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Sample records for paraplegia spg13 compromises

  1. Lysosomal abnormalities in hereditary spastic paraplegia types SPG15 and SPG11

    Science.gov (United States)

    Renvoisé, Benoît; Chang, Jaerak; Singh, Rajat; Yonekawa, Sayuri; FitzGibbon, Edmond J; Mankodi, Ami; Vanderver, Adeline; Schindler, Alice B; Toro, Camilo; Gahl, William A; Mahuran, Don J; Blackstone, Craig; Pierson, Tyler Mark

    2014-01-01

    Objective Hereditary spastic paraplegias (HSPs) are among the most genetically diverse inherited neurological disorders, with over 70 disease loci identified (SPG1-71) to date. SPG15 and SPG11 are clinically similar, autosomal recessive disorders characterized by progressive spastic paraplegia along with thin corpus callosum, white matter abnormalities, cognitive impairment, and ophthalmologic abnormalities. Furthermore, both have been linked to early-onset parkinsonism. Methods We describe two new cases of SPG15 and investigate cellular changes in SPG15 and SPG11 patient-derived fibroblasts, seeking to identify shared pathogenic themes. Cells were evaluated for any abnormalities in cell division, DNA repair, endoplasmic reticulum, endosomes, and lysosomes. Results Fibroblasts prepared from patients with SPG15 have selective enlargement of LAMP1-positive structures, and they consistently exhibited abnormal lysosomal storage by electron microscopy. A similar enlargement of LAMP1-positive structures was also observed in cells from multiple SPG11 patients, though prominent abnormal lysosomal storage was not evident. The stabilities of the SPG15 protein spastizin/ZFYVE26 and the SPG11 protein spatacsin were interdependent. Interpretation Emerging studies implicating these two proteins in interactions with the late endosomal/lysosomal adaptor protein complex AP-5 are consistent with shared abnormalities in lysosomes, supporting a converging mechanism for these two disorders. Recent work with Zfyve26−/− mice revealed a similar phenotype to human SPG15, and cells in these mice had endolysosomal abnormalities. SPG15 and SPG11 are particularly notable among HSPs because they can also present with juvenile parkinsonism, and this lysosomal trafficking or storage defect may be relevant for other forms of parkinsonism associated with lysosomal dysfunction. PMID:24999486

  2. SPG20 mutation in three siblings with familial hereditary spastic paraplegia.

    Science.gov (United States)

    Dardour, Leila; Roelens, Filip; Race, Valerie; Souche, Erika; Holvoet, Maureen; Devriendt, Koen

    2017-07-01

    Troyer syndrome (MIM#275900) is an autosomal recessive form of complicated hereditary spastic paraplegia. It is characterized by progressive lower extremity spasticity and weakness, dysarthria, distal amyotrophy, developmental delay, short stature, and subtle skeletal abnormalities. It is caused by deleterious mutations in the SPG20 gene, encoding spartin, on Chromosome 13q13. Until now, six unrelated families with a genetically confirmed diagnosis have been reported. Here we report the clinical findings in three brothers of a consanguineous Moroccan family, aged 24, 17, and 7 yr old, with spastic paraplegia, short stature, motor and cognitive delay, and severe intellectual disability. Targeted exon capture and sequencing showed a homozygous nonsense mutation in the SPG20 gene, c.1369C>T (p.Arg457*), in the three affected boys. © 2017 Dardour et al.; Published by Cold Spring Harbor Laboratory Press.

  3. Motor activation in SPG4-linked hereditary spastic paraplegia

    DEFF Research Database (Denmark)

    Scheuer, KH; Nielsen, JE; Krabbe, Katja

    2006-01-01

    OBJECTIVE: The aim of this study was to investigate the extent of motor cortical functional reorganisation in patients with SPG4-linked hereditary spastic paraplegia by exploring cortical motor activation related to movements of clinically affected (lower) and unaffected (upper) limbs. METHODS: T...

  4. Non-motor Symptoms In Patients With Hereditary Spastic Paraplegia Caused By Spg4 Mutations.

    OpenAIRE

    Servelhere, K R; Faber, I; Saute, J A M; Moscovich, M; D'Abreu, A; Jardim, L B; Teive, H A G; Lopes-Cendes, I; Franca, M C

    2016-01-01

    Non-motor manifestations are frequently overlooked in degenerative disorders and little is known about their frequency and clinical relevance in SPG4 hereditary spastic paraplegia (SPG4-HSP). Thirty patients with SPG4-HSP and 30 healthy controls answered the Modified Fatigue Impact Scale, Epworth Sleepiness Scale, Brief Pain Inventory and Beck Depression Inventory. Student's t test was used to compare groups and linear regression was used to assess correlations. Patients had higher fatigue sc...

  5. Modeling neurodevelopment and cortical dysfunction in SPG11-linked hereditary spastic paraplegia using human induced pluripotent stem cells

    OpenAIRE

    Mishra, Himanshu Kumar

    2016-01-01

    Hereditary spastic paraplegias (HSPs) are a heterogeneous group of inherited motor neuron diseases characterized by progressive spasticity and weakness of the lower limbs. Mutations in the Spastic Paraplegia Gene11 (SPG11), encoding spatacsin, cause the most frequent form of autosomal recessive HSP. SPG11 patients are clinically distinguishable from most other HSPs, by severe cortical atrophy and presence of a thin corpus callosum (TCC), associated with cognitive deficits. Partly due to l...

  6. Motor activation in SPG4-linked hereditary spastic paraplegia

    DEFF Research Database (Denmark)

    Scheuer, KH; Nielsen, JE; Krabbe, Katja

    2006-01-01

    OBJECTIVE: The aim of this study was to investigate the extent of motor cortical functional reorganisation in patients with SPG4-linked hereditary spastic paraplegia by exploring cortical motor activation related to movements of clinically affected (lower) and unaffected (upper) limbs. METHODS......: Thirteen patients and 13 normal controls matched for age, gender and handedness underwent O15-labelled water positron emission tomography during (1) right ankle flexion-extension, (2) right shoulder flexion-extension and (3) rest. Within-group comparisons of movement vs. rest (simple main effects......, the supplementary motor areas and the right premotor cortex compared to controls. CONCLUSIONS: Motor cortical reorganisation may explain this result, but as no significant differences were recognised in the motor response of the unaffected limb, differences in functional demands should also be considered...

  7. SPG10 is a rare cause of spastic paraplegia in European families.

    NARCIS (Netherlands)

    Schule, R.; Kremer, H.P.H.; Kassubek, J.; Auer-Grumbach, M.; Kostic, V.; Klopstock, T.; Klimpe, S.; Otto, S.; Boesch, S.; Warrenburg, B.P.C. van de; Schols, L.

    2008-01-01

    BACKGROUND: SPG10 is an autosomal dominant form of hereditary spastic paraplegia (HSP), which is caused by mutations in the neural kinesin heavy chain KIF5A gene, the neuronal motor of fast anterograde axonal transport. Only four mutations have been identified to date. OBJECTIVE: To determine the

  8. SPG10 is a rare cause of spastic paraplegia in European families

    NARCIS (Netherlands)

    Schuele, R.; Kremer, B. P. H.; Kassubek, J.; Auer-Grumbach, M.; Kostic, V.; Klopstock, T.; Klimpe, S.; Otto, S.; Boesch, S.; van de Warrenburg, B. P.; Schoels, L.

    Background: SPG10 is an autosomal dominant form of hereditary spastic paraplegia (HSP), which is caused by mutations in the neural kinesin heavy chain KIF5A gene, the neuronal motor of fast anterograde axonal transport. Only four mutations have been identified to date. Objective: To determine the

  9. Data supporting mitochondrial morphological changes by SPG13-associated HSPD1 mutants

    Directory of Open Access Journals (Sweden)

    Yuki Miyamoto

    2016-03-01

    Full Text Available The data is related to the research article entitled “Hypomyelinating leukodystrophy-associated missense mutation in HSPD1 blunts mitochondrial dynamics” [1]. In addition to hypomyelinating leukodystrophy (HLD 4 (OMIM no. 612233, it is known that spastic paraplegia (SPG 13 (OMIM no. 605280 is caused by HSPD1’s amino acid mutation. Two amino acid mutations Val-98-to-Ile (V98I and Gln-461-to-Glu (Q461E are associated with SPG13 [2]. In order to investigate the effects of HSPD1’s V98I or Q461E mutant on mitochondrial morphological changes, we transfected each of the respective mutant-encoding genes into Cos-7 cells. Either of V98I or Q461E mutant exhibited increased number of mitochondria and short length mitochondrial morphologies. Using MitoTracker dye-incorporating assay, decreased mitochondrial membrane potential was also observed in both cases. The data described here supports that SPG13-associated HSPD1 mutant participates in causing aberrant mitochondrial morphological changes with decreased activities. Keywords: SPG13, HSPD1, Mitochondrion, Morphological change

  10. Prenatal diagnosis of autosomal dominant hereditary spastic paraplegia (SPG4) using direct mutation detection

    DEFF Research Database (Denmark)

    Nielsen, Jørgen E; Koefoed, Pernille; Kjaergaard, Susanne

    2004-01-01

    OBJECTIVE: To present a report on prenatal diagnosis using direct SPG4 gene analysis in a family with autosomal dominant hereditary spastic paraplegia (AD-HSP). METHODS: Genetic linkage and haplotype analysis were previously carried out with chromosome 2p markers. DNA was obtained from affected...... individuals, the affected father, the mother, and fetal DNA from an ongoing pregnancy by chorionic villus sampling (CVS) in the first trimester. The spastin gene (SPG4) was completely sequenced. RESULTS: A novel 832insGdelAA frameshift mutation, predicted to cause loss of functional protein, was identified...... in the affected father and in the fetal DNA. CONCLUSIONS: This is the first report on direct prenatal diagnosis of chromosome 2p-linked AD-HSP (SPG4). In addition, we report a novel SPG4-combined small insertion/deletion mutation in exon 5, which may be the first SPG4 mutational hot spot....

  11. SPG3A-linked hereditary spastic paraplegia associated with cerebral glucose hypometabolism.

    Science.gov (United States)

    Terada, Tatsuhiro; Kono, Satoshi; Ouchi, Yasuomi; Yoshida, Kenichi; Hamaya, Yasushi; Kanaoka, Shigeru; Miyajima, Hiroaki

    2013-04-01

    SPG3A-linked hereditary spastic paraplegia (HSP) is a rare autosomal dominant motor disorder caused by a mutation in the SPG3A gene, and is characterized by progressive motor weakness and spasticity in the lower limbs, without any other neurological abnormalities. SPG3A-linked HSP caused by a R239C mutation has been reported to present a pure phenotype confined to impairment of the corticospinal tract. However, there is still a debate about the etiology of this motor deficit with regard to whether it is peripheral or central. We herein report two patients who were heterozygous for a R239C mutation in the SPG3A gene. Two middle-aged Japanese sisters had been suffering from a pure phenotype of HSP since their childhood. Both patients had a significant decrease in glucose metabolism in the frontal cortex medially and dorsolaterally in a [(18)F]-fluorodeoxyglucose (FDG) positron emission photography (PET) study and low scores on the Frontal Assessment Battery. A real-time PCR analysis in normal subjects showed the frontal cortex to be the major location where SPG3A mRNA is expressed. The present finding that the frontal glucose hypometabolism was associated with frontal cognitive impairment indicates that widespread neuropathology associated with mutations in the SPG3A gene may be present more centrally than previously assumed.

  12. Non-motor symptoms in patients with hereditary spastic paraplegia caused by SPG4 mutations.

    Science.gov (United States)

    Servelhere, K R; Faber, I; Saute, J A M; Moscovich, M; D'Abreu, A; Jardim, L B; Teive, H A G; Lopes-Cendes, I; Franca, M C

    2016-02-01

    Non-motor manifestations are frequently overlooked in degenerative disorders and little is known about their frequency and clinical relevance in SPG4 hereditary spastic paraplegia (SPG4-HSP). Thirty patients with SPG4-HSP and 30 healthy controls answered the Modified Fatigue Impact Scale, Epworth Sleepiness Scale, Brief Pain Inventory and Beck Depression Inventory. Student's t test was used to compare groups and linear regression was used to assess correlations. Patients had higher fatigue scores than controls (31.0 ± 16.5 vs. 14.5 ± 16.0, P = 0.002) as well as pain (3.4 ± 2.7 vs. 1.0 ± 1.6, P = 0.001) and depression (12.7 ± 8.9 vs. 4.4 ± 3.8, P depression and possibly with disease severity (P = 0.008 and 0.07, respectively). Fatigue, pain and depression are frequent and often severe manifestations in patients with SPG4-HSP. © 2016 EAN.

  13. Multigeneration family with dominant SPG30 hereditary spastic paraplegia.

    Science.gov (United States)

    Roda, Ricardo H; Schindler, Alice B; Blackstone, Craig

    2017-11-01

    Autosomal recessive KIF1A missense mutations cause hereditary spastic paraplegia (HSP) type SPG30, while recessive truncations lead to sensory and autonomic neuropathy (HSN2C) and many de novo missense mutations are associated with cognitive impairment. Here, we describe family members across three generations with pure HSP. A heterozygous p.Ser69Leu KIF1A mutation segregates with those afflicted. The same variant was previously reported in a Finnish father and son with pure HSP as well as four members of a Sicilian kindred with more intrafamilial phenotypic variability. This further validates the pathogenicity of the p.Ser69Leu mutation and suggests that it may represent a mutation hot spot.

  14. Genetic and phenotypic characterization of complex hereditary spastic paraplegia

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    Kara, Eleanna; Tucci, Arianna; Manzoni, Claudia; Lynch, David S.; Elpidorou, Marilena; Bettencourt, Conceicao; Chelban, Viorica; Manole, Andreea; Hamed, Sherifa A.; Haridy, Nourelhoda A.; Federoff, Monica; Preza, Elisavet; Hughes, Deborah; Pittman, Alan; Jaunmuktane, Zane; Brandner, Sebastian; Xiromerisiou, Georgia; Wiethoff, Sarah; Schottlaender, Lucia; Proukakis, Christos; Morris, Huw; Warner, Tom; Bhatia, Kailash P.; Korlipara, L.V. Prasad; Singleton, Andrew B.; Hardy, John; Wood, Nicholas W.; Lewis, Patrick A.

    2016-01-01

    cases, next generation sequencing was carried out revealing variants in a number of other known complex spastic paraplegia genes, including five in SPG7 (5/97), four in FA2H (also known as SPG35 ) (4/97) and two in ZFYVE26 / SPG15 . Variants were identified in genes usually associated with pure spastic paraplegia and also in the Parkinson’s disease-associated gene ATP13A2 , neuronal ceroid lipofuscinosis gene TPP1 and the hereditary motor and sensory neuropathy DNMT1 gene, highlighting the genetic heterogeneity of spastic paraplegia. No plausible genetic cause was identified in 51% of probands, likely indicating the existence of as yet unidentified genes. PMID:27217339

  15. Dementia in SPG4 hereditary spastic paraplegia: clinical, genetic, and neuropathologic evidence.

    LENUS (Irish Health Repository)

    Murphy, S

    2012-02-01

    BACKGROUND: Cognitive impairment and dementia has been reported in autosomal dominant hereditary spastic paraparesis (HSP) linked to the SPG4 locus. There has only been one postmortem examination described; not all accept that progressive cognitive decline is a feature of this disorder. OBJECTIVE: A family with SPG4-HSP known to have a deletion of exon 17 in the spastin gene (SPG4delEx17) was cognitively assessed over a 7-year period. The index family member died and a postmortem examination was performed. METHODS: Thirteen family members older than 40 years were clinically and cognitively assessed using the Cambridge Cognitive Assessment over a 7-year period. The presence of SPG4delEx17 was assessed; a neuropathologic examination of the brain of the index family member was performed. RESULTS: Cognitive decline occurred in 6 of the 13 family members and in all 4 older than 60 years. Two genetic deletions were identified: SPG4delEx17 in 12 of the 13 family members and a deletion of SPG6 (SPG6del) in 5. Eight individuals had the SPG4delEx17 deletion only; 4 had evidence of progressive cognitive impairment. Four family members had both SPG4delEx17 and SPG6del; 2 of these had cognitive impairment. One family member with the SPG6del alone had neither HSP nor cognitive impairment. The index case with both deletions died with dementia; the brain showed widespread ubiquitin positivity within the neocortex and white matter. CONCLUSION: Cognitive decline and dementia is a feature of SPG4-HSP due to a deletion of exon 17 of the spastin gene.

  16. Hereditary spastic paraplegia type 43 (SPG43) is caused by mutation in C19orf12

    Science.gov (United States)

    Landouré, Guida; Zhu, Peng-Peng; Lourenço, Charles M.; Johnson, Janel O.; Toro, Camilo; Bricceno, Katherine V.; Rinaldi, Carlo; Meilleur, Katherine G.; Sangaré, Modibo; Diallo, Oumarou; Pierson, Tyler M.; Ishiura, Hiroyuki; Tsuji, Shoji; Hein, Nichole; Fink, John K.; Stoll, Marion; Nicholson, Garth; Gonzalez, Michael; Speziani, Fiorella; Dürr, Alexandra; Stevanin, Giovanni; Biesecker, Leslie G.; Accardi, John; Landis, Dennis M. D.; Gahl, William A.; Traynor, Bryan J.; Marques, Wilson; Züchner, Stephan; Blackstone, Craig; Fischbeck, Kenneth H.; Burnett, Barrington G.

    2013-01-01

    We report here the genetic basis for a form of progressive hereditary spastic paraplegia (SPG43) previously described in two Malian sisters. Exome sequencing revealed a homozygous missense variant (c.187G>C; p.Ala63Pro) in C19orf12, a gene recently implicated in neurodegeneration with brain iron accumulation (NBIA). The same mutation was subsequently also found in a Brazilian family with features of NBIA, and we identified another NBIA patient with a three-nucleotide deletion (c.197_199del; p.Gly66del). Haplotype analysis revealed that the p.Ala63Pro mutations have a common origin, but MRI scans showed no brain iron deposition in the Malian SPG43 subjects. Heterologous expression of these SPG43 and NBIA variants resulted in similar alterations in the subcellular distribution of C19orf12. The SPG43 and NBIA variants reported here as well as the most common C19orf12 missense mutation reported in NBIA patients are found within a highly-conserved, extended hydrophobic domain in C19orf12, underscoring the functional importance of this domain. PMID:23857908

  17. In Vivo Evidence for Lysosome Depletion and Impaired Autophagic Clearance in Hereditary Spastic Paraplegia Type SPG11.

    Directory of Open Access Journals (Sweden)

    Rita-Eva Varga

    2015-08-01

    Full Text Available Hereditary spastic paraplegia (HSP is characterized by a dying back degeneration of corticospinal axons which leads to progressive weakness and spasticity of the legs. SPG11 is the most common autosomal-recessive form of HSPs and is caused by mutations in SPG11. A recent in vitro study suggested that Spatacsin, the respective gene product, is needed for the recycling of lysosomes from autolysosomes, a process known as autophagic lysosome reformation. The relevance of this observation for hereditary spastic paraplegia, however, has remained unclear. Here, we report that disruption of Spatacsin in mice indeed causes hereditary spastic paraplegia-like phenotypes with loss of cortical neurons and Purkinje cells. Degenerating neurons accumulate autofluorescent material, which stains for the lysosomal protein Lamp1 and for p62, a marker of substrate destined to be degraded by autophagy, and hence appears to be related to autolysosomes. Supporting a more generalized defect of autophagy, levels of lipidated LC3 are increased in Spatacsin knockout mouse embryonic fibrobasts (MEFs. Though distinct parameters of lysosomal function like processing of cathepsin D and lysosomal pH are preserved, lysosome numbers are reduced in knockout MEFs and the recovery of lysosomes during sustained starvation impaired consistent with a defect of autophagic lysosome reformation. Because lysosomes are reduced in cortical neurons and Purkinje cells in vivo, we propose that the decreased number of lysosomes available for fusion with autophagosomes impairs autolysosomal clearance, results in the accumulation of undegraded material and finally causes death of particularly sensitive neurons like cortical motoneurons and Purkinje cells in knockout mice.

  18. Hereditary spastic paraplegia with cerebellar ataxia: a complex phenotype associated with a new SPG4 gene mutation

    DEFF Research Database (Denmark)

    Nielsen, Jørgen Erik; Johnson, B; Koefoed, Pernille

    2004-01-01

    Complex forms of hereditary spastic paraplegia (HSP) are rare and usually transmitted in an autosomal recessive pattern. A family of four generations with autosomal dominant hereditary spastic paraplegia (AD-HSP) and a complex phenotype with variably expressed co-existing ataxia, dysarthria......, unipolar depression, epilepsy, migraine, and cognitive impairment was investigated. Genetic linkage analysis and sequencing of the SPG4 gene was performed and electrophysiologic investigations were carried out in six individuals and positron emission tomography (PET) in one patient. The disease was linked...... in those individuals who were clinically affected by a complex phenotype consisting of HSP and cerebellar ataxia. Other features noted in this kindred including epilepsy, cognitive impairment, depression, and migraine did not segregate with the HSP phenotype or mutation, and therefore the significance...

  19. Multimodal MRI-based study in patients with SPG4 mutations.

    Directory of Open Access Journals (Sweden)

    Thiago J R Rezende

    Full Text Available Mutations in the SPG4 gene (SPG4-HSP are the most frequent cause of hereditary spastic paraplegia, but the extent of the neurodegeneration related to the disease is not yet known. Therefore, our objective is to identify regions of the central nervous system damaged in patients with SPG4-HSP using a multi-modal neuroimaging approach. In addition, we aimed to identify possible clinical correlates of such damage. Eleven patients (mean age 46.0 ± 15.0 years, 8 men with molecular confirmation of hereditary spastic paraplegia, and 23 matched healthy controls (mean age 51.4 ± 14.1years, 17 men underwent MRI scans in a 3T scanner. We used 3D T1 images to perform volumetric measurements of the brain and spinal cord. We then performed tract-based spatial statistics and tractography analyses of diffusion tensor images to assess microstructural integrity of white matter tracts. Disease severity was quantified with the Spastic Paraplegia Rating Scale. Correlations were then carried out between MRI metrics and clinical data. Volumetric analyses did not identify macroscopic abnormalities in the brain of hereditary spastic paraplegia patients. In contrast, we found extensive fractional anisotropy reduction in the corticospinal tracts, cingulate gyri and splenium of the corpus callosum. Spinal cord morphometry identified atrophy without flattening in the group of patients with hereditary spastic paraplegia. Fractional anisotropy of the corpus callosum and pyramidal tracts did correlate with disease severity. Hereditary spastic paraplegia is characterized by relative sparing of the cortical mantle and remarkable damage to the distal portions of the corticospinal tracts, extending into the spinal cord.

  20. Late-onset spastic paraplegia type 10 (SPG10) family presenting with bulbar symptoms and fasciculations mimicking amyotrophic lateral sclerosis.

    Science.gov (United States)

    Kaji, Seiji; Kawarai, Toshitaka; Miyamoto, Ryosuke; Nodera, Hiroyuki; Pedace, Lucia; Orlacchio, Antonio; Izumi, Yuishin; Takahashi, Ryosuke; Kaji, Ryuji

    2016-05-15

    Pathogenic mutations in the KIF5A-SPG10 gene, encoding the kinesin HC5A, can be associated with autosomal dominant hereditary spastic paraplegia (ADHSP). It accounts for about 10% of the complicated forms of ADHSP. Peripheral neuropathy, distal upper limb amyotrophy, and cognitive decline are the most common additional clinical features. We examined a 66-year-old Japanese woman manifesting gait disturbance and spastic dysarthria for 6years with positive family history. She showed evidence of upper and lower motor neuron involvement and fasciculations, thus mimicking amyotrophic lateral sclerosis (ALS). Genetic analysis revealed a heterozygous variant in KIF5A (c.484C>T, p.Arg162Trp) in 2 symptomatic members. The mutation was also identified in 4 asymptomatic members, including 2 elderly members aged over 78years. Electromyography in the 2 symptomatic members revealed evidence of lower motor neuron involvement and fasciculation potentials in distal muscles. This report describes the first known Asian family with a KIF5A mutation and broadens the clinical and electrophysiological spectrum associated with KIF5A-SPG10 mutations. Given that our cases showed pseudobulbar palsy, fasciculation and altered penetrance, KIF5A-SPG10 might well be considered as a differential diagnosis of sporadic ALS. Copyright © 2016 Elsevier B.V. All rights reserved.

  1. Complicated spastic paraplegia in patients with AP5Z1 mutations (SPG48)

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    Hirst, Jennifer; Madeo, Marianna; Smets, Katrien; Edgar, James R.; Schols, Ludger; Li, Jun; Yarrow, Anna; Deconinck, Tine; Baets, Jonathan; Van Aken, Elisabeth; De Bleecker, Jan; Datiles, Manuel B.; Roda, Ricardo H.; Liepert, Joachim; Züchner, Stephan; Mariotti, Caterina; De Jonghe, Peter; Blackstone, Craig

    2016-01-01

    Objective: Biallelic mutations in the AP5Z1 gene encoding the AP-5 ζ subunit have been described in a small number of patients with hereditary spastic paraplegia (HSP) (SPG48); we sought to define genotype–phenotype correlations in patients with homozygous or compound heterozygous sequence variants predicted to be deleterious. Methods: We performed clinical, radiologic, and pathologic studies in 6 patients with biallelic mutations in AP5Z1. Results: In 4 of the 6 patients, there was complete loss of AP-5 ζ protein. Clinical features encompassed not only prominent spastic paraparesis but also sensory and motor neuropathy, ataxia, dystonia, myoclonus, and parkinsonism. Skin fibroblasts from affected patients tested positive for periodic acid Schiff and autofluorescent storage material, while electron microscopic analysis demonstrated lamellar storage material consistent with abnormal storage of lysosomal material. Conclusions: Our findings expand the spectrum of AP5Z1-associated neurodegenerative disorders and point to clinical and pathophysiologic overlap between autosomal recessive forms of HSP and lysosomal storage disorders. PMID:27606357

  2. Autosomal recessive spastic paraplegia (SPG30) with mild ataxia and sensory neuropathy maps to chromosome 2q37.3.

    Science.gov (United States)

    Klebe, Stephan; Azzedine, Hamid; Durr, Alexandra; Bastien, Patrick; Bouslam, Naima; Elleuch, Nizar; Forlani, Sylvie; Charon, Celine; Koenig, Michel; Melki, Judith; Brice, Alexis; Stevanin, Giovanni

    2006-06-01

    The hereditary spastic paraplegias (HSPs) are a clinically and genetically heterogeneous group of neurodegenerative diseases characterized by progressive spasticity in the lower limbs. Twenty-nine different loci (SPG) have been mapped so far, and 11 responsible genes have been identified. Clinically, one distinguishes between pure and complex HSP forms which are variably associated with numerous combinations of neurological and extra-neurological signs. Less is known about autosomal recessive forms (ARHSP) since the mapped loci have been identified often in single families and account for only a small percentage of patients. We report a new ARHSP locus (SPG30) on chromosome 2q37.3 in a consanguineous family with seven unaffected and four affected members of Algerian origin living in Eastern France with a significant multipoint lod score of 3.8. Ten other families from France (n = 4), Tunisia (n = 2), Algeria (n = 3) and the Czech Republic (n = 1) were not linked to the newly identified locus thus demonstrating further genetic heterogeneity. The phenotype of the linked family consists of spastic paraparesis and peripheral neuropathy associated with slight cerebellar signs confirmed by cerebellar atrophy on one CT scan.

  3. Hereditary spastic paraplegia and amyotrophy associated with a novel locus on chromosome 19

    Science.gov (United States)

    Meilleur, K.G.; Traoré, M.; Sangaré, M.; Britton, A.; Landouré, G.; Coulibaly, S.; Niaré, B.; Mochel, F.; La Pean, A.; Rafferty, I.; Watts, C.; Littleton-Kearney, M. T.; Blackstone, C.; Singleton, A.; Fischbeck, K.H.

    2010-01-01

    We identified a family in Mali with two sisters affected by spastic paraplegia. In addition to spasticity and weakness of the lower limbs, the patients had marked atrophy of the distal upper extremities. Homozygosity mapping using single nucleotide polymorphism arrays showed that the sisters shared a region of extended homozygosity at chromosome 19p13.11-q12 that was not shared by controls. These findings indicate a clinically and genetically distinct form of hereditary spastic paraplegia with amyotrophy, designated SPG43. PMID:20039086

  4. Genetics Home Reference: spastic paraplegia type 7

    Science.gov (United States)

    ... in the arms; speech difficulties (dysarthria); difficulty swallowing (dysphagia); involuntary movements of the eyes (nystagmus); mild hearing ... AH, Warner TT. A clinical, genetic and biochemical study of SPG7 mutations in hereditary spastic paraplegia. Brain. ...

  5. Retinal nerve fibre layer loss in hereditary spastic paraplegias is restricted to complex phenotypes

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    Wiethoff Sarah

    2012-11-01

    Full Text Available Abstract Background Reduction of retinal nerve fibre layer (RNFL thickness was shown as part of the neurodegenerative process in a range of different neurodegenerative pathologies including Alzheimer′s disease (AD, idiopathic Parkinson’s disease (PD, spinocerebellar ataxia (SCA and multiple system atrophy (MSA. To further clarify the specificity of RNFL thinning as a potential marker of neurodegenerative diseases we investigated RNFL thickness in Hereditary Spastic Paraplegia (HSP, an axonal, length-dependent neurodegenerative pathology of the upper motor neurons. Methods Spectral domain optical coherence tomography (OCT was performed in 28 HSP patients (clinically: pure HSP = 22, complicated HSP = 6; genetic subtypes: SPG4 = 13, SPG5 = 1, SPG7 = 3, genetically unclassified: 11 to quantify peripapillary RNFL thickness. Standardized examination assessed duration of disease, dependency on assistive walking aids and severity of symptoms quantified with Spastic Paraplegia Rating Scale (SPRS. Results HSP patients demonstrated no significant thinning of global RNFL (pglobal = 0.61. Subgroup analysis revealed significant reduction in temporal and temporal inferior sectors for patients with complex (p Conclusion Clinically pure HSP patients feature no significant reduction in RNFL, whereas complex phenotypes display an abnormal thinning of temporal and temporal inferior RNFL. Our data indicate that RNFL thinning does not occur unspecifically in all neurodegenerative diseases but is in HSP restricted to subtypes with multisystemic degeneration.

  6. ALS5/SPG11/ KIAA1840 mutations cause autosomal recessive axonal Charcot–Marie–Tooth disease

    Science.gov (United States)

    Montecchiani, Celeste; Pedace, Lucia; Lo Giudice, Temistocle; Casella, Antonella; Mearini, Marzia; Gaudiello, Fabrizio; Pedroso, José L.; Terracciano, Chiara; Caltagirone, Carlo; Massa, Roberto; St George-Hyslop, Peter H.; Barsottini, Orlando G. P.; Kawarai, Toshitaka

    2016-01-01

    Abstract Charcot–Marie–Tooth disease is a group of hereditary peripheral neuropathies that share clinical characteristics of progressive distal muscle weakness and atrophy, foot deformities, distal sensory loss, as well as diminished tendon reflexes. Hundreds of causative DNA changes have been found, but much of the genetic basis of the disease is still unexplained. Mutations in the ALS5/SPG11/ KIAA1840 gene are a frequent cause of autosomal recessive hereditary spastic paraplegia with thin corpus callosum and peripheral axonal neuropathy, and account for ∼40% of autosomal recessive juvenile amyotrophic lateral sclerosis. The overlap of axonal Charcot–Marie–Tooth disease with both diseases, as well as the common autosomal recessive inheritance pattern of thin corpus callosum and axonal Charcot–Marie–Tooth disease in three related patients, prompted us to analyse the ALS5/SPG11/ KIAA1840 gene in affected individuals with autosomal recessive axonal Charcot–Marie–Tooth disease. We investigated 28 unrelated families with autosomal recessive axonal Charcot–Marie–Tooth disease defined by clinical, electrophysiological, as well as pathological evaluation. Besides, we screened for all the known genes related to axonal autosomal recessive Charcot–Marie-Tooth disease (CMT2A2/HMSN2A2/ MFN2 , CMT2B1/ LMNA , CMT2B2/ MED25 , CMT2B5/ NEFL , ARCMT2F/dHMN2B/ HSPB1 , CMT2K/ GDAP1 , CMT2P/ LRSAM1 , CMT2R/ TRIM2 , CMT2S/ IGHMBP2 , CMT2T/ HSJ1 , CMTRID/ COX6A1 , ARAN-NM/ HINT and GAN/ GAN ), for the genes related to autosomal recessive hereditary spastic paraplegia with thin corpus callosum and axonal peripheral neuropathy (SPG7/ PGN , SPG15/ ZFYVE26, SPG21/ ACP33 , SPG35/ FA2H , SPG46/ GBA2 , SPG55/ C12orf65 and SPG56/ CYP2U1 ), as well as for the causative gene of peripheral neuropathy with or without agenesis of the corpus callosum ( SLC12A6 ) . Mitochondrial disorders related to Charcot–Marie–Tooth disease type 2 were also excluded by sequencing POLG and

  7. ALS5/SPG11/KIAA1840 mutations cause autosomal recessive axonal Charcot-Marie-Tooth disease.

    Science.gov (United States)

    Montecchiani, Celeste; Pedace, Lucia; Lo Giudice, Temistocle; Casella, Antonella; Mearini, Marzia; Gaudiello, Fabrizio; Pedroso, José L; Terracciano, Chiara; Caltagirone, Carlo; Massa, Roberto; St George-Hyslop, Peter H; Barsottini, Orlando G P; Kawarai, Toshitaka; Orlacchio, Antonio

    2016-01-01

    Charcot-Marie-Tooth disease is a group of hereditary peripheral neuropathies that share clinical characteristics of progressive distal muscle weakness and atrophy, foot deformities, distal sensory loss, as well as diminished tendon reflexes. Hundreds of causative DNA changes have been found, but much of the genetic basis of the disease is still unexplained. Mutations in the ALS5/SPG11/KIAA1840 gene are a frequent cause of autosomal recessive hereditary spastic paraplegia with thin corpus callosum and peripheral axonal neuropathy, and account for ∼ 40% of autosomal recessive juvenile amyotrophic lateral sclerosis. The overlap of axonal Charcot-Marie-Tooth disease with both diseases, as well as the common autosomal recessive inheritance pattern of thin corpus callosum and axonal Charcot-Marie-Tooth disease in three related patients, prompted us to analyse the ALS5/SPG11/KIAA1840 gene in affected individuals with autosomal recessive axonal Charcot-Marie-Tooth disease. We investigated 28 unrelated families with autosomal recessive axonal Charcot-Marie-Tooth disease defined by clinical, electrophysiological, as well as pathological evaluation. Besides, we screened for all the known genes related to axonal autosomal recessive Charcot-Marie-Tooth disease (CMT2A2/HMSN2A2/MFN2, CMT2B1/LMNA, CMT2B2/MED25, CMT2B5/NEFL, ARCMT2F/dHMN2B/HSPB1, CMT2K/GDAP1, CMT2P/LRSAM1, CMT2R/TRIM2, CMT2S/IGHMBP2, CMT2T/HSJ1, CMTRID/COX6A1, ARAN-NM/HINT and GAN/GAN), for the genes related to autosomal recessive hereditary spastic paraplegia with thin corpus callosum and axonal peripheral neuropathy (SPG7/PGN, SPG15/ZFYVE26, SPG21/ACP33, SPG35/FA2H, SPG46/GBA2, SPG55/C12orf65 and SPG56/CYP2U1), as well as for the causative gene of peripheral neuropathy with or without agenesis of the corpus callosum (SLC12A6). Mitochondrial disorders related to Charcot-Marie-Tooth disease type 2 were also excluded by sequencing POLG and TYMP genes. An additional locus for autosomal recessive Charcot

  8. Autosomal dominant spastic paraplegia with peripheral neuropathy maps to chr12q23-24.

    Science.gov (United States)

    Schüle, R; Bonin, M; Dürr, A; Forlani, S; Sperfeld, A D; Klimpe, S; Mueller, J C; Seibel, A; van de Warrenburg, B P; Bauer, P; Schöls, L

    2009-06-02

    Hereditary spastic paraplegias (HSP) are genetically exceedingly heterogeneous. To date, 37 genetic loci for HSP have been described (SPG1-41), among them 16 loci for autosomal dominant disease. Notwithstanding, further genetic heterogeneity is to be expected in HSP, as various HSP families do not link to any of the known HSP loci. In this study, we aimed to map the disease locus in a German family segregating autosomal dominant complicated HSP. A genome-wide linkage analysis was performed using the GeneChip Mapping 10Kv2.0 Xba Array containing 10,204 SNP markers. Suggestive loci were further analyzed by mapping of microsatellite markers. One locus on chromosome 12q23-24, termed SPG36, was confirmed by high density microsatellite fine mapping with a significant LOD score of 3.2. SPG36 is flanked by markers D12S318 and D12S79. Linkage to SPG36 was excluded in >20 additional autosomal dominant HSP families. Candidate genes were selected and sequenced. No disease-causing mutations were identified in the coding regions of ATXN2, HSPB8, IFT81, Myo1H, UBE3B, and VPS29. SPG36 is complicated by a sensory and motor neuropathy; it is therefore the eighth autosomal dominant subtype of complicated HSP. We report mapping of a new locus for autosomal dominant hereditary spastic paraplegia (HSP) (SPG36) on chromosome 12q23-24 in a German family with autosomal dominant HSP complicated by peripheral neuropathy.

  9. Hereditary spastic paraplegia with cerebellar ataxia

    DEFF Research Database (Denmark)

    Nielsen, J E; Johnsen, B; Koefoed, P

    2004-01-01

    Complex forms of hereditary spastic paraplegia (HSP) are rare and usually transmitted in an autosomal recessive pattern. A family of four generations with autosomal dominant hereditary spastic paraplegia (AD-HSP) and a complex phenotype with variably expressed co-existing ataxia, dysarthria......, unipolar depression, epilepsy, migraine, and cognitive impairment was investigated. Genetic linkage analysis and sequencing of the SPG4 gene was performed and electrophysiologic investigations were carried out in six individuals and positron emission tomography (PET) in one patient. The disease was linked...... in those individuals who were clinically affected by a complex phenotype consisting of HSP and cerebellar ataxia. Other features noted in this kindred including epilepsy, cognitive impairment, depression, and migraine did not segregate with the HSP phenotype or mutation, and therefore the significance...

  10. Co-segregation of Huntington disease and hereditary spastic paraplegia in 4 generations.

    Science.gov (United States)

    Panas, Marios; Karadima, Georgia; Kalfakis, Nikolaos; Vassilopoulos, Dimitris

    2011-07-01

    Huntington disease (HD) is an autosomal dominant neurodegenerative disease characterized by choreic hyperkinesias, cognitive decline, and psychiatric manifestations, caused by an increased number of CAG repeats in the IT15 gene on chromosome 4p16.3. Silver syndrome is a rare autosomal dominant form of complicated hereditary spastic paraplegia, characterized by lower limb spasticity in addition to amyotrophy of the small muscles of the hands. In addition to the previously identified locus SPG17 on chromosome 11q12-q14, a new locus (SPG38) on chromosome 4p16-p15 has been recently identified, a region that includes the HD gene. We present a Greek family with 5 members diagnosed with HD in 4 generations. All affected members also presented with clinical features of Silver syndrome showing severe spastic paraplegia and prominent atrophy of all small hand muscles bilaterally. None of the other family members showed features of either HD or spastic paraplegia. The reported coexistence of Silver syndrome with HD in 4 generations is not fortuitous, suggesting that these 2 distinct genetic disorders are in linkage disequilibrium. Although rare, it is reasonable to expect additional similar cases. Clinical neurologists should perhaps investigate this possibility in cases combining features of HD and involvement of the upper and lower motor neurons.

  11. Autosomal dominant spastic paraplegia with peripheral neuropathy maps to chr12q23-24.

    NARCIS (Netherlands)

    Schule, R.; Bonin, M.; Durr, A.; Forlani, S.; Sperfeld, A.D.; Klimpe, S.; Mueller, J.C.; Seibel, A.; Warrenburg, B.P.C. van de; Bauer, P.; Schols, L.

    2009-01-01

    OBJECTIVE: Hereditary spastic paraplegias (HSP) are genetically exceedingly heterogeneous. To date, 37 genetic loci for HSP have been described (SPG1-41), among them 16 loci for autosomal dominant disease. Notwithstanding, further genetic heterogeneity is to be expected in HSP, as various HSP

  12. Reduced regional cerebral blood flow in SPG4-linked hereditary spastic paraplegia

    DEFF Research Database (Denmark)

    Scheuer, Kristin H; Nielsen, Jørgen E; Krabbe, Katja

    2005-01-01

    flow (rCBF) as an indirect marker of regional neuronal activity. Eighteen SPG4 patients and 18 matched control subjects were studied. Resting state rCBF was measured using Positron Emission Tomography (PET) and the (15)O-labelled water bolus technique and relative group differences were explored using...

  13. Impaired Mitochondrial Dynamics Underlie Axonal Defects in Hereditary Spastic Paraplegias.

    Science.gov (United States)

    Denton, Kyle; Mou, Yongchao; Xu, Chong-Chong; Shah, Dhruvi; Chang, Jaerak; Blackstone, Craig; Li, Xue-Jun

    2018-05-02

    Mechanisms by which long corticospinal axons degenerate in hereditary spastic paraplegia (HSP) are largely unknown. Here, we have generated induced pluripotent stem cells (iPSCs) from patients with two autosomal recessive forms of HSP, SPG15 and SPG48, which are caused by mutations in the ZFYVE26 and AP5Z1 genes encoding proteins in the same complex, the spastizin and AP5Z1 proteins, respectively. In patient iPSC-derived telencephalic glutamatergic and midbrain dopaminergic neurons, neurite number, length and branching are significantly reduced, recapitulating disease-specific phenotypes. We analyzed mitochondrial morphology and noted a significant reduction in both mitochondrial length and their densities within axons of these HSP neurons. Mitochondrial membrane potential was also decreased, confirming functional mitochondrial defects. Notably, mdivi-1, an inhibitor of the mitochondrial fission GTPase DRP1, rescues mitochondrial morphology defects and suppresses the impairment in neurite outgrowth and late-onset apoptosis in HSP neurons. Furthermore, knockdown of these HSP genes causes similar axonal defects, also mitigated by treatment with mdivi-1. Finally, neurite outgrowth defects in SPG15 and SPG48 cortical neurons can be rescued by knocking down DRP1 directly. Thus, abnormal mitochondrial morphology caused by an imbalance of mitochondrial fission and fusion underlies specific axonal defects and serves as a potential therapeutic target for SPG15 and SPG48.

  14. Targeted disruption of the Mast syndrome gene SPG21 in mice impairs hind limb function and alters axon branching in cultured cortical neurons

    Science.gov (United States)

    Soderblom, Cynthia; Stadler, Julia; Jupille, Henri; Blackstone, Craig; Shupliakov, Oleg

    2017-01-01

    Mast syndrome (SPG21) is a childhood-onset, autosomal recessive, complicated form of hereditary spastic paraplegia (HSP) characterized by dementia, thin corpus callosum, white matter abnormalities, and cerebellar and extrapyramidal signs in addition to spastic paraparesis. A nucleotide insertion resulting in premature truncation of the SPG21 gene product maspardin underlies this disorder, likely leading to loss of protein function. In this study, we generated SPG21−/− knockout mice by homologous recombination as a possible animal model for SPG21. Though SPG21−/− mice appeared normal at birth, within several months they developed gradually progressive hind limb dysfunction. Cerebral cortical neurons cultured from SPG21−/− mice exhibited significantly more axonal branching than neurons from wild-type animals, while comprehensive neuropathological analysis of SPG21−/− mice did not reveal definitive abnormalities. Since alterations in axon branching have been seen in neurons derived from animal models of other forms of HSP as well as motor neuron diseases, this may represent a common cellular pathogenic theme. PMID:20661613

  15. Rapidly deteriorating course in Dutch hereditary spastic paraplegia type 11 patients

    Science.gov (United States)

    de Bot, Susanne T; Burggraaff, Rogier C; Herkert, Johanna C; Schelhaas, Helenius J; Post, Bart; Diekstra, Adinda; van Vliet, Reinout O; van der Knaap, Marjo S; Kamsteeg, Erik-Jan; Scheffer, Hans; van de Warrenburg, Bart P; Verschuuren-Bemelmans, Corien C; Kremer, Hubertus PH

    2013-01-01

    Although SPG11 is the most common complicated hereditary spastic paraplegia, our knowledge of the long-term prognosis and life expectancy is limited. We therefore studied the disease course of all patients with a proven SPG11 mutation as tested in our laboratory, the single Dutch laboratory providing SPG11 mutation analysis, between 1 January 2009 and 1 January 2011. We identified nine different SPG11 mutations, four of which are novel, in nine index patients. Eighteen SPG11 patients from these nine families were studied by means of a retrospective chart analysis and additional interview/examination. Ages at onset were between 4 months and 14 years; 39% started with learning difficulties rather than gait impairment. Brain magnetic resonance imaging showed a thin corpus callosum and typical periventricular white matter changes in the frontal horn region (known as the ‘ears-of the lynx'-sign) in all. Most patients became wheelchair bound after a disease duration of 1 to 2 decades. End-stage disease consisted of loss of spontaneous speech, severe dysphagia, spastic tetraplegia with peripheral nerve involvement and contractures. Several patients died of complications between ages 30 and 48 years, 3–4 decades after onset of gait impairment. Other relevant features during the disease were urinary and fecal incontinence, obesity and psychosis. Our study of 18 Dutch SPG11-patients shows the potential serious long-term consequences of SPG11 including a possibly restricted life span. PMID:23443022

  16. NIPA1 mutation in complex hereditary spastic paraplegia with epilepsy

    DEFF Research Database (Denmark)

    Svenstrup, K; Møller, R S; Christensen, J

    2011-01-01

    or signs are found. Mutations in the NIPA1 gene have been reported to cause spastic paraplegia type 6 (SPG6) in 10 families. SPG6 is a rare form of autosomal dominantly inherited HSP associated with a pure phenotype; however, in one complex SPG6 family, idiopathic generalized epilepsy (IGE) has been...... described and in addition, recurrent microdeletions at 15q11.2 including NIPA1 have been identified in patients with IGE. The purpose was to identify NIPA1 mutations in patients with pure and complex HSP. Methods: Fifty-two patients with HSP were screened for mutations in NIPA1. Results: One previously...... reported missense mutation c.316G>A, p.Gly106Arg, was identified in a complex HSP patient with spastic dysarthria, facial dystonia, atrophy of the small hand muscles, upper limb spasticity, and presumably IGE. The epilepsy co-segregated with HSP in the family. Conclusion: NIPA1 mutations were rare in our...

  17. Hereditary spastic paraplegia: LOD-score considerations for confirmation of linkage in a heterogeneous trait

    Energy Technology Data Exchange (ETDEWEB)

    Dube, M.P.; Kibar, Z.; Rouleau, G.A. [McGill Univ., Quebec (Canada)] [and others

    1997-03-01

    Hereditary spastic paraplegia (HSP) is a degenerative disorder of the motor system, defined by progressive weakness and spasticity of the lower limbs. HSP may be inherited as an autosomal dominant (AD), autosomal recessive, or an X-linked trait. AD HSP is genetically heterogeneous, and three loci have been identified so far: SPG3 maps to chromosome 14q, SPG4 to 2p, and SPG4a to 15q. We have undertaken linkage analysis with 21 uncomplicated AD families to the three AD HSP loci. We report significant linkage for three of our families to the SPG4 locus and exclude several families by multipoint linkage. We used linkage information from several different research teams to evaluate the statistical probability of linkage to the SPG4 locus for uncomplicated AD HSP families and established the critical LOD-score value necessary for confirmation of linkage to the SPG4 locus from Bayesian statistics. In addition, we calculated the empirical P-values for the LOD scores obtained with all families with computer simulation methods. Power to detect significant linkage, as well as type I error probabilities, were evaluated. This combined analytical approach permitted conclusive linkage analyses on small to medium-size families, under the restrictions of genetic heterogeneity. 19 refs., 1 fig., 1 tab.

  18. Hereditary spastic paraplegia: LOD-score considerations for confirmation of linkage in a heterogeneous trait.

    Science.gov (United States)

    Dubé, M P; Mlodzienski, M A; Kibar, Z; Farlow, M R; Ebers, G; Harper, P; Kolodny, E H; Rouleau, G A; Figlewicz, D A

    1997-03-01

    Hereditary spastic paraplegia (HSP) is a degenerative disorder of the motor system, defined by progressive weakness and spasticity of the lower limbs. HSP may be inherited as an autosomal dominant (AD), autosomal recessive, or an X-linked trait. AD HSP is genetically heterogeneous, and three loci have been identified so far: SPG3 maps to chromosome 14q, SPG4 to 2p, and SPG4a to 15q. We have undertaken linkage analysis with 21 uncomplicated AD families to the three AD HSP loci. We report significant linkage for three of our families to the SPG4 locus and exclude several families by multipoint linkage. We used linkage information from several different research teams to evaluate the statistical probability of linkage to the SPG4 locus for uncomplicated AD HSP families and established the critical LOD-score value necessary for confirmation of linkage to the SPG4 locus from Bayesian statistics. In addition, we calculated the empirical P-values for the LOD scores obtained with all families with computer simulation methods. Power to detect significant linkage, as well as type I error probabilities, were evaluated. This combined analytical approach permitted conclusive linkage analyses on small to medium-size families, under the restrictions of genetic heterogeneity.

  19. The Troyer syndrome (SPG20) protein spartin interacts with Eps15

    International Nuclear Information System (INIS)

    Bakowska, Joanna C.; Jenkins, Russell; Pendleton, James; Blackstone, Craig

    2005-01-01

    The hereditary spastic paraplegias comprise a group of inherited neurological disorders in which the primary manifestation is spastic weakness of the lower extremities. Troyer syndrome is an autosomal recessive form of spastic paraplegia caused by a frameshift mutation in the spartin (SPG20) gene. Currently, neither the localization nor the functions of the spartin protein are known. In this study, we generated anti-spartin antibodies and found that spartin is both cytosolic and membrane-associated. Using a yeast two-hybrid approach, we screened an adult human brain library for binding partners of spartin. We identified Eps15, a protein known to be involved in endocytosis and the control of cell proliferation. This interaction was confirmed by fusion protein 'pull-down' experiments as well as a cellular redistribution assay. Our results suggest that spartin might be involved in endocytosis, vesicle trafficking, or mitogenic activity, and that impairment in one of these processes may underlie the long axonopathy in patients with Troyer syndrome

  20. Identification of Novel Mutations in Spatacsin and Apolipoprotein B Genes in a Patient with Spastic Paraplegia and Hypobetalipoproteinemia

    Directory of Open Access Journals (Sweden)

    Leema Reddy Peddareddygari

    2015-01-01

    Full Text Available Complicated hereditary spastic paraplegia (HSP presents with complex neurological and nonneurological manifestations. We report a patient with autosomal recessive (AR HSP in whom laboratory investigations revealed hypobetalipoproteinemia raising the possibility of a shared pathophysiology of these clinical features. A lipid profile of his parents disclosed a normal maternal lipid profile. However, the paternal lipid profile was similar to that of the patient suggesting autosomal dominant transmission of this trait. Whole exome sequence analysis was performed and novel mutations were detected in both the SPG11 and the APOB genes. Genetic testing of the parents showed that both APOB variants were inherited from the father while the SPG11 variants were inherited one from each parent. Our results indicate that, in this patient, the hypobetalipoproteinemia and spastic paraplegia are unrelated resulting from mutations in two independent genes. This clinical study provides support for the use of whole exome sequencing as a diagnostic tool for identification of mutations in conditions with complex presentations.

  1. Spastic paraplegia proteins spastizin and spatacsin mediate autophagic lysosome reformation.

    Science.gov (United States)

    Chang, Jaerak; Lee, Seongju; Blackstone, Craig

    2014-12-01

    Autophagy allows cells to adapt to changes in their environment by coordinating the degradation and recycling of cellular components and organelles to maintain homeostasis. Lysosomes are organelles critical for terminating autophagy via their fusion with mature autophagosomes to generate autolysosomes that degrade autophagic materials; therefore, maintenance of the lysosomal population is essential for autophagy-dependent cellular clearance. Here, we have demonstrated that the two most common autosomal recessive hereditary spastic paraplegia gene products, the SPG15 protein spastizin and the SPG11 protein spatacsin, are pivotal for autophagic lysosome reformation (ALR), a pathway that generates new lysosomes. Lysosomal targeting of spastizin required an intact FYVE domain, which binds phosphatidylinositol 3-phosphate. Loss of spastizin or spatacsin resulted in depletion of free lysosomes, which are competent to fuse with autophagosomes, and an accumulation of autolysosomes, reflecting a failure in ALR. Moreover, spastizin and spatacsin were essential components for the initiation of lysosomal tubulation. Together, these results link dysfunction of the autophagy/lysosomal biogenesis machinery to neurodegeneration.

  2. Targeted NGS meets expert clinical characterization: Efficient diagnosis of spastic paraplegia type 11

    Directory of Open Access Journals (Sweden)

    Cristina Castro-Fernández

    2015-06-01

    Full Text Available Next generation sequencing (NGS is transforming the diagnostic approach for neurological disorders, since it allows simultaneous analysis of hundreds of genes, even based on just a broad, syndromic patient categorization. However, such an approach bears a high risk of incidental and uncertain genetic findings. We report a patient with spastic paraplegia whose comprehensive neurological and imaging examination raised a high clinical suspicion of SPG11. Thus, although our NGS pipeline for this group of disorders includes gene panel and exome sequencing, in this sample only the spatacsin gene region was captured and subsequently searched for mutations. Two probably pathogenic variants were quickly and clearly identified, confirming the diagnosis of SPG11. This case illustrates how combination of expert clinical characterization with highly oriented NGS protocols leads to a fast, cost-efficient diagnosis, minimizing the risk of findings with unclear significance.

  3. A hereditary spastic paraplegia mutation in kinesin-1A/KIF5A disrupts neurofilament transport

    Directory of Open Access Journals (Sweden)

    Brown Anthony

    2010-11-01

    Full Text Available Abstract Background Hereditary spastic paraplegias are a group of neurological disorders characterized by progressive distal degeneration of the longest ascending and descending axons in the spinal cord, leading to lower limb spasticity and weakness. One of the dominantly inherited forms of this disease (spastic gait type 10, or SPG10 is caused by point mutations in kinesin-1A (also known as KIF5A, which is thought to be an anterograde motor for neurofilaments. Results We investigated the effect of an SPG10 mutation in kinesin-1A (N256S-kinesin-1A on neurofilament transport in cultured mouse cortical neurons using live-cell fluorescent imaging. N256S-kinesin-1A decreased both anterograde and retrograde neurofilament transport flux by decreasing the frequency of anterograde and retrograde movements. Anterograde velocity was not affected, whereas retrograde velocity actually increased. Conclusions These data reveal subtle complexities to the functional interdependence of the anterograde and retrograde neurofilament motors and they also raise the possibility that anterograde and retrograde neurofilament transport may be disrupted in patients with SPG10.

  4. Identification of two novel KIF5A mutations in hereditary spastic paraplegia associated with mild peripheral neuropathy.

    Science.gov (United States)

    López, Eva; Casasnovas, Carlos; Giménez, Javier; Santamaría, Raúl; Terrazas, Jesús M; Volpini, Víctor

    2015-11-15

    Spastic paraplegia type 10 (SPG10) is a rare form of autosomal dominant hereditary spastic paraplegia (AD-HSP) due to mutations in KIF5A, a gene encoding the neuronal kinesin heavy-chain involved in axonal transport. KIF5A mutations have been associated with a wide clinical spectrum, ranging from pure HSP to isolated peripheral nerve involvement or complicated HSP phenotypes. Most KIF5A mutations are clustered in the motor domain of the protein that is necessary for microtubule interaction. Here we describe two Spanish families with an adult onset complicated AD-HSP in which neurological studies revealed a mild sensory neuropathy. Intention tremor was also present in both families. Molecular genetic analysis identified two novel mutations c.773 C>T and c.833 C>T in the KIF5A gene resulting in the P258L and P278L substitutions respectively. Both were located in the highly conserved kinesin motor domain of the protein which has previously been identified as a hot spot for KIF5A mutations. This study adds to the evidence associating the known occurrence of mild peripheral neuropathy in the adult onset SPG10 type of AD-HSP. Copyright © 2015 Elsevier B.V. All rights reserved.

  5. Loss of spastin function results in disease-specific axonal defects in human pluripotent stem cell-based models of hereditary spastic paraplegia

    Science.gov (United States)

    Denton, Kyle R.; Lei, Ling; Grenier, Jeremy; Rodionov, Vladimir; Blackstone, Craig; Li, Xue-Jun

    2013-01-01

    Human neuronal models of hereditary spastic paraplegias (HSP) that recapitulate disease-specific axonal pathology hold the key to understanding why certain axons degenerate in patients and to developing therapies. SPG4, the most common form of HSP, is caused by autosomal dominant mutations in the SPAST gene, which encodes the microtubule-severing ATPase spastin. Here, we have generated a human neuronal model of SPG4 by establishing induced pluripotent stem cells (iPSCs) from an SPG4 patient and differentiating these cells into telencephalic glutamatergic neurons. The SPG4 neurons displayed a significant increase in axonal swellings, which stained strongly for mitochondria and tau, indicating the accumulation of axonal transport cargoes. In addition, mitochondrial transport was decreased in SPG4 neurons, revealing that these patient iPSC-derived neurons recapitulate disease-specific axonal phenotypes. Interestingly, spastin protein levels were significantly decreased in SPG4 neurons, supporting a haploinsufficiency mechanism. Furthermore, cortical neurons derived from spastin-knockdown human embryonic stem cells (hESCs) exhibited similar axonal swellings, confirming that the axonal defects can be caused by loss of spastin function. These spastin-knockdown hESCs serve as an additional model for studying HSP. Finally, levels of stabilized acetylated-tubulin were significantly increased in SPG4 neurons. Vinblastine, a microtubule-destabilizing drug, rescued this axonal swelling phenotype in neurons derived from both SPG4 iPSCs and spastin-knockdown hESCs. Thus, this study demonstrates the successful establishment of human pluripotent stem cell-based neuronal models of SPG4, which will be valuable for dissecting the pathogenic cellular mechanisms and screening compounds to rescue the axonal degeneration in HSP. PMID:24123785

  6. Interaction of the SPG21 protein ACP33/maspardin with the aldehyde dehydrogenase ALDH16A1

    Science.gov (United States)

    2017-01-01

    Mast syndrome (SPG21) is an autosomal-recessive complicated form of hereditary spastic paraplegia characterized by dementia, thin corpus callosum, white matter abnormalities, and cerebellar and extrapyramidal signs in addition to spastic paraparesis. A nucleotide insertion resulting in premature truncation of the SPG21 gene product acidic cluster protein 33 (ACP33)/maspardin underlies this disorder, likely causing loss of protein function. However, little is known about the function of maspardin. Here, we report that maspardin localizes prominently to cytoplasm as well as to membranes, possibly at trans-Golgi network/late endosomal compartments. Immunoprecipitation of maspardin with identification of coprecipitating proteins by mass spectrometry revealed the aldehyde dehydrogenase ALDH16A1 as an interacting protein. This interaction was confirmed using overexpressed proteins as well as by fusion protein pull down experiments, and these proteins colocalized in cells. Further studies of the function of ALDH16A1 and the role of the maspardin–ALDH16A1 interaction in neuronal cells may clarify the cellular pathogenesis of Mast syndrome. PMID:19184135

  7. 6 CFR 13.46 - Compromise or settlement.

    Science.gov (United States)

    2010-01-01

    ... 6 Domestic Security 1 2010-01-01 2010-01-01 false Compromise or settlement. 13.46 Section 13.46 Domestic Security DEPARTMENT OF HOMELAND SECURITY, OFFICE OF THE SECRETARY PROGRAM FRAUD CIVIL REMEDIES § 13.46 Compromise or settlement. (a) Parties may Make offers of compromise or settlement at any time...

  8. A hereditary spastic paraplegia mouse model supports a role of ZFYVE26/SPASTIZIN for the endolysosomal system.

    Directory of Open Access Journals (Sweden)

    Mukhran Khundadze

    Full Text Available Hereditary spastic paraplegias (HSPs are characterized by progressive weakness and spasticity of the legs because of the degeneration of cortical motoneuron axons. SPG15 is a recessively inherited HSP variant caused by mutations in the ZFYVE26 gene and is additionally characterized by cerebellar ataxia, mental decline, and progressive thinning of the corpus callosum. ZFYVE26 encodes the FYVE domain-containing protein ZFYVE26/SPASTIZIN, which has been suggested to be associated with the newly discovered adaptor protein 5 (AP5 complex. We show that Zfyve26 is broadly expressed in neurons, associates with intracellular vesicles immunopositive for the early endosomal marker EEA1, and co-fractionates with a component of the AP5 complex. As the function of ZFYVE26 in neurons was largely unknown, we disrupted Zfyve26 in mice. Zfyve26 knockout mice do not show developmental defects but develop late-onset spastic paraplegia with cerebellar ataxia confirming that SPG15 is caused by ZFYVE26 deficiency. The morphological analysis reveals axon degeneration and progressive loss of both cortical motoneurons and Purkinje cells in the cerebellum. Importantly, neuron loss is preceded by accumulation of large intraneuronal deposits of membrane-surrounded material, which co-stains with the lysosomal marker Lamp1. A density gradient analysis of brain lysates shows an increase of Lamp1-positive membrane compartments with higher densities in Zfyve26 knockout mice. Increased levels of lysosomal enzymes in brains of aged knockout mice further support an alteration of the lysosomal compartment upon disruption of Zfyve26. We propose that SPG15 is caused by an endolysosomal membrane trafficking defect, which results in endolysosomal dysfunction. This appears to be particularly relevant in neurons with highly specialized neurites such as cortical motoneurons and Purkinje cells.

  9. A novel mutation in KIF5A in a Malian family with spastic paraplegia and sensory loss.

    Science.gov (United States)

    Guinto, Cheick O; Diarra, Salimata; Diallo, Salimata; Cissé, Lassana; Coulibaly, Thomas; Diallo, Seybou H; Taméga, Abdoulaye; Chen, Ke-Lian; Schindler, Alice B; Bagayoko, Koumba; Simaga, Assiatou; Blackstone, Craig; Fischbeck, Kenneth H; Landouré, Guida

    2017-04-01

    Hereditary spastic paraplegias (HSPs) are well-characterized disorders but rarely reported in Africa. We evaluated a Malian family in which three individuals had HSP and distal muscle atrophy and sensory loss. HSP panel testing identified a novel heterozygous missense mutation in KIF5A (c.1086G>C, p.Lys362Asn) that segregated with the disease (SPG10). Lys362 is highly conserved across species and Lys362Asn is predicted to be damaging. This study shows that HSPs are present in sub-Saharan Africa, although likely underdiagnosed. Increasing efficiency and decreasing costs of DNA sequencing will make it more feasible to diagnose HSPs in developing countries.

  10. Immunological effects of Sizofiran (SPG) combined with irradiation in patients with oral cancer

    International Nuclear Information System (INIS)

    Tominaga, Kazuhiro; Tominaga, Naohiro; Tokuhisa, Michio

    1995-01-01

    Effects of Sizofiran (SPG) combined with irradiation for primary oral squamous cell carcinoma were evaluated with regard to cell-mediated immunity. Twenty-four patients, who were managed with irradiation from 1989 to 1993, were divided into two groups, a SPG group and a control group. Thirteen patients received concomitant administration of SPG intramuscularly with irradiation as SPG group, and 11 patients were not administered any biological response modifiers as the control group. In the SPG group, depression of blastoid transformation of lymphocytes, IL-2 production and LAK activity due to irradiation were prevented, and recovery of blastoid transformation was promoted, while peripheral monocytes tended to increase. Upon comparison for survival (Kaplan-Meier method) between the SPG group and control group, the survival rate of the SPG group tended to be superior to that of the control group. However, no significant difference was observed between them. (author)

  11. Analisis Pengaruh Gaya Kepemimpinan terhadap Kinerja SPG PD. Sumber Jaya

    Directory of Open Access Journals (Sweden)

    Teguh Sriwidadi

    2011-05-01

    Full Text Available PD Sumber Jaya is a company engaged in the leather goods and accessories retailer that sells Braun Buffel brand which has several stores in Jakarta, Surabaya, and also a number of outlets throughout Indonesia. In each outlet is guarded by several Sales Promotion Girls (SPG. The purpose of this study is to analyze the influence of leadership style on the performance of SPG PD. Sumber Jaya. The method of analysis used in this study is Test Validity, Test Reliability, Mean, Pearson Correlation, and Regression . Data obtained from SPG assessment of managers by filling out the questionnaire that has been provided by using a Likert scale. The results achieved in this research is the leadership style which is run by managers PD. Sumber Jaya is a type of democratic and individual leadership styles have a strong influence of (0.195 or 19.50% and the direction (+ on the performance of SPG PD. Sumber Jaya.Leadership style has a fairly strong relationship (ryx1 = 0.442 on the performance of SPG

  12. Hereditary spastic paraplegia in Greece: characterisation of a previously unexplored population using next-generation sequencing.

    Science.gov (United States)

    Lynch, David S; Koutsis, Georgios; Tucci, Arianna; Panas, Marios; Baklou, Markella; Breza, Marianthi; Karadima, Georgia; Houlden, Henry

    2016-06-01

    Hereditary Spastic Paraplegia (HSP) is a syndrome characterised by lower limb spasticity, occurring alone or in association with other neurological manifestations, such as cognitive impairment, seizures, ataxia or neuropathy. HSP occurs worldwide, with different populations having different frequencies of causative genes. The Greek population has not yet been characterised. The purpose of this study was to describe the clinical presentation and molecular epidemiology of the largest cohort of HSP in Greece, comprising 54 patients from 40 families. We used a targeted next-generation sequencing (NGS) approach to genetically assess a proband from each family. We made a genetic diagnosis in >50% of cases and identified 11 novel variants. Variants in SPAST and KIF5A were the most common causes of autosomal dominant HSP, whereas SPG11 and CYP7B1 were the most common cause of autosomal recessive HSP. We identified a novel variant in SPG11, which led to disease with later onset and may be unique to the Greek population and report the first nonsense mutation in KIF5A. Interestingly, the frequency of HSP mutations in the Greek population, which is relatively isolated, was very similar to other European populations. We confirm that NGS approaches are an efficient diagnostic tool and should be employed early in the assessment of HSP patients.

  13. CYP7B1

    DEFF Research Database (Denmark)

    Roos, P; Svenstrup, K; Danielsen, E R

    2014-01-01

    UNLABELLED: The SPG5A subtype of Hereditary Spastic Paraplegia (HSP) is a rare autosomal recessive neurodegenerative disorder caused by mutations in the CYP7B1 gene, which encodes a steroid cytochrome P450 7α-hydroxylase. This enzyme provides the primary metabolic route for neurosteroids. Clinica......UNLABELLED: The SPG5A subtype of Hereditary Spastic Paraplegia (HSP) is a rare autosomal recessive neurodegenerative disorder caused by mutations in the CYP7B1 gene, which encodes a steroid cytochrome P450 7α-hydroxylase. This enzyme provides the primary metabolic route for neurosteroids.......945_947 dupGGC p.A316AA). CONCLUSION: SPG5A could be characterized as a predominantly pure HSP. MRS showing elevated mI/Cr ratio in the white matter may be indicative of SPG5A....

  14. NEUROTRANSMITTER ABNORMALITIES AND RESPONSE TO SUPPLEMENTATION IN SPG11

    Science.gov (United States)

    Vanderver, Adeline; Tonduti, Davide; Auerbach, Sarah; Schmidt, Johanna L.; Parikh, Sumit; Gowans, Gordon C.; Jackson, Kelly E.; Brock, Pamela L.; Patterson, Marc; Nehrebecky, Michelle; Godfrey, Rena; Zein, Wadih M.; Gahl, William; Toro, Camilo

    2012-01-01

    Objective To report the detection of secondary neurotransmitter abnormalities in a group of SPG11 patients and describe treatment with L-dopa/carbidopa and sapropterin. Design Case reports Setting National Institutes of Health in the context of the Undiagnosed Disease Program; Children’s National Medical Center in the context of Myelin Disorders Bioregistry Program Patients Four SPG11 patients with a clinical picture of progressive spastic paraparesis complicated by extrapyramidal symptoms and maculopathy Interventions L-dopa/carbidopa and sapropterin Results 3/4 patients presented secondary neurotransmitter abnormalities; 4/4 partially responded to L-dopa as well as sapropterin Conclusions In the SPG11 patient with extrapyramidal symptoms, a trial of L-dopa/carbidopa and sapropterin and/or evaluation of cerebrospinal fluid neurotransmitters should be considered. PMID:22749184

  15. The prevalence of depression in hereditary spastic paraplegia.

    Science.gov (United States)

    Vahter, L; Braschinsky, M; Haldre, S; Gross-Paju, K

    2009-09-01

    To evaluate the prevalence of depression and sensitivity and specificity of the single-item interview 'Are you depressed?' for people with hereditary spastic paraplegia in Estonia. Single-item interview 'Are you depressed?' was used as a screening question for depression; all participants then completed the Beck Depression Inventory. People with hereditary spastic paraplegia identified from the epidemiological database who agreed to participate in the study. Beck Depression Inventory, clinical interview. The epidemiological database consisted of 59 patients with clinically confirmed diagnosis of hereditary spastic paraplegia. Forty-eight of these consented to participate in the study. The Beck Depression Inventory score was higher than cut-off point in 58% (28/48) and lower in 42% (20/48). Of the study group, 44% (21/48) had mild, 13% (6/48) moderate and one person revealed severe depression. There was a statistically significant correlation between Beck Depression Inventory score and level of mobility; no other significant correlations with other measures were detected. Of the participants, 54% (26/48) had subjective complaints about depression and answered 'Yes' to the single-item interview 'Are you depressed?'. The sensitivity of the one-item interview in the hereditary spastic paraplegia group was 75% and specificity 75%. Our results show that mild depression is prevalent among people with hereditary spastic paraplegia. Although the single question may be helpful, it cannot be relied upon entirely when assessing a person for depression.

  16. "SP-G", a putative new surfactant protein--tissue localization and 3D structure.

    Directory of Open Access Journals (Sweden)

    Felix Rausch

    Full Text Available Surfactant proteins (SP are well known from human lung. These proteins assist the formation of a monolayer of surface-active phospholipids at the liquid-air interface of the alveolar lining, play a major role in lowering the surface tension of interfaces, and have functions in innate and adaptive immune defense. During recent years it became obvious that SPs are also part of other tissues and fluids such as tear fluid, gingiva, saliva, the nasolacrimal system, and kidney. Recently, a putative new surfactant protein (SFTA2 or SP-G was identified, which has no sequence or structural identity to the already know surfactant proteins. In this work, computational chemistry and molecular-biological methods were combined to localize and characterize SP-G. With the help of a protein structure model, specific antibodies were obtained which allowed the detection of SP-G not only on mRNA but also on protein level. The localization of this protein in different human tissues, sequence based prediction tools for posttranslational modifications and molecular dynamic simulations reveal that SP-G has physicochemical properties similar to the already known surfactant proteins B and C. This includes also the possibility of interactions with lipid systems and with that, a potential surface-regulatory feature of SP-G. In conclusion, the results indicate SP-G as a new surfactant protein which represents an until now unknown surfactant protein class.

  17. Unexplained spastIc paraplegia

    African Journals Online (AJOL)

    Of 330 Black patients admitted to hospital with paraplegia, the ... During the period of this study (January 1979 - May 1982) 330 patients were admitted to medical, surgical and orthopaedic. TABLE I. FINAL ... paraplegia of uncertain aetiology' seen at the ational Hospital,. Queen Square ... levels were all within normal limits.

  18. GAMBARAN RISIKO TROMBOSIS VENA PROFUNDA (TVP PADA SALES PROMOTION GIRL (SPG BERDASARKAN KRITERIA WELLS DI KOTA DENPASAR

    Directory of Open Access Journals (Sweden)

    Shelly Silvia Bintang

    2015-01-01

    Full Text Available Pekerjaan sebagai sales promotion girl (SPG menuntut berdiri dalam posisi statis dalam waktu kerja yang cukup lama. Dalam sebuah jurnal kesehatan masyarakat tahun 2013 mendapatkan hasil semakin lama SPG bekerja maka semakin lama juga durasi mereka memakai sepatu hak tinggi semakin besar risiko untuk mengalami gangguan kesehatan yang disebabkan sepatu hak tinggi. Gangguan dari aliran darah atau trombosis vena profunda (TVP merupakan dampak yang ditimbulkan. Tujuan dari penelitian ini untuk mengetahui angka tinggi pemakaian sepatu hak tinggi, angka pemakaian stocking dan gambaran SPG di Kota Denpasar yang memiliki risiko rendah, menengah dan tinggi mengalami TVP. Penelitian ini menggunakan metode deskriptif potong lintang. Subyek penelitian ini adalah 62 pegawai SPG yang bekerja di swalayan di Kota Denpasar. Skor kriteria Wells didapatkan untuk mengetahui gambaran risiko probabilitas TVP. Hasil gambaran risiko TVP pada SPG di Kota Denpasar berdasarkan kriteria Wells didapatkan 30 orang pekerja (48,38% menunjukan risiko ringan, dan 30 orang pekerja lainnya (48,38% menunjukan risiko menengah menderita TVP, sedangkan 2 orang pekerja (3,24% menunjukan risiko tinggi. Sebagian besar pegawai SPG di kota Denpasar memiliki risiko rendah dan sedang probabilitas TVP.

  19. Association of Early-Onset Spasticity and Risk for Cognitive Impairment With Mutations at Amino Acid 499 in SPAST.

    Science.gov (United States)

    Gillespie, Meredith K; Humphreys, Peter; McMillan, Hugh J; Boycott, Kym M

    2018-04-01

    Hereditary spastic paraplegia is a phenotypically and genetically heterogeneous group of neurodegenerative disorders characterized by lower extremity weakness and spasticity. Spastic paraplegia 4 (SPG4), caused by heterozygous mutations in the gene SPAST, typically causes a late-onset, uncomplicated form of hereditary spastic paraplegia in affected individuals. Additional clinical features in SPG4 have been reported on occasion, but no genotype-phenotype correlation has been established. Through targeted clinical testing, we identified 2 unrelated female patients with the same de novo p.Arg499His mutation in SPAST. Both patients presented with early-onset spasticity resulting in delayed motor milestones, which led to a diagnosis of cerebral palsy in one child and tethered cord in the other. Review of the literature identified several patients with mutations at amino acid 499 and early-onset symptoms associated with a risk of cognitive impairment. Early and accurate diagnosis of children with early-onset spasticity is important for informed prognosis and genetic counselling.

  20. Autosomal dominant hereditary spastic paraplegia with axonal sensory motor polyneuropathy maps to chromosome 21q 22.3.

    Science.gov (United States)

    Peddareddygari, Leema Reddy; Hanna, Philip A; Igo, Robert P; Luo, Yuqun A; Won, Sungho; Hirano, Michio; Grewal, Raji P

    2016-01-01

    Hereditary spastic paraplegia (HSP) are a genetically and clinically heterogeneous group of disorders. At present, 19 autosomal dominant loci for HSP have been mapped. We ascertained an American family of European descent segregating an autosomal dominant HSP associated with peripheral neuropathy. A genome wide scan was performed with 410 microsatellite repeat marker (Weber lab screening set 16) and following linkage and haplotype analysis, fine mapping was performed. Established genes or loci for HSP were excluded by direct sequencing or haplotype analysis. All established loci for HSP were excluded. Fine mapping suggested a locus on chromosome 21q22.3 flanked by markers D21S1411 and D21S1446 with a maximum logarithm of odds score of 2.05 and was supported by haplotype analysis. A number of candidate genes in this region were analyzed and no disease-producing mutations were detected. We present the clinical and genetic analysis of an American family with autosomal dominant HSP with axonal sensory motor polyneuropathy mapping to a novel locus on chromosome 21q22.3 designated SPG56.

  1. Analysis of miRNA expression profiles in melatonin-exposed GC-1 spg cell line.

    Science.gov (United States)

    Zhu, Xiaoling; Chen, Shuxiong; Jiang, Yanwen; Xu, Ying; Zhao, Yun; Chen, Lu; Li, Chunjin; Zhou, Xu

    2018-02-05

    Melatonin is an endocrine neurohormone secreted by pinealocytes in the pineal gland. It exerts diverse physiological effects, such as circadian rhythm regulator and antioxidant. However, the functional importance of melatonin in spermatogenesis regulation remains unclear. The objectives of this study are to: (1) detect melatonin affection on miRNA expression profiles in GC-1 spg cells by miRNA deep sequencing (DeepSeq) and (2) define melatonin affected miRNA-mRNA interactions and associated biological processes using bioinformatics analysis. GC-1 spg cells were cultured with melatonin (10 -7 M) for 24h. DeepSeq data were validated using quantitative real-time reverse transcription polymerase chain reaction analysis (qRT-PCR). A total of 176 miRNA expressions were found to be significantly different between two groups (fold change of >2 or melatonin could regulate the expression of miRNA to perform its physiological effects in GC-1 spg cells. These results should be useful to investigate the biological function of miRNAs regulated by melatonin in spermatogenesis and testicular germ cell tumor. Copyright © 2017 Elsevier B.V. All rights reserved.

  2. SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes

    Directory of Open Access Journals (Sweden)

    Naif A.M. Almontashiri

    2014-05-01

    Full Text Available Mitochondrial production of reactive oxygen species (ROS affects many processes in health and disease. SPG7 assembles with AFG3L2 into the mAAA protease at the inner membrane of mitochondria, degrades damaged proteins, and regulates the synthesis of mitochondrial ribosomes. SPG7 is cleaved and activated by AFG3L2 upon assembly. A variant in SPG7 that replaces arginine 688 with glutamine (Q688 is associated with several phenotypes, including toxicity of chemotherapeutic agents, type 2 diabetes mellitus, and (as reported here coronary artery disease. We demonstrate that SPG7 processing is regulated by tyrosine phosphorylation of AFG3L2. Carriers of Q688 bypass this regulation and constitutively process and activate SPG7 mAAA protease. Cells expressing Q688 produce higher ATP levels and ROS, promoting cell proliferation. Our results thus reveal an unexpected link between the phosphorylation-dependent regulation of the mitochondria mAAA protease affecting ROS production and several clinical phenotypes.

  3. Recent advances on acute paraplegia

    Directory of Open Access Journals (Sweden)

    Gentian Vyshka

    2016-11-01

    Full Text Available Paraplegia and spinal cord injuries are issues of major concern to the actual medicine. But recent advances have raised hopes for a better prognosis, which has always been poor or infaust since medicine was practiced. However and interestingly enough, some concepts and definitions on the occurrence have survived for millennia. Once encountered as an issue, traumatic or non-traumatic paraplegia needs a multidisciplinary approach and a careful staging of the problem. Different scales are available with Frankel's and American Spinal Injury Association most widely used as alternatives or complementary tools. The authors discuss therapeutic options with a special focus on the stem cell therapy which has seen an impressive increase on the number of trials for a successful treatment. Sourcing and yielding of stem cells are made possible through a number of techniques, with material aspirated from bone marrow or adipose tissue, which are used along with other sources of neuronal precursors such as olfactory ensheathing cells. Nevertheless, large and multicenter studies are still lacking. However, with the quality of the ongoing work and research, the optimistic attitude seems warranted. Meanwhile, other rehabilitation and medical care interventions, always at hand, need to be applied in every individual suffering from paraplegia and spinal cord injury.

  4. Paraplegia como manifestação inicial de medula presa: relato de caso Paraplegia as initial manifestation of tethered spinal cord: case report

    Directory of Open Access Journals (Sweden)

    ANA GUARDIOLA

    1999-03-01

    Full Text Available A medula presa (MP é entidade pouco frequente que ocorre quando há restrição da migração normal do cone medular por cistos, lipomas ou disrafismos espinhais. Esta patologia pode levar a paraplegia, distúrbios sensoriais e esficterianos. Relatamos o caso de uma menina de 22 meses que consultou com quadro de paraplegia. O diagnóstico de medula presa foi confirmado pela mielotomografia e a paciente foi submetida à cirurgia para liberação do filo terminal.Tethered spinal cord (TSC is a rare disorder; it occurs when the conus medularis is anchored to the base of the vertebral canal by thickened filum terminale cysts, lipoma and spinal dysraphia. This disorder may cause paraplegia, sensory and sphincter disturbance. We report a twenty-two months-old girl presenting with paraplegia. TSC diagnostic was confirmed by myelotomography. The patient was submitted to surgical relief of tethered filum terminale.

  5. Walking dreams in congenital and acquired paraplegia.

    Science.gov (United States)

    Saurat, Marie-Thérèse; Agbakou, Maité; Attigui, Patricia; Golmard, Jean-Louis; Arnulf, Isabelle

    2011-12-01

    To test if dreams contain remote or never-experienced motor skills, we collected during 6 weeks dream reports from 15 paraplegics and 15 healthy subjects. In 9/10 subjects with spinal cord injury and in 5/5 with congenital paraplegia, voluntary leg movements were reported during dream, including feelings of walking (46%), running (8.6%), dancing (8%), standing up (6.3%), bicycling (6.3%), and practicing sports (skiing, playing basketball, swimming). Paraplegia patients experienced walking dreams (38.2%) just as often as controls (28.7%). There was no correlation between the frequency of walking dreams and the duration of paraplegia. In contrast, patients were rarely paraplegic in dreams. Subjects who had never walked or stopped walking 4-64 years prior to this study still experience walking in their dreams, suggesting that a cerebral walking program, either genetic or more probably developed via mirror neurons (activated when observing others performing an action) is reactivated during sleep. Copyright © 2011 Elsevier Inc. All rights reserved.

  6. Basedow paraplegia: A possible misnomer

    African Journals Online (AJOL)

    Thyrotoxic myopathy frequently occurs in clinical practice; however, the ... The occurrence of hyperthyroidism with a flaccid, areflexic paraplegia appears ... It is important to consider and treat other causes, such as acute idiopathic polyneuritis.

  7. Evaluation of Time-Temperature Integrators (TTIs) with Microorganism-Entrapped Microbeads Produced Using Homogenization and SPG Membrane Emulsification Techniques.

    Science.gov (United States)

    Rahman, A T M Mijanur; Lee, Seung Ju; Jung, Seung Won

    2015-12-28

    A comparative study was conducted to evaluate precision and accuracy in controlling the temperature dependence of encapsulated microbial time-temperature integrators (TTIs) developed using two different emulsification techniques. Weissela cibaria CIFP 009 cells, immobilized within 2% Na-alginate gel microbeads using homogenization (5,000, 7,000, and 10,000 rpm) and Shirasu porous glass (SPG) membrane technologies (10 μm), were applied to microbial TTIs. The prepared micobeads were characterized with respect to their size, size distribution, shape and morphology, entrapment efficiency, and bead production yield. Additionally, fermentation process parameters including growth rate were investigated. The TTI responses (changes in pH and titratable acidity (TA)) were evaluated as a function of temperature (20°C, 25°C, and 30°C). In comparison with conventional methods, SPG membrane technology was able not only to produce highly uniform, small-sized beads with the narrowest size distribution, but also the bead production yield was found to be nearly 3.0 to 4.5 times higher. However, among the TTIs produced using the homogenization technique, poor linearity (R(2)) in terms of TA was observed for the 5,000 and 7,000 rpm treatments. Consequently, microbeads produced by the SPG membrane and by homogenization at 10,000 rpm were selected for adjusting the temperature dependence. The Ea values of TTIs containing 0.5, 1.0, and 1.5 g microbeads, prepared by SPG membrane and conventional methods, were estimated to be 86.0, 83.5, and 76.6 kJ/mol, and 85.5, 73.5, and 62.2 kJ/mol, respectively. Therefore, microbial TTIs developed using SPG membrane technology are much more efficient in controlling temperature dependence.

  8. Extradural lipomatosis presenting with paraplegia.

    Directory of Open Access Journals (Sweden)

    Deogaonkar M

    1995-07-01

    Full Text Available An unusual case with spinal extradural lipomatosis in a non-obese and otherwise healthy man is reported. The patient presented with a history of weakness of legs which progressed to paraplegia over a 40 day period.

  9. Extramedullary hematopoiesis and paraplegia in a patient with hemoglobin e-Beta thalassemia.

    Science.gov (United States)

    Alam, M R; Habib, M S; Dhakal, G P; Khan, M R; Rahim, M A; Chowdhury, A J; Mahmud, T K

    2010-07-01

    Extramedullary hematopoiesis (EMH) occurs in patients with various hematologic disorders involving a chronic increase in the production of red blood cells, and is often associated polycythemia vera and sickle cell anaemia, but is less common with thalassemia especially with hemoglobin E-beta thalassemia. Spinal cord compression due to EMH is a extremely rare complication of thalassemia and may present with paraparesis or paraplegia with or without sensory impairment. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with paraplegia have been treated with either surgery or radiotherapy with equal frequency and efficacy, almost all reported cases with paraplegia have been treated with surgery with or without radiation therapy. We hereby report a case of hemoglobin E-beta thalassemia with paraplegia treated successfully with radiotherapy.

  10. [Surgical treatment of thoracic and lumbar tuberculosis complicated with severe kyphotic deformity and paraplegia].

    Science.gov (United States)

    Hu, Jiang; Wang, Yue; Liu, Zhongqian; Tang, Liuyi; Wan, Lun; Zhang, Yaoming; Deng, Juncai

    2014-09-01

    To explore an method and the effectiveness of surgical treatment of thoracic and lumbar tuberculosis complicated with severe kyphotic deformity (Cobb angle ≥ 55°) and paraplegia. Between January 2009 and January 2013, 13 cases of thoracic and lumbar tuberculosis complicated with severe kyphotic deformity and paraplegia were treated by one-stage posterior vertebral column resection (PVCR), debridement, bone grafting, and instrumentation fixation. Of 13 patients, 7 were male and 6 were female with an average age of 23.5 years (range, 14-49 years). The disease duration was 13-38 months (mean, 19 months). The Cobb angle of kyphosis was (65.23 ± 7.95)°. The visual analogue scale score (VAS) was 7.38 ± 0.31. In 13 patients with incomplete paraplegia, 1 case was classified as Frankel grade B, 7 cases as grade C, and 5 cases as grade D. The lesion involved 2 vertebrae bodies in 7 cases (T8, 9 in 1 case, T11, 12 in 2 cases, and T12, L1 in 4 cases), 3 vertebrae bodies in 4 cases (T10-12 in 2 cases, T9-11 in 1 case, and T11-L1 in 1 case), and 4 vertebrae bodies in 2 cases (T4-7 in 1 case and T6-9 in 1 case). Imaging examination showed paravertebral abscess in 10 cases. Healing of incision by first intention was obtained in all patients. The neurological injury and pulmonary infection occurred in 3 cases and 2 cases respectively, which were cured after symptomatic treatment. Thirteen patients were followed up 12-48 months (mean, 17 months). The erythrocyte sedimentation rate restored to normal level in all cases at 3-7 months after operation. All the patients achieved bony fusion at 10-20 months (mean, 14 months) after operation. No fixation loosening, displacement, or fracture occurred during follow-up. Common toxic symptom of tuberculosis disappeared, and there was no recurrence of local tuberculosis. The Cobb angle of kyphosis was corrected to (22.38 ± 1.76)° at 1 week and (22.15 ± 1.83)° at last follow-up, showing significant difference when compared with

  11. Spinal cord potentials in traumatic paraplegia and quadriplegia.

    Science.gov (United States)

    Sedgwick, E M; el-Negamy, E; Frankel, H

    1980-01-01

    Cortical, cervical and lumbar somatosensory evoked potentials were recorded following median and tibial nerve stimulation in patients with traumatic paraplegia and quadriplegia. The isolated cord was able to produce normal potentials even during spinal shock if the vertical extent of the lesion did not involve the generator mechanisms. The cervical potentials showed subtle changes in paraplegia at Th5 levels and below. In high cervical lesions the early cervical potentials may still be present but the later potentials were absent or, in partial lesions, delayed. PMID:7420105

  12. Sexualidade e paraplegia: o dito, o explícito e o oculto

    OpenAIRE

    França, Inacia Sátiro Xavier de; Chaves, Adriana de Freitas

    2005-01-01

    INTRODUÇÃO: A intenção de contribuir para o desenvolvimento de ações educativas que desmistifiquem mitos e tabus relacionados a sexualidade da pessoa portadora de paraplegia e melhorar a qualidade de vida desse tipo de cliente motivou a seleção do objeto "sexualidade da mulher portadora de paraplegia". OBJETIVO: Compreender a construção de sentidos das mulheres portadoras de paraplegia acerca da sexualidade e relatar as barreiras enfrentadas por estas mulheres para vivenciar sua sexualidade. ...

  13. Endogenous spar tin, mutated in hereditary spastic paraplegia, has a complex subcellular localization suggesting diverse roles in neurons

    International Nuclear Information System (INIS)

    Robay, Dimitri; Patel, Heema; Simpson, Michael A.; Brown, Nigel A.; Crosby, Andrew H.

    2006-01-01

    Mutation of spartin (SPG20) underlies a complicated form of hereditary spastic paraplegia, a disorder principally defined by the degeneration of upper motor neurons. Using a polyclonal antibody against spartin to gain insight into the function of the endogenous molecule, we show that the endogenous molecule is present in two main isoforms of 85 kDa and 100 kDa, and 75 kDa and 85 kDa in human and murine, respectively, with restricted subcellular localization. Immunohistochemical studies on human and mouse embryo sections and in vitro cell studies indicate that spartin is likely to possess both nuclear and cytoplasmic functions. The nuclear expression of spartin closely mirrors that of the snRNP (small nuclear ribonucleoprotein) marker α-Sm, a component of the spliceosome. Spartin is also enriched at the centrosome within mitotic structures. Notably we show that spartin protein undergoes dynamic positional changes in differentiating human SH-SY5Y cells. In undifferentiated non-neuronal cells, spartin displays a nuclear and diffuse cytosolic profile, whereas spartin transiently accumulates in the trans-Golgi network and subsequently decorates discrete puncta along neurites in terminally differentiated neuroblastic cells. Investigation of these spartin-positive vesicles reveals that a large proportion colocalizes with the synaptic vesicle marker synaptotagmin. Spartin is also enriched in synaptic-like structures and in synaptic vesicle-enriched fraction

  14. Paraplegia due to extramedullary hematopoiesis in thalassemia treated successfully with radiation therapy.

    Science.gov (United States)

    Malik, Monica; Pillai, Lakshmi S; Gogia, Nidhi; Puri, Tarun; Mahapatra, M; Sharma, Daya Nand; Kumar, Rajat

    2007-03-01

    Spinal cord compression due to extramedullary hematopoiesis (EMH) is a rare complication of thalassemia and generally presents as paraparesis with sensory impairment. Complete paraplegia is extremely rare in EMH due to thalassemia although it is known to occur in polycythemia vera and sickle cell anemia. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with paraparesis have been treated with either surgery or radiotherapy with equal frequency and efficacy, almost all reported cases with paraplegia have been treated with surgery with or without radiation therapy. We hereby report a case of thalassemia intermedia with paraplegia treated successfully with radiotherapy.

  15. Unusual presentation of aortic dissection: Post-coital acute paraplegia with renal failure

    Directory of Open Access Journals (Sweden)

    Dinith P Galabada

    2014-01-01

    Full Text Available We report the case of a 45-year-old chronic smoker who presented with acute paraplegia occurring during coitus and subsequently developed acute renal failure (ARF requiring dialysis. He had absent peripheral pulses in the lower limbs with evidence of acute ischemia. Doppler study showed dissecting aneurysm of thoracic aorta, thrombotic occlusion of the distal aorta from L1 level up to bifurcation and occlusion of the right renal artery by a thrombus that was confirmed by magnetic resonance imaging of the spine. He was not subjected to any vascular intervention as his lower limbs were not salvageable due to delay in the diagnosis. Post-coital aortic dissection and aortic dissection presenting with acute paraplegia and ARF are very rare. This is probably the first case report with post-coital acute aortic dissection presenting with paraplegia and ARF. This case emphasizes the importance of a careful examination of peripheral pulses in patients presenting with ARF and paraplegia.

  16. Prevention of paraplegia after endovascular exclusion for stanford B thoracic aortic dissection aneurism

    International Nuclear Information System (INIS)

    Feng Rui; Jing Zaiping; Bao Junmin; Zhao Zhiqing; Zhao Jun; Feng Xiang; Lu Qingsheng; Huang Cheng

    2003-01-01

    Objective: To assess the prophylactic measures of paraplegia and paralysis after endovascular graft exclusion (EVE) for Stanford B thoracic aortic dissections (TAD). Methods: The records of 116 consecutive patients undergoing endovascular TAD repair from 1998 to 2001 were retrospectively reviewed. Steroids were administrated postoperatively in high risk patients likely to be candidates for paraplegia or paralysis. Results: No paraplegia or paralysis occurred postoperatively in all cases, including the patient undergone selective spinal artery angiography (SSAA). Conclusions: Transluminal repair can avoid spinal cord ischemia due to aortic cross-clamping, there is still a risk of spinal cord injury caused by occlusion of intercostal arteries under the cover of endograft. A combination of the prophylactic measures, including SSAA and steroids, have been able to reduce the risk of paraplegia and paralysis. A graft-stent of appropriate length is the key point of this procedure

  17. Rare presentations of hyperthyroidism--Basedow's paraplegia and pancytopenia.

    Science.gov (United States)

    Chen, Yi-Hsien; Lin, Hung-Jung; Chen, Kuo-Tai

    2009-02-01

    Typical presentations of hyperthyroidism are palpitation, nervousness, tremor, malaise, and weight loss. Hyperthyroidism affects nearly every system in the body, and some patients may manifest neurologic or hematologic symptoms. Atypical presentations of hyperthyroidism often pose a great challenge in diagnosis and treatment. We report a case of Basedow's paraplegia and pancytopenia that was precipitated by hyperthyroidism. The unusual manifestations led to unnecessary examinations and delayed the treatment of hyperthyroidism. The classical symptoms of Basedow's paraplegia are subacute symmetric weakness of the lower extremities with areflexia and sparing sensation or sphincter involvement. Control of the hyperthyroidism mitigated the neurologic and hematologic complications and prevented unnecessary studies.

  18. Paraplegia after intercostal neurolysis with phenol

    Directory of Open Access Journals (Sweden)

    Gollapalli L

    2014-11-01

    Full Text Available Lakshman Gollapalli, Rudramanaidu Muppuri Department of Anesthesiology and Pain Medicine, Wayne State University/Detroit Medical Center, Detroit, MI, USA Abstract: In patients with advanced stages of cancer, severe pain is commonly encountered and is very difficult to treat. It affects the quality of life of the patient and the families involved. Pain can be managed using analgesics and adjuvant therapy. However, studies have shown that at least 10%–15% of patients fail to control pain adequately and will experience severe pain. We discuss the case of a 66-year-old female with metastatic adenoid cystic carcinoma of the left submandibular gland and developed paraplegia following intercostal neurolysis with phenol. After a successful diagnostic T6 to T12 intercostal nerve block, the patient was scheduled for an intercostal neurolytic block. We injected 2 mL of 10% aqueous phenol at each level on the left from the T6 to T12 ribs. One hour after the procedure, the patient developed bilateral lower extremity weakness with difficulty moving. A physical examination showed the absence of sensation to pinpricks and vibration from T10 to S5 and an absence of anal sphincter tone and sensation. Magnetic resonance images of the thoracic and lumbar spine showed leptomeningeal metastatic disease and myelitis. We postulate that the paraplegia could be from phenol diffusing along either the spinal nerves or the paravertebral venous plexus into the subarachnoid space. This case report points to the risks involved with phenol neurolysis close to the spine, and we propose alternative methods to minimize neurological complications. Keywords: intercostal neurolysis, pain, phenol, paraplegia 

  19. Spatacsin and spastizin act in the same pathway required for proper spinal motor neuron axon outgrowth in zebrafish.

    Science.gov (United States)

    Martin, Elodie; Yanicostas, Constantin; Rastetter, Agnès; Alavi Naini, Seyedeh Maryam; Maouedj, Alissia; Kabashi, Edor; Rivaud-Péchoux, Sophie; Brice, Alexis; Stevanin, Giovanni; Soussi-Yanicostas, Nadia

    2012-12-01

    Hereditary spastic paraplegias (HSPs) are rare neurological conditions caused by degeneration of the long axons of the cerebrospinal tracts, leading to locomotor impairment and additional neurological symptoms. There are more than 40 different causative genes, 24 of which have been identified, including SPG11 and SPG15 mutated in complex clinical forms. Since the vast majority of the causative mutations lead to loss of function of the corresponding proteins, we made use of morpholino-oligonucleotide (MO)-mediated gene knock-down to generate zebrafish models of both SPG11 and SPG15 and determine how invalidation of the causative genes (zspg11 and zspg15) during development might contribute to the disease. Micro-injection of MOs targeting each gene caused locomotor impairment and abnormal branching of spinal cord motor neurons at the neuromuscular junction. More severe phenotypes with abnormal tail developments were also seen. Moreover, partial depletion of both proteins at sub-phenotypic levels resulted in the same phenotypes, suggesting for the first time, in vivo, a genetic interaction between these genes. In conclusion, the zebrafish orthologues of the SPG11 and SPG15 genes are important for proper development of the axons of spinal motor neurons and likely act in a common pathway to promote their proper path finding towards the neuromuscular junction. Copyright © 2012 Elsevier Inc. All rights reserved.

  20. Hereditary spastic paraplegia associated with thin corpus callosum Paraplegia espástica hereditária associada a hipoplasia de corpo caloso

    Directory of Open Access Journals (Sweden)

    Hélio A. Ghizoni Teive

    2001-09-01

    Full Text Available Autosomal recessive hereditary spastic paraplegia (AR-HSP associated with thin corpus callosum was recently described in Japan, and most families were linked to chromosome 15q13-15. We report two patients from two different Brazilian families with progressive gait disturbance starting at the second decade of life, spastic paraparesis, and mental deterioration. One patient presented cerebellar ataxia. Magnetic resonance imaging (MRI of the head of both patients showed a thin corpus callosum. AR-HSP with a thin corpus callosum is a rare disorder, mainly described in Japanese patients. We found only 4 Caucasian families with AR-HSP with thin corpus callosum described in the literature. Further studies including additional Caucasian families of AR-HSP with thin corpus callosum are required to delineate the genetic profile of this syndrome in occidental countries.A paraplegia espástica hereditária autossômica recessiva (PEH-AR associada com hipoplasia de corpo caloso foi inicialmente descrita no Japão. Estudos de ligação genética mostram que a maioria das famílias estão relacionadas ao cromossomo 15q13-15. Relatamos dois pacientes de famílias brasileiras, não relacionadas, com distúrbio de marcha com início na segunda década de vida, paraparesia espástica e comprometimento das funções cognitivas. Um dos pacientes apresentava ataxia cerebelar. A ressonância magnética de encéfalo de ambos os pacientes mostrou hipoplasia de corpo caloso. PEH-AR associada com hipoplasia de corpo caloso é uma condição rara, descrita principalmente em pacientes do Japão. Encontramos apenas 4 famílias caucasianas com PEH-AR e hipoplasia de corpo caloso. Mais estudos com famílias caucasianas são necessários para delinear o perfil genético dessa síndrome em países ocidentais.

  1. Cardiovascular disease risk factors in persons with paraplegia: the Stockholm spinal cord injury study.

    Science.gov (United States)

    Wahman, Kerstin; Nash, Mark S; Westgren, Ninni; Lewis, John E; Seiger, Ake; Levi, Richard

    2010-03-01

    To examine cardiovascular disease risk factors and risk clusters in Swedish persons with traumatic wheelchair-dependent paraplegia. Prospective examination. A total of 135 individuals aged 18-79 years with chronic (>or= 1 year) post-traumatic paraplegia. Cardiovascular disease risk factors; dyslipidemia, impaired fasting glucose, hypertension, overweight, smoking, and medication usage for dyslipidemia, hypertension, and diabetes mellitus, were analyzed according to authoritative guidelines. Stepwise regression tested the effects of age, gender, and injury characteristics on cardiovascular disease risks. High-prevalence risk factors were dyslipidemia (83.1%), hypertension (39.3%), and overweight (42.2%) with pervasive clustering of these risks. Being older was related to increased cardiovascular disease risk, except for dyslipidemia. Hypertension was more common in low-level paraplegia. Prevalence of impaired fasting glucose was lower than previously reported after paraplegia. A high percentage of persons being prescribed drug treatment for dyslipidemia and hypertension failed to reach authoritative targets for cardiovascular disease risk reduction. Swedish persons with paraplegia are at high risk for dyslipidemia, hypertension, and overweight. Impaired fasting glucose was not as common as reported in some previous studies. Pharmacotherapy for dyslipidemia and hypertension often failed to achieve recommended targets. Population-based screening and therapeutic countermeasures to these cardiovascular disease risks are indicated.

  2. Case report: Basedow paraplegia: A possible misnomer | Smith ...

    African Journals Online (AJOL)

    Thyrotoxic myopathy frequently occurs in clinical practice; however, the association of hyperthyroidism with a flaccid, areflexic paraplegia, so-called ... It is important to consider and treat other causes, such as acute idiopathic polyneuritis.

  3. Paraplegia following intraaortic balloon circulatory assistance

    Directory of Open Access Journals (Sweden)

    Benício Anderson

    1999-01-01

    Full Text Available Intraaortic balloon counterpulsation is frequently used in patients experiencing severe ventricular dysfunction following maximal drug therapy. However, even with the improvement of percutaneous insertion techniques, the procedure has always been followed by vascular, infectious, and neurological complications. This article describes a case of paraplegia due to intraaortic balloon counterpulsation in the postoperative period of cardiac surgery.

  4. Spastin subcellular localization is regulated through usage of different translation start sites and active export from the nucleus

    International Nuclear Information System (INIS)

    Claudiani, Pamela; Riano, Elena; Errico, Alessia; Andolfi, Gennaro; Rugarli, Elena I.

    2005-01-01

    Most cases of autosomal-dominant hereditary spastic paraplegia are linked to mutations in SPG4 encoding spastin, a protein involved in microtubule dynamics and membrane trafficking. In pyramidal neurons of the motor cortex and in immortalized motor neurons, spastin is localized to the synaptic terminals and growth cones. However, in other neurons and in proliferating cells spastin is prevalently nuclear. The mechanisms that determine targeting of spastin to the nucleus or the cytoplasm are unknown. We show here that the SPG4 mRNA is able to direct synthesis of two spastin isoforms, 68 and 60 kDa, respectively, through usage of two different translational start sites. Both isoforms are imported into the nucleus, but the 68-kDa isoform contains two nuclear export signals that efficiently drive export to the cytoplasm. Nuclear export is leptomycin-B sensitive. The cytoplasmic 68-kDa spastin isoform is more abundant in the brain and the spinal cord than in other tissues. Our data indicate that spastin function is modulated through usage of alternative translational start sites and active nuclear import and export, and open new perspectives for the pathogenesis of hereditary spastic paraplegia

  5. Insulin resistance in tetraplegia but not in mid-thoracic paraplegia: is the mid-thoracic spinal cord involved in glucose regulation?

    Science.gov (United States)

    Bluvshtein, V; Korczyn, A D; Pinhas, I; Vered, Y; Gelernter, I; Catz, A

    2011-05-01

    Controlled experimental human study. To assess insulin resistance (IR) in tetraplegia and paraplegia, and the role of the spinal cord (SC) in glucose regulation. Laboratory of Spinal Research, Loewenstein Rehabilitation Hospital. Glucose and insulin levels and the heart rate variation spectral components LF (low frequency), HF (high frequency) and LF/HF were studied at supine rest, head-up tilt and after a standard meal in three groups: 13 healthy subjects, 7 patients with T(4)-T(6) paraplegia and 11 patients with C(4)-C(7) tetraplegia. Glucose and insulin increased significantly after the meal in all groups (Pinsulin level tended to accompany increases in LF/HF after the meal in the tetraplegia and control groups but not in the paraplegia group. Post-prandial IR appears in C(4)-C(7) but not in T(4)-T(6) SC injury. The results of the study, combined with previously published findings, are consistent with the hypotheses that IR is related to activation of the sympathetic nervous system, and that below T(4) the mid-thoracic SC is involved in the regulation of glucose and insulin levels.

  6. Hand muscles corticomotor excitability in hereditary spastic paraparesis type 4.

    Science.gov (United States)

    Ginanneschi, Federica; Carluccio, Maria A; Mignarri, Andrea; Tessa, Alessandra; Santorelli, Filippo M; Rossi, Alessandro; Federico, Antonio; Dotti, Maria T

    2014-08-01

    Transcranial magnetic stimulation (TMS) studies on the pathways to the upper limbs have revealed inconsistent results in patients harboring mutations in SPAST/SPG4 gene, responsible for the commonest form of hereditary spastic paraplegia (HSP). This paper is addressed to study the corticomotor excitability of the pathways to the upper limbs in SPG4 subjects. We assessed the corticomotor excitability of hand muscles in 12 subjects belonging to 7 unrelated SPG4 families and in 12 control subjects by stimulus-response curve [input-output (I-O) curve]. All the parameters of the recruitment curve (threshold, V50, slope and plateau) did not differ significantly from those of the controls. Presence of upper limb hyper-reflexia did not influence the results of I-O curve. Considering the multiplicity of possible genes/loci accounting for pure HSPs, performing TMS analyses could be helpful in differential diagnosis of pure HSPs in the absence of other clinical or neuroimaging tools.

  7. Management of ruptured anterior communicating artery aneurysms presenting with sudden paraplegia

    Directory of Open Access Journals (Sweden)

    Jiu-hong MA

    2016-10-01

    Full Text Available Objective  To explore the causes of ruptured anterior communicating artery aneurysms presenting with paraplegia, and summarize the key points of diagnosis and treatment methods. Methods  A total of 260 patients with ruptured anterior communicating artery aneurysms were received medical treatment in the Department of Neurosurgery, Shanxi Provincial People's Hospital from Jan. 2012 to Mar. 2015. Of which 6 patients were clinically presented with paraplegia, their clinical data including CT/MR/DSA were retrospectively analyzed, and based on the analysis, aneurysm embolization and anti-vasospasm treatment were performed. Results  Besides headache and discomfort in the neck, 5 of the 6 patients were with double lower limbs paraplegia, and the another one presented quadriplegia. By symptomatic treatment of aneurysm embolization and anti vasospasm, the myodynamia of the paraplegic limbs recovered from 0-Ⅰto Ⅳ-Ⅴgrade, and 2 of the 6 patients spent a shorter recovery time (about 2 weeks, the other 4 recovered in 3 months. The limbs myodynamia of the 6 patients recovered completely in half-and one year follow up. Conclusions  The mechanism of ruptured anterior communicating artery aneurysms presenting with paraplegia may be the insufficient blood supply to the primary motor area and supplementary motor area (SMA of brain cortex caused by aneurysms rupture. Aneurysm embolization should be performed in clinical treatment, supplemented with anti vasospasm and symptomatic treatment of improving neurological function. DOI: 10.11855/j.issn.0577-7402.2016.09.14

  8. Radiotherapy of paraplegias caused by extramedullar hemopoietic tissue

    International Nuclear Information System (INIS)

    Dietz, R.; Niewald, M.; Schnabel, K.; Berberich, W.

    1985-01-01

    The presentation by computed tomography of an extramedullar epidural intraspinal hemopoisis has only been described sporadically. It has been possible to visualize by CT-scans the regression under radiotherapy of a sensory and motor paraplegia in a 17 years old patient with β-thalassemia major. The central nevous system (thoracic and lumbar zone as well as os sacrum) was exposed to a total reference dose of 30 Gy which was administered within twenty days by a 60 Co unit in single doses of 2 Gy each. From 4 Gy on, the paraplegic symptoms regressed continuously. The control CT taken after 26 Gy - the paraplegia had completely disappeared as this time - showed a significant regression of the intraspinal soft tissue masses. Radiotherapy is very important in the treatment of this disease. A direct correlation is found between clinical symptoms and CT-presentation. (orig.) [de

  9. Fitzsimmons Syndrome: Spastic Paraplegia, Brachydactyly, and Cognitive Impairment

    NARCIS (Netherlands)

    Armour, Christine M.; Humphreys, Peter; Hennekam, Raoul C. M.; Boycott, Kym M.

    2009-01-01

    Fitzsimmons syndrome is an infrequently described entity comprising slowly progressive spastic paraplegia, brachydactyly, and cone-shaped epiphyses, dysarthria, and low-normal intelligence. Five patients with this syndrome have been reported. The cause remains unknown. Here we describe a 16-year-old

  10. Technical and surgical aspects of the sphenopalatine ganglion (SPG) microstimulator insertion procedure

    DEFF Research Database (Denmark)

    Assaf, A T; Hillerup, S; Rostgaard, J

    2016-01-01

    into the pterygopalatine fossa (PPF) is presented herein. Technical aspects include detailed descriptions of the preoperative planning using computed tomography or cone beam computed tomography scans for presurgical digital microstimulator insertion into the patient-specific anatomy and intraoperative verification......), and removal (n=5). This SPG microstimulator insertion procedure has sequelae comparable to other oral cavity procedures including tooth extractions, sinus surgery, and dental implant placement. Twenty-five of 29 subjects (86%) completing a self-assessment questionnaire indicated that the surgical effects were...

  11. Cellular Pathways of Hereditary Spastic Paraplegia*

    OpenAIRE

    Blackstone, Craig

    2012-01-01

    Human voluntary movement is controlled by the pyramidal motor system, a long CNS pathway comprising corticospinal and lower motor neurons. Hereditary spastic paraplegias (HSPs) are a large, genetically diverse group of inherited neurologic disorders characterized by a length-dependent distal axonopathy of the corticospinal tracts, resulting in lower limb spasticity and weakness. A range of studies are converging on alterations in the shaping of organelles, particularly the endoplasmic reticul...

  12. Clinical features and management of hereditary spastic paraplegia

    Directory of Open Access Journals (Sweden)

    Ingrid Faber

    2014-03-01

    Full Text Available Hereditary spastic paraplegia (HSP is a group of genetically-determined disorders characterized by progressive spasticity and weakness of lower limbs. An apparently sporadic case of adult-onset spastic paraplegia is a frequent clinical problem and a significant proportion of cases are likely to be of genetic origin. HSP is clinically divided into pure and complicated forms. The later present with a wide range of additional neurological and systemic features. To date, there are up to 60 genetic subtypes described. All modes of monogenic inheritance have been described: autosomal dominant, autosomal recessive, X-linked and mitochondrial traits. Recent advances point to abnormal axonal transport as a key mechanism leading to the degeneration of the long motor neuron axons in the central nervous system in HSP. In this review we aim to address recent advances in the field, placing emphasis on key diagnostic features that will help practicing neurologists to identify and manage these conditions.

  13. Hereditary spastic paraparesis in adults. A clinical and genetic perspective from Tuscany.

    Science.gov (United States)

    Orsucci, Daniele; Petrucci, Loredana; Ienco, Elena Caldarazzo; Chico, Lucia; Simi, Paolo; Fogli, Antonella; Baldinotti, Fulvia; Simoncini, Costanza; LoGerfo, Annalisa; Carlesi, Cecilia; Arnoldi, Alessia; Bassi, Maria Teresa; Siciliano, Gabriele; Bonuccelli, Ubaldo; Mancuso, Michelangelo

    2014-05-01

    Hereditary spastic paraparesis or paraplegias (HSPs) are a group of neurogenetic conditions with prominent involvement of the pyramidal tracts. Aim of this study is the clinical and molecular characterization of a cohort of patients with HSP. Moreover, we aim to study the minimum prevalence of HSP in our area and to propose a schematic diagnostic approach to HSP patients based on the available data from the literature. Retrospective/perspective study on the subjects with clinical signs and symptoms indicative of pure or complicated HSP, in whom other possible diagnosis were excluded by appropriate neuroradiological, neurophysiologic and laboratory studies, who have been evaluated by the Neurogenetic Service of our Clinic in last two years (2011-2012). 45 patients were identified. The minimum prevalence of HSP in our area was of about 2.17-3.43/100,000. The SF-36 (quality of life) and SPRS (disease progression) scores were inversely related; the time-saving, four-stage scale of motor disability could predict the SPRS scores with a high statistical significance, and we encourage its use in HSP. Our study confirms SPG4 as the major cause of HSP. All SPG4 patients had a pure HSP phenotype, and the dominant inheritance was evident in the great majority of these subjects. SPG7 was the second genetic cause. Other genotypes were rarer (SPG10, SPG11, SPG17). Exact molecular diagnosis will allow a more accurate patient counseling and, hopefully, will lead to specific, targeted, therapeutic options for these chronic, still incurable diseases. Copyright © 2014. Published by Elsevier B.V.

  14. Increased cardiovascular disease risk in Swedish persons with paraplegia: The Stockholm spinal cord injury study.

    Science.gov (United States)

    Wahman, Kerstin; Nash, Mark S; Lewis, John E; Seiger, Ake; Levi, Richard

    2010-05-01

    Comparison of prevalence of cardiovascular disease risks in persons with chronic traumatic paraplegia with those in the general population. Cross-sectional comparative study. A total of 135 individuals, age range 18-79 years, with chronic (> or = 1 year) traumatic paraplegia. The prevalences of diabetes mellitus, dyslipidaemia, hypertension, overweight, and smoking, were assessed in the study population and were compared with an age- and gender-matched sample of the general population in the region under study. History of myocardial infarction and medication for dyslipidaemia, hypertension, and diabetes mellitus were also recorded. chi2 tests were used to compare the paraplegic cohort with the general population sample. Significantly more persons with paraplegia reported a history of myocardial infarction (5.9%) than those in the comparison group (0.7%). The prevalences of diabetes mellitus (5.9%), dyslipidaemia (11.1%), and hypertension (14.1%) were also significantly higher in the paraplegic group, as were drug treatment for these disorders. Persons with paraplegia report increased prevalences of diabetes mellitus, hypertension, and dyslipidaemia, in particular, compared with the general population. Population-based screening and therapeutic counter-measures for these conditions may therefore be particularly indicated for this patient group.

  15. Paraplegia after contrast media application: a transient or devastating rare complication? Case report.

    Science.gov (United States)

    Mielke, Dorothee; Kallenberg, Kai; Hartmann, Marius; Rohde, Veit

    2016-05-01

    The authors report the case of a 76-year-old man with a spinal dural arteriovenous fistula. The patient suffered from sudden repeated reversible paraplegia after spinal digital subtraction angiography as well as CT angiography. Neurotoxicity of contrast media (CM) is the most probable cause for this repeated short-lasting paraplegia. Intolerance to toxicity of CM to the vulnerable spinal cord is rare, and probably depends on the individual patient. This phenomenon is transient and can occur after both intraarterial and intravenous CM application.

  16. Loss of spatacsin function alters lysosomal lipid clearance leading to upper and lower motor neuron degeneration.

    Science.gov (United States)

    Branchu, Julien; Boutry, Maxime; Sourd, Laura; Depp, Marine; Leone, Céline; Corriger, Alexandrine; Vallucci, Maeva; Esteves, Typhaine; Matusiak, Raphaël; Dumont, Magali; Muriel, Marie-Paule; Santorelli, Filippo M; Brice, Alexis; El Hachimi, Khalid Hamid; Stevanin, Giovanni; Darios, Frédéric

    2017-06-01

    Mutations in SPG11 account for the most common form of autosomal recessive hereditary spastic paraplegia (HSP), characterized by a gait disorder associated with various brain alterations. Mutations in the same gene are also responsible for rare forms of Charcot-Marie-Tooth (CMT) disease and progressive juvenile-onset amyotrophic lateral sclerosis (ALS). To elucidate the physiopathological mechanisms underlying these human pathologies, we disrupted the Spg11 gene in mice by inserting stop codons in exon 32, mimicking the most frequent mutations found in patients. The Spg11 knockout mouse developed early-onset motor impairment and cognitive deficits. These behavioral deficits were associated with progressive brain atrophy with the loss of neurons in the primary motor cortex, cerebellum and hippocampus, as well as with accumulation of dystrophic axons in the corticospinal tract. Spinal motor neurons also degenerated and this was accompanied by fragmentation of neuromuscular junctions and muscle atrophy. This new Spg11 knockout mouse therefore recapitulates the full range of symptoms associated with SPG11 mutations observed in HSP, ALS and CMT patients. Examination of the cellular alterations observed in this model suggests that the loss of spatacsin leads to the accumulation of lipids in lysosomes by perturbing their clearance from these organelles. Altogether, our results link lysosomal dysfunction and lipid metabolism to neurodegeneration and pinpoint a critical role of spatacsin in lipid turnover. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

  17. Role of the AP-5 adaptor protein complex in late endosome-to-Golgi retrieval.

    Directory of Open Access Journals (Sweden)

    Jennifer Hirst

    2018-01-01

    Full Text Available The AP-5 adaptor protein complex is presumed to function in membrane traffic, but so far nothing is known about its pathway or its cargo. We have used CRISPR-Cas9 to knock out the AP-5 ζ subunit gene, AP5Z1, in HeLa cells, and then analysed the phenotype by subcellular fractionation profiling and quantitative mass spectrometry. The retromer complex had an altered steady-state distribution in the knockout cells, and several Golgi proteins, including GOLIM4 and GOLM1, were depleted from vesicle-enriched fractions. Immunolocalisation showed that loss of AP-5 led to impaired retrieval of the cation-independent mannose 6-phosphate receptor (CIMPR, GOLIM4, and GOLM1 from endosomes back to the Golgi region. Knocking down the retromer complex exacerbated this phenotype. Both the CIMPR and sortilin interacted with the AP-5-associated protein SPG15 in pull-down assays, and we propose that sortilin may act as a link between Golgi proteins and the AP-5/SPG11/SPG15 complex. Together, our findings suggest that AP-5 functions in a novel sorting step out of late endosomes, acting as a backup pathway for retromer. This provides a mechanistic explanation for why mutations in AP-5/SPG11/SPG15 cause cells to accumulate aberrant endolysosomes, and highlights the role of endosome/lysosome dysfunction in the pathology of hereditary spastic paraplegia and other neurodegenerative disorders.

  18. A case of acute paraplegia that improved with dialysis.

    Science.gov (United States)

    Rajendiran, Govarthanan; Jayabalan, Rajamahesh; Chandrahasan, Saravanan; Mani, Ashwin Kumar

    2008-01-01

    Acute severe hyperkalemia can present as acute paraplegia independent of cardiac effects, even though cardiac muscle is more sensitive to serum potassium changes. We managed a patient with acute hyperkalemic paralysis who did not have threatening cardiac/electrocardiographic manifestations. The limb weakness became normal after hemodialysis.

  19. Mechanistic basis of an epistatic interaction reducing age at onset in hereditary spastic paraplegia.

    Science.gov (United States)

    Newton, Timothy; Allison, Rachel; Edgar, James R; Lumb, Jennifer H; Rodger, Catherine E; Manna, Paul T; Rizo, Tania; Kohl, Zacharias; Nygren, Anders O H; Arning, Larissa; Schüle, Rebecca; Depienne, Christel; Goldberg, Lisa; Frahm, Christiane; Stevanin, Giovanni; Durr, Alexandra; Schöls, Ludger; Winner, Beate; Beetz, Christian; Reid, Evan

    2018-05-01

    Many genetic neurological disorders exhibit variable expression within affected families, often exemplified by variations in disease age at onset. Epistatic effects (i.e. effects of modifier genes on the disease gene) may underlie this variation, but the mechanistic basis for such epistatic interactions is rarely understood. Here we report a novel epistatic interaction between SPAST and the contiguous gene DPY30, which modifies age at onset in hereditary spastic paraplegia, a genetic axonopathy. We found that patients with hereditary spastic paraplegia caused by genomic deletions of SPAST that extended into DPY30 had a significantly younger age at onset. We show that, like spastin, the protein encoded by SPAST, the DPY30 protein controls endosomal tubule fission, traffic of mannose 6-phosphate receptors from endosomes to the Golgi, and lysosomal ultrastructural morphology. We propose that additive effects on this pathway explain the reduced age at onset of hereditary spastic paraplegia in patients who are haploinsufficient for both genes.

  20. Functional MR imaging and traumatic paraplegia: preliminary report.

    Science.gov (United States)

    Sabbah, P; Lévêque, C; Pfefer, F; Nioche, C; Gay, S; Sarrazin, J L; Barouti, H; Tadie, M; Cordoliani, Y S

    2000-12-01

    To evaluate residual activity in the sensorimotor cortex of the lower limbs in paraplegia. 5 patients suffering from a complete paralysis after traumatic medullar lesion (ASIA=A). Clinical evaluation of motility and sensitivity. 1. Control functional MR study of the sensorimotor cortex during simultaneous movements of hands, imaginary motor task and passive hands stimulation. 2. Concerning the lower limbs, 3 fMRI conditions: 1-patient attempts to move his toes with flexion-extension, 2-mental imagery task of the same movement, 3-peripheral passive proprio-somesthesic stimulation (squeezing) of the big toes. Activations were observed in the primary sensorimotor cortex (M1), premotor regions and in the supplementary motor area (SMA) during movement and mental imaginary tasks in the control study and during attempt to move and mental imaginary tasks in the study concerning the lower limbs. Passive somesthesic stimulation generated activation posterior to the central sulcus for 2 patients. Activations in the sensorimotor cortex of the lower limbs can be generated either by attempting to move or mental evocation. In spite of a clinical evaluation of complete paraplegia, fMRI can show a persistence of sensitive anatomic conduction, confirmed by Somesthesic Evoked Potentials.

  1. Clinical Evaluation of Percutaneous Vertebroplasty in a Patient with Paraplegia and Immobilization Syndrome: A Case Report

    OpenAIRE

    Masala, Salvatore; Calabria, Eros; Nezzo, Marco; De Vivo, Dominique; Neroni, Luca; Simonetti, Giovanni

    2013-01-01

    We will discuss a potential role of percutaneous vertebroplasty (PVP) in the management of a patient with immobilization syndrome due to paraplegia and vertebral osteoporotic fractures. While PVP is commonly used for the treatment of osteoporotic thoracolumbar vertebral compression fractures, its role in vertebral stabilization in patient with immobilization syndrome has not been reported in the literature. A 73-year-old woman affected by immobilization syndrome due to paraplegia and vertebra...

  2. Hereditary spastic paraplegia.

    Science.gov (United States)

    Blackstone, Craig

    2018-01-01

    The hereditary spastic paraplegias (HSPs) are a heterogeneous group of neurologic disorders with the common feature of prominent lower-extremity spasticity, resulting from a length-dependent axonopathy of corticospinal upper motor neurons. The HSPs exist not only in "pure" forms but also in "complex" forms that are associated with additional neurologic and extraneurologic features. The HSPs are among the most genetically diverse neurologic disorders, with well over 70 distinct genetic loci, for which about 60 mutated genes have already been identified. Numerous studies elucidating the molecular pathogenesis underlying HSPs have highlighted the importance of basic cellular functions - especially membrane trafficking, mitochondrial function, organelle shaping and biogenesis, axon transport, and lipid/cholesterol metabolism - in axon development and maintenance. An encouragingly small number of converging cellular pathogenic themes have been identified for the most common HSPs, and some of these pathways present compelling targets for future therapies. Copyright © 2018 Elsevier B.V. All rights reserved.

  3. Microtubule-targeting drugs rescue axonal swellings in cortical neurons from spastin knockout mice

    Directory of Open Access Journals (Sweden)

    Coralie Fassier

    2013-01-01

    Mutations in SPG4, encoding the microtubule-severing protein spastin, are responsible for the most frequent form of hereditary spastic paraplegia (HSP, a heterogeneous group of genetic diseases characterized by degeneration of the corticospinal tracts. We previously reported that mice harboring a deletion in Spg4, generating a premature stop codon, develop progressive axonal degeneration characterized by focal axonal swellings associated with impaired axonal transport. To further characterize the molecular and cellular mechanisms underlying this mutant phenotype, we have assessed microtubule dynamics and axonal transport in primary cultures of cortical neurons from spastin-mutant mice. We show an early and marked impairment of microtubule dynamics all along the axons of spastin-deficient cortical neurons, which is likely to be responsible for the occurrence of axonal swellings and cargo stalling. Our analysis also reveals that a modulation of microtubule dynamics by microtubule-targeting drugs rescues the mutant phenotype of cortical neurons. Together, these results contribute to a better understanding of the pathogenesis of SPG4-linked HSP and ascertain the influence of microtubule-targeted drugs on the early axonal phenotype in a mouse model of the disease.

  4. A rare complication of tuberculosis: Acute paraplegia | Aydın ...

    African Journals Online (AJOL)

    A rare complication of tuberculosis: Acute paraplegia. Teoman Aydın, Özgür Taşpınar, Yasar Keskin, Müge Kepekçi, Meryem Güneşer, Adil Çamlı, Hakan Seyithanoğlu, Huriye Kızıltan, Ali Hikmet Eriş ...

  5. [A case of leptomeningeal melanomatosis with acute paraplegia and multiple cranial nerve palsies].

    Science.gov (United States)

    Hattori, Kasumi; Matsuda, Nozomu; Murakami, Takenobu; Ito, Eiichi; Ugawa, Yoshikazu

    2017-12-27

    A 62-year-old man with acute paraplegia was transferred to our hospital. He had flaccid paraplegia and multiple cranial nerve palsies, such as mydriasis of the left pupil, abduction palsy of the left eye, hoarseness and dysphagia, but no meningeal irritation signs. MRI of the spinal canal showed swellings of the conus medullaris and the cauda equine, and also contrast enhancement of the spinal meninges. The cerebrospinal fluid (CSF) showed pleocytosis and protein increment. The lymph node was swollen in his right axilla. The biopsy specimen from the right axillary lymph node revealed metastasis of malignant melanoma histologically. Careful check-up of his whole body found a malignant melanoma in the subungual region of the right ring finger. Repeated cytological examination revealed melanoma cells in the CSF, confirming the diagnosis of leptomeningeal melanomatosis. His consciousness was gradually deteriorated. His family members chose supportive care instead of chemotherapy or surgical therapy after full information about his conditions. Finally, he died 60 days after transfer to our hospital. This is a rare case of leptomenigeal melanomatosis presenting with acute paraplegia and multiple cranial nerve palsies. Careful follow-up and repeated studies are vital for the early diagnosis of leptomenigeal melanomatosis in spite of atypical clinical presentation.

  6. Acute Paraplegia due to Thoracic Hematomyelia

    Directory of Open Access Journals (Sweden)

    Aykut Akpınar

    2016-01-01

    Full Text Available Spontaneous intraspinal intramedullary hemorrhage is a rare entity with the acute onset of neurologic symptoms. The etiology of idiopathic spontaneous hematomyelia (ISH is unknown, and there are few published case reports. Hematomyelia is mostly associated with trauma, but the other nontraumatic etiologies are vascular malformations, tumors, bleeding disorders, syphilis, syrinx, and myelitis. MRI is a good choice for early diagnosis. Hematomyelia usually causes acute spinal cord syndrome due to the compression and destruction of the spinal cord. A high-dose steroid treatment and surgical decompression and evacuation of hematoma are the urgent solution methods. We present idiopathic spontaneous hematomyelia of a previously healthy 80-year-old male with a sudden onset of back pain and paraplegia.

  7. Hereditary spastic paraplegias: membrane traffic and the motor pathway

    OpenAIRE

    Blackstone, Craig; O’Kane, Cahir J.; Reid, Evan

    2011-01-01

    Voluntary movement is a fundamental way in which animals respond to, and interact with, their environment. In mammals, the main CNS pathway controlling voluntary movement is the corticospinal tract, which encompasses connections between the cerebral motor cortex and the spinal cord. Hereditary spastic paraplegias (HSPs) are a group of genetic disorders that lead to a length-dependent, distal axonopathy of fibres of the corticospinal tract, causing lower limb spasticity and weakness. Recent wo...

  8. Postoperative Paraplegia as a Result of Undiagnosed Primitive Neuroectodermal Tumor, Not Epidural Analgesia

    Directory of Open Access Journals (Sweden)

    Pei-Ching Hung

    2007-10-01

    Full Text Available Postoperative paraplegia is a rare complication after epidural analgesia and often occurs with spinal hematoma or cord injury. We present the case of a 16-year-old girl who suffered from a tumor mass in the neck and abdomen who underwent gynecologic operation. Preoperatively, liver metastasis was found by computed tomography. Pathologic findings revealed that the abdominal mass was an ovarian dermoid cyst. After the operation, the patient complained of paraplegia while receiving epidural analgesia for postoperative pain control. A peripheral primitive neuroectodermal tumor in the thoracic and lumbar spines with spinal cord compression was later detected using magnetic resonance imaging. Learning from this case, we suggest that when a patient is preoperatively diagnosed with tumor metastasis, back pain and soreness, spinal cord compression from tumor metastasis should be excluded before epidural analgesia is implemented.

  9. Converging cellular themes for the hereditary spastic paraplegias.

    Science.gov (United States)

    Blackstone, Craig

    2018-05-10

    Hereditary spastic paraplegias (HSPs) are neurologic disorders characterized by prominent lower-extremity spasticity, resulting from a length-dependent axonopathy of corticospinal upper motor neurons. They are among the most genetically-diverse neurologic disorders, with >80 distinct genetic loci and over 60 identified genes. Studies investigating the molecular pathogenesis underlying HSPs have emphasized the importance of converging cellular pathogenic themes in the most common forms of HSP, providing compelling targets for therapy. Most notably, these include organelle shaping and biogenesis as well as membrane and cargo trafficking. Published by Elsevier Ltd.

  10. Relationship Between Hand Contact Angle and Shoulder Loading During Manual Wheelchair Propulsion by Individuals with Paraplegia.

    Science.gov (United States)

    Requejo, Philip Santos; Mulroy, Sara J; Ruparel, Puja; Hatchett, Patricia E; Haubert, Lisa Lighthall; Eberly, Valerie J; Gronley, JoAnne K

    2015-01-01

    Shoulder loading during manual wheelchair propulsion (WCP) contributes to the development of shoulder pain in individuals with spinal cord injury (SCI). To use regression analysis to investigate the relationships between the hand contact angle (location of the hand on the pushrim at initial contact and release during the push phase of the WCP cycle) with propulsion characteristics, pushrim forces, and shoulder kinetics during WCP in individuals with paraplegia. Biomechanical data were collected from 222 individuals (198 men and 24 women) with paraplegia from SCI during WCP on a stationary ergometer at a self-selected speed. The average age of participants was 34.7 years (±9.3), mean time since SCI was 9.3 years (±6.1), and average body weight was 74.4 kg (±15.9). The majority (n = 127; 56%) of participants had lower level paraplegia (T8 to L5) and 95 (42%) had high paraplegia (T2 to T7). Increased push arc (mean = 75.3°) was associated with greater velocity (R = 0.384, P contact angle and hand release angles were equally associated with cycle distance and cadence, whereas a more anterior release angle was associated with greater velocity (R = 0.372, P contact angle was associated with greater posterior shoulder net joint force (R = 0.229, P = .001) and greater flexor net joint moment (R = 0.204, P = .002), whereas a more anterior hand release angle was significantly associated with increased vertical (R = 0.270, P contact and hand release must be considered in WCP training. It is recommended that participants should reach back to initiate contact with the pushrim to maximize push arc but avoid a more anterior hand position at release, because this could increase shoulder load during the push phase of WCP.

  11. Heterozygous KIDINS220/ARMS nonsense variants cause spastic paraplegia, intellectual disability, nystagmus, and obesity

    NARCIS (Netherlands)

    Josifova, Dragana J.; Monroe, Glen R.; Tessadori, Federico; de Graaff, Esther; van der Zwaag, Bert; Mehta, Sarju G.; Harakalova, Magdalena; Duran, Karen J.; Savelberg, Sanne M. C.; Nijman, Isaäc J.; Jungbluth, Heinz; Hoogenraad, Casper C.; Bakkers, Jeroen; Knoers, Nine V.; Firth, Helen V.; Beales, Philip L.; van Haaften, Gijs; van Haelst, Mieke M.

    2016-01-01

    We identified de novo nonsense variants in KIDINS220/ARMS in three unrelated patients with spastic paraplegia, intellectual disability, nystagmus, and obesity (SINO). KIDINS220 is an essential scaffold protein coordinating neurotrophin signal pathways in neurites and is spatially and temporally

  12. Serial casting for neuromuscular flatfoot and vertical talus in an adolescent with hereditary spastic paraplegia.

    Science.gov (United States)

    Sweet, Laurene A; OʼNeill, Lindsey M; Dobbs, Matthew B

    2014-01-01

    The purpose of this report is to explore assessment and serial casting intervention for painful rigid flatfoot deformities with vertical talus in an adolescent girl with hereditary spastic paraplegia who was nonambulatory. The participant's right foot underwent 2 phases of casting with correction first toward hindfoot inversion and then dorsiflexion. Because of a vertical talus, her left foot required an intermediate casting toward plantar flexion, inversion, and forefoot adduction prior to casting toward dorsiflexion. The patient improved despite the underlying progressive neuromuscular disorder. Pain ameliorated and she returned to supported standing and transfers. Spasticity decreased bilaterally and the flexibility of her foot deformities improved to allow orthotic fabrication in subtalar neutral. Results were maintained at 12 and 16 months. Individualized multiphase serial casting requires further investigation with patients such as those with hereditary spastic paraplegia.

  13. Compromise and Toleration

    DEFF Research Database (Denmark)

    Rostbøll, Christian F.

    Political compromise is akin to toleration, since both consist of an "agreement to disagree." Compromise and toleration also share a predicament of being regarded as ambiguous virtues that require of us to accept something we actually regard as wrong. However, we misunderstand the nature, justifi...... in compromise are more stringent than those for being tolerated. Still, the limits of compromise cannot be drawn to narrowly if it is to remain its value as a form of agreement that respects and embodies the differences of opinion in society.......Political compromise is akin to toleration, since both consist of an "agreement to disagree." Compromise and toleration also share a predicament of being regarded as ambiguous virtues that require of us to accept something we actually regard as wrong. However, we misunderstand the nature......, justification, and limits of compromise if we see it merely as a matter of toleration. While toleration is mainly a matter of accepting citizens' equal right to co-existence as subjects to law, political compromise includes the parties in making law – it makes them co-authors of law. Toleration entails...

  14. A unique case of Babesia gibsoni infected dog with paraplegia

    OpenAIRE

    Gonde, Suresh; Chhabra, S.; Uppal, S. K.; Singla, L. D.; Randhawa, S. S.

    2016-01-01

    A 4 year old male Rottweiler was presented to Teaching Veterinary Clinics of GADVASU with a primary complaint of paraplegia, fever and inappetence. The dog was found to be dull with elevated rectal temperature (105 °F), heart rate (158 per minute) and respiration rate (57 per minute). Blood smear examination and PCR assay revealed that dog was positive for Babesia gibsoni. Elevated bilirubin, alanine aminotransferase, alkaline phosphatase, creatinine, total leucocyte count, neutrophils and hy...

  15. [Risk factors for the development of rotator cuff tears in individuals with paraplegia : A cross-sectional study].

    Science.gov (United States)

    Pepke, W; Brunner, M; Abel, R; Almansour, H; Gerner, H J; Hug, A; Zeifang, F; Kentar, Y; Bruckner, T; Akbar, M

    2018-02-27

    Shoulder pain and rotator cuff tears are highly prevalent among wheelchair dependent individuals with paraplegia. The purpose of this study was to identify potential risk factors associated with the development of rotator cuff tears in this population. A total of 217 wheelchair dependent individuals with paraplegia were included in this cross-sectional study (level of evidence III). The mean age of this population was 47.9 years and the mean duration of wheelchair dependence was 24.1 years. Each individual was asked to complete a questionnaire designed to identify risk factors for rotator cuff tears and underwent a standardized clinical examination with the documentation of the Constant-Murley shoulder outcome score and magnetic resonance imaging (MRI) of both shoulder joints. MRI analysis revealed at least one rotator cuff tear in 93 patients (43%). Multiple logistic regression analysis identified the following factors to be associated with the presence of rotator cuff tear: patient age, duration of spinal cord injury/wheelchair dependence, gender, and wheelchair athletic activity. Neither BMI nor the level of spinal cord injury was found to pose a risk factor in the population studied. With respect to patient age, the risk of developing a rotator cuff tear increased by 11% per annum. In terms of duration of spinal cord injury, the analysis revealed a 6% increased risk per year of wheelchair dependence (OR = 1.06). Females had a 2.6-fold higher risk of developing rotator cuff tears than males and wheelchair sport activity increased the risk 2.3-fold. There is a high prevalence of rotator cuff tears in wheel-chair dependent persons with paraplegia. Risk factors such as age, gender, duration of paraplegia, and wheel chair sport activity seem to play an important role in the development of rotator cuff tears.

  16. Hereditary spastic paraplegia: More than an upper motor neuron disease.

    Science.gov (United States)

    Parodi, L; Fenu, S; Stevanin, G; Durr, A

    2017-05-01

    Hereditary spastic paraplegias (HSPs) are a group of rare inherited neurological diseases characterized by extreme heterogeneity in both their clinical manifestations and genetic backgrounds. Based on symptoms, HSPs can be divided into pure forms, presenting with pyramidal signs leading to lower-limb spasticity, and complex forms, when additional neurological or extraneurological symptoms are detected. The clinical diversity of HSPs partially reflects their underlying genetic backgrounds. To date, 76 loci and 58 corresponding genes [spastic paraplegia genes (SPGs)] have been linked to HSPs. The genetic diagnosis is further complicated by the fact that causative mutations of HSP can be inherited through all possible modes of transmission (autosomal-dominant and -recessive, X-linked, maternal), with some genes showing multiple inheritance patterns. The pathogenic mutations of SPGs primarily lead to progressive degeneration of the upper motor neurons (UMNs) comprising corticospinal tracts. However, it is possible to observe lower-limb muscle atrophy and fasciculations on clinical examination that are clear signs of lower motor neuron (LMN) involvement. The purpose of this review is to classify HSPs based on their degree of motor neuron involvement, distinguishing forms in which only UMNs are affected from those involving both UMN and LMN degeneration, and to describe their differential diagnosis from diseases such as amyotrophic lateral sclerosis. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  17. Effects of walkbot gait training on kinematics, kinetics, and clinical gait function in paraplegia and quadriplegia.

    Science.gov (United States)

    Hwang, Jongseok; Shin, Yongil; Park, Ji-Ho; Cha, Young Joo; You, Joshua Sung H

    2018-04-07

    The robotic-assisted gait training (RAGT) system has gained recognition as an innovative, effective paradigm to improve functional ambulation and activities of daily living in spinal cord injury and stroke. However, the effects of the Walkbot robotic-assisted gait training system with a specialized hip-knee-ankle actuator have never been examined in the paraplegia and quadriplegia population. The aim of this study was to determine the long-term effects of Walkbot training on clinical for hips and knee stiffness in individuals with paraplegia or quadriplegia. Nine adults with subacute or chronic paraplegia resulting from spinal cord injury or quadriplegia resulting from cerebral vascular accident (CVA) and/or hypoxia underwent progressive conventional gait retraining combined with the Walkbot RAGT for 5 days/week over an average of 43 sessions for 8 weeks. Clinical outcomes were measured with the Functional Ambulation Category (FAC), Modified Rankin Scale (MRS), Korean version of the Modified Barthel Index (K-MBI), Modified Ashworth Scale (MAS). Kinetic and kinematic data were collected via a built-in Walkbot program. Wilcoxon signed-rank tests showed significant positive intervention effects on K-MBI, maximal hip flexion and extension, maximal knee flexion, active torque in the knee joint, resistive torque, and stiffness in the hip joint (P quadriplegia who had reached a plateau in motor recovery after conventional therapy.

  18. Autoimmune Regulator (AIRE) Is Expressed in Spermatogenic Cells, and It Altered the Expression of Several Nucleic-Acid-Binding and Cytoskeletal Proteins in Germ Cell 1 Spermatogonial (GC1-spg) Cells.

    Science.gov (United States)

    Radhakrishnan, Karthika; Bhagya, Kongattu P; Kumar, Anil Tr; Devi, Anandavalli N; Sengottaiyan, Jeeva; Kumar, Pradeep G

    2016-08-01

    Autoimmune regulator (AIRE) is a gene associated with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED). AIRE is expressed heavily in the thymic epithelial cells and is involved in maintaining self-tolerance through regulating the expression of tissue-specific antigens. The testes are the most predominant extrathymic location where a heavy expression of AIRE is reported. Homozygous Aire-deficient male mice were infertile, possibly due to impaired spermatogenesis, deregulated germ cell apoptosis, or autoimmunity. We report that AIRE is expressed in the testes of neonatal, adolescent, and adult mice. AIRE expression was detected in glial cell derived neurotrophic factor receptor alpha (GFRα)(+) (spermatogonia), GFRα(-)/synaptonemal complex protein (SCP3)(+) (meiotic), and GFRα(-)/Phosphoglycerate kinase 2 (PGK2)(+) (postmeiotic) germ cells in mouse testes. GC1-spg, a germ-cell-derived cell line, did not express AIRE. Retinoic acid induced AIRE expression in GC1-spg cells. Ectopic expression of AIRE in GC1-spg cells using label-free LC-MS/MS identified a total of 371 proteins that were differentially expressed. 100 proteins were up-regulated, and 271 proteins were down-regulated. Data are available via ProteomeXchange with identifier PXD002511. Functional analysis of the differentially expressed proteins showed increased levels of various nucleic-acid-binding proteins and transcription factors and a decreased level of various cytoskeletal and structural proteins in the AIRE overexpressing cells as compared with the empty vector-transfected controls. The transcripts of a select set of the up-regulated proteins were also elevated. However, there was no corresponding decrease in the mRNA levels of the down-regulated set of proteins. Molecular function network analysis indicated that AIRE influenced gene expression in GC1-spg cells by acting at multiple levels, including transcription, translation, RNA processing, protein transport, protein

  19. Democratic Respect and Compromise

    DEFF Research Database (Denmark)

    Rostbøll, Christian F.

    2017-01-01

    Compromise has attained renewed interest among political theorists writing on pluralism and disagreement. It is controversial, however, whether compromise is a mere pragmatic necessity or if it has non-instrumental value. This article argues that the reasons for compromise are inherent in the dem......Compromise has attained renewed interest among political theorists writing on pluralism and disagreement. It is controversial, however, whether compromise is a mere pragmatic necessity or if it has non-instrumental value. This article argues that the reasons for compromise are inherent...

  20. Compound heterozygous mutations in two different domains of ALDH18A1 do not affect the amino acid levels in a patient with hereditary spastic paraplegia

    DEFF Research Database (Denmark)

    Steenhof, Maria; Kibæk, Maria; Larsen, Martin J.

    2018-01-01

    with mutations affecting the GR5P domain. We present a 19-year old male patient with autosomal recessive spastic paraplegia and compound heterozygosity for two ALDH18A1 mutations, one in each of the P5CS domains. This young man has spastic paraplegia with onset in childhood and temporal lobe epilepsy, but normal...

  1. Hereditary spastic paraplegias: membrane traffic and the motor pathway.

    Science.gov (United States)

    Blackstone, Craig; O'Kane, Cahir J; Reid, Evan

    2011-01-01

    Voluntary movement is a fundamental way in which animals respond to, and interact with, their environment. In mammals, the main CNS pathway controlling voluntary movement is the corticospinal tract, which encompasses connections between the cerebral motor cortex and the spinal cord. Hereditary spastic paraplegias (HSPs) are a group of genetic disorders that lead to a length-dependent, distal axonopathy of fibres of the corticospinal tract, causing lower limb spasticity and weakness. Recent work aimed at elucidating the molecular cell biology underlying the HSPs has revealed the importance of basic cellular processes — especially membrane trafficking and organelle morphogenesis and distribution— in axonal maintenance and degeneration.

  2. Paraplegia following epidural analgesia: A potentially avoidable cause?

    Directory of Open Access Journals (Sweden)

    Jeson R Doctor

    2014-01-01

    Full Text Available Neurological deficit is an uncommon but catastrophic complication of epidural anesthesia. Epidural hematomas and abscesses are the most common causes of such neurological deficit. We report the case of a patient with renal cell carcinoma with lumbar vertebral metastasis who developed paraplegia after receiving thoracic epidural anesthesia for a nephrectomy. Subsequently, on histo-pathological examination of the laminectomy specimen, the patient was found to have previously undiagnosed thoracic vertebral metastases which led to a thoracic epidural hematoma. In addition, delayed reporting of symptoms of neurological deficit by the patient may have impacted his outcome. Careful pre-operative investigation, consideration to using alternative modalities of analgesia, detailed patient counseling and stringent monitoring of patients receiving central neuraxial blockade is essential to prevent such complications.

  3. A large primary vaginal calculus in a woman with paraplegia.

    Science.gov (United States)

    Avsar, Ayse Filiz; Keskin, Huseyin Levent; Catma, Tuba; Kaya, Basak; Sivaslioglu, Ahmet Akın

    2013-01-01

    The study aimed to report a primary vaginal stone, an extremely rare entity, without vesicovaginal fistula in a woman with disability. We describe the case of a large primary vaginal calculus in a 22-year-old woman with paraplegia, which, surprisingly, was not diagnosed until she was examined under general anesthesia during a preparation for laparoscopy for an adnexal mass. The stone had not been identified by physical examination with the patient in a recumbent position or by transabdominal ultrasonography and pelvic tomography during the preoperative preparation. Vaginoscopy was not performed because the vagina was completely filled with the mass. As a result of its size and hard consistency, a right-sided episiotomy was performed and a 136-g stone was removed using ring forceps. A vesicovaginal fistula was excluded. There was no evidence of a foreign body or other nidus on the cut section of the stone, and it was determined to be composed of 100% struvite (ammonium magnesium phosphate). Culture of urine obtained via catheter showed Escherichia coli. After the surgical removal of the calculus without complications, a program of intermittent catheterization was started. The follow-up period was uneventful, and the patient was symptom free at 6 months after the operation. We postulate that the calculus formed as a consequence of urinary contamination of the vagina in association with incontinence and prolonged maintenance in a recumbent posture. This report is important because it highlights that, although vaginal stones are very rare, their possibility should be considered in the differential diagnosis of individuals with long-term paraplegia.

  4. MRI of autosomal dominant pure spastic paraplegia

    DEFF Research Database (Denmark)

    Krabbe, K.; Nielsen, J.E.; Fallentin, E.

    1997-01-01

    We examined 16 patients with autosomal dominant pure spastic paraplegia (HSP) and 15 normal controls matched for age and sex using MRI of the brain and spinal cord. Images were assessed qualitatively by two independent radiologists, blinded to the clinical diagnosis. Areas of the brain and corpus...... callosum on one midsagittal slice and the area of the brain on one axial slice were measured and a "corpus-callosum index" expressing the size of the corpus callosum relative to that of the brain was calculated. Cross-sectional areas and anteroposterior and transverse diameters of the spinal cord...... at the levels of C 2, C 5, T 3, T 6, T 9 and T 11 were measured. No significant differences between patients and controls were found on qualitative evaluation of the images. The patients had a significantly smaller corpus callosum and "corpus-callosum index" than controls. This finding, not reported previously...

  5. 40 CFR 13.25 - Standards for compromise.

    Science.gov (United States)

    2010-07-01

    ... 13.25 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY GENERAL CLAIMS COLLECTION STANDARDS... consider, among other factors, the following: (1) Individual debtors. (i) Age and health of the debtor; (ii... substantial doubt concerning the Government's ability to prove its case in court for the full amount of the...

  6. Thyrotoxic Neuropathy: A rare cause of acute flaccid paraplegia

    Directory of Open Access Journals (Sweden)

    Aiman K. Al-Wahaibi

    2018-01-01

    Full Text Available Acute polyneuropathy is a rare manifestation of severe hyperthyroidism. We report a 22-year-old Omani male who presented to the Sohar Hospital, Sohar, Oman, in 2016 with acute-onset rapidly progressive flaccid areflexic paraplegia as the presenting manifestation of thyrotoxicosis. Nerve conduction studies revealed mixed axonal and demyelinating polyneuropathy in both the motor and sensory nerves. Treatment of the hyperthyroidism with β-blockers and carbimazole along with physiotherapy resulted in the patient’s full recovery and the alleviation of his symptoms. Besides highlighting this rare association, this report underscores the importance of including thyroid function tests in the evaluation of patients with acute polyneuropathy.

  7. Acquired urethral diverticulum in a man with paraplegia presenting with a scrotal mass: a case report

    Directory of Open Access Journals (Sweden)

    El Ammari Jalal Eddine

    2012-11-01

    Full Text Available Abstract Introduction Male urethral diverticula are rare. Patients with paraplegia may present with acquired diverticula as a result of prolonged catheterization. Diverticula may be asymptomatic or lead to lower urinary tract symptoms. Rarely, the diverticulum may initially present as a scrotal mass. Case presentation We report the case of a male 45-year-old Arab with paraplegia who presented with a mass in the peno-scrotal junction. He had in his medical history iterative prolonged urethral catheterizations associated with urine leakage through the urethral meatus upon applying compression. Diagnosis confirmation of urethral diverticula is obtained by retrograde urethrography. The patient underwent a diverticulectomy with urethroplasty. Conclusion Male acquired urethral diverticula can be found in patients who have a spinal cord injury because of prolonged urethral catheterization. Clinical presentations are different and sometimes can be misleading. Retrograde urethrography is the key to diagnosis and open surgery is the treatment of reference.

  8. A comparison of 2 circuit exercise training techniques for eliciting matched metabolic responses in persons with paraplegia.

    Science.gov (United States)

    Nash, Mark S; Jacobs, Patrick L; Woods, Jeffrey M; Clark, James E; Pray, Tanya A; Pumarejo, Alex E

    2002-02-01

    To test whether acute metabolic (VO(2)), chronotropic (heart rate), and perceptual (rating of perceived exertion; RPE) responses to exercise by persons with paraplegia differ when the exercise is on a multistation isoinertial exercise system (MultiGym) or on a customized system of Thera-Band resistance bands (ElasticGym). Within-subjects comparison of 2 treatments. Academic medical center. Sixteen men and 1 woman with complete paraplegia (T4-L1), as defined by the American Spinal Injury Association. A circuit resistance training (CRT) program for persons with paraplegia was adapted to both a MultiGym and a customized ElasticGym. Exercises used for training and testing used 6 resistance maneuvers at 50% of the 1-repetition maximum (1-RM), with interposed rapid arm spinning. Subjects were habituated to both conditions for 2 weeks before testing on randomized nonconsecutive days. VO(2) (L/min) was measured by portable spirometry, heart rate (beats/min) by a chest strap monitor, and RPE by the Borg Scale of Perceived Exertion (6-20). No significant effects of test condition on average VO(2) or heart rate were observed, with differences between conditions reflecting only .08L/min and 6.4 beats/min, respectively. Average RPE was significantly higher in testing under the ElasticGym condition (P < .05). CRT on a customized ElasticGym system elicited acute metabolic and chronotropic responses that did not differ from responses to exercise on a MultiGym, though RPE was greater with the ElasticGym. Copyright 2002 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation

  9. Repetitive Transcranial Magnetic Stimulation in Patients with Hereditary Spastic Paraplegia

    Directory of Open Access Journals (Sweden)

    Mehmet Ağırman

    2011-06-01

    Full Text Available Hereditary spastic paraplegia (HSPP is a heterogeneous genetic disease characterized by progressive spasticity of lower extremities. Spasticity is a major cause of long-term disability in HSPP and significantly affects the functional life of patients. Repetitive transcranial magnetic stimulation (rTMS is widely used in diagnosis and treatment of many neurological and psychiatric diseases. Although the positive impacts of rTMS for spasticity have been reported, no study has been found on HSPP. We present two HSPP patients treated with low frequency rTMS (20 minutes at a frequency of 1 Hz (1200 pulses, for a period of 10 treatment sessions.

  10. Repetitive Transcranial Magnetic Stimulation in Patients with Hereditary Spastic Paraplegia

    Directory of Open Access Journals (Sweden)

    Mehmet Ağırman

    2011-06-01

    Full Text Available Hereditary spastic paraplegia (HSPP is a heterogeneous genetic disease characterized by progressive spasticity of lower extremities. Spasticity is a major cause of long-term disability in HSPP and significantly affects the functional life of patients. Repetitive transcranial magnetic stimulation (rTMS is widely used in diagnosis and treatment of many neurological and psychiatric diseases. Although the positive impacts of rTMS for spasticity have been reported, no study has been found on HSPP. We present two HSPP patients treated with low frequency rTMS (20 minutes at a frequency of 1 Hz (1200 pulses, for a period of 10 treatment sessions

  11. Does low self-esteem predict health compromising behaviours among adolescents?

    Science.gov (United States)

    Mcgee, R; Williams, S

    2000-10-01

    It is often believed that low self-esteem is associated with such health-compromising behaviours in adolescence as substance use, early sexual activity, eating problems and suicidal ideation. Surprisingly, there is little longitudinal research addressing this issue. This longitudinal study examines the predictive association between both global and academic self-esteem from ages 9 to 13 years, and a variety of health compromising behaviours at age 15, in a large sample of young New Zealanders. Levels of global self-esteem significantly predicted adolescent report of problem eating, suicidal ideation, and multiple health compromising behaviours. Earlier levels of self-esteem were unrelated to later substance use and early sexual activity. The findings are discussed in terms of their implications for efforts to raise self-esteem among young people. Copyright 2000 The Association for Professionals in Services for Adolescents.

  12. Physically Compromised and Physically Talented Children in Northeastern Slovenia

    Directory of Open Access Journals (Sweden)

    Planinšec Jurij

    2012-12-01

    Full Text Available The aim of our research was to determine the share of physically compromised and physically talented children in northeastern Slovenia. The sample comprised 621 children aged nine to eleven years, among which there were 316 girls (M=10; SD=0.8 and 305 boys (M=10; SD=0.81. In order to assess their motor skills, seven different tests were used, mostly from Eurofit test battery, which covered explosive power, repetitive power balance, eye–hand coordination, speed of simple movements, whole body coordination, and endurance. The assessment was made for each physical fitness test separately. The cut-off points for determination of physically compromised and physically talented children were set at -1SD and +1SD, respectively. The results of all physical fitness tests showed that the share of physically compromised children exceeded ten percent for both genders. The largest number of boys and girls were physically compromised with regard to endurance and balance, respectively. On the other hand, boys proved to be most physically talented with regard to endurance, and girls with regard to explosive power. Gender differences were most obvious with regard to general endurance, as 21 per cent of the boys were physically compromised as opposed to 13 per cent of the girls. As for physical talent, we observed less gender-related differences. The results indicate increasing differences in physical fitness among children from northeastern Slovenia. The implementation of curricular and extracurricular sports activities should aim at reducing the number of physically compromised children. On the other hand, it would make sense to encourage physically talented children to get involved in organized forms of exercise.

  13. Arsenite promotes centrosome abnormalities under a p53 compromised status induced by 4-(methylnitrosamino)-1-(3-pyridyl)-1-butanone (NNK)

    International Nuclear Information System (INIS)

    Liao, W.-T.; Yu, H.-S.; Lin Pinpin; Chang, Louis W.

    2010-01-01

    Epidemiological evidence indicated that residents, especially cigarette smokers, in arseniasis areas had significantly higher lung cancer risk than those living in non-arseniasis areas. Thus an interaction between arsenite and cigarette smoking in lung carcinogenesis was suspected. In the present study, we investigated the interactions of a tobacco-specific carcinogen 4- (methylnitrosamino)-1-(3-pyridyl)-1-butanone (nicotine-derived nitrosamine ketone, NNK) and arsenite on lung cell transformation. BEAS-2B, an immortalized human lung epithelial cell line, was selected to test the centrosomal abnormalities and colony formation by NNK and arsenite. We found that NNK, alone, could enhance BEAS-2B cell growth at 1-5 μM. Under NNK exposure, arsenite was able to increase centrosomal abnormality as compared with NNK or arsenite treatment alone. NNK treatment could also reduce arsenite-induced G2/M cell cycle arrest and apoptosis, these cellular effects were found to be correlated with p53 dysfunction. Increased anchorage-independent growth (colony formation) of BEAS-2B cells cotreated with NNK and arsenite was also observed in soft agar. Our present investigation demonstrated that NNK could provide a p53 compromised status. Arsenite would act specifically on this p53 compromised status to induce centrosomal abnormality and colony formation. These findings provided strong evidence on the carcinogenic promotional role of arsenite under tobacco-specific carcinogen co-exposure.

  14. Development of novel cilostazol-loaded solid SNEDDS using a SPG membrane emulsification technique: Physicochemical characterization and in vivo evaluation.

    Science.gov (United States)

    Mustapha, Omer; Kim, Kyung Soo; Shafique, Shumaila; Kim, Dong Shik; Jin, Sung Giu; Seo, Youn Gee; Youn, Yu Seok; Oh, Kyung Taek; Lee, Beom-Jin; Park, Young Joon; Yong, Chul Soon; Kim, Jong Oh; Choi, Han-Gon

    2017-02-01

    The objective of this study was to develop a novel solid self-nanoemulsifying drug delivery system (SNEDDS) using a membrane emulsification technique involving Shirasu porous glass (SPG) which produced very small and uniform emulsion droplets, resulting in enhanced solubility, dissolution and oral bioavailability of poorly water-soluble cilostazol. The effects of carriers on the drug solubility were assessed, and pseudo-ternary phase diagrams were plotted. Among the liquid SNEDDS formulations tested, the liquid SNEDDS composed of peceol (oil), Tween 20 (surfactant) and Labrasol (cosurfactant) at a weight ratio of 15/55/30, produced the smallest emulsion droplet size. The cilostazol-loaded liquid SNEDDS formulation was suspended in the distilled water and subjected to SPG membrane emulsification. Calcium silicate was added as a solid carrier in this liquid SNEDDS, completely suspended and spray-dried, leading to the production of a cilostazol-loaded solid SNEDDS. The emulsion droplet size, solubility and dissolution of the emulsified solid SNEDDS were assessed as compared to the solid SNEDDS prepared without emulsification. Moreover, the physicochemical characteristics and pharmacokinetics in rats were evaluated with the emulsified solid SNEDDS. The emulsified solid SNEDDS provided significantly smaller and more uniform nanoemulsions than did the non-emulsified solid SNEDDS. The emulsified solid SNEDDS showed significantly higher drug solubility and dissolution as compared to the non-emulsified solid SNEDDS. The crystalline drug in it was converted into the amorphous state. Moreover, in rats, it gave significantly higher initial plasma concentrations and AUC compared to the drug powder, suggesting its improved oral bioavailability of cilostazol. Thus, this novel solid SNEDDS developed using a membrane emulsification technique represents a potentially powerful oral delivery system for cilostazol. Copyright © 2016 Elsevier B.V. All rights reserved.

  15. Compromises.

    Science.gov (United States)

    Sizer, Theodore R.

    1984-01-01

    Taking as examples the issues of improving students'"high order thinking skills" and arriving at more equitable teacher salaries and school budgets, the author discusses the need for compromise solutions to widespread problems. (JBM)

  16. Sexualidade e paraplegia: o dito, o explícito e o oculto Sexualydad y paraplejía: lo dicho, lo explícito y lo oculto Sexuality and paraplegics: the saying, the explicit and the occult

    OpenAIRE

    Inacia Sátiro Xavier de França; Adriana de Freitas Chaves

    2005-01-01

    INTRODUÇÃO: A intenção de contribuir para o desenvolvimento de ações educativas que desmistifiquem mitos e tabus relacionados a sexualidade da pessoa portadora de paraplegia e melhorar a qualidade de vida desse tipo de cliente motivou a seleção do objeto "sexualidade da mulher portadora de paraplegia". OBJETIVO: Compreender a construção de sentidos das mulheres portadoras de paraplegia acerca da sexualidade e relatar as barreiras enfrentadas por estas mulheres para vivenciar sua sexualidade. ...

  17. Overlapping molecular pathological themes link Charcot-Marie-Tooth neuropathies and hereditary spastic paraplegias.

    Science.gov (United States)

    Timmerman, Vincent; Clowes, Virginia E; Reid, Evan

    2013-08-01

    In this review we focus on Charcot-Marie-Tooth (CMT) neuropathies and hereditary spastic paraplegias (HSPs). Although these diseases differ in whether they primarily affect the peripheral or central nervous system, both are genetically determined, progressive, long axonopathies that affect motor and sensory pathways. This commonality suggests that there might be similarities in the molecular pathology underlying these conditions, and here we compare the molecular genetics and cellular pathology of the two groups. Copyright © 2012 Elsevier Inc. All rights reserved.

  18. Clinical and socio-demographic characteristics of persons with traumatic paraplegia living in São Paulo, Brazil Características clínicas e sócio demográficas de pessoas com paraplegia traumática na cidade de São Paulo

    Directory of Open Access Journals (Sweden)

    Leila Blanes

    2009-06-01

    Full Text Available OBJECTIVE: To evaluate the clinical profile of individuals with paraplegia living in São Paulo, Brazil. METHOD: The sample consisted of 60 outpatients with traumatic paraplegia from whom clinical and demographic data were obtained. RESULTS: The patients were predominately men (86.7%, single (61.7%, with mean age of 32.9 (SD=9.47 years, and complete or incomplete primary education (63.3%. Although 41.7% were born in different states, all patients were current residents of São Paulo, Brazil. The most frequent cause of paraplegia was firearm injury (63.3% followed by car accident (20%. The most common complications observed in the patients were urinary (88.3% and anal (45% incontinence, muscle spasm (65%, and pressure ulcers (26.7%. CONCLUSION: The data revealed that the sample consisted predominantly of young males with low education level, showing complications due to SCI, and who were victims of urban violence.OBJETIVO: Avaliar o perfil de paraplégicos que vivem na cidade de São Paulo. MÉTODO: Foram incluídos 60 paraplégicos por causa traumática. O instrumento de coleta de dados constava de dados demográficos e clínicos. RESULTADOS: A maioria era do sexo masculino (86,7%, com média de idade igual a 32,9 (DP=9,47 anos, sendo 61,7% solteiros. Em relação à naturalidade 41,7% nasceram em outros estados, porém todos residiam em São Paulo. A causa mais freqüente da paraplegia foi por ferimento por arma de fogo (63,3%, seguida por acidente automobilístico (20%. Quanto às complicações decorrentes da lesão medular, destaca-se a presença de incontinência urinária (88,3% e anal (45%, o espasmo muscular (65% e a úlcera por pressão (26,7%. CONCLUSÃO: Os resultados encontrados permitiram verificar uma amostra de pessoas em sua maioria do sexo masculino, jovem, com baixa escolaridade e que apresentam complicações decorrentes da lesão medular e em grande parte, vítimas da violência dos grandes centros urbanos.

  19. S113R mutation in SLC33A1 leads to neurodegeneration and augmented BMP signaling in a mouse model

    Directory of Open Access Journals (Sweden)

    Pingting Liu

    2017-01-01

    Full Text Available The S113R mutation (c.339T>G (MIM #603690.0001 in SLC33A1 (MIM #603690, an ER membrane acetyl-CoA transporter, has been previously identified in individuals with hereditary spastic paraplegia type 42 (SPG42; MIM #612539. SLC33A1 has also been shown to inhibit the bone morphogenetic protein (BMP signaling pathway in zebrafish. To better understand the function of SLC33A1, we generated and characterized Slc33a1S113R knock-in mice. Homozygous Slc33a1S113R mutant mice were embryonic lethal, whereas heterozygous Slc33a1 mutant mice (Slc33a1wt/mut exhibited behavioral abnormalities and central neurodegeneration, which is consistent with hereditary spastic paraplegia (HSP phenotypes. Importantly, we found an upregulation of BMP signaling in the nervous system and mouse embryonic fibroblasts of Slc33a1wt/mut mice. Using a sciatic nerve crush injury model in vivo and dorsal root ganglion (DRG culture in vitro we showed that injury-induced axonal regeneration in Slc33a1wt/mut mice was accelerated and mediated by upregulated BMP signaling. Exogenous addition of BMP signaling antagonist, noggin, could efficiently alleviate the accelerated injury-induced axonal regrowth. These results indicate that SLC33A1 can negatively regulate BMP signaling in mice, further supporting the notion that upregulation of BMP signaling is a common mechanism of a subset of hereditary spastic paraplegias.

  20. Sports practice, resilience, body and sexual esteem, and higher educational level are associated with better sexual adjustment in men with acquired paraplegia.

    Science.gov (United States)

    Dos Passos Porto, Isabela; Cardoso, Fernando Luiz; Sacomori, Cinara

    2016-10-12

    To analyse the association of team sports practice and physical and psychological factors with sexual adjustment in men with paraplegia. More specifically, we aimed to compare athletes and non-athletes regarding sexual adjustment, resilience, body and sexual self-esteem, and functional independence. Cross-sectional study with a paired design. The study included 60 men with paraplegia (30 athletes and 30 non-athletes). We used a sociodemographic questionnaire (age, education, and time since injury); a physical and sexual esteem questionnaire; a resilience questionnaire; and Functional Independence Measure (FIM). The dependent variable, sexual adjustment, was determined by the sum of 5 questions about sexual frequency, desire, and satisfaction and physical and psychological adjustment. Data were analysed by using the χ2 test, Wilcoxon's test, Spearman's correlation test, and hierarchical multiple linear regression analysis, with p Athletes had significantly higher sexual adjustment (p = 0.001) and higher body and sexual esteem (p esteem, higher educational level, and higher resilience levels (R2 = 58%). There was an interaction between sports practice and body and sexual esteem (p = 0.024; R2 = 62%). Participation in sports influenced the sexual adjustment of the men with paraplegia, even when controlled for psychological (resilience and body and sexual esteem) and physical (functional independence) aspects.

  1. Voluntary Ambulation by Upper Limb-Triggered HAL® in Patients with Complete Quadri/Paraplegia Due to Chronic Spinal Cord Injury.

    Science.gov (United States)

    Shimizu, Yukiyo; Kadone, Hideki; Kubota, Shigeki; Suzuki, Kenji; Abe, Tetsuya; Ueno, Tomoyuki; Soma, Yuichiro; Sankai, Yoshiyuki; Hada, Yasushi; Yamazaki, Masashi

    2017-01-01

    Patients with complete paraplegia after spinal cord injury (SCI) are unable to stand or walk on their own. Standing exercise decreases the risk of decubitus ulcers, osteoporosis, and joint deformities in patients with SCI. Conventional gait training for complete paraplegia requires excessive upper limb usage for weight bearing and is difficult in cases of complete quadriplegia. The purpose of this study was to describe voluntary ambulation triggered by upper limb activity using the Hybrid Assistive Limb® (HAL) in patients with complete quadri/paraplegia after chronic SCI. Four patients (3 men, 1 woman) were enrolled in this study. The mean patient age ± standard deviation was 37.2 ± 17.8 (range, 20-67) years. Clinical evaluation before intervention revealed the following findings: case 1, neurological level C6, American Spinal Cord Injury Association impairment scale (AIS) grade B; case 2, T6, AIS A; case 3, T10 AIS A; and case 4, T11, AIS A. The HAL intervention consisted of 10 sessions. Each HAL session lasted 60-90 min. The HAL electrodes for hip and knee flexion-extension were placed on the anterior and posterior sides of the upper limbs contralaterally corresponding to each of the lower limbs. Surface electromyography (EMG) was used to evaluate muscle activity of the tensor fascia lata and quadriceps femoris (Quad) in synchronization with a Vicon motion capture system. The modified Ashworth scale (mAs) score was also evaluated before and after each session. All participants completed all 10 sessions. Cases 1, 2, and 3 demonstrated significant decreases in mAs score after the sessions compared to pre-session measurements. In all cases, EMG before the intervention showed no apparent activation in either Quad. However, gait phase dependent activity of the lower limb muscles was seen during voluntarily triggered ambulation driven by upper limb muscle activities. In cases 3 and 4, active contraction in both Quads was observed after intervention. These findings

  2. Voluntary Ambulation by Upper Limb-Triggered HAL® in Patients with Complete Quadri/Paraplegia Due to Chronic Spinal Cord Injury

    Directory of Open Access Journals (Sweden)

    Yukiyo Shimizu

    2017-11-01

    Full Text Available Patients with complete paraplegia after spinal cord injury (SCI are unable to stand or walk on their own. Standing exercise decreases the risk of decubitus ulcers, osteoporosis, and joint deformities in patients with SCI. Conventional gait training for complete paraplegia requires excessive upper limb usage for weight bearing and is difficult in cases of complete quadriplegia. The purpose of this study was to describe voluntary ambulation triggered by upper limb activity using the Hybrid Assistive Limb® (HAL in patients with complete quadri/paraplegia after chronic SCI. Four patients (3 men, 1 woman were enrolled in this study. The mean patient age ± standard deviation was 37.2 ± 17.8 (range, 20–67 years. Clinical evaluation before intervention revealed the following findings: case 1, neurological level C6, American Spinal Cord Injury Association impairment scale (AIS grade B; case 2, T6, AIS A; case 3, T10 AIS A; and case 4, T11, AIS A. The HAL intervention consisted of 10 sessions. Each HAL session lasted 60–90 min. The HAL electrodes for hip and knee flexion-extension were placed on the anterior and posterior sides of the upper limbs contralaterally corresponding to each of the lower limbs. Surface electromyography (EMG was used to evaluate muscle activity of the tensor fascia lata and quadriceps femoris (Quad in synchronization with a Vicon motion capture system. The modified Ashworth scale (mAs score was also evaluated before and after each session. All participants completed all 10 sessions. Cases 1, 2, and 3 demonstrated significant decreases in mAs score after the sessions compared to pre-session measurements. In all cases, EMG before the intervention showed no apparent activation in either Quad. However, gait phase dependent activity of the lower limb muscles was seen during voluntarily triggered ambulation driven by upper limb muscle activities. In cases 3 and 4, active contraction in both Quads was observed after intervention

  3. Machado-Joseph disease versus hereditary spastic paraplegia: case report Doença de Machado-Joseph versus paraplegia espástica hereditária: relato de caso

    Directory of Open Access Journals (Sweden)

    Hélio A. Ghizoni Teive

    2001-09-01

    Full Text Available Machado-Joseph disease (MJD is the most common autosomal dominant spinocerebellar ataxia and presents great phenotypic variability. MJD presenting with spastic paraparesis was recently described in Japanese patients. We report the case of 41-year-old woman with the phenotype of complicated hereditary spastic paraplegia. Her father died at the age of 56 years due to an undiagnosed progressive neurological disease that presented parkinsonism. She had an expanded allele with 66 CAG repeats and a normal allele with 22 repeats in the gene of MJD. MJD should be considered in the differential diagnosis of autosomal dominant complicated HSP. A patient with the phenotype of complicated HSP and relatives with other clinical features of a neurodegenerative disease should raise the suspicion of MJD.A doença de Machado-Joseph (DMJ é a ataxia espinocerebelar autossômica dominante mais prevalente e apresenta grande variabilidade fenotípica. DMJ apresentando-se com o fenótipo de paraparesia espástica foi recentemente descrita em pacientes japoneses. Relatamos o caso de uma paciente com fenótipo de paraplegia espástica hereditária (PEH "complicada". Seu pai faleceu com 56 anos de uma doença neurológica progressiva que cursava com parkinsonismo. Estudo genético pela técnica de reação em cadeia da polimerase mostrou um alelo de tamanho normal com 22 repetições e um alelo de tamanho expandido com 66 repetições para o tripleto CAG no gene da DMJ. A DMJ deve ser considerada no diagnóstico diferencial das PEH complicadas autossômicas dominantes. Em um paciente com quadro de PEH e outros familiares com fenótipos diferentes de doenças degenerativas deve-se suspeitar de DMJ.

  4. Comparison of 24-hour cardiovascular and autonomic function in paraplegia, tetraplegia, and control groups: implications for cardiovascular risk.

    Science.gov (United States)

    Rosado-Rivera, Dwindally; Radulovic, M; Handrakis, John P; Cirnigliaro, Christopher M; Jensen, A Marley; Kirshblum, Steve; Bauman, William A; Wecht, Jill Maria

    2011-01-01

    Fluctuations in 24-hour cardiovascular hemodynamics, specifically heart rate (HR) and blood pressure (BP), are thought to reflect autonomic nervous system (ANS) activity. Persons with spinal cord injury (SCI) represent a model of ANS dysfunction, which may affect 24-hour hemodynamics and predispose these individuals to increased cardiovascular disease risk. To determine 24-hour cardiovascular and ANS function among individuals with tetraplegia (n=20; TETRA: C4-C8), high paraplegia (n=10; HP: T2-T5), low paraplegia (n=9; LP: T7-T12), and non-SCI controls (n=10). Twenty-four-hour ANS function was assessed by time domain parameters of heart rate variability (HRV); the standard deviation of the 5-minute average R-R intervals (SDANN; milliseconds/ms), and the root-mean square of the standard deviation of the R-R intervals (rMSSD; ms). Subjects wore 24-hour ambulatory monitors to record HR, HRV, and BP. Mixed analysis of variance (ANOVA) revealed significantly lower 24-hour BP in the tetraplegic group; however, BP did not differ between the HP, LP, and control groups. Mixed ANOVA suggested significantly elevated 24-hour HR in the HP and LP groups compared to the TETRA and control groups (Pcontrol groups (Pcontrol groups (P<0.01). Twenty-four-hour SDANN was significantly increased in the HP group compared to the LP and TETRA groups (P<0.05) and rMSSD was significantly lower in the LP compared to the other three groups (P<0.05). Elevated 24-hour HR in persons with paraplegia, in concert with altered HRV dynamics, may impart significant adverse cardiovascular consequences, which are currently unappreciated.

  5. Recessive loss-of-function mutations in AP4S1 cause mild fever-sensitive seizures, developmental delay and spastic paraplegia through loss of AP-4 complex assembly

    DEFF Research Database (Denmark)

    Hardies, Katia; May, Patrick; Djémié, Tania

    2015-01-01

    We report two siblings with infantile onset seizures, severe developmental delay and spastic paraplegia, in whom whole-genome sequencing revealed compound heterozygous mutations in the AP4S1 gene, encoding the σ subunit of the adaptor protein complex 4 (AP-4). The effect of the predicted loss-of-...... in reported patients, highlighting that seizures are part of the clinical manifestation of the AP-4 deficiency syndrome. We also hypothesize that endosomal trafficking is a common theme between heritable spastic paraplegia and some inherited epilepsies....

  6. Active lower limb orthosis with one degree of freedom for people with paraplegia.

    Science.gov (United States)

    Gloger, Michal; Obinata, Goro; Genda, Eiichi; Babjak, Jan; Pei, Yanling

    2017-07-01

    The main challenges of designing devices for paraplegic walking can be summarized into three groups, stability and comfort, high efficiency or low energy consumption, dimensions and weight. A new economical device for people with paraplegia which tackles all problems of the three groups is introduced in this paper. The main idea of this device is based on HALO mechanism. HALO is compact passive medial hip joint orthosis with contralateral hip and ankle linkage, which keeps the feet always parallel to the ground and assists swinging the leg. The medial hip joint is equipped with one actuator in the new design and the new orthosis is called @halo. Due to this update, we can achieve more stable and smoother walking patterns with decreased energy consumption of the users, yet maintain its compact and lightweight features. It is proven by the results from preliminary experiments with able-bodied subjects during which the same device with and without actuator was evaluated. Waddling and excessive vertical elevation of the center of gravity were decreased by 40% with significantly smaller standard deviations in case of the active orthosis. There was 52% less energy spent by the user wearing @halo which was calculated from the vertical excursion difference. There was measured 38.5% bigger impulse in crutches while using passive orthosis. The new @halo device is the first active orthosis for lower limbs with just one actuated degree of freedom for users with paraplegia.

  7. Compromise and Disagreement in Comtemporary Political Theory

    DEFF Research Database (Denmark)

    Rostbøll, Christian F.; Scavenius, Theresa

    Compromise and Disagreement in Contemporary Political Theory provides a critical discussion of when and to what extent compromise is the best response to pluralism and disagreement in democratic decision-making and beyond. Organized into four parts, the book begins by discussing the justification...... and limits of compromise. Part II discusses the practice of compromise and considers the ethics required for compromise as well as the institutions that facilitate compromise. Part III focuses on pluralism and connects the topic of compromise to current discussions in political theory on public reason...

  8. Cognitive dysfunction in hereditary spastic paraplegias and other motor neuron disorders

    Directory of Open Access Journals (Sweden)

    Ingrid Faber

    Full Text Available ABSTRACT Hereditary spastic paraplegia (HSP is a diverse group of single-gene disorders that share the predominant clinical feature of progressive lower limb spasticity and weakness. More than 70 different genetic subtypes have been described and all modes of inheritance are possible. Intellectual dysfunction in HSP is frequent in recessive forms but rare in dominant families. It may manifest by either mental retardation and/or cognitive decline. The latter may be subtle, restricted to executive dysfunction or may evolve to severe dementia. The cognitive profile is thought to depend largely on the genetic subtype of HSP, although wide phenotypic variability within the same genetic subtype and also within the same family can be found.

  9. Targeted high-throughput sequencing identifies mutations in atlastin-1 as a cause of hereditary sensory neuropathy type I.

    Science.gov (United States)

    Guelly, Christian; Zhu, Peng-Peng; Leonardis, Lea; Papić, Lea; Zidar, Janez; Schabhüttl, Maria; Strohmaier, Heimo; Weis, Joachim; Strom, Tim M; Baets, Jonathan; Willems, Jan; De Jonghe, Peter; Reilly, Mary M; Fröhlich, Eleonore; Hatz, Martina; Trajanoski, Slave; Pieber, Thomas R; Janecke, Andreas R; Blackstone, Craig; Auer-Grumbach, Michaela

    2011-01-07

    Hereditary sensory neuropathy type I (HSN I) is an axonal form of autosomal-dominant hereditary motor and sensory neuropathy distinguished by prominent sensory loss that leads to painless injuries. Unrecognized, these can result in delayed wound healing and osteomyelitis, necessitating distal amputations. To elucidate the genetic basis of an HSN I subtype in a family in which mutations in the few known HSN I genes had been excluded, we employed massive parallel exon sequencing of the 14.3 Mb disease interval on chromosome 14q. We detected a missense mutation (c.1065C>A, p.Asn355Lys) in atlastin-1 (ATL1), a gene that is known to be mutated in early-onset hereditary spastic paraplegia SPG3A and that encodes the large dynamin-related GTPase atlastin-1. The mutant protein exhibited reduced GTPase activity and prominently disrupted ER network morphology when expressed in COS7 cells, strongly supporting pathogenicity. An expanded screen in 115 additional HSN I patients identified two further dominant ATL1 mutations (c.196G>C [p.Glu66Gln] and c.976 delG [p.Val326TrpfsX8]). This study highlights an unexpected major role for atlastin-1 in the function of sensory neurons and identifies HSN I and SPG3A as allelic disorders.

  10. Full Body Gait Analysis May Improve Diagnostic Discrimination Between Hereditary Spastic Paraplegia and Spastic Diplegia: A Preliminary Study

    Science.gov (United States)

    Bonnefoy-Mazure, A.; Turcot, K.; Kaelin, A.; De Coulon, G.; Armand, S.

    2013-01-01

    Hereditary spastic paraplegia (HSP) and spastic diplegia (SD) patients share a strong clinical resemblance. Thus, HSP patients are frequently misdiagnosed with a mild form of SD. Clinical gait analysis (CGA) has been highlighted as a possible tool to support the differential diagnosis of HSP and SD. Previous analysis has focused on the lower-body…

  11. Short-term effects of upper extremity circuit resistance training on muscle strength and functional independence in patients with paraplegia.

    Science.gov (United States)

    Yildirim, Adem; Sürücü, Gülseren Dost; Karamercan, Ayşe; Gedik, Dilay Eken; Atci, Nermin; Dülgeroǧlu, Deniz; Özgirgin, Neşe

    2016-11-21

    A number of exercises to strengthen the upper extremities are recommended to increase functional independence and quality of life (QoL) in patients with paraplegia. Circuit resistance training (CRT) is a type of progressive resistive exercise performed repeatedly at fixed mechanical exercise stations. The aim of this study was to investigate the potential benefits of CRT for upper extremity muscle strength, functional independence, and QoL in patients with paraplegia. Twenty-six patients with paraplegia who were participating in a conventional rehabilitation program at a tertiary education and research hospital were enrolled in this study. The participants were randomly assigned to two groups. The exercise group participated in the CRT program, which consisted of repetitive exercises for the upper extremities performed at fixed mechanical stations 5 sessions per week for 6 weeks, in addition to conventional rehabilitation. Participants in the control group received only conventional rehabilitation over the same period. We compared the groups with respect to QoL, as well as isokinetic muscle test outcomes in the upper extremities, using the Functional Independence Measure (FIM) and Borg's scale. We observed significant increases in scores on the physical component of the FIM, Borg's scale, and QoL in both the exercise and control groups. Furthermore, the large majority of isokinetic values were significantly more improved in the exercise group compared to the control group. When post-treatment outcomes were compared between the groups, improvements in scores on the physical component of the FIM and in most isokinetic values were significantly greater in the exercise group. This study showed that CRT has positive effects on muscle strength in the upper extremities and the physical disability components of the FIM when added to conventional rehabilitation programs for paraplegic patients. However, we observed no significant improvement in QoL scores after adding CRT

  12. A novel ABCD1 mutation detected by next generation sequencing in presumed hereditary spastic paraplegia: A 30-year diagnostic delay caused by misleading biochemical findings.

    Science.gov (United States)

    Koutsis, Georgios; Lynch, David S; Tucci, Arianna; Houlden, Henry; Karadima, Georgia; Panas, Marios

    2015-08-15

    To present a Greek family in which 5 male and 2 female members developed progressive spastic paraplegia. Plasma very long chain fatty acids (VLCFA) were reportedly normal at first testing in an affected male and for over 30 years the presumed diagnosis was hereditary spastic paraplegia (HSP). Targeted next generation sequencing (NGS) was used as a further diagnostic tool. Targeted exome sequencing in the proband, followed by Sanger sequencing confirmation; mutation segregation testing in multiple family members and plasma VLCFA measurement in the proband. NGS of the proband revealed a novel frameshift mutation in ABCD1 (c.1174_1178del, p.Leu392Serfs*7), bringing an end to diagnostic uncertainty by establishing the diagnosis of adrenomyeloneuropathy (AMN), the myelopathic phenotype of X-linked adrenoleukodystrophy (ALD). The mutation segregated in all family members and the diagnosis of AMN/ALD was confirmed by plasma VLCFA measurement. Confounding factors that delayed the diagnosis are presented. This report highlights the diagnostic utility of NGS in patients with undiagnosed spastic paraplegia, establishing a molecular diagnosis of AMN, allowing proper genetic counseling and management, and overcoming the diagnostic delay that can be rarely caused by false negative VLCFA analysis. Copyright © 2015 Elsevier B.V. All rights reserved.

  13. Car transfer and wheelchair loading techniques in independent drivers with paraplegia

    Directory of Open Access Journals (Sweden)

    Lisa Lighthall Haubert

    2015-09-01

    Full Text Available Car transfers and wheelchair (WC loading are crucial for independent community participation in persons with complete paraplegia from spinal cord injury, but are complex, physically demanding, and known to provoke shoulder pain. This study aimed to describe techniques and factors influencing car transfer and WC loading for individuals with paraplegia driving their own vehicles and using their personal WCs. Sedans were the most common vehicle driven (59%. Just over half (52% of drivers place their right leg only into the vehicle prior to transfer. Overall, the leading hand was most frequently placed on the driver’s seat (66% prior to transfer and the trailing hand was most often place on the WC seat (48%. Vehicle height influenced leading hand placement but not leg placement such that driver’s of higher profile vehicles were more likely to place their hand on the driver’s seat than those who drove sedans. Body lift time was negatively correlated with level of injury and age and positively correlated with vehicle height and shoulder abduction strength. Drivers who transferred with their leading hand on the steering wheel had significantly higher levels of shoulder pain than those who placed their hand on the driver’s seat or overhead. The majority of participants used both hands (62% to load their WC frame and, overall, most loaded their fame into the back (62% vs. the front seat. Sedan drivers were more likely to load their frame into the front seat than drivers of higher profile vehicles (53% vs. 17%. Average time to load the WC frame (10.7 seconds was 20% of the total WC loading time and was not related to shoulder strength, frame weight or demographic characteristics. Those who loaded their WC frame into the back seat had significantly weaker right shoulder internal rotators. Understanding car transfers and WC loading in independent drivers is crucial to prevent shoulder pain and injury and preserve community participation.

  14. MRI of autosomal dominant pure spastic paraplegia

    International Nuclear Information System (INIS)

    Krabbe, K.; Fallentin, E.; Herning, M.; Nielsen, J.E.; Fenger, K.

    1997-01-01

    We examined 16 patients with autosomal dominant pure spastic paraplegia (HSP) and 15 normal controls matched for age and sex using MRI of the brain and spinal cord. Images were assessed qualitatively by two independent radiologists, blinded to the clinical diagnosis. Areas of the brain and corpus callosum on one midsagittal slice and the area of the brain on one axial slice were measured and a ''corpus-callosum index'' expressing the size of the corpus callosum relative to that of the brain was calculated. Cross-sectional areas and anteroposterior and transverse diameters of the spinal cord at the levels of C 2, C 5, T 3, T 6, T 9 and T 11 were measured. No significant differences between patients and controls were found on qualitative evaluation of the images. The patients had a significantly smaller corpus callosum and ''corpus-callosum index'' than controls. This finding, not reported previously, might indicate that the disease process in pure HSP is not confined to the spinal cord. The anteroposterior diameters of the spinal cord at T 3 and T 9 were significantly smaller in patients than in controls. This might correspond to the degeneration of the pyramidal tracts and the dorsal columns described at neuropathological examination. (orig.). With 1 fig., 3 tabs

  15. MRI of autosomal dominant pure spastic paraplegia

    Energy Technology Data Exchange (ETDEWEB)

    Krabbe, K.; Fallentin, E.; Herning, M. [Danish Research Center of Magnetic Resonance, Hvidovre Hospital, Kettegaard alle 30, DK-2650 Hvidovre (Denmark); Nielsen, J.E.; Fenger, K. [Institute of Medical Biochemistry and Genetics, Laboratory of Medical Genetics, Section of Neurogenetics, University of Copenhagen (Denmark)

    1997-10-01

    We examined 16 patients with autosomal dominant pure spastic paraplegia (HSP) and 15 normal controls matched for age and sex using MRI of the brain and spinal cord. Images were assessed qualitatively by two independent radiologists, blinded to the clinical diagnosis. Areas of the brain and corpus callosum on one midsagittal slice and the area of the brain on one axial slice were measured and a ``corpus-callosum index`` expressing the size of the corpus callosum relative to that of the brain was calculated. Cross-sectional areas and anteroposterior and transverse diameters of the spinal cord at the levels of C 2, C 5, T 3, T 6, T 9 and T 11 were measured. No significant differences between patients and controls were found on qualitative evaluation of the images. The patients had a significantly smaller corpus callosum and ``corpus-callosum index`` than controls. This finding, not reported previously, might indicate that the disease process in pure HSP is not confined to the spinal cord. The anteroposterior diameters of the spinal cord at T 3 and T 9 were significantly smaller in patients than in controls. This might correspond to the degeneration of the pyramidal tracts and the dorsal columns described at neuropathological examination. (orig.). With 1 fig., 3 tabs.

  16. How moral disagreement may ground principled moral compromise

    DEFF Research Database (Denmark)

    Kappel, Klemens

    2018-01-01

    In an influential article, Simon C. May forcefully argued that, properly understood, there can never be principled reasons for moral compromise (May, 2005). While there may be pragmatic reasons for compromising that involve, for instance, concern for political expediency or for stability, there a......In an influential article, Simon C. May forcefully argued that, properly understood, there can never be principled reasons for moral compromise (May, 2005). While there may be pragmatic reasons for compromising that involve, for instance, concern for political expediency or for stability......, there are properly speaking no principled reasons to compromise. My aim in the article is to show how principled moral compromise in the context of moral disagreements over policy options is possible. I argue that when we disagree, principled reasons favoring compromises or compromising can assume a more significant...... part of what makes a position all things considered best, and in this way disagreement can ground moral compromise....

  17. Modeling of axonal endoplasmic reticulum network by spastic paraplegia proteins.

    Science.gov (United States)

    Yalçın, Belgin; Zhao, Lu; Stofanko, Martin; O'Sullivan, Niamh C; Kang, Zi Han; Roost, Annika; Thomas, Matthew R; Zaessinger, Sophie; Blard, Olivier; Patto, Alex L; Sohail, Anood; Baena, Valentina; Terasaki, Mark; O'Kane, Cahir J

    2017-07-25

    Axons contain a smooth tubular endoplasmic reticulum (ER) network that is thought to be continuous with ER throughout the neuron; the mechanisms that form this axonal network are unknown. Mutations affecting reticulon or REEP proteins, with intramembrane hairpin domains that model ER membranes, cause an axon degenerative disease, hereditary spastic paraplegia (HSP). We show that Drosophila axons have a dynamic axonal ER network, which these proteins help to model. Loss of HSP hairpin proteins causes ER sheet expansion, partial loss of ER from distal motor axons, and occasional discontinuities in axonal ER. Ultrastructural analysis reveals an extensive ER network in axons, which shows larger and fewer tubules in larvae that lack reticulon and REEP proteins, consistent with loss of membrane curvature. Therefore HSP hairpin-containing proteins are required for shaping and continuity of axonal ER, thus suggesting roles for ER modeling in axon maintenance and function.

  18. Paraplegia after myocardial revascularization. Case report.

    Science.gov (United States)

    Nigro Neto, Caetano; Iza, Milton Patricio Chango; Tardelli, Maria Angela

    2010-01-01

    Developments in anesthesiology have improved safety indices. Several techniques and agents are used to control the hemodynamic response and minimize adverse effects triggered by surgical stimuli in patients undergoing cardiac procedures. This is a 70 years old male patient, 1.74 m, 75 kg, ASA III, and NYHA II. The patient had controlled dyslipedemia, type II diabetes mellitus, and hypertension; history of smoking, peripheral vascular disease, and myocardial infarction 20 years ago. The patient underwent revascularization with the left internal mammary artery and saphenous grafts with extracorporeal circulation with intermittent clamping of the aorta. During the first 24 hours in the ICU, the patient developed hemodynamic instability, sudden hypotension, and atrial fibrillation. Twenty-six hours after the end of the surgery, the patient was awake, hemodynamically stable, and with good respiratory dynamics, being extubated. The patient was talkative and oriented, but immobile and negative reflexes in the lower limbs. Neurological evaluation showed: cranial nerves without changes, no complaints of pain below the hips, preserved superficial and deep sensitivity, adequate distal perfusion without edema, and flaccid paraplegia below T8. The echocardiogram did not show any changes. CT scan of the lumbosacral spine was negative for compressive mass in the epidural space or adjacent to it. Anterior spinal artery syndrome should be considered in procedures with manipulation of the aorta. Prevention, especially in patients at risk, is necessary. Computed tomography, for the differential diagnosis, and MRI, to localize the lesion, are important.

  19. The DALY, context and the determinants of the severity of disease: an exploratory comparison of paraplegia in Australia and Cameroon.

    Science.gov (United States)

    Allotey, Pascale; Reidpath, Daniel; Kouamé, Aka; Cummins, Robert

    2003-09-01

    This paper summarises the findings of an empirical investigation of some of the technical and social assumptions on which the disability adjusted life year (DALY) is based. The objectives of the study were to examine the notion that the burden of disease is broadly similar without regard to country, environment, gender or socio-economic status and to develop detailed descriptions of the experiences of the burden of disease as they related to these contextual factors. The study was a multi-factorial exploratory study employing qualitative and quantitative techniques to obtain data on the effects of country (development), environment (urban versus rural), gender and socio-economic status on people with paraplegia. The data provided an extensive and detailed compilation of context rich descriptions of living with paraplegia. Striking features of the data were the differences between countries with respect to the impact of the health conditions on functioning and highlight a context in which paraplegia of like clinical severity can be fatal in one environment and not in another. While there has been some focus on the control of social determinants of disease, there has been little work on the social determinants of the severity of disease. The underlying assumptions of the DALY, which ignore context in the assessment of the burden of disease, risk exacerbating inequalities by undervaluing the burden of disease in less-developed countries. There is a need to continue to subject the development of indicators to rigorous debate to determine a balance between the assumption of a global "average social milieu" and the treatment of each individual as belonging to their own context in the assessment of population health in order for indicators to be meaningful cross-culturally.

  20. Spastin-Interacting Protein NA14/SSNA1 Functions in Cytokinesis and Axon Development

    Science.gov (United States)

    Chang, Jaerak; Blackstone, Craig

    2014-01-01

    Hereditary spastic paraplegias (HSPs) are a genetically diverse group of inherited neurological disorders (SPG1-72) with the cardinal feature of prominent lower-extremity spasticity due to a length-dependent axonopathy of corticospinal motor neurons. The most frequent form of autosomal dominant HSP results from mutations of the SPG4 gene product spastin. This is an ATPase associated with diverse cellular activities (AAA) protein that binds to and severs microtubules. While spastin participates in crucial cellular processes such as cytokinesis, endosomal tubulation, and axon development, its role in HSP pathogenesis remains unclear. Spastin interacts in cells with the NA14 protein, a major target for auto-antibodies in Sjögren's syndrome (nuclear autoantigen 1; SSNA1). Our analysis of endogenous spastin and NA14 proteins in HeLa cells and rat cortical neurons in primary culture revealed a clear distribution of both proteins to centrosomes, with NA14 localizing specifically to centrioles. Stable NA14 knockdown in cell lines dramatically affected cell division, in particular cytokinesis. Furthermore, overexpression of NA14 in neurons significantly increased axon outgrowth and branching, while also enhancing neuronal differentiation. We postulate that NA14 may act as an adaptor protein regulating spastin localization to centrosomes, temporally and spatially regulating the microtubule-severing activity of spastin that is particularly critical during the cell cycle and neuronal development. PMID:25390646

  1. Spastin-interacting protein NA14/SSNA1 functions in cytokinesis and axon development.

    Directory of Open Access Journals (Sweden)

    Uma Goyal

    Full Text Available Hereditary spastic paraplegias (HSPs are a genetically diverse group of inherited neurological disorders (SPG1-72 with the cardinal feature of prominent lower-extremity spasticity due to a length-dependent axonopathy of corticospinal motor neurons. The most frequent form of autosomal dominant HSP results from mutations of the SPG4 gene product spastin. This is an ATPase associated with diverse cellular activities (AAA protein that binds to and severs microtubules. While spastin participates in crucial cellular processes such as cytokinesis, endosomal tubulation, and axon development, its role in HSP pathogenesis remains unclear. Spastin interacts in cells with the NA14 protein, a major target for auto-antibodies in Sjögren's syndrome (nuclear autoantigen 1; SSNA1. Our analysis of endogenous spastin and NA14 proteins in HeLa cells and rat cortical neurons in primary culture revealed a clear distribution of both proteins to centrosomes, with NA14 localizing specifically to centrioles. Stable NA14 knockdown in cell lines dramatically affected cell division, in particular cytokinesis. Furthermore, overexpression of NA14 in neurons significantly increased axon outgrowth and branching, while also enhancing neuronal differentiation. We postulate that NA14 may act as an adaptor protein regulating spastin localization to centrosomes, temporally and spatially regulating the microtubule-severing activity of spastin that is particularly critical during the cell cycle and neuronal development.

  2. Energy policy - compromise or change?

    International Nuclear Information System (INIS)

    Lewitz, J.C.

    1998-01-01

    Is there a contrast between compromise and change? In the author's opinion, there is not. Society changes. Sometimes, change is brought about more easily on the basis of a compromise. A pluralistic society needs compromises in order to participate in change with convictions of its own. To reach a compromise including a large part of one's conviction, the public must be convinced. This is achieved most easily by somebody who is well informed, educated, and trained. In this respect, not only specialized knowledge counts, but also the ability to handle the language and to know how to speak, and the behavior in public. Expert knowledge is acquired at the university, in the execution of one's profession, and in advanced training. Knowledge may be enhanced, for instance, by dealing with the arguments used by the other side. This will help in arguing one's own point more effectively. Individual talks, and events at which information and knowledge are disseminated, enable persons to assume an opinion based on their comprehension. Many uniform opinions create a majority opinion. (orig.) [de

  3. Voluntary ambulation using voluntary upper limb muscle activity and Hybrid Assistive Limb® (HAL®) in a patient with complete paraplegia due to chronic spinal cord injury: A case report.

    Science.gov (United States)

    Shimizu, Yukiyo; Kadone, Hideki; Kubota, Shigeki; Suzuki, Kenji; Saotome, Kousaku; Ueno, Tomoyuki; Abe, Tetsuya; Marushima, Aiki; Watanabe, Hiroki; Endo, Ayumu; Tsurumi, Kazue; Ishimoto, Ryu; Matsushita, Akira; Koda, Masao; Matsumura, Akira; Sankai, Yoshiyuki; Hada, Yasushi; Yamazaki, Masashi

    2018-01-19

    We sought to describe our experience with the Hybrid Assistive Limb® (HAL®) for active knee extension and voluntary ambulation with remaining muscle activity in a patient with complete paraplegia after spinal cord injury. A 30-year-old man with complete paraplegia used the HAL® for 1 month (10 sessions) using his remaining muscle activity, including hip flexor and upper limb activity. Electromyography was used to evaluate muscle activity of the gluteus maximus, tensor fascia lata, quadriceps femoris, and hamstring muscles in synchronization with the Vicon motion capture system. A HAL® session included a knee extension session with the hip flexor and voluntary gait with upper limb activity. After using the HAL® for one month, the patient's manual muscle hip flexor scores improved from 1/5 to 2/5 for the right and from 2/5 to 3/5 for the left knee, and from 0/5 to 1/5 for the extension of both knees. Knee extension sessions with HAL®, and hip flexor and upper-limb-triggered HAL® ambulation seem a safe and feasible option in a patient with complete paraplegia due to spinal cord injury.

  4. Cerebral metabolic and structural alterations in hereditary spastic paraplegia with thin corpus callosum assessed by MRS and DTI

    International Nuclear Information System (INIS)

    Dreha-Kulaczewski, Steffi; Dechent, Peter; Helms, Gunther; Frahm, Jens; Gaertner, Jutta; Brockmann, Knut

    2006-01-01

    Hereditary spastic paraplegia with thin corpus callosum (HSP-TCC) is a complicated form of autosomal-recessive hereditary spastic paraplegia. Characteristic clinical features comprise progressive spastic gait, cognitive impairment, and ataxia. Diagnostic MRI findings include thinning of the corpus callosum and non-progressive white matter (WM) alterations. To study the extent of axonal involvement, we performed localized proton magnetic resonance spectroscopy (MRS) of the cerebral WM and cortical grey matter (GM) in a patient with HSP-TCC at 20 and 25 years of age. The second investigation included diffusion tensor imaging (DTI). While MRS of the GM was normal, affected WM was characterized by major metabolic alterations such as reduced concentrations of N-acetylaspartate and N-acetylaspartyl-glutamate, creatine and phosphocreatine, and choline-containing compounds as well as elevated levels of myo-inositol. These abnormalities showed progression over a period of 5 years. DTI revealed increased mean diffusivity as well as reduced fractional anisotropy in periventricular WM. The metabolic and structural findings are consistent with progressive neuroaxonal loss in the WM accompanied by astrocytic proliferation - histopathological changes known to occur in HSP-TCC. Our results are in agreement with the hypothesis that the primary pathological process in HSP-TCC affects the axon, possibly due to impaired axonal trafficking. (orig.)

  5. Loss of function of glucocerebrosidase GBA2 is responsible for motor neuron defects in hereditary spastic paraplegia.

    Science.gov (United States)

    Martin, Elodie; Schüle, Rebecca; Smets, Katrien; Rastetter, Agnès; Boukhris, Amir; Loureiro, José L; Gonzalez, Michael A; Mundwiller, Emeline; Deconinck, Tine; Wessner, Marc; Jornea, Ludmila; Oteyza, Andrés Caballero; Durr, Alexandra; Martin, Jean-Jacques; Schöls, Ludger; Mhiri, Chokri; Lamari, Foudil; Züchner, Stephan; De Jonghe, Peter; Kabashi, Edor; Brice, Alexis; Stevanin, Giovanni

    2013-02-07

    Spastic paraplegia 46 refers to a locus mapped to chromosome 9 that accounts for a complicated autosomal-recessive form of hereditary spastic paraplegia (HSP). With next-generation sequencing in three independent families, we identified four different mutations in GBA2 (three truncating variants and one missense variant), which were found to cosegregate with the disease and were absent in controls. GBA2 encodes a microsomal nonlysosomal glucosylceramidase that catalyzes the conversion of glucosylceramide to free glucose and ceramide and the hydrolysis of bile acid 3-O-glucosides. The missense variant was also found at the homozygous state in a simplex subject in whom no residual glucocerebrosidase activity of GBA2 could be evidenced in blood cells, opening the way to a possible measurement of this enzyme activity in clinical practice. The overall phenotype was a complex HSP with mental impairment, cataract, and hypogonadism in males associated with various degrees of corpus callosum and cerebellar atrophy on brain imaging. Antisense morpholino oligonucleotides targeting the zebrafish GBA2 orthologous gene led to abnormal motor behavior and axonal shortening/branching of motoneurons that were rescued by the human wild-type mRNA but not by applying the same mRNA containing the missense mutation. This study highlights the role of ceramide metabolism in HSP pathology. Copyright © 2013 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  6. A novel lung slice system with compromised antioxidant defenses

    Energy Technology Data Exchange (ETDEWEB)

    Hardwick, S.J.; Adam, A.; Cohen, G.M. (Univ. of London (England)); Smith, L.L. (Imperial Chemical Industries PLC, Cheshire (England))

    1990-04-01

    In order to facilitate the study of oxidative stress in lung tissue, rat lung slices with impaired antioxidant defenses were prepared and used. Incubation of lung slices with the antineoplastic agent 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU) (100 {mu}M) in an amino acid-rich medium for 45 min produced a near-maximal (approximately 85%), irreversible inhibition of glutathione reductase, accompanied by only a modest (approximately 15%) decrease in pulmonary nonprotein sulfhydryls (NPSH) and no alteration in intracellular ATP, NADP{sup +}, and NADPH levels. The amounts of NADP(H), ATP, and NPSH were stable over a 4-hr incubation period following the removal from BCNU. The viability of the system was further evaluated by measuring the rate of evolution of {sup 14}CO{sub 2} from D-({sup 14}C(U))-glucose. The rates of evolution were almost identical in the compromised system when compared with control slices over a 4-hr time period. By using slices with compromised oxidative defenses, preliminary results have been obtained with paraquat, nitrofurantoin, and 2,3-dimethoxy-1,4-naphthoquinone.

  7. A novel lung slice system with compromised antioxidant defenses

    International Nuclear Information System (INIS)

    Hardwick, S.J.; Adam, A.; Cohen, G.M.; Smith, L.L.

    1990-01-01

    In order to facilitate the study of oxidative stress in lung tissue, rat lung slices with impaired antioxidant defenses were prepared and used. Incubation of lung slices with the antineoplastic agent 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU) (100 μM) in an amino acid-rich medium for 45 min produced a near-maximal (approximately 85%), irreversible inhibition of glutathione reductase, accompanied by only a modest (approximately 15%) decrease in pulmonary nonprotein sulfhydryls (NPSH) and no alteration in intracellular ATP, NADP + , and NADPH levels. The amounts of NADP(H), ATP, and NPSH were stable over a 4-hr incubation period following the removal from BCNU. The viability of the system was further evaluated by measuring the rate of evolution of 14 CO 2 from D-[ 14 C(U)]-glucose. The rates of evolution were almost identical in the compromised system when compared with control slices over a 4-hr time period. By using slices with compromised oxidative defenses, preliminary results have been obtained with paraquat, nitrofurantoin, and 2,3-dimethoxy-1,4-naphthoquinone

  8. Career Compromises: Framings and Their Implications.

    Science.gov (United States)

    Gati, Itamar; Houminer, Daphna; Aviram, Tamar

    1998-01-01

    Career compromise was investigated in three framings (alternatives, aspect importance, within-aspect preference). Young adults and school counselors rated hypothetical stories. Results of four studies with different designs (Average N=106) supported the hypothesis. The alternatives framing was associated with greater compromise and decision…

  9. A de novo mosaic mutation in SPAST with two novel alternative alleles and chromosomal copy number variant in a boy with spastic paraplegia and autism spectrum disorder.

    Science.gov (United States)

    Matthews, A M; Tarailo-Graovac, M; Price, E M; Blydt-Hansen, I; Ghani, A; Drögemöller, B I; Robinson, W P; Ross, C J; Wasserman, W W; Siden, H; van Karnebeek, C D

    2017-10-01

    Here we report a 12 year old male with an extreme presentation of spastic paraplegia along with autism and dysmorphisms. Whole exome sequencing identified a predicted pathogenic pair of missense variants in SPAST at the same chromosomal location, each with a different alternative allele, while a chromosome microarray identified a 1.73 Mb paternally inherited copy gain of 1q21.1q21.2 resulting in a blended phenotype of both Spastic paraplegia 4 and 1q21.1 microduplication syndrome. We believe that the extreme phenotype observed is likely caused by the presence of cells which contain only mutant SPAST, but that the viability of the patient is possible due mosaicism of mutant alleles observed in different proportions across tissues. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  10. Effect the exercise program on neuropathic pain intensity in patients with paraplegia Spinal Cord Injury

    Directory of Open Access Journals (Sweden)

    Sedghi Goyaghaj N

    2015-11-01

    Full Text Available Background and Objective: Patients with spinal cord injury suffer from continuous and persistent neuropathic pain that has a destructive impact on their quality of life. Exercise therapy is one of the non-pharmacological interventions that is recommended to control chronic pain, This study aimed to determine the effect of exercise program on neuropathic pain intensity in patients with paraplegia Spinal Cord Injury. Materials and Method: This study is a clinical trial.that population was the all of the patients with spinal cord injury, who referred to one of the educational hospitals in Tehran in 2014, 40 patient were selected based on purposive sampling and were randomly allocated into two groups of experimental and control. Exercise program for paraplegia spinal cord injury was implemented in experimental group during twelve 45-60minutes sessions, twice a week. Data collection was done before and one week after the intervention through using personal information form and, The International Spinal Cord Injury Pain Basic Data Set. Data were analyzed with statistical software SPSS19 and Fisher's exact test, Independent samples T-test Paired T-test and Chi square. Results: The mean score of neuropathic pain intensity before the intervention was 8.05 ± 1.51 in intervention group and 7.57 ± 1.21 in the control group. These amounts after the intervention were 5.55 ± 1.61 and 7.37 ± 1.05 respectively (p < 0.001. Conclusion: Results showed that the regular exercise program can reduce neuropathic pain severity in patients with spinal cord injuries and it can be recommended as a non-pharmacological method of pain control in these patients.

  11. Dental management of medically compromised patients

    Directory of Open Access Journals (Sweden)

    Sherly Horax

    2016-06-01

    Full Text Available These days, treatment in dentistry is no longer for patient without complication, but also for patient with bad medical record. With correct treatment management in handling medical condition of patient, not only for dental treatment but also their systematic disease, all the dental staff also can improve for the better quality of life of the patient. Patient with medical compromised start to realize that  keeping good oral hygiene is so important for their lives, therefore dental staff need to improve their science and technology and also for facing patient with medical compromised. This article will discuss and suggest various treatment consideration and protocol for the patient of with medical compromised.

  12. Clinical and Paraclinical Indicators of Motor System Impairment in Hereditary Spastic Paraplegia: A Pilot Study.

    Directory of Open Access Journals (Sweden)

    Andrea Martinuzzi

    Full Text Available Hereditary spastic paraplegias (HSP are a composite and genetically heterogeneous group of conditions mainly expressed by the impairment of the central motor system ("pure" forms. The involvement of other components of the central nervous system or of other systems is described in the "complicate" forms. The definition of an investigation protocol capable, by assembling clinical and paraclinical indicators to fully represent the extent of the motor system impairment, would help both the clinical handling of these conditions and contribute to our understanding of their pathogenesis.We applied a clinical and paraclinical protocol which included tools exploring motor and non motor functioning, neurophysiology and MRI to a composite cohort of 70 molecularly defined HSP patients aged 3 to 65, to define for each indicator its significance in detailing the presence and the severity of the pathology.Clinically increased deep tendon reflexes and lower limb (LL weakness are constant findings in all patients. The "complicated" forms are characterized by peripheral motor impairment, cognitive and cerebellar involvement. The Spastic Paraplegia Rating Scale efficiently reflects the severity of functional problems and correlates with disease duration. Neurophysiology consistently documents the impairment of the central motor pathway to the LLs. Nevertheless, the upper extremities and sensory system involvement is a frequent finding. MRI diffusion tensor imaging (DTI highlighted a significant alteration of FA and MD. Combining the sampling of the various portion of the cortico-spinal tract (CST DTI consistently discriminated patients from controls.We propose a graded clinical and paraclinical protocol for HSP phenotype definition, indicating for each tool the discriminative and descriptive capacity. Our protocol applied to 9 different forms of HSP showed that the functional impairment often extends beyond the CST. The novel DTI approach may add significant

  13. Clinical and Paraclinical Indicators of Motor System Impairment in Hereditary Spastic Paraplegia: A Pilot Study.

    Science.gov (United States)

    Martinuzzi, Andrea; Montanaro, Domenico; Vavla, Marinela; Paparella, Gabriella; Bonanni, Paolo; Musumeci, Olimpia; Brighina, Erika; Hlavata, Hana; Rossi, Giuseppe; Aghakhanyan, Gayane; Martino, Nicola; Baratto, Alessandra; D'Angelo, Maria Grazia; Peruch, Francesca; Fantin, Marianna; Arnoldi, Alessia; Citterio, Andrea; Vantaggiato, Chiara; Rizzo, Vincenzo; Toscano, Antonio; Bresolin, Nereo; Bassi, Maria Teresa

    2016-01-01

    Hereditary spastic paraplegias (HSP) are a composite and genetically heterogeneous group of conditions mainly expressed by the impairment of the central motor system ("pure" forms). The involvement of other components of the central nervous system or of other systems is described in the "complicate" forms. The definition of an investigation protocol capable, by assembling clinical and paraclinical indicators to fully represent the extent of the motor system impairment, would help both the clinical handling of these conditions and contribute to our understanding of their pathogenesis. We applied a clinical and paraclinical protocol which included tools exploring motor and non motor functioning, neurophysiology and MRI to a composite cohort of 70 molecularly defined HSP patients aged 3 to 65, to define for each indicator its significance in detailing the presence and the severity of the pathology. Clinically increased deep tendon reflexes and lower limb (LL) weakness are constant findings in all patients. The "complicated" forms are characterized by peripheral motor impairment, cognitive and cerebellar involvement. The Spastic Paraplegia Rating Scale efficiently reflects the severity of functional problems and correlates with disease duration. Neurophysiology consistently documents the impairment of the central motor pathway to the LLs. Nevertheless, the upper extremities and sensory system involvement is a frequent finding. MRI diffusion tensor imaging (DTI) highlighted a significant alteration of FA and MD. Combining the sampling of the various portion of the cortico-spinal tract (CST) DTI consistently discriminated patients from controls. We propose a graded clinical and paraclinical protocol for HSP phenotype definition, indicating for each tool the discriminative and descriptive capacity. Our protocol applied to 9 different forms of HSP showed that the functional impairment often extends beyond the CST. The novel DTI approach may add significant elements in

  14. Compromise in cooperative game and the VIKOR method

    Directory of Open Access Journals (Sweden)

    Opricović Serafim

    2009-01-01

    Full Text Available Five approaches in conflict resolution are distinguished, based on cooperativeness and aggressiveness in resolving conflict. Compromise based on cooperativeness is emphasized here as a solution in conflict resolution. Cooperative game theory oriented towards aiding the conflict resolution is considered and the compromise value for TU(transferable utility-game is presented. The method VIKOR could be applied to determine compromise solution of a multicriteria decision making problem with noncommensurable and conflicting criteria. Compromise is considered as an intermediate state between conflicting objectives or criteria reached by mutual concession. The applicability of the cooperative game theory and the VIKOR method for conflict resolution is illustrated.

  15. New domains of neural cell-adhesion molecule L1 implicated in X-linked hydrocephalus and MASA syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Jouet, M.; Kenwick, S. [Univ. of Cambridge (United Kingdom); Moncla, A. [Hopital d`Enfants de la Timone, Marseillas (United Kingdom)] [and others

    1995-06-01

    The neural cell-adhesion molecule L1 is involved in intercellular recognition and neuronal migration in the CNS. Recently, we have shown that mutations in the gene encoding L1 are responsible for three related disorders; X-linked hydrocephalus, MASA (mental retardation, aphasia, shuffling gait, and adducted thumbs) syndrome, and spastic paraplegia type I (SPG1). These three disorders represent a clinical spectrum that varies not only between families but sometimes also within families. To date, 14 independent L1 mutations have been reported and shown to be disease causing. Here we report nine novel L1 mutations in X-linked hydrocephalus and MASA-syndrome families, including the first examples of mutations affecting the fibronectin type III domains of the molecule. They are discussed in relation both to phenotypes and to the insights that they provide into L1 function. 39 refs., 5 figs., 3 tabs.

  16. 22 CFR 213.25 - Standards for compromise.

    Science.gov (United States)

    2010-04-01

    ... proceedings. In evaluating the acceptability of the offer, the CFO may consider, among other factors, the... applicable exemptions available to the debtor under State and Federal law in determining the Government's ability to enforce collection. (b) USAID may compromise a claim, or recommend acceptance of a compromise...

  17. Kyphoplasty for osteoporotic fractures with spinal canal compromise

    International Nuclear Information System (INIS)

    Gan Minfeng; Yang Huilin; Zou Jun; Wang Genlin; Mei Xin; Zhou Feng; Chen Liang; Jiang Weimin

    2010-01-01

    Objective: To explore the feasibility and clinical outcome of kyphoplasty in the treatment of osteoporotic fractures with canal compromise. Methods: A total of 16 patients with osteoporotic fractures with canal compromise without neurological deficit were attempted to be treated by kyphoplasty. During kyphoplasty, modified techniques including staged bone cement injection and dynamic fluoroscopic monitoring were used. Pain was measured using the self-reporting Visual Analogue pain Scale (VAS) preoperatively, postoperatively and in the final follow-up. Disability was measured using the Oswestry Disability questionnaire (ODI) preoperatively, postoperatively and in the final follow-up. The height of the compromised vertebral body, the kyphotic angle and the spinal canal compromise were measured preoperatively, postoperatively and in the final follow-up. Results: Operations were completed smoothly, with the exception of one patient with less cement leakage but without clinical symptom occurred. Relief of pain was achieved after kyphoplasty. The mean VAS score of these patients decreased from 8.1 ± 1.2 pre-operatively to 2.7 ± 0.6 post-operatively (P 0.05). In the final follow-up, the spinal canal compromise was (14.4 ± 3.1)%. Conclusion: Kyphoplasty is a relatively safe and effective method for the treatment of osteoporotic fractures with canal compromise without neurological deficit. (authors)

  18. 20 CFR 255.18 - Compromise of overpayments.

    Science.gov (United States)

    2010-04-01

    ... 20 Employees' Benefits 1 2010-04-01 2010-04-01 false Compromise of overpayments. 255.18 Section 255.18 Employees' Benefits RAILROAD RETIREMENT BOARD REGULATIONS UNDER THE RAILROAD RETIREMENT ACT... standards which the Board applies in exercising its authority under 31 U.S.C. 3711 to compromise an...

  19. 16 CFR 1.96 - Compromise of penalty.

    Science.gov (United States)

    2010-01-01

    ... 16 Commercial Practices 1 2010-01-01 2010-01-01 false Compromise of penalty. 1.96 Section 1.96 Commercial Practices FEDERAL TRADE COMMISSION ORGANIZATION, PROCEDURES AND RULES OF PRACTICE GENERAL... may compromise any penalty or proposed penalty at any time, with leave of court when necessary, taking...

  20. Acute gastrointestinal compromise in neonates with congenital diaphragmatic hernia prior to repair.

    Science.gov (United States)

    Fox, Catherine; Stewart, Michael; King, Sebastian K; Patel, Neil

    2016-12-01

    Congenital diaphragmatic hernia (CDH) affects 1 in 3000 live births. Modern management strategies include delayed repair of the diaphragm to permit pre-operative optimization of cardiorespiratory status. We describe a cohort of neonates in whom early emergency operative intervention was required for potentially fatal intestinal compromise. A retrospective review was performed of all neonatal CDH patients managed at a tertiary center in an 8-year period (2005-2012). A total of 126 CDH patients were managed during the 8-year period. Five neonates (male - 1; gestation 37+4-39+7; birth weight 2.9-3.7kg; left CDH - 5) required emergency operative intervention for presumed gastrointestinal compromise. All five neonates demonstrated systemic hypotension despite inotropic support, raised serum lactate (>2mmol/L), and abnormal radiographic findings. Operative intervention occurred within 3days of birth (1-3days). Findings included gastric volvulus, jejunal volvulus, and perforated caecum. All patients underwent primary diaphragmatic repair without a patch. Temporary ileostomy was required in 1 patient. All patients remain alive. Gastrointestinal compromise is a rare, but potentially catastrophic, complication of CDH. Emergency operative intervention may be required in a select cohort of patients. Early deterioration following birth should alert clinicians to the possibility of significant intestinal pathology. Level IV case series with no comparison group. Copyright © 2016 Elsevier Inc. All rights reserved.

  1. Effects of prandial challenge on triglyceridemia, glycemia, and pro-inflammatory activity in persons with chronic paraplegia

    Science.gov (United States)

    Ellenbroek, Dennis; Kressler, Jochen; Cowan, Rachel E.; Burns, Patricia A.; Mendez, Armando J.; Nash, Mark S.

    2015-01-01

    Context/Objective Exaggerated postprandial lipemia has been reported after spinal cord injury (SCI). We examined metabolite and accompanying pro-inflammatory biomarker responses to repeat feeding of typical high-fat meals in individuals with chronic paraplegia. Design Descriptive trial. Methods Metabolites (triglycerides, glucose, and insulin) and inflammatory biomarkers (interleukin-6 and high-sensitivity C-reactive protein (hsCRP)) were measured under fasting conditions in 11 recreationally active individuals with chronic (>1 year) paraplegia. Subjects received high-fat meals at time point 0 and again at minute 240. Antecubital venous blood was obtained at time points −30 (fasting), 0 (first meal), 30, 60, 90, 120, 240 (second meal), 360, and 480 minutes. Correlations were examined among the study variables. Exploratory subgroup analysis was performed for subjects with levels of postprandial glucose greater than >200 mg/dl. Results Triglycerides showed a significant rise 4 hours after eating. Basal inflammatory markers were elevated, and did not undergo additional change during the testing. Additionally, subjects with excessive postprandial glucose responses showed higher hsCRP levels than those having typical glucose responses both for fasting (11.8 ± 6.5 vs. 2.9 ± 2.7 mg/l, P = 0.064) and postprandial (11.1 ± 4.9 vs. 3.7 ± 3.8 mg/l, P = 0.018) values. Conclusions Despite elevations in metabolic response markers, inflammatory markers did not change significantly after consumption of population-representative (i.e. hypercaloric) mixed-nutrient meals. Levels of fasting CRP in the high-risk range are consistent with other reports in persons with SCI and continue to pose concern for their cardiovascular disease risk. The possible association between postprandial metabolic responses and inflammatory states warrants further investigation to identify individual component risks for this secondary health hazard. PMID:24617559

  2. Strained Compromises?

    DEFF Research Database (Denmark)

    Ibsen, Christian Lyhne

    The Danish version of flexicurity is not only about a balance between labour market flexibility and social security. Arguably, it is also series of more or less stable underlying compromises between social partners about the main mechanisms and aims of labour market regulation which – supposedly...... – should be focused on employment rather than jobs and competition on quality rather than on labour costs. However, most studies on Danish flexicurity have been carried out under favourable economic conditions with social partners almost naturally agreeing to the merits of the model – at least in principle...

  3. Compromised data from social media to big data

    CERN Document Server

    Redden, Joanna; Langlois, Ganaele

    2015-01-01

    There has been a data rush in the past decade brought about by online communication and, in particular, social media (Facebook, Twitter, Youtube, among others), which promises a new age of digital enlightenment. But social data is compromised: it is being seized by specific economic interests, it leads to a fundamental shift in the relationship between research and the public good, and it fosters new forms of control and surveillance. Compromised Data: From Social Media to Big Data explores how we perform critical research within a compromised social data framework. The expert, international l

  4. Flow-based Compromise Detection

    NARCIS (Netherlands)

    Hofstede, R.J.

    2016-01-01

    Brute-force attacks are omnipresent and manyfold on the Internet, and aim at compromising user accounts by issuing large numbers of authentication attempts on applications and daemons. Widespread targets of such attacks are Secure SHell (SSH) and Web applications, for example. The impact of

  5. Knockdown of Pnpla6 protein results in motor neuron defects in zebrafish

    Directory of Open Access Journals (Sweden)

    Yang Song

    2013-03-01

    Mutations in patatin-like phospholipase domain containing 6 (PNPLA6, also known as neuropathy target esterase (NTE or SPG39, cause hereditary spastic paraplegia (HSP. Although studies on animal models, including mice and Drosophila, have extended our understanding of PNPLA6, its roles in neural development and in HSP are not clearly understood. Here, we describe the generation of a vertebrate model of PNPLA6 insufficiency using morpholino oligonucleotide knockdown in zebrafish (Danio rerio. Pnpla6 knockdown resulted in developmental abnormalities and motor neuron defects, including axon truncation and branching. The phenotypes in pnpla6 knockdown morphants were rescued by the introduction of wild-type, but not mutant, human PNPLA6 mRNA. Our results also revealed the involvement of BMP signaling in pnpla6 knockdown phenotypes. Taken together, these results demonstrate an important role of PNPLA6 in motor neuron development and implicate overexpression of BMP signaling as a possible mechanism underlying the developmental defects in pnpla6 morphants.

  6. The N355K atlastin 1 mutation is associated with hereditary sensory neuropathy and pyramidal tract features.

    Science.gov (United States)

    Leonardis, L; Auer-Grumbach, M; Papić, L; Zidar, J

    2012-07-01

      Mutations in atlastin-1 (ATL-1), a gene known to cause pure, early-onset autosomal dominant hereditary spastic paraplegia SPG3A, have been recently reported to cause hereditary sensory neuropathy I (HSN I). We describe the detailed clinical and electrophysiologic findings in the first family with ulcero-mutilating sensory neuropathy carrying the c. C1065A, p.N355K mutation in ATL-1.   Detailed clinical and electrophysiologic studies were performed in affected and at-risk family members. Motor and sensory nerve conductions studies (NCS) were carried out in upper and lower limbs. ATL-1 was screened for mutations by direct sequencing.   Ten patients were found to carry the N355K mutation. With the exception of the two youngest patients, all had trophic skin changes in the feet consisting mainly of painless ulcers. Frequently, amputation of toes, feet, or even more proximal parts of the lower legs became necessary. A variable degree of increased muscle tone was observed in younger patients, whilst some older affected individuals only presented with hyperreflexia of patellar tendon reflexes. NCS revealed signs of an axonal motor and sensory neuropathies.   Our family carrying the N355K ATL1 mutation, which was initially diagnosed as HSN I, enlarges the SPG3A phenotype. We therefore suggest that patients with HSN I excluded for more common causes of HSN I, and in particular, affected individuals who exhibit additional pyramidal tract features should also be screened for mutations in ATL1. © 2012 The Author(s) European Journal of Neurology © 2012 EFNS.

  7. Outcomes of Soft Tissue Reconstruction for Traumatic Lower Extremity Fractures with Compromised Vascularity.

    Science.gov (United States)

    Badash, Ido; Burtt, Karen E; Leland, Hyuma A; Gould, Daniel J; Rounds, Alexis D; Azadgoli, Beina; Patel, Ketan M; Carey, Joseph N

    2017-10-01

    Traumatic lower extremity fractures with compromised arterial flow are limb-threatening injuries. A retrospective review of 158 lower extremities with traumatic fractures, including 26 extremities with arterial injuries, was performed to determine the effects of vascular compromise on flap survival, successful limb salvage and complication rates. Patients with arterial injuries had a larger average flap surface area (255.1 vs 144.6 cm2, P = 0.02) and a greater number of operations (4.7 vs 3.8, P = 0.01) than patients without vascular compromise. Patients presenting with vascular injury were also more likely to require fasciotomy [odds ratio (OR): 6.5, confidence interval (CI): 2.3-18.2] and to have a nerve deficit (OR: 16.6, CI: 3.9-70.0), fracture of the distal third of the leg (OR: 2.9, CI: 1.15-7.1) and intracranial hemorrhage (OR: 3.84, CI: 1.1-12.9). After soft tissue reconstruction, patients with arterial injuries had a higher rate of amputation (OR: 8.5, CI: 1.3-53.6) and flap failure requiring a return to the operating room (OR: 4.5, CI: 1.5-13.2). Arterial injury did not correlate with infection or overall complication rate. In conclusion, arterial injuries resulted in significant complications for patients with lower extremity fractures requiring flap coverage, although limb salvage was still effective in most cases.

  8. A Hydatid Cyst of the Lumbar Spine: A Rare Cause of Paraplegia

    Directory of Open Access Journals (Sweden)

    Suhail Ur Rehman

    2017-05-01

    Full Text Available Introduction Hydatid cyst is a zoonotic disease, affecting humans and other mammals worldwide. It is caused by tapeworms of the genus Echinococcus, which is most frequently encountered in the liver and lungs. Although involvement of the central nervous system and spine is rare, it can lead to severe neurological deficits due to direct compression. Case Presentation We report a case of intradural extramedullary hydatid cyst in the lumbar region with a sudden onset, causing progressive paraplegia and areflexia over the past 20 days. After surgical removal, the cyst was sent for histopathological examination. The results showed inner laminated membranes and an outer fibrous layer, surrounded by foreign-body giant cells. The primary objective during surgery was to avoid perforation of the cyst, thereby reducing the risk of systemic dissemination and local seeding of the parasite. During the postoperative period, there was a steady improvement in the neurological deficit, and the patient was discharged with anthelmintics to prevent any distant dissemination. Conclusions An accurate and precise diagnosis is necessary when dealing with cystic pathologies.

  9. Impending Airway Compromise due to Cystic Hygroma

    Directory of Open Access Journals (Sweden)

    Itai Shavit

    2011-05-01

    Full Text Available We report on a 3-month-old infant, who arrived in the pediatric emergency department (ED with a cervical cystic hygroma causing an impending compromise of the airway. We recognize that such a lesion can rapidly progress, and the judicious use of imaging in the ED may help to avoid airway compromise and possibly fatal complications. [West J Emerg Med. 2011;12(4:368–369.

  10. 41 CFR 105-55.019 - Compromise of claims.

    Science.gov (United States)

    2010-07-01

    ... and a recommendation for the acceptance of the compromise offer. Justice Department approval is not... exercise the authorities in this section. (b) Unless otherwise provided by law, when the principal balance... Department of Justice. GSA will evaluate the compromise offer, using the factors set forth in § 105-55.020...

  11. Evaluation of a training program for persons with SCI paraplegia using the Parastep 1 ambulation system: part 4. Effect on physical self-concept and depression.

    Science.gov (United States)

    Guest, R S; Klose, K J; Needham-Shropshire, B M; Jacobs, P L

    1997-08-01

    To determine whether persons with spinal cord injury (SCI) paraplegia who participated in an electrical stimulation walking program experienced changes in measures of physical self-concept and depression. Before-after trial. Human SCI applied research laboratory. Volunteer sample of 12 men and 3 women with SCI paraplegia, mean age 28.75 +/- 6.6yrs and mean duration of injury 3.8 +/- 3.2yrs. Thirty-two FNS ambulation training sessions using a commercially available system (Parastep 1). The hybrid system consists of a microprocessor-controlled stimulator and a modified walking frame with finger-operated switches that permit the user to control the stimulation parameters and activate the stepping. The Tennessee Self-Concept Scale (TSCS) and the Beck Depression Inventory (BDI) were administered before and after training. Only the Physical Self subscale of the TSCS was analyzed. After training, individual interviews were performed to assess participants' subjective reactions to the training program. A repeated measures analysis of variance indicated that desired directional and statistically significant changes occurred on the Physical Self subscale of the TSCS (F(1,14) = 8.54, p self-concept scores and decreases in depression scores.

  12. The role of postoperative hematoma on free flap compromise.

    Science.gov (United States)

    Ahmad, Faisal I; Gerecci, Deniz; Gonzalez, Javier D; Peck, Jessica J; Wax, Mark K

    2015-08-01

    Hematomas may develop in the postoperative setting after free tissue transfer. When hematomas occur, they can exert pressure on surrounding tissues. Their effect on the vascular pedicle of a free flap is unknown. We describe our incidence of hematoma in free flaps and outcomes when the flap is compromised. Retrospective chart review of 1,883 free flaps performed between July 1998 and June 2014 at a tertiary referral center. Patients with free flap compromise due to hematoma were identified. Etiology, demographic data, and outcomes were evaluated. Eighty-eight (4.7%) patients developed hematomas. Twenty (22.7%) of those had flap compromise. Twelve compromises (60%) showed evidence of pedicle thrombosis. The salvage rate was 75% versus 54% in 79 flaps with compromise from other causes (P = .12). Mean time to detection of the hematoma was 35.3 hours in salvaged flaps compared to 91.6 hours in unsalvageable flaps (P = .057). Time to operating room (OR) from detection was 2.8 hours in salvageable flaps compared to 12.4 hours in nonsalvageable flaps (P = .053). The salvage rate for flaps that returned to the OR in hematomas developed rarely. When they did, 23% went on to develop flap compromise. Prompt recognition and re-exploration allowed for a high salvage rate. Vessel thrombosis predicted inability to salvage the flap. 4 © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

  13. Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease.

    Science.gov (United States)

    Casari, G; De Fusco, M; Ciarmatori, S; Zeviani, M; Mora, M; Fernandez, P; De Michele, G; Filla, A; Cocozza, S; Marconi, R; Dürr, A; Fontaine, B; Ballabio, A

    1998-06-12

    Hereditary spastic paraplegia (HSP) is characterized by progressive weakness and spasticity of the lower limbs due to degeneration of corticospinal axons. We found that patients from a chromosome 16q24.3-linked HSP family are homozygous for a 9.5 kb deletion involving a gene encoding a novel protein, named Paraplegin. Two additional Paraplegin mutations, both resulting in a frameshift, were found in a complicated and in a pure form of HSP. Paraplegin is highly homologous to the yeast mitochondrial ATPases, AFG3, RCA1, and YME1, which have both proteolytic and chaperon-like activities at the inner mitochondrial membrane. Immunofluorescence analysis and import experiments showed that Paraplegin localizes to mitochondria. Analysis of muscle biopsies from two patients carrying Paraplegin mutations showed typical signs of mitochondrial OXPHOS defects, thus suggesting a mechanism for neurodegeneration in HSP-type disorders.

  14. Sensible biological models to be exposed to VDT (Video Display Terminal) radiations in human male reproduction

    International Nuclear Information System (INIS)

    Tritto, J.; North, M.-O.; Laverdure, A.M.; Surbeck, J.

    1999-01-01

    Temperature and environmental effects, particularly endocrine disrupters and EMF radiations, are actively investigated in human and non-human reproduction experimental models. Sensitivity and specificity of the different cell types of the testes seminiferous tubules in animals and in human are evaluated, showing a specific responsiveness of spermatogonia (SPG) and resting pachytene spermatocytes (SPC). At 32 o C the 24 h short-term cultures of biopsies of normal human testis show an expected low occurrence of apoptotic SPG (1 %) that increases to 3,4 % in peer samples exposed to VDT for the same period, with the appearance of apoptotic SPC (4,6 %). In samples from a thermically-impaired testis of the same subject the apoptotic occurrence of SPG is 2,6 % with 15,4 % for SPC after 24 h cultures. After 24 h exposure to VDT the apoptotic score is 7,6 % for SPG and 18,5 % for SPC in thermically impaired peer samples. With EMF-bioshields the apoptotic score for SPG is 0,8 % in normal 2,2 % for SPG and 13,8 % for SPC in T-impaired peer-samples. NMRS of the cultures fluids show a proportional production of lactate, corresponding to the different degrees of histopathological impairment of the samples. IVOS (Integrated Visual Optic System) analysis of sperm samples from thermically-impaired, not-repaired and repaired testes exposed to VDT shows sensible variations on straightness (STR), linearity (LIN) and lateral head displacement (LHD) parameters. To evaluate the thermic and non-thermic potential bioeffects of VDT on human spermatogenesis the specificity, the sensitivity and the reproducibility of the biological models on one side and the specificity of the methodologies on the other side must be provided. (author)

  15. Paraplegia espastica familiar com amiotrofia: estudo clinico, eletromiografico, histoquimico e microdissecção

    Directory of Open Access Journals (Sweden)

    Lineu Cesar Werneck

    1979-06-01

    Full Text Available Relato de quatro casos de paraplegia, espástica familiar com amiotrofia, em irmãos provenientes de um matrimônio consanguíneo. A investigação laboratorial rotineira estava normal. A eletromiografia e biópsia muscular com histoquímica demonstraram denervação com reinervação. As conduções nervosas motoras estavam diminuídas em três casos no nervo peroneiro. A análise por microdissecção do nervo sural foi anormal nos quatro casos, revelando desmielinização e remielinização. Após revisão da literatura, os autores acreditam que as alterações encontradas são decorrentes de degeneração axonal distai, com regeneração secundária e levantam a hipótese de defeito no fluxo axoplásmico do sistema nervoso central e periférico.

  16. Palmo-Plantar hyperkeratosis, intellectual disability, and spastic paraplegia in two maternal half brothers: further evidence for an X-linked inheritance.

    Science.gov (United States)

    Isidor, Bertrand; Lefebvre, Tiphaine; Barbarot, Sébastien; Perrier, Julie; Mercier, Sandra; Péréon, Yann; Le Caignec, Cédric; David, Albert

    2013-06-01

    In 1983, Fitzsimmons et al. reported four brothers with an unrecognized disorder characterized by intellectual disability, spastic paraplegia, and palmo-plantar hyperkeratosis (OMIM 309500). In this report, we describe a family in which two males, maternal half-brothers, had learning disabilities. Both patients also showed spasticity in the lower limbs and palmo-plantar hyperkeratosis. The mother of the affected boys had learning difficulties but did not show any dermatological symptoms. This report confirms that the association of features reported by Fitzsimmons et al. is a distinct entity and further suggests an X-linked mode of inheritance. Copyright © 2013 Wiley Periodicals, Inc.

  17. Combating QR-Code-Based Compromised Accounts in Mobile Social Networks.

    Science.gov (United States)

    Guo, Dong; Cao, Jian; Wang, Xiaoqi; Fu, Qiang; Li, Qiang

    2016-09-20

    Cyber Physical Social Sensing makes mobile social networks (MSNs) popular with users. However, such attacks are rampant as malicious URLs are spread covertly through quick response (QR) codes to control compromised accounts in MSNs to propagate malicious messages. Currently, there are generally two types of methods to identify compromised accounts in MSNs: one type is to analyze the potential threats on wireless access points and the potential threats on handheld devices' operation systems so as to stop compromised accounts from spreading malicious messages; the other type is to apply the method of detecting compromised accounts in online social networks to MSNs. The above types of methods above focus neither on the problems of MSNs themselves nor on the interaction of sensors' messages, which leads to the restrictiveness of platforms and the simplification of methods. In order to stop the spreading of compromised accounts in MSNs effectively, the attacks have to be traced to their sources first. Through sensors, users exchange information in MSNs and acquire information by scanning QR codes. Therefore, analyzing the traces of sensor-related information helps to identify the compromised accounts in MSNs. This paper analyzes the diversity of information sending modes of compromised accounts and normal accounts, analyzes the regularity of GPS (Global Positioning System)-based location information, and introduces the concepts of entropy and conditional entropy so as to construct an entropy-based model based on machine learning strategies. To achieve the goal, about 500,000 accounts of Sina Weibo and about 100 million corresponding messages are collected. Through the validation, the accuracy rate of the model is proved to be as high as 87.6%, and the false positive rate is only 3.7%. Meanwhile, the comparative experiments of the feature sets prove that sensor-based location information can be applied to detect the compromised accounts in MSNs.

  18. Combating QR-Code-Based Compromised Accounts in Mobile Social Networks

    Directory of Open Access Journals (Sweden)

    Dong Guo

    2016-09-01

    Full Text Available Cyber Physical Social Sensing makes mobile social networks (MSNs popular with users. However, such attacks are rampant as malicious URLs are spread covertly through quick response (QR codes to control compromised accounts in MSNs to propagate malicious messages. Currently, there are generally two types of methods to identify compromised accounts in MSNs: one type is to analyze the potential threats on wireless access points and the potential threats on handheld devices’ operation systems so as to stop compromised accounts from spreading malicious messages; the other type is to apply the method of detecting compromised accounts in online social networks to MSNs. The above types of methods above focus neither on the problems of MSNs themselves nor on the interaction of sensors’ messages, which leads to the restrictiveness of platforms and the simplification of methods. In order to stop the spreading of compromised accounts in MSNs effectively, the attacks have to be traced to their sources first. Through sensors, users exchange information in MSNs and acquire information by scanning QR codes. Therefore, analyzing the traces of sensor-related information helps to identify the compromised accounts in MSNs. This paper analyzes the diversity of information sending modes of compromised accounts and normal accounts, analyzes the regularity of GPS (Global Positioning System-based location information, and introduces the concepts of entropy and conditional entropy so as to construct an entropy-based model based on machine learning strategies. To achieve the goal, about 500,000 accounts of Sina Weibo and about 100 million corresponding messages are collected. Through the validation, the accuracy rate of the model is proved to be as high as 87.6%, and the false positive rate is only 3.7%. Meanwhile, the comparative experiments of the feature sets prove that sensor-based location information can be applied to detect the compromised accounts in MSNs.

  19. Constraining the brachial plexus does not compromise regional control in oropharyngeal carcinoma

    International Nuclear Information System (INIS)

    Robert, Mutter W; Wolden, Suzanne L; Lee, Nancy Y; Lok, Benjamin H; Dutta, Pinaki R; Riaz, Nadeem; Setton, Jeremy; Berry, Sean L; Goenka, Anuj; Zhang, Zhigang; Rao, Shyam S

    2013-01-01

    Accumulating evidence suggests that brachial plexopathy following head and neck cancer radiotherapy may be underreported and that this toxicity is associated with a dose–response. Our purpose was to determine whether the dose to the brachial plexus (BP) can be constrained, without compromising regional control. The radiation plans of 324 patients with oropharyngeal carcinoma (OPC) treated with intensity-modulated radiation therapy (IMRT) were reviewed. We identified 42 patients (13%) with gross nodal disease <1 cm from the BP. Normal tissue constraints included a maximum dose of 66 Gy and a D 05 of 60 Gy for the BP. These criteria took precedence over planning target volume (PTV) coverage of nodal disease near the BP. There was only one regional failure in the vicinity of the BP, salvaged with neck dissection (ND) and regional re-irradiation. There have been no reported episodes of brachial plexopathy to date. In combined-modality therapy, including ND as salvage, regional control did not appear to be compromised by constraining the dose to the BP. This approach may improve the therapeutic ratio by reducing the long-term risk of brachial plexopathy

  20. 32 CFR 310.50 - Lost, stolen, or compromised information.

    Science.gov (United States)

    2010-07-01

    ... Official for Privacy within 24 hours of discovering that a breach of personally identifiable information... Privacy Office of the breach within 48 hours upon being notified that a loss, theft, or compromise has... (CONTINUED) PRIVACY PROGRAM DOD PRIVACY PROGRAM Privacy Act Violations § 310.50 Lost, stolen, or compromised...

  1. A muscle-driven approach to restore stepping with an exoskeleton for individuals with paraplegia.

    Science.gov (United States)

    Chang, Sarah R; Nandor, Mark J; Li, Lu; Kobetic, Rudi; Foglyano, Kevin M; Schnellenberger, John R; Audu, Musa L; Pinault, Gilles; Quinn, Roger D; Triolo, Ronald J

    2017-05-30

    Functional neuromuscular stimulation, lower limb orthosis, powered lower limb exoskeleton, and hybrid neuroprosthesis (HNP) technologies can restore stepping in individuals with paraplegia due to spinal cord injury (SCI). However, a self-contained muscle-driven controllable exoskeleton approach based on an implanted neural stimulator to restore walking has not been previously demonstrated, which could potentially result in system use outside the laboratory and viable for long term use or clinical testing. In this work, we designed and evaluated an untethered muscle-driven controllable exoskeleton to restore stepping in three individuals with paralysis from SCI. The self-contained HNP combined neural stimulation to activate the paralyzed muscles and generate joint torques for limb movements with a controllable lower limb exoskeleton to stabilize and support the user. An onboard controller processed exoskeleton sensor signals, determined appropriate exoskeletal constraints and stimulation commands for a finite state machine (FSM), and transmitted data over Bluetooth to an off-board computer for real-time monitoring and data recording. The FSM coordinated stimulation and exoskeletal constraints to enable functions, selected with a wireless finger switch user interface, for standing up, standing, stepping, or sitting down. In the stepping function, the FSM used a sensor-based gait event detector to determine transitions between gait phases of double stance, early swing, late swing, and weight acceptance. The HNP restored stepping in three individuals with motor complete paralysis due to SCI. The controller appropriately coordinated stimulation and exoskeletal constraints using the sensor-based FSM for subjects with different stimulation systems. The average range of motion at hip and knee joints during walking were 8.5°-20.8° and 14.0°-43.6°, respectively. Walking speeds varied from 0.03 to 0.06 m/s, and cadences from 10 to 20 steps/min. A self-contained muscle

  2. Whole-Exome Sequencing Identifies Homozygous AFG3L2 Mutations in a Spastic Ataxia-Neuropathy Syndrome Linked to Mitochondrial m-AAA Proteases

    Science.gov (United States)

    Martinelli, Paola; Cherukuri, Praveen F.; Teer, Jamie K.; Hansen, Nancy F.; Cruz, Pedro; Mullikin for the NISC Comparative Sequencing Program, James C.; Blakesley, Robert W.; Golas, Gretchen; Kwan, Justin; Sandler, Anthony; Fuentes Fajardo, Karin; Markello, Thomas; Tifft, Cynthia; Blackstone, Craig; Rugarli, Elena I.; Langer, Thomas; Gahl, William A.; Toro, Camilo

    2011-01-01

    We report an early onset spastic ataxia-neuropathy syndrome in two brothers of a consanguineous family characterized clinically by lower extremity spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy. Whole-exome sequencing identified a homozygous missense mutation (c.1847G>A; p.Y616C) in AFG3L2, encoding a subunit of an m-AAA protease. m-AAA proteases reside in the mitochondrial inner membrane and are responsible for removal of damaged or misfolded proteins and proteolytic activation of essential mitochondrial proteins. AFG3L2 forms either a homo-oligomeric isoenzyme or a hetero-oligomeric complex with paraplegin, a homologous protein mutated in hereditary spastic paraplegia type 7 (SPG7). Heterozygous loss-of-function mutations in AFG3L2 cause autosomal-dominant spinocerebellar ataxia type 28 (SCA28), a disorder whose phenotype is strikingly different from that of our patients. As defined in yeast complementation assays, the AFG3L2Y616C gene product is a hypomorphic variant that exhibited oligomerization defects in yeast as well as in patient fibroblasts. Specifically, the formation of AFG3L2Y616C complexes was impaired, both with itself and to a greater extent with paraplegin. This produced an early-onset clinical syndrome that combines the severe phenotypes of SPG7 and SCA28, in additional to other “mitochondrial” features such as oculomotor apraxia, extrapyramidal dysfunction, and myoclonic epilepsy. These findings expand the phenotype associated with AFG3L2 mutations and suggest that AFG3L2-related disease should be considered in the differential diagnosis of spastic ataxias. PMID:22022284

  3. Whole-exome sequencing identifies homozygous AFG3L2 mutations in a spastic ataxia-neuropathy syndrome linked to mitochondrial m-AAA proteases.

    Directory of Open Access Journals (Sweden)

    Tyler Mark Pierson

    2011-10-01

    Full Text Available We report an early onset spastic ataxia-neuropathy syndrome in two brothers of a consanguineous family characterized clinically by lower extremity spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy. Whole-exome sequencing identified a homozygous missense mutation (c.1847G>A; p.Y616C in AFG3L2, encoding a subunit of an m-AAA protease. m-AAA proteases reside in the mitochondrial inner membrane and are responsible for removal of damaged or misfolded proteins and proteolytic activation of essential mitochondrial proteins. AFG3L2 forms either a homo-oligomeric isoenzyme or a hetero-oligomeric complex with paraplegin, a homologous protein mutated in hereditary spastic paraplegia type 7 (SPG7. Heterozygous loss-of-function mutations in AFG3L2 cause autosomal-dominant spinocerebellar ataxia type 28 (SCA28, a disorder whose phenotype is strikingly different from that of our patients. As defined in yeast complementation assays, the AFG3L2(Y616C gene product is a hypomorphic variant that exhibited oligomerization defects in yeast as well as in patient fibroblasts. Specifically, the formation of AFG3L2(Y616C complexes was impaired, both with itself and to a greater extent with paraplegin. This produced an early-onset clinical syndrome that combines the severe phenotypes of SPG7 and SCA28, in additional to other "mitochondrial" features such as oculomotor apraxia, extrapyramidal dysfunction, and myoclonic epilepsy. These findings expand the phenotype associated with AFG3L2 mutations and suggest that AFG3L2-related disease should be considered in the differential diagnosis of spastic ataxias.

  4. m-AAA proteases, mitochondrial calcium homeostasis and neurodegeneration.

    Science.gov (United States)

    Patron, Maria; Sprenger, Hans-Georg; Langer, Thomas

    2018-03-01

    The function of mitochondria depends on ubiquitously expressed and evolutionary conserved m-AAA proteases in the inner membrane. These ATP-dependent peptidases form hexameric complexes built up of homologous subunits. AFG3L2 subunits assemble either into homo-oligomeric isoenzymes or with SPG7 (paraplegin) subunits into hetero-oligomeric proteolytic complexes. Mutations in AFG3L2 are associated with dominant spinocerebellar ataxia (SCA28) characterized by the loss of Purkinje cells, whereas mutations in SPG7 cause a recessive form of hereditary spastic paraplegia (HSP7) with motor neurons of the cortico-spinal tract being predominantly affected. Pleiotropic functions have been assigned to m-AAA proteases, which act as quality control and regulatory enzymes in mitochondria. Loss of m-AAA proteases affects mitochondrial protein synthesis and respiration and leads to mitochondrial fragmentation and deficiencies in the axonal transport of mitochondria. Moreover m-AAA proteases regulate the assembly of the mitochondrial calcium uniporter (MCU) complex. Impaired degradation of the MCU subunit EMRE in AFG3L2-deficient mitochondria results in the formation of deregulated MCU complexes, increased mitochondrial calcium uptake and increased vulnerability of neurons for calcium-induced cell death. A reduction of calcium influx into the cytosol of Purkinje cells rescues ataxia in an AFG3L2-deficient mouse model. In this review, we discuss the relationship between the m-AAA protease and mitochondrial calcium homeostasis and its relevance for neurodegeneration and describe a novel mouse model lacking MCU specifically in Purkinje cells. Our results pledge for a novel view on m-AAA proteases that integrates their pleiotropic functions in mitochondria to explain the pathogenesis of associated neurodegenerative disorders.

  5. Acute toxemic schistosomiasis complicated by a acute flaccid paraplegia due to schistosomal myeloradiculopathy in Sudan

    Energy Technology Data Exchange (ETDEWEB)

    Ahmed, Abdelmonim F [Dept. of Medicine, Faculty of Medicine, Taibah Univ., Al-Madinah Al-Munwarah (Saudi Arabia); Kareem, Abid M [Dept. of Gastroenterology, King Fahd Hospital, Al- Madinah Al-Munwarah (Saudi Arabia); Dawoud, Talal A [Dept. of Neurology, King Fahd Hospital, Al-Madinah Al-Munawarh (Saudi Arabia); Idris, Abdelrehman S [Dept. of Neurology, Hiraa Hospital, Mecca (Saudi Arabia)

    2008-07-01

    A 55-year old Sudanese physician presented with one month history of diarrhea, loss of weight 10 kg and low grade nocturnal fever. Following colonoscopy, he rapidly developed paraparesis and retention of urine. Magnetic resonance imaging (MRI) of the spinal cord showed low cord lesion suggestive of transverse myelitis. We present a detailed account of diagnostic and management challenges and a literature review of the final diagnosis of acute toxemic schistosomiasis, complicated by acute flaccid paraplegia due to schistosomal myeloradiculopathy. We are reporting this case to increase the awareness of physicians of schistosomal myeloradiculopathy, as it needs urgent specific treatment praziquantel and steroids. An early follow-up with MRI of the spinal cord 2 weeks treatment may help in preventing unnecessary neurosurgical intervention. Bilharziasis may be contracted on the banks of river White Nile in urban areas. Finally clinicians should make use of the Google search for diagnosis in difficult cases. (author)

  6. Mitochondrial energy metabolism is required for lifespan extension by the spastic paraplegia-associated protein spartin

    Directory of Open Access Journals (Sweden)

    Julia Ring

    2017-11-01

    Full Text Available Hereditary spastic paraplegias, a group of neurodegenerative disorders, can be caused by loss-of-function mutations in the protein spartin. However, the physiological role of spartin remains largely elusive. Here we show that heterologous expression of human or Drosophila spartin extends chronological lifespan of yeast, reducing age-associated ROS production, apoptosis, and necrosis. We demonstrate that spartin localizes to the proximity of mitochondria and physically interacts with proteins related to mitochondrial and respiratory metabolism. Interestingly, Nde1, the mitochondrial external NADH dehydrogenase, and Pda1, the core enzyme of the pyruvate dehydrogenase complex, are required for spartin-mediated cytoprotection. Furthermore, spartin interacts with the glycolysis enhancer phospo-fructo-kinase-2,6 (Pfk26 and is sufficient to complement for PFK26-deficiency at least in early aging. We conclude that mitochondria-related energy metabolism is crucial for spartin’s vital function during aging and uncover a network of specific interactors required for this function.

  7. Acute toxemic schistosomiasis complicated by a acute flaccid paraplegia due to schistosomal myeloradiculopathy in Sudan

    International Nuclear Information System (INIS)

    Ahmed, Abdelmonim F.; Kareem, Abid M.; Dawoud, Talal A.; Idris, Abdelrehman S.

    2008-01-01

    A 55-year old Sudanese physician presented with one month history of diarrhea, loss of weight 10 kg and low grade nocturnal fever. Following colonoscopy, he rapidly developed paraparesis and retention of urine. Magnetic resonance imaging (MRI) of the spinal cord showed low cord lesion suggestive of transverse myelitis. We present a detailed account of diagnostic and management challenges and a literature review of the final diagnosis of acute toxemic schistosomiasis, complicated by acute flaccid paraplegia due to schistosomal myeloradiculopathy. We are reporting this case to increase the awareness of physicians of schistosomal myeloradiculopathy, as it needs urgent specific treatment praziquantel and steroids. An early follow-up with MRI of the spinal cord 2 weeks treatment may help in preventing unnecessary neurosurgical intervention. Bilharziasis may be contracted on the banks of river White Nile in urban areas. Finally clinicians should make use of the Google search for diagnosis in difficult cases. (author)

  8. Compromised Agency: The Case of BabyLegs

    Directory of Open Access Journals (Sweden)

    Max Liboiron

    2017-09-01

    Full Text Available The concept of agency is ubiquitous in STS, particularly regarding cases of alternative ways of knowing and doing science such as civic, citizen, and feminist sciences, among others. Yet the focus on agency often glosses over the constraints placed on agents, particularly within asymmetrical power relations. This article follows the case of BabyLegs, a do-it-yourself monitoring tool for marine microplastic pollution, and the attempt to keep the technology open source within an intellectual property (IP system set up to privatize it. The tactics used to design BabyLegs as a feminine, silly, doll-tool to discredit the device in the eyes of an IP system that valued traditional gender roles lead to the eventual success of keeping the device open source. Yet, those same tactics also reinforced and reproduced the structures of power and essentialism they were designed to resist. I characterize this technological ambivalence as compromise, and argue that all agency exercised within asymmetrical power relations is compromised. This is not to say resistance is futile, but that agency is never pure, and this recognition lets us be more intentional in how we might compromise as practitioners of diverse scientific knowledges.

  9. Compromise decision support problems for hierarchical design involving uncertainty

    Science.gov (United States)

    Vadde, S.; Allen, J. K.; Mistree, F.

    1994-08-01

    In this paper an extension to the traditional compromise Decision Support Problem (DSP) formulation is presented. Bayesian statistics is used in the formulation to model uncertainties associated with the information being used. In an earlier paper a compromise DSP that accounts for uncertainty using fuzzy set theory was introduced. The Bayesian Decision Support Problem is described in this paper. The method for hierarchical design is demonstrated by using this formulation to design a portal frame. The results are discussed and comparisons are made with those obtained using the fuzzy DSP. Finally, the efficacy of incorporating Bayesian statistics into the traditional compromise DSP formulation is discussed and some pending research issues are described. Our emphasis in this paper is on the method rather than the results per se.

  10. Near-infrared spectroscopy for detection of vascular compromise in paediatric supracondylar fractures

    International Nuclear Information System (INIS)

    Skowno, Justin J; De Lima, Jonathan; Quick, Tom J; Carpenter, Eleanor C; Gibbons, Paul J; Little, David G

    2014-01-01

    Children suffering supracondylar fractures of the humerus are at risk of vascular compromise, which is currently assessed clinically, although other modalities such as angiography, pulse oximetry, Doppler ultrasound and magnetic resonance angiography have been used. We sought to ascertain whether tissue haemoglobin oxygenation (StO 2 ) measurement could distinguish between patients with and without clinical vascular compromise following supracondylar fractures of the humerus. We prospectively observed StO 2  using near-infrared spectroscopy in 29 paediatric patients with supracondylar fractures requiring operative manipulation. The injured and uninjured volar forearm compartments were monitored immediately before and after fracture reduction. The relationship between StO 2  in the injured and uninjured limb, and the presence of pre-operative vascular compromise was assessed. Seven out of 29 children presented with vascular compromise. Patients with clinical vascular compromise had significantly lower pre-reduction StO 2  (63.5% ± 15%, mean ± standard deviation), compared to those without compromise (80.9% ± 10%). StO 2  normalized following surgery in all children with vascular compromise. These improvements in muscle StO 2  were associated, in all patients, with the clinical return of pulses and resolution of neurological symptoms if present. StO 2  monitoring can identify patients with clinical vascular compromise, can identify the return of adequate perfusion following operative correction of supracondylar fractures, and may be a useful adjunct to clinical assessment. (paper)

  11. When data representation compromise data security

    DEFF Research Database (Denmark)

    Simonsen, Eivind Ortind; Dahl, Mads Ronald

    WHEN DATA REPRESENTATION COMPROMISE DATA SECURITY The workflow of transforming data into informative representations makes extensive usage of computers and software. Scientists have a conventional tradition for producing publications that include tables and graphs as data representations....... These representations can be used for multiple purposes such as publications in journals, teaching and conference material. But when created, stored and distributed in a digital form there is a risk of compromising data security. Data beyond the once used specifically to create the representation can be included...... on the internet over many years? A new legislation proposed in 2012 by the European Commission on protection of personal data will be implemented from 2015. The new law will impose sanction options ranging from a warning to a fine up to 100.000.000 EUR. We argue that this new law will lead to especially...

  12. Revisiting genotype-phenotype overlap in neurogenetics: triplet-repeat expansions mimicking spastic paraplegias.

    Science.gov (United States)

    Bettencourt, Conceição; Quintáns, Beatriz; Ros, Raquel; Ampuero, Israel; Yáñez, Zuleima; Pascual, Samuel Ignacio; de Yébenes, Justo García; Sobrido, María-Jesús

    2012-09-01

    Hereditary spastic paraplegias (HSPs) constitute a heterogeneous group of neurological disorders, characterized primarily by progressive spasticity and weakness of the lower limbs. HSPs are caused by mutations in multiple genes (at least 48 loci and 28 causative genes). The clinical spectrum of HSPs is wide and important differences have been reported between patients with distinct mutations in the same gene, or even between different family members bearing the same mutation. Many patients with HSP present clinical deficits related to the involvement of neuronal systems other than corticospinal tracts, namely, peripheral nerves, sensory, or cerebellar pathways. These cases may be difficult to differentiate from other neurological diseases (e.g., hereditary ataxias), also genetically and clinically heterogeneous. As an illustration of how overlapping this genotype-phenotype relationship is, and the difficulties that it brings upon the development of neurogenetic algorithms and databases, we review the main clinical and genetic features of HSPs, and summarize reports on cases of triplet-repeat spinocerebellar ataxias that can mimic HSP phenotypes. This complex scenario makes the necessity of high-quality, curated mutation databases even more urgent, in order to develop adequate diagnostic guidelines, correct interpretation of genetic testing, and appropriate genetic counseling. © 2012 Wiley Periodicals, Inc.

  13. Tax Administration: IRS Should Evaluate the Changes to Its Offer in Compromise Program

    National Research Council Canada - National Science Library

    2002-01-01

    ...) Offer in Compromise (OIC) Program. An offer in compromise is an agreement between a taxpayer and the IRS to settle or compromise the taxpayer's tax liability for less than the full amount owed...

  14. The study on the immunological effect of Sizofilan combined with radiotherapy in patients with uterine cervical cancer

    International Nuclear Information System (INIS)

    Sekiguchi, Isao; Suzuki, Mitsuaki; Izumi, Akio; Aida, Ichiro; Tamada, Taro

    1990-01-01

    To investigate the immunological effect of Sizofilan (SPG) combined with radiotherapy, we evaluated the immunological parameters in 22 patients with uterine cervical cancer. Twelve patients were treated with SPG combined with radiotherapy (SPG group), and the other ten patients with radiotherapy only (control group). During radiotherapy, the numbers of lymphocyte and CD2 positive cell decreased in the SPG and control groups. After radiotherapy, however, its numbers in the SPG group became significantly higher than in the control group (p<0.05). The number of CD3 positive cell also presented a tendency to increase after radiotherapy in the SPG group. As for CD20 positive cell, its numbers were kept unchanged after radiotherapy in both groups, and no significant difference was observed between them. NK cell activity decreased during radiotherapy in both groups. After radiotherapy, its activity in the SPG group recovered to its pre-value and became significantly higher than that in the control group (p<0.05). SPG did not have any prominent effect on CD4/CD8 ratio. The adverse effect of SPG to liver or kidney function was not observed in our patients. The SCC level in the SPG group decreased rapidly by radiotherapy as well as that in the control group, and no significant difference was observed in SCC levels between them. So it was suggested that SPG did not suppress the cytocidal effect of radiation to cancer cells. Based on these findings, it was concluded that SPG prompted the recovery of not only lymphocyte, especially T cell, but also NK cell activity. These immunological findings presented a usefulness of clinical application of SPG to radiotherapy in patients with uterine cervical cancer. (author)

  15. Genome-wide Analyses Identify KIF5A as a Novel ALS Gene.

    Science.gov (United States)

    Nicolas, Aude; Kenna, Kevin P; Renton, Alan E; Ticozzi, Nicola; Faghri, Faraz; Chia, Ruth; Dominov, Janice A; Kenna, Brendan J; Nalls, Mike A; Keagle, Pamela; Rivera, Alberto M; van Rheenen, Wouter; Murphy, Natalie A; van Vugt, Joke J F A; Geiger, Joshua T; Van der Spek, Rick A; Pliner, Hannah A; Shankaracharya; Smith, Bradley N; Marangi, Giuseppe; Topp, Simon D; Abramzon, Yevgeniya; Gkazi, Athina Soragia; Eicher, John D; Kenna, Aoife; Mora, Gabriele; Calvo, Andrea; Mazzini, Letizia; Riva, Nilo; Mandrioli, Jessica; Caponnetto, Claudia; Battistini, Stefania; Volanti, Paolo; La Bella, Vincenzo; Conforti, Francesca L; Borghero, Giuseppe; Messina, Sonia; Simone, Isabella L; Trojsi, Francesca; Salvi, Fabrizio; Logullo, Francesco O; D'Alfonso, Sandra; Corrado, Lucia; Capasso, Margherita; Ferrucci, Luigi; Moreno, Cristiane de Araujo Martins; Kamalakaran, Sitharthan; Goldstein, David B; Gitler, Aaron D; Harris, Tim; Myers, Richard M; Phatnani, Hemali; Musunuri, Rajeeva Lochan; Evani, Uday Shankar; Abhyankar, Avinash; Zody, Michael C; Kaye, Julia; Finkbeiner, Steven; Wyman, Stacia K; LeNail, Alex; Lima, Leandro; Fraenkel, Ernest; Svendsen, Clive N; Thompson, Leslie M; Van Eyk, Jennifer E; Berry, James D; Miller, Timothy M; Kolb, Stephen J; Cudkowicz, Merit; Baxi, Emily; Benatar, Michael; Taylor, J Paul; Rampersaud, Evadnie; Wu, Gang; Wuu, Joanne; Lauria, Giuseppe; Verde, Federico; Fogh, Isabella; Tiloca, Cinzia; Comi, Giacomo P; Sorarù, Gianni; Cereda, Cristina; Corcia, Philippe; Laaksovirta, Hannu; Myllykangas, Liisa; Jansson, Lilja; Valori, Miko; Ealing, John; Hamdalla, Hisham; Rollinson, Sara; Pickering-Brown, Stuart; Orrell, Richard W; Sidle, Katie C; Malaspina, Andrea; Hardy, John; Singleton, Andrew B; Johnson, Janel O; Arepalli, Sampath; Sapp, Peter C; McKenna-Yasek, Diane; Polak, Meraida; Asress, Seneshaw; Al-Sarraj, Safa; King, Andrew; Troakes, Claire; Vance, Caroline; de Belleroche, Jacqueline; Baas, Frank; Ten Asbroek, Anneloor L M A; Muñoz-Blanco, José Luis; Hernandez, Dena G; Ding, Jinhui; Gibbs, J Raphael; Scholz, Sonja W; Floeter, Mary Kay; Campbell, Roy H; Landi, Francesco; Bowser, Robert; Pulst, Stefan M; Ravits, John M; MacGowan, Daniel J L; Kirby, Janine; Pioro, Erik P; Pamphlett, Roger; Broach, James; Gerhard, Glenn; Dunckley, Travis L; Brady, Christopher B; Kowall, Neil W; Troncoso, Juan C; Le Ber, Isabelle; Mouzat, Kevin; Lumbroso, Serge; Heiman-Patterson, Terry D; Kamel, Freya; Van Den Bosch, Ludo; Baloh, Robert H; Strom, Tim M; Meitinger, Thomas; Shatunov, Aleksey; Van Eijk, Kristel R; de Carvalho, Mamede; Kooyman, Maarten; Middelkoop, Bas; Moisse, Matthieu; McLaughlin, Russell L; Van Es, Michael A; Weber, Markus; Boylan, Kevin B; Van Blitterswijk, Marka; Rademakers, Rosa; Morrison, Karen E; Basak, A Nazli; Mora, Jesús S; Drory, Vivian E; Shaw, Pamela J; Turner, Martin R; Talbot, Kevin; Hardiman, Orla; Williams, Kelly L; Fifita, Jennifer A; Nicholson, Garth A; Blair, Ian P; Rouleau, Guy A; Esteban-Pérez, Jesús; García-Redondo, Alberto; Al-Chalabi, Ammar; Rogaeva, Ekaterina; Zinman, Lorne; Ostrow, Lyle W; Maragakis, Nicholas J; Rothstein, Jeffrey D; Simmons, Zachary; Cooper-Knock, Johnathan; Brice, Alexis; Goutman, Stephen A; Feldman, Eva L; Gibson, Summer B; Taroni, Franco; Ratti, Antonia; Gellera, Cinzia; Van Damme, Philip; Robberecht, Wim; Fratta, Pietro; Sabatelli, Mario; Lunetta, Christian; Ludolph, Albert C; Andersen, Peter M; Weishaupt, Jochen H; Camu, William; Trojanowski, John Q; Van Deerlin, Vivianna M; Brown, Robert H; van den Berg, Leonard H; Veldink, Jan H; Harms, Matthew B; Glass, Jonathan D; Stone, David J; Tienari, Pentti; Silani, Vincenzo; Chiò, Adriano; Shaw, Christopher E; Traynor, Bryan J; Landers, John E

    2018-03-21

    To identify novel genes associated with ALS, we undertook two lines of investigation. We carried out a genome-wide association study comparing 20,806 ALS cases and 59,804 controls. Independently, we performed a rare variant burden analysis comparing 1,138 index familial ALS cases and 19,494 controls. Through both approaches, we identified kinesin family member 5A (KIF5A) as a novel gene associated with ALS. Interestingly, mutations predominantly in the N-terminal motor domain of KIF5A are causative for two neurodegenerative diseases: hereditary spastic paraplegia (SPG10) and Charcot-Marie-Tooth type 2 (CMT2). In contrast, ALS-associated mutations are primarily located at the C-terminal cargo-binding tail domain and patients harboring loss-of-function mutations displayed an extended survival relative to typical ALS cases. Taken together, these results broaden the phenotype spectrum resulting from mutations in KIF5A and strengthen the role of cytoskeletal defects in the pathogenesis of ALS. Copyright © 2018 Elsevier Inc. All rights reserved.

  16. Recessive loss-of-function mutations in AP4S1 cause mild fever-sensitive seizures, developmental delay and spastic paraplegia through loss of AP-4 complex assembly

    Science.gov (United States)

    Hardies, Katia; May, Patrick; Djémié, Tania; Tarta-Arsene, Oana; Deconinck, Tine; Craiu, Dana; Helbig, Ingo; Suls, Arvid; Balling, Rudy; Weckhuysen, Sarah; De Jonghe, Peter; Hirst, Jennifer; Afawi, Zaid; Barisic, Nina; Baulac, Stéphanie; Caglayan, Hande; Depienne, Christel; De Kovel, Carolien G.F.; Dimova, Petia; Guerrero-López, Rosa; Guerrini, Renzo; Hjalgrim, Helle; Hoffman-Zacharska, Dorota; Jahn, Johanna; Klein, Karl Martin; Koeleman, Bobby P.C.; Leguern, Eric; Lehesjoki, Anna-Elina; Lemke, Johannes; Lerche, Holger; Marini, Carla; Muhle, Hiltrud; Rosenow, Felix; Serratosa, Jose M.; Møller, Rikke S.; Stephani, Ulrich; Striano, Pasquale; Talvik, Tiina; Von Spiczak, Sarah; Weber, Yvonne; Zara, Federico

    2015-01-01

    We report two siblings with infantile onset seizures, severe developmental delay and spastic paraplegia, in whom whole-genome sequencing revealed compound heterozygous mutations in the AP4S1 gene, encoding the σ subunit of the adaptor protein complex 4 (AP-4). The effect of the predicted loss-of-function variants (p.Gln46Profs*9 and p.Arg97*) was further investigated in a patient's fibroblast cell line. We show that the premature stop mutations in AP4S1 result in a reduction of all AP-4 subunits and loss of AP-4 complex assembly. Recruitment of the AP-4 accessory protein tepsin, to the membrane was also abolished. In retrospect, the clinical phenotype in the family is consistent with previous reports of the AP-4 deficiency syndrome. Our study reports the second family with mutations in AP4S1 and describes the first two patients with loss of AP4S1 and seizures. We further discuss seizure phenotypes in reported patients, highlighting that seizures are part of the clinical manifestation of the AP-4 deficiency syndrome. We also hypothesize that endosomal trafficking is a common theme between heritable spastic paraplegia and some inherited epilepsies. PMID:25552650

  17. Objections to the Libertarian Stem Cell Compromise

    Directory of Open Access Journals (Sweden)

    Walter E. Block

    2010-11-01

    Full Text Available In Block (2010 I offered a compromise between the pro choice position that fervently supports stem cell research, and the pro life philosophy which bitterly opposes it. The compromise was a contest: allow would be researchers to create as many fertilized eggs as they wished. But, also, these should be offered up to would be parents to adopt all of these “children” as they wanted. If and only if there were any unadopted fetuses remaining in the laboratories of the nation would it be licit, on libertarian grounds, for research on them to take place. In the present paper I respond to several objections to this “modest proposal.”

  18. Spinal Trauma is Never without Sin: A Tetraplegia Patient Presented Without any Symptoms

    OpenAIRE

    EFEOGLU, Melis; AKOGLU, Haldun; AKOGLU, Tayfun; EROGLU, Serkan Emre; ONUR, Ozge Ecmel; DENIZBASI, Arzu

    2016-01-01

    SUMMARY: Spinal cord injuries are amongst the most dangerous injuries, leading to high mortality and morbidity. Injured patients are occasionally faced with life-threatening complications and quality-of-life changing neurological deficits. Thoracic and cervical spinal segments are the most effected sites of injury and a wide range of complications including paraplegia, respiratory and cardiovascular compromise secondary to autonomic dysfunction or tetraplegia may ensue. We aim to draw attenti...

  19. The Drosophila DOCK family protein Sponge is required for development of the air sac primordium

    Energy Technology Data Exchange (ETDEWEB)

    Morishita, Kazushge; Anh Suong, Dang Ngoc; Yoshida, Hideki; Yamaguchi, Masamitsu, E-mail: myamaguc@kit.ac.jp

    2017-05-15

    Dedicator of cytokinesis (DOCK) family genes are known as DOCK1-DOCK11 in mammals. DOCK family proteins mainly regulate actin filament polymerization and/or depolymerization and are GEF proteins, which contribute to cellular signaling events by activating small G proteins. Sponge (Spg) is a Drosophila counterpart to mammalian DOCK3/DOCK4, and plays a role in embryonic central nervous system development, R7 photoreceptor cell differentiation, and adult thorax development. In order to conduct further functional analyses on Spg in vivo, we examined its localization in third instar larval wing imaginal discs. Immunostaining with purified anti-Spg IgG revealed that Spg mainly localized in the air sac primordium (ASP) in wing imaginal discs. Spg is therefore predicted to play an important role in the ASP. The specific knockdown of Spg by the breathless-GAL4 driver in tracheal cells induced lethality accompanied with a defect in ASP development and the induction of apoptosis. The monitoring of ERK signaling activity in wing imaginal discs by immunostaining with anti-diphospho-ERK IgG revealed reductions in the ERK signal cascade in Spg knockdown clones. Furthermore, the overexpression of D-raf suppressed defects in survival and the proliferation of cells in the ASP induced by the knockdown of Spg. Collectively, these results indicate that Spg plays a critical role in ASP development and tracheal cell viability that is mediated by the ERK signaling pathway. - Highlights: • Spg mainly localizes in the air sac primordium in wing imaginal discs. • Spg plays a critical role in air sac primordium development. • Spg positively regulates the ERK signal cascade.

  20. 7 CFR 1956.68 - Compromise or adjustment without debtor's signature.

    Science.gov (United States)

    2010-01-01

    ... 7 Agriculture 14 2010-01-01 2009-01-01 true Compromise or adjustment without debtor's signature... Loan Programs and Multi-Family Housing § 1956.68 Compromise or adjustment without debtor's signature... made to obtain the debtor's signature and the date(s) of such effort. (c) The specific reasons why it...

  1. 32 CFR 842.99 - Compromise, termination, and suspension of collection.

    Science.gov (United States)

    2010-07-01

    ... 32 National Defense 6 2010-07-01 2010-07-01 false Compromise, termination, and suspension of collection. 842.99 Section 842.99 National Defense Department of Defense (Continued) DEPARTMENT OF THE AIR... States (31 U.S.C. 3701, 3711-3719) § 842.99 Compromise, termination, and suspension of collection. This...

  2. 10 CFR 15.41 - When a claim may be compromised.

    Science.gov (United States)

    2010-01-01

    ... it has not been referred to DOJ for litigation. (b) Unless otherwise provided by law, when the... with the DOJ. The NRC will evaluate the compromise offer, using the factors set forth in this part. If an offer to compromise any debt in excess of $100,000 is acceptable to the NRC, the NRC shall refer...

  3. Strained Compromises? Danish Flexicurity During Crisis

    DEFF Research Database (Denmark)

    Ibsen, Christian Lyhne

    2011-01-01

    The Danish concept of flexicurity in a ‘Golden Triangle’ of low job protection, high income security and high employment security is not only about a balance between labor market flexibility and social security. Arguably, it is also a series of more or less stable underlying compromises between...

  4. Interleukin-4 and interleukin-13 compromise the sinonasal epithelial barrier and perturb intercellular junction protein expression.

    Science.gov (United States)

    Wise, Sarah K; Laury, Adrienne M; Katz, Elizabeth H; Den Beste, Kyle A; Parkos, Charles A; Nusrat, Asma

    2014-05-01

    Altered expression of epithelial intercellular junction proteins has been observed in sinonasal biopsies from nasal polyps and epithelial layers cultured from nasal polyp patients. These alterations comprise a "leaky" epithelial barrier phenotype. We hypothesize that T helper 2 (Th2) cytokines interleukin (IL)-4 and IL-13 modulate epithelial junction proteins, thereby contributing to the leaky epithelial barrier. Differentiated primary sinonasal epithelial layers cultured at the air-liquid interface were exposed to IL-4, IL-13, and controls for 24 hours at 37°C. Epithelial resistance measurements were taken every 4 hours during cytokine exposure. Western blot and immunofluorescence staining/confocal microscopy were used to assess changes in a panel of tight and adherens junction proteins. Western blot densitometry was quantified with image analysis. IL-4 and IL-13 exposure resulted in a mean decrease in transepithelial resistance at 24 hours to 51.6% (n = 6) and 68.6% (n = 8) of baseline, respectively. Tight junction protein junctional adhesion molecule-A (JAM-A) expression decreased 42.2% with IL-4 exposure (n = 9) and 37.5% with IL-13 exposure (n = 9). Adherens junction protein E-cadherin expression decreased 35.3% with IL-4 exposure (n = 9) and 32.9% with IL-13 exposure (n = 9). Tight junction protein claudin-2 showed more variability but had a trend toward higher expression with Th2 cytokine exposure. There were no appreciable changes in claudin-1, occludin, or zonula occludens-1 (ZO-1) with IL-4 or IL-13 exposure. Sinonasal epithelial exposure to Th2 cytokines IL-4 and IL-13 results in alterations in intercellular junction proteins, reflecting increased epithelial permeability. Such changes may explain some of the phenotypic manifestations of Th2-mediated sinonasal disease, such as edema, nasal discharge, and environmental reactivity. © 2014 ARS-AAOA, LLC.

  5. IL-4 and IL-13 Compromise the Sinonasal Epithelial Barrier and Perturb Intercellular Junction Protein Expression

    Science.gov (United States)

    Wise, Sarah K.; Laury, Adrienne M.; Katz, Elizabeth H.; Den Beste, Kyle A.; Parkos, Charles A.; Nusrat, Asma

    2014-01-01

    Introduction Altered expression of epithelial intercellular junction proteins has been observed in sinonasal biopsies from nasal polyps and epithelial layers cultured from nasal polyp patients. These alterations comprise a “leaky” epithelial barrier phenotype. We hypothesize that Th2 cytokines IL-4 and IL-13 modulate epithelial junction proteins thereby contributing to the leaky epithelial barrier. Methods Differentiated primary sinonasal epithelial layers cultured at the air-liquid interface were exposed to IL-4, IL-13, and controls for 24 hours at 37°C. Epithelial resistance measurements were taken every 4 hours during cytokine exposure. Western blot and immunofluorescence staining/confocal microscopy were used to assess changes in a panel of tight and adherens junction proteins. Western blot densitometry was quantified with image analysis. Results IL-4 and IL-13 exposure resulted in a mean decrease in transepithelial resistance at 24 hours to 51.6% (n=6) and 68.6% (n=8) of baseline, respectively. Tight junction protein JAM-A expression decreased 42.2% with IL-4 exposure (n=9) and 37.5% with IL-13 exposure (n=9). Adherens junction protein E-cadherin expression decreased 35.3% with IL-4 exposure (n=9) and 32.9% with IL-13 exposure (n=9). Tight junction protein claudin-2 showed more variability but had a trend toward higher expression with Th2 cytokine exposure. There were no appreciable changes in claudin-1, occludin, or ZO-1 with IL-4 or IL-13 exposure. Conclusion Sinonasal epithelial exposure to Th2 cytokines IL-4 and IL-13 results in alterations in intercellular junction proteins, reflecting increased epithelial permeability. Such changes may explain some of the phenotypic manifestations of Th2-mediated sinonasal disease, such as edema, nasal discharge, and environmental reactivity. PMID:24510479

  6. Securing Single Points of Compromise (SPoC)

    Energy Technology Data Exchange (ETDEWEB)

    Belangia, David Warren [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

    2015-06-25

    Securing the Single Points of Compromise that provide central services to the institution’s environment is paramount to success when trying to protect the business. (Fisk, 2014) Time Based Security mandates protection (erecting and ensuring effective controls) that last longer than the time to detect and react to a compromise. When enterprise protections fail, providing additional layered controls for these central services provides more time to detect and react. While guidance is readily available for securing the individual critical asset, protecting these assets as a group is not often discussed. Using best business practices to protect these resources as individual assets while leveraging holistic defenses for the group increases the opportunity to maximize protection time, allowing detection and reaction time for the SPoCs that is commensurate with the inherent risk of these centralized services.

  7. The voltage-gated potassium channel subunit, Kv1.3, is expressed in epithelia

    DEFF Research Database (Denmark)

    Grunnet, Morten; Rasmussen, Hanne B; Hay-Schmidt, Anders

    2003-01-01

    The Shaker-type voltage-gated potassium channel, Kv1.3, is believed to be restricted in distribution to lymphocytes and neurons. In lymphocytes, this channel has gained intense attention since it has been proven that inhibition of Kv1.3 channels compromise T lymphocyte activation. To investigate...

  8. Alchemy or Science? Compromising Archaeology in the Deep Sea

    Science.gov (United States)

    Adams, Jonathan

    2007-06-01

    In the torrid debate between archaeology and treasure hunting, compromise is often suggested as the pragmatic solution, especially for archaeology carried out either in deep water or beyond the constraints that commonly regulate such activities in territorial seas. Both the wisdom and the need for such compromise have even been advocated by some archaeologists, particularly in forums such as the internet and conferences. This paper argues that such a compromise is impossible, not in order to fuel confrontation but simply because of the nature of any academic discipline. We can define what archaeology is in terms of its aims, theories, methods and ethics, so combining it with an activity founded on opposing principles must transform it into something else. The way forward for archaeology in the deep sea does not lie in a contradictory realignment of archaeology’s goals but in collaborative research designed to mesh with emerging national and regional research and management plans.

  9. Strained Compromises? Danish Flexicurity During Crisis

    Directory of Open Access Journals (Sweden)

    Christian Lyhne Ibsen

    2011-01-01

    Full Text Available The Danish concept of flexicurity in a `Golden Triangle´ of low job protection, high income security and high employment security is not only about a balance between labor market flexibility and social security. Arguably, it is also a series of more or less stable underlying compromises between social partners about the main mechanisms and aims of labor market regulation which - supposedly - should be focused on employment rather than jobs, and competition on quality rather than on labor costs. However, the `Golden Triangle´ - this article argues - seems in need of complementary concepts. The article therefore introduces, `centralized decentralization´ - a concept that directs our attention to forms of flexibility and security primarily for people in work. Most studies on Danish flexicurity have been carried out under favorable economic conditions. In light of the economic slump hitting Denmark in 2008, this article investigates if and how the recession challenged these compromises by comparing two rounds of case-based interviews in three metalworking companies in 2007 and 2009. It is shown that practice has indeed changed - albeit modestly - due to worsened economic circumstances. For example the case studies show that the hypothesized preference for external numerical flexibility is perhaps too crude as employers use different ways to restructure employment. Especially the examples of de facto concessionary bargaining to save jobs are important here - although the extent of concessions is modest. The evidence thus suggests that the `Golden Triangle´ flexicurity compromises are indeed strained by the economic cycle and that responses to impetus for restructuring are far more nuanced than sometimes portrayed. It is argues that more company studies across national labor markets and industrial relations institutions will enhance our understanding of the dynamics during times of restructuring.

  10. Intensity of Mobile Phone Use and Health Compromising Behaviours--How Is Information and Communication Technology Connected to Health-Related Lifestyle in Adolescence?

    Science.gov (United States)

    Leena, Koivusilta; Tomi, Lintonen; Arja, Rimpela

    2005-01-01

    The association of mobile phone use with health compromising behaviours (smoking, snuffing, alcohol) was studied in a survey comprising a representative sample of 14-16-year-olds (N=3485) in 2001. Mobile phone was used by 89% of respondents and by 13% for at least 1 h daily. The intensity of use was positively associated with health compromising…

  11. COMPROMISE EFFECT ON CONSUMERS’ BEHAVIOR

    Directory of Open Access Journals (Sweden)

    Markus Surkamta Eric Santosa

    2016-09-01

    Full Text Available The way consumers think about the products they will buy determines their buying behavior. The decision to buy a particular product is obviously in accordance with the buyer’s attitude. The buyers will also feel more comfortable if their behavior meets with the approval and expectations of the people close to them. While the development of a certain attitude has no effect on subjective judgment, the effect of compromise is likely to make a contribution to its development. Since it is still unclear, this study’s main purposed is to clarify this. In addition, while an attitude is theorized as being a predictor of behavior, through behavioral intention, the study’s secondary purpose is to boost the earlier findings. Likewise, in accordance with the theory of planned behavior, the study will also examine the other predictors of behavioral intention, in relation to the behavioral intention itself. A sample consisting of a 100 respondents was compiled by using the convenience and judgment technique. The data were analyzed using Amos 16.0 and SPSS 16.0. As expected, the compromise effect had a significant influence on whether the customers’ attitude or the subjective norm was the main determinant. Likewise, the customers’ attitude, the subjective norm and perceived behavioral control were confirmed as good predictors of customers’ behavioral intentions.

  12. Bluish Discolouration of Urine Drainage Tube and Bag in a Female Patient with Spina Bifida, Paraplegia, and Suprapubic Cystostomy

    Directory of Open Access Journals (Sweden)

    Subramanian Vaidyanathan

    2007-01-01

    Full Text Available We present a female patient with spina bifida, paraplegia, suprapubic cystostomy, and chronic constipation, who became anxious when she noticed a bluish discolouration of her urine drainage system. Urine microbiology revealed growth of Providencia stuartii and Staphylococcus aureus. There were no systemic features of infection and, therefore, antibiotics were not prescribed for asymptomatic bacteriuria. This patient was advised to change the urine bag every day, and was prescribed senna to facilitate bowel evacuation. She was reassured that bluish discolouration of the urine drainage tube and bag was a transient, benign phenomenon and not indicative of any underlying pathology. Over the next 7 days, the bluish discolouration gradually faded away. Clinical characteristics of patients who are likely to develop this phenomenon and the underlying biochemical mechanism for bluish discolouration of the urine drainage system are discussed in brief.

  13. Paraplegia is not a diagnosis: Spinal tuberculosis deserves a place on the clinical radar screen: Awakening call to clinicians

    Directory of Open Access Journals (Sweden)

    Liaqat Ali Chaudhry

    2012-01-01

    Full Text Available Disseminated Tuberculosis(TB occurs through lymphohaematogenous dissemination of M. tuberculosis. Although it is more common in young children and those who are having risk factors like having acquired or innate immunodeficiency, weak immune system being on immunosuppressive treatments; it can also occur in immunocompetent indviduals with heavy load of infection. Inhalation being the commonest mode of entry of the pathogen results in pulmonary tuberculosis being the commonest seat of primary infection,other systems commonly involved as extra-pulmonary sites are lymphnodes, bone, brain, peritonium and genitalia depending on the amount of relative blood supply and lymphatics. We present here case of a young female who was immunocompetent, having fever, progressive lower limb weakness and loss of bowel and bladder control, becoming bed bound, and admitted in our hospital for rehabilitation with a diagnosis of paraplegia.

  14. [When a patient falls (asleep) and can't get up: conversion disorder - paraplegia following general anesthesia].

    Science.gov (United States)

    Mason, Chawla LaToya

    This case report describes the rare occurrence of paraplegia caused by conversion disorder in a woman who received general anesthesia for breast surgery. A 46-year-old healthy woman received general anesthesia for excision of a left breast fibroepithelial lesion. In the post-anesthesia care unit, she reported bilateral loss of both sensation and motor function below the knees. Physical signs and symptoms did not correlate with any anatomical or neurological patterns; imaging revealed no abnormalities. Psychiatric consultation was performed wherein familial stressor circumstances were identified, leading to diagnosis and management of conversion disorder. Conversion disorder is characterized by alteration of physical function due to expression of an underlying psychological ailment. Its diagnosis requires thorough evaluation including appropriate workup to exclude organic causes. The meshing together of anesthesiology and psychiatry - as demonstrated by this case report - offers an opportunity to highlight important information pertaining to the definition, diagnosis, and management of conversion disorder as it may be encountered in the postanesthesia recovery period. Copyright © 2015 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

  15. MUTUAL CONCESSIONS - SPECIFIC ELEMENT OF THE COMPROMISE/TRANSACTION CONTRACT

    Directory of Open Access Journals (Sweden)

    Georgeta-Bianca Spîrchez

    2012-11-01

    Full Text Available Given the usefulness and practical importance of the compromise contract conclusion and of the amicably dispute resolution, within the business world, we aim to analyze, in what follows, the concrete means by which these kind of settlement are achieved. Two questions become legitimate in the context of concerns about mutual concessions which the parties make in a compromise contract. These questions are the following: “What are the mutual concessions? Do mutual concessions mean equivalent concessions?” and “How mutual concessions are required to complete a valid settlement? Is the requirement of mutual concessions grounded?”

  16. Knowledge is power: averting safety-compromising events in the OR.

    Science.gov (United States)

    Catalano, Kathleen

    2008-12-01

    Surgical procedures can be unpredictable, and safety-compromising events can jeopardize patient safety. Perioperative nurses should be watchful for factors that can contribute to safety-compromising events, as well as the errors that can follow, and know how to avert them if possible. Knowledge is power and increased awareness of patient safety issues and the resources that are available to both health care practitioners and consumers can help perioperative nurses ward off patient safety problems before they occur.

  17. Vatican is lone opponent of world conference's compromises on abortion.

    Science.gov (United States)

    1994-09-07

    Three years in the making, the draft program of action of the 1994 International Conference on Population and Development sets nonbinding policy guidelines to contain the world's population at 7.27 billion in 2015. Although the Vatican was pleased to see Pakistan put forward a compromise formula developed to appease Catholic and Muslim objectors of abortion, the Church was unprepared to accept the compromise immediately and requested further discussion. The Vatican's rejection drew a strong chorus of vocal disapproval from other conference delegates. Even Iran accepted the draft as a "perfect text," while Sweden grudgingly accepted it as a "rock-bottom compromise." With no Catholic countries objecting to the compromise, the Vatican stood alone in its refusal to compromise with the rest of the world's leaders and peoples. Germany, speaking for the European Union, warned that enough concessions had already been made. The rationale for Vatican opposition was unclear since the section explicitly rejects abortion as a means of family planning and urges countries to minimize both the incidence of unsafe abortion and abortion overall by improving family planning. Prevention of unwanted pregnancies must be given highest priority and women should have ready access to compassionate counselling, with abortion never promoted as a means of family planning. Moreover, there is no longer a reference to sexual health education, a plea to governments to review their laws and policies on abortion, and a call to consider women's health rather than relying upon criminal codes and punitive measures. Participants said the Vatican objected to a phrase stating that abortions, where legal, should be safe, while the Church representative argued that any suggestion that abortion is safe contradicts church doctrine on the sanctity of life.

  18. Unusual Presentation of a Primary Ewing's Sarcoma of the Spine with Paraplegia: A Case Report.

    Science.gov (United States)

    Kannan, Karthik Kailash; Sundarapandian, Rajkumar Jayachandran; Surulivel, Vignesh Jayabalan

    2015-03-01

    Ewing's sarcoma is a primary malignancy of the bone affecting individuals in the second decade of life. Primary sarcomas of the spine are rare and the occurrence of Primary Ewing's sarcoma in the spine is very rare. Ewing's sarcoma occurring in the spine is divided into two types, Ewing's sarcoma of sacral spine which are very aggressive with poor prognosis and Ewing's sarcoma of the non sacral spine which is an extremely rare occurrence. Patient may present with neurological deficit when the tumour extends into the spinal canal causing spinal cord compression. Magnetic resonance imaging (MRI) is very sensitive in diagnosing the tumour and defining the extent of the tumour. Here we report an 18-year-old boy who presented with back pain and complete paraplegia of two months duration. The MRI gave a differential diagnosis of infective pathology due to the fluid collection in the paraspinal region, followed by primary malignancy as the second diagnosis. Patient underwent posterior spinal decompression and stabilization, and intaoperatively there was significant collection of pus whose culture showed no growth. The histopathology and immunohistochemistry studies confirmed the diagnosis of Ewing's sarcoma and patient was started on combination chemotherapy and radiotherapy.

  19. Alteration of Fatty-Acid-Metabolizing Enzymes Affects Mitochondrial Form and Function in Hereditary Spastic Paraplegia

    Science.gov (United States)

    Tesson, Christelle; Nawara, Magdalena; Salih, Mustafa A.M.; Rossignol, Rodrigue; Zaki, Maha S.; Al Balwi, Mohammed; Schule, Rebecca; Mignot, Cyril; Obre, Emilie; Bouhouche, Ahmed; Santorelli, Filippo M.; Durand, Christelle M.; Oteyza, Andrés Caballero; El-Hachimi, Khalid H.; Al Drees, Abdulmajeed; Bouslam, Naima; Lamari, Foudil; Elmalik, Salah A.; Kabiraj, Mohammad M.; Seidahmed, Mohammed Z.; Esteves, Typhaine; Gaussen, Marion; Monin, Marie-Lorraine; Gyapay, Gabor; Lechner, Doris; Gonzalez, Michael; Depienne, Christel; Mochel, Fanny; Lavie, Julie; Schols, Ludger; Lacombe, Didier; Yahyaoui, Mohamed; Al Abdulkareem, Ibrahim; Zuchner, Stephan; Yamashita, Atsushi; Benomar, Ali; Goizet, Cyril; Durr, Alexandra; Gleeson, Joseph G.; Darios, Frederic; Brice, Alexis; Stevanin, Giovanni

    2012-01-01

    Hereditary spastic paraplegia (HSP) is considered one of the most heterogeneous groups of neurological disorders, both clinically and genetically. The disease comprises pure and complex forms that clinically include slowly progressive lower-limb spasticity resulting from degeneration of the corticospinal tract. At least 48 loci accounting for these diseases have been mapped to date, and mutations have been identified in 22 genes, most of which play a role in intracellular trafficking. Here, we identified mutations in two functionally related genes (DDHD1 and CYP2U1) in individuals with autosomal-recessive forms of HSP by using either the classical positional cloning or a combination of whole-genome linkage mapping and next-generation sequencing. Interestingly, three subjects with CYP2U1 mutations presented with a thin corpus callosum, white-matter abnormalities, and/or calcification of the basal ganglia. These genes code for two enzymes involved in fatty-acid metabolism, and we have demonstrated in human cells that the HSP pathophysiology includes alteration of mitochondrial architecture and bioenergetics with increased oxidative stress. Our combined results focus attention on lipid metabolism as a critical HSP pathway with a deleterious impact on mitochondrial bioenergetic function. PMID:23176821

  20. Adolescent Health-Compromising Behaviors: Motivating School Counselors.

    Science.gov (United States)

    Nagel, Liza; Scherer, David G.; Lee, William

    2000-01-01

    Investigated middle and high school counselors' perceptions of adolescent health-compromising behaviors and motivations to intervene. Data from a survey based on protection motivation theory showed differences in counselors' perceptions of the severity of risk-taking behaviors. Perceptions were highly correlated with intentions to seek out…

  1. Predicting intrapartum fetal compromise using the fetal cerebro-umbilical ratio.

    Science.gov (United States)

    Sabdia, S; Greer, R M; Prior, T; Kumar, S

    2015-05-01

    The aim of this study was to explore the association between the cerebro-umbilical ratio measured at 35-37 weeks and intrapartum fetal compromise. This retrospective cross sectional study was conducted at the Mater Mothers' Hospital in Brisbane, Australia. Maternal demographics and fetal Doppler indices at 35-37 weeks gestation for 1381 women were correlated with intrapartum and neonatal outcomes. Babies born by caesarean section or instrumental delivery for fetal compromise had the lowest median cerebro-umbilical ratio 1.60 (IQR 1.22-2.08) compared to all other delivery groups (vaginal delivery, emergency delivery for failure to progress, emergency caesarean section for other reasons or elective caesarean section). The percentage of infants with a cerebro-umbilical ratio cerebro-umbilical ratio between the 10th-90th centile and 9.6% of infants with a cerebro-umbilical ratio > 90th centile required delivery for the same indication (p cerebro-umbilical ratio was associated with an increased risk of emergency delivery for fetal compromise, OR 2.03 (95% CI 1.41-2.92), p cerebro-umbilical ratio measured at 35-37 weeks is associated with a greater risk of intrapartum compromise. This is a relatively simple technique which could be used to risk stratify women in diverse healthcare settings. Copyright © 2015 Elsevier Ltd. All rights reserved.

  2. The medically compromised patient: Are dental implants a feasible option?

    Science.gov (United States)

    Vissink, A; Spijkervet, Fkl; Raghoebar, G M

    2018-03-01

    In healthy subjects, dental implants have evolved to be a common therapy to solve problems related to stability and retention of dentures as well as to replace failing teeth. Although dental implants are applied in medically compromised patients, it is often not well known whether this therapy is also feasible in these patients, whether the risk of implant failure and developing peri-implantitis is increased, and what specific preventive measures, if any, have to be taken when applying dental implants in these patients. Generally speaking, as was the conclusion by the leading review of Diz, Scully, and Sanz on placement of dental implants in medically compromised patients (J Dent, 41, 2013, 195), in a few disorders implant survival may be lower, and the risk of a compromised peri-implant health and its related complications be greater, but the degree of systemic disease control outweighs the nature of the disorder rather than the risk accompanying dental implant treatment. So, as dental implant treatment is accompanied by significant functional benefits and improved oral health-related quality of life, dental implant therapy is a feasible treatment in almost any medically compromised patient when the required preventive measures are taken and follow-up care is at a high level. © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. All rights reserved.

  3. Measuring the collateral network pressure to minimize paraplegia risk in thoracoabdominal aneurysm resection.

    Science.gov (United States)

    Etz, Christian D; Zoli, Stefano; Bischoff, Moritz S; Bodian, Carol; Di Luozzo, Gabriele; Griepp, Randall B

    2010-12-01

    To minimize paraplegia during thoracoabdominal aortic aneurysm repair, the concept of the collateral network was developed. That is, spinal cord perfusion is provided by an interconnecting complex of vessels in the intraspinal, paraspinous, and epidural space and in the paravertebral muscles, including intercostal and lumbar segmental as well as subclavian and hypogastric arteries. Collateral network pressure was measured with a catheter in the distal end of a ligated segmental artery in pigs and human beings. In the pig, collateral network pressure was 75% of the simultaneous mean aortic pressure. With complete segmental arterial ligation, it fell to 27% of baseline, recovering to 40% at 24 hours and 90% at 120 hours. Spinal cord injury occurred in approximately 50% of animals. When all segmental arteries were taken in 2 stages a week apart, collateral network pressure fell only to 50% to 70% of baseline, and spinal cord injury was rare. In human beings, baseline collateral network pressure also was 75% of mean aortic pressure, fell in proportion to the number of segmental arteries ligated, and began recovery within 24 hours. Collateral network pressure was lower with nonpulsatile distal bypass than with pulsatile perfusion. After subtraction of a measure of spinal cord outflow pressure (cerebrospinal fluid pressure or central venous pressure), collateral network pressure provides a clinically useful estimate of spinal cord perfusion pressure. Copyright © 2010. Published by Mosby, Inc.

  4. 14 CFR 1261.414 - Compromise of claims.

    Science.gov (United States)

    2010-01-01

    ... potential income; (3) Inheritance prospects; (4) The possibility that assets have been concealed or... compromised. The practical benefits of vigorous collection of a small claim may include a demonstration to... requiring a waiver of the tax-loss-carry-back rights of the debtor. (i) Joint and several liability. When...

  5. 31 CFR 902.2 - Bases for compromise.

    Science.gov (United States)

    2010-07-01

    ... 31 Money and Finance: Treasury 3 2010-07-01 2010-07-01 false Bases for compromise. 902.2 Section 902.2 Money and Finance: Treasury Regulations Relating to Money and Finance (Continued) FEDERAL CLAIMS... necessary to further an enforcement principle, such as the Government's willingness to pursue aggressively...

  6. A generalized Frattini subgroup of a finite group

    Directory of Open Access Journals (Sweden)

    Prabir Bhattacharya

    1989-01-01

    Full Text Available For a finite group G and an arbitrary prime p, let SP(G denote the intersection of all maximal subgroups M of G such that [G:M] is both composite and not divisible by p; if no such M exists we set SP(G = G. Some properties of G are considered involving SP(G. In particular, we obtain a characterization of G when each M in the definition of SP(G is nilpotent.

  7. Factors influencing treatment decision-making for maintaining or extracting compromised teeth.

    Science.gov (United States)

    Lang-Hua, Bich Hue; McGrath, Colman P J; Lo, Edward C M; Lang, Niklaus P

    2014-01-01

    To evaluate treatment decision-making with respect to maintaining periodontally compromised teeth among dentists with or without postgraduate qualifications in implant dentistry. A series of patient scenarios with varying degrees of periodontal disease levels was presented to dental practitioners. Practitioners' decision-making outcome was determined, and intention to retain the compromised teeth was analyzed in bivariate and regression analyses (accounting for postgraduate implant training, gender, years in dental practice, and implant placement experience). This study involved 30 dental practitioners with postgraduate implant qualifications (GDPP), 33 dental practitioners without postgraduate implant qualifications (GDP), and 27 practitioners undergoing training for postgraduate implant qualifications (GDPT). Variations in treatment decision-making were evident between the three groups. Differences in treatment approaches to retaining compromised teeth were apparent. Furthermore, variations in rehabilitation of extracted scenarios existed in terms of use of implant and number of implants need for rehabilitation. Accounting for dentist and practice factors in regression analyses, GDPP/GDPT were three times as likely to retain periodontally compromised upper molar, with or without pain, compared to GDP (without pain OR 3.10, 95%CI 1.04, 10.62 P = 0.04; with pain OR 3.08, 95%CI 1.09, 8.14 P = 0.03). Variations in treatment decision-making with respect to retaining periodontally compromised teeth exist between dental practitioners with and those without postgraduate training in implant dentistry. Furthermore differences in management approaches in how they would retain the teeth or rehabilitate the dental arch were apparent. © 2013 John Wiley & Sons A/S. Published by Blackwell Publishing Ltd.

  8. The role of electron localization in the atomic structure of transition-metal 13-atom clusters: the example of Co13, Rh13, and Hf13.

    Science.gov (United States)

    Piotrowski, Maurício J; Piquini, Paulo; Cândido, Ladir; Da Silva, Juarez L F

    2011-10-14

    The crystalline structure of transition-metals (TM) has been widely known for several decades, however, our knowledge on the atomic structure of TM clusters is still far from satisfactory, which compromises an atomistic understanding of the reactivity of TM clusters. For example, almost all density functional theory (DFT) calculations for TM clusters have been based on local (local density approximation--LDA) and semilocal (generalized gradient approximation--GGA) exchange-correlation functionals, however, it is well known that plain DFT fails to correct the self-interaction error, which affects the properties of several systems. To improve our basic understanding of the atomic and electronic properties of TM clusters, we report a DFT study within two nonlocal functionals, namely, the hybrid HSE (Heyd, Scuseria, and Ernzerhof) and GGA+U functionals, of the structural and electronic properties of the Co(13), Rh(13), and Hf(13) clusters. For Co(13) and Rh(13), we found that improved exchange-correlation functionals decrease the stability of open structures such as the hexagonal bilayer (HBL) and double simple-cubic (DSC) compared with the compact icosahedron (ICO) structure, however, DFT-GGA, DFT-GGA+U, and DFT-HSE yield very similar results for Hf(13). Thus, our results suggest that the DSC structure obtained by several plain DFT calculations for Rh(13) can be improved by the use of improved functionals. Using the sd hybridization analysis, we found that a strong hybridization favors compact structures, and hence, a correct description of the sd hybridization is crucial for the relative energy stability. For example, the sd hybridization decreases for HBL and DSC and increases for ICO in the case of Co(13) and Rh(13), while for Hf(13), the sd hybridization decreases for all configurations, and hence, it does not affect the relative stability among open and compact configurations.

  9. Fuzzy compromise: An effective way to solve hierarchical design problems

    Science.gov (United States)

    Allen, J. K.; Krishnamachari, R. S.; Masetta, J.; Pearce, D.; Rigby, D.; Mistree, F.

    1990-01-01

    In this paper, we present a method for modeling design problems using a compromise decision support problem (DSP) incorporating the principles embodied in fuzzy set theory. Specifically, the fuzzy compromise decision support problem is used to study hierarchical design problems. This approach has the advantage that although the system modeled has an element of uncertainty associated with it, the solution obtained is crisp and precise. The efficacy of incorporating fuzzy sets into the solution process is discussed in the context of results obtained for a portal frame.

  10. 45 CFR 1177.12 - Compromise, suspension and termination.

    Science.gov (United States)

    2010-10-01

    ... 45 Public Welfare 3 2010-10-01 2010-10-01 false Compromise, suspension and termination. 1177.12 Section 1177.12 Public Welfare Regulations Relating to Public Welfare (Continued) NATIONAL FOUNDATION ON...) debts arising from GAO audit exceptions. ...

  11. 15 CFR 19.7 - When will Commerce entities compromise a Commerce debt?

    Science.gov (United States)

    2010-01-01

    ... 15 Commerce and Foreign Trade 1 2010-01-01 2010-01-01 false When will Commerce entities compromise a Commerce debt? 19.7 Section 19.7 Commerce and Foreign Trade Office of the Secretary of Commerce COMMERCE DEBT COLLECTION Procedures To Collect Commerce Debts § 19.7 When will Commerce entities compromise...

  12. F-18-fluoride PET for early diagnosis and evaluation of therapeutic outcome in patients with heterotopic ossification (HO) after recent paraplegia due to spinal cord injury

    International Nuclear Information System (INIS)

    Baum, R.P.; Niesen, A.; Schmuecking, M.; Przetak, C.; Ruhwedel, H.; Boehm, H.

    2002-01-01

    Aim: Heterotopic ossification (HO) is the presence of bone in soft tissue. The acquired form of HO most frequently is seen with either musculoskeletal trauma, spinal cord injury or central nervous system injury. Fever, swelling, erythema, and occasional joint tenderness seen in early HO can be difficult to distinguish from cellulitis, osteomyelitis or thrombophlebitis. As compared to paraplegia alone, combination of HO and paraplegia, especially excessive delay of diagnosis, is associated with a significantly higher incidence for thrombosis, immobilization, decubitus leading to a reduced expectation and quality of life. To evaluate the role of F-18-fluoride PET for the early diagnosis and the evaluation of the therapeutic outcome, 38 patients (56 PET examinations) were analyzed prospectively. Material and Methods: Within 8 weeks after acquired paraplegia, each patient was studied by F-18-fluoride PET in addition to clinical, serologic and conventional radiographic examinations. Whole-body PET studies (ECAT Exact 47, attenuation corrected, iterative reconstruction) were obtained 150 min. after injection of 12 MBq F-18-fluoride/kg body weight. For semi-quantitative analysis, standardized-uptake values (SUV) and the metabolic transverse diameters (MTD) of the lesions were assessed. To prevent post-traumatic neurogenic HO, patients received physiotherapy and NSA (indomethacin 3 x 50 mg per day for 4 months). In addition, external beam radiation therapy (EBRT, 1x7 Gy according to ICRU) was administered, if PET demonstrated HO. Therapeutic outcome was compared with a historical group of patients receiving only physiotherapy. Kaplan-Meier-Method, log-rank-, chi-square- and Wilcoxon-test were used for statistical analyses. Results: In the course of HO, 4/38 patients received EBRT with 1x7 Gy for a second time due to an increasing SUV and/or MTD and rising levels of alkaline phosphatase. Within a follow-up period of at least 30 months, none of the patients showed clinical

  13. Results of endoscopic surgery and intralesional steroid therapy for airway compromise due to tracheobronchial Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    S. A. R. Nouraei

    2008-04-01

    Full Text Available Background: Upper airway compromise due to tracheobronchial stenosis commonly occurs in patients with Wegener's Granulomatosis (WG. There is at present no consensus on the optimal management of this life-threatening condition. Objective: To assess the results of laryngo-tracheo-bronchoscopy, intralesional steroid therapy, laser surgery, and dilatation in managing obstructive tracheobronchial WG. Methods: Records of eighteen previously-untreated stridulous patients with obstructive tracheobronchial WG, treated between 2004 and 2006 were prospectively recorded on an airway database and retrospectively reviewed. Information about patient and lesion characteristics and treatment details were recorded. Treatment progress was illustrated using a timeline plot, and intervention-free intervals were calculated with actuarial analysis. Results: There were nine males and the average age at presentation was 40 (16 years [range 13–74]. There were thirteen patients with tracheal, and five patients with tracheal and bronchial lesions. The average tracheal lesion height was 8 (3 mm, located 23 (9 mm below the glottis. There were 1, 10 and 7 Myer-Cotton grade I, II and III lesions respectively. Mean intervention-free interval following minimally-invasive treatment was 26 (2.8 months. Following endobronchial therapy the median intervention-free interval was 22 months (p>0.8 vs. tracheal lesions. No patient required a tracheostomy or endoluminal stenting. Conclusions: Intralesional steroid therapy and conservative endoluminal surgery is an effective strategy for treating airway compromise due to active tracheal and bronchial WG, obviating the need for airway bypass or stenting. We recommend the combination of endotracheal dilatation, conservative laser surgery and steroid therapy as the standard of care for treating airway compromise due to obstructive tracheobronchial WG.

  14. Radiofrequency ablation of sphenopalatine ganglion for head and neck cancer pain management

    Directory of Open Access Journals (Sweden)

    Priti R Sanghavi

    2017-01-01

    Full Text Available Introduction: Pain in advanced head and neck cancer is intractable and many a times difficult to manage with pharmacological agents. Sphenopalatine ganglion (SPG block provides excellent pain relief in patients who are suffering from various types of orofacial pain. Role of radiofrequency ablation (RFA of SPG is described for orofacial cancer pain. Aim: The aim of the study was to observe efficacy and duration of pain relief by RFA of SPG in advanced head and neck cancer patients. Materials and Methods: Thirty-three patients suffering from advanced head and neck cancer were enrolled in this study between September 2013 and February 2016. All patients underwent RFA of SPG, under fluoroscopy guidance following a successful diagnostic block of SPG with local anesthetic. Pain was assessed before the procedure, immediately after the procedure, and during each follow-up visit. Follow-up visits were weekly for 4 weeks and then monthly till the end of life. Patients were continued on oral morphine, but the dose was reduced to one-third of total dose and was adjusted according to patients' requirement during each follow-up. Duration of analgesia, morphine requirements, and incidence of complications were noted. Results: There were 32 males and one female. Mean age of patients was 43.24 ± 13.52 (mean ± standard deviation [SD], ranging from 19 to 58 years. Three patients did not undergo RFA due to cheek hematoma formation during the procedure. Visual analog score was reduced from 8.43 ± 1.10 (preprocedure to 1.36 ± 1.61 (postprocedure (mean ± SD. Mean duration of analgesia was 17.55 ± 26.12 (mean ± SD weeks. Mean reduction in the dose of morphine was from 124.65 ± 46.78 to 40.00 ± 18.05 mg (mean ± SD immediately after the procedure. One patient was followed up for 30 months. Sixteen patients died within 3 months and had good pain relief. None of the patients had any serious complications. Conclusion: RFA of SPG is a good adjuvant method of pain

  15. Roles of Melatonin in Fetal Programming in Compromised Pregnancies

    Directory of Open Access Journals (Sweden)

    Yu-Chieh Chen

    2013-03-01

    Full Text Available Compromised pregnancies such as those associated with gestational diabetes mellitus, intrauterine growth retardation, preeclampsia, maternal undernutrition, and maternal stress may negatively affect fetal development. Such pregnancies may induce oxidative stress to the fetus and alter fetal development through the epigenetic process that may affect development at a later stage. Melatonin is an oxidant scavenger that reverses oxidative stress during the prenatal period. Moreover, the role of melatonin in epigenetic modifications in the field of developmental programming has been studied extensively. Here, we describe the physiological function of melatonin in pregnancy and discuss the roles of melatonin in fetal programming in compromised pregnancies, focusing on its involvement in redox and epigenetic mechanisms.

  16. Roles of Melatonin in Fetal Programming in Compromised Pregnancies

    Science.gov (United States)

    Chen, Yu-Chieh; Sheen, Jiunn-Ming; Tiao, Miao-Meng; Tain, You-Lin; Huang, Li-Tung

    2013-01-01

    Compromised pregnancies such as those associated with gestational diabetes mellitus, intrauterine growth retardation, preeclampsia, maternal undernutrition, and maternal stress may negatively affect fetal development. Such pregnancies may induce oxidative stress to the fetus and alter fetal development through the epigenetic process that may affect development at a later stage. Melatonin is an oxidant scavenger that reverses oxidative stress during the prenatal period. Moreover, the role of melatonin in epigenetic modifications in the field of developmental programming has been studied extensively. Here, we describe the physiological function of melatonin in pregnancy and discuss the roles of melatonin in fetal programming in compromised pregnancies, focusing on its involvement in redox and epigenetic mechanisms. PMID:23466884

  17. The impact of purchase quantity on the compromise effect: The balance heuristic

    Directory of Open Access Journals (Sweden)

    Yin-Hui Cheng

    2012-07-01

    Full Text Available Most prior research on the compromise effect has focused on single rather than multiple choices. This research investigates the potential effects of purchase quantity on the compromise effect. We propose that the share of the middle option in a trinary choice set decreases as the purchase quantity increases, because people tend to employ a balance heuristic to distribute their multiple choices among the available options to achieve a balanced state and to satisfy their variety-seeking tendency. Furthermore, we propose that the need for justification and an optimal stimulation level moderate the relationship between the number of purchase items and the compromise effect. These proposed hypotheses are supported by results from three experiments.

  18. Burn from car seat heater in a man with paraplegia: case report

    Science.gov (United States)

    Benjamin, Cheryl; Gittler, Michelle; Lee, Ray

    2011-01-01

    Objective/background Heated car seats are a common feature in newer automobiles. They are increasingly being recognized as potential hazards as there have been multiple reports of significant burns to its users. The potential for harm is considerably increased in those with impaired sensation with the possibility of a devastating injury. Methods Case report and literature review. Results A 26-year-old male with a T8 ASIA A paraplegia presented to the outpatient clinic for management of a hip burn. Two weeks prior to his visit he was driving a 2004 Jeep Cherokee for approximately 30 minutes. He was unaware that the driver's side seat warmer was set on high. He denied that his seat belt was in direct contact with the skin of his right hip. He presented to an acute care hospital that evening with a hip burn where he was prescribed silver sulfadiazine cream and instructed to apply it until his scheduled follow-up clinic visit. In clinic, the hip wound was unstageable with approximately 95% eschar. A dressing of bismuth tribromophenate in petrolatum was applied to the wound and he was instructed to change the dressing daily. This was later changed to an antimicrobial alginate dressing. The ulcer eventually healed. Conclusions This case illustrates the significant risk of car seat heaters in individuals with spinal cord injuries or neurological impairment who have decreased sensation. Additionally, it highlights an atypical area of potential for burn. Furthermore, it emphasizes the need for a heightened awareness for this unique and dangerous situation. PMID:21756574

  19. 47 CFR 1.1915 - Exploration of compromise.

    Science.gov (United States)

    2010-10-01

    ... justification of the offer and addressing the bases for compromise at 31 CFR 902.2. Debtors will provide full.... Unless otherwise provided by law, when the principal balance of a debt, exclusive of interest, penalties... evaluate an offer, using the factors set forth in 31 CFR 902.2 and, as appropriate, refer the offer with...

  20. 36 CFR 1011.7 - When will the Presidio Trust compromise a debt?

    Science.gov (United States)

    2010-07-01

    ... 36 Parks, Forests, and Public Property 3 2010-07-01 2010-07-01 false When will the Presidio Trust... Procedures To Collect Presidio Trust Debts § 1011.7 When will the Presidio Trust compromise a debt? (a... debt owed to the Presidio Trust that is not recovered as the result of a compromise will be reported to...

  1. Poster - 52: Smoothing constraints in Modulated Photon Radiotherapy (XMRT) fluence map optimization

    International Nuclear Information System (INIS)

    McGeachy, Philip; Villarreal-Barajas, Jose Eduardo; Zinchenko, Yuriy; Khan, Rao

    2016-01-01

    Purpose: Modulated Photon Radiotherapy (XMRT), which simultaneously optimizes photon beamlet energy (6 and 18 MV) and fluence, has recently shown dosimetric improvement in comparison to conventional IMRT. That said, the degree of smoothness of resulting fluence maps (FMs) has yet to be investigated and could impact the deliverability of XMRT. This study looks at investigating FM smoothness and imposing smoothing constraint in the fluence map optimization. Methods: Smoothing constraints were modeled in the XMRT algorithm with the sum of positive gradient (SPG) technique. XMRT solutions, with and without SPG constraints, were generated for a clinical prostate scan using standard dosimetric prescriptions, constraints, and a seven coplanar beam arrangement. The smoothness, with and without SPG constraints, was assessed by looking at the absolute and relative maximum SPG scores for each fluence map. Dose volume histograms were utilized when evaluating impact on the dose distribution. Results: Imposing SPG constraints reduced the absolute and relative maximum SPG values by factors of up to 5 and 2, respectively, when compared with their non-SPG constrained counterparts. This leads to a more seamless conversion of FMS to their respective MLC sequences. This improved smoothness resulted in an increase to organ at risk (OAR) dose, however the increase is not clinically significant. Conclusions: For a clinical prostate case, there was a noticeable improvement in the smoothness of the XMRT FMs when SPG constraints were applied with a minor increase in dose to OARs. This increase in OAR dose is not clinically meaningful.

  2. Poster - 52: Smoothing constraints in Modulated Photon Radiotherapy (XMRT) fluence map optimization

    Energy Technology Data Exchange (ETDEWEB)

    McGeachy, Philip; Villarreal-Barajas, Jose Eduardo; Zinchenko, Yuriy; Khan, Rao [Department of Medical Physics, CancerCare Manitoba, Winnipeg, MB, CAN, Department of Physics and Astronomy, University of Calgary, Calgary, AB, CAN, Department of Mathematics and Statistics, University of Calgary, Calgary, AB, CAN, Department of Radiation Oncology, Washington University School of Medicine, St Louis, MO (United States)

    2016-08-15

    Purpose: Modulated Photon Radiotherapy (XMRT), which simultaneously optimizes photon beamlet energy (6 and 18 MV) and fluence, has recently shown dosimetric improvement in comparison to conventional IMRT. That said, the degree of smoothness of resulting fluence maps (FMs) has yet to be investigated and could impact the deliverability of XMRT. This study looks at investigating FM smoothness and imposing smoothing constraint in the fluence map optimization. Methods: Smoothing constraints were modeled in the XMRT algorithm with the sum of positive gradient (SPG) technique. XMRT solutions, with and without SPG constraints, were generated for a clinical prostate scan using standard dosimetric prescriptions, constraints, and a seven coplanar beam arrangement. The smoothness, with and without SPG constraints, was assessed by looking at the absolute and relative maximum SPG scores for each fluence map. Dose volume histograms were utilized when evaluating impact on the dose distribution. Results: Imposing SPG constraints reduced the absolute and relative maximum SPG values by factors of up to 5 and 2, respectively, when compared with their non-SPG constrained counterparts. This leads to a more seamless conversion of FMS to their respective MLC sequences. This improved smoothness resulted in an increase to organ at risk (OAR) dose, however the increase is not clinically significant. Conclusions: For a clinical prostate case, there was a noticeable improvement in the smoothness of the XMRT FMs when SPG constraints were applied with a minor increase in dose to OARs. This increase in OAR dose is not clinically meaningful.

  3. Differences in sensitivity of murine spermatogonia and somatic cells in vivo to sister-chromatid exchange induction by nitrosoureas.

    Science.gov (United States)

    Morales-Ramírez, P; Cruz-Vallejo, V; Rodríguez-Reyes, R

    2001-07-01

    Previously published data indicate that spermatogonia (SPG) are less sensitive to a sister-chromatid exchange (SCE) induction for different mutagens. In an earlier study, we have observed that bromodeoxyuridine (BrdU) substituted murine SPG are less sensitive to SCE induction by gamma ray in cells, than bone marrow (BM) and salivary gland (SG) cells in vivo. This was interpreted to mean that SPG are more efficient in DNA repair or are less prone to SCE induction. That the lower induction of SCE could be due to a reduced accessibility of mutagens to the SPG by virtue of a physiological barrier, was discarded by using gamma radiation. The aim of the present study was to establish whether or not there are differences in SCE induction by nitrosoureas among SPG, SG and BM cells with BrdU substituted or unsubstituted DNA. It was observed that SCE induction by methylnitrosourea (MNU) or by ethylnitrosourea (ENU) in SPG was, respectively, five and two times lower than in SG, and ten and three times lower than in BM. In SPG after BrdU incorporation, there was no increase in efficiency of SCE induction; in fact, there was even a slight decrease by exposure to MNU or ENU. BM and SG cells showed an increased efficiency in SCE induction after BrdU incorporation. This implies that SPG are also less sensitive to SCE induction by nitrosoureas, which cause a different kind of damage from previously assayed mutagens.

  4. The Sphenopalatine Ganglion: Anatomy, Pathophysiology, and Therapeutic Targeting in Headache.

    Science.gov (United States)

    Robbins, Matthew S; Robertson, Carrie E; Kaplan, Eugene; Ailani, Jessica; Charleston, Larry; Kuruvilla, Deena; Blumenfeld, Andrew; Berliner, Randall; Rosen, Noah L; Duarte, Robert; Vidwan, Jaskiran; Halker, Rashmi B; Gill, Nicole; Ashkenazi, Avi

    2016-02-01

    The sphenopalatine ganglion (SPG) has attracted the interest of practitioners treating head and face pain for over a century because of its anatomical connections and role in the trigemino-autonomic reflex. In this review, we discuss the anatomy of the SPG, as well as what is known about its role in the pathophysiology of headache disorders, including cluster headache and migraine. We then address various therapies that target the SPG, including intranasal medication delivery, new SPG blocking catheter devices, neurostimulation, chemical neurolysis, and ablation procedures. © 2015 American Headache Society.

  5. Delayed spinal extradural hematoma following thoracic spine surgery and resulting in paraplegia: a case report

    Directory of Open Access Journals (Sweden)

    Parthiban Chandra JKB

    2008-05-01

    Full Text Available Abstract Introduction Postoperative spinal extradural hematomas are rare. Most of the cases that have been reported occured within 3 days of surgery. Their occurrence in a delayed form, that is, more than 72 hours after surgery, is very rare. This case is being reported to enhance awareness of delayed postoperative spinal extradural hematomas. Case presentation We report a case of acute onset dorsal spinal extradural hematoma from a paraspinal muscular arterial bleed, producing paraplegia 72 hours following surgery for excision of a spinal cord tumor at T8 level. The triggering mechanism was an episode of violent twisting movement by the patient. Fresh blood in the postoperative drain tube provided suspicion of this complication. Emergency evacuation of the clot helped in regaining normal motor and sensory function. The need to avoid straining of the paraspinal muscles in the postoperative period is emphasized. Conclusion Most cases of postoperative spinal extradural hematomas occur as a result of venous bleeding. However, an arterial source of bleeding from paraspinal muscular branches causing extradural hematoma and subsequent neurological deficit is underreported. Undue straining of paraspinal muscles in the postoperative period after major spinal surgery should be avoided for at least a few days.

  6. Is patient confidentiality compromised with the electronic health record?: a position paper.

    Science.gov (United States)

    Wallace, Ilse M

    2015-02-01

    In order for electronic health records to fulfill their expected benefits, protection of privacy of patient information is key. Lack of trust in confidentiality can lead to reluctance in disclosing all relevant information, which could have grave consequences. This position paper contemplates whether patient confidentiality is compromised by electronic health records. The position that confidentiality is compromised was supported by the four bioethical principles and argued that despite laws and various safeguards to protect patients' confidentiality, numerous data breaches have occurred. The position that confidentiality is not compromised was supported by virtue ethics and a utilitarian viewpoint and argued that safeguards keep information confidential and the public feels relatively safe with the electronic health record. The article concludes with an ethically superior position that confidentiality is compromised with the electronic health record. Although organizational and governmental ways of enhancing the confidentiality of patient information within the electronic health record facilitate confidentiality, the ultimate responsibility of maintaining confidentiality rests with the individual end-users and their ethical code of conduct. The American Nurses Association Code of Ethics for nurses calls for nurses to be watchful with data security in electronic communications.

  7. Integer goal programming approach for finding a compromise ...

    African Journals Online (AJOL)

    In second model the cost and time spent on repairing the components are considered as two different objectives. Selective maintenance operation is used to select the repairable components and a multi-objective goal programming algorithm is proposed to obtain compromise selection of repairable components for the two ...

  8. Polyploidization of glia in neural development links tissue growth to blood-brain barrier integrity.

    Science.gov (United States)

    Unhavaithaya, Yingdee; Orr-Weaver, Terry L

    2012-01-01

    Proper development requires coordination in growth of the cell types composing an organ. Many plant and animal cells are polyploid, but how these polyploid tissues contribute to organ growth is not well understood. We found the Drosophila melanogaster subperineurial glia (SPG) to be polyploid, and ploidy is coordinated with brain mass. Inhibition of SPG polyploidy caused rupture of the septate junctions necessary for the blood-brain barrier. Thus, the increased SPG cell size resulting from polyploidization is required to maintain the SPG envelope surrounding the growing brain. Polyploidization likely is a conserved strategy to coordinate tissue growth during organogenesis, with potential vertebrate examples.

  9. Robust Public Key Cryptography — A New Cryptosystem Surviving Private Key Compromise

    Science.gov (United States)

    Shaik, Cheman

    A weakness of the present-day public key cryptosystems is that these cryptosystems do not survive private-key compromise attacks resulting from an internal breach of trust. In a competitive business environment, private key compromise is a common incident that voids the strength of public key cryptosystems such as RSA and ECC. Bribing corporate employees to disclose their secret keys and inadvertently disclosing secret information are among a plethora of practical attacks that occur at the implementation level. Once a breach of trust takes place and subsequently the private key is revealed, any public key cryptosystem fails to secure electronic data in Internet communications. The revealed key may be used by an attacker to decipher the intercepted data at an intermediary router. This weakness of public key cryptography calls for an additional security measure that enables encryptions to survive private key compromise attacks.

  10. On The Physico-Mechanics, Thermal and Microstructure Properties of Hybrid Composite Epoxy-Geopolymer for Geothermal Pipe Application

    Directory of Open Access Journals (Sweden)

    Firawati Ira

    2017-01-01

    Full Text Available The objective of this study is to determine the effect of epoxy resin on the physico-mechanics, thermal and microstructure properties of geopolymers hybrid composites for geothermal pipe application. Hybrid composite epoxy-geopolymers pipes were produced through alkali activation method of class-C fly ash and epoxy resin. The mass of epoxy-resin was varied relative to the mass of fly ash namely 0% (SPG01, 5% (SPG02, 10% (SPG03, 15% (SPG04, and 20% (SPG05. The resulting materials were stored in open air for 28 days before conducting any measurements. The densities of the product composites were measured before and after the samples immersed in boiling water for 3 hours. The mechanical strength of the resulting geothermal pipes was measured by using splitting tensile measurement. The thermal properties of the pipes were measured by means of thermal conductivity measurement, differential scanning calorimetry (DSC and fire resistance measurements. The chemical resistance was measured by immersing the samples into 1M H2SO4 solution for 4 days. The microstructure properties of the resulting materials were examined by using x-ray diffraction (XRD and Scanning Electron Microscopy-Energy Dispersive Spectroscopy (SEM-EDS. The results of this study showed that hybrid composite epoxy-geopolymers SPG02 and SPG03 are suitable to be applied as geothermal pipes.

  11. Identifying genetic relatives without compromising privacy.

    Science.gov (United States)

    He, Dan; Furlotte, Nicholas A; Hormozdiari, Farhad; Joo, Jong Wha J; Wadia, Akshay; Ostrovsky, Rafail; Sahai, Amit; Eskin, Eleazar

    2014-04-01

    The development of high-throughput genomic technologies has impacted many areas of genetic research. While many applications of these technologies focus on the discovery of genes involved in disease from population samples, applications of genomic technologies to an individual's genome or personal genomics have recently gained much interest. One such application is the identification of relatives from genetic data. In this application, genetic information from a set of individuals is collected in a database, and each pair of individuals is compared in order to identify genetic relatives. An inherent issue that arises in the identification of relatives is privacy. In this article, we propose a method for identifying genetic relatives without compromising privacy by taking advantage of novel cryptographic techniques customized for secure and private comparison of genetic information. We demonstrate the utility of these techniques by allowing a pair of individuals to discover whether or not they are related without compromising their genetic information or revealing it to a third party. The idea is that individuals only share enough special-purpose cryptographically protected information with each other to identify whether or not they are relatives, but not enough to expose any information about their genomes. We show in HapMap and 1000 Genomes data that our method can recover first- and second-order genetic relationships and, through simulations, show that our method can identify relationships as distant as third cousins while preserving privacy.

  12. Loss of Association of REEP2 with Membranes Leads to Hereditary Spastic Paraplegia

    Science.gov (United States)

    Esteves, Typhaine; Durr, Alexandra; Mundwiller, Emeline; Loureiro, José L.; Boutry, Maxime; Gonzalez, Michael A.; Gauthier, Julie; El-Hachimi, Khalid H.; Depienne, Christel; Muriel, Marie-Paule; Acosta Lebrigio, Rafael F.; Gaussen, Marion; Noreau, Anne; Speziani, Fiorella; Dionne-Laporte, Alexandre; Deleuze, Jean-François; Dion, Patrick; Coutinho, Paula; Rouleau, Guy A.; Zuchner, Stephan; Brice, Alexis; Stevanin, Giovanni; Darios, Frédéric

    2014-01-01

    Hereditary spastic paraplegias (HSPs) are clinically and genetically heterogeneous neurological conditions. Their main pathogenic mechanisms are thought to involve alterations in endomembrane trafficking, mitochondrial function, and lipid metabolism. With a combination of whole-genome mapping and exome sequencing, we identified three mutations in REEP2 in two families with HSP: a missense variant (c.107T>A [p.Val36Glu]) that segregated in the heterozygous state in a family with autosomal-dominant inheritance and a missense change (c.215T>A [p.Phe72Tyr]) that segregated in trans with a splice site mutation (c.105+3G>T) in a family with autosomal-recessive transmission. REEP2 belongs to a family of proteins that shape the endoplasmic reticulum, an organelle that was altered in fibroblasts from an affected subject. In vitro, the p.Val36Glu variant in the autosomal-dominant family had a dominant-negative effect; it inhibited the normal binding of wild-type REEP2 to membranes. The missense substitution p.Phe72Tyr, in the recessive family, decreased the affinity of the mutant protein for membranes that, together with the splice site mutation, is expected to cause complete loss of REEP2 function. Our findings illustrate how dominant and recessive inheritance can be explained by the effects and nature of mutations in the same gene. They have also important implications for genetic diagnosis and counseling in clinical practice because of the association of various modes of inheritance to this new clinico-genetic entity. PMID:24388663

  13. Salivary changes in medically compromised patients: A clinical and biochemical study

    Directory of Open Access Journals (Sweden)

    Yehoshuva R Tummuru

    2017-01-01

    Full Text Available Introduction: Medically compromised patients require special attention when dental procedures are performed on them. These individuals may require modified or slightly altered techniques. Aims and Objectives: The present study was taken up with two main objectives. The first one being examining and recording various oral manifestations in medically compromised patients, and the second objective was to collect samples of saliva from such patients and to analyze and establish any salivary changes in such medically compromised patients. Materials and Methods: A total of 100 patients were selected for the study. These patients were divided into four groups of 25 patients each: diabetes mellitus group, chronic renal failure group, liver cirrhosis group and control group. All the selected patients were subjected to a detailed general and intra oral examinations and the relevant data was recorded on a specially designed proforma; salivary analysis was done to know the flow rate, pH, total salivary proteins, sodium, potassium, and LDH levels. Results: From the findings, it can be inferred that salivary changes namely changes in salivary pH, salivary flow rates, salivary sodium, salivary potassium, salivary total proteins, and salivary lactate dehydrogenase are significant in medically compromised patients namely uncontrolled diabetes mellitus, chronic renal failure, cirrhosis of liver compared to the control group. Conclusion: pH of saliva was elevated in chronic renal failure patients. Salivary flow rates and sodium were decreased in diabetes mellitus, chronic renal failure, and cirrhosis of liver patients. There was a significant elevation of salivary potassium in chronic renal failure patients. LDH elevation was significant in uncontrolled diabetes mellitus.

  14. Unusual Presentation of a Primary Ewing’s Sarcoma of the Spine with Paraplegia: A Case Report

    Science.gov (United States)

    Sundarapandian, Rajkumar Jayachandran; Surulivel, Vignesh Jayabalan

    2015-01-01

    Ewing’s sarcoma is a primary malignancy of the bone affecting individuals in the second decade of life. Primary sarcomas of the spine are rare and the occurrence of Primary Ewing’s sarcoma in the spine is very rare. Ewing’s sarcoma occurring in the spine is divided into two types, Ewing’s sarcoma of sacral spine which are very aggressive with poor prognosis and Ewing’s sarcoma of the non sacral spine which is an extremely rare occurrence. Patient may present with neurological deficit when the tumour extends into the spinal canal causing spinal cord compression. Magnetic resonance imaging (MRI) is very sensitive in diagnosing the tumour and defining the extent of the tumour. Here we report an 18-year-old boy who presented with back pain and complete paraplegia of two months duration. The MRI gave a differential diagnosis of infective pathology due to the fluid collection in the paraspinal region, followed by primary malignancy as the second diagnosis. Patient underwent posterior spinal decompression and stabilization, and intaoperatively there was significant collection of pus whose culture showed no growth. The histopathology and immunohistochemistry studies confirmed the diagnosis of Ewing’s sarcoma and patient was started on combination chemotherapy and radiotherapy. PMID:25954672

  15. Self-esteem, personality, and gender self-perception

    Directory of Open Access Journals (Sweden)

    2015-09-01

    Results: No significant difference was found between males in the high and low market value conditions either on explicit self-perceived gender (t(26 = -.418, p = .679 or on visual SPG (t(26 = .390, p = .700. Nor did females in the market value conditions differ significantly (explicit SPG: t(64 = -.365, p = .716; visual SPG: t(64 = .684, p = .497. Relationships between SPG, self-esteem, and personality measures were reported. Conclusions: Self-esteem and particular personality traits are possible mediators in shifting an observer’s self-perceived gender. However, no definitive demonstration of these relationships was found with this study. Suggestions for future work were explored in light of these results.

  16. A combination of additives can synergically decrease acrylamide content in gingerbread without compromising sensory quality.

    Science.gov (United States)

    Komprda, Tomáš; Pridal, Antonin; Mikulíková, Renata; Svoboda, Zdeněk; Cwiková, Olga; Nedomová, Šárka; Sýkora, Vladimír

    2017-02-01

    The present study tested whether replacement of the leavening agent ammonium carbonate by sodium hydrogen carbonate in combination with calcium cation and acidifying agent will synergically decrease acrylamide (AA) content in gingerbread. The type of leavening agent and the presence of Ca 2+ and citric acid accounted for 33.6%, 13.2% and 53.2% of the explained variability of the AA content, respectively (P sensory analysis did not indicate any significant deterioration (P > 0.05) in the organoleptic quality of gingerbread produced using calcium cation and citric acid. The present study demonstrates that the combination of additives NaHCO 3 /Ca 2+ /citric acid synergically decreases AA content in gingerbread without compromising the sensory quality. © 2016 Society of Chemical Industry. © 2016 Society of Chemical Industry.

  17. Cyber indicators of compromise: a domain ontology for security information and event management

    Science.gov (United States)

    2017-03-01

    heuristics, mapping, and detection. CybOX is aimed at supporting a broad range of important cyber security domains to include [31]: • Digital...REPORT TYPE AND DATES COVERED Master’s thesis 4. TITLE AND SUBTITLE CYBER INDICATORS OF COMPROMISE: A DOMAIN ONTOLOGY FOR SECURITY INFORMATION AND...Distribution is unlimited. CYBER INDICATORS OF COMPROMISE: A DOMAIN ONTOLOGY FOR SECURITY INFORMATION AND EVENT MANAGEMENT Marsha D. Rowell

  18. 26 CFR 301.6331-3 - Restrictions on levy while offers to compromise are pending.

    Science.gov (United States)

    2010-04-01

    ... are pending. 301.6331-3 Section 301.6331-3 Internal Revenue INTERNAL REVENUE SERVICE, DEPARTMENT OF... for Collection of Taxes § 301.6331-3 Restrictions on levy while offers to compromise are pending. Cross-reference. For provisions relating to the making of levies while an offer to compromise is pending...

  19. Sexualidade e paraplegia: o dito, o explícito e o oculto Sexualydad y paraplejía: lo dicho, lo explícito y lo oculto Sexuality and paraplegics: the saying, the explicit and the occult

    Directory of Open Access Journals (Sweden)

    Inacia Sátiro Xavier de França

    2005-09-01

    Full Text Available INTRODUÇÃO: A intenção de contribuir para o desenvolvimento de ações educativas que desmistifiquem mitos e tabus relacionados a sexualidade da pessoa portadora de paraplegia e melhorar a qualidade de vida desse tipo de cliente motivou a seleção do objeto "sexualidade da mulher portadora de paraplegia". OBJETIVO: Compreender a construção de sentidos das mulheres portadoras de paraplegia acerca da sexualidade e relatar as barreiras enfrentadas por estas mulheres para vivenciar sua sexualidade. MÉTODO: Estudo descritivo onde os sujeitos foram selecionados por acessibilidade, conforme critérios pré-estabelecidos e, utilizando-se a entrevista semi-estruturada, a coleta de dados e a análise de discurso emergiram categorias. RESULTADOS: As categorias sinalizaram o processo de negativação da auto-imagem, do auto-conceito e de tendência a auto-discriminação, além de culpabilização, quando da sensação de orgasmo. Também emergiram categorias sinalizadoras da crença no casamento monogâmico como realização pessoal, e que o orgasmo é uma questão de conhecimento do próprio corpo e do corpo do parceiro. CONCLUSÕES: Conclui-se que o mito da beleza e o mito adâmico normatizam a sexualidade dos sujeitos. Mas nem todas as mulheres estudadas seguem, à risca, as regras da cultura, de modo que um grupo de mulheres portadoras de paraplegia vivencia a sexualidade de forma diferente das demais.INTRODUCCIÓN: La intención de contribuir para el desarrollo de acciones educativas que desmistifiquen mitos y tabus relacionados a lasexualidad de la persona portadora de paraplejía y mejorar la calidad de vida de este tipo de cliente ha motivado la selección del objeto "la sexualidad de la mujer portadora de paraplegia". OBJETIVO: Compreender la formación de los sentimientos de las mujeres portadoras de paraplejía sobre la sexualidad y relatar las barreras enfrentadas por dichas mujeres para poder vivenciar su sexualidad. MÉTODO: Estudio

  20. Sustainable Development Compromise[d] in the Planning of Metro Vancouver’s Agricultural Lands—the Jackson Farm Case

    Directory of Open Access Journals (Sweden)

    Meg Holden

    2013-11-01

    Full Text Available This research provides analysis of the case of the Jackson Farm development application, embedded within the particular dynamics of the municipal, regional, and provincial sustainability land use policy culture of the Metro Vancouver region, in Canada. Within a culture of appreciation of the increasing need for sustainability in land use policy, including the protection of agricultural lands at the provincial level through the Agricultural Land Reserve (ALR, to urban intensification and protection of the green zone at the regional scale, lies a political conflict that comes into focus in individual land use decisions, within municipalities struggling for autonomy. This case is neither driven strictly by “the politics of the highest bidder” nor by policy failure; the case of the Jackson Farm is instead a case of the challenges of implementing inter-governmental coordination and collaborative governance in a context of both significant sustainability policy and urban growth. The process can be seen to follow an ecological modernization agenda, seeking “win–win” alternatives rather than recognizing that typical compromises, over time, may tip the direction of development away from sustainability policy goals. Understanding the twists, turns, and eventual compromise reached in the case of the Jackson Farm brings to light the implications of the shift in the regional planning culture which may necessitate a less flexible, more structured prioritization of competing goals within plans and policies in order to meet sustainability goals. We highlight this, and present an alternative implementation process within the existing policy regime with potential to aid the specific goal of agricultural land protection.

  1. Application of system-process-goal approach for description of TRIGA RC-1 system

    International Nuclear Information System (INIS)

    Gadomski, Adam M.

    1986-01-01

    The new methodology of the goal oriented description of an artificial system is presented. In the SPG approach (System-Process-Goal) it is assumed that the knowledge necessary for achieving the goal is available but it is not ordered or ordered for other purposes. The aim of SPG is to give the description of the analyzed system in form of network by decomposition of goal-system relationships using uniform and mathematical formalism. The SPG approach is useful to build a reactor operator aid system. This paper presents the conception of the application of the SPG approach to the decomposition of TRIGA RC-1 dynamics and for designing of TRIGA diagnostic algorithms. (author)

  2. Sterile diets for the immuno-compromised: Is there a need?

    Science.gov (United States)

    Butterweck, Joseph S.

    1995-02-01

    There is a general misunderstanding in the radiation processing industry about the use of sterile diets in the medical profession. Sterile diets are used on a limited basis in hospitals that specialize in cancer treatment and organ transplants. These patients are severely immuno-compromised. There are many other patients that are immuno-compromised that do not require sterile diets. These patients may require a diet that is pathogen-free and are aslo "low-microbial diets". Nosocomial infections have become a major issue in US hospitals. The "infection control committee" is the focus group responsible to assure nosocomial infections incidence are below the hospital goals. Application of ionizing radiation to sterilize diets has not been chosen because the product is not available at a reasonable total cost. This paper will discuss the hospitals views.

  3. Genotype-phenotype correlations and expansion of the molecular spectrum of AP4M1-related hereditary spastic paraplegia

    Directory of Open Access Journals (Sweden)

    Conceição Bettencourt

    2017-11-01

    Full Text Available Abstract Background Autosomal recessive hereditary spastic paraplegia (HSP due to AP4M1 mutations is a very rare neurodevelopmental disorder reported for only a few patients. Methods We investigated a Greek HSP family using whole exome sequencing (WES. Results A novel AP4M1A frameshift insertion, and a very rare missense variant were identified in all three affected siblings in the compound heterozygous state (p.V174fs and p.C319R; the unaffected parents were carriers of only one variant. Patients were affected with a combination of: (a febrile seizures with onset in the first year of life (followed by epileptic non-febrile seizures; (b distinctive facial appearance (e.g., coarse features, bulbous nose and hypomimia; (c developmental delay and intellectual disability; (d early-onset spastic weakness of the lower limbs; and (e cerebellar hypoplasia/atrophy on brain MRI. Conclusions We review genotype-phenotype correlations and discuss clinical overlaps between different AP4-related diseases. The AP4M1 belongs to a complex that mediates vesicle trafficking of glutamate receptors, being likely involved in brain development and neurotransmission.

  4. Gamma processing of Arabic bread for immune system-compromised cancer patients

    International Nuclear Information System (INIS)

    Grecz, N.; Brannon, R.; Jaw, R.; Al-Harithy, R.; Hahn, E.W.

    1985-01-01

    Arabic bread prepared from local Saudi flour contained a total of up to 105 4 organisms per g. Most of these were bacterial spores that survived the baking process (1.3 x 10 2 to 3.5 x 10 3 ) and a small number of yeasts and molds (10 to 40 cells per g). The organisms in Arabic bread appear to be harmless to healthy individuals. However, for immune system-compromised cancer patients and bone marrow transplant recipients, it is prudent to irradiate the bread to reduce microbial contamination. The decimal reduction doses (10% survival) for the most radiation-resistant organisms (spore formers) in bread were 0.11 to 0.15 Mrad. Accordingly, 0.6 Mrad was sufficient to reduce the number of spores in Arabic bread by a factor of 10,000, i.e., to <1/g. This treatment constitutes radiation pasteurization (radicidation), and to this extent, provides a margin of microbiological safety. Sensory evaluation by the nine-point hedonic scale showed no detectable loss of organoleptic quality of bread up to 0.6 Mrad, while irradiation to 2.5 Mrad induced unacceptable organoleptic changes

  5. Neuronavigated percutaneous approach to the sphenopalatine ganglion.

    Science.gov (United States)

    Benedetto, Nicola; Perrini, Paolo

    2017-02-01

    The sphenopalatine ganglion (SPG) has been assumed to be involved in the genesis of several types of facial pain, including Sluder's neuralgia, trigeminal neuralgia, persistent idiopathic facial pain, cluster headache, and atypical facial pain. The gold standard treatments for SPG-related pain are percutaneous procedures performed with the aid of fluoroscopy or CT. In this technical note the authors present, for the first time, an SPG approach using the aid of a neuronavigator.

  6. Stimulation of the sphenopalatine ganglion induces reperfusion and blood-brain barrier protection in the photothrombotic stroke model.

    Directory of Open Access Journals (Sweden)

    Haviv Levi

    Full Text Available The treatment of stroke remains a challenge. Animal studies showing that electrical stimulation of the sphenopalatine ganglion (SPG exerts beneficial effects in the treatment of stroke have led to the initiation of clinical studies. However, the detailed effects of SPG stimulation on the injured brain are not known.The effect of acute SPG stimulation was studied by direct vascular imaging, fluorescent angiography and laser Doppler flowmetry in the sensory motor cortex of the anaesthetized rat. Focal cerebral ischemia was induced by the rose bengal (RB photothrombosis method. In chronic experiments, SPG stimulation, starting 15 min or 24 h after photothrombosis, was given for 3 h per day on four consecutive days. Structural damage was assessed using histological and immunohistochemical methods. Cortical functions were assessed by quantitative analysis of epidural electro-corticographic (ECoG activity continuously recorded in behaving animals.Stimulation induced intensity- and duration-dependent vasodilation and increased cerebral blood flow in both healthy and photothrombotic brains. In SPG-stimulated rats both blood brain-barrier (BBB opening, pathological brain activity and lesion volume were attenuated compared to untreated stroke animals, with no apparent difference in the glial response surrounding the necrotic lesion.SPG-stimulation in rats induces vasodilation of cortical arterioles, partial reperfusion of the ischemic lesion, and normalization of brain functions with reduced BBB dysfunction and stroke volume. These findings support the potential therapeutic effect of SPG stimulation in focal cerebral ischemia even when applied 24 h after stroke onset and thus may extend the therapeutic window of currently administered stroke medications.

  7. Modulation of pulmonary fibrosis by IL-13Rα2.

    Science.gov (United States)

    Lumsden, Robert V; Worrell, Julie C; Boylan, Denise; Walsh, Sinead M; Cramton, Jennifer; Counihan, Ian; O'Beirne, Sarah; Medina, Maria Fe; Gauldie, Jack; Fabre, Aurelie; Donnelly, Seamas C; Kane, Rosemary; Keane, Michael P

    2015-04-01

    Pulmonary fibrosis is a progressive and fatal disease that involves the remodeling of the distal airspace and the lung parenchyma, which results in compromised gas exchange. The median survival time once diagnosed is less than three years. Interleukin (IL)-13 has been shown to play a role in a number of inflammatory and fibrotic diseases. IL-13 modulates its effector functions via a complex receptor system that includes the IL-4 receptor (R) α, IL-13Rα1, and the IL-13Rα2. IL-13Rα1 binds IL-13 with low affinity, yet, when it forms a complex with IL-4α, it binds with much higher affinity, inducing the effector functions of IL-13. IL-13Rα2 binds IL-13 with high affinity but has a short cytoplasmic tail and has been shown to act as a nonsignaling decoy receptor. Transfection of fibroblasts and epithelial cells with IL-13Rα2 inhibited the IL-13 induction of soluble collagen, TGF-β, and CCL17. Adenoviral overexpression of IL-13Rα2 in the lung reduced bleomycin-induced fibrosis. Our work shows that overexpression of IL-13Rα2 inhibits the IL-13 induction of fibrotic markers in vitro and inhibits bleomycin-induced pulmonary fibrosis. In summary our study highlights the antifibrotic nature of IL-13Ra2. Copyright © 2015 the American Physiological Society.

  8. A Compromise Programming Model for Highway Maintenance Resources Allocation Problem

    Directory of Open Access Journals (Sweden)

    Hui Xiong

    2012-01-01

    Full Text Available This paper formulates a bilevel compromise programming model for allocating resources between pavement and bridge deck maintenances. The first level of the model aims to solve the resource allocation problems for pavement management and bridge deck maintenance, without considering resource sharing between them. At the second level, the model uses the results from the first step as an input and generates the final solution to the resource-sharing problem. To solve the model, the paper applies genetic algorithms to search for the optimal solution. We use a combination of two digits to represent different maintenance types. Results of numerical examples show that the conditions of both pavements and bridge decks are improved significantly by applying compromise programming, rather than conventional methods. Resources are also utilized more efficiently when the proposed method is applied.

  9. The compromised gut in the neonate : Diagnostic and clinical aspects

    NARCIS (Netherlands)

    Schurink, Maarten

    2016-01-01

    Necrotizing enterocolitis (NEC) is a devastating gastrointestinal disease predominantly occurring in preterm infants, causing significant patient morbidity and mortality. It is characterized by loss of bowel wall integrity due to ischemia (lack of oxygen due to a compromised blood flow) and

  10. Whatever It Takes: Health Compromising Behaviors in Female Athletes

    Science.gov (United States)

    Waldron, Jennifer J.; Krane, Vikki

    2005-01-01

    The power and performance model of sport stresses a sport ethic of doing "whatever it takes" to win (Coakley, 2004). Uncritical acceptance of this model may lead to various health-compromising behaviors. Employing achievement goal theory, we examine why female athletes may adopt the power and performance approach. An ego motivational climate and a…

  11. Compromised Cerebral Blood Flow(CBF) in Congestive Heart Failure (CHB): non-invasive quantification with {sup 99m}Tc-ECD radionuclide angiography

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jae Seung; Kim, Jae Joong; Lim, Ki Chun; Lee, Hee Kyung; Moon, Dae Hyuk [Ulsan University College of Medicine, Seoul (Korea, Republic of)

    2002-07-01

    Recent reports revealed that cerebral metabolism in CHF was abnormally deranged and proposed as a potential marker of disease severity. Since deranged cerebral metabolism in CHF may result from compromised cerebral perfusion, quantification of CHF may be useful for accurate risk stratification of CHF. Therefore, we investigated whether CHF in patients with CHF is compromised and correlated with clinical parameters. Fifteen patients (M/F:11/5, 45{+-}9yr) with CHF (LVEF<40%) and 7 healthy controls (M/F:5/2, 41{+-}8yr) were prospectively studied. All patients underwent radionuclide angiography including cerebral hemispheres and aortic arch using {sup 99m}Tc-ECD. Global CBF was measured non-invasively by the application of Patlak graphical plot analysis. All patients were also evaluated using a standardized protocol that included echocardiography and clinical evaluation. Global CBF (40.3{+-}5.2 ml/min/100g) of the patients with CHF were significantly lower than those (49.7{+-}2.4 ml/min/100g) of controls (p<0.01). Global CBF were correlated with NYHA functional class (r=-0.617, p=0.43), but not correlated with other clinical parameters such as age (r=-0.463, p=0.082), duration (r=0.237, p>0.1), systolic BP (r=-0.063, p>0.5), LVEF (r=-0.13, p>0.1), LV dimension(r=0.139, p>0.5), and PV pressure gradients (r=0.072, p>0.5). Cerebral perfusion of the patients with CHF was compromised and not correlated with cardiopulmonary hemodynamic parameters.

  12. 48 CFR 239.7102-2 - Compromising emanations-TEMPEST or other standard.

    Science.gov (United States)

    2010-10-01

    ... INFORMATION TECHNOLOGY Security and Privacy for Computer Systems 239.7102-2 Compromising emanations—TEMPEST or... requiring activity is responsible for providing to the contracting officer— (a) The required protections, i...

  13. Sôbre as paraplegias cifoscolióticas: tratamento neurocirúrgico

    Directory of Open Access Journals (Sweden)

    Roberto Melaragno Filho

    1951-03-01

    Full Text Available Ao que julgam os autores, o caso que ora registram representam o primeiro caso brasileiro de cifoscoliose congênita tratada neurocirurgicamente. Estudaram inicialmente as diversas doutrinas explicativas do sofrimento medular nessa enfermidade. Insistiram no papel da compressão da medula entre as duas faces da dura-máter, anterior e posterior: a primeira aplicada contra a convexidade óssea e a segunda, fortemente distendida pelas raízes nervosas, contra a face posterior da medula. A maior incidência de fenômenos medulares entre os 15 e 20 anos de vida, época do maior crescimento do indivíduo, comprova o papel desempenhado, em sua patogenia, pelo estiramento longitudinal da dura-máter, sòlidamente fixada no orifício magno e no sacro. Finalmente, a torção da coluna e a possibilidade de compressão de vasos sangüíneos, com conseqüentes mielomalácias, deve ser considerada diante de cada caso. Nesses fundamentos baseia-se a indicação da abertura da dura-máter e sua não suturação após a laminectomia. Nos casos da literatura em que a intervenção cirúrgica se limitou à abertura do canal raquidiano, os resultados foram pouco brilhantes. Registraram os autores a observação de um jovem de 17 anos, que, ano e meio antes, ao suportar a coluna um esforço exagerado, começou a apresentar dores lombares, as quais motivaram a indicação de um colete de gèsso. Entretanto, pouco a pouco, foram-se desenvolvendo sinais de déficit motor e sensitivo nos membros inferiores. Ao exame, foi observada uma paraplegia crural absoluta, sendo possíveis, apenas, pequenos movimentos de flexão e extensão dos grandes artelhos. Reflexos profundos exaltados, trepidação e clono nos membros inferiores; sinal de Babinski bilateral; hipoestesia táctil, térmica e dolorosa até T8; anestesia profunda nos membros inferiores. Cifose dorsal alta pronunciada, com escoliose dorsal média dextro-convexa e ligeira escoliose dorsolombar sinistro-convexa. O

  14. A libertarian perspective on the stem cell debate: compromising the uncompromisible.

    Science.gov (United States)

    Block, Walter

    2010-08-01

    The present paper attempts to forge a compromise between those who maintain that stem cell research is out-and-out murder of young helpless human beings and those who favor this practice. The compromise is predicated upon the libertarian theory of private property rights. Starting out with the premise that not only the fetus but even the fertilized egg is a human being, with all rights thereto, it offers a competition between those who fertilize eggs for research and those who wish to adopt them. If and only if the former win this competition will they be allowed to use these very young human beings for the purposes they have constructed them. This is justified on grounds of avoiding child abuse.

  15. Anthropogenic noise compromises antipredator behaviour in European eels.

    Science.gov (United States)

    Simpson, Stephen D; Purser, Julia; Radford, Andrew N

    2015-02-01

    Increases in noise-generating human activities since the Industrial Revolution have changed the acoustic landscape of many terrestrial and aquatic ecosystems. Anthropogenic noise is now recognized as a major pollutant of international concern, and recent studies have demonstrated impacts on, for instance, hearing thresholds, communication, movement and foraging in a range of species. However, consequences for survival and reproductive success are difficult to ascertain. Using a series of laboratory-based experiments and an open-water test with the same methodology, we show that acoustic disturbance can compromise antipredator behaviour--which directly affects survival likelihood--and explore potential underlying mechanisms. Juvenile European eels (Anguilla anguilla) exposed to additional noise (playback of recordings of ships passing through harbours), rather than control conditions (playback of recordings from the same harbours without ships), performed less well in two simulated predation paradigms. Eels were 50% less likely and 25% slower to startle to an 'ambush predator' and were caught more than twice as quickly by a 'pursuit predator'. Furthermore, eels experiencing additional noise had diminished spatial performance and elevated ventilation and metabolic rates (indicators of stress) compared with control individuals. Our results suggest that acoustic disturbance could have important physiological and behavioural impacts on animals, compromising life-or-death responses. © 2014 John Wiley & Sons Ltd.

  16. Creating clones, kids & chimera: liberal democratic compromise at the crossroads.

    Science.gov (United States)

    Adams, Nathan A

    2004-01-01

    The objective of this article is to find middle ground between the supporters and opponents of biotechnology by perpetuating the existing legal compromise pertaining to the complete range of health and welfare doctrines relevant to the biotechnological industry. The author aspires neither to add to nor detract from this liberal democratic consensus, but to preserve its constitutive balance between positivism and natural law and over-regulation and under-regulation in the hopes of stabilizing new political fault lines developing around the few biotechnological innovations already grabbing headlines. The most feasible solution is to extend the existing liberal democratic compromise with respect to equal protection, reproductive rights, the First Amendment, human subject experimentation, patent law, and parental rights. This includes banning or monopolizing certain biotechnologies and extending substantive special respect to the ex vivo living human embryo. Biotechnology must not be left to regulate itself.

  17. No-compromise reptation quantum Monte Carlo

    International Nuclear Information System (INIS)

    Yuen, W K; Farrar, Thomas J; Rothstein, Stuart M

    2007-01-01

    Since its publication, the reptation quantum Monte Carlo algorithm of Baroni and Moroni (1999 Phys. Rev. Lett. 82 4745) has been applied to several important problems in physics, but its mathematical foundations are not well understood. We show that their algorithm is not of typical Metropolis-Hastings type, and we specify conditions required for the generated Markov chain to be stationary and to converge to the intended distribution. The time-step bias may add up, and in many applications it is only the middle of a reptile that is the most important. Therefore, we propose an alternative, 'no-compromise reptation quantum Monte Carlo' to stabilize the middle of the reptile. (fast track communication)

  18. Pulmonary, neurological and hepatic compromise in paragonimiasis: case report

    International Nuclear Information System (INIS)

    Uriza Carrasco, Alfonso Jose; Cuervo Valencia, Catalina; Valencia M, Andres Mauricio; Echeverri Toro, Lina Maria

    2011-01-01

    Paragonimiasis is a parasitic infectious disease, frequently with pulmonary abnormalities, although erratic parasite migration can compromise other organs. this disease is endemic in areas of southeast Asia, but it has been described in other tropical and subtropical regions. In this article, we report a case of indigenous patient with pulmonary abnormalities by Paragonimiasis, neurologic and hepatic abnormalities too.

  19. Would Enforcing Competition Law Compromise Industry Policy Objectives?

    OpenAIRE

    Evenett, Simon J.

    2005-01-01

    One recurring concern in the debate over the efficacy of enacting competition laws in developing countries is that its enforcement may compromise important industrial policy goals. This concern has been raised in regional fora and in multilateral organizations such as the World Trade Organization, where officials have considered the pros and cons of including competition provisions in international trade agreements. However, the concern is broader and often national debates over the merits of...

  20. Principles of treatment planning for compromised first permanent molars in mixed dentition period: A review article

    Directory of Open Access Journals (Sweden)

    Behrad Tanbakuchi

    2017-02-01

    Full Text Available Background and Aims: The first permanent molar is susceptible to caries, endodontic complications and developmental anomalies. Compromised teeth with poor prognosis could cause the clinician to face dilemmas. The aim of this review article was to assess recent guidelines on the treatment planning of compromised first permanent molars in mixed dentition period. Materials and Methods: The design of the study was based on review of literature by searching the MEDLINE, Web of science and Google scholar. Key words (permanent first molar, extraction, treatment plan were used. This search was limited to the English articles published after the year 2000. Conclusion: A number of factors influenced decision-making process regarding compromised first permanent molars including the restorative status of the tooth, dental age of the patient, degree of crowding and occlusal relationships were assessed. The ideal time for removal of these teeth was 8-9 years of age. However, the current evidence for managing compromised first permanent molars demands clinical trials.

  1. Emergency Closed Reduction of a C4/5 Fracture Dislocation with Complete Paraplegia Resulting in Profound Neurologic Recovery

    Directory of Open Access Journals (Sweden)

    Christian W. Müller

    2013-01-01

    Full Text Available Introduction. Cervical spinal cord injuries due to traumatic fractures are associated with persistent neurological deficits. Although clinical evidence is weak, early decompression, defined as <24–72 h, has been frequently proposed. Animal studies show better outcomes after early decompression within one hour or less, which can hardly ever be achieved in clinical practice. Case Presentation. A 37-year-old patient was hospitalized after being hit by a shying horse. After diagnosis of C4/5 fracture dislocation and complete paraplegia, she was intubated and sedated with deep relaxation. Emergency reduction was performed at approximately 120 minutes after trauma. Subsequently, a standard anterior decompression, discectomy, and fusion were carried out. She was then transferred to a specialized rehabilitation hospital. Her neurologic function improved from AIS grade A on admission to grade B postoperatively and grade D after four months of rehabilitation. One year after the accident, she was ambulatory without walking aids and restarted horse riding. Discussion and Conclusion. Rarely in clinical practice, decompression of the spine canal can be performed as early as in this case. This case highlights the potential benefit of utmost early reduction in cervical fracture dislocations with compression of the spinal cord.

  2. A fair compromise to break the climate impasse. A major economies forum approach to emissions reductions budgeting

    Energy Technology Data Exchange (ETDEWEB)

    Grasso, Marco [Univ. of Milan-Bicocca (Italy). International Environmental Policy; J. Roberts, Timmons [Brown Univ., Providence, RI (United States). Environmental Studies and Sociology; The Brookings Institution, Washington, DC (United States)

    2013-04-15

    Key messages of the study are: Given the stalemate in U.N. climate negotiations, the best arena to strike a workable deal is among the members the Major Economies Forum on Energy and Climate (MEF); The 13 MEF members—including the EU-27 (but not double-counting the four EU countries that are also individual members of the MEF)—account for 81.3 percent of all global emissions; This proposal devises a fair compromise to break the impasse to develop a science-based approach for fairly sharing the carbon budget in order to have a 75 percent chance of avoiding dangerous climate change; To increase the likelihood of a future climate agreement, carbon accounting must shift from production-based inventories to consumption-based ones; The shares of a carbon budget to stay below 2 deg C through 2050 are calculated by cumulative emissions since 1990, i.e. according to a short-horizon polluter pays principle, and national capability (income), and allocated to MEF members through emission rights. This proposed fair compromise addresses key concerns of major emitters; According to this accounting, no countries have negative carbon budgets, there is substantial time for greening major developing economies, and some developed countries need to institute very rapid reductions in emissions; and, To provide a 'green ladder' to developing countries and to ensure a fair global deal, it will be crucial to agree how to extend sufficient and predictable financial support and the rapid transfer of technology.

  3. Secretion of salivary statherin is compromised in uncontrolled diabetic patients

    Directory of Open Access Journals (Sweden)

    Masahiro Izumi

    2015-06-01

    Conclusions and general significance: The results show that synthesis and secretion of statherin is reduced in diabetics and this reduction is salivary gland specific. As compromised salivary statherin secretion leads to increased oral health risk, this study indicates that routine oral health assessment of these patients is warranted.

  4. Influence of political opposition and compromise on conservation outcomes in the Tongass National Forest, Alaska.

    Science.gov (United States)

    Beier, Colin M

    2008-12-01

    To understand how a highly contentious policy process influenced a major conservation effort, I examined the origins, compromises, and outcomes of the Alaska National Interest Lands Conservation Act of 1980 (ANILCA) for the Tongass National Forest. Tongass wilderness designation was among the most controversial issues in the ANILCA debate, and it faced strong opposition from influential lawmakers, land managers, and Alaska residents. To investigate the influence of this opposition on Tongass conservation outcomes, I conducted a gap analysis of Tongass reserves and a policy analysis of the ANILCA debate and traced the influence of specific interests through the amendments, negotiations, and resulting compromises needed to enact ANILCA. Overall, I found that Tongass reserves comprise a broadly representative cross-section of ecosystems and species habitats in southeastern Alaska. Redrawn reserve boundaries, industry subsidies, and special access regulations reflected compromises to minimize the impact of wilderness conservation on mining, timber, and local stakeholder interests, respectively. Fragmentation of the Admiralty Island National Monument-the most ecologically valuable and politically controversial reserve-resulted from compromises with Alaskan Native (indigenous peoples of Alaska) corporations and timber interests. Despite language to accommodate "reasonable access" to wilderness reserves, ongoing access limitations highlight the concerns of Alaska residents that opposed ANILCA several decades ago. More broadly, the Tongass case suggests that early and ambitious conservation action may offset strong political opposition; compromises needed to establish key reserves often exacerbate development impacts in unprotected areas; and efforts to minimize social conflicts are needed to safeguard the long-term viability of conservation measures.

  5. Quality of life and self-esteem of persons with paraplegia living in São Paulo, Brazil.

    Science.gov (United States)

    Blanes, Leila; Carmagnani, Maria Isabel S; Ferreira, Lydia M

    2009-02-01

    To evaluate the quality of life (QoL) and self-esteem of paraplegic persons. The sample consisted of 60 outpatients with traumatic paraplegia living in São Paulo, Brazil, from whom clinical and demographic data were obtained. QoL was assessed by the 36-item Short-Form (SF-36) health survey questionnaire, and self-esteem was measured by Rosenberg's Self-Esteem (RSE) scale. Statistical analysis was performed using Student's t-test, analysis of variance and Fisher's least significant difference (LSD) test at a significance level of 5%. Participants were predominately men (86.7%) with a mean age of 32.9 (standard deviation [SD] = 9.47) years, low education level and low income. The SF-36 dimensions that received the lowest scores were physical functioning, role physical and role emotional. Cronbach's alpha for the SF-36 questionnaire was 0.80. A significant statistical difference was found between the presence of pressure ulcers and low scores on mental health (P = 0.001), as determined by Student's t-test. The mean self-esteem score was 8.35 and there was a significant statistical difference between low self-esteem scores and occupation (P = 0.008). Participants reported low QoL and self-esteem. The results provide background information that may be useful in the development of strategies to reduce the impact of spinal cord injury (SCI) on the life and health of persons with SCI, improving their QoL.

  6. Diagnosis of hemodynamic compromise in patients with chronic cerebral ischemia

    International Nuclear Information System (INIS)

    Kuroda, Satoshi; Sakuragi, Mitsugi; Motomiya, Mineo; Nakagawa, Tango; Mitsumori, Kenji; Tsuru, Mitsuo; Takigawa, Shugo; Kamiyama, Hiroyasu; Abe, Hiroshi.

    1990-01-01

    To evaluate the efficacy of tests for selecting patients with hemodynamic compromise, measurement of cerebral blood volume (CBV) with 99m Tc-RBC single photon emission computed tomography (SPECT) was performed in thirteen patients with occlusive cerebrovascular disease, and was compared with results obtained by 133 Xe SPECT and acetazolamide (Diamox) test. All patients in our study suffered TIA, RIND, or minor completed stroke. Cerebral angiography demonstrated severe stenosis or occlusion in the ipsilateral internal carotid artery or middle cerebral artery, although plain CT scan or MRI revealed no or, if any, only localized infarcted lesions. Regional cerebral blood volume (rCBV) was measured with 99m Tc-RBC SPECT and regional cerebral blood flow (rCBF) was measured with 133 Xe SPECT before and after intravenous injection of 10 - 12 mg/kg acetazolamide (Diamox). Our results suggest that the ipsilateral rCBV/rCBF (mean transit time) is a more sensitive index of the cerebral perfusion reserve than the use of only rCBV or rCBF of the ipsilateral hemisphere. Also, the ipsilateral rCBV/rCBF is significantly correlated (r= -0.72) with the Diamox reactivity of rCBF, which is considered to represent the cerebral vasodilatory capacity in patients with chronic cerebral ischemia. Postoperative SPECT study revealed remarkable improvement of ipsilateral rCBV/rCBF and Diamox reactivity in four patients who underwent EC/IC bypass surgery to improve the hemodynamic compromise. In conclusion, our results suggest that the measurement of rCBV/rCBF with 133 Xe SPECT and 99m Tc-RBC SPECT is useful for detecting the hemodynamic compromise in patients with occlusive cerebrovascular disease. (author)

  7. A double-blind, placebo-controlled study of repetitive transnasal sphenopalatine ganglion blockade with tx360(®) as acute treatment for chronic migraine.

    Science.gov (United States)

    Cady, Roger; Saper, Joel; Dexter, Kent; Manley, Heather R

    2015-01-01

    bupivacaine group and 12 in the saline group. A repeated measures analysis of variance showed that subjects receiving treatment with bupivacaine experienced a significant reduction in the numeric rating scale scores compared with those receiving saline at baseline (M=3.78 vs M=3.18, P=.10), 15 minutes (M=3.51 vs M=2.53, P<.001), 30 minutes (M=3.45 vs M=2.41, P<.001), and 24 hours after treatment (M=4.20 vs M=2.85, P<.001), respectively. Headache Impact Test-6 scores were statistically significantly decreased in subjects receiving treatments with bupivacaine from before treatment to the final treatment (Mdiff  = -4.52, P=.005), whereas no significant change was seen in the saline group (Mdiff  = -1.50, P=.13). SPG blockade with bupivacaine delivered repetitively for 6 weeks with the Tx360(®) device demonstrates promise as an acute treatment of headache in some subjects with CM. Statistically significant headache relief is noted at 15 and 30 minutes and sustained at 24 hours for SPG blockade with bupivacaine vs saline. The Tx360(®) device was simple to use and not associated with any significant or lasting adverse events. Further research on sphenopalatine ganglion blockade is warranted. © 2014 The Authors. Headache published by Wiley Periodicals, Inc. on behalf of American Headache Society.

  8. A Double-Blind, Placebo-Controlled Study of Repetitive Transnasal Sphenopalatine Ganglion Blockade With Tx360® as Acute Treatment for Chronic Migraine

    Science.gov (United States)

    Cady, Roger; Saper, Joel; Dexter, Kent; Manley, Heather R

    2015-01-01

    included 38 subjects, 26 in the bupivacaine group and 12 in the saline group. A repeated measures analysis of variance showed that subjects receiving treatment with bupivacaine experienced a significant reduction in the numeric rating scale scores compared with those receiving saline at baseline (M = 3.78 vs M = 3.18, P = .10), 15 minutes (M = 3.51 vs M = 2.53, P < .001), 30 minutes (M = 3.45 vs M = 2.41, P < .001), and 24 hours after treatment (M = 4.20 vs M = 2.85, P < .001), respectively. Headache Impact Test-6 scores were statistically significantly decreased in subjects receiving treatments with bupivacaine from before treatment to the final treatment (Mdiff = −4.52, P = .005), whereas no significant change was seen in the saline group (Mdiff = −1.50, P = .13). Conclusion SPG blockade with bupivacaine delivered repetitively for 6 weeks with the Tx360® device demonstrates promise as an acute treatment of headache in some subjects with CM. Statistically significant headache relief is noted at 15 and 30 minutes and sustained at 24 hours for SPG blockade with bupivacaine vs saline. The Tx360® device was simple to use and not associated with any significant or lasting adverse events. Further research on sphenopalatine ganglion blockade is warranted. PMID:25338927

  9. Autobiographical memory compromise in Alzheimer's disease: a cognitive and clinical overview.

    Science.gov (United States)

    El Haj, Mohamad; Roche, Jean; Gallouj, Karim; Gandolphe, Marie-Charlotte

    2017-12-01

    Autobiographical memory refers to memory for personal information. The compromise of autobiographical memory in Alzheimer's disease (AD) results in a loss of knowledge about events and facts that defined the patients' life, and consequently, degradation of their self-knowledge and sense of identity. The compromise of autobiographical memory in AD can be attributed to a diminished subjective experience of memory and a diminished sense of the self. Our review provides a comprehensive overview of cognitive and clinical processes that may be involved in difficulties to retrieve autobiographical memories in Alzheimer's disease. Our review also proposes a theoretical model according to which, the diminished ability to retrieve contextual information and the overgenerality of recall result in a diminished subjective experience of past and future thinking. Besides its theoretical contribution, our review proposes clinical applicability for evaluation and rehabilitation of autobiographical memory in AD.

  10. Orthodontic space closure using simple mechanics in compromised first molar extraction spaces: Case series

    Directory of Open Access Journals (Sweden)

    Prashant M Dhole

    2018-01-01

    Full Text Available Orthodontic treatments involving missing or compromised first permanent molars are often challenging cases to treat considering the loss of potential anchor tooth. The case series presents orthodontic treatment in three patients with mutilated/absent first permanent molars. The treatment goals were to be accomplished by extraction of compromised teeth and retention of healthy dentition. The diagnosis and problem list needed extractions to accomplish the treatment goals. The possibility of extracting compromised first permanent molars instead of other healthy teeth was considered. A setup was done to determine the final occlusion in asymmetric extraction cases. Fixed appliances were used with simple mechanics without any additional anchorage devices. Case-based retention protocols were followed. All the three patients achieved the predetermined treatment objectives of improved esthetics and healthy and stable functional occlusion. The simple and efficient mechanics and finishing techniques for comprehensive orthodontic treatment with first molar extractions presented in the report should help clinicians when treating similar patients.

  11. Long-term effectiveness of sphenopalatine ganglion stimulation for cluster headache

    DEFF Research Database (Denmark)

    Jürgens, Tim P; Barloese, Mads; May, Arne

    2017-01-01

    Objectives The sphenopalatine ganglion (SPG) plays a pivotal role in cluster headache (CH) pathophysiology as the major efferent parasympathetic relay. We evaluated the long-term effectiveness of SPG stimulation in medically refractory, chronic CH patients. Methods Thirty-three patients were...... attacks (180.5 ± 344.8, range 2-1581 per patient) were evaluated. At 24 months, 45% ( n = 15) of patients were acute responders. Among acute responders, a total of 4340 attacks had been treated, and in 78% of these, effective therapy was achieved using only SPG stimulation (relief from moderate or greater...... response through the 24-month evaluation. Conclusions In the population of disabled, medically refractory chronic CH patients treated in this study, SPG stimulation is an effective acute therapy in 45% of patients, offering sustained effectiveness over 24 months of observation. In addition, a maintained...

  12. Gender self-perception – Fixed or fluid?

    Directory of Open Access Journals (Sweden)

    2015-09-01

    Results: An independent samples t test showed that there was no significant difference between the two female market value conditions on either explicit SPG (t(114=-0.638, p=0.192 or visual SPG (t(114=-1.73, p=0.84. Similarly, males showed no significant differences on either explicit (t(63=-1.65,p=0.69 or visual SPG (t(63, 0.472, p=0.42. Conclusions: Self-perceived gender was not shifted by viewing high/low gendered same sex others in this case. Possible reasons for this result were proposed.

  13. Stimulation of the sphenopalatine ganglion in intractable cluster headache

    DEFF Research Database (Denmark)

    Jürgens, Tim P; Schoenen, Jean; Rostgaard, Jørgen

    2014-01-01

    , but only very few randomized controlled studies exist in the field of neuromodulation for the treatment of drug-refractory headaches. Based on the prominent role of the cranial parasympathetic system in acute cluster headache attacks, high-frequency sphenopalatine ganglion (SPG) stimulation has been shown...... patients and the first commercially available CE-marked SPG neurostimulator system has been introduced for cluster headache, patient selection and care should be standardized to ensure maximal efficacy and safety. As only limited data have been published on SPG stimulation, standards of care based...

  14. Iran. Nuclear crisis: the continuous compromise?

    International Nuclear Information System (INIS)

    Cherief, Hamza

    2016-01-01

    The author comments and discusses the content and implications of the Joint Comprehensive Plan of Action which has been adopted in July 2015 by the Republic of Iran and the members of the E3/EU+3 group. According to this text, a flexible normative and institutional framework is defined, according to which negotiation must prevail on the reinstatement of economic sanctions. While significantly lowering objectives related to non proliferation, this plan aims (through various arrangements which are discussed by the author) at maintaining Iran under the threshold of nuclear power. The author also comments various aspects related to the implementation of this Plan of Action, and outlines that they rely on the search for a continuous compromise: negotiations and involvement of international bodies are foreseen before application of sanctions

  15. Validation of satellite based precipitation over diverse topography of Pakistan

    Science.gov (United States)

    Iqbal, Muhammad Farooq; Athar, H.

    2018-03-01

    This study evaluates the Tropical Rainfall Measuring Mission (TRMM) Multi-Satellite Precipitation Analysis (TMPA) product data with 0.25° × 0.25° spatial and post-real-time 3 h temporal resolution using point-based Surface Precipitation Gauge (SPG) data from 40 stations, for the period 1998-2013, and using gridded Asian Precipitation ˗ Highly Resolved Observational Data Integration Towards Evaluation of Water Resources (APHRODITE) data abbreviated as APH data with 0.25° × 0.25° spatial and daily temporal resolution for the period 1998-2007, over vulnerable and data sparse regions of Pakistan (24-37° N and 62-75° E). To evaluate the performance of TMPA relative to SPG and APH, four commonly used statistical indicator metrics including Mean Error (ME), Mean Absolute Error (MAE), Root Mean Square Error (RMSE), and Correlation Coefficient (CC) are employed on daily, monthly, seasonal as well as on annual timescales. The TMPA slightly overestimated both SPG and APH at daily, monthly, and annual timescales, however close results were obtained between TMPA and SPG as compared to those between TMPA and APH, on the same timescale. The TMPA overestimated both SPG and APH during the Pre-Monsoon and Monsoon seasons, whereas it underestimated during the Post-Monsoon and Winter seasons, with different magnitudes. Agreement between TMPA and SPG was good in plain and medium elevation regions, whereas TMPA overestimated APH in 31 stations. The magnitudes of MAE and RMSE were high at daily timescale as compared to monthly and annual timescales. Relatively large MAE was observed in stations located over high elevation regions, whereas minor MAE was recorded in plain area stations at daily, monthly, and annual timescales. A strong positive linear relationship between TMPA and SPG was established at monthly (0.98), seasonal (0.93 to 0.98) and annual (0.97) timescales. Precipitation increased with the increase of elevation, and not only elevation but latitude also affected the

  16. A method for identifying compromised clients based on DNS traffic analysis

    DEFF Research Database (Denmark)

    Stevanovic, Matija; Pedersen, Jens Myrup; D’Alconzo, Alessandro

    2017-01-01

    DNS is widely abused by Internet criminals in order to provide reliable communication within malicious network infrastructure as well as flexible and resilient hosting of malicious content. This paper presents a novel detection method that can be used for identifying potentially compromised clien...

  17. Respect in forensic psychiatric nurse-patient relationships: a practical compromise.

    Science.gov (United States)

    Rose, Donald N; Peter, Elizabeth; Gallop, Ruth; Angus, Jan E; Liaschenko, Joan

    2011-03-01

    The context of forensic psychiatric nursing is distinct from other psychiatric settings as, it involves placement of patients in secure environments with restrictions determined by the courts. Previous literature has identified that nurses morally struggle with respecting patients who have committed heinous offences, which can lead to the patient being depersonalized and dehumanized. Although respect is fundamental to ethical nursing practice, it has not been adequately explored conceptually or empirically. As a result, little knowledge exists that identifies how nurses develop, maintain, and express respect for patients. The purpose of this study is to analyze the concept of respect systematically, from a forensic psychiatric nurse's perspective using the qualitative methodology of focused ethnography. Forensic psychiatric nurses were recruited from two medium secure forensic rehabilitation units. In the first interview, 13 registered nurses (RNs) and two registered practical nurses (RPNs) participated, and although all informants were invited to the second interview, six RNs were lost to follow-up. Despite this loss, saturation was achieved and the data were interpreted through a feminist philosophical lens. Respect was influenced by factors categorized into four themes: (1) emotive-cognitive reactions, (2) nonjudgmental approach, (3) social identity and power, and (4) context. The data from the themes indicate that forensic psychiatric nurses strike a practical compromise, in their understanding and enactment of respect in therapeutic relationships with forensic psychiatric patients. © 2011 International Association of Forensic Nurses.

  18. Live attenuated S. Typhimurium vaccine with improved safety in immuno-compromised mice.

    Directory of Open Access Journals (Sweden)

    Balamurugan Periaswamy

    Full Text Available Live attenuated vaccines are of great value for preventing infectious diseases. They represent a delicate compromise between sufficient colonization-mediated adaptive immunity and minimizing the risk for infection by the vaccine strain itself. Immune defects can predispose to vaccine strain infections. It has remained unclear whether vaccine safety could be improved via mutations attenuating a vaccine in immune-deficient individuals without compromising the vaccine's performance in the normal host. We have addressed this hypothesis using a mouse model for Salmonella diarrhea and a live attenuated Salmonella Typhimurium strain (ssaV. Vaccination with this strain elicited protective immunity in wild type mice, but a fatal systemic infection in immune-deficient cybb(-/-nos2(-/- animals lacking NADPH oxidase and inducible NO synthase. In cybb(-/-nos2(-/- mice, we analyzed the attenuation of 35 ssaV strains carrying one additional mutation each. One strain, Z234 (ssaV SL1344_3093, was >1000-fold attenuated in cybb(-/-nos2(-/- mice and ≈100 fold attenuated in tnfr1(-/- animals. However, in wt mice, Z234 was as efficient as ssaV with respect to host colonization and the elicitation of a protective, O-antigen specific mucosal secretory IgA (sIgA response. These data suggest that it is possible to engineer live attenuated vaccines which are specifically attenuated in immuno-compromised hosts. This might help to improve vaccine safety.

  19. Parental care compromises feeding in the pumpkinseed ( Lepomis gibbosus)

    Science.gov (United States)

    Zięba, G.; Dukowska, M.; Przybylski, M.; Fox, M. G.; Smith, C.

    2018-04-01

    Providing parental care is potentially costly. Costs can arise through elevated energy expenditure or from an increased risk of mortality. A cost of parental care can also occur because a parent is compromised in their ability to forage. We used pumpkinseed Lepomis gibbosus, a fish with an alternative male mating strategy, to test whether parental males differed in their feeding in comparison with females and cuckolder males. To address this question, we examined the stomach contents of female, cuckolder male, and parental male pumpkinseed during the breeding season over an entire diel cycle. We showed that parental males had a lower total weight of food in their stomachs in comparison with females, while cuckolder males did not. Parental males also had a lower weight and number of chironomids in their stomachs. The temporal pattern of feeding of parental males diverged from that of females, and they had a lower probability of pupal chironomids in their stomachs, which implies spatial segregation in foraging. Parental males had a greater probability of conspecific eggs in their stomachs than females, while the probability of egg cannibalism did not differ between cuckolder males and females. Overall, these finding meet predictions in accordance with an assumption that parental care and territoriality can compromise feeding.

  20. 7 CFR 4290.1710 - Secretary's authority to collect or compromise claims.

    Science.gov (United States)

    2010-01-01

    ... BUSINESS-COOPERATIVE SERVICE AND RURAL UTILITIES SERVICE, DEPARTMENT OF AGRICULTURE RURAL BUSINESS INVESTMENT COMPANY (âRBICâ) PROGRAM Financial Assistance for RBICs (Leverage) Miscellaneous § 4290.1710... consideration as he or she deems reasonable, collect or compromise all claims relating to obligations he or she...

  1. Novel La(Fe,Si){sub 13}/Cu composites for magnetic cooling

    Energy Technology Data Exchange (ETDEWEB)

    Lyubina, Julia; Hannemann, Ullrich; Ryan, Mary P. [Department of Materials, Imperial College London, London, SW7 2AZ (United Kingdom); Cohen, Lesley F. [Department of Physics, Imperial College London, London, SW7 2AZ (United Kingdom)

    2012-11-15

    An approach to engineering magnetic refrigerant materials with defined thermal transport properties is demonstrated using the example of high magnetocaloric performance La-Fe-Si alloys. A tunability of up to 300% of the thermal conductivity can be achieved in composites consisting of a La(Fe,Si){sub 13} compound and Cu prepared by electroless copper plating without compromising the magnitude of the magnetocaloric effect. (Copyright copyright 2012 WILEY-VCH Verlag GmbH and Co. KGaA, Weinheim)

  2. CLINICAL CASE OF TREATMENT WITH RIBOSOMAL COMPLEX IN CHILD WITH COMPROMISED IMMUNITY

    Directory of Open Access Journals (Sweden)

    A.A. Alekseeva

    2011-01-01

    Full Text Available The leading pathology in children is acute respiratory infections (ARI according to the expert data of WHO. The incidence of prolonged and recurrent types of ARI increases during recent years. Patients with these diseases subsequently form the group of children with compromised immunity. Immunogens and immunomodulators are the drugs of nonspecific prophylaxis which are used for the prevention of ARI. The group of bacterial immunomodulators is big but most well-studied systemic drug from this group is Ribomunyl. Ribosomal complex is effective and safe in pediatric practice. The article presents the clinical case of treatment with ribosomal complex in immunocompronised child with allergic pathology.Key words: children with compromised immunity, allergy, acute respiratory infections, treatment.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2011; 10 (2: 211–215

  3. Heavy Cigarette Smokers in a Chinese Population Display a Compromised Permeability Barrier

    Science.gov (United States)

    Xin, Shujun; Ye, Li; Lv, Chengzhi; Elias, Peter M.

    2016-01-01

    Cigarette smoking is associated with various cutaneous disorders with defective permeability. Yet, whether cigarette smoking influences epidermal permeability barrier function is largely unknown. Here, we measured skin biophysical properties, including permeability barrier homeostasis, stratum corneum (SC) integrity, SC hydration, skin surface pH, and skin melanin/erythema index, in cigarette smokers. A total of 99 male volunteers were enrolled in this study. Smokers were categorized as light-to-moderate (hydration and skin melanin/erythema index on the dorsal hand, forehead, and cheek. Basal transepidermal water loss (TEWL) and barrier recovery rates were assessed on the forearm. A Skin-pH-Meter pH900 was used to measure skin surface pH. Our results showed that heavy cigarette smokers exhibited delayed barrier recovery after acute abrogation (1.02% ± 13.06 versus 16.48% ± 6.07), and barrier recovery rates correlated negatively with the number of daily cigarettes consumption (p = 0.0087). Changes in biophysical parameters in cigarette smokers varied with body sites. In conclusion, heavy cigarette smokers display compromised permeability barrier homeostasis, which could contribute, in part, to the increased prevalence of certain cutaneous disorders characterized by defective permeability. Thus, improving epidermal permeability barrier should be considered for heavy cigarette smokers. PMID:27437403

  4. Calcitonin gene-related peptide and its receptor components in the human sphenopalatine ganglion -- interaction with the sensory system

    DEFF Research Database (Denmark)

    Csati, Anett; Tajti, Janos; Tuka, Bernadett

    2012-01-01

    , Western blot technique was used to demonstrate the existence of CGRP receptor components in rat SPG. CGRP immunoreactive fibers were frequently found intraganglionic in the SPG in the vicinity of neurons. CLR immunoreactivity was observed in satellite glial cells (SGCs) as well as in nerve fibers......, but not in neurons. RAMP1 immunoreactivity was localized in many neurons and SGCs. Thus, the two CGRP receptor components together were found in the SGCs. In addition, Western blot revealed the presence of RAMP1 and CLR in rat SPG. Our results suggest a possible sensory influence in the parasympathetic cranial...

  5. 17D yellow fever vaccine elicits comparable long-term immune responses in healthy individuals and immune-compromised patients

    NARCIS (Netherlands)

    Wieten, R. W.; Goorhuis, A.; Jonker, E. F. F.; de Bree, G. J.; de Visser, A. W.; van Genderen, P. J. J.; Remmerswaal, E. B. M.; ten Berge, I. J. M.; Visser, L. G.; Grobusch, M. P.; van Leeuwen, E. M. M.

    2016-01-01

    The 17D live attenuated yellow fever (YF) vaccine is contra-indicated in immune-compromised individuals and may elicit a suboptimal immunologic response. The aim of this study is to assess whether long-term immune responses against the YF vaccine are impaired in immune-compromised patients. Fifteen

  6. Evaluation of instant cup noodle, irradiated for immuno-compromised patients

    International Nuclear Information System (INIS)

    Lee, Ji-Hye; Kim, Jae-Kyung; Park, Jae-Nam; Yoon, Young-Min; Sung, Nak-Yun; Kim, Jae-Hun; Song, Beom-Seok; Yook, Hong-Sun; Kim, Byeong-Keun; Lee, Ju-Woon

    2012-01-01

    In the present study, initial microbial load of instant cup noodle (ICN) was investigated and gamma irradiation applied to develop immuno-compromised patients food for their safe consumption. The initial microbial population of dried vegetable and meat, and noodle was below the detection limit (1 log CFU/g); however, that of seasoning powder was just above 4 log CFU/g. Moreover, rehydrated-ICN with water at 100 °C still show above 3 log CFU/g of microbial load, which indicates the need for an additional process to control microbial safety of the seasoning powder. The total aerobic bacteria in seasoning powder and rehydrated-ICN could be controlled with 17 kGy gamma irradiation. This result referred 17 kGy gamma irradiation could reach ‘practical sterility’ of ICN. The overall difference in sensory properties between the non-irradiated and irradiated ICN was insignificant. Thus, gamma irradiation could improve the microbial quality of ICN, and reduce the risk of infection posed by the seasoning powder, without any adverse effects on their sensory quality. These results suggest that gamma-irradiated ICN can be used as a snack food for immuno-compromised patients.

  7. Evaluation of instant cup noodle, irradiated for immuno-compromised patients

    Science.gov (United States)

    Lee, Ji-Hye; Kim, Jae-Kyung; Park, Jae-Nam; Yoon, Young-Min; Sung, Nak-Yun; Kim, Jae-Hun; Song, Beom-Seok; Yook, Hong-Sun; Kim, Byeong-Keun; Lee, Ju-Woon

    2012-08-01

    In the present study, initial microbial load of instant cup noodle (ICN) was investigated and gamma irradiation applied to develop immuno-compromised patients food for their safe consumption. The initial microbial population of dried vegetable and meat, and noodle was below the detection limit (1 log CFU/g); however, that of seasoning powder was just above 4 log CFU/g. Moreover, rehydrated-ICN with water at 100 °C still show above 3 log CFU/g of microbial load, which indicates the need for an additional process to control microbial safety of the seasoning powder. The total aerobic bacteria in seasoning powder and rehydrated-ICN could be controlled with 17 kGy gamma irradiation. This result referred 17 kGy gamma irradiation could reach 'practical sterility' of ICN. The overall difference in sensory properties between the non-irradiated and irradiated ICN was insignificant. Thus, gamma irradiation could improve the microbial quality of ICN, and reduce the risk of infection posed by the seasoning powder, without any adverse effects on their sensory quality. These results suggest that gamma-irradiated ICN can be used as a snack food for immuno-compromised patients.

  8. Construction of a Solid State Research Facility, Building 3150

    International Nuclear Information System (INIS)

    1993-07-01

    The Department of Energy (DOE) proposes to construct a new facility to house the Materials Synthesis Group (MSG) and the Semiconductor Physics Group (SPG) of the Solid State Division, Oak Ridge National Laboratory (ORNL). The location of the proposed action is Roane County, Tennessee. MSG is involved in the study of crystal growth and the preparation and characterization of advanced materials, such as high-temperature superconductors, while SPG is involved in semiconductor physics research. All MSG and a major pardon of SPG research activities are now conducted in Building 2000, a deteriorating structure constructed in the 1940. The physical deterioration of the roof; the heating, ventilation, and air conditioning (HVAC) system; and the plumbing make this building inadequate for supporting research activities. The proposed project is needed to provide laboratory and office space for MSG and SPG and to ensure that research activities can continue without interruption due to deficiencies in the building and its associated utility systems

  9. Differential diagnosis of the signal-compromised lunate in MRI

    International Nuclear Information System (INIS)

    Schmitt, R.; Christopoulos, G.; Coblenz, G.; Froehner, S.; Brunner, H.; Kalb, K.; Krimmer, H.; Lanz, U.

    2005-01-01

    Purpose: To define both the underlying pathology and diagnostic criteria in lunates presenting with conspicuous signal pattern in MRI. Materials and Methods: The retrospective evaluation of 2940 MRI examinations revealed 203 patients with signal alterations of the lunate. All MRI examinations were performed on 1.5-Tesla platforms using dedicated surface coils and an intravenous contrast agent. To establish a definitive diagnosis, a total of 252 MRI examinations (49 follow-ups), 22 CT examinations and 4 arthroscopic studies were obtained in addition to the obligatory conventional radiographs. Results: Incorporating all clinical data, radiographs and MRI examinations succeeded in assigning a diagnosis in 136 signal-compromised lunates (67.0%), whereas additional diagnostic procedures or follow-up examinations were required for the definitive diagnosis in 57 cases (33.0%). The most frequent entities were 51 cases of Kienboeck's disease (25.1%), 47 cases of ulnolunate-(triquetral) impaction syndromes (23.2%) and 44 cases of intra-osseous ganglion cysts (21.7%). Other pathologies included 23 degenerative, 19 traumatic and 10 inflammatory changes as well as 9 congenital conditions. For MRI assessment of the altered lunate, the most important parameters were location and morphology as well as involvement of the articular and osseous structures of the carpus. Conclusion: The lunate may be affected by different pathological states of the wrist. In total, only one quarter of the signal-compromised lunate represented Kienboeck's disease. (orig.)

  10. [Tuberculosis in compromised hosts].

    Science.gov (United States)

    2003-11-01

    Recent development of tuberculosis in Japan tends to converge on a specific high risk group. The proportion of tuberculosis developing particularly from the compromised hosts in the high risk group is especially high. At this symposium, therefore, we took up diabetes mellitus, gastrectomy, dialysis, AIDS and the elderly for discussion. Many new findings and useful reports for practical medical treatment are submitted; why these compromised hosts are predisposed to tuberculosis, tuberculosis diagnostic and remedial notes of those compromised hosts etc. It is an important question for the future to study how to prevent tuberculosis from these compromised hosts. 1. Tuberculosis in diabetes mellitus: aggravation and its immunological mechanism: Kazuyoshi KAWAKAMI (Department of Internal Medicine, Division of Infectious Diseases, Graduate School and Faculty of Medicine, University of the Ryukyus). It has been well documented that diabetes mellitus (DM) is a major aggravating factor in tuberculosis. The onset of this disease is more frequent in DM patients than in individuals with any underlying diseases. However, the precise mechanism of this finding remains to be fully understood. Earlier studies reported that the migration, phagocytosis and bactericidal activity of neutrophils are all impaired in DM patients, which is related to their reduced host defense to infection with extracellular bacteria, such as S. aureus and E. colli. Host defense to mycobacterial infection is largely mediated by cellular immunity, and Th1-related cytokines, such as IFN-gamma and IL-12, play a central role in this response. It is reported that serum level of these cytokines and their production by peripheral blood mononuclear cells (PBMC) are reduced in tuberculosis patients with DM, and this is supposed to be involved in the high incidence of tuberculosis in DM. Our study observed similar findings and furthermore indicated that IFN-gamma and IL-12 production by BCG-stimulated PBMC was lower

  11. Happenstance and compromise: a gendered analysis of students' computing degree course selection

    Science.gov (United States)

    Lang, Catherine

    2010-12-01

    The number of students choosing to study computing at university continues to decline this century, with an even sharper decline in female students. This article presents the results of a series of interviews with university students studying computing courses in Australia that uncovered the influence of happenstance and compromise on course choice. This investigation provides an insight into the contributing factors into the continued downturn of student diversity in computing bachelor degree courses. Many females interviewed made decisions based on happenstance, many males interviewed had chosen computing as a compromise course, and family helped in the decision-making to a large degree in both genders. The major implication from this investigation is the finding that students of both genders appear to be socialised away from this discipline, which is perceived as a support or insurance skill, not a career in itself, in all but the most technical-oriented (usually male) student.

  12. Is ridge preservation/augmentation at periodontally compromised extraction sockets safe? A retrospective study.

    Science.gov (United States)

    Kim, Jung-Ju; Ben Amara, Heithem; Schwarz, Frank; Kim, Hae-Young; Lee, Jung-Won; Wikesjö, Ulf M E; Koo, Ki-Tae

    2017-10-01

    This study aimed to evaluate the safety of ridge preservation/augmentation procedures when performed at compromised extraction sockets. Patients subject to ridge preservation/augmentation at periodontally compromised sockets at Seoul National University Dental Hospital (SNUDH) were evaluated in a chart review. Tooth extractions due to acute infection were not included in our study as chronically formed lesions are the only lesions that can be detected from radiographic images. If inflammatory symptoms persisted following ridge preservation/augmentation and antimicrobial and anti-inflammatory therapy, the patient was categorized as a re-infection case and implanted biomaterial removed. Of 10,060 patients subject to tooth extractions at SNUDH, 2011 through 2015, 297 cases meeting inclusion criteria were reviewed. The severity and type of lesions were not specific because extracting data was only done by radiographic images and chart records. The review identified eight patients exhibiting inflammatory symptoms that required additional antimicrobial and anti-inflammatory therapy. Within this group, re-infection occurred in two patients requiring biomaterials removal. The final safety rate for the ridge preservation/augmentation was 99.3%. None of the demographic factors, systemic conditions or choice of biomaterial affected the safety of ridge preservation/augmentation. Alveolar ridge preservation/augmentation at periodontally compromised sockets appears safe following thorough removal of infectious source. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  13. Corporate corruption of the environment: sustainability as a process of compromise.

    Science.gov (United States)

    Nyberg, Daniel; Wright, Christopher

    2013-09-01

    A key response to environmental degradation, climate change and declining biodiversity has been the growing adoption of market principles in an effort to better value the social good of nature. Through concepts such as 'natural capitalism' and 'corporate environmentalism', nature is increasingly viewed as a domain of capitalist endeavour. In this article, we use convention theory and a pluralist understanding of social goods to investigate how the social good of the environment is usurped by the alternate social good of the market. Through analysis of interviews with sustainability managers and corporate documentation, we highlight how organizational actors employ compromise to temporally settle disputes between competing claims about environmental activities. Our findings contribute to an understanding of the processes of empirically grounded critique and the under-theorized concept of compromise between social goods. Rather than protecting the environment, the corporate promotion of sustainability facilitates the corruption of the social good of the environment and its conversion into a market commodity. © London School of Economics and Political Science 2013.

  14. Structural annotation of Beta-1,4-N-acetyl galactosaminyltransferase 1 (B4GALNT1) causing Hereditary Spastic Paraplegia 26.

    Science.gov (United States)

    Dad, Rubina; Malik, Uzma; Javed, Aneela; Minassian, Berge A; Hassan, Muhammad Jawad

    2017-08-30

    Beta-1,4-N-acetyl galactosaminyltransferase 1, B4GALNT1, is a GM2/GD2 synthase, involved in the expression of glycosphingolipids (GSLs) containing sialic acid. Mutations in the gene B4GALNT1 cause Hereditary Spastic Paraplegia 26 (HSP26). In present study we have made attempt to predict the potential structural of the human B4GALNT1 protein. The results illustrated that the amino acid sequences of B4GALNT1 are not 100% conserved among selected twenty species. One signal peptide and one transmembrane domain predicted in human wild type B4GALNT1 protein with aliphatic index of 92.76 and theoretical (iso-electric point) pI of 8.93. It was a kind of unstable protein with Grand average of hydropathicity (GRAVY) of -0.127. Various post-translational modifications were also predicted to exist in B4GALNT1 and predicted to interact with different proteins including ST8SIA5, SLC33A1, GLB1 and others. In the final round, reported missense mutations have shown the further decrease in stability of the protein. This in-silico analysis of B4GALNT1 protein will provide the basis for the further studies on structural variations and biological pathways involving B4GALNT1 in the HSP26. Copyright © 2017. Published by Elsevier B.V.

  15. Protozoan predation in soil slurries compromises determination of contaminant mineralization potential

    International Nuclear Information System (INIS)

    Badawi, Nora; Johnsen, Anders R.; Brandt, Kristian K.; Sørensen, Jan; Aamand, Jens

    2012-01-01

    Soil suspensions (slurries) are commonly used to estimate the potential of soil microbial communities to mineralize organic contaminants. The preparation of soil slurries disrupts soil structure, however, potentially affecting both the bacterial populations and their protozoan predators. We studied the importance of this “slurry effect” on mineralization of the herbicide 2-methyl-4-chlorophenoxyacetic acid (MCPA, 14 C-labelled), focussing on the effects of protozoan predation. Mineralization of MCPA was studied in “intact” soil and soil slurries differing in soil:water ratio, both in the presence and absence of the protozoan activity inhibitor cycloheximide. Protozoan predation inhibited mineralization in dense slurry of subsoil (soil:water ratio 1:3), but only in the most dilute slurry of topsoil (soil:water ratio 1:100). Our results demonstrate that protozoan predation in soil slurries may compromise quantification of contaminant mineralization potential, especially when the initial density of degrader bacteria is low and their growth is controlled by predation during the incubation period. - Highlights: ► We studied the protozoan impact on MCPA mineralization in soil slurries. ► Cycloheximide was used as protozoan inhibitor. ► Protozoa inhibited MCPA mineralization in dilute topsoil slurry and subsoil slurry. ► Mineralization potentials may be underestimated when using soil slurries. - Protozoan predation may strongly bias the quantification of mineralization potential when performed in soil slurries, especially when the initial density of degrader bacteria is low such as in subsoil or very dilute topsoil slurries.

  16. Optimizing Maxillary Aesthetics of a Severe Compromised Tooth through Orthodontic Movement and Dental Implants

    Directory of Open Access Journals (Sweden)

    Rafael Scaf de Molon

    2014-01-01

    Full Text Available Treatment of severe compromised tooth in the maxillary anterior area still poses great challenge to the clinicians. Several treatment modalities have been proposed to restore the function and aesthetics in teeth with advanced periodontal disease. The present study aims to report a case of traumatic injury of a left-maxillary central incisor with ridge preservation, orthodontic movement, and implant therapy. A 45-year-old woman underwent the proposed treatment for her left central incisor: basic periodontal therapy, xenogenous bone graft, and guided bone regeneration (GBR. Six months after the graft procedure, orthodontic movement by means of alignment and leveling was made and a coronal displacement of the gingival margin and vertical bone apposition could be observed after 13 months of active movement. Afterwards, a dental implant was placed followed by a connective tissue graft and immediate provisionalization of the crown. In conclusion, orthodontic movement was effective to improve the gingival tissue and alveolar bone prior to implant placement favoring the aesthetic results. Six years postoperatively, the results revealed height and width alveolar bone gain indicating that the treatment proposed was able to restore all the functional and aesthetic parameters.

  17. Activation of lower back muscles via FES for pressure sores prevention in paraplegia: a case study.

    Science.gov (United States)

    Vanoncini, M; Holderbaum, W; Andrews, B J

    2010-04-01

    The aim of this paper is to show the feasibility of the use of functional electrical stimulation (FES) applied to the lower back muscles for pressure sores prevention in paraplegia. The hypothesis under study is that FES induces a change in the pressure distribution on the contact area during sitting. Tests were conducted on a paraplegic subject (T5), sitting on a standard wheelchair and cushion. Trunk extensors (mainly the erector spinae) were stimulated using surface electrodes placed on the skin. A pressure mapping system was used to measure the pressure on the sitting surface in four situations: (a) no stimulation; (b) stimulation on one side of the spine only; (c) stimulation on both sides, at different levels; and (d) stimulation at the same level on both sides, during pressure-relief manoeuvres. A session of prolonged stimulation was also conducted. The experimental results show that the stimulation of the erector spinae on one side of the spine can induce a trunk rotation on the sagittal plane, which causes a change in the pressure distribution. A decrease of pressure on the side opposite to the stimulation was recorded. The phenomenon is intensified when different levels of stimulation are applied to the two sides, and such change can be sustained for a considerable time (around 5 minutes). The stimulation did not induce changes during pressure-relief manoeuvres. Finally, from this research we can conclude that the stimulation of the trunk extensors can be a useful tool for pressure sores prevention, and can potentially be used in a routine for pressure sores prevention based on periodical weight shifts.

  18. The mechanism of neurofeedback training for treatment of central neuropathic pain in paraplegia: a pilot study.

    Science.gov (United States)

    Hassan, Muhammad Abul; Fraser, Matthew; Conway, Bernard A; Allan, David B; Vuckovic, Aleksandra

    2015-10-13

    Central neuropathic pain has a prevalence of 40% in patients with spinal cord injury. Electroencephalography (EEG) studies showed that this type of pain has identifiable signatures, that could potentially be targeted by a neuromodulation therapy. The aim of the study was to investigate the putative mechanism of neurofeedback training on central neuropathic pain and its underlying brain signatures in patients with chronic paraplegia. Patients' EEG activity was modulated from the sensory-motor cortex, electrode location C3/Cz/C4/P4 in up to 40 training sessions Results. Six out of seven patients reported immediate reduction of pain during neurofeedback training. Best results were achieved with suppressing Ɵ and higher β (20-30 Hz) power and reinforcing α power at C4. Four patients reported clinically significant long-term reduction of pain (>30%) which lasted at least a month beyond the therapy. EEG during neurofeedback revealed a wide spread modulation of power in all three frequency bands accompanied with changes in the coherence most notable in the beta band. The standardized low resolution electromagnetic tomography analysis of EEG before and after neurofeedback therapy showed the statistically significant reduction of power in beta frequency band in all tested patients. Areas with reduced power included the Dorsolateral Prefrontal Cortex, the Anterior Cingulate Cortex and the Insular Cortex. Neurofeedback training produces both immediate and longer term reduction of central neuropathic pain that is accompanied with a measurable short and long term modulation of cortical activity. Controlled trials are required to confirm the efficacy of this neurofeedback protocol on treatment of pain. The study is a registered UKCRN clinical trial Nr 9824.

  19. Towards a legitimate compromise?: An exploration of Integrated Reporting in the Netherlands

    NARCIS (Netherlands)

    van Bommel, K.

    2014-01-01

    Purpose – The purpose of this paper is to examine the multiplicity of views on integrated reporting and to consider the possibility of, and impediments to, reconciling these multiple rationales (“orders of worth”) and thus gain legitimacy through a compromise. This sheds light on the understanding

  20. Safety and Efficacy of Alginate Adhesion Barrier Gel in Compromised Intestinal Anastomosis

    NARCIS (Netherlands)

    Chaturvedi, A.; Yauw, S.T.K.; Lomme, R.M.L.M.; Hendriks, T.; Goor, H. van

    2017-01-01

    BACKGROUND: For any anti-adhesive barrier developed for abdominal surgery, the use under conditions in which anastomotic healing is compromised needs to be investigated. The current study evaluates the effect of a new ultrapure alginate gel on early healing of high-risk anastomoses in the ileum and

  1. Political realities of statewide smoking legislation: the passage of California's Assembly Bill 13.

    Science.gov (United States)

    Macdonald, H R; Glantz, S A

    1997-01-01

    To prepare a history of the enactment of California Assembly Bill 13 (AB 13), a state law prohibiting smoking in most workplaces passed in 1994, and to discuss its initial impacts. Data were gathered from open ended interviews with representatives of voluntary health organisations, local government organisations, and principal legislators involved in the process, as well as observers around the state who could provide insight into the legislative process. Information was also obtained from legislative hearings and debates, public documents, letters and personal communications, internal memoranda, and news reports. The success of local tobacco control legislation in California led to a situation in which some health groups were willing to accept state preemption in order to attract the support of the state restaurant association for a bill. The decision to accept this preemption compromise was made by the state level offices of the voluntary health agencies without consulting the broader tobacco control community within California. In contrast, local tobacco control advocates did not accept this compromise, in part because of their belief that local legislation was a better device to educate the public, generate media coverage, and build community support for enforcement and implementation of clean indoor air and other tobacco control laws. Enactment of AB 13 was associated with a slowing of all local tobacco control legislation, including youth oriented laws. Because its supporters initially doubted that AB 13 would pass, there was never an effort to reconcile the policy differences between state oriented and locally oriented tobacco control policies. This lack of consensus, combined with the political realities inherent in passing any state legislation, led to a bill with ambiguous preemption language which replaced the "patchwork of local laws" with a "patchwork of local enforcement."

  2. Fibre reinforcement in a structurally compromised endodontically treated molar: a case report

    Directory of Open Access Journals (Sweden)

    Renita Soares

    2016-05-01

    Full Text Available The reconstruction of structurally compromised posterior teeth is a rather challenging procedure. The tendency of endodontically treated teeth (ETT to fracture is considerably higher than vital teeth. Although posts and core build-ups followed by conventional crowns have been generally employed for the purpose of reconstruction, this procedure entails sacrificing a considerable amount of residual sound enamel and dentin. This has drawn the attention of researchers to fibre reinforcement. Fibre-reinforced composite (FRC, designed to replace dentin, enables the biomimetic restoration of teeth. Besides improving the strength of the restoration, the incorporation of glass fibres into composite resins leads to favorable fracture patterns because the fibre layer acts as a stress breaker and stops crack propagation. The following case report presents a technique for reinforcing a badly broken-down ETT with biomimetic materials and FRC. The proper utilization of FRC in structurally compromised teeth can be considered to be an economical and practical measure that may obviate the use of extensive prosthetic treatment.

  3. Striking a Balance between Program Requirements and GT Principles: Writing a compromised GT proposal

    Directory of Open Access Journals (Sweden)

    Sherry L. Xie, Ph.D. Candidate

    2009-06-01

    Full Text Available Glaser’s term “compromised GT proposal” (2001, p.114 refers to the type of Grounded Theory (GT proposal that is written in order to conform to the requirements of a standardized qualitative research proposal. A GT proposal needs only to supply information on the area of interest, the data source and a statement of method to the effect that the researcher begin to collect, code and analyse the data and let the theory emerge. Thus, the proposal may only occupy “a page or two” (Glaser, 2001, p. 111. Whilst being consistent with the methodology, a GT proposal sometimes has to give way to the format specified by a PhD program or committee even though the format was not defined for a GT proposal and in some areas, conflicts with GT principles; for example, the format may require a literature review. This short paper reports on my experience of writing a compromised GT proposal as a first-time GT researcher. It describes how both Glaser’s advice on writing compromised GT research proposals and the characteristics of the substantive area of the proposed research were used to satisfy program requirements while still maintaining GT fundamentals.

  4. Algorithmic processing of intrinsic signals in affixed transmission speckle analysis (ATSA) (Conference Presentation)

    Science.gov (United States)

    Ghijsen, Michael T.; Tromberg, Bruce J.

    2017-03-01

    Affixed Transmission Speckle Analysis (ATSA) is a method recently developed to measure blood flow that is based on laser speckle imaging miniaturized into a clip-on form factor the size of a pulse-oximeter. Measuring at a rate of 250 Hz, ATSA is capable or obtaining the cardiac waveform in blood flow data, referred to as the Speckle-Plethysmogram (SPG). ATSA is also capable of simultaneously measuring the Photoplethysmogram (PPG), a more conventional signal related to light intensity. In this work we present several novel algorithms for extracting physiologically relevant information from the combined SPG-PPG waveform data. First we show that there is a slight time-delay between the SPG and PPG that can be extracted computationally. Second, we present a set of frequency domain algorithms that measure harmonic content on pulse-by-pulse basis for both the SPG and PPG. Finally, we apply these algorithms to data obtained from a set of subjects including healthy controls and individuals with heightened cardiovascular risk. We hypothesize that the time-delay and frequency content are correlated with cardiovascular health; specifically with vascular stiffening.

  5. Mutation in CPT1C Associated With Pure Autosomal Dominant Spastic Paraplegia.

    Science.gov (United States)

    Rinaldi, Carlo; Schmidt, Thomas; Situ, Alan J; Johnson, Janel O; Lee, Philip R; Chen, Ke-Lian; Bott, Laura C; Fadó, Rut; Harmison, George H; Parodi, Sara; Grunseich, Christopher; Renvoisé, Benoît; Biesecker, Leslie G; De Michele, Giuseppe; Santorelli, Filippo M; Filla, Alessandro; Stevanin, Giovanni; Dürr, Alexandra; Brice, Alexis; Casals, Núria; Traynor, Bryan J; Blackstone, Craig; Ulmer, Tobias S; Fischbeck, Kenneth H

    2015-05-01

    The family of genes implicated in hereditary spastic paraplegias (HSPs) is quickly expanding, mostly owing to the widespread availability of next-generation DNA sequencing methods. Nevertheless, a genetic diagnosis remains unavailable for many patients. To identify the genetic cause for a novel form of pure autosomal dominant HSP. We examined and followed up with a family presenting to a tertiary referral center for evaluation of HSP for a decade until August 2014. Whole-exome sequencing was performed in 4 patients from the same family and was integrated with linkage analysis. Sanger sequencing was used to confirm the presence of the candidate variant in the remaining affected and unaffected members of the family and screen the additional patients with HSP. Five affected and 6 unaffected participants from a 3-generation family with pure adult-onset autosomal dominant HSP of unknown genetic origin were included. Additionally, 163 unrelated participants with pure HSP of unknown genetic cause were screened. Mutation in the neuronal isoform of carnitine palmitoyl-transferase (CPT1C) gene. We identified the nucleotide substitution c.109C>T in exon 3 of CPT1C, which determined the base substitution of an evolutionarily conserved Cys residue for an Arg in the gene product. This variant strictly cosegregated with the disease phenotype and was absent in online single-nucleotide polymorphism databases and in 712 additional exomes of control participants. We showed that CPT1C, which localizes to the endoplasmic reticulum, is expressed in motor neurons and interacts with atlastin-1, an endoplasmic reticulum protein encoded by the ATL1 gene known to be mutated in pure HSPs. The mutation, as indicated by nuclear magnetic resonance spectroscopy studies, alters the protein conformation and reduces the mean (SD) number (213.0 [46.99] vs 81.9 [14.2]; P lipid droplets on overexpression in cells. We also observed a reduction of mean (SD) lipid droplets in primary cortical neurons

  6. 32 CFR 310.14 - Notification when information is lost, stolen, or compromised.

    Science.gov (United States)

    2010-07-01

    ... THE SECRETARY OF DEFENSE (CONTINUED) PRIVACY PROGRAM DOD PRIVACY PROGRAM Systems of Records § 310.14... the individual of any loss, theft, or compromise (See also, § 310.50 for reporting of the breach to Senior Component Official for Privacy and the Defense Privacy Office). (1) The notification shall be made...

  7. Osseous pseudo-myelomatose compromise, in leukemia chronic lymphoid

    International Nuclear Information System (INIS)

    Martinez Betancur, Octavio; Lopez de Goenaga, Maria Ines

    2000-01-01

    It was described a case of chronic lymphocytic leukemia in a 75 year old man, with pseudomyelomatosis osteolytic lesions in the skull, excluding other potential causes of osteolytic lesions in the clinical context of malignant lymphoproliferative neoplasm. The real frequency of osseous compromise in chronic lymphocytic leukemia is 10%. Lesions are defined as generalized osteoporosis and osteolysis with lacunar aspect, similar to myeloma lesions. Because histopathology in lymphoproliferative neoplasms may be similar, it might be difficult to diagnose chronic lymphocytic leukemia certainly, if the clinical manifestations are not considered. Differential diagnosis with other lymphoproliferative neoplasm is based basically in absolute lymphocytosis greater than 10 X 109/L, with lymphocytes with mature appearance

  8. The AAA+ ATPase TRIP13 remodels HORMA domains through N-terminal engagement and unfolding

    Energy Technology Data Exchange (ETDEWEB)

    Ye, Qiaozhen; Kim, Dong Hyun; Dereli, Ihsan; Rosenberg, Scott C.; Hagemann, Goetz; Herzog, Franz; Tóth, Attila; Cleveland, Don W.; Corbett, Kevin D.

    2017-06-28

    Proteins of the conserved HORMA domain family, including the spindle assembly checkpoint protein MAD2 and the meiotic HORMADs, assemble into signaling complexes by binding short peptides termed “closure motifs”. The AAA+ ATPase TRIP13 regulates both MAD2 and meiotic HORMADs by disassembling these HORMA domain–closure motif complexes, but its mechanisms of substrate recognition and remodeling are unknown. Here, we combine X-ray crystallography and crosslinking mass spectrometry to outline how TRIP13 recognizes MAD2 with the help of the adapter protein p31comet. We show that p31comet binding to the TRIP13 N-terminal domain positions the disordered MAD2 N-terminus for engagement by the TRIP13 “pore loops”, which then unfold MAD2 in the presence of ATP. N-terminal truncation of MAD2 renders it refractory to TRIP13 action in vitro, and in cells causes spindle assembly checkpoint defects consistent with loss of TRIP13 function. Similar truncation of HORMAD1 in mouse spermatocytes compromises its TRIP13-mediated removal from meiotic chromosomes, highlighting a conserved mechanism for recognition and disassembly of HORMA domain–closure motif complexes by TRIP13.

  9. Bilateral sphenopalatine ganglion block reduces blood pressure in never treated patients with essential hypertension. A randomized controlled single-blinded study.

    Science.gov (United States)

    Triantafyllidi, Helen; Arvaniti, Chrysa; Schoinas, Antonios; Benas, Dimitris; Vlachos, Stefanos; Palaiodimos, Leonidas; Pavlidis, George; Ikonomidis, Ignatios; Batistaki, Chrysanthi; Voumvourakis, Costas; Lekakis, John

    2018-01-01

    Sympathetic fibers connect sphenopalatine ganglion (SPG) with the central nervous system. We aimed to study the effect of SPG block in blood pressure (BP) in never treated patients with stage I-II essential hypertension. We performed bilateral SPG block with lidocaine 2% in 33 hypertensive patients (mean age 48±12years, 24 men) and a sham operation with water for injection in 11 patients who served as the control group (mean age 51±12years, 8 men). All patients have been subjected to 24h ambulatory blood pressure monitoring prior and a month after the SBG block in order to estimate any differences in blood pressure parameters. We defined as responders to SBG block those patients with a 24h SBP decrease ≥5mmHg. We found that 24h and daytime DBP (p=0.02) as well as daytime DBP load (p=0.03) were decreased in the study group a month after SPG block. In addition, a significant response was noted in 12/33 responders (36%) regarding: a. SBP and DBP during overall 24h and daytime (pblock is a promising, minimally invasive option of BP decrease in hypertensives, probably through SNS modulation. Additionally, due to its anesthetic effect, SPG block might act as a method of selection for those hypertensive patients with an activated SNS before any other invasive antihypertensive procedure. Copyright © 2017 Elsevier B.V. All rights reserved.

  10. Paraplegia-quadriplegia Independently Increases All Percutaneous Nephrolithotomy Complications: A Comparative Study Using the Modified Clavien System.

    Science.gov (United States)

    Danawala, Zeeshan A; Singh, Dinesh

    2015-05-01

    To investigate the perioperative complication rates for paraplegic-quadriplegic patients (PQPs) undergoing percutaneous nephrolithotomy (PCNL) as compared with non-PQPs using a standardized method of complication reporting via the Clavien system. Two hundred thirteen consecutive PCNLs performed by a single surgeon were analyzed. There were 31 and 115 patients separated into PQP and non-PQP groups, respectively. Data collection included demographic and clinical factors, as well as perioperative and delayed complications. Complications were organized by the Clavien grade. All- and initial-procedure complications were analyzed. The rate of adverse events for each Clavien grade was calculated, and statistical comparisons were made. The relationship between PQP and complication severity was investigated using univariate and multivariate analyses. There were 38 and 43 initial-procedure complications in the PQP and non-PQP groups, respectively. The rate of adverse events was higher across the spectrum of Clavien grades for the PQP group, specifically grade 1 (48.4% vs 20.2%; P = .002), grade 2 (22.6% vs 5.3%; P = .004), grade 3b (12.9% vs 2.6%; P = .038), grade 4a (6.5% vs 0%), and grade 4b (9.7% vs 1.8%; P = .066). Approximately 51.6% and 31.5% of PQPs and non-PQPs experienced ≥ 1 complications, respectively (odds ratio = 2.34; P = .05). Multivariate analysis demonstrated paraplegia or quadriplegia status to be an independent risk factor for the development of perioperative complications after adjusting for confounding factors (odds ratio = 2.91; P = .040). PCNL complication rates are higher in PQPs compared with non-PQPs. This study is one of the first in PCNL to use a standardized reporting system to highlight high-risk individuals within the stone population. Copyright © 2015 Elsevier Inc. All rights reserved.

  11. Low dose tubulin-binding drugs rescue peroxisome trafficking deficit in patient-derived stem cells in Hereditary Spastic Paraplegia

    Directory of Open Access Journals (Sweden)

    Yongjun Fan

    2014-05-01

    Full Text Available Hereditary Spastic Paraplegia (HSP is a genetically heterogeneous group of disorders, diagnosed by progressive gait disturbances with muscle weakness and spasticity, for which there are no treatments targeted at the underlying pathophysiology. Mutations in spastin are a common cause of HSP. Spastin is a microtubule-severing protein whose mutation in mouse causes defective axonal transport. In human patient-derived olfactory neurosphere-derived (ONS cells, spastin mutations lead to lower levels of acetylated α-tubulin, a marker of stabilised microtubules, and to slower speed of peroxisome trafficking. Here we screened multiple concentrations of four tubulin-binding drugs for their ability to rescue levels of acetylated α-tubulin in patient-derived ONS cells. Drug doses that restored acetylated α-tubulin to levels in control-derived ONS cells were then selected for their ability to rescue peroxisome trafficking deficits. Automated microscopic screening identified very low doses of the four drugs (0.5 nM taxol, 0.5 nM vinblastine, 2 nM epothilone D, 10 µM noscapine that rescued acetylated α-tubulin in patient-derived ONS cells. These same doses rescued peroxisome trafficking deficits, restoring peroxisome speeds to untreated control cell levels. These results demonstrate a novel approach for drug screening based on high throughput automated microscopy for acetylated α-tubulin followed by functional validation of microtubule-based peroxisome transport. From a clinical perspective, all the drugs tested are used clinically, but at much higher doses. Importantly, epothilone D and noscapine can enter the central nervous system, making them potential candidates for future clinical trials.

  12. Low dose tubulin-binding drugs rescue peroxisome trafficking deficit in patient-derived stem cells in Hereditary Spastic Paraplegia

    Science.gov (United States)

    Fan, Yongjun; Wali, Gautam; Sutharsan, Ratneswary; Bellette, Bernadette; Crane, Denis I.; Sue, Carolyn M.; Mackay-Sim, Alan

    2014-01-01

    ABSTRACT Hereditary Spastic Paraplegia (HSP) is a genetically heterogeneous group of disorders, diagnosed by progressive gait disturbances with muscle weakness and spasticity, for which there are no treatments targeted at the underlying pathophysiology. Mutations in spastin are a common cause of HSP. Spastin is a microtubule-severing protein whose mutation in mouse causes defective axonal transport. In human patient-derived olfactory neurosphere-derived (ONS) cells, spastin mutations lead to lower levels of acetylated α-tubulin, a marker of stabilised microtubules, and to slower speed of peroxisome trafficking. Here we screened multiple concentrations of four tubulin-binding drugs for their ability to rescue levels of acetylated α-tubulin in patient-derived ONS cells. Drug doses that restored acetylated α-tubulin to levels in control-derived ONS cells were then selected for their ability to rescue peroxisome trafficking deficits. Automated microscopic screening identified very low doses of the four drugs (0.5 nM taxol, 0.5 nM vinblastine, 2 nM epothilone D, 10 µM noscapine) that rescued acetylated α-tubulin in patient-derived ONS cells. These same doses rescued peroxisome trafficking deficits, restoring peroxisome speeds to untreated control cell levels. These results demonstrate a novel approach for drug screening based on high throughput automated microscopy for acetylated α-tubulin followed by functional validation of microtubule-based peroxisome transport. From a clinical perspective, all the drugs tested are used clinically, but at much higher doses. Importantly, epothilone D and noscapine can enter the central nervous system, making them potential candidates for future clinical trials. PMID:24857849

  13. Radiotherapy of heterotopic bone formation in patients with paraplegia. Preliminary results; Strahlentherapie heterotoper Ossifikationen bei Querschnittsgelaehmten. Praeliminaere Ergebnisse

    Energy Technology Data Exchange (ETDEWEB)

    Sautter-Bihl, M.L. [Klinik fuer Strahlentherapie, Staedtisches Klinikum Karlsruhe (Germany); Liebermeister, E. [Klinik fuer Strahlentherapie, Staedtisches Klinikum Karlsruhe (Germany); Heinze, H.G. [Klinik fuer Strahlentherapie, Staedtisches Klinikum Karlsruhe (Germany); Nanassy, A. [Klinik fuer Orthopaedie, Rehabilitationskrankenhaus Langensteinbach (Germany); Stoltze, D. [Klinik fuer Orthopaedie, Rehabilitationskrankenhaus Langensteinbach (Germany)

    1995-08-01

    In 20 patients with paralysis, 25 regions were irradiated with (mostly) 10 Gy in single fractions of 2 to 2.5 Gy using 8 MW photons. In 15 patients radiotherapy was performed as a primary treatment in the status of myositis; 7 patients were treated after (subtotal) resection of already manifest ossifications (2 patients were treated twice, primarily and postoperatively). In a minimum follow-up 12 weeks, none of the 20 irradiated patients showed any progression of the developing or already manifest ossification; thus mobilisation and rehabilitation could be carried out as desired. No side effects occurred. The preliminary results of the present study suggest that radiotherapy is an effective local treatment with minimal side effects for the prevention of heterotopic bone formation in patients with paraplegia. (orig.) [Deutsch] Bei 20 Patienten (18 Maenner, zwei Frauen, Alter 19 bis 62 Jahre) mit Querschnittssyndrom wurden 25 Regionen mit ueberwiegend 10 Gy a 2 bis 2,5 Gy Einzeldosis mit 8-MW-Photonen bestrahlt. Die Radiatio erfolgte bei 15 Patienten als Primaerprophylaxe im entzuendlichen Stadium, bei sieben Patienten sekundaer nach (subtotaler) Resektion von Ossifikationen (zwei Patienten wurden sowohl primaer als auch sekundaer bestrahlt). Bei einer Mindestnachbeobachtungszeit von zwoelf Wochen trat in keinem Fall eine Progression der sich entwickelnden bzw. bereits bestehenden heterotopen Ossifikationen auf: saemtliche Patienten konnten wunschgemaess mobilisiert und im Rahmen ihrer neurologischen Ausfaelle rehabilitiert werden. Nebenwirkungen traten nicht auf. Die vorliegende praeliminaeren Ergebnisse deuten hin, dass die Strahlentherapie eine effektive und nebenwirkungsarme lokale Therapie zur Verhinderung heterotoper Ossifikationen beim Querschnittssyndrom darstellt. (orig.)

  14. Comment on "Compromised birth outcomes and infant mortality among racial and ethnic groups"

    NARCIS (Netherlands)

    Van der Veen, WJ

    Frisbie, Forbes, and Pullum (1996) show that it is meaningful to account for low birth weight, preterm delivery, and intrauterine growth-retardation when analyzing differences in compromised birth outcomes and infant mortality among racial and ethnic groups. I compare their findings for the 1987

  15. Morgellons: contested illness, diagnostic compromise and medicalisation.

    Science.gov (United States)

    Fair, Brian

    2010-05-01

    The case of Morgellons illustrates how the emergence of a new medically contested illness intersected with and impacted on the diagnostic processes of an existing uncontested psychiatric condition, Delusional Parasitosis (DP). More specifically, the sociopolitical processes at play in the contested illness, Morgellons, dubiously reflect patient empowerment, as well the resilience and power of medical jurisdiction. This research offers insights into the contested illness and medicalisation literatures, and aims to bridge these two approaches towards the relationship between patient empowerment and medical authority, which I do through the notion of doctor-patient compromise. The data for this research come from a comprehensive qualitative analysis of Morgellons discourse through four key sources: the pro-Morgellons website Morgellons.org; the anti-Morgellons website Morgellonswatch.com; the popular media's portrayal of Morgellons; and the DP and Morgellons articles published in peer-reviewed medical journals, as made available on PubMed.

  16. Addressing diversity in schools through dialogue and compromise

    DEFF Research Database (Denmark)

    Olsen, Tore Vincents

    2018-01-01

    This article evaluates a decentralized Danish model for dealing with cultural and religious diversity at individual schools. This evaluation is based upon normative theories of toleration, recognition and domination and examines whether the model implies compromise with the (liberal) educational...... values stipulated in the national legislation. The model, reconstructed from government publications, is based on reaching accommodation through dialogue between school staff and parents/students, with the pragmatic aim of facilitating the participation of students in everyday school activities....... The model is noteworthy because it appears to break with the widespread ‘retreat from multiculturalism’ predicated on the defence of liberal values, and because properly dealing with diversity at schools is important for ensuring students’ well-being and academic success....

  17. Management and design of hospital pens relative to behavior of the compromised dairy cow: A questionnaire survey of Iowa dairy farms

    DEFF Research Database (Denmark)

    Fogsgaard, Katrine Kop; Herskin, Mette S.; Gorden, Patrick J.

    2016-01-01

    on best prac-tices for housing and management of compromised cows. The purpose of this study was to providedescriptive information about management and husbandry practices of compromised cows on dairyfarms in Iowa. A questionnaire-based survey was designed to examine demographic information, designand...

  18. Effect of reinforcement with resin composite on fracture strength of structurally compromised roots.

    Science.gov (United States)

    Fukui, Yuji; Komada, Wataru; Yoshida, Keiichi; Otake, Shiho; Okada, Daizo; Miura, Hiroyuki

    2009-09-01

    This study was aimed at evaluating the fracture resistance of structurally compromised roots restored with four different post and core systems. Thirty-two bovine roots were uniformly shaped to simulate human mandibular premolar roots. The roots were divided into four groups based on the type of restoration: cemented cast post and core (Group MC), resin composite build-up (Group CR), resin composite and prefabricated glass fiber post build-up (Group FRC), and thick-layer dual-cured resin composite-reinforced small-diameter tapered cast post and core (Group CRM). After a static loading test, the failure mode and fracture resistance were recorded. Group CRM (719.38+/-196.73 N) exhibited a significantly high fracture resistance compared with the other groups (Group MC: 429.56+/-82.43 N; Group CR: 349.56+/-66.21 N; Group FRC: 398.94+/-112.71 N; pCRM exhibited better mechanical properties for structurally compromised roots with no ferrules, although all types of restorations showed non-restorable fracture modes.

  19. Relationship between Lower Tendency to Deceive in Aging and Inhibitory Compromise.

    Science.gov (United States)

    El Haj, Mohamad; Antoine, Pascal

    2018-01-01

    Deception can be associated with a heterogeneous network of concepts such as exaggeration, misleading, white lies, and faking. This paper assesses the tendency to deceive in aging. Our main aim was to assess whether older adults would demonstrate a low tendency to deceive. A total of 42 older adults (mean age 67.64 years, SD 7.87) and 45 younger adults (mean age 21.71 years, SD 2.66) were administered a deception scale including items such as "I sometimes tell lies if I have to" or "I never take things that don't belong to me." Participants were also administered an inhibition task. The results demonstrated a low tendency to deceive and low inhibition in older adults compared with younger ones. The low tendency to deceive in the older adults was significantly correlated with their diminished inhibitory ability. The low tendency to deceive in aging seems to be related to a difficulty in inhibiting an honest response. Since inhibitory compromise has been considered mainly to trigger negative consequences for cognition, the present paper illustrates how this age-related compromise can be associated with positive social outcomes, i.e., a low tendency to deceive. © 2017 S. Karger AG, Basel.

  20. Political crisіs and compromise as the casual determinants in the process of political decisions making

    Directory of Open Access Journals (Sweden)

    Shkuro Anton Sergiyovich

    2013-12-01

    Full Text Available There is a risk of certain ineffective results in the system of political decisions making. That’s why we should pay a special attention to the criteria of political crisis evaluation. Today the problem of national politics lies only in an adequate raising of questions about the way out of the political crisis using the mechanism of compromise achieving. The article is about the fundamental role of the crisis and the compromise and about their influence on the formation of the strategic development of policy.

  1. Establishment of normal gut microbiota is compromised under excessive hygiene conditions.

    Directory of Open Access Journals (Sweden)

    Bettina Schmidt

    Full Text Available BACKGROUND: Early gut colonization events are purported to have a major impact on the incidence of infectious, inflammatory and autoimmune diseases in later life. Hence, factors which influence this process may have important implications for both human and animal health. Previously, we demonstrated strong influences of early-life environment on gut microbiota composition in adult pigs. Here, we sought to further investigate the impact of limiting microbial exposure during early life on the development of the pig gut microbiota. METHODOLOGY/PRINCIPAL FINDINGS: Outdoor- and indoor-reared animals, exposed to the microbiota in their natural rearing environment for the first two days of life, were transferred to an isolator facility and adult gut microbial diversity was analyzed by 16S rRNA gene sequencing. From a total of 2,196 high-quality 16S rRNA gene sequences, 440 phylotypes were identified in the outdoor group and 431 phylotypes in the indoor group. The majority of clones were assigned to the four phyla Firmicutes (67.5% of all sequences, Proteobacteria (17.7%, Bacteroidetes (13.5% and to a lesser extent, Actinobacteria (0.1%. Although the initial maternal and environmental microbial inoculum of isolator-reared animals was identical to that of their naturally-reared littermates, the microbial succession and stabilization events reported previously in naturally-reared outdoor animals did not occur. In contrast, the gut microbiota of isolator-reared animals remained highly diverse containing a large number of distinct phylotypes. CONCLUSIONS/SIGNIFICANCE: The results documented here indicate that establishment and development of the normal gut microbiota requires continuous microbial exposure during the early stages of life and this process is compromised under conditions of excessive hygiene.

  2. The subpolar North Atlantic - Response to North Atlantic oscillation like forcing and Influence on the Atlantic meridional overturning circulation

    Science.gov (United States)

    Lohmann, Katja; Drange, Helge; Jungclaus, Johann

    2010-05-01

    The extent and strength of the North Atlantic subpolar gyre (SPG) changed rapidly in the mid-1990s, going from large and strong in 1995 to substantially weakened in the following years. The abrupt change in the intensity of the SPG is commonly linked to the reversal of the North Atlantic Oscillation (NAO) index, changing from strong positive to negative values, in the winter 1995/96. In this study we investigate the impact of the initial SPG state on its subsequent behavior by means of an ocean general circulation model driven by NCEP-NCAR reanalysis fields. Our sensitivity integrations suggest that the weakening of the SPG cannot be explained by the change in the atmospheric forcing alone. Rather, for the time period around 1995, the SPG was about to weaken, irrespective of the actual atmospheric forcing, due to the ocean state governed by the persistently strong positive NAO during the preceding seven years (1989 to 1995). Our analysis indicates that it was this preconditioning of the ocean, in combination with the sudden drop in the NAO in 1995/96, that lead to the strong and rapid weakening of the SPG in the second half of the 1990s. In the second part, the sensitivity of the low-frequency variability of the Atlantic meridional overturning circulation to changes in the subpolar North Atlantic is investigated using a 2000 year long control integration as well as sensitivity experiments with the MPI-M Earth System Model. Two 1000 year long sensitivity experiments will be performed, in which the low-frequency variability in the overflow transports from the Nordic Seas and in the subpolar deep water formation rates is suppressed respectively. This is achieved by nudging temperature and salinity in the GIN Sea or in the subpolar North Atlantic (up to about 1500m depth) towards a monthly climatology obtained from the last 1000 years of the control integration.

  3. Teste de Bender com disléxicos: comparação de dois sistemas de pontuação Bender Test with dyslexics: Comparison of two systems of punctuation

    Directory of Open Access Journals (Sweden)

    Acácia Aparecida Angeli dos Santos

    2007-06-01

    Full Text Available Este estudo teve como objetivo avaliar aspectos maturacionais e disfuncionais referentes à percepção visomotora de disléxicos valendo-se, para isso, do Teste de Bender analisado sob dois sistemas de correção, o sistema de pontuação gradual (B-SPG e o sistema Lacks. Participaram da pesquisa 20 disléxicos com idade entre 9 e 16 anos (M = 12, sendo 16 do sexo masculino e 4 do sexo feminino. Os dados mostraram que a média de erros dos disléxicos foi acima do que é esperado para crianças de nove e dez anos que compõem a amostra normativa do B-SPG. De acordo com o sistema Lacks de pontuação os fatores mais comprometidos na amostra de disléxicos foram referentes a mudanças na forma da gestalt e distorção da gestalt, fatores esses equivalentes à distorção da forma no B-SPG. O índice de correlação entre os dois sistemas foi significativo e alto (r = 0,76.The present study aimed to evaluate maturity and dysfunctional aspects referred to visualmotor perception of dyslexics using for this, the Bender Test analyzed under two correction systems, being the gradual scoring system (B-SPG and Lacks system. Twenty dyslexics have taken part in the research with ages from 9 to 16 years (average=12, being 16 males and 4 females. The data showed the average of mistakes committed by the dyslexics was above the expected for children from nine to ten years who compose the B-SPG normative sample. According to the Lacks scoring system the factors more affected in the sample of dyslexics were referred to changes on the gestalt form and distortions, factors which are equivalent to the form distortions on the B-SPG. The correlation index between these two systems was significant (r = 0.76.

  4. The role of dentists on medically compromised children’s oral and dental prophylaxis in Hospital

    Directory of Open Access Journals (Sweden)

    Roosje Rosita Oewen

    2008-12-01

    Full Text Available Background: One of dentist’s main roles is to coordinate the management of medically compromised children. The term of medically compromised refers to those children who have medical conditions which affect the dental treatment or manifest as a specific oral and dental problem. Patient’s visit to Special Care for Dentistry Clinic Dr. Hasan Sadikin Hospital Bandung showed a remarkable increase. From under 10 new visit in 2003, now July–December 2008 showed 81 new visit. Purpose: This paper discusses several medical problems (cardiovascular, hematology, respiratory system, and genetic disorder in children and the role of dentist in the treatment of those patients in the hospital. Review: The increase of attention by all level to these medically compromised children in the hospital also increases the dentist’s role in supporting the prognosis of the disease and patient’s quality of life. The most important effort is oral and dental prophylaxis to prevent oral pathology which is caused by the manifestation of disease as well as the side effects of treatment. Conclusion: It is, concluded that role of the dentist in managing these patients is giving preventive efforts and dental treatment that may be improve patient’s quality of life. The preventive effort and dental treatment is customize according to the patients condition. Nevertheless, cooperation from the dentist and other professional is needed in treating these patients.

  5. Mitigating Reptile Road Mortality: Fence Failures Compromise Ecopassage Effectiveness

    Science.gov (United States)

    Baxter-Gilbert, James H.; Riley, Julia L.; Lesbarrères, David; Litzgus, Jacqueline D.

    2015-01-01

    Roadways pose serious threats to animal populations. The installation of roadway mitigation measures is becoming increasingly common, yet studies that rigorously evaluate the effectiveness of these conservation tools remain rare. A highway expansion project in Ontario, Canada included exclusion fencing and ecopassages as mitigation measures designed to offset detrimental effects to one of the most imperial groups of vertebrates, reptiles. Taking a multispecies approach, we used a Before-After-Control-Impact study design to compare reptile abundance on the highway before and after mitigation at an Impact site and a Control site from 1 May to 31 August in 2012 and 2013. During this time, radio telemetry, wildlife cameras, and an automated PIT-tag reading system were used to monitor reptile movements and use of ecopassages. Additionally, a willingness to utilize experiment was conducted to quantify turtle behavioral responses to ecopassages. We found no difference in abundance of turtles on the road between the un-mitigated and mitigated highways, and an increase in the percentage of both snakes and turtles detected dead on the road post-mitigation, suggesting that the fencing was not effective. Although ecopassages were used by reptiles, the number of crossings through ecopassages was lower than road-surface crossings. Furthermore, turtle willingness to use ecopassages was lower than that reported in previous arena studies, suggesting that effectiveness of ecopassages may be compromised when alternative crossing options are available (e.g., through holes in exclusion structures). Our rigorous evaluation of reptile roadway mitigation demonstrated that when exclusion structures fail, the effectiveness of population connectivity structures is compromised. Our project emphasizes the need to design mitigation measures with the biology and behavior of the target species in mind, to implement mitigation designs in a rigorous fashion, and quantitatively evaluate road

  6. Mitigating reptile road mortality: fence failures compromise ecopassage effectiveness.

    Directory of Open Access Journals (Sweden)

    James H Baxter-Gilbert

    Full Text Available Roadways pose serious threats to animal populations. The installation of roadway mitigation measures is becoming increasingly common, yet studies that rigorously evaluate the effectiveness of these conservation tools remain rare. A highway expansion project in Ontario, Canada included exclusion fencing and ecopassages as mitigation measures designed to offset detrimental effects to one of the most imperial groups of vertebrates, reptiles. Taking a multispecies approach, we used a Before-After-Control-Impact study design to compare reptile abundance on the highway before and after mitigation at an Impact site and a Control site from 1 May to 31 August in 2012 and 2013. During this time, radio telemetry, wildlife cameras, and an automated PIT-tag reading system were used to monitor reptile movements and use of ecopassages. Additionally, a willingness to utilize experiment was conducted to quantify turtle behavioral responses to ecopassages. We found no difference in abundance of turtles on the road between the un-mitigated and mitigated highways, and an increase in the percentage of both snakes and turtles detected dead on the road post-mitigation, suggesting that the fencing was not effective. Although ecopassages were used by reptiles, the number of crossings through ecopassages was lower than road-surface crossings. Furthermore, turtle willingness to use ecopassages was lower than that reported in previous arena studies, suggesting that effectiveness of ecopassages may be compromised when alternative crossing options are available (e.g., through holes in exclusion structures. Our rigorous evaluation of reptile roadway mitigation demonstrated that when exclusion structures fail, the effectiveness of population connectivity structures is compromised. Our project emphasizes the need to design mitigation measures with the biology and behavior of the target species in mind, to implement mitigation designs in a rigorous fashion, and quantitatively

  7. Study of flood defense structural measures priorities using Compromise Programming technique

    Science.gov (United States)

    Lim, D.; Jeong, S.

    2017-12-01

    Recent climate change of global warming has led to the frequent occurrence of heavy regional rainfalls. As such, inundation vulnerability increases in urban areas with high population density due to the low runoff carrying capacity. This study selects a sample area (Janghang-eup, the Republic of Korea), which is one of the most vulnerable areas to flooding, analyzing the urban flood runoff model (XP-SWMM) and using the MCDM (Multi-Criteria Decision Making) technique to establish flood protection structural measures. To this end, we compare the alternatives and choose the optimal flood defense measure: our model is utilized with three flood prevention structural measures; (i) drainage pipe construction; (ii) water detention; and (iii) flood pumping station. Dividing the target area into three small basins, we propose flood evaluations for an inundation decrease by studying the flooded area, the maximum inundation depth, the damaged residential area, and the construction cost. In addition, Compromise Programming determines the priority of the alternatives. As a consequent, this study suggests flood pumping station for Zone 1 and drainage pipe construction for Zone 2 and Zone 3, respectively, as the optimal flood defense alternative. Keywords : MCDM; Compromise Programming; Urban Flood Prevention; This research was supported by a grant [MPSS-DP-2013-62] through the Disaster and Safety Management Institute funded by Ministry of Public Safety and Security of Korean government.

  8. Long-term efficacy of a double-blind, placebo-controlled, randomized study for repetitive sphenopalatine blockade with bupivacaine vs. saline with the Tx360 device for treatment of chronic migraine.

    Science.gov (United States)

    Cady, Roger K; Saper, Joel; Dexter, Kent; Cady, Ryan J; Manley, Heather R

    2015-04-01

    number of headache days 1 month post-treatment (Mdiff  = -5.71), whereas those receiving saline only saw a slight improvement (Mdiff  = -1.93). Headache Impact Test 6 scores were decreased in the bupivacaine group at 1 month (Mdiff  = -5.13) and 6 months (Mdiff  = -4.78) post-treatment, but only a modest reduction was seen for those receiving saline at 1 and 6 months, respectively (Mdiff  = -2.08, Mdiff  = -1.58). Furthermore, subjects receiving bupivacaine reported a reduction in acute medication usage and improved quality of life measures (average pain in the previous 24 hours, mood, normal work, and general activity) up to 6 months post-treatment. The changes in these measures for the saline group were minimal. Data from this exploratory pilot study suggest that there may be long-term clinical benefits with the use of repetitive SPG blockades with bupivacaine delivered with the simple to use Tx360 device. These include a sustained reduction of headache days and improvement in several important quality of life assessments. The SPG blockades were not associated with any significant or lasting adverse events. Further research on SPG blockade is warranted. © 2015 The Authors. Headache published by Wiley Periodicals, Inc. on behalf of American Headache Society.

  9. Structure of ganglioside with CAD blood group antigen activity

    International Nuclear Information System (INIS)

    Gillard, B.K.; Blanchard, D.; Cartron, J.P.; van Kuik, G.A.; Vliegenthart, J.F.G.; Marcus, D.M.

    1986-01-01

    The novel erythrocyte ganglioside which carries the blood group Cad determinant has been isolated, and its structure has been determined. The ganglioside contained Glu:Gal:GalNAc:GlcNAc in a molar ratio of 1.00:1.94:0.93:0.95. The ganglioside binds Helix pomatia lectin and its chromatographic mobility is similar to G/sub D3/. After treatment with β-hexosaminidase (human placenta HexA) the product migrated with sialosylparagloboside (SPG), no longer binds Helix lectin, and binds a human anti-SPG antibody. Treatment of this material with neuraminidase (V. cholera) yielded a product with the mobility of paragloboside that bound monoclonal antibody 1B2. NMR analysis revealed that the terminal GalNAc is linked β1-4 to Gal, and confirms the structure proposed previously: GalNAcβ1-4(NeuAcα2-3)Galβ1-4GlcNAcβ1-3Galβ1-4Glc-Cer. This structure is consistent with the previous demonstration that a compound with the same chromatographic mobility as the Cad ganglioside could be synthesized by enzymatic transfer of GalNAc to sialosylparagloboside

  10. Encapsulated eucalyptus oil in ionically cross-linked alginate microcapsules and its controlled release.

    Science.gov (United States)

    Noppakundilograt, Supaporn; Piboon, Phianghathai; Graisuwan, Wilaiporn; Nuisin, Roongkan; Kiatkamjornwong, Suda

    2015-10-20

    Sodium alginate microcapsules containing eucalyptus oil were prepared by oil-in-water emulsification via Shirasu porous glass (SPG) membrane and cross-linked by calcium chloride (CaCl2). SPG membrane pore size of 5.2μm was used to control the size of eucalyptus oil microdroplets. Effects of sodium alginate, having a mannuronic acid/guluronic acid (M/G) ratio of 1.13, eucalyptus oil and CaCl2 amounts on microdroplet sizes and size distribution were elucidated. Increasing sodium alginate amounts from 0.1 to 0.5% (wv(-1)) sodium alginate, the average droplets size increased from 42.2±2.0 to 48.5±0.6μm, with CVs of 16.5±2.2 and 30.2±4.5%, respectively. CaCl2 successfully gave narrower size distribution of cross-linked eucalyptus oil microcapsules. The optimum conditions for preparing the microcapsules, oil loading efficiency, and controlled release of the encapsulated eucalyptus oil from the microcapsules as a function of time at 40°C were investigated. Release model for the oil from microcapsules fitted Ritger-Peppas model with non-Fickian transport mechanism. Copyright © 2015 Elsevier Ltd. All rights reserved.

  11. The Swiss approach to finding compromises in nuclear waste governance

    Energy Technology Data Exchange (ETDEWEB)

    Kuppler, Sophie; Grunwald, Armin [Karlsruhe Institute of Technology (Germany). Inst. for Technology Assessment and Systems Analysis

    2015-07-01

    In Switzerland, a new site selection procedure is being implemented since 2008. This procedure, which is laid down in a 'sectoral plan', shows strong elements of public participation and transparency and can be considered a step away from the classical 'decide-announce-defend' approach in decision-making. This procedure tends towards a more governance-oriented approach based on ideas of 'civility' of decision-making. Despite this renewal, the Swiss case clearly shows that any kind of selection process has to be considered as a 'working compromise', which needs to be adapted when new challenges emerge.

  12. The Swiss approach to finding compromises in nuclear waste governance

    International Nuclear Information System (INIS)

    Kuppler, Sophie; Grunwald, Armin

    2015-01-01

    In Switzerland, a new site selection procedure is being implemented since 2008. This procedure, which is laid down in a 'sectoral plan', shows strong elements of public participation and transparency and can be considered a step away from the classical 'decide-announce-defend' approach in decision-making. This procedure tends towards a more governance-oriented approach based on ideas of 'civility' of decision-making. Despite this renewal, the Swiss case clearly shows that any kind of selection process has to be considered as a 'working compromise', which needs to be adapted when new challenges emerge.

  13. Understanding Youth's Health-Compromising Behaviors in Germany: An Application of the Risk-Behavior Framework.

    Science.gov (United States)

    Hazard, Barbara P.; Lee, Che-Fu

    1999-01-01

    Analyzed the health-compromising behaviors of German youth using responses of 2,330 seventh, eighth, and ninth graders from the German Youth Study. Smoking and drinking are not seen by these students as health-threatening behaviors, but as socially appealing behaviors. Discusses implications for health education. (SLD)

  14. Resale Price Maintenance Under the Hong Kong Competition Ordinance—An Uneasy Compromise

    OpenAIRE

    Mark Jephcott; Adelaide Luke; Lisa Geary; Molly Herron

    2015-01-01

    However, the compromise position reached in the Guideline provides relatively little in terms of legal certainty as to the circumstances in which RPM may be acceptable. Mark Jephcott, Adelaide Luke, Lisa Geary, & Molly Herron (Herbert Smith Freehills)

  15. Conflicting perspectives compromising discussions on cardiopulmonary resuscitation.

    LENUS (Irish Health Repository)

    Groarke, J

    2010-09-01

    Healthcare professionals, patients and their relatives are expected to discuss resuscitation together. This study aims to identify the differences in the knowledge base and understanding of these parties. Questionnaires examining knowledge and opinion on resuscitation matters were completed during interviews of randomly selected doctors, nurses and the general public. 70% doctors, 24% nurses and 0% of a public group correctly estimated survival to discharge following in-hospital resuscitation attempts. Deficiencies were identified in doctor and nurse knowledge of ethics governing resuscitation decisions. Public opinion often conflicts with ethical guidelines. Public understanding of the nature of cardiopulmonary arrests and resuscitation attempts; and of the implications of a \\'Do Not Attempt Resuscitation (DNAR)\\' order is poor. Television medical dramas are the primary source of resuscitation knowledge. Deficiencies in healthcare professionals\\' knowledge of resuscitation ethics and outcomes may compromise resuscitation decisions. Educational initiatives to address deficiencies are necessary. Parties involved in discussion on resuscitation do not share the same knowledge base reducing the likelihood of meaningful discussion. Public misapprehensions surrounding resuscitation must be identified and corrected during discussion.

  16. 76 FR 17843 - Intent To Compromise Claim Against the District of Columbia Public Schools

    Science.gov (United States)

    2011-03-31

    ... District of Columbia Public Schools (DCPS) now pending before the Office of Administrative Law Judges (OALJ... DEPARTMENT OF EDUCATION Intent To Compromise Claim Against the District of Columbia Public Schools... Application for Review of this PDL with the Office of Administrative Law Judges (OALJ) on September 10, 2007...

  17. Saint Petersburg magnetic observatory: from Voeikovo subdivision to INTERMAGNET certification

    Science.gov (United States)

    Sidorov, Roman; Soloviev, Anatoly; Krasnoperov, Roman; Kudin, Dmitry; Grudnev, Andrei; Kopytenko, Yury; Kotikov, Andrei; Sergushin, Pavel

    2017-11-01

    Since June 2012 the Saint Petersburg magnetic observatory is being developed and maintained by two institutions of the Russian Academy of Sciences (RAS) - the Geophysical Center of RAS (GC RAS) and the Saint Petersburg branch of the Pushkov Institute of Terrestrial Magnetism, Ionosphere and Radio Wave Propagation of RAS (IZMIRAN SPb). On 29 April 2016 the application of the Saint Petersburg observatory (IAGA code SPG) for introduction into the INTERMAGNET network was accepted after approval by the experts of the first definitive dataset over 2015, produced by the GC RAS, and on 9 June 2016 the SPG observatory was officially certified. One of the oldest series of magnetic observations, originating in 1834, was resumed in the 21st century, meeting the highest quality standards and all modern technical requirements. In this paper a brief historical and scientific background of the SPG observatory foundation and development is given, the stages of its renovation and upgrade in the 21st century are described, and information on its current state is provided. The first results of the observatory functioning are discussed and geomagnetic variations registered at the SPG observatory are assessed and compared with geomagnetic data from the INTERMAGNET observatories located in the same region.

  18. Saint Petersburg magnetic observatory: from Voeikovo subdivision to INTERMAGNET certification

    Directory of Open Access Journals (Sweden)

    R. Sidorov

    2017-11-01

    Full Text Available Since June 2012 the Saint Petersburg magnetic observatory is being developed and maintained by two institutions of the Russian Academy of Sciences (RAS – the Geophysical Center of RAS (GC RAS and the Saint Petersburg branch of the Pushkov Institute of Terrestrial Magnetism, Ionosphere and Radio Wave Propagation of RAS (IZMIRAN SPb. On 29 April 2016 the application of the Saint Petersburg observatory (IAGA code SPG for introduction into the INTERMAGNET network was accepted after approval by the experts of the first definitive dataset over 2015, produced by the GC RAS, and on 9 June 2016 the SPG observatory was officially certified. One of the oldest series of magnetic observations, originating in 1834, was resumed in the 21st century, meeting the highest quality standards and all modern technical requirements. In this paper a brief historical and scientific background of the SPG observatory foundation and development is given, the stages of its renovation and upgrade in the 21st century are described, and information on its current state is provided. The first results of the observatory functioning are discussed and geomagnetic variations registered at the SPG observatory are assessed and compared with geomagnetic data from the INTERMAGNET observatories located in the same region.

  19. Small intestine epithelial barrier function is compromised in pigs with low feed intake at weaning.

    NARCIS (Netherlands)

    Spreeuwenberg, M.A.; Verdonk, J.M.; Gaskins, H.R.; Verstegen, M.W.A.

    2001-01-01

    Compromising alterations in gastrointestinal architecture are common during the weaning transition of pigs. The relation between villous atrophy and epithelial barrier function at weaning is not well understood. This study evaluated in vitro transepithelial transport by Ussing metabolic chambers,

  20. Sarcoidosis with Major Airway, Vascular and Nerve Compromise

    Directory of Open Access Journals (Sweden)

    Hiroshi Sekiguchi

    2013-01-01

    Full Text Available The present report describes a 60-year-old Caucasian woman who presented with progressive dyspnea, cough and wheeze. A computed tomography scan of the chest showed innumerable bilateral inflammatory pulmonary nodules with bronchovascular distribution and a mediastinal and hilar infiltrative process with calcified lymphadenopathy leading to narrowing of lobar bronchi and pulmonary arteries. An echocardiogram revealed pulmonary hypertension. Bronchoscopy showed left vocal cord paralysis and significant narrowing of the bilateral bronchi with mucosal thickening and multiple nodules. Transbronchial biopsy was compatible with sarcoidosis. Despite balloon angioplasty of the left lower lobe and pulmonary artery, and medical therapy with oral corticosteroids, her symptoms did not significantly improve. To the authors’ knowledge, the present report describes the first case of pulmonary sarcoidosis resulting in major airway, vascular and nerve compromise due to compressive lymphadenopathy and suspected concurrent granulomatous infiltration. Its presentation mimicked idiopathic mediastinal fibrosis.

  1. Advanced medical life support procedures in vitally compromised children by a helicopter emergency medical service.

    NARCIS (Netherlands)

    Gerritse, B.M.; Schalkwijk, A.; Pelzer, B.J.; Scheffer, G.J.; Draaisma, J.M.T.

    2010-01-01

    BACKGROUND: To determine the advanced life support procedures provided by an Emergency Medical Service (EMS) and a Helicopter Emergency Medical Service (HEMS) for vitally compromised children. Incidence and success rate of several procedures were studied, with a distinction made between procedures

  2. 28 CFR 0.163 - Approval by Solicitor General of action on compromise offers in certain cases.

    Science.gov (United States)

    2010-07-01

    ... advice from the Solicitor General that the principles of law involved do not require appellate review in... JUSTICE ORGANIZATION OF THE DEPARTMENT OF JUSTICE Authority To Compromise and Close Civil Claims and...

  3. A Pirate too Needs to be Heard: Procedural Compromises in Online Copyright Infringement Cases in the UK

    Directory of Open Access Journals (Sweden)

    Poorna Mysoor

    2014-08-01

    Full Text Available Online copyright infringement is one of the toughest challenges the content industry has faced in the twenty first century. Article 8(3 of the Information Society Directive, implemented under section 97A of the UK Copyright, Designs and Patents Act, 1988, provides an injunctive remedy in response to such online infringement. Given the elusive nature of the website operators and the sheer number of their users, rights owners have turned to the Internet Service Providers (ISPs to implement the injunctions granted under section 97A to block websites with infringing content. In their battle to keep pace with online infringers, the UK courts have permitted significant compromises to the procedures while granting these injunctive blocking orders. One of the pioneers in this line of cases in the UK is the Pirate Bay case (TPB case, which ruled as a matter of law that the procedure under Section 97A does not require the alleged infringers to be made parties to those proceedings. TPB case also marks the trend of the ISPs consenting to an order of injunction, and not defending the proceedings. This has resulted in the absence of any adverse party in the proceedings under section 97A. This threatens the basic tenets of procedural fairness and propriety, having adverse consequences on both the substantive and procedural aspects, evident in the cases that followed TPB case. This article examines the aspects of the judgment in TPB case that led to these procedural compromises, whether it had any basis in the pre-existing case law, what the nature of an injunction under section 97A is and how the subsequent line of cases have followed TPB case as a precedent. It further explores the adverse consequences of the procedural compromises and whether any safeguards are available against such compromises.

  4. Hydro-environmental management of groundwater resources: A fuzzy-based multi-objective compromise approach

    Science.gov (United States)

    Alizadeh, Mohammad Reza; Nikoo, Mohammad Reza; Rakhshandehroo, Gholam Reza

    2017-08-01

    Sustainable management of water resources necessitates close attention to social, economic and environmental aspects such as water quality and quantity concerns and potential conflicts. This study presents a new fuzzy-based multi-objective compromise methodology to determine the socio-optimal and sustainable policies for hydro-environmental management of groundwater resources, which simultaneously considers the conflicts and negotiation of involved stakeholders, uncertainties in decision makers' preferences, existing uncertainties in the groundwater parameters and groundwater quality and quantity issues. The fuzzy multi-objective simulation-optimization model is developed based on qualitative and quantitative groundwater simulation model (MODFLOW and MT3D), multi-objective optimization model (NSGA-II), Monte Carlo analysis and Fuzzy Transformation Method (FTM). Best compromise solutions (best management policies) on trade-off curves are determined using four different Fuzzy Social Choice (FSC) methods. Finally, a unanimity fallback bargaining method is utilized to suggest the most preferred FSC method. Kavar-Maharloo aquifer system in Fars, Iran, as a typical multi-stakeholder multi-objective real-world problem is considered to verify the proposed methodology. Results showed an effective performance of the framework for determining the most sustainable allocation policy in groundwater resource management.

  5. Methamphetamine compromises gap junctional communication in astrocytes and neurons.

    Science.gov (United States)

    Castellano, Paul; Nwagbo, Chisom; Martinez, Luis R; Eugenin, Eliseo A

    2016-05-01

    Methamphetamine (meth) is a central nervous system (CNS) stimulant that results in psychological and physical dependency. The long-term effects of meth within the CNS include neuronal plasticity changes, blood-brain barrier compromise, inflammation, electrical dysfunction, neuronal/glial toxicity, and an increased risk to infectious diseases including HIV. Most of the reported meth effects in the CNS are related to dysregulation of chemical synapses by altering the release and uptake of neurotransmitters, especially dopamine, norepinephrine, and epinephrine. However, little is known about the effects of meth on connexin (Cx) containing channels, such as gap junctions (GJ) and hemichannels (HC). We examined the effects of meth on Cx expression, function, and its role in NeuroAIDS. We found that meth altered Cx expression and localization, decreased GJ communication between neurons and astrocytes, and induced the opening of Cx43/Cx36 HC. Furthermore, we found that these changes in GJ and HC induced by meth treatment were mediated by activation of dopamine receptors, suggesting that dysregulation of dopamine signaling induced by meth is essential for GJ and HC compromise. Meth-induced changes in GJ and HC contributed to amplified CNS toxicity by dysregulating glutamate metabolism and increasing the susceptibility of neurons and astrocytes to bystander apoptosis induced by HIV. Together, our results indicate that connexin containing channels, GJ and HC, are essential in the pathogenesis of meth and increase the sensitivity of the CNS to HIV CNS disease. Methamphetamine (meth) is an extremely addictive central nervous system stimulant. Meth reduced gap junctional (GJ) communication by inducing internalization of connexin-43 (Cx43) in astrocytes and reducing expression of Cx36 in neurons by a mechanism involving activation of dopamine receptors (see cartoon). Meth-induced changes in Cx containing channels increased extracellular levels of glutamate and resulted in higher

  6. Clustering of health-compromising behavior and delinquency in adolescents and adults in the Dutch population

    NARCIS (Netherlands)

    van Nieuwenhuijzen, Maroesjka; Junger, Marianne; Klein Velderman, Mariska; Wiefferink, Karin H.; Paulussen, Theo W. G. M.; Hox, Joop; Reijneveld, Sijmen A.

    Objective. This study investigates the clustering of a broad range of health-compromising and delinquent behaviors. We examine whether these behaviors belong to a single but broad cluster. 'risk-taking behavior', and whether the nature and degree of clustering in adolescents differs from that in

  7. Towards mesoscience the principle of compromise in competition

    CERN Document Server

    Li, Jinghai

    2014-01-01

    This brief is devoted to providing a complete outline of meso-science by briefing the relevant contents from the published book and by updating evidences and concepts of meso-science. The importance of meso-science in solving various problems in energy, resource, and the environment is introduced.  The whole evolutionary development of the EMMS principle is reviewed to show how a simple idea on the customized modeling of particle clustering in gas-solid systems was developed, verified, extended, and finally generalized into the common principle of compromise in competition between dominant mechanisms for all mesoscale phenomena in science and engineering, leading to the proposition of meso-science. More importantly, updates on the concept of meso-science and perspectives are presented, along with new insights and findings from after the publication of the original book. In this way, we hope to help readers more easily familiarize themselves with meso-science, and to trigger interest and attention to this int...

  8. Estudio transcultural con la prueba de Bender: Sistema de puntuación gradual (Cross-Cultural Study with Bender Test- Gradual Scoring System

    Directory of Open Access Journals (Sweden)

    Fabián Javier Marín Rueda

    2013-12-01

    Full Text Available RESUMEN: El objetivo del presente estudio es comparar el desempeño en la Prueba Gestáltica Visomotriz de Bender - Sistema de Puntuación Gradual (B-SPG en un grupo de niños peruanos en función a los datos ofrecidos por el manual brasilero de la prueba. Participaron 82 niños, de ambos sexos, con edades entre los 8 y 10 años (M = 9.21, DT = 0.83. Los niños provenían de los distritos de Pueblo Libre (43.9% y Rímac (51.2%, de la provincia de Lima, así como también de Lima Metropolitana (4.9%. El B-SPG fue aplicado de forma colectiva. Los promedios de puntos obtenidos por los niños peruanos en el B-SPG fueron significativamente superiores a los obtenidos por los niños brasileros en cada una de las edades estudiadas. Se destaca la importancia de investigar evidencias de validez y de confiabilidad para que la prueba pueda ser usada de forma adecuada en el Perú, considerando las particularidades de desarrollo de los niños del país. ABSTRACT: The objective of the study is to compare performance on the Bender Visual Motor Gestalt test - system of Gradual punctuation (B-SPG in a group of Peruvian children in connection with the data provided by the Brazilian manual of the test. In this research 82 Peruvian children were involved, both sexes, with ages between 8 and 10 years (M = 9.21, DT = 0.83. The hildren came from the districts of Pueblo Libre (43.9% and Rimac (51.2% in the province of Lima, as well as from metropolitan Lima (4.9%. The B-SPG was collectively applied in the children’s schools. The average points earned by the Peruvian children in the B-SPG were significantly higher than those obtained by Brazilian children in each one of the ages studied. It emphasizes the importance of investigating evidence of validity and reliability, so that, the test can be used appropriately in Peru, considering the peculiarities of the development of children in the country.

  9. Mactosylceramide Prevents Glial Cell Overgrowth by Inhibiting Insulin and Fibroblast Growth Factor Receptor Signaling

    DEFF Research Database (Denmark)

    Gerdøe-Kristensen, Stine; Lund, Viktor K; Wandall, Hans H

    2017-01-01

    , in which the mannosyltransferase Egghead controls conversion of glucosylceramide (GlcCer) to mactosylceramide (MacCer). Lack of elongated GSL in egghead (egh) mutants causes overgrowth of subperineurial glia (SPG), largely due to aberrant activation of phosphatidylinositol 3-kinase (PI3K). However, to what...... of the Drosophila Insulin Receptor (InR) and the FGFR homolog Heartless (Htl) in wild type SPG, and is suppressed by inhibiting Htl and InR activity in egh. Knockdown of GlcCer synthase in the SPG fails to suppress glial overgrowth in egh nerves, and slightly promotes overgrowth in wild type, suggesting that RTK...... hyperactivation is caused by absence of MacCer and not by GlcCer accumulation. We conclude that an early product in GSL biosynthesis, MacCer, prevents inappropriate activation of Insulin and Fibroblast Growth Factor Receptors in Drosophila glia. This article is protected by copyright. All rights reserved....

  10. Comparative Accuracy of Facial Models Fabricated Using Traditional and 3D Imaging Techniques.

    Science.gov (United States)

    Lincoln, Ketu P; Sun, Albert Y T; Prihoda, Thomas J; Sutton, Alan J

    2016-04-01

    The purpose of this investigation was to compare the accuracy of facial models fabricated using facial moulage impression methods to the three-dimensional printed (3DP) fabrication methods using soft tissue images obtained from cone beam computed tomography (CBCT) and 3D stereophotogrammetry (3D-SPG) scans. A reference phantom model was fabricated using a 3D-SPG image of a human control form with ten fiducial markers placed on common anthropometric landmarks. This image was converted into the investigation control phantom model (CPM) using 3DP methods. The CPM was attached to a camera tripod for ease of image capture. Three CBCT and three 3D-SPG images of the CPM were captured. The DICOM and STL files from the three 3dMD and three CBCT were imported to the 3DP, and six testing models were made. Reversible hydrocolloid and dental stone were used to make three facial moulages of the CPM, and the impressions/casts were poured in type IV gypsum dental stone. A coordinate measuring machine (CMM) was used to measure the distances between each of the ten fiducial markers. Each measurement was made using one point as a static reference to the other nine points. The same measuring procedures were accomplished on all specimens. All measurements were compared between specimens and the control. The data were analyzed using ANOVA and Tukey pairwise comparison of the raters, methods, and fiducial markers. The ANOVA multiple comparisons showed significant difference among the three methods (p 3D-SPG showed statistical difference in comparison to the models fabricated using the traditional method of facial moulage and 3DP models fabricated from CBCT imaging. 3DP models fabricated using 3D-SPG were less accurate than the CPM and models fabricated using facial moulage and CBCT imaging techniques. © 2015 by the American College of Prosthodontists.

  11. 'Compromise position' image alignment to accommodate independent motion of multiple clinical target volumes during radiotherapy: A high risk prostate cancer example.

    Science.gov (United States)

    Rosewall, Tara; Yan, Jing; Alasti, Hamideh; Cerase, Carla; Bayley, Andrew

    2017-04-01

    Inclusion of multiple independently moving clinical target volumes (CTVs) in the irradiated volume causes an image guidance conundrum. The purpose of this research was to use high risk prostate cancer as a clinical example to evaluate a 'compromise' image alignment strategy. The daily pre-treatment orthogonal EPI for 14 consecutive patients were included in this analysis. Image matching was performed by aligning to the prostate only, the bony pelvis only and using the 'compromise' strategy. Residual CTV surrogate displacements were quantified for each of the alignment strategies. Analysis of the 388 daily fractions indicated surrogate displacements were well-correlated in all directions (r 2  = 0.95 (LR), 0.67 (AP) and 0.59 (SI). Differences between the surrogates displacements (95% range) were -0.4 to 1.8 mm (LR), -1.2 to 5.2 mm (SI) and -1.2 to 5.2 mm (AP). The distribution of the residual displacements was significantly smaller using the 'compromise' strategy, compared to the other strategies (p 0.005). The 'compromise' strategy ensured the CTV was encompassed by the PTV in all fractions, compared to 47 PTV violations when aligned to prostate only. This study demonstrated the feasibility of a compromise position image guidance strategy to accommodate simultaneous displacements of two independently moving CTVs. Application of this strategy was facilitated by correlation between the CTV displacements and resulted in no geometric excursions of the CTVs beyond standard sized PTVs. This simple image guidance strategy may also be applicable to other disease sites that concurrently irradiate multiple CTVs, such as head and neck, lung and cervix cancer. © 2016 The Royal Australian and New Zealand College of Radiologists.

  12. National Report presented by the Mexican United States to satisfy the compromises of the Nuclear Safety Convention

    International Nuclear Information System (INIS)

    1998-01-01

    In order to satisfy to the compromises derived of the ratification by part of the Mexican Government for the Nuclear Safety Convention it is presented this National Report which is based on the directives proposed as a result of the preparatory meetings held in the IAEA Headquarters in the city of Vienna, Austria. This National Report represents a document summary and activities realized at present in relation with the only nuclear facility in Mexico: the Nuclear Power Plant in Laguna Verde, Veracruz. This report consists of two parts: In the first one it is described how have been satisfied each one of the compromises. The second one talks about the Laws and Regulations on nuclear activities in the country. (Author)

  13. Compromises produced by the dialectic between self-verification and self-enhancement.

    Science.gov (United States)

    Morling, B; Epstein, S

    1997-12-01

    Three studies of people's reactions to evaluative feedback demonstrated that the dialectic between self-enhancement and self-verification results in compromises between these 2 motives, as hypothesized in cognitive-experiential self-theory. The demonstration was facilitated by 2 procedural improvements: Enhancement and verification were established by calibrating evaluative feedback against self appraisals, and degree of enhancement and of verification were varied along a continuum, rather than categorically. There was also support for the hypotheses that processing in an intuitive-experiential mode favors enhancement and processing in an analytical-rational mode favors verification in the kinds of situations investigated.

  14. Effect of exoskeletal joint constraint and passive resistance on metabolic energy expenditure: Implications for walking in paraplegia.

    Directory of Open Access Journals (Sweden)

    Sarah R Chang

    Full Text Available An important consideration in the design of a practical system to restore walking in individuals with spinal cord injury is to minimize metabolic energy demand on the user. In this study, the effects of exoskeletal constraints on metabolic energy expenditure were evaluated in able-bodied volunteers to gain insight into the demands of walking with a hybrid neuroprosthesis after paralysis. The exoskeleton had a hydraulic mechanism to reciprocally couple hip flexion and extension, unlocked hydraulic stance controlled knee mechanisms, and ankles fixed at neutral by ankle-foot orthoses. These mechanisms added passive resistance to the hip (15 Nm and knee (6 Nm joints while the exoskeleton constrained joint motion to the sagittal plane. The average oxygen consumption when walking with the exoskeleton was 22.5 ± 3.4 ml O2/min/kg as compared to 11.7 ± 2.0 ml O2/min/kg when walking without the exoskeleton at a comparable speed. The heart rate and physiological cost index with the exoskeleton were at least 30% and 4.3 times higher, respectively, than walking without it. The maximum average speed achieved with the exoskeleton was 1.2 ± 0.2 m/s, at a cadence of 104 ± 11 steps/min, and step length of 70 ± 7 cm. Average peak hip joint angles (25 ± 7° were within normal range, while average peak knee joint angles (40 ± 8° were less than normal. Both hip and knee angular velocities were reduced with the exoskeleton as compared to normal. While the walking speed achieved with the exoskeleton could be sufficient for community ambulation, metabolic energy expenditure was significantly increased and unsustainable for such activities. This suggests that passive resistance, constraining leg motion to the sagittal plane, reciprocally coupling the hip joints, and weight of exoskeleton place considerable limitations on the utility of the device and need to be minimized in future designs of practical hybrid neuroprostheses for walking after paraplegia.

  15. Smoking Habit in Severe Obese after bariatric procedures.

    Science.gov (United States)

    Maniscalco, Mauro; Carratù, Pierluigi; Faraone, Stanislao; Cerbone, Maria Rosaria; Cristiano, Stefano; Zedda, Anna; Resta, Onofrio

    2015-01-01

    Bariatric procedures provide an effective means of short term weight loss and sustained weight control for the morbidly obese. The effect of bariatric procedures on smoking habit in obese subjects is not well known. Therefore, we examined the short term effect of bariatric surgery on smoking habit of severe obese patients up to 12 months from the intervention. Smoking habit was assessed in a cohort of 78 morbid smoking obese patients followed at our clinic for bariatric procedures. They underwent non surgical intra-gastric balloon (IB) or surgical procedures such as lap-band laparoscopic surgery (LAGB) or sleeve gastrectomy/gastric by-pass (SPG). Subjects were administered a written questionnaire about their smoking habit before and 3, 6 and 12 months after the procedures. No differences were found among the three groups at 6 and 12 months after the procedures (IB 21 %, LAGB 6 %, SPG 5 %; and IB 14 %, LAGB 3 %, SPG 5 %). Only after 3 months, the rate of quitting of the IB group was higher than LAGB and SPG groups (36 %, 6 % and 5 %, respectively; p = 0.02). Bariatric procedures have no effects on smoking habit of moderate-to-heavy smoker severe obese patients. The use of other traditional smoking cessation methods in patients undergone to bariatric procedures should be implemented.

  16. Chiral recognition of phenylglycinol enantiomers based on N-acetyl-L-cysteine capped CdTe quantum dots in the presence of Ag+

    Science.gov (United States)

    Guo, Yuan; Zeng, Xiaoqing; Yuan, Haiyan; Huang, Yunmei; Zhao, Yanmei; Wu, Huan; Yang, Jidong

    2017-08-01

    In this study, a novel method for chiral recognition of phenylglycinol (PG) enantiomers was proposed. Firstly, water-soluble N-acetyl-L-cysteine (NALC)-capped CdTe quantum dots (QDs) were synthesized and experiment showed that the fluorescence intensity of the reaction system slightly enhancement when added PG enantiomers to NALC-capped CdTe quantum dots (QDs), but the R-PG and S-PG could not be distinguished. Secondly, when there was Ag+ presence in the reaction system, the experiment result was extremely interesting, the PG enantiomers cloud make NALC-capped CdTe QDs produce different fluorescence signal, in which the fluorescence of S-PG + Ag+ + NALC-CdTe system was significantly enhanced, and the fluorescence of R-PG + Ag+ + NALC-CdTe system was markedly decreased. Thirdly, all the enhanced and decreased of the fluorescence intensity were directly proportional to the concentration of R-PG and S-PG in the linearly range 10- 5-10- 7 mol·L- 1, respectively. So, the new method for simultaneous determination of the PG enantiomers was built too. The experiment result of the method was satisfactory with the detection limit of PG can reached 10- 7 mol·L- 1 and the related coefficient of S-PG and R-PG are 0.995 and 0.980, respectively. The method was highly sensitive, selective and had wider detection range compared with other methods.

  17. Lower Extremity Salvage with Thoracodorsal Artery Perforator Free Flap in Condition of Symmetrical Peripheral Gangrene

    Directory of Open Access Journals (Sweden)

    Soo Yeon Lim

    2018-01-01

    Full Text Available Symmetrical peripheral gangrene (SPG is rare but devastating complication which is characterized by symmetrical ischemic change of the distal extremities. In this report, we describe our management protocol for SPG, focusing on surgical approaches. Between January 2007 and February 2016, 10 thoracodorsal artery perforator (TDAP free flaps were performed in 6 patients with SPG. Three patients were male and mean age was 56 (range, 44–69 years. All the patients were in shock. The causes of shock were sepsis in 4 cases, respiratory arrest in 1 case, and hypovolemia in 1 case. Eight transmetatarsal amputations and 2 Lisfranc amputations were performed. Flap sizes ranged from 7 × 11 cm to 25 × 15 cm. There were 3 cases of partial necrosis of the flap: two healed conservatively with dressings and one required skin graft. Three of the patients were later able to walk independently at Functional Ambulation Classification (FAC level 6, one patient could walk independently on level surfaces at FAC level 5, and 2 could walk independently using walking aids, classified at FAC level 4. The average follow-up period was 18 (range, 6–54 months. In patients with SPG, minimal bone amputation and foot salvage with TDAP flaps were successful. Separate reconstruction of bone and soft tissue had good outcomes.

  18. Growth of CoSi2 on Si(001) by reactive deposition epitaxy

    International Nuclear Information System (INIS)

    Lim, C.W.; Shin, C.-S.; Gall, D.; Zuo, J.M.; Petrov, I.; Greene, J.E.

    2005-01-01

    CaF 2 -structure CoSi 2 layers were formed on Si(001) by reactive deposition epitaxy (RDE) and compared with CoSi 2 layers obtained by conventional solid phase growth (SPG). In both sets of experiments, Co was deposited by ultrahigh-vacuum magnetron sputtering and CoSi 2 formed at 600 deg. C. However, in the case of RDE, CoSi 2 formation occurred during Co deposition while for SPG, Co was deposited at 25 deg. C and silicidation took place during subsequent annealing. X-ray diffraction pole figures and transmission electron microscopy results demonstrate that RDE CoSi 2 layers are epitaxial with a cube-on-cube relationship (001) CoSi 2 parallel (001) Si and [100] CoSi 2 parallel[100] Si . In contrast, SPG films are polycrystalline with an average grain size of ≅1000 A and a mixed 111/002/022/112 orientation. We attribute the striking difference to rapid Co diffusion into the Si(001) substrate during RDE for which the high Co/Si reactivity gives rise to a flux-limited reaction resulting in the direct formation of the disilicide phase. In contrast, sequential nucleation and transformation among increasingly Si-rich phases--from orthorhombic Co 2 Si to cubic CoSi to CoSi 2 --during SPG results in polycrystalline layers with a complex texture

  19. Life-threatening systemic toxicity and airway compromise from a common European adder bite to the tongue

    DEFF Research Database (Denmark)

    Hoegberg, L C G; Jessen, C L; Lambertsen, K

    2009-01-01

    , pharyngeal and facial oedema compromised the airway, leading to respiratory failure, concomitant with circulatory failure related to hypoxaemia and systemic toxic effects. Acute tracheotomy secured the airway, and two doses of antivenom successfully treated the systemic, toxic effects. The reaction...

  20. Thiamine absorption is not compromised in folate-deficient rats

    International Nuclear Information System (INIS)

    Walzem, R.L.; Clifford, A.J.

    1988-01-01

    Thiamine absorption and excretion were assessed in rats with severe folate deficiency (FD) by determining the fate of oral 3 H-labeled and intravenous 14 C-labeled thiamine over a 6-h test period. Thiamine status was evaluated in these same rats by measuring transketolase activity levels of blood before (TKA) and after (TPPE) addition of thiamine pyrophosphate to the incubation mixture of the assay procedure. Two additional experiments assessed active transport of thiamine and the effect of dietary succinylsulfathiazole (SST) on TKA and TPPE in rats with moderate FD. Intestinal absorption in general and thiamine absorption in particular and thiamine status were unaltered in rats with severe FD. Inanition associated with severe FD may impair thiamine status. Thiamine absorption by active transport was not compromised in FD, and dietary succinylsulfathiazole did not affect thiamine status

  1. 2-Sulfonylpyrimidines: Mild alkylating agents with anticancer activity toward p53-compromised cells.

    Science.gov (United States)

    Bauer, Matthias R; Joerger, Andreas C; Fersht, Alan R

    2016-09-06

    The tumor suppressor p53 has the most frequently mutated gene in human cancers. Many of p53's oncogenic mutants are just destabilized and rapidly aggregate, and are targets for stabilization by drugs. We found certain 2-sulfonylpyrimidines, including one named PK11007, to be mild thiol alkylators with anticancer activity in several cell lines, especially those with mutationally compromised p53. PK11007 acted by two routes: p53 dependent and p53 independent. PK11007 stabilized p53 in vitro via selective alkylation of two surface-exposed cysteines without compromising its DNA binding activity. Unstable p53 was reactivated by PK11007 in some cancer cell lines, leading to up-regulation of p53 target genes such as p21 and PUMA. More generally, there was cell death that was independent of p53 but dependent on glutathione depletion and associated with highly elevated levels of reactive oxygen species and induction of endoplasmic reticulum (ER) stress, as also found for the anticancer agent PRIMA-1(MET)(APR-246). PK11007 may be a lead for anticancer drugs that target cells with nonfunctional p53 or impaired reactive oxygen species (ROS) detoxification in a wide variety of mutant p53 cells.

  2. Using Emergence Theory-Based Curriculum to Teach Compromise Skills to Students with Autistic Spectrum Disorders

    Science.gov (United States)

    Fein, Lance; Jones, Don

    2015-01-01

    This study addresses the compromise skills that are taught to students diagnosed with autistic spectrum disorders (ASD) and related social and communication deficits. A private school in the southeastern United States implemented an emergence theory-based curriculum to address these skills, yet no formal analysis was conducted to determine its…

  3. Upper limb function and functional independence in patients with shoulder pain after stroke

    OpenAIRE

    Nickel, Renato; Lange, Marcos; Stoffel, Diane Priscila; Navarro, Elaine Janeczko; Zetola, Viviane F

    2017-01-01

    ABSTRACT Objective To examine the frequency of shoulder pain following stroke. Methods Stroke patient function was evaluated using the Functional Independence Measure (FIM) and Scale for Upper Limb Function in Stroke (SULFS). Function scores were examined and compared between the shoulder pain group (SPG) and the no shoulder pain group (No-SPG). Results A total of 58 patients, 22 women (37.9%), were included in this study. The mean patient age was 49.2±10.8 years and study evaluations w...

  4. A Comparison between the Cytotoxicity Induced by Gossypol in Two Testicular Cell Lines

    OpenAIRE

    Neda MahdinezhadGorji; SeyedGholamAli Jorsaraei; Vida Hojati; Ebrahim Zabihi; Asieh Khalilpour; Zainab Abedian; Eisa Tahmasbpour

    2014-01-01

    Background: Gossypol is a yellow toxic pigment from the cottonseed that can cause acute or chronic toxicity in humans and animals by affecting the testicular tissues. Nowadays cottonseed is used as food supplement for ruminants specially the sheep. In this study, two different stem cell lines of testicular tissue including GC1-spg (mouse testis) and SFTF-PI43 (sheep testis) cells were used to evaluation of gossypol cytotoxicity. Methods: The GC-1spg and the SFTF_PI43 cells were cultured in...

  5. Esthetic management of compromised ridge in the anterior maxilla, a modified prosthetic approach

    Directory of Open Access Journals (Sweden)

    R Lambodharan

    2017-01-01

    Full Text Available Replacing a missing tooth with an implant, especially in the esthetic zone has been increasing demand for the patient. Placing dental implants in the esthetic zone, especially in the compromised ridge with thin gingival thin biotype is considered to be the ultimate challenge for many dentists. This case report illustrates the implant placement in the traumatic anterior maxilla with thin gingival biotype (Class IV and a modified approach in prosthetic placement.

  6. Managing Campus Energy: Compromising between Rapid Needs and Environmental Requirement

    Science.gov (United States)

    Ambariyanto, Ambariyanto; Utama, Yos J.; Purwanto

    2018-02-01

    The utilization of energy, especially electricity at Diponegoro University campus continues to increase in line with the development of the university. This increase has a direct impact on the increased costs to be paid by the university. Some of the causes of increased utilization of electrical energy is the construction of new buildings to meet the needs, increased learning activities and education, research activities in the laboratory, and various other activities. On the other hand, the increase of energy utilization is considered not good from the environment point of view, especially the utilization of electrical energy coming from non sustainable resources. Efforts to compromise on both are to develop policies in developing environmentally friendly buildings, efficiency in utilization of electrical energy, and development of sustainable energy sources.

  7. CO2-driven compromises to marine life along the Chilean coast

    Science.gov (United States)

    Mayol, E.; Ruiz-Halpern, S.; Duarte, C. M.; Castilla, J. C.; Pelegrí, J. L.

    2010-12-01

    CO2-driven compromises to marine life were examined along the Chilean sector of the Humboldt Current System, a particularly vulnerable hypoxic and upwelling area, applying the Respiration index (RI = log10 pO2) and the pH-dependent aragonite saturation (Ω) to delineate the water masses where aerobic and calcifying organisms are stressed. There was a remarkable negative relationship between oxygen concentration and pH or pCO2 in the studied area, with the subsurface hypoxic Equatorial Subsurface Waters extending from 100 m to about 300 m depth and supporting elevated pCO2 values. The RI reached a minimum at about 200 m depth and decreased towards the Equator. Increased pCO2 in the hypoxic water layer reduced the RI values by as much as 0.59 RI units, with the upper water layer that presents conditions suitable for aerobic life (RI>0.7) declining by half between 42° S and 28° S. The intermediate waters hardly reached those stations closer to the equator so that the increased pCO2 lowered pH and the saturation of aragonite. A significant fraction of the water column along the Chilean sector of the Humboldt Current System suffers from CO2-driven compromises to biota, including waters corrosive to calcifying organisms, stress to aerobic organisms or both. The habitat free of CO2-driven stresses was restricted to the upper mixed layer and to small water parcels at about 1000 m depth. pCO2 acts as a hinge connecting respiratory and calcification challenges expected to increase in the future, resulting in a spread of the challenges to aerobic organisms.

  8. Synthesis of highly interconnected 3D scaffold from Arothron stellatus skin collagen for tissue engineering application.

    Science.gov (United States)

    Ramanathan, Giriprasath; Singaravelu, Sivakumar; Raja, M D; Sivagnanam, Uma Tiruchirapalli

    2015-11-01

    The substrate which is avidly used for tissue engineering applications should have good mechanical and biocompatible properties, and all these parameters are often considered as essential for dermal reformation. Highly interconnected three dimensional (3D) wound dressing material with enhanced structural integrity was synthesized from Arothron stellatus fish skin (AsFS) collagen for tissue engineering applications. The synthesized 3D collagen sponge (COL-SPG) was further characterized by different physicochemical methods. The scanning electron microscopy analysis of the material demonstrated that well interconnected pores with homogeneous microstructure on the surface aids higher swelling index and that the material also possessed good mechanical properties with a Young's modulus of 0.89±0.2 MPa. Biocompatibility of the 3D COL-SPG showed 92% growth for both NIH 3T3 fibroblasts and keratinocytes. Overall, the study revealed that synthesized 3D COL-SPG from fish skin will act as a promising wound dressing in skin tissue engineering. Copyright © 2015 Elsevier Ltd. All rights reserved.

  9. Natural Tooth Pontic: An Instant Esthetic Option for Periodontally Compromised Teeth—A Case Series

    Directory of Open Access Journals (Sweden)

    Rishi Raj

    2016-01-01

    Full Text Available Sudden tooth loss in the esthetic zone of the maxillary or mandibular anterior region can be due to trauma, periodontal disease, or endodontic failure. The treatment options for replacing the missing tooth can vary between removable prosthesis, tooth-supported prosthesis, and implant-supported prosthesis. Irrespective of the final treatment, the first line of management would be to provisionally restore the patient’s esthetic appearance at the earliest, while functionally stabilizing the compromised arch. Using the patient’s own natural tooth as a pontic offers the benefits of being the right size, shape, and color and provides exact repositioning in its original intraoral three-dimensional position. Additionally, using the patient’s platelet concentrate (platelet rich fibrin facilitates early wound healing and preservation of alveolar ridge shape following tooth extraction. The abutment teeth can also be preserved with minimal or no preparation, thus keeping the technique reversible, and can be completed at the chair side thereby avoiding laboratory costs. This helps the patient better tolerate the effect of tooth loss psychologically. The article describes a successful, immediate, and viable technique for rehabilitation of three different patients requiring replacement of a single periodontally compromised tooth in an esthetic region.

  10. Can Neglected Tropical Diseases Compromise Human Wellbeing in Sex-, Age-, and Trait-Specific Ways?

    Directory of Open Access Journals (Sweden)

    David C Geary

    2016-04-01

    Full Text Available Traits that facilitate competition for reproductive resources or that influence mate choice have evolved to signal resilience to infectious disease and other stressors. As a result, the dynamics of competition and choice can, in theory, be used to generate predictions about sex-, age-, and trait-specific vulnerabilities for any sexually reproducing species, including humans. These dynamics and associated vulnerabilities are reviewed for nonhuman species, focusing on traits that are compromised by exposure to parasites. Using the same approach, sex-, age-, and trait-specific vulnerabilities to parasitic disease are illustrated for children's and adolescent's physical growth and fitness. Suggestions are then provided for widening the assessment of human vulnerabilities to include age-appropriate measures of behavioral (e.g., children's play and cognitive (e.g., language fluency traits. These are traits that are likely to be compromised by infection in age- and sex-specific ways. Inclusion of these types of measures in studies of neglected tropic diseases has the potential to provide a more nuanced understanding of how these diseases undermine human wellbeing and may provide a useful means to study the efficacy of associated treatments.

  11. Prosthodontic decision-making relating to dentitions with compromised molars: the perspective of Swedish General Dental Practitioners.

    Science.gov (United States)

    Korduner, E-K; Collin Bagewitz, I; Vult von Steyern, P; Wolf, E

    2016-12-01

    The aim of this investigation was to study the clinical prosthodontic decision-making process relating to dentitions with compromised molars among Swedish general dental practitioners (GDPs). Eleven Swedish GDPs were purposively selected, and all agreed to participate. Then, in-depth, semi-structured interviews were conducted and covered treatment considerations concerning two authentic patient cases, initially with complete dental arches, and later, a final treatment based on a shortened dental arch (SDA) was discussed. The cases involved patients with compromised teeth situated mainly in the molar regions. One patient suffered from extensive caries and the other from severe periodontal disease. Qualitative content analysis was used to analyse the data. In the systematic analysis, two main categories were identified: holistic and functional approach. Among the interviewed GDPs, focus was put on patients' needs, background history and motivation for treatment as well as the preservation of molar support. Within the limitations of this study, the following can be concluded: keeping a dental arch with molars seems to be important to Swedish general dental practitioners. The SDA concept does not seem to have a substantial impact on the prosthodontic decision-making relating to dentitions with compromised molars. The dentist's experiences, as well as colleagues' or consulting specialist advice together with aetiological factors and the patient's individual situation, influence the decision-making more than the SDA concept. The conflicting results in the prosthetic decision-making process concerning the relevance of age and the need for molar support need further investigation, for example based on decisions made in the dentist's own clinical practice. © 2016 John Wiley & Sons Ltd.

  12. Chronic Lymphocytic Leukemia as an Unusual Cause of Rapid Airway Compromise

    Directory of Open Access Journals (Sweden)

    Adrian R. Bersabe

    2017-01-01

    Full Text Available Chronic Lymphocytic Leukemia (CLL is the most prevalent form of non-Hodgkin’s lymphoma (NHL in Western countries predominantly affecting adults over the age of 65. CLL is commonly indolent in nature but can present locally and aggressively at extranodal sites. Although CLL may commonly present with cervical lymphadenopathy, manifestation in nonlymphoid regions of the head and neck is not well described. CLL causing upper airway obstruction is even more uncommon. We describe a case of a patient with known history of CLL and stable lymphocytosis that developed an enlarging lymphoid base of tongue (BOT mass resulting in rapid airway compromise.

  13. Management of compromised anterior maxillary edentulous arch with symphyseal bone graft

    Directory of Open Access Journals (Sweden)

    Sunil Kumar Mishra

    2018-01-01

    Full Text Available Achieving optimal aesthetics, function and phonetics with implant-supported restorations depends largely on the amount of bone present at the site where implant has to be placed. Sometimes, patient reports with compromised conditions which makes the implant placement difficult. Today, reconstruction procedures such as autogenous bone graft and guided bone regeneration make the implant placement possible in different ridge deficient cases. This case report describes step-by-step procedure of restoring the missing maxillary right central incisor with implant-supported prostheses. In missing left central incisor region, the alveolar bony defect was corrected by autogenous bone graft obtained from symphysis region, for restoration with implant prostheses.

  14. The European directive on renewable electricity: conflicts and compromises

    International Nuclear Information System (INIS)

    Rowlands, I.H.

    2005-01-01

    As part of its efforts to increase the use of renewable energy in Europe, a Directive regarding renewable electricity was agreed by the European Union in 2001. The purpose of this article is to examine this Directive, examining how the discussions surrounding its content unfolded. The investigation focuses upon three contentious issues that were debated during the Directive's development: the definition of 'renewable', the national targets for renewable electricity (their levels, as well as whether they should be 'binding' or 'indicative') and the questions associated with harmonisation (whether one Union-wide 'support scheme' for renewable electricity should be in place, and, if so, what it should be). During the 5 years that the Directive was negotiated, many intra-Union conflicts were eventually resolved, at least temporarily, by compromises. Nevertheless, some difficult decisions regarding the promotion of renewable electricity in the European Union still have to be taken

  15. Enhanced susceptibility to predation in corals of compromised condition.

    Science.gov (United States)

    Bright, Allan J; Cameron, Caitlin M; Miller, Margaret W

    2015-01-01

    The marine gastropod, Coralliophila abbreviata, is an obligate corallivore that causes substantial mortality in Caribbean Acropora spp. Considering the imperiled status of Acropora cervicornis and A. palmata, a better understanding of ecological interactions resulting in tissue loss may enable more effective conservation strategies. We examined differences in susceptibility of A. cervicornis to C. abbreviata predation based on coral tissue condition. Coral tissue condition was a strong determinant of snail prey choice, with snails preferring A. cervicornis fragments that were diseased or mechanically damaged over healthy fragments. In addition, snails always chose fragments undergoing active predation by another snail, while showing no preference for a non-feeding snail when compared with an undisturbed prey fragment. These results indicate that the condition of A. cervicornis prey influenced foraging behavior of C. abbreviata, creating a potential feedback that may exacerbate damage from predation in coral populations compromised by other types of disturbance.

  16. Biosynthetic Studies of 13-Desmethylspirolide C Produced by Alexandrium ostenfeldii (= A. peruvianum): Rationalization of the Biosynthetic Pathway Following Incorporation of (13)C-Labeled Methionine and Application of the Odd-Even Rule of Methylation.

    Science.gov (United States)

    Anttila, Matthew; Strangman, Wendy; York, Robert; Tomas, Carmelo; Wright, Jeffrey L C

    2016-03-25

    Understanding the biosynthesis of dinoflagellate polyketides presents many unique challenges. Because of the remaining hurdles to dinoflagellate genome sequencing, precursor labeling studies remain the only viable way to investigate dinoflagellate biosynthesis. However, prior studies have shown that polyketide chain assembly does not follow any of the established processes. Additionally, acetate, the common precursor for polyketides, is frequently scrambled, thus compromising interpretation. These factors are further compounded by low production yields of the compounds of interest. A recent report on the biosynthesis of spirolides, a group belonging to the growing class of toxic spiroimines, provided some insight into the polyketide assembly process based on acetate labeling studies, but many details were left uncertain. By feeding (13)C methyl-labeled methionine to cultures of Alexandrium ostenfeldii, the producing organism of 13-desmethylspirolide C, and application of the odd-even methylation rule, the complete biosynthetic pathway has been established.

  17. The temporal profile of the reaction of microglia, astrocytes, and macrophages in the delayed onset paraplegia after transient spinal cord ischemia in rabbits.

    Science.gov (United States)

    Matsumoto, Satoshi; Matsumoto, Mishiya; Yamashita, Atsuo; Ohtake, Kazunobu; Ishida, Kazuyoshi; Morimoto, Yasuhiro; Sakabe, Takefumi

    2003-06-01

    In the present study, we sought to elucidate the temporal profile of the reaction of microglia, astrocytes, and macrophages in the progression of delayed onset motor dysfunction after spinal cord ischemia (15 min) in rabbits. At 2, 4, 8, 12, 24, and 48 h after reperfusion (9 animals in each), hind limb motor function was assessed, and the lumbar spinal cord was histologically examined. Delayed motor dysfunction was observed in most animals at 48 h after ischemia, which could be predicted by a poor recovery of segmental spinal cord evoked potentials at 15 min of reperfusion. In the gray matter of the lumbar spinal cord, both microglia and astrocytes were activated early (2 h) after reperfusion. Microglia were diffusely activated and engulfed motor neurons irrespective of the recovery of segmental spinal cord evoked potentials. In contrast, early astrocytic activation was confined to the area where neurons started to show degeneration. Macrophages were first detected at 8 h after reperfusion and mainly surrounded the infarction area later. Although the precise roles of the activation of microglia, astrocytes, and macrophages are to be further determined, the results indicate that understanding functional changes of astrocytes may be important in the mechanism of delayed onset motor dysfunction including paraplegia. Microglia and macrophages play a role in removing tissue debris after transient spinal cord ischemia. Disturbance of astrocytic defense mechanism, breakdown of the blood-spinal cord barrier, or both seemed to be involved in the development of delayed motor dysfunction.

  18. Video-Assisted versus Open Lobectomy in Patients with Compromised Lung Function: A Literature Review and Meta-Analysis.

    Directory of Open Access Journals (Sweden)

    Ruoyu Zhang

    Full Text Available It has been suggested that video-assisted (VATS lobectomy is safer than open lobectomy in patients with compromised lung function, but data regarding this are limited. We assessed acute outcomes of VATS compared to open lobectomy in these high-risk patients using a systematic literature review and meta-analysis of data.The databases PubMed and Scopus were searched for studies published between 2000 and 2013 that reported mortality and morbidity of VATS in high-risk lung cancer patients defined as having compromised pulmonary or cardiopulmonary function. Study selection, data collection and critical assessment of the included studies were performed according to the recommendations of the Cochrane Collaboration.Three case-control studies and three case series that included 330 VATS and 257 open patients were identified for inclusion. Operative mortality, overall morbidity and pulmonary morbidity were 2.5%, 39.3%, 26.2% in VATS patients and 7.8%, 57.5%, 45.5% in open lobectomy group, respectively. VATS lobectomy patients experienced significantly lower pulmonary morbidity (RR = 0.45; 95% CI, 0.30 to 0.67; p = 0.0001, somewhat reduced operative mortality (RR = 0.51; 95% CI, 0.24 to 1.06; p = 0.07, but no significant difference in overall morbidity (RR = 0.68; 95% CI, 0.41 to 1.14; p = 0.14.The existing data suggest that VATS lobectomy is associated with lower risk for pulmonary morbidity compared with open lobectomy in lung cancer patients with compromised lung function.

  19. Orientation of handle for successful prosthetic treatment in patients with an anatomic compromise after a maxillectomy.

    Science.gov (United States)

    Sumita, Yuka I; Hattori, Mariko; Elbashti, Mahmoud E; Taniguchi, Hisashi

    2017-05-01

    This clinical technique provides a key procedure for successful prosthetic treatment in patients with an anatomical compromise after a maxillectomy. When making a preliminary impression, the clinician must keep the handle of the stock tray parallel to the patient's interpupillary line extraorally to maintain the correct position of the tray and to make a record of the inclination of the occlusal plane. This simple consideration during this first step of making a preliminary impression can be an important guide for both dentists and dental technicians. Information about the correct positioning of the tray and the inclination of the occlusal plane obtained by using a stock tray handle will greatly reduce the stress experienced by dentists, dental technicians, and patients during the fabrication of the prosthesis and will ultimately lead to successful prosthetic treatment in patients with anatomic compromise resulting from a maxillectomy. Copyright © 2016 Editorial Council for the Journal of Prosthetic Dentistry. Published by Elsevier Inc. All rights reserved.

  20. Multi-objective experimental design for (13)C-based metabolic flux analysis.

    Science.gov (United States)

    Bouvin, Jeroen; Cajot, Simon; D'Huys, Pieter-Jan; Ampofo-Asiama, Jerry; Anné, Jozef; Van Impe, Jan; Geeraerd, Annemie; Bernaerts, Kristel

    2015-10-01

    (13)C-based metabolic flux analysis is an excellent technique to resolve fluxes in the central carbon metabolism but costs can be significant when using specialized tracers. This work presents a framework for cost-effective design of (13)C-tracer experiments, illustrated on two different networks. Linear and non-linear optimal input mixtures are computed for networks for Streptomyces lividans and a carcinoma cell line. If only glucose tracers are considered as labeled substrate for a carcinoma cell line or S. lividans, the best parameter estimation accuracy is obtained by mixtures containing high amounts of 1,2-(13)C2 glucose combined with uniformly labeled glucose. Experimental designs are evaluated based on a linear (D-criterion) and non-linear approach (S-criterion). Both approaches generate almost the same input mixture, however, the linear approach is favored due to its low computational effort. The high amount of 1,2-(13)C2 glucose in the optimal designs coincides with a high experimental cost, which is further enhanced when labeling is introduced in glutamine and aspartate tracers. Multi-objective optimization gives the possibility to assess experimental quality and cost at the same time and can reveal excellent compromise experiments. For example, the combination of 100% 1,2-(13)C2 glucose with 100% position one labeled glutamine and the combination of 100% 1,2-(13)C2 glucose with 100% uniformly labeled glutamine perform equally well for the carcinoma cell line, but the first mixture offers a decrease in cost of $ 120 per ml-scale cell culture experiment. We demonstrated the validity of a multi-objective linear approach to perform optimal experimental designs for the non-linear problem of (13)C-metabolic flux analysis. Tools and a workflow are provided to perform multi-objective design. The effortless calculation of the D-criterion can be exploited to perform high-throughput screening of possible (13)C-tracers, while the illustrated benefit of multi

  1. Navigating safety necessary compromises and trade-offs : theory and practice

    CERN Document Server

    Amalberti, René

    2013-01-01

    Managing safety in a professional environment requires constant negotiation with other competitive dimensions of risk management (finances, market and political drivers, manpower and social crisis). This is obvious, although generally not said in safety manuals. The book provides a unique vision of how to best find these compromises, starting with lessons learnt from natural risk management by individuals, then applying them to the craftsman industry, complex industrial systems (civil aviation, nuclear energy) and public services (like transportation and medicine). It offers a unique, illustrated, easy to read and scientifically based set of original concepts and pragmatic methods to revisit safety management and adopt a successful system vision. As such, and with illustrations coming from many various fields (aviation, fishing, nuclear, oil, medicine), it potentially covers a broad readership.

  2. Tract-Specific Analyses of Diffusion Tensor Imaging Show Widespread White Matter Compromise in Autism Spectrum Disorder

    Science.gov (United States)

    Shukla, Dinesh K.; Keehn, Brandon; Muller, Ralph-Axel

    2011-01-01

    Background: Previous diffusion tensor imaging (DTI) studies have shown white matter compromise in children and adults with autism spectrum disorder (ASD), which may relate to reduced connectivity and impaired function of distributed networks. However, tract-specific evidence remains limited in ASD. We applied tract-based spatial statistics (TBSS)…

  3. Left Ventricular Gene Expression Profile of Healthy and Cardiovascular Compromised Rat Models Used in Air Pollution Studies

    Science.gov (United States)

    The link between pollutant exposure and cardiovascular disease (CVD) has prompted mechanistic research with animal models of CVD. We hypothesized that the cardiac gene expression patterns of healthy and genetically compromised, CVD-prone rat models, with or without metabolic impa...

  4. Commonly Employed African Neonatal Skin Care Products Compromise Epidermal Function in Mice.

    Science.gov (United States)

    Man, Mao-Qiang; Sun, Richard; Man, George; Lee, Dale; Hill, Zelee; Elias, Peter M

    2016-09-01

    Neonatal mortality is much higher in the developing world than in developed countries. Infections are a major cause of neonatal death, particularly in preterm infants, in whom defective epidermal permeability barrier function facilitates transcutaneous pathogen invasion. The objective was to determine whether neonatal skin care products commonly used in Africa benefit or compromise epidermal functions in murine skin. After twice-daily treatment of 6- to 8-week-old hairless mice with each skin care product for 3 days, epidermal permeability barrier function, skin surface pH, stratum corneum hydration, and barrier recovery were measured using a multiprobe adapter system physiology monitor. For products showing some benefits in these initial tests, the epidermal permeability barrier homeostasis was assessed 1 and 5 hours after a single application to acutely disrupted skin. All of the skin care products compromised basal permeability barrier function and barrier repair kinetics. Moreover, after 3 days of treatment, most of the products also reduced stratum corneum hydration while elevating skin surface pH to abnormal levels. Some neonatal skin care products that are widely used in Africa perturb important epidermal functions, including permeability barrier homeostasis in mice. Should these products have similar effects on newborn human skin, they could cause a defective epidermal permeability barrier, which can increase body fluid loss, impair thermoregulation, and contribute to the high rates of neonatal morbidity and mortality seen in Africa. Accordingly, alternative products that enhance permeability barrier function should be identified, particularly for use in preterm infants. © 2016 Wiley Periodicals, Inc.

  5. Consequences of early extraction of compromised first permanent molar: a systematic review.

    Science.gov (United States)

    Saber, Afnan M; Altoukhi, Doua H; Horaib, Mariam F; El-Housseiny, Azza A; Alamoudi, Najlaa M; Sabbagh, Heba J

    2018-04-05

    The aim of this study was to systematically review the literature to determine the sequelae of early extraction of compromised first permanent molars (FPMs) with regard to the skeletal and dental development of 5- to 15-year-old children. Meta-analysis was conducted when applicable. Our research protocol included a search strategy, inclusion/exclusion criteria, and a data extraction plan. The search engines used were PubMed, Scopus, and Science Direct. Study selection was performed independently by three reviewers. Articles published from 1960 to 2017 were reviewed based on inclusion and exclusion criteria. Meta-analysis was performed to compare space closure between upper and lower arches. Eleven studies fulfilled the inclusion criteria. The consequences were decrease in post extraction space, accelerated development and eruption of second permanents molars (SPMs) and third molars, a decrease in caries and/or fillings on the proximal surfaces of adjacent teeth, lingual tipping and retrusion of incisors, and counter clockwise rotation of the occlusal plane. There were several consequences of early extraction of FPMs, which were related to skeletal and dental development. Our systematic review suggests that comprehensive evaluation of the compromised FPMs should be performed before planning an extraction. The ideal time for FPM extraction is when the SPM is at the early bifurcation stage in order to achieve complete closure of the extraction space by the SPM. Benefits should be weighed over the risks to decrease the risk of unfavorable outcomes as much as possible. However, due to the limited evidence on the outcomes and variables that influence them, high-quality prospective studies are needed.

  6. Compromised Global and Regional Cerebral Blood Flow in Congestive Heart Failure

    Energy Technology Data Exchange (ETDEWEB)

    Kim, J. S.; Kim, J. J.; Im, K. C.; Moon, D. H. [College of Medicine, Univ. of Ulsan, Seoul (Korea, Republic of)

    2003-07-01

    It has been known that cerebral perfusion is maintained in patients with congestive heart failure (CHF) by a complex series of compensatory mechanisms. However cognitive impairment is a common problem experienced by patients with CHF and may result from deranged cerebral perfusion. We prospectively investigated the global and regional CBF of patients with CHF and compared the results with that of normal controls. Thirty two patients (M/F: 22/10, 4211 yr) with CHF (LVEF=218.1%) and 10 healthy controls (M/F: 6/4, 398 yr) were prospectively studied. No patients had cerebrovascular disease or other disease affecting cognitive function. All patients and normal controls underwent radionuclide angiography including cerebral hemispheres and aortic arch, and brain perfusion SPECT using Tc-99m ECD. Global CBF was measured non-invasively by the application of Patlak plot graphical analysis. Quantitative rCBF images were obtained from SPECT image using global CBF values, regional/global count ratios, and Lassen's linearization correction algorithm. Difference of regional CBF between CHF and normal control were assessed using a SPM99 without global count normalization (uncorrected p<0.0001, extent threshold>100 voxels). Global CBF (41.54.7 ml/min/100g) of the patients with CHF were significantly lower than those (49.15.7 ml/min/100g) of controls (p<0.001). Regional CBF was significantly decreased in frontal, temporal and parietal neocortex of both cerebral hemispheres compared to normal controls. Regional rCBF of basal ganglia, thalamus, and brain stem were preserved, even though global CBF was variably compromised. Our study show that global CBF is significantly decreased in CHF and regional CBF of frontal, parietal and temporal neocortex is compromised preferentially. Further studies would be needed to investigate the relationship of rCBF change and cognitive impairment in patients with CHF.

  7. β-Cell Ca(2+) dynamics and function are compromised in aging.

    Science.gov (United States)

    Barker, Christopher J; Li, Luosheng; Köhler, Martin; Berggren, Per-Olof

    2015-01-01

    Defects in pancreatic β-cell function and survival are key components in type 2 diabetes (T2D). An age-dependent deterioration in β-cell function has also been observed, but little is known about the molecular mechanisms behind this phenomenon. Our previous studies indicate that the regulation of cytoplasmic free Ca(2+) concentration ([Ca(2+)]i) may be critical and that this is dependent on the proper function of the mitochondria. The [Ca(2+)]i dynamics of the pancreatic β-cell are driven by an interplay between glucose-induced influx of extracellular Ca(2+) via voltage-dependent Ca(2+) channels and the inositol 1,4,5-trisphosphate (Ins(1,4,5)P3)-mediated liberation of Ca(2+) from intracellular stores. Our previous work has indicated a direct relationship between disruption of Ins(1,4,5)P3-mediated Ca(2+) regulation and loss of β-cell function, including disturbed [Ca(2+)]i dynamics and compromised insulin secretion. To investigate these processes in aging we used three mouse models, a premature aging mitochondrial mutator mouse, a mature aging phenotype (C57BL/6) and an aging-resistant phenotype (129). Our data suggest that age-dependent impairment in mitochondrial function leads to modest changes in [Ca(2+)]i dynamics in mouse β-cells, particularly in the pattern of [Ca(2+)]i oscillations. These changes are driven by modifications in both PLC/Ins(1,4,5)P3-mediated Ca(2+) mobilization from intracellular stores and decreased β-cell Ca(2+) influx over the plasma membrane. Our findings underscore an important concept, namely that even relatively small, time-dependent changes in β-cell signal-transduction result in compromised insulin release and in a diabetic phenotype. Copyright © 2014 Elsevier Ltd. All rights reserved.

  8. Enhanced susceptibility to predation in corals of compromised condition

    Directory of Open Access Journals (Sweden)

    Allan J. Bright

    2015-09-01

    Full Text Available The marine gastropod, Coralliophila abbreviata, is an obligate corallivore that causes substantial mortality in Caribbean Acropora spp. Considering the imperiled status of Acropora cervicornis and A. palmata, a better understanding of ecological interactions resulting in tissue loss may enable more effective conservation strategies. We examined differences in susceptibility of A. cervicornis to C. abbreviata predation based on coral tissue condition. Coral tissue condition was a strong determinant of snail prey choice, with snails preferring A. cervicornis fragments that were diseased or mechanically damaged over healthy fragments. In addition, snails always chose fragments undergoing active predation by another snail, while showing no preference for a non-feeding snail when compared with an undisturbed prey fragment. These results indicate that the condition of A. cervicornis prey influenced foraging behavior of C. abbreviata, creating a potential feedback that may exacerbate damage from predation in coral populations compromised by other types of disturbance.

  9. Compromised epidermal barrier stimulates Harderian gland activity and hypertrophy in ACBP-/- mice

    DEFF Research Database (Denmark)

    Sørensen, Signe Bek; Neess, Ditte; Dixen, Karen

    2015-01-01

    of the eye lid. We show that disruption of the Acbp gene leads to a significant enlargement of this gland with hypertrophy of the acinar cells and increased de novo synthesis of monoalkyl diacylglycerol, the main lipid species produced by the gland. Mice with conditional targeting of the Acbp gene......Acyl-CoA binding protein (ACBP) is a small, ubiquitously expressed intracellular protein that binds C14-C22 acyl-CoA esters with very high affinity and specificity. We have recently shown that targeted disruption of the Acbp gene leads to a compromised epidermal barrier and that this causes delayed...

  10. 16 CFR 1207.1 - Scope, purpose, and findings.

    Science.gov (United States)

    2010-01-01

    ...) quadriplegia and paraplegia resulting from users (primarily adults using the swimming pool slide for the first... address the risk of quadriplegia and paraplegia (except insofar as the standard specifies a low angle of...

  11. Neurological Complications Following Endoluminal Repair of Thoracic Aortic Disease

    International Nuclear Information System (INIS)

    Morales, J. P.; Taylor, P. R.; Bell, R. E.; Chan, Y. C.; Sabharwal, T.; Carrell, T. W. G.; Reidy, J. F.

    2007-01-01

    Open surgery for thoracic aortic disease is associated with significant morbidity and the reported rates for paraplegia and stroke are 3%-19% and 6%-11%, respectively. Spinal cord ischemia and stroke have also been reported following endoluminal repair. This study reviews the incidence of paraplegia and stroke in a series of 186 patients treated with thoracic stent grafts. From July 1997 to September 2006, 186 patients (125 men) underwent endoluminal repair of thoracic aortic pathology. Mean age was 71 years (range, 17-90 years). One hundred twenty-eight patients were treated electively and 58 patients had urgent procedures. Anesthesia was epidural in 131, general in 50, and local in 5 patients. Seven patients developed paraplegia (3.8%; two urgent and five elective). All occurred in-hospital apart from one associated with severe hypotension after a myocardial infarction at 3 weeks. Four of these recovered with cerebrospinal fluid (CSF) drainage. One patient with paraplegia died and two had permanent neurological deficit. The rate of permanent paraplegia and death was 1.6%. There were seven strokes (3.8%; four urgent and three elective). Three patients made a complete recovery, one had permanent expressive dysphasia, and three died. The rate of permanent stroke and death was 2.1%. Endoluminal treatment of thoracic aortic disease is an attractive alternative to open surgery; however, there is still a risk of paraplegia and stroke. Permanent neurological deficits and death occurred in 3.7% of the patients in this series. We conclude that prompt recognition of paraplegia and immediate insertion of a CSF drain can be an effective way of recovering spinal cord function and improving the prognosis

  12. 'Compromise position' image alignment to accommodate independent motion of multiple clinical target volumes during radiotherapy: A high risk prostate cancer example

    International Nuclear Information System (INIS)

    Rosewall, Tara; Alasti, Hamideh; Bayley, Andrew; Yan, Jing

    2017-01-01

    Inclusion of multiple independently moving clinical target volumes (CTVs) in the irradiated volume causes an image guidance conundrum. The purpose of this research was to use high risk prostate cancer as a clinical example to evaluate a 'compromise' image alignment strategy. The daily pre-treatment orthogonal EPI for 14 consecutive patients were included in this analysis. Image matching was performed by aligning to the prostate only, the bony pelvis only and using the 'compromise' strategy. Residual CTV surrogate displacements were quantified for each of the alignment strategies. Analysis of the 388 daily fractions indicated surrogate displacements were well-correlated in all directions (r 2 = 0.95 (LR), 0.67 (AP) and 0.59 (SI). Differences between the surrogates displacements (95% range) were −0.4 to 1.8 mm (LR), −1.2 to 5.2 mm (SI) and −1.2 to 5.2 mm (AP). The distribution of the residual displacements was significantly smaller using the 'compromise' strategy, compared to the other strategies (p 0.005). The 'compromise' strategy ensured the CTV was encompassed by the PTV in all fractions, compared to 47 PTV violations when aligned to prostate only. This study demonstrated the feasibility of a compromise position image guidance strategy to accommodate simultaneous displacements of two independently moving CTVs. Application of this strategy was facilitated by correlation between the CTV displacements and resulted in no geometric excursions of the CTVs beyond standard sized PTVs. This simple image guidance strategy may also be applicable to other disease sites that concurrently irradiate multiple CTVs, such as head and neck, lung and cervix cancer.

  13. Managing forest and marginal agricultural land for multiple tradeoffs : compromising on economic, carbon and structural biodiversity objectives

    NARCIS (Netherlands)

    Krcmar, E.; Kooten, van G.C.; Vertinsky, I.

    2005-01-01

    In this paper, we use compromise programming to solve a multiple-objective land use and forest management planning model. Long- and short- (`fast¿) term carbon uptake, maintenance of structural diversity, and economic (net returns to forestry and agriculture) objectives are simultaneously achieved

  14. Orthodontic management of a periodontally compromised dentition

    Directory of Open Access Journals (Sweden)

    Prashant K Zaveri

    2016-01-01

    Full Text Available Malocclusion superimposed with severe periodontitis may present a great challenge to clinicians while providing orthodontic treatment due the episodic and site-specific nature of the disease with risk of rapid tissue breakdown. However, orthodontic treatment in such situation may contribute significantly to the overall rehabilitation both functionally and esthetically. In this article, a case report outlines a combined periodontic-orthodontic management of compromised dentition. A 37-year-old female patient with significant medical history was treated for Class II Division 1 type of malocclusion associated with spaced upper and lower anterior teeth, deep overbite, and increased overjet, superimposed with chronic generalized periodontitis and bone loss. Treatment was completed using temporary anchorage devices assisted strategically applied force and modified tandem retraction biomechanics amidst management of acute inflammatory episodes during and mucogingival complication after treatment. Affected areas healed very well after post-orthodontic periodontal treatment with minimal pocket depth, and bleeding on probing, and a healthy zone of attached gingiva at the follow up visits. The orthodontic results lead to improvement in patient's facial profile, lip posture, and correction of protrusion which addressed her main concern. One year follow-up shows good orthodontic and periodontic stability. The report highlights the importance of identifying “at risk” individuals and continuous monitoring of disease status during treatment. Despite all precautionary measures, a flare-up during the treatment can be anticipated.

  15. Which Statistic Should Be Used to Detect Item Preknowledge When the Set of Compromised Items Is Known?

    Science.gov (United States)

    Sinharay, Sandip

    2017-09-01

    Benefiting from item preknowledge is a major type of fraudulent behavior during educational assessments. Belov suggested the posterior shift statistic for detection of item preknowledge and showed its performance to be better on average than that of seven other statistics for detection of item preknowledge for a known set of compromised items. Sinharay suggested a statistic based on the likelihood ratio test for detection of item preknowledge; the advantage of the statistic is that its null distribution is known. Results from simulated and real data and adaptive and nonadaptive tests are used to demonstrate that the Type I error rate and power of the statistic based on the likelihood ratio test are very similar to those of the posterior shift statistic. Thus, the statistic based on the likelihood ratio test appears promising in detecting item preknowledge when the set of compromised items is known.

  16. Neglecting legumes has compromised human health and sustainable food production.

    Science.gov (United States)

    Foyer, Christine H; Lam, Hon-Ming; Nguyen, Henry T; Siddique, Kadambot H M; Varshney, Rajeev K; Colmer, Timothy D; Cowling, Wallace; Bramley, Helen; Mori, Trevor A; Hodgson, Jonathan M; Cooper, James W; Miller, Anthony J; Kunert, Karl; Vorster, Juan; Cullis, Christopher; Ozga, Jocelyn A; Wahlqvist, Mark L; Liang, Yan; Shou, Huixia; Shi, Kai; Yu, Jingquan; Fodor, Nandor; Kaiser, Brent N; Wong, Fuk-Ling; Valliyodan, Babu; Considine, Michael J

    2016-08-02

    The United Nations declared 2016 as the International Year of Pulses (grain legumes) under the banner 'nutritious seeds for a sustainable future'. A second green revolution is required to ensure food and nutritional security in the face of global climate change. Grain legumes provide an unparalleled solution to this problem because of their inherent capacity for symbiotic atmospheric nitrogen fixation, which provides economically sustainable advantages for farming. In addition, a legume-rich diet has health benefits for humans and livestock alike. However, grain legumes form only a minor part of most current human diets, and legume crops are greatly under-used. Food security and soil fertility could be significantly improved by greater grain legume usage and increased improvement of a range of grain legumes. The current lack of coordinated focus on grain legumes has compromised human health, nutritional security and sustainable food production.

  17. Sterilization of freeze dried manila clam (Ruditapea philippinarum) porridge for immuno-compromised patients

    Energy Technology Data Exchange (ETDEWEB)

    Song, Beom Seok; Park, Jae Nam [Team for Radiation Food Science and Biotechnology, Advanced Radiation Technology Institute, Korea Atomic Energy Research Institute, Jeongeup (Korea, Republic of)

    2017-02-15

    This study was conducted to evaluate the combined effect of gamma irradiation and different conditions (vacuum packaging, antioxidant and freezing) on the microbiological and sensory characteristics of freeze dried Manila clam porridge (MCP) for immuno-compromised patient food. McP can be sterilized at 1 kGy to 10 kGy. the initial counts of total aerobic bacteria and yeast molds in the non-irradiated MCP were 2.4±0.5 and 1.2±0.3 log CFU g{sup -}'1, respectively, but gamma irradiation significantly decreased the total aerobic bacteria to below the detection limit (1 log CFU g{sup -1}) (5 kGy). Moreover, gamma irradiation effectively eliminated yeasts/molds at dose below than 1 kGy. However, gamma irradiation accelerated the increase of lipid oxidation and therefore, decreased the sensory characteristics of MCP as irradiation dose increased. to improve the sensory qualities of gamma irradiated MCP, combination treatment (vacuum packaging, 0.1% vitamin c) were applied. there was no significant difference in the overall acceptance scores between the combined-treatment sample (5.6 points) and the non-irradiated samples (6.0). the results indicate that combination treatment (vacuum packaging, 0.1% vitamin c) may help to maintain the quality of MCP. therefore, it considered that irradiation of MCP with combined treatment and this is an effective method for the consumption as a special purpose food such as for space travel or immuno-compromised patients.

  18. Sterilization of freeze dried manila clam (Ruditapea philippinarum) porridge for immuno-compromised patients

    International Nuclear Information System (INIS)

    Song, Beom Seok; Park, Jae Nam

    2017-01-01

    This study was conducted to evaluate the combined effect of gamma irradiation and different conditions (vacuum packaging, antioxidant and freezing) on the microbiological and sensory characteristics of freeze dried Manila clam porridge (MCP) for immuno-compromised patient food. McP can be sterilized at 1 kGy to 10 kGy. the initial counts of total aerobic bacteria and yeast molds in the non-irradiated MCP were 2.4±0.5 and 1.2±0.3 log CFU g"-'1, respectively, but gamma irradiation significantly decreased the total aerobic bacteria to below the detection limit (1 log CFU g"-"1) (5 kGy). Moreover, gamma irradiation effectively eliminated yeasts/molds at dose below than 1 kGy. However, gamma irradiation accelerated the increase of lipid oxidation and therefore, decreased the sensory characteristics of MCP as irradiation dose increased. to improve the sensory qualities of gamma irradiated MCP, combination treatment (vacuum packaging, 0.1% vitamin c) were applied. there was no significant difference in the overall acceptance scores between the combined-treatment sample (5.6 points) and the non-irradiated samples (6.0). the results indicate that combination treatment (vacuum packaging, 0.1% vitamin c) may help to maintain the quality of MCP. therefore, it considered that irradiation of MCP with combined treatment and this is an effective method for the consumption as a special purpose food such as for space travel or immuno-compromised patients

  19. Cerebral hematocrit decreases with hemodynamic compromise in carotid artery occlusion: a PET study.

    Science.gov (United States)

    Yamauchi, H; Fukuyama, H; Nagahama, Y; Katsumi, Y; Okazawa, H

    1998-01-01

    This study investigated whether in patients with internal carotid artery occlusion the regional cerebral hematocrit correlates with cerebral hemodynamics or metabolic state and, if so, how the regional cerebral hematocrit changes in the hemodynamically compromised region. We used positron emission tomography to study seven patients with unilateral internal carotid artery occlusion and no cortical infarction in the chronic stage. The distributions of red blood cell and plasma volumes were assessed using oxygen-15-labeled carbon monoxide and copper-62-labeled human serum albumin-dithiosemicarbazone tracers, respectively. The calculated hematocrit value was compared with the hemodynamic and metabolic parameters measured with the oxygen-15 steady-state technique. In the cerebral cortex, the value of the cerebral hematocrit varied but was correlated with the hemodynamic and metabolic status. Stepwise regression analysis revealed that the large vessel hematocrit, the cerebral metabolic rate of oxygen, and the cerebral blood flow or the oxygen extraction fraction accounted for a significant proportion of variance of the cerebral hematocrit. The oxygen extraction fraction and the cerebral metabolic rate of oxygen negatively correlated with the cerebral hematocrit, whereas the cerebral blood flow correlated positively: patients with reduced blood supply relative to metabolic demand (decreased blood flow with increased oxygen extraction fraction) showed low hematocrit values. In carotid artery occlusion in the chronic stage, regional cerebral hematocrit may vary according to cerebral hemodynamics and metabolic status. Regional cerebral hematocrit may decrease with hemodynamic compromise unless oxygen metabolism concomitantly decreases.

  20. [A multi-measure analysis of the similarity, attraction, and compromise effects in multi-attribute decision making].

    Science.gov (United States)

    Tsuzuki, Takashi; Matsui, Hiroshi; Kikuchi, Manabu

    2012-12-01

    In multi-attribute decision making, the similarity, attraction, and compromise effects warrant specific investigation as they cause violations of principles in rational choice. In order to investigate these three effects simultaneously, we assigned 145 undergraduates to three context effect conditions. We requested them to solve the same 20 hypothetical purchase problems, each of which had three alternatives described along two attributes. We measured their choices, confidence ratings, and response times. We found that manipulating the third alternative had significant context effects for choice proportions and confidence ratings in all three conditions. Furthermore, the attraction effect was the most prominent with regard to choice proportions. In the compromise effect condition, although the choice proportion of the third alternative was high, the confidence rating was low and the response time was long. These results indicate that the relationship between choice proportions and confidence ratings requires further theoretical investigation. They also suggest that a combination of experimental and modeling studies is imperative to reveal the mechanisms underlying the context effects in multi-attribute, multi-alternative decision making.

  1. Arsenic promotes centrosome abnormalities and cell colony formation in p53 compromised human lung cells

    International Nuclear Information System (INIS)

    Liao Weiting; Lin Pinpin; Cheng, T.-S.; Yu, H.-S.; Chang, Louis W.

    2007-01-01

    Epidemiological evidence indicated that residents, especially cigarette smokers, in arseniasis areas had significantly higher lung cancer risk than those living in non-arseniasis areas. Thus, an interaction between arsenic and cigarette smoking in lung carcinogenesis was suspected. p53 dysfunction or mutation in lung epithelial cells was frequently observed in cigarette smokers. Our present study was to explore the differential effects by arsenic on H1355 cells (human lung adenocarcinoma cell line with mutation in p53), BEAS-2B (immortalized lung epithelial cell with functional p53) and pifithrin-α-treated BEAS-2B cells (p53-inhibited cells). These cells were treated with different doses of sodium arsenite (0, 0.1, 1, 5 and 10 μM) for 48 h. A greater reduction in cell viability was observed in the BEAS-2B cells vs. p53 compromised cells (H1355 or p53-inhibited BEAS-2B). Similar observation was also made on 7-day cell survival (growth) study. TUNEL analysis confirmed that there was indeed a significantly reduced arsenite-induced apoptosis found in p53-compromised cells. Centrosomal abnormality has been attributed to eventual chromosomal missegregation, aneuploidy and tumorigenesis. In our present study, reduced p21 and Gadd45a expressions and increased centrosomal abnormality (atopic and multiple centrosomes) were observed in both arsenite-treated H1355 and p53-inhibited BEAS-2B cells as compared with similarly treated BEAS-2B cells. Increased anchorage-independent growth (colony formation) of BEAS-2B cells co-treated with pifithrin-α and 5 μM sodium arsenite was also observed in soft agar. Our present investigation demonstrated that arsenic would act specifically on p53 compromised cells (either with p53 dysfunction or inhibited) to induce centrosomal abnormality and colony formation. These findings provided strong evidence on the carcinogenic promotional role of arsenic, especially under the condition of p53 dysfunction

  2. Sensitivity of North Atlantic subpolar gyre and overturning to stratification-dependent mixing: response to global warming

    Energy Technology Data Exchange (ETDEWEB)

    Marzeion, Ben [Massachusetts Institute of Technology, EAPS, Cambridge, MA (United States); Nansen Environmental and Remote Sensing Center and Bjerknes Centre for Climate Research, Bergen (Norway); University of Innsbruck, Tropical Glaciology Group, Institute of Geography, Innsbruck (Austria); Levermann, Anders [Potsdam University, Earth System Analysis, Potsdam Institute for Climate Impact Research and Institute of Physics, Potsdam (Germany); Mignot, Juliette [Universite Pierre et Marie Curie, LOCEAN, Paris (France)

    2010-04-15

    We use a reduced complexity climate model with a three-dimensional ocean component and realistic topography to investigate the effect of stratification-dependent mixing on the sensitivity of the North Atlantic subpolar gyre (SPG), and the Atlantic meridional overturning circulation (AMOC), to idealized CO{sub 2} increase and peaking scenarios. The vertical diffusivity of the ocean interior is parameterized as {kappa} {proportional_to} N {sup -{alpha}}, where N is the local buoyancy frequency. For all parameter values 0 {<=} {alpha} {<=} 3, we find the SPG, and subsequently the AMOC, to weaken in response to increasing CO{sub 2} concentrations. The weakening is significantly stronger for {alpha} {>=} {alpha}{sub cr} {approx} 1.5. Depending on the value of {alpha}, two separate model states develop. These states remain different after the CO{sub 2} concentration is stabilized, and in some cases even after the CO{sub 2} concentration has been decreased again to the pre-industrial level. This behaviour is explained by a positive feedback between stratification and mixing anomalies in the Nordic Seas, causing a persistent weakening of the SPG. (orig.)

  3. Tracheostomy in neurologically compromised paediatric patients: role of starplasty.

    Science.gov (United States)

    Gupta, A; Stokken, J; Krakovitz, P; Malhotra, P; Anne, S

    2015-10-01

    Starplasty tracheostomy is an alternative to traditional tracheostomy. This paper reviews neurologically compromised paediatric patients with tracheostomies and discusses the role of starplasty tracheostomy. A retrospective review was conducted of paediatric patients with a neurological disorder who underwent tracheostomy between 1997 and 2011. Forty-eight patients, with an average age of 7.3 years, were identified. The most common indications for tracheostomy were: ventilator dependence (39.6 per cent), an inability to tolerate secretions or recurrent aspiration pneumonia (33.3 per cent), and upper respiratory obstruction or hypotonia (12.5 per cent). The most common underlying neurological diagnosis was cerebral palsy. There were no early complications. Eighteen (43 per cent) of 42 patients with follow up experienced at least 1 delayed complication. Only 12 patients (28.6 per cent) were decannulated. Patients with primary neurological diagnoses have low rates of decannulation; starplasty tracheostomy should be considered for these patients. Patients with seizure disorder or acute neurological injury tended to have a higher short-term decannulation rate; traditional tracheostomy is recommended in these patients.

  4. Differential responses of neuronal and spermatogenic cells to the doppel cytotoxicity.

    Directory of Open Access Journals (Sweden)

    Kefeng Qin

    Full Text Available Although structurally and biochemically similar to the cellular prion (PrP(C, doppel (Dpl is unique in its biological functions. There are no reports about any neurodegenerative diseases induced by Dpl. However the artificial expression of Dpl in the PrP-deficient mouse brain causes ataxia with Purkinje cell death. Abundant Dpl proteins have been found in testis and depletion of the Dpl gene (Prnd causes male infertility. Therefore, we hypothesize different regulations of Prnd in the nerve and male productive systems. In this study, by electrophoretic mobility shift assays we have determined that two different sets of transcription factors are involved in regulation of the Prnd promoter in mouse neuronal N2a and GC-1 spermatogenic (spg cells, i.e., upstream stimulatory factors (USF in both cells, Brn-3 and Sp1 in GC-1 spg cells, and Sp3 in N2a cells, leading to the expression of Dpl in GC-1 spg but not in N2a cells. We have further defined that, in N2a cells, Dpl induces oxidative stress and apoptosis, which stimulate ataxia-telangiectasia mutated (ATM-modulating bindings of transcription factors, p53 and p21, to Prnp promoter, resulting the PrP(C elevation for counteraction of the Dpl cytotoxicity; in contrast, in GC-1 spg cells, phosphorylation of p21 and N-terminal truncated PrP may play roles in the control of Dpl-induced apoptosis, which may benefit the physiological function of Dpl in the male reproduction system.

  5. Streptomyces songpinggouensis sp. nov., a Novel Actinomycete Isolated from Soil in Sichuan, China.

    Science.gov (United States)

    Guan, Xuejiao; Li, Wenchao; Liu, Chongxi; Jin, Pinjiao; Guo, Siyu; Wang, Xiangjing; Xiang, Wensheng

    2016-12-01

    During a screening for novel and biotechnologically useful actinobacteria, a novel actinobacteria with weak antifungal activity, designated strain NEAU-Spg19 T , was isolated from a soil sample collected from pine forest in Songpinggou, Sichuan, southwest China. The strain was characterized using a polyphasic taxonomic approach which confirmed that it belongs to the genus Streptomyces. Growth occurred at a temperature range of 10-30 °C, pH 5.0-11.0 and NaCl concentrations of 0-5 %. The cell wall peptidoglycan consisted of LL-diaminopimelic acid and glycine. The major menaquinones were MK-9(H 6 ), MK-9(H 8 ) and MK-9(H 4 ). The phospholipid profile contained diphosphatidylglycerol (DPG), phosphatidylethanolamine and phosphatidylinositol. The major fatty acids were iso-C 15:0 , iso-C 16:0 , and C 16:0 . 16S rRNA gene sequence similarity studies showed that strain NEAU-Spg19 T belongs to the genus Streptomyces with the highest sequence similarities to Streptomyces tauricus JCM 4837 T (98.6 %) and Streptomyces rectiviolaceus JCM 9092 T (98.3 %). Some physiological and biochemical properties and low DNA-DNA relatedness values enabled the strain to be differentiated from S. tauricus JCM 4837 T and S. rectiviolaceus JCM 9092 T . Hence, on the basis of phenotypic and genetic analyses, it is proposed that strain NEAU-Spg19 T represents a novel species of the genus Streptomyces, for which the name Streptomyces songpinggouensis sp. nov. is proposed. The type strain is NEAU-Spg19 T (=CGMCC 4.7140 T =DSM 42141 T ).

  6. MRI contrast agent for targeting glioma: interleukin-13 labeled liposome encapsulating gadolinium-DTPA.

    Science.gov (United States)

    Liu, Xiaoli; Madhankumar, Achuthamangalam B; Miller, Patti A; Duck, Kari A; Hafenstein, Susan; Rizk, Elias; Slagle-Webb, Becky; Sheehan, Jonas M; Connor, James R; Yang, Qing X

    2016-05-01

    Detection of glioma with MRI contrast agent is limited to cases in which the blood-brain barrier (BBB) is compromised as contrast agents cannot cross the BBB. Thus, an early-stage infiltrating tumor is not detectable. Interleukin-13 receptor alpha 2 (IL-13Rα2), which has been shown to be overexpressed in glioma, can be used as a target moiety. We hypothesized that liposomes conjugated with IL-13 and encapsulating MRI contrast agent are capable of passing through an intact BBB and producing MRI contrast with greater sensitivity. The targeted MRI contrast agent was created by encapsulating Magnevist (Gd-DTPA) into liposomes conjugated with IL-13 and characterized by particle size distribution, cytotoxicity, and MRI relaxivity. MR image intensity was evaluated in the brain in normal mice post injection of Gd-DTPA and IL-13-liposome-Gd-DTPA one day apart. The specificity for glioma detection by IL-13-liposome-Gd-DTPA was demonstrated in an intracranial glioma mouse model and validated histologically. The average size of IL-13-liposome-Gd-DTPA was 137 ± 43 nm with relaxivity of 4.0 ± 0.4 L/mmole-s at 7 Tesla. No significant cytotoxicity was observed with MTS assay and serum chemistry in mice. The MRI signal intensity was enhanced up to 15% post injection of IL-13-liposome-Gd-DTPA in normal brain tissue following a similar time course as that for the pituitary gland outside of the BBB. MRI enhanced by IL-13-liposome-Gd-DTPA detected small tumor masses in addition to those seen with Magnevist-enhanced MRI. IL-13-liposome-Gd-DTPA is able to pass through the uncompromised BBB and detect an early stage glioma that cannot be seen with conventional contrast-enhanced MRI. © The Author(s) 2015. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  7. Autophagy Deficiency Compromises Alternative Pathways of Respiration following Energy Deprivation in Arabidopsis thaliana.

    Science.gov (United States)

    Barros, Jessica A S; Cavalcanti, João Henrique F; Medeiros, David B; Nunes-Nesi, Adriano; Avin-Wittenberg, Tamar; Fernie, Alisdair R; Araújo, Wagner L

    2017-09-01

    Under heterotrophic conditions, carbohydrate oxidation inside the mitochondrion is the primary energy source for cellular metabolism. However, during energy-limited conditions, alternative substrates are required to support respiration. Amino acid oxidation in plant cells plays a key role in this by generating electrons that can be transferred to the mitochondrial electron transport chain via the electron transfer flavoprotein/ubiquinone oxidoreductase system. Autophagy, a catabolic mechanism for macromolecule and protein recycling, allows the maintenance of amino acid pools and nutrient remobilization. Although the association between autophagy and alternative respiratory substrates has been suggested, the extent to which autophagy and primary metabolism interact to support plant respiration remains unclear. To investigate the metabolic importance of autophagy during development and under extended darkness, Arabidopsis ( Arabidopsis thaliana ) mutants with disruption of autophagy ( atg mutants) were used. Under normal growth conditions, atg mutants showed lower growth and seed production with no impact on photosynthesis. Following extended darkness, atg mutants were characterized by signatures of early senescence, including decreased chlorophyll content and maximum photochemical efficiency of photosystem II coupled with increases in dark respiration. Transcript levels of genes involved in alternative pathways of respiration and amino acid catabolism were up-regulated in atg mutants. The metabolite profiles of dark-treated leaves revealed an extensive metabolic reprogramming in which increases in amino acid levels were partially compromised in atg mutants. Although an enhanced respiration in atg mutants was observed during extended darkness, autophagy deficiency compromises protein degradation and the generation of amino acids used as alternative substrates to the respiration. © 2017 American Society of Plant Biologists. All Rights Reserved.

  8. The Sub-Polar Gyre Index - a community data set for application in fisheries and environment research

    DEFF Research Database (Denmark)

    Berx, Barbara; Payne, Mark

    2017-01-01

    Scientific interest in the sub-polar gyre of the North Atlantic Ocean has increased in recent years. The sub-polar gyre has contracted and weakened, and changes in circulation pathways have been linked to changes in marine ecosystem productivity. To aid fisheries and environmental scientists, we...... series length are explored but found not to be important factors in terms of the SPG-I's interpretation. Our time series compares well with indices presented previously. The SPG-I time series is freely available online (http://dx.doi.org/10.7489/1806-1), and we invite the community to access, apply...

  9. Safety and Efficacy of Alginate Adhesion Barrier Gel in Compromised Intestinal Anastomosis.

    Science.gov (United States)

    Chaturvedi, Ankit A; Yauw, Simon T K; Lomme, Roger M L M; Hendriks, Thijs; van Goor, Harry

    For any anti-adhesive barrier developed for abdominal surgery, the use under conditions in which anastomotic healing is compromised needs to be investigated. The current study evaluates the effect of a new ultrapure alginate gel on early healing of high-risk anastomoses in the ileum and compares this with the gold standard used in clinical practice. In 75 adult male Wistar rats, a 5 mm ileal segment was resected and continuity was restored by construction of an inverted anastomosis. Rats were divided randomly into a control group and groups receiving either alginate gel or a sodium hyaluronate carboxymethylcellulose (HA/CMC) film around the anastomosis (n = 25 each). Carprofen, given in a daily dose of 1.25 mg/kg, was used to compromise anastomotic healing. At day three, animals were killed and scored for signs of anastomotic leakage and the presence of adhesions. The incidence of adhesion formation was 95% in the HA/CMC film group, which was significantly higher than in the controls (64%, p = 0.010) and the alginate gel group (52%, p = 0.004). The adhesion score was nearly 40% lower in the alginate gel group compared with the HA/CMC film group. The incidence of ileal leakage in the HA/CMC film group (92%) was significantly higher than in the controls (68%, p = 0.016). Leakage rate did not differ between the alginate gel and control groups. There was no significant difference between groups in either incision bursting pressure or incision breaking strength. Ultrapure alginate gel does not interfere with repair of ileal anastomoses constructed under conditions in which chances of anastomotic dehiscence are high. The alginate gel performs better than the HA/CMC film.

  10. Phenotypic compromises in a long-distance migrant during the transition from migration to reproduction in the High Arctic

    NARCIS (Netherlands)

    Vezina, Francois; Williams, Tony D.; Piersma, Theunis; Morrison, R. I. Guy; Dawson, Alistair

    1. Seasonal carry-over effects may be important structuring components of avian life-history cycles. However, little is known on physiological transitions between stages and on phenotypic compromises that may be made at such time to improve fitness. 2. We studied postmigratory body remodelling in

  11. Identifying compromised systems through correlation of suspicious traffic from malware behavioral analysis

    Science.gov (United States)

    Camilo, Ana E. F.; Grégio, André; Santos, Rafael D. C.

    2016-05-01

    Malware detection may be accomplished through the analysis of their infection behavior. To do so, dynamic analysis systems run malware samples and extract their operating system activities and network traffic. This traffic may represent malware accessing external systems, either to steal sensitive data from victims or to fetch other malicious artifacts (configuration files, additional modules, commands). In this work, we propose the use of visualization as a tool to identify compromised systems based on correlating malware communications in the form of graphs and finding isomorphisms between them. We produced graphs from over 6 thousand distinct network traffic files captured during malware execution and analyzed the existing relationships among malware samples and IP addresses.

  12. Protecting Privacy of Shared Epidemiologic Data without Compromising Analysis Potential

    Directory of Open Access Journals (Sweden)

    John Cologne

    2012-01-01

    Full Text Available Objective. Ensuring privacy of research subjects when epidemiologic data are shared with outside collaborators involves masking (modifying the data, but overmasking can compromise utility (analysis potential. Methods of statistical disclosure control for protecting privacy may be impractical for individual researchers involved in small-scale collaborations. Methods. We investigated a simple approach based on measures of disclosure risk and analytical utility that are straightforward for epidemiologic researchers to derive. The method is illustrated using data from the Japanese Atomic-bomb Survivor population. Results. Masking by modest rounding did not adequately enhance security but rounding to remove several digits of relative accuracy effectively reduced the risk of identification without substantially reducing utility. Grouping or adding random noise led to noticeable bias. Conclusions. When sharing epidemiologic data, it is recommended that masking be performed using rounding. Specific treatment should be determined separately in individual situations after consideration of the disclosure risks and analysis needs.

  13. Protecting Privacy of Shared Epidemiologic Data without Compromising Analysis Potential

    International Nuclear Information System (INIS)

    Cologne, J.; Nakashima, E.; Funamoto, S.; Grant, E.J.; Chen, Y.; Hiroaki Katayama, H.

    2012-01-01

    Objective. Ensuring privacy of research subjects when epidemiologic data are shared with outside collaborators involves masking (modifying) the data, but over masking can compromise utility (analysis potential). Methods of statistical disclosure control for protecting privacy may be impractical for individual researchers involved in small-scale collaborations. Methods. We investigated a simple approach based on measures of disclosure risk and analytical utility that are straightforward for epidemiologic researchers to derive. The method is illustrated using data from the Japanese Atomic-bomb Survivor population. Results. Masking by modest rounding did not adequately enhance security but rounding to remove several digits of relative accuracy effectively reduced the risk of identification without substantially reducing utility. Grouping or adding random noise led to noticeable bias. Conclusions. When sharing epidemiologic data, it is recommended that masking be performed using rounding. Specific treatment should be determined separately in individual situations after consideration of the disclosure risks and analysis needs

  14. Phenotypic compromises in a long-distance migrant during the transition from migration to reproduction in the High Arctic

    NARCIS (Netherlands)

    Vézina, F.; Williams, T.D.; Piersma, T.; Morrison, R.I.G.

    2012-01-01

    1. Seasonal carry-over effects may be important structuring components of avian life-history cycles. However, little is known on physiological transitions between stages and on phenotypic compromises that may be made at such time to improve fitness.
    2. We studied postmigratory body remodelling in

  15. The CarbonTracker Data Assimilation System for CO2 and δ13C (CTDAS-C13 v1.0): retrieving information on land-atmosphere exchange processes

    Science.gov (United States)

    van der Velde, Ivar R.; Miller, John B.; van der Molen, Michiel K.; Tans, Pieter P.; Vaughn, Bruce H.; White, James W. C.; Schaefer, Kevin; Peters, Wouter

    2018-01-01

    To improve our understanding of the global carbon balance and its representation in terrestrial biosphere models, we present here a first dual-species application of the CarbonTracker Data Assimilation System (CTDAS). The system's modular design allows for assimilating multiple atmospheric trace gases simultaneously to infer exchange fluxes at the Earth surface. In the prototype discussed here, we interpret signals recorded in observed carbon dioxide (CO2) along with observed ratios of its stable isotopologues 13CO2/12CO2 (δ13C). The latter is in particular a valuable tracer to untangle CO2 exchange from land and oceans. Potentially, it can also be used as a proxy for continent-wide drought stress in plants, largely because the ratio of 13CO2 and 12CO2 molecules removed from the atmosphere by plants is dependent on moisture conditions.The dual-species CTDAS system varies the net exchange fluxes of both 13CO2 and CO2 in ocean and terrestrial biosphere models to create an ensemble of 13CO2 and CO2 fluxes that propagates through an atmospheric transport model. Based on differences between observed and simulated 13CO2 and CO2 mole fractions (and thus δ13C) our Bayesian minimization approach solves for weekly adjustments to both net fluxes and isotopic terrestrial discrimination that minimizes the difference between observed and estimated mole fractions.With this system, we are able to estimate changes in terrestrial δ13C exchange on seasonal and continental scales in the Northern Hemisphere where the observational network is most dense. Our results indicate a decrease in stomatal conductance on a continent-wide scale during a severe drought. These changes could only be detected after applying combined atmospheric CO2 and δ13C constraints as done in this work. The additional constraints on surface CO2 exchange from δ13C observations neither affected the estimated carbon fluxes nor compromised our ability to match observed CO2 variations. The prototype presented

  16. Identifying and Embedding Common Indicators of Compromise in Virtual Machines for Lab-Based Incident Response Education

    Science.gov (United States)

    2015-09-01

    as an ever more popular method of exploiting hosts. Because the Internet is used for things like ecommerce , trading, voting, government, services...HTTP hypertext transfer protocol IE Internet Explorer IMAP Internet message access protocol IOC indicators of compromise IP Internet protocol MD5...notification of detection. These questions not only deal with how an incident is detected, but with the detection system itself. Things like whether

  17. Paraplegia após revascularização cirúrgica do miocárdio: relato de caso Paraplejia después de la revascularización quirúrgica del miocardio: relato de caso Paraplegia after myocardial revascularization: case report

    Directory of Open Access Journals (Sweden)

    Caetano Nigro Neto

    2010-04-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: Os avanços ocorridos na Anestesiologia permitiram melhores índices de segurança. Várias técnicas e agentes são utilizados visando controlar a resposta hemodinâmica e minimizar os efeitos adversos do estimulo cirúrgico em pacientes submetidos a procedimentos cardíacos. RELATO DO CASO: Paciente de 70 anos, masculino, 1,74 m, 75 kg, ASA III, e NYHA II. Portador de dislipidemia, diabetes mellitus tipo II e hipertensão arterial controladas; tabagismo, enfermidade vascular periférica e história de infarto agudo do miocárdio há 20 anos. O paciente foi submetido a revascularização com artéria mamária interna esquerda e enxertos de safena com pinçamento intermitente da aorta em circulação extracorpórea. Durante as primeiras 24 horas na UTI apresentou eventos de instabilidade hemodinâmica, hipotensão súbita e fibrilação atrial. Após 26 horas do final do procedimento cirúrgico, o paciente estava acordado, hemodinamicamente estável e com boa dinâmica respiratória quando foi extubado. O paciente estava comunicativo, orientado, porém com imobilidade e reflexos abolidos nos membros inferiores. Na avaliação neurológica: pares de nervos cranianos sem alteração, ausência de queixa de dor de qualquer tipo da cintura pélvica para baixo, preservação da sensibilidade superficial e profunda, perfusão distal adequada sem edemas, e paraplegia flácida na região abaixo de T8. Exame de ecocardiografia sem alterações. A tomografia computadorizada da coluna lombo-sacra, não mostrou massa compressiva no espaço epidural ou adjacente. CONCLUSÃO: A síndrome da artéria espinhal anterior, deve ser sempre considerada nos procedimentos de manipulação da aorta. A prevenção, particularmente nos pacientes de risco é necessária. A tomografia computadorizada é importante para o diagnóstico diferencial e a ressonância magnética para a localização da lesão.JUSTIFICATIVA Y OBJETIVOS: En las últimas

  18. Compromised careers: the occupational transition of immigration and resettlement.

    Science.gov (United States)

    Suto, Melinda

    2009-01-01

    Work is a significant occupational transition that occurs with immigration and resettlement. Problems finding work and regaining economic capital are multi-factorial, differentiated by gender and mediated by specific contexts. Surprisingly, past education and work experience are unreliable predictors of successful employment outcomes. Critical theory and ethnographic concepts informed the methodological approach. Data were generated primarily through in-depth interviews, conducted in English, with 14 well-educated women who immigrated to Canada as adults and sought employment in their professions. The thematic findings were analyzed using Bourdieu's [7] concepts of capital, field and habitus. The theme Compromised Careers describes the downward occupational (work) mobility that occurs despite expectations that education, credentials and work experience are transferable to desirable employment. A devaluation of foreign qualifications and no relevant Canadian work experience function with gendered responsibilities, less social support, and time spent in resettlement activities to create negative work trajectories. The role that federal policies and professional organizations play is examined to reveal the tension between individuals' efforts to find employment and institutional barriers that impede these actions. A critical inquiry approach examined the ruling relations to show how power and privilege function in relation to migrants' occupational transitions.

  19. RNA interference targeting carbohydrate sulfotransferase 3 diminishes macrophage accumulation, inhibits MMP-9 expression and promotes lung recovery in murine pulmonary emphysema.

    Science.gov (United States)

    Kai, Yoshiro; Tomoda, Koichi; Yoneyama, Hiroyuki; Yoshikawa, Masanori; Kimura, Hiroshi

    2015-12-09

    Chondroitin sulfate proteoglycans are an important mediators in inflammation and leukocyte trafficking. However, their roles in pulmonary emphysema have not been explored. In a murine model of elastase-induced pulmonary emphysema, we found increased carbohydrate sulfotransferase 3 (CHST3), a specific enzyme that synthesizes chondroitin 6-sulfate proteoglycan (C6SPG). To elucidate the role of C6SPG, we investigated the effect of small interfering RNA (siRNA) targeting CHST3 that inhibits C6SPG-synthesis on the pathogenesis of pulmonary emphysema. Mice were intraperitoneally injected with CHST3 siRNA or negative control siRNA on day0 and 7 after intratracheal instillation of elastase. Histology, respiratory function, glycosaminoglycans (GAGs) content, bronchoalveolar lavage (BAL), elastin staining and gene expressions of tumor necrosis factor (TNF)-α and matrix metalloproteinase (MMP)-9 mRNA were evaluated on day7 and/or day21. CHST3 mRNA increased at day 7 and decreased thereafter in lung. CHST3 siRNA successfully inhibited the expression of CHST3 mRNA throughout the study and this was associated with significant reduction of GAGs and C6SPG. Airway destruction and respiratory function were improved by the treatment with CHST3 siRNA. CHST3 siRNA reduced the number of macrophages both in BAL and lung parenchyma and also suppressed the increased expressions of TNF-α and MMP-9 mRNA. Futhermore, CHST3 siRNA improved the reduction of the elastin in the alveolar walls. CHST3 siRNA diminishes accumulation of excessive macrophages and the mediators, leading to accelerate the functional recovery from airway damage by repair of the elastin network associated with pulmonary emphysema.

  20. Design and characterization of 3D hybrid collagen matrixes as a dermal substitute in skin tissue engineering

    Energy Technology Data Exchange (ETDEWEB)

    Ramanathan, Giriprasath; Singaravelu, Sivakumar [Bioproducts Lab, CSIR-Central Leather Research Institute, Chennai 600020, Tamilnadu (India); Muthukumar, Thangavelu [Department of Physiology, College of Veterinary Medicine, Chonbuk National University, 79 Gobong-ro, Iksan-city, Jeollabuk-Do 570-752 (Korea, Republic of); Thyagarajan, Sitalakshmi [Bioproducts Lab, CSIR-Central Leather Research Institute, Chennai 600020, Tamilnadu (India); Perumal, Paramasivan Thirumalai [Organic Chemistry Division, CSIR-Central Leather Research Institute, Adyar, Chennai, 600020, Tamilnadu (India); Sivagnanam, Uma Tiruchirapalli, E-mail: suma67@gmail.com [Bioproducts Lab, CSIR-Central Leather Research Institute, Chennai 600020, Tamilnadu (India)

    2017-03-01

    The highly interconnected porous dressing material was fabricated with the utilization of novel collagen (COL-SPG) for the efficient healing of the wound. Herein, we report the fabrication of 3D collagen impregnated with bioactive extract (COL-SPG-CPE) to get rid of infection at the wound site. The resultant 3D collagen matrix was characterized physiochemically using Fourier transform infrared spectroscopy (FTIR), scanning electron microscopy (SEM) and mechanical property. The dressing substrate possesses the high swelling ability, increase in the porosity, in vitro enzymatic degradability and antibacterial property. The in vitro biocompatibility and fluorescence activity of the collagen scaffold against both NIH 3T3 fibroblast and Human keratinocyte (HaCaT) cell lines assisted in excellent cell adhesion and proliferation over the collagen matrix. Furthermore, the in vivo evaluation of the COL-SPG-CPE 3D sponge exhibited with enhanced collagen synthesis and aids in faster reepithelialization. However, the rate of wound healing was influenced by the expression of vascular endothelial growth factor (VEGF), epidermal growth factor (EGF) and transforming growth factor (TGF-β) growth factors promotes the collagen synthesis, thereby increases the healing efficiency. Based on the results, COL-SPG-CPE has a potential ability in the remodeling of the wound with the 3D collagen as wound dressing material. - Highlights: • Fabrication of highly interconnected 3D collagen scaffold as a wound construct • The 3D collagen matrix mimics the function of the extra cellular matrix. • Biocompatibility was assessed with fibroblast and keratinocytes by MTT assay. • Bioactive extract aides good mechanical properties and antimicrobial activity. • In vivo evaluation exhibited efficient wound construct for rapid wound healing.

  1. Design and characterization of 3D hybrid collagen matrixes as a dermal substitute in skin tissue engineering

    International Nuclear Information System (INIS)

    Ramanathan, Giriprasath; Singaravelu, Sivakumar; Muthukumar, Thangavelu; Thyagarajan, Sitalakshmi; Perumal, Paramasivan Thirumalai; Sivagnanam, Uma Tiruchirapalli

    2017-01-01

    The highly interconnected porous dressing material was fabricated with the utilization of novel collagen (COL-SPG) for the efficient healing of the wound. Herein, we report the fabrication of 3D collagen impregnated with bioactive extract (COL-SPG-CPE) to get rid of infection at the wound site. The resultant 3D collagen matrix was characterized physiochemically using Fourier transform infrared spectroscopy (FTIR), scanning electron microscopy (SEM) and mechanical property. The dressing substrate possesses the high swelling ability, increase in the porosity, in vitro enzymatic degradability and antibacterial property. The in vitro biocompatibility and fluorescence activity of the collagen scaffold against both NIH 3T3 fibroblast and Human keratinocyte (HaCaT) cell lines assisted in excellent cell adhesion and proliferation over the collagen matrix. Furthermore, the in vivo evaluation of the COL-SPG-CPE 3D sponge exhibited with enhanced collagen synthesis and aids in faster reepithelialization. However, the rate of wound healing was influenced by the expression of vascular endothelial growth factor (VEGF), epidermal growth factor (EGF) and transforming growth factor (TGF-β) growth factors promotes the collagen synthesis, thereby increases the healing efficiency. Based on the results, COL-SPG-CPE has a potential ability in the remodeling of the wound with the 3D collagen as wound dressing material. - Highlights: • Fabrication of highly interconnected 3D collagen scaffold as a wound construct • The 3D collagen matrix mimics the function of the extra cellular matrix. • Biocompatibility was assessed with fibroblast and keratinocytes by MTT assay. • Bioactive extract aides good mechanical properties and antimicrobial activity. • In vivo evaluation exhibited efficient wound construct for rapid wound healing.

  2. The CarbonTracker Data Assimilation System for CO2 and δ13C (CTDAS-C13 v1.0: retrieving information on land–atmosphere exchange processes

    Directory of Open Access Journals (Sweden)

    I. R. van der Velde

    2018-01-01

    Full Text Available To improve our understanding of the global carbon balance and its representation in terrestrial biosphere models, we present here a first dual-species application of the CarbonTracker Data Assimilation System (CTDAS. The system's modular design allows for assimilating multiple atmospheric trace gases simultaneously to infer exchange fluxes at the Earth surface. In the prototype discussed here, we interpret signals recorded in observed carbon dioxide (CO2 along with observed ratios of its stable isotopologues 13CO2∕12CO2 (δ13C. The latter is in particular a valuable tracer to untangle CO2 exchange from land and oceans. Potentially, it can also be used as a proxy for continent-wide drought stress in plants, largely because the ratio of 13CO2 and 12CO2 molecules removed from the atmosphere by plants is dependent on moisture conditions.The dual-species CTDAS system varies the net exchange fluxes of both 13CO2 and CO2 in ocean and terrestrial biosphere models to create an ensemble of 13CO2 and CO2 fluxes that propagates through an atmospheric transport model. Based on differences between observed and simulated 13CO2 and CO2 mole fractions (and thus δ13C our Bayesian minimization approach solves for weekly adjustments to both net fluxes and isotopic terrestrial discrimination that minimizes the difference between observed and estimated mole fractions.With this system, we are able to estimate changes in terrestrial δ13C exchange on seasonal and continental scales in the Northern Hemisphere where the observational network is most dense. Our results indicate a decrease in stomatal conductance on a continent-wide scale during a severe drought. These changes could only be detected after applying combined atmospheric CO2 and δ13C constraints as done in this work. The additional constraints on surface CO2 exchange from δ13C observations neither affected the estimated carbon fluxes nor compromised our ability to match observed CO2 variations

  3. APORTES TEÓRICOS E METODOLÓGICOS PARA A CONSTITUIÇÃO DE UM GRUPO DE PLANEJAMENTO CONJUNTO COM DOCENTES DA LICENCIATURA EM FÍSICA

    Directory of Open Access Journals (Sweden)

    João Ricardo Neves da Silva

    2014-01-01

    Full Text Available This paper presents share of a research results with the intention of promoting an interactive communicative room among teachers with degree in physics therefore planning their courses together. From the theoretical recommendations acquired on Theory of Communicative Action, by Jürgen Habermas, it was possible to draw a rationality background crisis on training degree related to physics in Brazil in addition to purpose the creation of a Set Planning Group (SPG to teachers of knowledge branch specifically in physics and disciplines about physics teaching. There was a debate about the relationship among disciplines for delivering a project to future high school teacher formation. All meetings held over a school year on SPC was recorded, transcribed and analyzed with a part of a methodological analysis feature of the intersubjective interactions. This methodology was developed in particular for the research. The conclusions related to the constructions were developed by the SPG, as well as local contexts and the possibility of extension of this group. The constructions combined undertaken from the group point out a defense for creating a SPG academically educated physics degree teachers at the university who will instruct these courses.

  4. Accomplishments and Compromises in Prediction Research for World Records and Best Performances in Track and Field and Swimming

    Science.gov (United States)

    Liu, Yuanlong; Paul, Stanley; Fu, Frank H.

    2012-01-01

    The conductors of this study reviewed prediction research and studied the accomplishments and compromises in predicting world records and best performances in track and field and swimming. The results of the study showed that prediction research only promises to describe the historical trends in track and field and swimming performances, to study…

  5. ACCOUNTING – THE REZULT OF A COMPROMISE BETWEEN EXPECTATIONS AND EXIGENCIES

    Directory of Open Access Journals (Sweden)

    Mihaela TULVINSCHI

    2017-12-01

    Full Text Available Current concerns in the field of accounting are circumscribed to the need to perfect a unanimously accepted accounting language to be included in the application, as uniformly as possible, of the principles and accounting treatments as a basis in making financial statements. This approach is complicated and implies an extraordinary dynamics. The objective of the article is to investigate whether, in the current economic environment, accounting is able to issue an objective message to meet the expectations and exigencies of all users of financial information. Accounting can provide only a single representation of reality, and the information produced by accounting can only be a compromise between the respect for accounting principles and the care for reflecting economic reality. Accounting is an information system that quantifies, processes and transmits financial information about an economic entity. It establishes the link between economic activities and decision-makers.

  6. Macronutrient manipulations of cheese resulted in lower energy content without compromising its satiating capacity

    DEFF Research Database (Denmark)

    Hansen, Thea Toft; Sjödin, Anders Mikael; Ritz, Christian

    2018-01-01

    Manipulation of food’s macronutrient composition in order to reduce energy content without compromising satiating capacity may be helpful in body weight control. For cheeses, substituting fat with protein may provide such opportunity. We aimed at examining the acute effect of cheeses with different...... macronutrient compositions on accumulated energy intake and subjective appetite sensation. A total of thirty-nine normal-weight (average BMI 24·4 kg/m2) men and women completed the partly double-blind, randomised crossover study with high-protein/low-fat (HP/LF, 696 kJ), high-protein/high-fat (HP/HF, 976 k...

  7. CT-morphological characterization of respiratory syncytial virus (RSV) pneumonia in immune-compromised adults

    International Nuclear Information System (INIS)

    Mayer, J.L.; Kauczor, H.U.; Lehners, N.; Egerer, G.; Heussel, C.P.

    2014-01-01

    Characterization and follow-up evaluation of chest CT of RSV pneumonia in immune-compromised adults during a seasonal epidemic. Retrospective analysis of 132 chest CT examinations of 51 adult immune-compromised patients (29 m/22f, diameter 58 years) with clinical signs of pneumonia and positive RSV test in winter 2011/2012. Two experienced chest radiologists evaluated the morphology (bronchial wall thickening, tree-in-bud, nodules, halo, ground-glass opacities, consolidations, pleural fluid) of the CT scans by consensus. Pathological findings were in 86 % of the chest CT scans: Areas of ground-glass attenuation in 64 %, consolidations in 56 %, nodules in 55 % (diameter 8 mm in maximal diameter, with halo in 71 %), pleural fluid in 44 % (diameter 2 cm), tree-in-bud in 36 %, bronchial wall thickening in 27 % and more than one morphological finding in 72 %. There were no pathological CT findings in 14 % of patients with clinical symptoms of pneumonia because these patients did not undergo follow-up. Radiological progression was found in 45 % of patients and regression in 33 % in follow-up examinations. In 37 % an additional examination of the paranasal sinuses was performed and showed sinusitis in 63 % of cases. 90 % of the patients had sinusitis as well as pneumonia. In addition to RSV, a further pathogenic agent was found in bronchoalveolar lavage of five patients (Aspergillus spec., herpes simplex virus, Pseudomonas aeruginosa). The most characteristic signs in chest CT scans were at the beginning of pneumonia with nodules and tree-in-bud often combined with bronchial wall thickening. The following CT scans showed characteristic but not pathognomonic chest CT findings of RSV pneumonia. These morphological findings should be recognized seasonally (winter) especially at the beginning of the case of pneumonia. RSV-associated additional sinusitis is probably common and should be noticed.

  8. Staurosporine induces necroptotic cell death under caspase-compromised conditions in U937 cells.

    Directory of Open Access Journals (Sweden)

    Zsuzsanna A Dunai

    Full Text Available For a long time necrosis was thought to be an uncontrolled process but evidences recently have revealed that necrosis can also occur in a regulated manner. Necroptosis, a type of programmed necrosis is defined as a death receptor-initiated process under caspase-compromised conditions. The process requires the kinase activity of receptor-interacting protein kinase 1 and 3 (RIPK1 and RIPK3 and mixed lineage kinase domain-like protein (MLKL, as a substrate of RIPK3. The further downstream events remain elusive. We applied known inhibitors to characterize the contributing enzymes in necroptosis and their effect on cell viability and different cellular functions were detected mainly by flow cytometry. Here we report that staurosporine, the classical inducer of intrinsic apoptotic pathway can induce necroptosis under caspase-compromised conditions in U937 cell line. This process could be hampered at least partially by the RIPK1 inhibitor necrotstin-1 and by the heat shock protein 90 kDa inhibitor geldanamycin. Moreover both the staurosporine-triggered and the classical death ligand-induced necroptotic pathway can be effectively arrested by a lysosomal enzyme inhibitor CA-074-OMe and the recently discovered MLKL inhibitor necrosulfonamide. We also confirmed that the enzymatic role of poly(ADP-ribosepolymerase (PARP is dispensable in necroptosis but it contributes to membrane disruption in secondary necrosis. In conclusion, we identified a novel way of necroptosis induction that can facilitate our understanding of the molecular mechanisms of necroptosis. Our results shed light on alternative application of staurosporine, as a possible anticancer therapeutic agent. Furthermore, we showed that the CA-074-OMe has a target in the signaling pathway leading to necroptosis. Finally, we could differentiate necroptotic and secondary necrotic processes based on participation of PARP enzyme.

  9. HIV treatment as prevention: debate and commentary--will early infection compromise treatment-as-prevention strategies?

    Directory of Open Access Journals (Sweden)

    Myron S Cohen

    Full Text Available Universal HIV testing and immediate antiretroviral therapy for infected individuals has been proposed as a way of reducing the transmission of HIV and thereby bringing the HIV epidemic under control. It is unclear whether transmission during early HIV infection--before individuals are likely to have been diagnosed with HIV and started on antiretroviral therapy--will compromise the effectiveness of treatment as prevention. This article presents two opposing viewpoints by Powers, Miller, and Cohen, and Williams and Dye, followed by a commentary by Fraser.

  10. 20 CFR 408.950 - Will we accept a compromise settlement of an overpayment debt or suspend or terminate collection...

    Science.gov (United States)

    2010-04-01

    ...' Benefits SOCIAL SECURITY ADMINISTRATION SPECIAL BENEFITS FOR CERTAIN WORLD WAR II VETERANS Underpayments... (31 CFR 900.3 and parts 902 and 903). (c) Effect of compromise, suspension or termination. When we... accordance with provisions of the Social Security Act, other laws, and the standards set forth in 31 CFR...

  11. Treatment of chest burn contracture causing respiratory compromise with island release and grafting using cross-link collagen and Integra™ bilayer dressing

    Directory of Open Access Journals (Sweden)

    Neil Doctor

    2014-04-01

    Full Text Available Post-burn skin contractures of the anterior and lateral abdomen and chest may result in respiratory compromise due to limitation of rib excursion. This case report describes a young man with respiratory compromise limiting his daily activity and function, as a result of a 90% burn sustained 6 years previously. Release of his chest and upper abdomen was achieved using “island” scar releases and a cross-linked bovine tendon collagen and glycosaminoglycan and a semi-permeable polysiloxane bilayer matrix dressing (Integra™ followed by subsequent split thickness skin graft. An immediate increase in maximal inspiratory volume was obtained intra-operatively and in the im­mediate post-operative period, and this improvement was sustained after healing of all wounds with subjective relief of the patient’s symptoms.

  12. A panel study on patients with dominant cerebellar ataxia highlights the frequency of channelopathies.

    Science.gov (United States)

    Coutelier, Marie; Coarelli, Giulia; Monin, Marie-Lorraine; Konop, Juliette; Davoine, Claire-Sophie; Tesson, Christelle; Valter, Rémi; Anheim, Mathieu; Behin, Anthony; Castelnovo, Giovanni; Charles, Perrine; David, Albert; Ewenczyk, Claire; Fradin, Mélanie; Goizet, Cyril; Hannequin, Didier; Labauge, Pierre; Riant, Florence; Sarda, Pierre; Sznajer, Yves; Tison, François; Ullmann, Urielle; Van Maldergem, Lionel; Mochel, Fanny; Brice, Alexis; Stevanin, Giovanni; Durr, Alexandra

    2017-06-01

    Autosomal dominant cerebellar ataxias have a marked heterogeneous genetic background, with mutations in 34 genes identified so far. This large amount of implicated genes accounts for heterogeneous clinical presentations, making genotype-phenotype correlations a major challenge in the field. While polyglutamine ataxias, linked to CAG repeat expansions in genes such as ATXN1, ATXN2, ATXN3, ATXN7, CACNA1A and TBP, have been extensively characterized in large cohorts, there is a need for comprehensive assessment of frequency and phenotype of more 'conventional' ataxias. After exclusion of CAG/polyglutamine expansions in spinocerebellar ataxia genes in 412 index cases with dominantly inherited cerebellar ataxias, we aimed to establish the relative frequencies of mutations in other genes, with an approach combining panel sequencing and TaqMan® polymerase chain reaction assay. We found relevant genetic variants in 59 patients (14.3%). The most frequently mutated were channel genes [CACNA1A (n = 16), KCND3 (n = 4), KCNC3 (n = 2) and KCNA1 (n = 2)]. Deletions in ITPR1 (n = 11) were followed by biallelic variants in SPG7 (n = 9). Variants in AFG3L2 (n = 7) came next in frequency, and variants were rarely found in STBN2 (n = 2), ELOVL5, FGF14, STUB1 and TTBK2 (n = 1 each). Interestingly, possible risk factor variants were detected in SPG7 and POLG. Clinical comparisons showed that ataxias due to channelopathies had a significantly earlier age at onset with an average of 24.6 years, versus 40.9 years for polyglutamine expansion spinocerebellar ataxias and 37.8 years for SPG7-related forms (P = 0.001). In contrast, disease duration was significantly longer in the former (20.5 years versus 9.3 and 13.7, P=0.001), though for similar functional stages, indicating slower progression of the disease. Of interest, intellectual deficiency was more frequent in channel spinocerebellar ataxias, while cognitive impairment in adulthood was similar among the three groups. Similar

  13. Controlling the fluoride dosage in a patient with compromised salivary function.

    Science.gov (United States)

    Eichmiller, Frederick C; Eidelman, Naomi; Carey, Clifton M

    2005-01-01

    High-concentration topical fluorides are used commonly to with compromised salivary function due to irradiation and chemotherapy. The authors describe a 50-year-old man with previously treated cancer who was using tray-applied topical fluoride gel. He complained of gastric symptoms, difficulty in swallowing, leg muscle soreness and knee joint soreness. A computed tomographic scan revealed thickening of the esophageal walls. An upper endoscopy revealed abnormal motility. The motility test indicated high-amplitude peristalsis and hypertensive lower esophageal sphincter, and urine testing indicated high levels of systemic fluoride. The patient's fluoride regimen was altered, and within a short period his urinary fluoride levels returned to normal and his symptoms resolved. Clinicians prescribing home-applied high-concentration fluorides need to be cognizant of the symptoms of fluoride toxicity, carefully monitor the patient's compliance with the treatment regimen, and adjust the dosage or mode of application to control the total ingested dose of fluoride.

  14. Risk-Controlled Multiobjective Portfolio Selection Problem Using a Principle of Compromise

    Directory of Open Access Journals (Sweden)

    Takashi Hasuike

    2014-01-01

    Full Text Available This paper proposes a multiobjective portfolio selection problem with most probable random distribution derived from current market data and other random distributions of boom and recession under the risk-controlled parameters determined by an investor. The current market data and information include not only historical data but also interpretations of economists’ oral and linguistic information, and hence, the boom and recession are often caused by these nonnumeric data. Therefore, investors need to consider several situations from most probable condition to boom and recession and to avoid the risk less than the target return in each situation. Furthermore, it is generally difficult to set random distributions of these cases exactly. Therefore, a robust-based approach for portfolio selection problems using the only mean values and variances of securities is proposed as a multiobjective programming problem. In addition, an exact algorithm is developed to obtain an explicit optimal portfolio using a principle of compromise.

  15. Full-field initialized decadal predictions with the MPI earth system model: an initial shock in the North Atlantic

    Science.gov (United States)

    Kröger, Jürgen; Pohlmann, Holger; Sienz, Frank; Marotzke, Jochem; Baehr, Johanna; Köhl, Armin; Modali, Kameswarrao; Polkova, Iuliia; Stammer, Detlef; Vamborg, Freja S. E.; Müller, Wolfgang A.

    2017-12-01

    Our decadal climate prediction system, which is based on the Max-Planck-Institute Earth System Model, is initialized from a coupled assimilation run that utilizes nudging to selected state parameters from reanalyses. We apply full-field nudging in the atmosphere and either full-field or anomaly nudging in the ocean. Full fields from two different ocean reanalyses are considered. This comparison of initialization strategies focuses on the North Atlantic Subpolar Gyre (SPG) region, where the transition from anomaly to full-field nudging reveals large differences in prediction skill for sea surface temperature and ocean heat content (OHC). We show that nudging of temperature and salinity in the ocean modifies OHC and also induces changes in mass and heat transports associated with the ocean flow. In the SPG region, the assimilated OHC signal resembles well OHC from observations, regardless of using full fields or anomalies. The resulting ocean transport, on the other hand, reveals considerable differences between full-field and anomaly nudging. In all assimilation runs, ocean heat transport together with net heat exchange at the surface does not correspond to OHC tendencies, the SPG heat budget is not closed. Discrepancies in the budget in the cases of full-field nudging exceed those in the case of anomaly nudging by a factor of 2-3. The nudging-induced changes in ocean transport continue to be present in the free running hindcasts for up to 5 years, a clear expression of memory in our coupled system. In hindcast mode, on annual to inter-annual scales, ocean heat transport is the dominant driver of SPG OHC. Thus, we ascribe a significant reduction in OHC prediction skill when using full-field instead of anomaly initialization to an initialization shock resulting from the poor initialization of the ocean flow.

  16. Reduction of Cone-Beam CT scan time without compromising the accuracy of the image registration in IGRT

    DEFF Research Database (Denmark)

    Westberg, Jonas; Jensen, Henrik R; Bertelsen, Anders

    2010-01-01

    In modern radiotherapy accelerators are equipped with 3D cone-beam CT (CBCT) which is used to verify patient position before treatment. The verification is based on an image registration between the CBCT acquired just before treatment and the CT scan made for the treatment planning. The purpose...... of this study is to minimise the scan time of the CBCT without compromising the accuracy of the image registration in IGRT....

  17. 26 CFR 13.0-13.3 - [Reserved

    Science.gov (United States)

    2010-04-01

    ... 26 Internal Revenue 14 2010-04-01 2010-04-01 false [Reserved] 13.0-13.3 Section 13.0-13.3 Internal Revenue INTERNAL REVENUE SERVICE, DEPARTMENT OF THE TREASURY (CONTINUED) INCOME TAX (CONTINUED) TEMPORARY INCOME TAX REGULATIONS UNDER THE TAX REFORM ACT OF 1969 §§ 13.0-13.3 [Reserved] ...

  18. The influence of verbal training and visual feedback on manual wheelchair propulsion.

    Science.gov (United States)

    DeGroot, Keri K; Hollingsworth, Holly H; Morgan, Kerri A; Morris, Carrie L; Gray, David B

    2009-03-01

    To determine if verbal training with visual feedback improved manual wheelchair propulsion; to examine propulsion differences between an individual with paraplegia and an individual with tetraplegia. Quasi-experimental study: Nine manual wheelchair-using adults participated in propulsion assessments and training. Baseline propulsion performance was measured on several tasks on different surfaces. Participants were trained on a wheelchair treadmill with verbal and visual feedback to increase push length, reduce push frequency and to modify propulsion pattern. Handrim biomechanics were measured with an instrumented wheel. Changes in propulsion were assessed. Differences in propulsion characteristics between a participant with paraplegia and a participant with tetraplegia were examined. Push length increased (p propulsion characteristics between a participant with paraplegia and a participant with tetraplegia. Verbal training may produce changes in push biomechanics of manual wheelchair users. Longer training periods may be needed to sustain propulsion changes. Findings from this study support other studies that have shown propulsion differences between people with tetraplegia and paraplegia. Propulsion training for populations with upper-extremity impairments warrants further study.

  19. Testing the effect of time pressure on asymmetric dominance and compromise decoys in choice

    Directory of Open Access Journals (Sweden)

    Jonathan Pettibone

    2012-07-01

    Full Text Available Dynamic, connectionist models of decision making, such as decision field theory (Roe, Busemeyer, and Townsend, 2001, propose that the effect of context on choice arises from a series of pairwise comparisons between attributes of alternatives across time. As such, they predict that limiting the amount of time to make a decision should decrease rather than increase the size of contextual effects. This prediction was tested across four levels of time pressure on both the asymmetric dominance (Huber, Payne, and Puto, 1982 and compromise (Simonson, 1989 decoy effects in choice. Overall, results supported this prediction, with both types of decoy effects found to be larger as time pressure decreased.

  20. IL-4/IL-13 Heteroreceptor Influences Th17 Cell Conversion and Sensitivity to Regulatory T Cell Suppression To Restrain Experimental Allergic Encephalomyelitis.

    Science.gov (United States)

    Barik, Subhasis; Ellis, Jason S; Cascio, Jason A; Miller, Mindy M; Ukah, Tobechukwu K; Cattin-Roy, Alexis N; Zaghouani, Habib

    2017-10-01

    IL-4 and IL-13 have been defined as anti-inflammatory cytokines that can counter myelin-reactive T cells and modulate experimental allergic encephalomyelitis. However, it is not known whether endogenous IL-4 and IL-13 contribute to the maintenance of peripheral tolerance and whether their function is coordinated with T regulatory cells (Tregs). In this study, we used mice in which the common cytokine receptor for IL-4 and IL-13, namely the IL-4Rα/IL-13Rα1 (13R) heteroreceptor (HR), is compromised and determined whether the lack of signaling by endogenous IL-4 and IL-13 through the HR influences the function of effector Th1 and Th17 cells in a Treg-dependent fashion. The findings indicate that mice-deficient for the HR (13R -/- ) are more susceptible to experimental allergic encephalomyelitis than mice sufficient for the HR (13R +/+ ) and develop early onset and more severe disease. Moreover, Th17 cells from 13R -/- mice had reduced ability to convert to Th1 cells and displayed reduced sensitivity to suppression by Tregs relative to Th17 effectors from 13R +/+ mice. These observations suggest that IL-4 and IL-13 likely operate through the HR and influence Th17 cells to convert to Th1 cells and to acquire increased sensitivity to suppression, leading to control of immune-mediated CNS inflammation. These previously unrecognized findings shed light on the intricacies underlying the contribution of cytokines to peripheral tolerance and control of autoimmunity. Copyright © 2017 by The American Association of Immunologists, Inc.

  1. The value of predicting restriction of fetal growth and compromise of its wellbeing: Systematic quantitative overviews (meta-analysis) of test accuracy literature.

    Science.gov (United States)

    Morris, Rachel K; Khan, Khalid S; Coomarasamy, Aravinthan; Robson, Stephen C; Kleijnen, Jos

    2007-03-08

    Restriction of fetal growth and compromise of fetal wellbeing remain significant causes of perinatal death and childhood disability. At present, there is a lack of scientific consensus about the best strategies for predicting these conditions before birth. Therefore, there is uncertainty about the best management of pregnant women who might have a growth restricted baby. This is likely to be due to a dearth of clear collated information from individual research studies drawn from different sources on this subject. A series of systematic reviews and meta-analyses will be undertaken to determine, among pregnant women, the accuracy of various tests to predict and/or diagnose fetal growth restriction and compromise of fetal wellbeing. We will search Medline, Embase, Cochrane Library, MEDION, citation lists of review articles and eligible primary articles and will contact experts in the field. Independent reviewers will select studies, extract data and assess study quality according to established criteria. Language restrictions will not be applied. Data synthesis will involve meta-analysis (where appropriate), exploration of heterogeneity and publication bias. The project will collate and synthesise the available evidence regarding the value of the tests for predicting restriction of fetal growth and compromise of fetal wellbeing. The systematic overviews will assess the quality of the available evidence, estimate the magnitude of potential benefits, identify those tests with good predictive value and help formulate practice recommendations.

  2. Upper airway stabilization by osteopathic manipulation of the sphenopalatine ganglion versus sham manipulation in OSAS patients: a proof-of-concept, randomized, crossover, double-blind, controlled study.

    Science.gov (United States)

    Jacq, Olivier; Arnulf, Isabelle; Similowski, Thomas; Attali, Valérie

    2017-12-20

    Osteopathic manipulative treatment (OMT) of the sphenopalatine ganglion (SPG) is used empirically for the treatment of rhinitis and snoring and is thought to increase pharyngeal stability. This trial was designed to study the effects of this treatment on pharyngeal stability evaluated by critical closing pressure in obstructive sleep apnoea syndrome. This single-centre, randomized, crossover, double-blind study compared active manipulation and sham manipulation of the SPG. Randomization was computer-generated. Patients each received one active manipulation and one sham manipulation at an interval of 21 days and were evaluated 30 min and 48 h after each session administered by a qualified osteopath. Neither the patients, nor the investigator performing the evaluations were informed about the order of the two techniques (double-blind). The primary endpoint was the percentage of responding patients presenting increased pharyngeal stability defined by a variation of critical closing pressure (Pcrit) of at least -4 cmH 2 O at 30 min. Secondary endpoints were the variation of Pcrit in absolute values, sleepiness and snoring. Others endpoints were lacrimation (Schirmer's test), induced pain, sensations experienced during OMT. Ten patients were included and nine (57 [50; 58] years, comprising 7 men, with an apnoea-hypopnoea index of 31.0 [25.5; 33.2]/h; (values are median [quartiles])) were analysed. Seven patients were analysed for the primary endpoint and nine patients were analysed for secondary endpoints. Five patients responded after active manipulation versus no patients after sham manipulation (p = 0.0209). Active manipulation induced more intense pain (p = 0.0089), increased lacrimation (ns) and more tactile, nociceptive and gustatory sensations (13 versus 1) compared to sham manipulation. No significant difference was observed for the other endpoints. Osteopathic manipulative treatment of the SPG may improve pharyngeal stability in obstructive sleep

  3. Sustained NMDA receptor hypofunction induces compromised neural systems integration and schizophrenia-like alterations in functional brain networks.

    Science.gov (United States)

    Dawson, Neil; Xiao, Xiaolin; McDonald, Martin; Higham, Desmond J; Morris, Brian J; Pratt, Judith A

    2014-02-01

    Compromised functional integration between cerebral subsystems and dysfunctional brain network organization may underlie the neurocognitive deficits seen in psychiatric disorders. Applying topological measures from network science to brain imaging data allows the quantification of complex brain network connectivity. While this approach has recently been used to further elucidate the nature of brain dysfunction in schizophrenia, the value of applying this approach in preclinical models of psychiatric disease has not been recognized. For the first time, we apply both established and recently derived algorithms from network science (graph theory) to functional brain imaging data from rats treated subchronically with the N-methyl-D-aspartic acid (NMDA) receptor antagonist phencyclidine (PCP). We show that subchronic PCP treatment induces alterations in the global properties of functional brain networks akin to those reported in schizophrenia. Furthermore, we show that subchronic PCP treatment induces compromised functional integration between distributed neural systems, including between the prefrontal cortex and hippocampus, that have established roles in cognition through, in part, the promotion of thalamic dysconnectivity. We also show that subchronic PCP treatment promotes the functional disintegration of discrete cerebral subsystems and also alters the connectivity of neurotransmitter systems strongly implicated in schizophrenia. Therefore, we propose that sustained NMDA receptor hypofunction contributes to the pathophysiology of dysfunctional brain network organization in schizophrenia.

  4. Regenerative Needs Following Alveolar Ridge Preservation Procedures in Compromised and Noncompromised Extraction Sockets: A Cone Beam Computed Tomography Study.

    Science.gov (United States)

    Koutouzis, Theofilos; Lipton, David

    2016-01-01

    The aim of this study was to evaluate the necessity for additional regenerative procedures following healing of compromised and noncompromised extraction sockets with alveolar ridge preservation procedures through the use of virtual implant imaging software. The cohort was comprised of 87 consecutive patients subjected to a single maxillary tooth extraction with an alveolar ridge preservation procedure for subsequent implant placement. Patients were divided into two main groups based on the integrity of the buccal bone plate following teeth extraction. Patients in the compromised socket (CS) group (n = 52) had partial or complete buccal bone plate loss, and patients in the noncompromised socket (NCS) group (n = 35) exhibited no bone loss of their socket walls following tooth extraction. Following 4 to 6 months of healing, all patients had a cone beam computed tomography (CBCT) study. Root-formed implants were placed virtually in an ideal prosthetic position. The number of implants per group and location (anterior, premolar, molar) exhibiting exposed buccal implant surface was calculated. In the CS group, 5 out of 19 anterior implants (26.3%), 4 out of 14 premolar implants (28.5%), and 7 out of 19 molar implants (36.8%) had exposed buccal surfaces. In the NCS group, 4 out of 9 anterior implants (44.4%), 2 out of 9 premolar implants (22.2%), and 4 out of 17 molar implants (23.5%) had exposed buccal surfaces. There were no statistically significant differences for intragroup and intergroup comparisons (χ² test, P > .05). This study failed to find statistically significant differences in the frequency of implants with exposed buccal surfaces placed virtually, following treatment of compromised and noncompromised sockets. A high proportion (22% to 44%) of sites had implants that potentially needed additional regenerative procedures.

  5. Fracture resistance of structurally compromised and normal endodontically treated teeth restored with different post systems: An in vitro study

    Directory of Open Access Journals (Sweden)

    Vajihesadat Mortazavi

    2012-01-01

    Full Text Available Background: With the aim of developing methods that could increase the fracture resistance of structurally compromised endodontically treated teeth, this study was conducted to compare the effect of three esthetic post systems on the fracture resistance and failure modes of structurally compromised and normal roots. Materials and Methods: Forty five extracted and endodontically treated maxillary central teeth were assigned to 5 experimental groups (n=9. In two groups, the post spaces were prepared with the corresponding drills of the post systems to be restored with double taper light posts (DT.Light-Post (group DT.N and zirconia posts (Cosmopost (group Zr.N. In other 3 groups thin wall canals were simulated to be restored with Double taper Light posts (DT.W, double taper Light posts and Ribbond fibers (DT+R.W and Zirconia posts (Zr.W. After access cavity restoration and thermocycling, compressive load was applied and the fracture strength values and failure modes were evaluated. Data were analyzed using two-way ANOVA, Tukey and Fisher exact tests (P<0.05. Results: The mean failure loads (N were 678.56, 638.22, 732.44, 603.44 and 573.67 for groups DT.N, Zr.N, DT.W, DT+R.W and Zr.w respectively. Group DT+R.W exhibited significantly higher resistance to fracture compared to groups Zr.N, DT.W and Zr.w (P<0.05. A significant difference was detected between groups DT.N and Zr.W (P=0.027. Zirconia posts showed significantly higher root fracture compared to fiber posts (P=0.004. Conclusion: The structurally compromised teeth restored with double taper light posts and Ribbond fibers showed the most fracture resistance and their strengths were comparable to those of normal roots restored with double taper light posts. More desirable fracture patterns were observed in teeth restored with fiber posts.

  6. Suboptimal T-cell receptor signaling compromises protein translation, ribosome biogenesis, and proliferation of mouse CD8 T cells.

    Science.gov (United States)

    Tan, Thomas C J; Knight, John; Sbarrato, Thomas; Dudek, Kate; Willis, Anne E; Zamoyska, Rose

    2017-07-25

    Global transcriptomic and proteomic analyses of T cells have been rich sources of unbiased data for understanding T-cell activation. Lack of full concordance of these datasets has illustrated that important facets of T-cell activation are controlled at the level of translation. We undertook translatome analysis of CD8 T-cell activation, combining polysome profiling and microarray analysis. We revealed that altering T-cell receptor stimulation influenced recruitment of mRNAs to heavy polysomes and translation of subsets of genes. A major pathway that was compromised, when TCR signaling was suboptimal, was linked to ribosome biogenesis, a rate-limiting factor in both cell growth and proliferation. Defective TCR signaling affected transcription and processing of ribosomal RNA precursors, as well as the translation of specific ribosomal proteins and translation factors. Mechanistically, IL-2 production was compromised in weakly stimulated T cells, affecting the abundance of Myc protein, a known regulator of ribosome biogenesis. Consequently, weakly activated T cells showed impaired production of ribosomes and a failure to maintain proliferative capacity after stimulation. We demonstrate that primary T cells respond to various environmental cues by regulating ribosome biogenesis and mRNA translation at multiple levels to sustain proliferation and differentiation.

  7. Radiotherapy of vertebral hemangiomas

    International Nuclear Information System (INIS)

    Sakata, Kohichi; Hareyama, Masato; Oouchi, Atushi; Sido, Mitsuo; Nagakura, Hisayasu; Tamakawa, Mituharu; Akiba, Hidenari; Morita, Kazuo

    1997-01-01

    Between 1975 and 1996, 14 patients (11 females, 3 males) with vertebral hemangioma received treatment with radiotherapy. Thirteen patients had a history of back pain or lumbago and 2 patients had neurological symptoms such as sensory impairment or paraplegia. The standard dose administered was 36 Gy in 18 fractions (five treatments per week). In the 13 patients with pain, this was completely or partially relieved. The condition of a man with hypesthesia of the legs deteriorated and a woman with paraplegia who was treated with decompressive laminectomy followed by radiotherapy recovered completely after irradiation. CT scan before irradiation showed thickened trabeculae as small punctate areas of sclerosis in all patients. At MR imaging before irradiation, T2-weighted MR images showed areas of high intensity in all patients and MR images demonstrated lesion enhancement. However, none of the patients who were treated successfully with radiation demonstrated any changes of the affected vertebra in the conventional radiographic films, CT scan or MR imaging, even 5 years after irradiation. Radiological imaging is indispensable for the diagnosis of vertebral hemangiomas but does not appear to be useful for evaluating the effects of radiotherapy. (orig.)

  8. Protein catabolism in pregnant snakes (Epicrates cenchria maurus Boidae) compromises musculature and performance after reproduction.

    Science.gov (United States)

    Lourdais, O; Brischoux, F; DeNardo, D; Shine, R

    2004-07-01

    In many species the high energetic demands of reproduction induce a negative energy balance, and thus females must rely on tissue catabolism to complete the reproductive process. Previous works have shown that both fat and protein are energy resources during prolonged fasting in vertebrates. While many ecological studies on energy costs of reproduction have focused on variations in fat stores, the impact of protein investment on the female has not been thoroughly investigated. Notably, as there is no specialized storage form for proteins, intense catabolism is likely to entail structural (musculature) loss that may compromise maternal physical performance after reproduction. Measurements on captive rainbow boas ( Epicrates cenchria maurus) confirm that reproducing females undergo significant protein catabolism (as indicated by elevated plasma uric acid levels) and show considerable musculature loss during gestation (as detected by reduced width of the epaxial muscles). Protein mobilization entailed a significant functional loss that was illustrated by decrements in tests of strength and constriction after parturition. In wild situations, such effects are likely to decrease the snakes' ability to forage and apprehend prey. Hence, the time period needed to recover from reproduction can be extended not only because the female must compensate losses of both fat stores and functional muscle, but also because the ability to do so may be compromised. Performance alteration is likely to be of equal or greater importance than reduced energy stores in the physiological mediation of elevated post-reproduction mortality rates and infrequent reproductive bouts (e.g. biannual or triannual), two common ecological traits of female snakes.

  9. Conjoined lumbosacral nerve roots compromised by disk herniation: sagittal shoulder sign for the preoperative diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Chang Ho [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, Seoul (Korea); Korea University College of Medicine, Department of Radiology, Anam Hospital, Seoul (Korea); Shin, Myung Jin; Kim, Sung Moon; Lee, Sang Hoon; Kim, Hee Kyung; Ryu, Jeong Ah [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, Seoul (Korea); Lee, Choon-Sung [University of Ulsan College of Medicine, Asan Medical Center, Department of Orthopedic Surgery, Seoul (Korea); Kim, Sam Soo [Kangwon National University College of Medicine, Department of Radiology, Kangwon (Korea)

    2008-03-15

    The objective was to determine the importance of the ''sagittal shoulder sign'' on magnetic resonance (MR) images for the diagnosis of conjoined lumbosacral nerve roots (CLNR) that are compromised by herniated disks. Magnetic resonance images of 11 patients (6 men and 5 women; age range, 25-71 years; average age, 48.7 years) with surgically proven CLNR, which was compromised by herniated disks, were retrospectively evaluated by two musculoskeletal radiologists. MR images were evaluated for the presence or absence of the sagittal shoulder sign - a vertical structure connecting two consecutive nerve roots and overlying disk on the sagittal MR images. The radiologists noted the type of accompanying disk herniation and bony spinal canal changes, as well as other characteristic MR features of CLNR, the common passage of two consecutive nerve roots through the neural foramen on axial MR images. The sagittal shoulder sign was identified with a mean frequency of 90.9% by the two observers (in 10 of 11 patients). The common passage of two consecutive nerve roots through the neural foramen on axial MR images was identified with a mean frequency of 59.1% (in 7 and 6 out of 11 patients, by observers 1 and 2, respectively). Good interobserver agreement for the sagittal shoulder sign was present (k = 0.621, p < 0.05). Observation of the sagittal shoulder sign may prove helpful for diagnosing CLNR in patients with disk herniation. In particular, this sign appears to be useful when there is no evidence of CLNR on axial MR images. (orig.)

  10. Management of periodontally compromised patient by orthodontic treatment: Does it help esthetically and biologically?

    Directory of Open Access Journals (Sweden)

    Subhash C Rao

    2012-01-01

    Full Text Available Adults are always keen to know whether they can still opt for orthodontic treatment and they often ask the same question to the orthodontist and the orthodontist replies, it primarily depends on the health of the bone supporting the teeth. Yes, it is the bone health which is of prime importance to undergo orthodontic treatment. Here is a case report of a patient who underwent orthodontic treatment because of lower midline spacing and protrusion of the upper anteriors. The bone health of the upper and lower anteriors was compromised. At the end of the treatment, there was marked improvement in the bone level and the profile of the patient.

  11. SUCCESSFUL APPLICATION OF PERIPHERAL VENO-ARTERIAL EXTRACORPOREAL MEMBRANE OXYGENATION FOR CARDIAC ALLOGRAFT ANTIBODY-MEDIATED REJECTION WITH SEVERE HEMODYNAMIC COMPROMISE

    Directory of Open Access Journals (Sweden)

    V. N. Poptsov

    2015-01-01

    Hg, PAP 31/21/12 mm Hg, PCWP 12 mm Hg, CI 3,4 l/min/м2, HR 85 in min, LVEF 53%, IVS 1.3 cm, mitral valve regurgitation <1 degree, inotropic support was discontinued. Control EMB showed resolution of AMR. Duration of VA ECMO was 7 days. Patient was discharged from ICU on the 2nd and 26th day after VA ECMO in stable clinical status. Conclusion. VA ECMO should be crucial component of treatment of cardiac allogaft antibody-mediated rejection with severe hemodynamic compromise

  12. Science Cases for the Velocity-Resolved Mid-J CO and 13CO and [Nii] Emissions

    Science.gov (United States)

    Okada, Yoko

    2018-01-01

    When studying PDRs, the contribution of shocks to the emission of mid-J CO lines is often debated. Plane-parallel PDR models fail to reproduce strong mid-J CO emissions, frequently leading to the conclusion of a shock contribution, while clumpy PDR models predict a flatter CO ladder in agreement with observations. One way to assign the origin of the mid-J CO lines is to investigate their velocity profile. Observations of mid-J 13CO emission profiles provide additional information as these lines are more likely optically thin. With AtLAST located on Cerro Chajnantor, a better atmospheric transparency compared to the ALMA plateau would enable efficient observations of the CO(6-5) to (8-7) lines. Under best weather conditions, it would be possible to observe up to CO(13-12) and the [NII]205 micron emission, which is essential to distinguish the ionized gas contribution to the [CII] emission observed from SOFIA. High angular resolution mapping in nearby galaxies with a large single dish telescope at high frequencies has a good synergy with interferometric observations at lower frequencies. Note that being at the high site does not compromise the zero-spacing-filling at low frequencies.

  13. The value of predicting restriction of fetal growth and compromise of its wellbeing: Systematic quantitative overviews (meta-analysis of test accuracy literature

    Directory of Open Access Journals (Sweden)

    Robson Stephen C

    2007-03-01

    Full Text Available Abstract Background Restriction of fetal growth and compromise of fetal wellbeing remain significant causes of perinatal death and childhood disability. At present, there is a lack of scientific consensus about the best strategies for predicting these conditions before birth. Therefore, there is uncertainty about the best management of pregnant women who might have a growth restricted baby. This is likely to be due to a dearth of clear collated information from individual research studies drawn from different sources on this subject. Methods/Design A series of systematic reviews and meta-analyses will be undertaken to determine, among pregnant women, the accuracy of various tests to predict and/or diagnose fetal growth restriction and compromise of fetal wellbeing. We will search Medline, Embase, Cochrane Library, MEDION, citation lists of review articles and eligible primary articles and will contact experts in the field. Independent reviewers will select studies, extract data and assess study quality according to established criteria. Language restrictions will not be applied. Data synthesis will involve meta-analysis (where appropriate, exploration of heterogeneity and publication bias. Discussion The project will collate and synthesise the available evidence regarding the value of the tests for predicting restriction of fetal growth and compromise of fetal wellbeing. The systematic overviews will assess the quality of the available evidence, estimate the magnitude of potential benefits, identify those tests with good predictive value and help formulate practice recommendations.

  14. Respiratory Failure Associated with the Lipodystrophy Syndrome in an HIV-Positive Patient with Compromised Lung Function

    Directory of Open Access Journals (Sweden)

    Natasha Press

    2001-01-01

    Full Text Available Protease inhibitors, used as treatment in human immunodeficiency virus (HIV infection, are associated with a syndrome of peripheral lipodystrophy, central adiposity, hyperlipidemia and insulin resistance. An HIV-positive patient with chronic obstructive pulmonary disease is presented who developed the lipodystrophy syndrome that is associated with the use of protease inhibitors. It is postulated that the lipodystrophy syndrome further compromised his lung function, leading to respiratory failure. Patients who have pulmonary disease and are taking protease inhibitors require monitoring of clinical status and pulmonary function tests.

  15. Dopamine D2 receptor function is compromised in the brain of the methionine sulfoxide reductase A knockout mouse

    OpenAIRE

    Oien, Derek B.; Ortiz, Andrea N.; Rittel, Alexander G.; Dobrowsky, Rick T.; Johnson, Michael A.; Levant, Beth; Fowler, Stephen C.; Moskovitz, Jackob

    2010-01-01

    Previous research suggests that brain oxidative stress and altered rodent locomotor behavior are linked. We observed bio-behavioral changes in methionine sulfoxide reductase A knockout mice associated with abnormal dopamine signaling. Compromised ability of these knockout mice to reduce methionine sulfoxide enhances accumulation of sulfoxides in proteins. We examined the dopamine D2-receptor function and expression, which has an atypical arrangement and quantity of methionine residues. Indeed...

  16. Synergistic effects of sodium 
ascorbate and acetone to restore compromised bond strength 
after enamel bleaching.

    Science.gov (United States)

    Boruziniat, Alireza; Manafi, Safa; Cehreli, Zafer C

    To evaluate the effect of a new experimental solution containing sodium ascorbate (SA) and acetone on reversing compromised bonding to enamel immediately after bleaching. The buccal surface of intact, extracted human premolars (n = 60) was bleached. The teeth were then randomly assigned to 6  groups according to the type of pretreatment applied prior to adhesive procedures: 10% SA in acetone-water solution applied for 1 and 5 min (groups 1 and 2, respectively); aqueous solution of 10% SA applied for 10 min (group 3); 100% acetone applied for 10 min (group 4); no pretreatment (negative control; group 5). An additional group (positive control; group 6) comprised unbleached teeth (n = 12). Two composite microcylinders were bonded on each specimen for evaluation of microshear bond strength (MBS) and failure modes. Data were analyzed using the one-way ANOVA and Tukey's post-hoc and chi-square tests at P = 0.05. Groups 1 and 2 yielded similar MBS values to groups 4 and 6 (positive control). The mean MBS of groups 3 and 5 (negative control) were similar, and significantly lower than that of the positive control group. The application of 10% SA in an acetone-water solution prior to bonding procedures can restore compromised enamel bond strength to its unbleached state within a clinically acceptable time of 1 min.

  17. 6 CFR 13.13 - Parties to the hearing.

    Science.gov (United States)

    2010-01-01

    ... 6 Domestic Security 1 2010-01-01 2010-01-01 false Parties to the hearing. 13.13 Section 13.13 Domestic Security DEPARTMENT OF HOMELAND SECURITY, OFFICE OF THE SECRETARY PROGRAM FRAUD CIVIL REMEDIES § 13.13 Parties to the hearing. (a) The parties to the hearing will be the Defendant and the Authority...

  18. Utilisation of symmetrical components in a communication-based protection for loop MV feeders with variable short-circuit power

    DEFF Research Database (Denmark)

    Ciontea, Catalin-Iosif; Bak, Claus Leth; Blaabjerg, Frede

    2018-01-01

    -circuit power is presented. It relies on utilisation of symmetrical components of the short-circuit currents and on communication between the protection relays. The proposed method addresses the Single Phase to Ground (SPG) faults occurring in directly grounded distribution networks, with focus on closed......Variability of the available short-circuit power also implies variation of the fault level, which can potentially cause several protection problems in the electric networks. In this paper, a novel protection method that is insensitive to the fault level changes caused by variable short......-loop Medium Voltage (MV) feeders. Case studies are presented, which demonstrate that the proposed protection scheme is capable of effectively detecting the SPG faults in closed-loop feeders with variable short-circuit power....

  19. Doppler changes as the earliest parameter in fetal surveillance to detect fetal compromise in intrauterine growth-restricted fetuses

    Directory of Open Access Journals (Sweden)

    Bansal Saloni

    2016-01-01

    Full Text Available Introduction. It is estimated that 3-10% of infants are growth restricted. Growth disturbances may have long-term issues. Doppler allows insight into the fetal response to intrauterine stress. Objective. The aim of this study was to detect fetal compromise in intrauterine growth-restricted (IUGR fetuses by means of biophysical profile (BPP vis-а-vis Doppler velocimetry studies of the fetal umbilical artery, and to find out which of the two is a better and earlier predictor of fetal compromise. Methods. A prospective study was conducted on a total of 50 singleton pregnancies with IUGR between 28 and 42 weeks of gestation. Study patients were managed expectantly with nonstress testing and amniotic fluid assessment, BPP and Doppler velocimetry studies of the fetal umbilical artery. Results. Fetal outcome was poor in 5/50 (10% of the fetuses, defined as presence of all of the following: poor Apgar test score, neonatal intensive care unit stay, necrotizing enterocolitis, and low birth weight. Of the four with abnormal BPP, 50% had poor fetal outcomes. Out of 46 with normal BPP, 6.5% had poor fetal outcomes. Conclusion. Inference drawn from the study is that the Doppler technology provides us the opportunity for repetitive noninvasive hemodynamic monitoring in IUGR pregnancies.

  20. Signal Timing Optimization Based on Fuzzy Compromise Programming for Isolated Signalized Intersection

    Directory of Open Access Journals (Sweden)

    Dexin Yu

    2016-01-01

    Full Text Available In order to optimize the signal timing for isolated intersection, a new method based on fuzzy programming approach is proposed in this paper. Considering the whole operation efficiency of the intersection comprehensively, traffic capacity, vehicle cycle delay, cycle stops, and exhaust emission are chosen as optimization goals to establish a multiobjective function first. Then fuzzy compromise programming approach is employed to give different weight coefficients to various optimization objectives for different traffic flow ratios states. And then the multiobjective function is converted to a single objective function. By using genetic algorithm, the optimized signal cycle and effective green time can be obtained. Finally, the performance of the traditional method and new method proposed in this paper is compared and analyzed through VISSIM software. It can be concluded that the signal timing optimized in this paper can effectively reduce vehicle delays and stops, which can improve traffic capacity of the intersection as well.