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  1. Bardet-Biedl Syndrome

    Science.gov (United States)

    ... Honor a Loved One Monthly Giving Employee Matching Workplace Giving Remember FFB in Your Will or Estate ... have small genitalia (testes and penis). Because female sexual organ size is more ... aids and orientation as well as from mobility training. Researchers have ...

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

    International Nuclear Information System (INIS)

    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)

  3. Síndrome de Bardet-Biedl: relato de dois casos Bardet-Biedl syndrome: two case reports

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    Jacó Lavinsky

    2003-10-01

    Full Text Available A síndrome de Bardet-Biedl é doença de herança autossômica recessiva caracterizada por distrofia retiniana, polidactilia, obesidade, retardo mental e hipogenitalismo. Um ou mais dos achados acima que caracterizam a síndrome podem estar ausentes, mas a distrofia retiniana é achado consistente. Esta se manifesta clinicamente na infância com progressiva perda visual, causando grave dificuldade visual na adolescência. Os autores descrevem dois casos de síndrome de Bardet-Biedl, os resultados da acuidade visual, biomicroscopia, oftalmoscopia, angiografia, campo visual e eletrorretinograma. Foi realizada revisão bibliográfica com ênfase na identificação dos sinais sistêmicos, envolvimento ocular, testes eletrofisiológicos e avaliação genética.Bardet-Biedl syndrome is a hereditary autosomal recessive disease characterized by retinal dystrophy, polydactyly, obesity, mental retardation, and hypogenitalism. One or more of the clinical features characterizing the syndrome may be absent, but retinal dystrophy is a consistent finding. It becomes clinically manifest in early childhood, with progressive loss of visual function, leading to severe visual disability in early adolescence. The authors describe two cases of Bardet-Biedl syndrome, the results of visual acuity, slit-lamp examination, ophthalmoscopy, angiography, visual fields and electroretinograms. The literature was reviewed stressing the importance of identifying systemic signs, ocular involvement, electrophysiologic tests and genetic evaluation.

  4. Intrafamilial variation of the phenotype in Bardet-Biedl syndrome

    OpenAIRE

    Riise, R.; Andreasson, S.; Borgstrom, M.; Wright, A; Tommerup, N.; Rosenberg, T; Tornqvist, K

    1997-01-01

    AIMS—To describe the variation of the phenotype within families with several individuals with Bardet-Biedl syndrome.
METHODS—The phenotypes of affected siblings in 11 Scandinavian families were compared with two or more members who had at least three of the features: retinal dystrophy, polydactyly, obesity, hypogenitalism, and mental retardation. Individuals without retinal dystrophy were excluded.
RESULTS—Intrafamilial variation of expressivity of the features obesity, polydactyly, abnormal ...

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

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    Valavi Ehsan

    2009-01-01

    Full Text Available 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 retar-dation, 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.

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

    International Nuclear Information System (INIS)

    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. 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; Nishimura, Darryl Y; Riise, Ruth; Sheffield, Val C; Rosenberg, Thomas; Brøndum-Nielsen, Karen

    2010-01-01

    Bardet-Biedl syndrome (BBS) is an autosomal recessive disease characterized by retinal dystrophy, polydactyly, obesity, learning disabilities, renal involvement, and male hypogenitalism. BBS is genetically heterogeneous with mutations of 14 genes, accounting for approximately 70% of cases...

  8. Nephrolithiasis with Bardet-Biedl syndrome in a three-year-old girl: A case report

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    Meral Torun Bayram

    2014-04-01

    Full Text Available Bardet-Biedl syndrome is a multisystemic developmental disorder diagnosed on the basis of the presence of obesity, retinal defects, polydactyly, hypogonadism, renal dysfunction, and learning disabilities. Renal disease is clinically heterogeneous, but is recognized as a cardinal feature and is a major cause of mortality in BBS. We here presented a three-year-old girl with renal stone and Bardet-Biedl syndrome.

  9. 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. PMID:26762677

  10. A novel homozygous 10 nucleotide deletion in BBS10 causes Bardet-Biedl syndrome in a Pakistani family

    NARCIS (Netherlands)

    Agha, Z.; Iqbal, Z.; Azam, M.; Hoefsloot, L.H.; Bokhoven, J.H.L.M. van; Qamar, R.

    2013-01-01

    Bardet-Biedl Syndrome is a multisystem autosomal recessive disorder characterized by central obesity, polydactyly, hypogonadism, learning difficulties, rod-cone dystrophy and renal dysplasia. Bardet-Biedl Syndrome has a prevalence rate ranging from 1 in 100,000 to 1 in 160,000 births although there

  11. 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....

  12. Case Study of Bardet-Biedl Syndrome with Fetal Lobulation and Focal Segmental Glomerulosclerosis

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    Yılmaz Tabel

    2008-05-01

    Full Text Available Bardet-Biedl syndrome is a genetic autosomal recessive disease characterized by abdominal obesity, mental retardation, dysmorphic extremities, retinal dystrophy or pigmentary retinopathy, hypogonadism or hypogenitalism, and kidney involvement. Patients with renal involvement may present with both structural and functional abnormalities. Here, we present a 15-year-old male with Bardet-Biedl syndrome who has both fetal lobulation, a rare type of structural abnormality, and focal segmental glomerulosclerosis, having not been reported before. Turk Jem 2008; 12: 32-4

  13. Exome sequencing identifies a novel and a recurrent BBS1 mutation in Pakistani families with Bardet-Biedl syndrome

    NARCIS (Netherlands)

    Ajmal, M.; Khan, M.I.; Neveling, K.; Tayyab, A.; Jaffar, S.; Sadeque, A.; Ayub, H.; Abbasi, N.M.; Riaz, M.; Micheal, S.; Gilissen, C.F.H.A.; Ali, S.H.; Azam, M.; Collin, R.W.J.; Cremers, F.P.M.; Qamar, R.

    2013-01-01

    PURPOSE: To determine the genetic cause of Bardet-Biedl syndrome (BBS) in two consanguineous Pakistani families. METHODS: Clinical characterization of the affected individuals in both families was performed with ophthalmic examination, electroretinography, electrocardiography, and liver and renal pr

  14. Clinical pathology and retinal vascular structure in the Bardet-Biedl syndrome.

    OpenAIRE

    Bek, T; Rosenberg, T

    1995-01-01

    A comparative study of clinical pathology and retinal vascular structure is described as studied by vascular casting in an eye of a patient with the Bardet-Biedl syndrome. At the time of examination the eye had been almost blind for at least 4 years. The histopathological examination showed a largely uniform loss of the outer retinal layers. The gross pathological examination of the cast ocular fundus showed three distinct zones, an inner zone inside the temporal vascular arcades where retina...

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

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    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.

  16. New criteria for improved diagnosis of Bardet-Biedl syndrome: results of a population survey

    OpenAIRE

    Beales, P; Elcioglu, N; Woolf, A; Parker, D.; Flinter, F

    1999-01-01

    Bardet-Biedl syndrome (BBS) is an autosomal recessive condition characterised by rod-cone dystrophy, postaxial polydactyly, central obesity, mental retardation, hypogonadism, and renal dysfunction. BBS expression varies both within and between families and diagnosis is often difficult. We sought to define the condition more clearly by studying 109 BBS patients and their families, the largest population surveyed to date. The average age at diagnosis was 9 years, which is late for such a debili...

  17. Two Brothers with Bardet-Biedl Syndrome Presenting with Chronic Renal Failure

    OpenAIRE

    Cem Sahin; Bulent Huddam; Gulhan Akbaba; Hasan Tunca; Emine Koca; Mustafa Levent

    2015-01-01

    Bardet-Biedl Syndrome (BBS) is a rarely seen autosomal recessive transfer disease characterised by retinal dystrophy, obesity, extremity deformities, mental retardation, and renal and genital system anomalies. BBS shows heterogenic transfer. To date, 18 genes (BBS1–18) and 7 BBS proteins have been defined as related to BBS. All of the defined BBS genes have been shown to be related to the biogenesis or function of cilia. Renal failure accompanying the syndrome, especially in the advanced stag...

  18. Cognitive, Sensory, and Psychosocial Characteristics in Patients with Bardet-Biedl Syndrome

    OpenAIRE

    Brinckman, Danielle D.; Keppler-Noreuil, Kim M.; Blumhorst, Catherine; Leslie G Biesecker; Sapp, Julie C; Johnston, Jennifer J; Wiggs, Edythe A

    2013-01-01

    Forty-two patients with a clinical diagnosis of Bardet-Biedl syndrome ages 2-61 years were given a neuropsychological test battery to evaluate cognitive, sensory and behavioral functioning. These tests included the Wechsler scales of intelligence, Rey Auditory Verbal Learning Test, Boston Naming Test, D-KEFS Verbal Fluency Test, D-KEFS Color-Word Interference Test, D-KEFS Sorting Test, Wide Range Achievement Test: Math and Reading Subtests, Purdue Pegboard, The University of Pennsylvania Smel...

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

    OpenAIRE

    Hamlington, Barbara; Lauren E Ivey; 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 chi...

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

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    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.

  1. Transient ciliogenesis involving Bardet-Biedl syndrome proteins is a fundamental characteristic of adipogenic differentiation.

    Science.gov (United States)

    Marion, Vincent; Stoetzel, Corinne; Schlicht, Dominique; Messaddeq, Nadia; Koch, Michael; Flori, Elisabeth; Danse, Jean Marc; Mandel, Jean-Louis; Dollfus, Hélène

    2009-02-10

    Bardet-Biedl syndrome (BBS) is an inherited ciliopathy generally associated with severe obesity, but the underlying mechanism remains hypothetical and is generally proposed to be of neuroendocrine origin. In this study, we show that while the proliferating preadipocytes or mature adipocytes are nonciliated in culture, a typical primary cilium is present in differentiating preadipocytes. This transient cilium carries receptors for Wnt and Hedgehog pathways, linking this organelle to previously described regulatory pathways of adipogenesis. We also show that the BBS10 and BBS12 proteins are located within the basal body of this primary cilium and inhibition of their expression impairs ciliogenesis, activates the glycogen synthase kinase 3 pathway, and induces peroxisome proliferator-activated receptor nuclear accumulation, hence favoring adipogenesis. Moreover, adipocytes derived from BBS-patients' dermal fibroblasts in culture exhibit higher propensity for fat accumulation when compared to controls. This strongly suggests that a peripheral primary dysfunction of adipogenesis participates to the pathogenesis of obesity in BBS. PMID:19190184

  2. Hydrometrocolpos and polydactyly: a common neonatal presentation of Bardet-Biedl and McKusick-Kaufman syndromes

    OpenAIRE

    David, A; Bitoun, P.; Lacombe, D.; Lambert, J; Nivelon, A.; Vigneron, J.; Verloes, A

    1999-01-01

    McKusick-Kaufman syndrome (MKKS) is a rare, recessively inherited syndrome reported mainly in young children and is characterised by vaginal atresia with hydrometrocolpos, postaxial polydactyly, and congenital heart defect. Bardet-Biedl syndrome (BBS) is the generic name for a genetically heterogeneous group of autosomal recessive disorders characterised by retinal dystrophy or retinitis pigmentosa (appearing usually between 10 and 20 years of age), postaxial polydactyly, obesity, nephropathy...

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

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    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.

  4. Mapping of Genes Involved in Bardet-Biedl Syndrome (BBS in Pakistani Population

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    Shiraz Ahmad

    2012-07-01

    Full Text Available Bardet-Biedl Syndrome (BBS, one of an autosomal recessive or clinically and genetically heterogeneous disorder, which prevails all over the world and results due to increased rate of consanguinity. All of these BBS genes are involved either directly or indirectly in signaling pathways such as Leptin receptor signaling pathway and Wnt signaling pathway. The study presented here includes genetic mapping of two consanguineous families (A & B with BBS. (21.63-Mb region was found to be critical as it was gene rich and contains approximately eighty known and predicted genes. Out of eighty genes six (FGF2, BBS7, BBS12, NUDT6, SPATA5 and SPRY1 were found to be candidate genes. On mutations screening, sequencing of the coding exon 2 of BBS12 in affected individuals identified a novel homozygous c.2103C 1 A mutation, which is predicted to insert a stop codon at position 701 of the BBS12 protein (p.S701X. Identification of BBS12 mutation in families B can increase our understanding of molecular genetics and pathophysiology of BBS.

  5. Phenotypic differences among patients with Bardet-Biedl syndrome linked to three different chromosome loci

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    Carmi, R.; Elbedour, K. [Ben-Gurion Univ., Beer-Sheva (Israel); Stone, E.M.; Sheffield, V.C. [Univ. of Iowa, IA (United States)

    1995-11-06

    Bardet-Biedl syndrome (BBS) is an autosomal-recessive disorder of mental retardation, obesity, retinal dystrophy, polydactyly, and hypogenitalism. Renal and cardiac abnormalities are also frequent in this disorder. Previous clinical suggestions of heterogeneity of BBS were confirmed recently by the identification of four different chromosome loci linked to the disease. In this study we compared clinical manifestations of the syndrome in patients form 3 unrelated, extended Arab-Bedouin kindreds which were used for the linkage mapping of the BBS loci to chromosomes 3, 15, and 16. The observed differences included the limb distribution of the postaxial polydactyly and the extent and age-association of obesity. It appears that the chromosome 3 locus is associated with polydactyly of all four limbs, while polydactyly of the chromosome 15 type is mostly confined to the hands. On the other hand, the chromosome 15 type is associated with early-onset morbid obesity, while the chromosome 16 type appears to present the {open_quotes}leanest{close_quotes} form of BBS. Future cloning of the various BB genes will contribute to the understanding of the molecular basis of limb development and the identification of human obesity-related genes. 22 refs., 1 fig., 4 tabs.

  6. Pitfalls of homozygosity mapping: an extended consanguineous Bardet-Biedl syndrome family with two mutant genes (BBS2, BBS10), three mutations, but no triallelism.

    Science.gov (United States)

    Laurier, Virginie; Stoetzel, Corinne; Muller, Jean; Thibault, Christelle; Corbani, Sandra; Jalkh, Nadine; Salem, Nabiha; Chouery, Eliane; Poch, Olivier; Licaire, Serge; Danse, Jean-Marc; Amati-Bonneau, Patricia; Bonneau, Dominique; Mégarbané, André; Mandel, Jean-Louis; Dollfus, Hélène

    2006-11-01

    The extensive genetic heterogeneity of Bardet-Biedl syndrome (BBS) is documented by the identification, by classical linkage analysis complemented recently by comparative genomic approaches, of nine genes (BBS1-9) that account cumulatively for about 50% of patients. The BBS genes appear implicated in cilia and basal body assembly or function. In order to find new BBS genes, we performed SNP homozygosity mapping analysis in an extended consanguineous family living in a small Lebanese village. This uncovered an unexpectedly complex pattern of mutations, and led us to identify a novel BBS gene (BBS10). In one sibship of the pedigree, a BBS2 homozygous mutation was identified, while in three other sibships, a homozygous missense mutation was identified in a gene encoding a vertebrate-specific chaperonine-like protein (BBS10). The single patient in the last sibship was a compound heterozygote for the above BBS10 mutation and another one in the same gene. Although triallelism (three deleterious alleles in the same patient) has been described in some BBS families, we have to date no evidence that this is the case in the present family. The analysis of this family challenged linkage analysis based on the expectation of a single locus and mutation. The very high informativeness of SNP arrays was instrumental in elucidating this case, which illustrates possible pitfalls of homozygosity mapping in extended families, and that can be explained by the rather high prevalence of heterozygous carriers of BBS mutations (estimated at one in 50 in Europeans). PMID:16823392

  7. Cognitive, sensory, and psychosocial characteristics in patients with Bardet-Biedl syndrome.

    Science.gov (United States)

    Brinckman, Danielle D; Keppler-Noreuil, Kim M; Blumhorst, Catherine; Biesecker, Leslie G; Sapp, Julie C; Johnston, Jennifer J; Wiggs, Edythe A

    2013-12-01

    Forty-two patients with a clinical diagnosis of Bardet-Biedl syndrome ages 2-61 years were given a neuropsychological test battery to evaluate cognitive, sensory, and behavioral functioning. These tests included the Wechsler scales of intelligence, Rey Auditory Verbal Learning Test, Boston Naming Test, D-KEFS Verbal Fluency Test, D-KEFS Color-Word Interference Test, D-KEFS Sorting Test, Wide Range Achievement Test: Math and Reading Subtests, Purdue Pegboard, The University of Pennsylvania Smell Identification Test, Social Communication Questionnaire, Social Responsiveness Scale, and Behavior Assessment System for Children, Second Edition, Parent Rating Scale. On the age appropriate Wechsler scale, the mean Verbal Comprehension was 81 (n = 36), Working Memory was 81 (n = 36), Perceptual Reasoning was 78 (n = 24) and Full Scale IQ was 75 (n = 26). Memory for a word list (Rey Auditory Verbal Learning Test) was in the average range with a mean of 89 (n = 19). Fine motor speed was slow on the Purdue with mean scores 3-4 standard deviations below norms. All subjects were microsmic on the University of Pennsylvania Smell Identification Test. Of these 42 patients, only 6 were able to complete all auditory and visual tests; 52% were unable to complete the visual tests due to impaired vision. A wide range of behavioral issues were endorsed on questionnaires given to parents. Most had social skill deficits but no pattern of either externalizing or internalizing problems. We identify a characteristic neuro-behavioral profile in our cohort comprised of reduced IQ, impaired fine-motor function, and decreased olfaction. PMID:24194441

  8. Identification of 28 novel mutations in the Bardet-Biedl syndrome genes: the burden of private mutations in an extensively heterogeneous disease.

    Science.gov (United States)

    Muller, Jean; Stoetzel, C; Vincent, M C; Leitch, C C; Laurier, V; Danse, J M; Hellé, S; Marion, V; Bennouna-Greene, V; Vicaire, S; Megarbane, A; Kaplan, J; Drouin-Garraud, V; Hamdani, M; Sigaudy, S; Francannet, C; Roume, J; Bitoun, P; Goldenberg, A; Philip, N; Odent, S; Green, J; Cossée, M; Davis, E E; Katsanis, N; Bonneau, D; Verloes, A; Poch, O; Mandel, J L; Dollfus, H

    2010-03-01

    Bardet-Biedl syndrome (BBS), an emblematic disease in the rapidly evolving field of ciliopathies, is characterized by pleiotropic clinical features and extensive genetic heterogeneity. To date, 14 BBS genes have been identified, 3 of which have been found mutated only in a single BBS family each (BBS11/TRIM32, BBS13/MKS1 and BBS14/MKS4/NPHP6). Previous reports of systematic mutation detection in large cohorts of BBS families (n > 90) have dealt only with a single gene, or at most small subsets of the known BBS genes. Here we report extensive analysis of a cohort of 174 BBS families for 12/14 genes, leading to the identification of 28 novel mutations. Two pathogenic mutations in a single gene have been found in 117 families, and a single heterozygous mutation in 17 families (of which 8 involve the BBS1 recurrent mutation, M390R). We confirm that BBS1 and BBS10 are the most frequently mutated genes, followed by BBS12. No mutations have been found in BBS11/TRIM32, the identification of which as a BBS gene only relies on a single missense mutation in a single consanguineous family. While a third variant allele has been observed in a few families, they are in most cases missenses of uncertain pathogenicity, contrasting with the type of mutations observed as two alleles in a single gene. We discuss the various strategies for diagnostic mutation detection, including homozygosity mapping and targeted arrays for the detection of previously reported mutations. PMID:20177705

  9. McKusick-Kaufman or Bardet-Biedl syndrome? A new borderline case in an Italian nonconsanguineous healthy family

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    Massimiliano Chetta

    2011-01-01

    Full Text Available McKusick-Kaufman syndrome (MKS, OMIM #236700 is a rare syndrome inherited in an autosomal recessive pattern with a phenotypic triad comprising hydrometrocolpos (HMC, postaxial polydactyly (PAP, and congenital cardiac disease (CHD. The syndrome is caused by mutations in the MKKS gene mapped onto chromosome 20p12 between D20S162 and D20S894 markers. Mutations in the same gene causes Bardet-Biedl-6 syndrome (BBS-6, OMIM #209900 inherited in an autosomal recessive pattern. BBS-6 comprises retinitis pigmentosa, polydactyly, obesity, mental retardation, renal and genital anomalies. HMC, CHD, and PAP defects can also occur in BBS-6, and there is a significant clinical overlap between MKS and BBS-6 in childhood. We describe a new borderline case of MKS and BBS syndrome and suggest insights for understanding correlation between MKKS gene mutations and clinical phenotype. Here, we report the results of molecular analysis of MKKS in a female proband born in an Italian nonconsanguineous healthy family that presents HMC and PAP. The mutational screening revealed the presence of two different heterozygous missense variants (p.242A>S in exon 3, p.339 I>V in exon 4 in the MKKS gene, and a nucleotide variation in 5′UTR region in exon 2 (-417 A>C.

  10. Value of MRI olfactory bulb evaluation in the assessment of olfactory dysfunction in Bardet-Biedl syndrome.

    Science.gov (United States)

    Braun, J J; Noblet, V; Kremer, S; Molière, S; Dollfus, H; Marion, V; Goetz, N; Muller, J; Riehm, S

    2016-07-01

    Olfactory bulb (OB) volume evaluation by magnetic resonance imaging (MRI) has been demonstrated to be related to olfactory dysfunction in many different diseases. Olfactory dysfunction is often overlooked in Bardet-Biedl syndrome (BBS) patients and is rarely objectively evaluated by MRI. We present a series of 20 BBS patients with olfactory dysfunction. The OB was evaluated separately and blindly by two radiologists (SR and SM) with 3 Tesla MRI imaging comparatively to 12 normal control subjects by global visual evaluation and by quantitative measurement of OB volume. In the 12 control cases OB visual evaluation was considered as normal in all cases for radiologist (SR) and in 10 cases for radiologist (SM). In the 20 BBS patients, OB visual evaluation was considered as abnormal in 18 cases for SR and in all cases for SM. OB volumetric evaluation for SR and SM in BBS patients was able to provide significant correlation between BBS and olfactory dysfunction. This study indicates that OB volume evaluation by MRI imaging like structural MRI scan for gray matter modifications demonstrates that olfactory dysfunction in BBS patients is a constant and cardinal symptom integrated in a genetical syndrome with peripheral and central olfactory structure alterations. PMID:26586152

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

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    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

  12. 巴德-毕氏综合征研究的现状及意义%Current status and implication of research on Bardet-Biedl syndrome

    Institute of Scientific and Technical Information of China (English)

    沈涛; 严新民; 肖春杰

    2013-01-01

    Bardet-Biedl syndrome (BBS) is a rare autosomal recessive disease initially reported by Bardet and Biedl in the 1920s.BBS is a pleiotropic and genetically heterogeneous disorder characterized by retinopathy,obesity,polydactyly,renal malformations and functional abnormalities,learning disabilities and hypogenitalism.BBS patients are also prone to diabetes mellitus,hypertension and congenital heart disease.To date,16 BBS genes (BBS1-BBS16)have been identified.However,the molecular etiology of BBS is not yet entirely clear.In this article,we have reviewed recent research on BBS and discussed its implications for understanding of ciliopathology.%巴德-毕氏综合征(Bardet-Biedl syndrome,BBS)是一种罕见的常染色体隐性遗传疾病,由Bardet 和Biedl在1920年首次报道.BBS具有高度的遗传异质性,其临床症状包括视网膜营养不良、肥胖、多指/趾、肾畸形或肾功异常、学习障碍及性腺发育不全等.BBS患者常伴糖尿病、高血压及先心病等继发症状.迄今已发现16个BBS基因(BBS1~BBS16)异常可以导致BBS表型,但其分子致病机制仍不完全清楚.

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

    OpenAIRE

    Valavi Ehsan; Ansari Mohammad Javad; Ahmadzadeh Ali

    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 menta...

  14. Targeted high-throughput sequencing for diagnosis of genetically heterogeneous diseases: efficient mutation detection in Bardet-Biedl and Alström Syndromes

    Science.gov (United States)

    Redin, Claire; Le Gras, Stéphanie; Mhamdi, Oussema; Geoffroy, Véronique; Stoetzel, Corinne; Vincent, Marie-Claire; Chiurazzi, Pietro; Lacombe, Didier; Ouertani, Ines; Petit, Florence; Till, Marianne; Verloes, Alain; Jost, Bernard; Chaabouni, Habiba Bouhamed; Dollfus, Helene; Mandel, Jean-Louis; Muller, Jean

    2012-01-01

    Background Bardet-Biedl syndrome (BBS) is a pleiotropic recessive disorder that belongs to the rapidly growing family of ciliopathies. It shares phenotypic traits with other ciliopathies, such as Alström syndrome (ALMS), nephronophthisis (NPHP) or Joubert syndrome. BBS mutations have been detected in 16 different genes (BBS1-BBS16) without clear genotype-to-phenotype correlation. This extensive genetic heterogeneity is a major concern for molecular diagnosis and genetic counselling. While various strategies have been recently proposed to optimise mutation detection, they either fail to detect mutations in a majority of patients or are time consuming and costly. Method We tested a targeted exon-capture strategy coupled with multiplexing and high-throughput sequencing on 52 patients: 14 with known mutations as proof-of-principle and 38 with no previously detected mutation. Thirty genes were targeted in total including the 16 BBS genes, the 12 known NPHP genes, the single ALMS gene ALMS1 and the proposed modifier CCDC28B. Results This strategy allowed the reliable detection of causative mutations (including homozygous/heterozygous exon deletions) in 68% of BBS patients without previous molecular diagnosis and in all proof-of-principle samples. Three probands carried homozygous truncating mutations in ALMS1 confirming the major phenotypic overlap between both disorders. The efficiency of detecting mutations in patients was positively correlated with their compliance with the classical BBS phenotype (mutations were identified in 81% of ‘classical’ BBS patients) suggesting that only a few true BBS genes remain to be identified. We illustrate some interpretation problems encountered due to the multiplicity of identified variants. Conclusion This strategy is highly efficient and cost effective for diseases with high genetic heterogeneity, and guarantees a quality of coverage in coding sequences of target genes suited for diagnosis purposes. PMID:22773737

  15. Identification of a novel BBS gene (BBS12) highlights the major role of a vertebrate-specific branch of chaperonin-related proteins in Bardet-Biedl syndrome.

    Science.gov (United States)

    Stoetzel, Corinne; Muller, Jean; Laurier, Virginie; Davis, Erica E; Zaghloul, Norann A; Vicaire, Serge; Jacquelin, Cecile; Plewniak, Frederic; Leitch, Carmen C; Sarda, Pierre; Hamel, Christian; de Ravel, Thomy J L; Lewis, Richard Alan; Friederich, Evelyne; Thibault, Christelle; Danse, Jean-Marc; Verloes, Alain; Bonneau, Dominique; Katsanis, Nicholas; Poch, Olivier; Mandel, Jean-Louis; Dollfus, Helene

    2007-01-01

    Bardet-Biedl syndrome (BBS) is primarily an autosomal recessive ciliopathy characterized by progressive retinal degeneration, obesity, cognitive impairment, polydactyly, and kidney anomalies. The disorder is genetically heterogeneous, with 11 BBS genes identified to date, which account for ~70% of affected families. We have combined single-nucleotide-polymorphism array homozygosity mapping with in silico analysis to identify a new BBS gene, BBS12. Patients from two Gypsy families were homozygous and haploidentical in a 6-Mb region of chromosome 4q27. FLJ35630 was selected as a candidate gene, because it was predicted to encode a protein with similarity to members of the type II chaperonin superfamily, which includes BBS6 and BBS10. We found pathogenic mutations in both Gypsy families, as well as in 14 other families of various ethnic backgrounds, indicating that BBS12 accounts for approximately 5% of all BBS cases. BBS12 is vertebrate specific and, together with BBS6 and BBS10, defines a novel branch of the type II chaperonin superfamily. These three genes are characterized by unusually rapid evolution and are likely to perform ciliary functions specific to vertebrates that are important in the pathophysiology of the syndrome, and together they account for about one-third of the total BBS mutational load. Consistent with this notion, suppression of each family member in zebrafish yielded gastrulation-movement defects characteristic of other BBS morphants, whereas simultaneous suppression of all three members resulted in severely affected embryos, possibly hinting at partial functional redundancy within this protein family. PMID:17160889

  16. Genetics Home Reference: Bardet-Biedl syndrome

    Science.gov (United States)

    ... vision. Over time, these blind spots enlarge and merge to produce tunnel vision. Most people with Bardet- ... result from mutations in at least 14 different genes (often called BBS genes). These genes are known ...

  17. [Prenatal discovery of Joubert syndrome associated with small bowel volvulus].

    Science.gov (United States)

    Aurégan, C; Donciu, V; Millischer, A-E; Khen-Dunlop, N; Deloison, B; Sonigo, P; Magny, J-F

    2016-03-01

    Joubert syndrome and prenatal volvulus are difficult to diagnose during pregnancy. Joubert syndrome and related diseases should be considered in case of prenatal abnormal features of the fourth ventricle. Small bowel volvulus is also a surgical emergency because of the risk of intestinal necrosis before or after delivery. This type of condition justifies the transfer of pregnant women to a specialized hospital where the newborn may receive appropriate care. We report the case of a 31-week and 4-day gestational-age fetus in whom intrauterine growth retardation and small-bowel volvulus were diagnosed. Additional imaging revealed associated Joubert syndrome. This highlights the need for regular ultrasound monitoring during pregnancy and the comanagement of obstetricians and pediatricians to provide appropriate care before and after delivery. PMID:26850151

  18. Inhibiting severe acute respiratory syndrome-associated coronavirus by small interfering RNA

    Institute of Scientific and Technical Information of China (English)

    张仁礼; 郭中敏; 陆家海; 孟锦绣; 周灿权; 詹希美; 黄冰; 余新炳; 黄民; 潘兴华; 凌文华; 陈系古; 万卓越; 郑焕英; 鄢心革; 王一飞; 冉延超; 刘新健; 马俊鑫; 王承宇; 张必良

    2003-01-01

    Objective To evaluate the effectiveness of small interfering RNA (siRNA) on inhibiting severe acute respiratory syndrome (SARS)-associated coronavirus replication, and to lay bases for the future clinical application of siRNA for the treatment of viral infectious diseases.Methods Vero-E6 cells was transfected with siRNA before SARS virus infection, and the effectiveness of siRNA interference was evaluated by observing the cytopathic effect (CPE) on Vero-E6 cells.Results Five pairs of siRNA showed ability to reduce CPE dose dependently, and two of them had the best effect. Conclusion siRNA may be effective in inhibiting SARS-associated coronavirus replication.

  19. Chromosomal and related Mendelian syndromes associated with Hirschsprung's disease.

    Science.gov (United States)

    Moore, S W

    2012-11-01

    Hirschsprung's disease (HSCR) is a fairly frequent cause of intestinal obstruction in children. It is characterized as a sex-linked heterogonous disorder with variable severity and incomplete penetrance giving rise to a variable pattern of inheritance. Although Hirschsprung's disease occurs as an isolated phenotype in at least 70% of cases, it is not infrequently associated with a number of congenital abnormalities and associated syndromes, demonstrating a spectrum of congenital anomalies. Certain of these syndromic phenotypes have been linked to distinct genetic sites, indicating underlying genetic associations of the disease and probable gene-gene interaction, in its pathogenesis. These associations with HSCR include Down's syndrome and other chromosomal anomalies, Waardenburg syndrome and other Dominant sensorineural deafness, the Congenital Central Hypoventilation and Mowat-Wilson and other brain-related syndromes, as well as the MEN2 and other tumour associations. A number of other autosomal recessive syndromes include the Shah-Waardenburg, the Bardet-Biedl and Cartilage-hair hypoplasia, Goldberg-Shprintzen syndromes and other syndromes related to cholesterol and fat metabolism among others. The genetics of Hirschsprung's disease are highly complex with the majority of known genetic sites relating to the main susceptibility pathways (RET an EDNRB). Non-syndromic non-familial, short-segment HSCR appears to represent a non-Mendelian condition with variable expression and sex-dependent penetrance. Syndromic and familial forms, on the other hand, have complex patterns of inheritance and being reported as autosomal dominant, recessive and polygenic patterns of inheritance. The phenotypic variability and incomplete penetrance observed in Hirschsprung's disease could also be explained by the involvement of modifier genes, especially in its syndromic forms. In this review, we look at the chromosomal and Mendelian associations and their underlying signalling pathways

  20. 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 Dise...s (C10.228.140.617) > Bardet-Biedl Syndrome (C10.228.140.617.200) Congenital, Hereditary, and Neonatal Disea... Multiple (C16.131.077) > Bardet-Biedl Syndrome (C16.131.077.112) 99A0284114 ...

  1. Miller Fisher syndrome associated with Q fever.

    OpenAIRE

    Diaz Ortuño, A; Maeztu, C; Muñoz, J. A.; Reigadas, R; Rodriguez, T.; Valdés, M.

    1990-01-01

    A 51 year old woman with pneumonia developed Miller Fisher syndrome. Indirect immunofluorescence tests showed antibodies against Coxiella burnetti. Miller Fisher syndrome associated with Q fever has not been described previously.

  2. 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.

  3. 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 Syst...Diseases (C10.228.140.617) > Bardet-Biedl Syndrome (C10.228.140.617.200) Congenital, Hereditary, and Neonata...lities, Multiple (C16.131.077) > Bardet-Biedl Syndrome (C16.131.077.112) 99A0284114 ...

  4. [A case of Sweet's syndrome associated with Crohn's disease].

    Science.gov (United States)

    Gonzálvez Perales, J L; Tamarit Ortí, R; Ballester Fayos, J; Jiménez Martínez, A; Antón Conejero, M D; Rodríguez Gil, F J; Moreno Osset, E; González Martínez, M A

    1997-03-01

    The Sweet syndrome or acute febrile neutrophilic dermatosis is a well characterized cutaneous disease from a clinical and histological point of view and is frequently associated with systemic diseases. Prognosis is favorable with good response to corticoid therapy. A well documented case of Sweet syndrome associated with an outbreak of Crohn's disease with peculiar good therapeutic response is reported. PMID:9162534

  5. Active transport and diffusion barriers restrict Joubert Syndrome-associated ARL13B/ARL-13 to an Inv-like ciliary membrane subdomain.

    Directory of Open Access Journals (Sweden)

    Sebiha Cevik

    Full Text Available Cilia are microtubule-based cell appendages, serving motility, chemo-/mechano-/photo- sensation, and developmental signaling functions. Cilia are comprised of distinct structural and functional subregions including the basal body, transition zone (TZ and inversin (Inv compartments, and defects in this organelle are associated with an expanding spectrum of inherited disorders including Bardet-Biedl syndrome (BBS, Meckel-Gruber Syndrome (MKS, Joubert Syndrome (JS and Nephronophthisis (NPHP. Despite major advances in understanding ciliary trafficking pathways such as intraflagellar transport (IFT, how proteins are transported to subciliary membranes remains poorly understood. Using Caenorhabditis elegans and mammalian cells, we investigated the transport mechanisms underlying compartmentalization of JS-associated ARL13B/ARL-13, which we previously found is restricted at proximal ciliary membranes. We now show evolutionary conservation of ARL13B/ARL-13 localisation to an Inv-like subciliary membrane compartment, excluding the TZ, in many C. elegans ciliated neurons and in a subset of mammalian ciliary subtypes. Compartmentalisation of C. elegans ARL-13 requires a C-terminal RVVP motif and membrane anchoring to prevent distal cilium and nuclear targeting, respectively. Quantitative imaging in more than 20 mutants revealed differential contributions for IFT and ciliopathy modules in defining the ARL-13 compartment; IFT-A/B, IFT-dynein and BBS genes prevent ARL-13 accumulation at periciliary membranes, whereas MKS/NPHP modules additionally inhibit ARL-13 association with TZ membranes. Furthermore, in vivo FRAP analyses revealed distinct roles for IFT and MKS/NPHP genes in regulating a TZ barrier to ARL-13 diffusion, and intraciliary ARL-13 diffusion. Finally, C. elegans ARL-13 undergoes IFT-like motility and quantitative protein complex analysis of human ARL13B identified functional associations with IFT-B complexes, mapped to IFT46 and IFT74

  6. Congenital Anomalies of the Kidney and the Urinary Tract (CAKUT).

    Science.gov (United States)

    Rodriguez, Maria M

    2014-01-01

    This article reviews the majority of Congenital Anomalies of the Kidney and Urinary Tract (CAKUT) with emphasis in Pediatric Pathology describing and illustrating lesions as varied as ureteral duplications, ureteropelvic junction obstruction, horseshoe kidney, posterior urethral valve and prune belly syndrome, obstructive renal dysplasia, nonmotile ciliopathies and several syndromes associated with renal malformations (Meckel-Joubert, short rib, Bardet-Biedl, asplenia/polysplenia, hereditary renal adysplasia, Zellweger, trisomies, VACTER-L, Potter, caudal dysplasia, and sirenomelia), as well as ADPK, and ARPK. The purpose of this review is not only to describe the congenital renal anomalies, but also to analyze the more recent therapeutic interventions that may modify the natural history of some of these severe conditions. PMID:25313840

  7. Secondary Dystonia-Clinical Clues and Syndromic Associations

    Directory of Open Access Journals (Sweden)

    Susanne A Schneider

    2009-10-01

    Full Text Available Background: Dystonia is a hyperkinetic movement disorder defined by involuntary sustained muscle spasms and unusual postures. Etiologically, dystonic syndromes can be broadly divided into primary and secondary forms, dystonia-plus syndromes and heredodegenerative forms. In particular, diagnosis of secondary dystonic syndromes can be challenging in view of the variety of causes. Purpose: The purpose of this article is to highlight some clinical clues and syndromic associations as well as investigational findings which may be helpful in the approach to a patient with suspected secondary dystonia. Methods: We outline characteristic clinical and neuroimaging findings which may be directive in the diagnostic process of dystonia patients and facilitate making the correct diagnosis, thus allowing initiating the best treatment. Results: Secondary causes of dystonia include, among others, strategic brain lesions of various origins, metabolic disease, neurodegenerative conditions, and previous exposure to drugs or toxins. Presence of clinical signs including prominent oromandibular involvement, eye movement disorders, retinitis pigmentosa, deafness, peripheral neuropathy, parkinsonism or progressive dementia should alert the clinician to consider a secondary cause. Strategic lesions within the basal ganglia, but also within the brainstem, cerebellum or cortical areas may underlie dystonia and should thus be excluded. Conclusions: When thorough clinical examination reveals features atypical of primary dystonia, syndromic associations may help the clinician to narrow down the list of differential diagnosis. Directive investigations like neuroimaging may confirm the clinical suspicion.

  8. 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.

  9. 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

  10. Currarino syndrome associated with Hirschsprung's disease: Case report and literature review

    OpenAIRE

    Shigeyuki Furuta; Hideaki Sato; Shiho Hamano; Hiroaki Kitagawa

    2015-01-01

    We present a rare family consisting of a 10-month-old boy with complete Currarino syndrome associated with Hirschsprung's disease. His mother had been diagnosed as having incomplete Currarino syndrome. Only 11 cases of Currarino syndrome associated with Hirschsprung's disease have been reported in the literature. Our report suggests that it is necessary to perform preoperative examinations to rule out Hirschsprung's disease, if the patient presents with a history of severe defecation disorder...

  11. 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.

  12. A case of swyer syndrome associated with advanced gonadal dysgerminoma involving long survival.

    Science.gov (United States)

    Da Silva Rios, Salete; Monteiro, Isabella Christina Mazzaro; Braz Dos Santos, Larissa Gonçalves; Caldas, Natasha Garcia; Chen, Ana Carolina Rios; Chen, Juliana Rios; Silva, Helena Spindola Camargo

    2015-01-01

    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. PMID:25960730

  13. Disease: H00910 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available denburg syndrome [DS:H00759] Mowat-Wilson syndrome [DS:H...Following syndromes are Hirschsprung-like phenotypes. Bardet-Biedl syndrome [DS:H00418] Shprintzen-Goldberg syndrome [DS:H00659] Waar

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

    Science.gov (United States)

    2016-01-26

    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

  15. Churg-Strauss Syndrome associated with montelukast: Three Case Reports

    OpenAIRE

    Yıldız, Fatih; Zorludemir, Suzan; Erken, Eren

    2014-01-01

    Churg-Strauss syndrome (new name Eosinophilic granulomatosis and polyangiitis); asthma, fever, peripheral blood eosinophilia, eosinophilic tissue infiltration, small and medium sized arteries characterized by necrotizing granulomatous inflammation is a multisystemic disorder. Classified in ANCA associated vasculitis. The drugs such as leukotriene receptor antagonists (montelukast, zafirlukast, pranlukast), inhaled glucocorticoids, omalizumab, cocaine and clarithromycin is thought to be associ...

  16. 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. PMID:27433451

  17. Nephrotic Syndrome Associated with Lung Cancer: A Rare Case of Malignancy Associated with AA Amyloidosis

    Science.gov (United States)

    Gueutin, Victor; Langlois, Anne-Lyse; Shehwaro, Nathalie; Elharraqui, Ryme; Rouvier, Philippe; Izzedine, Hassane

    2013-01-01

    Nonhematologic malignancies are rarely reported to be associated with AA amyloidosis. Although the association between renal cell carcinoma and systemic AA amyloidosis has been established, the evidence linking pulmonary cancer to AA amyloidosis is scarce. Here, a case of biopsy-proven renal AA amyloidosis complicated with nephrotic syndrome associated with lung carcinoma is reported. PMID:24558629

  18. Nephrotic Syndrome Associated with Lung Cancer: A Rare Case of Malignancy Associated with AA Amyloidosis

    OpenAIRE

    Victor Gueutin; Anne-Lyse Langlois; Nathalie Shehwaro; Ryme Elharraqui; Philippe Rouvier; Hassane Izzedine

    2013-01-01

    Nonhematologic malignancies are rarely reported to be associated with AA amyloidosis. Although the association between renal cell carcinoma and systemic AA amyloidosis has been established, the evidence linking pulmonary cancer to AA amyloidosis is scarce. Here, a case of biopsy-proven renal AA amyloidosis complicated with nephrotic syndrome associated with lung carcinoma is reported.

  19. Nephrotic Syndrome Associated with Lung Cancer: A Rare Case of Malignancy Associated with AA Amyloidosis

    Directory of Open Access Journals (Sweden)

    Victor Gueutin

    2013-01-01

    Full Text Available Nonhematologic malignancies are rarely reported to be associated with AA amyloidosis. Although the association between renal cell carcinoma and systemic AA amyloidosis has been established, the evidence linking pulmonary cancer to AA amyloidosis is scarce. Here, a case of biopsy-proven renal AA amyloidosis complicated with nephrotic syndrome associated with lung carcinoma is reported.

  20. Your heart on a chip: iPSC-based modeling of Barth-syndrome-associated cardiomyopathy.

    Science.gov (United States)

    Zweigerdt, Robert; Gruh, Ina; Martin, Ulrich

    2014-07-01

    Disease-specific induced pluripotent stem cells (iPSCs) are invaluable tools for studying genetic disorders in a dish. A recent paper by Wang et al. (2014) powerfully combines analysis of human iPSCs with genome editing and tissue engineering, in conjunction with biochemical and physiological assays, to provide insights into Barth-syndrome-associated cardiomyopathy. PMID:24996164

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

    Directory of Open Access Journals (Sweden)

    Paulo Sérgio Gonçalves da Costa

    2011-10-01

    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.

  2. Ramsay Hunt Syndrome Associated with Central Nervous System Involvement in an Adult.

    Science.gov (United States)

    Chan, Tommy L H; Cartagena, Ana M; Bombassaro, Anne Marie; Hosseini-Moghaddam, Seyed M

    2016-01-01

    Ramsay Hunt syndrome associated with varicella zoster virus reactivation affecting the central nervous system is rare. We describe a 55-year-old diabetic female who presented with gait ataxia, right peripheral facial palsy, and painful vesicular lesions involving her right ear. Later, she developed dysmetria, fluctuating diplopia, and dysarthria. Varicella zoster virus was detected in the cerebrospinal fluid by polymerase chain reaction. She was diagnosed with Ramsay Hunt syndrome associated with spread to the central nervous system. Her facial palsy completely resolved within 48 hours of treatment with intravenous acyclovir 10 mg/kg every 8 hours. However, cerebellar symptoms did not improve until a tapering course of steroid therapy was initiated. PMID:27366189

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

    OpenAIRE

    Da Silva Rios, Salete; Monteiro, Isabella Christina Mazzaro; Braz dos Santos, Larissa Gonçalves; Caldas, Natasha Garcia; Chen, Ana Carolina Rios; Chen, Juliana Rios; Silva, Helena Spindola Camargo

    2015-01-01

    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 ...

  4. Evaluation of prophilaxis of dry eye syndrome associated with soft contact lenses

    Directory of Open Access Journals (Sweden)

    Erkin Bilalov

    2013-12-01

    Full Text Available The purpose of the paper was to investigate the possibilities of dry eye syndrome prevention associated with soft contact lenses wearing. The algorithm of dry eye syndrome diagnostics and soft contact lenses selection has been developed based on the results of this work. The research showed that “Slezol Forte” is highly effective in the prevention of dry eye syndrome associated with wearing of soft contact lenses.

  5. A postmenopausal woman presenting with Ekbom syndrome associated with recurrent depressive disorder: a case report

    OpenAIRE

    Mahler, Courtney; MacQueen, Glenda; Samaan, Zainab

    2008-01-01

    Background Ekbom syndrome is a rare psychiatric disorder that can manifest as a delusion, overvalued idea or hallucination of parasitic infestations. It is more prevalent in postmenopausal women and patients are usually seeking dermatology rather than psychiatry consultation for their symptoms. Case presentation We present a case of Ekbom syndrome associated with recurrent depressive disorder in an elderly patient. The patient presented with tactile hallucinations of insects crawling just und...

  6. De Quervain's syndrome associated with osteopoikilosis: a case report and review of the literature.

    Science.gov (United States)

    Kaparov, Asylbek; Uludag, Murat; Sari, Hidayet; Akarirmak, Ulkü

    2011-06-01

    Osteopoikilosis is a rare, usually asymptomatic, autosomal-dominant bone disorder, which is generally diagnosed incidentally on X-ray. De Quervain's syndrome is a disorder characterized by pain on the radial (thumb) side of the wrist, impairment of thumb function and thickening of the ligamentous structure covering the tendons in the first dorsal compartment of the wrist. In this case report, we present a young woman with De Quervain's syndrome associated with osteopoikilosis. PMID:19856179

  7. Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia

    OpenAIRE

    Saeed, Muhammad; Anwar ul HAQ; Khaqan QADIR

    2014-01-01

    How to Cite This Article: Saeed M, Haq A, Qadir Kh.Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia. Iran J Child Neurol. 2014 Autumn;8(4): 76-79.AbstractObjectiveBart’s syndrome is defined as congenital localized absence of skin, and associated with epidermolysis bullosa. A newborn with Bart’s syndrome is reported because it is a very rare condition, especially when associated with corpus callosum agenesis and concomitant choanal atresia. Clinically it is characterized...

  8. [Hypokalemic paralysis revealing Sjögren's syndrome associated with auto-immune thyroiditis].

    Science.gov (United States)

    Baaj, Mohamed; Safi, Somaya; Hassikou, Hassna; Tabache, Fatima; Mouden, Karim; Hadri, Larbi

    2010-02-01

    We report a case of 36-year-old woman, admitted for hypotonic tetraparesis. Laboratory tests revealed severe hypokalaemia, acidosis, hyperchloremia and alkaline urinary pH allowing the diagnosis of distal tubular acidosis. Additional investigations led to the diagnosis of primary Sjögren's syndrome associated with Hashimoto's thyroïditis. The evolution was favorable under potassium citrate alkalinisation, the corticosteroid therapy and hormonal substitution. Based on this observation, the pathogenesis of distal tubular acidosis during auto-immune diseases (Sjögren's syndrome, monoclonal hypergammaglobulinemia, hypothyroidism) was discussed as well as its consequences and management. PMID:19836323

  9. Antiphospholipid syndrome associated with non-infective mitral valve endocarditis: a case report

    Directory of Open Access Journals (Sweden)

    Dragan Stevanović

    2014-04-01

    Full Text Available We present a rare case of antiphospholipid syndrome associated with non-infectious thrombotic endocarditis of the mitral valve. The patient was admitted to hospital for examination because of skin lesions manifested through a discoid skin rash. During the hospitalization antiphospholipid syndrome was diagnosed along with ultrasound verification of vegetations on the mitral valve, including both leaflets, with moderate to severe mitral regurgitation. Adequate and opportunely introduced therapy led to regression of all symptoms, including endocarditis of the mitral valve on checkup ultrasound verifications, with a prevention of arterial and/or venous thrombosis in patient's future.

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

    DEFF Research Database (Denmark)

    Jeppesen, Palle B

    2014-01-01

    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...... 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...... energy losses by approximately 0.8 MJ/day (∼200 kcal/day). In two subsequent 24-week, phase 3 studies, teduglutide reduced the need for parenteral support in the same magnitude. Teduglutide had an acceptable tolerability profile, where adverse events generally were of gastrointestinal origin consistent...

  11. Hypopituitarism and diabetes insipidus with localized hypertrophic pachymeningitis (Tolosa-Hunt syndrome) associated with Hashimoto thyroiditis.

    Science.gov (United States)

    Yamakita, Noriyoshi; Hanamoto, Takayuki; Muraoka, Noriaki; Ikeda, Tsuneko; Hirata, Toshifumi; Yasuda, Keigo; Sano, Toshiaki

    2004-01-01

    We report a 69-year-old woman with intracranial pachymeningitis showing hypopituitarism, diabetes insipidus, and Tolosa-Hunt syndrome associated with Hashimoto thyroiditis confirmed by autopsy. A large tumorous lesion of the hypothalamo-pituitary gland was revealed on magnetic resonance imaging, after the patient complained of gait and visual field disturbance. These symptoms subsided after thyroid hormone supplementation. Hypopituitarism and diabetes insipidus were diagnosed after cessation of the treatment by the patient herself. Multiple cranial nerve palsies and orbito-frontalgia appeared. Methylprednisolone pulse therapy improved the symptoms, but they recurred when the dose of glucocorticoid was decreased. The patient died of brain thrombosis. Autopsy revealed typical findings of Hashimoto thyroiditis and intracranial pachymeningitis involving the cranial base and pituitary gland. The high titer of rheumatoid factor and Hashimoto thyroiditis in this patient suggest an immunological role in the pathogenesis of pachymeningitis. PMID:14722395

  12. A case of 3p deletion syndrome associated with cerebellar hemangioblastoma.

    Science.gov (United States)

    Suzuki-Muromoto, Sato; Hino-Fukuyo, Naomi; Haginoya, Kazuhiro; Kikuchi, Atsuo; Sato, Hiroki; Sato, Yuko; Nakayama, Tojo; Kubota, Yuki; Kakisaka, Yosuke; Uematsu, Mitsugu; Kumabe, Toshihiro; Md, Shigeo Kure

    2016-02-01

    We described clinical course of a 24-year-old woman with 3p deletion syndrome associated with cerebellar hemangioblastoma at the age of 16 years old. She presented dysmorphic facial features, growth retardation and severe psychomotor retardation associated with 3p deletion syndrome. We identified de novo 3p deletion encompassing p25 by using array-based comparative genomic hybridization, where causative gene of von Hippel-Lindau (VHL) disease located. Surgical therapy for cerebellar hemangioblastoma was performed, and histological examination was consistent in cerebellar hemangioblastoma. She showed no other tumors associated VHL disease till 24 years old. This is the first case report of a patient with 3p deletion syndrome whose cerebellar hemangioblastoma may be associated with VHL disease. Repeat imaging studies were recommended for the patients with 3p deletion syndrome. PMID:26365017

  13. Syndromes associated with children exposure to mycotoxins and health risk assessment to multiple mycotoxins in infant foods

    OpenAIRE

    Alvito, Paula; Martins, Carla; Assunção, Ricardo; Pires, M.J.; Calhau, Maria Antónia

    2015-01-01

    1. Children health and mycotoxins; 2. Routes of exposure; 3. Syndromes Syndromes associated associated with children children exposure exposure to mycotoxins: to mycotoxins: ingestion and inhalation; 4. Health risk assessment to multiple mycotoxins in infant foods -MYCOMIX project (PTDC/DTP-FTO/0417/2012); 5. Critical role of health professionals

  14. Case Report: Myelodysplastic syndrome- associated myeloid sarcoma: an unusual clinical presentation of a rare disease.

    Science.gov (United States)

    Horvath, Emoke; Demian, Smaranda; Nagy, Elod

    2016-01-01

    Myeloid sarcoma results from the extramedullary homing and proliferation of immature myeloid precursors. We present the timeline, events and diagnostic pitfalls related to a 66 year-old male patient's case, admitted to the Hematology Clinic for pancytopenia, fever, weight loss and fatigue. The severe cytopenia and the few blasts observed in his blood smear indicated a bone marrow biopsy. The bone marrow showed hypercellularity and multilineage dysplasia with the presence of 15% myeloblasts. After the biopsy, he promptly developed paraplegia and nuclear magnetic resonance revealed an epidural tumour which was then resected.In the epidural tumour mass blast-like, round cells were observed with a complex immunophenotype, characterized by myeloperoxidase, CD117, CD15, CD99, leucocyte common antigen positivity and a high Ki-67 proliferation index. Considering the main differential diagnostic issues, the final diagnosis was stated as myelodysplastic syndrome-associated myeloid sarcoma. The prognosis was unfavourable, the bone marrow was quickly invaded by proliferating blast cells, and despite chemotherapy attempts, the patient died. PMID:27019694

  15. Predictors of immune reconstitution inflammatory syndrome associated with Kaposi's sarcoma: a case report.

    Science.gov (United States)

    Cattelan, Anna Maria; Mattiolo, Adriana; Grassi, Angela; Piano, Maria Assunta; Sasset, Lolita; Trevenzoli, Marco; Zanovello, Paola; Calabrò, Maria Luisa

    2016-01-01

    We present here a case of immune reconstitution inflammatory syndrome associated with Kaposi's sarcoma (KS-IRIS) developed in an AIDS patient two months after initiation of antiretroviral therapy (ART). Baseline characteristics of this IRIS-KS case, within a cohort of 12 naïve AIDS-KS patients, were analyzed. No statistically significant differences in CD4 cell counts, plasma HIV RNA load, KS clinical staging, human herpesvirus 8 (HHV8) antibody titers and HHV8 load in peripheral blood mononuclear cells and saliva were evidenced. HHV8 load in plasma was found to be significantly higher in the KS-IRIS patient (> 6 log10 genome equivalents/ml, p = 0.01, t-test) compared to the 11 patients with KS regression. This case highlights that measurement of HHV8 load in plasma may be useful to identify patients at risk for KS-IRIS, and that this parameter should be included in the design of larger studies to define KS-IRIS risk predictors. PMID:26848307

  16. 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.

  17. Immune reconstitution inflammatory syndrome associated with pulmonary sarcoidosis in an HIV-infected patient: an immunohistochemical study

    Directory of Open Access Journals (Sweden)

    Érique José Peixoto de Miranda

    2011-12-01

    Full Text Available Sarcoidosis has been rarely described in literature as a cause of interstitial pulmonary disease associated with AIDS. This study reports a case of immune reconstitution inflammatory syndrome associated with pulmonary sarcoidosis in a patient with a history of previous pulmonary tuberculosis concomitant with HIV infection. Results of the immunohistochemical study of samples from the resected right lower lobe are described. Pathological findings suggest a role of Th1, Th2 and Th17 response in IRIS associated sarcoidosis.

  18. Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia

    Directory of Open Access Journals (Sweden)

    Muhammad SAEED*

    2014-12-01

    Full Text Available How to Cite This Article: Saeed M, Haq A, Qadir Kh.Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia. Iran J Child Neurol. 2014 Autumn;8(4: 76-79.AbstractObjectiveBart’s syndrome is defined as congenital localized absence of skin, and associated with epidermolysis bullosa. A newborn with Bart’s syndrome is reported because it is a very rare condition, especially when associated with corpus callosum agenesis and concomitant choanal atresia. Clinically it is characterized by raw beefy areas of denuded skin mainly on hands and feet.We report a rare case of a term female newborn born to non-consanguineous parents who presented with congenital absence of skin in, face, trunk and extremities. To the best of our knowledge, this is the first report presenting a case of Bart’s syndrome associated with corpus callosum agenesis.ReferencesBart BJ, Garlin RJ, Anderson VE, Lynch FW. Congenital localized absence of skin and associated abnormalities resembling epidermolysis bullosa. A new syndrome. Arch Dermatol 1966; 93: 296-304.Bart BJ. Epidermolysis bullosa and congenital localized absence of skin. Arch Dermatol 1970; 101: 78-81.Skoven I, Drzewiecki KT. Congenital localized skin defect and epidermolysis bullosa hereditaria letalis. Acta Derm Venereol 1979; 59: 533-537.Wojnarowska FT, Eady RA, Wells RS. Dystrophic epidermolysis bullosa presenting with congenital localized absence of skin: report of four cases. Br J Dermatol 1983; 108: 477-483.Kanzler MH, Smoller B, Woodley DT. Congenital localized absence of the skin as a manifestation of epidermolysis bullosa. Arch Dermatol 1992; 128:1087-90.Maman E, Maor E, Kachko L, Carmi R. Epidermolysis bullosa, pyloric atresia, aplasia cutis congenita: histopathological delineation of an autosomal recessive disease. Am J Med Genet 1998; 78: 127-133.McCarthy MA, Clarke T, Powell FC. Epidermolysis bullosa and aplasia cutis. Int J Derm 1991; 30: 481-484.Puvabanditsin S, Garrow E, Daeun K

  19. 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. 

  20. Hypothyroidism and Sturge-Weber Syndrome associated with Bilateral Port-wine Nevus

    Science.gov (United States)

    Saroj, Gyanendra; Gangwar, Anshul

    2016-01-01

    ABSTRACT Sturge-Weber syndrome (SWS) is a rare, nonhereditary developmental condition that is characterized by a hamartomatous vascular proliferation of the brain, resulting in multiple angiomas that occur on the same side due to arteriovenous malformations. It is believed to be caused by persistence of a vascular plexus around the cephalic portion of the neural tube and is present at birth in about 1 in every 50,000 babies. It is one of the phakomatoses which is often associated with port-wine stains (PWSs) of the face, glaucoma, seizures, mental retardation and ipsilateral leptomeningeal angioma. Many people with SWS probably never know they have it. Hypothyroidism is a condition that arises from inadequate release of thyroid-stimulating hormone to stimulate an otherwise normal thyroid gland. This condition is often associated with a deficient secretion of other pituitary hormone, and growth hormone deficiency occurs with an increased prevalence in SWS, presumably secondary to involvement of the hypothalamic-pituitary axis. Diagnosis is made by the presence of a facial PWS and evidence of leptomeningeal angioma either by skull X-ray or computed tomography scan that shows intracranial calcifications. Presently, there is no specific treatment for SWS, and the management of the clinical manifestations and complications is still far from adequate. Here, we report the case of hypothyroidism associated with SWS with oral and facial manifestations in an 11-year-old boy. How to cite this article: Saroj G, Gangwar A, Dhillon JK. Hypothyroidism and Sturge-Weber Syndrome associated with Bilateral Port-wine Nevus. Int J Clin Pediatr Dent 2016;9(1): 82-85. PMID:27274162

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

    International Nuclear Information System (INIS)

    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

  2. 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.

  3. Arf-like Protein 3 (ARL3) Regulates Protein Trafficking and Ciliogenesis in Mouse Photoreceptors.

    Science.gov (United States)

    Hanke-Gogokhia, Christin; Wu, Zhijian; Gerstner, Cecilia D; Frederick, Jeanne M; Zhang, Houbin; Baehr, Wolfgang

    2016-03-25

    Arf-like protein 3 (ARL3) is a ubiquitous small GTPase expressed in ciliated cells of plants and animals. Germline deletion ofArl3in mice causes multiorgan ciliopathy reminiscent of Bardet-Biedl or Joubert syndromes. As photoreceptors are elegantly compartmentalized and have cilia, we probed the function of ARL3 (ADP-ribosylation factor (Arf)-like 3 protein) by generating rod photoreceptor-specific (prefix(rod)) and retina-specific (prefix(ret))Arl3deletions. In predegenerate(rod)Arl3(-/-)mice, lipidated phototransduction proteins showed trafficking deficiencies, consistent with the role of ARL3 as a cargo displacement factor for lipid-binding proteins. By contrast,(ret)Arl3(-/-)rods and cones expressing Cre recombinase during embryonic development formed neither connecting cilia nor outer segments and degenerated rapidly. Absence of cilia infers participation of ARL3 in ciliogenesis and axoneme formation. Ciliogenesis was rescued, and degeneration was reversed in part by subretinal injection of adeno-associated virus particles expressing ARL3-EGFP. The conditional knock-out phenotypes permitted identification of two ARL3 functions, both in the GTP-bound form as follows: one as a regulator of intraflagellar transport participating in photoreceptor ciliogenesis and the other as a cargo displacement factor transporting lipidated protein to the outer segment. Surprisingly, a farnesylated inositol polyphosphate phosphatase only trafficked from the endoplasmic reticulum to the Golgi, thereby excluding it from a role in photoreceptor cilia physiology. PMID:26814127

  4. PDGFRaa Signaling Is Regulated through the Primary Cilium in Fibroblasts

    DEFF Research Database (Denmark)

    Schneider, Linda; Clement, Christian Alexandro; Teilmann, S.C.;

    2005-01-01

    or mislocation of ciliary signal components affects human pathologies, such as polycystic kidney disease [ 7 ] and disorders associated with Bardet-Biedl syndrome [ 8 ]. Primary cilia are essential for hedgehog ligand-induced signaling cascade regulating growth and patterning [ [9] and [10] ]. Here...

  5. Genetics Home Reference: retinitis pigmentosa

    Science.gov (United States)

    ... by the combination of vision loss and hearing loss beginning early in life. Retinitis pigmentosa is also a feature of several other genetic syndromes, including Bardet-Biedl syndrome ; Refsum disease ; and neuropathy, ... for retinitis pigmentosa lead to a gradual loss of rods and cones in the retina. The ...

  6. 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 charact...

  7. Clinical and Molecular Investigations Into Ciliopathies

    Science.gov (United States)

    2016-08-31

    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

  8. A Case of Ectrodactyly, Ectodermal Dysplasia, Cleft Lip and Palate Syndrome Associated with Hydrocephaly

    Directory of Open Access Journals (Sweden)

    Buket Uysal Aladag

    2013-06-01

    Full Text Available Ectrodactyly, ectodermal dysplasia, cleft lip, and palate syndrome (EEC is a genetic developmental disorder characterized by ectrodactyly, ectodermal dysplasia and orofacial clefts (cleft lip/ palate. A few cases have been reported in literature. The cardinal components of the syndrome are ectrodactyly and syndactyly of the hands and feet, cleft lip with or without cleft palate, and abnormalities ectodermal structures including skin (i.e. hypopigmented and dry skin, hyperkeratosis, skin atrophy, hair (sparse hair and eye brows, teeth (small, absent or dysplastic teeth, nails (nail dystrophy and exocrine glands (reduction/ absence of sweat, sebaceous and salivary glands. A multidisciplinary approach for treatment is needed which is co-ordinated by orthopedic, plastic, dental surgeons, ophthalmologist, dermatologists and speech therapists, psychologists. We presented EEC syndrome case with hydrocephaly by the literature. [Cukurova Med J 2013; 38(3.000: 531-535

  9. Membranous glomerulonephropathy and nephrotic syndrome associated with iatrogenic metallic mercury poisoning in a cat.

    Science.gov (United States)

    Shull, R M; Stowe, C M; Osborne, C A; O'Leary, T P; Vernier, R L; Hammer, R F

    1981-02-01

    The nephrotic syndrome, characterized by nonselective proteinuria, hypoproteinemia, hypoalbuminemia, and ascites, was observed in a 10-month-old male cat. Profound glomerular changes and renal tubular changes appear to have been induced by iatrogenic chronic exposure to metallic mercury originally contained in a rectal thermometer. Large concentrations of mercury were present in the kidneys, liver, spleen, and urine. Evaluation of glomeruli by immunofluorescent microscopy revealed interrupted granular deposition of immuno-globulin G and the third component of complement in glomerular capillary walls and the mesangium. Electron microscopic evaluation of glomeruli revealed diffuse alterations in glomerular basement membranes and visceral epithelial cells. Small electron dense deposits were observed in capillary walls, but they were not characteristic of immune complexes. The mechanism(s) responsible for the mercury induced glomerulonephropathy in this patient could not be determined on the basis of available data. PMID:7257162

  10. The Fowler syndrome-associated protein FLVCR2 is an importer of heme.

    Science.gov (United States)

    Duffy, Simon P; Shing, Jennifer; Saraon, Punit; Berger, Lloyd C; Eiden, Maribeth V; Wilde, Andrew; Tailor, Chetankumar S

    2010-11-01

    Mutations in FLVCR2, a cell surface protein related by homology and membrane topology to the heme exporter/retroviral receptor FLVCR1, have recently been associated with Fowler syndrome, a vascular disorder of the brain. We previously identified FLVCR2 to function as a receptor for FY981 feline leukemia virus (FeLV). However, the cellular function of FLVCR2 remains unresolved. Here, we report the cellular function of FLVCR2 as an importer of heme, based on the following observations. First, FLVCR2 binds to hemin-conjugated agarose, and binding is competed by free hemin. Second, mammalian cells and Xenopus laevis oocytes expressing FLVCR2 display enhanced heme uptake. Third, heme import is reduced after the expression of FLVCR2-specific small interfering RNA (siRNA) or after the binding of the FY981 FeLV envelope protein to the FLVCR2 receptor. Finally, cells overexpressing FLVCR2 are more sensitive to heme toxicity, a finding most likely attributable to enhanced heme uptake. Tissue expression analysis indicates that FLVCR2 is expressed in a broad range of human tissues, including liver, placenta, brain, and kidney. The identification of a cellular function for FLVCR2 will have important implications in elucidating the pathogenic mechanisms of Fowler syndrome and of phenotypically associated disorders. PMID:20823265

  11. The Fowler Syndrome-Associated Protein FLVCR2 Is an Importer of Heme ▿

    Science.gov (United States)

    Duffy, Simon P.; Shing, Jennifer; Saraon, Punit; Berger, Lloyd C.; Eiden, Maribeth V.; Wilde, Andrew; Tailor, Chetankumar S.

    2010-01-01

    Mutations in FLVCR2, a cell surface protein related by homology and membrane topology to the heme exporter/retroviral receptor FLVCR1, have recently been associated with Fowler syndrome, a vascular disorder of the brain. We previously identified FLVCR2 to function as a receptor for FY981 feline leukemia virus (FeLV). However, the cellular function of FLVCR2 remains unresolved. Here, we report the cellular function of FLVCR2 as an importer of heme, based on the following observations. First, FLVCR2 binds to hemin-conjugated agarose, and binding is competed by free hemin. Second, mammalian cells and Xenopus laevis oocytes expressing FLVCR2 display enhanced heme uptake. Third, heme import is reduced after the expression of FLVCR2-specific small interfering RNA (siRNA) or after the binding of the FY981 FeLV envelope protein to the FLVCR2 receptor. Finally, cells overexpressing FLVCR2 are more sensitive to heme toxicity, a finding most likely attributable to enhanced heme uptake. Tissue expression analysis indicates that FLVCR2 is expressed in a broad range of human tissues, including liver, placenta, brain, and kidney. The identification of a cellular function for FLVCR2 will have important implications in elucidating the pathogenic mechanisms of Fowler syndrome and of phenotypically associated disorders. PMID:20823265

  12. Case Report: Myelodysplastic syndrome- associated myeloid sarcoma: an unusual clinical presentation of a rare disease [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Emoke Horvath

    2016-02-01

    Full Text Available Myeloid sarcoma results from the extramedullary homing and proliferation of immature myeloid precursors. We present the timeline, events and diagnostic pitfalls related to a 66 year-old male patient’s case, admitted to the Hematology Clinic for pancytopenia, fever, weight loss and fatigue. The severe cytopenia and the few blasts observed in his blood smear indicated a bone marrow biopsy. The bone marrow showed hypercellularity and multilineage dysplasia with the presence of 15% myeloblasts. After the biopsy, he promptly developed paraplegia and nuclear magnetic resonance revealed an epidural tumour which was then resected.In the epidural tumour mass blast-like, round cells were observed with a complex immunophenotype, characterized by myeloperoxidase, CD117, CD15, CD99, leucocyte common antigen positivity and a high Ki-67 proliferation index. Considering the main differential diagnostic issues, the final diagnosis was stated as myelodysplastic syndrome-associated myeloid sarcoma. The prognosis was unfavourable, the bone marrow was quickly invaded by proliferating blast cells, and despite chemotherapy attempts, the patient died.

  13. Hemophagocytic Syndrome Associated with Immune Reconstitution Inflammatory Syndrome in a Patient with AIDS Related Burkitt’s Leukemia/Lymphoma

    Directory of Open Access Journals (Sweden)

    Metin Kanıtez

    2014-01-01

    Full Text Available Highly active antiretroviral therapy (HAART has markedly decreased human immunodeficiency virus- (HIV- related mortality and the incidence of opportunistic infections. The dramatic reduction in HIV-1 RNA and increase in CD4 lymphocyte count mean a recovery in immune function. This restoration in immune function may be associated with paradoxical deterioration in subclinical opportunistic infections in some patients, a condition called immune reconstitution inflammatory syndrome (IRIS. IRIS, a “paradoxical” inflammatory response to either previously treated or subclinical infections or noninfectious diseases, can manifest during the restoration phase of immunity hemophagocytic syndrome (HS which is a very rare complication in patients with acquired immune deficiency syndrome (AIDS. We describe a case of hemophagocytic syndrome associated with IRIS in a patient with AIDS related Burkitt’s leukemia/lymphoma (BL. IRIS was probably the cause of hemophagocytosis for our patient. Zoster infection may facilitate to IRIS. With the increasing number of people with HIV infection and the accompanying use of HAART, much more clinical manifestations of IRIS will be experienced especially in patients given high dose chemotherapy, just like in our case.

  14. Miller-Dieker syndrome associated with duplication of 17p13.3 confirmed by fluorescence in situ hybridization (FISH)

    Energy Technology Data Exchange (ETDEWEB)

    Li, S.; Tuck-Muller, C.M.; Martinez, J.E. [Univ. of South Alabama, Mobile, AL (United States)] [and others

    1994-09-01

    Miller-Dieker syndrome is characterized by profound mental retardation, craniofacial abnormalities, and lissencephaly (smooth brain). Microscopic or submicroscopic deletions of the 17p13.3 region have been reported in Miller-Dieker patients. We report a patient with this syndrome in whom a duplication of the 17p13.3 region was detected by FISH. The 9-year-old female proband was referred because of features of Miller-Dieker syndrome: microcephaly, profound psychomotor retardation, seizures, characteristic facies, and lissencephaly shown by MRI studies. High-resolution G-banding failed to demonstrate an abnormality in chromosome 17. However, FISH analysis with the DNA probe (Oncor No. 5101) specific for Miller-Dieker region of chromosome 17p13.3 demonstrated duplication of this segment instead of the classic deletion. We know of no other report of Miller-Dieker syndrome associated with duplication of 17p13.3. The family study revealed normal chromosomes in both parents by cytogenetic and FISH analysis. Our investigation suggests that duplications, as well as deletions, of the 17p13.3 region are associated with the Miller-Dieker syndrome. The presence of deletions or duplications of the same chromosomal region in patients with features of Miller-Dieker syndrome suggests that its pathogenesis may be due to gene dosage effects.

  15. Linguistic rehabilitation nursing of pregnancy induced hypertension syndrome associated with cerebral hemorrhage%妊娠高血压综合征并发脑出血的语言康复护理

    Institute of Scientific and Technical Information of China (English)

    刘焕美; 石文利

    2003-01-01

    @@ BACKGROUND:Pregnancy induced hypertension syndrome associated with cerebral hemorrhage is the common cause of obstetric death and even emergency treatment succeeds,living quality is affected because of lalopathy.

  16. Síndrome serotoninérgica associada ao uso de paroxetina: relato de caso Serotonin syndrome associated to the use of paroxetine: case report

    OpenAIRE

    LUÍS OTÁVIO CAVALLAZZI; ANDERSON K. GREZESIUK

    1999-01-01

    Relatamos um caso de síndrome serotoninérgica pelo uso de inibidor da recaptação da serotonina, a paroxetina. Tal síndrome por esta droga, sem combinações, ainda não tinha sido descrita na literatura.We report on a case of serotonin syndrome associated to the use of the paroxetine, a serotonin reuptake inhibitor drug. Serotonin syndrome related to this drug not combined with other drugs had not yet been described in literature.

  17. Síndrome de Guillain-Barré associada a pancreatite aguda: relato de caso Guillain-Barré syndrome associated with acute pancreatitis: case report

    OpenAIRE

    ANDERSON KUNTZ GRZESIUK; LUIZ AUGUSTO DOS SANTOS

    1999-01-01

    Relatamos um caso clínico de síndrome de Guillain-Barré associada a pancreatite aguda, e realizamos revisão na literatura sobre complicações clínicas relacionadas à síndrome de Guillain- Barré. Sugerimos estarem os eventos clínicos relacionados imunologicamente.We report a clinical case of Guillain-Barré syndrome associated with acute pancreatitis, and perform a review in literature about clinical complications related to Guillain-Barré syndrome. We suggest the possibility of being the events...

  18. Clinicopathologic features of Turcot syndrome associated with intestinal Burkitt lymphoma%Turcot综合征合并肠道Burkitt淋巴瘤的临床病理观察

    Institute of Scientific and Technical Information of China (English)

    张淑红; 朱红; 周小鸽; 郑媛媛; 张彦宁; 陈光勇

    2012-01-01

    Purpose To investigate the clinicopathologic characteristics of Turcot syndrome associated with intestinal Burkitt lymphoma. Methods One case of Turcot syndrome associated with intestinal Burkitt lymphoma was examined by microscopy and immunohistochemical staining, together with review of the literatures. Results The patient underwent three operations; the first pathological diagnosis was multiple tubulovillous adenomas of colon, the second was anaplastic astrocytoma of brain, and the third was multiple tubulovillous adenomas of colon associated with colonic Burkitt lymphoma. Conclusion Based on clinicopathologic characteristics, diagnosis of Turcot syndrome is relatively easy, but its association with lymphoma is very rare. Relationships between Turcol syndrome and lymphoma need further studies.%目的 探讨Turcot综合征合并肠道Burkitt淋巴瘤的临床病理特征.方法 对1例Turcot综合征合并肠道Burkitt淋巴瘤进行光镜观察及免疫组化标记,并结合文献进行分析.结果 患者先后行3次手术.第1次手术病理诊断为结肠多发性绒毛管状腺瘤;第2次手术病理诊断为脑间变性星形细胞瘤;第3次手术病理诊断为结肠多发性绒毛管状腺瘤及结肠Burkitt淋巴瘤.结论 依靠临床病理特点,Turcot综合征的诊断相对容易,但其合并淋巴瘤十分罕见.二者之间是否存在一定的关系尚需进一步研究.

  19. Cilium transition zone proteome reveals compartmentalization and differential dynamics of ciliopathy complexes.

    Science.gov (United States)

    Dean, Samuel; Moreira-Leite, Flavia; Varga, Vladimir; Gull, Keith

    2016-08-30

    The transition zone (TZ) of eukaryotic cilia and flagella is a structural intermediate between the basal body and the axoneme that regulates ciliary traffic. Mutations in genes encoding TZ proteins (TZPs) cause human inherited diseases (ciliopathies). Here, we use the trypanosome to identify TZ components and localize them to TZ subdomains, showing that the Bardet-Biedl syndrome complex (BBSome) is more distal in the TZ than the Meckel syndrome (MKS) complex. Several of the TZPs identified here have human orthologs. Functional analysis shows essential roles for TZPs in motility, in building the axoneme central pair apparatus and in flagellum biogenesis. Analysis using RNAi and HaloTag fusion protein approaches reveals that most TZPs (including the MKS ciliopathy complex) show long-term stable association with the TZ, whereas the BBSome is dynamic. We propose that some Bardet-Biedl syndrome and MKS pleiotropy may be caused by mutations that impact TZP complex dynamics. PMID:27519801

  20. OBEZİTENİN NADİR BİR NEDENİ: BARDET BİEDLE SENDROMU

    OpenAIRE

    Üstün, İhsan; Arı, Mustafa; Gökçe, Cumali; Arıca, Vefik; Mesut COŞKUN; Çelik, Murat; Cezgin, Kamil; Akçay, Burak

    2010-01-01

    Bardet-Biedl Sendromu (BBS), pleitropik bir bozukluk olup değişken genetik geçişi mevcuttur. Bugüne kadar 14 adet BBS gen mutasyonu bildirilmiştir. Siller birçok hücrenin yüzeyinde bulunan mikrotübül çıkıntılardır ve Bardet Biedle Sendromunda bu sil yapılarında bozukluk tespit edilmiştir. Bu sendromda retinitis pigmentoza, gövdesel obezite, polidaktili, hipogonadizm, renal fonksiyon bozukluğu, gelişme geriliği ve konuşmada gecikme, öğrenme bozuklukları ve diyabetes mellitus yer alır. Obezites...

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

    OpenAIRE

    Ram Kumar Marwaha; Inusha Panigrahi; Sheetal Sharda

    2011-01-01

    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 increasin...

  2. Screening for Hormonal, Monogenic, and Syndromic Disorders in Obese Infants and Children

    OpenAIRE

    Mason, Kelly; Page, Laura; Balikcioglu, Pinar Gumus

    2014-01-01

    The prevalence of pediatric obesity in the United States is nearly 17%. Most cases are “exogenous”, resulting from excess energy intake relative to energy expenditure over a prolonged period of time. However, some cases of obesity are “endogenous”, associated with hormonal, genetic, or syndromic disorders such as hypothyroidism, Cushing’s syndrome, growth hormone deficiency, defective leptin signaling, mutations in the melanocortin 4 receptor, and Prader-Willi and Bardet-Biedl syndromes. This...

  3. Inherited Retinal Degenerative Disease Registry

    Science.gov (United States)

    2016-03-21

    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

  4. A Novel Familial BBS12 Mutation Associated with a Mild Phenotype: Implications for Clinical and Molecular Diagnostic Strategies

    OpenAIRE

    Pawlik, B.; Mir, A.; Iqbal, H.; Li, Y; Nürnberg, G; Becker, C.; Qamar, R.; Nürnberg, P; Wollnik, B

    2010-01-01

    Bardet-Biedl syndrome (BBS) is an autosomal recessively inherited ciliopathy mainly characterized by rod-cone dystrophy, postaxial polydactyly, obesity, renal tract anomalies, and hypogonadism. To date, 14 BBS genes, BBS1 to BBS14, have been identified, accounting for over 75% of mutations in BBS families. In this study, we present a consanguineous family from Pakistan with postaxial polydactyly and late-onset retinal dysfunction. Adult affected individuals did not show any renal or genital a...

  5. Sensory Ciliogenesis in Caenorhabditis elegans: Assignment of IFT Components into Distinct Modules Based on Transport and Phenotypic Profiles

    OpenAIRE

    Ou, Guangshuo; Koga, Makato; Oliver E Blacque; Murayama, Takashi; Ohshima, Yasumi; Schafer, Jenny C.; LI, Chunmei; Yoder, Bradley K.; Leroux, Michel R.; Scholey, Jonathan M.

    2007-01-01

    Sensory cilium biogenesis within Caenorhabditis elegans neurons depends on the kinesin-2–dependent intraflagellar transport (IFT) of ciliary precursors associated with IFT particles to the axoneme tip. Here we analyzed the molecular organization of the IFT machinery by comparing the in vivo transport and phenotypic profiles of multiple proteins involved in IFT and ciliogenesis. Based on their motility in wild-type and bbs (Bardet-Biedl syndrome) mutants, IFT proteins were classified into grou...

  6. Pyoderma Gangrenosum: A Paraneoplastic Syndrome Associated with Occult Colorectal Malignancy? Report of A Case and Review of Literature

    Directory of Open Access Journals (Sweden)

    Khalid Ahmed

    2015-12-01

    Case Report: A 74-year-old patient presented to the emergency department with central abdominal pain and vomiting for 24 hours. Recently, the patient was worked up for iron deficiency anemia and was due to have colonoscopy. She was also discovered to have multiple skin lesions on her left hand, which were ulcerated, erythematous, indurated, and thick with scaly plaque. Initially the differential diagnosis included: neutrophilic dermatosis, atypical mycoblastosis or mycosis. Skin biopsy revealed PG. The skin lesion did not respond to high-dose systemic steroids. Two months later during presentation to the emergency department with abdominal pain, abdominal and pelvic CT showed proximal sigmoid colon mass with nodular extension probably involving adjacent small bowel loops but no liver metastasis. Colonoscopy showed stenosing sigmoid mass, which was confirmed by biopsy to be adenocarcinoma. Laparotomy with resection of the sigmoid, upper part of the rectum, and a short segment of the involved small bowel was performed. Histology confirmed adenocarcinoma of the sigmoid (T4N0Mx. The skin lesions healed spontaneously following surgery. Conclusion: Early treatment of CRC can enhance the outcome of patients with PG, which may point toward a related pathological process. Furthermore, PG may be an important predictor of CRC. We advise on detailed gastrointestinal tract GIT investigation for unresponsive PG. [Arch Clin Exp Surg 2015; 4(4.000: 226-230

  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. Increased intestinal absorption in the era of teduglutide and its impact on management strategies in patients with short bowel syndrome-associated intestinal failure.

    Science.gov (United States)

    Seidner, Douglas L; Schwartz, Lauren K; Winkler, Marion F; Jeejeebhoy, Khursheed; Boullata, Joseph I; Tappenden, Kelly A

    2013-03-01

    Short bowel syndrome-associated intestinal failure (SBS-IF) as a consequence of extensive surgical resection of the gastrointestinal (GI) tract results in a chronic reduction in intestinal absorption. The ensuing malabsorption of a conventional diet with associated diarrhea and weight loss results in a dependency on parenteral nutrition and/or intravenous fluids (PN/IV). A natural compensatory process of intestinal adaptation occurs in the years after bowel resection as the body responds to a lack of sufficient functional nutrient-processing intestinal surface area. The adaptive process improves bowel function but is a highly variable process, yielding different levels of symptom control and PN/IV independence among patients. Intestinal rehabilitation is the strategy of maximizing the absorptive capacity of the remnant GI tract. The approaches for achieving this goal have been limited to dietary intervention, antidiarrheal and antisecretory medications, and surgical bowel reconstruction. A targeted pharmacotherapy has now been developed that improves intestinal absorption. Teduglutide is a human recombinant analogue of glucagon-like peptide 2 that promotes the expansion of the intestinal surface area and increases the intestinal absorptive capacity. Enhanced absorption has been shown in clinical trials by a reduction in PN/IV requirements in patients with SBS-IF. This article details the clinical considerations and best-practice recommendations for intestinal rehabilitation, including optimization of fluids, electrolytes, and nutrients; the integration of teduglutide therapy; and approaches to PN/IV weaning. PMID:23343999

  9. [General Anesthesia Using Remifentanil for Cesarean Section in a Parturient with Marfan Syndrome Associated with Heart Failure due to Severe Mitral Regurgitation].

    Science.gov (United States)

    Fujita, Masahide; Satsumae, Tsuyoshi; Tanaka, Makoto

    2016-05-01

    A 24-year-old woman with Marfan syndrome was scheduled for cesarean section in order to avoid progression of heart failure due to severe mitral regurgitation and aortic dissection during labor. Cesarean section was performed under general anesthesia using remifentanil. Anesthesia was induced and maintained with remifentanil (0.1-0.3 μg x kg(-1) x min(-1)) and continuous administration of propofol (target-controlled infusion, 2-3 ng x ml(-1)). The trachea was intubated without a significant hemodynamic change. The patient's systolic blood pressure was maintained between 90 and 120 mmHg during surgery. Intraoperatively, we conducted a transesophageal echocardiography examination, and no remarkable change was seen in the severity of mitral regurgitation and the size of an ascending aorta. An infant was delivered 6 minutes after anesthesia induction. The Apgar scores were 4 at 1 min, 5 at 5 min and 8 at 10 min. Postoperative course was uneventful. We conclude that remifentanil can be used successfully to manage cesarean section of a parturient with Marfan syndrome associated with heart failure due to severe mitral regurgitation under general anesthesia. PMID:27319100

  10. Sweet syndrome associated with interferon

    OpenAIRE

    Rodriguez-Lojo, Romina; Castineiras, Iria; Juarez, Yolanda; Lueiro, Mercedes; Armesto, Ana; Fernandez-Diaz, M. Luisa

    2015-01-01

    Although still very rare, drug-related cases of Sweet’s syndrome have been reported. The more frequent associated medications with drug induced Sweet´s syndrome was: tetracyclines, trimethoprim-sulphamethaxazol, azatioprine, all trans retinoic acid, nitrofurantoin, granulocyte colony-stimulating factor, hydralazine, tripharil, lithium, oral contraceptives, furosemide, celecoxib and azathioprine. We only found one case of drug-induced Sweet´s syndrome secondary to pegylated interferon...

  11. Prader-Willi Syndrome Association

    Science.gov (United States)

    ... Events Find a Chapter Contact Us International Membership Shopping Bag Shop Members Only Events Find a Chapter ... With our many On The Move Walks and online fundraisers such as eWalk, we have something available ...

  12. [Nephrotic syndrome associated with ampulloma].

    Science.gov (United States)

    López-Navidad, A; Colomina, J; Domingo, P; Franco, M; Algaba, F

    1989-06-01

    A patient is reported who presented simultaneously classic nephrotic syndrome and adenocarcinoma of the Vater ampulla. Morphological study of the renal biopsy revealed changes characteristic of membranous glomerulonephritis and subepithelial deposits. These deposits stained specifically for IgG and C'3 with a granular pattern, but deposits of CEA-antiCEA immune complexes were not found in glomerular capillaries. The association of nephrotic syndrome with lymphoproliferative diseases and a large variety of solid tumors, like carcinoma of the breast, bronchogenic, colon and stomach has been communicated, but the present case constitutes the first known association with adenocarcinoma of the Vater ampulla. PMID:2772382

  13. 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.

  14. Lynch syndrome-associated neoplasms

    DEFF Research Database (Denmark)

    Shia, Jinru; Holck, Susanne; Depetris, Giovanni;

    2013-01-01

    It was a century ago that Warthin, a pathologist, first described the clinical condition now known as Lynch syndrome. One hundred years later, our understanding of this syndrome has advanced significantly. Much of the progress took place over the last 25 years and was marked by a series of intera...

  15. Síndrome de Brown bilateral associada com hipermobilidade articular benigna: relato de caso Bilateral Brown's syndrome associated with benign joint hypermobility: a case report

    Directory of Open Access Journals (Sweden)

    David Kirsch

    2007-03-01

    this case the authors describe a case of Brown's syndrome associated with Benign joint hipermobility and call attention to a little described association in the literature. J.C.S, male, 6 years old, mulatto, student, was referred to the University of Santo Amaro with complaint of exotropia for 2 years that it increased in supraversion. The patient with the diagnosis of bilateral Brown's syndrome, was diagnosed as having benign joint hipermobility by the reumatologist. The patient with benign joint hipermobility can develop symptoms such as arthralgia caused by a joint inflammation. We believe in the possibility that Brown syndrome has occurred, caused by an inflammatory process in the trochlea that started because of the benign joint hypermobility.

  16. Cyclooxygenase-2 Expression in Non-Small Cell Lung Cancer Correlates With Hypertrophic Osteoarthropathy.

    Science.gov (United States)

    Rotas, Ioannis; Cito, Giovanni; Letovanec, Igor; Christodoulou, Michel; Perentes, Jean Y

    2016-02-01

    Hypertrophic osteoarthrpathy (HO) is a rare paraneoplasic syndrome associated with non-small cell lung cancer (NSCLC). The pathophysiology of HO is unknown but was recently related to enhanced levels of urine prostaglandin E2 (PGE2). Here, we report the case of a patient that presented HO in association with a resectable left upper lobe NSCLC. Following surgery and adjuvant chemotherapy, HO resolved and did not recur with development of a brain metastasis 1 year later. Interestingly, tumor cyclooxygenase-2, an enzyme responsible the synthesis of PGE2, was expressed in the primary tumor but not in the resected metastasis. PMID:26777972

  17. Genetics of Obesity.

    Science.gov (United States)

    Srivastava, Apurva; Srivastava, Neena; Mittal, Balraj

    2016-10-01

    Numerous classical genetic studies have proved that genes are contributory factors for obesity. Genes are directly responsible for obesity associated disorders such as Bardet-Biedl and Prader-Willi syndromes. However, both genes as well as environment are associated with obesity in the general population. Genetic epidemiological approaches, particularly genome-wide association studies, have unraveled many genes which play important roles in human obesity. Elucidation of their biological functions can be very useful for understanding pathobiology of obesity. In the near future, further exploration of obesity genetics may help to develop useful diagnostic and predictive tests for obesity treatment. PMID:27605733

  18. The Shwachman-Bodian-Diamond syndrome associated protein interacts with HsNip7 and its down-regulation affects gene expression at the transcriptional and translational levels

    International Nuclear Information System (INIS)

    The Shwachman-Bodian-Diamond syndrome (SDS) is an autosomal disorder with pleiotropic phenotypes including pancreatic, skeletal and bone marrow deficiencies and predisposition to hematological dysfunctions. SDS has been associated to mutations in the SBDS gene, encoding a highly conserved protein that was shown to function in ribosome biogenesis in yeast. In this work, we show that SBDS is found in complexes containing the human Nip7 ortholog. Analysis of pre-rRNA processing in a stable SBDS knock-down HEK293-derivative cell line revealed accumulation of a small RNA which is a further indication of SBDS involvement in rRNA biosynthesis. Global transcription and polysome-bound mRNA profiling revealed that SBDS knock-down affects expression of critical genes involved in brain development and function, bone morphogenesis, blood cell proliferation and differentiation, and cell adhesion. Expression of a group of growth and signal transduction factors and of DNA damage response genes is also affected. In SBDS knock-down cells, 34 mRNAs showed decreased and 55 mRNAs showed increased association to polysomes, among which is a group encoding proteins involved in alternative splicing and RNA modification. These results indicate that SBDS is required for accurate expression of genes important for proper brain, skeletal, and blood cell development

  19. Intra- and interfamily phenotypic diversity in pain syndromes associated with a gain-of-function variant of NaV1.7

    Directory of Open Access Journals (Sweden)

    Estacion Mark

    2011-12-01

    Full Text Available Abstract Background Sodium channel NaV1.7 is preferentially expressed within dorsal root ganglia (DRG, trigeminal ganglia and sympathetic ganglion neurons and their fine-diamter axons, where it acts as a threshold channel, amplifying stimuli such as generator potentials in nociceptors. Gain-of-function mutations and variants (single amino acid substitutions of NaV1.7 have been linked to three pain syndromes: Inherited Erythromelalgia (IEM, Paroxysmal Extreme Pain Disorder (PEPD, and Small Fiber Neuropathy (SFN. IEM is characterized clinically by burning pain and redness that is usually focused on the distal extremities, precipitated by mild warmth and relieved by cooling, and is caused by mutations that hyperpolarize activation, slow deactivation, and enhance the channel ramp response. PEPD is characterized by perirectal, periocular or perimandibular pain, often triggered by defecation or lower body stimulation, and is caused by mutations that severely impair fast-inactivation. SFN presents a clinical picture dominated by neuropathic pain and autonomic symptoms; gain-of-function variants have been reported to be present in approximately 30% of patients with biopsy-confirmed idiopathic SFN, and functional testing has shown altered fast-inactivation, slow-inactivation or resurgent current. In this paper we describe three patients who house the NaV1.7/I228M variant. Methods We have used clinical assessment of patients, quantitative sensory testing and skin biopsy to study these patients, including two siblings in one family, in whom genomic screening demonstrated the I228M NaV1.7 variant. Electrophysiology (voltage-clamp and current-clamp was used to test functional effects of the variant channel. Results We report three different clinical presentations of the I228M NaV1.7 variant: presentation with severe facial pain, presentation with distal (feet, hands pain, and presentation with scalp discomfort in three patients housing this NaV1.7 variant

  20. Small pleasures

    OpenAIRE

    Wood, Philippa

    2010-01-01

    This book simply examines the differences between what men and women consider their 'small pleasures' in life. The book combines contemporary text matter juxtaposed with traditional illustrations. A double-sided concertina book that uses the theme of minutiae as both topic and form.

  1. 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.

  2. Waardenburg's syndrome associated with total aganglionosis.

    OpenAIRE

    Farndon, P A; Bianchi, A.

    1983-01-01

    A Pakistani child of consanguineous parents had signs of Waardenburg's syndrome and total intestinal aganglionosis. This association seems to be a distinct clinical entity with an autosomal recessive mode of inheritance.

  3. Churg-Strauss syndrome associated with montelukast

    OpenAIRE

    Mukhopadhyay, A.; Stanley, N

    2001-01-01

    Churg-Strauss syndrome (CSS) has recently been reported in patients with asthma receiving leukotriene receptor antagonists (LTRAs). In this paper a case of CSS after treatment with montelukast is described. As in other LTRA treated cases, prior withdrawal of maintenance oral steroid may have unmasked a previously occult CSS in the patient, but a dramatic improvement in his eosinophilia after withdrawing montelukast implied that the drug also had a direct effect in activating this condition.

...

  4. Callosal Disconnection Syndrome Associated with Relapsing Polychondritis.

    Science.gov (United States)

    Baba, Toru; Kanno, Shigenori; Shijo, Tomomi; Nishio, Yoshiyuki; Iizuka, Osamu; Kamimura, Naoto; Ishii, Tomonori; Mori, Etsuro

    2016-01-01

    Relapsing polychondritis (RP) is a rare inflammatory disorder of the cartilagenous structures, and it sometimes involves the central nervous system. Encephalitis associated with RP causes a wide variety of symptoms according to the affected sites. We herein report the first case of 72-year-old right-handed man who developed acute meningoencephalitis associated with RP involving the corpus callous. After immunosuppressive therapy, his symptoms dramatically improved, but difficulty in performing bimanual movements with occasional diagonistic dyspraxia in his right hand remained. Because callosal signs are easily missed, especially in acute settings, it would be useful to know that RP can sometimes cause callosal disconnection syndrome. PMID:27150878

  5. Mosaic Turner syndrome associated with schizophrenia

    OpenAIRE

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

    2014-01-01

    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. HO...

  6. Turner Syndrome Associated with Ulcerative Colitis

    OpenAIRE

    Takaya, Junji; Teraguchi, Masayuki; Ikemoto, Yumiko; Yoshimura, Ken; Yamato, Fumiko; Higashino, Hirohiko; Kobayashi, Yohnosuke; Kaneko, Kazunari

    2006-01-01

    We report the case of a 7-yr-old girl with Turner syndrome, ulcerative colitis (UC) and coarctation of the aorta. The diagnosis of Turner syndrome was made in early infancy (karyotype analysis 45, X). Growth hormone treatment was started at 3 yr and 2 mo of age. From the age of 4 yr and 5 mo, the patient suffered from persistent diarrhea with traces of blood and intermittent abdominal discomfort. As these symptoms gradually deteriorated, she was referred to our clinic at the age of 7 yr for f...

  7. 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

    2014-01-01

    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. PMID:24926463

  8. Lane-Hamilton syndrome: association or coincidence.

    Science.gov (United States)

    Paksu, Sule; Paksu, Muhammet Sukru; Kalayci, Ayhan Gazi; Sancak, Recep

    2012-03-01

    The combination of idiopathic pulmonary hemosiderosis (IPH) and celiac disease (CD) is rare. The clinical importance of this association is that a significant improvement can be obtained with gluten free diet not only in intestinal but also in pulmonary symptoms. A four and half-years old girl was admitted with complaints of cough, difficulty in breathing and paleness. She had intermittent episodes of abdominal pain and diarrhea. She had dyspnea and tachycardia, and oxygen saturation 88%. The patient was diagnosed with CD and concomitant IPH. With gluten-free diet and corticosteroid treatment, both intestinal and pulmonary symptoms were controlled. PMID:22484746

  9. 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....

  10. 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.

  11. 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. PMID:18383111

  12. Noonan syndrome associated with cronic autoimmune thyroiditis

    Directory of Open Access Journals (Sweden)

    Ana Valea

    2012-01-01

    Full Text Available Noonan syndrome is a genetic dominant disease that presents a wide variety of clinical characteristics. Frequently, the syndrome is associated with some autoimmune diseases, especially with chronic autoimmune thyroiditis. We report the case of a 21 years old girl diagnosed with Noonan syndrome and autoimmune thyroiditis relatively late, at the age of 20. The diagnosis was suggested by a cluster of significant clinical elements: facial dysmorphism, short stature, moderate mental retardation. The hormonal profile and karyotype contributed to the final certainty diagnosis. At this moment she is under thyroid substitution treatment.

  13. Goldenhar Syndrome Associated with Extensive Arterial Malformations

    Directory of Open Access Journals (Sweden)

    Renee Frances Modica

    2015-01-01

    Full Text Available Goldenhar Syndrome is characterized by craniofacial, ocular and vertebral defects secondary to abnormal development of the 1st and 2nd branchial arches and vertebrae. Other findings include cardiac and vascular abnormalities. Though these associations are known, the specific anomalies are not well defined. We present a 7-month-old infant with intermittent respiratory distress that did not improve with respiratory interventions. Echocardiogram suggested a double aortic arch. Cardiac CT angiogram confirmed a right arch and aberrant, stenotic left subclavian artery, dilation of the main pulmonary artery, and agenesis of the left thyroid lobe. Repeat echocardiograms were concerning for severely dilated coronary arteries. Given dilation, a rheumatologic workup ensued, only identifying few weakly positive autoantibodies. Further imaging demonstrated narrowing of the aorta below the renal arteries and extending into the common iliac arteries and proximal femoral arteries. Given a physical exam devoid of rheumatologic findings, only weakly positive autoantibodies, normal inflammatory markers, and presence of the coronary artery dilation, the peripheral artery narrowings were not thought to be vasculitic. This case illustrates the need to identify definitive anomalies related to Goldenhar Syndrome. Although this infant’s presentation is rare, recognition of specific vascular findings will help differentiate Goldenhar Syndrome from other disease processes.

  14. 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.

  15. Syndromes Associated with Sexually Transmitted Infections

    OpenAIRE

    Max Chernesky; David Patrick; Rosanna Peeling

    2005-01-01

    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 ...

  16. 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.

  17. Psychiatric syndromes associated with atypical chest pain

    OpenAIRE

    Nikolić Gordana; Tasić Ivan; Manojlović Snežana; Samardžić Ljiljana; Tošić Suzana; Ćirić Zoran

    2010-01-01

    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 expos...

  18. 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.

  19. Hemophagocytic syndrome associated with Plasmodium falciparum infection

    Directory of Open Access Journals (Sweden)

    P N Vinoth

    2011-01-01

    Full Text Available Hemophagocytic syndrome (HPS has been associated with infections, hematological malignancies and autoimmune conditions. Malaria is rarely reported to cause HPS. We report a case of an 11-month-old infant with fever, hepatosplenomegaly, pancytopenia, high serum ferritin, hypertriglyceridemia, and bone marrow hemophagocytosis, consistent with hemophagocytic syndrome. Gametocytes of plasmodium falciparum were identified on bone marrow aspiration. Rapid recovery was observed after treatment with antimalarials.

  20. The retinal ciliopathies.

    Science.gov (United States)

    Adams, N A; Awadein, Ahmed; Toma, Hassanain S

    2007-09-01

    While the functions of many of the proteins located in or associated with the photoreceptor cilia are poorly understood, disruption of the function of these proteins may result in a wide variety of phenotypes ranging from isolated retinal degeneration to more pleiotropic phenotypes. Systemic findings include neurosensory hearing loss, developmental delay, situs-inversus, infertility, disorders of limb and digit development, obesity, kidney disease, liver disease, and respiratory disease. The concept of "retinal ciliopathies" brings to attention the importance of further molecular analysis of this organelle as well as provides a potential common target for therapies for these disorders. The retinal ciliopathies include retinitis pigmentosa, macular degeneration, cone-dystrophy, cone-rod dystrophy, Leber congenital amaurosis, as well as retinal degenerations associated with Usher syndrome, primary ciliary dyskinesia, Senior-Loken syndrome, Joubert syndrome, Bardet-Biedl syndrome, Laurence-Moon syndrome, McKusick-Kaufman syndrome, and Biemond syndrome. Mutations for these disorders have been found in retinitis pigmentosa-1 (RP1), retinitis pigmentosa GTPase regulator (RPGR), retinitis pigmentosa GTPase regulator interacting protein (RPGR-IP), as well as the Usher, Bardet-Biedl, and nephronophthisis genes. Other systemic disorders associated with retinal degenerations that may also involve ciliary abnormalities include: Alstrom, Edwards-Sethi, Ellis-van Creveld, Jeune, Meckel-Gruber, Orofaciodigital Type 9, and Gurrieri syndromes. Understanding these conditions as ciliopathies may help the ophthalmologist to recognize associations between seemingly unrelated diseases and have a high degree of suspicion that a systemic finding may be present. PMID:17896309

  1. 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)

    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

  2. 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...

  3. 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 ...

  4. 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 ...

  5. BRANDING IN SMALL BUSINESS

    OpenAIRE

    Mihai Răzvan Constantin BARBU; Radu Florin OGARCĂ; Cătălin Mihail BARBU

    2010-01-01

    In this paper we analyzed the branding in small business. Using a desk research on Internet and the press we have identified the practices small businesses use to enhance their brand and the brand dynamics in small business. Our main contribution is that we tried to figure out the strategy of branding in small business. This need further to be investigated in order to understand how branding works in small business and to better capture the role of branding in small business.

  6. 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...

  7. Increased Contractile Response to Noradrenaline Induced By Factors Associated with the Metabolic Syndrome in Cultured Small Mesenteric Arteries

    DEFF Research Database (Denmark)

    Blædel, Martin; Sams, Anette; Boonen, Harrie C M; Sheykhzade, Majid

    UNLABELLED: This study investigated the effect of the metabolic syndrome associated risk factors hyperglycemia (glucose [Glc]), hyperinsulinemia (insulin [Ins]) and low-grade inflammation (tumor necrosis factor α [TNFα]) on the vasomotor responses of resistance arteries. Isolated small mesenteric...... arteries from 3-month-old Sprague-Dawley rats, were suspended for 21-23 h in tissue cultures containing either elevated Glc (30 mmol/l), Ins (100 nmol/l), TNFα (100 ng/ml) or combinations thereof. After incubation, the vascular response to noradrenaline (NA), phenylephrine, isoprenaline and NA in the...... presence of propranolol (10 µmol/l) was measured by wire myography. RESULTS: Arteries exposed only to combinations of the risk factors showed a significant 1.6-fold increase in the contractile NA sensitivity, which suggests that complex combinations of metabolic risk factors might lead to changes in...

  8. Small wind turbines

    CERN Document Server

    Wood, David

    2011-01-01

    Small Wind Turbines provides a thorough grounding in analysing, designing, building, and installing a small wind turbine. Small turbines are introduced by emphasising their differences from large ones and nearly all the analysis and design examples refer to small turbines.The accompanying software includes MATLAB(R) programs for power production and starting performance, as well as programs for detailed multi-objective optimisation of blade design. A spreadsheet is also given to help readers apply the simple load model of the IEC standard for small wind turbine safety. Small Wind Turbines repr

  9. BRANDING IN SMALL BUSINESS

    Directory of Open Access Journals (Sweden)

    Mihai Răzvan Constantin BARBU

    2010-01-01

    Full Text Available In this paper we analyzed the branding in small business. Using a desk research on Internet and the press we have identified the practices small businesses use to enhance their brand and the brand dynamics in small business. Our main contribution is that we tried to figure out the strategy of branding in small business. This need further to be investigated in order to understand how branding works in small business and to better capture the role of branding in small business.

  10. Small bowel resection - discharge

    Science.gov (United States)

    ... incision is red, warm, swollen, or more painful Short of breath or chest pain Swollen legs or pain in your calves Alternative Names Small intestine surgery - discharge; Bowel resection - small intestine - discharge; Resection of ...

  11. Securitization and small business

    OpenAIRE

    Wilcox, James A.

    2011-01-01

    Small businesses have relied considerably on securitized markets for credit. The recent financial crisis led to a virtual cessation of securitization of some of the loans used by small businesses, such as commercial real estate mortgages, vehicle, and credit card loans. In addition, values of commercial and residential real estate, which small businesses often use as collateral for loans, dropped dramatically. As a consequence, small businesses may have experienced tighter credit conditions t...

  12. Chairing a Small Department.

    Science.gov (United States)

    Bowker, Lee H.; Lynch, David M.

    Ten management problems for chairs of small departments in small colleges are discussed, along with problem-solving strategies for these administrators. Serious disagreements within a small and intimate department may create a country club culture in which differences are smoothed over and the personal idiosyncrasies of individual members are…

  13. 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...

  14. Molecular diagnosis reveals genetic heterogeneity for the overlapping MKKS and BBS phenotypes.

    Science.gov (United States)

    Schaefer, Elise; Durand, Myriam; Stoetzel, Corinne; Doray, Bérénice; Viville, Brigitte; Hellé, Sophie; Danse, Jean-Marc; Hamel, Christian; Bitoun, Pierre; Goldenberg, Alice; Finck, Sonia; Faivre, Laurence; Sigaudy, Sabine; Holder, Muriel; Vincent, Marie-Claire; Marion, Vincent; Bonneau, Dominique; Verloes, Alain; Nisand, Israël; Mandel, Jean-Louis; Dollfus, Hélène

    2011-01-01

    Hydrometrocolpos and polydactyly diagnosed in the prenatal period or early childhood may raise diagnostic dilemmas especially in distinguishing McKusick-Kaufman syndrome (MKKS) and the Bardet-Biedl syndrome (BBS). These two conditions can initially overlap. With time, the additional features of BBS appearing in childhood, such as retinitis pigmentosa, obesity, learning disabilities and progressive renal dysfunction allow clear differentiation between BBS and MKKS. Genotype overlap also exists, as mutations in the MKKS-BBS6 gene are found in both syndromes. We report 7 patients diagnosed in the neonatal period with hydrometrocolpos and polydactyly who carry mutations in various BBS genes (BBS6, BBS2, BBS10, BBS8 and BBS12), stressing the importance of wide BBS genotyping in patients with this clinical association for diagnosis, prognosis and genetic counselling. PMID:21044901

  15. Specialized Cilia in Mammalian Sensory Systems

    Directory of Open Access Journals (Sweden)

    Nathalie Falk

    2015-09-01

    Full Text Available Cilia and flagella are highly conserved and important microtubule-based organelles that project from the surface of eukaryotic cells and act as antennae to sense extracellular signals. Moreover, cilia have emerged as key players in numerous physiological, developmental, and sensory processes such as hearing, olfaction, and photoreception. Genetic defects in ciliary proteins responsible for cilia formation, maintenance, or function underlie a wide array of human diseases like deafness, anosmia, and retinal degeneration in sensory systems. Impairment of more than one sensory organ results in numerous syndromic ciliary disorders like the autosomal recessive genetic diseases Bardet-Biedl and Usher syndrome. Here we describe the structure and distinct functional roles of cilia in sensory organs like the inner ear, the olfactory epithelium, and the retina of the mouse. The spectrum of ciliary function in fundamental cellular processes highlights the importance of elucidating ciliopathy-related proteins in order to find novel potential therapies.

  16. Study of cilia assembly in Tetrahymena and the role of cytosolic chaperonin CCT

    OpenAIRE

    Seixas, Ana Cecília Fernandes, 1974-

    2008-01-01

    Os cílios são organelos conservados evolutivamente que são requeridos num vasto número de processos celulares tais como locomoção, quimiotaxia, movimento de fluídos e transdução de sinais. Nos últimos anos, um grande número de publicações tem demonstrado o impacto que pequenas alterações no correcto funcionamento dos cílios tem no Homem. Várias doenças humanas que se caracterizam por quadros clínicos complexos, como o síndrome de Bardet-Biedl (BBS) e o síndrome de Meckel-Gruber (MKS) foram re...

  17. Inherited retinal disorders in South Africa and the clinical impact of evolving technologies.

    Science.gov (United States)

    Roberts, L; Goliath, R; Rebello, G; Bardien, S; September, A V; Bartmann, L; Loubser, F; Greenberg, L J; Ramesar, R S

    2016-01-01

    Retinal degenerative disorders (RDDs) encompass a group of inherited diseases characterised by vision loss. The genetic and clinical complexity poses a challenge in unravelling the molecular genetic aetiology of this group of disorders. Furthermore, the population diversity in South Africa (SA) presents researchers with a particularly complicated task. Rapid advances in the development of cutting-edge technological platforms over the past two decades, however, have assisted in overcoming some of the challenges. The RDD research team has utilised these escalating technologies, which has facilitated a corresponding increase in molecular diagnoses. A biorepository has been established and comprises ~3 200 patient DNA samples archived with many forms of RDD (including retinitis pigmentosa, macular dystrophies, Stargardt disease, Leber congenital amaurosis, Usher syndrome and Bardet Biedl syndrome). A comprehensive review is presented of the SA journey spanning 25 years, into elucidating the molecular genetic basis of various forms of RDD in SA. PMID:27245521

  18. 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.

  19. 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.

  20. Development of Small Company

    OpenAIRE

    Fiedler, Viktor

    2015-01-01

    The subject of bachelor thesis is analysis of possibilities and directions of the development of small company. It describes the structure of small company and the problems of its development. It analyses present economical, financial and business situation inside and outside the company. In the second part author introduces some proposals for improving several parts of company.

  1. Small States in Europe

    DEFF Research Database (Denmark)

    This book offers an accessible, coherent and informative analysis of contemporary and future foreign policy challenges facing small states in Europe.......This book offers an accessible, coherent and informative analysis of contemporary and future foreign policy challenges facing small states in Europe....

  2. Small Mammal Intrigue.

    Science.gov (United States)

    Cristol, Daniel A.

    1985-01-01

    Gives introductory information about the study of small mammals including the selection and use of harmless live-traps, handling and identification, techniques for observation and trapping in the wild, and safety measures. Suggests useful references for teachers wishing to develop a small mammal study program for their students. (JHZ)

  3. 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

  4. Wind: small is beautiful

    Energy Technology Data Exchange (ETDEWEB)

    Vries, E. de

    2005-10-01

    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.

  5. Wind: small is beautiful

    International Nuclear Information System (INIS)

    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

  6. 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

  7. Small satellites - An overview

    Science.gov (United States)

    Ward, A. K.

    The present review of small satellites examines spacecraft activities in the U.K. and compiles a checklist of advantages and applications for the class. These advantages are illustrated with references to recent small satellite missions and technologies developed to facilitate such launches and projects. Specific programs examined include AMPTE-UKS, Viking, and the UoSAT program, and information is given regarding the Small Explorer program, the RAE Space Technology Research Vehicle, the AEA Argos Program, and space research programs in both Japan and India. Low-cost launches are shown to be available in the form of the Ariane Structure for Auxiliary Payloads, the Pegasus and Delta vehicles, and with the Shuttle Free-flying Getaway Special. Small-satellite technologies that play key roles in their effective implementation are: structure/thermal advances, attitude control systems, on-board communications, and power and data-handling systems.

  8. 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) Symptoms The most common symptoms are: Abdominal ...

  9. Small & Large Intestine

    Science.gov (United States)

    ... the large intestine produces no digestive enzymes. Chemical digestion is completed in the small intestine before the chyme reaches the large intestine. Functions of the large intestine include the absorption of water and electrolytes and the elimination of ...

  10. Small bowel resection

    Science.gov (United States)

    ... reconnect, your surgeon makes an opening called a stoma through the skin of your belly. The small ... of your belly. Stool will go through the stoma into a drainage bag outside your body. This ...

  11. 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 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 devices, Part II: Cartographic design for small-display devices, Part III: Study on the GiMoDig Client...... ? Portal 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...

  12. 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...

  13. Small Mammal Trapping 2003

    Data.gov (United States)

    US Fish and Wildlife Service, Department of the Interior — Small mammal traps were placed in the Baring division and in the Edmunds division of Moosehom National Wildlife Refuge. There were a total of 98 traps set for up to...

  14. Research using small tokamaks

    International Nuclear Information System (INIS)

    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

  15. SMALL SCALE MORPHODYNAMICAL MODELLING

    Institute of Scientific and Technical Information of China (English)

    D. Ditschke; O. Gothel; H. Weilbeer

    2001-01-01

    Long term morphological simulations using complete coupled models lead to very time consuming computations. Latteux (1995) presented modelling techniques developed for tidal current situations in order to reduce the computational effort. In this paper the applicability of such methods to small scale problems is investigated. It is pointed out that these methods can be transferred to small scale problems using the periodicity of the vortex shedding process.

  16. 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.

  17. UNDERSTANDING SMALL BUSINESS SCAMS

    OpenAIRE

    Michael T. Schaper; PAUL WEBER

    2012-01-01

    This paper provides an overview of the current state of knowledge about small business scams. A scam is a form of dishonest action, based upon an invitation to participate in an activity. Victims are encouraged, mislead or induced to voluntarily interact with the perpetrator, and ultimately to willingly surrender over money, information or other valuable resources. Common forms of scams directed towards small business include phishing, false business valuations and sales, fake overpayments, f...

  18. Radionuclide Small Intestine Imaging

    OpenAIRE

    Jiri Dolezal; Marcela Kopacova

    2013-01-01

    The aim of this overview article is to present the current possibilities of radionuclide scintigraphic small intestine imaging. Nuclear medicine has a few methods—scintigraphy with red blood cells labelled by means of 99mTc for detection of the source of bleeding in the small intestine, Meckel's diverticulum scintigraphy for detection of the ectopic gastric mucosa, radionuclide somatostatin receptor imaging for carcinoid, and radionuclide inflammation imaging. Video capsule or deep enteroscop...

  19. 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....

  20. Small simple impact craters

    CERN Document Server

    Sparavigna, Amelia Carolina

    2010-01-01

    The paper discusses some examples of image processing applied to improve optical satellite imagery of small craters (Kamil, Veevers, Haviland). The examples show that image processing can be quite useful for further in-situ researches, because the resultant imagery helps to have a better picture of the crater shape and of the distribution of debris about it. The paper is also disclosing an interesting underwater structure, with shape and size of a small crater, located on the coast-line of Sudan.

  1. 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...

  2. Gas Quenching Small Components

    Institute of Scientific and Technical Information of China (English)

    Paul Stratton

    2004-01-01

    Gas quenching is an environmentally friendly and effective way of quenching components. However it has not been possible to apply it to small components because of the difficulties of jigging. A method of gas quenching such small components is described in which they are levitated in a confined gas stream. The method is suitable for quenching low alloy steel components that weigh between 1 and 10g.

  3. 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.

  4. 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. PMID:27168147

  5. Small Animal Retinal Imaging

    Science.gov (United States)

    Choi, WooJhon; Drexler, Wolfgang; Fujimoto, James G.

    Developing and validating new techniques and methods for small animal imaging is an important research area because there are many small animal models of retinal diseases such as diabetic retinopathy, age-related macular degeneration, and glaucoma [1-6]. Because the retina is a multilayered structure with distinct abnormalities occurring in different intraretinal layers at different stages of disease progression, there is a need for imaging techniques that enable visualization of these layers individually at different time points. Although postmortem histology and ultrastructural analysis can be performed for investigating microscopic changes in the retina in small animal models, this requires sacrificing animals, which makes repeated assessment of the same animal at different time points impossible and increases the number of animals required. Furthermore, some retinal processes such as neurovascular coupling cannot be fully characterized postmortem.

  6. 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.

  7. 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

  8. Small is beautiful

    Energy Technology Data Exchange (ETDEWEB)

    Woof, M.; Casteel, K.

    1997-12-01

    While safety standards in modern mechanised underground coal mines in Britain are good, so are standards in their smaller competitors using much older equipment. Working practices and conditions at several Federation of Independent Mines (FIM) operations are described, detailing the use of equipment and methods that would be considered obsolete in larger mines. Although these mines are small the high quality of the coal that they mine means that they are economic. Drilling equipment used in small mines, and scoops are also described. 7 photos. 1 tab.

  9. 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

  10. Restless legs syndrome: association with streptococcal or mycoplasma infection.

    Science.gov (United States)

    Matsuo, Muneaki; Tsuchiya, Katsunori; Hamasaki, Yuhei; Singer, Harvey S

    2004-08-01

    Group A beta-hemolytic streptococcal infections have been reported to cause neuropsychiatric symptoms, such as chorea, tics, and obsessive-compulsive disorder, presumably through autoimmune damage to basal ganglia. Mycoplasma pneumoniae infections have also been reported to cause damage to the basal ganglia. Restless legs syndrome is a movement disorder with focal restlessness, an irresistible desire to move, and exacerbation by long periods of sitting or lying. We present three children with transient restless legs syndrome-like symptoms possibly associated with group A beta-hemolytic streptococcal infection or Mycoplasma pneumoniae infection. One of three patients had persistently elevated enzyme-linked immunosorbent optical density values against human caudate and putamen. PMID:15301831

  11. [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. PMID:17040633

  12. 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.

  13. Anticonvulsant hypersensitivity syndrome associated with carbamazepine administration: Case series

    Directory of Open Access Journals (Sweden)

    Maulin Mehta

    2014-01-01

    Full Text Available Hypersensitivity reactions are common adverse drug reactions (ADRs associated with antiepileptics. Carbamazepine is one of the routinely prescribed drugs for the treatment of epilepsy and neuropathic pain. ADRs due to carbamazepine range from mild maculopapular rash to severe anticonvulsant hypersensitivity syndrome (AHS. AHS is the triad of fever, rash, and internal organ involvement occurring 1-8 weeks after exposure to an anticonvulsant (1 in 1,000 to 10,000 exposures. Spontaneously reported three cases of AHS-drug hypersensitivity reactions induced by carbamazepine are discussed here. Seven to ten days after starting therapy, patients developed maculopapular skin rashes, fever and liver or kidney involvement. The causal relationship between drug and ADR was found to be ′certain′ in one case and ′probable′ in other two cases with both WHO-UMC and Naranjo causality assessment scale. All the three cases show category 4a according to Hartwig′s severity scale as ADR was the cause for hospital admission. On assessing preventability of ADRs by modified Schumock and Thorntons′ scale, one case was falling into category of ′definitely preventable′ and other two were ′not preventable′. AHS is rare but serious reaction with carbamazepine which requires vigilant monitoring by physicians to avoid major consequences.

  14. Devic syndrome associated to systemic lupus erythematosus. Case report.

    Directory of Open Access Journals (Sweden)

    Juan Camilo Botero

    2009-11-01

    Full Text Available El síndrome de Devic es una las enfermedades desmielinizantes del sistema nervioso central, de etiología desconocida. Se relaciona mucho con el lupus eritematoso sistémico siendo una complicación rara y grave. Este síndrome se presenta como mielitis transversa de compromiso a nivel cervical y neuritis óptica. Se describe el caso de de una paciente de 32 años, que se presentó al servicio de urgencias por dolor en región torácica, pérdida de la fuerza muscular progresiva en los miembros inferiores, pérdida del control del esfínter urinario y disminución de la agudeza visual. También relató antecedentes de lupus eritematoso sistémico. Al examen físico se encontró atrofia y palidez del disco óptico derecho, disminución en la fuerza muscular 3/5 en miembros superiores e inferiores, alteración en la sensibilidad y propiocepción de extremidades inferiores. La resonancia magnética mostró hallazgos sugestivos de mielitis transversa de C2 a C6. En el laboratorio se encontraron anticuerpos anticardiolipina elevados y anticuerpos antinucleares: 1:320. Se manejó con esteroides, hubo notable mejoría en los síntomas y se le dio de alta al sexto día, con tratamiento ambulatorio. Los hallazgos al examen físico, laboratorio e imagenología permitieron hacer el diagnóstico de síndrome de Devic. Con base en la revisión bibliográfica, se concluye que este síndrome es poco frecuente; sin embargo, se debe tener en cuenta como posible diagnóstico en todo paciente con antecedentes de lupus eritematoso sistémico y síntomas compatibles.

  15. Devic syndrome associated to systemic lupus erythematosus. Case report.

    OpenAIRE

    Juan Camilo Botero; Alejandro García; Juliana Hidalgo

    2009-01-01

    El síndrome de Devic es una las enfermedades desmielinizantes del sistema nervioso central, de etiología desconocida. Se relaciona mucho con el lupus eritematoso sistémico siendo una complicación rara y grave. Este síndrome se presenta como mielitis transversa de compromiso a nivel cervical y neuritis óptica. Se describe el caso de de una paciente de 32 años, que se presentó al servicio de urgencias por dolor en región torácica, pérdida de la fuerza muscular progresiva en los miembros inferio...

  16. A Case of Gardner's Syndrome Associated with Desmoid Tumor

    OpenAIRE

    Chung, Jimin; Namkoong, Sun; Jung, Kyoung Eun; Park, Jong Wook; Park, Byung Cheol; Cinn, Yong Woo; Kim, Myung Hwa

    2010-01-01

    Desmoid tumors are uncommon benign neoplasm of the fibroblasts. They occur rarely in the general population, but they are comparatively common in patients with familial polyposis coli with or without other elements of Gardner's syndrome. Herein, we report a 16-year-old woman with Gardner's syndrome complicated by desmoid tumors on the right subscapular area.

  17. A case of William's syndrome associated peripheral pulmonary arterial stenosis

    International Nuclear Information System (INIS)

    William's syndrome, in order to more completely delineate the total spectrum of the disorder, indicates that 'infantile hypercalcemia', 'peculiar facies' and 'supravalvular aortic stenosis.' In has other many vascular anomalies, such as peripheral pulmonary arterial stenosis, coronary arterial stenosis, celiac arterial stenosis, and renal aterial stenosis. Only 32% of the patients have evidence of supravalvular aortic stenosis. And it is very rare disease entity that has been reported rarely in Korea. Recently authors experienced a case that was questioned William's syndrome with peripheral pulmonary arterial stenosis, clinically and preliminary radiologically and this case was confirmed by operation. Here we report a case of William's syndrome with peripheral pulmonary arterial stenosis and reviewed literatures

  18. Sweet’s syndrome associated with Hashimoto’s thyroiditis

    OpenAIRE

    Francisco, Christina Raissa I; Patal, Perpetua C; Cubillan, Eileen A; Isip-Tan, Iris Thiele

    2011-01-01

    Sweet’s syndrome or acute febrile neutrophilic dermatosis is characterised by the abrupt onset of painful erythematous plaques or nodules, pyrexia (>38°F) and histopathologic evidence of a dense neutrophilic infiltrate without vasculitis. It has been reported in association with many diseases, however, its association with Hashimoto’s thyroiditis is rare. A 47-year-old Filipino woman with a 30-year history of an asymptomatic anterior neck mass developed painful, erythematous annular plaques o...

  19. Sweet Syndrome Associated with Upper Respiratory Infection and Amoxicillin Use.

    Science.gov (United States)

    Volpe, Mark

    2016-01-01

    Sweet syndrome (acute febrile neutrophilic dermatosis) is an uncommon dermatologic eruption characterized by acute onset of painful papules, plaques or nodules on the skin that are red, blue, or violaceous in color. It has been associated with various infections, medications, and malignancies. Here we report the case of a middle-aged male who presents with Sweet syndrome after an upper resipiratory infection and while using amoxicillin. We also review the diagnostic criteria, laboratory testing, and treatment options. PMID:27186450

  20. Sweet's syndrome associated with cellulitis - a challenging diagnosis*

    OpenAIRE

    Resende, C; Santos, R.; T. Pereira; Brito, C.

    2016-01-01

    Sweet's syndrome is a neutrophilic dermatosis with worldwide distribution that has been associated with inflammatory autoimmune diseases, infections, malignancies, drugs, and pregnancy. The disease is idiopathic in up to 50% of patients. A 64-year-old woman, diagnosed with right limb cellulitis (4 days of evolution), was seen at our department, due to persistent cellulitis and progressive appearance of painful nodules and plaques in both shins and the right forearm (2 days of evolution). Take...

  1. Sweet Syndrome Associated with Pregnancy: A Case Report

    Directory of Open Access Journals (Sweden)

    Seval Doğruk Kaçar

    2015-10-01

    Full Text Available Acute febrile neutrophilic dermatosis also known as Sweet Syndrome is an inflammatory skin disease with an unknown etiopathogenesis, characterized clinically by sudden onset, painful papules, nodules and plaques and histopathologically dense dermal neutrophilic infiltrate. Malignancies, drugs, infections, systemic inflammatory diseases and rarely pregnancy are all considered in the etiology. Here is a patient diagnosed as Sweet Syndrome, with lesions appeared shortly after delivery in her first pregnancy and in the last trimester in her second pregnancy. Her lesions quickly responded to systemic steroid treatment.

  2. Usher syndrome associated with Fuchs’ heterochromic uveitis: a case report

    Directory of Open Access Journals (Sweden)

    Turan-Vural E

    2011-04-01

    Full Text Available Ece Turan-Vural, Banu Torun-Acar, Nejla Tükenmez, M Şahin Sevim, Bulent Buttanri, Suphi AcarOphthalmology Clinic, Haydarpasa Numune Education and Research Hospital, Istanbul, TurkeyAbstract: We report a case of Usher syndrome in association with unilateral Fuchs' heterochromic uveitis.Keywords: Fuchs’ heterochromic uveitis, Usher syndrome, deafness, blindness

  3. Posterior compartment syndrome associated with clopidogrel therapy following trivial trauma

    OpenAIRE

    Byrne, A‐M; Kearns, S. R.; Kelly, E P

    2006-01-01

    Haematomata caused by blunt trauma may potentially induce a compartment syndrome by raising intra‐compartmental pressure. We report a case of acute posterior compartment syndrome following minimal trauma to the leg of an elderly patient on the antiplatelet agent clopidogrel. This case highlights the high index of clinical suspicion required to detect compartment syndrome in those on long term antiplatelet therapy and prompt surgical decompression is recommended.

  4. Wells' Syndrome Associated with Churg-Strauss Syndrome

    OpenAIRE

    Lee, Soo Hyun; Roh, Mi Ryung; Jee, Hyunjoong; Chung, Kee Yang; Jung, Jin Young

    2011-01-01

    Churg-Strauss syndrome (CSS) is a multisystem granulomatous vasculitis that is characterized by peripheral eosinophilia and the infiltration of eosinophils into systemic organs. The skin lesions of CSS consist mainly of palpable purpura and nodules. Wells' syndrome (WS) is a rare inflammatory dermatosis that is associated with recurrent granulomatous dermatitis and eosinophilia. Since these two diseases are rare, any overlap between them is very unusual. Herein, we report a patient with CSS, ...

  5. Contracted foal syndrome associated with multiple malformations in two foals.

    Science.gov (United States)

    Binanti, D; Zani, D D; De Zani, D; Turci, T; Zavaglia, G; Riccaboni, P

    2014-02-01

    Congenital anomalies in horses are very rare, and contracted foal syndrome is one of the most commonly reported. This malformation is characterized by contraction of the joints of the forelimbs and/or hindlimbs. In addition, the syndrome can be characterized by vertebral column malformations, such as scoliosis or torticollis, and cranial deformity. The present report describes the radiological and necroscopical findings of multiple rare malformations in two foals. Both foals showed skeletal abnormalities and fenestration of the abdominal cavity. Other pathological findings include a interventricular septal defect in one and a unilateral hydronephrosis and partial hydroureter in the other foal. Although in this report a specific aetiology could not be provided, insecticides treatment provided during the second month of pregnancy might play a role in the pathogenesis of these malformations. PMID:23406278

  6. Reversible sinusoidal obstruction syndrome associated with tacrolimus following liver transplantation

    OpenAIRE

    Shen, Tian; Feng, Xiao-Wen; Geng, Lei; Zheng, Shu-Sen

    2015-01-01

    Sinusoidal obstruction syndrome (SOS), previously known as hepatic veno-occlusive disease, is a rare disorder in solid organ transplant patients, and is an uncommon complication after liver transplantation. Severe SOS with hepatic failure causes considerable mortality. Tacrolimus has been reported to be an offending agent, which potentially plays a role in the pathophysiological process of SOS. SOS due to tacrolimus has been reported in lung and pancreatic transplantations, but has never been...

  7. Reversible sinusoidal obstruction syndrome associated with tacrolimus following liver transplantation

    Science.gov (United States)

    Shen, Tian; Feng, Xiao-Wen; Geng, Lei; Zheng, Shu-Sen

    2015-01-01

    Sinusoidal obstruction syndrome (SOS), previously known as hepatic veno-occlusive disease, is a rare disorder in solid organ transplant patients, and is an uncommon complication after liver transplantation. Severe SOS with hepatic failure causes considerable mortality. Tacrolimus has been reported to be an offending agent, which potentially plays a role in the pathophysiological process of SOS. SOS due to tacrolimus has been reported in lung and pancreatic transplantations, but has never been described in a liver transplant recipient. Herein, we present a case of SOS after liver transplantation, which was possibly related to tacrolimus. A 27-year-old man developed typical symptoms of SOS with painful hepatomegaly, ascites and jaundice after liver transplantation, which regressed following withdrawal of tacrolimus. By excluding other possible predisposing factors, we concluded that tacrolimus was the most likely cause of SOS. PMID:26034381

  8. Catatonic syndrome associated with lead intoxication: a case report

    OpenAIRE

    Modabbernia, Mohammad Jafar; Mirsafa, Ali Reza; Modabbernia, Amirhossein; Pilehroodi, Farhad; Shirazi, Maryam

    2009-01-01

    Introduction Little is known about catatonia associated with lead intoxication. Case presentation A retired printing house worker man presented with one week history of refusal to eat and mutism. He was treated with possible diagnosis of catatonia with administration of Lorazepam 3 mg P.O. daily. Significant improvement occurred after 48 hours. In further examinations, there was no evidence of physical and mental disorders while impairment in neuropsychiatry test, identification of Dohle body...

  9. Macrophage Activation Syndrome-Associated Markers in Severe Dengue

    OpenAIRE

    Ab-Rahman, Hasliana Azrah; Rahim, Hafiz; AbuBakar, Sazaly; Wong, Pooi-Fong

    2016-01-01

    Hemophagocytosis, a phenomenon of which activated macrophages phagocytosed hematopoietic elements was reportedly observed in severe dengue patients. In the present study, we investigated whether markers of macrophage activation syndrome (MAS) can be used as differential diagnostic markers of severe dengue. Two hundred and eight confirmed dengue patients were recruited for the study. Sandwich ELISA was used to determine serum ferritin, soluble CD163 (sCD163), and soluble CD25 (sCD25) levels. T...

  10. 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.

  11. Meige's syndrome associated with basal ganglia and thalamic functional disorders

    International Nuclear Information System (INIS)

    Magnetic resonance imaging (MRI) or single positron emission computed tomography (SPECT) or both were performed and the responses of surface electromyography (EMG) were examined in seven cases of Meige's syndrome. MRI or SPECT or both demonstrated lesions of the basal ganglia, the thalamus, or both in five of the cases. Surface EMG revealed abnormal burst discharges in the orbicularis oculi and a failure of reciprocal muscular activity between the frontalis and orbicularis oculi in all the cases. These findings suggest that voluntary motor control and reciprocal activity in the basal ganglia-thalamocortical circuits are impaired in Meige's syndrome. In addition, good responses were seen to clonazepam, tiapride and trihexyphenidyl in these cases. Therefore, we conclude that dopaminergic, cholinergic, and γ-aminobutyric acid (GABA) ergic imbalances in the disorders of the basal ganglia and thalamus in Meige's syndrome cause control in the excitatory and inhibitory pathways to be lost, resulting in the failure of integration in reciprocal muscular activity and voluntary motor control. This failure subsequently causes the symptoms of Meige's syndrome. (author)

  12. 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.

  13. 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.

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

    Science.gov (United States)

    Nalini, A.; Desai, Anita; Mahato, Simendra Kumar

    2009-01-01

    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. PMID:20142861

  15. Ehlers-Danlos syndrome associated with cleft lip and palate

    OpenAIRE

    Tan, Pearlie WW; Song, Colin; Lalonde, Donald

    2009-01-01

    Ehlers-Danlos syndrome (EDS) is a hereditary connective tissue disorder caused by abnormal collagen synthesis and presents with classic features of hyperextensibility, fragility of skin and joint hypermobility. It has not been commonly described as being associated with cleft lip and/or palate, and a literature search has disclosed only one case (1984) of type IV EDS with cleft lip and palate. The authors have seen another case of EDS associated with cleft lip and palate. The etiology and cli...

  16. Immune reconstitution inflammatory syndrome associated with biologic therapy.

    Science.gov (United States)

    Gupta, Malika; Jafri, Kashif; Sharim, Rebecca; Silverman, Susanna; Sindher, Sayantani B; Shahane, Anupama; Kwan, Mildred

    2015-02-01

    The use of biologics in the treatment of autoimmune disease, cancer, and other immune conditions has revolutionized medical care in these areas. However, there are drawbacks to the use of these medications including increased susceptibility to opportunistic infections. One unforeseen risk once opportunistic infection has occurred with biologic use is the onset of immune reconstitution inflammatory syndrome (IRIS) upon drug withdrawal. Although originally described in human immunodeficiency virus (HIV) patients receiving highly active antiretroviral therapy, it has become clear that IRIS may occur when recovery of immune function follows opportunistic infection in the setting of previous immune compromise/suppression. In this review, we draw attention to this potential pitfall on the use of biologic drugs. PMID:25504263

  17. Neonatal Bartter syndrome associated with ileal atresia and cystic fibrosis

    OpenAIRE

    Akuma, A. O.; Mittal, S K; Sambo, A. A.

    2013-01-01

    A rare case of neonatal Bartter syndrome presenting with severe hyperkalemia is reported in a preterm child born to consanguineous parents. This child also had ileal atresia, and meconium plugs were found at laparotomy. The diagnosis of cystic fibrosis was subsequently made on genetic testing. Despite full intensive care management and surgical interventions, he died of respiratory failure after 70 days. This is the first reported case of such conglomeration of pathologies in a newborn child....

  18. Neuroleptic Malignant Syndrome Associated with Colon-Cancer

    OpenAIRE

    Katsuhiko Tsukada; Hiroshi Azuhata; Hiroaki Yonekura; Masashi Haraguchi; Hiroyuki Katoh; Hitoshi Kimura; Hiroyuki Kuwano

    2007-01-01

    Neuroleptic malignant syndrome (NMS), a potentially hazardous side effect of antipsychotic agents, usually develops within 10 days after receiving these agents. We describe a 65-year-old male who developed NMS after taking neuroleptics for alcohol dependence syndrome for several years. He was successfully treated by stopping these agents but three months later, an advanced sigmoid colon cancer was detected. He underwent surgery and the clinical course was uneventful. The present case is rare ...

  19. Neuroleptic Malignant Syndrome Associated with Refractory Acute Disseminated Encephalomyelitis

    Directory of Open Access Journals (Sweden)

    Silvia R. Delgado

    2016-04-01

    Full Text Available We present the case of a young man who was transferred to our hospital with worsening acute disseminated encephalomyelitis (ADEM despite treatment with intravenous methylprednisolone, intravenous immunoglobulin and plasma exchange. He developed neuroleptic malignant syndrome (NMS without the use of dopamine-modulating drugs. His progressive clinical improvement started after treatment with intravenous cyclophosphamide and methylprednisolone. In our patient, acute demyelination with severe bilateral inflammation of the basal ganglia could have caused a state of central dopamine depletion, creating proper conditions for the development of NMS. Significant clinical improvement of our case after treatment with intravenous cyclophosphamide and steroids provides further evidence for a possible role of the inflammatory lesions in the pathogenesis of NMS in association with ADEM.

  20. Is pseudoexfoliation syndrome associated with coronary artery disease?

    Directory of Open Access Journals (Sweden)

    Mehmet Yunus Emiroglu

    2010-01-01

    Full Text Available Background: Pseudoexfoliation syndrome (PEX is recognised by chronic deposition of abnormal pseudoexfoliation material on anterior segment structures of the eye, especially the anterior lens capsule. In recent years, several studies have shown the presence of vascular, cardiac and other organ pseudoexfoliative material in patients with ocular pseudoexfoliation. Aims : The purpose of this study is to determine whether an association exists between ocular pseudoexfoliation and coronary artery disease, aortic aneurysms and peripheric vascular disease. Patients and Methods: 490 patients who underwent coronary angiography (CAG at Kosuyolu Cardiovascula Research and Training Hospital were included in the study. Patients were evaluated for conventional risk factors such as age, sex, family history, hypertension, diabetes, dislipidemia and smoking. Detailed eye examinations including evaluation of lens were done in all patients. The presence of PEX material in the anterior segment was best appreciated by slit lamp after pupillary dilation. The patients were divided into two groups according to the presence of PEX, and compared for the presence of CAD and other risk factors. Results: CAD was present in 387 patients. 103 patients had normal coronary angiography. 20 (5.2 % of CAD patients and 4 (3.9% of normal CAG patients were found to have PEX (p>0.05. There was no significant relationship between CAD and the presence of PEX (p>0.05. When patients were grouped according to the presence of PEX, only age was significantly different between the two groups (r: 0.25, p<0.001. Conclusion: There is no significant relationship between the presence of PEX and CAD. Further studies in larger scales with elderly population may be more valuable.

  1. [Fructose Consumption and the Metabolic Syndrome: Association or Causality?].

    Science.gov (United States)

    Gerber, Philipp A

    2016-06-22

    Fructose consumption has increased significantly during the past decades – in particular by using added sugar in food and beverages, either sugars containing free fructose, but also sugars containing fructose in bound form (e. g. sucrose). The metabolism of fructose exhibits distinct differences compared to the metabolism of glucose. Association studies performed in the past years suggest an association of fructose consumption and adverse effects on metabolism. Intervention studies, conducted in part with healthy individuals, could prove such effects and deliver explanations of the mechanisms leading to these adverse effects. A reduction of consumption of added fructose should be recommended, but there is no evidence to support a restriction of fruit consumption (as a natural source of fructose). PMID:27329707

  2. Hemophagocytic lymphohistiocytosis as a paraneoplastic syndrome associated with ovarian dysgerminoma.

    Science.gov (United States)

    Nosratian-Baskovic, Mana; Tan, Brent; Folkins, Ann; Chisholm, Karen M; Dorigo, Oliver

    2016-08-01

    •Ovarian dysgerminoma associated with paraneoplastic fever, cytopenia and splenomegaly•Complete symptom resolution resulted from tumor resection and medical management•Non-hematolymphoid neoplasms are part of differential diagnosis in secondary HLH. PMID:27354999

  3. Bilateral carpal tunnel syndrome associated with interleukin 2 therapy.

    OpenAIRE

    Heys, S. D.; Mills, K. L.; Eremin, O.

    1992-01-01

    We report the development of synchronous bilateral carpal tunnel syndrome in a woman with metastatic colorectal cancer, undergoing treatment with recombinant interleukin 2. A carpal tunnel decompression was carried out on the hand which was more severely affected, with a gradual recovery in median nerve function. To the best of our knowledge, this is the first reported case of carpal tunnel syndrome in association with recombinant interleukin 2.

  4. Acute respiratory distress syndrome associated with severe ulcerative colitis

    Institute of Scientific and Technical Information of China (English)

    Shiho; Sagara; Yasuo; Horie; Yumiko; Anezaki; Hideaki; Miyazawa; Masahiro; Iizuka

    2010-01-01

    Various extraintestinal manifestations including pulmonary abnormalities have been reported in patients with ulcerative colitis. Acute respiratory distress syndrome (ARDS) is a serious and fatal pulmonary manifestation. We have experienced a 67-year-old male patient with ARDS associated with a severe type of ulcerative colitis (UC). Severe dyspnea symptoms occurred during the treatment of UC in a previous hospital and the patient was transferred to our hospital on June 27, 2007. Both blood and sputa culture...

  5. 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.

  6. Acquired immunodeficiency syndrome associated with blood-product transfusions

    International Nuclear Information System (INIS)

    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

  7. 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...

  8. 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...

  9. 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.

  10. Guillain-Barré syndrome associated with tuberculosis.

    OpenAIRE

    Vyravanathan, S; Senanayake, N

    1983-01-01

    Two cases of Guillain-Barré syndrome occurring in association with chronic pulmonary tuberculosis are reported. A cell-mediated, delayed hypersensitivity reaction to, or invasion of the nerve roots by tubercle bacilli would seem to be the likely explanation of the neuropathy.

  11. Ehlers-Danlos Syndrome associated with cardiomyopathy hypertrophic obstructive*

    OpenAIRE

    Pinto, Raimundo José Almeida de Oliveira; dos Santos, Adaílton Araújo; Azevedo, Mablo de Castro; Meira, Saulo Sacramento

    2015-01-01

    Ehlers-Danlos syndrome is a rare clinical condition caused by a genetic change that results in the formation of structurally or functionally altered collagen. The clinical manifestations are varied, being the most obvious skin hypermotility and increased joint flexibility, although other systems - such as cardiovascular, respiratory and neurological - may also be affected. This paper presents the report of a patient who sought medical attention with complaints of atypical chest pain. Clinical...

  12. Ehlers-Danlos Syndrome associated with cardiomyopathy hypertrophic obstructive.

    Science.gov (United States)

    Pinto, Raimundo José Almeida de Oliveira; Santos, Adaílton Araújo dos; Azevedo, Mablo de Castro; Meira, Saulo Sacramento

    2015-01-01

    Ehlers-Danlos syndrome is a rare clinical condition caused by a genetic change that results in the formation of structurally or functionally altered collagen. The clinical manifestations are varied, being the most obvious skin hypermotility and increased joint flexibility, although other systems - such as cardiovascular, respiratory and neurological - may also be affected. This paper presents the report of a patient who sought medical attention with complaints of atypical chest pain. Clinical evaluation enabled hypothetical diagnosis of hypertrophic obstructive cardiomyopathy and Ehlers-Danlos syndrome. Initial electrocardiogram, echocardiogram and 24 hours holter allowed the confirmation of the first hypothesis. A skin biopsy performed later associated clinical data and confirmed the second hypothesis. PMID:26312722

  13. Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia

    Institute of Scientific and Technical Information of China (English)

    Siham Al Sinani; Yusria Al Rawahi; Hamed Abdoon

    2012-01-01

    A number of disorders have been described to cause protein losing enteropathy (PLE) in children.Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE.Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them.The principal treatment for PIL is a high protein,low fat diet with medium chain triglycerides supplementation.Supportive therapy includes albumin infusion.Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL.There are no publications on the treatment of PIL with octreotide in patients with HS.We report two children with HS and PLE in which we used octreotide to decrease intestinal protein loss.In one patient,octreotide increased serum albumin to an acceptable level without further need for albumin infusions.The other patient responded more dramatically with near normal serum albumin levels and cessation of albumin infusions.In achieving a good response to octreotide in both patients,we add to the publications supporting the use of octreotide in PIL and suggest that octreotide should be tried in patients with PIL secondary to HS.To the best of our knowledge,this is the first case report on the use of octreotide in HSassociated PIL.

  14. Burning mouth syndrome associated with varicella zoster virus.

    Science.gov (United States)

    Nagel, Maria A; Gilden, Don

    2016-01-01

    We present two cases of burning mouth syndrome (BMS)-of 8-month duration in a 61-year-old woman and of 2-year duration in a 63-year-old woman-both associated with increased levels of antivaricella zoster virus (VZV) IgM antibodies in serum and with pain that improved with antiviral treatment. Combined with our previous finding of BMS due to herpes simplex virus type 1 (HSV-1) infection, we recommend evaluation of patients with BMS not only for VZV or HSV-1 DNA in the saliva, but also for serum anti-VZV and anti-HSV-1 IgM antibodies. Both infections are treatable with oral antiviral agents. PMID:27382016

  15. Cronkhite-Canada syndrome associated with myelodysplastic syndrome

    Institute of Scientific and Technical Information of China (English)

    Rei Suzuki; Atsushi Irisawa; Takuto Hikichi; Yuta Takahashi; Hiroko Kobayashi; Hiromi Kumakawa; Hiromasa Ohira

    2009-01-01

    We report a case of Cronkhite-Canada syndrome (CCS) associated with myelodysplastic syndrome (MDS). A 54-year-old woman, diagnosed as MDS the prior year after evaluation of anemia, visited our hospital with the chief complaint of epigastric discomfort. She also had dysgeusia, alopecia, atrophic nail change, and pigmentation of the palm, all of which began several months ago. Blood tests revealed severe hypoalbuminemia. Colonoscopy (CS) showed numerous, dense, red polyps throughout the colon and rectum. Biopsy specimens showed stromal edema, infiltration of lymphocytes, and cystic dilatation of the crypt. Her clinical manifestations and histology were consistent with CCS. We prescribed corticosteroids, which dramatically improved her physical findings, laboratory data, and endoscopic findings. This is the first report of CCS in a patient with MDS.

  16. Sick sinus syndrome associated with topical timolol maleate instillation

    Directory of Open Access Journals (Sweden)

    Harpreet S Walia

    2011-01-01

    Full Text Available A 70-year-old black woman presented with intermittent palpitations and dyspnea was found to be bradycardic with a normal sinus rhythm. She had instilled her topical timolol maleate approximately 30 minutes prior to each of these episodes. Topical timolol was discontinued and the conduction abnormality resolved. She was diagnosed as having intermittent sinus bradycardia with intermittent atrioventricular block, likely induced by topical beta-blocker therapy. Topical timolol maleate is an effective treatment for ocular hypertension, acting by reducing aqueous fluid production. However, it can induce systemic side effects and should be used with caution in patients with, or predisposed to, cardiac or respiratory depression.

  17. Small public private partnerships

    DEFF Research Database (Denmark)

    Koch, Christian; Jensen, Jesper Ole

    2009-01-01

    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...

  18. Small hydrogen liquefier

    International Nuclear Information System (INIS)

    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)

  19. 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…

  20. 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.

  1. Small Group Research

    Science.gov (United States)

    McGrath, Joseph E.

    1978-01-01

    Summarizes research on small group processes by giving a comprehensive account of the types of variables primarily studied in the laboratory. These include group structure, group composition, group size, and group relations. Considers effects of power, leadership, conformity to social norms, and role relationships. (Author/AV)

  2. Small can be beautiful

    Energy Technology Data Exchange (ETDEWEB)

    Albrand, Solveig (Institut des Sciences Nucleaires, IN2P3-CNRS/Universite Joseph Fourier, 53, avenue des Martyrs, 38026, Grenoble Cedex (France)); Ravel, J.C. (Institut des Sciences Nucleaires, IN2P3-CNRS/Universite Joseph Fourier, 53, avenue des Martyrs, 38026, Grenoble Cedex (France))

    1994-12-15

    Small laboratories with limited financial means can use low-cost personal computers for many control applications. Examples of control systems developed at SARA are discussed, illustrating the evolution of the system in step with that of the PC, but each time with a considerable re-use of software. ((orig.))

  3. Small is beautiful

    Energy Technology Data Exchange (ETDEWEB)

    Hindley, M.

    1994-09-01

    This article focusses on small hydroelectric power development in Finland. Legislative and financial incentives, and use of standard tubular turbines are discussed. The Vuotos reservoir project is described, and the preparation of an environmental assessment of the project is noted. (UK)

  4. Research using small tokamaks

    International Nuclear Information System (INIS)

    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 and Magical

    Institute of Scientific and Technical Information of China (English)

    2011-01-01

    Ant genome study offers promising evidence in medical and anti-aging research While children often love observing ants happily for hours, grownups are more likely to ignore these small creatures. Some scientists, however, show greater interest in ants than children do.

  6. Small is Beatiful

    DEFF Research Database (Denmark)

    Hansen, Henrik Aldershaab Boje; Pleman, Allan; Koch, Christian

    1999-01-01

    The life and death of a small Danish software house developing ERP-software in collaboration with costumers is analysed.User producer relationships are central drivers in the company's history. This reactive development strategy creates a niche at the marketplace but produces internal...

  7. 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...

  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. Libraries for Small Museums.

    Science.gov (United States)

    Anderson, Linda M.

    Presented are the very basic requirements for establishing a small special library operating under a limited budget. Physical plant organization, cataloging, book processing, circulation procedures, book selection and ordering and instructions for typists are covered. Although the practices discussed were established for a museum library, what is…

  10. Small hydroelectric power plants

    International Nuclear Information System (INIS)

    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

  11. 77 FR 30227 - Small Business Size Regulations, Small Business Innovation Research (SBIR) Program and Small...

    Science.gov (United States)

    2012-05-22

    ... BUSINESS ADMINISTRATION 13 CFR Part 121 Small Business Size Regulations, Small Business Innovation Research (SBIR) Program and Small Business Technology Transfer (STTR) Program AGENCY: U.S. Small Business... to amend its regulations governing size and eligibility for the Small Business Innovation...

  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 intestine bacterial overgrowth].

    Science.gov (United States)

    Leung Ki, E L; Roduit, J; Delarive, J; Guyot, J; Michetti, P; Dorta, G

    2010-01-27

    Small intestine bacterial overgrowth (SIBO) is a condition characterised by nutrient malabsorption and excessive bacteria in the small intestine. It typically presents with diarrhea, flatulence and a syndrome of malabsorption (steatorrhea, macrocytic anemia). However, it may be asymptomatic in the eldery. A high index of suspicion is necessary in order to differentiate SIBO from other similar presenting disorders such as coeliac disease, lactose intolerance or the irritable bowel syndrome. A search for predisposing factor is thus necessary. These factors may be anatomical (stenosis, blind loop), or functional (intestinal hypomotility, achlorydria). The hydrogen breath test is the most frequently used diagnostic test although it lacks standardisation. The treatment of SIBO consists of eliminating predisposing factors and broad-spectrum antibiotic therapy. PMID:20214190

  14. Small steps for hydro

    International Nuclear Information System (INIS)

    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)

  15. 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...... 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......The application of small wind turbines for residential and commercial applications depends on how a microgrid can operate in a suitable way. Because there is variable demand and a random nature of wind resources, it is usually necessary the installation of controllable end-use loads, storage...

  16. Advanced Small Rechargeable Batteries

    Science.gov (United States)

    Halpert, Gerald

    1989-01-01

    Lithium-based units offer highest performance. Paper reviews status of advanced, small rechargeable batteries. Covers aqueous systems including lead/lead dioxide, cadmium/nickel oxide, hydrogen/nickel oxide, and zinc/nickel oxide, as well as nonaqueous systems. All based on lithium anodes, nonaqueous systems include solid-cathode cells (lithium/molybdenum disulfide, lithium/titanium disulfide, and lithium/vanadium oxide); liquid-cathode cells (lithium/sulfur dioxide cells); and new category, lithium/polymer cells.

  17. Small reactor return

    International Nuclear Information System (INIS)

    Current state of the development of present-day small reactors in different countries is performed. Various designs of low and middle power reactors, among which are CAREM (25 MW, PWR), KLT-40 (40 MW, PWR), MRX (30 MW, PWR), IRIS (50 MW, PWR), SMART (1000 MW, PWR), Modular SBWR (50 MW, BWR), PBMR (120 MW, HTGR), GT-HMR (285 MW, HTGR), are discussed

  18. Small and Magical

    Institute of Scientific and Technical Information of China (English)

    TANG YUANKAI

    2011-01-01

    While children often love observing ants happily for hours,grown-ups are more likely to ignore these small creatures.Some scientists,however,show greater interest in ants than children do.One of them is Zhang Guojie,Associate Director of the Bioinformatics Center and Leader of Genome Projects in BGI.BG1 is China's largest genome and bioinformatics analysis center in Shenzhen,in south China's Guangdong Province.

  19. 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.

  20. 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.

  1. Leadership in Small Societies

    OpenAIRE

    Stephen Younger

    2010-01-01

    Multi-agent simulation was used to study several styles of leadership in small societies. Populations of 50 and100 agents inhabited a bounded landscape containing a fixed number of food sources. Agents moved about the landscape in search of food, mated, produced offspring, and died either of hunger or at a predetermined maximum age. Leadership models focused on the collection and redistribution of food. The simulations suggest that individual households were more effective at meeting their ne...

  2. Think Small Go Big

    Institute of Scientific and Technical Information of China (English)

    汤维维

    2006-01-01

    Vepoo公司在创立之前,经历了三次创业转型。用他们的话来说,从“think big go small”转到“think small go big”用了一年的时间。这期间他们耗尽了初期筹备资金,幸运的是在最后一刻迎来了黎明的曙光。

  3. Small Cell City

    OpenAIRE

    Dehghan, S.; Steele, R.

    1997-01-01

    Traditionally, mobile operators have planned their networks to accommodate mobile terminals at ground level. Increasingly, mobile users communicate while stationary from within high-rise buildings. With mobiles operating at a variety of different heights and mobilities, plus the requirement to accommodate increasing teletraffic and multimedia services, there is a need to compact small cells into the three-dimensional city space. This article is concerned with using city buildings to act as el...

  4. Small Animal Bone Biomechanics

    OpenAIRE

    Vashishth, Deepak

    2008-01-01

    Animal models, in particular mice, offer the possibility of naturally achieving or genetically engineering a skeletal phenotype associated with disease and conducting destructive fracture tests on bone to determine the resulting change in bone’s mechanical properties. Several recent developments, including nano- and micro- indentation testing, microtensile and microcompressive testing, and bending tests on notched whole bone specimens, offer the possibility to mechanically probe small animal ...

  5. When small is beautiful. Boom time for small hydro?

    Energy Technology Data Exchange (ETDEWEB)

    Osborne, John

    2002-08-01

    Although small hydropower projects generally contribute a small proportion of most nations' electricity needs their importance often exceeds their size. The author, a freelance industrial journalist considers prospects for small hydro, how such schemes are benefiting communities throughout the world and why some countries are more successfully using small hydro than others. (Author)

  6. 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.

  7. The protein X4 of severe acute respiratory syndrome-associated coronavirus is expressed on both virus-infected cells and lung tissue of severe acute respiratory syndrome patients and inhibits growth of Balb/c 3T3 cell line

    Institute of Scientific and Technical Information of China (English)

    CHEN Ying-yu; GAN Qi-ni; ZHANG Xin; ZHENG Ying; LIU Shun-ai; WANG Xiao-ning; ZHONG Nan-shan; MA Da-long; SHUANG Bao; TAN Ya-xia; MENG Min-jie; HAN Pu; MO Xiao-ning; SONG Quan-sheng; QIU Xiao-yan; LUO Xin

    2005-01-01

    Background The genome of the severe acute respiratory syndrome-associated coronavirus (SARS-CoV) includes sequences encoding the putative protein X4 (ORF8, ORF7a), consisting of 122 amino acids. The deduced sequence contains a probable cleaved signal peptide sequence and a C-terminal transmembrane helix, indicating that protein X4 is likely to be a type I membrane protein. This study was conducted to demonstrate whether the protein X4 was expressed and its essential function in the process of SARS-CoV infection.Methods The prokaryotic and eukaryotic protein X4-expressing plasmids were constructed. Recombinant soluble protein X4 was purified from E. Coli using ion exchange chromatography, and the preparation was injected into chicken for rising specific polyclonal antibodies. The expression of protein X4 in SARS-CoV-infected Vero E6 cells and lung tissues from patients with SARS was performed using immunofluorescence assay and immunohistochemistry technique. The preliminary function of protein X4 was evaluated by treatment with and over-expression of protein X4 in cell lines. Western blot was employed to evaluate the expression of protein X4 in SARS-CoV particles. Results We expressed and purified soluble recombinant protein X4 from E.coli, and generated specific antibodies against protein X4. Western blot proved that the protein X4 was not assembled in the SARS-CoV particles. Indirect immunofluorescence assays revealed that the expression of protein X4 was detected at 8 hours after infection in SARS-CoV-infected Vero E6 cells. It was also detected in the lung tissues from patients with SARS. Treatment with and overexpression of protein X4 inhibited the growth of Balb/c 3T3 cells as determined by cell counting and MTT assays. Conclusion The results provide the evidence of protein X4 expression following SARS-CoV infection, and may facilitate further investigation of the immunopathological mechanism of SARS.

  8. Spacecraft at Small NEO

    CERN Document Server

    Scheeres, D

    2006-01-01

    The impact risk has been sharply curtailed for the largest km-sized Near Earth Objects (NEOs) through a concerted period of observation spanning the last decade. Thus the risk of impact has now migrated to the smaller and more numerous members of the Near Earth Object (NEO) population. Characterization and mitigation missions to these smaller objects become more complex from a spacecraft operations perspective, even as the prospects for nudging such lower mass bodies off course become more feasible. This paper details the challenges for spacecraft operations at small bodies and indicates areas where more research and analysis of this problem is needed.

  9. Rapid small lot manufacturing

    Energy Technology Data Exchange (ETDEWEB)

    Harrigan, R.W.

    1998-05-09

    The direct connection of information, captured in forms such as CAD databases, to the factory floor is enabling a revolution in manufacturing. Rapid response to very dynamic market conditions is becoming the norm rather than the exception. In order to provide economical rapid fabrication of small numbers of variable products, one must design with manufacturing constraints in mind. In addition, flexible manufacturing systems must be programmed automatically to reduce the time for product change over in the factory and eliminate human errors. Sensor based machine control is needed to adapt idealized, model based machine programs to uncontrolled variables such as the condition of raw materials and fabrication tolerances.

  10. Rolling at small scales

    DEFF Research Database (Denmark)

    Nielsen, Kim L.; Niordson, Christian F.; Hutchinson, John W.

    2016-01-01

    The rolling process is widely used in the metal forming industry and has been so for many years. However, the process has attracted renewed interest as it recently has been adapted to very small scales where conventional plasticity theory cannot accurately predict the material response. It is well...... plasticity. Metals are known to be stronger when large strain gradients appear over a few microns; hence, the forces involved in the rolling process are expected to increase relatively at these smaller scales. In the present numerical analysis, a steady-state modeling technique that enables convergence...

  11. Small changes for works

    Institute of Scientific and Technical Information of China (English)

    Jiarui Wu

    2011-01-01

    The central dogma of molecular biology indicates that genetic information from DNA is transmitted to RNA,and then translated into proteins that play various roles during all physiological and pathological processes.However,most of the proteins have to make small chemical changes on their amino acid chains,so-called post-translational modifications,before they start to work.The collection of four articles in this issue presents timely developments in the area of protein modifications,ranging from how proteins carry out their biological functions based on protein modifications to what are molecular mechanisms of protein modifications.

  12. Business Small company

    OpenAIRE

    KREJČOVÁ, Kateřina

    2011-01-01

    This diploma work deals with a Business Small company. The work moves on the concrete company, which is analyzed and on the basis of analyses the new strategy is formulated. At the theoretical part, summary of solving problems is introduced. It’s needed for the analytic part of work. At the beginning, business is defined in economic terms. Found in this part are basic concepts that are linked to business. From the general definition of business the work fluently proceeds to more concrete...

  13. 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.

  14. 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.

  15. Small mirror fusion reactors

    International Nuclear Information System (INIS)

    Basic requirements for the pilot plants are that they produce a net product and that they have a potential for commercial upgrade. We have investigated a small standard mirror fusion-fission hybrid, a two-component tandem mirror hybrid, and two versions of a field-reversed mirror fusion reactor--one a steady state, single cell reactor with a neutral beam-sustained plasma, the other a moving ring field-reversed mirror where the plasma passes through a reaction chamber with no energy addition

  16. Securitization of Small Business Loans

    OpenAIRE

    Beshouri, Christopher P.; Peter J. Nigro

    1995-01-01

    This paper assesses the potential impact of securitization in improving small businesses’ access to credit. It begins by examining the nature of small business lending and the factors that make banks the primary providers of credit to small businesses. The paper then examines the conditions under which the benefits of securitization are fully realized and whether the nature of small business lend­ing satisfies those conditions. We argue that certain characteristics of small firm finance, espe...

  17. 77 FR 76215 - Small Business Size Regulations, Small Business Innovation Research (SBIR) Program and Small...

    Science.gov (United States)

    2012-12-27

    ... (available at http://www.gpo.gov/fdsys/pkg/FR-2012-05-15/pdf/2012-11586.pdf ), the U.S. Small Business... ADMINISTRATION 13 CFR Part 121 RIN 3245-AG46 Small Business Size Regulations, Small Business Innovation Research... regulations governing size and eligibility for the Small Business Innovation Research (SBIR) and...

  18. 78 FR 11745 - Small Business Size Regulations, Small Business Innovation Research (SBIR) Program and Small...

    Science.gov (United States)

    2013-02-20

    ... RIN 3245-AG46 Small Business Size Regulations, Small Business Innovation Research (SBIR) Program and... related to size and eligibility for the Small Business Innovation Research (SBIR) and Small Business... . SUPPLEMENTARY INFORMATION: On May 15, 2012, at 77 FR 28520 (available at...

  19. 77 FR 28520 - Small Business Size Regulations, Small Business Innovation Research (SBIR) Program and Small...

    Science.gov (United States)

    2012-05-15

    ... ADMINISTRATION 13 CFR Part 121 RIN 3245-AG46 Small Business Size Regulations, Small Business Innovation Research... its regulations governing size and eligibility for the Small Business Innovation Research (SBIR) and.... Background On July 22, 1982, Congress enacted and the President signed into law the Small Business...

  20. Small caliber guided projectile

    Science.gov (United States)

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

    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.

  1. 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.

  2. 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.

  3. Seminar on small PWRs

    International Nuclear Information System (INIS)

    The first of the three papers presented, reviews the history of small and medium power reactors. Although the category includes all reactors with an output up to 600MWe, the concentration is on the range 250-300MWe. Their suitability for developing countries and their economic aspects are discussed. The other two papers are concerned with the construction, transportation, installation and management of the Shore Test Facility for the prototype of the PWR 2, a reactor designed for use in submarines. The test facility was built at Barrow-in-Furness and then moved to its site in Caithness, Scotland. The methods used to move the enormous loads involved are described in detail. The construction and installation was planned over 5 years and involved a large number of people from the construction company, the Royal Navy and the contractors who operate the facility. The project management is described. (U.K.)

  4. Small-crack test methods

    Science.gov (United States)

    Larsen, James M.; Allison, John E.

    This book contains chapters on fracture mechanics parameters for small fatigue cracks, monitoring small-crack growth by the replication method, measurement of small cracks by photomicroscopy (experiments and analysis), and experimental mechanics of microcracks. Other topics discussed are the real-time measurement of small-crack-opening behavior using an interferometric strain/displacement gage; direct current electrical potential measurement of the growth of small cracks; an ultrasonic method for the measurement of the size and opening behavior of small fatigue cracks; and the simulation of short crack and other low closure loading conditions, utilizing constant K(max) Delta-K-decreasing fatigue crack growth procedures.

  5. Small Active Radiation Monitor

    Science.gov (United States)

    Badhwar, Gautam D.

    2004-01-01

    A device, named small active radiation monitor, allows on-orbit evaluations during periods of increased radiation, after extravehicular activities, or at predesignated times for crews on such long-duration space missions as on the International Space Station. It also permits direct evaluation of biological doses, a task now performed using a combination of measurements and potentially inaccurate simulations. Indeed the new monitor can measure a full array of radiation levels, from soft x-rays to hard galactic cosmic-ray particles. With refinement, it will benefit commercial (nuclear power-plant workers, airline pilots, medical technicians, physicians/dentists, and others) and military personnel as well as the astronauts for whom thermoluminescent dosimeters are inadequate. Civilian and military personnel have long since graduated from film badges to thermoluminescent dosimeters. Once used, most dosimeters must be returned to a central facility for processing, a step that can take days or even weeks. While this suffices for radiation workers for whom exposure levels are typically very low and of brief duration, it does not work for astronauts. Even in emergencies and using express mail, the results can often be delayed by as much as 24 hours. Electronic dosimeters, which are the size of electronic oral thermometers, and tattlers, small electronic dosimeters that sound an alarm when the dose/dose rate exceeds preset values, are also used but suffer disadvantages similar to those of thermoluminescent dosimeters. None of these devices fully answers the need of rapid monitoring during the space missions. Instead, radiation is monitored by passive detectors, which are read out after the missions. Unfortunately, these detectors measure only the absorbed dose and not the biologically relevant dose equivalent. The new monitor provides a real-time readout, a time history of radiation exposures (both absorbed dose and biologically relevant dose equivalent), and a count of the

  6. 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 ...

  7. Small intestine contrast injection (image)

    Science.gov (United States)

    ... and throat, through the stomach into the small intestine. When in place, contrast dye is introduced and ... means of demonstrating whether or not the small intestine is normal when abnormality is suspected.

  8. Brighter for small power plants

    International Nuclear Information System (INIS)

    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

  9. 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...

  10. Thinking small is big again

    Energy Technology Data Exchange (ETDEWEB)

    Butler, C.; Didsbury, R.; Strati, G.L.; Sur, B. [Atomic Energy of Canada Limited, Chalk River, Ontario (Canada)

    2012-12-15

    This article introduces a series of articles in the field of small reactors. Activity in this field has been on the upswing in North America. The major advantages of a small modular reactor are improved safety, lower cost and a small-risk venture. Canada has a long history of small reactor designs for research reactors as well as power reactors. Some of these reactors are ZEEP, ZED-2, NRX, NRU, WR-1, SLOWPOKE and CANDU power reactors.

  11. 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...

  12. Training in Chinese Small Firms

    OpenAIRE

    Dai, Zhejun

    2007-01-01

    The dissertation aims to review the currently predominant literature on the factors influencing the training in small business in the industrialized Western economies and to carry out empirical investigation on the determinants of training in Chinese small business and then analyze whether the Western-based literature is consistent with the practices of training in Chinese small firms. In addition, relevant problems with training in small business are presented and recommendations on how to r...

  13. Small Hydropower - The comeback of small hydropower stations

    International Nuclear Information System (INIS)

    This issue of the 'Erneuerbare Energien' (renewable energies) magazine published by the Swiss Solar Energy Society takes a look at small hydropower projects in Switzerland. In a number of interviews and articles, various topics concerning small hydropower are dealt with. First of all, an interview with Bruno Guggisberg, previously responsible for small hydro at the Swiss Federal Office of Energy, examines the potential of small hydro and the various political, technical and economic influences on such projects. Further articles provide an overview of the various types of small hydro schemes, including power generation using height differences in drinking-water and wastewater installations. As far as the components of small hydro schemes are concerned, various types of turbines and further system components that are needed are examined. A further article takes a look at the small hydro market and the market players involved. Ecological aspects and research activities are discussed in further articles. In a second interview with Martin Boelli, presently responsible for small hydropower at the Swiss Federal Office of Energy, the unused potential for the use of hydropower in Switzerland is discussed. Examples of small-scale hydro schemes are examined and the support offered by the Small Hydropower Program is discussed. Finally the question is asked, if the small hydro market in Switzerland is overheated as a result of promotion schemes such as cost-covering remuneration for electricity from renewable energy sources.

  14. Modified Small Business Network Security

    Directory of Open Access Journals (Sweden)

    Md. Belayet Ali

    2012-02-01

    Full Text Available This paper covers some likely threats and effectivesteps for a secure small business. It also involves a flowchart tocomprehend the overall small business network security easilyand we identify a set of security issues and applyappropriate techniques to satisfy the correspondingsecurity requirements. In respect of all, this document isstrong enough for any small business network security.

  15. Small intestine aspirate and culture

    Science.gov (United States)

    Small intestine aspirate and culture is a lab test to check for infection in the small intestine. ... A sample of fluid from the small intestine is needed. A procedure ... done to get the sample. The fluid is placed in a special dish in ...

  16. Research using small tokamaks

    International Nuclear Information System (INIS)

    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

  17. Small Hydropower in Nigeria

    Institute of Scientific and Technical Information of China (English)

    P.EOKRANEFE; S.OWOLABI

    2002-01-01

    Nigeria is the most populaous black nation in the world. It has an area of 913,072 square kilometres. The country population is about 120 million. There are three major languages, Hausa, Yoruba and Igbo, although there is still more than 380 dialects in local languages. There are large number of household on the African continent that are unelectrified.In Nigeria about 70 million people remain literally in the dark without access to electricity. The majority of these numbers are in the rural areas. This workshop is apt in a number of ways. It is a joint effort between government, private sector, the academic and other practitioners in small hydro power station, it is also a promotion of business and industrial activities as well as development of renewable energy resources. I wish to commend the organizers of this workshop. The focus of the workshop is relevant to the agenda of the Federal Ministry of Power and Steel and aspirations of the Federal Government of Nigeria to provide regular and steady electricity to majority of Nigerians before the end of year 2001.

  18. Exposition regarding small windmills

    International Nuclear Information System (INIS)

    Emphasis is laid on the elucidation of the current situation of small windmills in Denmark by estimating the industry's strengths and weaknesses and evaluating the technical status and user economy relevant to these wind turbines, in addition to their technical and marketing potentials within the next 3-5 years. It is also attempted to survey and forecast their situation within a competitive market which includes marketing and sales potential outside Denmark, often by way of development projects financed by DANIDA (Danish International Development Agency, a department under the Danish Foreign Ministry that takes responsibility for Danish aid to developing countries, often by direct agreement). It is noted that it is difficult to make predictions in the light of currently rapidly changing global political aspects, but it is suggested that sales of smaller Danish windmills within the country could amount to 300-700 wind turbines by the year 2000. Regarding sales abroad, it is concluded that the current number will not increase unless great efforts are made within the export market and the instigation of close cooperation with foreign companies which use an international sales network and have access to local distribution channels. (AB) (15 refs.)

  19. 48 CFR 970.1907 - Subcontracting with Small Business, Small Disadvantaged Business and Woman-Owned Small Business...

    Science.gov (United States)

    2010-10-01

    ... 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... Business, Small Disadvantaged Business and Woman-Owned Small Business Concerns. 970.1907 Section...

  20. Joint research using small tokamaks

    International Nuclear Information System (INIS)

    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 coordinated 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 Coordinated Research Project, is presented

  1. Joint research using small tokamaks

    International Nuclear Information System (INIS)

    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)

  2. 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.

  3. Small diameter carbon nanopipettes

    International Nuclear Information System (INIS)

    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 deg. C behaving like an amorphous semiconductor. Vacuum annealing of the CNP tips at temperatures up to 2000 deg. 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.

  4. 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.

  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 angle neutron scattering

    International Nuclear Information System (INIS)

    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 about 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 nano-metric 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 information 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 (2. 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 spectrometer

  7. Research using small tokamaks

    International Nuclear Information System (INIS)

    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

  8. Successful budgeting for small business

    OpenAIRE

    Cheng, Yafang

    2006-01-01

    The most important rule for financial management in small companies, although it is general rule for all sizes of company, is not to run out of cash. Being a financial manager for a small company, it is more serious to manage company's budget because the smaller enterprise faces greater challenge in raising cash. The following subjects are areas to be covered in addressing the issue of budgeting in a small business: -the current academic views and literature reviews on the subject o...

  9. Sustainability for a Small Country

    OpenAIRE

    Edwards, Geoff W.

    1992-01-01

    The main objective in this paper is to promote thinking on what "sustainable development" or "ecologically sustainable development" means for a small trading country. After a brief acknowledgement of some of the diverse literature on sustainability, there is a consideration of some realities facing a small trading economy. The balance of the paper comprises a discussion of the scope for a small country to enhance sustainability. This is done under several headings, corresponding to different ...

  10. Small Spacecraft Activities at JPL

    OpenAIRE

    Jones, Ross

    1991-01-01

    This paper presents a brief technical description of some of the small spacecraft concepts prepared by JPL for various sponsors. Some of JPL's work in microspacecraft is presented. The paper contains brief technical descriptions of the following four small spacecraft conceptual designs: 1) Lunar GAS, 2) Polar Mesoscale Explorer, 3) DARPA SHF and 4) Discovery. Since 1986, JPL has studied more than 10 small spacecraft including those to be presented here.

  11. Leadership in a small enterprise

    OpenAIRE

    Palmgren, Helena

    2010-01-01

    Leadership is often seen as the premier force behind the success and failure of enterprises and the well-being of the personnel. In spite of the vast amount of studies on leadership, leadership in small enterprises has seldom been in focus in leadership research. The increasing importance of small firms to the economic growth and competitiveness raises questions about the role and practice of leadership in small enterprises. This study aims to deepen our understanding of leadership as a socia...

  12. Organization of Small Business Accounting

    OpenAIRE

    Makarova, Oksana

    2012-01-01

    Along with continuing changes in the tax law, the requirements to the organization of small business accounting are changing, too. The article provides the analysis of normative legal documents governing small business accounting. The accounting requirements for small businesses of general and simplified tax accounts have been systematized. The necessity and essentiality of drawing primary documents has been grounded. The list and order of making tax registers and preparation of financial sta...

  13. 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.

  14. Deterministic Small-World Networks

    OpenAIRE

    Comellas, Francesc; Sampels, Michael

    2001-01-01

    Many real life networks, such as the World Wide Web, transportation systems, biological or social networks, achieve both a strong local clustering (nodes have many mutual neighbors) and a small diameter (maximum distance between any two nodes). These networks have been characterized as small-world networks and modeled by the addition of randomness to regular structures. We show that small-world networks can be constructed in a deterministic way. This exact approach permits a direct calculatio...

  15. 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…

  16. Small white matter lesion detection in cerebral small vessel disease

    Science.gov (United States)

    Ghafoorian, Mohsen; Karssemeijer, Nico; van Uden, Inge; de Leeuw, Frank E.; Heskes, Tom; Marchiori, Elena; Platel, Bram

    2015-03-01

    Cerebral small vessel disease (SVD) is a common finding on magnetic resonance images of elderly people. White matter lesions (WML) are important markers for not only the small vessel disease, but also neuro-degenerative diseases including multiple sclerosis, Alzheimer's disease and vascular dementia. Volumetric measurements such as the "total lesion load", have been studied and related to these diseases. With respect to SVD we conjecture that small lesions are important, as they have been observed to grow over time and they form the majority of lesions in number. To study these small lesions they need to be annotated, which is a complex and time-consuming task. Existing (semi) automatic methods have been aimed at volumetric measurements and large lesions, and are not suitable for the detection of small lesions. In this research we established a supervised voxel classification CAD system, optimized and trained to exclusively detect small WMLs. To achieve this, several preprocessing steps were taken, which included a robust standardization of subject intensities to reduce inter-subject intensity variability as much as possible. A number of features that were found to be well identifying small lesions were calculated including multimodal intensities, tissue probabilities, several features for accurate location description, a number of second order derivative features as well as multi-scale annular filter for blobness detection. Only small lesions were used to learn the target concept via Adaboost using random forests as its basic classifiers. Finally the results were evaluated using Free-response receiver operating characteristic.

  17. Adenocarcinoma of the small bowel

    International Nuclear Information System (INIS)

    Adenocarcinoma of small bowel is generally a rather rare primary tumour of small bowel with a prevalence rate of 0.5 - 3.0 / 100.000 population, but the most frequent tumour of small intestine. It more often involves the duodenum and jejunum than the ileum. The aim of this paper is also to point out the value of small bowel follow through (SBFT) in the diagnosis of stenosing lesions. An 83 - year old male patient suffered from abdominal pain, malaise, vomiting, cachexia and diarrhoea for 3 months. The result of occult blood testing was negative. Haemoglobin level was normal. Proctoscopy, colonoscopy, upper gastrointestinal (GI) endoscopy, and ultrasonography (US) did not explain the patient's problems. Ileus of the small bowel was established with abdominal plain film. Small bowel follow through (SBFT) and computer tomography (CT) showed a stenosing tumour in the jejunum. Adenocarcinoma of the small bowel was established with histological examination after resection of the tumor. SBFT, with manual compression of all segments of the small bowel, can be a very accurate diagnostic investigation for evaluation of stenosing lesions in this part of the intestine. (author)

  18. What Is a Small Business?

    OpenAIRE

    Jerome S. Osteryoung; Newman, Derek

    1993-01-01

    This paper argues that a definition of small business is important for both research and governmental policy. A chronological history of definitions is discussed along with their limitations. A new definition of small business is advocated when a business has personal guarantees and has no public stock. This definition is measurable, meaningful, and congruent with the perceptions of the market system.

  19. Standard Deviation for Small Samples

    Science.gov (United States)

    Joarder, Anwar H.; Latif, Raja M.

    2006-01-01

    Neater representations for variance are given for small sample sizes, especially for 3 and 4. With these representations, variance can be calculated without a calculator if sample sizes are small and observations are integers, and an upper bound for the standard deviation is immediate. Accessible proofs of lower and upper bounds are presented for…

  20. 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 for school-community…

  1. Board Effectiveness in Small Firms

    OpenAIRE

    Söderqvist, Anette; WÀgar, 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 ...

  2. Cosmic Strings with Small Tension

    CERN Document Server

    Halyo, Edi

    2009-01-01

    We describe cosmic F--term strings with exponentially small tension which are D3 branes wrapped on deformed $A_3$ singularities. We show that brane instanton effects which can be calculated after a geometric transition give rise to an exponentially small volume for the node on which the D3 branes wrap leading to a string with small tension. We generalize our description to the case of non--Abelian cosmic strings and argue that these strings are stable against monopole--anti monopole pair creation.

  3. Small Probe Reentry System Project

    Data.gov (United States)

    National Aeronautics and Space Administration — Global Aerospace Corporation (GAC), and its research partner, Cal Poly San Luis Obispo (CPSLO), will develop an integrated Small Probe Reentry System (SPRS) for low...

  4. 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....

  5. 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...

  6. Small Sat Analysis Laboratory Project

    Data.gov (United States)

    National Aeronautics and Space Administration — Develop Small Satellite Analysis Laboratory (SatLab): A simulation-of-simulations framework to integrate component and engineering simulations into a single larger...

  7. Quantum Small-world Networks

    CERN Document Server

    Wei, Zong-Wen; Han, Xiao-Pu

    2011-01-01

    Quantum networks are critical to quantum communication and distributed quantum computing. Here we propose a small-world model of large-scale quantum repeater networks, where "small-world" is a fundamental concept rooted in complex networks, which describe a broad range of real systems. The core of the model is to relate the hierarchical fashion of measurements to coarse-graining process, when quantum repeater protocols are implemented. We demonstrate that quantum repeater networks with fractal structure can be enlarged with certain length scale in geographic space, while preserving topology by performing renormalization. Actually, renormalization here serves as an organizing principle determining the distribution of long-range entangled links over quantum networks, which gives rise to fractal to small-world transition. Furthermore, by iterative implementation of renormalization on the former coarse-grained network, we eventually obtain an onion-like, hierarchical quantum small-world network, where the distanc...

  8. 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.)

  9. Small for gestational age (SGA)

    Science.gov (United States)

    ... is called intrauterine growth restriction. The most common definition of small for gestational age (SGA) is a ... M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health ...

  10. Business Opportunities for Small Reactors

    International Nuclear Information System (INIS)

    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)

  11. 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...

  12. Very small ionization pressure gauge

    International Nuclear Information System (INIS)

    A very small ionization pressure gauge has been developed to operate in the pressure range 10-6 Torr to 100μ. A metal construction and external cooling fins result in a very rugged device with exceptionally small outgassing properties. The gauge also incorporates a replaceable filament-grid assembly on a single plug which allows the simple replacement of both the filament and the grid when needed

  13. Ecology in Small Aquatic Ecosystems

    DEFF Research Database (Denmark)

    Andersen, Mikkel René

    % supersaturated in oxygen while the bottom waters becomes anoxic. Dense charophyte stands influenced the hydrodynamics and created favorable conditions for the apical parts in the surface waters, while the basal parts withstood anoxia for up to 12 hours in the bottom waters. Nocturnal convective mixing oxygenated......) both in the small lakes and in the small ephemeral streams on the Great Alvar. Downstream respiration was decoupled from GPP as respiration rates were much higher due to agricultural impact....

  14. Small Bowel Review - Part I

    OpenAIRE

    Thomson, ABR; Wild, G.

    1997-01-01

    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...

  15. Small windpower systems are beautiful

    Energy Technology Data Exchange (ETDEWEB)

    Conway, A.

    1988-11-01

    Today's small wind turbine generators perform well and reliably, thanks to brushless alternators, composite blades and protection against high winds. Wind turbines of such dimensions would often be employed for battery chargers. Bigger, geared machines, with rotors of up to 17 m diameter, driving synchronous or induction generators, would be coupled with diesel sets for supplying small communities. Battery chargers, island and hybrid systems and possible sites are discussed.

  16. Unitarity at small Bjorken x

    OpenAIRE

    Weigert, Heribert

    2000-01-01

    This paper presents a solution to the nonlinear small x ``projectile side'' evolution equations as derived by Balitskii in 1996. The solution is based on functional Fokker-Planck methods. The fixed point at small x is explicitly calculated and all correlation functions in this limit are determined. They show clear saturation and unitarization properties. Scaling laws that hold during the saturation phase and throughout the whole course of the evolution are established. The corresponding Lange...

  17. 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.

  18. 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.

  19. 78 FR 48537 - Small Business Innovation Research and Small Business Technology Transfer Programs...

    Science.gov (United States)

    2013-08-08

    ... ADMINISTRATION Small Business Innovation Research and Small Business Technology Transfer Programs... Administration (SBA) is publishing the Small Business Innovation Research (SBIR) and Small Business Technology... Director, Office of Innovation, Small Business Administration, 409 Third Street SW., Washington, DC...

  20. 78 FR 59410 - Small Business Innovation Research and Small Business Technology Transfer Programs...

    Science.gov (United States)

    2013-09-26

    ... ADMINISTRATION Small Business Innovation Research and Small Business Technology Transfer Programs... period for the Small Business Innovation Research (SBIR) and Small Business Technology Transfer (STTR... Edsel Brown, Jr., Assistant Director, Office of Innovation, Small Business Administration, 409...

  1. Runoff processes and small watersheds

    International Nuclear Information System (INIS)

    Full text: Small watersheds are a fundamental landscape unit for quantifying inputs and outputs of water, sediment and nutrients. Small watersheds have been used historically for defining runoff processes and flood response to storm precipitation. Early conceptualizations of runoff production during the International Hydrological Decade in the 1960s focused on the importance and movement of event water as overland flow to the stream channel. Use of mass balance mixing models using stable isotope tracers in the 1970s and 1980s directly challenged early ideas of where water goes when it rains, residence time of catchment waters and flow paths of subsurface runoff towards the stream. These data showed that the majority of water in the stream during a precipitation event was water that existed in the watershed prior to the event. While credible physical mechanisms of old water mobilization have only been defined in the past decade, stable isotope tracer approaches are now mature enough to offer new potential for informing new model structures of how small watersheds work. Isotope tracer data in small watersheds and mass balance separations also represent new ways of validating and calibrating watershed models. This presentation will chronicle the use of isotope tracers in small watersheds and provide examples of how these data can be used in models of runoff processes and for providing valuable input for water resource management at larger basin scales. (author)

  2. Small satellites for tropical applications

    Science.gov (United States)

    Montpetit, Marie-Jose; Bonn, Ferdinand

    1993-11-01

    A number of mission studies were performed to assess the suitability of small satellite systems for tropical data acquisition. These studies took into account the specifics of the tropical user communities and were focused on remote sensing and resource management issues. The requirements and potential solutions for four application areas are discussed. For monitoring of forest and agricultural vegetation, a small synthetic aperture radar is considered with P, C, or X band imaging, possibly supplemented by a high resolution multispectral imager. The radar would have the capability to monitor below cloud cover which is often found in tropical regions. Optical, microwave, or spectrographic imaging would also be useful in small satellites for disaster monitoring (notably of floods), land management, and air pollution monitoring. A small satellite with data storage and forwarding capability is also envisioned to collect data from dependable, low-power, and low-cost ground sensors via a simple ultrahigh frequency uplink and download the data on a very high frequency downlink. All the small satellites would be launched in low inclination orbits to ensure a number of consecutive passes over the targeted tropical area.

  3. 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.

  4. 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…

  5. Studies in small field inflation

    International Nuclear Information System (INIS)

    We explore some issues in slow roll inflation in situations where field excursions are small compared to Mp. We argue that for small field inflation, minimizing fine tuning requires low energy supersymmetry and a tightly constrained structure. Hybrid inflation is almost an inevitable outcome. The resulting theory can be described in terms of a supersymmetric low energy effective action and inflation completely characterized in terms of a small number of parameters. Demanding slow roll inflation significantly constrains these parameters. In this context, the generic level of fine tuning can be described as a function of the number of light fields, there is an upper bound on the scale of inflation, and an (almost) universal prediction for the spectral index. Models of this type need not suffer from a cosmological moduli problem

  6. 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....../implications: The conceptualization of SBSR communication would be enhanced by in-depth, nuanced empirical research. Practical implications: This paper has policy implications for those seeking to understand CSR among SMEs using formal communication mechanisms as a metric and control. Our paper suggests this to be...... intrinsic motivations) are confronting owner-managers’ ethos. We explain theoretically how small business managers respond to the challenge when they are required to formalize and display for external surveillance that which would otherwise be informal and part of the non-public or private sphere....

  7. Studies in Small Field Inflation

    CERN Document Server

    Dine, Michael

    2011-01-01

    We explore some issues in slow roll inflation in situations where field excursions are small compared to $M_p$. We argue that for small field inflation, minimizing fine tuning requires low energy supersymmetry and a tightly constrained structure. Hybrid inflation is {\\it almost} an inevitable outcome. The resulting theory can be described in terms of a supersymmetric low energy effective action and inflation completely characterized in terms of a small number of parameters. Demanding slow roll inflation significantly constrains these parameters. In this context, the generic level of fine tuning can be described as a function of the number of light fields, there is an upper bound on the scale of inflation, and an (almost) universal prediction for the spectral index. Models of this type need not suffer from a cosmological moduli problem.

  8. Trust Propagation in Small Worlds

    DEFF Research Database (Denmark)

    Gray, Elizabeth; Seigneur, Jean-Marc; Chen, Yong; Jensen, Christian D.

    do not scale well. We aim to develop trust-based security mechanisms using small world concepts to optimise formation and propagation of trust amongst entities in these vast networks. In this regard, we surmise that in a very large mobile ad hoc network, trust, risk, and recommendations can be...... propagated through relatively short paths connecting entities. Our work describes the design of trust-formation and risk-assessment systems, as well as that of an entity recognition scheme, within the context of the small world network topology....

  9. Small Wind Turbine Technology Assessment

    International Nuclear Information System (INIS)

    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 m2) 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

  10. Orbit Mechanics about Small Asteroids

    Science.gov (United States)

    Scheeres, D. J.

    2007-01-01

    Space missions to small solar system bodies must deal with multiple perturbations acting on the spacecraft. These include strong perturbations from the gravity field and solar tide, but for small bodies the most important perturbations may arise from solar radiation pressure (SRP) acting on the spacecraft. Previous research has generally investigated the effect of the gravity field, solar tide, and SRP acting on a spacecraft trajectory about an asteroid in isolation and has not considered their joint effect. In this paper a more general theoretical discussion of the joint effects of these forces is given.

  11. Particles in small volume injections.

    Science.gov (United States)

    Taylor, S A; Spence, J

    1983-12-01

    The level of particulate contamination in small volume injections has been examined using the light blockage (HIAC) and electrical sensing zone (Coulter counter) techniques, the HIAC system being found to be the more suitable. Particle counts on the same batch of injection showed a large and variable difference between the HIAC and the Coulter counter results, especially below 5 micron. None of the injections examined complied with the British Pharmacopoeia limits for particulates in large volume parenterals, suggesting the unsuitability of the limits for small volume parenterals. PMID:6141237

  12. Small Business Administration Semiannual Regulatory Agenda

    Science.gov (United States)

    2010-04-26

    ... Part XVI Small Business Administration Semiannual Regulatory Agenda ] SMALL BUSINESS ADMINISTRATION (SBA) SMALL BUSINESS ADMINISTRATION 13 CFR Ch. I Semiannual Regulatory Agenda AGENCY: U.S. Small... General Counsel for Legislation and Appropriations, U.S. Small Business Administration, 409 Third...

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

    International Nuclear Information System (INIS)

    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

  14. Small Parts Assembler Work Sample.

    Science.gov (United States)

    Shawsheen Valley Regional Vocational-Technical High School, Billerica, MA.

    This manual contains a work sample intended to assess a handicapped student's interest in and potential to enter a training program in small parts assembly or in a similar job. Section 1 describes the assessment, correlates the work performed and worker traits required for completing the work sample, and lists related occupations and DOT codes.…

  15. Small Business. Lesson Plan Three.

    Science.gov (United States)

    Dicke, Thomas S.

    1996-01-01

    Presents a lesson plan that requires students to use their research and analytical skills in studying the social and economic importance of small business. Activities include interviewing local business owners and producing a written summary, developing a survey showing and explaining area land use, and researching opposition to Wal-Mart stores.…

  16. 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

  17. 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.

  18. 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.

  19. Small animal radiotherapy research platforms

    International Nuclear Information System (INIS)

    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. Small animal radiotherapy research platforms

    Science.gov (United States)

    Verhaegen, Frank; Granton, Patrick; Tryggestad, Erik

    2011-06-01

    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.

  1. Sonography of the small intestine

    Institute of Scientific and Technical Information of China (English)

    Kim Nylund; Svein (φ)degaard; Trygve Hausken; Geir Folvik; Gülen Arslan Lied; Ivan Viola; Helwig Hauser; Odd-Helge Gilja

    2009-01-01

    In the last two decades, there has been substantial development in the diagnostic possibilities for examining the small intestine. Compared with computerized tomography, magnetic resonance imaging, capsule endoscopy and double-balloon endoscopy, ultrasonography has the advantage of being cheap, portable, flexible and user- and patient-friendly, while at the same time providing the clinician with image data of high temporal and spatial resolution. The method has limitations with penetration in obesity and with intestinal air impairing image quality. The flexibility ultrasonography offers the examiner also implies that a systematic approach during scanning is needed. This paper reviews the basic scanning techniques and new modalities such as contrast-enhanced ultrasound, elastography, strain rate imaging, hydrosonography, allergosonography, endoscopic sonography and nutritional imaging, and the literature on disease-specific findings in the small intestine. Some of these methods have shown clinical benefit, while others are under research and development to establish their role in the diagnostic repertoire. However, along with improved overall image quality of new ultrasound scanners, these methods have enabled more anatomical and physiological changes in the small intestine to be observed. Accordingly, ultrasound of the small intestine is an attractive clinical tool to study patients with a range of diseases.

  2. Small Signal Loudspeaker Impedance Emulator

    DEFF Research Database (Denmark)

    Iversen, Niels Elkjær; Knott, Arnold

    2014-01-01

    from driver to driver. Therefore, a loudspeaker emulator capable of adjusting its impedance to that of a given driver is desired for measurement purposes. This paper proposes a loudspeaker emulator circuit for small signals. Simulations and experimental results are compared and show that it is possible...

  3. 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 tran

  4. Small Turing universal signal machines

    Directory of Open Access Journals (Sweden)

    Jérôme Durand-Lose

    2009-06-01

    Full Text Available This article aims at providing signal machines as small as possible able to perform any computation (in the classical understanding. After presenting signal machines, it is shown how to get universal ones from Turing machines, cellular-automata and cyclic tag systems. Finally a halting universal signal machine with 13 meta-signals and 21 collision rules is presented.

  5. Microwave Levitation Of Small Objects

    Science.gov (United States)

    Watkins, John L.; Jackson, Henry W.

    1991-01-01

    Microwave radiation in resonant cavities used to levitate small objects, according to proposal. Feedback control and atmosphere not needed. Technique conceived for use in experiments on processing of materials in low gravitation of outer space, also used in normal Earth gravitation, albeit under some limitations.

  6. Small bowel transplantation: An overview

    NARCIS (Netherlands)

    R.W.F. de Bruin (Ron); E. Heineman (Erik); R.L. Marquet (Richard)

    1994-01-01

    textabstractSmall bowel transplantation (SBT) would, in theory, be the treatment of choice for patients suffering from the short bowel syndrome. Although SBT has been done with a considerable degree of success in some centers [36,145], it is by no means an established or widely applicable therapy fo

  7. Proceedings of Small Hydro 2009

    International Nuclear Information System (INIS)

    This conference was attended by speakers from government, industry and client organizations who addressed the growing interest in small hydro. Small hydro has been steadily increasing for years and will likely continue to increase as the supply of fossil fuels dwindles. The conference provided an opportunity to assess this renewable resources in terms of new estimates of micro (less than 100 kW), mini (100 kW to 1 MW) and small hydro (1 MW to 50 MW) facilities. Some presentations identified new locations for potential hydroelectric facilities and the development of projects at existing structures. New development mechanisms were reviewed, such as government funding initiatives, prospects for private funding, risk assessment and allocation. Local issues regarding small hydro development include quick routes to licensing and assessing and minimizing environmental impacts. The conference also provided an opportunity to highlight advances in technology to increase efficiency; refurbish and uprate existing, aging hydropower plants; and software developments. The conference featured 25 presentations, of which 10 have been catalogued separately for inclusion in this database. refs., tabs., figs

  8. Small rocket research and technology

    Science.gov (United States)

    Schneider, Steven; Biaglow, James

    1993-01-01

    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

  9. Small is beautiful: models of small neuronal networks

    Science.gov (United States)

    Lamb, Damon G; Calabrese, Ronald L

    2013-01-01

    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. PMID:22364687

  10. 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.

  11. General Information about Small Cell Lung Cancer

    Science.gov (United States)

    ... Cancer Prevention Lung Cancer Screening Research Small Cell Lung Cancer Treatment (PDQ®)–Patient Version General Information About Small Cell Lung Cancer Go to Health Professional Version Key Points Small ...

  12. Stages of Small Cell Lung Cancer

    Science.gov (United States)

    ... Cancer Prevention Lung Cancer Screening Research Small Cell Lung Cancer Treatment (PDQ®)–Patient Version General Information About Small Cell Lung Cancer Go to Health Professional Version Key Points Small ...

  13. Treatment Option Overview (Small Cell Lung Cancer)

    Science.gov (United States)

    ... Cancer Prevention Lung Cancer Screening Research Small Cell Lung Cancer Treatment (PDQ®)–Patient Version General Information About Small Cell Lung Cancer Go to Health Professional Version Key Points Small ...

  14. Propulsion Challenges for Small Spacecraft: 2005

    Institute of Scientific and Technical Information of China (English)

    Vadim Zakirov; LI Luming

    2006-01-01

    Small (<100 kg) spacecrafts are being developed in many countries but their propulsion systems still have many challenges. Although there is demand for small spacecraft propulsion, the number of missions at present is small due to several commercial and technical reasons. Poor performance of existing small spacecraft propulsion systems is one of the main reasons for the small number of missions. Several reasons are given for the poor performance of existing small spacecraft propulsion. Suggested improvements focus on small spacecraft and propulsion hardware mass optimization rather than on specific impulse enhancement. Propellantless propulsion systems are also recommended for small spacecraft interplanetary missions.

  15. Going global - growing small businesses

    International Nuclear Information System (INIS)

    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

  16. Trust Propagation in Small Worlds

    DEFF Research Database (Denmark)

    Gray, Elizabeth; Seigneur, Jean-Marc; Chen, Yong;

    2003-01-01

    do not scale well. We aim to develop trust-based security mechanisms using small world concepts to optimise formation and propagation of trust amongst entities in these vast networks. In this regard, we surmise that in a very large mobile ad hoc network, trust, risk, and recommendations can be...... propagated through relatively short paths connecting entities. Our work describes the design of trust-formation and risk-assessment systems, as well as that of an entity recognition scheme, within the context of the small world network topology.......The possibility of a massive, networked infrastructure of diverse entities partaking in collaborative applications with each other increases more and more with the proliferation of mobile devices and the development of ad hoc networking technologies. In this context, traditional security measures...

  17. Remotely sensed small reservoir monitoring

    Science.gov (United States)

    Eilander, Dirk; Annor, Frank; Iannini, Lorenzo; van de Giesen, Nick

    2013-04-01

    A new 'growing' maximum likelihood classification algorithm for small reservoir delineation has been developed and is tested with Radarsat-2 data for reservoirs in the semi-arid Upper East Region, Ghana. The delineation algorithm is able to find the land-water boundary from SAR imagery for different weather and environmental conditions. As such, the algorithm allows for remote sensed operational monitoring of small reservoirs. Multipurpose small reservoirs (1-100 ha) are important for many livelihoods in rural semi-arid West Africa. In order to manage and plan these reservoirs and to assess their hydrological impact at a river basin scale, it is important to monitor their water storage fluctuation. Several studies on remotely sensed reservoir mapping have recently been published, but no single method yields good results for all weather and environmental conditions. Detection of small reservoirs from optical satellite imagery using supervised maximum likelihood classification is a well proved method. The application of this method for the monitoring of small reservoirs is however limited because of its dependence on cloud-free day-acquisitions. Delineation from SAR images is promising, but because of difficulties with wind induced Bragg-scattering and low contrast between the water surface and the dried-out surroundings at the end of the dry season, only quasi manual methods have been applied successfully. A smart combination of optical satellite based detection combined with a delineation method for SAR imagery is proposed. From the optical satellite based small reservoir detection the reservoir window is determined in which the 'growing' maximum likelihood classification on SAR images is performed. A water-class seed and land-class seed are implemented and grown dependent on the likelihood of a pixel to belong to one class. The likelihood is calculated based on the probability distributions of the growing land and water populations. Combinations of single

  18. Unveiling small sphere's scattering behavior

    CERN Document Server

    Tzarouchis, Dimitrios C; Sihvola, Ari

    2016-01-01

    A classical way for exploring the scattering behavior of a small sphere is to approximate Mie coefficients with a Taylor series expansion. This ansatz delivered a plethora of insightful results, mostly for small spheres supporting electric localized plasmonic resonances. However, many scattering aspects are still uncharted, especially for the case of magnetic resonances. Here, an alternative system ansatz is proposed based on the Pad\\'e approximants for the Mie coefficients. The extracted results reveal new aspects, such as the existence of a self-regulating radiative damping mechanism for the first magnetic resonance. Hence, a systematic way of exploring the scattering behavior is introduced, sharpening our understanding about sphere's scattering behavior and its emergent functionalities.

  19. Knowledge Management in Small Firms

    Science.gov (United States)

    Panyasorn, Jessada; Panteli, Niki; Powell, Philip

    This paper explores knowledge management in small and medium-sized firms (SMEs). It investigates the use of Lotus Notes in SMEs of a developing country as a counterpoint to the large firm, developed country emphasis of existing research. It develops taxonomy of Lotus Notes use within the context of different knowledge management processes; notably communicating, co-ordinating and collaborating. The study employs an interpretive approach using three case studies. The key findings suggest that publishing, searching, sharing and retrieving are the user modes for enabling sharing and storing information. Evidence of knowledge creation is found at the departmental level but not at the organizational level. Further, small firms may explore more groupware potential than large organizations and this reflects their different context. Finally, implications for further research are identified.

  20. [Sarcomas of the small intestine].

    Science.gov (United States)

    Beyrouti, M L; Abid, M; Beyrouti, R; Ben Amar, M; Gargouri, F; Frikha, F; Affes, N; Boujelbene, S; Ghorbel, A

    2005-03-12

    Sarcomas of the small intestine are rare, clearly differentiated, malignant, mesenchymatous tumours that can be of smooth muscle, Schwann cell or fibroblastic origin. From a clinical point of view, the pain and abdominal mass are the 2 types of symptoms that frequently reveal the disease. In rare cases, sarcomas of the small intestine are manifested by an acute complication. No imaging method can clearly confirm the diagnosis. Before immunohistochemistry, differential diagnosis was made on undifferentiated mesenchymatous "stromal" tumours, which are also rare. Exeresis must be complete and without perforation of the tumour because of the risk of locoregional relapse. The benefits provided by chemotherapy and radiotherapy are limited because of the low mitotic activity of the tumour cells and its weak vascularisation. Long-term survival is limited by poor prognosis criteria: high grade malignancy, size greater than 5 cm, tumour extension, perforation of the tumour, quality of surgical resection and histological type. PMID:15859576

  1. Parton distributions at small x

    International Nuclear Information System (INIS)

    We perform a next-to-leading order quantum chromodynamics (QCD) analysis of the recent data for deep-inelastic lepton-nucleon scattering and related processes, in which we pay particular attention to the forms of the parton distributions at very small x. We discuss in detail, and we incorporate in the analysis, the theoretical QCD results leading to the singular x-1/2 type of behaviour of the gluon and sea quark distributions, as well as the modifications due to shadowing effects. We find the QCD shadowing corrections are significant for x ''approx -3 even though the parton distributions are below their saturation limit. We give predictions for the structure functions F2 and FL accessible at HERA, and for W and Z productions up to linear hard core pinch devices (LHC) and superconducting supercollider (SSC) energies. We discuss the possibility of experiments at these colliders probing the parton distributions in the very small x region. (author)

  2. Unitarity at small Bjorken x

    CERN Document Server

    Weigert, H

    2002-01-01

    This paper presents a solution to the nonlinear small x ``projectile side'' evolution equations as derived by Balitskii in 1996. The solution is based on functional Fokker-Planck methods. The fixed point at small x is explicitly calculated and all correlation functions in this limit are determined. They show clear saturation and unitarization properties. Scaling laws that hold during the saturation phase and throughout the whole course of the evolution are established. The corresponding Langevin equations are given as a basis for numerical simulations opening the field for future studies of dynamical issues of the evolution not analytically accessible. The methods used may be extended to the ``target side'' equations of Jalilian-Marian, Kovner, Leonidov and Weigert.

  3. Unitarity at small Bjorken x

    International Nuclear Information System (INIS)

    This paper presents a solution to the nonlinear small-x 'projectile side' evolution equations as derived by Balitskii in 1996. The solution is based on functional Fokker-Planck methods. The fixed point at small x is explicitly calculated and all correlation functions in this limit are determined. They show clear saturation and unitarization properties. Scaling laws that hold during the saturation phase and throughout the whole course of the evolution are established. The corresponding Langevin equations are given as a basis for numerical simulations opening the field for future studies of dynamical issues of the evolution not analytically accessible. The methods used may be extended to the 'target side' equations of Jalilian-Marian, Kovner, Leonidov and Weigert

  4. Unitarity at small Bjorken x

    Energy Technology Data Exchange (ETDEWEB)

    Weigert, Heribert E-mail: heribert.weigert@physik.uni-regensburg.de

    2002-06-03

    This paper presents a solution to the nonlinear small-x 'projectile side' evolution equations as derived by Balitskii in 1996. The solution is based on functional Fokker-Planck methods. The fixed point at small x is explicitly calculated and all correlation functions in this limit are determined. They show clear saturation and unitarization properties. Scaling laws that hold during the saturation phase and throughout the whole course of the evolution are established. The corresponding Langevin equations are given as a basis for numerical simulations opening the field for future studies of dynamical issues of the evolution not analytically accessible. The methods used may be extended to the 'target side' equations of Jalilian-Marian, Kovner, Leonidov and Weigert.

  5. Small black holes on cylinders

    International Nuclear Information System (INIS)

    We find the metric of small black holes on cylinders, i.e. neutral and static black holes with a small mass in d-dimensional Minkowski space times a circle. The metric is found using an ansatz for black holes on cylinders proposed in J. High Energy Phys. 05, 032 (2002). We use the new metric to compute corrections to the thermodynamics which is seen to deviate from that of the (d+1)-dimensional Schwarzschild black hole. Moreover, we compute the leading correction to the relative binding energy which is found to be non-zero. We discuss the consequences of these results for the general understanding of black holes and we connect the results to the phase structure of black holes and strings on cylinders

  6. Neutronic design of small reactors

    International Nuclear Information System (INIS)

    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)

  7. Doing Astronomy with Small Telescopes

    OpenAIRE

    Kangujam Yugindro Singh; Irom Ablu Meitei; Salam Ajitkumar Singh

    2014-01-01

    We are playing a lead role for growth of astronomy and its quality teaching and research in Manipur, a State located at northeast India (longitude = 93°58'E; latitude = 24°44'N; altitude = 782 m). We have innovatively designed and constructed three cost effective observatories, each costing a few hundred USD. These observatories are completely different in design and are perfectly usable for doing serious work on astronomical observation and measurements, using small ground-...

  8. Metagenomic small molecule discovery methods

    OpenAIRE

    Charlop-Powers, Zachary; Milshteyn, Aleksandr; Brady, Sean F

    2014-01-01

    Metagenomic approaches to natural product discovery provide the means of harvesting bioactive small molecules synthesized by environmental bacteria without the requirement of first culturing these organisms. Advances in sequencing technologies and general metagenomic methods are beginning to provide the tools necessary to unlock the unexplored biosynthetic potential encoded by the genomes of uncultured environmental bacteria. Here, we highlight recent advances in sequence- and functional- bas...

  9. Nucleation barrier in small volumes

    Czech Academy of Sciences Publication Activity Database

    Kožíšek, Zdeněk; Demo, Pavel; Sveshnikov, Alexey; Tichá, Petra

    Bratislava: N, 2009 - (Koman, M.; Mikloš, D.), s. 36-37 ISBN 978-80-89088-81-2. [Development of Materials Science in Research and Education - DMS -RE 2009 /19./. Závažná Poruba (SK), 31.08.2009-04.09.2009] R&D Projects: GA AV ČR IAA100100806 Institutional research plan: CEZ:AV0Z10100521 Keywords : nucleation * phase transition in small volumes Subject RIV: BM - Solid Matter Physics ; Magnetism

  10. Design of small table saws

    OpenAIRE

    Stuchlík, Petr

    2016-01-01

    The main aim of this thesis is the engineering design of a small table circular saw according to the specified parameters. At first, the types of machines available on the market are named and analyzed in detail. Afterwards, the engineering design of the machine is created. The following part is concerned with the individual parts,, tilting of the saw blade, maximum cutting pull through linkage with motion screw and checking of deformation of he critical parts of the machine. The previously s...

  11. Small gauge vitrectomy: Recent update

    OpenAIRE

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

    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 vitre...

  12. Small intestinal bacterial overgrowth syndrome

    OpenAIRE

    Jan Bures, Jiri Cyrany, Darina Kohoutova, Miroslav Förstl, Stanislav Rejchrt, Jaroslav Kvetina, Viktor Vorisek, Marcela Kopacova

    2010-01-01

    Human intestinal microbiota create a complex polymicrobial ecology. This is characterised by its high population density, wide diversity and complexity of interaction. Any dysbalance of this complex intestinal microbiome, both qualitative and quantitative, might have serious health consequence for a macro-organism, including small intestinal bacterial overgrowth syndrome (SIBO). SIBO is defined as an increase in the number and/or alteration in the type of bacteria in the upper gastrointestina...

  13. Small intestinal bacterial overgrowth syndrome.

    OpenAIRE

    Bures, J.; Cyrany, J.; Kohoutova, D.; Förstl, M.; Rejchrt, S.; Kvetina, J.; Vorisek, V.; Kopacova, M.

    2010-01-01

    Human intestinal microbiota create a complex polymicrobial ecology. This is characterised by its high population density, wide diversity and complexity of interaction. Any dysbalance of this complex intestinal microbiome, both qualitative and quantitative, might have serious health consequence for a macro-organism, including small intestinal bacterial overgrowth syndrome (SIBO). SIBO is defined as an increase in the number and/or alteration in the type of bacteria in the upper gastrointestina...

  14. The small world of psychopathology

    OpenAIRE

    Denny Borsboom; Cramer, Angélique O. J.; Schmittmann, Verena D.; Sacha Epskamp; Waldorp, Lourens J.

    2011-01-01

    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, fe...

  15. The small world of psychopathology

    OpenAIRE

    Borsboom, D; Cramer, A.O.J.; Schmittmann, V.D.; Epskamp, S.; Waldorp, L.J.

    2011-01-01

    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, feat...

  16. Rhapsody on small processor platforms

    OpenAIRE

    Andersson, Per-Oskar

    2008-01-01

    Rhapsody is a Model-Driven Development (MDD) tool for embedded and real-time system design. The purpose of this thesis is to determine if Rhapsody can be used for software development on small processor platforms such as the Atmel AVR. Rhapsody is normally used on platforms running an operating system. Therefore certain adaptations are needed in order to use it on platforms without an operating system. These adaptations and their affect on the usability of the tool, advantages and disadvantag...

  17. Twisted Yangians of small rank

    Science.gov (United States)

    Guay, Nicolas; Regelskis, Vidas; Wendlandt, Curtis

    2016-04-01

    We study quantized enveloping algebras called twisted Yangians associated with the symmetric pairs of types CI, BDI, and DIII (in Cartan's classification) when the rank is small. We establish isomorphisms between these twisted Yangians and the well known Olshanskii's twisted Yangians of types AI and AII, and also with the Molev-Ragoucy reflection algebras associated with symmetric pairs of type AIII. We also construct isomorphisms with twisted Yangians in Drinfeld's original presentation.

  18. Innovation in small food firms

    OpenAIRE

    Avermaete, Tessa

    2012-01-01

    Internationalisation of the world food market, structural changes in the food chain, changing consumption patterns and the strengthening of food regulation lie on the basis of the fast developments in the food and drink industry. Facing increased competition, small food firms are generally limited in exploiting economies of scale. Alternatively, they are more likely to seek for strategies that are based on the introduction of new products, optimising processing, the reorganisation of manageme...

  19. 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.

  20. 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.

  1. 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.

  2. Vibration Testing for Small Satellites

    OpenAIRE

    Wilson, Delbert

    1989-01-01

    Most people involved in the design and construction of small satellites are unfamiliar with vibration testing. Yet most satellites undergo vibration testing to qualify them for flight. Some familiarity with the basic aspects of vibration testing is needed to insure that a vibration test on a satellite is valid. This paper sets forth the basic equipment, practices and concepts of vibration testing. It provides guidelines for specifying a vibration test, designing fixtures, attaching instrument...

  3. Implantable telemetry for small animals

    Science.gov (United States)

    1982-03-01

    A series of totally implantable telemetry devices for use in measuring deep body parameters in small animals were developed. Under a collaborative agreement with NASA, several of these systems; the continuous wave Doppler ultrasonic flowmeter, the multichannel telemetry system, and the inductively-powered dual channel cardiac pacer were evaluated in a series of ten mongrel dogs (15 to 20 kg.). These systems were used to measure ascending aortic and coronary blood flow, aortic pressure, and subcutaneous EKG.

  4. The evolution of small change

    OpenAIRE

    Thomas J. Sargent; Francois R. Velde

    1997-01-01

    Western Europe was plagued with currency shortages from the 14th to the 19th century, at which time a `standard formula' had been devised to cure the problem. We document the evolution of mon- etary theory, policy experiments and minting tech- nology over the course of six hundred years. In a companion paper, we use a cash-in-advance model of commodity money to provide an analytical frame- work for the problem of small change.

  5. Small intestinal bacterial overgrowth syndrome

    Institute of Scientific and Technical Information of China (English)

    Jan; Bures; Jiri; Cyrany; Darina; Kohoutova; Miroslav; Frstl; Stanislav; Rejchrt; Jaroslav; Kvetina; Viktor; Vorisek; Marcela; Kopacova

    2010-01-01

    Human intestinal microbiota create a complex polymi-crobial ecology. This is characterised by its high population density, wide diversity and complexity of interaction. Any dysbalance of this complex intestinal microbiome, both qualitative and quantitative, might have serious health consequence for a macro-organism, including small intestinal bacterial overgrowth syndrome (SIBO).SIBO is defined as an increase in the number and/or alteration in the type of bacteria in the upper gastro-intestinal tract. There...

  6. 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.

  7. Superconductivity of small metal grains

    Institute of Scientific and Technical Information of China (English)

    ZHENG; Renrong; CHEN; Zhiqian; ZHU; Shunquan

    2005-01-01

    The formulas of the energy gap and superconducting critical temperature appropriate for systems with both odd and even number of electrons are derived; the bases of the derivations are BCS theory and energy level statistics. Numerical results qualitatively agree with the experimental phenomena. i.e., the superconductivity of small metallic grains will first enhance then decrease to zero when the grain are getting smaller and smaller. The calculations indicate that the above phenomena happen in the metallic grains belonging to Gaussian Orthogonal Ensemble (GOE) and Gaussian Unitary ensemble (GUE) with zero spin; The superconductivity of small metallic grains in Gaussian Symplectic Ensemble (GSE) will monotonically decrease to zero with the decreasing of the grain size. The analyses suggest that the superconductivity enhancements come from pairing and the balance of the strengths between spin-orbital coupling and external magnetic field. In order to take the latter into account, it is necessary to include the level statistics given by Random Matrix Theory (RMT) in describing small metallic grains.

  8. Small Break Air Ingress Experiment

    Energy Technology Data Exchange (ETDEWEB)

    Chang Oh; Eung Soo Kim

    2011-09-01

    The small break air-ingress experiment, described in this report, is designed to investigate air-ingress phenomena postulated to occur in pipes in a very high temperature gas-cooled reactor (VHTRs). During this experiment, air-ingress rates were measured for various flow and break conditions through small holes drilled into a pipe of the experimental apparatus. The holes were drilled at right angles to the pipe wall such that a direction vector drawn from the pipe centerline to the center of each hole was at right angles with respect to the pipe centerline. Thus the orientation of each hole was obtained by measuring the included angle between the direction vector of each hole with respect to a reference line anchored on the pipe centerline and pointing in the direction of the gravitational force. Using this reference system, the influence of several important parameters on the air ingress flow rate were measured including break orientation, break size, and flow velocity . The approach used to study the influence of these parameters on air ingress is based on measuring the changes in oxygen concentrations at various locations in the helium flow circulation system as a function of time using oxygen sensors (or detectors) to estimate the air-ingress rates through the holes. The test-section is constructed of a stainless steel pipe which had small holes drilled at the desired locations.

  9. Chronic Hemodialysis in Small Children.

    Science.gov (United States)

    Novljan, Gregor; Rus, Rina R; Premru, Vladimir; Ponikvar, Rafael; Battelino, Nina

    2016-06-01

    When peritoneal dialysis is inapplicable, chronic hemodialysis (HD) becomes the only available treatment option in small children. Due to small patient size, central venous catheters (CVC) are mainly used for vascular access. Over the past 4 years, four children weighing less than 15 kg received chronic HD in our unit. A total of 848 dialysis sessions were performed. Altogether, 21 catheters were inserted. In all but one occasion, uncuffed catheters were used. Catheter revision was performed 15 times during the study period, either due to infection or catheter malfunction. The median number of catheter revisions and the median line survival was 3.0/patient-year and 53 days (range; 6-373 days), respectively. There were 14 episodes of catheter related infections requiring 11 CVC revisions (78.6%). The median rate of line infections was 2.8/patient-year. Chronic HD in small children is demanding and labor intensive. Issues pertain mainly to CVCs and limit its long-term use. PMID:27312919

  10. 76 FR 61626 - Small Business Subcontracting

    Science.gov (United States)

    2011-10-05

    ... ADMINISTRATION 13 CFR Parts 121 and 125 RIN 3245-AG22 Small Business Subcontracting AGENCY: U.S. Small Business Administration. ACTION: Proposed rule. SUMMARY: The U.S. Small Business Administration (SBA or Agency) is... submissions: Dean Koppel, U.S. Small Business Administration, Office of Government Contracting, 409...

  11. Small satellite solar array substrate

    Science.gov (United States)

    Fiore, John N.; Rosanova, Giulio

    1994-01-01

    The SMall EXplorer (SMEX) Fast Auroral SnapshoT (FAST) spacecraft was developed to investigate plasma physics of auroral phenomena at high orbital altitude. The FAST satellite comprises a variety of deployable booms with sensors on the ends, and instruments that protrude from the main body of the spacecraft to obtain the plasma and electromagnetic fields data. This required the plasma disturbance around the satellite to be kept to a minimum. A non deployable, body mounted solar array was implemented. This led to the design of a light weight solar array substrate with a high degree of structural integrity.

  12. Emissions from small heating plants

    International Nuclear Information System (INIS)

    This study treats emissions from and economy of small biofuelled heating plants. In order to reduce emissions of particulates below 100 mg/nm3, the plants must be equipped with flue gas cleaning systems superior to cyclones/multicyclones. The use of pellets or pulverized fuel does not necessarily give emissions lower than 100 mg/nm3. However, the study shows that the CO-emissions can be kept low. An economic analysis for biofuelled plants is also presented 18 tabs, 18 figs

  13. GEF small grants programme - overview

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1997-12-01

    This paper describes the GEF small grants program which seeks to enhance the role of households and communities in conserving global biodiversity, mitigating global climate change, and protecting international waters. Grants up to $50k have been granted for projects in 33 countries, with plans for 12 other countries. The author describes the framework that the program works under, and the methodology followed in developing and planning projects. The approach to climate change concerns is to emphasize the development of non-carbon energy development activities to provide energy sources and economic development.

  14. Valorization of small uranium deposits

    International Nuclear Information System (INIS)

    For the valorization of small uranium deposits, the Total Mining Co. has developed an original leaching process in immersed live stalls, allied to the treatment of uraniferous liquors on ion exchanger resins. This technology was put in operation at Saint-Pierre (Cantal) in 1976, at La Ribiere (Creuse) in 1982 and in Bertholene (Aveyron) in 1985. In the last mentioned installation, 75 t of uranium can be produced per year on the basis of 60 000 t of ore containing 1.5 kg of uranium per ton. The investments per kg of uranium have thus been reduced to a quarter of their value in a conventional plant

  15. Small angle scattering and polymers

    International Nuclear Information System (INIS)

    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

  16. Small helical flux compression amplifiers

    International Nuclear Information System (INIS)

    Small, explosively compressed, magnetic flux transducers with many closely spaced helical turns are investigated theoretically and experimentally. The analysis is limited to linear operation, but takes into account load influence, proximity effects, and switching delays. The latter are due to retarded breakdown in the wire insulation and to the finite decay time of the magnetic field in the wire. More than 150 experiments showed considerable data scatter. Shots which exhibited low clocking and high amplification were in good agreement with the theory. The main conclusion is that device performance is limited not only by flux loss, but by flux remaining in the generator after compression

  17. 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.

  18. 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...... 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....

  19. 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

  20. Fuzzy Small Submodule and Jacobson -Radical

    OpenAIRE

    Saifur Rahman; Helen K. Saikia

    2011-01-01

    Using the notion of fuzzy small submodules of a module, we introduce the concept of fuzzy coessential extension of a fuzzy submodule of a module. We attempt to investigate various properties of fuzzy small submodules of a module. A necessary and sufficient condition for fuzzy small submodules is established. We investigate the nature of fuzzy small submodules of a module under fuzzy direct sum. Fuzzy small submodules of a module are characterized in terms of fuzzy quotient modules. This chara...

  1. Small bowel neoplasia in coeliac disease

    OpenAIRE

    Rampertab, S D; Forde, K A; Green, P. H. R.

    2003-01-01

    There is an increased risk of small bowel adenocarcinoma in patients with coeliac disease compared with the normal population. It has been suggested that adenocarcinoma of the small intestine in coeliac disease arises through an adenoma-carcinoma sequence but there has been only one reported case of a small bowel adenoma in a patient with coeliac disease. We report three additional cases of a small bowel adenoma in the setting of coeliac disease. In addition, four cases of small bowel adenoca...

  2. Small crater populations on Vesta

    CERN Document Server

    Marchi, S; O'Brien, D P; Schenk, P; Mottola, S; De Sanctis, M C; Kring, D A; Williams, D A; Raymond, C A; Russell, C T

    2013-01-01

    The NASA Dawn mission has extensively examined the surface of asteroid Vesta, the second most massive body in the main belt. The high quality of the gathered data provides us with an unique opportunity to determine the surface and internal properties of one of the most important and intriguing main belt asteroids (MBAs). In this paper, we focus on the size frequency distributions (SFDs) of sub-kilometer impact craters observed at high spatial resolution on several selected young terrains on Vesta. These small crater populations offer an excellent opportunity to determine the nature of their asteroidal precursors (namely MBAs) at sizes that are not directly observable from ground-based telescopes (i.e., below ~100 m diameter). Moreover, unlike many other MBA surfaces observed by spacecraft thus far, the young terrains examined had crater spatial densities that were far from empirical saturation. Overall, we find that the cumulative power-law index (slope) of small crater SFDs on Vesta is fairly consistent with...

  3. Railgun launch of small bodies

    Energy Technology Data Exchange (ETDEWEB)

    Drobyshevski, E.M.; Zhukov, B.G.; Sakharov, V.A. [Russian Academy of Sciences, St. Petersburg (Russian Federation). Ioffe Physico-Technical Inst.

    1995-01-01

    The small body launching using gas or plasma faces the fundamental problem caused by excess energy loss due to great wall surface/volume of the barrel ratio. That is why the efficiency of the plasma armature (PA) railgun acceleration is maximum for 8--10 mm-size bodies and drops as their size decreases. For the nuclear fusion applications, where {number_sign}1--2 mm-size pellets at 5--10 km/s velocity are desirable, one is forced to search for compromise between the body size (3--4 mm) and its velocity (3 km/s). Under these conditions, EM launchers did not demonstrate an advantage over the light-gas guns. When elaborating the {number_sign}1 mm railgun, the authors made use of the ideology of the body launching at constant acceleration close to the body strength or the electrode skin-layer explosion limits. That shortened the barrel length sufficiently. The system becomes highly compact thus permitting rapid test of new operation modes and different modifications of the design including the magnetic field augmentation. As a result of these refinements, the difficulties caused by the catastrophic supply of mass ablated from the electrodes were overcome and regimes of {number_sign}1 mm body non-sabot speed-up to 4.5 km/s were found. Potentialities of the small system created are far from being exhausted.

  4. 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.

  5. Management of Small Vessel Vasculitides.

    Science.gov (United States)

    Lopalco, Giuseppe; Rigante, Donato; Venerito, Vincenzo; Emmi, Giacomo; Anelli, Maria Grazia; Lapadula, Giovanni; Iannone, Florenzo; Cantarini, Luca

    2016-06-01

    Inflammation mediated by cells of the immune system and necrosis are the most striking features observed at the histologic level in patients with vasculitides, clinical entities classified according to pathologic findings involving different organs, to etiology, or to size of vessels involved. Small vessel vasculitides (SVV) are a peculiar group of systemic disorders electively involving small intraparenchymal arteries, arterioles, capillaries, or venules and leading to different levels of vascular obstruction, tissue ischemia and risk of infarction; they can be divided into anti-neutrophil cytoplasmic antibody-associated vasculitides and immune complex vasculitides. Despite the significant advances in understanding the whole disease process and pathophysiology of SVV, strong efforts are still needed to draft, share and spread guidelines in the therapeutic management of these protean disorders. After an accurate evaluation of different open or double-blind trials and cohort studies in this review, we analyze the actual medical tools suggested for treating granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, Henoch-Schönlein purpura, cryoglobulinemic vasculitis, anti-glomerular basement membrane disease and hypocomplementemic urticarial vasculitis. PMID:27118389

  6. Small-sized centrifugal extractors

    International Nuclear Information System (INIS)

    Small-sized centrifugal extractors (E) and small-sized clarifier (SCC) are described. The SCE construction provides their remote service with the help of manipulators. The arrangement of sections in the block of all apparatuses is the same and it differs only in the number of sections (4-14 sections). Phase mixing and separation are carried out in the sections. The sections rotate with the help of electromotor remotely replaced in case of necessity. The main construction material of the extractors is chromium-nickel steel, stainless bearings, interlaying material -polyethylene. On the basis of SCE construction the SCC is developed with the operating volume up to 1000 cm3 and capacity up to 200 l/h. The clarifier can be used for the control purification of solutions from solid ingredients in hot chambers with remote service. On the basis of the tests performed the conclusion may be made on the advisability of SCC application for preparing initial solutions before extraction

  7. Small Spacecraft for Planetary Science

    Science.gov (United States)

    Baker, John; Castillo-Rogez, Julie; Bousquet, Pierre-W.; Vane, Gregg; Komarek, Tomas; Klesh, Andrew

    2016-07-01

    As planetary science continues to explore new and remote regions of the Solar system with comprehensive and more sophisticated payloads, small spacecraft offer the possibility for focused and more affordable science investigations. These small spacecraft or micro spacecraft (electronics, advanced manufacturing for lightweight structures, and innovative propulsion are making it possible to fly much more capable micro spacecraft for planetary exploration. While micro spacecraft, such as CubeSats, offer significant cost reductions with added capability from advancing technologies, the technical challenges for deep space missions are very different than for missions conducted in low Earth orbit. Micro spacecraft must be able to sustain a broad range of planetary environments (i.e., radiations, temperatures, limited power generation) and offer long-range telecommunication performance on a par with science needs. Other capabilities needed for planetary missions, such as fine attitude control and determination, capable computer and data handling, and navigation are being met by technologies currently under development to be flown on CubeSats within the next five years. This paper will discuss how micro spacecraft offer an attractive alternative to accomplish specific science and technology goals and what relevant technologies are needed for these these types of spacecraft. Acknowledgements: Part of this work is being carried out at the Jet Propulsion Laboratory, California Institute of Technology under contract to NASA. Government sponsorship acknowledged.

  8. Low-energy small house

    Energy Technology Data Exchange (ETDEWEB)

    Kouhia, I.; Nieminen, J.; Erat, B. [Ekosolar Oy, Espoo (Finland); Kalema, T. [Tampere Univ. of Technology (Finland). Dept. of Thermal Engineering; Haapala, T. [Tampere Univ. of Technology (Finland). Dept. of Thermal Engineering; Punttila, A. [Rakennus Ekono Oy, Espoo (Finland); Nyman, M. [Technical Research Centre of Finland, Espoo (Finland). Lab. of Heating and Ventilation; Pulakka, S. [Technical Research Centre of Finland, Espoo (Finland). Lab. of Urban Planning and Building Design Engineering; Peippo, K. [Helsinki Univ. of Technology, Otaniemi (Finland). Dept. of Technical Physics

    1993-12-31

    Low-energy small-house designs for various heating energy consumption levels have been planned in this research project. The actual house designs have been presented in earlier reports of the project. The potential and profitability of various energy-saving measures have been examined in this report. The investigations indicated that improvement of the thermal insulation of the building envelope, utilization of advanced windows and the heat recovery from the exhaust air are the most profitable saving measures. These measures examined in terms of the annual costs of buildings are valuable saving measures even at an energy prize of 25 p/kWh. By using the above mentioned measures, it is possible to reduce the annual consumption of heating energy to 80 kWh/room m{sup 2}. The project has taken part in IEA cooperation by taking part in the solar research programmes project `Advanced solar low-energy buildings`, TASK XIII. A small house requiring very little energy has been planned within the framework of this cooperation, the annual consumption of the bought-in heating energy of which being around 3.0 kWh/room-m{sup 2}. A house will be built in Pietarsaari for the housing fair to be held in the summer 1994 (orig.)

  9. Small Spacecraft for Planetary Science

    Science.gov (United States)

    Baker, John; Castillo-Rogez, Julie; Bousquet, Pierre-W.; Vane, Gregg; Komarek, Tomas; Klesh, Andrew

    2016-07-01

    As planetary science continues to explore new and remote regions of the Solar system with comprehensive and more sophisticated payloads, small spacecraft offer the possibility for focused and more affordable science investigations. These small spacecraft or micro spacecraft (science-grade sensors and electronics, advanced manufacturing for lightweight structures, and innovative propulsion are making it possible to fly much more capable micro spacecraft for planetary exploration. While micro spacecraft, such as CubeSats, offer significant cost reductions with added capability from advancing technologies, the technical challenges for deep space missions are very different than for missions conducted in low Earth orbit. Micro spacecraft must be able to sustain a broad range of planetary environments (i.e., radiations, temperatures, limited power generation) and offer long-range telecommunication performance on a par with science needs. Other capabilities needed for planetary missions, such as fine attitude control and determination, capable computer and data handling, and navigation are being met by technologies currently under development to be flown on CubeSats within the next five years. This paper will discuss how micro spacecraft offer an attractive alternative to accomplish specific science and technology goals and what relevant technologies are needed for these these types of spacecraft. Acknowledgements: Part of this work is being carried out at the Jet Propulsion Laboratory, California Institute of Technology under contract to NASA. Government sponsorship acknowledged.

  10. Small Space Launch: Origins & Challenges

    Science.gov (United States)

    Freeman, T.; Delarosa, J.

    2010-09-01

    The United States Space Situational Awareness capability continues to be a key element in obtaining and maintaining the high ground in space. Space Situational Awareness satellites are critical enablers for integrated air, ground and sea operations, and play an essential role in fighting and winning conflicts. The United States leads the world space community in spacecraft payload systems from the component level into spacecraft, and in the development of constellations of spacecraft. In the area of launch systems that support Space Situational Awareness, despite the recent development of small launch vehicles, the United States launch capability is dominated by an old, unresponsive and relatively expensive set of launchers in the Expandable, Expendable Launch Vehicles (EELV) platforms; Delta IV and Atlas V. The United States directed Air Force Space Command to develop the capability for operationally responsive access to space and use of space to support national security, including the ability to provide critical space capabilities in the event of a failure of launch or on-orbit capabilities. On 1 Aug 06, Air Force Space Command activated the Space Development & Test Wing (SDTW) to perform development, test and evaluation of Air Force space systems and to execute advanced space deployment and demonstration projects to exploit new concepts and technologies, and rapidly migrate capabilities to the warfighter. The SDTW charged the Launch Test Squadron (LTS) with the mission to develop the capability of small space launch, supporting government research and development space launches and missile defense target missions, with operationally responsive spacelift for Low-Earth-Orbit Space Situational Awareness assets as a future mission. This new mission created new challenges for LTS. The LTS mission tenets of developing space launches and missile defense target vehicles were an evolution from the squadrons previous mission of providing sounding rockets under the Rocket

  11. Mental retardation, premature balding, small genitalia, small acra and small patellae in brothers: Confirmation of an entity

    NARCIS (Netherlands)

    A.M. Vandersteen; R.C. Hennekam

    2010-01-01

    We describe two brothers with moderate to severe mental retardation, short stature, an unusual skull shape, early anterior balding, unusual facial morphology, hypogonadotrophic hypogonadism, small genitalia, and small patellae. The older sib had generalized hypotonia without focal neurological abnor

  12. Small Data Archives and Libraries

    Science.gov (United States)

    Holl, A.

    2010-10-01

    Preservation is important for documenting original observations, and existing data are an important resource which can be re-used. Observatories should set up electronic data archives and formulate archiving policies. VO (Virtual Observatory) compliance is desirable; even if this is not possible, at least some VO ideas should be applied. Data archives should be visible and their data kept on-line. Metadata should be plentiful, and as standard as possible, just like file formats. Literature and data should be cross-linked. Libraries can play an important role in this process. In this paper, we discuss data archiving for small projects and observatories. We review the questions of digitization, cost factors, manpower, organizational structure and more.

  13. Mycoplasmas: small but perfectly formed

    Directory of Open Access Journals (Sweden)

    Robin NICHOLAS

    2015-07-01

    Full Text Available Interest in mycoplasmas stems from their importance as serious animal and human pathogens, their frequent contamination of cell lines and, because of their small size, for their use as models of the minimal cell concept.They have traditionally been seen as highly host specific but this is being reviewed in view of recent detections of animal mycoplasmas in unlikely hosts including humans. Some highlights include:Mycoplasmas, containing as few as 450 genes, are the smallest free-living organisms on the planetThey were the first synthetically created life formsWhile the majority of mycoplasmas are harmless, the World Organisation for Animal Health (OIE have listed 4 mycoplasma diseases because of their serious socio-economic impacts10-15% of cell lines used for virus research are contaminated with mycoplasma Are they the real the causes of TSEs and other undiagnosed human conditions?

  14. Small-scale classification schemes

    DEFF Research Database (Denmark)

    Hertzum, Morten

    2004-01-01

    important means of discretely balancing the contractual aspect of requirements engineering against facilitating the users in an open-ended search for their system requirements. The requirements classification is analysed in terms of the complementary concepts of boundary objects and coordination mechanisms......Small-scale classification schemes are used extensively in the coordination of cooperative work. This study investigates the creation and use of a classification scheme for handling the system requirements during the redevelopment of a nation-wide information system. This requirements...... classification inherited a lot of its structure from the existing system and rendered requirements that transcended the framework laid out by the existing system almost invisible. As a result, the requirements classification became a defining element of the requirements-engineering process, though its main...

  15. Semitransparent peroral small bowel imaging

    Energy Technology Data Exchange (ETDEWEB)

    Emons, D.

    1981-10-01

    171 follow-through examinations of the small bowel performed in children and adolescents with a large contrast medium meal and the high voltage-low density barium technique (10 to 25 g BaSO/sub 4//100 ml, depending on age), are described. A ready made suspension, diluted with water, proved unsatisfactory. Coating properties and stability of the diluted, weak suspension were then greatly improved by hydroxyethylcellulose as a thickening agent and in addition by premedication of the patient with cimetidine. Pure cellulose solution instead of the last portion of barium prevented thickening in the ileum. The procedure has the well known advantages of a large contrast medium meal without the problem of overly dense superpositions.

  16. 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.

  17. Aeration equipment for small depths

    Science.gov (United States)

    Sluše, Jan; Pochylý, František

    2015-05-01

    Deficit of air in water causes complications with cyanobacteria mainly in the summer months. Cyanobacteria is a bacteria that produces poison called cyanotoxin. When the concentration of cyanobacteria increases, the phenomena "algal bloom" appears, which is very toxic and may kill all the organisms. This article describes new equipment for aeration of water in dams, ponds and reservoirs with small depth. This equipment is mobile and it is able to work without any human factor because its control is provided by a GPS module. The main part of this equipment consists of a floating pump which pumps water from the surface. Another important part of this equipment is an aerator where water and air are blended. Final aeration process runs in the nozzles which provide movement of all this equipment and aeration of the water. Simulations of the flow are solved by multiphase flow with diffusion in open source program called OpenFOAM. Results will be verified by an experiment.

  18. Evaluation of small reactor economics

    International Nuclear Information System (INIS)

    The EPRI small reactor design program requires a procedure to analyze the economic benefits of specific design improvements suggested by the participating contractors, and to evaluate the total impacts of a new plant design on nuclear power generation costs. To that purpose, an analysis procedure is now being developed by EPRI and its participating contractors. This procedure involves the use of two separate costing models: 1. A top-down total generation cost model that integrates, in a relatively simplified manner, capital operating and fuel expenses, to yield total unit costs on an annual as well as on a lifetime levelized basis. A bottoms-up model that enables the analysis of specific system changes on station capital costs. Using a detailed reference plant cost breakdown, proposed design improvements can be measured against the reference data for their economic benefits

  19. Small Cell Network Topology Comparison

    Directory of Open Access Journals (Sweden)

    Jan Oppolzer

    2013-01-01

    Full Text Available One of the essential problems in a mobile network with small cells is that there is only a limited number of (PCIs available. Due to this fact, operators face the inevitable need for reusing (PCIs. In our contribution, we are dealing with a (PCI assignment to FAPs in three different topologies. The first model places FAPs randomly within the network while respecting overlapping defined. The second model places FAPs in a grid without other restrictions. The third model forms a grid as well, although buildings and roads are taken into account and (FAPs are always inside buildings. The proposed models are compared and a conclusion is made based on simulation results.

  20. Small Minors in Dense Graphs

    CERN Document Server

    Fiorini, Samuel; Theis, Dirk Oliver; Wood, David R

    2010-01-01

    A fundamental result in structural graph theory states that every graph with large average degree contains a large complete graph as a minor. We prove this result with the extra property that the minor is small with respect to the order of the whole graph. More precisely, we describe functions $f$ and $g$ such that every graph with $n$ vertices and average degree at least $f(t)$ contains a $K_t$-model with at most $g(t)\\cdot\\log n$ vertices. The logarithmic dependence on $n$ is best possible. For $t\\leq 4$, we determine the least value of $f(t)$; in particular $f(3)=2+\\eps$ and $f(4)=4+\\eps$. For $t\\leq4$, we establish similar results for graphs embedded on surfaces, where the size of the $K_t$-model is bounded.

  1. 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.

  2. Small government or big government?

    Directory of Open Access Journals (Sweden)

    MATEO SPAHO

    2015-03-01

    Full Text Available Since the beginning of the twentieth century, economists and philosophers were polarizedon their positions beyond the role that the government should have in the economy. On one hand John Maynard Keynes represented, within the optics of market economy, a position where the state should intervene in the economy to maintain the aggregate demand and the employment in the country, without hesitation in creating budget deficits and public debt expansion. This approach happens especially in the moments when the domestic economy and global economic trends show a weak growth or a recession. This means a heavy interference inthe economy, with higher income but with high expenditure to GDP too. On the other side, Liberals and Neoliberalsled by Friedrich Hayek advocated a withdrawal of the government from economic activity not just in moments of economic growth but also during the crisis, believing that the market has self-regulating mechanisms within itself. The government, as a result will have a smaller dimension with lower income and also low expenditures compared to the GDP of the country. We took the South-Eastern Europe countries distinguishing those with a "Big Government" or countries with "Small Government". There are analyzed the economic performances during the global crisis (2007-2014. In which countries the public debt grew less? Which country managed to attract more investments and which were the countries that preserved the purchasing power of their consumers? We shall see if during the economic crisis in Eastern Europe the Great Government or the Liberal and "Small" one has been the most successful the model.

  3. Small boiler uses waste coal

    Energy Technology Data Exchange (ETDEWEB)

    Virr, M.J. [Spinheat Ltd. (United States)

    2009-07-15

    Burning coal waste in small boilers at low emissions poses considerable problem. While larger boiler suppliers have successfully installed designs in the 40 to 80 MW range for some years, the author has been developing small automated fluid bed boiler plants for 25 years that can be applied in the range of 10,000 to 140,000 lbs/hr of steam. Development has centered on the use of an internally circulating fluid bed (CFB) boiler, which will burn waste fuels of most types. The boiler is based on the traditional D-shaped watertable boiler, with a new type of combustion chamber that enables a three-to-one turndown to be achieved. The boilers have all the advantages of low emissions of the large fluid boilers while offering a much lower height incorporated into the package boiler concept. Recent tests with a waste coal that had a high nitrogen content of 1.45% demonstrated a NOx emission below the federal limit of 0.6 lbs/mm Btu. Thus a NOx reduction on the order of 85% can be demonstrate by combustion modification alone. Further reductions can be made by using a selective non-catalytic reduction (SNCR) system and sulfur absorption of up to 90% retention is possible. The article describes the operation of a 30,000 lbs/hr boiler at the Fayette Thermal LLC plant. Spinheat has installed three ICFB boilers at a nursing home and a prison, which has been tested on poor-grade anthracite and bituminous coal. 2 figs.

  4. Tissue engineering the small intestine.

    Science.gov (United States)

    Spurrier, Ryan G; Grikscheit, Tracy C

    2013-04-01

    Short bowel syndrome (SBS) results from the loss of a highly specialized organ, the small intestine. SBS and its current treatments are associated with high morbidity and mortality. Production of tissue-engineered small intestine (TESI) from the patient's own cells could restore normal intestinal function via autologous transplantation. Improved understanding of intestinal stem cells and their niche have been coupled with advances in tissue engineering techniques. Originally described by Vacanti et al of Massachusetts General Hospital, TESI has been produced by in vivo implantation of organoid units. Organoid units are multicellular clusters of epithelium and mesenchyme that may be harvested from native intestine. These clusters are loaded onto a scaffold and implanted into the host omentum. The scaffold provides physical support that permits angiogenesis and vasculogenesis of the developing tissue. After a period of 4 weeks, histologic analyses confirm the similarity of TESI to native intestine. TESI contains a differentiated epithelium, mesenchyme, blood vessels, muscle, and nerve components. To date, similar experiments have proved successful in rat, mouse, and pig models. Additional experiments have shown clinical improvement and rescue of SBS rats after implantation of TESI. In comparison with the group that underwent massive enterectomy alone, rats that had surgical anastomosis of TESI to their shortened intestine showed improvement in postoperative weight gain and serum B12 values. Recently, organoid units have been harvested from human intestinal samples and successfully grown into TESI by using an immunodeficient mouse host. Current TESI production yields approximately 3 times the number of cells initially implanted, but improvements in the scaffold and blood supply are being developed in efforts to increase TESI size. Exciting new techniques in stem cell biology and directed cellular differentiation may generate additional sources of autologous intestinal

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

    Science.gov (United States)

    2010-10-28

    ...: I. Background On October 7, 2010 (75 FR 62258), the Small Business Administration (SBA) issued a... From the Federal Register Online via the Government Publishing Office SMALL BUSINESS ADMINISTRATION Small Entity Compliance Guide: Women-Owned Small Business Program AGENCY: Small...

  6. 76 FR 5680 - Small Business, Small Disadvantaged Business, HUBZone, and Service-Disabled Veteran-Owned...

    Science.gov (United States)

    2011-02-02

    ... From the Federal Register Online via the Government Publishing Office ] SMALL BUSINESS ADMINISTRATION 13 CFR Parts 121, 124, 125, 126, and 134 RIN 3245-AF65 Small Business, Small Disadvantaged.... AGENCY: U.S. Small Business Administration. ACTION: Final rule. SUMMARY: The U.S. Small...

  7. 77 FR 46909 - Small Business Innovation Research (SBIR) Program and Small Business Technology Transfer (STTR...

    Science.gov (United States)

    2012-08-06

    .... 77, No. 151 / Monday, August 6, 2012 / Notices#0;#0; ] SMALL BUSINESS ADMINISTRATION Small Business Innovation Research (SBIR) Program and Small Business Technology Transfer (STTR) Program Policy Directives... Small Business Innovation Research (SBIR) and Small Business Technology Transfer Program (STTR)...

  8. 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.

  9. 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...

  10. Small-angle neutron-scattering experiments

    International Nuclear Information System (INIS)

    A brief introduction to small-angle scattering (SAS) is given. Initial results obtained using the AERE small-angle diffractometer are presented for four materials of interest: doped uranium dioxide, nitrided steel, graphite and alumina. (U.K.)

  11. Cancer Statistics: Cancer of the Small Intestine

    Science.gov (United States)

    ... party. HPF: SEER Stat Fact Sheets: Small Intestine Cancer Expand All Collapse All Lifetime risk estimates are ... Or More after Being Diagnosed with Small Intestine Cancer? Relative survival statistics compare the survival of patients ...

  12. 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 ... Horn L, Eisenberg R, Gius D, et al. Cancer of the lung. In: Niederhuber JE, Armitage JO, Doroshow JH, Kastan ...

  13. Specifics of Small Satellite Propulsion: Part 1

    OpenAIRE

    Zakirov, Vadim; Sweeting, Martin; Erichsen, Peter; Lawrence, Timothy

    2001-01-01

    Small satellite propulsion is a subject of unique constraints and requirements. Based on University of Surrey experience in small satellite building and operation, these features are listed and explained. Available volume is often identified as the most severe constraint for a small satellite with power and cost being the other two major constraints. Mass is often only of secondary importance for small satellites. Propulsion dry mass fraction for a spacecraft grows upon the system scaling-dow...

  14. Conservation of small RNA pathways in platypus

    OpenAIRE

    Murchison, Elizabeth P.; Kheradpour, Pouya; Sachidanandam, Ravi; Smith, Carly; Hodges, Emily; Xuan, Zhenyu; Kellis, Manolis; Grützner, Frank; Stark, Alexander; Hannon, Gregory J.

    2008-01-01

    Small RNA pathways play evolutionarily conserved roles in gene regulation and defense from parasitic nucleic acids. The character and expression patterns of small RNAs show conservation throughout animal lineages, but specific animal clades also show variations on these recurring themes, including species-specific small RNAs. The monotremes, with only platypus and four species of echidna as extant members, represent the basal branch of the mammalian lineage. Here, we examine the small RNA pat...

  15. Small Business Performance in Urban Tourism

    OpenAIRE

    Daskalopoulou, Irene; Petrou, Anastasia

    2006-01-01

    Research findings indicate that the successful performance of small businesses is an important determinant of regional development. Successful business performance is affected by a number of firm-specific factors including human and social capital. Although, small and medium firms comprise the vast majority of the tourism production system, research on small business performance in tourism is rather limited. Drawing on recent advances and empirical evidence from enterpreneurship and small bus...

  16. Is small business a safety problem?

    OpenAIRE

    Larsson, Tore J

    2003-01-01

    Common beliefs in relation to safety in small business are of two types; a) small businesses are more unsafe, apply unsafe practices, and thus have a higher incidence of occupational injury and a higher average severity of injury, and b) small business have poor knowledge of, and exhibit low compliance with, laws, rules and regulations, and are increasingly difficult to control. A survey of 100 small business operators in the suburbs of Melbourne, Australia was undertaken. Ten different indus...

  17. Marketing Channels Used by Small Tennessee Farmers

    OpenAIRE

    Tegegne, Fisseha; Pasirayi, Simbarashe; Singh, Surendra P.; Ekanem, Enefiok P.

    2012-01-01

    One of the key challenges that small farmers face is marketing their products. National and international markets are difficult to tap into for small farmers due to their inability to compete with large farm operators that dominate these markets. The objective of this study was to examine marketing channels used by small Tennessee Farmers. A mail survey was sent to 250 selected small farmers in Tennessee. Ninety-two completed responses, representing about 37% response rate, were received. Ove...

  18. 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

  19. Understanding the statistics of small risks

    International Nuclear Information System (INIS)

    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

  20. Generation of Small Enterprises’ Multivariate Sample Aggregates

    OpenAIRE

    Bokun, N. Ch.

    2007-01-01

    The work gives methodology of small enterprises’ multivariate sample aggregates in order to provide more comprehensive statistical evaluation of small institutional units’ economic activity. The strategy is found on usage cluster analysis. The article provides results of small enterprises’ selection carried out in Minsk City.

  1. Yes, small firms can commercialize photonics

    Science.gov (United States)

    Nelson, Carl W.

    1997-01-01

    Small young companies can commercialize photonics. Many are already doing so and may more are getting started with a small government subsidy from Small Business Innovation Research. Capital is gushing, even begging for good investment opportunities, for companies ready to commit themselves to commercial success.

  2. Small Satellite Space Environments Effects Test Facility

    OpenAIRE

    Dennison, JR; Hartley, Kent; Montierth Phillipps, Lisa; Johnson, Robert; Dekany, Justin; Dyer, James

    2014-01-01

    A versatile space environments test facility has been designed and built to study the effects on small satellites and system components. Testing for potentially environmental-induced modifications of small satellites is critical to avoid possible deleterious or catastrophic effects over the duration of space missions. This is increasingly more important as small satellite programs have longer mission lifetimes, expand to more hash env...

  3. 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...

  4. 78 FR 59798 - Small Business Subcontracting: Correction

    Science.gov (United States)

    2013-09-30

    ..., 2013 (78 FR 42391). The document amended SBA's regulations governing small business subcontracting to... From the Federal Register Online via the Government Publishing Office SMALL BUSINESS ADMINISTRATION 13 CFR Part 125 RIN 3245-AG22 Small Business Subcontracting: Correction AGENCY: U.S....

  5. Leiomyosarcoma in leiomyomatosis of the small intestine.

    OpenAIRE

    EL-OMAR, M.; Davies, J.; Gupta, S.; Ross, H.; Thompson, R.

    1994-01-01

    Multiple leiomyomata of the small intestine are rare. We report one such case where a leiomyosarcoma had arisen from a leiomyoma in the small intestine 8 years after presentation. The possible origin of the leiomyomata is discussed and it is concluded that small intestinal leiomyomatosis should be regarded as a premalignant condition.

  6. New examples of small Polish structures

    OpenAIRE

    Dobrowolski, Jan

    2013-01-01

    We answer some questions from a paper of Krupi\\'nski by giving suitable examples of small Polish structures. First, we present a class of small Polish group structures without generic elements. Next, we construct a first example of a small non-zero-dimensional Polish $G$-group.

  7. Small-bowel permeability in collagenous colitis

    DEFF Research Database (Denmark)

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

    2006-01-01

    , indicating that CC is a pan-intestinal disease. In small-intestinal disease, the intestinal barrier function may be impaired, and the permeability of the small intestine altered. The purpose of this research was to study small-bowel function in patients with CC as expressed by intestinal permeability....

  8. 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...

  9. Describing the hexapeptide identity platform between the influenza A H5N1 and Homo sapiens proteomes.

    Science.gov (United States)

    Kanduc, Darja

    2010-01-01

    We searched the primary sequence of influenza A H5N1 polyprotein for hexamer amino acid sequences shared with human proteins using the Protein International Resource database and the exact peptide matching analysis program. We find that the viral polyprotein shares numerous hexapeptides with the human proteome. The human proteins involved in the viral overlap are represented by antigens associated with basic cell functions such as proliferation, development, and differentiation. Of special importance, many human proteins that share peptide sequences with influenza A polyprotein are antigens such as reelin, neurexin I-α, myosin-IXa, Bardet-Biedl syndrome 10 protein, Williams syndrome transcription factor, disrupted in schizophrenia 1 protein, amyotrophic lateral sclerosis 2 chromosomal region candidate gene 17 protein, fragile X mental retardation 2 protein, and jouberin. That is, the viral-vs-human overlap involves human proteins that, when altered, have been reported to be potentially associated with multiple neurological disorders that can include autism, epilepsy, obesity, dystonia, ataxia-telangiectasia, amyotrophic lateral sclerosis, sensorineural deafness, sudden infant death syndrome, Charcot-Marie-Tooth disease, and myelination. The present data are discussed as a possible molecular basis for understanding influenza A viral escape from immunosurveillance and for defining anti-influenza immune-therapeutic approaches devoid of collateral adverse events. PMID:20859452

  10. A case of McKusick-Kaufman syndrome

    Directory of Open Access Journals (Sweden)

    Se Hyung Son

    2011-05-01

    Full Text Available McKusick-Kaufman syndrome (MKS is an autosomal recessive multiple malformation syndrome characterized by hydrometrocolpos (HMC and postaxial polydactyly (PAP. We report a case of a female child with MKS who was transferred to the neonatal intensive care unit of Seoul National University Children’s Hospital on her 15th day of life for further evaluation and management of an abdominal cystic mass. She underwent abdominal sonography, magnetic resonance imaging, genitography and cystoscopy which confirmed HMC with a transverse vaginal septum. X-rays of the hand and foot showed bony fusion of the left third and fourth metacarpal bones, right fourth dysplastic metacarpal bone and phalanx, right PAP and hypoplastic left foot with left fourth and fifth dysplastic metatarsal bones. In addition, she had soft palate cleft, mild hydronephroses of both kidneys, hypoplastic right kidney with ectopic location and mild rotation, uterine didelphys with transverse vaginal septum and low-type imperforated anus. She was temporarily treated with ultrasoundguided transurethral aspiration of the HMC. Our patient with HMC and PAP was diagnosed with MKS because she has two typical abnormality of MKS and she has no definite complications of retinal disease, learning disability, obesity and renal failure that develop in Bardet-Biedl syndrome, but not in MKS until 33 months of age. Here, we describe a case of a Korean patient with MKS.

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

    Directory of Open Access Journals (Sweden)

    Rannar Airik

    Full Text Available 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, ERC1, and with non-muscle myosin motor proteins (MYH9, MYH10, MYH14 at the centrosome. Furthermore, we show that RABEP2 localization at the centrosome is regulated by SDCCAG8. siRNA mediated RABEP2 knockdown in hTERT-RPE1 cells leads to defective ciliogenesis, indicating a critical role for RABEP2 in this process. Together, this study identifies several centrosome-associated proteins as novel SDCCAG8 interaction partners, and provides new insights into the function of SDCCAG8 at this structure.

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

    Science.gov (United States)

    Schueler, Markus; Airik, Merlin; Cho, Jang; Ulanowicz, Kelsey A.; Porath, Jonathan D.; Hurd, Toby W.; Bekker-Jensen, Simon; Schrøder, Jacob M.; Andersen, Jens S.; Hildebrandt, Friedhelm

    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, ERC1), and with non-muscle myosin motor proteins (MYH9, MYH10, MYH14) at the centrosome. Furthermore, we show that RABEP2 localization at the centrosome is regulated by SDCCAG8. siRNA mediated RABEP2 knockdown in hTERT-RPE1 cells leads to defective ciliogenesis, indicating a critical role for RABEP2 in this process. Together, this study identifies several centrosome-associated proteins as novel SDCCAG8 interaction partners, and provides new insights into the function of SDCCAG8 at this structure. PMID:27224062

  13. 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.

  14. Experience of a single center with congenital hepatic fibrosis: A review of the literature

    Directory of Open Access Journals (Sweden)

    Ali Shorbagi, Yusuf Bayraktar

    2010-02-01

    Full Text Available Congenital hepatic fibrosis (CHF is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. It is one of the fibropolycystic diseases, which also include Caroli disease, autosomal dominant polycystic kidney disease, and autosomal recessive polycystic kidney disease. Clinically it is characterized by hepatic fibrosis, portal hypertension, and renal cystic disease. CHF is known to occur in association with a range of both inherited and non-inherited disorders, with multiorgan involvement, as a result of ductal plate malformation. Because of the similarities in the clinical picture, it is necessary to differentiate CHF from idiopathic portal hypertension and early liver cirrhosis, for which a liver biopsy is essential. Radiological tests are important for recognizing involvement of other organ systems. With regards to our experience at Hacettepe University, a total of 26 patients have been diagnosed and followed-up between 1974 and 2009 with a diagnosis of CHF. Presentation with Caroli syndrome was the most common diagnosis, with all such patients presenting with symptoms of recurrent cholangitis and symptoms related to portal hypertension. Although portal fibrosis is known to contribute to the ensuing portal hypertension, it is our belief that portal vein cavernous transformation also plays an important role in its pathogenesis. In all patients with CHF portal vein morphology should be evaluated by all means since portal vein involvement results in more severe and complicated portal hypertension. Other associations include the Joubert and Bardet-Biedl syndromes.

  15. Functional coordination of intraflagellar transport motors.

    Science.gov (United States)

    Ou, Guangshuo; Blacque, Oliver E; Snow, Joshua J; Leroux, Michel R; Scholey, Jonathan M

    2005-07-28

    Cilia have diverse roles in motility and sensory reception, and defects in cilia function contribute to ciliary diseases such as Bardet-Biedl syndrome (BBS). Intraflagellar transport (IFT) motors assemble and maintain cilia by transporting ciliary precursors, bound to protein complexes called IFT particles, from the base of the cilium to their site of incorporation at the distal tip. In Caenorhabditis elegans, this is accomplished by two IFT motors, kinesin-II and osmotic avoidance defective (OSM)-3 kinesin, which cooperate to form two sequential anterograde IFT pathways that build distinct parts of cilia. By observing the movement of fluorescent IFT motors and IFT particles along the cilia of numerous ciliary mutants, we identified three genes whose protein products mediate the functional coordination of these motors. The BBS proteins BBS-7 and BBS-8 are required to stabilize complexes of IFT particles containing both of the IFT motors, because IFT particles in bbs-7 and bbs-8 mutants break down into two subcomplexes, IFT-A and IFT-B, which are moved separately by kinesin-II and OSM-3 kinesin, respectively. A conserved ciliary protein, DYF-1, is specifically required for OSM-3 kinesin to dock onto and move IFT particles, because OSM-3 kinesin is inactive and intact IFT particles are moved by kinesin-II alone in dyf-1 mutants. These findings implicate BBS ciliary disease proteins and an OSM-3 kinesin activator in the formation of two IFT pathways that build functional cilia. PMID:16049494

  16. An essential role for DYF-11/MIP-T3 in assembling functional intraflagellar transport complexes.

    Directory of Open Access Journals (Sweden)

    Chunmei Li

    2008-03-01

    Full Text Available MIP-T3 is a human protein found previously to associate with microtubules and the kinesin-interacting neuronal protein DISC1 (Disrupted-in-Schizophrenia 1, but whose cellular function(s remains unknown. Here we demonstrate that the C. elegans MIP-T3 ortholog DYF-11 is an intraflagellar transport (IFT protein that plays a critical role in assembling functional kinesin motor-IFT particle complexes. We have cloned a loss of function dyf-11 mutant in which several key components of the IFT machinery, including Kinesin-II, as well as IFT subcomplex A and B proteins, fail to enter ciliary axonemes and/or mislocalize, resulting in compromised ciliary structures and sensory functions, and abnormal lipid accumulation. Analyses in different mutant backgrounds further suggest that DYF-11 functions as a novel component of IFT subcomplex B. Consistent with an evolutionarily conserved cilia-associated role, mammalian MIP-T3 localizes to basal bodies and cilia, and zebrafish mipt3 functions synergistically with the Bardet-Biedl syndrome protein Bbs4 to ensure proper gastrulation, a key cilium- and basal body-dependent developmental process. Our findings therefore implicate MIP-T3 in a previously unknown but critical role in cilium biogenesis and further highlight the emerging role of this organelle in vertebrate development.

  17. Sensory ciliogenesis in Caenorhabditis elegans: assignment of IFT components into distinct modules based on transport and phenotypic profiles.

    Science.gov (United States)

    Ou, Guangshuo; Koga, Makato; Blacque, Oliver E; Murayama, Takashi; Ohshima, Yasumi; Schafer, Jenny C; Li, Chunmei; Yoder, Bradley K; Leroux, Michel R; Scholey, Jonathan M

    2007-05-01

    Sensory cilium biogenesis within Caenorhabditis elegans neurons depends on the kinesin-2-dependent intraflagellar transport (IFT) of ciliary precursors associated with IFT particles to the axoneme tip. Here we analyzed the molecular organization of the IFT machinery by comparing the in vivo transport and phenotypic profiles of multiple proteins involved in IFT and ciliogenesis. Based on their motility in wild-type and bbs (Bardet-Biedl syndrome) mutants, IFT proteins were classified into groups with similar transport profiles that we refer to as "modules." We also analyzed the distribution and transport of fluorescent IFT particles in multiple known ciliary mutants and 49 new ciliary mutants. Most of the latter mutants were snip-SNP mapped and one, namely dyf-14(ks69), was cloned and found to encode a conserved protein essential for ciliogenesis. The products of these ciliogenesis genes could also be assigned to the aforementioned set of modules or to specific aspects of ciliogenesis, based on IFT particle dynamics and ciliary mutant phenotypes. Although binding assays would be required to confirm direct physical interactions, the results are consistent with the hypothesis that the C. elegans IFT machinery has a modular design, consisting of modules IFT-subcomplex A, IFT-subcomplex B, and a BBS protein complex, in addition to motor and cargo modules, with each module contributing to distinct functional aspects of IFT or ciliogenesis. PMID:17314406

  18. Caenorhabditis elegans DYF-2, an orthologue of human WDR19, is a component of the intraflagellar transport machinery in sensory cilia.

    Science.gov (United States)

    Efimenko, Evgeni; Blacque, Oliver E; Ou, Guangshuo; Haycraft, Courtney J; Yoder, Bradley K; Scholey, Jonathan M; Leroux, Michel R; Swoboda, Peter

    2006-11-01

    The intraflagellar transport (IFT) machinery required to build functional cilia consists of a multisubunit complex whose molecular composition, organization, and function are poorly understood. Here, we describe a novel tryptophan-aspartic acid (WD) repeat (WDR) containing IFT protein from Caenorhabditis elegans, DYF-2, that plays a critical role in maintaining the structural and functional integrity of the IFT machinery. We determined the identity of the dyf-2 gene by transgenic rescue of mutant phenotypes and by sequencing of mutant alleles. Loss of DYF-2 function selectively affects the assembly and motility of different IFT components and leads to defects in cilia structure and chemosensation in the nematode. Based on these observations, and the analysis of DYF-2 movement in a Bardet-Biedl syndrome mutant with partially disrupted IFT particles, we conclude that DYF-2 can associate with IFT particle complex B. At the same time, mutations in dyf-2 can interfere with the function of complex A components, suggesting an important role of this protein in the assembly of the IFT particle as a whole. Importantly, the mouse orthologue of DYF-2, WDR19, also localizes to cilia, pointing to an important evolutionarily conserved role for this WDR protein in cilia development and function. PMID:16957054

  19. An essential role for DYF-11/MIP-T3 in assembling functional intraflagellar transport complexes.

    Science.gov (United States)

    Li, Chunmei; Inglis, Peter N; Leitch, Carmen C; Efimenko, Evgeni; Zaghloul, Norann A; Mok, Calvin A; Davis, Erica E; Bialas, Nathan J; Healey, Michael P; Héon, Elise; Zhen, Mei; Swoboda, Peter; Katsanis, Nicholas; Leroux, Michel R

    2008-03-01

    MIP-T3 is a human protein found previously to associate with microtubules and the kinesin-interacting neuronal protein DISC1 (Disrupted-in-Schizophrenia 1), but whose cellular function(s) remains unknown. Here we demonstrate that the C. elegans MIP-T3 ortholog DYF-11 is an intraflagellar transport (IFT) protein that plays a critical role in assembling functional kinesin motor-IFT particle complexes. We have cloned a loss of function dyf-11 mutant in which several key components of the IFT machinery, including Kinesin-II, as well as IFT subcomplex A and B proteins, fail to enter ciliary axonemes and/or mislocalize, resulting in compromised ciliary structures and sensory functions, and abnormal lipid accumulation. Analyses in different mutant backgrounds further suggest that DYF-11 functions as a novel component of IFT subcomplex B. Consistent with an evolutionarily conserved cilia-associated role, mammalian MIP-T3 localizes to basal bodies and cilia, and zebrafish mipt3 functions synergistically with the Bardet-Biedl syndrome protein Bbs4 to ensure proper gastrulation, a key cilium- and basal body-dependent developmental process. Our findings therefore implicate MIP-T3 in a previously unknown but critical role in cilium biogenesis and further highlight the emerging role of this organelle in vertebrate development. PMID:18369462

  20. The BBSome controls IFT assembly and turnaround in cilia.

    Science.gov (United States)

    Wei, Qing; Zhang, Yuxia; Li, Yujie; Zhang, Qing; Ling, Kun; Hu, Jinghua

    2012-09-01

    The bidirectional movement of intraflagellar transport (IFT) particles, which are composed of motors, IFT-A and IFT-B subcomplexes, and cargoes, is required for the biogenesis and signalling of cilia(1,2). A successful IFT cycle depends on the proper assembly of the massive IFT particle at the ciliary base and its turnaround from anterograde to retrograde transport at the ciliary tip. However, how IFT assembly and turnaround are regulated in vivo remains elusive. From a whole-genome mutagenesis screen in Caenorhabditis elegans, we identified two hypomorphic mutations in dyf-2 and bbs-1 as the only mutants showing normal anterograde IFT transport but defective IFT turnaround at the ciliary tip. Further analyses revealed that the BBSome (refs 3, 4), a group of conserved proteins affected in human Bardet-Biedl syndrome(5) (BBS), assembles IFT complexes at the ciliary base, then binds to the anterograde IFT particle in a DYF-2- (an orthologue of human WDR19) and BBS-1-dependent manner, and lastly reaches the ciliary tip to regulate proper IFT recycling. Our results identify the BBSome as the key player regulating IFT assembly and turnaround in cilia. PMID:22922713

  1. 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. PMID:15916950

  2. Electric propulsion for small satellites

    International Nuclear Information System (INIS)

    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. (paper)

  3. 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.

  4. Assessing prospects for small reactors

    International Nuclear Information System (INIS)

    The use of nuclear power plants, like that of many other technologies, has been characterized by substantial growth in spread, numbers, equipment and plant sizes, with economies of scale generally favouring the large plants. Small and medium sized nuclear power plants, or SMPRs, are generally understood to be plants below the sizes being exploited commercially in industrialized countries. Such plants could be more readily adapted to smaller electric grids, particularly in developing countries, fulfilling requirements of low load growth, or serve remote locations or special purposes. The SMPR range is currently understood as the 200 to 500 megawatt (MWe) size for the generation of electricity and sometimes smaller for process-steam and/or low-temperature heat supply. For statistical purposes a size limit of 600 MWe has been in use by IAEA for some years. By this criterion some 140 nuclear power plants, or more than one-third of the world total, could be considered SMPRs. However, most of them are rather old vintage. Prospects are assessed under headings: indications of revived interest; today's SMPR supply situation; evolving IAEA programme - new study; market indications; economic competitiveness and financing aspects. (author)

  5. Public acceptance of small reactors

    International Nuclear Information System (INIS)

    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

  6. Small Technology Business Incubation Needs

    Energy Technology Data Exchange (ETDEWEB)

    None

    2007-12-31

    This report contains a summary of typical business incubation needs of small technology companies. This document will serve as a guide in the design and implementation of services offered by the National Security Technology Incubator (NSTI), an incubator program being designed and developed as part of the National Security Preparedness Project (NSPP), performed under a Department of Energy (DOE)/National Nuclear Security Administration (NNSA) grant. This report includes a brief description of the methodology used to perform the needs assessment and services proposed to meet the needs of client companies. The purpose of the NSPP is to promote national security technologies through business incubation, technology demonstration and validation, and workforce development. The NSTI will focus on serving businesses with national security technology applications by nurturing them through critical stages of early development. The vision of the NSTI is to be a successful incubator of technologies and private enterprise that assist the NNSA in meeting new challenges in national safety, security, and protection of the homeland.

  7. Small, moveable nuclear power plant

    International Nuclear Information System (INIS)

    Most reliable projections indicate that by the year 2000 approximately 25% of the world's electrical energy and possibly 10% of its process heat for industry will be provided by nuclear power plants. Whilst much of this will come from large nuclear steam raising units-in excess of 900 M.W.(e)-an increasing proportion will be provided from small modular plant, designed for portabiity or mobility. The technical, environmental and economic parameters of such a plant which will usually be of a size around 600 M.W.(e) or less and which in general terms derives its basic design features from nuclear marine propulsion units is reviewed. If suitably standardised, such a nuclear plant, mounted on barges, installed in ships or even airlifted into difficult locations, could provide an attractive solution to the energy problems of developing countries or remote communities. Apart from electrical energy production, the units could operate in a duplex mode to supply steam for central heating, sea-water or bore water distillation or industrial process heat. (Author)

  8. Limited small cell lung cancer

    International Nuclear Information System (INIS)

    This paper assesses the prognosis of patients with limited small cell lung cancer (LSCLC) not achieving complete response (CR) to induction combination chemotherapy (ICC) but who achieve CR after thoracic irradiation (TI). Twenty-four patients had CRs to ICC (CR- ICC) of two cycles of cytoxan, Adriamycin, and vincristine alternating with two cycles of cisplatin with VP-16. Another 24 had CR after consolidation with subsequent T1 (CR-T1): 45 Gy in daily fractions of 2.5 Gy or twice-daily fractions of 1.5 Gy. The CR-ICC and CR-TI patients had similar prognostic factors and treatment. Comparing CR-ICC and CR-TI, survival was 40% versus 26% at 2 years and 35% versus 4% at 5 years (P < .05). There were eight (33%) long-term survivors (≥3 years) in the CR-ICC group versus three (13%) in the CR-TI group. Local control for CR-ICC patients was 59% at 5 years versus 21% for the CR-TI patients (not significant). Freedom from DM for the CR-ICC patients was 41% at 5 years versus 8% for the CR-TI patients (P < .05)

  9. 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.

  10. Small drops from large nozzles

    Science.gov (United States)

    Castrejon-Pita, Alfonso Arturo; Said Mohamed, Ahmed; Castrejon-Pita, Jose Rafael; Herrada, Miguel Angel

    2015-11-01

    We report experimental and numerical results of the generation of drops which are significantly smaller than the nozzle from which they are generated. The system consists of a cylindrical reservoir and two endplates. One plate is a thin metal sheet with a small orifice in its centre which acts as the nozzle. The other end consists of a piston which moves by the action of an elecromechanical actuator which in turn is driven by sine-shape pull-mode pulses. The meniscus (formed at the nozzle) is thus first overturned, forming a cavity. This cavity collapses and a thin and fast jet emerges from its centre. Under appropriate conditions the tip of this jet breaks up and produces a single diminutive drop. A good agreement between the experimental and numerical results was found. Also, a series of experiments were performed in order to study the effects that the pulse amplitude and width, together with variations in the liquid properties, have over the final size of the droplet. Based on these experiments, a predictive law for the droplet size has been derived. This work was funded by the Royal Society (University Research Fellowship and Research Grant), the John Fell Fund (Oxford University Press), the Ministry of Science and Education (DPI2013-46485 Spain), and the Junta de Andalucia (P08-TEP-31704128 Spain).

  11. 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.

  12. 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. PMID:12267640

  13. Small is beautiful, and dry

    Institute of Scientific and Technical Information of China (English)

    SHERIDAN; John

    2010-01-01

    Thousands of plant and animal species have been observed to have superhydrophobic surfaces that lead to various novel behaviors. These observations have inspired attempts to create artificial superhydrophobic surfaces, given that such surfaces have multitudinous applications. Superhydrophobicity is an enhanced effect of surface roughness and there are known relationships that correlate surface roughness and superhydrophobicity, based on the underlying physics. However, while these examples demonstrate the level of roughness they tell us little about the independence of this effect in terms of its scale. Thus, they are not capable of explaining why such naturally occurring surfaces commonly have micron-submicron sizes. Here we report on the discovery of a new relation, its physical basis and its experimental verification. The results reveal that scaling-down roughness into the micro-submicron range is a unique and elegant strategy to not only achieve superhydrophobicity but also to increase its stability against environmental disturbances. This new relation takes into account the previously overlooked but key fact that the accumulated line energy arising from the numerous solid-water-air intersections that can be distributed over the apparent contact area, when air packets are trapped at small scales on the surface, can dramatically increase as the roughness scale shrinks. This term can in fact become the dominant contributor to the surface energy and so becomes crucial for accomplishing superhydrophobicity. These findings guide fabrication of stable super water-repellant surfaces.

  14. Aeration equipment for small depths

    Directory of Open Access Journals (Sweden)

    Sluše Jan

    2015-01-01

    Full Text Available Deficit of air in water causes complications with cyanobacteria mainly in the summer months. Cyanobacteria is a bacteria that produces poison called cyanotoxin. When the concentration of cyanobacteria increases, the phenomena „algal bloom“ appears, which is very toxic and may kill all the organisms. This article describes new equipment for aeration of water in dams, ponds and reservoirs with small depth. This equipment is mobile and it is able to work without any human factor because its control is provided by a GPS module. The main part of this equipment consists of a floating pump which pumps water from the surface. Another important part of this equipment is an aerator where water and air are blended. Final aeration process runs in the nozzles which provide movement of all this equipment and aeration of the water. Simulations of the flow are solved by multiphase flow with diffusion in open source program called OpenFOAM. Results will be verified by an experiment.

  15. Fast Development Of China's Small Satellite Industry

    Institute of Scientific and Technical Information of China (English)

    Sun Hongjin

    2009-01-01

    @@ China Spacesat Co., Ltd of China Academy of Space Technology (CAST) recently said, along with the successful launch of HJ-1A/B for the environment and disaster monitoring and forecasting small satellite constellation and after years of efforts, small satellite development technology has achieved fruitful results, and the development status has been greatly improved.China's small satellite technology has realized a great-leap-forward in development from a single satellite model to series model, from the satellite program to space industry. China has explored a development road for China's small satellite industrialization, and a modern small satellite development base has resulted.

  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)

    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. New developments in small reactors

    International Nuclear Information System (INIS)

    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

  19. Quality Control in Small Groups

    Science.gov (United States)

    Lemmens, L. F.

    2008-11-01

    The smallness of some groups in a set up to control the quality of a service using questionnaires limits the size of the samples, this limitation has several consequences. Indeed the common approach used for relatively large groups, based on the central limit theorem and the law of large numbers, cannot be used anymore to construct estimators for the parameters of the model. Using an inverse probability will lift these restrictions. A questionnaire is a collection of items. In an item the respondent indicates on a Likert scale his or her agreement with a statement. Dimensions are a set of items dealing with one aspect of the service. In a questionnaire several dimensions are addressed but usually the items are presented in a random sequence. The model for an item is hierarchical with following components: a multivariate hypergeometric model takes the sampling in a finite population into account, the multinomial serves as a prior for the sampling and the Dirichlet-distribution serves as a prior for the multinomials. The composition of dimensions allows to use the posterior for one of the items as a prior for another item of that dimension and so on. After analysis of several questionnaires using this model, the reliability of the responses from some respondents turned out to be a key-problem, in the sense the responses can be classified into at least two classes and a decision rule had to be developed to neglect some of them. The influence of rejecting some answers, on the confidence for the most plausible statement can be estimated. This leads often to the result that there is only minimal evidence for the most probable statement.

  20. What is cerebral small vessel disease?

    International Nuclear Information System (INIS)

    An accumulating amount of evidence suggests that the white matter hyperintensities on T2 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)