemistry Program Includes The Use Of Informatics Tools, GIS And SAS Software Applications.

PubMed

D'Souza, Malcolm J; Kashmar, Richard J; Hurst, Kent; Fiedler, Frank; Gross, Catherine E; Deol, Jasbir K; Wilson, Alora

Wesley College is a private, primarily undergraduate minority-serving institution located in the historic district of Dover, Delaware (DE). The College recently revised its baccalaureate biological chemistry program requirements to include a one-semester Physical Chemistry for the Life Sciences course and project-based experiential learning courses using instrumentation, data-collection, data-storage, statistical-modeling analysis, visualization, and computational techniques. In this revised curriculum, students begin with a traditional set of biology, chemistry, physics, and mathematics major core-requirements, a geographic information systems (GIS) course, a choice of an instrumental analysis course or a statistical analysis systems (SAS) programming course, and then, students can add major-electives that further add depth and value to their future post-graduate specialty areas. Open-sourced georeferenced census, health and health disparity data were coupled with GIS and SAS tools, in a public health surveillance system project, based on US county zip-codes, to develop use-cases for chronic adult obesity where income, poverty status, health insurance coverage, education, and age were categorical variables. Across the 48 contiguous states, obesity rates are found to be directly proportional to high poverty and inversely proportional to median income and educational achievement. For the State of Delaware, age and educational attainment were found to be limiting obesity risk-factors in its adult population. Furthermore, the 2004-2010 obesity trends showed that for two of the less densely populated Delaware counties; Sussex and Kent, the rates of adult obesity were found to be progressing at much higher proportions when compared to the national average.

Adaptive radiotherapy for head and neck cancers: Fact or fallacy to improve therapeutic ratio?

PubMed

Li, Y Q; Tan, J S H; Wee, J T S; Chua, M L K

2018-04-23

Modern standards of precision radiotherapy, primarily driven by the technological advances of intensity modulation and image guidance, have led to increased versatility in radiotherapy planning and delivery. The ability to shape doses around critical normal organs, while simultaneously "painting" boost doses to the tumor have translated to substantial therapeutic gains in head and neck cancer patients. Recently, dose adaptation (or adaptive radiotherapy) has been proposed as a novel concept to enhance the therapeutic ratio of head and neck radiotherapy, facilitated in part by the onset of molecular and functional imaging. These contemporary imaging techniques have enabled visualisation of the spatial molecular architecture of the tumor. Daily cone-beam imaging, besides improving treatment accuracy, offers another unique angle to explore radiomics - a novel high throughput feature extraction and selection workflow, for adapting radiotherapy based on real-time tumor changes. Here, we review the existing evidence of molecular and functional imaging in head and neck cancers, as well as the current application of adaptive radiotherapy in the treatment of this tumor type. We propose that adaptive radiotherapy can be further exploited through a systematic application of molecular and functional imaging, including radiomics, at the different phases of planning and treatment. Copyright © 2018 Société française de radiothérapie oncologique (SFRO). Published by Elsevier SAS. All rights reserved.

Complexity and network dynamics in physiological adaptation: an integrated view.

PubMed

Baffy, György; Loscalzo, Joseph

2014-05-28

Living organisms constantly interact with their surroundings and sustain internal stability against perturbations. This dynamic process follows three fundamental strategies (restore, explore, and abandon) articulated in historical concepts of physiological adaptation such as homeostasis, allostasis, and the general adaptation syndrome. These strategies correspond to elementary forms of behavior (ordered, chaotic, and static) in complex adaptive systems and invite a network-based analysis of the operational characteristics, allowing us to propose an integrated framework of physiological adaptation from a complex network perspective. Applicability of this concept is illustrated by analyzing molecular and cellular mechanisms of adaptation in response to the pervasive challenge of obesity, a chronic condition resulting from sustained nutrient excess that prompts chaotic exploration for system stability associated with tradeoffs and a risk of adverse outcomes such as diabetes, cardiovascular disease, and cancer. Deconstruction of this complexity holds the promise of gaining novel insights into physiological adaptation in health and disease. Published by Elsevier Inc.

Mechanisms of epileptogenesis in pediatric epileptic syndromes: Rasmussen encephalitis, infantile spasms, and febrile infection-related epilepsy syndrome (FIRES).

PubMed

Pardo, Carlos A; Nabbout, Rima; Galanopoulou, Aristea S

2014-04-01

The mechanisms of epileptogenesis in pediatric epileptic syndromes are diverse, and may involve disturbances of neurodevelopmental trajectories, synaptic homeostasis, and cortical connectivity, which may occur during brain development, early infancy, or childhood. Although genetic or structural/metabolic factors are frequently associated with age-specific epileptic syndromes, such as infantile spasms and West syndrome, other syndromes may be determined by the effect of immunopathogenic mechanisms or energy-dependent processes in response to environmental challenges, such as infections or fever in normally-developed children during early or late childhood. Immune-mediated mechanisms have been suggested in selected pediatric epileptic syndromes in which acute and rapidly progressive encephalopathies preceded by fever and/or infections, such as febrile infection-related epilepsy syndrome, or in chronic progressive encephalopathies, such as Rasmussen encephalitis. A definite involvement of adaptive and innate immune mechanisms driven by cytotoxic CD8(+) T lymphocytes and neuroglial responses has been demonstrated in Rasmussen encephalitis, although the triggering factor of these responses remains unknown. Although the beneficial response to steroids and adrenocorticotropic hormone of infantile spasms, or preceding fever or infection in FIRES, may support a potential role of neuroinflammation as pathogenic factor, no definite demonstration of such involvement has been achieved, and genetic or metabolic factors are suspected. A major challenge for the future is discovering pathogenic mechanisms and etiological factors that facilitate the introduction of novel targets for drug intervention aimed at interfering with the disease mechanisms, therefore providing putative disease-modifying treatments in these pediatric epileptic syndromes.

Search for an X-ray identification of a strong gamma-ray source. [sas-3 observations

NASA Technical Reports Server (NTRS)

Lamb, R. C.

1979-01-01

X-rays from Cygnus X-3 were observed during early 1978 with the detectors of the SAS-3 satellite. These observations in conjunction with earlier UHURU and ANS data indicate that the 4.8 hr period of Cygnus X-3 is increasing at the rate of P/P = (5/1 plus or minus 1.3) x 10 to the minus 6 power/1 yr. The sign and magnitude for this change are incompatible with a rotation model for the period and are in reasonable agreement with model predictions for orbital changes associated with mass loss and transfer in a binary system.

Catatonic syndrome: From detection to therapy.

PubMed

Madigand, J; Lebain, P; Callery, G; Dollfus, S

2016-08-01

, catatonic syndrome remains a common entity of which it is important to be aware, especially in case of rapid installation of the symptoms. Diagnostic scales should be used and a lorazepam test should be performed to avoid delaying the diagnosis. Second-line therapy requires further study. This concerns in particular diazepam, anti-NMDA (N-methyl-D-aspartate) and rTMS (repetitive transcranial magnetic stimulation). Some specificities of catatonia, such as the periodic form and cases in patients with schizophrenia, also require further evaluations. Copyright © 2016 L’Encéphale, Paris. Published by Elsevier Masson SAS. All rights reserved.

Total physical activity volume, physical activity intensity, and metabolic syndrome: 1999-2004 National Health and Nutrition Examination Survey.

PubMed

Churilla, James R; Fitzhugh, Eugene C

2012-02-01

This study examined the association of total physical activity volume (TPAV) and physical activity (PA) from three domains [leisure-time physical activity (LTPA), domestic, transportation] with metabolic syndrome. We also investigated the relationship between LTPA intensity and metabolic syndrome risk. Sample included adults who participated in the 1999-2004 National Health and Nutrition Examination Survey. Physical activity measures were created for TPAV, LTPA, domestic PA, and transportational PA. For each, a six-level measure based upon no PA (level 1) and quintiles (levels 2-6) of metabolic equivalents (MET)·min·wk(-1) was created. A three-level variable associated with the current Department of Health and Human Services (DHHS) PA recommendation was also created. SAS and SUDAAN were used for the statistical analysis. Adults reporting the greatest volume of TPAV and LTPA were found to be 36% [odds ratio (OR) 0.64; 95% confidence interval (CI) 0.49-0.83] and 42% (OR 0.58; 95% CI 0.43-0.77), respectively, less likely to have metabolic syndrome. Domestic and transportational PA provided no specific level of protection from metabolic syndrome. Those reporting a TPAV that met the DHHS PA recommendation were found to be 33% (OR 0.67; 95%; CI 0.55-0.83) less likely to have metabolic syndrome compared to their sedentary counterparts. Adults reporting engaging in only vigorous-intensity LTPA were found to be 37% (OR 0.63; 95 CI 0.42-0.96) to 56% (OR 0.44; 95% CI 0.29-0.67) less likely to have metabolic syndrome. Volume, intensity, and domain of PA may all play important roles in reducing the prevalence and risk of metabolic syndrome.

How does an animal behave like a plant? Physiological and molecular adaptations of zooxanthellae and their hosts to symbiosis.

PubMed

Allemand, Denis; Furla, Paola

2018-04-09

Cnidarians (corals and sea anemones) harbouring photosynthetic microalgae derive several benefits from their association. To allow this association, numerous symbiotic-dependent adaptations in both partners, resulting from evolutionary pressures, have been selected. The dinoflagellate symbionts (zooxanthellae) are located inside a vesicle in the cnidarian host cell and are therefore exposed to a very different environment compared to the free-living state of these microalgae in terms of ion concentration and carbon content and speciation. In addition, this intracellular localization imposes that they rely completely upon the host for their nutrient supply (nitrogen, CO 2 ). Symbiotic-dependent adaptations imposed to the animal host by phototrophic symbiosis are more relevant to photosynthetic organisms than to metazoans: indeed, the cnidarian host often harbours diurnal changes of morphology to adapt itself to the amount of light and possesses carbon-concentrating mechanisms, antioxidative defences and UV sunscreens similar to that present in phototrophs. These adaptations and the contrasting fragility of the association are discussed from both ecological and evolutionary points of view. Copyright © 2018 Académie des sciences. Published by Elsevier Masson SAS. All rights reserved.

Health and Social Outcomes in Adults with Williams Syndrome: Findings from Cross-Sectional and Longitudinal Cohorts

ERIC Educational Resources Information Center

Elison, Sarah; Stinton, Chris; Howlin, Patricia

2010-01-01

Previous studies have investigated trajectories of cognitive, language and adaptive functioning in Williams syndrome (WS) but little is known about how other aspects of the Williams syndrome behavioural phenotype change across the life-span. Therefore, the present study examined age associated changes in a number of different domains of…

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

PubMed

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.

Forestier syndrome and obstructive sleep apnea: Surgical treatment.

PubMed

Ribeiro, D K; Pinto, J A; Freitas, G S

2018-01-20

We report a case of obstructive sleep apnea that occurred as a result of Forestier disease and describe the surgical treatment that was performed. The patient is a 56-year-old man who presented dysphagia for solids and liquids, snoring (score 10) and excessive daytime sleepiness for 5 years. On fiber optic laryngoscopy examination, there was interarytenoid edema and protrusion of the posterior wall of the larynx. The cervical X-Ray showed protrusion of intervertebral disc between C3-C5 (skeletal hyperostosis) and the polysomnography revealed apnea-hypopnea index (AHI) of 56 events/h. Surgery was performed by the otorhinolaryngology and orthopedic teams. The patient evolved with complete symptom resolution and an AHI of 3,9 events/h on the control polysomnography. This is the first reported case of Forestier Syndrome (FS) associated with Obstructive Sleep Apnea (OSA) that was proposed surgical treatment and the patient evolved with complete symptom improvement. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

[The problems of diagnosis and correction of autism in children (an example of Asperger's syndrome)].

PubMed

Iovchuk, N M; Severnyĭ, A A

Based on the analysis of literature and own clinical experience, we discuss diagnostic issues of early autistic disorders in children. Main differential-diagnostic signs that permit to differentiate mild forms of autism in childhood diagnosed as Asperger's syndrome from childhood schizophrenia, residual organic CNS damage, circular affective disorders are described. Cases of Asperger's syndrome followed up for many years and recommendations for social and psychological adaptation of children and adolescents with Asperger's syndrome in different age periods are presented.

Hypothermic general cold adaptation induced by local cold acclimation.

PubMed

Savourey, G; Barnavol, B; Caravel, J P; Feuerstein, C; Bittel, J H

1996-01-01

To study relationships between local cold adaptation of the lower limbs and general cold adaptation, eight subjects were submitted both to a cold foot test (CFT, 5 degrees C water immersion, 5 min) and to a whole-body standard cold air test (SCAT, 1 degree C, 2 h, nude at rest) before and after a local cold acclimation (LCA) of the lower limbs effected by repeated cold water immersions. The LCA induced a local cold adaptation confirmed by higher skin temperatures of the lower limbs during CFT and a hypothermic insulative general cold adaptation (decreased rectal temperature and mean skin temperature P < 0.05) without a change either in metabolic heat production or in lower limb skin temperatures during SCAT after LCA. It was concluded that local cold adaptation was related to the habituation process confirmed by decreased plasma concentrations of noradrenaline (NA) during LCA (P < 0.05). However, the hypothermic insulative general cold adaptation was unrelated either to local cold adaptation or to the habituation process, because an increased NA during SCAT after LCA (P < 0.05) was observed but was rather related to a "T3 polar syndrome" occurring during LCA.

[Cyclophosphamide in idiopathic nephrotic syndrome: Outcome and outlook].

PubMed

Berkane, Majda; Adarmouch, Latifa; Amine, Mohamed; Bourrahouat, Aicha; Ait Sab, Imane; Sbihi, Mohamed

2018-04-01

Cyclophosphamide (CYP) has been used for over 40 years in patients with steroid-sensitive nephrotic syndrome (NSSS) presenting frequent relapses (NSRF) or steroid dependence (NSSD). However, the long-term success of treatment with cyclophosphamide is difficult to predict. The objectives of this study are to determine long-term outcomes of cyclophosphamide and identify the factors associated with sustained remission. We retrospectively studied the data from 50 patients with idiopathic nephrotic syndrome, treated by oral cyclophosphamide and followed at service of pediatric for more than 8 years for idiopathic nephrotic syndrome and related factors for survival without relapse were evaluated by univariate analysis. The median age at the time of diagnosis was 4.3 years, and median follow-up time was 1.7 years with the median of 8 years at the first use of CYC. Patients had received a median cumulative dose of 168mg/kg. At the end of follow-up, 38% of patients entered into remission after using CYC while 62% failed to respond and further relapses then occur. The median time of stopping corticosteroid therapy was three month. The survival without relapse was respectively 56% (28 patients), 52% (26 patients), 48% (24 patients), and 38% (19 patients), at 6 months, one year, two years and more than two years. In univariate analysis, the survival without relapse was related to the age at the moment of starting the therapy par CYC (the median was 5 months for an age < 8 years and 41 months for an age≥8 years; P=0.049), the type of nephrotic syndrome [36 months for SNRF, 4 months for NSSD and nephrothic syndrome steroid resistant (NSSR); P=0.068], and the histological lesion (6 months for diffuse mesangial proliferation, 2 months for segmental glomerulosclerosis; P=0.009). The age at the moment of diagnosis, the sex and the cumulative dose of CYC did not have significant influence. The results presented in this study suggest the use of oral cyclophosphamide for short

Do the exome: A case of Williams-Beuren syndrome with severe epilepsy due to a truncating de novo variant in GABRA1.

PubMed

Popp, Bernt; Trollmann, Regina; Büttner, Christian; Caliebe, Almuth; Thiel, Christian T; Hüffmeier, Ulrike; Reis, André; Zweier, Christiane

2016-10-01

Williams-Beuren syndrome (WBS) is a relatively common, clinically recognizable microdeletion syndrome. In most cases the typical heterozygous deletion of 1.5 Mb on chromosome 7q11.23 spanning about 26 genes can be identified. Also some larger or smaller atypical deletions have been reported and associated with additional or atypical phenotypic aspects. We report on an individual with typical WBS due to the common deletion and with refractory infantile spasms. Using trio-exome sequencing, we identified a de novo truncating variant c.1200del, p (Lys401Serfs*25) in GABRA1 as the likely cause of the early onset epilepsy. This unique case not only allows to further define the phenotypic spectrum of infantile epileptic encephalopathy associated with rare de novo GABRA1 variants but exemplifies the need for a sensitive review of unclear associations in clinically defined syndromes and for extended diagnostic work-up in individuals with unusual presentations of a genetically confirmed diagnosis. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

[Prospective, monocentric, uncontrolled study of efficacy, tolerance and adherence of cyclosporin 0.1 % for severe dry eye syndrome].

PubMed

Boujnah, Y; Mouchel, R; El-Chehab, H; Dot, C; Burillon, C; Kocaba, V

2018-02-01

To evaluate the efficacy, tolerability and treatment adherence of Ikervis ® (Santen, SAS) (ciclosporine 0.1 %) for first line therapy or following treatment with Restasis ® (Allergan, Inc.) (ciclosporine 0.05 %) for severe dry eye syndrome. A prospective, monocentric, uncontrolled study was conducted between January 2012 and March 2015 on 110 eyes of 55 patients with severe dry eye on first line therapy or previously treated with Restasis ® who required the introduction of Ikervis ® . Patients' quality of life was assessed before and after treatment was started using a standardized questionnaire (Ocular Surface Disease Index © [OSDI]), clinical efficacy was quantified at the slit lamp, by measurement of the Break Up time Test (BUT) and the Oxford classification. Tolerability and adherence to treatment were measured using a simple questionnaire. A total of 72 eyes of 37 patients were included. Etiologies of dry eye syndrome were dominated by Sjögren syndrome (32 %) and severe ocular surface conditions (48 %). The mean age was 57.7 years (±17.45) and mean follow-up was 458 days (±292). The mean BUT increased by 2.043seconds [1.522-2.563] (P<0.0001). Corneal/conjunctival involvement evaluated by the Oxford classification was also improved with a difference in level of 1.68 [1.290-2.071] (P<0.0001). Ocular Surface Disease Index © (OSDI) decreased by 21.7 [16.372-27.024] (P<0.0001). Treatment tolerability was moderate, with more than 50 % of patients experiencing pain on instillation. Overall satisfaction with treatment was good, with more than 60 % of patients feeling better after initiation of treatment. Ikervis ® is an effective treatment of severe dry eye. Its indications tend to evolve towards less severe dry eye. However, the tolerability profile remains poor, and an improvement in this would be desirable. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Maternal Sensitivity, Child Functional Level, and Attachment in Down Syndrome.

ERIC Educational Resources Information Center

Atkinson, Leslie; Chrisholm, Vivienne C.; Scott, Brian; Goldberg, Susan; Vaughn, Brian E.; Blackwell, Janis; Dickens, Susan; Tam, Frances

1999-01-01

Investigated the influence of child intellectual/adaptive functioning and maternal sensitivity on attachment security, using a sample of children with Down syndrome. Found a relationship between attachment security in DS related to the interaction of maternal sensitivity and cognitive competence. (JPB)

Numerical exploration of non-axisymmetric divertor closure in the small angle slot (SAS) divertor at DIII-D

DOE PAGES

Frerichs, H.; Schmitz, O.; Covele, B.; ...

2018-02-28

Numerical simulations of toroidal asymmetries in a tightly baffled small angle slot (SAS) divertor on the DIII-D tokamak show that toroidal asymmetries in divertor closure result in (non-axisymmetric) local onset of detachment within a density window of 10-15% on top of the nominal threshold separatrix density. The SAS divertor is explored at DIII-D for improving access to cold, dissipative/detached divertor conditions. The narrow width of the slot divertor coupled with a small magnetic field line-to-target angle facilitates the buildup of neutral density, thereby increasing radiative and neutrals-related (atoms and molecules) losses in the divertor. Therefore, small changes in the strikemore » point location can be expected to have a large impact on diverter conditions. The combination of misaligned slot structure and non-axisymmetric perturbations to the magnetic field configuration causes the strike point to move along the divertor target plate, possibly leaving the diverter slot at some locations. The latter extreme case essentially introduces an opening in the divertor slot from where recycling neutrals can easily escape, and thereby degrade the performance of the slot divertor. Such a strike point dislocation is approximated by a finite gap in the divertor baffle for which three dimensional edge plasma and neutral gas simulations are performed with the EMC3-EIRENE code.« less

Numerical exploration of non-axisymmetric divertor closure in the small angle slot (SAS) divertor at DIII-D

DOE Office of Scientific and Technical Information (OSTI.GOV)

Frerichs, H.; Schmitz, O.; Covele, B.

Numerical simulations of toroidal asymmetries in a tightly baffled small angle slot (SAS) divertor on the DIII-D tokamak show that toroidal asymmetries in divertor closure result in (non-axisymmetric) local onset of detachment within a density window of 10-15% on top of the nominal threshold separatrix density. The SAS divertor is explored at DIII-D for improving access to cold, dissipative/detached divertor conditions. The narrow width of the slot divertor coupled with a small magnetic field line-to-target angle facilitates the buildup of neutral density, thereby increasing radiative and neutrals-related (atoms and molecules) losses in the divertor. Therefore, small changes in the strikemore » point location can be expected to have a large impact on diverter conditions. The combination of misaligned slot structure and non-axisymmetric perturbations to the magnetic field configuration causes the strike point to move along the divertor target plate, possibly leaving the diverter slot at some locations. The latter extreme case essentially introduces an opening in the divertor slot from where recycling neutrals can easily escape, and thereby degrade the performance of the slot divertor. Such a strike point dislocation is approximated by a finite gap in the divertor baffle for which three dimensional edge plasma and neutral gas simulations are performed with the EMC3-EIRENE code.« less

[Cayler syndrome: A case report and review of the literature].

PubMed

Bellaiche, J; Correia, N; Bouche Pillon Persyn, M A; Chiriac, S; Bodin, F; François, C

2016-08-01

Facial asymmetries to the tears are rare. We report a pediatric original case that may fall within the framework of a Cayler syndrome. Through its clinical presentation, we will discuss differential diagnoses, associated forms, its etiology, and its management. At the maternity unit, in a male infant, after vaginal delivery at term without extraction, was discovered a lack of mobility of the labial commissure on the right side, only when crying. The rest of the examination was unremarkable, except ipsilateral microtia. Genetically, karyotype was 46,XY, 22q11 without microdeletion. The head and neck MRI and echocardiogram were normal. Asymmetry with tears has been described in the literature, through association with microdeletion 22q11 syndrome. The originality of this case was the presence of an isolated muscle abnormality. Muscles affected by this syndrome are: Musculus depressor labii inferioris, the Depressor anguli oris, and Mentalis musculus. The three muscles can be affected concomitantly. Isolated involvment of the Depressor anguli oris muscle has also been described. The mechanical dysfunction can be either linked to muscle innervation agenesis or to a defect thereof. There is no specific treatment. The symptoms improve with age by decreasing the frequency of crying. However, it is important to know this pathology in order to seek an optimum balance further in search of associated abnormalities (FISH 22q11, cardiac Doppler ultrasound) but also to educate, to reassure families often worried by the situation. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Adaptation and readaptation medical concerns of a Mars trip

NASA Technical Reports Server (NTRS)

Johnson, Philip C.

1986-01-01

The ability of the human body to adapt to microgravity environments and to later readapt to a gravity environment was examined. Issues specifically relating to the effects of long duration space flight on the adaptation/readaptation process are discussed. The need for better health prediction techniques is stressed in order to be able to better anticipate crew health problems and to perform corrective actions. Several specific examples are discussed of latent diseases which could occur during a long duration space mission, even after having subjected the crew to thorough premission checkups. The need for learning how to prevent or ameliorate such problems as space adaptation syndrome, bone and muscle (and possibly tissue) atrophy, immune system atrophy, and heart arrythmias is also discussed. The implications of the age of the crew, the influence of an onboard low level gravity field, and drugs are briefly addressed as factors in the adaptation/readaptation process.

Cultural adaptation: translatability assessment and linguistic validation of the patient-reported outcome instrument for irritable bowel syndrome with diarrhea

PubMed Central

Delgado-Herrera, Leticia; Lasch, Kathryn; Popielnicki, Ana; Nishida, Akito; Arbuckle, Rob; Banderas, Benjamin; Zentner, Susan; Gagainis, Ingrid; Zeiher, Bernhardt

2016-01-01

Background and objective Following a 2009 US Food and Drug Administration guidance, a new patient-reported outcome (PRO) instrument was developed to support end points in multinational clinical trials assessing irritable bowel syndrome with diarrhea (IBS-D) symptom severity. Our objective was to assess the translatability of the IBS-D PRO instrument into ten languages, and subsequently perform a cultural adaptation/linguistic validation of the questionnaire into Japanese and US Spanish. Materials and methods Translatability assessments of the US English version of the IBS-D PRO were performed by experienced PRO translators who were native speakers of each target language and currently residing in target-language countries. Languages were Chinese (People’s Republic of China), Dutch (the Netherlands), French (Belgium), German (Germany), Japanese (Japan), Polish (Poland), Portuguese (Brazil), Russian (Russia), Spanish (Mexico), and Spanish (US). The project team assessed the instrument to identify potential linguistic and/or cultural adaptation issues. After the issues identified were resolved, the instrument was translated into Spanish (US) and Japanese through a process of two forward translations, one reconciled translation, and one backward translation. The project team reviewed the translated versions before the instruments were evaluated by cognitive debriefing interviews with samples of five Spanish (US) and five Japanese IBS-D patients. Results Linguistic and cultural adaptation concerns identified during the translatability assessment required minor revisions, mainly the presentation of dates/times and word structure. During the cognitive debriefing interviews, two of five Spanish respondents misunderstood the term “bowel movement” to mean only diarrhea in the Spanish version. Consequently, the term was changed from “movimiento intestinal” to “evacuaciones”. None of the Japanese respondents identified issues with the Japanese version. Conclusion

Cultural adaptation: translatability assessment and linguistic validation of the patient-reported outcome instrument for irritable bowel syndrome with diarrhea.

PubMed

Delgado-Herrera, Leticia; Lasch, Kathryn; Popielnicki, Ana; Nishida, Akito; Arbuckle, Rob; Banderas, Benjamin; Zentner, Susan; Gagainis, Ingrid; Zeiher, Bernhardt

2016-01-01

Following a 2009 US Food and Drug Administration guidance, a new patient-reported outcome (PRO) instrument was developed to support end points in multinational clinical trials assessing irritable bowel syndrome with diarrhea (IBS-D) symptom severity. Our objective was to assess the translatability of the IBS-D PRO instrument into ten languages, and subsequently perform a cultural adaptation/linguistic validation of the questionnaire into Japanese and US Spanish. Translatability assessments of the US English version of the IBS-D PRO were performed by experienced PRO translators who were native speakers of each target language and currently residing in target-language countries. Languages were Chinese (People's Republic of China), Dutch (the Netherlands), French (Belgium), German (Germany), Japanese (Japan), Polish (Poland), Portuguese (Brazil), Russian (Russia), Spanish (Mexico), and Spanish (US). The project team assessed the instrument to identify potential linguistic and/or cultural adaptation issues. After the issues identified were resolved, the instrument was translated into Spanish (US) and Japanese through a process of two forward translations, one reconciled translation, and one backward translation. The project team reviewed the translated versions before the instruments were evaluated by cognitive debriefing interviews with samples of five Spanish (US) and five Japanese IBS-D patients. Linguistic and cultural adaptation concerns identified during the translatability assessment required minor revisions, mainly the presentation of dates/times and word structure. During the cognitive debriefing interviews, two of five Spanish respondents misunderstood the term "bowel movement" to mean only diarrhea in the Spanish version. Consequently, the term was changed from "movimiento intestinal" to "evacuaciones". None of the Japanese respondents identified issues with the Japanese version. The translatability of the IBS-D PRO instrument into ten target languages was

A tumor profile in Patau syndrome (trisomy 13).

PubMed

Satgé, Daniel; Nishi, Motoi; Sirvent, Nicolas; Vekemans, Michel; Chenard, Marie-Pierre; Barnes, Ann

2017-08-01

Individuals with trisomic conditions like Down syndrome and Edwards syndrome are prone to certain types of malignancy. However, for Patau syndrome (constitutional trisomy 13), which occurs in 1/10,000-1/20,000 live births, the tumor profile has not been well characterized. An awareness of susceptibility to malignancies can improve care of affected individuals, as well as further our understanding of the contribution of trisomy to carcinogenesis. Therefore, we conducted an extensive review of the literature; we found 17 malignancies reported in individuals with Patau syndrome. These comprised eight embryonic tumors, three leukemias, two malignant germ cell tumors, two carcinomas, a malignant brain tumor, and a sarcoma. Benign tumors were mainly extragonadal teratomas. The small number of reported malignant tumors suggests that there is not an increased risk of cancer in the context of trisomy 13. The tumor profile in Patau syndrome differs from that observed in Edwards syndrome (trisomy 18) and Down syndrome (trisomy 21), suggesting that the supernumerary chromosome 13 could promote particular tumor formations as it does particular malformations. No general and direct relationships of tumor occurrence with organ weight, congenital malformations, histological changes, or presence of tumor suppressor genes on chromosome 13 were observed. However, some tumors were found in tissues whose growth and development are controlled by genes mapping to chromosome 13. Recent reports of successful outcomes following surgical treatment and adapted chemotherapy indicate that treatment of cancer is possible in Patau syndrome. © 2017 Wiley Periodicals, Inc.

Neuromuscular and muscle-tendon system adaptations to isotonic and isokinetic eccentric exercise.

PubMed

Guilhem, G; Cornu, C; Guével, A

2010-06-01

To present the properties of an eccentric contraction and compare neuromuscular and muscle-tendon system adaptations induced by isotonic and isokinetic eccentric trainings. An eccentric muscle contraction is characterized by the production of muscle force associated to a lengthening of the muscle-tendon system. This muscle solicitation can cause micro lesions followed by a regeneration process of the muscle-tendon system. Eccentric exercise is commonly used in functional rehabilitation for its positive effect on collagen synthesis but also for resistance training to increase muscle strength and muscle mass in athletes. Indeed, eccentric training stimulates muscle hypertrophy, increases the fascicle pennation angle, fascicles length and neural activation, thus inducing greater strength gains than concentric or isometric training programs. Eccentric exercise is commonly performed either against a constant external load (isotonic) or at constant velocity (isokinetic), inducing different mechanical constraints. These different mechanical constraints could induce structural and neural adaptive strategies specific to each type of exercise. The literature tends to show that isotonic mode leads to a greater strength gain than isokinetic mode. This observation could be explained by a greater neuromuscular activation after IT training. However, the specific muscle adaptations induced by each mode remain difficult to determine due to the lack of standardized, comparative studies. 2010 Elsevier Masson SAS. All rights reserved.

[Update Churg-Strauss syndrome].

PubMed

Moosig, F; Hellmich, B

2012-11-01

The Churg-Strauss syndrome (CSS) is the rarest subtype of the so-called anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) and has the lowest frequency of ANCA-positivity (around 30%). In addition to asthma and blood eosinophilia, CSS is characterized by end-organ damage, which can be caused by either vasculitis and/or tissue infiltration of eosinophilic granulocytes. The CSS shares many etiological and clinical features of other hypereosinophilic syndromes. Recently, a distinct genetic background could be demonstrated for both the ANCA-positive and ANCA-negative subtypes of CSS as compared to the other two forms of AAV. Among other cytokines, interleukin-5 (IL-5) could be identified as a key mediator of eosinophilia. Therefore, recent clinical trials in CSS aimed to target IL-5. Outside of clinical trials, treatment of CSS is adapted to disease stage and activity, as recommended for other types of AAV.

[The Confusion Assessment Method: Transcultural adaptation of a French version].

PubMed

Antoine, V; Belmin, J; Blain, H; Bonin-Guillaume, S; Goldsmith, L; Guerin, O; Kergoat, M-J; Landais, P; Mahmoudi, R; Morais, J A; Rataboul, P; Saber, A; Sirvain, S; Wolfklein, G; de Wazieres, B

2018-05-01

The Confusion Assessment Method (CAM) is a validated key tool in clinical practice and research programs to diagnose delirium and assess its severity. There is no validated French version of the CAM training manual and coding guide (Inouye SK). The aim of this study was to establish a consensual French version of the CAM and its manual. Cross-cultural adaptation to achieve equivalence between the original version and a French adapted version of the CAM manual. A rigorous process was conducted including control of cultural adequacy of the tool's components, double forward and back translations, reconciliation, expert committee review (including bilingual translators with different nationalities, a linguist, highly qualified clinicians, methodologists) and pretesting. A consensual French version of the CAM was achieved. Implementation of the CAM French version in daily clinical practice will enable optimal diagnosis of delirium diagnosis and enhance communication between health professionals in French speaking countries. Validity and psychometric properties are being tested in a French multicenter cohort, opening up new perspectives for improved quality of care and research programs in French speaking countries. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

SAS molecular tests Salmonella detection kit. Performance tested method 021202.

PubMed

Bapanpally, Chandra; Montier, Laura; Khan, Shah; Kasra, Akif; Brunelle, Sharon L

2014-01-01

The SAS Molecular tests Salmonella Detection method, a Loop-mediated Isothermal Amplification method, performed as well as or better than the U.S. Department of Agriculture-Food Safety Inspection Service Microbiology Laboratory Guidebook and the U.S. Food and Drug Administration Bacteriological Analytical Manual reference methods for ground beef, beef trim, ground turkey, chicken carcass rinses, bagged mixed lettuce, and fresh spinach. The ground beef (30% fat, 25 g test portion), poultry matrixes and leafy greens were validated in a 6-7 h enrichment, and ground beef (30% fat, 375 g composite test portion) and beef trim (375 g composite test portion) were validated in a 16-20 h enrichment. The method performance for meat and leafy green matrixes was shown to be acceptable under conditions of co-enrichment with Escherichia coli 0157. Thus, after a short 6-7 h co-enrichment step, ground beef, beef trim, lettuce, and spinach can be tested for both Salmonella and E. coli O157. Inclusivity and exclusivity testing revealed no false negatives and no false positives among the 100 Salmonella serovars and 30 non-Salmonella species examined. The method was shown to be robust when enrichment time, DNA extract hold time, and DNA volume were varied.

Annotated Computer Output for Illustrative Examples of Clustering Using the Mixture Method and Two Comparable Methods from SAS.

DTIC Science & Technology

1987-06-26

BUREAU OF STANDAR-S1963-A Nw BOM -ILE COPY -. 4eo .?3sa.9"-,,A WIN* MAT HEMATICAL SCIENCES _*INSTITUTE AD-A184 687 DTICS!ELECTE ANNOTATED COMPUTER OUTPUT...intoduction to the use of mixture models in clustering. Cornell University Biometrics Unit Technical Report BU-920-M and Mathematical Sciences Institute...mixture method and two comparable methods from SAS. Cornell University Biometrics Unit Technical Report BU-921-M and Mathematical Sciences Institute

[Alport syndrome: Hereditary nephropathy associated with mutations in genes coding for type IV collagen chains].

PubMed

Heidet, Laurence; Gubler, Marie-Claire

2016-12-01

Alport syndrome is an inherited disorder characterized by the association of a progressive haematuric nephropathy with ultrastructural abnormalities of the glomerular basement membranes, a progressive sensorineural hearing loss and sometimes ocular involvement. Its incidence is less than 1 per 5000 individuals and the disease is the cause of about 2% of end stage renal disease in Europe and the United States. Alport syndrome is clinically and genetically heterogeneous. It is related to mutations in the genes encoding one of three chains, α3, α4 α5 of type IV collagen, the main component of basement membranes, expressed in the glomerular basement membrane. COL4A5 mutations are associated with X-linked Alport syndrome, which represents 80 to 85% of cases and is more severe in boys than in girls. Mutations in COL4A3 or COL4A4 are associated with autosomal Alport syndrome. The expression of collagen chains in skin and kidney basement membranes allows for the diagnosis and characterization of the mode of transmission in most patients. It is necessary to diagnose this syndrome because its family involvement, its severity, and the importance of genetic counseling. Angiotensin blockers are increasingly prescribed in proteinuric patients. Prospective studies are needed to assess the effectiveness of these treatments on proteinuria and progression of kidney failure, and to specify indications. Animal studies have shown the potential value of different molecules (protease inhibitors, chemokine receptor blockers, transforming growth factor-β1 inhibitors, hydroxy-methyl-coenzyme A reductase inhibitors, bone morphogenetic protein-7 inhibitors), hematopoietic stem cells, and of a anti-micro-RNA. Copyright © 2016. Published by Elsevier SAS.

Exogenous glucagon-like peptide-2 improves outcomes of intestinal adaptation in a distal-intestinal resection neonatal piglet model of short bowel syndrome.

PubMed

Suri, Megha; Turner, Justine M; Sigalet, David L; Wizzard, Pamela R; Nation, Patrick N; Ball, Ron O; Pencharz, Paul B; Brubaker, Patricia L; Wales, Paul W

2014-10-01

Endogenous glucagon-like peptide-2 (GLP-2) levels and intestinal adaptation are reduced in distal-intestinal resection animal models of short bowel syndrome (SBS) that lack remnant ileum. We hypothesized that exogenous GLP-2 would improve intestinal adaptation in a distal-intestinal resection neonatal piglet model of SBS. In all, 35 piglets were randomized to 2 treatment and 3 surgical groups: control (sham), 75% mid-intestinal resection (JI), and 75% distal-intestinal resection (JC). Parenteral nutrition (PN) commenced on day 1 and was weaned as enteral nutrition (EN) advanced. IV GLP-2 (11 nmol/kg/d) or saline was initiated on day 2. Piglets were maintained for 14 d. Clinical, functional, morphological, and histological outcomes were obtained. JC-GLP-2 piglets had fewer days on PN (10.0 ± 0.6 vs. 13.8 ± 0.2), more days on EN (4.0 ± 0.6 vs. 0.2 ± 0.2), a higher percentage of EN at termination (92 ± 5 vs. 52 ± 10%), fewer days of diarrhea (8.0 ± 0.7 vs. 12.3 ± 0.4), increased intestinal length (19 ± 4 vs. -5 ± 3%), and deeper jejunal crypts (248 ± 21 vs. 172 ± 12 μm), compared with saline piglets. GLP-2 therapy improves clinical, morphological, and histological outcomes of intestinal adaptation in a distal-intestinal resection model of SBS. Since this anatomical subtype represents the majority of clinical cases of neonatal SBS, these results support a potential role for GLP-2 therapy in pediatric SBS.

A novel model for simultaneous study of neointestinal regeneration and intestinal adaptation.

PubMed

Jwo, Shyh-Chuan; Tang, Shye-Jye; Chen, Jim-Ray; Chiang, Kun-Chun; Huang, Ting-Shou; Chen, Huang-Yang

2013-01-01

The use of autologous grafts, fabricated from tissue-engineered neointestine, to enhance insufficient compensation of intestinal adaptation for severe short bowel syndrome is a compelling idea. Unfortunately, current approaches and knowledge for neointestinal regeneration, unlike intestinal adaptation, are still unsatisfactory. Thus, we have designed a novel model of intestinal adaptation with simultaneous neointestinal regeneration and evaluated its feasibility for future basic research and clinical application. Fifty male Sprague-Dawley rats weighing 250-350 g underwent this procedure and sacrificed at 4, 8, and 12 weeks postoperatively. Spatiotemporal analyses were carried out by gross, histology, and DNA/protein quantification. Three rats died of operative complications. In early experiments, the use of hard silicone stent as tissue scaffold in 11 rats was unsatisfactory for neointestinal regeneration. In later experiments, when a soft silastic tube was used, the success rate increased up to 90.9%. Further analyses revealed that no neointestine developed without donor intestine; regenerated lengths of mucosa and muscle were positively related to time postsurgery but independent of donor length with 0.5 or 1 cm. Other parameters of neointestinal regeneration or intestinal adaptation showed no relationship to both time postsurgery and donor length. In conclusion, this is a potentially important model for investigators searching for solutions to short bowel syndrome. © 2013 by the Wound Healing Society.

Behavior and neuropsychiatric manifestations in Angelman syndrome

PubMed Central

Pelc, Karine; Cheron, Guy; Dan, Bernard

2008-01-01

Angelman syndrome has been suggested as a disease model of neurogenetic developmental condition with a specific behavioral phenotype. It is due to lack of expression of the UBE3A gene, an imprinted gene located on chromosome 15q. Here we review the main features of this phenotype, characterized by happy demeanor with prominent smiling, poorly specific laughing and general exuberance, associated with hypermotor behavior, stereotypies, and reduced behavioral adaptive skills despite proactive social contact. All these phenotypic characteristics are currently difficult to quantify and have been subject to some differences in interpretation. For example, prevalence of autistic disorder is still debated. Many of these features may occur in other syndromic or nonsyndromic forms of severe intellectual disability, but their combination, with particularly prominent laughter and smiling may be specific of Angelman syndrome. Management of problematic behaviors is primarily based on behavioral approaches, though psychoactive medication (eg, neuroleptics or antidepressants) may be required. PMID:18830393

Prevalence of the metabolic syndrome in people of Asian Indian origin: outcomes by definitions.

PubMed

Das, M; Pal, S; Ghosh, A

2011-01-01

The prevalence of the metabolic syndrome (MS) is high among south Asian Indians. In order to better comprehend the MS, its definition and modifications require region-specific cut-off values and common minimum criteria for people of Indian origin. To define the MS, the criteria as defined in the National Cholesterol Education Program (NCEP): expert panel on detection, evaluation, and treatment of high blood cholesterol in adults (Adult Treatment Panel III) (ATP III 2001), followed by the modified ATP III of 2005 were used, along with a modified version specific to the people of south Asian origin (ATP III SAS, 2009). The three definitions showed differences in prevalence of the MS among the adult Asian Indians. According to the criteria of NCEP ATP III 2001, the prevalence was found to be 32.3%. Using the modified ATP III 2005, the prevalence was 48.3%, and for south Asian-specific (SAS) ATP III, it was 31.4%. For all three definitions, females had a considerably higher prevalence of the MS than males. It was also observed that that a large number of individuals were misclassified due to lack of common minimum criteria. In order to curb the growing threat of the MS, and to aid clinical management among people of Indian origin, a more comprehensive definition of the MS is urgently required.

[Irritable bowel syndrome: New pathophysiological hypotheses and practical issues].

PubMed

Duboc, H; Dior, M; Coffin, B

2016-08-01

In 2015, besides the fact that it still fills the gastroenterologists' offices and impairs patient's quality of life, the irritable bowel syndrome has considerably evolved on several points. The pathophysiology is now organized around a consensual hypothesis called the "brain-gut axis", which gather all the influences of peripheral factors as gut microbiota or local serotonin secretion, on the central pain perception, contributing to visceral hypersensitivity and transit modifications. About the diagnosis, the key message is "avoid over-prescription" of additional tests, and reminds that a positive clinical diagnosis based on Rome III criteria is possible after the elimination of simple clinical warning signs. Finally, the food component, a neglected and historical claim of patients, finally finds a strong scientific rational, with a diet low in fermentable sugar and polyols, that gives positive and reproducible results. Copyright © 2016 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Brief Report: Autism in Individuals with Down Syndrome

ERIC Educational Resources Information Center

Starr, Elizabeth M.; Berument, Sibel Kazak; Tomlins, Megan; Papanikolaou, Katerina; Rutter, Michael

2005-01-01

As an off-shoot of a study examining the reliability and validity of an adapted version of the Pre-Linguistic Autism Diagnostic Observation Schedule (A-PL-ADOS), 13 individuals with Down syndrome with IQs ranging between 24 and 48 were administered the Autism Diagnostic Interview-Revised (ADI-R) and the A-PL-ADOS, which are well-validated…

SPSS and SAS programs for determining the number of components using parallel analysis and velicer's MAP test.

PubMed

O'Connor, B P

2000-08-01

Popular statistical software packages do not have the proper procedures for determining the number of components in factor and principal components analyses. Parallel analysis and Velicer's minimum average partial (MAP) test are validated procedures, recommended widely by statisticians. However, many researchers continue to use alternative, simpler, but flawed procedures, such as the eigenvalues-greater-than-one rule. Use of the proper procedures might be increased if these procedures could be conducted within familiar software environments. This paper describes brief and efficient programs for using SPSS and SAS to conduct parallel analyses and the MAP test.

Al-Awadi-Raas-Rothschild syndrome with dental anomalies and a novel WNT7A mutation.

PubMed

Kantaputra, Piranit Nik; Kapoor, Seema; Verma, Prashant; Kaewgahya, Massupa; Kawasaki, Katsushige; Ohazama, Atsushi; Ketudat Cairns, James R

2017-12-01

Al-Awadi-Raas-Rothschild syndrome (AARRS; OMIM 276820) is a very rare autosomal recessive limb malformation syndrome caused by WNT7A mutations. AARRS is characterized by various degrees of limb aplasia and hypoplasia. Normal intelligence and malformations of urogenital system are frequent findings. Complete loss of WNT7A function has been shown to cause AARRS, however, its partial loss leads to the milder malformation, Fuhrmann syndrome. An Indian boy affected with AARRS is reported. A novel homozygous base substitution mutation c.550A > C (p.Asn184Asp) is identified in the patient. Parents were heterozygous for the mutation. In addition to the typical features of AARRS, the patient had agenesis of the mandibular left deciduous lateral incisor. The heterozygous parents had microdontia of the maxillary left permanent third molar and taurodontism (enlarged dental pulp chamber at the expense of root) in a number of their permanent molars. Whole exome sequencing of the patient and his parents ruled out mutations in 11 known hypodontia-associated genes including WNT10A, MSX1, EDA, EDAR, EDARADD, PAX9, AXIN2, GREM2, NEMO, KRT17, and TFAP2B. In situ hybridization during tooth development showed Wnt7a expression in wild-type tooth epithelium at E14.5. All lines of evidence suggest that WNT7A has important role in tooth development and its mutation may lead to tooth agenesis, microdontia, and taurodontism. Oral examination of patients with AARRS and Fuhrmann syndromes is highly recommended. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Growing up with Down syndrome: Development from 6 months to 10.7 years.

PubMed

Marchal, Jan Pieter; Maurice-Stam, Heleen; Houtzager, Bregje A; Rutgers van Rozenburg-Marres, Susanne L; Oostrom, Kim J; Grootenhuis, Martha A; van Trotsenburg, A S Paul

2016-12-01

We analysed developmental outcomes from a clinical trial early in life and its follow-up at 10.7 years in 123 children with Down syndrome. To determine 1) strengths and weaknesses in adaptive functioning and motor skills at 10.7 years, and 2) prognostic value of early-life characteristics (early developmental outcomes, parental and child characteristics, and comorbidity) for later intelligence, adaptive functioning and motor skills. We used standardized assessments of mental and motor development at ages 6, 12 and 24 months, and of intelligence, adaptive functioning and motor skills at 10.7 years. We compared strengths and weaknesses in adaptive functioning and motor skills by repeated-measures ANOVAs in the total group and in children scoring above-average versus below-average. The prognostic value of demographics, comorbidity and developmental outcomes was analysed by two-step regression. Socialisation was a stronger adaptive skill than Communication followed by Daily Living. Aiming and catching was a stronger motor skill than Manual dexterity, followed by Balance. Above-average and below-average scoring children showed different profiles of strengths and weaknesses. Gender, (the absence or presence of) infantile spasms and particularly 24-month mental functioning predicted later intelligence and adaptive functioning. Motor skills, however, appeared to be less well predicted by early life characteristics. These findings provide a reference for expected developmental levels and strengths and weaknesses in Down syndrome. Copyright © 2016 Elsevier Ltd. All rights reserved.

Characteristics And Development Of Children With CHARGE Association-Syndrome

ERIC Educational Resources Information Center

Salem-Hartshorne, Nancy; Jacob, Susan

2004-01-01

CHARGE association-syndrome refers to a group of congenital anomalies occurring together more often than chance. Parents of children with CHARGE were surveyed and asked to indicate whether their child had various features commonly found among individuals with CHARGE (e.g., vision or hearing impairment) and to complete an adaptive behavior scale…

A SAS-based solution to evaluate study design efficiency of phase I pediatric oncology trials via discrete event simulation.

PubMed

Barrett, Jeffrey S; Jayaraman, Bhuvana; Patel, Dimple; Skolnik, Jeffrey M

2008-06-01

Previous exploration of oncology study design efficiency has focused on Markov processes alone (probability-based events) without consideration for time dependencies. Barriers to study completion include time delays associated with patient accrual, inevaluability (IE), time to dose limiting toxicities (DLT) and administrative and review time. Discrete event simulation (DES) can incorporate probability-based assignment of DLT and IE frequency, correlated with cohort in the case of DLT, with time-based events defined by stochastic relationships. A SAS-based solution to examine study efficiency metrics and evaluate design modifications that would improve study efficiency is presented. Virtual patients are simulated with attributes defined from prior distributions of relevant patient characteristics. Study population datasets are read into SAS macros which select patients and enroll them into a study based on the specific design criteria if the study is open to enrollment. Waiting times, arrival times and time to study events are also sampled from prior distributions; post-processing of study simulations is provided within the decision macros and compared across designs in a separate post-processing algorithm. This solution is examined via comparison of the standard 3+3 decision rule relative to the "rolling 6" design, a newly proposed enrollment strategy for the phase I pediatric oncology setting.

[Morel-Lavallée syndrome and post-traumatic nodular fat necrosis: Two post-traumatic complications mimicking cellulitis].

PubMed

Moulin, C; Barthélémy, I; Emering, C; D'Incan, M

Dermal and subcutaneous inflammation following direct trauma is initially evocative of soft-tissue infection. However, two differential diagnoses must be considered: Morel-Lavallée syndrome and post-traumatic nodular fat necrosis. Case 1: a 51-year-old woman fell off her motorbike and had dermabrasions on her right and left tibial ridges that rapidly developed into dermo-hypodermitis of the entire limb. There was no improvement after 3 weeks of antibiotics. The patient was apyretic. She had a soft, non-inflammatory tumefaction on the inner aspect of her left knee. Ultrasound revealed subcutaneous collection in both legs. The surgeons confirmed a diagnosis of Morel-Lavallée syndrome and drained the two collections. Progress was good and the patient healed without major consequences. Case 2: following a fall on her stairs, a 40-year-old woman presented dermabrasions and haematomas on her left leg. Antibiotic therapy failed to prevent the progression of dermo-hypodermitis. The patient remained apyretic and there was no inflammatory syndrome. A CT scan showed thickening of a subcutaneous fat and fluid collection, resulting in diagnosis of post-traumatic nodular fat necrosis. Management was surgical and the outcome was good. These two cases show two post-traumatic cutaneous complications: Morel-Lavallée syndrome and post-traumatic nodular fat necrosis. Morel-Lavallée syndrome occurs after tangential trauma next to richly vascularized tissue. Post-traumatic nodular fat necrosis is defined as necrosis of adipocytes. In both cases, diagnosis is confirmed by imagery (Ultrasonography, tomography). Our two case reports show that inflammatory presentation of both Morel-Lavallée syndrome and post-traumatic nodular fat necrosis can lead to diagnostic and therapeutic errors while a surgical procedure is necessary since tissue necrosis can occur. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Adapting the Surgical Apgar Score for Perioperative Outcome Prediction in Liver Transplantation: A Retrospective Study

PubMed Central

Pearson, Amy C. S.; Subramanian, Arun; Schroeder, Darrell R.; Findlay, James Y.

2017-01-01

Background The surgical Apgar score (SAS) is a 10-point scale using the lowest heart rate, lowest mean arterial pressure, and estimated blood loss (EBL) during surgery to predict postoperative outcomes. The SAS has not yet been validated in liver transplantation patients, because typical blood loss usually exceeds the highest EBL category. Our primary aim was to develop a modified SAS for liver transplant (SAS-LT) by replacing the EBL parameter with volume of red cells transfused. We hypothesized that the SAS-LT would predict death or severe complication within 30 days of transplant with similar accuracy to current scoring systems. Methods A retrospective cohort of consecutive liver transplantations from July 2007 to November 2013 was used to develop the SAS-LT. The predictive ability of SAS-LT for early postoperative outcomes was compared with Model for End-stage Liver Disease, Sequential Organ Failure Assessment, and Acute Physiology and Chronic Health Evaluation III scores using multivariable logistic regression and receiver operating characteristic analysis. Results Of 628 transplants, death or serious perioperative morbidity occurred in 105 (16.7%). The SAS-LT (receiver operating characteristic area under the curve [AUC], 0.57) had similar predictive ability to Acute Physiology and Chronic Health Evaluation III, model for end-stage liver disease, and Sequential Organ Failure Assessment scores (0.57, 0.56, and 0.61, respectively). Seventy-nine (12.6%) patients were discharged from the ICU in 24 hours or less. These patients’ SAS-LT scores were significantly higher than those with a longer stay (7.0 vs 6.2, P < 0.01). The AUC on multivariable modeling remained predictive of early ICU discharge (AUC, 0.67). Conclusions The SAS-LT utilized simple intraoperative metrics to predict early morbidity and mortality after liver transplant with similar accuracy to other scoring systems at an earlier postoperative time point. PMID:29184910

Cognitive functioning in children and adults with Smith-Magenis syndrome.

PubMed

Osório, Ana; Cruz, Raquel; Sampaio, Adriana; Garayzábal, Elena; Carracedo, Angel; Fernández-Prieto, Montse

2012-06-01

Smith-Magenis Syndrome (SMS) is a genetic neurodevelopmental disorder caused by a microdeletion on chromosome 17p11.2. This syndrome is characterized by a distinctive profile of physical, medical and neuropsychological characteristics. The latter include general mental disability, with the majority of individuals falling within the mild to moderate range. This study reports a detailed cognitive assessment of children and adults with SMS with the use of the Wechsler intelligence scales at three distinct levels of analysis: full scale IQ, factorial indices, and subtests. Child and adult samples were each compared to samples of age and gender-matched typically developing individuals. To our knowledge, this is the first study to systematically analyse the cognitive profile of individuals with SMS in Southern Europe. The present study confirmed mental disability, particularly within the moderate category, as a consistent feature of children and adults with SMS. Furthermore, both child and adult samples evidenced significant impairments in all four indices when compared with their typically developing counterparts. A specific pattern of strengths and weaknesses was discernible for both samples, with Verbal Comprehension emerging as a relative strength, whereas Working Memory appeared as a relative weakness. Finally, with the exception of two subtests in the perceptual domain, we found no evidence for a general cognitive decline with age. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

Digital disruption ?syndromes.

PubMed

Sullivan, Clair; Staib, Andrew

2017-05-18

The digital transformation of hospitals in Australia is occurring rapidly in order to facilitate innovation and improve efficiency. Rapid transformation can cause temporary disruption of hospital workflows and staff as processes are adapted to the new digital workflows. The aim of this paper is to outline various types of digital disruption and some strategies for effective management. A large tertiary university hospital recently underwent a rapid, successful roll-out of an integrated electronic medical record (EMR). We observed this transformation and propose several digital disruption "syndromes" to assist with understanding and management during digital transformation: digital deceleration, digital transparency, digital hypervigilance, data discordance, digital churn and post-digital 'depression'. These 'syndromes' are defined and discussed in detail. Successful management of this temporary digital disruption is important to ensure a successful transition to a digital platform. What is known about this topic? Digital disruption is defined as the changes facilitated by digital technologies that occur at a pace and magnitude that disrupt established ways of value creation, social interactions, doing business and more generally our thinking. Increasing numbers of Australian hospitals are implementing digital solutions to replace traditional paper-based systems for patient care in order to create opportunities for improved care and efficiencies. Such large scale change has the potential to create transient disruption to workflows and staff. Managing this temporary disruption effectively is an important factor in the successful implementation of an EMR. What does this paper add? A large tertiary university hospital recently underwent a successful rapid roll-out of an integrated electronic medical record (EMR) to become Australia's largest digital hospital over a 3-week period. We observed and assisted with the management of several cultural, behavioural and

Causes of metabolic syndrome and obesity-related co-morbidities Part 1: A composite unifying theory review of human-specific co-adaptations to brain energy consumption

PubMed Central

2014-01-01

One line summary Metabolic syndrome and obesity-related co-morbidities are largely explained by co-adaptations to the energy use of the large human brain in the cortico-limbic-striatal and NRF2 systems. The medical, research and general community is unable to effect significantly decreased rates of central obesity and related type II diabetes mellitus (TIIDM), cardiovascular disease (CVD) and cancer. All conditions seem to be linked by the concept of the metabolic syndrome (MetS), but the underlying causes are not known. MetS markers may have been mistaken for causes, thus many treatments are destined to be suboptimal. The current paper aims to critique current paradigms, give explanations for their persistence, and to return to first principles in an attempt to determine and clarify likely causes of MetS and obesity related comorbidities. A wide literature has been mined, study concepts analysed and the basics of human evolution and new biochemistry reviewed. A plausible, multifaceted composite unifying theory is formulated. The basis of the theory is that the proportionately large, energy-demanding human brain may have driven co-adaptive mechanisms to provide, or conserve, energy for the brain. A ‘dual system’ is proposed. 1) The enlarged, complex cortico-limbic-striatal system increases dietary energy by developing strong neural self-reward/motivation pathways for the acquisition of energy dense food, and (2) the nuclear factor-erythroid 2-related factor 2 (NRF2) cellular protection system amplifies antioxidant, antitoxicant and repair activity by employing plant chemicals, becoming highly energy efficient in humans. The still-evolving, complex human cortico-limbic-striatal system generates strong behavioural drives for energy dense food procurement, including motivating agricultural technologies and social system development. Addiction to such foods, leading to neglect of nutritious but less appetizing ‘common or garden’ food, appears to have occurred

SPSS and SAS procedures for estimating indirect effects in simple mediation models.

PubMed

Preacher, Kristopher J; Hayes, Andrew F

2004-11-01

Researchers often conduct mediation analysis in order to indirectly assess the effect of a proposed cause on some outcome through a proposed mediator. The utility of mediation analysis stems from its ability to go beyond the merely descriptive to a more functional understanding of the relationships among variables. A necessary component of mediation is a statistically and practically significant indirect effect. Although mediation hypotheses are frequently explored in psychological research, formal significance tests of indirect effects are rarely conducted. After a brief overview of mediation, we argue the importance of directly testing the significance of indirect effects and provide SPSS and SAS macros that facilitate estimation of the indirect effect with a normal theory approach and a bootstrap approach to obtaining confidence intervals, as well as the traditional approach advocated by Baron and Kenny (1986). We hope that this discussion and the macros will enhance the frequency of formal mediation tests in the psychology literature. Electronic copies of these macros may be downloaded from the Psychonomic Society's Web archive at www.psychonomic.org/archive/.

Celestial diffuse gamma radiation above 30 MeV observed by SAS-2

NASA Technical Reports Server (NTRS)

Fichtel, C. E.; Kniffen, D. A.; Hartman, R. C.

1973-01-01

The Small Astronomy Satellite (SAS)-2, launched on November 15, 1972, carried into orbit a 32-deck magnetic-core digitized spark chamber gamma ray telescope to study celestial gamma radiation in the energy range above 30 MeV. In the study of several regions with b sub 2 15 deg, a finite, diffuse flux of gamma rays with a steep energy spectrum in the energy region from 35 to 200 MeV is observed. Representing the energy spectrum by a power law of the form dJ/dE = AE to - alpha power over this energy range, alpha is found along with the integral flux above 100 MeV. Combining this result with existing low energy gamma ray data yields an energy spectrum which is not a simple power law in energy, as in the X-ray region, but which demonstrates first an increase and then a decrease in slope, consistent within uncertainties with that predicted by cosmological theories, including the continuous production of high energy gamma rays primarily from neutral pi mesons throughout the history of the universe.

Incidence of Radiologically Isolated Syndrome: A Population-Based Study.

PubMed

Forslin, Y; Granberg, T; Jumah, A Antwan; Shams, S; Aspelin, P; Kristoffersen-Wiberg, M; Martola, J; Fredrikson, S

2016-06-01

Incidental MR imaging findings resembling MS in asymptomatic individuals, fulfilling the Okuda criteria, are termed "radiologically isolated syndrome." Those with radiologically isolated syndrome are at high risk of their condition converting to MS. The epidemiology of radiologically isolated syndrome remains largely unknown, and there are no population-based studies, to our knowledge. Our aim was to study the population-based incidence of radiologically isolated syndrome in a high-incidence region for MS and to evaluate the effect on radiologically isolated syndrome incidence when revising the original radiologically isolated syndrome criteria by using the latest radiologic classification for dissemination in space. All 2272 brain MR imaging scans in 1907 persons obtained during 2013 in the Swedish county of Västmanland, with a population of 259,000 inhabitants, were blindly evaluated by a senior radiologist and a senior neuroradiologist. The Okuda criteria for radiologically isolated syndrome were applied by using both the Barkhof and Swanton classifications for dissemination in space. Assessments of clinical data were performed by a radiology resident and a senior neurologist. The cumulative incidence of radiologically isolated syndrome was 2 patients (0.1%), equaling an incidence rate of 0.8 cases per 100,000 person-years, in a region with an incidence rate of MS of 10.2 cases per 100,000 person-years. There was no difference in the radiologically isolated syndrome incidence rate when applying a modified version of the Okuda criteria by using the newer Swanton classification for dissemination in space. Radiologically isolated syndrome is uncommon in a high-incidence region for MS. Adapting the Okuda criteria to use the dissemination in space-Swanton classification may be feasible. Future studies on radiologically isolated syndrome may benefit from a collaborative approach to ensure adequate numbers of participants. © 2016 by American Journal of Neuroradiology.

Physical Activity Engagement in Young People with Down Syndrome: Investigating Parental Beliefs.

PubMed

Alesi, Marianna; Pepi, Annamaria

2017-01-01

Despite the wide documentation of the physical/psychological benefits derived from regular physical activity (PA), high levels of inactivity are reported among people with Down syndrome. This study aims to explore parental beliefs concerning involvement, facilitators/barriers and benefits of PA in young people. Semi-structured interviews were conducted with 13 parents of young people with Down syndrome. Three facilitation themes were identified: (i) the support derived from family; (ii) the availability of APA (Adapted Physical Activity) expert instructors and coaches; (iii) the challenging nature of sport activities. Three barrier themes were identified: (i) the lack of APA expert coaches and specialized gyms; (ii) the characteristics of Down syndrome; and (iii) the parental beliefs and worries. Family plays a key role, as facilitator and barrier, to the participation of their children with Down syndrome in PA. Crucial is the implementation of evidence-based exercise programmes involving people with Down syndrome and their families. © 2015 John Wiley & Sons Ltd.

[Diogenes syndrome and Hoarding disorder: Same or different?

PubMed

Lavigne, B; Hamdan, M; Faure, B; Merveille, H; Pareaud, M; Tallon, E; Bouthier, A; Clément, J-P; Calvet, B

2016-10-01

patients suffering from excessive hoarding had a relative with a dimension of hoarding. Finally, Mattaix-Cols et al. decided to create a new syndrome in the DSM-5, and the Hoarding disorder was born. The discussion begins with relationships between the Hoarding disorder and the Diogenes syndrome. A patient with hoarding, and a poor insight, could be very isolated, and could persist in a lack of calling for help, because of not being aware of his pathology. Thus, it could be diagnosed as a Hoarding syndrome with a poor insight, or as a Diogenes syndrome, with the first major criterion (lack of calling for help) and one of the three minor criteria, the syllogomania, or hoarding. Moreover, some authors have described old people living for many years with a tendency to hoard. Progressively, some of them had a congested and cluttered home, and a few were living in squalor, a description very close to the Diogenes syndrome. Finally, we discuss the comorbidity of Hoarding disorder and Diogenes syndrome. In particular, the first one is associated with Attention deficit and hyperactivity disorders; and some authors also described the links between ADHD, bipolar disorder and frontotemporal dementia which is one of the aetiologies of the Diogenes syndrome. A psychodynamic model in which ADHD, Hoarding disorder and Diogenes syndrome are linked can be imagined, and the last one could be an overlooked evolution of the two first syndromes. In conclusion, we can imagine a dimensional model, based on two dimensions: hoarding and squalor. Hoarding disorder is the major expression of the first dimension, and Diogenes syndrome the major expression of the second. Both of them could be a different expression of one central aetiology. More studies are needed to complete this vision. Copyright © 2016 L'Encéphale, Paris. Published by Elsevier Masson SAS. All rights reserved.

[McCune-Albright syndrome revealed by Blaschko-linear café-au-lait spots on the back].

PubMed

Jung, A-J; Soskin, S; Paris, F; Lipsker, D

2016-01-01

McCune-Albright syndrome is a rare sporadic disease defined by the triad of café-au-lait spots, fibrous dysplasia of bone and endocrine disorder. Diagnosis is classically confirmed by the presence of bone lesions or precocious puberty. We report a case of McCune-Albright syndrome diagnosed solely on the basis of the cutaneous signs. A four-year-old girl was seen in our clinic due to the presence of congenital café-au-lait spots on her back. These macules were irregular, with jagged borders, and were disposed in a broad band on the left shoulder and in the lumbar region, in a Blaschko-linear pattern. McCune-Albright syndrome was immediately suspected, despite the absence of other signs of the disease. Genetic assessment carried out a year and a half later confirmed the diagnosis, with arginine substitution at position 201 of Gs alpha protein. The child was still asymptomatic. Regular radiographic and endocrine assessments remained normal for three years until the sudden appearance at the age of seven years of precocious puberty and radiographic evidence of fibrous dysplasia of the right hand. Café-au-lait spots are very common in the general population. An underlying genetic disorder should only be sought when such spots are multiple. However, in the case of McCune-Albright syndrome, it is the irregular borders and the Blaschko-linear arrangement of the spots in broad irregular bands that are pathognomonic, reflecting as they do the genetic mosaicism characteristic of this disease. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Photosynthetic adaptation strategy of Ulva prolifera floating on the sea surface to environmental changes.

PubMed

Zhao, Xinyu; Tang, Xuexi; Zhang, Huanxin; Qu, Tongfei; Wang, Ying

2016-10-01

For 8 consecutive years, a green tide has originated in the southern Yellow Sea and spread to the Qingdao offshore area. The causative species, Ulva prolifera, always forms a very thick thallus mat that is capable of drifting long distances over long periods. During this process, although the thalli face disturbance by complex environmental factors, they maintain high biomass and proliferation. We hypothesized that some form of photosynthetic adaptation strategy must exist to protect the thalli. Therefore, we studied the different photosynthetic response characteristics of the surface and lower layers of the floating thallus mats, and investigated the physiological and molecular-level adaptation mechanisms. The results showed that: (1) U. prolifera has strong photosynthetic capability that ensures it can gain sufficient energy to increase its biomass and adapt to long-distance migration. (2) Surface layer thalli adapt to the complex environment by dissipating excess energy via photosynthetic quantum control (energy quenching and energy redistribution between PSII/PSI) to avoid irreversible damage to the photosynthetic system. (3) Lower layer thalli increase their contents of Chlorophyll a (Chl a) and Chlorophyll b (Chl b) and decrease their Chl a/Chl b ratio to improve their ability to use light energy. (4) U. prolifera has strong photosynthetic plasticity and can adapt to frequent exchange between the surface and lower layer environments because of wave disturbance. Pigment component changes, energy quenching, and energy redistribution between PSII/PSI contribute to this photosynthetic plasticity. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

An Integrated Syndromic Surveillance System for Monitoring Scarlet Fever in Taiwan

PubMed Central

Wu, Wan-Jen; Liu, Yu-Lun; Kuo, Hung-Wei; Huang, Wan-Ting; Yang, Shiang-Lin; Chuang, Jen-Hsiang

2013-01-01

Objective To develop an integrated syndromic surveillance system for timely monitoring and early detection of unusual situations of scarlet fever in Taiwan, since Hong Kong, being so close geographically to Taiwan, had an outbreak of scarlet fever in June 2011. Introduction Scarlet fever is a bacterial infection caused by group A streptococcus (GAS). The clinical symptoms are usually mild. Before October, 2007, case-based surveillance of scarlet fever was conducted through notifiable infectious diseases in Taiwan, but was removed later from the list of notifiable disease because of improved medical care capacities. In 2011, Hong Kong had encountered an outbreak of scarlet fever (1,2). In response, Taiwan developed an integrated syndromic surveillance system using multiple data sources since July 2011. Methods More than 99% of the Taiwan population is covered by National Health Insurance. We first retrospectively evaluated claims data from the Bureau of National Health Insurance (BNHI) by comparing with notifiable diseases reporting data from Taiwan Centers for Disease Control (TCDC). The claims data included information on scarlet fever diagnosis (ICD-9-CM code 034.1), date of visits, location of hospitals and age of patients from outpatient (OPD), emergency room (ER) and hospital admissions. Daily aggregate data of scarlet fever visits or hospitalizations were prospectively collected from BNHI since July 2011. Over 70% of the deaths in Taiwan are reported to the Office of Statistics of Department of Health electronically. We obtained daily data on electronic death certification data and used SAS Enterprise Guide 4.3 (SAS Institute Inc., Cary, NC, USA) for data management and analysis. Deaths associated with scarlet fever or other GAS infections were identified by text mining from causes of death with keywords of traditional Chinese ‘scarlet fever’, ‘group A streptococcus’ or ‘toxic shock syndrome’ (3). Results From January 2006 to September 2007, the

Audiological follow-up of 24 patients affected by Williams syndrome.

PubMed

Barozzi, Stefania; Soi, Daniela; Spreafico, Emanuela; Borghi, Anna; Comiotto, Elisabetta; Gagliardi, Chiara; Selicorni, Angelo; Forti, Stella; Cesarani, Antonio; Brambilla, Daniele

2013-09-01

Williams syndrome is a neurodevelopmental disorder associated with cardiovascular problems, facial abnormalities and several behavioural and neurological disabilities. It is also characterized by some typical audiological features including abnormal sensitivity to sounds, cochlear impairment related to the outer hair cells of the basal turn of the cochlea, and sensorineural or mixed hearing loss, predominantly in the high frequency range. The aim of this report is to describe a follow-up study of auditory function in a cohort of children affected by this syndrome. 24 patients, aged 5-14 years, were tested by means of air/bone conduction pure-tone audiometry, immittance test and transient evoked otoacoustic emissions. They were evaluated again 5 years after the first assessment, and 10 of them underwent a second follow-up examination after a further 5 years. The audiometric results showed hearing loss, defined by a pure tone average >15 dB HL, in 12.5% of the participants. The incidence of hearing loss did not change over the 5-year period and increased to 30% in the patients who underwent the 10-year follow-up. Progressive sensorineural hearing loss was detected in 20% of the patients. A remarkable finding of our study regarded sensorineural hearing impairment in the high frequency range, which increased significantly from 25% to 50% of the participants over the 5-year period. The increase became even more significant in the group of patients who underwent the 10-year follow-up, by which time the majority of them (80%) had developed sensorineural hearing loss. Otoacoustic emissions were found to be absent in a high percentage of patients, thus confirming the cochlear fragility of individuals with Williams syndrome. Our study verified that most of the young Williams syndrome patients had normal hearing sensitivity within the low-middle frequency range, but showed a weakness regarding the high frequencies, the threshold of which worsened significantly over time in

Altered cerebellar feedback projections in Asperger syndrome.

PubMed

Catani, Marco; Jones, Derek K; Daly, Eileen; Embiricos, Nitzia; Deeley, Quinton; Pugliese, Luca; Curran, Sarah; Robertson, Dene; Murphy, Declan G M

2008-07-15

It has been proposed that the biological basis of autism spectrum disorder includes cerebellar 'disconnection'. However, direct in vivo evidence in support of this is lacking. Here, the microstructural integrity of cerebellar white matter in adults with Asperger syndrome was studied using diffusion tensor magnetic resonance tractography. Fifteen adults with Asperger syndrome and 16 age-IQ-gender-matched healthy controls underwent diffusion tensor magnetic resonance imaging. For each subject, tract-specific measurements of mean diffusivity and fractional anisotropy were made within the inferior, middle, superior cerebellar peduncles and short intracerebellar fibres. No group differences were observed in mean diffusivity. However, people with Asperger syndrome had significantly lower fractional anisotropy in the short intracerebellar fibres (p<0.001) and right superior cerebellar (output) peduncle (p<0.001) compared to controls; but no difference in the input tracts. Severity of social impairment, as measured by the Autistic Diagnostic Interview, was negatively correlated with diffusion anisotropy in the fibres of the left superior cerebellar peduncle. These findings suggest a vulnerability of specific cerebellar neural pathways in people with Asperger syndrome. The localised abnormalities in the main cerebellar outflow pathway may prevent the cerebral cortex from receiving those cerebellar feedback inputs necessary for a successful adaptive social behaviour.

Thyroid Allostasis–Adaptive Responses of Thyrotropic Feedback Control to Conditions of Strain, Stress, and Developmental Programming

PubMed Central

Chatzitomaris, Apostolos; Hoermann, Rudolf; Midgley, John E.; Hering, Steffen; Urban, Aline; Dietrich, Barbara; Abood, Assjana; Klein, Harald H.; Dietrich, Johannes W.

2017-01-01

The hypothalamus–pituitary–thyroid feedback control is a dynamic, adaptive system. In situations of illness and deprivation of energy representing type 1 allostasis, the stress response operates to alter both its set point and peripheral transfer parameters. In contrast, type 2 allostatic load, typically effective in psychosocial stress, pregnancy, metabolic syndrome, and adaptation to cold, produces a nearly opposite phenotype of predictive plasticity. The non-thyroidal illness syndrome (NTIS) or thyroid allostasis in critical illness, tumors, uremia, and starvation (TACITUS), commonly observed in hospitalized patients, displays a historically well-studied pattern of allostatic thyroid response. This is characterized by decreased total and free thyroid hormone concentrations and varying levels of thyroid-stimulating hormone (TSH) ranging from decreased (in severe cases) to normal or even elevated (mainly in the recovery phase) TSH concentrations. An acute versus chronic stage (wasting syndrome) of TACITUS can be discerned. The two types differ in molecular mechanisms and prognosis. The acute adaptation of thyroid hormone metabolism to critical illness may prove beneficial to the organism, whereas the far more complex molecular alterations associated with chronic illness frequently lead to allostatic overload. The latter is associated with poor outcome, independently of the underlying disease. Adaptive responses of thyroid homeostasis extend to alterations in thyroid hormone concentrations during fetal life, periods of weight gain or loss, thermoregulation, physical exercise, and psychiatric diseases. The various forms of thyroid allostasis pose serious problems in differential diagnosis of thyroid disease. This review article provides an overview of physiological mechanisms as well as major diagnostic and therapeutic implications of thyroid allostasis under a variety of developmental and straining conditions. PMID:28775711