WorldWideScience

Sample records for bardet-biedl syndrome-associated small

  1. Bardet-Biedl Syndrome

    Science.gov (United States)

    ... Conference Audio/Video Recordings For Researchers & Industry Funding Philosophy Funded Grants & Researchers Apply for Funding Clinical Research ... problems but rarely polydactyly. Polydactyly is a defining feature of Bardet-Biedl syndrome, while neurologic problems almost ...

  2. Genetics Home Reference: Bardet-Biedl syndrome

    Science.gov (United States)

    ... poor visual acuity) and become legally blind by adolescence or early adulthood. Obesity is another characteristic feature of Bardet-Biedl syndrome . Abnormal weight gain typically begins in early childhood and continues to be an issue throughout life. ...

  3. Bardet-biedl syndrome presenting with end stage renal failure

    International Nuclear Information System (INIS)

    Ansari, R.M.; Junejo, A. M.

    2006-01-01

    A young male presented in the Nephro-Urology Department with advanced renal failure, blindness in early childhood, polydactaly,obesity, decreased mentation and hypogonadism. With these phenotypical features and renal ultrasonographic findings, he was diagnosed as a case of Bardet-Biedl syndrome. Only one younger sister of patient had similar features. Renal impairment is frequent and an important cause of death. End stage renal disease (ESRD) is rarely seen in younger patient of Bardet-Biedl syndrome. However, ESRD in early age is associated with substantially reduced survival. (author)

  4. Mutation analysis of the MKKS gene in McKusick-Kaufman syndrome and selected Bardet-Biedl syndrome patients

    NARCIS (Netherlands)

    Slavotinek, A. M.; Searby, C.; Al-Gazali, L.; Hennekam, R. C. M.; Schrander-Stumpel, C.; Orcana-Losa, M.; Pardo-Reoyo, S.; Cantani, A.; Kumar, D.; Capellini, Q.; Neri, G.; Zackai, E.; Biesecker, L. G.

    2002-01-01

    McKusick-Kaufman syndrome comprises hydrometrocolpos, polydactyly, and congenital heart defects and overlaps with Bardet-Biedl syndrome, comprising retinitis pigmentosa, polydactyly, obesity, mental retardation, and renal and genital anomalies. Bardet-Biedl syndrome is genetically heterogeneous with

  5. Bardet-Biedl syndrome presenting with steroid sensitive nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    K K Singh

    2015-01-01

    Full Text Available Bardet-Biedl syndrome (BBS is a rare autosomal recessive disorder characterized by postaxial polydactyly, retinitis pigmentosa, central obesity, mental retardation, hypogonadism, and renal involvement. Renal involvement in various forms has been seen in BBS. Cases with nephrotic range proteinuria not responding to steroid have been described in this syndrome. Here we report a case of BBS who presented with nephrotic range proteinuria. The biopsy findings were suggestive of minimal change disease. The child responded well to steroid therapy and remains in remission.

  6. Bardet-biedl syndrome in a child with chronic kidney disease

    International Nuclear Information System (INIS)

    Valavi, Ehsan; Ahmadzadeh Ali; Ansari, Mohammad Javad Alemzadeh

    2009-01-01

    A 4-year old boy was referred for evaluation of renal failure, posterior urethral valve (PUV) and urinary tract infection. His parents added complaints of polyuria, polydipsia, enuresis, shortness of stature, and inappropriate obesity. Serum blood urea nitrogen and creatinine levels were 45 and 3.5 mg/dL, respectively. Urine culture was positive for Pseudomonas aeruginosa, and abdominal ultrasound revealed bilateral small kidneys. The patient's history included mild to moderate mental retardation and postaxial polydactyly of both lower limbs amputated two years ago. The combination of mental retardation, obesity, postaxial polydactyly, and bilateral renal hypoplasia were compatible with the diagnosis Bardet-Biedl syndrome (BBS). The combination of PUV and BBS is a rare condition that caused this early onset of renal failure and inappropriate obesity guided us to the diagnosis. (author)

  7. Genetics of human Bardet-Biedl syndrome, an updates.

    Science.gov (United States)

    Khan, S A; Muhammad, N; Khan, M A; Kamal, A; Rehman, Z U; Khan, S

    2016-07-01

    Bardet-Biedl syndrome (BBS) is an autosomal recessive multisystemic human genetic disorder characterized by six major defects including obesity, mental retardation, renal anomalies, polydactyly, retinal degeneration and hypogenitalism. In several cases of BBS, few other features such as metabolic defects, cardiovascular anomalies, speech deficits, hearing loss, hypertension, hepatic defects and high incidence of diabetes mellitus have been reported as well. The BBS displays extensive genetic heterogeneity. To date, 19 genes have been mapped on different chromosomes causing BBS phenotypes having varied mutational load of each BBS gene. In this review, we have discussed clinical spectrum and genetics of BBS. This report presents a concise overview of the current knowledge on clinical data and its molecular genetics progress upto date. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  8. A novel founder BBS1 mutation explains a unique high prevalence of Bardet-Biedl syndrome in the Faroe Islands

    DEFF Research Database (Denmark)

    Hjortshøj, T Duelund; Grønskov, K; Brøndum-Nielsen, K

    2009-01-01

    Bardet-Biedl syndrome is a multiorgan disease presenting with retinitis pigmentosa leading to blindness. The aim of the study was to investigate the genetic background of Bardet-Biedl syndrome in the Faroe Island. It was hypothesised that a common genetic background for the syndrome would be found....

  9. Inactivation of Bardet-Biedl syndrome genes causes kidney defects

    Science.gov (United States)

    Guo, Deng-Fu; Beyer, Andreas M.; Yang, Baoli; Nishimura, Darryl Y.; Sheffield, Val C.

    2011-01-01

    Bardet-Biedl syndrome (BBS) is a rare hereditary autosomal recessive disease associated with several features including obesity, hypertension, and renal abnormalities. The underlying mechanisms of renal defects associated with BBS remain poorly defined. We examined the histological, molecular, and functional renal changes in BBS mouse models that have features of the human disorder. Interestingly, obese hypertensive Bbs4−/− mice exhibited inflammatory infiltration and renal cysts, whereas the obese normotensive Bbs2−/− mice had only minor inflammatory infiltration. Accordingly, the expression level of inducible nitric oxide synthase was elevated in the kidney of both BBS mice with a more marked increase in Bbs4−/− mice. In contrast, endothelial nitric oxide synthase expression was decreased in Bbs4−/−, but not Bbs2−/−, mice. Similarly, the expression levels of transient receptor potential vanilloid 1 and 4 channels as well as β- and γ-subunits of epithelial Na channel were significantly reduced only in the kidney of Bbs4−/− mice. Metabolic studies revealed changes in urine output and urinary concentrations of creatinine, blood urea nitrogen, sodium, and potassium with a more pronounced effect in Bbs4−/− mice. Finally, we found that calorie restriction which prevented obesity in BBS mice reversed the morphological and molecular changes found in Bbs2−/− and Bbs4−/− mice, indicating the kidney abnormalities associated with BBS are obesity related. These findings extend our understanding of the function of BBS proteins and emphasize the importance of these proteins in renal physiology. PMID:21106857

  10. Bardet-Biedl syndrome: A rare case report from North India

    Directory of Open Access Journals (Sweden)

    Sumir Kumar

    2012-01-01

    Full Text Available The Bardet-Biedl syndrome (BBS is a rare ciliopathic human autosomal-recessive disorder, affecting multiple organ systems. Less than 15 cases have been reported from India. The authors present a classical case of BBS presenting to dermatology outpatient with hypogonadism and features such as marked central obesity, retinal dystrophy, polydactyly, structural renal abnormalities and mental retardation, along with a brief review of the literature. This case exemplifies the need for multidisciplinary management in such cases.

  11. Risk Factors for Severe Renal Disease in Bardet-Biedl Syndrome.

    Science.gov (United States)

    Forsythe, Elizabeth; Sparks, Kathryn; Best, Sunayna; Borrows, Sarah; Hoskins, Bethan; Sabir, Ataf; Barrett, Timothy; Williams, Denise; Mohammed, Shehla; Goldsmith, David; Milford, David V; Bockenhauer, Detlef; Foggensteiner, Lukas; Beales, Philip L

    2017-03-01

    Bardet-Biedl syndrome is a rare autosomal recessive, multisystem disease characterized by retinal dystrophy, renal malformation, obesity, intellectual disability, polydactyly, and hypogonadism. Nineteen disease-causing genes ( BBS1-19 ) have been identified, of which mutations in BBS1 are most common in North America and Europe. A hallmark of the disease, renal malformation is heterogeneous and is a cause of morbidity and mortality through the development of CKD. We studied the prevalence and severity of CKD in 350 patients with Bardet-Biedl syndrome-related renal disease attending the United Kingdom national Bardet-Biedl syndrome clinics to further elucidate the phenotype and identify risk indicators of CKD. Overall, 31% of children and 42% of adults had CKD; 6% of children and 8% of adults had stage 4-5 CKD. In children, renal disease was often detected within the first year of life. Analysis of the most commonly mutated disease-associated genes revealed that, compared with two truncating mutations, two missense mutations associated with less severe CKD in adults. Moreover, compared with mutations in BBS10 , mutations in BBS1 associated with less severe CKD or lack of CKD in adults. Finally, 51% of patients with available ultrasounds had structural renal abnormalities, and 35% of adults were hypertensive. The presence of structural abnormalities or antihypertensive medication also correlated statistically with stage 3b-5 CKD. This study describes the largest reported cohort of patients with renal disease in Bardet-Biedl syndrome and identifies risk factors to be considered in genetic counseling. Copyright © 2017 by the American Society of Nephrology.

  12. Genetic linkage analysis in 26 families with Bardet-Biedl syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Wright, A.F.; Bruford, E.A.; Mansfield, D.C. [Western General Hospital, Edinburgh (United Kingdom)] [and others

    1994-09-01

    Bardet-Biedl syndrome is an autosomal recessive disorder characterized by polydactyly, obesity, hypogonadism, retinitis pigmentosa, renal anomalies and mental retardation. Clinical heterogeneity is quite marked both within and between families. Linkage has been reported between Bardet-Biedl syndrome and the D16408 marker in chromosomal region 16q21 in an extended Bedouin kindred and, more recently, in a subset of 17 out of 31 families using the PYGM/D11S913 markers in chromosomal region 11q13. We have analyzed linkage to the 16q21 and 11q13 regions and used markers covering chromosomes 2, 3, 17 and 18 in a set of 26 Bardet-Biedl families, each containing at least two affected individuals, with a total of 57 affected members. Evidence of linkage to the D11S527 locus has been identified assuming linkage homogeneity with a lod score of 2.72 at a recombination fraction of 0.11 (95% limits 0.03-0.25).

  13. Pigmentary retinopathy due to Bardet-Biedl syndrome: case report and literature review Retinopatia pigmentar devido a síndrome de Bardet-Biedl: relato de caso e revisão da literatura

    Directory of Open Access Journals (Sweden)

    Luis Jesuino de Oliveira Andrade

    2009-10-01

    Full Text Available Bardet-Biedl syndrome (BBS is a rare autosomal recessive disorder with clinical and genetic heterogeneity. This syndrome was first described by Laurence and Moon in 1866 and additional cases were described by Bardet and Biedl between 1920 and 1922. The main features are obesity, polydactyly, pigmentary retinopathy, learning disabilities, various degrees of intellectual impairment, hypogonadism, and renal abnormalities. Bardet-Biedl syndrome is both phenotypically and genetically heterogeneous. Clinical diagnosis is based on the presence of 4 of the 5 cardinal features. The authors present a typical case of pigmentary retinopathy due to Bardet-Biedl syndrome and made a brief commentary about the disease's cardinal manifestations.A síndrome de Bardet-Biedl (BBS é uma desordem autossômica recessiva rara, com heterogeneidade clínica e genética. Esta síndrome foi descrita pela primeira vez por Laurence e Moon em 1866 e outros casos foram descritos por Bardet e Biedl entre 1920 e 1922. As principais características são obesidade, polidactilia, retinopatia pigmentar, dificuldades de aprendizagem, graus de deficiência intelectual diversos, hipogonadismo e anomalias renais. Síndrome de Bardet-Biedl é fenotipicamente e geneticamente heterogêneos. O diagnóstico clínico baseia-se na presença de quatro dos cinco sinais principais da síndrome. Os autores apresentam um caso típico de retinopatia pigmentar devido à síndrome de Bardet-Biedl e fazem uma breve revisão sobre as manifestações da síndrome com especial atenção à retinopatia pigmentar.

  14. Visual acuity and retinal function in patients with Bardet-Biedl syndrome

    Directory of Open Access Journals (Sweden)

    Adriana Berezovsky

    2012-01-01

    Full Text Available OBJECTIVE: Bardet-Biedl syndrome is a genetic, multisystem disorder that causes severe visual impairment. This condition is characterized by retinal dystrophy, obesity, digit anomalies, renal disease, and hypogonadism. The purpose of this study was to analyze visual acuity and full-field electroretinogram findings in patients with the Bardet-Biedl syndrome phenotype. METHODS: The visual acuity of a group of 23 patients (15 males with ages ranging from 6-36 years (mean = 15.8±6.4; median = 14.7 was assessed. Retinal function was evaluated by full-field electroretinography, and dark-adapted thresholds were assessed. RESULTS: Visual acuity in the better-seeing eye was 20/40 or better in 5 patients (21.7%, 20/50-20/150 in 13 (56.5% patients, 20/200-20/400 in 2 (8.7% patients and worse than 20/400 in one (4.3% patient. The mean acuity in the better-seeing eye was 0.7±0.6 logMAR (20/100, Snellen equivalent. Scotopic rod and maximal responses were nondetectable in 21 (91.3% patients, and cone responses were non-detectable in 15 (65.2% patients. Elevated darkadapted visual thresholds were observed in all 19 patients who were able to be assessed, with 10 (52.6% patients having thresholds greater than 30 dB. CONCLUSIONS: In a relatively young cohort of patients with Bardet-Biedl syndrome, only 21% had 20/40 or better vision. ERG scotopic responses were absent in the majority of cases, with cone responses being observed in less than half of cases. These findings showed the early deleterious effects in retinal function and visual acuity caused by this condition.

  15. Characterization of Courtesy Stigma Perceived by Parents of Overweight Children with Bardet-Biedl Syndrome

    Science.gov (United States)

    Hamlington, Barbara; Ivey, Lauren E.; Brenna, Ethan; Biesecker, Leslie G.; Biesecker, Barbara B.; Sapp, Julie C.

    2015-01-01

    Background A child’s obesity is generally perceived by the public to be under the control of the child’s parents. While the health consequences of childhood obesity are well understood, less is known about psychological and social effects of having an obese child on parents. We set out to characterize stigma and courtesy stigma experiences surrounding obesity among children with Bardet-Biedl syndrome (BBS), a multisystem genetic disorder, and their parents. Methods Twenty-eight parents of children with BBS participated in semi-structured interviews informed by social stigmatization theory, which describes courtesy stigma as parental perception of stigmatization by association with a stigmatized child. Parents were asked to describe such experiences. Results Parents of children with BBS reported the child’s obesity as the most frequent target of stigmatization. They perceived health care providers as the predominant source of courtesy stigma, describing interactions that resulted in feeling devalued and judged as incompetent parents. Conclusions Parents of children with BBS feel blamed by others for their child’s obesity and described experiences that suggest health care providers may contribute to courtesy stigma and thus impede effective communication about managing obesity. Health care providers may reinforce parental feelings of guilt and responsibility by repeating information parents may have previously heard and ignoring extremely challenging barriers to weight management, such as a genetic predisposition to obesity. Strategies to understand and incorporate parents’ perceptions and causal attributions of their children’s weight may improve communication about weight control. PMID:26473736

  16. Characterization of Courtesy Stigma Perceived by Parents of Overweight Children with Bardet-Biedl Syndrome.

    Directory of Open Access Journals (Sweden)

    Barbara Hamlington

    Full Text Available A child's obesity is generally perceived by the public to be under the control of the child's parents. While the health consequences of childhood obesity are well understood, less is known about psychological and social effects of having an obese child on parents. We set out to characterize stigma and courtesy stigma experiences surrounding obesity among children with Bardet-Biedl syndrome (BBS, a multisystem genetic disorder, and their parents.Twenty-eight parents of children with BBS participated in semi-structured interviews informed by social stigmatization theory, which describes courtesy stigma as parental perception of stigmatization by association with a stigmatized child. Parents were asked to describe such experiences.Parents of children with BBS reported the child's obesity as the most frequent target of stigmatization. They perceived health care providers as the predominant source of courtesy stigma, describing interactions that resulted in feeling devalued and judged as incompetent parents.Parents of children with BBS feel blamed by others for their child's obesity and described experiences that suggest health care providers may contribute to courtesy stigma and thus impede effective communication about managing obesity. Health care providers may reinforce parental feelings of guilt and responsibility by repeating information parents may have previously heard and ignoring extremely challenging barriers to weight management, such as a genetic predisposition to obesity. Strategies to understand and incorporate parents' perceptions and causal attributions of their children's weight may improve communication about weight control.

  17. Characterization of Courtesy Stigma Perceived by Parents of Overweight Children with Bardet-Biedl Syndrome.

    Science.gov (United States)

    Hamlington, Barbara; Ivey, Lauren E; Brenna, Ethan; Biesecker, Leslie G; Biesecker, Barbara B; Sapp, Julie C

    2015-01-01

    A child's obesity is generally perceived by the public to be under the control of the child's parents. While the health consequences of childhood obesity are well understood, less is known about psychological and social effects of having an obese child on parents. We set out to characterize stigma and courtesy stigma experiences surrounding obesity among children with Bardet-Biedl syndrome (BBS), a multisystem genetic disorder, and their parents. Twenty-eight parents of children with BBS participated in semi-structured interviews informed by social stigmatization theory, which describes courtesy stigma as parental perception of stigmatization by association with a stigmatized child. Parents were asked to describe such experiences. Parents of children with BBS reported the child's obesity as the most frequent target of stigmatization. They perceived health care providers as the predominant source of courtesy stigma, describing interactions that resulted in feeling devalued and judged as incompetent parents. Parents of children with BBS feel blamed by others for their child's obesity and described experiences that suggest health care providers may contribute to courtesy stigma and thus impede effective communication about managing obesity. Health care providers may reinforce parental feelings of guilt and responsibility by repeating information parents may have previously heard and ignoring extremely challenging barriers to weight management, such as a genetic predisposition to obesity. Strategies to understand and incorporate parents' perceptions and causal attributions of their children's weight may improve communication about weight control.

  18. [Algorithm for the molecular analysis of Bardet-Biedl syndrome in Spain].

    Science.gov (United States)

    Castro-Sánchez, Sheila; Álvarez-Satta, María; Pereiro, Inés; Piñeiro-Gallego, M Teresa; Valverde, Diana

    2015-08-21

    Bardet-Biedl syndrome (BBS) is a multisystemic genetic disorder, which is not widespread among the Caucasian population, characterized by a highly variable phenotype and great genetic heterogeneity. BBS belongs to a group of diseases called ciliopathies, caused by defects in the structure and/or function of cilia. Due to the diagnostic complexity of the syndrome, the objective of this study was to analyse our whole group of patients in order to create an algorithm to facilitate the routine molecular diagnosis of BBS. We also calculated several epidemiological parameters in our cohort. We analysed 116 BBS patients belonging to 89 families from the whole Spanish geography. All probands fulfilled diagnosis criteria established for BBS. For this, we used: genotyping microarray, direct sequencing and homozygosis mapping (in consanguineous families). By means of the different approaches, it was possible to diagnose 47% of families (21% by genotyping microarray, 18% by direct sequencing of predominant BBS genes, and 8% by homozygosis mapping). With regard to epidemiological data, a prevalence value of 1:407,000 was obtained for BBS in Spain, and a sex ratio of 1.4:1 (men:women). The proposed algorithm, based on the analysis of predominant BBS genes combined with homozygosis mapping, allowed us to confirm the molecular diagnosis in a significant percentage of families with clinically suspected BBS. This diagnostic algorithm will be useful for the improvement of the efficiency of molecular analysis in BBS. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

  19. [Elizabethkingia meningosepticum bacteremia in a patient with Bardet-Biedl syndrome and chronic renal failure].

    Science.gov (United States)

    Bayrak, Burcu; Fıncanci, Muzaffer; Bınay, Umut Devrim; Çımen, Cansu; Özkantar Ünlügüneş, Gülay Ulkü

    2014-07-01

    Elizabethkingia meningosepticum, a gram-negative opportunistic pathogen may cause life-threatening nosocomial infections especially in newborns and immunosuppressive patients. This bacterium has a peculiar antibiotic resistance profile. It is resistant to most of the antibiotics against gram-negative bacteria and susceptible to antibiotics that are used to treat gram-positive bacteria, such as vancomycin and trimethoprim-sulphamethoxazole (SXT). For this reason appropriate treatment of E.meningosepticum infections are based on the proper identification of bacteria. In this report, a case of catheter-related E.meningosepticum bacteremia in a patient with chronic renal failure due to Bardet-Biedl syndrome, a genetic disorder characterized by multiorgan dysfunction, was presented. A 25-year-old male patient with Bardet-Biedl syndrome was admitted to the emergency room with the complaints of high fever with shivers that started the day before. The patient had a femoral dialysis catheter. Venous blood samples drawn at the time of administration were cultured immediately. Two days later, blood cultures which yielded positive signals were passaged onto blood and MacConkey agar plates and after incubation at 37°C for 16 hours, wet-raised colonies with clear margin, gray colour and large size similar to gram-negative bacterial colonies were detected on blood agar medium. No growth was observed on MacConkey agar plate at the end of five days. The isolate was found positive for KOH, oxidase, catalase, urease, esculine and MOI (Motility Indole Ornithine) tests, whereas it was citrate negative. Gram staining revealed faintly stained thin gram-negative bacilli. The isolate was identified as E.meningosepticum by Vitek® 2 system (bioMérieux, USA), and confirmed by sequence analysis of 16S RNA gene region amplified with PCR method. The antibiotic susceptibility profile of the strain was detected by the Vitek 2 system, while vancomycin susceptibility was investigated by Kirby

  20. Bardet-Biedl syndrome with end-stage kidney disease in a four-year-old Romanian boy: a case report

    Directory of Open Access Journals (Sweden)

    Marshall Jan D

    2011-08-01

    Full Text Available Abstract Background Bardet-Biedl syndrome is a significant genetic cause of chronic kidney disease in children. Kidney abnormalities are a major cause of morbidity and mortality in Bardet-Biedl syndrome, but the onset of end-stage renal disease at an early age and continuous ambulatory peritoneal dialysis, however, are not commonly mentioned in the literature. Case presentation We present the case of a four-year-old Romanian boy who presented to our department with 'febrile seizures'. After an initial evaluation, we diagnosed our patient as having hypertension, severe anemia and end-stage renal disease. He met the major and minor criteria for the diagnosis of Bardet-Biedl syndrome and underwent continuous ambulatory peritoneal dialysis. Conclusions Close follow-up for renal involvement in patients with Bardet-Biedl syndrome and Alström syndrome from an early age is highly recommended to prevent end-stage renal disease and so renal replacement therapy can be started immediately.

  1. EURO-WABB: an EU rare diseases registry for Wolfram syndrome, Alström syndrome and Bardet-Biedl syndrome.

    Science.gov (United States)

    Farmer, Amy; Aymé, Ségolène; de Heredia, Miguel Lopez; Maffei, Pietro; McCafferty, Susan; Młynarski, Wojciech; Nunes, Virginia; Parkinson, Kay; Paquis-Flucklinger, Véronique; Rohayem, Julia; Sinnott, Richard; Tillmann, Vallo; Tranebjaerg, Lisbeth; Barrett, Timothy G

    2013-08-27

    Wolfram, Alström and Bardet-Biedl (WABB) syndromes are rare diseases with overlapping features of multiple sensory and metabolic impairments, including diabetes mellitus, which have caused diagnostic confusion. There are as yet no specific treatments available, little or no access to well characterized cohorts of patients, and limited information on the natural history of the diseases. We aim to establish a Europe-wide registry for these diseases to inform patient care and research. EURO-WABB is an international multicenter large-scale observational study capturing longitudinal clinical and outcome data for patients with WABB diagnoses. Three hundred participants will be recruited over 3 years from different sites throughout Europe. Comprehensive clinical, genetic and patient experience data will be collated into an anonymized disease registry. Data collection will be web-based, and forms part of the project's Virtual Research and Information Environment (VRIE). Participants who haven't undergone genetic diagnostic testing for their condition will be able to do so via the project. The registry data will be used to increase the understanding of the natural history of WABB diseases, to serve as an evidence base for clinical management, and to aid the identification of opportunities for intervention to stop or delay the progress of the disease. The detailed clinical characterisation will allow inclusion of patients into studies of novel treatment interventions, including targeted interventions in small scale open label studies; and enrolment into multi-national clinical trials. The registry will also support wider access to genetic testing, and encourage international collaborations for patient benefit.

  2. A Splice Variant of Bardet-Biedl Syndrome 5 (BBS5 Protein that Is Selectively Expressed in Retina.

    Directory of Open Access Journals (Sweden)

    Susan N Bolch

    Full Text Available Bardet-Biedl syndrome is a complex ciliopathy that usually manifests with some form of retinal degeneration, amongst other ciliary-related deficiencies. One of the genetic causes of this syndrome results from a defect in Bardet-Biedl Syndrome 5 (BBS5 protein. BBS5 is one component of the BBSome, a complex of proteins that regulates the protein composition in cilia. In this study, we identify a smaller molecular mass form of BBS5 as a variant formed by alternative splicing and show that expression of this splice variant is restricted to the retina.Reverse transcription PCR from RNA was used to isolate and identify potential alternative transcripts of Bbs5. A peptide unique to the C-terminus of the BBS5 splice variant was synthesized and used to prepare antibodies that selectively recognized the BBS5 splice variant. These antibodies were used on immunoblots of tissue extracts to determine the extent of expression of the alternative transcript and on tissue slices to determine the localization of expressed protein. Pull-down of fluorescently labeled arrestin1 by immunoprecipitation of the BBS5 splice variant was performed to assess functional interaction between the two proteins.PCR from mouse retinal cDNA using Bbs5-specific primers amplified a unique cDNA that was shown to be a splice variant of BBS5 resulting from the use of cryptic splicing sites in Intron 7. The resulting transcript codes for a truncated form of the BBS5 protein with a unique 24 amino acid C-terminus, and predicted 26.5 kD molecular mass. PCR screening of RNA isolated from various ciliated tissues and immunoblots of protein extracts from these same tissues showed that this splice variant was expressed in retina, but not brain, heart, kidney, or testes. Quantitative PCR showed that the splice variant transcript is 8.9-fold (+/- 1.1-fold less abundant than the full-length transcript. In the retina, the splice variant of BBS5 appears to be most abundant in the connecting cilium

  3. Brain tissue- and region-specific abnormalities on volumetric MRI scans in 21 patients with Bardet-Biedl syndrome (BBS

    Directory of Open Access Journals (Sweden)

    Johnston Jennifer

    2011-07-01

    Full Text Available Abstract Background Bardet-Biedl syndrome (BBS is a heterogeneous human disorder inherited in an autosomal recessive pattern, and characterized by the primary findings of obesity, polydactyly, hypogonadism, and learning and behavioural problems. BBS mouse models have a neuroanatomical phenotype consisting of third and lateral ventriculomegaly, thinning of the cerebral cortex, and reduction in the size of the corpus striatum and hippocampus. These abnormalities raise the question of whether humans with BBS have a characteristic morphologic brain phenotype. Further, although behavioral, developmental, neurological and motor defects have been noted in patients with BBS, to date, there are limited reports of brain findings in BBS. The present study represents the largest systematic evaluation for the presence of structural brain malformations and/or progressive changes, which may contribute to these functional problems. Methods A case-control study of 21 patients, most aged 13-35 years, except for 2 patients aged 4 and 8 years, who were diagnosed with BBS by clinical criteria and genetic analysis of known BBS genes, and were evaluated by qualitative and volumetric brain MRI scans. Healthy controls were matched 3:1 by age, sex and race. Statistical analysis was performed using SAS language with SAS STAT procedures. Results All 21 patients with BBS were found to have statistically significant region- and tissue-specific patterns of brain abnormalities. There was 1 normal intracranial volume; 2 reduced white matter in all regions of the brain, but most in the occipital region; 3 preserved gray matter volume, with increased cerebral cortex volume in only the occipital lobe; 4 reduced gray matter in the subcortical regions of the brain, including the caudate, putamen and thalamus, but not in the cerebellum; and 5 increased cerebrospinal fluid volume. Conclusions There are distinct and characteristic abnormalities in tissue- and region- specific volumes

  4. Bardet-Biedl syndrome: Mapping of a new locus to chromosome 3 and fine-mapping of the chromosome 16 linked locus

    Energy Technology Data Exchange (ETDEWEB)

    Kwitek-Black, A.E.; Rokhlina, T.; Nishimura, D.Y. [Univ. of Iowa, Iowa City, IA (United States)] [and others

    1994-09-01

    Bardet-Biedl syndrome (BBS) is a heterogeneous autosomal recessive disorder characterized by mental retardation, post-axial polydactyly, obesity, retinitis pigmentosa, and hypogonadism. Other features of this disease include renal and cardiovascular abnormalities and an increased incidence of hypertension and diabetes mellitus. The molecular etiology for BBS is not known. We previously linked BBS to chromosome 16q13 in a large inbred Bedouin family, and excluded this locus in a second large inbred Bedouin family. We now report linkage of this second family to markers on chromosome 3q, proving non-allelic, genetic heterogeneity in the Bedouin population. A third large inbred Bedouin family was excluded from the 3q and 16q BBS loci. In addition to the identification of a new BBS locus on chromosome 3, we have identified and utilized additional short tandem repeat polymorphisms (STRPs) in the 16q BBS region to narrow the candidate interval to 3 cM. Additional recombinant individuals will allow further refinement of the interval. Identification of genes causing BBS has the potential to provide insight into diverse genetic traits and disease processes including obesity, hypertension, diabetes, retinal degeneration, and abnormal limb, renal and cardiac development.

  5. Genetic characterization of Italian patients with Bardet-Biedl syndrome and correlation to ocular, renal and audio-vestibular phenotype: identification of eleven novel pathogenic sequence variants.

    Science.gov (United States)

    Esposito, Gabriella; Testa, Francesco; Zacchia, Miriam; Crispo, Anna Alessia; Di Iorio, Valentina; Capolongo, Giovanna; Rinaldi, Luca; D'Antonio, Marcella; Fioretti, Tiziana; Iadicicco, Pasquale; Rossi, Settimio; Franzè, Annamaria; Marciano, Elio; Capasso, Giovanbattista; Simonelli, Francesca; Salvatore, Francesco

    2017-02-01

    Bardet-Biedl syndrome (BBS) is a rare genetic disorder that features retinal degeneration, obesity, polydactyly, learning disabilities and renal abnormalities. The diagnosis is often missed at birth, the median age at diagnosis being 9 years. In the attempt to shed light on BBS and improve its diagnosis and treatment, we evaluated the genotype-phenotype relationship in patients with a molecular diagnosis of BBS. We analyzed three common BBS genes, BBS1, BBS10 and BBS2, in 25 Italian patients fulfilling the clinical criteria of BBS. In 12 patients, we identified gene-specific biallelic variants and thus correlated genotype to the ophthalmic, renal and audio-vestibular phenotypes. At least one sequence variant was found in 60% of patients. The most common mutated gene was BBS1 followed by BBS10. Of the 17 sequence variants we found, 11 have not previously been associated with BBS. In 12 patients, we identified biallelic pathogenic variants; they had retinitis pigmentosa with early onset of visual impairment. However, retinal dystrophy was less severe in patients with BBS1 than in those with BBS10 variants. Overall, we found a high prevalence of renal dysmorphism and dysfunction. Notably, patients with BBS10 variants had the most severe renal impairment, which resulted in a critical decline in renal function. All the patients who underwent audio-vestibular evaluation had dysfunction of the cochlear outer hair cells, thus confirming the presence of hearing defects. BBS1, BBS2 and BBS10 are major causative genes in Italian BBS patients. BBS10 was associated with the worse outcome in terms of the renal, ocular and audiovestibular phenotypes. Cochlear dysfunction should be included among the hallmarks of BBS.

  6. Bardet-Biedl syndrome in Denmark-report of 13 novel sequence variations in six genes

    DEFF Research Database (Denmark)

    Hjortshøj, Tina Duelund; Grønskov, Karen; Philp, Alisdair R

    2010-01-01

    . Triallelic inheritance has been suggested in about 5% of cases. Forty-nine unrelated BBS patients were screened for mutations by DHPLC analysis in BBS1, BBS2, BBS4, BBS6/MKKS, BBS10, and BBS12. The selected genes either account for more than 5% of the mutational load or are commonly reported in triallelic...... inheritance. Eight patients with only one or no BBS mutation were further investigated by single nucleotide polymorphism (SNP) analysis. In total, mutations were detected in 44 patients. Twenty percent had two mutations in BBS1, 18% in BBS2, 4% in BBS9, 43% in BBS10, and 2% in BBS12. Five patients were...... heterozygous for a sequence variation in BBS6/MKKS. We found eight patients with three sequence variations in two genes, which could be explained by triallelic inheritance, by the prevalence of heterozygous carriers or the third sequence variations representing rare polymorphisms. All changes found in a second...

  7. Cat eye syndrome associated with aganglionosis of the small and large intestine.

    OpenAIRE

    Ward, J; Sierra, I A; D'Croz, E

    1989-01-01

    A newborn male infant is presented with the characteristic phenotype of the cat eye syndrome and a small supernumerary chromosome shorter than a 22. He also had complete absence of parasympathetic ganglion cells throughout the small and large intestine.

  8. Cat eye syndrome associated with aganglionosis of the small and large intestine.

    Science.gov (United States)

    Ward, J; Sierra, I A; D'Croz, E

    1989-01-01

    A newborn male infant is presented with the characteristic phenotype of the cat eye syndrome and a small supernumerary chromosome shorter than a 22. He also had complete absence of parasympathetic ganglion cells throughout the small and large intestine. Images PMID:2585462

  9. Gorlin syndrome associated with small bowel carcinoma and mesenchymal proliferation of the gastrointestinal tract: case report and review of literature

    Directory of Open Access Journals (Sweden)

    Meyer Günther

    2010-07-01

    Full Text Available Abstract Background and Case Presentation A patient with nevoid basal cell carcinoma syndrome (Gorlin syndrome presented with two unusual clinical features, i.e. adenocarcinoma of the small bowel and extensive mesenchymal proliferation of the lower gastrointestinal tract. Conclusions We discuss the possibility that these two features are pathogenetically linked to the formerly undescribed patient's PTCH germ line mutation.

  10. Genetic and bibliographic information: BBS5 [GenLibi

    Lifescience Database Archive (English)

    Full Text Available BBS5 Bardet-Biedl syndrome 5 human Bardet-Biedl Syndrome (MeSH) Nervous System Diseases... (C10) > Central Nervous System Diseases (C10.228) > Brain Diseases (C10.228.140) > Hypothalamic Diseases... (C10.228.140.617) > Bardet-Biedl Syndrome (C10.228.140.617.200) Congenital, Hereditary, and Neonatal Diseases

  11. Bardet-Biedl Syndrome, Crohn Disease, Primary Sclerosing Cholangitis, and Autoantibody Positive Thyroiditis: A Case Report and A Review of a Cohort of BBS Patients

    Directory of Open Access Journals (Sweden)

    Ugur Halac

    2012-01-01

    Full Text Available Bardet-Biedel syndrome (BBS is a rare autosomal recessive, genetically heterogeneous ciliopathy. Although the disease has been described in a patient with psoriasis, individuals with BBS are not known to be at risk of developing autoimmune disorders. Our objective was to describe a 14-year-old patient with BBS who presented with Crohn disease (CD, primary sclerosing cholangitis (PSC, and thyroiditis in the context of a cohort review at Sainte-Justine Hospital and to alert clinicians to the increased risk of autoimmune disorders in these patients. The cohort contained fifteen patients (9 boys, followed from 1968 to 2009 during a median period of 12 years (range 9 months–26 years. Three of the 15 patients (20% developed a chronic autoimmune disease: one had juvenile rheumatoid arthritis; a second one had type 1 diabetes mellitus in association with Hashimoto thyroiditis and psoriasis; a third one developed CD, PSC, and Hashimoto thyroiditis. As chronic autoimmune diseases occurred in 20% of our cohort of children with BBS, it is appropriate to keep this association in mind during the followup.

  12. Lynch syndrome-associated neoplasms

    DEFF Research Database (Denmark)

    Shia, Jinru; Holck, Susanne; Depetris, Giovanni

    2013-01-01

    of such information. This article provides an overview of the development of histopathology and immunohistochemistry in Lynch syndrome-associated tumors, particularly in colorectal and endometrial cancers, and outlines the issues and current status of these specific pathologic aspects in not only the major tumors...

  13. Paraneoplastic chronic demyelinating neuropathy and Lambert-Eaton myasthenic syndrome associated with multiple anti-neural antibodies and small-cell lung cancer.

    Science.gov (United States)

    Rozsa, Csilla; Vincent, Angela; Aranyi, Zsuzsanna; Kovacs, Gabor G; Komoly, Samuel; Illes, Zsolt

    2008-09-30

    Lambert-Eaton myasthenic syndrome (LEMS) developed in a patient with presumed chronic inflammatory demyelinating polyneuropathy (CIDP) and negative chest CT. Since antibodies against both Hu and voltage-gated calcium channel (VGCC) were detected, repeated chest CT was performed, which eventually showed a pulmonary mass lesion. Biopsy revealed small cell lung cancer (SCLC) indicating the importance of repeated chest CT in LEMS even when an existing autoimmune-like disease and negative CT may suggest an autoimmune origin. This is the first report of paraneoplastic CIDP and LEMS associated with anti-Hu, anti-VGCC and SCLC.

  14. Prune belly syndrome associated with incomplete VACTERL

    Directory of Open Access Journals (Sweden)

    Ghritlaharey R

    2007-01-01

    Full Text Available A Prune Belly syndrome with VATER/VACTERL association is an extremely rare. They are either stillborn or die within few days of life, only few such cases have been reported in literature. We are presenting here a male neonate of Prune Belly syndrome associated with incomplete VACTERL with brief review of literature.

  15. Genetic and bibliographic information: ARL6 [GenLibi

    Lifescience Database Archive (English)

    Full Text Available ARL6 ADP-ribosylation factor-like 6 human Bardet-Biedl Syndrome (MeSH) Nervous System Diseases... (C10) > Central Nervous System Diseases (C10.228) > Brain Diseases (C10.228.140) > Hypothalamic Diseases... (C10.228.140.617) > Bardet-Biedl Syndrome (C10.228.140.617.200) Congenital, Hereditary, and Neonatal Diseases

  16. Netherton syndrome associated with idiopathic congenital hemihypertrophy.

    Science.gov (United States)

    Yerebakan, Ozlem; Uğuz, Ayşen; Keser, Ibrahim; Lüleci, Güven; Ciftçioğlu, Mehmet Akif; Başaran, Erdal; Alpsoy, Erkan

    2002-01-01

    Netherton syndrome is a rare genodermatosis comprised of anichthyosiform dermatitis, hair shaft defects, and atopic features. Other problems associated with Netherton syndrome are delayed growth and development, immune abnormalities, recurrent infections, and intermittent aminoaciduria. We describe an 18-month-old girl with Netherton syndrome who had idiopathic congenital hemihypertrophy on her right side with contralateral benign nephromegaly in addition to the characteristic clinical signs of the syndrome. To our knowledge, this is the first case of Netherton syndrome associated with idiopathic congenital hemihypertrophy to be reported.

  17. Dominantly inherited isolated hyperparathyroidism: a syndromic association?

    International Nuclear Information System (INIS)

    Kozlowski, K.; Czerminska-Kowalska, A.; Kulczycka, H.; Rowinska, E.; Pronicka, E.

    1999-01-01

    Dominantly inherited isolated hyperparathyroidism (DIIH) is rare in childhood. It may be the first biochemical abnormality in the multiple endocrine neoplasia type I (MEN I) and type II (MEN II) syndromes. Its clinical course is usually asymptomatic or of low morbidity. Radiographic examination is most often normal. We describe six members of a family with distinctive phenotype and DIIH. Limited systemic symptoms and severe radiographic osteitis fibrosa cystica were further unusual features in this family. The diagnosis of DIIH was made only after a 9-year-old girl developed hypercalcaemic crisis after a pathological femoral fracture. Distinctive phenotype, unusual clinical course and unparalleled radiographic changes suggest a not yet described syndromic association. (orig.)

  18. Stemcell Information: SKIP000063 [SKIP Stemcell Database[Archive

    Lifescience Database Archive (English)

    Full Text Available -Bardet)-Biedl Syndrome 209900 ... -- -- ... Yes No intractable disease-specific iPSC, Bardet-Biedl syndrome... 難病性疾患克服事業iPS細胞バンク Bardet-Biedl syndrome human ES-like -- Sendai virus Sendai Virus (DNAVEC: CytoTuneTM-i... SKIP000063 ... Diseased A000088#12 A000088#12 ... ローレンス・ムーン症候群 Q878 Laurence-Moon(

  19. Epidermal Nevus Syndrome Associated with Brain Malformations and Medulloblastoma

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2013-01-01

    Full Text Available Researchers at Juntendo University and Tokyo Women’s Medical University, Japan; and University of California, San Francisco, Ca, report a male infant with epidermal nevus syndrome associated with brainstem and cerebellar malformations and neonatal medulloblastoma.

  20. UAB HRFD Core Center: Core A: The Hepato/Renal Fibrocystic Diseases Translational Resource

    Science.gov (United States)

    2017-09-15

    Hepato/Renal Fibrocystic Disease; Autosomal Recessive Polycystic Kidney Disease; Joubert Syndrome; Bardet Biedl Syndrome; Meckel-Gruber Syndrome; Congenital Hepatic Fibrosis; Caroli Syndrome; Oro-Facial-Digital Syndrome Type I; Nephronophthisis; Glomerulocystic Kidney Disease

  1. Muir-Torre syndrome-associated pleomorphic liposarcoma arising in a previous radiation field.

    Science.gov (United States)

    Yozu, Masato; Symmans, Pennie; Dray, Michael; Griffin, Jennifer; Han, Catherine; Ng, Daniel; Parry, Susan; Wong, Kp

    2013-03-01

    Muir-Torre syndrome is a variant of Lynch syndrome, characterised by sebaceous neoplasia and/or keratoacanthomas associated with visceral malignancies. Muir-Torre syndrome is caused by germline mutations of one of the mismatch repair genes, frequently MSH2 and less frequently MLH1 and MSH6. Visceral malignancies associated with Muir-Torre syndrome and Lynch syndrome include colorectal, endometrial and other gastrointestinal, urological and gynaecological malignancies. Small numbers of Lynch syndrome-associated soft tissue sarcomas have been reported, but there are no reported cases of soft tissue sarcomas in Muir-Torre syndrome. In this study, we report a 74-year-old man with known Muir-Torre syndrome with confirmed MSH2 germline mutation, diagnosed with pleomorphic liposarcoma of the right buttock in a previous radiation field. The tumour showed loss of expression of MSH2 and MSH6 on immunohistochemistry. Immunohistochemistry on another pleomorphic liposarcoma in a different patient with no previous history of Muir-Torre syndrome or Lynch syndrome showed no loss of expression of mismatch repair proteins. This is the first report of Muir-Torre syndrome-associated sarcoma and the first case of post-radiation sarcoma in Lynch syndrome.

  2. Gitelman syndrome associated with chondrocalcinosis: description of two cases

    Directory of Open Access Journals (Sweden)

    E. Filippucci

    2011-06-01

    Full Text Available Gitelman syndrome is a rare inherited tubulopathy, characterized by hypomagnesemia, hypokalemia, metabolic alkalosis, hypocalciuria and hyperreninemic hyperaldosteronism. The clinical spectrum is wide and includes: cramps, myalgies, muscle weakness, until episodes of carpo-podalic spasm, tetania, rabdomyolisis and paralysis. Some cases have been described in literature underlining the association of this condition with chondrocalcinosis, as a typical example of hypomagnesemia-induced crystal deposition disease. The therapy of Gitelman syndrome consists on the administration of defective electrolytes, althought not always effective. We describe two cases of Gitelman syndrome associated with chondrocalcinosis showing the wide range of presentation of this clinical condition.

  3. Posterior reversible encephalopathy syndrome associated with nephrotic syndrome.

    Science.gov (United States)

    Zhang, Hong-Liang; Yang, Yi; Wu, Jiang

    2010-01-01

    Kabicek and colleagues described a case of nephritic-syndrome-associated posterior reversible encephalopathy syndrome (PRES) (Kabicek, et al. 2010). This adds to the accumulating evidence that PRES can be associated with disorders other than hypertension. However, we wonder how the authors would explain the neuroimaging findings unsuggestive of vasogenic oedema. PRES (also named reversible posterior leukoencephalopathy syndrome, RPLS) represents a clinicoradiological syndrome characterized by vasogenic oedema as revealed by apparent diffusion coefficient (ADC) map of diffusion-weighted imaging (DWI) (Bartynski, 2008). The pathogenesis of PRES has been suggested to be autoregulation failure and endothelial dysfunction (Sharma, et al.). ...

  4. Case report: nephrotic syndrome associated with a total hydatidiform mole.

    Science.gov (United States)

    Komatsuda, A; Nakamoto, Y; Asakura, K; Yasuda, T; Imai, H; Miura, A B

    1992-05-01

    The authors describe a 51-year-old Japanese woman who developed nephrotic syndrome in association with a total hydatidiform mole. The nephrotic syndrome remitted completely following hysterectomy. A renal biopsy performed before the operation showed diffuse mesangial cell proliferation of a moderate degree, and thickened capillary walls with focal and segmental subendothelial deposits, as well as circumferential mesangial interposition. Occasional foci of the mesangiolysis were also observed. Immunofluorescence microscopy revealed granular staining of IgM along the glomerular capillary walls in a fringe pattern. A review of the literature revealed that this patient appears to be the first case of nephrotic syndrome associated with a total mole, although there have been two cases of nephrotic syndrome due to preeclamptic nephropathy associated with a partial or transitional mole.

  5. Capgras Syndrome associated with the use of psychoactive substances.

    Science.gov (United States)

    Mercurio, Ezequiel N

    2011-04-01

    Capgras syndrome has originally been described as psychiatric syndrome. However, in the last few years reports of patients with this syndrome has significantly increased in patients with neurological, metabolic, and infectious diseases and those who consume alcohol. Different hypotheses have been proposed to explain the neurobiology of this very unusual symptom, such as changes in the dopamine circuit and specific dysfunctions in facial processing. In this work we present a new case of Capgras syndrome, associated with an acute cocaine overdose, which was transient and reversible. The neurobiological bases of this syndrome are analysed, along with their relationship with the changes induced by cocaine use. Thus, Capgras syndrome could be the expression of functional changes at frontal-temporal level, and the paralimbic region secondary to the consumption of psychoactive substances such as cocaine. Copyright © 2010 SEP y SEPB. Published by Elsevier Espana. All rights reserved.

  6. A case of Werner's syndrome associated with osteosarcoma.

    Science.gov (United States)

    Murata, K; Hatamochi, A; Shinkai, H; Ishikawa, Y; Kawaguchi, N; Goto, M

    1999-10-01

    We described a case of Werner's syndrome associated with osteosarcoma. A 37-year-old Japanese man was diagnosed as having Werner's syndrome by the presence of juvenile cataracts, skin sclerosis and hyperpigmentation of the feet, high-pitched voice, characteristic bird-like appearance of the face with beak-shaped nose, thinning of the entire skin and hyperkeratoses on soles, hyperlipemia, hyperuricemia, diabetes melitus, and the mutated responsible gene (WRN). He had a 3-month history of a tumor on his left forearm. Histologically, the tumor included four histological patterns; a malignant fibrous histiocytoma-like, a desmoid-like, a dermatofibrosarcoma protuberans-like, and a chondrosarcoma-like pattern. Tumoral osteoid formation was also found in the tumor. Therefore, the tumor was diagnosed as osteosarcoma.

  7. Mirizzi syndrome associated with hepatic artery pseudoaneurysm: a case report

    Directory of Open Access Journals (Sweden)

    Anderson Oliver

    2008-11-01

    Full Text Available Abstract Introduction This is the first case report of Mirizzi syndrome associated with hepatic artery pseudoaneurysm. Case presentation A 54-year-old man presented with painful obstructive jaundice and weight loss. Computed tomography showed a hilar mass in the liver. Following an episode of haemobilia, angiography demonstrated a pseudoaneurysm of a branch of the right hepatic artery that was embolised. At surgery, a gallstone causing Mirizzi type II syndrome was found to be responsible for the biliary obstruction and a necrotic inflammatory mass and haematoma were found to be extending into the liver. The mass was debrided and drained, the obstructing stones removed and the bile duct drained with a t-tube. The patient made a full recovery. Conclusion This case highlights another situation where there may be difficulty in differentiating Mirizzi syndrome from biliary tract cancer.

  8. Orbital apex syndrome associated with herpes zoster ophthalmicus

    Directory of Open Access Journals (Sweden)

    Kurimoto T

    2011-11-01

    Full Text Available Takuji Kurimoto1, Masahiro Tonari1, Norihiko Ishizaki1, Mitsuhiro Monta2, Saori Hirata2, Hidehiro Oku1, Jun Sugasawa1, Tsunehiko Ikeda11Department of Ophthalmology, Osaka Medical College, 2Department of Ophthalmology, Shitennoji Hospital, Osaka, JapanAbstract: We report our findings for a patient with orbital apex syndrome associated with herpes zoster ophthalmicus. Our patient was initially admitted to a neighborhood hospital because of nausea and loss of appetite of 10 days' duration. The day after hospitalization, she developed skin vesicles along the first division of the trigeminal nerve, with severe lid swelling and conjunctival injection. On suspicion of meningoencephalitis caused by varicella zoster virus, antiviral therapy with vidarabine and betamethasone was started. Seventeen days later, complete ptosis and ophthalmoplegia developed in the right eye. The light reflex in the right eye was absent and anisocoria was present, with the right pupil larger than the left. Fat-suppressed enhanced T1-weighted magnetic resonance images showed high intensity areas in the muscle cone, cavernous sinus, and orbital optic nerve sheath. Our patient was diagnosed with orbital apex syndrome, and because of skin vesicles in the first division of the trigeminal nerve, the orbital apex syndrome was considered to be caused by herpes zoster ophthalmicus. After the patient was transferred to our hospital, prednisolone 60 mg and vidarabine antiviral therapy was started, and fever and headaches disappeared five days later. The ophthalmoplegia and optic neuritis, but not the anisocoria, gradually resolved during tapering of oral therapy. From the clinical findings and course, the cause of the orbital apex syndrome was most likely invasion of the orbital apex and cavernous sinus by the herpes virus through the trigeminal nerve ganglia.Keywords: varicella zoster virus, orbital apex syndrome, herpes zoster ophthalmicus, complete ophthalmoplegia

  9. A Case of Swyer Syndrome Associated with Advanced Gonadal Dysgerminoma Involving Long Survival

    Directory of Open Access Journals (Sweden)

    Salete Da Silva Rios

    2015-03-01

    Full Text Available Swyer syndrome is caused by abnormal sex differentiation during the embryonic period, resulting in incomplete intrauterine masculinization and undifferentiated gonads. The current case report describes a patient with Swyer syndrome associated with stage 3 gonadal dysgerminoma who has survived for 23 years. At age 18, this patient sought assistance for primary amenorrhea from the Gynecological Services Department of the University of Brasília Hospital. A physical examination revealed that the patient was at Tanner stage 4 with respect to axillary hair, breasts, and pubic hair; she presented with a eutrophic vagina and a small cervix. She was treated with a combination of estrogens and progestogens to induce cycling. Approximately 4 years later, a complex tumor was found and resected; a histopathological analysis revealed that this tumor was a right adnexal dysgerminoma with peritoneal affection. The patient was also subjected to chemotherapy. Her follow-up has continued to the present time, with no signs of tumor recurrence. In conclusion, this report describes an extremely rare case in which Swyer syndrome was associated with ovarian dysgerminoma; relative to similar patients, the described patient has survived for an unusually prolonged time.

  10. Porites white patch syndrome: associated viruses and disease physiology

    Science.gov (United States)

    Lawrence, S. A.; Davy, J. E.; Wilson, W. H.; Hoegh-Guldberg, O.; Davy, S. K.

    2015-03-01

    In recent decades, coral reefs worldwide have undergone significant changes in response to various environmental and anthropogenic impacts. Among the numerous causes of reef degradation, coral disease is one factor that is to a large extent still poorly understood. Here, we characterize the physiology of white patch syndrome (WPS), a disease affecting poritid corals on the Great Barrier Reef. WPS manifests as small, generally discrete patches of tissue discolouration. Physiological analysis revealed that chlorophyll a content was significantly lower in lesions than in healthy tissues, while host protein content remained constant, suggesting that host tissue is not affected by WPS. This was confirmed by transmission electron microscope (TEM) examination, which showed intact host tissue within lesions. TEM also revealed that Symbiodinium cells are lost from the host gastrodermis with no apparent harm caused to the surrounding host tissue. Also present in the electron micrographs were numerous virus-like particles (VLPs), in both coral and Symbiodinium cells. Small (cells from diseased colonies. There was no apparent increase in prokaryotic or eukaryotic microbial abundance in diseased colonies. Taken together, these results suggest that viruses infecting the coral and/or its resident Symbiodinium cells may be the causative agents of WPS.

  11. It's not All Doom and Gloom: Prune Belly Syndrome Associated with VACTERL.

    Science.gov (United States)

    Awad, Karim; Lall, Anupam

    2016-01-01

    Prune belly syndrome is a rare abnormality; its association with VACTERL is even rarer. This association has been reported in literature a few times since first reported in 1993 and so far the majority have either been stillbirths or died shortly after birth. We present a case of Prune belly syndrome associated with VACTERL who is now one year old.

  12. Adult Reye-like syndrome associated with serologic evidence of acute parvovirus B19 infection

    Directory of Open Access Journals (Sweden)

    Paulo Sérgio Gonçalves da Costa

    Full Text Available Reye's syndrome is an infrequently diagnosed medical condition affecting mainly children. The etiology, epidemiology and natural history of Reye's syndrome have been cloudily written in footnotes of medical books and exotic papers since the initial description in early 1950s. We report here a case of adult Reye's syndrome associated with serologic evidence of parvovirus B19 infection.

  13. CNS syndromes associated with antibodies against metabotropic receptors.

    Science.gov (United States)

    Lancaster, Eric

    2017-06-01

    Autoantibodies to Central nervous system (CNS) metabotropic receptors are associated with a growing family of autoimmune brain diseases, including encephalitis, basal ganglia encephalitis, Ophelia syndrome, and cerebellitis. The purpose of this review is to summarize the state of knowledge regarding the target receptors, the neurological autoimmune disorders, and the pathogenic mechanisms. Antibodies to the γ-aminobutyric acid B receptor are associate with limbic encephalitis and severe seizures, often with small cell lung cancers. Metabotropic glutamate receptor 5 (mGluR5) antibodies associate with Ophelia syndrome, a relatively mild form of encephalitis linked to Hodgkin lymphoma. mGluR1 antibodies associate with a form of cerebellar degeneration, and also Hodgkin lymphoma. Antibodies to Homer 3, a protein associated with mGluR1, have also been reported in two patients with cerebellar syndromes. Dopamine-2 receptor antibodies have been reported by one group in children with basal ganglia encephalitis and other disorders. CNS metabotropic receptor antibodies may exert direct inhibitory effects on their target receptors, but the evidence is more limited than with autoantibodies to ionotropic glutamate receptors. In the future, improved recognition of these patients may lead to better outcomes. Understanding the molecular mechanisms of the diseases may uncover novel treatment strategies.

  14. A case of Pallister-Killian syndrome associated with West syndrome.

    Science.gov (United States)

    Yamamoto, Hitoshi; Fukuda, Miho; Murakami, Hiroshi; Kamiyama, Noriko; Miyamoto, Yusaku

    2007-09-01

    We report the case of a 19-month-old boy with Pallister-Killian syndrome associated with West syndrome. The child was born at term to a healthy mother after an uneventful pregnancy. He was born by cesarean section because of fetal macrosomia. He was observed to have nystagmus, craniofacial dysmorphism, and mental retardation. Intractable epileptic spasms developed 17 months after birth, and electroencephalography revealed a modified hypsarrhythmia. The seizures were uncontrollable with sodium valproate monotherapy. At the age of 19 months, the child was diagnosed with Pallister-Killian syndrome of mosaic tetrasomy 12p by fluorescence in situ hybridization. Combination treatment with high-dose pyridoxal phosphate and sodium valproate eliminated seizures and improved the electroencephalographic abnormalities. To our knowledge, this is the first reported case of Pallister-Killian syndrome associated with West syndrome.

  15. Reconstitution Inflammatory Syndrome Like Reactive Hemophagocytic Syndrome Associated with Disseminated Histoplasmosis in a HIV Patient.

    Science.gov (United States)

    Gómez-Espejo, Sandra M; Olalla-Sierra, Julián; Marí-Jiménez, Pilar; Pereda-Salguero, Teresa; Pérez-Stachowski, Javier; de-la-Torre-Lima, Javier; Del-Arco-Jiménez, Alfonso; Prada-Pardal, José L

    2017-08-01

    We describe an unusual clinical association of disseminated histoplasmosis with reactive hemophagocytic syndrome. We report the case of a new HIV-positive patient with reconstitution inflammatory syndrome like reactive hemophagocytic syndrome associated with disseminated histoplasmosis. We describe the clinical case, the procedures performed, the treatment provided and the patient's evolution. A figure of liver biopsy Grocott's silver methenamine stain that shows lots of uniform ovoid yeasts in portal spaces' macrophages that supports the diagnosis of disseminated histoplasmosis in our case.

  16. Initial therapy for acquired immunodeficiency syndrome-associated cryptococcosis with fluconazole.

    Science.gov (United States)

    Nightingale, S D

    1995-03-13

    Published opinion has generally favored amphotericin B over fluconazole as initial therapy for acquired immunodeficiency syndrome-associated cryptococcosis, although data that support this recommendation are limited. Retrospective review of 30 consecutive patients with acquired immunodeficiency syndrome-associated cryptococcosis seen at a single institution over a 1-year period and given fluconazole, 400 mg/d, as initial therapy. No patient died within the first 30 days of therapy, and none of the 14 patients who died within 1 year had clinically detectable infection when last seen or at death. Pretreatment blood cultures were positive in 26 of 27 patients; cerebrospinal fluid cryptococcal antigen titer was greater than 1:1024 in 12 of 23 patients; and five of 30 patients presented with altered mental status. The median CD4 count at diagnosis was 0.042 x 10(9)/L (42/microL). Eight of 25 patients who were followed up for more than 30 days relapsed, as evidenced by a positive culture; all relapses were successfully treated with fluconazole, either by reinstitution of therapy or by increase of dosage. This experience supports the use of fluconazole as initial therapy for acquired immunodeficiency syndrome-associated cryptococcosis.

  17. Bardet-biedel syndrome: a case report and a review of literature ...

    African Journals Online (AJOL)

    Background: Bardet- Biedl syndrome (BBS) is a group of autosomal recessive conditions with overlapping phenotype of rod-cone dystrophy, central obesity, mental retardation, and hypogonadism. There are very few reports of BBS from Sub-Saharan Africa. Objective: To report a case of BBS and review existing literature on ...

  18. Non-essential role for cilia in coordinating precise alignment of lens fibres

    Czech Academy of Sciences Publication Activity Database

    Sugiyama, Y.; Shelley, E.J.; Yoder, B.K.; Kozmik, Zbyněk; May-Simera, H.L.; Beales, P.L.; Lovicu, F.J.; McAvoy, J.W.

    2016-01-01

    Roč. 139, FEB 2016 (2016), s. 10-17 ISSN 0925-4773 R&D Projects: GA ČR GA15-23675S Institutional support: RVO:68378050 Keywords : Lens * Primary cilium * ift88 * Bardet-Biedl Syndrome (BBS) * Planar cell polarity (PCP) Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 1.333, year: 2016

  19. EURO-WABB

    DEFF Research Database (Denmark)

    Farmer, Amy; Aymé, Ségolène; de Heredia, Miguel Lopez

    2013-01-01

    Wolfram, Alström and Bardet-Biedl (WABB) syndromes are rare diseases with overlapping features of multiple sensory and metabolic impairments, including diabetes mellitus, which have caused diagnostic confusion. There are as yet no specific treatments available, little or no access to well...

  20. Clinical and Molecular Investigations Into Ciliopathies

    Science.gov (United States)

    2018-03-27

    Autosomal Recessive Polycystic Kidney Disease; Congenital Hepatic Fibrosis; Caroli's Disease; Polycystic Kidney Disease; Joubert Syndrome; Cerebro-Oculo-Renal Syndromes; COACH Syndrome; Senior-Loken Syndrome; Dekaban-Arima Syndrome; Cogan Oculomotor Apraxia; Nephronophthisis; Bardet-Biedl Syndrome; Alstrom Syndrome; Oral-Facial-Digital Syndrome

  1. West syndrome associated with administration of a histamine H1 antagonist, oxatomide.

    Science.gov (United States)

    Yamashita, Yushiro; Isagai, Takeo; Seki, Yoshitaka; Ohya, Takashi; Nagamitsu, Shinichiro; Matsuishi, Toyojiro

    2004-01-01

    We report a 4-month-old female infant who developed West syndrome eleven days after administration of a histamine H1 antagonist, oxatomide, for atopic dermatitis. It has been reported that some histamine H1 antagonists induce seizures in epileptic patients. The age, the interval between oxatomide administration, and the onset of West syndrome and its clinical course were similar to two previously reported 3-month-old infants with West syndrome associated with ketotifen administration. We should be cautious in using the histamine H1 antagonists, oxatomide and ketotifen, in young infants because such agents could potentially disturb the anticonvulsive central histaminergic system.

  2. A confirmed case of toxic shock syndrome associated with the use of a menstrual cup

    OpenAIRE

    Mitchell, Michael A; Bisch, Steve; Arntfield, Shannon; Hosseini-Moghaddam, Seyed M

    2015-01-01

    Menstrual cups have been reported to be an acceptable substitute for tampons. These flexible cups have also been reported to provide a sustainable solution to menstrual management, with modest cost savings and no significant health risk. The present article documents the first case of toxic shock syndrome associated with the use of a menstrual cup in a woman 37 years of age, using a menstrual cup for the first time. Toxic shock syndrome and the literature on menstrual cups is reviewed and a p...

  3. A confirmed case of toxic shock syndrome associated with the use of a menstrual cup.

    Science.gov (United States)

    Mitchell, Michael A; Bisch, Steve; Arntfield, Shannon; Hosseini-Moghaddam, Seyed M

    2015-01-01

    Menstrual cups have been reported to be an acceptable substitute for tampons. These flexible cups have also been reported to provide a sustainable solution to menstrual management, with modest cost savings and no significant health risk. The present article documents the first case of toxic shock syndrome associated with the use of a menstrual cup in a woman 37 years of age, using a menstrual cup for the first time. Toxic shock syndrome and the literature on menstrual cups is reviewed and a possible mechanism for the development of toxic shock syndrome in the patient is described.

  4. Transient altitude-induced compartment syndrome associated with fiberglass casts using waterproof cast padding.

    Science.gov (United States)

    Kadzielski, John; Bae, Donald S

    2013-01-01

    Changes in aircraft cabin pressure and its interplay with a fixed diameter fiberglass cylindrical cast and the closed air cells in waterproof cast padding may cause a transient altitude-induced compartment syndrome. In this case series, 2 patients reported transient compartment syndromes that resolved with aircraft decent. As proof of concept, this work displays photographic and video evidence showing the difference in air cell volume from experimental data in a vacuum chamber as well as real-world volume changes at cruise altitude in a commercial airliner. Transient altitude-induced compartment syndromes associated with fiberglass casts using waterproof cast padding are real and surgeons and patients should be advised of this potentially devastating complication.

  5. New approaches to the treatments of short bowel syndrome-associated intestinal failure

    DEFF Research Database (Denmark)

    Jeppesen, Palle B

    2014-01-01

    preceding the US Food and Drug Administration and the European Medicines Agency approval of subcutaneous teduglutide for this orphan condition. RECENT FINDINGS: In a 3-week, phase 2, metabolic balance study, teduglutide increased intestinal wet weight absorption by approximately 700 g/day and reduced fecal...... with the known mechanism of action. SUMMARY: Teduglutide will add incremental benefit to the limited medical treatment armamentarium in SBS patients by maximizing intestinal absorption, decreasing fecal losses, thereby decreasing or even eliminating the need for parenteral support. Future research should target......PURPOSE OF REVIEW: Teduglutide, a recombinant analog of human glucagon-like peptide 2, has recently been approved in the US and Europe (Gattex and Revestive, respectively) as the first targeted treatment of short bowel syndrome-associated intestinal failure (SBS-IF). Glucagon-like peptide 2...

  6. A Case of Swyer-James-Macleod Syndrome Associated with Middle Lobe Hypoplasia and Arteriovenous Malformation

    Directory of Open Access Journals (Sweden)

    Hatice Kaplanoglu

    2012-01-01

    Full Text Available A 58-year-old female patient presented to the hospital with hearing loss. In the chest radiography obtained before her ear surgery, volume decrease in the right hemithorax, elevation of the right diaphragm, and increase of ventilation in the right lung were detected. At the thorax CT-CT angiography, hypoplasia of the main pulmonary artery and its branches and arteriovenous malformation localized in the middle lobe of the right lung were detected. Thus, diagnosis of Swyer-James-Macleod syndrome associated with right lung middle lobe hypoplasia and arteriovenous malformation was made. This kind of association has not been reported earlier, so we are presenting it in the light of the literature knowledge.

  7. Primary biliary cirrhosis--autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome.

    Science.gov (United States)

    Pamfil, Cristina; Candrea, Elisabeta; Berki, Emese; Popov, Horațiu I; Radu, Pompilia I; Rednic, Simona

    2015-03-01

    Autoimmune liver diseases may be associated with extrahepatic autoimmune pathology. We report the case of a 52-year old woman who initially presented to the gastroenterology department for extreme fatigue, pale stools, dark urine and pruritus. Laboratory tests showed significant cholestasis and elevation of aminotransferase levels. Immunological tests revealed positive antinuclear (ANA=1:320) and antimitochondrial antibodies (AMA=1:40) with negative anti-smooth muscle and liver kidney microsomal type 1 antibodies. The biopsy was compatible with overlap syndrome type 1. The patient was commenced on immunosuppressive therapy according to standard of care (azathioprine 50mg, ursodeoxycholic acid and prednisone 0.5mg/kg), with moderate biochemical improvement. She subsequently developed proximal symmetrical weakness and cutaneous involvement and was diagnosed with biopsy-proven dermatomyositis. The immunosuppressive regimen was intensified to 150 mg azathioprine. At the three-month follow-up, her symptoms subsided and aminotransferases and muscle enzymes normalized. Upon further investigation the patient was diagnosed with autoimmune thyroiditis and antiphospholipid syndrome. To our knowledge, this is the first case of primary biliary cirrhosis - autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome.

  8. Parinaud's oculoglandular syndrome associated with paracoccidioidomycosis Síndrome oculoglandular de Parinaud associada à paracoccidioidomicose

    Directory of Open Access Journals (Sweden)

    Paulo Sérgio Gonçalves da COSTA

    2002-02-01

    Full Text Available The authors report one case of Parinaud's oculoglandular syndrome associated with Paracoccidioides brasiliensis infection. No other medical report of this condition was found in the medical literature available at Index Medicus and Medline. The eye involvement has been rather uncommon in paracoccidioidomycosis and this report emphasizes the possibility of this kind of presentation making it also necessary to include paracoccidioidomycosis among the several known causes of Parinaud's oculoglandular syndrome.Os autores relatam um caso de síndrome oculoglandular de Parinaud associada à infecção por Paracoccidioides brasiliensis. Nenhum outro relato desta condição foi encontrado na literatura médica disponível pelo Index Medicus e Medline. O envolvimento ocular é incomum na paracoccidioidomicose e este relato enfatiza a possibilidade deste tipo de apresentação, tornando-se necessário incluir a paracoccidioidomicose entre as várias causas conhecidas de síndrome oculoglandular de Parinaud.

  9. Bilateral simultaneous traumatic upper arm compartment syndromes associated with anabolic steroids.

    Science.gov (United States)

    Erturan, Gurhan; Davies, Nev; Williams, Huw; Deo, Sunny

    2013-01-01

    Acute compartment syndrome, a surgical emergency, is defined as increased pressure in an osseofascial space. The resulting reduction of capillary perfusion to that compartment requires prompt fasciotomy. Treatment delay has a poor prognosis, and is associated with muscle and nerve ischemia, resultant infarction, and late-onset contractures. We report a case of traumatic bilateral upper limb acute compartment syndrome associated with anabolic steroids, requiring bilateral emergency fasciotomies. A 25-year-old male bodybuilder taking anabolic steroids, with no past medical history, presented to the Emergency Department 25 min after a road traffic accident. Secondary survey confirmed injuries to both upper limbs with no distal neurovascular deficit. Plain radiographs demonstrated bilateral metaphyseal fractures of the distal humeri. Within 2 h of the accident, the patient developed clinical features that were consistent with bilateral upper arm compartment syndrome. Bilateral fasciotomies of both anterior and posterior compartments were performed, confirming clinical suspicion. We suggest consideration of a history of anabolic steroid use when evaluating patients with extremity trauma. Copyright © 2013 Elsevier Inc. All rights reserved.

  10. Persistent Hiccups—An Unusual Presentation of Bilateral Pheochromocytoma without Syndromic Association: A Case Report

    Directory of Open Access Journals (Sweden)

    Nitin Aherrao

    2012-01-01

    Full Text Available Pheochromocytoma is a rare catecholamine-producing tumor arising from chromaffin tissue in the adrenal medulla, occurring in less than 0.2 percent of patients with hypertension. The mean age at diagnosis is about 40 years. Pheochromocytomas are commonly inherited as features of multiple endocrine neoplasia type 2 or several other pheochromocytoma-associated syndromes and have variable clinical presentation. Among the presenting symptoms, episodes of palpitations, headaches, and profuse sweating are typical and constitute a classic triad. We report a case of a 17-year-old male patient with rare bilateral pheochromocytoma presenting with persistent hiccups for 4 months and blurring of vision for 1 week, later followed by hypertensive crisis. There was neither family history of pheochromocytoma nor any classic symptoms. Patient was diagnosed with bilateral pheochromocytoma without any syndromic association. But still this patient needs to be followed for future development of medullary carcinoma of thyroid because it could be an initial presentation of MEN 2A/2B/VHL syndromes. Our paper highlights the importance of maintaining a high level of suspicion for persistent hiccups and careful clinical screening for hypertension even in absence of associated syndromes of pheochromocytoma and classical symptoms to achieve prompt diagnosis and to avoid improper management.

  11. Lynch Syndrome Associated Colon Adenocarcinoma Resembling Lymphoma on Fluoro-Deoxyglucose-Positron Emission Tomography/Computed Tomography

    International Nuclear Information System (INIS)

    Aparici, Carina Mari; Win, Aung Zaw

    2015-01-01

    The patient was a 46-year-old Asian male diagnosed with lynch syndrome associated colon adenocarcinoma in the right ascending colon. A presurgical staging 18-fluoro-deoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) found increased metabolic activity in the cervical, axillary, mediastinal, supraclavicular, para-aortic and mesenteric lymph nodes. This pattern of metastasis was very unusual for lynch syndrome associated colon adenocarcinoma and the involvement of those lymph nodes resembles the pattern of spread of lymphoma. He underwent right hemicolectomy and he was subsequently treated with 12 cycles of folinic acid (leucovorin), fluorouracil (5-FU), irinotecan. A restaging FDG-PET/CT at the end of the chemotherapy showed interval decrease in size and metabolic activity in the affected lymph nodes. FDG-PET/CT is a useful imaging modality in following-up the treatment response in colon adenocarcinoma

  12. The Clinical and Neurophysiological Features of Epileptic Syndromes Associated with Benign Focal Epileptiform Discharges of Childhood

    Directory of Open Access Journals (Sweden)

    N. A. Ermolaenko

    2014-01-01

    Full Text Available Benign focal epileptiform discharge of childhood (BFEDC is an age-dependent pattern determined in electroencephalograms (EEGs, which is associated with idiopathic benign focal epilepsy (BFE. Studies of BFE revealed symptomatic phenocopies in patients with cerebral struc- tural abnormalities in such conditions as infantile cerebral palsy and malformations. Some arguments against the «benign» nature of BFEDC are presented, since BFEDC may impair various cognitive functions and behavior (e.g., cause epileptic encephalophathies. Objective. To determine the clinical and neurophysiological features of epileptic syndromes associated with prolonged epileptiform activity during sleep in children and adolescents, as well as approaches to rational therapy. Patients and Methods. A total of 1862 children aged 2–18 admitted to the specialized Department of Psychoneurology of the Voronezh Regional Children Clinical Hospital No 1 in 2004–2007, who had epileptic seizures and non-epileptic neurological disorders, were exam- ined. The children underwent assessment of the neurological status, neuropsychological assessment, and video-EEG monitoring. The spike- wave index (SWI was calculated and magnetic resonance imaging (MRI of the brain was performed to register the epileptiform activity during sleep. Results and Discussion. It was demonstrated that when ISW of BFEDC patterns is >30%, evolution into epileptic encephalopathy was observed in 66% of patients (including epilepsy with electrical status epilepticus in sleep in 49% of patients and cognitive epileptiform disinte- gration in 17% of patients. The results prove the justification of prescribing antiepileptic drugs to patients with SWI ≥30% even if they have no epileptic seizures. Duo-therapy with valproate and ethosuximide or levetiracetam is most the effective therapy. Further prospective studies for children with BFEDC will give new insight into this area. 

  13. Inherited Retinal Degenerative Disease Registry

    Science.gov (United States)

    2017-09-13

    Eye Diseases Hereditary; Retinal Disease; Achromatopsia; Bardet-Biedl Syndrome; Bassen-Kornzweig Syndrome; Batten Disease; Best Disease; Choroidal Dystrophy; Choroideremia; Cone Dystrophy; Cone-Rod Dystrophy; Congenital Stationary Night Blindness; Enhanced S-Cone Syndrome; Fundus Albipunctatus; Goldmann-Favre Syndrome; Gyrate Atrophy; Juvenile Macular Degeneration; Kearns-Sayre Syndrome; Leber Congenital Amaurosis; Refsum Syndrome; Retinitis Pigmentosa; Retinitis Punctata Albescens; Retinoschisis; Rod-Cone Dystrophy; Rod Dystrophy; Rod Monochromacy; Stargardt Disease; Usher Syndrome

  14. Síndrome de Sweet asociado a neoplasias Sweet's syndrome associated with neoplasms

    Directory of Open Access Journals (Sweden)

    Mariana Franco

    2006-10-01

    : idiopathic, parainflammatory, paraneoplastic, drug-induced, and pregnancy-related. Twenty percent of cases are associated with malignancies; 85% out of them involve hematologic alignancies and the remaining 15%, solid tumors. A series of seven cases of Sweet's syndrome associated with neoplasms which were diagnosed from 2002 to 2006 is presented. Six cases were related to oncohematologic diseases and one to solid tumors. These results highlight the importance of the diagnosis of the syndrome, since it may predict tumor relapse or underlying disease progression. The timely use of diagnostic and treatment methods may improve the quality of life of these patients. The fact that oncology patients take multiple medications (a colony-stimulating factor which may be associated with the onset of this entity must also be considered in excluding possible causes.

  15. Production of acquired immunodeficiency syndrome-associated retrovirus in human and nonhuman cells transfected with an infectious molecular clone

    International Nuclear Information System (INIS)

    Adachi, A.; Gendelman, H.E.; Koenig, S.; Folks, T.; Willey, R.; Rabson, A.; Martin, M.A.

    1986-01-01

    The authors considered an infectious molecular clone of acquired immunodeficiency syndrome-associated retrovirus. Upon transfection, this clone directed the production of infectious virus particles in a wide variety of cells in addition to human T4 cells. The progeny, infectious virions, were synthesized in mouse, mink, monkey, and several human non-T cell lines, indicating the absence of any intracellular obstacle to viral RNA or protein production or assembly. During the course of these studies, a human colon carcinoma cell line, exquisitely sensitive to DNA transfection, was identified

  16. Mechanisms underlying acquired von Willebrand syndrome associated with an IgM paraprotein.

    Science.gov (United States)

    Mayerhofer, M; Haushofer, A; Kyrle, P A; Chott, A; Müllner, C; Quehenberger, P; Worel, N; Traby, L; Eichinger, S

    2009-09-01

    Acquired von Willebrand (vW) syndrome is a rare bleeding disorder which is frequently associated with immunological, malignant or cardiovascular disorders. The underlying pathomechanisms, particularly in patients with IgM monoclonal gammopathies, often remain unknown. We report a patient with indolent small B-cell lymphoma (immunocytoma) and plasmacytic differentiation with an IgM kappa paraprotein who was admitted with retroperitoneal haematoma. Medical history and coagulation testing were consistent with acquired vW syndrome. vW immunohistochemistry showed normal cytoplasmic labelling of endothelial cells and megakaryocytes, whereas the lymphomatous infiltrate was negative. Acquired vW syndrome due to adsorption of vW factor on malignant cells was thus excluded. In the multimeric analysis, all multimers were present similar to that in type 1 vW syndrome, but the triplet structures were blurred. The bands on serum immunofixation electrophoresis were also atypically broadened, which suggested complex formation between the IgM and vW factor. Immunoprecipitation studies showed that the 176-kDa proteolytic fragment of vW factor co-precipitated with the IgM paraprotein in the patient but not in the controls, suggesting a specific interaction between vW factor and the paraprotein in the patient. The patient required surgery and was successfully managed by chemotherapy consisting of rituximab and fludarabin as well as plasma exchange.

  17. Mild aerobic exercise blocks elastin fiber fragmentation and aortic dilatation in a mouse model of Marfan syndrome associated aortic aneurysm.

    Science.gov (United States)

    Gibson, Christine; Nielsen, Cory; Alex, Ramona; Cooper, Kimbal; Farney, Michael; Gaufin, Douglas; Cui, Jason Z; van Breemen, Cornelis; Broderick, Tom L; Vallejo-Elias, Johana; Esfandiarei, Mitra

    2017-07-01

    Regular low-impact physical activity is generally allowed in patients with Marfan syndrome, a connective tissue disorder caused by heterozygous mutations in the fibrillin-1 gene. However, being above average in height encourages young adults with this syndrome to engage in high-intensity contact sports, which unfortunately increases the risk for aortic aneurysm and rupture, the leading cause of death in Marfan syndrome. In this study, we investigated the effects of voluntary (cage-wheel) or forced (treadmill) aerobic exercise at different intensities on aortic function and structure in a mouse model of Marfan syndrome. Four-week-old Marfan and wild-type mice were subjected to voluntary and forced exercise regimens or sedentary lifestyle for 5 mo. Thoracic aortic tissue was isolated and subjected to structural and functional studies. Our data showed that exercise improved aortic wall structure and function in Marfan mice and that the beneficial effect was biphasic, with an optimum at low intensity exercise (55-65% V̇o 2max ) and tapering off at a higher intensity of exercise (85% V̇o 2max ). The mechanism underlying the reduced elastin fragmentation in Marfan mice involved reduction of the expression of matrix metalloproteinases 2 and 9 within the aortic wall. These findings present the first evidence of potential beneficial effects of mild exercise on the structural integrity of the aortic wall in Marfan syndrome associated aneurysm. Our finding that moderate, but not strenuous, exercise protects aortic structure and function in a mouse model of Marfan syndrome could have important implications for the medical care of young Marfan patients. NEW & NOTEWORTHY The present study provides conclusive scientific evidence that daily exercise can improve aortic health in a mouse model of Marfan syndrome associated aortic aneurysm, and it establishes the threshold for the exercise intensity beyond which exercise may not be as protective. These findings establish a platform

  18. Calf Compartment Syndrome associated with the Use of an Intra-osseous Line in an Adult Patient: A Case Report

    Directory of Open Access Journals (Sweden)

    Malhotra R

    2016-11-01

    Full Text Available We present a case of a lower limb compartment syndrome associated with the use of an intra-osseous line inserted into the proximal tibia in an adult patient. An unconscious 59-year old male with multiple injuries presented to our Emergency Department after a road traffic accident. Bilateral proximal tibial intra osseous-lines were inserted due to poor venous access. After resuscitation his left leg was noted to be tense and swollen with absent pulses. Acute compartment syndrome was diagnosed both clinically and with compartment pressure measurement. Two incision fasciotomy on his left lower leg was performed. Intra osseous-lines in the proximal tibia are increasingly used in adult patients in the pre-hospital setting by paramedics and emergency physicians. Their use, along with the possible complications of these devices, such as the development of compartment syndrome or osteomyelitis leading to amputation, is well reported in the paediatric literature. To the best of our knowledge, there have not been any previous reports of complications in the adult patient. We present a case of lower leg compartment syndrome developing from the use of an intra-osseous line in the proximal tibia in an adult patient. With the increasing use of intra-osseous lines in adult patients, clinicians should be aware of the possibility of developing compartment syndrome which may lead to disability or amputation in severe cases.

  19. Genetic Syndromes Associated with Congenital Cardiac Defects and Ophthalmologic Changes - Systematization for Diagnosis in the Clinical Practice

    Directory of Open Access Journals (Sweden)

    Priscila H. A. Oliveira

    Full Text Available Abstract Background: Numerous genetic syndromes associated with heart disease and ocular manifestations have been described. However, a compilation and a summarization of these syndromes for better consultation and comparison have not been performed yet. Objective: The objective of this work is to systematize available evidence in the literature on different syndromes that may cause congenital heart diseases associated with ocular changes, focusing on the types of anatomical and functional changes. Method: A systematic search was performed on Medline electronic databases (PubMed, Embase, Cochrane, Lilacs of articles published until January 2016. Eligibility criteria were case reports or review articles that evaluated the association of ophthalmic and cardiac abnormalities in genetic syndrome patients younger than 18 years. Results: The most frequent genetic syndromes were: Down Syndrome, Velo-cardio-facial / DiGeorge Syndrome, Charge Syndrome and Noonan Syndrome. The most associated cardiac malformations with ocular findings were interatrial communication (77.4%, interventricular communication (51.6%, patent ductus arteriosus (35.4%, pulmonary artery stenosis (25.8% and tetralogy of Fallot (22.5%. Conclusion: Due to their clinical variability, congenital cardiac malformations may progress asymptomatically to heart defects associated with high morbidity and mortality. For this reason, the identification of extra-cardiac characteristics that may somehow contribute to the diagnosis of the disease or reveal its severity is of great relevance.

  20. Hemihypertrophy and primary small intestinal lymphangiectasia in incontinentia pigmenti achromians.

    Science.gov (United States)

    Riyaz, A; Riyaz, Najeeba; Anoop, P; Chandni, B; Noushad, K

    2004-10-01

    A rare occurrence of primary small intestinal lymphangiectasia in an 8-year-old girl with incontinentia pigmenti achromians is reported. In addition, she had right sided hemihypertrophy. Though intestinal lymphangiectasia is known to have a few syndromic associations, its co-existence in a child with incontinentia pigmenti achromians has not yet been reported in the literature. Hemihypertrophy is also extremely rare in the latter and only very few instances of an association between the two have been documented previously.

  1. Dynamic innate immune responses of human bronchial epithelial cells to severe acute respiratory syndrome-associated coronavirus infection.

    Directory of Open Access Journals (Sweden)

    Tomoki Yoshikawa

    2010-01-01

    Full Text Available Human lung epithelial cells are likely among the first targets to encounter invading severe acute respiratory syndrome-associated coronavirus (SARS-CoV. Not only can these cells support the growth of SARS-CoV infection, but they are also capable of secreting inflammatory cytokines to initiate and, eventually, aggravate host innate inflammatory responses, causing detrimental immune-mediated pathology within the lungs. Thus, a comprehensive evaluation of the complex epithelial signaling to SARS-CoV is crucial for paving the way to better understand SARS pathogenesis. Based on microarray-based functional genomics, we report here the global gene response of 2B4 cells, a cloned bronchial epithelial cell line derived from Calu-3 cells. Specifically, we found a temporal and spatial activation of nuclear factor (NFkappaB, activator protein (AP-1, and interferon regulatory factor (IRF-3/7 in infected 2B4 cells at 12-, 24-, and 48-hrs post infection (p.i., resulting in the activation of many antiviral genes, including interferon (IFN-beta, -lambdas, inflammatory mediators, and many IFN-stimulated genes (ISGs. We also showed, for the first time, that IFN-beta and IFN-lambdas were capable of exerting previously unrecognized, non-redundant, and complementary abilities to limit SARS-CoV replication, even though their expression could not be detected in infected 2B4 bronchial epithelial cells until 48 hrs p.i. Collectively, our results highlight the mechanics of the sequential events of antiviral signaling pathway/s triggered by SARS-CoV in bronchial epithelial cells and identify novel cellular targets for future studies, aiming at advancing strategies against SARS.

  2. Thrombospondin-derived peptide attenuates Sjögren's syndrome-associated ocular surface inflammation in mice.

    Science.gov (United States)

    Contreras Ruiz, L; Mir, F A; Turpie, B; Masli, S

    2017-04-01

    Sjögren's syndrome is the second most common rheumatic disease in which autoimmune response targets exocrine glands (salivary and lacrimal glands) result in clinical symptoms of dry mouth and dry eye. Inflammation of the lacrimal gland induces tear abnormalities that contribute to the inflammation of the ocular surface, which includes ocular mucosa. Thrombospondin-1 (TSP-1) plays a critical regulatory role in the ocular mucosa and as such TSP-1 -/- mice develop spontaneously chronic ocular surface inflammation associated with Sjögren's syndrome. The autoimmune pathology is also accompanied by a peripheral imbalance in regulatory (T reg ) and inflammatory Th17 effectors. In this study, we demonstrate an in-vitro effect of a CD47-binding TSP-derived peptide in the induction of transforming growth factor (TGF)-β1-secreting forkhead box protein 2 (Foxp3 + ) T regs from activated CD4 + CD25 - T cells and the inhibition of pathogenic T helper type 17 (Th17)-promoting interleukin (IL)-23 derived from antigen-presenting cells. The in-vivo administration of this peptide promotes Foxp3 + T reg induction and inhibition of Th17 development. Consistent with these results, topical administration of CD47-binding TSP peptide, both before and after the onset of the disease, attenuates clinical symptoms of SS-associated dry eye in TSP-1 -/- mice. Augmented expression of Foxp3 detected in the draining lymph nodes of TSP peptide -treated mice compared to those treated with control peptide suggests the ability of TSP peptide to restore peripheral immune imbalance. Thus, our results suggest that TSP-derived peptide attenuates Sjögren's syndrome-associated dry eye and autoimmune inflammation by preventing Th17 development while promoting the induction of T regs . Collectively, our data identify TSP-derived peptide as a novel therapeutic option to treat autoimmune diseases. © 2017 British Society for Immunology.

  3. SEPT9_v1 Functions in Breast Cancer Cell Division

    Science.gov (United States)

    2012-01-01

    to the primary cilium. Science 320(5884):1777–1781. Kremer BE, Adang LA , Macara IG. 2007. Septins regulate actin organization and cell-cycle arrest...Chlamydomonas eugametos gametes. Planta 167(4):544–553. Mykytyn K, Sheffield VC. 2004. Establishing a connection between cilia and Bardet-Biedl syndrome...782–793. Williams CL, Li C, Kida K, Inglis PN, Mohan S, Semenec L, Bia- las NJ, Stupay RM, Chen N, Blacque OE., et al. 2011. MKS and NPHP modules

  4. Angelman syndrome-associated ubiquitin ligase UBE3A/E6AP mutants interfere with the proteolytic activity of the proteasome.

    Science.gov (United States)

    Tomaić, V; Banks, L

    2015-01-29

    Angelman syndrome, a severe neurodevelopmental disease, occurs primarily due to genetic defects, which cause lack of expression or mutations in the wild-type E6AP/UBE3A protein. A proportion of the Angelman syndrome patients bear UBE3A point mutations, which do not interfere with the expression of the full-length protein, however, these individuals still develop physiological conditions of the disease. Interestingly, most of these mutations are catalytically defective, thereby indicating the importance of UBE3A enzymatic activity role in the Angelman syndrome pathology. In this study, we show that Angelman syndrome-associated mutants interact strongly with the proteasome via the S5a proteasomal subunit, resulting in an overall inhibitory effect on the proteolytic activity of the proteasome. Our results suggest that mutated catalytically inactive forms of UBE3A may cause defects in overall proteasome function, which could have an important role in the Angelman syndrome pathology.

  5. Small Data

    NARCIS (Netherlands)

    S. Pemberton (Steven)

    2014-01-01

    htmlabstractThe term “Open Data” often goes hand in hand with the term “Big Data”, where large data sets get released allowing for analysis, but the Cinderella of the Open Data ball is Small Data, small amounts of data, nonetheless possibly essential, that are too small to be put in some database or

  6. Small Data

    OpenAIRE

    Pemberton, Steven

    2014-01-01

    htmlabstractThe term “Open Data” often goes hand in hand with the term “Big Data”, where large data sets get released allowing for analysis, but the Cinderella of the Open Data ball is Small Data, small amounts of data, nonetheless possibly essential, that are too small to be put in some database or online dataset to be put to use. RDFa is a technology that allows Cinderella to go to the ball.

  7. Anti-radiation vaccine: Immunologically-based Prophylaxis of Acute Toxic Radiation Syndromes Associated with Long-term Space Flight

    Science.gov (United States)

    Popov, Dmitri; Maliev, Vecheslav; Jones, Jeffrey; Casey, Rachael C.

    2007-01-01

    Protecting crew from ionizing radiation is a key life sciences problem for long-duration space missions. The three major sources/types of radiation are found in space: galactic cosmic rays, trapped Van Allen belt radiation, and solar particle events. All present varying degrees of hazard to crews; however, exposure to high doses of any of these types of radiation ultimately induce both acute and long-term biological effects. High doses of space radiation can lead to the development of toxicity associated with the acute radiation syndrome (ARS) which could have significant mission impact, and even render the crew incapable of performing flight duties. The creation of efficient radiation protection technologies is considered an important target in space radiobiology, immunology, biochemistry and pharmacology. Two major mechanisms of cellular, organelle, and molecular destruction as a result of radiation exposure have been identified: 1) damage induced directly by incident radiation on the macromolecules they encounter and 2) radiolysis of water and generation of secondary free radicals and reactive oxygen species (ROS), which induce chemical bond breakage, molecular substitutions, and damage to biological molecules and membranes. Free-radical scavengers and antioxidants, which neutralize the damaging activities of ROS, are effective in reducing the impact of small to moderate doses of radiation. In the case of high doses of radiation, antioxidants alone may be inadequate as a radioprotective therapy. However, it remains a valuable component of a more holistic strategy of prophylaxis and therapy. High doses of radiation directly damage biological molecules and modify chemical bond, resulting in the main pathological processes that drive the development of acute radiation syndromes (ARS). Which of two types of radiation-induced cellular lethality that ultimately develops, apoptosis or necrosis, depends on the spectrum of incident radiation, dose, dose rate, and

  8. Colonic fermentation of polyphenols from Chilean currants (Ribes spp.) and its effect on antioxidant capacity and metabolic syndrome-associated enzymes.

    Science.gov (United States)

    Burgos-Edwards, Alberto; Jiménez-Aspee, Felipe; Theoduloz, Cristina; Schmeda-Hirschmann, Guillermo

    2018-08-30

    The Chilean wild currants Ribes magellanicum and R. punctatum are a good source of polyphenols. Polyphenolic-enriched extracts (PEEs) from both species were submitted to in vitro colonic fermentation to assess the changes in phenolic composition, antioxidant capacity and inhibition of metabolic syndrome-associated enzymes. The phenolic profiles of the fermented samples showed significant changes after 24 h incubation. Nine metabolites, derived from the microbial fermentation, were tentatively identified, including dihydrocaffeic acid, dihydrocaffeoyl-, dihydroferuloylquinic acid, 1-(3,4-dihydroxyphenyl)-3-(2,4,6-trihydroxyphenyl)propan-2-ol (3,4-diHPP-2-ol), among others. The content of anthocyanins and hydroxycinnamic acids was most affected by simulated colonic conditions, with a loss of 71-92% and 90-100% after 24 h incubation, respectively. The highest antioxidant capacity values (ORAC) were reached after 8 h incubation. The inhibitory activity against the enzyme α-glucosidase was maintained after the fermentation process. Our results show that simulated colonic fermentation exerts significant changes on the polyphenolic composition of these berries, modifying their health-promoting properties. Copyright © 2018 Elsevier Ltd. All rights reserved.

  9. Small hydro

    International Nuclear Information System (INIS)

    Bennett, K.; Tung, T.

    1995-01-01

    A small hydro plant in Canada is defined as any project between 1 MW and 15 MW but the international standard is 10 MW. The global market for small hydro development was considered good. There are some 1000 to 2000 MW of generating capacity being added each year. In Canada, growth potential is considered small, primarily in remote areas, but significant growth is anticipated in Eastern Europe, Africa and Asia. Canada with its expertise in engineering, manufacturing and development is considered to have a good chance to take advantage of these growing markets

  10. Small Families

    Science.gov (United States)

    ... attention and educational advantages, which generally raise her self-esteem. Children in small families, especially first and only ... be for you both to accept the increasing definition of personality that needs to occur as she ...

  11. Retroperitoneal liposarcoma associated with small plaque parapsoriasis.

    Science.gov (United States)

    Tartaglia, Francesco; Blasi, Sara; Sgueglia, Monica; Polichetti, Paolo; Tromba, Luciana; Berni, Alberto

    2007-07-09

    Extremely rare cases of paraneoplastic syndromes or ectopic production of proteins associated with liposarcoma are reported in literature. Production of Granulocyte-Colony Stimulating Factor, alpha-fetoprotein, paraneoplastic pemphigus and leucocytosis, Acrokeratosis paraneoplastica (Bazex's syndrome) are reported. The present report describes a case of retroperitoneal liposarcoma associated with small plaque parapsoriasis. Our search in the English literature of such a kind of association did not reveal any case reported. A 74 year male patient was admitted to our hospital because of the presence of an abdominal mass in right iliac fossa. He also complained of a two-year history of psoriasiform eruptions. The CT scan showed a retroperitoneal pelvic mass. Therefore surgical resection of the tumor was performed. After surgery, the skin eruptions disappeared completely in seven days and so a diagnosis of parapsoriasis syndrome was done. Parallel disappearing of skin eruptions after surgery, typical clinical picture and not specific histology of the cutaneous lesions suggest the diagnosis of small plaque parapsoriasis. Therefore we propose to add Small Plaque Parapsoriasis to the list of paraneoplastic syndromes associated to liposarcoma.

  12. Retroperitoneal liposarcoma associated with small plaque parapsoriasis

    Directory of Open Access Journals (Sweden)

    Polichetti Paolo

    2007-07-01

    Full Text Available Abstract Background Extremely rare cases of paraneoplastic syndromes or ectopic production of proteins associated with liposarcoma are reported in literature. Production of Granulocyte-Colony Stimulating Factor, alpha-fetoprotein, paraneoplastic pemphigus and leucocytosis, Acrokeratosis paraneoplastica (Bazex's syndrome are reported. The present report describes a case of retroperitoneal liposarcoma associated with small plaque parapsoriasis. Our search in the English literature of such a kind of association did not reveal any case reported. Case presentation A 74 year male patient was admitted to our hospital because of the presence of an abdominal mass in right iliac fossa. He also complained of a two-year history of psoriasiform eruptions. The CT scan showed a retroperitoneal pelvic mass. Therefore surgical resection of the tumor was performed. After surgery, the skin eruptions disappeared completely in seven days and so a diagnosis of parapsoriasis syndrome was done. Conclusion Parallel disappearing of skin eruptions after surgery, typical clinical picture and not specific histology of the cutaneous lesions suggest the diagnosis of small plaque parapsoriasis. Therefore we propose to add Small Plaque Parapsoriasis to the list of paraneoplastic syndromes associated to liposarcoma.

  13. Prader-Willi Syndrome Association

    Science.gov (United States)

    ... Find a Chapter Contact Us International Membership Blog Shopping Bag Shop PWSA (USA) Archive Events Find a ... With our many On The Move Walks and online fundraisers such as eWalk, we have something available ...

  14. Nephrotic syndrome associated with meningioma

    Directory of Open Access Journals (Sweden)

    P P Zachariah

    2013-01-01

    Full Text Available A 58-year-old man presented with recurrent frontal meningioma and nephrotic syndrome. Renal biopsy could not be done in view of the rapid neurological deterioration. The patient underwent surgical resection of the tumor. Within 4 weeks, the edema decreased, serum albumin improved, and proteinuria decreased spontaneously. At three months of followup, the patient had attained complete remission of nephrotic state.

  15. Alagille Syndrome Associated with Xerophthalmia

    Directory of Open Access Journals (Sweden)

    Elad Moisseiev

    2013-12-01

    Full Text Available Purpose: To report the occurrence of xerophthalmia and keratomalacia in a patient with Alagille syndrome. Methods: The patient's record and relevant literature were reviewed. Results: A 3-year-old boy with Alagille syndrome was examined at our institution due to severe bilateral ocular irritation. A corneal ulcer and keratomalacia were found in the right eye and severe dryness with corneal opacification was found in the left eye. He was treated with topical fortified antibiotics in the right eye, followed by amniotic membrane transplantation. Due to his systemic condition, characterized by severe cholestasis and intestinal malabsorption, a suspicion of vitamin A deficiency was raised and was later confirmed in serum analysis. Conclusion: This is the first report of xerophthalmia in a patient with Alagille syndrome. Vitamin A deficiency leading to xerophthalmia is common in developing countries; however, its occurrence in the West is rare, leading to a reduced awareness of this disorder amongst clinicians. Unusual eating habits, intestinal malabsorption and liver disease are possible causes for such a deficiency. The purpose of this case report is to raise awareness to the possibility of vitamin A deficiency in children with keratopathy, especially when associated with these disorders.

  16. Alagille Syndrome Associated with Xerophthalmia

    OpenAIRE

    Moisseiev, Elad; Cohen, Shlomi; Dotan, Gad

    2013-01-01

    Purpose: To report the occurrence of xerophthalmia and keratomalacia in a patient with Alagille syndrome. Methods: The patient's record and relevant literature were reviewed. Results: A 3-year-old boy with Alagille syndrome was examined at our institution due to severe bilateral ocular irritation. A corneal ulcer and keratomalacia were found in the right eye and severe dryness with corneal opacification was found in the left eye. He was treated with topical fortified antibiotics in the right ...

  17. The Native Fruit Geoffroea decorticans from Arid Northern Chile: Phenolic Composition, Antioxidant Activities and In Vitro Inhibition of Pro-Inflammatory and Metabolic Syndrome-Associated Enzymes

    Directory of Open Access Journals (Sweden)

    Felipe Jiménez-Aspee

    2017-09-01

    Full Text Available The native tree Geoffroea decorticans (chañar grows in the arid lands of northern Chile. It has been used as a food plant since prehistoric times. Phenolic-enriched extracts (PEEs of Chilean chañar fruits were assessed for their chemical composition, antioxidant properties and inhibition of pro-inflammatory and metabolic syndrome-associated enzymes. Phenolic profiles were determined by HPLC-DAD-MS/MS. The PEEs of G. decorticans showed a strong effect towards the enzymes COX-1/COX-2, with inhibition percentages ranging from inactive to 92.1% and inactive to 76.0% at 50 µg PEE/mL, respectively. The IC50 values of the PEEs towards lipoxygenase and phospholipase A2 inhibitory activity were between 43.6–96.8 and 98.9–156.0 μg PEE/mL, respectively. Samples inhibited α-glucosidase (IC50 0.8–7.3 μg PEE/mL and lipase (9.9 to >100 μg PEE/mL. However, samples did not inhibit α-amylase. The HPLC-DAD-MS analysis of the PEEs allowed the tentative identification of 53 compounds, mainly flavonol glycosides and procyanidins. The procyanidin content of the Chilean G. decorticans pulp was positively correlated with the antioxidant activity and the inhibition of the enzyme α-glucosidase. These results indicate that the Chilean chañar fruit contains bioactive polyphenols with functional properties.

  18. Adam Small

    African Journals Online (AJOL)

    Owner

    oudste dogter (MOOC 29304). Jan Small (vroeër: Jan Dampies) het ek as Oupa Jan geken en Fatimah was vir my Ouma Tiema. Uncle erken ruiterlik sy ma se sterk invloed op sy verbeelding en lewensbenadering, soos hy dit ietwat enigmaties gestel het: 'My skryfwerk lê iewers.' In sy huldeblyk,. “Onaf gedig”, wat hy tydens ...

  19. Small Composers

    DEFF Research Database (Denmark)

    Holgersen, Sven-Erik; Bruun, Peter; Tjagvad, Mette

    2018-01-01

    The present chapter discusses roles and responsibilities of the collaborating partners in a creative music workshop called Small Composers. The aim is to be attentive to a number of potential alterations implicated by the collaborating partners’ different backgrounds. The following questions guided...... the study: What expectations do the class teacher and the professional musicians have to the creative practice, i.e. to the collaboration and to the musical outcome? To which extent do the collaborating partners share a common understanding of the aim, content and method of the workshop? How do the roles...... and responsibilities of the collaborating partners become visible through the practice? How do the professional identities of the teacher and the musicians become visible and what are the implications for the workshop as a musical community of practice?...

  20. Small talk

    Directory of Open Access Journals (Sweden)

    Ryszard Przybylski

    2016-12-01

    Full Text Available The poem Small talk conjures up a communicative situation in which the main character, a newcomer from Poland, answers conventional questions related to their country. Bearing in mind the fact that this poem is set during a military dictatorship, superficial interest in his homeland may trigger a feeling of impatience. This is at least the impression formed if we adopt the perspective defined within the romantic tradition, and when taking into account the conventional poetry of martial law in Poland. Nevertheless, Barańczak retains an ironic distance towards such communicative situations and, as a consequence, does not create poetry that meets most readersʼ expectations. His poetic imperative for verbal art to be the expression of mistrust remains valid.

  1. Small Composers

    DEFF Research Database (Denmark)

    Holgersen, Sven-Erik; Bruun, Peter; Tjagvad, Mette

    2018-01-01

    The present chapter discusses roles and responsibilities of the collaborating partners in a creative music workshop called Small Composers. The aim is to be attentive to a number of potential alterations implicated by the collaborating partners’ different backgrounds. The following questions guid...... and responsibilities of the collaborating partners become visible through the practice? How do the professional identities of the teacher and the musicians become visible and what are the implications for the workshop as a musical community of practice?...... the study: What expectations do the class teacher and the professional musicians have to the creative practice, i.e. to the collaboration and to the musical outcome? To which extent do the collaborating partners share a common understanding of the aim, content and method of the workshop? How do the roles...

  2. Hipodoncia y hueso navicular accesorio: una interesante asociación sindrómica Hypodontia and accessory navicular bone: an interesting syndromic association

    Directory of Open Access Journals (Sweden)

    Mario Cantín

    2012-09-01

    the teeth 1.7, 2.7, 2.8, 3.8 and 4.8; and the presence of an accessory navicular bone in the left foot. It is interesting to suggest that this rare association, with clear dominant autosomal inheritance, might exist; since the dental agenesis and the presence of accessory navicular bone have similar prevalence, which could point to a new syndromic association probably related to the lack of PAX9.

  3. Small Sar Satellite Using Small Standard Bus

    OpenAIRE

    Ono, Kiyonobu; Fujimura, Takashi; Ogawa, Toshiaki; Kimura, Tsunekazu

    2011-01-01

    This paper introduces a new small SAR (Synthetic Aperture Radar) satellite that follows the small optical sensor satellite, ASNARO. USEF, NEDO and NEC are developing ASNARO satellite, which is a small LEO satellite (total mass

  4. Small Business Development Center

    Data.gov (United States)

    Small Business Administration — Small Business Development Centers (SBDCs) provide assistance to small businesses and aspiring entrepreneurs throughout the United States and its territories. SBDCs...

  5. Small Scale Organic Techniques

    Science.gov (United States)

    Horak, V.; Crist, DeLanson R.

    1975-01-01

    Discusses the advantages of using small scale experimentation in the undergraduate organic chemistry laboratory. Describes small scale filtration techniques as an example of a semi-micro method applied to small quantities of material. (MLH)

  6. Lung cancer - small cell

    Science.gov (United States)

    Cancer - lung - small cell; Small cell lung cancer; SCLC ... About 15% of all lung cancer cases are SCLC. Small cell lung cancer is slightly more common in men than women. Almost all cases of SCLC are ...

  7. Systems with small dissipation

    CERN Document Server

    Braginsky, V B; Panov, V I

    1985-01-01

    Introduction ; mechanical oscillators with small dissipation ; electromagnetic resonators with small dissipation ; high-quality electromagnetic resonators in physical experiments ; mechanical oscillators in physical experiments

  8. Small Business Size Standards

    Data.gov (United States)

    Small Business Administration — Certain government programs, such as SBA loan programs and contracting opportunities, are reserved for small business concerns. In order to qualify, businesses must...

  9. Oralfacialdigital-like syndrome with respiratory tract symptoms from birth and ultrastructural centriole/basal body disarray.

    Science.gov (United States)

    Stenram, Unne; Cramnert, Catarina; Axfors-Olsson, Helene

    2007-07-01

    A girl with polydactyly has had respiratory tract problems, including atelectasis, since birth. She has a high arched palate, a tongue hamartoma and dysmorphic face. Electron microscopy of nasal and bronchial brush biopsies repeatedly revealed centriole/basal body disarray and extreme sparseness of cilia. At the age of 2 years and 11 months, she displayed retardation of both motor and mental skills. The manifestations tally with a ciliopathy, partly with the Bardet--Biedl syndrome (BBS) but especially with the oralfacialdigital syndrome (OFDS); however, with the addition of persistent respiratory tract problems. As these two syndromes are considered to be due to mutations affecting the centriole/basal body apparatus, the ultrastructural demonstration of disarray of these structures, never before demonstrated in such a patient, is of fundamental interest.

  10. The blind leading the obese: the molecular pathophysiology of a human obesity syndrome.

    Science.gov (United States)

    Sheffield, Val C

    2010-01-01

    Bardet-Biedl syndrome (BBS) is a genetically heterogeneous disorder affecting multiple organ systems and resulting in blindness, obesity, cognitive impairment, and congenital defects. Interest in the etiology of this disorder stems, in part, from the fact that patients with BBS develop common clinical problems, including obesity, diabetes and hypertension. Twelve genes independently causing BBS have been identified. The heterogeneity is explained by the existence of two BBS complexes, the BBSome consisting of seven known BBS proteins, and the BBS chaperone complex consisting of three known BBS proteins. The formation of the BBSome requires the function of the BBS chaperone complex. Both mouse and zebrafish data support a role for BBS genes in cilia function, and in intracellular and intraflagellar trafficking. From the work described here, a common primary function of BBS proteins has emerged, specifically the mediation and regulation of microtubule-based intracellular transport.

  11. SDCCAG8 Interacts with RAB Effector Proteins RABEP2 and ERC1 and Is Required for Hedgehog Signaling

    DEFF Research Database (Denmark)

    Airik, Rannar; Schueler, Markus; Airik, Merlin

    2016-01-01

    Recessive mutations in the SDCCAG8 gene cause a nephronophthisis-related ciliopathy with Bardet-Biedl syndrome-like features in humans. Our previous characterization of the orthologous Sdccag8gt/gt mouse model recapitulated the retinal-renal disease phenotypes and identified impaired DNA damage...... response signaling as an underlying disease mechanism in the kidney. However, several other phenotypic and mechanistic features of Sdccag8gt/gt mice remained unexplored. Here we show that Sdccag8gt/gt mice exhibit developmental and structural abnormalities of the skeleton and limbs, suggesting impaired...... Hedgehog (Hh) signaling. Indeed, cell culture studies demonstrate the requirement of SDCCAG8 for ciliogenesis and Hh signaling. Using an affinity proteomics approach, we demonstrate that SDCCAG8 interacts with proteins of the centriolar satellites (OFD1, AZI1), of the endosomal sorting complex (RABEP2, ERC...

  12. MOMO Syndrome with Holoprosencephaly and Cryptorchidism: Expanding the Spectrum of the New Obesity Syndrome

    Directory of Open Access Journals (Sweden)

    Sheetal Sharda

    2011-01-01

    Full Text Available There are multiple genetic disorders with known or unknown etiology grouped under obesity syndromes. Inspite of having multisystem involvement and often having a characteristic presentation, the understanding of the genetic causes in the majority of these syndromes is still lacking. The common obesity syndromes are Bardet-Biedl, Prader-Willi, Alstrom, Albright's hereditary osteodystrophy, Carpenter, Rubinstein-Taybi, Fragile X, and Börjeson-Forssman-Lehman syndrome. The list is ever increasing as new syndromes are being added to it. One of the recent additions is MOMO syndrome, with about five such cases being reported in literature. Expanding the spectrum of clinical features, we report the first case of MOMO syndrome from India with lobar variant of holoprosencephaly and cryptorchidism, which have not been reported previously.

  13. Cep70 and Cep131 contribute to ciliogenesis in zebrafish embryos

    Directory of Open Access Journals (Sweden)

    Carl Matthias

    2009-03-01

    Full Text Available Abstract Background The centrosome is the cell's microtubule organising centre, an organelle with important roles in cell division, migration and polarity. However, cells can divide and flies can, for a large part of development, develop without them. Many centrosome proteins have been identified but the roles of most are still poorly understood. The centrioles of the centrosome are similar to the basal bodies of cilia, hair-like extensions of many cells that have important roles in cell signalling and development. In a number of human diseases, such Bardet-Biedl syndrome, centrosome/cilium proteins are mutated, leading to polycystic kidney disease, situs inversus, and neurological problems, amongst other symptoms. Results We describe zebrafish (Danio rerio embryos depleted for two uncharacterised, centrosome proteins, Cep70 and Cep131. The phenotype of these embryos resembles that of zebrafish mutants for intraflagellar transport proteins (IFTs, with kidney and ear development affected and left-right asymmetry randomised. These organs and processes are those affected in Bardet-Biedl syndrome and other similar diseases. Like these diseases, the root cause of the phenotype lies, in fact, in dysfunctional cilia, which are shortened but not eliminated in several tissues in the morphants. Centrosomes and basal bodies, on the other hand, are present. Both Cep70 and Cep131 possess a putative HDAC (histone deacetylase interacting domain. However, we could not detect in yeast two-hybrid assays any interaction with the deacetylase that controls cilium length, HDAC6, or any of the IFTs that we tested. Conclusion Cep70 and Cep131 contribute to ciliogenesis in many tissues in the zebrafish embryo: cilia are made in cep70 and cep131 morphant zebrafish embryos but are shortened. We propose that the role of these centrosomal/basal body proteins is in making the cilium and that they are involved in determination of the length of the axoneme.

  14. STOOKE SMALL BODIES MAPS

    Data.gov (United States)

    National Aeronautics and Space Administration — The Stooke Small Bodies Map collection contains maps of small solar system bodies in various projections, with and without latitude/longiude grids. The maps are...

  15. Gender Segregation Small Firms

    OpenAIRE

    Kenneth R Troske; William J Carrington

    1992-01-01

    This paper studies interfirm gender segregation in a unique sample of small employers. We focus on small firms because previous research on interfirm segregation has studied only large firms and because it is easier to link the demographic characteristics of employers and employees in small firms. This latter feature permits an assessment of the role of employer discrimination in creating gender segregation. Our first finding is that interfirm segregation is prevalent among small employers. I...

  16. [Burnout syndrome associated factors in gynecologists].

    Science.gov (United States)

    Palmer-Morales, Yusvisaret; Prince-Vélez, Roberto; Searcy-Bernal, Roberto

    2007-07-01

    To determine risk factors associated to Burnout Syndrome in medical gynecologist staff who works at the Gynecology-pediatrics with Family Medicine Hospital number 31 of the IMSS at Mexicali city, Baja California. A cross sectional study was performed in 27 gynecologists (100% of the total gynecologist that works at that particular hospital). A validated survey instrument developed by Maslach was applied to them in order to identify the presence of the syndrome and a general questionnaire including sociodemographic information was also applied to the doctors. The statistical analysis was both descriptive and inferential including chi square, Student t and logistic regression. 74.1% of the doctors who showed signs of Burnout Syndrome were males and 25.9% were females. The average doctors age of the group who developed Burnout was 44.81 +/- 6.11 years. The mean time in years of work for the doctors with the syndrome was 15.56 +/- 7.11 years. Only one doctor was divorced and she presented Burnout Syndrome, on the other hand, 62.5% of the married doctors showed evidence of Burnout Syndrome (p > 0.05). The general prevalence was 59.2%. Of those who showed signs of burnout, 87.5% presented it in low degree, 6.2% presented moderate degree, 6.2% presented high burnout degree. Out of all the organizational factors type, none resulted significative. The prevalence of the reported syndrome is apparently high when compared to the medical anesthesiology staff of the same hospital; however this difference is not statistically significative. In the unvaried and multivariate analyses, no organizational factor shows significance as a potential risk. It is necessary to consider psychological support to the affected doctors and to value the organization of the service, looking for an administrative reengineering.

  17. Primary Sjogren's syndrome associated with inappropriate ...

    African Journals Online (AJOL)

    1990-03-30

    Mar 30, 1990 ... ethanol, paracetamol, barbiturates and benzodiazepines were negative. An ECG, chest radiography, blood gas analysis, thyroid function tests and the cortisol level were norm~ and screening for porphyrin in urine and stool was negative. Computed tomography of the brain and cerebrospinal fluid.

  18. Sneddon syndrome associated with Protein S deficiency

    Directory of Open Access Journals (Sweden)

    Refah Sayin

    2012-01-01

    Full Text Available Sneddon syndrome (SS is rare, arterio-occlusive disorder characterized by generalized livedo racemosa of the skin and various central nervous symptoms due to occlusion of medium-sized arteries of unknown. Seizure, cognitive impairment, hypertension, and history of repetitive miscarriages are the other symptoms seen in this disease. Livedo racemosa involves persisting irreversible skin lesions red or blue in color with irregular margins. Usually, SS occurs in women of childbearing age. Protein S deficiency is an inherited or acquired disorder associated with an increased risk of thrombosis. We present a 33-year-old woman with SS with diffuse livedo racemosa, recurrent cerebrovascular diseases, migraine-type headache, sinus vein thrombosis, and protein S deficiency. Protein S deficiency and with Sneddon syndrome rarely encountered in the literature.

  19. Poland's syndrome associated with chronic granulocytic leukemia.

    Science.gov (United States)

    Costa, R; Afonso, E; Benedito, M; Maricato, L

    1991-10-01

    Poland's syndrome has been sporadically associated with haematological neoplasms, namely acute lymphoblastic and myeloblastic leukaemias and non-Hodgkin's lymphomas. The authors present the case of a child in whom this syndrome coexists with a Philadelphia negative, chronic granulocytic leukaemia, which has only required one course of treatment with busulphan in two and a half years of follow-up.

  20. Syndromes Associated with Sexually Transmitted Infections

    Directory of Open Access Journals (Sweden)

    Max Chernesky

    2005-01-01

    Full Text Available Excellent technologies have been developed to identify the specific microbial agents of chlamydia, gonorrhea, syphilis, herpes, chancroid, trichomoniasis, human papillomavirus and HIV infection. However, it is also crucial to recognize syndromes that may be caused by one or more sexually transmitted pathogens. When laboratory services are lacking or are inadequate to provide timely results to enable appropriate treatment, some patients must be managed and treated syndromically. Most Canadian laboratories should be able to provide diagnostic services to determine the etiology of syndromes such as cervicitis, urethritis, pelvic inflammatory disease, prostatitis, genital ulcers, sexually transmitted infection (STI-related enteric infections, epididymitis, hepatitis, ophthalmia neonatorum, vulvovaginitis and vaginosis.

  1. Restless legs syndrome associated with major diseases

    Science.gov (United States)

    Allen, Richard; Högl, Birgit; Paulus, Walter

    2016-01-01

    Recent publications on both the genetics and environmental factors of restless legs syndrome (RLS) defined as a clinical disorder suggest that overlapping genetic risk factors may play a role in primary (idiopathic) and secondary (symptomatic) RLS. Following a systematic literature search of RLS associated with comorbidities, we identified an increased prevalence of RLS only in iron deficiency and kidney disease. In cardiovascular disease, arterial hypertension, diabetes, migraine, and Parkinson disease, the methodology of studies was poor, but an association might be possible. There is insufficient evidence for conditions such as anemia (without iron deficiency), chronic obstructive pulmonary disease, multiple sclerosis, headache, stroke, narcolepsy, and ataxias. Based on possible gene–microenvironmental interaction, the classifications primary and secondary RLS may suggest an inappropriate causal relation. We recognize that in some conditions, treatment of the underlying disease should be achieved as far as possible to reduce or eliminate RLS symptoms. RLS might be seen as a continuous spectrum with a major genetic contribution at one end and a major environmental or comorbid disease contribution at the other. PMID:26944272

  2. Asperger Syndrome: Associated Psychiatric and Medical Conditions.

    Science.gov (United States)

    Ghaziuddin, Mohammad

    2002-01-01

    This article explores the association of medical and psychiatric conditions with Asperger syndrome, based mainly on publications from the last two decades. It examines comorbidity of Asperger syndrome with mood disorders, schizophrenia, obsessive-compulsive disorder, attention deficit/hyperactivity disorder, tic disorders, violence and aggression,…

  3. Carmi syndrome associated with gastric perforation

    African Journals Online (AJOL)

    Here, we report a case of Carmi syndrome in a neonate who presented with an uncommon complication of gastric perforation. Ann Pediatr Surg 9:122–123 c 2013 Annals of. Pediatric ... Here, we report these three uncommon conditions, that is, CPA and EB ... membrane resulting from minor mechanical friction or trauma.

  4. Hemophagocytic Syndrome Associated with Mycoplasma pneumoniae Pneumonia

    Directory of Open Access Journals (Sweden)

    Yuji Koike

    2013-01-01

    Full Text Available Mycoplasma pneumoniae (Mp sometimes causes immunological complications in children. We present a rare case of hemophagocytic syndrome (HPS caused by Mp in a previously healthy 7-year-old Japanese girl. A chest radiograph obtained to evaluate the source of her fever showed infiltration in the lower right lung with mild splenomegaly. We could diagnose the patient with HPS on the basis of the hemophagocytic-lymphohistiocytosis- (HLH 2004 criteria. She met the criteria for fever, splenomegaly, neutrophil count (265 mg/dL, and ferritin level (>500 ng/mL. Furthermore, a peripheral blood smear showed an increased number of monocytes/macrophages with erythrophagocytosis. Treatment with clarithromycin and prednisolone, which was initiated soon after the diagnosis, was successful. Mp infection might partly progress to HPS in certain conditions. Clinicians should be aware of HPS caused by Mp and start appropriate treatment as soon as possible if the disease is suspected.

  5. Plasma exchange in Goodpasture syndrome associated with ...

    African Journals Online (AJOL)

    Background: Good pasture syndrome (GPS) has been paid much attention recently for the dangerous illnessand high mortality. Objective: To investigate the efficiency of plasma exchange (PE) to treat Goodpasture syndrome (GPS) in children associated with Turner's syndrome. Method: We report a case of a 15 year old ...

  6. Vena cava superior syndrome associated with sarcoidosis

    International Nuclear Information System (INIS)

    Wurm, K.; Walz, M.; Reidemeister, J.C.; Donhuijsen, K.

    1988-01-01

    We report the first observation of clinical manifestations of vena cava superior syndrome (VCSS) associated with sarcoidosis. Twenty-four years after the first signs of the disease had been noted, mediastinal lymphomas penetrating the wall of the vena cava superior caused complete obstruction. It is most unusual for the vessel wall to be destroyed in this way, which explains why VCSS is often missed in sarcoidosis. The obstructed vessel was resected and successfully replaced by a Gore-Tex prosthesis. The importance of VCSS for the differential diagnosis is pointed out. Two further peculiarities are the simultaneous occurrence of elevated intraocular pressure and VCSS, and the familial incidence. (orig.) [de

  7. Psychiatric syndromes associated with atypical chest pain

    Directory of Open Access Journals (Sweden)

    Nikolić Gordana

    2010-01-01

    Full Text Available Background/Aim. Chest pain often indicates coronary disease, but in 25% of patients there is no evidence of ischemic heart disease using standard diagnostic tests. Beside that, cardiologic examinations are repeated several times for months. If other medical causes could not be found, there is a possibility that chest pain is a symptom of psychiatric disorder. The aim of this study was to determine the presence of psychiatric syndromes, increased somatization, anxiety, stress life events exposure and characteristic of chest pain expression in persons with atypical chest pain and coronary patients, as well as to define predictive parameters for atypical chest pain. Method. We compared 30 patients with atypical chest pain (E group to 30 coronary patients (K group, after cardiological and psychiatric evaluation. We have applied: Mini International Neuropsychiatric Interview (MINI, The Symptom Checklist 90-R (SCL-90 R, Beck Anxiety Inventory (BAI, Holms-Rahe Scale of stress life events (H-R, Questionnaire for pain expression Pain-O-Meter (POM. Significant differences between groups and predictive value of the parameters for atypical chest pain were determined. Results. The E group participants compared to the group K were younger (33.4 ± 5.4 : 48.3 ± 6,4 years, p < 0.001, had a moderate anxiety level (20.4 ± 11.9 : 9.6 ± 3.8, p < 0.001, panic and somatiform disorders were present in the half of the E group, as well as eleveted somatization score (SOM ≥ 63 -50% : 10%, p < 0.01 and a higher H-R score level (102.0 ± 52.2 : 46.5 ± 55.0, p < 0.001. Pain was mild, accompanied with panic. The half of the E group subjects had somatoform and panic disorders. Conclusion. Somatoform and panic disorders are associated with atypical chest pain. Pain expression is mild, accompained with panic. Predictive factors for atypical chest pain are: age under 40, anxiety level > 20, somatization ≥ 63, presence of panic and somatoform disorders, H-R score > 102, and a lack of positive diagnostic test of coronary disease. Defining of these parameters could be useful for early psychiatric evaluation of persons with atypical chest pain.

  8. Primary Sjogren's syndrome associated with inappropriate ...

    African Journals Online (AJOL)

    A patient in whom primary Sjogren's syndrome and inappropriate antiduretic hormone secretion were associated is reported. This is the first report of such an association. The possible pathophysiological mechanisms are discussed and vasculitis proposed as the underlying pathogenetic mechanism.

  9. Parinaud's oculoglandular syndrome associated with Sporothrix schenckii

    Directory of Open Access Journals (Sweden)

    Cassio Porto Ferreira

    2014-01-01

    Full Text Available The Parinaud oculoglandular syndrome is a rare eye disease caused by different etiologic agents, including bacteria, viruses and fungi. It is characterized by a granulomatous conjunctivitis, accompanied by adjacent preauricular lymphadenopathy and can bring consequences if not treated promptly. We present a case of Parinaud oculoglandular syndrome caused by Sporothrix schenckii from the sporotrichosis epidemic in its zoonotic form occurring in Rio de Janeiro, Brazil.

  10. Fragile X syndrome associated with tic disorders.

    Science.gov (United States)

    Schneider, Susanne A; Robertson, Mary M; Rizzo, Renata; Turk, Jeremy; Bhatia, Kailash P; Orth, Michael

    2008-06-15

    Movement disorders other than late onset tremor-ataxia in association with fragile X syndrome, the most common identifiable cause of inherited mental retardation, seem to be rare. Here we describe five male patients from three unrelated families with fragile X syndrome that presented with motor and phonic tics. Clinically, 4 patients fulfilled diagnostic criteria for Gilles de la Tourette syndrome (GTS) while 1 patient would have been diagnosed with an adult onset tic disorder. However, in all patients onset of tics was considerably later than in typical GTS. Three patients had atypical tics and two patients reported waxing and waning of tic intensity over time. Four of the 5 patients showed clinical signs typical of fragile X syndrome, in particular dysmorphic features, learning difficulties and speech and language problems that required special treatment. All patients had co-morbidities common to both GTS and fragile X syndrome. We suggest considering fragile X syndrome in GTS complicated by co-morbidity with late onset of atypical tics, in particular when learning disability and dysmorphic features are present. (c) 2008 Movement Disorder Society

  11. Posterior Reversible Leukoencephalopathy Syndrome Associated with Pazopanib

    Directory of Open Access Journals (Sweden)

    Robert Foerster

    2013-04-01

    Full Text Available A 62-year-old female patient with metastatic renal cell carcinoma under third-line treatment with pazopanib for 8 weeks suddenly developed severe headaches, grand mal seizures and paresis of the left arm in combination with gait instability as well as nausea and vomiting during her vacation abroad. The emergency physician measured systolic blood pressure values over 300 mm Hg and suspected a stroke. The CT imaging without contrast agent in a local hospital did not show any pathologic findings despite bone metastases. The colleagues suspected cerebral metastases or meningeosis carcinomatosa and referred the patient to our department for further diagnostics and treatment planning. An MRI scan ruled out the suspected cerebral metastases or meningeosis carcinomatosa, but showed signs of reversible posterior leukoencephalopathy syndrome (RPLS in the form of band-like hyperintensities as a sign of cytotoxic edema in the gray and white matter of the left parietal lobe. The patient then reported that similar blood pressure values had been measured shortly after the start of a first-line therapy with sunitinib, so that we discontinued the current treatment with pazopanib. Within 6 days the neurologic symptoms vanished and the patient was discharged. An intermittent hypertension persisted. A follow-up MRI 3 weeks later showed an RPLS-typical cortical infarction in the affected area. RPLS should be considered as the actual reason for neurologic findings in hypertensive patients with known metastatic cancers under tyrosine kinase inhibitor therapy.

  12. Genetic syndromes associated with overgrowth in childhood

    Directory of Open Access Journals (Sweden)

    Jung Min Ko

    2013-09-01

    Full Text Available Overgrowth syndromes comprise a diverse group of conditions with unique clinical, behavioral and molecular genetic features. While considerable overlap in presentation sometimes exists, advances in identification of the precise etiology of specific overgrowth disorders continue to improve clinicians' ability to make an accurate diagnosis. Among them, this paper introduces two classic genetic overgrowth syndromes: Sotos syndrome and Beckwith-Wiedemann syndrome. Historically, the diagnosis was based entirely on clinical findings. However, it is now understood that Sotos syndrome is caused by a variety of molecular genetic alterations resulting in haploinsufficiency of the NSD1 gene at chromosome 5q35 and that Beckwith-Wiedemann syndrome is caused by heterogeneous abnormalities in the imprinting of a number of growth regulatory genes within chromosome 11p15 in the majority of cases. Interestingly, the 11p15 imprinting region is also associated with Russell-Silver syndrome which is a typical growth retardation syndrome. Opposite epigenetic alterations in 11p15 result in opposite clinical features shown in Beckwith-Wiedemann syndrome and Russell-Silver syndrome. Although the exact functions of the causing genes have not yet been completely understood, these overgrowth syndromes can be good models to clarify the complex basis of human growth and help to develop better-directed therapies in the future.

  13. [Neurological syndromes associated with homocystein dismetabolism].

    Science.gov (United States)

    Shirokov, E A; Leonova, S F

    2006-01-01

    The article summarizes the results of clinical, neurological, and laboratory examination of patients with hyperhomocysteinemia. The data obtained suggest the existence of common pathobiochemical mechanisms of homocystein, cholesterol, and myelin dysmetabolism. The authors demonstrate that neurological manifestations of hyperhomocysteinemia are associated with the processes of demyelinization in the central and peripheral nervous systems.

  14. Goodpasture's syndrome associated with pulmonary eosinophilic vasculitis.

    Science.gov (United States)

    Komadina, K H; Houk, R W; Vicks, S L; Desrosier, K F; Ridley, D J; Boswell, R N

    1988-08-01

    Lung hemorrhage and antiglomerular basement membrane (anti-GBM) antibody mediated nephritis define Goodpasture's syndrome. We present the case of a 19-year-old Caucasian woman with unique clinical findings of Goodpasture's syndrome. Our patient initially presented with leukocytoclastic vasculitis of the skin followed by the development of nephritis and lung hemorrhage. An open lung biopsy done prior to diagnosing anti-GBM antibody disease demonstrated an intense eosinophilic vasculitis. Skin vasculitis has only been rarely reported, and to our knowledge this is the first reported case of pulmonary eosinophilic vasculitis associated with Goodpasture's syndrome.

  15. A Hypereosinophilic Syndrome Associated with HIV Infection

    Science.gov (United States)

    1992-01-01

    mucosa of patients with coeliac disease . J Exp Med 1992; 175: 293 29. Enockihara H, Kajitani H, Nagashima S, et al. Interleukin-5 activity in sera...exhibited clinical and histopathologic similarities to the idiopathic hypereominophilic syndrome as well as acute graft -versus -host disease . A serum...MD **** Paul M Benson LTC MC USA * and the Military Medical Consortium for Applied Retroviral Research * Department of Bacterial Diseases Walter Reed

  16. Toxic metabolic syndrome associated with HAART

    DEFF Research Database (Denmark)

    Haugaard, Steen B

    2006-01-01

    (HAART) may encounter the HIV-associated lipodystrophy syndrome (HALS), which attenuates patient compliance to this treatment. HALS is characterised by impaired glucose and lipid metabolism and other risk factors for cardiovascular disease. This review depicts the metabolic abnormalities associated...... with HAART by describing the key cell and organ systems that are involved, emphasising the role of insulin resistance. An opinion on the remedies available to treat the metabolic abnormalities and phenotype of HALS is provided....

  17. Hemolytic uremic syndrome associated with paraquat intoxication.

    Science.gov (United States)

    Jang, Ha Nee; Bae, Eun Jin; Hwang, Kyungo; Kang, Yeojin; Yun, Seongeun; Cho, Hyun Seop; Chang, Se-Ho; Park, Dong Jun

    2014-06-01

    We report a case of a 66-year-old patient with paraquat intoxication resulting in the requirement for hemoperfusion, hemodialysis, and plasma exchange. His initial serum paraquat level was 0.24 µg/mL (0.0-0.1 µg/mL). Activated charcoal (50 g) was administered orally, and high-dose N-acetylcysteine (150 mg/kg) was administered intravenously. In addition, immediate 4 h hemoperfusion was also performed for three consecutive days after admission. Hemodialysis was started on the 4th day after admission because of uremia. On the 9th day after admission, laboratory findings demonstrated hemolytic uremic syndrome (HUS): microangiopathic hemolytic anemia (MAHA), thrombocytopenia, elevated reticulocyte count, and lactate dehydrogenase (LDH). Plasma exchange was performed three times consecutively. Anemia and thrombocytopenia were improved, and LDH was normalized after plasma exchange. Urine output increased to 2240 mL/day on the 18th day after admission, and hemodialysis was discontinued. He is currently being observed at our follow-up clinic without renal impairment or pulmonary dysfunction for 1.5 years since discharge. We should suspect paraquat-associated HUS when thrombocytopenia and anemia are maintained for a long time after paraquat intoxication. © 2013 Wiley Periodicals, Inc.

  18. Mosaic Turner syndrome associated with schizophrenia.

    Science.gov (United States)

    Jung, Sook Young; Park, Joo Won; Kim, Dong Hyun; Jun, Yong Hoon; Lee, Jeong Seop; Lee, Ji Eun

    2014-03-01

    Turner syndrome is a sex-chromosome disorder; occurring in 1 in 2,500 female births. There are sporadic few case reports of concomitant Turner syndrome with schizophrenia worldwide. Most Turner females had a 45,X monosomy, whereas the majority of comorbidity between Turner syndrome and schizophrenia had a mosaic karyotype (45,X/46,XX). We present a case of a 21-year-old woman with Turner syndrome, mosaic karyotype (45,X/46,XX), showing mental retardation, hypothyroidism, and schizophrenia. HOPA gene within Xq13 is related to mental retardation, hypothyroidism, and schizophrenia. Our case may be a potential clue which supports the hypothesis for involvement of genes on X chromosome in development of schizophrenia. Further studies including comorbid cases reports are need in order to discern the cause of schizophrenia in patients having Turner syndrome.

  19. Validity and reliability of the Spanish version of the DN4 (Douleur Neuropathique 4 questions questionnaire for differential diagnosis of pain syndromes associated to a neuropathic or somatic component

    Directory of Open Access Journals (Sweden)

    Bouhassira Didier

    2007-12-01

    Full Text Available Abstract Background This study assesses the validity and reliability of the Spanish version of DN4 questionnaire as a tool for differential diagnosis of pain syndromes associated to a neuropathic (NP or somatic component (non-neuropathic pain, NNP. Methods A study was conducted consisting of two phases: cultural adaptation into the Spanish language by means of conceptual equivalence, including forward and backward translations in duplicate and cognitive debriefing, and testing of psychometric properties in patients with NP (peripheral, central and mixed and NNP. The analysis of psychometric properties included reliability (internal consistency, inter-rater agreement and test-retest reliability and validity (ROC curve analysis, agreement with the reference diagnosis and determination of sensitivity, specificity, and positive and negative predictive values in different subsamples according to type of NP. Results A sample of 164 subjects (99 women, 60.4%; age: 60.4 ± 16.0 years, 94 (57.3% with NP (36 with peripheral, 32 with central, and 26 with mixed pain and 70 with NNP was enrolled. The questionnaire was reliable [Cronbach's alpha coefficient: 0.71, inter-rater agreement coefficient: 0.80 (0.71–0.89, and test-retest intra-class correlation coefficient: 0.95 (0.92–0.97] and valid for a cut-off value ≥ 4 points, which was the best value to discriminate between NP and NNP subjects. Discussion This study, representing the first validation of the DN4 questionnaire into another language different than the original, not only supported its high discriminatory value for identification of neuropathic pain, but also provided supplemental psychometric validation (i.e. test-retest reliability, influence of educational level and pain intensity and showed its validity in mixed pain syndromes.

  20. Small cell glioblastoma or small cell carcinoma

    DEFF Research Database (Denmark)

    Hilbrandt, Christine; Sathyadas, Sathya; Dahlrot, Rikke H

    2013-01-01

    was admitted to the hospital with left-sided loss of motor function. A MRI revealed a 6 cm tumor in the right temporoparietal area. The histology was consistent with both glioblastoma multiforme (GBM) and small cell lung carcinoma (SCLC) but IHC was suggestive of a SCLC metastasis. PET-CT revealed...

  1. Small Quantum Structures with Small State Spaces

    Science.gov (United States)

    Navara, Mirko

    2008-01-01

    We summarize and extend results about “small” quantum structures with small dimensions of state spaces. These constructions have contributed to the theory of orthomodular lattices. More general quantum structures (orthomodular posets, orthoalgebras, and effect algebras) admit sometimes simplifications, but there are problems where no progress has been achieved.

  2. Small Vessel Security Strategy

    National Research Council Canada - National Science Library

    2008-01-01

    ...), narcotics, aliens, and other contraband, and other criminals. Small vessels have also been successfully employed overseas by terrorists to deliver Waterborne Improvised Explosive Devices (WBIEDs...

  3. Small Intestine Disorders

    Science.gov (United States)

    ... disease Crohn's disease Infections Intestinal cancer Intestinal obstruction Irritable bowel syndrome Ulcers, such as peptic ulcer Treatment of disorders of the small intestine depends on the cause.

  4. Small Wastewater Systems Research

    Science.gov (United States)

    Small communities face barriers to building and maintaining effective wastewater treatment services, challenges include financial/economic limitations, lack of managerial training and geographic isolation/remoteness.

  5. Small Intestine Cancer Treatment

    Science.gov (United States)

    ... all of an organ that contains cancer. The resection may include the small intestine and nearby organs (if the cancer has spread). The doctor may remove the section of the small intestine that contains cancer and perform an anastomosis (joining the cut ends of the intestine together). ...

  6. Small School Reform

    Directory of Open Access Journals (Sweden)

    Carroll E. Bronson

    2013-05-01

    Full Text Available This qualitative ethnographic case study explored the evolution of a public urban high school in its 3rd year of small school reform. The study focused on how the high school proceeded from its initial concept, moving to a small school program, and emerging as a new small high school. Data collection included interviews, observations, and document review to develop a case study of one small high school sharing a multiplex building. The first key finding, “Too Many Pieces, Not Enough Glue,” revealed that the school had too many new programs starting at once and they lacked a clear understanding of their concept and vision for their new small school, training on the Montessori philosophies, teaching and learning in small schools, and how to operate within a teacher-cooperative model. The second key finding, “A Continuous Struggle,” revealed that the shared building space presented problems for teachers and students. District policies remain unchanged, resulting in staff and students resorting to activist approaches to get things done. These findings offer small school reform leaders suggestions for developing and sustaining a small school culture and cohesion despite the pressures to revert back to top-down, comprehensive high school norms.

  7. Industrial Education. "Small Engines".

    Science.gov (United States)

    Parma City School District, OH.

    Part of a series of curriculum guides dealing with industrial education in junior high schools, this guide provides the student with information and manipulative experiences on small gasoline engines. Included are sections on shop adjustment, safety, small engines, internal combustion, engine construction, four stroke engines, two stroke engines,…

  8. Small Business Tax Regimes

    OpenAIRE

    Yilmaz, Fatih; Coolidge, Jacqueline

    2016-01-01

    Simplified tax regimes for micro and small enterprises in developing countries are intended to facilitate voluntary tax compliance. However, survey evidence suggests that small business taxation based on simplified bookkeeping or turnover is sometimes perceived as too complex for microenterprises in countries with high illiteracy levels. Very simple fixed tax regimes not requiring any book...

  9. Epitope mapping and biological function analysis of antibodies produced by immunization of mice with an inactivated Chinese isolate of severe acute respiratory syndrome-associated coronavirus (SARS-CoV)

    International Nuclear Information System (INIS)

    Chou, Te-hui W.; Wang, Shixia; Sakhatskyy, Pavlo V.; Mboudoudjeck, Innocent; Lawrence, John M.; Huang Song; Coley, Scott; Yang Baoan; Li Jiaming; Zhu Qingyu; Lu Shan

    2005-01-01

    Inactivated severe acute respiratory syndrome-associated coronavirus (SARS-CoV) has been tested as a candidate vaccine against the re-emergence of SARS. In order to understand the efficacy and safety of this approach, it is important to know the antibody specificities generated with inactivated SARS-CoV. In the current study, a panel of twelve monoclonal antibodies (mAbs) was established by immunizing Balb/c mice with the inactivated BJ01 strain of SARS-CoV isolated from the lung tissue of a SARS-infected Chinese patient. These mAbs could recognize SARS-CoV-infected cells by immunofluorescence analysis (IFA). Seven of them were mapped to the specific segments of recombinant spike (S) protein: six on S1 subunit (aa 12-798) and one on S2 subunit (aa 797-1192). High neutralizing titers against SARS-CoV were detected with two mAbs (1A5 and 2C5) targeting at a subdomain of S protein (aa 310-535), consistent with the previous report that this segment of S protein contains the major neutralizing domain. Some of these S-specific mAbs were able to recognize cleaved products of S protein in SARS-CoV-infected Vero E6 cells. None of the remaining five mAbs could recognize either of the recombinant S, N, M, or E antigens by ELISA. This study demonstrated that the inactivated SARS-CoV was able to preserve the immunogenicity of S protein including its major neutralizing domain. The relative ease with which these mAbs were generated against SARS-CoV virions further supports that subunit vaccination with S constructs may also be able to protect animals and perhaps humans. It is somewhat unexpected that no N-specific mAbs were identified albeit anti-N IgG was easily identified in SARS-CoV-infected patients. The availability of this panel of mAbs also provided potentially useful agents with applications in therapy, diagnosis, and basic research of SARS-CoV

  10. SmallSat Database

    Science.gov (United States)

    Petropulos, Dolores; Bittner, David; Murawski, Robert; Golden, Bert

    2015-01-01

    The SmallSat has an unrealized potential in both the private industry and in the federal government. Currently over 70 companies, 50 universities and 17 governmental agencies are involved in SmallSat research and development. In 1994, the U.S. Army Missile and Defense mapped the moon using smallSat imagery. Since then Smart Phones have introduced this imagery to the people of the world as diverse industries watched this trend. The deployment cost of smallSats is also greatly reduced compared to traditional satellites due to the fact that multiple units can be deployed in a single mission. Imaging payloads have become more sophisticated, smaller and lighter. In addition, the growth of small technology obtained from private industries has led to the more widespread use of smallSats. This includes greater revisit rates in imagery, significantly lower costs, the ability to update technology more frequently and the ability to decrease vulnerability of enemy attacks. The popularity of smallSats show a changing mentality in this fast paced world of tomorrow. What impact has this created on the NASA communication networks now and in future years? In this project, we are developing the SmallSat Relational Database which can support a simulation of smallSats within the NASA SCaN Compatability Environment for Networks and Integrated Communications (SCENIC) Modeling and Simulation Lab. The NASA Space Communications and Networks (SCaN) Program can use this modeling to project required network support needs in the next 10 to 15 years. The SmallSat Rational Database could model smallSats just as the other SCaN databases model the more traditional larger satellites, with a few exceptions. One being that the smallSat Database is designed to be built-to-order. The SmallSat database holds various hardware configurations that can be used to model a smallSat. It will require significant effort to develop as the research material can only be populated by hand to obtain the unique data

  11. Wind: small is beautiful

    International Nuclear Information System (INIS)

    Vries, E. de

    2005-01-01

    The small wind sector (0.5-100 kW) is often overlooked but could provide decentralised energy systems. Small wind turbines have been used for homes, farms and small businesses for over 80 years (e.g. in the USA and the Netherlands), receiving a boost in the 1970s and 1980s following the 1973 oil crisis when a new generation of turbines entered the European and US markets. Bergey Windpower and Southwest Windpower from the USA are the market leaders in this sector in terms of sales volume but are still classed as medium-sized enterprises. Small turbines have the disadvantage of higher costs compared with large turbines due to higher manufacturing costs, technical factors associated with the tendency to use small turbines on relatively short towers, small production runs and a failure to keep up with the latest design developments such as cost-effective state-of-the-art frequency converters. Most small turbines are horizontal axis turbines, though vertical axis turbines are produced by some manufacturers. Examples of the systems available from European suppliers are described

  12. 78 FR 11745 - Small Business Size Regulations, Small Business Innovation Research (SBIR) Program and Small...

    Science.gov (United States)

    2013-02-20

    ..., Small Business Innovation Research (SBIR) Program and Small Business Technology Transfer (STTR) Program... Innovation Research (SBIR) and Small Business Technology Transfer (STTR) programs. DATES: Effective February...

  13. Small scale optics

    CERN Document Server

    Yupapin, Preecha

    2013-01-01

    The behavior of light in small scale optics or nano/micro optical devices has shown promising results, which can be used for basic and applied research, especially in nanoelectronics. Small Scale Optics presents the use of optical nonlinear behaviors for spins, antennae, and whispering gallery modes within micro/nano devices and circuits, which can be used in many applications. This book proposes a new design for a small scale optical device-a microring resonator device. Most chapters are based on the proposed device, which uses a configuration know as a PANDA ring resonator. Analytical and nu

  14. Small - Display Cartography

    DEFF Research Database (Denmark)

    Nissen, Flemming; Hvas, Anders; Münster-Swendsen, Jørgen

    This report comprises the work carried out in the work-package of small display cartography. The work-package has aimed at creating a general framework for the small-display cartography. A solid framework facilitates an increased use of spatial data in mobile devices - thus enabling, together...... Service Communication and finally, Part IV: Concluding remarks and topics for further research on small-display cartography. Part II includes a separate Appendix D consisting of a cartographic design specification. Part III includes a separate Appendix C consisting of a schema specification, a separate...

  15. Small satellite product assurance

    Science.gov (United States)

    Demontlivault, J.; Cadelec, Jacques

    1993-01-01

    In order to increase the interest in small satellites, their cost must be reduced; reducing product assurance costs induced by quality requirements is a major objective. For a logical approach, small satellites are classified in three main categories: satellites for experimental operations with a short lifetime, operational satellites manufactured in small mass with long lifetime requirements, operational satellites (long lifetime required), of which only a few models are produced. The various requirements as regards the product assurance are examined for each satellite category: general requirements for space approach, reliability, electronic components, materials and processes, quality assurance, documentation, tests, and management. Ideal product assurance system integrates quality teams and engineering teams.

  16. Lightweight Small Arms Technologies

    National Research Council Canada - National Science Library

    Spiegel, Kori; Shipley, Paul

    2006-01-01

    .... The Lightweight Small Arms Technologies program was established to address this critical issue. The goals of the program prioritize weight reduction over any other characteristic, while balancing the requirements of lethality, reliability, and cost...

  17. The Power of Small

    Indian Academy of Sciences (India)

    Home; Journals; Resonance – Journal of Science Education; Volume 20; Issue 12. The Power of Small: Championing the Underdogs of Modern Medicine. Suvasini Ramaswamy Anirban Mitra. General Article Volume 20 Issue 12 December 2015 pp 1136-1153 ...

  18. Small Satellite Transporter Project

    Data.gov (United States)

    National Aeronautics and Space Administration — The primary objective is to determine whether this small satellite transporter is capable of transporting at least four 6U CubeSats is possible for a given set of...

  19. Small bowel bacterial overgrowth

    Science.gov (United States)

    ... Surgical procedures that create a loop of small intestine where excess bacteria can grow. An example is a Billroth II type of stomach removal ( gastrectomy ). Some cases of irritable bowel syndrome (IBS).

  20. Small-x physics

    Energy Technology Data Exchange (ETDEWEB)

    Mueller, A.H. [Columbia Univ., New York, NY (United States). Dept. of Physics

    1997-06-01

    After a brief review of the kinematics of deep inelastic lepton-proton scattering, the parton model is described. Small-x behavior coming from DGLAP evolution and from BFKL evolution is discussed, and the two types of evolution are contrasted and compared. Then a more detailed discussion of BFKL dynamics is given. The phenomenology of small-x physics is discussed with an emphasis on ways in which BFKL dynamics may be discussed and measured. 45 refs., 12 figs.

  1. Small - Display Cartography

    OpenAIRE

    Nissen, Flemming; Hvas, Anders; Münster-Swendsen, Jørgen; Brodersen, Lars

    2003-01-01

    This report comprises the work carried out in the work-package of small display cartography. The work-package has aimed at creating a general framework for the small-display cartography. A solid framework facilitates an increased use of spatial data in mobile devices - thus enabling, together with the rapidly evolving positioning techniques, a new category of position-dependent, map-based services to be introduced. The report consists of the following parts: Part I: Categorization of handheld...

  2. Research using small tokamaks

    International Nuclear Information System (INIS)

    1989-07-01

    These proceedings of the IAEA-sponsored meeting held in Nice, France 10-11 October, 1988, contain the manuscripts of the 21 reports dealing with research using small tokamaks. The purpose of this meeting was to highlight some of the achievements of small tokamaks and alternative magnetic confinement concepts and assess the suitability of starting new programs, particularly in developing countries. Papers presented were either review papers, or were detailed descriptions of particular experiments or concepts. Refs, figs and tabs

  3. Small test SDHW systems

    DEFF Research Database (Denmark)

    Vejen, Niels Kristian

    1999-01-01

    Three small test SDHW systems was tested in a laboratory test facility.The three SDHW systems where all based on the low flow principe and a mantle tank but the design of the systems where different.......Three small test SDHW systems was tested in a laboratory test facility.The three SDHW systems where all based on the low flow principe and a mantle tank but the design of the systems where different....

  4. Small wind turbine

    OpenAIRE

    Vélez Castellano, Didier

    2010-01-01

    The main objective is to develop a project on installing a small wind turbine at the University of Glyndwr in Wrexham Wales. Today are immersed in a world seeking clean energy for reduce greenhouse gases because this problem is becoming a global reality. So installing a small wind turbine at the university would provide large quantity of clean energy to supply a workshop and also reduce the expulsion of CO2 into the atmosphere. The main characteristic of the turbine under...

  5. Small woodland ownership management

    Science.gov (United States)

    Albert J. Childs

    1977-01-01

    Small woodlot ownerships are a commodity on the real estate market which have cycled through a supply and demand curve on a somewhat irregular basis. In order to have some understanding of what it is we are talking about, it is necessary to define what a small woodlot is, and where it may be found. In size these parcels can range from ten acres to fifteen acres on up...

  6. How small is a small cloud?

    Directory of Open Access Journals (Sweden)

    I. Koren

    2008-07-01

    Full Text Available The interplay between clouds and aerosols and their contribution to the radiation budget is one of the largest uncertainties of climate change. Most work to date has separated cloudy and cloud-free areas in order to evaluate the individual radiative forcing of aerosols, clouds, and aerosol effects on clouds.

    Here we examine the size distribution and the optical properties of small, sparse cumulus clouds and the associated optical properties of what is considered a cloud-free atmosphere within the cloud field. We show that any separation between clouds and cloud free atmosphere will incur errors in the calculated radiative forcing.

    The nature of small cumulus cloud size distributions suggests that at any resolution, a significant fraction of the clouds are missed, and their optical properties are relegated to the apparent cloud-free optical properties. At the same time, the cloudy portion incorporates significant contribution from non-cloudy pixels.

    We show that the largest contribution to the total cloud reflectance comes from the smallest clouds and that the spatial resolution changes the apparent energy flux of a broken cloudy scene. When changing the resolution from 30 m to 1 km (Landsat to MODIS the average "cloud-free" reflectance at 1.65 μm increases from 0.0095 to 0.0115 (>20%, the cloud reflectance decreases from 0.13 to 0.066 (~50%, and the cloud coverage doubles, resulting in an important impact on climate forcing estimations. The apparent aerosol forcing is on the order of 0.5 to 1 Wm−2 per cloud field.

  7. Small intestine and microbiota.

    Science.gov (United States)

    Cotter, Paul D

    2011-03-01

    To highlight the recent studies which have enhanced our appreciation of the composition of the microbiota in the human small intestine and its relevance to the health of the host. In the past number of years, the composition of the microorganisms present in our small intestines has been the subject of greater scrutiny than ever before. These investigations have been possible as a consequence of the development and utilization of new molecular tools which have revolutionized the field of microbial ecology and have focused predominantly on the small intestinal microbiota associated with pediatric celiac disease, inflammatory bowel disease, irritable bowel syndrome and pouchitis. The impact of invasive procedures, such as small bowel transplant, ileostomy and ileal pouch anal anastomosis, on the ileal microbiota has also been investigated. The ever greater appreciation of the link between the small intestinal microbiota and the health status of the host has the potential to lead to the development of new strategies to alter this microbiota in a targeted way to prevent or treat specific disorders.

  8. Small intestine diverticuli

    International Nuclear Information System (INIS)

    Pomakov, P.; Risov, A.

    1991-01-01

    The routine method of contrast matter passage applied to 850 patients with different gastrointestinal diseases proved inefficient to detect any small-intestinal diverticuli. The following modiffications of the method have been tested in order to improve the diagnostic possibilities of the X-ray: study at short intervals, assisted passage, enteroclysm, pharmacodynamic impact, retrograde filling of the ileum by irrigoscopy. Twelve diverticuli of the small-intestinal loops were identified: 5 Meckel's diverticuli, 2 solitary of which one of the therminal ileum, 2 double diverticuli and 1 multiple diverticulosis of the jejunum. The results show that the short interval X-ray examination of the small intestines is the method of choice for identifying local changes in them. The solitary diverticuli are not casuistic scarcity, its occurrence is about 0.5% at purposeful X-ray investigation. The assisted passage method is proposed as a method of choice for detection of the Meckel's diverticulum. 5 figs., 3 tabs. 18 refs

  9. Small public private partnerships

    DEFF Research Database (Denmark)

    Koch, Christian; Jensen, Jesper Ole

    2009-01-01

    businesses and represent operable units for the clients and citizens. Cases are focused within education and healthcare. The analysis suggests that another type of economy apparently is in play. It is thus characteristic within education projects in UK that the largest portion of small PPPs are of a size......Public Private Partnerships (PPP) are frequently mobilized as a purchasing form suitable for large infrastructure projects. And it is commonly assumed that transaction costs linked to the establishment of PPP make them prohibitive in small sizes. In a Danish context this has been safeguarded...... by the authorities, which recommend sizes over 13,5 million € (100 million DKr). PPP is here understood as Design, Build, Finance and Operate projects. The paper shows, when looking at Germany, Italy and United Kingdom, that small PPP (below 13,5 million €) are widespread in two investigated countries; United...

  10. Small Intestinal Infections.

    Science.gov (United States)

    Munot, Khushboo; Kotler, Donald P

    2016-06-01

    Small intestinal infections are extremely common worldwide. They may be bacterial, viral, or parasitic in etiology. Most are foodborne or waterborne, with specific etiologies differing by region and with diverse pathophysiologies. Very young, very old, and immune-deficient individuals are the most vulnerable to morbidity or mortality from small intestinal infections. There have been significant advances in diagnostic sophistication with the development and early application of molecular diagnostic assays, though these tests have not become mainstream. The lack of rapid diagnoses combined with the self-limited nature of small intestinal infections has hampered the development of specific and effective treatments other than oral rehydration. Antibiotics are not indicated in the absence of an etiologic diagnosis, and not at all in the case of some infections.

  11. Síndrome de ativação macrofágica associada com artrite idiopática juvenil sistêmica Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Clovis Artur A. Silva

    2004-12-01

    Full Text Available OBJETIVO: Descrever as características da síndrome de ativação macrofágica associada a artrite idiopática juvenil. DESCRIÇÃO DOS CASOS: Foram analisados retrospectivamente os prontuários de 462 pacientes com artrite idiopática juvenil. Destes, sete (1,5% pacientes desenvolveram síndrome de ativação macrofágica; todos tinham a forma sistêmica da doença. A mediana de idade de início da artrite idiopática juvenil foi de 3 anos e 10 meses, e a mediana do tempo de duração da artrite idiopática juvenil antes da síndrome de ativação macrofágica foi de 8 anos e 4 meses. Todos os pacientes apresentaram febre, icterícia, hepatoesplenomegalia, sangramentos, pancitopenia e elevação das enzimas hepáticas e dos tempos de coagulação e bilirrubina direta. Três casos apresentaram infecções associadas e um caso desenvolveu a síndrome de ativação macrofágica 2 semanas após a introdução de sulfasalazina. Três pacientes morreram. Proliferação macrofágica e hemofagocitose foram evidenciadas em cinco. A terapêutica da síndrome de ativação macrofágica incluiu pulsoterapia com metilprednisolona em todos, ciclosporina em três, plasmaférese em dois e gamaglobulina endovenosa em dois. COMENTÁRIOS: A síndrome de ativação macrofágica é uma complicação da artrite idiopática juvenil sistêmica com alta morbidade e mortalidade.OBJECTIVE: To describe the characteristics of macrophage activation syndrome associated with juvenile idiopathic arthritis. DESCRIPTION: This is a retrospective study involving 462 patients with juvenile idiopathic arthritis. Seven (1.5% of those patients suffered from systemic onset juvenile idiopathic arthritis and developed macrophage activation syndrome. The median age of the juvenile idiopathic arthritis onset was 3 years and 10 months and the median duration of juvenile idiopathic arthritis before macrophage activation syndrome was 8 years and 4 months. All of them presented with fever

  12. Small hydro in Africa

    CSIR Research Space (South Africa)

    Jonker Klunne, W

    2011-10-01

    Full Text Available the gold mines at Pilgrim?s Rest in South Africa were powered by two 6kW hydro turbines as early as 1892, complemented by a 45kW turbine in 1894 to power the first electrical railway. In several African countries church missions built small hydro... that feed in to the national grid as this ensures a steady income stream for the enterprise. Several African countries have established renewable energy feed-in tariffs (Kenya, South Africa and Uganda) that do support the establishment of small scale...

  13. Small finance banks: Challenges

    Directory of Open Access Journals (Sweden)

    Jayadev M

    2017-12-01

    Full Text Available A recent innovation in the Indian banking structure has been the formation of a new banking institution—small finance banks (SFBs. These banks are expected to penetrate into financial inclusion by providing basic banking and credit services with a differentiated banking model to the larger population. In this context the new SFBs have multiple challenges in coming out with a new, differentiated business model. The challenges include building low cost liability portfolio, technology management, and balancing the regulatory compliances. This paper also presents the top of mind views of three senior executives of new small finance banks.

  14. Small hydroelectric engineering practice

    CERN Document Server

    Leyland, Bryan

    2014-01-01

    Small Hydroelectric Engineering Practice is a comprehensive reference book covering all aspects of identifying, building, and operating hydroelectric schemes between 500 kW and 50 MW. In this range of outputs there are many options for all aspects of the scheme and it is very important that the best options are chosen.As small hydroelectric schemes are usually built against a limited budget it is extremely important that the concept design is optimum and every component is designed to maximise the benefi t and minimise the cost. As operating costs are often a high proportion of the income it i

  15. Small Column Ion Exchange

    International Nuclear Information System (INIS)

    Huff, Thomas

    2010-01-01

    Small Column Ion Exchange (SCIX) leverages a suite of technologies developed by DOE across the complex to achieve lifecycle savings. Technologies are applicable to multiple sites. Early testing supported multiple sites. Balance of SRS SCIX testing supports SRS deployment. A forma Systems Engineering Evaluation (SEE) was performed and selected Small Column Ion Exchange columns containing Crystalline Silicotitanate (CST) in a 2-column lead/lag configuration. SEE considered use of Spherical Resorcinol-Formaldehyde (sRF). Advantages of approach at SRS include: (1) no new buildings, (2) low volume of Cs waste in solid form compared to aqueous strip effluent; and availability of downstream processing facilities for immediate processing of spent resin.

  16. How Small are Small Stars Really?

    Science.gov (United States)

    2002-11-01

    VLT Interferometer Measures the Size of Proxima Centauri and Other Nearby Stars [1] Summary At a distance of only 4.2 light-years, Proxima Centauri is the nearest star to the Sun currently known [2]. It is visible as an 11-magnitude object in the southern constellation of Centaurus and is the faintest member of a triple system, together with Alpha Centauri , the brightest (double) star in this constellation. Proxima Centauri is a very-low-mass star, in fact barely massive enough to burn hydrogen to helium in its interior. It is about seven times smaller than the Sun, and the surface temperature is "only" about 3000 degrees, about half of that of our own star. Consequently, it is also much fainter - the intrinsic brightness is only 1/150th of that of our Sun. Low-mass stars are very interesting objects , also because the physical conditions in their interiors have much in common with those of giant planets, like Jupiter in our solar system. A determination of the sizes of the smallest stars has been impossible until now because of their general faintness and lack of adequate instrumentation. However, astronomers have long been keen to move forward in this direction, since such measurements would provide indirect, crucial information about the behaviour of matter under extreme conditions. When the first observations with the VLT Interferometer (VLTI), combining the light from two of the 8.2-m VLT Unit Telescopes (ANTU and MELIPAL), were made one year ago ( ESO PR 23/01 ), interferometric measurements were also obtained of Proxima Centauri . They formed part of the VLTI commissioning and the data were soon released to the ESO community, cf. the special website. Now, an international team of astronomers from Switzerland, France and ESO/Chile has successfully analysed these observations by means of newly developed, advanced software. For the first time ever, they obtained a highly accurate measurement of the size of such a small star . Three other small stars were also

  17. On small clusters

    International Nuclear Information System (INIS)

    Bernardes, N.

    1984-01-01

    A discussion is presented of zero-point motion effects on the binding energy of a small cluster of identical particles interacting through short range attractive-repulsive forces. The model is appropriate to a discussion of both Van der Waals as well as nuclear forces. (Author) [pt

  18. Small Public Library Management

    Science.gov (United States)

    Pearlmutter, Jane; Nelson, Paul

    2012-01-01

    Anyone at the helm of a small public library knows that every little detail counts. But juggling the responsibilities that are part and parcel of the job is far from easy. Finally, here's a handbook that includes everything administrators need to keep a handle on library operations, freeing them up to streamline and improve how the organization…

  19. Small rover exploration capabilities

    NARCIS (Netherlands)

    Salotti, J.; Laithier, C.; Machut, B.; van Marie, A.; Bruneau, A.; Groemer, G.; Foing, B.H.

    2015-01-01

    For a human mission to the Moon or Mars, an important question is to determine the best strategy for the choice of surface vehicles. Recent studies suggest that the first missions to Mars will be strongly constrained and that only small unpressurized vehicles will be available. We analyze the

  20. Small Open Reading Frames

    Indian Academy of Sciences (India)

    small ORFs and presence of their stable peptide products. Further, examples of biologically active peptides with ... enormous growth in whole genome sequencing. Hence today, genome sequence. Recently, ORFs ... Similarly, during the embryo development of zebrafish, 58 aa long toddler polypeptide produced by a short ...

  1. Small Windmills in Denmark

    DEFF Research Database (Denmark)

    Petersen, H.

    1980-01-01

    The report describes a project for small windmills funded by the Ministry of Energy. The test plant is described and a survey of Danish windmills is presented. Some requirements for windmills are mentioned and regulations governing the interface between grid-connected windmills and the electric u...

  2. Radiotherapy in small countries.

    Science.gov (United States)

    Barton, Michael B; Zubizarreta, Eduardo H; Polo Rubio, J Alfredo

    2017-10-01

    To examine the availability of radiotherapy in small countries. A small country was defined as a country with a population less than one million persons. The economic status of each country was defined using the World Bank Classification. The number of cancers in each country was obtained from GLOBOCAN 2012. The number of cancer cases with an indication or radiotherapy was calculated using the CCORE model. There were 41 countries with a population of under 1 million; 15 were classified as High Income, 15 Upper Middle Income, 10 Lower Middle Income and one Low Income. 28 countries were islands. Populations ranged from 799 (Holy See) to 886450 (Fiji) and the total number of cancer cases occurring in small countries was 21,043 (range by country from 4 to 2476). Overall the total number of radiotherapy cases in small countries was 10982 (range by country from 2 to 1239). Radiotherapy was available in all HIC islands with 80 or more new cases of cancer in 2012 but was not available in any LMIC island. Fiji was the only LMIC island with a large radiotherapy caseload. Similar caseloads in non-island LMIC all had radiotherapy services. Most non-island HIC did not have radiotherapy services presumably because of the easy access to radiotherapy in neighbouring countries. There are no radiotherapy services in any LMIC islands. Copyright © 2017. Published by Elsevier Ltd.

  3. Small bowel resection

    Science.gov (United States)

    ... Ileostomy and your diet Ileostomy - caring for your stoma Ileostomy - changing your pouch Ileostomy - discharge Ileostomy - what to ask your doctor Low-fiber diet Preventing falls Small bowel resection - discharge Surgical wound care - open Types of ileostomy Ulcerative colitis - discharge When ...

  4. Research using small tokamaks

    International Nuclear Information System (INIS)

    1993-01-01

    This document consists of a collection of papers presented at the IAEA Technical Committee Meeting on Research Using Small Tokamaks. It contains 22 papers on a wide variety of research aspects, including diagnostics, design, transport, equilibrium, stability, and confinement. Some of these papers are devoted to other concepts (stellarators, compact tori). Refs, figs and tabs

  5. Small Business Innovations

    Science.gov (United States)

    1993-01-01

    Under an Army Small Business Innovation Research (SBIR) grant, Symbiotics, Inc. developed a software system that permits users to upgrade products from standalone applications so they can communicate in a distributed computing environment. Under a subsequent NASA SBIR grant, Symbiotics added additional tools to the SOCIAL product to enable NASA to coordinate conventional systems for planning Shuttle launch support operations. Using SOCIAL, data may be shared among applications in a computer network even when the applications are written in different programming languages. The product was introduced to the commercial market in 1993 and is used to monitor and control equipment for operation support and to integrate financial networks. The SBIR program was established to increase small business participation in federal R&D activities and to transfer government research to industry. InQuisiX is a reuse library providing high performance classification, cataloging, searching, browsing, retrieval and synthesis capabilities. These form the foundation for software reuse, producing higher quality software at lower cost and in less time. Software Productivity Solutions, Inc. developed the technology under Small Business Innovation Research (SBIR) projects funded by NASA and the Army and is marketing InQuisiX in conjunction with Science Applications International Corporation (SAIC). The SBIR program was established to increase small business participation in federal R&D activities and to transfer government research to industry.

  6. Small angle neutron scattering

    International Nuclear Information System (INIS)

    Gupta, Sanjay

    1982-01-01

    The technique of small angle neutron scattering was first used in Germany less than two decades ago. Since then it has developed very rapidly, and today it is regarded as one of the most powerful techniques in materials, chemical and biological research. During the last decade the combination of high flux reactors and sophisticated instrumentation has revolutionized the technique. This paper endeavours to present a brief but comprehensive review of small angle scattering of neutrons and its applications in solid state research. The domain in which small angle neutron scattering is particularly useful is delineated and some of the methods used in the analysis of data are discussed with special emphasis on recent developments. Typical small angle neutron scattering cameras are described. Finally some experimental results on heterogeneities in metallic systems (both static and dynamic studies), radiation damage in materials, superconductivity, magnetic materials and the technologically very important area of non-destructive testing are reviewed in order to illustrate the wide range of applicability of this technique to problems in solid state research. (author)

  7. Small hydroelectric power plants

    International Nuclear Information System (INIS)

    Helgesen, Boerre

    2002-01-01

    Small hydroelectric power plants are power plants of 1 - 10 MW. For a supplier, this is an unnatural limit. A more natural limit involves compact engine design and simplified control system. The article discusses most of the engine and electrotechnical aspects in the development, construction and operation of such a plant

  8. Deburring small intersecting holes

    Energy Technology Data Exchange (ETDEWEB)

    Gillespie, L.K.

    1980-08-01

    Deburring intersecting holes is one of the most difficult deburring tasks faced by many industries. Only 14 of the 37 major deburring processes are applicable to most intersecting hole applications. Only five of these are normally applicable to small or miniature holes. Basic process capabilities and techniques used as a function of hole sizes and intersection depths are summarized.

  9. Imaging the small bowel.

    LENUS (Irish Health Repository)

    Murphy, Kevin P

    2014-03-01

    Radiologic investigations continue to play a pivotal role in the diagnosis of pathologic conditions of the small intestine despite enhancement of capsule endoscopy and double-balloon endoscopy. Imaging techniques continue to evolve and new techniques in MRI in particular, are being developed.

  10. Small hydrogen liquefier

    International Nuclear Information System (INIS)

    Airoldi, V.J.T.; Corat, E.J.; Minucci, M.A.S.; Leite, V.S.F.O.

    1986-09-01

    In this work the deign and construction of a small hydrogen liquefier (two liters per hour maximum production) is described. The isenthalpic expansion process is used, because its construction is simple and it is generally cheaper to operate. A comparison with other liquefier processes, and considerations about their basic theory are also presented. (author) [pt

  11. Small Open Reading Frames

    Indian Academy of Sciences (India)

    are conserved across diverse species, and the functional char- acterization of their peptides reveals their critical role in a broad spectrum of regulatory mechanisms. Further analy- sis of small ORFs is likely throw light on many exciting, un- explored regulatory mechanisms in different developmental stages and tissue types.

  12. Small Engine & Accessory Test Area

    Data.gov (United States)

    Federal Laboratory Consortium — The Small Engine and Accessories Test Area (SEATA) facilitates testaircraft starting and auxiliary power systems, small engines and accessories. The SEATA consists...

  13. Small steps for hydro

    International Nuclear Information System (INIS)

    Wicke, Peter

    1998-01-01

    The government in Peru has decided to utilise its gas reserves and restrict hydro to relatively small schemes. A number of reasons for the decision are given. In 1997, the Shell-Mobile-Bechtel-COSAPI consortium was formed and agreements were signed regarding exploiting Gas de Camisea. The country's energy needs to 2010 are being assessed. It is likely that by 2001 the whole of south Peru will be receiving gas from Camisea. The Peru situation is discussed under the headings of (i) existing capacity, (ii) growing demands, (iii) a history of hydro in Peru, (iv) electrification and SHP and (v) outlook. The future for Peru's electric energy development is bright. While most of its new power capacity will come from natural gas, the small hydros also have a part to play. A stronger commitment of national and regional political authorities to consider supplies outside the big cities is said to be needed. (UK)

  14. Small reactor operating mode

    International Nuclear Information System (INIS)

    Snell, V.G.

    1997-01-01

    There is a potential need for small reactors in the future for applications such as district heating, electricity production at remote sites, and desalination. Nuclear power can provide these at low cost and with insignificant pollution. The economies required by the small scale application, and/or the remote location, require a review of the size and location of the operating staff. Current concepts range all the way from reactors which are fully automatic, and need no local attention for days or weeks, to those with reduced local staff. In general the less dependent a reactor is on local human intervention, the greater its dependence on intrinsic safety features such as passive decay heat removal, low-stored energy and limited reactivity speed and depth in the control systems. A case study of the design and licensing of the SLOWPOKE Energy System heating reactor is presented. (author)

  15. Small Business Innovations (Photodetector)

    Science.gov (United States)

    1991-01-01

    Epitaxx, Inc. of Princeton, NJ, developed the Epitaxx Near Infrared Room Temperature Indium-Gallium-Arsenide (InGaAs) Photodetector based on their Goddard Space Flight Center Small Business Innovation Research (SBIR) contract work to develop a linear detector array for satellite imaging applications using InGaAs alloys that didn't need to be cooled to (difficult and expensive) cryogenic temperatures. The photodetectors can be used for remote sensing, fiber optic and laser position-sensing applications.

  16. Small Business Innovations (MISER)

    Science.gov (United States)

    1991-01-01

    Lightwave Electronics Corporation, Mountain View, CA, developed the Series 120 and 122 non-planner diode pumped ring lasers based on a low noise ring laser with voltage tuning that they delivered to Jet Propulsion Laboratory under a Small Business Innovation Research (SBIR) contract. The voltage tuning feature allows "phase-locking" the lasers, making them "electronic," similar to radio and microwave electronic oscillators. The Series 120 and 122 can be applied to fiber sensing, coherent communications and laser radar.

  17. Big Data, Small Sample.

    Science.gov (United States)

    Gerlovina, Inna; van der Laan, Mark J; Hubbard, Alan

    2017-05-20

    Multiple comparisons and small sample size, common characteristics of many types of "Big Data" including those that are produced by genomic studies, present specific challenges that affect reliability of inference. Use of multiple testing procedures necessitates calculation of very small tail probabilities of a test statistic distribution. Results based on large deviation theory provide a formal condition that is necessary to guarantee error rate control given practical sample sizes, linking the number of tests and the sample size; this condition, however, is rarely satisfied. Using methods that are based on Edgeworth expansions (relying especially on the work of Peter Hall), we explore the impact of departures of sampling distributions from typical assumptions on actual error rates. Our investigation illustrates how far the actual error rates can be from the declared nominal levels, suggesting potentially wide-spread problems with error rate control, specifically excessive false positives. This is an important factor that contributes to "reproducibility crisis". We also review some other commonly used methods (such as permutation and methods based on finite sampling inequalities) in their application to multiple testing/small sample data. We point out that Edgeworth expansions, providing higher order approximations to the sampling distribution, offer a promising direction for data analysis that could improve reliability of studies relying on large numbers of comparisons with modest sample sizes.

  18. Ecology in Small Aquatic Ecosystems

    DEFF Research Database (Denmark)

    Andersen, Mikkel René

    Small ecosystems are many-fold more abundant than their larger counterparts. Both on regional and global scale small lakes outnumber medium and large lakes and account for a much larger surface area. Small streams are also far more common than rivers. Despite their abundance small ecosystems are ...

  19. The small library manager's handbook

    CERN Document Server

    Graves, Alice

    2014-01-01

    The Small Library Manager's Handbook is for librarians working in all types of small libraries. It covers the everyday nuts-and-bolts operations that all librarians must perform. This handbook, written by experts who are small librarians themselves, will help all small librarians to do multiple jobs at the same time.

  20. The Small Mars System

    Science.gov (United States)

    Fantino, E.; Grassi, M.; Pasolini, P.; Causa, F.; Molfese, C.; Aurigemma, R.; Cimminiello, N.; de la Torre, D.; Dell'Aversana, P.; Esposito, F.; Gramiccia, L.; Paudice, F.; Punzo, F.; Roma, I.; Savino, R.; Zuppardi, G.

    2017-08-01

    The Small Mars System is a proposed mission to Mars. Funded by the European Space Agency, the project has successfully completed Phase 0. The contractor is ALI S.c.a.r.l., and the study team includes the University of Naples ;Federico II;, the Astronomical Observatory of Capodimonte and the Space Studies Institute of Catalonia. The objectives of the mission are both technological and scientific, and will be achieved by delivering a small Mars lander carrying a dust particle analyser and an aerial drone. The former shall perform in situ measurements of the size distribution and abundance of dust particles suspended in the Martian atmosphere, whereas the latter shall demonstrate low-altitude flight in the rarefied planetary environment. The mission-enabling technology is an innovative umbrella-like heat shield, known as IRENE, developed and patented by ALI. The mission is also a technological demonstration of the shield in the upper atmosphere of Mars. The core characteristics of SMS are the low cost (120 M€) and the small size (320 kg of wet mass at launch, 110 kg at landing), features which stand out with respect to previous Mars landers. To comply with them is extremely challenging at all levels, and sets strict requirements on the choice of the materials, the sizing of payloads and subsystems, their arrangement inside the spacecraft and the launcher's selection. In this contribution, the mission and system concept and design are illustrated and discussed. Special emphasis is given to the innovative features and to the challenges faced in the development of the work.

  1. Small Business Innovations (Cryostat)

    Science.gov (United States)

    1991-01-01

    General Pneumatics Corporation, Scottsdale, AZ, developed an anti- clogging cryostat that liquifies gases by expansion for high pressure through a nozzle to produce cryorefrigeration based on their Kennedy Space Center Small Business Innovation Research (SBIR) work to develop a Joule-Thomson (JT) expansion valve that is less susceptible to clogging by particles or condensed contaminants in the flow than a non-contaminating compressor in a closed cycle Linde-Hampson cryocooler used to generate cryogenic cooling for infrared sensors, super conductors, supercooled electronics and cryosurgery.

  2. Small Business Innovations (Helicopters)

    Science.gov (United States)

    1992-01-01

    The amount of engine power required for a helicopter to hover is an important, but difficult, consideration in helicopter design. The EHPIC program model produces converged, freely distorted wake geometries that generate accurate analysis of wake-induced downwash, allowing good predictions of rotor thrust and power requirements. Continuum Dynamics, Inc., the Small Business Innovation Research (SBIR) company that developed EHPIC, also produces RotorCRAFT, a program for analysis of aerodynamic loading of helicopter blades in forward flight. Both helicopter codes have been licensed to commercial manufacturers.

  3. Small radioisotope powered batteries

    International Nuclear Information System (INIS)

    Myatt, J.

    1975-06-01

    Various methods of converting the large amounts of energy stored in radioisotopes are described. These are based on:- (a) the Seebeck effect; (b) thermionic emission of electrons from a hot body; (c) the Stirling Cycle; and (d) radiovoltaic charge separation in 'p-n' junctions. Small generators in the range 0 to 100 W(e) developed using these effects are described and typical applications for each of these systems are given. These include data collection and transmission from remote sites, implantable medical devices, lighthouses, radio beacons, and space power supplies. (author)

  4. Small circuits for cryptography.

    Energy Technology Data Exchange (ETDEWEB)

    Torgerson, Mark Dolan; Draelos, Timothy John; Schroeppel, Richard Crabtree; Miller, Russell D.; Anderson, William Erik

    2005-10-01

    This report examines a number of hardware circuit design issues associated with implementing certain functions in FPGA and ASIC technologies. Here we show circuit designs for AES and SHA-1 that have an extremely small hardware footprint, yet show reasonably good performance characteristics as compared to the state of the art designs found in the literature. Our AES performance numbers are fueled by an optimized composite field S-box design for the Stratix chipset. Our SHA-1 designs use register packing and feedback functionalities of the Stratix LE, which reduce the logic element usage by as much as 72% as compared to other SHA-1 designs.

  5. Neutron small angle scattering

    International Nuclear Information System (INIS)

    Ibel, K.

    1975-01-01

    The neutron small-angle scattering system at the High-Flux Reactor in Grenoble consists of three major parts: the supply of cold neutrons via bent neutron guides; the small angle camera D11; and the data handling facilities. The camera D11 has an overall length of 80 m. The effective length of the camera is variable. The length of the collimator before the fixed sample position can be reduced by movable neutron guides; the secondary flight path of 40 m full length contains detector sites in various positions. Thus, a large domain of momentum transfers can be exploited. Scattering angles between 5.10 -4 and 0.5 rad and neutron wavelengths from 0.2 to 2.0 nm are available with the same instrument and the same relative resolution. A large-area position-sensitive detector is used which allows simultaneous recording of intensities scattered into different angles; it is a multiwire proportional chamber. 3808 elements of 1 cm 2 are arranged in a two-dimensional matrix. Future development comprises an increase of the limit in the count rate due to the electronic interface between the detector and on-line computer, actually at 5.10 4 per sec. by one order of magnitude

  6. 77 FR 30227 - Small Business Size Regulations, Small Business Innovation Research (SBIR) Program and Small...

    Science.gov (United States)

    2012-05-22

    ... Business Size Regulations, Small Business Innovation Research (SBIR) Program and Small Business Technology... eligibility for the Small Business Innovation Research (SBIR) and Small Business Technology Transfer (STTR... Investment and Innovation at [email protected] . SUPPLEMENTARY INFORMATION: I. Background Information On...

  7. Problems of Small Debris

    Directory of Open Access Journals (Sweden)

    V. V. Zelentsov

    2015-01-01

    Full Text Available During the exploration of outer space (as of 1/1 2011 6853 was launched spacecraft (SC are successful 6264, representing 95% of the total number of starts. The most intensively exploited space Russia (USSR (3701 starts, 94% successful, USA (2774 starts, 90% successful, China (234 starts, 96% successful and India (89 starts, 90% successful. A small part of running the spacecraft returned to Earth (manned spacecraft and transport, and the rest remained in orbit. Some of them are descended from orbit and burned up in the atmosphere, the rest remained in the OCP and turned into space debris (SD.The composition of the Cabinet is diverse: finish the job spacecraft; boosters and the last stage of launch vehicles left in orbit after SC injection; technological waste arising during the opening drop-down structures and fragments of the destroyed spacecraft. The resulting explosion orbital SD forms ellipsoidal region which orbits blasted object. Then, as a result of precession, is the distribution of objects in orbit explosion exploding spacecraft.The whole Cabinet is divided into two factions: the observed (larger than 100 mm and not observed (less than 100 mm. Observed debris katalogalizirovan and 0.2% of the total number of SD, there was no SD is the bulk - 99.8%.SC meeting working with a fragment observed SD predictable and due to changes in altitude spacecraft avoids a possible meeting. Contact spacecraft with large fragment lead to disaster (which took place at a meeting of the Russian communications satellite "Cosmos-2251" and the American machine "Iridium". Meeting with small SD is not predictable, especially if it was formed by an explosion or collision fragments together. Orbit that KM is not predictable, and the speed can be up to 10 km / s. Meeting with small particle SD no less dangerous for the spacecraft. The impact speed of spacecraft with space debris particles can reach up to 10 ... 15 km / s at such speeds the breakdown probability thin

  8. Small Wind Energy Systems

    DEFF Research Database (Denmark)

    Simoes, Marcelo; Farret, Felix Alberto; Blaabjerg, Frede

    2015-01-01

    devices, and a centralized distribution control. In order to establish a small wind energy system it is important to observe the following: (i) Attending the energy requirements of the actual or future consumers; (ii) Establishing civil liabilities in case of accidents and financial losses due to shortage...... or low quality of energy; (iii) Negotiating collective conditions to interconnect the microgrid with the public network or with other sources of energy that is independent of wind resources; (iv) Establishing a performance criteria of power quality and reliability to end-users, in order to reduce costs...... and guaranteeing an acceptable energy supply. This paper discuss how performance is affected by local conditions and random nature of the wind, power demand profiles, turbine related factors, and presents the technical issues for implementing a self-excited induction generator system, or a permanent magnet based...

  9. Small Business Innovations

    Science.gov (United States)

    1996-01-01

    Under a Small Business Innovation Research (SBIR) contract to Kennedy Space Center, EIC Laboratories invented a Raman Spectrograph with fiber optic sampling for space applications such as sensing hazardous fuel vapors and making on-board rapid analyses of chemicals and minerals. Raman spectroscopy is a laser-based measurement technique that provides through a unique vibrational spectrum a molecular 'fingerprint,' and can function in aqueous environments. EIC combined optical fiber technology with Raman methods to develop sensors that can be operated at a distance from the spectrographic analysis instruments and the laser excitation source. EIC refined and commercialized the technology to create the Fiber Optic Raman Spectrograph and the RamanProbe. Commercial applications range from process control to monitoring hazardous materials.

  10. [Is allastrim small pox?].

    Science.gov (United States)

    Tiexeira, L A

    2000-01-01

    Between 1910 and 1913, two renowned physicians in the city of Sao Paulo found themselves engaged in a scientific controversy regarding the classification of a disease then assailing the state. Antonio Carini, director of the Instituto Pasteur de Sao Paulo, believed the illness to be small pox, while Emilio Ribas, director of the Servico Sanitario, claimed it was allastrim, or milk pox. The controversy started in the Sociedade de Medicina e Cirurgia but later migrated to other forums and came to incorporate other figures as well. This presentation and discussion of the polemic is meant as a contribution to our understanding of the process by which a scientific consensus is constructed and solidified within the field of the biomedical sciences.

  11. Small angle neutron scattering

    International Nuclear Information System (INIS)

    Dasannacharya, B.A.; Goyal, P.S.

    1997-01-01

    Small angle neutron scattering (SANS) is one of the most popular neutron scattering technique both for the basic research and as a tool in the hands of applied scientist. SANS is used for studying the structure of a material on a length scale of 10 - 1000 A. SANS is a diffraction experiment that involves scattering of a monocromatic beam of neutrons in order to obtain structural information about macromolecules and heterogeneities. This paper will discuss the design of SANS spectrometers with a special emphasis on the instruments which are better suited for medium flux reactors. The design of several different types of SANS spectrometers will be given. The optimization procedures and appropriate modifications to suit the budget and the space will be discussed. As an example, the design of a SANS spectrometer at CIRUS reactor Trombay will be given. (author)

  12. Small dose... big poison.

    Science.gov (United States)

    Braitberg, George; Oakley, Ed

    2010-11-01

    It is not possible to identify all toxic substances in a single journal article. However, there are some exposures that in small doses are potentially fatal. Many of these exposures are particularly toxic to children. Using data from poison control centres, it is possible to recognise this group of exposures. This article provides information to assist the general practitioner to identify potential toxic substance exposures in children. In this article the authors report the signs and symptoms of toxic exposures and identify the time of onset. Where clear recommendations on the period of observation and known fatal dose are available, these are provided. We do not discuss management or disposition, and advise readers to contact the Poison Information Service or a toxicologist for this advice.

  13. Small intestinal transplantation.

    LENUS (Irish Health Repository)

    Quigley, E M

    2012-02-03

    The past few years have witnessed a considerable shift in the clinical status of intestinal transplantation. A great deal of experience has been gained at the most active centers, and results comparable with those reported at a similar stage in the development of other solid-organ graft programs are now being achieved by these highly proficient transplant teams. Rejection and its inevitable associate, sepsis, remain ubiquitous, and new immunosuppressant regimes are urgently needed; some may already be on the near horizon. The recent success of isolated intestinal grafts, together with the mortality and morbidity attendant upon the development of advanced liver disease related to total parenteral nutrition, has prompted the bold proposal that patients at risk for this complication should be identified and should receive isolated small bowel grafts before the onset of end-stage hepatic failure. The very fact that such a suggestion has begun to emerge reflects real progress in this challenging field.

  14. Small caliber guided projectile

    Science.gov (United States)

    Jones, James F [Albuquerque, NM; Kast, Brian A [Albuquerque, NM; Kniskern, Marc W [Albuquerque, NM; Rose, Scott E [Albuquerque, NM; Rohrer, Brandon R [Albuquerque, NM; Woods, James W [Albuquerque, NM; Greene, Ronald W [Albuquerque, NM

    2010-08-24

    A non-spinning projectile that is self-guided to a laser designated target and is configured to be fired from a small caliber smooth bore gun barrel has an optical sensor mounted in the nose of the projectile, a counterbalancing mass portion near the fore end of the projectile and a hollow tapered body mounted aft of the counterbalancing mass. Stabilizing strakes are mounted to and extend outward from the tapered body with control fins located at the aft end of the strakes. Guidance and control electronics and electromagnetic actuators for operating the control fins are located within the tapered body section. Output from the optical sensor is processed by the guidance and control electronics to produce command signals for the electromagnetic actuators. A guidance control algorithm incorporating non-proportional, "bang-bang" control is used to steer the projectile to the target.

  15. Small rover exploration capabilities

    Science.gov (United States)

    Salotti, Jean-Marc; Laithier, Corentin; Machut, Benoit; Marie, Aurélien; Bruneau, Audrey; Grömer, Gernot; Foing, Bernard H.

    2015-05-01

    For a human mission to the Moon or Mars, an important question is to determine the best strategy for the choice of surface vehicles. Recent studies suggest that the first missions to Mars will be strongly constrained and that only small unpressurized vehicles will be available. We analyze the exploration capabilities and limitations of small surface vehicles from the user perspective. Following the “human centered design” paradigm, the team focused on human systems interactions and conducted the following experiments: - Another member of our team participated in the ILEWG EuroMoonMars 2013 simulation at the Mars Desert Research Station in Utah during the same period of time. Although the possible traverses were restricted, a similar study with analog space suits and quads has been carried out. - Other experiments have been conducted in an old rock quarry close to Bordeaux, France. An expert in the use of quads for all types of terrains performed a demonstration and helped us to characterize the difficulties, the risks and advantages and drawbacks of different vehicles and tools. The vehicles that will be used on the surface of Mars have not been defined yet. Nevertheless, the results of our project already show that using a light and unpressurized vehicle (in the order of 150 kg) for the mobility on the Martian surface can be a true advantage. Part of the study was dedicated to the search for appropriate tools that could be used to make the vehicles easier to handle, safer to use and more efficient in the field to cross an obstacle. The final recommendation is to use winches and ramps, which already are widely used by quad drivers. We report on the extension of the reachable areas if such tools were available. This work has been supported by ILEWG, EuroMoonMars and the Austrian Space Forum (OEWF).

  16. Small Area Fair Market Rent

    Data.gov (United States)

    Department of Housing and Urban Development — Due to the increasing demand for more localized rents for a variety of purposes, HUD is making Small Area FMRs for all metropolitan areas available. Small Area FMRs...

  17. Brighter for small power plants

    International Nuclear Information System (INIS)

    Haaland, Leif

    2003-01-01

    The article presents a small tunnel drilling machine aimed at using for the construction of small hydroelectric power plants and mentions briefly some advantages economically and environmentally of both the machine and the power production solution

  18. Small intestine aspirate and culture

    Science.gov (United States)

    ... ency/article/003731.htm Small intestine aspirate and culture To use the sharing features on this page, please enable JavaScript. Small intestine aspirate and culture is a lab test to check for infection ...

  19. Globalization, Entrepreneurship and Small Business

    OpenAIRE

    Stan ªtefania Anca

    2012-01-01

    New product innovation often comes from small business. Most the radical inventions of the last century, such as the computer, come from small companies, not large ones. And this will not change. Globalization will continue to modify and mold the environment or small business and entrepreneurship. The competition from abroad places strong pressure on small business in terms of efficiency, price, quality, cost, control, marketing, expertise, customer satisfaction and innovativeness. Globalizat...

  20. Small-cell osteosarcoma

    International Nuclear Information System (INIS)

    Edeiken, J.; Raymond, A.K.; Ayala, A.G.; Benjamin, R.S.; Murray, J.A.; Carrasco, H.C.

    1987-01-01

    Small-cell osteosarcoma, a subtype of osteogenic sarcoma, consists of sheets of round cells that produce an osteoid matrix. It may be confused with Ewing sarcoma if the osteoid matrix is not included in the biopsy. The distinctive radiographic features of an osteoblastic tumor and a pattern of permeative destruction will confirm the histologic diagnosis or indicate the true nature if tumor osteoid is not included in the histological sections. We add 13 patients to the 32 previously reported in the literature. Fourteen (31%) of the 45 are living and well, though three have been followed for only 2 months. The treatments have been so varied that a statistically significant evaluation cannot be developed. The radiographic features are not distinctive, but the diagnosis may be suggested when a tumor has osteoblastic features in the metaphysis and extends well down into the shaft with a pattern of permeative destruction. The radiographic features are especially important when limited biopsies reveal only sheets of round cells, thus suggesting Ewing sarcoma. The presence of an osteoid-producing tumor as evident by osteoblastic new bone formation will lead to the correct diagnosis. (orig.)

  1. Research using small tokamaks

    International Nuclear Information System (INIS)

    1991-01-01

    The technical reports contained in this collection of papers on research using small tokamaks fall into four main categories, i.e., (i) experimental work (heating, stability, plasma radial profiles, fluctuations and transport, confinement, ultra-low-q tokamaks, wall physics, a.o.), (ii) diagnostics (beam probes, laser scattering, X-ray tomography, laser interferometry, electron-cyclotron absorption and emission systems), (iii) theory (strong turbulence, effects of heating on stability, plasma beta limits, wave absorption, macrostability, low-q tokamak configurations and bootstrap currents, turbulent heating, stability of vortex flows, nonlinear islands growth, plasma-drift-induced anomalous transport, ergodic divertor design, a.o.), and (iv) new technical facilities (varistors applied to establish constant current and loop voltage in HT-6M), lower-hybrid-current-drive systems for HT-6B and HT-6M, radio-frequency systems for HT-6M ICR heating experimentation, and applications of fiber optics for visible and vacuum ultraviolet radiation detection as applied to tokamaks and reversed-field pinches. A total number of 51 papers are included in the collection. Refs, figs and tabs

  2. 48 CFR 970.1907 - Subcontracting with Small Business, Small Disadvantaged Business and Woman-Owned Small Business...

    Science.gov (United States)

    2010-10-01

    ... Business, Small Disadvantaged Business and Woman-Owned Small Business Concerns. 970.1907 Section 970.1907... MANAGEMENT AND OPERATING CONTRACTS Small, Small Disadvantaged and Women-Owned Small Business Concerns 970.1907 Subcontracting with Small Business, Small Disadvantaged Business and Woman-Owned Small Business...

  3. Joint research using small tokamaks

    International Nuclear Information System (INIS)

    Gryaznevich, M.P.; Del Bosco, E.; Malaquias, A.; Mank, G.; Oost, G. van

    2005-01-01

    Small tokamaks have an important role in fusion research. More than 40 small tokamaks are operational. Research on small tokamaks has created a scientific basis for the scaling-up to larger tokamaks. Well-known scientific and engineering schools, which are now determining the main directions of fusion science and technology, have been established through research on small tokamaks. Combined efforts within a network of small and medium size tokamaks will further enhance the contribution of small tokamaks. A new concept of interactive co-ordinated research using small tokamaks in the mainstream fusion science areas, in testing of new diagnostics, materials and technologies as well as in education, training and broadening of the geography of fusion research in the scope of the IAEA Co-ordinated Research Project is presented. (author)

  4. Small kernel2

    Science.gov (United States)

    Yang, Yan-Zhuo; Ding, Shuo; Wang, Yong; Li, Cui-Ling; Shen, Yun; Meeley, Robert; McCarty, Donald R; Tan, Bao-Cai

    2017-06-01

    Vitamin B 6 , an essential cofactor for a range of biochemical reactions and a potent antioxidant, plays important roles in plant growth, development, and stress tolerance. Vitamin B 6 deficiency causes embryo lethality in Arabidopsis ( Arabidopsis thaliana ), but the specific role of vitamin B 6 biosynthesis in endosperm development has not been fully addressed, especially in monocot crops, where endosperm constitutes the major portion of the grain. Through molecular characterization of a small kernel2 ( smk2 ) mutant in maize, we reveal that vitamin B 6 has differential effects on embryogenesis and endosperm development in maize. The B 6 vitamer pyridoxal 5'-phosphate (PLP) is drastically reduced in both the smk2 embryo and the endosperm. However, whereas embryogenesis of the smk2 mutant is arrested at the transition stage, endosperm formation is nearly normal. Cloning reveals that Smk2 encodes the glutaminase subunit of the PLP synthase complex involved in vitamin B 6 biosynthesis de novo. Smk2 partially complements the Arabidopsis vitamin B 6 -deficient mutant pdx2.1 and Saccharomyces cerevisiae pyridoxine auxotrophic mutant MML21. Smk2 is constitutively expressed in the maize plant, including developing embryos. Analysis of B 6 vitamers indicates that the endosperm accumulates a large amount of pyridoxamine 5'-phosphate (PMP). These results indicate that vitamin B 6 is essential to embryogenesis but has a reduced role in endosperm development in maize. The vitamin B 6 required for seed development is synthesized in the seed, and the endosperm accumulates PMP probably as a storage form of vitamin B 6 . © 2017 American Society of Plant Biologists. All Rights Reserved.

  5. Small angle neutron scattering

    Directory of Open Access Journals (Sweden)

    Cousin Fabrice

    2015-01-01

    Full Text Available Small Angle Neutron Scattering (SANS is a technique that enables to probe the 3-D structure of materials on a typical size range lying from ∼ 1 nm up to ∼ a few 100 nm, the obtained information being statistically averaged on a sample whose volume is ∼ 1 cm3. This very rich technique enables to make a full structural characterization of a given object of nanometric dimensions (radius of gyration, shape, volume or mass, fractal dimension, specific area… through the determination of the form factor as well as the determination of the way objects are organized within in a continuous media, and therefore to describe interactions between them, through the determination of the structure factor. The specific properties of neutrons (possibility of tuning the scattering intensity by using the isotopic substitution, sensitivity to magnetism, negligible absorption, low energy of the incident neutrons make it particularly interesting in the fields of soft matter, biophysics, magnetic materials and metallurgy. In particular, the contrast variation methods allow to extract some informations that cannot be obtained by any other experimental techniques. This course is divided in two parts. The first one is devoted to the description of the principle of SANS: basics (formalism, coherent scattering/incoherent scattering, notion of elementary scatterer, form factor analysis (I(q→0, Guinier regime, intermediate regime, Porod regime, polydisperse system, structure factor analysis (2nd Virial coefficient, integral equations, characterization of aggregates, and contrast variation methods (how to create contrast in an homogeneous system, matching in ternary systems, extrapolation to zero concentration, Zero Averaged Contrast. It is illustrated by some representative examples. The second one describes the experimental aspects of SANS to guide user in its future experiments: description of SANS spectrometer, resolution of the spectrometer, optimization of

  6. Small 'l' leadership.

    Science.gov (United States)

    Parsons, Jenni

    2009-05-01

    Recently I attended the RACGP Leadership Masterclass in Sydney. When I enrolled, I thought, 'Yes... sounds interesting...good speakers... I need to learn something about leadership...'As the time drew closer I started to get a bit anxious about the whole thing. I realised that the title, 'Masterclass', probably implied that the attendees were expected to already know something about leadership and its theories, if not have considerable expertise and experience in leadership. I also wondered how the workshop sessions were going to go and I started to feel a bit sorry for the facilitators. Imagine trying to facilitate a group of 10 aspiring leaders... a bit like trying to herd cats. A few days later I received a call from the organisers,saying they were a bit short of facilitators and could I help out if necessary. Great... better do a crash course in cat herding! Then there was the first 'predisposing activity'. Step 1: think of leaders you admire. Easy enough. Leaders of social justice and social change on a world stage, people who have shown great courage of their convictions and great orators popped into my head... Ghandi, Martin Luther King, Mandela, JFK. Step 2:describe the ways in which you are like these leaders. Whoa!Never going to measure up here. I wondered if there was going to be sessions on 'leadership for introverts', or 'leadership of small things', or 'leaders without grand vision or fabulous oratory skills', or perhaps 'leadership for people who are deeply suspicious of the corrupting influence of power'.

  7. Small Mercury Relativity Orbiter

    Science.gov (United States)

    Bender, Peter L.; Vincent, Mark A.

    1989-01-01

    The accuracy of solar system tests of gravitational theory could be very much improved by range and Doppler measurements to a Small Mercury Relativity Orbiter. A nearly circular orbit at roughly 2400 km altitude is assumed in order to minimize problems with orbit determination and thermal radiation from the surface. The spacecraft is spin-stabilized and has a 30 cm diameter de-spun antenna. With K-band and X-band ranging systems using a 50 MHz offset sidetone at K-band, a range accuracy of 3 cm appears to be realistically achievable. The estimated spacecraft mass is 50 kg. A consider-covariance analysis was performed to determine how well the Earth-Mercury distance as a function of time could be determined with such a Relativity Orbiter. The minimum data set is assumed to be 40 independent 8-hour arcs of tracking data at selected times during a two year period. The gravity field of Mercury up through degree and order 10 is solved for, along with the initial conditions for each arc and the Earth-Mercury distance at the center of each arc. The considered parameters include the gravity field parameters of degree 11 and 12 plus the tracking station coordinates, the tropospheric delay, and two parameters in a crude radiation pressure model. The conclusion is that the Earth-Mercury distance can be determined to 6 cm accuracy or better. From a modified worst-case analysis, this would lead to roughly 2 orders of magnitude improvement in the knowledge of the precession of perihelion, the relativistic time delay, and the possible change in the gravitational constant with time.

  8. Small diameter carbon nanopipettes

    Science.gov (United States)

    Singhal, Riju; Bhattacharyya, Sayan; Orynbayeva, Zulfiya; Vitol, Elina; Friedman, Gary; Gogotsi, Yury

    2010-01-01

    Nanoscale multifunctional carbon probes facilitate cellular studies due to their small size, which makes it possible to interrogate organelles within living cells in a minimally invasive fashion. However, connecting nanotubes to macroscopic devices and constructing an integrated system for the purpose of fluid and electrical signal transfer is challenging, as is often the case with nanoscale components. We describe a non-catalytic chemical vapor deposition based method for batch fabrication of integrated multifunctional carbon nanopipettes (CNPs) with tip diameters much smaller (10-30 nm) than previously reported (200 nm and above) and approaching those observed for multiwalled carbon nanotubes. This eliminates the need for complicated attachment/assembly of nanotubes into nanofluidic devices. Variable tip geometries and structures were obtained by controlled deposition of carbon inside and outside quartz pipettes. We have shown that the capillary length and gas flow rate have a marked effect on the carbon deposition. This gives us a flexible protocol, useful for growing carbon layers of different thicknesses at selective locations on a glass pipette to yield a large variety of cellular probes in bulk quantities. The CNPs possess an open channel for fluid transfer with the carbon deposited inside at 875 °C behaving like an amorphous semiconductor. Vacuum annealing of the CNP tips at temperatures up to 2000 °C yields graphitic carbon structures with an increase in conductivity of two orders of magnitude. Penetration of the integrated carbon nanoprobes into cells was shown to produce minimal Ca2+ signals, fast recovery of basal Ca2+ levels and no adverse activation of the cellular metabolism during interrogation times as long as 0.5-1 h.

  9. 77 FR 28520 - Small Business Size Regulations, Small Business Innovation Research (SBIR) Program and Small...

    Science.gov (United States)

    2012-05-15

    ... Business Innovation Research (SBIR) Program and Small Business Technology Transfer (STTR) Program AGENCY... Innovation Research (SBIR) and Small Business Technology Transfer (STTR) Programs. This proposed rule would...), which established the Small Business Innovation Research (SBIR) Program. The statutory purpose of the...

  10. Small Business Innovation Research and Small Business Technology Transfer Programs

    Science.gov (United States)

    Garrison, Lynn; Jasper, Gwen

    2015-01-01

    The Small Business Innovation Research (SBIR)/Small Business Technology Transfer (STTR) programs fund the research, development, and demonstration of innovative technologies that fulfill NASA's needs as described in the annual Solicitations and have significant potential for successful commercialization. The only eligible participants are small business concern (SBC) with 500 or fewer employees or a nonprofit research institute such as a university or a research laboratory with ties to an SBC. These programs are potential sources of seed funding for the development of small business innovations.

  11. Research using small tokamaks

    International Nuclear Information System (INIS)

    1991-05-01

    The technical reports in this document were presented at the IAEA Technical Committee Meeting ''Research on Small Tokamaks'', September 1990, in three sessions, viz., (1) Plasma Modes, Control, and Internal Phenomena, (2) Edge Phenomena, and (3) Advanced Configurations and New Facilities. In Section (1) experiments at controlling low mode number modes, feedback control using external coils, lower-hybrid current drive for the stabilization of sawtooth activity and continuous (1,1) mode, and unmodulated and fast modulated ECRH mode stabilization experiments were reported, as well as the relation to disruptions and transport of low m,n modes and magnetic island growth; static magnetic perturbations by helical windings causing mode locking and sawtooth suppression; island widths and frequency of the m=2 tearing mode; ultra-fast cooling due to pellet injection; and, finally, some papers on advanced diagnostics, i.e., lithium-beam activated charge-exchange spectroscopy, and detection through laser scattering of discrete Alfven waves. In Section (2), experimental edge physics results from a number of machines were presented (positive biasing on HYBTOK II enhancing the radial electric field and improving confinement; lower hybrid current drive on CASTOR improving global particle confinement, good current drive efficiency in HT-6B showing stabilization of sawteeth and Mirnov oscillations), as well as diagnostic developments (multi-chord time resolved soft and ultra-soft X-ray plasma radiation detection on MT-1; measurements on electron capture cross sections in multi-charged ion-atom collisions; development of a diagnostic neutral beam on Phaedrus-T). Theoretical papers discussed the influence of sheared flow and/or active feedback on edge microstability, large edge electric fields, and two-fluid modelling of non-ambipolar scrape-off layers. Section (3) contained (i) a proposal to construct a spherical tokamak ''Proto-Eta'', (ii) an analysis of ultra-low-q and runaway

  12. Small Wind Site Assessment Guidelines

    Energy Technology Data Exchange (ETDEWEB)

    Olsen, Tim [Advanced Energy Systems LLC, Eugene, OR (United States); Preus, Robert [National Renewable Energy Lab. (NREL), Golden, CO (United States)

    2015-09-01

    Site assessment for small wind energy systems is one of the key factors in the successful installation, operation, and performance of a small wind turbine. A proper site assessment is a difficult process that includes wind resource assessment and the evaluation of site characteristics. These guidelines address many of the relevant parts of a site assessment with an emphasis on wind resource assessment, using methods other than on-site data collection and creating a small wind site assessment report.

  13. Board Effectiveness in Small Firms

    OpenAIRE

    Söderqvist, Anette; Wägar, Karolina

    2010-01-01

    Purpose: This study investigates boards of directors in small firms and explores the link between board effectiveness and the composition, roles and working styles of the boards. Design/methodology/approach: The study analyses data from a telephone survey of boards in 45 small firms. The survey included both the CEO and the chairperson of the board. Findings: The study identifies three groups of small firms: ‘paperboards’, ‘professional boards’, and ‘management lead’ boards. Results show that...

  14. Patient Safety Outcomes in Small Urban and Small Rural Hospitals

    Science.gov (United States)

    Vartak, Smruti; Ward, Marcia M.; Vaughn, Thomas E.

    2010-01-01

    Purpose: To assess patient safety outcomes in small urban and small rural hospitals and to examine the relationship of hospital and patient factors to patient safety outcomes. Methods: The Nationwide Inpatient Sample and American Hospital Association annual survey data were used for analyses. To increase comparability, the study sample was…

  15. Financial management for small companies

    Science.gov (United States)

    Bruce Hansen; Jeff Palmer; Jeff Palmer

    2000-01-01

    The wood-products industry is characterized by many small manufacturers that lack the staff to compile and analyze information on their operations and investments. Two computer programs, FRAN and JEFFI, have been developed by the USDA Forest Service at Princeton, West Virginia,to help small companies better analyze and monitor current performance, and better evaluate...

  16. Stages of Small Intestine Cancer

    Science.gov (United States)

    ... all of an organ that contains cancer. The resection may include the small intestine and nearby organs (if the cancer has spread). The doctor may remove the section of the small intestine that contains cancer and perform an anastomosis (joining the cut ends of the intestine together). ...

  17. Small millets Policy Briefing ggg

    International Development Research Centre (IDRC) Digital Library (Canada)

    dietary calories, small millets provide vital micronutrients like vitamin B, calcium, iron, folic acid and sulfur, as well as dietary fiber, making them a valuable tool in the fight against malnutrition. Why the decline in small millets? A number of factors are responsible for a steep decline in the production and consumption of millets.

  18. [Pain therapy in small pets].

    Science.gov (United States)

    Tacke, Sabine; Gollwitzer, Andrea; Grammel, Lukas; Henke, Julia

    2017-02-09

    Although many advances in pain therapy have been made in recent years, pain therapy is more difficult in the small domestic animal than in cats and dogs. However, there is the ethical obligation that these animals also receive adequate pain therapy. An analgesic is rarely authorized for use in small pets, with pharmacological investigations often lacking and dosages frequently only determined empirically. The small size of the animals often requires a higher dose per kilogram bodyweight compared to cats and dogs. The dosage itself is also difficult to apply in small animals, because many analgesics must be diluted before their use. In addition, frequent manipulation of small animals for analgesic administration induces stress in the patient, which can intensify the pain. In the present article, those analgesics suitable for use in the small domestic animal are described and the indications for the use of the various types of analgesics are explained. A specialized section concentrates on pain detection and algesimetry in the small domestic animal. The detection of pain is much more difficult in small domestic animals. In the last few years so-called "grimace scales" have been developed which are used to assess the facial expression of the animals.

  19. Introduction: innovation and small business

    NARCIS (Netherlands)

    A.R. Thurik (Roy)

    1996-01-01

    textabstractThis paper introduces the special issue of Small Business Economics on Innovation. What binds the papers together is either their focus on the effect of firm size on the causes and consequences of innovation or their focus on the role small firms play in reshaping the industrial

  20. Improving the Small School Image.

    Science.gov (United States)

    Wolfe, Richard O.

    1982-01-01

    Now is the time for an image change in small school education. Based on the 1981 Coleman Report, small school education overflows with advantages: low student-teacher ratio, opportunity to view each student in a holistic way, and the ability to control the behavior of the student body as a whole. (LC)

  1. Resourcing Change in Small Schools

    Science.gov (United States)

    Anderson, Michelle; White, Simone

    2011-01-01

    The theme of this article is the challenge that school leaders face in creating the conditions for learning in small schools. We draw on the concepts of "social capital" and "social entrepreneurship" to identify tensions and possibilities for school leaders in a case study of a small rural school as they seek to find resources…

  2. The perception of small crime

    NARCIS (Netherlands)

    Douhou, S.; Magnus, J.R.; van Soest, A.H.O.

    2011-01-01

    In this paper we measure perceptions of incorrect behavior or ‘small crime’, based on a questionnaire administered to a large representative sample from the Dutch population. In the questionnaire we ask the respondents to rate the severity or justifiability of a number of small crimes. We present

  3. The Perception of Small Crime

    NARCIS (Netherlands)

    Douhou, S.; Magnus, J.R.; van Soest, A.H.O.

    2010-01-01

    Violations of social norms can be costly to society and they are, in the case of large crimes, followed by prosecution. Minor misbehaviors — small crimes — do not usually result in legal proceedings. Although the economic consequences of a single small crime can be low, such crimes generate

  4. Benthic invertebrate fauna, small streams

    Science.gov (United States)

    J. Bruce Wallace; S.L. Eggert

    2009-01-01

    Small streams (first- through third-order streams) make up >98% of the total number of stream segments and >86% of stream length in many drainage networks. Small streams occur over a wide array of climates, geology, and biomes, which influence temperature, hydrologic regimes, water chemistry, light, substrate, stream permanence, a basin's terrestrial plant...

  5. Optimising India's small hydro resources

    International Nuclear Information System (INIS)

    Kumar, A.

    1995-01-01

    A brief history is given of an initiation to develop small scale hydropower projects in the Himalayas. The experience of the Indian project managers in utilising international funds from the Global Environment Facility could serve as a model for other small remote communities in the rest of the world. Lessons learned are reported. (UK)

  6. Managing Small-scale Fisheries

    International Development Research Centre (IDRC) Digital Library (Canada)

    In addition, small-scale fisheries are often based in small coastal communities that depend on local resources that can be affected, positively or negatively, by surrounding economic activities. ...... Image Provide a means of communicating the intentions and needs of fisheries to new stakeholders; for example, donors.

  7. Small dams need better management

    Science.gov (United States)

    Balcerak, Ernie

    2012-03-01

    Many small dams around the world are poorly maintained and represent a safety hazard, according to Pisaniello et al. Better oversight of small dams is needed, the authors argue. The researchers reviewed literature, conducted case studies in four states in Australia, and developed policy benchmarks and best practices for small-dam management. Small dams, often just several meters high and typically privately owned by individual farmers, have historically caused major damage when they fail. For instance, in China in 1975, 230,000 people died when two large dams failed because of the cumulative failure of 60 smaller upstream dams. In the United States, in 1977 the 8-meter-high Kelly Barnes Lake dam failed, killing 39 people. Many other small-dam failures around the world have resulted in casualties and severe ecological and economic damage.

  8. Small bowel faeces sign in patients without small bowel obstruction

    International Nuclear Information System (INIS)

    Jacobs, S.L.; Rozenblit, A.; Ricci, Z.; Roberts, J.; Milikow, D.; Chernyak, V.; Wolf, E.

    2007-01-01

    Aim: To evaluate frequency and clinical relevance of the 'small bowel faeces' sign (SBFS) on computed tomography (CT) in patients with and without small bowel obstruction (SBO) presenting with acute abdominal or acute abdominal and flank pain. Methods: Abdominal CTs of consecutive patients presenting to the emergency department with abdominal or flank pain over a 6 month period were retrospectively reviewed by six radiologists, independently, for the presence of the SBFS. Examinations with positive SBFS were further evaluated in consensus by three radiologists, blinded to the final diagnosis. The small bowel was graded as non-dilated ( 4 cm) dilated. The location of SBFS and presence of distal small bowel collapse indicative of SBO was recorded. Imaging findings were subsequently correlated with the final diagnosis via chart review and compared between patients with and without SBO. Results: Of 1642 CT examinations, a positive SBFS was found in 100 (6%) studies. Of 100 patients with a positive SBFS, 32 (32%) had documented SBO. The remaining 68 patients had other non-obstructive diagnoses. SBFS was located in proximal, central, distal and multisegmental bowel loops in one (3.1%), eight (25.0%), 21 (65.6%) and two (6.3%) patients with SBO, and in zero (0%), 10 (14.7%), 53 (77.9%) and five (7.4%) of patients without SBO (p < 0.273). The small bowel was non-dilated and mildly, moderately or severely dilated in one (3%), five (16%), 20 (62%) and six (19%) patients with SBO, and in 61(90%), seven (10%), zero (0%) and zero (0%) patients without SBO. Normal or mildly dilated small bowel was seen in all (100%) patients without SBO, but only in six (19%) of 32 patients with SBO (p < 0.0001). Moderate or severe small bowel dilatation was seen in 26 (81%) patients with SBO (p < 0.0001), but it was absent in patients without SBO. Distal small bowel collapse was found in 27 (84.4%) of 32 patients with SBO, but not in patients without SBO (p < 0.0001). A combination of SBFS

  9. Editorial : entrepreneurship and small business development in small islands

    OpenAIRE

    Baldacchino, Godfrey; Fairbairn, Te’o I. J.;

    2006-01-01

    This paper reviews the limited literature on successful small business and entrepreneurship in small islands, with a focus on Pacific and European research. It argues that the notable specific contribution of this collection is its focus on tangible examples of successful island entrepreneurship, and the specific challenges towards entrepreneurship faced by island people. This approach is micro-oriented and very close to the actual human entrepreneurs that lead and shine by exampl...

  10. Morocco - Small-Scale Fisheries

    Data.gov (United States)

    Millennium Challenge Corporation — The final performance evaluation roadmap for the Small-Scale Fisheries Project (PPA-MCC) is developed using a grid constructed around indicators relating to Project...

  11. Small and Rural Wastewater Systems

    Science.gov (United States)

    Many tools, training, technical assistance, and funding resources are available to develop and maintain reliable and affordable wastewater treatment systems in small and rural communities including in tribal and U.S.-Mexico Border area.

  12. JPL Small Body Database Browser

    Data.gov (United States)

    National Aeronautics and Space Administration — The JPL Small-Body Database Browser provides data for all known asteroids and many comets. Newly discovered objects and their orbits are added on a daily basis....

  13. Business Opportunities for Small Reactors

    International Nuclear Information System (INIS)

    Minato, Akio; Nishimura, Satoshi; Brown, Neil W.

    2007-01-01

    This report assesses the market potential and identifies a number of potential paths for developing the small nuclear reactor business. There are several potential opportunities identified and evaluated. Selecting a specific approach for the business development requires additional information related to a specific market and sources of capital to support the investment. If and how a market for small nuclear plants may develop is difficult to predict because of the complexity of the economic and institutional factors that will influence such development. Key factors are; economics, safety, proliferation resistance and investment risk. The economic and political interest of any of the identified markets is also dependent on successful demonstration of the safety and reliability of small nuclear reactor. Obtaining a US-NRC Standard Design approval would be an important development step toward establishing a market for small reactors. (authors)

  14. Small Airplane Certification Compliance Program

    Science.gov (United States)

    1997-01-02

    This advisory circular (AC) provides a compilation of historically acceptable means of compliance to specifically selected sections of Part 23 of the Federal Aviation Regulations that have become burdensome for small low performance airplanes to show...

  15. Small scale mining in Britain

    Energy Technology Data Exchange (ETDEWEB)

    Down, C.G.

    1985-01-01

    The resurgence of interest in small-scale mines and the reasons for their existence such as low capital costs, legal requirements to prevent competition, market forces and topological difficulties are discussed. In Great Britain there are large numbers of small-scale licenced coal mines, limestone quarries, slate and clay works, although national production comes from a few large-scale mines. The merits of small-scale mining are reviewed - resource utilisation, social and economic benefits and high productivity - and contrasted with possible detrimental factors - lower safety standards, worse environmental effects. Comparisons are drawn between small British licensed coal mines and Chinese local mines (not run by a Ministry) and it is concluded that the advantages enjoyed by the latter could apply to British mines. 7 references.

  16. Development project of small accelerator

    CERN Document Server

    Yamada, S

    2002-01-01

    The object of this project is demonstration of a small proton and heavy ion synchrotron and a small hard X-ray photon radiation source by using new technology and application of them to therapy, diagnosis, material science and life science. In this paper, a part of small proton and heavy ion synchrotron is discussed. Nine organizations joined in this project. There are four development themes such as optimization of laser-ion 100 TW class source target, beam storage and cooling device, small synchrotron ring and FFAG accelerator. Outline and contents of development of them are explained. This project is planning to generate a few MeV/u carbon ions in fully ionized states by impact of laser with about 100 TW output. 3 T maximum bending magnetic field using normal conduction AC magnet will be actualized for synchrotron with 200 MeV proton beam. (S.Y.)

  17. Entrepreneurship and small business sustainability

    African Journals Online (AJOL)

    ; human development strategies aimed at higher productivity and lower labour costs; and a strong focus on customer service. A strong emphasis was also reported on the realignment of the small business with its changing customer profile and.

  18. Small Sat Analysis Laboratory Project

    Data.gov (United States)

    National Aeronautics and Space Administration — The NASA Low-Cost Small Spacecraft Program is focused on the technologies, subsystems, methodologies, and mission concepts for space missions which lower the...

  19. Small satellites and their regulation

    CERN Document Server

    Jakhu, Ram S

    2014-01-01

    Since the launch of UoSat-1 of the University of Surrey (United Kingdom) in 1981, small satellites proved regularly to be useful, beneficial, and cost-effective tools. Typical tasks cover education and workforce development, technology demonstration, verification and validation, scientific and engineering research as well as commercial applications. Today the launch masses range over almost three orders of magnitude starting at less than a kilogram up to a few hundred kilograms, with budgets of less than US$ 100.00 and up to millions within very short timeframes of sometimes less than two years. Therefore each category of small satellites provides specific challenges in design, development and operations. Small satellites offer great potentials to gain responsive, low-cost access to space within a short timeframe for institutions, companies, regions and countries beyond the traditional big players in the space arena. For these reasons (particularly the low cost of construction, launch and operation), small (m...

  20. Lipo sarcoma in small intestine

    International Nuclear Information System (INIS)

    Rodriguez Iglesias, J.; Pineyro Gutierrez, A.; Taroco Medeiros, L.; Fein Kolodny, C.; Navarrete Pedocchi, H.

    1987-01-01

    A case is presented by primitive liposarcoma in small intestine , an extensive bibliographical review foreigner and national in this case. It detach the exceptional of the intestinal topography of the liposarcomas; and making stress in the relative value of the computerized tomography and ultrasonography in the diagnose of the small intestine tumors . As well as in the sarcomas of another topography, chemo and radiotherapy associated to the exeresis surgery, it can be of benefit [es

  1. Future of small scale mining

    Energy Technology Data Exchange (ETDEWEB)

    Meyer, R.F.; Carman, J.S. (eds.)

    1978-01-01

    The first international conference on the future of small scale mining was sponsored by The United Nations Institute for Training and Research (UNITAR) and The Consejo de Recursos Minerales and Secretaria de Patrimonio y Fomento Industrial (SEPAFIN) of the Government of Mexico and held 26 November-5 December 1978, at Jurica, Queretaro, Mexico. Six papers have been entered individually into EDB. The papers mainly concern small metal mining operations and only those concerning coal, uranium, and oil shales have been entered. (LTN)

  2. Water Loss in Small Settlements

    Directory of Open Access Journals (Sweden)

    Mindaugas Rimeika

    2014-10-01

    Full Text Available The main performance indicators of a water supply system include the quality and safety of water, continuous work, relevant pressure and small water loss. The majority of foreign and local projects on reducing water loss have been carried out in the water supply systems of metropolitans; however, the specificity of small settlements differs from that of big cities. Differences can be observed not only in the development of infrastructure and technical indicators but also in the features of water consumption. The article presents the analysis of water loss formation and describes reduction measures in a small settlement. The conducted research defines that water loss in big cities is much smaller than that in small settlements. The major part of water used in small settlements is applied for agrarian purposes rather than for domestic needs. It has been found that water is employed for the irrigation of plants and livestock watering, which often is not accounted. Research also shows that slight (<0.2 m³/h physical water loss (holes in the network that occur in small settlements may compose up to 30% of all water supplied to the water network.

  3. World Small Hydropower Development Report

    Energy Technology Data Exchange (ETDEWEB)

    Liu, Heng; Esser, Lara (ICSGP (China)); Masera, Diego (UNIDO, Vienna (Austria))

    2013-07-01

    Currently, small hydropower plants with a capacity of 10 MW, exist in 148 countries or territories worldwide. Four other countries have been identified with resource potential. This report aims to identify the development status and resource potential of small hydro in various countries, territories and regions throughout the world. Working with experts at the ground level to compile and share existing information, experiences and challenges, one comprehensive report was created. Decision-makers, stakeholders and potential investors clearly need this comprehensive information to more effectively promote small hydropower as a renewable and rural energy source for sustainable development and to overcome the existing development barriers. The findings of this report show that small hydropower potential globally is approximated at almost 173 GW. The figure is arrived by totaling data from a wide range of sources with potential compromise of data integrity to varying degrees. For example, research data on economically feasible potential were more readily available in developed countries than those in the least developed or developing countries. More than half of the world's known hydropower potential is located in Asia, around one third can be found in Europe and the Americas. It is possible in the future that more small hydropower potential might be identified both on the African and American continents. The installed small hydropower capacity (up to 10 MW) is estimated to be 75 GW in 2011/2012. The report provides detailed data for each country/region, including recommendations on the national, regional and international level.

  4. Small

    Energy Technology Data Exchange (ETDEWEB)

    Montoya, Joseph

    2013-07-18

    Representing the Center on Nanostructuring for Efficient Energy Conversion (CNEEC), this document is one of the entries in the Ten Hundred and One Word Challenge. As part of the challenge, the 46 Energy Frontier Research Centers were invited to represent their science in images, cartoons, photos, words and original paintings, but any descriptions or words could only use the 1000 most commonly used words in the English language, with the addition of one word important to each of the EFRCs and the mission of DOE energy. The mission of CNEEC is to understand how nanostructuring can enhance efficiency for energy conversion and solve fundamental cross-cutting problems in advanced energy conversion and storage systems.

  5. DEVELOPMENT OF SMALL INJECTION MOULDING MACHINE FOR FORMING SMALL PLASTIC ARTICLES FOR SMALL-SCALE INDUSTRIES

    Directory of Open Access Journals (Sweden)

    OYETUNJI, A.

    2010-03-01

    Full Text Available Development of small injection moulding machine for forming small plastic articles in small-scale industries was studied. This work which entailed design, construction and test small injection moulding machine that was capable of forming small plastic articles by injecting molten resins into a closed, cooled mould, where it solidifies to give the desired products was developed. The machine was designed and constructed to work as a prototype for producing very small plastic components. Design concept, operation, and assembly of components parts were made. Also, working drawings and materials selection were made based on calculations of the diameter of injection plunger, number of teeth required for the plunger rack and spur gear, the angular velocity, number of revolution, torque and power obtained from the electric motor selected and the leverage on the handle of the machine. The machine parts/components were then assembled in line with the designed made, thereafter the constructed machine was tested using high density polyethylene and master batch. The results obtained from the test were satisfactory.

  6. Small plasma physics experiments 2

    International Nuclear Information System (INIS)

    Lee, S.; Sakanaka, P.H.

    1990-01-01

    This book contains 9 review papers and 13 contributed papers presented at the second symposium on Small Scale Laboratory Plasma Physics Experiments, held May 15 - June 9, 1989, at the Institute for Theoretical Physics, Trieste, Italy. The main objective of the symposium was the gathering of researchers of small experimental laboratory plasma physics groups to give lectures and present papers. The meeting also included workshops as a forum for exchange of experiences, partial research results and interesting solutions found for experimental problems. The Symposium serves as a counterpart of the rest of the Spring College activities, in which many of the presentations come from the large laboratories with budgets hundreds of times larger than those for small scale laboratory plasma experimentation reported on here. In the 3-day Symposium, 9 lectures and 28 contributed papers including poster papers were given and 10 workshops were conducted on themes of small scale laboratory experiments. Twenty-five research groups from 20 countries participated. The review lectures are divided into devices: Rotamak, Tokamak, compact tori, and plasma focus; and into techniques: pulse technology, optical and electric diagnostics. The contributed papers range from pinch and focus devices and experiments to vacuum spark, a multi-magnetic dipole device and a small FRC experiment, and includes techniques such as surface preparation, electrostatic lens and multiframe holographic interferometry. There is also a section on theoretical work including dimensionality of fluctuations, heat transport and classical resistive decay. Refs, figs and tabs

  7. Industry Research and Recommendations for Small Buildings and Small Portfolios

    Energy Technology Data Exchange (ETDEWEB)

    Langner, Rois [National Renewable Energy Lab. (NREL), Golden, CO (United States); Hendron, Bob [National Renewable Energy Lab. (NREL), Golden, CO (United States); Pless, Shanti [National Renewable Energy Lab. (NREL), Golden, CO (United States); Huppert, Mark [National Trust for Historic Preservation, Washington, DC (United States); Cochrane, Ric [National Trust for Historic Preservation, Washington, DC (United States)

    2013-12-01

    Small buildings have been left behind in the energy efficiency marketplace because financial and technical resources have flowed to larger commercial buildings. DOE's Building Technologies Office works with the commercial building industry to accelerate the uptake of energy efficiency technologies and techniques in existing and new commercial buildings (DOE 2013). BTO recognizes the SBSP sector'spotential for significant energy savings and the need for investments in resources that are tailored to this sector's unique needs. The industry research and recommendations described in this report identify potential approaches and strategic priorities that BTO could explore over the next 3-5 years that will support the implementation of high-potential energy efficiency opportunities for thisimportant sector. DOE is uniquely positioned to provide national leadership, objective information, and innovative tools, technologies, and services to support cost-effective energy savings in the fragmented and complex SBSP sector. Properly deployed, the DOE effort could enhance and complement current energy efficiency approaches. Small portfolios are loosely and qualitatively defined asportfolios of buildings that include only a small number of small buildings. This distinction is important because the report targets portfolio owners and managers who generally do not have staff and other resources to track energy use and pursue energy efficiency solutions.

  8. Marketing the Uniqueness of Small Towns. Small Town Strategy.

    Science.gov (United States)

    Hogg, David H.; Dunn, Douglas

    A small town can strengthen its local economy as a result of business people and concerned citizens collectively identifying that community's uniqueness and then capitalizing on it via advertising, personal selling, sales promotion, or publicity. This publication relates the science of marketing to communities. Seven simple techniques are provided…

  9. Small systems at the LHC

    Science.gov (United States)

    Preghenella, Roberto

    2018-02-01

    In these proceedings, I report on a selection of recent LHC results in small systems from ALICE [1], ATLAS [2] and CMS [3] experiments. Due to the fact that the investigation of QCD in small systems at high multiplicity is becoming an increasingly large subject, interesting the heavy-ion community and more in general the high-energy physics community, not all the related topics can be discussed in this paper. The focus will be given to some of the measurements addressing the physics of collective phenomena in small systems and to the recent results on strangeness enhancement in proton-proton collisions. The reader must be informed that a large number of interesting results did not find space in the discussion reported here.

  10. Small Bowel Review - Part I

    Directory of Open Access Journals (Sweden)

    ABR Thomson

    1997-01-01

    Full Text Available Significant advances have been made in the study of the small bowel. Part I of this two-part review of the small bowel examines carbohydrates, including brush border membrane hydrolysis and sugar transport; amino acids, dipeptides, proteins and food allergy, with a focus on glutamine, peptides and macromolecules, and nucleosides, nucleotides and polyamines; salt and water absorption, and diarrhea, including antidiarrheal therapy and oral rehydration treatment; lipids (digestion and absorption, fatty acid binding proteins, intracellular metabolism, lipoproteins and bile acids; and metals (eg, iron and vitamins.

  11. Starting a Small Instrument Company

    Science.gov (United States)

    Warburton, W. K.

    1997-03-01

    The author is now the owner of an 8 person company that develops specialized electronics to support the detectors used at synchrotron radiation facilities and is spinning this expertise off into developing spectrometry instruments for specialized medical markets. This talk will begin with a short history illustrating the random walk processes leading to the present situation and then consider some of the issues which a small company must deal with in order to survive. From this perspective, the author will then extract a list of steps the prospective physicist/entreprenuer can take, while still a student or post-doc, to prepare for success in growing a small business.

  12. Small Wind Turbine Technology Assessment

    International Nuclear Information System (INIS)

    Avia Aranda, F.; Cruz Cruz, I.

    1999-01-01

    The result of the study carried out under the scope of the ATYCA project Test Plant of Wind Systems for Isolated Applications, about the state of art of the small wind turbine technology (wind turbines with swept area smaller than 40 m 2 ) is presented. The study analyzes the collected information on 60 models of wind turbines from 23 manufacturers in the worldwide market. Data from Chinese manufacturers, that have a large participation in the total number of small wind turbines in operation, are not included, due to the unavailability of the technical information. (Author) 15 refs

  13. Small Molecule PET-Radiopharmaceuticals

    NARCIS (Netherlands)

    Elsinga, Philip H.; Dierckx, Rudi A. J. O.

    This review describes several aspects required for the development of small molecule PET-tracers. Design and selection criteria are important to consider before starting to develop novel PET-tracers. Principles and latest trends in C-11 and F-18-radiochemistry are summarized. In addition an update

  14. Small Bowel Follow-Through

    Science.gov (United States)

    ... real-time x-ray called fluoroscopy and a barium-based contrast material to produce images of the small intestine. ... procedure, the patient drinks a liquid that contains barium or an iodine -based contrast, a contrast material that enhances x-ray images. ( ...

  15. VEGA, a small launch vehicle

    Science.gov (United States)

    Duret, François; Fabrizi, Antonio

    1999-09-01

    Several studies have been performed in Europe aiming to promote the full development of a small launch vehicle to put into orbit one ton class spacecrafts. But during the last ten years, the european workforce was mainly oriented towards the qualification of the heavy class ARIANE 5 launch vehicle.Then, due also to lack of visibility on this reduced segment of market, when comparing with the geosatcom market, no proposal was sufficiently attractive to get from the potentially interrested authorities a clear go-ahead, i.e. a financial committment. The situation is now rapidly evolving. Several european states, among them ITALY and FRANCE, are now convinced of the necessity of the availability of such a transportation system, an important argument to promote small missions, using small satellites. Application market will be mainly scientific experiments and earth observation; some telecommunications applications may be also envisaged such as placement of little LEO constellation satellites, or replacement after failure of big LEO constellation satellites. FIAT AVIO and AEROSPATIALE have proposed to their national agencies the development of such a small launch vehicle, named VEGA. The paper presents the story of the industrial proposal, and the present status of the project: Mission spectrum, technical definition, launch service and performance, target development plan and target recurring costs, as well as the industrial organisation for development, procurement, marketing and operations.

  16. Small Business Innovations (Crystal Components)

    Science.gov (United States)

    1991-01-01

    Scientific Materials Corporation, Bozeman, MT developed the SciMax line of improved Nd:Yag crystals under an Small Business Innovation Research (SBIR) contract with Langley Research Center. They reduced the amount of water trapped in the crystals during growth to improve the optical quality and efficiency. Applications of the crystals include fiber optics, telecommunications, welding, drilling, eye surgery and medical instrumentation.

  17. Calculator. Owning a Small Business.

    Science.gov (United States)

    Parma City School District, OH.

    Seven activities are presented in this student workbook designed for an exploration of small business ownership and the use of the calculator in this career. Included are simulated situations in which students must use a calculator to compute property taxes; estimate payroll taxes and franchise taxes; compute pricing, approximate salaries,…

  18. Small animal radiotherapy research platforms

    International Nuclear Information System (INIS)

    Verhaegen, Frank; Granton, Patrick; Tryggestad, Erik

    2011-01-01

    Advances in conformal radiation therapy and advancements in pre-clinical radiotherapy research have recently stimulated the development of precise micro-irradiators for small animals such as mice and rats. These devices are often kilovolt x-ray radiation sources combined with high-resolution CT imaging equipment for image guidance, as the latter allows precise and accurate beam positioning. This is similar to modern human radiotherapy practice. These devices are considered a major step forward compared to the current standard of animal experimentation in cancer radiobiology research. The availability of this novel equipment enables a wide variety of pre-clinical experiments on the synergy of radiation with other therapies, complex radiation schemes, sub-target boost studies, hypofractionated radiotherapy, contrast-enhanced radiotherapy and studies of relative biological effectiveness, to name just a few examples. In this review we discuss the required irradiation and imaging capabilities of small animal radiation research platforms. We describe the need for improved small animal radiotherapy research and highlight pioneering efforts, some of which led recently to commercially available prototypes. From this, it will be clear that much further development is still needed, on both the irradiation side and imaging side. We discuss at length the need for improved treatment planning tools for small animal platforms, and the current lack of a standard therein. Finally, we mention some recent experimental work using the early animal radiation research platforms, and the potential they offer for advancing radiobiology research. (topical review)

  19. Small animal radiotherapy research platforms

    Energy Technology Data Exchange (ETDEWEB)

    Verhaegen, Frank; Granton, Patrick [Department of Radiation Oncology (MAASTRO), GROW-School for Oncology and Developmental Biology, Maastricht University Medical Center, Maastricht 6201 BN (Netherlands); Tryggestad, Erik, E-mail: frank.verhaegen@maastro.nl [Department of Radiation Oncology and Molecular Radiation Sciences, The Johns Hopkins University School of Medicine, Baltimore, MD 21231 (United States)

    2011-06-21

    Advances in conformal radiation therapy and advancements in pre-clinical radiotherapy research have recently stimulated the development of precise micro-irradiators for small animals such as mice and rats. These devices are often kilovolt x-ray radiation sources combined with high-resolution CT imaging equipment for image guidance, as the latter allows precise and accurate beam positioning. This is similar to modern human radiotherapy practice. These devices are considered a major step forward compared to the current standard of animal experimentation in cancer radiobiology research. The availability of this novel equipment enables a wide variety of pre-clinical experiments on the synergy of radiation with other therapies, complex radiation schemes, sub-target boost studies, hypofractionated radiotherapy, contrast-enhanced radiotherapy and studies of relative biological effectiveness, to name just a few examples. In this review we discuss the required irradiation and imaging capabilities of small animal radiation research platforms. We describe the need for improved small animal radiotherapy research and highlight pioneering efforts, some of which led recently to commercially available prototypes. From this, it will be clear that much further development is still needed, on both the irradiation side and imaging side. We discuss at length the need for improved treatment planning tools for small animal platforms, and the current lack of a standard therein. Finally, we mention some recent experimental work using the early animal radiation research platforms, and the potential they offer for advancing radiobiology research. (topical review)

  20. Joint research using small tokamaks

    Czech Academy of Sciences Publication Activity Database

    Gryaznevich, M.P.; Del Bosco, E.; Malaquias, A.; Mank, G.; Van Oost, G.; He, Yexi; Hegazy, H.; Hirose, A.; Hron, Martin; Kuteev, B.; Ludwig, G.O.; Nascimento, I.C.; Silva, C.; Vorobyev, G.M.

    2005-01-01

    Roč. 45, č. 10 (2005), S245-S254 ISSN 0029-5515. [Fusion Energy Conference contributions. Vilamoura, 1.11.2004-6.11.2004] Institutional research plan: CEZ:AV0Z20430508 Keywords : small tokamaks * thermonuclear fusion Subject RIV: BL - Plasma and Gas Discharge Physics Impact factor: 3.418, year: 2005

  1. Small Mammals, Reptiles, and Amphibians

    Science.gov (United States)

    Bryce Rickel

    2005-01-01

    This chapter focuses on small mammals, reptiles, and amphibians that inhabit the grasslands within the Southwestern Region of the USDA Forest Service. The chapter is not intended to be an all inclusive list of species, but rather to address the species that play important roles in grassland ecosystems and that often are associated with the management of grasslands....

  2. Angiosarcoma of the small intestine

    African Journals Online (AJOL)

    shobha

    dissemination and rapid fatality. Diagnosis was based on histological morphology using Hematoxylin and Eosin (H and E) stains, plus reticulin special stain .... least common in the heart and orbit (3% each). No occurrence in the small bowel or the gastrointestinal. Figure 1: Malignant cells arranged in vasoformative pattern.

  3. Nuclear physics at small distances

    Indian Academy of Sciences (India)

    calculations [1,2]. Guidance for appropriate degrees of freedom at small length scales can be obtained from the available information on the structure of ... In this picture the spin-orbit potential is calculated first by calculating the .... where V,G and t are (2x2) matrices, because in calculating the t-matrix we include both,.

  4. Small Big Data Congress 2017

    NARCIS (Netherlands)

    Doorn, J.

    2017-01-01

    TNO, in collaboration with the Big Data Value Center, presents the fourth Small Big Data Congress! Our congress aims at providing an overview of practical and innovative applications based on big data. Do you want to know what is happening in applied research with big data? And what can already be

  5. small scale irrigation management practices

    African Journals Online (AJOL)

    It yis against this background that this study focuses on small scale irrigation fadama farming being a ... inputs, fertilizer and irrigation water. ` The functions .... chemical fertilizer. More of irrigation water could also be used for better eсмciency. The farmers in the study area are not making eсicient use of their resources. There.

  6. Small Business Incubator Resource Kit.

    Science.gov (United States)

    Small Business Administration, Washington, DC.

    This kit consists of a set of resources to assist those interested in the start-up and management (incubation) of a new business. A guide to starting and managing a small business incubator (SBI) is provided. Included in the guide are the following: a discussion of the role and characteristics of the SBI concept; guidelines for carrying out the…

  7. Managing a Small Business Incubator.

    Science.gov (United States)

    Kirchner, Charles, Ed.

    An increasingly popular economic development tool to improve the success rate of new firms is the small business incubator. These are buildings in which a number of new or growing businesses can locate and operate at a much lower overhead cost than in conventional space where market rates prevail. Incubator facilities are characterized by access…

  8. New Russian Combat Small Boats

    Directory of Open Access Journals (Sweden)

    Aleksandr F. Mitrofanov

    2016-12-01

    Full Text Available The article presents an overview of small combat boats. The author provides a description and gives an analysis of the characteristics of the boat "Raptor", boat "BK-16", boat "Strizh-4-1 DSh", and assault boat "BK-10".

  9. Small firm transformation through IS

    NARCIS (Netherlands)

    Levy, Margi; Powell, Philip

    2008-01-01

    Globally, Small and Medium-sized Enterprises (SMEs) are encouraged, particularly by governments, to embrace c-business. Fully adopting e-business involves substantial change in firms, both internally and externally. However, there is little understanding of the mechanisms by which such business

  10. Angiosarcoma of the small intestine

    African Journals Online (AJOL)

    shobha

    You need a software (e.g. RSSReader, Feed Demon, FeedReader, My Yahoo!, NewsGator and. NewzCrawler) to get advantage of this tool. RSS feeds can also be read through FireFox or Microsoft Outlook 2007. Once any of these small (and mostly free) software is installed, add www.annalsafrmed.org/rssfeed.asp as one.

  11. Small for Gestational Age (SGA)

    Science.gov (United States)

    ... Throat (ENT) Dental/Orthodontics Urology Orthopedics Child Development Psychology Special Education Services DO WE NEED TO IMPROVE ... continued research and education about Small for Gestational Age and Russell-Silver syndrome by ... our closed SGA Facebook group for parents. After you request to join, please ...

  12. Sales Training for Small Companies

    Science.gov (United States)

    Kinder, James F.

    1974-01-01

    The author suggests methods a sales manager in a small company can use to train effective salesmen; he defines the sales manager's role, and cites internal and external training resources. The absence of sales training courses in metropolitan Canada is noted. (AJ)

  13. Small rocket research and technology

    Science.gov (United States)

    Schneider, Steven; Biaglow, James

    1993-11-01

    Small chemical rockets are used on nearly all space missions. The small rocket program provides propulsion technology for civil and government space systems. Small rocket concepts are developed for systems which encompass reaction control for launch and orbit transfer systems, as well as on-board propulsion for large space systems and earth orbit and planetary spacecraft. Major roles for on-board propulsion include apogee kick, delta-V, de-orbit, drag makeup, final insertions, north-south stationkeeping, orbit change/trim, perigee kick, and reboost. The program encompasses efforts on earth-storable, space storable, and cryogenic propellants. The earth-storable propellants include nitrogen tetroxide (NTO) as an oxidizer with monomethylhydrazine (MMH) or anhydrous hydrazine (AH) as fuels. The space storable propellants include liquid oxygen (LOX) as an oxidizer with hydrazine or hydrocarbons such as liquid methane, ethane, and ethanol as fuels. Cryogenic propellants are LOX or gaseous oxygen (GOX) as oxidizers and liquid or gaseous hydrogen as fuels. Improved performance and lifetime for small chemical rockets are sought through the development of new predictive tools to understand the combustion and flow physics, the introduction of high temperature materials to eliminate fuel film cooling and its associated combustion inefficiency, and improved component designs to optimize performance. Improved predictive technology is sought through the comparison of both local and global predictions with experimental data. Results indicate that modeling of the injector and combustion process in small rockets needs improvement. High temperature materials require the development of fabrication processes, a durability data base in both laboratory and rocket environments, and basic engineering property data such as strength, creep, fatigue, and work hardening properties at both room and elevated temperature. Promising materials under development include iridium-coated rhenium and a

  14. Small is beautiful: models of small neuronal networks.

    Science.gov (United States)

    Lamb, Damon G; Calabrese, Ronald L

    2012-08-01

    Modeling has contributed a great deal to our understanding of how individual neurons and neuronal networks function. In this review, we focus on models of the small neuronal networks of invertebrates, especially rhythmically active CPG networks. Models have elucidated many aspects of these networks, from identifying key interacting membrane properties to pointing out gaps in our understanding, for example missing neurons. Even the complex CPGs of vertebrates, such as those that underlie respiration, have been reduced to small network models to great effect. Modeling of these networks spans from simplified models, which are amenable to mathematical analyses, to very complicated biophysical models. Some researchers have now adopted a population approach, where they generate and analyze many related models that differ in a few to several judiciously chosen free parameters; often these parameters show variability across animals and thus justify the approach. Models of small neuronal networks will continue to expand and refine our understanding of how neuronal networks in all animals program motor output, process sensory information and learn. Copyright © 2012 Elsevier Ltd. All rights reserved.

  15. Small Bodies, Big Discoveries: NASA's Small Bodies Education Program

    Science.gov (United States)

    Mayo, L.; Erickson, K. J.

    2014-12-01

    2014 is turning out to be a watershed year for celestial events involving the solar system's unsung heroes, small bodies. This includes the close flyby of comet C/2013 A1 / Siding Spring with Mars in October and the historic Rosetta mission with its Philae lander to comet 67P/Churyumov-Gerasimenko. Beyond 2014, the much anticipated 2015 Pluto flyby by New Horizons and the February Dawn Mission arrival at Ceres will take center stage. To deliver the excitement and wonder of our solar system's small bodies to worldwide audiences, NASA's JPL and GSFC education teams in partnership with NASA EDGE will reach out to the public through multiple venues including broadcast media, social media, science and math focused educational activities, observing challenges, interactive visualization tools like "Eyes on the Solar System" and more. This talk will highlight NASA's focused education effort to engage the public in small bodies mission science and the role these objects play in our understanding of the formation and evolution of the solar system.

  16. Paraneoplastic cerebellar syndromes associated with antibodies against Purkinje cells.

    Science.gov (United States)

    Schwenkenbecher, Philipp; Chacko, Lisa; Pul, Refik; Sühs, Kurt-Wolfram; Wegner, Florian; Wurster, Ulrich; Stangel, Martin; Skripuletz, Thomas

    2017-12-18

    The paraneoplastic cerebellar syndrome presents as severe neuroimmunological disease associated with malignancies. Antibodies against antigens expressed by tumor cells cross-react with proteins of cerebellar Purkinje cells leading to neuroinflammation and neuronal loss. These antineuronal antibodies are preferentially investigated by serological analyses while examination of the cerebrospinal fluid is only performed infrequently. We retrospectively investigated 12 patients with antineuronal antibodies against Purkinje cells with a special focus on cerebrospinal fluid. Our results confirm a subacute disease with a severe cerebellar syndrome in 10 female patients due to anti-Yo antibodies associated mostly with gynecological malignancies. While standard cerebrospinal fluid parameters infrequently revealed pathological results, all patients presented oligoclonal bands indicating intrathecal IgG synthesis. Analyses of anti-Yo antibodies in cerebrospinal fluid by calculating the antibody specific index revealed intrathecal synthesis of anti-Yo antibodies in these patients. In analogy to anti-Yo syndrome, an intrathecal production of anti-Tr antibodies in one patient who presented with a paraneoplastic cerebellar syndrome was detected. In an additional patient, anti-Purkinje cell antibodies of unknown origin in the cerebrospinal fluid but not in serum were determined suggesting an isolated immune reaction within the central nervous system (CNS) and underlining the importance of investigating the cerebrospinal fluid. In conclusion, patients with a cerebellar syndrome display a distinct immune reaction within the cerebrospinal fluid including intrathecal synthesis of disease-specific antibodies. We emphasize the importance of a thorough immunological work up including investigations of both serum and cerebrospinal fluid.

  17. An Overwhelming Postsplenectomy Infection Syndrome Associated with Diabetes M ellitus

    OpenAIRE

    Mai, Tomizawa; Takashi, Nagai; Kazunori, Tonouchi; Naoko, Tonooka; Gen, Takahashi; Mie, Kotake; Ken, Masubuchi; Takao, Ishizuka; Kunihiko, Iizuka; Yoshio, Umegae

    2009-01-01

    A 72-year-old male diabetic patient was admitted due to autoimmune hemolytic anemia (AIHA).We started 1mg/kg weight of prednisolone daily and multiple daily insulin infusion for hyperglycemia.Gall bladder cancer and early stage gastric cancer were then diagnosed. We removed the gall bladderand the spleen for treatment of AIHA without prednisolone for better control of diabetes mellitus. Fiveweeks later, endoscopic gastromucosal resection (EMR) was performed for gastric cancer. The patientsuff...

  18. Immune reconstitution inflammatory syndrome associated with secondary syphilis.

    Science.gov (United States)

    Frunza-Stefan, Simona; Acharya, Gyanendra; Kazlouskaya, Viktoryia; Vukasinov, Paunel; Chiou, Yushan; Thet, Zeyar

    2017-03-01

    Immune reconstitution inflammatory syndrome (IRIS) is a condition associated with paradoxical worsening and/or new onset of an opportunistic infection in HIV patients following the initiation of anti-retroviral therapy or switching to more potent antiretroviral therapy (ART) regimen. Although IRIS associated with many opportunistic infections (OIs) has been well reported, syphilis has very rarely been mentioned in this regard. A 52-year-old male, diagnosed with AIDS six weeks ago, presented with the disseminated non-pruritic painless skin rash. He denied any fever, cough, shortness of breath, and joint pain or swelling. The patient had no similar symptoms, genital ulcers, or any medical illness in the past. CD4 cell count and viral load were 40 cells/mm 3 and 280,000 copies/ml, respectively, while screening tests for OIs including rapid plasma reagin test, quantiferon, cryptococcal antigen, and toxoplasma tests were negative at the time of HIV diagnosis. After three days of initiation of anti-retroviral therapy, he developed the above-mentioned skin rash. Repeat rapid plasma regain (RPR) test at this time was also negative. Punch biopsy of the skin lesion demonstrated findings suggestive of secondary syphilitic lesions, which was confirmed by immunostain. The repeat RPR, CD4 cell count, and viral load showed a titer of 1:256, 257 cells/mm 3 , and 5000 copies/ml, respectively. His skin rashes faded away, and RPR titer trended down on treatment with benzathine penicillin without discontinuation of ART. The presence of an IRIS response does not predict overall HIV or OI treatment responses, and discontinuation of ART is not generally recommended as the benefits of treating HIV infection outweighs the risk associated with IRIS.

  19. Reversible pathologic jealousy (Othello syndrome) associated with amantadine.

    Science.gov (United States)

    McNamara, P; Durso, R

    1991-01-01

    We describe a case of pathologic jealousy (Othello syndrome) in a patient with Parkinson disease, which abated after discontinuing amantadine. We indicate that early recognition and treatment of the syndrome in this disease may avert physical violence. We also believe that our report further suggests a link between this specific behavioral disorder and dopaminergic activity.

  20. Atypical Neuroleptic Malignant Syndrome Associated with Use of Clozapine

    Directory of Open Access Journals (Sweden)

    Quevedo-Florez Leonardo

    2017-01-01

    Full Text Available The Neuroleptic Malignant Syndrome (NMS is a medical emergency of infrequent presentation in the emergency department, which is associated with the use of psychiatric drugs, such as typical and atypical antipsychotics. Our case addresses a 55-year-old patient diagnosed with undifferentiated schizophrenia for 10 years, who had been receiving clozapine and clonazepam as part of their treatment. This patient presents the symptoms of Neuroleptic Malignant Syndrome without fever, which improves with treatment especially with the withdrawal of clozapine. In the absence of fever and clinical improvement, the patient is considered to have an atypical presentation of this disease.

  1. Multiple organ dysfunction syndrome associated with Mycoplasma pneumoniae infection

    Directory of Open Access Journals (Sweden)

    Shu-Bo Zhai

    2012-03-01

    Full Text Available In this study, we report one case of a three-year-old boy infected with Mycoplasma pneumonia (MP and presenting concomitant multiple organ damage of the heart, kidney, lung and liver, among others, together with a brief review for the diagnosis and treatment of MP infection with multiple organ dysfunction syndrome (MODS.

  2. Atypical haemolytic uraemic syndrome associated with a hybrid complement gene.

    Directory of Open Access Journals (Sweden)

    Julian P Venables

    2006-10-01

    Full Text Available BACKGROUND: Sequence analysis of the regulators of complement activation (RCA cluster of genes at chromosome position 1q32 shows evidence of several large genomic duplications. These duplications have resulted in a high degree of sequence identity between the gene for factor H (CFH and the genes for the five factor H-related proteins (CFHL1-5; aliases CFHR1-5. CFH mutations have been described in association with atypical haemolytic uraemic syndrome (aHUS. The majority of the mutations are missense changes that cluster in the C-terminal region and impair the ability of factor H to regulate surface-bound C3b. Some have arisen as a result of gene conversion between CFH and CFHL1. In this study we tested the hypothesis that nonallelic homologous recombination between low-copy repeats in the RCA cluster could result in the formation of a hybrid CFH/CFHL1 gene that predisposes to the development of aHUS. METHODS AND FINDINGS: In a family with many cases of aHUS that segregate with the RCA cluster we used cDNA analysis, gene sequencing, and Southern blotting to show that affected individuals carry a heterozygous CFH/CFHL1 hybrid gene in which exons 1-21 are derived from CFH and exons 22/23 from CFHL1. This hybrid encodes a protein product identical to a functionally significant CFH mutant (c.3572C>T, S1191L and c.3590T>C, V1197A that has been previously described in association with aHUS. CONCLUSIONS: CFH mutation screening is recommended in all aHUS patients prior to renal transplantation because of the high risk of disease recurrence post-transplant in those known to have a CFH mutation. Because of our finding it will be necessary to implement additional screening strategies that will detect a hybrid CFH/CFHL1 gene.

  3. Acquired immunodeficiency syndrome associated with blood-product transfusions

    Energy Technology Data Exchange (ETDEWEB)

    Jett, J.R.; Kuritsky, J.N.; Katzmann, J.A.; Homburger, H.A.

    1983-11-01

    A 53-year-old white man had fever, malaise, and dyspnea on exertion. His chest roentgenogram was normal, but pulmonary function tests showed impaired diffusion capacity and a gallium scan showed marked uptake in the lungs. Results of an open-lung biopsy documented Pneumocystis carinii pneumonia. Immunologic test results were consistent with the acquired immunodeficiency syndrome. The patient denied having homosexual contact or using intravenous drugs. Twenty-nine months before the diagnosis of pneumocystis pneumonia was made, the patient had had 16 transfusions of whole blood, platelets, and fresh-frozen plasma during coronary artery bypass surgery at another medical center. This patient is not a member of any currently recognized high-risk group and is believed to have contracted the acquired immunodeficiency syndrome from blood and blood-product transfusions.

  4. [Pathogenesis of lipodystrophy and metabolic syndromes associated with HIV infection].

    Science.gov (United States)

    Muñoz-Sanz, Agustín; Rodríguez-Vidigal, Francisco F; Domingo, Pere

    2006-09-30

    Lipodystrophy, and the metabolic alterations (dislipemia, insulin-resistance) associated with human immunodeficiency virus (HIV) infection, is a multifactorial syndrome due to the interaction of host related factors (cellular immune status, diet, gene mutations), viral factors (cytokine synthesis, polyunsaturated fatty acid or PUFA depletion), and pharmacological effects (mitochondrial DNA-polymerase inhibition, lipolysis inhibition, adiponectin synthesis reduction). HIV probably modifies the adipocyte differentiation and the lipid metabolism. This retroviral effect is mediated by proinflammatory cytokines (tumor necrosis factor) and the participation of other factors (drugs, diet), all in the context of a particular host genetic setting. The adipocyte (and several cellular receptors, fatty acids, membrane proteins, and cytokines) plays a central role in the pathogenesis of HIV-associated lipodystrophy.

  5. Werewolf syndrome associated with gingival fibromatosis: A rare case report

    Directory of Open Access Journals (Sweden)

    Mysore K Sunil

    2016-01-01

    Full Text Available Hypertrichosis is a rare disorder that causes abnormal excessive body hair growth. Hypertrichosis has informally been called werewolf syndrome because the appearance is similar to that of a werewolf. It is often associated with additional anomalies including gingival hyperplasia, deafness, cardiomegaly and bone abnormalities. The association of gingival fibromatosis and a coarse facies could further worsen the esthetics. Thus, a multidisciplinary approach involving a psychologist, a dentist as well as a dermatologist would be mandatory. We present this rare syndrome describing a case of a 9-year-old boy with congenital hypertrichosis and gingival fibromatosis. The growth being normal, the features of this boy were similar to hypertrichosis. Hypertrichosis has informally been called werewolf syndrome because the appearance is similar to the werewolf.

  6. Werewolf syndrome associated with gingival fibromatosis: A rare case report

    OpenAIRE

    Mysore K Sunil; Ashwarya Trivedi; Saloni Arora; Sonam Gupta

    2016-01-01

    Hypertrichosis is a rare disorder that causes abnormal excessive body hair growth. Hypertrichosis has informally been called werewolf syndrome because the appearance is similar to that of a werewolf. It is often associated with additional anomalies including gingival hyperplasia, deafness, cardiomegaly and bone abnormalities. The association of gingival fibromatosis and a coarse facies could further worsen the esthetics. Thus, a multidisciplinary approach involving a psychologist, a dentist a...

  7. Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne

    DEFF Research Database (Denmark)

    Vinkel, Caroline; Thomsen, Simon F

    2017-01-01

    have been reported in the literature including pyoderma gangrenosum, acne, and pyogenic arthritis (PAPA), pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH), pyoderma gangrenosum, acne, and spondyloarthritis (PASS), pyoderma gangrenosum, acne, pyogenic arthritis, and hidradenitis...

  8. [The Overlap Syndrome: association of COPD and Obstructive Sleep Apnoea].

    Science.gov (United States)

    Weitzenblum, E; Chaouat, A; Kessler, R; Canuet, M; Hirschi, S

    2010-04-01

    Chronic obstructive pulmonary disease (COPD) and the obstructive sleep apnoea-hypopnoea syndrome (OSAHS) are both common diseases affecting respectively 10 and 5% of the adult population over 40 years of age. Their coexistence, which is denominated "Overlap Syndrome", can be expected to occur in about 0.5% of this population. Two recent epidemiologic studies have shown that the prevalence of OSAHS is not higher in COPD than in the general population, and that the coexistence of the two conditions is due to chance and not through a pathophysiological linkage. Patients with "overlap" have a higher risk of sleep-related O(2) desaturation than do patients with COPD alone and the same degree of bronchial obstruction. They have an increased risk of developing hypercapnic respiratory failure and pulmonary hypertension when compared with patients with OSAHS alone and with patients with "usual" COPD. In patients with overlap, hypoxaemia, hypercapnia, and pulmonary hypertension can be observed in the presence of mild to moderate bronchial obstruction, which is different from "usual" COPD. Treatment of the overlap syndrome consists of nasal continuous positive airway pressure or nocturnal non-invasive ventilation (NIV), with or without nocturnal O(2). Patients who are markedly hypoxaemic during the daytime (PaO(2)<55-60 mmHg) should be given conventional long-term O(2) therapy in addition to nocturnal ventilation. Copyright 2010 SPLF. Published by Elsevier Masson SAS. All rights reserved.

  9. Cantú Syndrome Associated with Ovarian Agenesis

    OpenAIRE

    Fryssira, Helena; Psoni, Stavroula; Amenta, Styliani; Tsoutsou, Eirini; Sofocleous, Christalena; Manolakos, Emmanouil; Gavra, Maria; Lüdecke, Hermann-Joseph; Czeschik, Johanna-Christina

    2017-01-01

    Cantú syndrome is a very rare autosomal dominant disorder characterized by generalized congenital hypertrichosis, neonatal macrosomia, coarse face, cardiomegaly, and occasionally, skeletal abnormalities. The syndrome has been attributed to mutated ABCC9 or KCNJ8 genes. We present a 4-year-old girl with developmental delay, distinctive coarse facial features, and generalized hypertrichosis apparent since birth. The investigation revealed absent ovaries and a hypoplastic uterus which have not b...

  10. Serotonin syndrome associated with sertraline use: case report

    Directory of Open Access Journals (Sweden)

    Bárbara Werner Griciunas

    2017-06-01

    Full Text Available Case report of serotonin syndrome in patient who initiated the use of sertraline at a dose greater than twice the recommended for the treatment of psychotic depression. The patient presented contracture of the limbs, puzzled look, mutism and blood pressure 230x110 mmHg. The syndrome is increasingly common, although it is not well recognized. Many medications can cause it and this possibility should be considered in patients taking serotonergic drugs presenting autonomic or mental disorders and neurological symptoms. The findings of clonus, oculogyric crisis, hyperreflexia and hypertonicity should lead to the medication review. Treatment focuses on interruption of causative agents, treatment of a possible hyperthermia and use of benzodiazepines to decrease hypertonus and neurological excitability.

  11. [Muir-Torre syndrome associated with Waldenstrom's macroglobulinemia].

    Science.gov (United States)

    Velter, C; Bourlond, F; Wettle, C; Lioure, B; Lipsker, D; Maugard, C; Cribier, B

    2016-12-01

    Muir-Torre syndrome (MTS), a cutaneous variant of Lynch syndrome, consists of hereditary predisposition to cutaneous tumours and gastrointestinal and gynaecological neoplasms, with autosomal dominant transmission. It is associated with mutations in genes coding for proteins in the DNA mismatch repair system. Herein, we report a case of a male patient presenting Waldenstrom's macroglobulinemia since the age of 50 and which, after the age of 65 years, developed into sebaceous tumours (5 sebaceous adenomas, 1 sebaceoma, 1 sebaceous carcinoma) and colonic lesions (4 adenomas). The clinical phenotype was consistent with MTS. Somatic analysis carried out on one sebaceous tumour showed instability of the microsatellites with loss of expression of MSH2 and MSH6 although constitutional genetic analysis showed no germline mutations known to be harmful. This noteworthy case raises a number of questions, including the possibility of association between STM and Waldenstrom's macroglobulinemia, which is discussed herein. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  12. Is Marfan syndrome associated with symptomatic intracranial aneurysms?

    NARCIS (Netherlands)

    van den Berg, J. S.; Limburg, M.; Hennekam, R. C.

    1996-01-01

    BACKGROUND AND PURPOSE: Marfan syndrome is a heritable disorder of connective tissue caused by a deficiency of the glycoprotein fibrillin. In several publications and neurological textbooks, a relationship between Marfan syndrome and intracranial aneurysms has been assumed. METHODS: The records of

  13. Clinical Syndromes Associated with Cardiovascular Diseases: A Review

    Directory of Open Access Journals (Sweden)

    Xing Sheng Yang, MD, PhD, FACC, FAHA

    2017-02-01

    Full Text Available In clinical practice, a variety of syndromes are associated with cardiovascular disease and have characteristic findings. Most of them are an autosomal dominant genetic disorder and have different types of cardiovascular abnormalities, including electrocardiographic conduction defects, arrhythmias, cardiomyopathy, vascular and valvular diseases, cardiac septal defects, and pulmonary problems. There is a growing need for physicians to pay more attention to these syndromes.

  14. Hemolytic Uremic Syndrome Associated with Pneumococcal Pneumonia. A Case Report

    OpenAIRE

    Ariel Efrén Uriarte Méndez; Andrés Prieto Apesteguía; Jesús Vila Díaz; Jorge Luis Capote Padrón; Kendrie Villavicencio Cardoso; Alnilam Fernández González

    2013-01-01

    Hemolytic uremic syndrome is a condition characterized by hemolytic microangiopathic anemia, thrombocytopenia and acute renal failure. In its classic form it is associated with diarrhea and it has a good prognosis. When there is an invasive pneumococcal disease as underlying condition, it has a mortality rate of 25%, and half of the surviving cases develop end-stage renal disease (ESRD). We present the case of a 17-month-old child with hemolytic uremic syndrome secondary to pneumonia with emp...

  15. A case of Pearson syndrome associated with multiple renal cysts.

    Science.gov (United States)

    Gürgey, A; Ozalp, I; Rötig, A; Coşkun, T; Tekinalp, G; Erdem, G; Akeören, Z; Caglar, M; Bakkaloglu, A

    1996-10-01

    A 41-day-old infant who had severe metabolic acidosis, anemia, bleeding, hypoglycemia, and proximal tubulopathy was diagnosed with Pearson syndrome. Fibrosis in the liver, severe iron deposition in hepatocytes, and multiple renal cortical cysts were found on postmortem examination. Southern blot analysis of mitochondrial DNA obtained from peripheral blood revealed a heteroplasmic deletion of approximately 3.5 kilobases.

  16. A case of nephrotic syndrome associated with hydatiform mole

    Directory of Open Access Journals (Sweden)

    Tarlan Hamidehkhou

    2010-12-01

    Full Text Available The present case study is on a 16-year-old woman who was suffering from nephritic syndrome after recovery from complete type of hydatiform mole. She admitted in hospital because of proteinurea and hematuria. In her past medical history she had a suction curettage for hydatiform mole. After she received 4 courses chemotherapy, she completely recovered and βhCG has fallen from 1980 IU/L to under 10 IU/mL. Then she showed generalized edema, proteinurea and hematuria compatible with nephritic syndrome. After six courses chemotherapy the symptoms of nephrotic syndrome and invasive mole diminished, she released from hospital and scheduled for follow-up.

  17. A case of nephrotic syndrome associated with hydatiform mole.

    Science.gov (United States)

    Mohammadjafari, Razieh; Abedi, Parvin; Belady, Syfolah; Hamidehkho, Tarlan; Razi, Taghi

    2010-12-31

    The present case study is on a 16-year-old woman who was suffering from nephrotic syndrome after recovery from complete type of hydatiform mole. She was admitted in hospital because of proteinurea and hematuria. Then she was showing a generalized edema compatible with neprhotic syndrome. In her past medical history she had a suction curettage for hydatiform mole. After she received 4 courses chemotherapy, she completely recovered and βhCG has fallen from 12127 IU/L to under 10 IU/mL. Then she showed generalized edema, proteinurea and hematuria compatible with nephritic syndrome. After six courses chemotherapy the symptoms of nephrotic syndrome and invasive mole diminished, she released from hospital and scheduled for follow-up.

  18. Loose anagen hair syndrome associated with colobomas and dysmorphic features

    DEFF Research Database (Denmark)

    Hansen, Lars Kjaersgård; Brandrup, Flemming; Clemmensen, Ole

    2004-01-01

    Loose anagen hair syndrome is an uncommon congenital disorder. It may occur in association with other syndromes and dysmorphic features. We report a girl who fulfilled the diagnostic criteria for this syndrome as proposed by Tosti (Arch Dermatol 2002, 138: 521-522). She also had several other...

  19. Churg-Strauss syndrome associated with AA amyloidosis: a case ...

    African Journals Online (AJOL)

    Churg Strauss syndrome is a rare systemic and pulmonary vasculitis exceptionally associated with AA amyloidosis. We report the case of a 65-year old woman with past medical history of asthma. She developed polyarthralgia, headache and purpura. A laboratory workout found hypereosinophilia (1150/μL), positive ...

  20. Stevens-Johnson syndrome associated with antimicrobial agent

    Directory of Open Access Journals (Sweden)

    Ivo Cavalcanti Pita Neto

    2008-01-01

    Full Text Available Among the severe reactions to medications, immune-mediated Mucocutaneous Disorders are widely represented. Steven-Johnson’s syndrome, or great multiform erythema, appears as a systemic disturbance, involving the skin and mucous membranes, and is related to several factors, such as, viral or bacterial infections and particularly the administration of medicines, in general painkillers and antibiotics. The objective of this article is report the onset of ulcerative vesicle –blister lesions in the regions of the lips, gums, tongue and genital mucosa membrane in a 26 year-old patient, a leukoderm man, being treated with sulfamethoxazole trimethoprim for a respiratory infection, after being diagnosed as having the Steven-Johnson syndrome. SSJ1 558187121279.

  1. Fading Puppy Syndrome Associated with Toxocara canis Infection

    Directory of Open Access Journals (Sweden)

    Fayez Salib

    2013-07-01

    Full Text Available Aim of this work was studying the role of Toxocara canis infection in fading puppy syndrome and evaluating its treatment. Twelve German shepherd puppies suffered from fading puppy syndrome and their dam were presented. The dead puppies (N.=8 were necropsied. On the other hand, living puppies (N.=4 and their dam were examined both clinically and parasitologically. The collected worms from necropsied puppies were parasitologically identified as Toxocara canis and their counts ranged from 48 to 75 per puppy. The live cases were treated by Flubendazole. The parasitological examinations of stool of live puppies and dam revealed eggs identified as Toxocara canis eggs and their counts ranged from 50 to 350 eggs per gram stool. Post-treatment counts of Toxocara canis worms in stool of live puppies and their dam were ranged from 13 to 79 worms per animal. The results of treatment proved the efficacy of Flubendazole for eradication of Toxocara canis. Toxocara canis considers one of the causes of fading puppy syndrome and could be cured by Flubendazole .

  2. Kounis syndrome associated with amoxicillin/clavulanic acid

    Directory of Open Access Journals (Sweden)

    A Shimi

    2016-01-01

    Full Text Available Kounis syndrome (KS is a life-threatening medical condition that causes severes allergic reaction and acute coronary syndrome (ACS. We describe the case of 56-year-old woman who developed ACS following an anaphylactic reaction to amoxicillin/clavulanic acid. Immediately after the administration of amoxicillin/clavulanic acid, she presented a chest pain, cutaneous allergic, hypotension, and ST depression on the electrocardiogram. After the necessary diagnostic test, the final diagnosis was variant I of KS.

  3. Lance-Adams syndrome associated with cerebellar pathology.

    Science.gov (United States)

    Waddell, Adam; Dirweesh, Ahmed; Ordonez, Fausto; Kososky, Charles; Reddy Peddareddygari, Leema; Grewal, Raji P

    2017-07-01

    Lance-Adams syndrome (LAS) is an uncommon neurological disorder characterized by the development of chronic post-hypoxic myoclonus. There are relatively few cases described following successful cardiopulmonary resuscitation. We report a patient who developed LAS 3 months after successful resuscitation. Cerebral imaging studies indicate that brain pathology in LAS patients is not uniform, suggesting that the pathophysiology of myoclonus may vary from patient to patient. Our patient adds to this etiological heterogeneity by demonstrating the unusual feature of cerebellar pathology by both cerebral magnetic resonance imaging and single-photon emission computed tomography scans. There is also heterogeneity of these patients in their response to therapy. Unlike some patients described in the literature, our patient has not responded to drug treatment. Studies of these rare patients with LAS are important as they provide insight into the pathophysiology of this condition which, it is hopefully, will facilitate the development of more effective therapy.

  4. Superior canal dehiscence syndrome associated with scuba diving.

    Science.gov (United States)

    Kitajima, Naoharu; Sugita-Kitajima, Akemi; Kitajima, Seiji

    2017-06-01

    A 28-year-old female diver presented with dizziness and difficulty clearing her left ear whilst scuba diving. Her pure-tone audiometry and tympanometry were normal. Testing of Eustachian tube function revealed tubal stenosis. Video-oculography revealed a predominantly torsional nystagmus while the patient was in the lordotic position. Fistula signs were positive. High-resolution computed tomography (HRCT) of the temporal bone revealed a diagnosis of bilateral superior semicircular canal dehiscence (SCDS). Cervical vestibular-evoked myogenic potential (cVEMP) testing showed that the amplitude of the cVEMP measured from her left ear was larger than that from the right. In electronystagmography (ENG), nose-pinched Valsalva manoeuvres caused eye movements to be mainly directed counterclockwise with a vertical component. Tullio phenomenon was also positive for both ears. SCDS patients tend to be misdiagnosed and misunderstood; common misdiagnoses in these cases are alternobaric vertigo (AV), inner ear barotrauma, and inner-ear decompression sickness. It is difficult to diagnose vertigo attacks after scuba diving as SCDS; however, when the patient develops sound- and/or pressure-induced vertical-torsional nystagmus, HRCT should be conducted to confirm a diagnosis of SCDS.

  5. Temporomandibular dysfunction syndrome associated with scuba diving mouthpieces.

    Science.gov (United States)

    Hobson, R S

    1991-01-01

    As previous reports have highlighted that temporomandibular joint dysfunction syndrome can occur during scuba diving due to the use of a diving mouthpiece, 74 divers of varied experience (62 male, 12 female) were asked to evaluate the efficiency of the mouthpiece for the ease of grip, insertion into the mouth, clearing of water, air sharing, comfort and overall efficiency. They also recorded the level of muscle and joint discomfort experienced during diving and non-diving activities. The results indicate that temporomandibular joint problems unrelated to diving are compounded by the use of a diving mouthpiece. The diver's assessment of muscle tension and comfort while using the mouthpiece was found to be a good predictor of whether temporomandibular dysfunction would occur and the assessment scores have been used in a formula to aid selection of a mouthpiece. PMID:1913032

  6. Stevens-Johnson syndrome associated with Malarone antimalarial prophylaxis.

    Science.gov (United States)

    Emberger, Michael; Lechner, Arno Michael; Zelger, Bernhard

    2003-07-01

    To the best of our knowledge, Stevens-Johnson syndrome (SJS) has not been reported previously as an adverse reaction to Malarone, which is a combination of atovaquone and proguanil hydrochloride used for antimalarial prophylaxis and therapy. We describe a 65-year-old patient who had SJS with typical clinical and histopathological findings associated with the use of Malarone prophylaxis for malaria. This report should alert physicians to this severe cutaneous reaction, and Malarone should be added to the list of drugs that can potentially cause SJS.

  7. Williams-Beuren syndrome associated with single kidney and ...

    African Journals Online (AJOL)

    Williams-Beuren syndrome is a rare neurodevelopmental disorder, characterized by congenital heart defects, abnormal facial features, mental retardation with specific cognitive and behavioral profile, growth hormone deficiency, renal and skeletal anomalies, inguinal hernia, infantile hypercalcaemia. We report a case with ...

  8. Moyamoya Syndrome Associated with Henoch-Schönlein Purpura

    Science.gov (United States)

    SHIARI, Reza; TABATABAEI NODUSHAN, Seyed Mohamad Hossein; MOHEBBI, Mohamad Mahdi; KARIMZADEH, Parvaneh; JAVADZADEH, Mohsen

    2016-01-01

    Some reports have shown the association between Moyamoya syndrome and autoimmune diseases. Herewith, we present a 3.5 yr old girl with Henoch- Schönleinpurpura (HSP) who was treated with steroids because of sever colicky abdominal pain. However, central nervous system manifestations such as headache, ataxia and vision impairment developed during 6 months of her outpatient follow-up. More evaluation using MRA revealed intracranial stenosis of internal carotid artery and arterial collaterals that were in favor of Moyamoya syndrome. To our knowledge, this is the first report of Moyamoya syndrome following henoch-schönleinpurpura. PMID:27843469

  9. Estrogens, MSI and Lynch syndrome-associated tumors

    NARCIS (Netherlands)

    Ferreira, Ana Monteiro; Westers, Helga; Albergaria, Andre; Seruca, Raquel; Hofstra, Robert M. W.

    2009-01-01

    Estrogens play a major role in the biology of hormone-responsive tissues but also in the normal physiology of various non-typical hormone-responsive tissues. In disease, estrogens have been associated with tumor development, in particular with tumors such as breast, endometrium, ovary and prostate.

  10. Progressive anticonvulsant hypersensitivity syndrome associated with change of drug product

    DEFF Research Database (Denmark)

    Sabroe, T.P.; Sabers, A.

    2008-01-01

    This report describes the laboratory and physical manifestations of lamotrigine-like toxicity in a young man with refractory epilepsy receiving lamotrigine presenting as anticonvulsant hypersensitivity syndrome (AHS) associated with an abrupt change of drug product Udgivelsesdato: 2008/6...

  11. Referral Patterns for Syndromes Associated With Frontotemporal Lobar Degeneration

    OpenAIRE

    Chow, Tiffany W.; Hodges, John R.; Dawson, Kate E.; Miller, Bruce L.; Smith, Virginia; Mendez, Mario F.; Lipton, Anne M.

    2005-01-01

    We compared demographics of subjects diagnosed with frontotemporal degeneration (FTD) at a group of 5 clinics specializing in this non-Alzheimer dementia against those subjects diagnosed at standard Alzheimer disease centers, to determine any differences in referral patterns between such clinics.

  12. Acquired immunodeficiency syndrome associated with blood-product transfusions

    International Nuclear Information System (INIS)

    Jett, J.R.; Kuritsky, J.N.; Katzmann, J.A.; Homburger, H.A.

    1983-01-01

    A 53-year-old white man had fever, malaise, and dyspnea on exertion. His chest roentgenogram was normal, but pulmonary function tests showed impaired diffusion capacity and a gallium scan showed marked uptake in the lungs. Results of an open-lung biopsy documented Pneumocystis carinii pneumonia. Immunologic test results were consistent with the acquired immunodeficiency syndrome. The patient denied having homosexual contact or using intravenous drugs. Twenty-nine months before the diagnosis of pneumocystis pneumonia was made, the patient had had 16 transfusions of whole blood, platelets, and fresh-frozen plasma during coronary artery bypass surgery at another medical center. This patient is not a member of any currently recognized high-risk group and is believed to have contracted the acquired immunodeficiency syndrome from blood and blood-product transfusions

  13. Carmi Syndrome Associated with Gastric Perforation | Das | Annals ...

    African Journals Online (AJOL)

    Annals of Pediatric Surgery. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 9, No 3 (2013) >. Log in or Register to get access to full text downloads.

  14. Flail arm-like syndrome associated with HIV-1 infection

    Directory of Open Access Journals (Sweden)

    Nalini A

    2009-01-01

    Full Text Available During the last 20 years at least 23 cases of motor neuron disease have been reported in HIV-1 seropositive patients. In this report we describe the clinical picture of a young man with HIV-1 clade C infection and flail arm-like syndrome, who we were able to follow-up for a long period. We investigated and prospectively monitored a 34-year-old man with features of flail arm syndrome, who developed the weakness and wasting 1 year after being diagnosed with HIV-1 infection after a routine blood test. He presented in 2003 with progressive, symmetrical wasting and weakness of the proximal muscles of the upper limb of 2 years′ duration. He had severe wasting and weakness of the shoulder and arm muscles. There were no pyramidal signs. He has been on HAART for the last 4 years and the weakness or wasting has not worsened. At the last follow-up in July 2007, the patient had the same neurological deficit and no other symptoms or signs of HIV-1 infection. MRI of the spinal cord in 2007 showed characteristic T2 hyperintense signals in the central part of the spinal cord, corresponding to the central gray matter. Thus, our patient had HIV-1 clade C infection associated with a ′flail arm-like syndrome.′ The causal relationship between HIV-1 infection and amyotrophic lateral sclerosis (ALS-like syndrome is still uncertain. The syndrome usually manifests as a lower motor neuron syndrome, as was seen in our young patient. It is known that treatment with antiretroviral therapy (ART stabilizes/improves the condition. In our patient the weakness and atrophy remained stable over a period of 3.5 years after commencing HAART regimen.

  15. Paraneoplastic Acrokeratosis (Bazex Syndrome associated with lung adenocarcinoma

    Directory of Open Access Journals (Sweden)

    2017-09-01

    This case reports an unusual association of Bazex syndrome with lung adenocarcinoma and highlights the importance of recognizing it for an early diagnosis and a better prognosis of the underlying disease.

  16. Acute Respiratory Distress Syndrome Associated With Rabies: A Case Report

    Directory of Open Access Journals (Sweden)

    Yung-Hsiang Hsu

    2006-02-01

    Full Text Available Acute respiratory distress syndrome (ARDS is the first potentially lethal complication in rabies virus infection, although its occurrence is rare. We report on a fatal case of rabies virus infection in a 45-year-old woman from Hu-Nan Province, China. The neurologic signs of limb numbness and water phobia occurred from 61 days after the dog bite; the clinical course was progressive, with the most severe clinical manifestations being fever, encephalitis, and ARDS. The woman expired 12 days after admission to the hospital. An autopsy proved rabies encephalitis, mainly involving the medulla oblongata, the thalamus, part of the pons, the cerebellum, and the hippocampus. The lung pathologic examination revealed the organizing phase of ARDS with diffuse alveolar damage, hyaline membrane formation, type II alveolar cell hyperplasia accompanied by proliferation of fibroblasts and infiltration of mononuclear cells into the interstitial space. Immunohistochemistry stain and reverse transcription-polymerase chain reaction for rabies virus failed to demonstrate the organism in the lung tissue. Strong expression of inducible nitric oxide synthase (iNOS was detected in the alveolar macrophages. An immunologic mechanism with iNOS expression in the absence of direct invasion of the organism may participate in the pathogenesis of ARDS associated with rabies.

  17. Autoimmune neurological syndromes associated limbic encephalitis and paraneoplastic cerebellar degeneration.

    Science.gov (United States)

    Ayas, Zeynep Özözen; Kotan, Dilcan; Aras, Yeşim Güzey

    2016-10-06

    Autoimmune neurological syndrome is a group of disorders caused by cancer affecting nervous system by different immunological mechanisms. In this study, we aim to study the clinical symptoms, cerebrospinal fluid (CSF) findings, autoantibody tests, computed tomography (CT), magnetic resonance imaging (MRI) signs and treatment outcome of patients with autoimmune syndromes. In this study, 7 patients (4 male, 3 female) diagnosed with autoimmune neurological syndrome were retrospectively examined. Five of patients were diagnosed with limbic encephalitis, two of them were paraneoplastic cerebellar degeneration. Confusion and seizure were the most seen symptoms. Two patients had psychiatric disturbances (28,5%) followed by seizure. Headache was seen in 2 patients (% 28,5), disartria in 1 patient (% 14,2), and gait disorder in 2 patients (28,5%). The duration of symptoms was 46 (3-150) days on average. CSF abnormalities were detected in 2 patients. CT and MRI of the brain was available in all patients. Five patients had involvement of mesiotemporal region, two patients had diffuse cerebellar atrophy. One of patients had anti-GABAR B1 positivity. Tumors were detected in 2 patients while investigation for paraneoplasia screening. Remission is only possible with the detection and treatment of the malignancy. Early diagnosis and treatment are of paramount importance. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  18. MRI imaging of posterior reversible encephalopathy syndrome associated with pregnancy

    Directory of Open Access Journals (Sweden)

    Noha Mohamed AbdelMaboud Ibrahim

    2014-06-01

    Conclusion: The diagnosis of PRES has important therapeutic and prognostic value. The use of diffusion-weighted imaging and ADC maps allows an earlier and clearer differentiation of cytotoxic and vasogenic edema, which can predict the development of infarction.

  19. Pigment dispersion syndrome associated with spontaneous subluxation of crystalline lens

    Directory of Open Access Journals (Sweden)

    Vikas Veerwal

    2017-01-01

    Full Text Available Pigment dispersion syndrome (PDS is an ocular condition characterized by a dispersion of iris pigment throughout the eye. This pigment is deposited in a characteristic manner on the corneal endothelium as Krukenberg's spindle, anterior surface of the iris, in the trabecular meshwork, on the lens and zonule and occasionally on the anterior hyaloid face. Even with deposition of pigment on zonular fibers, no zonular weakness, or zonular dehiscence has been reported in these cases. We report a unique case of PDS with bilateral spontaneous subluxation of crystalline lens. With characteristic findings of pigment distribution in both his eyes, the patient had concave iris configuration with heavily pigmented trabecular meshwork confirming the diagnosis of PDS. The patient had bilateral 180° temporal subluxation of crystalline lens in both his eyes. The usual cause of lens subluxation such as Marfan's Syndrome and Ehler's Danlos Syndrome was ruled out. The patient underwent right eye followed by left eye intracapsular cataract extraction with ab-interno technique with postoperative best-corrected visual acuity (BCVA of 6/9 in both eyes. Spontaneous subluxation of crystalline lens in isolated PDS is not known to occur and has been reported by means of this case. We recommend a thorough assessment of zonular status in all cases of PDS.

  20. Nephrotic syndrome associated with metastatic thymoma treated with chemotherapy.

    Science.gov (United States)

    Yoo, Shin Hye; Kim, Hyean-Ji; Kim, Jeong-Han; Lee, Gyeong-Won; Lee, Jeong Hee; Kim, Se Hyun; Kim, Ji-Won; Kim, Jin Won; Lee, Jeong-Ok; Kim, Yu Jung; Lee, Keun-Wook; Kim, Jee Hyun; Bang, Soo-Mee; Lee, Jong Seok

    2017-01-01

    Nephropathy with concurrent invasive thymoma is a type of paraneoplastic syndrome. We report a 32-year-old female with nephrotic syndrome that was first diagnosed along with invasive thymoma and treated by means of cisplatin-based chemotherapy for the thymoma. The patient initially presented with dyspnea and generalized edema. Chest radiography and computed tomography scans revealed right pleural effusion and a mass in the right middle lung field, which were confirmed by a percutaneous lung biopsy as metastatic invasive thymoma. Severe hypoalbuminemia, heavy proteinuria, hyponatremia, and hypercholesterolemia were features of the nephrotic syndrome. A kidney needle biopsy suggested focal segmental glomerulosclerosis. All of the symptoms of nephrotic syndrome were resolved simultaneously during the first 2 cycles of chemotherapy. The patient was on regular follow-up with no specific treatment for nephrotic syndrome and underwent successful resection of the left pleura and anterior thymoma. The patient has shown no evidence of recurrence for 2 years. We conclude that chemotherapy for invasive thymoma is an effective treatment for nephrotic syndrome accompanying the thymoma.

  1. Thymoma with immunodeficiency/Good syndrome associated with myasthenia gravis.

    Science.gov (United States)

    Takai, Shunsuke; Tagawa, Asako; Ogawa, Tomoko; Kato, Hiroyuki; Saito, Noriko; Okada, Shinya

    2017-05-27

    Good syndrome is a rare condition in which thymoma is associated with hypogammaglobulinemia; it is characterized by repeated respiratory or systemic infections caused by bacteria, viruses, and fungi, as well as with various autoimmune disorders such as pure red cell aplasia. A 65-year-old woman was admitted to our hospital with ptosis and abdominal muscle weakness. Based on the presence of anti-acetylcholine receptor (Ach-R) antibodies, she was diagnosed with myasthenia gravis (MG). At that time, invasive thymoma of Masaoka stage IVa was also detected. Regression of thymoma and clinical remission of MG was achieved by chemotherapy followed by high-dose corticosteroid. However, several months later, the patient started developing repeated bacterial respiratory tract infections, cytomegalovirus infections, and esophageal and systemic candidiasis. Laboratory tests revealed a marked decrease of serum gamma-globulin levels (IgG 586 mg/dl, IgA 32 mg/dl, IgM 29 mg/dl) and severe reduction in the B cells ratio, as well as a decrease in the CD4+CD25+T cell to CD4+CD25-T cell ratio indicative of deregulation of CD4+T cell activation. These results suggested that the patient impaired humoral and cell-mediated immune responses. We continued the treatment with antibiotics and regular immunoglobulin supplementation through intravenous injections. Although autoimmune disorders are often observed in Good syndrome, the association with MG is quite rare. The case report is followed by the review of literature.

  2. Parry-Romberg Syndrome Associated with Localized Scleroderma

    Directory of Open Access Journals (Sweden)

    Jelena Maletic

    2010-06-01

    Full Text Available Parry-Romberg syndrome is a rare neurocutaneous disorder of unknown origin. It is characterized by progressive facial hemiatrophy and frequently overlaps with a condition known as linear scleroderma ‘en coup de sabre’. Neurological involvement is frequently described in these patients, including migraine, facial pain and epilepsy, which represent the commonest neurological conditions, sometimes associated with brain abnormalities ipsilaterally to the skin lesions. We present a case of Parry-Romberg syndrome with neurological involvement in a patient with diagnosed localized scleroderma (morphea.

  3. Parry-Romberg Syndrome Associated with Localized Scleroderma

    Science.gov (United States)

    Maletic, Jelena; Tsirka, Vassiliki; Ioannides, Panos; Karacostas, Dimitrios; Taskos, Nikolaos

    2010-01-01

    Parry-Romberg syndrome is a rare neurocutaneous disorder of unknown origin. It is characterized by progressive facial hemiatrophy and frequently overlaps with a condition known as linear scleroderma ‘en coup de sabre’. Neurological involvement is frequently described in these patients, including migraine, facial pain and epilepsy, which represent the commonest neurological conditions, sometimes associated with brain abnormalities ipsilaterally to the skin lesions. We present a case of Parry-Romberg syndrome with neurological involvement in a patient with diagnosed localized scleroderma (morphea). PMID:20671858

  4. Idiopathic hypereosinophilic syndrome associated with rheumatoid arthritis A case report

    Directory of Open Access Journals (Sweden)

    P. Quattrocchi

    2011-09-01

    Full Text Available The idiopathic hypereosinophilic sindrome (HES is a disease characterized by persistent blood eosinophilia (> 1500 eosinophils/mm3 > 6 months-in absence of other ethiologies for eosinophilia (parasitic, allergic, immunological or malignant diseases-associated with multiple organ involvement (heart, lung, central nervous system, skin, bone marrow, gastrointestinal tract. Reports on rheumatologic manifestations in patients with HES are very rare. In the case we report a typical rheumatoid arthritis developed in a 58-year-old woman with HES treated with glucocorticoids. Because of the marked glucocorticoids side effects shown by the patient(cushingoid habitus, hyperglycemia, we stopped this treatment and replaced it at first by methotrexate and later by cyclosporin, both of them associated with sulfasalazine. These drugs revealed very efficacious both on articular pathology and on the clinical and laboratory manifestations of HES. These data suggest that common pathogenetic mechanisms are likely acting in rheumatoid arthritis and idiopathic hypereosinophilic syndrome.

  5. Pseudo-Meig's Syndrome associated with huge Uterine Leiomyoma ...

    African Journals Online (AJOL)

    Background: Pseudo-Meigs syndrome consists of pleural effusion, ascites, and benign tumors of the ovary other than fibromas. These benign tumors include the tumors of fallopian tube or uterus, mature teratomas, struma ovarii, and ovarian leiomyomas. In a postmenopausal woman presence of complex pelvic mass, ...

  6. Osteogenesis imperfecta/lobstein syndrome associated with dentinogenesis imperfecta.

    Science.gov (United States)

    Lingaraju, Naresh; Nagarathna, P J; Vijayalakshmi, R; Sheshadri, P

    2013-01-01

    Osteogenesis imperfecta is a collagen related disorder characterized by increased bone fragility and low bone mass. The important oral finding in osteogenesis imperfect is the presence of dentinogenesis imperfecta. This article presents a case of osteogenesis imperfecta (type IV B) with dentinogenesis imperfecta where a 7-year-old girl had opalacent primary teeth associated with severe bone deformity, scoliosis, barrel shaped rib cage, and short stature. The clinical, radiographic ad histologic features are reviewed along with management aspects.

  7. Loose anagen hair syndrome associated with colobomas and dysmorphic features

    DEFF Research Database (Denmark)

    Hansen, Lars Kjaersgård; Brandrup, Flemming; Clemmensen, Ole

    2004-01-01

    Loose anagen hair syndrome is an uncommon congenital disorder. It may occur in association with other syndromes and dysmorphic features. We report a girl who fulfilled the diagnostic criteria for this syndrome as proposed by Tosti (Arch Dermatol 2002, 138: 521-522). She also had several other ano...

  8. Malformação da transição crânio-vertebral como causa de síndrome do ângulo ponto-cerebelar: relato de dois casos Cerebello-pontine angle syndrome associated with cranio-vertebral malformation: report of two cases

    Directory of Open Access Journals (Sweden)

    Amauri Batista da Silva

    1972-03-01

    Full Text Available Os autores relatam dois casos de síndrome do ângulo ponto-cerebelar nos quais não havia neoplasia nessa região. Ambos os pacientes apresentavam uma associação de impressão basilar com síndrome de Arnold-Chiari. Após cirurgia descompressiva de fossa posterior os casos evoluiram favoravelmente com a diminuição progressiva dos sintomas neurológicos, restando apenas, no segundo paciente, certo grau de paralisia e de espasmo facial, à direita. São feitas considerações em tôrno das causas mais freqüentes da síndrome do ângulo ponto-cerebelar, bem como acêrca dos quadros clínicos mais usualmente encontrados em doentes que apresentam malformações da transição crânio-vertebral. Finalmente, os autores tecem breves considerações a respeito do possível mecanismo lesionai do VIII, do V e do VII nervos cranianos nos dois casos relatados.Two cases of cerebelo-pontine angle syndrome associated with basilar impression and Arnold-Chiari malformation are reported. In both cases neuroradiological studies and surgical exploration failled to demonstrate any space occupying lesion. After surgery the patients progressively recovered disapearing the neurological symptoms except for a slaight hemifacial spasm that remained in case 2. Clinical signs present in cerebello-pontine angle lesions and the occipito-cervical malformations are discussed. A possible mechanism to explain the association of both conditions is discussed.

  9. Small-Engine Research Laboratory (SERL)

    Data.gov (United States)

    Federal Laboratory Consortium — Description: The Small-Engine Research Laboratory (SERL) is a facility designed to conduct experimental small-scale propulsion and power generation systems research....

  10. Network for Early Onset Cystic Kidney Diseases-A Comprehensive Multidisciplinary Approach to Hereditary Cystic Kidney Diseases in Childhood.

    Science.gov (United States)

    König, Jens Christian; Titieni, Andrea; Konrad, Martin

    2018-01-01

    Hereditary cystic kidney diseases comprise a complex group of genetic disorders representing one of the most common causes of end-stage renal failure in childhood. The main representatives are autosomal recessive polycystic kidney disease, nephronophthisis, Bardet-Biedl syndrome, and hepatocyte nuclear factor-1beta nephropathy. Within the last years, genetic efforts have brought tremendous progress for the molecular understanding of hereditary cystic kidney diseases identifying more than 70 genes. Yet, genetic heterogeneity, phenotypic variability, a lack of reliable genotype-phenotype correlations and the absence of disease-specific biomarkers remain major challenges for physicians treating children with cystic kidney diseases. To tackle these challenges comprehensive scientific approaches are urgently needed that match the ongoing "revolution" in genetics and molecular biology with an improved efficacy of clinical data collection. Network for early onset cystic kidney diseases (NEOCYST) is a multidisciplinary, multicenter collaborative combining a detailed collection of clinical data with translational scientific approaches addressing the genetic, molecular, and functional background of hereditary cystic kidney diseases. Consisting of seven work packages, including an international registry as well as a biobank, NEOCYST is not only dedicated to current scientific questions, but also provides a platform for longitudinal clinical surveillance and provides precious sources for high-quality research projects and future clinical trials. Funded by the German Federal Government, the NEOCYST collaborative started in February 2016. Here, we would like to introduce the rationale, design, and objectives of the network followed by a short overview on the current state of progress.

  11. Prenatal Diagnosis of Isolated Fetal Hydrocolpos Secondary to Congenital Imperforate Hymen

    Directory of Open Access Journals (Sweden)

    Jenn-Jhy Tseng

    2008-06-01

    Full Text Available A 32-year-old primigravida was referred to our hospital at 36 weeks of gestation with a fetal pelvic mass. Ultrasonography showed the fluid-filled area to be a 9 × 4 × 5-cm pear-shaped retrovesical mass with a funnel-shaped blind pouch at the distal end of the fetal vagina. Marked left hydronephrosis resulting from mass compression was also detected. Fetal magnetic resonance imaging further defined a pelvic lesion extending cephalically into the abdomen and caudally into the vagina. Membranal protrusion of the introitus was clearly identified. Therefore, the diagnosis of congenital imperforate hymen with hydrocolpos was established. At 38 weeks of gestation, a 2,966-g female infant was delivered vaginally with good Apgar scores. Physical examination of the neonate revealed a bulging membrane covering the vaginal opening. The presence of syndromic disorders (McKusick-Kaufman, Ellis-van Creveld or Bardet-Biedl syndromes, genitourinary and anorectal anomalies were excluded. The karyotype was 46, XX. A hymenotomy was performed on the second day of life. The infant recovered fully after hymenotomy.

  12. Unraveling the genetics of human obesity.

    Directory of Open Access Journals (Sweden)

    David M Mutch

    2006-12-01

    Full Text Available The use of modern molecular biology tools in deciphering the perturbed biochemistry and physiology underlying the obese state has proven invaluable. Identifying the hypothalamic leptin/melanocortin pathway as critical in many cases of monogenic obesity has permitted targeted, hypothesis-driven experiments to be performed, and has implicated new candidates as causative for previously uncharacterized clinical cases of obesity. Meanwhile, the effects of mutations in the melanocortin-4 receptor gene, for which the obese phenotype varies in the degree of severity among individuals, are now thought to be influenced by one's environmental surroundings. Molecular approaches have revealed that syndromes (Prader-Willi and Bardet-Biedl previously assumed to be controlled by a single gene are, conversely, regulated by multiple elements. Finally, the application of comprehensive profiling technologies coupled with creative statistical analyses has revealed that interactions between genetic and environmental factors are responsible for the common obesity currently challenging many Westernized societies. As such, an improved understanding of the different "types" of obesity not only permits the development of potential therapies, but also proposes novel and often unexpected directions in deciphering the dysfunctional state of obesity.

  13. Identification and characterization of a novel allele of Caenorhabditis elegans bbs-7.

    Directory of Open Access Journals (Sweden)

    Kara Braunreiter

    Full Text Available Primary cilia play a role in the sensation of and response to the surrounding environment. Caenorhabditis elegans (C. elegans have primary cilia only on the distal tips of some dendrites. In order to better understand the relationship between receptor localization to cilia, cilia structure and cilia function, we have characterized a mutation originally identified in a forward genetic screen for mutants with defective PKD-2 ciliary localization. Through behavioral assays and examination of the structure of cilia in the cil-5 (my13 mutant animals, we have found that my13 disrupts not only receptor localization, but also some cilia-mediated sensory behaviors and cilia structural integrity. We have identified the my13 lesion and found that it is a missense mutation in bbs-7, an ortholog of human BBS-7, a gene known to affect human cilia and to be involved in Bardet-Biedl syndrome. Finally, we show that bbs-7(my13 also affects the glia cells which support the cilia.

  14. The eye as a window to rare endocrine disorders

    Science.gov (United States)

    Chopra, Rupali; Chander, Ashish; Jacob, Jubbin J.

    2012-01-01

    The human eye, as an organ, can offer critical clues to the diagnosis of various systemic illnesses. Ocular changes are common in various endocrine disorders such as diabetes mellitus and Graves’ disease. However there exist a large number of lesser known endocrine disorders where ocular involvement is significant. Awareness of these associations is the first step in the diagnosis and management of these complex patients. The rare syndromes involving the pituitary hypothalamic axis with significant ocular involvement include Septo-optic dysplasia, Kallman's syndrome, and Empty Sella syndrome all affecting the optic nerve at the optic chiasa. The syndromes involving the thyroid and parathyroid glands that have ocular manifestations and are rare include Mc Cune Albright syndrome wherein optic nerve decompression may occur due to fibrous dysplasia, primary hyperparathyroidism that may present as red eye due to scleritis and Ascher syndrome wherein ptosis occurs. Allgrove's syndrome, Cushing's disease, and Addison's disease are the rare endocrine syndromes discussed involving the adrenals and eye. Ocular involvement is also seen in gonadal syndromes such as Bardet Biedl, Turner's, Rothmund's, and Klinefelter's syndrome. This review also highlights the ocular manifestation of miscellaneous syndromes such as Werner's, Cockayne's, Wolfram's, Kearns Sayre's, and Autoimmune polyendocrine syndrome. The knowledge of these relatively uncommon endocrine disorders and their ocular manifestations will help an endocrinologist reach a diagnosis and will alert an ophthalmologist to seek specialty consultation of an endocrinologist when encountered with such cases. PMID:22629495

  15. Hippocampal and cortical primary cilia are required for aversive memory in mice.

    Directory of Open Access Journals (Sweden)

    Nicolas F Berbari

    Full Text Available It has been known for decades that neurons throughout the brain possess solitary, immotile, microtubule based appendages called primary cilia. Only recently have studies tried to address the functions of these cilia and our current understanding remains poor. To determine if neuronal cilia have a role in behavior we specifically disrupted ciliogenesis in the cortex and hippocampus of mice through conditional deletion of the Intraflagellar Transport 88 (Ift88 gene. The effects on learning and memory were analyzed using both Morris Water Maze and fear conditioning paradigms. In comparison to wild type controls, cilia mutants displayed deficits in aversive learning and memory and novel object recognition. Furthermore, hippocampal neurons from mutants displayed an altered paired-pulse response, suggesting that loss of IFT88 can alter synaptic properties. A variety of other behavioral tests showed no significant differences between conditional cilia mutants and controls. This type of conditional allele approach could be used to distinguish which behavioral features of ciliopathies arise due to defects in neural development and which result from altered cell physiology. Ultimately, this could lead to an improved understanding of the basis for the cognitive deficits associated with human cilia disorders such as Bardet-Biedl syndrome, and possibly more common ailments including depression and schizophrenia.

  16. Autophagy promotes primary ciliogenesis by removing OFD1 from centriolar satellites.

    Science.gov (United States)

    Tang, Zaiming; Lin, Mary Grace; Stowe, Timothy Richard; Chen, She; Zhu, Muyuan; Stearns, Tim; Franco, Brunella; Zhong, Qing

    2013-10-10

    The primary cilium is a microtubule-based organelle that functions in sensory and signalling pathways. Defects in ciliogenesis can lead to a group of genetic syndromes known as ciliopathies. However, the regulatory mechanisms of primary ciliogenesis in normal and cancer cells are incompletely understood. Here we demonstrate that autophagic degradation of a ciliopathy protein, OFD1 (oral-facial-digital syndrome 1), at centriolar satellites promotes primary cilium biogenesis. Autophagy is a catabolic pathway in which cytosol, damaged organelles and protein aggregates are engulfed in autophagosomes and delivered to lysosomes for destruction. We show that the population of OFD1 at the centriolar satellites is rapidly degraded by autophagy upon serum starvation. In autophagy-deficient Atg5 or Atg3 null mouse embryonic fibroblasts, OFD1 accumulates at centriolar satellites, leading to fewer and shorter primary cilia and a defective recruitment of BBS4 (Bardet-Biedl syndrome 4) to cilia. These defects are fully rescued by OFD1 partial knockdown that reduces the population of OFD1 at centriolar satellites. More strikingly, OFD1 depletion at centriolar satellites promotes cilia formation in both cycling cells and transformed breast cancer MCF7 cells that normally do not form cilia. This work reveals that removal of OFD1 by autophagy at centriolar satellites represents a general mechanism to promote ciliogenesis in mammalian cells. These findings define a newly recognized role of autophagy in organelle biogenesis.

  17. Diabetic ketoacidosis associated with tacrolimus in solid organ transplant recipients

    International Nuclear Information System (INIS)

    Maqsood, M.Q.; Rabbani, M.; Habib, M.; Saleem, T.

    2011-01-01

    Diabetic ketoacidosis in patients receiving tacrolimus in the post-transplant setting is rare. We describe two such cases in solid-organ transplant recipients. The first patient, a 17-year-old male, presented with severe diabetic ketoacidosis and was managed with intravenous fluids and insulin infusion. He was a known case of Laurence-Moon-Bardet-Biedl syndrome and had received a renal transplant 2 years ago and was receiving tacrolimus since then. Although diabetic ketoacidosis resolved in 24 hours, large doses of subcutaneous insulin (unto 130 units per day) were needed to keep serum glucose within the normal range. Substitution of tacrolimus with cyclosporine obviated the need for insulin or oral hypoglycaemics. The second patient, a 55-year-old woman, presented with a history of polyuria for 3 days. She had received a hepatic transplant 2 years ago and tacrolimus was being used since then. Mild diabetic ketoacidosis was managed with fluid resuscitation and subcutaneous insulin. Her insulin requirement after an uneventful recovery has been 54 - 70 units per day. Clinicians should be cognizant of the possibility of hyperglycaemic crisis presenting as sudden onset of diabetic ketoacidosis in patients receiving tacrolimus. Use of an alternative calcineurin inhibitor may provide a safer solution to minimize future morbidity in such patients. (author)

  18. Obesity in Childhood and Adolescence, Genetic Factors.

    Science.gov (United States)

    Kostovski, Marko; Tasic, Velibor; Laban, Nevena; Polenakovic, Momir; Danilovski, Dragan; Gucev, Zoran

    2017-12-01

    Obesity and excess weight are a pandemic phenomenon in the modern world. Childhood and adolescent obesity often ends up in obesity in adults. The costs of obesity and its consequences are staggering for any society, crippling for countries in development. Childhood obesity is also widespread in Macedonia. Metabolic syndrome, dyslipidemia and carbohydrate intolerance are found in significant numbers. Parents and grandparents are often obese. Some of the children are either dysmorphic, or slightly retarded. We have already described patients with Prader-Willi syndrome, Bardet-Biedl syndrome or WAGR syndrome. A genetic screening for mutations in monogenic obesity in children with early, rapid-onset or severe obesity, severe hyperphagia, hypogonadism, intestinal dysfunction, hypopigmentation of hair and skin, postprandial hypoglycaemia, diabetes insipidus, abnormal leptin level and coexistence of lean and obese siblings in the family discovers many genetic forms of obesity. There are about 30 monogenic forms of obesity. In addition, obesity is different in ethnic groups, and the types of monogenic obesity differ. In brief, an increasing number of genes and genetic mechanisms in children continue to be discovered. This sheds new light on the molecular mechanisms of obesity and potentially gives a target for new forms of treatment.

  19. Functional genomics of the cilium, a sensory organelle.

    Science.gov (United States)

    Blacque, Oliver E; Perens, Elliot A; Boroevich, Keith A; Inglis, Peter N; Li, Chunmei; Warner, Adam; Khattra, Jaswinder; Holt, Rob A; Ou, Guangshuo; Mah, Allan K; McKay, Sheldon J; Huang, Peter; Swoboda, Peter; Jones, Steve J M; Marra, Marco A; Baillie, David L; Moerman, Donald G; Shaham, Shai; Leroux, Michel R

    2005-05-24

    Cilia and flagella play important roles in many physiological processes, including cell and fluid movement, sensory perception, and development. The biogenesis and maintenance of cilia depend on intraflagellar transport (IFT), a motility process that operates bidirectionally along the ciliary axoneme. Disruption in IFT and cilia function causes several human disorders, including polycystic kidneys, retinal dystrophy, neurosensory impairment, and Bardet-Biedl syndrome (BBS). To uncover new ciliary components, including IFT proteins, we compared C. elegans ciliated neuronal and nonciliated cells through serial analysis of gene expression (SAGE) and screened for genes potentially regulated by the ciliogenic transcription factor, DAF-19. Using these complementary approaches, we identified numerous candidate ciliary genes and confirmed the ciliated-cell-specific expression of 14 novel genes. One of these, C27H5.7a, encodes a ciliary protein that undergoes IFT. As with other IFT proteins, its ciliary localization and transport is disrupted by mutations in IFT and bbs genes. Furthermore, we demonstrate that the ciliary structural defect of C. elegans dyf-13(mn396) mutants is caused by a mutation in C27H5.7a. Together, our findings help define a ciliary transcriptome and suggest that DYF-13, an evolutionarily conserved protein, is a novel core IFT component required for cilia function.

  20. Small Business Social Responsibility Communication

    DEFF Research Database (Denmark)

    Morsing, Mette; Spence, Laura J.

    2015-01-01

    Purpose: Corporate social responsibility communication by small and medium sized enterprises is theorized to form the concept of Small Business Social Responsibility (SBSR) Communication. Design/methodology/approach: This is a conceptual paper that draws on Foucault’s theory of governmentality...... to conceptualization of SBSR communication as we develop a framework which suggests a spectrum of disciplining effects of social responsibility in relation to implicit and explicit CSR. We analyse these from the perspectives of surveillance, examination and normalization. Research limitations...... of conduct and CSR reporting (i.e. CSR as a “regime of truth”, Foucault) and an internally prescribed ethos of a personal philosophy of ethics and norms such as family identity and employee values, concern for local community and legacy of the company (i.e. CSR as personally held values). We contribute...

  1. Going global - growing small businesses

    International Nuclear Information System (INIS)

    Anderson, D.R.

    1994-01-01

    The Going Global Energy Steering Committee was established to help small and medium-sized Canadian enterprises to compete in the electrical power industry in the Asia-Pacific region primarily, but also in Eastern Europe and Latin America. The aim is to provide market intelligence, and help with forming consortia for financing. A small to medium-sized business can be defined as one with 50 to 500 employees. Big businesses no longer have the same competitive advantages that they once had, because automated systems can make short production runs just as cost-effective as long ones, and because computerization, automation and rising productivity mean that fewer workers are required than formerly

  2. Small Business Innovations (Fiber Optics)

    Science.gov (United States)

    1991-01-01

    Foster-Miller, Inc. Waltham, MA developed the In-Situ Fiber Optic Polymer Reaction Monitor which could lead to higher yields and lower costs in complex composite manufacturing. The monitor, developed under a Small Business Innovation Research (SBIR) contract with Langley Research Center, uses an infrared, fiber optic sensor to track the molecular vibrational characteristics of a composite part while it is being cured. It is the first analytical system capable of directly measuring the chemistry of advanced composite materials.

  3. Small Business Innovations (Robotic Wrist)

    Science.gov (United States)

    1991-01-01

    Under a Langley Research Center Small Business Innovation Research (SBIR) contract, Ross-Hime Designs, Inc. Minneapolis, MN, developed the Omni-Wrist actuator, which has a 25-pound capacity, 180 degrees of pitch/yaw, and 360 degrees of roll. Company literature calls it "the first successful singularity-free high-precision (robotic) wrist." Applications include spray painting, sealing, ultrasonic testing, welding and a variety of nuclear industry, aerospace and military uses.

  4. Tumors of the small intestine

    International Nuclear Information System (INIS)

    Alonso Gamboa, Tatiana

    2013-01-01

    Differential diagnoses are performed to establish the cause of chronic abdominal pain in patients. Histological types are considered in patients with primary tumors of unknown origin. Benign and malignant neoplasms are described, including methods of diagnosis and treatment. Clinical manifestations are cited. Early and accurate diagnoses are important for an acceptable outcome in patients with malignant small bowel tumors. Recurrence is provoked many deaths, suggesting the importance of adjuvant chemotherapy [es

  5. Neutronic design of small reactors

    International Nuclear Information System (INIS)

    Chabert, L.; Bonaccorsi, T.; Boyard, M.; Lefevre, E.; Lamoine, L.; Piela, J.

    2010-01-01

    Small reactors design is one of the main activities of AREVA TA. At the time, AREVA TA main projects are oriented towards research reactors and reactors for military naval propulsion. Due to differences in the physics and performances to meet, each kind of small reactor leads to specific modelling needs. Many computing tools have been developed in order to successfully carry out these projects. These schemes are mainly based on the use of TRIPOLI, MCNP, APOLLO2 and CRONOS2 codes. In that framework, a multi-purpose pre/post processing tool named CHARM is being developed by AREVA NP in partnership with AREVA TA in order to integrate small reactors specification. CHARM is used to elaborate APOLLO2 input data while various dedicated tools are used to automatically generate TRIPOLI and MNCP input data. These 3D numerical models need a very accurate spatial description to perform specific calculations. As an example, for the JHR design, after calculating 3D burn up by APOLLO2/MOC models, the data is fed back into a TRIPOLI model used for safety analyses. This paper presents our methodology for the small core design and 3 examples: 1) The calculation scheme for the JHR (Jules Horowitz Reactor) neutronic studies. These design studies are a recent illustration of combined use of both deterministic and probabilistic codes, 2) The use of CHARM, with the modelling of a JHR core. The purpose of CHARM- V2, based on Open Cascade Technology, is to provide a pre/post processing tool for APOLLO2/MOC, TRIPOLI4 and MCNP solvers, 3) The depletion Monte Carlo calculation of a MTR core. (author)

  6. Small Business Innovations (Integrated Database)

    Science.gov (United States)

    1992-01-01

    Because of the diversity of NASA's information systems, it was necessary to develop DAVID as a central database management system. Under a Small Business Innovation Research (SBIR) grant, Ken Wanderman and Associates, Inc. designed software tools enabling scientists to interface with DAVID and commercial database management systems, as well as artificial intelligence programs. The software has been installed at a number of data centers and is commercially available.

  7. Electrocatalytic activation of small molecules

    OpenAIRE

    Liu, YuPing

    2017-01-01

    Electrochemsitry and electrocatalysis are useful techniques for energy conversion and energy storage applications. In this project, the electrocatalytic activation of small molecules, H₂O, methanol and ethanol, and CO₂, has been studied as potential methods for energy storage and conversion. A hexaniobate Lindqvist ion assisted Co and Ni nanostructure deposition method has been developed. Efficient catalytic activity towards water oxidation has been observed with high TOF values obtained ...

  8. Small Business and International Entrepreneurship

    OpenAIRE

    Radchenko, Anastasiia

    2017-01-01

    Small and medium-sized enterprises together with start-ups are highly important in the nowadays economic environment. Such companies contribute raise of employment, stimulate the economic growth and innovation development. However, not all start-ups can survive more than five-year period. Support from government and private investors can help to overcome such problems. Moreover, SMEs internationalization and global functioning can support businesses during the tough times. The goal of the Mas...

  9. Tropical birds take small risks

    OpenAIRE

    Anders Pape Møller; Wei Liang

    2013-01-01

    The life history of tropical birds differs from that of their temperate counterparts by late start of reproduction, small clutch sizes, and high rates of adult survival. Thus, tropical species should have greater residual reproductive value than temperate species. Therefore, tropical birds can be predicted to take smaller risks than closely related temperate birds in order not to jeopardize their prospects of survival, which is the single most important component of fitness, and which is grea...

  10. Small Body Hopper Mobility Concepts

    Science.gov (United States)

    Howe, A. Scott; Gernhardt, Michael L.; Lee, Dave E.; Crues, E. Zack; Dexter, Dan E.; Abercromby, Andrew F. J.; Chappell, Steve P.; Nguyen, Hung T.

    2015-01-01

    A propellant-saving hopper mobility system was studied that could help facilitate the exploration of small bodies such as Phobos for long-duration human missions. The NASA Evolvable Mars Campaign (EMC) has proposed a mission to the moons of Mars as a transitional step for eventual Mars surface exploration. While a Mars transit habitat would be parked in High-Mars Orbit (HMO), crew members would visit the surface of Phobos multiple times for up to 14 days duration (up to 50 days at a time with logistics support). This paper describes a small body surface mobility concept that is capable of transporting a small, two-person Pressurized Exploration Vehicle (PEV) cabin to various sites of interest in the low-gravity environment. Using stored kinetic energy between bounces, a propellant-saving hopper mobility system can release the energy to vector the vehicle away from the surface in a specified direction. Alternatively, the stored energy can be retained for later use while the vehicle is stationary in respect to the surface. The hopper actuation was modeled using a variety of launch velocities, and the hopper mobility was evaluated using NASA Exploration Systems Simulations (NExSyS) for transit between surface sites of interest. A hopper system with linear electromagnetic motors and mechanical spring actuators coupled with Control Moment Gyroscope (CMG) for attitude control will use renewable electrical power, resulting in a significant propellant savings.

  11. Evolution of small prokaryotic genomes

    Directory of Open Access Journals (Sweden)

    David José Martínez-Cano

    2015-01-01

    Full Text Available As revealed by genome sequencing, the biology of prokaryotes with reduced genomes is strikingly diverse. These include free-living prokaryotes with ~800 genes as well as endosymbiotic bacteria with as few as ~140 genes. Comparative genomics is revealing the evolutionary mechanisms that led to these small genomes. In the case of free-living prokaryotes, natural selection directly favored genome reduction, while in the case of endosymbiotic prokaryotes neutral processes played a more prominent role. However, new experimental data suggest that selective processes may be at operation as well for endosymbiotic prokaryotes at least during the first stages of genome reduction. Endosymbiotic prokaryotes have evolved diverse strategies for living with reduced gene sets inside a host-defined medium. These include utilization of host-encoded functions (some of them coded by genes acquired by gene transfer from the endosymbiont and/or other bacteria; metabolic complementation between co-symbionts; and forming consortiums with other bacteria within the host. Recent genome sequencing projects of intracellular mutualistic bacteria showed that previously believed universal evolutionary trends like reduced G+C content and conservation of genome synteny are not always present in highly reduced genomes. Finally, the simplified molecular machinery of some of these organisms with small genomes may be used to aid in the design of artificial minimal cells. Here we review recent genomic discoveries of the biology of prokaryotes endowed with small gene sets and discuss the evolutionary mechanisms that have been proposed to explain their peculiar nature.

  12. Turbulent diffusion of small particles

    Energy Technology Data Exchange (ETDEWEB)

    Margolin, L.G.

    1977-11-01

    The diffusion of small, spherical, rigid particles suspended in an incompressible turbulent fluid, but not interacting with each other, was studied. As a stochastic process, the turbulent fluid velocity field is assumed to be homogeneous, isotropic and stationary. Assuming the Stokes regime, a particle of equation of motion is used which includes only the effects of Stokes drag and a virtual mass force and an exact solution is found for the particle velocity correlation function, for all times and initial conditions, in terms of a fluid velocity correlation function measured along the motion of the particle. This shows that for times larger than a certain time scale, the particle velocity correlation becomes stationary. The effect of small shears in the fluid velocity was considered, under the additional restrictions of a certain high frequency regime for the turbulence. The shears convected past the particle much faster than the growth of the boundary layer. New force terms due to the presence of such shears are calculated and incorporated into the equation of motion. A perturbation solution to this equation is constructed, and the resultant particle velocity correlation function and diffusion coefficient are calculated. To lowest order, the particle diffusivity is found to be unaltered by the presence of small mean flow shears. The last model treated is one in which particles traverse a turbulent fluid with a large mean velocity. Among other restrictions, linearized form drag is assumed. The diffusion coefficient for such particles was calculated, and found to be much smaller than the passive scalar diffusion coefficient. This agrees within 5 percent with the experimental results of Snyder and Lumley.

  13. Turbulent diffusion of small particles

    International Nuclear Information System (INIS)

    Margolin, L.G.

    1977-11-01

    The diffusion of small, spherical, rigid particles suspended in an incompressible turbulent fluid, but not interacting with each other, was studied. As a stochastic process, the turbulent fluid velocity field is assumed to be homogeneous, isotropic and stationary. Assuming the Stokes regime, a particle of equation of motion is used which includes only the effects of Stokes drag and a virtual mass force and an exact solution is found for the particle velocity correlation function, for all times and initial conditions, in terms of a fluid velocity correlation function measured along the motion of the particle. This shows that for times larger than a certain time scale, the particle velocity correlation becomes stationary. The effect of small shears in the fluid velocity was considered, under the additional restrictions of a certain high frequency regime for the turbulence. The shears convected past the particle much faster than the growth of the boundary layer. New force terms due to the presence of such shears are calculated and incorporated into the equation of motion. A perturbation solution to this equation is constructed, and the resultant particle velocity correlation function and diffusion coefficient are calculated. To lowest order, the particle diffusivity is found to be unaltered by the presence of small mean flow shears. The last model treated is one in which particles traverse a turbulent fluid with a large mean velocity. Among other restrictions, linearized form drag is assumed. The diffusion coefficient for such particles was calculated, and found to be much smaller than the passive scalar diffusion coefficient. This agrees within 5 percent with the experimental results of Snyder and Lumley

  14. Japanese Small Type Coastal Whaling

    Directory of Open Access Journals (Sweden)

    Sue Fisher

    2016-07-01

    Full Text Available 2016 marks the 70th anniversary of the International Convention for the Regulation of Whaling (ICRW as well as the 30th anniversary of the International Whaling Commission’s (IWC moratorium on commercial whaling. It also marks three decades of effort by Japan to overturn this ban. Its strategy to circumvent the moratorium by issuing permits to kill protected whales for scientific research is famous—even the subject of a 2014 lawsuit at the International Court of Justice. Less well known is Japan’s strategy to overturn the ban by persuading the Commission to authorise a category of commercial whaling known as Small Type Coastal Whaling (STCW that is conducted on minke and other small whales in Japanese waters but has never been regulated, or even formally recognised, by the IWC. For three decades Japan has sought STCW catch limits for four communities which it claims are still suffering distress as a result of the moratorium. While the Commission has rejected each proposal, mainly citing concerns that the commercial nature and purpose of STCW violates the moratorium, Japan has persisted, exhibiting great flexibility in its approach. Its tactics changed significantly in 2014; it no longer denied (or defended the commerciality of the hunt, but argued that it is irrelevant since it sought only a small exemption to the moratorium which would remain intact for all other populations. This is a perspective on Japan’s evolving STCW strategy and the risk that lifting, or modifying, the moratorium would pose to the conservation of whales.

  15. Small animal models of xenotransplantation.

    Science.gov (United States)

    Wang, Hao

    2012-01-01

    Organ transplantation has become a successful and acceptable treatment for end-stage organ failure. Such success has allowed transplant patients to resume a normal lifestyle. The demands for transplantation have been steadily increasing, as more patients and new diseases are being deemed eligible for treatment via transplantation. However, it is clear that human organs will never meet the increasing demand of transplantation. Therefore, scientists must continue to pursue alternative therapies and explore new treatments to meet the growing demand for the limited number of organs available. Transplanting organs from animals into humans (xenotransplantation) is one such therapy. The observed enthusiasm for xenotransplantation, irrespective of the severe shortage of human organs and tissues available for transplantation, can be said to stem from at least two factors. First, there is the possibility that animal organs and tissues might be less susceptible than those of humans to the recurrence of disease processes. Second, a xenograft might be used as a vehicle for introducing novel genes or biochemical processes which could be of therapeutic value for the transplant recipient.To date, millions of lives have been saved by organ transplantation. These remarkable achievements would have been impossible without experimental transplantation research in animal models. Presently, more than 95% of organ transplantation research projects are carried out using rodents, such as rats and mice. The key factor to ensure the success of these experiments lies in state-of-the art experimental surgery. Small animal models offer unique advantages for the mechanistic study of xenotransplantation rejection. Currently, multiple models have been developed for investigating the different stages of immunological barriers in xenotransplantation. In this chapter, we describe six valuable small animal models that have been used in xenotransplantation research. The methodology for the small animal

  16. SMALL VESICLES, BIG VEHICLES: EXOSOMES.

    Directory of Open Access Journals (Sweden)

    Saiz-Lopez P

    2016-09-01

    Full Text Available Exosomes are small membranous vesicles released by different cell types. Since their discovery, they have evolved from being considered simple vehicles for the liberation of cellular wastes, to become one of the most promising fields in the area of biomedical research, and more specifically in oncology, since the different malignant tumors release exosomes to all biological fluids, being involved in various functions of the neoplastic process. At present, it is possible to study these vesicles by minimally invasive techniques in patients, which approach us to obtain a more detailed diagnosis and prognosis, as well as to the discovery of new antitumoral therapies

  17. Small signal microwave amplifier design

    CERN Document Server

    Grosch, Theodore

    2000-01-01

    This book explains techniques and examples for designing stable amplifiers for high-frequency applications in which the signal is small and the amplifier circuit is linear. An in-depth discussion of linear network theory provides the foundation needed to develop actual designs. Examples throughout the book will show you how to apply the knowledge gained in each chapter leading to the complex design of low noise amplifiers. Many exercises at the end of each chapter will help students to practice their skills. The solutions to these design problems are available in an accompanying solutions book

  18. Native Small Airways Secrete Bicarbonate

    OpenAIRE

    Shamsuddin, A. K. M.; Quinton, Paul M.

    2014-01-01

    Since the discovery of Cl− impermeability in cystic fibrosis (CF) and the cloning of the responsible channel, CF pathology has been widely attributed to a defect in epithelial Cl− transport. However, loss of bicarbonate (HCO3−) transport also plays a major, possibly more critical role in CF pathogenesis. Even though HCO3− transport is severely affected in the native pancreas, liver, and intestines in CF, we know very little about HCO3− secretion in small airways, the principle site of morbidi...

  19. ATFSR: a small torsatron reactor

    International Nuclear Information System (INIS)

    Houlberg, W.A.; Lacatski, J.T.; Uckan, N.A.

    1985-01-01

    A small (average minor radius anti a approx. = 1 m), moderate-aspect-ratio torsatron reactor based on the Advanced Toroidal Facility (ATF) is proposed as a starting point for improved stellarator reactor designs. The major limitation of the compact size is the lack of space under the helical coils for the blanket and shield. Neoclassical confinement models for helically trapped particles show that a large electric potential (radial electric field) is necessary to achieve ignition in a device of this size, although high-Q operation is still attainable with more modest potentials

  20. Small angle scattering and polymers

    Energy Technology Data Exchange (ETDEWEB)

    Cotton, J.P. [Laboratoire Leon Brillouin (LLB) - Centre d`Etudes de Saclay, 91 - Gif-sur-Yvette (France)

    1996-12-31

    The determination of polymer structure is a problem of interest for both statistical physics and industrial applications. The average polymer structure is defined. Then, it is shown why small angle scattering, associated with isotopic substitution, is very well suited to the measurement of the chain conformation. The corresponding example is the old, but pedagogic, measurement of the chain form factor in the polymer melt. The powerful contrast variation method is illustrated by a recent determination of the concentration profile of a polymer interface. (author) 12 figs., 48 refs.

  1. Small intestinal cytochromes P450.

    Science.gov (United States)

    Kaminsky, L S; Fasco, M J

    1991-01-01

    Small intestinal cytochromes P450 (P450) provide the principal, initial source of biotransformation of ingested xenobiotics. The consequences of such biotransformation are detoxification by facilitating excretion, or toxification by bioactivation. P450s occur at highest concentrations in the duodenum, near the pylorus, and at decreasing concentrations distally--being lowest in the ileum. Highest concentrations occur from midvillus to villous tip, with little or none occurring in the crypts of Lieberkuehn. Microsomal P4503A, 2C8-10, and 2D6 forms have been identified in human small intestine, and P450s 2B1, possibly 2B2, 2A1, and 3A1/2 were located in endoplasmic reticulum of rodent small intestine, while P4502B4 has been purified to electrophoretic homogeneity from rabbit intestine. Some evidence indicates a differential distribution of P450 forms along the length of the small intestine and even along the villus. Rat intestinal P450s are inducible by xenobiotics--with phenobarbital (PB) inducing P4502B1, 3-methylcholanthrene (3-MC) inducing P4501A1, and dexamethasone inducing two forms of P4503A. Induction is most effectively achieved by oral administration of the agents, and is rapid--aryl hydrocarbon hydroxylase (AHH) was increased within 1 h of administration of, for example, 3-MC. AHH, 7-ethoxycoumarin O-deethylase (ECOD), and 7-ethoxyresorufin O-deethylase (EROD) have been used most frequently as substrates to characterize intestinal P450s. Dietary factors affect intestinal P450s markedly--iron restriction rapidly decreased intestinal P450 to beneath detectable values; selenium deficiency acted similarly but was less effective; Brussels sprouts increased intestinal AHH activity 9.8-fold, ECOD activity 3.2-fold, and P450 1.9-fold; fried meat and dietary fat significantly increased intestinal EROD activity; a vitamin A-deficient diet increased, and a vitamin A-rich diet decreased intestinal P450 activities; and excess cholesterol in the diet increased intestinal

  2. Small Bowel Review: Part I

    Directory of Open Access Journals (Sweden)

    ABR Thomson

    2000-01-01

    Full Text Available In the past year, there have been many advances in the area of small bowel physiology and pathology. More than 1500 papers were assessed in preparation for this review. Some were selected and reviewed, with a particular focus on presenting clinically useful information for the practising gastroenterologist. Relevant review articles have been highlighted, and important clinical learning points have been stressed. The topics are varied in scope, and wherever possible show a logical progression from basic physiology to pathophysiology to clinical disorders and management.

  3. Legibility of small icons with color combinations in small displays.

    Science.gov (United States)

    Huang, Shih-Miao

    2007-02-01

    The present study explored the effects of figure/ground color combinations on legibility of small icons. This experiment used a 2 (icon size) x 3 (icon hue) x 3 (icon saturation) x 3 (background hue) x 3 (background saturation) x2 (luminance contrast) factorial design. Legibility was investigated by response time of subicons with different factors t hat affect discrimination. 11 undergraduate students from the National Formosa University in Taiwan were recruited to participate in the experiment. Their mean age was 21.8 yr. (SD=3.3). Analysis also showed that Icon Size x Luminance Contrast, Background Hue x Luminance Contrast, and Background Saturation x Luminance Contrast significantly affected response time. The results suggested that the higher Luminance Contrast could promote icon legibility but not the icon chromaticity or background chromaticity.

  4. Thrust sensing for small UAVs

    Science.gov (United States)

    Marchman, Christopher Scott

    Unmanned aerial vehicles (UAVs) have become prevalent in both military and civilian applications. UAVs have many size categories from large-scale aircraft to micro air vehicles. The performance, health, and efficiency for UAVs of smaller sizes can be difficult to assess and few associated instrumentation systems have been developed. Thrust measurements on the ground can characterize systems especially when combined with simultaneous motor power measurements. This thesis demonstrates the use of strain measurements to measure the thrust produced by motor/propeller combinations for such small UAVs. A full-bridge Wheatstone circuit and electrical resistance strain gauges were used in conjunction with constant-stress cantilever beams for static tests and dynamic wind tunnel tests. An associated instrumentation module monitored power from the electric motor. Monitoring the thrust data over time can provide insights into optimal propeller and motor selection and early detection of problems such as component failure. The approach provides a system for laboratory or field measurements that can be scaled for a wide range of small UAVs.

  5. Investigation of small ruptured aneurysms

    International Nuclear Information System (INIS)

    Abiko, Masaru; Ikawa, Fusao; Obayashi, Naohiko; Mitsuhara, Takafumi; Nosaka, Ryou; Inagawa, Tetsuji

    2008-01-01

    Subarachnoid hemorrhage (SAH) patients with ruptured aneurysms smaller than 5 mm in diameter are sometimes encountoied at our hospital. However, the operative indication for unruptured aneurysm is over 5 mm according to the JAPANESE SOCIETY FOR DETECTION OF ASYMPTOMATIC BRAIN DISEASES. We therefore analyzed the characteristics of ruptured aneurysms smaller than 5 mm in diameter. A retrospective review of all SAH patients using digital subtraction angiography (DSA) between April 1999 and March 2004 was conducted. We compared 201 SAH patients with DSA (group A) and 61 with aneurysms smaller than 5 mm (group B). Age, gender, familial history, hypertension, and location of the ruptured aneurysm were examined. In males younger than 40 years old, anterior communicating artery/distal anterior cerebral artery (Acom/DACA) aneurysms were significantly correlated with group B rather than group A. Familial history and hypertension were not found to be characteristic in group B in this study. Small unruptured aneurysms may have a risk of rupture especially in young males with Acom/DACA aneurysms. Further investigations on small aneurysms are needed. (author)

  6. Small gauge vitrectomy: Recent update

    Science.gov (United States)

    Khanduja, Sumeet; Kakkar, Ashish; Majumdar, Saptrishi; Vohra, Rajpal; Garg, Satpal

    2013-01-01

    Small gauge vitrectomy, also known as minimally invasive vitreous surgery (MIVS), is a classic example of progress in biomedical engineering. Disparity in conjunctival and scleral wound location and reduction in wound diameter are its core principles. Fluidic changes include increased pressure head loss with consequent reduction in infusional flow rate and use of higher aspiration vacuum at the cutter port. Increase An increase in port open/port closed time maintains an adequate rate of vitreous removal. High Intensity Discharge (HID) lamps maintain adequate illumination in spite of a decrease in the number of fiberoptic fibers. The advantages of MIVS are, a shorter surgical time, minimal conjunctival damage, and early postoperative recovery. Most complications are centered on wound stability and risk of postoperative hypotony, endophthalmitis, and port site retinal break formation. MIVS is suited in most cases, however, it can cause dehiscence of recent cataract wounds. Retraction of the infusion cannula in the suprachoroidal space may occur in eyes with scleral thinning. As a lot has been published and discussed about sutureless vitrectomy a review of this subject is necessary. A PubMed search was performed in December 2011 with terms small gauge vitrectomy, 23-gauge vitrectomy, 25-gauge vitrectomy, and 27 gauge vitrectomy, which were revised in August 2012. There were no restrictions on the date of publication but it was restricted to articles in English or other languages, if there abstracts were available in English. PMID:23772118

  7. Small gauge vitrectomy: Recent update

    Directory of Open Access Journals (Sweden)

    Sumeet Khanduja

    2013-01-01

    Full Text Available Small gauge vitrectomy, also known as minimally invasive vitreous surgery (MIVS, is a classic example of progress in biomedical engineering. Disparity in conjunctival and scleral wound location and reduction in wound diameter are its core principles. Fluidic changes include increased pressure head loss with consequent reduction in infusional flow rate and use of higher aspiration vacuum at the cutter port. Increase An increase in port open/port closed time maintains an adequate rate of vitreous removal. High Intensity Discharge (HID lamps maintain adequate illumination in spite of a decrease in the number of fiberoptic fibers. The advantages of MIVS are, a shorter surgical time, minimal conjunctival damage, and early postoperative recovery. Most complications are centered on wound stability and risk of postoperative hypotony, endophthalmitis, and port site retinal break formation. MIVS is suited in most cases, however, it can cause dehiscence of recent cataract wounds. Retraction of the infusion cannula in the suprachoroidal space may occur in eyes with scleral thinning. As a lot has been published and discussed about sutureless vitrectomy a review of this subject is necessary. A PubMed search was performed in December 2011 with terms small gauge vitrectomy, 23-gauge vitrectomy, 25-gauge vitrectomy, and 27 gauge vitrectomy, which were revised in August 2012. There were no restrictions on the date of publication but it was restricted to articles in English or other languages, if there abstracts were available in English.

  8. Small nuclear reactors for desalination

    International Nuclear Information System (INIS)

    Goldsmith, K.

    1978-01-01

    Small nuclear reactors are considered to have an output of not more than 400MW thermal. Since they can produce steam at much higher conditions than needed by the brine heater of a multi-flash desalination unit, it may be economically advantageous to use small reactors for a dual-purpose installation of appropriate size, producing both electricity and desalted water, rather than for a single-purpose desalination plant only. Different combinations of dual-purpose arrangements are possible depending principally on the ratio of electricity to water output required. The costs of the installation as well as of the products are critically dependent on this ratio. For minimum investment costs, the components of the dual-purpose installation should be of a standardised design based on normal commercial power plant practice. This then imposes some restrictions on the plant arrangement but, on the other hand, it facilitates selection of the components. Depending on the electricity to water ratio to be achieved, the conventional part of the installation - essentially the turbines - will form a combination of back-pressure and condensing machines. Each ratio will probably lead to an optimum combination. In the economic evaluation of this arrangement, a distinction must be made between single-purpose and dual-purpose installations. The relationship between output and unit costs of electricity and water will be different for the two cases, but the relation can be expressed in general terms to provide guidelines for selecting the best dimensions for the plant. (author)

  9. Reactions of small molecular systems

    Energy Technology Data Exchange (ETDEWEB)

    Wittig, C. [Univ. of Southern California, Los Angeles, CA (United States)

    1993-12-01

    This DOE program remains focused on small molecular systems relevant to combustion. Though a number of experimental approaches and machines are available for this research, the authors` activities are centered around the high-n Rydberg time-of-flight (HRTOF) apparatus in this laboratory. One student and one postdoc carry out experiments with this machine and also engage in small intra-group collaborations involving shared equipment. This past year was more productive than the previous two, due to the uninterrupted operation of the HRTOF apparatus. Results were obtained with CH{sub 3}OH, CH{sub 3}SH, Rg-HX complexes, HCOOH, and their deuterated analogs where appropriate. One paper is in print, three have been accepted for publication, and one is under review. Many preliminary results that augur well for the future were obtained with other systems such as HNO{sub 3}, HBr-HI complexes, toluene, etc. Highlights from the past year are presented below that display some of the features of this program.

  10. Revascularização distal com laqueação arterial (DRIL: Tratamento de eleição para a isquémia em síndrome de roubo sintomático associado a FAV Distal revascularization with interval ligation (DRIL: First line treatment for symptomatic steel syndrome associated to AVF

    Directory of Open Access Journals (Sweden)

    Pedro Amorim

    2012-03-01

    Full Text Available As complicações relacionadas com os acessos de hemodiálise (HD são o principal motivo de internamento dos doentes renais crónicos. O tratamento cirúrgico do síndrome de roubo associado a isquemia grave e crítica é mandatório e urgente. A única técnica cirúrgica que garante segurança nos resultados no que diz respeito à resolução da isquemia com preservação do acesso vascular é o DRIL. Os autores apresentam o caso de uma doente, 78 anos, com insuficiência renal crónica hipertensiva, fístula úmero-cefálica e isquemia crítica da mão esquerda. O quadro clínico, com 13 meses de evolução, foi refractário à terapêutica médica pelo que foi realizado um DRIL. Verificou-se remissão imediata da dor e melhoria progressiva das lesões tróficas dos dedos da mão com preservação do acesso. Os autores defendem a realização do DRIL como primeira linha no tratamento destes doentes.Complications related to vascular access for hemodialysis (HD are the main reason for hospitalization of chronic renal patients. Surgical treatment of steal syndrome associated with severe and critical limb ischemia is mandatory and urgent. The only surgical technique that ensures safety in the results with regard to the resolution of ischemia and preservation of vascular access is DRIL. The authors present the case of a 78 year old patient with hypertensive chronic renal failure, humeral-cephalic fistula and critical ischemia of the left hand. The clinical picture, with 13 months of evolution, was refractory to medical therapy and so a DRIL was performed. There was immediate remission of pain and progressive improvement of ulcerations of the fingers. At the same time the access for HD was preserved and early punctured. The authors advocate the implementation of DRIL as first-line treatment in these patients.

  11. Discriminator with a small hysteresis

    International Nuclear Information System (INIS)

    Korol'ko, E.V.; Suvorov, V.V.

    1977-01-01

    The electric circuitry of a discriminator is described which comprises a current shaping circuit, a tunnel diode and a shunting circuit. The novel shunting circuit design ensures a small hysteresis and quick response of the system as a whole. The shunting circuit employs two n-p-n and p-n-p type transistors, their emitters being interconnected and the bases being coupled to voltage dividers. A diagram is presented to illustrate the function of the tunnel diode - shunting circuit combination. The discriminator has the following characteristics: hysteresis about 5%, response time about 15 nsec., attenuation factor about 3. The discriminator is useful for forming pulses having a duration equal to the threshold exceeding time

  12. Post accidental small breaks analysis

    International Nuclear Information System (INIS)

    Depond, G.; Gandrille, J.

    1980-04-01

    EDF ordered to FRAMATOME by 1977 to complete post accidental long term studies on 'First Contrat-Programme' reactors, in order to demonstrate the safety criteria long term compliance, to get information on NSSS behaviour and to improve the post accidental procedures. Convenient analytical models were needed and EDF and FRAMATOME respectively developped the AXEL and FRARELAP codes. The main results of these studies is that for the smallest breaks, it is possible to manually undertake cooling and pressure reducing actions by dumping the steam generators secondary side in order to meet the RHR operating specifications and perform long term cooling through this system. A specific small breaks procedure was written on this basis. The EDF and FRAMATOME codes are continuously improved; the results of a French set of separate effects experiments will be incorporated as well as integral system verification

  13. Disturbances in small bowel motility.

    LENUS (Irish Health Repository)

    Quigley, E M

    2012-02-03

    Recently, the small intestine has become the focus of investigation as a potential site of dysmotility in the irritable bowel syndrome (IBS). A number of motor abnormalities have been defined in some studies, and include \\'clustered\\' contractions, exaggerated post-prandial motor response and disturbances in intestinal transit. The significance of these findings remains unclear. The interpretation of available studies is complicated by differences in subject selection, the direct influence of certain symptoms, such as diarrhoea and constipation, and the interference of compounding factors, such as stress and psychopathology. Dysmotility could also reflect autonomic dysfunction, disturbed CNS control and the response to heightened visceral sensation or central perception. While motor abnormalities may not explain all symptoms in IBS, sensorimotor interactions may be important in symptom pathogenesis and deserve further study.

  14. Minimum Q Electrically Small Antennas

    DEFF Research Database (Denmark)

    Kim, O. S.

    2012-01-01

    Theoretically, the minimum radiation quality factor Q of an isolated resonance can be achieved in a spherical electrically small antenna by combining TM1m and TE1m spherical modes, provided that the stored energy in the antenna spherical volume is totally suppressed. Using closed-form expressions...... for the stored energies obtained through the vector spherical wave theory, it is shown that a magnetic-coated metal core reduces the internal stored energy of both TM1m and TE1m modes simultaneously, so that a self-resonant antenna with the Q approaching the fundamental minimum is created. Numerical results...... for a multiarm spherical helix antenna confirm the theoretical predictions. For example, a 4-arm spherical helix antenna with a magnetic-coated perfectly electrically conducting core (ka=0.254) exhibits the Q of 0.66 times the Chu lower bound, or 1.25 times the minimum Q....

  15. Small Business Innovations (Mass Microbalance)

    Science.gov (United States)

    1991-01-01

    Femtometrics of Costa Mesa, CA, developed the Model 200-1 SAW Mass Microbalance under a NASA Small Business Innovation Research (SBIR) contract with Langley Research Center. The product is described as "the next generation of aerosol mass microbalance technology," because a new type of sensor, the Surface Acoustic Wave (SAW) piezoelectric crystal, offers mass resolution two orders of magnitude greater than the Quartz Crystal Microbalance cascade impactor (QCM) (used at Langley since 1979 for collection and measurement of aerosol particles in the upper atmosphere). The Model 200-1 SAW Mass Microbalance, which provides a 400-fold increase in mass sensitivity per unit area over the QCM, can be used for real-time particle monitoring in clean rooms, measuring chemical vapors in very low concentrations, measuring target chemicals in the stratosphere and in industry as a toxic vapor monitor.

  16. Small Scale High Speed Turbomachinery

    Science.gov (United States)

    London, Adam P. (Inventor); Droppers, Lloyd J. (Inventor); Lehman, Matthew K. (Inventor); Mehra, Amitav (Inventor)

    2015-01-01

    A small scale, high speed turbomachine is described, as well as a process for manufacturing the turbomachine. The turbomachine is manufactured by diffusion bonding stacked sheets of metal foil, each of which has been pre-formed to correspond to a cross section of the turbomachine structure. The turbomachines include rotating elements as well as static structures. Using this process, turbomachines may be manufactured with rotating elements that have outer diameters of less than four inches in size, and/or blading heights of less than 0.1 inches. The rotating elements of the turbomachines are capable of rotating at speeds in excess of 150 feet per second. In addition, cooling features may be added internally to blading to facilitate cooling in high temperature operations.

  17. The Nothuesli small hydro project

    International Nuclear Information System (INIS)

    Balachandran, S.; Jorde, K.

    2008-01-01

    This report for the Swiss Federal Office of Energy (SFOE) discusses the project for a small 16-kilowatt hydro plant on the Gonzenbaechli stream in eastern Switzerland. The site, which was used even before 1860 for obtaining power from the stream is briefly described, as are the present remains of earlier installations. An old Francis turbine has been retrieved and could possibly be reused. Water-flow figures and fall-heights are noted. Design flows and residual water quantities required by legislation are noted and discussed, as are the geology and topology of the catchment area. The proposals for a new hydro-power plant are described, including the apparatus proposed with a power of 20 kVA. Environmental aspects are also discussed, as are the investment costs and the economic viability of the project. The paper is completed with a comprehensive appendix, including detailed cost estimates.

  18. Immunization against Small Ruminant Lentiviruses

    Directory of Open Access Journals (Sweden)

    Beatriz Amorena

    2013-08-01

    Full Text Available Multisystemic disease caused by Small Ruminant Lentiviruses (SRLV in sheep and goats leads to production losses, to the detriment of animal health and welfare. This, together with the lack of treatments, has triggered interest in exploring different strategies of immunization to control the widely spread SRLV infection and, also, to provide a useful model for HIV vaccines. These strategies involve inactivated whole virus, subunit vaccines, DNA encoding viral proteins in the presence or absence of plasmids encoding immunological adjuvants and naturally or artificially attenuated viruses. In this review, we revisit, comprehensively, the immunization strategies against SRLV and analyze this double edged tool individually, as it may contribute to either controlling or enhancing virus replication and/or disease.

  19. Cogeneration for small SAGD projects

    Energy Technology Data Exchange (ETDEWEB)

    Albion, Stuart [AMEC BDR Limited (United Kingdom)

    2011-07-01

    As many SAGD projects are being developed in remote locations, the supply of a steady source of power to them becomes an important question. Connecting these remote facilities to a grid can often be difficult and costly. This presentation, by AMEC BDR Limited, promotes the use of cogeneration in small SAGD projects. Cogeneration is the generation of two forms of energy from one fuel source. In this particular case, the energy forms would be electricity and heat. In many SAGD projects, a gas turbine system is used to generate the electricity, while a heat recovery system is utilized to generate steam. The use of cogeneration systems in SAGD projects, as opposed to using separate heat and electricity systems, has the potential to significantly reduce the amount of energy lost, the amount of emissions and power costs, in addition to ensuring that there is a reliable supply of steam and electricity.

  20. Small Molecule Fluoride Toxicity Agonists

    Science.gov (United States)

    Nelson1, James W.; Plummer, Mark S.; Blount, Kenneth F.; Ames, Tyler D.; Breaker, Ronald R.

    2015-01-01

    SUMMARY Fluoride is a ubiquitous anion that inhibits a wide variety of metabolic processes. Here we report the identification of a series of compounds that enhance fluoride toxicity in Escherichia coli and Streptococcus mutans. These molecules were isolated by using a high-throughput screen (HTS) for compounds that increase intracellular fluoride levels as determined via a fluoride riboswitch-reporter fusion construct. A series of derivatives were synthesized to examine structure-activity relationships, leading to the identification of compounds with improved activity. Thus, we demonstrate that small molecule fluoride toxicity agonists can be identified by HTS from existing chemical libraries by exploiting a natural fluoride riboswitch. In addition, our findings suggest that some molecules might be further optimized to function as binary antibacterial agents when combined with fluoride. PMID:25910244

  1. Holography in small bubble chambers

    International Nuclear Information System (INIS)

    Lecoq, P.

    1984-01-01

    This chapter reports on an experiment to determine the total charm cross section at different incident momenta using the small, heavy liquid bubble chamber HOBC. Holography in liquid hydrogen is also tested using the holographic lexan bubble chamber HOLEBC with the aim of preparing a future holographic experiment in hydrogen. The high intensity tests show that more than 100 incident tracks per hologram do not cause a dramatic effect on the picture quality. Hydrogen is more favorable than freon as the bubble growth is much slower in hydrogen. An advantage of holography is to have the maximum resolution in the full volume of the bubble chamber, which allows a gain in sensitivity by a factor of 10 compared to classical optics as 100 tracks per hologram look reasonable. Holograms are not more difficult to analyze than classical optics high-resolution pictures. The results show that holography is a very powerful technique which can be used in very high resolution particle physics experiments

  2. Small Molecule Organic Optoelectronic Devices

    Science.gov (United States)

    Bakken, Nathan

    Organic optoelectronics include a class of devices synthesized from carbon containing 'small molecule' thin films without long range order crystalline or polymer structure. Novel properties such as low modulus and flexibility as well as excellent device performance such as photon emission approaching 100% internal quantum efficiency have accelerated research in this area substantially. While optoelectronic organic light emitting devices have already realized commercial application, challenges to obtain extended lifetime for the high energy visible spectrum and the ability to reproduce natural white light with a simple architecture have limited the value of this technology for some display and lighting applications. In this research, novel materials discovered from a systematic analysis of empirical device data are shown to produce high quality white light through combination of monomer and excimer emission from a single molecule: platinum(II) bis(methyl-imidazolyl)toluene chloride (Pt-17). Illumination quality achieved Commission Internationale de L'Eclairage (CIE) chromaticity coordinates (x = 0.31, y = 0.38) and color rendering index (CRI) > 75. Further optimization of a device containing Pt-17 resulted in a maximum forward viewing power efficiency of 37.8 lm/W on a plain glass substrate. In addition, accelerated aging tests suggest high energy blue emission from a halogen-free cyclometalated platinum complex could demonstrate degradation rates comparable to known stable emitters. Finally, a buckling based metrology is applied to characterize the mechanical properties of small molecule organic thin films towards understanding the deposition kinetics responsible for an elastic modulus that is both temperature and thickness dependent. These results could contribute to the viability of organic electronic technology in potentially flexible display and lighting applications. The results also provide insight to organic film growth kinetics responsible for optical

  3. Surface heterogeneity of small asteroids

    Science.gov (United States)

    Sasaki, Sho

    A rubble pile model of asteroid origin would predict averaged rather homogeneous surface of an asteroid. Previous spacecraft observations (mostly S-type asteroids) did not show large color/albedo variation on the surface. Vesta would be exceptional since HST observation suggested that its surface should be heterogeneous due to the impact excavation of the interior. As for a young asteroid (832) Karin (age being 5Ma), Sasaki et al. (2004) detected variation of infrared spectra which could be explained by the difference of the space weathering degree. They discussed the possibility of the survival of the old surface. However, the variation was not confirmed by later observation (Chapman et al., 2007; Vernazza et al., 2007). Recent observation of a small (550m) asteroid Itokawa by Hayabusa spacecraft revealed that Itokawa is heterogeneous in color and albedo although the overall rocky structure is considered as a rubble pile (Saito et al., 2006). The color difference can be explained by the difference of weathering degree (Ishiguro et al., 2008). The heterogeneity could be explained by mass movement caused by rapid rotation from YORP effect (Scheeres et al., 2007) or seismic shaking (Sasaki, 2006). Probably small silicate asteroids without significant regolith could have heterogeneous in color and albedo. On large asteroids (˜ a few 10km), regolith reaccumulation should have covered the underlying heterogeneity. References: Chapman, C. R. et al (2007) Icarus, 191, 323-329 Ishiguro, M. et al. (2008) MAPS, in press. Saito, J. et al. (2006) Science, 312, 1341-1344 Sasaki, S. (2006) in Spacecraft Reconnaissance of Asteroid and Comet Interiors Sasaki, T. et al (2004) Astrophys. J. 615, L161-L164 Scheeres, D. J. (2007) Icarus 188, 425-429 Vernazza, P. et al. (2007) Icarus 191, 330-336.

  4. Geophysics of Small Planetary Bodies

    Science.gov (United States)

    Asphaug, Erik I.

    1998-01-01

    As a SETI Institute PI from 1996-1998, Erik Asphaug studied impact and tidal physics and other geophysical processes associated with small (low-gravity) planetary bodies. This work included: a numerical impact simulation linking basaltic achondrite meteorites to asteroid 4 Vesta (Asphaug 1997), which laid the groundwork for an ongoing study of Martian meteorite ejection; cratering and catastrophic evolution of small bodies (with implications for their internal structure; Asphaug et al. 1996); genesis of grooved and degraded terrains in response to impact; maturation of regolith (Asphaug et al. 1997a); and the variation of crater outcome with impact angle, speed, and target structure. Research of impacts into porous, layered and prefractured targets (Asphaug et al. 1997b, 1998a) showed how shape, rheology and structure dramatically affects sizes and velocities of ejecta, and the survivability and impact-modification of comets and asteroids (Asphaug et al. 1998a). As an affiliate of the Galileo SSI Team, the PI studied problems related to cratering, tectonics, and regolith evolution, including an estimate of the impactor flux around Jupiter and the effect of impact on local and regional tectonics (Asphaug et al. 1998b). Other research included tidal breakup modeling (Asphaug and Benz 1996; Schenk et al. 1996), which is leading to a general understanding of the role of tides in planetesimal evolution. As a Guest Computational Investigator for NASA's BPCC/ESS supercomputer testbed, helped graft SPH3D onto an existing tree code tuned for the massively parallel Cray T3E (Olson and Asphaug, in preparation), obtaining a factor xIO00 speedup in code execution time (on 512 cpus). Runs which once took months are now completed in hours.

  5. 75 FR 66411 - Small Entity Compliance Guide: Women-Owned Small Business Program

    Science.gov (United States)

    2010-10-28

    ... participation in the WOSB program, and is intended to help small businesses understand the regulation and how it... SMALL BUSINESS ADMINISTRATION Small Entity Compliance Guide: Women-Owned Small Business Program AGENCY: Small Business Administration. ACTION: Notice: Availability of Compliance Guide. SUMMARY: The...

  6. 75 FR 1296 - Small Business Size Regulations; 8(a) Business Development/Small Disadvantaged Business Status...

    Science.gov (United States)

    2010-01-11

    ... SMALL BUSINESS ADMINISTRATION 13 CFR Parts 121 and 124 Small Business Size Regulations; 8(a) Business Development/Small Disadvantaged Business Status Determinations AGENCY: U.S. Small Business Administration. ACTION: Notice of public meetings; request for comments. SUMMARY: The U.S. Small Business...

  7. Chemisorption and Reactions of Small Molecules on Small Gold Particles

    Directory of Open Access Journals (Sweden)

    Geoffrey C. Bond

    2012-02-01

    Full Text Available The activity of supported gold particles for a number of oxidations and hydrogenations starts to increase dramatically as the size falls below ~3 nm. This is accompanied by an increased propensity to chemisorption, especially of oxygen and hydrogen. The explanation for these phenomena has to be sought in kinetic analysis that connects catalytic activity with the strength and extent of chemisorption of the reactants, the latter depending on the electronic structure of the gold atoms constituting the active centre. Examination of the changes to the utilisation of electrons as particle size is decreased points to loss of metallic character at about 3 nm, as energy bands are replaced by levels, and a band gap appears. Detailed consideration of the Arrhenius parameters (E and ln A for CO oxidation points clearly to a step-change in activity at the point where metallic character is lost, as opposed to there being a monotonic dependence of rate on a physical property such as the fraction of atoms at corners or edges of particles. The deplorable scarcity of kinetic information on other reactions makes extension of this analysis difficult, but non-metallic behaviour is an unavoidable property of very small gold particles, and therefore cannot be ignored when seeking to explain their exceptional activity.

  8. Enabling Technology for Small Satellite Launch Project

    Data.gov (United States)

    National Aeronautics and Space Administration — Access to space for Small Satellites is enabled by the use of excess launch capacity on existing launch vehicles. A range of sizes, form factors and masses of small...

  9. 78 FR 59798 - Small Business Subcontracting: Correction

    Science.gov (United States)

    2013-09-30

    ... assignment of compliance responsibilities between contracting offices, small business offices, and program...)(1)(ii).'' List of Subjects in 13 CFR Part 125 Government contracting programs, Small business... Administrator for Government Contracting and Business Development. BILLING CODE 8025-01-M ...

  10. Lung cancer - non-small cell

    Science.gov (United States)

    Cancer - lung - non-small cell; Non-small cell lung cancer; NSCLC; Adenocarcinoma - lung; Squamous cell carcinoma - lung ... Smoking causes most cases (around 90%) of lung cancer. The risk depends on the number of cigarettes ...

  11. Small Proteins Can No Longer Be Ignored

    Science.gov (United States)

    Storz, Gisela; Wolf, Yuri I.; Ramamurthi, Kumaran S.

    2014-01-01

    Small proteins, here defined as proteins of 50 amino acids or less in the absence of processing, have traditionally been overlooked due to challenges in their annotation and biochemical detection. In the past several years however, increasing numbers of small proteins have been identified either through the realization that mutations in “intergenic” regions actually are within unannotated small protein genes, or through the discovery that some small, regulatory RNAs (sRNAs) encode small proteins. These insights together with comparative sequence analysis indicate that tens if not hundreds of small proteins are synthesized in a given organism. This review will summarize what has been learned about the functions of several of these bacterial small proteins, most of which act at the membrane, illustrating the astonishing range of processes in which the small proteins act and pointing to several general conclusions. Important questions for future studies of these overlooked proteins also will be discussed. PMID:24606146

  12. Cruise tourism development in a small destination

    OpenAIRE

    Karreman, Japke

    2013-01-01

    This master thesis looks at the development of cruise tourism in a small destination. Cruise tourism is a rapidly growing industry in both large and small destinations, but since small destinations are affected by cruise tourism development different than large destinations, this study focuses on a small destination, in this case Skjolden. Skjolden has a recent history of cruise tourism. Interviews are taken with residents who are involved with cruise tourism, to find out about th...

  13. 76 FR 8221 - Small Business Size Regulations; 8(a) Business Development/Small Disadvantaged Business Status...

    Science.gov (United States)

    2011-02-11

    ... Vol. 76 Friday, No. 29 February 11, 2011 Part VII Small Business Administration 13 CFR Parts 121 and 124 Small Business Size Regulations; 8(a) Business Development/Small Disadvantaged Business Status... Regulations#0;#0; [[Page 8222

  14. Myoglobin in pelagic small cetaceans.

    Science.gov (United States)

    Dolar, M L; Suarez, P; Ponganis, P J; Kooyman, G L

    1999-02-01

    Although myoglobin (Mb) is considered to contribute significantly to the oxygen and diving capacity of marine mammals, few data are available for cetaceans. Cetacean by-catch in the tuna driftnet fisheries in the Sulu Sea, Philippines, afforded the opportunity to examine Mb content and distribution, and to determine muscle mass composition, in Fraser's (Lagenodelphis hosei) and spinner (Stenella longirostris) dolphins and a pygmy killer whale (Feresa attenuata). Age was estimated by body length determination. Stomach contents were analyzed for the presence or absence of milk and solid foods. It was hypothesized (a) that Mb concentration ([Mb]) would be higher in Fraser's and spinner dolphins than in other small cetaceans because of the known mesopelagic distribution of their prey, (b) that [Mb] would vary among different muscles according to function during diving, and (c) that [Mb] would increase with age during development. The results were as follows. (1) Myoglobin concentrations of the longissimus muscle in adult Fraser's (6.8-7.2 g 100 g-1 muscle) and spinner (5-6 g 100 g-1 muscle) dolphins and in an immature pygmy killer whale (5.7 g 100 g-1 muscle) were higher than those reported previously for small cetaceans. (2) [Mb] varied significantly among the different muscle types in adult dolphins but not in calves; in adults, swimming muscles had significantly higher [Mb] than did non-swimming muscles, contained 82-86 % of total Mb, and constituted 75-80 % of total muscle mass. (3) Myoglobin concentrations in Fraser's and spinner dolphins increased with size and age and were 3-4 times greater in adults than in calves. The high Mb concentrations measured in the primary locomotory muscles of these pelagic dolphins are consistent with the known mesopelagic foraging behaviour of Fraser's and spinner dolphins and suggest that the pygmy killer whale is also a deep-diving species. The high Mb concentrations in epaxial, hypaxial and abdominal muscle groups also support

  15. Mutual Guarantee Institutions and Small Business Finance

    NARCIS (Netherlands)

    Columba, F.; Gambacorta, L.; Mistrulli, P.E.

    2009-01-01

    A large literature showed that small firms experience difficulties in accessing the credit market due to informational asymmetries; these may be mitigated by collateral or relationship lending, possibilities often precluded to small business. We investigate the effect on small business finance of an

  16. 27 CFR 19.912 - Small plants.

    Science.gov (United States)

    2010-04-01

    ... 27 Alcohol, Tobacco Products and Firearms 1 2010-04-01 2010-04-01 false Small plants. 19.912... OF THE TREASURY LIQUORS DISTILLED SPIRITS PLANTS Distilled Spirits For Fuel Use Permits § 19.912 Small plants. Persons wishing to establish a small plant shall apply for a permit as provided in this...

  17. The Ecological Psychology of the Small Town.

    Science.gov (United States)

    Miller, Duane I.

    1989-01-01

    Uses principles of ecological theory to predict architectural needs of a small town. Examines Barker's concept of undermanning which led to six hypotheses regarding small-town behavior and corresponding design recommendations. Concludes that psychology of small-town resident is unique because of special ecological demands placed on the people.…

  18. 7 CFR 201.42 - Small containers.

    Science.gov (United States)

    2010-01-01

    ... 7 Agriculture 3 2010-01-01 2010-01-01 false Small containers. 201.42 Section 201.42 Agriculture... REGULATIONS Sampling in the Administration of the Act § 201.42 Small containers. In sampling seed in small containers that it is not practical to sample as required in § 201.41, a portion of one unopened container or...

  19. Small-angle scattering, topography and radiography

    International Nuclear Information System (INIS)

    Schelten, J.

    1978-01-01

    A table is given showing scattering and imaging methods for X-rays and neutrons, followed, by a discussion of such topics as 1. Radiography 2. Topography 3. Small-angle scattering 3.1. The differential cross section 3.2. Comparison of X-ray and neutron small-angle scattering 3.3. Examples of small-angle scattering. (orig.) [de

  20. Understanding the statistics of small risks

    International Nuclear Information System (INIS)

    Siddall, E.

    1983-10-01

    Monte Carlo analyses are used to show what inferences can and cannot be drawn when either a very small number of accidents result from a considerable exposure or where a very small number of people, down to a single individual, are exposed to small added risks. The distinction between relative and absolute uncertainty is illustrated. No new statistical principles are involved

  1. Finding the "Right Staff" in Small Firms

    Science.gov (United States)

    Barrett, Rowena; Neeson, Robyn; Billington, Leo

    2007-01-01

    Purpose: The purpose of this paper is to explore what owners of small firms are looking for from new employees. The aim is to pursue this in light of the debate around formality and informality of small firm HRM, exploring the extent to which the small firms studied had formalized HRM practices. Design/methodology/approach: The data reported here…

  2. Oesophagostomosis, moniaziasis and trichuriasis of small ruminants ...

    African Journals Online (AJOL)

    For this study, the intestines (small and large intestines) were collected from 300 small ruminants (200 goats and 100 sheep) at necropsy and examined by the Hansen and Perry method. Out of the 300 small ruminants examined during the study period, the intestines revealed the presence of Oesophagostomum sp, ...

  3. 78 FR 77352 - Small Business Policy

    Science.gov (United States)

    2013-12-23

    ... 2700-AD97 Small Business Policy AGENCY: National Aeronautics and Space Administration. ACTION: Direct... information and citations that have changed in a regulation that establishes NASA's small business policy and... is also being amended to include a reference to NASA's general policy for small business programs and...

  4. Informal Learning of Women Small Business Owners

    Science.gov (United States)

    Sharafizad, Jalleh

    2018-01-01

    Purpose: The purpose of this paper is to investigate women small business owners' informal learning behaviour. There is limited qualitative research that examines women small business owners' learning process and this study aims to address this gap. The study was driven by the following research questions: "Do women small business owners…

  5. Neutron radiography using small cyclotrons

    International Nuclear Information System (INIS)

    Nishihara, Yoshiaki; Tarumi, Yuji; Ozaki, Masakazu; Kakihira, Shoji; Tazawa, Shuichi; Nakanii, Takehiko

    1995-01-01

    For neutron radiography, as a neutron source, a small cyclotron has been used in this facility, which was developed for the production of short life radioisotopes by Sumitomo Heavy Industries Ltd. Further, the development of the use of fast ion beam for industrial fields has been advanced, Neutron radiography was carried out earliest in 1983, and thereafter, the activation analysis using charged particles, the ion irradiation to semiconductor elements, the measurement of wear by using the nuclides produced with the cyclotron as the tracer and so on have been performed. The specification of this neutron radiography facility is shown. The method of irradiating proton beam to a beryllium target is adopted. The features of the moderator, the device for improving neutron flux, the collimators and so on are explained. The heightening of neutron characteristics by the improvement of the moderator is shown. Thermal neutrons have the large cross section of absorption or scattering in hydrogen and special elements, and show the transparent property in general metals. Accordingly, the have the merit that the photographing of good contrast can be done for the hydrocarbon materials in metallic enclosures. The examples of the application of thermal neutron radiography to space rockets, aircrafts, automobiles and others are shown. (K.I.)

  6. The small world of psychopathology.

    Directory of Open Access Journals (Sweden)

    Denny Borsboom

    Full Text Available BACKGROUND: Mental disorders are highly comorbid: people having one disorder are likely to have another as well. We explain empirical comorbidity patterns based on a network model of psychiatric symptoms, derived from an analysis of symptom overlap in the Diagnostic and Statistical Manual of Mental Disorders-IV (DSM-IV. PRINCIPAL FINDINGS: We show that a half of the symptoms in the DSM-IV network are connected, b the architecture of these connections conforms to a small world structure, featuring a high degree of clustering but a short average path length, and c distances between disorders in this structure predict empirical comorbidity rates. Network simulations of Major Depressive Episode and Generalized Anxiety Disorder show that the model faithfully reproduces empirical population statistics for these disorders. CONCLUSIONS: In the network model, mental disorders are inherently complex. This explains the limited successes of genetic, neuroscientific, and etiological approaches to unravel their causes. We outline a psychosystems approach to investigate the structure and dynamics of mental disorders.

  7. Electric propulsion for small satellites

    Science.gov (United States)

    Keidar, Michael; Zhuang, Taisen; Shashurin, Alexey; Teel, George; Chiu, Dereck; Lukas, Joseph; Haque, Samudra; Brieda, Lubos

    2015-01-01

    Propulsion is required for satellite motion in outer space. The displacement of a satellite in space, orbit transfer and its attitude control are the task of space propulsion, which is carried out by rocket engines. Electric propulsion uses electric energy to energize or accelerate the propellant. The electric propulsion, which uses electrical energy to accelerate propellant in the form of plasma, is known as plasma propulsion. Plasma propulsion utilizes the electric energy to first, ionize the propellant and then, deliver energy to the resulting plasma leading to plasma acceleration. Many types of plasma thrusters have been developed over last 50 years. The variety of these devices can be divided into three main categories dependent on the mechanism of acceleration: (i) electrothermal, (ii) electrostatic and (iii) electromagnetic. Recent trends in space exploration associate with the paradigm shift towards small and efficient satellites, or micro- and nano-satellites. A particular example of microthruster considered in this paper is the micro-cathode arc thruster (µCAT). The µCAT is based on vacuum arc discharge. Thrust is produced when the arc discharge erodes some of the cathode at high velocity and is accelerated out the nozzle by a Lorentz force. The thrust amount is controlled by varying the frequency of pulses with demonstrated range to date of 1-50 Hz producing thrust ranging from 1 µN to 0.05 mN.

  8. Public acceptance of small reactors

    International Nuclear Information System (INIS)

    McDougall, D.S.

    1997-01-01

    The success of any nuclear program requires acceptance by the local public and all levels of government involved in the decision to initiate a reactor program. Public acceptance of a nuclear energy source is a major challenge in successful initiation of a small reactor program. In AECL's experience, public acceptance will not be obtained until the public is convinced that the specific nuclear program is needed, safe and economic and environmental benefit to the community. The title of public acceptance is misleading. The objective of the program is a fully informed public. The program proponent cannot force public acceptance, which is beyond his control. He can, however, ensure that the public is informed. Once information has begun to flow to the public by various means as will be explained later, the proponent is responsible to ensure that the information that is provided by him and by others is accurate. Most importantly, and perhaps most difficult to accomplish, the proponent must develop a consultative process that allows the proponent and the public to agree on actions that are acceptable to the proponent and the community

  9. Small business development: who pays?

    Science.gov (United States)

    Hellinger, D

    1985-11-01

    This article traces the trends in the aid community towards income generation and small business projects in developing countries since the 1950s. 1 of the great weakness of the development assistance community in the area of income generation remains the lack of analysis of population differentiation in the communities in which they work. A very different approach to the problem of the informal sector and income generation is one that responds directly to the needs of poor communities. Institutions working within poor communities and supporting multi-sectoral development while strengthening local organizations are much better situated to respond to the diverse needs of the community than are single-sector organizations with only 1 skill or service to offer. To assist significant numbers of poor people, particularly women, grouping of some sort is essential. This is not to say that group enterprise development is an easy process. The intensive and costly organizational and management assistance required to prepare a poor group for credit must be weighed along with the potential for creating a basis of long-range change within the community. To work with individual family enterprizes in a community can also have important social and economic effects, although the long-range impact may be more limited. Today, more than ever, the poor face the challenge of creating new survival mechanisms within their communities. Development agencies and assistance programs have the responsibility to help rather than hinder this process.

  10. Small Aircraft Data Distribution System

    Science.gov (United States)

    Chazanoff, Seth L.; Dinardo, Steven J.

    2012-01-01

    The CARVE Small Aircraft Data Distribution System acquires the aircraft location and attitude data that is required by the various programs running on a distributed network. This system distributes the data it acquires to the data acquisition programs for inclusion in their data files. It uses UDP (User Datagram Protocol) to broadcast data over a LAN (Local Area Network) to any programs that might have a use for the data. The program is easily adaptable to acquire additional data and log that data to disk. The current version also drives displays using precision pitch and roll information to aid the pilot in maintaining a level-level attitude for radar/radiometer mapping beyond the degree available by flying visually or using a standard gyro-driven attitude indicator. The software is designed to acquire an array of data to help the mission manager make real-time decisions as to the effectiveness of the flight. This data is displayed for the mission manager and broadcast to the other experiments on the aircraft for inclusion in their data files. The program also drives real-time precision pitch and roll displays for the pilot and copilot to aid them in maintaining the desired attitude, when required, during data acquisition on mapping lines.

  11. Holographic gunsights for small arms

    Science.gov (United States)

    Tai, Anthony M.; Sieczka, Eric J.; Radler, Richard; Upatnieks, Juris

    1996-05-01

    Holographic gunsights were first demonstrated in the mid 1970s by researchers at the Environmental Research Institute of Michigan (ERIM) under contracts with the Air Force and the Army. The sights utilized He-Ne gas lasers and were designed for use with large weapons systems. With the advent of low cost visible laser diode, ERIM formed a new company, EOTech, to develop, manufacture and market a holographic gun sight for small arms. A hologram is used to reconstruct the image of a reticle pattern that appears at the target plane. Unlike red-dot sights, virtually any reticle pattern, 2D or 3D, can be formed. The design challenges include an opto-mechanical package that is compact, light weight and low cost which can withstand recoils up to 4,000 Gs and provide fine elevation/windage pointing adjustments, and optics that are aberration-free and stable over a wide temperature range. Manufacturing challenges include the mass production of high quality holographic optics at low cost and the precision alignment of the very low f/number optics.

  12. Neurological examination in small animals

    Directory of Open Access Journals (Sweden)

    Viktor Paluš

    2014-03-01

    Full Text Available This clinical review about the neurological examination in small animals describes the basics about the first steps of investigation when dealing with neurological patients. The knowledge of how to perform the neurological examination is important however more important is how to correctly interpret these performed tests. A step-by-step approach is mandatory and examiners should master the order and the style of performing these tests. Neurological conditions can be sometimes very distressing for owners and for pets that might not be the most cooperating. The role of a veterinary surgeon, as a professional, is therefore to collect the most relevant history, to examine a patient in a professional manner and to give to owners an educated opinion about the further treatment and prognosis. However neurological examinations might look challenging for many. But it is only the clinical application of neuroanatomy and neurophysiology to an every-day situation for practicing veterinarians and it does not require any specific in-to-depth knowledge. This clinical review is aimed not only to provide the information on how to perform the neurological examination but it is also aimed to appeal on veterinarians to challenge their daily routine and to start practicing on neurologically normal patients. This is the best and only way to differentiate between the normal and abnormal in a real situation.

  13. Helicity evolution at small x

    International Nuclear Information System (INIS)

    Kovchegov, Yuri V.; Pitonyak, Daniel; Sievert, Matthew D.

    2016-01-01

    We construct small-x evolution equations which can be used to calculate quark and anti-quark helicity TMDs and PDFs, along with the g 1 structure function. These evolution equations resum powers of α s ln 2  (1/x) in the polarization-dependent evolution along with the powers of α s ln (1/x) in the unpolarized evolution which includes saturation effects. The equations are written in an operator form in terms of polarization-dependent Wilson line-like operators. While the equations do not close in general, they become closed and self-contained systems of non-linear equations in the large-N c and large-N c   N f limits. As a cross-check, in the ladder approximation, our equations map onto the same ladder limit of the infrared evolution equations for the g 1 structure function derived previously by Bartels, Ermolaev and Ryskin http://dx.doi.org/10.1007/s002880050285.

  14. A small scale honey dehydrator.

    Science.gov (United States)

    Gill, R S; Hans, V S; Singh, Sukhmeet; Pal Singh, Parm; Dhaliwal, S S

    2015-10-01

    A small scale honey dehydrator has been designed, developed, and tested to reduce moisture content of honey below 17 %. Experiments have been conducted for honey dehydration by using drying air at ambient temperature, 30 and 40 °C and water at 35, 40 and 45 °C. In this dehydrator, hot water has been circulated in a water jacket around the honey container to heat honey. The heated honey has been pumped through a sieve to form honey streams through which drying air passes for moisture removal. The honey streams help in increasing the exposed surface area of honey in contact with drying air, thus resulting in faster dehydration of honey. The maximum drying rate per square meter area of honey exposed to drying air was found to be 197.0 g/h-m(2) corresponding to the drying air and water temperature of 40 and 45 °C respectively whereas it was found to be minimum (74.8 g/h-m(2)) corresponding to the drying air at ambient temperature (8-17 °C) and water at 35 °C. The energy cost of honey moisture content reduction from 25.2 to 16.4 % was Rs. 6.20 to Rs. 17.36 (US $ 0.10 to US $ 0.28 (One US $ = 62.00 Indian Rupee on February, 2014) per kilogram of honey.

  15. Vascular anatomy of strictured small bowel.

    Science.gov (United States)

    Mansard, Magnus Jayaraj; Rao, Upender; Pradeep, R; Sekaran, Anuradha; Rao, G V; Reddy, D N

    2011-01-01

    To investigate the role of ischemia in the pathogenesis of small bowel strictures. Vascular anatomy of 39 small bowel strictures was studied using modified Spalteholtz method. Ten normal small bowel segments were studied as controls. 71.8% of small bowel strictures showed block in the mesenteric vessels (p=0.008). Subset analysis of tuberculous strictures showed block in the mesenteric vessels in 70.8% of strictures (p=0.0098). Ischemia plays a significant role in the pathogenesis of small bowel strictures. Mesenteric vasculopathy has a significant association with tuberculous strictures of the intestine.

  16. Mastering Microsoft Windows Small Business Server 2008

    CERN Document Server

    Johnson, Steven

    2010-01-01

    A complete, winning approach to the number one small business solution. Do you have 75 or fewer users or devices on your small-business network? Find out how to integrate everything you need for your mini-enterprise with Microsoft's new Windows Server 2008 Small Business Server, a custom collection of server and management technologies designed to help small operations run smoothly without a giant IT department. This comprehensive guide shows you how to master all SBS components as well as handle integration with other Microsoft technologies.: Focuses on Windows Server 2008 Small Business Serv

  17. CT enteroclysis in small bowel Crohn's disease

    International Nuclear Information System (INIS)

    Kohli, Marc D.; Maglinte, Dean D.T.

    2009-01-01

    The diagnostic evaluation of small bowel Crohn's disease has changed dramatically over the last decade. The introduction of wireless capsule endoscopy, double balloon endoscopy and the introduction of newer therapeutic agents have changed the role of imaging in the small bowel. Additionally, advances in multidetector CT technology have further changed how radiologic investigations are utilized in the diagnosis and management of small bowel Crohn's disease. This article describes how we perform CT enteroclysis in the investigation of small bowel Crohn's disease and discusses the role of CT enteroclysis in the current management of small bowel Crohn's disease.

  18. A novel Direct Small World network model

    Directory of Open Access Journals (Sweden)

    LIN Tao

    2016-10-01

    Full Text Available There is a certain degree of redundancy and low efficiency of existing computer networks.This paper presents a novel Direct Small World network model in order to optimize networks.In this model,several nodes construct a regular network.Then,randomly choose and replot some nodes to generate Direct Small World network iteratively.There is no change in average distance and clustering coefficient.However,the network performance,such as hops,is improved.The experiments prove that compared to traditional small world network,the degree,average of degree centrality and average of closeness centrality are lower in Direct Small World network.This illustrates that the nodes in Direct Small World networks are closer than Watts-Strogatz small world network model.The Direct Small World can be used not only in the communication of the community information,but also in the research of epidemics.

  19. New developments in small reactors

    International Nuclear Information System (INIS)

    McDonnell, F.N.; Reed, A.

    1990-08-01

    During the fifty years since nuclear fission was discovered, nuclear energy has emerged to play an increasingly important role in meeting global energy needs. At the recent World Energy Conference in Montreal, 1989 September, experts agreed that nuclear power will continue to be an essential part of the future energy mix. The demand for economic and reliable energy sources is driven by the growth in the world's population and the essential role energy plays in industrial development. Global energy requirements, expected to double over the next 40 years, will seriously challenge suppliers in their ability to meet the demand. Ultimately, efficient energy utilization will become singularly important. Industrialization and economic development manifest themselves in urbanization. Urban dwellers consume significantly more energy per capita compared with their rural neighbours. Consequently, concentrated and environmentally acceptable energy sources, combined with efficient distribution systems, are now recognized as essential to meet urban energy demands. In considering the alternatives that will meet these requirements, nuclear energy qualifies as both a concentrated and environmentally benign source. Nuclear electricity generation is a mature technology that paves the way for other applications. If nuclear energy is to realize its full potential as a safe and cost-effective alternative to fossil fuels, applications beyond those that are currently being serviced by large, central nuclear power stations must be identified, and appropriately designed and sized reactors developed as an investment in the future. To meet this potential, new small reactor concepts are being developed to satisfy the expected energy demands, while also displaying characteristics that address current public concerns for providing minimal environmental impact. Concepts ranging in sized from 10 MW(t) to 1000 MW(t) are being pursued in a number of countries, including Canada, USA, UK, China, and

  20. Resectable pancreatic small cell carcinoma

    Directory of Open Access Journals (Sweden)

    Dana K. Andersen

    2011-03-01

    Full Text Available Primary pancreatic small cell carcinoma (SCC is rare, with just over 30 cases reported in the literature. Only 7 of these patients underwent surgical resection with a median survival of 6 months. Prognosis of SCC is therefore considered to be poor, and the role of adjuvant therapy is uncertain. Here we report two institutions’ experience with resectable pancreatic SCC. Six patients with pancreatic SCC treated at the Johns Hopkins Hospital (4 patients and the Mayo Clinic (2 patients were identified from prospectively collected pancreatic cancer databases and re-reviewed by pathology. All six patients underwent a pancreaticoduodenectomy. Clinicopathologic data were analyzed, and the literature on pancreatic SCC was reviewed. Median age at diagnosis was 50 years (range 27-60. All six tumors arose in the head of the pancreas. Median tumor size was 3 cm, and all cases had positive lymph nodes except for one patient who only had five nodes sampled. There were no perioperative deaths and three patients had at least one postoperative complication. All six patients received adjuvant therapy, five of whom were given combined modality treatment with radiation, cisplatin, and etoposide. Median survival was 20 months with a range of 9-173 months. The patient who lived for 9 months received chemotherapy only, while the patient who lived for 173 months was given chemoradiation with cisplatin and etoposide and represents the longest reported survival time from pancreatic SCC to date. Pancreatic SCC is an extremely rare form of cancer with a poor prognosis. Patients in this surgical series showed favorable survival rates when compared to prior reports of both resected and unresectable SCC. Cisplatin and etoposide appears to be the preferred chemotherapy regimen, although its efficacy remains uncertain, as does the role of combined modality treatment with radiation.

  1. What is cerebral small vessel disease?

    International Nuclear Information System (INIS)

    Onodera, Osamu

    2011-01-01

    An accumulating amount of evidence suggests that the white matter hyperintensities on T 2 weighted brain magnetic resonance imaging predict an increased risk of dementia and gait disturbance. This state has been proposed as cerebral small vessel disease, including leukoaraiosis, Binswanger's disease, lacunar stroke and cerebral microbleeds. However, the concept of cerebral small vessel disease is still obscure. To understand the cerebral small vessel disease, the precise structure and function of cerebral small vessels must be clarified. Cerebral small vessels include several different arteries which have different anatomical structures and functions. Important functions of the cerebral small vessels are blood-brain barrier and perivasucular drainage of interstitial fluid from the brain parenchyma. Cerebral capillaries and glial endfeet, take an important role for these functions. However, the previous pathological investigations on cerebral small vessels have focused on larger arteries than capillaries. Therefore little is known about the pathology of capillaries in small vessel disease. The recent discoveries of genes which cause the cerebral small vessel disease indicate that the cerebral small vessel diseases are caused by a distinct molecular mechanism. One of the pathological findings in hereditary cerebral small vessel disease is the loss of smooth muscle cells, which is an also well-recognized finding in sporadic cerebral small vessel disease. Since pericytes have similar character with the smooth muscle cells, the pericytes should be investigated in these disorders. In addition, the loss of smooth muscle cells may result in dysfunction of drainage of interstitial fluid from capillaries. The precise correlation between the loss of smooth muscle cells and white matter disease is still unknown. However, the function that is specific to cerebral small vessel may be associated with the pathogenesis of cerebral small vessel disease. (author)

  2. EFFECT OF MARKET ORIENTATION ON SMALL BUSINESS PERORMANCE IN SMALL TOWN IN MALAYSIA: AN EMPRICAL STUDY ON MALAYSIAN SMALL FIRMS

    Directory of Open Access Journals (Sweden)

    Muhammad Masroor ALAM

    2010-01-01

    Full Text Available Most research on market orientation, and performance was related to big firms. In this study, based on the theoretical framework, a model was developed to investigate the effect of market orientation on business performance in small firms. To test the relationships among the variables, data from 53 small firms in the small town of Chunglun at Sintok, Kedah was used. The findings show that the three components of market orientation are related positive to business performance of small firms. The further analysis also confirmed that customer orientation and competitor orientation are strong predictors of small firm performance. The findings of this study confirm that market orientation behavior also applies to small firms.

  3. Silencing of SARS-CoV spike gene by small interfering RNA in HEK 293T cells

    International Nuclear Information System (INIS)

    Qin Zhaoling; Zhao Ping; Zhang Xiaolian; Yu Jianguo; Cao Mingmei; Zhao Lanjuan; Luan Jie; Qi Zhongtian

    2004-01-01

    Two candidate small interfering RNAs (siRNAs) corresponding to severe acute respiratory syndrome-associated coronavirus (SARS-CoV) spike gene were designed and in vitro transcribed to explore the possibility of silencing SARS-CoV S gene. The plasmid pEGFP-optS, which contains the codon-optimized SARS-CoV S gene and expresses spike-EGFP fusion protein (S-EGFP) as silencing target and expressing reporter, was transfected with siRNAs into HEK 293T cells. At various time points of posttransfection, the levels of S-EGFP expression and amounts of spike mRNA transcript were detected by fluorescence microscopy, flow cytometry, Western blot, and real-time quantitative PCR, respectively. The results showed that the cells transfected with pEGFP-optS expressed S-EGFP fusion protein at a higher level compared with those transfected with pEGFP-S, which contains wildtype SARS-CoV spike gene sequence. The green fluorescence, mean fluorescence intensity, and SARS-CoV S RNA transcripts were found significantly reduced, and the expression of SARS-CoV S glycoprotein was strongly inhibited in those cells co-transfected with either EGFP- or S-specific siRNAs. Our findings demonstrated that the S-specific siRNAs used in this study were able to specifically and effectively inhibit SARS-CoV S glycoprotein expression in cultured cells through blocking the accumulation of S mRNA, which may provide an approach for studies on the functions of SARS-CoV S gene and development of novel prophylactic or therapeutic agents for SARS-CoV

  4. Small Modular Reactors: Institutional Assessment

    Energy Technology Data Exchange (ETDEWEB)

    Joseph Perkowski, Ph.D.

    2012-06-01

    ? Objectives include, among others, a description of the basic development status of “small modular reactors” (SMRs) focused primarily on domestic activity; investigation of the domestic market appeal of modular reactors from the viewpoints of both key energy sector customers and also key stakeholders in the financial community; and consideration of how to proceed further with a pro-active "core group" of stakeholders substantially interested in modular nuclear deployment in order to provide the basis to expedite design/construction activity and regulatory approval. ? Information gathering was via available resources, both published and personal communications with key individual stakeholders; published information is limited to that already in public domain (no confidentiality); viewpoints from interviews are incorporated within. Discussions at both government-hosted and private-hosted SMR meetings are reflected herein. INL itself maintains a neutral view on all issues described. Note: as per prior discussion between INL and CAP, individual and highly knowledgeable senior-level stakeholders provided the bulk of insights herein, and the results of those interviews are the main source of the observations of this report. ? Attachment A is the list of individual stakeholders consulted to date, including some who provided significant earlier assessments of SMR institutional feasibility. ? Attachments B, C, and D are included to provide substantial context on the international status of SMR development; they are not intended to be comprehensive and are individualized due to the separate nature of the source materials. Attachment E is a summary of the DOE requirements for winning teams regarding the current SMR solicitation. Attachment F deserves separate consideration due to the relative maturity of the SMART SMR program underway in Korea. Attachment G provides illustrative SMR design features and is intended for background. Attachment H is included for overview

  5. Density of very small meteoroids

    Science.gov (United States)

    Kikwaya Eluo, Jean-Baptiste

    2015-08-01

    Knowing the density of meteoroids helps to determine the physical structure and gives insight into the composition of their parent bodies. The density of meteoroids can provide clues to their origins, whether cometary or asteroidal. Density helps also to characterize the risk meteoroids may pose to artificial satellites.Ceplecha (1968) calculated the density of small meteoroids based on a parameter KB (meteoroid beginning height) and classified them in four categories (A,B,C,D) with densities going from 2700 to 180 kgm-3.Babadzhanov(2002) applied a model based on quasi-continuous fragmentation (QCF) on 413 photographic Super-Schmidt meteors by solely fitting their light curves. Their densities range from 400 to 7800 kgm-3. Bellot Rubio et al. (2002) analyzed the same 413 photographic meteors assuming the single body theory based on meteoroid dynamical properties and found densities ranging from 400 to 4800 kgm-3. A thermal erosion model was used by Borovicka et al. (2007) to analyze, simultaneously, the observed decelerations and light curves of six Draconid meteors. The density was found to be 300 kgm-3, consistent with the fact that the Draconid meteors are porous aggregates of grains associated with the Jupiter-family-comet 21P/Giacobini-Zinner (Jacchia, L.G., 1950).We used the Campbell-Brown and Koschny (2004) model of meteoroid ablation to determine the density of faint meteoroids from the analysis of both observed decelerations and light curves of meteoroids (Kikwaya et al., 2009; Kikwaya et al., 2011). Our work was based on a collection of six and ninety-two sporadic meteors. The grain masses used in the modeling ranged from 10-12 Kg to 10-9 Kg. We computed the orbit of each meteoroid and determined its Tisserand parameter. We found that meteoroids with asteroidal orbits have bulk densities ranging from 3000-5000 kgm-3. Meteoroids consistent with HTC/NIC parents have bulk densities from 400 kgm-3 to 1600 kg m-3. JFC meteoroids were found to have surprisingly

  6. 78 FR 48537 - Small Business Innovation Research and Small Business Technology Transfer Programs...

    Science.gov (United States)

    2013-08-08

    ... SMALL BUSINESS ADMINISTRATION [Docket Number: 2013-0008] Small Business Innovation Research and... Innovation Research (SBIR) and Small Business Technology Transfer (STTR) program Commercialization Benchmark.... Mail/Hand Delivery/Courier: Edsel Brown, Jr., Assistant Director, Office of Innovation, Small Business...

  7. 78 FR 59410 - Small Business Innovation Research and Small Business Technology Transfer Programs...

    Science.gov (United States)

    2013-09-26

    ... SMALL BUSINESS ADMINISTRATION [Docket Number: 2013-0008] Small Business Innovation Research and... Business Administration (SBA) is reopening the comment period for the Small Business Innovation Research... Delivery/Courier: Edsel Brown, Jr., Assistant Director, Office of Innovation, Small Business Administration...

  8. SMALL ENTERPRENEURSHIP IN HOSPITALITY: CROATIAN EXPERIENCES

    Directory of Open Access Journals (Sweden)

    Slobodan Ivanovic

    2013-03-01

    Full Text Available After introductory explanations regarding the selection problems of small enterprises in the Croatian hotel industry, the author provides a number of key indicators of their role in improving the competitiveness of the Croatian hotel industry in the global tourism market. In developed tourism countries, small enterprises have a dominant share in the structure of the hotel industry, and their importance is growing even in the former socialist countries of Europe. Small hotels in Italy accounted for more than 50%, and in Austria with more than 70% of lodging facilities and represent a generator of hotel business in a large number of European tourism countries, and great attention is given to the small enterpreneurs stimulating their growth with different measures of tourist and general economy politics. The author reflects on the Croatian experience of the small businesses in the hospitality through research of development of small family hotels and their importance for the improvement of the supply of the Croatian hotel industry which future is built on personal approach to guests and present trends in the tourism market. States that the role of the National Association of family and small hotels, which represents the specific interests of small hoteliers and enables the continuous improvement of the quality of their offerings, as well as measures to encourage small business development at the macro level. Based on the made analysis the measures of increasing the efficiency of small businesses in the Croatian hospitality are given.

  9. Are small hyperbolic universes observationally detectable?

    International Nuclear Information System (INIS)

    Gomero, G.I.; Reboucas, M.J.; Tavakol, R.; Queen Mary University of London, London

    2001-06-01

    Using recent observational constraints on cosmological density parameters, together with recent mathematical results concering small volume hyperbolic manifolds, we argue that, by employing pattern repetitions, the topology of nearly flat small hyperbolic universes can be observationally undetectable. This is important in view of the facts quantum cosmology may favour hyperbolic universes with small volumes, and from the expectation, coming from inflationary scenarios, that Ω 0 is likely to be very close to one. (author)

  10. Small is beautiful: Russian compact reactors

    International Nuclear Information System (INIS)

    Ivanov, A.; Perera, J.

    1994-01-01

    Large areas of Russian territory still have no centralized electricity supply making them particularly suitable for the small nuclear power plants described in this article. Thirty small reactor designs have been assessed each generating between 1 MWe and 150 MWe. Six designs were selected as winners, two in each section of small, medium and large designs, capable of producing low cost electricity, efficiently. These are described briefly. plans are underway for the construction of some of the design winners in remote locations. (UK)

  11. Small banks and local economic development

    OpenAIRE

    Hakenes , Hendrik; Hasan, Iftekhar; Molyneux, Phil; Xie , Ru

    2014-01-01

    This article discusses the effects of small banks on economic growth. We first theoretically show that small banks operating at a regional level can spur local economic growth. As compared with big interregional banks, small regional banks are more effective in promoting local economic growth, especially in regions with lower initial endowments and severe credit rationing. We then test the model predictions using a sample of German banks and corresponding regional statistics. We find that sma...

  12. Are small hyperbolic universes observationally detectable?

    Energy Technology Data Exchange (ETDEWEB)

    Gomero, G.I. [Centro Brasileiro de Pesquisas Fisicas (CBPF), Rio de Janeiro, RJ (Brazil)]|[Universidade Catolica de Petropolis, RJ (Brazil); E-mail: gomero@cbpf.br; Reboucas, M.J. [Centro Brasileiro de Pesquisas Fisicas (CBPF), Rio de Janeiro, RJ (Brazil); E-mail: reboucas@cbpf.br; Tavakol, R. [Centro Brasileiro de Pesquisas Fisicas (CBPF), Rio de Janeiro, RJ (Brazil)]|[Queen Mary University of London, London (United Kingdom). School of Mathematical Sciences, Astronomy Unit]. E-mail: r.tavakol@qmw.ac.uk

    2001-06-01

    Using recent observational constraints on cosmological density parameters, together with recent mathematical results concering small volume hyperbolic manifolds, we argue that, by employing pattern repetitions, the topology of nearly flat small hyperbolic universes can be observationally undetectable. This is important in view of the facts quantum cosmology may favour hyperbolic universes with small volumes, and from the expectation, coming from inflationary scenarios, that {omega}{sub 0} is likely to be very close to one. (author)

  13. FACTORS CRISIS OF SMALL BUSINESS DEVELOPMENT

    Directory of Open Access Journals (Sweden)

    A. I. Khorev

    2015-01-01

    Full Text Available Today it became clear that Russia needs not only economic growth, but also economic growth of a certain quality. Re¬gions are not an exception, since they are the most vulnerable to changes in market conditions resource exports. The main economic indicators for larger regions of the country should become entrepreneurs. The main economic indicators for larger regions of the country should become entrepreneurs. Not accidentally retrospective analysis of the evolutionary development of small business shows that the most important role in the economic development of Europe and America have played and continue to play a small business. Results of activity of small businesses are generally positive; the country saw an increase in the main indicators: the number of small enterprises, the average number of employees in small enterprises, turnover of small businesses and investments in fixed assets. The statistics of recent years show a decline of interest in the business population. Number of small businesses created in the last few years there has been a downward trend, while the proportion of surviving for three years after their registration is low. The greatest negative impact on the business affairs of the factors of regulatory and administrative regulation of small business, corruption and bribery of officials and the activities of control and inspection bodies. Addressing these challenges will require proper coordination of regional and local resources in building infrastructure, forming investment mechanisms to support small enterprises, the development of the entrepreneurial potential of the population.

  14. Small-bowel permeability in collagenous colitis

    DEFF Research Database (Denmark)

    Wildt, Signe; Madsen, Jan L; Rumessen, Jüri J

    2006-01-01

    OBJECTIVE: Collagenous colitis (CC) is a chronic inflammatory bowel disease that affects the colon. However, some patients with CC present with accompanying pathologic small-bowel manifestations such as coeliac disease, defects in bile acid absorption and histopathologic changes in small-intestin......OBJECTIVE: Collagenous colitis (CC) is a chronic inflammatory bowel disease that affects the colon. However, some patients with CC present with accompanying pathologic small-bowel manifestations such as coeliac disease, defects in bile acid absorption and histopathologic changes in small...

  15. Small wind planning and building successful installations

    CERN Document Server

    Clark, Nolan

    2013-01-01

    This book provides a cohesive guide to achieving successful small wind installations. It is a comprehensive information resource from one of the world's most experienced small wind professionals, covering all the key issues for small wind system development, from site and machine selection to international standards compliance. Engineers who are tasked with planning and developing these small wind systems, from choosing the best site and accurately estimating likely power output to obtaining proper permitting and troubleshooting operational inefficiencies, will find this book an ess

  16. Brain networks: small-worlds, after all?

    International Nuclear Information System (INIS)

    Muller, Lyle; Destexhe, Alain; Rudolph-Lilith, Michelle

    2014-01-01

    Since its introduction, the ‘small-world’ effect has played a central role in network science, particularly in the analysis of the complex networks of the nervous system. From the cellular level to that of interconnected cortical regions, many analyses have revealed small-world properties in the networks of the brain. In this work, we revisit the quantification of small-worldness in neural graphs. We find that neural graphs fall into the ‘borderline’ regime of small-worldness, residing close to that of a random graph, especially when the degree sequence of the network is taken into account. We then apply recently introducted analytical expressions for clustering and distance measures, to study this borderline small-worldness regime. We derive theoretical bounds for the minimal and maximal small-worldness index for a given graph, and by semi-analytical means, study the small-worldness index itself. With this approach, we find that graphs with small-worldness equivalent to that observed in experimental data are dominated by their random component. These results provide the first thorough analysis suggesting that neural graphs may reside far away from the maximally small-world regime. (paper)

  17. Structural peculiarities in magnetic small particles

    International Nuclear Information System (INIS)

    Haneda, K.; Morrish, A.H.

    1993-01-01

    Nanostructured magnetic materials, consisting of nanometer-sized crystallites, are currently a developing subject. Evidence has been accumulating that they possess properties that can differ substantially from those of bulk materials. This paper illustrates how Moessbauer spectroscopy can yield useful information on the structural peculiarities associated with these small particles. As illustrations, metallic iron and iron-oxide systems are considered in detail. The subjects discussed include: (1) Phase stabilities in small particles, (2) deformed or nonsymmetric atomic arrangements in small particles, and (3) peculiar magnetic structures or non-collinear spin arrangements in small magnetic oxide particles that are correlated with lower specific magnetizations as compared to the bulk values. (orig.)

  18. Advanced Deployable Structural Systems for Small Satellites

    Science.gov (United States)

    Belvin, W. Keith; Straubel, Marco; Wilkie, W. Keats; Zander, Martin E.; Fernandez, Juan M.; Hillebrandt, Martin F.

    2016-01-01

    One of the key challenges for small satellites is packaging and reliable deployment of structural booms and arrays used for power, communication, and scientific instruments. The lack of reliable and efficient boom and membrane deployment concepts for small satellites is addressed in this work through a collaborative project between NASA and DLR. The paper provides a state of the art overview on existing spacecraft deployable appendages, the special requirements for small satellites, and initial concepts for deployable booms and arrays needed for various small satellite applications. The goal is to enhance deployable boom predictability and ground testability, develop designs that are tolerant of manufacturing imperfections, and incorporate simple and reliable deployment systems.

  19. Subjective Expected Utility Theory with "Small Worlds"

    DEFF Research Database (Denmark)

    Gyntelberg, Jacob; Hansen, Frank

    We model the notion of a "small world" as a context dependent state space embedded into the "grand world". For each situation the decision maker creates a "small world" reflecting the events perceived to be relevant for the act under consideration. The "grand world" is represented by an event space...

  20. Governance of ecosystem services on small islands

    NARCIS (Netherlands)

    Polman, Nico; Reinhard, Stijn; Bets, van L.K.J.; Kuhlman, Tom

    2016-01-01

    Natural ecosystems provide an attractive focus for tourism on small islands. However, at the same time tourism and other human actions can be detrimental to these ecosystems especially because governance of the ecosystem may be difficult due to the limited resilience of small island ecosystems.

  1. Allosteric small-molecule kinase inhibitors

    DEFF Research Database (Denmark)

    Wu, Peng; Clausen, Mads Hartvig; Nielsen, Thomas E.

    2015-01-01

    current barriers of kinase inhibitors, including poor selectivity and emergence of drug resistance. In spite of the small number of identified allosteric inhibitors in comparison with that of inhibitors targeting the ATP pocket, encouraging results, such as the FDA-approval of the first small...

  2. Radiation Hardness Assurance (RHA) for Small Missions

    Science.gov (United States)

    Campola, Michael J.

    2016-01-01

    Varied mission life and complexity is growing for small spacecraft. Small missions benefit from detailed hazard definition and evaluation as done in the past. Requirements need to flow from the system down to the parts level and aid system level radiation tolerance. RHA is highlighted with increasing COTS usage.

  3. Competitive analysis of small hydroelectric power plants

    International Nuclear Information System (INIS)

    Assad, L.S.; Placido, R.

    1990-01-01

    The agreement between CPFL/UNICAMP/EFEI for developing energetic planning of Small Hydroelectric Power Plants construction is described. Some notions for showing the more economic alternative between decide by Small Hydroelectric Power Plants construction and continue supply the market by inter ligated system generation are shown in this stage of the agreement. (author)

  4. Small Drinking Water Systems Communication and Outreach ...

    Science.gov (United States)

    As part of our small drinking water systems efforts, this poster highlights several communications and outreach highlights that EPA's Office of Research and Development and Office of Water have been undertaking in collaboration with states and the Association of State Drinking Water Administrators. To share information at EPA's annual small drinking water systems workshop

  5. Treatment Option Overview (Small Intestine Cancer)

    Science.gov (United States)

    ... all of an organ that contains cancer. The resection may include the small intestine and nearby organs (if the cancer has spread). The doctor may remove the section of the small intestine that contains cancer and perform an anastomosis (joining the cut ends of the intestine together). ...

  6. General Information about Small Intestine Cancer

    Science.gov (United States)

    ... all of an organ that contains cancer. The resection may include the small intestine and nearby organs (if the cancer has spread). The doctor may remove the section of the small intestine that contains cancer and perform an anastomosis (joining the cut ends of the intestine together). ...

  7. CT findings in acute small bowel diverticulitis

    International Nuclear Information System (INIS)

    Ferstl, F.J.; Obert, R.

    2004-01-01

    Small bowel diverticulitis is a rare cause of an acute abdomen. Originating from acquired diverticula of the jejunum, less often of the ileum, or Meckel diverticulum, the symptoms are non-specific, simulating other acute inflammatory disorders, such as appendicitis, cholecystitis or colonic diverticulitis. The diagnosis of small bowel diverticulitis is solely based on radiologic findings, with computed tomography (CT) regarded as the method of choice. In recent years, a number of case reports have described the spectrum of the CT features in acute small bowel diverticulitis and its dependence on the severity of the inflammatory process. Typical findings are an inflamed diverticulum, inflammatory mesenteric infiltration, extraluminal gas collection and mural edema of adjacent small bowel loops with resultant separation of bowel loops. An enterolith is rarely found in an inflamed diverticulum. Complications include abscesses, fistulae, small bowel obstruction and free perforation with peritonitis. Small bowel diverticulitis can be a diagnostic problem if it involves the terminal ileum or Meckel's diverticulum. For preoperative confirmation of the presumed diagnosis of small bowel diverticulitis on CT, an enteroclysis for acquired diverticula or a technetium scan for Meckel's diverticulum should be performed. We present the CT findings in three patients of acute small bowel diverticulitis, two affecting the jejunum and one a Meckel's diverticulum. (orig.) [de

  8. Environmental Education and Small Business Environmental Activity

    Science.gov (United States)

    Redmond, Janice; Walker, Beth

    2011-01-01

    Environmental education is seen as a key driver of small business environmental management, yet little is known about the activities small business owner-managers are undertaking to reduce their environmental impact or in what areas they may need education. Therefore, research that can identify environmental management activities being undertaken…

  9. Gravity Compensation Technique Uses Small dc Motor

    Science.gov (United States)

    Hollow, Richard

    1988-01-01

    Small dc servomotor powered by simple constant-current source and with suitable gearing used to cancel effect of gravity upon load. Lead-screw positioning system has load counterbalanced by small supplementary motor powered by constant current source. Motor lighter and more compact alternative to counterbalance. Used in variety of mechanical systems where load positioned or accelerated in vertical plane.

  10. XP in a Small Software Development Business

    DEFF Research Database (Denmark)

    Babb, Jeffry; Hoda, Rashina; Nørbjerg, Jacob

    2014-01-01

    While small software development shops have trended towards the adoption of Agile methods, local conditions and high iteration pressure typically cause adaptations and appropriations of Agile methods. This paper shares evidence from a study concerning how a small software development company adopts...

  11. Small-bowel permeability in collagenous colitis

    DEFF Research Database (Denmark)

    Wildt, Signe; Madsen, Jan L; Rumessen, Jüri J

    2006-01-01

    Collagenous colitis (CC) is a chronic inflammatory bowel disease that affects the colon. However, some patients with CC present with accompanying pathologic small-bowel manifestations such as coeliac disease, defects in bile acid absorption and histopathologic changes in small-intestinal biopsies...

  12. Energy efficient design of cognitive small cells

    NARCIS (Netherlands)

    Wildemeersch, Matthias; Wildemeersch, Matthias; Quek, Tony Q.S.; Rabbachin, Alberto; Slump, Cornelis H.; Huang, Aiping; Kim, Dong-In; Mueller, P.

    2013-01-01

    Heterogeneous networks consisting of a macrocell tier and a small cell tier are considered an attractive solution to cope with the fierce increase of mobile traffic demand. Nevertheless, a massive deployment of small cell access points (SAPs) leads also to a considerable increase in energy

  13. Small Hydropower in the United States

    Energy Technology Data Exchange (ETDEWEB)

    Hadjerioua, Boualem [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States); Johnson, Kurt [Telluride Energy, Telluride, CO (United States)

    2015-09-01

    Small hydropower, defined in this report as hydropower with a generating capacity of up to 10 MW typically built using existing dams, pipelines, and canals has substantial opportunity for growth. Existing small hydropower comprises about 75% of the current US hydropower fleet in terms of number of plants. The economic feasibility of developing new small hydropower projects has substantially improved recently, making small hydropower the type of new hydropower development most likely to occur. In 2013, Congress unanimously approved changes to simplify federal permitting requirements for small hydropower, lowering costs and reducing the amount of time required to receive federal approvals. In 2014, Congress funded a new federal incentive payment program for hydropower, currently worth approximately 1.5 cents/kWh. Federal and state grant and loan programs for small hydropower are becoming available. Pending changes in federal climate policy could benefit all renewable energy sources, including small hydropower. Notwithstanding remaining barriers, development of new small hydropower is expected to accelerate in response to recent policy changes.

  14. Hate Speech on Small College Campuses.

    Science.gov (United States)

    Hemmer, Joseph J., Jr.

    A study identified and evaluated the approach of small colleges in dealing with hate speech and/or verbal harassment incidents. A questionnaire was sent to the Dean of Students at 200 randomly-selected small (500-2000 students), private, liberal arts colleges and universities. Responses were received from 132 institutions, for a response rate of…

  15. Ecological Resilience of Small Urban Parks

    DEFF Research Database (Denmark)

    JasmaniI, Zanariah Binti

    . There are similarities in plant species across all the small parks studied, which may be due to the landscaping guidelines provided by the municipality. Overall, exotic vegetation was planted more than native vegetation. Small urban parks were used mainly for utilitarian purposes (42%, e.g. for walking or motorcyclist...

  16. Astrophysics with small satellites in Scandinavia

    DEFF Research Database (Denmark)

    Lund, Niels

    2003-01-01

    The small-satellites activities in the Scandinavian countries are briefly surveyed with emphasis on astrophysics research. (C) 2002 COSPAR. Published by Elsevier Science Ltd. All rights reserved.......The small-satellites activities in the Scandinavian countries are briefly surveyed with emphasis on astrophysics research. (C) 2002 COSPAR. Published by Elsevier Science Ltd. All rights reserved....

  17. Video capsule endoscopy of the small bowel.

    Science.gov (United States)

    Eliakim, Rami

    2013-03-01

    Small bowel capsule endoscopy (SBCE) was introduced 13 years ago by Given Imaging (Yokneam, Israel). It has become one of the most important investigational tools of the small bowel. The capsule is swallowed with water after a 12 h fast, propelled via peristalsis through the gastrointestinal tract and excreted naturally. A nonvideo patency capsule was developed to confirm functional patency of the gastrointestinal tract. Four additional companies have introduced competitive small bowel video capsules, some of which are Food and Drug Admistration (FDA) approved. Due to the easiness of the procedure, SBCE has become a first-line tool to detect small bowel abnormalities. The main indications for SBCE include obscure gastrointestinal bleeding, suspected Crohn's disease, small bowel tumors and practically any abnormal small bowel imaging. New indications are emerging like small bowel motility and monitoring of drug therapy and mucosal healing. The present review will describe the available capsules in the market, the procedure itself, present indications and future expectations. It will focus on the PillCam SB of Given Imaging as it is the one on which most of the literature is written. Over the past 10 years, SBCE has become a routine, first-line investigational tool of many small bowel pathologies.

  18. Small Business Administration Semiannual Regulatory Agenda

    Science.gov (United States)

    2010-12-20

    ... represent that it will make good faith efforts to award subcontracts to small businesses at the same... measurement of management and technical assistance projects conducted by Women's Business Center program... Business Administration Semiannual Regulatory Agenda] Part XVII Small Business Administration Semiannual...

  19. OSH management in small and micro enterprises

    NARCIS (Netherlands)

    Zwetsloot, G.I.J.M.

    2014-01-01

    Small and medium-sized enterprises (SMEs) are widely acknowledged as the backbone of the European economy. According to EUROSTAT statistics [1], [2], 29.6% of the EU employees work in micro enterprises (<10 employees), while 20.6 % are employed in small firms (<50 employees). Indeed, half of the

  20. Why Small Businesses Fail in Tunisia?

    OpenAIRE

    Anis Omri; Mohamed Frikha

    2014-01-01

    This paper aims at drawing up an average cognitive map in order to explain the failure factors of Tunisian small businesses. The method adopted in our study is the cognitive approach. Our study extends entrepreneurship literature and previous literature by proposing a new approach in order to build an average cognitive map for the explanation of small businesses failures.

  1. Small plasma source for materials application.

    Science.gov (United States)

    Vizir, A; Oks, E M; Salvadori, M C; Teixeira, F S; Brown, I G

    2007-08-01

    We describe a small hollow-cathode plasma source suitable for small-scale materials synthesis and modification application. The supporting electrical system is minimal. The gaseous plasma source delivers a plasma ion current of up to about 1 mA. Here we outline the source construction and operation, and present some of its basic performance characteristics.

  2. Brexit and small states in Europe

    DEFF Research Database (Denmark)

    Wivel, Anders; Thorhallsson, Baldur

    2018-01-01

    The aim of this chapter is to unpack how Brexit influences small states in Europe. The main argument is that while all small states are negatively affected by the British decision to invoke Article 50 of the Treaty of Lisbon and thereby to effectuate the outcome of the British 2016-referendum on ...

  3. Small Group Multitasking in Literature Classes

    Science.gov (United States)

    Baurain, Bradley

    2007-01-01

    Faced with the challenge of teaching American literature to large, multilevel classes in Vietnam, the writer developed a flexible small group framework called "multitasking". "Multitasking" sets up stable task categories which rotate among small groups from lesson to lesson. This framework enabled students to work cooperatively…

  4. "small ACCELERATORS" 24 May - 2 June 2005

    CERN Document Server

    2005-01-01

    CERN Accelerator School and Kernfysisch Versneller Instituut (KVI) Groningen, the Netherlands announce a course on "Small Accelerators", Hotel Golden Tulip Drenthe, Zeegse, the Netherlands, 24 May - 2 June 2005. This specialised course is dedicated to the physics and the main applications of small accelerators. The course will review the different accelerator types as well as their specificities in terms of accelerator physics.

  5. NIF small optics laser damage test specifications

    International Nuclear Information System (INIS)

    Sheehan, L

    1999-01-01

    The Laser Damage Group is currently conducting tests on small optics samples supplied for initial evaluation of potential NIF suppliers. This document is meant to define the specification of laser-induced damage for small optics and the test methods used to collect the data. A rating system which will be applied for vendor selection is presented

  6. Anatomy and Physiology of the Small Bowel.

    Science.gov (United States)

    Volk, Neil; Lacy, Brian

    2017-01-01

    Comprehension of small intestine physiology and function provides a framework for the understanding of several important disease pathways of the gastrointestinal system. This article reviews the development, anatomy and histology of the small bowel in addition to physiology and digestion of key nutrients. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Entrepreneurship and small business development in Ethiopia ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    Entrepreneurship and small business development in Ethiopia. This project aims to improve the Ethiopian government's policies and practices, and those of other actors, to support small- and medium-sized enterprise (SME) development in the country. Since the early 2000s, the Ethiopian government has implemented ...

  8. Encouraging the Domestic Small Turbine Market

    International Nuclear Information System (INIS)

    Forsyth, T.

    2001-01-01

    The state incentives for home-based renewables in the domestic market continue to grow and change creating opportunities for the small wind turbine market. Tracking the opportunities to get small wind turbines included in incentive policies and developing a proactive industry approach is important because market changes can occur anytime. There are near-term opportunities to work with states in developing their strategies for disbursing system benefit charges, adding tags to existing policies for other small renewables to include small wind, and developing state-wide net metering programs. Other opportunities to improve the domestic market exist but will be quite challenging to implement. Other opportunities include federal tax credits, state wind access laws, equipment verification for specific states, and leasing programs for small wind turbines

  9. Use of small RNA as antiaging cosmeceuticals.

    Science.gov (United States)

    Zhang, Pingjing; Chen, Jianxin; Li, Tiejun; Zhu, York Yuanyuan

    2013-01-01

    Over the past two decades, RNA interference (RNAi) has achieved great improvements in medicine, which has benefited the development of innovative cosmeceutical products, particular, to antiaging cosmeceuticals. A variety of ongoing research has tried to employ small RNAs-small interference RNA and microRNA as new cosmeceutical ingredients. Furthermore, several skin care companies have released new small RNA products in cosmetic market. In this review, we will describe the latest and most advanced approaches and strategies of using small RNA as antiaging cosmetics, including investigations on aging-related genes that small RNA target, method of delivering them, and challenges in the development of RNAi-based therapeutics for skin care cosmeceuticals. It is certain that advancement in this direction will evolve a new landscape for innovative antiaging cosmeceuticals.

  10. Endovascular treatment of very small intracranial aneurysms

    DEFF Research Database (Denmark)

    Iskandar, A; Nepper-Rasmussen, J

    2011-01-01

    to large aneurysms (> 3 mm). However the data also suggest that endovascular treatment of very small aneurysms might be associated with an increased risk of procedural ruptures and mortality. At nine-month follow-up results indicate significantly less compaction in the very small aneurysms....... endovascular treatment was attempted in 956 consecutive intracranial aneurysms. Of 956 aneurysms, 111 aneurysms were very small aneurysms with a maximal diameter of 3 mm or less. We conducted a retrospective analysis of angiographic and clinical outcome following coiling of very small aneurysms...... aneurysms and less than 90% aneurysm occlusion in six aneurysms. Complications occurred in the treatment of 15 aneurysms, including eight procedural ruptures, six thromboembolic events and one case of early hemorrhage. Compared with larger aneurysms, treatment of very small aneurysms was associated...

  11. Small scale structure on cosmic strings

    International Nuclear Information System (INIS)

    Albrecht, A.

    1989-01-01

    I discuss our current understanding of cosmic string evolution, and focus on the question of small scale structure on strings, where most of the disagreements lie. I present a physical picture designed to put the role of the small scale structure into more intuitive terms. In this picture one can see how the small scale structure can feed back in a major way on the overall scaling solution. I also argue that it is easy for small scale numerical errors to feed back in just such a way. The intuitive discussion presented here may form the basis for an analytic treatment of the small structure, which I argue in any case would be extremely valuable in filling the gaps in our resent understanding of cosmic string evolution. 24 refs., 8 figs

  12. Small cell networks deployment, management, and optimization

    CERN Document Server

    Claussen, Holger; Ho, Lester; Razavi, Rouzbeh; Kucera, Stepan

    2018-01-01

    Small Cell Networks: Deployment, Management, and Optimization addresses key problems of the cellular network evolution towards HetNets. It focuses on the latest developments in heterogeneous and small cell networks, as well as their deployment, operation, and maintenance. It also covers the full spectrum of the topic, from academic, research, and business to the practice of HetNets in a coherent manner. Additionally, it provides complete and practical guidelines to vendors and operators interested in deploying small cells. The first comprehensive book written by well-known researchers and engineers from Nokia Bell Labs, Small Cell Networks begins with an introduction to the subject--offering chapters on capacity scaling and key requirements of future networks. It then moves on to sections on coverage and capacity optimization, and interference management. From there, the book covers mobility management, energy efficiency, and small cell deployment, ending with a section devoted to future trends and applicat...

  13. Small x phenomenology. Summary of the 3rd Lund small x workshop in 2004

    International Nuclear Information System (INIS)

    Andersen, J.R.; Baranov, S.; Bartels, J.

    2006-04-01

    A third workshop on small-x physics, within the Small-x Collaboration, was held in Hamburg in May 2004 with the aim of overviewing recent theoretical progress in this area and summarizing the experimental status. (Orig.)

  14. Small Grain Production Pt 2: Growth and Development of Small Grains

    OpenAIRE

    Jackson, Lee; Williams, Jack

    2006-01-01

    Part 2 of the 14-part Small Grain Production Manual describes the stages of small grain growth, including germination, tillering, vegetative growth, stem elongation, heading, flowering, grain filling, growth habit and yield components.

  15. Collaborative Manufacturing for Small-Medium Enterprises

    Science.gov (United States)

    Irianto, D.

    2016-02-01

    Manufacturing systems involve decisions concerning production processes, capacity, planning, and control. In a MTO manufacturing systems, strategic decisions concerning fulfilment of customer requirement, manufacturing cost, and due date of delivery are the most important. In order to accelerate the decision making process, research on decision making structure when receiving order and sequencing activities under limited capacity is required. An effective decision making process is typically required by small-medium components and tools maker as supporting industries to large industries. On one side, metal small-medium enterprises are expected to produce parts, components or tools (i.e. jigs, fixture, mold, and dies) with high precision, low cost, and exact delivery time. On the other side, a metal small- medium enterprise may have weak bargaining position due to aspects such as low production capacity, limited budget for material procurement, and limited high precision machine and equipment. Instead of receiving order exclusively, a small-medium enterprise can collaborate with other small-medium enterprise in order to fulfill requirements high quality, low manufacturing cost, and just in time delivery. Small-medium enterprises can share their best capabilities to form effective supporting industries. Independent body such as community service at university can take a role as a collaboration manager. The Laboratory of Production Systems at Bandung Institute of Technology has implemented shared manufacturing systems for small-medium enterprise collaboration.

  16. Optimisation of Investment Resources at Small Enterprises

    Directory of Open Access Journals (Sweden)

    Shvets Iryna B.

    2014-03-01

    Full Text Available The goal of the article lies in the study of the process of optimisation of the structure of investment resources, development of criteria and stages of optimisation of volumes of investment resources for small enterprises by types of economic activity. The article characterises the process of transformation of investment resources into assets and liabilities of the balances of small enterprises and conducts calculation of the structure of sources of formation of investment resources in Ukraine at small enterprises by types of economic activity in 2011. On the basis of the conducted analysis of the structure of investment resources of small enterprises the article forms main groups of criteria of optimisation in the context of individual small enterprises by types of economic activity. The article offers an algorithm and step-by-step scheme of optimisation of investment resources at small enterprises in the form of a multi-stage process of management of investment resources in the context of increase of their mobility and rate of transformation of existing resources into investments. The prospect of further studies in this direction is development of a structural and logic scheme of optimisation of volumes of investment resources at small enterprises.

  17. Pseudolisthesis secondary to small L5

    International Nuclear Information System (INIS)

    Bagheri, Mohammad Hadi; Hosseini, Mehrdad Mohammad; Foroughi, Amin Aboulhassani

    2011-01-01

    Rationale and objective: Small AP diameter of L5 can mimic spondylolisthesis in lumbosacral MRI. The aim of the present study was to evaluate the prevalence of small L5 mimicking spondylolisthesis in lumbosacral MRI of the patients referred to an imaging center by any symptoms. Association between small anteroposterior diameter of L5 and presence and absence of unilateral or bilateral spondylolysis at L5 were also evaluated. Materials and methods: 2000 lumbosacral MRI of the patients referred to an imaging center for any reasons were evaluated. The posterior step of L5 was measured and the cases with posterior step of more than 2 mm were selected. These cases were evaluated for presence or absence of spondylolysis. Results: Small anteroposterior diameters of L5 were detected in 38 cases (2%), mimicking the spondylolisthesis in lumbosacral MRI. The prevalence of small L5 in lumbosacral MRI of the patients referred by any symptoms was about 2%. In these selected cases, 6 (15.8%) show unilateral spondylolysis and 5 (13.1%) show bilateral spondylolysis. Conclusion: Pseudospondylolisthesis secondary to small AP diameter of L5 has prevalence of about 2% in lumbosacral MRI of cases referred for any reasons and can be overlooked in reporting of LS MRI. There was an association between small anteroposterior diameter of L5 and presence of spondylolysis at L5.

  18. Transforming a small business: A learning intervention

    Directory of Open Access Journals (Sweden)

    Dirk J. Geldenhuys

    2012-03-01

    Motivation for the study: This study explored the important role of small businesses; the role of management; and, more specifically, organisation development models that were developed for large corporations; as these emphasise the need to study the purpose of organisation development interventions in small businesses. Research design, approach and method: The study reported on in this article was a qualitative case study in a small brokers company with 21 staff members. The data were analysed manually by means of qualitative content analysis. Main findings: The main finding of the research concerned how experiential learning intervention provided a space for participants to start exploring, and gradually changing the dynamics of their small business, by transforming their business into a more formal company. Practical/managerial implications: The primary implication is that experiential learning interventions that are operationalised, from a psychodynamic perspective, can be valuable when consulted by small businesses during their transformation towards becoming more formal companies. Contribution/value-add: This article contributes to the OD literature on small businesses, with fewer than 50 employees, by providing data on how the learning process, that commenced during an experiential learning intervention, assisted a small company to develop its identity towards that of a more formal company.

  19. Great governance in small societies : Effective public sector practices in small-scale democracies

    NARCIS (Netherlands)

    Douglas, Scott

    2011-01-01

    Every government faces its own particular challenges. For a small-scale democracy, the size of its society might be the obvious limitation. Most discussion about small societies thus focuses on the governance failures caused by this small size. This book argues that it would be more useful to

  20. 47 CFR 90.1017 - Bidding credits for small businesses and very small businesses.

    Science.gov (United States)

    2010-10-01

    ...) SAFETY AND SPECIAL RADIO SERVICES PRIVATE LAND MOBILE RADIO SERVICES Competitive Bidding Procedures for the 220 MHz Service § 90.1017 Bidding credits for small businesses and very small businesses. A... 47 Telecommunication 5 2010-10-01 2010-10-01 false Bidding credits for small businesses and very...